Takatsuki Shinichi, Soep Jennifer B, Calderbank Michelle, Ivy David Dunbar
Department of Pediatrics, The Children's Hospital, University of Colorado Denver School of Medicine, 3123 East 16th Avenue, B100, Aurora, CO 80045, USA.
Pediatr Cardiol. 2011 Aug;32(6):828-33. doi: 10.1007/s00246-011-9950-7. Epub 2011 Mar 30.
Our case series describes three children who were initially diagnosed as having severe pulmonary arterial hypertension (PAH) and subsequently found to be positive for specific autoantibodies suggestive of an underlying autoimmune process. The signs and symptoms of PAH are subtle and may be part of the initial presentation of childhood connective tissue disease (CTD). Evaluation for connective tissue disease in the newly diagnosed pulmonary hypertension (PH) patient is important because early diagnosis of PH as well as CTD is crucial in the successful management of these complex patients. Ongoing monitoring for CTD in patients with severe PAH is warranted.
我们的病例系列描述了三名儿童，他们最初被诊断为患有重度肺动脉高压（PAH），随后发现特定自身抗体呈阳性，提示存在潜在的自身免疫过程。PAH的体征和症状较为隐匿，可能是儿童结缔组织病（CTD）初始表现的一部分。对新诊断的肺动脉高压（PH）患者进行结缔组织病评估很重要，因为早期诊断PH以及CTD对于成功管理这些复杂患者至关重要。对重度PAH患者进行CTD的持续监测是必要的。
