A reduced segment II/III graft for neonatal liver failure with absence of detectable hepatocytes. A case report and literature review

Background: Neonatal hemochromatosis (NH) is a rare and severe liver disease of mainly intra-uterine onset, characterized by neonatal liver failure, hepatic and extrahepatic iron acquisition.NH is also called as Gestational alloimmune liver disease (GALD). This leads to an altered iron metabolism with resulting siderosis ,multi-organ failure and infants may be stillborn or present with advance, overwhelming liver disease. The disease represents the most common cause of liver failure in neonates and is also the most common indication for neonatal liver transplantation. We present an neonate who died at 15 days of age,and who was found to have massive iron overload in the liver. Initial treatment consisted of chelation therapy and antioxidants, but lack of laboratory and clinical improvement led to an exchange transfusion followed by intravenous immunoglobulin (IVIG). Irrespective of all above treatment no improvement of general condition of the patient. The unfavourable course of the disease is described in this case report.

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T4 + (helper phenotype) chronic lymphocytic leukemia: Case report with liver failure and literature review

Medical and Pediatric Oncology ◽

10.1002/mpo.2950160514 ◽

1988 ◽

Vol 16 (5) ◽

pp. 356-362 ◽

Cited By ~ 7

Author(s):

John P. Greer ◽

John B. Cousar ◽

Robert F. Miller ◽

Larry B. Vogler ◽

Julia C. Goodin ◽

...

Keyword(s):

Case Report ◽

Chronic Lymphocytic Leukemia ◽

Literature Review ◽

Liver Failure ◽

Lymphocytic Leukemia ◽

Chronic Lymphocytic Leukemia Case

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Neonatal liver failure due to deoxyguanosine kinase deficiency

BMJ Case Reports ◽

10.1136/bcr.12.2011.5317 ◽

2012 ◽

Vol 2012 (apr02 1) ◽

pp. bcr1220115317-bcr1220115317 ◽

Cited By ~ 18

Author(s):

S. Nobre ◽

M. Grazina ◽

F. Silva ◽

C. Pinto ◽

I. Goncalves ◽

...

Keyword(s):

Liver Failure ◽

Deoxyguanosine Kinase ◽

Neonatal Liver ◽

Neonatal Liver Failure

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Extrahepatic Nonreticuloendothelial Siderosis Is Not Specific to Gestational Alloimmune Liver Disease

Pediatric and Developmental Pathology ◽

10.1177/1093526619826429 ◽

2019 ◽

Vol 22 (4) ◽

pp. 356-364 ◽

Cited By ~ 1

Author(s):

Hao Wu ◽

William Ferguson ◽

Eumenia Castro ◽

Debra Kearney ◽

Milton Finegold ◽

...

Keyword(s):

Liver Disease ◽

Liver Failure ◽

Adrenal Glands ◽

Infant Deaths ◽

Duration Of Illness ◽

History Of ◽

Neonatal Liver ◽

Neonatal Liver Failure ◽

The Mean ◽

Bronchial Glands

Autopsy reports of 78 stillbirths and early infant deaths (up to age 8 weeks) were reviewed to investigate the prevalence of extrahepatic nonreticuloendothelial siderosis (EHNRS) in the context of neonatal liver failure. Of these, 10 liveborns (12.8%), M:F 3:2, with mean gestational age 37.6 weeks (range: 35–39) and mean age at the time of demise 19.1 days (range: 7–42), showed significant liver injury: infection (n = 7, viral > fungal), congenital malformations (n = 2), and ischemia (n = 1). None had maternal history of gestational alloimmune liver disease (GALD) or previous fetal/neonatal death due to liver failure. Seven of 10 cases (70%) showed EHNRS: pancreas (n = 6), kidneys (n = 4), thyroid and adrenal glands (n = 3), and bronchial glands and heart (n = 2). Iron deposition was most frequent in the pancreas (60%), most diffuse in the kidneys, and seen in at least 2 organs, with pancreas and kidney being the most frequent combination. Hepatic C5b-9 expression was variable (1+ to 4+) except 1 case (100% necrosis). The duration of illness and the mean age at the time of demise tended to be higher in those with EHNRS. In summary, hepatic and EHNRS, with or without C5b-9 expression, are not specific for GALD. Other causes of liver failure should be investigated as clinically and pathologically appropriate.

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