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Idiopathic hypothalamus-pituitary dysfunction: review of five cases
S Cianfarani1, R M Nicholl, S Medbach
1Department of Endocrinology, St. Bartholomew's Hospital, London, UK.
Hormone Research
|January 1, 1993
Summary
Early signs of evolving hypothalamic-pituitary dysfunction in children include weight gain, impaired thirst, and hyperprolactinaemia. These symptoms, alongside diabetes insipidus, signal progressive pituitary deficiencies requiring monitoring and hormone replacement.
Area of Science:
- Pediatric Endocrinology
- Neuroendocrinology
- Pediatric Neurology
Background:
- Hypothalamic-pituitary dysfunction can manifest subtly in children.
- Early identification is crucial for timely intervention and management.
Observation:
- Five children (4 girls, aged 4-14) presented with varied symptoms including polyuria, polydipsia, weight gain, hyperphagia, and growth failure.
- Over 1-5 years, they developed diabetes insipidus, osmoreceptor dysfunction, hyperprolactinaemia, and deficiencies in growth hormone, ACTH, and TSH.
- Structural hypothalamic lesions were identified in 2 patients via MRI/CT scans after 1.5 and 3.5 years.
Findings:
- Weight gain, impaired thirst, and hyperprolactinaemia were identified as early indicators of evolving hypothalamic-pituitary dysfunction.
- These early signs were often accompanied by diabetes insipidus and progressive anterior pituitary deficiencies.
- Normal neuroimaging in some cases highlights the importance of clinical presentation in diagnosis.
Implications:
- Clinical and neuroradiological surveillance is vital for children with symptoms suggesting hypothalamic lesions.
- Hormone replacement therapy is essential for managing progressive pituitary deficiencies.
- Understanding early clinical signs aids in the diagnosis and management of pediatric neuroendocrine disorders.