BACKGROUND Kawasaki disease(KD)is an acute self-limited vasculitis with a predilection for coronary arteries.Children with KD may have altered lipid metabolism and abnormal lipid profiles that may last for prolonged p...BACKGROUND Kawasaki disease(KD)is an acute self-limited vasculitis with a predilection for coronary arteries.Children with KD may have altered lipid metabolism and abnormal lipid profiles that may last for prolonged periods.However,there is a paucity of literature on the role of adipocytokines in KD.AIM To estimate the levels of adipocytokines(adiponectin,leptin and resistin)during the convalescent phase of KD.METHODS Twenty children,who had KD at least three years earlier,were enrolled in this study.In addition,20 healthy controls were also enrolled.Clinical and laboratory profiles of patients were obtained from hospital records.Serum adiponectin,leptin and resistin levels were estimated by enzyme-linked immunosorbent assay.RESULTS Mean age of the patients in the study group was 10.15±3 years and the male:female ratio was 1.5:1.Median serum resistin levels in patients with KD(27.77 ng/mL;[IQR:18.66,48.90])were decreased compared to controls(21.20 ng/mL;[IQR:14.80,27.00])(P=0.04).Median serum leptin levels in cases and controls were 1.83 ng/mL;(IQR:1.13,3.80),and 1.10 ng/mL;(IQR:0.41,2.88),respectively(P=0.09).Median serum adiponectin levels were similar in both cases(12.20μg/mL;[IQR:9.76,17.97])and controls(13.95μg/mL;[IQR:11.17,22.58]);(P=0.18).There was no significant difference in all 3 adipocytokines between children with(4/20)and without coronary artery abnormalities(16/20).CONCLUSION Serum resistin levels were significantly elevated in patients with KD during the convalescent phase compared to controls.Serum leptin levels appeared to be higher in patients with KD,although the difference was not statistically significant.Adiponectin levels were similar in both cases and controls.Raised resistin and leptin levels may partially explain lipid perturbations observed during the convalescent phase of KD.展开更多
Inflammatory bowel disease(IBD)is more common in adults than in children.Onset of IBD before 17 years of age is referred as pediatric onset IBD and is further categorized as very early onset IBD(VEO-IBD)for children w...Inflammatory bowel disease(IBD)is more common in adults than in children.Onset of IBD before 17 years of age is referred as pediatric onset IBD and is further categorized as very early onset IBD(VEO-IBD)for children who are diagnosed before 6 years of age,infantile IBD who had the disease before 2 years of age and neonatal onset IBD for children less than 28 days of life.Children presenting with early onset disease may have a monogenic basis.Knowledge and awareness of the clinical manifestations facilitates early evaluation and diagnosis.Next generation sequencing is helpful in making the genetic diagnosis.Treatment of childhood IBD is difficult;targeted therapies and hematopoietic stem cell transplantation form the mainstay.In this review we aim to summarize the genetic defects associated with IBD phenotype.We describe genetic location and functions of various genetic defect associated with VEO-IBD with their key clinical manifestations.We also provide clinical clues to suspect these conditions and approaches to the diagnosis of these disorders and suitable treatment options.展开更多
文摘BACKGROUND Kawasaki disease(KD)is an acute self-limited vasculitis with a predilection for coronary arteries.Children with KD may have altered lipid metabolism and abnormal lipid profiles that may last for prolonged periods.However,there is a paucity of literature on the role of adipocytokines in KD.AIM To estimate the levels of adipocytokines(adiponectin,leptin and resistin)during the convalescent phase of KD.METHODS Twenty children,who had KD at least three years earlier,were enrolled in this study.In addition,20 healthy controls were also enrolled.Clinical and laboratory profiles of patients were obtained from hospital records.Serum adiponectin,leptin and resistin levels were estimated by enzyme-linked immunosorbent assay.RESULTS Mean age of the patients in the study group was 10.15±3 years and the male:female ratio was 1.5:1.Median serum resistin levels in patients with KD(27.77 ng/mL;[IQR:18.66,48.90])were decreased compared to controls(21.20 ng/mL;[IQR:14.80,27.00])(P=0.04).Median serum leptin levels in cases and controls were 1.83 ng/mL;(IQR:1.13,3.80),and 1.10 ng/mL;(IQR:0.41,2.88),respectively(P=0.09).Median serum adiponectin levels were similar in both cases(12.20μg/mL;[IQR:9.76,17.97])and controls(13.95μg/mL;[IQR:11.17,22.58]);(P=0.18).There was no significant difference in all 3 adipocytokines between children with(4/20)and without coronary artery abnormalities(16/20).CONCLUSION Serum resistin levels were significantly elevated in patients with KD during the convalescent phase compared to controls.Serum leptin levels appeared to be higher in patients with KD,although the difference was not statistically significant.Adiponectin levels were similar in both cases and controls.Raised resistin and leptin levels may partially explain lipid perturbations observed during the convalescent phase of KD.
文摘Inflammatory bowel disease(IBD)is more common in adults than in children.Onset of IBD before 17 years of age is referred as pediatric onset IBD and is further categorized as very early onset IBD(VEO-IBD)for children who are diagnosed before 6 years of age,infantile IBD who had the disease before 2 years of age and neonatal onset IBD for children less than 28 days of life.Children presenting with early onset disease may have a monogenic basis.Knowledge and awareness of the clinical manifestations facilitates early evaluation and diagnosis.Next generation sequencing is helpful in making the genetic diagnosis.Treatment of childhood IBD is difficult;targeted therapies and hematopoietic stem cell transplantation form the mainstay.In this review we aim to summarize the genetic defects associated with IBD phenotype.We describe genetic location and functions of various genetic defect associated with VEO-IBD with their key clinical manifestations.We also provide clinical clues to suspect these conditions and approaches to the diagnosis of these disorders and suitable treatment options.
