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)

Sickle cell disease is the most common inherited disease in the African American population. Multiorgan pathologic features with a predilection for thoracic organs predominate. Acute cardiopulmonary diseases include acute chest syndrome, pneumonia, and left ventricular failure. Cardiomegaly, pleural effusions, pulmonary consolidation, pulmonary edema on chest radiographs, and ground-glass opacities on computed tomographs are characteristic. Chronic changes include sickle cell lung disease with lung fibrosis, pulmonary arterial hypertension, hyperkinetic circulation related to severe anemia, and thoracic skeletal abnormalities; the latter are H-shaped vertebrae, rib infarction, and extramedullary hematopoesis.
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PMID:Thoracic manifestations of sickle cell disease. 955 90

Renal involvement is common in homozygous sickle cell disease (HbSS), including glomerular hypertension and hypertrophy similar to that seen in rodent models of ablative nephrectomy and stage I diabetic nephropathy (DN). The proteinuria in the rodent models is attenuated by angiotensin converting enzyme inhibition (ACEI). Microalbuminuria (MA) is a sensitive marker for renal involvement in DN prior to the development of proteinuria, and is also attenuated with ACEI. Elevated urinary microalbumin/creatinine ratios (U Alb/Cr) >20 mg/g Cr are reported in 39%-43% of adults with HbSS, and studies are ongoing in this age group to assess the effect of attenuated proteinuria by ACEI on long-term renal function. The purpose of this study was to prospectively investigate the prevalence of MA in children with HbSS and determine factors which affect its expression. U Alb/Cr values were measured on spot urine samples in 102 children (aged 2-18 years, mean 9.47+/-4.62, M:F=53:49) by rate nephelometry. Children with prior known proteinuria, hypertension, or fever/pain episode in the last 15 days were excluded. MA was present in 26.5% of all children with HbSS. However, in children between the ages of 10 and 18 years, the prevalence was 46% (similar to the prevalence in adults). There was a strong correlation between patient age and prevalence of MA (P<0.0001) by both univariate and multivariate analysis. However, pain frequency, hospitalization, transfusion program, ferritin levels, and Cr clearance (C(Cr)) did not correlate with prevalence, although C(Cr) (as estimated by Schwartz formula) was elevated in all. We conclude that the prevalence of MA in the 2nd decade of life is similar to that in adults.
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PMID:Prevalence of microalbuminuria in children with sickle cell disease. 974 72

Cerebrovascular disorders are frequently associated with sickle cell disease, mainly in homozygous children. We report the case a 25-old-patient with known sickle cell disease who presented with coma inaugurated by manifestations of intracranial hypertension. CT revealed bilateral thalamic infarcts and angiography confirmed the thrombosis of internal cerebral veins. Treatment included heparin and blood transfusion. Severe cerebral oedema resulted in the lethal outcome three days later.
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PMID:[Sickle cell anemia and internal cerebral vein thrombosis]. 975 Jun 46

Sickle cell disease (homozygotes SS) is known as a risk factor for both ischemic and hemorrhagic stroke, but heterozygotes AS seem to be spared. We carried out a retrospective study to assess the main risk factors and the influence of hemoglobin abnormalities on stroke in Guadeloupe. The percentages of AS, AC, and AA on 295 patients admitted for stroke were compared to the prevalence obtained on 72,000 newborn babies. Ischemic, hemorrhagic stroke and stroke complications represented respectively 83 p. 100, 10 p. 100 and 7 p. 100. Seventy one per 100 of patients had hypertension and 19 p. 100 had an association of diabetes and hypertension. The percentage of heterozygotes AS was significantly lower in the group with ischemic stroke (4 p. 100) in comparison with controls (8.5 p. 100), while AS were more represented in hemorrhagic stroke (16 p. 100). The risk of hemorrhagic stroke was 10 fold higher in AS patients admitted for stroke and the risk of ischemic stroke was reduced by 15 fold. These data suggest that the sickle cell trait could be associated to red cell and/or endothelial specificities which could prevent for ischemic stroke. The influence of AS heterozygote on the occurrence of stroke needs to be examined in a longitudinal, prospective study.
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PMID:[Epidemiology of stroke in Guadeloupe and role of sickle cell trait]. 1042 98

Cerebral internal venous thrombosis are rare and diagnosis is difficult. We report three cases in male adults. Clinical data were headaches, vomiting, dizziness and coma, in relation with an intracranial hypertension, or in a case, cardiocirculatory arrest. Cerebral internal veinous thrombosis was diagnosed by a CT scan and cerebral angiography twenty four hours after the admission in neurosurgical intensive care. CT scan showed hemorrhagic and ischemic lesions of thalami in two cases, diffuse cerebral edema in two patients, early or delayed hydrocephaly in two cases. No patient survived despite intensive treatment including heparinotherapy, ventricular CSF drainage, osmotherapy, dehydration, barbiturate, other antiepileptic drugs and mechanical ventilation. In two cases, general or local illness was found, sickle cell disease or radiotherapy for pineal tumor, and in case 3 clinical signs evoked autoimmune disease, not demonstrated by biological samples.
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PMID:[Cerebral deep vein thrombosis: three cases]. 1048 48

Sickle cell disease (SCD) is predominantly a problem of black community in whom a higher prevalence of hypertension has been reported. Despite these observations, a lower mean arterial blood pressure is observed to be common in homozygous sickle cell anaemia (SCA) patients, especially from outside Africa. In view of the well known diversity in both clinical and haematological presentations of SCD, we have evaluated the effects of sickled haemoglobin on blood pressure distribution in Nigerians with SCA. Arterial blood pressure was measured using mercury sphygmomanometer and the mean arterial pressure calculated in 64 adult Nigerians with SCA and 60 normal individuals. The mean age of SCA group was 22.14 +/- 4.90 years with a range of 18-57 years and in the controls was 27.18 +/- 8.06 years with a range of 18-54 years. The mean values of MAP 72.69 +/- 7.30 mmHg in SCA was significantly lower than 87.25 +/- 9.98 mmHg in the controls p < 0.007. The cause of this observed lower blood pressure in SCA is still speculative. We postulate that the differences in weight between SCA patients and normal individuals may contribute to this phenomenon. However, it is possible that nitric oxide (NO) or endothelial relaxing factor (EDRF) may also contribute to this lower blood pressure and the decrease in peripheral resistance in SCA.
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PMID:Arterial blood pressure in adult Nigerian sickle cell anaemia patients. 1050 68

Sickle cell anemia and the related hemoglobinopathies are associated with a large spectrum of renal abnormalities. The patients have impaired urinary concentrating ability, defects in urinary acidification and potassium excretion, and supranormal proximal tubular function. The latter is manifest by increased secretion of creatinine and by reabsorption of phosphorus and beta(2)-microglobulin. Young patients with sickle cell disease (SCD) have supranormal renal hemodynamics with elevations in both effective renal plasma flow (ERPF) and glomerular filtration rate (GFR). These parameters decrease with age as well as following the administration of prostaglandin inhibitors. Proteinuria, a common finding in adults with sickle cell disease, may progress to the nephrotic syndrome. Proteinuria, hypertension, and increasing anemia predict end-stage renal disease (ESRD). While ESRD can be managed by dialysis and/or renal transplantation, there may be an increased rate of complications in renal transplant recipients with SCD. Hematuria is seen in individuals with all of the SCDs as well as with sickle cell trait. In most cases the etiology of the hematuria turns out to be benign. However, there does appear to be an increased association between SCD and renal medullary carcinoma. Therefore, those SCD patients who present with hematuria should initially undergo a thorough evaluation in order to exclude this aggressive neoplasm. Papillary necrosis may occur due to medullary ischemia and infarction. Erythropoietin levels are usually lower than expected for their degree of anemia and decrease further as renal function deteriorates. An abnormal balance of renal prostaglandins may be responsible for some of the changes in sickle cell nephropathy. Acute renal failure is a component of the acute multiorgan failure syndrome (MOFS). Finally, progression of sickle cell nephropathy to ESRD may be slowed by adequate control of hypertension and proteinuria. However, the prevention of the renal complications of SCD will require a cure for this genetic disorder.
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PMID:Renal abnormalities in sickle cell disease. 1142 1

The differential diagnosis of hematuria with or without proteinuria is extensive, and isolated hematuria is a common problem in children and adolescents. Extensive evaluation is often necessary for the child presenting with macroscopic plus microscopic hematuria including nonglomerular and glomerular etiologies, while children with only isolated microscopic hematuria can generally be followed after baseline evaluation to rule out infection, hypercalciuria, familial hematuria, sickle cell disease, post-streptococcal glomerulonephritis (GN), and structural abnormalities (cysts, stones, obstruction, Wilms tumor). Children with the combination of hematuria and proteinuria require rapid systematic evaluation, generally including renal biopsy, except in cases where post-streptococcal GN can be clearly documented. Post-streptococcal GN occurs 7-21 days after a streptococcal infection, is associated with an acute fall in C3 levels with return to normal by approximately 8 weeks, rarely causes acute renal failure, and in children has a pattern of gradual resolution of hypertension, hematuria, and proteinuria over a course of 6-12 months.
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PMID:Asymptomatic hematuria in childhood: a practical approach to evaluation. 1079 63

Since early recurrence occurs in at least 10% of patients presenting with their first stroke in childhood in the reported series, the search for modifiable risk factors should be a priority. Risk factors for stroke in adults include hypertension, diabetes, and smoking, as well as cardiac disease and sickle cell anemia; asymptomatic cerebrovascular disease and transient ischemic events may predict stroke in this age group. The investigation of a child with a stroke has traditionally focused on finding a single cause rather than looking for risk factors to which the patient may be exposed life long. Approximately half of children presenting with stroke have a known predisposing condition, but some have unexpected pathologies such as primary cerebrovascular disease associated with congenital heart anomalies, or may have modifiable risk factors such as hypertension associated with sickle cell disease. The literature on children presenting with initially unexplained (cryptogenic) stroke suggests that there is a daunting list of possible causes, but since the series have mainly been small, it has been difficult to evaluate the relative importance of the reported associations. This paper reviews the literature on congenital, genetic, and acquired risk factors for stroke in childhood, and includes data from the large series of patients seen at Great Ormond Street Hospital over the past 10 years. The majority have arteriographic abnormalities and there is little evidence for asymptomatic cardiac disease. Genetic predisposition, trauma, infection, and nutritional deficiencies appear to be important, although case-control studies will be required to prove causation. Appropriate screening for modifiable risk factors may lead to prevention of recurrence in some patients. In the long term, an understanding of the multiple etiologies of childhood cerebrovascular disease and ischemic stroke may lead to primary prevention in this age group, and perhaps in adults.
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PMID:Risk factors for arterial ischemic stroke in children. 1083 Jan 96

In a prospective study spanning 12 1/2 years (July 1983 to December 1995), 272 children with nephrotic syndrome seen at the University of Nigeria Teaching Hospital Enugu, Nigeria, were followed up and reviewed at the end of the study period. The demographic, clinical and laboratory features, response to treatment and prognosis were documented. Nephrotic syndrome made up 1.34% of all paediatric admissions. There were 164 males and 108 females giving a male to female ratio of 1.5:1. The ages ranged from 2 to 16 years, with a mean of 7.9 +/- 3.4 years and peak age of 5-7 years. The major clinical features were generalized oedema (100%), hypertension (23%), fever (20%), oliguria (10%) and cough (7%). Haematuria was present in 26%, mean serum albumin was 16 +/- 5, 1 gm/L, serum cholesterol 9.53 +/- 1.6 mmol/L Malaria parasitaemia was present in 38.7% and 9 patients (3.3%) had sickle cell disease (SS). Treatment with diuretics, pooled plasma, prednisolone or cyclophosphamide in various combination achieved 63.9% remission. Mortality was 5.5% being mainly due to chronic renal failure, hypertension and infections. The study calls for more trials in the use of steroids and cyclophosphamide in the treatment of childhood nephrotic syndrome in the tropics.
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PMID:Childhood nephrotic syndrome in Enugu, Nigeria. 1107 Jul 50


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