von Hippel-Lindau disease and adenoma sebaceum
Congenital nephrosis (Finnish type) and medullary cysts
Tuberous sclerosis and angiomyolipoma
Autosomal dominant polycystic kidney disease (ADPKD) and salt-losing nephropathy
Autosomal recessive polycystic kidney disease (ARPKD) and colonic diverticulosis
found in young men and boys.
renal artery intimal disease.
Age of the patient
Duration of renal failure
Recent initiation of hemodialysis
Escherichia coli infection
Genetic defect on chromosome 16
b and c
(c) In newborns, ultrasound findings include very enlarged kidneys with increased parenchymal echogenicity.
(a) The most severe forms develop later in childhood or adolescence.
(b) No matter the severity of the renal disease, all patients will have liver involvement in the form of congenital hepatic fibrosis.
a and b
Proliferation of epithelial cells in segments of the renal collecting system
Hypertrophy of the basement membrane within the ascending loop of Henle
An imbalance of the secretory and absorptive properties in proliferating tubular epithelial cells
Glomerular outpouching resulting from elevated glomerular hydrostatic pressure
Accumulation of fluid within an expanding segment of the glomerulus
The incidence of renal cell carcinoma in ADPKD is twice that in the normal population.
The genetic defect is located on the short arm of chromosome 16.
Renal cysts are infrequently seen on ultrasonographic scans of affected patients before 30 years of age.
Most affected infants have congenital hepatic fibrosis.
Glomerular cysts are never found in the kidneys of newborns diagnosed with ADPKD.
intracranial (berry) aneurysms.
mitral valve prolapse.
Cysts are usually found in communication with each other when injected intracystically with contrast material.
There is an absence of communication between cysts on ultrasonographic scans.
Multicystic dysplastic kidneys appear more often in females and more often on the right side.
The majority of multicystic dysplastic kidneys become smaller or ultrasonographically undetectable with time.
The sine qua non for diagnosis of a multicystic dysplastic kidney is the presence of primitive ducts.
renal cell carcinoma.
bleeding into a cyst.
in males between 4 and 30 years of age.
in females younger than 4 years of age and in males older than 30 years of age.
equally in both sexes before 4 years of age and in females after 30 years of age.
equally in both sexes before 4 years of age and in males after 30 years of age.
in males younger than 4 years of age and in females older than 30 years of age.
NPH presents with polyuria and polydipsia, while MCKD does not.
In patients with NPH, renal failure occurs in the third to fourth decade, while in patients with MCKD, renal failure typically occurs in adolescence.
NPH is an autosomal recessive disorder, while MCKD is an autosomal dominant disease.
Most patients with MCKD have extrarenal manifestations of the disease, while patients with NPH are usually affected only in the kidneys.
NPH is diagnosed histologically with severe interstitial fibrosis, while MCKD is diagnosed by the presence of glomerulosclerosis.
Medullary sponge kidney
Acquired renal cystic disease
von Hippel-Lindau disease
In patients with MCDK, the contralateral renal moiety is frequently affected by urologic disease.
MCDK is one of the most common causes of an abdominal mass in the newborn.
Data from large series show that MCDK is associated with an increased risk for hypertension.
MCDK is often difficult to differentiate from severe ureteropelvic junction obstruction.
Roughly 40% of MCDKs will spontaneously involute over time.
Renal angiomyolipoma and cardiac rhabdomyoma
Mitral valve prolapse, renal angiolipoma, and gingival fibromas
Hamartomatous rectal polyps and facial adenoma sebaceum
Multiple renal cysts, hepatic fibrosis, and pheochromocytoma
Renal angiomyolipoma and multiple renal cysts
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