Severe pulmonary hypertension in Ullrich-Noonan syndrome.
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چکیده
منابع مشابه
Severe pulmonary hypertension in Ullrich-Noonan syndrome.
A 19 year old woman with Ullrich-Noonan syndrome presented with a six month history of progressive dyspnoea and cyanosis. Clinical features were suggestive of severe pulmonary hypertension. Cross sectional echocardiography showed pronounced right ventricular pressure overload with right ventricular hypertrophy. Cardiac catheterisation confirmed severe pulmonary hypertension. Attempts to reduce ...
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A 65 year-old female with a history of xerostomia and xerophthalmia was presented with dyspnea on exertion (New York Heart Association class III). Echocardiography and cardiac catheterization demonstrated severe pulmonary hypertension (PH). Laboratory examinations showed positive anti-nuclear and anti-Ro/SS-A antibodies. Schirmer's test was positive and salivary gland scintigraphy revealed seve...
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A 4-year-old boy with the Ullrich-Noonan syndrome is described. Asymmetric septal hypertrophy was diagnosed by echocardiography and confirmed at cardiac catheterisation. The aortic subvalvar gradient was reduced from 56 mmHg to 10 mmHg with intravenous propranolol. Relatives of patients with the syndrome should be screened by echocardiography in the hope that the early detection of asymmetric ...
متن کاملThe Bonnevie-Ullrich syndrome.
Turner's syndrome, the association of webbing of the neck, cubitus valgus and infantilism, is well recognized. Much less attention, however, has been paid to a condition occurring in infancy characterized by symmetrical webbing of the neck and lymphangiectatic oedema of the extremities as well as other features, and is termed the BonnevieUllrich syndrome. This condition has been extensively stu...
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STUDY OBJECTIVE Hyperuricemia occurs frequently in patients with myeloproliferative and lymphoproliferative disorders and in patients with congenital heart disease associated with polycythemia. Whether hyperuricemia is common in patients with severe pulmonary hypertension is not known. DESIGN, PATIENTS, MEASUREMENTS In the Pulmonary Hypertension Center at the University of Colorado Health Sci...
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ژورنال
عنوان ژورنال: Heart
سال: 1989
ISSN: 1355-6037
DOI: 10.1136/hrt.62.1.74