Recommendations for diagnosis and treatment of methemoglobinemia

Methemoglobinemia is a rare disorder associated with oxidization of divalent ferro‐iron of hemoglobin (Hb) to ferri‐iron of methemoglobin (MetHb). Methemoglobinemia can result from either inherited or acquired processes. Acquired forms are the most common, mainly due to the exposure to substances th...

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Published inAmerican journal of hematology Vol. 96; no. 12; pp. 1666 - 1678
Main Authors Iolascon, Achille, Bianchi, Paola, Andolfo, Immacolata, Russo, Roberta, Barcellini, Wilma, Fermo, Elisa, Toldi, Gergely, Ghirardello, Stefano, Rees, Davis, Van Wijk, Richard, Kattamis, Antonis, Gallagher, Patrick G., Roy, Noemi, Taher, Ali, Mohty, Razan, Kulozik, Andreas, De Franceschi, Lucia, Gambale, Antonella, De Montalembert, Mariane, Forni, Gian Luca, Harteveld, Cornelis L., Prchal, Josef
Format Journal Article
LanguageEnglish
Published Hoboken, USA John Wiley & Sons, Inc 01.12.2021
Wiley Subscription Services, Inc
Subjects
Children
Consensus
Critical Review
Critical Reviews
Diagnosis
Diagnosis, Differential
Disease Management
Hematology
Hemoglobin
Humans
Iron
Methemoglobin
Methemoglobinemia
Methemoglobinemia - diagnosis
Methemoglobinemia - physiopathology
Methemoglobinemia - therapy
Neonates
Oxidation
Online AccessGet full text

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Abstract Methemoglobinemia is a rare disorder associated with oxidization of divalent ferro‐iron of hemoglobin (Hb) to ferri‐iron of methemoglobin (MetHb). Methemoglobinemia can result from either inherited or acquired processes. Acquired forms are the most common, mainly due to the exposure to substances that cause oxidation of the Hb both directly or indirectly. Inherited forms are due either to autosomal recessive variants in the CYB5R3 gene or to autosomal dominant variants in the globin genes, collectively known as HbM disease. Our recommendations are based on a systematic literature search. A series of questions regarding the key signs and symptoms, the methods for diagnosis, the clinical management in neonatal/childhood/adulthood period, and the therapeutic approach of methemoglobinemia were formulated and the relative recommendations were produced. An agreement was obtained using a Delphi‐like approach and the experts panel reached a final consensus >75% of agreement for all the questions.
AbstractList Methemoglobinemia is a rare disorder associated with oxidization of divalent ferro‐iron of hemoglobin (Hb) to ferri‐iron of methemoglobin (MetHb). Methemoglobinemia can result from either inherited or acquired processes. Acquired forms are the most common, mainly due to the exposure to substances that cause oxidation of the Hb both directly or indirectly. Inherited forms are due either to autosomal recessive variants in the CYB5R3 gene or to autosomal dominant variants in the globin genes, collectively known as HbM disease. Our recommendations are based on a systematic literature search. A series of questions regarding the key signs and symptoms, the methods for diagnosis, the clinical management in neonatal/childhood/adulthood period, and the therapeutic approach of methemoglobinemia were formulated and the relative recommendations were produced. An agreement was obtained using a Delphi‐like approach and the experts panel reached a final consensus >75% of agreement for all the questions.
Methemoglobinemia is a rare disorder associated with oxidization of divalent ferro‐iron of hemoglobin (Hb) to ferri‐iron of methemoglobin (MetHb). Methemoglobinemia can result from either inherited or acquired processes. Acquired forms are the most common, mainly due to the exposure to substances that cause oxidation of the Hb both directly or indirectly. Inherited forms are due either to autosomal recessive variants in the CYB5R3 gene or to autosomal dominant variants in the globin genes, collectively known as HbM disease. Our recommendations are based on a systematic literature search. A series of questions regarding the key signs and symptoms, the methods for diagnosis, the clinical management in neonatal/childhood/adulthood period, and the therapeutic approach of methemoglobinemia were formulated and the relative recommendations were produced. An agreement was obtained using a Delphi‐like approach and the experts panel reached a final consensus >75% of agreement for all the questions.
Methemoglobinemia is a rare disorder associated with oxidization of divalent ferro-iron of hemoglobin (Hb) to ferri-iron of methemoglobin (MetHb). Methemoglobinemia can result from either inherited or acquired processes. Acquired forms are the most common, mainly due to the exposure to substances that cause oxidation of the Hb both directly or indirectly. Inherited forms are due either to autosomal recessive variants in the CYB5R3 gene or to autosomal dominant variants in the globin genes, collectively known as HbM disease. Our recommendations are based on a systematic literature search. A series of questions regarding the key signs and symptoms, the methods for diagnosis, the clinical management in neonatal/childhood/adulthood period, and the therapeutic approach of methemoglobinemia were formulated and the relative recommendations were produced. An agreement was obtained using a Delphi-like approach and the experts panel reached a final consensus >75% of agreement for all the questions.Methemoglobinemia is a rare disorder associated with oxidization of divalent ferro-iron of hemoglobin (Hb) to ferri-iron of methemoglobin (MetHb). Methemoglobinemia can result from either inherited or acquired processes. Acquired forms are the most common, mainly due to the exposure to substances that cause oxidation of the Hb both directly or indirectly. Inherited forms are due either to autosomal recessive variants in the CYB5R3 gene or to autosomal dominant variants in the globin genes, collectively known as HbM disease. Our recommendations are based on a systematic literature search. A series of questions regarding the key signs and symptoms, the methods for diagnosis, the clinical management in neonatal/childhood/adulthood period, and the therapeutic approach of methemoglobinemia were formulated and the relative recommendations were produced. An agreement was obtained using a Delphi-like approach and the experts panel reached a final consensus >75% of agreement for all the questions.
Author De Montalembert, Mariane
Kattamis, Antonis
Van Wijk, Richard
Mohty, Razan
Toldi, Gergely
Russo, Roberta
Iolascon, Achille
Andolfo, Immacolata
Kulozik, Andreas
Gambale, Antonella
Harteveld, Cornelis L.
Fermo, Elisa
Rees, Davis
Taher, Ali
Gallagher, Patrick G.
De Franceschi, Lucia
Roy, Noemi
Prchal, Josef
Bianchi, Paola
Ghirardello, Stefano
Forni, Gian Luca
Barcellini, Wilma
AuthorAffiliation 4 Department of Neonatology Birmingham Women's and Children's Hospital Birmingham UK
12 Department of Pediatric Oncology, Hematology and Immunology University of Heidelberg, Hopp‐ Children's Cancer Research Center (KiTZ) Heidelberg Germany
14 Department of Laboratory Medicine (DAIMedLab), UOC Medical Genetics ‘Federico II’ University Hospital Naples Italy
7 Central Diagnostic Laboratory University Medical Center Utrecht, Utrecht University Utrecht The Netherlands
9 Departments of Pediatrics, Pathology, and Genetics Yale University New Haven Connecticut USA
15 Pédiatrie générale et maladies infectieuses Centre de référence de la drépanocytose, Hôpital Necker‐Enfants Malades, APHP Paris Paris France
18 Hematology University of Utah & Huntsman Cancer Center Salt Lake City Utah USA
1 Dipartimento di Medicina Molecolare e Biotecnologie Mediche Università degli Studi di Napoli Federico II Napoli Italy
5 Neonatal Intensive Care Unit Fondazione IRCCS Policlinico San Matteo Pavia Italy
16 Centro della M
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– name: 1 Dipartimento di Medicina Molecolare e Biotecnologie Mediche Università degli Studi di Napoli Federico II Napoli Italy
– name: 9 Departments of Pediatrics, Pathology, and Genetics Yale University New Haven Connecticut USA
– name: 16 Centro della Microcitemia e Anemie Congenite Genova Italy
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BackLink https://www.ncbi.nlm.nih.gov/pubmed/34467556$$D View this record in MEDLINE/PubMed
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CorporateAuthor SWG of red cell and iron of EHA and EuroBloodNet
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SecondaryResourceType review_article
Snippet Methemoglobinemia is a rare disorder associated with oxidization of divalent ferro‐iron of hemoglobin (Hb) to ferri‐iron of methemoglobin (MetHb)....
Methemoglobinemia is a rare disorder associated with oxidization of divalent ferro-iron of hemoglobin (Hb) to ferri-iron of methemoglobin (MetHb)....
SourceID pubmedcentral
proquest
pubmed
crossref
wiley
SourceType Open Access Repository
Aggregation Database
Index Database
Enrichment Source
Publisher
StartPage 1666
SubjectTerms Children
Consensus
Critical Review
Critical Reviews
Diagnosis
Diagnosis, Differential
Disease Management
Hematology
Hemoglobin
Humans
Iron
Methemoglobin
Methemoglobinemia
Methemoglobinemia - diagnosis
Methemoglobinemia - physiopathology
Methemoglobinemia - therapy
Neonates
Oxidation
Title Recommendations for diagnosis and treatment of methemoglobinemia
URI https://onlinelibrary.wiley.com/doi/abs/10.1002%2Fajh.26340
https://www.ncbi.nlm.nih.gov/pubmed/34467556
https://www.proquest.com/docview/2601570285
https://www.proquest.com/docview/2568251622
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Volume 96
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