Metahemoglobinemia y hemólisis intravascular aguda después de una intoxicación por naftaleno en un paciente pediátrico / Methemoglobinemia and acute intravascular hemolysis after naphthalene poisoning in a pediatric patient

La intoxicación por naftaleno es poco frecuente en los niños. Es causada por la ingesta, la inhalación o el contacto con la piel de sustancias que contienen naftaleno. Los pacientes suelen tener orina de color marrón oscuro, diarrea acuosa y vómito bilioso. Los signos incluyen fiebre, taquicardia, hipotensión y valores bajos en la oximetría de pulso, incluso con oxigenoterapia. Los análisis de sangre detectan anemia hemolítica, metahemoglobinemia, insuficiencia renal e hiperbilirrubinemia. Además del tratamiento sintomático, se hacen transfusiones de eritrocitos y se les administran ácido ascórbico, azul de metileno y N-acetilcisteína. En este artículo, presentamos el caso de un paciente masculino de 23 meses de edad con metahemoglobinemia y hemólisis intravascular aguda que recibió atención en la unidad de cuidados intensivos durante cinco días por intoxicación por naftaleno. Si bien la intoxicación por naftaleno es muy poco frecuente, tiene consecuencias mortales y se debe ejercer precaución con su uso y venta.

Poisoning by naphthalene is uncommon in children. It is a type of poisoning brought on by ingesting, inhaling, or coming into touch with naphthalene-containing substances on the skin. Patients typically present with an initial onset of dark brown urine, watery diarrhea, and bile vomit. The signs include fever, tachycardia, hypotension, and low pulse oximetry readings even with oxygen support. Hemolytic anemia, methemoglobinemia, renal failure, and hyperbilirubinemia are all detected in blood tests. Erythrocyte transfusion, ascorbic acid, methylene blue, and N-acetylcysteine (NAC) therapies are provided to inpatients in addition to symptomatic treatment. We present a 23-month-old male patient who developed methemoglobinemia and acute intravascular hemolysis, who was followed up in the intensive care unit for five days due to naphthalene intoxication. Although naphthalene poisoning is very rare, it should be known that it has fatal consequences, and more care should be taken in its use and sale.

Sulfohemoglobinemia secundaria a zopiclona. Casos clínicos / Sulfhemoglobinemia secondary to the use of zopiclone. Report of two cases

Sulfhemoglobin (SulfHb) is formed by hemoglobin (Hb) oxidation by sulfur compounds. Sulfhemoglobinemia is mainly associated with drugs or intestinal bacterial overgrowth. Patients present with central cyanosis, an abnormal pulse oximetry and normal arterial oxygen partial pressure. These features are shared with methemoglobinemia (MetHb) whose diagnosis requires an arterial co-oximetry. Depending on the device used, SulfHb may produce interference with this technique. We report two females aged 31 and 43 years, consulting at the emergency room with cyanosis. Both had a history of acute and chronic, high dose zopiclone ingestion. Pulse oximetry showed desaturation but with normal arterial oxygen partial pressure. Cardiac and pulmonary diseases were ruled out. Co-oximetry in two different analyzers showed interference or normal MetHb percentages. No other complications ensued, and cyanosis decreased over days. Since MetHb was discarded among other causes of cyanosis in a compatible clinical context, the diagnosis of sulfhemoglobinemia was made. The confirmatory method is not available in Chile. The presence of SulfHb is difficult to diagnose, confirmatory tests are not readily available, and it frequently interferes with arterial co-oximetry. This is attributed to a similar absorbance peak of both pigments in arterial blood. Venous co-oximetry can be useful in this context. SulfHb is a self-limited condition in most cases, however it must be differentiated from methemoglobinemia to avoid inappropriate treatments like methylene blue.

Metahemoglobinemia, una entidad de diagnóstico complejo. Reporte de un caso

Methemoglobinemia is a rare condition with serious consequences if not diagnosed. We report the case of a 64-year-old woman with a history of allergy to sulfa drugs and a recent diagnosis of a small vessel vasculitis (ANCA-p) who started induction therapy with corticosteroids and rituximab. Due to the need for infectious prophylaxis, and considering her history, dapsone was administered instead of cotrimoxazole after ruling out glucose-6-phosphate dehydrogenase deficiency. During the admission to the hospital for her second dose of rituximab, and while being asymptomatic, she persistently presented a pulse oximetry ≪ 90% despite the administration of O2. Therefore, the infusion was postponed to study the patient. The arterial gasometric study by direct potentiometry revealed an O2 saturation of 98%, with a saturation gap > 5%. Considering the use of dapsone, a methemoglobinemia was suspected and confirmed by co-oximetry (methemoglobinemia 9%). Dapsone was suspended and one week later, her methemoglobinemia was absent.

Cianosis en un paciente de 14 años: Presentación de un caso de metahemoglobinemia / Cyanosis in 14-year-old patient: Methemoglobinemia: case report

La cianosis es la coloración azulada de la piel y las mucosas debida al aumento de la concentración de hemoglobina reducida en los capilares o a la presencia de metahemoglobina en concentraciones mayores de las normales. Es importante pensar en metahemoglobinemia como diagnóstico diferencial frente a un paciente con cianosis que no responde a la administración de oxígeno cuando no existen causas cardiorrespiratorias que la justifiquen, dado que requiere de otros métodos diagnósticos y de tratamiento específico. Se presenta el caso de un paciente adolescente de 14 años de edad con cianosis secundaria a metahemoglobinemia de probable etiología congénita. Se discuten las causas, forma de presentación, diagnóstico y tratamiento.

The bluish coloration of skin and mucous membranes, called as cyanosis, could be explained by high reduced hemoglobin in the capillaries, or the presence of elevated methemoglobin concentration. It is important to think of methemoglobinemia as a differential diagnosis in a cyanotic patient who does not respond to oxygen administration once cardiorespiratory causes are discarded; since it requires other diagnostic methods and specific treatment. We described a case of cyanosis in a fourteen-year-old adolescent with probable congenital methemoglobinemia. We discussed their probable causes, clinic presentation, diagnosis and treatment.

Metahemoglobinemia relacionada con ingesta de puré de acelgas / Methemoglobinemia due to ingestion of chard puree

La metahemoglobinemia es un síndrome clínico dado por la presencia de una forma aberrante de hemoglobina, ocasionada por diversos agentes oxidantes. Se describe un caso clínico de metahemoglobinemia severa asociada a la ingesta de puré de acelgas con alto contenido en nitratos y nitritos. Paciente de un año, con antecedentes de comunicación interauricular (CIA), que presentó un cambio en coloración de la piel 7 h antes, en forma progresiva, acompañado de vómitos. Ingresó al Departamento de Emergencia con cianosis generalizada que no mejoró con oxigenoterapia, taquicardia y tendencia a hipotensión arterial. En cuidados intensivos se realizó ecocardiograma que evidenció CIA sin repercusión hemodinámica. Metahemoglobinemia 37%. Se realizó dosis de azul de metileno al 1% por vía intravenosa, con franca mejoría clínica a la hora de la administración del antídoto y descenso de niveles de metahemoglobina. Alta médica a las 36 horas del ingreso. Existía una relación cronológica entre la exposición a nitratos por ingesta de un puré de acelgas y la aparición del cuadro. Los niveles de nitratos hallados en dicho alimento fueron muy elevados considerando estándares internacionales, lo que sumado a una inadecuada conservación del alimento cocido los días previos, permitió confirmar el planteo etiológico realizado. Resulta importante sospechar esta entidad patológica poco frecuente frente a cianosis que no mejora con oxígeno, y prevenir cuadros similares al descrito mediante una adecuada manipulación y conservación de las verduras con alto contenido en nitratos.

Methemoglobinemia is a clinical syndrome due to the presence of an aberrant form of hemoglobin, caused by various oxidizing agents. The study reports a case of severe methemoglobinemia associated with the ingestion of chard puree with high levels of nitrates and nitrites. A 1-year-old patient with a history of atrial septal defect (ASD), who progressively showed change of skin color 7 hours earlier, accompanied by vomiting. She was admitted to the Emergency Department with generalized cyanosis not improving with oxygen therapy, tachycardia and tendency to hypotension. In the intensive care unit, an echocardiogram showed ASD without hemodynamic complications. Methemoglobinemia 37%. A 1% methylene blue dose was administered intravenously, with clinical improvement one hour after antidote administration and decrease in methemoglobin levels. Medical discharge at 36 hours of admission. There was a chronological relationship between nitrates exposure by ingestion of chard puree and the clinical onset of methemoglobinemia. The toxic cause was confirmed after high nitrates levels were found in this vegetable considering international standards, and an inadequate preservation of the cooked chard on previous days. It is important to suspect this rare pathological entity when cyanosis fails to improve with oxygen, and to prevent poisonings similar to those described by an adequate manipulation and preservation of vegetables with high nitrate levels.

Methemoglobinemia and dapsone levels in patients with leprosy

The objective of this work was to determine the methemoglobinemia and correlate with dapsone levels in multibacillary leprosy patients under leprosy multi-drug therapy. Thirty patients with laboratory and clinical diagnosis of multibacillary leprosy were enrolled. Dapsone was analyzed by high performance liquid chromatography and methemoglobinemia by spectrophotometry. The mean dapsone concentrations in male was 1.42 g/mL and in female was 2.42 g/mL. The mean methemoglobin levels in male was 3.09 µg/mL; 191 percent, and in female was 2.84 ± 1.67 percent. No correlations were seen between dapsone levels and methemoglobin in male and female patients. Our results demonstrated that the dosage of dapsone in leprosy treatment does not promote a significant methemoglobinemia.

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.

Acute methemoglobinemia induced by dapsone / Metahemoglobinemia aguda induzida por dapsona: relato de caso no tratamento para hanseníase

Acute methemoglobinemia is an uncommon but potentially treatable disorder in witch patient can present with dramatic signs and symptoms. The early diagnostic and treatment are essentials to prevent this fatal medicine reaction.

Transient methemoglobinemia in an infant.

We report a case of transient methemoglobinemia in an infant due to gastroenteritis. Methemoglobinemia should be suspected in infants with a history of diarrhea and cyanosis that is out of proportion to the history and clinical examination. Methemoglobinemia can be life threatening, but outcome is good when treated with IV methylene blue.

Associação de metemoglobinemia e deficiência de glicose-6-fosfato desidrogenase em pacientes com malária tratados com primaquina / Association of methemoglobinemia and glucose-6-phosphate dehydrogenase deficiency in malaria patients treated with primaquine

Este estudo teve como objetivo verificar a ocorrência de metemoglobinemia em indivíduos deficientes da glicose-6-fosfato desidrogenase durante o tratamento da infecção malárica com primaquina. Foram selecionados pacientes com diagnóstico para malária por Plasmodium vivax ou mista V+F (Plasmodium vivax + Plasmodium falciparum), Grupo 1: com 74 indivíduos com diagnóstico clínico de metemoglobinemia e Grupo 2: 161 indivíduos sem diagnóstico clínico de metemoglobinemia. Quanto à deficiência da G6PD, nos Grupos 1 e 2, houveram 51,3 por cento (38) e 8,7 por cento (14) de indivíduos enzimopênicos, respectivamente, demonstrando através de tais dados, significância estatística na associação com a metemoglobinemia somente nos indivíduos do Grupo 1 (p<0,05). A comparação da relação da metemoglobinemia à deficiência da G6PD mostrou haver uma possível associação de indivíduos enzimopênicos desenvolverem metemoglobinemia com maior freqüência.

This study had the aim of investigating occurrences of methemoglobinemia among individuals with glucose-6-phosphate dehydrogenase deficiency during treatment for malaria infection using primaquine. Patients with a diagnosis of malaria caused by Plasmodium vivax or the V+F mixture (Plasmodium vivax + Plasmodium falciparum) were selected. Group 1 consisted of 74 individuals with a clinical diagnosis of methemoglobinemia and Group 2 consisted of 161 individuals without a clinical diagnosis of methemoglobinemia. The glucose-6-phosphate dehydrogenase deficiency rates (numbers of enzymopenic individuals) in Groups 1 and 2 were 51.3 percent (38) and 8.7 percent (14) respectively. These data demonstrated a statistically significant association with methemoglobinemia only among the individuals in Group 1 (p<0.05). Investigation of the relationship between methemoglobinemia and glucose-6-phosphate dehydrogenase deficiency showed that there was a possible association such that enzymopenic individuals may develop methemoglobinemia more frequently.

A Thai boy with hereditary enzymopenic methemoglobinemia type II.

Individuals with methemoglobin exceeding 1.5 g/dl have clinically obvious central cyanosis. Hereditary methemoglobinemia is due either to autosomal dominant M hemoglobins or to autosomal recessive enzymopenic methemoglobinemia. Four types of enzymopenic methemoglobinemia have been described. In addition to methemoglobinemia, individuals with type II, which is the generalized cytochrome b5 reductase deficiency, have severe and progressive neurological disabilities. Here we report a 3-year-old Thai boy with type II hereditary enzymopenic methemoglobinemia. He was born to a second-cousin couple. His central cyanosis was first observed around 10 months of age. His neurological abnormalities were seizures beginning at 1 year of age, microcephaly, and inability to hold his head up. His cardiovascular and pulmonary evaluations were unremarkable. Methemoglobin level by spectral absorption pattern was 18 per cent. A qualitative enzymatic assay confirmed the deficiency of the cytochrome b5 reductase enzyme. With this definite diagnosis, a prenatal diagnosis for the next child of this couple will be possible.

Diagnóstico presuntivo de metahemoglobinemia con infusión intravenosa continua de procaína al 1 por ciento: la importancia del gráfico de tendencias en anestesiología / Presumptive diagnosis of methemoglobinemia: the graphic of tendencies in anesthesiology

Se describe un caso donde se utiliza anestesia intravenosa total (midazolam-propofol-fentanilo-procaína) para la realización de una colecistectomía. Durante el intraoperatorio se detecta saturación de la oxihemoglobina coincidentemente con la infusión de una solución de procaína al 1 por ciento como complemento analgésico. Se analizan las causas que provocan la caída de la saturación y la fisiopatología de la metahemoglobinemia. La detección del fenómeno fue posible gracias a la visualización gráfica de la caída constante de la saturación de la hemoglobina en porcentajes aislados no significativos. La asociación de procaína con desaturación acompañada o no de cianosis debe hacer pensar en una posible metahemoglobinemia. Se destaca el valor del gráfico de tendencias en el monitoreo anestésico y su importancia para la detección precoz de problemas intraoperatorios.

Metahemoglobinemia adquirida por intoxicación con dapsona: presentación de un caso / Acquired metahemglobinemia by dapsone intoxication

Presentamos un caso de Metahemoglobinemia adquirida por la ingesta de 40 comprimidos de Dapsona, con intención suicida, produciéndole a la paciente una cianosis periférica marcada y deterioro del sensorio; se realizó tratamiento con Azul de Metileno y Acido Ascórbico, registrándose cifras posterapéuticas de Metahemoglobinemia de 19 por ciento (valores normales hasta 2 por ciento). Teniendo buena respuesta clínica, con evolución favorable, sin secuelas posteriores