| Familial perinatal liver disease and fetal thrombotic vasculopathy. | |
| | |
MedLine Citation:
|
PMID: 17990937 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
The association between placental fetal thrombotic vasculopathy (FTV) and perinatal liver disease was not recognized until 2002, when Dahms and colleagues reported a series of 3 patients in whom severe liver disease developed in the first 2 days of life. All had abnormal liver histology and showed a variety of abnormalities, including Budd-Chiari syndrome, changes mimicking extrahepatic obstruction, lobular fibrosis, cholestasis, and hepatocyte giant cell transformation. We report recurrent significant perinatal liver disease in a family, associated with proven FTV in at least 1 pregnancy. A 30-year-old gravida 4 female with a history of heterozygous methylenetetrahydrofolate A1298C mutation had a normal 1st pregnancy and then experienced an intrauterine fetal demise at 38 weeks of gestation. Placental examination revealed extensive occlusive and mural thrombi of chorionic vessels associated with a large focus of avascular villi. Histologic examination of the liver showed extensive giant cell transformation and hepatocyte dropout. No excess hemosiderin pigment was present in the liver, pancreas, or heart. A 3rd pregnancy produced a live-born term infant with transient neonatal cholestasis. The 4th pregnancy also produced a term neonate who presented with acute hepatic failure of unknown cause, ultimately requiring liver transplantation. Fetal thrombotic vasculopathy is an underrecognized association with perinatal liver disease that may be associated with abnormal liver perfusion and that may recur in families, especially when a genetic thrombophilia is present. |
| | |
Authors:
|
Linda M Ernst; Andrew B Grossman; Eduardo D Ruchelli |
Related Documents
:
|
15504077 - Splenic pregnancy: a case report and review of the literature. 3474357 - Acute leukemia during pregnancy: the toronto leukemia study group experience with long-... 8525147 - Renal reserve in pregnancy. 1742827 - Anaesthesia for caesarean section in a patient with acute fatty liver of pregnancy. 9408927 - Pregnancy-induced hypertension: genesis of and response to endothelial injury and the r... 17962967 - Histopathological classification of tympanosclerotic plaques. |
Publication Detail:
|
Type: Case Reports; Journal Article Date: 2007-07-02 |
Journal Detail:
|
Title: Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society Volume: 11 ISSN: 1093-5266 ISO Abbreviation: Pediatr. Dev. Pathol. Publication Date: 2008 Mar-Apr |
Date Detail:
|
Created Date: 2008-04-29 Completed Date: 2008-06-12 Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 9809673 Medline TA: Pediatr Dev Pathol Country: United States |
Other Details:
|
Languages: eng Pagination: 160-3 Citation Subset: IM |
Affiliation:
|
Department of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA. ernst@email.chop.edu |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Adult Budd-Chiari Syndrome / complications, pathology* Chorionic Villi / blood supply Family* Female Fetal Death / etiology, pathology Humans Infant, Newborn Liver Failure, Acute / complications, pathology* Placenta Diseases / pathology* Pregnancy Pregnancy Complications Thrombosis / complications, pathology* Vascular Diseases / complications, pathology* |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Adenoviral appendicitis presenting clinically as acute appendicitis.
Next Document: Pathologist interobserver variability of histologic features in childhood brain tumors: results from...