Severe Disease Activity Based on the Paris Classification Is Associated with the Development of Extraintestinal Manifestations in Korean Children and Adolescents with Ulcerative Colitis

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.

Incidentally detected gallbladder agenesis in a child: the importance of identifying anatomic structure

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.

Acute pancreatitis associated with indigo naturalis in pediatric severe Crohn’s disease

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.

Outcome of neonatal palliative procedure for pulmonary atresia with ventricular septal defect or tetralogy of Fallot with severe pulmonary stenosis: experience in a single tertiary center / 소아과

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.

Systemic steroid treatment of acute bronchiolitis: A retrospective study

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.

Clinical Characteristics of Febrile UTI First Developed Over 5 Years of Age

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.

Sleep Problems and Daytime Sleepiness in Children with Nocturnal Enuresis

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.