1/21. recurrence of focal segmental glomerulosclerosis associated with Kimura's disease after kidney transplantation.A 13-year-old Brazilian boy with Kimura's disease (eosinophylic lymphoid granuloma) and nephrotic syndrome is reported. Native kidney biopsy showed focal segmental glomerulosclerosis (FSGS). Treatment with prednisolone resulted in partial remission of proteinuria, and he had a progressive loss in renal function, requiring initiation of chronic dialysis, which he underwent for 46 months. After kidney transplantation, the patient developed proteinuria. A renal biopsy showed recurrence of focal segmental glomerulosclerosis, and subsequently he developed renal insufficiency.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
2/21. Steroid-sensitive nephrotic syndrome associated with Kimura disease.We report an 11-year-old Japanese boy with Kimura disease and associated nephrotic syndrome. Before the diagnosis of Kimura disease was established, the patient had three episodes of swelling on the left cheek with subsequent nephrotic syndrome. steroids were effective for both conditions. However, both conditions recurred within months of discontinuation of steroids. For the fourth episode of swelling on the left cheek, cyclosporine (CsA) was used. The subcutaneous tumor responded to CsA and disappeared within a few days. There has been no subsequent relapse of the nephrotic syndrome to date.- - - - - - - - - - ranking = 7keywords = nephrotic syndrome (Clic here for more details about this article) |
3/21. nephrotic syndrome associated with Kimura disease.Kimura disease presents as benign subcutaneous swelling predominantly around the head and neck region. It has a high incidence of renal involvement. However, the pathogenesis of this association remains elusive. Only 2 pediatric cases and 11 adult cases of Kimura disease with renal involvement have been reported in the literature. In recent years many immunopathogenetic features suggesting an underlying T-cell and related cytokine defect have been noted in Kimura disease. We describe a unique case of an Asian boy who presented with nephrotic syndrome resistant to steroid and cytotoxic therapy, and 5 years later developed cervical lymphadenopathy consistent with Kimura disease. We also review the literature, summarizing the presentation, differential diagnosis, incidence of renal disease, prognosis, immunopathogenetic features, and therapy.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
4/21. Steroid-sensitive anemia in a boy on dialysis--an association with Kimura disease.The cause of hypereosinophilia (EO) in hemodialysis (HD) patients is multifactorial and is felt to be a benign laboratory abnormality related to dialysis or uremia. Kimura disease (KD) is an angiolymphoid proliferative disorder of unknown etiology. Many cases are associated with nephrotic syndrome in children; however, it has seldom been reported in children on dialysis. We report here a 13-year-old boy who developed persistent EO and subsequent anemia after maintenance HD; he later developed KD. The atypical clinical manifestation of KD and its relationship to HD and erythropoietin hyposensitive anemia in this patient are discussed.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
5/21. Steroid-resistant nephrotic syndrome associated with Kimura's disease.Kimura's disease is a chronic inflammatory disease characterized by tumor-like lesions in the soft tissue and lymph nodes of head and neck area or parotid gland. It has a high frequency of an association with nephrotic syndrome. Reported cases of nephrotic syndrome and Kimura's disease were mostly from adult patients with only 5 children mentioned. This study reports the case of a 15-year-old-boy who manifested with steroid-resistant nephrotic syndrome for 4 years. Pathological examination of the kidney revealed mild mesangial proliferation. Subsequently, he developed a soft-tissue mass in the parotid gland area. Histopathological investigation of the mass revealed eosinophilic infiltration together with plasma cells and mast cells which is a main characteristic of Kimura's disease. The patient, however, continued to have nephrotic-range proteinuria after removing the subcutaneous mass.- - - - - - - - - - ranking = 7keywords = nephrotic syndrome (Clic here for more details about this article) |
6/21. Recurrent angiolymphoid hyperplasia with eosinophilia mimicking temporal arteritis associated with nephrotic syndrome.We report on a middle-aged Caucasian male who presented with nephrotic syndrome that on 2 consecutive recurrences was accompanied by a pulsating tumor suggesting temporal arteritis. Renal biopsies showed features of a low-grade mesangial-proliferative glomerulonephritis. The resected tumor in the temporal region revealed a lesion consistent with angiolymphoid hyperplasia with eosinophilia (ALHE), with moderate inflammatory involvement of the temporal artery. The patient was successfully treated with oral prednisolone in addition to removal of the tumor, but has remained steroid-dependent. To our knowledge, only 2 cases of ALHE and nephrotic syndrome have been reported so far in non-Japanese individuals [Altman et al. 1995, Sonkodi et al. 1987], and we are not aware of any previous case combining these features while simultaneuosly mimicking temporal arteritis.- - - - - - - - - - ranking = 6keywords = nephrotic syndrome (Clic here for more details about this article) |
7/21. Kimura disease in a patient with renal allograft failure secondary to chronic rejection.Recent studies suggest that CD4( )T helper 2 (Th2) cell proliferation and overexpression of Th2 cytokines may play an important role in the development of Kimura disease. Chronic rejection of a renal allograft by the indirect allo recognition pathway is also induced by Th2 cytokines. We report a 12-year-old boy who had presented with nephrotic syndrome 10 years previously. He was found to have focal segmental glomerulosclerosis, which was attributed to vesicoureteral reflux, and he underwent renal transplantation at the age of 5 years. Allograft dysfunction secondary to chronic rejection was noted by 2 years post transplant, after which continuous ambulatory peritoneal dialysis was instituted. After discontinuation of immunosuppressive therapy, he progressively developed peripheral eosinophilia and eczema, followed by cervical lymphadenopathy and then epitrochlear lymphadenopathy. Kimura disease was diagnosed on lymph node biopsy. Our patient demonstrated that Kimura disease can occur after renal allograft failure secondary to chronic rejection. Both disorders involve the Th2-dominant immune response, according to previous observations.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
8/21. Kimura disease with advanced renal damage with anti-tubular basement membrane antibody.Kimura disease (KD) is an autoimmune eosinophilic granulomatous disorder with generalized lymphadenopathy. A handful of pediatric patients with renal disease have been described, none of whom have been African-American (AA). We present an AA boy with KD and nephrotic syndrome (NS). Two months after stopping steroids, fever, asthma, eczema, and proteinuria recurred. His NS did not relapse but his platelet count decreased to 51,000/microl (x10(6)/l). On restarting prednisone, his platelet count normalized. A kidney biopsy revealed 23 of 37 glomeruli obsolescent and advanced damage with over 50% of cortical tissue replaced by interstitial fibrosis and chronic inflammation. Glomerular immunofluorescence was largely negative; very intense linear anti-tubular basement membrane (TBM) deposits of IgA, IgG, C3, and C4 were noted. At present, 36 months from onset, serum creatinine is 1.2 mg/dl (106 micromol/l). We present a 4-year-old AA boy with KD, NS, relapsing thrombocytopenia, and renal damage with anti-TBM antibody.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
9/21. Long-term effects of steroid treatment on nephrotic syndrome associated with Kimura's disease and a review of the literature.A 68-year-old Japanese male (Case 1) and a 59-year-old Japanese male immigrant to brazil (Case 2) who suffered from subcutaneous eosinophilic lymphoid granuloma (Kimura's disease) of several years duration, developed nephrotic syndrome. Renal biopsy demonstrated membranous nephropathy in Case 1 and minimal-change lesion in Case 2. Both patients were treated with prednisolone for several months. There was complete remission of nephrotic syndrome in eight years in Case 1 and in three months in Case 2. proteinuria did not reappear after remission of nephrotic syndrome in either case. In contrast subcutaneous tumors subsided in both cases during steroid treatment but again became active immediately following discontinuation of the drug. These clinical observations suggest that, though some common factor(s) might mediate the development of both skin and renal lesions, the mechanism of action on these two target organs may differ, perhaps serving only as a "trigger" for the initiating mechanism of renal disease.- - - - - - - - - - ranking = 7keywords = nephrotic syndrome (Clic here for more details about this article) |
10/21. vincristine treatment of nephrotic syndrome complicated by Kimura disease.Kimura disease is a rare inflammatory condition of unknown aetiology. It typically presents in young Asian males with the triad of non-tender subcutaneous swellings in the head and neck region, peripheral eosinophilia and raised serum IgE. About 16% of cases have associated renal disease. We present the case of a 10-year-old boy with a past history of steroid responsive, frequently relapsing nephrotic syndrome who developed a right submandibular swelling and eosinophilia. Kimura disease was diagnosed on the basis of clinical and histological findings. The condition recurred during relapses of nephrotic syndrome. Because of poor adherence with oral medication, our patient was treated with intravenous vincristine with synchronous remissions of his nephrotic syndrome and Kimura swellings on each occasion.- - - - - - - - - - ranking = 7keywords = nephrotic syndrome (Clic here for more details about this article) |
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