Hyperimmunoglobulinemia D and periodic fever syndrome (HIDS) in a child with normal serum IgD, but increased serum IgA concentration.
This report describes a boy with hyperimmunoglobulinemia D and periodic fever syndrome (HIDS). The serum IgD level was normal, but the serum IgA concentration was markedly elevated. In addition, he had a history of orchitis on two occasions, a previously unreported manifestation of HIDS. This report expands the clinical and laboratory features associated with HIDS and serves to emphasize that a normal serum IgD level does not exclude the diagnosis of HIDS.
Saulsbury
Frank T
FT
Division of Immunology and Rheumatology, Department of Pediatrics, University of Virginia Health System, Charlottesville, Virginia 22908, USA. fts@virginia.edu
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002648:Child; D004252:DNA Mutational Analysis; D010505:Familial Mediterranean Fever / Q000097:blood* / Q000188:drug therapy / Q000235:genetics; D015870:Gene Expression / Q000235:genetics; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood*; D007072:Immunoglobulin D / Q000097:blood*; D008297:Male; D017853:Phosphotransferases (Alcohol Group Acceptor) / Q000235:genetics; D017354:Point Mutation / Q000235:genetics; D011239:Prednisolone / Q000627:therapeutic use
D000893:Anti-Inflammatory Agents; D007070:Immunoglobulin A; D007072:Immunoglobulin D; D011239:Prednisolone; D017853:Phosphotransferases (Alcohol Group Acceptor); C021796:mevalonate kinase