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Going to a Cleveland Clinic location? Disorders of sexual differentiation are rare congenital conditions in which a baby is born with either both male and female reproductive organs, atypical sex chromosomes or atypical appearances to their genitals. In such cases, it is not always possible to tell right away differentiate whether the baby is a boy or a girl.
In the past, disorders of sex differentiation were given names such as intersex or hermaphroditism. That leaves the sex asment unclear or a mixture of both male and female. There are different types of DSD and each type has a different cause. The most common DSD types in children include:. The tissue that eventually turns into testes or ovaries is present early in fetal development. Sexual organ development is influenced by genetics chromosomeshormones, and environmental factors.
The cause of a DSD is not always known. The symptoms of a DSD depend on the type of condition. In general, genitals that are not typical indicate a DSD. However, a DSD can sometimes be found only internally. Some DSDs are discovered in the infant at birth because of noticeable s, such as undescended testicles or an abnormal or unclear genital.
When that happens, the doctor may Atypical sex mature a few tests to confirm the diagnosis. The tests may also help the doctor to determine if there are any other medical problems that may need to be treated immediately. In other cases, DSDs may not be noticed early because there may be no obvious s. Parents may start to have concerns only when they do not notice any puberty changes in an older. Some children may mature into adulthood with a DSD that neither doctors nor parents noticed. Treatment of with DSD should start by assuring the family that their child will likely become a well-adjusted, functional member of society.
It is important to diagnose the type of DSD correctly as soon as possible to advise the parents appropriately. When the test are back, specialists will explain the to you, and discuss whether you should raise your child as a boy or a girl. Most children do not need emergency treatment for a DSD. DSD is best managed by team of healthcare providers. The specialists may include a pediatric urologist, a psychiatrist, and a pediatric endocrinologist, in addition to the primary care doctor.
The team will help the parents, and possibly the child, to decide among treatments that may include medications, hormones, and surgery. The decisions may include postponing treatment until is able to provide his or her input.
Most adults with a DSD decide to stay with the sex that they were ased as babies. The person with a DSD might decide to change genders if the original asment does not seem to be correct. It is possible that an adult with DSD will be infertile.
He or she might need hormone therapy and psychological support. Sometimes a DSD may not be noticed at birth. You should talk to your healthcare provider if your child does not experience the expected changes at puberty, such as hair growth, or menstruation. If your concerns are confirmed, a team of healthcare specialists will work closely with you and your. Most likely, your child may need hormone therapy and psychological support.
At that point, your child may decide whether he or she wants to change physical appearance via surgery. Getting the right care and support will help the child understand DSD and live well. It may not be easy to remember all your questions when the doctor discusses the treatment plan for your. It may be helpful to write down all the questions to make sure not to leave out important concerns.
Those questions may include the following:. If you are the parent of who has a DSD, or you yourself have a DSD, it may be a good idea to contact or support groups. Ask your healthcare team to provide you with a list of national Atypical sex mature local support groups. Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission.
We do not endorse non-Cleveland Clinic products or services. Disorders of Sex Differentiation Disorders of sexual differentiation occur when a baby is born with both male and female sex organs. What are the types of disorders of sex differentiation DSD? Genitals may be male or ambiguous not having a clear sex. The child may have an enlarged clitoris a female sex organ that looks like a penis. The lower section of the vagina may be closed. with CAH does not have a specific enzyme that the body needs to make the hormones cortisol and aldosterone. Without these two hormones, the body produces more androgens male sex hormones.
If the affected child is female, the high androgen levels before birth cause the genitals to become more male in appearance. This condition can cause serious health issues later on, such as life-threatening kidney problems that need to be treated as soon as possible. Female with male chromosomes 46, XY DSD : Some female children have male chromosomes XY but their external genitals may appear entirely female or unclear.
In addition, the womb may or may not be present. The testicles may be absent or not properly formed. Several different causes are behind this condition. Androgen insensitivity syndrome AIS is one possible cause. With AIS, the body either ignores androgens or is not sensitive to them. For this reason, the child appears to be female. The testes usually remain inside the body and the womb does not develop.
Mixed genitals and sex organs 46, XX Ovotesticular : This is a Atypical sex mature rare type of DSD where the child has tissue from both ovaries and testicles. The genitals may appear female, male, or a mix of both. Children with this type of DSD have female chromosomes.
Although the cause of this condition is not known, some cases have been linked to genetic material normally found on the Y chromosome that is misplaced on the X chromosome.
Sex chromosome DSD : Some children have neither male nor female chromosomes. Their sex organs are, however, normally formed as either male or female. Those children may not go through normal sexual development at puberty. For example, with female sex organs may not start having periods, and may have small breasts.
Rokitansky syndrome : Some females are born without a womb, cervix, and upper vagina. Some may have underdeveloped organs. In this condition, the ovaries and external genitalia vulva are still present. They will still develop breasts and pubic hair as they get older. The cause of Rokitansky syndrome is not clear. Girls and women with this condition have normal XX chromosomes. The first of Rokitansky syndrome is that a girl does not start having periods. Sex may also be difficult because the vagina is shorter than normal. Women with Rokitansky syndrome who have no womb cannot become pregnant.
It is sometimes possible to take eggs from the individual to make a surrogate pregnant. What are the symptoms of a disorder of sex differentiation DSD? What questions should I ask the doctor? Those questions may include the following: What kind of experience do you have treating children with DSDs? Should I talk to my child about this condition? Is there a Atypical sex mature care plan? Will be my child live a normal life? Will the treatment affect how my child feels in the long run? Resources How can I get more help? Show More.Atypical sex mature
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Disorders of Sex Differentiation