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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Spontaneous spinal epidural hematoma (SSEH) is a rare disease entity; its causative factors and the factors determining the outcome are still controversial. We reviewed our clinical experiences and analyzed the various factors related to the outcome for SSEH. We investigated 14 patients (11 men and 3 women) who underwent hematoma removal for SSEH from April 1998 to August 2004. We reviewed age, gender, hypertension, anticoagulant use and the preoperative neurological status using the Japanese Orthopaedics Association score by examining medical records, operative records, pathology reports, and radiographies, retrospectively. We were checking for factors such as the degree of cord compression owing to hematoma and the extent and location of the hematoma. Most patients included in the study were in their twenties or fifties. Four hematoma were located in the cervical region (29%), three were cervicothoracic (21%), four were thoracic (29%) and three were in the lumbar (21%) region and also 12 were located at the dorsal aspect of the spinal cord. In all cases, the neurological outcome improved after the surgical operation. There was a statistically significant difference between the incomplete and complete neurological injury for the preoperative status (P<0.05). The neurological outcome was good in those cases that had their hematoma removed within 24 h (P<0.05). The patients with incomplete neurological injury who had a surgical operation performed within 12 h had an excellent surgical outcome (P<0.01). Spontaneous spinal epidural hematoma was favorably treated by the means of a surgical operation. The favorable factors for SSEH operations were incomplete neurological injury at the time of the preoperative status and the short operative time interval.
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PMID:Surgical management of spontaneous spinal epidural hematoma. 1675 10

Cushing's syndrome caused by adrenocorticotropic hormone (ACTH)-independent macronodular adrenal hyperplasia (AIMAH) is an extremely rare disease, which shows bilateral macronodular adrenal hypertrophy and autonomous cortisol production. We herein report a case of AIMAH treated successfully by minimally invasive simultaneous bilateral laparoscopic adrenalectomy. A 73-year-old woman with hypertension, diabetes mellitus, and osteoporosis was referred to our hospital because of an incidentally found huge bilateral adrenal mass. An abdominal computed tomography scan showed large bilateral adrenal glands with multiple nodules. A diagnosis of AIMAH was made and a simultaneous bilateral laparoscopic adrenalectomy was thus performed. The total operation time was 310 min and blood loss was 70 g. Both glands were hypertrophic (right 5 x 3 cm, 48.5 g and left 4 x 2 cm, 39.2 g) and consisted of multiple golden yellow macronodules. The postoperative course was uneventful. A simultaneous bilateral adrenalectomy for AIMAH performed by an experienced surgical team is therefore considered to be a safe and minimally invasive procedure.
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PMID:Simultaneous bilateral laparoscopic adrenalectomy for adrenocorticotropic hormone-independent macronodular adrenal hyerplasia: report of a case. 1679 2

Primary systemic amyloidosis is a rare disease with protean manifestations. Presence of nephrotic syndrome in the absence of diabetes and hypertension, cardiomyopathy in the absence of ischemia, restrictive cardiac defect, demyelinating polyneuropathy, or unexplained hepatomegaly should alert the physician to the possibility of amyloidosis. Initial steps in the diagnostic evaluation of patients with suspected amyloidosis include serum and urine immunoelectrophoresis and immunofixation studies. Demonstration of amyloid material on tissue biopsy (e.g., subcutaneous fat) is required for diagnosis. Availability of effective treatments has improved the outlook of patients with primary systemic amyloidosis. Early diagnosis is critical to optimizing the chances of effective therapy.
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PMID:Primary systemic amyloidosis. Early diagnosis and therapy can improve survival rates and quality of life. 1691 52

Pulmonary arterial hypertension is a life-threatening, rare disease characterised by vasoconstriction and vascular remodelling of pulmonary artery vessels. Pulmonary arterial hypertension can occur without an obvious cause or can be secondary. Until several years ago, therapeutic approaches were represented mainly by 'conventional therapy' (anticoagulants, calcium channel blockers, diuretics and digoxin, and oxygen therapy). But recently 'specific therapies' (i.e., therapies targeting specific pathogenic pathways) have become available; these are therapies represented by prostacyclin and its derivatives, endothelin receptor antagonists or phosphodiesterase-5 inhibitors. Sildenafil citrate is a phosphodiesterase-5 inhibitor and is the second oral pharmacological agent recently approved for the treatment of pulmonary arterial hypertension. Sildenafil has demonstrated short- and long-term clinical efficacy in the treatment of various forms of pulmonary arterial hypertension, either alone or in combination with other agents, but its safety profile needs further assessment.
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PMID:Sildenafil for pulmonary arterial hypertension: when blue turns into white. 1692 6

The prevalence of malignant pheochromocytoma is about 10%, and is somewhat higher for paraganglioma. A problem for clinical follow-up is that patients with "benign" histopathologic findings may develop metastatic disease. At the first international symposium on pheochromocytoma in Bethesda (2005) experts from different disciplines and patients shared their experiences, and the present knowledge of this rare disease was updated. The discussion related to future strategies for better clinical/histopathologic diagnosis and understanding of different geno- and phenotypes. Curative surgery can only seldom be performed because of multiple metastases. The main therapeutic goal is therefore often tumor reduction and control of hypertension. To date the best adjunct to surgery is radionuclide therapy using 131I-MIBG, but the background information for optimal treatment is still incomplete. Certain patients may benefit from 131I-MIBG combined with radiotherapy via somatostatin receptors expressed by the tumor, or the combination with chemotherapy. The need for future multicenter studies was emphasized. In experimental models the work on enhanced expression of amine transporters critical for radiotherapy is continued. Ongoing microarray studies will reveal novel intracellular pathways of importance for proliferation/cell cycle control, which can be inhibited by pharmacologic tools.
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PMID:Malignant pheochromocytoma: state of the field with future projections. 1710 14

Pulmonary hypertension is a rare disease with a poor prognosis. It was first described in the late 19th century as a clinical-pathological syndrome characterised by obstruction of the small pulmonary arteries and right ventricular hypertrophy in patients presenting with severe dyspnoea and cyanosis. After the development of right heart catheterisation in the second half of the 20th century, it was found that many diseases could cause pulmonary hypertension, which is now recognised to be high blood pressure in the arteries that supply the lungs. In the 1960s, an epidemic of pulmonary hypertension caused by appetite suppressants initiated a systematic collection of information on pulmonary hypertension, leading to the first international classification of pulmonary hypertension. Increased understanding of the pathogenesis of the various forms of pulmonary hypertension has led to novel treatments and holds promise for the future.
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PMID:Diagnosis and management of pulmonary hypertension over the past 100 years. 1722 44

Pulmonary artery hypertension is a rare disease with significant morbidity and mortality. Initial and serial noninvasive assessment of these patients can be accomplished with transthoracic echocardiography and/or cardiac magnetic resonance imaging. These complementary techniques provide the structural and functional information required to care for patients with pulmonary artery hypertension and are discussed in this review.
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PMID:Noninvasive cardiac imaging in pulmonary hypertension. 1730 96

Intractable fever in cancer patients is problematic and the causes of this fever can be diverse. Paroxysmal persistent hyperthermia after sudden mental change or neurologic deficit can develop via autonomic dysregulation without infection or any other causes of fever. Paroxysmal hyperthermic autonomic dysregulation is a rare disease entity. It manifests as a form of paroxysmal hypertension, fever, tachycardia, tachypnea, pupillary dilation, agitation and extensor posturing after traumatic brain injury, hydrocephalus, brain hemorrhage or brain neoplasm. We recently experienced a case of paroxysmal hyperthermia following intracerebral hemorrhage along with brain neoplasm. Extensive fever workups failed to show an infectious or inflammatory source and/or hormonal abnormality. Empirical treatments with antibiotics, antipyretics, morphine, steroid and antiepileptic agents were also ineffective. However, Propranolol, a lipophilic beta-blocker, successfully controlled the fever and stabilized the patient. Fever in cancer patients is a common phenomenon, but a central origin should be considered when the fever is intractable. Propranolol is one of the most effective drugs for treating paroxysmal hyperthermia that is due to autonomic dysregulation.
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PMID:Paroxysmal autonomic dysregulation with fever that was controlled by propranolol in a brain neoplasm patient. 1742 48

Idiopathic pulmonary arterial hypertension (IPAH) is a rare disease of unknown etiology. The exact pathogenesis of pulmonary arterial hypertension is still not well known. In the past decades, many protein molecules have been found to be involved in the development of IPAH. With proteomic techniques, profiling of human plasma proteome becomes more feasible in searching for disease-related markers. In present study, we showed the protein expression profiles of the serum of IPAH and healthy controls after depleting a few high-abundant proteins in serum. Thirteen spots had changed significantly in IPAH compared with healthy controls and were identified by LC-MS/MS. Alpha-1-antitrypsin and vitronectin were down-regulated in IPAH and may be valuable candidates for further explorations of their roles in the development of IPAH.
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PMID:Proteomic analysis of the serum in patients with idiopathic pulmonary arterial hypertension. 1744 95

Pulmonary arterial hypertension (PAH), a rare disease involving the pulmonary vascular circuit, is defined as an elevation in pulmonary arterial pressures and is characterized by symptoms of dyspnea, chest pain, and syncope. If left untreated, the disease carries a high mortality rate, with the most common cause of death being decompensated right heart failure. Over the past 5 years, there have been significant advances in this field in regards to understanding the pathogenesis, diagnosis, and classification of PAH. The availability of newer drugs has resulted in a radical change in the management of this disease with significant improvement in both quality of life and mortality. Ongoing research promises to lead to a more comprehensive understanding of the genetics, etiology, and pathogenesis of pulmonary arterial hypertension, which may ultimately translate into more effective therapeutic options.
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PMID:Pulmonary arterial hypertension: evaluation and management. 1745


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