Assuntos
Osso e Ossos , Osteomalacia , Humanos , Osteomalacia/patologia , Osso e Ossos/patologia , BiópsiaRESUMO
Phosphaturic mesenchymal tumor (PMT) is a morphologically heterogeneous soft tissue and bone neoplasm, producing a paraneoplastic syndrome due to phosphate wasting. These tumors produce fibroblast growth factor 23, which is implicated in renal tubule phosphate loss. Medical records of patients seen from 1999 to 2013 with osteomalacia associated or not with a tumor were reviewed. Clinical and laboratory data, radiographic studies, and follow-up of 8 patients were tabulated. Histologic features and the immunoprofile of the tumors were analyzed. There were 208 patients with osteomalacia, but only 8 (3.84%) had osteomalacia associated with a tumor. The median age of the patients was 40 years. The tumor size ranged from 1.5 to 4 cm. Five were located in soft tissues and skin; and 3, in bones. Osteomalacia symptoms lasted from 2 to 14 years with a median of 6 years. Laboratory data showed hypophosphatemia and phosphaturia in all patients. All tumors were histologically benign. Histologically, the salient features were a hemangiopericytoid pattern, chronic hemorrhage, and microcystic areas. All neoplasms were diffusely positive for vimentin and focally positive for epithelial membrane antigen, CD34, and S-100 protein. Ki-67 was positive in approximately 10% of neoplastic cells in 2 cases and less than 1% in the remainder. We report 8 cases of PMTs producing osteomalacia, from a single third-level Mexican medical institution. These tumors occurred in soft tissues, skin, and bones. All tumors were benign, small, not easily detected by physical examination and diagnosed due to the metabolic abnormalities.
Assuntos
Hipofosfatemia/patologia , Mesenquimoma/patologia , Neoplasias de Tecido Conjuntivo e de Tecidos Moles/patologia , Osteomalacia/patologia , Adulto , Feminino , Humanos , Hipofosfatemia/etiologia , Masculino , Mesenquimoma/complicações , México , Pessoa de Meia-Idade , Neoplasias de Tecido Conjuntivo e de Tecidos Moles/complicações , Osteomalacia/etiologia , Estudos RetrospectivosRESUMO
Bone mineral density (BMD) at the lumbar vertebrae (L(1)-L(4)) was assessed by dual-energy X-ray absorptiometry (DXA) in 20 children with chronic kidney disease (CKD) on dialysis, and its results were compared with bone biopsy and biochemical parameters. Biopsy specimens provided evidence of hyperparathyroid bone disease in eight cases (40%), and low bone turnover in 12 (60%). For BMD, expressed as Z-scores relative to normal, median Z-scores were -1.05 (range -2.36 to 1.06) for hyperparathyroid patients and -1.05 (range -4.40 to -0.03) for low bone turnover patients, with no statistical differences between groups (P = 0.512). In relation to BMD, of the whole sample, five (25%) had a Z-score under -2.0. When it was corrected for height, BMD was in the normal range. Additionally, there were no significant differences in single samples of serum calcium, alkaline phosphatase, phosphorus and intact parathyroid hormone (PTH) between groups with high or low bone turnover. Assessment of nutritional status, through height/age, showed that ten patients had Z-scores below -2.0 (median -2.12, range -7.13 to 0.73). In conclusion, renal osteodystrophy (ROD) seems to have a high prevalence among CKD pediatric patients, although only approximately a quarter of them developed changes in BMD. In children with CKD, measurements of bone mineral density may not be used for classification of various forms of ROD.
Assuntos
Densidade Óssea , Doenças Ósseas/patologia , Osso e Ossos/patologia , Falência Renal Crônica/fisiopatologia , Falência Renal Crônica/terapia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Falência Renal Crônica/patologia , Masculino , Osteíte/patologia , Osteomalacia/patologiaRESUMO
UNLABELLED: Chronic kidney disease (CKD) leads to reduced bone mineral density (BMD) in pre-dialysis and dialysis patients. A few studies have used dual-energy x-ray absorptiometry (DEXA) to assess BMD in pre-dialysis CKD patients and have shown low BMD to be highly prevalent. Until now there have been no studies reporting the histological features of low BMD in pre-dialysis CKD patients. AIM: To determine the prevalence and histological features of low BMD in pre-dialysis CKD patients. METHOD: Pre-dialysis CKD patients (n = 103, 46 females/57 males), median creatinine clearance of 29 (10 - 78) ml/min/ 1.73 m2, were evaluated using biochemical analysis and DEXA. Bone biopsies were obtained from those with low BMD. RESULTS: Fifty (48.5%) out of the 103 patients had low BMD (LBD group) and 53 (51.5%) had normal BMD (NBD group). The risk for low BMD was increased in those patients with alkaline phosphatase levels above 190 U/l and intact-PTH (iPTH) below 70 pg/ml (p < 0.05). Demographic and biochemical parameters from both groups were comparable, except for lower body mass index (BMI) in LBD subjects (p = 0.04). Women who had been post-menopausal for at least 1 year comprised 65% (13/20) and 50% (13/26) of the LBD and NBD groups, respectively (p = NS). In 40 LBD patients, bone histomorphometry revealed adynamic bone disease (ABD, 52.5%), osteomalacia (OM, 42.5%) and mixed bone disease (MBD, 5%). Trabecular bone volume (BV/TV) was lower in ABD and OM patients. A nearly significant association was found between ABD and iPTH < or = 150 pg/ml (p = 0.056), whereas higher values of iPTH were associated with OM. Total alkaline phosphatase < or = 190 U/l was significantly associated with ABD, whereas higher values were associated with OM. No correlation was observed between BV/TV and BMD. CONCLUSION: Low BMD is frequent in pre-dialysis CKD patients, and low turnover bone disease, manifesting as ABD and OM, was the hallmark of this bone loss.
Assuntos
Densidade Óssea , Falência Renal Crônica/patologia , Rim/patologia , Absorciometria de Fóton , Adulto , Idoso , Fosfatase Alcalina/sangue , Índice de Massa Corporal , Doenças Ósseas/metabolismo , Doenças Ósseas/patologia , Creatinina/metabolismo , Feminino , Humanos , Falência Renal Crônica/metabolismo , Masculino , Pessoa de Meia-Idade , Osteomalacia/metabolismo , Osteomalacia/patologia , PrevalênciaRESUMO
The various forms of renal osteodystrophy are predominant hyperparathyroid bone disease, mixed uremic osteodystrophy, low turnover osteomalacia, and adynamic bone disease. The present study analyses a total number of 1,209 bone biopsies from 5 different countries (Brazil, Uruguay, Argentina, Portugal, and Spain). Low turnover osteomalacia and mixed uremic osteodystrophy were more common in Brazil, Uruguay, and Argentina than in Portugal and Spain whereas predominant hyperparathyroid bone disease was seen more often in Portugal and Spain. In all centers, independent of the aluminum staining technique used, the extent of aluminum deposited in bone was greater in patients presenting with low bone turnover, whether from low turnover osteomalacia or adynamic bone disease, than in the predominant hyperparathyroid bone disease. In summary, even though recent reports have indicated that, over the last decade, the incidence of aluminum-induced toxicity was reduced, aluminum still seems to be implicated in a great percentage of symptomatic low bone remodelling lesions in Iberoamerica.
Assuntos
Alumínio/análise , Osso e Ossos/química , Distúrbio Mineral e Ósseo na Doença Renal Crônica/epidemiologia , Distúrbio Mineral e Ósseo na Doença Renal Crônica/patologia , Hormônio Paratireóideo/sangue , Argentina/epidemiologia , Biópsia/estatística & dados numéricos , Doenças Ósseas/sangue , Doenças Ósseas/epidemiologia , Doenças Ósseas/patologia , Osso e Ossos/metabolismo , Osso e Ossos/patologia , Brasil/epidemiologia , Distúrbio Mineral e Ósseo na Doença Renal Crônica/sangue , Distúrbio Mineral e Ósseo na Doença Renal Crônica/classificação , Comorbidade , Humanos , Hiperparatireoidismo/sangue , Hiperparatireoidismo/epidemiologia , Hiperparatireoidismo/patologia , Falência Renal Crônica/sangue , Falência Renal Crônica/patologia , Osteomalacia/sangue , Osteomalacia/epidemiologia , Osteomalacia/patologia , Portugal/epidemiologia , Prevalência , Espanha/epidemiologia , Uruguai/epidemiologiaRESUMO
BACKGROUND: A positive correlation between successful kidney transplantation, few rejection episodes, greater susceptibility to infection and morbidity in patients with high tissue levels of aluminium (Al) indicate that the metal may play a role in the immune response. The aim of this study was to determine if experimental aluminium intoxication could result in significant changes in lymphocyte activity in uraemic and nonuraemic rats. METHODS: Lewis rats were divided into four groups: normals (N), nephrectomized control (U), and Al-treated (N + Al) and nephrectomized Al-treated (U + Al), which received a cumulative dose of 30 mg Al over a 4-week period. Al quantification, histology, histochemical analysis and immunological assays were performed after Al intoxication. RESULTS: High tissue levels of Al and positive histochemical staining in bones were seen in Al-treated rats. Bone histology revealed osteomalacia in U + Al rats. No statistical differences were observed in mixed lymphocyte cultures from controls and Al-treated rats, whereas U and Al-treated rats showed a decrease in lymphoproliferative response to mitogen and natural killer cell cytotoxic activity. A decreased helper T lymphocyte: cytotoxic T lymphocyte cell ratio and a reduction in interleukin-2 production were observed only in the U + Al group. A reduced number of total T lymphocytes was detected in the spleens of all Al-treated rats. CONCLUSIONS: These findings suggest that aluminium toxicity may contribute to immunological impairment in chronic renal failure.
Assuntos
Alumínio/toxicidade , Falência Renal Crônica/imunologia , Linfócitos T/imunologia , Animais , Osso e Ossos/patologia , Divisão Celular , Células Cultivadas , Concanavalina A/farmacologia , Citotoxicidade Imunológica/imunologia , Imunidade Celular , Interleucina-2/biossíntese , Falência Renal Crônica/induzido quimicamente , Ativação Linfocitária/efeitos dos fármacos , Ativação Linfocitária/imunologia , Contagem de Linfócitos , Masculino , Nefrectomia , Osteomalacia/induzido quimicamente , Osteomalacia/patologia , Ratos , Ratos Endogâmicos Lew , Linfócitos T/efeitos dos fármacos , Linfócitos T Citotóxicos/efeitos dos fármacos , Linfócitos T Citotóxicos/imunologia , Linfócitos T Auxiliares-Indutores/efeitos dos fármacos , Linfócitos T Auxiliares-Indutores/imunologia , Uremia/induzido quimicamente , Uremia/imunologiaRESUMO
We evaluated the course of severe aluminum-related bone disease (ARBD) after the first year of a successful renal transplantation (RTx) in 11 adult patients. Bone pain and muscle weakness, presented in all patients previously to RTx, subsided, and all were able to walk, even the ones who were confined to wheelchairs. Bone necrosis developed in 6 patients, but none required surgical repair. Serum alkaline phosphatase activity increased 2.5 times the upper normal level, up to the 5th month and then declined to normal levels up the 12th month (p < 0.05). The inverse profile was observed in both serum calcium and phosphorus levels. In bone biopsies, there was a significant decrease in all of the following histomorphometric static parameters: osteoid volume, thickness and surface and also in aluminum surface. Also, there was a significant increase in all the dynamic parameters of mineralization: mineral apposition rate, mineralization surface, bone formation rate and adjusted apposition rate. In conclusion, ARBD remarkably improves after 1 year of successful RTx.