• Title/Summary/Keyword: Congenital

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DENTAL TREATMENT IN A PATIENT WITH PIERRE ROBIN SYNDROME UNDER GENERAL ANESTHESIA : A CASE REPORT (피에르 로빈 증후군 환아의 전신마취 하 치아우식 치료 증례 보고)

  • Ryu, Jiyeon;Shin, Teo Jeon;Hyun, Hong-Keun;Kim, Young Jae;Kim, Jung-Wook;Jang, Ki-Taeg;Kim, Chong-Chul;Lee, Sang-Hoon
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.12 no.2
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    • pp.87-91
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    • 2016
  • Pierre Robin syndrome (PRS) is characterized by the triad of congenital mandibular hypoplasia, glossoptosis and cleft palate. Infant PRS patients are frequently suffering from upper airway obstruction, gastroesophageal reflux and growth retardation caused by above mentioned problems. We report a dental caries treatment of 3-year old girl with Pierre Robin syndrome with multiple caries. The cause of multiple caries was mainly presumed as patient's eating habit caused by her general condition. She had some feeding problems and had history of gastric tube. She was still using milk bottle and took more than an hour to finish a meal. The treatment was performed under general anesthesia considering patient's condition; mild autism, poor cooperation and respiratory problem due to micrognathia. Severely affected upper incisors were treated with pulp treatment and restored with zirconia crown for esthetic purpose. Lower incisors were treated with pulp treatment and restored with composite resin. Upper right first primary molar was restored with stainless steel crown and other primary molars were treated with composite resin. There were no postoperative complications. According to her parents, the patient's compliance to oral hygiene management was greatly improved after the treatment since she was very pleased with the esthetic result and highly motivated by her looks. The treatment without sedation or general anesthesia would be possible once the airway is improved as the mandible grows.

The Effect of Lip Adhesion in Unilateral Complete Cleft Lip (일측성 완전구순열에서 구순접합술의 효과)

  • Ryu Sun-Youl;Kim Tae-Hee;Hwang Ung;Kook Min-Suk;Kim Sun-Kook;Han Chang-Hun
    • Korean Journal of Cleft Lip And Palate
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    • v.7 no.1
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    • pp.1-16
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    • 2004
  • In the recent time, early correction of the congenital anomalies has become the focus of contemporary cleft lip care, The reason of that is mostly psychologic factor of children are respected, Although the propound object of lip adhesion is not esthetic improvement, that able to satisfy sufficiently parents of cleft children, In the unilateral or bilateral clefts, a preliminary lip adhesion has been advocated as a mean of narrowing the cleft at an early phase, of improving the nasal contour, of molding the alveolar arch, and of easing and improving the result of a definitive lip repair. The present study was earned out to investigate the effect of lip adhesion. We performed the Millard's high-half underminded adhesion and Seibert's lip adhesion followed by modified Millard's cheiloplasty for five infants had unilateral complete cleft lip. The lip adhesion reduced the actual deformity by molding the maxillary alveolar segments into better relationship and allows a easy cheiloplasty so that led to more perfect final lip result, Both Millard's high-half underminded adhesion and Seibert's lip adhesion were available methods to adhere a wide cleft lip, Especially, Seibert's lip adhesion had more advantages such as enhancement of the force of adhesion, correction of the deviated columella and acquirement of the esthetic upper lip continuity. These results suggest that the lip adhesion followed by cheiloplasty for wide unilateral complete cleft lip patients provide more favorable final result by molding the maxillary alveolar segments into better relationship.

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A Case of Bronchial Artery Aneurysm Presenting with Massive Hemoptysis (대량 객혈이 발생된 기관지 동맥류 1예)

  • Choi, Hyo-Jin;Ok, Kyung-Sun;Jung, Sung-Mo;Lee, Young-Min;Kang, Yun-Kyung;Jin, Jae-Youg;Jin, Sung-Lim;Lee, Hyuk-Pyo;Choi, Soo-Jeon;Yum, Ho-Kee
    • Tuberculosis and Respiratory Diseases
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    • v.52 no.1
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    • pp.86-91
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    • 2002
  • A bronchial artery aneurysm(BAA) is rare, and has an unclear etiology. However, it may be caused by congenital abnormalities and acquired diseases like as bronchiectasis, tuberculosis, and other infections. The pathogenesis of a bronchial artery dilatation and the formation of an aneurysm results in an increase in the systemic blood flow to the chronic inflammatory pathologic lungs such as bronchiectasis or tuberculosis. It can be divided into the mediastinal and intrapulmonary BAA according to their location. The most common symptom is hemoptysis. Chest computed tomography and bronchial artery angiography may be used for a diagnosis. Treatment is mainly by a surgical resection of the aneurysmal artery. However, when patient is unstable due to massive hemoptysis or recurrent hemoptysis, bronchial artery embolization is useful. Here, we experienced a case of a bronchial artery aneurysm presenting as a massive hemoptysis.

MULTIPLE ANKYLOSIS ON MAXILLARY AND MANDIBULAR PRIMARY MOLARS WITHOUT PERMANENT SUCCESSOR (계승치의 결손을 동반한 상, 하악 유구치의 다발성 유착에 대한 증례보고)

  • Jung, Hwi-Hoon;Choi, Hyung-Jun;Kim, Seong-Oh;Choi, Byung-Jai;Lee, Jae-Ho
    • Journal of the korean academy of Pediatric Dentistry
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    • v.32 no.3
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    • pp.403-408
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    • 2005
  • Ankylosis is defined as a fusion of cementum or dentine with alveolar bone. Due to the loss of the periodontal ligament on the ankylotic area, the tooth is incapable of continued eruption and hence is unable to follow the normal vertical development of the neighboring teeth and alveolar process. A 6-year-old female was referred to the Dept. of Pediatric Dentistry for ankylosis of primary molars and congenital missing of permanent premolars on both jaws. She had neither specific past medical history nor trauma and infection history in oral and maxillofacial region. Radiographic finding is that the maxillary primary molars were the early onset of ankylosis and had fast root resorption rate. However the mandibular primary molars were ankylosed later and being resorbed slower than maxillary primary molars. The object of treating this case is to maintain the proper alveolar bone growth and retention of deciduous molars. The point of managing this case is as follows: Proper treatment (observation, restoration, or extraction) should be established after thorough consideration of the time of onset, the root resorption rate, progression of infraocclusion and the development of alveolar bone support. We should consider the timing of extraction of the ankylosed teeth without problem of neighbouring alveolar bone growth and tilting of adjacent teeth in the view of growth spurt. Early diagnosis is important to avoid many of the complications with infraoccluded primary molars.

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Physiologic state and behavioral response to sponge bathing in premature infants (스폰지 목욕에 대한 미숙아의 생리적상태 및 행동반응)

  • Lee Hae Kyung;Hong Kyung Ja;Nam Eun Sook;Lee Young Hee;Jung Eun Ja
    • Child Health Nursing Research
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    • v.6 no.1
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    • pp.32-50
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    • 2000
  • A descriptive exploratory design was used in this study to evaluate the effects of sponge bathing on physiological(heart rate, heart period, vagal tone, oxygen saturation, respiration) and behavioral responses in newly born 40 preterm infants from intensive care unit of S University Hospital in Seoul. Data has been collected from October, 1997 to March, 1999. The infants were between 27-33 weeks gestational age at birth, and were free of congenital defects. The subjects entered the protocol when they were medically stable (determined by initiation of feeding and discontinuation of all respiratory support) but still receiving neonatal intensive care. The infants' physiologic parameters were recorded a 10 - minute before, during, and after bathing. Continuous heart rate data were recorded on a notebook computer from the infant's EKG monitor. The data were digitized off-line on software(developed by Lee and Chang in Wavelet program) which detected the peak of the R wave for each heart beat and quantified sequential R-R intervals in msec(i.e. heart periods). Heart period data were edited to remove movement artifact. Heart period data were quantified as : 1) mean heart period; 2) vagal tone. Vagal tone was quantitfied with a noninvasive measure developed by Porges(1985) in Mxedit software. To determine behavioral status, tools were developed by Scafidi et al(1990) were used. Collected data were analyzed with the SPSS program using paried t-test, ANOVA, and Pearson correlation. The result were as follow. 1. The results of the ANOVAs indicated that vagal tone were signifcantly lower during bathing than baseline and post-bathing. There were significant differences in heart period and heart rate levels across the bathing. But the mean oxygen saturations and respirations were no differences. Also, there were no significant differences on behavioral sign, motor activity, behavioral distress, weight changes, morbidity, and hospitalization period. 2. To evaluate the relation between vagal tone and subsequent parameters, the two groups (the high group had 19 subjects and low group had 21subjects) were divided by the mean baseline vagal tone. Vagal tone measured prior to bathing were significantly associated with respiration before bathing, vagal tone during bathing, and the magnitude of change in both vagal tone. But, other subsequent reactivities were no differences in two groups. 3. Correlations were also calculated between vagal tone and the subsequent physiological reactivities from baseline through after- bathing. Correlations were significant between baseline vagal tone and baseline heart rate, between baseline vagal tone and baseline heart period, between baseline vagal tone and oxygen saturation after bathing. In summary, the bathing in this study showed a stressful stimulus on premature infants through there was significance in the physiological parameters. In addition, our study represents the documentation that vagal tone reactivity in response to clearly defined external stimulation provides an index of infant's status.

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Role of Trophobolast in Implantation and Placenta Development (착상 및 태반 발달과정에 따른 영양막세포의 역할)

  • Kim, Gi-Jin
    • Clinical and Experimental Reproductive Medicine
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    • v.37 no.3
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    • pp.181-189
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    • 2010
  • The placenta, which is a temporary organ derived from the fetus during pregnancy, is critical to support fetus development via optimal regulation between mother and fetus. Trophoblast as a major cell population of the placenta is one of the earliest to differentiate and shows an extensive proliferation or/and differentiation up to the formation of the placenta. The role of the trophoblast show dynamic changes from early embryo implantation to placentation during pregnancy. Implantation of the blastocyst into the endometrium of the maternal uterus is mediated by invasion of the differentiated trophoblast (e.g. syncytiotrophoblast) from the trophectoderm. During pregnancy, the unique role of the trophoblast is to invasion, eroding, and metastasizing in the placenta as well as to ensure appropriate bidirectional nutrient or waste flow required for growth and maturation of the embryo. The dysfunction of the trophoblast during pregnancy can result in several gynecological diseases including preeclampsia and congenital malformation in neonatal medicine. Therefore, trophoblasts act as a conclusive factor in placental and fetal development. This brief review outlines the classification of trophoblast and its function in the placenta during pregnancy. Also, we introduce the latest research in trophoblast for implantation and the placenta development, and the application potential of trophoblast for infertility and obstetrical diseases.

Mycobacterium abscessus Lung Disease in a Patient with Kartagener Syndrome

  • Kim, Jung Hoon;Song, Won Jun;Jun, Ji Eun;Ryu, Duck Hyun;Lee, Ji Eun;Jeong, Ho Jung;Jeong, Suk Hyeon;Kang, Hyung Koo;Kim, Jung Soo;Lee, Hyun;Chon, Hae Ri;Jeon, Kyeongman;Kim, Dohun;Kim, Jhingook;Koh, Won-Jung
    • Tuberculosis and Respiratory Diseases
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    • v.77 no.3
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    • pp.136-140
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    • 2014
  • Primary ciliary dyskinesia (PCD) is characterized by the congenital impairment of mucociliary clearance. When accompanied by situs inversus, chronic sinusitis and bronchiectasis, PCD is known as Kartagener syndrome. The main consequence of impaired ciliary function is a reduced mucus clearance from the lungs, and susceptibility to chronic respiratory infections due to opportunistic pathogens, including nontuberculous mycobacteria (NTM). There has been no report of NTM lung disease combined with Kartagener syndrome in Korea. Here, we report an adult patient with Kartagener syndrome complicated with Mycobacterium abscessus lung disease. A 37-year-old female presented to our hospital with chronic cough and sputum. She was ultimately diagnosed with M. abscessus lung disease and Kartagener syndrome. M. abscessus was repeatedly isolated from sputum specimens collected from the patient, despite prolonged antibiotic treatment. The patient's condition improved and negative sputum culture conversion was achieved after sequential bilateral pulmonary resection.

MRI of Hydrosyringomyelia Combined to Hydrocephalus and Occipital Dysplasia in a Dog (개에서 뇌수두증과 후두골 이형성증을 동반한 척수공동증의 자기공명영상학적 평가 1례)

  • Choi Chi-Bong;Bae Chun-Sik;Kim Hwi-Yool
    • Journal of Life Science
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    • v.15 no.4 s.71
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    • pp.664-667
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    • 2005
  • Hydrosyringomyelia is a dilation of the spinal cord central canal. In human it may be caused by congenital malformations such as Dandy-Walker syndrome and Chiari malformations or may be acquired as a result of infection, trauma or neoplasia. Hydrocephalus is an excessive accumulation of cerebrospinal fluid within the ventricles and occipital dysplasia is the dorsal extension of the foramen magnum. Hydrosyringomyelia and hydrocephalus can be confirmed by computed tomography or magnetic resonance imaging (MRI). A 3-year-old male maltese was presented with a history of long-term seizure. Blood examination was all unremarkable. On rostrodorsal-caudoventral oblique radiograph of the skull showed severe occipital dysplasia. On brain sonography through the persistent fontanelle, severe lateral ventriculomegaly was revealed. MRI examination revealed hydrocephalus and hydrosyringomyelia. Diuretic therapy didn't reduce clinical symptoms and surgical decompression was conducted. The dog responded well with ventriculo-peritoneal shunting. MRI is the most superior modality to diagnose hydrocephalus and hydrosyringomyelia, to plan therapy and to determine the prognosis.

Venous Flaps Applied for New Clinical Indications with using Various Methods (새로운 적응증에 다양한 방법으로 실시한 정맥피판)

  • Kim, Nam Gyun;Choi, Jae Hoon;Choi, Tae Hyun;Lee, Kyung Suk;Kim, Jun Sik;Lee, Hyuk Gu
    • Archives of Plastic Surgery
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    • v.34 no.1
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    • pp.52-59
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    • 2007
  • Purpose: The venous flap is used as an alternative method to the standard free flap for the management of small and thin soft tissue defects. Especially, the venous flap has advantages of being thin, easy harvest and various donor sites, as well as it having lower morbidity. Yet their clinical applications have been limited by their unstable postoperative course and also by their complications such as partial necrosis. The aim of this study is to extend the clinical indications of venous flaps with using various methods. Methods: From May 2005 to March 2006, total of 19 patients(21 cases) underwent various venous flaps for soft tissue defects as a result of trauma(15 cases), facial skin cancer(3 cases), chronic ulcer(1 case) and surgical wound for congenital anomaly(2 cases). The arterialized venous free flap were applied in 18 cases, the pure venous free flap was applied in 1 case and the pure venous pedicled flap were applied in 2 cases. Among them, two flow-through arterialized venous free flaps were applied that used the great saphenous vein to reconstructed major arteries as well as the injured skin and soft tissues in the arm. All the flap were harvested from the volar wrist(11 cases), dorsum of foot(5 cases), thenar(2 cases), and medial thigh(3 cases). Results: The sizes of the flap ranged from $0.75cm^2$ to $264cm^2$(mean size: $40.06cm^2$). The follow-up period ranged from two to twelve months. In the majority of cases, we obtained satisfying results, which was the excellent reconstruction of skin and soft tissue defects and especially in the case of limb salvage, replantation and cancer reconstruction. However, there were 5 cases of partial necrosis and 2 cases of complete failure. The donor sites were closed primarily in 7 cases and wound closure with skin graft were in 14 cases. Conclusion: We conclude that the venous flap will not only be useful for reconstruction of small defect in the hand and foot, but also be useful for various other clinical indications.

Prenatal Diagnosis of Accompanying Alveolar Cleft and Cleft Palate in Fetuses with Cleft Lip Using Prenatal 3D Sonographic Identification and Antenatal Counseling (구순열 태아에서 3D 산전 초음파를 이용한 치조열 및 구개열의 동반 유무 진단 및 산전상담)

  • Koh, Kyung Suck;Kim, Hoon;Choi, Jong Woo;Won, Hye Sung;Kim, Sun Kwon
    • Archives of Plastic Surgery
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    • v.34 no.2
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    • pp.181-185
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    • 2007
  • Purpose: Cleft lip and/or palate is the most common congenital facial anomaly whose incidence is about 1 in 500~1000 live births. As this anomaly may be associated with the serious chromosomal anomalies or the multiple organ abnormalities resulting in the fetal loss or perinatal maternal morbidity and mortality, careful prenatal counseling with early and accurate detection is important. Although conventional prenatal ultrasound(US) examination in midterm pregnancy has been applied for screening of cleft lip, there are definite limitations in the diagnosis of accompanying cleft palate or alveolar cleft. We applied high-resolution 3D US along the serial axial, coronal and sagittal plane so that we could diagnose the cleft palate and/or alveolar cleft in fetuses with cleft lip. Methods: From May 2005 to September 2005, 20 fetuses with cleft lip were examined with prenatal 3D US. Average maternal age was 28.8 years old(24-35 years old), and average gestational age was 24.8 weeks(17.6 to 34.2 weeks). Consecutive axial, coronal and sagittal multislice view were obtained via prenatal 3D US examination and diagnosis of cleft palate and/or alveolar cleft in cleft lip fetuses was followed. Results: With noninvasive and safe prenatal 3D US examination, 17 of 20 cleft lip fetuses were demonstrated to have cleft palate and/or alveolar cleft. Prenatal counseling according to the result was made. Conclusion: Existing prenatal US examination is suitable for screening the cleft lip fetuses but has limitation in identifying the related existence of cleft palate and/ or alveolar cleft. Authors verify the presence of cleft palate and/or alveolar cleft acquiring the successive multislice axial, coronal, and sagittal view with prenatal 3D US examination. Therefore, prenatal 3D US examination could be regarded as a noninvasive and secure screening modality in fetuses with cleft lip for confirming whether cleft palate and/or alveolar cleft is accompanied.