Environmental fiber-induced pleuro-pulmonary diseases in an Anatolian village: an epidemiologic study

This study was designed to determine the prevalence of pleural mesothelioma and other malignancies in the Anatolian village of Tuzk?y, where neither asbestos nor any environmental carcinogen has been detected. Another village (Kizilk?y) located 12 km from Tuzk?y was selected as a control. Three hundred twelve subjects from Tuzk?y who were at least 25 yr of age and 95 subjects from Kizilk?y were studied. Analysis of X-rays of the Tuzk?y group revealed that subjects had calcified pleural plaques (17%), pleural thickening (10.5%), obscured costophrenic angles (15%), and diffuse interstitial pulmonary fibrosis (12.1%). Sixty-seven deaths were records in Tuzk?y during the previous 3 yr, 41 of which resulted from malignant diseases. There were no X-ray abnormalities or deaths resulting from malignancies in the control group. Because of the high incidence of mesothelioma and lung cancer which usually results from asbestos exposure, the presence of asbestos in Tuzk?y was investigated, but none was detected in spite to Tuzk?y's volcanic location. Nevertheless, zeolite, an asbestiform mineral, was detected in the stones of buildings and in the village soil, as well as in the lung and pleura of the patients during biopsy. Thus, this mineral was considered to be responsible for the fiber-induced pleuro-pulmonary diseases in Tuzk?y. No zeolite was found in the soil and stones of the control village.     

Residential exposure to magnetic fields: an empirical examination of alternative measurement strategies

OBJECTIVES: To investigate the impact of measuring a single home then imputing information from another home among subjects who lived in two homes in a subset of the National Cancer Institute/Children's Cancer Group (NCI/CCG) investigation of residential exposure to magnetic fields and risk of childhood leukaemia. METHODS: Each subject's summary time weighted average (TWA) exposure was derived from measurements of two homes, weighted by the fraction of the reference period lived in the residence. The three cost efficient field work strategies examined were measuring: (a) the longer lived in home; (b) the currently lived in home; and (c) the former lived in home. Two different methods were used for imputing the missing values: (a) control mean imputation, (b) status specific mean imputation. The subject's summary exposure to magnetic fields estimated with each approach was compared with the subject's TWA calculated from measurements in both homes. The association between estimated exposure to magnetic fields and the risk of leukaemia under different approaches was examined with unconditional logistic regression analysis. RESULTS: The Pearson correlation coefficient between the two measurements within subjects was 0.31 (p < 10(-4), indicating a lack of independence of measurements. Differences were found between mean exposures in current and former homes of cases, and between longer and shorter lived in homes of controls. All methods with measurements from one of the homes in conjunction with imputation of measurements for the second home led to marked attenuation of risk estimates at the highest exposure category, particularly when measurements from current homes were used and those from former homes were imputed. CONCLUSION: Results argue against attempting to estimate lifetime magnetic field exposure from imputed values derived from current residences to fill in gaps caused by unmeasured residences previously lived in.     

Constitutional mismatch repair deficiency and Lynch syndrome among consecutive Arab Bedouins with colorectal cancer in Israel

We assessed the molecular characteristics and the frequency of mutations in mismatch-repair genes among Bedouin patients with colorectal cancer (CRC) in Israel. Bedouin patients with a diagnosis of CRC at a major hospital in the southern part of Israel were deemed eligible for this study. The primary screening method was immunohistochemical staining for mismatch-repair proteins (MLH1, MSH2, MSH6, and PMS2). For subjects with abnormal immunohistochemical staining, we performed microsatellite instability (MSI) analyses, and for tumors with a loss of MLH1 expression we also performed BRAF testing. In MSI high cases we searched further for germline mutations. Of the 24 patients enrolled, four subjects (16.7%) had MSI high tumors: one subject was found to harbor a biallelic PMS2 mutation, one subject had Lynch syndrome (LS) with MSH6 mutation and two subjects had a loss of MLH1/PMS2 proteins/BRAF ^{wild type}/normal MLH1 sequence. Ten patients (41.7%) were younger than 50 at the time of diagnosis and none had first degree relatives with CRC. In conclusion, in this cohort of 24 consecutive Arab Bedouins with CRC, one patient was found to harbor a constitutional mismatch repair deficiency, one patient had LS with MSH6 mutation, and two patients had unresolved loss of MLH1/PMS2 proteins/BRAF ^{wild type} phenotype.     

CT findings in malignant pleural mesothelioma related to nonoccupational exposure to asbestos and fibrous zeolite (erionite).

Endemic malignant pleural mesothelioma (MPM) in Turkey is related to two mineral fibers, tremolite asbestos and fibrous zeolite (erionite). Thirteen cases of MPM from the Cappadocian area, where the soil is rich in erionite, and 29 cases of MPM, from villages whose occupants have high asbestos exposure, were examined by CT. The CT findings of the two groups of MPM were compared with respect to the configuration of the pleural lesions, stage of disease, fissural involvement, pleural effusion, presence of calcified pleural plaques, and chronic fibrosing pleuritis. In erionite-related MPM the pleural lesions were flat and smooth in 69.1%; in asbestos-related MPM the lesions were nodular in 55.1%. Stage IV disease, calcified pleural plaques, and chronic fibrosing pleuritis were more common in the erionite-related MPM. The rest of the findings were similar in both groups. The early radiological diagnosis of erionite-related MPM may be even more difficult because of the similarity of the pleural lesions to chronic fibrosing pleuritis.     

Effects of hyperbaric oxygen therapy on distraction osteogenesis

Investigators in the present study explored the effects of hyperbaric oxygen (HBO) therapy on distraction osteogenesis in a rabbit limb-lengthening model. HBO treatment was provided to participants in a small animal pressure chamber once a day during the latent period of distraction osteogenesis at 2.5 absolute atmospheres of pressure. Bone mineral density measurements were obtained through torsional testing. The control group received no HBO treatment. Animals were killed 8 wk postoperatively for biomechanical testing. There was a statistically significant increase in bone mineral density in the HBO group compared with that in the non-HBO group, but no statistically significant differences were observed between biomechanical parameters of the 2 groups. The investigators suggest that these results must be further studied through histologic examination to determine the stepwise effects of HBO during and after collagen synthesis.     

Abnormal neuromuscular transmission in cluster headache

OBJECTIVE: To identify and investigate any dysfunction of neuromuscular transmission in episodic cluster headache. BACKGROUND: Abnormal neuromuscular transmission has been shown in migraine with aura and in migraine without aura by using single fiber electromyography. Especially for migraine with aura, a genetic cause has been postulated. Episodic cluster headache is a primary headache disorder in which genetic factors may, at times, play a strong role. METHODS: Single fiber electromyography during voluntary contraction of the extensor digitorum communis muscle, nerve conduction studies of upper and lower extremities, and concentric needle electromyography of the extensor digitorum communis were performed on 6 patients with episodic cluster headache and 6 age-matched normal controls. Twenty potential pairs were recorded from each subject. Twenty individual jitter values and a mean jitter value were calculated for each subject. Both mean individual jitter values and numbers of abnormal individual jitter values were compared in patients with cluster headache versus normal controls. RESULTS: Three of 6 patients with episodic cluster headache showed pronounced neuromuscular transmission abnormalities by single fiber electromyography. None of the controls had any neuromuscular transmission abnormality. Another patient with episodic cluster headache had borderline dysfunction of neuromuscular transmission. Transmission was clearly normal in only 2 of 6 patients with cluster headache. CONCLUSION: These results suggest that episodic cluster headache may share the same abnormality of neuromuscular transmission observed in migraine.     

Low Density Granulocytes and Dysregulated Neutrophils Driving Autoinflammatory Manifestations in NEMO Deficiency

Journal of Clinical Immunology - NF-κB essential modulator (NEMO, IKK-γ) deficiency is a rare combined immunodeficiency caused by mutations in the IKBKG gene. Conventionally, patients are...