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1/28. Basic fibroblast growth factor in HIV-associated hemolytic uremic syndrome.

    Endothelial injury is the primary pathogenic event leading to the renal thrombotic microangiopathic lesions typical of the hemolytic uremic syndrome (HUS). Basic fibroblast growth factor (bFGF) is an angiogenic growth factor released by injured endothelial cells. In a previous study we have found a significant accumulation of bFGF in human immunodeficiency virus (HIV)-transgenic mice with renal disease. Here we investigated whether bFGF was accumulated in the circulation and kidneys of two children with HIV-associated HUS (HIV-HUS), and studied the mechanisms involved in this process. The plasma levels of bFGF in children with HIV-HUS (124 /-20 pg/ml) were increased compared with five children with HIV nephropathy (49 /-6 pg/ml) and twenty HIV-infected children without renal disease (26 /-4 pg/ml, P<0.001). immunohistochemistry and receptor binding studies showed that bFGF was accumulated bound to heparan sulfate proteoglycans in renal glomeruli and interstitium surrounding renal tubules in HIV-HUS kidneys. Basic FGF stimulated the proliferation of mesangial and urinary renal tubular epithelial cells isolated from both patients. These findings support the hypothesis that bFGF and its low-affinity binding sites may play a relevant role in modulating the process of glomerular and renal tubular regeneration during the acute stages of HIV-HUS. A follow-up study in a larger sample population is required to confirm these results.
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keywords = nephropathy
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2/28. Resolution of HIV-associated nephrotic syndrome with highly active antiretroviral therapy delivered by gastrostomy tube.

    There is no consensus regarding the specific management of HIV-associated nephrotic syndrome. We report a child whose first manifestation of human immunodeficiency virus type 1 (hiv-1) infection was nephropathy and wasting syndrome associated with profound immunodeficiency. The patient had a dramatic clinical and immunologic response to triple antiretroviral therapy delivered through a gastrostomy tube, with complete resolution of nephrotic syndrome. A 51/2-year-old African-American girl presented with a 2-week history of cough, chest pain, vomiting, loose stools, abdominal distention, anorexia, and fever. In addition, she had recurrent oral thrush. Her weight and height were below the 5th percentile. She was chronically ill, appearing with oropharyngeal thrush and pitting edema in lower extremities. She had scattered rhonchi and decreased breath sounds on both lung bases. Her abdomen was distended and diffusely tender. A chest radiograph showed consolidation of the right upper and left lower lobes with bilateral pleural effusion. Admission laboratories were consistent with nephrotic syndrome. streptococcus pneumoniae grew from the blood culture and the child responded well to treatment with intravenous ceftriaxone. She was found to be HIV-infected, her CD4( ) cell count was 3 cells/mcL and her plasma hiv-1 rna was >750 000 copies/mL. A percutaneous gastrostomy tube was placed for supplemental nutrition. She was treated with stavudine, lamivudine, and nelfinavir via gastrostomy tube with good clinical response. Twenty-one months after instituting antiretroviral therapy, her weight and height had increased to the 50th and 10th percentile respectively, and she had complete resolution of her nephrotic syndrome. Her CD4( ) cell count increased to 1116 cells/mcL and her viral load has remained undetectable. hiv-1 associated nephrotic syndrome has been described in children with profound immunodeficiency. The course of untreated HIV-associated nephrotic syndrome is rapid progression to renal failure in up to 40% of the children. Regardless of the presence of renal insufficiency, if untreated, it is uniformly fatal. A modest improvement of hiv-1 associated nephrotic syndrome has been observed in patients treated with zidovudine. Steroid and cyclosporine treatment have resulted in improved renal function but long-term use of immunosuppressive therapy has raised concerns about safety. We have described, to our knowledge, the first child with HIV-associated nephrotic syndrome who had a remarkable clinical, immunologic, and virologic response to triple-drug combination therapy given by gastrostomy tube, with complete resolution of proteinuria and normalization of the serum albumin. She also had a striking improvement in weight, height, and quality-of-life. Whether the presence of a gastrostomy tube contributed to the excellent response because of improved compliance is unknown, but warrants systematic evaluation.
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keywords = nephropathy
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3/28. HIV-associated nephropathy: case study and review of the literature.

    Human immunodeficiency virus type 1 (hiv-1)-seropositive patients are at risk for the development of a variety of acute and chronic renal diseases. The most common cause of chronic renal failure in hiv-1-seropositive patients is HIV-associated nephropathy (HIVAN). HIVAN occurs almost exclusively in black patients and the majority of published cases are of patients who present with acquired immunodeficiency syndrome (AIDS). This disease is currently the third leading cause of end-stage renal disease in blacks aged 20-64. Because hiv-1-seropositive patients may develop a wide variety of acute and chronic renal diseases, definitive diagnosis requires renal biopsy. Emerging data suggest a direct role of hiv-1 infection of kidney cells in the pathogenesis of HIVAN. There have been no well-controlled clinical trials in the treatment of HIVAN. The therapeutic agents with the most promise are angiotensin-converting enzyme inhibitors and antiretroviral medications. Long-term renal prognosis may be changing in the setting of improved aggressive antiretroviral therapy. Patient survival is determined primarily by the stage of hiv-1 infection. In this article, we present the case history of a patient who developed HIVAN. We then review the current literature concerning the epidemiology, differential diagnosis, etiology, and treatment of HIVAN.
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ranking = 5
keywords = nephropathy
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4/28. Resolution of organ-specific complications of human immunodeficiency virus infection in children with use of highly active antiretroviral therapy.

    opportunistic infections are a major source of morbidity and mortality in children and adults infected with human immunodeficiency virus (HIV). In addition, organ-specific complications of HIV infection, such as cardiomyopathy, nephropathy, encephalopathy, and others, contribute substantially to the morbidity and mortality associated with HIV infection. Highly active antiretroviral therapy (HAART) has produced a dramatic decline in the incidence of opportunistic infections among patients with HIV infection. Nevertheless, there is very little information concerning the value of HAART for organ-specific complications of HIV infection. In this report, we describe 3 children with HIV infection in whom the dominant clinical manifestations were cardiomyopathy, red cell aplasia, and nephropathy. HAART produced a decrease in the HIV ribonucleic acid level, an increase in the CD4 cell count, and resolution of the organ-specific complications in all patients. These cases add to our knowledge concerning the benefits of HAART for children with HIV infection.
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ranking = 2
keywords = nephropathy
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5/28. Hiv-associated nephropathy occurring before HIV antibody seroconversion.

    It currently is thought that human immunodeficiency virus-associated nephropathy (HIVAN) occurs late in the course of HIV infection. Although HIVAN may be the presenting manifestation of acquired immunodeficiency syndrome (AIDS), it usually occurs after a prolonged period of viral infection often associated with high levels of HIV viremia. The patient described here developed HIVAN as a manifestation of acute retroviral syndrome. A 41-year-old black man presented with nephrotic range proteinuria, renal insufficiency, and acute gastrointestinal and pulmonary symptoms. He recently had been treated for primary syphilis. Two HIV serologic tests, performed 3 months apart, were negative. Renal biopsy was consistent with HIVAN. After the biopsy, the patient was discovered to have more than 700,000 viral copies per mL in his blood. CD4( ) count was greater than 500/mm(3). Six weeks later, enzyme-linked immunosorbent assay and Western blot analyses for HIV antibody became positive. HIVAN can occur early in the course of HIV infection, even during acute infection before seroconversion, and prolonged exposure to virus is not necessary for this renal involvement to occur in the susceptible host.
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ranking = 5
keywords = nephropathy
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6/28. legionella micdadei lung abscess in a patient with HIV-associated nephropathy.

    A patient with end-stage renal disease due to human immunodeficiency-associated nephropathy developed fever, cough and chest pain over a week's duration. He was diagnosed with lung abscess and started on antibiotic coverage. He underwent bronchoscopy because of progression of his illness and persistent fever and bronchoalveolar lavage culture isolated legionella micdadei. In spite of appropriate antibiotic therapy, the patient remained febrile for 10 days, necessitating chest tube drainage. After a 6-week course of antibiotics and drainage, the patient made an uneventful recovery. Infections due to L. micdadei may be hard to diagnose because of difficulties in isolating this bacteria.
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ranking = 5
keywords = nephropathy
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7/28. Treatment of HIV-associated nephropathy.

    HIV-associated nephropathy (HIVAN) is the most common cause of renal failure in patients infected with type 1 human immunodeficiency virus (hiv-1). The renal prognosis for HIVAN is poor and is typically associated with rapid progression to renal death. We report a patient with biopsy-proven HIVAN who was successfully treated with corticosteroids and review the currently available evidence supporting the specific treatments of this condition. A 34-year-old African-American male with a 2-year history of uncomplicated HIV disease developed progressive azotemia despite treatment with highly active antiretroviral therapy (HAART). He was treated with an uncomplicated 4-month course of prednisone, which improved his serum creatinine from 2.9 to 1.9 mg/dl and decreased his degree of proteinuria from 8 to 2.1 g/day. Two years post-steroid treatment his renal function remains stable. Increasing evidence supports that both ACE inhibitors and HAART are effective in preventing and in some cases of reversing HIVAN induced renal failure. In selected patients who progress despite these measures, a limited course of corticosteroid may achieve long-standing disease remissions. In general, with adequate supervision, corticosteroid therapy appears to be well tolerated and has an acceptable side effect profile. Although persuasive in view of the abysmal natural history of HIVAN, the currently available studies are subject to major methodological limitations. Appropriate randomized controlled trials are urgently required in order to further examine the efficacy, optimal timing, and potential side effects of these treatments.
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ranking = 5
keywords = nephropathy
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8/28. Asymptomatic HIV patient with cardiomyopathy and nephropathy: case report and literature review.

    We report a previously asymptomatic HIV patient with high cd4 lymphocyte count and low HIV1 viral load who developed cardiac and renal disease. Management with ACE inhibitor, diuretics and triple antiretroviral combination therapy yielded a rapid clinical response. An understanding of the spectrum of renal and cardiac derangements is essential for clinicians in managing patients with HIV disease.
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ranking = 4
keywords = nephropathy
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9/28. diagnosis and treatment of HIV-associated nephropathy.

    Human immunodeficiency virus-associated nephropathy (HIVAN) is a distinct clinico-pathological syndrome that occurs almost exclusively in black patients with an AIDS defining diagnosis. It is characterized by rapidly progressive renal failure with a severe nephrotic syndrome. The renal biopsy typically shows a collapsing glomerular sclerosis and variable tubulo-interstitial nephritis. The pathogenesis most likely involves infection of renal tubular and epithelial cells with HIV. The use of ACE-inhibitors and steroids may slow down the progression to end-stage renal failure. With the introduction of highly active anti-retroviral therapy, HIVAN may now be treated effectively although clinical data are so far limited to case-reports.
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ranking = 5
keywords = nephropathy
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10/28. Resolution of renal failure after initiation of HAART: 3 cases and a discussion of the literature.

    Renal failure is a known complication of HIV infection. The most common form is HIV-associated nephropathy, or HIVAN. It is characterized by high-grade proteinuria with rapid progression to end-stage renal disease. The kidneys of affected patients appear enlarged on ultrasonography. Histopathologically, there is focal segmental glomerulosclerosis with glomerular collapse. Before the era of HAART, patients with HIVAN had limited survival, although in some cases this was prolonged if dialysis was instituted. Over the past few years, isolated case reports have shown that patients with HIVAN will recover renal function following initiation of HAART. We report 3 patients believed to have HIVAN who exhibited marked improvement in renal function after treatment with a regimen comprising 2 nucleoside reverse transcriptase inhibitors and a protease inhibitor.
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ranking = 1
keywords = nephropathy
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