Cases reported "Disease Progression"

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11/81. Successful steroid therapy for cefdinir-induced acute tubulointerstitial nephritis with progressive renal failure.

    A 58-year-old woman was admitted to our hospital because of renal dysfunction that continued to progress even after withdrawal of cefdinir, the presumed cause of acute renal failure. Renal histologic findings included interstitial fibrosis accompanied by moderate lymphocytic infiltration, and tubular atrophy with reduced numbers of epithelial cells. mesangial cells and glomerular basement membranes were nearly normal. Scintigraphy with 67gallium disclosed diffuse abnormal accumulation in both kidneys. A lymphocyte stimulation test with cefdinir was positive. The patient was diagnosed with acute tubulointerstitial nephritis caused by cefdinir. serum creatinine concentrations continued to rise after withdrawal of the drug, but steroid therapy was effective in normalizing renal function.
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12/81. Secondary amyloidosis in progressive systemic sclerosis.

    Progressive systemic sclerosis (PSS) is a connective tissue disease that may affect many organs, including the kidneys. It is quite rare to see secondary amyloidosis due to PSS. We present a patient with a 9-year history of PSS who developed nephrotic syndrome, and whose renal biopsy was compatible with secondary amyloidosis. He died from massive upper gastrointestinal bleeding caused by oesophageal telangiectasia.
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13/81. serum creatine kinase levels parallel the clinical course for rhabdomyomatous wilms tumor.

    A right-sided renal mass in an 11-month-old girl was diagnosed by percutaneous needle biopsy as wilms tumor, which on histologic examination was found to be predominantly rhabdomyomatous. As part of the examination, serum creatine kinase (CK) and CK-MB levels were measured and were significantly elevated at 994 U/L (reference range, 42-180 U/L) and 40 U/L (reference range, 0-3 U/L), respectively. Subsequently, an 8-month-old girl was admitted to the hospital with septicemia and was found to have an abdominal mass. A diagnosis of bilateral wilms tumor was made following percutaneous biopsy of both kidneys; histologic examination confirmed that the tumor was predominantly rhabdomyomatous. serum CK and CK-MB levels also were measured and were significantly elevated at 685 U/L and 84.4 U/L, respectively. In both cases, the serum CK and CK-MB levels reflected the clinical course; elevation in serum levels was associated with tumor recurrence, infarction, or chemotherapy-related necrosis. We conclude that these enzymes have clinical usefulness as markers for wilms tumor showing rhabdomyomatous morphologic features.
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14/81. Progressive demyelinating neuropathy after initiation of continuous ambulatory peritoneal dialysis--report of two cases.

    We report on two patients who developed rapidly progressive demyelinating neuropathy within 6 to 10 weeks after the initiation of continuous ambulatory peritoneal dialysis. The neuropathy in one patient resolved after kidney transplantation while that of the other patient improved with immunosuppressive therapy. The close temporal relationship between the initiation of peritoneal dialysis and the onset of neuropathy suggests that these two events may be causally related.
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15/81. Progression of hepatic damage during cold storage after procurement in a liver and kidney donor with hellp syndrome.

    BACKGROUND: HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome and acute fatty liver of pregnancy are associated with preeclampsia and fetal defects in fatty acid metabolism. This defect causes the accumulation of metabolites that are harmful to the maternal liver. CASE REPORT: We report a liver and kidney donor with hellp syndrome and describe the progression of disease in the liver during cold storage. Before procurement, liver biopsy demonstrated minimal necrosis. However, after cold storage, repeat biopsy demonstrated more than 30% necrosis. The liver was not engrafted; the kidneys were transplanted without complication. CONCLUSION: Livers procured from patients with hellp syndrome should be carefully evaluated for progression of hepatic damage during cold storage and transport.
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16/81. Spontaneous rupture of a renal angiosarcoma.

    Here we describe the clinical, radiologic, histopathological and immunohistochemical features of a rare spontaneous rupture of renal angiosarcoma detected in the left kidney of a 55-year-old male. Due to the rarity of this neoplasm (less than 15 cases have been reported), there is no unanimous consensus for therapy following radical nephrectomy. In our case there was no flank trauma and retroperitoneal haematoma around the left kidney was seen on computed tomography. The patient underwent left radical nephrectomy and died about 3 months after operation due to multiple bone and liver metastases. To our knowledge, this is the first report of spontaneous rupture of renal angiosarcoma and we suggest that it should be borne in mind that renal angiosarcoma may be a cause of retroperitoneal haematomas.
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17/81. Fatal cytotoxic T-cell proliferation in chronic active Epstein-Barr virus infection in childhood.

    Histopathologic features of 5 cases (4 boys and 1 girl; 4-9 years old) with severe chronic active Epstein-Barr virus (EBV) infection are discussed. All patients died within 3 years after disease onset without developing hematolymphoid malignant neoplasms. The pathology specimens (autopsy, 2 cases; multiple organs and tissues obtained by surgery or biopsy, 3 cases) showed polymorphic lymphocytic proliferation in the lymph nodes (4/5) and spleen (3/3), and systemic lymphocytic infiltration of the liver (4/4), lung (2/2), bone marrow (3/4), and kidney (2/2). skin lesions were noted clinically in 3 of 5 cases. Two cases had coronary artery aneurysm due to lymphocytic vasculitis. The lymphocytes had a characteristic phenotype of cytotoxic T cells expressing CD3, CD8, and cytotoxic molecules, and were negative for CD4. EBV-encoded small nonpolyadenylated RNAs were detected in the nuclei of the lymphocytes, but latent membrane protein 1 and EBNA2 were not seen. In 4 of 4 cases, an oligoclonal growth pattern of EBV was determined after detecting terminal repetitive sequences by Southern blot. In 3 of 3 cases, the lymphocytes did not have T-cell receptor beta or J(H) gene rearrangement.
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18/81. Extragonadal seminoma after renal transplantation and immunosuppression; treatment in the presence of renal dysfunction: a case report and literature review.

    A 37-yr-old man who had undergone renal transplantation for end-stage renal failure presented with a large right pelvic mass obstructing the transplanted kidney. Initially, this was diagnosed as an anaplastic tumor while he had been on immunosuppressive treatment for kidney allograft rejection after transplantation. Despite difficulties of classic histopathology to reveal the origin of his tumor, FISH analysis revealed the presence of chromosome 12p abnormalities, strongly indicative of a germ-cell tumor-more likely seminoma-with extragonadal presentation. Because of renal dysfunction, he was treated with carboplatin (dose adjusted according to renal clearance) and etoposide, and when he experienced a rather atypical progression with bone metastases, he was treated with single-agent paclitaxel, and died almost 13 mo after initial presentation. The case adds further to the existing small list of seminoma/GCTs developing in transplant recipients, points to the unusual presentation patterns and diagnostic histopathology challenges, and presents the difficulty in therapeutic options, as a result of frequent renal dysfunction and intercurrent immunosuppressive therapy. All of these issues together with an extensive literature review are discussed in detail.
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keywords = kidney
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19/81. 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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20/81. Primary chronic interstitial nephritis in Crohn's disease.

    BACKGROUND & AIMS: In Crohn's disease, cases of interstitial nephritis with renal failure have been reported in connection with the use of mesalamine. methods: We observed 4 patients with severe interstitial nephritis proven by examination of kidney biopsy specimens. Renal failure was discovered before or simultaneously with the diagnosis of Crohn's disease, and patients were not treated with mesalamine. Impairment of renal function progressed to end-stage renal failure in 3 of the 4 patients. RESULTS: Our results show that the kidney can be an extraintestinal target of Crohn's disease. CONCLUSIONS: Several unanswered questions remain concerning the frequency of interstitial nephritis in patients with Crohn's disease, as well as the exact role of mesalamine in the development of chronic interstitial nephritis.
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