PDF(Pubmed)
Abstract：
UNASSIGNED: Takayasu artery (TAK) is a chronic inflammatory disease that mainly affects the aorta and its major branches and is rarely reported in infants. We aimed to summarize the clinical features of infant TA (I-TA) in a tertiary care center.
UNASSIGNED: We performed a retrospective study involving 10 infants diagnosed with TAK. A comprehensive evaluation of clinical, laboratory, radiographic features, disease activity, treatment and outcomes was carried out.
UNASSIGNED: A consecutive cohort was composed of 8 girls and 2 boys, with an age at diagnosis of 11.1 (1.7-36) months. The median time to diagnosis and the average time to follow-up were 9.5 days (2-235 days) and 10.9 (1-21) months, respectively. The most common initial manifestations were malaise (80%), fever (70%), hypertension (50%) and rash (30%). The mean Pediatric Vasculitis Activity Score (PVAS), Takayasu Clinical Activity Score (ITAS-2010) and ITAS-A scores were 2.8/63, 2.6/51, and 5.6/54, respectively. All patients had aberrant laboratory parameters. The most common lesions were in the thoracic aorta (60%) and abdominal aorta (60%). Corticosteroids combined with cyclophosphamide followed by long-term mycophenolate mofetil were initiated in most cases (70%). Biologics were attempted in 5 cases. Mortality was 40%.
UNASSIGNED: It is challenging to diagnose TAK in infants in a timely manner. Considering the more vessels involved, more severe inflammation and higher mortality, aggressive treatment is warranted in infants. GCs and CYC treatment seem to be effective.
UNASSIGNED: We performed a retrospective study involving 10 infants diagnosed with TAK. A comprehensive evaluation of clinical, laboratory, radiographic features, disease activity, treatment and outcomes was carried out.
UNASSIGNED: A consecutive cohort was composed of 8 girls and 2 boys, with an age at diagnosis of 11.1 (1.7-36) months. The median time to diagnosis and the average time to follow-up were 9.5 days (2-235 days) and 10.9 (1-21) months, respectively. The most common initial manifestations were malaise (80%), fever (70%), hypertension (50%) and rash (30%). The mean Pediatric Vasculitis Activity Score (PVAS), Takayasu Clinical Activity Score (ITAS-2010) and ITAS-A scores were 2.8/63, 2.6/51, and 5.6/54, respectively. All patients had aberrant laboratory parameters. The most common lesions were in the thoracic aorta (60%) and abdominal aorta (60%). Corticosteroids combined with cyclophosphamide followed by long-term mycophenolate mofetil were initiated in most cases (70%). Biologics were attempted in 5 cases. Mortality was 40%.
UNASSIGNED: It is challenging to diagnose TAK in infants in a timely manner. Considering the more vessels involved, more severe inflammation and higher mortality, aggressive treatment is warranted in infants. GCs and CYC treatment seem to be effective.
摘要：
■Takayasu动脉（TAK）是一种慢性炎症性疾病，主要影响主动脉及其主要分支，在婴儿中很少报道。我们旨在总结三级护理中心婴儿TA（I-TA）的临床特征。
■我们进行了一项回顾性研究，涉及10例诊断为TAK的婴儿。临床综合评价,实验室,射线照相特征，疾病活动,进行治疗和结果。
■连续的队列由8个女孩和2个男孩组成，诊断年龄为11.1（1.7-36）个月。中位诊断时间和平均随访时间分别为9.5天（2-235天）和10.9（1-21）个月，分别。最常见的最初表现是不适（80％），发烧（70%），高血压（50％）和皮疹（30％）。平均小儿血管炎活动评分（PVAS），Takayasu临床活动评分（ITAS-2010）和ITAS-A评分分别为2.8/63、2.6/51和5.6/54。所有患者均有异常的实验室参数。最常见的病变是胸主动脉（60％）和腹主动脉（60％）。在大多数情况下（70％）开始使用皮质类固醇联合环磷酰胺，然后长期使用霉酚酸酯。5例尝试使用生物制剂。死亡率为40%。
■及时诊断婴儿的TAK具有挑战性。考虑到涉及的船只越多，更严重的炎症和更高的死亡率，积极的治疗是必要的婴儿。GCs和CYC治疗似乎是有效的。
■我们进行了一项回顾性研究，涉及10例诊断为TAK的婴儿。临床综合评价,实验室,射线照相特征，疾病活动,进行治疗和结果。
■连续的队列由8个女孩和2个男孩组成，诊断年龄为11.1（1.7-36）个月。中位诊断时间和平均随访时间分别为9.5天（2-235天）和10.9（1-21）个月，分别。最常见的最初表现是不适（80％），发烧（70%），高血压（50％）和皮疹（30％）。平均小儿血管炎活动评分（PVAS），Takayasu临床活动评分（ITAS-2010）和ITAS-A评分分别为2.8/63、2.6/51和5.6/54。所有患者均有异常的实验室参数。最常见的病变是胸主动脉（60％）和腹主动脉（60％）。在大多数情况下（70％）开始使用皮质类固醇联合环磷酰胺，然后长期使用霉酚酸酯。5例尝试使用生物制剂。死亡率为40%。
■及时诊断婴儿的TAK具有挑战性。考虑到涉及的船只越多，更严重的炎症和更高的死亡率，积极的治疗是必要的婴儿。GCs和CYC治疗似乎是有效的。
