Disturbance of Blood Coagulation in Juvenile Chronic Arthritis, and Epidcmiology, Clinical Studies on CINCA Syndrome.

为探讨幼年慢性关节炎(JCA)患者凝血功能的异常，我们测定了FV11a、vWF：Ag、D-二聚体和TAT。其中系统性JCA 14例，少关节JCA 16例，无DIC、血栓形成和肝功能障碍的多关节JCA 16例。各型JCA均表现为FV11a、D-二聚体和TAT升高，尤其是系统性JCA具有明显的vWF：Ag升高。我们的结论是，所有类型的JCA都构成了一种由组织损伤引起的亚临床高凝状态，此外，全身性JCA还涉及到由脉管炎引起的血栓前状态。我们报告一例日本儿童CINCA综合征。该病例伴有严重的生长发育迟缓和无青春期发育。但生长激素分泌正常。生长激素疗法没能增加他的身高。胰岛素刺激试验显示生长激素分泌减少，这是长期糖皮质激素治疗影响下丘脑-垂体轴抑制的结果。而这位患者生长迟缓的主要原因是生长激素早期关闭，因为L多巴的GH分泌和GRF尿GH和IGF-1正常，而且他在一年内没有接受生长激素治疗(0.5U/kg/周)。生长激素治疗对JRA患者生长速度的影响是JRA与CINCA综合征的区别所在。在这一点上，CINCA综合征在发病机制上与JRA有本质的不同。缩写：FVIIa：活化因子VIIa；vWF：Ag：von Willebrand因子抗原；TAT：凝血酶-抗凝血酶III复合体；JCA：幼年型慢性关节炎；CINCA综合征：慢性、婴儿、神经、皮肤和关节综合征

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