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Translational Medical Sciences > Gordens Hypertension Syndrome > Flashcards

Gordens Hypertension Syndrome Flashcards

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USMLE® Step 1 flashcards
1
Q

What is Hypertension?

A

Is the sustained elevation of blood pressure

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2
Q

What is systolic blood pressure?

A

> 140 mm Hg

Systolic pressure> cuff pressure

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3
Q

What is diastolic blood pressure?

A

> 90 mm Hg

Diastolic pressure> cuff pressure.]

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4
Q

What is normal blood flow?

A

No occlusion of blood flow

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5
Q

What is Blood Occlusion?

A

cuff pressure blocks blood flow

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6
Q

What is the normal blood pressure?

A

120/80

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7
Q

What is the blood pressure for pre-hypertension?

A

120-139/80-88

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8
Q

What is the blood pressure for stage 1 hypertension?

A

140-159/90-99

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9
Q

What is the blood pressure for stage 2 hypertension?

A

> 160/100

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10
Q

From what blood pressure does cardiovascular disease risk start to double with each increment of 20/10mmHg?

A

115/75

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11
Q

What are the environmental risk factors which contribute to hypertension?

A

Smoking, bad diet and stress.

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12
Q

What are the genetic risk factors which contribute to hypertension?

A 
Mitochondrial genome 
- signalling 
- energy transduction 
- cell death 
Nuclear genome 
- arterial factors 
- kidney and RAAS - mutations 
- metabolic and local hormonal factors 
-CNS
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13
Q

What is Gordons Syndrome?

A

Rare familial form of hypertension; monogenic; fully penetrant- sporadic cases have been reported.
Alternative names - Pseudohypoalsteronsim type II
Familial H& H

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14
Q

What are the features of Gordons syndrome?

A

Hypertenison
Hyperalkalaemia
Normal renal function
Very sensitive to thiazide diuretics.

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15
Q

How is hypertenision affected in Gordons Syndrome?

A

Low-renin type - salt dependent
- Aldosterone levels low for degree of hyperkalaemia.
Alternate names
FHHt + PHA2

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16
Q

How is hyperkalaemia affected in Gordons Syndrome?

A

High serum potassium,

  • May be severe (>8mmol/l)
  • Metabolic acidosis/ hyperchloraemia(Cl-)
  • Muscle weakness
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17
Q

What is the treatment goal in Gordons Syndrome?

A

Is to reduce the overall cardiovascular risk factors and control BP by the least intrusive means possible.
BP<140/90
In patients with diabetes or renal disease the goal is <130/80

18
Q

What are the complications of hypertension?

A

Target organ diseases

can occur in the heart brain, kidney and eyes.

19
Q

What can causes kidney diseases?

A

High blood pressure

20
Q

How does the kidney control salt levels?

A

The Sodium chloride co-transporter
Sodium potassium chloride cotransporter.
There activity, defines salt concentration in urine,
Affecting the blood volume and arterial pressure,
Could be inhibited by loop or thiazide-type diuretics,

21
Q

How much of the blood pumped to the heart is filtered?

A

20%

22
Q

What does the Sodium chlorine transporter do?

NCC -

A

Transports sodium and chlorine into the blood. which also maintains ion homeostasis in the blood.
NCC - transmembrane protein.

23
Q

What happens at the N-terminus of the NCC?

A

Phosphorylated by SPAK/OSR1, which binds to the RFX1 domain

24
Q

What is TAL?

A

Thick accendini loop

25
Q

Where is the NKCC2? and what does it do?

A

In the Thick ascending loops.

It extracts Sodium potassium and chlorine from the urine to be reabsorbed into the blood.

26
Q

Analysis of NCC phosphorylation in the DCT cells

A

Culture the cells in dishes and treat it with hypotonic low Cl-, The cells are phosphorylated, then in osmotic stress

27
Q

What would the radio active sodium uptake assay?

A

To measure the activity

28
Q

What does Gordons Syndrome entail?

A

Severe hypertension
High serum levels
Increased Na reabsorption
Sensitivity to thiazide diuretics, antagonists of the Na-Cl co-transporter (NCC).

29
Q

What has sequence similarity with the MEKK-like kinases?

A

The kinase domain of WNK

30
Q

How does WNK 1/4 phosphorylation and activates SPAK and OSR1?

A

Activation is mediated by T-loop phosphorylation

31
Q

SPAK and OSR1 are 68% identical, what does this effect?

A

Possess a highly similar kinase catalytic domain

32
Q

what does the CC terminal domain on the SPAK/OSR1 bind too?

A

The RFQV motif on the NKCC2, and the RFTI motif on the NCC, Leucine 502 is important for the binding, and is very conserved.

33
Q

How does WNK1 signalling pathway work?

A
  1. Osmotic stress activates WNK1 by phosphorylation, which phosphorylated SPAK/OSR1, on the T-loop.
  2. Which regulates the ion channels through phosphorylation NKCC1 NKCC2 NCC
34
Q

Ubiquitination System

A
  1. E1 Activates ubiquitin at the C-terminus through adenylation
  2. And transfers it to the E2 (Ubiquitin-conjugating enzyme) by transthiolation
  3. E2 and E3 come together forming a complex, Called ubiquitin ligase.
  4. E3 binds to degron in the protein, E3 ligates the c-terminus carboxyl group of the ubiquitin to a lysine group on the protein more are added, which can then be recognised by the proteasome.
35
Q

What is the Proteasome?

A

Compartmentalised protease, which active sites are sequestered (Inaccessible without admission into its proteolytic chamber)

36
Q

How does the proteasome work?

A
  1. Ubiquitin receptor holds the protein in place.
  2. Ubiquitin hydrolase (cleaves Ubiquitin from substrate proteins.
  3. AAA proteins form a hexametric protein unfoldase, it requires ATP, protein is unfolded, Nucleophile attacks bonds in aa.
37
Q

What mutations cause Gordons Syndrome?

A

KLHL3 and CUL3

38
Q

A patient has high blood pressure and high potassium levels what disease could they have?

A

Gordons Syndrome

39
Q

A patient with low blood pressure and low serum levels, are likely to have what disease?

A

Gitelman

40
Q

What affect does thiazide diuretic have on the body?

A

Increase renal excretion of Sodium, Potassium and Hydrogen ions (causing metabolic alkalosis)
Decreases renal excretion of calcium

41
Q

What are the treatments of Gordons trying to do?

A

Decrease the phosphorylation of NCC

Translational Medical Sciences (10 decks)

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