Week 8 Flashcards
Which germ layer is the urogenital system formed?
Intermediate mesoderm
What does the intermediate mesoderm develop into first before starting its 3 stages?
urogenital ridge
Stages of development of urinary system
- 1st
- 2nd
- 3rd
- Pronephros: most cranial, non-functional
- Mesonephros: functional for 18 weeks, develops into the collecting tubules and wolffian duct
- Metanephros; develops into the kidney
Name the intermediate structure, genes involved, adult derivative
- mesonephric duct
- metanephric mesoderm
- endoderm
- urteric bud; WT-1, GDNF, c-RET; Ureter renal pelvis, calyces and collecting duct
- metanephric vesicles-> renal tubules; BMP7, Wnt-4; Collecting tubule, DCT, loop of henle, PCT and bowmans capsule
- urogenital sinus; urinary bladder
What is WT-1?
- codes for Wilms tumor protein which is an oncogene
GDNF
- what does ti stand for?
- what is it?
- receptor?
- Glial derived neurotrophic factor
- morphogen
- ## cRet which is another protooncogene
15 m/o male w/o irises and w/ palpable nontender mass on left side of abdomen. What is wrong? - cause? - signs and symptoms. - risk factors
wilms tumor
- mutations to multiple genes
- abdominal mass (mostly relating to size of mass, not severity) that may be symptomatic (fever, hematuria, dysuria, constipation, pain = not necessary for diagnosis)
- family history and a horseshoe kidney (developing kidneys may cross over and fuse together forming a U-shaped structure)
15 y/o girl w/ R lower quadrant pain abdominal pain after menarche. No vomiting or diarrhea. Afebrile with RLQ tendernss. Normal blood. US showed cystic mass over RLQ and right renal agenesis.
Dx?
What is this?
- Helyn-Werner-Wunderlich Syndrome
- extremely rare congenital anomaly related to abnormality of Mullerian duct) can lead to ipsilateral renal agenesis
28 yr old male w/ recurrent pain in LLQ for 1 month and an episode of hematuria. Pt had a 3-4 hr pain attack 2 days before and passed 2 small stones in urine then the pain passed. Pyelogram revealed left kidney was not located in normal position and was instead found at L4-S1.
Dx?
- what does that mean?
- what can this cause?
- Pelvic Kidney
- does not ascend from its original position in the pelvis to its final location during fetal development & the renal artery follows,
- possible complications include infection (closer to the outside of body than normal, can develop possible UTI)
19 m/o girl w/ history of spinabifida presents with abdominal pain. Umbilicus was protuberant and erythematous. White cell count was elevated with lots of neutrophils. CT showed cyst extending from umbilicus to dome of bladder but was not connected to umbilicus or bladder.
- Dx
- what is a urachus? what sould it be?
- urachal cyst
- remnant of allantois; median umbilical ligament
Where does bladder come from?
- its epithelium?
- connection initially
- vesical part of urogenital sinus
- endoderm
- allantosis from hindgut
Key structures formed during renal development
- ureteric bud, what is it? what does it make?
- metanephric mesenchyme, other name? what is it?
- outgrows mesonephric duct, turns into ureter, pelvis, calyces, and collecting duct
- metanephric duct, mesoderm tissue that interacts with ureteric bud to form glomerulus to DCT
Wilms tumor
- etiology
- how does it happen?
- genes
- most common renal malignancy in children
- proliferation of metanephric blastema
- mutation to WT1
Renal Agenesis
- how?
- what does this cause?
- affecting 1 vs both kidneys
- renal bud fails to form
- lack of signals to mesenchyme
- 1: other kidney compensates; 2: oligohydraminos (very little amniotic fluid)
Multi-cystic dysplastic kidney
- how does it happen?
- consequence?
- sides?
- abnormal ureteric bud and mesenchyme interaction
- Kidney replaced with cysts; none/little functioning renal tissue
- unilateral
uretero pelvic junction obstruction
- what happens?
- effect
- sides?
- narrowing of proximal ureter
- causes hydronephrosis and leads to poor urinary flow which causes kidney stones/UTI
- single sided
Duplex collecting sxs
- what is it?
- effect
- 2 ureteric buds on 1 side or bifurcation of a single ureteric bud
- Kidney divided into upper and lower since it is drained by 2 colecting ducts-> poor urine flow -> hydronephrosis -> UTI
Vesico-ureteral reflex
- what is it?
- effect
- primary causes
- secondary causes
- backwards urine flow -> from bladder to kidneys
- causes recurrent UTI
- abnormal closure of ureterovesical junction and associated with duplex ureteres
- increase in bladder pressure -> pushes urine back; seen with posterior uretheral valves
Potters Syndrome
- consequence
- prognosis
- bilateral renal agenesis
- posterior uretheral valve
- autosomal recessive polycystic kidney
- amniotic fluid is produced from fetal urine -> severe renal malfunction -> decrease in amniotic fluid -> fetus is not as cushioned -> squished face and abnormal limb development, alteration in lung formation because there is not enough fluid
- usually fatal
- Neither kidney is formed -> no urine can be produced -> no amniotic fluid
- tissue obstructs bladder outflow, more common in male, both kidneys can be affected
- cysts in kidneys and biliary tree, both affected bc this is genetic
Horshoe Kidney
- what happens?
- affect on position of kidney
- symptoms
- common with
- inferior poles of kidneys fuse
- Kidney cannot ascend into correct position because it is blocked by inferior mesenteric a
- asymptomatic
- down syndrome and turners syndrome
Urachus
- what is it?
- normal way to get rid of it?
- what happens with dx?
- effects
- connects dome of bladder to umbilicus
- it is supposed to be obliterated at birth and become median umbilical ligament
- fails to be obliterated
- urine can leak from umbilicus and increase the risk of infection and can lead to adenocarcinoma of bladder
