Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Significant glomerular changes occur in a substantial number of renal cadaver allotransplants. Transplant glomerulopathy and recurrent glomerulonephritis account for most of the lesions whereas the development of de novo glomerulonephritis is a rare event. Only a few cases of membranous glomerulonephritis in the graft have been documented. The four patients presented all developed heavy proteinuria of 11.5 to 14 g/day 5 months to 1 year after transplantation. Three cases of de novo membranous glomerulonephritis were transplanted because of renal failure due to chronic pyelonephritis, chronic glomerulonephritis and medullary sponge kidney. One patient has recurrent membranous glomerulonephritis. Transplant biopsy revealed only minimal glomerular changes by light microscopy in all cases. Immunofluorescence and electron microscopy demonstrated typical membranous glomerulonephritis.
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PMID:Recurrent and de novo membranous glomerulonephritis in renal cadaver allotransplants. 699 Nov 85

We report a patient with medullary sponge kidney (MSK) who presented with hematuria and nephrotic-range proteinuria. Renal biopsy revealed a diagnosis of renal AA amyloidosis. No secondary factors contributing to renal amyloidosis were demonstrated. To the best of our knowledge, this is the first reported case that demonstrates the coexistence of MSK and renal AA amyloidosis.
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PMID:Coexistence of medullary sponge kidney and renal AA amyloidosis in a patient with nephrotic range proteinuria. 2035 55

Hemihypertrophy is a clinical condition defined as an asymmetric enlargement of one side of the body. Inguinal hernias, renal cysts, cryptorchidism, ectasias of collecting tubes, medullary sponge kidney and horse-shoe kidney are examples of abnormalities associated with hemihypertrophy that have been described in the literature. We report here a 17.2-year-old patient with a left hemihypertrophy and renal agenesis with contralateral right compensatory renal hypertrophy together with normal renal function and blood pressure in the absence of proteinuria. He also presented with faint macular hyperpigmented skin lesions on his left upper arm, forehead and abdomen. To the best of our knowledge, this is the first report of renal agenesis and hemihypertrophy in the same patient. Hemihypertrophy can be seen as a component of Klippel-Trenaunay-Weber syndrome. The other components of this syndrome are varicose veins, skin naevus and arteriovenous malformations. Although renal agenesis, hemihypertrophy and hyperpigmented macular skin lesions (skin naevuses) may be incidental findings, together they may form a variant of Klippel-Trenaunay-Weber syndrome, as in our case.
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PMID:Renal agenesis in a child with ipsilateral hemihypertrophy. 2040 12

Genetic disorders of the kidney include cystic diseases, metabolic diseases and immune glomerulonephritis. Cystic diseases include autosomal dominant and recessive polycystic kidney disease (ADPKD, ARPKD, respectively). Neonates with enlarged, cystic kidneys should be evaluated for PKD. Patients with ADPKD have cysts and renal enlargement. Most patients present with hypertension, hematuria or flank pain; the most common extrarenal manifestation is polycystic liver disease. Oligohydramnios, bilaterally enlarged kidneys and decreased urine are featured in utero in ARPKD. Medullary sponge kidney is uncommon and features nephrocalcinosis, recurrent calcium stones and a history of polyuria/nocturia and/or urinary tract infections. Alport syndrome (AS) is an inherited disease of the glomerular basement membrane that is usually inherited as an X-linked dominant trait. Most patients with AS present in the first two decades of life with persistent microscopic or gross hematuria. Later, proteinuria is seen and its presence portends disease progression. Other findings may include sensorineural hearing loss and ocular abnormalities. There are various inherited tubulopathies, including Bartter syndrome, a group of renal tubular disorders that consist of two phenotypes with four genotypes. Patients usually present early in life with salt wasting, hypokalemia and metabolic alkalosis. Other features, depending on genotype, may include polyhydramnios and premature birth. Gitelman syndrome is also a salt-losing tubulopathy characterized by hypokalemic alkalosis. The majority of patients with Gitelman syndrome present during adolescence or early adulthood.
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PMID:Inherited renal diseases. 2508 62

Nephrocalcinosis (NC) is a renal disease characterized by deposition of calcium salts into the renal medulla. There are several causes, organic, iatrogenic, hereditary and sometimes related to extrarenal diseases. We studied 34 children affected by NC, 21 M and 13 F (average age at diagnosis 7.8 months), with the aim of analyzing the associated diseases, clinical manifestations, metabolic abnormalities, growth and renal function at onset and after follow-up. At onset 70% of patients were asymptomatic and diagnosis was occasional. Renal function was normal in 33 patients. The most frequent clinical symptoms were failure to thrive (9%), abdominal pain (6%), proteinuria/hematuria (7%). The associated diseases were: tubulopathies (8 pcs - tubular acidosis, Dent, Bartter and Lowe Syndromes), medullary sponge kidney, policalicosis (3 pcs ), Short bowel Syndrome (3 pcs), hyperparathyroidism, hypothyroidism (2 pcs), thalassemia (1pc), tyrosinemia (1 pc.). We recognized two forms of hypervitaminosis D. In a pc NC would be correlated with prematurity, another one with lipid necrosis. Among the metabolic abnormalities, observed in 25% of pcs, hyperoxaluria is the most frequent (47%), hypercalciuria (20%), hypercalcemia (15%). In some cases we found endocrine non pathogenic alterations: hypovitaminosis D (2 pcs) and hypoparathyroidism (6 pcs). During follow-up the growth was normal in 87% of cases and glomerular function was stable in 90% of pcs; IRC developed in 3 cases. From our analysis, it appears that the treatment of the underlying condition of NC is associated with catch-up growth and stabilization of renal function in most patients, but not with the reduction of the degree of the NC.
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PMID:[Nephrocalcinosis in children]. 2971 Apr 39