Home | RSS

Parenting Guide
WebMD Health Information
Health and Biology

Ambiguous Genitalia Wiki, causes and pictures

Aug 07, 2011 > Various
Are you looking for ambiguous genitalia wiki and pictures? The first concept that an examiner might want to know if they were asking the question - or if you were approached with the clinical thing - is that there are many different levels of sex, and not just chromosomes. We all tend to focus on chromosomes but thatís just one. Gonads, ovaries or testes? What are the internal ducts? What do the external genitals look like? What is the hormonal sex; androgen or estrogen circulating? And what is the psychosocial sex? All these things must be consistent in order to have a successful outcome of sex assignment.

Ambiguous Genitalia Wiki and pictures

Starting with chromosomal sex, since thatís the thing we often think about first when we see a baby who has ambiguous genitalia. Let me tell you, the chromosomes wonít tell you everything. They arenít always perfect predictors of what the sex of the child is. I think one obvious example is that a karyotype only sees big changes. You might not see the missing piece that will turn an XY person into a perfectly normal looking female. You may not see extra pieces that will turn an XX person into a very normal looking male. What about the concept that if some of the cells are one type - say 45 X - and the other types are 46 XY, what actually happens. And thatís an important concept of mosaicism. Iím not sure they will ask you this on the Boards but in real life you have to remember that you can get mixes of chromosomes, and perhaps that explains some of the cases that we see that are very, very much in between. Now we do a lot of focusing on the gonadal sex. That is, is it an ovary or a testes? But guess what? Itís not always clear. While it normally follows the rules that if you have a Y chromosome you get a testes. Sometimes you do get a little of both. It may have ovarian and testicular tissue in the same gonad. You can imagine how that confuses issues. Sometimes the gonad is missing. It either never developed in the first place or else it was there and then it disappeared. Sometimes it just doesnít make the hormones it is expected to, even though itís there.

Ambiguous Genitalia Wiki, causes and pictures Now the third thing - and I think this is very important for diagnostic purposes - are the internal genital ducts, the tubing as you would have it. I donít know, maybe you have your mnemonic. I just remember that M stands for mother and W stands for wolf-whistle, to remember which ones are the "female" and which ones are the "male" structures. But really the thing that I think is important is that there are two mullerian structures that are diagnostically very very important, and I put them in orange. And one of them is the uterus. If you see a uterus on the ultrasound, there was something there that a male normally makes that wasnít made. If you see the upper two-thirds of the vagina is there, you know that that male factor wasnít active, that keeps that from being made. So itís very important because these are structures you can see on ultrasound, in the case of the uterus, or on your physical exam, if you do a pelvic exam.

Ambiguous Genitalia Causes

Now the thing that we see in the delivery room, the thing that I think really leads to a lot of the distress, is the external genitalia, the ambiguity of the external genitalia. The rule is - and this is what an examiner might try to ask a question, to see if you understand - is that for every female genital structure there is a male equivalent, and vice-versa. So the clitoris is the analog of the penis. On the other hand, sometimes you have something and you donít know whether itís a large clitoris or a small penis. There are also other analogs as well. The labia minora in a male would form a tube around the urethra and recall the corpus spongiosum. The labia majora would fuse in a male to become a scrotum. And the lower third of the vagina is a prostatic utricle. Thatís not as useful, but these other things are very very important to know. And in between you can get things like hypospadias, or you can get posterior fusion and you donít know if itís fused labia majora or actually a partially fused scrotum. Just the idea that all these things can be in between - and Iíll show you some clinical examples in just a little bit. Now there is obviously the idea of do you have estrogens or androgens in your system? And does your body respond? Iíll show you an example of when the body doesnít respond to androgens, you get a female outcome. What they call the androgen insensitivity syndrome.

There is also the psychosocial aspect and I wonít get into that now, but from a management point of view, this is very important; that whatever decision you finally make on a sex assignment case, that the family, the physicians, everybody is firmly on that same wave length.

So really the key concept, number two -which is just a corollary of concept number one - is that many of these different levels may be discordant. They donít always follow together. For example, if you have a question that asks, "Here is somebody with a uterus, do they definitely have a 46 XX karyotype?" I think you probably know the answer. Definitely no, they may not. Hereís another classic example. Somebody has well developed breasts, normal female genitalia, 100% female identity, does this automatically guarantee they have a 46 XX karyotype? Again, not necessarily. If they have primary amenorrhea, a blind short vagina, and no axillary or pubic hair, they may very well be 46 XY but they didnít respond to testosterone. In many cases this is the classic example of androgen insensitivity. Where somebody who looks like this turns out to have a hernia with a testes in place.

An important key concept, pathophysiologically, is that the default pathway for sexual differentiation is female. So that if you donít have any ovaries - if you have a fetus who has no gonads whatsoever - the default pathway will be female duct and genital development. On the other hand, the testes has to be there to suppress those mullerian ducts, those female ducts like the uterus and upper part of the vagina, and two, to stimulate the development of male genitals. So to make a male you have to have the right chromosomal signal, usually on the Y chromosome. You have to have two signals from the testes; one, the mullerian inhibiting substance to inhibit the female ducts, and two, testosterone to stimulate the male genitalia formation. You need to be able to convert testosterone one final step to dihydrotestosterone that finishes off the genital development, and you do also need pituitary signals to insure that the penis is full length, for example.

There is an important concept medically, that there is a critical period for external genitalia development and this is week 8-12. After the second month. Before that the male and female genitalia in the fetus look exactly alike. Between 8 and 12 weeks any androgen - whether itís something the mother took, whether itís a tumor the mother has, or whether itís an adrenal problem that gives the mother too much testosterone - will lead to full virilization, where if you had somebody who was a 46 XX female and was exposed to a lot of androgen in this critical period, they would end up looking quite male. After 12 weeks, the fetus is no longer quite as sensitive and while youíll get some virilization, you wonít get the extreme ambiguity you would at the earlier period.

You need to know what are some of the signs of virilization. Clitoromegaly is one. But I will tell you that there are standards, but itís very hard to measure a clitoris and itís sort of like the Supreme Court definition of pornography, "you know when you see it." Now I want to warn you that the clitoris of a premature infant can look very prominent because there isnít a lot of labial fat on the sides, so it looks like the clitoris is sticking out there. But I think most of us would realize that this is not clitoromegaly. But once in a while we get some calls because someone thinks the clitoris looks very prominent. Another thing that you have to be careful of; this is a normal female infant. Her labia minora are very stimulated, perhaps because of her motherís hormones, and this will go down as the infant grows up. This is not clitoromegaly. These are just very stimulated, estrogen-stimulated labia minora.

Another thing that is important is that if you see a migration of the urethral opening to the shaft of what would be the clitoris - and the urethral opening in a male should be at the tip of the corona, and in a female is down at the base of the phallic structure, but it could be anywhere in between depending on how much androgen the person has. Another very important feature for virilization is any kind of posterior fusion at the labia. One thing you can look at is see where the fusion is, or see where the posterior part of the vaginal opening, and see if itís closer to the base of the clitoris than it is to the anus. If it is, thatís a sign of significant posterior fusion and maybe a sign of virilization in a female.

You have to know the converse too. What does an under-virilized male look like? Obviously a small penis is one thing, and you can remember the one inch rule as a rule of thumb for a term newborn. A penis that is less than one inch in length is more than 2 Ĺ standard deviations below the mean. That should be easy to remember, hopefully. Obviously hypospadias can be a sign of under-virilization, although it may happen sporadically with no hormone problems. And another thing thatís come up - actually very recently we had a case - where a child may have a bifid scrotum. Iíll show you a picture of each of these in a second. I donít think any of us need to be told that this is a micropenis. You do need to push the suprapubic fat pad back to make sure that you are getting the full length of the penis, and you do need to measure the stretched penile length. Not just look at it and try to estimate. The hypospadias, or mild hypospadias, may be a sign of under-virilization. Certainly if the urethra is down here near the base of the penis, thatís a very distinct sign of under-virilization and should increase your level of suspicion for an androgen disorder.

Here is an example Ö this is a bifid scrotum where you actually have some fusion in the midline right here, and that can be a sign of under-virilization as well.

Now any exposure to excess androgens during that critical 8-12 week period will lead to virilization and the most common thing that you will run into, and have a very good chance of running into - particularly if you deal with a Hispanic population or you live in New York City - and that would be virilizing CAH, or congenital adrenal hyperplasia. And you should know this entity relatively well. Iíll cover that in a second. Obviously if mother had an androgen-excreting tumor and she herself was virilized, or if she were taking any androgenic meds, that would be a smoking gun as well. But much less common in our practice.

That's some stuff about Ambiguous Genitalia Wiki and pictures

Other Articles

Ambiguous Genitalia Wiki, causes and pictures #0Apnea in children and adults
Apnea is strictly defined as the absence of breathing. The term is also used to refer to an interruption in breathing that occurs in some premature babies and also during the sleep of some children and adults. Apnea in infants The usual cause of apnea in infants is immaturity of the brain centers that regulate breathing. From time to time, the infant suddenly stops breathing completely and turns blue. If the baby is stimulated in some way (for example, by a flick of the finger on the bottom of the foot), he or she will usually start breathing normally at once. Seldom is it necessary to use first-aid measures or a mechanical respirator to restart breathing. However, because a history of apnea is one of the factors that may be associated with sudden infant death syndrome (SIDS), physicians sometimes recommend monitoring high-risk infants with equipment that sounds an alarm if any stoppage of breathing is detected. In some cases, the tendency toward apnea disappears a few week after birth, when the breathing control centers have matured.
Ambiguous Genitalia Wiki, causes and pictures #1Myelodysplastic Syndrome Treatment Guidelines
What is Myelodysplastic syndromes? Myelodysplastic syndromes (MDS) is a term for a group of disorders that cause the bone marrow to produce an inadequate number of healthy blood cells ó red blood cells, white blood cells and platelets. In MDS, cells in the bone marrow responsible for making blood cells (stem cells) don't mature, don't make enough blood cells or make defective cells. Myelodysplastic Syndrome Treatment The prognosis depends on the following: Whether the myelodysplastic syndrome occurred after chemotherapy or radiation therapy for another disease. The number of blast cells in the bone marrow. Whether one or more types of blood cells are affected. Certain changes in the chromosomes. Treatment options depend on the following: Whether the myelodysplastic syndrome occurred after chemotherapy or radiation therapy for another disease. Whether the myelodysplastic syndrome has progressed after being treated. The age and general health of the patient.
Ambiguous Genitalia Wiki, causes and pictures #2Miranda Kerr Diet Plan and Exercise
Supermodel and new mum Miranda Kerr has revealed how she maintains her slender figure and glowing vitality. Writing on her KoraOrganics blog, the Victoria's Secret model and mother of two-month old Flynn, attributes her perfect figure to the Blood Type diet and a healthy dose of supplements to maximise her daily nutrition intake.
Ambiguous Genitalia Wiki, causes and pictures #3Abdominal Pain Symptoms and Treatment
A common primary care problem, especially among females, that is most oftenbenign but can be related to serious diagnoses. Functional pain is the most common recurrent form of pain. Generally a diagnosis of exclusion, it is defined as nonorganic pain related to everyday stress. School, peer, and family problems are commonly associated stressors. Abdominal pain can be related to school absenteeism and may be a sign of depression.
Ambiguous Genitalia Wiki, causes and pictures #4Symptoms Ovarian Cancer
Are you looking for article about symptoms ovarian cancer? Here is good stuff about ovarian cancer symptoms. The 5-year survival rate for ovarian cancer by stage is not significantly different from the 5-year survival rate for other gynecologic cancers; however, there is a significant difference in stages, with ovarian cancer usually having spread into the abdomen in about two thirds of patients at the time of diagnosis. It is clear from these data that the single most important factor in the large number of deaths from ovarian cancer is the failure to diagnosis the disease at an early stage. The reasons for this failure correspond to the growth and spread patterns of the disease. Because the ovary floats freely in the pelvic cavity, a tumor can grow for some time without producing symptoms associated with involvement of, or pressure on, other organs.
Ambiguous Genitalia Wiki, causes and pictures #5Enlarged Heart Treatment
Enlarged heart Definition Enlarged heart (cardiomegaly) isn't a disease, but rather a symptom of another condition.
Ambiguous Genitalia Wiki, causes and pictures #6Chicken Pox Causes, Symptoms, Complications, Treatment and Prevention
Chicken pox is an extremely contagious disease that is characterized by a blistery rash. It occurs most frequently in children between the ages of five and eight; less than 20 percent of the cases in the United States affect people over 15 years old. Chicken pox is transmitted so easily that most everyone gets the disease at some time. Chicken Pox Causes Chicken pox is caused by infection with the varicella zoster virus. Chicken pox is contracted by touching an infected personís blisters or anything that has been contaminated by contact with them. The virus also thought by some researchers to be air borne, since it may be caught from an infected person before the rash develops. Another way to get chicken pox is by exposure to shingles, a localized rash caused by the same virus. The incubation period (the time between exposure to the illness and the appearance of symptoms) of chicken pox is 10 to 21 days. It is contagious for about six to eight days after day rash appears or until all of the blisters have dried out.
Ambiguous Genitalia Wiki, causes and pictures #7Transient ischemic attacks Symptoms and Treatment
Transient ischemic attacks (TIAs) are neurogical deficits (such as loss of vision in one eye, inability to speak, paralysis, or weakness of one side of the body) of sudden onset that last for less than 24 hours. Although they are symptomatically similar to minor strokes, they do no discernible lasting damage to brain function. The major importance of TIAs is their role as a predictor of stroke. When a person describes a ďlight strokeĒ, it is often a TIA that is meant. If left untreated, TIAs can indeed lead to major strokes, with permanent damage to all parts affected.
Ambiguous Genitalia Wiki, causes and pictures #8Heart Attack Symptoms Warning Signs
As the atherosclerotic plaques enlarge, they become cracked and ulcerated. The clotting factors in the blood may treat this ulceration like a cut and form a clot over the plaque, completely blocking the artery. When a branch of a coronary artery becomes totally obstructed, the heart cells that it supplies die, resulting in a heart attack. Heart attack is medically referred to as a myocardial infarction, meaning death of the heart muscle. Although clot formation over an ulcerated plaque is believed to cause the majority of heart attacks, others are caused when a piece of plaque or clot breaks off, obstructing the artery below the actual site of atherosclerosis. A few may be caused when the diseased artery contracts because of irritation from the plaque. About 1.5 million persons have a heart attack in the United States each year.
arteries pictures&h=150&w=200&q=100Arteries of the Body Function and Pictures
Arteries facts Arteries are vessels that conduct blood away from the heart Arteries transport oxygenated blood under pressure to the cells of the body. (The pulmonary artery is an exception; it transports deoxygenated blood to the lungs) The arterial wall consists of three primary layers: The innermost layer consists of simple squamous epithelium (the endothelium) surrounded by a connective tissue basement membrane with elastic fibers.The middle layer consists mainly of smooth muscle and is usually the thickest layer. It  not only provide support for the vessel but also changes vessel diameter to regulate blood flow and blood pressure.The outermost layer, which attaches the vessel to the surrounding tissue, consists of connective tissue with varying amounts of elastic and collagen fibers.
Ambiguous Genitalia Wiki, causes and pictures #10Angina pectoris Sympstoms and Treatment
Angina pectoris is a symptom of inadequate blood flow through the coronary arteries to the heart. It means chest pain (from the Latin angere meaning choke and pectoralis meaning chest). Angina pectoris occurs when some part of the heart muscle is not getting enough blood, usually during times of exertion or stress. At such times, the heart must work harder and faster. If one or more of the heartís arteries is narrowed by disease, the extra blood and oxygen required to nourish the heart cannot reach a region of the heart muscle. Angina pain is a signal that muscle cells are being strained by an insufficiency of oxygen; they are, in effect, gasping for air. The attacks are usually brief, lasting only a matter of minutes, and they normally stop when the person rests. Angina pectoris seldom causes permanent damage to the heart muscle.
Ambiguous Genitalia Wiki, causes and pictures #11Diabetes Mellitus Treatment
Diabetes mellitus is a chronic disease which is difficult to cure. Management concentrates on keeping blood sugar levels as close to normal ("euglycemia") as possible without presenting undue patient danger. This can usually be with close dietary management, exercise, and use of appropriate medications (insulin only in the case of type 1 diabetes mellitus. Oral medications may be used in the case of type 2 diabetes, as well as insulin).
Ambiguous Genitalia Wiki, causes and pictures #12The kind diet recipes and review
Like many celebrities before her, actress Alicia Silverstone has put together a book that she says reveals the secrets of her great looks and knockout figure. In The Kind Diet, Silverstone attributes her health and beauty to her totally organic, vegan diet, free of "nasty" meat, dairy, white sugar, and processed foods. In The Kind Diet, Silverstone, a passionate animal activist and conservationist, says a plant-based diet is not only good for your body but also saves animals and is one of the "greenest" things you can do. here is some review for The kind diet.

Copyright (c) 2011 by DRZ
Privacy Policy | Contact Us