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Renal agenesis is ten times less common than pyelonephritis or hydronephrosis, but the pathology causes discomfort to the patient. The vice appears during embryonic development, lies in the absence of one bean-shaped organ or two natural filters in the fetus.

To prevent a serious defect, it is important to know what factors increase the risk of renal agenesis. Doctors explain how a congenital defect occurs and what to do if a child has only one kidney. Factors provoking defeat urinary tract embryo, methods of treatment and prevention of agenesis, possible complications are described in the article.

General information

Kidney aplasia is the absence of one or both natural filters in the body. Without bean-shaped organs, it is impossible to remove fluid and breakdown products, when accumulated toxic substances above permissible limits, severe intoxication and death occur.

It is important to remember the functions of the kidneys:

produce urine, with the help of which decay products, toxins, drug residues, and other harmful substances are removed from the body;
produce important hormones: renin, erythropoietin;
produce calcitriol for optimal absorption of phosphorus and calcium from the gastrointestinal tract;
maintain electrolytic balance.

The absence of one organ increases the load on the remaining natural filter, but if a number of conditions are met, a person can live and work relatively comfortably. Many patients complain that unpleasant symptoms periodically occur, but in general the organ copes with excretory, excretory, and endocrine functions. Restrictions apply physical activity, nutrition, taking medications, but severe complications do not occur unless you violate the rules prescribed by your doctor. It is important to regularly visit a nephrologist, have your urine tested, and have your urine tested.

Reasons for the development of pathology

Congenital defect develops under the influence negative factors during the formation of organs. A child with anomalies of natural filters is born due to a combination of environmental and genetic factors, and when a woman violates the rules that preserve the health of the fetus. Doctors often identify a combination negative factors, which increases the risk of anomalies.

Causes of congenital absence of one or two kidneys:

the influence of poisons and toxins;
transferred viral infections during pregnancy;
hormonal imbalance;
venereal diseases. Syphilis is most dangerous for the fetus;
hereditary predisposition;
drug and alcohol use by the expectant mother;
endocrine pathologies, diabetes mellitus;
exposure to high background radiation for the fruit.

Renal agenesis ICD code - 10 - Q60.

Types and forms of the disease

The severity of congenital pathology depends on the preservation of elements of the urinary system. The greater the tissue damage, the lower the likelihood of ensuring the process of cleansing the body of toxins.

Unilateral (ureter preserved)

Congenital defect of the first clinical type. With a unilateral type of pathology, the remaining kidney functions with double the load, and hyperplasia of the organ is often observed. Increasing the number of elements that ensure the accumulation, filtration and excretion of urine allows for the timely removal of toxins, poisons, and nitrogenous breakdown products from the body. Injury to the only natural filter leads to serious problems with blood purification.

Unilateral (no ureter)

The anomaly develops on early stages formation of the urinary tract in the embryo. There is no ureteral orifice in this type of pathology; the male fetus also lacks a duct intended for the introduction of seminal fluid. Additionally, negative changes occur in the seminal vesicles.

Anatomical disorders lead to unpleasant symptoms and sexual dysfunction. Men feel pain in the sacrum and groin area. Discomfort also occurs during ejaculation. The combination of negative factors negatively affects men's health and the ability to conceive.

Bilateral agenesis

Dangerous vice intrauterine development, third clinical type. The most common birth observed dead child. In some cases, a baby was born alive, but death occurred within the first few days.

Thanks to medical advances, it is possible to perform a kidney transplant in a newborn to cleanse the body. Important points: quick selection donor organ, with which difficulties arise, timely diagnosis of the defect. Doctors should determine within the first 24 hours whether the baby has bilateral renal aplasia or other urinary tract abnormalities.

Diagnostics

Modern methods of examining a pregnant woman make it possible to identify many abnormalities in the development of the unborn child. IN mandatory an ultrasound is prescribed for different dates to control fetal development. Standard ultrasound examination gives an accurate picture of the condition of the urinary tract.

If an anomaly is detected, they are additionally prescribed, and. If one of the bean-shaped organs is not visible on the monitor during the scan, doctors suspect unilateral renal aplasia.

To analyze the functioning of the bean-shaped organs in the fetus, during the scan, doctors measure the volume of amniotic fluid in which the unborn child is located. Aquatic environment reduces risk traumatic injuries of a growing organism, allows the lungs to mature. Oligohydramnios (lack of the required volume of amniotic fluid) often develops when one or two bean-shaped organs are malfunctioning. TO dangerous consequences oligohydramnios can be attributed to damage to the lungs, which cannot form fully.

Characteristic signs and symptoms

Bilateral renal aplasia can be suspected based on several additional signs:

development arterial hypertension;
a sharp increase in the volume of urine excreted;

Potter syndrome - that's what it's called characteristic features, indicating pathological process during the formation of bean-shaped organs. A group of defects develops in the absence of urine or a small amount of fluid in the embryonic kidneys. Instead of amniotic fluid with a balanced composition amniotic fluid contains a high percentage of urine.

External signs of agenesis of bean-shaped organs:

the ears are deformed and are below the usual border;
the fetus has a flat, wide nose;
the frontal lobes protrude noticeably;
face puffy;
hypoplasia of the lungs is observed;
lower limbs are deformed;
hypertelorism of the eyes appears;
an epicanthus appears - a special fold of skin covering the lacrimal tubercle;
the skin is folded.

In the first days, newborns experience:

dehydration;
renal failure;
high blood pressure;
vomit.

Note! Unilateral agenesis of natural filters does not have characteristic external signs, as with bilateral anomalies; most often the pathology is detected by chance.

General rules and methods of treatment

Normally, a person should have two natural filters. In the absence of one kidney, transplantation of the missing element of the urinary system is required. The patient takes medications to prevent inflammation, improve urine outflow, and reduce the load on the renal glomeruli. In most cases, herbal formulations are prescribed.

If the queue for an organ transplant has not yet come, then with unilateral agenesis you can live without serious problems, if you follow the recommendations given by the nephrologist. Any violation of the rules quickly provokes damage to the kidney tissue, deterioration of health, and intoxication of the body. In severe cases, renal failure develops and death is possible.

Renal agenesis in adults and children

In the presence of one kidney, the bean-shaped organ increases in size: it is necessary to work with double load to ensure all functions. Increased stress often provokes disruption of processes in the natural filter, increased blood pressure, fluid retention in the body, and the development of swelling. Sometimes blood appears in the urine and in the background inflammatory process in the surviving kidney.

Patients diagnosed with renal agenesis are registered at a medical institution. The nephrologist monitors the patient’s condition and periodically prescribes ultrasound examinations of organs genitourinary system, a blood test to monitor the functioning of the surviving bean-shaped organ.

The patient receives recommendations on dietary nutrition, dosing of physical activity, drinking regimen. At the first signs of the inflammatory process, the provoking factor is eliminated and prescribed antibacterial therapy, intake of herbal compositions that improve the outflow of urine.

What is cystic and how to treat the disease? We have the answer!

Treatment methods for stenosis renal arteries And arterial hypertension described on the page.

Go to the address and find out about the norm and reasons for the deviation of red blood cells in the urine during pregnancy.

Helpful Tips:

Excessive loads of any kind should not be allowed: damage to a single kidney worsens the process of blood purification, leading to intoxication due to the accumulation of decay products;
it is important not to overuse medications;
you need to eat right;
you cannot lift weights;
to maintain the health of your only kidney, you will have to give up alcohol, smoking, and working in hazardous industries;
Kidney agenesis in children requires regular visits to a nephrologist. Kidney aplasia in newborns negatively affects the condition of the child while the body is not yet strong. You will need increased attention to drinking regime, nutrition, rest and physical development baby.

When identifying a congenital defect of the bean-shaped organs or the absence of one kidney, it is important to tune in to the positive development of the baby after birth, learn more information about aplasia and methods of preventing complications. Doctors give many examples where, even with one natural filter, patients led an active lifestyle. Required condition To maintain health, strictly comply with the requirements of the nephrologist.

In combination with other pathologies of the genitourinary system of the body, kidney agenesis can occur.

This is a rare congenital disease characterized by the absence of an organ, either one or two kidneys at the same time.

About pathology

Kidney agenesis occurs in practice in 7-11 percent of cases of all pathologies of the genitourinary system.

Cases when one organ is missing in a newborn occur 1 time in 1000, but bilateral renal agenesis is recorded even less often 1 time in 4000-10000.

When a child is born with one kidney, there may be serious complications. All functions will be transferred to healthy organ.

He, in turn, most often has an increased scale, since he is forced to cope with a large number loads.

According to these statistics, organ agenesis is most often diagnosed in male newborns.

Complications of the pathology usually include urolithiasis, arterial hypertension or pyelonephritis.

About the factors provoking the development of agenesis

To date, scientists have still not been able to establish what the true causes of the pathology actually are.

But thanks to a number of studies, it was found that there are certain factors that contribute to the development of agenesis.

Very often, renal agenesis can be triggered by an anomaly that can develop during intrauterine formation. This may be due to exogenous factors.

First of all, doctors note that if there is a genetic predisposition to this pathology in the family, then the risks of it being diagnosed in newborns are very high. Those. Heredity plays a big role.

During pregnancy, very unfavorable conditions can also be created that trigger the development of pathology. These include cases:

the course of infectious diseases (flu, rubella in a pregnant woman in the first 3 months);
improper intake of hormones;
irradiation;
presence of diabetes mellitus and other malfunctions endocrine system pregnant women, as well as sexually transmitted pathologies. Syphilis is especially dangerous;
drug, alcohol and tobacco abuse.

If there is at least one of the above problems, then the expectant mother must undergo an ultrasound scan to understand whether there are risks of developing agenesis.

Diagnostic measures

To correctly diagnose whether a child has agenesis of the right kidney or the left organ, they resort not only to special instrumental methods, but also to some other nuances of the inspection.

The doctor carefully examines the child. If he has agenesis of the right kidney or the left, then the disease will be reflected in external signs, making itself felt in facial defects.

The baby may be too flat or very wide nose. You may also notice puffiness or ocular hypertelorism on the face.

The specialist must pay attention to the localization of the ear concha. If the position is too low or strongly curved, this indicates that a possible diagnosis is agenesis of the right kidney or the absence of the left organ.

An interesting fact is that even by the curvature of the ear, you can understand on which side there is a malfunction in the anatomical structure.

Examination of the abdomen is of no small importance. If it is large, then this is also a sign of pathology, in fact, like the presence of many folds.

It is also necessary to pay attention to other signs of malfunction in the body. If a child does not have a kidney, then there may be a malfunction of the genitourinary system.

Girls suffer from uterine defects and organ hypoplasia, but boys have problems with the absence of the vas deferens.

All this is fraught with serious painful attacks during ejaculation. In adulthood, a guy with kidney agenesis may face impotence or infertility.

If an adult consults a doctor for suspected agenesis of one of the kidneys, the specialist must take an anamnesis.

He necessarily takes into account the circumstances and time when the person began to think that he had a similar illness.

It is also important whether there have been examinations for this complaint previously. It is important to establish whether the patient turned to the doctor for help in the treatment of pyelonephritis or glomerulonephritis.

The doctor must find out whether the patient had congenital pathologies of the renal system, whether genetic abnormalities, whether he hit his kidneys, whether operations were performed on the organ.

Manifestation

Over the course of many years, the fact that a child lacks a kidney may not make itself felt at all. There have been cases when a person learned about agenesis in adulthood.

If, while carrying a child in the womb, the mother did not do an ultrasound in accordance with the established plan, and after birth the baby did not undergo examinations, then the diagnosis of the disease can only take place several years later.

For example, a child before school or before getting a job will undergo a study and then it will be determined that he has organ agenesis.

In cases where one of the kidneys is missing, the second organ takes over its tasks. It copes with the assigned function, but only 75 percent.

For this reason, a person may not even suspect that such a pathology is present in his body. There is no discomfort, and therefore the patient will not find any reason to worry.

The situation may turn out differently when the symptoms manifest themselves as external signs.

As already mentioned, agenesis will affect the face, the child may have a small lung capacity, as well as some deformities lower limbs.

Inside, a person with agenesis experiences confusion internal organs. Subsequently, the child may experience dehydration, increased blood pressure, vomiting and polyuria.

If diagnostics show abnormalities during the child’s intrauterine development, but it is not possible to understand that this is bilateral agenesis, hypoplasia, dysplasia, doctors advise terminating the pregnancy artificially.

Classification division

Today, doctors divide the absence of kidneys in humans into several types.

Each of them will be discussed below:

agenesis of the left kidney. A congenital disease characterized by the absence of an organ. The right kidney will do all the work;
agenesis of the right kidney. The pathology is identical to the previous one. Only this time the right organ is missing, and the left one has taken over all its functions;
bilateral agenesis. The disease means that a person does not have a single kidney. Life prognosis is unfavorable. After birth, the child dies a couple of hours later. Of course, progress has also affected medicine, and therefore ways to resist the disease are known. To solve the problem, doctors resort to an organ transplant procedure, as well as regular hemodialysis.

Differences between agenesis of the right and left kidneys

Dwelling on this issue, it is worth noting that the absence of the left and right kidneys are no different from each other.

If we look at medical statistics, agenesis of the right organ is more common in practice; girls suffer from it.

From the first days of life, the presence of pathology is manifested by external signs. But the left organ allows you to maintain the functionality of the genitourinary system, taking responsibility for the work of the missing kidney.

Agenesis of the left kidney is aggravated by severe symptoms. The right kidney takes over the load, but it should be noted that it is not adapted to such work on such a large scale.

If we talk about how pathology manifests itself in men according to external signs, then in women they are much more noticeable.

Course for recovery

Abnormal development of the kidneys, called agenesis, does not pose a serious threat to human life, provided that it is not a bilateral form of the disease.

Often, a child with such a diagnosis is not able to survive. If, in the first hours of life, an operation to transplant the missing organ is performed, then there is a chance of survival.

Subsequently, regular hemodialysis will be required. The procedure involves artificial blood purification.

It is possible to remove toxic products obtained during metabolism from the body and normalize water or electrolyte balance.

Doctors are required to provide emergency care to a newborn with bilateral renal agenesis. Since a child can live no more than a few hours with such an anomaly.

With a unilateral form of the defect, the chances of survival are high. A person with such a diagnosis is required to undergo regular examinations of the body, as well as lead a healthy lifestyle, eat right, and not overload oneself with heavy physical activity.

If any signs of a malfunction in the body appear, you should not hesitate to visit a doctor.

Forecasts

With bilateral renal agenesis, cases of 100 percent death are observed. The baby may die while still inside the mother's womb, or, after being born, he will live no more than a couple of hours. The forecasts are disappointing.

The same cannot be said about cases with unilateral agenesis. The forecasts will be favorable. If there are no complications, then the chances of survival are high.

The patient only needs to monitor his condition. Such an anomaly will not affect a person’s quality of life. These predictions apply to both the right and left organs.

But keep in mind that you will need to undergo blood and urine tests annually, and if discomfort occurs, immediately contact a doctor.

Of course, a person diagnosed with the absence of a right or left kidney should take special measures.

They are related to complete ban on drinking alcohol, avoiding hypothermia, and avoiding highly salted foods.

On an individual basis, the doctor will give special recommendations to the patient. They will be based on the characteristics of the human body.

Registration of disability

It is very rare, but there are always cases when, during agenesis, doctors prescribe registration of disability to the patient. This will depend on what form of pathology is in the body and its severity.

Registration is possible if observed chronic form renal failure stage 2a. Doctors also call it polyuric or compensated.

In this case, it is worth understanding that a single kidney is not able to cope with the task assigned to it. The disability group will be determined based on the condition of the person diagnosed with agenesis.

In the end

One should not take the diagnosis of agenesis of one of the kidneys as a death sentence. The disease requires careful adherence to the principles healthy image life.

It is important to understand that there is only one kidney, it performs a double load, and therefore you should not overload it even more.

If you eat, then exclusively healthy products nutrition. And if you play sports, then in moderation. Also, do not forget about regular examination of the body. Once a year it is recommended to donate blood and urine for tests, as well as undergo an ultrasound.

Agenesis of one organ will not bring any particular inconvenience, but will require a person to take care of himself.

If we talk about a bilateral disease, then we cannot do without a kidney transplant. But there is no need to despair here either.

Modern medicine is developing, but if you start a course of treatment correctly and in a timely manner, then everyone has a chance of recovery.

If future parents know that the child will be born with the absence of two kidneys, then they have a chance to prepare for childbirth and subsequent organ transplant surgery.

Of course, it is worth considering that such treatment is expensive and is not always possible in city clinics.

Useful video

Kidneys are vital organs in humans; they remove water and other substances from the body and actively participate in metabolism. U healthy person There are two kidneys, however, there are congenital abnormalities. In the case of a complete absence of one of them or both at once in a person, kidney agenesis is called in medicine.

Agenesis and aplasia

When there is underdevelopment of an organ that is unable to fully perform its function, then they talk about aplasia. Renal aplasia is similar in nature to the disease with agenesis, but is a less serious anomaly and is usually detected during examination for another disease. Kidneys with this pathology do not have legs and, therefore, are not able to function and secrete urine.

With agenesis - the complete absence of one of the kidneys - no rudiments of renal tissue and ureters are observed in place of the missing organ. In most cases, unilateral organ absence occurs; bilateral pathology is incompatible with life.

The absence of a right or left kidney in a child often does not manifest itself clinically; to identify the pathology you will need diagnostic examination. In addition, the following external signs should alert you:

low position of the ears;
deformation of the lower extremities;
enlarged belly;
wide-set eyes;
displacement of the genital organs.

Causes of the disease

Specific reasons for the development of agenesis have not been identified. Kidneys begin to form in the fetus in the fifth week and continue throughout pregnancy.

The main cause of pathology is congenital malformations of the fetus. The risk increases in the following cases:

There are two types of agenesis:

one-sided;
bilateral.

Bilateral renal agenesis in the fetus is quite rare. As a rule, the baby is born dead or dies during the first days of life. In such cases, termination of pregnancy at any stage is recommended.

With unilateral agenesis and aplasia, all functions are taken over by a second healthy organ, which accordingly takes on the main load and performs a greater amount of work. In addition, the ureter is usually absent. It is very rare that he is present. In this case, timely diagnosis is important.

Underdevelopment of one of the organs—renal aplasia—is considered a relatively favorable pathology compared to agenesis. Aplasia of the right kidney, with healthy and full functioning of the left, often does not manifest itself with specific symptoms.

This is what kidney aplasia looks like

Such pathology is rarely detected due to the lack of specific clinical manifestations. Typically diagnosed on comprehensive examination. After diagnosis, dispensary registration is required.

In this case, treatment is usually not required. Aplasia of the left kidney is often accompanied by underdevelopment nearby organs, for example, the urinary system. It is diagnosed quite rarely, in most cases in men. In the absence of complications does not require treatment, only compliance preventive measures to prevent the development of bacterial infection.

Right-sided agenesis

Agenesis of the right kidney occurs more often than the left and is observed mainly in females. In this case, women experience abnormalities in the development of the uterus, vagina and appendages.

If the left organ is fully functioning, right-sided kidney agenesis does not threaten health and does not require treatment. However, in this case it is required constant surveillance see a doctor and undergo regular diagnostic examinations. A person with this pathology must observe throughout his life strict diet and preventive measures to prevent the development of diseases.

If the left organ is unable to fully perform its work, symptoms appear during the first days of the baby’s life.

Mandatory ongoing consultation with a doctor

In this case, the child experiences the following disorders:

frequent regurgitation, vomiting;
dehydration, dryness skin;
high blood pressure;
general intoxication of the body.

Left-sided agenesis

It is more difficult for a person to tolerate, since the right kidney is less adapted to fully perform its functions. Men are most susceptible to developing this pathology. Agenesis of the left kidney manifests itself as follows:

present painful sensations in the groin area;
there is a violation of sexual functions;
infertility is diagnosed because there is underdevelopment and absence of the vas deferens;
there is pain in sacral region.

Treatment depends on the degree of functioning of the healthy organ. If the right organ is fully functioning, then antibacterial measures are sufficient to reduce the risk of developing kidney diseases or diseases of the urinary system.

Unilateral renal agenesis does not threaten health if it is not accompanied by obvious symptoms. Constant monitoring by a urologist or nephrologist, regular diagnostic examinations and compliance with preventive measures are required.

In case of complications, concomitant diseases and obvious symptoms of the disease, lifelong antihypertensive therapy is required, and sometimes.

Each patient may have individual recommendations from the attending physician. In rare cases, disability is issued.

With unilateral agenesis, the following preventive measures must be observed:

Avoid heavy physical activity - playing sports with kidney pathologies is a big question. With intensive training, the load on the body doubles functional organ. It should be borne in mind that any injury, especially severe injury, can sometimes be fatal. In addition, increased stress can trigger the development of many diseases.
Follow a certain diet and drinking regime. Nutrition and drinking regime play a role important role in the proper functioning of the body system.
Lead a healthy lifestyle, increasing your immunity.

First of all, a woman needs to take care of her health during pregnancy:

exclude bad habits– smoking, alcohol, drugs;
observe proper diet, maintain drinking balance;
be observed by a doctor and monitor the development of the fetus;
If signs indicating the development of an anomaly are detected, you should seek qualified medical help.

People and children who have one kidney live full lives. If unpleasant symptoms occur or painful sensations, you should seek medical help to avoid complications.

During a mild course of the disease, symptoms may be completely absent, so it is necessary to regularly consult a doctor. Especially if the family has precedents for the appearance of pathology. Timely monitoring will help reduce negative manifestations and give the patient’s life the necessary colors

Renal agenesis is caused either by a violation of the stages of embryogenesis during the transition from pronephros to metanephros, or by underdevelopment of the ureteric bud. Renal agenesis can be bilateral or unilateral.

Unilateral agenesis means the absence of one kidney, usually on the left, and is often familial in nature. The incidence rate averages 1 case per 1000 newborns, but is not always diagnosed prenatally. This is explained by the fact that with a unilateral violation, normal amount amniotic fluid and the bladder echo is visualized. In addition, the image of the adrenal gland or even the renal bed may be mistaken for the image of the kidney.

An indirect sign indicating unilateral agenesis, a compensatory enlargement of the contralateral kidney may serve. With Doppler in case of unilateral renal agenesis the renal artery from the affected side is not visualized. Of the combined anomalies, unilateral renal agenesis is most often accompanied by other anomalies of the genitourinary system (adrenal agenesis, in girls - vaginal anomalies, in boys - epididymis and vas deferens), defects gastrointestinal tract(imperforate anus is more common), developmental disorders lumbar region spine, as well as syndromic pathology (for example, VACTERL).

Prognosis for unilateral renal agenesis favorable, however, in this group of children the risk of developing renal pathology, urinary tract infections and the development of chronic renal failure and other urological problems is higher than in the population.

Bilateral renal agenesis(DAP) means complete absence kidneys, ureters and is one of the most pronounced MVS anomalies. The incidence rate is on average 1 case in 3000-5000 births. In male fetuses, the incidence of DAP is twice as high as in girls. DAP is either represented by sporadic cases or is part of syndromes with different types of inheritance (VATER, Fraser). Teratogenic factors include diabetes mellitus, rubella, cocaine, and alkalis.

During ultrasound examination bilateral renal agenesis usually established when a characteristic triad of signs is detected:
- oligohydramnios (can be detected after 16-18 weeks;
- absence of bladder echoten;
- lack of image of the kidneys.

Prenatal ultrasound diagnosis of bilateral renal agenesis

Bilateral renal agenesis can be very difficult due to a similar echographic picture with other pronounced bilateral changes in the kidneys (aplasia, hypoplasia, dysplasia). According to A. Reuss et al. and J. Scott and M. Renwick, the accuracy of prenatal diagnosis of DAP is 69-73%. According to the results of a multicenter study conducted in 17 European countries in 1995-1999, it was found that the accuracy of prenatal ultrasound diagnostics DAP was 78.2%.

An important additional criterion bilateral renal agenesis is the absence of images of the renal arteries in the color Doppler mapping (CDC) mode. In our studies, it was established that the accuracy of diagnosing DAP when using only the B-mode was 80%, in the CD mode - 100%. However, the CDC cannot always help in establishing final diagnosis DAP, since the renal arteries may have a sharply narrowed lumen and not be visualized in dysplastic and hypoplastic kidneys.

Bilateral renal agenesis often accompanied by intrauterine growth retardation (IUGR) and the Potter phenotype, which refers to the consequences of oligohydramnios and includes:
- pulmonary hypoplasia;
- specific facial features (epicanthus, flattened nose, cut chin, pterygoid skin folds, low-set ears);
- deformities of the upper and lower extremities (bone curvature, clubfoot, congenital dislocations hips).

Combined defects in bilateral renal agenesis are almost always present. DAP has been described in more than 140 syndromes with multiple malformations.

Prognosis for bilateral renal agenesis lethal. The causes of death are pulmonary failure due to pulmonary hypoplasia, lack of MVS activity with progressive uremia, combined defects and IUGR. Therefore, if DAP is detected, it is advisable to recommend termination of pregnancy at any stage. Even if it is not possible to accurately establish (distinguish bilateral agenesis from severe bilateral hypoplasia or dysplasia), pregnancy should also be terminated, since these conditions are associated with severe oligohydramnios and its fatal consequences and renal failure. Karyotyping must first be performed to exclude chromosomal abnormalities. A pathological examination should be mandatory to exclude syndromic pathology.

- « Multicystic renal dysplasia. Ultrasound diagnosis of multicystic fetal kidney dysplasia.”

1. Congenital defects and abnormalities of the urinary system. Ultrasound diagnosis of fetal urinary system defects.
2. Agenesis of the fetal kidneys. Ultrasound diagnosis of fetal renal agenesis.
3. Multicystic renal dysplasia. Ultrasound diagnosis of multicystic fetal kidney dysplasia.
4. Autosomal recessive polycystic kidney disease. Autosomal dominant polycystic kidney disease.
5. Hyperechoic fetal kidneys. Causes of hyperechoic fetal kidneys.
6. Doubling of fetal kidneys. Dystopia of the fetal kidneys.
7. Horseshoe-shaped fetal kidney. Single fetal kidney cysts.
8. Tumors of the kidneys and adrenal glands of the fetus. Diagnosis of fetal kidney tumors.
9. Pyeelectasia of the fetal kidneys. Hydronephrosis of the fetal kidneys.
10. Prognosis of fetal kidney pyelectasis. Prognosis for hydronephrosis of the fetal kidneys.

Since the kidney is a paired organ, a person can exist with one kidney. If the child has one kidney, then it is necessary to clarify the type of agenesis (this is the name of this pathology). Each type of agenesis has its own clinical picture and a certain prognosis for later life sick. Agenesis or aplasia is a congenital pathology characterized by underdevelopment of an organ or its complete absence. In such a situation, the ureter may function fully or be absent altogether.

Features of the disease

Kidney agenesis is a congenital pathology

In medical terminology, two terms are often used to define this pathology - agenesis or aplasia. It is necessary to clarify that kidney aplasia is an underdevelopment of an organ that is not able to fully function. With this pathology, a section of the ureter may be preserved, and sometimes an entire canal that ends in a dead end and is not connected to any organ. That is why renal agenesis with preservation of the ureter is aplasia.

Kidney agenesis is a congenital pathology in which there is a complete absence of one or two kidneys. Moreover, in the place where the organ should be, there are not even its rudiments, and there cannot be a ureter or any part of it. Renal agenesis and renal aplasia occur among all renal pathologies in 7-11% of cases. Children with unilateral agenesis are born quite rarely - approximately 1 in a thousand, and bilateral pathology occurs in one in 4000-10000.

Kidney aplasia, like agenesis, provokes the development of the following concomitant diseases:

pyelonephritis; ICD; arterial hypertension.

If a girl is born with one kidney, pathologies of the genital organs are also detected, namely their underdevelopment. With this developmental anomaly, all functions are assigned to the healthy kidney, which often increases in size as it has to cope with an increased amount of work.

Important: according to statistics, such a renal anomaly is more often diagnosed in boys than in girls.

Reasons

The fetus's kidneys begin to form in the fifth week of pregnancy

There is no consensus why the child was born with one kidney. The main thing is that the genetic factor does not play any role in this pathology. That is why it is a congenital condition. The fetus's kidneys begin to form in the fifth week of pregnancy. This process continues throughout intrauterine development. That is why it is very difficult to identify the cause of this pathology.

Kidney aplasia or its complete absence can be caused by the following factors:

Viral and other infectious diseases of women in the 1st trimester of pregnancy. Typically, this pathology is caused by influenza and rubella. Congenital absence of a kidney can be caused by exposure to ionizing radiation during pregnancy. diagnostic purposes. Diabetes mellitus in pregnant women is also considered a risk factor. This could be the consequences of taking strong medications or hormonal contraceptives that the patient took without consulting a doctor. Drinking alcohol and chronic alcoholism. Various sexually transmitted diseases can cause the absence of a kidney in the fetus.

Clinical picture

Kidney agenesis in a baby may not manifest itself in any way for many years.

Kidney agenesis in a baby may not manifest itself in any way for many years. If routine ultrasounds were not performed during gestation, and the baby was not thoroughly examined after delivery, then the disease can be detected completely by accident many years after birth. For example, during a profile examination at work or at school.

If a child has one kidney, the second organ takes on 75 percent of its functions, so the patient does not experience any discomfort. However, sometimes external symptoms of kidney development abnormalities appear immediately after the birth of the baby.

Renal agenesis in a newborn can be suspected based on the presence of the following symptoms:

Defects of the facial structure (widened and flattened nose, puffiness of the face, ocular hypertelorism). The ears are low and significantly curved. As a rule, such an ear is observed on the side of the missing kidney. There are a lot of folds on the child's body. The baby may have a big belly. The child has a small volume of respiratory organs. This is called pulmonary hypoplasia. A newborn's legs may be deformed. There is a shift in the location of internal organs.

Attention: children may subsequently develop associated symptoms– polyuria, vomiting, hypertension and dehydration.

The absence of one kidney, namely agenesis, is very often accompanied by anomalies in the formation of the genital organs. Female representatives may have a bicornuate or unicornuate uterus, as well as its hypoplasia or vaginal atresia. In boys, the pathology is accompanied by the absence of the vas deferens and pathological abnormalities in the seminal vesicles. This can subsequently lead to groin pain, impotence, painful ejaculation and infertility.

If during pregnancy it is not possible to accurately diagnose fetal developmental abnormalities, namely, hypoplasia, dysplasia or bilateral agenesis, then it is recommended to terminate the pregnancy. The thing is that such pathologies can lead to oligohydramnios and severe disturbances in the functioning of the kidneys in the fetus.

Varieties

Aplasia of the right kidney is most often diagnosed

The main classification of agenesis is based on the number of organs:

Bilateral agenesis is the complete absence of both organs. This pathology is simply incompatible with life. If during an ultrasound scan of a pregnant woman such a pathology of the fetus is revealed even at later, then shown artificial birth. Aplasia of the right kidney is most often diagnosed. The thing is that the right kidney in all people is lower than the left. It is more mobile and smaller in size, therefore it is more vulnerable. Most often, the left organ successfully copes with the functions of the right kidney.

Important: if left kidney for some reason cannot compensate for the lack of the right organ, then the symptoms of the pathology will begin to appear from the very birth of the baby. In the future, such a child is likely to develop kidney failure.

As a rule, agenesis of the left kidney is diagnosed less frequently, but is more difficult to tolerate, since the right organ is by its nature less functional and mobile, and therefore is less able to compensate for the lack of a paired organ. With this pathology, all the classic symptoms of aplasia and agenesis appear.

In addition, there are several forms of agenesis:

Damage to the organ on one side with preservation of the ureter. This form of pathology poses virtually no threat to a person’s normal life. If the second organ fully copes with the double load, and the person adheres to a healthy lifestyle, then there is no risk. Moreover, nothing bothers the person, so he may not even be aware of his pathology. Damage to the organ on one side with the absence of the ureter. In the absence of a ureter, abnormalities of the genital organs are also observed, so there can be no question of a full life.

Organ aplasia is divided into several types:

Large aplasia is represented by fibrolipomatous tissue, which contains small cystic inclusions. In this case, the ureter and nephrons are also not detected. Minor aplasia is characterized by the presence of a small number of nephrons and the presence of a ureter with an orifice. However, the ureter itself very often does not reach the renal parenchyma and is greatly thinned.

Diagnosis and treatment

This disease is usually diagnosed by a nephrologist.

Typically, the diagnosis of this disease is carried out by a nephrologist. After familiarizing yourself with clinical picture The doctor prescribes the following examinations:

Contrast urography. Ultrasound of the kidneys. CT. Renal angiography. Cystoscopy.

The only one dangerous form agenesis – bilateral. If such children did not die in utero or during childbirth and were born alive, they died in the first day after birth due to rapidly progressing renal failure.

Today, thanks to the development of perinatal medicine, there is a possibility of saving the lives of such children. To achieve this, a kidney transplant is performed immediately after delivery. Subsequently, it is necessary to systematically do hemodialysis. However, this is only possible if a quick differential diagnosis abnormalities of fetal development, which will exclude defects of other organs of the urinary system.

Aplasia of the left kidney or right organ, as well as unilateral agenesis give the most favorable prognosis. If the patient does not have any discomfort or unpleasant symptoms, then once a year you need to take urine and blood tests and take care of the only healthy organ that has a double burden. It is important not to drink alcohol, not to get too cold and not to eat too much salty food. The doctor will also give individual recommendations for each patient, taking into account the characteristics of his body.

Important: when severe form agenesis and aplasia may require hemodialysis and sometimes transplantation.

Very rarely, in the presence of agenesis, disability is issued. This usually happens when there is chronic failure in stage 2a. It is called compensated or polyuric. With her the only organ cannot cope with the load placed on it, and its activity is compensated by the liver and other organs. Moreover, the disability group is determined depending on the patient’s condition.

Vital important bodies human are the kidneys. Their main task is to remove water and water-soluble substances from the body. They perform a regulatory function in relation to water-salt, acid-base balance, and participate in metabolism. A healthy person has both kidneys, but 2.5-3% of newborns have abnormalities.

One of congenital pathologies, known since the time of Aristotle, is kidney agenesis, characterized by the absence of one or two organs. This anomaly should not be confused with kidney aplasia, in which the organ is represented by an underdeveloped rudiment that does not have a formed pedicle and pelvis. According to statistics, renal agenesis most often occurs in boys. Medicine does not have reliable information that the pathology is hereditary. Deviations in the formation of kidneys in the fetus can be caused by exogenous factors affecting the embryonic stage of fetal development. The risk increases due to:

viral (infectious) diseases suffered by the pregnant woman; ionizing radiation; presence of diabetes mellitus; alcohol, drug addiction; venereal disease.

There are two types of renal agenesis:

one-sided; bilateral.

Unilateral pathology is distinguished, in addition to the presence of one organ, by an absent ureter. With rare exceptions, it may be present. The main burden falls on the formed organ.

Kidney agenesis - diagram

Agenesis of the right kidney

Agenesis of the right kidney is much more common, especially in women. They may have deviations in the development of the uterus, vagina and appendages. The vulnerability of the right organ is associated with smaller size and mobility compared to the left. If the left kidney is unable to perform a compensatory function, symptoms of the anomaly may appear in the first days of the child’s life. In this case, kidney agenesis is accompanied by polyuria, frequent regurgitation and vomiting, dry skin, indicating dehydration, increased blood pressure, general intoxication of the body, renal failure. If the compensatory function is performed by the left organ, then the pathology is usually detected as a result of a special examination. However, the attending physician should note special attention for external signs, expressed in puffiness of the face, very low-set ears, ocular hypertelorism, a wide flattened nose, and a deformed skull.

Agenesis of the left kidney

Agenesis of the left kidney is similar in essence to the previous type. However, it is more difficult for a person to bear, since the entire load falls on right kidney, less adapted in such cases to full implementation compensatory function. Symptoms are more severe, especially in men, who are more susceptible to this type of anomaly. As a rule, this pathology accompanied by pain in the groin area, sacral region, sexual dysfunction, underdevelopment or absence of the vas deferens, which leads to infertility. External signs indicating a possible anomaly are the same as with agenesis of the right kidney.

Agenesis of the right or left kidneys, which does not threaten health and is not accompanied by obvious symptoms, requires constant monitoring by a urologist or nephrologist, regular examination, and the adoption of preventive measures to reduce the risk of kidney diseases. Otherwise, treatment includes lifelong antihypertensive therapy, and also possible transplantation organ.

Bilateral renal agenesis

This pathology is the most dangerous type. With bilateral renal agenesis, that is, their complete absence, fetal death occurs in the womb or several hours/days after birth. In such a situation, if the diagnosis is timely and accurate, organ transplantation and regular hemodialysis can help.

Diagnosis and additional measures to prevent complications

Kidney agenesis - diagnosis and additional measures prevention of complications

The examination, prescribed by a doctor at least once a year, includes:

urography with contrast agent; computed tomography; ultrasound examination; renal angiography; cystoscopy.

The specialist identifies the factors that influenced the occurrence of the pathology. Genetic counseling families, an ultrasound examination of the kidneys in close relatives will also make it possible to more accurately determine the cause of renal agenesis in the fetus. Along with agenesis, other diseases of the genitourinary system may also appear in the body. Unilateral renal agenesis usually requires the patient to take basic preventive measures:

avoid hypothermia and heavy physical exertion; follow a certain diet and drinking regime; increase immunity, preventing the occurrence of viral and infectious diseases, inflammatory processes.

The question of whether it is admissible to play sports with such an anomaly is still open. The absence of one kidney does not generally prevent such activity, but the performance of a compensatory function by a healthy organ doubles the load on it. Therefore, the consequences of severe injury can be fatal. In addition, the presence increased loads provokes the development of polyuria, arterial hypertension, and fatigue.

Nutrition plays an important role in maintaining the normal functioning of a single kidney. Smoked, canned, spicy, salted, flour products, and any mineral and alcoholic drinks are excluded from the diet as much as possible. It's better to replace coffee herbal tea, fruit drink. It is recommended to consume boiled fish and meat no more than 3 times a week. Preference is also given to oatmeal and buckwheat porridge, black and white grain bread. The consumption of dairy and fermented milk products is limited.

Expectant mothers should also take care of their own health, monitor the development of the fetus, and refrain from alcoholic drinks, narcotic substances, smoking. If signs indicating a disorder in the development of the fetus's kidneys are detected, you should contact a qualified specialist. In case of unilateral pathology, pre- and postnatal karyotyping is used. With bilateral renal agenesis, a symmetrical form of fetal growth retardation, as well as oligohydramnios, is observed. In case of prenatal detection of such an anomaly before 22 weeks, termination of pregnancy is indicated. medical indications. If this procedure is refused and the deviation is diagnosed late, conservative obstetric tactics are used.

Maintaining a healthy lifestyle, timely medical examination and appeal to qualified specialists with any signs indicating any abnormalities - all this will ensure the birth healthy baby and will significantly reduce the risk of developing possible pathologies. Unilateral renal agenesis in a child in the absence of other pathologies allows him to full life. It is extremely rare that disability registration is required. It is important to pay due attention medical recommendations and regular examination by your doctor.

Renal agenesis is an anatomical disorder in which one or both of these organs are absent. If one or both kidneys are represented by underdeveloped buds, this condition is called aplasia.

Manifestations of the disease and prognosis usually depend on whether at least one of the organs is present and which one.

Prevalence

The incidence of renal agenesis has been studied more than once, and the results for different types of abnormalities differ markedly.

It is believed that congenital absence of both kidneys occurs in 1-3 children out of 10 thousand. Boys suffer more often, the ratio is about 2.6:1. One kidney is missing in approximately 1 in 500 newborns, most often the left organ.

Boys are affected three times more often than girls. With renal agenesis in children, other abnormalities in the structure of the body are often found, for example, underdevelopment or absence of the vas deferens, prostate gland and one of the testicles in boys or abnormalities in the structure of the uterus, vagina and ovaries in girls.

Causes of renal agenesis

The reasons for the formation of this pathology have not been fully established, but the main and statistically confirmed ones are:

Genetic predisposition. It is known that in some families this violation occurs more often.
Infectious diseases suffered by a woman while expecting a baby. Influenza and rubella are considered the most dangerous in the first trimester.
Exposure of the expectant mother to ionizing radiation.
Incorrect and uncontrolled use of hormonal contraceptives.
Diabetes mellitus and some others endocrine diseases at expectant mother.
Chronic alcohol abuse in women, as well as smoking and drug use.
Sexually transmitted diseases mothers, especially.

If at least one of these problems is detected in the expectant mother, special attention should be paid to regular ultrasound examinations, which will help to identify the pathology in a timely manner.

It is not possible to diagnose renal agenesis or aplasia based on external signs alone. Usually additional examinations prescribed if the newborn has the following symptoms:

Too wide and flat nose;
The ears have a non-standard shape and are located too low;
The face is puffy with protruding frontal lobes;
The distance between the eyes has been increased;
Underdeveloped jawbone;
Epicanthus present.

Also often observed in parallel with agenesis are hypoplasia of the lungs, too noticeable folding of the skin and an enlarged abdomen.

All these signs are usually noticeable with bilateral renal agenesis. The disease is also accompanied by renal failure and the resulting hypertension and polyuria. Children with this diagnosis are not viable.

The manifestations of unilateral agenesis depend on how well the second kidney is developed and how it copes with the load. Very often doctors do not notice the problem at all, and it is discovered only in adulthood during inflammatory diseases second organ or routine ultrasound examinations.

Approximately 15% of patients with this pathology experience vesicoureteral reflux.

Normal healthy kidney begins to increase after one month of the baby’s life, but growth trends appear even before birth. If a healthy organ is not capable of compensation, then the child experiences excessive urination, leading to dehydration, regurgitation and vomiting, increased blood pressure, intoxication and other manifestations of renal failure. This usually happens with agenesis of the left kidney. The right organ is smaller, more mobile and less functional. It has also been noted that the absence of the left kidney is often accompanied by disorders in the sexual sphere.

Renal agenesis can be diagnosed both in utero and after the baby is born. Pathology is usually determined using ultrasound. The absence of kidneys in the fetus is indicated by oligohydramnios during pregnancy and the absence of an echo shadow of the urinary tract during a dynamic study. Agenesis is also indicated by the absence of renal arteries on color Doppler mapping.

If the fetus is missing only one of the organs, it is very difficult to determine this by ultrasound due to the fact that:

The volume of amniotic fluid in this case does not change;
Bladder the embryo can be identified;
Sometimes the adrenal gland is mistaken for a kidney.
If renal agenesis is suspected, an ultrasound can be done after birth. Other studies are also carried out:
Excretory urography- with agenesis of the kidney, you can notice a compensatory enlargement of the paired organ, and on the other side contrast agent absent;
Nephroscintigraphy - radioisotopes do not accumulate in the affected side;
Cystoscopy - it allows you to detect hypoplasia of Lieto’s triangle in the ureter, formed by part urethra and the orifices of the ureters;
Angiography - there are no renal vessels on the side of the affected organ.

Treatment

Agenesis of one kidney does not require any treatment, since the second organ is quite capable of performing all the necessary functions. It is advisable to undergo periodic examinations to make sure that everything is normal and to comply with special diet. Also, some experts recommend regular courses of preventive metabolic therapy to maintain the working organ.

When treating diseases of other organs, patients with agenesis must choose drugs that do not affect the kidneys and are excreted primarily by the liver. If the only functioning organ is malfunctioning, a transplant from a donor may be required. And the absence of two kidneys usually leads to fatal outcome, no treatment exists yet.

Forecast

Agenesis of both kidneys leads to 100% mortality. Death occurs in utero or a few hours after the birth of a sick baby. And unilateral agenesis has quite favorable prognosis. If there are no associated disorders, and the patient carefully monitors his health, he has every chance of living a full life.