1/96. A case of renal pseudotumor associated with chronic pachymeningitis.BACKGROUND: A 56-year-old woman was referred to our hospital with a left renal mass. methods/RESULTS: Radiologic studies demonstrated a solitary space-occupying lesion in the left kidney and a malignant tumor was suspected. Left radical nephrectomy was then performed. Pathological examination revealed a sclerotic fibrous lesion with a rather distinct margin and no evidence of malignancy. These pathological findings were consistent with the diagnosis of a renal pseudotumor. CONCLUSIONS: This patient had a history of chronic pachymeningitis that formed a thoracic epidural focus causing spinal cord compression and the histologic appearance of this focus was similar to the renal lesion. It was concluded that this was a rare case of a renal pseudotumor associated with multifocal fibrosclerosis.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/96. Irreversible glomerular lesions induced by crystal precipitation in a renal transplant after foscarnet therapy for cytomegalovirus infection.AIMS: foscarnet is an antiviral agent used to treat cytomegalovirus infection in AIDS patients and in transplant recipients. In most cases, foscarnet induces reversible tubulo-interstitial lesions which can be avoided by correct hydration. We report the first case of crystal foscarnet precipitation within glomerular capillaries in a renal transplant. methods AND RESULTS: The recipient, a 49-year-old man, developed a nephrotic syndrome with haematuria and an acute renal failure after foscarnet therapy for cytomegalovirus (CMV) infection. The polarization examination of the first graft biopsy revealed the presence of birefringent crystals within glomeruli and tubules. Infrared analysis attested to the presence of trisodium foscarnet salts and mixed sodium calcium salts coloured by Von Kossa's reaction. A second biopsy showed glomerular sclerosis, interstitial fibrosis, tubular atrophy and crystal vanishing. polymerase chain reaction (PCR) in situ applied to this biopsy confirmed the diagnosis of cytomegalovirus infection. CONCLUSIONS: These adverse effects might be the result of a toxic synergy between foscarnet and other drugs. In cases with crystalline precipitation, graft biopsy remains the best mean of diagnosis and follow-up of glomerular damage.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
3/96. apolipoprotein e2/E5 variants in lipoprotein glomerulopathy recurred in transplanted kidney.Lipid abnormalities are associated with various disorders ranging from generalized atherosclerosis to renal diseases, including lipoprotein glomerulopathy that is characterized by glomerular lipoprotein thrombi and causes type III hyperlipoproteinemia, proteinuria, and renal failure. This study examines lipoprotein glomerulopathy, which recurred in a transplanted kidney. Molecular biologic analysis of the patient's apolipoprotein (apo) E gene demonstrated E2/E5 type variants. Immunohistochemical analysis of the diseased kidney demonstrated various lipid peroxidation-specific protein adducts, suggesting a potential role of oxidative stress in this disorder. recurrence in the transplanted kidney suggested a pathogenic role of extraglomerular humoral component(s) resulting from abnormal lipoprotein metabolism, presumably linked to apo E and other genetic or acquired factor(s). Furthermore, the finding that the patient showed pathologic abnormalities in the transplanted kidney with no clinical signs or symptoms of renal disease indicated that lipoprotein glomerular damage progresses early before any clinical manifestations.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
4/96. Hereditary glomerulopathy associated with a mitochondrial tRNA(Leu) gene mutation.Several cases of hereditary glomerulopathy associated with an A to G transition at position 3243 in mitochondrial dna, which is known to be associated with most cases of melas syndrome (myopathy, encephalopathy, lactic acidosis, and stroke-like episodes), have been recently reported. These patients share the characteristics of hereditary progressive glomerular disease and hearing loss with Alport syndrome. We therefore screened 27 patients with kidney disease clinically mimicking Alport syndrome for the presence of the 3243 mitochondrial mutation, and found one girl with the mutation and a positive family history. Her clinical features were very similar to those of all cases reported to date. An absence of hematuria, severe kidney involvement in a female, pathological changes of focal segmental glomerulosclerosis with no basket-weave change of the glomerular capillary wall, and the frequent association of steroid-induced diabetes are the major features that distinguish this condition from Alport syndrome. Careful neurological examination may detect neuromuscular symptoms compatible with mitochondrial cytopathies. In conclusion, progressive glomerulopathy should be included in the broad spectrum of mitochondrial cytopathies, especially in cases of melas syndrome. This mutation should also be included in the etiologies of secondary focal segmental glomerulosclerosis and in the differential diagnosis of Alport syndrome.- - - - - - - - - - ranking = 2keywords = sclerosis (Clic here for more details about this article) |
5/96. A novel 18-amino acid deletion in apolipoprotein E associated with lipoprotein glomerulopathy.BACKGROUND: Lipoprotein glomerulopathy (LPG) is a novel disease characterized by proteinuria, lipoprotein thrombi in glomeruli, and an increased concentration of plasma apolipoprotein (apo) E. Previous studies have shown that a genetic disorder of apo E may be associated with the genesis of this disease. methods: An apo E mutation was analyzed in a 57-year-old Japanese male with LPG and systemic atherosclerotic complications. Apo E phenotypes were analyzed by isoelectric focusing and immunoblotting. Apo E genotypes were determined by restriction fragment isotyping with HhaI. polymerase chain reaction (PCR) products of apo E coding region exons 3 and 4 were cloned into pT7Blue-T-vector and were sequenced. RESULTS: A novel apo E mutation was identified in this patient and his family. There was a discrepancy between an apo E phenotype (E1/3) and genotype (E3/3). sequence analysis showed a 54 bp deletion in exon 4 of the apo E gene, causing the 18-amino acid deletion (Gln 156-Gly 173-->0). This deletion mutation was further confirmed by the detection of a short fragment of PCR-amplified dna using polyacrylamide gel electrophoresis. The patient was a heterozygote with apo E1, and this mutation was determined to be the structural basis for the apo E1 phenotype. One of two daughters was a heterozygous carrier of apo E1, although she did not have proteinuria or atherosclerotic diseases. CONCLUSIONS: Apo E1 (Gln 156-Gly 173-->0) is a novel mutation of apo E that may be etiologically related to LPG and to the development of atherosclerosis. The result of this family study suggests that the occurrence of LPG may involve other genetic or environmental factors.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
6/96. Transcriptional regulation of PDGF-A and TGF-beta by KTS WT1 deletion mutants and a mutant mimicking denys-drash syndrome.denys-drash syndrome (DDS) and frasier syndrome (FS) are rare diseases caused by the mutations of wilms tumor gene, WT1. The common denominator in these syndromes is a nephropathy which is manifested by early-onset proteinuria, nephrotic syndrome and end stage renal failure. Although these syndromes are genetic models of nephropathy and the mutations of WT1 gene are characterized in these patients the mechanism how mutations of WT1 gene affect the embryonic kidney adversely has not been elucidated. Recently, there was a report that FS is caused by mutations in the donor splice site of WT1. These mutations predicted loss of KTS isoform, which is one of the four splicing variants of WT1. In this study, two KTS deletion mutants of WT1 were made as well as a WT1 mutant mimicking a mutation found in a patient who had diffuse mesangial sclerosis, end stage renal failure and wilms tumor. Mutant embryonic kidney cell lines were established by transfection of 293 embryonic kidney cells with WT1 mutants. We investigated the transcription regulation of mutant WT1 among these cell lines using the reporter vectors containing PDGF-A and TGF-beta promoter sequence. Our results showed that the promoter activity of PDGF-A and TGF-beta, which are related to the progression of glomerular diseases, was modestly increased in the mutant cell mimicking the patent, while those activities were markedly increased in other two deletion mutant cell lines. This study demonstrated that KTS WT1 mutation found in DDS affected the cytokine expression adversely in vitro. From these results, we suggest that the alteration of KTS WT1 expression be responsible for the rapid progression of renal diseases in DDS and FS.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
7/96. Disappearance of nodular mesangial lesions in a patient with light chain nephropathy after long-term chemotherapy.A 64-year-old man developed multiple myeloma (kappa light chain type), nephrotic syndrome, and renal insufficiency in 1993. A renal biopsy showed typical histological findings of light chain nephropathy: nodular glomerulosclerosis with deposition of kappa light chains in the mesangial area and subendothelial space of the glomerular capillary walls. Long-term intermittent MEVP chemotherapy (melphalan, 4 mg/d for 4 days; cyclophosphamide, 100 mg/d for 4 days; vincristine, 1 mg/d; prednisolone, 40 mg/d for 4 days) diminished proteinuria and improved renal function. In April 1999, a follow-up biopsy showed remarkable diminution of nodular lesions and disappearance of kappa light chain deposits. Although the prognosis of light chain nephropathy has been considered poor, long-term successful chemotherapy can clear light chain deposits and restore renal function.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
8/96. Leukocytoclastic vasculitis in a child with epidermolysis bullosa simplex.A 10-year-old boy with epidermolysis bullosa simplex (Weber-Cockayne variant) together with leukocytoclastic vasculitis is presented. He was admitted to the hospital with the provisional diagnoses of infected epidermolysis bullosa simplex or drug eruption. On the sixth day of hospitalization he developed palpable purpura, abdominal pain and bloody diarrhea, together with hematuria and proteinuria. A generalized tonic-clonic convulsion, changes in mental status, fluctuations in arterial blood pressure and intractable pain in his extremities occurred during the course of hospitalization. Systemic pulse steroid therapy, antibiotics, and antihypertensive and anticonvulsive drugs were given. On the 30th day of hospitalization, a skin graft was performed to replace a large tissue defect on his left hand. Despite high dose steroid therapy, his hematuria, proteinuria and hypertension continued after his discharge, suggesting a steroid-resistant renal pathology, such as focal glomerulosclerosis, that occurred secondary to leukocytoclastic vasculitis.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
9/96. Nephropathy subsequent to hyperlipidemia.The relationship between lipid abnormalities and the pathogenesis of renal disease is still unclear. Although most patients with primary hyperlipidemia do not develop renal function impairment, experimental and clinical data indicate a possible damaging effect of a disturbed lipid metabolism on the kidney. We report the case history of a patient with hyperlipidemia and mild nephropathy in which an accidentally removed kidney showed intrarenal arteriosclerosis which occured before the development of other cardiovascular risk factors, indicating that primary dyslipidemia induced nephroangiosclerosis.- - - - - - - - - - ranking = 2keywords = sclerosis (Clic here for more details about this article) |
10/96. hamartoma and renal transplant implications.A 19-year-old woman with tuberous sclerosis, bilateral renal hamartomas and polycystic kidneys required separate nephrectomies for severe hemorrhages 6 months apart. The case is presented, the diagnoses of the disease in relation to a negative family history are considered and the importance of early diagnosis in a potential transplant recipient and the use of leukocyte-free transufusions are discussed.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
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