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Neonatal hemochromatosis

Sarah A Taylor, Susan Kelly, Estella M Alonso, Peter F Whitington
OBJECTIVES: To evaluate pregnancy outcomes in pedigrees of neonatal hemochromatosis to determine the spectrum of gestational alloimmune liver disease (GALD) in a large cohort. STUDY DESIGN: We prospectively collected data from women with a prior offspring with proven neonatal hemochromatosis between 1997 and 2015 and analyzed pregnancy outcomes. RESULTS: The pedigrees from 150 women included 350 gestations with outcomes potentially related to GALD...
February 27, 2018: Journal of Pediatrics
Hiroko Midorikawa, Tatsuki Mizuochi, Jun-Ichiro Okada, Tadashi Hisano
No abstract text is available yet for this article.
November 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
Khaled Alenezi, Binita M Kamath, Iram Siddiqui, Christopher Tomlinson, Govind B Chavhan
BACKGROUND: There are limited data on utility of magnetic resonance imaging (MRI) in the assessment of suspected neonatal hemochromatosis (NH). OBJECTIVES: The aim of the study was to present our experience with utilization of multi-echo sequence MRI technique in the evaluation of NH and to compare MRI findings in infants with and without NH. METHODS: MRI performed for suspected NH were retrospectively reviewed to note the presence and severity of iron deposition (ID) in liver, spleen, pancreas, and kidneys on multi-echo sequences...
April 2018: Journal of Pediatric Gastroenterology and Nutrition
Carlos Zozaya Nieto, Beatriz Fernández Caamaño, Gema Muñoz Bartolo, Juan J Menéndez Suso, Esteban Frauca Remacha, Eva Valverde Núñez
OBJECTIVES: To describe the epidemiological features, clinical characteristics and outcomes of neonates diagnosed with liver failure, as well as determine prognostic factors. METHODS: Cohort study conducted at a single tertiary referral and university-affiliated pediatric center. Hospital records of all neonates diagnosed with liver failure between January 2003 and December 2015 were retrospectively reviewed, and data on clinical and laboratory findings, treatment, and outcomes were collected...
May 2017: Journal of Pediatric Gastroenterology and Nutrition
Mirta Ciocca, Fernando Álvarez
Neonatal acute liver failure is a rare, very severe disease with a high rate of mortality. It is clinically and etiologically different from acute liver failure seen in older children and adults. Coagulopathy with an international normalized ratio ≥ 3 is the critical parameter that defines it. The most common causes are fetal alloimmune hepatitis, previously called neonatal hemochromatosis, viral infections, metabolic disorders, and hemophagocytic lymphohistiocytosis. There is a group of treatable diseases that require a very early diagnosis for the prescription of an adequate treatment...
April 1, 2017: Archivos Argentinos de Pediatría
Carolina Roos Mariano da Rocha, Renata Rostirola Guedes, Carlos Oscar Kieling, Marina Rossato Adami, Carlos Thadeu Schmidt Cerski, Sandra Maria Gonçalves Vieira
Neonatal liver failure (NLF) is a major cause of neonatal morbidity and mortality, presenting as acute liver failure and/or congenital cirrhosis. Many affected patients show antenatal signs of fetal injury. There are several causes of NLF and early diagnosis is mandatory to elucidate the etiology and determine a specific treatment or the best management strategy. Gestational alloimmune liver disease associated with neonatal hemochromatosis (GALD-NH) is a rare but potentially treatable cause of NLF. It should be considered in any neonate with fetal signs of disease and postnatal signs of liver failure with no other identifiable causes...
2017: Case Reports in Pediatrics
Christiane Sokollik, Bianca Kreiter, Rainer Wolf
No abstract text is available yet for this article.
May 2017: Journal of Pediatrics
Aiko Shimono, Yuko Imoto, Haruhiko Sakamoto, Yoichi Chiba, Koichi Matsumoto, Machi Kawauchi, Takashi Kusaka, Hirokazu Tanaka, Toshiyuki Hata, Yoshio Kushida, Masaki Ueno
INTRODUCTION: Neonatal hemochromatosis (NH) is a rare neonatal disorder that results in liver cirrhosis with hemosiderin deposition in the liver and other organs, similarly to hereditary hemochromatosis. Excess iron is transferred from the mother to fetus through the placenta in NH. We examined the expression of iron metabolism-related substances in placental syncytiotrophoblasts (STB) by immunostaining to clarify how the transfer of iron through STB increases in NH. METHODS: Immunostaining was performed using formalin-fixed, paraffin-embedded sections of placentae from three NH cases, four gestational age-matched controls, and, depending on the antibody examined, five to seven full-term controls...
December 2016: Placenta
Sara Laliena Aznar, Inés Martínez Redondo, María J Oliván Del Cacho, María Martínez Del Moral, Raquel Pinillos Pisón
Gestational alloimmune liver disease, previously known as neonatal hemochromatosis, is characterized by severe liver disease in neonatal period, associated with intra and extrahepatic iron accumulation. It is postulated an alloimmune origin, which has opened new opportunities in the treatment and prevention during risk pregnancies, changing the prognosis of this pathology. We report the case of a newborn that presents early liver failure, with clinical and analytical features compatible with gestational alloimmune liver disease...
December 1, 2016: Archivos Argentinos de Pediatría
Hannah B Anastasio, Maureen Grundy, Meredith L Birsner, Karin J Blakemore
BACKGROUND: Gestational alloimmune liver disease, a form of profound liver failure in the newborn, is the main underlying cause of the entity formerly known as neonatal hemochromatosis. Antepartum maternal intravenous immunoglobulin (IVIG) has been shown to prevent gestational alloimmune liver disease, which otherwise has a recurrence risk above 90% in subsequent pregnancies. CASE: A 30-year-old woman, gravida 3 para 0120, presented early in gestation. Her previous pregnancy had been complicated by fetal growth restriction, oligohydramnios, and ultimately fatal fulminant neonatal liver failure...
November 2016: Obstetrics and Gynecology
Noriki Okada, Yoshiyuki Ihara, Taizen Urahashi, Yukihiro Sanada, Naoya Yamada, Yuta Hirata, Masahisa Tashiro, Takumi Katano, Kentaro Ushijima, Shinya Otomo, Hironori Takahashi, Shigeki Matsubara, Koichi Mizuta
Neonatal hemochromatosis (NH) is a rare disease with a poor prognosis, particularly prior to 2008. Antenatal maternal high-dose immunoglobulin (Ig) is effective in preventing NH recurrence, but the adverse effects of this treatment have not been documented as yet. Here, we report on a patient who underwent high-dose Ig treatment to prevent NH recurrence. The patient was a 31-year-old pregnant Japanese woman. Her first child died of NH after receiving living donor liver transplantation. The patient received high-dose Ig treatment to prevent recurrence of NH from gestational weeks 16 to 35...
October 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
Shin Jie Choi, Jong Sub Choi, Peter Chun, Jung Kyung Yoo, Jin Soo Moon, Jae Sung Ko, Woo Sun Kim, Gyeong Hoon Kang, Nam-Joon Yi
Neonatal hemochromatosis (NH) is a severe neonatal liver injury that is confirmed by extra-hepatic iron accumulation. Although a recent study described treating NH with exchange transfusions and intravenous immunoglobulin, liver transplantation should be considered for patients with severe liver failure that does not respond to other medical treatment. Herein, we report the case of a two-month-old female infant who presented with persistent ascites and hyperbilirubinemia. Her laboratory findings demonstrated severe coagulopathy, high indirect and direct bilirubin levels, and high ferritin levels...
June 2016: Pediatric Gastroenterology, Hepatology & Nutrition
Sara Nóbrega, Isabelle Scheers, Francoise Smets, Massimiliano Paganelli, Christine Sempoux, Xavier Stephenne, Etienne M Sokal
No abstract text is available yet for this article.
July 11, 2016: Journal of Pediatric Gastroenterology and Nutrition
Aiko Sasaki, Kenichiro Motomura, Fumiwo Suyama, Junko Nagasawa, Michi Hisano, Reiko Ito, Osamu Miyazaki, Atsuko Nakazawa, Yushi Ito, Mureo Kasahara, Akihiro Asai, Kenji Matsumoto, Haruhiko Sago, Koshi Yamaguchi
No abstract text is available yet for this article.
November 2016: Journal of Pediatric Gastroenterology and Nutrition
Haizhen Wang, Xue Jiang, Jieyu Wu, Linqiang Zhang, Jingfei Huang, Yuru Zhang, Xiaoju Zou, Bin Liang
The trace element iron is crucial for living organisms, since it plays essential roles in numerous cellular functions. Systemic iron overload and the elevated level of ferritin, a ubiquitous intracellular protein that stores and releases iron to maintain the iron homeostasis in cells, has long been epidemiologically associated with obesity and obesity-related diseases. However, the underlying mechanisms of this association remain unclear. Here, using Caenorhabditis elegans, we show that iron overload induces the expression of sgk-1, encoding the serum and glucocorticoid-inducible kinase, to promote the level of ferritin and fat accumulation...
May 2016: Genetics
L Korkmaz, O Baştuğ, G Daar, S Doğanay, K Deniz, S Kurtoğlu
Neonatal hemochromatosis (NH) is a form of neonatal liver failure caused by maternal-fetal alloimmune injury to hepatocytes. The etiology of neonatal hemochromatosis is not exactly understood. However, according to one theory neonatal hemochromatosis is believed to be an alloimmune disorder causing liver injury in the fetus. In order to diagnose neonatal hemochromatosis there are some criteria that should be taken into account, such as positive family history, high serum ferritin levels, high serum alpha-fetoprotein levels and siderosis demonstrated by histology or with magnetic resonance...
2015: Journal of Neonatal-perinatal Medicine
Uta Koura, Shinjiro Horikawa, Mako Okabe, Yukako Kawasaki, Masami Makimoto, Koichi Mizuta, Taketoshi Yoshida
We report the first surviving case of neonatal hemochromatosis with renal tubular dysgenesis. Renal failure was treated with peritoneal dialysis. Although hepatic failure from neonatal hemochromatosis was progressive, repeated exchange transfusions improved jaundice and coagulopathy. The patient gained weight and received a liver transplantation from her father.
August 2015: Clinical Case Reports
C Molera Busoms, J Quintero Bernabeu, J Martín de Carpi
Neonatal hemochromatosis is the most common cause of acute liver failure in the neonatal period. It is associated with high morbidity and mortality due to iron overload in hepatic and extra-hepatic tissues. New evidence has emerged during the last few years as regards its alloimmune etiology, which have had an important repercussion on the diagnosis, treatment and prognosis of these patients. Treatment with immunoglobulins and exchange transfusions has radically changed the prognosis without liver transplant...
September 2015: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
H Melin-Aldana, C Park, X Pan, M Fritsch, P Malladi, P Whitington
Gestational alloimmune liver disease (GALD) is the result of neonatal complement-mediated severe liver injury mediated by maternal alloantibodies, which is detected by immunohistochemistry staining for the complement C5b-9 complex. GALD leads to the neonatal hemochromatosis (NH) phenotype, which also shows extrahepatic siderosis, and can result in neonatal death. At autopsy, the histologic damage of the liver in GALD may be subtle and misinterpreted as non-specific post-mortem changes, resulting in the cause of death classified as indeterminate...
March 12, 2015: Journal of Neonatal-perinatal Medicine
Sharon Lewin, James B Bussel
The fetoplacental interface plays a unique role in pathologies of the fetus and neonate, and is increasingly being recognized for effects on fetal and neonatal development that resonate into adulthood. In this review, we will use several exemplary disorders involving each of the 3 types of blood cells to explore the effect of perinatal insults on subsequent development of the affected cell line. We will present new data regarding outcomes of infants treated prenatally for fetal and neonatal alloimmune thrombocytopenia (FNAIT) and contrast these with outcomes of infants affected by hemolytic disease of the fetus and newborn...
January 2015: Clinical Advances in Hematology & Oncology: H&O
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