Differential action of pamidronate on trabecular and cortical bone in women with involutional osteoporosis.

Since osteoporotic fractures are mainly related to the diminution of the bone mineral density (BMD), the effect of pamidronate (3-amino-1-hydroxy-propylidene) 1,1-bisphosphonate on the BMD of the spine, proximal femur and radius shaft was evaluated in an initial cohort of 35 postmenopausal women with at least one vertebral fracture due to involutional osteoporosis. Pamidronate was given continuously during 18 months in a daily oral dose of 4.8 to 6.0 mg/kg supplemented with calcium (1 g/day). BMD--measured by dual photon absorptiometry--increased after one year 5.3 +/- 1.0% (P less than 0.001) in lumbar spine and 5.3 +/- 1.5% (P less than 0.001) over trochanter. However no significant changes were observed in the BMD of the femoral neck, Ward's triangle or in the cortical bone of the radius shaft measured by single photon absorptiometry. Pamidronate also decreased significantly urinary hydroxyproline-creatinine excretion after 6 months and thereafter maintained a plateau. After 18 months of treatment the diminution was 42.6 +/- 4.9% (P less than 0.001). The differing effects of pamidronate on the BMD of lumbar spine and proximal femur might be ascribed to dissimilarities between the proportions of trabecular and cortical bone in these. These results suggest that pamidronate may be prescribed to prevent fractures in cases of involutional osteoporosis with a significant decrease of BMD in lumbar spine and/or trochanter.

Metastases of carcinoma in the pagetic bone. A report of two cases.

Two men, aged 68 and 77 years, had metastases of carcinoma in pagetic bones. These cases were initially diagnosed clinically as Paget's sarcoma. Roentgenograms, scintigrams, and bone puncture biopsy specimens (BPBs) of the right scapula and the sacrum, respectively, showed only Paget's disease. However, an additional BPB obtained with the aid of computed axial tomography (CAT) revealed the presence of metastases of lung carcinoma in the right scapula and of a carcinoma of probable prostatic origin in the pagetic sacrum in the other. The observations testify to the usefulness of CAT-directed BPB when Paget's sarcoma is suspected, especially in cases located in the scapula, pelvis, or spine.

Disminución del contenido mineral oseo en los pacientes en hemodialisis de mantenimiento / Decrease of bone mineral content in patients in maintenance hemodialysis

Expónense los resultados de la valoración del contenido mineral óseo (CMO) de la cortical periférica, mediante la técnica de absorción de fotones, en 178 pacientes de ambos sexos, con insuficiencia renal terminal, sometidos a hemodiálisis de mantenimiento (HDM). Esta población correspondía a la ciudad de Buenos Aires y conurbano y la edad se limitó entre 21 y 80 años. El estudio transversal de todos los pacientes, con una permanencia media en HDM de 26,2ñ19,9 meses, reveló una disminución del CMO cortical en relación con la duración del tratamiento, ajustada a una función exponencial (r=0,38; p<0,001), caracterizada por una mayor reducción durante los 24 meses iniciales del tratamiento, seguida de una caída más gradual. El estudio longitudinal realizado en 12 pacientes durante un período promedio de 15 meses, confirmó un promedio de reducción mensual de CMO de -0,23ñ0,08. Destácase que el sexo y la edad no incidieron significativamente en las variaciones del CMO

Disminución del contenido mineral oseo en los pacientes en hemodialisis de mantenimiento / Decrease of bone mineral content in patients in maintenance hemodialysis

Expónense los resultados de la valoración del contenido mineral óseo (CMO) de la cortical periférica, mediante la técnica de absorción de fotones, en 178 pacientes de ambos sexos, con insuficiencia renal terminal, sometidos a hemodiálisis de mantenimiento (HDM). Esta población correspondía a la ciudad de Buenos Aires y conurbano y la edad se limitó entre 21 y 80 años. El estudio transversal de todos los pacientes, con una permanencia media en HDM de 26,2ñ19,9 meses, reveló una disminución del CMO cortical en relación con la duración del tratamiento, ajustada a una función exponencial (r=0,38; p<0,001), caracterizada por una mayor reducción durante los 24 meses iniciales del tratamiento, seguida de una caída más gradual. El estudio longitudinal realizado en 12 pacientes durante un período promedio de 15 meses, confirmó un promedio de reducción mensual de CMO de -0,23ñ0,08. Destácase que el sexo y la edad no incidieron significativamente en las variaciones del CMO (AU)

Treatment of Pagets bone disease with a bisphosphonate.

Exponese la experiencia obtenida con el tratamiento de 57 pacientes afectados de osteitis de Paget con 3-amino-1-hidroxipropilidene (APD) suministrado en dosis iniciales de 8,0-8,5 mg/Kg. Dieciocho pacientes no habian sido tratados anteriormente, en tanto que 39 habian sido medicados previamente con etidronato bisodico y/o con calcitonina. Se verifico mejoria clinica en el 77,8% y un descenso significativo de la fosfatasa alcalina suerica y de la hidroxiprolina urinaria en el 78% de los pacientes. Las respuestas clinicas y humorales fueron aparentemente mejores en los pacientes sin tratamiento previo, aunque las diferencias no fueron estadisticamente significativas. Se destaca que hubo intolerancia gastrica en el 12,3%, ausencia de remision clinica y/o agravacion de la sintomatologia en el 22,2% y ausencia de respuesta humoral en el 22,0% de los casos. Se comprobo aumentos significativos de la densidad radiologica osea en cinco casos. Se considera que la indicacion del APD corresponde preferentemente en: 1) pacientes que han dejado de reaccionar al etidronato bisodico o que no toleran la calcitonina; 2) pacientes con lesiones poliostosicas y valores elevados de fosfatasa alcalina e hidroxiprolina urinaria; 3) lesiones predominantemente osteoliticas, y 4) localizaciones craneales que se acompanan de fosfatasa alcalina muy elevada

Treatment of Paget's bone disease with a bisphosphonate.

Exponese la experiencia obtenida con el tratamiento de 57 pacientes afectados de osteitis de Paget con 3-amino-1-hidroxipropilidene (APD) suministrado en dosis iniciales de 8,0-8,5 mg/Kg. Dieciocho pacientes no habian sido tratados anteriormente, en tanto que 39 habian sido medicados previamente con etidronato bisodico y/o con calcitonina. Se verifico mejoria clinica en el 77,8% y un descenso significativo de la fosfatasa alcalina suerica y de la hidroxiprolina urinaria en el 78% de los pacientes. Las respuestas clinicas y humorales fueron aparentemente mejores en los pacientes sin tratamiento previo, aunque las diferencias no fueron estadisticamente significativas. Se destaca que hubo intolerancia gastrica en el 12,3%, ausencia de remision clinica y/o agravacion de la sintomatologia en el 22,2% y ausencia de respuesta humoral en el 22,0% de los casos. Se comprobo aumentos significativos de la densidad radiologica osea en cinco casos. Se considera que la indicacion del APD corresponde preferentemente en: 1) pacientes que han dejado de reaccionar al etidronato bisodico o que no toleran la calcitonina; 2) pacientes con lesiones poliostosicas y valores elevados de fosfatasa alcalina e hidroxiprolina urinaria; 3) lesiones predominantemente osteoliticas, y 4) localizaciones craneales que se acompanan de fosfatasa alcalina muy elevada

Healing of osteitis fibrosa cystica after partial parathyroidectomy despite persistent parathyroid hyperfunction: a case report.

The diagnosis of primary hyperparathyroidism was made in a 55-year-old female with marked hypercalcemia (15 mg/100 ml) and hypercalciuria and skeletal x-rays showing typical lesions of subperiosteal resorption and numerous cystic areas. At surgery a left lower parathyroid adenoma presumably was totally excised. Serum calcium returned to normal levels immediately postoperatively, but 4 months after surgery hypercalcemia (11.5 mg/100 ml) was again found. During the following 18 months the patient was free of symptoms, and radiologic studies revealed complete healing of the subperiosteal resorption and repair of the bone cysts. Cortical bone mineral content also increased. However, elevated levels of serum parathyroid hormone (PTH) and calcium clearly indicated the persistence of primary hyperparathyroidism. Thus, a decrease in the degree of PTH hypersecretion permitted the repair of the skeletal lesions caused by a more severe level of parathyroid hyperfunction.

[Evaluation of clinical and humoral data in 148 patients with Piaget's bone disease]. / Osteitis deformante de Paget. Manifestaciones clínicas y humorales en 148 pacientes.

Pain was present in 113 out of a total of 148 patients with Paget's bone disease who were evaluated. The symptom was originated by: a) bone, in 56.6 % of the cases; b) joints, in 32.7 %; c) skull compression in 7.1 % and spine compression in 3.5 % of the cases. Bone deformities were seen in 50.7 % of patients. Of this total, 41.4 % were localized in femorae. Pathological fractures were found in 16.9 % of patients. Of this total, 44.0 % were localized in femore and 19.2 % in the humerae. The localization of Paget's bone disease in the humerus appears to be specially risky due to the high percentage of fractures found in the present study as well as the frequency of the sarcomatous degeneration described in the literature. Two patients (1.4 %) developed an osteogenic sarcoma in bones previously affected by Paget's disease. It was localized in the tibia in one case and in iliac bone in the other. Frequent associated diseases were osteoporosis, verified in 31.8 % of the patients, arthritis deformans in 28.6 % and nephrolithiasis, verified in 31.8 % of the patients, arthritis deformans in 28.6 % and nephrolithiasis in 12.2 %. Associated neoplastic diseases were mammary cancer in 6.5 % of 77 women and colonic cancer in 2.0 of 148 patients of both sexes. The diagnosis has been made previously or during the period of the present study. The effects of the extent of Paget's bone disease as well as the effect of Paget's skull complication on serum alkaline phosphatase and urinary total hydroxyproline were investigated. Serum alkaline phosphatase and urinary total hydroxyproline were higher in polyostotic then in oligostotic patients although the difference was not significant for serum alkaline phosphatase in a group of 50 polyostotic with skull lesions versus 16 oligostotic with skull lesions. There was a significant increase of serum alkaline phosphatase in groups of 29 polyostotic without skull lesions versus 45 oligostotic without skull lesions (p < 0.001) and in groups of 50 polyostotics with skull lesions versus 29 polyostotics with skull lesions (p < 0.05), and of 16 oligostotic withh skull lesions versus 45 oligostotics without skull lesions (p < 0.02). There was a significant increase of urinary total hydroxyproline in a group of 46 polyostotics with skull lesions versus 15 oligostotics with skull lesions (p < 0.01) and of 25 polyostotics without skull lesions versus 41 oligostotics without skull lesions (p < 0.001), as well of 46 polyostotics with skull lesions versus 25 polyostotics without skull lesions (p < 0.005) and 15 oligostics with skull lesions versus 41 oligostotics without skull lesions (p < 0.02). Although the differences should be referred to a greater number of patients, the results suggest that the effects of Paget skull lesions are more evident on serum alkaline phosphatase than on urinary total hydroxyproline.

The treatment of Paget's bone disease with sodium ethidronate.

Of 58 patients with Paget's disease treated with disodium ethane-1-hydroxy-1,1-diphosphonate (EHDP) in doses of 20 and 10 mg/kg/day, a group of 20 patients with the oligostotic form of the disease showed a significantly greater incidence of complications, such as worsening of bone pains, when compared with a group of 38 patients with the polyostotic disease (P less than 0.05). The group of 32 patients that received 20 mg/kg/day showed a greater although not significant incidence of clinical complications than the group of 26 patients treated with 10 mg/kg/day. Bone biopsies performed in one polyostotic and three oligostotic cases who suffered episodes of bone pain worsening during treatment with 20 mg/kg/day disclosed a severe osteomalacia. Both groups treated with 20 and 10 mg/kg showed a highly significant decrease of urinary hydroxyproline (THP) excretion and of serum alkaline phosphatase (P less than 0.01) after two and six months of treatment although the trend comparison between doses was not significant. It is suggested that the dose of EHDP should be related to the extent of the disease.

Pycnodysostosis: some aspects concerning kinetics of calcium metabolism and bone pathology.

A 23-year-old white woman suffering pycnodysostosis whose parents and five siblings were unaffected was investigated. Chromosomal morphology was normal. Histologic examination of a biopsy specimen obtained from the distal phalanx of the left thumb, corresponding to an area on an x-ray film of osteolysis, showed a fibrous dysplasia-like picture. Kinetic studies of calcium metabolism revealed that exchangeable pool size, turnover, and bone accretion rate were all decreased. Intestinal calcium absorption was investigated simultaneously by a double isotope technique and by deconvolution of the plasmatic specific activity curve of 47Ca given per os. Results obtained by both methods coincided in that values were found to be similarly increased. Endogenous fecal calcium was also determined and revealed a similar increase. Some physiopathological implications of these findings are discussed.