Optic Nerve Hypoplasia and Growth Hormone Deficiency in a Cholestatic Infant

Xinias, Ioannis; Papadopoulou, Maria; Papastavrou, Theodouli; Patsiaoura, Kalliopi; Spiroglou, Kleomenis

doi:10.1016/j.pediatrneurol.2005.10.001

Cited by 5 publications

(2 citation statements)

References 18 publications

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“…9 Since then, a few reports have associated neonatal hepatitis with idiopathic hypopituitarism. [3][4][5][6]10 The mechanism of liver dysfunction and the development of cholestasis in hypopituitarism are still the subjects of debate. Isolated thyroid hormone, thyroid stimulating hormone (TSH), GH, ACTH, and cortisol deficiencies have been shown to cause conjugated hyperbilirubinemia but combined deficiencies have also been described.…”

Section: Discussionmentioning

confidence: 99%

“…1,2 Hypoglycemia due to panhypopituitarism is typically detected shortly after birth, but it may occur several weeks after the neonatal period. 3 Herman et al first postulated that the hormonal deficiencies due to congenital panhypopituitarism were the cause of neonatal cholestasis. 4 Although the causes are still unknown and the incidence is low, it is still crucial to evaluate any cholestatic infant to prevent progressive hepatic disease and neurological complications.…”

Section: Introductionmentioning

confidence: 99%

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Neonatal Hypopituitarism: Unusual Presentation

Abu-Libdeh

Abu‐Libdeh

Abdulhag

2017

JCS

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Objective We report an infant with panhypopituitarism presenting with cholestatic jaundice, hypoglycemia, and high ferritin level. Methods We conducted clinical and laboratory investigations, including metabolic, infectious, and hormonal evaluation. Results Hormonal evaluation revealed panhypopituitarism (cortisol deficiency, growth hormone deficiency, and central hypothyroidism). Other causes of cholestasis were ruled out. Surprisingly, serum ferritin level was very high suggesting neonatal hemochromatosis, which was ruled out by the absence of hemosiderin deposition in buccal mucosal biopsy. Replacement therapy with glucocorticoids and L-thyroxin showed improvement of liver function tests, resolved cholestatic jaundice, and significantly decreased serum ferritin level. These findings support the assumption that thyroid hormone and cortisol affect the bile acid-independent bile flow. Conclusion This is the first description of an infant with congenital panhypopituitarism, presenting with cholestasis, hypoglycemia, and high serum ferritin level. Panhypopituitarism should be considered in any infant who presents with cholestasis, hypoglycemia, and other manifestations of pituitary malfunction. High serum ferritin level probably reflects acute phase reaction.

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