Introduction to pediatric nephrology
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Introduction to Pediatric Nephrology. Dr.Fahad Gadi, MD Pediatrics Demonstrator King Abdulaziz University Rabigh Medical School. Kidney ontogenesis.

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Introduction to pediatric nephrology

Introduction to Pediatric Nephrology

Dr.Fahad Gadi, MD

Pediatrics Demonstrator

King Abdulaziz University

Rabigh Medical School


Kidney ontogenesis
Kidney ontogenesis

  • The embryological development of the kidney is a long and continuous process which begins in the 3rd week and is completed by about 34-35 weeks of fetal life.

  • Kidney organogenesis is characterised by 3 distinct and linked stages: pronephros, mesonephros and metanephros.


Kidney ontogenesis1
Kidney ontogenesis

  • In humans, the first two are transient structures with little excretory capacity but they are important for the appropriate development of the metanephros, which is the direct precursor of the adult kidney.


Metanephros
METANEPHROS

  • The final stage of the kidney is the differentation of the metanephros and arise from the ureteric bud and the metanephric blastema (mesenchyme).

  • The renal pelvis, major and minor calyces and terminal collecting duct are formed by the 10-13th wks of ges.

  • After morphogenesis each kidney contains approx a million nephrons.


Renal development

aorta

nefrotomy

Pronephros

Przednercze

3 t.ż.

4-8 t.ż.

przewód Wolffa

Mesonephros

Śródnercze

5 t.ż.

stek

pączek moczowodowy

Nerka ostateczna

Metanephros

blastema nerki ostatecznej


Antenatal period
Antenatal Period

  • The most common cause is physiologic dilation.

  • Metanephric urine production begins at 8 weeks, even before ureteral canalization is complete.

  • Transient obstruction with hydronephrosis occurs.



Molecular aspect
MOLECULAR ASPECT

  • The development of the metanephric kidney depends on inductive interaction between the ureteric bud (UB) and the metanephric mesenchyme (MM).

  • A large number of genes have been found to be crucial during kidney development.


Nephrons
Nephrons

  • In the fetus at 36 weeks’ gestation there is an adult complement of nephrons- approx. one million

  • All further growth of the kidney is via hyperplasia mainly in the tubules.


Fetal kidney
Fetal kidney

  • Nephrogenesis is completed between the 28 and 36th gestational week in the human, the renal tissue and particularly the tubular cells continue to develop postnatally.

  • Several of the major transporters in the tubular epithelial cells undergo postnatal maturation


Fetal kidney1
Fetal kidney

  • Outer cortical glomeruli are relatively underperfused compared with inner cortical glomeruli.

  • Following birth, renal perfusion to superficial cortical nephrons rises compared with deeper glomeruli


Fetal kidney2
Fetal kidney

  • Angiotensin-converting enzyme inhibitors and angiotensin-receptor antagonists impair nephrogenesis and so are contraindicated in pregnancy


Production of urine
Production of urine

  • Production of urine starts at the age of 10-12 weeks of gestation:

  • 1. very dilute urine

  • 2. small amount of urine

  • Fetal urine is a major constituent of amniotic fluid and urinary flow rate increases from 12ml/hr at 32 weeks’gestation to 28ml/hr at 40 weeks’gestation.

  • Similar increases are described during the maturation of premature newborns.


Glomerular filtration rate gfr
Glomerular Filtration Rate (GFR)

  • Glomerular filtration begins between the 9th and 12th week of gestation in humans.

  • The GFR is relatively low at birth especially in the premature infant.

  • The values of GFR nearly double between 3 and 7 days and thereafter GFR continues to increase, by 1 to 2 yrs of age the GFR is the same as in an older child- 80% of mature kidney.



Kidney of newborn
Kidney of newborn

  • The kidney of the newborn infant has a limited capacity to regulate the excretion of fluid and electolytes.

  • The high sodium excretion during the first 2 to 3 weeks often results in a negative sodium balance and predisposes to hyponatremia.


Creatinine clearance
Creatinine Clearance

  • Newborn: 40-65 ml/min/1.73 m2

  • <40 yrs: 97-137 ml/min/1.73 m2


Renal failure in the newborn
Renal failure in the newborn

  • Renal failure in the newborn:

  • severe asphyxia,

  • the majority suffered from nonoliguric renal failure


Cakut
CAKUT

  • Congenital

  • Anomalies of

  • Kidney and

  • Urogenital

  • Tract


Cakut1
CAKUT

  • Chronic renal failure (children):

    Obstructive nephropathy- 47%

    Reflux nephropathy- 18,5%

    Hypo/dysplasia 8,7%


Renal abnormalities
RENAL ABNORMALITIES

  • Renal agenesis:

    bilateral fetal death- Potter syndrome 1:4000 pregnancies

    unilateral other organ- 1:2900 pregnancies abnormalites


Renal abnormalities

Agenesis Aplasia Hypoplasia


Renal abnormalities1
RENAL ABNORMALITIES

  • Hydronephrosis


Renal abnormalities2
RENAL ABNORMALITIES

  • Obstractive uropathy:

    A. ureteropelvic junction obstruction- dilated renal pelvis with/ without caliectasis and no dilation of the ureter

    B. ureterovesical junction obstruction (megaureter)- pelviectasis and caliectasis with significant ureter dilation


Renal abnormalities3
RENAL ABNORMALITIES

  • C. posterior urethral valve

  • D. ureterocele- cystic dilatation of the distal ureter that protrudes into the urinary bladder, may extend past the bladder into urethra

  • E. ectopic ureters

  • F. constriction (stenosis)of urethra


Posterior urethral valve

Type I – Type II Type III


Duplication of urinary tract

UreterUreterUreter

duplex fissus


Vesico-ureteral reflux

Frequency of VUR

• Isolated1% (0.4-4%)

• UTI in the past 29-50%

• Siblings with VUR 32-45%

• Mothers with VUR in the past 60%


Renal abnormalities4
RENAL ABNORMALITIES

  • Polycystic kidney:

    autosomal dominant p.k.disease

    autosomal recessive p.k. disease



Pronephros
PRONEPHROS

  • Pronephros is a transitory non-functional kidney, the first tubules appear the middle of the 3rd week and arise from intermediate mesodermal cells.

  • The pronephric tubules persist for only a short time and undergo degeneration by the 5th week.

  • At the time the pronephros is degenerating the mesonephric tubules and duct are developing.



Vesico ureteral reflux
Vesico- ureteral reflux

Normal kidney, ureter, and bladder


Vesico ureteral reflux1
Vesico- ureteral reflux

Grade I Vesicoureteral Reflux:urine (shown in blue) refluxes part-way up the ureter


Vesico ureteral reflux2
Vesico- ureteral reflux

  • Grade II Vesicoureteral Reflux:urine refluxes all the way up the ureter


Vesico ureteral reflux3
Vesico- ureteral reflux

  • Grade III Vesicoureteral Reflux:urine refluxes all the way up the ureter with dilatation of the ureter and calyces (part of the kidney where urine collects)


Vesico ureteral reflux4
Vesico- ureteral reflux

  • Grade IV Vesicoureteral Reflux:urine refluxes all the way up the ureter with marked dilatation of the ureter and calyces


Vesico ureteral reflux5
Vesico- ureteral reflux

  • Grade V Vesicoureteral Reflux:massive reflux of urine up the ureter with marked tortuosity and dilatation of the ureter and calyces



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