Tubular and cystic disease Flashcards

1
Q

Acute tubular necrosis is a common cause of

A

intrinsic AKI

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2
Q

What causes acute tubular necrosis

A

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

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3
Q

What urine feature is associated with acute tubular necrosis

A

Oliguria
Muddy brown casts in urine

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4
Q

What are the histological features of acute tubular necrosis

A

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

dilatation of the tubules

necrotic cells obstruct the tubule lumen

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5
Q

What is pyelonephritis

A

Infection of the renal pelvis

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6
Q

How does infection usually spread to the kidneys to cause pyelonephritis

A

Mostly from urinary tract

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7
Q

What is the most common causative pathogen of pyelonephritis

A

E.coli

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8
Q

Pyelonephritis more commonly affects male/female

A

Female

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9
Q

Why does pyelonephritis more commonly affect females

A

Shorter urethra = easier for bacteria to reach the kidneys

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10
Q

Symptoms of pyelonephritis

A

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

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11
Q

Investigation for pyelonephritis

A

Mid-stream urine for cultures
CT

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12
Q

Management of pyelonephritis

A

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

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13
Q

Causes of acute interstitial nephritis

A

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

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14
Q

Most common cause of acute interstitial nephritis

A

Drug toxicity

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15
Q

What are the drugs that can cause acute interstitial nephritis

A

penicillin
rifampicin
NSAIDs
allopurinol
furosemide

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16
Q

Symptoms of acute interstitial nephritis

A

Delayed presentation of:
fever
rash
arthralgia

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17
Q

Investigations for interstitial nephritis

A

Bloods
Urinalysis

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18
Q

What is the typical blood result for interstitial nephritis

A

Eosinophilia

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19
Q

What is the typical urinalysis result for interstitial nephritis

A

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

20
Q

Management of interstitial nephritis

A

Stop the drug

21
Q

What are the genetic defects in autosomal dominant polycystic kidney disease

A

PKD1 on chromosome 16
PKD2 on chromosome 4

22
Q

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

A

PKD1

23
Q

What does PDK1 and PDK2 code for

A

PDK1- polycystin-1
PDK2- polycystic-2

24
Q

Which type of ADPKD has a worse prognosis

A

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

25
Q

Describe the pathophysiology of ADPKD

A

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

26
Q

What are the renal symptoms of ADPKD

A

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

27
Q

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

A

Loin pain + haematuria

28
Q

What are the extra-renal symptoms of ADPKD

A

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

29
Q

What is the most common extra-renal manifestation of ADPKD

A

Hepatic cysts

30
Q

What symptoms may occur due to hepatic cysts

A

SOB
pain
ankle swelling

31
Q

Where do the intracranial aneurysms in ADPKD usually present

A

junction of the anterior communicating artery and anterior cerebral artery

32
Q

Investigations for ADPKD

A

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

33
Q

What is the diagnostic criteria of ADPKD

A

On ultrasound:

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

34
Q

Management of ADPKD

A

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

35
Q

What is the use of tolvaptan

A

Can slow down progression of cysts and renal insufficiency

36
Q

Who is eligible for tolvaptan

A

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

37
Q

What are the complications of ADPKD

A

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

38
Q

What is polycythaemia

A

high concentration of red blood cells in your blood.

39
Q

How does ADPKD lead to polycythaemia

A

due to increase erythropoietin production

40
Q

Symptoms of polycythaemia

A

High haemoglobin level seen
Headache
Breathlessness
Blurry vision
Hearing problems
Splenomegaly

41
Q

Autosomal recessive polycystic kidney disease most commonly affects

A

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

42
Q

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

A

PKHD-1 on chromosome 6

43
Q

Children with ARPKD usually

A

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

44
Q

How does ARPKD cause oligohydramino

A

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

45
Q

What are the consequences of oligohydramino

A

Potter syndrome
Lung hypoplasia (small, underdeveloped lungs)

46
Q

When is ARPKD usually diagnosed

A

prenatal ultrasound or in early infancy with abdominal masses and renal failure

Kidney is always palpable

47
Q

What is the typical presentation of ARPKD renal biopsy

A

Bilateral and symmetrical involvement