Kidney hypoplasia is a disease that is diagnosed in children and is characterized by the complete preservation of its functions by the body with strong deviations of its size from the norm. Such an anomaly is explained by a deficit in the baby’s body of nephrons - their number can be several times more or less than standard parameters. As a result, the organ does not fully develop and it turns out much less than a full kidney.
Pathology often covers only one lobe: left or right. In addition to abnormal parameters, a small kidney is practically no different from a full-fledged organ and is even able to function normally, despite its miniature size. Deficiency is predominantly diagnosed in boys - in girls, the disease is recorded half as often.
Causes of the disease
Kidney hypoplasia in children, like any other congenital pathology, is a deviation of fetal development. Failures in the normal process of building an organ are explained by the detrimental effect of internal and external factors on the body of the expectant mother.
Kidney hypoplasia in children, the causes of which often lie in the insufficient development of blastema, can be a hereditary disease. This element is a miniature nodes formed from specific cells. If the blood flow in the blastema is impaired, it is not able to stimulate the construction of the renal ducts and other structures, against which the organ cannot form normally and acquire full dimensions.
Among the environmental factors that can lead to the occurrence of kidney hypoplasia in newborns, it is worth highlighting:
- future mothers consuming tobacco, alcohol or drugs;
- uncontrolled intake of potent drugs;
- prolonged overheating;
- adverse effects of radiation, ionization and ultraviolet radiation;
- mechanical injuries or too severe impact on the uterus - stretching, squeezing, shock;
- negative consequences of infectious pathologies transferred during pregnancy - measles, rubella, tuberculosis, flu.
Pathogenesis
As already noted, hypoplasia is a congenital abnormality of the kidney anatomy from the norm. Moreover, the violation applies only to the size of the body. What is the reason for this phenomenon? The pathological process begins in the baby, which is still in the mother’s womb. Deviations of heredity against the background of the accumulation of genetic disorders cause abnormal cell division in the first trimester, that is, during the period of active embryonic development. When it comes to kidney hypoplasia in children, it means that these structures shared too slowly. Because of this, the fetus can suffer certain intrauterine pathologies while still under the heart of the mother: for example, renal vascular thrombosis or pyelonephritis. However, the consequences of these diseases will fully manifest themselves after the birth of the baby, as an underdeveloped organ.
As for the anatomy, in the section the abnormal kidney has tissues of the cortical and brain layers that are ordinary for the renal structure, as well as a normal thin-walled artery. And this indicates the full functioning of the body, despite its miniature parameters.
Most children with kidney hypoplasia also face other serious pathologies. For example, often these babies are diagnosed with an inversion of the bladder, narrowing of the renal artery, doubling of the solitary lobe, abnormal localization of the urethra, cryptorchidism.
Varieties
Kidney hypoplasia is solitary, one-sided and two-sided. In the first case, the baby is born with one abnormal organ. Such a deviation is considered extremely dangerous, since a healthy proportion is subjected to a double load.
Bilateral hypoplasia can occur in combination with oligonephronia or dysplasia. Each of these conditions is accompanied by additional clinical signs and pathologies.
As for the one-sided view, most often the parents of a newborn baby are faced with a diagnosis of "left kidney hypoplasia in a child." What is it and what are the future forecasts? Anatomically, the left lobe is located slightly higher relative to the right, due to which, with this pathology, the clinical picture is characterized by more pronounced symptoms. Usually, a child is tormented by aching pain in the lower back. With the full functioning of the right kidney, hypoplasia of the left organ does not require specific treatment. A child needs only systematic examinations and examinations.
The disease of the right organ is practically no different from the pathology of the left lobe. Regular examinations, adherence to a gentle salt-free diet with kidney hypoplasia, a certain limitation of stress, the avoidance of viral diseases and hypothermia are such measures are important to maintain a normal lifestyle with one functioning kidney.
Symptoms
The signs of pathology in severity depend on the number of affected kidneys and the degree of deviation from the norm. If hypoplasia affects only one lobe, the patient will not have any specific symptoms due to the fact that the activity of the damaged organ will be taken over by the other side (a solitary kidney, that is, one that has remained healthy). In such situations, the risk is minimal and even the fact of renal underdevelopment can be revealed only with the help of ultrasound.
If a healthy kidney is not able to cope with increased stress, the affected organ can become inflamed. It is at this moment that the pathology will make itself felt in full. An underdeveloped kidney is more susceptible to various nephritis, especially pyelonephritis. At the onset of this disease, a clinical picture appears characteristic of it. So, a child has aching in the lower back, chills or fever. Pathology progresses, gradually distorting the underdeveloped kidney, against the background of which its normal form is lost - the body shrinks, the vessels are pinched. Permanent arterial hypertension entails an increase in total pressure to a critical level. This phenomenon often becomes the reason for the need to remove the affected kidney. After all, in fact, it is she who provokes the development of concomitant pathologies.
If the child has hypoplasia of both kidneys, the inability of the abnormally reduced organs to perform their functions provokes the occurrence of chronic failure. This condition is considered extremely dangerous and often entails the death of a child. True, hypoplasia of both organs is an extremely rare occurrence. Even with this form of pathology, one of the lobes suffers more than the second. As for the signs of incipient renal failure, they include a change in the structure of urine - it constantly increases the level of protein and urea. However, the child's blood pressure is steadily increasing.
In addition, kidney hypoplasia in children can cause the appearance of such symptoms:
- obvious lag of the baby in intellectual and physical development;
- excessive pallor of the skin, puffiness of the limbs and face;
- regularly occurring diarrhea;
- low-grade fever;
- numerous signs similar to manifestations of rickets - softening of bone tissue, flat nape, bloating, abnormal hair loss, curvature of legs, protruding parietal and frontal bones of the skull;
- chronic renal failure;
- arterial hypertension;
- regular nausea, vomiting.
Features
Kidney hypoplasia can lead to disability if pathology covers both lobes. This pathology has a poor prognosis for newborn babies up to a year. Indeed, in this case, both organs cannot function normally and are not transplantable.
Of all cases of children with hypoplasia, more than half are bilateral lesions, approximately 24% are left-sided and 20% are right-sided. Unfortunately, the statistics among babies with such a diagnosis is very sad. Approximately 20% of newborns with this disease die in the first months of their life amid cardiopulmonary failure. Infectious pathologies become the prerequisite for the death of another 30% of babies, purulent meningitis and sepsis are especially common.
Survival statistics for children with hypoplasia of both kidneys are even sadder: up to 56% of newborns die from renal failure. That is why it is simply impossible to meet an adult with such a diagnosis.
There are two periods of detection of a defect during which its symptoms can be most pronounced: immediately after birth and age over 7 years.
Diagnostics
In children, kidney hypoplasia is most often found in the first year of life (in almost 30% of all cases). And in some cases, doctors suspect abnormal organ parameters during pregnancy.
First of all, specialists conduct differential diagnosis with a wrinkled kidney and dysplasia. Hypoplasia is characterized by the preservation of normal anatomy and physiology against a background of reduced volume, as evidenced by examinations. In the process, instrumental methods are used:
- ultrasound;
- excretory urography;
- angiography;
- radiography;
- magnetic resonance imaging;
- dynamic nephroscintigraphy;
- retrograde ureteropyelography.
The unambiguous and most reliable clinical picture can be seen in the results of MRI and CT. Kidney hypoplasia in such high-quality images is clearly visible and makes it possible to assess the complexity of the pathology.
Without fail, a small patient is assigned a general analysis of urine and its bacterial culture. Among other things, a nephrologist should collect a family history and study the course of pregnancy.
In the child's body, hypoplasia manifests itself in the results of urine tests. An increased amount of protein is a serious cause for concern and the basis for a comprehensive diagnosis if the child has high blood pressure.
Preparation for the study of kidney ultrasound
A few days before the planned event, it is necessary to exclude from the child’s menu products that promote the formation of gas: whole milk, carbonated drinks, legumes, high-calorie desserts, fruits, brown bread. If the baby's body is prone to flatulence, you can take enterosorbents or enzymes several times.
If the baby is undergoing therapy with medications, you should consult a nephrologist. He will tell you how to properly prepare for the study of kidney ultrasound.
Parents should keep in mind that the procedure is performed on an empty stomach, approximately 8-12 hours after the last meal. If an ultrasound is prescribed for a newborn baby, the only thing necessary is to calculate the time so that it arrives right before the next feeding.
Treatment
Unilateral hypoplasia, which proceeds without any complications, simply does not need any specific therapy. In other cases, the doctor may suggest several treatment scenarios.
If more than a third of the excretory functionality of a healthy lobe is preserved, and there are no violations in the activity of the second organ, drug therapy can be dispensed with, which is restraining. Appropriate uroseptics and antibiotics are prescribed for the small patient, which will prevent the occurrence of inflammatory processes, as well as drugs that lower blood pressure.
If one share is healthy, and the second does not develop even a third of the required capacity, surgery is performed - nephroectomy. During this procedure, the affected organ is completely removed.
If both kidneys can not cope with their functions and can not provide a normal metabolism, against which chronic failure develops, the problem is solved by hemodialysis - temporary connection to a special drug that replaces the organ. However, it is worth noting that in this state, putting a child on its feet is possible only through transplantation.
Diet
It is very important for small patients with an underdeveloped organ to follow a certain diet. With kidney hypoplasia, doctors recommend abandoning:
- fast food;
- orange and bright yellow fruits and vegetables;
- purchased sauces, ketchups and mayonnaise;
- poorly fried meat with blood;
- exotic fruits;
- noble blue cheese;
- sweet carbonated drinks;
- raw fish, for example, in the form of rolls.
Useful products are:
- all kinds of natural goodies - seasonal fruits and berries, vegetables, meat, fish, legumes;
- honey;
- raisins;
- whole grain products;
- sour-milk treats - plain yogurt, fermented baked milk, cottage cheese, kefir, yogurt;
- foods with a lot of iron - oatmeal, rice, tomatoes, walnuts, beans, strawberries, raspberries, celery.
The same diet is recommended for women bearing a child to prevent the development of hypoplasia in the fetus.
Prevention of Complications
Against the background of the underdevelopment of a vital organ in the body of a small child, other pathological conditions may occur. Very common among children with this diagnosis:
- pyelonephritis;
- infectious malformations;
- nephrosclerosis, passing into renal failure;
- arterial hypertension.
Curing congenital abnormality is almost impossible. However, pathology itself does not always entail serious consequences and makes it possible to lead a normal lifestyle to an advanced age, without encountering serious diseases. For this, it is only necessary to adhere to several clinical recommendations for renal hypoplasia:
- healthy lifestyle;
- control of their own health, if necessary, visiting a doctor;
- avoidance of hypothermia and mechanical injuries;
- compliance with normal water and gentle salt balance.
As for the prevention of hypoplasia in infants, the expectant mother should adhere to special measures. A woman should eat well, completely abandon bad habits, protect her body from all sorts of infections and viruses.
Further prognosis for kidney hypoplasia in a child
If the pathology is diagnosed in the first months of life, attempts can be made to stabilize and correct the water-electrolyte balance, as well as to neutralize azotemia. However, with bilateral hypoplasia, infants often die in the first year of their life. On average, children with this diagnosis reach the age of 8-15 years. The kidneys simply can not cope with their functions. Children die from severe intoxication of the body due to renal failure.
It is noteworthy that with unilateral pathology, a decrease in the functions of the affected organ gradually occurs. That is why renal failure occurs.
The prognosis is usually unfavorable - children with a similar pathology live from about 9 to 15 years.
Adult patients with a diagnosis of bilateral renal hypoplasia cannot be found. But people with one affected share may well live quite a long time, but they are shown hemodialysis. Such people are recommended a strict diet, a healthy lifestyle and full compliance with all doctor's instructions.