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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
Q

Is the renal fusion common

A

Yes 1/400

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26
Q

What are some associated anomalies with fusion of kidneys

A

Duplications
cryptorchidism
Urethral abnormalit

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27
Q

What is a cake or disc kidney and when do you see it

A

In the case of combined Renal ectopia and fusion Where kidney are fused into one mass

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28
Q

What are some cystic renal disease

A 
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
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29
Q

What is Renal dysplasia

A

Abnormal differentiation of metanephric parenchyma with structures not found in normal nephrogenesis

30
Q

Presentation of renal dysplasia

A

Bilateral are unilateral flank masses

31
Q

Grass morphology of kidney in Reno dysplasia

A

Enlarged kidney
irregular and multicystic kidney
cyst with different sizes

32
Q

Histology of renal dysplasia

A

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
Q

Prognosis of unilateral renal dysplasia

A

Excellent with removal of dysplastic Kidney

34
Q

Prognosis of bilateral renal dysplasia

A

Very rare cases but generally end up with renal failure

35
Q

What is multicystic kidney

A

Extreme form of renal dysplasia with large cyst distributed peripherally and make kidney look like a bunch of grapes

36
Q

Why are multicystic kidney cases mostly unilateral

A

Because bilateral multisystem Kidney are mostly incompatible with extrauterine life

37
Q

Histology of multisystem Kidney

A

Cysts line by flattened epithelium
Variables stroma between cysts
dysplastic elements in middle of kidney

38
Q

Second name of adult polycystic kidney disease

A

Autosomal dominant polycystic kidney disease

39
Q

What is adult polycystic kidney disease

A

Enlargement of both kidneys by numerous cysts that eventually destroy parenchyma and cause renal failure

40
Q

What are the mutation involved in adult polycystic kidney disease

A

PKD1
PKD2
PKD3

41
Q

Clinical presentation of adult polycystic kidney disease

A

Enlarge kidneys like abdominal masses
Polycystic liver
mitral valve prolapse
Berry aneurysm that can rupture and cause severe subarachnoid hemorrhage

42
Q

Histology of adult polycystic kidney disease

A

Cortical and medullary cyst of various shapes and sizes
lined epithelium associated with normal parenchyma
scattered cystic Bowman capsules with the glomerular tufts

43
Q

Prognosis of adults polycystic kidney disease

A

Renal function until about 4th decade

Complications after

44
Q

What’s the second name of childhood plycystic kidney disease

A

Autosomal recessive polycystic kidney disease

45
Q

Clinical presentation of childhood polycystic kidney disease

A

Abnormal abdominal distention

bilateral flank masses seen at birth

46
Q

Grass morphology of childhood polycystic kidney disease

A

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
Q

Histology of childhood polycystic kidney

A

Saccular or a cylindrical dilatation of all collecting ducts with cuboidal epithelium

48
Q

Pregnancies of childhood polycystic kidney disease

A

Poor with infants dying from Reno failure and older children dying from liver disease complication

49
Q

What is medullary sponge kidney

A

Non hereditary disorder of the kidney with multiple cystic dilatation of collecting ducts in the medulla

50
Q

Do we know the etiology and pathogenesis of medullary sponge kidney

A

No

51
Q

Clinical presentation of medullary sponge kidney

A

Asymptomatic and discovered incidentally

52
Q

Gross morphology of medullary sponge kidney

A

Dilated capillary ducts in the medulla

small cyst

53
Q

Histology of medullary sponge kidney

A

Cyst lined by cuboidal and sometimes transitional epithelium

54
Q

Prognosis of medullary sponge kidney

A

Normal renal function part pyelonephritis And it’s calcifications may develop

55
Q

Nephronophthysis second name

A

Uremic medullary cystic disease

56
Q

What is Nephronophthysis

A

Group of renal disease inherited or sporadic with very variable number of medullary cysts with cortical tubular atrophy and interstitial fibrosis

57
Q

Four types of Nephronophthysis

A

Sporadic non-familial
familial juvenile which is autosomal recessive
renal retinal dysplasia -recessive
adult onset medullary cystic disease - dominant

58
Q

Clinical presentation of Nephronophthysis

A

Polyuria polydipsia
growth retardation
anemia

59
Q

Gross morphology of Nephronophthysis

A

Small contracted kidneys
granular surfaces
cysts in medulla ,prominent at cortico medullary Junction

60
Q

Histology of Nephronophthysis

A 
Cyst lined by flattened or cuboidal epithelium 
inflammatory cells or fibrosis 
Generalized tubular atrophy 
interstitial fibrosis 
normal glomeruli
61
Q

Prognosis of Nephronophthysis

A

Poor with progression to renal failure in childhood

62
Q

How do you mostly discover Simple cyst

A

Incidentally

63
Q

Grass morphology of simple cyst

A

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
Q

Histology of simple cyst

A

Single layer of flattened or cuboidal

65
Q

Prognosis of simple cyst

A

No clinical significance

66
Q

do dialysis associated cystic disease occur mostly in males or females

A

Males

67
Q

Clinical presentation of dialysis associated cystic disease

A

Asymptomatic

Or hematuria

68
Q

Grass morphology of dialysis associated cystic disease

A

Bilateral large kidney normal or small(common))

Cyst in the cortex ,smaller number in Midler

69
Q

Histology of dialysis associated cyst

A

Sis lined by flirting or hyperplastic epithelium with papillary formations

70
Q

Pregnancies of dialysis associated cyst

A

Can lead to Renal adenoma and renal cell carcinoma

retroperitoneal hemorrhage as complication

Renal (16 decks)

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