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

Twenty-nine hydropic infants were born in the Kandang Kerbau Hospital between 1980 and 1985, during which there were 131,658 deliveries, giving an incidence of 1 in 4,540 total births. Twenty-five of these cases were confirmed to be due to homozygous alpha thalassaemia. No case of fetal hydrops due to Rh isoimmunization was detected. The mean age of the mothers was 28.86 +/- 4.05 years (+/- SD). Eight patients had delivered 1 hydropic baby previously while 1 had a history of 2 babies with hydrops fetalis; 92% of the patients had been followed antenatally while 8% were first seen when they were admitted in labour; 25% of the patients had anaemia, 52% had polyhydramnios, 20% developed hypertension and 64% had bilateral lower limb oedema. None of the patients had concomitant hypertension, generalized oedema and proteinuria. In 4 cases of recurrent hydrops, serial ultrasound scans were performed from early pregnancy but ultrasonic features of hydrops fetalis were only seen from 27 weeks' gestation. Spontaneous labour occurred in 75% of patients at a mean of 32.3 +/- 3.3 weeks (+/- SD). All delivered vaginally and only 1 patient required abdominal decompression. Four patients required Caesarean section, 2 for failure to progress after induction of labour, 1 for major placenta praevia and the fourth for fetal distress; in the last case, diagnosis of hydrops fetalis was only made after delivery of the baby. All the babies in the series died within one hour of delivery. Homozygous alpha thalassaemia is the commonest cause of hydrops fetalis in Singapore and is an invariably fatal condition. It is associated with an increased incidence of maternal anaemia, polyhydramnios and prematurity.
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PMID:Bart's hydrops fetalis--clinical presentation and management--an analysis of 25 cases. 260 53

Post-transfusion hypertension, convulsion and cerebral haemorrhage is a serious complication that may occur in the thalassaemias. In this study we evaluated the effect of blood transfusion on blood pressure, plasma renin activity (PRA), blood viscosity, and urinary vanillylmandelic acid (VMA) and catecholamines in 11 beta-thalassaemia/haemoglobin E patients. The results showed that after each unit of blood transfusion the blood viscosity was increased and correlated with the increased in haematocrit level. At the same time the PRA level was significantly decreased and tended to return to the normal level in a few days after the transfusion. There was no alteration in the urinary VMA and catecholamine levels. During the study two patients developed hypertension and headache. Their PRA were still lower than the pre-transfusion levels and the blood pressure returned to the normal pre-transfusion levels within 30-90 minutes after the intravenous injection of furosemide.
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PMID:Study of mechanisms of post-transfusion hypertension in thalassaemic patients. 269 89

We describe a 3-year-old Pakistani girl who presented with recurrent urinary infections. She had a nonfunctioning hydronephrotic right kidney and hypertension. At operation a calculus was impacted in the right ureter with dilatation of the pelviocaliceal system. Nephrectomy was performed. Histology revealed end stage pyelonephritis. The calculus consisted of pure xanthine. Further investigations demonstrated low serum uric acid and absent urinary uric acid with increased excretion of xanthine. Eight months after nephrectomy blood pressure had decreased to normal. Her 5-year-old sister, who has beta-thalassemia, also has a low serum uric acid concentration and xanthinuria. The treatment of choice is to increase fluid intake so that the urine xanthine concentration remains below the level at which xanthine crystallizes. This may require adjustment of the urine pH.
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PMID:Hereditary xanthinuria in 2 Pakistani sisters: asymptomatic in one with beta-thalassemia but causing xanthine stone, obstructive uropathy and hypertension in the other. 333 36

Forty-six hydropic infants with homozygous alpha-thalassaemia born during a period of 10 years have been reviewed. The incidence was 1:1550 total births, and accounted for 81% of all non-immune hydrops. The male to female ratio was 1:1.4. There was increased incidence of anaemia, pregnancy induced hypertension, antepartum haemorrhage, malpresentation, prematurity, fetal distress, difficult vaginal delivery, caesarean section, retained placenta, postpartum haemorrhage and congenital abnormalities. Antenatal diagnosis by DNA hybridization with subsequent abortion of the affected fetuses is the best method to decrease maternal morbidity and to reduce the incidence of hydrops fetalis in couples at risk. For those with no previous history, but with early onset hypertension and/or polyhydramnios, sonography is useful in making an earlier diagnosis, and in reducing avoidable morbidity, because DNA analysis can be done before caesarean section and aggressive neonatal management is instituted.
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PMID:Homozygous alpha-thalassaemia: clinical presentation, diagnosis and management. A review of 46 cases. 401 25

Thrombus formation depends on adherence of blood-formed elements to the intimal surface through platelet-vessel surface interaction, platelet release phenomena and aggregation, formation of fibrin, and the enmeshing of blood cells. Arterial thrombi involve platelet aggregation, whereas venous thrombi found in low flow or during stasis have greater proportions of erythrocytes and fibrin. It is not known if or how abnormalities of flow resistance, platelet thrombus formation, or endothelial and dynamic parameters affect the microcirculation, largely due to the difficulty of obtaining comprehensive data from these systems. Increases of fibrinogen observed in many disorders may result in minor changes in blood viscosity without known physiologic consequence, but in most disorders in which thrombosis is observed, the pathophysiologic mechanisms are multifactorial and abnormal blood viscosity is presumed to be a significant but not limiting component. Therapeutic approaches in thrombotic disorders should recognize which elements of the thrombotic triad predominate. In arterial disorders focus should be on platelet activity, and the objectives of venous thrombosis treatment include prevention of morbidity and death from pulmonary embolism, reduction of morbidity resulting from the acute thrombotic episode, and prevention of the postphlebitic syndrome. Pathology, mechanism, and treatment for specific thrombogenic disorders are described. Treatments suggested for hyperviscosity involve giving antibiotics during crises. Also discussed are thalassemia, paroxysomal nocturnal hemoglobinuria, polycythemia, cryoglobulinemia, paraproteinemia, diabetes mellitus, and disseminated intravascular coagulation. Studies have established a relationship between thromboembolic disease and oral contraceptives (OCs). The risk is only increased while the patient is taking OCs but is compounded in women undergoing surgery or who have a disorder which predisposes to venous disease. The risk for myocardial infarction or stroke is significantly increased when OCs are taken over age 35 and when there is hypertension, smoking, type-II hyperlipoproteinemia, and diabetes mellitus. The risk appears to be a function of estrogen dosage, causing a 25% mean increase in calf venous volume and 30% decrease in vein velocity of venous blood compared to controls. Low flow rates may contribute to venous thromboembolism. OCs may alter precisely regulated systems of coagulation and fibrinolysis and recent studies confirm abnormalities in the hemostatic system attributed to OCs. 16% of women taking OCs have a 60% or greater reduction in antithrombin III activity. The multiple effects of OCs often result in low-grade activation of the hemostatic system, potentially lowering the threshold to precipitate thrombus formation and possibly explaining the increased incidence of thromboembolic disease. Heparin appears to reverse many of these problems.
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PMID:Blood viscosity and thrombosis: clinical considerations. 676 12

Case report of a 4-year-old boy with beta-thalassaemia who developed a syndrome of hypertension, convulsions and cerebral haemorrhage after multiple blood transfusions. He made a complete recovery within 25 days.
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PMID:Hypertension, convulsion, and cerebral haemorrhage in thalassaemic patients after multiple blood transfusions. 741 Jan 13

Little is known about the behaviour of central retinal vein occlusion (CRVO) in the young. We studied the course and visual outcome in 25 patients with CRVO aged less than 40 years with special reference to the association with underlying systemic disorders. Eight patients had ischaemic CRVO while 17 had non-ischaemic CRVO. Systemic disorders associated with ischaemic CRVO included end-stage renal disease in 3, and hypercholesterolaemia, beta-thalassaemia and hypertension in 1 each. All patients with ischaemic CRVO had unusually severe disease and a rapid downhill course. Management included panretinal photocoagulation in 7 eyes, trabeculectomy with 5-fluorouracil in 3 eyes and cyclocryopexy in 2 eyes. Despite this 3 eyes became phthisical and the final visual acuity in all eyes ranged from no light perception to 6/60. Three patients with end-stage renal disease died within 15 months of diagnosis of venous occlusion.
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PMID:Ischaemic central retinal vein occlusion in the young. 768 66

Thalassaemia major determines an impaired effort tolerance because of a condition of severe anaemia, progressive left ventricular dysfunction, pulmonary circulation compromise. The aim of our study is to evaluate haemodynamic response to exercise in thalassaemic patients without clinical features of heart failure. We have selected 13 patients affected by thalassaemia major (Thal+; 10-18 years). Each patient was transfused when haemoglobin values were < 9-9.5 g/dl and was treated with desferrioxamine (40 mg/kg sc) when serum ferritin values were > 2,000 ng/ml. Thal+ patients were compared with normal subjects (Thal- 10-16 years). No patient assumed hypotensive therapy, no had familiar history of hypertension. Both groups have undergone an ergometric stress test at the cycloergometer, with increase of 25 W every 2 min, up to the reaching of the maximum age-related heart rate, or up to muscle exhaustion or unbearable dyspnea, followed by a 10 min recovery phase. The following parameters were taken in consideration: systolic (SBP) and diastolic (DBP) blood pressure, heart rate (HR), the product of the heart rate by the systolic blood pressure (DP), at rest, at the maximum common work (MCW), at maximum stress and in the recovery phases. At rest, only DP showed significant differences between the two groups: in Thal+ patients higher than in Thal- (p = 0.045). At the MCW, Thal+ patients had SBP (p = 0.019), DBP (p = 0.01), HR (p = 0.035) and DP (p = 0.003) higher than Thal- patients. At maximum stress only DBP showed significant differences in Thal+ patients (p = 0.019), although Thal+ patients achieved lower levels of workload (p = 0.0001).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Cardiovascular adaptation to the stress test in subjects with Cooley's disease]. 780 70

Four children with beta-thalassemia intermedia ages 7 to 11 years developed a clinical picture characterized by headache, hypertension, convulsion, and cerebral hemorrhage after blood transfusion. Successive transfusions did not result in a similar picture. Factors responsible for this syndrome are discussed.
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PMID:Convulsion after blood transfusion in four beta-thalassemia intermedia patients. 782 50

The biventricular systolic function was evaluated by M mode and Doppler echocardiography in 16 patients with homozygous beta-thalassemia and beta-thalassemia Hb E disease, aged 5-14 yrs (9.9 +/- 2.4 yr). The left ventricular end-diastolic dimension and left ventricular mass were increased in 88 per cent of the patients. Left ventricular fractional shortening and cardiac index were normal in all but one patient. Fifteen patients had evidence of pulmonary artery hypertension manifested by abnormality in the ratio of right ventricular acceleration time to ejection time (AT/ET). There was good correlation between platelets count and AT/ET (r = -0.70, P = 0.002). Thrombocytosis was noted in 4 patients. Our findings indicated that in beta-thalassemic children, right ventricular dysfunction was detected earlier than left ventricular dysfunction and platelets may play a role in the pathogenesis of pulmonary artery hypertension.
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PMID:Cardiac dysfunction in beta-thalassemic children. 796 32


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