Sexual precocity: sex incidence and aetiology

The aetiology of 197 girls and 16 boys presenting with sexual precocity was reviewed. Ninety one girls and four boys had central precocious puberty (M:F 23:1); a cause was identified in all the boys but in only six girls. All boys with precocious puberty need detailed investigation; in girls investigation should be based on clinical findings, particularly the consonance of puberty.     

显微注射后家兔转基因受精卵的体外培养和移植成活率的观察研究

目的：研究家兔受精卵经显微注射转基因过程的耐受性，建立转基因动物技术平台。方法：(1)家兔超数排卵诱导；(2)对2000个家兔受精卵进行显微注射技术操作；(3)对1280个受精卵进行显微注射后体外培养观察；(4)216个注射后受精卵向13只受体母兔输卵管进行移植．结果：(1)经显微注射后受精卵进行体外培养有68％的受精卵能够发育至桑椹胚和囊胚；(2)目前有7只受体母兔经输卵管转基因受精卵移植分别产下3，4，5，2，6，13，7只仔兔。结论：(1)受精卵能够在体外经受显微注射转基因过程的损伤，但暴露于体外的操作时间应尽可能的缩短；(2)显微注射操作技术的熟练程度是实验成功的关键。     

同种异体原位心脏移植术后心电图分析

目的：探讨同种异体原位心脏移植术后患者的心电图改变特点，方法：对3例心脏移植的患者术后分别进行体表12导联心电图跟踪观察，主要观察心率、心律、ST-T演变情况，结果：共同表现的ST段抬高系心肌再灌注后损伤性改变，不完全性右束支传导阻滞与手术中机械刺激或损伤有关，由于去神经作用心率变化较小，应用标准术式患者易出现心律失常，应用双腔静脉原位心脏移植术后心律失常发生率较少，结论：心脏移植术前、术后心电图追踪观察有助于指导治疗和评估预后。     

尿微量蛋白与老年2型糖尿病合并缺血性心脏病关系的研究

目的探讨在老年2型糖尿病合并缺血性心脏病患者中尿微量蛋白在加重缺血性心脏病进展方面的作用。方法将66例老年2型糖尿病合并缺血性心脏病患者按24h尿微量蛋白排泄量分为:尿微量蛋白正常组及尿微量蛋白阳性组,分别对血脂、空腹血糖、血清胰岛素、胰岛素敏感指数、纤维蛋白原、颈动脉内-中膜厚度、颈总动脉粥样硬化等级积分、硬化斑块指数、心脏彩超室间隔厚度及射血分数等加重缺血性心脏病进展等指标进行比较分析。结果66例老年2型糖尿病合并缺血性心脏病患者尿微量蛋白阳性组高密度脂蛋白、载脂蛋白A、胰岛素敏感指数、年龄、纤维蛋白原、硬化斑块指数、颈总动脉粥样硬化等级积分、室间隔厚度及射血分数显著高于尿微量蛋白阴性组(P<0.05,P<0.01)。结论尿微量蛋白的出现能够加速糖尿病合并缺血性心脏病的发展进程,它的出现预示着缺血性心脏病的预后不良。     

Detection of carbamyl phosphate synthetase 1 deficiency using duodenal biopsy samples

The activity of urea cycle enzymes was assayed in duodenal biopsy specimens obtained from a female infant who presented with neonatal hyperammonaemia. All enzyme levels were normal except N-acetyl glutamate-dependent carbamyl phosphate synthetase 1 (CPS1) which was half the mean activity in normal control specimens. A similar deficiency of CPS1 was also shown in duodenal specimens from the patient's mother who became slightly symptomatic after relatively high protein meals and during pregnancy, and had spontaneously modified her diet to one with protein restriction. The patient is growing normally on a dietary regimen similar to that spontaneously adopted by her mother. Urea cycle enzyme activity in the duodenal biopsy material from the controls was similar to that found in the normal human liver and appears to have distinct advantages as a means of assaying for urea cycle defects in patients with hyperammonaemia and their relatives.     

Flexion compromises ventilation with the laryngeal tube suction II in children

Background: There are insufficient data as to the influence of the head and neck flexion, extension, and rotation on the ventilation with laryngeal tube suction II^® (LTS II). The purpose of this study was to investigate the influence of the head and neck position on oropharyngeal sealing pressure (primary outcome) and ventilation score (secondary outcome) during ventilation with the LTS II in children. Methods: We studied 33 children scheduled for elective surgery. Oropharyngeal sealing pressure and ventilation score were measured with the head and neck in a neutral position, flexed, extended and rotated to the right. The ventilation score was scored from 0 to 3 based on three items (no leakage with an airway pressure of 15 cmH₂O, bilateral chest excursion, and a square wave capnogram; each item scored 0 or 1 point). Peak inspiratory pressure (PIP) at a tidal volume of 10 ml·kg^?1 and fiberoptic laryngeal views were also assessed in each position. Results: Although the sealing pressure was lower in the extended position [22 (8) cmH₂O] than that in the neutral position [25 (7) cmH₂O], there was no significant leakage during ventilation with a tidal volume of 10 ml·kg^?1. In the neutral, extended and rotated positions, the median ventilation scores were better (3 point respectively) than that with the head and neck flexed (1 point). PIP was decreased with the head and neck extended or rotated but was significantly increased in flexion position. During fibreoptic examination, the vocal cords were more easily seen in extension and right rotation, compared with the neutral position and flexion. Conclusions: Although oropharyngeal sealing pressure is decreased with the head and neck extended, effective ventilation with LTS II can be performed like in the neutral position or the rotated position. While the sealing pressure is maintained with the head and neck flexed, flexion compromises the ventilation with LTS II in children.     

人膀胱移行细胞癌各种透明质酸合成酶基因亚型的表达及意义

目的:研究各种透明质酸合成酶亚型(HASs)mRNA在人膀胱移行细胞癌中的表达及其潜在的临床价值.方法:运用半定量RT-PCR方法,对145例不同恶性度的人膀胱移行细胞癌、22例正常人膀胱黏膜组织标本和4种人BTCC细胞系的HAS亚型mRNA的表达情况进行研究.结果:PCR 23循环后,正常膀胱黏膜组织中7例扩增出微弱的HASl mRNA条带(28%,7/25),其余无任何条带扩增出来;所有膀胱移行细胞癌组织标本和细胞系有一种以上的HAS亚型mRNA被扩增出来.不同膀胱癌组织、细胞系表达的HASs mRNA的种类及表达程度各不相同,但随着肿瘤恶性程度的增高,其表达的各种HASs mRNA的种类趋向相同.每个膀胱癌组织、细胞系均有一种HASs mRNA优势表达,但其类型及优势表达程度互不相同.结论:人膀胱移行细胞癌出现了HAS亚型mRNA的异常高表达,可能与肿瘤的发生、发展有密切关系,是BTCC生物治疗潜在的靶点;同时进一步证实了尿透明质酸作为膀胱癌的"标志物"的可靠性.     

膀胱癌术后腹壁切口种植转移的治疗与预后

目的　总结膀胱部分切除术后腹壁切口种植转移患者的诊治效果。　方法　 1985～1999年共收治膀胱癌术后腹壁切口种植转移患者 10例。男 9例 ,女 1例。年龄 39～ 6 8岁。移行细胞癌 6例 ,均为低分化G3 肿瘤 ;移行细胞癌合并鳞癌和腺癌各 1例 ,鳞癌和粘液腺癌各 1例。腹壁转移灶 1.0～ 6 .9cm× 1.4～ 11.5cm。　结果　采用膀胱全切加腹壁转移灶切除 2例 ,经尿道膀胱肿瘤电切术 (TURBT)加腹壁转移灶切除 1例 ,尿流改道加放疗和 (或 )化疗 2例 ,腹壁转移灶切除加放疗和 (或 )化疗 3例 ,单纯放疗和 (或 )化疗 2例。 10例中死亡 7例 ,其中 6例生存 4～ 14个月 ,1例生存4 1个月 ;3例存活者随访 5～ 9个月 ,未见复发。　结论　膀胱癌术后腹壁切口种植转移癌一般分化差 ,或合并其他癌。膀胱全切加肿物切除 ,辅助放化疗虽然能延长患者生存期 ,但预后很差     

Protein Losing Enteropathy in Severe Atopic Dermatitis in an Exclusively Breast-Fed Infant

Abstract:   We first report a case of protein losing enteropathy in severe atopic dermatitis in an exclusively breast-fed 5-month-old infant. Protein losing enteropathy was confirmed by fecal α₁-antitrypsin clearance test and imaged successfully by ^99mTc-human serum albumin scintigraphy. The present case highlights that protein losing enteropathy in severe infantile atopic dermatitis is being a topic of concern and also an issue even in exclusive breast feeding patients.