Multicystic dysplastic kidney disease is a form of renal dysplasia that is secondary to altered metanephric differentiation during embryogenesis . This disease usually results in an enlarged, nonfunctioning kidney in the neonate. 31 August 2013 | Journal of the Renin-Angiotensin-Aldosterone System, Vol. Multicystic renal disease, Potter’s type IIA, may be bilateral and inconsistent with survival or it may present as unilateral involvement of a kidney with the contralateral kidney free of any disease.1,2 Of the reported cases of unilateral multicystic renal disease … Each cilium consists of nine circumferentially arranged doublet microtubules that are enclosed by an extension of the cell membrane (The primary cilium is sensitive to shear stress, and it affects cell proliferation and differentiation by acting as a mechanosensor or flow-sensor receptor to urinary flux. We hereby present a case of a young adult female with unilateral multicystic dysplastic kidney disease complicated by pyelonephritis versus an infected renal cyst. The cortex of the kidneys make the urine. The infant underwent a voiding cystourethrogram (VCUG) procedure, which showed no signs of vesicourinary reflux. A segmental form of the disease, which commonly involves the upper pole of a duplex kidney, also may be seen with an associated ureterocele at the end of an atretic ureter (The gross appearance of the dysplastic kidney may vary from a small hypoplastic mass to a large nonreniform multicystic lesion resembling a bunch of grapes (The imaging appearance of a multicystic dysplastic kidney depends on the age of the patient. In the past, this type was called adult polycystic kidney disease, but children can develop the disorder. Lean Library can solve it It may otherwise go unrecognised, and may be a common cause of renal agenesis, following complete involution during childhood. Increased vascularity within or around the cyst, internal echoes, and associated soft tissue or calcification are absent. Complications such as nephrolithiasis, renal calculi, and urinary tract infection may be seen (At US, an echogenic appearance of the medullary pyramids is characteristic of medullary sponge kidney. One hypothesis is that a blockage in the genitourinary tract causes a ballooning effect in the renal parenchyma as it matures and begins to form urine; the pools of fluid form multiple cysts in the kidney and normal, functional renal tissue does not develop.Multicystic dysplastic kidney is separate from other cystic renal abnormalities in that it does not seem to be a familial disorder.Prior to recent advancements in sonographic imaging, MCDK generally went undetected unless a palpable mass was noted at birth or was seen as a lump on a small child.It is important when diagnosing MCDK to determine that the cysts are noncommunicating and that they are located in the renal cortex rather than in the renal pelvis.In the past, physicians chose to perform an immediate nephrectomy on the multicystic kidney due to concerns related to the development of systemic hypertension or malignancy in the affected kidney.Due to the nature of the obstructive process of MCDK, other genitourinary complications may accompany the disorder. In the past, management of this disease usually included a nephrectomy due to concerns over complications associated with hypertension and malignancy. 66, No. A disruption in the ureteric bud–metanephros interface during embryogenesis is the hypothesized cause (Most patients with medullary sponge kidney are asymptomatic; the kidney condition is often detected incidentally at intravenous urography performed for another reason. 16, No. 6Journal of Computer Assisted Tomography, Vol. It is often bilateral, but incidental and found only on radiologic imaging studies, with an incidence of 0.5 to 1% in adults. Flow within the renal tubule causes ciliary bending, which leads to calcium influx and intracellular calcium release. Patients with nonregression may present in adulthood with a cystic renal mass. FIG. Multicystic dysplastic kidney is present in approximately 1 in 4000 neonates; early detection of the diseased kidney(s) enables planning for appropriate treatment and subsequent follow-up. In patients with autosomal dominant polycystic kidney disease, a reduction in renal blood flow parallels the increase in total kidney volume, precedes a decline in growth factor receptor levels, and is predictive of structural and functional disease progression (Medullary cystic kidney disease is a rare inherited renal disorder characterized by multiple medullary cysts and tubular-interstitial nephropathy in kidneys that are small to normal in size. Signs and symptoms of ADPKD often develop between the ages of 30 and 40. It is generally unilateral and is characterized by multiple noncommunicating cysts in the renal parenchyma. Background: Unilateral renal cystic disease is a rare condition that shares morphological similarities with multicystic dysplastic kidney, the former often distinguished from the latter on some clinical and histopathological grounds. Bilateral MCDK is fatal. Unilateral incidence is estimated at 1:2500-4000. Vesicoureteral reflux (VUR) in the contralateral kidney is the most common associated anomaly in patients with MCDK.Although sonography is the preferred imaging modality for MCDK, other types of imaging and physiological testing are usually performed due to other health issues linked to MCDK.