Familial Occurrence of The Noonan Syndrome

The syndrome, usually termed the “male Turner syndrome”, is characterized by short stature, webbing of the neck, hypogonadism and cubitus valgus and occurs in males with a normal karyotype. A similar syndrome, but with normal gonadal development, occurs in girls. To avoid confusion with the 45XO Turner syndrome, it has been suggested that the syndrome be called the Noonan syndrome.Recent reports have confirmed the occurrence of the syndrome in families.We also have studied a case of two brothers aged fifteen and twelve years which illustrates some of the familial characteristics of the syndrome.They were not mentally retarded, but the elder had mental depression. So we were obliged to stop trying to correct his thoracic scoliosis because of his refusal.