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35 Cards in this Set
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MZ76 ANZCA Version [Mar06] Q103 |
ANSWER D
Nephron Destruction -as GFR fall, less PO4 is filtered and excreted -serum PO4 does not raise initially because of increased PTH secretion and therefore renal excretion -As GFR falls towards stages 4 and 5, hyperPO4 develops -HyperPO4 suppresses the hydroxylation of 25-hydroxyvitamin D to calcitriol (reduced Vit D) -Reduced Vit D -Reduced intestinal Ca reabsorption -Hypocalaemia Low serum calcitriol levels, hypocalcemia, and hyperphosphatemia have all been demonstrated to independently trigger PTH synthesis and secretion. As these stimuli persist in CKD, particularly in the more advanced stages, PTH secretion becomes maladaptive and the parathyroid glands, which initially hypertrophy, become hyperplastic. The persistently elevated PTH levels exacerbate hyperphosphatemia from bone resorption of phosphate. |
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MZ39 ANZCA version [2002-Mar] Q51, [2002-Aug] Q51, [2005-Apr] Q8, [2005-Sep] Q5 (Similar reported question in [Mar92]) [2006-Mar] Q1
Epidermolysis bullosa may be associated with A. oesophageal stricture B. anaemia C. amyloidosis D. porphyria E. all of the above |
ANSWER E
Epidermolysis bullosa (EB) is an hereditary group of skin disorders, which may involve mucous membranes, especially particularly those of the oropharynx and oesophagus. The disease is classified into three types: * Simplex * Junctional * Dystrophic Incidence Dystrophic EB occurs in about 1 in every 300,000 births EB Dystrophica * Pseudosyndactlyly * Constriction of the mouth (microstomia) * Oesophageal strictures * Teeth are often poor * Malnutrition * Anaemia * Electrolyte derangements * Hypoalbuminaemia Diseases associated with EB * Porphyria * Amyloidosis * Multiple myeloma * Diabetes mellitus * Hypercoagulable states Management Patients with epidermolysis bullosa are extremely prone to frictional trauma, so all adhesives should be avoided. For example, the eyes should be lubricated rather than taped, special ECG electrodes without tape shoud be used, and any areas of potential friction should be well lubricated. Petroleum jelly gauze is useful for the sites where the anaesthetists fingers support the chin. Although carefully place endrotracheal tubes and laryngeal mask airways are not contraindicated, techniques that do not require their use are ideal. Therefore regional and ketamine based techniques are idea. |
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AC147 [Mar06] Q126
A 26-year-old female with ulcerative colitis has a total colectomy with J pouch formation. Preoperatively she was on regular oxycodone, fluoxetine and prednisolone orally. She has normal renal function and liver function. Her postoperative pain management consists of PCA (patient controlled analgesia) with morphine, regular intravenous tramadol (100 mg every 6 hrs) and regular intravenous paracetamol (1 gm every 6 hrs). When you assess her 24 hrs postoperatively she is agitated, confused and sweaty with a pulse of 120, BP 150/95 and temperature of 38°C. You should: A. administer naloxone B. administer 100 mg of hydrocortisone C. cease her paracetamol D. cease her tramadol E. change her PCA morphine to PCA fentanyl |
ANSWER D
Sounds like serotonin syndrome - hence stopping tramadol is the best answer. |
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MZ72b ANZCA Version [Jul06] Q98
Correct statements regarding chronic fatigue syndrome are that A. cognitive behavioural therapy is a primary treatment of the condition B. it is associated with alcohol abuse (>40g/day) C. it is associated with morbid obesity (BMI>30) D. it is a major depressive disorder E. SSRIs (selective serotonin reuptake inhibitors) are the mainstay of treatment |
ANSWER A
Exclusion criteria 1. Alcohol abuse 2. Morbid obesity 3. Major depressive disorder Treatment 1. CBT |
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AC68a ANZCA version [2002-Aug] Q13, [Jul06] Q21 (Similar question reported in [Apr96] [Aug96] [Apr97] [Jul97] [Apr98])
The risk of seroconversion after a needlestick injury, with a hollow needle from a HIV positive patient, is: (type A) A. 30% B. 15% C. 3% D. 0.3% E. 0.03% |
ANSWER D
Risk of HIV seroconversion 1. Hollow needle : 0.3% 2. Mucous membrane : 0.003% |
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MZ48 ANZCA version [2004-Aug] Q127, [2005-Apr] Q71, [Apr07] [Jul07] (Similar reported question in [Apr97] [Jul97] [Apr98] [Aug99])
Patho-physiological features of patients with morbid obesity include A. a blood volume:body weight ratio which is similar to that of patients with normal body weight B. an increased blood pressure and systemic vascular resistance compared to that of patients with normal body weight C. decreased gastric motility due to increased gastrin secretion D. cardiac pathology resulting from excess body mass and increased metabolic demand E. cardiac pathology resulting mainly from fatty infiltration or fatty change of the heart |
ANSWER D
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TMP-Jul10-003
The COMMONEST organism causing meningitis post spinal is: A. Staph epidermidis B. Strep salivarius C. Staph aureus D. Strep pneumoniae E. ? |
ANSWER B
Sources of bacterial introduction 1. intrinsic or extrinsic contamination of needles, syringes, or injected medications 2. inadequately decontaminated patient skin 3. inadequately cleaned health-care provider hands 4. a contaminated sterile field 5. droplet transmission from the health-care provider's upper airway Most common organisms identified 1. Strep Salivarious 50-60% 2. Haemophilus influenzae, 3. Neisseria meningitides 4. Streptococcus pneumoniae. |
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TMP-Jul10-038
Chronic alcohol use. Which is not an associated complication ? A. Pancreatitis B. Atrial fibrillation C. Macrocytosis D. Nephrotic syndrome E. Hypertriglyceridaemia |
ANSWER D
CNS Wernicke–Korsakoff syndrome Peripheral neuropathy Autonomic dysfunction Metabolic Obesity Hyperlipidaemia Hypokalaemia Hypomagnesaemia Hyperuricaemia Hypoglycaemia CVS Cardiomyopathy Heart failure Hypertension Arrhythmias (e.g. AF, SVT, VT) Haematological Macrocytosis Thrombocytopenia Leucopoenia GI Alcoholic liver disease Pancreatitis Gastritis Oesophageal and bowel carcinoma Musculoskeletal Myopathy Osteoporosis Osteomalacia |
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MZ82
Ehlers-Danlos syndrome. Most important to specifically do all EXCEPT: A. Avoid hyperextension of the neck B. Damage to the teeth C. Avoid joint hypermobility D. Gastro oesophageal reflex E. Strict temperature regulation |
ANSWER E
The Ehlers-Danlos family of disorders is a group of related conditions that share a common decrease in the tensile strength and integrity of the skin, joints, and other connective tissues. Patients can often perform "amazing, almost unnatural, contortions" and worked in circuses. All forms of Ehlers-Danlos syndrome share the following primary features to varying degrees: * Skin hyperextensibility * Joint hypermobility and excessive dislocations * Tissue fragility * Poor wound healing, leading to wide thin scars ("cigarette paper scars") * Easy bruising |
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MZ80 [Mar10][Jul10]
Arterial blood gases in a patient: pH 7.12, PO2 100, PCO2 65, HCO3 20.3, BE -10. These results are consistent with? A. Chronic renal failure B. Malignant hyperthermia C. Diabetic ketoacidosis D. End-stage respiratory failure E. Ethylene glycol toxicity |
ANSWER B
A significant mixed acidosis is present (high CO2 AND low HCO3). This makes A/C/E unlikely as they will usually cause hyperventilation and a LOW PCO2. D is also unlikely to be correct, as chronic respiratory failure will cause the HCO3 to rise. The best answer is B - consistent with Malignant Hyperthermia. |
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MZ79 [Jul07]
In Marfan’s Syndrome which is NOT related: A. If develop aortic disease most likely to be aortic stenosis B. At risk iliac aneurysm C. development of mitral valve prolapse is more likely than in general population D. Cardiac myopathy due to medial cystic necrosis/atrophy/ degeneration E. Intracranial aneurysm |
ANSWER A
MARFANS SYNDROME OVERVIEW • AD inheritance • mutation in fibrillin 1 gene (connective tissue protein) • mean age survival 32years, death almost always due to cardiovascular abnormalities CLINICAL FEATURES Skeletal • Tall stature • Long tubular bones, joint hyper-extensibility • high arched palate Resp • pectus excavatum • kyphoscoliosis • high incidence spontaneous pneumothorax Ocular • lens dislocation, myopia, retinal detachment Cardiac • aortic dissection, aortic regurgitation • MVP • cardiac conduction abnormalities esp BBB ANAESTHETIC IMPLICATIONS • Airway difficulties are uncommon. TMJ dislocation is possible • Avoid sustained increases in systolic blood pressure in view of risk of aortic dissection, consider peri-op B-blockade & invasive monitoring • high index of suspicion for development of pneumothorax • easy joint dislocation |
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MZ78 ANZCA version [Jul07] Q138
Which of the following statements regarding patients with ankylosing spondylitis is FALSE? A. amyloid renal infiltration is rarely seen B. cardiac complications occur in less than 10% of cases C. normochromic anaemia occurs in over 85% of cases D. sacroileitis is an early sign of presentation E. uveitis is the most common extra-articular manifestation |
ANKYLOSING SPONDYLITIS
OVERVIEW • inflammatory arthritis of sacroiliac joints and spine, characterised by progressive stiffening and fusion of the axial skeleton • associated with HLAB27 in > 90% • male: female 4:1 ANAESTHETIC IMPLICATIONS ARTICULAR • Potentially difficult intubation: - kyphosis - fixation of the spine, risk of cervical spine fracture - atlanto-occipital subluxation - TMJ dysfunction - Crico-arytenoid arthritis • Spinal disease begins in the sacro-iliac joints and moves cranially • likely difficult mask ventilation also • Difficult neuraxial blocks - paramedian spinal most successful - increased incidence epidural haematomas • Limited chest expansion (arthritic involvement of costovertebral joints) • Can be difficult to position intraoperatively NON-ARTICULAR • anterior uveitis (most common extra-articular manifestation) synonymous with iritis. • fibrosing alveolitis, pulmonary fibrosis • Aortic Regurgitation (1%) • Rarely mitral valve involvement and conduction defects • Amyloidosis causing renal impairment • Cauda equina syndrome • Inflammatory bowel disease • normocytic anaemia TREATMENT • Anti-TNFa medication (infliximab, entanercept): - increases incidence of wound infection - avoid in severe heart failure |
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MZ74 ANZCA version [2005-Sep] Q116, [Mar06] Q52
Immunological evidence of immunity to hepatitis B is the presence of A. hepatitis B core antibodies B. hepatitis B core antigen C. hepatitis B surface antibodies D. hepatitis B surface antigen E. any of the above |
ANSWER C
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MZ73 ANZCA Version [Mar06] Q143
The double stranded hepatitis B virus can survive outside the body for A. less than 4 hours B. six to twelve hours C. one to two days D. two to seven days E. more than seven days |
ANSWER E
HBV can survive outside the body at least 7 days and still be capable of causing infection |
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MZ71 [2005-Sep] Q145 | Aug10
With regards to obstructive sleep apnoea (OSA), which of the following statements is INCORRECT? A. hypoxaemia is the main stimulus to arousal B. the main method of treating this syndrome is with Continuous Positive Airway Pressure (CPAP) C. this syndrome is the most likely diagnosis in patients presenting with excessive daytime sleepiness D. this syndrome occurs in up to 5% of adults E. this syndrome rarely has an obstructive component |
ANSWER E
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MZ70 ANZCA version [2004-Aug] Q105
Adults with chronic renal insufficiency can maintain a normal plasma potassium concentration via tubular secretion until glomerular filtration rate falls below A. 100ml/min B. 80ml/min C. 50ml/min D. 20ml/min E. 8ml/min |
ANSWER D
Classification * Chronic renal failure (CRF) is defined as a permanent reduction in glomerular filtration rate (GFR) sufficient to produce detectable alterations in well-being and organ function. This usually occurs at GFR below 25 ml/min. * Four stages of decreased renal function may be visualized: 1. Silent – GFR up to 50 ml/min. (=3000ml/hr) 2. Renal insufficiency – GFR 25 to 50 ml/min. (=1500-3000ml/hr) 3. Renal failure – GFR 5 to 25 ml/min (=300-1500ml/hr) 4. End-stage renal failure – GFR less than 5 ml/min. (<300ml/hr) |
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MZ69 ANZCA version [2004-Apr] Q95, [2004-Aug] Q10
A 32 year-old man has a four day history of progressive weakness in the extremities. He has been well apart from an upper respiratory tract infection 10 days ago. His temperature is 37.8OC and respiratory rate 42 breaths.min-1 and shallow. He has symmetric weakness on both sides of his face and the proximal and distal muscles of the extremities. Sensation is intact. His most likely diagnosis is A. acute disseminated encephalomyelitis B. Guillain-Barre syndrome C. myasthenia gravis D. poliomyelitis E. polymyositis |
ANSWER B
A. Acute disseminated myeloencephalopathy * ? B. Guillain-Barre syndrome - Absolutely correct ANSWER * Half have preceding URTI or GIT symptoms * RR 42 fits with respiratory muscle paralysis * More than 50% of patients have facial palsies * Bulbar involvement can be present (note another MCQ asks about pseudobulbar palsy) * Intact sensation (or mild impairment) is a feature of GBS * Usually afebrile at onset of symptoms D. Poliomyelitis - False * Uncommon in the West * 7-14 days incubation → acute febrile illness with URTI or GIT symptoms (90% infections subclinical) * Ventilation required in acute illness in 30% * Asymetrical LMN weakness in 1% E. Polymyositis - False * Weakness of proximal muscle groups (shoulders, hips, neck) * Dysphagia and pulmonary aspiration * ↑ CK * Heart block |
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MZ68 ANZCA version [2004-Apr] Q130
Osteogenesis imperfecta is associated with A. blindness B. cleft palate C. hypothermia D. mitral stenosis E. thrombocytopaenia |
ANSWER B
Osteogenesis imperfecta is a autosomal recessive disorder of collagen Presents with a triad of * Multiple bone fractures with easy fracturing of bone * Easy bruising * Deafness Clinical signs * Delayed bone age * Mutiple fractures * Phosph./calcium metabolism abn. * Restricted joint mobility * Teeth anomalies * Wormian bones * Blue sclerae * Cleft soft palate/bifid uvula * Lordosis * Scoliosis * Tendency for hyperthermia * AV and MV insufficency * Qualitative platelet defect |
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MZ66 ANZCA version [2003-Aug] Q94
Ehlers-Danlos syndrome is associated with each of the following EXCEPT: A. blood vessel fragility B. glaucoma C. mental retardation D. mitral valve prolapse E. resistance to local anaesthetics |
ANSWER C
The Ehlers-Danlos family of disorders is a group of related conditions that share a common decrease in the tensile strength and integrity of the skin, joints, and other connective tissues. Patients can often perform "amazing, almost unnatural, contortions" and worked in circuses. All forms of Ehlers-Danlos syndrome share the following primary features to varying degrees: * Skin hyperextensibility * Joint hypermobility and excessive dislocations * Tissue fragility * Poor wound healing, leading to wide thin scars ("cigarette paper scars") * Easy bruising |
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MZ67 ANZCA version [2001-Apr] Q124 (type K MCQ)
In adults with sleep apnoea presenting for surgery 1. sedative premedication should be avoided 2. the incidence of difficult intubation is higher than average 3. anaesthesia may increase the depression of hypoxic and hypercapnic responses 4. major neuraxial anaesthesia is contra-indicated because of interference with abdominal musculature |
ANSWER 1,2,3
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MZ65 ANZCA version [2003-Aug] Q150, [2004-Apr] Q22
Signs consistent with cocaine overdose include all of the following except A. arrhythmias B. dysphoria C. Hyperglycemia D. Hyperthermia E. Miosis |
ANSWER E
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MZ63 ANZCA version [2002-Mar] Q81
Paget's disease of bone is LEAST likely to affect the A. heart B. respiratory system C. renal system D. neurological system E. vertebral column |
ANSWER B
PAGETS DISEASE Chronic disorder characterised by breakdown & regrowth of bone, resulting in enlarged & deformed bones AETIOLOGY • men & women equally affected • familial tendency • increased incidence in patients > 50yrs, present in ~10% of those > 80yrs PATHOPHYSIOLOGY • Excessive osteoblastic and osteoclastic activity • Abnormally thick but weak bones • Bones most commonly involved: Pelvis, leg bones, vertebrae, humerus & clavicle • cause unknown, believed to be caused by a progressive infection, present for many years before symptoms appear TREATMENT • calcitonin • bisphosphonates (inhibit osteoclasts, treatment of choice) CLINICAL FEATURES • often asymptomatic (up to 70% patients with Pagets will be asymptomatic) • Bones: - bone pain (most common symptom) - enlarged bones - fractures - deformity - arthritis - sarcoma (rare - < 1% of patients with Pagets disease) • Neurological (nerve compression from enlarged bone) - hearing loss o may be sensorineural (VIII n compression) o may be conductive (involvement of middle ear ossicles) o or mixed - other CN palsies - headaches • High output cardiac failure • Renal calculi • Loose teeth (Pagets affecting the facial bones) REFERENCES Stoelting Anaesthesia & co-existing disease ‘Diagnosis & Treatment of Pagets disease of bone’ American family physician 2001 |
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MZ61 ANZCA version [2001-Aug] Q149, [2002-Mar] Q126
A 50 year old male with sleep apnoea presents for a laparoscopic cholecystectomy. Factors which may worsen this patient's tendency to airway obstruction post-operatively include 1. multiple attempts at intubation 2. supine position 3. narcotic analgesia 4. presence of a nasogastric tube |
ALL CORRECT
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MZ60 ANZCA version [2001-Aug] Q70, [2002-Mar] Q33, [2003-Aug] Q15, [2004-Apr] Q10
Conditions which cause a metabolic acidosis with normal anion gap include A. chronic renal failure B. diabetic ketoacidosis C. salicylate poisoning D. renal tubular acidosis E. ethylene glycol poisoning |
ANSWER D
Causes of metabolic acidosis with increased anion gap * Lactic acidosis * Circulatory arrest * Pulmonary oedema * Gram negative sepsis * ketoacidosis * toxins * nitroprusside * fructose * acid-citrate-dextrose blood [edit] Causes of metabolic acidosis with a normal anion gap: * renal tubular acidosis * diarrhoea * biliary / pancreatic fistulae * excessive infusion of normal saline A nice easy way to remember all of the common causes of metabolic acidosis - PKUSMAL (patients with DKA have Kussmall respiration) * Paraldehyde * Ketones * Uraemia * Salicylates * Methanol * Alcohol * Lactate |
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MZ58 ANZCA version [2001-Aug] Q126, [2002-Mar] Q129 (type K MCQ)
Clinical signs of a low serum magnesium level after prolonged I.V. nutrition include 1. muscular weakness 2. tetany 3. hallucinations 4. hyperventilation |
ANSWER 1, 2, 3
Hypomagnesemia -occurs in nearly 12 percent of hospitalized patients -60 to 65 percent of intensive care unit patients Signs and symptoms -generalized weakness -anorexia -positive Trousseau and Chvostek -Apathy -delirium -coma -generalized convulsions ECG changes -ventricular arrhythmias -widening of QRS complex -Peaked T waves -prolonged PR interval Associations 1. hypokaelaemia : diuretic therapy and diarrhoea 2. Inhibtion of PTH : hypocalaemia |
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MZ57 ANZCA version [Jul00] [2001-Aug] Q13, [2002-Mar] Q11, [2003-Apr] Q10
The following blood gas result is from a patient breathing room air who has been unwell for three days pH 7.56 PCO2 46 mmHg PO2 90 mmHg HCO3 39 mEq.L-1 B.E. +16.2 mEq.L-1 These results can best be described as A. primary respiratory acidosis with metabolic compensation B. uncompensated primary metabolic alkalosis C. compensated secondary respiratory alkalosis D. primary metabolic alkalosis with partial respiratory compensation E. mixed metabolic and respiratory alkalosis |
ANSWER D
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MZ55 ANZCA version [2001-Aug] Q77 (Similar reported question in [Apr99])
In patients with acute symptomatic post-operative hyponatraemia A. men and women are equally susceptible and have similar risks for severe morbidity and mortality B. rapid correction at a rate greater than 12 mmol.l-1/day carries a risk of osmotic demyelination syndrome C. treatment should consist of hypertonic saline alone and frusemide is relatively contra-indicated D. mortality is less than 10% and is lower in children compared withadults E. sodium deficit is calculated using the formula: mmol Na required = (desired Na - present Na) x 0.4 x weight (kg) |
ANSWER C
A. men and women are equally susceptible and have similar risks for severe morbidity and mortality FALSE. They are equally susceptible, but women appear to be at dramatically increased risk of severe morbidity and mortality. B. rapid correction at a rate greater than 12 mmol.l-1/day carries a risk of osmotic demyelination syndrome FALSE. It does carry this risk even in acute situations. The risk is very small however if the correction is less than 1mmol/hr in the setting of acute hyponatraemia. C. treatment should consist of hypertonic saline alone and frusemide is relatively contra-indicated TRUE. intracellular overhydration coupled with extracellular and intravascular fluid deficit. Conc Na fixes both. Pulmonary oedema is a potential risk but is actually quite rare unless the patient is particularly prone to it. Frusemide compounds the extracellular deficit without aiding the hypo-osmolar state. A hydro-retic agent (ie anti-desmopressin!) would work but we haven't got them yet. D. mortality is less than 10% and is lower in children compared withadults FALSE. If only SEVERE postoperative hyponatraemia is considered then the mortality is 25%. If all symptomatic hyponatraemia is considered, it is less than 5%. E. sodium deficit is calculated using the formula: mmol Na required = (desired Na - present Na) x 0.4 x weight (kg) False. The formula is based on TBW, ie 0.6 x weight. |
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MZ51 [Jul98]
Obstructive sleep apnoea: upper airway muscles vs diaphragm; not as sensitive to: A. IV induction agents B. Inhalation agents C. Opioids D. Neuromuscular blockers |
ANSWER D
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MZ47 ANZCA version [2001-Aug] Q36, [2002-Aug] Q37, [2003-Apr] Q42, [2005-Apr] Q35, [2005-Sep] Q20 (Similar reported question in [Apr96] [Apr97] [Jul97] )
Radiation from X-rays A. has a non-cumulative effect on the bone marrow B. affects the thyroid gland more in patients over 16 years old C. has a recommended maximum whole body dose for the general population of 1,000 µSv.year-1 D. should be limited to less than 100 µSv.year-1 in pregnancy E. has a recommended maximum whole body dose for occupational exposure of 500,000 µSv.year-1 |
ANSWER C
* A. False. Cumulative. * B. False. Affects patients under 16y more. * C. TRUE. * D. False. Should be limited to less than 1mSv per year (same as general population). * E. False. 50mSv per year = 50,000 microSv per year. |
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MZ46 [Aug95] [Apr97] [Jul97] [Jul98] [Apr99]
With obstructive sleep apnoea syndrome: A. More common in males than females B. Can develop pulmonary hypertension, polycythaemia, and cor pulmonale C. Commonly die of a cardiac event D. Can get anatomical pharyngeal narrowing just posterior to the tongue |
ALL TRUE
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MZ45b ANZCA version [2003-Apr] Q80
Pain continuing well after the rash of Herpes Zoster has disappeared (Post-Herpetic Neuralgia): A. Has an incidence of approximately 25% B. Is more common after ophthalmic than after spinal nerve involvement C. Is more common in the immunocompromised but is independent of age and gender D. is best treated with high dose opioids E. Has been shown to occur less commonly after acyclovir (Zovirax) |
ANSWER E
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MZ41b Actual ANZCA version [2003-Apr] Q41
Patients with ankylosing spondylitis usually exhibit A. skin rash B. raised Erythrocyte Sedimentation Rate (ESR) C. positive test for Rheumatoid Factor D. HLA B27 antigen E. urethritis |
ANSWER D
Extra-articular findings in ankylosing spondylitis * Acute anterior uveitis * Aortic insufficiency (about 3%) * Inflammatory bowel disease (symptomatic in 5-10%) * Slowly progressive upper lobe pulmonary fibrosis * Cardiac conduction defects including 3rd degree AV block * Prostatitis * Amyloidosis * Peptic ulcer disease secondary to treatment (steroids, NSAIDs) B27 >90%. ESR up in 75% |
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MZ39 ANZCA version [2002-Mar] Q51, [2002-Aug] Q51, [2005-Apr] Q8, [2005-Sep] Q5 (Similar reported question in [Mar92]) [2006-Mar] Q1
Epidermolysis bullosa may be associated with A. oesophageal stricture B. anaemia C. amyloidosis D. porphyria E. all of the above |
ANSWER E
The disease is classified according to three types: * Simplex * Junctional * Dystrophic Incidence Dystrophic EB occurs in about 1 in every 300,000 births (See reference) EB Dystrophica * Pseudosyndactlyly * Constriction of the mouth (microstomia) * Oesophageal strictures * Teeth are often poor * Malnutrition * Anaemia * Electrolyte derangements * Hypoalbuminaemia Diseases associated with EB * Porphyria * Amyloidosis * Multiple myeloma * Diabetes mellitus * Hypercoagulable states |
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MZ37 ANZCA version [2002-Mar] Q3 (Similar reported question in [Aug91])
C-reactive protein: A. is a reliable marker of metastatic breast cancer B. is a chronic phase protein produced in the lungs C. is usually increased dramatically in response to infection D. is found only in the cerebrospinal fluid (CSF) E. decreases in response to tissue trauma |
ANSWER C
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MZ29 [1989] [Mar90] [Aug91] [Aug96]
Neurofibromatosis is associated with:- A. Phaeochromocytoma B. Diabetes mellitus C. Spinal nerve root compression D. Increased serotonin levels E. Pulmonary aspiration F. Pulmonary fibrosis / (or Interstitial lung disease) |
ANSWER A, C, F
NEUROFIBROMATOSIS Group of hereditary diseases characterized by tendency to form tumors of ectodermal and mesodermal tissues. AETIOLOGY • incidence 1 in 3000, prevalence of 1 in 5000 • autosomal dominant, 100% penetrance, defect of tumor suppressor gene (Chromosome 17) • 50% sporadic mutation CLINICAL FEATURES * Two or more neurofibromas on or under the skin, or one plexiform neurofibroma (a large cluster of tumors involving multiple nerves); neurofibromas are the subcutaneous bumps characteristic of the disease, and increase in number with age. * Freckling of the groin or the axilla (arm pit). * Café au lait spots (pigmented, light brown macules located on nerves, with smooth edged * Skeletal abnormalities, such as sphenoid dysplasia or thinning of the cortex of the long bones of the body * Lisch nodules (hamartomas of iris), freckling in the iris * Tumors on the optic nerve, also known as an optic glioma * Macrocephaly in 30-50% of the pediatric population without any hydrocephalus * Epilepsy * Juvenile posterior lenticular opacity ANAESTHETIC CONSIDERATIONS • Airway : - Neurofibroma of tongue, pharynx or larynx may interfere with tracheal intubation - Suspicion raised by history of dysphagia, dysarthria, stridor or change of voice • Respiratory system - Intrapulmonary neurofibroma, pulmonary fibrosis may produce cough and dyspnoea - RV failure - Scoliosis, kyphosis • Cardiovascular system - Hypertension (consider pheochromcytoma or renal artery stenosis) - HOCM - Mediastinal tumors leading to SVC obstruction • Central nervous system - Cerebral and spinal neurofibromas are common - Increased incidence of epilepsy and learning disorders - CVA - Seizures • Gastrointestinal tract - Intestinal tumors may present with pain, GI haemorrhage or perforation - Carcinoid tumors/syndrome • Genitourinary system - Neurofibromas may cause ureteric/urethral obstruction • Musculoskeletal - Vertebral deformities or spinal cord tumors may make spinal/epidural techniques difficult |
