Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0022116 (ischemia)
 91,303 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neutropenic enterocolitis or typhlitis (from the Greek word typhlon, meaning cecum) is a clinical syndrome that occurs in the setting of disease or chemotherapy-induced neutropenia. The disease is characterized by an inflammatory process involving colon and/or small bowel, and it can result in ischemia, necrosis, bacteremia, hemorrhage, and perforation. The classic clinical features include fever and abdominal pain. The diagnosis is supported by the findings of bowel wall thickening on ultrasonography or CT imaging. The management of neutropenic enterocolitis is controversial. Neither prospective nor high-quality retrospective studies concerning medical or surgical therapies are available. Most authors will recommend initial conservative management with bowel rest, intravenous fluids, total parenteral nutrition, broad-spectrum antibiotics and normalization of neutrophil counts. Surgical intervention is recommended in the setting of obstruction, perforation, persistent gastrointestinal bleeding despite correction of thrombocytopenia and coagulopathy, and clinical deterioration.
 ...
PMID:Neutropenic enterocolitis: current issues in diagnosis and management. 1732 48

Thrombotic thrombocytopenic purpura (TTP) is a disorder of blood coagulation that presents classically with the pentad of fever, thrombocytopenia, microangiopathic hemolytic anemia, renal dysfunction and mental status changes. However, the clinical presentation can be quite variable making the diagnosis difficult in many cases. "Hyaline" microthrombi composed primarily of platelets and Von Willebrand Factor (VWF) are found in the small vessels of affected organs and represent the pathological hallmark of the disease. The accompanying tissue ischemia is thought to explain the clinical TTP signs and symptoms. Pathogenesis of TTP has been linked to dysfunction of ADAMTS13, a metalloprotease whose only known substrate is VWF. Interestingly, further investigation into the natural history of TTP has demonstrated that ADAMTS13 deficiency likely is necessary, but not sufficient for the development of this disease, suggesting that additional genetic and/or environmental factors are required for TTP pathogenesis. Recently, a mouse model of TTP was established that recapitulates many of the key clinical features of this disease, including the requirement for further genetic and environmental factors in addition to ADAMTS13 deficiency. Therefore, in addition to being useful for the direct study of disease pathophysiology in vivo, this mouse model may also play a key role in elucidating some of the important environmental and genetic contributors to disease pathogenesis. Here we will review TTP in humans, and then discuss recent information gained from the analysis of ADAMTS13-deficient mice.
 ...
PMID:Thrombotic thrombocytopenic purpura in humans and mice. 1752 62

After kidney transplantation thrombotic microangiopathy (TMA) may recur in patients with previous hemolytic uremic syndrome or may develop de novo. De novo TMA has been reported to occur in less than 1% of renal transplant recipients by large registries, but single center series reported an incidence of the disease as high as 14-20%. A number of factors may predispose to posttransplant TMA, including ischemia-reperfusion injury, acute rejection, viral infection. Immunosuppressive treatment can also contribute to the development of de novo TMA. Calcineurin inhibitors may cause or aggravate endothelial lesions through their pronecrotic, vasoactive and profibrotic activity. Anti-mTOR agents may delay the repair of the endothelial damage through their interference with endothelial growth factor. Usually, TMA develops in the early posttransplant period but may also occur later. Clinically, TMA is characterized by progressive renal failure and hypertension. Microangiopathic hemolytic anemia and thrombocytopenia may occur in about 60% of cases. Histologically, TMA may be localized to glomeruli or may involve arteries or both. The prognosis depends on the timely diagnosis and on histological picture. Treatment is based on the removal of inciting factors. Early plasmapheresis could improve clinical signs and symptoms and rescue renal function in a number of patients. Anecdotal successes have also been reported with intravenous immunoglobulins and rituximab.
 ...
PMID:De novo thrombotic microangiopathy. An underrated complication of renal transplantation. 1759 67

Preeclampsia, one of the main complications in pregnancy, affects 5-7% of all pregnancies, and is a leading cause of maternal and perinatal mortality. The placenta plays a pivotal role in the etiology of preeclampsia, and particularly, the trophoblast cells of the placenta. It is now believed that preeclampsia is a two stage disease. In the first stage, a defective implantation and placentation, causes a reduction in uteroplacental perfusion and placental ischemia/hypoxia. Placental ischemia may promote the release of a variety of factors to the maternal circulation. In the second stage, these factors initiate a cascade of cellular and molecular events leading to endothelial and vascular dysfunction. The endothelial dysfunction leads to the clinically recognized symptoms of the syndrome, which include hypertension, proteinuria, thrombocytopenia and impaired liver function. Hypertension is mediated by various endothelial and non-endothelial regulatory factors that are altered in preeclampsia. This review aims to summarize the recent knowledge on the implication of the placenta and various angiogenic factors in the pathogenesis of preeclampsia.
 ...
PMID:[Preeclampsia as a maternal vascular disease]. 1796 10

The last 10 years witnessed the publication of many studies on the pathophysiology of thrombotic thrombocytopenic purpura (TTP), a life-threatening disease characterized by microangiopathic hemolytic anemia, thrombocytopenia and multiorgan failure. The most important finding was the identification of a novel metalloprotease, named ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motives), that is involved in the regulation of the size of von Willebrand factor (VWF), a major modulator of platelet adhesion and aggregation in the microcirculation. Inherited or acquired deficiencies of ADAMTS13 impair VWF cleavage, leading in turn to the disseminated formation of platelet-rich thrombi in the micro-circulation and to symptoms of end-organ ischemia. By measuring ADAMTS13 in plasma, it has been clearly shown that patients with inherited TTP have severe ADAMTS13 deficiency. However, patients with acquired TTP present with clinical and laboratory heterogeneity, and there are unequivocal cases of acquired TTP with measurable plasma levels of ADAMTS13. This heterogeneity poses a challenge for understanding the pathogenesis of TTP and selecting appropriate therapies.
 ...
PMID:TTP and ADAMTS13: When Is Testing Appropriate? 1802 19

The pattern of use of glycoprotein (GP) IIb/IIIa receptor inhibitors in peripheral percutaneous interventions (PPI) remains unclear. Data on patients who received GP IIb/IIIa inhibitors during PPI were extracted from a prospective registry that tracks demographic, angiographic and in-hospital outcomes of patients at 2 medical centers. Primary success was defined as establishing thrombolysis in myocardial infarction (TIMI) 3 flow and < 30% residual in vessels treated. Primary safety endpoints included death, unplanned amputation, vascular access complications, major bleeding and thrombocytopenia. Patients were divided into planned versus bailout use of GP IIb/IIIa inhibitors. A total of 46 patients (128 vessels) were included in this study. The procedure was performed emergently, urgently and electively in 13%, 26.1% and 60.9% of patients, respectively. The mean age was 70.9 +/- 11.2 years and 52.2% of patients were males. The patients' Rutherford-Baker Classes III, IV and V-VI were observed in 32.6%, 32.6% and 34.8%, respectively. Patients had the following comorbidities: current smokers 37%, diabetics 35.8%, dyslipidemics 71.7% and hypertensives 78.3%. Angiographic thrombus was suspected in 45.7% of patients prior to and during the procedure. The primary success endpoint was met in 66.4% of vessels and 69.6% of patients. Primary safety endpoints were as follows: death 2.2%, vascular access complication 2.2%, major unplanned amputation 0%, major bleeding 0% and thrombocytopenia 2.2%. Treatment with GP IIb/IIIa inhibitors was planned in 13 (28.3%) patients and bailout in 33 patients (71.7%). Reasons for planned GP IIb/IIIa were the presence of angiographic thrombus in 7 (53.8%) patients, advanced limb ischemia (Rutherford-Baker IV-VI) with total occlusions in 5 (38.5%) patients and acute presentation with total occlusion in 1 (7.7%) patient. Reasons for bailout were slow-flow in 16 (48.5%) patients, thrombus with no slow-flow in 12 (36.4%) patients, poor runoff in 1 (3%) patient and preventative during the procedure in 4 (12%) patients. In patients who received planned GP IIb/IIIa treatment, slow-flow occurred in 1/13 (7.7%) and embolization in 0/13 (0%) patients. We conclude that GP IIb/IIIa inhibitors were used as adjunctive therapy prior to angioplasty in critical limb ischemia patients or thrombotic lesions or as bailout in patients experiencing slow-flow and thrombus during PPI. Planned GP IIb/IIIa inhibitors appear to have favorable outcomes with a low incidence of slow-flow and embolization, however, randomized data are needed before establishing the role of GP IIb/IIIa inhibitor use in high-risk PPI.
 ...
PMID:Utilization of GP IIb/IIIa inhibitors in peripheral percutaneous interventions: current applications and in-hospital outcomes at a tertiary referral center. 1852 17

Thrombotic thrombocytopenic purpura (TTP) related to a severely deficient activity of the von Willebrand factor cleaving protease, ADAMTS (A Disintegrin And Metalloprotease with ThromboSpondin type 1 repeats) 13, is a life-threatening event, the onset of which may occur as early as childhood. TTP is either inherited (Upshaw-Schulman syndrome) via ADAMTS13 gene mutations (neonatal onset) or acquired via anti-ADAMTS13 autoantibodies (childhood onset). TTP is due to platelet- and von-Willebrand-factor-rich thrombi of the microvasculature, inducing mechanical hemolytic anemia, consumption thrombocytopenia, and multivisceral ischemia. Clinical course consists of relapsing acute events triggered mostly by infections, associated icterus and hyperbilirubinemia, severe hemolytic anemia with schistocytosis and a negative Coombs test, severe thrombocytopenia, and sometimes symptoms related to visceral ischemia (renal failure, central nervous system vascular events, other organ failure). The recently available ADAMTS13 laboratory investigation combining measurement of ADAMTS13 activity in plasma, search for an ADAMTS13 circulating inhibitor, and anti-ADAMTS13 IgG and ADAMTS13 gene sequencing is a crucial addition to TTP diagnosis. Plasma exchanges are first-line treatment of acquired TTP, combined with steroids and immunosuppressive drugs. Curative treatment of acute events in Upshaw-Schulman syndrome relies on plasma infusions (provider of active ADAMTS13). Guidelines for preventive treatment of relapses are not clearly established but should associate plasmatherapy and caution to triggers of relapses. Therapeutic perspectives are focused on the development of concentrated plasma-derived ADAMTS13 or recombinant ADAMTS13.
 ...
PMID:Thrombotic thrombocytopenic purpura related to severe ADAMTS13 deficiency in children. 1857 2

Thrombotic thrombocytopenic purpura (TTP) is characterized by microangiopathic hemolytic anemia and thrombocytopenia, accompanied by microvascular thrombosis that causes variable degrees of tissue ischemia and infarction. About 10-20% of TTP cases are associated with the pregnancy. Preterm delivery and intrauterine fetal death are frequent pregnancy complications of TTP. The following paper presents the case of a 32-year-old woman with TTP relapse at 10 weeks of her second pregnancy. Despite regular fresh frozen plasma transfusions, intrauterine fetal death occurred at 21 weeks of gestation. Current views on TTP management during pregnancy have been presented in the article as well.
 ...
PMID:[Thrombotic thrombocytopenic purpura in pregnancy. A case report]. 1881 60

Thrombotic thrombocytopenic purpura (TTP) is a severe multisystemic microvascular disease defined by the association of hemolytic anemia, thrombocytopenia, acute renal failure, fever, and neurological disorders. The pathophysiology has recently been elucidated by the discovery of a von Willebrand factor-cleaving protease (ADAMTS13) deficiency involved in platelet aggregation and ischemia. The association between TTP and acute pancreatitis (AP) has rarely been reported, described either as a cause or a consequence. The role of ADAMTS13 during AP is still unknown. We describe the case of a 41-year-old woman who developed a TTP, with decreased ADAMTS13 activity, associated with severe AP. Published cases of thrombotic microangiopathy associated with AP are reviewed. The pathophysiology, management, prognostic factors, and rationale for treatment are discussed. AP should be sought in patients with TTP presenting with abdominal pain. On the other hand, TTP should be considered in patients with AP and thrombocytopenia.
 ...
PMID:Thrombotic thrombocytopenic purpura associated with severe acute pancreatitis in a context of decreased ADAMTS13 activity: a case report. 1898 46

We report the case of a 56 years old male patient, smoker, obese, with untreated arterial hypertension, hospitalized on 16.02.07 with the diagnosis of inferior acute myocardial infarction, for which he received thrombolysis with streptokinase, followed by anticoagulation with non fractioned heparin. Two days later he started to complain of acute abdominal pain, and laboratory findings showed a low hemoglobin level. Imaging findings (ultrasonography and CT scan) showed evidence of subcapsular liver haematoma, caused by bleeding at hepatic and splenic level. He received red blood packed cells, fresh frozen plasma, cryoprecipitate, activated factor VII and was transferred by helicopter to Fundeni Clinical Institute--Intensive care unit (ICU). On admission, the patient was conscious, anxious, dyspneic, with mild hypoxia, with no signs of low cardiac output and with a painful abdomen. ECG, echocardiography and elevated myocardial necrosis enzymes confirmed myocardial infarction. Shortly after admission there was a worsening of his clinical condition, with a decrease in hemoglobin level despite red blood packed cells administration (Hb=7.8 g/dl) and thrombocytopenia (82000/mmc), with normal coagulation tests, thus suggesting active intraabdominal bleeding. Echography and CT scan confirmed bleeding. Emergency surgery was performed, showing massive haemoperitoneum (approx 4.5 L of blood), due to spontaneous rupture of a subcapsular hematoma in the liver. The surgical hemostasis was performed on the liver parenchyma laceration. Duration of surgery was 4 hours. There were no significant cardiac events during surgery (no signs of ischemia on ECG, no ST elevation), despite the need for inotropic agent. After surgery, the patient was referred to the ICU, intubated and ventilated, with inotropic support - dobutamine. Sequential ECG's, enzymatic trend and echocardiographies were performed to monitor myocardial ischemia. The outcome was favourable, no further bleeding and no postoperative myocardial infarction occurred. Secondary prevention was started early (thromboprophylaxis, selective beta-blocker, angiotensin inhibitors and statins). The patient had a favorable outcome and was discharged from the ICU the fourth day after surgery. He had a total length of stay in hospital of seven days, with a follow-up in the cardiology department.
 ...
PMID:[Liver rupture of a subcapsular haematoma after pharmacologic revascularization (Streptokinase) for acute myocardial infarction--case report]. 1926 Jun 36


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>