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العنوان
Study the Role of Plasmapheresis versus Intravenous immunoglobulin in the treatment of Guillain-Barre syndrome in pediatric patients /
المؤلف
Hewade , Ahmed Saied Mahmoud .
هيئة الاعداد
باحث / أحمد سعيد محمود هويدي
مشرف / سامح عبدالله عبد النبي
مناقش / نجوان يسري صالح
مناقش / أحمد انور خطاب
الموضوع
Guillain-Barré syndrome. Pediatrics.
تاريخ النشر
2021.
عدد الصفحات
102 p. :
اللغة
الإنجليزية
الدرجة
ماجستير
التخصص
طب الأطفال ، الفترة المحيطة بالولادة وصحة الطفل
تاريخ الإجازة
20/2/2021
مكان الإجازة
جامعة المنوفية - كلية الطب - قسم طب الأطفال
الفهرس
Only 14 pages are availabe for public view

from 119

from 119

Abstract

Guillain-Barré syndrome (GBS) is a rare neurological disorder in which the body’s immune system mistakenly attacks part of its peripheral nervous system, the network of nerves located outside of the brain and spinal cord.
The incidence of Guillain-Barré syndrome in pediatric (GBS) range from 0.5 to 1.5 cases per 100,000 population in individuals younger than 18 years.
The exact cause of Guillain-Barré syndrome isn’t known. The disorder usually appears days or weeks after a respiratory or digestive tract infection. Rarely, recent surgery or immunization can trigger Guillain-Barré syndrome. In Guillain-Barré syndrome, your immune system, which usually attacks only invading organisms begins attacking the nerves.
It’s characterized by symmetrical limb weakness typically begins as proximal lower extremities weakness and ascends to involve the upper extremities, truncal muscles, and head Inability to stand or walk despite reasonable strength. Respiratory muscle weakness with shortness of breath may be present. Cranial nerve palsies (III-VII and IX-XII) and bulbar manifestation may be present.
Paresthesia in the hands and feet occurs in more than 80% of patients.
Autonomic dysfunction may include tachycardia, bradycardia, dysrhythmias, wide fluctuations in blood pressure, and postural hypotension, urinary retention, constipation.
Clinical subtypes include: AIDP, AMAN, AMSAN, Pharyngo-cervical-brachial variant, Miller Fisher syndrome.
Diagnostic criteria include acute progressive bilateral ascending symmetrical weakness of the extremities with areflexia or hyporeflexia; the cerebrospinal fluid (CSF) showing albumin cytological dissociation was defined as CSF with a raised protein and total cell count of ≤10/mm3 & electrophysiological study revealing features of demyelination / axonal neuropathy.
A characteristic finding in Guillain-Barré syndrome is an elevated protein level with normal level of white blood cells (”albumin cytological dissociation”), this distinguishes it from a number of other conditions (such as lymphoma and poliomyelitis) where both the protein and the cell count are elevated.
MRI with contrast is administered if the diagnosis is suspected as non-contrast sequences are essentially normal. Typical findings in Guillain-Barré syndrome are surface thickening and contrast enhancement on the conus medullaris and the nerve roots of the cauda equina.
Nerve conduction studies (NCS) are very helpful in the diagnostic workup and prognostic evaluation of patients with suspected GBS.
Complications of GBS include Breathing difficulties, Residual numbness or other sensations, Heart and blood pressure problems, Pain, Bowel and bladder function problems, Blood clots, Pressure sores, Relapse.
Treatment includes Prehospital care, emergency department care, ICU treatment. Medication, rehabilitation.
Summary
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Medications include IVIG, Plasmapheresis, steroids, Eculizumab.
Our aim was to compare the efficacy of plasmapheresis versus IVIG regarding outcome parameters in form of (recovery, mortality rate, residual disability, length of hospital and Pediatric intensive care unit (PICU) stay and Need for ventilation).
Our observational study was carried out on 70 patients diagnosed with Guillain barre syndrome, fulfilled all inclusion & exclusion criteria, were admitted to Pediatric Intensive Care Unit (PICU) of Menoufia University Hospital from period January 2019 to June 2020.
Informed consent was taken from the parents before enrollment of their children in the study. The studied patients were randomly distributed according to the line of treatment into two groups (IVIG, Plasmapheresis), randomization was performed using computer operated system.
Inclusion criteria included children from 1month to 18 years who had acute GBS and the diagnosis of GBS was based on the following criteria; acute progressive symmetrical weakness of the extremities with areflexia or hyporeflexia; the cerebrospinal fluid (CSF) showing albumin cytological dissociation was defined as CSF with a raised protein and total cell count of ≤10/mm3 & electrophysiological study revealing features of demyelination / axonal neuropathy.
Exclusion criteria included other causes which can produce symptoms similar to GBS symptoms e.g.: Acute myelopathy (Cord compression), muscle disorders (Polymyositis), Peripheral neuropathy (acute axonal neuropathy), delayed motor, mental and cerebral palsy.
All children included in this study were subjected to detailed history with special emphasis on:
Duration of PICU stay, duration of word stays, need of ventilation and duration of ventilation.
Complete physical examination especially Neurological assessment: Motor assessment (Tone, Power and reflexes), Sensation, Coordination. Every patient underwent initial scoring using the modified Barthel scale (MBS) &modified Rankin’s scale (MRS), Single breath count (SBC).
Labs include CBC, CRP, ESR, LFT, RFT, CK.
Nerve conduction was done to every patient.
Outcome of patients: was assessed using, MRS & MBS, SBC& outcome parameters as: recovery, mortality rate, residual disability, length of hospital and PICU stay, need of mechanical ventilation.
We found that:
✓ There was significant increase in mean age in group 1 compared with group 2 (p value= 0.01).
✓ The majority of affected patients with GBS were male (72.9%), from urban areas (81.42%) and of middle socioeconomic status (98.6%).
Summary
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✓ The most common finding was URTI (81.4%) followed by diarrhea (17.1%) and vaccination (1.4%).
✓ The total cohort patients were fully conscious and unable to walk.
✓ The majority of affected patients with GBS had normal speech (98.6%) and did not have bulbar signs (81.4%) while (18.6%) of patients had bulbar signs.
✓ The majority of affected patients with GBS had hypotonia (95.7%), abnormal power (97.1%), hyporeflexia (97.1 %) and normal sensation (85.7%).
✓ There was no significant difference among studied groups regarding nerve conduction velocity and the most common finding in NCV was demyelinated (55.7%) followed by mixed (22.8%), axonal (21.5%).
✓ There was significant increase in sepsis in group 1(17.3%) compared with group 2 (0.0%) p value=0.03.
✓ The proportion of patients suffered from VAP and dysautonomia was higher in group1 compared with group 2.
✓ There was significant increase in group 1 compared with group 2 regarding number of patients admitted in PICU and duration of admission in PICU (P value=0.03, 0.038 respectively).
✓ There was significant increase in group 1 compared with group 2 regarding need for mechanical Ventilation (p value=0.04).
✓ Mean duration of ventilation was higher in group 1 compared with group 2.
✓ Mean MRS score was lower in group 2 compared with group 1 on discharge (p value=0.003).
✓ Mean MBS score was higher in group 2 compared with group 1 on discharge with (p value=0.006).
✓ Mean Single breath count was higher in group 2 compared with group 1 on discharge (p value=0.047).