The Beneficial Effect of Three-month Induction Therapy with High-dose Prednisone and Mycophenolate Mofetil Followed by Maintenance Therapy in Acute Non-crescentic Nephritis Associated with Immunoglobulin A Deposition Disease in Adults.

The data available on immunoglobulin A (IgA) deposition disease indicate an inherited predisposition to the disease with autoimmune triggering. Hence, we prospectively evaluated the role of a new autoimmune regimen in the treatment of severe nephrotic or nephritic flares associated with noncrescentic nephritis in adult patients. Thirty-six patients were included, and the regimen consisted of an initial 3-month induction phase of prednisone and mycophenolate mofetil (MMF), followed by a maintenance phase of MMF alone for 21 months.

Acquired Perforating Dermatosis: A Disorder Treatable with Mycophenolate Mofetil.

Acquired perforating dermatosis (APD) is an adult skin disease characterized by an umbilicated papulonodular rash with transepidermal elimination of dermal components such as collagen and/or elastin. It is frequently associated with multiple medications and diseases such as diabetes and chronic renal failure. It is a disabling disease with severe pruritus in 83.

Long-term Rituximab Therapy in Adult Patients with Idiopathic Nephrotic Syndrome.

Patients with refractory idiopathic nephrotic syndrome (INS) are at risk of infections, renal failure, and the inherent side effects of immunosuppressive therapy. In the present study, we investigated the efficacy of yearly rituximab therapy in adult patients with this syndrome over 5-10 years. In the minimal change disease group, 14 of the 15 patients had complete remission (CR) and one had partial remission (PR).

"Recurrent Papillary Necrosis and Nephrocalcinosis Induced by Nonsteroidal Anti-Inflammatory Drugs for Gouty Arthritis Associated with Congenital Chloride-Losing Diarrhea: A Major Risk for Kidney Loss".

Congenital chloride-losing diarrhea (CCLD) is a rare genetic disorder due to autosomal recessive mutation in the SLC26A3 gene on chromosome 7. It is characterized with chronic watery diarrhea with high fecal chloride (Cl: >90 mmol/L), low potassium (K), and metabolic alkalosis with low urinary Cl and K. The overall long-term prognosis is favorable with optimal life-long salt and K supplementation.

A Self-Limited Facial Rash in a Lupus Patient: The Case of Primary Facial Raynaud's Phenomenon.

Skin is involved in 80% of systemic lupus erythematosus (SLE) and the second most affected after joint disease. Lupus-specific lesions include (a) acute ones viz. malar rash (80%), (b) subacute ones viz.

Partial Splenic Embolization for Hypersplenism Associated with Steatohepatitis in a Hemodialysis Patient.

Hypersplenism (HS) is a disorder characterized by a triad of splenomegaly, peripheral cytopenia due to premature destruction of blood cells and normocellular bone marrow. Its etiology is diverse and includes (a) primary autoimmune cytopenias, (b) secondary to congestion due to portal hypertension in cirrhosis and, other causes such asperiportal fibrosis, infections, autoimmune diseases, lymphoproliferative disorders, infiltrative diseases and hemolytic anemias. The latter diseases are common in patients with end-stage kidney disease.

Hypercalcemic crisis as a prodromal feature of pneumonia.

We describe a patient who had developed hypercalcemic crisis, with altered mental status and renal failure, one year following aggressive corticosteroid-therapy for lupus nephritis. Her disease relapsed after successful live-related kidney transplantation 11 years ago. She had normal parathyroid hormone and 25-hydroxyvitamin D yet high 1,25 dihydroxyvitamin D.

Complementary bodybuilding: A potential risk for permanent kidney disease.

We report our experience of renal disease associated with bodybuilders who had been on high-protein diet, anabolic androgenic steroids (AASs), and growth hormone (GH) for years. A total of 22 adult males who volunteered information about use of high protein diet and AAS or GH were seen over a six-year period with renal disease. Kidney biopsy revealed focal segmental glomerulosclerosis (FSGS) in eight, nephroangiosclerosis in four, chronic interstitial nephritis in three, acute interstitial nephritis in two, nephrocalcinosis with chronic interstitial nephritis in two, and single patients with membranous glomerulopathy, crescentic glomerulopathy, and sclerosing glomerulonephritis.

Biopsy of small kidneys: A safe and a useful guide to potentially treatable kidney disease.

Over the past four years, all patients with unexplained rapid progression of their renal disease were subjected to kidney biopsy, despite their small size (<9 cm), to define its etiology. Children, pregnant women, morbidly obese patients, and those with an unstable cardiovascular state, septicemia, bleeding diathesis as well as those kidney size with size <6 cm were excluded from the study. Doppler ultrasound was used to exclude renovascular/ischemic nephropathy.

Membranous glomerulopathy and massive cervical lymphadenopathy due to immunoglobulin G4-disease.

A 32-year-old male presented with acute and severe nephrotic syndrome as well as massive right cervical lymphadenopathy for <2 years. Computed tomography scan of the chest, abdomen, and pelvis did not reveal any lymphadenopathy. Histopathology and immunohistochemical testing of his lymph node biopsy showed infiltrate enriched with immunoglobulin G4 (IgG4)-positive plasma cells.

Multiple visceral venous thromboses associated with oral contraceptive use.

Peripheral venous thromboembolism (VTE) is a known complication of oral contraceptive drugs (OCs), yet its association with visceral VTE is rarely reported. We describe a 21-year-old female patient who presented with sudden left loin pain. Plain computed tomography (CT) urography did not show kidney lesion but was suspicious of left renal vein thrombosis.

Hilar and para-aortic necrotizing lymphadenopathy associated with antineutrophil cytoplasmic antibody-negative pauci-immune crescentic glomerulonephritis.

Lymph node involvement as a part of systemic vasculitis is rare. We report a case of women with rapidly progressive renal disease associated with recurrent epigastric pain, weight loss, and massive hilar as well as para-aortic lymphadenopathy. Ultrasound-guided biopsy of her scarred kidneys revealed antineutrophil cytoplasmic antibody-negative crescentic glomerulonephritis and that of lymph nodes showed severe necrotizing vasculitis.

Primary hyperoxaluria in an adult male: A rare cause of end-stage kidney disease yet potentially fatal if misdiagnosed.

Primary hyperoxaluria is an autosomal recessive disorder due to a deficiency in the activity of the peroxisomal hepatic enzyme alanine-glyoxylate aminotransferase. It is a common cause of urolithiasis and end-stage kidney disease in children contrary to the adult phenotypic presentation which is considered a mild disorder with occasional urolithiasis. In this case report, we describe a 25-year-old man who presented with advanced and irreversible kidney failure within three months following strenuous physical training in the police academy.

Wegner's granulomatosis developing for the first time in a patient eight years after starting maintenance hemodialysis.

Wegener's granulomatosis is a serious autoimmune disorder characterized by necrotizing small-vessel vasculitis. It is a multisystem disease that primarily affects the lung and kidneys. Previous studies indicated few relapses of vasculitis after hemodialysis due to uremic immunosuppression.

Refeeding syndrome in a patient with advanced kidney failure due to nephronophthisis.

Refeeding syndrome (RS) is a serious and potentially fatal disorder. It is caused by a shift of fluids, sodium, potassium, magnesium and phosphorus as well changes in the metabolism of glucose, protein, fat and vitamins following the refeeding of malnourished patients, whether enterally or parenterally. RS has rarely been reported in patients with advanced kidney disease probably due to the pre-existing hyperphosphatemia, hypermagnesemia and hyperkalemia in these patients.

Rituximab therapy for severe cutaneous leukocytoclastic angiitis refractory to corticosteroids, cellcept and cyclophosphamide.

We report our clinical experience with rituximab in the treatment of 2 patients with idiopathic cutaneous angiitis who relapsed after treatment with high-dose corticosteroids and cyclophosphamide. A 39-year-old woman and a 51-year-old man presented with ulcerating maculopapular rash in both lower limbs which relapsed 6 months after treatment with a combination of high-dose corticosteroids and cyclophosphamide. After treatment with 2 g of rituximab, the first patient has still been in clinical remission for 32 months while the second has finished 28 months.

Renal ablation using bilateral renal artery embolization for treatment of resistant nephrotic syndrome.

An 18-year-old man presented with severe nephrotic syndrome due to focal segmental glomerulosclerosis. His disease failed to remit with corticosteroids, calcineurin inhibitors, mycophenolate and rituximab. As his disease progressed with time, his anasarca became more resistant to high-dose combination diuretics and he developed multiple life-threatening bacterial infections.

Rituximab in treatment of idiopathic glomerulopathy.

The aim of our study was to assess the role of rituximab (Mabthera) in the treatment of patients with corticosteroid-resistant and calcineurin-inhibitors ± cellcept refractory idiopathic nephrotic syndrome (INS). A total of 83 patients who had required the previous treatment for a minimum of two years were included in the study. Our protocol included the use of rituximab in four-weekly slow infusions.

Chyluria associated with renal vasculitis.

Chyluria is usually associated with abnormal retrograde or collateral flow of lymph from intestinal lymphatics into lymphatics of the kidney, ureter or bladder. Chyluria has been described in patients with lymphoma, carcinoma, trauma, abscess, tuberculosis, filariasis, pregnancy and stenoses of the thoracic duct. In this case report we describe a patient who presented with chyluria associated with severe hyperlipidemia due to nephrotic syndrome induced by microscopic polyangiitis.

Hypertension in kuwait: the past, present and future.

Kuwait is a small country located on the northeastern part of the Arabian Peninsula. The most recent data on hypertension show: (a) a prevalence rate of 26.3%, (b) awareness of the disorder in only 23% of affected persons, (c) mild to moderate hypertension in 86% of subjects, (d) increased proportion of hypertensive patients at older age, (e) high prevalence in diabetics at age > 35 years and (f) high association with obesity.

A Case of Progressive Tubulo-Interstitial Nephritis due to Culture-negative Renal Tuberculosis.

This report describes a woman with progressive renal failure without proteinuria, urinary obstruction or overt systemic disease. The progressive renal disease without pelvicalyceal deformities in the left kidney was not consistent with the vesicoureteric reflux nephropathy. A needle biopsy of the left kidney showed interstitial caseating granulomata.

Granulomatous Interstitial Nephritis and Acute Renal Failure due to Renal-limited Sarcoidosis.

Renal failure secondary to granulomatous sarcoidosis without clinical features or radiological evidence of disease is rare. In this case report, we describe a 14-year old girl who developed progressive renal failure over a two-month period which was associated with weight loss and epigastric pain. Physical examination did not show any abnormality.

Combination of immunosuppressive agents in treatment of steroid-resistant minimal change disease and primary focal segmental glomerulosclerosis.

Background: Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are the most prevalent histopathological lesions in idiopathic nephrotic syndrome (INS). The latter is associated with high morbidity and mortality due to symptomatic anasarca, bacterial infections, venous and arterial thromboembolism, and potential progression to end-stage renal disease in the case of FSGS. Traditionally, most patients are treated with corticosteroids, cyclophosphamide (CTX) or calcineurin-inhibitors (C-I).