oxalates and Nephritis--Hereditary

Recurrence of the original disease in the transplanted kidney is observed in 5.6%-9.3% of the patients. However, the clinical significance of recurrence is often minor. Diagnosis is easy in diseases with specific renal lesions, e.g., in dense deposit disease and IgA-nephropathy, but may be difficult if such a marker is missing. Recurrence is of special clinical importance in the following conditions: Membranoproliferative GN type I (in 33%, often severe) and type II (= dense deposit disease, recurrence in 90%, often minor), focal segmental glomerulosclerosis (in 48% of patients with a rapid course (less than 3 years) and in 12% of patients with a longer duration of the original disease; often severe), membranous nephropathy (recurrence rather rare, but often serious), and primary hyperoxaluria (in 100%). Mesangial IgA deposits recur in half of the patients with IgA-nephropathy and anaphylactoid purpura, but clinical findings are often minimal. Recurrence in anti-GBM-nephritis and SLE is rare. The study of recurrence may contribute to a better understanding of many renal diseases.

Topics: Anti-Glomerular Basement Membrane Disease; Glomerulonephritis; Glomerulosclerosis, Focal Segmental; Humans; IgA Vasculitis; Kidney Diseases; Kidney Transplantation; Lupus Erythematosus, Systemic; Metabolism, Inborn Errors; Nephritis, Hereditary; Oxalates; Oxalic Acid; Recurrence

Topics: alpha 1-Antitrypsin Deficiency; Amyloidosis; Bone Marrow Transplantation; Fabry Disease; Gaucher Disease; Genetic Diseases, Inborn; Gout; Granulomatous Disease, Chronic; Hemoglobinopathies; Hemophilia A; Hepatolenticular Degeneration; Humans; Immunologic Deficiency Syndromes; Kidney Transplantation; Leukodystrophy, Metachromatic; Liver Transplantation; Lymphocytes; Metabolism, Inborn Errors; Mucopolysaccharidoses; Nephritis, Hereditary; Niemann-Pick Diseases; Osteopetrosis; Oxalates; Oxalic Acid; Transplantation; Tyrosine; Uremia

Topics: Acidosis, Renal Tubular; Alkaptonuria; Cystinuria; Diabetes Insipidus; Female; Glycine; Gout; Humans; Hyperlipidemias; Hyperparathyroidism; Kidney Diseases; Lesch-Nyhan Syndrome; Male; Metabolism, Inborn Errors; Nephritis, Hereditary; Oxalates; Porphyrias; Uric Acid; Urologic Diseases; Xanthines

The authors reported ten cases of familial nephropathy (two cystinosis, three Senior and Loken syndrome, one Alport's syndrome and four Oxalosis) associated to ocular manifestations. Aetiologic diagnosis was known from ocular symptoms in five cases (all cases of cystinosis and Senior and Loken syndrome). The authors undertook this study to analyse the value of ocular manifestations in determining the right aetiologic diagnosis in familial nephropathies. The results of this study showed that ocular manifestations are helpful for aetiologic diagnosis in the first diseases. Indeed, corneal injury is synonym of cystinosis and retinitis pigmentosa is usually associated with Senior and Loken syndrome. In Alport's syndrome, ocular manifestations: antcrior lenticonus cataractous and perimacular white points only have orientation value in the diagnosis of this disease. Oxalosis ocular manifestations which consist of retinal oxalate deposits appear late and are concomitant to familial renal insufficiency. They cannot help in the diagnostic search.

Topics: Adolescent; Child; Corneal Diseases; Cystinosis; Eye Diseases; Humans; Kidney Diseases; Nephritis, Hereditary; Oxalates; Renal Insufficiency; Retinal Diseases; Retinitis Pigmentosa

Topics: Acidosis, Renal Tubular; Adult; Calcinosis; Female; Humans; Kidney Cortex Necrosis; Kidney Diseases; Kidney Medulla; Kidney Papillary Necrosis; Male; Medullary Sponge Kidney; Nephritis, Hereditary; Oxalates; Oxalic Acid; Radiography; Tuberculosis, Renal

Topics: Fabry Disease; Finland; Humans; Nephritis, Hereditary; Nephrotic Syndrome; Oxalates; Retrospective Studies; Scandinavian and Nordic Countries

Topics: Adolescent; Adult; Amyloidosis; Child; Cystinosis; Diabetic Nephropathies; Fabry Disease; Female; Gout; Humans; Kidney Diseases; Kidney Failure, Chronic; Kidney Transplantation; Male; Metabolic Diseases; Middle Aged; Nephritis; Nephritis, Hereditary; Oxalates; Renal Dialysis; Retrospective Studies; Transplantation, Homologous

The results of kidney transplantation in a variety of renal diseases have been analyzed. The diseases causing end-stage kidney failure in recipients were Alport syndrome, amyloidosis, cystinosis, diabetes mellitus, Fabry disease, familial nephritis, gout, medullary cystic disease, oxalosis, and systemic lupus erythematosus. The data indicate that renal transplantation is justifiable and parallels functional results for the more common causes of end-stage renal disease in all but Fabry disease and oxalosis. Although Fabry disease did not recur in any grafted kidney, only three patients have a functioning graft one year after transplantation. From a group of ten patients with oxalosis who received a total of 14 kidneys, only one survives. In no other metabolic disease, except one instance of primary amyloidosis, did the metabolic disease notably affect the transplant as it did in oxalosis.

Topics: Adolescent; Adult; Amyloidosis; Cystinosis; Diabetes Complications; Evaluation Studies as Topic; Fabry Disease; Female; Follow-Up Studies; Gout; Humans; International Cooperation; Kidney Diseases; Kidney Transplantation; Lupus Erythematosus, Systemic; Male; Middle Aged; Nephritis; Nephritis, Hereditary; Oxalates; Registries; Transplantation, Homologous