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惊厥与新生儿甲状旁腺功能减退揭示母体甲状旁腺功能亢进

[Convulsions and neonatal hypoparathyroidism revealing maternal hyperparathyroidism].

作者信息

Brisse F, Breton D, Gagey V, Chéron G

机构信息

Unité de pédiatrie générale, hôpital Necker-Enfants-Malades, Paris, France.

出版信息

Arch Pediatr. 1994 Mar;1(3):255-9.

PMID:7994333
Abstract

BACKGROUND

Severe prolonged hypocalcemia may occur in neonates whose parathyroid hormone production has been blocked by maternal hyperparathyroidism. This report describes such a case.

CASE REPORT

A 7 day-old girl was admitted suffering from dyspnea and repeated convulsions that had continued for 2 days. Her birthweight was 3,500 g and her height 50 cm. Her mother had been given an iron preparation and calcium during pregnancy. Clinical examination of the newborn at birth revealed a cleft palate and micrognathia. The baby had been given milk-formula plus ergocalciferol, 1,200 IU/day. At admission, the baby was hypotonic. Her serum total calcium was 1.27 mmol/l; total proteins 61 g/l; ionized calcium 1.1 mmol/l; phosphorus 2.14 mmol/l; intact PTH 21 pg/ml (N = 10-65) and 25(OH)D 8 ng/ml (N = 8-30). She was given intravenous calcium gluconate (1 g/m2/d), diazepam (0.5 mg/kg) rectally, intravenous phenobarbital (20 mg/kg) but intravenous phenytoin was needed to stop clinical and electrical seizures. Her blood calcium was normalized 5 days later. Her mother, who was clinically normal, had: total serum calcium: 2.72 and 2.77 mmol/l; total proteins: 71 g/l; phosphorus: 0.85 mmol/l; intact PTH: 73 pg/ml; 25(OH)D: 6 ng/ml; Ultrasonography showed an adenoma of the right parathyroid. Further studies on the baby showed no signs of Di George syndrome.

CONCLUSION

Neonatal hypocalcemia always requires investigation of both the infant and mother. Measurements of vitamin D metabolites and intact PTH are required to recognize maternal hyperparathyroidism.

摘要

背景

患有严重持续性低钙血症的新生儿,其甲状旁腺激素的分泌可能因母亲甲状旁腺功能亢进而受阻。本文报告了这样一个病例。

病例报告

一名7日龄女婴因呼吸困难和反复惊厥入院,惊厥持续了2天。她出生体重3500克,身高50厘米。其母亲在孕期服用过铁剂和钙剂。新生儿出生时的临床检查发现腭裂和小颌畸形。该婴儿一直食用添加了1200国际单位/天麦角钙化醇的配方奶。入院时,婴儿肌张力低下。其血清总钙为1.27毫摩尔/升;总蛋白61克/升;离子钙1.1毫摩尔/升;磷2.14毫摩尔/升;完整甲状旁腺激素21皮克/毫升(正常范围10 - 65),25(OH)D 8纳克/毫升(正常范围8 - 30)。给予静脉注射葡萄糖酸钙(1克/平方米/天),直肠给予地西泮(0.5毫克/千克),静脉注射苯巴比妥(20毫克/千克),但需要静脉注射苯妥英钠以停止临床和电惊厥发作。5天后她的血钙恢复正常。其母亲临床检查正常,血清总钙分别为2.72和2.77毫摩尔/升;总蛋白71克/升;磷0.85毫摩尔/升;完整甲状旁腺激素73皮克/毫升;25(OH)D 6纳克/毫升;超声检查显示右侧甲状旁腺腺瘤。对婴儿的进一步检查未发现Di George综合征的迹象。

结论

新生儿低钙血症总是需要对婴儿和母亲进行检查。需要检测维生素D代谢产物和完整甲状旁腺激素以识别母亲甲状旁腺功能亢进。

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Hypocalcemic tetany in the newborn as a manifestation of unrecognized maternal primary hyperparathyroidism.新生儿低钙血症性手足搐搦作为未被识别的母亲原发性甲状旁腺功能亢进的一种表现。
Wien Klin Wochenschr. 2007;119(3-4):129-31. doi: 10.1007/s00508-006-0748-1.
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Rare presentation of maternal primary hyperparathyroidism.
妊娠合并原发性甲状旁腺功能亢进症的罕见表现。
BMJ. 2000 Jul 22;321(7255):223-4. doi: 10.1136/bmj.321.7255.223.