Caused by infective endocarditis, aortic dissection and trauma. Stroke volume declines and ventricular diastolic pressure rises. Premature closure of mitral valve may be seen by echocardiography. The first heart sound is soft or absent. Diastolic murmur is characteristically brief. Patient presents with pulmonary congestion, edema and hypotension. Severe acute regurgitation requires prompt surgical treatment. This [...]

Inflammatory and stenotic disease of medium and large arteries with predilection for aortic arch Subclavian artery -93%, common carotid -58%, aorta -47% Affects adolescent girls and young women Symptoms include arm claudication, Raynaud’s phenom., visual changes, TIA, HTN, renal failure, etc. Prednisone (40-60 mg/day) alleviates symptoms (impact on survival is unclear). Methotrexate is an alternative. [...]

Deficiency of the classical pathway components (C1q, C1r, C1s, C2 and C4). Propensity to immune-complex diseases (like SLE). Deficiency of C3, factor H, and factor I. Propensity to pyogenic infections (because of C3 role in opsonization). Deficiency of terminal components (C5, C6, C7, C8) and alternative pathway (properdin and factor D). Propensity for infections with [...]

This is acquired agammaglobulinemia (second and third decade of life). Males and females are equally affected. Cause is unknown, but viral infection (Epstain-Barr) has been implicated. B-cells are present but do not produce IgG-s. Activation of B-cells by the T-cells is defective. Patients are prone to pyogenic infections and to gastrointestinal protozoan Giardia lamblia (which [...]

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