[Hyper-IgD-syndrome].
We report on a 6-year-old Romanian girl with recently diagnosed hyper-IgD-syndrome. The leading symptom of this rare disease are periodic pyrexia, joint involvements (arthralgias/arthritis) and swollen lymph nodes. A permanent increase of alpha 1-acid glycoprotein fucosylation indicates persisting inflammation. Most important in differential diagnosis in familial Mediterranean fever. Therapy is merely supportive as yet, the long-term outlook seems good despite duration of the illness.


CONCLUSIONS
the hyper-IgD-syndrome must be considered in cases of otherwise unexplained periodic fever.
Suschke
J
J
Kinderpoliklinik der Universität München.
Walther
J U
JU
D003937:Diagnosis, Differential; D010505:Familial Mediterranean Fever / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006942:Hypergammaglobulinemia / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology; D007072:Immunoglobulin D / Q000097:blood*; D007223:Infant; D009961:Orosomucoid / Q000378:metabolism
D007072:Immunoglobulin D; D009961:Orosomucoid