Renal & Urogenital Development Flashcards

1
Q

renal embryology & reproductive embryology - overview

A

*the intermediate mesoderm gives rise to the urinary and genital systems
*intermediate mesoderm → pronephros → mesonephros (gives rise to the gonads) → metanephros (gives rise to the kidneys)

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2
Q

3 kidneys during development

A
  1. pronephros:
    -transient, nonfunctional structures
    -generate the mesonephric duct
  2. mesonephros:
    -functional from about 6-10 weeks of gestation
    -form the trigone of the bladder & give rise to ureteric bud
    -induces formation of paramesonephric (Mullerian) duct
    -form male duct structures in presence of testosterone; largely degenerates without testosterone
  3. metanephros:
    -ureteric bud forms renal pelvis and ureters; branches many times and indues mesenchyme to develop into nephrons
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3
Q

bladder development

A

*urorectal septum formation separates cloaca into urogenital sinus and rectum
*urogenital sinus forms the bladder
*connected to umbilicus by allantois, which condenses into urachus → median umbilical ligament

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4
Q

congenital malformation of the kidney: Wilm’s Tumor

A

*affects children less than 5 years old
*mutation in WT1 gene on chromosome 11
*unilateral abdominal mass that does not cross midline
*may be associated with other abnormalities:
-WAGR syndrome
-Denys-Drash Syndrome
-Beckwith-Wiedmann Syndrome
-Horseshoe kidney

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5
Q

congenital malformation of the kidney: renal agenesis

A

*may be unilateral or bilateral
*bilateral renal agenesis results in renal failure and associated with Potter sequence (anuria, oligohydramnios, hypoplastic lungs)
*may have other severe defects like absence or abnormalities of the vagina and uterus, vas deferens, and seminal vesicles
*common associated defects in other systems: cardiac anomalies, tracheal and duodenal atresias, cleft lip and palate, brain abnormalities

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6
Q

congenital malformation of the bladder:

A

*allantois is a transient connection between the cloaca and umbilicus, which should close off to become the urachus and eventually seal off and become the median umbilic ligament
*failure to close off can result in:
-urachal fistula
-urachal cyst
-urachal sinus

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7
Q

ascent of the kidneys

A

*kidneys normally “ascend” from the pelvic region to the lumbar region as the embryo grows
*the kidneys blood supply changes levels as they ascend, and previous renal arteries degenerate rather than ascending with the kidneys

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8
Q

failure of the kidneys to ascend: pelvic kidney

A

*as growing embryo lengthens, one or both kidneys may not ascent and therefore remain in the pelvis
*pelvic kidneys maintain their fetal blood supply

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9
Q

failure of the kidneys to ascend: horseshoe kidney

A

*kidneys fuse together
*as the embryo lengthens, the fused kidney gets caught on the inferior mesenteric artery
*the IMA “hooks” on to the kidney and drags it down into the pelvis
*can be associated with:
-increased risk of obstruction and stones
-infection
-poor perfusion
-Wilm’s tumor

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10
Q

genital ducts - undifferentiated

A

*early in development, the ducts and gonads are undifferentiated (could give rise to male or female)
*based on the presence or absence of the SRY gene on the Y chromosome, could give rise to male or female structures

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11
Q

SRY and genital development

A

*SRY gene is on the short arm of chromosome Y
*SRY gene makes testis-determining factors, initiating male gonadal differentiation
*TDF induces development of:
1. Sertoli cells - produce anti-Mullerian hormone, inhibiting female ducts
2. Leydig cells - produce testosterone, which supports male ducts (mesonephric / Wolffian) and masculinizes external genitalia

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12
Q

genital ridge: male vs. female derivatives

A

*male: testis
*female: ovary

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13
Q

primordial germ cell: male vs. female derivatives

A

*male: spermatozoa
*female: ova

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14
Q

mesonephric (Wolffian) duct: male vs. female derivatives

A

*male: ductus (vas) deferens, ejaculatory duct, epididymis, trigone bladder
*female: trigone bladder

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15
Q

paramesonephric (Mullerian) duct: male vs. female derivatives

A

*male: utricle of prostatic urethra
*female: uterine tubes, uterus, upper vagina

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16
Q

urogenital sinus (lower): male vs. female derivatives

A

*male: penile urethra
*female: lower vagina, vestibule/labia minora

17
Q

urogenital sinus (upper): male vs. female derivatives

A

*male: bladder, prostatic urethra, membranous urethra
*female: bladder, urethra

18
Q

genital tubercle: male vs. female derivatives

A

*male: penis
*female: clitoris

19
Q

genital swellings: male vs. female derivatives

A

*male: scrotum
*female: labia majora

20
Q

female reproductive structures - clinical correlates

A

*septate uterus (incomplete resorption)
*bicomuate uterus (incomplete fusion)
*didelphys uterus (incomplete fusion)

21
Q

male reproductive structures - clinical correlates

A

*hypospadias (urethral opening on ventral surface of penis)
*epispadias (urethral opening on dorsal surface of penis)
*communicating hydrocele
*abdominal, inguinal, and suprascrotal hernias

22
Q

ambiguous genitalia: congenital adrenal hyerplasia

A

*decreased steroid hormone and increased adrenocorticotropic hormones
*females are masculinized - virilization of female external genitalia
*males can be missed in the early newborn period, but then present with salt-wasting
*21-hydroxylase deficiency is the most common cause

23
Q

ambiguous genitalia: androgen insensitivity syndrome

A

*normal production of testosterone & dihydrotestosterone
*abnormal receptor activity
*androgens unable to induce differentiation of male genitalia
*may be partial or complete

24
Q

ambiguous genitalia: 5-alpha reductase deficiency

A

*inability to convert testosterone to dihydrotestosterone
*virilization at puberty