Othman Suhair A, Malik Amel A
Sea Ports Corporation Hospital and Red Sea University , Port Sudan , Sudan.
Sudan J Paediatr. 2016;16(2):80-85.
Congenital insensitivity to pain with anhidrosis (CIPA), also called hereditary sensory and autonomic neuropathy type IV (HSAN-IV), is an extremely rare autosomal recessive disease. It is characterized by insensitivity to pain, inability to sweat, episodes of hyperpyrexia, and intellectual disability. These factors render the affected persons to repeatedly injure and traumatise themselves to the degree that they become disabled. No specific treatment to the moment, but it needs multidisciplinary approach, and certain life adaptations and education. Here we report 4 unrelated Sudanese children affected with this rare neurological disorder, to raise the awareness on this rare disease, reflecting its spectrum, and the challenges which patients and their families face, especially when living in a hot country.
先天性无痛觉伴无汗症（CIPA），也称为遗传性感觉和自主神经病变IV型（HSAN-IV），是一种极其罕见的常染色体隐性疾病。其特征为对疼痛不敏感、无汗能力、高热发作以及智力残疾。这些因素致使患者反复自我伤害和创伤，直至残疾。目前尚无特效治疗方法，但需要多学科方法以及特定的生活调整和教育。在此，我们报告4名患有这种罕见神经疾病的苏丹儿童，他们彼此无亲缘关系，旨在提高对这种罕见疾病的认识，展现其症状范围，以及患者及其家庭所面临的挑战，尤其是生活在炎热国家时面临的挑战。
