Methemoglobinemia in a child with congenital epidermolysis bullosa

Ya. A. Lezhepekova, K. V. Pshenisnov, Y. Aleksandrovich
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Abstract

Introduction .  Methemoglobinemia is a rather rare cause of hypoxemia and hypoxia, however, with a severe course of the disease and an increase in the concentration of methemoglobin in the blood of more than 20 %, this can cause the development of multi-organ dysfunction and death.The objective was to descript the case of methemoglobinemia in a child with congenital epidermolysis bullosa with the use of a combined drug containing benzocaine.Materials and methods.  Newborn premature baby boy, from the first pregnancy, gestation period 36 weeks, suffering from congenital epidermolysis. Birth weight 2850 g, height 47 cm. Apgar score in the first minute – seven, on the fifth – eight points. As a local treatment, an aerosol for external use «Olazol» was prescribed. At the age of 56 hours from the moment of birth, a sharp deterioration in the child’s condition  was noted in the form of a decrease in SpO2 to 72–76 %, pronounced tachycardia, an increase in methemoglobin concentration in the blood (53.7 %).Results.  A distinctive feature of this case was the presence of severe methemoglobinemia, which led to severe metabolic acidosis, venous hypoxemia and tissue hypoxia. Timely diagnosis and adequate therapy in the early stages of the pathological process contributed to the  complete stabilization of the child’s condition and the normalization of the blood gas composition fifteen hours after the start of therapy.Conclusion.  The use of drugs containing benzocaine in the early neonatal period is a risk factor for the development of methemoglobinemia, which requires a dynamic assessment of the level of methemoglobin in the blood in order to prevent tissue hypoxia.
先天性表皮松解症患儿的高铁血红蛋白血症
导言 . 高铁血红蛋白血症是导致低氧血症和缺氧的一种相当罕见的病因,然而,如果病程严重,血液中高铁血红蛋白浓度增加超过 20%,就会导致多器官功能障碍和死亡。 新生早产男婴,第一次怀孕,孕期 36 周,患有先天性表皮松解症。出生体重 2850 克,身高 47 厘米。阿普加评分第一分钟为 7 分,第五分钟为 8 分。作为局部治疗,医生开了外用气雾剂 "Olazol"。出生 56 小时后,患儿病情急剧恶化,SpO2 下降到 72-76%,心动过速明显,血液中高铁血红蛋白浓度增加(53.7%)。 该病例的一个显著特点是存在严重的高铁血红蛋白血症,导致严重的代谢性酸中毒、静脉低氧血症和组织缺氧。在病理过程的早期阶段进行及时诊断和适当治疗,有助于患儿病情的完全稳定,并在开始治疗 15 小时后使血气成分恢复正常。 新生儿早期使用含苯佐卡因的药物是导致高铁血红蛋白血症的危险因素,因此需要动态评估血液中高铁血红蛋白的水平,以防止组织缺氧。
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