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Biomedical subjects

C Mautalen

Publications and source records attributed to C Mautalen.

At least 73 records · Page 4Linked to original sources

Intravenous aminopropylidene bisphosphonate (APD) in the treatment of Paget's bone disease.

We studied the effect of the intravenous administration of the bisphosphonate APD in 9 patients with Paget's bone disease. The medication was given in a daily dose of 25 mg for 7 days in 0.9% saline infusion over 2 hours. At the end of treatment a significant fall of serum calcium and phosphate was observed. The urinary excretion of calcium decreased markedly and the serum levels of the mid-molecule PTH fragment increased from (mean +/- SE) 85 +/- 11 to 122 +/- 16 pg/ml (p less than 0.05). A marked and rapid decline in the hydroxyprolinuria was observed from 297 +/- 61 mg/24 h to 194 +/- 51 mg/24 h (p less than 0.01); meanwhile the serum alkaline phosphatase decreased from 102 +/- 22 to 84 +/- 21 KAU (p less than 0.05). The effect of ADP on suppression of hydroxyprolinuria varied markedly from +1 to -81% and was negatively related to the basal hydroxyprolinuria (r = -0.90; p less than 0.001). The duration of the bone turnover suppression was short. A relapse greater than 30% in hydroxyprolinuria was observed in 6 of 8 patients 2 to 3 months after APD withdrawal. The short-term intravenous administration of ADP is a useful means to rapidly suppress the activity of Paget's bone disease. However, further studies should determine the optimum dose, the length of treatment, and the need to associate oral therapy to induce a prolonged remission.

Aged↗

Comparison of the acute effect of the intranasal and intramuscular administration of salmon calcitonin in Paget's disease.

On 7 patients with mild-to-moderately active Paget's disease, 200 IU of salmon calcitonin (SCT) nasal spray (NS), induced a significant decrease of the total urinary hydroxyproline excretion (THP) during the 8-16 hour and the 0-24 hour (P less than 0.05) periods after treatment as compared to control day. However, the administration of 100 IU of SCT intramuscularly (i.m.) caused a significantly greater effect than 200 IU-NS during the second 8 hour period after its administration (P less than 0.01) and on the over-all 24 hour effect (P less than 0.05). On 3 patients with severe Paget's disease, SCT-NS was essentially ineffective whereas the injection of SCT induced a marked diminution of the THP excretion.

Administration, Intranasal↗

Acute antiosteoclastic effect of salmon calcitonin in osteoporotic women.

Twenty-one women with primary osteoporosis received an intramuscular injection of 100 IU of salmon calcitonin at 8 AM. Blood samples IU of salmon calcitonin at 8 AM. Blood samples were collected up to 5 h postcalcitonin and urine was collected for 24 h in three periods of 8 h each. A significant fall of the total hydroxyproline excretion (THP) was observed in every period but the maximum effect took place 8-16 h postcalcitonin. The effect was more pronounced in those patients with a greater basal excretion of THP and in those with a more significant diminution of their bone mass. A significant diminution of the urinary excretion of calcium was found 8-24 h postcalcitonin, while phosphate excretion increased throughout the three 8 h periods. The effect of calcitonin in osteoporotic women was compared with the results obtained in 9 patients with Paget's disease. In absolute in 9 patients with Paget's disease. In absolute values the fall of THP excretion was significantly greater in the pagetic patients but the percentage diminution was not significantly different in osteoporotic women and in pagetic patients. Salmon calcitonin induced a significant acute diminution of bone turnover in patients with osteoporosis, as defined by decreases in total urinary hydroxyproline. The calcitonin-induced hypocalcemia does not seem to be an accurate index of the hormone action in osteoporosis.

Aged↗

Cortical bone mineral content in primary hyperparathyroidism. Changes after parathyroidectomy.

The bone mineral content (BMC) of 35 patients with primary hyperparathyroidism (PHPT) was measured at the mid radius (95% cortical bone) by photon absorptiometry of a 241Am source. The majority of the patients had an overt disease of moderate to severe degree. Average serum calcium of the group was 12.3 mg/100 ml (range 10.6 to 18.0 mg/100 ml). The percentage of normality of the BMC was (Av +/- 1 SD) 75.1 +/- 13.0% for the whole group. The average increment of BMC in 14 patients 9 to 26 months after parathyroidectomy was 9.9%, with a wide dispersion. However a highly significant negative correlation (r: 0.83; P less than 0.01) was found between the initial bone mass and the percentage increment per month after surgery. No further gain was observed 2 years after parathyroidectomy except in one patient with an extremely severe bone loss. In spite of the gain obtained after surgery the bone mass remained markedly diminished in most patients showing that the cortical bone loss caused by PHPT is mainly irreversible.

Adolescent↗

Spontaneous fractures of uninvolved bones in patients with Paget's disease during unduly prolonged treatment with disodium etidronate (EHDP).

Three patients with Paget's disease of bone were treated by disodium etidronate (EHDP) without interruption during periods of 18 to 30 months. In one case the daily dose was also unduly high (approximately equal to 18 mg/kg/day). A moderate to conspicuous diminution of the renal function was observed in all cases. The three patients developed skeletal pain in a gradual but progressively severe pattern. Seven nontraumatic fractures in nonpagetic bones were encountered. EHDP produces blockage of bone mineralization and excessive suppression of bone remodeling, therefore increasing the risks of fracture. EHDP affects not only pagetic bones but normal skeleton. A baseline evaluation of the renal function might help to identify those patients with greater risk to develop skeletal side effects during EHDP treatment.

Aged↗

Calcium phosphate metabolism and bone disease in patients with homozygous thalassemia.

Calcium and phosphate metabolism were studied in 22 patients with homozygous thalassemia. The overall results showed no significant difference for serum calcium, phosphorus, alkaline phosphatase, immunoreactive parathyroid hormone, or 25-hydroxyvitamin D between thalassemic and control children. However, during the winter, serum 25-hydroxycholecalciferol levels were very significantly decreased in thalassemic children. A study of the hands showed thin metacarpal cortices related to increased resorption. Histomorphometric study of four iliac bone biopsies showed normal osteoclastic resorption and decreased bone formation. Prussian blue staining and x-ray electron microprobe analysis showed iron deposits inside the bone. Whether this finding is critical in the pathogenesis of the bone disease in unknown.

Adolescent↗

Familial idiopathic hyperphosphatasia: a study of two young siblings treated with porcine calcitonin.

A study of two siblings with a severe infantile form of familial idiopathic hyperphosphatasia is reported. A girl aged one year was followed for two years while receiving intermittent treatment with porcine calcitonin. This induced a clinical remission, a reduction of both the high serum level of alkaline phosphatase and the raised urinary excretion of hydroxyproline, and a remarkable improvement in bone structure as seen radiologically. Her sister aged two months received porcine calcitonin for three weeks, during which clinical improvement, no change in the serum level of alkaline phosphatase and a marked decrease of the excretion of hydroxyproline were recorded.

Alkaline Phosphatase↗

Intestinal absorption of phosphate: action of protein synthesis inhibitors and glucocorticoids in the rat.

The effect of actinomycin D, cycloheximide and glucocorticoids on the intestinal absorption of phosphate was studied. The effective intestinal absorption of 32P and 47Ca was determined simultaneously in intact rats in vivo using a whole body counter. Both, actinomycin D and cycloheximide caused a significant diminution of the intestinal absorption of phosphate whereas calcium absorption was not altered. Experiments with the in situ ligated loop technique were performed to eliminate the possibility that the action of the protein synthesis inhibitors could be due to an altered intestinal motility effect. Phosphate absorption was also significantly diminished under this condition. On the other hand, the administration of glucocorticoids produced a significant inhibition of phosphate and calcium absorption in the rat in vivo. The reported results indicate that proteins and/or enzymes with a rapid turn-over are involved in the mechanism of phosphate intestinal absorption, and confirm previous observations that phosphate and calcium are transported across the intestine by different mechanisms.

Animals↗

Early skeletal effect of alkali therapy upon the osteomalacia of renal tubular acidosis.

The administration of alkaline agents to a 16-year-old girl with severe renal tubular acidosis and osteomalacia caused an almost immediate rise of the urinary excretion of total hydroxyproline. The increment of the dyalizable fraction predominated over the nondyalizable component. Gradually serum phosphate and serum alkaline phosphatase increased whereas urinary calcium and magnesium and phosphate clearance declined. Serum PTH remained elevated throughout. We suggest that the correction of the metabolic acidosis might increase the transport of phosphate and calcium across the functional bone membrane leading to a rapid deposition of lime salts in the uncalcified matrix with a concomitant increase in bone collagen turnover.

Acidosis, Renal Tubular↗

Congenital erythropoietic porphyria: skeletal manifestations and effect of pamidronate treatment.

Congenital erythropoietic porphyria (CEP) is a rare disorder of heme biosynthesis. Skeletal abnormalities have been described in patients with this disease. We report a 25-year-old woman with osteodystrophy from CEP. On examination, mild hepatosplenomegaly, multiple hyperpigmented scars, hypertrichosis, erythrodontia and red coloration of urine were found. Biochemical studies showed increased serum levels of alkaline phosphatase, fasting and total 24-h urinary calcium excretion. Serum 250H vitamin-D concentration was low due to avoidance of sun exposure. Skeletal radiographs disclosed marked vertical and horizontal trabecular pattern and biconcavity of most of the dorsal and lumbar vertebral bodies. Several round sclerotic lesions (1-3 cm in diameter) were seen in the skull, pelvis and one lumbar vertebrae. The sclerotic lesions were augmented in size and number compared to X-rays obtained 8 years before. Bone mineral density (evaluated by DEXA) was markedly reduced at the spine and moderately diminished at the proximal femur and total skeleton. Treatment for 11 months with pamidronate (and the addition of hydrochlorotiazide for the last 6 months) reduced to normal values the serum levels of alkaline phosphatase and fasting urinary calcium. The 24-h urinary excretion of calcium and hydroxyproline were also decreased. The BMD increased in all the skeletal areas with presumably hyperactive bone marrow: spine, head, ribs and pelvis (and total skeleton), but did not change at the extremities and diminished at the femoral neck. Patients with CEP may present osteodystrophy characterized by sclerotic lesions and osteopenia, most likely due to accelerated bone turnover in areas of active bone marrow.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗