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Nutritional status as a predictive factor for paediatric tuberous sclerosis complex-associated kidney

Andrew Limavady1, Matko Marlais2,3

  • 1Great Ormond Street Institute of Child Health, University College London, London, UK.

European Journal of Pediatrics
|March 14, 2024
PubMed

Insights

Nutritional status, alongside gender and genotype, is a key predictor of kidney disease in children with tuberous sclerosis complex (TSC). This finding highlights the importance of considering weight and diet in managing TSC-associated kidney disease.

Area of Science:

  • Pediatric Nephrology
  • Genetics
  • Medical Research

Background:

  • Gender and genetic mutations (TSC2) are established predictors for kidney disease in tuberous sclerosis complex (TSC).
  • The role of nutritional status in TSC-associated kidney disease has not been extensively studied.
  • Existing research indicates a need to explore factors beyond genetics and gender.

Purpose of the Study:

  • To identify predictive factors for the development and progression of kidney disease in pediatric patients with TSC.
  • To investigate the association between clinical factors, including nutritional status, and kidney lesions in children with TSC.
  • To determine predictors for chronic kidney disease (CKD) and high-risk angiomyolipomas in this population.

Main Methods:

  • Retrospective review of medical records from children diagnosed with TSC at a tertiary children's hospital.
  • Utilized Cox proportional hazards regression to identify predictors of kidney lesions.
  • Employed logistic regression to determine factors associated with CKD and large angiomyolipomas (>3 cm).

Main Results:

  • 79% of children with TSC exhibited abnormal kidney imaging findings.
  • Female gender was associated with a lower risk of cyst development and angiomyolipomas.
  • TSC2 mutations and obesity were linked to an increased risk of angiomyolipomas.
  • Underweight status was associated with a lower risk of angiomyolipomas.
  • 12% of tested children had CKD (stages II-V), with large angiomyolipomas being the sole predictor.
  • Overweight/obesity and older age were significantly associated with high-risk angiomyolipomas.

Conclusions:

  • Nutritional status (underweight, overweight/obesity) emerges as a novel predictor for TSC-associated kidney disease, complementing known factors like gender and genotype.
  • Findings suggest a complex interplay between hormonal factors, obesity, and kidney angiomyolipoma growth.
  • Further research into the impact of nutritional status on TSC-kidney disease progression is warranted, potentially informing management guidelines.

Keywords:
AngiomyolipomaNutritional statusPaediatricsTuberous sclerosis complex

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