Methylene blue in congenital methemoglobinemia: prophylactic or on demand?

Sharma, Deepak Kumar; Pandia, Mihir Prakash; Bithal, Parmod K.; Baraka, Anis; Ayoub, Chakib M.; Yazbeck‐Karam, Vanda G.; Kaddoum, Roland; Gerges, Frederic J.; Hadi, Usamah; Dagher, Carla M.

doi:10.1007/bf03021788

Cited by 7 publications

(6 citation statements)

References 3 publications

(1 reference statement)

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“…The prophylactic use of MB in people with hereditary cytochrome b5 reductase deficiency and methemoglobin concentrations of >20% is recommended before induction of anesthesia . However, prophylactic use of MB in the aforementioned group of people with methemoglobin concentrations of <20% remains controversial . The argument against its use is founded on the ability to carefully monitor people perioperatively and postoperatively using multiple wavelength co‐oximeters or co‐oximeter blood gas analyzers.…”

Section: Discussionmentioning

confidence: 99%

“…11 However, prophylactic use of MB in the aforementioned group of people with methemoglobin concentrations of <20% remains controversial. 12,13 The argument against its use is founded on the ability to carefully monitor people perioperatively and postoperatively using multiple wavelength co-oximeters or co-oximeter blood gas analyzers. These analyzers accurately detect oxyhemoglobin/methemoglobin saturation and methemoglobin concentration, respectively.…”

Section: Discussionmentioning

confidence: 99%

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Long‐term Treatment with Methylene Blue in a Dog with Hereditary Methemoglobinemia Caused by Cytochrome b5 Reductase Deficiency

Jaffey

Harmon

Villani

et al. 2017

Veterinary Internal Medicne

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A juvenile male mixed breed dog was presented for lethargy, exercise intolerance, and aggression when touched on the head. Cyanosis, tachycardia, and tachypnea were observed and persisted during oxygen supplementation. Arterial blood gas analysis by co‐oximetry identified an increased methemoglobin concentration (27%; normal, <2%) with normal arterial oxygen tension. The methemoglobinemia and associated clinical signs resolved after administration of methylene blue (1 mg/kg) IV, and the dog was discharged. The affected dog's whole‐genome sequence contained 2 potentially causal heterozygous CYB5R3 missense mutations suggesting that cytochrome b5 reductase deficiency was responsible for the methemoglobinemia. This hypothesis was confirmed by enzyme analysis that identified cytochrome b5 reductase activity in the affected dog's erythrocytes to only approximately 6% of that in a control sample. Clinical signs recurred 11 days after discharge but normalized and the methemoglobin concentration decreased with methylene blue administration PO (1.5 mg/kg, initially daily and then every other day).

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Long‐term Treatment with Methylene Blue in a Dog with Hereditary Methemoglobinemia Caused by Cytochrome b5 Reductase Deficiency

Jaffey

Harmon

Villani

et al. 2017

Veterinary Internal Medicne

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“…7 Although, a methemoglobin level of 18% may not require any extra intervention in an otherwise asymptomatic patient going for a less invasive procedure, our patient was undergoing a major cardiac procedure with added risk of bleeding, CPB, aortic cross-clamp, and cardioplegic arrest. In determining our management plan, we considered 4 options: (a) postpone the surgery to a later date and optimize the methemoglobinemia in consultation with the hematology service, (b) proceed without any change in the management as oxygen extraction appeared normal, (c) administer methylene blue and proceed, 8,9 and (d) perform an exchange transfusion before or during the CPB. We preferred not to postpone the surgery as we believed that it would not add any benefit to the management of the patient.…”

Section: Discussionmentioning

confidence: 99%

Intraoperative Management of a Child Undergoing Cardiac Surgery With Congenital Methemoglobinemia Detected in the Operating Room: A Case Report

et al. 2021

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Congenital methemoglobinemia is a rare disease that is easily overlooked in its mild form. It can lead to tissue hypoxia as methemoglobin does not possess oxygen-carrying capacity. Its management approach depends on the severity of the symptoms, methemoglobin level, and associated medical conditions. The perioperative management of congenital methemoglobinemia is well described in the literature; however, its management in children with congenital heart disease and undergoing cardiac surgery using cardiopulmonary bypass has not been reported. We present a case and its management where congenital methemoglobinemia was detected in the operating room in a child scheduled for cardiac surgery. (A&A Practice. 2021;15:e01395.) GLOSSARY ABG = arterial blood gas; ASA = American Society of Anesthesiologists; CPB = cardiopulmonary bypass; Cyb5R = cytochrome b5 reductase; Fio 2 = fraction of inspired oxygen; G6PD = glucose-6-phosphate dehydrogenase; Hb = hemoglobin; ICU = intensive care unit; MetHb = methemoglobin; NICU = neonatal intensive care unit; RVOT = right ventricular outflow tract; Sao 2 = oxygen saturation; VBG = venous blood gas a Diagnosis of methemoglobinemia was made intraoperatively, and exchange transfusion was done on CPB.

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“…Da mesma forma, a exposição a substâncias oxidantes pode aumentar níveis de MetHb e agudizar os sintomas 8, 12,32 . Portanto, a decisão de se tratar ou não o paciente deve ser individualizada e orientada sobretudo de acordo com o contexto clínico 41,42 . O azul-de-metileno é uma tiazina com propriedade anti-sép-tica e oxidante dose-dependente 5 .…”

Section: Tratamentounclassified

“…Similarly, exposure to oxidizing drugs can increase the levels of MetHb, leading to the development of acute symptoms 8,12,32 . Therefore, the decision to treat or not should be individualized and oriented according to the clinical presentation 41,42 . Methylene blue is a thiazine dye with dose-dependent antiseptic and oxidizing properties 5 .…”

Section: Treatmentmentioning

confidence: 99%

Metemoglobinemia: do diagnóstico ao tratamento

Nascimento¹,

Pereira²,

Mello³

et al. 2008

Rev. Bras. Anestesiol.

117

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Rev Bras AnestesiolARTIGO DIVERSO 2008; 58: 6: 651-664 MISCELLANEOUS RESUMO Nascimento TS, Pereira ROL, Mello HLD, Costa J -Metemoglobinemia: do Diagnóstico ao Tratamento. JUSTIFICATIVAS E OBJETIVOS: 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.

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Methylene blue in congenital methemoglobinemia: prophylactic or on demand?

Cited by 7 publications

References 3 publications

Long‐term Treatment with Methylene Blue in a Dog with Hereditary Methemoglobinemia Caused by Cytochrome b5 Reductase Deficiency

Long‐term Treatment with Methylene Blue in a Dog with Hereditary Methemoglobinemia Caused by Cytochrome b5 Reductase Deficiency

Intraoperative Management of a Child Undergoing Cardiac Surgery With Congenital Methemoglobinemia Detected in the Operating Room: A Case Report

Metemoglobinemia: do diagnóstico ao tratamento

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