We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Primary Pulmonary Arterial Hypertension and Autoimmune Polyendocrine Syndrome in a Pediatric Patient.
- Authors
Alghamdi, Mohammed H.; Steinraths, Michelle; Panagiotopoulos, Constadina; Potts, James E.; Sandor, George G. S.
- Abstract
Both primary pulmonary artery hypertension (PPAH) and autoimmune polyendocrine syndrome (APS) are rare disorders in children. We report a boy who was diagnosed with severe PPAH at 12 years of age. He was treated with prostacyclin for 6 years, briefly with adjunct bosentan, and eventually sildenafil was added. Six years later, after his diagnosis of PPAH, he developed APS in the form of hyperthyroidism and type 1 diabetes mellitus. No mutations were identified through genetic testing of bone morphogenetic protein receptor type II and the autoimmune-regulator gene. To our knowledge this is the first description of the combination of these two extremely rare diseases in a child.
- Subjects
CASE studies; PULMONARY artery diseases; HYPERTENSION in children; PEDIATRIC cardiology; AUTOIMMUNE diseases
- Publication
Pediatric Cardiology, 2010, Vol 31, Issue 6, p872
- ISSN
0172-0643
- Publication type
Article
- DOI
10.1007/s00246-010-9704-y