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/Aims: Although mucosal healing (MH) is acknowledged as the treatment target in the treat-to-target era, there are limitations on repeated endoscopic examinations, especially in pediatric patients. We aimed to investigate whether fecal calprotectin (FC) could serve as a surrogate marker for the assessment of MH in pediatric patients with Crohn’s disease (CD) who have achieved sustained clinical remission (CR) while treated with anti-tumor necrosis factor (TNF) agents. Methods: This multicenter retrospective cross-sectional study included pediatric CD patients who had sustained a CR for at least 6 months with anti-TNF agents and who simultaneously underwent ileocolonoscopy and FC tests during follow-up. MH was defined as the absence of any ulcer on ileocolonoscopy. Results: A total of 131 patients were included in this study. MH was observed in 87 patients (66.7%). The FC level was significantly lower in patients with MH than in those without MH (median 49.0 mg/kg vs 599.0 mg/kg; p<0.001). According to the multivariate logistic regression analysis, FC was the only factor associated with MH (odds ratio, 0.62; 95% confidence interval [CI], 0.52 to 0.73; p<0.001). According to the receiver operating characteristic curve analysis, the optimal cutoff value for FC for the association with MH was <140 mg/kg (area under the curve 0.890, 95% CI 0.829 to 0.951, sensitivity 78.2%, specificity 88.6%, p<0.001). Conclusions FC was associated with MH in pediatric patients with CD who had achieved a sustained CR for at least 6 months with anti-TNF agents. In these patients, FC can be used to stratify patients and guide decisions regarding ileocolonoscopy in the treat-to-target era.

Purpose: Macular edema including cystoid macular edema is one of the main causes of unfavorable visual outcomes after cataract surgery. The macular thickness and the occurrence of macular edema after uncomplicated cataract surgery was evaluated using optical coherence tomography (OCT) in this study. Methods: Macular map images were taken by OCT before surgery and at 1 week, 1 month, and 2 months postsurgery. The subjects were classified into two groups (group 1, patients with no macular edema; group 2, patients with macular edema). Group 2 was defined as increase in central macular thickness (CMT) by 30% compared with that before surgery. The risk factors for macular edema were evaluated. Group 2 was divided into two subgroups: subclinical macular edema (group 2A) and cystoid macular edema (group 2B) and they were assessed in terms of the clinical course of best-corrected visual acuity and CMT. Results: A total of 376 patients were enrolled in this study, of which 36 (9.57%, group 2) showed macular edema measured by OCT after the surgery. Univariate analysis for group 1 and 2 revealed that intracameral injection of epinephrine during phacoemulsification was associated with the development of macular edema. In group 2, five patients (1.33%) developed cystoid macular edema. Statistically significant differences in the clinical course of CMT were observed at 2 months (201.2 ± 23.1, 250.0 ± 29.8, and 371.0 ± 160.3 in group 1, group 2A, and group 2B, respectively; p < 0.001) and 1 month postoperatively (198.5 ± 23.6, 237.8 ± 40.9, and 314.0 ± 104.5 in group 1, group 2A, and group 2B, respectively; p < 0.001). Group 2B required additional treatment and eventually achieved best-corrected visual acuity of >0.2 with CMT in the normal range. Conclusions The intracameral injection of epinephrine may cause macular edema after uncomplicated cataract surgery. Examination of CMT using OCT is recommended for the early detection of macular edema.

Background: Vedolizumab (VDZ) is currently licensed for use in adults for the treatment of inflammatory bowel disease (IBD). We aimed to investigate the clinical course of pediatric-onset IBD following treatment with VDZ as more than a secondary biologic agent. We also evaluated factors associated with secondary loss of response (LOR) and durability of VDZ treatment. Methods: Pediatric-onset IBD patients diagnosed at an age younger than 18 years who had received VDZ as more than a secondary biologic agent were included in this retrospective observational study conducted at the Department of Pediatrics of two centers in Korea. Comparative analysis was conducted between groups divided according to the development of secondary LOR during VDZ treatment. Results: A total of 24 patients comprising 10 patients with Crohn’s disease and 14 with ulcerative colitis were included. Of these, 19 were male and 5 were female. The mean age at diagnosis was 14.6 ± 2.5 years. The mean age at initiation of VDZ was 20.5 ± 2.8 years. Nine patients (37.5%) had received two or more biologic agents before starting VDZ. During a median of 0.9 years follow-up from VDZ initiation, 9 patients (37.5%) experienced LOR requiring interval shortening and 4 patients (16.7%) were changed to a different biologic agent. According to multivariate Cox proportional hazard regression analysis, administration of two or more biologic agents before VDZ treatment was the only factor positively associated with LOR (hazard ratio [HR], 5.6; 95% confidence interval [CI], 1.026–30.56; P = 0.047), while LOR was the only factor negatively associated with VDZ durability (HR, 0.003; 95% CI, 0.00–0.08; P = 0.010). No adverse events were observed during treatment with VDZ. Conclusion VDZ is safe and efficacious for the treatment of pediatric-onset IBD patients failing a primary biologic agent. The durability of VDZ may be enhanced by introducing VDZ earlier in the disease course. Further prospective studies in children are required in the future to validate these findings.

Activated phosphoinositide 3-kinase δ syndrome (APDS)1 is caused by gain-of-function mutations in PIK3CD, which encodes the catalytic p110δ subunit of phosphoinositide 3 kinase. We describe three patients with APDS1, the first thereof in Korea. Therein, we investigated clinical manifestations of APDS1 and collected data on the efficacy and safety profile of sirolimus, a mammalian target of rapamycin inhibitor and pathway-specific targeted medicine. The same heterozygous PIK3CD mutation was detected in all three patients (E1021K). After genetic diagnosis, all patients received sirolimus and experienced an excellent response, including amelioration of lymphoproliferation and improvement of nodular mucosal lymphoid hyperplasia in the gastrointestinal tract. The median trough level of sirolimus was 5.5 ng/mL (range, 2.8–7.5) at a dose of 2.6–3.6 mg/m2. Two patients who needed highdose, short-interval, immunoglobulin-replacement treatment (IGRT) had a reduced requirement for IGRT after initiating sirolimus, and the dosing interval was extended from 2 and 3 weeks to 4 weeks. The IgG trough level after sirolimus treatment (median, 594 mg/dL; range, 332–799 mg/dL) was significantly higher than that before sirolimus treatment (median, 290 mg/dL; range, 163–346 mg/dL) (p<0.001). One episode of elevated serum creatinine with a surge of sirolimus (Patient 2) and episodes of neutropenia and oral stomatitis (Patient 1) were observed. We diagnosed the first three patients with APDS1 in Korea. Low-dose sirolimus may alleviate clinical manifestations thereof, including hypogammaglobulinemia.

Abscess ; Adolescent ; Body Mass Index ; Child ; Classification ; Colonic Diseases ; Crohn Disease ; Diagnosis ; Europe ; Fistula ; Humans ; Inflammatory Bowel Diseases ; Korea ; Male ; Pediatrics ; Phenotype ; Retrospective Studies

Background: Submental intubation is commonly used during general anesthesia for maxillofacial surgeries as it provides a safe unrestricted surgical access compared to tracheostomy. During submental intubation, soft tissues and blood clots can become lodged in the endotracheal tube. To overcome this problem, we used a laparoscopic trocar.Case: A 52-year-old man with maxillofacial injury was scheduled to undergo an open reduction and internal fixation. We performed submental intubation using laparoscopic trocar, which created sufficient space for the insertion of the endotracheal tube. Unlike conventional methods, our method did not require any blunt dissection and caused significantly less soft tissue damage and required significantly less time. Conclusions Submental intubation with laparoscopic trocar is a one-step method and is quick and easy-to-perform technique with less complications.

BACKGROUND: Recently, endovascular aortic repair (EVAR) and thoracic endovascular aortic repair (TEVAR), have been used for treatment of thoracic and abdominal aortic aneurysms. The purpose of this study was to analyze the outcome and predictors for 30-day mortality and complications, in patients that underwent EVAR and/or TEVAR under general anesthesia. METHODS: In this study, 151 cases of EVAR and/or TEVAR under general anesthesia in 140 patients during 2009–2017 were studied. The primary outcome was 30-day mortality after surgery. Multivariate logistic regression analysis was used, to clarify risk for postoperative 30-day mortality. RESULTS: Postoperative 30-day mortality rate was 9.9% in the study population (10.3% in EVAR, and 9.3% in TEVAR, respectively). Seventy-two cases (47.7%) experienced postoperative complications within 30 days. Elderly older than age 76.5 (odds ratio [ORs] = 48.89, 95% confidential interval [95% CI] 1.40–1,710.25, P = 0.032), technically expertness (OR = 0.01, 95% CI 0.00–0.40, P = 0.013), severity of systemic complications (OR = 23.24, 95% CI, 2.27–238.24, P = 0.008), and severity of local-vascular complications (OR = 31.87, 95% CI, 1.29–784.66, P = 0.034) were significantly associated with 30-day mortality. CONCLUSIONS: This study revealed that elderly, technically expertness, and severity of systemic and local-vascular complications were associated with 30-day mortality of EVAR and TEVAR in aortic aneurysm.
Aged ; Anesthesia, General ; Aortic Aneurysm ; Aortic Aneurysm, Abdominal ; Humans ; Length of Stay ; Logistic Models ; Mortality ; Postoperative Complications ; Risk Factors ; Treatment Outcome

BACKGROUND: We assessed the frequency and severity of hypoglycemia in type 2 diabetes mellitus patients treated with sulfonylurea monotherapy or sulfonylurea+metformin. METHODS: We conducted a retrospective, observational, cross-sectional study in 2011 and 2012 including patients with type 2 diabetes mellitus aged ≥30 years who were treated with ≥6 months of sulfonylurea monotherapy or sulfonylurea+metformin at 20 university-affiliated hospitals in Korea. At enrollment, glycated hemoglobin (HbA1c) was assessed; participants completed self-reported questionnaires describing hypoglycemia incidents over the past 6 months. A review of medical records up to 12 months before enrollment provided data on demographics, disease history, comorbidities, laboratory results, and drug usage. RESULTS: Of 726 enrolled patients, 719 were included (55.6% male); 31.7% and 68.3% were on sulfonylurea monotherapy and sulfonylurea+metformin, respectively. Mean±standard deviation age was 65.9±10.0 years; mean HbA1c level was 7.0%±1.0%; 77.8% of patients had hypertension (89.4% used antihypertensive medication); 60.5% had lipid disorders (72.5% used lipid-lowering medication); and 52.0% had one or more micro- or macrovascular diseases. Among patients with A1c measurement (n=717), 56.4% achieved therapeutic goals (HbA1c <7.0%); 42.4% (305/719) experienced hypoglycemia within 6 months of enrollment; and 38.8%, 12.9%, 12.7%, and 3.9% of patients experienced mild, moderate, severe, and very severe hypoglycemia symptoms, respectively. Several reported hypoglycemia frequency as 1–2 times over the last 6 months. The mean number of very severe hypoglycemia episodes was 3.5±5.5. CONCLUSION: Among type 2 diabetes mellitus patients treated with sulfonylurea-based regimens, glycemic levels were relatively well controlled but hypoglycemia remained a prevalent side effect.
Comorbidity ; Cross-Sectional Studies ; Demography ; Diabetes Mellitus, Type 2 ; Hemoglobin A, Glycosylated ; Humans ; Hypertension ; Hypoglycemia ; Korea ; Medical Records ; Metformin ; Retrospective Studies

PURPOSE: The incidence of inflammatory bowel disease (IBD) is rapidly increasing, and several reports have described the renal complications of IBD. We sought to evaluate the clinical manifestations of renal complications in children with IBD in order to enable early detection and prompt treatment of the complications. METHODS: We retrospectively reviewed the medical records of 456 children and adolescents aged < 20 years who had been diagnosed with IBD since 2000. We analyzed patient age, sex, medication use, IBD disease activity, and clinical manifestations of renal symptoms. RESULTS: Our study comprising 456 children with IBD included 299 boys (65.6%) and 157 girls (34.4%). The study included 346 children with Crohn disease and 110 children with ulcerative colitis. The incidence of kidney-related symptoms was 14.7%, which was significantly higher than that in normal children. We observed 26 children (38.8%) with isolated hematuria, 30 children (44.8%) with isolated proteinuria, and 11 children (16.4%) with hematuria and concomitant proteinuria. A renal biopsy was performed in 7 children. Histopathological examination revealed immunoglobulin A nephropathy in 5 children (71.4%). All children presented with mild disease and well-controlled disease activity of IBD. CONCLUSION: Children with IBD are more likely to show kidney-related symptoms than healthy children and adolescents are. Therefore, regular screening of urine and evaluation of renal function in such children are necessary for early detection of renal complications.
Adolescent* ; Biopsy ; Child* ; Colitis, Ulcerative ; Crohn Disease ; Female ; Glomerulonephritis, IGA ; Hematuria ; Humans ; Incidence ; Inflammatory Bowel Diseases* ; Kidney ; Mass Screening ; Medical Records ; Proteinuria ; Retrospective Studies