Urinary 24-hour copper excretion at the time of diagnosis in children with Wilson's disease.

The optimal cut-off value of 24-hour (h) urinary copper (Cu) levels to identify Wilson's disease (WD) has not been widely studied in children. In sixty-six children with confirmed WD and 88 children without WD, 24-h urinary excretion of Cu at the time of diagnosis was studied. The receiver operating characteristic (ROC) curves revealed that the optimal cut-off value of urinary Cu to identify WD was 70 mcg [area under the curve (AUC) = 0.894] with a sensitivity and specificity of 81.8% and 89.8%, respectively. When the serum ceruloplasmin level was < 20 mg/dl and the 24-h urinary excretion of Cu was >70 mcg, the sensitivity was 75.8%, and the specificity was 97.7%. After the exclusion of cholestatic patients, the ROC curves revealed that the optimal cut-off value for 24-h urinary Cu excretion was 55 mcg (AUC = 0.910) with a sensitivity and specificity of 83.3% and 90.3%, respectively. When the ceruloplasmin level was <20 mg/dl and the 24-h urinary Cu excretion was >55 mcg, the sensitivity and specificity were 77.3% and 98.4%, respectively. A 24-h urinary Cu level of >70 mcg plus a ceruloplasmin level of < 20 mg/dl in the patients, and a 24-h urinary Cu level of >55 mcg plus a ceruloplasmin level of <20 mg/dl in non-cholestatic patients exhibited the highest specificity and the highest positive and negative predictive values to identify WD in children.

Evaluation of an amplified Mycobacterium tuberculosis direct test in clinical specimens.

SETTING: An increase in tuberculosis cases has been observed since the last decade, and rapid and specific tests are needed to directly detect Mycobacterium tuberculosis in respiratory specimens. The Amplified Mycobacterium Tuberculosis Direct Test (AMTDT) is a direct specimen assay for the identification of M. tuberculosis. OBJECTIVE: To evaluate the sensitivity and specificity of the AMTDT. METHODS: We performed the test in 426 patients: 58 patients who had culture-proven and/or clinically diagnosed tuberculosis, and 368 patients who were negative for M. tuberculosis by culture and clinical criteria (the control group). The results were compared to culture and clinical diagnosis of tuberculosis. RESULTS: AMTDT was found to be positive in 35 of the 58 patients who had culture-proven and/or clinically diagnosed tuberculosis and 71 of the 368 controls. Seventeen of the tuberculosis group were smear-negative, and AMTDT was positive in 11 of these patients. The sensitivity and specificity of the test were respectively 60% and 80%. CONCLUSION: AMTDT can be used as a complementary technique in tuberculosis patients in the presence of high clinical suspicion.

Azathiopine-steroid combination therapy for pulmonary arterial aneurysms in Behçet's disease.

Vasculitis is the primary pathological feature of Behçet's disease and in which pulmonary arterial aneurysm formations are rare but serious complications representing poor prognosis. There is no agreement on how to treat major venous and arterial disease. The use of steroids alone or corticosteroid treatment supplemented with cyclophosphamide or azathioprine is still controversial. We present three young male patients with Behçet's disease with aneurysms in the pulmonary arteries. In two of them, cyclophosphamide was the first choice of treatment, but this was ceased due to side effects. All three patients were then treated with azathioprine (2 mg/kg per day) and methylprednisolone (1.5 mg/kg per day). All three showed clinical improvement. Regression was observed radiologically in two (one minimal and the other complete). No complication related to therapy was encountered. Steroid therapy supplemented with azathioprine may be a good choice for treatment of pulmonary arterial aneurysms in Behçet's disease.

Atopic status of an adult population with active and inactive tuberculosis.

The rise in allergic disorders over the past three decades has been suggested to be related to the decrease in infectious diseases. Recently, a negative association between tuberculin responses and atopic disorders has also been reported. We planned to investigate the effect of natural exposure to Mycobacterium tuberculosis on atopic status in patients with active tuberculosis and to compare the findings with the data of patients with inactive disease. A total of 97 subjects were divided into two groups. Group 1, patients with proven active pulmonary tuberculosis (n = 66); group 2, subjects who had a history of previous tuberculous disease, with negative bacteriologic studies and no clinical and/or roentgenographic evidence of current disease (n = 31). Current history of allergic diseases was recorded by a physician with the use of a questionnaire adapted from the European Community Respiratory Health Survey (ECRHS), and skin-prick tests (SPTs) were performed using a standardized panel. Total IgE and Phadiatop were measured by the Pharmacia uniCAP system. The rate of one or more positive SPTs was significantly lower in the patients with active tuberculosis than the inactive group (15% versus 48.4%, p < 0.001). The current history of atopic diseases was 7.6% and 29% in the active and inactive tuberculosis groups, respectively (p = 0.002). The rate of positive skin tests to inhalant allergens in patients with inactive disease was higher than the rate of healthy adult Turkish people (48.4% versus 25%, p = 0.001). Geometric mean of total IgE levels were lower in patients with inactive disease than patients with active pulmonary tuberculosis (74.97 kU/L versus 106.3 kU/L, p = 0.05). The ratios of Phadiatop positivity were 21% and 38.7% in the active and inactive tuberculosis groups, respectively (p = 0.008). We found lower atopy rates in patients with active pulmonary tuberculosis than subjects with inactive disease. Although our data support the hypothesis that M. tuberculosis may prevent the development of atopic disorders by inducing the production of cytokines antagonistic to Th2 development, we believe prospective and experimental studies are needed before attributing a direct cause-effect link to this association.

Repeated unintentional transthoracic needle aspiration of a pulmonary hydatid cyst.

Transthoracic needle aspiration is not a recommended diagnostic modality in hydatid disease. Percutaneous aspiration of a suspected hydatid cyst is believed to be associated with the risk of allergic reactions which can result in systemic anaphylaxis and possible spreading of the cyst contents. We present herein a illustrative case of pulmonary hydatid cyst and multiple mediastinal lymphadenopathies which was diagnosed after repeated transthoracic fine needle aspirations.

Coseismic and Postseismic Fault Slip for the 17 August 1999, M = 7.5, Izmit, Turkey Earthquake.

We use Global Positioning System (GPS) observations and elastic half-space models to estimate the distribution of coseismic and postseismic slip along the Izmit earthquake rupture. Our results indicate that large coseismic slip (reaching 5.7 meters) is confined to the upper 10 kilometers of the crust, correlates with structurally distinct fault segments, and is relatively low near the hypocenter. Continued surface deformation during the first 75 days after the earthquake indicates an aseismic fault slip of as much as 0.43 meters on and below the coseismic rupture. These observations are consistent with a transition from unstable (episodic large earthquakes) to stable (fault creep) sliding at the base of the seismogenic zone.