the child and newborn · dr ranjana chatterjee dr sutapa ganguly dr sumana kanjilal (dutta) dr atul...

The Child and NewbornWest Bengal Academy of Pediatrics, Oriental Apartments, Flat H115C, Canal Street, Kolkata 700 014Phone : 033 2265 4072, Email : [email protected], Website : www.wbap.inE-version of this journal available at website.

ISSN 0975-0894 RNI Registration No.:RNI/68911/97

EDITOR IN CHIEFDr Jaydeep ChoudhuryASSOCIATE EDITORSDr Subhasis BhattacharyaDr Sushmita BanerjeeCIRCULATION SECRETARYDr Kheya Ghosh UttamEDITORIAL BOARDDr Subroto DeyDr Debasish BandopadhyayDr Mihir SarkarDr Tryambak SamantaDr Moumita SamantaDr Bichitrobhanu SarkarDr Gautam Ghosh (Imm Past President, WBAP)Dr Aniruddha GhoshDr Suparna Guha

PAST EDITORSDr Umasankar SarkarDr Dilip MukherjeeDr Tapan Kr GhoshDr Subroto ChakraborttyDr Ranjana ChatterjeeDr Sutapa GangulyDr Sumana Kanjilal (Dutta)Dr Atul GuptaEX-OFFICIODr Arun Kr Manglik President, WBAPDr Pallab Chatterjee, Hony Secretary, WBAP

Special CorrespondanceDr Jaydeep ChoudhuryEditor-in-Chief, The Child and Newborn“Oriental Apartments” Flat H115C, Canal Street, Kolkata 700 014Email : [email protected] : [email protected]

Vol.22, No.1, January - March 2018 CONTENTS

Editorial Jaydeep Choudhury ........................................................................... 2

President’s Pen Neonatal Care : Where Do We Stand

Arun Kr Manglik .................................................................................. 3

Editors’ Forum Probiotics in Acute Childhood Diarrhea

Sutapa Ganguly .................................................................................. 4 Vitamin D and Respiratory Tract Infections : Are They Related?

Sumana Datta (Kanjilal) ....................................................................... 7

Review Section C-reactive Protein and Procalcitonin in Assessment of

Children with Fever in the Emergency DepartmentKalpana Datta, Supratim Datta ............................................................. 10

High Flow Nasal Cannula in ChildrenMihir Sarkar, Satyabrata Roychowdhury ............................................. 13

Gallery A Baby with Multiorgan Eosinophilic Infiltration

Abhisek Naskar,Madhumita Nandi ....................................................... 16 Fanconi Bickel Syndrome : A Rare Entity

Kausambi Basu, Arpan Saha, Jayati Das, Supratim Datta ...................... 20 Recurrent Pneumonia Due To Selective IgA Deficiency

in a 3 Months Old ChildAparajita Bera, Kalimuddin Khan, Suman Sarkar, Kallol Das .................. 22

Jaundice Due to Acute Hepatitis a With Underlying E-betaThalassemia and Wilson DiseaseSrikanta Baske, Suman Sarkar, Sayan Chatterjee ................................ 24

A 15 year Old Girl Presenting with Right Heart FailureTwo Years after Road Traffic Accident: Delayed Presentationof Tricuspid Valve InjuryAniruddha Ghosh, Mallar Mukherjee, Jaydeep Choudhury,Arunaloke Bhattacharya, Maya Mukhopadhyay ................................... 27

Medicolegal Tip Jaydeep Choudhury .......................................................................... 30

Editorial

The Child and Newborn has stepped into the 25th year of its publication this year. But the history of pediatricacademy in West Bengal and publications dates back to many years. The Calcutta Branch of Indian Academyof Pediatrics was formed in 1964 with Dr M L Biswas as President and Dr D N Chatterjee as Secretary withits office at the Institute of Child Health, Calcutta. In 1978 the Calcutta Branch was converted to West BengalBranch.The official journal of Indian Academy of Pediatrics, Indian Pediatrics, commenced publication in January1964. The office of the journal was in Calcutta. Institute of Child Health, Calcutta took the responsibility andDr Sisir Kumar Bose was the first Editor of the Journal. Followed by Dr D N Chatterjee. In 1969 the office of thenational journal, Indian Pediatrics was shifted to Delhi.Though IAP West Bengal Branch was formed in 1978 but it took some time to bring out our own journal TheChild, was first published in 1994. Later the journal was renamed The Child and Newborn. The first Editor-in-Chief was Dr Umasankar Sarkar who was succeeded by Dr Dilip Mukherjee and followed by Late DrTapan Kumar Ghosh. Over the years our journal has faced some hurdles but has managed to tide throughrough weather. We hope to carry forward the legacy and give it an identity.On this occasion of 25 years of The Child and Newborn we are glad to organize the first The Child andNewborn CME on 28 April 2018 where post graduate students will lock horns in a quiz contest and senioreditors will share their wisdom.In this era of publish or perish let us utilize this journal as a forum for exchange of knowledge where we canimbibe the wisdom of our senior members and a platform for the young pediatricans to present their casestudies and observations. I would like to end with the foreword of the first number of Indian Journal ofPediatrics in October 1933 where Dr K C Chaudhuri wrote “If the future of the nation really depends upon thewelfare of the child, it is necessary that steps should at once be taken to impress upon the people and theprofession the necessity of providing for the protection and safety of the child in every possible way”.

Dr Jaydeep ChoudhuryEditor in Chief

2 The Child and Newborn, Vol 22 No 1, January – March 2018

We are a country of about 130 crore people. With healthinsurance having not yet made significant inroads in mostfamilies, over 50% of our population still pays for their medicalexpenses on their own. This situation has led to a persistentincrease in poverty across all communities, with each passingyear several hundreds of families passing below BPL line dueto illness in the house.Care of sick neonates has been a persistent thorn in the fleshof most families because of a variety of reasons, eg noinsurance cover, no estimate of final expenses (duration ofhospital stay not known), final outcome of the baby uncertain,most doctors talking in negative tones etc. We as Pediatricians/Neonatologists have a very important role to ensure that thetruth is explained to the parents along with them also havingthe liberty to take reasonable decisions regarding treatmentof their baby.There are several issues which plague neonatal care. Withhuge advancements in neonatal care progressively we arehaving an increasing number of NICU graduates. But all goodthings do come at a cost. Issues include prematurity, LBW,sepsis, asphyxia, meconium and jaundice probably in thatorder of frequency. From the parents point of view, in ourcountry, with many parents bearing costs out of pocket, unlessthe patient is in a Public Sector Hospital or covered byInsurance, the costs to the family maybe huge. Do note, costswill include direct costs ie, what the family pays to the hospital,and indirect costs eg, loss of earnings, pay cuts etc. Whereinthese payments are made by the parents, no wonder they doexpect, albeit unrealistically 100% assured cure andoutcomes, despite being counseled almost always to thecontrary.

Neonatal Care: Where Do We Stand

Arun Kr ManglikPresident, West Bengal Academy of Pediatrics 2018

Correspondance : Arun K Manglik, Consultant Pulmonologist, Kolkata.Email : [email protected]

In 2018, intensive neonatal care may cost in various “five star”or corporate hospitals as high as Rs 20000/- to 50000/- perday, and duration of stay may extend to several weekssometimes. One can gauge that a month’s stay may entail anexpenditure upto 6-10 Lacs of rupees or more. Obviously ifoutcomes in the long run remain not very good, the parentstend to blame the Doctors and Hospital for their predicament.In the current scenario where Doctor – Patient trust is at a verylow level, accusations start flowing in at the slightestopportunity.In v iew of these diff icult situations, one may suggest,(a) complete transparency right from the very start of treatment,emphasizing on the rather very unpredictable long termoutcome, not forgetting the immediate chances of survival,(b) total explanation of the costs involved and how it mayescalate depending on stepping up of therapies as thesituation may demand, (c) parents should be free to take fullyinformed decisions regarding continuation of therapies asper their own choice, (d) option of stopping therapy will be agrey zone still, but must be undertaken with full judicialsanction, (e) the public sector must immensely invest into thissector, though somehow our decision makers are moreinclined towards cardiology and neurology etc rather thanneonatology (pardon me for being candid).I may have painted a not very bright picture of neonatal careas of now, but I am sure with time things will certainly improveand more Public Sector funds will flow here. With that theresponsibilities and work loads of our colleagues too will jackup.JAI HIND, JAI IAP, JAI WBAP

President’s Pen

The Child and Newborn, Vol 22 No 1, January – March 2018 3

Probiotics are defined as live microorganisms which whenadministered in adequate amount confer a health benefit onthe host (FAO/WHO 2001)1.The term probiotic is coined from Greek words ‘Pro’ (in favourof) and ‘Bios’ (life). Even in the pre-Christian era sour milk wasadministered as a health promotic food which had lactic acidbacillus as a natural component. Hippocrates the father ofmedicine, who lived from 460 BC to 350 BC had rightly saidthat “Let food be thy medicine and medicine be thy food”.Professor Elie Metchnikoff (1845-1916) a Russian scientist ofPasteur Institute and noble laureate, in the beginning of the20th century suggested that would be possible to modify thegut flora and replace the harmful microbes by useful ones”2.To be an ideal probitic agent, the microbes should be3

• From human source• Non-pathogenic• Resistant to gastric acid, bile and pancreatic digestion• Adhering to the intestinal epithelium to colonize the

enterocytes• Producing antibacterial substances like bacteriocins,

hydrogen peroxide, small chain fatty acids etc• Having favourable immunomodulation properties• Able to influence metabolic activitiesDifferent strains of Probiotic microbes which are commonlyusedLactobacillus• rhamnosus GG• acidophilus• plantarum• reutari• bulgaricus

Probiotics in Acute Childhood Diarrhea

Sutapa GangulyProfessor, Department of Pediatrics, KPC Medical College, Kolkata

Correspondance : Sutapa Ganguly. Professor, Dept of Pediatrics,KPC Medical College, Kolkata. Email : [email protected]

Bacillus• clausii, longneu, mesentericumBifidobacteria• bifidus, lactisStreptococcus• thermophilusNon-pathogenic yeast• saccharomyces-boulardii

According to current literature Bifidobacteria particularly lactisappear safest for newborns4. The most therapeutically effectivestrains on the controlled trials appear to be LGG andS.boulardii though some mixtures have also beneficialeffects5,6.

Origins of Lactobacilus rhamnosus GG (LrGG)

One of the most widely used probiotic agent with maximummember of research publications from lare number ofrandomized controlled trials. It was originally isolated fromfecal samples of healthy adult volunteer by Sherwood Gorbachand Barry Goldwin, explaining its surname letter GG7.

It has strong mucus adhering capacity compared to relateddairy strains (L.rhamnosus Lc705) and other probiotic strains8.This adherence property is attributed to fimbria likeappendages known as pili. It remains piliated in different stressconditions such as bile salts and low pH. The microbe persistslonger and in higher concentration in descending colon,recovered from feces even a week after administration.

Scenario of acute childhood diarrhea

Diarrhea accounted for 15% of 8.79 million under five deathsworldwide and 13% of the 1.83 million child deaths in India in2010, ranked 2nd to pneumonia as a major cause of mortalityin children. The severity of diarrhea in children is related toetiology rather than age and rotavirus is responsible for mostsevere cases. Dehydration is the main clinical feature andreflects severity.

Editors’ Forum


Guidelines in the management of acute childhooddiarrhea• Rehydration is the key treatment and reduced osmolarity

ORS containing sodium 75 mEq/L, glucose 75 mmol/Land osmolarity 245 mosm/L should be administered atlibitum

• Zinc deficiency is widespread amongst children living inthe developing countries like South Asia, Africa, LatinAmerica due to low intake of animal protein, high dietaryphytates and overall inadequate diets. Zinc plays a criticalrole in metalloenzymes, polyribosomes, in maintainingthe integrity of cell membrane and cellular functionsleading to the belief that it plays a key role in the cellulargrowth and function of the immune system.

IAP National Task Force and WHO have recommended zincas an adjunct to ORS and a dose of 10 mg elemental zinc forinfants 2 to 6 months of age and 20 mg/day for children 6months for 14 days. In the trials subjected to pooled analysis,zinc supplemented children had a 16% faster recovery and34% reduction in odds of acute diarrheal episodes lasting > 7days8. The total stool output was reduced by 31% than in theplacebo group.However oral rehydration therapy remains the main stay oftreatment. Regular feeding should not be interrupted andshould be carried on following initial rehydration. Antibioticsare not needed in most cases except Sheigollis, cholera, earlystage of campylobacter jejuni, systemic infection and inseverely malnourished children.In 2008 May, probiotics were included in the guideline formanagement of acute gastroenteritis by the joint committeeof ESPGHAN and ESPID. Documents were developed throughand incidence based systematic review quoting “probioticswith proven efficacy may be an effective adjunct to themanagement of diarrhea in appropriate doses”. Evidence ofefficacy were Ia for LrGG and IIB for S.boulardii9.The effect is highly significant among patients with acutewatery diarrhea and viral gastroenteritis, but not among thosewith invasive bacterial diarrhea. [https://www.clincinatichildrens.org/evidence[5a] accessed on 1stMarch, 2018]

A number of meta-analyses have evaluated the effects ofprobiotics for the treatment of AGE. A recent publication ofCochrane review which collected data from 63 RCT (n=8014)revealed probiotics reduced the duration of diarrhea byapproximately one day (35 RCTs n=4555, MD-25 hours) andrisk of diarrhea lasting 4 days {29 RCTS, n=2853). The majorityof the trials were carried out in infants and young children 46RCTs tested a single probiotic and 17 RCTs tested acombination of two to eight probiotics.LrGG:The Cochrane review found that use of LrGG reduced theduration of diarrhea by 27 hours, mean stool frequency on D2and the risk of diarrhea lasting 4 days. LrGG is more effectivewhen used in a daily dose of 10 colony forming units; treatmentwith LrGG to be started as early possible and to be continuedfor 5 to 7 days.S.boulardii:One meta analysis (search date August 2009) of 9 RCTsconcluded that in otherwise healthy infants and children, theuse of S.boulardii reduces the duration of diarrhea by 1 day.The risk of duration of diarrhea 4 days (6 RCTS n=606). Finally,the authors of the most recent review (search date October2011) included 13 RCTs carried out in Europe (mainly in Turkeyand one study in Italy) and in some non-European countries(Argentina, Bolivia, Cuba, India, Mexico, Myanmar andPakistan). The daily dose varied from 250 to 750 mg. None ofthe studies evaluated the effect of S.boulardii on stoolvolume10. Compared to the placebo or no intervention group,the sued of S.boulardii significantly reduced both the durationof diarrhea (11 RCTs, n=1306, MD-0.99 days) and risk ofdiarrhea on D3 (9 RCTs, n=1128). In hospitalized children,the use of S.boulardii reduced the risk of hospitalization.For other probiotic agents – Lactobacillus reuteri strain DSM17938, Heat Killed L.acidophilus LB, E.faecium,Bifidobacterium lactis Bb12, E.coli Nissde 1917 – quality ofevidence was very low and they are not recommended.Exclusion criteria for probiotic useIn the American Journal of Clinical Nutrition, Boyle et al haveidentified potential risk factors for probiotic sepsis and createda scoring system as shown in Table 1.

Major Factor Minor Factor• Immunocompromised state (malignancy, • Central venous catheter

steroids, debilitated state, preterms, HIV and • Impaired intestinal barrier (intestinal inflammationDiabetes Mellitus) concomitant administration of blood spectrum

antibiotics to which probiotic is resistant)• Valvular heart disease

One major factor or >one minor factor contain against use of probiotics.

Table 1 . Scoring system – factors against probiotics use


Limitation of conventional probiotic formulationProbiotics must pass through the harsh environment ofdigestive tract (gastric acid, bile acids, pancreatic juice andprotease r ich conditions) for optimal clinical benefits.Gastrointestinal survival rate is only 10-25% of the infesteddose. Many food and ingredients of food supplements aretoxic to probiotics and environmental conditions results inshortened shelf life.MicroencapsulationOne way to overcome all these limitations ismicroencapsulation of probiotic formulation.[12]Microencapsulation is the process by which small particlesor droplets are surrounded by a coating to producemicrocapsules in micrometer or millimeter range. Thistechnology is successfully applied in case of LrGG. Thecovering material should be safe for consumption and costeffective. Vegetable fat containing mono and diglycerides ofsaturated fatty acids, esterified polyglycerols and free fattyacids form good coating material. Agar, gram Arabic, cellulose,freeze dried silica alginate microbeads are also used forencapsulation. In recent years, the emulsion technology withmilk proteins as matrix material is a frequently investigatedtechnique14.Advantages of microencapsulation :It protects the bacterial cell from the environment: humidity,oxidation, pH and osmotic pressure. There is accelerationand amplification of the onset of beneficial effect. Gut survival

rate is enhanced upto 90% in encapsulated form with 5 timesmore efficient gut colonization and bacterial count than thesame uncoated strains. There is improvement in the stabilityand shelf-life of a probiotic formulation. The dosage of thecoated LrGG is 6 billion colony forming unit once daily.Probiotics to be protected from sunlight and moisture tomaintain stability; while using a probiotic preparation, vialshave to be shaken; sachets and dispersible tablets onceopened should be consumed immediately. The reconstitutedbottles have a short shelf-life and have to be protected fromsunlight usual side effects sun in practice are abdominaldiscomfort and flatulence.Conclusion• The use of probiotics (LrGG and S.boulardii) has been

suggested in the treatment of acute gastroenteritis, inaddition to early rehydration, zinc therapy and avoidanceof dietary restrictions.

• The effect is significant among patients with waterydiarrhea and viral gastroenteritis, but not among thosewith invasive bacterial diarrhea

• However one of the most important parameter whicheffects probiotic activity of a microorganism is its survivalduring gastroduodenal transit.

• Microencapsulation technique is a valid strategy tosignificantly improve gastro-resistance of strains, therapyenhancing their probiotic activity and allowing the use ofa 5 times lower amount.

Reference1. Joint FAO/WHO expert consultation on evaluation of health and

nutritional properties probiotics in food including powder milk withlive lactic acid bacteria. FAO/WHO report No. 10-1-2001.

2. Metchnikoff E. The prolongation of life: optimistic studies. London:GP Putnan and Sons; 1907.

3. Szajewska H,Mrukowicz JZ.Probiotics in the treatment andprevention of acute infectious diarrhea in infants and children: Asystematic review of published RCTs. J ped gastroenterol Nutr.2001; 33:S17-S25

4. Jhonston BC, Suprina AL et al. Probiotics for prevention of antibioticassociated diarrhea. Cochrane Database of Systematic Review2007, Issue 2, Art. No. CD004827. DOI: 10.1002/14651858.CD004827. Pub,2.

5. Szajewska H, Selly M et al. J Pediatr Gastroenterol Nutr, 2006:42: 454-475.

6. Szajewska H, Skorta A et al. Meta-analysis. Saccharomycesboulardii for treating acute diarrhea in children. Aliment PharmacolTher 2007; 25: 257-64.

7. Goldwin BR, Gorbach SL. Dig of Dis Sci, 1972.

8. Bhatnagar S, Lodha R etc. IAP Guidelines 2006 on managementof acute diarrhea. Indian Pediatr. Vol 44 – May 17, 2007

9. Szajewska H, Guarino A et al. Use of probiotics for management ofAcute Gastroenteritis: A position paper by ESPGHAN workingcommittee for probiotics and prebiotics JPGN. April 2014.

10. Ahmadi E, Alizadeh Navaei R et al. Efficacy of probiotic use in acuteRotavirus diarrhea in children: A systematic review andmetaanalysis. Caspian J Internal Med. 2015; 6: 187-195.

11. Boyle RJ, Robins-Browne RM et al. Probiotic use in clinical practicewhat are the risks? Am J Clin Nutr, 2006; 83: 1256-1264.

12. Delpiano M, Carmagnola S et al. Evaluation of intestinal colonizationby microencapsulated probiotic bacteria in comparison with thesame uncoated strains. J Clin Gastroenterol, 2010 Sep; 44 Suppl1: S42-6.

13. Serna-Cock L and Vallyo – Castillo V. Probiotic encapsulation.African J of Microbiol Res 2013 Oct; Vol. 7 (40): 4743-53.

14. Burgain J, Gaiani C et al. Encapsulation of LrGG in variousmicroparticles made of milk proteins. Innovative Food Science andEmerging Technologies. July 2013; 19: 233-242.


Respiratory tract infections (RTIs) are common worldwideand are responsible for significant morbidity and mortality. Themost common causal agents are the bacterium Streptococcuspneumoniae and influenza-virus. The preventive measure byvaccination may not be completely protective due to non-responders and microbial vaccine escape mechanisms.Treatment options include symptomatic treatment, antibioticsand antiviral drugs. Thus, additional ways to prevent orameliorate RTIs are needed and modulation of the hostimmune response could provide such an innovative approach.Over the years the role of vitamin D in modulating the immuneresponse to infection has generated considerable interest.The ability of vitamin D to enhance innate immunity throughits role in stimulating the production of a number ofantibacterial proteins, such as cathelicidin and defensins, andautophagosomes by activated monocytes and macrophagesis well documented1. Vitamin D also has a role in adaptiveimmunity reducing the acute inflammatory response andincreasing Th2 lymphocytes. Antimicrobial activity of vitaminD starts with recognition of bacterial products by toll-likereceptors2. Recent evidence suggests that vitamin D influencesseveral immune pathways, with the net effect of boostingmucosal defence while simultaneously dampening excessiveinflammation3. For example, vitamin D induces the geneencoding the antimicrobial peptide L3-L74. This peptide haspotent bactericidal capacity against a number of importantbacteria and viruses, including M. tuberculosis and influenza-virus5,6. Recent studies reported that there is an associationbetween vitamin D and RTI, but the exact mechanism is stillunclear.Vitamin D and its metabolites as in biologic setting, can besynthesized endogenously, so they are ‘hormones’ and‘hormone precursors’ rather than vitamins7. Less than 10% ofvitamin D is obtained from dietary source, most of circulating

Vitamin D and Respiratory Tract Infections : Are They Related?

Sumana Datta (Kanjilal)Professor , Department of Pediatric Medicine, IPGMER & SSKM Hospital, Kolkata

Correspondance : Sumana Datta (Kanjilal), Professor , Department of PediatricMedicine, IPGMER & SSKM Hospital, Kolkata, Email : [email protected]

vitamin D is provided from skin exposure to UVB radiation8. Atsolar noon the ratio of UVB and UVA is the highest and the onlytime when enough UVB photons reach the earth’s surface toproduce vitamin D is between 1000-1500 hours in the spring,and summer. Older people require more sun exposure thanchildren because children have greater capacity to producevitamin D. Specker, et al. reported that exposure to sunlight for30 min/week for infants in diaper and 2 hours/week for fullyclothed infants without head cover (since infants scalpprovides maximum surface area) maintained vitamin D level>11ng/dl. Asian children require more sunlight exposurebecause of dark skin colour. Excessive exposure to sunlightdoes not lead to vitamin D toxicity8.Vitamin D deficiency is the most common nutr itionaldeficiency and likely the most common medical condition inthe world. There is a multitude of causes of vitamin Ddeficiency, but the major cause has been the lack ofappreciation that the body requires 5- to 10-fold higher intakesthan is currently recommended by the Institute of Medicineand other health agencies9.The prevalence of vitamin D deficiency is 50-90% in the Indiansubcontinent and is attributed to low dietary calcium alongwith skin colour and changing lifestyle10.Among young Canadian children, function altering SNP inthe gene that encodes the vitamin D receptor (VDR) wasstrongly associated with the risk of acute lower respiratoryinfection (predominantly RSV bronchiolitis). These finding areconsistent with the hypothesis that Vitamin D-related pathwaysare implicated in the host immune response to viral respiratoryinfection11. Researchers have tried to find out any associationbetween Vitamin D status and RTIs by performing variousstudies. Larkin A, Lassetter J examined 18 studies and foundvitamin D deficiency was associated with increased risk orseverity of ALRI in 13 studies; associations were not found in 4studies12. Ahmed P, et al. performed a case control study onfifty children aged 2–60 months hospitalised with acute lowerrespiratory tract infections (ALRTI) and were studied

Editors’ Forum


prospectively. Each patient was matched with controls for ageand gender. In a multiple conditional logistic regressionmodel, only lower percentage of body surface area exposedto sunlight was associated with increased risk of acute LRTI.The percentage of body surface area exposed to sunlightwhile outdoors (P50.028) and vitamin D supplement use(P50.009) were independent determinants of vitamin Ddeficiency in the overall study population. They found acuteLRTI was not associated with vitamin D status, but wasassociated with less exposure to sunlight. Exposure to sunlightand vitamin D supplementation contributed to vitamin D statusin this population13. It has been seen that transplacentaltransport of vitamin D typically provides enough vitamin D forthe first two months of life unless there is severe maternalvitamin D deficiency. In a hospital based case control study inIndia on 150 children including 80 cases and 70 controls,aged 2-60 months the researchers found subclinical vitaminD deficiency and nonexclusive breastfeeding in the first 4months of life were significant risk factors for severe ALRI inIndian children14. There are various observational studiesstating that vitamin D deficiency is a predisposing factor forinfections and leads to increased risk of both upper RTI (URTI)and lower RTI (LRTI)12.14. There are various studies on relationbetween LRTI and vitamin D status, but there are lack of studiesrelating URTI and vitamin D status which are obviously needed.Recurrent respiratory tract infection (RRTI) is defined as achild having more than six times of URTI or two times of LRTIper year with an interval between two infections of at leastseven days. Children living in Northern latitudes lack sunlight;thus, vitamin D supplements and more sun exposure areneeded to prevent recurrent RTIs (RRTI) during winter. ZhangX, et al. performed a study on 1200 children between 6mo-12yrs age group in China and they concluded that a cut-off valueof vitamin D lower than 19.45 ng/mi may be a risk factor forRRTI15. In a study in New Zealand, it was found that adultpatients admitted to the hospital with pneumonia are more

likely to die if they have vitamin D deficiency. The associationbetween vitamin D deficiency and morbidity was not explainedby patient age, sex, co morbidities, the severity of the systemicinflammatory response, or other known prognostic factors16.In a study , no difference was observed in vitamin D levelsbetween the entire ALRI group and control groups17.In another study M. Cobey Lopez found that lower levels of25-OHD were correlated with severity of the disease18.So, majority of the studies revealed an association betweenvitamin D status and RTIs. But there are controversiesregarding its actual cause and effect relationship, becausethere are many confounding factors in these studies. As a partof vitamin D synthesis is dependent on dietary source , so foodhabit, breast feeding, formula feeding, mother’s vitamin D levelduring breast feeding – all are strongly related with vitamin Dstatus of a child. As major part of vitamin D synthesis isdependent on sunlight exposure, so duration of sun exposure,It’s timing percentage of body surface area exposed, seasonor climate – these factors have also direct relationship withvitamin D status of any individual. Other demographic factorslike, age, gender and ethnicity are other confounding factors.Colour of skin and geographic distribution of study populationmay also have some impact on Vitamin D level becausevitamin D status may vary significantly in populationsdepending on geographical, social and economic factors.With increasing awareness for detection of vitamin Ddeficiency state and ease of vitamin D supplementation incase of deficiency, vitamin D supplementation is likely todecrease susceptibility to infection, which is highly effectivein prevention of childhood morbidity and mortality. Morestudies are required to find out whether therapeutic dose ofsupplemental vitamin D in infants and children which is beinggiven globally is appropriate to prevent RTIs in children.So to conclude, "improved understanding of Vitamin D and itsrole in immunity” may help in better ways to prevent and/ortreat pneumonia and other respiratory tract infections.Reference

1. Hewison M. Vitamin D and immune function : an overview. ProcNutr Soc. 2012; 71:50-61.

2. Hewison M. Vitamin D and innate and adaptive immunity. VitamHorm. 2011; 86:23-62

3. Pfeffer PE, Hawrylowicz CM. Vitamin D and lung disease. Thorax2012; 67:1018–1020.

4. Gombart AF, Borregaard N, Koeffler HP. Human cathelicidinantimicrobial peptide (CAMP) gene is a direct target of the vitamin Dreceptor and is strongly up-regulated in myeloid cells by 1,25-dihydroxyvitamin D3. FASEB J. 2005; 19: 1067–1077.

5. Barlow PG, Svoboda P, Mackellar A, Nash AA, York IA, et al.

Antiviral activity and increased host defence against influenza infectionelicited by the human cathelicidin LL-37. PLoS One. 2011; 6: e25333

6. Rivas-Santiago B, Rivas Santiago CE, Castaneda-Delgado JE,Leon-Contreras JC, Hancock RE, et al. Activity of LL-37, CRAMPand antimicrobial peptide-derived compounds E2, E6 and CP26against Mycobacterium tuberculosis. Int J Antimicrob Agents. 2013;Feb; 41(2): 143-8.

7. Bringhurst FR, Demay MB, Krane SM, Kronenberg HM. Bone andMineral Metabolism in Health and Disease. In: Kasper DL, HauserSL, Jameson JLet al. Harrison’s Principle of Internal Medicine. 19thEdition ,United States of America: Mc Graw Hill; 2015: Pp.2454-66.


8. Specker BL, Valanis B, Hertzberg V, Edwards N, Tsang RC.Sunshine exposure and serum 25- hydroxy vitamin D concentrationsin exclusively breastfed infants. J. Pediatr. 1985; 107:372–376.

9. Holick MF. Vitamin D: extraskeletal health. Rheum Dis Clin NorthAm. 2012; 38:141-60.

10. Harinarayanan CV, Joshi SR. Vitamin D status in India- Its implicationand remedial measures. J Assoc Physician. 2009 ; 57: 40-8

11. Roth DE, Jones AB, Prosser C, Robinson JL & Vohra S. Vitamin Dreceptor polymorphism and the risk of acute lower respiratory tractinfection in early childhood. J Infect dis. 2008; 197: 676-680.

12. Larkin A, Lassetter J. Vitamin D deficiency and acute lower respiratoryinfections in children younger than 5 y: identification and treatment.J Pediatr Health Care. 2014;28:572–82.

13. Ahmed P, Babaniyi B, Yusuf KK et al. Vitamin D status andhospitalisation for childhood acute lower respiratory tract infectionsin Nigeria. Pediatr Int Chid Health. 2015;36: 151-6

14. Wayse V, Yousafzai A, Mogale K, Filteau S. Association of subclinicalvitamin D deficiency with severe acute lower respiratory infection inIndian children under 5 y. Eur J Clin Nutr. 2004;58:563–7.

15. Zhang X, Ding F, Li H, Zhao W, Jing H, Yan Y, et al. Low SerumLevels of Vitamins A, D, and E Are Associated with RecurrentRespiratory Tract Infections in Children Living in Northern China: ACase Control Study. PLoS ONE 2016;11(12): e0167689.

16. Wiley-Blackwell. Vitamin D deficiency in pneumonia patient associatedwith increased mortality. Science daily, 2011.

17. Sismanlar T, Aslan AT, Gülbahar Ö, Özkan S. The effect of vitamin Don lower respiratory tract infections in children. Turk Pediatri Ars.2016 1;51(2):94-9.

18. Cebey-López M. Role of Vitamin D in Hospitalized Children Withlower Tract Acute Respiratory Infections. J Pediatr GastroenterolNutr. 2016; 62(3) : 479-85

WBAP The Child and Newborn CME28 April 2018, The Gateway Hotel, Kolkata

09.00 – 11.00 The Child and Newborn PG QuizQuiz Masters: Arunaloke Bhattacharya, Anjan Das, Debadatta Mukhopadhyay

11.00 – 12.15 Lectures by Past EditorsKawasaki disease: My Observation – Subroto ChakrabarttyBehavioral problems in adolescents – Ranjana ChatterjeeProbiotics in Acute Gastroenteritis in Children –Sutapa GangulyEarly diagnosis of cerebral palsy – Sumana KanjilalTreating bronchiolitis – Atul Gupta

12.15 – 12.45 “The Child and Newborn” Dr Tapan Kr Ghosh Lecture :An Update on Prevention of Rabies. – Digant ShastriChairpersons: Dilip Mukherjee, Arup Roy

12.45 – 01.15 The Child and Newborn Felicitation


IntroductionFever is one of the most common presenting symptoms inpediatric emergency care, which accounts up to 10-30% ofconsultations1. Children up to an age of 18 months have onaverage eight febrile episodes; in 20-40% cases professionalhealth care provider is consulted2. Only minority (7-15%) offebrile children has a serious bacterial infection(SBI)3,4, withpneumonia and urinary tract infection(UTI) being mostfrequent type of infections. The incidence of SBI, and inparticular, the occurrence of bacterial sepsis and meningitisdropped markedly over the last few years owing to theexpansion of national vaccination schemes5. Yet, neither thenumber of febrile children visiting emergency care facilitiesnor parental health-seeking behavior has changed6. Also, anincrease in short stay hospital admission among children withacute infections was recently demonstrated7. Moreover, asignificant number of febrile children continue to receiveantibiotics that are unlikely to benefit them with the addedeffect of contributing to the development of antimicrobialresistance, while other children succumb to treatableinfectious diseases as a result of failure to recognise SBI atan early stage of disease8.As clinical signs and symptoms are often of limited value indistinguishing SBI from self limiting febrile illness9, biomarkers,such as C- reactive protein (CRP) and procalcitonin (PCT),are often used for this purpose.Physioogy of CRP and PCTCRP :(i) CRP was first described in 1930 by Tillet and Francis10.

They discovered a protein that precipitated withpneumococcal C- polysaccharide in patients withpneumococcus pneumonia. CRP is now known to beacute phase protein secreted by liver in response to tissuedamage or inflammation. Its clinical use is advocated in

C-reactive Protein and Procalcitonin in Assessment of Children with Fever inthe Emergency Department

Kalpana Datta*, Supratim Datta***Professor of Pediatrics, Medical College Kolkata , **Professor and HOD ,Pediatrics, IPGME&R,SSKM Hospital Kolkata

Correspondance : .Kalpana Datta. Professor of Pediatrics, MedicalCollege Kolkata. Email : [email protected]

a wide array of pediatric and adult diagnostic dilemmas.(ii) The CRP gene is located on chromosome 1 (1q23.2)11.

It is a member of the small pentraxins family. It has 224amino acids12, CRP binds to the phosphocholineexpressed on the surface of dead or dying cells and somebacteria. This activates the complement system,promoting phagocytosis by macrophages, which clearsnecrotic and apoptotic cells and bacteria.

(iii) This acute phase response occurs as a result of a rise inthe concentration of IL-6, which is produced bymacrophages13 as well as adipocytes14. in response to awide range of acute and chronic inflammatory conditionssuch as bacterial, viral, or fungal infections; rheumaticand other inflammatory diseases; malignancy; tissueinjury and necrosis. These conditions cause release ofinterleukin-6 and other cytokines that trigger the synthesisof CRP and fibrinogen by the liver.

PCT :(i) Increased levels of PCT in children with signs of systemic

infections were first reported in the early 1990s by Assicotet al15. Earlier studies in adult had looked at PCT inMedullary Thyroid carcinoma and staphylococcal toxicshock syndrome16. PCT is a 116- amino acid peptideand a precursor of calcitonin and is believed to be anamplifier of the inflammatory cascade17.

(ii) The reference value of procalcitonin in adults andchildren older than 72 hours is 0.15 ng/mL or less. Inneonates aged less than 72 hours, a procalcitonin levelof more than 1 ng/mL at birth, 100 ng/mL or more at age24 hours, and 50 ng/mL or more at age 48 hours suggestsserious bacterial infection.

Differences in protein kinetics between PCT and CRP resultin earlier increased serum concentration level of PCT thanCRP levels18-20. For example, PCT could be detected inplasma 2 hours after the intravenous injection of endotoxins,whereas CRP could be detected only after 12 hours. In

Review Section


addition, PCT concentration levels rose during the first 6-8hours and reached a plateau after approximately 12 hours,before returning to normal concentration levels within 2-3 days.In contrast CRP reached a plateau after 20-72 hours anddecreased to normal values within 3-7 days18-20.CRP is used mainly as a marker of inflammation. Apart fromliver failure, there are few known factors that interfere withCRP production. Interferon alpha inhibits CRP productionfrom liver cells which may explain the relatively low levels ofCRP found during viral infections compared to bacterialinfections21.Measuring and charting CRP values can prove useful indetermining disease progress or the effectiveness oftreatments. ELISA, immunoturbidimetry, nephelometry, rapidimmunodiffusion, and visual agglutination are all methodsused to measure CRP.A high-sensitivity CRP (hs-CRP) test measures low levels ofCRP using laser nephelometry. The test gives results in 25minutes with a sensitivity down to 0.04 mg/L.CRP, PCT and duration of feverIt has been suggested that PCT is a better marker than CRPto identify SBI in children with a short duration of fever. A studyby Lauces-Cubells, et al found PCT to be superior to CRP ininfants with fever of less than 8 hours duration. SimilarlyOlaciregui, et al concluded that PCT was more useful thanCRP in young febrile infants aged less than 3 months with anonset of fever less than 12 hours22.Discussion The management of febrile young children without apparentsource of infection remains controversial, because there hasbeen no test available with adequate sensitivity and specificityrequired to distinguish children with occult bacterial infectionfrom non-bacterial illness. Blood culture is the gold standardto detect occult bacteraemia; however, results are not quicklyavailableUsing biomarkers as a diagnostic tool in the evaluation offebrile children should always be supportive of a clinicalimpression and working diagnosis. However some evidencesuggest that routinely using biomarkers in febrile children willidentify children with an SBI that would otherwise have beenmissed on clinical judgment only3,7,4.

Conversely high levels of PCT and CRP in a child that isclinically at subjectively low risk for SBI should warrant areassessment safety netting prior to discharge home.Moreover clinicians must balance the harms and the cost ofroutinely testing all children with fever against the risk ofmissing children with SBI.SBI potentially have an aggressive clinical course withsignificant morbidity and mortality, and early detection of SBIof those children are needed. Deciding between CRP andPCT will depend on local preferences mainly, whileconsidering that scientific evidence of CRP somewhat moreextensive. Additionally at present CRP is cheaper of the twotest and CRP is available as a validated quantitative bed sidetest.Clinical bottom line(i) Children with serious bacterial infections and occult

bacteremia are more likely to have high CRP than childrenwith benign infections. (Grade B)

(ii) A single CRP value gives a probability but never a certaintyof presence or absence of serious bacterial infection.(Grade B)

(iii) The use of CRP alone or with other factors may enhanceclinicians' abilities in the early recognition of clinicallyundetectable serious bacterial infection, allowing for amore selective strategy for determining which childrenneed additional diagnostic studies and antibiotictherapy23.

Key points for clinical practiciceCRP and PCT are both equally suitable markers for ruling inor ruling out SBI in children with fever. Cut off of 20mg/L ofCRP or 0.5ng/ml for PCT are useful for ruling out SBI. Cut offs80mg/L for CRP or 2ng/ml for PCT are useful for ruling in SBI.Given the high diagnostic value of biomarkers in addition toclinical signs, it is recommended doing a CRP or PCT in allchildren with an increased risk of SBI, as for example thewarning signs by the guideline of NICE for the managementof febrile children.CRP and PCT values should always be interpreted in the lightof the clinical context and be used for decisive but rather assupportive tools in clinical decision making.

Reference1. Alpen ER, et al, pediatric emergency care 2006;22:689-6992. Hay AD, et al, Farm Pract 2005;22:367-3743. Craig JC, et al BMJ 2010;340:c15944. Nijman RG, et al BMJ 2013;346:f 1706

5. Marting NG, et al. Lancet Infect Dis 2014;14:397-4056. Cricetti M, et al. Pediatrics 2001;107:1241-12467. Gill PJ, et al . Arch Dis Child 2013; 98: 328-3348. Thompson MJ, et al. Lancet 2006;367:397-403


9. Van den Bruel, et al. Lancet. 2010;375:834-84510. Tillet WS, et al. J Exp Med1930;52:561-57111. https://www.ncbi.nlm.nih.gov/gene/140112. NCBI Entrez Protein #CAA3967113. Pepys MB, Hirschfield GM (Jun 2003). "C-reactive protein: a critical

update". The Journal of Clinical Investigation. 111 (12): 1805–12.doi:10.1172/JCI18921. PMC 161431? . PMID 12813013.

14. Jump up to:a b Lau DC, Dhillon B, Yan H, Szmitko PE, Verma S(May 2005). "Adipokines: molecular links between obesity andatheroslcerosis". American Journal of Physiology. Heart andCirculatory Physiology. 288 (5): H2031–41. doi:10.1152/

ajpheart.01058.2004. PMID 1565376115. Assicot M, et al. Lancet 1993;341:515-51816. Becker KL, et al. J Clic Endocrinol Metab 2004;89:1512-152517. Pierce R, et al. Curr Opin Pediatr 2014;26:292-29818. Luaces-Cubells C, et al, Pediatr. Inf.Dis. J 2012;31:645-64719. Pratt A, et al. Pediatr. Int 2007;40:31-3520. Andreola B, et al, Pediatr. Infect. Dis.J 2007;26:672-67721. Enocsson H, et al. Arthritis and Rheumatism. 60 (12): 3755-60.22. Olaciregui I, et al. Arch Dis Child 2009;94:501-50523. Arch Dis Child. 2006 Jun; 91(6): 533–535.


IntroductionOver the last decade High Flow Nasal Cannula (HFNC) oxygendelivery, also sometimes called Heated Humidified High FlowNasal Cannula (HHHFNC) has emerged as a new method toprovide respiratory support for neonates to adults with hypoxia1.Nasal cannulae, which were first used to administersupplemental oxygen (low-flow therapy) on a large scale, alsoshowed the capability for the administration of nCPAP throughhigh-flow nasal cannulae (HFNC) as it developed. Needlessto say, apart from meeting specific physical criteria, a relativehumidity of 100% and a temperature of 370C are the basicrequirements of this intervention2.The application of HHHFNC in the NICU (Neonatal IntensiveCare Unit) and PICU (Pediatric Intensive Care Unit) hasdeveloped significantly during the last decade due to the factthat not only is this system capable of providing a specificpercentage of the respiratory oxygen, but it can also administernoninvasive respiratory support of constant-flow CPAP withoutthe need for any further equipment3.In children, its use has particularly proliferated for infants andyoung children hospitalised with bronchiolitis. However, theevidence for the safety or effectiveness of HFNC as arespiratory support in children is relatively lacking, asunderlined in two Cochrane reviews from 20144. Despite that,HFNC has been increasingly implemented in clinical practice,and given that modification, it is essential that physiciansshould keep abreast of the latest knowledge.Definition of HFNCIn the Cochrane review from 2014, HHHFNC in children wasdefined as heated, humidified and blended air/oxygendelivered via nasal cannula at different flow rates 2 l/min,delivering both high concentrations of oxygen and potentiallycontinuous distending pressure4.

High Flow Nasal Cannula in Children

Mihir Sarkar*, Satyabrata Roychowdhury***Associate Professor, **RMO Cum Clinical Tutor, Department of Pediatrics, Medical College Kolkata

Correspondance : Mihir Sarkarik, Associate Professor, Department ofPediatrics, Medical College Kolkata Email : [email protected]

Low flow and high flow devicesOxygen applicators can be basically divided into low-flow andhigh-flow devices4. Low-flow devices such as nasal catheters,cannulas or simple masks deliver a limited flow of 100%oxygen (1 – 15 l/min maximum) which is mixed with the totalinspiratory gas flow of the patient. The effective inspiratoryoxygen concentration (FiO2) depends on the respiratory flowand breathing pattern of each individual patient and thereforeis difficult to estimate in advance5.Modern high-flow nasal cannula (HFNC) devices equippedwith an active humidification chamber can provide gas flowrates up to 70 l/min, which are higher than the patient’srespiratory flow, and therefore allow a controlled delivery of adefined FiO2 up to 1.0, independent of the breathing pattern ofeach patient4. HFNC might also reduce the work of breathing.HFNC requires active heating and humidification of therespiratory gases in order to prevent damage of the respiratoryepithelium and reduces discomfort during therapy.MechanismWhen the respiratory disease is accompanied by increasingrespiratory work, supportive mechanisms of HHHFNC arespecifically categorized as follows:Dead space ventilation in the nasopharyngeal space:High-flow therapy (HFT) may eventually enhance alveolarventilation by decreasing the dead space through establishingwashout in the nasopharyngeal space by gas insufflations(GI), which in turn increases the minute ventilation.Decrease in respiratory work:This is the result of providing some level of splinting in thenasopharynx, which has a significant ability of compliance.When HFT produces GIs beyond demand flow in thenasopharynx, it avoids the retraction of the nasopharynx wallin inspiration and with the lowering of resistance in this space;the respiratory work also decreases in inspiration. Moreover,during expiration, the expiratory flows face resistance in the

Review Section


nasopharynx and are redirected to the oropharynx, whicheventually decreases the expiratory work because of theoccurrence of the Coanda effect in the behavior of the gas.Improvement of airway conductance and pulmonarycompliance :By reducing the effect of cold air; an in vitro study has shownthat inspired gas with low humidity even for short periods mayresult in worsened function of human airway epithelial cellsinflammatory indices. Reduction of the metabolic cost of gasconditions by providing air with 100 % relative humidityProviding the maximum humidity and temperature:To establish optimal gas exchange, the airways increase thetemperature and RH of the inhaled gases to 370C and 100%,respectively, while the HFT systems blocks energy wastagein the airways by establishing these conditions and eventuallyimproving lung mechanics.Pressure generated by HFNCRecent studies have suggested limited pressure delivery asmeasured in pharynx and oesophagus, ranging from 2–4 cmH2O both in children5. Overall, the distending airway pressureappears to be dependent on the weight/ size of the patient,flow rate, and the diameter of the nasal cannula compared tothe nares, with a higher pressure being delivered when themouth is closed.Level of flowThe optimal maximal flow for HFNC is not known. Flow ratesup to 1.5-2 L/kg/min are being used in children both in generalpaediatric wards and PICUs. In a recently published reviewfrom Hutchings et al., a guideline for the initiation and strategiesfor escalation and weaning of HFNC in a general paediatricward was suggested6. In this local guideline, the initial flow isset dependent on age, and the flow is increased if the pointsin a particular patient scoring system are above a given triggerlevel.IndicationsHFNP are used for the same indications as the traditionalmethod of CPAP using a nasopharyngeal tube:• Respiratory distress from bronchiolitis, pneumonia,

congestive heart failure, etc• Respiratory support post extubation and mechanical

ventilation• Weaning therapy from mask CPAP or BIPAP• Respiratory support to children with neuromuscular

disease

• Apnoea of prematurityHigh flow can be used if there is hypoxaemia (SpO2<90%)and signs of moderate to severe respiratory distress despitestandard flow oxygen.Contraindications• Blocked nasal passages/choanal atresia• Trauma/surgery to nasopharanyxEquipmentFigure 1 shows the various equipments• Oxygen and air source• Blender• Flow meter• Humidifier• Circuit tubing to attach to humidifier

• Children <12.5kg: small volume circuit tubing• Children =12.5kg: adult oxygen therapy circuit tubing

• Nasal cannula (prongs) to attach to humidifier circuittubing (size to fit nares comfortably)• Newborn: Premature or Neonatal (maximum flow

8L/min)• Infants and children up to 10kg: Infant (max flow

20L/min) or up to 12.5kg: Paediatric cannula (maxflow 25L/min)

• Water bag for humidifier• Nasogastric tube

Fig 1. Equipments of HFNC


Patient management• Secure nasal cannula. Prongs should not totally occlude

nares.Flow rate• =10Kg : 2 L per kg per minute• >10Kg : 2 L per kg per minute for the first 10kg + 0.5L/kg/

min for each kg above that (max flow 50 L/min). [7]FiO2 – Start at 50-60% for bronchiolitis and respiratorydistress.Target range for SpO2 of 94%-98%• 75-85% in cyanotic congenital heart disease with

balanced circulationHumidification• Set humidifier on 370C invasive setting (length from

temperature probe to nares will result in temperature dropto comfortable level whilst maintaining optimal humidity)

Patient monitoring• Monitor patient for response• Respiratory rate• Heart rate• Degree of chest in-drawing• SpO2

• Within 2 hours it should be possible to reduce the FiO2and clinical stabilisation should be seen• The FiO2 required to maintain SpO2 in the target

range (as above) should decrease to <40%• The heart rate and respiratory rate should reduce by

20%• Chest in drawing and other signs of respiratory

distress should improve• Seek medical review if any of the following occurs:

• The patient is not stabilising as described above

• The degree of respiratory distress worsens• Hypoxaemia persists despite high gas flow• Requirement for >50% oxygen.

Patient nursing care• All infants on high flow should have a nasogastric tube• Once stable on high flow, the infant should be assessed

as to whether they can feed. Some infants can continueto breast feed, but most require feeding via a nasogastrictube

• Regularly aspirate the NG 2-4 hourly for air• Oral and nasal care must be performed 2-4 hourly• Note nasal prongs are in correct position and no pressure

areas to nares.Patient comfort with high flowIn a Norwegian study among newborns, no difference wasfound in patient comfort on HFNC and CPAP, but parentspreferred HFNC to CPAP, reporting that their child was moresatisfied, and that they perceived that it was easier to interactwith their child when they were on HFNC8. In a study on 46children with various causes of respiratory distresses from 0to 12 years of age, and found that patient comfort measuredby COMFORT scale improved when switching from oxygendelivered by nasal cannula or face mask to HFNC9.ConclusionHFNC is a relatively safe, well-tolerated and feasible methodfor delivering oxygen to infants and young children in a generalpaediatric ward. Most of the clinical studies in children havebeen observational studies conducted in infants withbronchiolitis. A positive clinical effect on various respiratoryparameters has been detected, and studies suggest thatHFNC may reduce the work of breathing. HFNC may alsodecrease the need of CPAP and invasive ventilation in infantsand children. RCTs performed.

Reference1. Lee JH, Rehder KJ, Williford L, Cheifetz IM, Turner DA. Use of high

flow nasal cannula in critically ill infants, children, and adults: a criticalreview of the literature. Intensive Care Med. 2013;39(2):247–257.

2. Wiswell TE, Courtney SE. Noninvasive Respiratory Support. In:Goldsmith JP, Karotkin EH, editors. Assisted Ventilation of the Neonate.5th ed. St. Louis: Elsevier Saunders; 2011. p. 140.

3. Dysart K, Miller TL, Wolfson MR, Shaffer TH. Research in high flowtherapy: Mechanism of action. Respir Med. 2009;103:1400–5.

4. Mayfield S, Jauncey-Cooke J, Hough JL, Schibler A, Gibbons K,Bogossian F. High-flow nasal cannula therapy for respiratory supportin children. Cochrane Database Syst Rev. 2014;3:CD009850.

5. Arora B, Mahajan P, Zidan MA, Sethuraman U. Nasopharyngealairway pressures in bronchiolitis patients treated with high-flow nasal

cannula oxygen therapy. Pediatr Emerg Care. 2012;28:1179–84.6. Hutchings FA, Hilliard TN, Davis PJ. Heated humidified high-flow

nasal cannula therapy in children. Arch Dis Child. 2015;100:571–5.7. Royal Children's Hospital, Melbourne, Australia, Clinical Practice

Guideline on High Flow Nasal Prong HFNP oxygen, [Internet, lastupdated 2013 July 24; cited 2018, January 01]. Available from: http://www.rch.org.au/clinicalguide/index.cfm

8. Klingenberg C, Pettersen M, Hansen EA, Gustavsen LJ, Dahl IA,Leknessund A, et al. Patient comfort during treatment with heatedhumidified high flow nasal cannulae versus nasal continuous positiveairway pressure: a randomized cross-over trial. Arch Dis Child FetalNeonatal Ed. 2014;99:F134–7.

9. Spentzas T, Minarik M, Patters AB, Vinson B, Stidham G. Childrenwith respiratory distress treated with high-flow nasal cannula. JIntensive Care Med. 2009;24:323–8.


IntroductionHerein we discuss a child presenting withfever,hepatoslpenomegaly,leucocytosis and hugely increasedabsolute eosinophil count and multiorgan eosinophilicinfiltration. We discuss the recent advances made in thediagnostic approach to a child with eosiniphilia , the latestmolecular technologies used to specify a diagnosis tostreamline the management modalities.Case reportA one year three month old male child born out of a nonconsanguineous marriage presented with fever and coughfor one month. On examination, the child had pallor andhepatosplenomegaly.Liver was 3 cm enlarged below costalmargin.It was non tender, soft in consistency with a roundedmargin and a smooth surface. Spleen was 1 cm palpablebelow left costal margin.No abnormality could be found inexamination of other systems.On investigating,Hb was 8.7gm/dl,platelet 1,56,000/ mm3.TotalWBC 41000/cu.mm(N42 L8 M3 E46 B1).So,absoluteeosinophil count was 19100/mm3. Chest X Ray was normal.Mantoux test was negative.Gastric lavage for CBNAAT wasnegative.He was further investigated to look for the cause of this severeeosinophilia. Stool for OPC was negative. Blood culture wasshowing no growth. In peripheral smear there was no blastcells. USG whole abdmonen was done, there was

A Baby with Multiorgan Eosinophilic Infiltration

Abhisek Naskar, Madhumita NandiDepartment of Pediatrics,NRS Medical College,Kolkata

Correspondance : Madhumita Nandi, Dept of Pediatrics,NRS MedicalCollege,Kolkata, E mail : [email protected]

hepatosplenomegaly. Colour doppler was showing noabnormality.Viral markers were negative.Total IgE level was1022.0IU/ml. Liver biopsy showed hepatocytes havingeosinophilic granular cytoplasm with eosinophilicmicroabscesses. Bone marrow biopsy showed myeloidhyperplasia with prominence of eosinophil,eosinophil myeloidprecursors 40%, 3% blasts and absence of parasites. CTscan of chest and echocardiography were within normal limits.Biopsy of lung or heart could not be done due to lack offacilities. So eosinophilic infiltration of liver and bone marrowcould be proved without any evidence of malignancy.After excluding secondary causes of eosinophilia, geneticstudies were undertaken as per recent guidelines todifferentiate between clonal and idiopathic eosinophilia todecide upon the management modalities (Figure 1 and 2).There was absence of mutation of platelet derived growthfactor receptor alpha/beta or fibroblast growth factor receptor1(PDGFR-A/FIP1L1).Thus,the diagnosis was IdiopathicHypereosinophilic Syndrome (HES).The child was given methylprednisolone pulse therapy(30mg/kg/day) for 3 days followed by oral prednisolone 2mg/kg/day. There was remarkable improvement. His fever subsidedand total WBC count came down to 11,000/cumm.(N51L45E3).Absolute eosinophilic count was now 330/mm3.On follow up at 6 months, there is satisfactory weight gain, noorganomegaly and is maintaining eosinophil count withnormal limits. He is continuing on a prednisolone dose ofaround 0.5 mg/kg/day because each time there was anattempt to reduce the dose of prednisolone further, his

AbstractA child with prolonged fever and hepatosplenomegaly was found to be having eosinophilic leucocytosis. After ruling out thesecondary causes by appropriate investigations,the cause of multiorgan eosinophilic infiltration was approached according tothe latest diagnostic algorithm proposed by WHO.He was ultimately diagnosed to be having Idiopathic HypereosinophilicSyndrome and managed according to latest recommendations.Key Words : Eosinophilia; hypereosinophilic syndrome

Gallery


eosinophil count seemed to go up again.DiscussionAccording to recent literature, when evaluating a patient witheosinophilia that is not thought to be secondary toinfections,allergy etc, 5 diagnostic possibilities should beconsidered: (1) myeloid or lymphoid neoplasms associated

with eosinophilia and PDGFR or FGR1 rearrangements,(2)clonal eosinophilia associated with an otherwiseWHO-definedmyeloid malignancy, (3)chronic eosinophilic leukemia, nototherwise specified;(4)lymphocytic variant hypereosinophilia,and (5) idiopathic eosinophilia including HES1(Figure 1). Thisrequires assessment of the peripheral blood smear, bonemarrow morphologic features, cytogenetic analysis, molecular

Fig 1.Diagnosis and treatment algorithm based on 2008 WHO classification of eosinophilic disorders


studies including screening for FIP1L1-PDGFRA, andperipheral blood lymphocyte phenotyping and T-cell receptorgene rearrangement studies. In this case, screening forFIP1L1-PDGFRA was negative, peripheral smear showed nosign of malignancy, no translocation was present in bonemarrow biopsy. So, it was idiopathic HypereosinophilicSyndrome (HES), a diagnosis by exclusion(Figure 2)In 1968 Hardy and Anderson coined the term“hypereosinophilic syndrome” to describe patients withprolonged eosinophilia ofunknown cause2. Chusid, et al in1975 used 3 diagnostic criteria for HES that are still utilizedtoday: (1) persistent eosinophilia of1.5x109/L (1500/mm3) forlonger than 6 months; (2) lack of evidence for parasitic, allergic,or other known causes of eosinophilia;and (3) signs andsymptoms of organ involvement3. In this case all three criteriawere matched.There were evidence of bone marrow and liverinfiltration of eosinophils.The diagnosis was thus confirmed.HES is predominantly a disease of men (male-female ratio,9:1)and is usually diagnosed between theages of 20 yearsand 50years4.The mean duration of disease was 4.8 years(range, 1-24years) Rare cases in infants and children havealso been described5-7. In one large series, eosinophilia was

discovered incidentally in 12% of patients4. The most commonpresenting signs and symptoms were weakness and fatigue(26%), cough (24%), dyspnea(16%), myalgias or angioedema(14%), rash or fever (12%), and rhinitis (10%)4.The systeminvolvement in HES include hematological;cardiovascularcausing cardiomyopathy,constrictivepericarditis ;neurologicalcausing thromboembolism,peripheralneuropathy;pulmonarycausing effusion, fibrosis4,8.In treatment, corticosteroids are indicated for organ diseaseand are useful for eliciting rapid reductions in the eosinophilcount9. Lack of steroid responsiveness warrants considerationof cytotoxic therapy. Hydroxyurea is an effective first-linechemotherapeutic for HES4,9; some benefit has also beenreported for second-line agents including vincristine,pulsedchlorambucil, cyclophosphamide, and etoposide 10,11.Interferon alpha can elicit long-term hematologic andcytogeneticresponses in HES.Imatinib is effective in FIP1L1-PDGFR rearrangement positive cases. A prior review of 57 HES cases included reports publishedbetween1919 and 1973.The median survival was 9 months,and the 3-year survival was only 12%9. These patientsgenerally had advanced disease, with congestive heart failure

Fig 2. Diagnostic algorithm for clonal or idiopathic eosinophilic. CEL-NOS = chronoc eosinophilic leukemia, not otherwise specified; FISH= fluorescence in situ hybridization; HES = hypereosinophilic syndrome; PDGFR = platelet-derived growth factor receptor; RT-PCR =reverse transcription polymerase chain reaction ; TCR = Tcell receptor; WHO = World health Organisation


accounting for 65% of the identified causes of death atautopsy3.ConclusionAccurate diagnosis is critical for effective management ofeosinophilia. Several mutations have recently been describedin myeloproliferative neoplasms, including those associated

with clonal eosinophilia,and this has greatly simplified ourcurrent diagnostic approach to these diseases.In particular,the discovery of FIP1L1- PDGFRA74 has opened our eyes tothe possibilities of not only deciphering the molecularpathogenesis of what we currently consider HES but also theprospect of effective molecular targeted therapy.

Reference1. Tefferi A, Patnaik MM, Pardanani A. Eosinophilia: secondary, clonal

and idiopathic. Br J Haematol. 2006;133(5):468-492.2. Hardy WR, Anderson RE. The hypereosinophilicsyndromes. Ann

Intern Med. 1968;68:1220-1229.3. Chusid MJ, Dale DC, West BC, Wolff SM. Thehypereosinophilic

syndrome. Analysis of fourteencases with review of the literature.Medicine.1975;54:1-27.

4. Fauci AS, Harley JB, Roberts WC, FerransVJ,Gralnick HR, BjornsonBH. NIH conference. Theidiopathic hypereosinophilic syndrome.Clinical,pathophysiologic, and therapeutic considerations.Ann InternMed.1982;97:78-92.

5. Alfaham MA, Ferguson SD, Sihra B, Davies J.The idiopathichypereosinophilic syndrome. Arch Dis Child. 1987;62:601-613.

6. Wynn SR, Sachs MI, Keating MU, et al. Idiopathichypereosinophilicsyndrome in a 5 1/2-month-oldinfant. J Pediatr. 1987;111:94-97.

7. Bakhshi S, Hamre M, Mohamed AM, Feldman G,Ravindranath Y.t(5;9)(q11;q34): a novel familial translocation involving Abelsononcogene andassociation with hypereosinophilia. J Pediatr HematolOncol. 2003;25:82-84.

8. Tai PC, Ackerman SJ, Spry CJ, Dunnette S, OlsenEG, Gleich GJ.Deposits of eosinophil granule proteins in cardiac tissues of patientswith eosinophilicendomyocardial disease. Lancet. 1987; 1:643-647.

9. Parrillo JE, Fauci AS, Wolff SM. Therapy of thehypereosinophilicsyndrome. Ann Intern Med. 1978;89:167-172.

10.Smit AJ, van Essen LH, de Vries EG. Successfullong-term control ofidiopathic hypereosinophilic syndrome with etoposide. Cancer.1991;67:2826-2827.

11.Bourrat E, Lebbe C, Calvo F. Etoposide for treating treatingthehypereosinophilic syndrome. Ann Intern Med. 1994;121:899-900.

Members are requested to submit News, Views, Reviews, Case Reports, Articles forThe “TheChild and Newborn”

Contact :Dr Jaydeep Choudhury

Editor-in-Chief, The Child and Newborn

“Oriental Apartments”, Flat H1, 15C, Canal Street, Kolkata 700 014Email : [email protected]

Request


Case report1 year 10 months old male child, born out of non-consanguinous marriage,presented to his local doctor withhistory of failure to thrive and delayed motor milestones notedsince age of 1 year.After evaluation,serum 25-hydroxy vitaminD level was found to be low (14ng/ml), X-ray of wrists showedrachitic changes,he was given a parenteral dose of Vitamin D(6 lakh IU), considering Vit D deficiency rickets. Three weekslater,X-ray wrists repeated but no signs of healing was seen. Arepeat dose of parenteral 25-hydroxy Vitamin D was given andreferred to our Institute as a case of refractory rickets. Therewas no history of jaundice, malabsorption, fracture or seizures.He was born at term weighing 2.4 kg, of an uneventfulpregnancy. Family history was not significant. Examinationrevealed severe retardation of both weight 7kg (Wt<3rd centile)and height 76 cm (Ht<3rd centile). He had somewhat roundeddoll-like facies, protuberant abdomen, features of rickets inform of frontal bossing, wrist widening, rachitic rosary andgenu valgum as shown in figure 1. Typical X-ry features ofwrists are shown in figure 2 and 3.Typical X-. Systemicexamination revealed non tender, firm hepatomegaly (liverspan 6cm) with 5cm firm splenomegaly.Investigations revealed compensated metabolic acidosis (pH-7.425, HCO3-18.6.1mmol/L, pCO2 29).Serumcreatinine(0.8mg/dl), serum calcium(9.5mg/dl), serum vitaminD(25-OH vit D) (34.2ng/ml) were normal.Serum 1,25Vit D wasraised(>200ng/ml),Low serum phosphorus (2.7 mg/dL; normal4-7) and elevated alkaline phosphatase (958 IU/L; normal 57-180) were found. Other investigations like serum uric acid,SGPT, PT, aPTT, Total serum bilirubin, Total protein, S.Albumin, S. Cholesterol were normal. Fasting hypoglycemia

Fanconi Bickel Syndrome : A Rare Entity

Kausambi Basu*, Arpan Saha*, Jayati Das*, Supratim Datta***Post graduate Trainee IPGME&R, **Professor and HOD, IPGME&R,SSKM Hospital,Kolkata

Correspondance: Suprat im Datta, Professor and HOD,IPGME&R,SSKM Hospital,Kolkata. Email : [email protected]

(blood sugar 28 mg/dL at 3 AM)was found. Critical samplecollected during hypoglycemia revealed normal blood lactate(5 mg/dL; normal 4.5-20), and serum ammonia (88 U/L;normal 10-90).Urinalysis revealedUrine albumin +,Urineglucose +++,Urine for phosphates +,Urine pH: 5.4, 24 hrs.Urinary Calcium 300 mg (WNL).Biopsy from liver revealedglycogen –laden hepatocytes.On reviewing history andinvestigations, the patient had features of r ickets,hepatosplenomegaly, Fanconi’ssyndrome and a glycogenstorage in liver. It was labelled as, Fanconi Bickel Syndromeor Glycogen Storage Disease type XI.Child was advised frequent feeds with complex carbohydratediet (uncooked corn starch), oral sodium bicarbonate (5 mEq/kg/d), potassium (2mEq/kg/day) and phosphate (40 mg/kg/d)supplement. On follow-up 2 months later, at 2 y age, he hadgained 2.0 kg in weight (9 kg). Liver span was 6 cm and featuresof rickets were present. The blood biochemistry was withinthe normal range.DiscussionThe gene GLUT-2 encodes glucose transporter protein 2expressed in hepatocytes, pancreatic beta cells, enterocytesand renal tubular cells. More than 34 different mutations arereported, which provides molecular basis of the disease2.Since the first description in 1949, more than 150 cases ofFBS have been reported, with three from India 3-5.Accumulation of glycogen in renal tubular cells causesproximal tubular dysfunction leading to Fanconi nephropathywith variable renal phosphate wasting 6. Presence ofhypercalciuria is rarely reported. Although it is postulated tobe due to phosphaturia induced down- regulation of renaltubular 1 alpha-hydroxylase activity, the etiology ofhypercalciuria remains uncertain7.FBS can present as galactosemia in neonatal screening as

Gallery

Abstrace :Fanconi-Bickel Syndrome,a glycogen storage disorder, is a rare disease which was referred to us as a case of resistant rickets


the same transporter is required for galactose8. Other atypicalpresentations like pseudotumorcerebri, intestinalmalabsorption and liver failure have been reported.No specific treatment has been identified. Small frequent feedsand uncooked corn starch at bedtime should be offered toprevent hypoglycemia. Since glucose wasting is very high,adequate calorie supplementation is very essential for normalgrowth. Long term replacement therapy for proximal tubular

losses is needed.Overall prognosis is good6. Hepatomegaly tends to regressafter puberty. Renal phosphate wasting and hypercalciuriacontribute to difficult to correct bone features and short adultstature. Mutation studies help not only in diagnosis but also toenable counseling. Genetic counselling and prenatal testingis recommended being an autosomal recessive disorder.

Reference1. Santer R, Steinmann B, Schaub J. Fanconi– Bickel syndrome- a

congenital defect of facilitative glucose transport. CurrMol Med.2002;2:213-27.

2. Santer R, Groth S, Kinner M, Dombrowski A, Berry GT, Brodehl J, etal. The mutation spectrum of the facilitative glucose transporter geneSLC2A2 (GLUT2) in patients with Fanconi-Bickel syndrome. HumanGenet. 2002;110:21-9

3. Gopalakrishnan A, Kumar M, Krishnamurthy S, Sakamoto O,Srinivasan S. Fanconi–Bickel syndrome in a 3-year-old Indian boywith a novel mutation in the GLUT2 gene. Clin Exp Nephrol.2011;15:745-8.

4. Mohandas Nair K, Sakamoto O, Jagadeesh S, Nampoothiri S.Fanconi–Bickel syndrome. Indian J Pediatr. 2012; 79:112-4.

5. Karande S, Kumbhare N, Kulkarni M. Fanconi Bickel syndrome.Indian Pediatr. 2007;44:223-5.

6. Santer R, Schneppenheim R, Suter D, Schaub J, Steinmann B.

Fanconi-Bickel syndrome - the original patient and his natural history,historical steps leading to the primary defect, and a review of theliterature. Eur J Pediatr. 1998;157:783-97.

7. Mannstadt M, Magen D, Segawa H, Stanley T, Sharma A, Sasaki S,et al. Fanconi-Bickel syndrome and autosomal recessive proximaltubulopathy with hypercalciuria (ARPTH) are allelic variants causedby GLUT2 mutations. J Clin Endocrinol Metab. 2012;97:E1978-86.

8. Müller D, Santer R, Krawinkel M, Christiansen B, Schaub J. Fanconi-Bickel syndrome presenting in neonatal screening for galactosaemia.J Inherit Metab Dis. 1997; 20:607-8.

9. Setoodeh A, Rabbani A. Transient neonatal diabetes as a presentationof Fanconi- Bickel Syndrome. Acta Med Iran. 2012;50:836-8.

10. Hamilton AJ, Bingham C, McDonald TJ, Cook PR, Caswell RC,Weedon MN, et al. The HNF4A R76 W mutation causes atypicaldominant Fanconi syndrome in addition to a a-cell phenotype. J MedGenet. 2014;51: 165.

Fig 1. Physical features in the child with Fanconi BickelSyndrome.

Fig 2. Xray Wrist

Fig 3. Xray Wrist


IntroductionRecurrent pneumonia (RP) is defined as two or more episodesof pneumonia in 12 months or three episodes altogether withradiographic clearance in between.It should be differentiated from persistent pneumonia, whichis defined as persistence of symptoms and radiologicalchanges for 6 weeks or more despite treatment. We report apatient who presented with recurrent pneumonia and wasfound to have a primary immunodeficiency due to IgAdeficiency. Selective IgA deficiency is the most commonimmunodeficiency disorder. Most affected children areasymtomatic. However, in rare patients, the deficiency leadsto an increased incidence of infection, particularlysinopulmonary infection.Case reportA 3 month, male baby was admitted in pediatric ward withrespiratory distress, fever for 5 days following cough and cold.On examination signs of severe respiratory distress waspresent. SpO2 was 83% in room air. On auscultation revealeddecreased air entry along with wheeze on right upper zoneand ejection systolic murmur of grade 3 in MLSB and ULSB.Initially he was managed with moist oxygen, intravenous fluidand nebulisation with 3%NaCl and salbutamol, but he wasdeteriorated, he was shifted to PICU, put on non invasiveventilation and received antibiotic. He was found to have rightupper lobe pneumonia on the initial chest radiograph. Thepatient had a history of two episodes of pneumonia in twodifferent lobes of lung within last one and half months forwhich he was hospitalized, but in both occasion he achievedradiological clearance after full course of antibiotic treatment.There was no significant family history. His anthropometry

Recurrent Pneumonia Due to Selective IgA Deficiency in a 3 Months Old Child

Aparajita Bera*, Kalimuddin Khan*, Suman Sarkar**, Kallol Das****Post Graduate Trainee, **Associate Professor, ***RMO Cum Clinical Tutor

Department of Pediatrics, Chittaranjan Seva Sadan College of Obstetrics Gynaecology & Child Health

Correspondance : Aparajita Bera, Post Graduate Trainee, Department ofPediatrics, Chittaranjan Seva Sadan College of Obstetrics Gynaecology &Child Health. Email : [email protected]

showed severe wasting. The patient’s white blood cell countwas 19600(N-72%,L-16%,) peripheral smear showednormocytic normochromic, no toxic granules, CRP was 56.2.Echo revealed tiny PDA, tiny PFO, no chamber enlargement.Mantoux test was negative, gastric lavage for AFB andCBNAAT was negative, blood c/s showed no growth, HIV-1and2 of parents was non-reactive, urine r/e, m/e, c/s wasnormal. HRCT was done, it revealed collapse consolidationin right upper lobe with focal consolidation in inferior lingual,apical segment of left lower lobe. BRONCHOSCOPY wasdone and it was normal. Broncho-alveolar lavage cultureshowed growth of Staphylococcus aureus after 48hrs whichwas sensitive to linezolid and antibiotic was upgraded toLinezolid. After stabilization sweat chloride and stool forelastase were sent and they were negative. His immunologicalprofile done, showed IgA and IgG deficiency and tissuetransglutaminase (TTG) antibody was negative. IgGsubclasses, flow cytometry and serum complement reportswere suggestibe of selective IgA deficiency. So it is a case ofrecurrent pneumonia due to IgA deficiency.DiscussionSelective IgA deficiency is one of the most common primaryimmunodeficiency diseases. Studies have indicated that asmany as one in every 500 Caucasian people has selective IgAdeficiency. The basic defect is unknown. Phenotypicallynormal blood B cells are present. Some patients with IgAdeficiency are more susceptible to infection than others, butthis is not the case for all patients with IgA deficiency whodevelop complications or for those who have low IgG2 and orIgG4 in addition to absent IgA. A common problem in selectiveIgA deficiency is susceptibility to infections, like recurrent earinfections, sinusitis, bronchitis and pneumonia. Some patientsalso have gastrointestinal infections and chronic diarrhea.The occurrence of these kinds of infections is easy tounderstand since IgA protects mucosal surfaces. A second

The Greek term Probiotic means ‘for life’. According to WHO,Probiotics are ‘live microorganism which, when administered inadequate amounts, confer a health benefit on the host’ 1 . Probiotics can be consumed as medicinal supplement or modified food.

Gallery


major problem in IgA deficiency is the occurrence ofautoimmune diseases. Allergies may also be more commonamong individuals with selective IgA deficiency than amongthe general population. Patients with IgA deficiency often haveIgG antibodies against cow’s milk and ruminant serumproteins. These antiruminant antibodies may cause falsepositive results in immunoassays for IgA that use goat antisera.

Serum antibodies to IgA are reported in as many as 44% ofpatients with selective IgA deficiency. If these antibodies are ofthe IgE isotype, they can cause severe or fatal anaphylacticreactions after intravenous administration of blood productscontaining IgA. Administration of IVIG which is >99% IgG, isnot indicated because most IgA deficient patients make IgGantibodies normally.

Bibliograpgy1. Bonilla FA, Bernstein IL, Khan DA, et al. Practice parameter for the

diagnosis and management of primary immunodeficiency. Ann AllergyAsthma Immunol. 2005;94:S1-S63.

2. Boyle JM, Buckley RH. Population prevalence of diagnosed primaryimmunodeficiency diseases in the United States. J Clin Immunol.2007;27:497-502

3. Ammann AJ, Hong R. Selective IgA deficiency and autoimmunity. ClinExp Immunol. 1970 Dec;7(6):833–838. [PMC freearticle] [PubMed]

4. Bachmann R. Studies on the serum gamma-A-globulin level. 3. Thefrequency of A-gamma-A-globulinemia. Scand J Clin Lab Invest.1965;17(4):316–320.[PubMed]

25 January : Chittaranjan Sishu Sadan22 February : Apollo Gleneagles Hospital29 March : Institute of Child Health, Kolkata26 April : Ramkrishna Mission Seva Prathisthan31 May : B C Roy Hospital for Children28 June : Calcutta National Medical College26 July : R G Kar Medical College30 August : NRS Medical College27 September : Medical College25 October : B R Singh Hospital29 November : IPGME&R20 December : Calcutta Medical Research Institute (CMRI)

Monthly Clinical Meeting of WBAP 2018

PG Class at WBAP Office

All the postgraduate students are requested toattend monthly PG class by eminent teachers atWBAp Office.

Contact : Mrs Bela Bhattacharjee (9830866712)


IntroductionViral hepatitis continues to be a major health problem in bothdeveloping and developed countries1. This disorder is causedby the pathogenic hepatotropic viruses recognized to date:hepatitides A (HAV), B (HBV), C (HCV), D (HDV), and E (HEV)viruses. In most pediatric patients, the acute phase causes noor mild clinical disease1. Wilson disease (hepatolenticulardegeneration) is an autosomal recessive disorder that can beassociated with degenerative changes in the brain, liverdisease, and Kayser-Fleischer (KF) rings in the cornea2. Itoccurs due to absence or malfunction of ATP7B which resultsin decreased biliary copper excretion and diffuseaccumulation of copper in the cytosol of hepatocytes2.Hemoglobin E-ß thalassemia is the genotype responsible forapproximately one-half of all severe beta-thalassemiaworldwide3. The disorder is characterized by marked clinicalvariability, ranging from mild asymptomatic anemia to a life-threatening disorder requiring transfusion from infancy.Case reportA 7 years old male patient was admitted with complains ofhigh grade fever and yellowish discolouration of urine andsclera for 5 days and history of similar illness in the community.No history of altered sensorium, bleeding manifestations, bloodtransfusion and no positive family history was there anduneventful neonatal period. Clinical examination revealedmoderate pallor, deep jaundice and hepatosplenomegaly with

Jaundice Due to Acute Hepatitis a with Underlying E-beta Thalassemia andWilson Disease

Srikanta Baske*, Suman Sarkar**, Sayan Chatterjee****Post Graduate Trainee, **Associate Professor, ***Assistant Professor

Department of Pediatrics, Chittaranjan Seva Sadan College of Obstetrics Gynaecology & Child Health

Correspondance : Srikanta Baske, Post Graduate Trainee, Department ofPediatrics, Chittaranjan Seva Sadan College of Obstetrics Gynaecology &Child Health. Email : [email protected].

Gallery

AbstractA 7 year old boy presented with jaundice. On evaluation, he had deep jaundice, moderate pallor, depressed nasal bridge,hepatosplenomegaly. Initially the patient was diagnosed as hepatitis A with E-beta thallasemia (EßT). After few months boy wasreadmitted for persistence of jaundice. Re-evaluation revealed low ceruloplasmin level, raised 24hrs urinary copper level. Liverbiopsy later diagnosed it as Wilson disease (WD).Keywords : Viral hepatitis, E-ß thalassemia, Wilson‘s disease

depressed nasal bridge. Routine investigations revealedmoderate anemia, highly raised liver enzymes, unconjugatedhyperbilirubinemia with altered albumin globulin ratio withnormal gamma-glutamyl transferase (GGT) level. Feverworkups were done but no significant positive findings werenoted. Coagulation profile was within normal limit. Viralserologies were sent which revealed hepatitis A infection.Routine workups for anemia were done and reports revealednegative direct coomb test (DCT), raised serum ferritin leveland peripheral blood smear (PBS) revealed moderateanisocytosis, poikilocytosis with marked hypochromia,normoblast. Serial complete blood count (CBC) reportsshowed persistence of anemia (Table 2), so we had sent highperformance liquid chromatography (HPLC) investigations ofthe patient and his parents also of his brother. Serial liverfunction tests (LFT) (Table-1) were done which revealedgradual decrease in total serum bilirubin (TSB) and enzymelevels. Patient was discharged with the diagnosis of hepatitisA infection was advised to follow up in the outpatientdepartment (OPD) with report of HPLC. Patient was readmittedafter 4 wks with complain of fever, pain abdomen withdecreased appetite and persistence of jaundice (Table 3).He was reassessed for the above complains and this timeinvestigations were sent to rule out autoimmune hepatitis andWilson disease. In the meantime HPLC reports came and itrevealed patient is having E-ß thalassemia, brother is ßthalassemia major, father is ß thalassemia carrier, and motheris E-ß thalassemia. Markers for autoimmune hepatitis werenegative. Serum ceruloplasmin level was low. 24hrs urinary


copper level after penicillamine challenge was high. Initiallywe had done genetic study for Wilson disease and it was

negative. Later we did liver biopsy of this patient and it wassuggestive of Wilson disease. Oral zinc was given to the patient.

Flow chart 1. Approach to diagnosis of Wilson disease (WD) in a patient with unexplained liver disease [5]( CPN- Ceruloplasmin, Cu- Copper )

Table 1Date TSB Direct Indirect AST ALT ALP Total protein Albumin

(mg\dl) bilirubin (mg\dl) bilirubin (mg\dl) (IU/L) (IU/L) (IU/L) (gm/dl) (gm/dl)12/11/17 31 5.6 25.4 620 1888 239 8 3.417/11/17 24.6 6.1 18.5 446 1560 230 8 3.622/11/17 17.7 3.9 13.8 236 568 186 8.1 3.9

( AST- Aspertate aminotransferase, ALT- Alanine aminotransferase, ALP- Alkaline phosphatase )Table 2

Date Hb (gm/dl) PCV (%) RBC (million/cumm) RDW (%)12/11/17 8.2 22 4.1 2218/11/17 8.4 24 4.2 21

(Hb- Haemoglobin, PCV- Packed cell volume, RBC- Red blood cells, RDW- Red cell distribution width )Table 3

Date TSB Direct Indirect AST ALT ALK Total protein Albumin(mg\dl) bilirubin (mg\dl) bilirubin (mg\dl) (IU/L) (IU/L) (IU/L) (gm/dl) (gm/dl)

24/12/17 5.0 1.8 3.2 214 130 230 8 3.930/12/17 4.0 1.2 2.8 180 115 186 8.1 4.0


DiscussionThe approach to unexplained liver disease is shown inflowchart 1.Wilson disease (WD) is progressive and potentiallyfatal if untreated; specific effective treatment is available.Owing to the insidious course and variable presentation.Wilson disease is often diagnosed months to years after theinitial symptoms. Rapid diagnostic investigation of thepossibility of Wilson disease in a patient presenting with anyform of liver disease, particularly if older than 5 year of age notonly facilitates early institution of management of Wilsondisease and related genetic counselling but also allowsappropriate treatment of non-Wilsonian liver disease oncecopper toxicosis is ruled out. On the other hand due to diversephenotypic variability of EßT and paucity of long term clinicaldata, most important challenge in recent time is in providingdefinitive recommendations for the optimal management of

patients. The remarkable variation, and the instability, of theclinical phenotype of EßT suggests that careful tailoring oftreatment is required for each patient, and therapeuticapproach should be reassessed over time3. The highestfrequencies are observed in India, Bangladesh and throughoutSouth Asia. The coocccurance of EßT and WD is very rare.There are few case reports of cooccurance of this twodiseases. One case report was by Thapa R, et al on 2008 of a11 yr girl with concomitant EßT and WD4. In our case thepatient always had subclinical jaundice which flared up afterhepatitis A infection. We initially diagnosed it as hepatitis A butas there was altered albumin globulin ratio, altered AST ALTratio in later stage with persistence of jaundice we looked forany underlying chronic liver disease. Both the conditions areassociated with variable degrees of haemolysis, WD in thesetting of E-ß thalassemia may remain masked andconsequently remain undiagnosed for a long duration.

Reference1. Kyle Jensen M, Balistreri WF. Viral Hepatitis. In: Richard E. Behrman.

Nelson textbook of pediatrics. New Delhi: Elsevier; 2016. P.1942-53.2. Menchise AN, Balistreri WF. Wilson Disease. In: Richard E. Behrman.

Nelson textbook of pediatrics. New Delhi: Elsevier; 2016. P.1939-40.

3. Olivieri NF, Pakbaz Z, Vichinsky E. Hb E/beta-thalassaemia: a common& clinically diverse disorder. Indian J Med Res.2011 Oct; 134(4);522-531.

4. Thapa R. et al. Acute Wilson disease associated with E beta-thalassemia. J Pediatr Hematol Oncol. 2008 Dec:30(12):925-7

5. Roberts EA, Schilsky ML. Hepatology. 2008; 47(8):2091.

WBAP office has 3 Air conditioned guest rooms for stay

Double Bed (LCD TV) available.

Guest Room 1 : Rs.1000/- per nightGuest Room 2 : Rs.1500/- per nightGuet Room 3 : Rs.1200/- per night

Food available on request.

Contact : Smt. Bela Bhattacharya (9830866712)

Announcement


A 15 year Old Girl Presenting with Right Heart Failure Two Years after Road Traffic Accident : Delayed Presentation of Tricuspid Valve Injury

Aniruddha Ghosh*, Mallar Mukherjee**, Jaydeep Choudhury***, Arunaloke Bhattacharya***,Maya Mukhopadhyay****

*Senior Resident, **Assistant Professor, ***Associate Professor, ****Additonal Director, Institute of Child Health, Kolkata

Correspondance : Aniruddha Ghosh. Senior Resident, Institute ofChild Health, Kolkata. Email : [email protected].

AbstractBlunt trauma to thorax is common but tricuspid valve rupture as a result of blunt chest trauma is exceptional. When this occurs,it can be clinically very subtle leading to a very late presentation. Here, we report the case of a 15-year-old patient who sustainedblunt trauma to her chest wall during a two-wheeler accident. She presented 1 year later with symptoms of right-heart failure,secondary to ruptured anterior tricuspid leaflet and resultant tricuspid regurgitation. Transthoracic echocardiography clinchedthe diagnosis. This case highlights the importance of early diagnosis and management of valvular injury due to blunt trauma tothoracic cage. As pathological process giving rise to tricuspid valve rupture may evolve over a long period of time and clinicaldiagnosis is often tricky, we suggest echocardiography to be included routinely in the blunt chest trauma management protocolseven in case of hemodynamically stable patients and preferably repeated during the follow up.Keywords : Tricuspid regurgitation, chest injury, tricuspid valve injury, echocardiography.

IntroductionCardiac valvular injuries are rare consequence of bluntthoracic trauma1. Incidence of severe tricuspid regurgitationdue to tricuspid valve injury following road traffic accident isbeing reported in increasing frequency all over the world maybe due to increase in the number of high speed automobileaccidents and advancement in cardiac imaging studies2.Often the condition presents late1 and is associated withsignificant long term morbidity.Case history A 15-year-old girl presented with respiratory distress, graduallyprogressive swelling of abdomen and both the legs for last 10days and puffiness of face for last 5 days. There was associatednon productive cough, profuse sweating, exertionalexacerbetaions and orthopnoea. There was no history of anyfever, joint pain, rash, chest pain, headache or syncope. Shefaced a two-wheeler accident two years back when shesustained trauma to left arm and chest but didn’t need anyhospitalisation. Family history, perinatal and developmentalhistories didn’t reveal any abnormality. Clinical presentationof the patient was consistent with class III New York HeartAssociation (NYHA) cardiac dysfunction.

On general examination, higher functions were normal, shewas visibly in distress which was relieved in sitting posture;heart rate was 128/min with regular rhythm, peripheral pulseswere feeble; respiratory rate was 48/min, blood pressure: 78/40 mm of Hg; oxygen saturation was 92% in room air; bilateralpitting pedal edema was there but cyanosis was absent.Cardiovascular examination revealed visible pulsation andpalpable thrill over left lower sternal border (LLSB); cardiacapex beat was located in left 5th intercostal space 1 cm outsidemid clavicular line, 1st and 2nd heart sounds were of normalintensity, 2nd heart sound had a wide and fixed split. Apansystolic murmur with grade 4 intensity was heard all overthe precordium but was loudest in LLSB without any definiteradiation. There was no change in character of murmur withrespirational or postural manoeuvers.Chest wall did not show any deformity or scar, bilateral basalcrackles were heard on auscultation of chest. Abdomen wassoft, swollen, nontender. Lower margin of liver was palpable 4cm below right costal margin along the mid clavicular line.Ascites was present with shifting dullness but fluid thrill wasabsent. Span of outstretched arms were lesser than the height.Also upper to lower segment ratio of the body was withinnormal range.Routine hematological parameters were within normal ranges.Liver and renal function tests, electrolytes, C-reactive protein

Gallery


were within normal limits. Urine test was negative for proteinand red blood cells.Chest X-ray posteroanterior view revealed cardiomegaly withbilateral “bat-wing hilar shadows” suggestive of pulmonaryedema. Transthoracic Echocardiography with Doppler studyrevealed severe tricuspid regurgitation (TR), dilated rightatrium (RA) and right ventricle (RV) with RV dysfunction, rightventricular systolic pressure (RVSP) 40 mm Hg and moderatepulmonary arterial hypertension (PAH). Most importantly, a tearin the anterior tricuspid leaflet (ATL) [ Figure 1] and restrictedmovement of the septal tricuspid leaflet (STL) of the tricuspidvalve was seen through which blood was regurgitating intoright atrium [Figure 2]. An incidental 10 mm ostium secundum

type of Atrial Septal Defect was also noted.After initial resuscitation, patient was maintained on oralfrusemide, spironolactone and enalapril. Gradually featuresof heart failure subsided and the patient resumed routine dailyactivities but exertional breathlessness still persisted. She wasdischarged from our hospital and was referred for corrective/replacement surgery to a tertiary care cardiosurgery institute.DiscussionBlunt cardiac injuries are seen only in 1.25% patients of motorvehicle accidents. However, this is thought to be anunderestimate as it does not include chronic conditions arisingfrom missed diagnosis of latent blunt cardiac injuries whichlead to occult valvular tear and heart failure3. Tricuspidregurgitation due to valvular rupture following blunt cardiacinjury is uncommon and sinister in presentation.The mechanism of tricuspid valve injury is best explained bygeneration of tractional pull due to anteroposteriorcompression of chest and sudden elevation in the rightventricular end diastolic pressure2,4. Presentation can be acuteif there is, as most commonly found during surgery, subvalvularrupture of the anterior papillary muscle5. Alternatively, delayedpresentation with congestive right heart failure may occur, asin our patient, as a result of papillary muscle contusion, withhemorrhage, inflammation, late necrosis resulting into valvulardisruption over time5,6. In a case series of 13 patients of bluntraumatic tricuspid insufficiency reported by van Son, et al7 themedian duration between trauma and surgery was 17 years(range: 1 month-37 years).Presently there is no consensus regarding routine use ofechocardiography in cases of blunt thoracic trauma especiallythe clinically “non-severe” ones. In a 2006 review, Kulik, et al 6suggested that patients who are hemodynamically unstableshould undergo a routine transesophageal echocardiography(TEE) and those without hemodynamic compromise shouldundergo transthoracic echocardiography (TTE) and serialassays of cardiac enzymes. If there is coexisting thoracicinjuries then transesophageal echocardiography is theoption4.Traumatic tricuspid regurgitation is amenable to surgicalrepair. Delay in diagnosis affects the success rate andoutcome of surgery5. Traditional indication for surgery issymptomatic cardiac failure. But operation should beperformed before development of right ventricular myocardialdysfunction. When tricuspid regurgitation is associated withcontusion induced pulmonary hypertension, medicaltreatment to eliminate pulmonary hypertension is required first

Fig 1. 2D echocardiography (apical 4 chamber view) showingtear (marked with red arrow) in anterior tricuspid leaflet

Fig 2. Colour doppler image of apical 4 chamber view showingflorid tricuspid regurgitation


for a successful and durable surgical repair7, 8, 9.This case highlights the importance of echocardiography asa primary screening cum follow up evaluation tool in cases of

blunt chest trauma. The earlier diagnosis and operative repairof a tricuspid valvular injury can prevent right ventriculardysfunction and increase the success of operative outcome.

Reference1. Schuster I, Graf S, Klaar U, Seitelberger R, Mundigler G, Binder T.

Heterogeneity of traumatic injury of the tricuspid valve: A report of fourcases. Wien Klin Wochenschr 2008;120: 499-503.

2. Lin SJ, Chen CW, Chou CJ, Liu KT, Su HM, Lin TH, Voon WC, LaiWT, Sheu SH. Traumatic tricuspid insufficiency with chordae tendinaerupture: a case report and literature review. Kaohsiung J Med Sci2006; 22: 626-9.

3. Ismailov RM, Weiss HB, Ness RB, Lawrence BA, Miller TR. Bluntcardiac injury associated with cardiac valve insufficiency: traumalinks to chronic disease? Injury 2005;36(9):1022- 8.

4. Khurana S, Puri R, Wong D, Dundon BK, Brown MA, Worthley MI,Worthley SG. Latent tricuspid valve rupture after motor vehicleaccident and routine echocardiography in all chest-wall traumas. TexHeart Inst J 2009;36: 615-7.

5. Doi A, Takahara Y, Mogi K, Hatakeyama M. Repair of traumatictricuspid regurgitation by bicuspidization. Gen Thorac CardiovascSurg 2007;55(12):499-501.

6. Kulik A, Al-Saigh M, Yelle JD, Rubens FD. Subacute tricuspid valverupture after traumatic cardiac and pulmonary contusions. Ann ThoracSurg 2006;81(3):1111-2.

7. van Son JA, Danielson GK, Schaff HV, Miller FA Jr. Traumatic tricuspidvalve insufficiency. Experience in thirteen patients. J ThoracCardiovasc Surg 1994;108(5):893-8.

8. Maisano F, Lorusso R, Sandrelli L, Torracca L, Coletti G, La CannaG, Alfieri O. Valve repair for traumatic tricuspid regurgitation. Eur JCardiothorac Surg 1996;10:867-73.

9. Bortolotti U, Scioti G, Milano A, Guglielmi C, Benedetti M, Tartarini G,Balbarini A. Post-traumatic tricuspid valve insufficiency. 2 cases ofdelayed clinical manifestation. Tex Heart Inst J 1997;24:223-5.


Breaking News

Jaydeep ChoudhuryInstitute of Child Health, Kolkata

Correspondance : Jaydeep Choudhury. Institute of Child Health,Kolkata. Email : [email protected]

The practice of medicine is not just a science and itrequires art and tact is most evident when a doctor hasto break news. The basic concept that a doctor has torealize is that a bad news cannot be converted to goodnews. Thus good communication is the key and thefoundation is empathy.Breaking news is not like giving an update, one must befocused and understand the situation from “inside” andthe approach should be caring. It is a complexcommunication task – requires expert verbal and non-verbal skills. Poor delivery can create seriousmiscommunications. The way a doctor or other health orsocial care professional delivers bad news places anindelible mark on the doctor/professional-patientrelationship.The person who delivers news may have to cope withsome difficulties. Like the savior complex, where aphysician feels that he is a savior and should alwayscome out with positive results. Perspective of disabilityand limitations of the physician is another issue. Onehas to realize that certain situations are beyond control.Then there may be discomfort with negative emotions.What is bad news?It may be “any information, which adversely and seriouslyaffects an individuals view of his or her future” or, insituations where there is either a feeling of no hope, athreat to a person’s mental or physical well-being, risk ofupsetting an established lifestyle, or where a messageis given which conveys to an individual fewer choices inhis or her life’1.The common denominator is that bad news is a message,which has the potential to shatter hopes and dreamsleading to very different lifestyle and future.

Withholding bad news from patients was commonlypracticed in the past2. In the present times this has beenreplaced by one that emphasizes patient autonomy andfull disclosure. Honest disclosure of diagnoses,prognoses, and treatment options allows patients to makeinformed healthcare decisions that are consistent withtheir goals and values3,4.Conveying information to patients and their familyOne should not break news in a hurry. The following isthe Girgis and Sanson-Fisher guidelines on conveyinginformation to patients about serious disease or death5.1. Ensuring privacy,2. Allowing adequate time,3. Assessing patients’ understanding,4. Giving information about diagnosis and prognosis

simply and honestly, avoiding euphemisms,5. Encouraging patients to express feelings,6. Being empathic,7. Giving a broad but realistic time-frame concerning

prognosis,8. Arranging a review.The bottomline is that parents want their doctor to behonest, compassionate, caring, hopeful and informative.Six step protocol for breaking newsRobert Buckman has outlined a six step protocol forbreaking bad news (6). The steps are:1. Getting started:The meeting should be held in a private setting with bothphysician and parent comfortably seated. It is helpful tostart with some questions to indicate to the parent thatthis conversation will be a two-way affair.

Medicolegal Tip


2. Finding out how much the patient knows:By asking a question such as, “What have you alreadybeen told about the illness?” one can begin to understandwhat the parent has already been told, or how much theparent understood about what’s been said, the parentslevel of knowledge and emotional state.3. Finding out how much the patient wants to know:It is useful to ask parents what level of detail they wish tocover Parents want additional information regarding thediagnosis, chances of cure, the side effects of therapyand a realistic estimate of recovery.4. Sharing the information:Decide on the agenda before sitting down with theparents, so that one has the relevant information at hand.The topics to consider in planning an agenda are:diagnosis, treatment, prognosis, and support or coping.Long lectures are overwhelming and confusing. Medicalterms should be translated into layman’s term.5. Responding to the patients feelings:If the parent’s reaction is not understood, it will leave alot of unfinished business, and one will miss anopportunity to be a caring physician. Learning to identifyand acknowledge a parent’s reaction is something thatdefinitely improves with experience.6. Planning and follow-through:At this point one needs to synthesize the parent’sconcerns and the medical issues into a concrete planthat can be carried out in the parent’s system of healthcare. Outline a step-by-step plan, explain it to the parent,and contract about the next step. Be explicit about yournext contact with the parent.Providing a planPlanning and follow-through is very important. The doctorshould synthesize the parent’s concerns and the medicalissues into a concrete plan that can be carried out.Patients who have a clear plan for the future are lesslikely to feel anxious and uncertain. It may be helpful ifthe patient has the option to speak to the professionaldelivering the bad news at a later stage.Some phrases and questions that help in goodconversation are:(i) “I wish I had better news” (as opposed to “I’m sorry,

I have bad news”),

(ii) “I admire your courage,”(iii) “I will be here for you,”(iv) “What gives you hope and strength?”Some statements which may be avoided include thefollowing:(i) “It could be worse,”(ii) “We all die,”(iii) “I understand how you feel,”(iv) “Nothing more can be done.”Things go wrongThings go wrong when we try to escape, we react inanger or we dilute the situation.We try to escape:We try to escape by inappropriate delegation. The seniormost member of the unit should break the news.Distraction, intellectualization, minimization and emptyreassurance should be avoided.React in anger:Denial, idealization, some “unreasonable” demands,anger, frustration and blame may be natural reactions ofthe parents. A physician should not react in anger undersuch circumstances.Dilute the agenda:It is not wise to dilute the agenda. It is improper to discussabout billing, practical arrangements or try to distract withtrivial comments. After all a bad news is a bad news andit is a serious matter to the parents.What is the importance of documentation?It is important that accurate records are maintained of theconversation and the information and details exchanged.These will assist in the future care of the patient andenhance communication within the multidisciplinary team.The whole interaction should be carried out in a placewhich is covered under closed circuit TV and therecordings preserved for medicolegal purposes.What if the parent starts to cry while breaking news?It is always better to wait for the person to recover andstop crying. If it seems appropriate, one canacknowledge and suggest a temporary break till theparent recovers. The doctor must not try to act as if tearsare an emergency that must be stopped. One shouldshow the willingness to deal with anything that comesup7,8.


Reference1. Breaking Bad News ...Regional Guidelines. Department of Health,

Social Services & Public Safety. Developed from Partnerships inCaring (2000) DHSSPS, 2003. URL: http://www.dhsspsni.gov.uk/breaking_bad_news.pdf. Accessed on 29 December 2010.

2. Vandekieft G K. Breaking Bad News. Am Family Phys 2001; 64:12. URL: www.aafp.org/afp. Accessed on 29 December 2010.

3. Taylor SE. Health Psychology, 1995. 3rd Ed. New York. NY:McGraw-Hill Book Company.

4. Buckman R. Breaking Bad News: A Guide for Health CareProfessionals 1992. Baltimore: Johns Hopkins University Press.

5. Girgis A, Sanson-Fisher R W. Breaking bad news: Consensusguidelines for medical practitioners. J Clin Oncol 1995; 13: 2449-56.

What is the impact on a health care professional?Breaking bad news can be extremely stressful for thedoctor or professionals involved. The evidence suggeststhat the bearer of bad news experiences strong emotionssuch as anxiety, a burden of responsibility for the news

and fear of a negative response. This stress can resultin a reluctance to deliver bad news9,10. One of the mostimportant jobs of a physician is to be a clearcommunicator and one of the toughest challenges for acommunicator is to deliver bad news.

6. Breaking Bad News. University of Washington School of Medicine.Ethics in Medicine. URL: http://depts.washington.edu/bioethx/topics/badnws.html. Accessed on 29 December 2010.

7. Miyaji N. The Power of Compassion: Truth Telling Among AmericanDoctors in the Care of Dying Patients. Soc Sci Med 1993; 36:249-64.

8. Siminoff AL, Fetting JH, Abeloff MD. Doctor-Patient Communicationabout Breast Cancer Adjuvant Therapy. J Clin Oncol 1989; 7:1192-200.

9. Maguire P. Barriers of Psychological Care to the Dying. BritishMedical Journal 1985; 291:1711-3.

10. Ptacek JT, Eberhardt TL. Breaking Bad News. A Review ofLiterature. JAMA 1996; 276: 496-502.
