Spontaneous Chylothorax in a Patient with Noonan Syndrome. Treatment with Prednizone
نویسندگان
چکیده
latively common hereditary syndrome with characteristic facial features and cardiovascular manifestations, with a large variety of clinical features. Congenital heart diseases are present in 50% of cases and congenital pulmonary stenosis, valvular or subvalvular, is the most common. In less than 20% of cases we also observe dysplasia of the lymph vessels. The case we present refers to a young adult male, 29 years old, with Noonan syndrome, spontaneous chylothorax and bilateral lymphedema.
منابع مشابه
The use of octreotide to treat congenital chylothorax.
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Noonan syndrome is a genetic condition inherited by an autosomally dominant manner, characterised by congenital heart disease, short stature, abnormal facies and the somatic feature of Turner's syndrome, but a normal karyotype. Noonan syndrome affects approximately 1 in 1500 live births. Congenital heart disease occurs in 35-50% of patients diagnosed with noonan syndrome. The most common cardia...
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