• Title/Summary/Keyword: Congenital malformation

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Effect of Subtotal Pancreatectomy of Female Mice on the Embryo and Offspring (암컷 생쥐의 췌장 부분절제가 배아와 신생자에 미치는 영향)

  • Cho, Young-Jae;Lee, Jung-Sun;Gu, Sun-Kyung;Park, Moon-Nyeo;Lee, Ha-Kye;Cho, Byung-Nam
    • Development and Reproduction
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    • v.10 no.3
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    • pp.185-192
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    • 2006
  • It is known that maternal diabetes induces a premature delivery and a congenital malformation. Although it was suggested that hyperglycemia is a main cause, little has been known. The effects of subtotal pancreactomized animal as a diabetic model on the embryo and the offspring were investigated. 1) Although survival rate of offsprings from the 90% pancreactomized female was lower than that of control, body weight of the survived offsprings was similar to that of control until 6 weeks after birth. When the liver was investigated, abnormal tissue was observed in both female and male offsprings from the pancreactomized mother. The level of major urinary protein(MUP) that is synthesized in the liver and secrets into urine was decreased in both female and male, supporting the liver abnormality. 2) In the pancreactomized female, size of the Langhan's island was increased and the liver also was observed abnormal. However, blood glucose level of the pancreactomized mother was not different from that of control. The low body weight and survival rate of offsprings from the $inhibin-{\alpha}$ overexpressed mice was very similar to those of the offsprings from diabetic mother. Thus when the mother itself was investigated after DNA injection, blood glucose level in the $inhibin-{\alpha}$ overexpressed mice was not different from that of control although size of the Langhan's island was decreased. Taken together, subtotal pancreactomy affected the offspring and embryo and influenced deeply the mother itself. However glucose level of the mother was not changed during investigation. It suggested that the relationship between the diabetic mother and embryo through offspring is very complex.

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Usefulness of Full-thickness Skin Graft from Anterolateral Chest wall in the Reconstruction of Facial Defects (안면부 재건에서 전외측 흉벽을 공여부로 하는 전층 피부이식술의 유용성)

  • Yoo, Won-Jae;Lim, So-Young;Pyon, Jai-Kyong;Mun, Goo-Hyun;Bang, Sa-Ik;Oh, Kap-Sung
    • Archives of Plastic Surgery
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    • v.37 no.5
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    • pp.589-594
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    • 2010
  • Purpose: Full thickness skin grafts are useful in the reconstruction of facial skin defects when primary closure is not feasible. Although the supraclavicular area has been considered as the choice of donor site for large facial skin defect, many patients are reluctant to get a neck scar and some patients do not have enough skin to cover the defect owing to the same insult occurred to the neck such as burn accident. We present several cases of reconstruction of facial skin defects by freehand full-thickness skin graft from anterolateral chest wall resulting aesthetically acceptable outcome with lesser donor site morbidity. Methods: Retrospective review was performed from March, 2007 to September, 2009. 15 patients were treated by this method. Mean age was 31.5 years. The ethiology was congenital melanocytic nevus in 7 cases, capillary malformation in 5 cases and burn scar contracture in 3 cases. Mean area of lesion was measured to 67.3 cm2 preoperatively. The lesion was removed beneath the subcutaneous fatty tissue layer. The graft was not trimmed to be thin except defatting procedure. For the larger size of defect, two pieces of grafts were harvested from both anterolateral chest wall in separation and combined by suture. Results: The mean follow up period was 9.7 months. All the grafts survived without any problem except small necrotic areas in 4 cases, which healed spontaneously under conventional dressings in 6 weeks postoperatively. Color match was relatively excellent. There were 2 cases of hyperpigmentation immediately, but all of them disappeared in a few months. Conclusion: In cases of large facial skin defects, the anterolateral chest wall may be a good alternative choice of full-thickness skin graft.

Effects of TGF-$\beta$3 on Epithelial-mesenchymal transformation and Epidermal growth factor receptor expression in palatogenesis of chicken embryo (계태아 발생시 TGF-$\beta$3가 구개판 내측돌기상피의 상피간엽변환 및 상피성장인자수용체 발현에 미치는 영향)

  • Yang Byoung-Eun;Lee Jong-Ho
    • Korean Journal of Cleft Lip And Palate
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    • v.4 no.1
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    • pp.13-26
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    • 2001
  • Cleft lip and/or palate is the congenital orofacial malformation most commonly occurred in humans, The disease is multifactorial and is probably caused by genetic and/or environmental factors, So, there are many problems in research concerning etiology and in treatment of the disease, Even the most practiced and sophisticated methods of surgical repair are necessarily followed by scar contraction and fibrosis, which result in skeletal defects, dental abnormalities, cosmetic disfigurement, and speech impairment, As a result, Fetal surgery can be considered but practiced rarely when the deformity is not fatal to life, And treatment of cleft palate is performed in the form of medicine projection into uterus in animal experiments, Many studies show that growth factor and its receptor emerge from the developing palate; and the epidermal growth factor receptors have a important role in craniofacial development and in palatal fusion, The palatal morphogenesis of the avine is different from the mammal's; it takes the form of physiologic cleft palate, Recently, cleft palate fusion experiment was performed when the avine were in the period of palate formation through the exogenous TGF-β3 addition, and it showed that the exogenous TGF-β3 makes fusion of divided palate through certain process when cleft palate is occurred in palatal formation, In this study, I had the conformation of the fusion of cleft palate through the addition of TGF-β in case of chicken embryo, and observed the effect of TGF-β in EGF receptor distribution, And the following is the results of this study, 1. In case of the TGF-βl and β3 addition group, there was the decrease of EGFR(Epidermal Growth Factor Receptor) immunoreactivity in mesenchymal cells beneath the medial edge epithelium and also in epithelial mesenchymal interface which is between medial edge epithelium and nasal septum in 72 hours, 2, The immunoreactivity of the control group resembles that of normal chicken embryo palate in development, 3. In the view through fluorescence confocal microscopy, there was confluence in TGF-β3 addition group, This shows that the confluence induced by exogenous TGF-β3 is related to EGFR expression in palate of chicken embryo, which is a physiologic cleft palate model.

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Double Processing Obturator for the Hemi-maxillectomy Treated Patient (Hemi-Maxillectomy 환자에서 이중온성법을 이용한 폐색장치의 제작)

  • Hong, Jun-won;Song, Kwang-yeob;Park, Mi-hee;Jeong, Soo-yang;Kim, Kyoung-il;Ahn, Seung-geun;Park, Ju-mi
    • Journal of Dental Rehabilitation and Applied Science
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    • v.25 no.4
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    • pp.329-336
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    • 2009
  • In the case of congenital malformation, acquired trauma, or maxillectomy by neoplasm, the defect in maxilla is combined with nasal cavity, maxillary sinus and oral cavity. This results in abnormal function in pronunciation, mastication, and swallowing. In this situation, the purposes of prosthodontics are also applied. The purposes of obturator for acquired maxillary deficiency patients are restoring pronunciation, mastication, and swallowing, and make patients comfortable and esthetic satisfaction. Reducing weight of the obturator makes edentulous ridge heathy and functional. It also improves patients' comfort. Hollow obturator also reduces unnecessary stress because improves physiologic function, as it weights less than 6.55-33.06% compare to usual one. This case presents the double-processing method for maxillary obturator in the patient treated hemi-maxillectomy due to malignancy neoplasm. We report this case because the patient acquired functional and esthetic satisfaction from double-processing obturator.

Late Results of Total Correction for Tetralogy of Fallot (활로 4징증의 완전교정술에 대한 장기 성적)

  • Lee, Yeon-Jae;Hwang, Sang-Won;Kim, Han-Yong;Yu, Byeong-Ha
    • Journal of Chest Surgery
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    • v.30 no.12
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    • pp.1184-1189
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    • 1997
  • Tetralogy of Falloff is the most common cyanotic congenital cardiac malformation. Between April 1984 and December 1993, we experinced with 39 cases of total correction for the tetralogy of Falloff at the department of Thoracic and Cardiovascular Surgery, Masan Samsung General Hospital. The results were as follows; There were 14 males and 25 females, and their ages ranged from 2 to 36 years, with an average age of 13.33 $\pm$7.40 years. There was type I VSD in 10 cases(25.6%), type II VSD in 19 cases(48.8%) and type 1+ II VSD in 10 cases(25.6%) Types of right ventricular outflow tract obstruction were valvar + infundibular stenosis in 19 cases(48.7%), valvar + infundibular + annular stenosis in 10 cases(25.6%), infundibular stenosis in 9 cases(23.1%)and pure valvar stenosis in 1 case(2.6%). Right ventricular outflow tract patch widening was necessary in 33 cases(84.6%) and a Iransannular patch including 2 cases of Monocusp-bearing outflow patch was used in 10 cases. n 36 hospital survivors, 23 patients were evaluated by 2 dimensional and doppler echocardiography. The most common complication was arrthymia, especially complete right bundle branch block, in 30 cases (76.9%). The operative mortality was 7.7%, and the cause of death was low cardiac output syndrome.

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A Case of Pulmonary Sequestration Treated with Arterial Embolization (동맥색전술로 시술한 폐격리증 1예)

  • Lee, Gun-Wha;Kim, Dong-Kyun;Na, Hyun-Sik;Bae, Jun-Yong;Choi, Jun-Ho;Kim, Yang-ki;Kim, Ki-up;Uh, Soo-taek;Kim, Dong-hoon;Hwang, Jung Hwa;Goo, Dong-Erk
    • Tuberculosis and Respiratory Diseases
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    • v.58 no.1
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    • pp.68-73
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    • 2005
  • Pulmonary sequestration is a very rare congenital malformation in which a mass of pulmonary tissue is detached from the normal lung and receives its blood supply from a systemic artery. It may be clinically asymptomatic or it has a wide spectrum of various clinical manifestations. The clinical therapeutic approach is to resect the sequestered lobe to prevent frequent complication such as infection. The arterial embolization of feeding artery is a new technique and a less invasive treatment than conventional surgical removal. We have experienced a 17-year-old male with pulmonary sequestration whose complaints were pain in left lower chest. He was diagnosed by computed tomography and aortography and successfully treated with embolization of feeding artery. We report a case of pulmonary sequestration treated with arterial embolization instead of surgery.

Review of Myositis Ossificans (골화성 근염에 대한 고찰)

  • Bae Sung-Soo;Park Rae-Joon;Han Dong-Uk
    • The Journal of Korean Physical Therapy
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    • v.12 no.2
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    • pp.255-265
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    • 2000
  • The term 'myositis ossificans' encompasses four categories of clinicopathological disorders. The first, myositis ossificans progressive(fibrodysplasia ossificans progressive), is a rare genetic disease characterized by progressive heterotopic ossification involving skeletal muscle, tendon, ligaments, and fascia, with congenital malformation of the great toes, and usually microdactyly, monophalangism, and mal formed proximal phalanges. with valgus deformity of metatarsophalangeal joint. The ossification begins shortly after birth and may contribute to the patient's death. The second, heterotopic ossificans, can occur in patients with neuromuscular and chronic diseases such as paraplegia, poliomyelitis, polymyositis, bum, tetanus, and infection. But the lesions in these cases often lack the typical histologic features of myositis ossificans. The third, myositis ossificans traumatica, is the most common; it develops in response to soft tissue trauma such as a single severe injury, minor repetitive injures, fracture, joint dislocation, stab wound, or surgical incision. The forth, nontraumatic myositis ossificans, also designated :pseudomalignant osseous tumors of extraskeletal soft tissues' and 'psedomalignant myositis ossificans', occurs in persons repeated small mechanical injures or nonmechanical soft tissue injuries due to local ischemia, inflammation. or other factors cannot be ruled out in such cases.

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Maternal Age and Infant Mortality in Korea (산모 연령과 영아 사망과의 관련성 연구)

  • Hong, Jae-Seok
    • Journal of Digital Convergence
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    • v.14 no.9
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    • pp.379-387
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    • 2016
  • The purpose of this study was to examine the relationship between maternal age and infant mortality in Korea(n=617,867). Data of Korean vital statistics linked National Infant Mortality Survey conducted on births in 1999 were used in this study. The odds ratios (ORs) of infant death by maternal age were estimated with the multiple logistic regression model, adjusting for gender, plurality, birth order, low birth weight, and congenital malformation. After adjusting for the relevant variables, the odds of infant death in the group of maternal age with less than 20 years(Odds ratio [OR], 5.29, 95% confidence interval [CI], 3.51-7.98), 20-24 years(OR, 1.44, 95% CI, 1.23-1.69), 35-39 years(OR, 1.28, 95% CI, 1.11-1.46), and more than 40 years(OR, 1.94, 95% CI, 1.53-2.45) was higher than that of reference group(25-29 years). In conclusion, the higher infant mortality in the group of maternal age with less than 25 years and more than 35 years in Korea appears to be due to higher proportion of low birth weight and pre-term birth. Prenatal care intervention programs which tackle biological factors for advanced maternal age and address socio-economic problems and social stigma for early maternal age should be devised.

Abberant Root Morphology in the Permanent First Molars : Case Reports (제1대구치에서 관찰되는 비정형적 치근형태에 대한 증례보고)

  • Lee, Eunkyoung;Kim, Youngjin;Kim, Hyunjung;Nam, Soonhyeun
    • Journal of the korean academy of Pediatric Dentistry
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    • v.42 no.2
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    • pp.172-179
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    • 2015
  • The developmental mechanism of root formation is a complex process. Hereditary and environmental factors may affect the morphology of the developing root. A total of 12 cases was presented with permanent first molars with abberant root morphology. Clinically, these teeth appeared as a normal crown. However, radiographically, the root was slender, twisted and characterized by irregular lengths. In addition, root trunk length was shorter and pulp chamber was obliterated. In these cases, periapical radiolucency and loss of lamina dura were often observed. In 6 cases, an abnormal root of the primary second molars were also present, as well as root malformation of permanent first molars. In 3 cases, permanent central incisors also had a dysmorphic crown. These cases almost all had medical history, such as premature birth, brain infection or congenital heart disease in infants. The present paper describes cases of permanent first molars with an abnormal root that are rarely reported in literature. This case may intensify the variation in the permanent first molar and is intended to reinforce the clinician's awareness of rare morphology of the roots.

Treatment of Pulmonary Sequestration with Thoracoscopic Approach (폐분리증의 치료와 흉강경적 절제술의 경험)

  • Cho, Min-Jeng;Kim, Tae-Hoon;Kim, Dae-Yeon;Kim, Seong-Chul;Kim, In-Koo
    • Advances in pediatric surgery
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    • v.16 no.2
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    • pp.154-161
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    • 2010
  • Pulmonary sequestration (PS) is a rare congenital malformation of the lower respiratory tract. The anomaly is characterized by absence of communication with the tracheobronchial tree and isolated blood supply from an anomalous systemic vessels. With the utilization of antenatal ultrasound, the diagnosis of asymptomatic neonatal PS has increased. Treatment options include observation, arterial embolization and surgical resection. The aim of the present study is to review the clinical course of PS and to share our experience with thoracoscopic resection. A total of 96 patients with PS were treated at Asan Children's Hospital between 1999 and 2010. The diagnosis of PS was established by CT in the cases managed by observation or embolization, and by tissue pathology in the surgical cases. Medical records and radiographic images were retrospectively reviewed. Thirty-nine patients were managed by embolization and 30 patients by surgery. The remaining 27 patients have been under observation without any procedures. Among 27 observation patients, 1 patient regressed completely and 10 patients were lost to follow up. Of the 39 embolizations patients, 2 had their lesion regress and sepsis was suspected after embolization. In 1 patient, the microcoil migrated to the iliac artery during the embolization procedure, and another patient developed renal abscess caused by renal artery embolization. Among 30 surgical cases, resection by thoracotomy was performed in 27 at the Department of Thoracic Surgery, and thoracoscopic resection in 3 at the Division of Pediatric Sugery. Only one wound complication ocurred. We conclud that surgical excision should be recommended for pulmonary sequestration, whether the sequestration is symptomatic or not because of the risk of infection, the low rate of natural regress, poor compliance, severe complications after embolization, and to exclude other pathology. In summary, thoracoscopic resection of the pulmonary sequestration is feasible, efficacious, safe and cosmetically superior even in neonatal period.

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