• Title/Summary/Keyword: Congenital syphilis

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2 Cases of Bilateral Choanal Atresia (후비공 폐쇄 2례)

  • 심강석;이석용;문태용;윤강묵
    • Proceedings of the KOR-BRONCHOESO Conference
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    • 1983.05a
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    • pp.18.3-19
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    • 1983
  • Choanal atresia, described first by Johann Roedere (1755) is an obstruction between the nasal cavity and nasopharyngeal vault, and the diagnosis and treatment were developed because of severity of the disease. Embryologically, incomplete development of olfactory pit, or failure of nasobuccal membrane to rupture, or persistent remaining of buccopharyngeal membrane, etc, all forms the congenital choanal atresia. And the acquired type was the result from syphilis or diphtheria with a resultant stenosing cicatrix and after the inexpert surgery and the trauma. Multiple abnormalities may be present particularly affecting the head, the heart and the alimentary system in the congenital type. The operative technique employed would depend upon the type of obstruction(whether membranous or bony), the age of the patient, and the presence or absence of any associated pathologic condition. Since Emmert (1853) first tried blind puncture of the atretic plate with the trocar, other surgical techniques have been introduced over the years for the correction of choanal atresia, which were the transnasal, transpalatal, transantral and the transseptal approach. Among them, transpalatal approach was proved to be a popular technique, that it provides a direct route, thus permitting an exact reconstruction and low restenosis rate. Recently, we have experienced two cases of choanal atresia and treated successfully with transpalatal approach, so authors report these cases with a review of the literature.

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Ruptured Aneurysm of Aortic sinus of Valsalva -A Report of 6 Cases- (대동맥동 동맥루 파열 -수술치험 6예-)

  • 이종명
    • Journal of Chest Surgery
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    • v.7 no.2
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    • pp.201-208
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    • 1974
  • The purpose of this paper is to present author's experience with 6 cases of ruptured aneurysm of sinus of Valsalva which were treated surgically during last 10 years. Among the 6 cases, 5 were male and one was female. All of them originated from the right coronary sinus and 5 cases were ruptured into the RV while remained one into RA. The diagnosis was obtained in 4 cases by cineangiocardiogram. Clinically, we had difficulties in differential diagnosis with combined cases of VSD with A.I. and had special experience in its differentiation during cardiac catheterization. By simultaneous trans-venous and trans-arterial catheterization, identified two catheter tips in the RV, and pull back tracing obtained aortic pressure directly from RV, and RA from RV pressure which were benefit in confirm ruptured aneurysm of the aortic sinus. Surgical correction was performed by means of direct suture closure or combined Teflon pledget Of patch enforcement graft after aneurysm resection by trans-RA or trans-RV approach. All patients had no history of bacterial endocarditis, syphilis, or tuberculosis and operative findings revealed intact coronary sinus except involved one moreover 3 cases combined with high VSD which uggested congenital in origin although pathologic reports revealed only fibrosis. Post-operative course were uneventful in all cases but one who had bleeding and 2 months to 9 years follow up results were good and spend their usual life in all cases.

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Correction of Nostril Stenosis with W-plasty, Perialar Flap and Foley Expansion Exercise (W-성형술, 비익변피판 및 도뇨관 훈련을 이용한 비공협착의 교정)

  • Hong, Ki-Yong;Lee, Yoon-Ho
    • Archives of Plastic Surgery
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    • v.37 no.2
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    • pp.143-147
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    • 2010
  • Purpose: Nostril stenosis is an uncommon deformity that develops as a consequence of smallpox, chickenpox, tuberculosis, syphilis, congenital malformations etc. There have been several studies on the surgical techniques to treat it. However, it is difficult to maintain the result for a long time. The goal of this study is to evaluate the use of Wplasty, perialar flap as an operative techniques and expansion exercise using Foley catheter as a method to keep the patency of nostril. Methods: This is a retrospective review of the senior surgeon's (Y.L.) patients who underwent W-plasty and a perialar flap. Patients treated from 2005 to 2009 were reviewed and the postoperative results were evaluated. Average patient age was 24 years, ranged from 1 to 61 years, average follow-up period was 27.5 months, ranged from 3 to 77 months. The mild deformity was released with an incision and expansion by the ballooning of a Foley catheter and corrected by W-plasty only. However, a severe deformity required an additional procedure including perialar flap transposition. During the postoperative period, the patients maintained a nasal stent and exercise using a Foley catheter to prevent recurrence. Results: Five cases of nostril stenosis in four patients were treated using this technique. One case was corrected with W-plasty only, but four cases were more severe and were corrected with W-plasty and a perialar flap. There were no perioperative complications. The patients were satisfied with the results and retained a good shape during the follow-up periods. Conclusion: Nostril stenosis can be corrected with W-plasty and a perialar flap. A perialar flap is added if W-plasty is unable to correct the deformity. A postoperative nasal stent and expansion with a Foley catheter can help in preventing recurrences.

Early Diagnosis of ABCB11 Spectrum Liver Disorders by Next Generation Sequencing

  • Lee, Su Jeong;Kim, Jung Eun;Choe, Byung-Ho;Seo, An Na;Bae, Han-Ik;Hwang, Su-Kyeong
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.20 no.2
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    • pp.114-123
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    • 2017
  • Purpose: The goal of this study was the early diagnosis of ABCB11 spectrum liver disorders, especially those focused on benign recurrent intrahepatic cholestasis and progressive familial intrahepatic cholestasis. Methods: Fifty patients presenting neonatal cholestasis were evaluated to identify underlying etiologies. Genetic analysis was performed on patients suspected to have syndromic diseases or ABCB11 spectrum liver disorders. Two families with proven ABCB11 spectrum liver disorders were subjected to genetic analyses to confirm the diagnosis and were provided genetic counseling. Whole exome sequencing and Sanger sequencing were performed on the patients and the family members. Results: Idiopathic or viral hepatitis was diagnosed in 34%, metabolic disease in 20%, total parenteral nutrition induced cholestasis in 16%, extrahepatic biliary atresia in 14%, genetic disease in 10%, neonatal lupus in 2%, congenital syphilis in 2%, and choledochal cyst in 2% of the patients. The patient with progressive familial intrahepatic cholestasis had novel heterozygous mutations of ABCB11 c.11C>G (p.Ser4*) and c.1543A>G (p.Asn515Asp). The patient with benign recurrent intrahepatic cholestasis had homozygous mutations of ABCB11 c.1331T>C (p.Val444Ala) and heterozygous, c.3084A>G (p.Ala1028Ala). Genetic confirmation of ABCB11 spectrum liver disorder led to early liver transplantation in the progressive familial intrahepatic cholestasis patient. In addition, the atypically severe benign recurrent intrahepatic cholestasis patient was able to avoid unnecessary liver transplantation after genetic analysis. Conclusion: ABCB11 spectrum liver disorders can be clinically indistinguishable as they share similar characteristics related to acute episodes. A comprehensive genetic analysis will facilitate optimal diagnosis and treatment.

A CASE REPORT OF SURGICAL CORRECTION OF NASOMAXILLARY HYPOPLASIA DUE TO CONGENITAL SYPHILIS BY LE FORT II OSTEOTOMY WITH CORONAL APPROACH (선천성 매독에의한 비상악골 부전증환자의 관상두피 접근법에 의한 Le Fort II 골절단술을 이용한 치험례)

  • Um, In-Woong;Kim, Chang-Soo
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.13 no.1
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    • pp.88-94
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    • 1991
  • Maxillofacial deformities are not considered to be a trouble in social life but function. So many maxillofacial plastc surgeons has made efforts to overcome these troubles and bring out more positive life. The proper proportion and shape decide esthetic quality. Lower third of face was consist with lip, cheek, mandibular lower border and mandibular angle. Widening lower third of face give a impression with muscular and recklessness. And lower and wide mandibular angle makes face square shape. Unilateral involvement cause asymmestric face. These face is considered unfavorable, especially in Korea or Japan. We prevent a number of with mandibular angle Bulging which was corrected with mandibular osteotomy or masseter myotomy.

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Comparative study of newborns of Asian immigrant and Korean women (외국인과 내국인 산모 출생아의 비교 연구)

  • Park, Hee-ok;Lim, Jae-woo;Jin, Hyun-seung;Shim, Jae-won;Kim, Min-hee;Kim, Chun-soo;Kim, Eun-ryoung;Kim, Seung-youn;Park, Sang-kee;Lee, Jung-joo
    • Clinical and Experimental Pediatrics
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    • v.52 no.10
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    • pp.1119-1126
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    • 2009
  • Purpose:This study compares the maternal characteristics and birth outcomes of infants of Asian immigrant mothers from developing countries with those of the infants of Korean mothers. Methods:In this multicenter and retrospective study, Asian immigrant women who had delivered between January 2005 and June 2008 were enrolled from9 Medical Centers. In all, 333 births to Asian immigrant women from developing countries (Asian-Korean infants) were included in this study. In addition, sex-, birth year-, and gestational age-matched 333 neonates born to Korean mothers were selected as the control group (Korean infants). On the basis of the hospital data, we investigated the nationality, age, and medical history of the mothers and compared the incidence of congenital infection, Apgar score, weight, height, and head circumference of Asian-Korean infants with those of the Korean infants. Results:The average maternal age of Asian women from developing countries at birth term was 26.7 years, which was significantly lower than that of Korean women (30.8 years, P<0.05). The birth weight of Asian-Korean infants (2,869 g) was significantly smaller than that of Korean infants (2,995 g, P<0.05). There was a significant difference in the incidence of congenital syphilis infection between the Asian-Korean infants and Korean infants (5 cases vs. 0 case, P<0.05). Conclusion:There were significant differences in the perinatal outcomes between the Korean and Asian-Korean infants. A multicenter large-scaled study should be performed to analyze the perinatal outcomes of Asian-Korean infants.