Intestinal failure (IF) is a clinical condition for which intestinal transplantation may be indicated. Patients without enteral autonomy have an increased risk of malnutrition which may be associated with immunodeficiency. To date, there is scarce data about IF associated immunodeficiency.

METHODS: We reviewed the immune status (quantitative immunoglobulins, lymphocyte subsets and vaccine titers) of 18 patients with IF who underwent pre-intestinal transplant evaluation from December 2013 to 2014.

RESULTS: Of 18 patients studied, 11 were males (61%). Mean age was 1.3 yr (range 3m- 14yr). The most common etiology leading to IF was Gastroschisis (n=8). Other etiologies included intestinal atresia (n=2), volvulus (n=1), NEC (n=1), pseudoobstruction (n=2), total aganglionosis (n=1), and other (n=3). Severe malnutrition was identified in 22% (z-score below -3). The rest of patients had adequate z-scores.

Gastroschisis: 100% of the children had hypoalbuminemia. Twenty five percent had low IgG. One patient had hypergammaglobulinemia and high IgA due to presumed autoimmunity possibly due to frequent transfusions. Percentages of lymphocyte populations (T, B and NK) were generally preserved. However, absolute B and T cell number were low in 25%.

Of the remaining 10 cases, 90% had hypoalbuminemia. In general the T cell compartment in these patients was preserved. However, 30% had low B cells and 50% had low NK cells. All patients with intestinal atresia had pan-hypogammaglobulinemia suggesting a more global immune defect, perhaps due to maldevelopment of the small intestine and poor lymphocyte homing to the gut during development.

CONCLUSION: Patients with immunodeficiency are prone to infections and worse post transplant outcomes. Pre transplant immune assessment identify abnormalities that may change patient prognosis after transplant. While hypogammaglobulinemia may be partly due to malnutrition, other underlying problems of immune development may exist. Possibly, immune globulin replacement may be a strategy toward better transplant outcomes.

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