Neonatal Hyperbilirubinemia and Hypoglycemia in Congenital Hypopituitarism

Copeland, Kenneth C.; Franks, Robert C.; Ramamurthy, Rajam S.

doi:10.1177/000992288102000808

Cited by 39 publications

(24 citation statements)

References 21 publications

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“…Gönç et al 16 showed that primary and central adrenal insufficiency can cause cholestatic hepatititis. Similarly, a study by Al-Hussaini et al 6 showed that cholestasis recovered after hydrocortisone therapy in 4 cases, of which two had primary and two had secondary adrenal failure. Cholestasis of our patient also recovered by 45 days with hydrocortisone and L-thyroxine therapy.…”

Section: Discussionmentioning

confidence: 89%

“…The pattern of liver dysfunction secondary to hormone deficiencies such as growth hormone deficiency, hypocortisolism, or hypothyroidism is variable and goes from predominantly direct hyperbilirubinemia with cholestasis, or cholestasis with elevated hepatic enzymes 4,13 . Some authors suggested that growth hormone deficiency was the major factor yielding cholestasis 6 . Drop et al 14 supported this hypothesis by observing that cholestasis did not resolve with thyroxin and hydrocortisone therapies in two infants with hypopituitarism.…”

Section: Discussionmentioning

confidence: 99%

“…It is unclear which hormone causes cholestasis in patients with congenital panhypopituitarism. Some authors suggested that growth hormone deficiency is the major cause of cholestasis [5][6][7] . But there is now good evidence that central adrenal insufficiency is the main cause of cholestatic hepatitis in hypopituitarism.…”

mentioning

confidence: 99%

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Cholestasis and hypercalcemia secondary to panhypopituitarism in a newborn

2017

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Section: Discussionmentioning

confidence: 89%

Section: Discussionmentioning

confidence: 99%

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Cholestasis and hypercalcemia secondary to panhypopituitarism in a newborn

2017

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“…Jaundice related to congenital hypopituitarism is usually cholestatic, whereas in our patient it was mostly unconjugated. [20][21][22][23] Specific tests to detect possible hypopituitarism were not performed in the first few days of life. Only TSH level as screening for congenital hypothyroidism was measured, which by itself is not sufficient for diagnosing secondary hypothyroidism.…”

Section: Figurementioning

confidence: 99%

Hypopituitarism in a Patient With Beckwith-Wiedemann Syndrome Due to Hypomethylation of KvDMR1

Baiocchi¹,

Yousuf

Hussain

2014

Pediatrics

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Beckwith-Wiedemann syndrome (BWS) is caused by dysregulation of imprinted genes on chromosome 11.p15.5. The syndrome includes overgrowth, macroglossia, organomegaly, abdominal wall defects, hypoglycemia, and long-term malignancy risk. No patient who has BWS has been reported with hypopituitarism. We describe a patient who presented at birth with macrosomia, macroglossia, respiratory distress, jaundice, and hypoglycemia, and who was followed for 4.5 years. Genetic test for BWS was performed, which detected loss of maternal methylation on region KvDMR1 (11p15.5). The hypoglycemia was attributable to hyperinsulinism and was treated with diazoxide and chlorothiazide. She responded well, but the hypoglycemia returned after reducing the diazoxide. It was possible to stop the diazoxide after 2.5 years. On routine follow-up she was noted to be developing short stature. Baseline pituitary and growth hormone (GH) stimulation tests detected GH deficiency and secondary hypothyroidism. A brain MRI showed a small anterior pituitary gland. Thereafter, thyroxine and replacement therapy with GH were started, which resulted in a remarkable improvement in growth velocity. This is the first patient to be reported as having hypopituitarism and BWS. It is unclear if the BWS and the hypopituitarism are somehow connected; however, further investigations are necessary. Hypopituitarism explains the protracted hypoglycemia and the short stature. In our patient, GH therapy seems to be safe, but strict follow-up is required given the increased cancer risk related to BWS.

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“…[1][2][3] Supporting signs include micropenis, midline defects such as cleft lip or palate, and ophthalmic abnormalities associated with septo-optic dysplasia such as nystagmus or optic atrophy. Primary adrenal insufficiency may also present with hypoglycaemia and cholestasis.…”

Section: Commentmentioning

confidence: 99%