1/6. Massive acute haemolysis in neonates with glucose-6-phosphate dehydrogenase deficiency.Three neonates with glucose-6-phosphate dehydrogenase (G6PD) deficiency are described. All three patients suffered an episode of massive acute haemolysis, in the absence of blood group incompatibilities, infection, or ingestion of oxidising agents known to trigger haemolysis. One patient died, but the other two survived after an exchange transfusion. This highlights that G6PD deficiency in the neonatal period may present with severe anaemia in association with hyperbilirubinaemia.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
2/6. Sideroblastic anaemia during fusidic acid treatment.OBJECTIVES AND methods: To describe cases of fusidic acid-associated sideroblastic anaemia from the French pharmacovigilance database. RESULTS: Six cases of sideroblastic anaemia associated with oral fusidic acid treatment were retrieved. Four females and two males (mean age 65.3 yr) developed severe anaemia (mean haemoglobin level: 6.9 g/dL) within 32-190 d (mean: 81 d) of treatment. bone marrow aspirates showed dyserythropoiesis and ringed sideroblasts in all patients. Four patients required repeated blood transfusions. After fusidic acid discontinuation in five patients, complete recovery was obtained. In one patient, rechallenge with fusidic acid resulted in recurrence of anaemia that resolved after definitive discontinuation of the drug. CONCLUSION: Our data indicate that fusidic acid should be added to the list of drugs that can cause sideroblastic anaemia.- - - - - - - - - - ranking = 9keywords = anaemia (Clic here for more details about this article) |
3/6. Rh iso-immunisation with syndrome of hepatocellular damage. An unusual presentation with review of literature.Rh iso-immunisation is prevalent in many underdeveloped countries. Severe haemolytic anaemia with or without hydrops fetalis, dangerous level of haemolytic unconjugated bilirubin and imminent bilirubin encephalopathy are the hallmarks of haemolytic disease of the newborn. The investigative protocols and efficient management of this entity are adequately described in literature. An unusual manifestation of this disease with severe hepatocellular damage and conjugated hyperbilirubinemia was noticed in a neonate. The literature has been reviewed and the specific symptom complex has been attributed to 'syndrome of hepatocellular damage', which is a rare accompaniment of haemolytic disease of the newborn. Attempts have been made to describe the syndrome in detail, with latest reference regarding complete workup and management. Few useful tips for prevention of the disease in a community background have been suggested.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
4/6. Glucuronyl transferase deficiency and mild hereditary spherocytosis: effect of splenectomy.In a 6-year-old girl an association of hereditary spherocytosis and a defect in hepatic bilirubin metabolism has been found. The patient suffered from mild compensated haemolytic anaemia and excessive hyperbilirubinaemia (maximum concentration 581 mumol/l), the serum activity of liver enzymes was slightly increased. Examination of the erythrocyte membrane proteins revealed a deficiency of the major membrane skeletal protein, spectrin (about 75% of normal) which is probably the basic genetic defect of hereditary spherocytosis. Examination of the patient's family revealed a recessive mode of inheritance. The concentration of bilirubin conjugates in the patient's serum was decreased due to a reduced UDP-glucuronyl transferase activity found in homogenates of liver tissue. Histological liver examination showed an intrahepatic cholestasis, which is a secondary and reversible alteration resulting from severe hyperbilirubinaemia. After splenectomy, normalization of the increased haemolysis and hepatic dysfunction was observed. The excessive hyperbilirubinaemia can be explained by the association of an increased bilirubin load due to haemolytic anaemia and the diminished hepatic conjugation of bilirubin.- - - - - - - - - - ranking = 2keywords = anaemia (Clic here for more details about this article) |
5/6. Acute viral hepatitis, intravascular haemolysis, severe hyperbilirubinaemia and renal failure in glucose-6-phosphate dehydrogenase deficient patients.Five patients with acute viral hepatitis developed severe intrasvascular haemolysis and unusually high levels of serum bilirubin (427 to 1368 mumol/l). All 5 had high fever, marked anaemia, reticulocytosis and neutrophilic leucocytosis. Three of them developed acute renal failure, which was of non-oliguric type in 2. The clinical course was protracted, but complete recovery occurred in 4 patients between 4 to 10 weeks. One patient with hepatic coma and oliguric renal failure died. Deficiency of the enzyme G-6-PD was confirmed in 4 cases. Massive haemolysis in the patients was probably induced by the administration of chloroquine and other drugs. Intravascular haemolysis should be suspected in patients with acute viral hepatitis, if they show unexplained anaemia and very high serum bilirubin levels, and measures to prevent renal failure should be instituted in such cases.- - - - - - - - - - ranking = 2keywords = anaemia (Clic here for more details about this article) |
6/6. Profile of urinary bile acids in familial intrahepatic cholestasis with Coombs' negative haemolytic anaemia.We present two male siblings with intrahepatic cholestasis and prolonged indirect hyperbilirubinaemia. Their familial intrahepatic cholestasis syndrome was characterized by Coombs' negative haemolytic anaemia, without giant cell transformation of hepatocytes and high concentrations of serum gamma-glutamyl transpeptidase and cholesterol. By gas chromatography-mass spectrometry, we detected large amounts of 1 beta-hydroxylated bile acids, especially 1 beta,3 alpha,7 alpha,12 alpha-tetrahydroxy-5 beta-cholan-24-oic acid (25.5-67.9% of total urine bile acids) in the urine during phenobarbital therapy. However, the amount of urinary 1 beta-hydroxylated bile acids gradually decreased as the disease progressed. At the end-stage, we detected large amounts of 7 alpha,12 alpha-dihydroxy-3-oxochol-4-en-24-oic acid (19.6% of total urine bile acids). The ratio of 7 alpha,12 alpha-dihydroxy-3-oxochol-4-en-24-oic acid to cholic acid in the urine was 0.8. We conclude that in infants with end-stage liver failure, the microsomal hydroxylation of bile acids is impaired and the excretion of delta 4-3-oxo bile acids is increased.- - - - - - - - - - ranking = 5keywords = anaemia (Clic here for more details about this article) |