Connective Tissue Disease Presenting With Signs and Symptoms  of Pulmonary Hypertension in Children

Connective Tissue Disease Presenting With Signs and Symptoms of Pulmonary Hypertension in Children

  • نوع فایل : کتاب
  • زبان : انگلیسی
  • مؤلف : Shinichi Takatsuki • Jennifer B. Soep • Michelle Calderbank • David Dunbar Ivy
  • چاپ و سال / کشور: 2011

Description

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.
Pediatr Cardiol (2011) 32:828–833 DOI 10.1007/s00246-011-9950-7 Received: 11 January 2011 / Accepted: 24 February 2011 / Published online: 30 March 2011
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