We measured FVlla, vWF : Ag, D-dimer and TAT to study the abnormality of coagulation in patients with juvenile chronic arthritis (JCA). Our subjects included 14 systemic JCA,16 pauciarticular JCA and 16 polyarticular JCA without DIC,thrombosis and liver dysfunction. All types of JCA showed an increased of FVlla, D-dimer and TAT.In particular, only systemic JCA has characteristically an clevation of vWF : Ag. We concluded that all types of JCA constitute a atate of subclinical hypercoagulopathy caused by tissue damage and that additionally systemic JCA involves a prothrombotic state brought on by vasculitis.
We report a Japanese child of the CINCA syndrome. The case was accompanied by the severely growth retardation and no pubertal development. However the growth hormone secretion was normal. The growth hormone therapy failed to increase his height. He showed the decrease of the GH secretion by insulin stimulation test, it caused that the long term glucocorticoid therapy influenced the suppression of hypothalanus-hypophysis axis. However he main cause of this patient's growth retardation is the early closure of physis, because GH secretion by L-DOPA and GRF urinary GH and IGF-1 were normal, moreover he had no effect of GH therapy (0.5U/kg/week) during one year. It is the difference between JRA and CINCA syndrome on the effect of GH therapy, because GH therapy effects on growth velocity of JRA patients. On this point, CINCA syndrome is essentially differrent from JRA on the pathogenesis. He had the dificiency of IgG3 and IgG4 with the decrease of B-cell population.
Abbreviations : FVIIa : activated factor VIIa ; vWF : Ag : von Willebrand factor antigen ; TAT : thrombin-antithrombin III complex ; JCA : Juvenile Chronic Arthritis ; CINCA syndrome : the Chronic, Infantile, Neurological, Cutaneous and Articular Syndrome