Cases reported "Disease Progression"

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21/81. Fatal intestinal tubercolosis in a uremic patient with a renal transplant.

    We report the case of a 52 year-old woman who was re-admitted to regular hemodialysis treatment because of chronic rejection of a renal transplant. She had received her mother's kidney 17 years before and had been treated for a long time with steroids, cyclosporin and azathioprine. In the last two months, fever had occurred, and persisted with the start of hemodialysis. She was admitted to our nephrology unit. Clinical, laboratory, radiological and endoscopic investigations did not lead to a precise diagnosis and broad-spectrum antimicrobial therapy failed. Some days later, a clear clinical picture of acute abdomen arose and at laparatomy a perforated jejunal ulcer was found. Histological investigation revealed caseous necrosis around the ulcer. Ziehl-Neelsen (ZN) stain showed a number of acid-fast resistant bacilli. polymerase chain reaction (PCR) confirmed the presence of mycobacterium tuberculosis. Specific therapy was started, but nevertheless the patient died a few days later, of septic shock. Our case shows that tuberculosis continues to be a significant, severe clinical problem in transplant recipients and is in fact still an important cause of death in these patients. The possibility of tuberculosis must be taken into account when a transplant patient shows fever and severe abdominal trouble with no clear evidence of another infection.
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22/81. Does type 2 diabetes mellitus delay renal failure in autosomal dominant polycystic kidney disease?

    Autosomal dominant polycystic kidney disease (ADPKD) is a common renal disease without an effective therapeutic intervention to delay renal failure. Within kindreds, renal dysfunction often develops at a similar age in affected individuals, although there are known modifying factors. Two kindreds with ADPKD have shown a striking pattern of delayed onset of renal insufficiency in those individuals also suffering from type 2 diabetes mellitus. Eight nondiabetic patients with ADPKD had onset of dialysis or renal death at ages 38-52 years, (mean /- SEM 46 /- 1.9, n = 7) as compared with four diabetics who started dialysis or are still off dialysis at the age of 61 /- 2.8 years (p < 0.01). Two of the four diabetics still have reasonable renal function at age 61 and 66. The diabetes was diagnosed at age 32 /- 2 years and was treated with oral hypoglycemics for 19 /- 2 years before institution of insulin. Cardiovascular disease dominated the clinical picture in the diabetics. In conclusion, onset of renal failure in ADPKD was delayed for over 15 years in individuals who also suffered from type 2 diabetes mellitus, in two ADPKD kindreds. Possible mechanisms are discussed, including glibenclamide inhibition of the cystic fibrosis transmembrane conductance regulator. The striking delay associated with type 2 diabetes mellitus in ADPKD induced renal failure should be evaluated further.
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keywords = kidney disease, kidney
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23/81. renal osteodystrophy presenting with multiple calcified periarticular swellings.

    renal osteodystrophy denotes skeletal abnormality in patients with renal disease. Although radiological and histological evidences of osseous abnormality are commonly seen in advanced renal failure, clinical symptoms of bone disease are uncommon. In this article a case of chronic renal failure presented with multiple calcified periarticular swellings measuring up to 5 cm in diameter is described. The patient also had severe osteopenia, subperiosteal erosions, bilateral shrunken echogenic kidneys as well as clinical and biochemical evidences of chronic renal failure. renal osteodystrophy is discussed and related literature is reviewed.
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24/81. Renal graft failure due to type 1 primary hyperoxaluria.

    Primary hyperoxaluria type 1 (PH1) usually presents with recurrent urolithiasis, nephrocalcinosis and progressive renal failure at a relatively young age. This report describes a patient who, due to the late onset of end-stage renal disease, had been diagnosed with PH1 only after failure of his second kidney graft. Retrospectively, his vascular problems, skeletal abnormalities and cardiac arrhythmias fit the picture of severe systemic oxalosis. Possible therapeutic options are discussed.
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25/81. Effect of renal artery stenting on the progression of renovascular renal failure: a case of intravascular ultrasound-confirmed renovascular disease.

    We report the case of a 71-year-old male, submitted to percutaneous transluminal renal angioplasty (PTA) plus stent implantation following the confirmation, at intravascular ultrasound, of severe unilateral renal artery stenosis in the setting of a single functional kidney and of evidence of renal insufficiency (serum creatinine value 300 mumol/l). At 6 months of follow-up the serum creatinine levels had returned to normal (98 mumol/l). This case shows the role of direct PTA on the overall renal function in a case of global renal ischemia.
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26/81. Kidney-pancreas transplantation in a long-term non-progressor HIV-infected recipient.

    With the introduction of highly active antiretroviral therapy (HAART), HIV infection has become a chronic disease with more frequent end-stage organ failures. As a result, the question of transplantation in HIV patients is raised more often. Although still subject to controversies, HIV infection is no longer an absolute contraindication to solid organ transplantation. We report a case of combined kidney-pancreas transplantation in a HIV recipient. HIV has remained stable without any antiviral therapy for up to 2 years after transplantation and has reached criteria for inclusion in the long-term nonprogressor (LTNP) group. Grafted organs demonstrated good function without rejection. This case emphasizes the need to consider LTNP HIV patients as a specific subgroup, when discussing solid organ transplantation. HAART is not required, thus sparing drug interactions and their unique immunological features, such as CCR5 mutation, might prevent rejection. This subgroup of HIV patients should be offered less restricted access to transplantation.
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keywords = kidney
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27/81. Renal involvement in Anderson-fabry disease.

    Anderson-fabry disease (AFd) is a rare X-linked lisosomal storage disorder of glycosphingolipid (GL) metabolism, caused by a deficiency of the activity of alpha-galactosidase A (alpha-gal A). The progressive accumulation of GL in tissues results in the clinical manifestations of the disease, that are more evident in hemizygous males, and include characteristic skin lesions (angiokeratomas), neurological symptoms (acroparesthesia), ocular features (cornea verticillata), cardiac involvement (left ventricular enlargement, conduction abnormalities), cerebrovascular manifestations (thromboses, hemorrhage, etc.), and kidney involvement with progression to end-stage renal failure (ESRF). ESRF is a common manifestation in hemizygous males (3rd-5th decade) and death occurs around the 5th decade of life because of severe cardiac and/or cerebrovascular complications. Heterozygous females have an attenuated form of this systemic disease. In the kidney, accumulation of GL occurs in the endothelial cells of every vessel, in the epithelial cells of every tubular segment, and in all kinds of glomerular cells. The broad spectrum of renal lesions is a pathophysiological continuum with progressive impairment in the renal function related to continuous intracellular deposition of GL. Electron microscopic study of renal biopsies shows typical osmiophilic inclusion bodies in the cytoplasm of all kind of renal cells, characterized by concentric lamellation of clear and dark layers (35-50 A of periodicity). ESRF is treated by dialysis and kidney transplantation: neither treatment modifies the progression of the cardiovascular and cerebrovascular lesions due to progressive GL deposition. The outcome of kidney transplantation seems to be similar to that found in other non-diabetic patients, but the survival rate on dialysis is lower than in patients with other causes of ESRF. Nowadays, treatment with enzyme replacement infusion with purified alpha-Gal A, produced by a genetically engineered human cell line or Chinese hamster ovocytes, seems to be effective and safe.
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keywords = kidney
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28/81. Collapsing glomerulopathy coexisting with membranous glomerulonephritis in native kidney biopsies: a report of 3 HIV-negative patients.

    Collapsing glomerulopathy (CG), a variant of idiopathic focal segmental glomerulosclerosis (FSGS), can occur in both human immunodeficiency virus (HIV)-positive and HIV-negative patients. Idiopathic membranous glomerulonephritis (MGN) has been reported to coexist with FSGS, but rarely with CG. We report 3 HIV-negative patients (2 men, 1 woman) who developed nephrotic syndrome secondary to MGN complicated by CG, with relatively rapid disease progression despite aggressive therapy.
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keywords = kidney
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29/81. Renal medullary carcinoma in a six-year-old boy with sickle cell trait.

    Renal medullary carcinoma (RMC), an aggressive malignant epithelial neoplasm, first emerged as a distinct clinicopathologic entity in 1995. It affects individuals 40 years of age or younger and is strongly associated with sickle cell disease or trait. The majority of patients with RMC have widely disseminated disease at the time of diagnosis and most fail to respond to both chemotherapy and radiotherapy. mortality approaches 100%, and death usually occurs within a few months to a year of diagnosis. We report a 6-year-old African-American boy with a history of gross hematuria who died four weeks after diagnosis of disseminated metastatic disease. autopsy showed a 4.4-cm renal mass with metastases to the contra lateral kidney, liver, lungs and multiregional lymph nodes. RMC should be included in the differential diagnosis of any patient 40 years old or younger with a history of hemoglobinopathy and gross hematuria and/or abdominal or flank pain. A brief discussion of the differential diagnosis, histogenesis and treatment is presented in this study.
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30/81. Kidney and liver transplantation in HIV-infected patients: case presentations and review.

    Until recently, HIV-infected patients have been excluded from consideration for solid organ transplantation. The relatively high mortality rates among HIV-infected transplant recipients observed in the era prior to the use of highly active antiretroviral therapy (HAART), coupled with long waiting times for cadaveric organs, made it difficult to support organ transplantation in this patient group. However, in response to the marked reductions in morbidity and mortality associated with HIV infection, several transplant centers have developed pilot studies or revised their clinical criteria to allow transplantation in this group of patients. We describe two cases, one kidney and one liver transplant recipient, and review the major clinical and research issues related to this topic. Reports of transplantations in the pre-HAART era highlight two important findings. First, some HIV-infected transplant recipients did very well with long survival periods. However, overall progression to AIDS and death appeared accelerated. We recently reported on our preliminary experience with 45 selected transplant recipients in the HAART era. One-year patient survival rates were similar to unmatched survival data from the United Network for Organ Sharing (UNOS) database. Median CD4 T-cell counts remained stable in the follow-up period compared to pretransplant. hiv-1 rna nearly uniformly continued to be suppressed below the limits of detection. Preliminary data are promising and support the current efforts to evaluate patient and graft survival among HIV-infected transplant recipients and to explore the mechanisms underlying the many potential complications of transplantation in this population.
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