Therefore, we believe that the article published by Connolly and coworkers addresses an important aspect of the liver microenvironment regarding the role of DCs in fibrosis progression. Additional studies are warranted to clarify the functional and potentially clinically relevant contribution of DCs for the progression or regression of liver fibrosis. “
“Niemann-Pick disease, types A and B, are lipid storage disorders caused by complete or partial deficiency of a lysosomal enzyme, acid sphingomyelinase. The disorders are inherited in an autosomal recessive pattern and result

in the accumulation of sphingomyelin in various organs including the spleen, liver, lungs, bone marrow and brain. The infantile GSK1120212 nmr variant (type A) usually presents within the first year of life with failure to thrive, hepatomegaly and neurological symptoms, sometimes resulting in liver failure with ascites and jaundice. Most babies die at 2–3 years of age. In Niemann-Pick type B disease, the diagnosis is usually made early in childhood because of failure to thrive and the development learn more of hepatosplenomegaly. Patients then develop progressive neurological symptoms and may have deteriorating pulmonary

function because of foam cell infiltrates. Most patients die in childhood. An important clue to the diagnosis of Niemann-Pick disease, types A and B, is the presence of large foamy macrophages in the bone marrow or liver. In most patients, the diagnosis is confirmed by genetic tests for mutations in the SMPD1 gene or by the demonstration of low acid sphingomyelinase tuclazepam activity in peripheral blood white cells or in cultured fibroblasts. At present, there is no specific treatment for Niemann-Pick disease. The patient illustrated below was 15-month-old female who presented with a 4-month history of hepatosplenomegaly and failure to thrive. She was the product

of a full-term normal pregnancy but her parents were consanguineous and a previous baby had died in the neonatal period. The parents described anorexia and recurrent pneumonia but no vomiting or diarrhea. The baby had a weight and height below the 5th percentile for age. Physical examination revealed marked hepatosplenomegaly but no ascites. Liver enzymes were abnormal with elevation of aspartate aminotransferase (311 u/l), alanine aminotransferase (251 u/l), gamma-glutamyltransferase (77 u/l) and alkaline phosphatase (724 u/l). She had a normal complete blood count, coagulation studies and serum albumin. A computed tomography scan of the chest revealed typical reticulonodular infiltrates in the upper lobes of both lungs (Figure 1). A percutaneous liver biopsy showed lipid-laden foam cells (Figure 2). The diagnosis of Niemann-Pick disease was confirmed by the demonstration of low acid sphingomyelinase activity in peripheral blood white cells.