Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Purpura fulminans is a rare disease characterized by purpura ecchymosis, hypotension, and fever associated with disseminated intravascular coagulation. It often begins as a benign infectious process and subsequently progresses to a severe, catastrophic outcome. It is recognized to originate from congenital or acquired protein C deficiency. We present an unusual case of an adult with Xanthomonas maltophilia sepsis that subsequently developed into purpura fulminans with involvement of the four extremities. We discuss the importance of the protein C system in coagulation homeostasis and its relationship to purpura fulminans.
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PMID:Purpura fulminans secondary to Xanthomonas maltophilia sepsis in an adult with aplastic anemia. 191 97

Purpura fulminans (PF) is a cutaneous manifestation of a dramatic and deadly syndrome of systemic disseminated intravascular coagulation (DIC). It is characterized by microvascular thrombosis in the dermis followed by perivascular haemorrhage. Since two other related syndromes involve the protein C (PC) system, we undertook a serial study to investigate the levels of PC and protein S (PS) in two patients with acquired PF. Laboratory findings were consistent with DIC, and both patients were treated with blood replacement and heparin therapy. The levels of PC activity were very low during the initial 24-36 h after onset and gradually increased until returning to normal levels. The total and 'free' PS were also abnormal during the initial onset of PF. The total and free PS increased to normal after 4-6 d. Although the pathogenesis is not fully understood, the infection and sepsis appears to consume PC and PS selectively during the PF and DIC phase. Acquired PF appears to selectively involve the PC system in a similar fashion to two other syndromes of PF-like lesions.
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PMID:Protein C and protein S levels in two patients with acquired purpura fulminans. 214 90

Purpura fulminans is a rare disease that typically begins as a benign infectious process and subsequently progresses to severe sepsis, hypotension, purpura ecchymosis, and disseminated intravascular coagulation. We present an unusual case of an adult who was seen initially with pneumococcal sepsis that subsequently developed into purpura fulminans with major extremity involvement. A multidisciplinary approach is needed in the treatment of this often catastrophic disease.
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PMID:Purpura fulminans. 229 60

Purpura fulminans is an uncommon catastrophic syndrome that occurs in children, typically one to four weeks after a seemingly benign infectious process. The child usually presents with a high fever, purpuric ecchymosis, hypotension, disseminated intravascular coagulation, and gangrene of the extremities. We have recently treated six children, whose mean age was 22 months; three were male and three were female. Five of the six had a change of mental status upon initial examination. Their mean temperature was 104 degrees F. All six children had purpuric involvement of their extremities; three had involvement of their hands, two had involvement of their faces, and two had involvement of their trunks. All had absent palpable pulses and sluggish capillary refill in the involved hands and feet. Two patients died shortly after admission as a result of severe end-stage sepsis. The platelet counts in these two patients, and the white blood cell counts were markedly depressed. The mean platelet count of the survivors was 370,000 and the mean white blood cell count was 25,000. Lumbar punctures were positive for bacterial meningitis in five patients and viral meningitis in one patient. All patients were treated with intravenous heparin. Of the four survivors, two lost significant tissue and required multiple plastic reconstructive procedures, and two improved on heparin alone with no tissue loss. In addition to systemic support and intravenous antibiotics, the mainstay of treatment is one of immediate heparinization and a continuous heparin drip. Heparin prevents subsequent small vessel thrombosis and limits tissue loss due to ongoing purpura. Conservative management of the purpuric lesions is the treatment of choice until final demarcation occurs.
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PMID:The surgical implications of purpura fulminans. 234 Feb 49

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 (PF), which describes the necrosis of soft tissue secondary to diffuse microvascular thrombosis induced by transient protein C deficiency associated with meningococcal sepsis, is unusual despite the approximately 15000 cases of bacterial meningitis which occur annually in the USA. PF has a reported mortality of 50 per cent secondary to multiple organ failure which commonly accompanies the syndrome and is associated with major long-term morbidity in those who survive. Children who develop multiple organ failure in association with purpura fulminans are difficult management problems and benefit from the unique surgical and critical care resources available in burn centres. We describe our recent experience with three such patients and suggest a management strategy, the key components of which include early excision and closure of deep wounds, aggressive critical care management and long-term follow-up should delayed epiphyseal growth occur.
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PMID:Management strategy in purpura fulminans with multiple organ failure in children. 871 18

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


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