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Endocrine Abstracts (2013) 33 P72 | DOI: 10.1530/endoabs.33.P72

Alderhey Childrens’ Hospital, Liverpool, UK.


Background: GH deficiency has been reported widely in association with myelomeningocele and other pituitary hormone deficiencies occur less frequently. Following an episode of adrenal crisis in a newborn infant with myelomeningocele and multiple pituitary hormone deficiencies we introduced a policy of screening affected, newborn infants for pituitary insufficiency, focusing on thyroid function and cortisol levels.

Patients and methods: Data from 26 infants (13 males) were reviewed retrospectively. TSH sufficiency was assessed from an unstimulated measure of TSH and fT4. ACTH sufficiency as assessed by the cortisol response to the low dose short Synacthen test (LDSST: Synacthen 150 ng). A peak cortisol ≥500 nmol/l was considered to be normal.

Results:

• Thyroid hormone levels were normal in all infants.

• Median (range) basal cortisol was 313 (<50–618) nmol/l and peak cortisol 693 (375–1617) nmol/l.

• 4/26 infants (16%) had a suboptimal cortisol response and were treated with hydrocortisone during periods of stress.

• Peak cortisol was >500 nmol/l in 3/4 patients with an impaired response during the neonatal period at reassessment 6 months later.

Conclusion: Infants with myelomeningocoele undergo a number of medical and surgical interventions in the first year of life. In this group of patients it is particularly important to identify infants who may have an impaired cortisol response to stress. Our data suggest that routine assessment of the pituitary, possibly limited to assessment of ACTH, should continue.

Volume 33

41st Meeting of the British Society for Paediatric Endocrinology and Diabetes

British Society for Paediatric Endocrinology and Diabetes 

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