Yoshida H, Akikusa B, Saeki N, Hasegawa S, Iesato K, Yamamoto S, Murotani N, Wakashin M, Shimada T
Departments of Pathology, Neurosurgery, and Postgraduate Medical Center, Chiba Social Insurance Hospital, Chiba, Japan.
Am J Kidney Dis. 1999 Jun;33(6):1158-63. doi: 10.1016/S0272-6386(99)70156-3.
A case of nephrotic syndrome complicated by acromegaly is presented. The first renal biopsy specimen showed minor glomerular abnormalities with glomerular hypertrophy, corresponding with minimal change nephrotic syndrome. Corticosteroid therapy led to a partial remission, followed by frequent relapses after reduction of the drug. A diagnosis of atypical focal segmental glomerulosclerosis (FSGS) was made based on the second renal biopsy results 6 months after the first. We combined steroid therapy with the administration of an anticoagulant, cytotoxic agents, angiotensin-converting enzyme inhibitor, and low-density lipoprotein adsorption. Except for the angiotensin-converting enzyme inhibitor, these medications were not effective in terms of allowing a reduction in the high dosage of steroid, which in turn threatened progressive osteoporosis and lumbar vertebrae fracture. Administering the steroid at a moderate dosage, treatment was focused on the complicating acromegaly from pituitary microadenoma. Subcutaneous injections of octreotide acetate, a somatostatin analogue, reduced proteinuria and increased urine volume. Subsequent transsphenoidal microsurgery of the adenoma resulted in the normalization of the elevated creatinine clearance and the further reduction in steroid dosage while maintaining a remission state. This is the first reported clinical case with acromegaly followed by FSGS, and it is suggested that hypersecretion of growth hormone participates in the development and progression of glomerular disease.
本文报告一例肾病综合征合并肢端肥大症的病例。首次肾活检标本显示肾小球轻度异常伴肾小球肥大,符合微小病变型肾病综合征。糖皮质激素治疗使病情部分缓解,但药物减量后频繁复发。根据首次肾活检6个月后的第二次肾活检结果,诊断为非典型局灶节段性肾小球硬化(FSGS)。我们将糖皮质激素治疗与抗凝剂、细胞毒性药物、血管紧张素转换酶抑制剂及低密度脂蛋白吸附治疗联合应用。除血管紧张素转换酶抑制剂外,这些药物在减少高剂量糖皮质激素方面均无效,而高剂量糖皮质激素反过来又会引发进行性骨质疏松和腰椎骨折。以中等剂量使用糖皮质激素,治疗重点在于垂体微腺瘤并发的肢端肥大症。皮下注射生长抑素类似物醋酸奥曲肽可减少蛋白尿并增加尿量。随后对腺瘤进行经蝶窦显微手术,使升高的肌酐清除率恢复正常,并进一步减少糖皮质激素剂量,同时维持缓解状态。这是首例报告的肢端肥大症继发FSGS的临床病例,提示生长激素分泌过多参与肾小球疾病的发生发展。