Kidney Development - Bolender Flashcards

1
Q

The entirety of the kidney develops from what precursor tissue?

Where it it located in the developing embryo (~Day 18-20)

A

Intermediate mesoderm.

Located lateral to the paraxial mesoderm.

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2
Q

What is the name of the structure from which the kidney and gonads both develop?

A

Both develop from the nephrogenic cord; the kidneys from the metanephron, the gonads from the mesonephron.

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3
Q

What are the two components of the mesonephric kidney?

Which of these two structures acts as a source of signaling?

Where is it located?

A

The mesonephric duct and tubules.

The duct is a source of inductive signaling.

The mesonephric kidneys are located bilaterally in the thoracolumbar region.

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4
Q

What is the cloaca?

How do the mesonephron and metanephron communicate with this structure?

A

A primitive outflow region for both the GU and GI tracts in the developing embryo.

Both drain to it, via the nephrogenic cord or a structure common to it and the metanephric diverticulum.

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5
Q

What is the function of the mesonephric tubules?

How do they develop?

A

Mesonephric tubules are immature nephrons, which serve renal function briefly until the mature kidneys form.

Develop via signals derived from the mesonephric duct, differentiating cranial to caudal.

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6
Q

What are the two components of the metanephric kidneys?

Can you recall what each one will ultimately give rise to?

A

Metanephric Diverticulum: Epithelial tissue which will form the bulk of the urinary collection system.

Metanephric Mesenchyme: Gives rise to tubular, vascular, and presumably interstitial tissue of the kidneys.

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7
Q

Describe the reciprocal interactions that occur between the metanephric diverticulum and mesenchyme.

A

The mesenchyme signals for formation & growth of the diverticulum into branches (GDNF).

Signals from the diverticulum prevent apoptosis of mesenchyme, and promote its differentiation and aggregation around the diverticulum.

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8
Q

Explain the pathophysiology and clinical findings in Oligohydramnios.

A

In oligohydramnios, a deficit of amniotic fluid results in pulmonary hypoplasia, growth deficiency, and fetal compression (Potter’s facies, limb defects).

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9
Q

Distinguish between Potter’s syndrome and Oligohydramnios.

A

Historically, Potter’s syndrome referred to the clinical syndrome seen in babies with oligohydramnios caused by renal agenesis. It is often used interchangeably with oligohydramnios, however.

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10
Q

What are some possible causes of Potter’s syndrome?

A

Bilateral renal agenesis

Polycystic kidney diseases

Severe urinary tract obstruction

Chronic amniotic leak

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11
Q

What conditions are associated with polyhydramnios?

A

Diabetes, multiple gestation, ancencephaly, esophageal atresia.

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12
Q

Describe how nephrons form.

What is the S-shaped tubule missing?

A

Signals from the tips of the metanephric diverticulum (Ampullae) cause the mesenchyme to aggregate and form an epithelial vesicle; the tubule forms from collecting duct backwards to the glomerulus.

S-shaped tubule is not vascularized.

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13
Q

What ultimately forms from the proximal, middle, and distal parts of the S-shaped nephron?

A

Proximal: Distal tubule & loop of henle

Middle: Proximal tubule

Distal: Renal corpuscle

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14
Q

What signals for migration of vascular tissue to the nephrons?

A

VEGF.

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15
Q

What is on the DDx for a neonatal unilateral flank mass?

What if it is a bilateral flank mass?

A

Unilateral: Hydronephrosis, Cystic kidney diseases, Renal tumors.

Bilateral: Most likely PCKD, but some other bilateral conditions are possible (eg Bilateral dysplastic kidney)

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16
Q

Multicystic Dysplastic Kidney

Describe its etiology.

What is found on biopsy?

How is renal function affected?

A

Multicystic Dysplastic Kidney

Congenital malformation of parenchyma, but not inherited.

Cysts, primitive ductules and cartilage.

Since the contralateral kidney hypertrophies, function is generally preserved.

17
Q

Multicystic Kidney Disease

What are some possible complications?

How is it treated?

A

Multicystic Kidney Disease

Hypertension. Reflux of the contralateral kidney.

Not treated at all unless failure is severe, then nephrectomy.

18
Q

How many generations of diverticular branches are there?

Distinguish between them.

A

Usually about 15 generations.

Early generations grow little. 3rd-6th form calyces/pelvis/ureter. Polar branches grow faster; distal to 5th & 6th form collecting ducts.

19
Q

T/F: The fetal kidney must be bisected to visualize lobes.

A

False; Renal lobes are visible externally.

20
Q

Summarize a few possible forms of renal developmental defect.

A

Hypoplasia, dysplasia, duplication (ureter/kidney), fusion (horseshoe kidney), ectopic kidney.

21
Q

What hereditary conditions cause congenital kidney dysfunction?

A

Usually polycystic kidney diseases. Medullary cystic disease is also inherited (autDom)

22
Q

What is a Wilm’s Tumor?

What mutation is it associated with?

A

A blastema containing many different tissue types, seen commonly in children.

Associated with WT1 mutation on chromosome 11

23
Q

Ureteropelvic Junction Obstruction

Distinguish between the adult and infant presentations.

How is it diagnosed?

A

Ureteropelvic Junction Obstruction

Infant: UTI, Flank mass, failure to thrive, sepsis

Adult: UTI, Flank pain, colicky pain, hematuria

Renal ultrasound, intravenous pyelogram. Palpation for hydronephrosis?