A procedure descried by Fontan and Baudet in 1971 successfully bypassed the right ventricle in Tricuspid Atresia patients, after then many modification of the Fontan operation had been described and applied to many cyanotic complex heart patients. Forty patients with a variety of cardiac malformation underwent the Fontan operation at Seoul National University hospital (September 1978 to June 1986). The age at operation ranged 2 months to 18 years. Each number of cases according to basic cardiac anomaly was as follows; 17 in Tricuspid Atresia, 17 in Univertricular Heart, 2 in Double outlet of Right Ventricle, 2 in Transposition of the Great Arteries and 2 in Criss-cross heart. Total mortality rate after the Fontan operation was 50%. There was only one late death (>30 days). Mortality rate under 4 years of age (67%) was higher than that between 4 and 18 years of age (40%). we observed a significantly higher mortality for patients who, in the immediate postoperative period, had central venous pressure greater than 25cm H2O. 45% among survivals did not require further medication. Although mortality rate after the Fontan operation is much higher than that in the foreign literature, operative mortality will decline with the increased expirence of surgeon and the effective patients selection.
Arteries ; Central Venous Pressure ; Crisscross Heart ; Fontan Procedure* ; Heart ; Heart Ventricles ; Humans ; Mortality ; Postoperative Period ; Seoul ; Tricuspid Atresia

Two case of a rare from of congenital ichthyosis in infants born to the same parents are presented. Typical features revealed of parchment like skin, ectropion of upper eyelids, and fixed semiflexion attitude of the upper limb. The skin lesion were followed by desquamation and appearance of normal looking skin from the the second day of life to a month. Diagnosis of collodion baby was established by clinical features, histopathological and laboratory studies. A brief feview of literatures were presented.
Collodion* ; Diagnosis ; Ectropion ; Eyelids ; Humans ; Ichthyosis ; Infant ; Parents ; Skin ; Upper Extremity

PURPOSE: The purpose of this study was to compare outcomes for surgical treatment with those for medical treatment of GB dyskinesia. METHODS: Retrospective analysis of medical records and telephone interviews of 67 patients diagnosed with GB dyskinesia was done at Pohang St. Mary's Hospital between January 2004 and December 2009. Group 1 (n=18) patients received laparoscopic cholecystectomy. Group 2 (n=49) patients received medical treatment. GB dyskinesia was the diagnosis if the patient had typical biliary colic symptoms without GB stones or other GI disease, and if the ejection fraction was less than 35% on Tc-99m-DISIDA scans. RESULTS: The average age of patients diagnosed with GB dyskinesia was 45.8 years old. The sex ratio was 15:52 (male:female). The average symptom duration was 25.4 days. All had RUQ and, or epigastric pain. There were no significant between group differences in age, sex ratio, symptom duration, symptoms, follow up period, and ejection fraction. In group 1, patient symptoms improved after treatment in 16 cases (88.9%); in group 2, patient symptoms improved in 19 cases (38.8%). Surgical treatment was significantly more effective than medical treatment. The reasons for choosing medical treatment were predominantly the preference of the doctors. CONCLUSION: Surgical treatment is a more effective treatment for GB dyskinesia than medical treatment. Therefore, laparoscopic cholecystectomy should be considered as the 1st line treatment of choice for GB dyskinesia.
Biliary Dyskinesia ; Cholecystectomy, Laparoscopic ; Colic ; Dyskinesias ; Follow-Up Studies ; Gallbladder ; Humans ; Interviews as Topic ; Medical Records ; Retrospective Studies ; Sex Ratio

We analyzed 20 cases of traumatic carotid cavernous fistula(CCF) during the recent 10 years The results are summarized as follows: 1) In 18 cases(90%), the clinical symptoms & signs of CCF occurred within 2 months after trauma. 2) The sites of fistulae were common in horizontal segment(40%) and at the junction(30%) between horizontal segment and posterior ascending segment of cavernous portion of internal carotid artery. 3) The main draining veins of CCF were the superior ophthalmic vein(90%) and the inferior petrosal sinus(70%). 4) The methods of treatment were occlusion of fistula with balloon(9 cases), occlusion of cavernous ICA with balloon(2 cases), ligation of cervical ICA with Poppen's clamp(4 cases) and trapping(2 cases). Two patients were not treated and another patient was healed spontaneously. 5) The frequency and severity of complication was significantly decreased in cases treated by detachable balloon occlusion than by direct cervical ICA ligation or trapping procedures. 6) The procedure using the self-sealed goldvalve balloon was simple, but had a risk of premature separation and premature deflation.
Balloon Occlusion ; Carotid Artery, Internal ; Fistula* ; Humans ; Ligation ; Veins

OBJECTIVES: Attention-deficit/hyperactivity disorder (ADHD) is a neurodevelopmental disorder showing many neuropsychological deficits. Many environmental risk factors have been thought to increase the risk for the disorder. We examined blood iron levels in children with ADHD and a control group to find an association between iron deficit and diagnosis, neuropsychological characteristics and clinical features. METHODS: An ADHD group (n=50) and control group (n=45) of children 6-12 years of age were recruited. Both groups were diagnosed by semi-structured interview, and they were evaluated using the Korean version of the ADHD Rating Scale (K-ARS), Korean version of IOWA Conner's Rating Scale (K-IOWA), intelligence quotient (IQ), and neurocognitive function tests (continuous performance test, children's color trails test, Stroop color-word test). Iron levels in blood were determined using the inductively coupled plasma mass spectrometry instrument. Independent t-test and correlation were used to determine the relation of blood iron levels with symptom ratings and neurocognitive function. Logistic regression was performed to determine the diagnostic value of blood iron levels. RESULTS: Blood iron levels were significantly lower in ADHD than in control and showed negative correlation with K-ARS and K-IOWA scores. Blood iron levels showed positive association with IQ and Stroop color-word test results and negative association with results of continuous performance testing. Low blood iron levels predicted the diagnosis of ADHD. CONCLUSION: Lower levels of blood iron were associated with ADHD symptom severity, IQ, and frontal lobe-mediated neurocognitive function. As blood iron levels may influence ADHD, measurement of iron levels in blood may be useful for evaluation of symptoms and neurocognitive function in ADHD.
Child* ; Diagnosis ; Humans ; Intelligence ; Iowa ; Iron* ; Logistic Models ; Mass Spectrometry ; Plasma ; Risk Factors

PURPOSE: Angiogeneisis is essential process for tumor growth and metastasis. Vascular endothelial growth factor (VEGF) is major regulator of angiogenesis. Recently, the incidence of papillary thyroid microcarcinoma (PTMC) increased because of development in diagnostic modality. Several recent reports have documented relationship of VEGF and papillary thyroid cancer. The aims of this study were to determine whether angiogenetic phenotype was changed or not changed and to evaluate the relationship between clinicopathologic features and VEGF, vascular endothelial growth factor receptor-1 (VEGFR-1), hypoxic induced factor-1alpha (HIF-1α) mRNA expression in PTMC. METHODS: VEGF, VEGFR-1, HIF-1α mRNA expression was examined by RT-PCR in 14 patients who had undergone thyroidectomy due to PTMC. The thyroid tumor tissue and adjacent normal thyroid tissue were collected in operation and preserved at -70℃ in RNA later solution. We evaluate the expression of VEGF, VEGFR-1, HIF-1α mRNA by RTPCR. The expression of mRNA was quantititated by densitometer and analyzed the relationship between clinicopathologic features and mRNA expression. RESULTS: Compared to normal tissues, in PTMC we observed higher expression of HIF-1α mRNA (P=0.024) and lower expression of VEGF mRNA (P=0.002). There was no difference in expression of VEGFR-1.The patients with nodal metastasis had higher expression of the VEGF mRNA in tumor tissues than those without nodal metastasis but not significantly. The VEGF mRNA of tumor tissues in patients with thyroid capsule invasion or not were expressed similarly. The lower expression of VEGF mRNA were observed more frequently in younger patients (<40). CONCLUSION: The expression of VEGF mRNA was lower in tumor tissue in spite of higher expression of HIF-1α mRNA. These results suggest that the reason for good prognosis and no progression to clinical cancer in PTMC was related to the unchanged angiogenic phenotype.
Humans ; Incidence ; Neoplasm Metastasis ; Phenotype ; Prognosis ; RNA ; RNA, Messenger* ; Thyroid Gland* ; Thyroid Neoplasms ; Thyroidectomy ; Vascular Endothelial Growth Factor A* ; Vascular Endothelial Growth Factor Receptor-1*

Myasthenia Gravis is a chronic disease of disputed etiology, possibly an auto-immune reaction to the moter end-plate, characterized by exacerbations and remissions, a rare disease entity in Korea. Myasthenia Gravis has offered many anesthetic problems because it affects respiratory muscles occasionally and bronchial secretion from preoperative anticholinesterase therapy. The chief concern is to ensure adequate respiration both during and after operation. During the year 1976-1982, we have experienced the anesthetic management of 8 patients with myasthenia gravis and thymectomy. From our experiences, we conclude that respiratory care and disuse of relaxants is the key to successful management.
Chronic Disease ; Humans ; Korea ; Myasthenia Gravis* ; Rare Diseases ; Respiration ; Respiratory Muscles ; Thymectomy

Alagille syndrome (AGS) is a rare autosomal dominant inherited disorder, with major clinical manifestations of bile duct paucity, cholestasis, cardiovascular anomaly, ophthalmic abnormalities, butterfly vertebrae, and dysmorphic facial appearance. It is caused by heterozygous mutations in JAG1 or NOTCH of the Notch signaling pathway presenting with variable phenotypic penetrance and involving multiple organ systems. The following case report describes a unique case of a 16-year-old female with AGS who presented with the primary complaint of renovascular hypertension. She had a medical history of ventricular septal defect and polycystic ovary syndrome. The patient had a dysmorphic facial appearance including frontal bossing, bulbous tip of the nose, a pointed chin with prognathism, and deeply set eyes with mild hypertelorism. Stenoocclusive changes of both renal arteries, celiac artery, lower part of the abdominal aorta, and left intracranial artery, along with absence of the left internal carotid artery were found on examination. Whole exome sequencing was performed and revealed a pathologic mutation of JAG1, leading to the diagnosis of AGS. Reverse phenotyping detected butterfly vertebrae and normal structure and function of the liver and gallbladder. While the representative symptom of AGS in most scenarios is a hepatic problem, in this case, the presenting clinical features were the vascular anomalies. Clinical manifestations of AGS are diverse, and this case demonstrates that renovascular hypertension might be in some cases a presenting symptom of AGS.

Purpose: The introduction of immune checkpoint inhibitors represents a major advance in the treatment of lung cancer, allowing sustained recovery in a significant proportion of patients. Nivolumab is a monoclonal anti–programmed death cell protein 1 antibody licensed for the treatment of locally advanced or metastatic non-small cell lung cancer (NSCLC) after prior chemotherapy. In this study, we describe the demographic and clinical outcomes of patients with advanced NSCLC treated with nivolumab in the Korean expanded access program. Materials and Methods: Previously treated patients with advanced non-squamous and squamous NSCLC patients received nivolumab at 3 mg/kg every 2 weeks up to 36 months. Efficacy data including investigator-assessed tumor response, progression data, survival, and safety data were collected. Results: Two hundred ninety-nine patients were treated across 36 Korean centers. The objective response rate and disease control rate were 18% and 49%, respectively; the median progression-free survival was 2.1 months (95% confidence interval [CI], 1.87 to 3.45), and the overall survival (OS) was 13.2 months (95% CI, 10.6 to 18.9). Patients with smoking history and patients who experienced immune-related adverse events showed a prolonged OS. Cox regression analysis identified smoking history, presence of immune-related adverse events as positive factors associated with OS, while liver metastasis was a negative factor associated with OS. The safety profile was generally comparable to previously reported data. Conclusion This real-world analysis supports the use of nivolumab for pretreated NSCLC patients, including those with an older age.