双侧肾上腺高功能皮质腺瘤致ACTH非依赖性库欣综合征3例临床诊疗探索

目的 探讨双侧肾上腺高功能皮质腺瘤致促肾上腺皮质激素（ACTH）非依赖性库欣综合征的临床特点、诊治思路,提高对该疾病的认知。 方法 收集2016～2021年四川大学华西医院内分泌代谢科收治的3例双侧肾上腺功能性皮质腺瘤致ACTH非依赖性库欣综合征患者行回顾性分析,分析患者的一般情况、症状及体征、辅助检查、诊治过程等临床资料,并结合文献进行复习和讨论。 结果 3例患者均表现为典型且严重的库欣综合征,并伴有显著的心脏和代谢并发症,影像学提示双侧肾上腺单发腺瘤;2例患者行双侧肾上腺静脉采血,均提示双侧肾上腺皮质醇高分泌且无优势侧;1例患者行双侧肾上腺肿物切除术,另外2例患者因为心功能不全严重,行分期双侧肾上腺肿物切除术,术后病理结果3例均提示双侧肾上腺皮质腺瘤,术后皮质醇浓度均明显降低,均给长期予糖激素替代治疗。 结论 双侧肾上腺高功能皮质腺瘤致ACTH非依赖性库欣综合征非常罕见,明确诊断并判断双侧功能定位非常重要,治疗上应根据患者的病情及并发症情况个体化的选择最适合患者的手术方式,术后应严密随访,及时给予并调整糖皮质激素替代治疗,并终身随访。     

Adrenocorticotropin-secreting carcinoid tumour identified and treated 12 years after presentation with Cushing's syndrome.

A case of Cushing''s syndrome due to an adrenocorticotropin (ACTH) secreting bronchial carcinoid tumour is described. Endocrine assessment suggested ectopic ACTH syndrome, but imaging revealed no tumour. Bilateral adrenalectomy was performed, and computed tomographic scans of chest and abdomen were performed annually. A small nodule became apparent in the right lung 12 years after the presentation, which postoperatively was confirmed as the bronchial carcinoid tumour responsible for the ectopic ACTH syndrome.     

Determinants for the course of acute sinusitis in adult general practice patients

BACKGROUND—Although the prognosis of acute sinusitis is important, little is known about it and the factors predicting its course in a general practice population.
OBJECTIVE—To determine the course of acute sinusitis and factors predicting it in adults in general practice.
METHODS—The prognostic value of demographic and clinical factors and the patient''s emotional state, for example anxious or depressed, were determined prospectively by means of multivariate analysis.
MAIN OUTCOME MEASUREMENTS—Resolution of facial pain, resumption of daily activities, and the patient''s reported improvement. Factors with a significant predictive value were used to classify the patients into three different groups: quick, moderate, and slow recovery.
RESULTS—The median time from enrolment to recovery was six (range percentile 25-75: 4-10) days in a population of 177 patients. Factors predictive of a prolonged clinical course were: female sex (hazard ratio (HR) 0.60; 95% confidence interval (CI) 0.42 to 0.83), history longer than 14 days before inclusion (HR 0.62; 95% CI 0.41 to 0.94), headache, cold, or cough as a reason for the encounter (HR 0.65; 95% CI 0.44 to 0.96), and absence of cervical adenopathy (HR 0.71; 95% CI 0.51 to 0.96). Antibiotic treatment did not influence the course of disease. The median time to recovery was three days for patients with a quick, five days for those with a moderate, and seven days for those with a slow recovery.
CONCLUSION—In general practice acute sinusitis is mostly a self limiting disease. A limited number of characteristics are predictive of a (slightly) prolonged clinical course of acute sinusitis in general practice.


     

Cogan's syndrome: an oculo-audiovestibular disease

Typical Cogan''s syndrome is a rare disease of young adults consisting of flares of interstitial keratitis and sudden onset of Ménière-like attacks (nausea, vomiting, tinnitus, vertigo and hearing loss). Life-threatening aortic insufficiency develops in 10% of reported cases. Atypical Cogan''s syndrome (audiovestibular dysfunction with other types of inflammatory eye disease) is associated with vasculitis in 20% of cases and has a less favourable prognosis than typical Cogan''s syndrome.


     

Do computer generated ECG reports improve interpretation by accident and emergency senior house officers?

OBJECTIVES—To determine whether access to a computer generated electrocardiogram (ECG) report can reduce errors of interpretation by senior house officers (SHOs) in an accident and emergency department.
METHODS—Ten SHOs were asked to interpret 50 ECGs each: 25 with computer generated reports, 25 without. Their answers, and the computer generated reports, were compared with a "gold standard" produced by two experienced clinicians. The primary outcome measure was the proportion of major errors of interpretation.
RESULTS—The computer reading system made two major errors (4%, 95% confidence interval (CI) 1.1% to 13.5%) compared with the gold standard. Access to the computer report did not significantly reduce major errors among SHOs (46 (18.4%) with report v 56 (22.4%) without, odds ratio 0.64, 95% CI 0.36% to 1.14%, p=0.13) or improve the proportion completely correct (104 (41.6%) with report v 91 (36.4%) without, odds ratio 1.43, 95% CI 0.88 to 2.33, p=0.15).
CONCLUSIONS—SHOs have a high error rate when interpreting ECGs, which is not significantly reduced by access to a computer generated report. Junior doctors should continue to seek expert senior help when they have to interpret a difficult ECG.


     

Intraoperative glove perforation—single versus double gloving in protection against skin contamination

Surgeons have the highest risk of contact with patients'' blood and body fluids, and breaches in gloving material may expose operating room staff to risk of infections. This prospective randomised study was done to assess the effectiveness of the practice of double gloving compared with single gloving in decreasing finger contamination during surgery.
In 66 consecutive surgical procedures studied, preoperative skin abrasions were detected on the hands of 17.4% of the surgeons. In the double gloving pattern, 32 glove perforations were observed, of which 22 were in the outer glove and 10 in the inner glove. Only four outer glove perforations had matching inner glove perforations, thus indicating that in 82% of cases when the outer glove is perforated the inner glove will protect the surgeon''s hand from contamination. The presence of visible skin contamination was also higher in perforation with the single gloving pattern (42.1%) than with the double gloving pattern (22.7%).
An overwhelming majority of glove perforations (83.3%) went unnoticed. Double gloving was accepted by the majority of surgeons, especially with repeated use. It is recommended that double gloves are used routinely in all surgical procedures in view of the significantly higher protection it provides.


     

Ectopic Cushing's syndrome and pulmonary carcinoid tumour identified by [111In-DTPA-D-Phe1]octreotide.

The differential diagnosis and management of Cushing''s syndrome remain difficult, particularly for ectopic adrenocorticotropin (ACTH) syndromes resulting from small bronchial carcinoids. We report the case of a 41-year-old man with ectopic ACTH-dependent Cushing''s syndrome. Two computed tomography scans of the thorax were normal and magnetic resonance imaging of the chest showed a 6-mm hyperintense T1-weighted area close to the left pulmonary hilus, interpreted as probably vascular by the radiologists. An [111In-DTPA-D-Phe1]octreotide scintigraphy scan demonstrated a positive image for somatostatin receptors in exactly the same location and surgery confirmed the presence of a small ACTH-secreting carcinoid tumour in the upper left lung lobe which was resected. Surgery cured the hypercorticism of the patient. The differential diagnosis of Cushing''s syndrome and the procedure for localisation of an ACTH source are discussed.     

Delivery of surgical care in a district general hospital without high dependency unit facilities

BACKGROUND—Many hospitals lack the facilities for high dependency care, and patients requiring this level of care are nursed on the surgical ward. The aim of this study was to assess the extent of this problem in a district general hospital, looking at the impact of providing high dependency unit (HDU) care at ward level.
METHODS—A 28 bed surgical ward was studied for 39 consecutive days. Patients were assessed as being either appropriately placed (routine) or inappropriately placed (HDU). Nursing interventions and observations over each 24 hour period were recorded for the most dependent patient in each group.
RESULTS—Data were collected for a total of 1092 bed days. Median bed occupancy was 22 patients/day (78%). Inappropriately placed HDU patients accounted for 55 bed days (5%, mean 1.4 patients/day). These patients required more nursing intervention than routine patients.
HDU patients received more observations during a 24 hour period than routine patients (mean 11.3 and 4.2 respectively, p<0.005). The number of observations recorded for a routine patient in a 24 hour period fell when a HDU patient was nursed concurrently on the ward (mean 5.1/24 hours, falling to 3.8 /24 hours in the presence of an HDU patient, p<0.02 ).
CONCLUSIONS—HDU patients require more intensive nursing care than routine surgical patients, and the nursing of HDU patients on the ward adversely affects the quantity of care available for less dependent patients. High dependency care should therefore be provided in dedicated units.
HDU is an essential facility for all surgical patients, including those who require intensive nursing, and the routine surgical patient whose nursing is compromised by the failure to provide comprehensive postoperative care.


     

Post-thymectomy collapse: an unusual case of acute adrenal insufficiency.

A 54-year-old man who had an appearance strongly suggestive of Cushing''s syndrome, collapsed after the removal of a carcinoid tumour of the thymus. The clinical suspicion of acute adrenal insufficiency was supported by invasive haemodynamic data and the diagnosis was subsequently confirmed biochemically. These post-operative events were subsequently ignored and a further crisis was probably prevented by tumour recurrence (and hence resumption of adrenocorticotropic hormone (ACTH) production). The ectopic production of ACTH from a carcinoid tumour of the thymus is recognised but a report of acute adrenal insufficiency from its removal is probably unique.     

Good prognosis in thyroid cancer found incidentally at surgery for thyrotoxicosis

An analysis of the outcome of thyroid carcinoma incidentally discovered in patients undergoing surgery for hyperthyroidism is presented. Among 986 patients with differentiated thyroid cancer, 23 had presented with symptoms and signs of hyperthyroidism. Graves'' disease was diagnosed in 11, multinodular goitre in eight and toxic adenoma in four. Following thyroidectomy, histology revealed papillary (18), follicular (four) and Hurthle cell (one) carcinoma. Tumour size ranged from 4 mm to 5.5 cm, multifocality was detected in three patients, and lymph node involvement in one. Two patients (one with associated Graves'' disease, one with multinodular goitre) relapsed locally and required further surgery; one developed distant metastases and died 7 years after initial presentation. Two patients died of unrelated causes; the remaining 20 patients are alive and well with a median follow-up of 16 (1-34) years. Differentiated thyroid cancer found incidentally at surgery for hyperthyroidism has a good prognosis.


     

异位促肾上腺皮质激素综合征的外科治疗

目的：探讨异位促肾上腺皮质激素（adrenocorticotrophic hormone,ACTH）综合征的诊断与外科治疗。方法：对1996 年1月至2016年12月北京协和医院收治的异位ACTH综合征57例的临床资料进行总结,其中男32例、女25例,年龄11~68岁,平均32岁。血ACTH显著升高,平均77.6 pmol/L（16.5~365.6 pmol/L,正常值为＜10.1 pmol/L）,垂体MRI未见占位,CT检查双侧肾上腺均有不同程度弥漫性或结节样增生。按照不同治疗方案选择分为3组：A组25例,未发现异位肿瘤的异位ACTH 综合征,行双侧或单侧肾上腺切除的手术治疗;B组16例,发现异位肿瘤行肿瘤根治性切除术;C组16例,非手术治疗。分析不同治疗方法疗效及预后。结果：57例患者中有40例随访6个月至10 年,A组因病情凶险程度不同、术中情况各异,采取的治疗方式也有差别,部分为分期双侧肾上腺切除或仅行单侧肾上腺切除术,其中4例死于严重库欣综合征(Cushing’s syndrome)导致的糖尿病、肺部感染,18例存活至今,3例失访,存活率为81%（18/22）。B组16例行异位肿瘤根治性切除者,5例术后0.5~6.0年死于肿瘤复发, 3例存活至今,8例失访,存活率为37.5%（3/8）。C组4例放射疗法加化学药物治疗者1年内均死于肿瘤转移、糖尿病或肺部感染, 6例单纯化学药物治疗者均于1年内死于肺部感染,另6例失访,无1例存活。结论：异位ACTH 综合征的治疗难度大,靶腺切除是有效的治疗方法,尤其适用于库欣综合征临床症状严重而原发肿瘤无法定位者。     

Permanent pacemaker insertion in a district general hospital: indications, patient characteristics, and complications

This report reviews the experience of permanent pacemaker insertion in a district general hospital (catchment population of 350 000) and makes a comparison with the national database and other hospitals in the UK.
METHODS—The records of all patients receiving a permanent pacemaker in the inclusive period January 1996 to December 1998 were reviewed. Data collected included number of patients paced each year, age, sex, indications, and complications.
RESULTS—In the three years reviewed 200 patients received new permanent pacemakers, a rate of 190 per million population per year, which is similar to the national implantation rate of permanent pacemakers but lower than that of most European countries (see discussion). The majority of patients paced were elderly (75% were above the age of 70 years).
Atrioventricular block (including complete heart block, 45%, and Mobitz type 2 block, 12.5%) was the commonest indication for permanent pacemaker insertion, followed by sick sinus syndrome (25%) and these findings are comparable to those reported previously. However, carotid sinus syndrome was responsible for 16% of the patients paced and this was higher than that reported in the national database (6.5%). Only 1% of the pacemaker modes used was inappropriate and the complication rate was low at 3%.
CONCLUSIONS—This report confirms that permanent pacemaker insertion can be effectively and safely provided locally for the increasingly ageing population. The implantation rate both locally and nationally is still much lower than that of some countries in Europe.


     

The effect of passive smoking on pulmonary function during childhood

Passive smoking, especially of maternal origin, is known to influence adversely the development of children''s pulmonary function. In this study, the effect of parental smoking on the pulmonary function of 360 primary school children aged 9-13 (mean 10.8±0.7) years was investigated. Information on parental smoking history was collected using a questionnaire, and spirometric measurements were performed on the children.
All spirometric indices were lower in children who had been passively exposed to parental tobacco smoke than those not exposed. The percentage of households in which at least one parent smoked was 81.5%. This figure was significantly lower for mothers (27.5%) than for fathers (79%). Paternal smoking was associated with reduced levels of forced expiratory flow between 25-75% of vital capacity, peak expiratory flow, and flow rates after 50% and 75% of vital capacity expired (p<0.05). Maternal smoking did not have statistically significant adverse effects on children''s pulmonary function. This result might be due to the low occurrence of either pre- or post-natal smoking among mothers and confirms that, in our population, the main target group for anti-tobacco campaigns should be fathers.


     

Amide Proton Transfer Magnetic Resonance Imaging of Alzheimer's Disease at 3.0 Tesla: A Preliminary Study

Background:

Amide proton transfer (APT) imaging has recently emerged as an important contrast mechanism for magnetic resonance imaging (MRI) in the field of molecular and cellular imaging. The aim of this study was to evaluate the feasibility of APT imaging to detect cerebral abnormality in patients with Alzheimer''s disease (AD) at 3.0 Tesla.

Methods:

Twenty AD patients (9 men and 11 women; age range, 67–83 years) and 20 age-matched normal controls (11 men and 9 women; age range, 63–82 years) underwent APT and traditional MRI examination on a 3.0 Tesla MRI system. The magnetic resonance ratio asymmetry (MTR_asym) values at 3.5 ppm of bilateral hippocampi (Hc), temporal white matter regions, occipital white matter regions, and cerebral peduncles were measured on oblique axial APT images. MTR_asym (3.5 ppm) values of the cerebral structures between AD patients and control subjects were compared with independent samples t-test. Controlling for age, partial correlation analysis was used to investigate the associations between mini-mental state examination (MMSE) and the various MRI measures among AD patients.

Results:

Compared with normal controls, MTR_asym (3.5 ppm) values of bilateral Hc were significantly increased in AD patients (right 1.24% ± 0.21% vs. 0.83% ± 0.19%, left 1.18% ± 0.18% vs. 0.80%± 0.17%, t = 3.039, 3.328, P = 0.004, 0.002, respectively). MTR_asym (3.5 ppm) values of bilateral Hc were significantly negatively correlated with MMSE (right r = −0.559, P = 0.013; left r = −0.461, P = 0.047).

Conclusions:

Increased MTR_asym (3.5 ppm) values of bilateral Hc in AD patients and its strong correlations with MMSE suggest that APT imaging could potentially provide imaging biomarkers for the noninvasive molecular diagnosis of AD.     

Comparison of different regimes of misoprostol for the termination of early pregnancy failure

Background

Nearly 20% of all confirmed pregnancies end in spontaneous abortion. Misoprostol''s use in early pregnancy failure is varied and dose and route are not well established. The aim of this study was to compare the efficacy and the side effects of different regimes of misoprostol in causing expulsion of products of conception in early pregnancy failure.

Method

Women patients with an ultrasound diagnosis of early pregnancy failure, less than 12 weeks gestation were divided into two, Group-A: tab. Misoprostol 800 mcg 6 hourly vaginally, upto 3 doses. Group-B tablet misoprostol 600 mcg 6 hourly, sublingually for 3 doses. All observations were noted and statistical analyzed.

Results

Mean gestational age was 7.93 weeks. Mean induction abortion interval 18.183 h. Women patients with less than six weeks gestational age had least mean induction-abortion interval time, 15.75 ± 2.82 h in vaginal group but was highest in sublingual group 22 ± 2 h and 18.43 h in overall (P = 0.02). Though after 8 weeks, both routes were equally effective. Mean dose required in group-A was 20044 mcg and in group-B was 1564 mcg (P < 0.001). Efficacy of protocol was 88.89% in group-A and 92.85% in group-B.

Conclusion

Both regimes had comparable efficacy, acceptability (90%) and side effects. In women patients less than six weeks period of gestation, the vaginal (800 mcg) route was distinctly superior, in women patients with 6–8 weeks the sublingual (600 mcg) route was more advantageous. The correct dose must be used for the route chosen. The route of administration should be decided in accordance with the preference of the patient and the clinical situation.     

Out-of-hours work in cardiothoracic surgery: implications of the New Deal and Calman for training

With the introduction of the New Deal and the Calman Report, the duration of higher specialist training will be halved. We have examined the effect of reduced on-call rotas on exposure to relatively uncommon out-of-hours emergencies in cardiothoracic surgery. Operations for post-infarction ventricular septal defect, aortic dissection or transection, oesophageal perforation and pulmonary embolus performed out-of-hours between 1990 and 1995 were identified from hospital records. Over 6 years, the period of higher specialist training in cardiothoracic surgery, a trainee would see seven aortic emergencies on a 1:2 rota, four on a 1:4 rota and two on a 1:6 rota. These figures provide a powerful argument in support of the English Clause which allows trainees to be available for 83 hours a week, equivalent to a 1:4 rota, rather than 56 hours a week, equivalent to a 1:6 rota under the New Deal. This may need supplementation by a mechanism whereby trainees are `on call for training''.


     

Adult small bowel Dieulafoy lesion

A 47 year old woman presented with melaena and haemodynamic instability. Preliminary investigations failed to locate the source of bleeding. At laparotomy an arteriovenous malformation was identified in the distal ileum. Histology revealed this to be of the Dieulafoy type. This is the first published case of a histologically proved ileal Dieulafoy lesion in an adult.


     

Consultations and referrals for dyspepsia in general practice--a one year database survey

OBJECTIVE—Dyspepsia usually presents first in primary care. There are many reasons for referral including urgent problems (for example, haematemesis and melaena), treatment failure, or to exclude serious pathology. Referral will change the population characteristics of primary and secondary care dyspeptics. Many of the guidelines for primary care dyspepsia, however, are based on secondary care research on these referred patients. The aim of this study was to describe the prevalence of dyspepsia in general practice, the characteristics of patients presenting with dyspepsia in primary care, and the clinical and non-clinical determinants of referral in these patients.
DESIGN—Cross sectional survey of the consultation records of patients presenting with dyspepsia in primary care during a one year period.
METHODS—Dyspeptic patients who consulted their general practitioner (GP) in 1997 were selected on the basis of International Classification of Primary Care codes using a computer search among 20 sentinel practices affiliated with the Utrecht Network of General Practitioners. Cross tables and logistic regression analysis were carried out to reveal patient characteristics and determinants of referral.
RESULTS—The prevalence of dyspepsia presenting in primary care in 1997 was 3.4% (1740/48958). These patients were usually not referred during the first consultation. Men, elderly patients, and patients with a previous history of dyspepsia were referred to secondary care more frequently than other dyspeptic patients. Patients diagnosed with both irritable bowel syndrome and dyspepsia were at risk of being referred most.
CONCLUSION—Dyspepsia is a frequently occurring complaint in primary care and patients are usually treated by their GP. Besides clinically relevant reasons for referral, dyspeptic patients with irritable bowel syndrome seem to be more "at risk" of being referred to secondary care than other dyspeptic patients. The differences between primary and secondary care dyspeptic patients should be taken into account when interpreting research for guideline purposes. Further research is needed to clarify the background of the relation between irritable bowel syndrome and dyspepsia and its influence on referral.


     

Delays by patients in seeking treatment for acute chest pain: implications for achieving earlier thrombolysis

A study was set up to identify why patients delay seeking medical assistance after myocardial infarction. The study was performed in 100 consecutive patients with suspected acute myocardial infarction admitted to either the University Hospital of Wales, Cardiff, UK, or the Royal Jubilee Hospital, Victoria, British Columbia, Canada (50 patients from each centre). The main outcome measure was the delay from the onset of symptoms to admission to hospital. The mean total delay before admission was 385 minutes (SEM 45). The mean delay incurred by the patient in seeking assistance was 172 minutes (SEM 27), representing 45% of the total. Delay was longer in patients with crescendo angina and shorter in those later confirmed to have myocardial infarction. Patients with prior ischaemic heart disease (74% of patients) presented later than those with no such history. No other demographic or clinical factors predicted early or late presentation.
Delays in seeking medical assistance after the onset of severe chest pain contribute significantly to total delays in patients'' hospital admission and thrombolysis. The unexpected observation that patients with known ischaemic heart disease delay longer before seeking help in spite of their frequent contact with doctors, suggests that opportunities for educating patients are being wasted. Major efforts are needed to understand and modify behaviour of patients with chest pain to further reduce delays in treatment.


     

Diagnostic difficulties in periodic Cushing's syndrome.

A 22-year-old black woman presented with symptoms suggestive of Cushing''s syndrome three years after chemotherapy for a presumed teratoma with cervical lymphadenopathy. Initially, the absence of clinical signs and the demonstration of two normal 24 h urinary free cortisols appeared to exclude the diagnosis, but an ectopic adrenocorticotropin-producing thymic carcinoid was subsequently removed surgically. Cushing''s syndrome due to ectopic adrenocorticotropin production can be difficult to diagnose, particularly if there is periodic hormonogenesis.