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Medical viva1 Flashcards

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1
Q

What is amyloidosis?

A

Extracellular deposition of fibrils

Amyloidosis is a condition characterized by the abnormal buildup of amyloid proteins in tissues and organs.

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2
Q

What are the two types of amyloidosis?

A
  • Primary - accumulation of immunoglobulin light chains
  • Secondary - associated with conditions like multiple myeloma and rheumatoid arthritis

Primary amyloidosis is often related to plasma cell disorders, while secondary amyloidosis occurs due to chronic inflammatory conditions.

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3
Q

What are common airway symptoms of amyloidosis?

A
  • Macroglossia
  • Laryngo-tracheo-bronchial stenosis
  • Stridor
  • Increased aspiration risk

These airway complications can significantly affect breathing and swallowing.

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4
Q

What cardiac issues are associated with amyloidosis?

A
  • Restrictive cardiomyopathy
  • Diastolic dysfunction
  • Arrhythmia
  • Conduction defects

Cardiac involvement is a serious aspect of amyloidosis and can lead to heart failure.

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5
Q

What respiratory complications can occur in amyloidosis?

A
  • Interstitial lung disease
  • Restrictive lung disease
  • Pulmonary hypertension

These respiratory issues can lead to decreased lung function and difficulty in breathing.

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6
Q

What gastrointestinal symptoms are associated with amyloidosis?

A
  • Dysphagia
  • Delayed gastric emptying
  • Autonomic neuropathy
  • Malnutrition

These symptoms can significantly impact nutrition and quality of life.

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7
Q

What renal complications can arise in amyloidosis?

A
  • Nephrotic syndrome
  • Renal failure

Renal involvement is critical and often leads to significant morbidity.

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8
Q

What neurological symptoms can be present in amyloidosis?

A
  • Mixed sensory neuropathy
  • Motor neuropathy
  • Autonomic neuropathy

Document deficits before regional/neuraxial.

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9
Q

What hematological issues are associated with amyloidosis?

A
  • Factor X deficiency
  • Platelet dysfunction
  • Anaemia of chronic disease

These hematological complications can increase the risk of bleeding.

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10
Q

What are the main treatment options for amyloidosis?

A
  • Steroids
  • Chemotherapy

Treatment aims to reduce amyloid production and manage symptoms.

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11
Q

Which specialists are typically involved in the management of amyloidosis?

A
  • Cardiologist
  • Immunologist
  • Haematologist

A multidisciplinary approach is essential for optimal care.

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12
Q

What is hereditary angioedema?

A

A genetic condition causing episodes of severe swelling.

It is often triggered by various factors such as stress and surgery.

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13
Q

What is Danazole?

A

A synthetic steroid used for prophylaxis in hereditary angioedema patients before and after elective surgery.

It should be taken 5 days before and 5 days after surgery, with a double dose if already on it.

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14
Q

What is Berinert?

A

C1 esterase inhibitor concentrate used for prophylaxis during dental, head and neck surgery, intubation, or airway instrumentation.

It helps prevent angioedema attacks in high-risk situations.

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15
Q

What is Icatibant?

A

A bradykinin antagonist used to treat acute attacks of hereditary angioedema.

It works by blocking the effects of bradykinin, which causes swelling.

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16
Q

What should be administered if Berinert or Icatibant is not available?

A

FFP (Fresh Frozen Plasma) 2-4 units.

FFP may worsen an attack as it contains C4, a substrate for bradykinin release.

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17
Q

What is the response of hereditary angioedema to adrenaline, steroids, and antihistamines?

A

Limited response.

These treatments are generally ineffective in managing symptoms.

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18
Q

What are common triggers for hereditary angioedema?

A

Intubation, infection, stress, menstruation, oral/dental surgery, trauma, and some medications like OCP and ACE-inhibitors.

These factors can provoke episodes of swelling.

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19
Q

What are the clinical features of hereditary angioedema?

A

Angioedema without pruritis or urticaria, abdominal pain (+/- nausea/vomiting), and laryngeal oedema.

Symptoms may be delayed by 60 minutes to 36 hours after a trigger.

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20
Q

Who should you consult for hereditary angioedema patients prior to surgery?

A

An immunologist.

Their expertise is crucial in managing prophylaxis and acute treatment.

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21
Q

How should Berinert be administered during induction?

A

25 units/kg over 1-2 minutes within 1 hour of induction.

Proper administration timing is critical for effective prophylaxis.

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22
Q

What is a recommended approach to avoid airway complications in hereditary angioedema?

A

Use regional/neuraxial anesthesia.

This technique minimizes the risk of airway obstruction.

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23
Q

What equipment should be available for managing difficult airways in hereditary angioedema?

A

Difficult airway trolley and FONA (Front of Neck Access).

These tools are essential for addressing potential airway emergencies.

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24
Q

What is thalessemia ? What are the consequences?

A

Decreased synthesis of alpha or beta chains of hemoglobin. This leads to chronic hemolytic anemia.

Jaundice, Cholestasis, Splenomegaly
Transfusion dependent
Alloimmunisation

Iron overload - cardiomyopathy, liver failure - cirrhosis, pancreatic fibrosis - diabetes,

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25
What are the compensatory mechanisms for chronic hemolytic anemia?
Increased cardiac output, increased 2,3-DPG, increased plasma volume ## Footnote These compensations help manage anemia.
26
What are common complications of chronic hemolytic anemia?
Cholilithiasis, splenomegaly ## Footnote These are common sequelae due to increased hemolysis.
27
What are the sequelae of multiple transfusions or iron overload?
Cardiomyopathy, dysrhythmias, hepatic fibrosis, diabetes, alloimmunization ## Footnote These complications arise from excess iron and immune response.
28
What characterizes Alpha thalassemia major?
Incompatible with life ## Footnote This condition is severe and typically fatal.
29
What are the complications associated with Beta thalassemia major?
Potential difficult airway - maxillary overgrowth, hemochromatosis, jaundice, hemolytic anemia ## Footnote Complications arise from bone marrow stimulation and iron deposition.
30
What is a potential airway issue in Beta thalassemia major?
Difficult airway due to maxillary overgrowth ## Footnote This occurs as a result of bone marrow stimulation.
31
What is hemochromatosis in the context of Beta thalassemia?
Deposition of hemosiderin into cardiac muscle leading to dilated cardiomyopathy, heart failure, conduction delays ## Footnote Iron overload can severely affect heart function.
32
What are the characteristics of Alpha/Beta thalassemia minor?
Mild hemolytic anemia & iron deficiency ## Footnote This form is less severe compared to major types.
33
What is the typical goal hemoglobin level for thalessemia to ensure a patient is not anemic for surgery?
Hemoglobin > 100 ## Footnote This goal helps ensure surgical safety.
34
What is the genetic inheritance pattern of haemochromatosis?
Autosomal recessive
35
What is a complication of haemochromatosis that affects the liver?
Cirrhosis
36
Which complication of haemochromatosis can lead to diabetes?
Pancreatic fibrosis
37
Name a joint-related complication of haemochromatosis.
Arthritis
38
What cardiac complications are associated with haemochromatosis?
Dilated cardiomyopathy, Arrhythmia, Conduction disturbances
39
What is the treatment for haemochromatosis?
Phlebotomy Ferritin <50 and Transferrin <30 Iron chelators - desferroxamine
40
What are the ferritin and transferrin levels that indicate the need for phlebotomy in haemochromatosis?
Ferritin <50 and Transferrin <30
41
What type of medication is desferroxamine?
Iron chelator
42
What is the primary condition associated with Sickle Cell?
Haemolytic anaemia ## Footnote Transfusion dependent with complications such as iron overload, antibodies, and viral hepatitis.
43
What are the complications of chronic pain in Sickle Cell patients?
Opioid tolerance ## Footnote Chronic pain management often leads to increased tolerance to opioids.
44
What is a vaso-occlusive crisis in Sickle Cell disease?
Infarction anywhere in the body, such as MI, Stroke, lung, renal, spleen, or penis (priapism) ## Footnote This crisis can lead to severe pain and organ damage.
45
Which cardiovascular complication is associated with Sickle Cell?
Left Ventricular Hypertrophy (LVH) and Diastolic heart failure ## Footnote These complications arise due to chronic hypoxia and increased workload on the heart.
46
What respiratory complications can occur in Sickle Cell patients?
Acute chest syndrome, lung infarct, pulmonary fibrosis, pulmonary hypertension, right ventricular failure ## Footnote These respiratory issues are critical and can be life-threatening.
47
What renal complications are associated with Sickle Cell disease?
Chronic Kidney Disease (CKD) and renal infarct ## Footnote Renal complications can lead to significant morbidity.
48
What splenic complications can occur in Sickle Cell patients?
Sequestration and infarct leading to splenectomy ## Footnote Splenic complications can increase the risk of infections.
49
What is the purpose of erythrocyte exchange in Sickle Cell management?
To reduce and maintain the haemoglobin S <30%, Hb 100, HCT 30% ## Footnote This helps in managing symptoms and preventing complications.
50
What medication is used to increase HbF in Sickle Cell patients?
Hydroxyurea ## Footnote Hydroxyurea is effective in increasing fetal hemoglobin levels.
51
What are the key considerations for intra-operative management in Sickle Cell patients?
Avoid pain, hypotension, hypovolemia, hypoxia, acidosis, hypothermia, venous stasis ## Footnote These factors are crucial to prevent complications during surgery.
52
Is Sickle Cell surgery typically suitable for day stay?
No ## Footnote Post-operative care usually requires more extensive monitoring.
53
What is the genetic inheritance pattern of Achondroplasia?
Autosomal dominant
54
What percentage of Achondroplasia cases are due to de novo mutations?
80%
55
List three physical characteristics of individuals with Achondroplasia.
* Large head to body ratio * Short limbs * Short neck
56
What airway considerations should be taken for patients with Achondroplasia?
* Limited neck extension * Large tongue * Atlantoaxial instability
57
What imaging should be considered for patients with Achondroplasia?
Consider imaging for airway and C spine difficulties
58
What is the recommendation for managing difficult airways in Achondroplasia patients?
* Prepare difficult airway trolley * Consider pediatric equipment
59
What condition has an increased incidence in Achondroplasia patients?
Obstructive Sleep Apnea (OSA) / Central Sleep Apnea (CSA)
60
What is advised to minimize opioid use in Achondroplasia patients?
* Use adjuncts such as paracetamol, NSAIDs, ketamine, alpha-2 agonists, magnesium, local anesthetics or regional anesthesia
61
Which type of anesthesia is preferred over general anesthesia in Achondroplasia patients?
Regional/neuraxial anesthesia
62
What should be considered for extubation in Achondroplasia patients?
Extubation onto Non-Invasive Ventilation (NIV) and monitoring in High Dependency Unit (HDU) post-operative bed
63
What spine abnormalities are associated with Achondroplasia?
* Kyphosis * Scoliosis * Spinal stenosis
64
What is the recommendation regarding local anesthetic (LA) spread in Achondroplasia patients?
Unpredictable spread of local anesthetic
65
What is the preferred method of anesthesia for spinal procedures in Achondroplasia patients?
Epidural > spinal with lower doses and slow titration
66
What is a key monitoring consideration for muscle tone in Achondroplasia patients?
Neuromuscular transmission (NMT) monitoring
67
What is the target Train-of-Four (TOF) ratio prior to extubation in Achondroplasia patients?
TOF > 0.9
68
What condition is characterized by increased growth hormone?
Acromegaly ## Footnote Acromegaly results from pituitary adenoma leading to excess growth hormone secretion.
69
What is the treatment for acromegaly?
Suppress GH with octreotide ## Footnote Octreotide is a somatostatin analogue used to manage acromegaly.
70
What are some symptoms of pituitary adenoma mass effect?
* Headache * Increased intracranial pressure (ICP) * Visual disturbance ## Footnote These symptoms occur due to the pressure exerted by the tumor on surrounding structures.
71
What is a common airway challenge associated with acromegaly?
Difficult airway due to macroglossia and enlarged jaw ## Footnote Macroglossia refers to an enlarged tongue, which can complicate intubation.
72
What airway management technique may be considered for patients with acromegaly?
AFOI and smaller ETT ## Footnote AFOI stands for awake fiberoptic intubation, which is often safer in difficult airway situations.
73
What screening tool is used to assess obstructive sleep apnea (OSA)?
STOPBANG ## Footnote STOPBANG is a questionnaire used to identify patients at risk for OSA.
74
What should be checked in patients with obesity hypoventilation syndrome (OHS)?
ABG ## Footnote Arterial blood gas (ABG) analysis helps assess respiratory function in OHS.
75
What are the cardiovascular complications associated with hypertension in acromegaly?
* Left ventricular hypertrophy (LVH) * Diastolic heart failure * Arrhythmia * Cardiomyopathy ## Footnote These complications arise from prolonged high blood pressure and increased cardiac workload.
76
What tests should be performed to evaluate cardiac function in patients with acromegaly?
* ECG * Transthoracic echocardiogram (TTE) ## Footnote These tests help identify structural and functional cardiac issues.
77
What is a contraindication for using a radial arterial line in acromegaly patients?
Poor collateral supply ## Footnote Allen's Test can assess collateral circulation before placing a radial arterial line.
78
What endocrine complications should be monitored in patients with acromegaly?
* Diabetes * Thyroid disease * Adrenal insufficiency ## Footnote Endocrine evaluations are crucial due to the potential for multiple hormonal imbalances.
79
What should be adjusted in diabetic patients prior to surgery?
Withhold oral agents, adjust insulin, minimize fasting ## Footnote Proper management of blood glucose levels is essential for surgical safety.
80
What electrolyte imbalances are associated with adrenal insufficiency?
* Decreased sodium (Na) * Decreased potassium (K) ## Footnote These imbalances require correction before surgery.
81
What positioning risk is increased in patients with acromegaly?
Neuropraxia/pressure sores due to enlarged bony prominences ## Footnote Careful positioning is important to prevent nerve injury and skin breakdown.
82
What common peripheral nerve condition may occur in patients with acromegaly?
Carpal tunnel syndrome ## Footnote Carpal tunnel syndrome may result from compression of the median nerve due to enlarged soft tissues.
83
What is Acute Porphyria?
Inherited enzymatic defects of heme synthesis leading to a build-up of precursors that cause attacks.
84
What should be checked before an acute porphyria attack?
Preexisting neurological and renal impairment, triggers, and previous anaesthetics.
85
List non-pharmacological triggers for Acute Porphyria.
* Fasting * Dehydration * Stress * Pain * Infection
86
List pharmacological triggers for Acute Porphyria.
* Barbiturates * Phenytoin * Diazepam * Ropivacaine * Corticosteroids * Ketamine
87
What is the prevention strategy for fasting in Acute Porphyria?
Minimize fasting: solids 6h, clear carbohydrate fluid until send.
88
What to do if a patient is not absorbing carbohydrates?
Carbohydrate load: dextrose 10% 100-200ml/hr, 300g/day.
89
Which premedications are recommended for Acute Porphyria?
Midazolam or clonidine NOT PRECEDEX.
90
What induction agents are safe for Acute Porphyria?
Propofol induction is safe; avoid barbiturates or ketamine.
91
What analgesics are safe for patients with Acute Porphyria?
All opioids, neuromuscular blockers, volatiles, N2O, paracetamol, and NSAIDs.
92
Which antibiotics are safe to use in Acute Porphyria?
* Cephazolin * Gentamicin * Vancomycin * Metronidazole
93
What antiemetics are safe for post-operative nausea and vomiting in Acute Porphyria?
Ondansetron, Droperidol, Cyclizine NOT DEXAMETHASONE.
94
What local anesthetics are safe for use in Acute Porphyria?
Lignocaine, Ropivacaine, or Bupivacaine for LA infiltration/regional/neuraxial.
95
Which reversal agents are safe for Acute Porphyria?
Neo/glyco + sugammadex are safe.
96
What uterotonics should be avoided in Acute Porphyria?
Oxytocin and Carboprost are safe; NOT ERGOMETRINE.
97
What are some neurological symptoms during an Acute Porphyria attack?
* Seizures * Hallucinations * Agitation * Coma * Autonomic/peripheral neuropathy * Vertigo (can be permanent)
98
What are some respiratory symptoms during an Acute Porphyria attack?
Respiratory failure.
99
What autonomic dysfunction symptoms can occur in Acute Porphyria?
* Labile BP * Hypertensive crisis * Tachycardia/arrhythmias
100
What gastrointestinal symptoms are associated with Acute Porphyria attacks?
* Abdominal pain * Vomiting
101
What renal impairment symptoms can occur in Acute Porphyria?
Red/purple urine.
102
What immediate actions should be taken during an Acute Porphyria crisis?
Stop surgery/trigger.
103
What airway management may be needed during an Acute Porphyria crisis?
Intubation if seizure/unresponsive/uncontrolled agitation.
104
What should be done for breathing support during an Acute Porphyria crisis?
Ventilation for respiratory failure.
105
What circulation management interventions are recommended during an Acute Porphyria crisis?
Beta blockers for ↑BP and HR; NOT HYDRALAZINE or AMIODARONE.
106
What medications are recommended for disability management during an Acute Porphyria crisis?
Midazolam, Keppra, MgSO4, or propofol for seizure control; NOT PHENYTOIN or BARBITURATES.
107
What type of analgesia is recommended during an Acute Porphyria crisis?
Opioids; NOT KETAMINE.
108
What electrolyte management is needed during an Acute Porphyria crisis?
Monitor for fluid retention and hyponatremia.
109
What is the antidote for Acute Porphyria?
Hematin 3mg/kg IV (inhibits ALA synthase).
110
What is the carbohydrate load recommended for Acute Porphyria treatment?
20g/hr.
111
What treatment can be done for severe cases of Acute Porphyria?
Plasmapheresis.

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