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印度北部儿童肾钙质沉着症的病因

Etiology of nephrocalcinosis in northern Indian children.

作者信息

Mantan Mukta, Bagga Arvind, Virdi Virenderjeet Singh, Menon Shina, Hari Pankaj

机构信息

Department of Pediatrics, Maulana Azad Medical College and Associated Hospitals, New Delhi, India.

出版信息

Pediatr Nephrol. 2007 Jun;22(6):829-33. doi: 10.1007/s00467-006-0425-7. Epub 2007 Feb 7.

Abstract

This retrospective survey examines the etiology of nephrocalcinosis (NC) in 40 patients (26 boys), over an 8-year period. The median age at onset of symptoms and presentation was 36 months and 72 months, respectively. Clinical features included marked failure to thrive (82.5%), polyuria (60%) and bony deformities (52.5%). The etiology of NC included distal renal tubular acidosis (RTA) in 50% patients and idiopathic hypercalciuria and hyperoxaluria in 7.5% each. Other causes were Bartter syndrome, primary hypomagnesemia with hypercalciuria, severe hypothyroidism and vitamin D excess. No cause for NC was found in 12.5% patients. Specific therapy, where possible, ameliorated the biochemical aberrations, although the extent of NC remained unchanged. At a median (range) follow up of 35 (14-240) months, glomerular filtration rate (GFR) had declined from 82.0 (42-114) ml/min per 1.73 m2 body surface area to 70.8 (21.3-126.5) ml/min per 1.73 m2 body surface area (P = 0.001). Our findings confirm that, even with limited diagnostic facilities, protocol-based evaluation permits determination of the etiology of NC in most patients.

摘要

这项回顾性调查研究了40例患者(26名男孩)在8年期间肾钙质沉着症(NC)的病因。症状出现和就诊时的中位年龄分别为36个月和72个月。临床特征包括明显生长发育迟缓(82.5%)、多尿(60%)和骨骼畸形(52.5%)。NC的病因包括50%的患者为远端肾小管酸中毒(RTA),7.5%的患者为特发性高钙尿症和高草酸尿症。其他病因包括巴特综合征、原发性低镁血症伴高钙尿症、严重甲状腺功能减退和维生素D过量。12.5%的患者未发现NC的病因。在可能的情况下,特异性治疗改善了生化异常,尽管NC的程度保持不变。在中位(范围)35(14 - 240)个月的随访中,肾小球滤过率(GFR)从每1.73平方米体表面积82.0(42 - 114)ml/min降至每1.73平方米体表面积70.8(21.3 - 126.5)ml/min(P = 0.001)。我们的研究结果证实,即使诊断设备有限,基于方案的评估也能在大多数患者中确定NC的病因。

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