Clinical features of cytopenia with bone marrow hypoplasia in children: an analysis of 100 cases / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To summarize the clinical features of cytopenia with bone marrow hypoplasia in 100 children and to investigate an effective treatment regimen for myelodysplastic syndrome (MDS) in children.</p><p><b>METHODS</b>A retrospective analysis was performed on the clinical data of 100 children non-randomly selected from Japan and China who were diagnosed with cytopenia with bone marrow hypoplasia between 2006 and 2011. The data of patients from China were subjected to prognostic analysis.</p><p><b>RESULTS</b>There was no significant difference in the proportion of MDS cases and acquired aplastic anemia (AA) cases between the Japanese and Chinese children. Of the 100 patients, there were 29 cases of acquired AA, 58 cases of refractory cytopenia of childhood (RCC) and 13 cases of refractory cytopenia with multilineage dysplasia (RCMD). There were significant differences in reticulocyte absolute value in peripheral blood and degree of bone marrow proliferation among the three patient groups (P<0.05). The patients from China were followed up for 16-70 months (median, 41 months). After being treated with cyclosporine (CsA) combined with stanozolol, the patients with AA had response rates of 25% and 75%, the patients with RCC had response rates of 47.1% and 82.4%, and the patients with RCMD had response rates of 60% and 60% respectively at 3 and 6 months after treatment.</p><p><b>CONCLUSIONS</b>There are significant differences in reticulocyte absolute value in peripheral blood and degree of bone marrow proliferation among patients with RCC, RCMD and acquired AA. CsA combined with stanozolol has a good therapeutic efficacy in the treatment of acquired AA and hypoplastic MDS in children, but studies of more cases and a longer follow-up duration are needed.</p>

Improvement of NASH with two-year treatment with oral polyenephosphatidylcholine / Journal of Rural Medicine

A 46-year-old female patient with non-alcoholic steatohepatitis (NASH) was administered nateglinide, an insulin secretagogue, for 7 months, and then polyenephosphatidylcholine, an anti-oxidant medication, in accordance with the two-step hypothesis of NASH, with insulin resistance as the initial pathogenesis and oxidative stress as the second. HOMA-R, an index of insulin resistance, improved, and hepatic marker levels improved markedly by four weeks after initiation of polyenephosphatidylcholine. The beneficial effects of treatment continued over the 24 months of the study. A liver biopsy evaluated using Brunt's criteria showed improvement from stage 2 to stage 0 after 9 months. These findings suggest the therapeutic efficacy of step by step treatment of NASH in accordance with the two-stage hypothesis.

A case report of an extended aortic arch anastomosis for an infant with the persistent fifth aortic arch associated with atresia of the fourth aortic arch / 日本心臓血管外科学会雑誌

The persistent fifth aortic arch is rare vascular anomaly. To our knowledge, this is the 24th reported case of the persistent fifth aortic arch. This patient was a 31 days old male infant and had the persistent fifth aortic arch associated with atresia of the fourth aortic arch, patent ductus arteriosus, a double-outlet right ventricle, and a mesocardia. He underwent a fifth aortic arch division and an extended aortic arch anastomosis with a division of ductus arteriosus. There was no blood pressure gradient between upper and lower limbs after the repair. However, no weaning from a cardiopulmonary bypass after the subsequent radical operation for double-outlet right ventricle caused his death.