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PubMed1977

Radiological/pathological correlations in uremic bone disease.

Skeletal radiographs and non-decalcified bone specimens from 17 chronically uremic patients with radiographic evidence of bone disease were studied quantitatively. The results of each morphological technique were compared in an attempt to define the roentgenographic manifestations of renal osteodystrophy histologically. The radiographs correlated best with the trabecular bone manifestations of osteitis fibrosa but showed poor correlation with histological evidence of osteomalacia. Radiographic signs of osteosclerosis could not be correlated with any radiographic or histiolgical feature of bone resorption.

Adult↗

PubMed1996

Uremic leontiasis ossea: "bighead" disease in humans? Radiologic, clinical, and pathologic features.

PURPOSE: To describe the clinical, radiologic, and pathologic findings in patients with uremic leontiasis ossea (ULO). MATERIALS AND METHODS: Five patients with renal osteodystrophy developed marked hyperostosis of the facial and cranial bones. Radiologic studies included plain radiography of the skull (n = 5), computed tomography with three-dimensional reconstruction (n = 4), magnetic resonance imaging (n = 3), and fluorine-18 sodium fluoride positron emission tomography (PET) (n = 1). Specimens from bone biopsies (three patients) were examined. RESULTS: Skull and facial alterations were remarkably similar. Numerous nodules of varying attenuation and signal intensity in the widened diploic space suggested brown tumors in different stages of evolution. Biochemical data and PET findings enabled confirmation of markedly increased bone turnover. Bone specimens demonstrated severe osteitis fibrosa. After parathyroidectomy, facial changes in all patients stabilized or improved mildly. CONCLUSION: A similar entity in animals, "bighead" disease, which results from nutritional and uremic secondary hyperparathyroidism, may provide a useful animal model for ULO in humans. Mild forms of this entity may be more common than the scarcity of previous reports suggests.

Adult↗

PubMed1980

Parathyroid hormone metabolism and its potential as a uremic toxin.

Secondary hyperparathyroidism is a universal complication of chronic renal failure. It has been proposed that the markedly elevated levels of immunoreactive parathyroid hormone (i-PTH) in uremia may represent a "uremic toxin" responsible for many of the abnormalities of the uremic state. Plasma i-PTH consists of a mixture of intact hormone, a single-chain polypeptide of 84 amino acids, and smaller molecular weight hormonal fragments from both the carboxy- and amino-terminal portion of the PTH molecule. The hormonal fragments arise from metabolism of intact PTH by peripheral organs as well as from secretion of fragments from the parathyroid glands. The structural requirements for the known biological actions of PTH reside in the amino-terminal portion of the PTH molecule. Carboxy-terminal fragments, biologically inactive at least in terms of adenylate cyclase activation, hypercalcemia, or phosphaturia, depend on the kidney for their removal from plasma, and thus accumulate in the circulation in chronic renal failure. It is unknown at the present time if other biological effects of these carboxy-terminal fragments may contribute to some of the biochemical alterations observed in uremia. The most significant consequence of increased PTH levels in uremia is the development of bone disease characterized by osteitis fibrosa. In addition, it would appear that PTH plays an important role in some of the abnormal electroencephalographic patterns observed in uremia. This may be due to a potential role of PTH in increasing calcium content of brain. Parathyroid hormone also has been implicated as a pathogenetic factor in many other alterations present in uremia, i.e., peripheral neuropathy, carbohydrate intolerance, hyperlipidemia, and other alterations. Unfortunately, outstanding clinical research is lacking in this field and conclusive experimental data are practically nonexistent. Further studies are necessary if one is to accept the concept of PTH being a significant "uremic toxin."

Animals↗

PubMedDate not supplied

Hyperparathyroidism and anemia in renal failure.

Patients with severe secondary hyperparathyroidism, usually associated with osteitis fibrosa on bone histology, show considerable resistance to Epoetin, partly because of replacement of the cellular components of the bone marrow by fibrous tissue. In case of unexplained resistance to Epoetin, investigation of secondary hyperparathyroidism is strongly recommended, with measurement of serum parathyroid hormone, calcium, phosphate, and alkaline phosphatase levels and, where needed, skeletal radiology and bone biopsy. Treatment of severe secondary hyperparathyroidism consists of active vitamin D metabolites or parathyroidectomy, although the marrow fibrosis, if present, may be irreversible. The finding of a progressive inability of the bone marrow to respond to Epoetin treatment with higher levels of parathyroid hormone suggests the importance to prevent metabolic bone disease and in particular secondary hyperparathyroidism. Moreover, there are several reports of a beneficial action on hemoglobin levels of an effective treatment of hyperparathyroidism. Larger, controlled studies are necessary to confirm these preliminary and exciting findings, and to elucidate the mechanisms underlying the improvement in anemia after medical or surgical treatment of hyperparathyroidism.

Anemia↗

PubMed2001

Acute respiratory failure due to "pulmonary calciphylaxis" in a maintenance haemodialysis patient.

Calciphylaxis is a rapidly developing, fatal process of vascular calcium deposition with prominent cutaneous manifestation. We treated a long-term haemodialysis patient who developed an analogous disorder limited to the lungs. A 57-year-old man was admitted for initiation of peritoneal dialysis because limited cardiac reserve precluded further haemodialysis. He was treated successfully for pneumonia until hypoxia and progressive hypercalcaemia developed. (99m)Tc-methylene disphosphonate scintigraphy showed diffusely increased pulmonary uptake. Death supervened despite aggressive and successful treatment of hypercalcaemia. Autopsy studies included immunohistochemistry and morphometric studies of bone. Alveolar capillary walls showed diffuse calcium deposition. Both gross and microscopical findings differed from those of typical metastatic calcification in dialysis patients. Immunoreactivity for parathyroid hormone-related protein was present in the lesions. Bone histomorphometry indicated mild osteitis fibrosa. Pneumonia is believed to have caused local synthesis of parathyroid hormone-related protein that, along with high calcium x phosphorus product, contributed to calcium deposition. By analogy with the cutaneous process we termed the deposition "pulmonary calciphylaxis".

Acute Disease↗

PubMed2003

Pathogenesis and treatment of renal osteodystrophy.

Renal osteodystrophy is the term used to describe the many different patterns of the skeletal abnormalities that occur in patients with chronic kidney disease. The main two conditions are osteitis fibrosa, characterized by high bone turnover, increased osteoclastic and osteoblastic activity, and high levels of circulating parathyroid hormone (PTH) and adynamic bone disease characterized by low bone turnover and low levels of circulating PTH. Retention of phosphorus, decreased levels of calcitriol in blood, decreased levels of serum ionized calcium, reduced numbers of vitamin D receptors and calcium sensors in the parathyroid gland, and skeletal resistance to the calcemic action of PTH play a major role in the development of renal osteodystrophy. This review will describe the current approach for the treatment of renal osteodystrophy.

Calcitriol↗

PubMed2004

Calcimimetics versus vitamin D: what are their relative roles?

A strict control of secondary hyperparathyroidism in patients with chronic kidney disease (CKD) is indicated to avoid serious complications linked to osteitis fibrosa and other parathyroid-hormone (PTH)-related bodily disturbances. However, such a control is often achieved only at the price of unacceptably high plasma calcium and phosphorus levels and the risk of soft tissue calcification, even when using the novel, so-called 'non-hypercalcemic' vitamin D analogs. The advent of a new class of drugs, the calcimimetics, should allow a more adequate control of the disturbed calcium-phosphorus metabolism in CKD patients. In my opinion, the calcimimetics will not replace currently used medications but will be a valuable supplement to presently available treatment options for this major complication in patients with renal failure.

Calcitriol↗

PubMed2006

Measurement of PTH fragments for assessment of renal bone disease in hemodialysis patients.

BACKGROUND: Renal bone pathology involves a spectrum from 'high-turnover' to 'low-turnover bone disease' (adynamic bone disease, classical osteomalacia). The diagnosis of the latter usually requires bone biopsy. Inhibitory parathyroid hormone (PTH) fragments may be useful for its noninvasive diagnosis. METHODS: A cross-sectional study was performed in 54 patients on chronic hemodialysis which involved measurements of intact PTH (iPTH; Nichols assay), total PTH (tPTH; Scantibodies assay), and the cyclase-activating PTH fragment (CAP). The level of cyclase-inactive PTH fragment (CIP) was calculated. At the same time, serum calcium, phosphorus, and alkaline phosphatase levels as well as the current therapy for secondary hyperparathyroidism were recorded. In selected patients, bone radiographs were evaluated for osteitis fibrosa. RESULTS: A high correlation (r = 0.94) was found between iPTH and tPTH, with the tPTH levels being lower by 30-40%. A similar association was also found for CAP (r = 0.988) and for CIP (r = 0.93). 3 out of the 54 patients had a CAP/CIP ratio of < or =1 which has been associated with adynamic bone disease. A higher CIP ratio was significantly associated with the use of aluminum-hydroxide- and calcium-containing phosphate binders. CONCLUSIONS: iPTH and tPTH assays are highly correlated. In a general hemodialysis patient population, low-turnover bone disease appears to be rare, when the CAP/CIP ratio is used as a marker. A high CIP value was associated with therapy using aluminum hydroxide, a drug known to carry a risk of adynamic bone disease.

Aged↗

PubMed1989

Hypercalcemia in patients with advanced chronic renal failure not yet requiring dialysis.

Six patients with progressive chronic renal failure not yet requiring dialysis and not consuming supplemental calcium or vitamin D developed hypercalcemia. Three had proven and 1 suspected tertiary hyperparathyroidism, 1 parathyroid carcinoma and 1 aplastic bone. None of the 3 patients who underwent bone biopsy had heavy bone aluminum staining. The patients with proven parathyroid-mediated hypercalcemia had marked elevation of C-terminal parathyroid hormone and alkaline phosphatase values and, when performed, radiographs consistent with osteitis fibrosa. When these findings are absent or the diagnosis is otherwise uncertain, a bone biopsy may provide a definitive diagnosis and guide management.

Adult↗

PubMed1995

Modulation of renal osteodystrophy by extrarenal production of calcitriol.

We report a patient with severe chronic renal failure who developed spontaneous bone fractures. He was found to have hypercalcemia, normal calcitriol levels (probably due to extrarenal production by noncaseating granulomas), and functional hypoparathyroidism. The bone biopsy showed low bone turn-over and the presence of noncaseating granulomas. Treatment with corticosteroids decreased the calcium and calcitriol levels and the parathyroid hormone levels rose. No further fractures occurred. A repeat bone biopsy revealed the presence of osteitis fibrosa. Renal osteodystrophy may be modulated by extrarenal production of calcitriol. In this case, excessive suppression of parathyroid hormone by endogenous calcitriol presumably caused an adynamic bone lesion and spontaneous fractures.

Adult↗

PubMed1986

Comparative evaluation of bone aluminum content and bone histology in patients on chronic hemodialysis and hemofiltration.

In order to compare hemofiltration (HF) and hemodialysis (HD) in connection with the risk of aluminum overload and renal osteodystrophy, double bone biopsies after double tetracycline labeling and a desferrioxamine test were performed in 12 patients on HF and 15 patients on HD. The aluminum concentration was low (less than 0.6 mumol/l) both in the dialysate and the substitution fluid. The duration of treatment (about 2 years) and the cumulative doses of Al(OH)3 and CaCO3 were comparable in the two groups. None of the patients was taking 1 alpha-OH-D. The aluminum balance during an HF run ranged from -22 to +1.8 mumol/l, the balance being positive only when the plasma aluminum was less than 0.5 mumol/l. Basal plasma aluminum and its increase induced by desferrioxamine were comparable in the two groups. Bone aluminum content was also comparable, but was about 10 times higher than in 7 nonuremic controls. Bone aluminum content and plasma aluminum increase after desferrioxamine were correlated to the Al(OH)3 cumulative dose. None of the patients had florid osteomalacia with increased osteoid thickness, and only 1 in each group had traces of stainable aluminum. The mineralization front was decreased in 8 of 12 HF and in 9 of 14 HD patients, so that no difference was observed between the means of the two groups. The predominant histological bone picture of the patients was osteitis fibrosa which was present in 10 of 12 HF and in 13 of 15 HD patients. Mean osteoclast count and active resorption surface were comparable in the two groups, but was increased (5-10 times the mean of the controls).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1986

Comparison of histomorphometry and computerized tomography of the spine in quantitating trabecular bone in renal osteodystrophy.

Computerized tomography (CT) has the advantage of allowing the isolation of trabecular bone of the axial skeleton, which is the more sensitive to metabolic changes. By means of single-energy X-ray CT we have devised a method for assessing trabecular bone mass (TBM) at L-4 vertebral body, which has an acceptable reproducibility (CV = 2.76%). Normal values where obtained after studying 29 normal individuals from both sexes and different ages. In order to know if the method is accurate, TBM was assessed in 17 patients (6 with creatinine clearance less than 6 ml/min and 11 on chronic hemodialysis), and in all of them the results were compared with the histomorphometry of the iliac crest bone biopsy. TBM assessed by CT correlated with trabecular bone volume (TBV; mineralized bone + osteoid) (r = 0.82; p less than 0.001) and this correlation was not improved after adding the volume of marrow fibrosis to TBV. TBM assessed by CT also correlated with mineralized bone volume but at a lower level of significance (r = 0.72; p = 0.001), and no correlation was found with relative osteoid volume alone. These findings suggest that final CT value is an integral of mineralized bone and osteoid but with a higher influence of mineralized bone. Osteomalacia and osteitis fibrosa were seen in situations of normal, decreased, or increased TBM assessed by CT, although the 2 patients with severe osteosclerosis suffered osteomalacia. We conclude that the assessment of TBM by CT at the axial skeleton of uremic patients has an acceptable reproducibility and accuracy.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1992

Prevention of hyperparathyroidism in patients on maintenance dialysis by intravenous 1-alpha-hydroxyvitamin D3 in association with Mg(OH)2 as sole phosphate binder. A randomized comparative study with the association CaCO3 +/- Mg(OH)2.

The purpose of this study is to evaluate the place of intravenous 1 alpha-hydroxyvitamin D3 (1 alpha-OH-D3) in the prevention of radiologically obvious hyperparathyroidism (HPT) in patients on maintenance dialysis while excluding aluminium phosphate binder and using a dialysate calcium concentration of 1.62 mmol which keeps the intradialytic calcium balance neutral. Therefore, 47 patients without subperiosteal resorption and previously treated by oral CaCO3 and if necessary Mg(OH)2 as phosphate binder while their dialysate calcium had a Ca level of 1.62 and a Mg level of 0.2 mmol/l were randomized into a control group of 24 who were maintained on the same treatment and an experimental group of 23. This group discontinued CaCO3 and received intravenous 1 alpha-OH-D3 after each dialysis at increasing doses up to 4 micrograms and increased Mg(OH)2 as their sole phosphate binder. When plasma Ca increased above 2.7 mmol/l, the dose of 1 alpha-OH-D3 was decreased. When plasma PO4 increased above 2 mmol/l, the dose of Mg(OH)2 was increased to the highest dose not inducing diarrhea, hypermagnesemia (less than 2 mmol/l) or hyperkalemia (less than 6 mmol/l). In case of persistent hyperphosphatemia, the dose of 1 alpha-OH-D3 was decreased. Since mean plasma alkaline phosphatase was normal, HPT was monitored on the plasma concentration of 1-84 PTH for which a previous histological study showed that frank osteitis fibrosa was present only when they were above 70 pg/ml, i.e. (about twice the upper limit of the normal value). Before the study, plasma PTH was below this limit in 16 patients of the CaCO3 group and in 14 patients of the 1 alpha-OH-D3 group. After 6 months, they remained below this limit in all patients except 2 of each group. Plasma PTH was initially above 70 pg/ml in 8 of the CaCO3 and did not change significantly throughout the study, 2 patients having at 6 months a PTH level below 70 pg/ml. In contrast with intravenous 1 alpha-OH-D3, all the 9 patients with initial frank HPT decreased their PTH levels after 2 months, the levels being below 70 pg/ml in 6 cases. However, because of hypercalcemia and/or of hyperphosphatemia in spite of a highest tolerable dose of Mg(OH)2, 1 alpha-OH-D3 doses had to be decreased down to 0.4 microgram per dialysis at the 6th month so that at 6 months 6 of 9 patients had their PTH levels above 70 pg/ml, a number comparable to that of patients treated with CaCO3 (6 of 8).(ABSTRACT TRUNCATED AT 400 WORDS)

Administration, Oral↗

PubMed1992

Tumoral calcifications in hemodialysis patients: possible role of aluminum intoxication.

Uremic patients may develop extraskeletal calcifications. Among the latter, periarticular tumoral calcifications (TC) represent massive, multiloculated calcium-phosphate deposits. The aim of this report was to analyze a series of 10 cases of TC in hemodialysis patients who were admitted at the Necker Hospital between 1974 and 1988. They were all male. An increased plasma calcium x phosphorus product was observed in 8 of the 10 patients. Plasma calcium level was increased in only 2 patients. In contrast, hyperphosphatemia was a constant feature in all the patients, as was the absence of an increase in plasma alkaline phosphatase activity. Using the bone histomorphometry technique, osteitis fibrosa of mild degree was observed in 2 patients, of moderate degree in 2 and of severe degree in 2 others. Evidence of aluminum (Al) overload was found in the 8 patients in whom it was searched based on bone histomorphometry, bone histochemistry, bone Al content and increased serum Al levels either in the basal state or after a deferoxamine test. In addition, Al overload was strongly suspected in the 2 remaining patients because of prolonged exposure to Al-contaminated dialysate. Various treatment strategies, including parathyroidectomy (PTx), were undertaken that remained unsuccessful in modifying the course of TC to a significant extent. Remarkably, TC occurred for the first time after PTx in 1 patient and worsened after PTx in 2 others. In conclusion, overt secondary hyperparathyroidism appears not to be an essential prerequisite for TC development in hemodialysis patients, and PTx must not be performed in such patients on the sole basis of the presence of TC.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1994

Erythropoietin treatment in haemodialysis patients with iron overload.

Erythropoietic response to exogenously administered recombinant human erythropoietin (rHuEpo) was examined in 11 maintenance haemodialysis patients with iron overload (IO). All had required numerous blood transfusions earlier (> 12 units/year). Diagnosis of IO was established by high serum ferritin (SF) levels (> 1,100 micrograms/l), high hepatic CT density (> 70 Hounsfield units; HU) and excessive iron stores in bone marrow aspirate (grade 6). None of the patients had osteitis fibrosa cystica, aluminium intoxication, haemoglobinopathy or haemochromatosis alleles (HLA A3, B7 and B14). All patients responded to rHuEpo treatment (target haemoglobin level of 9-10 g/dl). None of the patients required iron supplementation or developed 'functional anaemia'. During 30 +/- 3 months of therapy, the initial maintenance dose of rHuEpo (103 +/- 12 units/kg/week) and median SF levels (2,250 micrograms/l) fell (50 +/- 8 units/kg/week and 1,060 micrograms/l, respectively) (p = 0.0003 and 0.0007). The initial and final rHuEpo doses correlated well with the respective SF levels (r = 0.89, p < 0.001). The maintenance dose of rHuEpo required for patients with IO at the start of the treatment period was significantly higher than that (50 +/- 5 units/kg/week) required by a control group of patients with adequate iron stores (SF = 100-600 micrograms/l) who were matched for age, sex and frequency of previous blood transfusions (p = 0.002). The findings suggested that excessive IO caused relative resistance to erythropoiesis on exogenous administration of rHuEpo and that iron supplementation was not warranted during rHuEpo therapy in those patients.

Adult↗

PubMed1980

An evaluation of 1 alpha-hydroxy-and 1,25-dihydroxyvitamin D3 in the treatment of renal bone disease.

40 patients with bone disease due to chronic renal failure have been treated with 1 alpha-hydroxyvitamin D3 or 1,25-dihydroxyvitamin D3 for a total of 750 patient-months. Both compounds were very effective in relieving bone pain and muscle weakness, and in reversing the radiographic and biochemical indices of disturbed skeletal metabolism. Their effects as judged from bone biopsies were, however, less complete, and histological improvement occurred only in a few patients. Patients with the combination of osteitis fibrosa and osteomalacia responded better than patients with either abnormality alone. Factors of importance in adversely influencing the outcome of treatment included a high pre-treatment level of calcium or immunoreactive parathyroid hormone, and a failure to augment secretion of calcitonin during treatment. It is concluded that a major therapeutic advantage of 1 alpha-OHD3 and 1,25(OH)2D3 over previously available forms of vitamin D is their rapid onset and reversal of action. These drugs do not invariably reverse bone disease and may give rise to unwanted effects. They should therefore only be used with adequate clinical, biochemical and radiographic supervision. They should not be used indiscriminately in all renal patients.

Adolescent↗

PubMed1980

Hemodialysis patients with a unique mineralizing defect unresponsive to 1,25-dihydroxycholecalciferol. Dialysis osteomalacic syndrome.

5 patients are described who developed severe osteomalacia with spontaneous fractures after 2-4 years on dialysis. Phosphate control, vitamin D2 therapy and parathyroidectomy were ineffective. These individuals showed a hypercalcemic tendency but little histologic or radiographic evidence of osteitis fibrosa. After parathyroidectomy, the hypercalcemic tendency remained and bone biopsy revealed gross osteomalacia. A 6- to 12-month therapeutic trial with 1,25-dihydroxycholecalciferol (1,25[OH]2D3) in 3 did not arrest skeletal deterioration. 4 subsequently developed dialysis encephalopathy. These patients appear to have a unique mineralizing defect unresponsive to 1,25(OH)2D3. This "dialysis osteomalacic syndrome" may result from toxic substances associated with uremia or the hemodialysis regimen.

Adult↗

PubMed1991

Evolution of secondary hyperparathyroidism during oral calcitriol therapy in pediatric renal osteodystrophy.

The course of renal bone disease in children undergoing regular peritoneal dialysis has not been fully evaluated. In particular, only limited data are available regarding the therapeutic efficacy of oral calcitriol in this group of patients. Thus, bone biopsies were done before and after 12 months of treatment with daily doses of oral calcitriol in 33 pediatric patients undergoing CAPD or CCPD. In 10/13 patients with initial biopsy findings of osteitis fibrosa, skeletal lesions either failed to improve or worsened after 12 months of treatment; in contrast, 4/6 patients with mild lesions of secondary hyperparathyroidism demonstrated histologic improvement. Serum PTH levels, determined using a mid-region assay, were higher in patients who failed to improve during oral calcitriol therapy, and values increased progressively in this group. Serum PTH levels were unchanged in those who improved. These data indicate that secondary hyperparathyroidism remains a substantial clinical problem in pediatric patients receiving regular peritoneal dialysis; treatment with daily doses of oral calcitriol is only partially effective for the control of this disorder. Further assessment of alternatives to daily oral calcitriol administration such as pulse oral or intermittent intraperitoneal calcitriol therapy is warranted in order to provide more effective approaches to the management of secondary hyperparathyroidism in pediatric dialysis patients.

Administration, Oral↗