Related Experiment Video
Updated: May 1, 2026

07:43
Endoscopic Endonasal Trans-sphenoidal Approach: Minimally Invasive Surgery for Pituitary Adenomas
Published on: January 17, 2018
18.5K
Neonatal haemochromatosis with reversible pituitary involvement
Giuseppe Indolfi1, Rita Bèrczes, Isabella Pelliccioli
1Paediatric Hepatology, Meyer Children Hospital, Firenze, Italy.
Summary
Neonatal haemochromatosis, a severe gestational disease, can cause pituitary iron deposition leading to secondary hypothyroidism. Liver transplantation successfully treated these conditions, indicating pituitary dysfunction is a reversible manifestation.
Area of Science:
- Neonatology
- Endocrinology
- Immunology
Background:
- Neonatal haemochromatosis is a rare, severe alloimmune gestational disease with high mortality.
- It is characterized by neonatal liver failure and extrahepatic iron deposition (siderosis).
- Previously, pituitary dysfunction was not recognized as a complication.
Observation:
- This report details a neonate diagnosed with neonatal haemochromatosis.
- The neonate presented with secondary hypothyroidism due to iron deposition in the pituitary gland.
- This represents a novel extrahepatic manifestation of the disease.
Findings:
- The neonate exhibited pituitary iron deposition, a previously unreported finding in neonatal haemochromatosis.
- Secondary hypothyroidism was successfully treated.
- ABO-incompatible liver transplantation resolved both the liver failure and the pituitary dysfunction.
Implications:
- Pituitary gland dysfunction should be considered an extrahepatic manifestation of neonatal haemochromatosis.
- Early diagnosis and intervention, such as liver transplantation, can reverse pituitary iron deposition and associated endocrine dysfunction.
- This finding expands the understanding of neonatal haemochromatosis complications and treatment outcomes.
Related Concept Videos
Hepatic Encephalopathy
53
DefinitionHepatic encephalopathy is a reversible neurologic syndrome that results from advanced liver dysfunction or portosystemic shunting. It leads to disturbances in cognition, behavior, and motor function due to the brain’s exposure to gut-derived toxins that the liver fails to detoxify.EtiologyThis condition develops either in the setting of acute fulminant hepatitis or progressively during chronic liver disease, such as cirrhosis and portal hypertension. Portosystemic...
53
Portal Hypertension
50
Portal hypertension is an increase in blood pressure within the portal venous system. Normally, this pressure is less than 5 mmHg. It is considered clinically significant when it rises above 10 mmHg. At this threshold, complications from altered blood flow and venous congestion emerge.EtiologyPortal hypertension arises from conditions that impede blood flow through the liver. The most common cause is cirrhosis, in which chronic liver injury leads to fibrotic scarring. This fibrosis narrows or...
50
Inborn Errors of Metabolism
1.1K
Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...
1.1K

