Storm syndrome

Overview

A rare progressive genetic disorder characterized by premature aging and heart disease which results in premature death.

Symptoms

* Early progressive cardiopathy * Loss of eyebrows during adolescence * Thinning hair during adolescence * Graying hair during adolescence * Wrinkled skin * Polyarthropathy * Calcification of mitral valves * Calcification of aortic valves * Mitral prolapse * Myxedematous degeneration * Loss of fat under skin * Early hair loss * Osteoporosis * Cataracts * Type II diabetes * Muscle wasting * Aged skin * Premature death

Diagnosis

signs and symptoms of Storm syndrome may vary on an individual basis for each patient. Only your doctor can provide adequate diagnosis of any signs or symptoms and whether they are indeed Storm syndrome symptoms.

Treatment

Treatment depends on the nature and severity of associated symptoms. For example, heart failure requires digoxin, diuretics, a sodium-restricted diet and, in acute cases, oxygen. Other measures may include anticoagulant therapy or antiplatelet medications to prevent thrombus formation around diseased or replaced valves, prophylactic antibiotics before and after surgery or dental care, and valvuloplasty. An intra-aortic balloon pump may be used temporarily to reduce backflow by enhancing forward blood flow into the aorta. If the patient has severe signs and symptoms that can’t be managed medically, open heart surgery using cardiopulmonary bypass for valve replacement is indicated.