小儿肾性骨病40例的临床分析

A CLINICAL ANALYSIS OF RENAL BONE DISEASE IN CHILDREN (40 CASES)

本文报道40例小儿肾性骨病(RBD)。原发肾脏病为慢性肾功能衰竭(CRF)6例;肾小管性酸中毒(RTA)18例;范可尼综合征(FS)3例;低血磷性抗维生素D佝偻病(VDRR)13例。临床表现:生长落后90％;除CRF患儿外均有骨骼畸形;AKP增高者92.5％;VDRR及FS患儿均有低磷血症,RTA患儿中有77.78％;CRF患儿中高磷血症占66.67％,低钙血症为45％。长骨X片检查:82.5％的患儿显示活动性佝偻病征象,以骨质疏松最多见,也有骨龄落后、骨折和个别的骨膜下骨质吸收。

Renal bone disease (EBD) is one of the most common metabolic bone disorders in children. This article reports an analysis of 40 patients with EBD. The primary renal diseases were chronic renal failure (ORF) 6, renal tubular disease with acidosis (ETA) 18, the Fanconi syndrome (FS) 3, and vitamine D resistant rickets (VDER) 13. 90% of patients suffering from EBD exhibit growth retardation. All excepting CRF, Show various degrees of bone abnormalities. 92.5% show high AKP. Hypophosphatemia occurred in 77.78% of ETA. But 66.67% of OEF patients were hyperphosphatemic. 45% of the patients had reduced serum calcium levels. 82.5% patients showed long bone deformities by X-ray examination, osteoporosis was most commonly seen. The distal end of redias and ulra appeared pathological fractures (7 cases), retardation of bone age (5 cases), rickets lung (5 cases), subperiosteal bone absorption (2 cases).Conclusion; (1). The most common primary disease in children with EBD are hereditary renal tubular disease and congenital renal defects. Glomerulonep-hritis is rare. (2). EBD in children is frequently misdiagnosed due to predominant Signs and symptoms of the primary renal disease. Before 3 years of age, patient are often treated as vitamine D deficiency. Usually resulting in retardation of growth and development and bone deformities even fracture occur. (3). Severity of bone deformity is related to the age of onset and the duration of the disorder. (4). X-ray manifestations of rickitic bone abnormality, cystic fibrosis or ostitis is rare. (5). Treatment includes: (1). 1.25(OH)2D3 or 1α-OH-D3 therapy. (2). Treatment of the primary renal disease.