Clinical features and pulmonary function in children with Swyer-James-Macleod syndrome: A single center experience

Purpose@#Swyer-James-Macleod syndrome (SJMS) is a rare disorder characterized by the hyperlucency of a unilateral lung due to ipsilaterally arrested lung growth. No sufficient information on progressive lung function changes during growth is available in SJMS. @*Methods@#We retrospectively reviewed SJMS cases using a clinical database from January 2000 to August 2018. @*Results@#A total of 40 patients were enrolled in this study. The median age of the patients was 4.8 years (interquartile range [IQR], 2.7–9.0 years), boys (n = 21, 52.5%) were more affected, and left lungs (n = 27, 67.5%) were more involved. The interval between infection and diagnosis was 1.8 years (IQR, 0.7–6.4 years). Mycoplasma pneumoniae (n = 14) and adenovirus (n = 5) were most prevalent among the 21 subjects with presumably identifiable causes. Most of the 16 patients with available pulmonary function data presented moderate obstructive lung function (z-score, median; FEV 1: -4.320 [IQR, -5.955 to -3.400] and FVC: -2.600 [IQR, -3.590 to -1.570], respectively) with the more decreased median in forced expiratory flow between 25% and 75% of FVC (FEF 25%–75%) (z-score, -4.470; IQR, -5.330 to -3.170). Linear mixed effect models indicated that younger age at first infectious insult was significantly related to decreased FEV1 z-score (P = 0.041), and that the FEV 1/FVC and FEF 25%–75% z-scores were reduced by 0.082 (P = 0.005) and 0.069 (P ≤ 0.001) per year. @*Conclusion@#In children with SJMS, the initial infectious insult occurs in the early life with M. pneumoniae being the most common pathogen. Lung function may deteriorate with an obstructive pattern during growth. The impairment can progress slowly not in the FEV1, but in the FEV1/FVC or FEF25%–75%

Factors associated with Advanced Bone Age in Overweight and Obese Children / 대한소아소화기영양학회지

Mucosal Immunity Related to FOXP3⁺ Regulatory T Cells, Th17 Cells and Cytokines in Pediatric Inflammatory Bowel Disease

BACKGROUND: We aimed to investigate mucosal immunity related to forkhead box P3 (FOXP3+) regulatory T (Treg) cells, T helper 17 (Th17) cells and cytokines in pediatric inflammatory bowel disease (IBD). METHODS: Mucosal tissues from terminal ileum and colon and serum samples were collected from twelve children with IBD and seven control children. Immunohistochemical staining was done using anti-human FOXP3 and anti-RORγt antibodies. Serum levels of cytokines were analyzed using a multiplex assay covering interleukin (IL)-1β, IL-4, IL-6, IL-10, IL-17A/F, IL-21, IL-22, IL-23, IL-25, IL-31, IL-33, interferon (IFN)-γ, soluble CD40L, and tumor necrosis factor-α. RESULTS: FOXP3+ Treg cells in the lamina propria (LP) of terminal ileum of patients with Crohn's disease were significantly (P < 0.05) higher than those in the healthy controls. RORγt+ T cells of terminal ileum tended to be higher in Crohn's disease than those in the control. In the multiplex assay, serum concentrations (pg/mL) of IL-4 (9.6 ± 1.5 vs. 12.7 ± 3.0), IL-21 (14.9 ± 1.5 vs. 26.4 ± 9.1), IL-33 (14.3 ± 0.9 vs. 19.1 ± 5.3), and IFN-γ (15.2 ± 5.9 vs. 50.2 ± 42.4) were significantly lower in Crohn's disease than those in the control group. However, serum concentration of IL-6 (119.1 ± 79.6 vs. 52.9 ± 39.1) was higher in Crohn's disease than that in the control. Serum concentrations of IL-17A (64.2 ± 17.2 vs. 28.3 ± 10.0) and IL-22 (37.5 ± 8.8 vs. 27.2 ± 3.7) were significantly higher in ulcerative colitis than those in Crohn's disease. CONCLUSION: Mucosal immunity analysis showed increased FOXP3+ T reg cells in the LP with Crohn's disease while Th17 cell polarizing and signature cytokines were decreased in the serum samples of Crohn's disease but increased in ulcerative colitis.

The Analysis of Factors Causing the High Prevalence of Child Obesity in Jeju Island / 대한소아소화기영양학회지

PURPOSE: For 3 consecutive years from 2012–2014, we analyzed the causative factors for why the Jeju Island had the highest obesity prevalences of school children among the 15 provinces in Korea. METHODS: From our analysis of 28,026 elementary school children with obesity or normal weight in the 15 provinces, we analyzed 12 factors related to eating habits, exercise habits, lifestyle, and mental health. The differences between the obese and normal weight children were researched. Finally, Jeju was compared with Seoul, which has the lowest obesity prevalence in school age children. Statistical analysis was performed using the chi square test of PASW Statistics ver. 18.0. RESULTS: Compared to the normal weight group, the obese group had significantly higher rates of consuming soft drinks (p<0.001), fast food intake (p=0.019), skipping breakfast (p<0.001), insufficient sleep (p<0.001), bullying experiences (p=0.001) and runaway impulses (p=0.012). Compared to Seoul, Jeju Island had significantly higher rates of Ramen intake (3.4% vs. 5.4%, p=0.021) and meat intake (46.0% vs. 52.9%, p=0.003). On the other hand, Jeju Island was significantly lower than was Seoul in their fruit intake (83.4% vs. 67.1%, p<0.001), vegetable intake (71.4% vs. 64.2%, p=0.001), and intense physical activity (63.4% vs. 47.7%, p<0.001). Meanwhile, insufficient sleep (15.4% vs. 9.6%, p<0.001) and runaway impulses (5.6% vs. 3.3%, p=0.027) in children were significantly lower in Jeju Island than in Seoul. CONCLUSION: The results of the obesity factor analysis of elementary school students in Jeju Island can be used as useful educational material for lowering the obesity prevalence in Jeju community.

Concurrence of Diabetic Ketoacidosis and Acute Ischemic Stroke due to Moyamoya Disease / 대한소아신경학회지

Although the etiology of moyamoya disease (MMD) remains unknown, autoimmunity is one of the proposed pathogeneses. Unlike other autoimmune disorders that are associated with cerebral arteritis, concurrence of MMD and diabetes mellitus (DM) is rare. However, we encountered a patient with concurrent diabetic ketoacidosis (DKA) and acute ischemic stroke due to MMD. Our patient was diagnosed with glutamic acid decarboxylase antibody-positive type 2 DM (T2DM) based on laboratory and physical examination findings. Brain magnetic resonance images revealed an acute ischemic stroke in the left cerebral hemisphere and bilateral diffuse stenosis/occlusion in the middle cerebral artery and multiple collaterals. Thus, here, we report a patient with both T2DM and MMD who developed an acute ischemic stroke that was complicated by DKA.

Associations among the Degree of Nonalcoholic Fatty Liver Disease, Metabolic Syndrome, Degree of Obesity in Children, and Parental Obesity / 대한소아소화기영양학회지

PURPOSE: To analyze the associations among the degrees of nonalcoholic fatty liver disease (NAFLD) by ultrasonography and metabolic syndrome, degrees of obesity in children, and degrees of parental obesity. METHODS: A total of 198 children with obesity who visited a pediatric obesity clinic were prospectively enrolled in this study. The severity of NAFLD based on ultrasonography was classified into no, mild, moderate, or severe NAFLD group. The degree of obesity based on the percentage over standard weight for height per sex was classified into mild, moderate, or severe. RESULTS: Of 132 patients evaluated for the degree of NAFLD and metabolic syndrome, the p-value of correlation between the two factors was 0.009. Therefore, metabolic syndrome might significantly affect the degree of NAFLD. Of 158 patients evaluated for the degree of NAFLD and the degree of obesity, the p-value of correlation between the two factors was 0.122. Of 154 patients evaluated for the degree of obesity and father's obesity, the p-value was 0.076. Of 159 patients evaluated for the degree of obesity and mother's obesity, the p-value was 0.000, indicating that mother's obesity could significantly affect the degree of obesity in children. Of 142 patients evaluated for the degree of obesity and metabolic syndrome, the p-value was 0.288. CONCLUSION: Metabolic syndrome might significantly affect the degree of nonalcoholic fatty liver in children. In addition, mother's obesity might be a significant factor that affects the degree of obesity in children.