Role of Corticotropin-Releasing Hormone Testing in Assessment of Hypothalamic-Pituitary-Adrenal Axis Function in Infants with Congenital Central Hypothyroidism

Autor: Thomas Vulsma, David A. van Tijn, Jan J. M. de Vijlder
Přispěvatelé: Other Research, Paediatric Endocrinology
Rok vydání: 2008
Předmět:
Male
Hypothalamo-Hypophyseal System
endocrine system
medicine.medical_specialty
Pituitary gland
Time Factors
Hydrocortisone
Corticotropin-Releasing Hormone
Endocrinology
Diabetes and Metabolism
Clinical Biochemistry
Pituitary-Adrenal System
Context (language use)
Adrenocorticotropic hormone
Sensitivity and Specificity
Biochemistry
Diagnostic Techniques
Endocrine
Corticotropin-releasing hormone
Neonatal Screening
Endocrinology
Adrenocorticotropic Hormone
Internal medicine
Congenital Hypothyroidism
medicine
Humans
Prospective cohort study
business.industry
Decision Trees
Biochemistry (medical)
Infant
Newborn
Brain
Infant
medicine.disease
Congenital hypothyroidism
Radiography
medicine.anatomical_structure
Female
business
Algorithms
hormones
hormone substitutes
and hormone antagonists
Hypothalamic–pituitary–adrenal axis
medicine.drug
Zdroj: Journal of clinical endocrinology and metabolism, 93(10), 3794-3803. The Endocrine Society
ISSN: 1945-7197
0021-972X
DOI: 10.1210/jc.2008-0492
Popis: Context: The Dutch neonatal congenital hypothyroidism (CH) screening program detects infants with CH of central origin (CH-C). These infants have a high likelihood of multiple pituitary hormone deficiencies. ACTH deficiency especially poses an additional risk for brain damage and may be fatal. Objective: Our objective was to evaluate different tools for assessment of the integrity of the hypothalamus-pituitary-adrenocortex (HPA) axis in young infants, aiming for a strategy for reliable and timely diagnosis. Design, Setting: This is a Dutch nationwide prospective study (enrollment 1994–1996). Patients were included if neonatal CH screening results were indicative of CH-C and HPA axis function could be tested within 6 months of birth. Patients: Nine male and three female infants with CH-C and four infants with false-positive screening results or transient hypothyroidism were included in the study. Main Outcome Measures: CRH test results, multiple cortisol plasma concentrations, and cortisol excretion in 24-h urine were measured. Results: Six (50%) of the CH-C patients had abnormal CRH test results. Three of them had discordant test results: impaired increase of plasma cortisol in response to CRH, despite substantial increase of plasma ACTH. The other three infants, with concordant impaired responses of both ACTH and cortisol to CRH, had a very low urinary cortisol excretion in comparison with the subjects with normal CRH test results. Conclusions: The CRH test proves to be a fast and reliable tool in the assessment of HPA axis (dys)function. It enables timely diagnosis in (asymptomatic) neonates at risk for serious morbidity and mortality. The discordant response type, which has not been described before, may be an early phase of HPA axis dysfunction. Alternatively, patients with this response type may constitute a separate pathogenetic subset of HPA axis-deficient patients.
Databáze: OpenAIRE
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