Pediatric Methemoglobinemia Clinical Presentation

  • Author: Michael J Verive, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Nov 14, 2011
 

History

  • Congenital methemoglobinemia: The characteristic history is diffuse persistent slate-gray cyanosis, often present from birth, without evidence of cardiopulmonary disease.
  • Acquired methemoglobinemia
    • Presentation may be dramatic, with cyanosis, dyspnea, lethargy, headache, dizziness, deterioration of mental functioning, or stupor.
    • History of exposure to a known toxin or drug may not always be available but should be sought because long-term or repeated exposure may occur.
    • Discussion with a toxicologist may be necessary, especially when methemoglobinemia occurs shortly after exposure to a new medication, because the list of medications known to cause methemoglobinemia constantly changes. A comprehensive review of all medications, herbs, and other nutritional supplements may disclose exposure to a toxin not previously known to cause hemoglobinemia.
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Physical

  • Congenital methemoglobinemia
    • These patients are described as being more blue than sick.
    • Patients appear cyanotic with a diffuse slate-gray appearance.
    • Cyanosis is easily observed on the nose, cheeks, fingers, toes, and in the mucous membranes, including the fundi, and may go unrecognized for a long time in patients with more heavily pigmented skin or in patients with moderate-to-severe anemia. Clubbing is absent.
    • Methemoglobin levels of 10-20% are tolerated with no clinical symptoms, whereas levels of 30-40% may be associated with headaches and dyspnea, especially upon exertion.
  • Patients with hemoglobin M disease with the alpha chain variant can present at birth with cyanosis, whereas patients with the beta chain variants present in the later half of infancy.
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Causes

  • Acquired methemoglobinemia: Exposure to various drugs or toxins may result in acquired methemoglobinemia. These include the following:
    • Nitrites, particularly in well water (Prepackaged foods [including baby food] may contain significant levels of nitrites.)[6, 7]
    • Aniline dyes
    • Silver nitrate
    • Nitroprusside
    • Antimalarials
    • Zopiclone[8]
    • Local anesthetics (eg, benzocaine, prilocaine, lidocaine), particularly when applied to mucosa, such as during bronchoscopy, or after repeated cutaneous exposure to eutectic mixture of lidocaine-prilocaine (EMLA cream) over a short period
    • Nitric and nitrous oxides
    • Dapsone,[9] rasburicase,[10] and phenazopyridine
    • Inadequately cooked vegetables (eg, spinach, beets, carrots) contaminated with bacteria (Infants and patients on gastric acid-reduction therapy are particularly prone to developing methemoglobinemia because gastric acid production may not be sufficient to maintain low levels of nitrate-reducing bacteria in the intestine.)
    • Fava bean ingestion in patients with G-6-PD deficiency[11]
  • Hereditary methemoglobinemia: This may be due to the deficiency of nicotinamide adenine dinucleotide (NADH) cytochrome b5 reductase or NADPH-flavin reductase or the presence of hemoglobin M.
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Contributor Information and Disclosures
Author

Michael J Verive, MD  Medical Director, Pediatric Intensive Care, Department of Pediatrics, St Mary's Hospital for Women and Children

Michael J Verive, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, Pediatric Sedation, and Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Mudra Kumar, MD, MBBS, MRCP  Associate Professor, Department of Pediatrics, University of South Florida College of Medicine

Mudra Kumar, MD, MBBS, MRCP is a member of the following medical societies: American Academy of Pediatrics and American Society of Hematology

Disclosure: Nothing to disclose.

Specialty Editor Board

Sharada A Sarnaik, MBBS  Professor of Pediatrics, Wayne State University School of Medicine; Director, Sickle Cell Center, Attending Hematologist/Oncologist, Children's Hospital of Michigan

Sharada A Sarnaik, MBBS is a member of the following medical societies: American Association of Blood Banks, American Association of University Professors, American Society of Hematology, American Society of Pediatric Hematology/Oncology, New York Academy of Sciences, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Steven K Bergstrom, MD  Department of Pediatrics, Division of Hematology-Oncology, Kaiser Permanente Medical Center of Oakland

Steven K Bergstrom, MD is a member of the following medical societies: Alpha Omega Alpha, American Society of Clinical Oncology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and International Society for Experimental Hematology

Disclosure: Nothing to disclose.

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA  Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Mudra Kumar, MD, MBBS, MRCP, to the original writing and development of this article.

References

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Note the chocolate brown color of methemoglobinemia. Tube 1 and tube 2 have a methemoglobin concentration of 70%; tube 3, a concentration of 20%; and tube 4, a normal concentration.
 
 
 
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