Cases reported "Kidney Failure, Chronic"

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1/156. Focal segmental glomerulosclerosis: a need for caution in live-related renal transplantation.

    Focal segmental glomerulosclerosis (FSGS) has increasingly been recognized to occur in a familial pattern. We have observed the development of biopsy-confirmed FSGS and subsequent end-stage renal disease (ESRD) in one live related kidney donor and ESRD without biopsy in another. Both donors had family members with ESRD secondary to FSGS. Both donors were apparently healthy by routine physical examination, urinalysis, and serum creatinine at the time of evaluation as live related donors. We believe these cases emphasize the need for great caution when evaluating siblings as potential live related donors.
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2/156. Development of focal segmental glomerulosclerosis in the renal allograft of a patient with lupus.

    Nephritis has been a recognized complication of systemic lupus erythematosus since the early 1900s. Almost all lupus patients have some degree of renal involvement related to their condition, but a considerably smaller proportion of these patients actually progress to end-stage renal disease (ESRD). However, lupus patients are also susceptible to other primary renal insults that may significantly contribute to the deterioration in their renal function. We present a case of a patient with clinical and pathological evidence of lupus nephritis that progressed to ESRD and subsequently developed "recurrent" focal segmental glomerulosclerosis in her transplant kidney. Retrospective clinicopathologic correlation suggested the possibility of more than 1 primary renal process that eventually led to her dialysis-dependent state. This case illustrates the importance of meticulously examining both clinical and renal biopsy data in patients with lupus nephritis and considering the presence of co-existing renal pathologies to resolve an otherwise discordant picture of disease progression. These considerations may have important therapeutic and prognostic implications.
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3/156. nephrotic syndrome and end-stage renal disease with WT1 mutation detected at 3 years.

    We report a boy who presented at 3 years with nephrotic syndrome and end-stage renal failure. Although histopathological findings showed end-stage kidney, isolated diffuse mesangial sclerosis (IDMS) was suspected because of his clinical course, and was confirmed by the presence of WT1 (wilms tumor suppressor gene) mutation. He did not have ambiguous genitalia or wilms tumor. The karyotype was 46:XY. A constitutional mutation in exon 7 (953G-->A, 312Arg-->Gin) was detected. A few cases of male IDMS, associated with WT1 mutations, have been reported. We believe that investigation for the WT1 mutation should be performed not only in denys-drash syndrome and IDMS, but also in end-stage renal disease with unexplained nephrotic syndrome of early onset. WT1 mutation-associated nephrotic syndrome has an increased risk of wilms tumor. Careful ultrasound evaluations or bilateral nephrectomies are indicated.
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4/156. Nonocclusive mesenteric ischemia in a patient on maintenance hemodialysis.

    Nonocclusive mesenteric ischemia (NOMI) is known to occupy about 25% to 60% of intestinal infarction. NOMI has been reported to be responsible for 9% of the deaths in the dialysis population and the postulated causes of NOMI include intradialytic hypotension, atherosclerosis and medications, such as diuretics, digitalis and vasopressors. Clinical manifestations, such as fever, diarrhea and leukocytosis, are nonspecific, which makes early diagnosis of NOMI very difficult. Case: A 66-year-old woman on maintenance hemodialysis for 5 years was admitted with syncope, abdominal pain and chilly sensation. Since 7 days prior to admission, blood pressure on the supine position during hemodialysis had frequently fallen to 80/50 mmHg. Four days later, she complained of progressive abdominal pain. Rebound tenderness and leukocytosis (WBC 13900/mm3) with left shift were noted. Stool examination was positive for occult blood. Abdominal CT scan showed a distended gall bladder with sludge. Under the impression of acalculous cholecystitis, she was operated on. Surgical and pathologic findings of colon colon were compatible with NOMI. Because of recurrent intradialytic hypotension, we started midodrine 2.5 mg just before hemodialysis and increased the dose up to 7.5 mg. After midodrine therapy, blood pressure during dialysis became stable and the symptoms associated with hypotension did not recur. CONCLUSION: As NOMI may occur within several hours or days after an intradialytic hypotensive episode, abdominal pain should be carefully observed and NOMI should be considered as a differential diagnosis. In addition, we suggest that midodrine be considered to prevent intradialytic hypotensive episodes.
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5/156. Metanephric adenoma with embryonal hyperplasia of Bowman's capsular epithelium: previously unreported association.

    We report a case of a 9-year-old boy with focal, segmental glomerulosclerosis who, following peritoneal dialysis, underwent renal transplantation and bilateral nephrectomy. The kidneys showed histological features of embryonal hyperplasia of Bowman's capsular epithelium, an uncommon lesion that is seen most often in patients with chronic renal failure who are being maintained on dialysis. In addition, a 1-cm tumor in the left kidney showed features of metanephric adenoma. Although both lesions are uncommon, they share many similarities on a morphological, immunohistochemical, and ultrastructural basis. This association has not been previously reported and may shed some light on the histogenesis of these recently described lesions.
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6/156. C1q nephropathy presenting as rapidly progressive crescentic glomerulonephritis.

    C1q nephropathy is an immune complex glomerulonephritis defined by the presence of mesangial immunoglobulins and complement deposits, most notably C1q, and the absence of clinical and laboratory evidence of systemic lupus erythematosus. histology in C1q nephropathy is characterized by a slight to severe increase in mesangial cellularity and matrix, with or without segmental sclerosis. C1q nephropathy usually presents with nephrotic-range proteinuria in older children and young adults, and has a poor response to steroids. patients may have decreased creatinine clearance at presentation, but progression to end-stage renal disease (ESRD) is slow. Severe crescentic glomerulonephritis has not been reported in C1q nephropathy. We describe a 3-year-old Hispanic girl who presented with renal insufficiency. Kidney biopsy showed C1q nephropathy with severe crescentic glomerulonephritis. The clinical and serological evaluation ruled out systemic lupus erythematosus or other immunological or infectious etiologies. In spite of immunosuppressive therapy, she progressed to ESRD within 14 weeks and is currently on chronic peritoneal dialysis. The atypical features of C1q nephropathy observed in our patient, which have not been described in earlier reports, are an early age of onset, severe crescentic glomerulonephritis, and rapid progression to ESRD. C1q nephropathy should be added to the differential diagnosis of glomerulonephritis in young children and in the patient with crescentic glomerulonephritis.
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7/156. AL-amyloidosis of the kidney initially presenting as minimal change glomerulonephritis.

    Small amounts of amyloid in kidney biopsy specimens may be missed on routine examination unless specifically targeted. Occasionally, this oversight results in a diagnosis of minimal change glomerulonephritis (MCGN). This misdiagnosis may be facilitated by the fact that typical "minimal changes" with flattening and effacement of the epithelial foot processes can be found in capillary loops directly affected by amyloid deposition as well as in capillary loops of glomeruli with only mild amyloid deposition in the mesangium. Repeatedly, the diagnosis of MCGN had to be corrected to renal amyloidosis when re-examination by special techniques succeeded in detecting even small amounts of amyloid fibrils. We present the case of a previously healthy 49-year-old man who suddenly developed nephrotic syndrome. A first renal biopsy showed MCGN. proteinuria remained refractory to immunosuppressive treatments, and creatinine clearance deteriorated rapidly. Two years later, a repeat renal biopsy showed AL-amyloidosis. In this case, re-examination of the first biopsy in the light of the final diagnosis again did not show any deposition of amyloid fibrils. We suspect that proteinuria and epithelial podocyte changes in amyloidosis are caused by factors other than deposition of amyloid fibrils itself. Possibly a cytokine release during the early fibril formation leads to abnormalities even before the typical structural changes of renal amyloidosis can be detected. This is analogous to the hypothesis of a circulating factor that leads to proteinuria in focal segmental glomerulosclerosis or the speculation of altered lymphokine expression associated with the development of MCGN in Hodgkin's disease.
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8/156. Renal cystic disease associated with tuberous sclerosis complex: renal failure treated by cadaveric kidney transplantation.

    Chronic renal failure in patients with tuberous sclerosis may be secondary to diffuse cystic disease, a lesion less common than the better known hamartomatous angiomyolipomas. uremia, in the case of a nineteen-year old female with end-stage renal disease, was associated with severely atrophic kidneys that contained numerous collapsed and scarred cysts. No hamartomas were present. The patient survived for more than three years following cadaveric renal transplantation.
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9/156. Hemolytic uremic syndrome associated with denys-drash syndrome.

    The denys-drash syndrome is defined by the occurrence of combinations of pseudohermaphroditism, nephrotic syndrome with diffuse mesangial sclerosis, Wilms' tumor, and constitutional mutations in the WT1 suppressor gene. Most patients develop end-stage renal failure. Atypical hemolytic uremic syndrome (HUS) is defined by onset of acute hemolytic anemia with fragmented erythrocytes, thrombocytopenia, and renal failure in the absence of a gastrointestinal prodromal illness of bloody diarrhea. The purpose of this report is to describe the occurrence of features of atypical HUS and denys-drash syndrome in two African-American boys aged 13 and 16 months. Each had nephrotic syndrome, diffuse mesangial sclerosis, and WT1 point mutations. Both had grade III hypospadias and undescended testes. They had normal serum creatinine concentrations and hematology a month before presenting with HUS. Stool cultures for escherichia coli o157:H7 were negative. Each patient has been transplanted with cadaver kidneys without recurrence of HUS.
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10/156. Purtscher-like retinopathy in nephrotic syndrome associated with mild chronic renal failure.

    A sudden loss of vision attributable to Purtscher-like retinopathy occurred in a 4-year-old boy with focal segmental glomerulosclerosis and nephrotic syndrome as well as mild chronic renal failure. This retinopathy was bilateral. After treatment with intravenous methylprednisolone, infusion of 20% albumin, and low molecular weight heparin (nadroparin calcium), his visual acuity improved within 3 days. Ischemic retinal blanching and hemorrhages gradually disappeared. The pathogenesis of this disorder is unknown.
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