CHAPTER Tubulointerstitial Disease General Features PRINCIPAL CYSTIC DISEASES OF THE KIDNEY NongeneticAcquired disordersSimple renal cystssolitary or multipleCysts of the renal sinusor peripelvic l ID: 944040
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Cystic Diseases of the Kidneykidney cyst is a fluid-filled sac arising from a dilatation in anypart of the nephron or collecting duct. A sizable fraction of allclassified as cystic diseases of the kidney. In fact, the commonlyassociated with extrarenal manifestations of the inherited defect,ally are not part of a systemic disease.characteristics that enable correct identification of each entity. SomeMost of these features are illustrated in this chapter.Yves Pirson Dominique Chauveau CHAPTER Tubulointerstitial Disease General Features PRINCIPAL CYSTIC DISEASES OF THE KIDNEY NongeneticAcquired disordersSimple renal cysts(solitary or multiple)Cysts of the renal sinus(or peripelvic lymphangiectasis)Acquired cystic kidney disease(in patients withchronic renal impairment)Multilocular cyst (or multilocular cystic nephroma)Hypokalemia-related cystsDevelopmental disordersMedullary sponge kidneyMulticystic dysplastic kidneyPyelocalycea
l cystsGeneticAutosomal-dominantAutosomal-dominant polycystic kidney diseaseTuberous sclerosis complexvon Hippel-Lindau diseaseMedullary cystic diseaseGlomerulocystic kidney diseaseAutosomal-recessiveAutosomal-recessive polycystic kidney diseaseNephronophthisisX-linkedOrofaciodigital syndrome, type I FIGURE 9-1Principal cystic diseases of the kidney.Classification of the renal cystic disorders,Adapted fromFick and Gabow [1];Welling and Grantham [2]; Pirson, IMAGING CHARACTERISTICS OF THE MOST COMMON RENAL CYSTIC DISEASES DiseaseSimple renal cystsAcquired renal cystic diseaseMedullary sponge kidneyARPKDKidney SizeNormalMost often small, sometimes largeNormal or slightly enlargedEnlargedEnlargedCyst SizeVariable (mmÐ10 cm)Variable (mmÐ10 cm)mm increase with agemmÐ2 cm (when present)Cyst LocationPrecalycealMedullaryNormalNormalNormal (most often)Cysts (most often)Normal FIGURE 9-2Characteristics of the most common renal cystic d
iseases detectableby imaging techniques (ultrasonography, computed tomography,magnetic resonance). In the context of family history and clinicalin the vast majority of patients. ADPKDÑautosomal-dominant Cystic Diseases of the Kidney FIGURE 9-3Solitary simple cyst. Large solitary cyst found incidentally at ultra-ney. Criteria for the diagnosis of simple cyst include absence ofwalls, bright posterior wall echo (). The latter occur becausethe adjacent parenchyma. If these criteria are not satisfied, comput- Nongenetic Disorders PREVALENCE OF SIMPLE RENAL CYSTS DETECTED BY ULTRASONOGRAPHY Age group, y*Unilateral or bilateral. MÑmale; FÑFemale. 2 Cysts*3 Cysts*1 Cyst in Each KidneyPrevalence, % FIGURE 9-4Prevalence of simple renal cysts detected by ultrasonographyaccording to age in an Australian population of 729 personsprospectively screened by ultrasonography. The prevalence with age. Most simple cysts are located in the cortex.