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<meta name="keywords" content="C0025637, disease or syndrome, finding, methemoglobinemia, methemoglobinemias, autosomal dominant, autosomal recessive, birth defects, chromosomal disease, chromosome, clinical features, clinical findings, clinical genetics, clinical recommendations, clinvar, congenital chromosomal disease, consumer genetic resources, cytogenetic location, disease characteristics, disease definitions, disease descriptions, disease ontology, disease synonyms, disease vocabulary, dysmorphology, entrez, familial disease, gene, gene-disease relationship, genereviews, genetic disease, genetic disorder, genetic terminology, genetic testing registry, genetics home reference, genomic disease, gtr, hereditary disease, heritable disease, hpo, human phenotype ontology, inherited disease, management guidelines, maternal inheritance, medgen, medical genetics, medical subject headings, mesh, mitochondrial inheritance, mode of inheritance, national center for biotechnology information, national institutes of health, national library of medicine, ncbi, nih, nlm, omim, ordo, orphanet, paternal inheritance, phenome, position statements, professional practice guidelines, rare disease, reference sequence, refseq, snomed ct, syndrome, undiagnosed diseases, x-linked recessive" /><meta name="description" content="Abnormally increased levels of methemoglobin in the blood. In this form of hemoglobin, there is an oxidized ferric iron (Fe +3) rather than the reduced ferrous form (Fe 2+) that is normally found in hemoglobin. Methemoglobin has a reduced affinity for oxygen, resulting in a reduced ability to release oxygen to tissues." /><meta name="robots" content="index,nofollow,noarchive" />
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<title>Methemoglobinemia (Concept Id: C0025637)
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<!--imgCountBooks = 0--><h1 class="medgenTitle"><div class="MedGenTitleText">Methemoglobinemia</div></h1><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>6339</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information."><span class="highlight" style="background-color:">C0025637</span></a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome; Finding</dd></dl></div></div><table class="medgenTable"><tbody><tr><td>Synonym:</td>
<td>Methemoglobinemias</td></tr>
<tr><td><span class="bold">SNOMED CT: </span></td>
<td>Methemoglobinemia (38959009)</td></tr>
<tr><td colspan="2" class="small"> </td></tr><tr><td>HPO:</td>
<td><a target="_blank" title="Human Phenotype Ontology" href="https://hpo.jax.org/app/browse/term/HP:0012119">HP:0012119</a></td></tr>
<tr><td>Monarch Initiative:</td>
<td><a href="https://monarchinitiative.org/disease/MONDO:0001117" target="_blank">MONDO:0001117</a></td></tr>
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Definition">Definition</h1><a sid="100" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln">Abnormally increased levels of methemoglobin in the blood. In this form of hemoglobin, there is an oxidized ferric iron (Fe +3) rather than the reduced ferrous form (Fe 2+) that is normally found in hemoglobin. Methemoglobin has a reduced affinity for oxygen, resulting in a reduced ability to release oxygen to tissues. [from <a title="Human Phenotype Ontology" href="http://www.human-phenotype-ontology.org" class="defSource" target="_blank">HPO</a>]</div>
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<div class="portlet mgSection" id="ID_118">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Term_Hierarchy">Term Hierarchy</h1><a sid="118" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln HierarchyGTR"><div class="jig-ncbitabs"><ul><li><a href="#tabGTR">GTR</a></li><li><a href="#tabMGEN">MeSH</a></li></ul><div id="tabGTR"><div class="search_result"><div class="rprts"><div class="chiclet_legend"><span class="chiclet_list" style="position:static;"><span title="Clinical test" class="chiclet Ccolor round">C</span><span>Clinical test,  </span><span title="Research test" class="chiclet Rcolor round">R</span><span>Research test,  </span><span title="OMIM" class="chiclet Ocolor ">O</span><span>OMIM,  </span><span title="GeneReview" class="chiclet Gcolor">G</span><span><em>GeneReviews</em>,  </span><span title="ClinVar" class="chiclet Vcolor">V</span><span>ClinVar  </span></span></div><div id="hierarchy" class="margin_t1"><div class="ds_tree"><ul><li class="matched_ds"><span class="TLline">Methemoglobinemia</span></li></ul></div></div></div></div></div><div id="tabMGEN"><div class="ds_tree"><ul><li><span class="TLline"><a href="/medgen/867443" ref="tree=MeSH" title="MedGen record for Phenotypic abnormality">Phenotypic abnormality</a></span><ul><li><span class="TLline"><a href="/medgen/163092" ref="tree=MeSH" title="MedGen record for Abnormality of blood and blood-forming tissues">Abnormality of blood and blood-forming tissues</a></span><ul><li><span class="TLline"><a href="/medgen/488883" ref="tree=MeSH" title="MedGen record for Abnormal erythrocyte morphology">Abnormal erythrocyte morphology</a></span><ul><li><span class="TLline"><a href="/medgen/776552" ref="tree=MeSH" title="MedGen record for Abnormal hemoglobin">Abnormal hemoglobin</a></span><ul><li><span class="matched_ds">Methemoglobinemia</span><ul><li><span class="TLline"><a href="/medgen/473012" ref="tree=MeSH" title="MedGen record for Acquired methemoglobinemia">Acquired methemoglobinemia</a></span><ul><li><span class="TLline"><a href="/medgen/632786" ref="tree=MeSH" title="MedGen record for Drug-induced methemoglobinemia">Drug-induced methemoglobinemia</a></span></li><li><span class="TLline"><a href="/medgen/688234" ref="tree=MeSH" title="MedGen record for Toxic methemoglobinemia with cyanosis">Toxic methemoglobinemia with cyanosis</a></span></li></ul></li><li><span class="TLline"><a href="/medgen/473013" ref="tree=MeSH" title="MedGen record for Hereditary methemoglobinemia">Hereditary methemoglobinemia</a></span><ul><li><span class="TLline"><a href="/medgen/75661" ref="tree=MeSH" title="MedGen record for Deficiency of cytochrome-b5 reductase">Deficiency of cytochrome-b5 reductase</a></span></li><li><span class="TLline"><a href="/medgen/501116" ref="tree=MeSH" title="MedGen record for Methemoglobin reductase deficiency">Methemoglobin reductase deficiency</a></span></li><li><span class="TLline"><a href="/medgen/925090" ref="tree=MeSH" title="MedGen record for Methemoglobinemia type 4">Methemoglobinemia type 4</a></span></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></div></div></div></div>
</div>
<div class="portlet mgSection" id="ID_112">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Conditions_with_this_feature">Conditions with this feature</h1><a sid="112" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln clinfeat">
<div class="divPopper rprt" id="rdis_75661"><div><strong>Deficiency of cytochrome-b5 reductase</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>75661</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C0268193</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Methemoglobinemia due to NADH-cytochrome b5 reductase deficiency is an autosomal recessive disorder characterized clinically by decreased oxygen carrying capacity of the blood, with resultant cyanosis and hypoxia (review by Percy and Lappin, 2008).&#13; There are 2 types of methemoglobin reductase deficiency. In type I, the defect affects the soluble form of the enzyme, is restricted to red blood cells, and causes well-tolerated methemoglobinemia. In type II, the defect affects both the soluble and microsomal forms of the enzyme and is thus generalized, affecting red cells, leukocytes, and all body tissues. Type II methemoglobinemia is associated with mental deficiency and other neurologic symptoms. The neurologic symptoms may be related to the major role played by the cytochrome b5 system in the desaturation of fatty acids (Vives-Corrons et al., 1978; Kaplan et al., 1979).</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/75661">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_333645"><div><strong>METHEMOGLOBINEMIA, BETA TYPE</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>333645</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C1840779</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Methemoglobinemia is a clinical condition in which more than 1% of hemoglobin is oxidized to methemoglobin, a type of hemoglobin that contains the ferric (Fe3+) form of iron. Patients with hemoglobin M are cyanotic but otherwise asymptomatic. If the mutation occurs in the hemoglobin alpha subunit (141800), cyanosis is apparent at birth, whereas if the beta chain is affected, cyanosis appears later or intensifies when beta subunit production increases. If a newborn carries a fetal M hemoglobin (gamma subunit; 142250), cyanosis disappears when the complete gamma-beta-switch occurs (summary by Mansouri and Lurie, 1993).</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/333645">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_395437"><div><strong>ACETOPHENETIDIN SENSITIVITY</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>395437</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C1860214</a></dd><dt><span class="dotprefix"></span></dt><dd>Finding</dd></dl></div></div></div>
<div class="spaceAbove nowrap">See: <a href="/medgen/395437">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_462771"><div><strong>Cyanosis, transient neonatal</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>462771</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C3151421</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Neonatal cyanosis is characterized by symptoms in the fetus and neonate that gradually abate by 5 to 6 months of age. The disorder is caused by a defect in the fetal hemoglobin chain, which causes reduced affinity for oxygen due to steric inhibition of oxygen binding and/or due to increased oxidation of the fetal hemoglobin molecule to methemoglobin (Hb FM), which has decreased oxygen-binding capacity. Some patients develop anemia resulting from increased destruction of red cells containing abnormal or unstable hemoglobin. The cyanosis resolves spontaneously by 5 to 6 months of age or earlier, as the adult beta-globin chain (HBB; 141900) is produced and replaces the fetal gamma-globin chain (summary by Crowley et al., 2011).</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/462771">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_925090"><div><strong>Methemoglobinemia type 4</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>925090</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C4285231</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Methemoglobinemia and ambiguous genitalia (METAG) is due to isolated 17,20-lyase deficiency, defined by apparently normal 17-alpha-hydroxylase activity but severely reduced 17,20-lyase activity of the CYP17A1 enzyme (609300), which results in sex steroid deficiency but normal glucocorticoid and mineralocorticoid reserve. The clinical phenotype is characterized by male undermasculinization, with absent or disturbed pubertal development in both 46,XY and 46,XX individuals. Mild to severe methemoglobinemia has been reported in these patients (Idkowiak et al., 2012).&#13; Other autosomal recessive methemoglobinemias include types I and II (see 250800), caused by mutation in the CYB5R3 gene (613213). Isolated 17,20-lyase deficiency can also be caused by mutation in the CYP17A1 gene (609300), and mutation in the POR gene can manifest clinically as isolated 17,20-lyase deficiency (see 124015.0016).</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/925090">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_1635511"><div><strong>Methemoglobinemia, alpha type</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1635511</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C4693798</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Methemoglobinemia is a clinical condition in which more than 1% of hemoglobin is oxidized to methemoglobin, a type of hemoglobin that contains the ferric (Fe3+) form of iron. Patients with hemoglobin M are cyanotic but otherwise asymptomatic. If the mutation occurs in the hemoglobin alpha subunit, cyanosis is apparent at birth, whereas if the beta chain (141900) is affected, cyanosis appears later or intensifies when beta subunit production increases. If a newborn carries a fetal M hemoglobin (gamma subunit; 142250), cyanosis disappears when the complete gamma-beta-switch occurs (summary by Mansouri and Lurie, 1993).</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/1635511">Condition Record</a></div></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_395437" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">ACETOPHENETIDIN SENSITIVITY</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_462771" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Cyanosis, transient neonatal</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_75661" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Deficiency of cytochrome-b5 reductase</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_925090" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Methemoglobinemia type 4</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1635511" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Methemoglobinemia, alpha type</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_333645" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">METHEMOGLOBINEMIA, BETA TYPE</a></div></div>
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<div class="portlet_content ln"><h3 class="subhead">PubMed<a class="help jig-ncbi-popper" data-jig="ncbipopper" href="#guidelinesHelpPM"><img class="pulldown" src="//static.pubmed.gov/portal/portal3rc.fcgi/4223267/img/4204968" /></a></h3>
<div class="nl"><a target="_blank" href="/pubmed/37721023">2023 American Heart Association Focused Update on the Management of Patients With Cardiac Arrest or Life-Threatening Toxicity Due to Poisoning: An Update to the American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Lavonas EJ,
Akpunonu PD,
Arens AM,
Babu KM,
Cao D,
Hoffman RS,
Hoyte CO,
Mazer-Amirshahi ME,
Stolbach A,
St-Onge M,
Thompson TM,
Wang GS,
Hoover AV,
Drennan IR;
American Heart Association</span><br />
<span class="medgenPMjournal">Circulation</span>
2023 Oct 17;148(16):e149-e184.
Epub 2023 Sep 18
doi: 10.1161/CIR.0000000000001161.
<span class="bold">PMID: </span><a href="/pubmed/37721023" target="_blank">37721023</a></div>
<div class="nl"><a target="_blank" href="/pubmed/34467556">Recommendations for diagnosis and treatment of methemoglobinemia.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Iolascon A,
Bianchi P,
Andolfo I,
Russo R,
Barcellini W,
Fermo E,
Toldi G,
Ghirardello S,
Rees D,
Van Wijk R,
Kattamis A,
Gallagher PG,
Roy N,
Taher A,
Mohty R,
Kulozik A,
De Franceschi L,
Gambale A,
De Montalembert M,
Forni GL,
Harteveld CL,
Prchal J;
SWG of red cell and iron of EHA and EuroBloodNet</span><br />
<span class="medgenPMjournal">Am J Hematol</span>
2021 Dec 1;96(12):1666-1678.
Epub 2021 Sep 23
doi: 10.1002/ajh.26340.
<span class="bold">PMID: </span><a href="/pubmed/34467556" target="_blank">34467556</a><a href="/pmc/articles/PMC9291883" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/22024786">Methemoglobinemia: pathogenesis, diagnosis, and management.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Skold A,
Cosco DL,
Klein R</span><br />
<span class="medgenPMjournal">South Med J</span>
2011 Nov;104(11):757-61.
doi: 10.1097/SMJ.0b013e318232139f.
<span class="bold">PMID: </span><a href="/pubmed/22024786" target="_blank">22024786</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(%22methemoglobinemia%22%5Btiab%3A~0%5D)%20AND%20(%22english%20and%20humans%22%5BFilter%5D)%20AND%20(%20(%22practice%20guideline%22%5BFilter%5D)%20OR%20(practice*%5Btitl%5D%20AND%20(guideline%5Btitl%5D%20OR%20parameter%5Btitl%5D%20OR%20resource%5Btitl%5D%20OR%20bulletin%5Btitl%5D%20OR%20best%5Btitl%5D))%20OR%20(genetic*%5Btitl%5D%20AND%20(evaluation%5Btitl%5D%20OR%20counseling%5Btitl%5D%20OR%20screening%5Btitl%5D%20OR%20test*%5Btitl%5D))%20OR%20(clinical%5Btitl%5D%20AND%20((expert%5Btitl%5D%20AND%20consensus%5Btitl%5D)%20OR%20utility%5Btitl%5D%20OR%20guideline*%5Btitl%5D))%20OR%20(management%5Btitl%5D%20AND%20(clinical%5Btitl%5D%20OR%20diagnos*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20pain%5Btitl%5D%20OR%20surveillance%5Btitl%5D%20OR%20emergency%5Btitl%5D%20OR%20guideline*%5Btitl%5D%20OR%20therap*))%20OR%20(treatment%5Btitl%5D%20AND%20((evaluation%5Btitl%5D%20AND%20diagnosis%5Btitl%5D)%20OR%20(assessment%5Btitl%5D%20AND%20prevention%5Btitl%5D)%20OR%20therap*))%20OR%20(Diagnos*%5Btitl%5D%20AND%20(prenatal%5Btitl%5D%20OR%20treatment%5Btitl%5D%20OR%20follow-up%5Btitl%5D%20OR%20statement%5Btitl%5D%20OR%20criteria%5Btitl%5D%20OR%20newborn%5Btitl%5D%20OR%20differential%5Btitl%5D%20OR%20neonatal%5Btitl%5D%20OR%20neonate%5Btitl%5D))%20OR%20(guideline*%5Btitl%5D%20AND%20(pharmacogenetic*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20evidence-based%5Btitl%5D%20OR%20consensus%5Btitl%5D%20OR%20(technical%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20(molecular%5Btitl%5D%20AND%20testing%5Btitl%5D)))%20OR%20(risk%5Btitl%5D%20AND%20assessment%5Btitl%5D)%20OR%20(recommendation*%5Btitl%5D%20AND%20(statement%5Btitl%5D%20OR%20Evidence-based%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(care%20AND%20((Patient%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20primary%5Btitl%5D%20OR%20psychosocial%5Btitl%5D))%20OR%20(Health%5Btitl%5D%20AND%20supervision%5Btitl%5D)%20OR%20(statement%5Btitl%5D%20AND%20(policy%5Btitl%5D%20OR%20position%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(pharmacogenetics%5Btitl%5D%20AND%20(Dosing%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20genotype*%5Btitl%5D%20OR%20drug*%5Btitl%5D))%20OR%20(Chemotherapy%5Btitl%5D%20AND%20decision*%5Btitl%5D)%20OR%20(screening%5Btitl%5D%20AND%20(newborn%5Btitl%5D%20OR%20neonat*%5Btitl%5D%20OR%20detection%5Btitl%5D%20OR%20diagnos*%5Btitl%5D))%20OR%20(criteria%5Btitl%5D%20OR%20genotype*%5Btitl%5D)%20)%20NOT%20(%22Case%20reports%22%5BPublication%20type%5D%20OR%20%22clinical%20study%22%5BPublication%20Type%5D%20OR%20%22randomized%20controlled%20trial%22%5BPublication%20Type%5D)" title="PubMed search">See all (52)</a></div></div>
</div>
<div class="display-none help-popup" id="guidelinesHelpPM">These guidelines are articles in PubMed that match specific search criteria developed by MedGen to capture the most relevant practice guidelines. This list may not be comprehensive and may include broader topics as well. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div><div class="display-none help-popup" id="guidelinesHelpCurated">These guidelines are manually curated by the MedGen team
to supplement articles available in PubMed. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div>
<div class="portlet mgSection" id="ID_103">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Recent_clinical_studies">Recent clinical studies</h1><a sid="103" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><h3 class="subhead">Etiology</h3>
<div class="nl"><a target="_blank" href="/pubmed/36966432">JAK2 unmutated erythrocytosis: 2023 Update on diagnosis and management.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Gangat N,
Szuber N,
Tefferi A</span><br />
<span class="medgenPMjournal">Am J Hematol</span>
2023 Jun;98(6):965-981.
Epub 2023 Apr 3
doi: 10.1002/ajh.26920.
<span class="bold">PMID: </span><a href="/pubmed/36966432" target="_blank">36966432</a></div>
<div class="nl"><a target="_blank" href="/pubmed/36678214">Favism: Clinical Features at Different Ages.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Beretta A,
Manuelli M,
Cena H</span><br />
<span class="medgenPMjournal">Nutrients</span>
2023 Jan 10;15(2)
doi: 10.3390/nu15020343.
<span class="bold">PMID: </span><a href="/pubmed/36678214" target="_blank">36678214</a><a href="/pmc/articles/PMC9864644" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/21739343">2,4-dinitrophenol (DNP): a weight loss agent with significant acute toxicity and risk of death.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Grundlingh J,
Dargan PI,
El-Zanfaly M,
Wood DM</span><br />
<span class="medgenPMjournal">J Med Toxicol</span>
2011 Sep;7(3):205-12.
doi: 10.1007/s13181-011-0162-6.
<span class="bold">PMID: </span><a href="/pubmed/21739343" target="_blank">21739343</a><a href="/pmc/articles/PMC3550200" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/18049203">Methemoglobinemia and benzocaine.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Moos DD,
Cuddeford JD</span><br />
<span class="medgenPMjournal">Gastroenterol Nurs</span>
2007 Sep-Oct;30(5):342-5; quiz 346-7.
doi: 10.1097/01.SGA.0000296253.78457.f4.
<span class="bold">PMID: </span><a href="/pubmed/18049203" target="_blank">18049203</a></div>
<div class="nl"><a target="_blank" href="/pubmed/12845393">Methylene blue.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Clifton J 2nd,
Leikin JB</span><br />
<span class="medgenPMjournal">Am J Ther</span>
2003 Jul-Aug;10(4):289-91.
doi: 10.1097/00045391-200307000-00009.
<span class="bold">PMID: </span><a href="/pubmed/12845393" target="_blank">12845393</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Methemoglobinemia%22%20AND%20Etiology%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (388)</a></div><h3 class="subhead">Diagnosis</h3>
<div class="nl"><a target="_blank" href="/pubmed/34467556">Recommendations for diagnosis and treatment of methemoglobinemia.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Iolascon A,
Bianchi P,
Andolfo I,
Russo R,
Barcellini W,
Fermo E,
Toldi G,
Ghirardello S,
Rees D,
Van Wijk R,
Kattamis A,
Gallagher PG,
Roy N,
Taher A,
Mohty R,
Kulozik A,
De Franceschi L,
Gambale A,
De Montalembert M,
Forni GL,
Harteveld CL,
Prchal J;
SWG of red cell and iron of EHA and EuroBloodNet</span><br />
<span class="medgenPMjournal">Am J Hematol</span>
2021 Dec 1;96(12):1666-1678.
Epub 2021 Sep 23
doi: 10.1002/ajh.26340.
<span class="bold">PMID: </span><a href="/pubmed/34467556" target="_blank">34467556</a><a href="/pmc/articles/PMC9291883" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/33648998">Methemoglobinemia.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Kaminecki I,
Huang D</span><br />
<span class="medgenPMjournal">Pediatr Rev</span>
2021 Mar;42(3):164-166.
doi: 10.1542/pir.2020-000943.
<span class="bold">PMID: </span><a href="/pubmed/33648998" target="_blank">33648998</a></div>
<div class="nl"><a target="_blank" href="/pubmed/22024786">Methemoglobinemia: pathogenesis, diagnosis, and management.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Skold A,
Cosco DL,
Klein R</span><br />
<span class="medgenPMjournal">South Med J</span>
2011 Nov;104(11):757-61.
doi: 10.1097/SMJ.0b013e318232139f.
<span class="bold">PMID: </span><a href="/pubmed/22024786" target="_blank">22024786</a></div>
<div class="nl"><a target="_blank" href="/pubmed/17379039">Methemoglobinemia.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Camp NE</span><br />
<span class="medgenPMjournal">J Emerg Nurs</span>
2007 Apr;33(2):172-4.
Epub 2007 Feb 9
doi: 10.1016/j.jen.2006.11.007.
<span class="bold">PMID: </span><a href="/pubmed/17379039" target="_blank">17379039</a></div>
<div class="nl"><a target="_blank" href="/pubmed/11527852">Methemoglobinemia.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Rehman HU</span><br />
<span class="medgenPMjournal">West J Med</span>
2001 Sep;175(3):193-6.
doi: 10.1136/ewjm.175.3.193.
<span class="bold">PMID: </span><a href="/pubmed/11527852" target="_blank">11527852</a><a href="/pmc/articles/PMC1071541" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Methemoglobinemia%22%20AND%20Diagnosis%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (714)</a></div><h3 class="subhead">Therapy</h3>
<div class="nl"><a target="_blank" href="/pubmed/31532963">Acquired Methemoglobinemia.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Warren OU,
Blackwood B</span><br />
<span class="medgenPMjournal">N Engl J Med</span>
2019 Sep 19;381(12):1158.
doi: 10.1056/NEJMicm1816026.
<span class="bold">PMID: </span><a href="/pubmed/31532963" target="_blank">31532963</a></div>
<div class="nl"><a target="_blank" href="/pubmed/22024786">Methemoglobinemia: pathogenesis, diagnosis, and management.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Skold A,
Cosco DL,
Klein R</span><br />
<span class="medgenPMjournal">South Med J</span>
2011 Nov;104(11):757-61.
doi: 10.1097/SMJ.0b013e318232139f.
<span class="bold">PMID: </span><a href="/pubmed/22024786" target="_blank">22024786</a></div>
<div class="nl"><a target="_blank" href="/pubmed/17379039">Methemoglobinemia.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Camp NE</span><br />
<span class="medgenPMjournal">J Emerg Nurs</span>
2007 Apr;33(2):172-4.
Epub 2007 Feb 9
doi: 10.1016/j.jen.2006.11.007.
<span class="bold">PMID: </span><a href="/pubmed/17379039" target="_blank">17379039</a></div>
<div class="nl"><a target="_blank" href="/pubmed/11527852">Methemoglobinemia.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Rehman HU</span><br />
<span class="medgenPMjournal">West J Med</span>
2001 Sep;175(3):193-6.
doi: 10.1136/ewjm.175.3.193.
<span class="bold">PMID: </span><a href="/pubmed/11527852" target="_blank">11527852</a><a href="/pmc/articles/PMC1071541" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/10653076">Benzocaine-induced methemoglobinemia.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Gupta PM,
Lala DS,
Arsura EL</span><br />
<span class="medgenPMjournal">South Med J</span>
2000 Jan;93(1):83-6.
<span class="bold">PMID: </span><a href="/pubmed/10653076" target="_blank">10653076</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Methemoglobinemia%22%20AND%20Therapy%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (1351)</a></div><h3 class="subhead">Prognosis</h3>
<div class="nl"><a target="_blank" href="/pubmed/29762952">Dapsone: An Update.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Karjigi S,
Murthy SC,
Kallappa H,
Kusuma MR,
Aruna B,
Reddy YN</span><br />
<span class="medgenPMjournal">Indian J Lepr</span>
2016 Sep;87(4):233-239.
<span class="bold">PMID: </span><a href="/pubmed/29762952" target="_blank">29762952</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26991714">Preventing Tumor Lysis Syndrome: Two Case Studies of Unexpected Outcomes.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Sleutel MR,
Brown W,
Wells JN</span><br />
<span class="medgenPMjournal">Clin J Oncol Nurs</span>
2016 Apr;20(2):195-200.
doi: 10.1188/16.CJON.195-200.
<span class="bold">PMID: </span><a href="/pubmed/26991714" target="_blank">26991714</a></div>
<div class="nl"><a target="_blank" href="/pubmed/22171210">Unexplained hypoxemia.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Spoon KC,
Ramar K</span><br />
<span class="medgenPMjournal">J Clin Sleep Med</span>
2011 Dec 15;7(6):679-80.
doi: 10.5664/jcsm.1484.
<span class="bold">PMID: </span><a href="/pubmed/22171210" target="_blank">22171210</a><a href="/pmc/articles/PMC3227717" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/11936041">Methaemoglobinaemia and poisoning.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">McCrea S</span><br />
<span class="medgenPMjournal">Emerg Nurse</span>
2001 Sep;9(5):18-22.
doi: 10.7748/en2001.09.9.5.18.c1368.
<span class="bold">PMID: </span><a href="/pubmed/11936041" target="_blank">11936041</a></div>
<div class="nl"><a target="_blank" href="/pubmed/4025991">Intravenous mushroom poisoning.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Curry SC,
Rose MC</span><br />
<span class="medgenPMjournal">Ann Emerg Med</span>
1985 Sep;14(9):900-2.
doi: 10.1016/s0196-0644(85)80643-0.
<span class="bold">PMID: </span><a href="/pubmed/4025991" target="_blank">4025991</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Methemoglobinemia%22%20AND%20Prognosis%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (162)</a></div><h3 class="subhead">Clinical prediction guides</h3>
<div class="nl"><a target="_blank" href="/pubmed/34943887">The Potentials of Methylene Blue as an Anti-Aging Drug.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Xue H,
Thaivalappil A,
Cao K</span><br />
<span class="medgenPMjournal">Cells</span>
2021 Dec 1;10(12)
doi: 10.3390/cells10123379.
<span class="bold">PMID: </span><a href="/pubmed/34943887" target="_blank">34943887</a><a href="/pmc/articles/PMC8699482" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/29762952">Dapsone: An Update.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Karjigi S,
Murthy SC,
Kallappa H,
Kusuma MR,
Aruna B,
Reddy YN</span><br />
<span class="medgenPMjournal">Indian J Lepr</span>
2016 Sep;87(4):233-239.
<span class="bold">PMID: </span><a href="/pubmed/29762952" target="_blank">29762952</a></div>
<div class="nl"><a target="_blank" href="/pubmed/27922248">Recessive congenital methemoglobinemia in immediate generations.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Aslan D,
Türköz-Sucak G,
Percy MJ</span><br />
<span class="medgenPMjournal">Turk J Pediatr</span>
2016;58(1):113-115.
doi: 10.24953/turkjped.2016.01.019.
<span class="bold">PMID: </span><a href="/pubmed/27922248" target="_blank">27922248</a></div>
<div class="nl"><a target="_blank" href="/pubmed/25976309">Dietary Nitrate, Nitric Oxide, and Cardiovascular Health.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Bondonno CP,
Croft KD,
Hodgson JM</span><br />
<span class="medgenPMjournal">Crit Rev Food Sci Nutr</span>
2016 Sep 9;56(12):2036-52.
doi: 10.1080/10408398.2013.811212.
<span class="bold">PMID: </span><a href="/pubmed/25976309" target="_blank">25976309</a></div>
<div class="nl"><a target="_blank" href="/pubmed/20142303">CNS toxicity involving methylene blue: the exemplar for understanding and predicting drug interactions that precipitate serotonin toxicity.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Gillman PK</span><br />
<span class="medgenPMjournal">J Psychopharmacol</span>
2011 Mar;25(3):429-36.
Epub 2010 Feb 8
doi: 10.1177/0269881109359098.
<span class="bold">PMID: </span><a href="/pubmed/20142303" target="_blank">20142303</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Methemoglobinemia%22%20AND%20Clinical%20prediction%20guides%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (230)</a></div></div>
</div>
<div class="portlet mgSection" id="ID_104">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Recent_systematic_reviews">Recent systematic reviews</h1><a sid="104" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln">
<div class="nl"><a target="_blank" href="/pubmed/37468669">Rasburicase-induced hemolytic anemia and methemoglobinemia: a systematic review of current reports.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Hammami MB,
Qasim A,
Thakur R,
Vegivinti CTR,
Patton CD,
Vikash S,
Kumar A</span><br />
<span class="medgenPMjournal">Ann Hematol</span>
2024 Sep;103(9):3399-3411.
Epub 2023 Jul 19
doi: 10.1007/s00277-023-05364-6.
<span class="bold">PMID: </span><a href="/pubmed/37468669" target="_blank">37468669</a></div>
<div class="nl"><a target="_blank" href="/pubmed/35397145">Food-induced methemoglobinemia: A systematic review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">McNulty R,
Kuchi N,
Xu E,
Gunja N</span><br />
<span class="medgenPMjournal">J Food Sci</span>
2022 Apr;87(4):1423-1448.
Epub 2022 Mar 22
doi: 10.1111/1750-3841.16090.
<span class="bold">PMID: </span><a href="/pubmed/35397145" target="_blank">35397145</a></div>
<div class="nl"><a target="_blank" href="/pubmed/33138742">Drinking-water nitrate and cancer risk: A systematic review and meta-analysis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Essien EE,
Said Abasse K,
Côté A,
Mohamed KS,
Baig MMFA,
Habib M,
Naveed M,
Yu X,
Xie W,
Jinfang S,
Abbas M</span><br />
<span class="medgenPMjournal">Arch Environ Occup Health</span>
2022;77(1):51-67.
Epub 2020 Nov 3
doi: 10.1080/19338244.2020.1842313.
<span class="bold">PMID: </span><a href="/pubmed/33138742" target="_blank">33138742</a></div>
<div class="nl"><a target="_blank" href="/pubmed/32017306">Therapeutic whole blood exchange in the management of methaemoglobinemia: Case series and systematic review of literature.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Singh P,
Rakesh K,
Agarwal R,
Tripathi PP,
Dhooria S,
Sehgal IS,
Prasad KT,
Hans R,
Sharma R,
Sharma N,
Lad D,
Aggarwal AN,
Muthu V</span><br />
<span class="medgenPMjournal">Transfus Med</span>
2020 Jun;30(3):231-239.
Epub 2020 Feb 3
doi: 10.1111/tme.12666.
<span class="bold">PMID: </span><a href="/pubmed/32017306" target="_blank">32017306</a></div>
<div class="nl"><a target="_blank" href="/pubmed/21954509">Methemoglobinemia: a systematic review of the pathophysiology, detection, and treatment.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Ashurst J,
Wasson M</span><br />
<span class="medgenPMjournal">Del Med J</span>
2011 Jul;83(7):203-8.
<span class="bold">PMID: </span><a href="/pubmed/21954509" target="_blank">21954509</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Methemoglobinemia%22%20AND%20systematic%5Bsb%5D%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (15)</a></div></div>
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