Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.21.69 (APC)
 16,337 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Purpura fulminans must be treated as an emergency in internal medicine and dermatology. Its characteristic features are the sudden development of progressively enlarging haemorrhagic skin necrosis, severe disseminated intravascular coagulation with consumption of anticoagulant factors, and signs of shock. Purpura fulminans can be classified into a neonatal form with inherited protein C deficiency and an acquired type for which multiple causes are known. Clinically it is characterized by massive ecchymosis with haemorrhagic blebs and acral necrosis. Histologically the lesions show widespread extravasation of erythrocytes and thrombosis of small vessels. Thrombocytopenia, decrease of coagulation factors, the presence of fibrinogen split products and fragmented erythrocytes in the blood smear help to confirm the diagnosis. The therapy includes fresh-frozen plasma, heparin, antibiotics and surgical debridement of necrotic areas. It is important to recognize the disease promptly because the mortality rate is about 30-40% and only quick intervention helps to save the life of the patient.
 ...
PMID:[Purpura fulminans with extensive skin necroses]. 892 72

Purpura fulminans is associated with homozygous protein C and homozygous protein S deficiency or may follow bacterial or viral infections. We present 2 children from 2 unrelated Arab families with purpura fulminans who were double heterozygotes for factor V Leiden inherited from their fathers and protein S deficiency inherited from their mothers. No previous thrombotic events have occurred in either patient or their respective family members. In one patient sepsis accompanied by disseminated intravascular coagulation appeared to be the trigger of purpura fulminans. In the other patient varicella infection preceded purpura fulminans and was also associated with disseminated intravascular coagulation. This report emphasizes the need for evaluation of hereditary defects in the inhibitory mechanisms of blood coagulation in patients with purpura fulminans at any age.
 ...
PMID:Purpura fulminans induced by disseminated intravascular coagulation following infection in 2 unrelated children with double heterozygosity for factor V Leiden and protein S deficiency. 924 37

Purpura fulminans is a rare form of disseminated intravascular coagulation characterized by rapidly progressive purpuric lesions, hypotension and, in some cases, fever. In neonates, purpura fulminans usually develops following deficiency of anticoagulant protein C or S, although acquired forms have been described. The management of disseminated intravascular coagulation is still controversial, with some studies finding a positive effect of anticoagulants and others showing no effect or even a detrimental one. Therefore, at present, management is limited to the treatment of underlying disease and replacement of clotting factors. Personal experience is reported on the efficacy of heparin in combination with antithrombin III in the treatment of purpura fulminans in two preterm neonates who did not have inherited deficiency of protein C or S and developed the disease possibly following prolonged labor (36 hours) in the first case, and maternal neoplasia, in the second. Both neonates presented with widespread cyanotic areas rapidly evolving in purpuric lesions and hemorrhagic bullae. Laboratory tests (prolonged prothrombin and partial thromboplastin time, fibrinogen and antithrombin III concentrations below normal ranges, d-dimer highly positive) were consistent with disseminated intravascular coagulation. In both cases anticoagulant treatment with heparin (50 UI/kg in bolus followed by 15 UI/kg/h) and antithrombin III was associated with resolution of disseminated intravascular coagulation and prompt amelioration of the purpuric lesions, without apparent side effects.
 ...
PMID:[Purpura fulminans in the newborn. Report of two cases successfully treated with heparin and antithrombin III]. 957 59

Purpura fulminans is a rare but dramatic disease which occurs most commonly during or after an infection. It is characterized by extensive involvement of the skin and extremities and involvement of visceral organs. Purpura fulminans, when occurring after a viral infection such as varicella, is usually characterized by purpuric lesions involving the trunk, usually with sparing of the visceral organs. In this report we describe a child with purpura fulminans due to a transient protein C deficiency as a complication of chickenpox infection. A seven-year-old girl developed bruise-like lesions on her extremities on the fifth day after eruption of varicella exanthem. She had no previous history of bleeding tendency or thrombosis. Family history was also negative. On the seventh day of her illness she was admitted to Marmara University Hospital with widespread echymotic an partially crusted chickenpox lesions. CBC, urinalysis and blood chemistries were within normal limits. She had a prolonged aPT and apt with low serum fibrinogen and high D-dimers suggestive disseminated intravascular coagulation (DIC). Protein C activity was low. Punch skin biopsy was consistent with purpura fulminans. She was treated with heparin and fresh frozen plasma which helped her to recover clinically as well as hematologically. She was discharged with still low protein C activity that returned to normal by the next follow-up visit.
 ...
PMID:A case of purpura fulminans secondary to transient protein C deficiency as a complication of chickenpox infection. 1271 82

Disseminated intravascular coagulation (DIC) is a common phenomenon in patients with sepsis, but the clinical implications of this condition are not clear. Clinical trials with coagulation inhibitors have failed to show a significant benefit concerning survival. DIC is primarily a laboratory diagnosis, based on the combination of elevated fibrin-related markers (FRM), with decreased procoagulant factors and platelets. Non-overt DIC is observed in most patients with sepsis, whereas overt DIC is less frequent. Patients with overt DIC may display consumption coagulopathy and purpura fulminans. Consumption coagulopathy is a bleeding disorder caused by low levels of platelets and procoagulant factors associated with massive coagulation activation. Purpura fulminans is caused by widespread microvascular thrombosis, resulting in tissue necrosis. Treatment with drotrecogin alfa (activated) improves survival and other outcome parameters in severe sepsis, including a subgroup of patients fulfilling the laboratory criteria of overt DIC. No randomized trials demonstrating effective therapies in consumption coagulopathy have been published. Bleeding patients with consumption coagulopathy are most frequently treated with platelet transfusions and various plasma products including fresh frozen plasma and coagulation factor concentrates. Based on case reports, treatment with drotrecogin alfa (activated) or substitution of protein C have been recommended for adjuvant treatment of sepsis-related purpura fulminans.
 ...
PMID:Coagulopathy of sepsis. 1496 Nov 46

Purpura fulminans is a thrombotic disease that can occur during infections, disseminated intravascular coagulation or in the context of an acquired or congenital protein C or S deficiency. Here we report the case of a 4-year-old child who developed, 5 days after a chickenpox infection, large painful ecchymotic, necrotizing and retiform plaques on the lower extremities. Laboratory analyses revealed very low protein S levels as well as anticardiolipin antibodies. Aggressive treatment by low-molecular-weight heparin, steroids, intravenous immunoglobulins and fresh frozen plasma was able to prevent the extension of the lesions and to correct the coagulation abnormalities. No lesions required skin grafting. As in our patient, an acquired protein S deficiency is probably responsible for most cases of purpura fulminans occurring after varicella, but the concomitant presence of antiphospholipid antibodies may also play a role.
 ...
PMID:Purpura fulminans in a child as a complication of chickenpox infection. 1511 84

Necrotizing fasciitis and purpura fulminans are two destructive infections that involve both skin and soft tissue. Necrotizing fasciitis is characterized by widespread necrosis of subcutaneous tissue and the fascia. Historically, group A beta-hemolytic streptococcus has been identified as a major cause of this infection. However, this monomicrobial infection is usually associated with some underlying cause, such as diabetes mellitus. During the last two decades, scientists have found that the pathogenesis of necrotizing fasciitis is polymicrobial. The diagnosis of necrotizing fasciitis must be made as soon as possible by examining the skin inflammatory changes. Magnetic resonance imaging is strongly recommended to detect the presence of air within the tissues. Percutaneous aspiration of the soft tissue infection followed by prompt Gram staining should be conducted with the "finger-test" and rapid-frozen section biopsy examination. Intravenous antibiotic therapy is one of the cornerstones of managing this life-threatening skin infection. Surgery is the primary treatment for necrotizing fasciitis, with early surgical fasciotomy and debridement. Following debridement, skin coverage by either Integra Dermal Regeneration Template or AlloDerm should be undertaken. Hyperbaric oxygen therapy complemented by intravenous polyspecific immunoglobulin are useful adjunctive therapies. Purpura fulminans is a rare syndrome of intravascular thrombosis and hemorrhagic infarction of the skin; it is rapidly progressive and accompanied by vascular collapse. There are three types of purpura fulminans: neonatal purpura fulminans, idiopathic or chronic purpura fulminans, and acute infectious purpura fulminans. Clinical presentation of purpura fulminans involves a premonitory illness followed by the rapid development of a septic syndrome with fever, shock, and disseminated intravascular coagulation. The diagnosis and treatment of these conditions is best accomplished in a regional burn center in which management of multiple organ failure can be conducted with aggressive debridement and fasciotomy of the necrotic skin. The newest revolutionary advancement in the treatment of neonatal purpura fulminans is the use of activated protein C.
 ...
PMID:Massive soft tissue infections: necrotizing fasciitis and purpura fulminans. 1571 17

Purpura fulminans (PF) is a severe disorder of acute onset with high morbidity and mortality. In children, this rapidly progressive illness is usually associated with severe bacterial or viral infections. However, some other conditions may participate in the development of PF. Our objective was to investigate the underlying and associated disorders and the outcomes of the disease in 16 children, 7 males and 9 females ranging in age from 3.5 months to 12 years (median age, 2 years). Thirteen of the 16 children (81%) were 4 years of age or younger. The remaining 3 patients were 9, 10, and 12 years of age. Among these 13 infants and small children, 7 (43%) had infection, 2 infants had congenital cardiac disorders necessitating minor or major surgical intervention, and 1 infant and 3 children had different miscellaneous disorders. The factor V G1691A mutation was present in six of the 13 small children (46%). None of the 3 older children carried the mutation. Six (37.5%) of the 16 patients had protein C deficiencies, and 9 (56%) had protein S deficiencies. These deficiencies, except one for protein S, were acquired. Ten patients except two who were diagnosed at this center were treated with fresh frozen plasma. They were also given heparin. Nine (69%) of the 13 children 4 years of age or younger and one of the older children (33%) required amputation. Five of the six patients (83%) who had factor V G1691A mutation, and who also exhibited severe infection, required amputation. This study suggests that an age of 4 years or less is a risk factor for the development of PF during severe infections, especially in the presence of factor V G1691A mutation and congenital heart disease, necessitating major or minor surgical interventions. This study also shows that the amputation rate in 10 patients, after excluding the patients who had been referred to our center after development of sequelae, was 60%. The survival rate among these 10 patients may indicate that, with the treatment protocol, PF need not be regarded as a lethal disease any more. It is also suggested that effective immunization programs and better health care have probably resulted in some changes in the etiological profile of PF.
 ...
PMID:Outcome in children with purpura fulminans: report on 16 patients. 1613 55

Purpura fulminans is a serious disease associated with high rates of morbidity and mortality. It usually leads to disseminated intravascular coagulation and septic shock related to reduced levels of protein C. Recombinant protein C (rPC) activator has been used successfully to inhibit this process. Intracranial hemorrhages are the most important, life-threatening adverse effects of treatment with rPC activator. We report 3 cases of patients with meningococcal purpura fulminans who developed septic shock and multiorgan dysfunction. They were treated with the protocol for septic shock, antibiotics and rPC activator from the time of admission, and improvement in hemodynamic dysfunction was observed within hours in all patients. All received platelet replacement transfusions. Subarachnoid bleeding complications occurred in 2 patients. One patient died 5 days after admission and 2 were discharged from the intensive recovery care unit 28 days after admission.
 ...
PMID:[Meningococcal purpura fulminans: treatment with recombinant protein C activator in 3 cases]. 1799

Purpura fulminans (PF) is a protein C deficiency disease process with a high case fatality rate; however, overall incidence of the disease remains relatively very low. The similarity between skin necrosis secondary to PF and full-thickness skin burns provides the rationale for treating PF case in a burn center. In this case series we reviewed our experiences in managing PF and their associated favorable outcomes. Retrospective chart review of five PF cases managed between September 2004 and August 2006 at our Burn Center with 100% survival. Management of cases following the standard care of the Burn Center for a full-thickness burn included antibiotics, fluid resuscitation, surgical debridement with skin grafting, and activated protein C (Drotrecogin alfa) replacement. Two patients required amputations of extremities and all had surgical debridement. One required hemodialysis and two needed both hemodialysis and positive-pressure mechanical ventilator. No patient experienced any bleeding complications during or after surgery while receiving activated protein C. Early diagnosis and treatment at a burn center may reduce mortality and morbidity and loss of extremities in PF cases.
 ...
PMID:Purpura fulminans: a case series managed at a regional burn center. 1835 5


<< Previous 1 2 3 4 Next >>