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Solitary functioning kidney in children--a follow-up study

Gabriel Kolvek1, Ludmila Podracka, Jaroslav Rosenberger

  • 1Paediatric Department, Faculty of Medicine, Safarik University, Kosice, Slovak Republic.

Kidney & Blood Pressure Research
|August 30, 2014
PubMed

Insights

A significant number of children with spinal muscular atrophy (SMA) experience kidney damage, including high blood pressure and reduced kidney function. Early monitoring of kidney health is crucial for these children, especially those with congenital anomalies of the kidney and urinary tract (CAKUT).

Area of Science:

  • Pediatric Nephrology
  • Renal Medicine
  • Genetics

Background:

  • Spinal muscular atrophy (SMA) is a rare genetic neuromuscular disorder.
  • Children with SMA are at risk for various complications, including potential renal injury.
  • Early identification of renal dysfunction is critical for managing long-term health outcomes.

Purpose of the Study:

  • To determine the cumulative incidence of renal injury in children with SMA.
  • To assess the development of hypertension, albuminuria, and decreased estimated glomerular filtration rate (eGFR) in this cohort.
  • To analyze renal injury-free survival rates and identify risk factors.

Main Methods:

  • A prospective follow-up study included 42 children diagnosed with SMA.
  • Repeated assessments of blood pressure, albuminuria, and eGFR were conducted.
  • Cumulative incidence rates of renal injury and renal injury-free survival were evaluated, stratified by etiology.

Main Results:

  • The mean follow-up duration was 11.3 years.
  • Overall, 38.1% of children with SMA developed renal injury.
  • Renal injury manifestations included hypertension (23.8%), severe albuminuria (7.1%), impaired eGFR (11.9%), and medication use (26.2%).
  • Children with congenital anomalies of the kidney and urinary tract (CAKUT) within the SMA cohort showed a significantly higher incidence of renal injury.
  • The median time to renal injury onset was 12.8 years.

Conclusions:

  • A substantial proportion of children with SMA develop renal injury during childhood.
  • Children with SMA and CAKUT face a particularly elevated risk of renal complications.
  • Close monitoring of blood pressure, albuminuria, and eGFR is essential for early detection of chronic kidney disease in children with SMA.
Abstract

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