• Title/Summary/Keyword: 모세혈관확장증

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Hereditary Hemorrhagic Telangiectasia (유전성출혈모세혈관확장증의 증례 및 문헌 고찰)

  • Kwon, Jeong-Seung;Ahn, Hyung-Joon;Choi, Jong-Hoon
    • Journal of Oral Medicine and Pain
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    • v.37 no.3
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    • pp.135-139
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    • 2012
  • Hereditary hemorrhagic telangiectasia is a rare autosomal dorminant disease that features abnormal and fragile vascular dilations of terminal vessels in skin and mucous membranes, as well as arteriovenous malformations of internal organs, particularly lungs, brain, and liver. Often patients have not been diagnosed with HHT for a long time, and undiagnosed HHT patients unnecessarily develop serious complications such as severe life-threatening hemorrhage, stroke or brain abscess. Therefore, early detection and appropriate screening is very important. Early detection of HHT allows the appropriate screening for the presence of silent disease such as AVMs in the lungs, liver, or brain, and preventive treatment in the patient and their affected family members. Dentists should be familiar with HHT because the telangiectases on skin and oral mucosa are often the most dramatic and most easily identified component of HHT. Recently, we experienced a case of HHT. We present the case with a review of the literature.

A case of hereditary hemorrhagic telangiectasia (유전성 출혈성 모세혈관 확장증 1례)

  • Lee, Young Seung;Kim, Seonguk;Kang, Eun Kyeong;Park, June Dong
    • Clinical and Experimental Pediatrics
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    • v.50 no.10
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    • pp.1018-1023
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    • 2007
  • Hereditary hemorrhagic telagiectasia (HHT), which is characterized by the classic triad of mucocutaneous telangiectases, arteriovenous malformations (AVMs) and inheritance, is an autosomal dominant disorder. The characteristic manifestations of HHT are all due to abnormalities of the vascular structure. This report deals with the case of a 14-year-old girl with typical features of HHT that include recurrent epistaxis, mucocutanous telangiectases, pulmonary and cerebral AVMs and a familial occurrence.

A Familial Case of Hereditary Hemorrhagic Telangiectasia (유전출혈모세혈관확장증을 가진 가족 1예)

  • Kim, Min;Song, Hwa Young;Jeong, Hun;Park, I Nae;Choi, Sang Bong;Lee, Hyun Kyung;Lee, Sung-Soon;Lee, Young Min;Kim, Su Young;Kim, Yong Hoon;Huh, Jin Won
    • Tuberculosis and Respiratory Diseases
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    • v.66 no.4
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    • pp.314-318
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    • 2009
  • Hereditary hemorrhagic telangiectasia (HHT, also called Osler-Weber-Rendu Disease) is a rare systemic fibrovascular dysplasia characterized by recurrent epistaxis, cutaneous telangiectasia, and visceral arteriovenous malformations (AVMs). HHT is an autosomal dominant disease with a prevalence of 1 in 5,000~8,000. Recurrent epistaxis is often the first and most common manifestation, and about 30% of patients reveal pulmonary AVM. Presently, we report a familial case of HHT. A 61-year-old male with asymptomatic multiple pulmonary AVMs was successfully treated with embolization. His older brother who presented with recurrent epistaxis and multiple telangiectasias was treated with laser ablation. Their pedigree revealed a family history of recurrent epistaxis.

A Study on a Remedy of Telangiectasia using Electromagnetic Waves (전자파를 이용한 모세혈관 확장증 치료에 관한 연구)

  • Kim, Wang-Hyun;Cho, Jei-Won;Kwon, Do-Sung;Chung, Young-Seek;Cheon, Chang-Yul
    • Proceedings of the KIEE Conference
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    • 2009.07a
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    • pp.1570_1571
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    • 2009
  • 본 논문에서는 초고주파를 이용하여 하지정맥류(Varicose vein)의 초기증상인 모세혈관 확장증(Telangiectasia)을 치료하는 데에 목적을 두었다. 유전율 차이에 따라 전자파(Electromagnetic waves)를 흡수하는 양이 다르다는 것을 전제하에 다리 모델링에 필요한 피부, 혈관, 혈액, 피하조직의 유전율을 구하였고, 혈관과 피부의 유전율에 비해 혈액의 유전율이 높다는 것을 통해 다른 조직에 비해 혈액의 전자파 흡수율이 높다는 것을 확인하였다. 피부의 구조와 유전율을 이용하여 모델링 한 후, 시뮬레이션을 통해 SAR(Specific Absorption rate)분포를 확인하였다.

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Clinical Analysis of Primary Varicose Vein - review of 209 cases - (원발성 하지정맥류의 임상적 고찰 - 209례 보고 -)

  • Lee, Yuen-Jae;Park, Chul;Kim, Jong-Seok;Kim, Han-Yong;Yoo, Byung-Ha
    • Journal of Chest Surgery
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    • v.34 no.12
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    • pp.909-916
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    • 2001
  • Background : Varicose vein is a very common vascular disease and has recently become a matter of concern for thoracic and cardiovascular surgens. Material and Method : We analyzed 209 cases or 269 feet with varicose vein retrospectively, which had been treated in our hospital from April 1999 to December 2000. Result : Male : Female ratio was 1:3(Male : 52 cases, Female : 157 cases), mean age was 42.2$\pm$9.7 years old, mean duration of varicosities was 12.2$\pm$9.7 years, and mean follow up was 14.8$\pm$6.1 months from July 2001. Most common symptom was leg pain(122 cases, 58.4%). Long standing job(44 cases), pregnancy(37 cases), and family history related to varicose vein came to 79.9% as the major predisposing or precipitating factors. Anatomic classifications of main lesion were GSV (greater saphenous vein,126 cases), LSV(lesser saphenous vein,18 cases), and reticular veins and telangiectasias(65 cases). Main treatments were stripping of GSV, stab avulsion, ligation of saphenofemoral junction, sclerotherapy, and conservative treatment. Comparing A group (stripping of GSV) with B group(sclerotherapy of GSV), A group had more complications than B group; however, A group had less recurrences than B group(p 0.05). Comparing C group(stab avulsion of LSV) with D group(sclerotherapy of LSV), there were 2 cases of recurrence in D group; however, there were no statistical differences between the two groups in complication and recurrence(p>0.05). Comparing B group(sclerotherapy of GSV) with E group(sclerotherapy of reticular vein and telangiectasia), there were no differences in complication; however, B group had more recurrences than E group. Post-stripping complications were ankle numbness and tingling(2 cases), ankle pain(2 cases),ankle swelling(2 cases), and wound pain(1 case). Postsclerotherapy complications were thrombophlebitis(1 case) and skin ulcer(1 case). Conclusion: Sclerotherapy for varicose vein involving GSV had more recurrences than stripping for lesions involving GSV. Sclerotherapy for reticular vein and telangiectasia had less recurrences than sclerotherapy for lesion involving GSV. Sclerotherapy is a very convenient method without operation and admission, thus further research is demanded in case of varicose vein involving GSV.

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Tracheal Reconstruction with Perichondrial Graft - An Experimental Study in Rabbits - (연골피막편 이식후 기관 결손부위의 재생에 관한 실험적 연구(제 1 보))

  • 이원상;서장수;이성은;홍원표;박찬일
    • Proceedings of the KOR-BRONCHOESO Conference
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    • 1982.05a
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    • pp.10.3-11
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    • 1982
  • Recently through the advancement of medical and surgical managements and the development of low pressure cuffed endotracheal tube, incidence of tracheal stenosis was decreased significantly. Though its incidence was decreased markedly, stenosis was developted unfortunately in the situations such as long term use of respirator, heavy infection, trauma of the trachea and long term intubation etc. Tracheal stenosis had been handled with various methods such as mechanical dilatation, tissue graft techniques, luminal augumentation and end to end anastomosis due to their individual advantages but their effects were not satisfactory. In 1959 Lester had been found the regenerated cartilage from the perichondrium of the rib incidentaly. Since then Skoog, Sohn and Ohlsen were reported chondrogenic potential of perichondrium through the animal experiments. Though many different materials have been tried to rebuild stenosis and gaping defect of trachea, tracheal reconstruction has been a perplexing clinical problems. We choose the perichondrium as the graft material because cartilage is the normal supporting matrix of that structure and it will be an obvious advantage to be able to position perichondrium over a defect and obtain new cartilage there. The young rabbits, which were selected as our experimental animals, were sacrified from two to eight weeks after surgery. The results of our experiment were as follows; 1) In control group, the defect site of trachea was covered with fibrosis and vessels but graft site was covered with hypertrophied perichondrium and vessels. 2) Respiratory mucosa was completely regenerated in defect sites both control and grafted groups. 3) The histologic changes of the grafted sites were as follows: 2 weeks- microvessel dilatation, inflammatory reaction, initiation of fibrosis 4 weeks- decreased microvessel engorgement, submucosal fibrosis, decreased inflammatory reaction immatured cartilage island was noted in the grafted perichondrium (one specimen) 6 weeks- mild degree vascular engorgement submucosal fibrosis. chronic inflamatory reaction cartilage island and endochondrial ossification was noted in the grafted perichondrium (Two specimens) 8 weeks- minute vascular engorgement dense submucosal fibrosis. loss of inflammatory reaction. cartilage island was noted in the grafted perichondrium (two specimens) 4) There was no significant differences in regeneration between active surface in and out groups. 5) We observed immatured cartilage islands and endochondrial ossification in the perichondrial grafted groups where as such findings were not noted in control groups except fibrosis. We concluded that perichondrium was the adequate material for the reconstruction of defected trachea but our results was not sufficient in the aspect of chondrogenic potential of perichondrium. So further research has indicated possibility of chondrogenic potential of perichondrium.

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A Case of Multiple Pulmonary Arteriovenous Malformation in Pregnancy (임신중에 발견된 다발성 폐 동정맥 기형 1예)

  • Lee, Byung-Ho;Cho, Young-Seon;Choi, Cheon-Woong;Yoo, Jee-Hong;Kang, Hong-Mo
    • Tuberculosis and Respiratory Diseases
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    • v.52 no.5
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    • pp.545-549
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    • 2002
  • A pulmonary arteriovenous fistula is an uncommon malformation. In pregnancy altered hemodynamics and hormones cause changes in a PAVM(pulmonary arteriovenous malformation) that predispose them to deterioration. Therefore, a PAVM can cause serious and life-threatening complications in pregnancy. Death often results from a cerebral abscess and a rupture of the malformation with a massive hemorrhage. Recently, we experienced a case of a multiple PAVM in pregnant 38 year old woman, which could not be observed in the old chest PA, 1 year ago. The PAVM was confirmed by CT and was angiography and treated by percutaneous embolization. The patient is suspected to have HHT (Hereditary hemorrhagic telangiectasia).

Clinical Application of Dermatic Care by Laser Therapy in Korean Oriental Medicine (레이저를 이용한 피부치료의 한의학 임상활용-모세혈관 확장증을 중심으로)

  • Yoon So Won;Kim Min Hui;Yoon Hwa Jung;Ko Woo Shin
    • Journal of Physiology & Pathology in Korean Medicine
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    • v.18 no.3
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    • pp.939-941
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    • 2004
  • Phototherapy have perpetual history, we can see an instance of it in Yellow Emperor's Conon of Medicine(黃帝內經) and Laser therapy is a field of Phototherapy being used frequently in the latest time. We treated patients with telangiectasia by laser therapy and experimented it's efficacy and studied the means of clinical application in Korean medicine. We report the capabilities of application of laser therapy, based on Korean medical theories by using resemblance between laser therapy with kyung-rak(經絡) control technique in the line that connects with the past.

A Case of Pulmonary Arteriovenous Fistula with Family History (가족력이 동반된 폐동정맥루 1예)

  • Kuwon, Eun-Sang;Park, Won-Koung;Ryu, Jeong-Seon;Kwak, Seung-Min;Lee, Hong-Lyeol;Jo, Cheul-Ho;Rho, Hyung-Keun
    • Tuberculosis and Respiratory Diseases
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    • v.45 no.3
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    • pp.649-653
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    • 1998
  • Pulmonary arteriovenous fistula is a congenital vascular malformation in lung resulting from abnormal capillary development and the exact etiology is not well known but it may occur with or without hereditary hemorrhagic telangiectasia. Hereditary hemorrhagic telangiectasia (HHT) or Rendu-osler-Weber disease is an autosomal dominant vascular disorder which associates epistaxis, mucocutaneous and visceral telangicetases, and recurrent hemorrhage with chronic anemia and visceral shuntings. Recently we experienced a case of the pulmonary arteriovenous fistula in a 23 year-old woman with a family history of this disease, which was confirmed by pulmonary angiography.

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LOCALIZED SCLERODERMA IN A CHILD : CASE REPORT (소아 피부 경화증 환자의 치험례)

  • Kim, Eun-Young;You, Seung-Hoon;Kim, Jong-Soo
    • Journal of the korean academy of Pediatric Dentistry
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    • v.32 no.2
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    • pp.256-261
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    • 2005
  • Scleroderma is a connective tissue disease of unknown etiology, but known as a kind of auto-immune disease. It is most common in women especially in $30{\sim}50$ years, and very rare in childhood. It can be classified into two main classes : localized scleroderma & systemic sclerosis. Localized type has better prognosis, and usually involves skin only, or in some cases, the muscle below, except inner organs. Systemic type involves skin, oral mucosa and major internal organs. Involving facial skin, we can see small and sharp nose, expressionless stare and narrow oral aperture. Usually they have Raynaud's phenomenon, and in progress, show mouth opening limitation and sclerosis of tongue and gingiva. It is called CREST syndrome showing calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangioectasia. Treatment of scleroderma is systemic and localized steroid therapy, use of collagen-link inhibitor (D-penicillamine), immune depressor and etc. Mouth opening limitation can be improved by mouth stretching exercise. We report a 6 years old boy, diagnosed with localized scleroderma who had mouth opening limitation. We could get additional mouth opening, and have done successful restorative treatment of mandibular and maxillary 1st and 2nd deciduous molar under deep sedation with nitrous oxide and enflurane.

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