Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
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There are few reports of combined heart and liver transplantation (CHLT) for familial amyloidotic polyneuropathy (FAP). The technique for the operation remains to be defined. Four CHLTs were performed for amyloidogenic transthyretin-related (variant Glu89Gln-ATTR Glu89Gln) cardiomyopathy in our center. Patients 1 and 4 had no serious involvement of other organs, whereas patients 2 and 3 had evident peripheral neuropathy and gastrointestinal motility alterations. Patient 3 also had high-grade orthostatic hypotension. All four patients underwent cardiac and sequential hepatic transplantation with organs procured from the same donor. Venovenous bypass was used in patients 1 and 4 who experienced uncomplicated procedures. The amyloidotic liver of patient 4 was successfully utilized for a domino procedure to treat a patient with hepatocellular carcinoma on cirrhosis. The cardiac performance of patients 1 and 4 remains normal; there has been no progression of amyloidosis at 42 and 1 months after transplantation. Patient 2 had no intraoperative complications but experienced postoperative bleeding, renal failure, sepsis, and heart failure, and finally died of multiorgan failure 2 months after transplant. In patient 3, right hemicolectomy was required intraoperatively due to intestinal ischemia, without significant hemodynamic instability, while extracardiac symptoms of amyloidosis gradually worsened postoperatively. In conclusion, CHLT for ATTR Glu89Gln may be performed even in patients with advanced disease. However, the most compromised patients are more likely to display intraoperative risks, postoperative complications, and worsening of extracardiac, extrahepatic symptoms.
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PMID:Combined heart and liver transplantation in four adults with familial amyloidosis: experience of a single center. 1511 Jun 20

A young female with Fisher-Evans' syndrome and a previous melanoma developed acute renal failure with generalized lymphoadenopathy and fever. The appearance of renal lesions is common in the course of several hematological disorders, but is unusual in Fisher-Evans' syndrome. Fisher-Evans' syndrome, defined as Coombs' positive hemolytic anemia and immune thrombocytopenia, is more frequently associated with the other autoimmune diseases, but not with renal involvement. In our case report, having excluded amyloidosis, myeloma, interstitial nephritis and sepsis, the rapid involvement of renal function with enlarged renal size seemed to suggest renal lymphoma. However, the lack of a monoclonal T-lymphocyte population in the renal tissue and peripheral blood, along with a clinical course characterized by a rapid reversibility of acute renal failure made this diagnosis rather an unlikely one. Polyclonal lymphocyte infiltration in a patient with a persistent autoimmune disease made us suspect a hyperimmune reaction. This syndrome is a non-neoplastic proliferation of B-cells involving an exaggeration of lymphocyte transformation. However, the clinical course is progressive and fatal, and can trigger a lymphoproliferative systemic disease. In our patient, two elements led us to suspect it was not a typical hyperimmune syndrome: first, polyclonal lymphocytes had massively infiltrated the kidney and, secondly, the clinical outcome was extremely favorable. Therefore, we were faced with an "atypical" and "singular" hyperimmune reaction with renal involvement, polyclonal proliferation of T-lymphocytes that had exhausted itself over time. Infective or toxic agents or drugs such as cyprofloxacin could have triggered the phenomenon, in the presence of a favorable condition such as Fisher-Evans' syndrome.
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PMID:Acute renal failure in a young woman with Fisher-Evans' syndrome. 1559 44

Compared to young patients with Takayasu's arteritis (TA), little information about elderly patients with TA has been reported. Additionally, no reports were found regarding TA cases with complications of intestinal amyloidosis. This is a case report of an elderly female, who developed intestinal amyloidosis, during late-stage TA. After years of outpatient management, she developed sudden severe dyspnea with pulmonary effusion, requiring hospitalization. After this event, betamethasone was replaced by methotrexate (MTX) for the next 34 months, but it seemed ineffective. After 1.5 years, she developed intractable diarrhea, followed by increases in BUN and serum creatinine (Cr), requiring several courses of hemodialysis. Colonoscopy revealed the presence of amyloid in her intestine, although she died of complicated sepsis caused by MRSA infection. This may be the first paper describing intestinal amyloidosis in a TA patient. Additionally, her case is rare in that she lived more than 30 years after the onset and diagnosis of TA.
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PMID:An elderly female who survived more than 30 years following a diagnosis of Takayasu's arteritis, complicated by fatal intestinal amyloidosis. 1629 68

Combined liver kidney transplantation (LKT) has the potential to provide a complete recovery of liver and kidney failure; the literature reports an increase in LKT in the last few years and an improvement in patient and graft survival. In our experience 15 patients underwent LKT from 1997 to 2005. The mean age was 50 +/- 9 years (range 34 to 63). The patients were affected by viral (n = 9), alcoholic (n = 1), polycystic (n = 2), cholangitis (n = 1), cholestatic (n = 1), or amyloidotic (n = 1) chronic hepatopathy. Chronic renal failure (CRF) was due to polycystic kidney disease (n = 4), IgA (n = 2), interstitial nephropathy (n = 2), glomerulonephritis (n = 4), amyloidosis (n = 1), vascular nephropathy (n = 1), of unknown end-stage renal disease (n = 1). Twelve of 15 patients were on renal dialysis treatment, three patients had moderate/severe CRF. Two patients had previously been transplanted (kidney). All patients were selected based upon blood group identity and negative cross-match before kidney transplant. Histocompatibility matching (HLA) was not included in the selection criteria. We did not observe delayed graft function. After a mean follow-up was 23 +/- 32 months (range 5 to 99), 12 subjects show, normal hepatic and renal function. At the beginning of our experience two patients in bad clinical condition died within 3 months because of sepsis, and one died because of a malignancy after 7 years. Both organs were functioning well in the deceased patients. Survival analysis confirms LKT efficacy: at 5 years follow-up patient survival is 86%, graft survival censored for death 100%. Only two subjects had an acute rejection episode in the first year; the kidney rejection incidence was lower than that reported for an isolated kidney transplant (13% vs 21%).
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PMID:Combined liver-kidney transplantation--S. Orsola experience: nephrological aspects. 1675 83

The role of secondary amyloidosis in determining the prognosis of dialyzed patients with rheumatoid arthritis (RA) was examined in 22 patients with a mean age of 60.1 years included 21 renal amyloidosis. RA duration until the start of dialysis was 19.5 +/- 7.2 years and the observation period after introduction 27.1 +/- 26.4 months. Of the 14 dead cases, four died due to sepsis, three due to gastrointestinal tract bleeding, two due to congestive heart failure, and eight cases died within 5 months after starting dialysis. When comparing the eight survivors and the nine non-survivors who died within 2 years after the start of dialysis, the former patients showed significantly higher serum albumin, and lower electrocardiogram score and cardiothoracic ratios at the time of introduction to dialysis. The careful prevention and treatment of infection, cerebrovascular and/or gastrointestinal tract complications seem to be necessary to improve the prognosis of RA patients after the initiation of renal replacement therapy.
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PMID:Role of amyloidosis in determining the prognosis of dialyzed patients with rheumatoid arthritis. 1701 9

B-Type natriuretic peptide (BNP) is elevated in states of increased ventricular wall stress. BNP is most commonly used to rule out congestive heart failure (CHF) in dyspneic patients. BNP levels are influenced by age, gender and, to a surprisingly large extent, by body mass index (BMI). In addition, it can be elevated in a wide variety of clinical settings with or without CHF. BNP is elevated in other cardiac disease states such as the acute coronary syndromes, diastolic dysfunction, atrial fibrillation (AF), amyloidosis, restrictive cardiomyopathy (RCM), and valvular heart disease. BNP is elevated in non-cardiac diseases such as pulmonary hypertension, chronic obstructive pulmonary disease, pulmonary embolism, and renal failure. BNP is also elevated in the setting of critical illness such as in acute decompensated CHF (ADHF) and sepsis. This variation across clinical settings has significant implications given the increasing frequency with which BNP testing is being performed. It is important for clinicians to understand how to appropriately interpret BNP in light of the comorbidities of individual patients to maximize its clinical utility. We will review the molecular biology and physiology of natriuretic peptides as well as the relevant literature on the utilization of BNP in CHF as well as in other important clinical situations, conditions that are commonly associated with CHF and or dyspnea.
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PMID:Interpretation of B-type natriuretic peptide in cardiac disease and other comorbid conditions. 1734 60

A 30-year-old HIV-infected intravenous drug user presented with sepsis, acute renal failure, oedema, proteinuria and iron deficiency anaemia. After extensive investigation, a diagnosis of reactive systemic AA (amyloid, serum amyloid A protein) amyloidosis was made on the basis of renal, gastric and duodenal biopsies.
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PMID:Renal and gastrointestinal amyloidosis in an HIV-infected injection drug user. 1752 3

We report a case of a 68-year-old man who had dialysis-related amyloidosis. The patient underwent neck surgery due to spondyloarthritis. Unfortunately he died after the surgery in sepsis. The preoperative routine echocardiography didn't suggest cardiac involvement of amyloidosis. However, the pathological findings in autopsy revealed severe amyloid depositions of both atria in contrast to the quite mild involvement of ventricles. We have experienced that the cardiac involvement of dialysis-related amyloidosis may be confined to atria almost sparing ventricles. Therefore, in patients with hemodialysis-related amyloidosis, even if echocardiogram doesn't suggest any ventricular amyloidosis, physicians should consider the possibility of atrial amyloidosis and should check the detail of atrial findings. If the A wave in the mitral filling pattern seems small or absent, physicians have to proceed transesophageal echocardiogram to detect intra atrial thrombus and consider anti-coagulation therapy to prevent thromboembolic complications of atrial amyloidosis.
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PMID:A case of almost-exclusively atria-dominant cardiac amyloidosis related with hemodialysis. 1758 86

Secondary amyloidosis (AA amyloidosis) has rarely been described in patients with systemic lupus erythematosus (SLE). We, herein, present a 56-year-old female patient, who developed AA amyloidosis following a 22-year history of SLE. She developed severe mitral regurgitation complicated with chordae tendinea rupture that led to acute congestive heart failure and went on a mitral valve replacement, where no flare symptoms of SLE were present. Three months after the operation, she presented with a nephrotic-range proteinuria, acute renal failure, and severe sepsis. She was found to have new vegetations on replaced valve and multi-organ failure caused her death. Re-evaluation of the excised mitral valve revealed AA amyloid deposition. Post-mortem biopsies from the kidney and bone marrow also revealed secondary amyloidosis.
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PMID:AA amyloidosis associated with systemic lupus erythematosus: impact on clinical course and outcome. 1768 56

The assessment of systemic inflammation by means of laboratory tests often complements the results of medical examination. Traditionally, the erythrocyte sedimentation rate and leukocytosis with left shift are diagnostic markers for inflammatory and infectious diseases. The levels of acute-phase proteins--especially C-reactive protein--are used to assess both the presence of inflammation and any response to treatment. The determination of C-reactive protein levels may be advised in three types of pathological situation: infection, acute or chronic inflammation, and evaluation of metabolic risk. Procalcitonin is useful as a marker of sepsis and severe infection. The concentration of serum amyloid A predicts the chances of survival of patients with secondary (AA) amyloidosis. Ferritin and its glycosylated form are of interest in the study of specific diseases such as adult-onset Still's disease. Markers of cartilage and bone turnover are complementary to these markers of inflammation. Although cytokine serum levels are transiently crucial to the generation of inflammation, their usefulness in the clinic is still under investigation. Serum concentrations of cytokine inhibitors or soluble cytokine receptors, as well as the clinical response of patients to treatment with cytokine antagonists, might generate important information for monitoring autoinflammatory diseases.
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PMID:Primer: the practical use of biological markers of rheumatic and systemic inflammatory diseases. 1776 50


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