Spironolactone responsive familial hypertension. A potentially high prevalence of mineralocorticoid disease in Oman.
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Biomedical subjects
Publications and source records attributed to W J Johnston.
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All patients <13 years of age referred to the open-access paediatric echocardiography clinic at the Sultan Qaboos University Hospital, Muscat, Oman, during the five years from 1994 to 1998 were analysed. Among the 2633 patients studied, 1543 (58.6%) were normal, 845 (32.1%) had congenital heart disease, and 245 (9.3%) had acquired heart disease. The major congenital heart diseases identified were secundum atrial septal defect (22.5%), ventricular septal defect (22.5%), patent ductus arteriosus (15.7%), mitral valve prolapse (10.7%), pulmonary stenosis (9.7%) and atrioventricular septal defect (4.5%). Fifty-eight percent of the congenital heart diseases were identified in the first year of life. Among the acquired heart diseases, rheumatic heart disease (30.2%) and cardiac involvement secondary to haemoglobinopathies (16.7%), dilated cardiomyopathy (16.3%) and hypertrophic cardiomyopathy (12.7%) were significant. Although the presence of specific cardiac symptoms was associated with a high yield of abnormalities, such disorders were also discovered in a significant number of children with isolated cardiac murmur. The referral source did not influence significantly the frequency of heart diseases diagnosed in this study. Open-access echocardiography is important in early detection of heart disease in the paediatric population.
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Friedreich's ataxia (FA) was studied in a large inbred Arab family living near Jeddah, Saudi Arabia, in which DNA linkage studies localised the disease gene to 9q13-q21.1. Five siblings (aged 19-35 years), and their 27 year old cousin, had the typical features of FA, however in two patients, tendon reflexes were retained and were indeed brisk in the lower limbs, 13 and 19 years respectively after onset of symptoms: retention of lower limb tendon reflexes is exceptional in FA. Another 6 deceased individuals from two related families are presumed to have had FA.
A 2-year-old Omani child with hyperimmunoglobulin E syndrome (HIES) had a 5-week illness characterized by fever, heart failure and acute mitral regurgitation. Echocardiographic studies showed the evolution of an apical left ventricular aneurysm during this illness.
Oral vanadate treatment is effective in normalizing blood glucose in both Type I and Type II diabetics. Using Sprague Dawley rats we examined the effectiveness of such treatment in amelioration of hyperglycemia in diabetic pregnancy and its effect on fetal growth in both normal and diabetic pregnant dams. Initiation of vanadate treatment to diabetic and normal pregnant dams increased blood vanadium levels in both groups, but this concentration in the diabetic pregnant group reached approximately twice the value present in the normal group. Despite this high blood vanadium level in the diabetic pregnant dams, oral vanadate treatment was not effective in normalizing blood sugar in this group. Additionally, vanadate treatment was found to be toxic during diabetic pregnancy, causing death to 45% of the test animals. Maternal blood vanadium had a negative effect on fetal development, markedly reducing the number of live fetuses per pregnancy. In summary, oral vanadate treatment is toxic and ineffective during diabetic pregnancies and interferes with fetal growth and development in both normal and diabetic pregnancy.
Eleven normotensive diabetics with noninsulin-dependent diabetes mellitus (NIDDM) (mean age 52.5 +/- SD 8.2 years) and 11 controls (mean age 47.4 +/- SD 8.9 years) had their ambulatory blood pressure and heart rate recorded non-invasively by the Oxford Medilog System in standard hospital conditions. The results were averaged as hourly means of systolic blood pressure (SBP), diastolic blood pressure (DBP), mean arterial blood pressure (MAP), and heart rate (HR) for the 24-h period and similarly for the 'awake' period (14-16 h) and the 'asleep' period (8-10 h). Hourly means for diabetics and controls showed no differences in blood pressure and heart rate over the 24 h. During sleep, control subjects showed a significant drop in SBP (P < 0.001), DBP (P < 0.001), MAP (P < 0.001) and HR (P < 0.001). However, this nocturnal dip in blood pressure could not be demonstrated in the diabetic group. Blood pressure variability was significantly increased in diabetics compared to controls during waking hours (P < 0.01). These results indicate that in noninsulin-dependent diabetics during sleep there is loss of the nocturnal dip of BP seen in normal subjects, and they have increased BP variability. These may be contributing factors to the development of hypertension and the accelerated target organ damage (TOD) seen in diabetes.
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Radiosurgery has been used for many years with great success for the treatment of acoustic neuroma. We present three cases in Louisiana using the E L Fisher system, the results of treatment, and a discussion of acoustic neuroma.
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This article emphasizes the need for increased marketing activities in medicine in general and in graduate medical education in particular. This imperative is discussed, and normative models are developed. The actual application of the marketing concept to the residency program of the Department of Family Medicine at the Ohio State University is reported, and areas requiring future marketing efforts are outlined.
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Although serologic evidence suggests the presence of Q fever in humans and animals in Saudi Arabia and the United Arab Emirates, acute Q fever has not been reported on the Arabian Peninsula. We report the first two cases of acute Q fever in Oman.