• Title/Summary/Keyword: primary congenital

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Bioprosthesis in the Mitral Position: Bovine Pericardial versus Porcine Xenograft

  • Han, Dong Youb;Park, Sung Jun;Kim, Ho Jin;Jung, Sung-Ho;Choo, Suk Jung;Chung, Cheol Hyun;Lee, Jae Won;Kim, Joon Bum
    • Journal of Chest Surgery
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    • v.55 no.1
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    • pp.69-76
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    • 2022
  • Background: While the use of bioprosthetic valves for mitral valve replacement (MVR) is increasing, very few studies have compared bovine pericardial and porcine valves in the mitral position to help guide bioprosthetic selection. Methods: In the present study, patients who underwent MVR using bovine pericardial valves were compared with those who underwent MVR with porcine bioprostheses between January 1996 and July 2018. Those with prior MVR, infective endocarditis, congenital mitral valve disease, or ischemic mitral regurgitation were excluded. The primary outcomes were structural valve deterioration (SVD) and mitral valve reoperation from any cause, and death was regarded as a competing risk. Competing risk analysis and propensity score-matching were used for comparisons. Results: Among the 388 patients enrolled, pericardial and porcine bioprostheses were implanted in 217 (55.9%) and 171 (44.1%), respectively. Propensity score-matching yielded 122 pairs of patients that were well-balanced for all baseline covariates. No significant differences were observed between the groups in unadjusted (p=0.09) and adjusted overall survival (hazard ratio [HR], 1.13; 95% confidence interval [CI], 0.72-1.76; p=0.60). Competing risk analysis revealed no significant differences in the risks of mitral reoperation (HR, 1.07; 95% CI, 0.50-2.27; p=0.86) and development of SVD (HR, 1.57; 95% CI, 0.56-4.36; p=0.39) between the groups. Matched population analysis confirmed similar results regarding reoperation (HR, 0.99; 95% CI, 0.40-3.22; p=0.98) and SVD (HR, 1.39; 95% CI, 0.41-4.73; p=0.60). Conclusion: No significant differences in survival or valve durability were observed between bovine pericardial and porcine bioprosthetic MVR. These findings require further validation through studies with larger sample sizes.

Distribution of maternal risk factors for orofacial cleft in infants in Indonesia: a multicenter prospective study

  • Andi Tajrin;M. Ruslin;Muh. Irfan Rasul;Nurwahida;Hadira;Husni Mubarak;Katharina Oginawati;Nurul Fahimah;Ikeu Tanziha;Annisa Dwi Damayanti;Utriweni Mukhaiyar;Asri Arumsari;Ida Ayu Astuti;Farah Asnely Putri;Shinta Silvia
    • Archives of Craniofacial Surgery
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    • v.25 no.1
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    • pp.11-16
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    • 2024
  • Background: The pathogenesis of orofacial cleft (OFC) is multifactorial, involving both genetic and non-genetic factors, the latter of which play a key role in the development of these anomalies. This paper addresses the incidence of OFC in Indonesia, with a focus on identifying and examining the distribution of contributory factors, including parental medical history, pregnancy history, and environmental influences. Methods: The study was conducted through the collection of primary data. An interdisciplinary research team from Indonesia administered a standardized questionnaire to parents who had children with OFC and who had provided informed consent. The case group comprised 133 children born with cleft lip and/or palate, and the control was 133 noncleft children born full-term. The risk factors associated with OFC anomalies were analyzed using the chi-square test and logistic regression. All statistical analyses were performed using SPSS version 25. A p-value of 0.05 or less was considered to indicate statistical significance. Results: The study comprised 138 children, of whom 82 were boys (59.4%) and 56 were girls (40.6%). Among them, 45 patients (32.6%) presented with both cleft lip and cleft palate, 25 individuals (18.1%) had a cleft palate only, and 28 patients (20.3%) had a cleft lip only. OFC was found to be significantly associated with a maternal family history of congenital birth defects (p<0.05), complications during the first trimester (p<0.05), consumption of local fish (p<0.05), caffeine intake (p<0.05), prolonged medication use (p<0.05), immunization history (p<0.05), passive smoking (p<0.05), and X-ray exposure during pregnancy (p<0.05). Conclusion: The findings indicate close relationships between the incidence of OFC and maternal medical history, prenatal factors, and environmental influences.

Atypical Presentation of Chronic Granulomatous Disease in a Neonate with a Pulmonary Granuloma Mimicking a Tumor: A Case Report (신생아에서 종양으로 오인되는 폐 육아종의 비전형적인 소견을 보인 만성 육아종성 질환: 증례 보고)

  • Young Jin Yoo;Joo Sung Sun;Jang Hoon Lee;Hyun Joo Jung;Yeong Hwa Koh;Joonho Jung;Hyun Gi Kim
    • Journal of the Korean Society of Radiology
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    • v.81 no.4
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    • pp.990-995
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    • 2020
  • Chronic granulomatous disease (CGD) is an uncommon primary immune deficiency caused by phagocytes defective in oxygen metabolite production. It results in recurrent bacterial or fungal infections. Herein, we present a case of CGD with a large pulmonary granuloma in a neonate and review the imaging findings. The patient was a 24-day-old neonate admitted to the hospital with fever. A round opacified lesion was identified on the chest radiograph. Subsequent CT and MRI revealed a round mass with heterogeneous enhancement in the right lower lobe. There were foci of diffusion restriction in the mass. Surgical biopsy of the mass revealed chronic granuloma. Finally, the neonate was diagnosed with CGD caused by mutation of the gp91phox gene. Herein, we present the clinical and imaging findings of this unusual case of CGD.

A Study on Residual Hearing of Hearing Impaired Children (고도난청아(高度難聽兒)에 대(對)한 잔존청력(殘存聽力))

  • Rhee, Kyu-Shik;Kim, Doo-Hie
    • Journal of Preventive Medicine and Public Health
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    • v.6 no.1
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    • pp.51-63
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    • 1973
  • This paper illustrate residual hearing and socio-medical background on the hearing impaired children, 207 comming to Deaf School. attached to Hankuk Social Work College, Taegu, Korea. The survey was performed through interview with their parents and testing by diagnostic audio-meter (TRIO, AS 105 type) at soundproof room from March 10, to November 28, 1973. The results obtained were as follows. 1) The attendance rate of the compulsory primary school was markedly lower tendency in female than male according to directly proportional to prevalence rate of deafness among them. If was showed the deeper gap in the more superior school (middle and high school). 2) Who entered at the suitable age to each school (six years old to primary school, 12 years to middle and 15 years to high) was 11.3%. And who were enrolled in school age to each school (6-11 years for primary. 12-14 years for middle and 15-17 years for high) was 45.9% (43.7% in male, 50.0% in female). 3) As causative disease, congenital case, were 23.6% included of 13.5% of heredity and 10.1% of troubles during pregnancy; the total acquired cases were 47.9%, it was classified as 11.6% of convulsion from any other diseases, 7.7% of measles, 7.7% of other febrile diseases, 3.4% of drug (the most of streptomycin) intoxication, 2.4% of meningitis, 1.5% of epidemic encephalitis and 31.3% of other diseases; and unknown cases were 28.5%. 4) 31.4% of who included congenital cases lost their hearing within six months old, 11.6% in 6-11 months. 9.7% in 1-2 years old and 14.0% in 2-3years old. Consequently we obtained that the most cases 90.0% were lost their hearing within 3 years after birth. 5) According to qualities of hearing leases the most of cases were perceptive, 197(97.5%), only two cases were conductive, and eight cases were mixed. 6) The status of residual hearing according to average grade of hearing loss. $B(=\frac{a+2b+c}{4}$ as table 13) were as follows. Two cases were normal (one was mute and another was severe speach disorder). Ten cases, moderate. Moderately severe cases were 40 (19.3%). Severe cases, 38(18.4%). Scale out, profound cases, 48 (23.3%). And impossible testing cases because that were infantile or had some mental disorder were 69 (33.3%). 7) The using rate of hearing aides was only 12.0%. Among them who had some more residual hearing and could showed hearing effect with hearing aide have used more many proportionary but who were difficult to expect that effect were rare.

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Risk Factor Analysis and Surgical Indications for Pulmonary Artery Banding (폐동맥 밴딩의 위험인자 분석과 수술적응중)

  • Lee Jeong Ryul;Choi Chang Hyu;Min Sun Kyung;Kim Woong Han;Kim Yong Jin;Rho Joon Ryang;Bae Eun Jung;Noh Chung I1;Yun Yong Soo
    • Journal of Chest Surgery
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    • v.38 no.8 s.253
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    • pp.538-544
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    • 2005
  • Background: Pulmonary artery banding (PAB) is an initial palliative procedure for a diverse group of patients with congenital cardiac anomalies and unrestricted pulmonary blood flow. We proved the usefulness of PAB through retrospective investigation of the surgical indication and risk analysis retrospectively. Material and Method: One hundred and fifty four consecutive patients (99 males and 55 females) who underwent PAB between January 1986 and December 2003 were included. We analysed the risk factors for early mortality and actuarial survival rate. Mean age was $2.5\pm12.8\;(0.2\sim92.7)$ months and mean weight was $4.5\pm2.7\;(0.9\sim18.0)\;kg$. Preoperative diagnosis included functional single ventricle $(88,\;57.1\%)$, double outlet right ventricle $(22,\;14.2\%)$, transposition of the great arteries $(26,\;16.8\%)$, and atrioventricular septal defect $(11,\;7.1\%)$. Coarctation of the aorta or interrupted aortic arch $(32,\;20.7\%)$, subaortic stenosis $(13,\;8.4\%)$ and total anomalous pulmonary venous connection $(13,\;8.4\%)$ were associated. Result: The overall early mortality was $22.1\%\;(34\;of\;154)$, The recent series from 1996 include patients with lower age $(3.8\pm15.9\;vs.\;1.5\pm12.7,\;p=0.04)$ and lower body weight $(4.8\pm3.1\;vs.\;4.0\pm2.7,\;p=0.02)$. The early mortality was lower in the recent group $(17.5\%;\;16/75)$ than the earlier group $(28.5\%;\;18/45)$. Aortic arch anomaly (p=0.004), subaortic stenosis (p=0.004), operation for subaortic stenosis (p=0.007), and cardiopulmonary bypass (p=0.007) were proven to be risk factors for early death in univariate analysis, while time of surgery (<1996) (p=0.026) was the only significant risk factor in multivariate analysis. The mean time interval from PAB to the second-stage operation was $12.8\pm10.9$ months. Among 96 patients who survived PAB, 40 patients completed Fontan operation, 21 patients underwent bidirectional cavopulmonary shunt, and 35 patients underwent biventricular repair including 25 arterial switch operations. Median follow-up was $40.1\pm48.9$ months. Overall survival rates at 1 year, 5 years and 10 years were $81.2\%\;65.0\%,\;and\;63.5\%$ respectively. Conclusion: Although it improved in recent series, early mortality was still high despite the advances in perioperative management. As for conventional indications, early primary repair may be more beneficial. However, PA banding still has a role in the initial palliative step in selective groups.

Surgical Treatment of the Congenital Esophageal Atresia (선천성 식도 폐쇄증의 외과적 치료)

  • 최필조;전희재;이용훈;조광조;성시찬;우종수
    • Journal of Chest Surgery
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    • v.32 no.6
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    • pp.567-572
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    • 1999
  • Background: Surgical correction of the full spectrum of esophageal atresia with tracheoesophageal fistula has improved over the years, but the mortality and morbidity assoiated with repair of these anomalies still remains high. Material and Method: We retrospectively analyzes 27 surgically treated patients with esophageal atresia and tracheoesophageal fistula at Dong-A University Hospital between January 1992 and March 1997. Result: There were 21 male and 6 female patients. Mean birth weight was 2.62$\pm$.385 kg(2.0~3.4 kg). Twenty- four(88.9%) had esophageal atresia with distal tracheoesophageal fistula, and 3(11.1%) had pure esophageal atresia. Four(14.8%) infants were allocated to Waterston risk group A, 18(66.7%) to group B, and 5(18.5%) to group C. In eighteen(66.7%) infants with associated anomalies, cardiovascular anomalies were the most common. Three had a gap length of 3.5 cm or greater(ultra-long gap) between esophageal segments, 7 had 2.0 to 3.5 cm(long gap), 8 had 1.0 to 2.0 cm(medium gap), and 9 had 1 cm or less(short gap) gap length. Among 27 neonates, 3 cases underwent staged operation, late colon interposition was done in 2, and all other 24 cases underwent primary esophageal anastomosis. Oerative mortality was 2/27(7.4%). Causes of death included acute renal failure(n=1), empyema from anastomotic leak(n=1), necrotizing enterocolitis(n=1), sepsis(n=1), insulin-dependent diabetus mellitus(n=1 . There were 4 anastomosis- related complications including stricture in 3, leakage in 1. Mortality was related to the gap length(p<.05). Conclusion: Although the complication rate associated with surgical repair of these anomalies is high, this does not always implicate the operative mortality. The overall survival can be improved by effective treatment for combined anomalies and intensive postoperatve care.

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Valve Replacement in Children (소아심장판막치환술)

  • 김재현;이광숙;윤경찬;유영선;박창권;최세영
    • Journal of Chest Surgery
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    • v.32 no.4
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    • pp.341-346
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    • 1999
  • Background: Thirty children ranging from 3 to 15 years of age underwent cardiac valve replacement at Dongsan Medical Center from 1982 to 1997. Material and Method: There were 16 boys and 14 girls. The mean age was 12.1. The underlying pathological cause for valve replacement was congenital heart disease in 17 children and acquired heart disease in 13. The valve replaced was mitral in 15 children, aortic in 11, tricuspid in 3, and combined aortic and mitral in 1. Twenty-one mechanical and 10 tissue valves were placed: primary mechanical valve have been utilized since 1985. Eight of ten patients with tissue valves have had successful second valve replacements 4 to 11 years after the initial operation. Result: The operative mortality was 6.7%, but mortality was higher among patients less than 5 years of age and patients who had previous cardiac operations. Of the 28 operative survivors, 4 patients were lost to follow-up: the remaining patients were observed for a total of 2091 patient/months(mean 74.7 months, maximum 187 months). There was one late death from dilated cardiomyopathy after mitral valve replacement in 7 year-old patient with atrioventricular septal defect. After the operation, all patients with mechanical valves were placed on a strict anticoagulant regimen with Coumadin. The actuarial survival rate was 96% at the end of the follow-up. No instance of thromboembolism or major bleeding were observed in the survivors. Conclusion: These results indicate that valve replacement can be performed with low mortality in children, and with satisfactory long-term survival.

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TOOTH MOVEMENTS TO THE SITE OF ALVEOLAR BONE GRAFT (구순구개열 환아에서의 치조골이식)

  • Cho, Hae-Sung;Park, Jae-Hong;Kim, Gwang-Chul;Choi, Seong-Chul;Lee, Keung-Ho;Choi, Yeung-Chul
    • Journal of the korean academy of Pediatric Dentistry
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    • v.34 no.1
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    • pp.140-149
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    • 2007
  • Cleft lip and palate are congenital craniofacial malformation. Reconstruction of dental arch in patient with alveolo-palatal clefts is very important, because they have many problems in functions and esthetics. Malnutrition, poor oral hygiene, respiratory infections, speech malfunctions, maxillofacial deformity, and psychological problems may be occured without proper treatment during the long period of management of the cleft lip and palate. So the treatment should be managed with a multidisciplinary approach. Bone grafting is a consequential step in the dental rehabilitation of the cleft lip and palate patient A complete alveolar arch should be achieyed of the teeth to erupt in and to form a stable dentition. And the presence of the cleft complicate the orthodontic treatment. Therefore bone grafting in patients with cleft lip and palate is a widely adopted surgical procedure. Grafted bone stabilizes the alveolar process and allows the canine or incisor to move into the graft site. After the bone grafting, orthodontic closure of the maxillary arch has become a common practice for achieving dental reconstruction without any prosthodontic treatment. Various grafting materials have been used in alveolar clefts. Iliac bone is most widely fovoured, but tibia, rib, cranial bone, mandible have also been used. And according to its time of occurrence, the bone graft may be divided into primary, early secondary, secondary, late secondary. Bone grafting is called secondary when performed later, at the end of the mixed dentition. It is the most accepted procedure and has become part of treatment of protocol A secondary bone graft is performed preferably before the eruption of the permanent canine in order to provide adequate periodontal support for the eruption and preservation of the teeth adjacent to the cleft. In this report, we report here on a patient with unilateral cleft lip and palate, who underwent iliac bone graft. The cleft was fully obliterated by grafted bone in the region of the alveolar process. The presence of bone permitted physiologic tooth movement and the orthodontic movement of adjacent tooth into the former cleft area. Satisfactory arch alignment could be achieved in by subsequent orthodontic treatment.

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