Nutritional status of children with primary immunodeficiency: a single center experience

BACKGROUND Nutritional status in primary immunodeficiencies (PID) is a major factor influencing immune defence. We aimed to evaluate the nutritional status in patients with PID. METHODS Demographic findings, anthropometric measurements of 104 patients were recorded for this cross-sectional study. RESULTS Combined immunodeficiencies (n=49), predominantly antibody deficiencies (n=28) and phagocytic system disorders (n=17) were the major groups. In total, 44 (42.3%) patients had at least one or more anthropometric measurements below -2SD. Chronic, acute and mixed-type malnutrition were detected in 18.3%, 16.3%, 7.7% of the patients respectively. No significant difference was detected among groups regarding anthropometric measurements; higher malnutrition rates were observed in combined immune deficiency less profound than severe combined immunodeficiency (SCID) (52%), chronic granulomatous disease (66.6%), and X-linked agammaglobulinemia (50%) patients. Severe malnutrition was present in 22 (21.2%) of the patients, though not significant, it was more common in phagocytic system disorder group. All patients in SCID group were after hematopoietic stem cell transplantation (HSCT) and 50% of them had malnutrition. There was also no significant difference regarding age, sex, anthropometric indexes (WfA, L/HfA, BMI Z-scores), malnutrition types, and prevalence of malnutrition among three groups. Only the hospitalization history inversely affected body mass index (BMI) and weight for age Z-scores (p<0.0001). In patients with malnutrition, daily caloric intake was at least 20% or more below the requirement. CONCLUSIONS Regardless of the type of immunodeficiency, nutritional status was poor in PID and hospitalization is the most important determinant of nutritional status. Even in post-HSCT status, nutritional support should be continued.