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Biomedical subjects

F Pons

Publications and source records attributed to F Pons.

At least 109 records · Page 6Linked to original sources

Preventing postmenopausal bone loss with ossein-hydroxyapatite compounds. Results of a two-year, prospective trial.

OBJECTIVE: To evaluate, in postmenopausal women who refuse hormone replacement therapy (HRT), whether continuous administration of an osseinhydroxyapatite compound (OHC) reduces bone loss and protects from osteoporosis. STUDY DESIGN: Sixty postmenopausal women were included in an open study and were allocated to three groups. The first group (n = 19) received treatment consisting in 3.32 g/d of OHC per day, the second group (n = 21) received 2.5 g of calcium carbonate per day, and the third group (n = 20) was a treatment-free control group. Bone mineral density (BMD), assessed by dual x-ray absorptiometry, was measured prior to and at 12 and 24 months of treatment. RESULTS: Subjects on OHC therapy did not show significant changes related to baseline on bone mass across the study, whereas a significant decrease was detected in the calcium carbonate group during the second year (-3.7%, P < .05) and in the control group at the first and second BMD measurement (-3.5%, P < .05; -5.6%, P < .01). CONCLUSION: Continuous administration of OHC prevents bone loss in postmenopausal women, suggesting that this drug may be useful in the management of postmenopausal bone loss.

Aged↗

Mosaic expression of two dystrophins in a boy with progressive muscular dystrophy.

A boy with a Becker muscular dystrophy (BMD) phenotype presented unique muscular dystrophin expression. Western blot analysis showed the presence of two dystrophins of different sizes, i.e., a 400-kDa dystrophin and a 500-kDa form. An immunofluorescent study revealed mosaic expression of these dystrophins in the sarcolemma, with matching alpha-sarcoglycan and beta-dystroglycan staining patterns. DNA and RNA analysis did not reveal any mutation in the dystrophin gene, and the karyotype was normal.

Antibodies, Monoclonal↗

Has double-phase MIBI scintigraphy usefulness in the diagnosis of hyperparathyroidism?

The usefulness of double-phase parathyroid technetium-99m-MIBI scintigraphy for the detection of hyperplastic parathyroid tissue has been described. The aim of the present study was to establish the effectiveness of this new technique in the morphological and functional assessment of parathyroid glands in patients with different types of hyperparathyroidism. We performed 99mTc-MIBI scintigraphy (MIBI) and neck ultrasonography in 38 patients with primary (n=16) or secondary (n=22) hyperparathyroidism. All patients underwent surgical neck exploration, removing a total of 84 parathyroid glands. Before and after surgery, blood intact parathyroid hormone (iPTH) was measured peripherally and in both the right and left internal jugular veins. In patients with primary hyperparathyroidism, ultrasonography showed one enlarged gland in 11 cases (69%), while MIBI was positive in 15 (94%) (including two ectopic glands). The sensitivity of MIBI (93%) was greater than that of ultrasonography (68%), with a similar specificity (100 and 97%, respectively). In patients with secondary hyperparathyroidism, there was a discrepancy between both imaging modalities in 29 glands (33%). The sensitivity of both techniques was similar (41 and 54%, respectively), with the same specificity (89%). There were more difficulties in detecting the upper than the lower pathological glands. MIBI reflected more accurately the functionality of the glands, and ultrasonography has a better correlation with the volume and weight. In conclusion, Tc-99m-MIBI scintigraphy is a good technique to identify parathyroid hyperfunctioning tissue in cases of primary hyperparathyroidism and to detect ectopic glands, but it does not give significantly better results than conventional ultrasonography in patients with secondary hyperparathyroidism.

Female↗

Mid-arm sentinel lymph nodes showing surprising drainage from a malignant melanoma in the forearm.

A 51-year-old man with a malignant melanoma in his left forearm was studied to detect the sentinel lymph node and to assess the possibility of micrometastases in regional lymph nodes. Lymphoscintigraphy demonstrated two sentinel lymph nodes in the midarm. Two other nodes in the same location as well as in the left axilla were also observed. The exact location of the sentinel lymph nodes was identified with a gamma-ray detector. At the time of surgery, blue dye was injected around the primary lesion and the two sentinel lymph nodes on the inner side of the left arm were resected. Both lymph nodes were pigmented black. The histopathologic study demonstrated metastases from malignant melanoma in both nodes. This case reflects the main role of lymphoscintigraphy in identifying draining lymph nodes in unusual locations as observed in this patient.

Axilla↗

Congenital muscular dystrophy and cerebellar atrophy.

Two siblings and two other unrelated patients had congenital muscular weakness and dystrophic changes but normal immunocytochemical stainings for merosin, dystrophin, and dystrophin-related proteins on muscle biopsy. All had marked ataxia and cerebellar atrophy or hypoplasia. Cerebral white matter and cortical organization appeared normal.

Atrophy↗

Effects on bone mass of two oral contraceptives containing ethinylestradiol and cyproterone acetate or desogestrel: results of a 2-year follow-up.

BACKGROUND: There are conflicting data about the effects of oral contraceptives on bone mass in young women. Moreover, amenorrheic women may also be affected to varying degrees by osteopenia. Both conditions (oral contraceptive use and amenorrhea) are not infrequent in women during their reproductive years. AIM: To evaluate the safety for bone mass of two oral contraceptives, one containing cyproterone acetate and the other containing desogestrel. METHODS: A total of 67 women between 19 and 29 years of age were studied. Of these women, 35 were selected to receive an oral contraceptive containing 35 mg ethinylestradiol and 2 mg cyproterone acetate. A second group of 32 women received an oral contraceptive containing 30 mg ethinylestradiol and 150 mg desogestrel for 2 years. Immediately before and after the 12th and 24th months of therapy, bone mineral density was assessed by dual X-ray absorptiometry, and hormonal profiles and Ferriman-Gallwey scores were evaluated. RESULTS: After the 2-year follow-up, all subjects showed regular cycles, no pregnancies, and no changes in bone mass density. In addition, hirsute subjects showed a decrease of almost 42% in hirsutism scores. CONCLUSION: Oral contraceptives containing cyproterone acetate or desogestrel in the studied doses have no deleterious effects on bone marrow.

Adult↗

Effect of a 14-day spaceflight on dystrophin associated proteins complex in rat soleus muscle.

One of the most obvious effects of hypokinesia and hypodynamia is muscular atrophy. Changes in myosin expression are induced by the chronic elimination of the weight-bearing activity, particularly in the postural muscles, which occur during spaceflight and hindlimb suspension. Other morphological changes, such as the remodeling of myotendinous junction, are also induced by this reduction in mechanical stress. Moreover, the transversal interface between the cytoskeleton and the extracellular matrix of the muscle fiber can also be modified by the functional demand imposed on muscle. Dystrophin and its associated proteins appear to be essential for the stability of this interface and the deletion of one of these proteins results in a pathological phenotype. Changes in their expression appears to be induced by muscle disuse. We have quantified the changes in the expression of dystrophin and associated proteins induced by a 14-day spaceflight.

Animals↗

[Detection of sentinel lymph nodes by lymphatic gammagraphy and intraoperative gamma-ray probe in patients with malignant melanoma. Initial results].

In the last years there has been an arising concern in the sentinel lymph node identification, the first lymph node to receive direct draining from the primary tumour, specially in malignant melanoma (MM). We studied 20 patients with MM: 10 with palpable regional lymph nodes and 10 without palpable LN by performing a lymphoscintigraphy using 99mTc-nanocolloid and a gamma-ray detecting probe during the surgery to locate the sentinel lymph node. In patients with palpable LN, 13 sentinel lymph nodes were identified. Ten of them were MM involved. Furthermore, 82 LN were harvested from involved lymph basins and 30 of them were positive for MM. In patients without palpable LN, 14 sentinel lymph nodes were identified (3 positives and 11 negatives for MM) and other 76 LN were resected (all of them negative). There were not < > in any patient. These preliminary results support the utility of the technique for the diagnosis and lymphadenectomy selection in patients without palpable LN but which could be involved by micrometastases.

Adult↗

[Use of bone SPECT of the dorsolumbar spine in oncological patients with suspected bone metastases].

UNLABELLED: A retrospective study was made to evaluate the usefulness of bone SPECT of the dorsolumbar spine in identifying benign and malignant lesions. A study was made of oncological patients who presented active images in the spinal column visible on planar radionuclide scans who also underwent SPECT. The average follow-up period was 25+/-13 months. MATERIAL AND METHOD: In 43 neoplastic patients 55 visible lesions on SPECT were evaluated, of which 29 were benign and 26 metastatic. The criteria for benign lesions were the absence of change or involution without treatment in the follow-up period. The criteria for classifying metastases were: 1. Positive biopsy. 2. Neurological complications derived from the lesion. 3. Increased extension and tracer uptake by the lesion, and the appearance of other bone metastases. RESULTS: Twelve of 15 lesions located in the vertebral body or pedicle and 11/16 located in the body with extension toward the pedicles were metastases. Twenty-one of 24 lesions located on the anterior face of the vertebral body, articular processes, lamina, or intervertebral disk were benign. CONCLUSION: Bone SPECT was useful for locating the vertebral lesion, which in many cases allows differentiation of benign and metastatic lesions.

Adult↗

[Phrenic nerve malignant schwannoma: an exceptional diagnosis].

We report a case of malignant schwannoma of left phrenic nerve in a 65-year-old man. This uncommon diagnosis was suggested by radiological features and confirmed by histological findings on a needle biopsy and surgery specimens. We discuss management, follow-up and prognosis of malignant schwannomas.

Aged↗

Relationship between biochemical markers of bone turnover and bone scintigraphic indices in assessment of Paget's disease activity.

OBJECTIVE: To evaluate the relationship between biochemical markers of bone turnover and bone scan indices of disease activity, as well as to analyze their variations based on skeletal involvement, in Paget's disease. METHODS: Serum samples were obtained from 51 patients with Paget's disease to determine the levels of total alkaline phosphatase (total AP), bone alkaline phosphatase (bone AP), propeptide carboxyterminal of type I procollagen (PICP), propeptide aminoterminal of type I procollagen (PINP), osteocalcin, tartrate-resistant acid phosphatase, and telopeptide carboxyterminal of type I collagen. Urine samples were analyzed for levels of hydroxyproline (HYP), pyridinoline (PYR), deoxypyridinoline (DPYR), C-terminal telopeptide of type I collagen (CTx), and N-terminal telopeptide of type I collagen (NTx). In addition, 2 semiquantitative scintigraphic indices, disease activity (AI) and disease extent (EI), were obtained. Pagetic skeletal locations were evaluated individually, with special attention to skull involvement. RESULTS: All biochemical markers correlated with the AI and the EI. Serum PINP, bone AP, and total AP showed the highest proportions of increased values among the bone formation markers (94%, 82%, and 76%, respectively). Among the bone resorption markers, urinary NTx showed the highest proportion of increased values in patients with Paget's disease (96%), compared with PYR (69%), DPYR (71%), CTx (65%), and HYP (64%). In patients with mild disease activity, serum PINP was the marker with the highest proportion of increased values (71%). In contrast, serum PICP and urinary CTx were the most discriminative markers for skull involvement. Except for higher values for most of the biochemical markers of bone turnover in flat bones, no major differences in other skeletal locations were observed. CONCLUSION: The determination of serum PINP as a marker of bone formation and urinary NTx as a marker of bone resorption provided the best biochemical profile to ascertain the extent and activity of Paget's disease. In patients with skull involvement, serum PICP and urinary CTx were shown to be the most discriminative markers.

Acid Phosphatase↗

Osteoporosis and bone mineral metabolism disorders in cirrhotic patients referred for orthotopic liver transplantation.

The purpose of this study was to determine the prevalence of osteoporosis, to estimate the bone turnover and hormonal status, and to identify the factors associated with bone disease in patients with end-stage liver disease who were referred for orthotopic liver transplantation. A prospective study was performed on 58 cirrhotic patients (6 with primary biliary cirrhosis, 14 with alcoholic cirrhosis, and 38 with posthepatitic cirrhosis), who were referred for orthotopic liver transplantation. Patients, excluding those with primary biliary cirrhosis, were classified in Child-Pugh groups according to the severity of liver disease (class B [28 patients], class C [24 patients]). Biochemical parameters of bone mineral metabolism and standard liver function tests were measured in all patients. Additionally, serum osteocalcin, urinary hydroxyproline/creatinine ratio, serum intact parathyroid hormone, serum 25-hydroxyvitamin D, serum 1,25-dihydroxyvitamin D, follicle-stimulating hormone, and luteinizing hormone levels were determined in patients and controls within the same age range. Plasma testosterone, sex hormone-binding globulin levels, and free testosterone index were obtained for all men included in the study. Bone mass of the lumbar spine and femur were measured by dual X-ray absorptiometry (DPX-L), and were expressed as a standard deviation of mean values (Z-score) from a sex and age-matched control group. Spinal X-rays were obtained to assess vertebral fractures. Osteoporosis was considered as a factor in spinal bone mineral density with a Z-score below 2 or at least one vertebral fracture. Twenty-five patients (43%) had osteoporosis, with lower bone mass measurements in the lumbar spine than in the femoral neck (P < 0.005). Alcoholic and Child-Pugh C patients showed the lowest femoral bone mineral density values. Cirrhotic patients showed lower osteocalcin levels than controls (14.3 +/- 5.9 vs. 18.2 +/- 8.1 ng/ml; P < 0.05) and showed increased urinary hydroxyproline (125.1 +/- 51.5 vs. 107.9 +/- 26.6 nM/mg creatinine; P < 0.05). Serum 25-hydroxyvitamin D, 1,25-dihydroxyvitamin D and parathyroid hormone levels were significantly lower in cirrhotic patients than in controls (10.3 +/- 9.1 vs. 23.1 +/- 26.6 ng/ml; P = 0.000), (12.9 +/- 9.1 vs. 48.3 +/- 11.5 pg/ml; P = 0.000), (16.6 +/- 9.2 vs. 27.9 +/- 8.2 pg/ml; P = 0.000), with no differences between Child-Pugh groups. Alcoholic Child-Pugh C patients showed the lowest 25-hydroxyvitamin D serum values (4.5 +/- 2.2 ng/ml; P < 0.05). Male patients had lower testosterone levels than controls (302.5 +/- 229.4 vs. 556.7 +/- 146.5 ng/dl; P = 0.000), with increased sex hormone-binding globulin values. Levels of testosterone and gonadotropin were related to Child-Pugh classification. No correlation was found between bone mass and hormonal values. A significant decrease in bone mass, particularly in the lumbar spine, is seen in end-stage cirrhotic patients. Reduced bone formation and significant disorders of bone mineral metabolism, such as vitamin D deficiency, reduced parathyroid hormone levels, and hypogonadism are involved. Moreover, severity and etiology of the liver disease are the main risk factors for developing bone loss and mineral metabolism disorders in patients referred for orthotopic liver transplantation.

Adult↗

Biochemical markers of bone turnover in Camurati-Engelmann disease: a report on four cases in one family.

Moderate increases in "classical" biochemical markers of bone turnover have been described only in some patients with Camurati-Engelmann disease. However, the determination of the following "new" markers has not been previously performed: serum osteocalcin (BGP), bone alkaline phosphatase (BAP), carboxyterminal propeptide of type I procollagen (PICP), aminoterminal propeptide of type I procollagen (PINP), tartrate-resistant acid phosphatase (TRAP), telopeptide carboxyterminal of type I collagen (ICTP), urinary pyridinoline (PYR), crosslinked N-telopeptides of type I collagen (NTX), and Crosslaps (CL). Such a determination may improve the evaluation of the disease activity. To evaluate the usefulness of biochemical markers of bone turnover reflecting Camurati-Engelmann disease activity we measured the levels of all these markers in four affected patients. The results were compared with bone scintigraphic indices of disease activity. Except for PICP and TRAP, bone formation and resorption markers were abnormal in all patients and were related to bone scan indices of disease activity. Among the markers of bone formation PINP, BAP, and BGP showed the highest values, whereas NTX and CL were the most sensitive markers of bone resorption. These results suggest that the determination of NTX or CL, and PINP or either BAP and BGP, associated with bone scan evaluation, provides the best assessment of Camurati-Engelmann disease activity.

Acid Phosphatase↗