Abstract
Sweat tests by pilocarpine iontophoresis were performed on 13 children age 2-9 years with nephropathic cystinosis; controls were 100 children age 1-18 years examined consecutively because of respiratory symptoms or failure to thrive, but not having cystic fibrosis. The mean amount of sweat for the cystinotic children was 97 mg ± 35 SEM (range 25-450) compared with 281 mg ± 12 SEM (range 86-587) for 100 controls (p<0.001). Ten of 13 cystinotics, but only 3 of 100 controls, had sweat volumes less than 100 mg. One 12-year old benign cystinotic produced 420 mg of sweat. For 5 nephropathic cystinotics from whom an adequate amount of sweat for analysis was obtained, sweat chlorides were normal (range 15-23 mEq/1). Sweat volume was not related to the patient's age or history of cysteamine therapy. Symptoms did correlate with decreased sweat production. Eight cystinotic children, with sweat volumes of 25-87 mg each, experienced severe flushing and vomiting or practiced heat avoidance, while the remaining five (mean sweat volume 188 mg) had no clinically significant heat intolerance. Light microscopy of several skin biopsies revealed no sweat gland abnormalities. Cystinosis now joins fucosidosis as a lysosomal storage disorder with sweat abnormalities. Precautions against excessive heat exposure should be considered for children with nephropathic cystinosis.
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Gahl, W., Hubbard, V. & Orloff, S. DECREASED SWEAT VOLUME IN CYSTINOSIS. Pediatr Res 18 (Suppl 4), 221 (1984). https://doi.org/10.1203/00006450-198404001-00767
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DOI: https://doi.org/10.1203/00006450-198404001-00767