Green book 2 flashcards

1
Q

Inheritance of haemophilia A and B

A

X-linked

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2
Q

Joint most affected in haemophilia

A

Knee > elbows >ankles > shoulder/wrists

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3
Q

How to manage intramuscular bleeds in haemophiliacs

A

Intramuscular generally needs no treatment (except psoas)

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4
Q

Types of factor VIII replacement

A

Biostate
- plasma derived - factor VIII and vWF

Recombinant factor VIII
- extended half life or short acting

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5
Q

Side effects of DDVAP

A

Hyponatraemia
Fluid retention/weight gain
Tachyphylaxia
AMI/CVA in elderly patients

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6
Q

MOA of tranexamic acid

A

inhibits lysis by plasmin

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7
Q

Complications of haemophilia

A
Joint
Disability / obesity
Development of inhibitor antibodies
Chronic pain
Complications of blood products - infections
Psychosocial issues
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8
Q

Haemophilia arthropathy management

A

Non pharmacological

  • weight loss
  • exercise

Pharmacological
- Prophylaxis 3-4x/week

Surgical

  • Synovectomy
  • Joint replacement
  • Joint fusion
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9
Q

Perioperative management for haemophiliacs

A

Infusion/bolus of factor

No VTE prophylaxis

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10
Q

Haemophiliac not responding to factor

A

inhibitor

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11
Q

treatment for people with inhibitors

A

Novoseven (recombinant factor VII)

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12
Q

How often to monitor bloods for HIV

A

every 3-6 months:

  • CD4
  • HIV RNA
  • UEC, LFT, FBE

Every 6 months
- Lipids, fasting glucose

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13
Q

Opportunistic infections in HIV based on CD4 count

A

200-500
- Herpes, Pneumococcal, TB, lymphoma

50-200

  • TB, PJP, toxo
  • Cancers: Kaposi’s sarcoma, non hodgkin’s lymphoma

<50
- MAC,,CMV, cryptosporidosis

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14
Q

What HIV antiviral drug has higher potency

A

Integrase inhibitor

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15
Q

RF for virologic failure

A

High baseline HIV/low baseline CD4
Slow viral load reduction in response to treatment
Poor adhere/psychosocial barriers

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16
Q

What to ask about HIV progress in treatment experienced person

A
When diagnosed
Currently CD4 count/HIV load
CD4 nadir
Opportunistic infections
Treatment history and reasons for change
Current Mx
Monitoring/co infection
Co-morbidities
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17
Q

HIV exam

A

BP, BMI/waist circumference, lipodystrophy, oral candidiasis, CVS

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18
Q

When to start HIV therapy in setting of an opportunistic infection

A

When the patient is stable on OI treatment

Delayed further if cryptococcal or TB meningitis

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19
Q

nucleoside reverse transcriptase inhibitors

A

tenofovir
emtricitabine
abacavir
lamivudine

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20
Q

non-nucleoside reverse transcriptase inhibitors

A

rilpivirine
efavirenza
nevirapine
etravirine

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21
Q

when are protease inhibitors used

A

to boost levels of ritonavir or cobicistat

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22
Q

What are examples of protease inhibitors

A

atazanavir

darunavir

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23
Q

integrase inhibitors

A

dolutegravir
elvitegravir + cobicistat
raltegravir

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24
Q

types of HIV treatment failures

A

virologic
immunological
clinical progression

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25
reasons for treatment failure (virologic or immunologic)
``` adherence - psychosocial, toxicity drug resistance suboptimal potency of regimen suboptimal pharmacokinetics (drug interactions) more advanced immunodeficiency ```
26
HIV drugs that increase risk of cardiovascular diseases
``` Abacavir Protease inhibitors (darunavir) ```
27
What HIV drugs have CNS toxicities
``` Efavirenza Integrase inhibitors (gravirs) ```
28
What endocrine AE do all ARTs have
osteoporosis/osteopenis
29
Common reasons for switching ART
Simplification of regimen Enhance tolerability Mitigate drug-drug interactions Eliminate food requirements
30
How long after therapy would you expect the viral load to fall below 50 copies/mL
3 months
31
Interactions between CVS risk and HIV
General RFs of CVS Abacavir increases risk of AMI Immuno dysregulation causing atherosclerosis in untreated individuals
32
Biggest issue between HIV and transplant
drug-drug interaction (esp CNI)
33
Cyclosplorin AE
``` nephrotoxicity neurotoxicity gum hyperplasia hirsutism DM Hypophospho/hypomag ```
34
What SE does tacrolimus have more than cyclosplorin
Post transplant diabetes mellitus and neurotoxicity
35
MOA of mycophenolate
blocks purine synthesis and stops proliferation of T and B cells
36
splenectomy vaccines
pneumococcal meningococcal HIb flu vaccine
37
Definition for metabolic syndrome
Central obesity (WC men >94cm >80 women)/BMI >30 PLUS ``` any 2 of: raised triglycerides low HDL hypertension elevated fasting glucose ```
38
autoantibody in mixed connective tissue disease
U1RNP positive
39
treatment of mixed connective tissues disease
as per predominant disease
40
definition of osteoporosis
minimal trauma fracture T score < -2.5 on DEXA T score < -1.5 taking corticosteroids
41
Lifestyle treatment of patients with osteoporosis
``` optimise dietary calcium vit D replacement weight bearing exercise smoking cessation falls prevention ```
42
Secondary causes of osteoporosis
``` malnutrition hyperthyroidism coeliacs disease testosterone deficiency estrogen deficiency diabetes myeloma ```
43
Pharmacological therapy for osteoporosis
Antiresorptive - bisphosphonates - denosumab Anabolic agents - teriparatide
44
what is teriparatide
parathyroid hormone analog
45
how to monitor therapy in osteoporosis treatment
DEXA 1-2 years Bone turnover markers (C-telopeptide, P1NP) - expect lower range of normal Drug holiday after 3-5 years of bisphosphonate if fracture risk is low
46
what kind of vasculitis is polyarteritis nodosa
medium muscular arteries
47
what organs does polyarteritis nodosa affect
most organs can be affected except for lungs
48
treatment of polyarteritis nodosa
steroids, cyclophosphamide ?PLEX tx hypertension
49
What is vasculitis in rheumatoid arthritis associated with
positive rheumatoid factor
50
How does vasculitis in rheumatoid arthritis manifest
Polyneuropathy, mononeuritis multiplex Skin ulcers, gangrene Affected organ infarction
51
Link between osteoporosis and rheumatoid arthritis
steroid use and inflammatory disease
52
sarcoidosis staging on CXR
``` Stage 0 -> 4 0 - nothing 1 - hilar 2 - hilar + infiltrates 3 - infiltrates alone 4 - fibrosis ```
53
Investigations for sarcoidosis
``` CXR ACE levels HRCT Biopsy of affected tissues Resp func tests DLCO ECG/holer ```
54
What does CREST syndrome stand for
``` Calcinosis Raynaud's Esophageal dysmotility Sclerdactyl Telangiectasia ```
55
Organ involvement of scleroderma
``` Skin Vascular Renal Lung GI Cardiac MSK Neurological ```
56
Scleroderma GI manifestations
Oesophageal problems - dysmotility, stricture, dysphagia Bowel dysmotility - alternating diarrhoea and constipation, pseudoobstruction, bacterial small bowel overgrowth Biliary cirrhosis
57
Cardiac manifestations of scleroderma
pericardial disease myocarditis myocardial fibrosis conduction abnormalities
58
Scleroderma MSK manifestations
``` oedema swelling loss of function contractures myositis (overlap syndrome) arthralgias ```
59
risk factors for systemic sclerosis skin hypertensive renal crisis
steroid use | diffuse skin disease
60
What is the criteria for systemic lupus erythematosus
4 of 11: - malar rash - discoid rash - photosensitivity rash - oral ulcers - pleuritis/pericarditis - haematological (cytopenia) - renal lupus - non erosive arthritis - neurological disorder (seizure, psychosis) - immunological disorder (andit ds DNA, anti Sn, antiphospholipid antibody) - positive ana
61
Clinical markers of SLE severity
``` fatigue/malaise hair loss arthralgias pleuritic chest pain sx suggesting new organ involvement ```
62
Laboratory markers of SLE severity
ds DNA decreased complement cytopenias evidence of organ dysfunction
63
Wilson's disease inheritance
autosomal recessive
64
Laboratory diagnosis of wilsons
``` ceruloplasmin screening test urine copper (high) serum copper (low) ``` LIVER BIOPSY
65
Patterns of liver disease in Wilson's
Chronic liver hepatitis Cirrhosis Fulminant liver failure
66
Neuropsychiatric features of Wilson's disease
``` asymmetric tremor excessive salivation ataxia masklike facies personality changes psychiatric abnormalities - behavioural, affective, schizophrenic, cognitive ```
67
MSK manifestations of wilson's disease
OA like arthropathy | spine and large joints
68
What does Kayser-Fleischer rings look like
greenish gold brown
69
Familial screening of Wilson's disease
ceruloplasmin, urine copper, LFTs
70
Treatment for Wilson's disease
chelating agents: - penacillamine, trientine Copper absorption blocking agents - zinc treatment of complications