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Biomedical subjects

B Mazur

Publications and source records attributed to B Mazur.

82 records · Page 5Linked to original sources
PubMedDate not supplied

Characterization of zona glomerulosa function in patients with classic and non-classic forms of congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency.

The function of the adrenal zona glomerulosa was studied in 18 patients with 11-hydroxylase deficiency confirmed by elevated plasma levels of 11-deoxycortisol. Patients were divided into two groups. Group I (4 males, 7 females; aged 1.2-2.8 yrs) had symptoms at birth or shortly after (classic form), and Group II (4 males, 3 females; aged 7.3-20.1 yrs) had their first clinical manifestation during childhood (non-classic form). To study zona glomerulosa function, patients were given dexamethasone p.o. 2 mg/m2/day x6 days, thus suppressing the zona fasciculata. Six hours after the last dose of dexamethasone, the zona glomerulosa was stimulated by i.v. administration of furosemide 1.0 mg/kg as a single dose. Blood was drawn 2 h later. In the untreated state, all patients had striking elevation of ACTH (Group I: 1,070 +/- 380 pg/ml; Group II: 764 +/- 180 pg/ml), 11-deoxycortisol (Group I: 63,000 +/- 22,000 ng/dl; Group II: 17,200 +/- 5,200 ng/dl) and deoxycorticosterone (Group I: 1,100 +/- 67 ng/dl; Group II: 499 +/- 27 ng%) while plasma renin activity (< 0.5 ng/ml/h in both groups) and aldosterone (Group I: 3.0 +/- 1.8 ng/dl; Group II: 2.3 +/- 1.8 ng/dl) were markedly suppressed. After the administration of furosemide 4 patients in Group I were unable to increase aldosterone (2.8 +/- 0.9 ng/dl) secretion in spite of marked elevation of plasma renin activity (28 +/- 7 ng/ml/h), suggesting an impairment of 11-hydroxylase in the zona glomerulosa.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMedDate not supplied

Growth hormone secretion and effects of growth hormone therapy on growth velocity and weight gain in children with Prader-Willi syndrome.

Obesity, short stature, decreased growth rate and delayed skeletal maturation are common features of children with Prader-Willi syndrome (PWS). In contrast to PWS, children with simple exogenous obesity have normal or increased growth rate and normal or advanced skeletal maturation. Decreased growth hormone (GH) secretion evaluated by pharmacological or physiological testing associated with increased plasma insulin-like growth factor (IGF-I) and GH-binding protein (GH-BP) levels are also characteristic of simple obesity. In order to understand whether the suboptimal GH secretion in PWS is an artifact of the obesity, we studied 33 obese and 11 non-obese PWS children, aged 2-16 years.GH secretion was evaluated with three pharmacological stimuli (insulin, clonidine and L-dopa) and by spontaneous 24-hour GH secretion. Skeletal maturation was delayed in 70% whereas plasma IGF-I and GH-BP were either low or normal. Forty subjects, including ten non-obese children, had GH deficiency by standard testing (failure to respond to two pharmacological stimuli), and all but one had blunted spontaneous 24-h GH secretion. No significant correlation between body mass index (wt/ht2) and spontaneous 24-h GH secretion (r = 0.145), p > 0.06) or GH-BP levels (r = 0.19, p > 0.07) was found. Thirty documented GH deficient children have completed at least two years of GH therapy. With treatment the overall mean height SD and weight SD changed from -2.2 to -0.8 and from 3.5 to 2.4 respectively (p < 0.0001). No patient has developed diabetes mellitus. In conclusion, growth velocity, skeletal maturation, GH secretion and GH dependent proteins in PWS resemble GH deficiency more than simple obesity. Our ongoing study suggests that GH deficiency in PWS is not an artifact of obesity. Although it is unlikely that GH deficiency is the only cause of decreased growth velocity and increased adiposity in PWS, it is a common feature and significant contributory factor. Long term observation will be required until achievement of adult height to determine whether GH therapy actually improves final height.

Adolescent↗
PubMedDate not supplied

Feeding habits and their physiological determinants. Relationships between consumer's preference for sweetness of drinks and certain physiological parameters.

Investigations determining the preferred saccharose concentration in drinks (CP(S)), gustatory threshold values, blood glucose level, basal metabolism rate and body weight index (im) were carried out with 83 cadets, aged 21--23 years. CP(S) was directly proportional to the gustatory threshold for sweet tastee, blood glucose level and basal metabolism and the correlation found in all three cases was statistically significant (p less than 0.01). The accuracy of CP(S) estimation by cadets was strictly related to their threshold difference for sweet tast depending also on smoking.

Adult↗
PubMedDate not supplied

[Ullrich-Feichtiger syndrome in a 3-year-old boy].

A case of a 3 year old boy with Ullrich-Feichtiger syndrome is presented, because of the rarity of this syndrome. Ullrich-Feichtiger syndrome diagnosis was based on the following clinical signs and symptoms: a) micrognathia with several teeth developmental abnormalities; b) polydactyly (six toes); c) varied genital malformations; d) multiple ocular abnormalities. Cause of these multiple malformations remains unclear.

Abnormalities, Multiple↗
PubMedDate not supplied

[Normal values of peak expiratory flow in children from an environment with high degree of atmospheric air pollution].

Peak expiratory flow (PEF) has been measured with Vitalograph (in liters per minute) in 2,512 school-children aged between 7 and 15 years in Upper Silesian Industrial Region. Five hundred eighty one children from Zarki near Czestochowa served as a control group. The results have been analysed statistically. Determined PEF values for children from the Upper Silesian Industrial Region are considered as a biological reference values for assessment of PEF in both health and disease. PEF values calculated for children from Zarki were higher than those in the examined group, except the value for a 14-year old children.

Adolescent↗
PubMedDate not supplied

[Usefulness of forced expiratory flow rate measurements in diagnosis of selected lung diseases in children].

The study group was composed of 2512 healthy children (age range 7-15 years) randomly selected from schools of the Upper Silesian Industrial District. In each child 5 PEF measurements were carried out using the Vitalograph meter. A statistical analysis of the results was carried out. The results seen in tables and centile charts can be used as normal values for children in given age groups and sex. PEF is useful in clinical diagnosis of different respiratory diseases.

Adolescent↗