1/40. Macrophagelike vacuolated renal tubular cells in the urine of a male with osmotic nephrosis associated with intravenous immunoglobulin therapy. A case report.BACKGROUND: Osmotic nephrosis is a form of renal tubular injury that has been found in patients treated with intravenous immunoglobulin (IVIG). CASE: A 46-year-old male who had two courses of chemotherapy for acute myelogenous leukemia was found to have refractory thrombocytopenia. After IVIG (Sandoglobulin 12%, Novartis) administration (1 g/kg) for five consecutive days, the patient became oliguric and eventually anuric on the fifth dose. Hemodialysis was initiated, and urine production was noted on day 2 of hospitalization. Routine cytologic examination of fresh, voided urine showed numerous macrophagelike, bland epithelial cells with abundant, multivacuolated cytoplasm. Cytokeratin immunostain revealed positivity, thus confirming the epithelial origin of these cells. CONCLUSION: To our knowledge, this is the first such case reported in the English-language cytology literature. awareness of a patient's clinical history may be helpful in avoiding an incorrect diagnosis. urine cytology may be useful in obtaining an early diagnosis of osmotic nephrosis in patients receiving high-dose IVIG therapy that may eliminate the need for a renal biopsy.- - - - - - - - - - ranking = 1keywords = nephrosis (Clic here for more details about this article) |
2/40. 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 = 1.1666668805855keywords = nephrosis, kidney (Clic here for more details about this article) |
3/40. Immediate post-transplant nephrosis in a patient with congenital nephrotic syndrome.A 19-month-old girl with congenital nephrotic syndrome of the Finnish type underwent a living-related renal transplant; 24 h after transplantation she became massively nephrotic. She did not respond to steroids, plasmapheresis, and high-dose cyclosporine. A month later, a renal biopsy showed only glomerular foot process effacement. She was treated with high-dose methylprednisolone pulses and oral cyclophosphamide. She rapidly went into complete remission with no further relapses. Graft function has been stable 2 years after transplantation.- - - - - - - - - - ranking = 0.66666666666667keywords = nephrosis (Clic here for more details about this article) |
4/40. Congenital nephrosis of the Finnish type: overview of placental pathology and literature review.Congenital nephrosis of the Finnish type (CNF) is a rare, autosomal recessive disorder of glomerular filtration that results in massive proteinuria, edema, and ascites. Although previous studies describe the classic renal lesions characterizing this disorder, there are few documenting in detail the associated placental alterations. In this context, we present a case of CNF with emphasis on the placental pathology and compare our findings to what has been previously reported in the literature. A 36-year-old G2P1 with no significant medical history developed persistently elevated amniotic fluid alpha-fetoprotein in the absence of neural tube defects. Because of a clinical suspicion of CNF, she electively terminated the pregnancy at 19 weeks. Postmortem examination revealed characteristic renal changes, confirmed by electron microscopy, as well as significant placental villous edema. Although the placenta was not enlarged, the villi appeared profoundly hydropic. Extensive cystic vacuolar change was documented in both stem villi and tertiary villi, affecting 95% of the villi present. Since the fetus was not grossly edematous, the placental findings may represent the first sign of systemic hypoproteinemia.- - - - - - - - - - ranking = 0.83333333333333keywords = nephrosis (Clic here for more details about this article) |
5/40. 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 = 1.0000000427838keywords = nephrosis, kidney (Clic here for more details about this article) |
6/40. 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.- - - - - - - - - - ranking = 1.1666666666667keywords = nephrosis (Clic here for more details about this article) |
7/40. Rare causes of elevated maternal serum alpha-fetoprotein. A report of three cases.Three rare conditions--amniotic band disruption sequence, placental chorioangioma and congenital nephrosis--were diagnosed in midtrimester because of elevated maternal serum alpha-fetoprotein. The diagnosis is important for genetic counseling and obstetric follow-up.- - - - - - - - - - ranking = 0.16666666666667keywords = nephrosis (Clic here for more details about this article) |
8/40. Autoimmune ischemic optic neuropathy associated with positive rheumatoid factor and transient nephrosis.A young woman had recurrent anterior ischemic optic neuropathy for 18 years. During a recent episode of severe papillopathy in one eye, acute glomerulonephritis, transient low serum complement levels, and a high rheumatoid factor were detected. Despite long and careful follow-up, we could not diagnose systemic lupus erythematosus or any other specific connective tissue or autoimmune disease. We suspect transient disordered immunity may have contributed to provoking acute anterior ischemic optic neuropathy concomitant with acute glomerulonephritis despite the absence of generalized connective tissue disease. An extensive search for immunologic mechanisms in some patients with presumed idiopathic anterior ischemic optic neuropathy may be warranted because immunosuppressive treatment may be beneficial in preventing recurrences.- - - - - - - - - - ranking = 0.66666666666667keywords = nephrosis (Clic here for more details about this article) |
9/40. Sliding hernia containing the ureter--a rare cause of graft hydroureteronephrosis: a case report.Obstructive uropathy following renal transplantation is frequently reported. However, ureteral obstruction due to its incorporation in a sliding hernia is a rare event. Herein, we report a case of late graft hydroureteronephrosis secondary to a sliding hernia containing the transplanted ureter. The diagnosis was confirmed with the aid of magnetic resonance urography and antegrade urography. Following hernioplasty, a decrease of serum creatinine level was achieved with significant decompression of the system.- - - - - - - - - - ranking = 0.83333333333333keywords = nephrosis (Clic here for more details about this article) |
10/40. Congenital nephrosis, mesangial sclerosis, and distinct eye abnormalities with microcoria: an autosomal recessive syndrome.We observed the occurrence of congenital nephrotic syndrome (CNS) and distinct ocular anomalies in two unrelated families. Eleven children from both families presented with a similar course of renal disease starting with nephrotic syndrome and renal failure prenatally or immediately after birth that resulted in death before the age of 2 months. kidney histopathology showed diffuse mesangial sclerosis (DMS). Clinically obvious eye abnormalities were recognized in six of the eight patients in whom sufficient clinical data were available. Ocular anomalies included enlarged or large appearing corneae in some cases suggesting buphthalmos, and extremely narrow, nonreactive pupils (microcoria). Pathological examination of the eyes of two aborted fetuses revealed a more complex ocular maldevelopment including posterior lenticonus as well as anomalies of cornea and retina. On the basis of these observations and other cases in the literature, we delineate a previously unrecognized distinct entity characterized by congenital nephrotic syndrome, DMS, and eye abnormalities with microcoria as the leading clinical feature. Pedigrees of affected families with parental consanguinity support autosomal recessive inheritance. We propose that this syndrome should be designated microcoria-congenital nephrosis syndrome or Pierson syndrome. Possible overlap with Galloway-Mowat syndrome and relations to other oculo-renal syndromes are discussed.- - - - - - - - - - ranking = 0.83333333333333keywords = nephrosis (Clic here for more details about this article) |
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