• Clinical and Experimental Pediatrics
• /
• v.45 no.5
• /
• pp.711-715
• /
• 2002

The 18q-syndrome is a deletion disorder that occurs in humans. Clinical symptoms are mental retardation, craniofacial anomalies, skeletal deformity, seizure, and hearing loss. 18q- deletion occurs over a broad region, spanning the interval from 18q22.2 to 18qter rather than a single critical region containing 18q. We experienced a case of 18q-syndrome in a male child. It was diagnosed by clinical and chromosomal study. He was a 15month-old infant who was admitted because of prolonged fever and vomiting. And he manifested a depressed midface, esotropia, anhydrosis, and developmental delay. Peripheral blood chromosome studies showed deleted chromosomal material at the distal part of the long arm of chromosome 18. He showed right hydronephroureterosis on IVP. So, he was diagnosed as 18q-syndrome with right hydronephroureterosis and anhydrosis. We report this syndrome with a review and related literature.

• Journal of Chest Surgery
• /
• v.35 no.10
• /
• pp.724-729
• /
• 2002

Although variable surgical methods of sympathetic nerve for palmar hyperhidrosis are curative and safe therapeutic options, they have some limitations such as compensatory sweating and anhidrosis of hand in long term satisfaction rate. Material and Method: Therefore, we tried to decrease severity of compensatory sweating and prevent excessive dryness of hand through selective division of rami communicantes of thoracic sympathetic ganglia distributed to the hands(ramicotomy). Result: In postoperative results, about half of the patients maintained humidity of hands and most of them showed no more than mild degree of compensatory sweating. Conclusion: Therefore, ramicotomy of thoracic sympathetic ganglia can be recommended as selective and physiologic surgical method for palmar hyperhidrosis.

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

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

• 정보화사회
• /
• s.76
• /
• pp.14-26
• /
• 1994

체신부 정보통신부는 94년도 주요업무계획의 기조를 $\blacktriangle$UR타결에 따른 국제화 $\blacktriangle$개방화의 무한경쟁시대 대응 $\blacktriangle$국가.사회 및 경제활동의 효율화와 산업의 생산성 증대로 국가경쟁력 강화 $\blacktriangle$국가.사회 전반에 걸친 정보화 촉진 $\blacktriangle$정보통신기술개발 및 정보통신산업 육성을 위한 효과적 정책 수립 시행 등으로 설정했다.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.27 no.2
• /
• pp.222-228
• /
• 2000

Ectodermal dysplasia is a hereditary disease characterized by a congenital dysplasia of one or more ectodermal structures and their accessory appendages. At least 120 subtypes of ectodermal dysplasia have been reported. The disease is usually transmitted as an X-linked recessive trait in which the gene is carried by the female and manifested in the male. Hypohidrotic ectodermal dysplasia is manifested as a triad of defects that include hypohidrosis, hypotrichosis and hypodontia. The characteristic facial features consist of asteatosis, onchodysplasia, sparse and fine blond hair, prominent forehead, a depressed nasal bridge, thick everted lips. The patient may suffer from dry skin, hyperthermia and unexplained high fever as a result of the deficiency of sweat glands. This case report presents detailed procedures of rehabilitating functional and esthetic defect of a 6-year-old boy with hypohidrotic ectodermal dysplasia along with the review of relevant literatures.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.28 no.4
• /
• pp.626-632
• /
• 2001

Ectodermal dysplasia is a hereditary disease characterized by congenital dysplasia of one or more ectodermal structures. Intraorally, common findings are anodontia or oligodontia, conical teeth, and, consequently, generalized spacing. This case presented the oral rehabilitation of a child with hypohidrotic ectodermal dysplasia. Oral rehabilitation is important from functional, esthetic, and psychologic perspectives. Due to the absence of teeth, the volume of alveolar bone and its growth are decreased, resulting in a loss of vertical dimension and protuberant lips. The treatment involved increasing the patient's vertical dimension of occlusion, fabricating a maxillary partial denture, and using magnets to help retain the mandibular partial denture. A 5-year 7-month old Korean boy was referred to the pediatric department for examination, evaluation and treatment of his disorder. we used magnets on '73 and '83 for enhanced retention of a mandibular overdenture. The magnet used in this case was the Magfit system(GC Co., Japan).

• Journal of the korean academy of Pediatric Dentistry
• /
• v.26 no.1
• /
• pp.139-145
• /
• 1999

Congenital Insensivity to Pain with Anhidrosis(CIPA) is rare autosomal recessive disorder which is known to be hereditary sensory and autonomic neuropathies(HSAN) type IV. It is predilection for male and observed in early infant. Its clinical features include congenital analgesia, which leads to self-mutilation; inability to sweat, which leads to defective thermoregulation; and mild to moderate mental retardation. Its dental features include self-mutilation by nail peeling, which leads to gingival ulcer, tongue ulcer due to tongue biting, and enamel hypoplasia. Partial anodontia is often observed as well. Seventeen-months-old boy with CIPA was reported. This is the case in which the dental characteristics are described and the dental treatment of patient is discussed.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.46 no.4
• /
• pp.416-421
• /
• 2019

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare, autosomal recessive disorder; affected patients are characterized by inability to feel pain and to sweat over the entire body, as well as by mental retardation. Because, in the oral examination, no specific findings on soft or hard tissue may be found except possible lesions due to self-mutilation, early recognition and diagnosis are essential for these patients. Pediatric dentists must be aware of the clinical manifestations and treatment considerations related to uncontrolled body temperature, tactile hyperesthesia and lack of pain reflex. In this case report, dental management of CIPA was suggested by presenting a 6-year follow-up of young patient.

• Journal of Korean Medical classics
• /
• v.19 no.4
• /
• pp.136-146
• /
• 2006

파(把) "상한론(傷寒論)" 지중풍여상한적발병원인(之中風與傷寒的發病原因), 인위구분성풍사여한사(認爲區分成風邪與寒邪), 시불태적당(時不太適當). 우시(于是), 논자이사기적경중여체질(論者以邪氣的輕重與體質)(양기(陽氣))적성쇠(的盛衰), 진행중풍여상한적분석(進行中風與傷寒的分析), 득도료여하적결론(得到了如下的結論). 기일시(基一是), 중풍시양기허적인감수료경적풍한사(中風是陽氣虛的人感受了輕的風寒邪), 소이사기침입우기육(所以邪氣侵入于肌肉). 상한시양기성적인감수료중적풍한사(傷寒是陽氣盛的人感受了重的風寒邪), 소이사기유우부표(所以邪氣留于膚表). 기이시(其二是), 중풍인정사적대치상태소완(中風因正邪的代置狀態疎緩), 소이기맥위부완(所以其脈爲浮緩). 상한시정사대치우부표이집중우피모(傷寒是正邪代置于膚表而集中于皮毛), 소이기맥위부긴(所以其脈爲浮緊). 기삼시(其三是), 중풍지사기직접거영향우인체(中風之邪氣直接去影響于人體), 소이위(所以爲)“즉발열(卽發熱)”. 상한적(傷寒的) “혹미발열(或未發熱)”시(是), 성적양기가이억제사기적상태(盛的陽氣可以抑制邪氣的狀態). “혹기발열(或己發熱)” 시양기수성이부가이억제중사적상태(是陽氣雖盛而不可以抑制重邪的狀態). 기사시(其四是), 중풍시인양기허이불가이고섭(中風是因陽氣虛而不可以固攝), 소이위자한(所以爲自汗). 상한시강성적정사도취우부표이울폐(傷寒是强盛的正邪都聚于膚表而鬱閉), 소이위무한(所以爲無汗). 기오시(其五是), 중풍감수료경적사기(中風感受了輕的邪氣), 소이견풍이재손양기(所以見風而再損陽氣), 재회유악풍한적증상(才會有惡風寒的症狀). 상한시감수료중적사기(傷寒是感受了重的邪氣), 소이수거밀실이유악풍한적증(所以雖居密室而有惡風寒的症). 기육시(其六是), 계지탕유해기적효능(桂枝湯有解肌的效能), 소이능치중풍증(所以能治中風證). 마황탕유축부표지사기적효능(麻黃湯有逐膚表之邪氣的效能), 소이치상한증(所以治傷寒證). 기칠시(其七是), “발어양(發於陽)”시당우상한(是當于傷寒), “양(陽)”적의사상당시최표층부적부표(的意思相當是最表層部的膚表). “발어음(發於陰)”시당우중풍(是當于中風), “음(陰)”적의사상당우비부표교심부적기육(的意思相當于比膚表較深部的肌肉). 기팔시(其八是), “풍가(風家)”시인양허이역환중풍적인(是因陽虛而易患中風的人).