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Biomedical subjects

B Kalvachová

Publications and source records attributed to B Kalvachová.

At least 19 recordsLinked to original sources

[Turner syndrome: overview of problems, present status, proposals for care and a protocol for monitoring in childhood, adolescence and adulthood].

BACKGROUND: Girls and adolescents with Turner syndrome (TS) usually receive intensive medical care in a multidisciplinary team, coordinated by paediatric endocrinologist. Majority of them are discharged from specialist clinics following the induction of puberty and attainment of final height. Patients with Turner syndrome have a reduced life expectancy, they are known to have multi-system impairments in addition to the short stature and to the absence of sexual development. Aim of this study is to propos a continuous follow-up by multidisciplinary team of physicians starting in childhood and following the discharge from the paediatric care. METHODS AND RESULTS: This paper highlights the medical and psychosocial problems associated with Turner syndrome in childhood, adolescence and in adulthood. Analysis of these problems served as a background to management strategy. CONCLUSIONS: Women with Turner syndrome are at risk of number of medical problems. Quality of their life and the life expectancy can be improved with increasing awareness to comorbities associated with Turner syndrome. Assisted reproduction technologies has recently offered a chance for pregnancy and delivery of a healthy child also to women with Turner syndrome. Therefore, long-term follow-up by multidisciplinary team of physicians knowledgeable about these medical problems is necessary. Introduction of a centralised system of systematic multidisciplinary approach to patients with Turner syndrome from childhood and adolescence to adulthood seems to be very important.

Adolescent↗

[Reproductive medicine from childhood to adulthood].

Modern era brought new diagnostic and therapeutic methods to endocrinology and changed the approach to many diseases and developmental disorders. Explosion of new findings brought about the possibility of examinations on subcellular level. Possibility to study structure and function of various genes is not any more the futurology. However, still in medicine romain a roas which are considered as less important. It is usually assumed that questions of fertility will be dealt by the specialist for adults. We suppose that the given problems should be well advised by an interdisciplinary co-operating team in the paediatric patients already. However, it should be recognised that some reproduction disorders have their origin in the early age. The aim of this paper is to give an overview on endocrine disorders in relation to reproduction impairments and to outline possibilities of co-operation between different specialist treating patients from childhood trough adulthood.

Adult↗

[Adult height of patients after long-term treatment of idiopathic growth hormone deficiency. (Czech Registry 1991-1998)].

BACKGROUND: Final height was evaluated in patients from the Czech Register with idiopathic growth hormone deficiency treated with GH. METHODS AND RESULTS: 23 patients had the isolated growth hormone deficiency (group 1), and 37 suffered from multiple pituitary hormones deficiencies (group 2). The patients from group 1 and 2 were given growth hormone for 6.7 +/- 2.2 years and 9.6 +/- 3.0 years, respectively. The patients with isolated growth hormone deficiency reached final height -1.4 +/- 1.3 SDS, those with multiple hormone deficiencies were taller (-0.7 +/- 1.5 SDS). The height gain equalled +2.5 +/- 0.6 SDS and +3.4 +/- 1.1 SDS in group 1 and 2, respectively. 70% of the patients in group 1 and 93% of group 2 reached final heights within the target limits (+/- 2 SDS). CONCLUSION: The final height positively correlated with the target height (mid-parental height) and height at the onset of puberty (group 1 and 2). There was a negative correlation between the final height and the chronological age at the beginning of growth hormone therapy in group 1.

Adult↗

[The physiology of growth in children].

The presented paper is based on laws governing growth and development of humans, it reminds of various aspects of the comprehensive evaluation of the developmental stage of the human organism and makes the reader familiar with some factors of the endogenous and exogenous environment which influence growth and development. The author presents also an account of recent findings on the function and mutual harmony of neurohumoral regulations of intrauterine and postnatal growth, on the transmission of information and the response of target tissues.

Child↗

The effect of long-term glucocorticoid therapy on glucocorticoid receptor content and on steroid response to ACTH.

The effect of long-term glucocorticoid therapy for systemic diseases on glucocorticoid receptor (GR) content and on basal and ACTH-stimulated levels of plasma and salivary cortisol 17 alpha-hydroxy-progesterone, androstenedione, 11 beta-hydroxyandrostenedione, DHEA, its sulfate and sex hormone-binding globulin (SHBG), as well as on basal levels of aldosterone, was investigated in a group of 24 children treated with prednisone for at least 8 months. The therapy was interrupted 24 h before the ACTH test and before plasma and saliva sampling. The control group consisted of 21 healthy children of corresponding age and sex. The patients were divided into two subgroups with normal and subnormal basal cortisolemia, they also differed in their response to ACTH. The GR levels in patient groups were indistinguishable from those found in controls. No correlation was found between GR content and basal levels of the above steroids or their response to ACTH. The best markers, apart from basal cortisolemia, for evaluation of the degree of suppression of adrenal function appeared to be the response of salivary (but not of plasma) cortisol and 17 alpha-hydroxy-progesterone to ACTH. Surprisingly, significantly lower levels of SHBG levels, which rose markedly after ACTH, were found in all the patients.

Adolescent↗

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 laboratory test for the detection of androgen resistance using sex hormone-binding globulin].

A simple laboratory test for disclosure of peripheral androgen insensitivity has been suggested. The test is based on determination of sex hormone-binding globulin (SHBG) following single administration of testosterone preparation (Testoviron Depot, Schering, 2 mg/kg of body weight). The kinetics of SHBG response to exogenous testosterone was investigated first in a group of 5 healthy male volunteers. The highest highly significant decrease of SHBG level occurred 7th day after drug administration. The method of choice for SHBG determination is an immunoradiometric assay (IRMA). The test was used for a group of 15 children and young people suspected for androgen sensitivity disorder. In all cases, besides SHBG on days 0 and 7 testosterone, dihydrotestosterone and LH were measured, too. In three instances the response was negative: in the case of male Turner syndrome, with very low basal SHBG levels, in one case of confirmed diagnosis of testicular feminization and in the third case of suspected testicular feminization, which has not yet been concluded. In all other patients a significant decrease (in average to 62.8% of the basal level) was in agreement with clinical findings.

Adolescent↗

[Surgical treatment of cryptorchism in scrotal hypoplasia].

In the submitted paper the authors give an account of their experience with the treatment of a patient with severe scrotal hypoplasia which is a very rare inborn defect. In a group of 1403 boys operated in the last ten years on account of cryptorchism this anomaly recorded only once, i.e. in 0.071%. The authors recommend a two-stage operation. In the first stage they use a V-Y plastic operation to create a neoscrotum. In the second stage orchidopexy is performed. Collaboration with a child endocrinologist is important. The aim of endocrinological examination is not only evidence of the presence and function of the gonads by a hormonal test, but in case of associated anomalies, also the assessment of genetic, genital and gonadal sex.

Cryptorchidism↗

[Hyperandrogenic states in girls and young women].

The authors present a group of 20 girls and young women with various clinical symptoms of hyperandrogenism. The common finding in all these patients is an elevated excretion of desoxy-fraction of 17-oxosteroids, in particular androsterone in urine. The authors describe in detail the case-history of a girl with pubarché praecox and premature closure of the epiphyseal crevices and a young woman with polycystic ovary syndrome. The authors supplement the reflections on the aetiology of hyperandrogenism by suggestion of an algorithm examination and therapeutic procedure when excessive androgen production in the female organism is confirmed.

Adolescent↗