• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.2
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• pp.146-153
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• 2020

Purpose: Alpha-1 antitrypsin deficiency (A1ATD) in one of the most common genetic causes of liver disease in children. We aimed to analyze the clinical characteristics and outcomes of patients with A1ATD. Methods: This study included patients with A1ATD from five pediatric hepatology units. Demographics, clinical findings, genetics, and outcome of the patients were recorded (n=25). Results: Eight patients (32.0%) had homozygous PiZZ genotype while 17 (68.0%) had heterozygous genotype. Patients with PiZZ genotype had lower alpha-1 antitrypsin levels than patients with PiMZ genotype (37.6±7.7 mg/dL vs. 66.5±22.7 mg/dL, p=0.0001). Patients with PiZZ genotype were diagnosed earlier than patients with PiMZ genotype, but this was not significant (13±6.8 months vs. 23.7±30.1 months, p=0.192). Follow-up revealed the death of one patient (12.5%) with a homozygous mutation, and revealed that one patient had child A cirrhosis, five patients (62.5%) had chronic hepatitis, and one patient (12.5%) was asymptomatic. Nine of the 17 patients with a heterozygous mutation had chronic hepatitis (52.9%), two (11.7%) had child A cirrhosis, and six (35.2%) were asymptomatic. Overall, 18 (72%) of the 25 children had liver pathology in the long-term. Conclusion: Although prevalence is rare, patients with liver disorders should be checked for alpha-1 antitrypsin levels. Moreover, long-term follow-up is essential because most patients have a liver pathology.

• Journal of Korean Neurosurgical Society
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• v.50 no.3
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• pp.191-194
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• 2011

Objective : The aim of this study was to analyze the treatment outcome of patients with vein of Galen aneurysmal malformations (VGM). Methods : Clinical and angiographic data of six consecutive patients with VGM were retrospectively reviewed. VGMs were angiographically classified by Yasargil's method. Treatment outcomes were evaluated. Results : Mean age at initial treatment was $4.4{\pm}5.7$ months. Angiographic types of VGMs were type II in two patients and type III in four. Three patients had cardiac symptoms and the others were asymptomatic. Two patients were treated with transvenous embolization, three with transarterial embolization, and one was managed conservatively. Two patients died due to venous hypertension few days after transvenous approach. Of three patients who were transarterially embolized, one was completely occluded with Onyx and two were incompletely occluded. During the follow-up period (range, one to six years) two of three patients treated with transarterial approach were asymptomatic and the other showed mild symptoms. One patient who was managed conservatively showed normal performance. Conclusion : Transarterial embolization of VGMs may be better than transvenous approach in terms of the treatment outcome and complication. Further studies are needed because of the rarity of the disease and rapid advancement of endovascular techniques.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.35 no.1
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• pp.21-25
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• 2009

Objective: The purpose of this study is to determine the prevalence of sinus disease and abnormalities in patients scheduled for dental implant in maxillary posterior area using cone beam CT. Patients and Method: One hundred five maxillary sinuses in eighty-seven patients who underwent cone beam CT for dental implant in maxillary posterior area were included. Any patients who had previous history of sinus operations were not included. The sinus abnormalities were classified as follows ; normal (membrane thickness <2 mm), mucosal thickening (membrane thickness ${\geq}$ 2 mm and < 6 mm), partial opacification (membrane thickness > 6 mm but not full), full opacification and mucous retention cyst. The relationship between the remaining bone height, sinus symptoms and maxillary sinus abnormality was statistically surveyed. Results: Of 105 maxillary sinuses in 87 patients, 80 (76%) maxillary sinuses showed abnormalities ; 4 of 4 symptomatic patients and 76 of 101 asymptomatic patients. Mucosal thickening was the most common sinus abnormality. Only 3 (4%) of 80 maxillary sinus abnormalities were caused by the odontogenic origin. The prevalence of maxillary sinus abnormalities was higher in the symptomatic group than asymptomatic one (p<0.05). Conclusion: Maxillary sinus abnormalities were very common in the patients who were planning implantation in maxillary posterior areas. This result supports that thorough evaluation for maxillary sinus is recommended when implant treatment is planned for those areas.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.17
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• pp.7659-7661
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• 2015

Background: Essential thrombocythemia (ET) is a Philadelphia chromosome-negative myeloproliferative neoplasm characterized by sustained thrombocytosis and megakaryocytic hyperplasia. It is an uncommon hematological malignancy which primarily affects elderly individuals. The rational of this study was to determine its clinico-hematological profile along with risk stratification in Pakistan patients. Materials and Methods: In this retrospective cross sectional study, 21 patients with ET were enrolled from January 2011 to December 2014. Data was analyzed with SPSS version 21. Results: The mean age was $56.7{\pm}19.0years$ (range 18-87) and the male to female ratio was 1:1.1. Of the total, 62% of patients were above 50 years of age. Overall 61.9% were diagnosed incidentally and were asymptomatic. In symptomatic patients, major complaints were weakness (19%); erythromelalgia (14.2%), transit ischemic attack (9.5%) and gastrointestinal bleed (4.7%). The mean hemoglobin count was $11.7{\pm}2.4g/dl$ with a total leukocyte count of $13.3{\pm}8.1{\times}10^9/l$ and platelets count of $1188.8{\pm}522.2{\times}10^9/l$. Serum lactate dehydrogenase, serum creatinine and uric acid were $454.3{\pm}127.8$, $1.2{\pm}0.5$ and $7.4{\pm}3.4$ respectively. According to risk stratification, 57.1% were in high risk; 23.8% in intermediate risk while 19.1% in low risk group. Conclusions: ET in our patients in Pakistan, unlike in the West, is seen in a relatively young population. Primarily patients were asymptomatic and risk stratification revealed predominance of high risk disease in our setting.

• Journal of Korean Society of Forest Science
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• v.104 no.2
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• pp.332-335
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• 2015

Taphrina wiesneri, a pathogen of cherry witches' broom, is highly pathogenic to Prunus yedoensis Matsumura which are widely planted in parks and streets in South Korea. In order to control the disease, it is crucial to know the life cycle of the fungus. We attempted to detect the fungus tentatively overwintering in shoots and branches of cherry trees both having witches' broom and healthy before flowering and leafing in spring using PCR with species-specific primer set (TwITSF and TwITSR). Genomic DNAs were extracted from the symptomatic and the asymptomatic shoots or branches. Results indicated that T. wiesneri is present in leaf buds and inner bark not only in symptomatic branches but also in the asymptomatic branches in diseased trees. However, the fungus was not detected in flower buds of the symptomatic trees and any samples of healthy trees.

• Tuberculosis and Respiratory Diseases
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• v.69 no.6
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• pp.465-468
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• 2010

Spontaneous pneumothorax (SPTx) associated with primary lung cancer is quite rare, but has been reported as the initial presentation or a complication of disease progression. Moreover, chemotherapy-related SPTx in primary lung cancer occurs at a very low frequency, accounting for less than 0.05% of all cases. Here, we report the first case of erlotinib-related SPTx in a patient with advanced lung adenocarcinoma in Korea. After 3 cycles of cisplatin-based chemotherapy as first-line therapy, erlotinib was administered as second-line treatment. Asymptomatic SPTx accompanied by a significant decrease in tumor size was observed in the left lung 7 weeks later. The patient received continuous administration of erlotinib, without additional treatment. This case showed that SPTx can occur in patients with primary lung cancer receiving erlotinib, and asymptomatic chemotherapy-related SPTx in primary lung cancer may not require therapeutic intervention.

• Archives of Plastic Surgery
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• v.51 no.2
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• pp.208-211
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• 2024

Intraneural hematoma is a rare disease that results in an impaired nerve function because of bleeding around the peripheral nerve, with only 20 cases reported. Trauma, neoplasm, and bleeding disorders are known factors for intraneural hematoma. However, here we report atypical features of asymptomatic and spontaneous intraneural hematoma which are difficult to diagnose. A 60-year-old woman visited our clinic with the complaint of a palpable mass on the right calf. She reported no medical history or trauma to the right calf and laboratory findings showed normal coagulopathy. Ultrasonography was performed, which indicated hematoma near saphenous vein and sural nerve or neurogenic tumor. We performed surgical exploration and intraneural hematoma was confirmed on sural nerve. Meticulous paraneuriotomy and evacuation was performed without nerve injury. Histological examination revealed intraneural hematoma with a vascular wall. No neurologic symptoms were observed. In literature review, we acknowledge that understanding anatomy of nerve, using ultrasonography as a diagnostic tool and surgical decompression is key for intraneural hematoma. Our case report may help establish the implications of diagnosis and treatment. Also, we suggested surgical treatment is necessary even in cases that do not present symptoms because neurological symptoms and associated symptoms may occur later.

• Childhood Kidney Diseases
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• v.5 no.1
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• pp.1-8
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• 2001

Purpose : Clinical manifestations and pathologic findings of thin glumerular basement membrane disease, recognized as a common underlying disease of benign, familaiar and asymptomatic hematuria has not been reported systemically in Korera. We analyzed clinical and pathologic findings of patients who were diagnosed as thin glomerular basement membrane disease Methods : We analyzed clinical and pathologic findings of twenty-six patients who were diagnosed as thin glomerular basement membrane disease by renal biopsy among who complained asymptomatic hematuria from 1990 to 2000. Results : The subjects were aged 9.4${\pm}$3.2 (3.0-15.8) years-old at onset of hematuria, and 11.1${\pm}$2.2 (4.7-16.3) years-old at renal biopsy. Sexual discrepancy was more common in girls (eight boys and eighteen girls). A family history of hematuria was found in 8 patients(30.7$\%$). Major clinical manifestation on admission was microscopic hematuria according to the findings of 3case(11.5$\%$) of gross hematuria, 23cases(88.5$\%$9) of microscopic hematuria, and 1 case(3.8$\%$) of proteinuria. Microscopic hematuria persisted in all cases. Kidney biopsy showed few changes by light microscopy, but IgM, C3 and fibrinogen deposit in mesangium was found by immunofluorescent microscopy in a few cases. Electron microscopic findings have revealed thinning of the glomerular basement membrane varied from 180.9${\pm}$35.8nm. Conclusion : Thin glomerular basement membrane disease might be a common cause of microscopic hematuria of children and family history was revealed in about 30$\%$. Clinical progression was good in majorities.(J. Korean Soc Pediatr Nephrol 5 : 1-8, 2001)

• Journal of Chest Surgery
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• v.21 no.5
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• pp.905-910
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• 1988

Anomalous origin of the coronary artery from the pulmonary artery is a rare congenital coronary artery disease and the origin of the left coronary artery from the pulmonary artery represents the commonest form of these unusual lesions. Because of differences in symptomatology, clinical course and prognosis, this malformation has been divided into infant type[Bl-and-White-Garland syndrome] and adult type on the basis of the absence or presence of collateral circulation between the right and left coronary artery. The latter type has been reported relatively few cases. A 21-year-old male was admitted to the Yeungnam University Hospital, due to study of incidentally noticed heart murmur. At that time he was asymptomatic and past medical history was noncontributory. Chest roentgenogram was within normal limit and electrocardiogram was consistent with hypertrophy of left ventricle. Echocardiogram and aortogram demonstrated markedly dilated and tortuous right coronary artery and anomalous origin of the left coronary artery from the pulmonary artery. To prevent arteriosclerosis, progressive myocardial infarction, infection and aneurysmal rupture, Takeuchi operation which establish a two coronary system by transpulmonary arterial reconnection of the anomalous left coronary artery was done. Postoperative course was uneventful.

• Archives of Plastic Surgery
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• v.33 no.5
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• pp.577-580
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• 2006

Purpose: Hemangioma of the breast is an infrequent finding and usually encountered incidentally when checking for other disease. Most of hemangiomas of the breast are asymptomatic, not palpable perilobular type. Cavernous hemangioma of the breast is rare and only a few reports about this type of lesion are present. No example has been reported about reconstruction of the breast after resection of large cavernous hemangioma. Methods: We report here a case of immediate breast reconstruction using a Becker implant after subcutaneous mastectomy for a large cavernous hemangioma involving almost entire breast. Results: Symmetry is well maintained after 3 years without deformity or recurrence. Conclusion: The clinical prognosis of breast cavernous hemangioma is good after total excision and reconstruction.