Benign course of extreme hyperbilirubinemia in sickle cell anemia

Analysis of six cases

George R. Buchanan, Bertil E. Glader

Research output: Contribution to journalArticle

44 Citations (Scopus)

Abstract

Since the approach to the management and outcome of extreme hyperbilirubinemia in patients with sickle cell anemia is not clearly defined, we reviewed our experience with marked hyperbilirubinemia in six children with sickle cell disease. Intrahepatic sickling (sickle hepatopathy) rather than hepatitis or biliary stones appeared primarily responsible for the extreme jaundice in at least four children and possibly in all six. Signs and symptoms were few, and laboratory abnormalities were not striking other than marked hyperbilirubinemia (total serum bilirubin concentrations ranging from 20.4 to 57.6 mg/dl with approximately one half conjugated). All of the children improved within days to weeks and currently are well, without recurrence of hyperbilirubinemia or evidence of chronic liver disease. The patients described here suggest that sickling within the liver, previously reported to be a serious and even fatal syndrome, usually is a benign and self-limited process.

Original languageEnglish (US)
Pages (from-to)21-24
Number of pages4
JournalThe Journal of Pediatrics
Volume91
Issue number1
DOIs
StatePublished - 1977

Fingerprint

Hyperbilirubinemia
Sickle Cell Anemia
Jaundice
Bilirubin
Hepatitis
Signs and Symptoms
Liver Diseases
Chronic Disease
Recurrence
Liver
Serum

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Benign course of extreme hyperbilirubinemia in sickle cell anemia : Analysis of six cases. / Buchanan, George R.; Glader, Bertil E.

In: The Journal of Pediatrics, Vol. 91, No. 1, 1977, p. 21-24.

Research output: Contribution to journalArticle

@article{b654c14f729245eb801f0cb327d91441,
title = "Benign course of extreme hyperbilirubinemia in sickle cell anemia: Analysis of six cases",
abstract = "Since the approach to the management and outcome of extreme hyperbilirubinemia in patients with sickle cell anemia is not clearly defined, we reviewed our experience with marked hyperbilirubinemia in six children with sickle cell disease. Intrahepatic sickling (sickle hepatopathy) rather than hepatitis or biliary stones appeared primarily responsible for the extreme jaundice in at least four children and possibly in all six. Signs and symptoms were few, and laboratory abnormalities were not striking other than marked hyperbilirubinemia (total serum bilirubin concentrations ranging from 20.4 to 57.6 mg/dl with approximately one half conjugated). All of the children improved within days to weeks and currently are well, without recurrence of hyperbilirubinemia or evidence of chronic liver disease. The patients described here suggest that sickling within the liver, previously reported to be a serious and even fatal syndrome, usually is a benign and self-limited process.",
author = "Buchanan, {George R.} and Glader, {Bertil E.}",
year = "1977",
doi = "10.1016/S0022-3476(77)80436-8",
language = "English (US)",
volume = "91",
pages = "21--24",
journal = "Journal of Pediatrics",
issn = "0022-3476",
publisher = "Mosby Inc.",
number = "1",

}

TY - JOUR

T1 - Benign course of extreme hyperbilirubinemia in sickle cell anemia

T2 - Analysis of six cases

AU - Buchanan, George R.

AU - Glader, Bertil E.

PY - 1977

Y1 - 1977

N2 - Since the approach to the management and outcome of extreme hyperbilirubinemia in patients with sickle cell anemia is not clearly defined, we reviewed our experience with marked hyperbilirubinemia in six children with sickle cell disease. Intrahepatic sickling (sickle hepatopathy) rather than hepatitis or biliary stones appeared primarily responsible for the extreme jaundice in at least four children and possibly in all six. Signs and symptoms were few, and laboratory abnormalities were not striking other than marked hyperbilirubinemia (total serum bilirubin concentrations ranging from 20.4 to 57.6 mg/dl with approximately one half conjugated). All of the children improved within days to weeks and currently are well, without recurrence of hyperbilirubinemia or evidence of chronic liver disease. The patients described here suggest that sickling within the liver, previously reported to be a serious and even fatal syndrome, usually is a benign and self-limited process.

AB - Since the approach to the management and outcome of extreme hyperbilirubinemia in patients with sickle cell anemia is not clearly defined, we reviewed our experience with marked hyperbilirubinemia in six children with sickle cell disease. Intrahepatic sickling (sickle hepatopathy) rather than hepatitis or biliary stones appeared primarily responsible for the extreme jaundice in at least four children and possibly in all six. Signs and symptoms were few, and laboratory abnormalities were not striking other than marked hyperbilirubinemia (total serum bilirubin concentrations ranging from 20.4 to 57.6 mg/dl with approximately one half conjugated). All of the children improved within days to weeks and currently are well, without recurrence of hyperbilirubinemia or evidence of chronic liver disease. The patients described here suggest that sickling within the liver, previously reported to be a serious and even fatal syndrome, usually is a benign and self-limited process.

UR - http://www.scopus.com/inward/record.url?scp=0017760208&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0017760208&partnerID=8YFLogxK

U2 - 10.1016/S0022-3476(77)80436-8

DO - 10.1016/S0022-3476(77)80436-8

M3 - Article

VL - 91

SP - 21

EP - 24

JO - Journal of Pediatrics

JF - Journal of Pediatrics

SN - 0022-3476

IS - 1

ER -