Recurrent infections and staphylococcal liver abscess in a child with C1r deficiency.
Complete absence of the C1r portion of the first component of complement was found in a 2 1/2-year-old boy of Puerto Rican origin who presented with a staphylococcal liver abscess. His medical history also included two episodes of pneumonia complicated by a pneumatocele and empyema, purulent staphylococcal lymphadenitis, recurrent otitis media, and pneumococcal bacteremia. The C1s component of complement was 50% of normal, and C4 was elevated. Other immunologic tests, including nitroblue tetrazolium test, and IgE were normal. This is the tenth patient reported with C1r deficiency. The patient differs from other reported patients with C1r deficiency in that he presented with a liver abscess, an infection that has not been reported in patients with complement deficiencies, and in that he has an apparent susceptibility to staphylococcal infection.
Garty
B Z
BZ
Division of Allergy and Immunology, Children's Hospital of Philadelphia, University of Pennsylvania Medical School 19104.
Conley
M E
ME
Douglas
S D
SD
Kolski
G B
GB
D002675:Child, Preschool; D003172:Complement C1 / Q000172:deficiency*; D006801:Humans; D007239:Infections / Q000276:immunology; D008100:Liver Abscess / Q000276:immunology* / Q000382:microbiology; D008297:Male; D012008:Recurrence; D013203:Staphylococcal Infections / Q000276:immunology*
D003172:Complement C1