allopurinol and Nephrotic-Syndrome

Klippel-Trenaunay-Weber syndrome is a rare syndrome; unfortunately, very few studies of the connection between hypersplenism, nephrotic syndrome, and Klippel-Trenaunay-Weber syndrome have been published.. We report the case of a 40-year-old white man with a typical clinical presentation of Klippel-Trenaunay-Weber syndrome, including "port-wine stains," varicose veins, hypertrophy of lower extremities, and arteriovenous fistula, as well as an unfortunate development of hypersplenism and nephrotic syndrome.. This case report described considerable atypical relevance of Klippel-Trenaunay-Weber syndrome and hypersplenism together with nephrotic syndrome. A multidisciplinary approach was made. Unfortunately, hypersplenism is characterized by pancytopenia that suggests splenectomy, whereas nephrotic syndrome is an indication for renal biopsy; the splenectomy and renal biopsy were delayed due to our patient's severe condition. Deeper analysis including study of other patients with Klippel-Trenaunay-Weber syndrome would help us to understand the connection between elevated spleen and liver sizes, nephrotic syndrome, and Klippel-Trenaunay-Weber syndrome.

Topics: Adult; Allopurinol; Amlodipine; Angiotensin-Converting Enzyme Inhibitors; Anticholesteremic Agents; Antihypertensive Agents; Arteriovenous Fistula; Atorvastatin; Cholecalciferol; Erythrocyte Transfusion; Humans; Hypersplenism; Klippel-Trenaunay-Weber Syndrome; Male; Nephrotic Syndrome; Perindopril; Port-Wine Stain; Vitamins

We report a case of calcium pyrophosphate dihydrate deposition disease (CPDD) involving a patient on maintenance hemodialysis (MHD). The 32-year-old man presented in August 2016 with a complaint of left shoulder swelling of 8 months' duration with no trauma or fever. He was diagnosed with nephrotic syndrome in 1998, which progressed to ESRD. He commenced MHD in 2012. Examination at our hospital revealed a soft nontender swelling of the left shoulder. Blood biochemistry showed elevated serum urate, phosphate, β2 microglobulin, and parathyroid hormone. Imaging revealed joint effusion and dense heterogenous deposition. Aspirate analysis showed urate crystals 3+, and culture yielded no growth. Following rheumatology review, the working diagnosis was periarticular tissue tuberculosis, after excluding pseudogout and amyloidosis. Following 1 month of colchicine and allopurinol, synovial fluid microscopy showed CPDD crystals. Symptoms gradually resolved over the course of 6 months. In this rare case, a diagnosis of CPDD was made with a multidisciplinary approach that included imaging and biochemical investigations.

Topics: Adult; Allopurinol; Bone Diseases, Metabolic; Chondrocalcinosis; Colchicine; Humans; Kidney Failure, Chronic; Male; Nephrotic Syndrome; Renal Dialysis; Vietnam

A 12-year-old girl, who had been diagnosed as having Cockayne syndrome (CS), was admitted for emaciation and dehydration. On admission the patient had mild chronic renal failure (glomerular filtration rate: GFR 50 mL/min) and hyperuricemia. After rehydration, allopurinol was commenced for her hyperuricemia. Then, her renal function rapidly deteriorated (GFR 20 mL/min) with enhancement of proximal tubular dysfunction and hypertension. A renal biopsy showed that the patient had acute tubulointerstitial nephritis (ATIN). Based on this diagnosis, allopurinol was stopped and prednisolone was started (2 mg/kg per day), following which the renal tubular function improved. However, the proteinuria intensified to become nephrotic syndrome. After 1 month the patient developed a gastric ulcer. Famotidine was commenced but GFR deteriorated and renal proximal tubular dysfunction re-occurred. The renal pathology was evaluated by referring to the previous reports of renal pathology in CS. It is suggested that rapid deterioration of the renal function in CS patients might be the result of ATIN. In addition, the present nephrotic syndrome seemed to be accompanied by ATIN, as in other reports.

Topics: Acute Disease; Acute Kidney Injury; Allopurinol; Child; Cockayne Syndrome; Fatal Outcome; Female; Humans; Kidney Glomerulus; Microscopy, Electron, Transmission; Nephritis, Interstitial; Nephrotic Syndrome; Prednisolone; Recurrence

Topics: Aged; Allopurinol; Antineoplastic Agents; Autoantibodies; Child; Female; Glomerulonephritis; Humans; Hypersensitivity; Immunoglobulin A; Immunoglobulin G; Immunoglobulin M; Kidney; Male; Nephrotic Syndrome; Purpura; Skin

Topics: Arginase; Biopsy; Child; Child, Preschool; Chronic Disease; Female; Hepatitis; Humans; Infant; Infant, Newborn; Infections; Liver; Liver Cirrhosis; Liver Diseases; Male; Nephrotic Syndrome; Nutrition Disorders; Xanthine Oxidase