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)

Primary hyperoxaluria type 1 (PH 1) is complicated by a high rate of early end-stage renal failure (ESRF). In ESRF combined liver kidney transplantation has emerged as treatment of choice for teenagers and adults. In chronic renal failure (CRF) and for small children the situation is less clear. We report on three isolated liver transplantations and show the data of young children from the European Registry for liver transplantation in PH 1. Patient #1 developed ESRF at 3 months of age. Deficiency of alanine:glyoxylate aminotransferase proved PH 1. Progressive bone disease developed and the boy received a living related liver graft (LRLTx) at age two. Due to recurrent cholangitis kidney transplantation (KTx) is currently not feasible. Plasma oxalate decreased after LRLTx indicating correction of the metabolic defect. Patient #2 was diagnosed at the age of 14 months. He had nephrocalcinosis and hyperglycolic hyperoxaluria. Two years later he developed ESRF. At 5 years of age isolated liver transplantation was performed as a first step of therapy. Due to prolonged warm ischemia time organ function was poor. A severe bleeding complicated the course. The child died four weeks after transplantation from untreatable CMV septicemia. Patient #3 was evaluated for failure to thrive at 6 months of age. Urinary oxalate/creatinine ratio was 705 mumol/mol and gave rise to the diagnosis of PH 1. Renal failure slowly progressed to a creatinine clearance of 20 ml/min/1.73 m2 at 8 years, when liver transplantation (LTx) was performed. Four months later, GFR has not changed. Liver function and urinary oxalate/creatinine ratio are normal. Slowly deteriorating chronic renal failure can be stabilized through isolated liver transplantation and thus the rapid need for KTx will at least be delayed. Even more important, normalization of the oxalate metabolism prevents extrarenal oxalate deposits during renal failure.
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PMID:Transplantation procedures in primary hyperoxaluria type 1. 883 45

We report 81 of 107 cases of hemolytic uremic syndrome (HUS), admitted between July 1994 and February 1996, following an outbreak of Shigella dysenteriae type 1 dysentery in Kwazulu/Natal. All patients, excluding 1, were black with a mean age of 38 months (range 1-121); 50 (61.7%) were males. The mean duration of dysentery was 11.3 days (range 1-41) and HUS 15 days (range 1-91). Most patients had acute oliguric renal failure (90.1%), 42 (51.6%) required peritoneal dialysis. Complications included encephalopathy 30 (37.0%), convulsions 12 (14.8%) and hemiplegia 2 (2.3%), gastrointestinal perforation 8 (9.9%), protein losing enteropathy 26 (32.1%), toxic megacolon 4 (4.9%), rectal prolapse 5 (6.2%), hepatitis 11 (13.6%), myocarditis 5 (6.2%), congestive cardiac failure 3 (3.7%), cardiomyopathy 3 (3.7%), infective endocarditis 1 (1.2%), septicemia 15 (18.5%), disseminated intravascular coagulation 17 (21%). Leukemoid reactions were found in 74 (91.3%) patients, hyponatremia in 56 (69.1%), and hypoalbuminemia in 67 (82.7%). Stool culture for Shigella dysenteriae type I was positive in only 7 (8.6%) patients; Shiga toxin assays were not performed. Outcome was as follows: recovery 32 (39.5%), impaired renal function 8 (9.9%), chronic renal failure 26 (32.1%), end-stage renal disease 1 (1.2%), and death 14 (17.3%) patients.
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PMID:Post-dysenteric hemolytic uremic syndrome in children during an epidemic of Shigella dysentery in Kwazulu/Natal. 932 80

There is no doubt that acute renal failure (ARF) is associated with enhanced protein breakdown. It has been shown that protein split products can be measured in plasma samples of these patients. On the other hand, ARF frequently occurs in conditions of increased metabolic stress which leads to enhanced protein catabolism. Muscle wasting, loss of lean body weight, and a negative nitrogen balance result in malnutrition which considerably increases morbidity and mortality. Besides the accumulation of uremic toxins, several other factors are involved in the accelerated proteolysis in ARF. Metabolic acidosis appears to be one of the major catabolic factors in chronic renal failure, and probably in ARF as well. Insulin resistance, which is commonly attributed to uremia, also increases protein degradation. However, this derangement of carbohydrate metabolism is not directly accessible to therapy, in contrast to acidosis, which can be easily corrected by bicarbonate administration. There is further evidence that glucocorticoid excess contributes to the enhanced muscle proteolysis in ARF. Moreover, several studies have demonstrated that only in the presence of both glucocorticoids and acidosis could proteolysis occur. Investigation of the cellular mechanism by which muscle proteins are degraded indicates the importance of the cytosolic, soluble ATP- and ubiquitin-dependent proteolytic system. Successful treatment of various catabolic conditions with recombinant human growth hormone and insulin-like growth factor-I seems to be a promising strategy in severely catabolic patients with ARF. Anticytokine therapy appears to be another promising treatment in the course of catabolic illness due to sepsis; however, clinical application is still in its infancy.
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PMID:Protein catabolism in acute renal failure. 938 14

Loss of lean body mass is common in patients with acute or chronic renal failure but the mechanisms causing this loss are only beginning to be understood. One mechanism involves an inability of uremic patients to activate the critical metabolic responses that maintain protein balance when dietary protein is limited. Metabolic responses to dietary protein restriction include a sharp reduction in the degradation of essential amino acids and protein; changes in protein synthesis are less reliable. If uremia prevents suppression of essential amino acid or protein degradation when dietary protein is reduced by anorexia, negative nitrogen balance and loss of lean body mass will ensue. One complication of uremia, metabolic acidosis, stimulates the degradation of branched-chain amino acids and proteins and therefore blocks the ability of the patient to respond to a low-protein diet. The mechanisms require glucocorticoids and involve increased activity of branched-chain keto acid dehydrogenase and the ubiquitin-proteasome proteolytic pathway; there also is increased transcription of genes encoding components of enzymes involved in the pathways. Besides acidosis, a low insulin concentration and cytokines activate the ubiquitin-proteasome proteolytic pathway. Understanding how proteolysis is activated, including how these genes are stimulated, is important because the same pathways are activated in diabetes, cancer, sepsis, burns, starvation, and muscle denervation. Activation of the ubiquitin-proteasome pathway leads to reduced lean body mass.
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PMID:Robert H Herman Memorial Award in Clinical Nutrition Lecture, 1997. Mechanisms causing loss of lean body mass in kidney disease. 949 77

A 27-year-old woman with chronic renal failure, who had been treated with chronic ambulatory peritoneal dialysis and had developed sclerosing peritonitis, was admitted to the hospital with intra-abdominal sepsis. In spite of antibiotic therapy, sepsis recurred and was associated with intrahepatic cholestasis. In addition, over a period of about 4.5 weeks she developed hepatomegaly and portal hypertension unassociated with occlusion of the portal vein or one of its main extrahepatic branches. A wedge biopsy of the liver revealed extensive thick fibrosis of the liver capsule, intrahepatic cholestasis, diffuse swelling of hepatocytes, central veins that were difficult to visualize and small portal tracts. It is suggested that the sepsis was responsible for the intrahepatic cholestasis, swelling of hepatocytes and hepatomegaly. It is also suggested that the rigidity of the fibrotic liver capsule provided resistance to the development of hepatomegaly, with the result that intrahepatic pressure increased (compressing intrahepatic branches of the portal vein as well as portal tracts and central veins) and portal hypertension developed.
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PMID:Sclerosing peritonitis complicated by sepsis: a potential cause of portal hypertension. 951 60

Post-transplant neutrophilic interstitial nephritis (NIN) is characterized by an interstitial infiltrate consisting of polymorphonuclear cells that leads frequently to acute graft dysfunction. In 220 graft biopsies performed because of renal dysfunction over 2 years in our unit, 11 (5%) diagnoses of NIN were made. Only two patients had chronic pyelonephritis as original disease. Four patients had urological problems before transplantation. After transplantation, five patients had urinary tract infection, one had urethral stenosis, two had vesicourethral reflux and one patient had a perinephritic abscess. Seven patients had fever (63%). Only in six patients did urine culture lead to microorganism isolation. After 6 months, only two patients had a serum creatinine level < 1.4 mg/dl, five patients had abnormal function, three had lost their grafts, and one patient had died with sepsis. We conclude that 5% of the biopsies performed in our center disclosed NIN, an entity that causes graft dysfunction and progresses frequently to chronic renal failure. In some cases, no infectious etiology could be detected.
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PMID:Post-transplant neutrophilic interstitial nephritis--an important cause of graft dysfunction. 966 65

A 49-year-old black man with hypertension-induced chronic renal failure requiring hemodialysis and a history of arteriovenous access graft infection was admitted with Staphylococcus aureus sepsis, dyspnea, and peri-incisional erythema over his arteriovenous graft fistula. Results of a transthoracic echo demonstrated aortic sclerosis and concentric left ventricular hypertrophy. Results of a whole-body In-111 white cell (WBC) scan were negative over the arteriovenous graft site; however, an intense abnormal focus of labeled WBCs was evident to the left of the sternum. A subsequent transesophageal echocardiogram showed a mixed cystic-solid calcified mass adjacent the left aortic cusp. Surgery confirmed a perivalvular abscess. As a whole-body imaging modality, the In-111 WBC scintigram indicated the true location of the infectious process responsible for the patient's sepsis. The combination of echocardiography and radiolabeled WBC imaging increases sensitivity for detection of endocarditis/perivalvular abscess. Radiolabeled WBC imaging is more efficacious for monitoring therapy because the echocardiogram often does not change with treatment of endocarditis/perivalvular abscess.
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PMID:Perivalvular abscess complicating infective endocarditis: complementary role of echocardiography and indium-111-labeled leukocytes. 973 77

Calcific uremic arteriolopathy (calciphylaxis) is an uncommon complication of chronic renal failure that is associated with high morbidity and mortality. We report 16 patients (13 female) who presented between 1985 and 1996. All patients developed painful livido reticularis that progressed to cutaneous necrosis and ulceration (11 cases on the proximal extremities and five cases on the distal extremities). Two patients with predominately distal leg disease survived; the cause of death in the other 14 patients was sepsis (six patients), withdrawal from dialysis (three), cardiac arrest (three), and gastrointestinal hemorrhage (two). Mesenteric ischemia from intestinal vascular calcification occurred in two cases. Clinical factors identified included the use of warfarin therapy in seven cases and significant weight loss (>10% body weight) in seven cases in the 6 months preceding the development of calcific uremic arteriolopathy. Skin pathology was studied in 12 cases, with all showing calcific panniculitis and small vessel calcification. Electron microscopic spectral analysis of the mineral content of the calcific lesions in the subcutaneous tissue showed only calcium and phosphorous. In two cases, substitution of low molecular weight heparin for warfarin therapy resulted in clinical improvement. Current theories of pathogenesis and treatment are reviewed. This study confirms the high morbidity and mortality of calcific uremic arteriolopathy producing ischemic tissue necrosis while drawing attention to significant weight loss and warfarin therapy as risk factors for the development of ischemic tissue necrosis. Hyperbaric oxygen therapy warrants further study.
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PMID:Cutaneous necrosis from calcific uremic arteriolopathy. 974 Jan 72

The reasons and course of acute renal failure during pregnancy, labour and puerperium were presented in 30 women treated in Provincial Hospital in Kielce. The most frequent reason was haemorrhage--15 (50%) women, sepsis--10 (33,3%) women and preeclampsia--eclampsia--2(6, 6%) women. 15 women died as the consequence of multiple organ failure. Among 15 women who survived renal function has returned completely in 13 (86, 6%) ones. In remaining 2 (13, 4%) women chronic renal failure persisted.
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PMID:[Acute renal failure during pregnancy, labor and puerperium in women]. 974 88

We clinically investigated a total of 288 cases of bacteremia for the past ten years, from January 1986 to December 1995, at the Second Department of Internal Medicine in the jikei University Hospital. All of the subjects who had a positive reaction to blood culture or catheter tip culture were investigated for their basic disease, complications, and detected bacteria. Malignant tumors, chronic renal failure, diabetes mellitus, and hematologic disease were frequent by noted. The cases due to primary infection were mainly respiratory organ infection or urinary tract infection, which were 47.8% of the total. In 31.3% of the total, catheter tip cultures were positive. Except for catheter related infection, Gram-positive coccus were detected in 40.3%, which was most frequent. Methicillin resistant Staphylococcus aureus (MRSA) were 8.1% and Staphylococcus epidermidis were 11.2%. In catheter related infection, Gram-positive coccus were detected in 59.9%, which was most frequent amongst them, MRSA was 17.2%, S. epidermidis was 16.2%. The mortality of bacteremia was 12.5%, mainly from hematologic diseases, immunodeficiency due to long term steroid administration etc. Accordingly, the more the advance of chemotherapy, the better the prognosis of septicemia is. Appearance of catheter related infection was unexpected frequent. Increase of immunocompromised host is thought to be one of the main factors in the outbreak of bacteremia.
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PMID:[A clinical investigation of bacteremia for the past ten years at the Second Department of Internal Medicine, Jikei University Hospital]. 978 May 85


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