慢性胃病脾虚患者胃窦粘膜胃泌素细胞和分泌生长抑素细胞的变化及其意义

目的：观察以慢性胃病为主的脾虚患者胃窦粘膜分泌胃泌家(Gas)细胞(G细胞)、生长抑素(SS)细胞(D细胞)与脾虚证发生的关系。方法：将84例脾虚患者分为脾胃虚寒组、脾虚夹热组、胃阴不足组、脾胃湿热组、肝胃不和组，应用免疫组化技术标记胃窦粘膜G、D细胞，并定量分析。结果：以慢性胃病为主的脾虚证患者G、D细胞数均减少，D细胞面积缩小，G/D细胞数和细胞面积比值均增高(P＜0．05)。结论：G、D细胞的变化可能是慢性胃病脾虚证胃肠功能障碍的一个重要病理机制。     

新疆喀什1499名中小学生舌象调查分析

对西域古城喀什1499名中小学生做舌象调查,舌体适中约2/3,胖大略高于1/3,齿痕合约1/4,舌裂15.8%;舌质以淡红为主(86.9％),舌苔以白薄苔为常见(94.2％,82.5％)。资料分析表明舌体、齿痕舌与体重相关;同时舌体、花剥舌与性别有关;舌胖、舌裂与花剥苔还随年龄增大而增多。但关系最密切的还是舌象与民族有关,即维吾尔族舌裂、绛红舌质、花剥苔、齿痕舌均高于汉族,而后者的厚苔、黄苔和舌边尖瘀点则高于维吾尔族。     

云南红豆杉中四个新紫杉烷类四环二萜成分的分离和鉴定

从云南红豆杉(Taxus yunnanensis ChengetL.K.Fu)树皮的二氯甲烷部分又分离得到四个新紫杉烷类四环二萜化合物,分别命名为taxayuntin A,B,C和D。应用¹HNMR,¹³CNMR,¹H-¹HCOSY,¹³C-¹HCOSY及¹³C-¹HCOLOC等方法进行结构测定,证明它们都有5/7/6三环稠合的基本骨架,并在C₄和C₅上连有环氧丙烷。     

银鹿口服液的制备与应用

目的：通过对银鹿口服液生产工艺、质量标准的探讨。以确保临床应用效果。方法：生产工艺采取水提醇沉,含量测定采用分光光讴法,随机对照法进行临床观察。结果：在３０天为一疗程中,其总有效率为９１．４９％。结论：因此本品是一种安全有效的预防、治疗冠心病心绞痛的药物。     

云南红豆杉化学成分的研究IV：二个新紫杉烷类三环二萜成分的分离和鉴定

在继续从云南红豆杉（ＴａｓｕｓｙｕｎｎａｎｅｎｓｉｓＣｈｅｎｇｅｔＬ．Ｋ．Ｆｕ）树皮中寻找抗肿瘤活性成分的研究中，又分离出７个紫杉烷类二萜化合物，经光谱解析（ＭＳ，１ＨＮＭＲ，１Ｈ－１ＨＣＯＳＹ，１３ＣＮＭＲ，１３Ｃ－１ＨＣＯＳＹａｎｄ１３Ｃ－１ＨＣＯＬＯＣ），鉴定其中５个为已知化合物：７－ｅｐｉ－１０－ｄｅａｃｅｔｙｌｔａｘｏｌ（Ｉ），７－ｅｐｉ－１０－ｄｅａｃｅｔｙｌｃｅｐｈａｌｏｍａｎｎｉｎｅ（ＩＩ），１０－ｄｅａｃｅｔｙｌｔａｘｏｌ（ＩＩＩ），１０－ｄｅａｃｅｔｙｌｃｅｐｈａｌｏｍａｎｎｉｎｅ（ＩＶ）和１０－ｄｅａｃｅｔｙｌｂａｃｃａｔｉｎＩＩＩ（ＶＩＩ）；２个新化合物是１３（２′，３′－ｄｉｈｙｄｒｏｘｙ－３′－ｐｈｅｎｙｌ）－ｐｒｏｐｉｏｎｙｌｂａｃｃａｔｉｎ，ＩＩＩ（Ｖ）和９－ｄｅｏｘｏ－９α－ｈｙｄｒｏｘｙｔａｘｏｌ（ＶＩ），分别命名为云南红豆杉醇（ｙｕｎｎａｎｘｏｌ）和云南红豆杉胺（ｙｕｎｎａｎｘａｍｉｎｅ）。     

斑蝥素毒性及其药（毒）动力学研究

目的 研究斑蝥素对小鼠肝、肾毒性及其在小鼠体内的动态行为。方法 用小鼠半数致死量（LD50）及生化指标的变化来表征斑蝥素的毒性,用小鼠急性死亡率法测定斑螫素药物动力学参数。结果和结论 斑蝥素对小鼠肝、肾有明显的毒性作用,该药在小鼠体内动态变化符合一级动力学,呈二室开放模型,其表观药动学参数为:A=10.1mg/kg;α=1.56 h-1;t1/2α=0.44h;B=1.19mg/kg;β=0.123h-1;t1/2=5.63h;K21=0.274h-1;K10=0.700h-1;K12=0.709h-1;CL=0.071kg/kg·h-1;AUC=16.15 mg·h/kg;Vc=0.102kg/kg;Vp=0.264 kg/kg。     

Detection of circulating tumour DNA after neoadjuvant chemoradiotherapy in patients with locally advanced oesophageal cancer

Active surveillance instead of standard surgery after neoadjuvant chemoradiotherapy (nCRT) has been proposed for patients with oesophageal cancer. Circulating tumour DNA (ctDNA) may be used to facilitate selection of patients for surgery. We show that detection of ctDNA after nCRT seems highly suggestive of major residual disease. Tumour biopsies and blood samples were taken before, and 6 and 12 weeks after, nCRT. Biopsies were analysed with regular targeted next-generation sequencing (NGS). Circulating cell-free DNA (cfDNA) was analysed using targeted NGS with unique molecular identifiers and digital polymerase chain reaction. cfDNA mutations matching pre-treatment biopsy mutations confirmed the presence of ctDNA. In total, 31 patients were included, of whom 24 had a biopsy mutation that was potentially detectable in cfDNA (77%). Pre-treatment ctDNA was detected in nine of 24 patients (38%), four of whom had incurable disease progression before surgery. Pre-treatment ctDNA detection had a sensitivity of 47% (95% CI 24–71) (8/17), specificity of 85% (95% CI 42–99) (6/7), positive predictive value (PPV) of 89% (95% CI 51–99) (8/9), and negative predictive value (NPV) of 40% (95% CI 17–67) (6/15) for detecting major residual disease (>10% residue in the resection specimen or progression before surgery). After nCRT, ctDNA was detected in three patients, two of whom had disease progression. Post-nCRT ctDNA detection had a sensitivity of 21% (95% CI 6–51) (3/14), specificity of 100% (95% CI 56–100) (7/7), PPV of 100% (95% CI 31–100) (3/3), and NPV of 39% (95% CI 18–64) (7/18) for detecting major residual disease. The addition of ctDNA to the current set of diagnostics did not lead to more patients being clinically identified with residual disease. These results indicate that pre-treatment and post-nCRT ctDNA detection may be useful in identifying patients at high risk of disease progression. The addition of ctDNA analysis to the current set of diagnostic modalities may not improve detection of residual disease after nCRT. © 2022 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of The Pathological Society of Great Britain and Ireland.     

Recognition and epileptology of protracted CLN3 disease

Objective

This study was undertaken to analyze phenotypic features of a cohort of patients with protracted CLN3 disease to improve recognition of the disorder.

Methods

We analyzed phenotypic data of 10 patients from six families with protracted CLN3 disease. Haplotype analysis was performed in three reportedly unrelated families.

Results

Visual impairment was the initial symptom, with onset at 5–9 years, similar to classic CLN3 disease. Mean time from onset of visual impairment to seizures was 12 years (range = 6–41 years). Various seizure types were reported, most commonly generalized tonic–clonic seizures; focal seizures were present in four patients. Progressive myoclonus epilepsy was not seen. Interictal electroencephalogram revealed mild background slowing and 2.5–3.5-Hz spontaneous generalized spike–wave discharges. Additional interictal focal epileptiform discharges were noted in some patients. Age at death for the three deceased patients was 31, 31, and 52 years. Molecular testing revealed five individuals were homozygous for c.461-280_677 + 382del966, the "common 1-kb" CLN3 deletion. The remaining individuals were compound heterozygous for various combinations of recurrent pathogenic CLN3 variants. Haplotype analysis demonstrated evidence of a common founder for the common 1-kb deletion. Dating analysis suggested the deletion arose approximately 1500 years ago and thus did not represent cryptic familial relationship in this Australian cohort.

Significance

We highlight the protracted phenotype of a disease generally associated with death in adolescence, which is a combined focal and generalized epilepsy syndrome with progressive neurological deterioration. The disorder should be suspected in an adolescent or adult patient presenting with generalized or focal seizures preceded by progressive visual loss. The common 1-kb deletion has been typically associated with classic CLN3 disease, and the protracted phenotype has not previously been reported with this genotype. This suggests that modifying genetic factors may be important in determining this somewhat milder phenotype and identification of these factors should be the subject of future research.