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• W2057991214 abstract "<b><i>Background:</i></b> Congenital nasal pyriform aperture stenosis (CNPAS) is an increasingly recognised cause of upper airway obstruction associated with midline abnormalities. Studies have described pituitary dysfunction in 40% of patients. We aimed to develop guidelines for: (a) the early identification of pituitary insufficiency to minimise surgical risk and (b) to stratify patients for follow-up. <b><i>Methods:</i></b> Retrospective case note review of patients with CNPAS between 2000 and 2014 in a tertiary paediatric unit. <b><i>Results:</i></b> 20 patients (12 female:8 male) were analysed; 16 were diagnosed during the neonatal period while 4 were diagnosed later. There was no consistent approach in the evaluation of the pituitary axis at diagnosis. Pituitary dysfunction was identified in 3 (15%) patients, 2 of whom were found during evaluation of short stature in mid-late childhood. Hypoglycaemia and conjugated hyperbilirubinaemia, but not the degree of stenosis, were highly predictive of pituitary dysfunction (p < 0.05). Available height standard deviation score (SDS) data at 1 year of 70% of our patients identified both of the late-diagnosed growth hormone-deficient patients, with SDS of -2.6 and -3.6, respectively. <b><i>Conclusion:</i></b> All CNPAS patients should have MRI of the brain and baseline endocrine investigations at diagnosis. Growth monitoring for at least 1 year is recommended as low, or falling, height SDS at 1 year is a good predictor of pituitary dysfunction." @default.
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• W2057991214 date "2015-01-01" @default.
• W2057991214 modified "2023-09-27" @default.
• W2057991214 title "Early Identification of Pituitary Dysfunction in Congenital Nasal Pyriform Aperture Stenosis: Recommendations Based on Experience in a Single Centre" @default.
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• W2057991214 doi "https://doi.org/10.1159/000369805" @default.
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