Human epididymis protein 4 (HE4) levels inversely correlate with lung function improvement (delta FEV1) in cystic fibrosis patients receiving ivacaftor treatment

Background

We have recently shown that human epididymis protein 4 (HE4) levels correlate with the severity of cystic fibrosis (CF) lung disease. However, there are no data on how HE4 levels alter in patients receiving CFTR modulating therapy.

Relationship between insulin-like growth factor I, dehydroepiandrosterone sulfate and proresorptive cytokines and bone density in cystic fibrosis

Introduction Patients with cystic fibrosis (CF) are known to be at risk for early osteoporosis, and the mechanisms that mediate bone loss are still being delineated. The aim of the present investigation was to investigate if a correlation exists in these patients between skeletal measurements by dual-energy x-ray absorptiometry (DXA) and two anabolic factors, dehydroepiandrosterone (DHEA) and insulin-like growth factor I (IGF-I), and proresorptive factors such as the cytokines interleukin-1β, tumor necrosis factor α, and interleukin-6. Methods We studied 32 outpatients (18 females; mean age: 26.2 ± 7.9 years) at a tertiary care medical center. The subjects had venous samples obtained, underwent anthropometric and bone mineral density (BMD) measurements, and completed a health survey. Serum IGF-I concentrations were below the age-adjusted mean in 78% of the participants, and DHEA sulfate (DHEAS) concentrations were low in 72%. Serum concentrations of all cytokines were on the low side of normal; nonetheless, there was a modest inverse correlation between IL-1β and BMD at all sites. Results In univariate analyses, IGF-I and DHEAS were significant correlates of BMD or bone mineral content. In final multivariate models controlling for anthropometric and other variables of relevance to bone density, only IGF-I was identified as a significant independent skeletal predictor. While alterations in DHEAS, IGF-I, and specific cytokines may contribute to skeletal deficits in patients with CF, of these factors a low IGF-I concentration appears to be most strongly correlated with BMD. Conclusions These findings may have therapeutic implications for enhancing bone density in these patients.     

A comparative immunohistochemical study of malignant mesothelioma and renal cell carcinoma: the diagnostic utility of Leu-M1, Ber EP4, Tamm-Horsfall protein and thrombomodulin

Metastatic renal cell carcinoma has occasionally been reported to mimic malignant pleural mesothelioma. Morphologically, histochemically and immunohistochemically, similarities in the two tumours exist making their differentiation difficult, particularly in biopsy specimens. The aim of this study was to make a comparative immunohistochemical analysis of the two tumours by use of a panel of four antibodies (Leu M1; Ber EP4; thrombomodulin and Tamm-Horsfall protein). Their suitability in differentiating between the two tumours was assessed. We examined 20 cases of renal cell carcinoma and 20 cases of malignant pleural mesothelioma. On immunostaining with Leu M1, 14 of 20 renal cell carcinomas were positive, yielding 70% sensitivity and 95% specificity and one of 20 mesotheliomas. In comparison, Ber EP4 antibody stained only seven of 20 of the renal cell carcinomas. In addition, it was noted that four tubulopapillary pattern renal cell carcinomas stained positively with both anti-Leu M1 antibody and Ber EP4 antibody. Thrombomodulin immunostaining was present in 11 of 20 mesotheliomas (55% sensitivity and demonstrated 95% specificity) and one of 20 renal cell carcinomas. For epithelial mesotheliomas only, thromobomodulin staining was identified in 10 of 14 cases. In the differentiation of renal cell carcinoma from epithelial mesothelioma we recommend the use of Leu M1 and thrombomodulin as diagnostically useful markers. None of the antibodies used in this study was effective in distinguishing sarcomatoid renal cell carcinoma from sarcomatous mesothelioma. Tamm-Horsfall protein showed little diagnostic utility in differentiating the two tumours.     

Bilateral Hematogenous Pseudomonas aeruginosa Endophthalmitis After Lung Transplantation

Pseudomonas aeruginosa commonly colonizes the airways of patients with cystic fibrosis (CF). However, the occurrence of bacteremia with metastatic infection to the eye causing endogenous endophthalmitis is very rare. In the setting of lung transplantation, the significance of P. aeruginosa bacteremia in patients with CF whose airways are colonized before transplantation is unknown. We report a case of bilateral P. aeruginosa endogenous endophthalmitis in a patient with CF after lung transplant without documented bacteremia. The patient presented with acute eye symptoms in the presence of a left atrial thrombus and the disease followed a rapidly progressive course requiring aggressive medical-surgical treatment. Typically P. aeruginosa endophthalmitis has been associated with a poor visual prognosis. However, with combined medical-surgical management this patient retained useful vision in one eye without having retinal detachment or requiring enucleation. Endogenous endophthalmitis should be considered in the differential diagnosis of ocular complaints in patients with CF after lung transplant.     

16例胸膜间皮瘤临床分析

１６例胸膜间皮瘤，其中１例有石棉接触史，占６．１７％；＞４０岁者占８１．５％。主要临床表现为胸痛、气短、咳嗽、低热和胸腔积液，临床上易误诊为结核性胸膜炎、肺癌胸膜转移等。胸部ｘ线检查及胸部ＣＴ对该病的诊断有帮助，大都有特征性表现。确诊靠针刺胸膜活检及开胸活检。胸膜间皮瘤的治疗主要采用手术、放疗和化疗，对于局限型治疗首选手术切除。肿瘤的良、恶性、疾病的分期及治疗与预后有一定关系。     

Diffuse malignant pleural mesothelioma in an urban hospital: clinical spectrum and trend in incidence over time

This retrospective analysis reviews the clinical experience of a major urban referral hospital with diffuse malignant pleural mesothelioma during the 14-year period from 1973 through 1986. Seventy-five cases of definite or equivocal mesothelioma were identified. There were four cases of primary malignant peritoneal mesothelioma, seven cases of benign fibrous mesothelioma, and 64 cases of diffuse malignant pleural mesothelioma. In 43 cases (67%) of diffuse malignant pleural mesothelioma, there was historic evidence of asbestos exposure. In 21 cases (33%), there was no known history of asbestos exposure. An increase in annual incidence of diffuse malignant pleural mesothelioma was observed over the study period, from three cases in 1973 to ten cases in 1986. Despite greater awareness of this disease, the diagnosis remains a difficult one to establish given the nonspecific symptoms, signs and radiographic appearance, variable histologic appearance, and poor diagnostic sensitivity and specificity of thoracentesis and closed pleural biopsy. Thoracotomy, thoracoscopy, and CT-guided needle biopsies gave higher yields and are the diagnostic measures of choice when diffuse malignant pleural mesothelioma is suspected.     

免疫组化在恶性间皮瘤鉴别诊断中的应用价值

目的 对恶性间皮瘤进行免疫组化的对比性研究,以进一步评价它们在恶性间皮瘤鉴别诊断中的价值。方法 采用免疫组织化学染色(LSAB)法对22例恶性间皮瘤和20例对照组腺癌组织进行EMA、CEA、CK、vimentin、间皮细胞(M-cell)等5种单克隆抗体的标记。结果 恶性间皮瘤的阳性率分别为 M-cell 91％,CEA 9％,vimentin 73％,CK82％,EMA64％;而腺癌的阳性率为分别为M-cell5％,CEA 85％,vimentin 15％,CK100％,EMA 100％。结论 在恶性间皮瘤和腺癌的鉴别诊断中,M-cell和CEA是较理想的标记物,尤其是M-cell,CEA和vimentin三种抗体联合应用更具有价值。而在恶性间皮瘤和其他梭形细胞肿瘤的鉴别诊断中,还应联合使用CK和EMA。     

Mortality study of asbestos cement workers

The present study describes cause-specific mortality of asbestos cement workers in the Emilia Romagna region of Italy. The cohort included workers in ten factories, most of which started operating between 1955 and 1965. Asbestos, mainly chrysotile, constituted 10%–20% of the dry component of the mixture. Crocidolite range between 5% and 50% of total asbestos. Asbestos concentrations up to 44 ff/cc were reported prior to 1975, while in recent years they have usually been below 0–1 ff/cc. The cohort included 3341 workers who had at some time been employed in the ten factories under study. Their mortality experience was compared with that of the population resident in Emilia Romagna. Vital status was ascertained at 1989. Seventy-three subjects were lost to followup (2.2%). Mortality from all causes and from all types of cancer was increased in the cohort. Malignant neoplasms of the respiratory tract showed a significant increase (SMR: 134; 90% confidence interval: 101–175; 40 observed) due to lung cancer (SMR: 124; 90% confidence interval: 91–166; 33 observed) and neoplasms of the pleura, mediastinum, and other parts of the respiratory tract (SMR: 602; 90% confidence interval 237–1267; 5 observed). The discrepancy between observed and expected mortality mainly concerned subjects with at least 20 years of employment in the factories. Five more cases of histologically confirmed mesothelioma occurred after the end of follow-up.     

Different opinions of physicians on the importance of measures to prevent acquisition of Pseudomonas aeruginosa from the environment

BACKGROUND: Since chronic infection with mucoid Pseudomonas aeruginosa (PA) is associated with deteriorating lung function, many parents of young children with cystic fibrosis (CF) fear the first PA positive throat swab as a milestone in the progression of the disease. To reduce the risk of PA acquisition from the environment, they perform preventive measures at home or outdoors. METHODS: In an attempt to evaluate the attitude of CF physicians towards these measures and the respective consulting practice, we mailed a questionnaire to all 65 certified paediatric CF centres in Germany. RESULTS: Physicians from 54 (83%) CF clinics replied. They expressed widely different ideas about the impact of the environment for the acquisition of P. aeruginosa, and recommended a large spectrum of preventive measures. Some physicians proposed only few precautions, which focussed on the prevention of cross-infection between patients, whereas others suggested prevention of any contact with moist or wet places, e.g. use different toothbrushes for mornings and evenings, or do without air-conditioning in the car. CONCLUSIONS: CF physicians have different opinions on the risk of PA acquisition from the environment. Doctors who recommend strict precautions could engender a parental fear of a ubiquous threat from invisible bacteria. The resulting extended safety measures might impair the family's quality of life.