Patient Age Predicts Long Term Insufficient Weight Loss after Laparoscopic Sleeve Gastrectomy

Original Articl

Simple and robust methylation test for risk stratification of patients with juvenile myelomonocytic leukemia.

Juvenile myelomonocytic leukemia (JMML) is a rare myelodysplastic/myeloproliferative neoplasm that develops during infancy and early childhood. The array-based international consensus definition of DNA methylation has recently classified patients with JMML into the following 3 groups: high (HM), intermediate (IM), and low methylation (LM). To develop a simple and robust methylation clinical test, 137 patients with JMML were analyzed using the Digital Restriction Enzyme Analysis of Methylation (DREAM), which is a next-generation sequencing-based methylation analysis. Unsupervised consensus clustering of the discovery cohort (n = 99) using DREAM data identified HM (HM_DREAM; n = 35) and LM subgroups (LM_DREAM; n = 64). Of the 98 cases that could be compared with the international consensus classification, 90 HM (n = 30) and LM (n = 60) cases had 100% concordance with DREAM clustering results. Of the remaining 8 cases comprising the IM group, 4 were classified as belonging to the HM_DREAM group and 4 to the LM_DREAM group. A machine-learning classifier was successfully constructed using a support vector machine (SVM), which divided the validation cohort (n = 38) into HM (HM_SVM, n = 18) and LM (LM_SVM; n = 20) groups. Patients with the HM_SVM profile had a significantly poorer 5-year overall survival rate than those with the LM_SVM profile. In conclusion, we developed a robust methylation test using DREAM for patients with JMML. This simple and straightforward test can be easily incorporated into diagnosis to generate a methylation classification for patients so they can receive risk-adapted treatment in the context of future clinical trials

Allogeneic stem cell transplantation with reduced intensity conditioning for patients with adrenoleukodystrophy

Objective: The prognosis of adrenoleukodystrophy (ALD)with neurological involvement is generally dismal; however, allogeneic stem cell transplantation (SCT) is recognized as effective to stabilize or improve the clinical symptoms of ALD. Herein, we report the clinical outcomes of patients with ALD who consecutively underwent allogeneic stem cell transplantation with reduced intensity conditioning at our institution. Patients: Sixteen patients with ALD, who were symptomatic (n = 14) or presymptomatic (n = 2), received SCT from 2010 to 2016. The stem cell source was cord blood (n = 14), or bone marrow from a human leukocyte antigen identical sibling (n = 2). The conditioning regimen prior to transplantation was reduced intensity and consisted of fludarabine (125 mg/m2), melphalan (140 mg/m2) and low dose total body irradiation (TBI) of 4Gy (n = 15) or 3Gy (n = 1). Results: Primary engraftment was obtained in 11 patients, and 4 of the 5 patients who lost the primary graft received a second cord blood transplantation and were engrafted. Five years overall and event-free survival were 90.9% and 61.1% respectively, with a median of 45 months (range 16–91). Loes score stabilized or improved by 18 months after transplantation except for patients with internal capsule involvement. Conclusion: Allogeneic SCT with reduced intensity conditioning for patients with ALD was safely performed without major transplant-related complications even in symptomatic patients and neurological symptoms were stabilized after SCT in patients without internal capsule involvement. Keywords: Adrenoleukodystrophy, Allogeneic stem cell transplantation, Loes score, Very long chain fatty aci

The 2006 radio outbursts of a microquasar cygnus X-3 : Observations and data

We present the results of the multi-frequency observations of radio outburst of the microquasar Cyg X-3 in February and March 2006 with the Nobeyama 45-m telescope, the Nobeyama Millimeter Array, and the Yamaguchi 32-m telescope. Since the prediction of a flare by RATAN-600, the source has been monitored from Jan 27 (UT) with these radio telescopes. At the eighteenth day after the quench of the activity, successive flares exceeding 1 Jy were observed successfully. The time scale of the variability in the active phase is presumably shorter in higher frequency bands. 1 We also present the result of a follow-up VLBI observation at 8.4 GHz with the Japanese VLBI Network (JVN) 2.6 days after the first rise. The VLBI image exhibits a single core with a size of < 8 mas (80 AU). The observed image was almost stable, although the core showed rapid variation in flux density. No jet structure was seen at a sensitivity of Tb = 7.5 × 105 K

【角化症・炎症性角化症】 Sjoegren症候群と慢性甲状腺炎を合併した膿疱性乾癬

雑誌掲載版64歳女.特に誘因なく体幹,四肢に痒みを伴う紅色皮疹が出現した.痛風でアロプリノールを内服中であったためアロプリノールを中止したが,皮疹は拡大した.体幹,四肢に癒合傾向を伴い,一部環状を呈する暗赤色から淡紅色の紅斑が多発していた.紅斑の辺縁部には膿疱が集簇し,一部膿海を形成し,中心部から膜様に落屑していた.また,白血球増加,リンパ球減少,CRP高値,低蛋白血症,低カルシウム血症,抗核抗体陽性,抗SS-A抗体陽性,f-T3低値,抗サイログロブリン抗体陽性がみられた.HLAを検索したところ,A24,DR4,CW1,B35を検出した.膿疱性乾癬にSjoegren症候群,慢性甲状腺炎の合併が明らかとなり,皮疹はプレドニゾロン,エトレチナートの服用で改善し,Sjoegren症候群は塩酸セビメリン水和物内服を開始し,慢性甲状腺炎は経過観察とし