Acronym for Congenital Anomalies of the Kidney and Urinary Tract.

Includes the developmental anomalies of the kidney and the urinary tract.

Non-syndromiques anomalies:

• renal genesis
• congenital anomaly of the urachus
• duplication of the urethra
• renal tubular dysgenesis
• renal dysplasia
• polycystic kidney disease
• epispadias-bladder exstrophy
• congenital hydronephrosis
• renal hypoplasia
• congenital megacalicosis
• congenital primitive megaureter
• lower urinary tract obstruction
• oligomeganephronia
• spongy kidney
• megabladder-megaureter syndrome

Syndromic anomalies :

• autosomal dominant acro-osteolysis 
• renal, genital and middle ear anomalies
• VACTERL/VATER association
• acro-pectoro-renal dysplasia
• axial mesodermal dysplasia
• thymus-kidney-anus-lung dysplasia
• carpo-tarsal multicentric osteolysis with or without nephropathy
• caudal regression sequence
• acrorenal syndrome
• AREDYLD syndrome
• BNAR syndrome
• BOR syndrome
• cerebrorenodigital syndrome
• CHARGE syndrome
• Alagille Syndrome
• aniridia-renal agenesis-psychomotor retardation syndrome
• arthrogryposis-renal failure-cholestasis syndrome
• atherosclerosis-deafness-epilepsy-diabetes-nephropathy syndrome
• Beckwith-Wiedemann syndrome
• cataract-nephropathy-encephalopathy syndrome
• 22q11.2 deletion syndrome
• dyschondroosteosis-nephropathy syndrome
• renal pelvicaliceal dysmorphism-deafness syndrome
• autosomal dominant renal dysplasia-infundibulo-pelvic stenosis syndrome
• Ellis-Van Creveld syndrome
• Mayer-Rokitansky-Küster-Hauser syndrome
• Meckel syndrome
• nephrosis-deafness-urinary tract and fingers abnormalities syndrome
• Noonan syndrome
• Pallister-Hall syndrome
• spastic paraplegia-nephropathy-deafness syndrome
• Rubinstein-Taybi syndrome
• Schinzel-Giedion syndrome
• Simpson-Golabi-Behmel syndrome
• Smith-Lemli-Opitz syndrome
• Steinfeld syndrome
• Thomas syndrome
• Townes-Brocks syndrome
• Turner syndrome 
• hydrocephalus-blue sclerae-nephropathy syndrome
• hypoparathyroidism-deafness-renal dysplasia syndrome
• intestinal hypoperistaltism-microcolon-megabladder syndrome
• fibula-ulna hypoplasia-kidney anomalies syndrome
• ichthyosis-intellectual deficit-dwarfism-kidney anomaly syndrome
• Ivemark syndrome
• Ochoa syndrome
• Cat-eye syndrome
• Ulbright-Hodes syndrome
• double uterus-hemivagina-renal agenesis syndrome
• EEC syndrome
• faciocardiorenal syndrome
• renal cysts-diabetes syndrome
• Meckel-like syndrome associated with NPHP3
• neurofaciodigitorenal syndrome
• oculosqueletorenal syndrome
• orofaciodigital syndrome type 1
• kidney-coloboma syndrome
• thyrocerebrorenal syndrome
• WAGR syndrome
• Trisomy 13
• Trisomy 18

References : 

-         Toka R, Toka O, Hariri A, Nguyen HT.
Congenital anomalies of the kidney and urinary tract.
Seminars in Nephrology 2010; 30: 374-86.

Updated: April 2017