Test 3 Flashcards
in the 7th week, what does metanephros develop from?
- metanephric diverticulum (uteric bud)
- metanephric mass of the intermediate mesoderm
what is the uteric bud?
outgrowth of the mesonephric bud
what does the uteric bud give rise to?
- ureter
- renal pelvis
- calyces
- collecting tubules
by when dp the kidneys ascend to the adult position?
11 weeks
when do the kindeys start to produce urine?
11 weeks
at the 9th week what does the cloaca divide into?
- rectum posteriorly
- urogenital sinus anteriorly (female and most of male urethra, urinary bladder)
what is the bladder continuous with?
allantois
what does the allantois become?
a fibrous cord, the urachus, which extends from the apex of the bladder to the umbilicus
when is renal architecture finalized?
between 5-15 weeks of intrauterine development
what does the urachus become?
medial umbilical ligament
what does the bladder form from?
devlops from part of the upper part of urogenital sinus
when are female and male genitalia identical?
befpre 11 weeks
in males, when does testicular descent occur?
after 25 weeks gestation
what does evaluation of the AVF provide important info on?
fetal renal and placenta function
when does fetal urine production become a major source of amniotic fluid?
after 16 weeks
what is fetal lung development affected by?
- amniotic fluid adequancy
- available thoracic space
- neuromuscluar functions
what growth factors is lung development regulated by?
fluid volume and hyperoxia to cause cellular proliferation and differentiation
what is an important component of amniotic fluid?
fetal urine
during fetal development what is the kidney a major source of?
proline
what is proline?
aids in the formation of collagen and mesenchyme in the lung
what is one of the most frequent sites of congenital anaomalies?
urinary tract
what does VATER stand for?
- vertebral defect
- anal atresia
- tracheo-esophageal fistula
- radial defects and renal anomlaies
what does VACTERL stand for?
- vertebral defects
- anal atresia
- cardiac defects
- tracheo-esophageal fistula
- radial defects and renal anomlaies
- limb abnormalities
Bilateral renal agenesis is a_____congenital anomaly
lethal
describe bilateral renal agenesis?
- male predomincance
- uteric bud fails to develop
- nephrons do not form
- no urine is produced
- severe olighydraminos
- pulmonary hypoplasia is the major cause of death
what is potter sequence or syndrome?
case that presents with oligohydramnios or anhydramnios regardless of the source of the loss of amniotic fluid. (bilateral renal agenesis)
what is potter syndrome characterized by?
- renal failure
- severe oligohydramnios
- face
- limb deformities
- IUGR
what does the face look like with potter syndrome?
- beaked nose
- low set ears
- prominent epicanthic folds
- hypertelorsim
what are the sonographic findings for bilateral renal agenesis?
- no visualization of fetal kidneys
- no visulization of fetal bladder
- before 16 wks, AVF is not dependant upon fetal renal function and fetal urine may be normal
- after 16 wks, severe olgohydramnios
- lying down adrenal gland
- absent rena; arteries using color doppler
what may be mistaken for kidneys?
bowel or adrenal glans in the renal fossae
what may mimic the bladder?
urachal diverticulum
what excludes bilateral renal agenesis?
visualization of a normal fetal bladder
is unilateral or bilateral renal agenesis more common?
unilaterl (4x)
what is unilateral renal agenesis associtaed with?
- normal AVF
- normal visualization of bladder
what is a pitfall when imaging kidneys?
renal in far field because acoustic shadowing from the spine in TRV
if a kidney is not found in the renal fossa what will the other one look like?
contralateral kidney may be enlarged
what may happen with unilateral renal agenesis?
Vesicoureteral reflux may occur
what are associated abnormalities with unilateral renal agenesis?
- genital
- cardiac
- skeletal
- GI
where may renal ectopia be placed?
- pelvic
- crossed
- crossed fused
- horeshoe
what is the most common renal ectopia?
pelvic kidney
the ectopic kidney may be __________
hypoplastic or dysplastic
what is horse shoe kidney?
bridge of renal tissue connecting the lower poles
what does horseshoe kidney have a higher incidence in?
- vesicoureteral reflux
- renal calculi
- urinary tract infections
- hydronephrosis
what is horsehoe kidney frequently associated with?
- urogenital
- cardiac
- skeletal
- CNS
- chromosomal
what is the most common neoplasm in the fetus and newborn?
congenital mesoblastic nephroma
what is congenital mesoblastic nephroma?
benign hamartoma composed of mesochymal tissue
what is wilms tumor?
Wilms tumour is a malignant lesion that is extremely rare and composed of epithelial tissue
what is the classification of renal cytic disease?
- dysplastic cysts
- hereditary cysts
- non-dysplastic non-hereditary cysts
what results from early severe obstruction?
Multicystic dyspastic kidney and dysplastic kidney
dysplasia
abnormality of development or an epithelial anomaly of growth and differentiation
what is the most common form of cystic disease in childhood?
multi-cystic dysplastic kidney
what is multi-cystic dysplastic kidney associated with?
atretic ureter and pelvoinfundibular atresia
what is atresia?
a condition in which a body orifice or passage in the body is abnormally closed or absent
what does multi-cystic dysplastic kidney look like?
kidney is replaced by multiple cysts of varying size and no normal renal tissue present (kidney not functional)
where are the multiple cysts seen of varying sizes?
right paraspinous location
what does multi-cystic dysplastic kidney look like with other structures?
no normal appearing kidney
-the contralateral kidney, bladder and amniotic fluid are all normal
what is the MCDK main distinguishing feature?
the pattern of renal cysts. randomly distributed, non communicating and of variable size
what is important to assess with MCDK?
contralateral kidney
how often is multicystic renal dysplasia bilateral?
19%-24% of cases
what implies lethal renal disease?
MCDK is present with nonvisualization of the fetal bladder and severe oligohydramnios
what can unilateral obstructive cystic renal disease caused by?
uteropelvic or vesicourethral junction obstructon
what can bilateral obstructive cystic renal disease caused by?
severe bladder outlet obstruction, usually urethral atresia or posterior urethral valves
what is the severity of Obstructive Cystic Renal Dysplasia related to?
the timing and severity of the obstruction to urine flow
what is the sonographic appearance of cystic renal dysplasia?
- variable
- most typical finding, small echogenic kidney with one or several small subcapsualr cortical cysts plus evidence of urinary obstruction
- renal cysts may change in time
what must happen between an autosomal recessive disorder?
two copies of an abnormal gene must be present in order for the disease or trait to develop
what does Infantile Autosomal Recessive Polycystic Kidney Disease involve?
kidneys and liver
what is Perinatal Infantile Autosomal Recessive Polycystic Kidney Disease?
severe renal disease, minimal hepatic fibrosis and early death from pulmonary hypoplasia
what is Juvenile Autosomal Recessive Polycystic Kidney Disease?
minimal renal disease, marked hepatic fibrosis and longer survival
what are the sonographic findings of Autosomal Recessive Polycystic Kidney Disease?
- both kidneys are symmetrically enlarged and show increased echogenicity
- rena function is impaired
- severe oligohydramnios and bladder may not be seen.
do the size of the kidneys and cysts correlate with renal function
no
what is an indication of infantile polycystic kidney disease?
an elevated KC/AC ratio
what can IPKD be associated with?
meckel gruber syndrome
what is autosomial dominant?
A single abnormal gene on one of the first 22 non-sex (autosomal) chromosomes from either parent can cause an autosomal disorder
is adult autosomal dominant polycystic kidney disease unilateral or bilateral?
bilateral