ABSTRACT
Objective:To investigate the causes of secondary glaucoma after vitrectomy for familial vitreous amyloidosis associated with transthyretin (TTR) gene Gly83Arg mutation.Methods:A retrospective case study. From January 2008 to January 2020, 13 cases (23 eyes) with hereditary vitreous amyloidosis and treated by vitrectomy in the Affiliated Hospital of Zunyi Medical University were collected. Among them, there were 7 males with 12 eyes and 6 females with 11 eyes. The average age was 43.0±4.8 years. All the affected eyes underwent standard three-channel vitrectomy through the flat part of the ciliary body. According to whether complete vitreous detachment (PVD) was formed during the operation, it was divided into complete PVD group and incomplete PVD group; according to the occurrence time of secondary glaucoma and vitreous amyloidosis after surgery, it was divided into 1-12 months group and 13-36 months group, >37 months group. The average follow-up time after surgery was 36.7±6.0 months. The incidence of secondary glaucoma and the recurrence rate of vitreous amyloidosis between groups were compared by χ2 test; the correlation between recurrence of vitreous amyloidosis and secondary glaucoma after surgery was analyzed by Spearman rank correlation analysis. Results:Among the 23 eyes, there were 8 eyes in the complete PVD group and 15 eyes in the incomplete PVD group, respectively. Vitreous amyloidosis recurred in 15 eyes (65.22%, 15/23) after surgery. There were 14 (93.30%, 14/15) and 1 (6.70%, 1/15) eyes in the incomplete PVD group and the complete PVD group, respectively; the comparison of the recurrence rate of vitreous amyloidosis between the two groups was statistically significant ( χ2=11.676, P<0.01). 1-12 months group, 13-36 months group, >37 months group included 1 (4.35%, 1/23), 12 (52.17%, 12/23), 2 (8.70%, 2/23) Only eye. The recurrence rate in the 13-36 months group was significantly higher than that in the 1-12 months group and >37 month group. Secondary glaucoma occurred in 11 eyes (47.80%, 11/23) after surgery. 1-12 months group, 13-36 months group, above 37 months group were 1 (4.35%, 1/23), 8 (34.78%, 8/23), 2 (8.70%, 2/23) eyes. The incidence of secondary glaucoma in the 13-36 months group was higher than that in the 1-12 months group and >37 months group. Among 11 eyes with secondary glaucoma, 10 eyes had recurrence of vitreous amyloidosis after surgery, and 1 eye had no recurrence. The results of Spearman rank correlation analysis showed that there was a positive correlation between the recurrence of vitreous amyloidosis and the occurrence of secondary glaucoma ( rs=0.516, P=0.012). Conclusion:The incidence of secondary glaucoma after vitrectomy in a family with vitreous amyloidosis caused by the Gly83Arg mutation of TTR gene is higher, and its occurrence is significantly positively correlated with the recurrence of vitreous amyloidosis.
ABSTRACT
Objective To explore the prognostic factors for inability to walk independently in patients with multiple system atrophy (MSA).Methods A total of 123 patients with clinically confirmed MSA admitted to Navy General Hospital and Dongfang Hospital affiliated to the Second Clinical Medical College of Beijing University of Chinese Medicine , from February 2013 to February 2016, were retrospectively reviewed .Clinical data and all records were collected and all subjects were followed up by a telephone call in February 2016.The second milestone of activities of daily living scale (ADL), defined as inability to walk independently , was taken as the primary outcome .Eight possible prognostic factors were investigated and the survival analysis was performed with Cox proportional hazards model regression .Results Of all the MSA patients, 74 subjects were men and 49 were women with a sex radio of 1.51∶1(M∶F).Seventy cases were diagnosed with MSA-cerebellar type ( MSA-C ) and 53 with MSA-Parkinson type ( MSA-P ) (C∶P=1.32∶1).Mean age at the onset of first symptom was (53 ±8) years old.All patients had severe autonomic nervous dysfunction . At the last follow-up, 56 cases ( 45.5%) were unable to walk independently .The median survival time from the onset of MSA to inability to walk independently was 73 months.The age of onset ≥55 years (HR=1.969, 95%CI 1.095-3.542, P=0.024) and the interval time from disease onset to combined motor and autonomic involvement ≤3 years ( HR =2.308, 95%CI 1.158-4.600, P=0.017) were independent prognostic factors for inability to walk independently ,while gender, MSA clinical subtypes, initial symptoms, alcohol intake, smoking and toxic exposure were not indicators for independent walking (P>0.05).Conclusions The prognostic factors for inability to walk independently in patients with MSA are the age of onset ≥55 years and the interval time from disease onset to combined motor and autonomic involvement≤3 years.Although factors including gender , MSA clinical subtypes , initial symptoms , alcohol intake , smoking and toxic exposure are not the predictive factors for inability to walk independently in our MSA patients , their roles in the prognosis of MSA still need further investigation .