Green book 2 flashcards
Inheritance of haemophilia A and B
X-linked
Joint most affected in haemophilia
Knee > elbows >ankles > shoulder/wrists
How to manage intramuscular bleeds in haemophiliacs
Intramuscular generally needs no treatment (except psoas)
Types of factor VIII replacement
Biostate
- plasma derived - factor VIII and vWF
Recombinant factor VIII
- extended half life or short acting
Side effects of DDVAP
Hyponatraemia
Fluid retention/weight gain
Tachyphylaxia
AMI/CVA in elderly patients
MOA of tranexamic acid
inhibits lysis by plasmin
Complications of haemophilia
Joint Disability / obesity Development of inhibitor antibodies Chronic pain Complications of blood products - infections Psychosocial issues
Haemophilia arthropathy management
Non pharmacological
- weight loss
- exercise
Pharmacological
- Prophylaxis 3-4x/week
Surgical
- Synovectomy
- Joint replacement
- Joint fusion
Perioperative management for haemophiliacs
Infusion/bolus of factor
No VTE prophylaxis
Haemophiliac not responding to factor
inhibitor
treatment for people with inhibitors
Novoseven (recombinant factor VII)
How often to monitor bloods for HIV
every 3-6 months:
- CD4
- HIV RNA
- UEC, LFT, FBE
Every 6 months
- Lipids, fasting glucose
Opportunistic infections in HIV based on CD4 count
200-500
- Herpes, Pneumococcal, TB, lymphoma
50-200
- TB, PJP, toxo
- Cancers: Kaposi’s sarcoma, non hodgkin’s lymphoma
<50
- MAC,,CMV, cryptosporidosis
What HIV antiviral drug has higher potency
Integrase inhibitor
RF for virologic failure
High baseline HIV/low baseline CD4
Slow viral load reduction in response to treatment
Poor adhere/psychosocial barriers
What to ask about HIV progress in treatment experienced person
When diagnosed Currently CD4 count/HIV load CD4 nadir Opportunistic infections Treatment history and reasons for change Current Mx Monitoring/co infection Co-morbidities
HIV exam
BP, BMI/waist circumference, lipodystrophy, oral candidiasis, CVS
When to start HIV therapy in setting of an opportunistic infection
When the patient is stable on OI treatment
Delayed further if cryptococcal or TB meningitis
nucleoside reverse transcriptase inhibitors
tenofovir
emtricitabine
abacavir
lamivudine
non-nucleoside reverse transcriptase inhibitors
rilpivirine
efavirenza
nevirapine
etravirine
when are protease inhibitors used
to boost levels of ritonavir or cobicistat
What are examples of protease inhibitors
atazanavir
darunavir
integrase inhibitors
dolutegravir
elvitegravir + cobicistat
raltegravir
types of HIV treatment failures
virologic
immunological
clinical progression
reasons for treatment failure (virologic or immunologic)
adherence - psychosocial, toxicity drug resistance suboptimal potency of regimen suboptimal pharmacokinetics (drug interactions) more advanced immunodeficiency
HIV drugs that increase risk of cardiovascular diseases
Abacavir Protease inhibitors (darunavir)
What HIV drugs have CNS toxicities
Efavirenza Integrase inhibitors (gravirs)
What endocrine AE do all ARTs have
osteoporosis/osteopenis
Common reasons for switching ART
Simplification of regimen
Enhance tolerability
Mitigate drug-drug interactions
Eliminate food requirements
How long after therapy would you expect the viral load to fall below 50 copies/mL
3 months
Interactions between CVS risk and HIV
General RFs of CVS
Abacavir increases risk of AMI
Immuno dysregulation causing atherosclerosis in untreated individuals
Biggest issue between HIV and transplant
drug-drug interaction (esp CNI)
Cyclosplorin AE
nephrotoxicity neurotoxicity gum hyperplasia hirsutism DM Hypophospho/hypomag
What SE does tacrolimus have more than cyclosplorin
Post transplant diabetes mellitus and neurotoxicity
MOA of mycophenolate
blocks purine synthesis and stops proliferation of T and B cells
splenectomy vaccines
pneumococcal
meningococcal
HIb
flu vaccine
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
autoantibody in mixed connective tissue disease
U1RNP positive
treatment of mixed connective tissues disease
as per predominant disease
definition of osteoporosis
minimal trauma fracture
T score < -2.5 on DEXA
T score < -1.5 taking corticosteroids
Lifestyle treatment of patients with osteoporosis
optimise dietary calcium vit D replacement weight bearing exercise smoking cessation falls prevention
Secondary causes of osteoporosis
malnutrition hyperthyroidism coeliacs disease testosterone deficiency estrogen deficiency diabetes myeloma
Pharmacological therapy for osteoporosis
Antiresorptive
- bisphosphonates
- denosumab
Anabolic agents
- teriparatide
what is teriparatide
parathyroid hormone analog
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
what kind of vasculitis is polyarteritis nodosa
medium muscular arteries
what organs does polyarteritis nodosa affect
most organs can be affected except for lungs
treatment of polyarteritis nodosa
steroids, cyclophosphamide
?PLEX
tx hypertension
What is vasculitis in rheumatoid arthritis associated with
positive rheumatoid factor
How does vasculitis in rheumatoid arthritis manifest
Polyneuropathy, mononeuritis multiplex
Skin ulcers, gangrene
Affected organ infarction
Link between osteoporosis and rheumatoid arthritis
steroid use and inflammatory disease
sarcoidosis staging on CXR
Stage 0 -> 4 0 - nothing 1 - hilar 2 - hilar + infiltrates 3 - infiltrates alone 4 - fibrosis
Investigations for sarcoidosis
CXR ACE levels HRCT Biopsy of affected tissues Resp func tests DLCO ECG/holer
What does CREST syndrome stand for
Calcinosis Raynaud's Esophageal dysmotility Sclerdactyl Telangiectasia
Organ involvement of scleroderma
Skin Vascular Renal Lung GI Cardiac MSK Neurological
Scleroderma GI manifestations
Oesophageal problems - dysmotility, stricture, dysphagia
Bowel dysmotility - alternating diarrhoea and constipation, pseudoobstruction, bacterial small bowel overgrowth
Biliary cirrhosis
Cardiac manifestations of scleroderma
pericardial disease
myocarditis
myocardial fibrosis
conduction abnormalities
Scleroderma MSK manifestations
oedema swelling loss of function contractures myositis (overlap syndrome) arthralgias
risk factors for systemic sclerosis skin hypertensive renal crisis
steroid use
diffuse skin disease
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
Clinical markers of SLE severity
fatigue/malaise hair loss arthralgias pleuritic chest pain sx suggesting new organ involvement
Laboratory markers of SLE severity
ds DNA
decreased complement
cytopenias
evidence of organ dysfunction
Wilson’s disease inheritance
autosomal recessive
Laboratory diagnosis of wilsons
ceruloplasmin screening test urine copper (high) serum copper (low)
LIVER BIOPSY
Patterns of liver disease in Wilson’s
Chronic liver hepatitis
Cirrhosis
Fulminant liver failure
Neuropsychiatric features of Wilson’s disease
asymmetric tremor excessive salivation ataxia masklike facies personality changes psychiatric abnormalities - behavioural, affective, schizophrenic, cognitive
MSK manifestations of wilson’s disease
OA like arthropathy
spine and large joints
What does Kayser-Fleischer rings look like
greenish gold brown
Familial screening of Wilson’s disease
ceruloplasmin, urine copper, LFTs
Treatment for Wilson’s disease
chelating agents:
- penacillamine, trientine
Copper absorption blocking agents
- zinc
treatment of complications
