Part 2 Set D

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Part 2 Set D
2016-01-10 15:01:38

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  1. What is Caplan's Syndrome?
    • RA
    • Pneumoconiosis
  2. What is Sjogren's syndrome
    Autoimmune destruction of the exocrine glands,
  3. Gram positive rods in CSF = ?
    What antiBs wud you choose to treat?
    • LISTERIA!!
    • Ampicillin and GENT
  4. If the question is about lungs and it shows you a precipitins result what does it indicate?
  5. How does Addison's present?
    • Abdo Pain
    • Postural drops in BP
    • Hyperpigmentation
    • Wt loss
    • Hyperkalemia
    • Hyponatremia
    • Hypoglycaemia
    • Hypercalcaemia
    • Neutropenia
    • Lymphocytosis
    • Eosinophilia
    • Anaemia
    • Abnormal LFTs
  6. Causes of addisons in the UK in order of most comon?
    • Autoimmune destruction
    • TB
    • Haemorrhage
    • Sepsis (inc Waterhouse-friedrichson due to
    • Shock
    • Mets
    • HIV and opportunistic infections
  7. What conditions are assoc with APCKD?
    • Cerebral aneurysms
    • Hepatic + pancreatic cysts
    • Colonic diverticulae
    • Mitral valve prolapse
  8. What is Gardener's syndrome?
    • Autosomal Dominant
    • Intestinlal polyposis
    • Colon cancer (90% by age 45)
    • Osteomas
    • Soft tissue tumors (Epidermoid, lipomas fibromas)
    • Coloectomy advised once polyps appear.
  9. In a person with a Phaeo. What is your anti hypertensive of choice in the first few weeks?
    Phenoxybenzamine. Pure Alpha blocker. If you start beta blockade alone you leave alpha unopposed to cause a Hypertensive crisis.
  10. What is Refsum's Disease and how does it present?
    • Autosomal Recessive
    • Sensorimotor peripheral neuropathy

    Caused by defective alpha oxidisation of phytanic acid - leading to its accumulation in tissue and high serum levels.

    • Also presents with sensorineural deafness, anosmia, cerebellar ataxia, pes cavus and nightblindnessvisual disturbance secondary to retinitis pigmentosa.
    • Epiphyseal dysplasia causes characteristic SHORTENING of the 4th toe.
  11. Breast cancer and melanomas occuring in a familial cluster = ?
    Either CDKN2A positive or BRCA - 2 positive
  12. What is POEMS syndrome?
    • Polyneuropathy - bilat starting distally
    • Organomegaly
    • Endocrinopathy
    • MGUS
    • Skin changes - bilat

    Occurs in the setting of plasma cell dyscrasias - inc mgus
  13. What is the relationship between tardive dyskinesias and Dopamine antagonists?
    • TD can become chronic on DA therapy.
    • It can persist for many years even after the offending agent has been stopped. 
    • Stopping the offending agent can sometimes worsen symptoms!
    • In 23 of cases the TD resolves at 3 years.
  14. How does GVHD present in bone marrow transplant patients?
    How do you manage?
    • Skin Rash (can progress to erythroderma)
    • Diarrhoea (sometimes bloody)
    • Liver dysfunction (cholestatic)

    • Aggresive Methylpred
    • Dont stop the immunosuppressant
    • Give TPN if diarrhoea is bad and theyre not absorbing
  15. What is whipple's disease and how does it present?
    • Malabsorption, due to trophyerema whippeli.
    • Villi in GIT expand due to macrophage infiltrates which are PAS positive Acid-schiff positive.
    • Wt loss, steatorrhoea, Joint pains, dementia
  16. In a case of indeterminate colitis, what antibodies would point you in which direction?
    • pANCA = UC
    • ASCA = Crohns
    • Anti Saccharomyces cerevisae antibodies
  17. What are the diagnostic criteria of SIADH?
    • Serum sodium 125 or less
    • High urine osmolality
    • Low plasma osmolality
    • High urinary sodium
    • In a euvolemic patient with no evidence of renal, adrenal, thyroid, hepatic or cardiac disease
  18. What drug can be used to induce a nephrogenic diabetes insipidus in a patient with very low sodium. What if this doesnt work?
    • Dimethylchlorotetracycline
    • If that doesnt work. Hypertonic saline
  19. Who gets hepatic adenomas?
    What does a hepatic adenoma look like on ultrasound?
    What do you do with them?
    • Women in their 30s who are on the OCP and have a solitary liver mass.
    • Single, Subcapsular, Hypoechoic, asymptomatic.
    • Resect themLeave them - monitor with aFP.
  20. How is coxiella spread + how do humans get it?
    Spread between animals by ticks.

    Humans get it via unpasteurised milk
  21. which factors affect chance of successful pregnancy in a lady with cystic fibrosis?
    • Multiple exacerbations
    • Chronic pseudomonal infection
    • FEV<60%

    Otherwise chances are fairly good!!
  22. What is DAFNE?
    Dose adjustment for Normal eating.

    Education program for IDDM patients to titrate insulin accordingly
  23. What are the five main sydromes caused by Aspergillus?
    • Asthma - Type 1 hypersenstitivity
    • Aspergilloma
    • Invasive aspergillosis
    • Allergic Bronchopulmonary Aspergillosis - Type 3 hypersensitivity
    • Extrinsic allergic Alveolitis
  24. Oculomasticatory myorhythmia
    Pathognomic for Whipples

    Contraction of the jaw on pendular eye movements (which are constant) and up gaze palsy
  25. Treatment for Botulism?
    Trivalent antiserum + guanidine hydrochloride
  26. How does CADASIL present?
    • Migraine with aura
    • Stroke like episodes
    • Positive family history for migraine and EARLY dementia.

    All in the absence of vascular risk factors.
  27. What is Binswanger's disease?
    Encephalopathy secondary to small vessel arteriopathy caused by hypertension.
  28. Which features are suggestive of Mitochondrial disease?
    • Elevated blood and CSF lactate which increase dramatically on exercise.
    • Short stature
    • Diabetes
    • Heart disease
    • Optic nerve problems
  29. What drugs can cause positive Anti-histone antibodies?
    Drug-induced lupus

    Isoniazid, Phenytoin, Hydralazine, procainamide, chlorpromazine
  30. Causes of pulmonary renal syndromes?
    • Systemic disease
    • Granulomatosis with polyangitis
    • Goodpastures
    • Polyarteritis nodosa
    • Microscopic polyangitis
    • SLE
    • HSP
    • Churg-strauss

    • Primarily pulmonary
    • Bacterial pneumonia with AKI
    • Legionella pneumonia with interstitial nephritis

    • others
    • Uremic pneumonitis
    • Right sided bacterial endocarditis causing glomerulonephritis
    • ARF causing pulmonary oedema
  31. Phaeochromocytoma, cerebellar haemangioblastoma, polycystic kidneys and retinal lesions = ?
    Von-hipple Lindau
  32. CMV pneumonitis in hiv patients presents with...?
    • GI symptoms
    • Eye symptoms
    • Resp symptoms
  33. Fabry's disease?
    • Alpha galcatosidase A deficiency leading to creamed trihexose accumulation.
    • Xlinked recessive
    • Intermittent lancinating pains in extremeties.
    • Triggered by exercise, hot weather and febrile illness
    • HTN, cardiomegaly, renal failure and thrombotic infarction.
  34. Pseudohypoparathyroidism
    PTHreceptor is resistant to PTH. therefore high levels of PTH and low CA2+

    Short stature, short 45 metacarpals, Hypocalcaemia, Learning difficulties

    Treat with calcium and vit d supplements
  35. Causes of restless leg syndrome?
    • Assoc with:
    • Iron deficiency anaemia
    • COPD
    • ESRF
    • Gastric surgery
    • Chronic venous insufficiency
    • hypothyroidism

    Medications = Bblocker, H2antags, neuroleptics
  36. If LFTs show a mixed obstructive and restrictive picture whats the most likely diagnosis?
    Extrinsic allergic alveolitis. Go back and take a good history.
  37. First line treatment for LQTS?
    • B-blockade
    • Then ICD
  38. Which drug is contraindicated in amyloidosis?

    And you'll be tempted to use it because they'll come in with restrictive cardiomyopathy and AF and you'll want to give it....DONT give it. The dig binds to the amyloid and these patients are really sensitive to it.
  39. Features of drug-induced lupus?
    • Age onset 50-70
    • Antihistone bodies positive in drug induced not in SLE
    • C3/4 normal in drug induced, low in SLE
  40. What is the commonest idiopathic seizure syndrome? What do you know about it?
    Juvenile myoclonic epilepsy

    • Seizures types include
    • Absences
    • Myoclonic jerks and
    • Tonic-clonic seizures which tend to occur within an hour of waking.

    Precipitating factors include alcohol, menstruation and sleep deprivation.

    The condition is genetically linked to the short arm of chromosome 6.

    Prognosis is extremely favourable if the condition is treated correctly, with many patients becoming seizure-free. 

    Treatment options include sodium valproate, lamotrigine and topiramate.

    Lifelong drug treatment is usually necessary to avoid relapses in patients who achieve seizure-free status on medication.
  41. What do you get the highest points (3) for in the rockall scoring system?
    Liver failure, renal failure, Metastatic cancer
  42. Why cant you start Allopurinol on someone who is already on azathiprine?
    Allopurinol inhibits xanthine oxidase which stops the breakdown of 6Mercaptopurine.

    Aza needs to be dose reduced by 25%
  43. What factors confer a poor prognosis in Infective Endocarditis?
    Patient characteristics = 

    • Old age
    • Presence of prosthetic valve endocarditis
    • Insulin dependent diabetes mellitus
    • Severe co-morbidities.

    Disease factors

    • Endocarditis due to fungus or Gram negative bacilli
    • Endocarditis complications including heart failure, renal failure, stroke, septic shock and periannular complications
    • Echocardiographic findings including severe left sided valve regurgitation, low left ventricular ejection fraction, pulmonary hypertension, large vegetations and severe prosthetic valve dysfunction.
  44. How does lung cancer TNM translate into staging?
  45. What is the mode of action of Abatacept?
    Cytotoxic T Lymphocyte Antigen 4 Homologue
  46. What is the mode of action of Anakinra?
    Anti IL 1
  47. Name some Anti TNF agents?
    • Adalimumab,
    • etanercept,
    • infliximab,
    • golimumab
    • certolizumab
  48. Why would you start Prednisolone in PCP?
    If PaO2 is <60
  49. What is autonomic Dysreflexia?
    • Happens to people who have a spinal injury above C6.
    • Some Bladder or bowel disturbance results in a poorly understood cascade which increases vagal tone resulting in:

    • Bradycardia
    • Flushing
    • Severe hypertension
  50. What general test can suggest mitochondrial disease?
    Lactate of course!!