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)

An unselected population of 8,954 children, age 8 to 15 years, was screened for hematuria. Four urine specimens from each were examined; microscopic hematuria was found in one or more specimens in 4.1%, and in two or more specimens in 1.1% of the children. The prevalence was not age or sex dependent. Those with two or more positive samples were re-examined twice during a half-year period: 33 had hematuria of 6 or more RBC/0.9 mm3, or more than 100,000 RBC/hour, on both occasions; renal biopsy performed on 28 of them revealed two cases of IgA-IgG nephropathy, one of focal segmental sclerosis, one of extracapillary glomerulonephritis, and one of possible hereditary nephritis. In 12 patients the biopsy was entirely normal; the rest showed equivocal changes. Co-existing proteinuria and the degree of hematuria correlated well with the severity of the morphologic alterations. Pathologic findings in microscopic hematuria seem to be less frequent than in hematuria in general; in most such patients, renal biopsy is probably not indicated. In some children the low-grade hematuria may merely represent the upper end of physiologic variation.
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PMID:Microscopic hematuria in school children: epidemiology and clinicopathologic evaluation. 49 Feb 33

Hereditary nephritis is a disease presenting with hematuria, proteinuria, and systemic findings including ocular lesions and deafness. Renal failure frequently occurs in males who have the disease at a young age. We report the case of a female with hereditary nephritis who presented with the classic clinical and pathologic features of crescentic glomerulonephritis post-pregnancy. A sibling also had a comparable course. It is proposed that crescentic glomerulonephritis may be a heretofore unreported presentation of hereditary nephritis with terminal renal failure.
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PMID:Alport's syndrome representing as crescentic glomerulonephritis: a report of two siblings. 72 17

Hematuria is the presence of more than 5 RBC's in repeated urinary sediments. Erythrocyturia may be present as an isolated finding or it may be associated to other clinical findings that may lead to the etiology of the hematuria. Its origin may be renal or extrarenal. In the neonate, meatal or urethral bleeding, polycystic kidney or hydronephrosis must be considered. In the infant, hematuria may be due to vascular disease, renal vein thrombosis, as well as to urinary tract infection, urinary tract obstruction or acute tubular interstitial nephritis due to drug ingestion. Primary and secondary glomerulopathies, urinary tract infection and urolithiasis are the most frequent causes of hematuria in pre-school or school-age children. The diagnostic approach emphasizes the importance of the clinical history, familial background and the circumstances of presentation. RBC casts and proteinuria may suggest the presence of a glomerulopathy. Leukocyturia is more frequent in urinary tract infections and requires urine cultures and intravenous pyelogram. In cases of isolated hematuria, blood clotting test, P. T., P.T.T., platelet count and RBC's morphology may be required to rule out hematological disorders. The intravenous pyelogram, voiding cystogram, and occasionally cystoscopy will help to rule out urological abnormalities. If the previous results were negative, the renal biopsy will help to distinguish IgA mesangiopathy, Alport's syndrome or essential hematuria; this last diagnosis resulting by exclusion.
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PMID:[Diagnostic significance of hematuria in pediatrics]. 75 4

The selectivity of proteinuria has been determined immunochemically at least 4 times over periods of 3 years or more in 27 children and adolescents who had been investigated by renal biopsy. Variations of the selectivity outside the limits of experimental error were observed in 14 patients, in 8 of whom there was a progressive decline. Six of these 8 had focal and segmental glomerular lesions, including one case of Alport's syndrome, and 2 had proliferative glomerulonephritis. Two different anomalies of relative IgG clearance were noted: in proliferative glomerulonephritis there was a constantly low clearance, and in focal glomerulosclerosis an elevated clearance increasing with time. Indirect evidence suggests that the latter may be due to the presence of low molecular weight IgG fragments in serum and urine.
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PMID:The significance of variation in the selectivity of proteinuria. 80 62

Alport's syndrome is a hereditary disease striking cochlea, eye and kidney. The diseased women usually have a nonlethal degree of the kidney disease, but the prognosis for the men is worse. They often die from renal failure before the age of 35. Most cases of hearing loss occurs in men. The hearing loss is progressive after the age of 10. Audiological tests are characteristic for a cochlear lesion. The debut of the disease usually appears in the post natal period giving microscopic haematuria. It can, however, occur later with haematuria and proteinuria. The hearing loss may be the first symptom, which is an observandum to otologists. Two families with Alport's syndrome are described. The patients have been examined concerning kidney disease, audiological and vestibular pathology. The hereditary pattern is described.
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PMID:Two families with Alport's syndrome. 98 81

The syndrome of hereditary thrombocytopenia, deafness, and renal disease was manifest in at least eight members in three generations of a family. They had a lifelong history of bleeding, usually as epistaxis, bilateral sensorineural deafness starting in late childhood or the teenage years, and persistent proteinuria with varying degrees of renal dysfunction. Two members died at a young age, one from central nervous system hemorrhage, the other from chronic renal failure. Splenectomy and steroid therapy have been of transient benefit. There was dominant inheritance of the syndrome. Hematologic studies showed thrombocytopenia, large platelets, and megakaryocytic hyperplasia of the bone marrow. In contrast to a previous report, our studies showed that affected members had normal in-vitro platelet function and normal ultrastructural platelet morphology. At autopsy, histologic changes in the kidney of one affected family member were indistinguishable from those reported in classic hereditary nephritis with nerve deafness (Alport's syndrome).
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PMID:Hereditary thrombocytopenia, deafness, and renal disease. 113 59

Nine hundred and forty children with hematuria were admitted to the nephrology service from 1958 to 1973. Percutaneous renal biopsies were performed in all of them. Thirty cases out of this group had recurrent hematuria and constitute the clinical material of this study. The clinical picture was: acute nephrotic syndrome in 19; monosymptomatic hematuria in 8, anaphylactoid purpura nephritis in 2, and hematuria associated with nephrotic syndrome in one patient. All patients with nephritic syndrome showed in their biopsies various types of glomerular lesions; most of the patients with monosymptomatic hematuria had normal glomeruli; at the light microscopy, the immunofluorescence was positive in some of them. Both patients with anaphylactoid nephritis showed diffuse endocapillary and focal extracapillary proliferation and in the only one with nephrotic syndrome, the hematuria was familial and the biopsy showed features of Alport's syndrome. Twenty-four patients who were followed for over two years showed no relationship between the age of onset, sex, initial significant proteinuria, hypertension, frequency of bouts of hematuria and the clinical evolution. At the end of the study, 7 patients had prolonged remission: the light microscopy showed normal glomeruli, endocapillary proliferation and endo and extracapillary proliferation with less than 30% of the glomeruli affected by "crescents". The remaining cases were still active and one of them with endo and extracapillary glomerulonephritis with more than 30% of the glomeruli affected by "crescents", developed chronic renal insufficiency. In conclusion, the prognosis of recurrent hematuria of glomerular origen is related with the type of glomerular lesions and constitutes an indication for renal biopsy. Renal specimens must be studied under light microscopy and immunofluorescence techniques; electromicroscopy is required when hematuria is present in more than one member of the family.
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PMID:[Recurrent hematuria of glomerular origin]. 127 66

Seventy-three members of a 100-member kindred with asymptomatic proteinuria, nephrotic syndrome, and progressive renal failure were studied. Of those studied, 11 members had progressed to end-stage renal disease and seven had significant proteinuria (greater than 1 g/24 hours) with normal renal function. The genetic mode of inheritance was autosomal dominant with variable penetrance and expressivity. Histopathologic changes were variable but included focal segmental glomerulosclerosis and diffuse glomerulosclerosis. Renal failure usually occurred in the fifth decade of life. The most consistent clinical finding was proteinuria without microscopic hematuria or other significant urinary sediment elements. This disease differed from Alport's hereditary nephritis and congenital nephrotic syndrome in age of onset, urinary findings, and associated conditions, that is, nerve deafness. The hereditary proteinuria and nephrotic syndrome described in this kindred represents another facet in the spectrum of hereditary renal disease.
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PMID:Familial glomerular disease with asymptomatic proteinuria and nephrotic syndrome: a new clinical entity. 142 47

62 children (20 girls and 42 boys, ranging in age between 3 and 15 years), presenting with acute hypocomplementemic glomerulonephritis or morphologically confirmed endotheliomesangial glomerulonephritis, were admitted to the University Children's Hospital, Berne from 1970 to 1991. The annual incidence of cases of acute hypocomplementemic glomerulonephritis was stable during the study period. The site of the antecedent infection was the throat in 26 patients, upper respiratory tract in 15, the skin in 9, and unknown in 10. The latent period ranged from 0.5 to 3.5 weeks. 41 patients developed hypertension and 17 renal failure. Hypertensive complications were observed in 6 patients and remitted completely in 5 cases. A nephrotic syndrome (edema, proteinuria of 40 mg/[m2.h], albuminemia < 25 g/l) was observed in 11 patients. Microscopic hematuria persisted in many patients for one year or more. Proteinuria remitted in all but one patient, who was found to have Alport syndrome. This study shows the stable frequency of hypocomplementemic glomerulonephritis since 1970, its good prognosis, and the importance of the measurement of C3-complementemia in children presenting with acute glomerulonephritis.
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PMID:[Glomerulonephritis with transient C3 hypoclompimentemia and endotheliomesangial glomerulonephritis in childhood. A long-term experience]. 144 87

Anti-glomerular basement membrane (anti-GBM) nephritis occurring in kidneys transplanted in patients with Alport's syndrome (AS) has been reported repeatedly. Therefore, we studied graft survival and course of renal function in all 30 AS patients grafted at Hannover Medical School and compared them with non-diabetic, age and sex matched patients, transplanted on the date closest to the transplantation of the AS patient. Serum creatinine, proteinuria, urinary sediment and anti-GBM antibodies were examined in all AS patients with functioning grafts. Cases of patient or graft loss in the AS group were analyzed retrospectively. One- and five-year patient survival was 100 and 91% in AS and 89 and 78% in controls (p > 0.05, respectively). One- and five-year first graft survival was 79 and 66% in both groups. Graft histology was available in 34 biopsies obtained from 21 kidneys in 15 AS patients. Anti-GBM nephritis was not detected in any of the biopsies. No graft was lost due to anti-GBM nephritis. Anti-GBM antibodies were detectable temporarily only in one AS patient. He also had linear IgG staining in his graft GBM, but no other signs of anti-GBM nephritis. We conclude that patient survival and graft survival in AS patients following kidney transplantation is not different from non-AS patients. Allograft anti-GBM nephritis is a rare complication in patients with Alport's syndrome.
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PMID:Kidney transplantation in Alport's syndrome: long-term outcome and allograft anti-GBM nephritis. 146 59


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