Cases reported "Fibrosis"

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1/32. 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.
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2/32. Chronic unilateral ureteral obstruction represented as renin-dependent hypertension.

    A 50-year-old woman developed renin-dependent hypertension immediately after accidental unilateral ureteral ligation during hysterectomy, and the hypertension lasted for 5 months. Surgical release of the obstruction was carried out 157 days after the ligation. Then, her blood pressure was normalized. However, the obstructed kidney showed intensive tubulointerstitial fibrosis and functional recovery was not obtained. This case suggests that the renin-angiotensin system may be upregulated in human kidney during unilateral ureteral obstruction for a long duration.
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3/32. Multivisceral eosinophilic fibrosis: a new clinical presentation.

    We describe a patient who was referred to us with the diagnosis of pancreatic cancer but who had eosinophilic fibrosis of the pancreas and other organs, including the sub-mandibular salivary glands, retro-orbital tissue, liver, kidneys, and surrounding the abdominal aorta. He had no pain or other symptoms. After treatment with methylprednisolone, all lesions disappeared and now he seems to be cured of this apparently immune-mediated disorder. To our knowledge, involvement of all these particular organs by eosinophilic fibrosis in the absence of symptoms has never before been described.
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4/32. 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.
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5/32. Severe intrarenal fibrosis, infundibular stenosis, renal cysts, and persistent perilobar nephrogenic rests in a patient with beckwith-wiedemann syndrome 27 years after diffuse nephroblastomatosis and wilms tumor: natural progression or a consequence of treatment?

    A27-year-old woman presented with back and abdominal pain. She was diagnosed in infancy with beckwith-wiedemann syndrome and bilateral multifocal perilobar nephrogenic rests that progressed to diffuse nephroblastomatosis with neoplastic nephroblastomatous rests at 14 months of age and subsequently to a right wilms tumor at 5 years of age. Computed tomography of the abdomen during the current admission showed multiple obstructed calices. Ureteroscopic inspection of the left kidney revealed severe intrarenal scarring with multiple infundibular stenosis, hydrocalices, and nephrocalcinosis. Renal biopsy showed sclerotic glomeruli with calcification and scarring and persistent subcapsular nodular renal blastema. Electrocautery incision and balloon dilatation provided temporary pain relief. After discharge, the patient has had two or three episodes of recurrent pain associated with new areas of infundibular stenoses and renal cysts. Bilateral nephrectomy and renal transplantation is being considered for management of progressive disease and relief of intractable pain. The potential causes of progressive and severe intrarenal fibrosis, infundibular stenosis and nephrocalcinosis, and renal cysts in this patient may include abnormal renal development secondary to beckwith-wiedemann syndrome itself, radiation or chemotherapy damage, or a combination.
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6/32. Reflux nephropathy in a patient with crossed renal ectopia with fusion.

    Crossed renal ectopia with fusion is a rare congenital anomaly of the urinary tract. While vesicoureteral reflux has been noted frequently, reflux nephropathy has rarely been reported with this anomaly. I describe here a case of reflux nephropathy in a patient with crossed renal ectopia with fusion. A 14-year-old boy was referred to our hospital for further examination of proteinuria, detected by the school's annual urinary screening program. Laboratory study revealed proteinuria and renal insufficiency. Abdominal computed tomography and voiding cystography disclosed crossed renal ectopia with fusion and vesicoureteral reflux in each moiety. Renal biopsy of the orthotopic kidney showed diffuse interstitial fibrosis and mononuclear cell infiltration, periglomerular fibrosis, glomerular hypertrophy, and focal and global glomerulosclerosis, which were compatible with reflux nephropathy. A low-protein diet and an administration of angiotensin converting enzyme inhibitor decreased the urinary excretion of protein.
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7/32. nephrogenic fibrosing dermopathy: an unusual skin condition associated with kidney disease.

    We report the case of a patient who, while on long-term hemodialysis (HD), developed nephrogenic fibrosing dermopathy, a newly described sclerosing skin disorder. This disorder is characterized by thickened, hardened skin with brawny hyperpigmentation and raised plaques. The most common patient complaints are pruritus and dysesthesia. The extremities are predominantly involved with sparing of the torso and face. Dysfunction of internal organs has not been described, distinguishing it from other fibrosing conditions such as scleroderma. The skin biopsy is characterized by haphazardly arranged dermal collagen spindle cells in the reticular dermis. Extensive mucin deposits are interposed between collagen bundles and there are an increased number of fibroblast-type cells. In contrast to scleroderma, inflammatory cells are generally absent. Corticosteroid therapy can be tried, but in our patient was of no benefit.
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keywords = kidney disease, kidney
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8/32. nephrogenic fibrosing dermopathy after liver transplantation successfully treated with plasmapheresis.

    nephrogenic fibrosing dermopathy (NFD) is a recently described cutaneous fibrosing disorder associated with renal dysfunction. It appears similar to scleromyxedema but with some notable exceptions, including the lack of involvement of the face and absence of plasma cells on histology, systemic involvement, and paraproteinemia. patients can present with thickened or edematous skin with indurated papules and plaques involving the extremities and the trunk. We report the first three cases of NFD after liver transplantation successfully treated with plasmapheresis. Two patients underwent liver transplantation for hepatitis c virus-induced cirrhosis and one for hepatitis b virus-induced cirrhosis. All the patients had encephalopathy, refractory ascites, and malnutrition prior to transplantation. Like those patients with NFD, all three of our patients had renal dysfunction and required hemodialysis before and after transplantation. Two were not dependent on dialysis at the time of diagnosis, however. These patients had excellent liver allograft function, but the other patient had allograft failure secondary to recurrent hepatitis c. immunosuppression therapy consisted of basiliximab, mycophenolate mofetil, calcineurin inhibitor, and prednisone. The patients developed "woody" skin induration of the distal extremities, erythematous papules, and contractures at 1, 2, and 120 months after transplantation. skin biopsies resembled NFD. No paraproteinemia was evident. One to three 5-day courses of plasmapheresis resulted in moderate to marked clinical improvement. The improvement of the kidney function in two of our patients did not appear to correlate with that of the skin disorder, because the kidney function was improving at the time the diagnosis of NFD was made. In conclusion, we report the first three cases of NFD after liver transplantation. plasmapheresis was moderately successful in resolving the skin-indurated papules, severe skin induration, and associated joint contractures. Preliminary studies (unpublished data) show that decreasing plasma levels of transforming growth factor-beta1 after plasmapheresis appear to correlate with the amelioration of this clinical condition.
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9/32. Aristolochic acid nephropathy and the peritoneum: Functional, structural, and molecular studies.

    BACKGROUND: Aristolochic acid nephropathy (AAN) is a rapidly progressive interstitial nephropathy linked to the exposure to aristolochic acid (AA) and characterized by extensive fibrosis and urothelial atypia. Although the fibrotic process has been documented in extrarenal tissues, the involvement of the peritoneum, as well as the efficacy of peritoneal dialysis in AAN patients, remain uncertain. methods: The structure of the peritoneal membrane and the expression of basic fibroblast growth factor (bFGF), collagen type iii, endothelial nitric oxide synthase (eNOS), and aquaporin-1 (AQP1) were investigated in peritoneal biopsies from an index AAN patient, four other AAN patients, four regular peritoneal dialysis patients, and two controls. Similar methods were used to investigate a rabbit model of AAN after intraperitoneal exposure to high-dose AA. AA-dna adducts were screened by 32P-postlabeling analysis. RESULTS: The AAN patients had renal failure, renal fibrosis, and urothelial atypia. The peritoneum of AAN patients had a normal structure, lacked cellular atypia, and, in comparison with regular peritoneal dialysis patients and controls, did not show abnormal regulation of fibrotic and endothelial markers. Furthermore, specific AA-dna adducts were not identified in the peritoneum of AAN patients. In contrast, AA-dna adducts were detected in peritoneal and kidney tissues of all exposed rabbits, and one of them developed a malignant mesothelioma. CONCLUSION: These data demonstrate the lack of fibrotic and vascular alterations and the absence of cellular atypia in the peritoneum from AAN patients. Thus, peritoneal dialysis should not be discouraged in these patients. Nevertheless, studies in a rabbit model of high-dose AA exposure may suggest a potential risk of peritoneal malignancy.
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10/32. nephrogenic fibrosing dermopathy treated with extracorporeal photopheresis.

    nephrogenic fibrosing dermopathy (NFD) is a rare fibrosing skin disease of unknown etiology occurring in patients with terminal renal disease. It was first described in the year 2000. The histology of NFD shows an increased number of dendritic cells, fibroblasts and thickened collagen fibers resembling scleromyxedema. It can be distinguished from scleromyxedema by a different distribution pattern of the skin lesions with indurated plaques mainly on the extremities and the absence of paraproteinemia. As yet, no treatment for NFD has been proven to be uniformly efficient. We describe the case of a 40-year old patient with renal insufficiency who was treated with hemodialysis and who had undergone kidney transplantation. Two years after transplantation, she developed sclerodermiform brownish plaques on her extremities. The induration improved significantly after 4 cycles of extracorporeal photopheresis.
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