TY - CHAP M1 - Book, Section TI - Methemoglobinemia (Congenital) A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - Congenital presence of high levels of methemoglobin in blood. The presence of abnormally high levels of methemoglobin in the blood is the result of a congenital anomaly of one chain of hemoglobin (hemoglobins M) or congenital deficit in NADH cytochrome b5 reductase. Clinically, patients with a blood level around 15% present shortness of breath, cyanosis, mental status changes in 50% of patients, headache, fatigue, exercise intolerance, dizziness, and loss of hairlines. Patients with severe methemoglobinemia (>50%) have seizures, coma, and death (>70%). Healthy people may not have many symptoms with methemoglobin levels below 15%. However, patients with comorbidities, such as anemia, cardiovascular disease, lung disease, sepsis, or presence of other abnormal hemoglobin species (eg, carboxyhemoglobin, sulfhemoglobin, or sickle hemoglobin), may experience moderate-to-severe symptoms with levels as low as 5 to 8%. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/04/19 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1164078566 ER -