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激素抵抗性(假性)甲状旁腺功能减退症患者的肾脏对甲状旁腺激素的反应

Renal responses to PTH in patients with hormone-resistant (pseudo) hypoparathyroidism.

作者信息

Moses A M, Breslau N, Coulson R

出版信息

Am J Med. 1976 Aug;61(2):184-9. doi: 10.1016/0002-9343(76)90168-6.

DOI:10.1016/0002-9343(76)90168-6
PMID:181985
Abstract

Five patients with pseudohypoparathyroidism were compared to normal subjects and patients with hypoparathyroidism in their ability to respond to the infusion of parathyroid hormone (PTH) by altering excretion of calcium, sodium, potassium, phosphate and bicarbonate. In patients with pseudohypoparathyroidism, impairment in renal responses to PTH was more generalized than has been recognized. The patterns of response varied from patient to patient. The most commonly observed abnormality, aside from lack of increase in urinary cyclic adenosine 5'-monophosphate (AMP) was failure to decrease the calcium to sodium clearance ratio, and indication of impaired renal calcium reabsorption. The responses which most closely approximated normal, including a normal decrease in the calcium to sodium clearance ratio, occurred in a patient (Case 1) who had the largest, although impaired, response in cyclic AMP excretion. Conversely, the most abnormal responses occurred in three patients (Cases 2, 4 and 5) who had the smallest increases in cyclic AMP excretion after the administration of PTH. The impaired renal reabsorption of calcium after the administration of PTH (lack of decrease in calcium to sodium clearance ratio) may, when present, be in part responsible for hypocalcemia.

摘要

将五名假性甲状旁腺功能减退患者与正常受试者及甲状旁腺功能减退患者进行比较,观察他们在静脉输注甲状旁腺激素(PTH)后,通过改变钙、钠、钾、磷酸盐和碳酸氢盐的排泄来做出反应的能力。在假性甲状旁腺功能减退患者中,肾脏对PTH反应的损害比之前所认识到的更为普遍。患者之间的反应模式各不相同。除了尿中环磷酸腺苷(AMP)不增加外,最常观察到的异常是钙钠清除率比值未能降低,这表明肾脏对钙的重吸收受损。最接近正常的反应,包括钙钠清除率比值正常下降,出现在一名患者(病例1)身上,该患者虽然环磷酸腺苷排泄反应受损,但却是最大的。相反,最异常的反应出现在三名患者(病例2、4和5)身上,他们在注射PTH后环磷酸腺苷排泄增加最少。注射PTH后肾脏对钙的重吸收受损(钙钠清除率比值未降低),如果存在这种情况,可能部分导致低钙血症。

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J Med Genet. 1981 Dec;18(6):431-5. doi: 10.1136/jmg.18.6.431.
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Calcif Tissue Int. 1987 Nov;41(5):262-6. doi: 10.1007/BF02555227.
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