1/11. Familial idiopathic hyperphosphatasia (FIH): response to long-term treatment with pamidronate (APD).A 5-year-old child suffering from familial idiopathic hyperphosphatasia (FIH) was treated by: (1) intravenous infusion of pamidronate (APD) (3 h) (0.75 mg/kg/day) for 5 days; and (2) oral administration of APD (8 mg/kg/day) for 1 year, in association with calcium (1 g/day) as calcium gluconate. A decrease of both serum calcium and phosphate, and a slight PTH increase were observed immediately after the IV treatment; serum alkaline phosphatase did not change, but a marked and rapid decline in the hydroxyprolinuria was observed: basal 659 /- 207 during IV treatment 169 /- 59 (mean /- SD mg/24 h, P < 0.005). At the end of one year of oral APD treatment clinical and radiological findings showed a remarkable improvement. serum calcium, phosphate and PTH returned to the initial values. plasma alkaline phosphatase levels showed a 70% decrease: basal 1370 IU/l, 1 year 410 IU/l whereas the hydroxyprolinuria values were similar to those determined at the end of the intravenous treatment (212 /- 13 mg/24 h), but still significantly lower than the basal levels (P < 0.01). No side-effects were observed. APD appears to be a promising treatment for patients with FIH.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
2/11. Malignant transformation in Paget disease of bone.BACKGROUND. The previously reported incidence of malignant transformation of Paget disease up to 5.5% and its dismal prognosis have prompted the clinical investigation of a large population with Paget disease. methods. A chart review of symptomatic and asymptomatic patients with a diagnosis of Paget disease between 1970 and 1985 at four large Montreal hospitals revealed 1078 patients. Eight patients with malignant transformation were studied in detail. RESULTS. The incidence of malignant transformation was 0.7%, and the most frequent histologic type was osteogenic sarcoma. The most prevalent site was the femur, and pathologic fracture with focal osteolysis was present in 50% of patients at the initial appearance of the tumor. Healing at the fracture site was demonstrated in one patient. Another patient died of uncontrollable hemorrhage. A case of malignant lymphoma in Paget disease is recorded. CONCLUSIONS. Surgery or biopsy should be preceded by preoperative scintigraphic bone blood flow evaluation and, if necessary, administration of a preoperative course of mithramycin and selective embolization to minimize bleeding. The prognosis of malignant transformation in Paget disease is poor, although one patient with malignant lymphoma survived after aggressive treatment. Early biopsy and aggressive treatment should not be delayed.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
3/11. Two cases of monostotic Paget's disease: effects of bisphosphonate.We report two cases of monostotic Paget's disease which were effectively treated with bisphosphonate. Case 1 was a 60-year-old female. Medical examination revealed high alkaline phosphatase (ALP) levels making her visit our clinic. Hematological examination showed high levels of ALP isozyme 3 and bone metabolism markers, and bone scintigraphy demonstrated strong accumulation of 99mTc on the skull. With the diagnosis of monostotic Paget's disease of the skull, treatment with bisphosphonate (etidronate) was started. The response to etidronate was good and after 12 weeks of treatment, the ALP levels decreased to about 26% of the levels before treatment, without the appearance of any symptoms or lesion development. One year and three months later, ALP increased again, and etidronate administration was resumed. However, four years after the diagnosis of the disease, etidronate became ineffective and oral administration of alendronate, a stronger bisphosphonate, was started at 5 mg/day. The patient responded favorably to the bisphosphonate and is still under observation. Case 2 was a 71-year-old female. High ALP levels were found during the follow-up of type 2 diabetes, and the case was diagnosed as monostotic Paget's disease of the pelvis based on bone metabolism markers and bone scintigraphy. Etidronate treatment at 200 mg/day resulted in the improvement of bone metabolism markers and bone scintigraphy findings. When she died of colon cancer twelve months later, with no marked progress of the Paget's disease of bone observed clinically.- - - - - - - - - - ranking = 2keywords = administration (Clic here for more details about this article) |
4/11. MRI of Paget disease with temporal bone involvement presenting with sensorineural hearing loss.temporal bone involvement in Paget disease can result in hearing loss by a variety of mechanisms. One such mechanism is compression of neural structures within the internal auditory canal. This report describes such a case. magnetic resonance imaging characteristics are discussed, including the appearance of Paget disease following Gd-DTPA administration.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
5/11. Traumatic fracture in a healthy man: benign or pathologic?OBJECTIVE: To describe the challenge of determining the correct diagnosis in a healthy adult male patient with a recent femoral fracture and a history of multiple bone fractures. methods: We present clinical, radiologic, laboratory, and histopathologic details in a patient with a history of recurrent fractures associated with minimal trauma. Moreover, the various types of osteopetrosis are reviewed. RESULTS: A 34-year-old African American man was in his usual state of good health when he fell hard on concrete. Immediately after the fall, he was able to bear weight, although pain prompted him to seek medical care. Besides a personal history of multiple fractures, he had no other medical problems. He had never smoked, denied illicit drug use, and had no family history of bone disorders or recurrent fractures. Findings on physical examination were unremarkable. radiography disclosed an incomplete femoral fracture and osteosclerosis. Bone survey revealed diffuse, symmetric osteosclerosis of both the axial and the appendicular skeleton. The long bones showed areas of almost complete obliteration of the medullary canal, along with prominent hyperostosis. Additionally, a "bone-within-bone" appearance to the thickened endosteum was noted. A bone scan demonstrated numerous areas of symmetric radiotracer uptake. Laboratory analyses were unremarkable, including a complete blood cell count, electrolytes, serum protein electrophoresis, thyrotropin, and parathyroid hormone. Total alkaline phosphatase was mildly elevated at 162 U/L (normal range, 35 to 130). Seven needles were broken during attempts to perform a bone biopsy. Histologic examination showed normal bone marrow with "woven" bone and areas of primary spongiosa within mature osteoid. Autosomal dominant osteopetrosis type 2 was diagnosed on the basis of his clinical presentation and the radiologic and pathologic findings. CONCLUSION: The preliminary diagnosis for this patient's condition was Paget's disease, and determining the correct diagnosis of osteopetosis prevented the administration of inappropriate therapy. In addition, this case report reminds the clinician that genetic disease may manifest in adulthood.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
6/11. Pamidronate sodium and calcitonin-resistant Paget's disease. Immediate response in a patient.One three-day course of intravenous pamidronate sodium (3-amino-1-hydroxypropylidene-1,1-bisphosphonate), 30 mg/d, in a patient with calcitonin-resistant Paget's disease resulted in the following: marked clinical improvement within two weeks; normalization of urinary hydroxyproline value; fall of serum alkaline phosphatase value (900 to 250 U/L); a rise in serum osteocalcin value by the tenth week that returned to pretreatment levels in the 16th week; transient hypocalcemia with elevation of parathyroid hormone value; reduction in urinary calcium excretion; and improvement in bone scans. No adverse reactions occurred, with the exception of mild and transient hyperpyrexia for 48 hours during pamidronate administration. White blood cell counts did not change and serum interleukin 1 was undetectable before and after treatment with pamidronate. Pamidronate seems to be highly effective in the treatment of Paget's disease of the bone, but its profound effects on mineral and bone metabolism require close monitoring during the short-term period of intravenous treatment.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
7/11. Cardiac arrest associated with hypokalaemia in a patient receiving mithramycin.The case is reported of a patient who developed severe hypokalaemia, leading to cardiac arrest, while receiving mithramycin for painful Paget's disease. The patient was also receiving antihypertensive and anti-anginal treatment including bendrofluazide, but plasma potassium before the administration of mithramycin was normal. Hypokalaemia has been reported in previous studies of the use of mithramycin but has received little attention. The drug should be used with great care in patients with cardiovascular disease and in those receiving diuretics.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
8/11. spinal cord compression in Paget's disease of bone with reference to sarcomatous degeneration and calcitonin treatment.Four cases of spinal cord compression stemming from Paget's disease of bone tissue are reported. Sarcomatous degeneration was proved in one case and in another one malignancy was deduced by computed tomography (CT) scanning. Clinical and radiologic manifestations as well as surgical management are discussed. The value of CT scanning of the spine as an aid in the evaluation of structural changes and the size of the soft tissue mass in sarcomatous degeneration of the lesion is stressed. After calcitonin administration, alleviation of pain and improvement in neurological status have occurred in two treated patients.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
9/11. Paget's disease: ocular complications of disodium pamidronate treatment.Three cases of uveitis associated with disodium pamidronate have been notified to the Committee for safety of Medicines. Two new cases of bilateral anterior uveitis and episcleritis after administration of intravenous disodium pamidronate are described.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
10/11. Ototoxicity associated with intravenous bisphosphonate administration.The development of ototoxicity in association with intravenous pamidronate treatment of Paget's disease is described. The patient developed hearing loss, vertigo, and tinnitus which were exacerbated by subsequent infusions. This potentially serious drug side-effect does not appear to have been reported previously in patients with Paget's disease.- - - - - - - - - - ranking = 4keywords = administration (Clic here for more details about this article) |
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