What is a dysplastic kidney
Multicystic kidney dysplasia
Synonyms: MCDK, multicystic renal dysplasia, diffuse renal dysplasia
The multicystic kidney dysplasia is a mostly sporadic kidney disease that belongs to the group of PKD (polycystic kidney disease). The common feature of this heterogeneous group of diseases is the formation of a cyst kidney.
According to the AAP classification of cystic kidney diseases, multicystic kidney dysplasia is a non-genetic or non-hereditary cystic kidney disease. In the now historical Potter classification, it is classified as Potter II.
The cause of the disease is unclear. The changes occur sporadically during embryonic development and are not inherited. Familial clusters are rare.
The formation of cysts is based on a disturbed interaction between ureteral and kidney anlage, which means that the nephrons are not correctly applied.
Histologically one sees cysts, fibrous stroma and primitive duct structures with a single-layer squamous epithelium. In addition, metaplastic or hyaline cartilage and, in some cases, residual parenchyma may be present.
The age at which the disease manifests itself lies between birth and childhood. There are unilateral and bilateral forms. If the disease is unilateral, urinary tract infections can be indicative in childhood, but asymptomatic courses are also possible. The more difficult bilateral variant is much rarer and leads to kidney insufficiency requiring dialysis even in childhood.
Components of the diagnostic procedure are:
8 differential diagnoses
Therapy is symptomatic. Prevention and early detection of renal insufficiency by means of regular sonographic controls, laboratory tests for renal anemia and determination of creatinine clearance, as well as urine tests for hematuria and proteinuria is important. If arterial hypertension occurs, it must be treated promptly. In the case of terminal kidney failure, dialysis procedures and possibly a kidney transplant are used. It is important to treat a urinary tract infection that occurs. A laparoscopic cystostomy is only indicated if the pain is severe and usually has no effect on the course of the disease.
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