Nodular glomerulosclerosis in a patient with cystic fibrosis, but not diabetes mellitus: A paediatric case

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• 作者：A.D. Lalayiannis^a ; ^{alexander.lalayiannis@nhs.net" class="auth_mail" title="E-mail the corresponding author} ; C. Thompson^a ; R. Malcomson^b ; D.V. Milford^a
• 关键词：Cystic fibrosis ; Insulin dependent diabetes mellitus (IDDM) ; Kimmelstiel-Wilson nodules ; Nodular glomerulosclerosis
• 刊名：Respiratory Medicine Case Reports
• 出版年：2016
• 出版时间：2016
• 年：2016
• 卷：19
• 期：Complete
• 页码：58-60
• 全文大小：605 K

文摘

Nodular glomerulosclerosis is seen in insulin dependent diabetic patients with nephropathy. Kimmelstiel-Wilson nodules on biopsy are considered pathognomonic. Diabetic nephropathy is a spectrum of glomerular and tubular disease which correlates with the duration of the diabetes and the extent of glycaemic control.

Case report

An eleven year old girl with cystic fibrosis was referred with persistent heavy proteinuria. She underwent a renal biopsy which revealed nodular glomerulosclerosis with Kimmelstiel-Wilson-like nodules. Her investigations for diabetes were negative and she was treated with enalapril.

Conclusion

Nodular glomerulosclerosis in the absence of diabetes and poor glycaemic control have not previously been reported in a paediatric patient. In adult patients without diabetes, smoking, hypertension, hypercholestrolaemia and extrarenal vascular disease have been implicated.

The proteinuria decreased after commencement of treatment with enalapril. A recurrence of proteinuria responded to a dose increase.