克拉屈滨治疗经典型毛细胞白血病致间质性肺炎一例报道并文献复习

毛细胞白血病（HCL）是一种以脾大、全血细胞减少、骨髓组织增生等为临床表现的罕见慢性B淋巴细胞增殖性疾病。克拉屈滨作为其一线药物，对HCL的完全缓解率很高。本文报道了2017-09-04绍兴市人民医院收治的1例经典型HCL患者在克拉屈滨治疗期间，出现严重粒细胞缺乏伴高热，合并全身皮疹及间质性肺炎，经大剂量激素等治疗后好转的诊疗过程。并结合文献回顾了HCL的诊断及治疗方法，简述了克拉屈滨治疗HCL致间质性肺炎这一少见并发症的诊疗思路，以提高临床医师对该毒副作用的认识，为临床工作提供帮助。     

涎腺导管内癌的病理诊断及新进展

涎腺导管内癌(intraductal carcinoma,IDC)是一种主要发生在腮腺,在导管内或囊内生长为主的罕见肿瘤,但也可伴浸润性生长,生物学行为相对惰性。具有独特的形态学、免疫表型及分子遗传学特征。主要包括4种亚型:闰管型、顶浆分泌型、闰管-顶浆分泌混合型和嗜酸型。闰管型多数存在NCOA4-RET融合,个别存在STRN-ALK融合;混合型存在TRIM27-RET融合,顶浆分泌型具有PIK3CA和HRAS突变或TP53缺失;嗜酸型具有TRIM33-RET融合或BRAF V600E突变。目前多数观点认为与涎腺导管癌不同,即使存在浸润,仍具有良好的预后,罕见复发和淋巴结转移,无远处转移。因此精准诊断对于临床治疗的选择极其重要。     

Clinical analysis of peritoneal dialysis in the treatment of rapidly progressive glomerulonephritis

Objective To observe the clinical characteristics and prognosis of patients with rapidly progressive glomerulonephritis (RPGN) caused by lupus nephritis, antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis, or primary glomerulonephritis who were treated with peritoneal dialysis (PD) and then withdrew PD because of renal recovery. Methods Data of the above patients were retrospectively analyzed. The patients were diagnosed as RPGN and received PD therapy in Kidney Disease Center, the First Affiliated Hospital, College of Medicine, Zhejiang University from February 2009 to August 2018. The patients were divided into early withdrawal group (PD time≤183 days, n=24) and late withdrawal group (PD time＞183 day, n=24). The differences of clinical characteristics between the two groups were compared. The cumulative incidence of adverse events in both groups was analyzed using Kaplan-Meier curves. Cox proportional hazards model was used to analyze the risk factors influencing the prognosis of patients. Results Forty-eight RPGN patients were included. The median time of maintaining PD was 178(76, 378) days. Compared with the late withdrawal group, the patients in early withdrawal group had lower levels of urine volume, serum albumin and parathyroid hormone, and lower rates of gross hematuria and hypertension at the beginning of PD, and received higher rates of methylprednisolone impulse, combined immunosuppressive agents, and hemodialysis or continuous renal replacement therapy (all P＜0.05). At the time of PD withdrawal, the levels of serum creatinine, serum calcium, serum albumin and parathyroid hormone in the early withdrawal group were significantly lower than those in the late withdrawal group (all P＜0.05). The Kaplan-Meier curves showed that there was no significant difference in the cumulative survival of patients in both groups (log-rank test χ2=3.485, P=0.062). Cox regression analysis revealed serum creatinine≥209 μmol/L at the time of PD withdrawal was an independent risk factor for poor prognosis (HR=5.253，95%CI 1.757-15.702, P=0.003). Conclusions PD can be used for RPGN patients caused by lupus nephritis, ANCA-associated vasculitis and primary nephritis. Serum creatinine≥209 μmol/L at the time of PD withdrawal is an independent risk factor for poor prognosis.     

A study on various symptoms of chronic hepatitis treated with radix astragali and its regulative effect to levels of serum hormone

Sixty-seven cases of chronic hepatitis were treated with Radix Astragali. After treatment (2-month course), the clinical improvement rate in 38 cases of the Stagnation of the Liver-Qi and Deficiency of the Spleen type was 92.1%, and in 26 cases of the Deficiency of Liver Yin and Kidney Yin type was 88.5%, more effective than in the control group (P<0.05). The regulative effect to the levels of serum hormone was observed in the patients of the Stagnation of the Liver-Qi and Deficiency of the Spleen type treated with this medicine. The results showed that the levels of serum triiodothyronine, estradiol (female) and testosterone (male) were increased after treatment (1.40±1.38 ng/dl, 129.30±1.23 pg/ml and 496.24±1.47 ng/dl). Pre-treatment levels were 1.22±1.49 ng/dl, 104.60±1.45 pg/ml and 398. 17±1.55 ng/dl respectively (P<0.05); however, the level of serum prolactin (2.75±4.46 ng/ml) was lower after treatment than before treatment (3.20±3.82 ng/ml,P<0.05). No obvious changes were observed in the levels of serum follicle stimulating hormone, luteinizing hormone, thyroid stimulating hormone, thyroxine, triiodothyronine uptake ratio and cortisol after treatment.     

Immunohistochemical analysis of the vimentin filaments in Sertoli cells is a powerful tool for the prediction of spermatogenic dysfunction

The close interaction between male germ cells and Sertoli cells, a type of somatic cell found in the seminiferous tubules of mammalian testis, is essential for the normal progression of spermatogenesis in mammals. Vimentin is an intermediate filament protein that primarily provides mechanical support, preserves cell shape, and maintains the nuclear position, and it is often used as a marker to identify Sertoli cells. Vimentin is known to be involved in many diseases and aging processes; however, how vimentin is related to spermatogenic dysfunction and the associated functional changes is still unclear. In a previous study, we reported that vitamin E deficiency affected the testes, epididymis, and spermatozoa of mice, accelerating the progression of senescence. In this study, we focused on the Sertoli cell marker vimentin and explored the relationship between the cytoskeletal system of Sertoli cells and spermatogenic dysfunction using testis tissue sections that caused male reproductive dysfunction with vitamin E deficiency. The immunohistochemical analysis showed that the proportion of the vimentin-positive area in seminiferous tubule cross-sections was significantly increased in testis tissue sections of the vitamin E-deficient group compared with the proportion in the control group. The histological analysis of testis tissue sections from the vitamin E-deficient group showed that vimentin-positive Sertoli cells were greatly extended from the basement membrane, along with an increased abundance of vimentin. These findings suggest that vimentin may be a potential indicator for detecting spermatogenic dysfunction.     

未分化胚胎细胞转录因子1与恶性肿瘤关系的研究现状

未分化胚胎细胞转录因子1(UTF1)是干细胞相关基因之一,是参与胚胎干细胞分化的重要转录因子,能促进干细胞的自我更新及分化,调控胚胎细胞和生殖细胞的增殖分化。目前,对UTF1的研究大多在干细胞和生殖细胞中,而关于UTF1与肿瘤关系的研究成果较少。越来越多的证据显示,干细胞相关基因的异常表达与肿瘤发生、转移、复发及耐药性等密切相关。其转录和表观遗传胚胎程序可以在癌细胞中重新激活,使癌细胞具有干细胞表型,并参与肿瘤的发生、发展。为获得更好的疗效,部分学者长期致力于寻找各种有效的肿瘤标志物,其中UTF1在恶性肿瘤的诊断、治疗及预后方面具有重要的潜在应用价值。     

Patch-based local learning method for cerebral blood flow quantification with arterial spin-labeling MRI

Arterial spin-labeling (ASL) perfusion MRI is a non-invasive method for quantifying cerebral blood flow (CBF). Standard ASL CBF calibration mainly relies on pair-wise subtraction of the spin-labeled images and controls images at each voxel separately, ignoring the abundant spatial correlations in ASL data. To address this issue, we previously proposed a multivariate support vector machine (SVM) learning-based algorithm for ASL CBF quantification (SVMASLQ). But the original SVMASLQ was designed to do CBF quantification for all image voxels simultaneously, which is not ideal for considering local signal and noise variations. To fix this problem, we here in this paper extended SVMASLQ into a patch-wise method by using a patch-wise classification kernel. At each voxel, an image patch centered at that voxel was extracted from both the control images and labeled images, which was then input into SVMASLQ to find the corresponding patch of the surrogate perfusion map using a non-linear SVM classifier. Those patches were eventually combined into the final perfusion map. Method evaluations were performed using ASL data from 30 young healthy subjects. The results showed that the patch-wise SVMASLQ increased perfusion map SNR by 6.6% compared to the non-patch-wise SVMASLQ.     

Index-finger pointing in generalized tonic–clonic seizures

ObjectiveMost patients with localization-related epilepsy (LRE) and genetic generalized epilepsy (GGE) are classified based on semiology and video-EEG, but both features occasionally fail to provide a definitive diagnosis. Several reliable lateralizing signs have been described, although hand and finger posturing has received little attention. We sought to investigate the frequency of index-finger pointing (IFP) during generalized motor convulsions as a lateralizing semiology in LRE.MethodsWe retrospectively analyzed 98 videos of generalized convulsions in 64 consecutive patients who were admitted for diagnostic video-EEG (vEEG). Demographics were recorded, and IFP ipsilateral, contralateral, and bilateral to vEEG ictal correlate was compared between LRE, GGE, and nonepileptic attacks (NEAs). The angle of IFP was measured to quantify the mean degree of IFP in “pointers” versus “nonpointers”. Statistical analysis was completed using JMP 9.0.ResultsIndex-finger pointing was more common in epileptic GTC seizures than in convulsive NEAs (83.6% vs 12.0%; p < 0.001) and was more common in LRE compared with GGE (96% vs 56.6%; p ≤ 0.001). The frequency of contralateral, ipsilateral, or bilateral IFP did not differ between LRE and GGE. The average angle at the MCP joint in “pointers” was 35.8° (SD 22.0°) and in “nonpointers” 3.0° (SD 7.2°).SignificanceThis is the first study to examine hand and finger postures as a clinical sign to help classify epilepsy type. The presence of IFP was more common in patients with LRE than in patients with GGE and very rarely occurred in NEA. Index-finger pointing and other hand semiologies are potentially quantifiable localizing signs to aid in the characterization of patients with GTC seizures.