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Concomitant osteitis fibrosa cystica and ameloblastoma of the mandible.

A patient with a multiloculated cystic lesion of the mandible, and clinical as well as biological features of hyperparathyroidism is presented. This association suggesting the diagnosis of brown tumor was actually due to an ameloblastoma of the mandible. Such a concomitant association of an ameloblastoma with symptomatic hyperparathyroidism has never been reported to our knowledge.

Adenoma↗

Parathyroid hormone-like bioactivity in a patient with severe osteitis fibrosa cystica due to malignancy: renotropic actions of a tumour extract as assessed by cytochemical bioassay.

A patient is described with malignancy, hypercalcaemia and radiological evidence of severe parathyroid bone disease but undetectable concentrations of circulating immunoreactive PTH. Autopsy showed the tumour to be a metastatic bronchial carcinoid with no evidence of primary parathyroid disease. Extracts of the tumour had no PTH immunoreactivity but had high concentrations of a substance with identical activity to PTH in a cytochemical bioassay. The biological activity of the extract was not inhibited by PTH antibodies but was inhibited by an antagonist to PTH bioactivity.

Aged↗

Current advances in the therapy of secondary hyperparathyroidism and osteitis fibrosa.

Secondary hyperparathyroidism is commonly observed in dialysic patients. Recent observations demonstrate a direct inhibitory effect of calcitriol on parathyroid hormone (PTH) synthesis and secretion. These observations may have important clinical and therapeutical implications. Thus, several studies have shown that intravenous calcitriol, in dialysis patients with severe secondary hyperparathyroidism, has a direct inhibitory effect on PTH levels. Furthermore, the sigmoidal PTH-calcium relationship is shifted toward a more normal range after intravenous calcitriol. In addition, the use of calcitriol early during the course of renal failure prior to dialysis has demonstrated therapeutic benefits. Thus, both early therapy with oral calcitriol and later during maintenance dialysis, the addition of the intravenous form may provide various therapeutical alternatives which make surgical parathyroidectomy rarely necessary. Furthermore, the course of patients undergoing surgical parathyroidectomy is not benign.

Calcitriol↗

Osteitis fibrosa cystica with renal parathyroid hormone resistance: a review of pseudohypoparathyroidism with insight into calcium homeostasis.

Pseudohypoparathyroidism is a group of diseases characterized by renal resistance to parathyroid hormone. The patients typically have the bony manifestations of hyperparathyroidism, while being hypocalcemic. Pseudohypoparathyroidism has further been subdivided into types Ia, Ib, Ic, and II. Mutations involving any number of domains of the parathyroid hormone receptor, adenylate cyclase, or G proteins may alter the cellular response to parathyroid hormone. This wide range of possible sites of mutation may explain the heterogeneous biochemical, skeletal, and physical phenotypes associated with the various types of pseudohypoparathyroidism. We describe a patient with pseudohypoparathyroidism who was successfully treated with total parathyroidectomy and gland autotransplantation. The complexities of parathyroid hormone cellular interactions and calcium homeostasis are discussed. Pseudohypoparathroidism is an unusual disease; however, it provides an elegant model for studying problems of calcium balance.

Adult↗

[Primary hyperparathyroidism (osteitis fibrosa cystica generalisata Recklinghausen)].

The case of a 67-year-old man presenting with parathyroid gland adenoma associated with multiple organ manifestations of a rare normocalcemic form of primary hyperparathyroidism is described. The authors draw attention to the importance of correlating clinical and laboratory examinations with histological, radiological and scintigraphic findings for the establishment of diagnosis. They emphasize the role of two-phase 99mTC-MIBI (methoxyisobutyl-isonitril) scintigraphy not only for diagnostic purposes but also for exact localization of the adenoma by means of a gamma probe during surgery. They also point out the possibility of accompanying pathological conditions, in this case, the development of a tumor described as "brown tumor". This was shown by angiography as a hypervascularized and strongly stained expanding lesion. The authors conclude that the early surgical removal of this parathyroid gland adenoma was important for the patient's successful recovery.

Aged↗

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.

Bone Resorption↗

Cytokine accumulation in osteitis fibrosa of renal osteodystrophy.

Bone marrow fibrosis occurs in association with a number of pathological states. Despite the extensive fibrosis that sometimes characterizes renal osteodystrophy, little is known about the factors that contribute to marrow accumulation of fibrous tissue. Because circulating cytokines are elevated in uremia, possibly in response to elevated parathyroid hormone levels, we have examined bone biopsies from 21 patients with end-stage renal disease and secondary hyperparathyroidism. Bone sections were stained with antibodies to human interleukin-1alpha (IL-1alpha), IL-6, IL-11, tumor necrosis factor-alpha (TNF-alpha) and transforming growth factor-beta (TGF-beta) using an undecalcified plastic embedding method. Intense staining for IL-1alpha, IL-6, TNF-alpha and TGF-beta was evident within the fibrotic tissue of the bone marrow while minimal IL-11 was detected. The extent of cytokine deposition corresponded to the severity of fibrosis, suggesting their possible involvement in the local regulation of the fibrotic response. Because immunoreactive TGF-beta and IL-6 were also detected in osteoblasts and osteocytes, we conclude that selective cytokine accumulation may have a role in modulating bone and marrow cell function in parathyroid-mediated uremic bone disease.

Adult↗

[Von Recklinghausen osteitis fibrosa cystica. A clinical case].

In Recklinghausen's disease the skeleton lesions are often the first signal of the pathology. The main clinical manifestations are represented by bony lesions which appear as multicystic lesions with loss of the hard lamina and skull malformation and asymmetry. In this disease there is a relevant osteoclastic activity which prevails over the osteoblastic one associated to the fibrous substitution of the marrow, sometimes producing micro or macro cysts. The typical alteration consists of an increase along the endosteal and trabecular surfaces in the number of osteoclasts which can be found in small reabsorption gaps. This is the cause of a cortical and trabecular reduction which can appear as interrupted. Histologic lesions consist in the replacement of bone tissue with fibrous and osteoid tissue. These bony lesions are not characteristic of the disease but to be distinguished from other pathologies such as for example Paget's disease and other forms of fibrous dysplasia (Gardner's syndrome, Leontiasis ossea). A case personally observed is described: a women, 29 years old, suffering from Recklinghausen's disease with face and skull asymmetry, condyles and glenoid cavity deformation, abnormal face reduction. Observing the planigraphy on the right side of the temporomandibular articulation, flattened glenoid cavity and condyles with irregular outlines can be noticed, aplastic coronoid cuts, altered jaw. The patient was submitted to surgery for dental extraction followed by a biopsy which showed some regressive alterations on cellular level of the bony structure.

Adult↗