• The Korean Journal of Nuclear Medicine
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• v.14 no.1
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• pp.37-44
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• 1980

The usefulness of single, random measurements of serum FSH and LH in the diagnosis of primary amenorrhea by radioimmunoassay was investigated. The 16 patients were divided into 3 groups by the level of serum FSH and LH. The first group with increased level of serum FSH and LH is five patients, all of these are related to the acquired or congenital abnormality of the ovary. Further studies indicated include buccal smear, chromosome analysis, gynecography and laparosocopy. The second group with normal serum FSH and LH is nine patients, four patients of these are related to the developmental anomaly of the Mullerian duct and five patients are undo etermined origin. Further studies indicated include laparoscopy and gynecography. The third group with decreased serum FSH and normal or decreased serum LH is two patients, one of these is related to the pituitary function, isolated FSH deficiency, the other is undetermined origin. Further studies indicated include the pituitary function test, LH-RH stimulation test, skull radiography. Determination of serum FSH and LH levels does not permit a specific etiologic diagnosis of primary amenorrhea. However the serum levels of FSH and LH can be used to differentiate the principal area of the investigation and can be of assistance in choosing more specific testing procedures.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.299-305
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• 2007

Herlyn-Werner-Wunderlich syndrome(HWWs) is a rare variant of Mullerian ductal anomalies characterized by the presence of a hemivaginal septum, a didelphic uterus, and ipsilateral renal agenesis. It usually presents after menarche with progressive pelvic pain, and palpable mass due to hemihematocolpos. If a cystic mass is detected behind the urinary bladder in children, in association with the absence of a kidney, the diagnosis of uterus didelphys with imperforate vagina and hydrocolpos should be considered. When renal agenesis is found in asymptomatic children, the small size and the tubular shape of the uterus makes it almost impossible to evaluate uterine anomalies, so follow-up should be performed until the end of puberty. Appropriate preoperative diagnosis and treatment will prevent unnecessary procedures and offer relief of symptoms. We report one case of didelphic uterus with blind hemivagina and ipsilateral renal agenesis with biopsy- proven thin glomerular basement membrane disease which is not related to the above syndrome.

• Journal of the Korean Society of Radiology
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• v.83 no.4
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• pp.846-860
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• 2022

Persistent left superior vena cava (PLSVC) is a rare congenital, thoracic, and vascular anomaly. Although PLSVCs generally do not have a hemodynamic effect, several types of PLSVC and some cardiac anomalies may manifest with clinical symptoms. The presence of PLSVC can render catheterization via left subclavian access difficult when placing a pacemaker or central venous catheter. As such, recognizing a PLSVC that is typically incidentally discovered can prevent complications such as vascular injury. Differentiating vessels found in a similar location as PLSVC is necessary when performing thoracic vascular procedures. This pictorial essay explains the multi-detector CT findings of a PLSVC, and provides a summary of other blood vessels that require differentiation during thoracic vascular procedures.

• Advances in pediatric surgery
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• v.17 no.2
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• pp.154-161
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• 2011

Tracheoesophageal fistula without esophageal atresia (H-type TEF) is a congenital anomaly that is characterized by a fistula between the posterior wall of the trachea and the anterior wall of the esophagus, not accompanied by esophageal atresia. The purpose of this study is to investigate the clinical characteristics, diagnostic time, the side of cervical approach and short term result after surgery by searching medical records of patients treated for H-type TEF. The search was done at University of Ulsan, Department of Pediatric Surgery of Asan Medical Center, and the total number of patients from May 1989 to December 2010 was 9 with M:F ratio of 1:2. The median gestational age was $39^{+6}$ ($32^{+6}{\sim}41^{+0}$) wks. Seven out of nine patients were born at term and the other two were born premature. The clinical presentation was aspiration pneumonia, difficulty in feeding, chronic cough, vomiting, abdominal distension and growth retardation. The symptoms presented right after birth. The diagnosis was made with esophagography and the median time of diagnosis was 52 days of life. The majority of surgical corrections were performed within two weeks of diagnosis (median; 15d, range; 1d - 6m). Six patients had associated anomalies, and cardiac anomalies were most common. The cervical approach was utilized in all cases (right 2, left 7). Transient vocal cord palsy and minor esophageal leakage complicated two cases. Although the diagnosis of H-type TEF was difficult and often delayed, we had a good short term result. The left cervical approach was preferred.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.189-196
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• 2003

Purpose : Ureteral duplication is a common urologic anomaly and early diagnosis may prevent irreversible renal damage. We thus aimed to evaluate the benefits of prenatal sonography in early detection. Methods : We retrospectively studied 55 children with duplicated systems who were admitted to Severance Hospital, Yonsei University, Seoul, Korea from January 1998 to July 2003. Results : The mean age at diagnosis was 2.3 years old. 89% of the children had complete duplicated ureter. 47% of the children visited the hospital with an initial complaint of abnormal prenatal sonographic findings. Among these patients, 70.8% had DMSA defects. The mean differential renal function(DRF) in the DMSA scan was 48.2% in the group diagnosed prenatally. In the postnatally-diagnosed group, DMSA defects were found in 67.7% patients and the mean DRF was 45.5%. Conclusion : In comparison with the past studies, the mean age at diagnosis is becoming younger, and the proportion of abnormal prenatal sonography as an initial complaint larger. Other clinical manifestations were similar. The difference of the renal damage between the prenatally diagnosed group and the postnatally diagnosed group was not statistically significant. A multi-center study may help to prove the importance of prenatal sonography in early diagnosis and treatment of ureteral duplication. (J Korean Soc Pediatr Nephrol 2003;7:189-196)

• Korean Journal of Head & Neck Oncology
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• v.31 no.2
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• pp.74-77
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• 2015

A lingual thyroglossal duct cyst(LTGDC) is a rare congenital anomaly that account for only 0.5% to 2% of total thyroglossal duct cyst. LTGDC is frequently associated with respiratory problem in infants and pharyngeal foreign body sensation or dysphagia in adults. Because of its location and characteristics, lingual thyroid, dermoid cyst, and vallecular cyst should be included in differential diagnosis. Standard treatment for thyroglossal duct cyst is sistrunk's operation, but in terms of LTGDC, because of its location and cosmetic reasons, different kinds of treatments such as electrical cauterization, $CO_2$ laser, Robort surgery via transoral approach have been introduced. Recently authors encountered 21 years old woman with LTGDC and the mass was removed successfully via transoral approach using $CO_2$ laser. We report the clinical course with review of the literature.

• Journal of Chest Surgery
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• v.35 no.8
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• pp.616-620
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• 2002

Tracheal bronchus is a aberrant, accessory or ectopic bronchus arising almost invariably from the right lateral wall of the trachea and nay be related to inflammatory conditions affecting the lung, including recurrent pneumonia and bronchiectasis. Recently we experienced a case of tracheal bronchus associated with pulmonary actinomycosis. The 37-year-old male patient had suffered recurrent hemoptysis and had been medicated as a presumptive diagnosis of tuberculosis, but neither clinical nor radiologic improvement was not seen. Right upper lobectomy was performed and pulmonary actinomycosis was confirmed by the histologic examination. Postoperatively, the patient was medicated with penicillin and ampicillin for 3 months and completely recovered without any evidence of recurrence during the 6-month followup period.

• Journal of Korean Neurosurgical Society
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• v.61 no.1
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• pp.28-34
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• 2018

Objective : Duplication of the vertebral artery (VA) is a rare vascular variant. This paper describes the anatomy and embryological development of duplicated VAs and reviews the clinical significance. Methods : Computed tomography (CT) angiography was performed in 3386 patients (1880 females, 1506 males) between March 2014 and November 2015. We defined duplication of the VA as a condition in which the VA has two origins that fused at different levels of the neck. Results : Ten of the 3386 patients (0.295%) who received CT angiography had a dual origin of the VA; three on the left side, and seven on the right side. In all seven with right dual origin of the VA, both limbs of the VA origin originated from the right subclavian artery. In all three patients with left dual origin of the VA, both limbs of the VA originated from the left subclavian artery and aortic arch. In all 10 patients, the medial limb of the duplicated VA was located posteriorly and medially to the common carotid artery (CCA) and anteriorly and laterally to the vertebral transverse foramen. In two patients, the medial limb of the duplicated VA was located in close proximity to the CCA. In another two patients, the medial limb of the duplicated VA was located in close proximity to the CCA, carotid bifurcation, and proximal internal carotid artery. Conclusion : Although duplication of the VA is asymptomatic in most patients, clinicians should consider this anomaly during diagnosis and treatment.

• Journal of Chest Surgery
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• v.15 no.3
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• pp.346-354
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• 1982

A Complete transposition of the great arteries combined with V.S.D, A.S.D, dextrocardia, and P.S is a rare congenital anomaly. The patient was a 10 year-old female whose complaints were frequent URI, exertional dyspnea, and cyanosis at rest since birth. Cheat X-ray films showed Dextrocardia ; situs inversus, moderate cardiomegaly, and Characteristic egg-shape heart shadow. E.K.G, Echocardiography, Cardiac Catheterization, and Angio-Cardiography were performed. Open heart Surgery was done under diagnosis of d-TGA, Dextrocardia, V.S.D, A.S.D, and P.S. At the time of Operation, Dextroeardia, T.G.A, Secndum type A.S.D, A.S.D, and P.S. At the time of Operation, Dextroeardia, T.G.A, Secndum type A.S.D. ($2.0{\times}2.0cm$. in diameter), V.S.D. type II ($1.5cm{\times}1.5cm$ in diameter), and pulmonary valvular stenosis were noted. Mustard operation using pericardial Baffle in the atrium for T.G.A. was perforsned. Teflon patch graft for closure of V.S.D. through tricuspid orifice and pulmonary Valvulotomy through pulmonary arteriotomy were performed. The post-op, low cardiac output Syndrome and dysrhythmicawere developed till Postoperative day #7, so that was controlled by inotropic and antiarrhythmic agents. After that, patient's conditions were Uneventful.

• Annals of Clinical Neurophysiology
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• v.20 no.2
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• pp.85-88
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• 2018

Septo-optic dysplasia is a congenital anomaly with diverse phenotypes from normal to mixtures of visual abnormality, endocrine dysfunction, psychomotor retardations and epileptic seizures. It is characterized by optic atrophy, pituitary dysfunction and midline structure abnormalities in corpus callosum or septum pellucidum. Diagnosis of septo-optic dysplasia plus is made when cortical malformations accompanied. Here we report a middle-aged woman with septo-optic dysplasia plus having unilateral optic atrophy, agenesis of septum pellucidum and cortical malformations.