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Title: Case%20Management%20%20%20%20%20%20%20%20%2022nd%20Dec%202009

Case Management 22nd
Dec 2009
  • By
  • Mudita 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 Downs 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

  • MC, a 12 year old with a complex medical history
    -Noonans 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
  • 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
  • DRUG ALLERGIES Amphotericin B and Chloral
  • MEDICATIONS home TPN ,Oseltamivir x1day
  • DEVELOPMENT delayed, nonverbal
  • SOCIAL HISTORY lives with parents, brother,
    sister, dog, no smokers

  • 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
  • Blood culture ( PERIPHERAL AND CENTRAL ) no
    growth on day 2 AND afebrile for gt24 hrs, he was
    discharged home.
  • Home medications included Oseltamivir for 3 more

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
  • 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.

  • 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

  • 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
  • The cost to the health-care system is 25,000
    per episode
  • Nosocomial BSI prolong hospitalization by 7
  • Pittet D, Tarara D, Wenzel RP. JAMA. May 25
  • Soufir L et al. Infect Control Hosp Epidemiol
    1999 Jun20(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


Laboratory-confirmed bloodstream infection (LCBI)
  • Patient has at least one of the following signs
    or symptoms fever (gt38 C), chills, or
  • 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

Laboratory-confirmed bloodstream infection (LCBI)
  • Criterion 3
  • Patient lt 1 year of age has at least one of
    the following signs or symptoms fever (gt38 C
    core) hypothermia (lt36 C core), apnea, or
  • 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
Patients in United States Hospitals
Epidemiology, Clinical Features, and
Wisplinghoff H, et al Pediatr Infect Dis J.
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
  • Catheter-related bacteremia was confirmed by
    differential quantitative blood cultures
    (gtor31) or time to positivity (gt2 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
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).
  • CONCLUSIONSCVC 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 1549(8)1187-94.
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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

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Duration of therapy
  • Treatment for CLABSI depends on
  • Complications related to bacteremia(
  • 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 gt72 hours following
    catheter removal - treatment for at least 4 to 6

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 broadspectrum

Removal of catheter
  • Severe sepsis
  • Hemodynamic instability
  • Endocarditis or evidence of metastatic infection
  • Erythema or exudate due to suppurative
  • Persistent bacteremia after 72 hours of
    antimicrobial therapy to which the organism is
  • Candidial CLASBI

Trends in Central line assosciated blood stream
  • 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 491.
  • Mermel, LA. Prevention of intravascular
    catheter-related infections. Ann Intern Med 2000
  • Institute for Healthcare ImprovementGetting
    Started Kit Prevent Central Line Infections.
  • 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)396401, 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
  • Reduction of catheter related bloodstream
    infections in intensive care one for all, all
    for one?Helder OK, Latour JM.Nurs Crit Care. 2009
    May-Jun14(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-Jun14(3)107-8. Review
  • Management of bacteremia in patients undergoing
    hematopoietic stem cell transplantation.Castagnola
    E, Faraci M.

  • Expert Rev Anti Infect Ther. 2009
    Jun7(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
  • 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 74340
  • Friedman, ND, Korman, TM, Fairley, CK, et al.
    Bacteraemia due to Stenotrophomonas maltophilia
    an analysis of 45 episodes. J Infect 2002 4547.
  • Safdar, N, Handelsman, J, Maki, DG. Does
    combination antimicrobial therapy reduce
    mortality in Gram-negative bacteraemia? A
    meta-analysis. Lancet Infect Dis 2004 4519.
  • 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

  • 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

  • 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 (lt10)
  • 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
  • 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

NICU Course
  • Resp Remained on SIMV throughout
    admission, trialed off of vent DOL 3 and DOL
  • 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

NICU Course
  • Ophthalmology consulted abnl appearing fundus
    optic nerve that was avascular and bland
    appearing, no evidence of glaucoma or
  • 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

  • Review congenital muscular dystrophy
  • Discuss Walker-Warburg syndrome and its
    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
  • Collagen IV
  • a-dystroglycan
  • Merosin (laminin a2)

Walker-Warburg Syndrome
  • Detailed descriptions pioneered by Mette Warburg
    and A.Earl Walker
  • Originally HARDE
  • Incidence not known
  • Present at least in Europe, Western Hemisphere,
  • 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
  • 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

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  • 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
  • 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
  • Family income ,Family education
  • Mistrust of healthcare providers
  • Religious beliefs
  • Conclusions
  • Sensitivity to culturally mediated differences
  • 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
  • Skin biopsy pending
  • Muscle biopsy pending

  • Cormand et al. Clinical and genetic distinction
    between Walker-Warburg syndrome and
    muscle-eye-brain disease. Neurology
  • 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 200871312-321.
  • Nishino I, Ozawa E. Muscular dystrophies. Curr
    Opin Neurol 200215539-544.
  • Muntoni F, Sewry CA. Congenital muscular
    dystrophy. Neurology 199851 14-16.
  • 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
    20047 933-937.
  • Wocial LD. Life Support Decisions Involving
    Imperiled Infants. J Perinatal Neonatal
    Nursing. 200014 73-86.
  • Kopelman AE. Understanding, Avoiding, and
    Resolving End-of-Life Conflicts in the NICU. Mt.
    Sinai J Med 200673580-586.