Cases reported "Bone Resorption"

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1/8. Painful trigeminal neuropathy caused by severe mandibular resorption and nerve compression in a patient with systemic sclerosis: case report and literature review.

    Systemic sclerosis is a multi-system disorder characterized by abundant fibrosis of the skin, blood vessels, and visceral organs. Although resorption of the mandible has been commonly observed and reported, we found no report of resorption leading to a painful neuropathy of the inferior alveolar nerve. We report a case of a patient with systemic sclerosis, severe resorption of the angles, and inferior alveolar border of the mandible, resulting in a compression neuropathy of the inferior alveolar nerve. Diagnostic tests, medical treatment, and surgical treatment are discussed, and the relevant literature is reviewed.
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2/8. A case report of chronic neutropenia: clinical and ultrastructural findings.

    This case report of severe periodontitis concerns a young male patient with chronic idiopathic neutropenia seen between 1981 and 1983 by the School of Dental medicine of Geneva and from 1984 to 1988 by the Eastman Dental Hospital of london. The patient was maintained by intermittent systemic antibiotics, subgingival chlorhexidine irrigation, conventional debridement, brushing, and wire-mesh and composite splinting of loose teeth. After leaving school, at the patient's express wish, the extreme downhill pattern of the periodontitis resulted in removal of affected teeth and overdenture construction. The extracted teeth and associated soft tissues were examined for relevant plaque and host response features. The intact morphotypes associated with the advancing front of the lesion were invariably Gram-negative or positive coccoid cells, or less commonly short rods, as in all documented forms of periodotitis. connective tissue destruction was associated with a leucocyte accumulation comprising mainly polymorphonuclear neutrophils (PMN) and plasma cells. Both were observed degenerated more deeply than in routine adult periodontitis, including PMN lysosome loss and lymphocyte maturation within peripheral blood vessels. Only in one instance, one part of the superficial connective tissue of one block contained bacteria. It was concluded that the features of plaque and the lesion suggest a typical first line of defence response as in other forms of periodontitis. From the consideration of the 10 years clinical history of the patient, it is clear that non-surgical management is possible even in extreme cases, without any compromise of the patient's oral or systemic health, and in accord with the patient's wishes.
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3/8. Massive metastatic pulmonary calcification in an infant with aleukemic monocytic leukemia.

    An 18-month-old infant with aleukemic monocytic leukemia and osteoclastic bone resorption demonstrated in the pelvis, vertebral bodies, and ribs developed severe refractory hypercalcemia. Bilateral interstitial pulmonary radiopacities developed rapidly, accompanied by hypoxemia and hypercapnea. Von Kossa staining of an open lung biopsy revealed extensive, finely granular metastatic septal calcifications not apparent on H&E-stained sections. autopsy revealed the massive nature of the pulmonary calcinosis and the presence of additional calcifications in the atrial subendocardium, liver, kidneys, vessels, and skin. Metastatic pulmonary calcification has been infrequently described in infants, and premortem detection of such deposits has been rarely reported. The Von Kossa stain is useful in detecting minute pulmonary calcifications which may radiographically simulate infiltrates of infectious origin.
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4/8. Osseous changes due to a false aneurysm of the proper digital artery: a case report.

    A 24-year-old black man had a laceration and subsequent pseudoaneurysm of the radial digital artery of the small finger. Two months after the injury, the vessel, a lacerated radial digital nerve, and a flexor digitorum profundus were all repaired. A depression was noted in the middle phalanx at surgery. Osseous reconstruction of the defect in the middle phalanx was shown on follow-up x-ray films.
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5/8. Ultrastructural aspects of nerves, bones, and vessels in hereditary sensory neuropathy.

    This study concerns two children, from the same otherwise normal family, presenting progressive spontaneous amputations of the limbs, suggesting peripheral hereditary sensory neuropathy. Samples from the distal nerves and vessels and the distal tibial epiphyses were examined ultrastructurally. The nerves showed signs of degeneration, and a typical bone necrosis, without osteoclasts, was observed in the 2 cm margin of the samples. Simultaneous modifications of the vessels suggest a neurally initiated vascular reflex resulting in bone destruction. Theories that might explain the observations are discussed.
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6/8. Hereditary osteodysplasia with acro-osteolysis. (The hajdu-cheney syndrome).

    A mother and son with acro-osteolysis (hajdu-cheney syndrome) are described. In addition to osteolysis of the distal phalanges, these patients have a generalized osseous dysplasia with osteoporosis, premature loss of teeth, short stature and a distinctive facial appearance. In one of the cases an enlarged sella turcica was associated with no abnormality of endocrine function. A biopsy specimen taken from an area of active osteolysis in a phalanx was studied by light and electron microscopy. There was active replacement of central medullary bone by a fibrous and angiomatous process characterized by the presence of small, thick-walled vessels and an unusual number of interspersed nerve fibers and mast cells. A neurovascular dysfunction with local release of osteolytic mediators may be involved in the pathogenesis of the disorder, but the nature of the osteolytic factor is unknown.
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7/8. Nephropathy of idiopathic multicentric osteolysis.

    Progressive osteolysis with disappearance of the carpal and tarsal bones occurs as a rare syndrome associated with a serious nephropathy. The characteristic skeletal changes have an onset in early childhood and precede renal failure by 10--15 years. The present case, the ninth reported in the world literature, was a 22-year-old, white female who presented with carpal and tarsal swelling at age 2. Severe hypertension and azotemia were present when the patient died of pneumonia and extracellular fluid volume depletion. At necropsy, the kidneys weighed 55 g each. microscopy showed proliferation of vascular intima and medial hypertrophy of renal vessels of all sizes. Tubulointerstitial and glomerular changes are probably secondary to the severe vascular disease. Although similar skeletal lesions can be seen in kindred with autosomal dominant and recessive inheritance, all reported cases of nephropathy including the present one are sporadic.
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8/8. ehlers-danlos syndrome (type VIII).

    ehlers-danlos syndrome (EDS), a group of rare, autosomal dominantly inherited connective tissue dysplasias, characterized mainly by abnormal collagen synthesis, has been shown to exhibit extensive heterogeneity with at least 11 clinical entities differentiated by their clinical, biochemical, and genetic features. Of these, Type VIII EDS is of special interest from a dental viewpoint, due mainly to the resulting fragility of the oral mucosa and blood vessels, and an aggressive type of periodontitis causing severe loss of alveolar bone; all of these clinical manifestations of the entity make it necessary to exercise care during the dental treatment procedures. Here we present a case of EDS Type VIII with typical clinical findings, together with details of the dental treatment modalities, placing emphasis on surgical methods.
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