Multicystic renal dysplasia is a congenital kidney disease that is not hereditary. The severity of the disease varies. A cystic kidney forms.
What is multicystic renal dysplasia?
Multicystic renal dysplasia is assigned to the group of polycystic kidney diseases (PKD). In contrast to classic polycyclic kidney diseases, however, it is not hereditary. It occurs sporadically during embryonic development and, like polycystic kidney disease, leads to a cystic kidney. See foodezine for Everything about Meniscus Injuries.
The renal pelvic calyx system is often absent. There are unilateral and bilateral forms of the disease. While only one kidney is affected in unilateral multicyclic renal dysplasia, both kidneys are affected in the bilateral form. In the unilateral form, the course of the disease is usually asymptomatic because the healthy kidney takes over all functions. If left untreated, the bilateral form leads to death due to the complete atrophy of both kidneys.
Cystic kidney diseases are first classified according to genetically inheritable and non-hereditary forms. According to Potter’s classification, the cyst formation is based on the nephron segment. Thus, multicystic renal dysplasia is classified as a non-hereditary kidney disease and according to the Potter classification as Potter IIa. Multicystic renal dysplasia is considered the most common abdominal tumor or cystic disease of the kidneys in neonates. The probability of the disease is 1 in 4300 live births.
The causes of multicystic renal dysplasia are not known. It is only known that kidney changes occur sporadically during embryonic development and are not inherited. There are also no familial clusters. In contrast, there are polycystic kidney diseases that are genetic and can be inherited in either an autosomal recessive or autosomal dominant manner.
In multicystic renal dysplasia, there is a disrupted interaction between the ureter and kidney system during embryogenesis. As a result, the individual nephrons cannot be created correctly. According to one theory, the metanephritic blastema is abnormally induced by the ureteral bud. Viral infections as a cause of the malformation are also discussed.
Symptoms, Ailments & Signs
Symptoms of multicystic renal dysplasia vary from patient to patient. In addition to asymptomatic forms, serious kidney diseases up to renal insufficiency requiring dialysis can also occur. The course depends crucially on whether only one or both kidneys are affected. Unilateral renal involvement is common. Only one kidney is affected. In the further course it comes to the complete regression of the kidney. However, since there is still a healthy kidney, there are no symptoms.
The healthy kidney assumes full kidney function. Bilateral multicystic renal dysplasia is much less common. However, it leads to complete failure of the kidneys in the context of a cystic kidney. The first manifestations of the disease appear between birth and childhood. Although unilateral manifestations are often asymptomatic, they can also manifest themselves as frequent urinary tract infections in childhood. In the bilateral form, even small children develop renal insufficiency that requires dialysis.
In the further course, spontaneous involution occurs in both forms. That is, one or both kidneys regress spontaneously. This often later leads to the misdiagnosis of renal agenesis (hereditary absence of a kidney). The ureter is also sometimes characterized by malformations, which can lead to difficulties in urinating. Sometimes there is cystic dysplasia of the testicles. In the case of bilateral multicystic renal dysplasia, without dialysis, death can occur within eight weeks as part of a fatal Potter syndrome.
Diagnosis & course of disease
The finding of a multicystic renal dysplasia can only be determined unequivocally using various diagnostic methods. A hereditary component must first be ruled out in the context of a family history. Sonography and, if necessary, CT are used as imaging methods. If there are any suspicious changes, laboratory tests are an option.
The examination of creatinine clearance and blood count is important. Furthermore, a urine diagnosis and a long-term blood pressure measurement must be carried out. The excretion function of the kidneys is determined using a urine excretion program. However, the medullary spongy kidney and the renal cyst must be excluded in the differential diagnosis.
Not every case of this disease has to lead to special symptoms or complications. In some cases, it can also be asymptomatic and therefore does not lead to any particular complications or limitations. In a malignant course, however, kidney failure occurs, so that the affected person usually dies without treatment.
They are then dependent on either a donor kidney or dialysis to survive. Life expectancy is significantly reduced in a negative course of the disease and the quality of life is reduced. It can also lead to infections or inflammation of the urinary tract of the affected person, which in most cases is associated with pain and other unpleasant symptoms.
The testicles are also affected by the disease in some cases. If the position of the kidney in the body is changed, this complaint can also lead to the death of the person concerned. In the course of treatment, a kidney transplant is usually performed.
This can lead to various complications. However, if the transplant is successful, the outcome will be positive. Whether or not life expectancy will be reduced cannot generally be predicted.
When should you go to the doctor?
If irregularities in urine output are noticed in infants or children, a doctor must be consulted immediately. In many cases, obstetricians or paediatricians can already detect disorders during the first postnatal examinations. They automatically take further steps to ensure that the baby receives adequate health care so that no complications arise.
If there are abnormalities in the amount of urine, the color of the urine or an unusual odor in the further development and growth process of the newborn child, a doctor is needed. General peculiarities of going to the toilet or changes must be observed. If they are acute, if they last for a long time or if they increase in intensity, a doctor’s visit is necessary.
In the event of pain, swelling on the body or changes in the appearance of the skin, there is a health impairment that must be examined and treated. If the person concerned suffers from inner restlessness, apathy, sleep disorders or general vegetative abnormalities, a doctor’s visit is recommended. Refusal to drink fluids, increased incidence of urinary tract infections, and convulsions should be seen by a doctor.
If an acute health condition occurs, an ambulance is required. An emergency doctor is to be alerted and first aid measures are to be initiated, since the victim is at risk of premature death. If there is a lack of interest in social activities or if other behavioral problems are noticed, it is advisable to consult a doctor.
Treatment & Therapy
Unfortunately, multicystic renal dysplasia can only be treated symptomatically. A cure for the disease is impossible. The main focus here is on prevention and early detection of severe renal insufficiency. It is therefore necessary to carry out regular checks of the laboratory values.
This applies to sonographic examinations, constant determination of the creatinine clearance, the excretion functions of the kidneys and ureters and the examination of the urine for blood and protein components. Arterial hypertension needs to be treated quickly. If permanent renal insufficiency occurs, regular dialysis must first be ensured. A kidney transplant may also be necessary.
Treatment of persistent urinary tract infections is very important. If the pain is severe, laparoscopic cystostomies are performed. Cysts filled with fluid are opened via a mirror image. However, this treatment has no effect on the course of the disease. In the unilateral forms of the disease, symptomatic treatments are usually sufficient if symptoms occur at all. Kidney transplantation is often required for bilateral multicystic renal dysplasia.
Outlook & Forecast
Doctors rate the prospects of multicystic renal dysplasia as favorable. The causes of the disease remain in the dark to this day. The development in the embryonic stage cannot be prevented either. Nevertheless, the kidneys usually shrink by themselves in the first few years of life, which eliminates the risk of kidney failure. Regular check-ups are therefore necessary in the first years of life. Surgery is only necessary if the course is severe and the symptoms are large.
Multicystic renal dysplasia occurs in 1 in 4,300 newborns. Most young children have a unilateral form, which has the best chance of recovery. The bilateral manifestation is rarer and requires a great deal of action on the part of the physicians. This is especially true for malignant tumors. Very often, children are required to undergo dialysis procedures from a young age. The question of a transplant also arises.
Failure to treat will result in death within a few weeks if the condition is bilateral. In principle, life expectancy is shortened if a tumor cannot be completely removed. There is also often a risk of a recurrence forming.
Multicystic renal dysplasia cannot be prevented. It is a congenital kidney disease, but it is not hereditary. Only the course of the disease can be monitored permanently using laboratory tests and imaging methods in order to adapt further treatments. Regular treatment of urinary tract infections and the right start of dialysis treatment can be life-saving and delay the time for a kidney transplant.
Even if no treatment is given, the kidneys should be checked regularly in order to detect urinary tract infections or the development of renal insufficiency at an early stage. There is an increased risk for both due to renal dysplasia. Annual checks should also be carried out to detect renal anemia based on blood values.
Whether the malformed kidney has been removed or not, there are a few behavioral tips that should be followed. These help care for and protect the remaining kidney. As a rule, the amount you drink should be consistently high, but sometimes there are contraindications. An individually tailored daily drinking amount can then be agreed with the doctor.
Especially in the heat and during sporting activity, you should pay particular attention to the fluid intake. All substances that unnecessarily burden the kidneys should be avoided. These are, for example, over-the-counter painkillers such as ibuprofen, diclofenac or aspirin. Nothing stands in the way of occasional use, but other medications should be used for long-term pain therapy.
Especially in the time immediately after surgical removal of the dysplastic kidney, the corresponding part of the body should be spared. This includes that heavy lifting is not allowed and sporting activities are only practiced to a limited extent.
You can do that yourself
The right nutrition can already ensure more well-being in everyday life. Since when changing your diet you not only have to pay attention to nutrients, but rather to the severity of the disease, it is advisable not to do this on your own. A mistake regarding protein and fluid levels would be fatal depending on the severity of the condition.
Rather, it makes sense to contact a nutritionist to get an individually tailored nutrition plan. This should be optimized in particular with regard to salt, protein, potassium, phosphate and sodium in order not to influence kidney function. The goal is a balanced, healthy and, at the same time, kidney-friendly diet.
In order to minimize damage to the kidneys caused by frequent urinary tract infections, it is often not enough to avoid sitting on cold surfaces. Rather, it is important to pay attention to good intimate hygiene, which does not only refer to regular washing. The choice of contraceptive and going to the toilet quickly after sexual intercourse already play a major role. In women, a reduced estrogen level can lead to an increased risk of infection, so it is advisable to have it checked at the latest during menopause and to discuss further treatment options with the gynecologist. If dialysis is not required, you can also regularly enjoy bubble tea from the pharmacy.