• Journal of the Korean Orthopaedic Association
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• v.54 no.5
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• pp.463-468
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• 2019

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare disease that affects the sensory and autonomic nervous system. The patients do not have the ability to sense different sensations, such as pain, which tends to lead to different injuries. In addition, the patients suffer from fluctuations in body temperature due to autonomic involvement. The present case was a five-year-old girl with a neglected distal femur fracture. X-rays taken during the follow-up showed marked callus formation and pseudarthrosis of the distal femur. She had biting injuries of the tongue, auto-amputation of the fingers, some developmental delay and a history of recurrent fever with an unknown origin. The electrodiagnostic study was normal. The quantitative sudomotor axon reflex test revealed markedly reduced postganglionic sudomotor axonal responses at all sites recorded on the left. She was diagnosed with CIPA. As the initial presentation of CIPA involves the musculoskeletal system, orthopedic surgeons should have a high index of suspicion.

• The Korean Journal of Pain
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• v.23 no.3
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• pp.207-210
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• 2010

Fabry disease is an X-linked lysosomal disease caused by deficiency of ${\alpha}$-galactosidase, in which early diagnosis may be missed due to the wide variety of clinical symptoms presenting during disease progression. A 13 year-old boy visited our pain clinic complaining of pricking and burning pain in the toe tips of both feet. Continuous epidural infusion for pain management was performed because of oral analgesics ineffectiveness. The patient underwent ${\alpha}$-galactosidase A (GLA) enzyme analysis based on the clinical impression of Fabry disease from pain with a peripheral neuropathic component and history of anhidrosis. He was diagnosed with Fabry disease after confirming mutation of the GLA gene through a screening test of GLA activity. Enzyme replacement therapy was initiated and pain was tolerated with oral analgesics.

• Archives of Plastic Surgery
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• v.43 no.1
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• pp.95-97
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• 2016

• Journal of Chest Surgery
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• v.32 no.9
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• pp.818-822
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• 1999

Background: Thoracoscopic T2 sympathicotomy had been performed as a simple and effective method in treating palmar hyperhidrosis, however, this method had the complications of compensatory hyperhidrosis and facial anhidrosis. Therefore, a more limited and modified methods for T2 sympathicotomies were introduced and comparative analysis of the modified upper and lower T2 sympathicotomy were made in the treatment of palmar hyperhidrosis. Material and Method: From January 1997 to December 1998, 41 patients with palmar hyperhidrosis had been treated by modified T2 sympathicotomy at the Kon-Kuk University Seoul Hospital. Twenty-four patients underwent a modified upper T2 sympathicotomy(Group A), and seventeen patients underwent a modified lower sympathicotomy(Group B). A comparison between groups A and B were made according to the medical records and interview results. Result: All patients showed symptomatic improvements after the operation. The anisocoria, facial anhidrosis and dissatisfaction for compensatory hyperhidrosis were more common in Group A and the individual satisfaction for the operations were higher in Group B. Conclusion: The modified lower T2 sympathicotomy might be a more effective and less complicated method than the modified upper T2 sympathicotomy.

• Journal of Trauma and Injury
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• v.27 no.4
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• pp.201-203
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• 2014

Patients with Horner's syndrome exhibit a variety of symptoms, including miosis, palpebral ptosis, and anhidrosis. This syndrome is caused by interruptions of the sympathetic neural pathways. This paper describes two cases of patients with Horner's syndrome who experienced a first rib fracture after crushing injuries.

• Korean Journal of Head & Neck Oncology
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• v.10 no.2
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• pp.212-217
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• 1994

The Horner's syndrome includes anisocoria as a result of miosis of the involved pupil with ptosis of the upper and lower lids, which results in slight narrowing of the palpebral fissure. Ipsilateral facial hyperemia and anhidrosis over the face and neck are less common features. The findings with Horner's syndrome are a result of the loss of sympathetic innervation to the ipsilateral eye and face. Recently we experienced 3 cases of Horner's syndrome that developed postoperatively and report briefly with literature.

• The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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• v.28 no.4
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• pp.29-40
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• 2015

Objects : The aim of this study is to introduce latest research trend of cholinergic urticaria and consider results in view of Korean medicine.Methods : We investigate research papers, Chinese and Korean medicinal papers about Cholinergic Urticaria through Pubmed, CNKI(China National Knowledge Infrastructure) and OASIS(Oriental Medicine Advanced Searching Integrated System).Results : The pathogenesis of cholinergic urticaria is supposed to correlate with sweating, considering that many cholinergic urticaria patients are complicated with anhidrosis or hypohidrosis and have sweat hypersensitivity. And on the basis of those outcomes, diverse therapies on cholinergic urticaria are conducted recently.Conclusion : Korean medicinal approaches and treatment on cholinergic urticaria can be significant, accounting that sweating plays a important role in pathogenesis of cholinergic urticaria.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.4
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• pp.392-399
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• 2019

Wilson disease a rare autosomal recessive inherited disorder of copper metabolism, is characterized by excessive deposition of copper in the liver, brain, and other tissues. Wilson disease is often fatal if it is not recognized early and treated when it is symptomatic. Gitelman syndrome is also an autosomal recessive kidney disorder characterized by low blood levels of potassium and magnesium, decreased excretion of calcium in the urine, and elevated blood pH. Hereditary sensory autonomic neuropathy type IV (HSAN-IV), a very rare condition that presents in infancy, is characterized by anhidrosis, absence of pain sensation, and self-mutilation. It is usually accompanied by developmental delay and mental retardation. We report a case of Wilson disease manifested as fulminant hepatitis, acute pancreatitis, and acute kidney injury in a 15-year-old boy comorbid with HSAN-IV and Gitelman syndrome. Such concurrence of three genetic diseases is an extremely rare case.

• Journal of Chest Surgery
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• v.32 no.9
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• pp.813-817
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• 1999

Background: Conventional thoracoscopic thoracic sympathectomy or sympathicotomy is an effective method in treating localized hyperhidrosis; however, this may result in a postoperatively embarrassing compensatory hyperhidrosis or facial anhidrosis in the treatment of palmar hyperhidrosis. We modified the conventional sympathicotomy by limiting the extent of nerve transection. The purpose of this study was to assess the result of the limited thoracoscopic sympathetic nerve transection in hyperhidrosis. Material and Method: From May to August 1998, 17 patients underwent limited transection of the sympathetic nerve. For 9 patients with facial hyperhidrosis, we transected only the interganglionic fiber between the first and the second ganglion, whereas the conventional method cuts two interganglionic fibers. Eight patients with palmar hyperhidrosis underwent limited transection of the interganglionic fiber between the second and third ganglion. Result: Sixteen patients had improved symptom postoperatively. There was a recurred facial sweating in 1 patient 1 month after the operation. Among the 9 facial hyperhidrosis patients, postoperative compensatory hyperhidrosis was severe in 4, moderate in 4 and minimal in 1. But in 8 cases of palmar hyperhidrosis compensatory hyperhidrosis was moderate in 3, and minimal in 1, none in 4. Facial sweating was not disturbed postoperatively in all of the palmar hyperhidrosis patients. Conclusion: Limited sympathetic nerve transection is a practical and less invasive method for the treatment of localized hyperhidrosis and may reduce the incidence of compensatory truncal hyperhidrosis and facial anhidrosis in case of palmar hyperhidrosis.

• Journal of Chest Surgery
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• v.36 no.6
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• pp.448-450
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• 2003

Lipoma is a circumscribed mesenchymal tumor originating from the adipose tissue. The lesion is usually small and asymptomatic. The most common location is in the neck region, however, lipoma can be found in the mediastinum in rare occasions. Although lipoma reach to the large proportions in the mediastinum, it rarely compresses the neurovascular structure. We present a case of a 58-year-old man, in which a hourglass-type cervicomediastinal lipoma produced Horner's syndrome with voice change. The man presented a swelling at the right side of his neck, ptosis and anhidrosis on the right side of his face, and right chest discomfort. After the removal of the mass, all the symptoms which had been provoked by compression, as well as Horner's syndrome and hoarseness, nearly disappeared.