Causes of the disease
The following factors are considered to be the causes of the development of renal aplasia:
- genetic predisposition;
- infectious diseases (rubella, influenza) of the mother in the first trimester of pregnancy;
- maternal ionizing radiation during pregnancy;
- self-prescribed use of contraceptive hormonal drugs;
- diabetes;
- chronic alcoholism;
- venereal diseases (syphilis).
Kidney agenesis, kidney aplasia - types
- Bilateral anomaly (complete absence of kidneys) - bilateral agenesis or arena. As a rule, the fetus dies in utero, or the born child dies in the first hours or days of life due to renal failure. Modern methods make it possible to combat this pathology with the help of organ transplantation and regular hemodialysis.
- Agenesis of the right kidney is unilateral agenesis. This is an anatomical defect that is also congenital. A healthy kidney takes on the functional load, compensating for insufficiency as much as its structure and size allow.
- Agenesis of the left kidney is an identical case of agenesis of the right kidney.
- Aplasia of the right kidney is practically indistinguishable from agenesis, but the kidney is a rudimentary fibrous tissue without renal glomeruli, ureter and pelvis.
- Aplasia of the left kidney is an anomaly identical to the underdevelopment of the right kidney.
Variants of agenesis are also possible, in which the ureter is preserved and functions quite normally; in the absence of the ureter, the clinical manifestations of the pathology are more pronounced.
As a rule, in clinical practice a unilateral anomaly is encountered for obvious reasons - bilateral agenesis is not compatible with life.
Symptoms and course of the disease
Before the disease appears in a single kidney, this anomaly is asymptomatic.
The most common symptom is oligoanuria (decreased amount of urine, up to its complete absence).
With renal aplasia, the ureter (the tube connecting the kidney to the bladder) is most often also absent.
Sometimes there is an absence of the adrenal gland (the paired endocrine organ located above each kidney, which is responsible for the production of certain hormones).
One-sided renal colic (an attack of sharp pain due to urolithiasis), accompanied by anuria (lack of urine output), allows one to suspect the absence of one kidney.
In women, the malformation can be combined with a unicornuate or bicornuate uterus, uterine hypoplasia and vaginal underdevelopment (Rokitansky-Küster-Haser syndrome). The absence of the ipsilateral adrenal gland accompanies renal agenesis in 8-10% of cases. With this anomaly, compensatory hypertrophy of the contralateral kidney is almost always observed. Bilateral renal aplasia is incompatible with life.
Risk factors
So, aplasia and agenesis are developmental pathologies. And, as we said at the beginning of the article, the irresponsibility of the expectant mother usually leads to fetal pathologies. Smoking, drinking alcohol, taking illegal drugs, working in hazardous work can cause problems with kidney development. On the other hand, not everything depends on a woman’s behavior; no one is immune from illness, and if some serious illness affects the expectant mother, pathology may also arise.
A particular risk group is for contracting any infectious disease or sexually transmitted diseases during pregnancy (especially in the first trimester). The fetus is also at risk if a woman has chronic diseases of the endocrine system or took contraceptive medications for a long time before conception. Heredity also plays an important role.
Kidney aplasia - causes
Almost all kidney malformations occur in the prenatal stage. However, it is not always possible to detect them while the baby is in the mother’s belly. In most cases, directly violations at the stage of formation of internal organs provoke improper development of kidney tissue.
The following are provoking factors that have a negative impact on the mother’s body:
- smoking;
- alcohol;
- contact with toxic substances;
- work in hazardous production;
- exposure to ionizing rays.
Clinical symptoms
Manifestations of aplasia or agenesis depend on which kidney is affected by the pathology. It was mentioned above that if the problem affects the right kidney, this is a much more pleasant situation and the disease may not reveal itself at all throughout life. However, in some cases, symptoms are present, but mildly expressed; they can manifest themselves in the form of nagging pain in the lower abdomen, headache, increased blood pressure, a decrease or, conversely, an increase in the amount of urine, vomiting, swelling, and a general deterioration in well-being.
If the left kidney is absent, these symptoms are more acute. In addition, over time, the following complications are observed - the development of renal failure, hypertensive attacks (while the pills do not help reduce blood pressure), dehydration, nephropathy, hypertension.
In a separate line, it is worth mentioning that with left-sided aplasia and agenesis, the genitals can seriously suffer. Firstly, kidney pathology often leads to concomitant pathologies - in a boy’s child, the vas deferens may be absent, and defects of the seminal vesicles are diagnosed. In women, the uterine appendages and uterine septa are not developed. However, even if there are no pathologies in these organs, over time, problems such as pain in the groin area, difficulty ejaculating, sexual dysfunction, infertility, and impotence may arise.
The following “beacons” allow you to diagnose a defect in a newborn:
- frequent regurgitation;
- wide nose shape;
- low-set ears;
- enlarged belly;
- puffy face;
- wide-set eyes
Of course, these features do not guarantee the presence of a defect, but taken together they should attract attention.
Diagnosis of the disease
The diagnosis of renal agenesis (aplasia) can be made based on the results of computed tomography, urography and ultrasound, if the fetus has: a flat wide nose, deformed and low-lying ears, puffiness of the face, protruding frontal lobes, hypertelorism of the eyes, micrognathia, epicanthus. With renal aplasia, the following are often observed: excessive folding of the skin, deformation of the lower extremities, hypoplasia of the lungs and enlargement of the abdomen.
With unilateral renal agenesis, there are practically no symptoms of the disease, and it is diagnosed mostly by chance. Bilateral agenesis is accompanied by: hypertension, polyuria and renal failure.
Agenesis in the first days of life manifests itself in the form of symptoms: vomiting, dehydration, polyuria, hypertension and renal failure.
Diagnosis and treatment
Diagnosis begins with examining the patient and interviewing him to compile an anamnesis. Then the following tests and studies are prescribed:
- blood analysis;
- Analysis of urine;
- bacteriological culture of urine sediment;
- renal angiography;
- spiral computed tomography;
- Magnetic resonance imaging.
When the doctor has an accurate diagnosis, most often the decision is made not to carry out any therapy. Only if the pathology leads to complications expressed in certain manifestations, measures are taken, the choice of which depends on the specific problem. Usually the matter is limited to a diet that excludes fatty, fried, smoked, spicy and salty foods. Alcohol, soda, strong tea and coffee are strictly prohibited. Sometimes anti-edema medications, diuretics, and painkillers are prescribed. In addition, great importance is given to the patient’s immunity. A person with one kidney can live normally only if the immune system is maintained at the proper level.
How is the defect treated?
Kidney malformations
Treatment measures are indicated for patients with the following conditions:
- frequent severe pain in the kidney area;
- the occurrence of nephrogenic hypertension;
- reflux into an underdeveloped urinary tract.
If there is a defect in the right kidney, treatment is often not required. Patients are advised to follow a diet that reduces the load on the left organ. Therapeutic nutrition excludes fatty, spicy, fried, salty, smoked foods. The patient should avoid hot sauces, spices, and sour juices. Alcohol, carbonated drinks, strong tea and coffee are prohibited. For severe symptoms, painkillers, diuretics, and decongestants are prescribed.
For aplasia of the left organ, specific therapy is rarely carried out; more often, treatment consists of following preventive measures aimed at preventing infectious diseases. The normal functioning of a person with one kidney is ensured by maintaining immunity at the proper level.
Particular attention is paid to children with bilateral agenesis or aplasia. Early treatment of the pathology was not carried out, since the defect was a death sentence. Modern medicine performs successful kidney transplants among such infants. This gives the child a chance to live a normal life.
Important! In particularly severe cases of pathology, a person may be assigned a disability after a diagnosis is made and a decision is made by a medical commission.
Prevention
So, it is possible to live with aplasia and agenesis, but a person should always, as they say, keep their finger on the pulse - see a doctor, follow a diet, take measures to improve immunity.
However, all these problems may not exist if the expectant mother takes pregnancy planning seriously - gives up bad habits, protects herself from certain diseases, eats right, does not take any medications without consulting a doctor, avoids stressful situations, and their occurrence - to respond adequately. It is also important to stop taking hormonal contraceptives some time before conception and protect yourself with a condom.
It is especially important to pay attention to preventive measures for women who have a hereditary factor - that is, when kidney disease is not uncommon in the family.
Without the excretory system it is impossible to imagine the functioning of the human body. One of its components is a paired organ, the main task of which is to remove fluid and water-soluble elements. It is also directly involved in metabolism and regulation of acid-base balance. In the body of an absolutely healthy person, both kidneys are present. 2.5% of newborns have defects. Although rare, abnormal absence of a kidney does occur.
Prevention of pathology during pregnancy
To give birth to a healthy baby, every woman must adhere to preventive measures during pregnancy and before conception. These include:
- rejection of bad habits;
- pregnancy planning;
- timely and correct treatment of infectious diseases;
- taking medications only as prescribed by a doctor;
- maintaining proper nutrition;
- adequate assessment of stressful situations.
If a defect is detected, the patient should adhere to a healthy lifestyle and diet. If pain and other unpleasant symptoms appear, you should not self-medicate; you should consult a doctor as soon as possible.
Proper nutrition and a healthy lifestyle are the key to a full life for patients with kidney aplasia
Renal aplasia is a serious pathology that negatively affects the patient’s entire future life. Timely diagnosis of the disease and careful attention to your health will help prevent serious consequences and lead a fulfilling lifestyle.
What is renal aplasia (agenesis)?
The causes, symptoms and treatment methods for such rare diseases will be discussed below. First you need to understand what these terms mean.
In various medical reference books you can find a mixture of the two concepts “aplasia” and “agenesis”. Is there a difference between them? Agenesis is the complete absence of one or two kidneys at the same time. This pathology is congenital. In place of the missing organ there are not even the rudiments of kidney tissue. Aplasia is a phenomenon in which only a strand of connective tissue remains in the retroperitoneal cavity. In other words, this is an underdeveloped kidney that is unable to perform its function.
In clinical practice, it does not matter what form of the disease is found in the child. In both cases, a full-fledged organ is absent. Unified disease statistics are also not presented. It is known that they account for about 7-11% of all pathologies of the genitourinary system.
Diagnostics
Previously, in the absence of pronounced clinical symptoms, the absence of a kidney could be determined only by chance during examination for a completely different reason.
Diagnostics
Currently, while carrying a child, a woman is required to undergo several ultrasound scans.
Agenesis can be distinguished as early as 20 weeks of pregnancy. This allows future parents to properly prepare for the birth of a child with such a pathology.
It is necessary to obtain a consultation in advance from a pediatric nephrologist, and select a maternity hospital where doctors are familiar first-hand with renal agenesis in a newborn.
After the birth of the child, a more detailed examination is carried out using ultrasound diagnostic methods, computed tomography or magnetic resonance imaging, and angiography.
Laboratory tests of blood and urine are also done to assess the excretory function of the remaining kidney.
Historical reference
Kidney agenesis has been known to mankind since ancient times. Even Aristotle mentioned this disease in his works. He argued that if an animal cannot exist without a heart, then without a kidney or spleen it can. During the Renaissance, a scientist from Belgium became interested in this problem. In 1928, the Soviet doctor Sokolov began to identify its prevalence among the population. Since then, scientists have identified several forms of aplasia (agenesis), each of which has its own clinical picture and prognosis.
Treatment
There are no radical treatments for renal agenesis. Treatment methods are selected taking into account the patient's condition.
The birth of a baby with pathology
Particular attention is paid to children with bilateral agenesis. Previously, such a diagnosis was considered a death sentence, and a child in this condition could live no more than a few hours.
However, today it is possible to connect an infant to a hemodialysis machine and undergo an emergency kidney transplant.
Gentle diet
The chances of a successful outcome are increased if parents know about the diagnosis in advance and can prepare for the birth accordingly.
Treatment of unilateral agenesis is aimed at relieving the symptoms of complications and maximizing the preservation of the functions of the working kidney.
For this purpose, a special gentle diet is prescribed, which should be followed throughout life. It is recommended to avoid heavy physical activity and injuries.
People with agenesis are more susceptible to various inflammatory diseases of the urinary system and urolithiasis.
Therefore, regular (at least once a year, and preferably more often) examination by a nephrologist and a comprehensive examination are necessary. Maintaining the normal functioning of the immune system and protecting as much as possible from various contagious viruses and infections also plays an important role.
Kidney agenesis (renal aplasia) - development inside the womb is often accompanied by an abnormal structure of organs. These types of situations include: The bottom line is that agenesis is a congenital pathology characterized by the complete absence of a kidney or partial absence. As a result of this significant drawback, the main functions are transferred to another organ. Experts identify several types of this disease. The health and further safety of a person depends on it. Most often, this kind of anomaly is not life-threatening.
It is worth noting that kidney agenesis is the development of an organ that is distinguished by the presence of an anomaly. The process is formed during development inside the womb. As a result, the rudiment is formed with only one bud (right or left), or without buds. It is also possible that organs are present, but important elements are missing. It is worth noting that when renal agenesis is detected, the development of the ureter is not affected. There are cases when such a diagnosis did not exist.
Often the source of the problem is a defect that began its development during the period of embryonic development due to exogenous factors. Scientists have been studying this issue for a long time, and it was eventually found that there are several factors that significantly influence the occurrence of renal pathology.
- It has been scientifically proven that genetic predisposition plays a significant role in the development of this disease.
- The risk of developing pathology increases if a woman suffered an infectious disease during pregnancy. For example, I had the flu or rubella.
- Ionizing radiation has a negative effect on a pregnant woman. This factor is quite dangerous, because radioactive components, if they enter the body of a pregnant woman at least once, remain in it for a long time, crossing the barrier in the form of the placenta. Because of this process, the fetus develops while already irradiated.
- Scientists identify as a factor that accompanies the occurrence of the disease - this is the use of contraceptive drugs in an uncontrolled manner.
- If a pregnant woman is diagnosed with diabetes, then this significantly increases the risk of pathology.
- Chronic alcoholism or drinking alcohol during pregnancy increases the risk of kidney agenesis. Also, various sexually transmitted diseases, in particular syphilis and others, affect the further occurrence and development of the disease.
Types of agenesis
There are several classifications of this pathology. The main one distinguishes the disease by the number of organs.
- Right-sided renal agenesis (ICD-10 code - Q60.0). This form of the disorder is most often diagnosed in women. Typically, the right kidney is particularly vulnerable from birth. It is located below the left and is considered less mobile. In most cases, all functions are taken over by the paired organ, and the person does not experience much discomfort.
- Left-sided renal agenesis. This form of pathology is rare and is quite difficult to tolerate. The right kidney is less functional. She is not adapted to compensatory work.
Separately, it is worth considering bilateral agenesis. This type of disease is characterized by the absence of two organs at the same time. With it, babies rarely survive. After birth, they immediately require a transplant operation. What is the code for bilateral renal agenesis? ICD-10 includes this pathology under the code Q60.1.
Causes of congenital pathology
Aplasia rarely occurs in people, and this disease, which develops in utero, most often leads to the death of the baby. The disease occurs when the metanephros duct is underdeveloped and does not grow to its blastema. The anomaly is characterized by shortening or complete absence of the ureter. If the urinary organ does not take on a compensatory function, the baby dies in the womb.
With normal functioning of a healthy organ, such a pathological phenomenon can be diagnosed at an older or even elderly age. Kidney agenesis causes complications: urolithiasis, arterial hypertension, pyelonephritis. Aplasia of the left kidney or organ on the right is distinguished. Their complete absence is also possible.
Causes of the disorder
There is no consensus on the causes of renal agenesis in newborns. Doctors express different points of view. Some see a connection between pathology and genetic predisposition. Others deny this fact.
Long-term study of the disease and numerous studies have made it possible to identify several factors, the presence of which increases the likelihood of its occurrence. Aplasia or agenesis of the kidneys in the fetus is possible in the following cases:
- Chronic alcoholism, drinking alcohol during pregnancy.
- Diabetes mellitus in a expectant mother.
- Previous viral/infectious diseases in the first trimester.
- Uncontrolled use of medications by a woman for 9 months.
Kidneys begin to form in the fetus from the 5th week of its life in the womb. This process continues throughout pregnancy. However, the 1st and 2nd trimesters are considered the most dangerous. At this time, the likelihood of developing pathology, subject to exposure to unfavorable factors, is quite high.
Main reasons
According to statistics, kidney aplasia is diagnosed in 1 person out of 1200 other people.
A similar anomaly appears due to insufficient development of the metanephros duct.
The defect is accompanied by a normal or short ureter, and in some cases the child will not have one at all.
Agenesis and aplasia of the kidney are considered congenital developmental defects. Often, children with such anomalies experience death; death can occur in the womb or immediately after birth.
In situations where the second kidney can function normally and compensates for the deficiency of the second part of the paired organ, the disease can be diagnosed at an older age during a routine examination.
In some cases, the main symptoms of the pathology do not appear, their development is slow, so the abnormal structure can only be determined in old age.
Renal aplasia often causes kidney diseases, including:
- Increased intrarenal pressure.
- Pyelonephritis of various types and forms.
- Urolithiasis.
In medicine, there are several types of defects, and the least likely person will have a lack of kidneys.
This condition is the most dangerous; a child cannot live without a paired organ, so death occurs immediately after birth. Much more often, newborns do not have one kidney on either side.
Knowing what it is, it is important to understand the possible causes of the formation of a birth defect.
Doctors and scientists cannot say for sure why children are born with only one kidney, but it should be noted that genetics and hereditary predisposition do not play a role in the anomaly. Due to this, the defect is considered congenital.
The formation of a paired organ occurs from the 5th week of pregnancy. This process continues throughout development in the womb, making it difficult to determine the exact causes.
Kidney aplasia can be caused by several factors:
- Infection with viruses and other infections during pregnancy, namely in the 1st trimester. As a rule, the danger is rubella, influenza and similar diseases.
- Exposure to ionizing radiation during examination during pregnancy.
- The presence of diabetes in a pregnant woman is also a possible cause.
- The result is the use of powerful medications, hormonal drugs. The problem arises not just from pills, but from their incorrect use or independent selection of drugs for the treatment of various diseases and disorders.
- Abuse of alcohol or smoking during pregnancy and before conceiving a child, when no preparation is carried out.
- Any types of sexually transmitted pathologies in a woman.
- Use of contraceptives before pregnancy.
- Any disruption of the endocrine system.
The described reasons must be taken into account by doctors during pregnancy.
General clinical picture
Aplasia or agenesis of the kidney may not manifest itself for a very long time. Some mothers do not go for an ultrasound scan during pregnancy, and after the baby is born, they neglect tests. As a result, a person is not even aware of existing health problems. He learns about the unpleasant diagnosis years later. As a rule, during a preventive examination at school or when applying for a job. In this case, a healthy kidney takes on up to 75% of the functions of the missing or poorly functioning organ. Therefore, the person does not feel any discomfort.
Sometimes it begins to appear in a child in the first days of life. Among the main symptoms of the pathology, doctors identify:
- various facial defects (puffiness, wide and flat nose);
- a large number of folds on the body;
- voluminous belly;
- low location of the ears;
- deformities of the lower extremities;
- displacement of the position of some internal organs.
When renal agenesis is diagnosed in a child, the disease is usually accompanied by abnormalities in the development of the genital organs. For example, in girls a bicornuate uterus and vaginal atresia are detected. In boys, the vas deferens is absent. In the future, such problems may be complicated by impotence and even infertility.
Symptoms
There are several types of renal aplasia:
- The bilateral form of the anomaly is considered absolutely incompatible with life; children with such a lesion die soon after birth. The child can be saved through kidney transplantation or continuous hemodialysis;
- Aplasia of the right kidney is considered the most favorable from a prognostic point of view. Usually in such a situation, the left kidney assumes all the responsibilities of the right organ, so there may be no negative symptoms. Sometimes right-sided aplasia is detected during the diagnosis of nephropathology or persistent hypertension;
- Aplasia of the left kidney is considered more unfavorable, since with such underdevelopment of the function of the left kidney, the right one, which is considered less functional and more mobile, takes over. Renal aplasia on the left in men is usually accompanied by underdevelopment of adjacent structures such as the bladder, prostate, vas deferens or testicle. A feature of left renal aplasia is its frequent combination with underdevelopment of the lung. In women, such an anomaly is detected much less frequently, but is also accompanied by deviations of neighboring structures, for example, a bicornuate uterus or aplasia of the uterine body, absence of the ureter or appendages, vaginal duplication, underdevelopment of the septa inside the uterus.
Aplasia of the left kidney
Aplasia is less pronounced than agenesis.
In some cases, underdevelopment of the kidney does not manifest itself at all, only sometimes the patient may feel some pain in the lumbar region. Left-sided aplasia manifests itself somewhat more clearly than right-sided aplasia. In general, with such a defect the following manifestations are noted:
- Oligoanuria – a decrease in the amount of urine and even its complete disappearance;
- Underdevelopment of the ureter;
- Adrenal gland abnormalities;
- Renal colic on the affected side, caused by the proliferation of fibrous tissue;
- Nagging pain in the lower abdomen;
- Persistent hypertension;
- Sometimes pain from a normal kidney can be bothersome, which doctors associate with an increase in its size.
If aplasia is severe, then renal failure develops or the body becomes intoxicated with its own metabolites. Then the patient’s condition worsens, almost all body systems begin to function intermittently.
Diagnostic methods
When diagnosed with renal agenesis, an adult is always given a disability. However, you must first undergo a comprehensive examination. During the procedure, the doctor collects the patient’s medical history and studies his hereditary predisposition. To confirm a preliminary diagnosis, additional research methods may be required:
- Urography using a contrast agent;
- Renal angiography;
The absence or underdevelopment of kidneys in the fetus can be determined using ultrasound. Already at the first screening test, which is prescribed at 12-14 weeks, a specialist can confirm the pathology. After this, the woman is required to undergo additional examination for concomitant developmental defects.
Who to contact and how the diagnosis is carried out
If the disease is diagnosed in a recently born child, then you should contact a neonatologist. In other cases, the disease is treated by a nephrologist and urologist. If there are other pathologies, additional specialists are invited.
As part of the diagnosis, the following procedures are carried out:
- Ultrasound of the kidneys with ureters;
- urography;
- CT or MRI;
- blood and urine analysis for biochemistry.
It is enough to do an ultrasound to determine the presence of an anomaly; ideally, an ultrasound examination will be carried out in conjunction with Doppler sonography.
Therapeutic measures
The only form of the disease that is truly life-threatening is bilateral renal agenesis. Quite often, a fetus with this diagnosis dies in the mother’s womb or at the time of birth. Also, the probability of death in the first few days of life is quite high due to renal failure.
Thanks to the development of perinatal medicine, today it is possible to save a child with this pathology. To do this, it is necessary to undergo surgery in the first hours after birth and then regularly undergo hemodialysis. This method of treating the disease is quite realistic and is used in practice in large medical centers. However, initially a quick differential diagnosis should be organized and other defects of the urinary system should be excluded.
Unilateral renal agenesis has a favorable prognosis. If the patient’s pathology is not complicated by unpleasant symptoms, it is enough for him to undergo a preventive examination once a year and take the necessary tests. It is also necessary to take care of a healthy kidney. Detailed recommendations should be given by the attending physician. As a rule, they include following a diet excluding spicy and salty foods, and giving up bad habits.
Aplasia is not specifically treated. However, it is impossible to do without preventive recommendations to strengthen the immune system. It is necessary to eat right, avoid infectious and viral diseases. It is also recommended to avoid strenuous physical activity.
In severe cases of the pathological process, hemodialysis and even organ transplantation may be required. In all other cases, therapy is prescribed individually, taking into account the nature of the disease and the general clinical picture.
Treatment of kidney aplasia
With bilateral aplasia, immediate treatment is required, consisting of continuous hemodialysis and further transplantation of both kidneys.
With unilateral aplasia, the patient undergoes examination every six months with a mandatory assessment of urine excretion. A lifelong diet with limited salt and protein is recommended. The daily fluid requirement is no more than 1.5 liters.
Treatment according to syndromes:
- One kidney from birth: causes of anomaly, how to live with such a pathology
- Antihypertensive therapy - ACE inhibitors (enalapril, lisinopril), B-blockers (anaprilin, bisoprolol, metoprolol), sartans (Lozap, Lorista).
- Diuretics – furosemide, veroshpiron, triamterene.
- Antibacterial drugs in case of infection - cephalosporins (ceftriaxone, ceficom), uroseptics, protected aminopenicillins (Amoxiclav, Augmentin).
- Drugs that improve blood circulation - trental, pentoxifylline, chimes, dipyridamole.
- General strengthening therapy – vitamins B, C, E, A.
With the development of chronic renal failure, hemodialysis and surgical treatment in the form of organ transplantation are indicated.
Annual spa treatment is recommended.
Prevention methods
Aplasia and agenesis of the kidney have different ICD-10 codes. However, both of these pathologies cause concern for future parents. Is it possible to prevent their occurrence?
If we exclude a genetic predisposition to the disease, then the preventive measures are quite simple. Even at the planning stage, the expectant mother should begin to monitor her health (eat right, give up bad habits, engage in feasible sports). These recommendations should be followed even after successful conception of a baby.
If the development of pathology cannot be avoided, it is necessary to take measures to eliminate it. With bilateral agenesis, symmetrical fetal developmental delay and oligohydramnios are observed. If an anomaly is detected before the 22nd week of pregnancy, it is recommended to terminate it for medical reasons. When a woman refuses it, conservative obstetric tactics are used. It is worth noting that with unilateral absence of a kidney, a full life is possible. You only need to undergo preventive examinations and examinations occasionally.
- Causes of pathology, its types and symptoms Treatment measures
Among the anatomical anomalies that occur in humans, a congenital defect is distinguished - renal aplasia, characterized by the absence of the normal structure of the organ. Such a kidney looks like an underdeveloped process, the functional activity of which is reduced, and the work is compensated by its pair.
Agenesis (aplasia) can cause the death of the fetus while still in the womb. This pathology is diagnosed during a clinical examination or during a random examination.
Kidney agenesis refers to abnormalities of the urinary organs, which occurs during embryonic development, the consequence of which is the absence of one or both kidneys. In this case, rudimentary kidney units may not be formed, and the ureters will be developed normally or, conversely, absent.
Kidney aplasia develops when the ureters are underformed, shortened in size or completely absent. It can appear with age and can also cause various diseases: pyelonephritis, kidney stones or hypertension. In men, in addition, the vas deferens and one of the testicles on the same side may not develop in parallel.
Several factors have been identified that determine the causes of the defect:
- hereditary predisposition;
- previous infectious diseases in the 1st trimester (flu or rubella);
- irradiation of a pregnant woman;
- excess hormonal drugs;
- female diabetes mellitus;
- chronic alcoholism;
- syphilis and other sexually transmitted diseases.
Several types of anatomical abnormalities are clinically diagnosed and distinguished:
- Bilateral renal agenesis. For humans, this pathology is incompatible with life, so a child born into the world dies within a few hours. It is possible to overcome the disease by transplanting the necessary organ and performing continuous hemodialysis.
- Aplasia of the right kidney or left organ is represented by rudimentary fibrous tissue, which does not have urinary ducts, renal pelvis and glomeruli. This variant of pathology is considered more favorable than agenesis. The diagnosis can be made after a comprehensive examination and exclusion of other diseases. Signs that appear with aplasia include a nagging, mild pain in the lower abdomen or lower back.
- Agenesis of the kidney (left or right) refers to a unilateral congenital pathology. To stabilize functioning, the healthy kidney takes on the necessary part of the work. Symptoms of agenesis include copious and frequent urination. Vomiting and regurgitation, increased blood pressure, intoxication of the body, folding or wrinkling of the skin, deformation of the legs, increased abdominal volume, incomplete or partial development of the lungs may be observed. In a child, the pathology additionally manifests itself in puffiness of the face, deformation of the nose, forehead and ears.
There are forms of agenesis when the ureter remains and can perform its functions almost normally.
Diagnosis of the disease is carried out using an X-ray machine, angiography or spiral CT, MSCT are performed, magnetic resonance angiography, ultrasound diagnostics, and computed tomography can be used. Kidney aplasia cannot be detected by other methods.
- Bilateral anomaly (complete absence of kidneys) - bilateral agenesis or arena. As a rule, the fetus dies in utero, or the born child dies in the first hours or days of life due to renal failure. Modern methods make it possible to combat this pathology with the help of organ transplantation and regular hemodialysis.
- Agenesis of the right kidney is unilateral agenesis. This is an anatomical defect that is also congenital. A healthy kidney takes on the functional load, compensating for insufficiency as much as its structure and size allow.
- Agenesis of the left kidney is an identical case of agenesis of the right kidney.
- Aplasia of the right kidney is practically indistinguishable from agenesis, but the kidney is a rudimentary fibrous tissue without renal glomeruli, ureter and pelvis.
- Aplasia of the left kidney is an anomaly identical to the underdevelopment of the right kidney.
Variants of agenesis are also possible, in which the ureter is preserved and functions quite normally; in the absence of the ureter, the clinical manifestations of the pathology are more pronounced.
As a rule, in clinical practice a unilateral anomaly is encountered for obvious reasons - bilateral agenesis is not compatible with life.
Agenesis of the right kidney
According to clinical manifestations, agenesis of the right kidney is not much different from the anomaly of the left kidney, however, there is an opinion of authoritative urologists and nephrologists that the absence of the right kidney is much more common than agenesis of the left kidney, and in females. This may be due to anatomical specifics, because the right kidney is slightly smaller, shorter and more mobile than the left; normally it should be located lower, which makes it more vulnerable. Agenesis of the right kidney can appear from the first days of birth if the left kidney is not capable of compensatory function. Symptoms of agenesis are polyuria (excessive urination), constant regurgitation, which can be classified as vomiting, total dehydration, hypertension, general intoxication and renal failure.
If the left kidney takes over the function of the missing right kidney, then agenesis of the right kidney practically does not manifest itself symptomatically and is discovered randomly. The diagnosis can be confirmed using computed tomography, ultrasound examination and urography. Also, the pediatrician, as well as parents, should be wary of excessive puffiness of the child’s face, a flattened wide nose (flat bridge of the nose and a wide bridge of the nose), strongly protruding frontal lobes, and ears that are too low, possibly deformed. Ocular hypertelorism is not a specific symptom that indicates renal agenesis, but it often accompanies it in the same way as an enlarged abdomen and deformed lower limbs.
If agenesis of the right kidney does not pose a threat to health and does not manifest itself with obvious pathological symptoms, as a rule, this pathology does not require special treatment, the patient is under constant supervision of a urologist and undergoes regular screening examinations. It is helpful to maintain an adequate diet and take preventive measures to reduce the risk of developing kidney disease. If agenesis of the right kidney is accompanied by persistent renal hypertension or backflow of urine from the ureters into the kidney, lifelong antihypertensive therapy is prescribed, and indications for organ transplantation are possible.
Agenesis of the left kidney
This anomaly is almost identical to agenesis of the right kidney, except that normally the left kidney should be slightly more advanced than the right. Agenesis of the left kidney is a more complicated case, since its function should be performed by the right kidney, which is more mobile and less functional by nature. In addition, there is information, however, not confirmed by global urological statistics, that agenesis of the left kidney is more often accompanied by the absence of the ureteral orifice, this applies primarily to male patients. This pathology is combined with agenesis of the seminal duct, underdevelopment of the bladder and anomaly of the seminal vesicles.
Visually pronounced agenesis of the left kidney can be determined by the same parameters as the agenesis of the right kidney, which are formed as a result of congenital intrauterine defects - oligohydramnios and fetal compression: a wide bridge of the nose, excessively wide-set eyes (hypertelorism), a typical face with Potter syndrome - a puffy face with underdeveloped chin, low-set ears, with prominent epicanthic folds.
Agenesis of the left kidney in men is more pronounced in terms of symptoms; it manifests itself in constant pain in the groin area, pain in the sacrum, difficulty with ejaculation, and often leads to impaired sexual function, impotence and infertility. The treatment required for agenesis of the left kidney depends on the degree of activity of the healthy right kidney. If the right kidney enlarges compensatoryly and functions normally, then only symptomatic treatment is possible, including preventive antibacterial measures to reduce the risk of developing pyelonephritis or uropathology of the urinary system. A medical check-up with a nephrologist, and regular examinations of urine, blood, and ultrasound screening are also required. More severe cases of agenesis are considered an indication for kidney transplantation.
Aplasia of the right kidney
As a rule, underdevelopment of one of the kidneys is considered a relatively favorable anomaly compared to agenesis. Aplasia of the right kidney, with normal functioning of a healthy left kidney, may not manifest clinical signs throughout life. Often, aplasia of the right kidney is diagnosed randomly during a comprehensive examination for a completely different disease. Less commonly, it is identified as a possible cause of persistent hypertension or nephropathology. Only one third of all patients with an underdeveloped or “wrinkled” kidney, as it is also called, are registered with a nephrologist for aplasia during their lifetime. Clinical symptoms are nonspecific and, perhaps, this explains such a rare detection of this anomaly.
Among the signs that may indirectly indicate that a person may have underdeveloped one of the kidneys are periodic complaints of nagging pain in the lower abdomen, in the lumbar region. Painful sensations are associated with the growth of rudimentary fibrous tissue and pinching of nerve endings. Also, one of the signs may be persistent hypertension, which cannot be controlled with adequate therapy. Aplasia of the right kidney, as a rule, does not require treatment. A gentle diet is needed to reduce the risk of stress on the hypertrophied kidney, which performs a dual function. Also, for persistent hypertension, appropriate treatment is prescribed with gentle diuretics. Aplasia of the right kidney has a favorable prognosis; usually people with one kidney live a full, high-quality life.
Aplasia of the left kidney
Aplasia of the left kidney, as well as aplasia of the right kidney, is quite rare, not more than 5-7% of all patients with anomalies of the urinary system. Aplasia is often combined with underdevelopment of nearby organs, for example, with an anomaly of the bladder. It is believed that aplasia of the left kidney is most often diagnosed in males and is accompanied by underdevelopment of the lungs and genital organs. In men, aplasia of the left kidney is diagnosed along with aplasia of the prostate, testicle and vas deferens. In women - underdevelopment of the uterine appendages, ureter, aplasia of the uterus itself (bicornuate uterus), aplasia of the intrauterine septum, doubling of the vagina, and so on.
An underdeveloped kidney does not have a leg or pelvis and is not able to function and produce urine. Aplasia of the left kidney, just like aplasia of the right kidney, is called in urological practice a solitary kidney, that is, a single kidney. This refers to only the kidney that is forced to function, compensatory to do double work.
Aplasia of the left kidney is detected randomly, since it does not manifest itself with clinically significant symptoms. Only functional changes and pain in the collateral kidney can give rise to a urological examination.
The right kidney, which is forced to perform the work of the aplastic left kidney, is usually hypertrophied and may have cysts, but most often it has a completely normal structure and completely controls homeostasis.
Aplasia of the left kidney in both children and adults does not require specific treatment, with the exception of preventive measures to reduce the risk of bacterial infection in a single solitary kidney. A gentle diet, maintaining the immune system, and maximally avoiding infection with viruses and infections ensures a completely healthy, fulfilling life for a patient with one functioning kidney.
July 19, 2020
Congenital malformations of the urinary system include renal agenesis. The term implies the complete absence of a paired organ; the remaining kidney begins to hypertrophy in order to take on functional responsibilities. The cause of the pathology is an exogenous effect on the embryo during embryonic development.
Reasons for the development of the disease
Aplasia is formed in a situation where the metanephros canal cannot grow to the metanephrogenic blastema. Despite this, the ureter may remain normal, be shortened, or be absent altogether. The complete absence of the ureter in males is combined with the absence of the vas deferens, cysts on the seminal vesicle, absence or reduction of the testicle, etc.
The following etiological factors can be considered confirmed through statistical data and clinically established:
- Hereditary predisposition.
- Infectious pathologies in women identified in the first three months of pregnancy - influenza or rubella.
- Ionizing radiation of pregnant women.
- Uncontrolled consumption of contraceptive hormonal drugs.
- Diabetes mellitus during pregnancy.
- Alcoholism.
- Syphilis and other sexually transmitted pathologies.
Characteristics of the anomaly
Renal agenesis refers to the absence of one or two organs. Pathology belongs to the category of anatomical quantitative anomalies, when part of the child’s urinary system has not formed during the prenatal period.
Kidney aplasia is an anomaly that occurs with similar symptoms and has similar causes. In the case of aplasia, the organ is presented as a rudiment in the form of a connective tissue cord, which does not have a full-fledged stalk and pelvis. Due to the lack of natural kidney structure, it is unable to function normally. In both agenesis and aplasia, the second organ compensatory takes on all the work. Due to the increased daily load, its tissues hypertrophy, the kidney increases in size.
The cause of agenesis is not a hereditary factor, only developmental anomalies during the period of intrauterine maturation. In any case, the conditions for the appearance of this type of defect can be the following factors that are activated during pregnancy:
- diseases caused by infection at the initial stage of embryo development;
- diagnostic examinations based on radiation;
- alcohol abuse by pregnant women;
- sexually transmitted diseases;
- drugs with a high content of hormones.
If the expectant mother has a disease such as diabetes, the risk of pathology in the child increases significantly; the development of the fetus inside the womb is especially closely monitored.
Who doesn’t need treatment, and who needs a transplant?
After the examination has been carried out and all the causes of the disease have been identified, the patient is given recommendations on how to correct the situation.
If unilateral renal agenesis/aplasia does not show any symptoms and the healthy organ fully copes with its function, treatment is not required.
However, the patient should adhere to a gentle diet and periodically undergo a comprehensive kidney examination.
To reduce the risk of developing pyelonephritis, as well as support the immunological state of the body, the specialist prescribes symptomatic therapy, which consists of prescribing vitamins, diuretics and preventive antibacterial measures.
As a rule, people with one healthy kidney live full lives.
If agenesis is accompanied by persistent impairment of renal function, backflow of urine into the ureter, and hypertension, then the patient may be recommended lifelong treatment or organ transplantation.
Patients usually agree to the transplant. As a rule, this option is possible with agenesis of one of the kidneys - left or right.
Aplasia (underdevelopment) is considered a milder pathology and is detected completely by chance during examination for another disease.
Despite the fact that aplasia practically does not manifest itself in any way, patients may complain of abdominal pain.
An aplastic organ can cause renovascular hypertension. The disease appears suddenly. Men suffer after 50 years of age, women - before 30 years of age.
Kidney aplasia is usually accompanied by underdevelopment of nearby organs. In men, these are the urinary system, genitals and lungs. In women, it is expressed in underdevelopment of the uterus and its appendages, doubling of the vagina, and aplasia of the septa.
Agenesis is considered a more severe case in medical practice. With such a pathology, the right organ must take over the entire function, but this is complicated by the fact that it is more mobile and less functional.
Patients may suffer from difficulty ejaculating, problems in sexual life, and in more severe cases, the disease leads to infertility and severe nephritis.
The disease can provoke the development of urolithiasis, pyelonephritis, and arterial hypertension.
When unilateral aplasia is detected during pregnancy, preventive measures include preventing exposure to teratogenic factors.
These include:
- bad habits (smoking, alcohol, taking drugs);
- ionizing radiation;
- medications and other factors that can harm the life and health of the baby.
If an anomaly is detected during the examination, the patient is recommended to adhere to a healthy lifestyle, follow a gentle diet that reduces the load on the kidney, which performs a dual function, and also requires observation by a nephrologist at least once a year.
If agenesis/aplasia is asymptomatic and does not pose a threat to health, the pathology does not require special treatment and patients can enjoy a full life.
In case of pain or other unpleasant symptoms, it is recommended to seek medical help to avoid complications. Only a specialist, after a thorough examination, prescribes qualified treatment.
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Forms of development of the defect
Bilateral agenesis in the medical field is less common than unilateral agenesis, since the absence of both organs of the urinary system is incompatible with the patient’s life. Unilateral pathologies of abnormal kidney development:
- Right-sided agenesis. It makes up a small proportion of all pathologies and represents an anomaly in the structure of the right kidney.
- Agenesis of the left kidney. It is rare, no more than 7% of all clinical cases. Like the right-sided one, it can occur with or without preservation of the ureter.
These types of congenital defects differ little from each other. In both cases, the remaining kidney hypertrophies, increases in size and adapts to dual function.
Agenesis of the right kidney may be misdiagnosed as aplasia. This is due to the fact that the ureter may be present and even function successfully. At the same time, this pathology is more common; this is associated with the anatomical location of the right kidney. The organ is located lower, it is more mobile and slightly smaller in size.
Who doesn’t need treatment, and who needs a transplant?
After the examination has been carried out and all the causes of the disease have been identified, the patient is given recommendations on how to correct the situation.
If unilateral renal agenesis/aplasia does not show any symptoms and the healthy organ fully copes with its function, treatment is not required.
However, the patient should adhere to a gentle diet and periodically undergo a comprehensive kidney examination.
To reduce the risk of developing pyelonephritis, as well as support the immunological state of the body, the specialist prescribes symptomatic therapy, which consists of prescribing vitamins, diuretics and preventive antibacterial measures.
As a rule, people with one healthy kidney live full lives.
If agenesis is accompanied by persistent impairment of renal function, backflow of urine into the ureter, and hypertension, then the patient may be recommended lifelong treatment or organ transplantation.
Patients usually agree to the transplant. As a rule, this option is possible with agenesis of one of the kidneys - left or right.
Aplasia (underdevelopment) is considered a milder pathology and is detected completely by chance during examination for another disease.
Despite the fact that aplasia practically does not manifest itself in any way, patients may complain of abdominal pain.
An aplastic organ can cause renovascular hypertension. The disease appears suddenly. Men suffer after 50 years of age, women - before 30 years of age.
Kidney aplasia is usually accompanied by underdevelopment of nearby organs. In men, these are the urinary system, genitals and lungs. In women, it is expressed in underdevelopment of the uterus and its appendages, doubling of the vagina, and aplasia of the septa.
Agenesis is considered a more severe case in medical practice. With such a pathology, the right organ must take over the entire function, but this is complicated by the fact that it is more mobile and less functional.
Patients may suffer from difficulty ejaculating, problems in sexual life, and in more severe cases, the disease leads to infertility and severe nephritis.
The disease can provoke the development of urolithiasis, pyelonephritis, and arterial hypertension.
When unilateral aplasia is detected during pregnancy, preventive measures include preventing exposure to teratogenic factors.
These include:
- bad habits (smoking, alcohol, taking drugs);
- ionizing radiation;
- medications and other factors that can harm the life and health of the baby.
If an anomaly is detected during the examination, the patient is recommended to adhere to a healthy lifestyle, follow a gentle diet that reduces the load on the kidney, which performs a dual function, and also requires observation by a nephrologist at least once a year.
If agenesis/aplasia is asymptomatic and does not pose a threat to health, the pathology does not require special treatment and patients can enjoy a full life.
In case of pain or other unpleasant symptoms, it is recommended to seek medical help to avoid complications. Only a specialist, after a thorough examination, prescribes qualified treatment.
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Signs of abnormal development
The absence of one of the organs affects the work of the other with increased load. Because of this, agenesis may often not be noticed in a newborn, and the anomaly will be detected only after some time with the help of diagnostic measures (computed tomography, ultrasound or urography).
The child may exhibit the following external signs:
- some puffiness of the skin of the face;
- slightly flat nose - the bridge of the nose is wider than usual, together with a flat back;
- the lobes of the frontal zone are quite convex;
- ears located below normal levels or with deformed areas.
Agenesis on the left side has more pronounced symptoms:
- the volume of urine per day is significantly increased;
- frequent urge to urinate;
- feeling of nausea, possibly vomiting;
- pain syndrome in the groin area.
In males, agenesis is more common due to the structural features of the genitourinary system. Specific symptoms are expressed due to the absence of a duct, which is responsible for the removal of seminal fluid and is caused by pain in the groin area during ejaculation, the sacral region, sexual dysfunction (impotence) and infertility are possible.
In most cases, female agenesis is accompanied by various pathologies of the uterus; vaginal underdevelopment or organ hypoplasia is possible.
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Main symptoms
Kidney aplasia always differs from agenesis in less pronounced signs and the strength of their manifestations. Often the defect passes without characteristic manifestations.
Compared to a right-sided or left-sided problem, the symptoms will be stronger if the left kidney is affected. The main ones are:
- An increase in the volume of urine released throughout the day.
- Frequent urge to urinate.
- Attacks of vomiting.
- An increase in blood pressure that cannot be controlled with special medications.
- The appearance of folds on the body.
- Development of pain that radiates to the lumbar region and sacrum.
- The appearance of sexual dysfunction in men.
- General signs of deteriorating health and well-being.
- Swelling on the face.
To determine a pathological formation in childhood, parents need to monitor the presence of the following symptoms:
- Puffiness of the face.
- A wide nose that has a flattened back.
- Low position of the ears.
- Large belly size, which is not typical for children.
- The appearance of folds on the skin.
- The placement of the eyes is excessively wide.
The described visual features indicate an anomalous structure, but this can only be confirmed after a comprehensive diagnosis.
The described condition does not always indicate a defect. In children with a severe course of the anomaly, signs of severe intoxication as a result of metabolic processes appear, and failure of the paired organ develops.
Treatment and prevention
If the only healthy kidney functions normally, there are no symptoms and a characteristic enlargement of the organ is observed, then doctors will not take any additional actions to improve the functioning of the urinary system. The nephrologist will only prescribe dynamic monitoring of the kidney’s ability to produce and excrete urine, regular urinalysis and ultrasound examination.
Preventive actions are also provided that are aimed at preserving the functionality of the kidney:
- a diet that helps reduce the load on the only healthy organ is prescribed by a doctor, taking into account the individual characteristics of the patient;
- restrictions associated with contact sports (boxing, wrestling) to protect a healthy organ from injury and unnecessary damage;
- try not to lift heavy objects;
- maintain a constant body weight, avoid sudden weight loss;
- give up alcohol abuse and nicotine addiction;
- support the immune system with vitamins and minerals;
- avoid hypothermia to avoid the occurrence of infectious processes.
In the absence of function of the second kidney or with severe development of agenesis, when a healthy organ cannot cope with its work, intoxication with decay products and toxic substances occurs. In this case, hemodialysis and kidney transplantation are prescribed.
Therapy methods
Treatment of renal agenesis has several directions and is often carried out with the goal of saving a person’s life. This includes both medication and surgical intervention (in certain cases) for transplantation.
Traditional methods
Conservative medicine offers patients:
- follow a strict diet;
- stop drinking alcohol and caffeine;
- reduce the load on the organ.
You will have to give up salty, fried, fatty, protein-rich foods, maintain a drinking regime and strengthen the functioning of the organ with the help of the following medications:
- Diuretics are a group of drugs that have a diuretic effect; they are prescribed only when diuresis decreases or urine outflow is impaired.
- Herbal medicines that improve kidney function are a number of drugs that stimulate the filtration function, significantly improve the functioning of the organ, and compensate for the patient’s condition. Such drugs include Canephron.
Depending on the person’s condition, medications that lower blood pressure levels are also prescribed, if such an adjustment is appropriate.
ethnoscience
This is the use of decoctions of herbs and plants that improve the function of the urinary system. You can use any herbs that have a diuretic effect on the body:
- birch leaves;
- juniper;
- knotweed;
- nettle;
- lingonberry;
- rose hip.
From the above components you can prepare a medicinal mixture, mix them in equal proportions (5 grams each), pour boiling water (1 liter), leave for 2 hours in a thermos or 20 minutes in a water bath. Then strain, cool and take 300 ml 3 times a day.
Help for pregnant women
If a woman was diagnosed with aplasia during pregnancy, then there is a high probability of encountering edema and a complication such as gestosis during pregnancy.
This can be avoided with the help of corrective drug therapy; the following medications are recommended:
- Phytolysin;
- Canephron;
- Cyston.
Proper nutrition and drinking regimen will help support treatment.
Sources used:
- https://m.ilive.com.ua/health/aplaziya-ageneziya-pochki_75932i15945.html
- https://pocki.top/lekarstva/aplaziya-pochki-chto-eto-takoe/
- https://tvoyapochka.ru/bolezni/aplaziya
- https://himeq.ru/aplaziya-pochki/
- https://urohelp.guru/pochki/anomalii/aplaziya.html
- https://womanadvice.ru/aplaziya-pochki-prichiny-vidy-i-proyavleniya-patologii
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Brief description of the disease
There are no general statistical data on this matter, but among pathologies of ureteral development, about 10% include this anomaly. The absence of one organ in a child corresponds to a proportion of 1:700; calculations were made taking into account that there are a thousand children. If we are talking about the absence of not one kidney, but two organs, then a child is born with such a pathological feature, one in four to ten thousand.
It is worth saying that the absence of one kidney may not be noticeable for several years of a child’s life. This is possible if the mother did not do an ultrasound or take tests with the baby. Such cases are not uncommon when the patient learned about his pathology during school or work examinations.
Important! If there is a lack of one organ, the second kidney performs its functions with 75% of the other organ. Therefore, a person may not feel anything special for a long time. It is also possible to determine the development of renal anomalies after the birth of the child.
Experts identify the following rules for correct diagnosis:
- a child who has kidney disease exhibits external facial defects. This is a non-standard nose: a wide or flat nose. This may be ocular hypertelorism, or a puffy face;
- Agenesis is determined by the location of the ears. If they are low or bent strongly enough, this is a sure sign. Interestingly, the curve on the ear indicates which side the bud is missing;
- the child has many folds on the body and a large belly;
- pulmonary hypoplasia is diagnosed, that is, the respiratory organs are quite small in size;
- the baby's legs are deformed;
- The internal organs are displaced, which is why the baby suffers from vomiting and hypertension. Against this background, dehydration occurs.
In children with renal agenesis, the ureteral system is not observed, and pathologies are present in the genital organs. A woman suffers from a uterine defect, namely unicornuate or bicornuate uterine hypoplasia.
Boys who were born with this pathology have problems with the vas deferens, or rather its absence. This is fraught with groin pain, or painful sensations during ejaculation. In the future, this can lead the young man to infertility and impotence.
Treatment
So, what is renal agenesis and how to treat such a pathology? As it turned out, agenesis is an abnormal development of the kidneys, which is characterized by the absence of one or two kidneys. Only one type is dangerous to the life of a child - a bilateral form of pathology. After all, most often a child with such a diagnosis does not survive. If you perform a kidney transplant in the first hours of a baby’s life, and then constantly undergo hemodialysis, you can really save a life. Only organized and fastest possible actions by specialists will help save the child.
The most favorable form of agenesis is the one-sided form. In this case, you need to undergo examinations every year, follow a proper diet and moderate physical activity for the normal functional functioning of one kidney.
It is quite rare for doctors to issue a disability certificate for such a pathology. The disability group depends on the severity of the abnormal development.
Forms of the disease
There are several types of agenesis:
- unilateral with ureter;
- unilateral without ureter;
- bilateral.
Bilateral renal agenesis belongs to the third clinical type and is quite rare.
Newborns, mostly boys, are born dead. Sometimes a baby is born healthy, but literally dies in the first days of life due to kidney failure.
The cause of death is usually the absence of the urinary system, insufficient development of the lungs, or other signs.
If bilateral agenesis is detected, termination of pregnancy is recommended, regardless of the period. If it is not possible to accurately determine an abnormality, for example, hypoplasia, bilateral agenesis or dysplasia, the pregnancy is still terminated.
If this is not done, the pathology can lead to oligohydramnios, which can cause severe kidney problems.
If the disease is detected early, organ transplantation and hemodialysis are possible.
Unilateral agenesis of the left/right kidney with preservation of the ureter belongs to the first clinical type, a congenital anomaly.
With this development of the disease, the load falls entirely on the second kidney, so it has an increased volume (hyperplasia). However, the risk of complications from injury remains.
Agenesis of the right kidney occurs more often and mainly in women.
In this case, constant monitoring by a doctor - a urologist or nephrologist, diet and regular screening examinations are required.
Unilateral agenesis without a ureter appears in the first stages of development of the genitourinary system. The anomaly is expressed in the absence of the ureteric orifice.
If the pathology is detected in men, then it is combined with the absence of ducts that remove seminal fluid.
The disease is characterized by painful sensations in the groin area, sacrum, possible sexual dysfunction and pain during ejaculation.
Features and types of aplasia
Aplasia of the right/left kidney is similar in nature to agenesis, but the kidney looks like an underdeveloped rudiment without a pelvis and stalk. Boys are more often affected.
Aplasia major and minor are distinguished:
- When large , the kidney is presented in the form of fibrolipomatous tissue with small cysts. The ureter and nephrons were also not identified.
- In the small form , nephrons are present in small numbers, a ureter with an orifice is present, but it is very thin and often does not reach the renal parenchyma.