Cystic Nierenerkrankheitenn can be congenital or acquired. Congenital disorders may be inherited as an autosomal dominant or autosomal recessive disease or other causes (eg. As sporadic mutations, chromosomal abnormalities, teratogens). Some are part of a malformation syndrome (see Table: main groups of cystic nephropathies) main groups of cystic nephropathies Krankeit Clinical features Autosomal dominant autosomal dominant polycystic kidney disease flank and abdominal pain hematuria hypertension Large kidneys with multiple bilateral cysts Extrarenal cysts (liver, pancreas, intestine) cerebral aneurysms diverticulosis abdominal hernias ESRD in adulthood, if at all Branchio-oto-renal syndrome (Melnick-Fraser syndrome) branchiale fistulas and cysts Preauricular pits or Aussackungen hearing loss familial renal hamartomas Primary hyperparathyroidism ossifying fibroma of the jaw Autosomal dominant kidney disease (ADPKD) small to standard kidney polydipsia and polyuria missing-bis- mild proteinuria Inconspicuous urine sediment No severe hypertension during the early stages nocturia or enuresis in children ESRD in adulthood Sometimes gout “Oral-facial-digital syndrome” Partly columns in lip, tongue and alveolar ridges hypoplasia of the nasal septum Von Hippel-Lindau syndrome “proliferation HEMANGIOBLASTOMA” microcysts Benign in the kidneys tuberous sclerosis tumors of the brain, kidneys, and skin angiomyolipoma of the kidney in the retina, brain, spinal cord and adrenal renal cell carcinoma pheochromocytomas Autosomal recessive Alström syndrome obesity type 2 diabetes mellitus retinitis pigmentosa Autosomal recessive polycystic kidney disease Large kidneys with multiple bilateral cysts liver fibrosis hypertension ESRD in childhood Bardet-Biedl syndrome Male hypogonadism Mental disability retinopathy obesity polydactyly Ellis-van Creveld syndrome “Short-limb-dwarfism” polydactyly Common Cardiac failure Ivemark syndrome agenesis spleen cyanotic heart disease of the intestine malrotation Jeune syndrome (leading to suffocation thoracic dystrophy) dwarfism, chest, arms and legs on Joubert syndrome Mental handicap hypotension Irregular breathing abnormalities of eye movement Meckel-Gruber syndrome Occipital encephalocele polydactyly Craniofacial dysplasia Nephronophthisis Small to normal sized kidneys polydipsia and polyuria Weak proteinuria with benign urinary sediment ESRD possible in childhood cell walls Eger syndrome (zerebrohepatorenales syndrome) brain and liver defects retardation High serum iron and copper levels hypotension Other congenital * cysts nichttubulären origin (including glomerular, subcapsular and “pyelocalycea” cysts) Various clinical features malformation syndromes Several clinical features of medullary sponge kidney Tubular extensions and cysts of the collection tubes Associated renal tubular acidosis type 1 and kidney stones Designed sihc not Unilateral to ESRD Multicystic dysplastic kidney nichtreniforme mass of cysts and of the connective tissue, with a lack of generally functioning kidney tissue Cystic renal Connected to structural urinary tract obstruction or metanephrischer malformation degree of asymmetric dysplasia between renal trisomy 18 Pervasive Developmental Disorders malformations of the head, face, the hands and feet Acquired Acquired cystic disease of multiple cysts in the context of long-term dialysis, usually after> 10 years High risk of renal cell carcinoma cysts with tumors For example, renal cell carcinoma, or Wilms’ tumor solitary simple cysts Low risk of chronic kidney disease and hypertension in the context of aging * z. B. caused by sporadic mutations, chromosomal abnormalities, teratogens or unknown mechanisms. ESRD = “end-stage renal disease.”


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