Increased glycosylphosphatidylinositol-anchored protein-deficient granulocytes define a benign subset of bone marrow failures in patients with trisomy 8

Trisomy 8 (+8), one of the most common chromosomal abnormalities found in patients with myelodysplastic syndromes (MDS), is occasionally seen in patients with otherwise typical aplastic anemia (AA). Although some studies have indicated that the presence of +8 is associated with the immune pathophysiology of bone marrow (BM) failure, its pathophysiology may be heterogeneous. We studied 53 patients (22 with AA and 31 with low-risk MDS) with +8 for the presence of increased glycosylphosphatidylinositol-anchored protein-deficient (GPI-AP-) cells, their response to immunosuppressive therapy (IST), and their prognosis. A significant increase in the percentage of GPI-AP- cells was found in 14 (26%) of the 53 patients. Of the 26 patients who received IST, including nine with increased GPI-AP- cells and 17 without increased GPI-AP- cells, 14 (88% with increased GPI-AP- cells and 41% without increased GPI-AP- cells) improved. The overall and event-free survival rates of the +8 patients with and without increased GPI-AP- cells at 5 yr were 100% and 100% and 59% and 57%, respectively. Examining the peripheral blood for the presence of increased GPI-AP- cells may thus be helpful for choosing the optimal treatment for +8 patients with AA or low-risk MDS. © 2014 John Wiley & Sons A/S

The Impact of PNPLA3 rs738409 Genetic Polymorphism and Weight Gain ≥10 kg after Age 20 on Non-Alcoholic Fatty Liver Disease in Non-Obese Japanese Individuals.

Non-alcoholic fatty liver disease (NAFLD) in non-obese individuals is inadequately elucidated. We aim to investigate the impact of known genetic polymorphisms on NAFLD and the interaction between genetic risks and weight gain on NAFLD in obese and non-obese Japanese individuals. A total of 1164 participants who received health checkups were included. Participants with excessive alcohol consumption, with viral hepatitis or other inappropriate cases were excluded. Fatty liver was diagnosed by ultrasonography. Participants with a body mass index (BMI) of <18.5 kg/m2, 18.5-22.9 kg/m2, 23.0-24.9 kg/m2 and ≥25 kg/m2 were classified underweight, normal weight, overweight and obese, respectively. Self-administered questionnaire for lifestyle was assessed and a total of 8 previously reported genetic polymorphisms were chosen and examined. In all, 824 subjects were enrolled. The overall prevalence of NAFLD was 33.0%: 0% in underweight, 15.3% in normal weight, 41.1% in overweight and 71.7% in obese individuals. The prevalence of NAFLD is more affected by the G allele of patatin-like phospholipase domain-containing protein 3 (PNPLA3) rs738409 in normal weight (odds ratio (OR) 3.52; 95%-CI: 1.42-8.71; P = 0.0063) and in overweight individuals (OR 2.60; 95%-CI: 1.14-5.91; P = 0.0225) than in obese individuals (not significant). Moreover, the G allele of PNPLA3 rs738409 and weight gain ≥10 kg after age 20 had a joint effect on the risk of NAFLD in the normal weight (OR 12.00; 95% CI: 3.71-38.79; P = 3.3×10-5) and the overweight individuals (OR 13.40; 95% CI: 2.92-61.36; P = 0.0008). The G allele of PNPLA3 rs738409 is a prominent risk factor for NAFLD and the interaction between the PNPLA3 rs738409 and weight gain ≥10 kg after age 20 plays a crucial role in the pathogenesis of NAFLD, especially in non-obese Japanese individuals

Successful treatment of Trichosporon fungemia in a patient with refractory acute myeloid leukemia using voriconazole combined with liposomal amphotericin B

Trichosporon fungemia is a rare and fatal fungal infection that occurs in patients with prolonged neutropenia associated with hematologic malignancies. A 21-year-old male developed Trichosporon fungemia during remission induction therapy for acute myeloid leukemia (AML). Although two courses of induction therapy failed to induce a remission of AML, combination therapy with voriconazole and liposomal amphotericin B (L-AmB) followed by monocyte colony-stimulating factor ameliorated the Trichosporon fungemia and enabled the patient to receive reduced-intensity bone marrow transplantation (BMT) from his human leukocyte antigen-A one-locus mismatched mother. The patient achieved a durable remission after BMT without exacerbation of Trichosporon fungemia. The combination therapy with voriconazole and L-AmB may therefore be useful in controlling Trichosporon fungemia associated with prolonged neutropenia after remission induction therapy for AML

Caspases and matrix metalloproteases facilitate collective behavior of non-neural ectoderm after hindbrain neuropore closure

Background: Mammalian brain is formed through neural tube closure (NTC), wherein both ridges of opposing neural folds are fused in the midline and remodeled in the roof plate of the neural tube and overlying non-neural ectodermal layer. Apoptosis is widely observed from the beginning of NTC at the neural ridges and is crucial for the proper progression of NTC, but its role after the closure remains less clear. Results: Here, we conducted live-imaging analysis of the mid-hindbrain neuropore (MHNP) closure and revealed unexpected collective behavior of cells surrounding the MHNP. The cells first gathered to the closing point and subsequently relocated as if they were released from the point. Inhibition of caspases or matrix metalloproteases with chemical inhibitors impaired the cell relocation. Conclusions: These lines of evidence suggest that apoptosis-mediated degradation of extracellular matrix might facilitate the final process of neuropore closure

収穫期前の降雨が夏ダイズの子実品質に及ぼす影響

The cultivation of early maturing soybean is rarely seen in southwest of Japan. A considerable reason is the problem of low seed quality caused by high temperature and humidity of summer during seed maturation. The objective of this study was to evaluate the influence of precipitation during harvest time on the seed quality in early maturing soybean cultivars. Harvested dry pods of 63 cultivars matured in different season (summer and autumn) were soaked in distilled water for 0, 2, 4 and 6 days. The seed moisture content and the deterioration of seed quality after soaking were investigated. The results were as follows. In the pods of summer harvest, the seed moisture content, and the rate of damaged seeds (contained the seeds of decay, injury and seed coat cracking, etc.) were greatly higher than those of harvested in autumn by the pods soaking treatment. The seed damage occurred dramatically when the seed moisture content was more than 50%. Moreover, the water absorption of seeds through pod shell seemed to be different among cultivars since the low seed moisture content cultivars in summer harvest had shown also low seed moisture content in autumn harvest. It is suggested that the deterioration of seed quality by precipitation during harvest time could be relieved by the selection of cultivar.西南暖地において夏ダイズの栽培が減少した理由の一つには,登熟期が高温・多湿であるため,収穫された子実の品質や種子活力が著しく低下することが挙げられる.本研究では,収穫期前の雨が莢実の吸水性および子実品質に及ぼす影響を明らかにするために,夏と秋の異なる時期に登熟した夏ダイズ63品種の完熟莢を浸水処理し,莢実の吸水程度および自然乾燥後の子実品質の劣化程度について調査した.その結果,莢浸水による莢殻と子実の吸水程度は,夏収穫のものでは秋収穫のものよりかなり大きく,自然乾燥後の子実の被害粒(変形,変色,腐敗,カビ粒など)の発生率がかなり高かった.被害粒の発生率は,子実吸水率と強い正の相関関係があり,子実吸水率が50%を超えると急激に増加した.莢殻を通しての子実の吸水は莢殻の透水性に強く依存しているが,莢殻の厚さとの間には有意な相関関係はみられなかった.また,夏収穫において莢実が吸水しにくい品種は秋収穫した場合にも吸水しにくい傾向がみられたことから,莢殻の透水性には品種間差異があり,栽培品種の選択によっては収穫期前の降雨による子実品質の低下をある程度軽減できることが示唆された

Influence of Precipitation during Harvest Time on the Seed Quality in Early Maturing Soybean

The cultivation of early maturing soybean is rarely seen in southwest of Japan. A considerable reason is the problem of low seed quality caused by high temperature and humidity of summer during seed maturation. The objective of this study was to evaluate the influence of precipitation during harvest time on the seed quality in early maturing soybean cultivars. Harvested dry pods of 63 cultivars matured in different season (summer and autumn) were soaked in distilled water for 0, 2, 4 and 6 days. The seed moisture content and the deterioration of seed quality after soaking were investigated. The results were as follows. In the pods of summer harvest, the seed moisture content, and the rate of damaged seeds (contained the seeds of decay, injury and seed coat cracking, etc.) were greatly higher than those of harvested in autumn by the pods soaking treatment. The seed damage occurred dramatically when the seed moisture content was more than 50%. Moreover, the water absorption of seeds through pod shell seemed to be different among cultivars since the low seed moisture content cultivars in summer harvest had shown also low seed moisture content in autumn harvest. It is suggested that the deterioration of seed quality by precipitation during harvest time could be relieved by the selection of cultivar.西南暖地において夏ダイズの栽培が減少した理由の一つには,登熟期が高温・多湿であるため,収穫された子実の品質や種子活力が著しく低下することが挙げられる.本研究では,収穫期前の雨が莢実の吸水性および子実品質に及ぼす影響を明らかにするために,夏と秋の異なる時期に登熟した夏ダイズ63品種の完熟莢を浸水処理し,莢実の吸水程度および自然乾燥後の子実品質の劣化程度について調査した.その結果,莢浸水による莢殻と子実の吸水程度は,夏収穫のものでは秋収穫のものよりかなり大きく,自然乾燥後の子実の被害粒(変形,変色,腐敗,カビ粒など)の発生率がかなり高かった.被害粒の発生率は,子実吸水率と強い正の相関関係があり,子実吸水率が50%を超えると急激に増加した.莢殻を通しての子実の吸水は莢殻の透水性に強く依存しているが,莢殻の厚さとの間には有意な相関関係はみられなかった.また,夏収穫において莢実が吸水しにくい品種は秋収穫した場合にも吸水しにくい傾向がみられたことから,莢殻の透水性には品種間差異があり,栽培品種の選択によっては収穫期前の降雨による子実品質の低下をある程度軽減できることが示唆された