Efficacy and Safety of Therapeutic Plasma Exchange in Pediatric Patients with Guillain-Barré Syndrome: A Retrospective Study

Author:

Ali Tahani1,FAKHER Faihaa HKIMA ABOU1,Alawir Malek2,Allababidi Abdulsater2,Hasan Aya Sheikh3

Affiliation:

1. Children's University Hospital

2. University of Damascus

3. Al-Baath University

Abstract

Abstract 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 GBS. 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 by accelerating the restoration of normal neurological functions and yielding better outcomes. However, balancing its benefits with potential risks requires careful clinical judgment and rigorous monitoring to ensure patient safety and optimize outcomes.

Publisher

Research Square Platform LLC

Reference60 articles.

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2. R. A.C. Hughes and J. M. Newsom-Davis and G. D. Perkin and J. M. Pierce (1978) Controlled trial prednisolone in acute polyneuropathy. Lancet (London, England) 2: 750-753 https://doi.org/10.1016/S0140-6736(78)92644-2, Lancet, 80682, 10, Acute Disease,Adolescent,Adult,Aged,Child,Clinical Trial,Clinical Trials as Topic,Disability Evaluation,Drug Evaluation,Follow-Up Studies,Humans,J M Newsom-Davis,J M Pierce,MEDLINE,Middle Aged,NCBI,NIH,NLM,National Center for Biotechnology Information,National Institutes of Health,National Library of Medicine,Polyradiculoneuropathy / drug therapy*,Polyradiculoneuropathy / rehabilitation,Prednisolone / administration & dosage,Prednisolone / therapeutic use*,Preschool,PubMed Abstract,R A Hughes,Random Allocation,Randomized Controlled Trial,Time Factors,doi:10.1016/s0140-6736(78)92644-2,pmid:80682, 8093, 0140-6736, In a multicentre, randomised trial of prednisolone in acute polyneuropathy of undetermined ætiology (Guillain-Barr ésyndrome), 21 patients were treated with prednisolone (60 mg daily for one week, 40 mg daily for four days, and then 30 mg daily for three days) and 19 did not have steroid treatment. Patients were graded on a six-point scale by one of two neurologists who had no knowledge of the treatment schedule. Reassessment at one, three, and twelve months consistently showed greater improvement in the control than the prednisolone group but the only statistically significant result was in the improvement at three months among patients entered to the trial within a week of onset of illness. The 6 control patients had improved by 2 ·5 ±0 ·43 grades by three months from entry to the trial whereas the 10 prednisolone patients had only improved by 0 ·9 ±0 ·46 grades (P<0 ·05). There was 1 death related to the polyneuropathy in each group, and 1 suicide in a control patient during convalescence. 6 prednisolone patients were left with considerable disability compared with 1 control patient. There were 3 relapses in the prednisolone group, but none in the control group. The results indicate that steroid treatment is not beneficial and can be detrimental in acute neuropathy of undetermined ætiology. © 1978.

3. Martin Fosburg and Mary Dolan and Richard Propper and Lawrence Wolfe and Sherwin Kevy (1983) Intensive plasma exchange in small and critically ill pediatric patients: techniques and clinical outcome. Journal of clinical apheresis 1: 215-224 https://doi.org/10.1002/JCA.2920010405, J Clin Apher, 6400415, Adolescent,Anemia,Autoimmune / therapy,Child,Citrates / pharmacology,Citric Acid,Graft Rejection,Hemolytic,Heparin / therapeutic use,Humans,Immunoglobulin A / analysis,Immunoglobulin G / analysis,Immunoglobulin M / analysis,Infant,Kidney Transplantation,M Dolan,M Fosburg,MEDLINE,NCBI,NIH,NLM,National Center for Biotechnology Information,National Institutes of Health,National Library of Medicine,Plasma Exchange / adverse effects,Plasma Exchange / instrumentation,Plasma Exchange / methods*,Platelet Count,Preschool,PubMed Abstract,S Kevy,doi:10.1002/jca.2920010405,pmid:6400415, 4, 0733-2459, Standard apheresis techniques require modification of use in children, particularly those with serious concurrent medical problems, as they are prone to apheresis ‐induced disturbances of volume, metabolism, and coagulation. We report 112 plasma exchanges (TPE) on 11 children, 9 of whom weighed less than 20 kg and 7 of whom were critically ill. All were treated on continuous flow apparatus; seven on centrifugal systems (CS), two on a membrane filtration system (MFS), and two on both. Perturbations of blood and red blood cell (RBC) volume were prevented by priming the extracorporeal circuits with a red cell saline mixture having an hematocrit equal to or greater than the patient's hematocrit. Priming volume and minimal flow rates were 170 ml and 40 cc/min (MFS) and 350 ml and 10 cc/min (CS). TPE dose varied from 1.3 to 3 plasma volumes. Immunoglobulins fell by the following amounts: IgG 43.7%, IgA 36.7%, and IgM 41% per plasma volume. Platelets fell by 20 –90% (CS) and 5 –7% (MFS). Vascular access was obtained by various means including Thomas shunts, dialysis catheters, and standard 16 –19 gauge butterflies and angiocaths. Bleeding in patients with coagulopathies was prevented by using repeated small boluses of heparin to maintain a clotting time of 2.5 –3 minutes. Morbidity from TPE was limited to citrate toxicity (2 patients) and transient pulmonary edema (1 patient). Treatment outcome was successful in 8 out of 11 patients. We have shown that if PEX is otherwise indicated, it should not be withheld solely for patient size or the complexity of concurrent medical problems. Copyright © 1983 Wiley ‐Liss, Inc., A Wiley Company

4. R. P. Kleyweg and F. G.A. van der Mech é and J. Meulstee (1988) Treatment of Guillain-Barr é syndrome with high-dose gammaglobulin. Neurology 38: 1639-1641 https://doi.org/10.1212/WNL.38.10.1639, Neurology, 2458549, Adult,Aged,Case Reports,Child,F G van der Mech é,Female,Humans,Injections,Intravenous,J Meulstee,MEDLINE,Male,Middle Aged,NCBI,NIH,NLM,National Center for Biotechnology Information,National Institutes of Health,National Library of Medicine,Polyradiculoneuropathy / therapy*,PubMed Abstract,R P Kleyweg,doi:10.1212/wnl.38.10.1639,gamma-Globulins / administration & dosage,gamma-Globulins / therapeutic use*,pmid:2458549, 10, 0028-3878, Intravenous gammaglobulin (IVGG) can improve the clinical courae of several immune-mediated diseases. We report the first results of such treatment in eight patients with severe Guillain-Barr é syndrome (GBS). We observed that IVGG was beneficial in at least some of the patients with severe GBS. These results should be confirmed in a randomized trial. © 1988 American Academy of Neurology.

5. Mark A. Epstein and John T. Sladky (1990) The role of plasmapheresis in childhood Guillain-Barr é syndrome. Annals of neurology 28: 65-69 https://doi.org/10.1002/ANA.410280112, Ann Neurol, 2375635, Adolescent,Child,Electrophysiology,Female,Humans,Infant,J T Sladky,M A Epstein,MEDLINE,Male,NCBI,NIH,NLM,National Center for Biotechnology Information,National Institutes of Health,National Library of Medicine,Non-U.S. Gov't,P.H.S.,Plasmapheresis*,Polyradiculoneuropathy / physiopathology,Polyradiculoneuropathy / therapy*,Preschool,PubMed Abstract,Research Support,U.S. Gov't,doi:10.1002/ana.410280112,pmid:2375635, 1, 0364-5134, Plasmapheresis has been advocated in the treatment of childhood Guillain ‐Barr é syndrome under the assumption that the results of adult series can be extrapolated to children. To test this assumption, we retrospectively evaluated the medical charts of all children who were admitted to The Children's Hospital of Philadelphia between January 1984 and March 1989, with the diagnosis of Guillain ‐Barr é syndrome. Of the 30 patients identified, 7 were excluded because they had mild disease. Of the remaining 23, 9 underwent plasmapheresis and 14 served as historic control subjects. The two groups were similar with respect to age, presenting symptoms, finding on initial physical examination, and antecedent illnesses at the time of diagnosis. The mean time to recover to Grade 2 (independent ambulation) was significantly shorter in the plasmapheresis ‐treated group, 24.0 ± 25.4 days, compared to 60.2 ± 43.6 days in control subjects (mean ± 1 SD). Our results indicate that plasmapheresis diminishes morbidity in childhood Guillain ‐Barr é syndrome by shortening the interval until recovery of independent ambulation. Copyright © 1990 American Neurological Association

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