Case Management 22nd Dec 2009

ByMudita Mittal MBBS

&Kismet Baldwin MD

November Cases NG 9 yo with Type 1 DM presented

with asymmetric LE weakness, dx with non-polio enter-viral poliomyelitis MWH for rehab

UM 15 yo with Down’s Synd s/p MV replacement, on Warfarin, in CHF, came in with altered mental status, GI bleed, INR 4.8, found to be in MOF, declared DNR , expired a day later

HPI MC, a 12 year old with a complex medical history -

Noonan’s Syndrome with short – gut syndrome, was admitted on 10/17/2009 with complaint of fever, increased fatigue and decreased appetite.

Fever was documented as high as 105.6 Central line was repaired 2 days prior to onset of

fever. Positive sick contact : sister- H1N1 flu x 1 week

Past History PAST SURGICAL

HISTORY:

VSD and coarctation of Aorta repair at 1 week of age,

S/P orchidopexy, surgery on both LL for limb lengthening & midgut volvulus repair

Multiple hospitalizations for central line infection

IMMUNIZATIONS: UTD DRUG ALLERGIES:

Amphotericin B and Chloral hydrate

MEDICATIONS: home TPN ,Oseltamivir x1day

DEVELOPMENT: delayed, nonverbal

SOCIAL HISTORY: lives with parents, brother, sister, dog, no smokers

PHYSICAL EXAMINATION: General appearance:

In no acute distress Weight -29.2 kg. T-max 38.5 orally(ER), BP-109/49, pulse 94, RR 20, Sat 99% on room air.

HEENT: Oropharynx -small herpetic blisters on the left upper lip and tongue.

Chest: clear to auscultation

CVS- RRR, Crescendo-decrescendo murmur

Abdominal exam: Bowel sounds present, nontender.

Laboratory values

Laboratory values

Hospital Course The patient remained afebrile. Flu screen : negative. Continued on Cefepime and Vancomycin

intravenously , Oseltamivir 60 mg p.o. daily Continued on TPN & was able to tolerate p.o as well. Blood culture ( PERIPHERAL AND CENTRAL ) no

growth on day 2 AND afebrile for >24 hrs, he was discharged home.

Home medications included Oseltamivir for 3 more days.

Readmission on 10/20/09

Soon after being discharged, the pediatric GI service was notified that the patient had a positive central line culture (on 10/17/2009) which showed gram-positive cocci in pairs and chains.

He was readmitted for repeat central line culture and for the initiation of vancomycin.

On admission, the patient appeared well. He had no foci of infection , remained afebrile and had stable vital signs.

Readmission on 10/19/09

Patient discharged home on 10/20/09 with home nursing for continuation of vancomycin.

Final blood culture report ( from previous central line culture on 10/17/09)- Streptococcus viridans isolated, sensitive to Penicillin.

Final blood culture report from central line culture on readmission :no growth.

OBJECTIVES

To discuss The Incidence of central line infections The pathogens causing Central line Infection The treatment of Central line Infections The concept of Central Line Bundle. Trends in the last decade for Central line

infections

Incidence

Each year, an estimated 250,000 cases of Central Line Associated Blood Stream Infections (CLABSI)occur in hospitals in the United States,

An attributable mortality of 12%--25% for each infection The cost to the health-care system is $25,000 per

episode Nosocomial BSI prolong hospitalization by 7 dayswww.CDC.gov

Pittet D, Tarara D, Wenzel RP. JAMA. May 25 1994;271(20):1598-1601.

Soufir L et al. Infect Control Hosp Epidemiol 1999 Jun;20(6):396-401

Laboratory-confirmed bloodstream infection (LCBI)

Criterion 1: Recognized pathogen cultured from one or more

blood cultures & Organism cultured from blood is not related to an

infection at another site

www.cdc.gov

Laboratory-confirmed bloodstream infection (LCBI)

Patient has at least one of the following signs or symptoms: fever (>38 C), chills, or hypotension AND

signs and symptoms and positive laboratory results are not related to an infection at another site

AND common skin contaminant (i.e., Diphtheroids [Corynebacterium

sp.], Bacillus [not B. anthracis] sp., Propionibacterium sp., coagulase-negative Staphylococci [including S. epidermidis], viridans group Streptococci, Aerococcus spp., Micrococcus spp.) is cultured from two or more blood cultures drawn on separate occasions

Laboratory-confirmed bloodstream infection (LCBI)

Criterion 3

Patient < 1 year of age has at least one of the following signs or symptoms: fever (>38 C core) hypothermia (<36 C core), apnea, or bradycardia

and signs and symptoms and positive laboratory results

are not related to an infection at another site and Common skin contaminant is cultured from two or

more blood cultures drawn on separate occasions.

Nosocomial Bloodstream Infections in Pediatric Nosocomial Bloodstream Infections in Pediatric Patients in United States Hospitals: Epidemiology, Patients in United States Hospitals: Epidemiology,

Clinical Features, and SusceptibilitiesClinical Features, and Susceptibilities

Wisplinghoff H, et al: Pediatr Infect Dis J. 2003;22:686–691.

Special situations

Pseudomonas aeruginosa -burn patients. S. aureus in- HIV-infected patients . Gram-negative pathogens -hematologic and non-

hematologic malignancies. Hydrophilic gram-negative pathogens such as

Pseudomonas spp, Acinetobacter spp, and Serratia marcescens - needleless access device.

Management of the Catheter in Documented Catheter -Related CoNS bacteremia: Remove or Retain?

Methods :During the period from July 2005 through December 2007, retrospectively evaluated 188 patients with coagulase-negative staphylococcal bacteremia.

Catheter-related bacteremia was confirmed by differential quantitative blood cultures (>or=3:1) or time to positivity (>2 h).

RESULTS: Resolution of infection within 48 h after commencement of antimicrobial therapy was not influenced by CVC removal or exchange vs retention and occurred in 175 patients (93%).

Raad I, Kassar R, Ghannam D, Chaftari AM, Hachem R, Jiang Y.Clin Infect Dis. 2009 Oct 15;49(8):1187-94

Coagulase-negative Staphylococcal Bacteremia

Duration of therapy did not affect recurrence. Multiple logistic regression analysis - patients with

catheter retention were 6.6 times (95% CI, 1.8-23.9 times) more likely to have a recurrence than were those whose catheter was removed or exchanged (P = .004).

CONCLUSIONS:CVC retention does not have an impact on the resolution of coagulase-negative staphylococcal bacteremia but is a significant risk factor of recurrence.

Clin Infect Dis. 2009 Oct 15;49(8):1187-94.

The Central Line Bundle

Hand hygiene Maximal barrier precautions Chlorhexidine skin antisepsis Optimal catheter site selection, with

subclavian vein as the preferred site for non-tunneled catheters in adults

Daily review of line necessity with prompt removal of unnecessary lines

Treatment

Treatment

Duration of therapy

Treatment for CLABSI depends on Complications related to bacteremia( endocarditis) Line salvage needed. Organism type.

For uncomplicated CLASBI with negative blood cultures following catheter removal the duration of therapy is usually 10 to 14 days .

Persistent bacteremia >72 hours following catheter removal - treatment for at least 4 to 6 weeks.

Suspected Catheter-Related Candidemia

Empiric therapy for suspected catheter-related Candidemia should be administered for septic patients with the following risk factors

Total parenteral nutrition Prolonged use of broad-spectrum antibiotics Hematologic malignancy Bone marrow or solid organ transplant Femoral catheterization Colonization due to Candida species at

multiple sites NICU babies on prolonged broad-spectrum abx Multi-system trauma patients on broad–spectrum abx

Removal of catheter Severe sepsis Hemodynamic instability Endocarditis or evidence of metastatic infection Erythema or exudate due to suppurative

thrombophlebitis Persistent bacteremia after 72 hours of antimicrobial

therapy to which the organism is susceptible Candidial CLASBI

Trends in Central line assosciated blood stream infection

References Mermel, LA, Allon, M, Bouza, E, et al. Clinical practice guidelines for the diagnosis and management of

intravascular catheter-related infection: 2009 Update by the Infectious Diseases Society of America. Clin Infect Dis 2009; 49:1.

Mermel, LA. Prevention of intravascular catheter-related infections. Ann Intern Med 2000; 132:391

Institute for Healthcare Improvement:Getting Started Kit: Prevent Central Line Infections. 2005

Soufir, L., Timsit, J., Mahe, C., et al.: “Attributable Morbidity and Mortality of Catheter-Related Septicemia in Critically Ill Patients: A Matched, Risk-Adjusted, Cohort Study , ”Infection Control and Hospital Epidemiology. 20(6):396–401, 1999.

Management of the catheter in documented catheter-related coagulase-negative staphylococcal bacteremia: remove or retain?Raad I, Kassar R, Ghannam D, Chaftari AM, Hachem R, Jiang Y.Clin Infect Dis. 2009 Oct 15;49(8):1187-94.

Reduction of catheter related bloodstream infections in intensive care: one for all, all for one?Helder OK, Latour JM.Nurs Crit Care. 2009 May-Jun;14(3):107-8. Review

Reduction of catheter related bloodstream infections in intensive care: one for all, all for one?Helder OK, Latour JM.Nurs Crit Care. 2009 May-Jun;14(3):107-8. Review

Management of bacteremia in patients undergoing hematopoietic stem cell transplantation.Castagnola E, Faraci M.

References Expert Rev Anti Infect Ther. 2009 Jun;7(5):607-21. Review Seifert, H. Catheter-related infections due to gram-negative bacilli. In: Seifert H, Jansen B, Farr BM,

eds. Catheter-Related Infections. New York, NY: Marcel Drekker 1997. p. 111. Lorente, L, Jimenez, A, Santana, M, et al. Microorganisms responsible for intravascular catheter-

related bloodstream infection according to the catheter site. Crit Care Med 2007; 35:2424. Management of bacteremia in patients undergoing hematopoietic stem cell

transplantation.Castagnola E, Faraci M. Seifert, H, Strate, A, Pulverer, G. Nosocomial bacteremia due to Acinetobacter baumannii. Clinical

features, epidemiology, and predictors of mortality. Medicine (Baltimore) 1995; 74:340 Friedman, ND, Korman, TM, Fairley, CK, et al. Bacteraemia due to Stenotrophomonas maltophilia:

an analysis of 45 episodes. J Infect 2002; 45:47. Safdar, N, Handelsman, J, Maki, DG. Does combination antimicrobial therapy reduce mortality in

Gram-negative bacteraemia? A meta-analysis. Lancet Infect Dis 2004; 4:519. O'Grady, NP, Alexander, M, Dellinger, EP, et al. Guidelines for the prevention of intravascular

catheter-related infections. Centers for Disease Control and Prevention. MMWR Morb Mortal Wkly Rep 2002; 51(RR-10):1. Accessed at: http://www.cdc.gov/mmwr/preview/mmwrhtml/rr5110a1.htm.

HPI• BK, a 35 week 3/7day girl born to a 20yo G1P0

mom and 21yo father• GBS unknown, HIV unknown, otherwise prenatal

labs wnl• Pregnancy complications:

– Polyhydramnios s/p amnioreduction– Poor biophysical profile 6/10 – Clenched hands and abnl cerebellum on routine US– Fetal MRI: Dandy-Walker malformation, posterior fossa

cysts, absent inf. vermis, communicating b/l cisterna magna and 4th ventricles

HPI

Delivered via C-section Apgars 2,6,6 Limp, apneic dry stimulation, PPV,

intubated, admitted to NICU

Physical Exam

Wt: 2320g (26-50%) HC: 31.5cm (26-50%)

Length: 42.5cm (<10%)

Gen: intubated, little spontaneous movement

HEENT: wnl

Resp: no spontaneous respirations, on SIMV

CV: RRR, S1 S2, no murmurs

Abd: soft, flat, no HSM, scattered bowel sounds

GU: nl female genitalia Ext: 2+ femoral pulses,

mild contractures of hips, knees, elbows, toes, L club foot, clenched hands

Neuro: little spontaneous movement, occasional jerky movements or tremors

Skin: no rashes

Initial Labs ABG: 7.24/68/27/0/+29 CBC: 15.1 5 bands, 37 N, 43 L,

13.8 301 10 M,

43.5

Glucose: 112

Urine DRABs: negative

CXR: clear lungs, hypoinflated, ETT in good position

NICU Course Resp: Remained on SIMV throughout

admission, trialed off of vent DOL #3 and DOL #5

CV: Stable throughout ID: Stable, admission B/C and

Isolation/C negative Heme: Stable Fen/GI: NPO day 1 TPN reg TPN. no

stools, no spontaneous urination noted since DOL #2

NICU Course Neuro:

Jerky movements, clonus, ?eye deviations EEG: burst suppression patterns MRI: small brain stem and cerebellar vermis, no

definite cerebellar fossa, dilated 4th ventricle, marked decreased. sulci

Peds Neurology and Genetics consulted CPK, microarray, skin biopsy, muscle biopsy plasma

amino acids , urine organic acids sent CPK 348, lactate 2.2

Imaging

NICU Course Ophthalmology consulted: abnl appearing

fundus & optic nerve that was avascular and bland appearing, no evidence of glaucoma or micro-ophthalmos

Family meetings were held on DOL #3 and 5 Life support was discontinued on DOL #6 and

the patient died ~12min after ETT was removed surrounded by family

Objectives

Review congenital muscular dystrophy Discuss Walker-Warburg syndrome and

it’s differential diagnosis Discuss factors affecting parental

decision making in end of life situations

Congenital Muscular Dystrophy Heterogeneous group of inherited muscle

disorders Majority: muscle only, some eye and nervous

system also Among the most common of autosomal recessive

neuromuscular disorders Frequencies of different forms unknown Accurate clinical phenotype and comprehensive

protein and genetic analysis necessary for diagnosis of specific form

Currently 12 genetically defined forms of CMD

Three major groups based on class of proteins affected Collagen IV α-dystroglycan Merosin (laminin α2)

Walker-Warburg Syndrome Detailed descriptions pioneered by Mette

Warburg and A.Earl Walker Originally HARD+E Incidence not known Present at least in Europe, Western

Hemisphere, Japan Autosomal recessive inheritance

• Differential diagnosis:– Muscle-eye-brain disease– Fukuyama congenital muscular dystrophy– CMD without brain and eye abnormalities

• Several genes implicated– Protein O-mannosyltransferase 1 and 2

(POMT1 and 2)– Fukutin related protein (FKRP) genes– Only 10-20% of cases with these gene

mutations

Walker-Warburg Syndrome Major criteria:

CMD with hypoglycosylation of alpha-dystroglycan

High creatinine kinase Anterior or posterior eye anomalies Migrational brain defect w/type II

lissencephaly and hydrocephalus Abnormal brainstem/cerebellum

Major criteria: CMD with hypoglycosylation of alpha-

dystroglycan High creatinine kinase Anterior or posterior eye anomalies Migrational brain defect w/type II

lissencephaly and hydrocephalus Abnormal brainstem/cerebellum

Walker-Warburg Syndrome Workup:

Creatinine kinase muscle biopsy Ophthalmology exam

Prognosis: Most children die before 3yr old, usually in first

month of life Treatment:

No specific treatment Supportive and preventative care

End of life decision making What parents want:

Clear, accurate, timely exchange of information Factors affecting parents’ decisions:

Meaning parents attribute to providers’ comments

Acceptance of critical nature of situation Parents’ perception of provider humility Providers’ level of caring

Need for information Parents’ understanding and

comprehension of situation Presentation Trust of providers and information given Parents’ involvement in decisions

Experiences may reflect education, ethnicity, religion, health insurance, or combination

No studies examining racial differences in end of life decision making in critically ill infants

African American adults more likely than white adults to desire continuation of Life-Sustaining Medical Treatment (LSMT)

Moseley et al: Pilot study Examined frequency of withdrawal of life sustaining

medical treatment in AA parents vs. white parents Retrospective chart review

38 infant charts: 22 AA, 16 White 13/22 AA infants received recommendation to

LSMT 8 accepted recommendation (62%) 10/16 white infants received recs to LSMT

8/10 accepted (80%) Not statistically significant but, consistent with

adult literature Why?

Poor family and provider communication

Lack of provider and patient/family racial concordance

Family income ,Family education Mistrust of healthcare providers Religious beliefs

Conclusions: Sensitivity to culturally mediated differences

essential Knowledge of end of life concerns & preferences of

minorities needed Need larger study with sufficient power

Follow up

Autopsy: b/l microphthalmia Asymmetric crown of

head L club foot Hypoplastic nose bridge Distended bladder Dilated renal calyces Bile stained liver Atrophy of skeletal

muscle

Microarray: No significant DNA copy

number changes No increased

homozygosity Urine organic acids:

Marked excretion of N-acetyltyrosine

Serum aa: not suggestive of any inborn error of metabolism

Skin biopsy: pending Muscle biopsy: pending

References1. Cormand et al. Clinical and genetic distinction between Walker-Warburg

syndrome and muscle-eye-brain disease. Neurology 2001;56:1059-1069.

2. Peat RA, Smith JM, Compton AG, Baker NL, Pace RA, Burkin DJ, Kaufman SJ, Lamnade SR, North KN. Diagnosis and etiology of congenital muscular dystrophy. Neurology 2008;71:312-321.

3. Nishino I, Ozawa E. Muscular dystrophies. Curr Opin Neurol 2002;15:539-544.

4. Muntoni F, Sewry CA. Congenital muscular dystrophy. Neurology 1998;51: 14-16.

5. Moseley KL, Church A, Hempel B, Yuan H, Goold SD, Freed GL. End-of-Life Choices for African-American and White Infants in a Neonatal Intensive-Care Unit: A Pilot Study. J NMA 2004;7: 933-937.

6. Wocial LD. Life Support Decisions Involving Imperiled Infants. J Perinatal & Neonatal Nursing. 2000;14: 73-86.

7. Kopelman AE. Understanding, Avoiding, and Resolving End-of-Life Conflicts in the NICU. Mt. Sinai J Med 2006;73:580-586.