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Congenital Anomalies, Cystic Diseases of the Kidney and Hereditary Nephritis- Dr. Trif (Final Exam)

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What are the 3 sequential developmental phases to form the urogenital system?
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What are the 3 sequential developmental phases to form the urogenital system?
1) Pronephros: arises at 3 wks as segmental collections of mesoderm in the cervical region. Attached to paired pronephric ducts. It is nonfunctional in humans and undergoes regression at 4 wks.
2) Mesonephros: begins to develop as pronephros degenerates. Consists of glomeruli and mesonephric tubules which open into paired mesonephric (Wolffian) ducts, which are continuations of the pronephric ducts. It forms elongated interim kidneys that produce little, if any, urine in humans and undergoes regression at 5 weeks.
3) Metanephros: the permanent kidneys begin to develop as the mesanephros degenerates. Result from interaction b/w ureteric bud, a diverticulum from the mesonephric duct, and the metanephric mesenchyme (metanephric blastema). The collecting tubules, collecting ducts, calyces, renal pelvis and the ureter are all derived from ureteric bud. The collecting tubules induce the surrounding mesenchyme to grow and become tubules that form parts of the nephron: glomerulus, Bowman's capsule, convoluted tubules and the loop of Henle.
Image: What are the 3 sequential developmental phases to form the urogenital system?
Ascent of the kidneys
At 5-6 weeks the mature kidneys lie in the pelvis with their hilus directed anteriorly. With continued growth and elongation of the embryo, the kidneys move upward and eventually lie in a retroperitoneal position in the abdomen rotated so that the hilus face antero-medially. As the kidneys ascend, new blood vessels arise sequentially from the iliac artery, the distal aorta and finally, the upper abdominal aorta.
Image: Ascent of the kidneys
What is renal agenesis?
Failure of one or both kidneys to develop, usually due to ureteric bud degeneration and/or failure to reach the metanephric blastema.
1) Unilateral: more common, and compatible w/ life--> existing kidney undergoes compensatory hypertrophy
2) Bilateral: babies afflicted die shortly after birth. One of the causes of reduced formation of amniotic fluid (oligohydramnios) and is associated with Potter Syndrome.
Image: What is renal agenesis?
What is Potter Syndrome (sequence)?
1) Potter facies- flattened nose, recessed chin, prominent epicanthal folds, and low set abnormal ears
2) Pulmonary hypoplasia
3) Other malformations including skeletal (sirenomelia=mermaid syndrome), opthalmologic, abdominal and CV
4) Assoc. with bilateral agenesis
Image: What is Potter Syndrome (sequence)?
What is renal hypoplasia?
Condition in which there is a reduction in renal mass and # of nephrons, usually unilateral. When bilateral, kidneys are functional for 5-10 yrs. Constitute 20% of juvenile population on dialysis.
Image: What is renal hypoplasia?