Oligoclonal Expansion of the VH Family in Kawasaki Disease

PURPOSE: To search for evidence of B cell activation and superantigen involvement in Kawasaki disease. METHODS: Peripheral lymphocytes were first isolated from Kawasaki disease patients in the acute and subacute phases. The T cell and B cell distributions were analyzed, cDNA was generated from the total RNA extracts, and PCR amplification of the cDNA for each immunoglobulin heavy chain family was performed to determine the presence of VH family-specific oligoclonal expansion. CDR3 size analysis was then conducted by two-stage PCR. RESULTS: The percentage of B cells increased significantly(P<0.05) in both the acute and subacute phases. Random utilization of diverse VH family genes was observed in the acute phase, and no family-specific expansion was detected. In 13 out of 15 Kawasaki disease patients, an increase in B cells expressing the VH3 family was seen during the acute phase, making it the most frequently utilized family. Analysis of B cell clonal expansion showed the VH6 family clone of 9 amino acids to be the most common clone, observed in 5 out of 15 Kawasaki disease patients. Analysis of the CDR3 size profile in two patients showed that in the acute phase various prominent bands appeared, some disappearing in the subacute phase, while other newly developed bands appeared. CONCLUSION: VH family-specific B cell expansion was not detected, and clonal expansion of B cells was observed, suggesting that Kawasaki disease may be caused by a conventional antigen, and the antigenic stimulation seen during the acute phase seems to be continuous, resuming after clinical resolution.

The Effects of Growth Hormone on Carbohydrate and Lipid Metabolism / 대한소아내분비학회지

PURPOSE:Growth hormone stimulates longitudinal growth, and it also exerts various effects on the metabolism of carbohydrates, lipids and proteins, indirectly regulating fuel metabolism. A hallmark is the stimulation of lipolysis, suppression of glucose oxidation, and development of insulin resistance. These metabolic effects subsequently affect body composition and atherogenic risk factors. There have recently been numerous reports concerning the metabolic effects of growth hormone, but the results are conflicting and exact cellular mechanism of action is yet unknown. The aim of this study is to assess the effect of biosythetic growth hormone replacement on carbohydrate and lipid metabolism and its subsequent effect on atherogenic risk in growth hormone deficient and idiopathic short stature. METHODS:We studied 111 idiopathic short stature patients and 12 children diagnosed with growth hormone deficiency by growth hormone provocation tests. Subjects were divided into three groups on the basis of duration of treatment; those who had been receiving GH replacement for 6, 12 and 18 months. Growth hormone was adminstered in a subcutaneous dose of 0.1 units per kg 6 times a week, and levels of blood sugar, total cholesterol, triglycerides, HDL-cholesterol, free fatty acid were measured before and after treatment in each group and changes in the atherogenic index (calculated as total cholesterol/HDL-cholesterol) were compared. RESULTS: 1)Blood sugar levels showed no significant change after GH therapy in both idiopathic short stature and growth hormone deficient groups. 2)Levels of total cholesterol showed a signficant decrease 6, 12 and 18 months after GH therapy in the idiopathic short stature patients, but no signficant change was noted in the growth hormone deficient patients. 3)GrowthhormonetherapyshowednosignificanteffectoneitherHDL-cholesterol or atherogenic index in both idiopathic short stature and growth-hormone deficient children. 4)There was no significant change in triglyceride and free fatty levels in children with idiopathic short stature after growth hormone therapy. CONCLUSIONS:Growth hormone administration significantly lowered total cholesterol levels in idiopathic short stature children, but failed to have a significant effect on atherogenic risk. Despite the increasingly widespread use of growth hormone in the treatment of hypopituitarism and non-GHD short stature, data concerning the metabolic effects of growth hormone are conflicting and the precise underlying mechanism is yet to be revealed, making further research necessary to determine the long term consequences of growth hormone replacement.