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Renal > Congenital Renal Dx > Flashcards

Congenital Renal Dx Flashcards

1
Q

What is a sequence

A

Group of related to congenital anomalies in newborn which may be explained by single localized abnormality in organogenesis

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2
Q

What is a syndrome

A

Group of congenital anomalies in newborn which cannot be explained by single initiating abnormality in organogenesis

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3
Q

What is potter sequence

A

Phenotype of abnormalities caused by bilateral renal agenesis

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4
Q

Pathogenesis of potter sequence

A

Fatal compression restricting fetal growth due to oligohydramnios

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5
Q

What are some causes of oligohydramnios

A

Chronic amniotic fluid leakage from ruptured amnion

Utero placental insufficiency from maternal hypertension or toxemia

Fetal bilateral renal agenesis

Severe Renal dysplasia

Urinary tract obstruction

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6
Q

Clinical presentation of potter sequence

A 
V-shaped epicanthic folds
Large low nasal bridge
Parrot beak nose
Receding chin
Bowing of the legs with clubbed feet
broad spadE like hands
Pulmonary hypoplasia
Abundant loose skin
Growth retardation and amnion nodosum
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7
Q

POTTER mnemonic to recognize a case

A 
Pulmonary hypoplasia
Oligohydramnio
Twisted skin
Twisted face
Extremity deformities
Renal agenesis
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8
Q

Prognosis of potter sequence

A

Deaths in life born in fence due to generally respiratory difficulty from Hypoplastic lungs

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9
Q

What is bilateral agenesis of kidneys

A

Complete failure of both kidney to develop with total absence of Renal structures

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10
Q

Is bilateral agenesis compatible with life

A

No

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11
Q

Do you see Potter sequence in infants with absent kidneys

A

Yes

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12
Q

What is unilateral agenesis

A

One kidney failed to develop

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13
Q

Is unilateral agenesis compatible with life

A

Yes it is.

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14
Q

What happens to contralateral Kidney in unilateral agenesis

A

Compensatory hypertrophy and progressive glomerulosclerosis which can lead to chronic renal failure

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15
Q

What is Renal hypoplasia

A

Kidneys develop fail to reach a normal size

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16
Q

Is it more common to have unilateral or bilateral renal hypoplasia

A

Unilatera

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17
Q

What main difference would you see between Renal hypoplasia and acquired Atrophic kidneys

A

scars in acquired

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18
Q

What is renal ectopia

A

When the kidney is displaced during its cephalic journey

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19
Q

What is simple ectopia

A

Kidney stay on correct side but he’s at the level of pelvic brim or within pelvis

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20
Q

What is crossed ectopia

A

Kidney crisis midline and remain in pelvic brim or fuse with opposite kidney

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21
Q

Is renal ectopia associated with malformations

A

Yes

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22
Q

What is a Renal fusion

A

Fusion of lateralized kidneys resulting in the horseshoe kidney

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23
Q

90% cases of fusion of kidneys are at lower poles or upper Poles

A

Lower poles

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24
Q

When do you have a donut kidney

A

When both poles fuse

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25
Is the renal fusion common
Yes 1/400
26
What are some associated anomalies with fusion of kidneys
Duplications cryptorchidism Urethral abnormalit
27
What is a cake or disc kidney and when do you see it
In the case of combined Renal ectopia and fusion Where kidney are fused into one mass
28
What are some cystic renal disease
``` Renal dysplasia multicystic kidney dysplasia adult polycystic kidney disease childhood polycystic kidney disease medullary sponge kidney nephronophthysis Uremic medullary cystic disease simple cysts dialysis associated cystic disease ```
29
What is Renal dysplasia
Abnormal differentiation of metanephric parenchyma with structures not found in normal nephrogenesis
30
Presentation of renal dysplasia
Bilateral are unilateral flank masses
31
Grass morphology of kidney in Reno dysplasia
Enlarged kidney irregular and multicystic kidney cyst with different sizes
32
Histology of renal dysplasia
Disorganized renal architecture primitive ducts lined by cuboidal columnar epithelium layers of collagen and smooth muscle around epithelium island of primitive mesenchyme,cartilage poorly developed tubules Dilated thin walled blood vessels
33
Prognosis of unilateral renal dysplasia
Excellent with removal of dysplastic Kidney
34
Prognosis of bilateral renal dysplasia
Very rare cases but generally end up with renal failure
35
What is multicystic kidney
Extreme form of renal dysplasia with large cyst distributed peripherally and make kidney look like a bunch of grapes
36
Why are multicystic kidney cases mostly unilateral
Because bilateral multisystem Kidney are mostly incompatible with extrauterine life
37
Histology of multisystem Kidney
Cysts line by flattened epithelium Variables stroma between cysts dysplastic elements in middle of kidney
38
Second name of adult polycystic kidney disease
Autosomal dominant polycystic kidney disease
39
What is adult polycystic kidney disease
Enlargement of both kidneys by numerous cysts that eventually destroy parenchyma and cause renal failure
40
What are the mutation involved in adult polycystic kidney disease
PKD1 PKD2 PKD3
41
Clinical presentation of adult polycystic kidney disease
Enlarge kidneys like abdominal masses Polycystic liver mitral valve prolapse Berry aneurysm that can rupture and cause severe subarachnoid hemorrhage
42
Histology of adult polycystic kidney disease
Cortical and medullary cyst of various shapes and sizes lined epithelium associated with normal parenchyma scattered cystic Bowman capsules with the glomerular tufts
43
Prognosis of adults polycystic kidney disease
Renal function until about 4th decade | Complications after
44
What’s the second name of childhood plycystic kidney disease
Autosomal recessive polycystic kidney disease
45
Clinical presentation of childhood polycystic kidney disease
Abnormal abdominal distention | bilateral flank masses seen at birth
46
Grass morphology of childhood polycystic kidney disease
Kidneys enlarged and reniform Miniature cyst in cortical surface cortical medullary demarcation obscured or absent Closely apposed fusiform or cylindrical dilated ducts radiating from medullary to subscapular region replacing parenchyma
47
Histology of childhood polycystic kidney
Saccular or a cylindrical dilatation of all collecting ducts with cuboidal epithelium
48
Pregnancies of childhood polycystic kidney disease
Poor with infants dying from Reno failure and older children dying from liver disease complication
49
What is medullary sponge kidney
Non hereditary disorder of the kidney with multiple cystic dilatation of collecting ducts in the medulla
50
Do we know the etiology and pathogenesis of medullary sponge kidney
No
51
Clinical presentation of medullary sponge kidney
Asymptomatic and discovered incidentally
52
Gross morphology of medullary sponge kidney
Dilated capillary ducts in the medulla | small cyst
53
Histology of medullary sponge kidney
Cyst lined by cuboidal and sometimes transitional epithelium
54
Prognosis of medullary sponge kidney
Normal renal function part pyelonephritis And it’s calcifications may develop
55
Nephronophthysis second name
Uremic medullary cystic disease
56
What is Nephronophthysis
Group of renal disease inherited or sporadic with very variable number of medullary cysts with cortical tubular atrophy and interstitial fibrosis
57
Four types of Nephronophthysis
Sporadic non-familial familial juvenile which is autosomal recessive renal retinal dysplasia -recessive adult onset medullary cystic disease - dominant
58
Clinical presentation of Nephronophthysis
Polyuria polydipsia growth retardation anemia
59
Gross morphology of Nephronophthysis
Small contracted kidneys granular surfaces cysts in medulla ,prominent at cortico medullary Junction
60
Histology of Nephronophthysis
``` Cyst lined by flattened or cuboidal epithelium inflammatory cells or fibrosis Generalized tubular atrophy interstitial fibrosis normal glomeruli ```
61
Prognosis of Nephronophthysis
Poor with progression to renal failure in childhood
62
How do you mostly discover Simple cyst
Incidentally
63
Grass morphology of simple cyst
Multiple or single usually cortical cystic space of variable size If large size becomes translucent with gray glistening ,smooth lining filled with clear fluids
64
Histology of simple cyst
Single layer of flattened or cuboidal
65
Prognosis of simple cyst
No clinical significance
66
do dialysis associated cystic disease occur mostly in males or females
Males
67
Clinical presentation of dialysis associated cystic disease
Asymptomatic | Or hematuria
68
Grass morphology of dialysis associated cystic disease
Bilateral large kidney normal or small(common)) | Cyst in the cortex ,smaller number in Midler
69
Histology of dialysis associated cyst
Sis lined by flirting or hyperplastic epithelium with papillary formations
70
Pregnancies of dialysis associated cyst
Can lead to Renal adenoma and renal cell carcinoma | retroperitoneal hemorrhage as complication

Renal (16 decks)

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