PMID- 19948573
OWN - NLM
STAT- MEDLINE
DCOM- 20100204
LR  - 20131121
IS  - 1098-4275 (Electronic)
IS  - 0031-4005 (Linking)
VI  - 125
IP  - 1
DP  - 2010 Jan
TI  - Hereditary spherocytosis in neonates with hyperbilirubinemia.
PG  - 120-5
LID - 10.1542/peds.2009-0864 [doi]
AB  - OBJECTIVES: Hereditary spherocytosis (HS) is the most common inherited hemolytic 
      disease among people of Northern European decent. Neonates with HS can develop 
      significant hyperbilirubinemia, but we suspect that HS is underrecognized as a 
      cause of neonatal jaundice. METHODS: We used electronic record repositories of 
      Intermountain Healthcare to identify all neonates with a diagnosis of HS in a 
      recent 5-year period. We compared these with the number of new HS cases 
      anticipated on the basis of national prevalence and also with the number who had 
      elevations in mean corpuscular hemoglobin concentration (MCHC), red cell 
      distribution width (RDW), and bilirubin. We compared MCHC and RDW values of 
      neonates who had direct antiglobulin test-positive (DAT[+]) and DAT(-) results 
      and jaundice with values of neonates who had HS. RESULTS: Eight neonates received 
      a diagnosis of HS; however, we may have failed to diagnose 90% of cases. To 
      assess whether some with a missed diagnosis of HS developed significant 
      hyperbilirubinemia, we examined records of all 670 with a bilirubin level of 
      >or=20 mg/dL. Records of the 20 with the highest MCHC and RDW values suggested 
      that HS was present but unrecognized in at least 7. Follow-up revealed a 
      subsequent diagnosis of HS in 5; the other 2 are no longer in our health system. 
      MCHC and RDW values were highest in those with HS, intermediate in the DAT(+) 
      group, and lowest in the DAT(-) group. An MCHC of >or=36.0 g/dL had 82% 
      sensitivity and 98% specificity for identifying HS. CONCLUSION: We speculate that 
      HS is underrecognized as a cause of neonatal hyperbilirubinemia. We speculate 
      further that an MCHC of >or=36.0 g/dL can alert caregivers to the possibility of 
      HS.
FAU - Christensen, Robert D
AU  - Christensen RD
AD  - Department of Women and Newborns, Intermountain Healthcare, Salt Lake City, Utah 
      84403, USA. rdchris4@ihc.com
FAU - Henry, Erick
AU  - Henry E
LA  - eng
PT  - Comparative Study
PT  - Journal Article
DEP - 20091130
PL  - United States
TA  - Pediatrics
JT  - Pediatrics
JID - 0376422
RN  - RFM9X3LJ49 (Bilirubin)
SB  - IM
MH  - Bilirubin/blood/metabolism
MH  - Comorbidity
MH  - Confidence Intervals
MH  - Female
MH  - Humans
MH  - Hyperbilirubinemia, Neonatal/*diagnosis/*epidemiology
MH  - Incidence
MH  - Infant, Newborn
MH  - Male
MH  - Neonatal Screening/methods
MH  - Prognosis
MH  - Registries
MH  - Retrospective Studies
MH  - Risk Assessment
MH  - Severity of Illness Index
MH  - Spherocytosis, Hereditary/*diagnosis/*epidemiology
MH  - United States/epidemiology
EDAT- 2009/12/02 06:00
MHDA- 2010/02/05 06:00
CRDT- 2009/12/02 06:00
PHST- 2009/12/02 06:00 [entrez]
PHST- 2009/12/02 06:00 [pubmed]
PHST- 2010/02/05 06:00 [medline]
AID - peds.2009-0864 [pii]
AID - 10.1542/peds.2009-0864 [doi]
PST - ppublish
SO  - Pediatrics. 2010 Jan;125(1):120-5. doi: 10.1542/peds.2009-0864. Epub 2009 Nov 30.