The renal agenesis was known to mankind since ancient times. Aristotle mentioned this pathology, saying that if the animal can not exist without a heart, then it is completely without a spleen or kidney. Then the Belgian scientist Andreas Vesaliy became interested in renal dysplasia during the Renaissance. And already in 1928 the Soviet doctor Sokolov began to actively detect the frequency of this disease in humans. Today, several different types of agenesis have been identified, each of which has its own symptoms and forecasts for later life.
Kidney anatomy
In various medical sources, it is often possible to find a mixture of two terms - agenesis and aplasia of the kidney. Let's make clear: agenesis is an innate, complete absence of one kidney( or two) in a person. In this case, in place of the absent organ, there are no rudiments of renal tissue, there can not be a ureter and even a part of it.
Aplasia is an underdeveloped kidney that is not capable of fully fulfilling its function. In this case, a part of the ureter tube may remain, even a whole ureter that simply blindly terminates at the top without connecting with any organ. Therefore, when one speaks of agenesis with the retention of the ureter, in fact, we are talking about aplasia.
There is no unified statistics of this renal pathology. It is known that all renal anomalies occupy 7-11% of all pathologies of the genitourinary system. Unilateral agenesis of the kidney is registered in one newborn baby from 1,000( some sources report a ratio of 1: 700).Two-sided( absence of both organs) - in 1 child from 4-10 thousand.
Causes of
A common opinion about the causes of agenesis in newborn babies does not exist until now. The genetic factor here does not matter, so this anomaly is called congenital. The kidneys in the embryo begin to form already from the 5th week and continue throughout the pregnancy, so it's impossible to unequivocally name the cause of the problem.
There are several proven causes that can trigger kidney malnutrition and the birth of a baby with aplasia or agenesis.
To such risk factors are:
- Postponed infectious( virus) diseases in the mother in the first trimester: rubella, severe influenza, etc.
- Diagnostic procedures during pregnancy using ionizing radiation.
- Diagnosis of "diabetes mellitus" in a future mother.
- Admission of hormonal contraceptives during the bearing of a child( and also any potent drugs without the supervision of a doctor).
- Chronic alcoholism and drinking during pregnancy.
- The presence of sexually transmitted diseases in the patient.
Clinical picture
In the case of agenesis of one kidney, a child's disease may not appear for several years. If during the pregnancy of the mother there were no ultrasound, and after birth - various tests, the patient can learn about his features years later. For example, when examining at school or clinic at work.
On agenesis of the kidney in a newborn can indicate the following signs:
- facial malformations( wide and flat nose, eye hypertelorism, puffiness);
- ears are located low and strongly curved( usually on the side where there is no kidney);
- on the body an excessive number of wrinkles;
- large tummy;
- pulmonary hypoplasia( small volume of respiratory organs);
- deformation of the feet;
- displacement of the position of internal organs.
Subsequently, children may experience vomiting, polyuria, hypertension, develop dehydration.
When children are born with agenesis, that is, one ureter is missing completely, this is often accompanied by anomalies in the development of the genital organs. In girls, these are malformations of the uterus( unicorn, bicorne, uterine hypoplasia), atresia of the vagina, etc. In boys, there is no vas deferens, pathological changes in seminal vesicles. In the future, this can lead to pain in the groin, painful ejaculation, impotence and infertility.
Types of
There are different classifications of kidney agenesis, the first and the main one - by the number of organs. The following forms are distinguished here:
Two-sided agenesis, that is, the absence of both kidneys. This kind of anomaly is incompatible with life. If ultrasound shows bilateral agenesis even in late pregnancy, doctors often insist on artificial delivery.
Agenesis of the right kidney is the main type of pathology, and in women it is registered more often. The right kidney is usually slightly smaller and more mobile from birth than the right kidney, it is located below the left one, therefore it is considered especially vulnerable. In most cases, the function of the right organ is successfully carried out by the left kidney, and the person does not experience any discomfort.
Agenesis of the left kidney. This form of renal anomaly is much less common and is more difficult to tolerate. The right kidney is inherently less mobile, less functional and not adapted to a full compensatory function. Symptoms for this type of disease are classical for agenesis and aplasia.
In addition, the forms of agenesis are distinguished:
- unilateral with preservation of the ureter;
- unilateral with absence of ureter.
Unilateral agenesis( left and right), in which a part of the ureter or a whole organ is preserved, practically does not pose a threat to a full life. If the second kidney completely fulfills the functions of non-existent, and a person leads a healthy lifestyle, the disease will not bother him. Often in such cases, people learn about their characteristics by chance, during a comprehensive survey.
If agenesis of one kidney is accompanied by a loss of the ureter, this in most cases is fraught with anomalies of the genital organs. In boys, it is an underdeveloped lung, the vas deferens. At girls - various pathologies of development of a uterus and a vagina.
Treatment and forecasts
The only type of agenesis that is truly dangerous for life is two-way. A fetus with this diagnosis is either born already dead, or dies during childbirth, or on the first day after birth. Because of rapidly developing renal failure.
Now, thanks to the rapid development of the possibilities of perinatal medicine, there is an opportunity to save the child's life if in the first hours after childbirth to undergo transplantation and then regularly do hemodialysis. But this is realistic only if we organize a rapid differential diagnosis of the pathology of the fetus, in order to distinguish agenesis from other defects of the urinary system.
With unilateral agenesis, the prognosis is usually the most favorable. If the patient does not experience any unpleasant symptoms, he should do blood and urine tests once a year and protect the kidney, which already carries a double burden. That is, do not overcool, do not take alcohol, avoid excessively salty foods, etc. For each patient, there may also be individual recommendations of an observant physician.
In rare cases, with kidney agenesis, a disability is registered. This occurs when chronic renal failure( CRF) of stage 2-A develops.
This stage is called polyuric, or compensated. In this case, the only kidney can not cope with the load, and its work is compensated for by the activity of the liver and other organs. The disability group depends on the severity of the patient's condition.