Myasthenia Gravis: Novel Findings and Perspectives on Traditional to Regenerative Therapeutic Interventions.

The prevalence of myasthenia gravis (MG), an autoimmune disorder, is increasing among all subsets of the population leading to an elevated economic and social burden. The pathogenesis of MG is characterized by the synthesis of autoantibodies against the acetylcholine receptor (AChR), low-density lipoprotein receptor-related protein 4 (LRP4), or muscle-specific kinase at the neuromuscular junction, thereby leading to muscular weakness and fatigue. Based on clinical and laboratory examinations, the research is focused on distinguishing MG from other autoimmune, genetic diseases of neuromuscular transmission. Technological advancements in machine learning, a subset of artificial intelligence (AI) have been assistive in accurate diagnosis and management. Besides, addressing the clinical needs of MG patients is critical to improving quality of life (QoL) and satisfaction. Lifestyle changes including physical exercise and traditional Chinese medicine/herbs have also been shown to exert an ameliorative impact on MG progression. To achieve enhanced therapeutic efficacy, cholinesterase inhibitors, immunosuppressive drugs, and steroids in addition to plasma exchange therapy are widely recommended. Under surgical intervention, thymectomy is the only feasible alternative to removing thymoma to overcome thymoma-associated MG. Although these conventional and current therapeutic approaches are effective, the associated adverse events and surgical complexity limit their wide application. Moreover, Restivo et al. also, to increase survival and QoL, further recent developments revealed that antibody, gene, and regenerative therapies (such as stem cells and exosomes) are currently being investigated as a safer and more efficacious alternative. Considering these above-mentioned points, we have comprehensively reviewed the recent advances in pathological etiologies of MG including COVID-19, and its therapeutic management.

The Association of Erythropoietin and Age-Related Macular Degeneration in Hemodialysis Patients: A Nationwide Population-Based Cohort Study.

This population-based retrospective cohort study investigated the effectiveness of erythropoietin (EPO) treatment in reducing the risk of age-related macular degeneration (AMD) in hemodialysis patients, using the National Health Insurance Research Data of Taiwan. From the database, we identified 147,318 end-stage renal disease (ESRD) patients on hemodialysis who had been diagnosed in 2000−2014 to establish the propensity-score-matched EPO user cohort and non-EPO user cohort with equal sample size of 15,992. By the end of 2016, the cumulative incidence of AMD in EPO users was about 3.29% lower than that in non-EPO users (Kaplan−Meier survival p < 0.0001). The risk of AMD was 43% lower in EPO users than in non-EPO users, with an adjusted hazard ratio (aHR) of 0.57 (95% confidence interval (CI) = 0.51−0.64) estimated in the multivariate Cox model. A significant negative dose−response relationship was identified between the EPO dosage and the risk of AMD (p < 0.0001). Another beneficial effect of EPO treatment was a reduced risk of both exudative AMD (aHR = 0.48, 95% CI = 0.40−0.61) and non-exudative AMD (aHR = 0.61, 95% CI = 0.53−0.69), also in similar dose−response relationships (p < 0.0001). Our findings suggest that EPO treatment for hemodialysis patients could reduce AMD risk in a dose−response relationship.

Risk factors of synchronous multifocal necrotizing fasciitis: a case control study in comparison with monofocal necrotizing fasciitis in Taiwan.

BACKGROUND: Monofocal necrotizing fasciitis (MONF) involves a single site in a rapidly progressing infection and necrosis of the fascia and surrounding soft tissue. Synchronous multifocal necrotizing fasciitis (SMNF), the simultaneous development of NF in multiple noncontiguous sites, is rarely reported. This study aimed to compare the clinical characteristics and outcomes between patients with SMNF and MONF, and to determine the risk factors of SMNF. METHODS: Our retrospective case-control study compared the clinical characteristics and outcomes, between January 2006 and January 2013, of patients with SMNF and of patients with MONF of the extremities. RESULTS: We enrolled 144 patients with NF of the extremities: 19 with SMNF and 125 with MONF. The duration of symptoms before admission was significantly shorter for the former than for the latter (1.7 days vs. 3.3 days, p = 0.001); the prevalence of shock at the initial visit significantly higher (73.7% vs. 36%, p = 0.002); and the total-case postoperative mortality rate significantly higher (68.4% vs. 14.4%, p <  0.001). In further analysis of the total-case mortality, 9 in 13 SMNF deaths (69.2%) within 7 days after fasciotomy were in the majority while 13 with 28-day mortality (72.2%) was the majority of MONF deaths (p <  0.001). SMNF was significantly more likely to involve bacteremia (89.5% vs. 36%, p <  0.001). Independent risk factors for SMNF were liver cirrhosis (LC) (odds ratio [OR] 6.0, p = 0.001) and end-stage renal disease (ESRD) (OR 7.1, p = 0.035). Gram-negative bacteria were most common in SMNF, and Gram-positive bacteria in MONF (83.3% vs. 53.3%, p = 0.005). Vibrio species were the most common single microbial cause (35.4%) of all NF patients and were the overwhelming cause (73.7%) of SMNF. Staphylococcus aureus and group A ß-hemolytic streptococcus (45.6%) were the other predominant causes of MONF while both (10.5%) rarely caused multifocal NF. CONCLUSIONS: SMNF was more fulminant than was MONF. SMNF was attributable primarily to marine Gram-negative bacteria. Physicians should be aware of SMNF because of its extremely high mortality rate.

The relationship between age, axial length and retinal nerve fiber layer thickness in the normal elderly population in Taiwan: The Chiayi eye study in Taiwan.

AIMS: To interpret how the thickness of the peripapillary retinal nerve fiber layer (RNFL) changes with increasing age, axial length, or anterior chamber depth as measured by spectral domain optical coherence tomography (OCT) in the normal elderly population in Taiwan. METHODS: A total of 82 volunteers (143 eyes) were enrolled. Generalized estimating equations were used to evaluate the correlation. RESULTS: The RNFL was significantly thinner in the superonasal (p = 0.004), inferotemporal (p = 0.046), and temporolower (p = 0.009) segments with age. The same trend was also observed in the superotemporal (p = 0.330) segment, although it was not statistically significant. The global RNFL thickness decreased by 4.97 µm per decade (ß = -0.497; p = 0.021), and thinning was significant in the superonasal (-9.90 µm per decade, p < 0.001) and temporolower (-6.78 µm per decade, p < 0.001) segments; the same trend showed borderline significance in the superotemporal (-6.96 µm per decade, p = 0.073) and inferotemporal (-7.23 µm per decade, p = 0.059) segments. In eyes with longer axial length, the RNFLs significantly decreased in the non-temporal segments. Global RNFL thickness decreased by 3.086 µm for each additional millimeter of axial length (ß = -3.086; p < 0.001). CONCLUSIONS: Changes in RNFL thickness were correlated with age in the superonasal, superotemporal, inferotemporal, and temporolower segments, and were correlated with axial length in the non-temporal segments. Anterior chamber depth was not correlated with RNFL thickness.

Deep vein thrombosis due to continuous prone positioning after retinal detachment surgery.

In March 2014, a 56-year-old woman without previous underlying disease underwent encircling scleral buckling, 20-gauge pars plana vitrectomy, cryotherapy around a retinal tear, and gas-fluid exchange with 15% perfluoropropane flush for upper rhegmatogenous retinal detachment of the left eye. However, she developed progressive left leg swelling, pain, warmth, and redness, associated with difficulty in elevating her left leg after continuously maintaining a prone head position when either lying down or sitting for 2 days. When she arrived at the emergency room, she had an elevated D-dimer level. After undergoing Doppler ultrasound imaging, she was diagnosed as having deep vein thrombosis of the left leg. She received anticoagulation therapy with enoxaparin and warfarin overlapping for 7 days. The edema, pain, and paresthesia of her left leg were relieved. However, because of the risk of bleeding with anti-coagulation drug usage, the patient needed to be monitored for 6 months. Prone positioning for gas tamponade is important for anatomic and functional success in retinal detachment surgery; however, timely walking and rest between periods of continuous prone positioning should be encouraged to prevent deep vein thrombosis and other impaired circulation-related complications.

Rapid regression of cystoid macular edema associated with cytomegalovirus retinitis in adult acute myeloid leukemia by intravitreal methotrexate combined with oral valganciclovir: A case report with comparison of binocular outcome.

Cytomegalovirus (CMV) retinitis is a late complication of organ and hematopoietic stem cell transplant, the risk of which depends on the degree of immunosuppression. With the institution of preemptive ganciclovir therapy early after transplant, most patients survive episodes of life-threatening CMV infection during the early months (usually the first 3 months) after transplant and hence late onset of CMV disease, such as CMV retinitis, is being recognized more frequently. Direct involvement of the macula or optic head remains the leading cause of visual loss in patients with CMV retinitis, but there are few studies investigating the management of this condition. Herein, we present the case of 28-year-old man who had acute myeloid leukemia and developed CMV retinitis with bilateral cystoid macular edema and optic swelling in the right eye 6 months after bone marrow transplant. He received treatment with intravitreal methotrexate in the right eye in combination with oral valganciclovir. Visual acuity improved 1 month after four weekly injections of intravitreal methotrexate 400 µg/0.1 mL. Resolved disc swelling and regression of macular edema were also observed. By comparing binocular outcome, we present our findings and discuss the possible efficacy and safety of this treatment with respect to regression of anatomical damage and improvement in visual acuity.

Axial length and subfoveal choroidal thickness in individuals with age-related macular degeneration.

PURPOSE: To compare axial length (AL) and subfoveal choroidal thickness (SFCT) between individuals with age-related macular degeneration (AMD) and controls with no lesions. METHODS: This was a case-control study. In total, 853 eyes of 484 patients (>65 years), including 397 eyes at various AMD stages and 456 eyes with no fundus lesions (controls) were recruited. Using color fundus photography, eyes were grouped according to AMD degree. AL was automatically measured using IOL Master and SFCT was manually measured by two independent observers. The associations among age, AL, SFCT, and each AMD grade were analyzed. RESULTS: Out of 853 eyes, 456 had no lesions, 217 contained drusen only, 134 had early AMD, and 46 had late AMD. The eyes with late AMD were older (p = 0.007) and had longer AL (p ≥ 0.001) and thinner SFCT (p < 0.001) compared with groups of no fundus lesions, drusen only, and early AMD. SFCT in eyes with late AMD decreased by 19.20 µm (p = 0.049), 24.78 µm (p = 0.029), and 15.56 µm (p = 0.162) compared with groups of no fundus lesions, drusen only, and early AMD, respectively. SFCT decreased by 14.18 µm/mm increase in AL (p < 0.001). The odds ratio (OR) for late AMD by longer AL (≥25 mm) and thinner SFCT (<240 µm) was 4.54 (χ2 = 9.36; p = 0.002) and 4.86 (χ2 = 17.62; p < 0.001), respectively, and was 9.57 (χ2 = 18.07; p < 0.001) when both AL ≥ 25 ≥m and SFCT < 240 µm. CONCLUSION: Eyes with late AMD have distinct reduced SFCT and elongated AL. Eyes with thinner SFCT and longer AL showed high ORs for late AMD and even higher ORs when both factors were simultaneously present. These findings illustrate the crucial pathophysiological role of these two important ocular fac tors and arouse our attention to patients with both characteristics, especially in Asian countries where the prevalence of myopia are disturbingly high.