Objectives: To determine trends in clinical practice for individuals with DSD requiring gonadectomy. Design: Retrospective cohort study. Methods: Information regarding age at gonadectomy according to diagnosis; reported sex; time of presentation to specialist centre; and location of centre from cases reported to the International DSD Registry and who were over 16 years old in January 2019. Results: Data regarding gonadectomy were available in 668 (88%) individuals from 44 centres. Of these, 248 (37%) (median age (range) 24 (17, 75) years) were male and 420 (63%) (median age (range) 26 (16, 86) years) were female. Gonadectomy was reported from 36 centres in 351/668 cases (53%). Females were more likely to undergo gonadectomy (n = 311, P < 0.0001). The indication for gonadectomy was reported in 268 (76%). The most common indication was mitigation of tumour risk in 172 (64%). Variations in the practice of gonadectomy were observed; of the 351 cases from 36 centres, 17 (5%) at 9 centres had undergone gonadectomy before their first presentation to the specialist centre. Median age at gonadectomy of cases from high-income countries and low-/middle-income countries (LMIC) was 13.0 years (0.1, 68) years and 16.5 years (1, 28), respectively (P < 0.0001) with the likelihood of long-term retention of gonads being higher in LMIC countries. Conclusions: The likelihood of gonadectomy depends on the underlying diagnosis, sex of rearing and the geographical setting. Clinical benchmarks, which can be studied across all forms of DSD will allow a better understanding of the variation in the practice of gonadectomy.
- MeSH
- dospělí MeSH
- kastrace statistika a číselné údaje MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- poruchy sexuálního vývoje diagnóza epidemiologie chirurgie MeSH
- registrace MeSH
- retrospektivní studie MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
Vydání třetí, přepracované a doplněné 542 stran : ilustrace, grafy ; 21 cm
Publikace se zaměřuje na psychický vývoj jedince v období dětství a dospívání. Určeno odborné veřejnosti.
- MeSH
- dětská psychologie MeSH
- psychologie adolescentů MeSH
- vývojová psychologie MeSH
- Publikační typ
- monografie MeSH
- Konspekt
- Vývojová psychologie. Individuální psychologie
- NLK Obory
- psychologie, klinická psychologie
Článek shrnuje nové poznatky o metabolickém syndromu u dětí a adolescentů. Tento syndrom shrnuje soubor patologických znaků - hypertenzi, poruchy glukózového metabolismu, dyslipidemii, abdominální obezitu, které mohou být rizikem pro rozvoj kardiovaskulárního onemocnění v dospělosti. Metabolický syndrom je v pediatrii obtížně definován, není dosud jednotná klasifikace.
The article summarises new views of metabolic syndrome in pediatrics - hypertension, altered glucose metabolism, dyslipidemia, abdominal obesity, which can lead to risk of cardivascular diseases in adults. The definition of this syndrome in pediatrics is difficult, there is nonuniform classification.
BACKGROUND AND OBJECTIVE: It is unclear whether the proportion of infants with a disorder of sex development who are raised as male or female has changed over time. The temporal trends in sex assignment of affected cases entered in the International Disorder of Sex Development (I-DSD) Registry were studied. METHODS: Cases of disorders of sex development reported as partial androgen insensitivity syndrome (PAIS; n = 118), disorder of gonadal development (DGD; n = 232), and disorder of androgen synthesis (DAS; n = 104) were divided into those who were born before 1990, 1990-1999, and after 1999. External appearance of the genitalia was described by the external masculinization score. RESULTS: The median (5th-95th percentile) external masculinization scores of those infants with PAIS, DGD, and DAS who were raised as boys were 6 (2-9), 6 (3-9), and 6 (1-12), respectively, and were significantly higher than in those raised as girls (2 [0-6], 2 [0-7], and 0 [0-5], respectively); this difference was maintained in the 3 temporal birth cohorts (P < .01). Of the 118 cases in the pre-1990 cohort, 41 (35%) were raised as boys; of the 148 cases in the 1990-1999 cohort, 60 (41%) were raised as boys; and of the 188 cases in the post-1999 cohort, 128 (68%) were raised as boys. CONCLUSIONS: Although there is an association between the external appearance of the genitalia and the choice of sex assignment, there are clear temporal trends in this practice pointing toward an increased likelihood of affected infants being raised as boys. The impact of this change in practice on long-term health outcomes requires additional focus.
- MeSH
- časové faktory MeSH
- dospělí MeSH
- genderová identita * MeSH
- kohortové studie MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- následné studie MeSH
- poruchy sexuálního vývoje diagnóza epidemiologie terapie MeSH
- registrace MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
- práce podpořená grantem MeSH
- MeSH
- adrenokortikotropní hormon izolace a purifikace nedostatek MeSH
- analýza určování pohlaví * metody trendy využití MeSH
- časná diagnóza MeSH
- genetické testování metody trendy využití MeSH
- hormony izolace a purifikace nedostatek MeSH
- Klinefelterův syndrom MeSH
- lidé MeSH
- mezinárodní klasifikace nemocí * normy využití MeSH
- novorozenec MeSH
- ovotestikulární poruchy sexuálního vývoje MeSH
- poruchy sexuálního vývoje * diagnóza etiologie MeSH
- smíšená gonadální dysgeneze MeSH
- společnosti lékařské klasifikace normy MeSH
- Turnerův syndrom MeSH
- Check Tag
- lidé MeSH
- novorozenec MeSH
- Publikační typ
- abstrakt z konference MeSH
Je uvedena nová klasifikace vrozených poruch vývoje vnitřního a zevního pohlavního ústrojí a některé příklady.
New classification and some examples of inherit abnormalities of the development of internal and external genitalia is provided.
- MeSH
- dítě MeSH
- lidé MeSH
- poruchy gonád klasifikace MeSH
- poruchy sexuálního vývoje klasifikace MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
