The pituitary V3 vasopressin receptor and the corticotroph phenotype in ectopic ACTH syndrome.

Ectopic ACTH secretion occurs in highly differentiated and rather indolent tumors like bronchial carcinoids or, in contrast, in various types of aggressive and poorly differentiated neuroendocrine tumors. We explored this phenomenon using the recently cloned human pituitary V3 vasopressin receptor as an alternate molecular marker of the corticotroph phenotype. Expression of V3 receptor, corticotrophin releasing hormone (CRH) receptor, and proopiomelanocortin (POMC) genes was examined in tumors of pituitary and nonpituitary origin. A comparative RT-PCR approach revealed signals for both V3 receptor and CHR receptor mRNAs in 17 of 18 ACTH-secreting pituitary adenomas, and 6 of 6 normal pituitaries; in six growth hormone- or prolactin-secreting adenomas, a very faint V3 receptor signal was observed in three cases, and CRH receptor signal was undetected in all. Six of eight bronchial carcinoids responsible for the ectopic ACTH syndrome had both POMC and V3 receptor signals as high as those in ACTH-secreting pituitary adenomas; in contrast, no POMC signal and only a very faint V3 receptor signal were detected in six of eight nonsecreting bronchial carcinoids. Northern blot analysis showed V3 receptor mRNA of identical size in ACTH-secreting bronchial carcinoids and pituitary tumors. Other types of nonpituitary tumors responsible for ectopic ACTH syndrome presented much lower levels of both POMC and V3 receptor gene expression than those found in ACTH-secreting bronchial carcinoids. In contrast with the V3 receptor, CRH receptor mRNA was detected in the majority of neuroendocrine tumors irrespective of their POMC status. These results show that expression of the V3 receptor gene participates in the corticotroph phenotype. Its striking association with ACTH-secreting bronchial carcinoids defines a subset of nonpituitary tumors in which ectopic POMC gene expression is but one aspect of a wider process of corticotroph cell differentiation, and opens new possibilities of pharmacological investigations and even manipulations of this peculiar ACTH hypersecretory syndrome.     

Diseases of the pituitary gland: clinical aspects and biochemical evaluation

Endocrine inactive and prolactin-secreting adenomas are the most commonly observed forms of pituitary tumors counting for more than 50 percent of diseases. In most cases first clinical symptoms can be attributed to insufficiency of the adenohypophysis which is also true for gonadotropin and thyroid stimulating hormone (TSH) producing adenomas. Clinical signs and symptoms of secondary insufficiency and primary forms of endocrine deficits do not differ significantly and the diagnosis may be hampered by involvement of more than one organ system. In contrast, symptoms and signs of ACTH or GH producing tumors are more impressive leading to diagnosis at an earlier stage. In case of corticotropic and somatotropic dysfunction dynamic tests are necessary for diagnosis. Close cooperation of endocrinologists, radiologists, and neurosurgeons is necessary for the effective workup of pituitary gland diseases.     

垂体柄阻断综合征的临床及MR表现

分析垂体柄阻断综合征(PSIS)的MRI表现,以提高诊断水平.方法:回顾性分析4例PSIS患者的临床和MRI检查资料.结果:4例患者实验室检查示单或多种垂体激素缺乏,具备相应临床症状.MRI检查显示2例患者垂体柄未显示,1例中断,1例纤细,3例前后叶体积缩小,均见垂体后叶异位,其中3例表现为高信号.结论:PSIS表现为部分性或完全性垂体前叶功能减退,MRI特点为垂体体积缩小,垂体柄变细或缺如,垂体后叶异位.     

An Evaluation of the Distinction of Ectopic and Pituitary ACTH Dependent Cushing's Syndrome by Clinical Features, Biochemical Tests and Radiological Findings

The efficiency of various laboratory and radiological investigationsin the differentiation of ectopic from pituitary dependent Cushing'ssyndrome was studied, based on findings in 23 patients withverified Cushing's disease and seven patients with the ectopicACTH syndrome. Clinical features strongly favouring the ectopic type were malesex and history for less than 18 months. Basal biochemical featuresstrongly indicating the ectopic syndrome included plasma K⁺<3.0 mmol/l and HCO₃ >30 mmol/l; serum cortisol at 9 a.m.or midnight of > 800 nmol/l; urine free cortisol > 1300nmol/24 hours; plasma ACTH > 100 ng/1. In the high-dose dexamethasone suppression test, suppressionby < 50 per cent of 9 a. m. serum cortisol, urine free cortisolor 17-oxogenic steroids was usually indicative of an ectopicsource of ACTH. A mean suppressed value of > 450nmol/l forthe 9 a.m. and midnight cortisol combined occurred in all ofthose with the ectopic syndrome, but in none of the 23 patientswith Cushing's disease. For urine free cortisol, a mean suppressedvalue of < 1000 nmol/24 hours was found in all patients withCushing's disease, but in none of those in the ectopic group. In the metyrapone test, there was an increase of     

Dialysis arthropathy: a clinical, biochemical, radiological and histological study of 36 patients

Out of a population of 97 haemodialysis patients, 36 patients with dialysis arthropathy were identified. Dialysis arthropathy is a chronic symmetrical polyarthritis which affected 97 per cent of the patients who had been undergoing cuprophane haemodialysis for more than 10 years. It commonly affected the shoulders, hips, hands, knees and wrists, worsening with time and extending to other joints. Fifty-eight per cent of the patients complained of morning stiffness and 47 per cent complained of exacerbation of shoulder pain during or after haemodialysis. Half of the patients also suffered from carpal tunnel syndrome, which recurred and was associated with a long-lasting disability. The most common radiological abnormality was periarticular bone cysts, followed by articular erosions and a destructive spondyloarthropathy, but clinical symptoms were more common than radiological signs. Patients with dialysis arthropathy had a higher C-reactive protein level than patients without arthropathy (18.6 mg/l versus 11.4 mg/l), indicative of an inflammatory process. Some of the clinical manifestations of the disease correlated with levels of C-reactive protein and ferritin. Serum ferritin levels correlated strongly with the units of blood transfused in the past five years (RS = 0.83), and the logarithm of ferritin level correlated weakly with C-reactive protein (r = 0.32). Haemarthroses were documented in 19 per cent of patients. Mean serum beta 2-microglobulin was elevated in the patients with (57.3 mg/l) and without arthropathy (50.7 mg/l), and there was no difference in the parathormone or aluminium levels between these groups. Articular tissue was obtained in 25 patients; beta 2-microglobulin amyloid was present in 24. Larger deposits were present in the capsular tissue, and these appeared to replace collagen bundles in eight cases. Amyloid deposits replaced the lining layer in six cases. It is likely therefore that amyloid disrupts normal joint function by replacing normal joint tissue. Mild chronic synovitis with haemosiderin deposition were found in approximately 60 per cent of cases. These findings suggest that amyloid derived from beta 2-microglobulin has a primary role in the pathogenesis of dialysis arthropathy, but there was also evidence of inflammatory processes. It is suggested that iron overload or haemarthroses might contribute to the inflammation, but other factors, such as dialysis-related bioincompatibility reactions, may also have a role.     

黑斑息肉病临床诊断和影像检查意义

目的:黑斑息肉病(PJS)是常染色体显性遗传病,属少见病,通过临床表现及影像学分析,加深对此少见病的认识。方法:收集我院1994年10月~2004年6月入院诊治的PJS患者26例,男12例,女14例。22例行结肠镜检查;23例行全消化道造影检查;4例患者行腹部CT及CT三维结肠重建和仿真内窥镜检查。结果:①临床以口唇粘膜、肢端色素沉着为体征,腹痛、腹胀及消化道出血为症状。②结肠镜检查19例发现结肠多发息肉;全消化道造影21例发现小肠多发息肉;CT结肠仿真内镜均发现多发息肉;2例随诊病例确诊结肠腺癌。结论:PJS恶变率远高于正常人群,内窥镜活检及染色体检查是诊断关键,影像学检查对发现息肉,特别是小肠息肉有重要诊断意义,而且为无创的定期复查提供了平台。     

A case ascertainment study of septic discitis: clinical, microbiological and radiological features

We studied the spectrum of septic discitis presenting to two busy district general hospitals over 2.5 years (November 1996 to April 1999), surveying the case notes of all patients attending Royal Bournemouth and Poole Hospitals with probable septic discitis on magnetic resonance imaging (MRI). Twenty-two cases of septic discitis were identified, suggesting an annual incidence of 2/100 000/year. Seventy-three percent of patients were aged > or =65 years. In 91% of patients, back pain was the presenting symptom, with neurological signs evident in 45% of patients. Fever >37.5 degrees C was present in 68% of patients, and a marked elevation of erythrocyte sedimentation rate (ESR) in 91%. Diagnosis was originally by MRI in 86% of patients, with plain radiographs not diagnostic of discitis in the early stages of the infection. Staphylococcus aureus was the commonest pathogen (41%), but in 18% of patients, no organism was identified. The major predisposing factors to septic discitis were invasive procedures (41%), underlying cancer (25%) and diabetes (18%). Pre-existing degenerative spinal disease was found in 50% of patients. Four patients whose causative organism was not isolated had a poorer outcome: one death and three with increased morbidity. Our estimated incidence rate (2/100 000/year) is higher than that in previous studies and may be due to a higher detection rate with MRI and/or a genuine increase in the number of cases. Septic discitis should be considered in any patient who has severe localized pain at any spinal level, especially if accompanied by fever and elevated ESR, or in the immunosuppressed.     

垂体腺瘤侵袭性与临床特征相关性分析

目的探讨垂体腺瘤侵袭性与病人临床特征间的相关性。方法从我科1998年5月~2004年9月收治的113例垂体腺瘤病人中筛选临床资料较全的85例,对患者临床特征及肿瘤MR及CT表现进行回顾性分析。结果垂体腺瘤侵袭性强弱与病人性别、肿瘤大小、形态、有无卒中、坏死、囊变及术后尿崩的发生率、肿瘤术后复发率相关。结论侵袭性垂体腺瘤与非侵袭性垂体腺瘤之间并无严格界限。侵袭性强弱与病人的性别、肿瘤大小、形态、有无卒中、坏死、囊变及术后尿崩的发生率、肿瘤术后复发率相关。     

A reappraisal of clinical, roentgenographic, and endoscopic features of the Zollinger-Ellison syndrome

Recent experience with 40 patients with the Zollinger-Ellison syndrome at all Mayo Clinic suggests that traditional clinical criteria for diagnosis are often absent or invalid. Patients are younger have a shorter duration of symptoms, and often present without prior gastric surgery. Clinical, roentgenographic, and endoscopic findings indistinguishable from those of idiopathic duodenal ulcer or erosive duodenitis were the only presenting features in half of the patients in this series. Therefore, increased diagnostic use of serum levels of gastrin and gastric analysis appears desirable, particularly in patients selected for elective surgical treatment of duodenal ulcer disease, because specific therapeutic approaches may be required.     

Neuroendocrine carcinoma of the stomach: clinical features and CT findings

Purpose

The purpose of the study is to investigate the computed tomographic characteristic and clinical findings of gastric neuroendocrine carcinoma (G-NEC) to increase awareness of this disease.

Methods

Twenty-two patients with a diagnosis of G-NEC were identified through the PACS of our hospital from August 2010 to November 2014. The clinical data, computed tomography (CT) features, and pathology records were analyzed.

Results

Among the 22 patients, 21 were male (95.45%), and 1 was female (4.55%). The mean age was 63.5 years old. Positive rates of neuroendocrine markers were 77.28% for chromogranin A staining, 86.36% for synaptophysin staining. All cases were single lesions including 16 (72.73%) in the gastric fundus, 3 (13.64%) in the gastric body and 1 (4.55%) in the gastric angle. Additionally 2 (9.09%) were found in the gastric antrum. Gastric wall was local thickening in 15 cases, and mass formation in 7 cases, with the stenosis and deformation of the adjacent gastric cavity. The long-axis diameter of the lesions ranged from 1.2 to7.4 cm (mean diameter, 2.47 cm), and the long-axis diameter was <2 cm in 12 case, 2–7.4 cm in 10 cases. The radiodensity values of the lesions were homogeneous density in 15 cases ranging from 22 to 47 HU (mean 34 HU). An ulcer with an irregular base and slightly raised borders located in the stomach was seen in 19 cases. The CT images showed homogeneous enhancement in 15 cases and heterogeneous enhancement in 7 cases. Obvious enhancement was seen in two cases, moderate enhancement was seen in sixteen cases, and mildly enhancement was seen in four cases. The peak value occurred in the arterial phase in 5 cases and the peak value was seen in 17 cases in the portal phase. Eleven lesions invaded the gastric serosa, and lymphatic metastasis was observed in 21 cases, 8 of which were combined with liver metastasis. CT images revealed 2 cases of the liver metastasis had obvious enhancement.

Conclusion

The CT features regarding location, incidence rates of ulcer and enhancement pattern described in our findings are common in all malignant gastric tumors. Therefore, the diagnosis of G-NEC must be confirmed with pathological test.

     

Re-evaluation of the etiology and clinical and radiological features of community-acquired lobar pneumonia in adults

Objective

The aims of this study were to elucidate the frequency and etiology of community-acquired lobar pneumonia (CALP) and the clinical and radiological differences between CALP and tuberculous lobar pneumonia (TLP).

Whipple's disease: clinical, biochemical, and histopathologic features and assessment of treatment in 29 patients

Whipple's disease is a chronic systemic illness, the optimal treatment of which remains poorly defined. In our analysis of a 30-year, 29-patient experience with Whipple's disease at the Mayo Clinic, the frequent initial manifestations of diarrhea, weight loss, arthritis, and lymphadenopathy correlated with findings reported previously by other investigators. Antibiotic therapy yielded rapid symptomatic and biochemical improvement, and histologic changes in the small bowel occurred subsequently. Despite antimicrobial therapy, relapses in patients with Whipple's disease are common, and the central nervous system is considered the most serious site of involvement for recurrence. Administration of an antibiotic agent that is able to cross the blood-brain barrier may be more important in preventing relapse than prolonged duration of initial antimicrobial therapy.     

A case of transient left ventricular apical ballooning syndrome in a child: clinical features and imaging findings

Transient left ventricular apical ballooning syndrome (TLVABS) is an acute cardiac disease that is characterized by transient left ventricular systolic dysfunction involving the apical region. The symptoms and electrocardiographic changes of TLVABS mimic those observed in acute myocardial infarction while obstructive coronary arterial lesions are not seen in patients with TLVABS. TLVABS usually occurs in elderly women after physical or emotional stress. However, it is very rare in children and so it not well known to pediatricians. Accordingly, TLVABS in children can be misdiagnosed as myocarditis or cardiomyopathy. We report here on a case of child who showed the typical findings of TLVABS in association with pericarditis. He presented with dyspnea and pericardial effusion, which required pericardiocentesis. After pericardiocentesis, he showed the typical echocardiographic and electrocardiographic findings of TLVABS. The MRI findings at 14 days after the initial symptoms showed normal coronary arteries and normal left ventricular function without any wall motion abnormalities. In addition, no delayed hyper enhancement was found on delayed-enhanced (DE)-MRI. We also reviewed the other reported cases of TLVABS in patients who were under the age of 40.     

<Emphasis Type="Italic">Pneumocystis</Emphasis> pneumonia in patients with HIV infection: clinical manifestations,laboratory findings,and radiological features

Pneumocystis pneumonia (PCP) remains the most common opportunistic infection in patients with acquired immunodeficiency syndrome (AIDS). Familiarity with the clinical features of PCP is crucial for prompt diagnosis, even if the patient is unaware of their HIV serostatus. We describe herein the clinical features of 34 episodes in 32 patients with AIDS-associated PCP and review the existing literature. As for symptoms, the frequency of fever, cough, and dyspnea was 74%, 74%, and 65%, respectively, and the complete triad was present in only 14 of the 34 episodes on first examination. Median duration from onset of symptoms until diagnosis was 3 weeks, and AIDS-associated PCP tended to take an insidious clinical course. Although laboratory findings were generally nonspecific, measurement of β-D-glucan levels in the serum or plasma was highly useful in the diagnosis of PCP. All but 1 of the patients showed β-D-glucan levels higher than the cutoff value (median, 147 pg/ml; range, 5–6920 pg/ml). Typical radiographic features of PCP are bilateral, symmetrical ground-glass opacities, but a wide variety of radiographic findings were observed. In our patients, high-resolution computed tomography (HRCT) of the lung showed ground-glass opacities sparing the lung periphery (41% of episodes) or displaying a mosaic pattern (29%), or being nearly homogeneous (24%), ground-glass opacities associated with air-space consolidation (21%), associated with cystic formation (21%), associated with linear-reticular opacities (18%), patchily and irregularly distributed (15%), associated with solitary or multiple nodules (9%), and associated with parenchymal cavity lesions (6%).     

IL-6、ESR、CRP和常规生化检测用于诊断新型冠状病毒肺炎的临床价值分析

目的探讨白细胞介素-6(IL-6)、红细胞沉降率(ESR)和C反应蛋白(CRP)和常规生化指标在新型冠状病毒肺炎(COVID-19)诊断中的应用价值。方法收集2020年1-2月湖南中医药大学第一附属医院和厦门大学附属第一医院44例COVID-19确诊患者作为新冠肺炎组,30例体检健康者作为健康对照组。采集研究对象的血液标本,检测其IL-6、ESR、CRP和常规生化指标的水平,对IL-6、ESR、CRP作受试者工作特性曲线(ROC曲线),分析灵敏度和特异度,并且对IL-6、ESR、CRP和常规生化指标的结果进行综合比较。结果新冠肺炎组的IL-6水平为4.12(1.53~14.16)pg/mL,高于健康对照组,但差异无统计学意义(P>0.05);新冠肺炎组ESR和CRP的水平分别为61.50(19.75~85.00)mm/h、16.16(8.30~37.33)mg/L,显著高于健康对照组,差异有统计学意义(P<0.05)。IL-6、ESR和CRP单项检测诊断COVID-19的ROC曲线下面积(AUC)依次分别为0.577、0.872、0.905,灵敏度依次分别为38.6%、75.0%、72.7%,特异度依次分别为100.0%、96.7%、100.0%。CRP对COVID-19的诊断效能要优于ESR、IL-6,ESR对COVID-19的诊断效能要优于IL-6。而IL-6、ESR和CRP三项联合检测诊断COVID-19的AUC为0.939,灵敏度为86.4%,特异度为96.7%,要优于IL-6+ESR、IL-6+CRP、ESR+CRP两项联合检测及IL-6、ESR和CRP单项检测的诊断效能。同时40.91%的患者IL-6升高,79.55%的患者ESR升高,68.19%的患者CRP升高,93.18%的患者肌酸激酶(CK)正常,95.45%的患者肌酸激酶同工酶(CK-Mb)正常,48.00%的患者白细胞计数(WBC)正常,44.00%的患者WBC下降,64.00%的患者淋巴细胞百分率(LYMPH%)下降,52.00%的患者淋巴细胞计数(LYMPH)下降。结论IL-6、CRP和ESR三项指标联合检测的诊断效能优于IL-6、CRP、ESR单项检测和其他两项指标联合检测的诊断效能,所以IL-6、ESR和CRP对COVID-19有一定的诊断价值。