Pelvic osteotomy is commonly used to adjust acetabula dysplasia for congenital dislocation of the hip, whereas congenital insensitivity to pain with anhidrosis(CIPA) is a rare hereditary disease that often has the cha...Pelvic osteotomy is commonly used to adjust acetabula dysplasia for congenital dislocation of the hip, whereas congenital insensitivity to pain with anhidrosis(CIPA) is a rare hereditary disease that often has the characteristics of joint development deformity and easy fracture. This article reports the case involving a CIPA patient who was surgically treated by Chiari pelvic osteotomy and proximal femoral rotation osteotomy for congenital dislocation of the left hip joint and was provided long-term follow-up for redislocation and bilateral femoral head absorption.展开更多
Congenital insensitivity to pain and anhidrosis (CIPA) is a rare form of hereditary sensory and autonomic neuropathy. It is characterized by impaired perception of pain and temperature, anhidrosis and intellectual dis...Congenital insensitivity to pain and anhidrosis (CIPA) is a rare form of hereditary sensory and autonomic neuropathy. It is characterized by impaired perception of pain and temperature, anhidrosis and intellectual disability. Self mutilating behaviors lead to accidental injuries. The limb lesions are often infected and frequently progress to chronic osteomyelitis. In pediatrics, amyloidosis usually occurs secondary to chronic inflammatory diseases. The coexistence of amyloidosis and CIPA has not previously been reported in literature. A CIPA case complicated with nephrotic syndrome and renal amyloidosis following chronic osteomyelitis is presented here. This report emphasizes the importance of close follow-up of patients by urine analysis for the risk of developing amyloidosis particularly in the presence of chronic infections.展开更多
Congenital insensitivity to pain with anhidrosis （CIPA） is an autosomal recessive form of sensory neuropathymanifesting with the lack of perception of pain.^1 There have been few reports of Charcot spine in patients...Congenital insensitivity to pain with anhidrosis （CIPA） is an autosomal recessive form of sensory neuropathymanifesting with the lack of perception of pain.^1 There have been few reports of Charcot spine in patients with CIPA,^2 and surgery for such cases was accompanied by high revision proportion.^3＇4 The purpose of this report is to describe a patient with CIPA who developed a recurrent Charcot arthropathy in her lumbar spine and was treated by revision surgery, with the aim to discuss the strategy of surgical management for such cases.展开更多
Introduction:Congenital analgesia is a rare autosomal recessive hereditary disease.The primary damage of congenital analgesia is central structure damage of comprehensive pain perception.Case presentation:A 1-year-old...Introduction:Congenital analgesia is a rare autosomal recessive hereditary disease.The primary damage of congenital analgesia is central structure damage of comprehensive pain perception.Case presentation:A 1-year-old Han Chinese boy was admitted to hospital because of a tongue bite.He had no response to noxious stimulation of the body surface and was diagnosed with congenital analgesia.A small dose of remifentanil was intravenously injected during anesthetic induction to reduce the stress response caused by endotracheal intubation.A certain depth of anesthesia should be guaranteed during anesthetic induction and surgery to alleviate the stress response induced by endotracheal intubation and the operation.Conclusion:Opioid analgesics are not required for general anesthesia in patient with congenital insensitivity to pain.With a heat dissipation barrier in patients with congenital insensitivity to pain with anhidrosis,body temperature,end-tidal carbon dioxide and bispectral index should be monitored.展开更多
Congenital insensitivity to pain and anhidrosis （CIPA） is a rare entity.CIPA patients are subjected to repeated injuries,which are easily neglected.There is no specific treatment for CIPA,but supervision by parents ...Congenital insensitivity to pain and anhidrosis （CIPA） is a rare entity.CIPA patients are subjected to repeated injuries,which are easily neglected.There is no specific treatment for CIPA,but supervision by parents and patient training are important to avoid further damage and to maintain quality of life.We present two cases of CIPA involving a brother and sister,whose parents did not have a consanguineous marriage.展开更多
Purpose: To understand the multiple signs of Horner syndrome and to recommend protocols for pediatricians to obtain an accurate diagnosis of Horner syndrome. Methods: The medical records of 17 pediatric patients with ...Purpose: To understand the multiple signs of Horner syndrome and to recommend protocols for pediatricians to obtain an accurate diagnosis of Horner syndrome. Methods: The medical records of 17 pediatric patients with Horner syndrome, neonates to eighteen years of age, were collected and analyzed. Data recorded included age, presenting symptoms, other medical history, allergies, medications, pupil size, presence of anhidrosis, and presence of ptosis. From the available pupil sizes, average degree of anisocoria was calculated. Results: All 17 patients had other clinical findings of Horner syndrome in addition to anisocoria. On initial evaluation, 100% had ptosis and 25% had anhidrosis. Of the available pupil size data, the average level of anisocoria was 2.06 mm, with a standard deviation of 1.17 mm. Conclusion: Physicians are reminded to measure pupil size to determine the degree of anisocoria when present, as it may help distinguish benign conditions from underlying pathology. Educating pediatricians on measurement of anisocoria and additional signs of Horner syndrome will help with proper referral patterns.展开更多
文摘Pelvic osteotomy is commonly used to adjust acetabula dysplasia for congenital dislocation of the hip, whereas congenital insensitivity to pain with anhidrosis(CIPA) is a rare hereditary disease that often has the characteristics of joint development deformity and easy fracture. This article reports the case involving a CIPA patient who was surgically treated by Chiari pelvic osteotomy and proximal femoral rotation osteotomy for congenital dislocation of the left hip joint and was provided long-term follow-up for redislocation and bilateral femoral head absorption.
文摘Congenital insensitivity to pain and anhidrosis (CIPA) is a rare form of hereditary sensory and autonomic neuropathy. It is characterized by impaired perception of pain and temperature, anhidrosis and intellectual disability. Self mutilating behaviors lead to accidental injuries. The limb lesions are often infected and frequently progress to chronic osteomyelitis. In pediatrics, amyloidosis usually occurs secondary to chronic inflammatory diseases. The coexistence of amyloidosis and CIPA has not previously been reported in literature. A CIPA case complicated with nephrotic syndrome and renal amyloidosis following chronic osteomyelitis is presented here. This report emphasizes the importance of close follow-up of patients by urine analysis for the risk of developing amyloidosis particularly in the presence of chronic infections.
文摘Congenital insensitivity to pain with anhidrosis （CIPA） is an autosomal recessive form of sensory neuropathymanifesting with the lack of perception of pain.^1 There have been few reports of Charcot spine in patients with CIPA,^2 and surgery for such cases was accompanied by high revision proportion.^3＇4 The purpose of this report is to describe a patient with CIPA who developed a recurrent Charcot arthropathy in her lumbar spine and was treated by revision surgery, with the aim to discuss the strategy of surgical management for such cases.
基金Scientific and Technological Projects of Henan Province,China(no.182102310440)Medical Science R&D Program of Henan Province,China(no.2018020598)International Research Laboratory Program of Henan Province,China(no.201605-005)。
文摘Introduction:Congenital analgesia is a rare autosomal recessive hereditary disease.The primary damage of congenital analgesia is central structure damage of comprehensive pain perception.Case presentation:A 1-year-old Han Chinese boy was admitted to hospital because of a tongue bite.He had no response to noxious stimulation of the body surface and was diagnosed with congenital analgesia.A small dose of remifentanil was intravenously injected during anesthetic induction to reduce the stress response caused by endotracheal intubation.A certain depth of anesthesia should be guaranteed during anesthetic induction and surgery to alleviate the stress response induced by endotracheal intubation and the operation.Conclusion:Opioid analgesics are not required for general anesthesia in patient with congenital insensitivity to pain.With a heat dissipation barrier in patients with congenital insensitivity to pain with anhidrosis,body temperature,end-tidal carbon dioxide and bispectral index should be monitored.
文摘Congenital insensitivity to pain and anhidrosis （CIPA） is a rare entity.CIPA patients are subjected to repeated injuries,which are easily neglected.There is no specific treatment for CIPA,but supervision by parents and patient training are important to avoid further damage and to maintain quality of life.We present two cases of CIPA involving a brother and sister,whose parents did not have a consanguineous marriage.
文摘Purpose: To understand the multiple signs of Horner syndrome and to recommend protocols for pediatricians to obtain an accurate diagnosis of Horner syndrome. Methods: The medical records of 17 pediatric patients with Horner syndrome, neonates to eighteen years of age, were collected and analyzed. Data recorded included age, presenting symptoms, other medical history, allergies, medications, pupil size, presence of anhidrosis, and presence of ptosis. From the available pupil sizes, average degree of anisocoria was calculated. Results: All 17 patients had other clinical findings of Horner syndrome in addition to anisocoria. On initial evaluation, 100% had ptosis and 25% had anhidrosis. Of the available pupil size data, the average level of anisocoria was 2.06 mm, with a standard deviation of 1.17 mm. Conclusion: Physicians are reminded to measure pupil size to determine the degree of anisocoria when present, as it may help distinguish benign conditions from underlying pathology. Educating pediatricians on measurement of anisocoria and additional signs of Horner syndrome will help with proper referral patterns.
