• Title/Summary/Keyword: malformation

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A Case of Child with Obstructive Sleep Apnea Syndrome Recurred after Adenotonsillectomy (편도 및 아데노이드 절제술 후 재발한 폐쇄성 수면 무호흡 증후군 소아 1례)

  • Kim, Cu-Rie;Kim, Dong-Soon;Seo, Hyun-Joo;Shin, Hong-Beom;Kim, Eui-Joong;Shim, Hyun-Joon;Ahn, Young-Min
    • Sleep Medicine and Psychophysiology
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    • v.15 no.2
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    • pp.94-99
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    • 2008
  • The most common cause of obstructive sleep apnea syndrome (OSAS) in childhood is adenotonsillar hypertrophy. Adenotonsillectomy improves the symptoms quite well in most cases. However, some patients could experience the OSAS again after adenotonsillectomy, who might have several risk factors such as incomplete operation, misdiagnosis, combined anatomical malformation, sinusitis or chronic allergic rhinitis, obesity, initial severe OSAS, and early onset OSAS. We report a case of 11-year-old obese boy who presented with snoring for several years. He was obese with body mass index (BMI) of $26.3kg/m^2$ and also found to have fatty liver by ultrasonogram. Initial polysomnography (PSG) showed that he met the criteria of severe OSAS with the apnea-hypopnea index (AHI) of 70.5. He underwent adenotonsillectomy and symptoms improved immediately. Four months later symptoms were relieved with AHI of 0, but 1 year after the adenotonsillectomy he started to complain snoring again and the subsequent PSG results showed that OSAS has relapsed with AHI of 43. Paranasal sinus X-ray and physical examination showed sinusitis and re-growth of adenoid. Obesity was proved not to be a contributing factor because his BMI decreased to normal range ($23.1kg/m^2$) after diet control and regular exercise. Also, liver transaminase was normalized and fatty liver was disappeared on follow-up abdominal ultrasonogram. After treatment of sinusitis, symptoms were relieved with decreased AHI (8.5). This case suggests that simple adenotonsillectomy might not be the end of OSAS treatment in childhood. Patients who had adenotonsillectomy should be followed by subsequent PSG if symptoms recur. It is also important to be aware of risk factors in the recurrent OSAS for the proper intervention according to the cause.

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Effect of bronchial artery embolization in the management of massive hemoptysis : factors influencing rebleeding (대량객혈 환자에서 기관지 동맥색전술의 효과 : 색전술후 재발의 원인과 예측인자)

  • Kim, Byeong Cheol;Kim, Jeong Mee;Kim, Yeon Soo;Kim, Seong Min;Choi, Wan Young;Lee, Kyeong Sang;Yang, Suck Cheol;Yoon, Ho Joo;Shin, Dong Ho;Park, Sung Soo;Lee, Jung Hee;Kim, Chang Soo;Seo, Heung Suk
    • Tuberculosis and Respiratory Diseases
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    • v.43 no.4
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    • pp.590-599
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    • 1996
  • Background : Bronchial artery embolization has been established as an effective means to control hemoptysis, especially in patients with decreased pulmonary function and those with advanced chronic obstructive pulmonary disease. We evaluated the effect of arterial embolization in immediate control of massive hemoptysis and investigated the clinical and angiographic characteristics and the course of patients with reccurrent hemoptysis after initial succeseful embolization. Another purpose of this study was to find predictive that cause rebleeding after bronchial artery embolization. Method : We reviewed 47 cases that underwent bronchial artery embolization for the management of massive hemoptysis, retrospectively. We analyzed angiographic findings in all cases before bronchial artery embolization and also reviewed the angiographic findings of patients that underwent additional bronchial artery embolization for the control of reccurrent hemoptysis to find the clauses of rebleeding. Results : 1) Underlying causes of hemoptysis were pulmonary tuberculosis(n=35), bronchiectasis(n=5), aspergilloma(n=2), lung cancer(n=2), pulmonary A-V malformation(n=1), and unknown cases(n=2). 2) Overal immediate success rate was 94%(n=44), an6 recurrence rate was 40%(n=19). 3) The prognostic factors such as bilaterality, systemic-pulmonary artery shunt, multiple feeding arteries and degree of neovascularity were not statistically correlated with rebleeding tendency (p value>0.05). 4) At additional bronchial artery embolization, Revealed recannalization of previous embolized arteries were 14/18cases(78%) and the presence of new deeding arteries was 8/18cases(44%). 5) The complications(31cases, 66%) such as fever, chest pain, cough, voiding difficulty, paralytic ileus, motor and sensory change of lower extremity, atelectasis and splenic infarction were occured. Conclusion : Recannalization of previous embolized arteries is the major cause of recurrence after bronchial artery embolization. Despite high recurrence rate of hemoptysis, bronchial artery embolization for management of massive hemoptysis is a effective and saute procedure in immediate bleeding control.

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Identification of a New Potyvirus, Keunjorong mosaic virus in Cynanchum wilfordii and C. auriculatum (큰조롱과 넓은잎 큰조롱에서 신종 포티바이러스(큰조롱모자이크바이러스)의 동정)

  • Lee, Joo-Hee;Park, Seok-Jin;Nam, Moon;Kim, Min-Ja;Lee, Jae-Bong;Sohn, Hyoung-Rac;Choi, Hong-Soo;Kim, Jeong-Soo;Lee, Jun-Seong;Moon, Jae-Sun;Lee, Su-Heon
    • Research in Plant Disease
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    • v.16 no.3
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    • pp.238-246
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    • 2010
  • In 2006 fall, a preliminary survey of viruses in two important medicinal plants, Cynanchum wilfordii and C. auriculatum, was conducted on the experimental fields at the Agricultural Research and Extension Services of Chungbuk province in Korea. On each experimental fields, percentage of virus infection was ranged from 20 to 80%, and especially an average of disease incidence propagated by roots was twice higher than that by seeds. The various symptoms were observed in Cynanchum spp. plants, such as mosaic, mottle, necrosis, yellowing, chlorotic spot and malformation etc. In electron microscopic examination of crude sap extracts, filamentous rod particles with 390-730 nm were observed in most samples. The virus particles were purified from the leaves of C. wilfordii with typical mosaic symptom, and the viral RNA was extracted from this sample containing 430-845 nm long filamentous rod. To identify the viruses, reverse transcription followed by PCR with random primers was carried out. The putative sequences of P3 and coat protein of potyvirus were obtained. From a BLAST of the two sequences, they showed 26-38% and 62-72% identities to potyviruses, respectively. In SDS-PAGE analysis, the subunit of coat protein was approximately 30.3 kDa, close to the coat protein of potyvirus. In bioassay with 21 species in 7 families, Chenopodium quinoa showed local lesion on inoculated leave and chlorotic spot on upper leave, but the others were not infected. RT-PCR detection using specific primer of C. wilfordii and C. auriculatum samples, all of 24 samples with virus symptom was positive, and five out of seven samples without virus symptom were also positive. On the basis of these data, the virus could be considered as a new member of potyvirus. We suggested that the name of the virus was Keunjorong mosaic virus (KjMV) after the common Korean name of C. wilfordii.

Imaging Findings of Pneumothorax Caused by Bronchial Cartilage Hypoplasia in a Dog (개에서 기관지 연골 저형성에 의해 발생한 기흉의 영상학적 진단 증례)

  • Kim, Su-yeon;Kim, Seong-soo;Lee, Jeo-soon;Yun, Soo-kyung;Oh, Hyun-jung;Shon, Jung-min;Kim, Bo-eun;Kim, Wan-hee;Yoon, Jung-hee;Choi, Min-cheol
    • Journal of Veterinary Clinics
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    • v.32 no.5
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    • pp.469-472
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    • 2015
  • A 10-year-old, castrated poodle dog presented with a cough for 2 weeks, and the cough initially developed since very young age. On radiographs, pneumothorax was noticed by characteristics of radiolucent area without pulmonary markings along the thoracic wall and diaphragm, retracted lung lobes from the thoracic wall and severely decreased volume of the left cranial lung lobe with disconnected bronchus. Computed tomography (CT) findings identified several pulmonary air-filled cysts and collapsed lung with abnormal shape and non-tapered end of bronchus, bronchioles at the accessory lobe and left cranial lobe. Also, pneumothorax, pneumomediastinum and subcutaneous emphysema were found. Imaging diagnosis was the spontaneous pneumothorax caused by ruptured emphysematous bullae associated with congenital bronchial cartilage abnormality or bronchial tree malformation. On surgery, hypoplasia of the left cranial lobe, right middle lobe, and accessory lobe with a bulla where air was leaking was identified. The accessory lobe was partially resected and bronchial cartilage hypoplasia was confirmed by histopathologic examination.

Surgical Experiences of Shone's Syndrome (숀 증후군의 외과적 치료)

  • Won, Tae-Hui;Lee, Jeong-Ryeol;Kim, Yong-Jin;No, Jun-Ryang
    • Journal of Chest Surgery
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    • v.30 no.9
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    • pp.862-868
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    • 1997
  • Shone's syndrome is a congenital cardiac malformation that consists of multiple levels of left heart obstruction including supravalvular mitral ring, congenital mitral stenosis(parachute mitral valve), subaortic stenosis, and coarctation of aorta. This syndrome is a very rare congenital anomaly and its prognosis is poor. We experienced 9 patients with Shone's syndrome between 1985 and 1994. There were 8 male and 1 female patients, and mean age was 33.0$\pm$31.0 months ranged from 2 months to 1 1 years. The congenital mitral, stenosis and coarctation of aorta existed in all patients and the supravalvular mitral ring and subaortic stenosis in 4 patients. Two patients had all four anatomic lesions. 3 patients underwent one stage total correction and the other 6 patients underwent two staged operation that was initial coarctoplasty with thoracotomy and later correction of intracardiac anomalies with median sternotomy. A third operation was performed in 2 patients. These procedures included reoperation for coarctation and replacement of mitral valve for persistent mitral stenosis. There was no operative death at the first operation but two operative deaths at the second operation. The cause of death in two cases was severe heart failure secondary to left ventricular hypoplasia. There was no operative death at the third operation. The seven survivors have beeli followed from 11 months to 12 years(mean follow-up 6.7 $\pm$ 3.6 years). There was no late death and the New York HeArt Association activity level was class I for all patients. We conclude that a food lone-term outcome can be expected by proper surgical treatment tailred to each individual's anatomy and pathophysiology although the operative mortality and morbidity of Shone's syndrome are high.

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The Effectiveness of Intravenous Immunoglobulin for Clinically Suspected Neonatal Sepsis (임상적으로 의심되는 신생아 패혈증에서 정맥용 면역 글로불린의 치료 효과)

  • Na, Hyun Jung;Kim, Ji Young;Lee, Gyeong Hoon;Lee, Jun Hwa;Choi, Eun Jin;Kim, Jin Kyung;Chung, Hai Lee;Kim, Woo Taek
    • Clinical and Experimental Pediatrics
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    • v.48 no.11
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    • pp.1187-1192
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    • 2005
  • Purpose : The purpose of this study is to determine the effectiveness of intravenous immunoglobuin (IVIG) administration in fullterm neonates having clinically suspected neonatal sepsis. Methods : Forty full-term neonates admitted to the neonatal intensive care unit with clinically suspected neonatal sepsis, who had at least two positive diagnostic criteria were enrolled. Twenty neonates were enrolled into the IVIG arm and 20 in the placebo arm. Neonates with a gestational age of less than 36 weeks and those with any major congenital malformation were excluded. The neonates were randomized to receive 1 g/kg of IVIG or equivalent amount of normal saline. The treatments including antibiotics and supportive care were administered. Results : The neonates in the therapy and placebo groups were comparable in terms of birth weight, gestational age, sex distribution, duration of antibiotics therapy and admission, elevation of serum IgG level, mortality rate, change of CBC, and serum level of acute phase reactants etc. Conclusion : Serum IgG values increased significantly 5 days after administration of IVIG in the IVIG-treated group and decreased significantly 5 days after administration of normal saline in the placebo group. However, there was no significant difference in the duration of antibiotics therapy and admission, or of mortality between the IVIG-treated and placebo groups. No adverse reactions to the IVIG infusions were noted during the study. Our preliminary observations suggest that the administration of 1 g/kg IVIG to neonates had some effect on augmentation of humural immune status in neonates with clinically suspected sepsis. But further study is needed to verify the benefit of IVIG infusion to neonatal sepsis.

Multicenter clinical study on birth weight and associated anomalies of single umbilical artery (단일 배꼽 동맥 환아의 출생체중과 동반기형에 관한 다기관 연구)

  • Bae, Su-Nam;Lim, Jae-Woo;Ko, Kyong-Og;Jin, Hyun-Seung;Kim, Min-Hee;Lee, Bo-Young;Kim, Chun-Soo;Kim, Eun-Ryoung;Park, Sang-Kee;Lee, Jung-Joo
    • Clinical and Experimental Pediatrics
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    • v.52 no.6
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    • pp.655-660
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    • 2009
  • Purpose : To compare birth weight between infants with a single umbilical artery (SUA) and normal infants, investigate the associated anomalies of infants with SUA and isolated SUA (no abnormality of external appearance on birth, except SUA), and determine the prognosis of infants with isolated SUA. Methods : Live-born infants with SUA (n=59) detected by physical examination from among 15,193 live births in seven university hospitals in Korea between January 1, 2004, to August 1, 2007, were reviewed retrospectively, with 236 normal infants serving as the control group. Results : A statistical difference was observed between the groups in birth weight and in vitro fertilization. The incidence of infants with SUA was 0.37%. Congenital malformations were observed in 21 infants with cardiovascular (n=15, 25.4%), gastrointestinal (n=2, 3.4%), genitourinary (n=9, 15.3%), neuromusculoskeletal (n=6, 10.2%), central nervous system (n=1, 1.7%), chromosomal (n=1, 1.7%), and other (n=3, 5.1%) abnormalities. There were 49 (83.1%) infants with isolated SUA in this study population; among them, the associated congenital malformations were cardiovascular (n=6, 12.2%) and genitourinary (n=6, 12.2%) abnormalities. Two infants with cyanotic heart disease were operated and four infants with acyanotic heart disease showed improvements without any treatment. Six infants with genitourinary abnormalities on renal ultrasound had mild hydronephrosis without further consequences. Conclusion : The incidence of structural abnormalities in the cardiovascular and genitourinary systems is high and the genitourinary anomalies associated with isolated SUA have relatively good prognosis.

The Efficacy of Speech and Language Therapy for Children with Speech and Language Delays according to the Eiologies (말 언어 지연을 보인 소아에서 원인에 따른 말 언어 치료의 효과)

  • Kim, June Bum;Suh, Jin-Soon;Kim, Young-Hoon;Chung, Seung-Yun;Lee, In-Goo;Whang, Kyung-Tai;Lee, Byung-Churl
    • Clinical and Experimental Pediatrics
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    • v.48 no.9
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    • pp.924-928
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    • 2005
  • Purpose : Treatment efficacy for children with speech and language delay has been the subject of considerable debate in recent years. We evaluated the clinical features of children with delayed speech and language and their prognoses according to their etiologies after 6 months of speech and language therapy. Methods : From January, 2000 to March, 2004, we retrospectively reviewed 56 children with speech and language delay who were administered speech and language therapy for 6 months in Uijongbu St. Mary's Hospital. Results : Of 56 cases, the proportion of developmental language disorder was 66.1 percent, structural malformation 19.6 percent, mental retardation 12.5 percent, hearing defect 1.8 percent. The ratio of male to female was 4.6 : 1 and the most frequent age group was over 47 months. The mean age of first spontaneous words with useful meaning was 15.9 months. The mean gestational age of the subjects was 39.8 weeks. The proportion of full-term infants was 96.4 percent and of premature infants was 3.6 percent. As for the birth order, the proportion of the first baby was 51.8 percent, the one of second babies it was 42.9 percent, and percent of third babies it was 7.1 percent. After 6 months of language intervention, 32.4 percent of patients with developmental language disorder showed normal linguistic development. All the patients with mental retardation showed sustained language and speech delay. As for the patients with structural malformations, five out of 11 patients showed normal linguistic development. Conclusion : The relatively advanced old age of majority of participants in this study suggests the necessity of screening test for language delay in this local community.

Impact of fetal diagnosis of congenital heart disease on parents (선천성 심질환의 산전 진단이 보호자의 임신 유지 결정에 미치는 영향)

  • Choi, Eun Young;Lee, Chang Hoon;Yoon, Myung Ja;Han, Eun Sook;Hong, Joon Suk;Jung, Yun Sook;Choi, Jung Yun
    • Clinical and Experimental Pediatrics
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    • v.49 no.10
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    • pp.1073-1078
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    • 2006
  • Purpose : This study was performed to assess how a fetal diagnosis of congenital heart disease affects parents, as regards pregnancy management and care of infants after birth. Methods : Database search to find out abnormal fetal echocardiography performed at Seoul National University Children's Hospital from July 1988 to June 2003 revealed 370 examinations. After excluding both arrhythmias without structural cardiac disease and multiple pregnancies, 299 pregnancies remained and this data formed the basis of this analysis. We retrospectively reviewed the medical records with special attention to pregnancy outcomes and also tried to find out factors influencing parental decisions on whether to continue or terminate pregnancy. Results : In this study, the mean gestation age at diagnosis was $28{\pm}6.0weeks$. The mean age of mothers was $30{\pm}3.9$ years old. Younger gestational ages at diagnosis(P=0.000), more severe grades of fetal heart disease(P=0.002) and younger mothers(P=0.014) correlated with terminations of pregnanies. But the grades of fetal status, the grades of associated anomaly, whether in-vitro-fertilization was carried out or not and numbers of previous children were not significant. Conclusion : This study found that the earlier gestational ages at diagnosis, younger maternal age and higher grades of fetal heart disease tended to lead parent to select abortions. Fetal echocardiographies were performed too late. Moreover Koreans have a biased view that malformation is a something incurable and a tragedy not only to oneself, but also to a family. So parents select terminations of pregnancy, even in curable cases. This is very unethical.

Complications of transcatheter closure of atrial septal defects using the amplatzer septal occluder (심방 중격 결손의 경피적 폐쇄술 후 발생한 합병증에 관한 연구)

  • Jea, Seo Jin;Kwon, Hyo Jin;Jang, Gi Young;Lee, Jae Young;Kim, Soo Jin;Son, Chang Sung;Lee, Joo Won
    • Clinical and Experimental Pediatrics
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    • v.51 no.4
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    • pp.401-408
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    • 2008
  • Purpose : Transcatheter closure of atrial septal defects (ASD) is currently established therapy as an alternative to surgery. But rarely, complications are reported in some studies. We report early and intermediate term complications associated with transcatheter closure of atrial septal defects using the Amplatzer septal occluder (ASO). Methods : From June 2003 to May 2006, 64 patients underwent transcatheter closure of secundum ASD or patent foramen ovale using the ASO. The ratio of male to female was 1:2.4, the median age was 17 years (range: 2.6-64 years) and their median weight was 47.5 kg (range: 2.6-64 kg). Results : The median diameter of ASD measured with transthoracic or transesophageal echocardiography was 15 mm (range: 6-28 mm), the median balloon stretched diameter was 18 mm (range: 6.5-34 mm), and the median size of device was 19.5 mm (range: 6-36 mm), was little difference with balloon stretched diameter. There were 10 cases of complications: arrhythmia (2), device malformation (2), aorta to right atrial fistula (1), hemolytic anemia (1), mitral valve encroachment (1), malposition (1), residual shunt (1), and inferior vena cava perforation (1). Conclusion : Transcatheter closure of ASD using ASO is effective and safe therapy. However, significant complications such as aorta to atrial fistula, atrial erosion, or device embolization can happen, so an appropriate selection of patient and device in relevance to size and anatomy of ASD is important for successful closure.