Cases reported "Fibrosis"

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11/32. nephrotic syndrome and focal glomerulosclerosis in adult polycystic kidney disease.

    We report a 35-year-old man with adult polycystic kidney disease (APKD) that developed nephroptic syndrome accompanied by a rapid worsening of renal function. Histologic examination showed marked tubulointerstitial chronic abnormalities and focal glomerulosclerosis (FGS) lesions in 24% of the glomeruli. With captopril, an renal function continued to deteriorate. No other cases of nephrotic-range proteinuria were detected among 65 APKD patients with renal insufficiency. Histologic examination of an other 12 kidneys removed from patients with APKD showed striking interstitial lesions, most of the glomeruli being normal. However, those patients with higher amounts of proteinuria had more glomeruli (14-32%) with FGS lesions.
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keywords = kidney disease, kidney
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12/32. Giant hydronephrosis with increased carbohydrate antigen 19-9 both in serum and fluid.

    We report a case of right giant hydronephrosis. A 68-year-old man was admitted to our hospital with chief complaints of general fatigue, loss of appetite and a one-year history of progressive fullness on whole abdomen. Abdominal computed tomography scan exhibited a huge, homogeneous, low density mass originating from the right kidney. We performed right percutaneous nephrostomy and drained over 6,500 ml bloody fluid. Cytological examination of the drained fluid revealed atypical nuclear appearance defined as class III. Increased values of carbohydrate antigen 19-9 were observed both in the fluid as well as in the serum. We performed right nephrectomy. Macroscopic appearance of the resected kidney showed marked stenosis at the portion of ureteropelvic junction. Histological analysis of the stenotic portion demonstrated marked fibrosis without findings of malignancy.
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ranking = 0.030139169432366
keywords = kidney
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13/32. nephrogenic fibrosing dermopathy in two patients with systemic lupus erythematosus.

    nephrogenic fibrosing dermopathy (NFD) is a novel fibrosing disorder of the skin with characteristic histopathology. It affects patients with impaired renal function and appears to be independent from the type of kidney disease. Its aetiopathology is unknown and presently no standard therapy exists. We report a patient with systemic lupus erythematosus (SLE) and glomerulonephritis who developed diffuse indurated erythematous plaques covering nearly the entire legs and trunk. She had never received dialysis. The second patient suffered from SLE and antiphospholipid syndrome related thrombotic glomerulopathy. After 10 weeks of haemodialysis she developed the same skin condition. To the best of our knowledge, these are the first reports of NFD occurring in patients with SLE.
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ranking = 0.19698608305676
keywords = kidney disease, kidney
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14/32. Severe course of primary hyperoxaluria and renal failure after domino hepatic transplantation.

    We report herein a domino orthotopic liver transplantation (LT), from a 38-year-old woman undergoing liver-kidney transplantation (LKT) for primary hyperoxaluria type I (PH1) to a recipient with cirrhosis and hepatocellular carcinoma. Delayed onset of PH1 and renal failure and 10% residual alanine-glyoxylate aminotransferase (AGT) activity in domino liver justified its use for domino procedure. The clinical course after LKT was similar to that described in other series, including ours. Renal function started promptly and maintained despite sustained hyperoxaluria from dissolution of oxalotic deposits. Conversely, the domino recipient manifested severe hyperoxaluria and developed nephrolithiasis and renal insufficiency with rapid progression over 2 months. A new LT resulted in slow decrease of oxaluria and improvement of renal function. Therefore, PH1 behaved quite differently in these two patients, leading us to conclude that domino LT using livers from PH1 patients should be considered very carefully, only as a bridge to definitive LT in recipients with critical clinical conditions.
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ranking = 0.015069584716183
keywords = kidney
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15/32. Secondary renal amyloidosis due to long-standing tubulointerstitial nephritis in a patient with Sjogren syndrome.

    A 53-year-old patient with long-standing primary Sjogren syndrome presented with acute renal failure and nephrotic syndrome caused by secondary (AA) renal amyloidosis. Ten years before, he had been admitted because of exacerbation of the systemic disease. At that time, a pseudolymphoma of the kidney was diagnosed. To our knowledge, this is the first report of a patient with primary Sjogren syndrome and secondary (AA) amyloidosis with amyloid deposition in the kidneys causing nephrotic syndrome.
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ranking = 0.030139169432366
keywords = kidney
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16/32. nephrogenic fibrosing dermopathy: a case study.

    nephrogenic fibrosing dermopathy (NFD) is a rare disease which has only recently been described histopathologically. In the literature only about 100 cases of this disease have been described. NFD occurs most frequently in patients with renal insufficiency or after kidney transplantation. We present a case study of a patient who showed manifestations of NFD in the abdomen after a second transplantation of a cadaver kidney. The diagnosis was determined by an experienced histopathologist after excision of the focus. The authors discuss the etiology and genesis, which is not yet known, diagnosis of this disease and its treatment.
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ranking = 0.030139169432366
keywords = kidney
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17/32. Multiorgan involvement in nephrogenic fibrosing dermopathy: an autopsy case and review of the literature.

    nephrogenic fibrosing dermopathy is a recently recognized, scleromyxedema-like fibrosing skin condition that occurs in individuals with acute or chronic renal failure. Although the early descriptions of this disorder describe a purely cutaneous disease process, 2 recent autopsy reports have identified apparent multiorgan fibrosis with involvement of skeletal muscle, myocardium, lungs, kidneys, and testes. We describe a 23-year-old man with nephrogenic fibrosing dermopathy and significant fibrosis of the atrial myocardium and dura mater, which was identified at autopsy. Dural fibrosis is a previously undescribed systemic manifestation of nephrogenic fibrosing dermopathy. The literature is reviewed.
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ranking = 0.015069584716183
keywords = kidney
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18/32. retinitis pigmentosa and renal failure in a patient with mutations in INVS.

    BACKGROUND: Nephronophthisis (NPHP) is an autosomal recessive disease, which is the most common genetic cause of end-stage renal disease in the first three decades of life. The disease is caused by mutations in the NPHP 1-5 genes, and is referred to as NPHP types 1-5, respectively. The association of NPHP and retinitis pigmentosa (RP) is known as Senior-Loken syndrome (SLS). The RP is associated with 10% of cases of NPHP types 1, 3 and 4, and all cases of NPHP type 5, but never in NPHP type 2, the infantile form of NPHP. The NPHP type 2 is distinguished from other types of NPHP by its early age of onset and by cystic enlargement of the kidneys. methods: Mutational analysis of all five NPHP genes was performed by exon sequencing in a child with infantile NPHP and RP from a consanguineous kindred. RESULTS: A homozygous mutation was identified in exon 13 of inversin (INVS) (C2719T, R907X) in this child. CONCLUSIONS: This is the first report of the presence of RP in a patient with NPHP type 2 and INVS mutations. This report now extends the association of RP with NPHP to NPHP type 2.
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ranking = 0.015069584716183
keywords = kidney
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19/32. nephrogenic fibrosing dermopathy in children.

    nephrogenic fibrosing dermopathy (NFD) is an entity of unknown etiology that has only been reported in patients with impaired kidney function. It has characteristic diagnostic findings by skin biopsy and due to its systemic involvement in some reported cases, the term "nephrogenic systemic fibrosis" has been recently suggested as more appropriate for the nomenclature of this entity. There is no curative treatment currently available and very few cases have been reported in children. We hereby report two cases so as to alert pediatric health care providers about the existence of this disorder.
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ranking = 0.015069584716183
keywords = kidney
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20/32. Liesegang rings developing within intraperitoneal endometriotic implants. A case report.

    Liesegang rings are acellular, laminated structures that have been reported to accompany a variety of necrotizing and hemorrhagic tissue processes. Although most frequently seen in the kidneys and synovium, they can be present in other tissues. Liesegang rings are of importance because they are frequently confused by pathologists with helminth ova and nematodes, which they resemble. A case of Liesegang rings was associated with intraperitoneal endometriotic implants, with which the rings appeared to have originated.
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ranking = 0.015069584716183
keywords = kidney
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