Protein C Deficiency

An absence or deficiency in PROTEIN C which leads to impaired regulation of blood coagulation. It is associated with an increased risk of severe or premature thrombosis. (Stedman's Med. Dict., 26th ed.)

Also Known As:

Networked: 611 relevant articles (13 outcomes, 24 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Thrombosis (Thrombus)
2.	Sepsis (Septicemia)
3.	Necrosis
4.	Pulmonary Embolism
5.	Nephrotic Syndrome (Syndrome, Nephrotic)

Experts

1.	Bovill, Edwin G: 5 articles (12/2014 - 10/2004)
2.	Nowak-Göttl, Ulrike: 5 articles (02/2014 - 09/2002)
3.	Rosendaal, F R: 5 articles (01/2010 - 01/2000)
4.	Kurnik, Karin: 4 articles (02/2014 - 11/2002)
5.	Klai, Sarra: 4 articles (04/2013 - 01/2011)
6.	Fekih-Mrissa, Najiba: 4 articles (04/2013 - 01/2011)
7.	Bruley, Duane F: 4 articles (01/2011 - 01/2005)
8.	van der Meer, Jan: 4 articles (10/2008 - 08/2002)
9.	Vossen, Carla Y: 3 articles (12/2014 - 10/2004)
10.	Saxena, Renu: 3 articles (06/2013 - 06/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Protein C Deficiency:

1.	Protein CIBA 04/01/1994 - "Identification of the spatial relationships of natural mutations in the protein C model is helpful for understanding manifestations of protein C deficiency and for identification of novel, functionally important molecular features and exosites." 08/01/2009 - "pneum and severe protein C deficiency at baseline, a significantly greater proportion of patients were no longer severely protein C deficient after 4 days of DrotAA therapy. " 02/01/2014 - "Subcutaneous protein C concentrate on its own or combined with an oral anticoagulant appears to be safe and effective as maintenance treatment of severe protein C deficiency. " 04/15/1999 - "In the present study, we screened for protein C amidolytic activity in patients admitted to the National Cardiovascular Center Hospital, and we identified among them 43 probands and 51 relatives with heterozygous protein C deficiency. " 05/01/1994 - "The familial study revealed that her sister and mother had both a decreased concentration and depressed activity of protein C, indicating that this is a case of congenital protein C deficiency. "
2.	Warfarin (Coumadin)FDA LinkGeneric 11/01/2008 - "Diagnosis of heterozygous hereditary protein C deficiency was made, and the patient was placed on warfarin. " 12/01/2003 - "Because warfarin anticoagulation induces acquired protein C deficiency, thereby exacerbating the prothrombotic state of type II HIT, warfarin should be withheld until platelet counts increase to more than 100,000/microL and type II HIT is clearly resolving. " 06/01/1990 - "Progressive warfarin anticoagulation in protein C deficiency: a therapeutic strategy." 01/01/1989 - "Homozygous protein C deficiency: early treatment with warfarin." 02/01/1988 - "Homozygous protein C deficiency: observations on the nature of the molecular abnormality and the effectiveness of warfarin therapy."
3.	AntigensIBA 03/01/2007 - "Protein C deficiency (antigen 59%, activity 49%) was also present, suggesting a congenital type I heterozygous deficiency. " 11/01/2002 - "Protein C deficiency (1% to 3% of control) was noted by functional assay; chromogenic assay and antigen levels were 30% of control. " 09/01/1996 - "Two showed a decreased level of function with a normal antigen concentration consistent with type II protein C deficiency and the remaining 26 showed type I deficiency with decreases in both function and antigen level. " 08/02/1993 - "The case of an adult patient with moderately severe protein C deficiency (antigen 16%, activity 12%) is reported. " 07/01/1993 - "We describe five families presenting with type II hereditary protein C deficiency characterized by normal antigen and amidolytic activity levels but low anticoagulant activity. "
4.	AnticoagulantsIBA 01/01/1996 - "Protein C deficiency is probably the most extensively studied abnormality in natural anticoagulants. " 05/01/1992 - "The combined effects of protein C deficiency and lupus anticoagulants, exacerbated by other potentially thrombogenic conditions, are believed responsible for the severe thrombotic events experienced by this patient." 05/01/1992 - "Concurrent protein C deficiency and lupus anticoagulants." 06/01/1991 - "Laboratory diagnosis of protein C deficiency is complicated by the fact that many patients referred for investigation are already being treated with oral anticoagulants. " 01/01/1990 - "We report the case of a 57-year old woman whose severe protein C deficiency was revealed soon after oral anticoagulants were introduced into her treatment. "
5.	Factor VII (Proconvertin)IBA 04/01/2000 - "Combined factor VII/protein C deficiency results in intrauterine coagulopathy in mice." 07/01/1992 - "Congenital protein C deficiency and myocardial infarction:concomitant factor VII hyperactivity may play a role in the onset of arterial thrombosis." 07/15/1988 - "Combined factor VII and protein C deficiency found in a patient with peripheral pulmonary artery stenosis accompanied by progressive pulmonary hypertension and hemoptysis." 06/01/1991 - "Detection of protein C deficiency during oral anticoagulant therapy--use of the protein C:factor VII ratio." 07/15/1988 - "Since the genes encoding factor VII and protein C are located in different chromosomes, the 13th and the second chromosomes, respectively, expression of the combined hereditary deficiency is a random and very rare association on the basis of frequencies of 1:50,000 for factor VII and 1:16,000 for protein C deficiencies."
6.	Low-Molecular-Weight Heparin (Heparin, Low Molecular Weight)IBA 12/01/2008 - "This young woman had a personal history of protein C deficiency and was treated daily during her pregnancy with low-molecular-weight heparin. " 04/01/1998 - "Homozygous protein C deficiency: description of a new mutation and successful treatment with low molecular weight heparin." 08/01/2003 - "A patient with myeloid/natural killer (NK) cell precursor acute leukemia who was also homozygous for protein C deficiency was treated and showed a complete remission while he simultaneously received low molecular weight heparin. " 07/01/1992 - "Low molecular weight heparin was useful for disseminated intravascular coagulation (DIC) that complicated protein C deficiency in this patient." 10/01/2002 - "We treated three thrombophilia-complicated pregnant women (two antiphospholipid antibody syndrome, one protein C deficiency) with low molecular weight heparin (dalteparin). "
7.	Danazol (Azol)FDA LinkGeneric 05/01/1989 - "The observation is discussed with the evidences of danazol being useful in the treatment of patients with protein C deficiency." 02/01/1988 - "Protein C deficiency resulting from possible double heterozygosity and its response to danazol." 06/24/1985 - "Administration of danazol (600 mg/day) in two patients with protein C deficiency elevated this protein and discontinuation of the drug resulted in a reduction of protein C to pretreatment values. " 06/24/1985 - "Protein C deficiency--response to danazol and DDAVP." 05/01/1992 - "Danazol, a synthetic testosterone derivative, is used in the treatment of endometriosis, fibrocystic breast disease, menorrhagia protein C deficiency, and hemophilia. "
8.	Hydroxychloroquine (Plaquenil)FDA LinkGeneric 05/01/2013 - "Is treatment with hydroxychloroquine effective in surfactant protein C deficiency?" 05/01/2013 - "There is merely news from a few previous descriptions in the literature about the use of hydroxychloroquine for surfactant protein C deficiency with satisfactory results. " 05/01/2013 - "We present the case of two twin brothers with surfactant protein C deficiency who were treated with hydroxychloroquine for three years, with apparent success. " 01/13/2005 - "Hydroxychloroquine and surfactant protein C deficiency."
9.	factor V LeidenIBA 01/01/2011 - "The factor V Leiden mutation, non-O blood group and protein C deficiency had the highest incidences among patients both as a whole and in the 4 subgroups. " 07/01/2007 - "The protein C deficiency and factor V leiden was undetermined. " 06/01/2007 - "We report the case of a severely sick male preterm infant with a heterozygous factor V Leiden mutation and protein C deficiency. " 05/01/2006 - "Others included factor V Leiden mutation, found in 10 (6.7 per cent) and protein C deficiency, found in six (4.0 per cent). " 05/01/2004 - "Other prothrombogenic factors were negative in all but 2 patients (1 protein C deficiency, 1 factor V Leiden mutation). "
10.	Prothrombin (Factor II)IBA 04/01/2013 - "There was no significant difference between the two groups in prothrombin G20210A mutation (p=0.490) and protein C deficiency (p=0.609). " 01/01/2011 - "He has heterozygous Protein C deficiency (50%) and heterozygosity for the prothrombin gene G20210A mutation. " 10/01/2010 - "Possible exceptions are the prothrombin G20210A mutation in adults and protein C deficiency in children. " 01/01/2006 - "The prevalence of protein C deficiency was 9.5%, whereas those of factor II, V, VII, IX, and X deficiencies were 0, 66.7, 9.5, 0, and 57.1%, respectively. " 10/01/2001 - "Coagulation tests allowed us to identify one case of probable heterozygosis of Protein C deficiency and one case of G20210A prothrombotic prothrombin mutation. "

Therapies and Procedures

1.	Cardiopulmonary Bypass (Heart-Lung Bypass) 11/01/2007 - "Recent studies have examined cardiopulmonary bypass and inherited Protein C deficiency. " 11/01/2007 - "The objective of this article is to review the literature in order to discuss the occurrence, the aetiology and possible management of cancer patients with malignancy-induced Protein C deficiencies that require cardiopulmonary bypass for their surgery." 11/01/2007 - "However, surgery for cancer patients with a malignancy-induced Protein C deficiency involving cardiopulmonary bypass has not been reported. " 01/01/2007 - "Postoperative antithrombin and protein C deficiencies are not uncommon in adults undergoing cardiac surgery with cardiopulmonary bypass, but detection of these deficits did not identify patients at increased risk of systemic inflammatory response syndrome." 03/01/2001 - "The cardiac output also increased, and New York Heart Association functional class improved to I. Pulmonary thromboendarterectomy under cardiopulmonary bypass was effective for chronic pulmonary thromboembolism accompanied by protein C deficiency."
2.	Endarterectomy (Thromboendarterectomy) 01/01/2014 - "A case report of pulmonary thromboendarterectomy for chronic thromboembolism in a patient with protein C deficiency." 02/01/2006 - "Pulmonary thromboendarterectomy for chronic pulmonary thromboembolism in protein C deficiency." 03/01/2001 - "We have performed pulmonary thromboendarterectomy on a patient with chronic pulmonary thromboembolism accompanied by protein C deficiency. " 03/01/2001 - "The cardiac output also increased, and New York Heart Association functional class improved to I. Pulmonary thromboendarterectomy under cardiopulmonary bypass was effective for chronic pulmonary thromboembolism accompanied by protein C deficiency."
3.	Contraception (Birth Control) 08/05/1994 - "[Oral contraception in protein C deficiency]." 10/01/1992 - "This study investigates the association between protein C deficiency and pregnancy loss, thrombosis in pregnancy, and thrombosis with oral contraception. " 02/01/2001 - "The case-record describes a 23-year-old female patient hospitalized on account of phlebothrombosis of the right upper extremity, associated with protein C deficiency and the use of hormonal contraception. " 10/01/1992 - "Protein C deficiency increases the risk of thrombosis during pregnancy and with oral contraception. "
4.	Liver Transplantation 05/01/2015 - "Successful living domino liver transplantation in a child with protein C deficiency." 03/01/2009 - "As described here, the liver transplantation offers an alternative curative treatment for children with homozygous protein C deficiency." 03/01/2009 - "Long-term survival of a child with homozygous protein C deficiency successfully treated with living donor liver transplantation." 01/01/1995 - "We report the first case, to our knowledge, of the transmission of heterozygous protein C deficiency, an autosomal recessive genetic defect, associated with dysfibrinogenemia, an autosomal dominant trait, by liver transplantation. " 01/01/1995 - "Heterozygous protein C deficiency and dysfibrinogenemia acquired by liver transplantation."
5.	Transplants (Transplant) 01/01/2001 - "En bloc heterotopic auxiliary liver and bilateral renal transplant in a patient with homozygous protein C deficiency." 11/29/1997 - "Severe acquired protein-C deficiency in meningococcaemia is usually associated with substantial mortality: in survivors, skin grafts, amputation, and end-organ failure are not uncommon. " 10/01/1991 - "We speculate that the protein C deficiency and and resultant hypercoagulable state led to the stroke, and the deficiency of this anticoagulant was a sequela of the transplant." 03/01/2006 - "Cardiopulmonary bypass for a coronary artery bypass graft patient with heterozygous protein C deficiency and protein S deficiency." 09/01/2001 - "A leg ulcer due to protein C deficiency: successful treatment with split thickness skin graft after protein C supplementation."