Development Of Kidney

Question 1

From which Structures entire urogenital system is developed?

Answer 1

Intermediate mesoderm
Cloaca (endodermal Structure)

Question 2

What is cloaca?

Answer 2

It is hindgut distal to allantois

Question 3

Derivatives of cloaca

Answer 3

Primitive urogenital sinus
Primitive rectum

Question 4

Derivatives of Primitive urogenital sinus

Answer 4

1) Epithelial buds - prostate is developed from this structure

2) Vesicourethral canal - Bladder and prostatic urethra till opening of ejaculatory ducts

3) Definitive urogenital sinus

Question 5

Parts of definitive urogenital sinus

Answer 5

Pelvic part - From this part remaining prostatic urethra and membranous urethra are developed

Phalic part - Spongy urethra for males and vestibule for females is developed

Question 6

Derivatives of intermediate mesoderm

Answer 6

Mesonephric duct (wolffian duct)
Paramesonephric duct (mullerian duct)
Urogenital ridge

Question 7

Collecting system of kidney derived from?

Answer 7

Mesonephric duct - from mesonephric duct ureteric buds are developed , from ureteric buds collecting system of kidney is derived

Question 8

Excretory part of kidney derived from?

Answer 8

Urogenital ridge

Question 9

Extension of urogenital ridge

Answer 9

Cervical to sacral region of embryo ,situated adjacent to wolfian duct

Question 10

Derivatives of urogenital ridge

Answer 10

They have two pairs of swellings in either side of dorsal aorta

Medial pair of swellings are genital ridge

Lateral pair of swellings are nephrogenic cord

Question 11

What occurs in day 22(4th week) of iul?

Answer 11

Formation of pronephros
Regresess in few days

Question 12

What occurs in end of 4th week of iul ?

Answer 12

Formation of mesonephros
Regress by 4th month

Question 13

What happens in 5th week of iul?

Answer 13

Formation of mesenchyme of metanephric blastema from nephrogenic cord

Formation of epithelium of ureteric bud from mesonephric duct

Question 14

What occurs in 6th to 8th week of iul?

Answer 14

Epithelial mesenchymal interaction /transition (epithelium of ureteric bud and mesenchyme of metanephric bud)

Question 15

Factor required for epithelial mesenchymal interaction

Answer 15

WT1 factor

Transcription factor

Present in chr 11

Question 16

What happens in end of 8th week of iul?

Answer 16

1st Fully formed nephron

Question 17

What occurs in 9 to 10 weeks of iul?

Answer 17

Urine formation
Ascend of kidney with rotation

Question 18

What happens in 32 to 36 weeks of iul?

Answer 18

Completion of nephrogenesis

60 to 70% of nephrons are formed in 3rd trimester

Question 19

At what age urine concentration capacity reaches adult level?

Answer 19

At 1.5yrs

Question 20

Very important parameter defining number of nephrons ?

Answer 20

Birth weight

Question 21

What is ckd?

Answer 21

Reduction in number of functional nephrons

Presence of interstitial fibrosis, tubular atrophy, glomerulosclerosis

Question 22

Factor responsible for formation of ureteric bud?

Answer 22

PAX-2

Question 23

Location of kidney in fetus and adults

Answer 23

Adult T12 to L3

Fetus S1 to S2 (at 9 to 10 months of iul only ascend of kidney due to rotation occur)

Question 24

Diseases due to WT1 mutation

Answer 24

WAGR syndrome
Dennys Drash syndrome
Frasiers syndrome

Question 25

Components of WAGR syndrome?

Answer 25

Wilms tumour Aniridia Genitourinary abnormalities Mental retatdation

Question 26

Components of Dennys drash syndrome

Answer 26

Wilms tumour Disorders of sexual development (male pseudohermophroditism) Diffuse mesangial sclerosis (because of this there is early onset ckd 3-5yrs)

Question 27

Components of Frasiers Syndrome

Answer 27

Gonadoblastoma Gonadal dysgenesis but external genetalia normal In biopsy we can find focal segmental glomerulosclerosis Renal failure by 15 to 20 yrs

Question 28

Difference between Dennys drash syndrome and frasiers syndrome

Answer 28

Tumor in Dennys drash is wilms but in frasiers is gonadoblastoma Male pseudohermophroditism in Dennys but external genetalia normal in frasiers Biopsy findings in Dennys is diffuse mesangial sclerosis but in frasiers there is focal segmental glomerulosclerosis Early onset CKD (3 to 5 yrs) in Dennys drash but in Frasiers renal failure occur at 15 to 20yrs of age

Question 29

Diseases due to WT2 mutation

Answer 29

Beckwith wiedmann syndrome Russel silver syndrome

Question 30

Examples of genomic imprinting abnormality

Answer 30

Beckwith wieldmann syndrome Russel silver syndrome Prader willi syndrome Angelman syndrome

Question 31

Presentations of Beckwith wiedmann syndrome

Answer 31

Organomegaly (Nephromegaly,hepatomegaly,adrenomegaly) Because of organomegaly umblical hernia occur Wilms tumour Microcephaly Macroglossia Hypoglycemia Medullary sponge kidney (in adults) Hemi hypertrophy of limbs

Question 32

Russel silver syndrome presentation

Answer 32

Triangular facies Mental retardation

Question 33

Reason for unilateral renal agenesis

Answer 33

PAX-2 mutation

Question 34

Associations of unilateral renal agenesis

Answer 34

Optic nerve Coloboma Dextrocardia Hypertension (due to decrease in number of functional nephrons) Flat foot

Question 35

What is bilateral renal agenesis called?

Answer 35

Potter's syndrome

Question 36

What gene involved in bilateral renal agenesis?

Answer 36

RET gene/GDNF protein mutation

Question 37

Radiological features in potter's syndrome

Answer 37

Pulmonary hypoplasia Bilateral absence of kidneys Disc shaped adrenal gland

Question 38

What is appendix of epididymis ?

Answer 38

Remanant of mesonephric duct

Question 39

What is appendix of testis in males and hydatid of morgagni in females ?

Answer 39

They are remanant of paramesonephric duct