Fulminant meningococcemia in a child with hereditary deficiency of the seventh component of complement and proteinuria. 1980

C Loirat, and D Buriot, and A P Peltier, and P Berche, and Y Aujard, and C Griscelli, and H Mathieu

A previously healthy 14-year-old boy presented with fulminant meningococcemia. He was found to have a total deficiency of C7. His serum totally lacked bactericidal activity against Neisseria meningitidis. Addition of purified C7 restored the serum hemolytic and bactericidal activity. Susceptibility to disseminated Neisseria infections has previously been reported in 3 patients with C7 deficiency, as well as in a few patients with deficiency of C5, C6 and C8. These findings emphasize the importance of intact complement mediated bactericidal activity in host defense against disseminated Neisseria infections. Evaluation of the complement system in individuals with Neisseria infections appears mandatory.

UI MeSH Term Description Entries
D008297 Male Males
D008589 Meningococcal Infections Infections with bacteria of the species NEISSERIA MENINGITIDIS. Meningococcal Septicemia,Infections, Meningococcal,Meningococcal Disease,Infection, Meningococcal,Meningococcal Diseases,Meningococcal Infection,Septicemia, Meningococcal
D011507 Proteinuria The presence of proteins in the urine, an indicator of KIDNEY DISEASES. Proteinurias
D003184 Complement C7 A 93-kDa serum glycoprotein encoded by C7 gene. It is a polypeptide chain with 28 disulfide bridges. In the formation of MEMBRANE ATTACK COMPLEX; C7 is the next component to bind the C5b-6 complex forming a trimolecular complex C5b-7 which is lipophilic, resembles an integral membrane protein, and serves as an anchor for the late complement components, C8 and C9. C7 Complement,Complement 7,Complement Component 7,C7, Complement,Complement, C7,Component 7, Complement
D006680 HLA Antigens Antigens determined by leukocyte loci found on chromosome 6, the major histocompatibility loci in humans. They are polypeptides or glycoproteins found on most nucleated cells and platelets, determine tissue types for transplantation, and are associated with certain diseases. Human Leukocyte Antigen,Human Leukocyte Antigens,Leukocyte Antigens,HL-A Antigens,Antigen, Human Leukocyte,Antigens, HL-A,Antigens, HLA,Antigens, Human Leukocyte,Antigens, Leukocyte,HL A Antigens,Leukocyte Antigen, Human,Leukocyte Antigens, Human
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D012397 Rosette Formation The in vitro formation of clusters consisting of a cell (usually a lymphocyte) surrounded by antigenic cells or antigen-bearing particles (usually erythrocytes, which may or may not be coated with antibody or antibody and complement). The rosette-forming cell may be an antibody-forming cell, a memory cell, a T-cell, a cell bearing surface cytophilic antibodies, or a monocyte possessing Fc receptors. Rosette formation can be used to identify specific populations of these cells. Immunocytoadherence,Formation, Rosette,Formations, Rosette,Immunocytoadherences,Rosette Formations

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