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 language | English (US) |
|---|---|
| Pages (from-to) | 21-24 |
| Number of pages | 4 |
| Journal | The Journal of Pediatrics |
| Volume | 91 |
| Issue number | 1 |
| DOIs | |
| State | Published - 1977 |
Fingerprint
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 journal › Article
}
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 -