Cases reported "Glomerulonephritis"

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1/17. Laparoscopic renal biopsy in bilateral pelvic kidney with chronic glomerulonephritis.

    Percutaneous needle biopsy under the guidance of ultrasound or computerized tomography is the most valuable method in the diagnosis of parenchymal kidney diseases. However, sometimes it can be difficult to perform in the presence of certain anomalies, anatomic variations, or medical problems. In the presence of bilateral pelvic kidney, which is a rare anomaly, laparoscopy can be used to obtain biopsy. biopsy of kidney was planned in a 26-year-old woman who presented with a history of hypertension for 7 years and proteinuria with the diagnosis of nephrotic syndrome. For the biopsy, the laparoscopic approach was chosen since the patient had bilateral pelvic kidneys. Under general anesthesia, using three port sites, the right kidney was reached, which was located more anterior than the left one. Three biopsy specimens for histologic evaluation were taken with a Tru-Cut biopsy needle. No complications were encountered during or after the operation. The patient was ceased from urological follow-up after performing an ultrasound on the first postoperative day. This is the first case of pelvic kidney with chronic glomerulonephritis reported in the literature in which the histologic diagnosis was made with the help of laparoscopy. Laparoscopic kidney biopsy is a minimally invasive technique that can be done in cases with anatomic variations, making percutaneous needle biopsy impossible.
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2/17. Fibrillary glomerulonephritis: early diagnosis associated with steroid responsiveness.

    patients with fibrillary glomerulonephritis usually present with nephrotic proteinuria, microscopic hematuria, impaired renal function, and hypertension; 50% develop end-stage renal disease within a few years. There is no known effective therapy for fibrillary glomerulonephritis. We describe three patients with biopsy-proven fibrillary glomerulonephritis, in whom pathognomonic fibrillar deposits of 20-nm diameter were seen by electron microscopy. All patients had nephrotic syndrome and normal renal function at the time of diagnosis. They were treated initially with prednisone, 1 mg/kg body weight, then tapered individually according to the clinical course. Additional therapy consisted of an angiotensin-converting enzyme inhibitor and diuretics. proteinuria disappeared in two of three patients after 9 and 12 months of therapy. In one patient, proteinuria was reduced from 7.49 mg/mg creatinine to 0.63 mg/mg creatinine after 6 months of treatment. The kidney function remained normal in all three cases. Two patients are now free of steroid therapy for 9 and 6 months. They show no signs of recurrence of kidney disease and have normal renal function without significant proteinuria. Steroid therapy in patients with biopsy-proven fibrillary glomerulonephritis, starting with prednisone, 1 mg/kg body weight, and tapered individually according to the clinical course, is a promising strategy. Early start of treatment in patients with preserved renal function seems to be crucial for a favorable outcome.
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3/17. A case of chronic relapsing ANCA-associated microscopic polyangiitis successfully treated with plasma exchange.

    We report the case of a 4.5-year-old girl with microscopic polyangiitis (MPA) manifesting antineutrophil cytoplasmic autoantibody (ANCA)-positive necrotizing crescentic glomerulonephritis and pulmonary hemorrhage. She was initially on induction therapy with corticosteroids and azathioprine. plasma exchange (PE) combined with immunosuppressants was used to treat an episode of recurrent pulmonary hemorrhage, and achieved remission. At 9.8 years of age her kidney disease relapsed, associated with renal dysfunction and increased proteinuria. To minimize the toxic effects of immunosuppressants, she was treated with PE again, and her renal dysfunction resolved. plasma exchange was effective in reducing the risk of death and preserving long-term renal function without the severe adverse effects of immunosuppressants. Our preliminary results indicate that PE is likely to be a treatment option for children in acute phase of ANCA-associated MPA, who should be protected from the toxic effects of immunosuppressants.
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4/17. Psoriatic nephropathy--does an entity exist?

    psoriasis is an immune-mediated chronic inflammatory disorder of the skin. association with kidney disease has been debated for a long time. Secondary renal amyloidosis in psoriatic arthropathy and drug-induced renal lesions secondary to methotrexate or cyclosporine are accepted accompaniments of psoriasis. IgA nephropathy is also known to occur in psoriatics. We report three interesting cases of renal involvement in long-standing established psoriasis on topical therapy alone. The patients presented with hypertension, significant proteinuria, hypoalbuminemia, and dyslipidemia. Kidney biopsies revealed "mesangioproliferative glomerulonephritis with IgA nephropathy," "focal proliferative glomerulonephritis," and "membranous glomerulonephropathy." The former two had marked active urinary sediment. patients improved on prednisolone and angiotensin-converting enzyme inhibitors. Contrary to the belief that renal involvement in psoriasis is coincidental, we propose that kidney disease may be a common accompaniment of psoriasis, which may be labeled as "psoriatic nephropathy" or "psoriatic kidney disease." The exact mechanism of this entity is yet to be elucidated.
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5/17. High-dose intravenous IgG treatment and renal function.

    In an open trial of high dose intravenous IgG (IVIG) treatment in nephrotic patients with glomerulonephritis, the first six patients so far studied showed a transient rise in plasma creatinine. This increase was not associated with any symptoms and the urinary deposit remained unchanged. Two other patients with pre-existing renal impairment but without nephrotic syndrome had a transient and reversible rise in plasma creatinine immediately after IVIG. These observations suggest that high-dose IVIG infusion can produce short-lived disturbances in renal function in patients with kidney diseases.
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6/17. The kidney disease of Crow-Fukase (POEMS) syndrome: a clinico-pathological study of four cases.

    We studied four cases of Crow-Fukase syndrome with renal dysfunction. Kidney specimens obtained by needle biopsy showed glomerular lesions resembling those seen in conditions characterized by microangiopathy. Common glomerular findings by light microscopy were mesangial expansion and narrowing of the capillary lumina. An enlarged subendothelial space and mesangial area with deposition of amorphous material as well as swelling and vacuolization of endothelial cells were observed by electron microscopy. In an active phase, severe mesangial edema and segmental mesangiolysis, and in a late stage, mesangial cell interposition and sclerosis were seen. Tests by immunofluorescence microscopy for the presence of immunoglobulins A, M, G, lambda and kappa light chains, C3, and C4 were negative. Decay accelerating factor was found in glomeruli and in the vascular pole. Other findings included lymph node angiosclerosis, peripheral nerve microangiopathy and hemangioma formation with endothelial cell proliferation. These observations suggest that chronic endothelial injury constitutes the basic pathology of Crow-Fukase syndrome. Hemodialysis was required to manage anasarca in three of the patients although serum creatinine levels were below 5.0 mg/dl. urinalysis revealed mild abnormalities and did not reflect the severity of the glomerular lesion. Corticosteroids given to three of the patients were effective in controlling fever and the lymphadenopathy; in two cases the corticosteroids induced a recovery of renal function. Thus Crow-Fukase syndrome may be due to chronic endothelial injury; the clinical symptoms and renal involvement respond to corticosteroid therapy.
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7/17. nephrotic syndrome and rapid renal failure in autosomal dominant polycystic kidney disease.

    A 44-year-old man, with autosomal dominant polycystic kidney disease and hypertension under satisfactory control, developed nephrotic syndrome with negative serology. Open renal biopsy revealed focal glomerular sclerosis. Prior to the appearance of heavy proteinuria, serum creatinine was 1.7 mg/dl. After the nephrotic syndrome had been established, renal function deteriorated rapidly and hemodialysis was started within 2.6 years. In patients with autosomal dominant polycystic kidney disease, the appearance of nephrotic range proteinuria along with a rapid decline in renal function indicates the presence of a glomerular lesion, which needs to be investigated by renal biopsy.
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8/17. Rapidly progressive glomerulonephritis undetected by routine school urinalysis: a case report.

    A twelve year old girl with rapidly progressive glomerulonephritis (RPGN) is reported. First symptoms of renal failure developed in August 1987 just five months after a routine school urinalysis which detected no abnormalities. The patient developed end stage renal failure within 2 months of onset. Continuous ambulatory peritoneal dialysis (CAPD) was introduced to manage the renal failure, and open renal biopsy was performed simultaneously in order to elucidate the underlying renal disease in this patient. Microscopic examination of the biopsy specimen demonstrated marked formation of crescents in about 90% of the glomeruli, and some of the glomeruli were already sclerotic. Granular depositions of IgG(trace), IgA(1 ), IgM(3 ), properdine(3 ), Clq(3 ) and C3(3 ) were observed by immunofluorescence staining. A definite diagnosis of rapidly progressive glomerulonephritis was made as the cause of renal failure in this patient. It was suggested that open renal biopsy is useful in diagnosing the underlying kidney disease of end stage renal failure in cases where the clinical course is relatively short.
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9/17. adult hemolytic uremic syndrome associated with nonimmune deposit crescentic glomerulonephritis and alveolar hemorrhage.

    An 18-year-old girl presented with anuric rapidly progressive glomerulonephritis associated with hypoxemic respiratory failure due to diffuse lung hemorrhage. The course of the kidney disease terminated in end-stage renal failure. The patient had been on permanent hemodialysis since her first admission; episodes of lung hemorrhage recurred five times over a follow-up period of 8 months. Two months after her first admission, the patient manifested features suggestive of the hemolytic uremic syndrome, namely, microangiopathic hemolytic anemia, thrombocytopenia, and excessive hypertension complicated by hypertensive encephalopathy. Based on the sequence of events in the course of this patient's disease, the authors believe that the hematological abnormalities were superimposed on the glomerular disease rather than being a primary disorder. Possible mechanisms for the association of the two disorders are discussed.
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10/17. Regression of acquired cystic disease of the kidney after successful renal transplantation.

    The effect of renal transplantation on acquired cystic disease of the kidney in patients who have been on hemodialysis for more than 5 years was examined in 7 cases by computer-assisted tomography (CT scan). Almost all acquired cysts disappeared, and the size of the original kidneys decreased remarkably in 2 cases 8--10 months after transplantation. 3 other patients, in whom CT scans were performed only after transplantation, showed contracted scarred kidneys with few or no cysts. The involution of acquired cysts was incomplete in 1 case, in whom the cysts persisted for 3 years and 2 months, despite normal renal function. The last case, who was off hemodialysis only for 4 months, exhibited enlargement of the original kidneys and an increase in the number of cysts. These results suggest that a significant number of acquired renal cysts in dialyzed patients with end-stage kidney disease regress rapidly after successful renal transplantation, but this is not always so.
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