OSCEOSCE44thth March-08 March-08

Center For Child HealthCenter For Child HealthSir Ganga Ram HospitalSir Ganga Ram Hospital

• Spot the diagnosis with type• Views of picture A and B and what does

the MRI depicts• Early important clinical clue for

hydrocephalus after 1st month of life

Spot 1Spot 110 yr old male

child presented

with multiple

cranial nerve

palsies of 2 wks

duration. MRI

done is shown

here

• Severe communicating hydrocephaluscommunicating hydrocephalus

• Contrast-enhanced axial (A) and coronal (B) T1- weighted MR images depict diffuse leptomeningeal enhancementdiffuse leptomeningeal enhancement in the basal cistern. Strong enhancement of the bilateral thickened third nerves (arrowheads in A)

• Open squamo parietal sutureOpen squamo parietal suture beyond first month of life

• Name the investigation with view• Spot the diagnosis with grade• Significance of the diagnosis and

intervention• Protective antenatal factors

Spot 2Spot 2Prematurely (28 wks) born SGA baby, ventilated for 18 days, hemodynamically was unstable and had neonatal seizures. USG cranium done at 6 wks of age is shown here…

• Sonogram cranium (coronal view) showing cystic periventricular cystic periventricular leukomalacialeukomalacia

• Cystic Periventricular Leukomalacia, Grade III (De Veries classification)Grade III (De Veries classification)

• C-PVL strongest indicator of cerebral strongest indicator of cerebral palsypalsy, especially spastic diplegia so early stimulation programme with occupational therapy

• Antenatal steroidsAntenatal steroids and Anemia

3 yr old male child from Utter Pradesh admitted with high grade fever x 4-5 days and generalized seizures for 1 day. There were (B/L)6th and 7th CN palsy with dystonias. Similar cases are being reported from the same area. His most important investigation is given here….

Spot 3Spot 3

Name of the investigation and identify the SpotMain transmitter to manSubclinical cases, what %Preventive strategy

• T2-weighted MRI of the brain showing Panda signPanda sign

• Female Culex mosquito (Culex Culex tritanirhynchus and Vishnuitritanirhynchus and Vishnui)

• 99% cases are sub clinical• VaccineVaccine in the inter epidemic

period and Fogging Fogging with malathion sprayed in 3 km range from the infected cases

EEG of a 60 hrs old

newborn who required

resuscitation at Birth

with a 5 minute APGAR

of 5. Child had seizures

in first 12 hrs of life

Spot 4Spot 4

Identify the spotIts significanceName of the staging system other than Sarnat and Sarnat and give its component

• EEG showing Burst Suppression Burst Suppression patternpattern

• It indicates serious outcome in HIEserious outcome in HIE patients

• Levene’s staging systemLevene’s staging system (Mild, Moderate and Severe)– Consciousness

– Tone

– Seizures

– Sucking/Respiration

11 yrs old male child

admitted with slurring of

speech, ataxia and

dystonias. On inv.he found

to have renal tubular

acidosis and active rickets.

Spot 5Spot 5

Give the most possible diagnosisPattern of inheritanceMost specific investigationSpecific treatment and advise to family members

• Wilson diseaseWilson disease with lenticular

degeneration

• Autosomal recessive

• Hepatic copper contentHepatic copper content (µg/gm dry wt. of

liver- it exceeds >250 µg/gm dry wt.)

• D-Penicillamine with Pyridoxine and Zinc

and all family membersall family members should be screen

with slit lamp examinationwith slit lamp examination

4 yrs old male child brought with a history of developmental delay. He found to have mild mental retardation and Sensory neuropathy. He is from low SE group and his father is a laborer. X-ray is shown here…..

Spot 6Spot 6

Most probable diagnosisTreatment optionsIndication for treatmentUrine investigation

• Lead posioningLead posioning

• CaNaCaNa 22EDTAEDTA (Calcium Versanate) and BAL BAL

in symptomatic child

• If lead levels >45 µgm% (N <10 µgm%)

• Urine – Increased Zinc protoporphyrinZinc protoporphyrin

6 yrs old male child presented with seizures, delayed development, some problem in vision and fainting attack which are not due to seizures. He had myoclonic seizures during the infancy. His only clinical finding is given here

Spot 7Spot 7

Possible diagnosis and spot the arrowed structure Pattern of inheritanceInvestigation you would ask for and what do you expectOne eye findingTreatment of choice for seizures and its important ocular S?E

• Tuberous SclerosisTuberous Sclerosis, Ash leaf macule

• Autosomal Dominant traitAutosomal Dominant trait• CT head for subependymal nodes• Retinal hamartomaRetinal hamartoma

• VigabatrinVigabatrin and loss of peripheral vision

• Identify the Spot• Main Uses• Normal test indicates what?

Spot 8Spot 8

• BERABERA- Brainstem Evoked

Response Audiometry

• Post meningitis/Bilirubin induced

neurotoxity

• Normal test – normal retro normal retro

cochlear nervous pathwaycochlear nervous pathway

Spot 9Spot 9

Six days after undergoing liver transplantation, a 12 yr old patient's levels of gamma-glutamyl transferase (GGT), alkaline phosphatase, and bilirubin begin to rise.

•Which is the most appropriate next step in diagnosis?•What if kidneys were transplanted and BUN/Cr used to be increased and why?

• Ultrasound of biliary tract and Doppler studiesUltrasound of biliary tract and Doppler studies of the anastomosed vessels

• In all other solid organ transplants, deterioration of function 5-6 days out would suggest an acute rejection episode, and appropriate biopsiesappropriate biopsies would be done to confirm the diagnosis.

In the case of the liverIn the case of the liver, however, antigenic reactions are less common, whereas technical problems with the biliary and vascular anastomosis are the most common cause of early functional deterioration.

Spot 10Spot 10

A 3-year old boy presented with FTT. The child was apparently fine for the first couple of years. He began to have diarrhea with light colored stools. Although stool examinations were performed, it was unclear what the report is. The child was placed on a high protein, high calorie diet with vitamins and supplements. However, he showed very little improvement over a 4 month period. Barium exam showed "large dilated loops of hypotonic bowel"), the child was admitted with a diagnosis of celiac disease. Stool examinations has shown in the picture

•Identify the spot

•Best diagnostic

possibility

•Treatment ?

• This image contains a Giardia lamblia Giardia lamblia

trophozoitetrophozoite

• Three stool examinationsThree stool examinations on altrenate

days detects around 90%

• MetronidazoleMetronidazole is the treatment of

Choice x 5 days and Others are

Albendazole, Furazolidine

• A 13-year-old, obese boy complains of persistent knee pain persistent knee pain

for several weeksfor several weeks.. The family brings him in because he has

been limpinglimping. He sits in the examining table with the sole of

the foot on the affected side pointing to the other leg.

Physical examination is normal for the knee, but shows examination is normal for the knee, but shows

limited hip motionlimited hip motion. As the hip is flexed, the leg goes into

external rotation and cannot be rotated internallycannot be rotated internally.

Spot 11Spot 11

Which of the following is the most likely diagnosis?

• Slipped capital femoral epiphysisSlipped capital femoral epiphysis is an

orthopedic emergency.

– The clinical picture is classic: a chubby male

in his early teens who is limping and cannot

rotate his leg internally.

• A 14-year-old boy dives into the shallow end of a swimming pool and hits his head against the bottom. When he is rescued, he shows a complete lack of neurologic function below the necklack of neurologic function below the neck. He is still breathing on his ownbreathing on his own, but he cannot move or cannot move or feel his arms and legsfeel his arms and legs. The paramedics carefully immobilize his neck for transportation to the hospital, and they alert the emergency department to his impending arrival. – Once there, which treatment would most likely have an

immediate benefit for this patient? (other than ABC)

Spot 12Spot 12

• There is some evidence that high-high-

dose corticosteroids administered as dose corticosteroids administered as

soon as possiblesoon as possible after the injury will

result in a better ultimate outcome

• 8 yrs old child resident of Delhi is admitted with fever x 5-6 days, loss of appetite x 6 days and hepato splenomegaly. His peripheral smear is shown above– Spot the picture (name the form of parasite)– Name of the culture media– Drugs used to treat resistant cases

Spot 13Spot 13

• Kala Azar (LD bodies-Amastigote formLD bodies-Amastigote form,

non flagelated form)

• NNN mediaNNN media (Novy, MacNeil & Nicolle)

• AMB, Pentamidine, Aminosidine,

Miltefosine, Recombinant INF gamma,

Allopurinol and adjunct splenectomy

• Indications for changing diet in case of non or poor response

• What are diet A, B and C

2 month old male, "fussy", diarrhea and vomiting; flatulence. Breast-fed. He had Viral gastroenteritis 10 days previously. Before the infection, mother's milk was well tolerated. Body weight within normal limits. Moderately dehydrated. Urine shows presence of reducing sugars. No reaction for glucose. You make a diagnosis of lactose intolerance and start him on reduced lactose diet..

Spot 14Spot 14

• IndicationsIndications– Stool frequency >10 watery stool/day even

after 48 hrs of starting diet– Return of the signs of dehydration any time

after staring diet– Failure to establish wt,gain by 7th day of

dietary management

• Types of dietTypes of diet– Diet A (reduced lactose)– Diet B (lactose free)– Diet C (Monosaccharide diet)

2 yr old male child presented with diarrhea of 3 wksdiarrhea of 3 wks duration with failure to thrive. He was started with nutrition rich feeds (Simyl-MCT drops, HMF Simyl-MCT drops, HMF sachet and pedia suresachet and pedia sure) and antibiotics at a peripheral health center 7 fays back. Since then diarrhoea has even increased and patient is loosing weight. He was Moderately dehydrated at admission. His daily stool output comes around daily stool output comes around 185 ml185 ml and his stool Nastool Na++ 42 mEq/L and K 42 mEq/L and K+ + 3.8 3.8 mEq/LmEq/L. Sister didn’t sent the stool pH and reducing substance.

Spot 15Spot 15

What is the diagnosis based on these investigationsCalculate the osmotic GapWhat is the treatment next

• Chronic diarrhoea (Secondary lactose intolerance- Osmotic Osmotic diarrhoeadiarrhoea)

• Osmotic Gap : 290-(2 x Stool Na+K)If Gap >100 its osmotic diarrhoeaIf Gap >100 its osmotic diarrhoea

• Remove the osmotic load from the diet and stop feeding for 24 hrsstop feeding for 24 hrs and then restart the lactose free diet without adding any nutritional stuff

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