Urinary Tract Congenital Anomalies, Cystic Disease, Urolithiasis and Obstructive Diseases

Question 1

What percentage of renal failure in children is caused by congenital kidney malformations?

Answer 1

20%

Question 2

What percentage of people are born with potentially significant malformation of the kidney/urinary tract?

Answer 2

10%

Question 3

What percentage of chronic renal failure is due to ADPKD?

Answer 3

10%

Question 4

What are the types of malformation of the kidneys?

Answer 4

Agenesis of the kidney

Hypoplasia

Ectopic kidneys Horseshoe kidneys

Question 5

What is kidney hypoplasia?

Answer 5

Formed kidneys but very small

Question 6

What is ectopic kidneys?

Answer 6

Kidneys that are misplaced

Question 7

What is horseshoe kidney?

Answer 7

malformed kidneys that join together and never separate forming a horseshoe shape

Question 8

What causes agenesis of the kidney?

Answer 8

Major disruption of metanephric development at an early stage

Question 9

What is potter’s syndrome?

Answer 9

Bilateral agenesis of kidneys.

Question 10

What happens to babies without kidneys?

Answer 10

They develop without much amniotic fluid around it. This condition is called oligohydramnios.

They also get hypoplastic lungs if there is not enough amniotic fluid to breath in for them to open

Question 11

What does potter’s facies refer to?

Answer 11

A particular appearance associated with low exposure to amniotic fluid.

Question 12

What does potter’s facies look like?

Answer 12

Large low set ears

Wide set eyes

Flattened nose

Receding chin

Prominent skin fold below the eyes

Question 13

What does unilateral agenesis do to foetus?

Answer 13

Usually asymptomatic

Question 14

What happens to the present kidney in unilateral agenesis?

Answer 14

It undergoes compensatory hypertrophy.

Question 15

What happens to the ureter of the abscent kidney?

Answer 15

It disappears completely

Question 16

What causes ectopic kidneys?

Answer 16

Kidneys usually ascend during development to their position and end up staying around the pelvis.

Question 17

What are the cystic diseases of the kidney?

Answer 17

Cystic renal disease

Polycystic kidney disease (autosomal dominant PKD and autosomal recessive PKD)

Question 18

What are simple renal cysts?

Answer 18

Very common in normal kidneys especially people older than 50 years old.

Usually they cause no symptoms or signs

Occasionally they can rupture, cause haematuria, pain, abdominal mass, infection, and hypertension.

Question 19

How are cystic renal lesions diagnosed as tumours?

Answer 19

Multiple septa

Thickened cyst

Solid areas within or around the cyst

Question 20

What is cystic renal dysplasia?

Answer 20

Malformation of the kidney results inirregular cysts of varying sizes in the kidneys and is a common cause of an abdominal mass in infants

Question 21

What kind of cells can be found in cystic renal dysplasia?

Answer 21

Persistence of abnormal structures like cartilage and mesenchyme

Question 22

How do people get polycystic kidney disease?

Answer 22

It’s a congenital defect a dominant form (1 in 500 live births 10% of end stage renal failure have this disease) and recessive form.

Question 23

What happens to cysts over time in ADPCK?

Answer 23

They grow continuously

Question 24

At what age do patients present with ADPCK?

Answer 24

Patients start experiencing symptoms between 30 and 50 years old.

Question 25

What are the symptoms of ADPCK?

Answer 25

Flank pain due to abdominal mass or renal stones Pain from haemorrhage into a cyst Haematuria Hypertension

Question 26

What are other associated congenital abnormalities associated with APCKD?

Answer 26

Cysts in liver, pancreas, spleen, and lungs Intracranial berry aneurysms Mitral valve prolapse

Question 27

How are cysts different in Autosomal recessive polycystic kidney disease?

Answer 27

Smooth kidneys that have smaller cysts. They also get cysts on the liver causing fibrosis

Question 28

Autosomal recessive PKD:

Answer 28

rare and presents early in life

Question 29

What is obstructive uropathy?

Answer 29

Obstruction of urine to the urinary tract

Question 30

What are the modes of damage caused by obstructive uropathy?

Answer 30

Acute (stone) / chronic (prostate enlargement) Partial / complete Unilateral / bilateral Any level from the urethra to the renal pelvis Intrinsic / Extrinsic

Question 31

What is a common cause of extrinsic compression of the ureter?

Answer 31

Growth of lymph nodes caused by lymphoma

Question 32

What are some common causes of obstructive uropathy?

Answer 32

* Congenital anomalies such as posterior urethral valves * Urinary calculi * Benign prostatic hypertrophy * Tumours of prostate bladder lymph nodes and inflammation * Sloughed renal papillae (renal papillae can just die and pop off and block shit) / blood clots * Normal pregnancy Uterine prolapse and cystocoele Functional causes such as defects in muscle wall *: means common

Question 33

What happens to kidneys when obstruction takes place?

Answer 33

They kidneys get larger Renal pelvis and calyces get larger Ureters get big Hydronephrosis Bladder can get very thick

Question 34

What does hydronephrosis refers to?

Answer 34

Water inside kidney Distension of the walls and in turn can lead to atrophy of the kidney by squishing the renal parenchyma

Question 35

What are the symptoms of obstructive uropathy?

Answer 35

Pain from dilation of the renal capsule/ureter/bladder UTIs Urinary calculi (lead to precipitation of stuff in urine) Renal failure (can't concnetrate urine because kidney is failing) Partial bilateral obstruction causes an inability to concentrate urine Complete bilateral obstruction can be fatal

Question 36

What age groups get kidney stones most commonly?

Answer 36

20 - 30

Question 37

Who gets urolithiasis most commonly?

Answer 37

Men in their 20s with a family history (ME YAY....)

Question 38

What are the types of kidney stones?

Answer 38

Calcium oxalate Uric acid Struvite (associated with infections) Calcium phosphate Others

Question 39

What causes people to develop kidney stones?

Answer 39

Stones are solutes that occur in amounts too high to stay dissolved in urine so solutes precipitate and aggregate to form concretions (stones)

Question 40

What are the risk factors for calcium oxalate stones?

Answer 40

Dehydration Hypercalciuria (with or without hypercacaemia) Hyperoxaluric (odd diet into spinach, rhubarb, oxalate foods in general i.e superfoods) Hyperuricosuric Low urine volume

Question 41

What causes uric acid stones?

Answer 41

Dehydration Acidic urine Hypericaemia Chronic diarrhoea Diabetes Increased urinary excretion of uric acid (So anything that leads to rapid cell death and birth can cause it due to increased metabolism)

Question 42

What are struvite stones?

Answer 42

UTIs such as proteus and klebsiella have properties that can split urea in the urine creating ammonia which alters pH of urine which causes magnesium ammonium phosphate precipitation and causes formation of a cast of renal pelvis as well as caluses which come from tissue of renal pelvis. These stones are often called staghorn calculi due to filling of calyceal space in the kidney.

Question 43

What are the complications of urolithiasis?

Answer 43

Silent often (they can sit there for a long time without symptoms) If they pass into ureters they are very painful and cause bleeding Ulceration and bleeding Infection

Question 44

What is the pain associated with renal calculi known as?

Answer 44

Renal colic

Question 45

Where is pain associated with in renal colic?

Answer 45

Excruciating intermittent pain which radiates from the flank to the groin

Question 46

What other symptoms do people with renal colic get?

Answer 46

Urinary urgency Restlessness Haematuria Sweating Nausea Vomiting

Question 47

Summary

Answer 47

Structural or functional hindrance of urine flow which can lead to renal dysfunction Can occur at any point in the urinary tract or can affect one or both kidneys Comon cause is stones Common presentation of stones is renal colic Complications of renal colic include infection and renal failure