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CONGENITAL DISEASES IN RENAL-URINARY TRACT - Coggle Diagram
CONGENITAL DISEASES IN RENAL-URINARY TRACT
DYSPLASTIC KIDNEY
Malformation of the renal parenchyma
characterized by cysts and abnormal tissue
Unilateral!!
Bilateral??→descartar Inherited PKD
POLYCYSTIC
KIDNEY DISEASE (PKD)
Bilateral enlarged kidneys
with cysts in the renal cortex
and medulla
AR
Presents in infants → worsening renal failure + Hypertension
Presents in newborns →Potter sequence
Associated with congenital hepatic fibrosis + hepatic cysts→Portal hypertension.
AD
Presents in young adults → Hypertension (↑renin) + hematuria + worsening renal failure
Mutation APKD1 APKD2; Cysts develop over time
Associated with berry aneurysm, hepatic cysts, mitral valve prolapse.
RENAL AGENESIS
Absent kidney
formation; UNI/BI lateral
UNILATERAL: asymptomatic + compensatory hypertrophy contralateral kidney → Hyperfiltration ↑↑ risk renal failure
Anomalies in contralateral kidney are common
BILATERAL: Potter sequence→ oligohydramnios + flat face (retrognathia, flattened nose) + low set ears +limb deformities + lung hypoplasia (cause of death)
Etiology:
Chronic placental insufficiency
Reduced renal output: ARPKD,
obstructive uropathy
MEDULLARY CYSTIC
KIDNEY DISEASE
Cysts in the medullary
collecting ducts
Parenchymal fibrosis →shrunken kidneys
& worsening renal failure
HORSESHOE KIDNEY
Conjoined kidneys
connected at the lower pole
Gets caught on the inferior mesenteric artery root during its ascent from the pelvis to the abdomen
Kidney is abnormally located in the lower abdomen
Function normally BUT→ hydronephrosis (ureteropelvic junction obstruction) + renal stones + infections + ↑ risk of renal ca.
↑ incidence in aneuploidy: Turner (X0), Down (21), Edward (18) and Patau (13)
most common congenital
renal anomaly