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Isolated ACTH deficiency. Metabolic and endocrine studies in a 7-year-old boy

A Aynsley-Green, M W Moncrieff, S Ratter

    Archives of Disease in Childhood
    |June 1, 1978
    PubMed

    Insights

    Isolated ACTH deficiency, a rare condition, can cause hypoglycaemic convulsions in children. This study reveals impaired adrenaline secretion and alanine metabolism contribute to hypoglycemia in this disorder.

    Area of Science:

    • Pediatric Endocrinology
    • Metabolic Disorders
    • Neuroendocrinology

    Background:

    • Hypoglycemia is a critical condition in childhood, necessitating thorough etiological investigation.
    • Isolated ACTH deficiency is an uncommon endocrine disorder presenting diagnostic challenges.

    Observation:

    • A 7-year-old boy with recurrent hypoglycemic convulsions was studied.
    • Reduced adrenaline secretion during insulin-induced hypoglycemia was noted.
    • Low blood alanine and elevated ketone bodies were observed during starvation-induced hypoglycemia.

    Findings:

    • The patient was diagnosed with isolated ACTH deficiency.
    • Impaired alanine mobilization and reduced gluconeogenesis were identified as key contributors to hypoglycemia.
    • Oral alanine failed to adequately increase blood glucose levels during hypoglycemic episodes.

    Implications:

    • This study highlights the metabolic underpinnings of hypoglycemia in isolated ACTH deficiency.
    • Understanding these mechanisms can guide diagnosis and management of similar pediatric cases.
    • Further research into counter-regulatory hormone function in ACTH deficiency is warranted.

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