• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.2
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• pp.129-139
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• 2003

Purpose: The aim of the study was to evaluate the long-term outcome of glycogen storage disease (GSD) type 1 with particular reference to hepatic adenoma and hepatocellular carcinoma, and to analyze risk factors affecting the development of hepatic adenoma in GSD type 1. Methods: Forty-three GSD type 1 patients (31 males and 12 females, mean age $13.9{\pm}6.4$ years) were analyzed retrospectively. Hepatic adenoma was detected on abdominal USG and diagnosed on histologic examination. Clinical profiles were compared between patients with hepatic adenoma (n=16) and age-matched controls without hepatic adenoma (n=16). Results: 1) Of 43 GSD type 1 patients, 16 (37.2%) had hepatic adeoma. Hepatic adenoma was detected at the age of mean $14.2{\pm}4.1$ years (range: 7.9~25.7 years). Fourteen (87.5%) adenomas were multiple at detection. 2) Comparison of the clinical profiles between adenoma group and non-adenoma group revealed that age at first introduction of uncooked cornstarch treatment was significantly late in adenoma group compared with non-adenoma group ($9.1{\pm}5.2$ years vs. $3.0{\pm}1.8$ years, p=0.003). Portocaval shunt surgery was performed in 11 (68.8%) patients in adenoma group and 3 (18.8%) in non-adenoma group (p=0.004). Hepatic adenoma developed mean $5.8{\pm}4.2$ years after shunt operation. 3) One patient was diagnosed as hepatocellular carcinoma at the age of 25.7 years. Conclusion: Hepatic adenoma is an important late complication of GSD type 1 with the risk of malignant transformation. Early introduction of cornstarch therapy with strict metabolic control is needed to prevent the development of hepatic adenoma in GSD type 1.

• Clinical and Experimental Pediatrics
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• v.49 no.7
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• pp.784-789
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• 2006

Purpose : $Henoch-Sch{\ddot{o}}nlein$ purpura(HSP) is a systemic vasculitis involving small vessels of skin, gastrointestinal(GI) tract and kidney. Digestive involvement of HSP can be serious with massive GI bleeding, perforation, and intussusception. However, some patients do not respond to conventional corticosteroid therapy. In this study, we investigated the efficacy of intravenous immunoglobulin (IVIG) for serious digestive manifestations not responding to steroid. Methods : From April 1999 to January 2005, 22 children diagnosed as HSP with severe GI symptoms were included. Initially, all patients were treated with intravenous methylprednisolone. IVIG 2 g/kg of body weight was infused in children refractory to steroid therapy. Clinical data were reviewed retrospectively. Results : Among 22 children, 12 children underwent IVIG therapy. The mean duration of corticosteroid therapy was $5.61{\pm}4.9$ days before IVIG therapy, and 11 of 12 patients experienced disappearance of GI manifestations after the initiation of IVIG infusion. In one patient, IVIG was ineffective in relieving abdominal pain, but melena subsided. Comparison of the duration of hospitalization between IVIG group and corticosteroid group revealed no significant difference($12.8{\pm}7.6$ days vs. $13.2{\pm}7.8$ days, P=0.777). But, the total duration of abdominal pain decreased in IVIG group although the difference between two groups was not significant($8.8{\pm}8.1$ days vs. $14.8{\pm}16.9$ days, P=0.306). Among 10 children treated with steroid only, 2 children were operated for bowel perforation and intussusception. In contrast, there was no perforation in 12 children who underwent IVIG therapy. Conclusion : IVIG could be the alternative therapy to corticosteroids in children with severe digestive manifestations of HSP.

• Journal of Korean Society of Coastal and Ocean Engineers
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• v.19 no.5
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• pp.492-501
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• 2007

Coastal disasters have become one of the most important issues in every coastal country. In Korea, coastal disasters such as storm surge, sea level rise and extreme weather have placed many coastal regions in danger of being exposed or damaged during subsequent storms and gradual shoreline retreat. A storm surge is an onshore gush of water associated with a tow pressure weather system, typically in typhoon season. However, it is very difficult to predict storm surge height and inundation due to the irregularity of the course and intensity of a typhoon. To provide a new scheme of typhoon damage prediction model, the scenario which changes the central pressure, the maximum wind radius, the track and the proceeding speed by corresponding previous typhoon database, was composed. The virtual typhoon scenario database was constructed with individual scenario simulation and evaluation, in which it extracted the result from the scenario database of information of the hereafter typhoon and information due to climate change. This virtual typhoon scenario database will apply damage prediction information about a typhoon. This study performed construction and analysis of the simulation system with the storm surge/coastal inundation model at Masan coastal areas, and applied method for predicting using the scenario of the storm surge.

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.170-175
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• 1997

Purpose : Hemolytic uremic syndrome(HUS), known as a most common cause of childhood renal failure in western countries, has been a relatively rare disease in Korea. Although the reported cases were not related to any specific cause in Korea, there was an outbreak of HUS with bloody diarrhea in Japan last year. We report here that we experienced the several typical HUS last year. Patients : From Jan.1996 to Dec. 1996, five patients were diagnosed as HUS at Samsung Seoul Medical Center, Dept. Pediatrics. Results : 1) The age of onset was below 3 years in 3 cases and above 9 years in 2 cases. 2) All the cases happened between summer and autumn. Three patients had domestic travel and 4 patients drank well or spring water before the symptoms. 3) The clinical manifestation was generalized edema, oliguria, anuria and hematuria. The bloody diarrhea were present in 5 cases and 1 patient had operation with the impression of appendicitis. 4) There was no bacteria which was isolated from the blood or stool samples. 5) Renal biopsies were performed in 2 cases, because of protracted clinical course. One showed microthrombotic angiopathy and the other cortical necrosis with necrotizing glomeruli. 6) Complete recovery was the outcome in 4 cases and one case progressed to chronic renal failure. Conclusion : In conclusion, typical HUS associated with bloody diarrhea, epidemic and good prognosis can be found in Korea and careful surveillance of the pre-clinical cases will be necessary.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.280-287
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• 2007

Purpose : Urinary lithiasis is uncommon in children, however, it may lead to chronic renal insufficiency and even end stage renal disease. The etiology of stone formation in children is largely unknown; although the most common causes are known to be associated with congenital anomalies of the genito-urinary(G-U) tract, urinary tract infections(UTI), and metabolic diseases. Methods : A total of 73 children(male:female=42:31, mean age $6.6{\pm}5.3$ years) presented with urinary lithiasis between Sep. 1998 and Jul. 2007 at Seoul National University Children's Hospital. The medical records were reviewed retrospectively. Results : The most common presenting symptoms were gross hematuria(28/73, 38%) and flank or abdominal pain(23/73, 32%). The stones were located in the upper urinary tract in 48 patients(66%), in the bladder in 18(24%), and in both the bladder and upper urinary tract in 2 (3%). Congenital anomalies of the G-U tract with/without UTI were detected in 30 children (41%), hypercalciuria with/without hypercalcemia in 15(20%), and other metabolic diseases in 8(11%). In 17 patients(23%), no underlying cause of stone formation was detected. The majority of stones were infected stones(24/36, 67%), which were followed by calcium stones(8/36, 22%), uric acid stones(3/36, 8%). and cystine stones(1/36, 3%). Thirty-four patients(46%) underwent surgical procedures and/or extracorporeal shockwave lithotripsy for stone removal, and 13(18%) passed stones spontaneously with/without medical management. Stones recurred in 6 patients(8%): 4 with neurogenic bladder augmented by ileocystoplasty, 1 with cystinuria, and 1 with unknown etiology. Conclusion : The common causes of urinary lithiasis in children were congenital anomalies of the G-U tract with/without UTI and metabolic disorders including hypercalciuria/hypercalcemia. For the management of stones, minimally invasive procedures should be chosen on the basis of accompanying symptoms and the composition, locations and etiology of stones.

• Nuclear Medicine and Molecular Imaging
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• v.43 no.6
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• pp.565-571
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• 2009

Purpose: The aim of this study was to evaluate the clinical value of rectal gas distension F-18 FDG PET/CT imaging for the differentiation of the rectal focal uptake lesions. Materials and Methods: Twenty four patients (M:F=11:13, Age $62.8{\pm}12.4$ years) underwent rectal gas distension F-18 FDG PET/CT, prospectively: initial image at 50-60 min after the intravenous injection of F-18 FDG and rectal distension image after the infusion of air through the anus. Focally increased uptake lesions on initial images but disappeared on rectal distension images defined a physiological uptake. For the differential evaluation of persistent focal uptake lesions on rectal distension images, colonoscopy and histopathologic examination were performed. Results: Among the 24 patients, 27 lesions of focal rectal uptake were detected on initial images of F-18 FDG PET/CT. Of these, 7 lesions were able to judge with physiological uptake because the focal increased uptake disappeared from rectal distension image. Remaining 3 lesions were non-rectal lesions (2 lesions: rectovesical space, 1 lesion: uterine myoma). Among 17 lesions which was showed persistent increased uptake in rectal distension image, 15 lesions were confirmed as the malignant tumor (SUVmax=$15.9{\pm}6.8$) and 2 lesions were confirmed as the benign lesions including adenoma and inflammatory disease. Conclusion: The rectal distension F-18 FDG PET/CT imaging could be an important noninvasive method for the differentiation of malignant and benign focal rectal uptake lesions including physiologic uptake.

• Journal of Veterinary Clinics
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• v.29 no.2
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• pp.134-140
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• 2012

This retrospective study was performed to describe and evaluate ultrasonographic characteristics of splenic disorders and to pursue any relationship with cytologic or histopathologic diagnoses. Medical records were reviewed for patients that had undergone both abdominal ultrasonographic procedures and ultrasound-guided fine-needle aspiration (FNA) or surgical biopsy of splenic lesions or necropsy from January 2002 to July 2011 at Seoul National University Hospital for Animals. Total 124 dogs, 76 cases with FNA and 48 cases with biopsy, were available for this investigation. The age of dogs ranged from 2 to 17 years ($mean{\pm}SD$ = $9.54{\pm}3.34$ years), with the gender distribution of 61 females (26 spayed) and 63 males (40 neutered). In breed distribution, 114 were purebred dogs and 10 were mixed breed dogs. The dogs represented 26 breeds, with 113 dogs categorized into small-sized breeds. The ultrasonographic appearances of splenic disorders could be classified into 10 types including normal appearance, depending on the distribution and echogenicity of splenic lesions as well as the increase in spleen size and diffuse parenchymal changes. Among 124 cases, 44 (35.4%) had hypoechoic nodules/masses with the highest frequency, followed by 23 (18.5%) with multiple small hypoechoic nodules. Of 124 cases, 70 (56.5%) were benign and 54 (43.5%) were malignant lesions at cytologic or histopathologic results. Each ultrasonographic appearance had variable cytologic or histopathologic diagnoses. However, diffuse heterogenicity and diffuse hypoechogenicity were significantly associated with malignancy (p < 0.05), whereas hyperechoic nodules/masses were more often associated with benignity (p < 0.05). Based on our results, it may be considered that the ultrasonographic examination for spleen could be able to provide the least information necessary for benign and malignant lesions in prioritizing differential diagnoses.

• Radiation Oncology Journal
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• v.9 no.2
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• pp.285-291
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• 1991

One hundred and twenty six patients with early uterine cervical cancer who had been treated at Departmen of Radiation Oncology of Korea University Hospital from Jan.1981 to Dec.1988 were analysed retrospectively by the treatment result and pattern of of failures. All patients had stage Ia to IIa disease and were grouped whether they had combination of operation and postop irradiation or radiation therapy alone. 1) Sixty six patients belonged to the combination treatment group and 60 patients to the radiation alone group. 2) Combination group consisted of $18.1\%$(12/66) stage Ia, $71.2\%$(47/66) stage Ib and $10.6\%$ (7/66) stage IIa patients. There were no stage Ia, 18.8$\%$(l1/60) stage Ib and 81.6$\%$(49/60) stage IIa patients for RT alone gronp. 3) There were total 23$\%$(29/126) treatment failures,13 patients in combination group and 16 patients in RT alone group. In 66 patients of combination group, they were found to have 5 locoregional failures, 7 distant failures and 1 at both sites. In 60 patients of RT alone group, 9 locoreginal failure and 7 distant failures occured. Eighty six percent (25/29) of total failures appeared within 18 month after completion of treatment. About 60$\%$ of the patients with regional recurrences which were located at pelvic side wall or pelvic lymph nodes paesented their recurrent disease after 1 year of completion of treatment, whereas same percent of distant failures appeared within 6 month. 5) In RT alone group, the first sites of distant failure were mostly para-aortic lymph node and/or left supraclavicular lymph node (71.4$\%$,5/7). In combination group, various sites such as inguinal lymph node, mediastinal lymph node, liver, lung and bone appeared first or at the same time with para-aortic and supraclavicular lymph node metastasis. 6) Logistic regression analysis was done for multivariate analysis of the factors contributing to locoregional and distant failures. In combination group, adequacy of the resection margin and the presence of positive pelvic node were found to be the most significant factors (p=0.0423 & 0.0060 respectively). In RT alone group, less than complete regression of the tumor at the end of treatment was the only significant contributing factor for the treatment failures (p=0.0013) with good liklihood ratio.

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.101-109
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• 2013

Purpose: Pediatric urolithiasis is uncommon in children but is a cause of significant morbidity and damage to the kidney. Although much information on adult urolithiasis is available in the literature, large studies on the pediatric population are still scarce. In this report, we review our experience with pediatric urolithiasis over 22 years at a tertiary referral center. Method: We retrospectively reviewed the records of children with newly diagnosed urolithiasis between January 1991 and May 2013. We assessed the age, sex, family history, initial symptoms, location of stones, underlying cause, stone analysis, treatment, and recurrence among the patients. Results: In total, 137 patients (96 male, 41 female) were assessed. The age range was 0-17 years (mean age, 6.0 years). Forty-three (31%) children were aged <1 year, and 37% (16/43) had a history of intensive care unit (ICU) admission. Thirteen patients (9.5%) had a family history of stones. The most common symptoms at presentation among the patients were gross hematuria (56/137, 41%) and flank or abdominal pain (46/137, 34%). The stones were located in the kidney (85/137, 62%), ureter (29/137, 21%), bladder (2/137, 1.4%), and multiple locations (20/137, 15 %). Congenital abnormalities of the genitourinary (G-U) tract, with or without metabolic abnormality, or urinary tract infection (UTI) was detected in 26 children (19%). Ninety-one patients (66%) underwent metabolic examination, and 38% of these patients exhibited an abnormality. UTI, with or without abnormalities of the G-U tract, or metabolic abnormality was detected in 26 children (19%). Of the 35 stones analyzed, the majority were calcium stones (20/35, 57%), followed by infected stones (5/35, 14%), uric acid stones (4/35, 11%), carbonate apatite stones (3/35, 7%), cystine stones (2/35, 6%), and phosphate stones (1/35, 3%). Five patients (4%) required open procedures, with or without non-open procedures, whereas 77 patients (56%) were managed conservatively; the remaining 55 patients (40%) received some other form of intervention. Eighteen patients (13%) had stone recurrence during the follow-up period. Conclusions: Pediatric urolithiasis is commonly associated with abnormalities of the G-U tract and/or metabolic disorders and/or UTI. Half of the patients will pass their stones spontaneously, and all the techniques of minimally invasive surgery are applicable in the treatment of children with stones. As the recurrence rates are high among this population, long-term follow-up is recommended and the complete clearance of stones is important.

• The Korean Journal of Nuclear Medicine
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• v.36 no.6
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• pp.368-380
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• 2002

Purpose: Liver transplantation (LT), one of the therapeutic options of primary liver cancer has been suffering from recurrence caused by metastasis in 8-54% of patients. This study was performed to investigate whether FDG-PET is useful for detecting hidden metastasis in LT candidates. Materials and Methods: Twenty-six patients (male:female=23:3, mean age 55.7 years) underwent FDG-PET. Their previous conventional diagnostic studies (CDS) like abdomen US and CT, chest x-ray and CT, and bone scan were negative (n=22) or equivocal (n=4) for metastasis. Positive FDG-PET findings were confirmed by biopsy or clinical follow-up. Results: Among 4 patients with equivocal metastatic lesions on CDS, 3 had 6 hypermetabolic lesions on FDG-PET, which were confirmed as metastasis and subsequently LTs were cancelled. Of these, 5 lesions were initially negative on CDS. Remained 1 patient underwent LT with a negative FDG-PET result. Among 22 patients without metastasis on CDS, 5 had 7 hypermetabolic lesions on FDG-PET. One of these patients proved to have 2 metastatic lesions, and LT was cancelled. The other 4 patients had S hypermetabolic lesions on FDG-PET, which were confirmed as benign lesions, and 3 patients of them underwent LT. In summary, FDG-PET was useful in avoiding 4 unwarranted LT by detecting unsuspected metastatic lesions on CDS. A total of 17 patients underwent LT. In comparison with pathology, the sensitivity and specificity of FDG-PET for detecting viable primary liver cancer were 55.6% (5/9) and 87.5% (7/8), respectively. Conclusion: FDG-PET can detect additional hidden metastasis and contribute to reducing unwarranted LT in the patients with primary liver cancer.