Biomedical subjects
Publications and source records attributed to G Gács.
A patient with hyperimmunoglobulin E (Job's) syndrome is presented. The authors review the clinical and immunological characteristics of the disease and sum up the different explanations for the pathogenesis of the syndrome.
In 78 patients with idiopathic growth hormone (GH) deficiency the effect of the fathers' educational level on the age and the extent of growth retardation at diagnosis was studied. There was a tendency for an increase of the age and the degree of growth retardation with the decrease of the fathers' completed grades. The occurrence of height SD scores less than -4.5 was conversely related to the number of grades completed by the father (chi 2 = 19.2 p less than 0.001). Eighteen of the 70 patients treated with growth hormone discontinued treatment after 0.3 to 6 years. Compliance was closely related to the grades completed by the father (chi 2 = 24.7 p less than 0.001). Six out of 7 patients with a height SDS less than -4.5 at diagnosis and with a father of low level of education (less than 8 grades) became non compliant. It is concluded that the degree of growth retardation at diagnosis and compliance at treatment in GH deficiency is related to the educational level of the father.
To define the optimum blood levels of 17-hydroxyprogesterone, the hormonal effects of glucocorticoid treatment were studied during the neonatal period and infancy in 20 patients with 21-hydroxylase deficiency. Repeated daily profiles of blood spot 17-hydroxyprogesterone were used to monitor therapy and these data were related to serum concentrations of testosterone. A wide fluctuation of blood 17-hydroxyprogesterone levels was observed in patients with a mean daily value higher than 150 nmol/l indicating poor control. Serum testosterone levels decreased into normal range in female patients with blood-spot 17-hydroxy-progesterone levels between 50 and 150 nmol/l. Daily profiles with mean values less than 50 nmol/l showed low magnitude of circadian variation suggesting overtreatment. We conclude that the daily profile of blood-spot 17-hydroxyprogesterone is a practical alternative in hormonal monitoring of infants treated for congenital adrenal hyperplasia.
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Twenty four children with hypopituitarism were treated with growth hormone from 6-15 years of age until cessation of growth. The height deficit decreased from -4.2SD to -2.2SD. Final height was above the 3rd percentile in half of the patients. There was no difference in final height between patients with and without additional gonadotropin deficiency. Spontaneous puberty started late but at a normal bone age and its course was normal. In girls with gonadotropin deficiency low dose oestrogen given at unchanged growth hormone doses did not accelerate growth. Final height was closely correlated with the degree of growth retardation at the beginning of treatment (r = 0.73, p less than 0.001). In 17 of the 79 patients treated with growth hormone compliance was bad. The main cause of bad compliance was the low educational level of the parents. It is concluded that final height in growth hormone deficiency can be increased with earlier diagnosis and improved compliance.
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The effect of growth hormone treatment on the plasma lipoproteins was studied in 13 growth hormone deficient children. The pretreatment total cholesterol level was moderately (p less than 0.05) higher in growth hormone deficiency and it decreased significantly under the effect of growth hormone administration. This decrease was due to the reduction of HDL-cholesterol in the first week and to the decrease of LDL-cholesterol after one month of treatment. The level of LDL-cholesterol returned to normal at one month. The plasma triglyceride level increased significantly under the effect of growth hormone in the first week and returned to normal after one month. This change was due to the increase of VLDL-triglyceride level. There was no difference in the cholesterol and triglyceride concentrations of growth hormone deficient children with normal or subnormal plasma thyroxine concentrations.
We investigated the value of serum levels of adrenal steroids (dehydroepiandrosterone sulphate, testosterone, 17-hydroxyprogesterone, cortisol) in the identification in peripubertal females with late-onset congenital adrenal hyperplasia owing to 21-hydroxylase deficiency. Among 68 females (age 3-18 years) with virilization in childhood, peripubertally or postpubertally, we selected 21 girls for an ACTH test by measurement of basal blood-spot or serum 17-hydroxyprogesterone (17-OHP) levels. Eight of 21 patients had supranormal post-ACTH serum 17-OHP concentration (57-153 nmol/l) with low normal cortisol concentration. All of them had supranormal basal and post-ACTH 17-OHP to cortisol ratios. These data show a relatively high incidence (about 12%) of mild 21-hydroxylase deficiency among prepubertal and adolescent girls with virilization. It is concluded that the first step in the investigation of peripubertally virilized girls should be the determination of serum 17-OHP and cortisol. Patients with basal morning 17-OHP concentration and 17-OHP to cortisol ratio above reference range should be given an ACTH test.
The possible aetiological factors were studied in 73 children with idiopathic growth hormone deficiency. The following perinatal abnormalities were found to be more frequent than expected: breech presentation (32.9%) and asphyxia (15.0%). Parallel with an increased application of caesarean section there was a significant reduction in the incidence of breech birth among the hypopituitary patients. On the basis of TSH levels measured during TRH tests, most of the patients with breech delivery had a hypothalamic damage, while at vertex position the pituitary damage was more frequent. Of the two pairs of siblings with hypopituitarism both members of the first had breech birth and of the second high birth weight. It is concluded that perinatal abnormalities are important aetiological factors in hypopituitarism and that the occurrence of this disease can be reduced with improved obstetrical practice and perinatal care.
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The daily excretion of C-peptide in the urine was measured in 105 healthy infants and children from birth to 14 years of age. For technical reasons no studies were performed from 1-3 years of age. The excretion of C-peptide showed a close positive correlation with age and weight. The relationship with weight was already apparent in the 1st days of life. The C-peptide/weight and the C-peptide/creatinine ratios were constant throughout most of childhood with the exception of the age range of 1 month-1 year when the C-peptide/creatinine was significantly higher. In obese children the C-peptide/weight and C-peptide/creatinine ratios were similar to those found in children with normal weight. In growth hormone deficiency these ratios were low and increased during the 1st week of growth hormone therapy. It is concluded that urinary C-peptide is a reliable indicator of integrated insulin production and gives new information about insulin secretion in various conditions.
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The present paper summarizes our experiences with intraoperative fluorescein angiography in 50 external carotid artery-internal carotid artery bypass operations. This technique has provided precise localization of the ischemic lesion and aided in selecting the sites and direction of the anastomoses to be performed, as well as in checking the patency of the anastomoses and the area supplied by them. A comparison of X-ray angiography with fluorescein angiography indicates the reliability of the latter in the estimation of the area supplied by the anastomosis.
The diagnostic problems of acute, unilateral exophthalmus are discussed in connection with a case of spontaneous intraorbital haematoma by the rupture of a venous saccular aneurysm.