Clinical Study of Pseudohypoparathyroidism (PHP Type I, II) and Nephrogenic Diabetes Inspidus

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31 years old male with a long history of seizure showed marked hypocalcemia, but no increase of urinary c-AMP and phosphate excretion in response to exogenously administered parathyroid extract (PTE). This is the case termed as pseudohypoparathyroidism (PHP) Type I. In contrast with the case of PHP Type I, 22 years old female diagnosed as SjÖgren's syndrome and PHP Type II had a relatively mild hypocalcemia and demonstrated a marked rise of urinary c-AMP, but no increase of phosphate excretion in response to PTE. In both cases, 200 U PTE administration for 4 days had no effect on serum Ca and P. Acute infusion of calcium, immediately followed by PTE administration restored normal renal responsiveness to parathyroid hormone, e.g. the increase of urinary c-AMP and phosphate in both types. The patient ofPHP Type I received calcium and Vi D therapy and serum Ca increased to 7mg%. Under these conditions DBc-AMP of PTEwts given with the results of reappearance of PTH like action. DBc-AMP infusion induced the similar effects in patient ofPHP Type II, though she was not treated. In our case ofKIP Type I, it can be thought that the combined Ca-PTH infusion may have lead to activation of c-AMP generating system and enhancement ofCa++ influx, resulting in stimulation of the rather poorly defined process between c-AMP generation and the physiological effects of hormone. Thus addition of PTH or DBc-AMP under the condition that serum Ca was improved by the treatment may have induced at least qualitatively similar effects In PHP Type II, c-AMP generation system is intact, but the defect represents a failure of intracellular receptor of c-CMP. The fact that the combinedCa-PTH infusion induced renal responsiveness to PTH may also have resulted from improvement in the calcium environment of the cell. Though it is difficult to explain the reason why endogenously generated c-AMP had no effect, but DBc-AMP had on phosphaturia, it might be speculated that DBc-AMP may have much more strongeraffinity for intracellular receptor site of c-AMP. At any rate, these results described above clearly suggest that the nature of the metabolic defect in patients withPHP Type I and II is not genetically determined irreversible disorder, but rather functional one which can be restored by the special conditions. 18 years old male diagnosed as nephrogenic diabetes inspidus (N. D. I.) has had polydypsia and polyuia (daily urine output 8-10 l, urine osmolality 100-150mOsm/l) since childhood. Administration of vasopression, tolubtamide and clofibrate has had no effect, but thiazide cleary increased urine osmolality and decreased urine output. There was no increase of urinary c-AMP by vasopression infusion. Whether N. D. I. can be separated into such subtypes as PHP or hormonal effect may be restored by special conditions are still remained to be determined in future.
Japan Society of Clinical Chemistryの論文

Clinical Study of Pseudohypoparathyroidism (PHP Type I, II) and Nephrogenic Diabetes Inspidus

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