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Reduced hemolytic complement activity

MedGen UID:
1380457
Concept ID:
C4476774
Finding
Synonym: Reduced CH50
 
HPO: HP:0025434

Definition

A diminished activity of the classical complement pathway as measured by the assay for 50% haemolytic complement (CH50) activity of serum. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVReduced hemolytic complement activity

Conditions with this feature

Complement component 5 deficiency
MedGen UID:
91003
Concept ID:
C0343047
Disease or Syndrome
A rare genetic disorder with an autosomal recessive pattern of inheritance. It is caused by the ineffective or decreased biosynthesis of the fifth complement component, C5. C5 deficiency may also be acquired acutely post-infection. If C5 is adequately synthesized, its rapid depletion may result in a functional deficiency. Clinical signs of the inherited deficiency present within the second decade of life and are consistent with the signs of recurrent systemic infection. Deficiency of serum C5 and its major cleavage product, C5b, a component of the membrane attack complex, increases susceptibility to Neisserial infections.
Complement component 6 deficiency
MedGen UID:
436639
Concept ID:
C2676232
Disease or Syndrome
Any classic complement early component deficiency in which the cause of the disease is a mutation in the C6 gene.
Hereditary angioedema type 1
MedGen UID:
403466
Concept ID:
C2717906
Disease or Syndrome
A form of hereditary angioedema characterized by acute edema in subcutaneous tissues, viscera and/or the upper airway.
Complement component 4a deficiency
MedGen UID:
482272
Concept ID:
C3280642
Finding
Concentration of the complement component C4a in the blood circulation below the lower limit of normal.
C1Q deficiency 2
MedGen UID:
1841058
Concept ID:
C5830422
Disease or Syndrome
C1q deficiency (C1QD) is a rare autosomal recessive disorder characterized by recurrent skin lesions, chronic infections, and an increased risk of autoimmune diseases, particularly systemic lupus erythematosus (SLE; see 152700) or SLE-like diseases. It has also been associated with chronic glomerulonephritis and renal failure. C1q deficiency presents in 2 different forms, absent C1q protein or presence of a dysfunctional molecule (summary by Topaloglu et al., 1996 and Vassallo et al., 2007). For a discussion of genetic heterogeneity of C1q deficiency, see 613652.

Professional guidelines

PubMed

Liu Y, Thaker H, Wang C, Xu Z, Dong M
Toxins (Basel) 2022 Dec 23;15(1) doi: 10.3390/toxins15010010. PMID: 36668830Free PMC Article
Despotovic JM, Kim TO
Hematology Am Soc Hematol Educ Program 2022 Dec 9;2022(1):90-95. doi: 10.1182/hematology.2022000369. PMID: 36485161Free PMC Article
Chen R, Zhang N, Gao L, Zhong Y, Xu L, Liu H, Zheng Q, Li L
Expert Opin Investig Drugs 2021 Dec;30(12):1231-1240. Epub 2021 Dec 6 doi: 10.1080/13543784.2021.2010704. PMID: 34821184

Recent clinical studies

Etiology

Garnier A, Brochard K, Kwon T, Sellier-Leclerc AL, Lahoche A, Launay EA, Nobili F, Caillez M, Taque S, Harambat J, Michel-Bourdat G, Guigonis V, Fila M, Cloarec S, Djamal-Dine D, de Parscaux L, Allard L, Salomon R, Ulinski T, Frémeaux-Bacchi V, Morin C, Olivier-Abbal P, Colineaux H, Auriol F, Arnaud C, Kieffer I, Brusq C
J Am Soc Nephrol 2023 Sep 1;34(9):1561-1573. Epub 2023 Jun 12 doi: 10.1681/ASN.0000000000000182. PMID: 37303085Free PMC Article
Beard N, Frese M, Smertina E, Mere P, Katelaris C, Mills K
Cochrane Database Syst Rev 2022 Nov 3;11(11):CD013403. doi: 10.1002/14651858.CD013403.pub2. PMID: 36326435Free PMC Article
Manenti L, Rossi GM, Pisani I, Gentile M, Fontana F, Pilato FP, Delsante M, Ricco F, Mignani R, Mele C, Bresin E, Fiaccadori E
J Nephrol 2022 May;35(4):1091-1100. Epub 2021 Nov 10 doi: 10.1007/s40620-021-01189-6. PMID: 34757577
Röth A, Barcellini W, D'Sa S, Miyakawa Y, Broome CM, Michel M, Kuter DJ, Jilma B, Tvedt THA, Fruebis J, Jiang X, Lin S, Reuter C, Morales-Arias J, Hobbs W, Berentsen S
N Engl J Med 2021 Apr 8;384(14):1323-1334. doi: 10.1056/NEJMoa2027760. PMID: 33826820
Chapin J, Terry HS, Kleinert D, Laurence J
Transfus Apher Sci 2016 Apr;54(2):191-8. Epub 2016 Apr 25 doi: 10.1016/j.transci.2016.04.008. PMID: 27156108

Diagnosis

Liu Y, Thaker H, Wang C, Xu Z, Dong M
Toxins (Basel) 2022 Dec 23;15(1) doi: 10.3390/toxins15010010. PMID: 36668830Free PMC Article
Keragala CB, Medcalf RL
Blood 2021 May 27;137(21):2881-2889. doi: 10.1182/blood.2020008951. PMID: 33735914
Xiao L, Harrison DG
Can J Cardiol 2020 May;36(5):635-647. Epub 2020 Jan 24 doi: 10.1016/j.cjca.2020.01.013. PMID: 32389337Free PMC Article
Patel G, Pongracic JA
Allergy Asthma Proc 2019 Nov 1;40(6):441-445. doi: 10.2500/aap.2019.40.4267. PMID: 31690390
Rizk DV, Maillard N, Julian BA, Knoppova B, Green TJ, Novak J, Wyatt RJ
Front Immunol 2019;10:504. Epub 2019 Mar 19 doi: 10.3389/fimmu.2019.00504. PMID: 30941137Free PMC Article

Therapy

Garnier A, Brochard K, Kwon T, Sellier-Leclerc AL, Lahoche A, Launay EA, Nobili F, Caillez M, Taque S, Harambat J, Michel-Bourdat G, Guigonis V, Fila M, Cloarec S, Djamal-Dine D, de Parscaux L, Allard L, Salomon R, Ulinski T, Frémeaux-Bacchi V, Morin C, Olivier-Abbal P, Colineaux H, Auriol F, Arnaud C, Kieffer I, Brusq C
J Am Soc Nephrol 2023 Sep 1;34(9):1561-1573. Epub 2023 Jun 12 doi: 10.1681/ASN.0000000000000182. PMID: 37303085Free PMC Article
Beard N, Frese M, Smertina E, Mere P, Katelaris C, Mills K
Cochrane Database Syst Rev 2022 Nov 3;11(11):CD013403. doi: 10.1002/14651858.CD013403.pub2. PMID: 36326435Free PMC Article
Röth A, Barcellini W, D'Sa S, Miyakawa Y, Broome CM, Michel M, Kuter DJ, Jilma B, Tvedt THA, Fruebis J, Jiang X, Lin S, Reuter C, Morales-Arias J, Hobbs W, Berentsen S
N Engl J Med 2021 Apr 8;384(14):1323-1334. doi: 10.1056/NEJMoa2027760. PMID: 33826820
Chatham WW, Furie R, Saxena A, Brohawn P, Schwetje E, Abreu G, Tummala R
Arthritis Rheumatol 2021 May;73(5):816-825. Epub 2021 Mar 24 doi: 10.1002/art.41598. PMID: 33225631Free PMC Article
Li T, Jiang H, Liu H, Cooper DKC, Wang Y
Xenotransplantation 2020 Sep;27(5):e12618. Epub 2020 Sep 17 doi: 10.1111/xen.12618. PMID: 32940936

Prognosis

Garnier A, Brochard K, Kwon T, Sellier-Leclerc AL, Lahoche A, Launay EA, Nobili F, Caillez M, Taque S, Harambat J, Michel-Bourdat G, Guigonis V, Fila M, Cloarec S, Djamal-Dine D, de Parscaux L, Allard L, Salomon R, Ulinski T, Frémeaux-Bacchi V, Morin C, Olivier-Abbal P, Colineaux H, Auriol F, Arnaud C, Kieffer I, Brusq C
J Am Soc Nephrol 2023 Sep 1;34(9):1561-1573. Epub 2023 Jun 12 doi: 10.1681/ASN.0000000000000182. PMID: 37303085Free PMC Article
Liu Y, Thaker H, Wang C, Xu Z, Dong M
Toxins (Basel) 2022 Dec 23;15(1) doi: 10.3390/toxins15010010. PMID: 36668830Free PMC Article
Brant Pinheiro SV, de Freitas VB, de Castro GV, Rufino Madeiro BC, de Araújo SA, Silva Ribeiro TF, Simões E Silva AC
Curr Med Chem 2022;29(34):5543-5559. doi: 10.2174/0929867329666220613103316. PMID: 35702785
Manenti L, Rossi GM, Pisani I, Gentile M, Fontana F, Pilato FP, Delsante M, Ricco F, Mignani R, Mele C, Bresin E, Fiaccadori E
J Nephrol 2022 May;35(4):1091-1100. Epub 2021 Nov 10 doi: 10.1007/s40620-021-01189-6. PMID: 34757577
Rizk DV, Maillard N, Julian BA, Knoppova B, Green TJ, Novak J, Wyatt RJ
Front Immunol 2019;10:504. Epub 2019 Mar 19 doi: 10.3389/fimmu.2019.00504. PMID: 30941137Free PMC Article

Clinical prediction guides

Kajikawa T, Mastellos DC, Hasturk H, Kotsakis GA, Yancopoulou D, Lambris JD, Hajishengallis G
Semin Immunol 2022 Jan;59:101608. Epub 2022 Jun 9 doi: 10.1016/j.smim.2022.101608. PMID: 35691883
Röth A, Barcellini W, D'Sa S, Miyakawa Y, Broome CM, Michel M, Kuter DJ, Jilma B, Tvedt THA, Fruebis J, Jiang X, Lin S, Reuter C, Morales-Arias J, Hobbs W, Berentsen S
N Engl J Med 2021 Apr 8;384(14):1323-1334. doi: 10.1056/NEJMoa2027760. PMID: 33826820
Ozen A, Kasap N, Vujkovic-Cvijin I, Apps R, Cheung F, Karakoc-Aydiner E, Akkelle B, Sari S, Tutar E, Ozcay F, Uygun DK, Islek A, Akgun G, Selcuk M, Sezer OB, Zhang Y, Kutluk G, Topal E, Sayar E, Celikel C, Houwen RHJ, Bingol A, Ogulur I, Eltan SB, Snow AL, Lake C, Fantoni G, Alba C, Sellers B, Chauvin SD, Dalgard CL, Harari O, Ni YG, Wang MD, Devalaraja-Narashimha K, Subramanian P, Ergelen R, Artan R, Guner SN, Dalgic B, Tsang J, Belkaid Y, Ertem D, Baris S, Lenardo MJ
Nat Immunol 2021 Feb;22(2):128-139. Epub 2021 Jan 4 doi: 10.1038/s41590-020-00830-z. PMID: 33398182Free PMC Article
Xiao L, Harrison DG
Can J Cardiol 2020 May;36(5):635-647. Epub 2020 Jan 24 doi: 10.1016/j.cjca.2020.01.013. PMID: 32389337Free PMC Article
Dijkstra DJ, Joeloemsingh JV, Bajema IM, Trouw LA
Semin Immunol 2019 Oct;45:101339. Epub 2019 Nov 9 doi: 10.1016/j.smim.2019.101339. PMID: 31718864

Recent systematic reviews

Beard N, Frese M, Smertina E, Mere P, Katelaris C, Mills K
Cochrane Database Syst Rev 2022 Nov 3;11(11):CD013403. doi: 10.1002/14651858.CD013403.pub2. PMID: 36326435Free PMC Article
Rothschild-Rodriguez D, Causer AJ, Brown FF, Collier-Bain HD, Moore S, Murray J, Turner JE, Campbell JP
Exerc Immunol Rev 2022;28:1-35. PMID: 35452398
Nijs J, Nees A, Paul L, De Kooning M, Ickmans K, Meeus M, Van Oosterwijck J
Exerc Immunol Rev 2014;20:94-116. PMID: 24974723

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