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Clinical significance of complement deficiencies


, : Clinical significance of complement deficiencies. Annals of the New York Academy of Sciences 1173: 108-123

The complement system is composed of more than 30 serum and membrane-bound proteins, all of which are needed for normal function of complement in innate and adaptive immunity. Historically, deficiencies within the complement system have been suspected when young children have had recurrent and difficult-to-control infections. As our understanding of the complement system has increased, many other diseases have been attributed to deficiencies within the complement system. Generally, complement deficiencies within the classical pathway lead to increased susceptibility to encapsulated bacterial infections as well as a syndrome resembling systemic lupus erythematosus. Complement deficiencies within the mannose-binding lectin pathway generally lead to increased bacterial infections, and deficiencies within the alternative pathway usually lead to an increased frequency of Neisseria infections. However, factor H deficiency can lead to membranoproliferative glomerulonephritis and hemolytic uremic syndrome. Finally, deficiencies within the terminal complement pathway lead to an increased incidence of Neisseria infections. Two other notable complement-associated deficiencies are complement receptor 3 and 4 deficiency, which result from a deficiency of CD18, a disease known as leukocyte adhesion deficiency type 1, and CD59 deficiency, which causes paroxysmal nocturnal hemoglobinuria. Most inherited deficiencies of the complement system are autosomal recessive, but properidin deficiency is X-linked recessive, deficiency of C1 inhibitor is autosomal dominant, and mannose-binding lectin and factor I deficiencies are autosomal co-dominant. The diversity of clinical manifestations of complement deficiencies reflects the complexity of the complement system.

US$19.90

PMID: 19758139

DOI: 10.1111/j.1749-6632.2009.04633.x


Other references

Hassan, R.; Felisbino, F.; Stefanoff, C.Gustavo.; Pires, V.; Klumb, C.E.; Dobbin, J.; Seuánez, Héctor.N.; Renault, I.Zalcberg., 2007: Burkitt lymphoma/leukaemia transformed from a precursor B cell: clinical and molecular aspects. Burkitt lymphoma/leukaemia (BL/L) is a heterogeneous disease with respect to epidemiological patterns and cell origin. The occurrence of BL/L with an immature phenotype raises the question whether this phenotype might be a consequence of early B-c...

Meek, R.L.; Walsh, K.A.; Palmiter, R.D., 1982: The signal sequence of ovalbumin is located near the NH2 terminus. Ovalbumin, unlike other secretory proteins, is synthesized and secreted without cleavage of a hydrophobic signal peptide. Kinetic experiments were performed in a cell-free translation system to measure the minimum size of ovalbumin nascent chains...

Heaven, P.C., 1996: Personality and self-reported delinquency: a longitudinal analysis. This study assessed the personality factors associated with self-reported delinquency. Respondents were 282 14-year-olds who were traced for follow-up 2 years later. The follow-up success rate was more than 80%. In line with previous work which ha...

Brown, V.; Shurben, D.; Miller, W.; Crane, M., 1994: Cadmium toxicity to rainbow trout Oncorhynchus mykiss Walbaum and brown trout Salmo trutta L. over extended exposure periods. The toxicity of cadmium in water is well known, but there have been few reports on the long-term effects of this metal on fish at concentrations near the European inland water standard of 5 micrograms Cd liter-1. This paper describes experiments i...

Kamp, P.E.; D.W.t, W.M., 1968: Demonstration of the toxins of Amanita phalloides. Chromatography of a methanolextract of A. phalloides by thinlayer method in 2 directions (methylethylketon, aceton, water: 30, 3, 5; methylethylketon, aceton, water, n. butanol:20, 6, 5, 1.) gives with coloring with cinnamaldehyde and hydrochloric...

Levine B.W., 1970: Stratified dead space in excised perfused dog lung. Federation Proceedings: 269

Gerharz, E.W.; Weingärtner, K.; Köhl, U.; Riedmiller, H., 1994: Haemorrhagic infarction of a prolapsed orthotopic ureterocele causing upper urinary tract obstruction. British Journal of Urology 74(5): 670-671

Spurney, R.F.; Fulkerson, W.J.; Schwab, S.J., 1991: Acute renal failure in critically ill patients: prognosis for recovery of kidney function after prolonged dialysis support. Objective: To clarify the prognosis for eventual recovery of kidney function in patients who experience prolonged dialysis dependence after acute renal failure (ARF). Design: Retrospective, chart review. Setting: Inpatients of a large, referral-ba...

Devochkin F.A.; Evsyutina Z.S., 1976: Characteristics of growth and yield of carrot during mulching and chemical weed control. Doklady Moskovskoi Sel'skokhozyaistvennoi Akademii Imeni K A Timiryazeva 216

Lehmann A., 1968: Modification of the intensity of audiogenic attacks by substances acting upon biogenic amino acids in the mouses brain. Comptes Rendus des Seances de la Societe de Biologie et de ses Filiales 162(1): 24-27