Plasma Levels of Immunoreactive Corticotrophin in Patients with Cushing's Syndrome

Plasma levels of immunoreactive corticotrophin (A.C.T.H.) have been determined in 56 patients with Cushing''s syndrome by means of a homologous radioimmunoassay. In untreated Cushing''s disease (bilateral adrenal hyperplasia due to excessive A.C.T.H. secretion from the pituitary) plasma values ranged from 40 to 200 μμg./ml., between 8 and 10 a.m., compared with a range in normal subjects of 12 to 60 μμg./ml. Considerably raised levels, often above 2,000 μμg./ml., were found in patients with Cushing''s disease after bilateral adrenalectomy. A.C.T.H. concentrations were usually higher in patients with bilateral adrenal hyperplasia associated with ectopic A.C.T.H. production than in patients with untreated Cushing''s disease; whereas plasma A.C.T.H. was undetectable in the presence of an adrenocortical tumour. All patients with Cushing''s syndrome failed to show the normal circadian rhythm of circulating A.C.T.H. levels.     

Genetic Variants of von Willebrand's Disease

A specific antiserum against an antihaemophilic factor (AHF)-related plasma protein was raised in rabbits. A quantitative immunochemical method was used to determine the amount of this protein present in the plasma of 33 patients with haemophilia A and 70 patients with von Willebrand''s disease. The protein probably consisted of AHF residing in or complexed with the von Willebrand factor. The patients with von Willebrand''s disease were shown to fall into two separate genetic groups, one with decreased and one with normal amounts of the AHF-related protein. The patients with haemophilia A had normal amounts of the protein in their plasma.     

Attitudes of California Dermatologists Toward Worker's Compensation: Results of a Survey

A survey-questionnaire on Worker''s Compensation sent to 492 California dermatologists produced 268 replies, a recovery rate of 54 percent, representing approximately 10 percent of the practicing dermatologists in the United States. A total of 75 percent replied they treat Worker''s Compensation patients; most of the 25 percent who replied they do not gave strong reasons for refusing to assume the care of these patients. The answers to the questionnaire given by the 201 California dermatologists who treat Worker''s Compensation patients are tabulated and discussed. Although there exists considerable misunderstanding among physicians, insurance companies and employers on many aspects of Worker''s Compensation, the situation is not without hope for improvement. Better knowledge of work procedures, more availability of precise information on the ingredients of work contactants and cooperation among the interested parties should improve the quality of care for these patients in the future.     

In-vitro Inhibition of Leucocyte Migration in Crohn's Disease by a Sarcoid Spleen Suspension

A Kveim suspension has been shown to inhibit the migration of leucocytes in vitro from 12 out of 18 patients with Crohn''s disease but to have no comparable effect on leucocytes from patients with ulcerative colitis or from a group of patients with other diseases. These findings provide further evidence of cross-reactivity or of a possible aetiological link between Crohn''s disease and sarcoidosis and suggest a further immunological distinction between Crohn''s disease and ulcerative colitis.     

Raynaud's phenomenon as side effect of beta-blockers in hypertension.

A series of 102 hypertensive patients were assessed for the frequency of symptoms of Raynaud''s phenomenon and absent peripheral pulses. Out of 21 patients receiving methyldopa alone only one had cold hands and feet whereas among patients on beta-blockers the incidence was 50%. The frequency of both symptoms and absent pulses was highest in patients taking propranolol compared with those taking atenolol or oxprenolol. Patients without a foot pulse were much more likely to have cold hands. A change from propranolol to oxprenolol in some symptomatic patients resulted in improvement. In two patients the skin temperature fell after an 80-mg dose of propranolol. The mechanism by which beta-blockers induce Raynaud''s phenomenon is still not clear.     

Five cases of cyclical Cushing's syndrome.

Reported cases of cyclical Cushing''s syndrome are rare. Of 14 successive patients with Cushing''s syndrome nine collected sequential urine samples for the estimation of cortisol:creatinine ratio. Five had cyclical Cushing''s syndrome while two had considerable variation in urinary cortisol excretion without a cyclical pattern being established. Two of the five patients with a cyclical syndrome had paradoxical responses to dexamethasone. In only one patient with a cyclical pattern did the cortisol:creatinine ratio fall after treatment with bromocriptine or cyproheptadine, or both. The high incidence of the cyclical form of Cushing''s syndrome has important clinical implications. A high index of suspicion of the syndrome is required in patients with symptoms or signs of Cushing''s syndrome but with normal cortisol values, in patients with fluctuating cortisol values, and in patients with anomalous responses to dexamethasone. Because of possible variations in steroidogenesis the results of drug studies in Cushing''s syndrome must be interpreted cautiously.     

Residual activity of α-galactosidase A in Fabry's disease

The α-galactosidase A activity from fibroblasts of five Fabry patients and five controls has been separated from α-galactosidase B through small DEAE-cellulose columns and in some experiments by treatment of the fibroblast extracts with Sepharose coupled to anti-α-galactosidase B antibodies. By these independent methods, it has been shown that there is a residual α-galactosidase A in Fabry's disease, which is immunologically similar to the α-galactosidase A from the controls. The α-galactosidase A from all of the patients and controls has the same apparent K_m value for the synthetic substrate 4-methylumbelliferyl-α-galactoside. Four out of five patients have a thermostable α-galactosidase A, while the fifth has a thermolabile enzyme like that from the controls. The amount of immunologically active α-galactosidase A seems to be decreased in the patients tested.     

Unexplained left ventricular hypertrophy: consider a diagnosis of Fabry's disease

Lysosomal storage disorders are a group of disorders characterised by the deficiency of a specific lysosomal hydrolase. These diseases are rare, with only a few hundred patients in the Netherlands. Fabry''s disease, an X-linked lysosomal storage disorder, is caused by a deficiency of the lysosomal enzyme α-galactosidase A which results in, among other things, left ventricular hypertrophy, renal failure and cerebrovascular events. Patients with Fabry''s disease, especially males, have a decreased life expectancy. Recent studies have shown that Fabry''s disease may be much more common among patients with left ventricular hypertrophy (LVH) than previously thought. Up to 7% of male patients with left ventricular hypertrophy and up to 12% of female patients with unexplained LVH were found to suffer from Fabry''s disease. Thus, Fabry''s disease should be considered in patients with unexplained LVH. This case report summarises the main features of the disease. In addition recent developments concerning prevalence, diagnosis and the current available treatments are discussed and an algorithm on who and how to screen for Fabry''s disease is presented.     

Porphyria cutanea tarda: comparison of cases precipitated by alcohol and estrogens.

A group of seven patients with porphyria cutanea tarda (PCT) precipitated by excessive alcohol consumption (A) was compared with a group of nine patients with PCT precipitated by estrogen therapy (B). Comparison was based on clinical signs, biochemical and morphologic evidence of liver disease, results of serum iron studies and response to therapy. Group A patients were men of mean age 57 years; group B patients were women of mean age 39 years who had been taking estrogen orally, either for contraception (in combination with progesterone) or as replacement therapy. Clinical signs were essentially the same in the two groups. Some patients in both groups had biochemical and morphologic evidence of liver disease. Group A patients had elevated values for serum iron and total iron-binding capacity, whereas patients in group B had normal or low values. Cessation of estrogen therapy of less than a year''s duration brought about a spontaneous clinical and biochemical remission in group B patients. Otherwise, phlebotomy seemed to be the therapy of choice in both groups.     

A Pilot Study of Medicare Patients' Experiences and Attitudes

There has been widespread interest in the effect the Medicare law has had on the level of physicians'' charges. This subject and other aspects of the present Medicare method of physician reimbursement is currently under study by Congress and the Department of Health, Education and Welfare. A group of Medicare patients recently seen and treated by the author were queried as to their experiences in paying for physicians'' services under Medicare. A 64 percent response rate and the substance of the replies to a questionnaire suggests that this is a feasible mechanism for eliciting the attitudes and problems in a group of patients in the Medicare program.     

初始沙盘在临床心理中的诊断鉴别作用

目的:探讨患者初始沙盘在临床心理中的诊断鉴别作用。方法:选择4名在临床中被误诊的患者。其中A例患者诊断一般心理问题;B、C、D例患者诊断为精神分裂症;使用沙盘对该4例患者重新进行临床分析与诊断。结果:根据患者初始沙盘中是否给玩具赋予特殊意义、使用玩具的多少、玩具之间是否有联系、使用栅栏情况、沙盘主题以及患者对沙盘的解释等对4名被误诊的患者进行重新诊断;A例患者重新诊断应为精神分裂症;B例患者应为强迫症(强迫思维);C例患者应为边缘型人格障碍;D例患者应为人际关系障碍。结论:在临床心理工作中,患者的初始沙盘可以起到高效、准确的诊断鉴别作用。     

Effect of an oral serotonin antagonist,ketanserin, on plasma ACTH concentrations in Nelson's syndrome.

A study was performed to see whether ketanserin, a serotonin antagonist, would reduce the raised concentrations of adrenocorticotrophic hormone (ACTH) in patients with Nelson''s syndrome. Six patients who had undergone bilateral adrenalectomy for Cushing''s disease and who had Nelson''s syndrome were given ketanserin 40 mg twice daily and placebo, for at least two months each, in a double blind crossover study. Ketanserin had no effect on ACTH concentrations. In healthy people serotonin seems to have a stimulatory role in the regulation of ACTH secretion, and the effect of ketanserin in reducing the ACTH response to hypoglycaemia suggested that it might prove useful in Nelson''s syndrome. These results show that it is not indicated in these patients.     

Clinical Trial of Lincomycin Hydrochloride in Reiter's Disease

A double-blind trial comparing lincomycin hydrochloride (Mycivin) and placebo in 22 patients with Reiter''s disease showed no significant difference in clinical or laboratory findings between the two groups. It is concluded that lincomycin hydrochloride is no more effective than placebo in the treatment of Reiter''s disease.     

Patterns of contraceptive pill taking in an inner city practice.

A total of 161 patients completed a questionnaire about their pattern of taking the oral contraceptive pill. Only 28% (45) of patients were taking the pill according to the manufacturer''s instructions, and in the event of the pill being missed only 26% of patients would use a sheath. A tenth of the patients believed that amenorrhoea always indicated pregnancy, but 35% believed that amenorrhoea was harmful to the body. This group did not differ in their pill taking from the remaining 65% of patients.     

Diet and Crohn's disease: characteristics of the pre-illness diet.

Thirty newly diagnosed patients with Crohn''s disease were interviewed about their habitual, pre-illness diet and compared with 30 healthy controls, matched for age, sex, social class, and marital status. The patients ate substantially more refined sugar, slightly less dietary fibre, and considerably less raw fruit and vegetables than the controls. A diet high in refined sugar and low in raw fruit and vegetables precedes and may favour the development of Crohn''s disease.     

帕金森病伴抑郁患者DBS 术后帕罗西汀治疗疗效观察

目的:研究丘脑底核(STN)脑深部电刺激(DBS)治疗帕金森病(PD)合并抑郁障碍术后服用帕罗西汀治疗的疗效。方法:将38例合并抑郁障碍的PD患者随机分为三组,行丘脑底核脑深部电极植入术,术后空白对照组不服用任何抗抑郁药物,药物治疗组服用帕罗西汀每日一次,每次20mg,安慰剂组服用安慰剂。术前一周,术后1个月、2个月和3个月进行随访和临床评价。结果:抑郁患者术后抑郁障碍症状如焦虑、绝望和激越症状也有不同程度好转,应用安慰剂后,患者术后抑郁障碍程度好转程度大于空白对照组(P<0.05),而应用帕罗西汀后术后3个月汉密尔顿抑郁量表评分(HAMD)下降程度显著低于空白对照组及安慰剂组(P<0.05)。结论:表明STN-DBS术后PD患者的抑郁症状有所改善,辅助抗抑郁药物治疗效果更佳。     

Postal survey of patients' satisfaction with a general practice out of hours cooperative.

OBJECTIVE: To assess patients'' satisfaction with out of hours care by a general practice cooperative compared with that by a deputising service. DESIGN: Postal questionnaire survey. SETTING: A general practice cooperative in London and a deputising service operating in an overlapping area. SUBJECTS: Weighted samples of patients receiving telephone advice, a home visit, or attending a primary care centre after contacting either service in an eight week period. MAIN OUTCOME MEASURES: Patients'' overall satisfaction and scores for specific aspects of satisfaction. Satisfaction with telephone advice or attendance at centre compared with home visit. Relation between satisfaction and patient''s age, sex, ethnic group, car ownership, preference for consulting own doctor, and expectation of a visit. RESULTS: The overall response rate was 67% (1555/2312). There was little difference in overall satisfaction between patients contacting the cooperative or the deputising service, but patients contacting the latter were less satisfied with the explanation and advice received and the wait for a visit. There were significant differences between patients in different age and ethnic groups, with white patients and those aged over 60 years being more satisfied. Lower scores for overall satisfaction were reported by patients who received telephone advice, those who would have preferred to see their own doctor or who originally wanted a home visit, and those who waited longer for their consultation. Overall levels of patients'' satisfaction seemed to be lower than previously reported. CONCLUSIONS: There were larger differences in satisfaction between different groups of patients than between different models of organisation for out of hours care. A shift to a service based predominantly on telephone advice may lead to increased patient dissatisfaction.     

ABCA1基因多态性R219K与帕金森症和阿尔兹海默症发病率的相关性研究

目的:探讨与研究三磷酸腺苷结合盒转运体A1 (Adenosine triphosphate (ATP)-binding cassette transporter A1)基因多态性R219K与帕金森症(Parkinson disease,PD)和阿尔兹海默症(Alzheimer disease,AD)发病率的相关性。方法:选择2016年2月到2019年8月在本院门诊与住院的帕金森症患者42例作为PD组,同期选择本院门诊与住院的阿尔兹海默症患者42例作为AD组,同期选择本院门诊健康体检者84例作为对照组。调查入选者的一般资料,检测三组血液样本的ABCA1基因多态性R219K情况并进行相关性分析。结果:AD组低密度脂蛋白(low-density lipoprotein,LDL-C)、总胆固醇(total cholesterol,TC)、甘油三酯(triglyceride,TG)与尿酸(Uric acid,UA)均低于对照组,而高密度脂蛋白(high-density lipoprotein,HDL-C)、同型半胱氨酸(homocysteine,Hcy)值高于对照组(P0.05);AD组TC均低于PD组,而HDL高于PD组。PD患者HDL-C均低于对照组,而LDL、TC和TG与对照组无差异(P0.05),三组空腹血糖(Fasting blood glucose,FBG)值对比差异无统计学意义(P0.05)。PD组与AD组的ABCA1 R219K GA基因型、A等位基因频率都显著高于对照组(P0.05),PD组与AD组对比差异无统计学意义(P0.05)。在168例入选者中,直线相关分析显示ABCA1 R219K GA基因型与A等位基因与帕金森症或阿尔兹海默症发生有显著相关性(P0.05)。结论:ABCA1基因多态性R219K在帕金森症和阿尔兹海默症患者中比较常见,ABCA1 R219K GA基因型与A等位基因可诱发帕金森症和阿尔兹海默症的发生。     

双联抗血小板治疗急性冠脉综合征临床疗效观察

目的:探讨双联抗血小板治疗急性冠脉综合征(ACS)的临床疗效和安全性。方法:60例ACS患者随机分为治疗组和对照组。对照组给予阿司匹林单抗血小板治疗,治疗组采用阿司匹林+氯吡格雷双联抗血小板治疗,治疗3个月后评价临床疗效。结果:治疗组临床疗效总有效率为93.3%,显著高于对照组(76.7%),相比较有显著性差异(P<0.05);治疗后,两组LVEF、CO、E/A显著上升,与治疗前比较均有显著性差异(P<0.05);且治疗组与对照组比较有显著性差异(P<0.05)。结论:阿司匹林和氯吡格雷双联抗血小板药物治疗ACS,可以强化对血小板聚集的抑制,并增强抗栓效果,值得临床应用。