CHRONIC IDIOPATHIC HYPOPARATHYROIDISM WITH SUPERIMPOSED ADDISONS DISEASE IN A CHILD
CHRONIC IDIOPATHIC HYPOPARATHYROIDISM WITH SUPERIMPOSED ADDISONS DISEASE IN A CHILD
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DOI：
10.1210/jcem-6-7-493
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发表时间：
1946-01-01
影响因子：
5.8
通讯作者：
LEONARD, MF
中科院分区：
文献类型：
--
作者：
LEONARD, MF
34例原发性甲状旁腺功能减退症中，仅2例伴有肾上腺皮质功能不全，无病理报告。这促使报告的情况下，合并甲状旁腺和肾上腺不足，在91/2年。一个白色老女孩她在3岁时开始出现手足抽搐体征，并伴随皮肤、牙齿和毛发的进行性营养变化。她表现出特征性的血清钙降低和血清磷升高，在甲状旁腺激素注射后逆转，也没有佝偻病或肾病的迹象逐渐增加的皮肤色素沉着和降低的血压之前的急性肾上腺皮质功能不全的危象在11 l/2 yrs. The临床条件和低血清钠改善肾上腺皮质提取物和氯化钠。至死不渝，七年不渝。后来，需要甲状旁腺和肾上腺皮质治疗。尸检显示原发性肾上腺萎缩，肾上腺皮质完全破坏，伴随皮肤色素沉着过度，内脏淋巴组织普遍肿大，子宫、输卵管和卵巢发育不全，垂体无嗜碱性细胞;仔细检查无甲状旁腺;肺充血和水肿;下丘脑区轻度脑膜脑炎。实验文献资料提示肾上腺皮质和甲状旁腺分泌物之间存在拮抗关系，但由于治疗方法的差异，无法证实。该患者表现出的一些不太常见的甲状旁腺功能减退并发症是颅内压升高伴视乳头水肿在特定治疗下消退、弥漫性颅内钙化和严重的顽固性角结膜炎。
Of 34 published cases of well established idiopathic hypoparathyroidism, only 2 showed associated adrenal insufficiency, neither with pathologic report. This prompted the reporting of a case of combined parathyroid and adrenal deficiency in a 91/2 yr. old white girl. Her signs of tetany began at 3 yrs. and were accom- panied by progressive trophic changes in skin, teeth, and hair. She showed characteristic lowering of serum Ca and elevation of serum P with reversal after parathormone injn., and no evidence of rickets or renal disease. Gradually increasing skin pigmentation and diminishing blood pressure preceded a crisis of acute adrenal insufficiency at 11l /2 yrs. The clinical condition and low serum Na were improved by adrenal cortical extract and NaCl. Thereafter, until death 7 mos. later, both parathyroid and adrenal cortical therapy were required. Necropsy revealed primary atrophy of the adrenal glands with complete destruction of adrenal cortex, with attendant hyperpigmentation of skin, generalized enlargement of visceral lymphoid tissue, hypoplasia of uterus, tubes and ovaries, and absence of basophilic cells of pituitary; absence of parathyroid glands on careful search; congestion and edema of lungs; and slight meningoencephalitis of the hypothalamic region. Exptl. data from the literature suggest an antagonistic relationship between adrenal cortical and parathyroid secretions, but because of variations in treatment, no corroborative evidence could be evinced here. Some of the less common complications of hypoparathyroidism manifested by this patient were increased intracranial pressure with papilledema regressing under specific therapy, diffuse intracranial calcification, and severe intractible keratoconjunc-tivitis.