Severe diabetic ketoacidosis and autoimmune pancreatitis with SIRS in an adolescent with LRBA deficiency - A rare complication of a common primary immunodeficiency disease.
case_report · Level V
Where this comes from
- Record sourced from PubMed, PMID 35516664.
- Also identified by DOI 10.4103/jfmpc.jfmpc_1220_21 and PMC identifier 9067192.
- Licence recorded as CC BY-NC-SA.
- Because redistribution is not established, this page shows the abstract only. Follow the links below for the full text.
Abstract
Common variable immunodeficiency is the most common primary immunodeficiency disorder. Lipopolysaccharide (LPS)-responsive beige-like anchor protein (LRBA) deficiency is categorized as a common variable immunodeficiency associated with autoimmune manifestations and inflammatory bowel diseases. We report a rare case, an adolescent presenting with severe diabetic ketoacidosis (DKA) and acute pancreatitis with multiorgan dysfunction with common variable immunodeficiency (CVID) with homozygous <i>LRBA</i> mutation.