Storm syndrome
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]
Overview
Rare progressive genetic disease characterized by premature aging and structural cardiac defects seen in a German family.
Pathophysiology
Storm syndrome a rare genetic disease of the connective tissue, is transmitted from male to male. It is characterized by early progressive calcific cardiac valvular disease which dominate the clinical picture. <itral valves and aortic valves are involved predominantly in the disease process
Differentiating from other diseases
Differences from Werner syndrome were dominant inheritance and lack of cataracts and scleroderma-type changes.
Diagnosis
Symptoms
- Premature aging
- Cardiac symptoms
- Intestinal fat malabsorption
Physical Examination
- Taut skin over hands and face
- Excessive wrinkling of the palmar skin - giving an aged appearance
- Loss of eye brows and eyelashes
- Thinning and graying of scalp hair in adolescents
In some young individuals polyarticular arthropathy and rheumatic fever were diagnosed along with valvular heart disease.