F Zheng1, S Zhao, X Li. 1. Division of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China.
Abstract
OBJECTIVE: To recognize the etiologies, clinical, and biochemical manifestations of Fanconi syndnome (FS). METHODS: 42 patients with FS were analyzed retrospectively. RESULTS: 21 cases were idiopathic in 42 (50%) cases, and the other 21 (50%) cases were acquired (sjogren's 5, and interstitial nephritie 8). The patients were characterized by proximal renal tubular acidosis (n = 41) and multiple renal tubular transport dysfunctions, including hypokalemia (n = 21), hypophorphatemia (n = 29), hypourecemia (n = 19), renal glucosuria (n = 38), aminoaciduria (n = 36), low-molecular-weight proteinuria (n = 21). The clinical manifestations commonly presented with muscle weakness, polydipsia, polyuria and renal bone diseases. 18 patients presented with impaired renal function. Renal pathohistological studied in 14 patients showed renal tubulointerstitial changes of different degrees in all cases, and glomerular changes in 4. CONCLUSIONS: The etiology of FS is various and secondary FS is not uncommon. FS usually present proxmal RTA and renal tubulointerstitial lesions in pathology.
OBJECTIVE: To recognize the etiologies, clinical, and biochemical manifestations of Fanconi syndnome (FS). METHODS: 42 patients with FS were analyzed retrospectively. RESULTS: 21 cases were idiopathic in 42 (50%) cases, and the other 21 (50%) cases were acquired (sjogren's 5, and interstitial nephritie 8). The patients were characterized by proximal renal tubular acidosis (n = 41) and multiple renal tubular transport dysfunctions, including hypokalemia (n = 21), hypophorphatemia (n = 29), hypourecemia (n = 19), renal glucosuria (n = 38), aminoaciduria (n = 36), low-molecular-weight proteinuria (n = 21). The clinical manifestations commonly presented with muscle weakness, polydipsia, polyuria and renal bone diseases. 18 patients presented with impaired renal function. Renal pathohistological studied in 14 patients showed renal tubulointerstitial changes of different degrees in all cases, and glomerular changes in 4. CONCLUSIONS: The etiology of FS is various and secondary FS is not uncommon. FS usually present proxmal RTA and renal tubulointerstitial lesions in pathology.