Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0012739 (disseminated intravascular coagulation)
 8,673 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Purpura fulminans is a rare catastrophic disease of childhood that begins as a seemingly benign infectious process and subsequently progresses to high fever, purpuric ecchymosis of the skin, hypotension, disseminated intravascular coagulation, and gangrene. Initial treatment consists of resuscitation with Ringer's lactate, correction of clotting parameters with heparin, avoidance of adrenergic agents, and monitoring of limbs for compartment syndromes. Of 12 patients reported here, three had fasciotomies to limit the ischemic changes, but eventually 24 amputations were performed on the group as a whole. Delayed debridement of up to three weeks was the rule in order to allow ischemic tissue to fully demarcate. Split-thickness skin grafts were used to preserve as much length as possible, particularly where open growth plates were involved. Regardless of how hopeless the situation may initially appear, with prompt recognition and institution of appropriate care the prognosis for a productive life is good.
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PMID:Purpura fulminans. Medical, surgical, and rehabilitative considerations. 397 26

Purpura fulminans presents as a catastrophic illness with gangrene of the distal extremities and necrosis of skin. The clinical picture consists of septicemia, shock, and disseminated intravascular coagulation. The Shwartzman and Arthus reactions are thought to be responsible for the pathogenesis of purpura fulminans. The exact mechanisms of these reactions are not completely understood. Immediate resuscitation is the treatment for shock and sepsis. Heparin is recommended to reverse the disseminated intravascular coagulation component of this disease. Surviving patients require treatment of skin necrosis and digital and extremity gangrene. The former are managed in a fashion similar to the management of burns. Amputation should be delayed until maximal collateral circulation has developed. A series of 10 patients is presented and 58 cases from the literature are analyzed.
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PMID:Purpura fulminans. 646 Dec 69

An unusual case of a 67-year-old man is reported with fulminant pneumococcal sepsis. He had been healthy before, and the identified predisposing factors were only that he was a chronic alcohol drinker and was a HCV carrier. He presented signs of acute renal failure, liver dysfunction, adult respiratory distress syndrome and disseminated intravascular coagulation. Subsequently purpura fulminans (symmetrical peripheral gangrene) with major extremity involvement developed. He finally survived with amputation of both legs, right forearm and two fingers of left hand. Purpura fulminans is a rare catastrophic disease, with initial hemorrhagic skin lesions that progress to gangrene. It usually follows an infectious illness, and although it most commonly occurs in children, it can occur in adults with predisposing factors such as alcoholic, asplenia, AIDS and so on. In adults, pneumococcus and meningococcus are microorganisms that have been reported most frequently as caused agents in Europe and America. But in Japan the previously reported adult case was the only one complicating Xanthomonas maltophilia sepsis, and none accompanying pneumococcal sepsis. Congenital protein C deficiency is recognized to be able to cause purpura fulminans especially in patients with risk factors. In our case, protein C antigen was decreased in the acute stage but gradually increased later toward normal, so this decrease was thought to be concomitant with the initial disseminated intravascular coagulation rather than compatible with protein C deficiency.
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PMID:[Purpura fulminans complicating pneumococcal sepsis: a case report]. 796 3

Purpura fulminans is a rare syndrome of progressive hemorrhagic necrosis of the skin that may present as a dermatologic emergency. It most commonly affects children during the convalescent phase of a streptococcal infection or a viral exanthem. In adults, it may be associated with sepsis or acquired causes. Its pathogenesis has challenged physicians for decades. It has been discovered that purpura fulminans is almost always associated with disseminated intravascular coagulation and can occur in subjects with inherited or acquired deficiencies of the protein C anticoagulant pathway. Patients with liver compromise may also be potential candidates for coagulopathies secondary to hepatic dysfunction and impaired protein synthesis. It is widely recognized that individuals who consume alcohol on a long-term basis may develop severe hepatotoxicity from ingestion of therapeutic doses of acetaminophen (500 to 1000 mg every 4 to 6 hours). We have observed a patient with chronic alcoholism in whom hepatotoxicity and purpura fulminans developed secondary to the ingestion of acetaminophen.
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PMID:Acquired purpura fulminans induced by alcohol and acetaminophen. Successful treatment with heparin and vitamin K. 821 90

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.
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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.
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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 classically defined by ecchymotic skin lesions, fever, and hypotension. The majority of cases occur in association with bacterial sepsis, and disseminated intravascular coagulation (DIC) is usually present. Prompted by our experience with a patient with pneumococcal sepsis and purpura fulminans in whom hypotension was never observed, we evaluated the important parameters of sepsis in reports of this syndrome. 42 additional cases of pneumococcal bacteremia and purpura fulminans were identified. Hypotension was present in only 51%. Although DIC was present in 85% of patients, hypofibrinogenemia was documented in only 26%. By contrast, both hypotension and hypofibrinogenemia are present in the vast majority of patients described with purpura fulminans in association with meningococcal sepsis. These data confirm that hypotension is not a necessary feature of the syndrome of purpura fulminans associated with pneumococcal sepsis and suggest further that qualitative or quantitative differences exist in the DIC cascade of pneumococcal vs meningococcal sepsis.
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PMID:Purpura fulminans in pneumococcal sepsis: case report and review. 943 36

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.
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PMID:[Purpura fulminans in the newborn. Report of two cases successfully treated with heparin and antithrombin III]. 957 59

Purpura fulminans is a devastating disorder characterized by rapidly progressing hemorrhagic necrosis of the skin, vascular collapse, and disseminated intravascular coagulation. It is most often seen in children, and it is usually preceded by meningococcemia or another infection. Most often, the disorder results in severe skin loss, but it can also result in the need for extremity amputations. In extreme cases, wound coverage after excision may be problematic because of the limited existence of donor sites and the need for amputation revisions. The case of a 21/2-year-old male requiring amputations of all four extremities due to severe purpura fulminans is presented to illustrate the use of Integra Artificial Skin (Integra Lifesciences Corp., Plainsboro, NJ) to obtain immediate wound closure. Integra Artificial Skin is a bilayered skin substitute that engrafts to a viable wound bed. In the case presented here, where the viability of the underlying tissue of the amputated stumps was questionable, the artificial skin acted as an indicator of that viability. It engrafted well onto the upper extremity stumps, which were of excellent viability, but it needed to be replaced on the lower extremity stumps, which required further debridement and amputation revisions. The use of artificial skin spared the patient the immediate use of his limited and valuable autograft sites. In conclusion, Integra Artificial Skin can be a useful adjunct in the treatment of severe purpura fulminans that includes skin and extremity necrosis.
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PMID:Integra Artificial Skin as a useful adjunct in the treatment of purpura fulminans. 971 Jul 31

Purpura fulminans (PF) is a haemorrhagic condition usually associated with sepsis or previous infection. Features include tissue necrosis, small vessel thrombosis and disseminated intravascular coagulation. Gram-negative organisms are the commonest cause of the acute infectious type, which is often associated with multi-organ failure. An idiopathic variety, however, is often confined to the skin. The mortality rate has decreased with better treatment of secondary infections, supportive care and new treatments, but it remains a disabling condition often requiring major amputations. We describe two cases and review the various treatments for this condition.
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PMID:Review of management of purpura fulminans and two case reports. 1157 39


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