Abstract
From the metabolic standpoint, several features of RJS implicate a role for cAMP in the pathophysiology of this disease. ↑ plasma cAMP is found in RJS to corroborate this possibility. In the presence of a normal degrading system, cAMP should not be expected to be ↑ in the urine. However, 25% of cases have ↑ cAMP excretion. Since PTH and ADH are both known to ↑ cAMP production by the cells of the renal tubules, these hormones were measured in plasma, retrospectively, in untreated RJS patients. ↑ ADH and PTH levels were found which correlated with the severity of the disease. In two patients, a rapid decline in the PTH levels was seen in 3-5 hours. No significant changes in serum Ca, Mg, PO4, and osmolality were noted. These findings add two more hormones in addition to insulin, glucagon, ACTH, cortisol, GH, prolactin, and the catecholamines which are increased in the complexity of findings in RJS. A model which could accommodate some of these findings and lend insight to a possible mechanism involving Ca translocation whereby an underlying membrane lesion might be further aggravated by these abnormalities is proposed.
This work was supported in part by a grant from the USPHS, NIH HD 11657.
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Kano, E., Palmieri, G., Schwenzer, K. et al. 1139 PARATHYROID (PTH) AND ANTIDIURETIC HORMONE (ADH) IN REYE-JOHNSON SYNDROME (RJS). Pediatr Res 15 (Suppl 4), 632 (1981). https://doi.org/10.1203/00006450-198104001-01165
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DOI: https://doi.org/10.1203/00006450-198104001-01165
