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Background Guillain-Barré syndrome (GBS) is an autoimmune disease that affects the peripheral nervous system leading to motor, sensory, and sometimes autonomic manifestations. Therapeutic plasma exchange (TPE), which involves the selective removal of pathological molecules, such as auto-antibodies, from plasma, has proven to be safe and effective in adults with GBS. However, its application in pediatric patients lacks sufficient evidence. This study aims to evaluate the efficacy and safety of TPE in pediatric patients with severe GBS, in a low-resource setting. Methods This is a single-center retrospective study of 36 GBS patients aged between 2 and 13 years. A total of 122 TPE sessions were administered, with a median of four sessions per patient. A human albumin solution was the exchange fluid in all the sessions. Clinical improvement was evaluated through general examination and muscle power assessment using the Medical Research Council (MRC) scale. Results All patients showed clinical improvement upon treatment with TPE. The grade of power in the upper extremities increased from a mean of 1.7 ± 1.1 at the peak of illness to 3.7 ± 0.9 at discharge, indicating an increase of 2.0 ± 1.1 (95% CI, 1.6 to 2.4, p < 0.001). Alternatively, in the lower extremities, it increased from 1.2 ± 1.1 to 2.5 ± 0.8, indicating a significant rise of 1.4 ± 0.8 (95% CI, 1.1 to 1.6, p < 0.001). There was a significant improvement in the cranial, autonomic, and respiratory functions among all patients. Half of the patients were available for follow-up and showed full recovery, with six of them still exhibiting minimal residual deficits. TPE-related complications were mostly mild or moderate, with tachycardia, hypotension, and mild anemia being the most common. However, serious complications occurred in three of the patients, necessitating the discontinuation of the treatment in two of them. There was no mortality related to TPE in this study. Conclusions TPE shows promise in treating pediatric GBS. In this study, TPE was associated with the recovery of neurological functions, yielding positive outcomes with only minimal residual deficits. However, balancing its benefits with potential risks requires careful clinical judgment and rigorous monitoring to ensure patient safety and optimize outcomes. TPE was a more cost-effective and accessible option than IVIG in this financially restricted, low-income setting.
Background Guillain-Barré syndrome (GBS) is an autoimmune disease that affects the peripheral nervous system leading to motor, sensory, and sometimes autonomic manifestations. Therapeutic plasma exchange (TPE), which involves the selective removal of pathological molecules, such as auto-antibodies, from plasma, has proven to be safe and effective in adults with GBS. However, its application in pediatric patients lacks sufficient evidence. This study aims to evaluate the efficacy and safety of TPE in pediatric patients with severe GBS, in a low-resource setting. Methods This is a single-center retrospective study of 36 GBS patients aged between 2 and 13 years. A total of 122 TPE sessions were administered, with a median of four sessions per patient. A human albumin solution was the exchange fluid in all the sessions. Clinical improvement was evaluated through general examination and muscle power assessment using the Medical Research Council (MRC) scale. Results All patients showed clinical improvement upon treatment with TPE. The grade of power in the upper extremities increased from a mean of 1.7 ± 1.1 at the peak of illness to 3.7 ± 0.9 at discharge, indicating an increase of 2.0 ± 1.1 (95% CI, 1.6 to 2.4, p < 0.001). Alternatively, in the lower extremities, it increased from 1.2 ± 1.1 to 2.5 ± 0.8, indicating a significant rise of 1.4 ± 0.8 (95% CI, 1.1 to 1.6, p < 0.001). There was a significant improvement in the cranial, autonomic, and respiratory functions among all patients. Half of the patients were available for follow-up and showed full recovery, with six of them still exhibiting minimal residual deficits. TPE-related complications were mostly mild or moderate, with tachycardia, hypotension, and mild anemia being the most common. However, serious complications occurred in three of the patients, necessitating the discontinuation of the treatment in two of them. There was no mortality related to TPE in this study. Conclusions TPE shows promise in treating pediatric GBS. In this study, TPE was associated with the recovery of neurological functions, yielding positive outcomes with only minimal residual deficits. However, balancing its benefits with potential risks requires careful clinical judgment and rigorous monitoring to ensure patient safety and optimize outcomes. TPE was a more cost-effective and accessible option than IVIG in this financially restricted, low-income setting.
ObjectiveThe aim of this study was to investigate the causal effect of immune cell phenotype on GBS using two-sample Mendelian randomization (MR) approach.MethodsThis study used MR to investigate the causal relationship between 731 immune cell phenotypes and GBS. We used Inverse variance weighted, Weighted median, MR Egger, Simple mode, Weighted mode for MR analysis. We also used the Cochran Q test, MR-Egger intercept test, IVW regression and MR-PRESSO, leave-one-out analysis to assess the presence of horizontal pleiotropy, heterogeneity and stability, respectively.ResultsOur study revealed a causal relationship between 33 immune cell phenotypes and GBS. Twenty immunophenotypes were observed to be associated with GBS as risk factors. For example, CD20 on IgD+ CD38dim in the B cell group (OR = 1.313, 95%CI:1.042–1.654, p = 0.021), CD3 on CD4 Treg in Treg cell group (OR = 1.395, 95%CI:1.069–1.819, p = 0.014), CD3 on TD CD8br in Maturation stages of T cell group (OR = 1.486, 95%CI:1.025–2.154, p = 0.037), CD16 on CD14+ CD16+ monocyte in Monocyte group (OR = 1.285, 95%CI:1.018–1.621, p = 0.035), CD33dim HLA DR+ CD11b + %CD33dim HLA DR+ in Myeloid cell group (OR = 1.262, 95%CI:1.020–1.561, p = 0.032), HLA DR+ NK AC in TBNK cell group (OR = 1.568, 95%CI:1.100–2.237, p = 0.013). Thirteen immune phenotypes are associated with GBS as protective factors. For example, CD19 on PB/PC in the B cell group (OR = 0.577, 95%CI:0.370–0.902, p = 0.016), CD4 Treg AC in Treg cell group (OR = 0.727, 95%CI:0.538–0.983, p = 0.038), CD11c + monocyte %monocyte in cDC group (OR = 0.704, 95%CI:0.514–0.966, p = 0.030), CX3CR1 on CD14+ CD16− monocyte in Monocyte group (OR = 0.717, 95%CI:0.548–0.939, p = 0.016), Mo MDSC AC in Myeloid cell group (OR = 0.763, 95%CI:0.619–0.939, p = 0.011), CD45 on granulocyte in TBNK group (OR = 0.621, 95%CI:0.391–0.984, p = 0.042).ConclusionThe findings suggest that certain specific immune cell phenotypes, particularly B cell and Treg cell subpopulations, are causally associated with GBS, providing potential targets for the clinical treatment of GBS.
Background: Guillain-Barre Syndrome (GBS) is a severe autoimmune neuropathy that has to be treated quickly and efficiently in emergency situations, when both cost and effectiveness are vital. Objective: To compare the clinical outcomes and cost-effectiveness of plasmapheresis and intravenous immunoglobulin (IVIG) in managing acute GBS in emergency departments. Methodology: A prospective observational study conducted from January to December 2023 evaluated the treatment of adults with acute GBS using IVIG or plasmapheresis. Cost analysis was done in conjunction with clinical outcomes assessment, such as improvement in motor function, length of recovery, and length of hospital stay. Each therapy group consisted of 39 individuals, and data were gathered via patient evaluations and medical records. Through statistical analysis, the two regimens' costs and results were compared. Results: Plasmapheresis proved significantly more cost-effective than IVIG, with an average treatment cost of 295,000 Pakistani rupees (PKR) per patient versus 587,000 PKR for IVIG. Plasmapheresis showed a greater mean improvement in motor function (Glasgow Coma Scale (GCS) score change of 5.93 ± 2.17) compared to IVIG (4.98 ± 2.34), though the difference was not statistically significant (p=0.091). Recovery time was slightly shorter with plasmapheresis, averaging 8.26 ± 1.53 weeks versus 8.74 ± 1.87 weeks for IVIG (p=0.282). Hospital length of stay was marginally reduced with plasmapheresis (12.16 ± 3.28 days) compared to IVIG (12.95 ± 3.64 days, p=0.302). The time to reach maximum improvement was also shorter with plasmapheresis (6.51 ± 1.24 weeks) compared to IVIG (7.08 ± 1.32 weeks, p=0.260). Additionally, plasmapheresis had a lower complication rate, with 4 out of 39 patients (10.25%) experiencing complications, compared to 8 out of 39 patients (20.51%) with IVIG. Conclusion: Plasmapheresis is more cost-effective than IVIG for acute GBS management in emergency departments, offering similar or potentially superior clinical outcomes at a lower cost.
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