Cardiac manifestations in osteogenesis imperfecta. A case report and therapeutic implications

Osteogenesis imperfecta is an hereditary defect in the synthesis of collagen fibres. The disease can cause cardiac defects, such as aortic and mitral incompetence. Surgical repair of these defects is complicated by tissue friability and bleeding problems. We describe a case where an incompetent aortic valve was successfully replaced by a mechanical prosthesis.     

Case report: radical management of an adolescent with amelogenesis imperfecta

Amelogenesis imperfecta is a rare developmental abnormality of enamel, the main clinical problems of which are extensive loss of tooth tissue, poor aesthetics and tooth sensitivity. Management often involves complex and long-term treatment but is usually successful if patients are well motivated. This case report outlines the management of a teenager with amelogenesis imperfecta (hypocalcified type) who had requested a dental clearance at an early age.     

Osteogenesis imperfecta tarda--case report

The case of type IA of osteogenesis imperfecta tarda was described. Following results were presented: anamnesy, physical, ophthalmic and ORL examinations, laboratory findings, radiological and densitometric data. Clinical characteristic and mentioned above examinations allowed to take the definitive diagnosis. Family data showed features of this disease in the next generations and should be a base for diagnostic evaluation in whole family. Confirmation of diagnosis ought to be the important information concerning future life style, work etc. in the next generations.     

Osteogenesis imperfecta and coronary artery surgery. A case report

A new case of Osteogenesis Imperfecta (OI) suffering ischemic heart disease is reported. The patient was successfully operated on in our Institution and the bibliographic search showed only another case of such an association of diseases successfully treated by surgery. This patient proves that coronary artery surgery procedures are possible when OI complicates the cardiac ischemic syndrome.     

Metastatic oligodendroglioma. Case report

We report a case of remote metastasis of oligodendroglioma. Similar cases are reported in the literature for malignant cerebral tumors. Our case seems rather different because of low grade histology. Potential malignancy was evidenced only by further investigations with labeling indexes.     

Oncotic aneurysm. Case report

The author describes a case of intracerebral and subarachnoid hemorrhage associated with an intracranial aneurysm caused by a metastatic tumor. The aneurysm formation is explained as being result of tumor cells invading the vessel wall while still preserving the arterial circulation.     

Kenny-Caffey syndrome. Case report

We investigated whether survival was related to recent childbirth or parity in a cohort of 540 women diagnosed with breast cancer before the age of 45 years who were followed for up to 14 years. Women who had given birth within 2 years before their diagnosis of breast cancer were at increased risk of dying, compared with nulliparous women, with an adjusted relative risk of 3.1 (95% confidence interval = 1.8-5.4). There was a moderate association of parity with mortality, with an adjusted relative risk of 1.8 (95% confidence interval = 1.2-2.9) for women with three or more births, compared with nulliparous women.     

HELLP syndrome. Case report

Genitourinary sarcomas are very rare entities. This paper contributes one case report of an undifferentiated pelvic fibrosarcoma in a 53-year old patient. It also includes some observations on the clinical development, complementary studies used, difficulties for the pathoanatomical diagnosis and treatment issues.     

Current trends in endodontic treatment: report of a national survey

The authors surveyed 360 general dentists and 291 endodontists to obtain information on routine, nonemergency endodontic treatments adapted to clinical practice. Frequent practices and recent advances in treatment modalities-including instrumentation, obturation, intracanal preparations, medications and restorations-are highlighted.     

Benign symmetrical lipomatosis. Case report

We present a patient who had benign symmetrical lipomatosis. He was treated surgically (6 operations), and the results were satisfactory. we discuss various factors in this disease and suggest the possibility of a nonsurgical treatment being developed in the future.     

Primary leptomeningeal astrocytoma. Case report

Gliomas very rarely arise from the leptomeninges. They can be both solitary and diffuse, and histological examination reveals mostly astrocytic tumors. The authors report a case (the 12th reported in the literature) of a solitary primary glioma of the leptomeninges in a 79-year-old man who presented with repeated seizures. A magnetic resonance image revealed an ill-defined enhancing lesion in the cerebral meninges. Autopsy examination showed a poorly demarcated astrocytoma in the sylvian fissure infiltrating the adjacent subarachnoid space. The literature concerning primary leptomeningeal glioma is reviewed.     

Disseminated pulmonary ossification. Case report

Metaplastic pulmonary ossification is usually described as dendriform or nodular in patients with chronic inflammatory lung disease or long-standing pulmonary edema. We present a case of dendriform pulmonary ossification found accidentally at autopsy in a 66-year old man. In addition, some of the theories relating to the development of this rare phenomenon are discussed.     

Primary splenic pregnancy. Case report

A patient with a primary splenic pregnancy is described.     

Optic nerve ganglioglioma. Case report

Gangliogliomas of the optic nerve are extremely rare. The case is reported of a 38-year-old man who presented with a visual field deficit and was discovered to have an optic nerve ganglioglioma. The possible embryological origins of this neoplasm, its histological and immunohistochemical features, and its appearance on magnetic resonance imaging are examined. The prognoses of optic nerve glioma and of gangliogliomas occurring elsewhere in the nervous system are compared.