Medullary cystic kidney disease
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Medullary cystic kidney disease
Renal system
Renal and ureteral disorders
Renal agenesis
Horseshoe kidney
Potter sequence
Hyperphosphatemia
Hypophosphatemia
Hypernatremia
Hyponatremia
Hypermagnesemia
Hypomagnesemia
Hyperkalemia
Hypokalemia
Hypercalcemia
Hypocalcemia
Renal tubular acidosis
Minimal change disease
Diabetic nephropathy
Focal segmental glomerulosclerosis (NORD)
Amyloidosis
Membranous nephropathy
Lupus nephritis
Membranoproliferative glomerulonephritis
Poststreptococcal glomerulonephritis
Goodpasture syndrome
Rapidly progressive glomerulonephritis
IgA nephropathy (NORD)
Lupus nephritis
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
Bladder and urethral disorders
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
Renal system pathology review
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
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Medullary cystic kidney disease p. 628
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Transcript
Content Reviewers
Rishi Desai, MD, MPHContributors
Tanner Marshall, MS
Nephronophthisis, which means “nephron wasting”, and medullary cystic kidney disease, which refers to fluid-filled sacs in the medulla, are two kidney diseases that share some similar features: they’re both genetic, they both affect the nephrons, and both can lead to kidney cysts and renal failure over time.
To help understand these diseases, let’s first take a zoomed-in look at a nephron and talk about how it works. Alright so the outer layer of the kidney is called the cortex, and this is where the glomeruli live, which is where blood is initially filtered into the nephron, as well as the proximal convoluted tubule, where some of the filtered substances are reabsorbed back into the body.
The filtered substances, or filtrate, that don’t get reabsorbed then moves down through the medulla via the descending and then ascending parts of the loop of Henle.
The filtrate then goes back to the cortex briefly in the distal convoluted tubule, and then returns back to the medulla in the collecting duct.
Zooming back out a bit, the collecting ducts in each region of the kidney - called a renal pyramid, converge on the renal papilla, which dumps fully formed urine into a minor calyx.
From there the urine goes into the major calyx, and soon after, it goes into the ureter and the bladder.
And finally, zooming back in, surrounding each nephron’s tubule is the tubular interstitium, a hypertonic environment optimized to help resorb water and other substances from the tubules.
Alright so in nephronophthisis, which presents in childhood, the tubules atrophy and the interstitium gets infiltrated by macrophages and becomes fibrotic.
Inflammation of the tubules and the interstitium qualifies nephronophthisis as a tubulointerstitial nephritis, but don’t confuse this with nephritic syndrome, which is where red blood cells and protein escape in the urine as a result of damage to the glomerulus.
Sources
- "Robbins Basic Pathology" Elsevier (2017)
- "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
- "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
- "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
- "Association between congenital defects in papillary outgrowth and functional obstruction in Crim1 mutant mice" The Journal of Pathology (2012)
- "Neonatal vascularization and oxygen tension regulate appropriate perinatal renal medulla/papilla maturation" The Journal of Pathology (2016)
- "Ultrasound to address medullary sponge kidney: a retrospective study" BMC Nephrology (2020)
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