keyword
https://read.qxmd.com/read/33710405/magnetic-resonance-imaging-of-neonatal-hemochromatosis
#21
REVIEW
Govind B Chavhan, Binita M Kamath, Iram Siddiqui, Christopher Tomlinson
Neonatal hemochromatosis is a rare condition that causes neonatal liver failure, frequently resulting in fetal loss or neonatal death. It is thought that most cases of neonatal hemochromatosis are caused by gestational alloimmune liver disease (GALD), with neonatal hemochromatosis being a phenotype of GALD rather than a disease process. Extrahepatic siderosis in the pancreas, myocardium, thyroid and minor salivary gland is a characteristic feature of neonatal hemochromatosis. There is also sparing of the reticuloendothelial system with no iron deposition in the spleen...
February 2022: Pediatric Radiology
https://read.qxmd.com/read/33633086/applying-an-age-specific-definition-to-better-characterize-etiologies-and-outcomes-in-neonatal-acute-liver-failure
#22
JOURNAL ARTICLE
Kristin Borovsky, Anna M Banc-Husu, Samantha A Saul, Katie Neighbors, Susan Kelly, Estella M Alonso, Sarah A Taylor
OBJECTIVE: Neonatal acute liver failure (ALF) is a rare disease with high mortality for which no standard age-specific definition exists. To advance the understanding of neonatal ALF, we characterize the etiology, presenting features, treatment, and outcomes in infants within 1 month of life. METHODS: We performed a single-center 11-year retrospective chart review of neonates ≤30 days of life with ALF as defined by an INR of ≥2.0. Comparisons were made by etiology and survival with native liver (SNL)...
July 1, 2021: Journal of Pediatric Gastroenterology and Nutrition
https://read.qxmd.com/read/33632934/-piga-mutations-can-mimic-neonatal-hemochromatosis
#23
JOURNAL ARTICLE
Jaime Flores-Torres, Jane D Carver, Amarilis Sanchez-Valle
Neonatal hemochromatosis (NH), one of the most common causes of liver failure in the neonate, often causes fetal loss or death during the neonatal period. Most cases are thought to be due to gestational alloimmune disease; however, other rare causes have been reported. NH is generally considered congenital and familial but not heritable. We present an infant diagnosed with NH whose clinical course differed significantly from that of most NH cases: at 11 months of age he had normal levels of liver enzymes, ferritin, and bilirubin, and normal neurodevelopment...
March 2021: Pediatrics
https://read.qxmd.com/read/33541017/-an-updated-advance-of-autoimmune-hepatitis-in-children
#24
JOURNAL ARTICLE
Y Lu
Autoimmune liver disease is not common in children. In addition to autoimmune hepatitis, it also includes autoimmune sclerosing cholangitis, Giant cell hepatitis with autoimmune hemolytic anemia, and de novo autoimmune hepatitis after liver transplantation as well as two acquired autoimmune liver diseases: neonatal lupus and Gestational alloimmune liver disease (alternate name neonatal hemochromatosis). The age-specific systemic developmental characteristics and immune system association determine the type of autoimmune liver disease in children, and its clinical manifestations and prognostic transition may vary from adults...
January 20, 2021: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://read.qxmd.com/read/33158209/intravenous-immune-globulin-uses-in-the-fetus-and-neonate-a-review
#25
REVIEW
Mahdi Alsaleem
Intravenous immune globulin (IVIG) is made after processing plasma from healthy donors. It is composed mainly of pooled immunoglobulin and has clinical evidence-based applications in adult and pediatric populations. Recently, several clinical applications have been proposed for managing conditions in the neonatal population, such as hemolytic disease of the newborn, treatment, and prophylaxis for sepsis in high-risk neonates, enterovirus parvovirus and COVID-19 related neonatal infections, fetal and neonatal immune-induced thrombocytopenia, neonatal hemochromatosis, neonatal Kawasaki disease, and some types of immunodeficiency...
November 4, 2020: Antibodies
https://read.qxmd.com/read/32031146/neonatal-hemochromatosis-a-rare-cause-of-liver-failure-in-infancy
#26
JOURNAL ARTICLE
Priyanka M Chaudhuri, Bappa Mandal, Aniket Halder, Ranajoy Ghosh, Uttara Chatterjee, Suchandra Mukherjee
No abstract text is available yet for this article.
January 2020: Indian Journal of Pathology & Microbiology
https://read.qxmd.com/read/31978013/liver-failure-among-young-saudi-infants-etiology-clinical-presentation-and-outcome
#27
JOURNAL ARTICLE
Khurram Sadiq Lone, Badr AlSaleem, Ali Asery, Muhammed Salman Bashir, Abdulrahman Al-Hussaini
OBJECTIVES: The published data on early infantile liver failure (EILF) are scarce and limited to Caucasians. We conducted this study to describe the etiology and outcome of EILF among Arabs and identify prognostic factors. METHODS: We retrospectively reviewed our database of 524 infants presenting with liver impairment from 2008 to 2018, and identified cases of EILF defined as presence of biochemical pattern of liver disease and INR ≥2 (unresponsive to vitamin K) with onset before 3 months of life...
February 2020: Journal of Pediatric Gastroenterology and Nutrition
https://read.qxmd.com/read/31714476/liver-failure-among-young-saudi-infants-etiology-clinical-presentation-and-outcome
#28
JOURNAL ARTICLE
Khurram Sadiq Lone, Badr Al Saleem, Ali Asery, Muhammed Salman Bashir, Abdulrahman Al-Hussaini
OBJECTIVES: The published data on early infantile liver failure (EILF) are scarce and limited to Caucasians. We conducted this study to describe the etiology and outcome of EILF among Arabs and identify prognostic factors. METHODS: We retrospectively reviewed our database of 524 infants presenting with liver impairment from 2008 to 2018, and identified cases of EILF defined as presence of biochemical pattern of liver disease and INR ≥ 2 (unresponsive to vitamin K) with onset before 3 months of life...
November 9, 2019: Journal of Pediatric Gastroenterology and Nutrition
https://read.qxmd.com/read/31505487/managing-the-unusual-causes-of-fetal-anemia
#29
JOURNAL ARTICLE
Emeline Maisonneuve, Imane Ben M'Barek, Thierry Leblanc, Lydie Da Costa, Stéphanie Friszer, Françoise Pernot, Pauline Thomas, Vanina Castaigne, Cécile Toly N'Dour, Agnès Mailloux, Anne Cortey, Jean-Marie Jouannic
BACKGROUND: Rare causes of fetal anemia requiring intrauterine transfusion (IUT) are challenging for fetal medicine specialists. OBJECTIVES: The aim of this study was to describe the perinatal patterns and prognosis in a consecutive series of fetuses transfused for fetal anemia of rare or unknown etiology, and to propose a protocol of investigation for fetal anemia of undetermined cause and for the management of subsequent pregnancies. METHOD: We conducted a retrospective descriptive study on fetuses transfused for severe anemia of rare or unknown etiology managed in our national referral center (Centre National de Référence d'Hémobiologie Périnatale) and born between 2010 and 2017...
2020: Fetal Diagnosis and Therapy
https://read.qxmd.com/read/31424295/hemophagocytic-lymphohistiocytosis-mimicking-neonatal-hemochromatosis
#30
JOURNAL ARTICLE
Larisa Broglie, Bernadette Vitola, Monica S Thakar, Donald Basel, Sara Szabo, Rashmi Agni, Julie-An Talano
Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal illness characterized by impaired natural killer (NK) cell and cytotoxic T-cell function. Patients develop systemic inflammation, multisystem organ dysfunction, and if untreated, death. Patients who present in the neonatal period often have atypical presentations with evidence of liver dysfunction and cholestasis; this has a broad differential diagnosis including neonatal infection, congenital liver defects, or other causes of liver dysfunction, such as neonatal hemochromatosis...
October 2019: Pediatric Hematology and Oncology
https://read.qxmd.com/read/31407854/sub-acute-neonatal-hemochromatosis-in-an-infant-with-hypoplastic-left-heart-syndrome-on-ventricular-assist-device-awaiting-transplantation
#31
Hanna J Tadros, Dipankar Gupta, Megan Childress, Genie Beasley, Ashlie E Rubrecht, Archana Shenoy, Joseph Philip, Mark S Bleiweis, Desiree S Machado
Single-ventricle pediatric patients, amongst other children waiting for OHT, are a vulnerable population, especially if candidacy is established before any palliation. NH is a rare disease with poor prognosis in the post-natal period. We present a case of sub-acute NH diagnosed in an infant with HLHS who was listed for OHT while bridged with a pulsatile paracorporeal VAD, with an emphasis on the evolution of the condition throughout the patient's clinical course and the ultimate decision for compassionate deactivation of VAD...
November 2019: Pediatric Transplantation
https://read.qxmd.com/read/31359540/neonatal-hemochromatosis-associated-with-rupture-of-esophageal-varices
#32
JOURNAL ARTICLE
Kazuki Hatayama, Yosuke Washio, Tomoka Okamura, Takuo Noda, Hirokazu Tsukahara
No abstract text is available yet for this article.
July 2019: Pediatrics International: Official Journal of the Japan Pediatric Society
https://read.qxmd.com/read/31215296/neonatal-hemochromatosis-in-a-newborn-with-down-syndrome
#33
JOURNAL ARTICLE
Rasheeda M, Suvendu Purkait, Amit Kumar Satapathy, Joseph John, Susama Patra, Suvradeep Mitra
Background : Neonatal hemochromatosis (NH) is a cause of neonatal/pediatric acute liver failure. Liver dysfunction/failure in Down syndrome had been described in relation to increased susceptibility to infection and transient myeloproliferative disease (TMD). The occurrence of NH in Down syndrome is described in only a few case reports. Material and methods : A complete autopsy have been performed in a 79-day-old infant with severe liver dysfunction. TMD was suspected antemortem following a report of peripheral blood leukocytosis with 14% atypical cells...
February 2020: Fetal and Pediatric Pathology
https://read.qxmd.com/read/31081242/pediatric-liver-transplantation-for-neonatal-onset-niemann-pick-disease-type-c-japanese-multicenter-experience
#34
Naoya Yamada, Ayano Inui, Yukihiro Sanada, Yoshiyuki Ihara, Taizen Urahashi, Akinari Fukuda, Seisuke Sakamoto, Mureo Kasahara, Atsushi Yoshizawa, Shinya Okamoto, Hideaki Okajima, Tomoo Fujisawa, Koichi Mizuta
Niemann-Pick disease type C (NPC) is a rare autosomal recessive inherited disease characterized by lysosomal accumulation of free cholesterol in macrophages within multiple organs. Infantile-onset NPC often presents with jaundice and hepatosplenomegaly from birth, but these symptoms usually improve during early childhood, and it rarely progresses to liver failure. We report three cases from different hospitals in Japan; the patients developed neonatal-onset NPC, and liver transplantation (LT) was performed as a life-saving procedure...
May 12, 2019: Pediatric Transplantation
https://read.qxmd.com/read/30737051/a-case-of-niemann-pick-disease-type-c-with-neonatal-liver-failure-initially-diagnosed-as-neonatal-hemochromatosis
#35
JOURNAL ARTICLE
Tadayuki Kumagai, Hiroshi Terashima, Hajime Uchida, Akinari Fukuda, Mureo Kasahara, Motomichi Kosuga, Torayuki Okuyama, Tomoyuki Tsunoda, Ayano Inui, Tomoo Fujisawa, Aya Narita, Yoshikatsu Eto, Masaya Kubota
BACKGROUND: Niemann-Pick type C (NPC) is a lysosomal lipid storage disease with mutation of NPC1/NPC2 genes, which transport lipids in the endosome and lysosome, and various neurological symptoms. NPC patients also develop hepatosplenomegaly or liver disorder in the neonatal period, and 10% suffer severe liver failure. Neonatal hemochromatosis (NH) is a liver disorder characterized by hepatic and extrahepatic siderosis. Although the etiology of NH is unclear, recent reports suggest that the gestational alloimmune mechanism is the cause of NH...
May 2019: Brain & Development
https://read.qxmd.com/read/30702442/platelet-derived-%C3%AE-2m-regulates-monocyte-inflammatory-responses
#36
JOURNAL ARTICLE
Zachary T Hilt, Daphne N Pariser, Sara K Ture, Amy Mohan, Pearl Quijada, Akua A Asante, Scott J Cameron, Julie A Sterling, Alyssa R Merkel, Andrew L Johanson, Jermaine L Jenkins, Eric M Small, Kathleen E McGrath, James Palis, Michael R Elliott, Craig N Morrell
β-2 Microglobulin (β2M) is a molecular chaperone for the major histocompatibility class I (MHC I) complex, hemochromatosis factor protein (HFE), and the neonatal Fc receptor (FcRn), but β2M may also have less understood chaperone-independent functions. Elevated plasma β2M has a direct role in neurocognitive decline and is a risk factor for adverse cardiovascular events. β2M mRNA is present in platelets at very high levels, and β2M is part of the activated platelet releasate. In addition to their more well-studied thrombotic functions, platelets are important immune regulatory cells that release inflammatory molecules and contribute to leukocyte trafficking, activation, and differentiation...
March 7, 2019: JCI Insight
https://read.qxmd.com/read/30465890/prenatal-imaging-features-suggestive-of-liver-gestational-allo-immune-disease
#37
JOURNAL ARTICLE
Clémentine Sciard, Sophie Collardeau-Frachon, Anthony Atallah, Danièle Combourieu, Jérôme Massardier, Sophie Heissat, Pascal Gaucherand, Laurent Guibaud, Mona Massoud
We report prenatal imaging features of four cases of neonatal hemochromatosis due to an alloimmune disease. All cases exhibited intra uterine growth restriction (IUGR) without arguments for a vascular etiology, associated with oligohydramnios. Placental hydrops was present in 75% of cases. Splenomegaly was identified in one case. Other causes of NH have been ruled out during diagnostic workup including karyotype, detection of IGFBP-1 to evaluate a premature rupture of membranes, maternal serologic tests. MRI was performed in two cases and showed an atrophic liver associated with a low signal intensity on T2-sequence in one case...
January 2019: Journal of Gynecology Obstetrics and Human Reproduction
https://read.qxmd.com/read/30464860/heterogeneous-presentation-of-neonatal-hemochromatosis-in-dichorionic-twins
#38
JOURNAL ARTICLE
Yee Yuet Chee, Siu Chun Mabel Wong, Ming Sum Rosanna Wong
Acute liver failure (ALF) in neonates is rare. Although the incidence is reported to be rare, neonatal hemochromatosis (NH) has to be considered as one of the causes of neonatal ALF. We present a pair of dichorionic twin who had a diverse clinical presentation of NH. One twin passed away despite medical treatment with exchange transfusion and intravenous immunoglobulin (IVIg), whereas the other twin suffered from only mildly deranged liver function, which normalized spontaneously. Early identification of liver failure and clinical awareness of this disease entity are essential to its timely diagnosis and treatment...
October 2018: American Journal of Perinatology Reports
https://read.qxmd.com/read/30149691/biochemical-profile-in-an-infant-with-neonatal-hemochromatosis-shows-evidence-of-impairment-of-mitochondrial-long-chain-fatty-acid-oxidation
#39
JOURNAL ARTICLE
Karina Lucio de Medeiros Bastos, Caio Robledo Quaio, Fabiana Roberto Lima, Iana Manuelle Araújo, Candice Alves Tavares Araújo, Flávia Balbo Piazzon, Ismael Dale Cotrim Guerreiro da Silva, Gabriel Nuncio Benevides, Ana Cristina Tannuri, Uenis Tannuri, Ramiro Anthero Azevedo, Chong Ae Kim
No abstract text is available yet for this article.
August 28, 2018: Clinical and Molecular Hepatology
https://read.qxmd.com/read/30058407/broadening-the-spectrum-of-neonatal-hemochromatosis
#40
JOURNAL ARTICLE
Dídac Casas-Alba, Jordi Clotet, Emilio J Inarejos, Cristina Jou, Carme Fons, Cristina Molera
Neonatal hemochromatosis (NH) has been defined as neonatal liver disorder accompanied by extrahepatic siderosis, and gestational alloimmune liver disease (GALD) is the main cause of NH. We report an atypical case of NH that may have gone underdiagnosed. A male infant was born at term after an uneventful antenatal period. At 7 h of life, he was noted to be tachypneic. Chest X-ray was normal and capillary blood gas analysis showed severe lactic acidosis. An extended blood test showed elevated levels of tyrosine and methionine that, after excluding an inborn error of metabolism, led to the diagnosis of acute liver failure...
March 2020: Journal of Maternal-fetal & Neonatal Medicine
keyword
keyword
11454
2
3
Fetch more papers »
Fetching more papers... Fetching...
Remove bar
Read by QxMD icon Read
×

Save your favorite articles in one place with a free QxMD account.

×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"

We want to hear from doctors like you!

Take a second to answer a survey question.