Investigation and outcome of neonatal hepatitis in infants with hypopituitarism

Spray, CH

doi:10.1080/080352500750043413

Cited by 23 publications

(34 citation statements)

References 11 publications

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“…Data from our case series strongly support the hypothesis of other researchers[2916–18] that cortisol deficiency might have a major role in the pathogenesis of cholestasis in young infants. The pathophysiologic mechanism, however, remains ill defined.…”

Section: Discussionsupporting

confidence: 91%

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Isolated cortisol deficiency: A rare cause of neonatal cholestasis

Al–Hussaini

AlMutairi

Mursi

et al. 2012

Saudi J Gastroenterol

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For decades, congenital panhypopituitarism has been recognized to cause infantile cholestasis. However, the identity of the hormone whose deficiency causes such derangement of the liver is not clear. Here, we report four cases of isolated severe cortisol deficiency presenting with neonatal cholestasis and hypoglycemia, of whom two had familial primary glucocorticoid deficiency and the other two had isolated adrenocorticotropin deficiency. The resolution of cholestasis by hydrocortisone replacement therapy suggests a causal relationship between cortisol deficiency and the development of neonatal cholestasis. In conclusion, the presentation of a young infant with cholestasis and hypoglycemia should alert pediatricians to the possibility of cortisol deficiency and prompt investigation of adrenal function should be undertaken.

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Section: Discussionsupporting

confidence: 91%

“…Late diagnosis and initiation of hydrocortisone leads to liver cirrhosis and the need for liver transplantation during early childhood. [9]…”

Section: Discussionmentioning

confidence: 99%

Isolated cortisol deficiency: A rare cause of neonatal cholestasis

Al–Hussaini

AlMutairi

Mursi

et al. 2012

Saudi J Gastroenterol

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“…However, in one patient for whom hormone replacement therapy was started at 5 years old, cirrhosis and portal hypertension developed. [3] Although the association between liver damage and hypophyseal hormone deficit has been known since 1956,[3] the pathogenesis is not well understood. Some reports propose that neonatal hepatitis may be secondary to a deficit in cortisol and/or GH, which participate in the regulation, synthesis, and transport of biliary acids.…”

Section: Discussionmentioning

confidence: 99%

“…[3] Therefore, early recognition within the first few days after birth is essential for appropriate treatment. A high index of suspicion and careful investigation of infants who present with hepatitis, jaundice, and hypoglycemia is imperative to rule out congenital panhypopituitarism.…”

Section: Discussionmentioning

confidence: 99%

Hepatopathy in an adult, secondary to congenital untreated panhypopituitarism and ectopic posterior pituitary gland

Valle-Murillo

Pérez-Díaz

2012

Indian J Endocr Metab

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We report a rare case of an adult with advanced liver failure in the setting of an untreated congenital panhypopituitarism. A 32-years-old man presented with a newly onset seizure episode secondary to hypoglycemia. In the initial exploration, we found eunuchoid habitus, absence of secondary sexual characteristics, ascites, and hepatic encephalopathy. Hormonal evaluation confirmed the absence of anterior hypophyseal hormones and the liver function tests showed derangement of liver function. Magnetic Resonance Imaging (MRI) showed hypoplastic adenohypophysis and ectopic posterior pituitary gland. In the approach to liver disease, no cause was identified, besides the untreated panhypopituitarism.

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“…Die Ursache der neonatalen hypoglykämischen Krampfanfälle bei unserer Patientin war das Fehlen von ACTH, Kortisol und hGH. Rezidivierende Hypoglykämien mit konsekutiven Krampfanfällen sind oft Leitsymptom bei konnatalem Panhypopituitarismus[4,5,9,16].Hypophysenaplasie bzw. -hypoplasie sind selten Ursachen eines Panhypopituitarismus, häufiger findet man Mittelliniendefekte z.…”

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