Bladder exstrophy: effects on bone age, bone mineral density, growth, and metabolism

Area of Science:

• Pediatric Urology
• Pediatric Endocrinology
• Pediatric Orthopedics

Background:

• Bladder exstrophy (BE) is a complex congenital anomaly.
• Metabolic bone health, growth, and development in BE patients remain understudied.
• Previous research has not comprehensively evaluated these parameters in BE patients with and without augmentation.

Purpose of the Study:

• To investigate growth, bone age, bone metabolism, bone mineral density (BMD), blood gases, glomerular filtration rate (GFR), and electrolytes in BE patients.
• To compare findings between BE patients with and without bladder augmentation.
• To assess the prevalence of metabolic bone problems and growth disturbances in BE.

Main Methods:

• Studied 15 BE patients (with and without augmentation).
• Assessed growth percentiles, bone age, bone biochemistry, BMD (forearm, femur, lumbar vertebrae), blood gases, GFR, and electrolytes.
• Compared patient data with normal children and between augmented and non-augmented groups.

Main Results:

• All patients exhibited growth retardation and decreased bone age.
• Ten patients were below the 10th percentile for height; mean age/bone age ratio was 1.59.
• Seven patients had decreased BMD (osteopenia/osteoporosis), with Z scores below -1.
• Four patients had metabolic acidosis (pH < 7.35), and five had low bicarbonate levels.
• No significant differences in growth, BMD, or laboratory values were found between augmented and non-augmented groups.

Conclusions:

• BE patients, with or without augmentation, frequently experience significant growth retardation.
• Approximately 45% of BE patients have osteopenia or osteoporosis, irrespective of augmentation.
• BE patients show considerable deviations in height percentiles and bone ages compared to the general population.
• Close monitoring of linear growth, bone age, and bone mineral density is recommended for BE patients.