Desensitisation of the youngest patient with Pompe disease in response to alglucosidase alfa

sometimes GI diseases are not controlled well with IVIG replacement, because these preparations contain IgG which cannot reach the lumen of the intact gut and also contain very little amount of IgA which is a vital component of mucosal defence. Recent experimental evidence suggests that IVIG could exert an effect more than passive substitution of antibodies could do against pathogenic microbes; it rectifies the defective signalling and induces an optimal functioning of cellular compartment, thus re-establishing immune homeostasis. Theoretically, treatment with oral immunoglobulin has not been successful, because IgG is rapidly destroyed before reaching the small intestine. There is an animal study, which showed an induction of oral tolerance by oral administration of IVIG against anti-phospholipid syndrome in naïve mice. The presented case is a unique one, since the patient experienced improvement of chronic diarrhoea following oral administration of IVIG. Our results point to a possible role of oral IVIG in the improvement of chronic diarrhoea in CVID patients, especially in those infested by organisms such as Giardia. However, further original multi-centre studies are needed to test the efficacy of oral administration of immunoglobulin in improvement of diarrhoea severity in those with CVID.