Hypothalamic-pituitary-gonadal function in two infants with Smith-Lemli-Opitz syndrome

We report on the hypothalamic-pituitary-gonadal function in 2 male infants with the Smith-Lemli-Opitz (SLO or RSH) syndrome. Both infants had abnormal external genitalia. Basal and LHRH stimulated plasma gonadotropins were normal for age (1 month). Plasma testosterone, androstenedione, and dehydroepiandrosterone sulfate were normal for age and sex. Some forms of congenital adrenal hyperplasia (17,20-desmolase deficiency, 17α-hydroxylase deficiency, and 3β-hydroxysteroid dehydrogenase deficiency) were ruled out by hormonal studies. The endocrinological findings indicate a normal hypothalamic-pituitary-gonadal function and a normal adrenal steroid biosynthesis in these 2 patients. A partial androgen receptor defect causing the genital malformations seems possible in one patient. Whether 5α-reductase deficiency is the cause of the male pseudohermaphroditism in SLO syndrome remains the subject of future studies. © 1992 Wiley-Liss, Inc.     

覆醛氧化淀粉联合硝苯啶治疗老年慢性肾功能衰竭264例

对老年慢性肾功能衰竭264例患者(男148例,女116例;年龄66±7a)用硝苯啶10mg联合覆醛氧化淀粉5-10mg,tid,po。另设相似88例患者(男48例,女40例;年龄66±7a)单服覆醛氧化淀粉与之对照,用法同前。2组均连服16wk。结果:前者有效216例(81.8％),后者有效49例(56％),组间比较P<0.01。2组副作用相似,均可坚持服药。     

Liver transplantation for Budd-Chiari syndrome: A retrospective study

A retrospective study was performed on 11 patients who underwent orthotopic liver transplantation for Budd-Chiari syndrome (BCS), 3 of whom had fulminant type BCS and 8, chronic type BCS. Both the 3- and 5-year actuarial survival rates were 64%, after one patient with fulminant, and three with chronic disease died of sepsis or multiple organ failure following transplantation. Anticoagulation therapy in the early postoperative period was tailored to each individual patient. Most of the patients received heparin for several days and were then converted to Coumadin therapy, although some were not given heparin in the immediate postoperative period but were instead commenced on oral Coumadin after the prothrombin time had recovered to wihtin the normal range. All the long-term survivors had received Coumadin therapy and there was no recurrence of BCS and no early thrombotic or hemorrhagic event. One patient developed late thrombosis of the portal vein despite having received apparently adequate Coumadin therapy. It was thus concluded that liver transplantation is an effective therapy for both fulminant and chronic BCS, and that immediate postoperative heparinization is not mandatory for all patients.     

乳腺囊性增生病癌变过程中部分因素变化的意义

检测乳腺囊性增生病（ＦＣＤ）经不典型增生到癌变部分因素的变化。结果提示：从因明显ＦＣＤ症状活检至癌变为２～１０年；从Ⅱ级以上不典型增生到临床癌变需２～７年；癌变率为３．１％。ＦＣＤ患者存在性激素分泌调控失常，血浆雌激素和催乳素含量增加，导致上皮细胞增生。乳腺一般性增生细胞的ＤＮＡ含量和超微结构与正常乳腺上皮细胞相似；无肿瘤相关抗原及异常基因产物表达。而发生在一般性增生基础上的不典型增生则呈现细胞基因物质ＤＮＡ含量增加，部分为超４Ｃ的多倍体细胞；同时出现细胞膜和细胞核超微结构异常；雌激素受体含量增加，对性激素的依赖性和敏感性增强；部分不典型增生细胞出现胚胎性肿瘤相关抗原和异常基因产物表达。随不典型增生程度加重至乳腺癌，上述诸因素的变化趋势具有明显规律性。提示ＦＣＤ上皮细胞从一般性增生经不典型增生至乳腺癌为细胞生物学连续逐渐变化的过程。部分不典型增生细胞中具有癌倾向的细胞生物学行为异常和表型变化与乳腺癌发生密切相关。细胞核ＤＮＡ含量等异常变化及程度可作为乳腺癌前病变发展程度的客观标志     

骨髓腔静脉造影在特发性股骨头坏死诊断中的应用

本文介绍17例（34髋）特发性股骨头坏死、4例（4髋）正常人骨髓腔静脉造影所见。发现25髋有异常改变,并经病理证实。作者认为:①骨干返流;②2支或2支以上静脉不显影;③多条静脉明显变细;④干骺端瘀积是股骨头坏死的造影表现,前二者是可靠的诊断依据。造影结果提示髓腔静脉造影是一种安全而敏感的诊断技术。     

少见肺部原发性恶性肿瘤七例报告

报告我院１９８０年－１９９３年诊治的７例少见肺部原发性恶性肿瘤，包括来源于间叶组织的２种肉瘤，来源于间胚叶软组织肿瘤，来源于气管粘膜腺体上皮的肿瘤，１例由原始生细胞向胚外组织分化而成的内胚窦瘤，全部病例经病理活组织检查证实，重点讨论诊断的体会。     

安定东莨菪碱在产程中的联合应用（附176例分析）

产程延长传统处理方法是应用催产素静脉点滴，根据安定能选择性使宫颈肌纤维松驰；东莨菪碱能扩张血管，改善微循环，解除平滑肌痉挛，使宫颈水肿消失或减轻，我院妇产科自1988年5月至1989年4月，对176例在产程延长或宫颈水肿的产妇，联合应用安定、东莨菪碱，取得良好的效果，本文介绍了应用方法，并对二药的药理作用进行了讨论。     

Personality variables as moderators between hassles and subjective indications of distress

The research was designed to investigate the moderating effect of some personality traits on subjective distress caused by daily hassles. The traits were internal locus of control, repression, ego strength, and barrier (as studied and defined by S. Fisher). The last two variables were negatively correlated both with the somatic and emotional distress indications and with the frequency of hassles reportED; internal locus of control showed an inverse relationship only with frequency of hassles. The hypothesis is formulated that ego strength and barrier are personality factors influencing not only the outcomes of coping (ie the stress response), but also event appraisal.     

Irritable bowel syndrome: The role of psychosocial factors

The irritable bowel syndrome (IBS) is a very common disorder of intestinal function characterized by variable symptoms of abdominal pain, diarrhea, constipation and bloating. The spectrum of severity ranges from mild symptoms, not worthy of clinical attention, to intense and continuous symptoms associated with impaired health-related quality of life and high health care use. Psychosocial disturbance is commonly found among patients with irritable bowel, but until recently, its precise role in the disorder was uncertain. The data now suggest that irritable bowel is not a psychiatric disorder per se, but psychosocial factors including life stress, psychiatric comorbidity and abnormal illness behavior, among others, influence how the illness is experienced and acted upon. Patients with mild symptoms usually respond to education, reassurance, dietary modification and, when needed, antimotility agents. However, patients with more severe symptoms, who usually have greater psychosocial disturbance, will also require behavioral interventions, and possibly psychopharmacological agents. In sum, a graduated, multicomponent plan of care that includes dietary, behavioral and pharmacological treatments is recommended.     

The gap between legal rules and practice in advertising non-registered pharmaceutical products : A new method of analysis

The market of non-registered pharmaceutical products is growing fast in number and overall costs, not only in the Netherlands, but also in other European countries. These products often give the impression that the consumer may expect 'an effect as from a drug'. Legally, there is a clear distinction between 'drugs' and 'commodities' in the Netherlands; the question is whether legislation and practice concur. In an investigation we analysed texts of advertisements for non-registered pharmaceutical products published in a popular magazine. A method was developed, based on the legal definition of a drug and jurisprudence, to determine in a qualitative and quantitative way the application of medicinal claims. It transpired that in 65% of the analysed advertisements explicit or implicit claims were made. These products should therefore be subject to drugs legislation. Thus, in the Netherlands there is a gap between legislation and practice in advertising non-registered pharmaceutical products.