Search PubMed⌕ Search

Biomedical subjects

L Lisá

Publications and source records attributed to L Lisá.

At least 19 recordsLinked to original sources

[Giant bilateral adrenal myelolipoma associated with congenital adrenal hyperplasia].

BACKGROUND: Myelolipoma is a rare benign tumor formed by mature fat tissue with strata of haematopoiesis. It is mainly asymptomatic, only large myelolipomas manifest themselves by abdominal discomfort, bleeding or by symptoms of oppressing adjacent organs. It is usually localized in the region of the adrenal gland. Myelolipomas are mostly clinically inert, only a small number of them are associated with Cushing's type of endocrine disorders, Conn's syndrome, Addison's disease, etc. PATIENT AND METHODS: The authors present a rare case of a giant bilateral myelolipoma emerging out of the adrenal gland cortex in a congenital adrenal hyperplasia, with steroid 21-hydroxylase deficiency, in a woman with pronounced virilism. The principal diagnostic methods include sonography and computer tomography. The therapy available is either a surgical extirpation in the case of large or growing myelolipomas or observation in the case of small ones (< 5 cm). RESULTS: The condition was resolved by bilateral adrenalectomy with extirpation of both myelolipomas and subsequent hormonal substitution treatment during which there was a gradual regression of virilising symptoms. CONCLUSIONS: The coincidence of myelolipoma and congenital disorder with subsequent overproduction of the adrenocorticotropin hormone and androgens, might be explained by the incipient of myelolipoma through chronic hormonal stimulation of the adrenal gland cortex. However, the etiopathogenesis of myelolipoma is still unclear.

Adrenal Gland Neoplasms↗

Viability of basidiomycete strains after cryopreservation: comparison of two different freezing protocols.

The viability of 250 basidiomycete strains was determined after a 2-d and then after a 2-year storage under liquid nitrogen using two different freezing protocols. Using an original agar plug protocol (OP), 162 strains (65%) of the 250 strains survived a 2-d storage and 158 strains (63%) survived a 2-year storage in liquid nitrogen. Using a straw protocol (CP), 246 strains (98%) of the 250 strains survived a 2-d storage and 243 strains (97%) a 2-year storage in liquid nitrogen. In addition, other 106 strains were newly estimated using the CP protocol; 104 (98%) of them survived successfully a 2-d storage and 101 (95%) of them survived a 2-year storage in liquid nitrogen. The results indicate that the protocol used for cryopreservation can significantly influence strain survival. Markedly better results were obtained using the CP protocol.

Basidiomycota↗

Inhibin B, follicle stimulating hormone, luteinizing hormone and testosterone during childhood and puberty in males: changes in serum concentrations in relation to age and stage of puberty.

Inhibin B is a gonadal dimeric polypeptide hormone that regulates synthesis and secretion of follicle stimulating hormone (FSH) in a negative feedback loop. The aim of the present study was to determine changes in serum inhibin B, gonadotropins and testosterone concentrations during childhood and puberty in males. We studied the relationship between circulating inhibin B, gonadotropins and testosterone in serum of healthy boys during the first two years of life and then in pubertal development. Using a recently developed two-side enzyme-linked immunosorbent assay (ELISA), inhibin B levels were measured in the serum of 78 healthy boys divided into eleven age groups from birth to the end of pubertal development. In addition, serum levels of gonadotropins and testosterone were measured. Serum inhibin B, gonadotropins and testosterone increased during the first months of postnatal life. A peak in serum inhibin B and gonadotropins concentrations was observed around 3-4 months of age. There was a significant positive correlation between serum inhibin B and gonadotropins and testosterone levels during the first 2 years of life. After this early increase, serum inhibin B, gonadotropins and testosterone levels decreased significantly and remained low until puberty followed by an increase beginning with the onset of puberty. Serum levels of inhibin B reached a peak at stage G3 of puberty. Around midpuberty, inhibin B lost its positive correlation with luteinizing hormone (LH) and testosterone from early puberty, and developed a strong negative correlation with FSH, which persisted into adulthood. We conclude that inhibin B plays a key role in the regulation of the hypothalamic-pituitary-gonadal hormonal axis during male childhood and pubertal development. Inhibin B is a direct marker of the presence and function of Sertoli cells and appears to reflect testicular function in boys.

Aging↗

Leptin levels in obese children: effects of gender, weight reduction and androgens.

Obesity in children is accompanied by increased circulating leptin concentrations. Girls have higher leptin concentrations than boys. The aim of our study was to compare serum leptin levels before and after a five-week weight reduction program and to study the relationship of leptin levels, serum total cholesterol, and androgens (testosterone, dehydroepiandrosterone, dehydroepiandrosterone sulphate) in 33 obese boys (age: 12.7+/-1.97 years, BMI: 30.46+/-4.54) and 66 obese girls (age: 12.7+/-2.51 years, BMI: 29.31+/-4.62). We found that serum leptin concentrations in obese children were significantly decreased after a weight reduction program (before 20.79+/-9.61 ng/ml, after 13.50+/-8.65 ng/ml in girls; before 12.25+/-10.09 ng/ml and after 5.18+/-3.56 ng/ml in boys, p<0.0001 in both genders). Leptin levels correlated positively with the body mass index before and after weight reduction. There was a positive association in obese boys and a negative one in obese girls between leptin levels and the WHR (waist to hip circumference ratio). Serum leptin also shows a strong relationship to fat distribution (p=0.02 in boys, p<0.0001 in girls). No significant correlation was found between leptin concentrations and total cholesterol or androgens. We confirmed that leptin is a sensitive parameter of body composition and weight reduction in obese children.

Adolescent↗

Inhibin B, follicle stimulating hormone, luteinizing hormone, and estradiol and their relationship to the regulation of follicle development in girls during childhood and puberty.

Inhibin B, produced by granulosa cells in the ovary, is a heterodimeric glycoprotein suppressing synthesis and secretion of the follicle stimulating hormone (FSH). The aim of the present study was to determine hormone profiles of inhibin B, FSH, luteinizing hormone (LH), and estradiol in girls during childhood and puberty and to evaluate whether inhibin B is a marker of follicle development. We examined the correlation between inhibin B and gonadotropins and estradiol during the first two years and across the pubertal development. Using a specific two-side enzyme-linked immunosorbent assay (ELISA), inhibin B levels were measured in the serum of 53 healthy girls divided into 8 groups according to age. In addition, serum FSH, LH, and estradiol were measured by chemiluminescent immunoassay in all serum samples. A rise in serum levels of inhibin B (55.2+/-7.3 ng/l, mean +/- S.E.M.) and FSH (1.78+/-0.26 UI/l), concomitant with a moderate increment of serum LH (0.36+/-0.09 UI/l) and estradiol (45.8+/-12.2 pmol/l) concentrations was observed during the first three months of life and declined to prepubertal concentrations thereafter. A strong positive correlation between inhibin B and FSH (r = 0.48, p<0.05), LH (r = 0.68, p<0.001) and estradiol (r = 0.59, p<0.01) was demonstrated during the first 2 years of life. A rise in serum levels of inhibin B, FSH, LH, and estradiol was found throughout puberty. Inhibin B had a strong positive correlation with FSH (stage I of puberty: r = 0.64, p<0.05; stage II of puberty: r = 0.86, p<0.01), LH (I: r = 0.61, p<0.05; II: r = 0.67, p<0.05), and estradiol (II: r = 0.62, p<0.05) in early puberty. From pubertal stage II, inhibin B lost this relationship to gonadotropins and estradiol. Serum inhibin B and FSH levels increased significantly during pubertal development, with the highest peak found in stage III of puberty (133.5+/-14.3 ng/l), and decreased thereafter. In conclusion, inhibin B is produced in a specific pattern in response to gonadotropin stimulation and plays an important role in the regulation of the hypothalamic-pituitary-gonadal axis during childhood and puberty in girls. Inhibin B is involved in regulatory functions in developing follicles and seems to be a sensitive marker of ovarian follicle development.

Adolescent↗

Common CYP21 gene mutations in Czech patients and statistical analysis of worldwide mutation distribution.

CYP21 gene molecular analysis was performed to determine the mutational analysis of 87 unrelated Czech patients with different forms of steroid 21-hydroxylase deficiency. Eight of the most common point mutations (intron 2 splice, P30L, 8 bp deletion in exon 3, I172N, V281L, Q318X, R356W, P453S) were analyzed using Amplification-created restriction site method. Cluster in exon 6 mutation was analyzed using allele-specific oligonucleotide hybridisation. Deletions and conversions were screened by modification of an sequence specific oligonucleotides hybridisation. The most frequent mutation in Czech patients was intron 2 splice mutation (45.4%). The comparison of mutation frequencies was made among Czech and European population (high frequency of intron 2 splice, 8bp deletion, P30L, P453S and low frequency of deletions/conversions in Czech population) and among Czech and different regions worldwide (low frequency of deletions/large gene conversions, V281L, R356W, high frequency of intron2, 8bp deletion, P30L and P453S in Czech population). We compared common mutation frequencies of different regions worldwide (North and South America, Asia, North Africa, Europe). Significant differencies in selected regions were determined by ANOVA statistical analysis (One-sample t-test, confidence interval) at value of p<0.05.

Adolescent↗

Cryopreservation of basidiomycete strains using perlite.

A new alternative method using perlite as a particulate solid carrier in the growth medium with a cryoprotectant was successfully tested for cryopreservation of several basidiomycete species from different genera (Armillaria, Pleurotus, Pluteus, Polyporus) which failed to survive or retain their properties in cryopreservation procedures routinely used in our laboratory. Frozen basidiomycete strains were kept in cryovials submerged in liquid nitrogen and were either immediately after the freezing process or after a 6-month storage thawed and checked for viability, purity and changes in growth, morphology and biochemical characteristics. All cultures survived the cryopreservation procedure and no negative effects of cryopreservation by this method have been observed after 6 months of storage in liquid nitrogen.

Aluminum Oxide↗

Activities of ligninolytic enzymes in some white-rot basidiomycete strains after recovering from cryopreservation in liquid nitrogen.

Fourteen strains of white-rot basidiomycetes belonging to eight species of two genera (Inonotus and Pholiota) were tested for their ability to maintain the production of laccase, peroxidase and manganese-dependent peroxidase (enzymes involved in lignin biodegradation) after a short-time preservation in liquid nitrogen with different cryoprotectives (glycerol, dimethyl sulfoxide). No negative effect of cryopreservation or the used cryoprotective on production of the ligninolytic enzymes was found in the fungi tested.

Basidiomycota↗

[Anthropologic methods in the evaluation of the weight reduction process in obese children].

Childhood obesity is considered at present one of the most difficult problems in paediatrics and it is one of the major health problems of industrialized society. Growth and development of obese children is different in several aspects. The authors submit the results of an anthropometric survey implemented in 1989 to 1996 in a group of 1949 Czech obese children (720 boys and 1229 girls) aged 6 to 18 years. The patients were measured by the standard anthropometric technique according to Matrin and Saller at the beginning and end of six-week therapeutic weight reduction programme. The skinfold thickness at 14 sites was assessed by means of a Best caliper. The body composition was evaluated using Matiegka's equations. For evaluation of the weight reduction programme the paired t-test was used a criterium of the quality of the evaluated parameters. For the needs of the medical profession curves of the empirical percentiles of BMI for both sexes were plotted to evaluate the grade of obesity. From the analysis of anthropometric data using the paired t-test in 1949 probands ensues that the most valuable information as regards circumferential measurements in boys is provided by the circumference of the abdomen, the gluteal area followed by the chest circumference. In girls the results are partly different--a gluteal circumference of thigh highly dominates, gluteal circumference follows, chest circumference mesoeternal is the third. As to skinfolds the authors recommended to monitor in boys the supraileac, subscapular and abdominal skinfold. In girls the following order was assessed: supraileac, thoracic (at the level of the 10th rib) and subscapular skinfold. From the accurate assessment of the body composition of obese children subjected to weight reduction ensues our recommendation that the loss of adipose tissue should be at least seven times greater than the loss of musculature. A lower ratio should be an indication for changing the reducing treatment. Methods of direct anthropometry, incl. calipering can be unequivocally recommended as the method of first choice because of their non-invasive character.

Adolescent↗

[Changes in bone metabolism in girls with Turner's syndrome].

BACKGROUND: Data of the prevalence of osteoporosis in girls with Turner's syndrome are not uniform, and its causes have not been fully elucidated. Information on the mineralization of osseous tissue is controversial. The objective of the present work was to examine some more recent indicators of bone metabolism in a group of girls with Turner's syndrome. METHODS AND RESULTS: A group of girls aged 4-20 years was examined where genetic examination had revealed karyotype 45 X (7 patients) or mosaic 45 X (46 XX) (9 patients). On X-ray examination osteoporosis was found in 71%, densitometric evidence of reduced bone density was provided in 2% of the examined patients. As to biochemical osteologically oriented examinations, a significantly reduced osteocalcin value was found (1.18 +/- 0.42 mug/l, as compared with 11.38 +/- 0.03 in controls, p < 0.001), reduced values of alkaline phosphatase (2.67 mukat/l as compared with 8.46 +/- 4.16 mukat/l in controls, p < 0.005) and reduced values of the bone isoenzyme of alkaline phosphatase (1.47 mukat/l as compared with 6.04 +/- 0.27 in controls, p < 0.001). The values of calcemia (2.63 / 0.13 mmol/l) and phosphataemia (1.36 +/- 0.25 mmol/l) did not differ significantly from values recorded in controls. To six patients the authors administered for a period of three months 1,25(OH)2D3, 0.25 mg on alternate days. The osteocalcin values rose to 5.27 +/- 3.14 micrograms/l, similarly as alkaline phosphatase (6.32 +/- 1.83 mukat/l) and the bone isoenzyme of alkaline phosphatase (2.42 +/- 1.95 mukat/l), not evaluated statistically because of the small number of patients. CONCLUSIONS: In girl with Turner's syndrome a reduced bone density was revealed in 25%, reduced values of osteocalcin, alkaline phosphatase and its bone isoenzyme indicate a reduced osteoblast activity. It appears that administration of 1,25(OH)2D3 can have a favourable effect.

Adolescent↗

Osteocalcin in congenital adrenal hyperplasia.

Osteocalcin in the serum reflects bone turnover. It is known that prolonged therapy with glucocorticoids inhibits bone turnover. The aim of this study was to evaluate the osteocalcin level in children with congenital adrenal hyperplasia treated by glucocorticoids and mineralocorticoids and to assess the influence of 1,25(OH)2D3. The subjects were 75 children with congenital adrenal hyperplasia, aged 1-18 years, treated with glucocorticoids and mineralocorticoids in substitution doses from birth. These children demonstrated low levels of osteocalcin and alkaline phosphatase, whereas calcium and phosphate were in the normal ranges. Despite these abnormalities, no osteoporosis was detectable and a normal growth rate was confirmed, most probably because of higher levels of androgens; 17-OH progesterone averaged 11.8 nmol/l. After treatment with 1,25(OH)2D3, the osteocalcin levels increased, followed later by increases of alkaline phosphatase and bone isoenzyme.

Adrenal Hyperplasia, Congenital↗

[Complete isosexual precocious puberty (true, LHRH-dependent)-- personal experience].

The authors examined a group of 10 girls with true sexual precocity. The age of the patients was within the range of 11 months and seven and a half years. Height acceleration was on average almost one year, skeletal age acceleration was on average almost one and a half years; when BA was evaluated according to tables of visualization methods of the Motol Clinic and the TW2 and C method, a marked acceleration of BA was observed in the RUS method. To differentiate incomplete puberty-thelarche-in all girls the stimulation test, using Relefact LHRH (Hoechst), was performed, the diagnosis of true puberty was suggested by LH serum levels elevated above 12 IU/l. In incomplete puberty only FSH was elevated. In all patients oestrogenization of the vaginal epithelium, breast development (M2-3) and pubic hair was present, in three patients menarche. For treatment Decapeptyl-Depot (Ferring) was administered, the longest period of treatment was eight months. Therefore the change of HA and BA was not evaluated, but even during this brief period of treatment regression of pubertal sex signs occurred.

Child↗

Evaluation of SHBG test for disclosure of insensitivity to androgens.

A laboratory test enabling to reveal and confirm the suspicion on the insensitivity to androgens was evaluated. The test consisted of determination of the decline in plasma level of sex hormone binding globulin (SHBG) on the 7th day after administration of a single dose of testosterone preparation (Testoviron Depot 100, Schering, 2 mg/kg body weight). The upper limit of percentual SHBG decline, below which the patients can be considered normal responders (i.e. subjects without insensitivity to androgens), obtained from the follow up of the time course of SHBG changes after Testoviron application to 6 healthy men, was established as 83.6%. The level of percentual SHBG decline, above which the patients were considered non-responders with impaired peripheral sensitivity to androgens was assessed from the imprecision of immunoradiometric determination of SHBG and amounted 91.9%. The test was used and evaluated in 26 patients with 46 XY karyotype, representing various cases of intersex, gonadal dysgenesis and male hypogonadism, out of which four subjects with clinically confirmed impaired peripheral sensitivity to androgens were discovered. The test however, appeared not to be reliable in cases of very low basal SHBG levels, i.e. below 15 nmol/l.

Adolescent↗

[A non-classic form of congenital adrenal hyperplasia].

The authors examined five children with the non-classical form of adrenal hyperplasia. The clinical symptoms of the disease comprise: early congenital pubic hair, small stature, accelerated bone maturation, infertility, in girls hirsutism, acne, menstrual disorders, amenorrhoea, in boys oligospermia, acne. Laboratory examination reveals slightly elevated 17-OH progesterone values during the synactene loading test. It is an autosomal recessively hereditary disease. It is due to an allelic variant of the gene for 21 hydroxylase. The authors revealed a link with antigen B14. Even in the small group examined antigen B14 was present in 40%, although the prevalence in our population is 2.9%.

Adolescent↗

A case of hyperplasia of the anterior lobe of the pituitary imitating intrasellar expansion.

The authors describe the case of an obese girl with severe hypothyroidism which led to increased TSH secretion and finally to hyperplasia of the anterior lobe of the pituitary diagnosed by magnetic resonance. After substitution treatment with thyroid hormones was started, marked improvement of the general condition, loss of body weight and normalization of laboratory findings and regression of hyperplastic tissue occurred.

Child↗

[Juvenile granulosa cell tumor with subsequent occurrence of gastrointestinal polyposis, subcutaneous lipomatosis and nodular goiter].

A juvenile tumour from granulosa cells (M-8622/1), 13 x 8 x 6 cm, in the right ovary in a three-month-old girl produced some symptoms of pseudopubertas praecox isosexualis which disappeared after operation. Microscopic examination of the tumour revealed in addition to typical structures a less common differentiation to Sertoli cells. Despite actinotherapy and chemotherapy one and a half years after the onset of the disease X-ray examination revealed metastases in the lungs which were successfully cured by further doses of the above two types of treatment. Between the age of 6 and 15 years the girl developed successively polyposis of the stomach, small and large intestine (M-7564/0), subcutaneous lipomatosis of the trunk and left lower extremity (M-8881/0) and nodular goitre (M-7164/0), predominantly quiescent. In the literature a connection between gonadal stromal ovarian tumours and mesenchymal tumours, intestinal polyposis and disorders of the thyroid gland is described, but in different patients. The authors' observation is unique by the successive incidence of these changes in a single patient surviving 15 years after operation; and thus genetically conditioned associations could be involved.

Adolescent↗