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At least 19 recordsLinked to original sources
PubMed1990

Congenital nephrosis as a cause of elevated alpha-fetoprotein.

Two cases of congenital nephrosis were detected through routine maternal serum alpha-fetoprotein (MSAFP) screening of 95,135 patients. No other cases of congenital nephrosis from this group were reported, resulting in an incidence of approximately one in 47,500 in this low-risk population. In both of these cases, similar to other reported cases of congenital nephrosis having MSAFP screening, the protein concentrations were greater than or equal to 10 multiples of the median (MOM). Therefore, in the case of an MSAFP over 10 MOM and a normal ultrasound examination, congenital nephrosis should be included in counseling regarding the possibility of undetected malformations. Furthermore, in the case of a pregnancy with elevated amniotic fluid AFP with negative acetylcholinesterase and normal ultrasound, the possibility of congenital nephrosis should be mentioned, regardless of family history or ancestry. When a pregnancy is terminated because of these biochemical findings, special and immediate attention to the fetal kidneys using electron microscopy is necessary to evaluate properly the possibility of congenital nephrosis.

Female↗
PubMed1977

[The changing meaning of the term nephrosis since Noeggerath (author's transl)].

The term nephrosis has changed its meaning considerably since it was coined by Friedrich v. Müller. At the time of Noeggerath, whose 100 anniversary just has passed, nephrosis was defined by morphological terms describing various histological alterations of the renal tubuli which were considered to be degenerative. Today nephrosis is a clinical diagnosis, and usually called nephrotic syndrome. It is characterized by heavy proteinuria and hypoalbuminemia, and can be produced by various etiological causes. In pediatrics the most frequent type is lipoid nephrosis which is characterized by minimal glomerular lesions, negative immunofluorescence and steroid responsiveness. Patients with frequent relapses of lipoid nephrosis are difficult to handle by the physician, therefore the "International Study of Kidney Diseases in Children" and the "Arbeitsgemeinschaft für Pädiatrische Nephrologie" are conducting cooperative therapeutic trials, which results are mentioned briefly.

Child↗
PubMed1991

Effects of methylprednisolone on glomerular and medullary mRNA levels for extracellular matrices in puromycin aminonucleoside nephrosis.

We examined the effects of methylprednisolone (MPSL) on type IV collagen, laminin and heparan sulfate proteoglycan (HSPG) mRNA levels in the renal glomeruli and medulla of puromycin aminonucleoside (PAN) nephrosis. mRNA levels encoding for type IV collagen and laminin increased markedly, whereas those for HSPG decreased significantly in glomeruli of PAN nephrosis. Administration of MPSL partially ameliorated the abnormal gene expression for basement membrane components. Furthermore, we showed that medullary mRNA levels for all these basement membrane components decreased with age in PAN nephrosis with or without MPSL treatment, suggesting that neither PAN nor MPSL has any effect on basement membrane component mRNA levels in the renal medulla. In contrast, mRNA levels for the interstitial collagens including alpha 1 (I) and alpha 1 (III) chains in glomeruli showed little change with or without MPSL treatment, whereas those in medulla increased significantly in PAN nephrosis when compared with the control. MPSL ameliorated the abnormal gene expression of alpha 1 (I) and alpha 1 (III) collagen in renal medulla. These results indicate that PAN affects both glomerular mRNA encoding for basement membrane components and medullary mRNA encoding for interstitial collagens, and that MPSL has marked effects on the amelioration of abnormal gene expression in both glomeruli and medulla of PAN nephrosis.

Animals↗
PubMed1978

Prenatal diagnosis of congenital nephrosis in 23 high-risk families.

The efficacy of maternal serum and amniotic fluid alpha-fetoprotein (AFP) estimation for the prenatal detection of congenital nephrosis was assessed in 23 pregnancies of couples with a previously affected child. At 15 to 18 weeks' gestation, amniotic fluid AFP concentration was elevated in seven of 23 cases, and maternal serum AFP level in five of these. Legal abortion was carried out at 18 to 19 weeks in all those cases where he amniotic fluid AFP concentration was abnormally high, and in all cases the fetus was found to be affected. The diagnosis of intrauterine congenital nephrosis was obvious by electron microscopic examination of the fetal kidney, but not by light microscopy. The child was born without congenital nephrosis in all 16 cases where amniotic fluid AFP level was normal, and in 16 of 18 cases (89%) where maternal serum AFP concentration was normal. Thus, the amniotic fluid AFP assay is more reliable and is recommended whenever congenital nephrosis is suspected on the basis of family history.

Abortion, Legal↗
PubMed1991

Studies on antinephritic effect of TJ-8014, syo-saiko-to-kyo-shyokyo-ka-ouren-bukuryou (5): Effects on puromycin aminonucleoside nephrosis and its mechanisms.

The effect of TJ-8014, a lyophilized extract of Syo-Saiko-To without Zingiberis rhizoma, annexing Coptidis rhizoma and Hoelen, on puromycin aminonucleoside (PAN) nephrosis in comparison with the effect of dipyridamole was evaluated. PAN nephrosis was induced in rats by intraperitoneal injections of PAN, once daily for 6 days. When TJ-8014 was given from the initial day of PAN treatment (just before PAN injection) at 2.0 g and 4.0 g/kg/day, p.o., it was markedly inhibited urinary protein excretion and elevation of serum cholesterol content-throughout the experimental periods. In addition, the mild adhesion of Bowman's capsule to capillary walls in glomeruli was also improved by TJ-8014 at 2.0 g and 4.0 g/kg/day, p.o. Dipyridamole was also effective in inhibiting the urinary protein excretion as well as histopathological changes. TJ-8014 at 4.0 g/kg/day, p.o. inhibited the decrease in superoxide dismutase (SOD-like), catalase and glutathione peroxidase activities in the renal cortex and SOD-like activity in the glomeruli in PAN-induced nephrosis. Dipyridamole failed to inhibit the decrease in scavenger activities. These results suggest that TJ-8014 is effective against PAN-induced nephrosis, and they suggest that the mechanisms of action of this medicine may be partly due to the enhancing activities of scavengers in the renal cortex and glomeruli.

Animals↗
PubMed1992

Cyclosporin reduces proteinuria in rats with aminonucleoside nephrosis.

The effect of cyclosporin (CS) was assessed in Sprague-Dawley rats with puromycin aminonucleoside (PA) nephrosis induced by a single intraperitoneal injection of PA. Three groups of rats were injected intraperitoneally with CS (10 mg/kg body weight) daily, beginning 1 day before PA administration, or 5 or 10 days after PA administration, for 10 days. CS significantly reduced proteinuria in rats with PA nephrosis in comparison with untreated nephrotic controls. After discontinuation of the CS treatment, proteinuria gradually increased, reaching values similar to those in control nephrotic rats. CS pretreatment did not prevent the induction of PA-induced nephrotic syndrome. Light microscopy and assessment of anionic sites in the glomerular basement membrane revealed no differences between normal rats, nephrotic controls, and CS-treated rats. These results show that CS can reduce proteinuria in PA nephrosis, but cannot ameliorate the glomerular changes.

Animals↗
PubMed1992

Congenital nephrosis: detection of index cases through maternal serum alpha-fetoprotein screening.

Congenital nephrosis is an autosomal recessive disorder with an incidence of 1 in 8000 in Finland, but it is quite rare in non-Finnish populations. In families known to be at risk, prenatal detection is possible by means of maternal serum and/or amniotic fluid alpha-fetoprotein levels. We report the antenatal diagnosis of four cases of congenital nephrosis, three of which were index cases, through maternal serum alpha-fetoprotein screening. The diagnosis was confirmed at birth in two infants. Two patients elected to terminate their pregnancies, and the diagnoses were confirmed pathologically (obliteration of foot processes on electron microscopy of fetal glomeruli) in both. In cases of elevated maternal serum alpha-fetoprotein, with unexplained and marked elevations of amniotic fluid alpha-fetoprotein and normal acetylcholinesterase levels, the diagnosis of congenital nephrosis must be considered regardless of ethnic origin.

Adult↗
PubMed1979

Renal failure owing to oxalate nephrosis after jejunoileal bypass.

Oxalate nephrosis resulted in progressive renal failure in 4 patients after jejunoileal bypass for morbid obesity. In general, increased levels of oxalates in the blood and urine of such patients result from enhanced absorption of exogenous oxalates. Urinary calculous formation is determined further by concomitant deficiency of inhibitor substances, whereas oxalate nephrosis probably occurs as a result of oxalate deposition in renal interstitium via the blood stream. Clinical manifestations of oxalate nephrosis include pain, infection, hematuria and renal failure. Routine postoperative renal function studies and early renal biopsy in suspicious cases are urged to establish early diagnosis. Continued deterioration of renal function, despite therapy with oxalate restruction and oxalate binding agents, indicates a reversal of the bypass to preserve unaffected renal substance.

Adult↗
PubMed1991

Pathogenesis of interstitial fibrosis in chronic purine aminonucleoside nephrosis.

A cellular and molecular approach was used to gain new insight into the pathogenesis of interstitial fibrosis in chronic purine aminonucleoside nephrosis (PAN) nephrosis. Thirty experimental rats (PAN rats) were given 15 mg/100 g body wt of i.p. PAN at time 0, followed by 4.3 mg/100 g body wt i.p. on days 20, 27 and 34; 25 control rats received i.p. saline at the same time intervals. All rats had a right unilateral nephrectomy within the first four days. Groups of control and PAN rats were killed at 21, 37, 52, 72 and 91 days. Renal sections were studied by immunofluorescence to quantitate interstitial macrophages, T lymphocytes and fibroblasts, and to characterize the deposition of the extracellular matrix (ECM) proteins (collagens I, III and IV, fibronectin and laminin) and the tissue inhibitor of the metalloproteinases (TIMP). Steady state concentrations of mRNA from the whole kidney for these ECM proteins, the metalloproteinases, TIMP, and transforming growth factor beta (TGF-beta 1) were quantitated by Northern blot analysis. Significant increases in the number of interstitial macrophages and T lymphocytes were found in the PAN rat groups compared to that in controls. All ECM proteins examined were quantitatively increased in the tubulo-interstitium of PAN rats. The pattern of distribution of some ECM proteins was also modified in experimental animals. TIMP was increased in the interstitium of PAN rats; at later times, TIMP was most prominent in sclerotic regions of the glomeruli and in tubular protein droplets. Northern blot analysis revealed increased steady-state mRNA levels for components of each of the ECM proteins, no change for the metalloproteinases--stromelysin or collagenase--and a marked increase for TIMP and TGF-beta 1 in PAN animals. The results of this study suggest that the diffuse interstitial fibrosis found in chronic PAN nephrosis results from both increased production of ECM proteins and decreased matrix degradation.

Albuminuria↗
PubMed1975

Zinc metabolism in aminonucleoside-induced nephrosis.

Nephrosis was induced in Sprague-Dawley rats in two separate studies by injections of aminonucleoside with sacrifice of animals on days 20 and 54, respectively. Experimental animals in both studies showed the typical findings of nephrosis, i.e., hypoalbuminemia, hypercholesterolemia, and proteinuria. Biochemical findings included hypozincemia and hyperzincuria. A significant correlation between hypozincemia and hypoalbuminemia was noted in the short-term study. Proteinuria occurred on the 10th day in the short-term study and the 15th day in the long-term study and increased quantitatively over the remaining days. The hyperzincuria and proteinuria correlated significantly in the long-term study. Measurements of tissue zinc revealed no change in testes and kidney in the short-term study. Kidney and muscle zinc were increased, testicular zinc was unchanged and femur zinc was decreased in the long-term study. Our conclusions are that in the aminonucleoside induced nephrosis of the rat: 1) hypozincemia occurs probably as a result of hypoalbuminemia, 2) the hyperzincuria is likely due to proteinuria and 3) zinc deficiency is not observed in nephrotic rats who receive ample zinc in their diet and who are observed up to 6 weeks.

Animals↗
PubMed1975

Osmotic Nephrosis Induced by Water-Soluble Triiodinated Contrast Media in Man. A Retrospective Study of 47 Cases.

Renal biopsies were performed in 211 patients which 10 days of excretory urography or renal arteriogrpahy in which diatrizoate, iothalamate or ioxithalamate had been used. In 47 renal specimens, osmotic nephrosis of the proximal tubular cells was found. Previous renal function had been normal in 10 patients, moderately impaired in 19, and severely impaired in 18. Tubular atrophy and/or necrosis was associated with histological features in 29 of 47 patients. Diffuse osmotic nephrosis was more often found in patients biopsied soon after roentgenography and also with severe renal insufficiency, but was not necessarily associated with declining renal function. The mechanism(s) by which contrast media may induce osmotic nephrosis remains unclear.

Adolescent↗
PubMed1978

Lipoid nephrosis and focal glomerulosclerosis.

Forty-one renal biopsies from 34 patients presenting over a 7-year period with the nephrotic syndrome or significant proteinuria and considered initially to be cases of lipoid nephrosis have been reviewed, and the results correlated with clinical findings. Lipoid nephrosis was confirmed in 25 of the patients (20 males, 5 females) 6 of whom showed a small proportion of completely sclerosed glomeruli. Twenty-two of these patients were in remission at the time of review. Nine of the patients (4 males, 5 females) showed features typical of focal glomerulosclerosis; only 4 of these were in remission, 4 showed continuing proteinuria, and one had died. The study emphasizes the importance and problems of distinguishing lipoid nephrosis from focal glomerulosclerosis.

Adolescent↗
PubMedDate not supplied

[Immunopathological prediction index of frequent relapses in children with lipoid nephrosis].

Forty-four renal biopsies were performed in the same number of selected patients with the diagnosis of lipoid nephrosis. Immunopathologic studies were carried out in all the biopsies. Positive immunofluorescence to immunoglobulins, complement/or fibrin was found in 21 cases (48%). A correlation was found between immunopathological findings and the number of patients with frequent relapses. More number of patients with frequent relapses were found in the group with positive immunofluorescence (p less than 0.01) than in those with negative results. No explanation was found for this situation; however it is suggested that the glomerular detection of immune proteins with lipoid nephrosis could be a useful parameter to predict frequent relapses in children with lipoid nephrosis.

Adolescent↗
PubMed1991

Ultrasonographic findings in dogs and cats with oxalate nephrosis attributed to ethylene glycol intoxication: 15 cases (1984-1988).

Renal ultrasonographic findings in 12 dogs and 3 cats determined to have oxalate nephrosis presumed to be secondary to ethylene glycol intoxication were examined. Ultrasonographic changes varied from mild to marked increases in renal cortical echogenicity. A pattern of greater than normal cortical and medullary echogenicity with persistence of areas of lesser echo intensity at the corticomedullary junction and central medullary regions was observed. This pattern, termed the halo sign, was recognized in 7 dogs and 1 cat concurrent with the development of clinical anuria. Ultrasonographic patterns in these clinical cases were similar to those observed in a previous study of dogs with experimentally induced ethylene glycol nephrosis. Ultrasonographic findings were not considered pathognomonic of ethylene glycol nephrosis. Due to the high death rate reported in the cases surveyed, detection of ultrasonographic changes was considered to warrant a guarded to poor prognosis. Because of the association of the halo sign with anuria, its detection was considered to warrant a grave prognosis.

Animals↗
PubMedDate not supplied

[Lipoid nephrosis in a child with advanced Hodgkin's lymphoma].

This is a case of an eight year old male, with Hodgkin's disease grade IV-B, with initial clinical presentation of nephrotic syndrome. The renal biopsy showed minimal change lesions. He had a complete remission of the nephrotic syndrome and Hodgkin's disease after been given prednisone and chemotherapy respectively. The remission of the nephrotic persisted 22 months and the Hodgkin's disease, 23 months. It has been established that cellular immunity is altered in Hodgkin's disease. The etiopathogenesis of lipoid nephrosis is unknown. There are clinical and experimental evidences showing cellular immunity in patients with lipoid nephrosis, suggesting a possible involvement of this mechanism in the etiopathogenesis of lipoid nephrosis. This could explain a relationship between the nephrotic syndrome (lipoid) and Hodgkin's disease. Our patient, besides Hodgkin's disease, showed clinical, biochemical and histologic changes, as well as clinical course compatible with nephrotic syndrome with minimal changes lesions.

Adolescent↗
PubMed1979

Lipoproteins in experimental nephrosis: plasma levels and composition.

Experimental nephrosis was induced in rats by administration of puromycin aminonucleoside and the levels of plasma lipoproteins were examined 7 days later and compared to controls. As determined by density ultracentrifugation, VLDL, IDL, LDL, and HDL protein levels were increased by 8, 4, 5, and 5 times, respectively. These increases were accompanied by changes in lipid and apoprotein composition. The VLDL, IDL, and LDL fractions contained less triglyceride and more phospholipid and cholesterol, while HDL lipid composition was not altered. The apoprotein composition of VLDL and IDL were not measurably altered, but LDL contained less apoE. HDL had a markedly abnormal composition characterized by an almost complete absence of apoA-IV and apoE, increased apoA-1, and decreased apoC. While increased hepatic synthesis can account for much of the observed hyperlipoproteinemia in nephrosis, the changes in lipoprotein composition suggest impaired catabolism as a contributory factor.

Animals↗
PubMed1975

An ultrastructural study of the mechanisms of proteinuria in aminonucleoside nephrosis.

Sprague-Dawley rats injected i.v. with a single dose of puromycin aminonucleoside (PAN) developed massive proteinuria five days later. Electron microscopic studies of perfusion-fixed glomeruli showed that loss of epithelial foot processes and their replacement by flattened expanses of epithelial cytoplasm began at two days and was extensive by four days after the injection of PAN. At and after five days (correlating with the onset and persistence of massive proteinuria), areas of focal loss of the epithelial covering on the outside of the glomerular basement membrane (GBM) were observed in 30% of glomeruli. Intravenously administered ferritin was distributed normally in most sections of the GBM of nephrotic animals, but abnormally deep penetration of particles was observed in GBM segments that lacked an external covering of epithelium. The same changes were found following in situ fixation of superficially placed glomeruli of Munich-Wistar rats with PAN nephrosis. We propose that the massive, early proteinuria in PAN nephrosis may be primarily due to a glomerular epithelial lesion, leading to scattered focal defects in the external covering of the GBM. Increased bulk flow of glomerular filtrate across the GBM in such areas may explain the highly selective proteinuria found in this form of the nephrotic syndrome.

Animals↗