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Renal Tubular Acidosis. Bethesda, MD: National Kidney and Urologic Diseases Information Clearinghouse. 2008. 6 p.

Renal tubular acidosis (RTA) is a disease that occurs when the kidneys fail to excrete acids into the urine, which causes a person’s blood to remain too acidic. Without proper treatment, chronic acidity of the blood leads to growth retardation, kidney stones, bone disease, and progressive renal failure. This fact sheet reviews the diagnosis, the subtypes of RTA, therapy, and current research activities in RTA. To diagnose RTA, the doctor will check the acid-base balance in samples of the patient’s blood and urine. Physicians use a three-category classification system to describe the different types of RTA. Type 1, also called classic distal RTA, is an inherited disorder associated with diseases that affect many organ systems such as the autoimmune disorders Sjögren’s syndrome and lupus erythematosus. Type 2 is called proximal RTA and occurs most frequently in children as part of a disorder called Fanconi’s syndrome; it can also occur as a side effect of treatment with ifosfamide, a drug used in chemotherapy. Type 4 is caused by another defect in the kidney tubule but is different from classic or proximal RTA because it results in high levels of potassium in the blood instead of low levels. If treated early, most people with RTA will not develop permanent kidney failure. Therefore, the goal is early recognition and adequate therapy, which will need to be maintained and monitored throughout the patient’s lifetime. The fact sheet concludes with a summary of research programs in Renal tubular acidosis (RTA) area and a brief description of the activities of the National Kidney and Urologic Diseases Information Clearinghouse (NKUDIC), a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) that provides information about diseases of the kidneys and urologic system to patients and their families, the general public, and health care professionals. Readers are referred to the National Kidney Foundation at www.kidney.org or 1–800–622–9010, the American Association of Kidney Patients at www.aakp.org or 1–800–749–2257, and the American Kidney Fund at www.kidneyfund.org or 1–800–638–8299 for more information.

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Lupus Nephritis. Bethesda, MD: National Kidney and Urologic Diseases Information Clearinghouse. 2007. 2 p.

This fact sheet describes lupus nephritis, an inflammation of the kidney caused by systemic lupus erythematosus (SLE), a disease of the immune system. Written in a question-and-answer format, the fact sheet describes the symptoms of lupus nephritis, how the condition is diagnosed, treatment options, and where patients can get more information. Lupus nephritis may cause weight gain, high blood pressure, dark urine, or swelling around the eyes, legs, ankles, or fingers. Diagnosis may require urine and blood tests as well as a kidney biopsy. Treatment depends on the symptoms and test results. Corticosteroids may be used to decrease swelling and inflammation by suppressing the immune system. Patients may need medications to help control their blood pressure and a diet limited in sodium, protein, and potassium. The fact sheet includes the contact information for the National Institute of Arthritis and Musculoskeletal and Skin Diseases, and the Lupus Foundation of America, and a brief description of the goals and activities of the National Kidney and Urologic Diseases Information Clearinghouse.

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Glomerular Diseases. Bethesda, MD: National Kidney and Urologic Diseases Information Clearinghouse. 2006. 12 p.

This fact sheet reviews glomerular diseases, which involve problems with the glomeruli, the tiny units within the kidney where blood is cleaned. The fact sheet focuses on glomerulonephritis, defined as inflammation of the membrane tissue in the kidney; and glomerulosclerosis, the scarring or hardening of the tiny blood vessels within the kidney. Written in a question-and-answer format, the fact sheet covers the anatomy and function of the kidneys, how glomerular diseases interfere with kidney function, the symptoms of glomerular disease, diagnostic tests used to confirm glomerular disease, the causes of glomerular disease, renal failure, and end-stage renal disease (ESRD). Specific diseases covered include systemic lupus erythematosus (SLE), Goodpasture's syndrome, IgA nephropathy, Alport syndrome, infection-related glomerular disease, bacterial endocarditis, diabetic nephropathy, focal segmental glomerulosclerosis, and minimal change disease (MCD). The booklet summarizes the points covered, provides a brief glossary of terms, lists resource organizations for readers seeking additional information, and a briefly describes the goals and activities of the National Kidney and Urologic Diseases Information Clearinghouse. 2 figures.

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Spectrum of Renal Diseases Associated with Extreme Forms of Insulin Resistance. Clinical Journal of the American Society of Nephrology. 1(4): 616-622. July 2006.

This review article considers the spectrum of renal diseases associated with extreme forms of insulin resistance. In patients with syndromes of extreme insulin resistance, the proteinuric forms of renal disease are common, but the authors note that the renal pathology usually is not diabetic nephropathy. For example, in the lipodystrophy syndromes, membranoproliferative glomerulonephritis type 1 and type 2, focal segmental glomerulosclerosis, and also diabetic nephropathy are seen. In the syndromes of autoantibodies to the insulin receptor, the various forms of lupus glomerulonephritis are seen. Even in patients with type 2 diabetes, the renal pathology may not be diabetic nephropathy. The authors conclude that this means that renal biopsy has an important role in defining the pathology that leads to proteinuric nephropathy; this leads to a more successful therapeutic approach. 4 figures. 4 tables. 40 references.

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Evaluation and Preparation of Renal Transplant Candidates. IN: Danovitch, G.M. Handbook of Kidney Transplantation. Philadelphia, PA: Lippincott Williams and Wilkins. 2005. pp. 169-192.

The preparation of patients with end-stage renal disease for kidney transplantation should start from the time of recognition of progressive chronic kidney disease (CKD). This chapter on the evaluation and preparation of renal transplant candidates is from a handbook that offers a practical guide for health care providers who manage kidney transplant patients. The authors note that the management of these patients consists of an initial evaluation followed, if they are appropriate transplant candidates, by their supervision while awaiting transplantation. Initial evaluation is designed not only to assess the chances of recovery from surgery, but also to increase short- and long-term patient survival. The limited number of organs available has changed the focus of the transplant evaluation toward better long-term outcome over short-term benefits. The authors first focus on the initial recipient evaluation and then consider the management of the waiting list for deceased donor transplantation. Specific topics covered include the benefits of early referral, patient education, the routine evaluation, the evaluation of specific transplant risk factors related to organ system disease (cardiovascular disease, cerebrovascular and peripheral vascular disease, malignancy, infections, gastrointestinal disease, pulmonary disease, urologic evaluation, renal osteodystrophy and metabolic bone disease, and hypercoagulable states), and risk factors related to specific patient characteristics (aged, obesity, highly sensitized patients, previously transplanted patients, and candidates for double-organ transplants). An additional section considers the relevance of the etiology of renal disease to the transplant evaluation, including diabetes mellitus, focal and segmental glomerulosclerosis, recurrent glomerulonephritis, thrombotic thrombocytopenic purpura, systemic lupus erythematosus and vasculitis, oxalosis and oxaluria, Fabry disease, Alport syndrome, sickle cell disease, amyloidosis and plasma cell dyscrasias, and polycystic kidney disease. 1 figure. 5 tables. 16 references.

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Medical and Surgical Aspects of Kidney Donation. IN: Danovitch, G.M. Handbook of Kidney Transplantation. Philadelphia, PA: Lippincott Williams and Wilkins. 2005. pp. 135-168.

The appropriate identification and preparation of kidney donors (both living and deceased) contribute critically to the success of the transplant endeavor on both the individual and the national levels. This chapter on the medical and surgical aspects of kidney donation is from a handbook that offers a practical guide for health care providers who manage kidney transplant patients. The authors divide the chapter into two sections: Part I addresses the selection and evaluation of living donors and the surgical techniques of living donor nephrectomy; Part II discusses these same issues for deceased kidney donors. Specific topics include the role of informed consent, the evaluation process, the psychosocial evaluation, risks of donation, donor age, assessment of surgical risks, the risk of disease transmission to the recipient, evaluation of future risk to the donor, assessment of renal function (glomerular filtration rate, proteinuria, hematuria, hypertension, diabetes, obesity, nephrolithiasis, inherited renal disease, autosomal dominant polycystic kidney disease, Alport syndrome, familial primary glomerulonephritis, and systemic lupus erythematosus), surgical evaluation of the living kidney donor, surgical techniques for living donor nephrectomy, long-term postnephrectomy issues (renal function, pregnancy, employment and insurance, and long-term medical care), and controversies and innovations in living kidney donor practice, including biologically unrelated donors, incompatible donor and recipient, paid donation, and the living donor registry. The second section discusses contraindications to deceased donor donation, the role of donor age, expanded criteria donors, donor biopsy, nephron dose, donation after cardiac death, diagnosis of brain death, techniques of deceased donor organ retrieval, and deceased donor kidney preservation. 2 figures. 10 tables. 32 references.

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Oculorenal Manifestations in Systematic Autoimmune Diseases. American Journal of Kidney Diseases. 43(2): 209-222. February 2004.

Vasculitides form a heterogeneous group of diseases characterized by blood-vessel inflammation and necrosis (tissue death). Systemic necrotizing vasculitis is characterized by inflammation of blood vessels, which often affects the eyes and kidneys. Vasculitides have a wide spectrum of manifestations because of the involvement of arteries and other vessels of various sizes and locations. Early diagnosis and prompt treatment may decrease the morbidity and mortality associated with systemic autoimmune diseases. In addition, the eyes and kidneys can provide clues to the diagnosis of many systemic diseases and many important complications of these diseases occur in the eye. Therefore, examination of the eyes and kidneys should be a routine and important part of a general examination in systemic diseases. This article reviews the major types of oculorenal manifestations in systemic autoimmune diseases. Diseases discussed include giant cell (temporal) arteritis, polyarteritis nodosa, Kawasaki disease, Wegener's granulomatosis and microscopic polyarteritis, Goodpasture's syndrome, IgA nephropathy and Henoch-Schonlein purpura nephritis, Churg-Strauss syndrome, Behcet's disease, systemic lupus erythematosus, primary antiphospholipid syndrome (APS), sarcoidosis, Sjogren's syndrome, cryoglobulinemia, and tubulointerstitial nephritis and uveitis syndrome. 6 figures. 124 references.

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Update in Nephrology. Annals of Internal Medicine. 140(2): 106-111. January 2004.

This article summarizes important, recent clinical advances in nephrology; the author focuses on three key topics of interest: hypertension (high blood pressure), proteinuria (protein in the urine), and renal replacement therapy (RRT, which include dialysis and transplantation). Two single studies, one in the area of nephrolithiasis (kidney stones) and the other dealing with glomerulonephritis in the form of lupus-related nephropathy, are also reviewed. The editor chose studies that provide pathophysiologic insight into a given problem or that are likely to alter clinical practice. Each study is briefly summarized, with clinical strategies highlighted. 3 references.

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Recurrent Lupus Nephritis in Renal Transplant Recipients Revisited: It Is Not Rare. Transplantation. 75(5): 651-656. March 2003.

Although recurrent lupus nephritis (RLN) after kidney transplantation is reported to be rare (1 to 4 percent), recent studies suggest a higher incidence. This article reports on a study undertaken to determine the incidence of RLN in a large cohort of renal transplant recipients with systemic lupus erythematosus (SLE). The authors reviewed the records of 54 renal (kidney) transplant recipients with SLE. Thirty-one patients underwent biopsy because of worsening renal function and proteinuria (protein in the urine). Among the 50 patients with at least 3 months of followup, RLN was present in 15 (52 percent of patients who underwent biopsy, 30 percent of total patients). One patient had graft loss because of RLN at 10.5 years. The duration of dialysis before transplantation was not different between patients with RLN compared to patients without RLN. Overall patient survival was 96 percent at 1 year and 82 percent at 5 years. The authors conclude that RLN is more common than previously reported, but in this series, graft loss because of RLN was rare. Aggressive use of allograft biopsies and morphologic evaluation are important factors in the diagnosis of RLN. 2 figures. 2 tables. 29 references.

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Recurrent Lupus Nephritis in Renal Transplant Recipients Revisited: It Is Not Rare. Transplantation. 75(5): 651-656. March 2003.

Although recurrent lupus nephritis (RLN) after kidney transplantation is reported to be rare (1 to 4 percent), recent studies suggest a higher incidence. This article reports on a study undertaken to determine the incidence of RLN in a large cohort of renal transplant recipients with systemic lupus erythematosus (SLE). The records of 54 renal transplant recipients with SLE were reviewed; 31 patients underwent biopsy because of worsening renal function and proteinuria. Among the 50 patients with at least 3 months of follow up, RLN was present in 15 (52 percent of patients who underwent biopsy, 30 percent of total patients): mesangial lupus nephritis (LN) in eight patients, focal proliferative LN in four, and membranous LN in three patients. One patient had graft loss because of RLN at 10.5 years. The duration of dialysis before transplantation was not different between patients with RLN compared to patients without RLN. Overall patient survival (n = 50) was 96 percent at 1 year and 82 percent at 5 years. Graft survival was worse in patients who underwent biopsy compared with patients who never underwent biopsy. The authors conclude that RLN is more common than previously reported, but in this series, graft loss because of RLN was rare. Aggressive use of allograft biopsies and morphological evaluation are important factors in the diagnosis of RLN. 2 figures. 2 tables. 29 references.

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