Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Methemoglobinemia is a rare but easily diagnosed disease which may resemble cyanotic congenital heart disease. Toxic agents, mainly nitrate absorption, are often responsible and may reveal an underlying permanent or transient enzyme deficiency. Methemoglobinemia is of poor prognosis if secondary to hemoglobin disorders or congenital enzyme deficiency. Methylene blue is a good aid to diagnosis and a treatment of choice.
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PMID:[Non-cardiac cyanosis: methemoglobinemia in infants]. 164 40

Three patients with significant unilateral, gross hematuria owing to sickle cell hemoglobinopathy were treated successfully with retrograde instillation of 1 per cent silver nitrate. There have been no recurrences of gross hematuria during 11 to 15 months of followup. With standard endoscopic evaluation, verification of the etiology of hematuria from sickle hemoglobinopathy and effective treatment can be accomplished quickly and safely.
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PMID:Silver nitrate irrigation for hematuria from sickle cell hemoglobinopathy. 358 54

Intraoperative cyanosis is an utmost emergency for anesthesiologist. If the patient has adequate control ventilation, and normal cardiac pulmonary physiology, then methemoglobinemia must be considered. Reported here is a normal female with dark color lip on the second day after her second parturition and was undergoing tubal ligation. Twenty minutes after induction of general anesthesia and endotracheal intubation, dark blood at the incision site was noted by the operator. After emergent check up of the anesthesia machine, tubings, breathing sound and arterial blood gas, there was only one suspicion left. Methemoglobinemia was confirmed by the hematological examination. Methemoglobinemia is a product from the oxidation of the iron atom in the heme ring when oxygen dissociates from it. This process exists in nature, but can also be induced by nitrate or nitrite-containing drugs or foods or benzene-like organic compounds. Methemoglobinemia can be differentiated from normal hemoglobin by mass spectrometry. If acute illness develops, patients should be treated with methylene blue. Otherwise ascorbic acid will do. This case is reported to remind all anesthesia personnel about one of the rare but serious hemoglobinopathy.
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PMID:[Another reason for cyanosis--methemoglobinemia]. 803 75