PubMed Health⌕ Search

Biomedical subjects

J L Sebert

Publications and source records attributed to J L Sebert.

At least 55 records · Page 3Linked to original sources

[Bone involvement in primary oxalosis. Study of 20 cases].

The authors report 20 cases of primary oxalosis with bone involvement, late revealed in adults in 19 cases. They have studied the clinical, radiological and histological manifestations of this bone oxalosis. 19 cases had an end stage chronic renal failure, either treated by maintenance hemodialysis, or by renal (or liver-renal) graft. 17 patients complained of bone pain after starting hemodialysis; 3 had vertebral crush fractures, and 1 multiple spontaneous fractures. Diffuse bone sclerosis (with a homogeneous pattern on axial skeleton and a patchy appearance on the peripherical skeleton), bone translucency, subperiosteal phalangeal resorption were the main radiological symptoms. Oxalate crystals surrounded by a giant cells granuloma were always observed on bone biopsy (16 cases). Bone resorption was observed in 9 cases, hyperparathyroidism in 14 cases and osteomalacia in 7 cases. Hyperparathyroidism does'nt explain all the clinical and radiological manifestations (especially bone resorption). Bone resorption as other radiological and clinical manifestations can be found without hyperparathyroidism and can increase despite parathyroidectomy; so, bone resorption seems to be partly due to the granulomatous reaction around oxalate crystals rather than hyperparathyroidism.

Adolescent↗

Destructive spondyloarthropathy with beta 2-microglobulin amyloid deposits in a uremic patient before chronic hemodialysis.

We report a case of erosive arthropathies discovered radiologically before dialysis in a uremic patient with Alport syndrome. This patient had no hereditary amyloidosis or causes of acquired generalized amyloidosis (no chronic infections or inflammatory disease, neoplasia, lymphoma or monoclonal gammapathy). Erosive spondyloarthropathies of the cervical spine at the C5-C6 and C6-C7 levels, erosive arthropathy of the right acromioclavicular joint, metacarpal lacuna of the right hand, and lacuna of the left femoral neck were discovered 24 months before starting dialysis in this patient with chronic renal insufficiency of 17 years duration. Puncture of the vertebral disc before starting dialysis took a fragment showing amyloid deposits with permanganate-sensitive Congo red staining and positive staining with anti-beta 2-microglobulin antibodies. This observation suggests that beta 2-microglobulin amyloidosis in uremia may not be exclusively related to chronic kidney replacement therapy, but to uremia per se, especially when the latter is of long duration.

Amyloid↗

Comparison of intact, midregion, and carboxy terminal assays of parathyroid hormone for the diagnosis of bone disease in hemodialyzed patients.

The predictive value of three different RIAs of PTH for the diagnosis of the histological type of bone disease has been compared in 24 asymptomatic patients on chronic hemodialysis who had never been exposed to aluminum intoxication and who agreed to have a bone biopsy after double tetracycline labeling. The serum concentrations of PTH were measured using a two-site immunoradiometric assay for intact PTH(1-84) and region specific assays directed against the C-terminal (53-84) fragment or the midregion (44-68) of the molecule. The bone histomorphometric analysis showed that six patients had nonaluminic adynamic bone disease with low bone formation rate (BFR), eight had mild hyperparathyroidism characterized by increased bone resorption and normal BFR, nine had severe hyperparathyroidism with increased BFR, and only one had true osteomalacia with increased osteoid seam thickness. All PTH assays correlated with the various parameters of bone resorption and bone formation and were able to differentiate the histological type of bone disease only when groups of patients were considered. For classifying individual patients into severe hyperparathyroidism and adynamic bone disease groups, the intact PTH assay had the best predictive value with a sensitivity of 100% and a specificity of at least 70%. A nonaluminic adynamic bone disease was observed in more than 50% of the patients who had normal intact PTH levels (6/11). It is concluded that the intact PTH measurement is superior to C-terminal and midregion assays for the prediction of the histological type of bone disease in hemodialyzed patients and should be of considerable value to adapt their treatment in order to avoid the emergence of both severe hyperparathyroidism and adynamic bone disease. In the absence of aluminum intoxication it seems that maintaining intact PTH concentrations 1 to 1.5 times the upper limit of normal would correspond to the best bone histology.

Bone Diseases↗

[Fluoride bioavailability from sodium fluoride alone or in association with different calcium salts].

Fluoride pharmacokinetics were studied in twelve healthy subjects after oral administration of one tablet of osteofluor-containing 25 mg of sodium fluoride alone or associated with different calcium salts (500 mg Ca element) given as microcrystalline hydroxyapatite compound or powdered tri-calcium phosphate. Fluoride was measured in plasma and urine with a specific electrode and fluoride bioavailability from the three drugs was compared. Calcium absorption after oral load was assessed by measuring variations from the fasting state of plasma calcium and parathyroid hormone and of the urinary calcium to creatinine ratio. The bioavailability of fluoride is significantly decreased by the association to sodium fluoride of hydroxyapatite but not of tri-calcium phosphate. Calcium absorption seems greater after administration of hydroxyapatite compound. The decrease of the fluoride bioavailability is probably dependent on the capacity of calcium absorption from the calcium salts. The association of calcium to fluoride is absolutely required for treatment of osteoporosis. The microcrystalline hydroxyapatite compound seems preferable in view of its greater calcium absorption and of its limited decrease in fluoride bioavailability.

Administration, Oral↗

[Evaluation of the calcium content of diet by frequential self-questionnaire].

A frequential type self-questionnaire enabling evaluation of the calcium content of the diet of an individual as well as of a given population was designed on the basis of 20 different types of food (items) rich in calcium and/or frequently eaten in metropolitan France. Comparison with the weekly docket system, chosen as a reference method, validated the self-questionnaire. Similarity with the weekly docket method disappeared when the size of portions was left to one side, drinks were eliminated or only dairy products were taken into account. The self-questionnaire with its 20 items thus offers a simple and rapid method for estimation of the daily calcium intake of a given individual to within an accuracy of 20% and as result is a dietetic evaluation technique suitable for both clinical and epidemiological use.

Adolescent↗

Leukaemia-associated hypercalcaemia in a 10-year-old boy: effectiveness of aminohydroxypropylidene biphosphonate.

A 10-year-old boy with leukaemia-associated hypercalcaemia was treated with aminohydroxypropylidene biphosphonate (AHPrBP previously APD) in a total dosage of 60 mg over 5 days, when the condition failed to respond to rehydration and frusemide and no sustained effect was produced by haemodialysis with a calcium (Ca)-free dialysate. Bone films showed no lytic lesions, and AHPrBP, which is a potent inhibitor of osteoclast-mediated bone resorption was well tolerated and induced a rapid and sustained fall in plasma Ca (from 3.42 to 2.07 mM in 5 days). Plasma magnesium and alkaline phosphatase remained normal. The results could have been affected by other drugs [vincristine, cyclophosphamide, zorubicin (Rubidazone) L-asparaginase and prednisone] which were simultaneously administered. However, the observation that: (1) the response curve of plasma Ca was similar to that reported when AHPrBP was used alone, (2) there was complete inhibition of urinary Ca excretion and (3) hypocalcaemia occurred suggests that AHPrBP was the major cause of the reduction in plasma Ca. AHPrBP should be considered a potential therapy for hypercalcaemia in childhood malignancy.

Calcium↗

Disappearance of aluminic bone disease in a long term asymptomatic dialysis population restricting A1(OH)3 intake: emergence of an idiopathic adynamic bone disease not related to aluminum.

In dialysis centers using reverse osmosis-treated water but not restricting A1(OH)3 administration, a high prevalence of histological aluminum bone disease has been reported. To assess whether this is also the case in our center where A1(OH)3 intake has always been restricted and even completely given up after 1980 thanks to high doses of CaCO3, we reviewed 42 bone biopsies performed between 1975 and 1985 in patients dialyzed for a mean duration of 56 months. Seventeen of these patients had been dialyzed before 1978 with softened water moderately contamined by aluminum, 15 had always been dialyzed with reverse osmosis-treated water and 10 had been exclusively treated by hemofiltration. The prevalence of aluminum bone disease in the whole population was 9.5% (4 patients) and consisted only of adynamic bone disease, osteomalacia being totally absent. When the patients dialyzed with aluminum-contaminated water were excluded as well as 1 diabetic patient who had taken A1(OH)3 for 1.5 years the prevalence of aluminum bone disease was null in this population. When the whole population is considered the prevalence of the other types of bone disease was 76% for osteitis fibrosa and 14.5% for a non-aluminic adynamic bone disease (6 cases). These latter cases differed from the osteitis fibrosa group only by a relative hypoparathyroidism not explained by higher plasma concentrations and higher oral cumulative doses of calcium, magnesium and aluminum or by lower plasma concentrations of phosphate and bicarbonate. None had previous parathyroidectomy, one had an unsuccessful transplantation and one was diabetic. Iron overload was excluded by negative Perls staining.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗