Imported: Renal_ Leonard : Develolpmental Renal Disease

Front 1

if you have evidence that you have kidney problems and other organ involvement you should be thinking

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congenital problem

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Fusion of upper or lower poles of kidney=

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horseshoe kidney

Front 3

where is the abnormal location of a horseshoe kidney?

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Typically located at midline, may be lower than normal (may be pelvic)

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what is renal agenesis?

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absence of one or both kidneys and corresponding ureter(s)

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a baby is born with a flattened nose, low-set ears, recessed chin; limb contractures, pulmonary hypoplasia..what do they have?

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This is Potter Sequence which is associated with bilateral kidney agenesis... this is not compatible with life

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what are they symptoms of unilateral agenesis of the kidney?

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Asymptomatic;

compensatory hyperplasia of contralateral kidney (may undergo glomerulosclerosis)

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oligohydramnios =

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bilateral renal agenesis (potter's sequence)

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Reduced number and size of renal pyramids =

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Hypoplasia

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ectopic kidneys are often located where?

what are they associated with?

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Pelvis

URINE OUTFLOW OBSTRUCTION (or hydronephrosis, pyelonephritis)

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Autosomal Dominant polycystic disease is associated with adults or Children?

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ADPKD = ADult

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Autosomal resistant polycystic disease is associated with adults or Children?

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Children

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Large multicystic kidneys, liver cysts, berry aneurysms =

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adult polycystic kidney disease (ADPKD)

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40 year old presents with hematuria, flank pain, UTI, renal stones, and hypertension. It is found that his Father, his grandmother, and great grandfather have had similar problems..

what will be the typical outcome?

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ADPKD

outcome: chronic renal failure beginning at age 40-60

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Most common renal genetic disease?

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ADPKD

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enlarged cystic kidneys at birth =

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ARPKD

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is PKD (AD or AR) unilateral or bilateral

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BILATERAL

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child has the clinical features of hepatic fibrosis =

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ARPKD

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portal HTN can be seen in what

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ARPKD

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Developmental abnormality with persistence of abnormal lobules of immature metanephric structures and abnormal, disorganized mesenchymal tissue

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Multicystic Renal Dysplasia

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Inheritance pattern for:

Adult PKD

Child PKD

Multicystic Renal Dysplasia

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Adult: AD

Child: AR

Multicystic Renal Dysplasia: Not hereditary

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Acquired Cystic Disease is associated with what?

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Dialysis

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papillary neoplasia is associated with what disease

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Acquired Cystic Disease

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Cortical,; smooth, thin-walled, uniloculated cyst is what kind? malignant or benign?

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Simple cyst (most common)

Benign

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What is the classic triad in prune belly syndrome

-not on test

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Deficiency of abdominal musculature

Urinary tract anomalies

Bilateral cryptochidism (failure of the testes to descend during development)

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cryptorchidism has an increased risk for?

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ball cancer

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38 yr old man presetns with vauge flank pain and has passage of blood clots in urine. he has bilateral flank pain abdominal masses. labs show elevated BUN and Cr. urinalysis reveals hematuria proteniuira and oliguria. A ct discloses bilaterally massively enlarged kidneys. The pt subsequently develops ESRD and gets a renal transplant. What is the likely diagnosis

ADPKD
ARPKD
Medullary sponge kidney
Multicystic renal dysplasia

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ADPKD

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Developmental abnormality with persistence of abnormal lobules of immature metanephric structures and abnormal, disorganized mesenchymal tissues (e.g., immature cartilage)

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Multicystic Renal Dysplasia

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-Kidneys are enlarged by generally thin, radially oriented (perpendicular to renal capsule) cysts within the cortex and medulla (sponge-like appearance)

– Exterior surface of the kidney is smooth, not disrupted by bulging surface cysts

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ARPKD

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Congenital hepatic fibrosis: may be predominant feature in older children

– Portal hypertension, splenomegaly

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ARPKD

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Mutation in PKHD1 gene, seen in? Codes for?

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ARPKD

Codes for fibrocystin

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Cobble-stone appearance of kidneys

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ADPKD

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PKD1 gene (16p13.3) – codes for polycystin-1

– PKD2 gene (4q21) – codes for polycystin-2

Seen in? Worse prognosis?

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Seen in ADPKD

PKD1 has worse prognosis (also most common)

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Cortical; smooth, thin-walled, uniloculated

– Benign; may be hemorrhagic and confused with cystic RCC on certain imaging studies

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Simple Renal Cysts

**Most common form of renal cysts**