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Simultaneous onset of steroid resistant nephrotic syndrome and IDDM in two young children

Jameela A Kari1, Sherif M El-Desoky, Ghadeer Mokhtar

  • 1Pediatrics Department, King Abdulaziz University, Jeddah, Saudi Arabia. jkari@doctors.org.uk

BMJ Case Reports
|July 17, 2012
PubMed

Insights

Two children developed insulin-dependent diabetes mellitus (IDDM) shortly after nephrotic syndrome diagnosis. This case series is the first to report steroid-resistant nephrotic syndrome with near-simultaneous IDDM onset in young children.

Area of Science:

  • Pediatric Nephrology
  • Endocrinology
  • Diabetology

Background:

  • Nephrotic syndrome is a kidney disorder characterized by heavy protein loss in urine.
  • Insulin-dependent diabetes mellitus (IDDM), or Type 1 diabetes, is an autoimmune condition affecting insulin production.
  • Steroid-resistant nephrotic syndrome (SRNS) presents a significant clinical challenge in pediatric populations.

Observation:

  • Two young children presented with SRNS, with one diagnosed at 2 years 9 months and the other at 18 months.
  • Both patients developed IDDM within two weeks of their nephrotic syndrome diagnosis.
  • Neither patient initially responded to prednisolone treatment, indicating steroid resistance.

Findings:

  • The first patient's renal biopsy revealed mesangial proliferation.
  • The second patient, initially diagnosed with minimal change disease, developed diabetic glomerulosclerosis on follow-up biopsy after IDDM onset.
  • Genetic testing for NPHS2 and WT1 mutations was negative in both cases.

Implications:

  • This report highlights a potential, previously undocumented association between SRNS and IDDM in pediatric patients.
  • The findings suggest a need for heightened awareness and monitoring for IDDM in children diagnosed with SRNS.
  • Further research is warranted to elucidate the underlying mechanisms connecting these two conditions and explore potential therapeutic targets.

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