• Korean Journal of Clinical Pharmacy
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• v.29 no.2
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• pp.79-88
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• 2019

Backgrounds: Inflammatory bowel disease (IBD) including ulcerative colitis (UC) and Crohn's disease (CD) increased prevalence and economic burden. Objectives: This study aimed to investigate drug use pattern in IBD patients in a real world. Methods: National Health Insurance claim data from 2010 to 2014 were used in this population-based study. All IBD patients diagnosed during study period were enrolled. IBD medications included 5-aminosalicylic acid (ASA), glucocorticoid, immunomodulator and anti-tumor necrosis factor-${\alpha}$ agent(anti TNF-${\alpha}$). Growth rate of IBD prevalence, prescribed drug classes, duration of drug therapy and medication cost were analyzed. Number and percentage of patients for categorical variables, and mean and median for continuous variables were presented. Results: Total numbers of patients were 131,158 and 57,286 during 5 years, and their annual growth rate were 3.2 and 5.7% for UC and CD. UC and CD were prevalent in the 40-50 (41.2%) and 20-30 age groups (36.0%). About 60% of IBD patients was prescribed any of medications. 5-ASA was the most frequently prescribed, followed by corticosteroid and immunomodulator. Anti TNF-${\alpha}$ use was the lowest, but 5 times higher than UC in CD. Combination therapies with different class of drugs were in 29% for UC and 62% for CD. Mean prescription days per patient per year were 306 and 378, and the median medication cost per patient per year was KRW 420,000 (USD 383) and KRW 830,000 (USD755), for UC and CD, respectively. Conclusions: Increasing prevalence of IBD requires further studies to contribute to achieve better clinical outcomes of drug therapy.

• Journal of the Korean Child Neurology Society
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• v.26 no.4
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• pp.284-287
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• 2018

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder caused by mutation of one of two genes, TSC1 (encoding hamartin, 9q34) and TSC2 (encoding tuberin, 16p13). It invades the central nervous system and various parts of the body, causing various symptoms. Crohn's disease (CD) is a chronic immune-mediated disease that has not been clearly elucidated. It is thought to be caused by an excessive immune response of the body to bacteria that normally exist in the digestive tract with genetic factors. No cases have been reported in which both of the above-mentioned diseases occurred simultaneously. We report a case of CD in a patient with TSC. A 12-year-old boy was brought to our hospital because of abdominal pain. Skin lesions were observed in the TSC. Fundus examination revealed a hamartoma in the right retina. Brain magnetic resonance imaging revealed a subendothelial giant cell astrocytoma (SEGA). On the basis of these findings, he was diagnosed as having TSC. Blood test results showed increased levels of inflammatory markers. On abdominal ultrasonography, his colon walls were observed to be thickened with increased vascularity of the proximal ascending colon, ileocecal valve, and terminal ileum. Colonoscopy revealed discontinuous ulcerations and inflammations of the ileum, IC valve, and cecum, similar to those found in CD. Everolimus was administered orally for the SEGA but was discontinued frequently owing to the exacerbation of CD. The possibility of CD should be kept in mind in patients with TSC considering to undergo treatment for SEGA.

• Clinical and Experimental Pediatrics
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• v.57 no.11
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• pp.465-471
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• 2014

Inflammatory bowel disease (IBD) is a chronic relapsing disorder of unknown etiology, which is believed to be multifactorial. Recently, the incidence of pediatric IBD has steeply increased in Korea since 2000. Poorly controlled disease activity can result in complications such as intestinal fistulae, abscess, and stricture, as well as growth retardation and delayed puberty in children. Because of a lack of confirmative tests, various diagnostic modalities must be used to diagnose IBD. Onset age, location, behavior, and activity are important in selecting treatments. Monogenic IBD must be excluded among infantile and refractory very-early-onset IBD. Early aggressive therapy using biologics has recently been proposed for peripubertal children to prevent growth failure and malnutrition.

• Korean Journal of Radiology
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• v.22 no.10
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• pp.1640-1649
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• 2021

Objective: Flattening in the anteroposterior direction (AP flattening) of the terminal ileum (TI) or sigmoid colon (SC) lying across the psoas muscle, on magnetic resonance enterography (MRE), might mimic bowel inflammation in the coronal view. This study investigated the prevalence of AP flattening and the factors associated with its development. Materials and Methods: A total of 364 surgery-naïve patients with Crohn's disease (CD) who had undergone MRE were retrospectively reviewed. AP flattening was defined as a luminal collapse in the anteroposterior direction, with a bowel width in the axial plane < 1/4 of the normal diameter without reduction of bowel width in coronal images. The prevalence of AP flattening of the TI and SC on MRE in patients with bowel segments lying across the psoas muscle was determined. We further compared the rate of AP flattening between MRE and computed tomography enterography (CTE) in a subcohort of patients with prior CTE. The factors associated with AP flattening were analyzed using multivariable logistic regression in a subcohort of patients with endoscopic findings of TI. Results: Three hundred and twenty-two and 363 patients, respectively, had TI and SC lying across the psoas muscle. The prevalence of AP flattening on MRE was 7.5% (24/322) in TI and 5.2% (19/363) in SC. The prevalences were significantly higher on MRE than on CTE in both the TI (7.3% [12/164] vs. 0.6% [1/164]; p = 0.003) and SC (5.8% [11/190] vs. 1.6% [3/190]; p = 0.039). AP flattening of the TI was independently and strongly associated with the absence of CD inflammation on endoscopy, with an adjusted odds ratio of 0.066 (p = 0.003) for the presence versus the absence (reference) of inflammation. Conclusion: AP flattening of the TI or SC lying across the psoas muscle was uncommon and predominantly observed on MRE of the bowel without CD inflammation.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.2
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• pp.152-161
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• 2019

Purpose: We examined the prevalence of anemia, annual screening for anemia, and treatment of anemia with iron among children with inflammatory bowel disease (IBD). Methods: A retrospective study of U.S. pediatric patients with IBD was performed in the MarketScan commercial claims database from 2010-2014. Children (ages 1-21) with at least two inpatient or outpatient encounters for IBD who had available lab and pharmacy data were included in the cohort. Anemia was defined using World Health Organization criteria. We used logistic regression to determine differences in screening, incident anemia, and treatment based on age at first IBD encounter and sex. Results: The cohort (n=2,446) included 1,560 Crohn's disease (CD) and 886 ulcerative colitis (UC). Approximately, 85% of CD and 81% of UC were screened for anemia. Among those screened, 51% with CD and 43% with UC had anemia. Only 24% of anemia patients with CD and 20% with UC were tested for iron deficiency; 85% were iron deficient. Intravenous (IV) iron was used to treat 4% of CD and 4% UC patients overall and 8% of those with anemia. Conclusion: At least 80% of children with IBD were screened for anemia, although most did not receive follow-up tests for iron deficiency. The 43%-50% prevalence of anemia was consistent with prior studies. Under-treatment with IV iron points to a potential target for quality improvement.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.26 no.4
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• pp.193-200
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• 2023

Purpose: Crohn's disease (CD) is a chronic, idiopathic bowel disorder that can progress to partial or complete bowel obstruction. At present, there are no reliable diagnostic tests that can readily distinguish between acute inflammatory, purely fibrotic and mixed inflammatory and fibrotic. Our aim is to study the utility of contrast enhanced ultrasound (CEUS) in combination with shear wave elastography (SWE) to differentiate fibrotic from inflammatory strictures in children with obstructive CD of the terminal ileum. Methods: Twenty-five (19 male) children between 2016-2021 with CD of the terminal ileum were recruited into the study. Among these patients, 22 had CEUS kinetic measurements of tissue perfusion, including wash-in slope (dB/sec), peak intensity (dB), time to peak intensity (sec), area under the curve (AUC) (dB sec), and SWE. In total, 11 patients required surgery due to bowel obstruction. Histopathologic analysis was performed by a pathologist who was blinded to the CEUS and SWE test results. Results: Patients that underwent surgical resection had significantly higher mean area under the curve on CEUS compared to patients responsive to medical therapy (p=0.03). The AUC also correlated with the degree of hypertrophy and the percent fibrosis of the muscularis propria, as determined by histopathologic grading (p<0.01). There was no difference in the mean elastography measurements between these two patient groups. Conclusion: CEUS is a useful radiological technique that can help identify pediatric patients with medically refractory obstructive fibrotic strictures of the terminal ileum that should be considered for early surgical resection.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.14 no.1
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• pp.1-25
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• 2011

Inflammatory bowel disease (IBD) develops during childhood or adolescence in approximately 25% of patients with IBD. Recent studies on pediatric IBD have revealed that early-onset IBD has distinct phenotype differences compared to adult onset IBD. Pediatric early-onset IBD differs in many aspects including disease type, location of the lesions, disease behavior, gender preponderance and genetically attributable risks. This review examines the currently published data on the clinical, epidemiological and genetic differences between early-onset and adult-onset IBD. And finally, therapeutic considerations in the management of pediatric-onset IBD are also discussed.

• Journal of Yeungnam Medical Science
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• v.37 no.4
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• pp.332-336
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• 2020

Homozygous mutations in NUDT15 R139C are known as the major factor associated with thiopurine-induced early leukopenia, particularly in Asian patients. Therefore, NUDT15 genotyping is currently recommended before thiopurine treatment to identify patients who are NUDT15 poor metabolizers and consider the use of an alternative immunomodulatory therapy. We report a case of a 12-year-old Korean girl with Crohn's disease (CD), in whom thiopurine-induced leukopenia was prevented by initiation of azathioprine (AZA) therapy at a low dose (0.5 mg/kg/day) and early detection of significant hair loss and white blood cell (WBC) count decrease at 17 days from the start of AZA treatment. The WBC count dropped from 8,970/μL to 3,370/μL in 2 weeks, and AZA treatment was stopped because of concerns of potential leukopenia in the near future. Her WBC count recovered to 5,120/μL after 3 weeks. Gene analysis later revealed that she had a homozygous mutation in NUDT15 R139C, resulting in a poor metabolizing activity of NUDT15. In situations when NUDT15 genotyping is unavailable, initiation of AZA therapy at 0.5 mg/kg/day with close observation of hair loss and WBC counts within 2 weeks may be an alternative way to prevent thiopurine-induced early leukopenia in Asian children with CD.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.15 no.1
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• pp.13-18
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• 2012

The pathogenesis of inflammatory bowel diseases is not very well understood; it is currently thought to be caused by the interaction between genetic factors, environmental factors, intestinal microbes, and immune factors. Biological agents such as anti-tumor necrosis factor (anti-TNF) are widely being used as therapeutic agents. Infliximab, a chimeric monoclonal IgG1 antibody against tumor necrosis factor, has been demonstrated to have an effect in the induction and maintenance of remission in Crohn's disease in children. The effects of biological agents, typified by anti-TNFs, in inflammatory bowel disease in children; the recent concern on the administration of biological agents in combination with immunomodulators; and 'Top-down' therapy are some of the topics covered in this review.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.1
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• pp.50-56
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• 2019

The incidence of pediatric inflammatory bowel disease (IBD) is increasing worldwide, especially in the developing countries. It differs from adult disease in clinical manifestations, especially with regard to genetic predisposition in monogenic IBD. Pediatric disease also have a tendency to show more aggressive inflammation and greater extent of lesion. Newer drugs such as anti-tumor necrosis factor ${\alpha}$ have been known to make a difference in treating pediatric IBD. Recent studies suggested that the patients with high risk factors might have some benefits from earlier use of biologics. To achieve treatment goals such as relieving symptoms, optimizing growth, and improving quality of life while minimizing drug toxicity, more research is needed to develop tools for risk stratification in the use of biologics for pediatric IBD.