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1/93. Type I osteogenesis imperfecta: diagnostic difficulties.

    A 65-year-old woman presented with vertebral fractures of the lumbar spine and a history of pathological fractures following minor trauma, which had occurred before the onset of menopause. Her past medical history was significant for intermittent low back pain since childhood, which was attributed to thoracolumbar scoliosis. A diagnosis of unclassifiable osteoporosis was made until invasive diagnostic procedures suggested a mild form of type I osteogenesis imperfecta (OI). In unclear or atypical perimenopausal osteoporosis and diagnosis of OI should be considered. ( info)

2/93. Insufficiency fracture of the femoral neck after intramedullary nailing.

    We report a patient with insufficiency fracture that occurred after intramedullary nailing for a subtrochanteric fracture. Intramedullary nailing is speculated to have increased the stress in the already osteoporotic subcapital region. It therefore should be recognized as a causative factor in insufficiency fracture of the femoral neck. Careful follow-up is needed for patients with this condition. ( info)

3/93. Intrathecal infusion of bupivacaine with or without buprenorphine relieved intractable pain in three patients with vertebral compression fractures caused by osteoporosis.

    BACKGROUND AND OBJECTIVES: At present, there is no reliable method of relieving "refractory" pain in patients with compression fractures of the vertebral bodies caused by osteoporosis. We explored the possibility of relieving this type of pain by intrathecal (i.t.) infusion of bupivacaine with or without buprenorphine. methods: An 18-g nylon i.t. catheter was inserted via a lumbar interspace with its tip positioned at the level of the fractured vertebra from which the maximal pain originated. bupivacaine (2.375-5.0 mg/mL) with (n = 1) or without (n = 2) buprenorphine (0.015 mg/mL) was infused through the i.t. catheter from an external electronic pump. The infusion began in the operating room at a basic rate of 0.1-0.2 mL/h, with optional bolus doses (0.1 mL, 1-4 times/h) via patient controlled analgesia. The daily dose of i.t. bupivacaine was adjusted to provide satisfactory pain relief [visual analogue scores (VAS) = 0-2 on a scale of 0-10]. RESULTS: Satisfactory pain relief was obtained with daily doses of i.t. bupivacaine ranging from 10 to 70 (mean approximately 25) mg and buprenorphine from 0.02 to 0.2 (mean = 0.15) mg. The duration of i.t. treatment was 37, 387, and 407 days, respectively. Two patients terminated the i.t. treatment when it was no longer needed. Treatment was discontinued in the third patient because of death caused by irreversible heart failure. The 2 surviving patients were still free of pain 1,074 and 1,476 days after termination of the i.t. treatment. No severe complications occurred. CONCLUSIONS: Continuous intrathecal infusion of bupivacaine, with or without buprenorphine, appeared to be an effective method for the long-term treatment (months to > 1 year) of "refractory" pain from vertebral compression fractures, in this small group of patients. ( info)

4/93. A case of sternal insufficiency fracture.

    We report a case of insufficiency fracture of the sternum in a 70-year-old female patient with a review of the literature. She complained of sudden onset chest pain and aggravating dyspnea. She has been managed with corticosteroid due to chronic obstructive pulmonary disease for 15 years. diagnosis of sternal insufficiency fracture presented with thoracic kyphosis was made on the basis of absence of trauma history, radiologic findings of lateral chest radiograph, bone scintigraphy and chest computed tomography. Thoracic kyphosis and osteoporosis secondary to menopause, corticosteroid therapy and limited mobility due to chronic obstructive pulmonary disease were considered as predisposing factors of the sternal insufficiency fracture in this patient. ( info)

5/93. Opioids for chronic nonmalignant pain. Choosing suitable candidates for long-term therapy.

    Opioid maintenance analgesia for chronic nonmalignant pain can be successful in selected cases, but it is not a panacea for all pain, and management of patients using opioids can be an arduous process. A consistent and principle-based approach is recommended. Passion and chauvinism exist on both sides of the controversy and should be discouraged. ( info)

6/93. Cardiac cause of hypoxaemia in a kyphoscoliotic patient.

    This report presents the case of a 73 yr-old female in whom kyphoscoliosis, osteoporotic vertebra compression fractures and sternal injury resulted in severe respiratory failure and hypoxaemia. Pulmonary function testing showed moderate restrictive pattern and rare mismatches were found on lung ventilation/perfusion scanning. Transoesophageal echocardiography with contrast studies showed abnormal anatomic mediastinal interactions which led to right-to-left interatrial shunt, through patent foremen ovale. First-intention treatment, because of orthopaedic and respiratory surgical restraints, was to close the shunt using transcatheter devices. Follow-up after 6 months demonstrated that these interauricular umbrella devices corrected arterial hypoxaemia. True right-to-left interatrial shunts can be found in kyphoscoliotic patients, as a result of thoracic deformation, and can be safely treated with percutaneous trancatheter closure. ( info)

7/93. Successful therapy of myelodysplastic syndrome with menatetrenone, a vitamin K2 analog.

    Although vitamin K2 is an inducer of the in vitro differentiation of myeloid leukemic cell lines, its clinical efficacy in the treatment of myelodysplastic syndrome (MDS) is unclear. We administered a vitamin K2 analog, menatetrenone, at 45 mg daily to an 80-year-old woman with MDS (refractory anemia) heavily dependent on red-cell transfusions. The patient's pancytopenia gradually improved, and she became transfusion-independent after 14 months. pancytopenia recurred when menatetrenone was discontinued but recovered again with readministration. Administration of menatetrenone at a dose effective in improving osteoporosis may also be useful in restoring hematopoiesis in MDS patients, possibly by way of inducing differentiation. ( info)

8/93. osteoporosis: a factor in mortality following cardiac surgery.

    osteoporosis may be a significant factor in unfavorable results following open cardiac surgery. This brief case report describes the fatal outcome of mitral valve replacement in an elderly woman with congestive heart failure complicated by severe osteoporosis. Profound collapse of the thoracic spine resulted in pulmonary insufficiency. The discussion includes substantiation that this phenomenon is not unusual but rather can be expected to occur in postmenopausal women who are inactive, not receiving calcium and vitamin d supplements, and are suffering from congestive heart failure for which loop diuretics are administered. Bone mineral density testing is essential to determine and track the efficacy of treatment. ( info)

9/93. osteoporosis. An overview of the National osteoporosis Foundation clinical practice guide.

    During the past decade, numerous organizations and associations have published recommendations for the prevention and treatment of osteoporosis. For the primary care physician, the most applicable of these--due to its reliance on clinical trial data and its scope--is the clinical guide published by the National osteoporosis Foundation. The guide addresses risk assessment, bone mineral density testing, diagnosis, nutritional supplementation, and pharmacologic therapy, including consideration of the newer agents used to slow or manage osteoporosis progression. Reflecting one of the key deficiencies in the clinical trial data, the guide applies predominantly to a patient population of postmenopausal white females. The refined design of new osteoporosis studies will in time allow for recommendations that apply to a more diverse patient population. ( info)

10/93. Differential diagnosis of Hb EE and Hb E-beta(0)-thalassemia by protein and dna analyses.

    dna analysis was used to confirm the Hb EE genotype and to differentiate from the possible genotype of Hb E-beta(0)-thalassemia in two Malay patients. The first patient was a 13-year-old Malay girl, whose parents were available for family studies. The second patient was a 69-year-old Malay woman with no living family members. The presence of Hb E in both propositi was confirmed by: (1) its characteristic electrophoretic mobilities on alkaline/acid gels; (2) its chromatographic properties on anion/cation exchangers, and (3) its mildly insoluble properties. However, differential diagnosis of Hb EE and Hb E-beta(0)-thalassemia was challenging in these two cases. In the former, this was because of the possible interactions of the parents' phenotypes; i.e., the mother had a similar phenotype. In the latter, it was due to the lack of any living family members for family studies. In this communication, we present the protein and dna analyses, including data on the use of the restriction enzyme Mnl I, for the definitive diagnosis of the Hb EE genotype in the propositi of these two Malay families. ( info)
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