RESUMO
Adrenocortical oncocytoma is a rare epithelial tumor only described in adults and in most cases, benign and non-functioning. Histologically characterized by cells with eosinophilic granular cytoplasm and ultra-structurally by the presence of numerous closely packed mitochondria. We report a case of adrenal oncocytoma developed in a 10-year-old girl who presented a left adrenal mass with low voice tone, excessive hair growth, and elevation of the plasma testosterone and dehydroepiandrosterone sulfate levels as a result of overproduction of adrenal steroid hormones. After the left adrenalectomy, her hormone levels were normalized.
Assuntos
Adulto , Criança , Humanos , Adenoma Oxífilo , Adrenalectomia , Citoplasma , Sulfato de Desidroepiandrosterona , Eosinófilos , Cabelo , Mitocôndrias , Plasma , Testosterona , Virilismo , VozRESUMO
PURPOSE: The association of mitochondrial DNA (mtDNA) mutations, deletions and copy number with progressive changes in patients with some glomerular disease and end-stage renal disease have been reported. In this study, we performed mtDNA mutation analysis in children with IgA nephropathy to investigate its role in progressive clinical course. METHODS: Seven children with IgA nephropathy were involved in this study. MtDNA isolated from platelet was amplified by PCR and sequenced entirely. RESULTS: The mean age at renal biopsy was 11.5+/-2.2 year and the mean age at latest evaluation was 17.9+/-3.2 year. The mean follow-up period were 7.8+/-3.1 years. Patients was divided into 2 groups according to the amount of proteinuria at presenting manifestation. Group 2 patients were nephrotic syndrome. Renal function reveals within normal range in all patients. In group 2 patients, the mean serum albumin level was significantly lower than those of group 1 (3.7+/-0.6 g/dL vs. 4.7+/-0.2 g/dL, P=0.0241) and the mean total cholesterol level was significantly higher than those of group 1 (222.7+/-35.7 mg/dL vs. 148.3+/-29.1 mg/dL, P=0.0283). In Group 2 patients, total amount of protein of 24 hour collected urine also significantly higher than those of group 1 (1,466.0+/-742.5 mg vs. 122.5+/-48.1 mg, P=0.0135). Pr/Cr ratio in random urine sample was also higher in group 2 than those of group 1 but the statistical significance was not noted (1.8+/-1.6 vs. 0.2+/-0.2, P=0.0961). Deletion of mtDNA nt 8272-8281 were observed in two patients, one patient in each groups, respectively. This is non-coding lesion. No patients demonstrated the mtDNA mutations. CONCLUSIONS: We have identified a deletion of mtDNA nt 8272-8281 in two children with IgA nephropathy. Further studies are needed to clarify the role of mitochondrial function in the progressive change of IgA nephropathy.
Assuntos
Criança , Humanos , Biópsia , Plaquetas , Colesterol , Complexo I de Proteína do Envoltório , DNA , DNA Mitocondrial , Seguimentos , Glomerulonefrite por IGA , Imunoglobulina A , Falência Renal Crônica , Mitocôndrias , Síndrome Nefrótica , Reação em Cadeia da Polimerase , Proteinúria , Valores de Referência , Albumina SéricaRESUMO
Adrenocortical oncocytoma is a rare epithelial tumor only described in adults and in most cases, benign and non-functioning. Histologically characterized by cells with eosinophilic granular cytoplasm and ultra-structurally by the presence of numerous closely packed mitochondria. We report a case of adrenal oncocytoma developed in a 10-year-old girl who presented a left adrenal mass with low voice tone, excessive hair growth, and elevation of the plasma testosterone and dehydroepiandrosterone sulfate levels as a result of overproduction of adrenal steroid hormones. After the left adrenalectomy, her hormone levels were normalized.