Puberty in cystic fibrosis
Bracha Goldsweig1, Beth Kaminski2, Aniket Sidhaye3
1Division of Pediatric Endocrinology, Children's Hospital and Medical Center, 8200 Dodge Street, Omaha, NE 68114, USA.
Summary
Delayed puberty is less common in cystic fibrosis (CF) but still a risk for those with severe disease. Early evaluation is crucial for growth, bone health, and well-being in youth with CF.
Area of Science:
- Pediatric Endocrinology
- Cystic Fibrosis Research
- Adolescent Health
Background:
- Historically, delayed puberty was a hallmark of cystic fibrosis (CF).
- Recent data indicate normal pubertal progression in most individuals with CF.
- However, severe CF disease remains a risk factor for pubertal delay.
Purpose of the Study:
- To review the physiology of puberty.
- To discuss pubertal timing in individuals with CF.
- To outline the evaluation and management of delayed and precocious puberty in CF.
Main Methods:
- Literature review on puberty and CF.
- Analysis of current understanding of pubertal development in CF.
- Synthesis of diagnostic and management strategies.
Main Results:
- Pubertal timing in CF is variable, influenced by disease severity.
- Delayed puberty impacts linear growth, bone accrual, and psychosocial well-being.
- Precocious puberty also requires careful consideration in this population.
Conclusions:
- Careful monitoring of pubertal development is essential for children and adolescents with CF.
- Management strategies should address potential impacts on growth, bone health, and psychosocial factors.
- A comprehensive approach is needed for both delayed and precocious puberty in CF.
More Related Videos
Related Concept Videos
01:23Cystic Fibrosis: Pathogenesis
673
Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
673
01:24Cystic Fibrosis: Management
422
Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...
Sinus disease and chronic...
422
01:27Signs of Puberty
1.3K
Puberty is a critical phase, typically beginning between the ages of 8 and 13 in girls and 9 and 14 in boys, though timing can vary based on genetics, environmental factors, and overall health. This period is characterized by the development of secondary sexual characteristics and the attainment of reproductive potential. Endocrine changes underpin puberty, with hormonal surges of Luteinizing Hormone (LH) and Follicle-Stimulating Hormone (FSH) instigated by Gonadotropin-Releasing Hormone (GnRH)...
1.3K
01:15Development of the Sexual Organs in the Embryo and Fetus
3.1K
Development of the reproductive organs in an embryo starts from a bipotential state. This means the early embryo can develop either male or female reproductive organs. The formation of these organs begins with the growth of gonadal ridges that arise from the intermediate mesoderm during the fifth week of development.
Near the gonadal ridges, two duct systems are present: the mesonephric ducts (Wolffian ducts) and paramesonephric ducts (Müllerian ducts). These ducts form the basis for the...
Near the gonadal ridges, two duct systems are present: the mesonephric ducts (Wolffian ducts) and paramesonephric ducts (Müllerian ducts). These ducts form the basis for the...
3.1K
02:19The Y Chromosome Determines Maleness
7.8K
The Y chromosome is a sex chromosome found in several vertebrates and mammals, including humans. In addition to 22 pairs of autosomes, the human males have one X chromosome and one Y chromosome. In these organisms, the presence or absence of the Y chromosome determines the development of male traits.
Evolution
Around 300 million years ago, the two sex chromosomes diverged from two identical autosomal chromosomes. Over time, the Y chromosome has lost most of its genes, shrinking in size....
Evolution
Around 300 million years ago, the two sex chromosomes diverged from two identical autosomal chromosomes. Over time, the Y chromosome has lost most of its genes, shrinking in size....
7.8K
01:43Sex-linked Disorders
108.0K
Like autosomes, sex chromosomes contain a variety of genes necessary for normal body function. When a mutation in one of these genes results in biological deficits, the disorder is considered sex-linked.
108.0K