Closed Meningo(encephalo)cele: a new feature in Hunter syndrome.

BACKGROUND AND PURPOSE: Hunter syndrome (MPS type II) is a rare X-linked recessive disease caused by lysosomal enzyme iduronate-2-sulfatase deficiency, characterized by frequent and variable brain and skull involvement. Our objective was determine the frequency of closed cephaloceles in a large cohort of subjects affected with Hunter syndrome and to investigate possible correlations with clinical and neuroradiologic findings. MATERIALS AND METHODS: Brain MR imaging of 33 patients (32 males and 1 female, age range 2.5-30.8 years, mean age 10.4 years) affected with Hunter syndrome were retrospectively evaluated. Eleven (age range 3.6-30.8 years; mean age 15.1) presented with an "attenuated" phenotype, while 22 (age range 2.5-19.1 years; mean age 8.2) had a "severe" phenotype. RESULTS: A closed cephalocele was detected in 9/33 patients (27%) at the level of anterior and middle fossa in 6 and 3 cases, respectively; 6/9 subjects were affected with the attenuated phenotype and 1/9 suffered from epilepsy. Closed cephaloceles did not show a significant association with other brain and spine MR imaging features of Hunter disease, such as enlargement of perivascular spaces, cisterna magna, pituitary sella, ventricles and subarachnoid spaces, craniosynostosis, dens hypoplasia, white matter abnormalities, spinal stenosis due to periodontoid cap, platyspondylia, or intervertebral disk anomalies. CONCLUSIONS: Closed cephaloceles are frequent in Hunter syndrome and should be considered a neuroradiologic feature of this disease.

Repeated case-control studies as a method of surveillance for asthma in occupations.

Our aim was to establish whether the case-control design may be applied in surveillance for occupational asthma. In a region with intensive manufacturing industries, we carried out one case-control study from 1974 to 1978 and another from 1989 to 1993; 325 and 387 pairs of cases of asthma and of age- and sex-matched control subjects, respectively, were selected. Current risks were found to be higher than past estimates for painters, farmers, millers/bakers, textile, wood/ furniture, and chemical workers. High risks for asthma have recently appeared in leather, polyurethane plastics, hospital and food industry workers, welders, and shoemakers. It is concluded that the case-control approach may be used to describe variations in asthma risk by occupational categories and time. A method to measure the efficiency of the diagnostic process for occupational asthma is also proposed.

[Urban air pollutant exposure among traffic policemen]. / Esposizione agli inquinanti dell'aria urbana dei vigili municipali.

Exposure to dusts and benzene was studied in 65 traffic policemen. Samples of total dusts showed that mean personal exposure was 0.44 (SD = 0.30) mg/m3, with peaks of about 2 mg/m3. Exposure to 1-nitropyrene (1-NP), the main compound occurring in emissions from diesel engines, which was estimated from concentrations in dusts collected with high-flow samplers, was 0.28 (SD = 0.19) ng/m3 (range: 0.06-1.24 ng/m3). The mean concentration of benzene in the breathing zone was 41 (SD = 20) micrograms/m3, although a level of 100 micrograms/m3 was slightly exceeded in one subject. In urine samples collected before and after workshifts, two biological indicators of exposure to benzene were measured, urinary benzene and urinary trans, trans-muconic acid (MA). The mean values of urinary benzene before and after workshift were similar (98, SD = 81 and 83, SD = 55 ng/l; n = 63; Wilcoxon's T-test = not significant), while a moderate increase in the metabolite was observed (MA = 0.08, SD = 0.11; 0.11, SD = 0.09 mg/g creatinine, in pre- and post-shift samples respectively; Wilcoxon's T-test, z = 3.00; p < 0.01). The levels of exposure to dusts and 1-NP deriving from diesel engine emissions were comparable to those of other occupational groups with this type of risk (garage mechanics, workers operating diesel engine machinery, etc.). Traffic police exposure to benzene was similar to that of the whole population of Padova (40 micrograms/m3, mean annual 24-hour value). However, the values of urinary MA, like those reported by other authors for non-smoker controls, increased after the workshift, indicating low occupational exposure to this pollutant. It should be noted that traffic police exposure to benzene is much lower than that of other occupational categories, e.g., fuel pump distributors.

Excretion of mutagens, nicotine and its metabolites in urine of cigarette smokers.

Urine samples from 26 cigarette smokers on a restricted diet were collected in the late afternoon. Urine extracts on XAD-2 resin were tested for mutagenicity in the microsuspension assay using Salmonella typhimurium strain TA98 in the presence of metabolizing and deconjugating enzymes. Levels of urinary nicotine plus metabolites and cotinine were determined. Eighteen samples were clearly mutagenic, i.e. capable of doubling the number of spontaneous revertants at one of the assayed doses of urine. Urinary mutagenic activity ranged from 193 to 8462 net revertants/mmol of creatinine, while urinary nicotine plus metabolites and cotinine levels varied from 0.007 to 1.366 and from 0.011 to 0.297 mg/mmol creatinine. Urine samples with nicotine metabolite levels of < 0.33, 0.33- < 0.66 and > 0.66 mg/mmol creatinine had mean values +/- SD of mutagenic activity of 490 +/- 222 (n = 10), 964 +/- 560 (n = 9) and 2692 +/- 2807 (n = 7) revertants/mmol of creatinine, respectively, the statistical comparison between the groups being positive (Mann-Whitney U-test, P < 0.05). The mutagenic activity of urine samples from smokers correlated well with urinary nicotine plus metabolite levels (r = 0.658, P < 0.01). A less close correlation was found between urinary mutagenic activity and other indicators of tobacco smoke exposure, such as urinary cotinine (r = 0.504, P < 0.05), number of cigarettes smoked during the day of urine collection (r = 0.399, P < 0.05) and machine smoking-derived nicotine deliveries of the total number of cigarettes smoked (number of cigarettes multiplied by the nicotine content of each cigarette, as indicated by the manufacturer; r = 0.439, P < 0.05). These results suggest that the mutagenic activity of smokers' urine may be predicted by the urinary level of nicotine plus metabolites. The low degree of reliability of many presumptive indicators of exposure to tobacco smoke and the different urinary excretion kinetics of tobacco smoke mutagens with respect to cotinine (a frequently used biomarker for monitoring exposure to tobacco smoke) are both emphasized.

[The occupational risks in a company producing aluminum alloy wheels]. / Rischi lavorativi in una azienda produttrice di ruote in lega di alluminio.

An environmental hygiene study was carried out in a factory making aluminium alloy wheels via pressure moulding. Physical risk factors (noise and microclimate) and chemical risk factors (respirable dust, mineral oils, solvents, fluorides, formaldehyde, CO) were assessed. Analysis of the data showed that physical risk factors were prevalent, whereas chemical pollution was insignificant due to technical improvements made by the management in the course of several redesigns of the plants.

[Pollution by nitrous dioxide during diagnostic laparoscopy interventions]. / Inquinamento da protossido d'azoto durante interventi di laparoscopia diagnostica.

Here we have outlined the data relative to an environmental survey carried out in a laparoscopy clinic at the hospital of Padua, to determine the environmental concentration of nitrous dioxide (N2O) used for intra-abdominal inflation during diagnostic laparoscopy interventions. The data obtained have revealed cases of considerable N2O environmental pollution. This above all, due to spontaneous loss of gas during the intervention and to the lack of adequate ventilation systems.