Abstract
104 children with IDDM were examined for induration and thickening of the skin and for joint contractures. Five patients had both multiple joint involvement and skin changes; 3 were studied in detail. Their diabetes ranged from 7-16 years in duration and was characterized by short stature, hepatomegaly, 4 to 6 hospitalizations each year, and glucosylated Hgb levels of 12-16%. All 3 had restrictive pulmonary lung disease. Histopathology of skin biopsies (bxs) demonstrated increased accumulation of collagen in the lower dermis. Three skin bxs were homogenized and compared to 6 bxs from age matched controls. The extractability of collagen in 0.5 N acetic acid was decreased to about ½ normal in 2 of the patients, suggesting increased cross-linkage of collagen. The amount of hexose bound to protein in a ketoamine linkage was assayed as the 5-hydroxymethylfurfural derivative. In patients, the mean non-enzymatic glucosylation was 4.0 (range 0.9-8.5) ng hexose/ug hydroxyproline which was 13 times that of controls (mean=0.3, range 0.2-0.4, ng hexose/ug hydroxyproline). The results suggest that non-enzymatic glucosylation may alter the packing, cross-linking and turnover of collagen, thus contributing to the development of a scleroderma-like cutaneous syndrome in IDDM.
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Buckingham, B., Uitto, J., Sandborg, C. et al. 1103 SCLERODERMA-LIKE SYNDROME AND THE NON-ENZYMATIC GLUCOSYLATION OF COLLAGEN IN CHILDREN WITH POORLY CONTROLLED INSULIN DEPENDENT DIABETES (IDDM). Pediatr Res 15 (Suppl 4), 626 (1981). https://doi.org/10.1203/00006450-198104001-01129
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DOI: https://doi.org/10.1203/00006450-198104001-01129
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