Background/Aims: We aimed to compare the differences in pediatric Crohn’s disease (CD) and ulcerative colitis (UC) at diagnosis in Korea. Methods: This was a multicenter, registry-based, inception cohort study conducted at five centers in Korea between 2013 and 2017. Baseline demographics, clinical characteristics, and results from laboratory, endoscopic, radiologic examinations were compared between pediatric CD and UC patients who were <19 years old at diagnosis. Results: A total 307 patients were included (227 CD [73.9%] and 80 UC [26.1%]). The male to female ratio was 2.49:1 for CD, and 1.49:1 for UC (p=0.019). Median age at diagnosis was 14.4 years (interquartile range, 12.4 to 16.2) for CD, and 14.4 years (interquartile range, 11.7 to 16.5) for UC (p=0.962). Hematochezia was the only dominant symptom in UC patients compared to CD patients (86.2% vs 30.8%, p<0.001). White blood cell counts, platelet counts, erythrocyte sedimentation rate, and C-reactive protein levels were significantly higher, and serum albumin level was significantly lower in CD patients than in UC patient. Anti-Saccharomyces cerevisiae antibody was positive in 44.5% and 16.2% of CD and UC patients, respectively (p<0.001), and antineutrophil cytoplasmic antibody was positive in 15.0% and 58.8% of CD and UC patients, respectively (p<0.001). Terminal ileal involvement was prominent in CD, while rectal involvement was more prominent in UC. Small bowel involvement and perianal perforating diseases were also more prominent in CD. Conclusions This is the first a multicenter study in Korea to compare the differences between pediatric CD and UC at diagnosis in Korea. A large-scale, national study is expected to better clarify these findings in the future.

Background: There are limited data regarding the extraintestinal manifestations (EIMs) associated with pediatric inflammatory bowel disease (IBD) in Korea. We aimed to investigate the clinical features and factors associated with the development of EIMs in Korean children and adolescents with IBD. Methods: This multicenter, retrospective study was conducted from 2010 to 2017. Baseline clinicodemographic, laboratory findings, disease activity, disease phenotypes, and EIMs were investigated. Results: A total of 172 patients were included. One-hundred thirty-seven (79.7%) had Crohn's disease (CD), and 35 (20.3%) had ulcerative colitis (UC). EIMs occurred in 42 patients (24.4%). EIMs developed in 34/137 diagnosed with CD (24.8%), and in 8/35 diagnosed with UC (22.9%), during a median follow-up duration of 3.2 (interquartile range, 1.9–5.4) years for CD and 3.0 (1.0–4.0) years for UC, respectively. Arthritis/arthralgia was most commonly observed (n = 15, 35.7%), followed by stomatitis/oral ulcer (n = 10, 23.8%), hepatitis (n = 5, 11.9%), nephritis (n = 4, 9.5%), pancreatitis (n = 2, 4.8%), erythema nodosum (n = 2, 4.8%), pyoderma gangrenosum (n = 1, 2.4%), primary sclerosing cholangitis (n = 1, 2.4%), uveitis (n = 1, 2.4%), and ankylosing spondylitis (n = 1, 2.4%). A significant difference in disease severity based on the Paris classification (P = 0.011) and ESR at diagnosis (P = 0.043) was observed between the EIM positive and negative group in patients with UC. According to logistic regression analyses, S1 disease severity based on the Paris classification was the only factor that was significantly associated with the development of EIMs (odds ratio, 16.57; 95% confidence interval, 2.18–287.39; P = 0.017). Conclusion Severe disease activity based on the Paris classification in pediatric patients with UC was significantly associated with EIM development. As disease severity in the Paris classification is a dynamic parameter, treatment should be focused on disease control to minimize the occurrence of EIMs in Korean children and adolescents with UC.

The absence of a gallbladder is a very rare anomaly. While it is usually asymptomatic, it can cause biliary colic symptoms. For these reasons, gallbladder agenesis can be misdiagnosed as a hepatobiliary disease and is diagnosed correctly after surgery. This condition may also be detected through an autopsy for other causative diseases. Abdominal ultrasonography is used as a diagnostic method to detect gallbladder agenesis. Hepatobiliary scintigraphy, magnetic resonance cholangiopancreatography, and endoscopic cholangiopancreatography are also used to make a more accurate diagnosis. In the emergency room, however, gallbladder agenesis can still be misdiagnosed as acute or chronic cholecystitis, leading to the detection of gallbladder agenesis in the operating room. Although some cases of gallbladder agenesis detected in adults during surgery have been reported in Korea, there are no reports of gallbladder agenesis in pediatric patients to date. This paper reports a case of gallbladder agenesis in a symptomatic child that was detected incidentally by a radiographic examination.
Adult ; Autopsy ; Child ; Cholangiopancreatography, Magnetic Resonance ; Cholecystitis ; Colic ; Diagnosis ; Emergency Service, Hospital ; Gallbladder ; Humans ; Korea ; Methods ; Operating Rooms ; Pediatrics ; Radionuclide Imaging ; Ultrasonography

The incidence of inflammatory bowel disease (IBD) is rapidly increasing worldwide. Indigo naturalis is known to have an antiinflammatory effect. Indigo naturalis has been traditionally used in the treatment of IBD in China and Japan. Currently, it is used as a primary or adjunctive drug in patients with ulcerative colitis. There are some reports of the effects of indigo naturalis when used in patients with ulcerative colitis. However, its usage has been associated with adverse events, including liver dysfunction, headache, gastrointestinal disturbance, and pulmonary hypertension. Pancreatitis as an adverse event during treatment using indigo naturalis has not yet been reported. We report a case of recurrent events of pancreatitis that occurred briefly after starting medication with indigo naturalis in a child with severe Crohn’s disease. The pancreatitis improved after indigo naturalis was discontinued in 2 events. This is the first case to report the association between pancreatitis and indigo naturalis in the English literature.
Child ; China ; Colitis, Ulcerative ; Crohn Disease ; Headache ; Humans ; Hypertension, Pulmonary ; Incidence ; Indigo Carmine ; Inflammatory Bowel Diseases ; Japan ; Liver Diseases ; Medicine, Kampo ; Pancreatitis

PURPOSE: The present study aimed to evaluate progression and prognosis according to the palliation method used in neonates and early infants aged 3 months or younger who were diagnosed with pulmonary atresia with ventricular septal defect (PA VSD) or tetralogy of Fallot (TOF) with severe pulmonary stenosis (PS) in a single tertiary hospital over a period of 12 years. METHODS: Twenty with PA VSD and 9 with TOF and severe PS needed initial palliation. Reintervention after initial palliation, complete repair, and progress were reviewed retrospectively. RESULTS: Among 29 patients, 14 patients underwent right ventricle to pulmonary artery (RV-PA) connection, 11 palliative BT shunt, 2 central shunt, and 2 ductal stent insertion. Median age at the initial palliation was 13 days (1–98 days). Additional procedure for pulmonary blood flow was required in 5 patients; 4 additional BT shunt operations and 1 RV-PA connection. There were 2 early deaths among patients with RV-PA connection, one from RV failure and the other from severe infection. Finally, 25 patients (86%) had a complete repair. Median age of total correction was 12 months (range, 2–31 months). At last follow-up, 2 patients had required reintervention after total correction; 1 conduit replacement and 1 right ventricular outflow tract (RVOT) patch enlargements. CONCLUSION: For initial palliation of patients with PA VSD or TOF with severe PS, not only shunt operation but also RV-PA connection approach can provide an acceptable outcome. To select the most proper surgical strategy, we recommend thorough evaluation of cardiac anomalies such as RVOT and PA morphologies and consideration of the patient's condition.
Follow-Up Studies ; Heart Septal Defects, Ventricular* ; Heart Ventricles ; Humans ; Infant ; Infant, Newborn ; Methods ; Palliative Care ; Prognosis ; Pulmonary Artery ; Pulmonary Atresia* ; Pulmonary Valve Stenosis* ; Retrospective Studies ; Stents ; Tertiary Care Centers ; Tetralogy of Fallot*

PURPOSE: Febrile urinary tract infection (UTI) is one of the commonest bacterial infections in children. The purpose of this study is to investigate the clinical characteristics of the first episode of febrile UTI occurring in children over 5 years compared to those in infants younger than a year. METHODS: We retrospectively reviewed the medical records of 10 patients over 5 years, having febrile UTI, and 25 controls under 1 year. Clinical characteristics including symptoms at admission, the time interval between symptom onset and hospital visit and/or diagnosis, duration of fever, urinalysis, and other laboratory and imaging test results were compared between the two groups. RESULTS: Most patients in the control group showed only high fever at the time of presentation to the hospital. However, 60% of the case group had fever along with gastrointestinal (GI) symptoms such as abdominal and flank pain, vomiting, as well as relatively mild pyuria. The case group showed a longer duration between symptom onset and hospital visit and/or diagnosis. CONCLUSIONS: Delay in diagnosis and initiation of treatment of UTI increases the risk of permanent renal scarring and associated complications. Therefore, early diagnosis and treatment of febrile UTI is vital for very young infants, as well as children considering that febrile UTI could be an important cause of febrile illness in children over 5 years.
Bacterial Infections ; Child ; Cicatrix ; Diagnosis ; Early Diagnosis ; Fever ; Flank Pain ; Humans ; Infant ; Medical Records ; Pyuria ; Retrospective Studies ; Urinalysis ; Urinary Tract Infections ; Vomiting

PURPOSE: Previous studies have reported that clinical efficacy of steroid therapy for acute bronchiolitis is controversial. However, since it is still frequently used in clinical practice, we sought to re-evaluate its effectiveness. METHODS: This study included 277 children with acute bronchiolitis who were admitted to Kyungpook National University Children's Hospital from March 2013 to July 2016. Erythrocyte sedimentation rates, C-reactive protein (CRP) levels, and viral polymerase chain reaction testing results were obtained, and respiratory rate (RR) was measured periodically. Forty-eight patients were treated with an intravenous (IV) steroid (17.3%, IV group) and 19 patients were treated with a per oral (PO) steroid medication (6.9%, PO group). The remaining 210 patients were steroid-free patients (74.2%, nonsteroid group). RESULTS: RR and CRP levels were higher in the IV group, along with a longer hospitalization period and duration of wheezing. The rate of change from the fastest initial RR to the mean RR on the first treatment day was greatest in the IV group; this finding was statistically significant after controlling for initial RR (16.06% in the IV group, 3.94% in the PO group, 4.90% in the nonsteroid group; P < 0.01). CONCLUSION: There was a trend of IV steroid treatment toward more severe bronchiolitis. A significant reduction in RR on the first day of steroid treatment was observed in IV steroid-treated patients. IV steroid therapy may play a positive role in initial RR stabilization for severe bronchiolitis.
Blood Sedimentation ; Bronchiolitis* ; C-Reactive Protein ; Child ; Gyeongsangbuk-do ; Hospitalization ; Humans ; Polymerase Chain Reaction ; Respiratory Rate ; Respiratory Sounds ; Retrospective Studies* ; Treatment Outcome

PURPOSE: Nocturnal enuresis (NE) is one of the most common problems in childhood. NE has a multifactorial etiology and is influenced by sleep and arousal mechanisms. The aim of the present study was to prospectively evaluate sleep problems and patterns in children with NE compared with normal healthy controls. METHODS: Twenty-eight children with NE and 16 healthy controls were included in the study. To evaluate sleep habits and disturbances, parents and children filled out a questionnaire that included items about sleep patterns and sleep-related behaviors prior to treatment for NE. Demographic factors and other data were compared for the two groups based on the responses to the sleep questionnaire. RESULTS: Night awakening, sleepwalking, and periodic limb movements were more prevalent in children with NE, but symptoms of sleep-disordered breathing were not increased in this group. There were statistically significant differences in periodic limb movements and daytime sleepiness between the two groups. CONCLUSION: Children with NE seemed to have more sleep problems such as night awakening, sleepwalking, and periodic limb movements. In addition, a higher level of daytime sleepiness and hyperactivity in patients with NE suggested a relationship between NE and sleep disorders.
Arousal ; Child* ; Demography ; Extremities ; Humans ; Nocturnal Enuresis* ; Parents ; Prospective Studies ; Sleep Apnea Syndromes ; Sleep Wake Disorders ; Somnambulism

PURPOSE: The purpose of this study was to assess the safety and early outcomes of the Pipeline device for large/giant or fusiform aneurysms. MATERIALS AND METHODS: The Pipeline was implanted in a total of 45 patients (mean age, 58 years; M:F=10:35) with 47 large/giant or fusiform aneurysms. We retrospectively evaluated the characteristics of the treated aneurysms, the periprocedural events, morbidity and mortality, and the early outcomes after Pipeline implantation. RESULTS: The aneurysms were located in the internal carotid artery (ICA) cavernous segment (n=25), ICA intradural segment (n=11), vertebrobasilar trunk (n=8), and middle cerebral artery (n=3). Procedure-related events occurred in 18 cases, consisting of incomplete expansion (n=8), shortening-migration (n=5), transient occlusion of a jailed branch (n=3), and in-stent thrombosis (n=2). Treatment-related morbidity occurred in two patients, but without mortality. Both patients had modified Rankin scale (mRS) scores of 2, but had an improved mRS score of 0 at 1-month follow-up. Of the 19 patients presenting with mass effect, 16 improved but three showed no changes in their presenting symptoms. All patients had excellent outcomes (mRS, 0 or 1) during the follow-up period (median, 6 months; range, 2-30 months). Vascular imaging follow-up (n=31, 65.9%; median, 3 months, range, 1-25 months) showed complete or near occlusion of the aneurysm in 24 patients (77.4%) and decreased sac size in seven patients (22.6%). CONCLUSION: In this initial multicenter study in Korea, the Pipeline seemed to be safe and effective for large/giant or fusiform aneurysms. However, a learning period may be required to alleviate device-related events.
Aneurysm* ; Carotid Artery, Internal ; Follow-Up Studies ; Humans ; Korea* ; Learning ; Middle Cerebral Artery ; Mortality ; Retrospective Studies ; Thrombosis

BACKGROUND: We investigated the clinical features and treatment outcomes of patients with mantle cell lymphoma (MCL) in Korea. METHODS: We retrospectively analyzed the clinical characteristics and prognosis of 131 patients diagnosed with MCL between January 2004 and December 2009 at 15 medical centers in Korea; all patients received at least 1 chemotherapeutic regimen for MCL. RESULTS: The median age for the patients was 63 years (range, 26-78 years), and 77.9% were men. A total of 105 patients (80.1%) had stage III or IV MCL at diagnosis. Fifty-two patients (39.7%) were categorized with high- or high-intermediate risk MCL according to the International Prognostic Index (IPI). Eighteen patients (13.7%) were in the high-risk group according to the simplified MCL-IPI (MIPI). The overall incidence of extranodal involvement was 69.5%. The overall incidence of bone marrow and gastrointestinal involvements at diagnosis was 41.2% and 35.1%, respectively. Cyclophosphamide, doxorubicin, vincristine, prednisolone, and rituximab were used frequently as the first-line treatment (41.2%). With a median follow-up duration of 20.0 months (range, 0.2-77.0 months), the overall survival (OS) at 2 years was 64.7%, while the event-free survival (EFS) was 39.7%. Multivariate analysis showed that the simplified MIPI was significantly associated with OS. However, the use of a rituximab-containing regimen was not associated with OS and EFS. CONCLUSION: Similar to results from Western countries, the current study found that simplified MIPI was an important prognostic factor in Korean patients with MCL.
Bone Marrow ; Cyclophosphamide ; Diagnosis ; Disease-Free Survival ; Doxorubicin ; Drug Therapy ; Epidemiology ; Follow-Up Studies ; Humans ; Incidence ; Korea* ; Lymphoma* ; Lymphoma, Mantle-Cell* ; Male ; Multivariate Analysis ; Prednisolone ; Prognosis ; Retrospective Studies ; Vincristine ; Rituximab