A case of reversible pulmonary arterial hypertension associated with incontinentia pigmenti
نویسندگان
چکیده
منابع مشابه
Pulmonary hypertension and vasculopathy in incontinentia pigmenti: a case report
Incontinentia pigmenti (IP; Bloch-Sulzberger syndrome) is a rare, genetic syndrome inherited as an X-linked dominant trait. It primarily affects female infants and is lethal in the majority of males during fetal life. The clinical findings include skin lesions, developmental defects, and defects of the eyes, teeth, skeletal system, and central nervous system. Cardiovascular complications of thi...
متن کاملA case of incontinentia pigmenti associated with multiorgan abnormalities.
Incontinentia pigmenti is a systemic disorder affecting the skin, teeth, eyes, nervous tissue, hair, nails, musculoskeletal system, and heart. We describe an 11-month-old girl with incontinentia pigmenti associated with a ventricular septal defect, left hemiatrophy, hemangiomas, an abnormal labial frenum, and spastic cerebral palsy manifested as left hemiplegia and developmental delay. We belie...
متن کاملA case of severe pulmonary hypertension associated with common arterial trunk and VSD in a 9 years old child
Background: Common arterial trunk (CAT) is a rare congenital heart disease, and often leads to the early development of pulmonary hypertension and disability. Among the critical structural heart defects, the frequency of CAT is 3%, which reflects the severe hemodynamic disturbances. The natural course of the disease is characterized by a high mortality rate up to 88% during the first year of li...
متن کاملIncontinentia pigmenti: a case report.
Incontinentia pigmenti (IP) is a genodermatosis with an X-linked dominant mode of inheritance, characterized by ectodermal, mesodermal, neurological, ocular, and dental manifestations. The purpose of this case study was to report the oral and dental manifestations of an IP case in a Venezuelan pediatric patient. A 9 year-old Venezuelan girl was evaluated. She showed macular pigmented lesions in...
متن کاملA Case Report of Incontinentia Pigmenti
Corresponding Author: B. Basiri MD Tel: +98 9188121848 Email: [email protected] Abstract Objective Incontinentia Pigmenti (IP) (Bloch_Sulzberg syndrome) is a rare neurocutaneous syndrome characterized by multisystemic involvement that is prenatally lethal in the majority of affected males and shows great clinical variability when expressed in women. The diagnosis of IP is performed based o...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Pulmonary Circulation
سال: 2018
ISSN: 2045-8940,2045-8940
DOI: 10.1177/2045894018793983