1/14. prenatal diagnosis of congenital nephrosis by in utero kidney biopsy.The diagnosis of congenital nephrosis is difficult during the antepartum period. The combination of an elevated amniotic fluid alpha-fetoprotein, a negative acetylcholinesterase, and a negative ultrasound examination is highly indicative of congenital nephrosis; however, these findings can also be associated with a normal gestation. This is the first report of pathologic confirmation of congenital nephrosis from an in utero fetal kidney biopsy.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
2/14. Muconephrosis.We report a case of a mucus-filled kidney (muconephrosis) encountered during laparoscopic nephrectomy for presumed xanthogranulomatous pyelonephritis. Conversion to open nephrectomy and en-bloc right hemicolectomy were necessary because of severe perinephric fibrosis and suspected renal-enteral fistula. Pathologic examination revealed a renal pelvic villous adenoma and diffuse intestinal metaplasia of the urothelium; no enteral communication or gastrointestinal pathologic features were found. Urothelial villous adenoma is extremely rare, and one should consider neoplastic etiologies, including appendiceal mucinous cystadenocarcinoma and mucus-secreting adenocarcinoma of the renal pelvis, in directing the appropriate management of muconephrosis.- - - - - - - - - - ranking = 0.2keywords = kidney (Clic here for more details about this article) |
3/14. 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.- - - - - - - - - - ranking = 0.2keywords = kidney (Clic here for more details about this article) |
4/14. The nephrotic syndrome associated with neoplasia: an unusual paraneoplastic syndrome. Report of a case and review of the literature.The nephrotic syndrome complicating malignancy in the absence of renal vein thrombosis, amyloid or neoplastic infiltration of the kidney is an unusual occurrence. A case of diffuse, well differentiated, lymphocytic lymphoma and lipoid nephrosis documented by light microscopy, electron microscopy and immunofluorescent studies is reported. A review of the literature revealed 76 case reports in which the nephrotic syndrome was associated with neoplasia. The most frequently associated neoplasms are Hodgkin's disease, various carcinomas, nonHodgkin's lymphoma and leukemia in descending order. The most frequent renal lesion in patients with the nephrotic syndrome associated with various carcinomas is membranous glomerulonephritis (81 per cent) as opposed to patients with lymphomas or leukemias who have predominantly lipoid nephrosis (60 per cent). The evidence is reviewed suggesting that the lesions in membranous nephropathy are immunologically mediated by tumor or viral antigen-antibody complexes and in lipoid nephrosis perhaps by a defect in t-lymphocyte function.- - - - - - - - - - ranking = 0.2keywords = kidney (Clic here for more details about this article) |
5/14. Acute human immunodeficiency virus infection temporally associated with rhabdomyolysis, acute renal failure, and nephrosis.A previously healthy 29-year-old homosexual man presented with a 4-day history of fever, malaise, sore throat, and bleeding gums. rhabdomyolysis, acute renal failure, and nephrotic range proteinuria were also present. The patient was found to have acute human immunodeficiency virus (hiv) infection confirmed by the presence of hiv antigen in his serum and subsequent evolution of an hiv antibody profile typical of acute seroconversion. A kidney biopsy revealed acute tubular necrosis and mesangioproliferative glomerulonephritis, with tubuloreticular inclusions. In the presence of otherwise unexplained acute renal failure, rhabdomyolysis, or new onset nephrotic syndrome, acute hiv infection should be considered in the differential diagnosis.- - - - - - - - - - ranking = 0.2keywords = kidney (Clic here for more details about this article) |
6/14. Nephrosis in two siblings with infantile sialic acid storage disease.The diagnosis of infantile sialic acid storage disease (ISSD) was established in two siblings on the basis of typical clinical signs and the biochemical findings of hyperexcretion and intracellular storage of free sialic acid. A severe, steroid resistant nephrosis occurred in both siblings. The activities of lysosomal enzymes, including sialidase, were normal. A combined detection method for sialic acids with Limax flavus agglutinin labelling and phosphotungstic acid staining showed severely alterated sialic acid components in epithelial kidney cells and indicate a causal relationship between the nephrosis and the underlying biochemical defect. Further observations of ISSD patients with renal involvement will prove if a separate nephropathic phenotype exists.- - - - - - - - - - ranking = 0.2keywords = kidney (Clic here for more details about this article) |
7/14. Nephrosis and disturbances of neuronal migration in male siblings--a new hereditary disorder?Two male siblings (a boy aged 2 years 10 months at death and a male fetus aborted in gestational week 22) showed similar brain and kidney malformations, comprising paraventricular heterotopias, central canal abnormalities (including hydrocephalus in the boy), and glomerular kidney disease with proteinuria. There were no known hereditary diseases in the families of the parents, and there was one healthy sibling of either sex. The malformations thus seem to be hereditary in an autosomal or possibly X linked recessive fashion.- - - - - - - - - - ranking = 0.4keywords = kidney (Clic here for more details about this article) |
8/14. Nephrosis with varicocele: probable renal vein thrombosis.A 3-year-old boy with relapsing nephrosis presented with a left sided varicocele. Echographic studies demonstrated enlargement of the left kidney, and there was delayed excretion on renal scintigraphy suggesting the presence of renal vein thrombosis. The varicocele resolved with remission of his nephrosis.- - - - - - - - - - ranking = 0.2keywords = kidney (Clic here for more details about this article) |
9/14. Spontaneous disruption of the colon associated with pyonephrosis: report of a case.We report a case of reno-colic fistula with abscess formation, which was treated by staged surgical procedures. One must be wary of automatically implicating the colon as the primary source of the problem when the colon and adjacent organs are involved in an inflammatory process. In cases involving the superior segments of the colon, the kidney or pancreas may be the source of the fistula.- - - - - - - - - - ranking = 0.2keywords = kidney (Clic here for more details about this article) |
10/14. Fetal proteinuria in diagnosis of congenital nephrosis detected by raised alpha-fetoprotein in maternal serum.High concentrations of alpha-fetoprotein (alpha-FP) were found at 14, 19, and 21 weeks gestation in the serum of a woman with a history of unexplained fetal death in her previous pregnancies. The alpha-FP concentration of the liquor also was high at 21 weeks and the pregnancy was terminated. Though the fetus was macroscopically normal, measurement of albumin, alpha-FP, IgG, and alpha2-macroglobulin in the fetal urine showed a selective proteinuria, and congenital nephrosis was diagnosed after examination of the fetal kidneys by electron microscopy. Possibly some fetuses reported to be "false-positive for neural tube defect" may have had renal lesions of this nature. Examination of fetal urine may be the simplest initial diagnostic procedure in any future case.- - - - - - - - - - ranking = 0.2keywords = kidney (Clic here for more details about this article) |
| | Next -> |