Hirschsprung-associated inflammatory bowel disease: A multicenter study from the APSA Hirschsprung disease interest group

Background/PurposeA small number of Hirschsprung disease (HD) patients develop inflammatory bowel disease (IBD)-like symptoms after pullthrough surgery. The etiology and pathophysiology of Hirschsprung-associated IBD (HD-IBD) remains unknown. This study aims to further characterize HD-IBD, to identify potential risk factors and to evaluate response to treatment in a large group of patients.MethodsRetrospective study of patients diagnosed with IBD after pullthrough surgery between 2000 and 2021 at 17 institutions. Data regarding clinical presentation and course of HD and IBD were reviewed. Effectiveness of medical therapy for IBD was recorded using a Likert scale.ResultsThere were 55 patients (78% male). 50% (n = 28) had long segment disease. Hirschsprung-associated enterocolitis (HAEC) was reported in 68% (n = 36). Ten patients (18%) had Trisomy 21. IBD was diagnosed after age 5 in 63% (n = 34). IBD presentation consisted of colonic or small bowel inflammation resembling IBD in 69% (n = 38), unexplained or persistent fistula in 18% (n = 10) and unexplained HAEC >5 years old or unresponsive to standard treatment in 13% (n = 7). Biological agents were the most effective (80%) medications. A third of patients required a surgical procedure for IBD.ConclusionMore than half of the patients were diagnosed with HD-IBD after 5 years old. Long segment disease, HAEC after pull through operation and trisomy 21 may represent risk factors for this condition. Investigation for possible IBD should be considered in children with unexplained fistulae, HAEC beyond the age of 5 or unresponsive to standard therapy, and symptoms suggestive of IBD. Biological agents were the most effective medical treatment.Level of EvidenceLevel 4     

死亡结构域相关蛋白及其在宫颈病变中的研究进展

宫颈癌对妇女健康构成严重威胁,人乳头瘤病毒感染与宫颈病变及宫颈癌的发生密切相关。关于宫颈癌发生发展的机制仍在研究中。近年研究发现一种多功能核蛋白,即死亡结构域相关蛋白(death domain associated protein,Daxx),其与细胞内蛋白或病毒蛋白相互作用,参与调节细胞凋亡、转录调控、抗病毒等细胞活动,在不同途径中发挥不同的生理或病理作用。通过对Daxx功能及其作用机制的研究有助于进一步阐明宫颈癌发生发展的机制,有助于发现新的预防和治疗方法。综述Daxx的一般特性和研究现况及其在宫颈病变的研究进展。     

Bacteroides forsythus ATCC43037菌体蛋白与人类唾液酸性富脯蛋白的相互作用

目的：探讨福赛类杆菌与人类唾液富脯蛋白相互作用的蛋白分子。方法：Western—blot方法。将人工合成唾液富脯蛋白用生物素标记，福赛类杆菌全菌蛋白凝胶电泳，半干转移至纤维膜上，观察二者的相互作用。结果：富脯蛋白能与分子量为85KD、65KD、60KD、以及49KD的福赛类杆菌蛋白发生结合。结论：福赛类杆菌存在与人类唾液富脯蛋白相互结合的粘附素。     

MR T2加权成像显示胆囊壁增厚点状高信号的意义

目的研究病理组织学证实的胆囊腺肌瘤病、慢性胆囊炎和管壁增厚型胆囊腺癌在MRL加权成像（T2WI）显示病变胆囊壁点状高信号的特征。方法38例患者（胆囊腺肌瘤病16例，慢性胆囊炎13例，管壁增厚型胆囊腺癌9例），每例均进行了MR常规T1WI、常规T2WI和3mm薄层T2WI及MR胰胆管成像（MRCP）。所有患者均接受了胆囊切除手术。2名高年资放射科医生共同分析不同序列MRI，观察胆囊壁增厚及T2WI显示病变胆囊壁点状高信号的不同表现，将观察结果与病理组织学检查所见对照。结果所有患者的胆囊壁均明显增厚，厚度范围5～15mm，平均9mm。T2WI显示病变胆囊壁存在各种各样的点状高信号，在胆囊腺肌瘤病，点状高信号分布于整个增厚的胆囊壁，且数量较多（5～15个／cm^2），较大（直径2～7mm），边界清楚，呈现中等至明显高信号；在慢性胆囊炎，点状高信号主要位于增厚胆囊壁的黏膜侧，数量较少（3～5个／cm^2），较小（直径2～4mm），边界清楚，呈中等至明显高信号；在管壁增厚型胆囊腺癌，点状高信号边界欠清，呈现稍高信号，其数量和大小差异较大，分布范围取决于癌组织浸润胆囊壁的深度。结论胆囊腺肌瘤病、慢性胆囊炎和管壁增厚型胆囊腺癌在T2WI呈现的点状高信号有一定差别，正确识别这些点状高信号的特征有助于鉴别诊断良恶性胆囊壁增厚。     

上海市闵行区性病门诊本地和外来病人的比较研究

目的分析和比较上海市闵行区性病门诊本地与外来病人的流行病学特征。方法对2001~2004年上海市闵行区3家设有标准化性病门诊的公立医院进行门诊病历的回顾性分析。结果外来人口约占全部性病门诊病人的50%,其中女性病例占18.2%,未婚者占23.8%,均高于本地同类人员的比例;外来病人年龄偏低,多重感染者比例较高;男女外来病人与本地病人的首诊原因和症状排位相同,均以淋病感染为主;外来病人随访率(81.2%)较本地病人低(86.0%)。结论性病门诊本地和外来病人在人口学特征、诊疗行为上存在差异,防制措施应有所不同和侧重。     

甲状腺~(99)Tc~mO_4~-显像冷结节的临床诊断价值

目的　探讨甲状腺99TcmO-4 显像冷结节对不同性质甲状腺疾病的诊断价值。方法　采用静脉注射99TcmO-4 185MBq ,2 0min后甲状腺前位静态显像 ,采集总计数 1× 10 6。显像显示甲状腺结节局部放射性分布缺损或低于正常甲状腺组织则定性为冷结节。结果　 34 2例甲状腺99TcmO-4 显像发现冷结节 85例 ,发生率为2 4.9% ,85例冷结节中 ,37例 (4 3.5 % )为甲状腺瘤 ,15例为甲状腺癌 (17.6 % ) ,2 0例 (2 3.5 % )为结节性甲状腺肿 ,8例 (9.4% )为甲状腺炎 ,4例 (4 .7% )为单纯性甲状腺囊肿 ,1例 (1.3% )为甲状腺外肿物。结论　冷结节在甲状腺99Tcm O-4 显像时较为常见 ,且不同性质甲状腺疾病的冷结节及其周围组织有一定的特征性表现 ,因此99TcmO-4显像冷结节对甲状腺腺瘤、甲状腺癌、甲状腺囊肿、甲状腺炎等常见疾病具有较高的临床价值。     

阜新矿区1990～1995年传染病发病趋势

依据阜新矿区１９９０～１９９５年传染病发病情况的调查资料，对此５年间该地区常见传染病发病趋势进行了分析。结果表明，由于增大了预防力度，各种常见传染病发病率基本呈下降趋势，百分构成顺位居后。     

Hypertension in Jordanian children: a retrospective analysis of 70 cases

Seventy patients, aged 1–20 years, were seen at Jordan University Hospital with high blood pressure (BP) over a 3-year period. BP values ranged from 140 to 230 mmHg for systolic pressure and from 90 to 130 mmHg for diastolic pressure. Essential hypertension was seen in only 6 patients (8.6%); secondary hypertension (n=64 or 91.4%) was due to renal parenchymal diseases (RPD) in 46 patients (65.7%), reno-vascular lesions in 8 (11.4%), renal transplantation in 5 (7.2%), teenage pregnancy in 4 (5.7%), and phaeochromocytoma in 1 patient (1.4%). The aetiologies of RPD were as follows: end-stage renal disease requiring dialysis in 14 patients, acute glomerulonephritis in 14, idiopathic nephrotic syndrome in 10, chronic renal insufficiency in 5, and polycystic kidney in 3 patients. Surgical cure of hypertension was achieved in 5 of the children with reno-vascular lesions and in the patient with phaeochromocytoma.