Cases reported "Nephrotic Syndrome"

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1/22. foscarnet-induced crystalline glomerulonephritis with nephrotic syndrome and acute renal failure after kidney transplantation.

    foscarnet nephrotoxicity has been reported to be associated with acute tubulointerstitial nephritis. Crystals in glomerular capillary lumens have also been observed in patients with acquired immunodeficiency syndrome who were treated with foscarnet for cytomegalovirus disease. We describe a kidney transplant recipient who developed a nephrotic syndrome with microscopic hematuria and nonoliguric acute renal failure within 15 days after starting foscarnet therapy for cytomegalovirus infection. A kidney biopsy specimen showed the presence of crystals in all glomeruli and in proximal tubules. Fourier transform infrared microscopy analysis demonstrated that crystals were made from several forms of foscarnet salts: mixed calcium and sodium salts, and unchanged trisodium foscarnet salts. Renal function and proteinuria spontaneously improved, and a second transplant biopsy performed 8 months after the first one revealed fibrotic organization of half of the glomeruli and of interstitial tissue, and crystal vanishing. We were thus able to provide proof of the possible precipitation of foscarnet in a transplanted kidney.
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2/22. Fibrillary-immunotactoid glomerulopathy with renal deposits of IgAlambda: a rare cause of glomerulonephritis.

    We describe a 24-year-old patient who presented with a nephrotic syndrome. His renal biopsy revealed a diffuse mesangioproliferative glomerulonephritis with eosinophilic deposits. Electron microscopy showed organized, congo-red negative deposits, forming microtubules of about 20 nm width in the capillary walls and in the mesangium, establishing a diagnosis of fibrillary-immunotactoid glomerulopathy. Fibrillary-immunotactoid glomerulopathy is a rare cause of glomerulonephritis, characterized by congo-red-negative glomerular deposits of fibrils, sometimes organized in microtubules, predominantly containing IgG and C3. patients clinically present with the nephrotic syndrome, hematuria and hypertension. The pathogenesis of this glomerulopathy has not been elucidated yet. In our patient, the renal deposits contained IgAlambda. This peculiar feature is suggestive of an underlying paraproteinemia. However, in the serum no paraproteins or cryoglobulins were found, and also microscopical examination and immunophenotyping of the bone marrow did not point to the presence of a monoclonal plasma cell dyscrasia. Our patient was not treated with immunosuppressive drugs and he is currently progressing to end-stage renal disease.
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3/22. Congenital nephrotic syndrome.

    A female infant born at 34 weeks' gestation after several days of ruptured membranes had a Potter-like face and compression-induced limb posture consistent with oligohydramnios. Oedema developed on day 2; initial investigations showed massive proteinuria, hypoalbuminaemia, hyponatraemia, acidosis and marked renal insufficiency. The infant was intubated and despite albumin infusion and intravenous antibiotics she became oligoanuric by day 8 and required haemofiltration. Renal biopsy at this stage showed cystic dilatation of tubules in the cortex and glomerular lesions consisting of shrunken tufts with sclerotic centres and a corona of epithelial cells at the periphery. Due to a very poor prognosis treatment was withdrawn. Postmortem examination of the kidneys confirmed the histological diagnosis of diffuse mesangial sclerosis. Genetic studies found no mutations in WT1 and NPHS1 genes although the entire genes could not be screened for mutations due to lack of dna.
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4/22. Steroid responsive nephrotic syndrome associated with bilateral renal dysplasia.

    Renal dysplasia is characterized by hypoplastic kidneys that contain elements of primitive tubules. patients may develop end-stage renal failure early in life. nephrotic syndrome is one of the most common renal diseases in childhood and may occur in association with renal dysplasia. We report a case of a child with bilateral dysplastic kidneys and steroid responsive nephrotic syndrome (SRNS). An association between renal dysplasia with chronic renal failure and SRNS has not previously been reported in the English literature.
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5/22. Galloway-Mowat syndrome: a glomerular basement membrane disorder?

    We report a female infant with Galloway-Mowat syndrome. In addition to the characteristic dysmorphic appearance, neurological anomalies and early-onset nephrotic syndrome, she had arachnodactyly, an observation thus far reported uniquely in taiwan. Also, her elder sister had the same condition. Renal pathology on light microscopy showed cystic dilatation of the renal tubules. Electron microscopy showed an irregular glomerular basement membrane and effacement of foot processes. This observation suggests that malformation of the glomerular basement membrane may cause the glomerulopathy in Galloway-Mowat syndrome.
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6/22. Dysplasia of tubular cells in a kidney- transplanted patient treated with recombinant human growth hormone.

    After renal transplantation (Tx), children with growth retardation can be successfully treated with recombinant human growth hormone (rhGH). However, the impact of this treatment on kidney allograft function remains a source of concern. We report on one boy who received a cadaveric kidney transplant at 12 yr of age, after developing end-stage focal and segmental glomerulosclerosis and hyalinosis. The early post-transplant period was complicated by thrombosis of an arterial branch of the graft and two acute rejection episodes. Because of poor growth, the boy was treated with rhGH starting 22 months after the Tx. The renal function remained relatively stable for 22 months after initiation of rhGH therapy and then progressively deteriorated over a period of 10 months, with the patient ending up on dialysis. Several biopsies, performed for rejection episodes or before the start of rhGH, or to elucidate the deterioration of the renal function, were analyzed. Histologically, a progressive increase in the amount of hypertrophy of the tubules and of the glomeruli was seen after initiation of rhGH. hyperplasia of the tubular epithelium with crowding of cells of the proximal tubules, hyperchromasia and irregularities in the shape of the nuclei, and abrupt changes of chromatism along the tubuli, were also observed. These lesions of tubular dysplasia are extremely unusual in transplanted kidneys and are unlikely to be caused by compensatory hypertrophy secondary to destruction of renal tissue. They may be an effect of rhGH treatment. The prognostic significance of these lesions is unknown but merits attention.
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7/22. nephrotic syndrome associated with fanconi syndrome. Immunopathogenic studies of tubulointerstitial nephritis with autologous immune-complex glomerulonephritis.

    The nature of renal lesions in a patient with simultaneous onset of the fanconi syndrome and nephrotic syndrome was investigated by immunologic studies of the patient's serum, cryoproteins, and renal tissue. Acute severe tubulointerstitial nephritis and generalized segmental glomerulonephritis were present. Renal tubular epithelial (RTE) antigen, IgG, and Clq were localized in the glomerull and proximal tubules. Cryoprecipitates containing RTE antigen and anti-RTE antigen were isolated from the patient's serum antibody to RTE antigen was detected in the serum of the patient. However, antibody to tubular basement membrane was not found in the cryoproteins or serum. The unusual simultaneous presentation of these two syndromes in our patient possibly represents a common etiology: tubular damage with release of RTE antigen and subsequent development of immune-complex glomerulonephritis mediated by renal tubular epithelial antigen and antibody to this antigen.
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8/22. A case of atypical congenital nephrotic syndrome.

    We present a female newborn with the nephrotic syndrome of intrauterine onset and a unique set of extrarenal abnormalities, as well as atypical renal lesions. The extrarenal anomalies comprised a soft tissue hemangioma in the frontotemporal region, unilateral microphthalmia (with persistent hyperplastic corpus vitreous and detachment of the retina), and glaucoma in the other eye. Immature glomeruli and/or glomeruli with large cellular crescents were found in renal biopsy specimens in the 3rd week of life. On autopsy, 7 weeks later, diffuse mesangial sclerosis (DMS) was the predominant type of glomerular lesion. In addition, dilations of tubules, forming microcysts, as well as clusters of infiltrating cells in the interstitium, were found both in renal biopsy and autopsy specimens. Although the symptoms observed in our patient did not match any reported in association with the known forms of the congenital nephrotic syndrome (CNS), the most probable diagnosis seemed to be CNS due to DMS of intrauterine onset, with superimposed drug-related tubulointerstitial nephritis.
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9/22. An adult with acute poststreptococcal glomerulonephritis complicated by hemolytic uremic syndrome and nephrotic syndrome.

    We report the case of a 47-year-old man with the simultaneous occurrence of clinical and laboratory features consistent with acute poststreptococcal glomerulonephritis (APSGN), hemolytic uremic syndrome (HUS), and nephrotic syndrome. Acute nephritic syndrome occurred 3 weeks after having pharyngeal pain and diarrhea. He presented with edema and hypertension on admission. Laboratory evaluation showed hemolytic anemia with fragmentation, thrombocytopenia, elevated lactic dehydrogenase level, low haptoglobin level, low complement c3 level, and elevated antistreptolysin-O titer. serum creatinine level was 1.22 mg/dL (108 micromol/L), and urinalysis showed marked proteinuria, with protein of 8.7 g/d, and hematuria. The renal biopsy specimen was characteristic of APSGN, but not HUS. Moderate expansion of the mesangial matrix, moderate proliferation of epithelial and endothelial cells, and marked infiltration of neutrophils was seen by means of light microscopy, and many subepithelial humps were seen by means of electron microscopy. Neither fibrin deposition nor evidence of thrombotic microangiopathy was found. complement c3 deposition along the capillary wall and tubules was seen in an immunofluorescence study. The patient was administered plasma infusion at 320 mL/d and antihypertensive drugs. serum complement c3 and haptoglobin levels returned to normal within 3 weeks. This is a rare case of the simultaneous occurrence of APSGN, HUS, and nephrotic syndrome.
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10/22. A membranous nephropathy associated with adult polycystic kidney disease.

    A 53-year-old woman with adult polycystic kidney disease (PKD) developed a nephrotic syndrome. Evaluation of the renal biopsy specimens showed typical findings of membranous nephropathy (MN). There are few reports of nephrotic syndrome associated with PKD and only one proved to be MN. The possible mechanism of the association of PKD with MN was evaluated. autoantibodies against the brush border were not detected in this patient's serum by indirect immunofluorescence. Three monoclonal antibodies against the tubular brush border and epithelial cell of distal tubulus did not react with subepithelial deposits in the biopsy specimen. Therefore tubular brush border antigen which was reported to induce membranous nephropathy was not detected in the immune complexes deposited in the glomeruli. So we could not determine any direct relationship between PKD and MN. The reaction of antibodies against basement membrane components (type IV collagen, laminin, fibronectin, heparansulfate proteoglycan) with the basement membranes of the cysts was evaluated by indirect immunofluorescence. The reaction with anti-heparansulfate proteoglycan antibody was decreased compared with normal tubular basement membrane. The reactivity to anti-fibronectin antibody was remarkably increased in the cystic walls, tubules, and interstitium. Changes of tubular basement membrane antigens was observed in PKD.
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