ADPKD
Detailed family history is essential.
Most common presenting complaint in adults is pain.
Hypertension, polyuria, gross hematuria, nephrolithiasis, and UTIs are common.
ARPKD
Oligohy...
No currently approved targeted treatments to cure or slow progression
Medical management is supportive.
Pain is the most common symptom in ADPKD and can be difficult to treat.
In both conditions, dietary changes depend on the degree of renal failure.
Sodium...
Dell KM. The spectrum of polycystic kidney disease in children. Adv Chronic Kidney Dis. 2011;18(5):339–347. [View Abstract on OvidInsights]
Hoyer PF. Clinical manifestations of...
753.12 Polycystic kidney, unspecified type
753.13 Polycystic kidney, autosomal dominant
753.14 Polycystic kidney, autosomal recessive
Q61.3 Polycystic kidney, unspecified
Q61.19 Other polyc...
Q: What can be done to slow the progression of renal insufficiency in ADPKD?
A: Well-controlled BP and rapid treatment of UTIs may decrease the progression of renal failure.
Q: Should asymptomatic o...
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<bold>Figure 12-21</bold> Infantile polycystic kidney disease. Coronal image of a 24-week fetus demonstrates bilateral enlarged echogenic kidneys (arrows). Severe oligohydramnios is present.
<bold>Figure 12-21</bold> Infantile polycystic kidney disease. Coronal image of a 24-week fetus demonstrates bilateral enlarge...
<bold>Fig GU 8-2. Adult polycystic kidney disease.</bold> Nephrogram phase from selective arteriography of the left kidney demonstrates innumerable cysts ranging from pinhead size to 2 cm. The opposite kidney had an identical appearance.<sup>6</sup>
<bold>Fig GU 8-2. Adult polycystic kidney disease.</bold> Nephrogram phase from selective arteriography of the left kidney dem...
<bold>Fig GU 28-2. Adult polycystic kidney disease.</bold> (A) Parasagittal sonogram of the right kidney (PK) shows a random distribution of multiple cysts that vary dramatically in size. The normal reniform contour is maintained. (B) Parasagittal sonogram in a young, asymptomatic member of the family shows multiple cysts (C<sub>R</sub>, C<sub>L</sub>) in the right kidney (R) and liver (L). (D, diaphragm; H, head.)<sup>2</sup>
<bold>Fig GU 28-2. Adult polycystic kidney disease.</bold> (A) Parasagittal sonogram of the right kidney (PK) shows a random d...
<bold>Figure 9.42. Polycystic kidney and liver disease.</bold> Frontal <bold>(A)</bold> and lateral <bold>(B)</bold> radiographs of an infant after IVU show the dense nephrogram appearance to the massively enlarged kidneys. The lucencies within the kidneys represent the cysts. <bold>C.</bold> CT image shows multiple cysts in the liver. <bold>D.</bold> CT image through the kidneys in an adult patient show multiple renal cysts. Notice the enhancement of the surro...
<bold>Figure 9.42. Polycystic kidney and liver disease.</bold> Frontal <bold>(A)</bold> and lateral <bold>(B...
Infantile polycystic disease. The dilated cortical and medullary collecting ducts are arranged radially, and the external surface is smooth.
Infantile polycystic disease. The dilated cortical and medullary collecting ducts are arranged radially, and the external surface is smoot...
<bold>Figure 9.43. Polycystic kidney disease with renal failure. A.</bold> CT image shows multiple renal cysts. <bold>B.</bold> CT image through the pelvis shows a transplanted kidney (<i>T</i>).
<bold>Figure 9.43. Polycystic kidney disease with renal failure. A.</bold> CT image shows multiple renal cysts. <bold>B....
<bold><italic>Figure 19-9</bold> Hereditary polycystic kidney disease.</bold> Specimen from a 33-year-old man.
FIG. 3.132. Autosomal dominant polycystic kidney disease. Fat-suppressed T2-weighted image demonstrates extensive bilateral renal involvement with cysts (<italic>arrows</bold>).
FIG. 3.132. Autosomal dominant polycystic kidney disease. Fat-suppressed T2-weighted image demonstrates extensive bilateral renal involveme...