Is it feasible and effective to provide faculty development programs online for clinical teachers? / 한국의학교육

Purpose@#Seoul National University College of Medicine operates a faculty development program for clinical teachers at multiple affiliated teaching hospitals. In 2020, the program was moved online due to coronavirus disease 2019. The purpose of this study was to determine whether it is feasible and effective to provide faculty development programs online in terms of clinical teachers’ participation and satisfaction in comparison with offline programs. @*Methods@#Clinical teachers participated in the clinical teaching methods programs offline in 2019 and online in 2020. We analyzed participation rate and satisfaction level. All surveys items were rated on a 5-point Likert scale. We also interviewed instructors about the advantages and drawbacks of the online program. @*Results@#The participation rate of the online program (89.5%) was significantly higher than that of the offline program (67.8%). The overall satisfaction level for the online program (4.37) was similar to that for the offline program (4.50). @*Conclusion@#Faculty development programs online are feasible and effective in medical education. We need to design training content that fits online programs, consider various online training methods to reinforce the strengths of online programs, and support participants to make good use of these programs.

Is it feasible and effective to provide faculty development programs online for clinical teachers? / 한국의학교육

Purpose@#Seoul National University College of Medicine operates a faculty development program for clinical teachers at multiple affiliated teaching hospitals. In 2020, the program was moved online due to coronavirus disease 2019. The purpose of this study was to determine whether it is feasible and effective to provide faculty development programs online in terms of clinical teachers’ participation and satisfaction in comparison with offline programs. @*Methods@#Clinical teachers participated in the clinical teaching methods programs offline in 2019 and online in 2020. We analyzed participation rate and satisfaction level. All surveys items were rated on a 5-point Likert scale. We also interviewed instructors about the advantages and drawbacks of the online program. @*Results@#The participation rate of the online program (89.5%) was significantly higher than that of the offline program (67.8%). The overall satisfaction level for the online program (4.37) was similar to that for the offline program (4.50). @*Conclusion@#Faculty development programs online are feasible and effective in medical education. We need to design training content that fits online programs, consider various online training methods to reinforce the strengths of online programs, and support participants to make good use of these programs.

Establishing a Patient-centered Longitudinal Integrated Clerkship:Early Results from a Single Institution

Background@#Longitudinal integrated clerkships (LICs) have been adopted by medical schools to overcome the limitations of traditional block clerkship rotations and to promote continuity of care. In 2018, Seoul National University College of Medicine introduced a patient-centered LIC program as part of a new curriculum in parallel with traditional block rotation clerkships. The purpose of this study was to present the patient-centered LIC program and to investigate its educational effects. @*Methods@#In 2018 and 2019, a total of 298 third-year medical students participated in the LIC program. We divided the students into groups of eight, which were organized into corresponding discussion classes. Throughout the academic year, students followed up patients by interviewing them at the hospital or reviewing their electric medical records.Discussion classes on set topics were held seven times per year with facilitators and clinical faculties. Students completed a course evaluation questionnaire at the end of the academic year. The questionnaire included 22 items measured on a 5-point scale and two open-ended questions asking about the benefits and limitations of the program. The items covered three domains: student experience, satisfaction, and self-assessment. Final reflective essays were collected as both student assessments and data for qualitative analysis. @*Results@#During the study period, the overall experience of the students improved. We increased the number of faculty members and patients and decreased the number of students in each discussion class. We also provided additional feedback through an e-portfolio.Students' satisfaction changed positively. Compared to the rotational clerkship, students answered that the LIC provided additional help in learning the two core competencies.During the first 2 years of the program, the percentage of students who answered that the program was more helpful than the rotational clerkship increased from 23.7% to 46.4% for continuity of care (P < 0.001), and from 20.5% to 50.7% for patient-centered care (P < 0.001). @*Conclusion@#Our patient-centered LIC, in parallel with traditional block rotation clerkships, had a positive effect on students' experience of continuity of care and patient-centered care.

Successful Treatment of a Korean Infant with Giant Cell Hepatitis with Autoimmune Hemolytic Anemia Using Rituximab / 대한소아소화기영양학회지

Giant cell hepatitis with autoimmune hemolytic anemia (AHA) is a rare disease of infancy characterized by the presence of both Coombs-positive hemolytic anemia and progressive liver disease with giant cell transformation of hepatocytes. Here, we report a case involving a seven-month-old male infant who presented with AHA followed by cholestatic hepatitis. The clinical features included jaundice, pallor, and red urine. Physical examination showed generalized icterus and splenomegaly. The laboratory findings suggested warm-type AHA with cholestatic hepatitis. Liver biopsy revealed giant cell transformation of hepatocytes and moderate lobular inflammation. The patient was successfully treated with four doses of rituximab. Early relapse of hemolytic anemia and hepatitis was observed, which prompted the use of an additional salvage dose of rituximab. He is currently in clinical remission.

Establishing a Patient-centered Longitudinal Integrated Clerkship:Early Results from a Single Institution

Background@#Longitudinal integrated clerkships (LICs) have been adopted by medical schools to overcome the limitations of traditional block clerkship rotations and to promote continuity of care. In 2018, Seoul National University College of Medicine introduced a patient-centered LIC program as part of a new curriculum in parallel with traditional block rotation clerkships. The purpose of this study was to present the patient-centered LIC program and to investigate its educational effects. @*Methods@#In 2018 and 2019, a total of 298 third-year medical students participated in the LIC program. We divided the students into groups of eight, which were organized into corresponding discussion classes. Throughout the academic year, students followed up patients by interviewing them at the hospital or reviewing their electric medical records.Discussion classes on set topics were held seven times per year with facilitators and clinical faculties. Students completed a course evaluation questionnaire at the end of the academic year. The questionnaire included 22 items measured on a 5-point scale and two open-ended questions asking about the benefits and limitations of the program. The items covered three domains: student experience, satisfaction, and self-assessment. Final reflective essays were collected as both student assessments and data for qualitative analysis. @*Results@#During the study period, the overall experience of the students improved. We increased the number of faculty members and patients and decreased the number of students in each discussion class. We also provided additional feedback through an e-portfolio.Students' satisfaction changed positively. Compared to the rotational clerkship, students answered that the LIC provided additional help in learning the two core competencies.During the first 2 years of the program, the percentage of students who answered that the program was more helpful than the rotational clerkship increased from 23.7% to 46.4% for continuity of care (P < 0.001), and from 20.5% to 50.7% for patient-centered care (P < 0.001). @*Conclusion@#Our patient-centered LIC, in parallel with traditional block rotation clerkships, had a positive effect on students' experience of continuity of care and patient-centered care.

Endoscopic Management of Pancreaticopleural Fistula in a Child with Hereditary Pancreatitis / 대한소아소화기영양학회지

Pancreaticopleural fistula (PPF) a fistulous connection between the pancreas and pleural space due to prolonged chronic pancreatitis (CP). PPF is a very rare complication which presents in 0.4% of chronic pancreatitis cases, especially among children. We report a case involving a 3-year-old boy who presented with pleural effusion caused by a PPF, a complication of hereditary pancreatitis, which was, for the first time in Korea, successfully managed with endoscopic treatment. Chest radiography and computed tomography showed massive pleural effusion. Percutaneous catheter drainage was performed. High amylase levels were observed in the pleural fluid and serum, suggesting PPF. The patient was managed with bowel rest and octreotide infusion. Endoscopic retrograde cholangiopancreatography revealed CP, and pleural effusion was successfully managed with stent placement. PRSS1 genetic screening revealed R122H mutation.

Use of Anti-TNF Alpha Blockers Can Reduce Operation Rate and Lead to Growth Gain in Pediatric Crohn's Disease / 대한소아소화기영양학회지

PURPOSE: Pediatric Crohn's disease (CD) is directly related to growth and has a high probability of requiring surgical intervention(s); therefore, more active treatment for CD is required for children. This study investigated the impact of biologics on growth and disease course associated with surgery. METHODS: This was a retrospective cohort study involving patients diagnosed with CD at the Seoul National University Children's Hospital (Seoul, Korea) between January 2006 and October 2017. The aim was to determine the characteristics of pediatric patients with CD and whether biologics affected growth and the surgical disease course. RESULTS: Among patients who underwent surgery for CD, the mean number of operations per patient was 1.89. The mean time from initial diagnosis to surgery was 19.3 months. The most common procedure was fistulectomy (34%), followed by incision and drainage (25%). In all patients, the use of biologics increased the height (p=0.002) and body mass index (BMI) (p=0.005). Among patients who underwent surgery, height (p=0.004) and BMI (p=0.048) were increased in the group using biologics. Patients who used biologics exhibited a low operation rate only within 2 years after diagnosis, with no differences thereafter (p=0.027). CONCLUSION: Although biologics could not mitigate the operation rate in pediatric patients who underwent surgery for CD, biological therapy delayed disease progression within 2 years of disease onset. Additionally, biologics conferred growth and BMI benefits in this window period. Therefore, it may be helpful to use biologics for optimal growth in pediatric patients with a high probability of undergoing future surgery.

Heat shock protein 70 increases cell proliferation, neuroblast differentiation, and the phosphorylation of CREB in the hippocampus / 한국실험동물학회지

In the present study, we investigated the effects of heat shock protein 70 (HSP70) on novel object recognition, cell proliferation, and neuroblast differentiation in the hippocampus. To facilitate penetration into the blood–brain barrier and neuronal plasma membrane, we created a Tat-HSP70 fusion protein. Eight-week-old mice received intraperitoneal injections of vehicle (10% glycerol), control-HSP70, or Tat-HSP70 protein once a day for 21 days. To elucidate the delivery efficiency of HSP70 into the hippocampus, western blot analysis for polyhistidine was conducted. Polyhistidine protein levels were significantly increased in control-HSP70- and Tat-HSP70-treated groups compared to the control or vehicle-treated group. However, polyhistidine protein levels were significantly higher in the Tat-HSP70-treated group compared to that in the control-HSP70-treated group. In addition, immunohistochemical study for HSP70 showed direct evidences for induction of HSP70 immunoreactivity in the control-HSP70- and Tat-HSP70-treated groups. Administration of Tat-HSP70 increased the novel object recognition memory compared to untreated mice or mice treated with the vehicle. In addition, the administration of Tat-HSP70 significantly increased the populations of proliferating cells and differentiated neuroblasts in the dentate gyrus compared to those in the control or vehicle-treated group based on the Ki67 and doublecortin (DCX) immunostaining. Furthermore, the phosphorylation of cAMP response element-binding protein (pCREB) was significantly enhanced in the dentate gyrus of the Tat-HSP70-treated group compared to that in the control or vehicle-treated group. Western blot study also demonstrated the increases of DCX and pCREB protein levels in the Tat-HSP70-treated group compared to that in the control or vehicle-treated group. In contrast, administration of control-HSP70 moderately increased the novel object recognition memory, cell proliferation, and neuroblast differentiation in the dentate gyrus compared to that in the control or vehicle-treated group. These results suggest that Tat-HSP70 promoted hippocampal functions by increasing the pCREB in the hippocampus.

Deliberate Practice as an Effective Remediation Strategy for Underperforming Medical Students Focused on Clinical Skills: a Prospective Longitudinal Study

BACKGROUND: It is critical to develop remedial education for underperforming medical students, but little is known about how to create an effective remediation program. Deliberate practice (DP) is a structured and reflective activity that is designed to optimize performance. Here we applied the concept of DP to create remedial education to improve the clinical practices of medical students. We also analyzed the effectiveness of the remediation program. METHODS: Based on the expert performance approach of DP, we designed a 4-week remedial program for clinical performance that included feedback and reflection. There were 74 student participants in this program from 2014 to 2017. Their clinical performance was re-evaluated after completion, and changes in their clinical performance scores were analyzed. RESULTS: Students who completed the remediation program showed significant improvements in clinical performance scores (P < 0.001). Most students found the program to be instructive and helpful for improving their clinical performance. They reported that role play with peers was the most helpful for improving their skills. CONCLUSION: The DP-based remediation program improved the clinical performance of failing medical students. This remediation program should continue to be offered to underperforming students to ensure that medical school graduates are competent.

Association Between Vitamin D Deficiency and Suspected Nonalcoholic Fatty Liver Disease in an Adolescent Population / 대한소아소화기영양학회지

PURPOSE: Vitamin D deficiency is a condition widespread throughout the world. Recent studies have suggested that vitamin D deficiency was associated with obesity and metabolic syndrome. The purpose of the study was to examine the relationship between vitamin D deficiency and nonalcoholic fatty liver disease (NAFLD) in adolescents. METHODS: The data were obtained from the Korean National Health and Nutrition Examination Survey from 2008–2014. A total of 3,878 adolescents were included in the study. Vitamin D deficiency was defined as a 25-hydroxyvitamin D concentration 30 U/L. RESULTS: Vitamin D deficiency was noted in 78.9% of the studied population. Age, body mass index, waist circumference, and blood pressure, glucose, cholesterol, and triglyceride levels were significantly higher in adolescents with suspected NAFLD than in adolescents without suspected NAFLD, while the mean vitamin D level was significantly lower in adolescents with suspected NAFLD. The multivariate-adjusted odds of suspected NAFLD were higher with increased age, male gender, obesity, and metabolic syndrome. Individuals with vitamin D deficiency were at higher risk of suspected NAFLD (odds ratio, 1.77; 95% confidence interval, 1.07–2.95) after adjusting for age, gender, obesity, and metabolic syndrome. CONCLUSION: Vitamin D deficiency was associated with suspected NAFLD, independent of obesity and metabolic syndrome, in adolescents.

Changes of myelin basic protein in the hippocampus of an animal model of type 2 diabetes / 한국실험동물학회지

In this study, we observed chronological changes in the immunoreactivity and expression level of myelin basic protein (MBP), one of the most abundant proteins in the central nervous system, in the hippocampus of Zucker diabetic fatty (ZDF) rats and their control littermates (Zucker lean control; ZLC). In the ZLC group, body weight steadily increased with age; the body weight of the ZDF group, however, peaked at 30 weeks of age, and subsequently decreased. Based on the changes of body weight, animals were divided into the following six groups: early (12-week), middle (30-week), and chronic (52-week) diabetic groups and their controls. MBP immunoreactivity was found in the alveus, strata pyramidale, and lacunosum-moleculare of the CA1 region, strata pyramidale and radiatum of the CA3 region, and subgranular zone, polymorphic layer, and molecular layer of the dentate gyrus. MBP immunoreactivity was lowest in the hippocampus of 12-week-old rats in the ZLC group, and highest in 12-week-old rats in the ZDF group. Diabetes increased MBP levels in the 12-week-old group, while MBP immunoreactivity decreased in the 30-week-old group. In the 52-week-old ZLC and ZDF groups, MBP immunoreactivity was detected in the hippocampus, similar to the 30-week-old ZDF group. Western blot results corroborated with immunohistochemical results. These results suggested that changes in the immunoreactivity and expression of MBP in the hippocampus might be a compensatory response to aging, while the sustained levels of MBP in diabetic animals could be attributed to a loss of compensatory responses in oligodendrocytes.

Bacopa monnieri extract improves novel object recognition, cell proliferation, neuroblast differentiation, brain-derived neurotrophic factor, and phosphorylation of cAMP response element-binding protein in the dentate gyrus / 한국실험동물학회지

Bacopa monnieri is a medicinal plant with a long history of use in Ayurveda, especially in the treatment of poor memory and cognitive deficits. In the present study, we hypothesized that Bacopa monnieri extract (BME) can improve memory via increased cell proliferation and neuroblast differentiation in the dentate gyrus. BME was administered to 7-week-old mice once a day for 4 weeks and a novel object recognition memory test was performed. Thereafter, the mice were euthanized followed by immunohistochemistry analysis for Ki67, doublecortin (DCX), and phosphorylated cAMP response element-binding protein (CREB), and western blot analysis of brain-derived neurotrophic factor (BDNF). BME-treated mice showed moderate increases in the exploration of new objects when compared with that of familiar objects, leading to a significant higher discrimination index compared with vehicle-treated mice. Ki67 and DCX immunohistochemistry showed a facilitation of cell proliferation and neuroblast differentiation following the administration of BME in the dentate gyrus. In addition, administration of BME significantly elevated the BDNF protein expression in the hippocampal dentate gyrus, and increased CREB phosphorylation in the dentate gyrus. These data suggest that BME improves novel object recognition by increasing the cell proliferation and neuroblast differentiation in the dentate gyrus, and this may be closely related to elevated levels of BDNF and CREB phosphorylation in the dentate gyrus.

A Multicenter Study of the Antiviral Efficacy of Entecavir Monotherapy Compared to Lamivudine Monotherapy in Children with Nucleos(t)ide-naïve Chronic Hepatitis B

BACKGROUND: The aim of this study was to compare the long-term efficacy of entecavir (ETV) and lamivudine (LAM) therapy in children with chronic hepatitis B (CHB) who had not received nucleoside analogue treatment. METHODS: In this multicenter, retrospective study, we included pediatric CHB patients younger than 20 years who received ETV or LAM treatment for at least 12 months and had no concomitant diseases. All of the patients were followed up every 1 to 3 months. At each visit, the patients underwent clinical evaluation and biochemical testing. RESULTS: Eight (53.3%), 14 (93.3%), and 2 (15.4%) of the ETV-treated patients achieved virologic suppression, alanine aminotransferase (ALT) normalization and hepatitis B e antigen (HBeAg) seroconversion, respectively, at 1 year. In the ETV group, the cumulative rate of virologic suppression at 3 years was 91.7%, which was significantly higher than that in the LAM group (P < 0.001). The mean duration of treatment before virologic suppression was shorter in the ETV group than in the LAM group (P = 0.040). The cumulative rate of seroconversion in the ETV group at 3 years was 39.4%, which was not significantly different from that in the LAM group (P = 0.439). The ETV group showed lower cumulate rates of virologic breakthrough (33.3% at 6 years) and genotypic mutation than the LAM group (P = 0.033 and P = 0.011, respectively). CONCLUSION: ETV is superior to LAM in pediatric CHB treatment because of its higher virologic suppression rate and lower cumulative rates of virologic breakthrough and genotypic mutation.

Higher Morbidity of Monogenic Inflammatory Bowel Disease Compared to the Adolescent Onset Inflammatory Bowel Disease / 대한소아소화기영양학회지

PURPOSE: Monogenic inflammatory bowel disease (IBD) patients do not respond to conventional therapy and are associated with a higher morbidity. We summarized the clinical characteristics of monogenic IBD patients and compared their clinical outcomes to that of non-monogenic IBD patients. METHODS: We performed a retrospective cohort study of all children <18 years old who were diagnosed with IBD between 2005 and 2016. A total of 230 children were enrolled. Monogenic IBD was defined as a presentation age less than 6 years old with confirmation of a genetic disorder. We subdivided the groups into monogenic IBD (n=18), non-monogenic very early-onset IBD (defined as patients with a presentation age <6 years old without a confirmed genetic disorder, n=12), non-monogenic IBD (defined as all patients under 18 years old excluding monogenic IBD, n=212), and severe IBD (defined as patients treated with an anti-tumor necrosis factor excluding monogenic IBD, n=92). We compared demographic data, initial pediatric Crohn disease activity index/pediatric ulcerative colitis activity index (PCDAI/PUCAI) score, frequency of hospitalizations, surgical experiences, and height and weight under 3rd percentile among the patients enrolled. RESULTS: The initial PCDAI/PUCAI score (p < 0.05), incidence of surgery per year (p < 0.05), and hospitalization per year (p < 0.05) were higher in the monogenic IBD group than in the other IBD groups. Additionally, the proportion of children whose weight and height were less than the 3rd percentile (p < 0.05 and p < 0.05, respectively) was also higher in the monogenic IBD group. CONCLUSION: Monogenic IBD showed more severe clinical manifestations than the other groups.

An Outbreak of Measles in a University in Korea, 2014

Measles has been declared eliminated from the Korea since 2006. In April 2014, a measles outbreak occurred at a University in Seoul. A total of 85 measles cases were identified. In order to estimate vaccine effectiveness of measles vaccine, we reviewed the vaccination records of the university students. The vaccine effectiveness of two doses of measles containing vaccine was 60.0% (95% CI, 38.2–74.1; P < 0.05). Transmission was interrupted after the introduction of outbreak-response immunization. The outbreak shows that pockets of under-immunity among college students may have facilitated the disease transmission despite the high 2-dose vaccination coverage in the community.

Immunohistochemical localization of glucose transporter 1 and 3 in the scrotal and abdominal testes of a dog / 한국실험동물학회지

Glucose is essential for testicular function; the uptake of carbohydrate-derived glucose by cells is mediated by glucose transporters (GLUTs). In the present study, we investigated the activity of GLUT1 and GLUT3, the two main isoforms of GLUTs found in testes, in the left scrotal and right abdominal testes of a German Shepherd dog. Immunohistochemical analysis showed that GLUT1 immunoreactivity was absent in the scrotal and abdominal testes. In contrast, weak to moderate GLUT3 immunoreactivity was observed in mature spermatocytes as well as spermatids in the scrotal testis. In the abdominal testis, relatively strong GLUT3 immunoreactivity was detected in Leydig cells only and was absent in mature spermatocytes and spermatids. GLUT3 immunoreactivity was significantly decreased in the tubular region of abdominal testis and significantly increased in the extra-tubular (interstitial) region of abdominal testis compared to observations in the each region of scrotal testis, respectively. These results suggest that GLUT3 is the major glucose transporter in the testes and that abdominal testes may increase the uptake of glucose into interstitial areas, leading to an increased risk of developing cancer.

Epidemiology of Biliary Atresia in Korea

Biliary atresia (BA) is the major cause of cholestasis and the leading indication for liver transplantation (LT). However, the incidence of BA in Korea has not been reported. The aim of this study was to investigate the incidence and clinical outcomes of BA in Korea. We used the Korean universal health insurance database and extracted data regarding BA patients younger than 18 years of age admitted between 2011 and 2015. The incidence of BA was calculated by dividing the number of BA patients by the number of live births. Two hundred forty infants were newly diagnosed with BA. A total of 963 BA patients younger than 18 years of age were followed up for 5 years. The overall incidence of BA was 1.06 cases per 10,000 live births. The incidence of BA was 1.4 times higher for female patients than for male patients. Additionally, significant seasonal variation was observed; in particular, the incidence of BA was 2 times higher from June through August than from December through February. Congenital anomalies were found in 38 of 240 patients (15.8%). Congenital heart diseases were major associated congenital anomalies (6.3%). Several complications developed during the study period, including cholangitis (24.0%), varix (6.2%), and gastrointestinal bleeding (4.4%). Three hundred and one of the 963 BA patients under 18 years of age (31.3%) received LT for BA. The incidence of BA is higher in Korea than that in Western countries. We also report significant gender-associated differences and seasonal variation with respect to the incidence of BA.

Dendropanax morbifera Léveille extract ameliorates D-galactose-induced memory deficits by decreasing inflammatory responses in the hippocampus / 한국실험동물학회지

In the present study, we examined the effects of Dendropanax morbifera Léveille leaf extract (DML) on D-galactose-induced morphological changes in microglia and cytokines, including pro-inflammatory cytokines (interleukin [IL]-1β, IL-6, and tumor necrosis factor [TNF]-α) and anti-inflammatory cytokines (IL-4 and IL-10) in the hippocampus. Administration of DML to D-galactose-treated mice significantly improved D-galactose-induced reduction in escape latency, swimming speed, and spatial preference for the target quadrant. In addition, administration of DML to D-galactose-treated mice significantly ameliorated the microglial activation and increases of IL-1β, IL-6, and TNF-α levels in the hippocampus. Administration of D-galactose significantly reduced IL-4 levels in the hippocampus, while administration of DML to D-galactose-treated mice significantly increased IL-4 level. However, we did not observe any significant changes in IL-10 levels in hippocampal homogenates. These results suggest that DML reduces D-galactose-induced mouse senescence by reducing pro-inflammatory cytokines such as IL-1β, IL-6, and TNF-α, as well as increasing anti-inflammatory cytokine IL-4.

Effect of the Baseline Vitamin D Level on Growth Outcome in Pediatric Crohn Disease / 대한소아소화기영양학회지

PURPOSE: Vitamin D deficiency is common in Crohn disease (CD). The aim of the study was to examine the prevalence of vitamin D deficiency and evaluate the association between vitamin D status and growth outcome in Korean pediatric CD patients. METHODS: In this retrospective study, 17 children younger than 18 years old diagnosed with CD were enrolled and their serum 25-hydroxy vitamin D (25[OH]D) was checked between 2011 and 2015. We categorized the patients into two groups, Group 1 and Group 2. Group 1 included patients with serum 25(OH)D levels below 10 ng/mL, and Group 2 was for patients with a 25(OH)D serum levels between 10 ng/mL and 30 ng/mL. The z-scores for height (Htz), weight (Wtz), and body mass index (BMIz) were measured at baseline, 6 months, and 12 months. RESULTS: The mean serum 25(OH)D levels of the total 65 CD patients and 17 enrolled patients were 15.64±6.9 ng/mL and 13.1±5.1 ng/mL, respectively. There was no correlation at the beginning of the study between vitamin D level and growth parameters (Htz, Wtz, BMIz) or other variables including laboratory data and Pediatric Crohn Disease Activity Index. The Htz, Wtz, and BMIz in Group 1 showed no significant improvement at 6 months and 12 months follow-up. In Group 2, Wtz and BMIz showed significant improvements sustained until 12 months of follow-up. Htz showed no significant improvement at 6 months but there was significant improvement at 12 months. CONCLUSION: It seems that baseline vitamin D status affects growth outcome in pediatric CD.

A Novel Homozygous LIPA Mutation in a Korean Child with Lysosomal Acid Lipase Deficiency / 대한소아소화기영양학회지

Patients with lysosomal acid lipase (LAL) deficiency and glycogen storage disease (GSD) demonstrated hepatomegaly and dyslipidemia. In our case, a 6-year-old boy presented with hepatosplenomegaly. At 3 years of age, GSD had been diagnosed by liver biopsy at another hospital. He showed elevated serum liver enzymes and dyslipidemia. Liver biopsy revealed diffuse microvesicular fatty changes in hepatocytes, septal fibrosis and foamy macrophages. Ultrastructural examination demonstrated numerous lysosomes that contained lipid material and intracytoplasmic cholesterol clefts. A dried blood spot test revealed markedly decreased activity of LAL. LIPA gene sequencing identified the presence of a novel homozygous mutation (p.Thr177Ile). The patient's elevated liver enzymes and dyslipidemia improved with enzyme replacement therapy. This is the first report of a Korean child with LAL deficiency, and our findings suggest that this condition should be considered in the differential diagnosis of children with hepatosplenomegaly and dyslipidemia.