Embriologi Ginjal

O The nephrogenic cord develops into three

sets of nephric structures: the pronephros, mesonephros, and metanephros.

O 3 embryonic kidneys (from intermediate

mesoderm)O Pronephros- transient (week 3-5), nonfunctional,

5-7 paired segments.

Pronephros
Develops by the differentiation of mesoderm

within the nephrogenic cord to form pronephric tubules and the pronephric duct.*

Pronephros
The pronephros is the cranialmost nephric

structure and is a transitory structure that regresses completely by week 5.

The pronephros is not functional in humans.

O Mesonephros- transient (week 4-

month 4), excretory organ while metanephros begins development.

O Formation of nephric ducts/wolffian ducts

precedes development of mesonephric tubules. O Small number of elements from mesonephros persist to form reproductive tract.
O Males- efferent ductules of testes, epididymis

and vas- wolfian origin. O Females- nonfunctional mesosalpingeal structures.

Mesonephros

Mesonephros
Develops by differentiation of mesoderm within the

nephrogenic cord to form mesonephric tubules and the mesonephric duct (wolffian duct).*

Mesonephros
Most of the mesonephric tubules regress, but the mesonephric duct persists and opens into the urogenital sinus. * The mesonephros is functional for a short period.

Metanephros
Develops from an outgrowth of the

mesonephric duct (called the ureteric bud) and from a condensation of mesoderm within the nephrogenic cord called the metanephric mesoderm.

O
O Metanephros- definitive kidneys O Form as ureteric buds (from distal end of nephric duct) O Penetrates metanephric mesenchyme at 28 days to begin nephron formation- lobulated appearance. O Metanephric mesoderm forms nephron or excretory unit (glomerulus, proximal tubule, loop of Henle, distal tubule)form from metanephric mesenchyme.
O Older nephrons on inner part of kidney, newer at periphery.

Metanephros
The metanephros begins to form at week 5 and is

functional in the fetus at about week 10.

It develops into the definitive adult kidney.

Metanephros
The fetal kidney is divided into lobes in contrast to

the definitive adult kidney, which has a smooth contour.

Development of the Metanephros

Two Systems:* Collecting System* Excretory (Nephron) System*

Development of the Collecting System

The ureteric bud initially penetrates the metanephric mesoderm, and then undergoes repeated branching to form the ureters, renal pelvis, major calyces, minor calyces, and collecting ducts.

Development of the Collecting System

The ureteric bud initially penetrates the metanephric mesoderm, and then undergoes repeated branching to form the ureters, renal pelvis, major calyces, minor calyces, and collecting ducts.

Development of the Excretory System

The inductive influence of the collecting ducts causes the metanephric mesoderm to differentiate into metanephric vesicles which later give rise to primitive S-shaped renal tubules, which are critical to nephron formation.

Development of the Excretory System

The S-shaped renal tubules differentiate into the distal convoluted tubule, loop of Henle, proximal convoluted tubule, and Bowman's capsule.

Development of the Excretory System

Tufts of capillaries called glomeruli protrude into Bowman's capsule.

Development of the Excretory System

Nephron formation is complete at birth, but functional maturation of nephrons continues throughout infancy.

Ascent of the Kidneys

The fetal metanephros is located at vertebral level S1-S2, whereas the definitive adult kidney is located at vertebral level T12-L3.

The change in location results from a disproportionate growth of the embryo caudal to the metanephros.

Ascent of the Kidneys

During the ascent, the kidneys rotate 90causing the hilum, which initially faces ventrally, to finally face medially.

Blood Supply of the Kidneys

During the ascent of the kidneys, the kidneys receive their blood supply from arteries at progressively higher levels until the definitive renal arteries develop at L2. Arteries formed during the ascent may persist and are called supernumerary arteries. Supernumerary arteries are end arteries. Therefore, any damage to them results in necrosis of kidney parenchyma.

Congenital Malformations
O Renal agenesis
O Unilateral O Bilateral

O Renal hypoplasia O Congenital cystic kidneys

O Types 1 -5

O Horseshoe (fused) kidneys

O Wilms tumor

O Absence of kidneys

Renal Agenesis

O Unilateral (compatible with life)

O Affects 1 in every 800-1500 people O May occasionally present with genitalia anomolies O Trisomy of 18 O Addition or partial trisomy of 13 O Prenatal rubella infection

O Bilateral (incompatible with life)

O 40% stillborn O Of those born alive 95% die within 24 hours of birth O Potter syndrome and associated oligohydramnios

Renal agenesis occurs when the ureteric bud fails to develop, thereby eliminating the induction of metanephric vesicles and nephron formation.

Renal Agenesis
Unilateral renal agenesis is more common in males. This situation is asymptomatic and compatible with life because the remaining kidney hypertrophies.

Bilateral Renal Agenesis

Causes oligohydramnios, which results in compression of the fetus, Potter syndrome, (deformed limbs, wrinkly skin, abnormal facial appearance [flattened nose, wide interpupillary space low set ears] and tapering fingers).

Infants with bilateral renal agenesis are usually stillborn or die shortly after birth.

Renal hypoplasia
O Incomplete development of kidneys
O Unilateral (compatible with life) O Bilateral (incompatible with life) if condition

is severe
O Kidneys are small
O Decreased functional parenchyma

Congenital cystic kidneys

O Type 1
O Polycystic kidneys found in infants
O Bilateral and results in early death O Large renal pelvis and calyces

O Type 2
O Cysts are variable in size and shape O Usually unilateral O Affected kidney non functional

Congenital cystic kidneys (cont.)

O Type 3 O Affected kidneys contain both normal and abnormal tissue O Both kidneys involved O Autosomal dominant gene
O Trisomy of 13-15, 18, 21, 22

O Type 4 O Caused by urethral obstruction O If severe early death O Type 5 O Manifests during adult life, death by 50. O Autosomal dominant

Horseshoe (fused) kidney

O Fusion of two kidneys at their lower end
O Tissue that connects kidneys = isthmus

O 1:400 O Trisomy 13-15; 18, 21, Turners syndrome,

mosaicism

Renal Fusion
The most common type of renal fusion is the horseshoe kidney.

Renal Fusion
A horseshoe kidney occurs when the inferior poles of the kidneys fuse across the midline. Normal ascent of the kidneys is arrested because the fused portion gets trapped beneath the inferior mesenteric artery. Kidney rotation is also arrested so that the hilum faces ventrally.

Wilms Tumor
O Renal tumor of children
O Characterized by rapid growth and early

metastasis O Mesodermal origin

O Metanephric tissue that have failed to

differentiate into normal kidney tissue

Embryonic development
O Urinary system, internal

reproductive organs and external genitalia O Develop synchronously at an early embryologic age(table 5.6)