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)

We report on a 45-year-old patient, who first developed pemphigus vegetans of the Neumann type 9 years ago and who presented with typical manifestations of the disease 6 years after the first exacerbation. On systemic therapy with corticosteroids progression of the disease ceased. Additional application of azathioprine, cyclophosphamide or gold thiomalate, however, did not make it possible to reduce the high corticosteroid dosage without exacerbation of the disease. The long-term corticosteroid therapy led to sepsis, thrombosis of the leg and pelvic veins and osteoporosis with crush fractures of the vertebrae; corticosteroid therapy was therefore discontinued and replaced by cyclophosphamide bolus therapy associated with gold thiomalate. The subsequent exacerbation of the pemphigus was treated with methotrexate and dapsone. The skin lesions progressed further, however, and the patient died of sepsis 39 weeks after admission to the clinic.
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PMID:[Neumann type pemphigus vegetans with fatal outcome]. 844 2

Normal human ageing is associated with changes in body composition which include a decrease in muscle mass and strength, bone mass loss and increase in adipose mass. A physiological decrease in growth hormone (GH) and IGF-I secretion accompanies these changes. Many of the physiological changes that accompany normal human ageing are very similar to those found in GH deficient patients. Nevertheless, responsiveness to exogenous administered GH persists with advancing age. GH administration to elderly individuals has produced an decrease in fat mass and an increase in lean body mass, being this finding consistent with the hypothesis that GH deficiency could be a contributing cause to senescent changes in some elderly individuals. GH treatment has also been used with encouraging results in adult subjects with isolated or combined GH deficiency. On the other hand, several clinical studies have recently shown the efficiently of GH treatment on diverse pathological processes such as severe catabolic states (surgery, sepsis, trauma, buns), osteoporosis, diabetic ulcers and obesity. The most frequent side effects are sodium and water retention, impairment in glucose tolerance and carpal tunnel syndrome, although in general the treatment has been well tolerated. The clear definition of the therapeutical applications of GH in the adult warrants further clinical investigation.
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PMID:[New physiological and pharmacological aspects of the growth hormone (II). Therapeutic applications in adults]. 849 41

The vascular anatomy in the acetabular region involves a certain risk of arterial and venous injuries complicating orthopaedic surgery. These complications have been grouped into four categories: lacerations, thrombosis, pseudoaneurysms and arteriovenous fistula. In a period of 5 years, three injuries of the external iliac artery and four lesions of the femoral artery associated with total hip arthroplasty were treated surgically at the Department of Vascular Surgery of the University Hospital in Graz. In one case a concomitant lesion of the pelvic vein was observed. The incidence of iatrogenic vascular injuries in total hip surgery is 0.3%. Combined injury of the external iliac artery and vein led to a life-threatening bleeding complication. The vascular lesion became manifest as acute ischaemia of the lower extremity or as an acute haemorrhage 30 min to 2 h after primary surgery. The late complication of a false aneurysm of the femoral artery occurred in one patient 3 weeks after total hip replacement. Reconstruction of the vascular lesions was performed by direct suture, except that two arterial lesions required the use of polytetrafluoroethylene (PTFE) vascular grafts. Vessels in the pelvic region are at high risk if screw fixation acetabular components are used. Perforation of the iliac artery by protruded methylmethacrylate polymer components of cement has been documented. The obturator vessels are in a vulnerable position if the acetabular floor has been broached by operative instruments or eroded by loosening of the prosthesis facilitated by osteoporosis, steroids or sepsis. Femoral vessels are endangered by Hohmann retractors that are not placed directly on bone. Though vascular injury during hip operations is rare, recognition of such complications is important as safe and satisfactory treatment can be achieved. Rapid identification and immediate surgical repair of these lacerations are essential for their management.
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PMID:[Vascular lesions in surgery of the hip joint]. 915 60

Clinical, laboratory and radiological data of 35 patients (> 60 years) with septic arthritis were retrospectively analyzed in 2 groups submitted to different treatment protocols based on the radiological stage of destruction. Group I: intraarticular gentamicin-PMMA beads (8 cases), group II: resection-arthroplasty with gentamicincement spacer (27 cases). The average diagnostic delay was 4.8 months. Diagnostic errors were: osteoarthrosis in 9 cases, periarthritis of the shoulder in 5, femoral head necrosis in 4 cases, sciatic pain in 3, osteoporosis in 1, and thrombosis in 1 case. The re-operation rate in group I was 1.4 (range 1-2 operations) and in group II 1.2 (range 0-3 operations). Fifteen patients were left with a resection-arthroplasty. The restriction of motion remained moderate in 6 and severe in 9 cases. Three patients had sepsis and died. The final results were poor after both treatment protocols. Early diagnosis seems to be the most determining factor concerning the final outcome of septic arthritis.
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PMID:Diagnosis and treatment of joint infections in elderly patients. 968 51

Although the short- and medium-term (5-10 years) outcome of patients with lupus nephritis has been studied extensively, there are very few data on the second and subsequent decades. We studied outcome in 110 local patients investigated at a single centre before 1986, who all had potential follow-up of more than 10 years (actual 2-31 years, median 15.5 years). At last follow-up, 40 patients were dead and 70 alive, nine of whom were on maintenance dialysis or transplanted, actuarial survivals being 84%, 72%, 62%, 61% and 54% at 5, 10, 15, 20 and 25 years for the group as a whole. Survival was better in the cohort 1976-86 (n = 60) than in that from 1963-75 (n = 50) (90, 81 and 76% vs. 78, 56 and 43% at 5, 10 and 15 years, p < 0.001). Sepsis (12) and myocardial infarction (8) were the principal causes of death. Of living patients with renal function, 38% had normal urine and renal function, 11 were off all treatment (19%), 62% had persistent proteinuria and 18% had reduced but generally stable renal function. Renal failure, in those patients who developed it, occurred during the first decade and none of 67 patients actually followed more than 10 years subsequently went into renal failure. Induction treatment was with prednisolone, combined with azathioprine in more severe forms of nephritis, and from the middle 1970s to 1986, 30 with methylprednisolone and in 12 cases plasma exchange. Seventeen other patients were treated using oral cyclophosphamide during the 1960s. No patient received i.v. cyclophosphamide as induction therapy, although nine patients had this form of treatment later, largely because of non-compliance. Serious complications of lupus and/or its treatment occurred in 49%: sepsis in 32, ischaemic heart disease in 20, thrombosis in one and avascular necrosis of bone in eight. In contrast, fracturing osteoporosis occurred in only three, and cataracts requiring surgery and diabetes mellitus in none. The very long-term outlook of lupus nephritis, especially its more severe forms, has improved, but that with current management strategies only a minority of patients are able to stop treatment altogether, and the incidence of serious complications is high.
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PMID:The very long-term prognosis and complications of lupus nephritis and its treatment. 1039 9

We report an unusual case of congenital leukemia with leukemia cutis (LC) and diffuse calcinosis cutis. A newborn girl presented with widespread dusky red and yellowish cutaneous nodules and papules. Bone marrow morphology was consistent with the diagnosis of acute monocytic leukemia of the FAB M5 type. Skin biopsy specimens confirmed the presence of a leukemic infiltrate and revealed calcium salt deposition in the papillary and reticular dermis. Calcinosis was diffuse in the whole skin but spared other organs. Vascular calcification was not present. Serum calcium levels oscillated between 2.5 and 2.86 mmol/l, and phosphorus, parathyroid hormone and 25-hydroxyvitamin D(3) levels were normal. There were diffuse osteoporosis and spontaneous fractures of small tubular bones. The patient responded to chemotherapy but, following consolidation treatment, developed sepsis and died at 120 days of age. Congenital leukemia is rare and LC is uncommon. Hypercalcemia may be a complication of leukemia, which leads to multiorgan metastatic calcification. Despite the absence of frank hypercalcemia, the presence of bone lesions suggests that the patient's calcinosis cutis was of the metastatic type. However, the cutaneous leukemic infiltrate may also represent a triggering factor for calcium deposition in the skin.
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PMID:Diffuse calcinosis cutis in a patient with congenital leukemia and leukemia cutis. 1077 6

Tumor necrosis factor (TNF) is a major mediator of apoptosis as well as inflammation and immunity, and it has been implicated in the pathogenesis of a wide spectrum of human diseases, including sepsis, diabetes, cancer, osteoporosis, multiple sclerosis, rheumatoid arthritis, and inflammatory bowel diseases. The interaction of TNF with TNF receptor-1 (TNF-R1) activates several signal transduction pathways. A common feature of each pathway is the TNF-induced formation of a multiprotein signaling complex at the cell membrane. Over the past decade, many of the components and mechanisms of these signaling pathways have been elucidated. We provide an overview of current knowledge of TNF signaling and introduce an STKE Connections Map that depicts a canonical view of this process.
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PMID:TNF-R1 signaling: a beautiful pathway. 1204 Jan 73

This article reviews the current use of the wide variety of imaging modalities now available, presenting the imaging features of common and important causes of acute and chronic rheumatic disorders including juvenile idiopathic arthritis, spondyloarthropathies/enthesitis-related arthritis, sepsis, autoimmune diseases, vasculitis, and osteoporosis.
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PMID:Radiologic investigation of rheumatic diseases. 1793 72

Lung transplantation is currently the most effective means of improving survival and quality of life in patients with end-stage cystic fibrosis. In reviewing our 6-year experience we sought to evaluate complications and survival after sequential bilateral lung transplantation. Between October 1996 and October 2002, 114 patients with cystic fibrosis were referred to us from 15 Italian regional centers and 2 support centers for cystic fibrosis as possible candidates for lung transplantation. Of these 114 patients, 99 were included in the waiting list and 15 were refused. The mean time spent on the waiting list was 6.8+/-5.2 months (range 1 day-21 months) for those patients receiving lung transplantation, and 5.4+/-4.5 months (range 10 days-18 months) for those 35 patients who died while on the waiting list. A total 55 patients (6 children and 49 adults), mean age 25.6+/-6.6 years (range 9-52 years), 29 males, underwent bilateral sequential lung transplantation. One patient had a second transplantation 14 months after the first. The most frequent medical non-infective complications after transplantation were chronic renal failure (n=27 patients), diabetes (n=31), osteoporosis (n=17), arterial hypertension (n=14), seizures (n=4), transient cerebral ischaemia (n=1), and transient bilateral blindness (n=1). Bacterial lower airways respiratory infections with the organisms that colonized patients' airways before lung transplantation developed in 42 patients; cytomegalovirus (CMV) infection in 41; and opportunistic infections of the lung with Pneumocystis carinii in 3 patients. Cultures of sputum or bronchoalveolar lavage fluid grew Aspergillus fumigatus in nine patients; aspergillosis of right bronchial anastomosis developed in one patient and a lung infection in another. Another patient had a pulmonary infection secondary to Aspergillus niger. An average of 1.3 episodes of acute rejection developed per patient in the first 6 months after lung transplantation. Freedom from bronchiolitis obliterans syndrome was 95% at 1 year, 82.5% at 2 years, 70% at 3 years, and 65% at 4, 5 and 6 years. Actuarial survival rates were 80% at 1 month, 79% at 1 year, 74% at 2 years, 70% at 3 years and 58% at 4, 5 and 6 years. Ten patients (17.8%) died in the early postoperative period (1-30 days) for the following reasons: primary graft failure (n=4), multiorgan failure (n=3), Burkholderia cepacia sepsis (n=1), myocardial infarction (n=1), and pulmonary embolism (n=1). Mortality was accounted for by 9 patients (16%) who died from 9 to 43 months after lung transplantation, for the following reasons: P. carinii infection (n=2), bronchiolitis obliterans syndrome (n=4), A. fumigatus pulmonary infection (n=1), unknown cause (n=1) and suicide (n=1). In conclusion, the leading causes of morbidity after lung transplantation for cystic fibrosis are pulmonary bacterial infection and opportunistic infections. Bronchiolitis obliterans develops in more than half of lung transplant recipients who survive for more than 3 years and is an important cause of death in the late post transplantation period.
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PMID:Lung transplantation for cystic fibrosis: 6-year follow-up. 1591 93

Tumor necrosis factor-alpha (TNF) is a major mediator of apoptosis as well as immunity and inflammation. Inappropriate production of TNF or sustained activation of TNF signaling has been implicated in the pathogenesis of a wide spectrum of human diseases, including cancer, osteoporosis, sepsis, diabetes, and autoimmune diseases such as multiple sclerosis, rheumatoid arthritis, and inflammatory bowel disease. TNF binds to two specific receptors, TNF-receptor type I (TNF-R1, CD120a, p55/60) and TNF-receptor type II (TNF-R2, CD120b, p75/80). Signaling through TNF-R1 is extremely complex, leading to both cell death and survival signals. Many findings suggest an important role of phosphorylation of the TNF-R1 by number of protein kinases. Role of TNF-R2 phosphorylation on its signaling properties is understood less than TNF-R1. Other cellular substrates as TRADD adaptor protein, TRAF protein family and RIP kinases are reviewed in relation to TNF receptor-mediated apoptosis or survival pathways and regulation of their actions by phosphorylation.
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PMID:TNF signaling: early events and phosphorylation. 1806 42


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