GIT review Flashcards
congenital malformations of the kidney
20% of renal failure in kids is due to renal dysplasia or hypoplasia
agenesis of the kidney
complete absence of one or both kidneys
results from a major disruption of metanephric development at an early stage (interaction between the ureteric bud and metanephric blastema
mix of genetic and environmental factors including infections
bilateral agenesis
potter’s syndromes
- incompatible with life
- oligohydrammois (fetal urine produced around week 16, usually contributes to amniotic fluid)
- hypoplastic lungs
patter’s syndrome also called
bilateral renal agenesis
potters facies
large low set ears wide set eyes flattening of the nose receding chin prominent skin folds over the eyes
unilateral agenesis
- usually asymptomatic
- many have other abnormalities of the genitourinary tract like vesicoureteric reflux
- ipsilateral ureter is absent or rudimentary
- the contralateral kidney undergoes compensatory hypertrophy
hypoplasia of the kidneys
small but normally developed
usually occurs unilaterally
ectopic kidney
- A Kidney not located in its usual position
- usually just above the pelvic brim or within the pelvis (resulting from failure of the kidney to ascend during embryogenesis)
- rarely seen in the thoracic cavity
- normal or slightly small, flatenned
horseshoe kidney
fusion of the upper or lower poles of the two kidneys
usually lower poles
gets hooked at the inferior mesenteric artery (lower in the abdomen than normal kidneys)
common and usually incidentally detected
polycystic disease of the kidney
polycystic disease
- autosomal dominant PKD
- autosomal recessive PKD
- glomerulocystic kidney disease
cystic disease of the renal medulla
- nephronophthisis
- medullary sponge kidney
simple renal cysts
- common finding in normal kidneys
- usually cause no symptoms or signs
- occasionally can rupture, cause haematuria, pain, abdominal mass, infection, hypertension
- major issue is excluding a more serious disorder eg. malignancy
clues to malignancy in cystic renal lesions
- multiple septa
- thicket cyst wall
- solid areas within or around the cyst
cystic renal dysplasia
- malformation of the kidney
- unilateral or bilateral
- often associated with obstruction/reflux
- consists of irregular cysts or varying sizes
- common cause of an abdominal mass in infants
histology of cystic renal dysplasia
persistence of abnormal structures like cartilage, immature mesenchyme, immature collecting ductules, with abnormal lobular organisation