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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, consists 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 which factors increase the risk of renal agenesis. Doctors explain how a congenital defect occurs, 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 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 liquid and decay products, with accumulation toxic substances above the 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 excreted from the body;
• develop important hormones: renin, erythropoietin;
• produce calcitriol for optimal absorption of phosphorus and calcium from the digestive tract;
• maintain electrolytic balance.

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

Reasons for the development of pathology

A congenital defect develops under the influence of negative factors during organ formation. A child with anomalies of natural filters is born with a combination of environmental, genetic factors, when a woman violates the rules that preserve the health of the fetus. Often doctors identify a combination negative factors which increases the risk of anomalies.

Causes of congenital absence of one or two kidneys:

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

Kidney agenesis ICD code - 10 - Q60.

Types and forms of the disease

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

Unilateral (ureter preserved)

Congenital malformation of the first clinical type. With a unilateral variety of pathology, the remaining kidney functions with a double load, hyperplasia of the organ is often observed. An increase in the number of elements that ensure the accumulation, filtration and excretion of urine allows you to timely remove toxins, poisons, and nitrogenous decay products from the body. Injury to the only natural filter leads to serious problems with blood purification.

Unilateral (no ureter)

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

Anatomical disorders lead to unpleasant symptoms, impaired sexual function. Men feel pain in the sacrum and groin area. Ejaculation also causes discomfort. A combination of negative factors has a negative impact on man's health and fertility.

Bilateral agenesis

Dangerous Vice prenatal development, the third clinical type. The most common birth dead child. In some cases, a live baby was born, but due to death occurred in the first few days.

Thanks to the achievements of medicine, it is possible to perform a kidney transplant to a newborn and to cleanse the body. Important points: quick selection donor organ, with which there are difficulties, timely diagnosis of the defect. Doctors on the first day should determine if the baby has bilateral aplasia of the kidneys or other urinary tract anomalies.

Diagnostics

Modern methods of examination of a pregnant woman can reveal many deviations in the development of the unborn child. AT without fail order an ultrasound for different terms to control fetal development. A standard ultrasound examination gives an accurate picture of the condition of the urinary tract.

When 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 aplasia of the kidney.

To analyze the work 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. The aquatic environment reduces the risk traumatic injuries growing organism, allows the lungs to ripen. Oligohydramnios (lack of the required amount of amniotic fluid) often develops when one or two bean-shaped organs malfunction. To dangerous consequences oligohydramnios can be attributed to the defeat of the lungs, which cannot be formed in full.

Characteristic signs and symptoms

Bilateral renal aplasia can be suspected by several additional signs:

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

Potter Syndrome - this is the name received characteristics, 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:

• auricles are deformed, are below the usual border;
• the fetus has a flat, wide nose;
• frontal lobes prominently protrude;
• puffy face;
• lung hypoplasia is observed;
• the lower limbs are deformed;
• eye hypertelorism is manifested;
• epicanthus appears - a special fold of skin that covers the lacrimal tubercle;
• folded skin.

In the early days, newborns experience:

• dehydration;
• kidney failure;
• high blood pressure;
• vomit.

On a 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 drugs to prevent inflammation, improve urine outflow, 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 up, 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, intoxication of the body. In severe cases, renal failure develops, a fatal outcome is possible.

Renal agenesis in adults and children

In the presence of one kidney, the bean-shaped organ increases: you have to work with a double load to ensure all functions. An increased load often provokes a violation of the processes in the natural filter, an increase in blood pressure, fluid retention in the body, and the development of puffiness. Sometimes blood appears in the urine and against the background inflammatory process in the surviving kidney.

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

The patient receives advice on diet food, dosing physical activity, drinking regimen. At the first signs of the inflammatory process, the provoking factor is eliminated, prescribe antibiotic therapy, reception and herbal formulations that improve the outflow of urine.

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

Treatment methods for stenosis renal arteries and arterial hypertension described page.

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

Helpful Hints:

• excessive loads of any kind should not be allowed: damage to a single kidney worsens the process of blood purification, leads to intoxication against the background of accumulation of decay products;
• it is important not to abuse drugs;
• you need to eat right;
• you can not lift weights;
• to preserve the health of a single kidney, you will have to give up alcohol, smoking, work in hazardous industries;
• kidney agenesis in children requires regular visits to the nephrologist. Aplasia of the kidneys in newborns adversely affects the condition of the child, while the body is not yet strong. Increased attention to drinking regimen, nutrition, rest and physical development baby.

When identifying a congenital defect of the bean-shaped organs, 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 for preventing complications. Doctors give many examples when 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 ailment, characterized by the absence of an organ, both one and two kidneys at the same time.

About pathology

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

Cases when there is no one organ in a newborn occur 1 time out of 1000, but bilateral kidney agenesis is recorded even less often 1 time out of 4000-10000.

At the birth of a child 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, as he is forced to cope with a large number loads.

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

Pathological complications include urolithiasis, arterial hypertension, or pyelonephritis.

On the factors provoking the development of agenesis

To date, scientists have still not been able to establish what the true causes of pathology really 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, an anomaly that can develop during intrauterine formation can provoke kidney agenesis. This may be due to exogenous factors.

First of all, doctors note that if a family has a genetic predisposition to this pathology, then the risks of fixing it 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. This may include cases:

• the course of infectious diseases (influenza, rubella during the first 3 months of pregnancy);
• improper intake of hormones;
• exposure;
• presence of diabetes mellitus and other malfunctions endocrine system pregnant, as well as venereal pathologies. Syphilis is of particular danger;
• drug, alcohol and tobacco abuse.

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

Diagnostic Measures

In order 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 some other nuances of 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 the defects of the face.

The baby may be too flat or very wide nose. Puffiness or ocular hypertelorism can also be seen on the face.

Be sure the specialist pays attention to the localization of the ear shells. When located too low or strongly curved, this indicates that a diagnosis is possible - agenesis of the right kidney or the absence of the left organ.

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

Of no small importance is the examination of the abdomen. If it is large, then this is also a sign of pathology, in fact, as well as the presence of many folds.

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

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

All this is fraught with serious pain attacks during ejaculation. As an adult, a guy with kidney agenesis may experience impotence or infertility.

If an adult turns to the doctor for suspicion of agenesis of one of the kidneys, then the specialist should take an anamnesis.

He necessarily takes into account the circumstances and time when it began to seem to a person that he had a similar ailment.

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

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

Manifestation

For many years, the fact of the absence of a kidney in a child may not make itself felt at all. There were cases when a person learned about agenesis at an older age.

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

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

In cases where one of the kidneys is missing, the second organ takes over its tasks. He copes with the assigned function, but by 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 cause for concern.

The situation can be folded differently when the symptoms manifest themselves by external signs.

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

Inside a person with agenesis there is a mixture internal organs. Subsequently, the child may experience dehydration, increased pressure, bouts of vomiting and polyuria.

If the diagnosis showed deviations during the intrauterine development of the child, but it is not possible to understand that this is bilateral agenesis, hypoplasia, dysplasia, doctors advise to terminate the pregnancy artificially.

Classification division

To date, 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 ailment characterized by the absence of an organ. The right kidney will take over 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 has taken over all its functions;
• bilateral agenesis. The disease means that a person does not have a single kidney. Forecasts for life are unfavorable. After birth, the child dies after a couple of hours. Of course, progress has also affected medicine, and therefore there are ways to resist the disease. Doctors resort to organ transplantation to solve the problem, 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 turn to the data of medical statistics, then 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 in such a large volume.

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

course for recovery

The abnormal development of the kidneys called agenesis does not pose a serious threat to human life, but on condition 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 is performed to transplant the missing organ, then there are chances for survival.

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

From the body it is possible to remove toxic products obtained during metabolism, to normalize the water or electrolyte balance.

Doctors require emergency care for a newborn with bilateral agenesis of the kidneys. Since a child can live no more than a few hours with a similar anomaly.

With a one-sided form of the defect, the chances of survival are high. A person with a similar diagnosis is required to undergo regular examinations of the body, as well as lead a healthy lifestyle, eat right, and not burden themselves with heavy physical exertion.

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

Forecasts

With bilateral renal agenesis, cases of 100 percent death are observed. The baby can die while still inside the womb, or, having been born, he will live no more than a couple of hours. Forecasts are disappointing.

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

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

But keep in mind that you will need to take blood and urine tests annually, and in case of discomfort, immediately contact the doctor.

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

They are associated with complete ban on drinking alcohol, avoiding hypothermia, and avoiding highly salted foods.

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

Registration of disability

Very rarely, but there are all cases when, with agenesis, doctors prescribe a disability for the patient. It will depend on what form of pathology in the body and its severity.

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

In this case, it should be understood 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.

Eventually

It is necessary not to take the diagnosis of agenesis of one of the kidneys as a sentence. The disease requires careful adherence to the principles healthy lifestyle life.

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

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

Agenesia of one organ will not bring any particular inconvenience, but will require a careful attitude of a person to himself.

If we talk about a bilateral disease, then a kidney transplant is indispensable. But there is no need to despair here either.

Modern medicine is developing, but if you start the course of treatment competently 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 transplantation.

Of course, it should be borne in mind that such treatment is expensive and the possibility of conducting it in city clinics is not always available.

Useful video

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

Agenesia and aplasia

There is an underdevelopment of an organ that is unable to fully perform its function, then they talk about aplasia. Aplasia of the kidney is similar in nature to agenesis, but is a less severe anomaly and is usually detected during examination for another disease. The kidneys in this pathology do not have legs and, therefore, are not able to function and excrete urine.

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

The absence of the right or left kidney in a child is often not clinically manifested; diagnostic examination. In addition, the following external signs should alert:

• low position of the ears;
• deformity of the lower extremities;
• enlarged belly;
• widely spaced eyes;
• displacement of the position of the genitals.

Causes of the disease

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

The main cause of the pathology are 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 already dead or dies during the first days of life. In such cases, termination of pregnancy at any time is recommended.

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

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

This is what kidney aplasia looks like

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

Treatment in this case, as a rule, is not required. Aplasia of the left kidney is often accompanied by underdevelopment nearby organs such as the urinary system. It is diagnosed quite rarely, in most cases in men. In the absence of complications, no treatment is required, only compliance preventive measures to prevent bacterial infection.

Right-sided agenesis

Agenesis of the right kidney occurs more often than the left and is observed mainly in the female. At the same time, women have deviations in the development of the uterus, vagina and appendages.

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

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

Mandatory regular consultation with a doctor

In this case, the child has 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 carry, since the right kidney is less adapted to the full performance of functions. Men are most susceptible to the development of such a pathology. Agenesis of the left kidney is manifested as follows:

• present pain in the groin area;
• there is a violation of sexual functions;
• infertility is diagnosed, since there is underdevelopment and the absence of the vas deferens;
• pain is seen in sacral region.

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

Unilateral renal agenesis is not a health hazard unless accompanied by overt symptoms. You need constant monitoring by a urologist or nephrologist, regular diagnostic examinations and compliance with preventive measures.

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

For each patient, there may be individual recommendations from the attending physician. In rare cases, a disability is issued.

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

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

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 are found that indicate the development of an anomaly, you should seek qualified medical help.

People and children with one kidney live a full life. If you experience any symptoms or pain, you should seek medical help in order to avoid the development of complications.

With a calm course of the disease, symptoms may be completely absent, so it is necessary to consult a doctor regularly. Especially if the family has precedents for the appearance of pathology. Early monitoring can help reduce negative manifestations and give the life of the patient the necessary colors

Renal agenesis due to either a violation of the stages of embryogenesis during the transition from pronephros to metanephros, or underdevelopment of the ureteral rudiment. Renal agenesis can be bilateral or unilateral.

Unilateral agenesis means the absence of one kidney, usually on the left, and is more often familial. The frequency of occurrence averages 1 case per 1000 newborns, but is not always diagnosed prenatally. This is due to the fact that with a unilateral violation, the normal amount amniotic fluid and the bladder echo is visualized. In addition, an image of the adrenal gland or even the renal bed can be mistaken for an image of the kidney.

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

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

Bilateral renal agenesis(DAP) means complete absence kidneys, ureters and is one of the most pronounced anomalies of the MVS. The frequency of occurrence averages 1 case per 3000-5000 births. In male fetuses, the incidence of DAP is twice as common 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, alkalis.

On ultrasound bilateral renal agenesis usually established when a characteristic triad of signs is detected:
- oligohydramnios (can be detected after 16-18 weeks;
- absence of bladder echo;
- no picture 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 multicentre 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 lack of an image of the renal arteries in the mode of color Doppler mapping (CDC). In our studies, it was established that the accuracy of diagnosing DAP when using only the B-mode was 80%, in the CFM mode - 100%. However, the CDC may not 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 associated with intrauterine growth retardation (IUGR) and the Potter phenotype, which refers to the consequences of oligohydramnios and includes:
- lung hypoplasia;
- specific facial features (epicant, flattened nose, cut chin, pterygoid skin folds, low-lying ears);
- deformities of the upper and lower extremities (bone curvature, clubfoot, congenital dislocations hips).

Combined malformations 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 insufficiency due to lung hypoplasia, lack of MVS activity with progressive uremia, concomitant malformations and IUGR. Therefore, if DAP is detected, it is advisable to recommend termination of pregnancy at any time. 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 rule out chromosomal abnormalities. A pathoanatomical examination should be mandatory to exclude syndromic pathology.

- « Multicystic dysplasia of the kidney. Ultrasound diagnosis of multicystic fetal kidney dysplasia.

1. birth defects and anomalies of the urinary system. Ultrasound diagnosis of malformations of the urinary system of the fetus.
2. Fetal kidney agenesis. Ultrasound diagnosis of fetal kidney agenesis.
3. Multicystic kidney dysplasia. Ultrasound diagnosis of multicystic fetal kidney dysplasia.
4. Autosomal recessive polycystic kidney disease. Autosomal dominant polycystic kidney disease.
5. Hyperechoic kidneys of the fetus. Causes of hyperechoic fetal kidneys.
6. Doubling of the kidneys of the fetus. Fetal kidney dystopia.
7. Horseshoe kidney of the fetus. Solitary fetal kidney cysts.
8. Tumors of the kidneys and adrenal glands of the fetus. Diagnosis of fetal kidney tumors.
9. Pyeloectasia of the kidneys of the fetus. Hydronephrosis of the kidneys of the fetus.
10. Prognosis of pyelectasis of the kidneys of the fetus. Prognosis for hydronephrosis of the kidneys of the fetus.

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, which is characterized by underdevelopment of the 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 must be clarified that kidney aplasia is the underdevelopment of an organ that is not able to fully function. With such a pathology, a section of the ureter can be preserved, and sometimes the whole canal, ending in a dead end and not connecting with 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 can be no ureter or any part of it either. Renal agenesis and aplasia of the kidneys occur among all renal pathologies in 7-11% of cases. With unilateral agenesis, children are born quite rarely - about 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.

In the case of the birth of a girl with one kidney, pathologies of the genital organs are also detected, namely their underdevelopment. With this developmental anomaly, all functions are assigned to a 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.

The reasons

In the fetus, kidneys begin to form at the fifth week of pregnancy.

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

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

Viral and other infectious diseases of a woman in the 1st trimester of pregnancy. Usually such a pathology is given by influenza and rubella. Congenital absence of a kidney can be caused by the use of ionizing radiation during pregnancy in diagnostic purposes. Diabetes in a pregnant woman is also considered a risk factor. These may be the consequences of taking strong drugs or hormonal contraceptives, which the patient drank 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 for many years

Kidney agenesis in a baby may not manifest itself for many years. If planned ultrasounds were not performed during gestation, and after delivery the baby was not carefully examined, then the disease can be detected quite 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 over 75 percent of its functions, so the patient does not experience any discomfort. However, sometimes the external symptoms of an anomaly in the development of the kidneys appear immediately after the birth of the baby.

Kidney agenesis in a newborn may be suspected based on the presence of the following symptoms:

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

Caution: children may subsequently develop concomitant 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 atresia of the vagina. In boys, the pathology is accompanied by the absence of the vas deferens and pathological abnormalities in seminal vesicles. Subsequently, this can lead to groin pain, impotence, painful ejaculation and infertility.

If during pregnancy it is not possible to accurately diagnose abnormalities in the development of the fetus, namely, to clarify, 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 disorders in the activity of the kidneys in the fetus.

Varieties

Aplasia of the right kidney is most often diagnosed

The main classification of agenesis is carried out according to 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 fetal pathology is detected even at late term, then shown artificial childbirth. Aplasia of the right kidney is diagnosed most often. 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 renal failure.

As a rule, agenesis of the left kidney is diagnosed less often, but it is more difficult to tolerate, since the right organ is inherently less functional and mobile, and therefore compensates for the lack of a paired organ worse. With this pathology, all the classic symptoms of aplasia and agenesis are manifested.

In addition, there are several forms of agenesis:

The defeat of the body on the one hand with the preservation of the ureter. This form of pathology practically does not pose any threat to the normal life of a person. If the second organ fully copes with a double load, and a person adheres to a healthy lifestyle, then there is no risk. Moreover, a person is not worried about anything, so he may not be aware of his pathology. The defeat of the body on the one hand with the absence of the ureter. In the absence of the ureter, anomalies 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, in which there are small cystic inclusions. At the same time, the ureter and nephrons are also not detected. Small aplasia is characterized by the presence of a small number of nephrons, the presence of an ureter with an orifice. However, the ureter itself very often does not reach the renal parenchyma and is very thin.

Diagnosis and treatment

This disease is usually diagnosed by a nephrologist.

Usually, the diagnosis of this disease is carried out by a nephrologist. After getting acquainted with clinical picture the doctor prescribes the following examinations:

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

the only dangerous form agenesia - bilateral. If such children did not die in utero or during childbirth and were born alive, then they died on 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 life of such children. To do this, immediately after delivery, a kidney transplant is performed. Subsequently, it is necessary to systematically do hemodialysis. However, this is only possible if a quick differential diagnosis anomalies in the development of the fetus, which will eliminate the defects of other organs of the urinary system.

Aplasia of the left kidney or right organ, as well as unilateral agenesis give the best prognosis. If the patient does not have any discomfort and 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 load. It is important not to drink alcohol, not to get 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, a disability is issued. This usually happens when there is chronic insufficiency in stage 2a. It is called compensated or polyuric. With her sole 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 condition of the patient.

Vital important organs 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 the water-salt, acid-base balance, 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. Such an anomaly should not be confused with kidney aplasia, in which the organ is represented by an underdeveloped rudiment that does not have a well-formed stalk and pelvis. According to statistics, renal agenesis is most common in boys. Medicine does not have reliable information that the pathology is hereditary. Exogenous factors affecting the embryonic stage of fetal development can lead to deviations in the formation of kidneys in the fetus. The risk increases due to:

transferred pregnant viral (infectious) diseases; ionizing radiation; the presence of diabetes; 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 the absence of the ureter. With rare exceptions, it may be present. The main load falls on the formed organ.

Renal agenesis - scheme

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, the symptoms of an anomaly may appear in the first days of a 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, as a rule, is detected as a result of a special examination. However, the attending physician must Special attention on external signs, expressed in a puffy 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 variety. However, it is more difficult for a person to carry, since the entire burden falls on right kidney, less adapted in such cases to the full implementation compensatory function. Symptoms are more pronounced, especially in men who are more susceptible to this type of anomaly. Usually, this pathology accompanied by pain in the groin, 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 is not threatening to health and is not accompanied by obvious symptoms, involves constant monitoring by a urologist or nephrologist, regular examinations, and the adoption of preventive measures that reduce the risk of kidney disease. Otherwise, treatment includes lifelong antihypertensive therapy, as well as possible transplant organ.

Bilateral renal agenesis

This pathology is the most dangerous variety. With bilateral agenesis of the kidneys, that is, their complete absence, the death of the fetus occurs in the womb or after a few hours / days after birth. In such a situation, with the timeliness and accuracy of the diagnosis, organ transplantation, regular hemodialysis can help.

Diagnosis and additional measures to prevent complications

Kidney agenesis - diagnosis and additional measures prevention of complications

An examination ordered by a doctor at least once a year includes:

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

The specialist identifies the factors that influenced the occurrence of pathology. genetic counseling families, conducting an ultrasound examination of the kidneys in close relatives will also allow you to more accurately determine what is the cause of renal agenesis in the fetus. Along with agenesis, other diseases of the genitourinary system can be detected in the body. Unilateral renal agenesis usually involves the patient taking basic preventive measures:

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

The question of the admissibility of such an anomaly in sports is still open. The absence of one kidney as a whole does not prevent such activity, however, the fulfillment of a compensatory function by a healthy organ doubles the load on it. Therefore, the consequences of a severe injury can be fatal. In addition, the presence increased loads provokes the development of polyuria, hypertension, fatigue.

Nutrition plays an important role in maintaining the normal functioning of a single kidney. Smoked, canned, spicy, salty, flour products, any mineral and alcoholic drinks are excluded from the diet as much as possible. Coffee is better to replace herbal tea, sea. Boiled fish and meat are recommended to be consumed no more than 3 times a week. Preference is also given to oatmeal and buckwheat porridge, black and white grain bread. The use of dairy, sour-milk products is limited.

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

Maintaining a healthy lifestyle medical examination and appeal to qualified specialists with any signs indicating any deviations - all this will ensure the birth healthy baby and 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 is required. It is important to pay due attention to medical advice and regular check-ups with your doctor.

Kidney agenesis is an anatomical abnormality in which one or both of these organs are missing. If one or both kidneys are represented by underdeveloped rudiments, this condition is called aplasia.

The manifestations of the disease and prognosis usually depend on whether there is at least one of the organs and which one.

Prevalence

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

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

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

Causes of kidney agenesis

The reasons for the formation of such a pathology are not fully established, but the main and statistically confirmed ones are read:

• genetic predisposition. It is known that some families this violation occurs more frequently.
• Infectious diseases suffered by a woman while waiting for a baby. The most dangerous are influenza and rubella in the first trimester.
• Impact on the expectant mother of ionizing radiation.
• Incorrect and uncontrolled intake of hormonal contraceptives.
• Diabetes and some others endocrine diseases at future mother.
• Chronic alcohol abuse by a woman, as well as smoking and drug use.
• Venereal diseases mothers, especially.

If at least one of these problems is found in the expectant mother, special attention should be paid to regular ultrasound examinations, which will help to establish the pathology in time.

Diagnosing agenesis or aplasia of the kidneys by external signs alone will not work. Usually additional examinations prescribed if the newborn has the following symptoms:

• Too wide and flat nose;
• The auricles have a non-standard shape and are located too low;
• Face puffy with protruding frontal lobes;
• Increased distance between eyes;
• underdeveloped jawbone;
• Epicanthus is present.

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

All these signs are usually seen with bilateral renal agenesis. Also, the disease is accompanied by renal failure and the hypertension and polyuria provoked by it. Children with this diagnosis are not viable.

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

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

Fine healthy kidney begins to increase after one month of a baby's life, but growth trends appear even before birth. If a healthy organ is not capable of compensation, then the child has profuse urination leading to dehydration, regurgitation and vomiting, increased 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 is also noted that the absence of the left kidney is more often accompanied by disorders in the genital area.

Renal agenesis can be diagnosed both in utero and after the baby is born. Usually, pathology is determined using ultrasound. The lack of kidneys in the fetus is hinted at by oligohydramnios during pregnancy and the absence of an echo-shadow of the urea during a dynamic study. Agenesia is also indicated by the absence of renal arteries on color Doppler imaging.

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

• The volume of amniotic fluid in this case does not change;
• Bladder embryo can be identified;
• Sometimes the adrenal gland is mistaken for a kidney.
• If kidney agenesis is suspected, ultrasound can be done after birth. Other studies are also underway:
• excretory urography- with kidney agenesis, you can see a compensatory enlarged paired organ, and on the other side contrast agent missing;
• Nephroscintigraphy - radioisotopes do not accumulate in the affected side;
• Cystoscopy - it allows you to detect the hypoplasia of Lieto's triangle in the urea, formed by part urethra and mouths 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 may well perform all the necessary functions. It is advisable to periodically undergo examinations to make sure that everything is normal and comply with special diet. Also, some experts recommend regular courses of preventive metabolic therapy to maintain the working body.

In the treatment of diseases of other organs, patients with agenesis should choose drugs that do not affect the kidneys and are excreted mainly by the liver. In case of disruption of the only functioning organ, it may be necessary to transplant it from a donor. And the absence of two kidneys usually leads to lethal outcome there is no cure 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 prognoses. If there are no concomitant disorders, and the patient carefully monitors his health, he has every chance of living a full life.