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Flashcards in Cystic Disease of the Kidney Deck (40)
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1

Renal cystic disease is characterized by what?

Characterized by epithelium-lined cavities filled with fluid or semisolid debris within the kidneys.

2

Cystic Diseases of the Kidney
8

1. Simple cyst
2. Autosomal Dominant Polycystic 3. Kidney Disease
4. Autosomal Recessive
5. Polycystic Kidney Disease
6. Aquired Cystic Kidney Disease
7. Medullary Cystic Disorders
8. Medullary Sponge Kidney

3

Simple Cysts
1. How is it often found?
2. Frequency with what?
3. Present in what percent of the population over 50 yrs of age?
4. How do we differentiate benign from malignant?


5. What are the three criteria for a cyst being malignant or benign?

Often asymptomatic and usually incidental findings during imaging studies.

Increase in frequency with age.

Present in up to 50% of the population over 50 yrs of age.

Renal ultrasound, together with CT scan differentiate benign from malignant lesions in most instances.

5.
1. echo free
2. sharply demarcated
3. enchanced backwall (good transmission through the cyst-just fluid filled and not malignant cells)

4

Polycystic Kidney Disease
2 kinds


What is the difference?

1. Autosomal Dominant Polycystic Kidney Disease (ADPKD)
2. Autosomal Recessive Polycystic Kidney Disease (ARPKD)

Autosomal Dominant Polycystic Kidney Disease (ADPKD)
1. Referred to as adult PKD (Polycystic kidney Disease)

Autosomal Recessive Polycystic Kidney Disease (ARPKD)
2. Referred to as infantile or childhood PKD

5

1. What is suggestive of Autosomal Recessive Polycystic Kidney Disease (ARPKD)?

2. Can occur with what? and causes death from renal failure in how much time?

3. What does renal failure in adolescence cause?

1. Abdominal mass in neonate or older infant suggestive

2. Can occur with congenital fibrosis and causes death from renal failure during the first year of life.

3. Renal failure in adolescence suggested ARPKD or medullary cystic disease.

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7

ADPKD is caused by 2 different genes. WHat are they?


What is the protein with this gene called and what is it responsible for?

What percentage of these people will have ESRD by age 60?

ADPKD1
-Protein from this gene is called polycystin
-Responsible for cell-cell and cell-matrix interaction
ADPKD2 (slower progression of the disease. its on chromosome 4)


50%

8

Why does ADPKD keep getting passed on?


How do pts present? 2

Clinical manifestations rarely occur before age 20-25 years, therefore, affected people of childbearing age pass the genetic trait on to offspring while they are still asymptomatic.

Patients present either from
1. screening because of a family history, or for
2. evaluation of symptoms.

9

Clinical Symptoms of ADPKD?
5



1. Acute abdominal flank pain
2. Back pain
3. Hematuria
-Microhematuria at first
-Gross hematuria may occur
4. Non-specific dull lumbar pain
5. Sharp localized pain

10

What is the sharp localized pain from in ADPKD?
3

1. From cyst rupture,
2. infection or
3. passage of renal stone

11

Common Complications of ADPKD
4

1. Urinary tract infections
2. Pylonephritis (get cultures early- it can go septic really fast)
3. Cyst infections
4. HTN (ACE- diuretic isnt enough)
(UA can be normal on these)

12

Common Associations of ADPKD
4

1. Multiple, asymptomatic hepatic cysts (30%)

2. Mitral valve prolapse (25%)

3. Cerebral aneurysms (10%)

4. Diverticulosis

13

What should we treat these infections with?

Why are crit and hemoglobin maintained in ESRD?

cipro

Because the cyst itself secretes epo

14

Renal function impairment is variable. What are associated with a poor prognosis?

1. ADPKD1 gene
2. Male sex
3. Black race
4. HTN
5. Early age of clinical presentation
6. Episodes of gross hematuria

15

What do we want to diagnose kidney cysts with?

Renal ultrasound is best!

16

Why is renal ultrasound the best?
5

1. Multiple cysts throughout the parenchyma
2. Renal enlargement
3. Increased cortical thickness
4. Elongation and splaying of renal calyces
5. Bilateral renal involvement

17

When would we use CT scan?

Although can show more cystic involvement than ultrasound, primarily used for differentiation when few cysts are seen on ultrasound.

18

What kind of extrarenal involvement is particularly helpful in the diagnosis of ADPKD?

hepatic cysts

19

Diagnostic criteria for ADPKD1 were established by Ravine et al in 1994 and are as follows:
3

1. At least 2 cysts in 1 kidney or 1 cyst in each kidney in an at-risk patient younger than 30 years.

2. At least 2 cysts in each kidney in an at-risk patient aged 30-59 years.

3. At least 4 cysts in each kidney for an at-risk patient aged 60 years or older

20

What are the screening problems with ADPKD1?
4

1. Asymptomatic carriers.

2. Is expensive.

3. Requires cooperation of other family members.

4. Supplies no anatomic information.

21

What is the treatment for ADPKD1: Aimed at preventing complications and preserving renal function?

1. Treatment of hypertension.
2. Management of infections

22

What is the treatment for ADPKD1: All of the following penetrate cyst walls and attain therapeutic levels?
3

1. Bactrim
2. Chloramphenicol
3. Ciprofloxin

23

Treatment for ADPKD1 with ESRF? 2

1. Dialysis – for ESRF

2. Transplant – for ESRF

24

What do you want to do in pts with large kidneys or history of frequent and persistant UTIs?

Bilateral nephrectomy prior to transplantation

25

Name at four good questions to ask this patient in regards to symptoms of PKD?
6

1. Family history
2. UTI/stones/Pyleo hx
3. HTN
4. Fever
5. bdominal lumbar pain
6. Masses

26

1. Children born with autosomal recessive PKD usually develop kidney failure within how much time?

2. Severity of the disease varies. Babies with the worst cases die _____ or _____ after birth.

Children with an infantile version may have sufficient renal function for normal activities for a few years.

People with the juvenile version may live into their teens and twenties and usually will have liver problems as well.

1. a few years.

2. hours or days

27

Infantile– Autosomal recessive (ARPKD)
Clinical presentation?
5

1. Hx of oligohydramnios, (an unusually small amount or lack of amniotic fluid.)- kidneys arent functioning so they need that fluid
2. Difficult delivery because of enlarged fetal kidneys
3. Respiratory distress may be present
4. Pneumomediastinum and pneumothorax are common
5. Renal function is usually compromised

28

What are the symptoms of ARPKD?
Children with ARPKD experience: 3

The disease usually affects which parts of the body? 3
What does this combination result in? 3

Because kidney function is crucial for early physical development, children with ARPKD are usually what?

1. high blood pressure
2. urinary tract infections
3. frequent urination.

The disease usually affects the
1. liver,
2. spleen
3. pancreas,

resulting in
1. low blood-cell counts
2. varicose veins
3. hemorrhoids.

smaller than average size.

29

How is ARPKD diagnosed:
1. Ultrasound imaging of the fetus or newborn baby reveals cysts in the kidneys but does not distinguish what?

2. What else would we also want to ultrasound?

1. between the cysts of autosomal recessive and autosomal dominant PKD.

2. Because autosomal recessive PKD tends to scar the liver, ultrasound imaging of the liver also aids in diagnosis.

30

How is ARPKD treated?

5

1. Control of hypertension.
2. Antibiotics for UTI’s.
3. Eating increased amounts of nutritious food improves growth in children with autosomal recessive PKD.
4. In some cases, growth hormones are used.
5. In response to kidney failure, autosomal recessive PKD patients must receive dialysis or transplantation.