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Archives of Disease in Childhood Apr 2024Increasing numbers of children and adolescents experiencing gender dysphoria/incongruence are being referred to specialist gender services and there are various... (Review)
Review
BACKGROUND
Increasing numbers of children and adolescents experiencing gender dysphoria/incongruence are being referred to specialist gender services and there are various published guidelines outlining approaches to clinical care.
AIM
To examine the recommendations about the management of children and/or adolescents (age 0-18) experiencing gender dysphoria/incongruence in published guidelines or clinical guidance. A separate paper examines the quality and development of guidelines.
METHODS
A systematic review and narrative synthesis. Databases (Medline, Embase, CINAHL, PsycINFO, Web of Science) were searched to April 2022 and web-based searches and contact with international experts continued to December 2022, with results assessed independently by two reviewers. The Appraisal of Guidelines for Research and Evaluation tool was used to examine guideline quality.
RESULTS
23 guidelines/clinical guidance publications (1998-2022) were identified (4 international, 3 regional, 16 national). Guidelines describe a similar care pathway starting with psychosocial care for prepubertal children, puberty suppressants followed by hormones for eligible adolescents and surgical interventions as these adolescents enter adulthood. In general, there is consensus that adolescents should receive a multidisciplinary assessment, although clear guidance about the purpose or approach is lacking. There are differing recommendations about when and on what basis psychological and medical interventions should be offered. There is limited guidance about what psychological care should be provided, about the management of prepubertal children or those with a non-binary gender identity, nor about pathways between specialist gender services and other providers.
CONCLUSIONS
Published guidance describes a similar care pathway; however, there is no current consensus about the purpose and process of assessment for children or adolescents with gender dysphoria/incongruence, or about when psychological or hormonal interventions should be offered and on what basis.
PROSPERO REGISTRATION NUMBER
CRD42021289659.
PubMed: 38594048
DOI: 10.1136/archdischild-2023-326500 -
Journal of Cystic Fibrosis : Official... Feb 2024This systematic review summarizes the impact of cystic fibrosis (CF) on sexual and reproductive health (SRH) in males and females, covering pubertal development,... (Review)
Review
This systematic review summarizes the impact of cystic fibrosis (CF) on sexual and reproductive health (SRH) in males and females, covering pubertal development, hormonal function, family planning, and fertility. Included articles featured historical CF diagnostic criteria, preclinical or clinical data (retrospective cohorts or open label trials), while excluded articles lacked full text availability, explicit methodology, or comparisons between CF and non-CF patients. Genotype differences in CFTR mutations influenced symptom severity. Males with CF experienced delayed puberty, hypogonadism, infertility from obstructive azoospermia, and semen parameter issues. Female CF patients showed decreased fertility, possibly linked to disrupted ionic balance and ovarian cystic disease. Assistive reproductive technologies addressed fertility issues, but success varied based on disease severity and genotype. CFTR modulators aided pulmonary function and sexual health but require further assessment for fertility benefits.
PubMed: 38311513
DOI: 10.1016/j.jcf.2024.01.009 -
Zeitschrift Fur Kinder- Und... 2024Beyond NICE: Updated Systematic Review on the Current Evidence of Using Puberty Blocking Pharmacological Agents and Cross-Sex-Hormones in Minors with Gender Dysphoria...
Beyond NICE: Updated Systematic Review on the Current Evidence of Using Puberty Blocking Pharmacological Agents and Cross-Sex-Hormones in Minors with Gender Dysphoria The suppression of physiological puberty using puberty-blocking pharmacological agents (PB) and prescribing cross-sex hormones (CSH) to minors with gender dysphoria (GD) is a current matter of discussion, and in some cases, PB and CSH are used in clinical practice for this particular population. Two systematic reviews (one on PB, one on CSH treatment) by the British National Institute for Clinical Excellence (NICE) from 2020 indicated no clear clinical benefit of such treatments regarding critical outcome variables. In particular, these two systematic NICE reviews on the use of PB and CSH in minors with GD detected no clear improvements of GD symptoms. Moreover, the overall scientific quality of the available evidence, as discussed within the above-mentioned two NICE reviews, was classified as "very low certainty" regarding modified GRADE criteria. The present systematic review presents an updated literature search on this particular topic (use of PB and CSH in minors with GD) following NICE principles and PICO criteria for all relevant new original research studies published since the release of the two above-mentioned NICE reviews (updated literature search period was July 2020-August 2023). The newly conducted literature search revealed no newly published original studies targeting NICE-defined critical and important outcomes and the related use of PB in minors with GD following PICO criteria. For CSH treatment, we found two new studies that met PICO criteria, but these particular two studies had low participant numbers, yielded no significant additional clear evidence for specific and clearly beneficial effects of CSH in minors with GD, and could be classified as "low certainty" tfollowing modified GRADE criteria. The currently available studies on the use of PB and CSH in minors with GD have significant conceptual and methodological flaws. The available evidence on the use of PB and CSH in minors with GD is very limited and based on only a few studies with small numbers, and these studies have problematic methodology and quality. There also is a lack of adequate and meaningful long-term studies. Current evidence doesn't suggest that GD symptoms and mental health significantly improve when PB or CSH are used in minors with GD. Psychotherapeutic interventions to address and reduce the experienced burden can become relevant in children and adolescents with GD. If the decision to use PB and/or CSH is made on an individual case-by-case basis and after a complete and thorough mental health assessment, potential treatment of possibly co-occurring mental health problems as well as after a thoroughly conducted and carefully executed individual risk-benefit evaluation, doing so as part of clinical studies or research projects, as currently done in England, can be of value in terms of generation of new research data. The electronic supplement (ESM) 1 is an adapted and abreviated English version of this work.
Topics: Humans; Gender Dysphoria; Adolescent; Child; Female; Male; Puberty; Minors; Gonadal Steroid Hormones; Puberty Suppression
PubMed: 38410090
DOI: 10.1024/1422-4917/a000972 -
Genes Mar 2024Among aneuploidies compatible with life, trisomy 22 mosaicism is extremely rare, and only about 25 postnatal and 18 prenatal cases have been described in the literature... (Review)
Review
BACKGROUND
Among aneuploidies compatible with life, trisomy 22 mosaicism is extremely rare, and only about 25 postnatal and 18 prenatal cases have been described in the literature so far. The condition is mainly characterized by facial and body asymmetry, cardiac heart defects, facial dysmorphisms, growth failure, delayed puberty, and variable degrees of neurodevelopmental delay.
PROBLEM
The scattered information regarding the condition and the dearth of data on its natural history and developmental outcomes restrict genetic counseling, particularly in prenatal settings. Moreover, a prompt diagnosis is frequently delayed by the negative selection of trisomic cells in blood, with mosaicism percentage varying among tissues, which often entails the need for further testing. Purpose/topic: The aim of our work is to provide assistance in prenatal and postnatal genetic counseling by systematically delineating the current knowledge of the condition. This entails defining the prenatal and postnatal characteristics of the condition and presenting novel data from three cases, both prenatally and postnatally. Additionally, we report the developmental outcomes observed in two new patients.
Topics: Pregnancy; Female; Humans; Mosaicism; Prenatal Diagnosis; Trisomy; Chromosomes, Human, Pair 22; Uniparental Disomy; Chromosome Disorders
PubMed: 38540405
DOI: 10.3390/genes15030346 -
Archives of Disease in Childhood Apr 2024Treatment to suppress or lessen effects of puberty are outlined in clinical guidelines for adolescents experiencing gender dysphoria/incongruence. Robust evidence...
BACKGROUND
Treatment to suppress or lessen effects of puberty are outlined in clinical guidelines for adolescents experiencing gender dysphoria/incongruence. Robust evidence concerning risks and benefits is lacking and there is a need to aggregate evidence as new studies are published.
AIM
To identify and synthesise studies assessing the outcomes of puberty suppression in adolescents experiencing gender dysphoria/incongruence.
METHODS
A systematic review and narrative synthesis. Database searches (Medline, Embase, CINAHL, PsycINFO, Web of Science) were performed in April 2022, with results assessed independently by two reviewers. An adapted version of the Newcastle-Ottawa Scale for cohort studies was used to appraise study quality. Only moderate-quality and high-quality studies were synthesised. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses reporting guidelines were used.
RESULTS
11 cohort, 8 cross-sectional and 31 pre-post studies were included (n=50). One cross-sectional study was high quality, 25 studies were moderate quality (including 5 cohort studies) and 24 were low quality. Synthesis of moderate-quality and high-quality studies showed consistent evidence demonstrating efficacy for suppressing puberty. Height increased in multiple studies, although not in line with expected growth. Multiple studies reported reductions in bone density during treatment. Limited and/or inconsistent evidence was found in relation to gender dysphoria, psychological and psychosocial health, body satisfaction, cardiometabolic risk, cognitive development and fertility.
CONCLUSIONS
There is a lack of high-quality research assessing puberty suppression in adolescents experiencing gender dysphoria/incongruence. No conclusions can be drawn about the impact on gender dysphoria, mental and psychosocial health or cognitive development. Bone health and height may be compromised during treatment. More recent studies published since April 2022 until January 2024 also support the conclusions of this review.
PROSPERO REGISTRATION NUMBER
CRD42021289659.
PubMed: 38594047
DOI: 10.1136/archdischild-2023-326669 -
Hormone Research in Paediatrics Jan 2024Recently, numerous studies have addressed the long-term effects of treatment with gonadotropin-releasing hormone analogue (GnRHa) in patients with central precocious...
BACKGROUND
Recently, numerous studies have addressed the long-term effects of treatment with gonadotropin-releasing hormone analogue (GnRHa) in patients with central precocious puberty (CPP). However, the effects of GnRHa treatment on body mass index (BMI) in patients with CPP remain controversial.
OBJECTIVES
This systematic review and meta-analysis aimed to evaluate the association between GnRHa treatment and BMI in patients with CPP.
METHOD
A systematic search of databases, PubMed, EMBASE and Web of Science published before August 2021 identified relevant studies. The overall effect analysis was performed using STATA version statistical software 15.0.
RESULTS
The study included a total of 28 studies. At the end of GnRHa treatment, the BMI-standard deviation score (BMI-SDS) was greater than baseline BMI-SDS (weighted mean difference (WMD) = 0.14, 95% CI, 0.04-0.23; P = .004), especially in girls with CPP (WMD = 0.15, 95% CI, 0.05-0.25; P = 0.005) and in patients with normal weight (WMD = 0.34, 95% CI, 0.19-0.48, P < 0.001). After reaching adult height, BMI-SDS returned to baseline, suggesting that the effect of GnRHa treatment on BMI would disappear as the child grew (WMD = -0.03, 95% CI, -0.39 to 0.32; P = 0.815).
CONCLUSION
For patients with CPP, while treatment with GnRHa may increase the BMI in the short term after treatment, the BMI is likely to return to normal when the patients reach adult height.
PubMed: 38185120
DOI: 10.1159/000535132 -
The Experience of Menarche for Young People With Sensory and Motor Impairments: A Systematic Review.The Journal of Adolescent Health :... Feb 2024Young people with disabilities may face barriers to optimal management of menstrual health, including the management of menarche, a unique stage of development. This... (Review)
Review
Young people with disabilities may face barriers to optimal management of menstrual health, including the management of menarche, a unique stage of development. This systematic review examined the barriers to and facilitators of a positive experience of menarche for young people with visual, hearing, or mobility impairments. The MEDLINE, Embase, PsychINFO bibliographic databases, and grey literature were systematically searched in February 2022 to identify relevant research. Thematic synthesis of the extracted study findings was undertaken, using a team-based approach. A total of seven primary qualitative, and 1 mixed method studies were eligible for inclusion. The themes identified through metasynthesis were: timeliness of pre-menarcheal access, with many being unaware of menarche before it occurred, leading to fear and shock at their first period; resonance, with individuals reporting positive impacts of guidance from those with similar disabilities; and frame, the impact of surrounding societal narratives on the experience of menarche. Families, and those involved in the care of children with visual, hearing, and mobility impairments should be made aware of the importance of timely and resonant menarcheal support and guidance for these individuals and of broadcasting positive surrounding narratives of menarche. This should enable a positive experience of menarche for these vulnerable young people, supporting a good trajectory for later-life menstrual health.
Topics: Female; Humans; Fear; Menarche; Menstruation; Motor Disorders
PubMed: 37815768
DOI: 10.1016/j.jadohealth.2023.08.014 -
Current Medicinal Chemistry Jan 2024Kisspeptin was initially known as metastin for its role in suppressing metastasis in melanoma and breast cancer. Later, based on its ability to stimulate GPR54, its...
BACKGROUND
Kisspeptin was initially known as metastin for its role in suppressing metastasis in melanoma and breast cancer. Later, based on its ability to stimulate GPR54, its importance in maintaining an intact hypothalamic-pituitary-ovarian axis was recognised, which is the basis for the widespread application of the drug in several conditions such as secondary amenorrhea, regulation of puberty onset, ovarian function, trophoblast invasion, fertility regulation, parturition, and lactation. This systematic study aims to evaluate the current status of kisspentin in clinical trials.
METHODS
The keywords 'kisspeptin' or 'metastin' were used in the clinicaltrials.gov website and Clinical Trial Registry of India (CTRI) to find eligible clinical trials or records carried out without time constraints until February 26, 2023.
RESULTS
A total of 33 records were identified through clinical trial databases. All records were screened, and four trials were rejected as they failed to meet the inclusion criteria. Finally, 29 (87.9%) reports of interventional clinical trials with kisspeptin were reviewed.
CONCLUSION
Kisspeptin can be viewed as a multipurpose drug with considerably fewer side effects due to its effects simulating normal physiological processes in our body.
PubMed: 38265397
DOI: 10.2174/0109298673251224230919093656 -
Iranian Journal of Public Health Jan 2024Adolescent idiopathic scoliosis (AIS) is one of the most prevalent spinal abnormalities, appearing far into puberty. Schroth's exercises (SE) and core-stabilization... (Review)
Review
BACKGROUND
Adolescent idiopathic scoliosis (AIS) is one of the most prevalent spinal abnormalities, appearing far into puberty. Schroth's exercises (SE) and core-stabilization exercises (CE) have become very prominent among the specialized and general methods to scoliosis treatment. However, their effects are still unclear and there is insufficient evidence of superiority between them. Therefore, we aimed to evaluate and compare the effectiveness of these methods on Cobb angles (CA) in AIS.
METHODS
A systematic review was performed on SE and CE papers. The databases included PubMed, Scopus, MEDLINE, Cochrane Library, CINAHL, and Google Scholar from January 2005 to June 2022. The physiotherapy evidence database (PEDro) scale assessed the quality and provided internal validity and the statistical information of the papers.
RESULTS
After screening 410 papers, nine papers (SE=5, CE=3 and SE vs. CE=1), with 339 participants, were admitted for review (PEDro=6). Among them, three (SE=moderate evidence) and two papers (CE=limited evidence) reported clinically significant reductions in CA>5°, alone or compared to control or other exercise methods, while only one paper (very limited evidence) showed the relative superiority of SE (-7.16°) compared to CE (-3.27°). However, three papers did not show clinically significant results (CA<5°).
CONCLUSION
Moderate and limited evidence suggests that both SE and CE can effectively improve CA, respectively. There is very limited evidence to suggest that SE is more effective than CE in treating AIS. Thus, the definitive answers to the questions, especially concerning the superiority between these two methods, remained unclear. Further high-quality studies must be conducted in the future.
PubMed: 38694867
DOI: 10.18502/ijph.v53i1.14685 -
Archives of Disease in Childhood Apr 2024Clinical guidelines outline the use of hormones for masculinisation/feminisation in adolescents experiencing gender dysphoria or incongruence. Robust evidence concerning...
BACKGROUND
Clinical guidelines outline the use of hormones for masculinisation/feminisation in adolescents experiencing gender dysphoria or incongruence. Robust evidence concerning risks and benefits is lacking. There is a need to aggregate evidence as research becomes available.
AIM
Identify and synthesise studies assessing the outcomes of hormones for masculinisation/feminisation in adolescents experiencing gender dysphoria/incongruence.
METHODS
Systematic review and narrative synthesis. Database searches (MEDLINE, Embase, CINAHL, PsycINFO, Web of Science) were performed in April 2022, with results assessed independently by two reviewers. An adapted version of the Newcastle-Ottawa Scale for Cohort Studies was used to assess study quality. Moderate- and high-quality studies were synthesised.
RESULTS
12 cohort, 9 cross-sectional and 32 pre-post studies were included (n=53). One cohort study was high-quality. Other studies were moderate (n=33) and low-quality (n=19). Synthesis of high and moderate-quality studies showed consistent evidence demonstrating induction of puberty, although with varying feminising/masculinising effects. There was limited evidence regarding gender dysphoria, body satisfaction, psychosocial and cognitive outcomes, and fertility. Evidence from mainly pre-post studies with 12-month follow-up showed improvements in psychological outcomes. Inconsistent results were observed for height/growth, bone health and cardiometabolic effects. Most studies included adolescents who received puberty suppression, making it difficult to determine the effects of hormones alone.
CONCLUSIONS
There is a lack of high-quality research assessing the use of hormones in adolescents experiencing gender dysphoria/incongruence. Moderate-quality evidence suggests mental health may be improved during treatment, but robust study is still required. For other outcomes, no conclusions can be drawn. More recent studies published since April 2022 until January 2024 also support the conclusions of this review. CRD42021289659.
PubMed: 38594053
DOI: 10.1136/archdischild-2023-326670