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.
N1 - Funding Information:
From the Division of Hematology-Oncology, Department of Medicine, Children's Hospital Medical Center, and the Department of Pediatrics, Harvard Medical School. Supported by United States Public Health Service Grant A M-05581. *Reprint address: Department of Pediatrics, University of Texas Health Science Center at Dallas, 5323 Harry Hines Blvd., Dallas TX 75235. **Recipient of a Research Career Development A ward (A M-00156) from the National Institutes of Health.
PY - 1977/7
Y1 - 1977/7
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.
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U2 - 10.1016/S0022-3476(77)80436-8
DO - 10.1016/S0022-3476(77)80436-8
M3 - Article
C2 - 874660
AN - SCOPUS:0017760208
SN - 0022-3476
VL - 91
SP - 21
EP - 24
JO - The Journal of pediatrics
JF - The Journal of pediatrics
IS - 1
ER -