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Intravenous immunoglobulin in very severe childhood Guillain-Barré syndrome
S C Singhi1, M Jayshree, P Singhi
1Pediatric Intensive Care Unit, Postgraduate Institute of Medical Education and Research, Chandigarh, India. medinst@pgi.chd.nic.in
Insights
Intravenous immunoglobulin (IVIG) therapy significantly improves outcomes for children with severe Guilain-Barré syndrome (GBS). IVIG reduces respiratory support needs, shortens intensive care unit stays, and accelerates recovery and ambulation in pediatric GBS patients.
Area of Science:
- Pediatric Neurology
- Immunology
- Critical Care Medicine
Background:
- Guillain-Barré syndrome (GBS) is a rare autoimmune disorder affecting the peripheral nervous system.
- Very severe GBS in children can lead to quadriparesis and respiratory muscle weakness, necessitating intensive care.
- Standard supportive and respiratory care are crucial for managing severe GBS.
Purpose of the Study:
- To evaluate the efficacy of intravenous immunoglobulin (IVIG) therapy in children with very severe GBS.
- To assess the impact of IVIG on respiratory support requirements, intensive care unit (ICU) stay, and long-term functional outcomes.
- To compare outcomes between children treated with IVIG and those receiving standard care.
Main Methods:
- A prospective study of 22 children with very severe GBS receiving IVIG (0.4 g/kg/day for 5 days) alongside supportive care.
- A retrospective control group of 11 children with similar GBS severity and age, receiving only supportive care.
- Comparison of muscle power recovery, ICU length of stay, need for mechanical ventilation, and functional status at 1 and 3 months post-treatment.
Main Results:
- The IVIG group showed significantly earlier onset of muscle power recovery (14.8 days vs. 20.9 days).
- Length of PICU stay was significantly shorter in the IVIG group (20.5 days vs. 50.5 days).
- More children in the IVIG group achieved functional improvement (72.7% vs. 18%) and independent ambulation (68% vs. 36%) compared to controls.
Conclusions:
- Intravenous immunoglobulin therapy markedly improves outcomes in children with very severe GBS.
- IVIG therapy reduces the need for endotracheal intubation and mechanical ventilation.
- IVIG shortens ICU stays and promotes earlier ambulation in pediatric GBS patients.
Abstract:
To evaluate intravenous immunoglobulin (IVIG) therapy in children with very severe Guilain-Barré syndrome (GBS) with reference to the need for respiratory support, ICU stay and long-term outcome, we studied 33 children with very severe GBS and quadriparesis and/or respiratory muscle weakness admitted to the Pediatric Intensive Care Unit (PICU) of PGIMER, Chandigarh. Cases (n = 22, IVIG group) were enrolled prospectively, and controls (n = 11), similar to cases in age and severity of illness, retrospectively. All children received similar supportive and respiratory care. In addition, cases were given IVIG (Sandoglobulin, Sandoz) 0.4 g/kg bodyweight per day for 5 days. The mean age, duration of symptoms prior to admission and severity of illness in the two groups were similar. In the IVIG group, onset of recovery of muscle power was significantly earlier (day 14.8 (6.8) of illness vs day 20.9 (8.6), p < 0.05) and the length of PICU stay significantly shorter (20.5 (13.0) days vs 50.5 (33.3) days, p < 0.01). Sixteen (72.7%) children in the IVIG group had improved by at least one functional grade after 1 month and 15 (68%) were walking independently after 3 months compared with two (18%) and four (36%) controls, respectively (p < 0.05). The number of children who needed endotracheal intubation and mechanical ventilation and the duration of mechanical ventilation was significantly less in the IVIG-treated group. We conclude that in very severe GBS in children IVIG therapy improves outcome to a remarkable extent, reduces the need for intubation and mechanical ventilation, shortens the length of stay in ICU, and promotes ambulation sooner.