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Recurrence of vitreous amyloidosis and need of surgical reintervention in portuguese patients with familial amyloidosis attr v30m.

机译:葡萄牙家族性淀粉样变性attr v30m患者的玻璃体淀粉样变性复发和需要手术再干预。

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PURPOSE: : Vitreous amyloid deposits are one of the most common ocular manifestations of familial amyloidosis ATTR V30M (FAP-I), which can be the only manifestation of the disease and can appear even after liver transplantation. Removal by vitrectomy is usually performed, but vitreous amyloid recurrence has been frequently reported. This study was undertaken to evaluate the recurrence of vitreous amyloidosis and its relationship with the degree of previous vitreous removal. METHODS: : Fifty-four vitrectomized eyes from 32 patients with FAP-I were evaluated in the course of a follow-up period of 30.7 +/- 17.2 months (range, 8-78; median = 30 months). An extensive, as possible, vitrectomy with indentation was performed in 41 eyes (complete), and in the others 13 eyes only a vitrectomy without indentation (incomplete) was performed. The parameters evaluated were the incidence of amyloid deposits and visual outcomes. RESULTS: : A noteworthy visual acuity gain was observed, although a few patients had a subsequent decrease of visual acuity related to new vitreous amyloid deposition in the visual axis. These new amyloid deposits did not occur in eyes that had undergone extensive vitreous removal, but only in nonextensive vitrectomized eyes (P < 0.001). CONCLUSION: : Recurrence of amyloid deposition only occurred in nonextensive vitrectomized eyes and represents a false recurrence associated with incomplete vitrectomy.
机译:目的:玻璃体淀粉样沉积物是家族性淀粉样变性病ATTR V30M(FAP-1)最常见的眼部表现之一,可能是该疾病的唯一表现,甚至在肝移植后也可能出现。通常通过玻璃体切除术进行切除,但是玻璃体淀粉样蛋白复发的报道很多。进行这项研究以评估玻璃体淀粉样变性病的复发及其与先前玻璃体切除程度的关系。方法:在30.7 +/- 17.2个月的随访期间(范围8-78;中位数= 30个月),对32例FAP-I患者的54只玻璃体切割的眼睛进行了评估。在41眼(完全)中进行了广泛的,有压痕的玻璃体切除术,在其他13眼中,仅进行了无压痕的玻璃体切除术(不完全)。评估的参数是淀粉样沉积物的发生率和视觉结果。结果:观察到明显的视力增加,尽管少数患者随后的视力下降与视轴上新的玻璃状淀粉样蛋白沉积有关。这些新的淀粉样蛋白沉积物在经历了广泛的玻璃体切除的眼睛中没有发生,而仅发生在未扩大玻璃体切除术的眼睛中(P <0.001)。结论:淀粉样蛋白沉积的复发仅发生在非广泛性玻璃体切除术的眼睛中,代表与不完全玻璃体切除术相关的假复发。

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