Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary leptomeningeal lymphoma (PLML) is a rare disease. The most common presentation is symptoms of increased intracranial pressure. Confusion, dysarthria, hearing loss, paraparesis and lumbosacral spinal root symptoms have also been reported. Chemotherapy and radiotherapy have been tried, but its prognosis is usually poor. We experienced a case of PLML with a relatively benign course in an 18-year-old girl. Initial diagnosis was made as idiopathic intracranial hypertension. Lumbosacral shunt was done with good response for 3 years. When headache recurred, she was reevaluated and was correctly diagnosed as PLML.
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PMID:Primary leptomeningeal lymphoma with long-term survival: a case report. 1102 96

Sneddon's syndrome is a rare disease with strong gender prevalence of females. This syndrome is characterized by livedo racemosa and cerebrovascular lesions. Since no specific test is available, the clinical differentiation from other disorders with similar symptomatology may raise difficulties. The cerebral involvement includes strokes with cases of more than one ischaemic event having been reported. Associations with convulsions, heart valve disease, systemic hypertension, and renal impairment have been described. We report the case of a 5-year-old boy who was anaesthesized for dental surgery. Due to the fact that 50% of Sneddon's syndrome patients develop mental retardation, even minor procedures require general anaesthesia. A review of the literature is added and specific anaesthesiological aspects of the perioperative care of Sneddon's syndrome are discussed.
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PMID:Anaesthesiological considerations in patients with Sneddon's syndrome. 1111 4

Cutaneous polyarteritis nodosa is a form of polyarteritis nodosa. It is a rare disease in children and is characterized by its benign and chronic course. There is no evidence of hypertension or organ dysfunction. The aim of this study was to present a case of cutaneous polyarteritis nodosa and to review the literature. We describe the case of a 3-year-old male child with multiple, red, painful, edematous nodules of the extremities and trunk. Dermatologic findings were accompanied by fever and arthritis. Laboratory findings showed an acute phase response associated with raised antistreptolysin titer. Diagnosis was based on biopsy of the affected skin lesion in which necrotizing arteritis of the lower dermis with neutrophilic and eosinophilic infiltrates was found. The patient responded well to corticosteroid therapy. The evaluation of children with cutaneous polyarteritis nodosa should include laboratory studies to detect streptococcal infection.
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PMID:[Childhood cutaneous polyarteritis nodosa]. 1133 82

Little is known on the epidemiology of Cushing's disease (CD) as relevant data on such a rare disease can only be obtained from large-scale studies. We addressed this topic analyzing the data obtained in the Italian multicenter study which comprised nearly 300 patients with CD. The number of newly diagnosed patients with CD increased markedly in the second decade of the study (from 7.4 +/- 0.71 pts/year prior to 1987 to 26.4 +/- 4.12 after 1987) probably reflecting the heightened awareness of the disease and the increased availability of diagnostic tools. Urinary free cortisol (UFC) levels were significantly higher in men than in women and were inversely correlated with the time interval between appearance of symptoms and diagnosis. Recognition of CD among patients presenting with common diseases such as obesity, diabetes and hypertension requires highly sensitive screening tests (e.g. UFC, midnight cortisol in saliva, overnight dexamethasone suppression test) which however may yield false positive results. In doubt, second line testing using dex-CRH or desmopressin may distinguish between CD and pseudo Cushing. The different prevalence of CD and ectopic ACTH secretion (ES) undermines the diagnostic accuracy of tests used for the differential diagnosis of ACTH-dependent Cushing's syndrome (i.e. CRH, high dose dexamethasone, IPSS). Tests aimed at identifying ES rather than CD are needed to overcome this bias. Transsphenoidal surgery was the preferred choice of treatment for patients with CD, resulting in remission in 70% operated patients with a 15% relapse rate over 10 years follow-up. Definition of remission after surgery and parametres predictive of relapse, however, vary according to studies and long-term follow-up is required to establish their validity. Most clinical manifestations of hypercortisolism disappeared after remission although some long-lasting effects on the cardiovascular system had been observed. Finally, according to recent reports, mortality rates for patients cured of CD appear comparable to those of the general population.
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PMID:Epidemiology and follow-up of Cushing's disease. 1135 89

Takayasu's arteritis is a non-specific form of vasculitis involving the aorta, its main branches and pulmonary arteries. It is a rare disease in our country, contrasting with the high prevalence in Southeast Asia, Africa and South America. We discuss the course of the disease in our patient who was a young women who developed Takayasu's arteritis associated with autoimmune thyroiditis and malabsorption syndrome due to celiac sprue. Long-term immunosuppressive therapy contributed to stabilizing the associated diseases, but did not stop the progression of the vascular lesions. The main adverse outcome in our patient was the development of severe renovascular hypertension. Bilateral renal artery stenosis was treated by angioplasty with stent implantation. Based on literature reports, the association of Takayasu's disease with multiple autoimmune disorders is a rare event. However, it would appear that the arteritis was the limiting disease for prognosis in our patient.
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PMID:[Takayasu's disease associated with autoimmune thyroiditis and celiac disease. Clinical course and limitations of treatment]. 1143 24

Retroperitoneal fibrosis has been described as a rare occurrence during the course of inflammatory bowel disease, mainly Crohn's disease. This is the third report on retroperitoneal fibrosis occurring during the course of ulcerative colitis. A 62-year-old male patient with a 5-year history of ulcerative colitis developed stenosis of the left ureter due to retroperitoneal fibrosis. Treatment consisted in surgically releasing the ureter from the mass and steroids. During a 2.5-year follow-up, renal function was stable and ulcerative colitis in remission. Important aspects of this case are the moderate course of ulcerative colitis, ultrasound confirmation of normal kidney structure before manifestation of fibrosis, hypertension diagnosed four years before retroperitoneal fibrosis, a non-functioning kidney at diagnosis, and reduction of retroperitoneal mass after steroid treatment. Retroperitoneal fibrosis, although a rare disease entity should be considered when a patient with ulcerative colitis develops otherwise unexplained renal insufficiency.
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PMID:Retroperitoneal fibrosis during the course of ulcerative colitis. A simple coincidence? 1181 49

Primary lung hypertension (PLH) is a rare disease of unknown etiology seen largely in young persons. PLH shows rapid progression with lethal outcome 3-5 years after registration of the first symptoms. Early diagnosis of the disease is difficult. A case described in the article illustrates real difficulties in PLH diagnosis and treatment. The patient several times lost consciousness for a short time at insignificant exercise. This phenomenon has not been described in the literature so far.
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PMID:[A case of primary pulmonary hypertension]. 1185 26

Hemolytic-uremic syndrome (HUS) is a rare disease but may be fatal in the absence of appropriate treatment. It is the first cause of acute renal failure (ARF) in children and is less frequently observed in adults. We report the case of a 50 years old woman who was first admitted in the surgery department for intestinal occlusion. Haematological and renal disorders were not recognised initially. Specific treatment with plasma exchanges was beneficial. This case was idiopathic but in 50% of HUS in adults, an aetiology can be found and sometimes treated: drugs, pregnancy, systemic diseases, hypertension or cancer. Although still debated, treatment is based on fresh frozen plasma infusion, associated or not to plasma exchanges. Some other therapies can be added: steroids, polyvalent immunoglobulin infusion, vincristin or splenectomy. In all cases, blood pressure control must be obtained, especially with ACE inhibitors. Response to treatment is often excellent, but chronic renal failure persists in about 25% of cases in adults. Recurrences are rare and occur mainly in patients with an hereditary factor H deficiency. The hemolytic-uremic syndrome should be diagnosed and treated immediately.
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PMID:[Hemolytic and uremic syndrome in the adult]. 1212 22

Superior mesenteric artery (SMA) syndrome is a rare disease in which the third portion of the duodenum is compressed by SMA. There are many causes leading to the SMA syndrome, however it's extremely rare that aortic aneurysm causes a SMA syndrome. We report a case of a successfully treated SMA syndrome due to an abdominal aortic aneurysm in a renal transplant recipient. The patient was a 52-yr-old woman with a thin stature (weight 40 kg, height 164 cm). She received a renal transplant 8 yr before, and had hypertension and abdominal aortic aneurysm. Her SMA syndrome developed in a prolonged supine position for the accidental rib fractures and was diagnosed by clinical and radiological findings. After a surgical correction (resection of an aneurysm and aortobiiliac bypass with an inverted Y graft), her symptoms relieved without deterioration of the graft function.
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PMID:Superior mesenteric artery syndrome due to an aortic aneurysm in a renal transplant recipient. 1217 55

A right adrenal tumor was discovered incidentally in an ultrasonographic exam in a 45-year-old man. He was referred to our hospital for further examination and treatment of the adrenal tumor. The hormonal levels in his blood and urine were normal and hypertension was not observed. An ultrasonograph, computerized tomographic scan and magnetic resonance imaging showed a right adrenal tumor 7 cm in size. An adrenal hemangioma was most suspected, but a malignant tumor could not be excluded due to its size. Accordingly, a right adrenalectomy was performed. The histological diagnosis of adrenal hematoma was made. Adrenal hematoma is a rare disease in adults. It is very difficult to distinguish a large adrenal hematoma from a malignant tumor, the final diagnosis has to be made by histological study of the resected specimen.
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PMID:[A case of adrenal hematoma]. 1222 86


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