Mantan Mukta, Bagga Arvind, Virdi Virenderjeet Singh, Menon Shina, Hari Pankaj
Department of Pediatrics, Maulana Azad Medical College and Associated Hospitals, New Delhi, India.
Pediatr Nephrol. 2007 Jun;22(6):829-33. doi: 10.1007/s00467-006-0425-7. Epub 2007 Feb 7.
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.
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。