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Biomedical subjects

V Plavsić

Publications and source records attributed to V Plavsić.

35 records · Page 2Linked to original sources

Coincidence of pseudohypoaldosteronism with gluten-enteropathy.

This is a 21-month-old boy with pseudohypoaldosteronism (PHA) in coincidence with celiac disease. The diagnosis of PHA was made on the basis of hyponatremia, hyperkalemia and large urinary salt losses, as well as high renin activity and aldosterone levels and increased urinary plasma aldosterone. Whereas mineralocorticoid therapy was ineffective, salt therapy has proven successful. The patient's HLA type was found to be characteristic of gluten-enteropathy (A1, B8, DR3). The combination of PHA and celiac disease has not yet been described and is probably a coincidence. However, it is suggested that other PHA patients be typed in order to investigate the segregation between HLA type, PHA and celiac disease.

Aldosterone↗

Infarction of FSH-secreting pituitary adenoma.

A patient with an FSH-producing adenoma is reported. Two hours after LRH administration (100 microgram iv) the clinical signs of tumour infarction developed. The patient followed a severe clinical course. FSH levels normalised but further deterioration of pituitary function was documented. Worsening of the vision without improvement after surgical resection of the adenoma was observed. The role of LRH testing in adenoma infarction is discussed.

Adenoma↗

Plasma renin activity and plasma aldosterone concentration in infants with various potassium intakes.

Plasma renin activity (PRA) and aldosterone concentration (PA) were measured in 20 healthy infants from 1 to 12 months of age during normal (3.03 mmol/kg/d) and high (5.20 mmol/kg/d) potassium intake. PRA did not change between regimes. PA rose significantly from 618 pg/ml during normal to 1136 pg/ml during high potassium intake. It is concluded that the potassium intake should be taken into consideration when studying plasma aldosterone in infancy.

Aldosterone↗

Plasma renin activity and plasma aldosterone concentration in infants with various sodium intakes.

Plasma renin activity (PRA) and plasma aldosterone concentration (PA) were measured in 20 healthy infants 2-12 months of age, after taking a commercial modified cow's milk formula with 8.2 mmol/l sodium for at least five days, and after at least five days on a home-made, undiluted acidified cow's milk formula with 12.6 mmol/l sodium. The mean sodium intakes were 1.20 and 1.87 mmol/kg of body weight per day respectively. The arithmetic means for the corresponding PRA were 6.16 and 1.08 ng/ml . h (difference not significant) and the corresponding arithmetic means for PA were 603 and 355 pg/ml, the difference being highly significant (P less than 0.01). It is concluded that one has to consider the sodium intake in the milk formula when studying plasma aldosterone in infancy.

Aldosterone↗

Preliminary investigations of the metabolism and pharmacological activity of beta-hydroxytryptamines in mammals.

beta-Hydroxytryptamine and beta-hydroxy-5-hydroxytryptamine were incubated with rat liver slices and oxidative deamination was established as the main route of metabolism: in both instances the corresponding indole-3-glycollic acids and indole-3-ethane diols were the major metabolites. However, the rates of deamination of beta-hydroxylated tryptamines, as measured manometrically, were found to be much slower than those of tryptamines nonhydroxylated in the side chain. The pharmacological activities of beta-hydroxylated tryptamines were tested in guinea-pigs on resistance of respiratory pathways, spontaneous respiration, electrocardiogram, blood pressure and isolated ileum, using tryptamine and 5-HT as reference substances. The effects of tryptamines hydroxylated in the side chain were in general similar to those of corresponding tryptamines but of much lower intensities; only in increasing the blood pressure was beta-hydroxytryptamine as active as tryptamine. The different reactions of these two groups of substances in the presence of some antagonists indicate that the receptors are probably not the same.

Animals↗

[Primary adrenocortical micronodular dysplasia].

Two girls (11 and 13 years old) with Cushing's syndrome due to primary adrenocortical micronodular dysplasia (PAMD) are presented. High plasma cortisol concentrations, elevated urinary free cortisol and 17-ketogenic steroids excretion, in addition to low or normal plasma adrenocorticotropic hormone (ACTH) levels pointed towards independent adrenal cortisol hypersecretion. In both girls bilateral adrenalectomy was performed, followed by replacement therapy with glucocorticoids and mineralocorticoids. Pathohistological findings of otherwise enlarged adrenal glands, showed characteristic small nodules measuring 1-2 mm, composed of cells resembling those of zona fasciculata, with abundant, clear cytoplasm. Our younger patient fulfilled the criteria of "Carney complex", because beside PAMD she has had the lentigines.

Adolescent↗

[Low-renin hypertension and inherited mineralocorticoid diseases].

Liddle's syndrome, apparent mineralocorticoid excess (AME) and glucocorticoid remediable aldosteronism (GRA) are inherited diseases characterized by hypertension and low plasma renin activity. Constitutive activation of distal renal epithelial sodium channel (Liddle's syndrome), defect in 11 beta-hydroxysteroid dehydrogenase activity (AME) and unequal crossing over, fusing regulatory sequences of 11 beta-hydroxylase gene to coding sequences of aldosterone synthase gene and forming a new chimeric gene (GRA), cause apparent or real mineralocorticoid excess. This diseases are often being unrecognized and classified as essential hypertension, especially in patients with normal serum potassium level. Family history of hypertension and characteristic serum and urine++ steroid profile direct us to diagnosis, and genetic analysis will confirm it.

Humans↗

[Importance of the CRH (corticotropin releasing hormone) test in the differential diagnosis of Cushing's syndrome].

In the group of 13 patients with Cushing's syndrome (CS) CRH test was performed by sampling the blood from peripheral vein and in eight patients also after inferior petrosal sinus catheterization (IPSC) to resolve the disease etiology. In the group of patients with Cushing's disease (CD, n = 11), which was proven by surgery and adenoma immunohistochemistry, 10/11 had in CRH test the significant increase of cortisol and ACTH in the peripheral blood. Among two patients with ectopic ACTH syndrome one had the significant increase of both hormones in CRH test. After IPSC the ratio of ACTH in the petrosal sinus and in the peripheral vein was significant in 4/8 patients before, and in 6/8 after CRH administration. The intersinus gradient was significant in 3/8 patients before, and in 4/8 after CRH test. According to our results we can conclude that the determination of ACTH in the blood from peripheral veins after CRH administration is a very sensitive method for differential diagnosis of CS, while the results after IPSC were less sensitive in our conditions than those described in the literature.

ACTH Syndrome, Ectopic↗

[Detection of heterozygosity in congenital adrenal hyperplasia caused by 21-hydroxylase deficiency in the general population].

The detection of heterozygotes among the family members of the patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21-OH) can be done by means of two methods: typing of HLA as the first and measuring of 17-hydroxyprogesterone (17-OHP) concentrations before and 60 or 360 minutes after ACTH stimulation as the second one. In general population, however, heterozygotes can be revealed only by means of the latter method. Nowadays, the shorter, 60-minute ACTH test predominates, because of being fast and simple test to perform. Thus, with the certain degree of reliability healthy homozygotes can be distinguished from heterozygotes for CAH due to 21-OH deficiency. This study presents the results obtained by measuring concentrations of 17-OHP before and 60 minutes after ACTH stimulation in 69 examinees ranged in four groups: patients with classic type of CAH due to 21-OH deficiency (7 examinees), heterozygotes revealed by means of HLA typing (parents, brothers and sisters of patients--29 examinees), healthy homozygotes proved by means of HLA typing (brothers and sisters of patients--5 examinees) and finally examinees chosen from general population of unknown genotype (28 examinees). The results obtained by measuring concentrations of 17-OHP before and 60 minutes after ACTH stimulation are presented in form of a nomogram and represent the referred data for our population. By using a computer programme and the estimation method it is estimated that 15% of heterozygotes could be by mistake declared as healthy homozygotes (false negative results) and 15% of healthy homozygotes, respectively (false positive results). It should be included in interpretation of the results.(ABSTRACT TRUNCATED AT 250 WORDS)

17-alpha-Hydroxyprogesterone↗

[Prenatal diagnosis of congenital adrenal hyperplasia due to 21-hydroxylase enzyme deficiency].

Prenatal diagnosis of the "classical" forms of congenital adrenal hyperplasia (CAH) which is a result of 21-hydroxylase (21-OH) deficiency either complete, with salt-wasting or incomplete without salt wasting, is performed in two ways: by measuring concentration of 17-hydroxyprogesterone (17-OHP) and androstendione (delta 4) in amniotic fluid and by HLA typing of fetal cells from amniotic fluid. Having ones own normal values is the basic condition for the safe prenatal diagnosis of CAH 21-OH deficiency by measuring steroid concentration in amniotic fluid. Normal concentrations of 17-OHP in amniotic fluid achieved by amniocentesis in 85 pregnant women from 16-23 gestation week have been measured, as well as concentrations of delta 4 in 66 pregnant women in the same period of gestation. It has been proved that there are no differences between the concentrations of delta 4 in amniotic fluid regarding the sex. As far as 17-OHP is concerned, the same was confirmed earlier. The results of 9 prenatal diagnosis in 8 families, having already one child with "classical" form of CAH with salt-wasting, have been presented. It was achieved by combination of two methods: by measuring concentration of 17-OHP and delta 4 in amniotic fluid and HLA typing of fetal cells from amniotic fluid. In 8 fetuses at risk the birth of healthy children was correctly predicted, which was confirmed after the birth in three cases by HLA typing and measuring concentration of 17-OHP and delta 4 and from the blood of newborn babies.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenal Hyperplasia, Congenital↗

[Parameters of thyroid gland function in acute myocardial infarct].

In 31 patients with acute myocardial infarction triiodothyronine (T3), free triiodothyronine (FT3), thyroxine (T4), free thyroxine (FT4), thyrotropin (TSH) and TBG were measured and T3-test was performed on the 1st and the 10th day of hospitalization. The 1st day values for T3, FT3 and TBG were significantly lower, and T4 and TSH were significantly higher than in the control group. The same differences were noted on the 10th day for T3, FT3 i T4. TBG was significantly higher than the 1st day. TSH was lower and it was not significantly different from control values. These results are compatible with clinical observations described in severe nonthyroidal illnesses.

Aged↗

[X-linked adrenoleukodystrophy--2 case reports].

Cases of a ten-year-old boy with childhood cerebral adrenoleukodystrophy (ALD) and a 22-year-old youngster with adrenomyeloneuropathy (AMN) are reported. ALD is an inherited, X-linked perixisomal disorder associated with the accumulation of very long chain fatty acids (VLCFA). Neurological symptoms occur due to progressive demyelination and destruction of cerebral white matter and primary adrenal insufficiency. The boy with ALD manifested neurological signs (impaired spatial orientation, visual disturbances, poor handwriting, seizures). Latent primary adrenal insufficiency was established, and successfully treated by gluco- and mineralocorticoids. Lorenzo's oil (mixture of glyceroltrioleate:glyceroltrierucate 4:1) treatment significantly reduced elevated concentrations of VLCFA, but in spite of that, neurological symptoms progressed and the boy died a year after the initial clinical presentation of the disease. The boy with AMN revealed primary adrenal insufficiency at the age of 15 years. AMN was suspected when hair and eyebrows loss occurred and the diagnosis was established due to elevated VLCFA levels in the serum at the age of 22 years. On examination no neurologic signs of the disease could be detected. Adrenal insufficiency is well controlled by gluco- and mineralocorticoids. In addition to the previously described two women who were symptomatic heterozygotes we now also report on two patients with ALD and AMN. The patients reported are the first four with this peroxisomal disorder described in Croatia so far. Probably a great number of such patients remains unrecognised. Therefore, it is necessary to measure the serum VLCFA levels in males with primary adrenal insufficiency, and in those with signs of progressive central demyelination and destruction of cerebral white matter accompanied by neurological symptoms of unknown etiology.

Adrenoleukodystrophy↗