Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032463 (polycythemia vera)
 3,374 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Seventeen patients treated for recurrent epistaxis due to Osler's disease in the nose were followed up 6 months - 19 years after treatment. A written questionnaire was answered by 13 of the 14 patients surviving at follow-up. They had undergone either septal dermoplasty, or irradiation with 1200--1400 rad on both sides of the nose, or both kinds of treatment. All patients were satisfied with their treatment. The amount of nose-bleeding was reduced in all but one of the surgically treated patients, and the frequency was reduced in nine of the 13 patients. Nasal crusts, often causing nasal obstruction, were a problem, at least shortly after operation. Pathological vessels were found within the skin graft at secondary operation in one patient, showing that telangiectasia can develop in the graft area and cause recurrences of the epistaxis, sometimes necessitating further operations.
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PMID:Follow-up of patients with hereditary hemorrhagic telangiectasia in the nose, with special reference to septal dermoplasty. 635 5

Vascular disorders of the liver except portal hypertension are rare. They can be visualised via ultrasound, as demonstrated by two cases of cavernous transformation of the portal vein, one case of Osler's disease of the liver, and one case of Budd-Chiari's syndrome. The cavernous transformation and the occlusion of the hepatic veins can be diagnosed by ultrasound, while Osler's disease cannot be differentiated with certainty from other disorders.
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PMID:[Vascular diseases of the liver: cavernous transformation of the portal vein, Osler disease and Budd-Chiari syndrome]. 647 45

Adenosine deaminase (ADA) has been assayed in plasma, erythrocytes, and lymphocytes from 29 patients with haematological and autoimmune diseases. ADA activity was uniformly low in erythrocytes and lymphocytes from patients with non-Hodgkin lymphoma and multiple myeloma (p less than 0.001). High levels of ADA activity was found in plasma, erythrocytes, and lymphocytes from patients with myeloid leukemia (p less than 0.001). ADA was high in plasma but low in erythrocytes and lymphocytes from patients with autoimmune diseases treated with immunosuppressive drugs (p less than 0.05). 4 adults with congenital immunodeficiency showed decreased ADA activity. In the control group of normal blood donors we found a 34-year-old female with low ADA activity in plasma, erythrocytes, and lymphocytes without any immunological abnormalities. This is the 3rd case of a healthy individual deficient for ADA. 1 patient with Osler's disease and high ADA activity in erythrocytes showed the importance of the purine salvage enzyme not only in lymphocytes.
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PMID:Adenosine deaminase activity in plasma and blood cells of patients with haematological and autoimmune diseases. 678 72

Four patients with single or multiple pulmonary arteriovenous aneurysms, including three with Rendu-Osler's disease, were treated by occlusion with metallic spirals of the supplying pedicles during angiography. Results are compared with those of 16 cases treated by the same method and reported in the literature. The technique and its therapeutic indications are discussed based on these 20 cases and a review of documented data concerning 502 other cases with these aneurysms.
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PMID:[Nonsurgical treatment of pulmonary arteriovenous aneurysms]. 687 7

Hepatic hereditary telangiectasia in 2 females was treated with hepatic artery embolization. In one patient both peripheral and central arterial occlusion was performed; the patient died of massive gastro-intestinal bleeding for 2 months later. Autopsy showed extensive regions with necrosis in the right liver lobe. In the next patient only central occlusion of the right hepatic artery was performed and the arterio-venous shunting in the left liver lobe left remaining. After a long reconvalescence period the patient recovered completely. It is advocated to centrally occlude only that hepatic artery supplying the most affected parts of the liver in Osler's disease. Thus extensive necrosis with possible ensuing death may be avoided.
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PMID:Hepatic telangiectasia in Osler's disease treated with arterial embolization. Report of 2 cases. 708 36

We describe a new method for successful management of epistaxis in patients with hereditary hemorrhagic telangiectasias. By so doing, even pulmonary arteriovenous fistulas and gastrointestinal hemorrhages can be successfully managed. During a four year period, 14 patients were treated with an oral contraceptive preparation which combined a reduced dosage of estrogen and progesterone. With this therapy, 11 patients stopped bleeding while 3 responded with significant improvement. We believe that this effect on bleeding by the hormone therapy is a sign of an abnormal increase of specific hormone requirements in patients with Osler's disease.
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PMID:[A new method for managing epistaxis in Osler's disease (author's transl)]. 721 47

A 77-year-old male having multiple small nevi over the lips, face, mucous membrane of mouth, tongue, neck and thorax was admitted to our hospital because of severe anemia. He has been diagnosed as Rendu-Osler-Weber disease (Osler's disease) since his 42 years of age. Although the stools were positive for occult blood, hematologic examination disclosed no abnormalities except for severe hypochromic and microcytic anemia. Gastrointestinal examination showed multiple stigmata in the esophagus, stomach, duodenum, and several tumors in the colon. Histological findings of biopsy specimens obtained from these tumors revealed adenocarcinomas and tubular adenomas. Surgical resection was carried out. Resected specimens showed two tumors (one is in ascending colon appeared to be 2 type carcinoma, 1.9 x 2.3 cm in size, another is in caecum, 3 type, 7.0 x 5.0 cm in size) and three Yamada-II approximately III type polyps. Pathohistologically, the tumor in ascending colon showed moderately differentiated adenocarcinoma, another one in caecum showed mucinous carcinoma, and polyps showed tubular adenomas, respectively. Unfortunately, the anemia of this case was regarded as stemming from Osler's disease, so the precise gastrointestinal examination was not performed for a long time. It should be emphasized to be faithful to the fundamentals of medical diagnosis.
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PMID:[A case of Rendu-Osler-Weber disease associated with simultaneous, multiple advanced cancers in the colon]. 770 55

Hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome, Osler's disease) is an inherited abnormality of the vasculature characterized by abnormal subepithelial vessels. Treatment has included repeated intranasal cautery, intra-arterial embolization and arterial ligation. Historically, the operation of septodermoplasty and the use of systemic/topical oestrogens have been the most effective and lasting treatments, but over time the telangiectasias recur. There is increasing interest in the use of a variety of lasers for intranasal photocoagulation. A report of nine patients suffering from hereditary haemorrhagic telangiectasia who have undergone laser treatment with the Chromos pulsed dye laser is presented. This laser produces light energy at a wavelength of 585 nm and causes localized thermal damage to the blood vessel wall. Initially, no patient saw an improvement in their symptoms, but following an average of approximately three courses of treatment, all have reported a measurable reduction in the number of epistaxes suffered. Although no treatment completely resolves the epistaxis associated with this condition, we have found the pulsed dye laser to be effective at treating telangiectasias within the anterior nasal cavity.
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PMID:Treatment of hereditary haemorrhagic telangiectasia by the pulsed dye laser. 947 72

Cold knife surgery, electrosurgery and laser surgery all offer techniques, instruments, equipment and systems for resecting and destroying mucosal lesions and for hemostasis in the upper aerodigestive tract. When used in the head and neck, argon plasma surgery (APS) offers a new, contact-free, electrosurgical technique in which high frequency current is applied through ionized, and thus electrically conductive, argon(argon plasma) to the tissue undergoing treatment. Especially noteworthy in APS are its advantages for removing a lesion and controlling bleeding: the technique is easy to control, and the depth of the thermal tissue destruction is limited to a maximum of 3 mm even in wide-area application, so that damage to adjacent or submucosal tissues can be avoided. Initial results with APS in the reduction of hyperplastic nasal turbinates, treatment of hereditary hemorrhagic teleangiectasia (Osler's disease) in the nasal mucosa, and in treating progressive juvenile papillomatosis of the larynx have shown clear advantages for APS over other methods used.
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PMID:[Argon plasma surgery (APC) in the upper aerodigestive tract. Initial results]. 973 34

A 45-year-old Japanese woman with splenomegaly and thrombocytopenia was referred to our hospital. The diagnosis of Osler-Weber-Rendu disease (Osler's disease) was made because of spotty telangiectasia on her tongue, recurrent epistaxis since childhood, and a diathesis indicated by her family history. The patient's laboratory examination revealed anemia, thrombocytopenia, and other data consistent with chronic disseminated intravascular coagulation (DIC). Bone marrow examination was normal. Abdominal computed tomography showed marked enlargement of the spleen with deformity and calcified plaque, not homogeneously enhancing. Hypersplenism was not observed. Platelet scintigraphy indicated a remarkable uptake in the spleen. She was diagnosed as having chronic DIC associated with vascular lesions of Osler's disease in the spleen. Splenectomy was performed and the subsequent pathological findings indicated that fragility of the fine vascular architecture of the splenic red pulp might have been responsible for pathogenesis. The large pooling of blood with coagulation was thought to be secondary.
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PMID:Splenomegaly and chronic disseminated intravascular coagulation in Osler-Weber-Rendu disease: a case report. 1107 62


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