Rossi R, Gödde A, Kleinebrand A, Rath B, Jürgens H
University Children's Hospital, Münster, Germany.
Ren Fail. 1995 Sep;17(5):551-7. doi: 10.3109/08860229509037619.
Diabetes insipidus renalis has only occasionally been reported in ifosfamide-induced renal Fanconi's syndrome, but in two studies on subclinical renal impairment, low morning urine osmolarity was found in high frequencies. This study was performed to assess the frequency of defective concentrating capacity in patients with ifosfamide-induced renal Fanconi's syndrome or severe impairment of proximal tubular function.
Seven patients with overt Fanconi's syndrome and 5 with a generalized but subclinical tubulopathy were examined. Beside proximal tubular solute reabsorption and estimation of glomerular filtration rate, urinary osmolarity was measured after overnight fast and DDAVP (1-D-amino-8-D-arginine vasopressin) testing.
Five out of 7 patients with overt Fanconi's syndrome, but no patient with only subclinical tubular damage, had decreased osmolarities by at least one test. Increased sodium excretion was additionally found in 4 of these patients.
Impaired renal concentrating capacity is a rare event in ifosfamide-induced renal dysfunction and confined to patients with overt Fanconi's syndrome. These patients should, however, undergo evaluation of concentrating capacity and renal sodium handling as decreased concentrating capacity and increased sodium excretion would render a patient at risk for dehydration episodes.
肾性尿崩症仅偶尔在异环磷酰胺诱导的肾性范科尼综合征中被报道，但在两项关于亚临床肾功能损害的研究中，发现晨尿渗透压降低的频率较高。本研究旨在评估异环磷酰胺诱导的肾性范科尼综合征或近端肾小管功能严重损害患者中浓缩功能缺陷的频率。
对7例显性范科尼综合征患者和5例广泛性但亚临床肾小管病患者进行了检查。除了近端肾小管溶质重吸收和肾小球滤过率评估外，还在过夜禁食和去氨加压素（1-D-氨基-8-D-精氨酸加压素）试验后测量了尿渗透压。
7例显性范科尼综合征患者中有5例至少一项试验显示渗透压降低，但仅亚临床肾小管损伤的患者中无此情况。这些患者中有4例还发现钠排泄增加。
在异环磷酰胺诱导的肾功能障碍中，肾浓缩功能受损是罕见事件，且仅限于显性范科尼综合征患者。然而，这些患者应进行浓缩功能和肾钠处理评估，因为浓缩功能降低和钠排泄增加会使患者有发生脱水发作的风险。
