JOURNAL OF THE CZECH PEDIATRIC SOCIETY AND THE SLOVAK PEDIATRIC SOCIETY

Česko-slovenská pediatrie, 2022 (vol. 77), Suppl.3

EditorialEditorial

Hana Cabrnochová a Jan Lebl

Čes-slov Pediat 2022, 77(Suppl.3):S4  

Minipuberty - an important and still neglected period of sexual developmentComprehensive Report

Jiřina Zapletalová

Čes-slov Pediat 2022, 77(Suppl.3):S5-S10 | DOI: 10.55095/CSPediatrie2022/052  

Minipuberty is defined as temporary postnatal activation of the hypothalamic-pituitary-gonadal (HPG) axis. The HPG axis is triggered three times during lifetime. The first time in the middle of gestation, when it participates in the development and maturation of the sex glands. This is followed by the so-called minipuberty, which begins a few days after birth and lasts 4-6 months in boys and 2-4 y ears in girls. After the "hormonally sillent" childhood comes classical sexual adolescence with the development of secondary sexual signs in the second decade of life and is completed by a complete maturation of the sex glands allowing future reproduction....

Current perspectives on the aetiology of tall stature in children and adolescents (1): Syndromes associated with tall statureComprehensive Report

Kateřina Adamovičová, Lukáš Plachý, Jan Lebl, Stanislava Koloušková, Štěpánka Průhová

Čes-slov Pediat 2022, 77(Suppl.3):S11-S17 | DOI: 10.55095/CSPediatrie2022/053  

Tall stature is defined as body height more than +2 standard deviations (SD) for age and sex. The aetiology of tall stature can be genetic or non-genetic. Syndromic tall stature belongs to the genetic causes - e.g. Marfan syndrome, Klinefelter syndrome, Beckwith- Wiedemann syndrome, Sotos syndrome, Simpson-Golabi-Behmel syndrome, Malan syndrome, Weaver syndrome, Proteus syndrome, as well as syndro mes caused by gonosomic trisomy (47,XXX, 47,XYY) or fragile X syndrome and homocystinuria etc. The diagnostics of tall stature is based on clinical evaluation of the typical signs and molecular genetic examination. Syndromologic tall stature can be associated...

Current perspectives on the aetiology of tall stature in children and adolescents (2): Non-syndromic tall statureComprehensive Report

Kateřina Adamovičová, Lukáš Plachý, Jan Lebl, Stanislava Koloušková, Štěpánka Průhová

Čes-slov Pediat 2022, 77(Suppl.3):S18-S23 | DOI: 10.55095/CSPediatrie2022/054  

Non-syndromic tall stature can be caused by growth hormone overproduction, hyperthyroidism, obesity, hypogonadism, precocious puberty or as constitutional growth acceleration. There are conditions caused by a genetic cause as well such as non-sydromic tall stature, typically familial tall stature or, more rarely, pituitary gigantism, which includes X-linked acrogigantism (XLAG). Tall stature can r arely occur in neurofibromatosis type I or in glucocorticoid resistance. After excluding the common endocrinopathies the patients are, in clinical practice, assigned as "familial tall stature" or "constitutional growth acceleration". Recently, there have...

Endocrine causes of obesity in childhood and adolescenceComprehensive Report

Renata Pomahačová, Petra Paterová, Eva Nykodýmová, Eva Sládková, Eva Skalická, Josef Sýkora

Čes-slov Pediat 2022, 77(Suppl.3):S24-S29 | DOI: 10.55095/CSPediatrie2022/055  

The most common type of childhood obesity is simple obesity which develops as a result of a combination of certain genetic and environmental factors. Secondary obesity is caused by rare genetic forms of obesity, by certain drugs and by the following endocrine diseases: hypothyroidism, Cushing' s syndrome, growth hormone deficiency, hypogonadism, pseudohypoparathyroidism type Ia, insulinoma, hypothalamic obesity and polycystic ovary syndrome. Early detection of the endocrine cause of obesity leads to a reduction in morbidity and, in some cases, mortality in these children. Doctors must diagnose secondary obesity, distinguish it from simple obesity,...

Children and adolescents with gender incongruence: current approaches and situation in the Czech RepublicComprehensive Report

Marta Šnajderová, Jiřina Zapletalová, Olga Magnová, Hana Fifková, Petr Weiss, David Neumann

Čes-slov Pediat 2022, 77(Suppl.3):S30-S35 | DOI: 10.55095/CSPediatrie2022/056  

In recent years, there has been a significant increase in new cases of children and adolescents with gender incongruence. Most of them are aged 15-17. Many individuals seek endocrine care on the recommendation of a sexologist. The cause of the rising trend is not entirely clear, some attitudes to the proposed procedures also differ. This overview article summarizes current approaches and opinions on the care of children and adolescents with gender incongruence, and also presents the current situation in the Czech Republic.

First experience with long-acting growth hormoneShort News

Jan Lebl, Ledjona Toni, Adriana Dankovčíková, Ľudmila Košťálová, Olga Magnová, Stanislava Koloušková

Čes-slov Pediat 2022, 77(Suppl.3):S36-S40 | DOI: 10.55095/CSPediatrie2022/057  

Therapeutic use of growth hormone spans back over 65 years. The drug molecule remained unchanged, despite a switch in production technology. It is being administered in form of subcutaneous daily injections for multiple years in most patients, which may represent a significant burden for the child and his/her family. Three modified molecules of long-acting growth hormone (LAGH) with once weekly ad ministration have been developed and are approaching clinical use. Their growth effect is equivalent to daily growth hormone injections. Lonapegsomatropin is a pegylated pro-molecule temporarily conjugated with a carrier, allowing regular release of native...

Mystický Hagibor a jeho místo v historii české pediatrieNews

Jan Lebl

Čes-slov Pediat 2022, 77(Suppl.3):S41-S43