Congenital insensitivity to pain with anhidrosis and progressing acro-osteolysis:a case report with 7-year follow-up  

作　　者：HU Jun ZHANG Ai-bin LIN Zhen ZHOU Jiang-nan 

机构地区：[1]Department of Orthopaedics, First Affiliated Hospital of Shantou University Medical College, Shantou 515041, China [2]Department of Orthopaedics, Xiangya Hospital, Central South University, Changsha 410078, China

出　　处：《Chinese Medical Journal》2006年第24期2134-2137,共4页中华医学杂志（英文版）

摘　　要：Congenital insensitivity to pain is a rare disorder, first described by Dearborn in 1932. Since the discovery of congenital insensitivity to pain with anhidrosis or hereditary sensory neuropathy type Ⅳ in 1983, fewer than 60 cases have been reported. Congenital insensitivity to pain with anhidrosis and progressing acro-osteolysis is a very rare disorder characterized by absence of painful perception after birth. Severe problems may arise if pain sensation is absent, causing injury to oral structures as teeth, lips and the tongue by self mutilation. The patient is at a risk of late presentation with systemic illnesses associated with pain, such as fracture and joint dislocation. Importantly, the patient may suffer from acro-osteolysis with growth, for instance, osteolysis of the distal extremities.Congenital insensitivity to pain is a rare disorder, first described by Dearborn in 1932. Since the discovery of congenital insensitivity to pain with anhidrosis or hereditary sensory neuropathy type Ⅳ in 1983, fewer than 60 cases have been reported. Congenital insensitivity to pain with anhidrosis and progressing acro-osteolysis is a very rare disorder characterized by absence of painful perception after birth. Severe problems may arise if pain sensation is absent, causing injury to oral structures as teeth, lips and the tongue by self mutilation. The patient is at a risk of late presentation with systemic illnesses associated with pain, such as fracture and joint dislocation. Importantly, the patient may suffer from acro-osteolysis with growth, for instance, osteolysis of the distal extremities.

关 键 词：hereditary sensory and autonomic neuropathies hypohidrosis OSTEOLYSIS 

分 类 号：R747.5[医药卫生—神经病学与精神病学] R681[医药卫生—临床医学]