Flashcards in Kidney Deck (379)
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1
Infantile polycystic kidney disease: Inheritance.
Autosomal recessive.
2
Infantile polycystic kidney disease:
A. Gene and its location.
B. Encoded protein.
A. PKHD1 on chromosome 6p21-23.
B. Polyductin / fibrocystin.
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Infantile polycystic kidney disease: Presentation (2).
Abdominal distention.
Stillbirth or early neonatal death in most cases.
4
Infantile polycystic kidney disease: Associated abnormalities (2).
Pulmonary hypoplasia.
Congenital hepatic fibrosis.
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Infantile polycystic kidney disease: Gross pathology.
Massive enlarged kidneys with smooth surface.
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Infantile polycystic kidney disease:
A. Location and arrangement of cysts.
B. Calyceal system.
A. Cortex and medulla; oriented perpendicular to the renal capsule.
B. Normal.
7
Infantile polycystic kidney disease: Histopathology (2).
Dilated collecting ducts lined by bland cuboidal cells.
Nephrons appear normal.
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Medullary cystic disease: Presentation.
Renal dysfunction in childhood:
− Polyuria and polydipsia.
− Uremia.
− Growth retardation.
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Medullary cystic disease: Gross pathology.
Kidneys are small; both are affected.
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Medullary cystic disease: Number, size, and location of cysts.
Many.
Up to 2 cm in diameter.
Corticomedullary junction.
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Medullary sponge kidney: Presentation.
Found in children or adolescents.
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Medullary sponge kidney: Gross pathology.
Kidneys are typically of normal size.
13
Medullary sponge kidney: Size and location of cysts.
Less than 0.5 cm.
Medullary pyramids, renal papillae; communication with collecting ducts.
14
Medullary sponge kidney: Prognosis.
Renal function is usually normal.
Progression to end-stage renal disease occurs rarely.
15
Renal dysplasia: Clinical significance (2).
Dysplastic kidneys usually do not function.
Nearly always accompanied by other abnormalities of the urinary tract.
16
Renal dysplasia: Presentation (2).
If large: Palpable mass.
If small: May remain asymptomatic for many years.
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Renal dysplasia: Possible outcomes in utero (2).
Oligohydramnios with Potter's sequence.
Death from pulmonary hypoplasia.
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Renal dysplasia: Gross pathology.
Distorted kidneys with variable of cysts.
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Renal dysplasia: Histopathology (3).
Disorganized renal parenchyma containing cartilage.
Dysplastic ducts lined by columnar epithelium and surrounded by spindle cells.
Cystic spaces lined by flat epithelium.
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Adult polycystic kidney disease: Type of inheritance.
Autosomal dominant.
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Adult polycystic kidney disease: Genes, locations, proteins (2).
PKD1, chromosome 16, polycystin 1.
PKD2, chromosome 4, polycystin 2.
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Presentation of adult polycystic kidney disease:
A. Age of patient.
B. Laboratory abnormalities (2).
A. Fourth or fifth decade.
B. Hematuria or proteinuria.
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Adult polycystic kidney disease: Extrarenal manifestations (4).
Berry aneurysms.
Colonic diverticula.
Cysts in pancreas, liver.
Cardiac valvular abnormalities.
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Adult polycystic kidney disease: Gross pathology (2).
Early: Normal-sized kidneys with a few cysts.
Later: Markedly enlarged kidneys with irregular contour due to cysts.
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Adult polycystic kidney disease: Size and location of cysts.
From a few millimeters to several centimeters in diameter.
Cortical and medullary.
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Adult polycystic kidney disease: Histopathology (2).
Cysts: Flat or cuboidal epithelial lining.
Parenchyma: Fibrosis, atrophy.
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Adult polycystic kidney disease: Complications (2).
End-stage renal disease.
Renal-cell carcinoma.
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Acquired cystic disease: Presentation (2).
Usually asymptomatic.
Cysts develop in the setting of chronic renal failure, usually dialysis dependent.
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Acquired cystic disease:
A. Inheritance.
B. Extrarenal manifestations.
A,B. None.
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