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骨质疏松症合并肾小管性酸中毒及干燥综合征

Osteoporosis Complicating Renal Tubular Acidosis in Association With Sjogren's Syndrome.

作者信息

Furqan Saira, Banu Sabiha, Ram Nanik

机构信息

Endocrinology, Aga Khan University Hospital, Karachi, PAK.

出版信息

Cureus. 2021 Sep 29;13(9):e18373. doi: 10.7759/cureus.18373. eCollection 2021 Sep.

DOI:10.7759/cureus.18373
PMID:34725619
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8554731/
Abstract

Chronic metabolic acidosis increases alkali mobilization from the bone and promotes the development of osteoporosis. We report the case of a 35-year-old Caucasian female who presented for surgical fixation of a left femoral fracture sustained six months previously from a ground level fall with known primary hypothyroidism (for 12 years, on thyroxine replacement) with history of hypokalemia for the last 13 years (on regular oral potassium supplements). There was no history of fracture in past. There was no history of renal stones. There was positive history of hypokalemic periodic paralysis twice in past (12 and 13 years back). There was no history of recurrent oral or ocular ulcers. On examination there was no uveitis, oral ulcers, lacrimal or parotid gland enlargement. Upon workup the patient was diagnosed with left-sided femur fracture (neck) and was admitted for surgical management. She underwent left dynamic hip screw fixation under general anesthesia which she tolerated well. Upon further workup she had normal anion gap with hyperchloremic metabolic acidosis, low vitamin D level and dual-energy x-ray absorptiometry (DEXA) scan revealed osteoporosis at femur and hip joint. Vitamin D was replaced, she was started on Ibandronate and calcium supplements. Her further workup revealed positive anti-SSA. Our final clinical diagnosis in this case is possible or incomplete Sjogren's syndrome causing type-1 renal tu-bular acidosis (RTA; distal RTA) with osteoporosis.

摘要

慢性代谢性酸中毒会增加骨骼中的碱动员,并促进骨质疏松症的发展。我们报告了一例35岁的白种女性病例,她因六个月前从地面摔倒导致的左股骨骨折前来接受手术固定,已知患有原发性甲状腺功能减退症(12年,接受甲状腺素替代治疗),过去13年有低钾血症病史(定期口服钾补充剂)。既往无骨折史。无肾结石病史。既往有两次低钾性周期性麻痹病史(分别在12年和13年前)。无复发性口腔溃疡或眼部溃疡病史。检查时无葡萄膜炎、口腔溃疡、泪腺或腮腺肿大。经检查,患者被诊断为左侧股骨骨折(颈部),并入院接受手术治疗。她在全身麻醉下接受了左动力髋螺钉固定术,耐受性良好。进一步检查发现她的阴离子间隙正常,伴有高氯性代谢性酸中毒,维生素D水平低,双能X线吸收法(DEXA)扫描显示股骨和髋关节骨质疏松。补充了维生素D,开始使用伊班膦酸钠和钙剂。进一步检查发现抗SSA阳性。本例最终临床诊断为可能的或不完全性干燥综合征导致1型肾小管酸中毒(RTA;远端RTA)并伴有骨质疏松症。

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Osteomalacia complicating renal tubular acidosis in association with Sjogren's syndrome.骨软化症合并肾小管酸中毒及干燥综合征
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