Chronic granulomatous disease (CGD), an inherited immunodeficiency resulting from defects in the reduced NAD phosphate oxidase complex rendering phagocytes deficient in producing the superoxide anion necessary for normal killing of bacterial and fungal microorganisms, leads to severe and recurrent infections, inflammatory granulomatous complications, and autoimmune diseases. 1 It is associated with substantial morbidity and premature death. Hematopoietic cell transplantation (HCT) is the only long-term curative therapy; however, historically, utility was limited by high rates of graft failure and transplant-related morbidity and mortality. Transplant survival and graft outcome have improved dramatically over the past 10 years as a result of reduced-toxicity conditioning (RTC) regimens, detailed graftselection hierarchy, superior HLA-matching technology, better cell-dosed grafts, greater availability of grafts, improved supportive care, and more effective antimicrobial therapy. We studied transplant survival post-HCT for CGD in a supraregional immunology transplant center in the United Kingdom.

Complications were recorded until the 30 th postoperative day.Minor complications, defined as any deviations from normal postoperative course with or without administration of pharmacological treatment, are the most common ( infections, metabolic, hematological disturbances ).About a quarter of patients had severe complications, defined with uni or multiorgan dysfunction, need for reoperation and death, which is still high.Predictor factors for severe postoperative complications were very low birth weight ( BW<1500 g), GA < 32 weeks and abdominal operations.This single centre retrospective analyses show that very low gestation age, very low birth weight and abdominal surgery present risk factors for severe postoperative complications in our NICU patients.

IgE is 268IU/ml. Specific IgE to Tomato-0.02, Strawberry-0.01, Kiwi fruit-0.03, . Thyroid function test, MRI brain were normal. Investigations performed for Wilson's disease, which were unremarkable. Coeliac screen was normal. Hereditary angioedema scree is normal There was no record of basal serum tryptase level. Treatment Initial diagnosis of idiopathic anaphylaxis made. Later with new neurological and eye symptoms, diagnosis of mast cell activation syndrome suspected, and he was started on trial of oral sodium cromoglycate capsules.In next clinic follow up, his symptoms were completely resolved and feedback from school was excellent in terms of concentration. Discussion Symptoms of allergic disorders are very wide. Mast cell activation syndrome especially with multisystem involvement should be a differential to Idiopathic Anaphylaxis.