Pediatrics Department, Jawaharlal Nehru Medical College, Sawangi (Meghe), Wardha, Maharashtra, India.
Pan Afr Med J. 2022 Oct 11;43:71. doi: 10.11604/pamj.2022.43.71.36406. eCollection 2022.
Atypical hemolytic uremic syndrome (aHUS) is a group of disorders that affect kidneys which is rare type of HUS that differs from classical hemolytic uremic syndrome (HUS) by absence of prodromal phase consisting of episodes of diarrhoea due to preceding shiga toxin E. coli (STEC-HUS) infection and is 5% of all HUS cases. Approximately 50% cases present with clinical triad of hemolytic anemia, thrombocytopenia and renal insufficiency. However, it can have unusual clinical features in form of central nervous system involvement. This case, of a 15-year-old Indian boy, is one such rare presentation of atypical haemolytic uremic syndrome associated with posterior reversible encephalopathy syndrome (PRES), or reversible posterior leukoencephalopathy syndrome (RPLS) who presented with anaemia, anasarca, papilledema, hypertension, episodic seizures and significant magnetic resonance imaging (MRI) brain findings. We report this uncommon combination of two syndromes to provide useful insight for clinicians to approach and diagnose such presentation in paediatric patients.
非典型溶血尿毒症综合征(aHUS)是一组影响肾脏的疾病,是一种罕见的 HUS 类型,与由先前的志贺毒素大肠杆菌(STEC-HUS)感染引起的腹泻前驱期不同,其占所有 HUS 病例的 5%。大约 50%的病例表现为溶血性贫血、血小板减少和肾功能不全的三联征。然而,它可能以中枢神经系统受累的不寻常临床表现为特征。本例为 15 岁印度男孩,为不典型溶血尿毒症综合征伴后部可逆性脑病综合征(PRES)或可逆性后部白质脑病综合征(RPLS)的罕见表现,表现为贫血、全身性水肿、视盘水肿、高血压、阵发性癫痫发作和显著的磁共振成像(MRI)脑发现。我们报告了这两种综合征的不常见组合,为临床医生提供了有用的见解,以便在儿科患者中对此类表现进行诊治。