Evolving hypopituitarism in children with central nervous system lesions

Area of Science:

• Pediatric Endocrinology
• Neuroendocrinology
• Pediatric Neurology

Background:

• Subnormal growth rate in children often prompts testing for human growth hormone (hGH) deficiency.
• Organic central nervous system (CNS) lesions can impact endocrine function, including growth hormone secretion.
• Initial assessment may not always reveal hGH deficiency despite clinical signs.

Purpose of the Study:

• To investigate the diagnostic course of growth hormone (GH) deficiency in children with organic CNS lesions and initially normal GH levels.
• To determine if subnormal growth rate in this cohort predicts later development of GH deficiency.
• To assess the potential for evolving hypopituitarism in children with CNS pathology.

Main Methods:

• Study included seven pediatric patients with organic CNS lesions and subnormal growth rates but normal initial hGH concentrations.
• Patients were followed and retested for GH levels and other pituitary hormone deficiencies over 0.5 to 4.6 years.
• Diagnosis of CNS lesions included histiocytosis X, septo-optic dysplasia, neonatal meningitis, anterior encephalocele, and neurofibromatosis.

Main Results:

• All seven patients with initially normal hGH levels eventually developed documented growth hormone deficiency upon retesting.
• Four patients initially presented with other pituitary hormone deficiencies; two developed additional deficiencies later.
• Subnormal growth rate persisted or was the primary indicator necessitating further investigation.

Conclusions:

• Children with organic CNS lesions and subnormal growth may have evolving hypopituitarism, not just isolated hGH deficiency.
• Initial normal GH levels do not exclude future deficiency; continued observation and retesting are crucial.
• Deficient somatomedin generation might contribute to subnormal growth in these complex cases.