sulfhemoglobinemia

{{Short description|Medical condition caused by the combination of iron in the hemoglobin with sulfide compounds}}

{{Infobox medical condition (new)

| name = Sulfhemoglobinemia

| synonyms =

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| field = Hematology

| symptoms = Cyanosis, urinary tract infection and chronic constipation

| complications = Hypoxemia, methemoglobinemia, and hypoxia

| onset =

| duration = 100-120 days (lifespan of red blood cells)

| types =

| causes = Sulfur medications such as phenacetin, metoclopramide, dapsone, phenzopyridine, and trimethoprim-sulfamethoxazole; hydrogen-sulfide-producing intestinal bacteria, such as Morganella morganii

| risks = Pulmonary arteriovenous malformation

| diagnosis =

| differential =

| prevention = Avoidance of sulfur-containing compounds including drugs

| treatment = Blood transfusions

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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.{{Cite journal|last1=Gharahbaghian|first1=Laleh|last2=Massoudian|first2=Bobby|last3=DiMassa|first3=Giancarlo|date=August 2009|title=Methemoglobinemia and Sulfhemoglobinemia in Two Pediatric Patients after Ingestion of Hydroxylamine Sulfate|journal=Western Journal of Emergency Medicine|volume=10|issue=3|pages=197–201|issn=1936-900X|pmc=2729224|pmid=19718385}}{{cite book |last=Curry |first=Steven |date=June 6, 2007 |chapter=14 |chapter-url=https://www.sciencedirect.com/science/article/pii/B9780721606934500190 |editor1-last=Shannon |editor1-first=Michael |editor2-last=Borron |editor2-first=Stephen |editor3-last=Burns |editor3-first=Michael |title=Haddad and Winchester's Clinical Management of Poisoning and Drug Overdose |url=https://www.sciencedirect.com/book/9780721606934/haddad-and-winchesters-clinical-management-of-poisoning-and-drug-overdose |edition=4 |publisher=Saunders |page=289 |doi=10.1016/B978-0-7216-0693-4.50019-0 |isbn=9780721606934 |archive-url=https://web.archive.org/web/20210728165806/https://www.sciencedirect.com/book/9780721606934/haddad-and-winchesters-clinical-management-of-poisoning-and-drug-overdose |archive-date=July 28, 2021 |access-date=July 29, 2021 |url-status=bot: unknown }} 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.{{citation needed|date=July 2020}}

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.{{cn|date=November 2021}}

It can also be caused by phenazopyridine.{{cite journal |vauthors=Gopalachar AS, Bowie VL, Bharadwaj P |s2cid=22812461 |title=Phenazopyridine-induced sulfhemoglobinemia |journal=Ann Pharmacother |volume=39 |issue=6 |pages=1128–30 |date=June 2005 |pmid=15886294 |doi=10.1345/aph.1E557 |url=http://www.theannals.com/cgi/pmidlookup?view=long&pmid=15886294 |archive-url=https://archive.today/20130419072011/http://www.theannals.com/cgi/pmidlookup?view=long&pmid=15886294 |url-status=dead |archive-date=2013-04-19 }}

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.{{cn|date=November 2021}}

References

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