Neonatal alveolar rhabdomyosarcoma with skin and brain metastases.

BACKGROUND: Approximately 5-10% of patients with rhabdomyosarcomas (RMS) are diagnosed during the first year of life, and their clinical characteristics have been well documented. However, because RMS rarely occurs during the neonatal period, little is known about neonatal RMS. METHODS: Four patients with neonatal RMS were treated at St. Jude Children's Research Hospital between 1962 and 1999. The authors report the results of a review of these patients and of cases described in the literature. Clinical, radiologic, and pathologic features of these patients and their outcomes were evaluated. RESULTS: One patient with embryonal RMS was treated successfully with a combination of systemic chemotherapy and local control measures. The other three patients had alveolar RMS. Two of them had multiple skin and subcutaneous metastatic nodules at the time of diagnosis and developed brain metastases early in their course. In one of these patients, the PAX3-FKHR fusion transcript was detected. Three other similar cases of neonatal alveolar RMS with metastases to the skin and brain have been reported in the literature. CONCLUSIONS: A distinct syndrome of neonatal RMS is described. This syndrome is characterized by alveolar histology, multiple skin and subcutaneous metastases, and fatal outcome as the result of early brain metastasis.

An evaluation of the significance of mouth and hand contamination for lead absorption in lead-acid battery workers.

The present study was conducted to evaluate the role of ingestion through hand and mouth contamination in the absorption of lead in 25 lead-acid battery workers. Levels of personal exposure to airborne lead ranged from 0.004 to 2.58 mg/m3 [geometric mean 0.098, with 25% of samples exceeding threshold limit values (ACGIH) of 0.15 mg/m3]; the mean (SD) blood lead level was 48.9 (10.8) micrograms/dl. Mean hand lead contents increased 33-fold from preshift levels on Monday mornings (33.5 micrograms/500 ml) to midshift levels on Thursday afternoons (1121 micrograms/500 ml). Mouth lead contents increased 16-fold from 0.021 micrograms/50 ml on Mondays to 0.345 micrograms/50 ml on Thursdays. The typical Malay racial habit of feeding with bare hands and fingers without utensils (closely associated with mouth and hand lead levels on Mondays) explained the bulk of the variance in blood lead levels (40%), with mouth lead on Thursdays (closely associated with poor personal hygiene) explaining a further 10%. Air lead was not a significant explanatory variable. The implementation of a programme of reinforcing hand-washing and mouth-rinsing practices resulted in a reduction of the blood lead level by 11.5% 6 months later. These results indicate that parenteral intake from hand and mouth contamination is an important cause of lead absorption in lead-exposed workers.

Dehydration from outdoor work and urinary stones in a tropical environment.

A questionnaire survey was carried out to determine the prevalence of urinary stone disease in 406 male workers in several occupations. There were 119 quarry drilling and crusher workers (outdoor, physically active), 77 quarry truck and loader drivers (outdoor, physically inactive), 92 postal deliverymen (outdoor, physically active), 75 postal clerks (indoor, physically inactive), and 43 hospital maintenance workers (indoor, physically active). The prevalence of urinary stone disease was five times higher in outdoor workers (5.2 per cent) compared to indoor workers (0.85 per cent, P less than 0.05). Contrary to expectation, no increased risk of urolithiasis was apparent in physically inactive workers. Chronic dehydration is likely to be the most important risk factor for the increased risk of urolithiasis in outdoor workers in the tropics, and should be easily prevented by increased water intake.