Successful unrelated donor bone marrow transplantation for Shwachman-Diamond syndrome with leukemia.

Shwachman-Diamond syndrome (SDS) is a rare congenital disorder featuring exocrine pancreatic insufficiency, growth retardation, and bone marrow dysfunction. Reports suggest that nearly 25% of all cases are complicated with leukemia. Although stem cell transplantation is the sole option for these patients, successful results are rarely obtained.

Polymorphisms of heme oxygenase-1 and bilirubin UDP-glucuronosyltransferase genes are not associated with Kawasaki disease susceptibility.

Kawasaki disease (KD) is a systemic vasculitis and occurs among Japanese children at a high incidence. Serum bilirubin and heme oxygenase-1 (HO-1) expression are known to play a significant role in the protection of vascular endothelial cells. Japanese have unique polymorphic distribution patterns of (TA)7 or G71R of the bilirubin UDP-glucuronosyltransferase (B-UGT) gene and of (GT)n repeats of the HO-1 gene.

Haemophagocytic lymphohistiocytosis following measles vaccination.

Unlabelled: A 19-month-old girl developed haemophagocytic lymphohistiocytosis following a measles vaccination. She developed persistent high fever 1 week after vaccination, and then showed pancytopenia, liver dysfunction and hepatosplenomegaly with marked haemophagocytosis. Based on the clinical and laboratory findings, she was diagnosed as having haemophagocytic lymphohistiocytosis probably due to measles vaccination.

[Acute pancreatitis following hematopoietic stem cell transplantation: prevalence and cause of pancreatic amylasemia].

To clarify the frequency and cause of acute pancreatitis following hematopoietic stem cell transplantation (SCT), we examined retrospectively 57 patients who underwent hematopoietic SCT in our institute from 1984 to 2000. Twelve (21%) of the patients showed an elevated level of serum pancreatic amylase following SCT. However, only 3 patients were clinically diagnosed as having acute pancreatitis.