EDWARD FI SHER, MD PGY- 3

JUNE 1 1 , 201 4
Morning Report
5 yo boy with cough and fever
Presents with cough and fever x5 days, up to 103.5 F

Dx with viral illness at PCP 2 days prior to admission

Intermittent emesis, poor PO intake

Brought to PCH ED for worsening sx
5 yo boy with cough and fever
PMH: Healthy, no surgeries
No meds, NKDA
Imms: Up to date
FH: Older brother died at home at 15 months of
pneumonia/strep pneumo bacteremia (was fully
immunized, prior to PCV 13). Lots of pneumonia
on paternal side?
SH: Lives with parents, older brother, no tobacco
exposure
5 yo boy with cough and fever
T 37.3, HR 163, RR 36, 94% on RA
Gen: pale but well-appearing, tachypneic, NAD
HEENT: normal
CV: Tachycardic, no murmurs, good pulses
Resp: Diminished breath sounds on L with
crackles. No retractions
Abd: soft, NT/ND, normal BS
Skin: normal
Neuro: normal
In the ED
Due to history of poor PO, bolused 40 mL/kg
WBC 10.6 (22% bands), Hct 36.6, Plts 164
Blood cx sent
CXR: LLL pneumonia with effusion
Not hypoxic
Admit to RTU, IV ampicillin
In the RTU
Worsening work of breathing, tachypnea, increasing
effusion on CXR

As he is being transferred, blood culture turns
positive @16 hrs, DNA for S pneumo detected

Ampicillin --> ceftriaxone
On the floor
Worsening effusion --> chest tube placement

Mild hyponatremia (SIADH?)

Not hypoxic

Subsequent blood cultures negative
Overnight
Called to evaluate pt ~2300

Worsened tachycardia (130s --> 170s)

Looks more pale/yellow, more swollen

Nurse is concerned he looks bad, and wants to call a
rapid response
Overnight
T 37.6, HR 167, RR 40, BP 107/62, 88% on RA
Gen: pale/yellow/greenish, not wanting to interact
HEENT: very pale conjunctiva
CV: Tachycardic, no murmurs, good pulses, cap
refill hard to elicit
Resp: Diminished breath sounds on L with
crackles. No retractions. Chest tube in place
Abd: soft, NT/ND, normal BS, no HSM
Skin: normal
Neuro: normal
Differential diagnosis
Heme
Blood loss (Chest tube,
hemothorax, abdomen, GI
bleed)
Hemolysis
Anemia of inflammatory
state
DIC
TTP
Rheum
Vasculitis

Renal
HUS
Labs
WBC 17.6 (23% bands), Hgb 3.9, Hct 11.1, Plts 46

Smear 2+ schistocytes

Na 136, K 4.7, Cl 113, bicarb 17, BUN 33, creat
0.71, gluc 121, alb 2.1, bili 2.1, ALT 26, AST 213

PT/INR 15.4/1.2, PTT 48, fibrinogen 633

D-dimer 4467
Hemolytic-Uremic Syndrome
Microangiopathic hemolytic anemia

Thrombocytopenia

Acute kidney injury
HUS Classifications
Diarrhea positive/negative

Primary (complement gene mutations, antibodies to
complement factor B)
Secondary (usually infectious)
Epidemiology
STEC: 90% of HUS cases

Pneumococcus: ~5% (40% of non-STEC cases)

HUS in ~0.5% of pneumococcal infections
Pathogenesis (?)
Certain serotypes of strep
pneumo expose Thomsen-
Friedenreich (T) antigen
(serotype 19A)
Preformed host IgM bind
T antigen
Cascade of events lead to
HUS
Alternative complement-
mediated pathway?
Management
Treat underlying pneumococcal infection -
empirically if necessary
Consider rates of resistance
HUS therapy largely supportive
RBCs and platelets
STOP nephrotoxic drugs
Nephrology involvement (dialysis?)
Avoid FFP, plasmapheresis (theoretical)
Risk Factors and Outcome
Generally more severe than STEC HUS
Mortality higher (5-10% vs 12%)
More likely to require dialysis (67-80%)
Need more transfusions (PRBs and plts)
Death usually due to infection
Biggest risk factor: meningitis (88% of deaths in the largest
review)
~10% evolve to ESRD requiring dialysis
References
Banerjee R et al. Streptococcus pneumoniae-associated hemolytic
uremic syndrome among children in North America. Pediatr Infect Dis
J 30:736, 2011
Copelovitch L, Kaplan BS. Streptococcus pneumoniae-associated
hemolytic uremic syndrome. Pediatr Nephrol 23:1951-56, 2008
Copelovitch L, Kaplan BS. Streptococcus pneumoniae-associated
hemolytic uremic syndrome: classification and the emergence of
serotype 19A. Pediatrics 125:174-182, 2010
Geary DF. Hemolytic uremic syndrome and Streptococcus
pneumoniae: improving our understanding. J Pediatr 151:113, 2007
Spinale JM et al. Update on Streptococcus pneumoniae-associated
hemolytic uremic syndrome. Curr Opin Pediatr 25:203-8, 2013