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patho II > Renal II (a) -Congenital disroders of the kidney/ Tumours > Flashcards

Renal II (a) -Congenital disroders of the kidney/ Tumours Flashcards

1
Q

——–: Absent ureter or failure to extend to the bladder resulting in a blind ending

A

Ureteral Artesia

* Artesia –> poor development of an organ during emryonal development

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2
Q

patho of Ureteral Artesia:
Failure of ———– of a segment of ureter during the process of Embryonal development and elongation of the ureteric bud

A

Failure of canalisation of a segment of ureter during the process of development and elongation of the ureteric bud

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3
Q

Epi and associated symptoms of Ureteral Artesia

A
  • Rare
  • Asso w/ –> dysplastic non functioning kidney

dysplastic : under developed

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4
Q

CF of Bilateral Ureteral Artesia

A

Incompatible with life

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5
Q

CF of Unilateral Artesia

A

asymptomatic but may
cause hypertension

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6
Q

Complete Duplication of the ureters are often asso w/?

A

Vesico-Ureteral Reflux, Ectopic Ureterocele, or Ectopic Ureteral insertion

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7
Q

Incomplete Dublication of the Ureters are often asso. w/?

A

Ureteral Reflux or Uretero-Pelvic-Junction (UPJ) obstruction of the lower pole of the Kidney

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8
Q

———–: Two separate Pelvi-Calyceal Systems joined at UPJ

A

Bifid Pelvis

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9
Q

——-: Two separate Ureters proximally that join
at any point below the UPJ but before entering into
the Bladder

A

Bifid Uterus

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10
Q

Epi of Prune Belly Syndrome

A

more common in males (during birth)

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11
Q

CM of Prune Belly Syndrome

A

Classical triad:
1. Urinary Tract anomalies
2. Deficient abdominal musculature (wrinkled/Prune-like Appearnace of the abdomin wall)
3. Bilateral Cryptorchidism

other CF:
1) Club foot (Talipes Equinovarus
2) Pulmonary hypoplasia
3) Imperforate Anus
4) Arthrogryposis (joint stiffness at birth)

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12
Q

——— :Abnormal placement of the kidenys due to faulty migration during embryonal development

A

Renal Ectopia

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13
Q

Simple Ectopy vs Crossed Renal Ectopy

A
  • Simple Ectopy –> kidenys on their normla anatomical body side, but one is located in the pelvis or over the brim
  • Crossed Ectopy –> the two kidenys are on the same side, completely independant
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14
Q

Epi of Horseshoe kidney

A

M > F

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15
Q

Cause of Horseshoe kideny?

A

associated with a narrow pelvis, as seen in Trisomy 18

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16
Q

In a horseshoe kidney there are Two ——– and Two ———

A

Two excretory systems and two ureters

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17
Q

CF of Horseshoe kidney in children

A

i. Urinary Tract Infections (UTIs),
ii. Abdominal mass
iii. Haematuria

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18
Q

complications of Horseshoe kidney

A

Stone disease, UPJ obstruction, Trauma,
Infections and Tumours

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19
Q

Causes of Potter’s syndrome

A

i. Bilateral Renal Agenesis, (absence of both kidneys)
ii. Infantile Polycystic Kidney Disease,
iii. Renal Hypoplasia and
iv. Obstructive Uropathy

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20
Q

Bilateral Renal Agenesis –> [Compatible/Incompatible] w/ life?

A

Incompatible

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21
Q

CF of Potter’s Syndrome

A

1) Oligohydramnios –> Pulmonary Agenesis –> Fatal Outcome
2) Characteristic Facies: Folds under the Eyes, Flat Nose, Lowset Ears and Receding Chin

* Oligohydramnios : too little amniotic fluid

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22
Q

Potter’s syndrome is Kidney failure due to ———-

A

Oligohyrdamnios

  • Amniotic fluid is the urine produced by the kidneys of the featus
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23
Q

———– : Absence of one Kidney

A

Unilateral Renal Agenesis

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24
Q

CF of Unilateral Renal Agenesis

A

No symptoms; incidental finding

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25
Q

————– : Either one small and one larger than normal Kidneys or both Kidneys small

A

Renal Hypoplasia

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26
Q

Renal Hypoplasia (small kidneys) <> small renal arteries –> ——–

A

HTN

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27
Q

Causes of Cystic Disease of the Kidney

A

can be Hereditary, Developmental or Acquired disorders

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28
Q

Macroscopic Features:
* Size: 1-5cm
* Localisation: Cortex
* Translucent
* Gray, glistening, smooth membrane
* Content of cyst: Clear fluid

Microscopic Findings:
* Single layer of cuboidal or flattened cuboidal epithelium

features of?

A

Simple Renal Cysts (Localized)

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29
Q

Simple Renal Cysts

Macroscopic Features:
* Size: 1-5cm
* Localisation: ———–
* Translucent
* Gray, glistening, smooth membrane
* Content of cyst: ———-

Microscopic Findings:
* Single layer of cuboidal or flattened cuboidal epithelium

A

Macroscopic Features:
* Size: 1-5cm
* Localisation: Cortex
* Translucent
* Gray, glistening, smooth membrane
* Content of cyst: Clear fluid

Microscopic Findings:
* Single layer of cuboidal or flattened cuboidal epithelium

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30
Q

Epi of Dialysis Assocaited Aquired Renal cysts

A

Patients with End-Stage Kidney disease and prolonged Dialysis

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31
Q

location of Dilaysis Ass. Aquired renal cysts

A

Cortex and medulla

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32
Q

Complications of Dialysis ass. aquired renal Cysts

A

1) Haematuria
2) Development of Renal Adenoma or Papillary Adenocarcinoma

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33
Q

Macroscopic features:
* Loc of Cysts: Cortex and medulla

Microscopic features:
* Multiple cysts
* Cyst lined by simple cuboidal or flattened cells

features of ?

A

Dialysis ass aquired renal cysts

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34
Q

Autosomal Dominant (Adult) Polycystic Kidney disease are caused by Mutations in ——- (85-90% of cases) and ——- (10-15% of cases)

A

Mutations in PKD1 (85-90% of cases) and PKD2 (10-15% of cases)

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35
Q

Macroscopic Features:
* Very large Kidneys
* Cut surface: Mass of Cysts (up to 3-4 cm), with no
intervening parenchyma
* Content: Clear, turbid or haemorrhagic fluid

Microscopic Findings:
* Variable lining epithelia of the cystic spaces
* Occasionally, presence of glomerular tufts within the cysts

features of?

A

AD (Adult) Polycystic Kidney disease

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36
Q

CF of Autosomal Dominant (Adult) Polycystic kideny disease

A

1) Flank pain
2) Heavy dragging sensation
3) Palpation of an Abdominal Mass
4) Intermittent Gross Haematuria
5) Berry Aneurysms, with high incidence of Subarachnoid Haemorrhage (10-30% of patients)

37
Q

Adults w/ AD Polycystic Kidney disease have a high incidence of developing?

A

Subarachnoid
Haemorrhage (10-30% of patients)

38
Q

complications of AD (Adult) Polycystic kideny disease

A
  • Hypertension (75% of cases)
  • Urinary infection
39
Q

Autosomal Recessive (Childhood) Polycystic Kidney disease are caused by mutations in the ——– gene

A

Autosomal Recessive (Childhood) Polycystic Kidney disease are caused by mutations in the PKHD1 gene

40
Q

Macroscopic Features:
* Enlarged organs, with a smooth external surface
* Cut surface: Numerous small Cysts in the Cortex and
Medulla –> Sponge-like appearance

Microscopic Findings:
* Dilated, elongated cystic spaces, vertical to the cortical surface
* Uniform lining of cuboidal cells
* Cysts in the Liver

Features of?

A

AR (Childhood) Polycystic Kidney disease

41
Q

Cf of AR (Childhood) polycystic kideny disease

A
  • Death of young infants, from Hepatic or Renal Failure
  • Patients, who survive infancy -> Development of Liver
    Cirrhosis
42
Q

Genetic predisposition of Nephronophthisis-Medullary Cystic Disease Complex

A

Nine responsible gene loci (NHP1-9)

43
Q

Macroscopic Features:
* Small, shrunken Kidneys

Microscopic Findings:
* Cysts lined by flattened or cuboidal epithelium
* Chronic Tubulo-Interstitial Nephritis
* Tubular Atrophy
* Progressive Interstitial Fibrosis

features of?

A

Nephronophthisis-Medullary Cystic Disease Complex

44
Q

CF of Nephronophthisis-Medullary Cystic Disease Complex

A

Polyuria and Polydipsia

45
Q

Progression of Nephronophthisis-Medullary Cystic Disease Complex

A

End-Stage Kidney Disease <> Within 5-10 yrs.

46
Q

Most common type of Renal stones

A

Calcium oxalate or mixture of Calcium oxalate with
Calcium phosphate (80%)

46
Q

Renal stones are aka?

A

Urolithiasis

47
Q

cause of Renal stones (Urolithiasis)

A

Increased urinary concentration of the ston’s componenets

48
Q

location of Renal stones

A

Renal Pelves, Calyces and Bladder

49
Q

Macroscopic features:
* Staghorn Calculi in the renal pelvis
* marked hydronephrosis

Microscopic features:
* mix of calcium oxalate w/ calcium phosphate stones (in the calcyces)

features of?

A

Renal stones - Uroluthiasis

Staghorn calculi : large renal stones in the renal pelvis

50
Q

CF of Renal stones (Urolithiasis)

A

1) Paroxysms (sudden attacks) of Flank pain radiating to the Groin
2) Macroscopic Haematuria

51
Q

Complications of Renal stones (Urolithiasis)

A

UTIs

52
Q

———– : Dilatation of the Renal Pelvis and Calyces, accompanied by Parenchymal Atrophy, as a result of Urine-Outflow obstruction

A

hydronephrosis

53
Q

Congenital causes of Hydronephrosis

A

1) Urinary tract obsrtruction/ narrowing
2) Artesia of the urethra

54
Q

Aquired Causes of Hydronephrosis

A

1) Foreign bodies (e.g. Urinary calculi)
2) Proliferative lesions (e.g. BPH, Prostatic Adenocarcinoma, Bladder Tumours [Papilloma or Carcinoma], etc.)
3) Inflammation (e.g. Prostatitis, Ureteritis, Urethritis, etc.)
4) Neurogenic: Spinal Cord damage, with Bladder paralysis
5) Normal pregnancy

55
Q

Macroscopic Features:
* Marked Kidney enlargement
* Prominent distention of the Pelvi-Calyceal System
* Compression and Atrophy of the Renal Parenchyma
* Obliteration of the Papillae & Flattening of the Pyramids

Microscopic Findings:
* Tubular dilation
* Atrophy and Fibrosis of the tubular lining
* Atrophy and Loss of Glomeruli
* Coagulative Necrosis of the Renal Papillae
* Superimposed Pyelonephritis

features of?

A

Hydronephrosis

56
Q

Hydronephrosis

Pathogenesis:
* ————— –> Reflux of the filtrate into the
renal interstitium and peri-renal spaces –> Return into the Lymphatic and Venous System
* Constant and uninterrupted back diffusion of the —— –> ———— of the Renal ——— and ———-
* Generation of ———- in the Renal Pelvis –>
Compression of the ———— –>Arterial insufficiency and Venous Stasis
* Initial functional disturbances <=> Tubular; Manifested by impaired concentrating ability
* Later in the course of the disease –> Decrease in the
———–

A
  • Complete Obstruction –> Reflux of the filtrate into the renal interstitium and peri-renal spaces –> Return into the Lymphatic and Venous System
  • Constant and uninterrupted back diffusion of the filtrate –> Dilatation of the Renal Pelvis and Calyces
  • Generation of high pressure in the Renal Pelvis –> Compression of the Renal Vasculature ->Arterial insufficiency and Venous Stasis
  • Initial functional disturbances <=> Tubular; Manifested by impaired concentrating ability
  • Later in the course of the disease –> Decrease in the
    GFR
57
Q

CF of Hydronephrosis in cases of Complete, Bilateral Obstruction

A

Anuria

58
Q

CF of Hydronephrosis in cases of Incomplete, Bilateral Obstruction

A

Polyuria

59
Q

CF of Unilateral Hydronephrosis

A

Completely silent for long periods; Discovered incidentally on routine physical examination

60
Q

—————: Benign tumour, arising from the Oncocytes of Collecting Ducts

A

Oncocytoma

61
Q

Epi of Oncocytoma

A

10% of Renal Tumours

62
Q

Gentic Predispostion of Oncocytoma

A

Loss of Chromosomes 1, 14 and Y

63
Q

Complications of Oncocytoma

A

Spontaneous Haemorrhage

64
Q

Macroscopic Features:
* Tan colour
* Central Stellate Scar

Microscopic Findings:
* Numerous mitochondria, within neoplastic cells –> Finely granular eosinophilic cytoplasm
* Hypocellular Hyalinised Stroma
* small, round nuceli

features of?

A

Oncocytoma

65
Q

location of Renal Cell Carcinoma

A

renal Cortex

66
Q

Epi of Renal Cell Carcinoma

A

80-85% of all primary Renal Malignant Tumours
M>F

67
Q

Causes/Risk factors of Renal cell carcinoma

A

1) Smoking
2) HTN
3) Obesity
4) Acquired Polycystic Kidney Disease, in Chronic-Dialysis patients (30-fold increased risk)

68
Q

Epi of Clear cell carcinoma

A

65% of Renal Cell Cancers

69
Q

Cause of Clear cell carcinoma

A

Homozygous loss of the VHL gene
(risk factors: smoking, HTN, obesity)

70
Q

Macroscopic Features:
* Usually, solitary and large, sphaerical masses (3-15cm)
* Cut surface: Yellow to orange to gray-white
* Dilated renal Calcyes
* Central tumour Necrosis and Haemorrhage
* Invasion of Calyces and Pelvis, as well as of the Renal Vein, Inferior Vena Cava and even the Right Heart-side

Microscopic Findings:
* Vacuolated, Clear cells (due to intra-cytoplasmic glycogen and lipid) - classic sign
* Small, round nuclei
Solid cytological picture:
* Cells with pink granular cytoplasm (as tubular epithelium)

features of?

A

Renal Clear cell carcinoma

71
Q

Epi of Papillary Renal Cell Carcinomas

A

10-15% of all Renal Cancers

72
Q

Causes of Papillary renal cell carcinoma

A

Activating mutations of the MET gene, in familial cases, but also some sporadic ones

73
Q

Macroscopic Features:
* Multifocal and bilateral occurrence; Early-stage Tumours
* Cut surface: Weak orange-yellow colour; Necrosis,
Haemorrhage and Cystic degeneration

Microscopic Findings:
* Formation of Papillae with fibrovascular cores
* Foamy macrophages

features of?

A

Papillary Renal cell carcioma

74
Q

Epi of CHromophobe Renal Carcinomas

A

5% of all Renal Cell Carcinoma

75
Q

Macroscopic Features:
* Tan-brown colour

Microscopic findings:
* Clear, flocculent (Fine reticular) cytoplasm
* Prominent distinct, thick cell membranes
* Peri-nuclear “halos” of clear cytoplasm

features of?

A

Chromophobe Renal Carcinomas

76
Q

Chrombophobe Renal Carcinoma

Macroscopic Features:
* Tan-brown colour

Microscopic findings:
* Clear, flocculent (Fine reticular) cytoplasm
* Prominent distinct, thick cell membranes
* “————” of clear cytoplasm

A

Macroscopic Features:
* Tan-brown colour

Microscopic findings:
* Clear, flocculent (Fine reticular) cytoplasm
* Prominent distinct, thick cell membranes
* Peri-nuclear “halos” of clear cytoplasm

77
Q

CF of Chromophobe Renal carcinomas

A

Classic Triad:
1) Haematuria (>50% of cases)
2) Dull Flank Pain
3) Palpable Abdominal Mass

78
Q

WILMS tumour is aka?

A

Nephroblastoma

79
Q

Epi of Wilms Tumour

A
  • Most common primary Renal Tumour in children
  • Usually, children 2-5 years old
80
Q

Causes of Wilms tumour

A

1) Patients with the WAGR Syndrome (1/3 of Wilms Tumour) cases –> Loss of genetic material of WT1 gene
2) Patients with Denys-Drash Syndrome (90% –> Wilms Tumour); Dominant negative inactivating mutation in WT1 gene

81
Q

WILMS tumour causes

  • Patients with the ———- Syndrome (1/3 of cases -> Wilms Tumour); Loss of genetic material of WT1 gene
  • Patients with ———- Syndrome (90% -> Wilms Tumour); Dominant negative inactivating mutation in WT1 gene
A
  • Patients with the WAGR Syndrome (1/3 of cases -> Wilms Tumour); Loss of genetic material of WT1 gene
  • Patients with Denys-Drash Syndrome (90% -> Wilms Tumour); Dominant negative inactivating mutation in WT1 gene
82
Q

CF of WAGR Syndrome

A

1) Wilms Tumour
2) Aniridia (partial or complete absence of the iris)
3) Genito-Urinary anomalies
4) Mental Retardation

83
Q

CF of Denys-Drash Syndrome

A

1) Wilms Tumour
2) Intersex disorders (Gonadal dysgenesis)
3) Congenital Nephropathy

84
Q

Macroscopic Features:
* Most common, large, solitary, well-circumscribed mass
Cut surface:
* Soft, homogeneous Tumour, with tan to gray colour
* Foci of Cystic degeneration, Haemorrhage and Necrosis

Microscopic Findings:
* Classic triphasic combination of blastemal, stromal and epithelial cell types
- Blastemal component: Sheets of small blue cells
- Stromal component: Fibrocytic or Myxoid cells
- Epithelial component: Abortive Tubules or Glomeruli

features of?

A

WILMS tumor - Nephroblasmtoma

85
Q

WILMS tumour - Nephroblastoma

Macroscopic Features:
* Most common, large, solitary, well-circumscribed mass
Cut surface:
* Soft, homogeneous Tumour, with —- to —- colour
* Foci of Cystic degeneration, Haemorrhage and Necrosis

Microscopic Findings:
* Classic triphasic combination of ——–, ——– and ——– cell types
- Blastemal component: Sheets of ————- cells
- Stromal component: Fibrocytic or ——– cells
- Epithelial component: Abortive —— or ———-

A

Macroscopic Features:
* Most common, large, solitary, well-circumscribed mass
Cut surface:
* Soft, homogeneous Tumour, with tan to grey colour
* Foci of Cystic degeneration, Haemorrhage and Necrosis

Microscopic Findings:
* Classic triphasic combination of stromal, blastemal and epithelial cell types
- Blastemal component: Sheets of small blue cells
- Stromal component: Fibrocytic or Myxoid cells
- Epithelial component: Abortive Tubular or Glomeruli

86
Q

CF of WILMS tumour

A
  • Commonly: Palpable abdominal mass, extending across the midline and down into the pelvis
  • Less often: Fever, Abdominal Pain, and Haematuria
87
Q

Prgnosis of WILMS tumour

A

VERY GOOD

patho II (29 decks)

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