1/134. alpha-Interferon therapy for HBV-related glomerulonephritis.We report a case of a patient with hepatitis b virus (HBV)-related membranous glomerulonephritis (MGN) who showed improvement after interferon-alpha (IFN-alpha) therapy. A 35-year-old man with nephrotic syndrome and HBV antigens received a 24-week course of IFN-alpha. At the end of therapy there was an elevation in the level of plasma aminotransferase and an increase in proteinuria, which were followed by antigen/antibody seroconversion. This "flare-up" before seroconversion suggests an increase in disease activity in the liver and kidney, demonstrating in vivo HBV involvement in MGN.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
2/134. Possible induction of renal dysfunction in patients with lecithin:cholesterol acyltransferase deficiency by oxidized phosphatidylcholine in glomeruli.To clarify the causes of renal dysfunction in familial lecithin:cholesterol acyltransferase (LCAT) deficiency, kidney samples from 4 patients with LCAT deficiency (3 homozygotes and 1 heterozygote) were examined immunohistochemically. All of the patients exhibited corneal opacities, anemia, renal dysfunction, deficiencies in plasma high density lipoprotein and LCAT activity and mass, and an increase in the ratio of plasma unesterified cholesterol to esterified cholesterol. Renal lesions began with the deposition of lipidlike structures in the glomerular basement membrane, and these structures accumulated in the mesangium and capillary subendothelium. By electron microscopy, 2 types of distinctive structure were found in glomerular lesions: vacuole structures and cross-striated, membranelike structures. The plasma oxidized phosphatidylcholine (oxPC) -modified low density lipoprotein (LDL) levels in LCAT-deficient subjects were significantly (P<0.01) higher than those in controls (1.30 /-0.82 versus 0.42 /-0.32 ng/5 microg LDL, respectively), and a significant (P<0.01) difference was observed even after adjustment for confounding factors by an analysis of covariance. The patient with the highest plasma oxPC-modified LDL had the most membranelike structures in the glomeruli and showed the greatest renal deterioration from a young age. In glomerular lesions, although there was an abundance of apoB and apoE, oil red O-positive lipids, macrophages, apoA1, and malondialdehyde were scarce. OxPC was found extracellularly in glomerular lesions, and although its distribution differed from that of apolipoproteins, it was quite similar to that of phospholipids. In conclusion, these results indicate that oxPC in plasma and glomeruli is distinctive for patients with LCAT deficiency. Therefore, oxPC may be a factor in the deterioration of kidneys in patients with familial LCAT deficiency.- - - - - - - - - - ranking = 2keywords = kidney (Clic here for more details about this article) |
3/134. frasier syndrome: a cause of focal segmental glomerulosclerosis in a 46,XX female.The description of frasier syndrome until now has been restricted to XY females with gonadal dysgenesis, progressive glomerulopathy, and a significant risk of gonadoblastoma. Mutations in the donor splice site in intron 9 of the Wilms' tumor (WT1) gene have been shown to cause frasier syndrome and are distinct from WT1 exon mutations associated with denys-drash syndrome. The WT1 gene, which is essential for normal kidney and gonadal development, encodes a zinc finger transcription factor. The intron 9 alternative splice donor site mutation seen in frasier syndrome leads to loss of three amino acids ( KTS isoform), thus disrupting the normal ratio of the KTS/-KTS isoforms critical for proper gonadal and renal development. This study examines two sisters with identical intron 9 mutations. The proband carries a classic diagnosis of frasier syndrome with 46,XY gonadal dysgenesis, whereas her sister has progressive glomerulopathy but a 46,XX karyotype and normal female development. This indicates that the proper WT1 isoform ratio is critical for renal and testicular development, but apparently does not affect either ovarian development or function. It is proposed that the clinical definition of frasier syndrome should be broadened to include 46,XX females with normal genital development and focal segmental glomerulosclerosis associated with a WT1 intron 9 donor splice site mutation. Nephrologists need to consider the possibility of this heritable syndrome in evaluation of females with focal segmental glomerulosclerosis and to consider their risk for gonadal malignancy, as well as the risk for kidney disease, gonadal dysgenesis, and malignancy in their offspring.- - - - - - - - - - ranking = 2keywords = kidney (Clic here for more details about this article) |
4/134. Botryoid wilms tumor: case report and review of literature.A rare case of botryoid wilms tumor is presented. The main clinical manifestations were persistent low-grade fever, malaise, and proteinuria associated with microhematuria. ultrasonography revealed an echogenic mass in the right kidney, and a contrast-enhanced mass was found in the dilated collecting system by contrast-enhanced computed tomography. The surgically resected tumor was a polypoid, light-yellow, glistening mass that occupied a large part of the renal pelvis and originated from the pelvicaliceal wall. Part of the tumor extended to the proximal ureter, resulting in hydronephrosis in the involved kidney. No parenchymal lesion was observed. Microscopic examination revealed epithelial, stromal, and blastemal components, which indicated wilms tumor. infection had occurred in the hydronephrotic kidney, which presumably had caused the major presenting symptoms. The prognosis of our patient and previously reported cases of botryoid wilms tumor was good compared with that of typical wilms tumor, since the botryoid type can be detected at an early stage.- - - - - - - - - - ranking = 283.78501625316keywords = nephrosis, kidney (Clic here for more details about this article) |
5/134. bartter syndrome and focal segmental glomerulosclerosis: a possible link between two diseases.We describe a patient with signs and symptoms of classic bartter syndrome. The patient tested negative for all known genetic abnormalities associated with this tubular disorder. proteinuria was found within 1 year after the diagnosis of bartter syndrome. A renal biopsy performed 6 months later, when her kidney function was normal, revealed focal segmental glomerulosclerosis (FSGS). We propose a link between stimulation of the renin-angiotensin system and sclerotic changes in the glomerulus. This lesion may explain previous reports of kidney failure in patients with bartter syndrome.- - - - - - - - - - ranking = 2keywords = kidney (Clic here for more details about this article) |
6/134. Early development of the renal lesions in infantile cystinosis.To identify the early renal lesions in cystinosis, including whether the "swan neck" deformity of the proximal tubule is a congenital or an acquired lesion, we performed renal function tests and kidney biopsies on two cystinotic infants, on one at 5 and 14 months and on the other at 6 and 12 months of age. The "swan neck" deformity appears to be an acquired lesion for two reasons. First, the characteristic thin neck of the proximal tubule was not demonstrated by nephron microdissection or light microscopy until after 6 months of life. Second, electron microscopy revealed that prior to the development of the lesion, the tubular cells in the neck region of the proximal tubule were undergoing degenerative changes. Renal function tests indicated that the manifestations of the fanconi syndrome correlated with the stages of development of the "swan neck" lesion. Minute crystalline spaces having some of the characteristics of lysosomal cystine crystals appeared in the early biopsies only in that portion of the proximal tubule which was undergoing atrophy to form the "swan neck" lesion observed in the later biopsies. These findings provide evidence of at least a temporal relationship between apparent cellular cystine accumulation and the development of the "swan neck" lesion and the fanconi syndrome.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
7/134. Unbalanced 4;6 translocation and progressive renal disease.Two sibs are described with an unbalanced 4;6 translocation resulting in partial trisomy 6p and monosomy for distal 4p. growth retardation, psychomotor retardation, and characteristic facial appearance are present. The facial anomalies include high prominent forehead, blepharoptosis, blepharophimosis, high nasal bridge, bulbous nose, long philtrum, small mouth with thin lips, and low-set ears. Both children have small kidneys and have had proteinuria since early childhood. The older boy developed progressive renal disease including hypertension and renal failure necessitating renal transplantation at age 18 years. Renal biopsy of the younger girl also indicates significant renal involvement. Progressive renal disease is likely an important part of the trisomy 6p phenotype.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
8/134. Potential therapeutic effect of simvastatin on progressive renal failure and nephrotic-range proteinuria caused by renal cholesterol embolism.We report our experience with a 62-year-old Japanese man with cholesterol crystal embolism after angiographic procedures performed because of intermittent claudication. In addition to progressive renal failure and nephrotic-range proteinuria, cutaneous ischemia, consisting of livedo reticularis in the lower limbs and digital necrosis at the tip of the right toe, and fundoscopic findings showing several white spots in the branches of retinal artery were also observed. Progressive renal failure and nephrotic-range proteinuria were halted just after treatment with simvastatin. Thus, simvastatin can exert a beneficial therapeutic effect on renal cholesterol embolism.- - - - - - - - - - ranking = 0.0068424698668133keywords = necrosis (Clic here for more details about this article) |
9/134. The correlation of serum carbohydrate antigen 19-9 with benign hydronephrosis.PURPOSE: High serum carbohydrate antigen 19-9 in patients with hydronephrosis but without malignant disease is reportedly rare but to our knowledge the clinical features of hydronephrosis that affect this level have not yet been clarified. We examined the correlation of serum carbohydrate antigen 19-9 with hydronephrosis status in patients with benign hydronephrosis. MATERIALS AND methods: We used 123 serum samples from 68 patients with and 55 without hydronephrosis. All patients enrolled in this study had no malignant disease. serum carbohydrate antigen 19-9 was measured by immunoradiometric assay and that level was correlated with clinical factors. RESULTS: serum carbohydrate antigen 19-9 in patients with hydronephrosis was significantly higher than in those without hydronephrosis (p <0.0001). The serum level was elevated to greater than 37 units per ml. in 25% of the patients with but in only 1.8% of those without hydronephrosis. In the hydronephrosis group the clinical features that significantly correlated with the increased serum level were bilateral hydronephrosis, urinary tract infection, proteinuria, increased serum blood urea nitrogen, severe urinary tract occlusion and high grade hydronephrosis. CONCLUSIONS: serum carbohydrate antigen 19-9 was significantly elevated in patients with benign hydronephrosis. hydronephrosis causes false-positive results when screening for malignant disease by serum carbohydrate antigen 19-9 measurement.- - - - - - - - - - ranking = 4773.3452763037keywords = nephrosis (Clic here for more details about this article) |
10/134. Remission of nephrotic syndrome due to AA amyloidosis and initiation of glomerular repair after surgical resection of localized Castleman's disease.To shed further light on the eventual destiny of amyloid kidney deposits after interruption of amylogenic stimulus, we report a case of a 47-year-old woman with nephrotic syndrome due to renal amyloidosis, complicating abdominal Castleman's disease. After 5 courses of therapy with melphalan and prednisolone which failed to improve the nephrotic syndrome or her general clinical condition, and 1 year after the diagnosis of renal amyloidosis, surgical excision of the abdominal mass was performed. Whereas her clinical symptoms and other laboratory findings rapidly improved, the proteinuria took 18 months to disappear. A second renal biopsy, performed 30 months after surgical resection, showed persistence of the amyloid deposits in the same extent. However, electron microscopy revealed subtle reparative phenomena at the epithelial site of the basement membrane. We conclude that proteinuria associated with amyloidosis does not only depend on structural damage and that the new synthesized segment of basement membrane observed by us probably represents a mechanism of repair and the start of a long healing process.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
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