• Title/Summary/Keyword: 선천성 기형

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Pediatric Peritoneal Dialysis in Korea : Practical Solution to the Problems of Peritoneal Dialysis for Children (한국 소아복막투석의 현황 - 다기관 공동연구 결과보고 -)

  • Kim Pyung-Kil
    • Childhood Kidney Diseases
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    • v.2 no.2
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    • pp.95-103
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    • 1998
  • Purpose : To find and solve the common problems of peritoneal dialysis(PD) by analysing the clinical data of pediatric PD performed in Korea. Methods : 264 cases of CAPD and acute PD had been performed from Nov.1987 to Oct. 1997 in 17 institutions of pediatric nephrology in Korea. Results : CAPD was performed in 114 cases. The mean age of the patients was $10.5{\pm}6.6$ years and male to female ratio was 1.4:1. The original renal diseases of ESRD were proven in 92 cases($80\%$). The common renal disease of ESRD were FSGS($17\%$), reflux nephropathy ($11\%$), chronic glomerulonephritis($9.6\%$). Mean duration of CAPD was $20{\pm}16.9$ months. Peritonitis was the most common complication and incidence was one episode/18.2 patient-months. Other complications were exit site infection in 10 cases, obstruction in 7 cases, leakage of dialysate in 6 cases. The most common etiologic organism of peritonitis was staphylococcus aureus and the next was staphylococcus coagulase(-). Acute PD was performed in 150 cases. Most common underlying causes were congenital heart disease, hemolytic uremic syndrome, sepsis and dehydration. The mean duration was $10.3{\pm}11.3$ days. The most common complication was peritonitis($20.6\%$). The most common etiologic organism was staphylococcus aureus and coagulase(-), acinetobactor and pseudomonas. Conclusion : Reflux nephropathy should be emphasized in early diagnosis and treatment to prevent ESRD. Incidence of congenital anomaly($7\%$) as a original disease of ESRD was relatively low in Korea. Growth status was not significantly improved after CAPD. In acute PD, the incidence of peritonitis was rapidly increased at 2weeks after beginning of dialysis.

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CASE REPORT OF RUBINSTEIN-TABYI SYNDROME (Bubinstein-Tabyi syndrome에 관한 증례보고)

  • Jung, Sung-Ho;Yang, Kyu-Ho;Lee, Tae-Hee
    • Journal of the korean academy of Pediatric Dentistry
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    • v.28 no.1
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    • pp.166-170
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    • 2001
  • Rubinstein-Tabyi syndrome(RTS) is a congenital multisystem dysmorphic syndrome with many non-specific features, making diagnosis occasionally difficult. The major features of this syndrome include mental retardation, characteristic facial appearance, short stature, microcephaly, and broad thumbs and halluces. This syndrome was first described by Rubinstein and Tabyi in 1963, and many studies have been continued about this syndrome, but specific pathogenesis of the Rubinstein-Tabyi syndrome phenotype is still not clear. High arched palate, micrognathia and multiple caries etc have been reported in Rubinstein-Tabyi syndrome. In this report, a 6-year and 5-month-old boy visited at our department due to multiple dental caries, who showed broad thumbs, mental and physical development retardation, and characteristic facial appearance including both ptosis and ear deformity. This patient was diagnosed as a Rubinstein-Tabyi syndrome, and treated the multiple dental caries under general anesthesia. This study was aimed to observe the relationship between medical and dental characteristics.

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Intestinal Atresia - A Survey by the Korean Association of Pediatric Surgeons - (선천성 장폐쇄증 - 대한소아외과학회 정회원을 대상으로 한 전국조사 -)

  • Kim, I.K.;Kim, S.Y.;Kim, S.K.;Kim, W.K.;Kim, J.E.;Kim, J.C.;Kim, H.H.;Park, K.W.;Park, Y.S.;Park, W.H.;Song, Y.T;Yang, J.W.;Oh, S.M.;Yoo, S.Y.;Lee, D.S.;Lee, M.D.;Lee, S.K.;Lee, S.C.;Chang, S.I.
    • Advances in pediatric surgery
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    • v.5 no.1
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    • pp.75-81
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    • 1999
  • A survey on the intestinal atresias was made among 34 members of Korean Association of Pediatric Surgeons. The response rate was 82.4 %. Two hundred and fifteen patients from the January 1, 1994 to December 31, 1996 were analyzed. The lesions were 73 cases of duodenum(DA), 72 cases of jejunum(JA), 71 cases ileum(IA) and 2 cases cecum and sigmoid colon respectively. There were 2 cases of combined anomalies (DA + JA + IA and DA + JA). Male to female ratio was 1:1 in DA, and 1.8:1 in JA. Seventy four cases(34.3 %) were premature babies(DA 35.2 %, JA:48.6 %, IA:19.2 %), and 62 cases(28.7 %) had low birth weight (DA:39.4 %, JA 33.0 %, IA:13.7 %). Antenatal diagnosis was made in 92 cases(43.6 %). However 22 cases (23.9 %) of them were transferred to pediatric surgeon after delivery. Maternal polyhydramnios was observed in 63 cases(28.9 %). Seventy· five cases(34.4 %) were taken only simple abdominal film for diagnostic studies. The associated malformations were observed in 54 aresia and were observed more frequently in DA(35 cases, 47.9 %). Meconium peritonitis due to intrauterine bowel perforation was more frequently associated with IA compared to DA and JA. The overall mortality rate was 30 %. (Abbreuations: $P_{T}$;p-value in total, $P_{DJ,DI,JI}$;p value between two groups among duodenal, jejunal and ileal groups).

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Assessment of Chromosomal Analyses of 1,180 Cases Suspected of Chromosomal Aberrations (염색체이상을 의심한 1,180례의 염색체 분석 결과 검토)

  • Jeong, Hyeon Kyoung;Ahn, Eun Young;Rim, Sung Soo;Kim, Eun Young;Kim, Kyoung Sim;Kim, Yong Wook;Kim, Ki Bok
    • Clinical and Experimental Pediatrics
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    • v.45 no.3
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    • pp.311-319
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    • 2002
  • Purpose : We have performed this study to obtain reference data for the distribution of chromosomal aberrations in Korea. Methods : We analyzed 1,180 chromosomal study cases from Kwang ju Christian Hospital during the past 25 years. 756 cases suspected of characteristic chromosomal aberration syndromes and 424 cases with hermaphroditism, mild sexual abnormalities, multiple anomalies, or mental & growth retardation were included. Results : The male to female ratio of autosomal aberration syndromes was 1.2 : 1. 78.6% of autosomal aberrations were diagnosed under 1 year of age, whereas 89.8% of sex chromosomal aberrations were diagnosed over 12 years of age. Among 1,180 cases, 612 ones had chromosomal aberrations(51.9%) : 590 of 756 cases suspected of chromosomal aberration syndromes had aberrations( 78.0%), whereas 22 of 424 showing the above other features had aberrations(5.2%). Autosomal aberrations appeared in 514 cases(83.8%) and sex chromosomal aberrations appeared in 98 cases(16.2%). The most frequently observed abberation in autosomal aberrations was Down syndrome, followed by E, D, B, A and C group aberrations. The most common abberation in sex chromosomal aberrations was Turner syndrome, followed by Klinefelter syndrome and Fragile X syndrome. Conclusion : It is of vital importance that patients suspected of chromosomal aberrations undergo chromosomal analysis. Further advanced chromosomal staining and molecular genetic methods will raise the detection rate of chromosomal aberrations.

Maternal and Neonatal Outcome of Twin Pregnancies after in vitro Fertilization and Embryo Transfer (체외수정시술로 출생한 쌍생아의 임상적 경과에 대한 비교 분석)

  • Kim, Kyung-Ah;Min, Uoo-Gyung;Lim, Jae-Woo;Jun, Nu-Lee;Won, Hye-Sung;Kim, Chung-Hoon;Kim, Ellen Ai-Rhan;Lee, Pil-Ryang;Lee, In-Sik;Kim, Ki-Soo;Kim, Ahm;Pi, Soo-Young
    • Clinical and Experimental Pediatrics
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    • v.46 no.3
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    • pp.224-229
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    • 2003
  • Purpose : To examine various neonatal outcomes and perinatal factors resulting from assisted reproduction compared to that of spontaneous conception. Methods : This is a retrospective study. The control cases were all twins of spontaneous conception born between periods from January 1995 to June 2000. The study cases were identified from twins conceived by assisted reproduction in the same time peried. A total of 460 sets of twins consisted of 250 twins of spontaneous conception and 156 twins of assisted reproduction were studied. The primary outcomes were neonatal morbidity and mortality and the secondary outcomes were perinatal factors including number, length and cost of hospitalization for the delivery. Results : No differences were seen in various neonatal factors including gestational age, birth weight and incidences of respiratory distress syndrome, patent ductus arteriosus, necrotizing enterocolitis, hyperbilirubinemia, sepsis, intraventricular hemorrhage and the length of hospitalizations. Lower one minute and five minute Apgar scores and frequently encountered electrolyte abnormalities were observed in neonates of assisted reproduction. In general, the second twin of assisted reproduction had increased incidences of respiratory distress syndrome, sepsis and necrotizing enterocolitis than the first twin. Increased frequencies of preterm labor, hospitalization and elective cesarean section were seen among mothers who underwent artifical conception. However, overall hospital costs in terms of mothers hospitalization for the delivery and neonates hospitalization did not show differences. Conclusion : Assisted reproduction twins had similar neonatal morbidities, mortalities and perinatal morbidities compared to those born by spontaneous conception.

Changes in the Luminal Surface of the PTFE Graft used in Systemic- pulmonary Shunt Operation (체폐단락술에 사용되었던 PTFE 인조혈관 내면의 변화)

  • Park, Yeong-Hwan;Jang, Byeong-Cheol;Sin, Dong-Hwan;Jo, Beom-Gu
    • Journal of Chest Surgery
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    • v.29 no.8
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    • pp.836-843
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    • 1996
  • Histologic, and scanning electron microscopic observa ions were made of 12 biopsy specimens from polytetrdiluoroethylene (PTFE) grafts that had been Implanted as systemic-pulmonAry shunt for improving oxygenation of cyanotic congenital heart disease and harvested near the end of pulmonary artery side at the time of redo shunt or tonal correction between 1985 and 1992. The types of shunt operation are modified Blalock-Taussig shunt in 10 cases, Waterston-Cooley shunt in 1 And Potts shunt in 1 case. The 5 mm PTFE graft was used In 10, 4mm in 1 and 6mm in 1 case. The mean duration of implantalion was 0 $\pm$ 14.1 months(rl.on 12 months to 55 months). The plAtelets were aggregated between gaps'of Coretex surface and intimal thickening was noted about 10 to 20 months after implantation. Endothelial cells were found in the 40-months patent PTFE graft by light and scanning electron microscopy. In the specimen of poor flow or zero flow graft, severe intimal hyperplasia and thrombi which was made of platelets were noted . Based on this experience, we think that the patency will be maintained well if the connective tissue could be Hxed firmly over the Inner layer of the Goretex and the endothelial cell layer sllould form over the con- nective tissue and platelet aggregation should be prevented.

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Reconstruction of the Head and Neck Defects Using Lateral Thigh Free Flap (외측대퇴 유리피판을 이용한 두경부 결손의 재건)

  • Lee, Nae-Ho;Yang, Kyung-Moo
    • Archives of Reconstructive Microsurgery
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    • v.7 no.2
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    • pp.146-156
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    • 1998
  • Microvascular surgery has been widely used clinically for over 30 years. Although many types of free skin and myocutaneous flap are being used at present, surgeons are still looking for new flaps to suit the specific requirements of different recipient sites, to reduce the deformity at the donor site, to ease the management of the flap and to increase the success rate of those operations. The lateral thigh free flap was designed and reported simultaneously with the medial thigh free flap by Baek in 1983. The flap, based on the third perforator of the profunda femoris artery. is designed on the posterolateral aspect of the distal thigh. Clinically, the vascular variations and the locations of perforators of this system can be determined preoperatively with simple angiograms and Dopper audiometry. The lateral thigh free flap is suitable for reconstruction of defects in an oral floor with tongue and esophageal deficits, scalp defects with dural defects, and large full thickness defects of the lip. The advantages of this flap are safe elevation, a long vascular pedicles with a large lumen, skin that is generally thin, and good pliability. Furthermore, the skin territory is very wide and long. The donor site is hidden and therefore more acceptable to the patient. The disadvantage of this flap is that the anatomy of the pedicle vessels has irregular derivation from the main vessel. We had reconstructed lateral thigh free flap to the nine patients from January, 1997 to July, 1998 and got satisfactory results. In this paper we illustrate the arterial anatomy of the thigh and usefulness of this flap for the reconstruction of the head and neck.

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CABG for Treating Unstable Angina with Multivessel Coronary Artery Aneurysms - A case report- (다혈관 관상동맥류가 동반된 불안정성 협심증 환자에서의 관상동맥 우회로술)

  • Kim, Jin-Sik;Chee, Hyun-Keun;Chung, Jin-Woo;Kim, Jun-Seok;Shin, Je-Kyoun;Song, Meong-Gun
    • Journal of Chest Surgery
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    • v.43 no.6
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    • pp.743-746
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    • 2010
  • Coronary artery aneurysm is an uncommon disease. The optimal medical or surgical treatment for this disease remains obscure. The causes of coronary artery aneurysms include atherosclerosis, Kawasaki disease, infectious vascular disease, connective tissue disorder and congenital malformation. A 50 year old man visit our institution for chest pain that had started 3 days previously. After coronary angiography, multiple coronary aneurysms were diagnosed and successful surgical intervention was performed.

Simultaneous Nuss Operation and Mammoplasty in an Adult Patient with Pectus Excavatum - A case report - (성인 여자 오목가슴 환자에서 유방 확대술과 동시에 시행한 너스 수술 - 1예 보고 -)

  • Kim, Kyung-Soo;Cho, Deog-Gon;Cho, Kyu-Do;Jo, Min-Seop;Kang, Chul-Ung
    • Journal of Chest Surgery
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    • v.41 no.4
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    • pp.523-526
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    • 2008
  • Minimally Invasive repair of pectus excavatum using a metal bar, as advocated by Nuss, has been broadly accepted recently for its aesthetic aspect. There are and various treatment methods, as welt. Pectus excavatum may be accompaniedso many modified repair methods have been developed and performed. We report a case of a 33-year-old woman with pectus excavatum and breast hypoplasia, which were simultaneously corrected using a substernal steel bar and mammoplasty.

Analysis of Children with Unilateral Multicystic Dysplastic Kidney(MCDK) (편측성 다낭포성 신이형성증 (Unilateral multicystic dysplastic kidney)의 임상 경과)

  • Yoo Ji Hyung;Yook Jinwon;Kim Ji Hong;Kim Pyung-Kil;Han Sang Won;Kim Myung Joon
    • Childhood Kidney Diseases
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    • v.4 no.1
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    • pp.63-68
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    • 2000
  • Purpose: MCDK is regarded as a common cause of abdominal masses in children. And the presentation of the MCDK is usually a unilateral flank mass in the a newborn. Bialteral disease results in either fetal demise or necessity fer renal replacement therapy at birth. This study is designed to assess the clinical features and natural history of the unilateral multicystic dysplastic kidney. Patients and Methods: From January 1987 to January 2000 data were obtained retrospectively on 57 patients (28 boys and 29 girls, age ranged 1day-11years) who had a diagnosis of multicystic dysplastic kidney. The diagnosis of multicystic dysplastic kidney was confirmed by a combination of ultrasonography and radionuclide scan. Voiding cystourethrogram study in 31 patients were done to determine the condition of the contalateral kidney. Restllts: $84\%$ of the patients were diagnosed before birth by antenatal ultrasonography Clinical manifestations of children with postnatal diagnoses were palpable abdominal mass($3.5\%$), abdominal distension($17\%$), and incidental($10.5\%$). The abnormalities in contralateral kidney were hydronephrosis($21\%$), compensatory hypertrophy($12\%$), simple cyst($2\%$), bifid pelvis($2\%$). Surgical management was performed in 20 patients($35\%$) due to recurrent infection, for diagnostic purpose to differentiate from malignancy and abdominal distention. Follow-up in the remaining 37 patients continued (mean 18 months) and results of sonogram findings were involution change in 23 patients($40\%$) and no interval changes in 13 patient($23\%$). Conclusions : The apparent tendency to regression of the dysplastic kidney and no difference in the number of complications justify a conservative management rather than operative intervention except in associated severe complications such as urinary tract infection or rupture of cysts.

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