Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare, life-threatening disease, characterised by episodes of microangiopathic haemolytic anaemia (MAHA), thrombocytopenia and small vessel thrombosis. We describe a case of cTTP first diagnosed at age 70 years in a female presenting with an acute ischaemic stroke and thrombocytopenia, in whom A Disintegrin And Metalloproteinase with a Thrombospondin type 1 Motif, member 13 (ADAMTS13) levels were <10%, suggestive of thrombotic thrombocytopaenic purpura (TTP). The patient underwent plasma exchange and started rituximab for presumed immune TTP; however, anti-ADAMTS13 antibody titres were negative on two occasions. This, together with a history of pregnancies complicated by presumed disseminated intravascular coagulation, and two previous episodes of sepsis with MAHA, prompted investigation for cTTP, which was confirmed by genetic testing. Despite treatment with infusions of solvent/detergent-treated, virus-inactivated fresh frozen plasma, she has re-presented with further neurological deficit, associated with new infarcts on imaging. cTTP has a varied phenotype which, as demonstrated in this case, can include large vessel occlusion.
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PMID:Congenital thrombotic thrombocytopenic purpura presenting in adulthood with recurrent cerebrovascular events. 3158 56

The incidence of acute kidney injury in pregnancy (P-AKI) has markedly decreased over the last three decades in India, particularly due to decreased incidence of postabortion AKI. However, P-AKI still accounts for 3%-5% of cases of total AKI. Postabortion sepsis has decreased to between 0.9% and 1.5% in 2014 from 9.4% in 1980-1990 in the new millennium. Currently, in India, majority of P-AKI (70%-90%) occurs in the postpartum period and in late 3rd trimester similar to the developed countries, but causes are different. We observed that preeclampsia/eclampsia is the most common cause of P-AKI in the late 3rd trimester and postpartum period followed by puerperal sepsis and postpartum hemorrhage (PPH). Both puerperal sepsis and PPH are treatable and preventable etiologies of P-AKI. Timely and aggressive management of antepartum hemorrhage (APH/PPH) and puerperal sepsis are required to reduce the burden of P-AKI in developing countries. Specific-pregnancy disorders such as P-aHUS/thrombotic thrombocytopenic purpura, pregnancy-associated thrombotic microangiopathy, and acute fatty liver of pregnancy are the uncommon/rare causes of P-AKI in India and possibly also because of the lack of awareness toward diagnosis. Despite decreasing incidence of P-AKI, fetal mortality remained high and unchanged. However, maternal mortality has decreased to 5% from initial high mortality of 20%-25%. The incidence and severity of renal cortical necrosis have significantly decreased at our center.
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PMID:Changing epidemiology of acute kidney injury in pregnancy: A journey of four decades from a developing country. 3169 51

Acute respiratory distress syndrome (ARDS) is a life-threatening noncardiogenic circulatory disorder of the lungs associated with critical illnesses such as sepsis, trauma, and immune and collagen vascular disease. Its mortality rate is marginally improved with the best supportive care. The demise occurs due to progressive pulmonary hypoxia and multi-organ dysfunction syndrome (MODS) with severe inflammation. Complement activation is a part of immune response against pathogen or insult in which membrane attack complex (MAC) is formed and eliminates microbes. If complement regulatory protein such as endothelial CD59 is underexpressed, MAC may also cause pulmonary vascular injury to the innocent bystander endothelial cell of host and provokes endotheliopathy that causes inflammation and pulmonary vascular microthrombosis, leading to ARDS. Its pathogenesis is based on a novel "two-path unifying theory" of hemostasis and "two-activation theory of the endothelium" promoting molecular pathogenesis. Endotheliopathy activates two independent molecular pathways: inflammatory and microthrombotic. The former triggers the release inflammatory cytokines and the latter promotes exocytosis of unusually large von Willebrand factor multimers (ULVWF) and platelet activation. Inflammatory pathway initiates inflammation, but microthrombotic pathway more seriously produces "microthrombi strings" composed of platelet-ULVWF complexes, which become anchored on the injured endothelial cells, and causes disseminated intravascular microthrombosis (DIT). DIT is a hemostatic disease due to lone activation of ULVWF path without activated tissue factor path. It leads to endotheliopathy-associated vascular microthrombotic disease (EA-VMTD), which orchestrates consumptive thrombocytopenia, microangiopathic hemolytic anemia, and MODS. Thrombotic thrombocytopenic purpura (TTP)-like syndrome is the hematologic phenotype of EA-VMTD. ARDS is one of organ phenotypes among MODS associated with TTP-like syndrome. The most effective treatment of ARDS can be achieved by counteracting the activated microthrombotic pathway based on two novel hemostatic theories.
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PMID:Acute Respiratory Distress Syndrome as an Organ Phenotype of Vascular Microthrombotic Disease: Based on Hemostatic Theory and Endothelial Molecular Pathogenesis. 3177 24

Hemorrhagic rashes are observed in a wide variety of conditions, ranging from harmless to life-threatening. This review offers a stepwise approach, which helps limit the possible differential diagnoses based on the clinical manifestations and the clinical picture. The most common and most important conditions, including infectious, coagulation and embolic disorders, vasculitides, and vasculopathies, are briefly reviewed focusing on morphology. Dermatologists often need to distinguish among infectious, reactive, or autoimmune etiologies of the rash and determine if the condition is dangerous or even life-threatening in order to make the right decision. Dermatologic expertise provides vital input in the diagnosis and care of complex interdisciplinary patients, such as those with sepsis, purpura fulminans, and thrombotic thrombocytopenic purpura.
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PMID:The rash that becomes purpuric, petechial, hemorrhagic, or ecchymotic. 3219 46

We report this rare case of fatal fulminant sepsis in a 42-year-old African American female who presented with a three-day history of generalized pain and an evolving rash all over her body. On presentation, the patient was tachycardic, borderline hypotensive, and febrile. Physical examination was significant for diffuse petechiae and ecchymoses over the extremities, torso, and the face, especially confluent over her thighs and lower abdomen. She was admitted to the ICU, and initial investigations revealed a normal leukocyte count and hemoglobin but severe thrombocytopenia, elevated creatinine, blood urea nitrogen (BUN), bilirubin, transaminases, and an elevated INR. She also had a high anion gap metabolic acidosis with elevated lactate. Chest and abdomen CT findings were nonspecific, demonstrating fluid surrounding both kidneys, a moderate amount of fluid in the pelvis, and alveolar opacities at the bases of both lungs. Initial working diagnoses were a septic shock, thrombotic thrombocytopenic purpura (TTP), and vasculitis. She was initiated on broad-spectrum antibiotic coverage with vancomycin, piperacillin/tazobactam, and doxycycline pending culture reports. After a few hours, she became progressively hypothermic, developed disseminated intravascular coagulation (DIC) and hemodynamic instability, and was intubated due to acute hypoxic and hypercapnic respiratory failure. She progressively worsened hemodynamically with multi-organ dysfunction, and ultimately was pronounced dead roughly 18 hours after initial presentation. Blood cultures grew a Gram-negative organism, initially reported as Shewanella putrefaciens, but subsequently confirmed as Capnocytophaga canimorsus.
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PMID:Overwhelming Sepsis due to Capnocytophaga canimorsus in an Immunocompetent Individual: A Rare Case Study. 3302 57

Disseminated intravascular coagulation (DIC) can be correctly redefined as disseminated intravascular microthrombosis based on "two-path unifying theory" of in vivo hemostasis. "DIC" is a form of vascular microthrombotic disease characterized by "microthrombi" composed of platelets and unusually large von Willebrand factor multimers (ULVWF). Microthrombotic disease includes not only "DIC", but also microthrombosis occurring in thrombotic thrombocytopenic purpura (TTP), TTP-like syndrome, and focal, multifocal and localized microthrombosis. Being a hemostatic disease, microthrombotic disease occurs as a result of lone activation of ULVWF path via partial in vivo hemostasis. In endothelial injury associated with critical illnesses such as sepsis, the vascular damage is limited to the endothelial cell and activates ULVWF path. In contrast, in intravascular traumatic injury, the local damage may extend from the endothelial cell to subendothelial tissue and sometimes beyond, and activates both ULVWF and tissue factor (TF) paths. When endotheliopathy triggers exocytosis of ULVWF and recruits platelets, ULVWF path is activated and promotes microthrombogenesis to produce microthrombi composed of microthrombi strings, but when localized vascular damage causes endothelial and subendothelial tissue damage, both ULVWF and TF paths are activated and promote macrothrombogenesis to produce macrothrombus made of complete "blood clots". Currently, "DIC" concept is ascribed to activated TF path leading to fibrin clots. Instead, it should be correctly redefined as microthrombosis caused by activation of ULVWF path, leading to endotheliopathy-associated microthrombosis. The correct term for acute "DIC" is disseminated microthrombosis-associated hepatic coagulopathy, and that for chronic "DIC" is disseminated microthrombosis without hepatic coagulopathy. TTP-like syndrome is hematologic phenotype of endotheliopathy-associated microthrombosis. This correct concept of "DIC" is identified from novel theory of "in vivo hemostasis", which now can solve every mystery associated with "DIC" and other associated thrombotic disorders. Thus, sepsis-associated coagulopathy is not "DIC", but is endotheliopathy-associated vascular microthrombotic disease.
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PMID:Disseminated intravascular coagulation: new identity as endotheliopathy-associated vascular microthrombotic disease based on in vivo hemostasis and endothelial molecular pathogenesis. 3306 57


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