The misfits Flashcards

1
Q

Antisynthetase syndrome

A

Rare inflammatory muscle condition that is closely associated with dermatomyositis and polymyositis. The kicker –> Interstitial lung disease, and the give away in questions. Anti-Jo 1 antibody
Prognosis is based on pulmonary disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

ECOG Scale

A
0 = fully active
1 = restricted but ambulatory and light work
2 = ambulatory and capable of all self care - but requires rest ~50% of the day
3= limited self care: bed or chair more than 50%
4= completely disabled, cannot self care
5 = dead
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Granuloma annulare

A

Mediated by TNF alpha. Often occurs in areas that are knocked.
Disseminated form with mauve coloured patch associated with HIV.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Necrobiosis lipoidica

A

Rare disorder affecting the shin of insulin dependant diabetics. Can be round oval shaped or irregular

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Hypersensitivity syndromes

A

Type 1 = Allergy, Allergen - IgE interaction with degranulation of mast cells
Type II = cytotoxic hypersensitivity
Type III = immune complex hypersensitivity
Type IV = Cell mediated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Hypersensitivity syndromes primary mediators

A

1 = IgE, 2 = IgG/M, as with 3, 4 CD4 and CD8

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Type 1 hypersensitivity mechanism

A

Allergen-specific IgE antibodies bind to mast cells via their Fc receptor. When the specific allergen binds to the IgE, cross-linking of IgE induces degranulation of mast cells.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Type 2 Mechanism hypersensitivity

A

IgG or IgM antibody binds to cellular antigen, leading to complement activation and cell lysis. IgG can also mediate ADCC with cytotoxic T cells, natural killer cells, macrophages, and neutrophils.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Type 3 hypersensitivity mechanism

A

Antigen-antibody complexes are deposited in tissues. Complement activation provides inflammatory mediators and recruits neutrophils. Enzymes released from neutrophils damage tissue.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Type 4 hypersensitivity mechanism

A

Th2 cells secreted by cytokines which activate macrophages and cytotoxic T cells.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Type 1 hypersensitivity examples

A

Asthma, rhinitis, atopic eczema

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Type 2 hypersensitivity examples

A

Rhesus incompatibility, transfusion reactions, cell destruction due to autoantigens, drug induced haemolysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Type 3 hypersensitivity examples

A

GN, SLE, Farmers lung, vasculitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Type 4 hypersensitivity

A

Tuberculin reaction, granuloma formation, allergic contact dermatitis, type 1 diabetes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Hypersensitivity reactions mnemonic

A

“ACID”

The “A” stands for Allergic/Anaphylactic (Type I), the “C” stands for Cell-triggered (Type II), the “I” is for Immune Complex Deposition (Type III), and the “D” stands for ‘Delayed’ (Type IV).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

hypersensitivity examples mnemonic

A

AnGST –(as in, you’d probably feel angst about someone pouring acid on your skin.) A is for “anaphylaxis, asthma, atopy”, G for “Goodpasture’s disease, Grave’s disease, and myasthenia Gravis”, S for “Systemic lupus erythematous and serum sickness”, and T for “T-cell mediated diseases like the TB test and transplant rejection”.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Cardio/circulatory changes in pregnancy

A

Blood volume increases to 50% of that of normal circulating volume (by week 32)
Stroke volume initially increases to compensate, but later in the pregnancy it is in fact the heart rate that augments.
Pulse pressure widens as systemic vascular resistance falls

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Purpose of gemfibrozil

A

Increase HDL levels and decrease plama triglyceride levels

MOA is unclear ? Stimulates lipoprotein lipase activity via peroxisome proliferator activated receptor alpha (PPARa)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Targets for cholestrol/LDL levels

A
total Chol <4 
HDL >1
LDL <2 
Non-HDL-C <3
TG < 2 
`
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Statin MOA

A

 Competitively inhibits enzyme COA-reductase

 Increase hepatic uptake of cholesterol from blood, reduce LDL, modestly reduce triglycerides and increase HDL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Ezetemibe MOA

A

 Reduces absorption of dietary and biliary cholesterol.
 This leads to upregulation cellular LDL receptors on the surface of cells and increased LDL cholesterol uptake into cells.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

NAGMA

A

HCO3 loss and replaced with Cl- -> anion gap normal
if hyponatraemia is present the plasma [Cl-] may be normal despite the presence of a normal anion gap acidosis -> this could be considered a ‘relative hyperchloraemia’.

USEDCRAP

Ureteroenterostomies
Small bowel fistula
Excess Chloride
Diarrhoea
Carbonic anhydrase inhibitors
Renal tubular acidosis
Addisson’s disease
Pancreatoenterostomies
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is a normal anion GAP

A

4-10

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Commonest organism implicated in septic arthritis

A

Although Staphylococcus aureus is the most common cause of monomicrobial septic arthritis overall, among sexually active adults, N. gonorrhoeae is the most common causative organism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Congenital deficiency leading to recurrent Neisseria infections

A

Complement deficiency of C5,6,7,89, i..e can’t form MAC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

How to differentiate inflammatory vs. infective synovial fluid

A

WBC count - typically >2000 in inflammatory but greater than 20,000 in septic

Non-inflammatory will be less than 2000

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

How to differentiate gout from pseudogout on crystal analysis

A

Monosodium urate crystals are brightly birefringent and are needle shaped
Calcium pyrophosphate crystals have a rhomboid shape or rectangle they are WEAKly birefringent.
I.E. the same as ‘pseudo’ gout they are ‘pseudo’ refringent

As a random tidbit - if lipids are seen ‘a maltese cross sign’ then suspect fracture

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Place to do knee joint aspirate as per UpToDate

A

Medial epicondyle and patella as landmarks then aim postero-inferiorly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Primary signs of hyperaldosteronism

A

Hypertension and hypokalaemia +/- metabolic alkalosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Low Renin and High aldosterone doesn’t clinich the diagnosis of primary hyperaldosteronism unless one of three factors is also present

A

The exception to the requirement for confirmatory testing is the patient with:

  • Spontaneous hypokalemia
  • Undetectable PRA or PRC
  • PAC 555 pmol/L
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Ratio of Plasma aldosterone concentration to plasma renin activity

A

PAC/PRA ratio greater than 20 (depending upon the laboratory normals)

32
Q

Secondary hyperaldosteronism such as renovascular disease pattern of renin aldo

A

PRA (or PRC) and PAC are increased and the PAC/PRA ratio is <10

33
Q

Liddle’s syndrome

A

The underlying defect is a gain-of-function mutation in the collecting tubule sodium channel.
Autosomal dominant: presents with hypernatraemia and hypokalaemia

34
Q

HLA-A3 association

A

Haemachromatosis

35
Q

HLA B51 association

A

Bechets disease (positive pathergy test)

36
Q

HLA B27 positive association

A

Ank spondylitis
Reiters syndrome
Acute anterior uveitis

37
Q

HLA Dq2-DQ8 association

A

Coeliac disease

38
Q

HLA-DR2 association

A

Good pastures and Narcolepsy

39
Q

HLA DR3 association

A

Dermatitis herpetiformis
Sjogrens syndrome
Primary biliary cirrhosis

40
Q

HLA DR4

A

Type 1 diabetes

Rheumatoid arthritis in particular DR B1

41
Q

Burkitts lymphoma summary

A

8,14 mutation associated with c-myc. B cell malignancy with a strong association with EBV. Lymphoma with high risk of tumour lysis syndrome - therefore rasburicase given pre-chemotherapy. Its MOA is increasing the solubility of uric acid to allantonin

42
Q

Procedure that specifically do not require antimicrobials when you think they may

A

Genitourinary and gastrointestinal procedures. Cesarean and normal delivery also not required.

43
Q

Oral regimens for endocarditis prophylaxis

A

Amoxicllin 2 g if not penicillin allergic

Cephlex, clinda or azithro if allergic

44
Q

Typical history for acute closed angle glaucoma

A

Painful red eye, halos around lights, decrease in vision, headaches

Usually triggered by rapid pupillary dilatation i.e. in dark room.
Aqueous humour can’t escape and builds up between the iris and the cornea.

45
Q

Iritis aka anterior uveitis typical history

A

‘Ciliary flush’ may be seen - this is a red ring around the iris.
Consensual light reflex exhibits pain. i.e light in opposite eye.
Infection, autoimmune disease and inflammatory conditions can all predispose.

46
Q

Keratitis (i.e. corneal iritation) typical history

A

Can be bacterial or viral. Bacterial typically use contacts. Typically there is a corneal opacity. Herpes simplex keratitis appears as a serpigenous or dendritic ulcer on fluroscein

47
Q

Scleriritis typical history

A

Severe boring pain to eye. Red, inflamed. Worse with movement. Nodular sclera. COMMON WITH RHEUMATOID
IBD
Granulomatosis with polyangiitis

48
Q

Causes of a painless red eye

A

Blepheritis, subconjunctival haemorrhage, conjunctivitis, episcleritis, keratoconjuntivitis sicca

49
Q

Alcohol cessation drugs and reasoning

A

A brief intervention - even as short as 5 minutes has valid data in reducing alcohol use.

If pharmacotherpy is needs: “Remember Alcohol N Drugs”

A = Acamprosate: assists with anxiety, use in cirrhosis (except C), can use in heart disease

N = Naltrexone: Once daily, can use in heart disease and caution in renal disease (seek renal advice if Cr >120)

Disulfiram = Once daily, can’t use if complex medically (heart disease, liver disease, renal disease)

50
Q

Site of action of all diuretics

A

Carbonic anhydrase - NaHCO3 reabosorption in prox tubule

Thick ascending loop Na + K + Cl = loop diuretics

Thiazides in distal tubule on Na - Cl

K sparing/aldosterone blockers Na + K + H collecting duct.

51
Q

4 Main renal stone types and causes

A

Calcium stones = hyperparathyroid, renal calcium leak, hyperoxaluria (short gut or rhubarb), hypomag, hypocitraturia

Uric acid stones: pH less than 5, high intake of purine foods. Associated with gout

Struvite stones: gram neg urase positive organisms (proteus, klebsiella– NEVER E.Coli)

Cystine - intrinsic metabolic defect causing failure of renal tubules to absorb Cystine, lysin, ornithin, arginine.

52
Q

Best test for renal stones

A

CT non contrast KUB/abdo pelvis

Over 5mm unlikely to pass on their own

53
Q

Medical therapy post stones

A

Uric acid : lower with allopurinol is implicated. Also can make urine more alkaline with sodi bic.
Thiazide diuretics are recommended for patients with recurrent stones.
Cystine with D penicillamine and alkalinization.

54
Q

Which stones are radiolucent

A

Cystine are faint
Urin acid - completely radiolucent.
Likewise stones formed by indinanir (ICU is the mnemonic)

55
Q

Thiamine deficiency overview

A

Wet or dry beri beri:
Lack of thiamine or Vitamin B 1
Stores typically only last 1 month.

Important co-enzyme in the formation of glucose, acetyl-CoA and decarboxylation of alpha ketoacidosis

Typical of alcoholics, starvation. Can be from decrease absorption, severe liver disease and also with folate deficiency.

Dry beri beri: bilateral sensory and motor neuropathy and weakness with hyporeflexia.

Wet beri beri: tachycardia, vasodilatation, high cardiac output, fluid overload

Wernicke’s : alterned mental state, opthalmoplegia (horizontal nystagmus), ataxia and altered vestibular function

Korsakoff: chronic, memory loss and confabulation

56
Q

Rheumatoid arthritis and lung disease: most common finding

A

Pleural effusion

Lung disease is second to infection as a cause of death in patients with RA.

57
Q

Predictor of lung disease development in Scleroderma

A

Systemic sclerosis typically is associated with a pulmonary fibrosis ion a UIP pattern. The presence of SCL-70 antibodies is a strong predictor of lung fibrosis/lung involvement
Anti-centromere antibodies are protective.

58
Q

Typical cause of lung damage in polymyositis/dermatomyositis

A

Lung involvement does not primarily involve the pleura but instead typically is secondary to aspiration pneumonia from pharyngeal muscle weakness.

There is often also diaphragmatic elevation and decreased lung volumes.

If possessing anti-Jo-1 antibodies however this points toward chronic bronchioilitis /antisynthetase syndrome.

59
Q

Sjögrens syndrome lung involvement

A

Upper airways are bried. Often mucous plug inspiration can not be mobilised for ejection. Most commonly a pulm. fibrosis also.

60
Q

Mixed connective tissue disease definition and lung involvement

A

Typified by overlaps with SLE and SSc and PM.

Anti-U1-RNP is typical of this disease. Often lung disease is not a strong feature

61
Q

Churg-strauss lung disease

A

Also now called Eosinophilia granolomatosis with polyangiitis
Almost exclusively affects patients with a history of asthma.
Lungs are the most common organ involved. Allergic rhinits, eosinophilia and fever all point towards it.

62
Q

Upper lobe predominant bilateral pulmonary pathologies

A
SET CAP
Silicosis and sarcoid
Extrninsic alveolitis and Eosinophilic granuloma
TB/fungal
CF
Ank spond
PCP
63
Q

Lower lobe predominant bilateral pulmonary pathologies

A
Bronchiectasis
Aspiration
DIP
Rheumatoid
Asbestosis
Scleroderma
Hamman-Rich syndrome aka acute interstital pneumonia
64
Q

Which immunosuppressive drug most increases your risk of skin cancer ?

A

Azathioprine

65
Q

Rasburicase MOA

A

Catalyses Poorly soluable uric acid into allantoin with CO2 and H2O2

66
Q

Allopurinol MOA

A

Xanthine oxidase inhibitor

67
Q

Purine Catabolism pathway

A

Purine –>hypoxanthine –> xanthine –> uric acid –> allantoin (via urate oxidase)

68
Q

Mnemonic for seizures/first seizures

A

SICK DRIFTER

Substrates (Sugar, oxygen)
Isoniazid OD
Cations (Na, Ca, Mg)
Kids (Eclampsia)
Drugs (Crap = Cocain rum (alcohol), amphetamines, PCP)
Rum
illness (Chronically well mx seizures)
Fever
Trauma
Extra (TAIL = Theophyline Aspirin Isoniazid Lithium, anti-histamine, antidepressant, anticonvulsant (level low or high) Withdrawal (benzo)
Rat Poison (Organophosphate poinsoning - not actually rat poison)

69
Q

Clozapine side effects

A

Agranulocytosis, seizure, myocarditis

70
Q

Mirtazepine important side effect

A

Agranulocytosis

71
Q

Lithium side effects

A

Nephrogenic DI, tremor, hypothyroid, hyper PTH

EBSTEINS ANOMALY if taken in the first trimester

72
Q

Valproate important side effects

A

Transaminitis, thrombocytopenia, neural tube defects

73
Q

Two different types of pneumococcal vax and timing in asplenia

A

PCV13 (conjugate) and PPSV23 (polysaccharide) are both given 8 weeks apart (13 first then 23)

74
Q

Vaccinations recommended in asplenia and restrictions

A

Hib, Pneumococcus (S.Pneumo) and meningocuccs (N.Meningiditis) + Seasonal influenza
No restrictions on other vaccines (live)

75
Q

Two types of meningococcal vaccination

A

MenACWY (like YMCA but upside down back to front) and MenB

76
Q

Live attenuated vaccines

A
My Big Monster 'Ruck TIRES, Your crazy chicken
Measles,  
BCG
Mumps 
Rubella
Typhoid
Influenza
Rotavirus
Endemic typhus
Sabin (oral polio) 
Yellow fever
Chicken pox
Cholera