Clinical and biochemical effects of parachlorophenylalanine in a patient with the carcinoid syndrome

The clinical and biochemical features of a patient with flushing and severe diarrhoea due to the carcinoid syndrome are described. The patient had a paradoxical response to the tryptophan hydroxylase inhibitor parachlorophenylalanine with complete abolition of flushing and no effect on the diarrhoea. Treatment with this drug was limited by adverse effects.     

平喘固本汤合补肺汤治疗COPD患者的临床观察

目的 观察平喘固本汤合补肺汤对COPD肺肾气虚证患者临床疗效和免疫功能的影响.方法 采用前瞻、随机、对照研究方法,选择健康人群45例设为正常对照组.将COPD患者90例设为病例组,将其随机分为COPD治疗组与COPD对照组,各45例;对照组给予西医常规治疗3个月;治疗组除西医常规治疗外,加服平喘固本汤合补肺汤,每日一剂,常规水煎服,分2次口服,3个月为1个疗程.入选病例分别在治疗前后检测肺功能,测1秒钟用力呼气量预测值(FEV1pred)、1秒钟用力呼气量(FEV1)/用力肺活量(FVC);分别在治疗前后检测IgA、IgG、IgM、CD3、CD4、CD8及IL-13.所得数据用SPSS 13.0统计软件进行分析,数据用((x)±s)来表示,采用单因素方差分析及t检验,P ＜0.05为差异有统计学意义.结果 治疗组治疗后FEV1、FEV1/FVC、IL-13、IgA、IgG、IgM、CD3、CD4、CD8、CD4/CD8指标较治疗前均有改善(P＜0.05,或P＜0.01),与健康人群组、对照组比较亦有改善(P ＜0.05,或P＜0.01).结论 平喘固本汤合补肺汤治疗COPD肺肾气虚证患者疗效确切,能有效改善COPD肺肾气虚证患者的肺功能及免疫功能,降低炎症指标水平,具有一定的临床意义和价值.     

Periodic synoviosis (intermittent hydrarthrosis) with observations and studies on a patient

More than 200 instances of periodic synoviosis have been recorded. It is related to periodic oedema (hereditary angioedema). Various autonomic disturbances accompany episodes. Confusion with rheumatoid arthritis persists. Theoretically an inherent rhythm or feedback mechanism operates as the cause.     

阻塞性睡眠呼吸暂停低通气综合征患者代谢紊乱的临床观察

目的探讨阻塞性睡眠呼吸暂停低通气综合征（obstructive sleep apnea-hypopnea syndrome,OSAHS）与胰岛素抵抗的相互关系以及引起代谢综合征的作用机制。方法随机选择186例住院的原发性高血压病患者,根据夜间多导联睡眠图检测结果分成4组,对照组35例,轻度OSAHS组61例,中度OSAHS组52例,重度OSAHS组38例。对所有患者测量身高、体重、腰围、血压、血脂、空腹血糖、空腹胰岛素、血尿酸和肌酐,比较并分析4组间各变量。结果102例（67．6％）OSAHS患者患有代谢综合征,重度OSAHS组中33例（86．8％）符合代谢综合征诊断,与对照组比较差异有统计学意义（P〈0．01）。重度OSAHS组的体重指数、腰围、血尿酸／肌酐比值、舒张压均明显高于对照组和轻、中度OSAHS组（P〈0．01）。重度OSAHS组的收缩压明显高于轻、中度OSAHS组（P〈0．01）。重度OSAHS组的胰岛素抵抗明显高于对照组和轻度OSAHS组（P〈0．01）。多元回归分析表明,胰岛素抵抗与体重指数呈正相关,与最低血氧饱和度呈负相关;最低血氧饱和度与腰围和血尿酸／肌酐比值呈负相关。结论低氧血症和肥胖促进胰岛素抵抗的发生和发展。高血压病合并OSAHS患者容易出现代谢综合征,尤其是在重度OSAHS患者。     

Clinical Observations on Treatment of Infantile Cholestatic Hepatitis Syndrome by Rhubarb

Infantilecholestatichepatitissyndrome(ICHS)involvesseriousjaundice,reductionofbileeffluence,withthefeealcolourgettinglightorevenkaolin-likeinadditiontohepatomegaliaorchangeofliverquality,andthelevelofalanineaminotransferase(ALT)andglutamyltranspeptidase(γ-GT)raisedup.Treatmentofthesyndromeisquitedifficult.Inthepast,wehadtreatedICHSwithYinzhihuang(茵栀黄)injectionandgotsatisfactoryeff..t(1),onlyitneededintravenousadministration.DuringtheperiodofJunetoOctober1994,44caseswithICHSweretrea…     

早产儿应用脂肪乳的临床观察

目的 :探讨脂肪乳对早产儿生长发育的影响。方法 :对 87例早产儿从生后第 2天起给缓慢静脉输注脂肪乳 ,隔日 1次 ,共 3～ 5次 ,每 3天测体重 1次。同时将既往 72例作为对照组 ,观察两组体重下降率、住院天数和出现并发症的例数。结果 :治疗组与对照组比较患儿体重下降率少、持续时间短、住院天数缩短、并发症减少 ,两组比较有统计学差异 ( P<0 .0 5 )。结论 :早产儿短期、间断缓慢静脉输注脂肪乳能缩短病程 ,提高生存质量     

Membranous glomerulonephritis and the nephrotic syndrome in a patient with Landry-Guillain-Barré-Strohl syndrome

Membranous glomerulonephritis and the nephrotic syndrome concurrent with the Miller-Fisher variant of the Landry-Guillain-Barré-Strohl syndrome (LGBS), acute post-infective polyneuritis, is reported in a 49-year-old man. The onset of heavy proteinuria coincided with the development of the neurological disturbance. While immunosuppressive therapy appeared to hasten improvement in the neurological disease, no such improvement occurred in the glomerulopathy.     

Hallervorden-Spatz综合征患者一例的临床特征及泛酸激酶2基因突变检测

目的 研究中国Hallervorden-Spatz综合征患者的临床特征并对患者以及家系成员进行泛酸激酶2(PANK2)基因的突变检测.方法 对1例Hallervorden-Spatz综合征患者的临床特征进行分析,应用聚合酶链反应(PCR)、DNA直接测序技术检测患者和其父母及50名健康人PANK2基因序列.结果 患者主要临床表现为进行性加重的四肢不自主运动和构音不清,头部MRI T_2加权和FLAIR成像表现双侧苍白球对称性低信号,在低信号区的前内侧出现高信号,即"虎眼征";检测出PANK2基因一个新的复合杂合突变:位于第1外显子115位的G→T和第二外显子803位的A→G导致所编码的氨基酸发生改变(分别为E39X和D268G).父亲为G115T杂合突变,母亲为A803G杂合突变.结论 中国Hallervorden-Spatz综合征患者存在PANK2基因突变,PANK2基因A803G突变可能为中国大陆Hallervorden-Spatz综合征患者的突变热点.     

Aortic reoperation in a patient with Loeys-Dietz syndrome

Loeys-Dietz syndrome （LDS, OMIM #609192） is a recently recognized connective tissue disorder with clinical characteristics similar to Marfan syndrome. Aggressive aortic pathologies in LDS makes timely diagnosis and surgical intervention crucial compared to other genetic syndromes. We report a young man with LDS confirmed by genetic testing who twice underwent surgical operations to repair aortic aneurysm.