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Path - Chem Path > Acid - Base, LFTs, Porphyrias > Flashcards

Acid - Base, LFTs, Porphyrias Flashcards

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1
Q

Normal Ranges - pH

A

7.35-7.45

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2
Q

Normal Ranges - Co2

A

4.7-6 kPa

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3
Q

Normal Ranges - Bicarbonate

A

22-30 mmol/l

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4
Q

Normal Ranges - O2

A

10 -13 kPa

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5
Q

Steps to solve simple problems - Look at the case in this step by step way to determine the cause

A

1) pH - acidic or alkali?
2) CO2 - does it fit with the pH?
3) Bicarbonate - does it fit with the pH?
4) Compensation - is there any? Partial or Complete?

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6
Q

Metabolic Acidosis

  • pH?
  • Bicarbonate?
  • CO2?
  • Causes?
A
  • pH - low
  • Bicarbonate - low
  • CO2 - normal or low (if compensated)
  • Causes - Lactate build up, DKA, Renal tubular
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7
Q

Metabolic Alkalosis

  • pH?
  • Bicarbonate?
  • CO2?
  • Causes?
A
  • pH - High
  • Bicarbonate - High
  • CO2 -normal/ High (If compensated)
  • Causes - Pyloric Stenosis, Hypokalamia
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8
Q

Respiratory Acidosis

  • pH?
  • Bicarbonate?
  • CO2?
  • Causes?
A
  • pH - Low
  • Bicarbonate - Normal/High (if compensated)
  • CO2 - High
  • Causes - Lung Injury - pneumonia, COPD, Decreased Ventilation
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9
Q

Respiratory Alkalosis

  • pH?
  • Bicarbonate?
  • CO2?
  • Causes?
A
  • pH - High
  • Bicarbonate - Normal/ Low (if compensated)
  • CO2 - Low
  • Causes - Mechanical Ventilation, Anxiety/Panic attack
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10
Q

what is compensation?

A

Return of pH towards normal at the expense of other values

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11
Q

What is the Anion Gap?

A

(Na + K) - (Cl + HCO3)

Difference between total concentration of principal cations and principal anions = Concentration of unmeasured anions in the plasma

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12
Q

What is the biggest contributor to the Anion Gap?

A

Almost entirely contributed by Albumin (beware in hypoalbuminaemia)

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13
Q

Normal Range for the Anion Gap?

A

14-18 mmol/l

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14
Q

Mnemonic for elevated anion gap metabolic acidosis?

KULT

A 
K etoacidosis (DKA, alcoholic, starvation)
U raemia (renal failure)
L actic Acidosis
T oxins (ethylene glycol, methanol, paraldehyde, salicylate)
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15
Q

Osmolar Gap- what is a normal osmolar gap?

A

Osmolality (measured) - Osmolarity (calculated)

Normal osmolar gap = less than 10

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16
Q

What does an elevated osmolar gap suggest?

What does it differentiate?

A

An elevated osmolar gap provides indirect evidence for the presence of an abnormal solute.
it is increased by extra solutes in the plasma ( e.g. ethylene glycol, ethanol, methanol, mannitol)

Helpful in differentiating the cause of an elevated anion in metabolic acidosis

17
Q

Liver Function Tests - Markers of Liver Cell Damage

A 
ALT
AST
ALK phos
GGT
Bilirubin
18
Q

Liver Function Tests - Markers of Synthetic Function

A

Clotting (INR)
Albumin
Glucose

19
Q

Aminotransferases - AST/ALT

  • normal range?
  • causes of high levels?
  • ratio in Alcoholic liver disease?
  • ratio in Viral liver disease?
A
  • less than 40
  • raised when hepatocytes die
  • Alcoholic liver disease - 2:1
  • Viral liver disease 1:1
20
Q

Alkaline Phosphatase (ALP)

  • normal range?
  • raised when?
A
  • 30-150 iu/L

- Raised with cholestasis (either intrahepatic or extra hepatic) and bone disease, High in pregnancy

21
Q

Gamma GT (GGT)

  • Normal range?
  • When is it Elevated?
A
  • 30-150 iu/L
  • Elevated in chronic alcohol use
  • Also bile duct disease and metastases. Used to confirm hepatic source of increased ALP
22
Q

What is Porphyrias?

A

7 Disorders caused by deficiency in enzymes, involved in haem biosynthesis, leading to build up of toxic haem precursors

23
Q

Acute Intermittent Porphyria (AIP)

  • inheritance?
  • deficiency?
A

Autosomal dominant inheritance

HMB (Hydroxymethybilane) synthase deficiency

24
Q

Acute Intermittent Porphyria (AIP)

- Symptoms?

A

Neuro-visceral only
- abdo pain, seizures, psych disturbances, nausea and vomiting, tachycardia, hypertension, sensory loss, muscle weakness, constipation, urinary incontinence,

NO cutaneous manifestations due to absence of porphyrinogens

25
Q

Acute Intermittent Porphyria (AIP)

- Diagnosis?

A

ALA + PBG in urine (Port wine urine)

26
Q

Acute Intermittent Porphyria (AIP)

- Precipitating Factors?

A

ALA synthase inducers (steroids, ethanol, barbiturates)
Stress (infection, surgery)
Reduced caloric intake and endocrine factors (eg premenstrual)

27
Q

Acute Intermittent Porphyria (AIP)

- Treatment?

A

Avoid precipitating factors, analgesia, IV carbohydrate/ haem arginate

28
Q

Acute porphyrias with skin lesions

  • 2 types?
  • inheritance?
  • Symptoms and clinical signs?
A

-Hereditary coproporphyria (HCP) and Variegate porphyria (VP)
(these are 2 subtypes of porphyrias, have googled them and they both have the same sort of symptoms and signs, both have no treatment, although liver transplantation has been suggested)

  • Autosomal dominant
  • Neurovisceral + skin lesions
  • Raised porhyrins in faeces or urine
29
Q

Non-Acute porphyrias

A

Skin lesions ONLY

  • Congenital Erythopoietic porphyria (CEP)
  • Erythropoietic protoporphyria (EPP)
    • -Photosensitivity, burning, itching, oedema following sun exposure
  • Porphyria Cutanea Tarda (PCT)
30
Q

Porphyria Cutanea Tarda

  • inheritance?
  • deficiency?
  • symptoms?
  • diagnosis?
  • treatment?
A
  • inherited/ acquired
  • Uroporphyrinogen decarboxylase deficiency
  • Cutaneous symptoms - Vesicles (crusitng, pigmented, superficial scarring) on sun exposed sites
  • Diagnosis - increased urinary uroporphyrins + coproporphyrins + increased ferritin
  • Treatment - avoid precipitants (alcohol, hepatic compromise), phlebotomy

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