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

714 cases of Fallot's tetralogy have been encountered over the last thirty years. A closed heart operation was undertaken in 472 patients. The mean age at operation was 5,1 years. In 87% of cases, a Blalock-Taussig anastomosis was carried out. The operative mortality was of the order of 3%. The operative risk was almost three times as large before the age of two years as after. The patients benefit by a demonstrable improvement of function after this operation, but it does not protect them from the complications of the condition (cervica abscess, Osler's disease, cerebro-vascular accidents) or from death. Open heart surgery was carried out in 403 patients. 161 as a primary procedure, and 242 after an initial palliative operation. The mean age at operation was 13.3 years. The operative mortality was 15%. The functional improvement was striking, and the patients appeared to be protected from the usual sequelae of the condition. On the other hand they cannot always be considered as cured: 16 died some time after the operation; 37% had pulmonary incompetence; 15% had a residual BSD; 8% had a block of both bundles, and 3% had a definitive atrio-ventricular block.
Arch Mal Coeur Vaiss 1977 Sep
PMID:[Long-term results of palliative operations and complete cure of tetralogy of Fallot]. 7 17

The authors report sixteen consecutive cases of erythromelalgia, an infrequent disease in which local heat, redness, and pain develop in the hands and/or feet in recurrent attacks. The disease was essential in nine patients; in the remaining seven, the cause was a myeloproliferative syndrome (polycythemia vera in 3 cases and thrombocythemia in 1 case) or a drug (bromocriptine, nicardipine, and nifedipine, one case each). Acetylsalicylic acid was effective in only six of the nine essential cases. Intravascular platelet activation and aggregation with plugging of the arterioles has been suggested as the mechanism of erythromelalgia in patients with myeloproliferative disorders. Other, as yet unelucidated pathophysiologic events underlie the juvenile-onset forms, which usually fail to respond to acetylsalicylic acid.
Rev Rhum Mal Osteoartic 1992 Apr
PMID:[Erythromelalgia in adults. Apropos of 16 cases]. 149 72

Thrombotic events are frequent in polycythemia vera (PV) and in essential thrombocythemia (ET). The frequency of thrombotic complications at presentation of PV and ET is nearly 50%. The spectrum of thrombotic complications is broad: thrombosis of arteries, veins and microvessels have been reported. Venous thrombosis can involve all territories but PV and TE are the commonest underlying etiology for Budd-Chiari Syndrome and splanchnic veins thrombosis. Endogenous erythroid-colony formation may be seen in up to 78% of patients thought to have Budd Chiari Syndrome and in 48% of splanchnic veins thrombosis. Major arterial thrombotic complications occur in 20%, especially in the extremities and in cerebral circulation. Microcirculatory disturbances are common in ET, occurring in 29% at presentation and 27% during follow up. In the extremities, erythromelalgia, a characteristic syndrome of red and congested extremities with raised temperature and painful burning sensations, is noticed in 30 to 50% of TE. Other microcirculatory manifestations like acrocyanosis, blue toes, digital gangrene can occur. All of these manifestations are highly sensitive to aspirin. Cerebral microcirculatory symptoms occur in about one-third of patients: migraine, transient visual symptoms like scotomata, blurred vision are characterized by a sudden onset, a short duration and a sequential course. Three kinds of leg ulcers have been described: leg ulceration as a consequence of microcirculatory thrombosis, exceptionally, pyoderma gangrenosum, and leg ulcers attributed to side effects of hydroxyurea. Microcirculatory leg ulcers are the most common: they are painful, inflammatory and sometimes, necrotic. They heal with treatment of SMP. Hydroxyurea-induced leg ulcers are painful, fibrous and multiple in 60%. Cessation of hydroxyurea typically leads to wound healing. The Polycythemia Vera Study Group (PVSG) established diagnostic criteria for PV and TE. Because SMP can have incompletely expressed disease, other authors have proposed determination of serum erythropoietin, examination of bone marrow histology, and spontaneous endogenous colony assays for diagnosis of PV or TE. The individual thrombotic risk depends on elevated hematocrit for PV, age (> 60) and prior thrombosis for PV and TE. Congenital and acquired (antiphospholipid syndrome) thrombotic states probably increase the risk of thrombosis.
J Mal Vasc 2005 Feb
PMID:[When should a myeloproliferative syndrome be suggested in vascular medicine?]. 1592 69