Cirenei Cedric, Veyckemans Francis, Barbati Melissa, Bert Dina, Laffargue-Vetter Anne, Richart Pierre
From the Departments of Anesthesiology.
Pediatric Hematology, Centre Hospitalier Universitaire Lille, Jeanne de Flandre Hospital, Lille, France.
A A Pract. 2018 Jul 15;11(2):52-53. doi: 10.1213/XAA.0000000000000737.
Hemoglobinopathies caused by unstable hemoglobins represent a wide group of diseases that include hemoglobin Bibba. Hemoglobin instability may cause chronic hemolytic anemia and conformational changes in hemoglobin that affect affinity for oxygen and absorption of wavelengths of light used in pulse oximetry devices. We report the case of an adolescent with hemoglobin Bibba and α-thalassemia. SpO2 was unexpectedly low despite stable vital signs when his hemoglobin was closer to 10 g/dL and normal when his hemoglobin was lower, presumably when there was less unstable hemoglobin because of hemolysis.
由不稳定血红蛋白引起的血红蛋白病是一大类疾病,包括血红蛋白Bibba。血红蛋白不稳定可能导致慢性溶血性贫血以及血红蛋白的构象变化,从而影响对氧气的亲和力和脉搏血氧仪所使用光波长的吸收。我们报告了一例患有血红蛋白Bibba和α地中海贫血的青少年病例。尽管生命体征稳定,但当他的血红蛋白接近10g/dL时,SpO2意外偏低,而当他的血红蛋白较低时,SpO2正常,推测此时由于溶血导致不稳定血红蛋白较少。