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[Calcimimetics].

Current therapy for secondary hyperparathyroidism in uremia has relatively poor success in achieving the target levels of parathyroid hormone (PTH), calcium and phosphate established by the NKF-K/DOQI guidelines. The discovery and characterization of a new membrane receptor able to sense minimal Ca changes (CaSR) started intensive research in the attempt to characterize better its functions and its finding compounds, which could modulate its activity. CaSR is expressed not only in the cells that secrete calcium-regulating hormones (parathyroid cells and thyroid C-cells) and in cells involved in calcium transport mechanisms (ie intestinal cells, bone-forming osteoblasts, and cells of different nephron segments), but also in other tissues with, as yet, a not completely defined role. CaSR stimulation by the agonists is followed by the activation of a great number of G-proteins mediated intracellular signalling pathways (PLC, PLA, PLD, PKC, PKA, etc). At the level of parathyroid cells, the main effect is the increase in IP3, followed by a mobilization of intracellular Ca stores, which inhibit PTH secretion in a few seconds or minutes. Long-term CaSR stimulation is also able to induce a reduction in both PTH synthesis and parathyroid cell proliferation. More than 100 mutations of the gene coding for CaSR have been described. Some of these mutations are matched by a gain or reduction/loss of function. Notwithstanding, CaSR is widely represented on different tissue cells, the main clinical manifestations of the above genetic changes mainly involve PTH and calcium metabolism. A great number of inorganic and organic cations can interact with the Ca-sensitive N-terminus domain of CaSR, mimicking Ca effects (type I calcimimetics), but these substances have substantial limitations for use in clinical practice. A second class of compounds was produced (NPS R-467, S-467, R-568, S-568, AMG 073), for use in the clinical setting, type II calcimimetics. These compounds, after having interacted with the membrane-spanning domains of the CaSR, induce conformational changes in the N-terminus domain, increasing its affinity for Ca. The preclinical experiences with calcimimetics demonstrated that they were effective in reducing circulating PTH, preventing the progression of secondary hyperparathyroidism, suppressing parathyroid cell proliferation, and reversing osteitis fibrosa at least in animal models. Clinical studies were performed mainly using AMG 073, due to its greater bioavailability and more consistent pharmacokinetic profile. Clinical studies performed in primary hyperparathyroidism proved AMG 073 to be effective in reducing both PTH and Ca serum levels, with a good safety profile. Further studies, mainly focused on the efficacy of AMG 073 in the control of secondary hyperparathyroidism in uremia, confirmed the efficacy of this compound in reducing PTH levels >30% in about 50% of patients. Furthermore, the fall in PTH was matched by a reduction in both calcium and phosphate serum levels of about 5-7%, with a significant reduction in calcium x phosphate product (about 15%). The latter aspect represents a unique pharmacological profile, as compared to all the other available therapeutic means to control secondary hyperparathyroidism in uremia. In addition to their effectiveness, calcimimetics present a relatively safe profile, the only adverse events referred to consist of transient and easily remediable hypocalcemic episodes and some gastrointestinal discomfort symptoms. However, although calcimimetics represent a real advancement in the field of treating secondary hyperparathyroidism in uremic patients, their use should be matched by the awareness that previously the success of a high number of new drugs proposed have been flawed by negative consequences in the long term. Therefore, strict clinical control is necessary in the next few years when the use of these new compounds will widen.

Animals↗

PubMed1991

Renal bone disease in pediatric patients receiving treatment with maintenance peritoneal dialysis.

The present review summarizes the characteristics of renal bone disease in pediatric patients treated with maintenance peritoneal dialysis. Fifty-eight patients underwent iliac crest bone biopsy after double tetracycline labeling, measurements of aluminum in bone and various serum biochemical determinations including serum PTH, alkaline phosphatase, calcium, phosphorus and aluminum. Evidence of osteitis fibrosa was present in 45% of patients and mild lesions of secondary hyperparathyroidism were found in an additional 25%. Thus, secondary hyperparathyroidism remains the predominant bone lesion despite the use of oral calcitriol. Evidence of aluminum accumulation was substantially less prevalent, findings not surprising due to the widespread use of calcium carbonate as the main phosphate binder agent. However, aplastic bone lesion without aluminum staining was present in the majority of patients with low-turnover lesions of the bone without osteomalacic findings. The long-term evolution of such lesions remains to be evaluated. The potential value of alternative modes of calcitriol administration for the control of secondary hyperparathyroidism is discussed as well as the differences in the bioavailability of sterol according to the different routes for calcitriol administration.

Aluminum↗

PubMed1991

[Pathological analysis of renal osteodystrophy in 194 cases].

Renal osteodystrophy is a metabolic bone disease resulted from chronic renal failure. The long-standing alterations in a mineral metabolism generated by renal failure have a profound effect on the skeleton and induce severe systemic metabolic bone disease. Iliac crest biopsies of 194 patients of chronic renal failure were taken and among them 10 cases were examined for Calcium(Ca), phosphorus (p), parathyroid hormone (PTH), 1,25(OH)2D3 and aluminium (Al). The histological bone changes are characterized by development of osteitis fibrosa, increase of bone resorption and the number of osteoclast, increase of osteoid volume (osteoblastic osteoid and acellular osteoid), active remodelling of bone and aluminum deposition in the bone. According to histological appearance, advanced renal bone disease could be subdivided into three groups namely: Secondary hyperparathyroid bone disease (high turnover uremic osteodystrophy), osteomalacia (low turnover uremic osteodystrophy) and mixed uremic osteodystrophy consisting of mild to moderate hyperparathyroid bone disease and defective mineralization. Aluminum-related bone changes might be obtained in various extent in all these groups. Although this classification does not fully represent all the separated entities, and there is also transformation from one form to another, it seems no less significant as a reference for clinical considerations.

Adolescent↗

PubMed1991

The radiographic spectrum of renal osteodystrophy.

Chronic renal failure often results in bone changes, collectively known as renal osteodystrophy. These changes include osteitis fibrosa, osteosclerosis, soft tissue calcifications, osteomalacia (in adults) and rickets (in children). Early recognition of renal osteodystrophy allows more aggressive clinical control of serum calcium and phosphate levels. Intervention may spare the patient late complications such as fractures, pain and loss of skeletal function.

Aluminum Hydroxide↗

PubMed1990

Bone disease in primary hyperparathyroidism.

The classic bone disease of primary hyperparathyroidism, osteitis fibrosa cystica, was characterized by subperiosteal bone resorption, osteopenia, and "brown tumors." Since the description of this skeletal disorder, the clinical profile of primary hyperparathyroidism has evolved markedly. The disease today is often characterized by no more than asymptomatic hypercalcemia, and severe bone disease is a distinct rarity. However, as we have endeavored to show in this article, newer and more sensitive techniques show significant evidence of the effect of excess parathyroid hormone on the skeleton. Bone density as measured by photon absorptiometry and bone histomorphometry show a deficit of cortical bone and a preservation or increase in cancellous bone elements in mild primary hyperparathyroidism with no clinical evidence of skeletal disease. Important questions exist as to the therapeutic implications of these data. Does the presence of parathyroid hormone effect on sophisticated testing portend the development of clinical bone disease? Should these data be used as a rationale for surgical intervention in patients who might otherwise be followed conservatively with mild primary hyperparathyroidism? The answers to these questions must await further data collection and study.

Bone Diseases↗

PubMed1979

Renal osteopenia - an assessment of long-term therapy with vitamin D analogues.

Renal bone disease was assessed for an average of 5.5 years in 9 patients on maintenance haemodialysis. The investigative methods included serial biochemical estimations, radiographic skeletal surveys and quantitative bone histology. Repeated bone mineral analyses and neutron activation analyses of a hand were also performed in order to monitor changes in skeletal calcium content. Before treatment, progressive osteodystrophy was demonstrated by all techniques. Following therapy with the vitamin D analogues, all patients noted symptomatic improvement; serum alkaline phosphatase reverted to normal and serum parathyroid hormone concentrations decreased. Radiographically, subperiosteal erosions healed while the histological features of osteomalacia and osteitis fibrosa were abolished. Both bone mineral and neutron activation analyses indicated that progressive skeletal demineralisation had been halted. However, a sustained increase in the overall mineral content of bone was not demonstrated. Thus, vitamin D therapy although improving the biochemical, radiological, and histological features of renal osteodystrophy may not restore bone mass to osteopenic bone.

Adult↗

PubMed1990

Comparison of different parathyroid hormone radioimmunoassays in uremic patients with secondary hyperparathyroidism.

Parathyroid hormone (PTH) levels of patients with chronic renal failure (CRF) were measured by three different radioimmunoassays (RIA); RIA for mid-region PTH by antibody CH9 (i-PTH) (1), RIA for intact fragments of PTH (intact PTH) and RIA for C-terminal fragments of PTH (PTH-C). PTH levels were higher in CRF patients undergoing hemodialysis therapy (hemodialysis patients) by all three methods. However, PTH levels measured only by i-PTH assay and intact PTH assay were significantly higher in patients with CRF who were not on dialysis (non-dialyzed CRF patients). PTH levels were above normal when creatinine clearance was below 45 ml/min in the intact PTH assay and 66 ml/min in the i-PTH assay. I-PTH levels were well correlated with the severity of osteitis fibrosa evaluated by the degree of periosteal resorption in the digits of hemodialysis patients. Since special handling of the sample is required for the intact PTH assay, i-PTH assay is the most suitable method for diagnosing secondary hyperparathyroidism in patients with CRF.

Bone Diseases, Metabolic↗

PubMed1990

[Prevalence of histological types of bone disease in a hemodialysis center limiting the oral intake of aluminum hydroxide].

UNLABELLED: To assess the prevalence of histologic bone disease in our center where Al(OH)3 intake is restricted, we reviewed 42 bone biopsies performed between 1975 and 1985 in patients dialyzed more than 29 months. Bone biopsies were performed systematically (2/3 of the cases) or because of a mild hypercalcemia (1/3 of the cases). Seventeen of these patients had been dialyzed before 1978 with softened water moderately contaminated by aluminum. Fifteen had always been dialyzed with reverse osmosis treated water and 10 had been exclusively treated by hemofiltration. The prevalence of osteitis fibrosa was 76%, that of osteomalacia null and that of adynamic bone disease 24% (but only 9.5% with positive Aluminon staining). When the 17 patients dialyzed with aluminum contaminated water before 1978 were excluded, only one patient among 25 had an aluminum adynamic bone disease (4%). This low prevalence can probably be explained by the restricted intake of Al(OH)3 thanks to the systematic administration of Ca CO3 and in a few cases of Mg (OH). The adynamic bone disease group has lower serum concentration of PTH and shorter duration on dialysis whereas the serum levels of calcium, phosphorus, magnesium and aluminum and daily dose of Ca CO3, Mg (OH)2 and Al(OH)3 do not differ. The frequency of the positivity of aluminum staining is not statistically different in the 2 groups. In 4 cases, adynamic bone disease without aluminum or iron intoxications is found, associated with a relative hypoparathyroidism. It is not explained by previous parathyroidectomy, diabetes or steroid therapy. CONCLUSIONS: 1) Restriction of aluminum intake and dialysis with reverse osmosis treated water lead to a low prevalence of aluminum bone disease. 2) A new bone disease in uremia is described: the idiopathic adynamic bone disease associated with a relative hypoparathyroidism.

Adult↗

PubMed1985

Problems of differential diagnosis in a case of bone lesions caused by myelosclerosis associated with hyperparathyroidism.

The authors present a report on the case of a female patient with myelofibrosis where X-ray examinations of the bone skeleton predominantly showed such osteolytic changes which are no typical of myelosclerosis. Morphological criteria rather would have corresponded to osteitis fibrosa cystica generalisata. Laboratory examinations partially indicated primary hyperparathyroidism, this assumption could not be confirmed by other examinations. Also histological findings of repeated bone biopsies did not lead to a uniform diagnosis. Autopsy confirmed the presence of both diseases. The authors consider the synergistic effect of both diseases to be the cause for the development of those bone changes mentioned above.

Bone Resorption↗

PubMed1989

Asymptomatic intrathoracic extramedullary hematopoiesis: a report of three cases.

Extramedullary hematopoiesis (EMH) is observed in people suffering from severe anemia of prolonged duration and appears to be a compensatory mechanism for disturbed medullary hematopoiesis. The hemoglobinopathies (such as thalassemia, spherocytosis, and sickle cell disease), neoplastic diseases such as leukemia and lymphoma, and others, including myelofibrosis and osteitis fibrosa cystica, are associated with EMH. These diseases and their resultant anemia have in common the ability to stimulate erythropoietin production, which in turn may stimulate hematopoiesis in organs of mesenchymal origin. The liver and spleen are the most common sites of EMH; however, other sites, including the falx cerebri, thoracic cavity, retroperitoneal area and pelvis have been reported. When present, intrathoracic EMH is most frequently associated with thalassemia. Spinal cord compression and hemothorax have also been reported as complications of intrathoracic EMH.

Adult↗

PubMed1989

[Type I pseudohypoparathyroidism. Study and prolonged follow-up of a case].

The prolonged evolution of a case of pseudohypoparathyroidism with hereditary osteodystrophy (AHO) and osteitis fibrosa is presented. The diagnosis was confirmed by the existence of a peculiar phenotype, hypocalcaemia, hyperphosphatemia, increased PTH values, and a lack of tubular response after PTH and radiological signs of hyperparathyroidism. The clinical and biochemical evolution, under 1.25 (OH)2D3 therapy with special emphasis on the growth and development were shown. The bibliography was also reviewed.

Child↗

PubMed1989

Guidelines for recombinant human erythropoietin therapy.

Extensive testing has proven that recombinant human erythropoietin (r-HuEPO; EPOGEN [epoetin alfa], AMGEN Inc, Thousand Oaks, CA) corrects the anemia of end-stage renal disease and eliminates the need for transfusions in virtually all patients. Patients whose hematocrit levels are less than 0.30 or who are transfusion dependent are candidates for therapy. A dosage of 50 to 150 U/kg body weight intravenously three times a week produces an increase in hematocrit by approximately 0.01 to 0.02 per week. Once the hematocrit reaches 0.30 the dose is adjusted so that a target hematocrit of 0.32 to 0.38 is maintained. Eighty percent of patients need maintenance doses of r-HuEPO of less than or equal to 150 U/kg; the other 20% of patients require larger doses. Reasons for poor responses include iron deficiency, inflammation due to surgery or infection, and osteitis fibrosa. Most patients require iron supplementation to prevent functional iron deficiency. BP increased in one third of patients, and in 3% seizures occurred during the initial phase of therapy, often associated with a sudden increase in BP. This hypertension can be controlled with medication. Increased dialyzer clotting may occur, which is prevented when heparin doses are adjusted, and dialyzer solute clearances may decrease slightly. Treatment with r-HuEPO does not elicit an antibody response. The mechanism of action of r-HuEPO is identical to that of natural erythropoietin, and therefore is an appropriate therapy for the long-term management of anemia in chronic renal failure.

Anemia↗

PubMed1977

Calcium and phosphorus metabolism in nephrotic syndrome.

Calcium and phosphorus balance studies were performed on 13 nephrotic patients and eight patients during clinical remission of the nephrotic syndrome. Marked impairment of intestinal absorption of calcium was found among nephrotic patients, in eight of whom faecal calcium equalled or exceeded dietary calcium. The mean faecal:dietary calcium ratio of nephrotic patients, 1-06 +/- 0-23 (SD), was significantly higher (p less than 0-005) than that of patients in remission, 0-58 +/- 0-21 (SD). The mean 24-hour urinary excretion of calcium of nephrotic patients, 0-68 +/- 0-68 (SD) mmol, was significantly lower (p less than 0-005) than that of patients in remission, 3-02 +/- 1-91 (SD) mmol. Calciferol administered to three nephrotic patients in the dosage of 1.25 mg per day did not significantly influence intestinal absorption or renal excretion of calcium. There was no difference between the two groups of patients in intestinal absorption or renal excretion of phosphorus; there was net intestinal absorption in all subjects. Quantitative bone histology was studied in seven of the nephrotic patients. None had osteomalacia or osteitis fibrosa, while only one had evidence of mild osteoporosis.

Adolescent↗

PubMed1988

Renal osteodystrophy and aluminum bone disease in CAPD patients.

Renal failure is frequently associated with osteodystrophia due to secondary hyperparathyreoidism and/or increased aluminum intake. The problem of hypercalcemia and hyperphosphatemia can more easily controlled by CAPD than by hemodialysis. Total serum and ionized calcium levels are rapidly normalized by a CAPD regime of four 2-1 exchanges with 1.75 mmol/l Ca. Under the same CAPD regime 250-300 mg phosphate are removed per day. Depending on the ingestion of phosphate, 100-200 mg phosphate per day remain to be removed by phosphate binding agents. Since the main source of aluminum in CAPD patients is oral ingestion of aluminum-containing phosphate binders, serum levels should be regulated by diet and calcium carbonate. To suppress PTH secretion serum ionized calcium levels need to be maintained at the upper limit of normal. This can also be achieved by the use of oral calcium carbonate. Vitamin D or analogs should be prescribed only when clinically indicated by persistent hypocalcemia, osteitis fibrosa or non-aluminum related osteomalacia.

Aluminum↗

PubMed1988

Symmetric gangrene of the extremities in late renal failure: a case report and review of the literature.

Medical calcification of arteries is common in chronic renal failure. We report on a patient with extensive calcification of the arterial media who developed symmetrical acral gangrene and severe cardiac failure shortly after cadaveric renal transplantation. At necropsy, the medial calcification was found to be accompanied by extensive intimal proliferation and multiple antemortem fractures, some healing by callus formation. Such medial calcification, which is similar to Monckeberg's sclerosis, affected all systemic arteries except the aorta, pulmonary artery and transplanted renal artery. Six years before this terminal illness he had undergone total parathyroidectomy for osteitis fibrosa associated with ruptured tendons. We review previous reports of patients with the syndrome of acral gangrene in azotaemic renal failure and discuss the histopathological features and pathogenesis in relation to the unusual features of our patient.

Adult↗

PubMed1988

Osteomalacia as a very late manifestation of primary hyperparathyroidism.

An 86-year-old woman with a history of treated hyperthyroidism and a 20-year history of untreated primary hyperparathyroidism developed generalized bone pain and a pseudofracture of the midshaft of the left femur. Laboratory examinations revealed elevated serum calcium, alkaline phosphatase, and C-terminal parathyroid hormone levels. Serum inorganic phosphate was below normal and 25-hydroxyvitamin D levels were low-normal. An undecalcified transiliac bone biopsy specimen following tetracycline double labeling revealed osteomalacia and osteitis fibrosa. Following treatment with vitamin D and phosphate, the serum inorganic phosphate level rose to normal. There was a decrease in bone pain, and the pseudofracture healed. However, the serum calcium, alkaline phosphatase, and C-terminal parathyroid hormone levels remained elevated. Longstanding primary hyperparathyroidism causes chronic hypophosphatemia and may lead to osteomalacia. Osteomalacia and its consequences may be part of the spectrum of bone disease seen in patients with longstanding primary hyperparathyroidism.

Aged↗

PubMed1987

Parathyroid carcinoma versus parathyroid adenoma in patients with profound hypercalcemia.

The metabolic manifestations and operative findings in 10 patients with a diagnosis of parathyroid carcinoma were analyzed to determine whether they differ from those in patients with parathyroid adenomas and similar degrees of hypercalcemia. Two groups of patients with parathyroid adenomas were used for comparison. Group A consisted of eight patients with "atypical" benign adenomas (mean preoperative level of serum calcium: 13.4 mg/dl); group B consisted of 13 patients with benign typical adenomas--all with preoperative serum calcium levels greater than or equal to 13.0 mg/dl (mean: 14.2 mg/dl). The patients with carcinoma (mean preoperative level of serum calcium: 15.3 mg/dl) had a frequency of osteoporosis and osteitis fibrosa cystica (50%) comparable with that of group A (33%) and group B (62%). Seventy percent of the patients with carcinoma had renal disease (nephrolithiasis, nephrocalcinosis, or impaired renal function), whereas only 38% of group A and 15% of group B had similar disorders. The patients with carcinomas had the highest frequency of combined bone and renal disease (50% versus 14% in group A and 15% in group B). Anemia, peptic ulcer disease, and hypertension occurred with similar frequencies in the three groups. Three patients with recurrent parathyroid carcinoma died of profound hypercalcemia, renal failure, or cardiac arrhythmia. In general, although patients with parathyroid carcinomas have more profound metabolic abnormalities than do patients with primary hyperparathyroidism, the metabolic manifestations in patients with parathyroid carcinoma are comparable with those in patients with parathyroid adenomas and profound hypercalcemia. Furthermore atypical adenomas share many anatomic and histopathologic features with parathyroid carcinomas, and distinguishing between the two is sometimes possible only in cases of tumor recurrence.

Adenoma↗

PubMed1987

[Parathyroid carcinoma: report of a case].

A case of parathyroid carcinoma is presented. A 46-year-old female was admitted to our hospital for fractures of both femurs on July 29, 1983. Laboratory data revealed a serum calcium level of 15.2 mg/dl, serum phosphate level of 1.2 mg/dl, serum immunoreactive parathyroid hormone 9.35 ng/ml (less than 0.5), and % tubular reabsorption of phosphate of 57%. X-ray examination showed marked osteitis fibrosa cystica. The diagnosis of primary hyperparathyroidism was made. A hard tumor was palpable on the left anterior side of her neck. Neck exploration was carried out on August 10. The tumor was found to be localized in contact with the left lower lobe of the thyroid gland. Parathyroid carcinoma was strongly suspected, because the tumor severely adhered to surrounding tissues, thus the tumor was resected en bloc. The histopathological diagnosis was typical parathyroid carcinoma. Postoperative course and the treatment of the fractures were uneventful, and she was discharged able to walk five months after the operation. No evidence of recurrence or metastasis has been seen during the eighteen months since the operation. This is the 80th case in the Japanese literature to our knowledge and the clinical features of these 80 cases revealed an average age of 41.5 years old; male/female ratio of 32/48; average weight of tumor of 8.65 g, palpable neck mass in 72%, bone disease in 64%, and renal disease in 34%.

Carcinoma↗