Exanguinotransfusión como tratamiento de metahemoglobinemia secundaria a dapsona. reporte de caso / Exchange transfusion to treat dapsone-induced methemoglobinemia. a case report

La dapsona es una sulfona utilizada en una variedad de enfermedades dermatológicas que incluye el pénfigo, lametahemoglobinemia es un síndrome clínico potencialmente mortal debido a la disminución del aporte de oxígenodesde la sangre hacia los tejidos; la metahemoglobinemia adquirida es la forma más frecuente y es un conocidoefecto adverso del uso de dapsona. Describimos el caso de un varón con pénfigo foliáceo y tratamiento reciente condapsona, que presentó cianosis, disnea y choque circulatorio ante concentraciones elevadas de metahemoglobina(21%); el tratamiento inicial incluyó oxigenoterapia, soporte ventilatorio invasivo, hemodinámico y administraciónde azul de metileno intravenoso, ante la ausencia de respuesta clínica se realizó exanguinotransfusión como terapiade segunda línea con respuesta óptima, el nivel sérico de metahemoglobina fue 2,3% tres horas posteriores alrecambio sanguíneo.

Dapsone is a sulphone used for a variety of dermatologic conditions including pemphigus. Methemoglobinemia isa potentially lethal syndrome due to a diminished supply of oxygen from blood to tissues, the acquired form is themost frequent presentation and it is a recognized adverse effect of dapsone. We report the case of a male patientwith pemphigus treated with dapsone that developed cyanosis, dyspnea and shock in the presence of elevatedconcentrations of methemoglobin (21%). Initial treatment included oxygen, invasive ventilatory support andintravenous administration of methylene blue, but due to lack of response, exchange transfusion was performedwith optimal response, serum level of methemoglobin after the procedure was 2.3%.

[Case report: hereditary methemoglobinemia during general anesthesia, a diagnostic and therapeutic review]

The case report that will be presented is an inherited methemoglobinemia, during general anesthesia of 36year old patient and was suspected by some clinical examination such as decreased oxygen saturation in pulse oximetry and a normal finding in ABG sample that has been prepared at the preparation of patient for anesthesia. We are going to write about this problem in preoperative period and how to manage it

Thinner intoxication manifesting as methemoglobinemia.

Lacquer thinner, commonly used for removing household paints is known to contain mixture of various arometic hydrocarbons; halogenated hydrocarbons and naptha, if ingested may rarely cause methemoglobinemia. We report a 3 year old child who presented to us with history of accidental ingestion of thinner, used for removing household paint. Child was having cyanosis with minimal tachypnoea & the colour of his blood was chocolate brown. Later methemoglibulin level estimations were also done, which were suggestive of this diagnosis. Child was managed conservatively with oxygen therapy & vital monitoring, which lead to complete recovery and discharge from hospital later.

Metemoglobinemia: do diagnóstico ao tratamento / Metahemoglobinemia: del diagnóstico al tratamiento / Methemoglobinemia: from diagnosis to treatment

JUSTIFICATIVAS E OBJETIVOS: A metemoglobina é a forma oxidada da hemoglobina, que além de não se ligar ao oxigênio, aumenta a afinidade deste pela porção parcialmente oxidada da hemoglobina. A concentração aumentada da metemoglobina no sangue decorre de alterações congênitas e de exposição a agentes químicos diversos, resultando em quadro com múltiplos diagnósticos diferenciais, que se não tratado pode levar ao óbito. Foi feita revisão sobre o assunto, dando ênfase às informações relevantes para o manuseio clínico dos pacientes. CONTEÚDO: Quando a concentração sangüínea de metemoglobina está acima de 1,5 por cento surge a cianose, característica principal da doença. Os pacientes apresentam sangue arterial de coloração marrom-escuro com a PaO2 normal. O diagnóstico deve ser suspeitado em pacientes que apresentem cianose e baixa leitura de saturação ao oxímetro de pulso (SpO2), sem que haja comprometimento cardiopulmonar significativo. A co-oximetria é o método padrão-ouro e define o diagnóstico. No tratamento dos pacientes devem ser considerados o caráter agudo ou crônico da síndrome (etiologia) e a gravidade dos sintomas. A concentração sangüínea de metemoglobina é importante, sobretudo nos casos agudos. O tratamento básico consiste na remoção do agente causador, administração de oxigênio e observação. Casos graves devem ser tratados com azul-de-metileno, antídoto específico, porém ineficaz em algumas situações. CONCLUSÃO: A metemoglobinemia é condição potencialmente grave, cujo diagnóstico depende do alto grau de suspeição. Em geral, os anestesiologistas, no período perioperatório, são os primeiros a detectarem o problema e devem liderar a condução do tratamento.

BACKGROUND AND OBJECTIVES: Methemoglobin is the oxidized form of hemoglobin, which does not bind oxygen and increases the affinity of oxygen for the partially oxidized portion of hemoglobin. Increased levels of methemoglobin in the blood are secondary to congenital changes and exposure to several chemical agents, resulting in a disorder with several differential diagnoses, which it can lead to death if it is not treated. The objective of this report was to review this subject, emphasizing relevant information for the clinical management of patients with methemoglobinemia. CONTENTS: When the concentration of methemoglobin in the blood is above 1.5 percent, the patient develops cyanosis, the main characteristic of this disorder. The color of the arterial blood changes to dark brown with normal PaO2. One should suspect the diagnosis in patients with cyanosis and low saturation (SpO2) without significant cardiopulmonary dysfunction. Co-oximetry is the gold standard and defines the diagnosis. Treatment should be based on whether the syndrome is acute or chronic (etiology) and on the severity of symptoms. Blood levels of methemoglobin are important, especially in acute cases. Basic treatment includes removal of the agent responsible for the disorder, administration of oxygen, and observation. Severe cases should be treated with the specific antidote, methylene blue, which is not effective in some situations. CONCLUSIONS: Methemoglobinemia is a potentially severe disorder, whose diagnosis depends on a high degree of suspicion. In general, anesthesiologists are the first to detect the problem in the preoperative period and should lead the treatment.

JUSTIFICATIVAS Y OBJETIVOS: La metahemoglobina es la forma oxidada de la hemoglobina, que, además de no vincularse con el oxígeno, aumenta su afinidad por la porción parcialmente oxidada de la hemoglobina. La concentración aumentada de la metahemoglobina en la sangre, proviene de las alteraciones congénitas y de la exposición a agentes químicos diversos, trayendo como resultado, un cuadro con múltiples diagnósticos diferenciales, que si no se trata, puede conllevar al deceso. Se hizo una revisión sobre el asunto, dándole énfasis a las informaciones relevantes para el manejo clínico de los pacientes. CONTENIDO: Cuando la concentración sanguínea de metahemoglobina está por encima de 1,5 por ciento surge la cianosis, característica principal de la enfermedad. Los pacientes presentan sangre arterial de coloración marrón oscuro con la PaO2 normal. El diagnóstico debe suponerse en pacientes que presenten cianosis y una baja lectura de saturación al oxímetro de pulso (SpO2), sin que exista un comprometimiento cardiopulmonar significativo. La co-oximetría es el método estándar de oro y define el diagnóstico. En el tratamiento de los pacientes, deben ser considerados el carácter agudo o crónico del síndrome (etiología) y la gravedad de los síntomas. La concentración sanguínea de metahemoglobina es importante principalmente en los casos agudos. El tratamiento básico consiste en la retirada del agente causador, administración de oxígeno y observación. Casos graves deben ser tratados con azul de metileno, antídoto específico, sin embargo ineficaz en algunas situaciones. CONCLUSIÓN: La Metahemoglobinemia es una condición potencialmente grave, cuyo diagnóstico depende del alto grado de sospecha. En general, los anestesiólogos, en el período perioperatorio, son los primeiros que detectan el problema y deben liderar el tratamiento.

Metahemoglobinemia infantil causada por nitratos / Nitrate-induced methemoglobinemia in children

The intake of water or food with high levels of nitrates produces methemoglobinemia in infants up to 3 months of age. The study reviews factors in the environment and in the host that account for the increased risk in this age group. In order to reduce the prevalence of this problem, health authorities should control nitrate concentrations in drinking water, create adequate water supply systems, implement educational programs aimed at informing parents about preventive measures, and call on physicians to report cases of methemoglobinemia