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Sulfhemoglobinemia

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Medical condition caused by the combination of iron in the hemoglobin with sulfide compounds Medical condition
Sulfhemoglobinemia
SpecialtyHematology
SymptomsCyanosis, urinary tract infection and chronic constipation
ComplicationsHypoxemia, methemoglobinemia, and hypoxia
Duration100-120 days (lifespan of red blood cells)
CausesSulfur medications such as phenacetin, metoclopramide, dapsone, phenzopyridine, and trimethoprim-sulfamethoxazole; hydrogen-sulfide-producing intestinal bacteria, such as Morganella morganii
Risk factorsPulmonary arteriovenous malformation
PreventionAvoidance of sulfur-containing compounds including drugs
TreatmentBlood transfusions

Sulfhemoglobinemia is a rare condition in which there is excess sulfhemoglobin (SulfHb) in the blood. The pigment is a greenish derivative of hemoglobin which cannot be converted back to normal, functional hemoglobin. It causes cyanosis even at low blood levels.

It is a rare blood condition in which the β-pyrrole ring of the hemoglobin molecule has the ability to bind irreversibly to any substance containing a sulfur atom. When hydrogen sulfide (H2S) (or sulfide ions) and ferrous ions combine in the heme of hemoglobin, the blood is thus incapable of transporting oxygen to the tissues.

Presentation

Symptoms include a blueish or greenish coloration of the blood (cyanosis), skin, and mucous membranes, even though a blood count test may not show any abnormalities in the blood. This discoloration is caused by greater than 5 grams per cent of deoxyhemoglobin, or 1.5 grams per cent of methemoglobin, or 0.5 grams per cent of sulfhemoglobin, all serious medical abnormalities.

Causes

Sulfhemoglobinemia is usually drug induced, with drugs associated with it including sulphonamides, such as sulfasalazine or sumatriptan. Another possible cause is occupational exposure to sulfur compounds.

It can also be caused by phenazopyridine.

Diagnosis

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Treatment

The condition generally resolves itself with erythrocyte (red blood cell) turnover, although blood transfusions can be necessary in extreme cases.

References

  1. Gharahbaghian, Laleh; Massoudian, Bobby; DiMassa, Giancarlo (August 2009). "Methemoglobinemia and Sulfhemoglobinemia in Two Pediatric Patients after Ingestion of Hydroxylamine Sulfate". Western Journal of Emergency Medicine. 10 (3): 197–201. ISSN 1936-900X. PMC 2729224. PMID 19718385.
  2. Curry, Steven (June 6, 2007). "14". In Shannon, Michael; Borron, Stephen; Burns, Michael (eds.). Haddad and Winchester's Clinical Management of Poisoning and Drug Overdose (4 ed.). Saunders. p. 289. doi:10.1016/B978-0-7216-0693-4.50019-0. ISBN 9780721606934. Archived from the original on July 28, 2021. Retrieved July 29, 2021.{{cite book}}: CS1 maint: bot: original URL status unknown (link)
  3. Gopalachar AS, Bowie VL, Bharadwaj P (June 2005). "Phenazopyridine-induced sulfhemoglobinemia". Ann Pharmacother. 39 (6): 1128–30. doi:10.1345/aph.1E557. PMID 15886294. S2CID 22812461. Archived from the original on 2013-04-19.

External links

ClassificationD
External resources
Diseases of red blood cells
Polycythemia
Anemia
Nutritional
Hemolytic
(mostly normo-)
Hereditary
Acquired
AIHA
Aplastic
(mostly normo-)
  • Hereditary: Fanconi anemia
  • Diamond–Blackfan anemia
  • Blood tests
    Other
    Proteins that contain heme (hemoproteins)
    Globins
    Hemoglobin
    Subunits
    Alpha locus on 16:
    Beta locus on 11:
    Tetramers
    stages of
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    Embryonic
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    pathology:
    Compounds
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    see also disorders of globin and globulin proteins
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