Methemoglobinemia is a rare disorder associated with oxidization of divalent ferro-iron of hemoglobin (Hb) to ferri-iron of methemoglobin (MetHb). Methemoglobinemia can result from either inherited or acquired processes. Acquired forms are the most common, mainly due to the exposure to substances that cause oxidation of the Hb both directly or indirectly. Inherited forms are due either to autosomal recessive variants in the CYB5R3 gene or to autosomal dominant variants in the globin genes, collectively known as HbM disease. Our recommendations are based on a systematic literature search. A series of questions regarding the key signs and symptoms, the methods for diagnosis, the clinical management in neonatal/childhood/adulthood period, and the therapeutic approach of methemoglobinemia were formulated and the relative recommendations were produced. An agreement was obtained using a Delphi-like approach and the experts panel reached a final consensus >75% of agreement for all the questions.

中文翻译：

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高铁血红蛋白血症的诊断和治疗建议

高铁血红蛋白血症是一种罕见的疾病，与血红蛋白 (Hb) 的二价铁铁氧化成高铁血红蛋白 (MetHb) 的铁铁有关。高铁血红蛋白血症可由遗传或获得性过程引起。获得性形式是最常见的，主要是由于暴露于直接或间接导致 Hb 氧化的物质。遗传形式是由于CYB5R3中的常染色体隐性变异基因或珠蛋白基因中的常染色体显性变异，统称为HbM病。我们的建议基于系统的文献检索。就主要体征和症状、诊断方法、新生儿/儿童/成年期的临床管理、高铁血红蛋白血症的治疗方法等问题制定了一系列问题并提出了相关建议。使用类似 Delphi 的方法获得了一致意见，专家小组对所有问题达成了 >75% 的最终共识。