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Lalayiannis AD, Thompson C, Malcomson R, et al. Nodular glomerulosclerosis in a patient with cystic fibrosis, but not diabetes mellitus: A paediatric case. Respir Med Case Rep. 2016;19:58-60doi: 10.1016/j.rmcr.2016.07.005.
Lalayiannis, A. D., Thompson, C., Malcomson, R., & Milford, D. V. (2016). Nodular glomerulosclerosis in a patient with cystic fibrosis, but not diabetes mellitus: A paediatric case. Respiratory medicine case reports, 1958-60. https://doi.org/10.1016/j.rmcr.2016.07.005
Lalayiannis, A D, et al. "Nodular glomerulosclerosis in a patient with cystic fibrosis, but not diabetes mellitus: A paediatric case." Respiratory medicine case reports vol. 19 (2016): 58-60. doi: https://doi.org/10.1016/j.rmcr.2016.07.005
Lalayiannis AD, Thompson C, Malcomson R, Milford DV. Nodular glomerulosclerosis in a patient with cystic fibrosis, but not diabetes mellitus: A paediatric case. Respir Med Case Rep. 2016 Jul 14;19:58-60. doi: 10.1016/j.rmcr.2016.07.005. eCollection 2016. PMID: 27489763; PMCID: PMC4961824.
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Respir Med Case Rep. 2016 Jul 14;19:58-60. doi: 10.1016/j.rmcr.2016.07.005. eCollection 2016.

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

Respiratory medicine case reports

A D Lalayiannis, C Thompson, R Malcomson, D V Milford

Affiliations

  1. Nephrology Department, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, B4 6NH, United Kingdom.
  2. Histopathology Department, Leicester Royal Infirmary, Infirmary Square, Leicester, LE1 5WW, United Kingdom.

PMID: 27489763 PMCID: PMC4961824 DOI: 10.1016/j.rmcr.2016.07.005

Abstract

BACKGROUND: 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.

Keywords: Cystic fibrosis; Insulin dependent diabetes mellitus (IDDM); Kimmelstiel-Wilson nodules; Nodular glomerulosclerosis

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