Hanafy Ehab, Alshareef Duaa, Osman Suhaila, Al Jabri Abdullah, Nazim Faisal, Mahmoud Gihan
Prince Sultan Oncology Center, King Salman Armed Forces Hospital, Tabuk, 100, Kingdom of Saudi Arabia.
Pediatric Department, King Salman Armed Forces Hospital, Tabuk, Kingdom of Saudi Arabia.
J Med Case Rep. 2018 Feb 1;12(1):24. doi: 10.1186/s13256-017-1559-x.
Posterior reversible encephalopathy syndrome is a neurotoxic condition that occurs as a result of the failure of posterior circulatory autoregulation in response to acute changes in blood pressure. Overperfusion with resultant disruption of the blood-brain barrier results in vasogenic edema, but not infarction. Posterior reversible encephalopathy syndrome can be the presenting feature of postinfectious glomerulonephritis, which has been reported in approximately 5% of hospitalized children, and it has been reported in very few cases of adult patients with sickle cell anemia. We report a very rare case of posterior reversible encephalopathy syndrome that occurred in a child with sickle cell anemia. This presentation should be differentiated from other neurologic manifestations that occur in patients with sickle cell anemia, because management is totally different.
We report what is to our knowledge the first reported case of a 9-year-old Saudi girl with sickle cell anemia who developed posterior reversible encephalopathy syndrome secondary to asymptomatic poststreptococcal glomerulonephritis. This occurred after full recovery from acute chest syndrome and severe vaso-occlusive crisis.
The purpose of this report is to emphasize that all efforts should be made to explore the causes of different neurologic manifestations that occur in patients with sickle cell anemia, because this will require different pathways of management.
后部可逆性脑病综合征是一种神经毒性疾病,它是由于后循环自动调节功能在应对血压急性变化时失败而发生的。过度灌注导致血脑屏障破坏,进而引发血管源性水肿,但不会导致梗死。后部可逆性脑病综合征可能是感染后肾小球肾炎的首发症状,据报道约5%的住院儿童会出现这种情况,而成人镰状细胞贫血患者中仅有极少数病例有相关报道。我们报告了一例非常罕见的后部可逆性脑病综合征发生在一名镰状细胞贫血患儿身上的病例。这种表现应与镰状细胞贫血患者出现的其他神经学表现相鉴别,因为治疗方法完全不同。
我们报告了据我们所知首例9岁沙特女孩镰状细胞贫血患者,继发于无症状性链球菌感染后肾小球肾炎而发生后部可逆性脑病综合征。这发生在急性胸部综合征和严重血管闭塞性危机完全康复之后。
本报告的目的是强调应尽一切努力探究镰状细胞贫血患者出现不同神经学表现的原因,因为这需要不同的治疗途径。