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

青少年慢性关节炎的凝血障碍、流行病学、CINCA 综合征的临床研究。

• 批准号: 04670626
• 负责人: INAMO Yasuji
• 金额: $ 0.45万
• 依托单位: Nihon University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for General Scientific Research (C)
• 财政年份: 1992
• 资助国家: 日本
• 起止时间: 1992 至 1994
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/en/grant/KAKENHI-PROJECT-04670626/
• 关键词: juvenile chronic arthritis; CINCA Syndrome; FVIIa : activated factor VIIa; vWF : Ag : von Willebrand fator antigen ;; JRA; 組織因子; 成長障害; 活性化VII因子; TFPI

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

我们检测了FVlla、vWF：Ag、D-二聚体和达特，以研究幼年慢性关节炎（JCA）患者的凝血异常。我们的研究对象包括14例系统性JCA，16例少关节JCA和16例多关节JCA，无DIC、血栓形成和肝功能损害。所有类型的JCA均表现为FVIIa、D-二聚体和达特的升高，尤其是系统性JCA有特征性的vWF：Ag升高。我们的结论是，所有类型的JCA构成了一个atate的亚临床高凝状态引起的组织损伤，另外全身JCA涉及血栓前状态所带来的血管literation.We报告一个日本儿童的CINCA综合征。该病例伴有严重的生长迟缓和无青春期发育。但生长激素分泌正常。生长激素治疗未能增加他的身高。他通过胰岛素刺激试验显示GH分泌减少，这是由于长期糖皮质激素治疗影响了下丘脑-垂体轴的抑制。然而，该患者生长迟缓的主要原因是生长骨骺板早期闭合，因为L-DOPA和GRF分泌GH，尿GH和IGF-1正常，并且他在一年内没有GH治疗（0.5U/kg/周）的效果。生长激素治疗对JRA患者生长速度的影响是JRA与CINCA综合征在生长激素治疗效果上的差异。在这一点上，CINCA综合征与JRA在发病机制上有本质的区别。缩略语：FVIIa：活化因子VIIa ; vWF：Ag：血管性血友病因子抗原;达特：凝血酶-抗凝血酶Ⅲ复合物; 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

项目成果

稲葉康司: "CINCA症候群(小児慢性神経皮膚関節症候群)" 小児科. 1145-1149 (1994)

Koji Inaba：“CINCA 综合征（儿童慢性神经皮肤关节综合征）”儿科学 1145-1149 (1994)。

Yasuji Inamo: "Case report : Chronic, Infantile, Neurological, Cutaneous and Articular Syndrome in Japan." Clinical and Experimental Rheumatology. vol.12 no.3. 447-449 (1994)

Yasuji Inamo：“病例报告：日本的慢性、婴儿、神经、皮肤和关节综合症。”

稲毛康司: "全身発症型にみられる血液凝固・線溶能亢進について" 小児内科. 24. 356-360 (1992)

Koji Inage：“关于全身性发作型中所见的血液凝固和纤维蛋白溶解增加”《小儿内科医学》24. 356-360 (1992)。

稲毛康司: "若年性関節リウマチおよびSLEにみられた血液凝固・線溶能亢進状態についての検討" リウマチ. 33. 31-31 (1991)

Koji Inage：“幼年类风湿性关节炎和 SLE 中观察到的高凝和纤维蛋白溶解状态的研究”风湿病学 33. 31-31 (1991)。

稲毛康司: "CINCA症候群と考えられる2例" リウマチ. 32. 697-697 (1992)

Koji Inage：“被认为是 CINCA 综合征的两例”风湿病学 32. 697-697 (1992)。