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[Current function tests in hypoparathyroidism in children].

E Mallet, B Gugi, J P Basuyau

    Annales D'Endocrinologie
    |January 1, 1983
    PubMed
    Summary
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    Measuring parathormone levels and their effects is crucial for diagnosing endocrine disorders. This study highlights differences in parathormone receptor sensitivity in pseudohypoparathyroidism and hypoparathyroidism.

    Area of Science:

    • Endocrinology
    • Biochemistry
    • Molecular Biology

    Context:

    • Direct measurement of circulating parathormone (PTH) and assessment of its renal effects via cyclic adenosine monophosphate (cAMP) assays are clinically available.
    • Radio-immunoassay techniques for PTH utilize antisera with amino-terminal or carboxy-terminal specificity.
    • Amino-terminal specificity reflects biologically active PTH, while carboxy-terminal specificity aids in detecting hormonal hypersecretion in pseudohypoparathyroidism due to fragment survival.

    Purpose:

    • To evaluate the sensitivity of renal and bone receptors to parathormone in children with pseudohypoparathyroidism and hypoparathyroidism.
    • To compare the response to exogenous parathormone in pseudohypoparathyroid children versus normal and hypoparathyroid children.

    Summary:

    • The Ellsworth-Howard test, measuring plasma cAMP gradient after exogenous PTH injection, showed a complete absence of response in pseudohypoparathyroid children.

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  • Hypoparathyroid children exhibited a rapid cAMP response, with levels peaking at 10 minutes and increasing up to 20-fold baseline.
  • Assessing bone receptor sensitivity through serum calcium and urinary hydroxyproline changes post-PTH injection proved difficult to interpret.

    • Impact:

    • This research provides insights into the distinct mechanisms of parathormone resistance in pseudohypoparathyroidism.
    • Understanding receptor sensitivity is vital for accurate diagnosis and management of parathormone-related disorders.
    • The findings underscore the utility of cAMP assays in differentiating endocrine conditions affecting calcium homeostasis.