Transcript Case Management 22nd Dec 2009 By Mudita Mittal MBBS & Kismet Baldwin MD November Cases  NG  UM 9 yo with Type 1 DM presented with asymmetric LE weakness, dx with.

Case Management
22nd Dec 2009
By
Mudita Mittal MBBS
&
Kismet Baldwin MD
November Cases
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NG
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UM
9 yo with Type 1 DM presented
with asymmetric LE weakness, dx
with non-polio enter-viral
poliomyelitis
MWH for rehab
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
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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
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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
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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:
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General appearance:
In no acute distress
Weight -29.2 kg. T-max
38.5 orally(ER), BP109/49, pulse 94, RR
20, Sat 99% on room
air.
HEENT: Oropharynx small herpetic blisters
on the left upper lip and
tongue.
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Chest: clear to
auscultation
CVS- RRR, Crescendodecrescendo murmur
Abdominal exam: Bowel
sounds present,
nontender.
Laboratory values
Laboratory values
Hospital Course
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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
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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
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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
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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
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Each year, an estimated 250,000 cases of Central
Line Associated Blood Stream Infections
(CLABSI)occur in hospitals in the United States,
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An attributable mortality of 12%--25% for each
infection
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The cost to the health-care system is $25,000 per
episode
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Nosocomial BSI prolong hospitalization by 7 days
www.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:
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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)
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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)
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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
Patients in United States Hospitals: Epidemiology,
Clinical Features, and Susceptibilities
Wisplinghoff H, et al: Pediatr Infect Dis J. 2003;22:686–691.
Special situations
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Pseudomonas aeruginosa -burn patients.
S. aureus in- HIV-infected patients .
Gram-negative pathogens -hematologic and nonhematologic 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?
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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
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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
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Hand hygiene
Maximal barrier precautions
Chlorhexidine skin antisepsis
Optimal catheter site selection, with
subclavian vein as the preferred site for nontunneled 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.
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Suspected Catheter-Related Candidemia
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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
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Multi-system trauma patients on broad–spectrum abx
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Removal of catheter
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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
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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
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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 catheterrelated 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
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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:
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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
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Delivered via C-section
Apgars 2,6,6
Limp, apneic dry stimulation, PPV,
intubated, admitted to NICU
Physical Exam
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Wt: 2320g (26-50%)
HC: 31.5cm (26-50%)
Length: 42.5cm (<10%)
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Gen: intubated, little
spontaneous movement
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HEENT: wnl
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Resp: no spontaneous
respirations, on SIMV
CV: RRR, S1 S2, no
murmurs
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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
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NICU Course
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Resp:
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CV:
ID:
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Heme:
Fen/GI:
Remained on SIMV throughout
admission, trialed off of vent DOL
#3 and DOL #5
Stable throughout
Stable, admission B/C and
Isolation/C negative
Stable
NPO  day 1 TPN  reg TPN. no
stools, no spontaneous urination
noted since DOL #2
NICU Course
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Neuro:
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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
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CPK, microarray, skin biopsy, muscle biopsy
plasma amino acids , urine organic acids sent
CPK 348, lactate 2.2
Imaging
NICU Course
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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
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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
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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
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Currently 12 genetically defined forms
of CMD
Three major groups based on class of
proteins affected
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Collagen IV
α-dystroglycan
Merosin (laminin α2)
Walker-Warburg Syndrome
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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
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Differential diagnosis:
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Muscle-eye-brain disease
Fukuyama congenital muscular dystrophy
CMD without brain and eye abnormalities
Several genes implicated
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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
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Major criteria:
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CMD with hypoglycosylation of alphadystroglycan
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:
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CMD with hypoglycosylation of alphadystroglycan
High creatinine kinase
Anterior or posterior eye anomalies
Migrational brain defect w/type II
lissencephaly and hydrocephalus
Abnormal brainstem/cerebellum
Walker-Warburg Syndrome
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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
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What parents want:
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Clear, accurate, timely exchange of
information
Factors affecting parents’ decisions:
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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
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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
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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?
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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
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Follow up
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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
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Microarray:
 No significant DNA copy
number changes
 No increased
homozygosity
Urine organic acids:
 Marked excretion of Nacetyltyrosine
Serum aa: not suggestive of
any inborn error of
metabolism
Skin biopsy: pending
Muscle biopsy: pending
References
1.
2.
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5.
6.
7.
Cormand et al. Clinical and genetic distinction between WalkerWarburg syndrome and muscle-eye-brain disease. Neurology
2001;56:1059-1069.
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.
Nishino I, Ozawa E. Muscular dystrophies. Curr Opin Neurol
2002;15:539-544.
Muntoni F, Sewry CA. Congenital muscular dystrophy. Neurology
1998;51: 14-16.
Moseley KL, Church A, Hempel B, Yuan H, Goold SD, Freed GL. Endof-Life Choices for African-American and White Infants in a Neonatal
Intensive-Care Unit: A Pilot Study. J NMA 2004;7: 933-937.
Wocial LD. Life Support Decisions Involving Imperiled Infants. J
Perinatal & Neonatal Nursing. 2000;14: 73-86.
Kopelman AE. Understanding, Avoiding, and Resolving End-of-Life
Conflicts in the NICU. Mt. Sinai J Med 2006;73:580-586.