Vargas-Poussou Rosa, Cochat Pierre, Le Pottier Nelly, Roncelin Isabelle, Liutkus Aurelia, Blanchard Anne, Jeunemaître Xavier
Département de Pédiatrie Médicale et Faculté de Médecine et de Pharmacie Rouen, Centre Hospitalier Universitaire de Rouen, Rouen, France.
Pediatr Nephrol. 2008 Jan;23(1):149-53. doi: 10.1007/s00467-007-0584-1. Epub 2007 Sep 26.
The etiologies of early onset nephrocalcinosis in consanguineous families include five major inherited recessive disorders: primary hyperoxaluria (PH), familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC), distal renal tubular acidosis (dRTA), hereditary hypophosphatemic rickets with hypercalciuria (HHRH) and antenatal Bartter syndrome. In this paper, we describe two girls from consanguineous parents with early onset nephrocalcinosis. Based on both clinical and biochemical assessment in combination with molecular genetics, we have shown that the etiology of nephrocalcinosis is different in each girl: one had FHHNC and her sister had dRTA.
原发性高草酸尿症(PH)、家族性低镁血症伴高钙尿症和肾钙质沉着症(FHHNC)、远端肾小管酸中毒(dRTA)、遗传性低磷性佝偻病伴高钙尿症(HHRH)和产前巴特综合征。在本文中,我们描述了两名来自近亲父母的早发性肾钙质沉着症女孩。基于临床和生化评估并结合分子遗传学,我们发现每个女孩肾钙质沉着症的病因不同:一个患有FHHNC,她的妹妹患有dRTA。