(HOME) Subject: amyloidosis/iron

   
   Curr Treat Options Cardiovasc Med 2000 Oct;2(5):431-438
   
Restrictive Cardiomyopathy.

    Artz G, Wynne J
    
   Division of Cardiology, Harper Hospital, 3990 John R. Street, Detroit,
   MI 48201, USA. jwynne@intmed.wayne.edu
   
   [Record supplied by publisher]
   
   Of the three major functional categories of cardiomyopathies (dilated,
   hypertrophic, and restrictive), the restrictive cardiomyopathies
   (RCMs) are the least common in the Western world, but unfortunately
   often are associated with the greatest morbidity and mortality.
   Infiltrative disease of the myocardium (often caused by amyloidosis)
   is a common cause of RCMs and has a significantly lower long-term
   survival rate when compared to cardiomyopathies of various other
   causes. Treatment of the RCM is, in general, difficult and often
   ineffective. Because of the abnormalities of diastolic filling that
   are characteristic of this condition, general measures to reduce
   venous and systemic congestion such as with the use of diuretics, are
   desirable but often result in an attendant reduction in stroke volume
   and cardiac output. Digoxin, calcium channel blocking drugs, and
   beta-adrenergic blocking agents are of limited value, although they
   may be used with benefit to control the heart rate response in the
   subgroup of patients with atrial fibrillation. Angiotensin-converting
   enzyme inhibitors are generally ineffective in RCM. Targeted therapy
   directed against specific causal entities (such as the use of
   somatostatin analogues in carcinoid syndrome or iron chelation with
   desferrioxamine in hemochromatosis) may be more effective than simple
   symptomatic therapy. Differentiation of RCM from constrictive
   pericarditis is crucial, since constriction may be treated effectively
   by surgical removal of the thickened pericardium. A limited number of
   patients appear to have benefited from novel treatment strategies,
   such as autologous stem cell transplant in amyloidosis, balloon
   valvuloplasty of stenotic tricuspid or pulmonary valves in cardiac
   carcinoid syndrome, and cardiac transplantation. Truly effective
   therapy for RCM is generally lacking, and the best chance for
   optimizing the clinical outcome is early detection and aggressive
   medical treatment in an attempt to maintain the highest possible level
   of patient function for as long as possible.
   
   PMID: 11096547
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Subject: amyloidosis/iron/bird

   
   Ann N Y Acad Sci 1992 Jun 16;653:184-90
   
Amyloidosis, hemochromatosis, and atherosclerosis in a roseate flamingo
(Phoenicopterus ruber).

    Brayton C
    
   Laboratory Animal Research Center, Rockefeller University, New York,
   New York 10021.
   
   An aged male roseate flamingo, in a private collection in the British
   Virgin Islands, was found acutely "down." After four days of
   supportive therapy, the flamingo succumbed. At necropsy gross lesions
   included emaciation; collapsed and thickened, yellow abdominal air
   sac; dark red liver, partially covered by friable yellow material; and
   a raised, intimal plaque in the aorta near the iliac trifurcation.
   Histologic examination revealed severe, diffuse, pyogranulomatous air
   sacculitis with associated locally extensive
   pleuroperitonitis/perihepatitis. Pansystemic, predominantly
   periarteriolar distribution of amyloid deposition was evident, as was
   massive intrahepatocellular accumulation of iron pigment
   (hemachromatosis/hemosiderosis). A locally extensive, nonobstructive,
   fibroatheromatous plaque was present in the distal aorta. Amyloidosis,
   hemochromatosis/hemosiderosis, and atherosclerosis have been
   recognized in Phoenicopteriformes and other marine or aquatic birds.
   Their pathogenesis and pathogenicity remain a matter of debate.
   
   PMID: 1626869, UI: 92328426
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