الفهرس 
IntroductionOnly 14 pages are availabe for public view
 |  | from | 156 |  |  |
| | | from | 156 | | |
Abstract
Primary immunodeficiency diseases (PIDs) are a heterogeneous group of inherited disorders affecting the immune system and responsible to many life-threatening complications.
Plasma-derived immunoglobulin G (IgG) is on WHO’s Essential Medicines List, yet developing countries face severe shortages of this critical treatment.
This hospital based prospective non randomized cross over clinical trial on a cohort of 21 patients diagnosed as primary immunodeficiency disease (PID) in Assiut university pediatric hospital on standard IVIG therapy, in the years from October 2020 to October 2022. with age ranged from 1year to 18 years
This study was trying to confirm the safety and efficacy of a newly developed preparation of MP-IVIG in children with primary immunodeficiency (PID)
All study group patients were subjected to complete history taking and clinical examination and the ten worning signs of JMF were reviewed.
MP-IVIG was prepared in our plasma fractionation lab of CBTS by using single use processing system for minipool intravenous immunoglobulin set from VIPS company. A simple and practical method for producing virally inactivated MP-IVIG from domestic recovered plasma collected was developed by El Ekiaby et al, 2015. The methods allow for preparation MP-IVIG from 20 plasma donations
MP-IVIG dose: equivalent to 0.7 g/ kg /4 weeks (Blood group specific) over a 4-hour to 6-hour period (initial infusions rate of 3 ml/1st 30minute then Volumes could be increased to 15ml/30minutes followed by maximum of 60 ml/h as tolerated. MP-IVIG Infusion through a syringe pump, the vital signs of patients were monitored by ICU monitor and clinical signs of any adverse effect were recorded.
Laboratory investigations were done to the patients as Complete Blood Count (CBC), Kidney function test, Liver function test, Electrolyte (sodium (Na+), potassium(K+), magnesium (Mg), calcium and finally immunoglobulin levels assay.
In this study, Of the 21 PID-patients who received MP-IVIG, 7 (6.2%) patients had a mild side effect, no moderate or severe side effects had occurred comparing to 5 (5.3%) patients on standard IVIG had mild side effect with no moderate or severe side effect was occurred. No hemolytic transfusion reactions were recorded with MP-IVIG. The rate of hospital admition was decreased from 95.2% to 23.8% with MP-IVIG and Both MP-IVIG and STD-IVIG decreased severe bacterial infection and hospital admission with no clinical significance difference (p. value >0.05).The rate of serious bacterial infection as pneumonia before the study was 66.6%, decreased to 9.5% with MP-IVIG and to 5.9% with STD-IVIG while the rate of acute bacterial gastroenteritis was 4.5% with MP-IVIG and 5.9% with STD-IVIG
The level of serum IgG before the study (n=21) was 465.19±87.79 mg/dl while after the end of the MP-IVIG phase (24h after the last 6th dose of MP-IVIG) (the peak level) was1485.50±373.09 mg/dl.
In this study, we demonstrate that early diagnosis of PID is mandatory for early treatment and avoidance of complications, Mini-Pool IVIG plasma fractionation can be implemented easily in the developing countries with minimal resources and the local preparation of plasma products MP-IVIG with safety and efficacy similar to industrial products