Division of Pediatric Emergency Medicine, The Hospital for Sick Children, University of Toronto, Toronto, ON.
Safra Children's Hospital, Sheba Medical Center, Tel Hashomer, Israel.
CJEM. 2020 Sep;22(5):673-677. doi: 10.1017/cem.2020.33.
Acquired methemoglobinemia (MetHb) is an uncommon presentation of cyanosis in the pediatric emergency department (ED), making its diagnosis and management a clinical challenge. Through this case series we hope to improve clinician ability to recognize the potential for MetHb in pediatric ED patients and to avoid overlooking this important cause of cyanosis.
This was a case series using a health records review, investigating patients diagnosed with MetHb at our pediatric ED during 2007-2018. We included only cases with methemoglobin saturation ≥5%.
Ten patients were diagnosed with MetHb in our pediatric ED during the study period. Five had an underlying hematologic disease who received a pharmacologic trigger known to induce MetHb as well (four dapsone, one rasburicase). The other five patients were previously healthy, who presented with a clinical picture of hemolytic anemia, all of whom were diagnosed with previously unknown glucose-6-phosphate dehydrogenase (G6PD) deficiency. Two of the patients received methylene blue, and five patients needed packed red blood cells. All of the patients survived the acute MetHb episode.
Acquired MetHb in the pediatric ED is a rare but important cause of cyanosis. Diagnosis and management of acute, acquired MetHb in the ED requires a high level of suspicion, and a background knowledge of the common precipitants and underlying conditions associated with this condition. We hope this case series will help ED physicians to consider MetHb in pediatric patients presenting with cyanosis and persistent hypoxia. Exposure to known precipitants (e.g., medications and foods), particularly in the setting of active treatment for malignancy or with symptoms of hemolytic anemia should further increase suspicion.
获得性高铁血红蛋白血症(MetHb)是儿科急诊(ED)中出现发绀的罕见表现,这使其诊断和治疗成为临床挑战。通过本病例系列,我们希望提高临床医生识别儿科 ED 患者发生 MetHb 的潜在能力,并避免忽视这种导致发绀的重要原因。
这是一项病例系列研究,通过病历回顾,调查了我们儿科 ED 在 2007 年至 2018 年期间诊断为 MetHb 的患者。我们仅纳入 MetHb 饱和度≥5%的病例。
在研究期间,我们儿科 ED 共诊断出 10 例 MetHb 患者。其中 5 例有潜在的血液系统疾病,且接受了已知会诱发 MetHb 的药物触发因素(4 例使用了氨苯砜,1 例使用了拉布立酶)。其他 5 例患者之前健康,表现为溶血性贫血的临床特征,均被诊断为以前未知的葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症。其中 2 例患者接受了亚甲蓝治疗,5 例患者需要输注红细胞悬液。所有患者均在急性 MetHb 发作中存活。
儿科 ED 中的获得性 MetHb 是一种罕见但重要的发绀原因。ED 中急性、获得性 MetHb 的诊断和治疗需要高度怀疑,并需要了解与这种情况相关的常见诱发因素和潜在疾病。我们希望本病例系列能帮助 ED 医生在出现发绀和持续低氧血症的儿科患者中考虑到 MetHb。暴露于已知的诱发因素(例如药物和食物),特别是在恶性肿瘤的积极治疗或出现溶血性贫血症状时,应进一步增加怀疑。