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)

We reported a family with dentato-rubro-pallido-luysian atrophy (DRPLA) and chronic renal failure. The proband was a 66-year-old woman who developed gait disturbance, limb ataxia, pyramidal tract signs, and dementia since age 54. T2-weighted brain MR images revealed symmetric high-signal lesions in the cerebral white matter, in addition to cerebellar, brainstem, and cerebral cortical atrophy. She suffered from renal failure and became dialysis-dependent at the age of 59, four years after the onset of chronic nephritic syndrome. At the age of 66, she was admitted to our hospital because of hyperthermia and disturbance of consciousness, and died of DIC. Her CAG repeats in the DRPLA gene were 58 and 12. An autopsy was performed. The brain weighed 910 g. Histological findings confirmed the diagnosis of DRPLA. Her mother died of chronic renal failure. All three siblings had cerebellar ataxia, and two siblings had chronic nephritic syndrome. Among them, only her younger brother was diagnosed as non-IgA glomerulonephritis based on kidney biopsy findings at the age of 48. Though the nature of the association between DRPLA and renal dysfunction remains obscure, the DRPLA gene abnormality may be correlated with chronic renal failure in this family.
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PMID:[A family with DRPLA and chronic renal failure]. 1096 59

We describe here two cases of chronic disseminated intravascular coagulation(DIC) secondary to aortic aneurysms. The patients were 78- and 84-year-old males, who visited our hospital to receive hemodialysis therapy for chronic renal failure probably due to nephrosclerosis. They had mild bleeding tendency and thrombocytopenia(< 10 x 10(4)/microliter). Coagulation test revealed the findings of chronic DIC in both patients, and computed tomography showed abdominal and thoracoabdominal aortic aneurysms with mural thrombi, respectively. In one patient, subcutaneous hemorrhage after vascular access surgery had continued for a month. However, the hemorrhage and swelling of the limb disappeared after continuous subcutaneous heparin infusion(CSHI) therapy in a daily dose of 10,000-14,000 unit. These findings suggest that chronic DIC secondary to aortic aneurysm should be considered when bleeding tendency and thrombocytopenia are observed in aged patients, and that CSHI is the choice of therapy for the bleeding tendency of chronic DIC.
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PMID:[Two aged patients with chronic renal failure and chronic disseminated intravascular coagulation secondary to aortic aneurysms: effect of continuous subcutaneous heparin infusion therapy]. 1115 5

Hepatocyte growth factor (HGF) facilitates the regeneration of injured kidney in acute renal failure (ARF). HGF is produced as a single-chain precursor by cells of mesenchymal origin and is converted to a biologically active, heterodimeric molecule by proteolytic processing. We studied HGF mRNA and protein levels in systemic organs of glycerol-induced ARF rats, a model of crush syndrome. HGF protein concentration of tissue homogenate was measured by ELISA. Both mRNA and protein levels were increased in liver and spleen at 24 hours after the glycerol injection whereas HGF protein level was decreased in the injured kidney. Expression of HGF receptor/c-met mRNA was elevated only in the kidney. These results suggest that HGF supplied in an endocrine manner may play an important role in the regenerating process following ARF. Next, we measured serum HGF concentration by ELISA in 8 ARF patients caused by crush syndrome and the molecular size of serum HGF was determined by immunoblotting. Although serum HGF levels elevated in all patients, the HGF levels did not associate with their prognoses. While a single-chain molecule was predominantly observed in sera from chronic renal failure patients and healthy subjects, the majority of serum HGF was a heterodimeric form in 7 ARF patients. In one patient who developed disseminated intravascular coagulation syndrome and had a poor prognosis, a single-chain molecule was predominant although the serum HGF concentration was equivalent. These data suggest that the activity of proteolytic processing may be also an important factor for the expression of the biological function of HGF.
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PMID:Production and activation of hepatocyte growth factor in acute renal failure. 1149 73

A 75-year-old man presented with a rare case of chordoma in the sella turcica of the skull base. He had been treated for hypertension and chronic renal failure since 1990. Computed tomography detected a tumor in the sella turcica in 1994, but the patient had no clinical complaints and the serum pituitary hormone levels were normal. He died of disseminated intravascular coagulation, myocardial infarction, pulmonary infection, and multiple cerebral infarctions in 2000. At autopsy, the tumor in the sella turcica was 3.1 cm in greatest diameter and had compressed the pituitary gland posteriorly. Histological examination found oval cells and vacuolated short spindle-shaped cells which showed morphological changes similar to myxoma cells. The tumor was lobulated by narrow connective tissues. The tumor did not contain any cartilaginous tissue components, and was stained positively for epithelial membrane antigen but negatively for S-100 protein. The final diagnosis was chordoma. There was no association between the tumor and the cause of death.
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PMID:Chordoma in the sella turcica. 1216 Mar 11

Severe anemia, growth retardation, diabetes mellitus, cardiac disorders, and, infrequently, stroke are well-known complications of thalassemia major. We report a girl, age 7 years, 2 months, with beta-thalassemia major associated with chronic renal failure, diabetes mellitus, and cardiomyopathy in whom a silent stroke was noted during follow-up. She was diagnosed with thalassemia major at age 6 months, chronic renal failure at age 3 years, 3 months, and diabetes mellitus and cardiomyopathy at age 7 years. Although cranial computed tomography was found to be normal at the age of 3 years, 3 months, magnetic resonance imaging showed cerebral infarct in the right frontal region at 7 years, 2 months. A thrombophilic panel revealed increased factor VIII and decreased protein C concentrations. She died from disseminated intravascular coagulation at age 7 years, 9 months. We did not record any clinical findings of stroke during her follow-up. We think that diabetes mellitus, dilated cardiomyopathy, and increased factor VIII and decreased protein C concentrations led to the occurrence of cerebral infarct. In conclusion, we emphasize that children with thalassemia major should be monitored closely for stroke. We also suggest that stroke can show a silent progression in severely affected children, as in our case.
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PMID:Silent stroke in a case of beta-thalassemia major associated with chronic renal failure and diabetes mellitus. 1469 9

We report a very high risk case of reoperation for pseudoaneurysm after ascending aortic replacement for acute aortic dissection in a 78-year-old man with chronic renal failure and disseminated intravascular coagulation (DIC). Computed tomography 5 years after the 1st operation showed huge pseudoaneurysm originated from the distal anastomosis and the angiogram showed moderate aortic regurgitation. Hemodialysis and congestive heart failure associated with DIC complicated his general condition. Preoperative DIC score was 7 with D-dimer of 39.8 microg/ml. The patient underwent reoperation through night anterior thoracotomy. At 20 degrees C of urinary bladder temperature, we started re-median sternotomy and ablated the adhesion. When the pseudoaneurysm ruptured, we started hypothermic circulatory arrest with selective cerebral perfusion immediately. And Bentall operation and hemi-arch replacement were performed. Postoperative recovery required long period and he was transferred to another hospital at 3 months after the surgery. Postoperative data showed reduction of DIC score to 3.
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PMID:[Reoperation for postoperative pseudoaneurysm after repair for acute aortic dissection associated with disseminated intravascular coagulation and chronic renal failure]. 1989 70

Hemolytic uremic syndrome (HUS) is related to a renal thrombotic microangiopathy, inducing hypertension and acute renal failure (ARF). Its pathogenesis involves an activation/lesion of microvascular endothelial cells, mainly in the renal vasculature, secondary to bacterial toxins, drugs, or autoantibodies. An overactivation of the complement alternate pathway secondary to a heterozygote deficiency of regulatory proteins (factor H, factor I or MCP) or to an activating mutation of factor B or C3 can also result in HUS. Less frequently, renal microthrombi are due to an acquired or a constitutional deficiency in ADAMTS-13, the protease cleaving von Wilebrand factor. Hemolytic anemia with schistocytes, thrombocytopenia without evidence of disseminated intravascular coagulation, and renal failure are consistently found. In typical HUS, a prodromal diarrhea, with blood in the stools, is observed, related to pathogenic enterobacteria, most frequently E. Coli O157:H7. HUS may also occur in the post partum period, and is then related to a factor H or factor I deficiency. HUS may also occur after various treatments such as mitomycin C, gemcitabine, ciclosporin A, or tacrolimus, and as reported more recently bevacizumab, an anti VEGF antibody. Atypical HUS are not associated with diarrhea, may be sporadic or familial, and can be related to an overactivation of the complement alternate pathway. More recently, some of them have been related to a mutation of thrombomodulin, which also regulates the alternate pathway of complement. In adults, several HUS are encountered in the course of chronic nephropathies: nephroangiosclerosis, chronic glomerulonephritis, post irradiation nephropathy, scleroderma, disseminated lupus erythematosus, antiphospholipid syndrome. Overall the prognosis of HUS has improved, with a patient survival greater than 85% at 1 year. Chronic renal failure is observed as a sequella in 20 to 65% of the cases. Plasma infusions and plasma exchanges are effective in most of the cases to treat hemolysis and thrombocytopenia. Steroid therapy is debated, as well as immunosuppressive drugs, including rituximab, in autoimmune forms. A new monoclonal anti-C5 antibody is tested, and seems to be effective in atypical HUS with abnormal complement alternate pathway activation. If terminal renal failure occurs, renal transplantation can be performed but the risk of recurrence, which very low in post infectious forms of HUS, is about 70 to 80% in genetic forms of complement regulatory protein deficiency.
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PMID:[Hemolytic uremic syndrome in adults]. 2039 68

Plasmic and platelet components of hemostasis were examined in 50 patients with terminal chronic renal failure (CRF) aged 23 to 67 years and 30 healthy controls of the same age. A plasmic hemostasis component was studied basing on 11 parameters of coagulogram. A platelet hemostasis component was studied by platelet aggregation: spontaneous and induced by ADP (in concentration 1.25, 2.5 and 5.0 mkg/ml), collagen, adrenalin and ristomycin. All CRF patients before hemodialysis had a significant alterations of 6 indices of a plasmic component of hemostasis: activated partial thromboplastic time, content of soluble fibrinmonomeric complexes, thrombine time; of 3 from 7 tests of aggregatogram (ADP, collagen, ristomycin induced aggregation). After hemodialysis severity of the above pathological shifts deteriorated (1.5 to 5 times). Thus, CRF patients on hemodialysis showed aggrevation of impairment of all hemostasis components. They are at risk of hypercoagulation, DIC-syndrome, massive thromboembolism. The above impairment of hemostasis should be considered in prescription of anticoagulant therapy to CRF patients. Monitoring of hemodialysis and adequate correction of the hemostasis system defects may contribute to improvement of quality of life of patients with terminal CRF and lowering of their mortality rate.
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PMID:[Changes in plasmic and platelet hemostasis in patients with chronic renal failure in hemodialysis]. 2264 97

Acquired factor XIII (FXIII) deficiency is a common disease and seldom causes bleeding. However, severe FXIII deficiency may result in life-threatening bleeding. Although the inhibitor against FXIII has recently been focused as the cause of haemorrhagic acquired FXIII deficiency, the pathophysiology of inhibitor-negative cases could also be involved. We report a case of an 85-year-old Japanese man with serious subdural haemorrhage showing a remarkable decreased level of FXIII activity. He also manifested complications of compensated disseminated intravascular coagulation (DIC) with chronic renal failure, abdominal aortic aneurysm (AAA) and right renal carcinoma. Despite the successful evacuation of the haemorrhage, acute subdural haemorrhage subsequently developed that necessitated further craniotomies. Plasma cross-mixing studies and dot blot assay revealed no inhibitors against FXIII. We speculated that the decreased FXIII activity could be mainly due to hyperconsumption by DIC and surgery. Because plasma-derived FXIII concentrates are available to stop bleeding, clinicians should be aware of severe acquired inhibitor-negative FXIII deficiency in cases of unexplained excessive bleeding.
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PMID:Severe inhibitor-negative acquired factor XIII/13 deficiency with aggressive subdural haemorrhage. 2351 1

Life threatening event due to central airway obstruction caused by very large blood clot formation with profuse on going bleeding its very challenging to manage. Interventional pulmonologist must aware about this situation which can lead to respiratory failure. There are several choices to treat this unlikely situation, in example flexible bronchoscopy with forceps, bronchial lavage, and suction. We present a case with post-surgical tracheostomy bleeding which caused a giant blood clot formation in a disseminated intravascular coagulation due to severe sepsis in end stage renal disease patient, successfully managed with cryoextraction and argon plasma coagulation. Combinations of two endobronchial approaches can give a quick, safe and cost effective lifesaving treatment.
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PMID:Combination of bronchoscopic cryoextraction and argon plasma coagulation in treatment of total central airway obstruction caused by giant blood clot formation in massive airway bleeding. 2733 Sep 65


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