Saito H, Saito M, Saito K, Terauchi A, Kobayashi T, Tominaga T, Hosoi E, Senoo M, Saito K, Saito T
Department of Internal Medicine, Saito Hospital, Tokushima, Japan.
Am J Med Sci. 1989 Apr;297(4):247-50. doi: 10.1097/00000441-198904000-00010.
Patients with latent disorders of hormone response mechanism are rarely found. This paper reports a case of subclinical pseudohypoparathyroidism type II in which physiological adjustment of calcium (Ca) metabolism became insufficient only in the second half of pregnancy. A 34-year-old woman examined for a slight bruise on the head was incidentally found to have marked intracranial calcification and a full set of false teeth. From her history of past pregnancy, it was revealed that she suffered from symptoms of hypocalcemia during late gestation (serum total Ca level, 4.8-6.4 mg/dl), which disappeared spontaneously after delivery. When the woman was not pregnant, although only the total Ca level was slightly below the normal range (7.7-8.4 mg/dl), the serum ionized Ca, phosphorus (P), magnesium, 1,25-dihydroxycholecalciferol and 24,25-hydroxycholecalciferol levels, plasma parathyroid hormone (PTH) level and urinary excretion of Ca were all normal without treatment. Intravenous infusion of 30 mg/kg EDTA-2Na resulted in marked elevation of plasma PTH associated with significant reduction of serum ionized Ca. In contrast, although her urinary excretion of phosphorous per hour was within the normal range in the basal state, she showed no proportional change in urinary phosphorous excretion with increase in urine cyclic AMP induced by administration of PTH(1-34). From these findings, she was diagnosed as having an incomplete form of pseudohypoparathyroidism Type II. This abnormality seems to be rare, but we consider that the present observations provide important information for preventive care of pregnant women and fetuses during gestation.
激素反应机制潜在紊乱的患者很少见。本文报告了一例II型亚临床假性甲状旁腺功能减退症病例，该患者仅在妊娠后半期钙（Ca）代谢的生理调节变得不足。一名34岁女性因头部轻微擦伤接受检查时，偶然发现有明显的颅内钙化和一副假牙。从她过去的妊娠史来看，发现她在妊娠晚期出现低钙血症症状（血清总钙水平为4.8 - 6.4mg/dl），产后自行消失。该女性未怀孕时，虽然仅总钙水平略低于正常范围（7.7 - 8.4mg/dl），但血清离子钙、磷（P）、镁、1,25 - 二羟胆钙化醇和24,25 - 二羟胆钙化醇水平、血浆甲状旁腺激素（PTH）水平以及尿钙排泄量在未治疗时均正常。静脉输注30mg/kg乙二胺四乙酸二钠（EDTA - 2Na）导致血浆PTH显著升高，同时血清离子钙显著降低。相比之下，虽然她基础状态下每小时尿磷排泄量在正常范围内，但给予甲状旁腺激素（1 - 34）诱导尿中环磷酸腺苷（cAMP）增加时，尿磷排泄量未呈现相应变化。根据这些发现，她被诊断为不完全型II型假性甲状旁腺功能减退症。这种异常似乎很罕见，但我们认为目前的观察结果为妊娠期孕妇和胎儿的预防保健提供了重要信息。
