Renal agenesis

Last updated: February 04, 2023

Renal agenesis

Nephrology

Nephrology

Renal system anatomy and physiology
Hydration
Body fluid compartments
Movement of water between body compartments
Renal clearance
Glomerular filtration
TF/Px ratio and TF/Pinulin
Measuring renal plasma flow and renal blood flow
Regulation of renal blood flow
Tubular reabsorption and secretion
Tubular secretion of PAH
Tubular reabsorption of glucose
Urea recycling
Tubular reabsorption and secretion of weak acids and bases
Proximal convoluted tubule
Loop of Henle
Distal convoluted tubule
Renin-angiotensin-aldosterone system
Sodium homeostasis
Potassium homeostasis
Phosphate, calcium and magnesium homeostasis
Osmoregulation
Antidiuretic hormone
Kidney countercurrent multiplication
Free water clearance
Vitamin D
Erythropoietin
Physiologic pH and buffers
Buffering and Henderson-Hasselbalch equation
The role of the kidney in acid-base balance
Acid-base map and compensatory mechanisms
Respiratory acidosis
Metabolic acidosis
Plasma anion gap
Respiratory alkalosis
Metabolic alkalosis
Horseshoe kidney
Potter sequence
Hyperphosphatemia
Hypophosphatemia
Hypernatremia
Hyponatremia
Hypermagnesemia
Hypomagnesemia
Hyperkalemia
Hypokalemia
Hypercalcemia
Hypocalcemia
Renal agenesis
Renal tubular acidosis
Minimal change disease
Diabetic nephropathy
Focal segmental glomerulosclerosis (NORD)
Amyloidosis
Membranous nephropathy
Lupus nephritis
Membranoproliferative glomerulonephritis
Poststreptococcal glomerulonephritis
Rapidly progressive glomerulonephritis
IgA nephropathy (NORD)
Alport syndrome
Kidney stones
Hydronephrosis
Acute pyelonephritis
Chronic pyelonephritis
Prerenal azotemia
Renal azotemia
Acute tubular necrosis
Postrenal azotemia
Renal papillary necrosis
Renal cortical necrosis
Chronic kidney disease
Polycystic kidney disease
Multicystic dysplastic kidney
Medullary cystic kidney disease
Medullary sponge kidney
Renal artery stenosis
Renal cell carcinoma
Angiomyolipoma
Nephroblastoma (Wilms tumor)
WAGR syndrome
Beckwith-Wiedemann syndrome
Posterior urethral valves
Hypospadias and epispadias
Vesicoureteral reflux
Bladder exstrophy
Urinary incontinence
Neurogenic bladder
Lower urinary tract infection
Transitional cell carcinoma
Non-urothelial bladder cancers
Congenital renal disorders: Pathology review
Renal tubular defects: Pathology review
Renal tubular acidosis: Pathology review
Acid-base disturbances: Pathology review
Electrolyte disturbances: Pathology review
Renal failure: Pathology review
Nephrotic syndromes: Pathology review
Nephritic syndromes: Pathology review
Urinary incontinence: Pathology review
Urinary tract infections: Pathology review
Kidney stones: Pathology review
Renal and urinary tract masses: Pathology review
Osmotic diuretics
Carbonic anhydrase inhibitors
Loop diuretics
Thiazide and thiazide-like diuretics
Potassium sparing diuretics
ACE inhibitors, ARBs and direct renin inhibitors
Pediatric urological conditions: Clinical
Elimination disorders: Clinical
Hyponatremia: Clinical
Hyperkalemia: Clinical
Hypokalemia: Clinical
Parathyroid conditions and calcium imbalance: Clinical
Metabolic and respiratory acidosis: Clinical
Metabolic and respiratory alkalosis: Clinical
Toxidromes: Clinical
Medication overdoses and toxicities: Pathology review
Environmental and chemical toxicities: Pathology review
Acute kidney injury: Clinical
Chronic kidney disease: Clinical
Urinary tract infections: Clinical
Nephritic and nephrotic syndromes: Clinical

Transcript

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Alright, so renal agenesis—genesis is the origin or formation of something, and the prefix a means not, and renal refers to the kidneys, so renal agenesis is when the kidneys don’t form. Since there are two kidneys, renal agenesis can refer to just one kidney not developing, called unilateral renal agenesis, or URA, or neither kidney developing, called bilateral renal agenesis, or BRA.

Alright so during fetal development, first off, you’ve got this structure called the mesonephric duct which is involved in development of urinary and reproductive organs, and during the 5th week of gestation, a little guy called the ureteric bud starts pushing its way into another structure called the metanephric blastema, and together, these two little embryologic structures go on to develop into a kidney. At about the 7th week, nephrogenesis, or formation of the kidneys, starts under the influence of that ureteric bud.

By about 20 weeks, the ureteric bud has formed the ureters, the renal calyces, collecting ducts, and collecting tubules, while the metanephric blastema develops into the nephron itself, which includes the epithelial cells and the podocytes of the Bowman’s capsule.

In the third trimester and throughout infancy, the kidneys continue to grow and mature.

With renal agenesis, the ureteric bud fails to induce development of the metanephric blastema, and so either one or both kidneys don’t develop. Although not completely known, it’s thought that this is a result of a combination of genetic as well as in utero environmental factors like toxins and infections.

Newborns with unilateral renal agenesis are usually asymptomatic if the other kidney’s otherwise healthy. Now that one kidney’s doing all the filtering, though, over time unilateral renal agenesis can lead to hypertrophy, or growth of the kidney, which later in life can increase the risk of hypertension as well as renal failure.

Alright so let’s move to bilateral agenesis, which is where there are no kidneys. Normally, as the kidneys start to function around week 16, they start to filter the fetal blood, which means that some fetal urine gets produced, some of which contributes to the amniotic fluid that the fetus floats around in.

Key Takeaways

Renal agenesis is a medical condition in which fetal kidneys fail to develop, it may be either unilateral or bilateral. This results in the absence of one or both kidneys, which can lead to several complications. Bilateral renal agenesis is incompatible with life because prolonged absence of amniotic fluid results in pulmonary hypoplasia leading to severe respiratory insufficiency at birth.

People with unilateral renal agenesis may have no symptoms, while others may experience symptoms such as high blood pressure, swelling, and difficulty urinating. Diagnosis of renal agenesis can be done through imaging tests such as ultrasound, CT scan, or MRI. Treatment typically involves managing symptoms and complications, such as high blood pressure and kidney failure. In cases of bilateral renal agenesis, dialysis or a kidney transplant may be necessary.

Sources

  1. "Robbins Basic Pathology" Elsevier (2017)
  2. "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
  3. "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
  4. "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
  5. "Control of Murine Kidney Development by Sonic Hedgehog and its GLI Effectors" Cell Cycle (2006)
  6. "A Gene Implicated in Activation of Retinoic Acid Receptor Targets Is a Novel Renal Agenesis Gene in Humans" Genetics (2017)
  7. "Special Imaging Casebook" Journal of Perinatology (2000)