Abstract
Acute liver failure is unusual unusual in sickle cell anaemia. We describe a child with homozygous sickle cell anaemia who developed acute liver disease of abrupt onset during an episode of limb pain. She presented with sudden onset of persistent vomiting, headache, lethargy, epistaxis, and painful liver enlargement. Laboratory investigations were indicative of cholestasis and severe liver failure with profound prolonged clotting times, hypofibrinogenaemia, elevated serum ammonia and lactic acidosis. The symptoms were promptly and completely reversed by two partial exchange transfusions. No evidence of viral infection was found. Cholelithiasis was ruled out by ultrasonography. The child recovered from what appeared to be massive hepatic sickling with no apparent sequelae.
Conclusion
Massive hepatic sickling should be considered in the differential diagnosis of a child with homozygous sickle cell disease who suddenly develops acute liver failure. Exchange transfusion should be promptly carried out so as to reverse ischaemic hepatic injury.
Similar content being viewed by others
References
Barret-Connore E (1968) Sickle disease and viral hepatitis. Ann Intern Med 69:517–527
Bauer TW, Moore GW, Hutchins GM (1980) The liver in sickle cell disease. A clinicopathologic study of 70 patients. Am J Med 69:833–837
Brozovic M, Davies SC, Brownell AI (1987) Acute admissions of patients with sickle cell disease who live in Britain. BMJ 294:1206–1208
Buchanan GR, Glader BE (1977) Benign course of externe hyperbilirubinemia in sickle cell anemia: analysis of six cases. J Pediatr 91:21–24
Diggs LW, (1966) Sickle cell crisis. Am J Clin Pathol 44:1–5
Green TW, Conley CL, Berthrong M (1953) The liver in sickle cell anemia. Johns Hopkins Med J 92:99–127
Hatton CSR, Bunch C, Weatherall DJ (1985) Hepatic sequestration in sickle cell anaemia. BMJ 290:744–745
Lama M (1993) Hepatic abscess in sickle cell anemia: a rare manifestation. Arch Dis Child 69:242–243
Lanzkowsky P, Shende A, Karayalcin G, Kim YJ, Aballi AJ (1978) Partial exchange transfusion in sickle cell anemia. Use in children with serious complications. Am J Dis Child 132: 1205–1208
Mallouh AA, Asha MI (1988) Acute cholestatic jaundice in children with sickle cell disease: hepatic crises or hepatitis? Pediatr Infect Dis J 7 (10): 689–692
Middleton JP, Wolper JC (1984) Hepatic biloma complicating sickle cell disease. Gastroenterology 86:743–744
Mills LR, Mwakyusa D, Milner PF (1988) Histopathologic features of liver biopsy specimens in sickle cell disease. Arch Pathol Lab Med 112 (3): 290–294
Owen DM, Aldridge JE, Thompson RB (1965) An unusual hepatic sequelae of sickle cell anemia: a report of five cases. Am J Med Sci 249: 175–185
Prasas AS, Cossack ZT (1984) Zinc supplementation in sickle cell diseases. Ann Intern Med 100:367–371
Russel MO, Goldbert H, Reis L (1976) Transfusion therapy for cerebrovascular abnormalities in sickle cell disease. J Pediatr 88:382–387
Schubert TT (1986) Hepatobiliary system in sickle cell disease. Gastroenterology 90:2013–2021
Sheehy TW (1977) Sickle cell hepatopathy. South Med J 70:683–688
Sheehy TW, Law DE, Wade B (1980) Exchange transfusion for sickle cell intrahepatic cholestasis. Arch Intern Med 140:1364–1366
Yohannan MF, Arif M, Ramia S (1990) Aetiology of icteric hepatitis and fulminant hepatic failure in children and the possible predisposition to hepatic failure by sickle cell disease. Acta Paediatr Scand 79 (2):201–205
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Stéphan, J.L., Merpit-Gonon, E., Richard, O. et al. Fulminant liver failure in a 12-year-old girl with sickle cell anaemia: Favourable outcome after exchange transfusions. Eur J Pediatr 154, 469–471 (1995). https://doi.org/10.1007/BF02029357
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02029357