Lekakis John, Dimopoulos Meletios A, Prassopoulos Vassilios, Mavrikakis Myron, Gerali Sofia, Sifakis Nicos, Athanassoulis Theodoros, Stamatelopoulos Stamatios
Department of Clinical Therapeutics, Athens University School of Medicine, Athens, Greece.
Amyloid. 2003 Jun;10(2):117-20. doi: 10.3109/13506120309041733.
Three patients, 2 women and 1 man, with primary (AL) amyloidosis without congestive heart failure are described; all 3 patients presented reduced I-123 metaiodobenzylguanidine (MIBG) myocardial uptake suggesting marked cardiac sympathetic denervation. This is the first time myocardial adrenergic denervation is described in patients with AL amyloidosis without evidence of congestive heart failure; the observed denervation could be implicated in the pathogenesis of cardiac conduction disturbances which are common in this disease.
本文描述了3例原发性（AL）淀粉样变性患者，其中2例女性，1例男性，均无充血性心力衰竭；所有3例患者的I-123间碘苄胍（MIBG）心肌摄取均降低，提示明显的心脏交感神经去神经支配。这是首次在无充血性心力衰竭证据的AL淀粉样变性患者中描述心肌肾上腺素能去神经支配；观察到的去神经支配可能与该疾病中常见的心脏传导障碍的发病机制有关。
