Tubular and cystic disease

Question 1

Acute tubular necrosis is a common cause of

Answer 1

intrinsic AKI

Question 2

What causes acute tubular necrosis

Answer 2

Sepsis
Severe dehydration -> shock
Drugs - gentamicin
Contrast exposure
Rhabdomyolysis

Question 3

What urine feature is associated with acute tubular necrosis

Answer 3

Oliguria
Muddy brown casts in urine

Question 4

What are the histological features of acute tubular necrosis

Answer 4

loss of nuclei and detachment of tubular cells from the basement membrane

dilatation of the tubules

necrotic cells obstruct the tubule lumen

Question 5

What is pyelonephritis

Answer 5

Infection of the renal pelvis

Question 6

How does infection usually spread to the kidneys to cause pyelonephritis

Answer 6

Mostly from urinary tract

Question 7

What is the most common causative pathogen of pyelonephritis

Answer 7

E.coli

Question 8

Pyelonephritis more commonly affects male/female

Answer 8

Female

Question 9

Why does pyelonephritis more commonly affect females

Answer 9

Shorter urethra = easier for bacteria to reach the kidneys

Question 10

Symptoms of pyelonephritis

Answer 10

High fever
loin pain
nausea/vomiting
Dysuria and urinary frequency
Pain on palpation over the renal angle

Question 11

Investigation for pyelonephritis

Answer 11

Mid-stream urine for cultures
CT

Question 12

Management of pyelonephritis

Answer 12

Cefalexin for 7-10 days
+/- Ciprofloxacin
Review after culture results

Question 13

Causes of acute interstitial nephritis

Answer 13

Drug toxicity
SLE
Sjogren’s
Sarcoidosis
Infection - staphylococci, Hanta virus

Question 14

Most common cause of acute interstitial nephritis

Answer 14

Drug toxicity

Question 15

What are the drugs that can cause acute interstitial nephritis

Answer 15

penicillin
rifampicin
NSAIDs
allopurinol
furosemide

Question 16

Symptoms of acute interstitial nephritis

Answer 16

Delayed presentation of:
fever
rash
arthralgia

Question 17

Investigations for interstitial nephritis

Answer 17

Bloods
Urinalysis

Question 18

What is the typical blood result for interstitial nephritis

Answer 18

Eosinophilia

Question 19

What is the typical urinalysis result for interstitial nephritis

Answer 19

Sterile pyuria (lots of WCC in urine)
White cell casts

Question 20

Management of interstitial nephritis

Answer 20

Stop the drug

Question 21

What are the genetic defects in autosomal dominant polycystic kidney disease

Answer 21

PKD1 on chromosome 16
PKD2 on chromosome 4

Question 22

Which gene defect in autosomal dominant polycystic kidney disease is more common

Answer 22

PKD1

Question 23

What does PDK1 and PDK2 code for

Answer 23

PDK1- polycystin-1
PDK2- polycystic-2

Question 24

Which type of ADPKD has a worse prognosis

Answer 24

ADPKD type 1 (mutation at PDK1) - presents with renal failure earlier

Question 25

Describe the pathophysiology of ADPKD

Answer 25

abnormal proliferation of tubular cells and changes in extracellular matrix causing large cysts to form over time

Question 26

What are the renal symptoms of ADPKD

Answer 26

Early hypertension Chronic pain Haematuria - If cysts rupture, becomes infected Fever Renal failure

Question 27

What is a classic presentation of rupture of a cyst in ADPKD

Answer 27

Loin pain + haematuria

Question 28

What are the extra-renal symptoms of ADPKD

Answer 28

Hepatic cysts Intra-cranial aneurysms Mitral/aortic valve prolapse Aortic regurgitation Diverticular disease Hernias

Question 29

What is the most common extra-renal manifestation of ADPKD

Answer 29

Hepatic cysts

Question 30

What symptoms may occur due to hepatic cysts

Answer 30

SOB pain ankle swelling

Question 31

Where do the intracranial aneurysms in ADPKD usually present

Answer 31

junction of the anterior communicating artery and anterior cerebral artery

Question 32

Investigations for ADPKD

Answer 32

Ab Ultrasound - screen those with family history MRI if ultrasound is unclear

Question 33

What is the diagnostic criteria of ADPKD

Answer 33

On ultrasound: < 30 years - 2 cysts unilateral or bilateral 30-59 years - 2 cysts in both > 60 years - 4 cysts in both

Question 34

Management of ADPKD

Answer 34

Control of hypertension Some may be eligible for tolvaptan genetic counselling and screening other members

Question 35

What is the use of tolvaptan

Answer 35

Can slow down progression of cysts and renal insufficiency

Question 36

Who is eligible for tolvaptan

Answer 36

have chronic kidney disease stage 2 or 3 evidence of rapidly progressing disease

Question 37

What are the complications of ADPKD

Answer 37

Cyst rupture Cyst infection CKD Subarachnoid haemorrhage due to aneurysm Intracerebral haemorrhage due to hypertension Polycythaemia

Question 38

What is polycythaemia

Answer 38

high concentration of red blood cells in your blood.

Question 39

How does ADPKD lead to polycythaemia

Answer 39

due to increase erythropoietin production

Question 40

Symptoms of polycythaemia

Answer 40

High haemoglobin level seen Headache Breathlessness Blurry vision Hearing problems Splenomegaly

Question 41

Autosomal recessive polycystic kidney disease most commonly affects

Answer 41

young children, can be perinatal/neonatal/infantile/juvenile

Question 42

Where is the mutation at that causes autosomal recessive polycystic kidney disease

Answer 42

PKHD-1 on chromosome 6

Question 43

Children with ARPKD usually

Answer 43

Develop into hepatic fibrosis Have Oligohydramino consequences Slow decline in eGFR 1/3 reach ESRF

Question 44

How does ARPKD cause oligohydramino

Answer 44

kidney function in utero is decreased this results is lower than normal production of amniotic fluid

Question 45

What are the consequences of oligohydramino

Answer 45

Potter syndrome Lung hypoplasia (small, underdeveloped lungs)

Question 46

When is ARPKD usually diagnosed

Answer 46

prenatal ultrasound or in early infancy with abdominal masses and renal failure Kidney is always palpable

Question 47

What is the typical presentation of ARPKD renal biopsy

Answer 47

Bilateral and symmetrical involvement