• Neonatal Medicine
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• v.25 no.1
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• pp.49-52
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• 2018

Arterial tortuosity syndrome (ATS) is a very rare autosomal recessive connective tissue disease characterized by generalized elongation and tortuosity of the medium- to large-sized arteries, and other systemic connective tissue manifestations. To date, this disease entity has not been reported in Korea. We report a case of ATS diagnosed in a neonate who presented with severe elongation and tortuosity of the aorta and its major branches, as well as the intracranial arteries. Additionally, the patient presented with a tortuous dilatation of the inferior vena cava, an aneurysmal dilatation of the extra-hepatic bile ducts, and an inguinal and sliding hiatal hernia. The diagnosis was confirmed using DNA sequencing analysis, and the patient demonstrated a compound heterozygosity for two novel mutations (c.738delG [p.Gln247Serfs*33] and c.362T>C [p.Ile121Thr]) in exon 2 of the SLC2A10 gene. Genetic analysis also confirmed that both parents were heterozygous carriers of the responsible mutations. Owing to such clinical manifestations, ATS is often misdiagnosed as other connective tissue diseases including Loeys-Dietz syndrome, Marfan syndrome, and Ehlers-Danlos syndrome. In patients presenting with a high index of suspicion, thorough clinical evaluation and screening for ATS including computed tomography or magnetic resonance angiography and target gene analysis are necessary for early diagnosis and management.

• Journal of Preventive Medicine and Public Health
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• v.36 no.2
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• pp.147-152
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• 2003

Objectives : To develop a Diagnosis-Related Group (DRG) fraud candidate detection method, using data mining techniques, and to examine the efficiency of the developed method. Methods ; The Study included 79,790 DRGs and their related claims of 8 disease groups (Lens procedures, with or without, vitrectomy, tonsillectomy and/or adenoidectomy only, appendectomy, Cesarean section, vaginal delivery, anal and/or perianal procedures, inguinal and/or femoral hernia procedures, uterine and/or adnexa procedures for nonmalignancy), which were examined manually during a 32 months period. To construct an optimal prediction model, 38 variables were applied, and the correction rate and lift value of 3 models (decision tree, logistic regression, neural network) compared. The analyses were peformed separately by disease group. Results : The correction rates of the developed method, using data mining techniques, were 15.4 to 81.9%, according to disease groups, with an overall correction rate of 60.7%. The lift values were 1.9 to 7.3 according to disease groups, with an overall lift value of 4.1. Conclusions : The above findings suggested that the applying of data mining techniques is necessary to improve the efficiency of DRG fraud candidate detection.

• Advances in pediatric surgery
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• v.8 no.2
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• pp.95-100
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• 2002

Lymphangiomas or cystic hygromas are quite commonly seen in children. During a 22-year period, from January 1980 to December 2001, 117 patients with lymphangioma were treated and followed in the Department of Pediatric Surgery at Hanyang University Hospital. The male-to-female ratio was 1.9:1(77:40) with a male preponderance. As for the age incidence at time of diagnosis, 10(8.5 %) patients were noted under 1 month of age, 37(31.6%) were between 1 month and 1 year of age, 12(10.3%) between 1 and 2 years of age, so 59(50.4%) were under 2 years of age. Sixty one (52.1%) lymphangioma-cases were located in the neck, including one case at the nape. Axilla was the second in frequency and the rest were scattered at various sites. The intrascrotal lymphangioma is very rare but we have experienced one case of primary intrascrotal lymphangioma. Eighteen (15.4%) cases were located in the intraabdominal area, 10 in the mesentery, 2 in the greater omentum and 6 in the retroperitoneum. The chief complaints of intraabdominal lymphangioma were abdominal pain, intestinal obstruction, inguinal hernia, palpable mass, and/or abdominal distension. Among 77 histologically proven cases, 14 cases were cavernous lymphangiomas and the rest were cystic lymphangiomas. Bleeding in the lymphangioma was noted in 20(17.1%) cases of all. As for the treatment, a complete excision was performed in 77(65.8%) patients and $AgNO_3$ sclerotherapy after incomplete excision was performed in 23(19.7%). Picibanil (OK-432) sclerotherapy was performed in 17(14.5%) patients. Recurrence rate was 7.7 % and mortality occurred in one case who had a large neck lymphangioma extending into the mediastinum.

• Advances in pediatric surgery
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• v.2 no.1
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• pp.1-7
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• 1996

Clinical experiences of 833 hydrocele children presented at Hanyang University Hospital, of whom 456 children were operated by one pediatric surgeon from September 1979 to December 1993, were analyzed. Eight hundred and twenty three children were boys(right 476, left 279, bilateral 49, and unknown 19), and 10 were girls(8 right and 2 left). Operation was performed on 446 boys and all girls. Of boys diagnosed before the age of 6 months, 15.6% was operated and 68.7% of those after 6 months of age was operated. Among the boys operated after 2 years old, 16.5% had had hydrocele before 6 months of age, 20.4% before 1 year old and 34.6% before 2 years old. On the other hand, 28.8% of boys diagnosed after 2 years of age did not undergo hydrocelectomy. Sixteen children with hydroceles had contralateral hernias at the same time. After repair of unilateral hydroceles, contralateral hydroceles developed in 7 and hernias in 3 children. After disappearance of unilateral hydroceles, it reappeared at the same site in 4 and contralateral hydroceles or hernias developed in 2 children each. Hydroceles converted to hernias in 6 children before treatment. Hydroceles developed after ventriculo-peritoneal shunt in 5 children. The pathophysiology of hydrocele and inguinal hernia seems to be the same because of the similar distribution of onset age between them but hydrocele has various clinical courses. The results that 34.6% of boys operated after 2 years old had had hydrocele before 2 years of age and 28.8% of boys diagnosed after 2 years old did not undergo hydrocelectomy could not imply the proper age when hydrocelectomy could be performed. But operative repair of hydroceles after the age of 6 months seems to be recommendagle.

• Journal of Gastric Cancer
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• v.21 no.3
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• pp.279-297
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• 2021

Purpose: Various changes in nutrition, metabolism, immunity, and psychological status occur through multiple mechanisms after gastrectomy. The purpose of this study was to predict disease status after gastrectomy by analyzing diseases pattern that occur or change after gastrectomy. Materials and Methods: A retrospective cohort study was conducted using nationwide claims data. Patients with gastric cancer who underwent gastrectomy or endoscopic resection were included in the study. Eighteen target diseases were selected and categorized based on their underlying mechanism. The incidence of each target disease was compared by dividing the study sample into those who underwent gastrectomy (cases) and those who underwent endoscopic resection for early gastric cancer (controls). The cases were matched with controls using propensity score matching. Thereafter, Cox proportional hazard models were used to evaluate intergroup differences in disease incidence after gastrectomy. Results: A total of 97,634 patients who underwent gastrectomy (84,830) or endoscopic resection (12,804) were included. The incidence of cholecystitis (P<0.0001), pancreatitis (P=0.034), acute kidney injury (P=0.0083), anemia (P<0.0001), and inguinal hernia (P=0.0007) were higher after gastrectomy, while incidence of dyslipidemia (P<0.0001), vascular diseases (ischemic heart disease, stroke, and atherosclerosis; P<0.0001, P<0.0001, and P=0.0005), and Parkinson's disease (P=0.0093) were lower after gastrectomy. Conclusions: This study identifies diseases that may occur after gastrectomy in patients with gastric cancer.

• The Journal of the Korean bone and joint tumor society
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• v.1 no.2
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• pp.133-144
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• 1995

After resection of intraarticular, periacetabulum(P2) and pubic rami(P23) and extraarticular, proximal femur(P2-H12) by Enneking classification, reconstruction is very difficult. We experienced three cases of saddle prostheses for reconstruction after P2, P23, and P2-H12 resection in pelvic malignancies. Case 1 was a high grade chondrosarcoma in 36 year-old-man and P2 resection was done. But he died of disease 19 months after operation. Case 2 was a malignant giant cell tumor in 32 year-old-woman. P23 resection was given and she is disease-free 32 months after operation. Case 3 was an osteosarcoma of 27-year-old-man and P2-H12 resection was performed and he is disease-free postoperative 12 months now. According to MSTS functional evaluatin system, all three patients showed no pain(5), intermediate function(2), emotinally satisfied(3), one cane or crutch supported(1), limited walking(3), and minor cosmetic gait(3). There was no significant complication and no dislocation except intermittent inguinal hernia in case 2. All patients started crutch walking 3 weeks after operation. Around 6 months postoperatively, the preserved iliac wing(P1 component) was hypertrophied enough to endure the full weight bearing. All could have squating and kneeling positions. In conclusion, saddle prosthesis would be a very useful method of reconstruction after P2, P23, and/or H1-2 resection to shorten the operation time and to reduce the infection rate without significant loss of function.

• Journal of Preventive Medicine and Public Health
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• v.33 no.3
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• pp.334-342
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• 2000

Objectives . This study was conducted in order to investigate the degree of recognition, acceptability, and altitude towards day surgery of patients who were hospitalized with diseases that were candidates for day surgery; in order to analyze the average length of stay for treatment of the ailments; and to analyze the percentage of patients who could be discharged on the same day after the surgery ,using the post-anesthesia discharge scoring system. Methods : Data was collected between February 1 and March 31, 1999 from 353 patients who received surgery for cataract, adenoid hypertrophy, inguinal hernia, strabismus, ptosis, cholelithiasis, hemorrhoid, or anal fistula, at a general hospital in Daegu city. The patients were interviewed and surveyed by a post-anesthesia discharge scoring system(PADS) in order to collect data on patient condition such as vital signs, activity and mental status, pain, nausea and vomiting, surgical bleeding, intake and output after the surgery. Results : Among the 353 patients, 52.7% were after of the day surgery and 52.7% were interested in day surgery. Of the respondents, 43.1% said 'my ailment was not serious and the surgery was simple' and 30.4% said 'according to my condition rest at home was desirable' as the reasons for wanting day surgery Alternatively, 56.5% of those declining day surgery said the 'uncertainty of staying home' was the reason. The greatest concern in discharging within 24 hours after surgery was a post-op emergency situation. On the other hand, the shortened hospitalization was the largest advantage of day surgery with 39.1% responding this way, followed by the savings in hospitalization costs (25.8%) and emotional stability (13.7%). The majority of those surveyed (47.6%) believed that discharge should be determined within 1-2 days after the surgery. The average hospital stay was 3.1 days for dischargeable ailments. Pain (45.6%), nausea and vomiting (10.5%), and headache (7.9%) were the common symptoms following surgery. The percentage of patients who were able to be discharged within 24 hours after surgery revealed 95.2% were dischargeable after approximately 3 hours, 99.2% dischargeable after 12 hours, and 100% dischargeable after 24 hours. Conclusions : According to the PADS score, the cataract extract and strabismus correction patients were eligible for day surgery and the further evaluation concerning the reason for delayed recovery of the other diseases is needed.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.213-221
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• 2005

Purpose : Urachal anomalies are rare but are known to develop several complications, especially infection. Moreover, uniform guidelines for management have not been presented because of the variable clinical characteristics of these anomalies. The purpose of this report is to review our experience with urachal anomalies and attempt to determine the optimal management. Methods : We retrospectively reviewed the records of fourteen children with a variety of urachal anomalies who had been treated from January 1996 to June 2005 at Dong Kang General Hospital. Results : The age distribution of the patients(mean age; 3.8 years) was six neonates, one infant, five preschool-age and two school-age children. The male to female ratio was 1:1. Six cases of urachal cyst, four cases of patent urachus, two cases of urachal sinus and two cases of urachal diverticulum were found. Three patients with patent urachus and one with urachal cyst had hydronephrosis. Other associated anomalies included an inguinal hernia in one patient with urachal sinus and a vesicoureteral reflux in one patient with urachal diver ticulum. As a first-line diagnostic tool, high-resolution ultrasound examination was performed in thirteen cases and computed tomography in one case. Surgical excision was performed in nine patients with urachal anomaly. Five cases out of six neonatal cases experienced spontaneous improvement during a three-month follow up period. Due to frequent infection of the umbilicus, surgical excision was performed on one neonate with urachal sinus. Conclusion : All patients with urachal anomalies should undergo investigation for associated anomalies. The neonate with urachal anomalies, especially patent urachus, do not require surgical excision unless the patient has multiple episodes of recurrent infection. (J Korean Soc Pediatr Nephrol 2005;9:213-221)

• Clinical and Experimental Pediatrics
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• v.49 no.1
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• pp.24-28
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• 2006

Purpose : The purpose of this study was to investigate the health status and nutritional condition of children living in a low-income community through anthropometric, laboratory evaluation. Methods : A community-based survey identified children below 15 years living in a low-income community. Their weight, height, visual acuity, hearing level and dental status were measured. Blood sample were obtained on June and July, 2004. Hemoglobin, serum cholesterol, Hepatitis B antigen/antibody, AST and ALT were measured. Results : A total of 285 students(M : F=141 : 144) aged 6 to 14 years were included in this study. The heights and weights in some grades were smaller than controls. The prevalence of obesity was 10.6 percent in males and 10.7 percent in females. The prevalence of abnormal visual acuity, hearing impairments and dental carries were 20.5 percent, 0.3 percent and 69.4 percent. The prevalence of anemia was 10.1 percent. Serum total cholesterol was over 200 mg/dL in 7 percent. They complained of abdominal pain(22.1 percent) and headache(17.1 percent). Hyperthyroidism, cataract, neurofibromatosis, severe atopic dermatitis, ventricular septal defect, strabismus and inguinal hernia were newly diagnosed. Conclusion : Mean heights and weights of children in the low-income community were smaller than controls. The prevalence of abnormal visual acuity, hearing impairment and dental carries were higher than in the 2003 national health survey. Additional research is needed to evaluate the health status of the low-income community.

• Clinical and Experimental Pediatrics
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• v.52 no.3
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• pp.295-302
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• 2009

Purpose : To report our experience of gastrointestinal (GI) operations (OP) performed in very low birth weight infants (VLBWI) and to evaluate their clinical characteristics. Methods : Among the 1,117 VLBWI admitted to the SMC neonatal intensive care unit from November 1994 to February 2007, the medical records of 37 infants who underwent GI OP (except inguinal hernia OP) and 1,080 VLBWI without GI OP were retrospectively reviewed. Results : The mean gestational age ($27^{+6}2^{+3}$ vs. $28^{+5}{\pm}$2^{+6}$) and birth weight (979${\pm}$241 g vs. 1,071${\pm}$271 g) of the 37 VLBWI who underwent the GI OP was lower than the VLBWI without GI OP group (n=1,080). Mortality rates in the GI OP group were significantly higher than in the non GI OP group (28% vs. 15%, P<0.001). The incidence of cholestasis, retinopathy of prematurity and periventricular leukomalacia were higher in the GI OP group than in the non GI OP group, but the incidence of bronchopulmonary dysplasia was not significantly different between the GI OP group and the non GI OP group. For GI OP indications, focal intestinal perforation was most common and showed a more favorable outcome than necrotizing enterocolitis. Compared with an earlier 7-year period, 1994-2000, the incidence and survival rates increased in the subsequent 2001-2007 period. Conclusion : GI OP was associated with high mortality and morbidity in VLBWI. Further efforts to improve outcomes of GI OP in VLBWI should be investigated to improve the quality of care in VLBWI.