Rozentsveig V, Katz A, Weksler N, Schwartz A, Schilly M, Klein M, Gurman G M
Division of Anesthesiology and Critical Care Medicine, Soroka University Medical Center, Faculty of Health Sciences, Ben Gurion University of the Negev, Beer Sheva, Israel.
Paediatr Anaesth. 2004 Apr;14(4):344-8. doi: 10.1046/j.1460-9592.2003.01235.x.
Congenital insensitivity to pain with anhidrosis (CIPA, or hereditary sensory and autonomic neuropathy type IV) is a rare, autosomal recessive disease, related to a mutation in the TrkA gene, characterized by inability to sweat, insensitivity to pain and recurrent episodes of hyperpyrexia. There are two Bedouin tribes in Israel with different mutations of the TrkA gene: one in the southern region and the other in the northern region. The Soroka University Medical Center is the referral centre for the entire southern region of Israel. One in 4500 anaesthesia cases involves a patient with CIPA.
We reviewed 40 anaesthesia records of 20 patients with CIPA for anaesthetic technique and incidence of side-effects.
Sixteen patients developed complications in the immediate perioperative period: mild hypothermia in one patient and cardiovascular events in 15 others with one case of cardiac arrest. These complications were unrelated to the anaesthetic drug administered. There were no events of hyperthermia or postoperative nausea.
Cardiovascular complications following anaesthesia are common in patients with the southern Israel variant of CIPA. Hyperthermia, previously recognized as a major concern in patients with congenital insensitivity to pain with anhydrous, was not seen in our patients. We conclude that cardiovascular involvement is frequently encountered in CIPA patients following anaesthesia and is the major concern in their anaesthetic management.
先天性无痛觉伴无汗症（CIPA，或遗传性感觉和自主神经病变IV型）是一种罕见的常染色体隐性疾病，与TrkA基因突变有关，其特征为无汗、对疼痛不敏感以及反复出现高热发作。以色列有两个贝都因部落存在不同的TrkA基因突变：一个在南部地区，另一个在北部地区。索罗卡大学医学中心是以色列整个南部地区的转诊中心。每4500例麻醉病例中就有1例涉及CIPA患者。
我们回顾了20例CIPA患者的40份麻醉记录，以了解麻醉技术和副作用发生率。
16例患者在围手术期即刻出现并发症：1例患者出现轻度体温过低，15例出现心血管事件，其中1例心脏骤停。这些并发症与所使用的麻醉药物无关。未发生高热或术后恶心事件。
以色列南部CIPA变异型患者麻醉后心血管并发症很常见。我们的患者中未出现先前被认为是先天性无痛觉伴无汗症患者主要问题的高热。我们得出结论，CIPA患者麻醉后经常出现心血管问题，这是其麻醉管理中的主要关注点。
