Objective: The purpose of this study was to investigate the prevalence of peri pheral neuropathy in patients with β thalas saemia. Methods: Thirty six patie nts with a mean age of 29.2 ±.8.2 years and 17 healthy...Objective: The purpose of this study was to investigate the prevalence of peri pheral neuropathy in patients with β thalas saemia. Methods: Thirty six patie nts with a mean age of 29.2 ±.8.2 years and 17 healthy controls with a mean age of 27.6 ±.9.1 were included in this study. Measurements included the neuropath y symptoms score (NSS), the neuropathy disability score (NDS) as well as nerve c onduction studies of two motor (ulnar and peroneal) and two sensory (ulnar and s ural) nerves of the right limbs. Results: A mainly sensory axonal polyneuropathy was present in 19 out of 36 patients (52.7%). Eight out of these 19 patients a lso had abnormal NDS values. The neuropathy correlated significantly with the ag e of the patients and the hematocrit. However, it did not correlate with the pre sence of antibodies against HCV, the ferritin levels, or with a history of trans fusions, desferrioxamine treatment, or splenectomy. Conclusions: This study show ed a high prevalence of a predominantly sensory neuropathy in patients with β thalassaemia. The electrophysiological data suggest that the underlying patholog y is an axonopathy. Chronic hypoxia of the nerves resulting from severe anaemia may contribute to the pathogenesis of this neuropathy.展开更多
文摘Objective: The purpose of this study was to investigate the prevalence of peri pheral neuropathy in patients with β thalas saemia. Methods: Thirty six patie nts with a mean age of 29.2 ±.8.2 years and 17 healthy controls with a mean age of 27.6 ±.9.1 were included in this study. Measurements included the neuropath y symptoms score (NSS), the neuropathy disability score (NDS) as well as nerve c onduction studies of two motor (ulnar and peroneal) and two sensory (ulnar and s ural) nerves of the right limbs. Results: A mainly sensory axonal polyneuropathy was present in 19 out of 36 patients (52.7%). Eight out of these 19 patients a lso had abnormal NDS values. The neuropathy correlated significantly with the ag e of the patients and the hematocrit. However, it did not correlate with the pre sence of antibodies against HCV, the ferritin levels, or with a history of trans fusions, desferrioxamine treatment, or splenectomy. Conclusions: This study show ed a high prevalence of a predominantly sensory neuropathy in patients with β thalassaemia. The electrophysiological data suggest that the underlying patholog y is an axonopathy. Chronic hypoxia of the nerves resulting from severe anaemia may contribute to the pathogenesis of this neuropathy.
