Neonatal cholestasis and hypopituitarism.

Kaufman, Francine; Costin, Gertrude; Thomas, Dan W.; Sinatra, Frank; Roe, Thomas; Neustein, Harry B.

doi:10.1136/adc.59.8.787

Cited by 54 publications

(37 citation statements)

References 5 publications

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“…ONH with an absent septum pellucidum has the highest incidence of multiple pituitary endocrinopathies 89 and of neonatal hypoglycaemia, 90 which may be associated with neonatal cholestatic jaundice. 91 Growth hormone deficiency, adrenocorticotrophic hormone deficiency, thyroidstimulating hormone deficiency, and diabetes insipidus have all been described. 92 Identification of hypopituitarism to identify low cortisol is essential.…”

Section: Clinical Featuresmentioning

confidence: 99%

Congenital disorders of the optic nerve: excavations and hypoplasia

Dutton

2004

Eye

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Section: Clinical Featuresmentioning

confidence: 99%

Congenital disorders of the optic nerve: excavations and hypoplasia

Dutton

2004

Eye

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“…None of the patients showed any permanent liver damage during follow-up; however, this can be the case if diagnosis is not suspected during infancy (17,19).…”

Section: Discussionmentioning

confidence: 91%

Neonatal cholestasis in congenital pituitary hormone deficiency and isolated hypocortisolism: characterization of liver dysfunction and follow-up

Braslavsky

Keselman

Galoppo

et al. 2011

Arq Bras Endocrinol Metab

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INTRODUCTION: Neonatal cholestasis due to endocrine diseases is infrequent and poorly reco-gnized. Referral to the pediatric endocrinologist is delayed. OBJECTIVE: We characterized cholestasis in infants with congenital pituitary hormone deficiencies (CPHD), and its resolution after hormone replacement therapy (HRT). SUBJECTS AND METHODS: Sixteen patients (12 males) were included; eleven with CPHD, and five with isolated central hypocortisolism. RESULTS: Onset of cholestasis occurred at a median age of 18 days of life (range 2-120). Ten and nine patients had elevated transaminases and γGT, respectively. Referral to the endocrinologist occurred at 32 days (range 1 - 72). Remission of cholestasis occurred at a median age of 65 days, whereas liver enzymes occurred at 90 days. In our cohort isolated, hypocortisolism was a transient disorder. CONCLUSION: Cholestasis due to hormonal deficiencies completely resolved upon introduction of HRT. Isolated hypocortisolism may be a transient cause of cholestasis that needs to be re-evaluated after remission of cholestasis.

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“…Patients with this condition may in fact be hypothyroid, although often there are other features such as hypoglycemia, micropenis, blindness, and developmental delay. 21 Often these patients are diagnosed by direct examination of the optic nerve or a magnetic resonance image of the brain.…”

Section: Setting?mentioning

confidence: 99%