Long case spiels Flashcards

1
Q

Heart failure/Cardiomyopathy

A
Differentials:
Idiopathic
Ischemic
Hypertension
Valve related
Myocarditis
Alcohol
Rate related
Takusubo
Post partum
Haemochromatosis/amyloidosis/sarcoidosis
Endocrinopathies: Hyperthyroidism/acromegaly
HCM/ARVC

Investigations:
CXR - pumonary odema, cardiomegaly
ECG - arrythmias, q-waves to suggest previous ischemia
ECHO - LV function, regional wall motion abnormalites, valve disease
ETT/ESE/Angiogram - to exclude ischemia
Bloods - renal, liver, iron studies, TFTs, anaemia, SFLC
Cardiac MRI
Endomyocardial biopsy

Management:
Non-pharmacological
- education
 - daily weights (greater than 2kgs over target weight, should prompt action)
- fluid restriction in severe CHF less than 1.5L
- low salt diet
- heart failure nurse
- cardiac rehabilitation
- avoid alcohol 
- smoking cessation
- vaccinations
- driving (no driving with ICD or symptomatic CHF)
- advanced care planning
Pharmacological
- diuretics - loop and thiazides (symptoms only)
- ACE-I (survival)
- B-blockers (survival)
- Aldosterone antagonist (survival)
- Digoxin (controversial, increase in mortality)
- Long acting nitrates (symptoms)
- Others - Ivabradine, angiotensin neprilisn inhibiton
Devices
- CRT (survival)
- ICD (survival benefit)
Surgery
- heart transplant
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2
Q

Parkinson’s disease

A

Key features:
Motor: Tremor, akinesia, rigidity, postural instability
Non-motor: cognitive dysfunction and dementia, psychosis and hallucinations, mood disorders, sleep disorders, autonomic dysfunction (postural hypotension/constipation/sexual dysfunction/urinary incontinence)

Differentials:
Idiopathic parkinson's disease
Dementia with lewy bodies
Atypical parkinsons syndromes (PSP, MSA, corticobasal degeneration)
Drug induced
Normal pressure hydrocephalus
Wilson's disease (young patients)
Vascular parkinsons

Investigations:
Clinical diagnosis
Confirmed with response to levodopa therapy
MRI can exclude other causes

Management:
Non-pharmacological
- education
- support groups
- physiotherapy
- speech and language therapist
- dietitian
- social worker (carer stress)
- occupational therapy
- cognitive behavioural therapy/counselling
- orthostatic advice
- increase fluid and fibre intake (constipation)
- asessment of cognition
- advanced care planning
Pharmacological
- laxatives
- consider cholinesterase inhibitor for cognitive impairment
- psychosis - only evidence is for Clozapine but quetiapine can be used (others make much worse)
- Levodopa combined with peripheral decarboxylase inhibitor = best therapy
- Dopamine agonists (risk of impulse control disorder)
- Mao-B inhibitors, anticholinergics, amantadine, COMT inhibitors
Surgery
- deep brain stimulation (subthalamic nucleus/globus pallidus)

Monitor for on/off phenomenon and dyskinesias

  • best treatment is to fractionate L-dopa - give low doses more frequently
  • add COMT inhibitor (entacapone etc.)
  • DBS early
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3
Q

Cognitive impairment

A
Differentials:
Delirium (illness, drugs, alcohol withdrawal etc.)
Mild cognitive impairment
Alzheimer
Vascular
Lewy body demenita
Frontotemporal 
Normal pressure hydrocephalus
Depression

Investigations:
Remember clock drawing and AMT in long case
Collateral history
Review medication chart
Bloods: electrolyte derangement, inflammatory markers, B12/folate, TFTs
Urinalysis, bowel chart
CT head: vascular change, NPH
Geriatric depression scale
MOCA/ACE-III
Functional assessment (important in distinguishing from MCI and dementia)

Management:
Non pharmacological
- Involve family and main supports
- EPOA
- Education
- Consider safety, communication, environment
- Manage pain
- Assess hearing and vision
- Support groups e.g Alzheimer society
- Dietitian - high risk of malnutrition
- SLT - dysphagia
- Social worker - supports, respite, carer stress
- OT and PT
- advanced care planning
- sleep hygiene

Pharmacological

  • treat precipitating causes (infections, remove offending drugs)
  • consider cholinesterase inhibitor (except for in Frontotemporal dementia)
  • anti psychotics for management of aggression/behaviours (try to avoid if possible due to increased stroke risk)
  • treat depression with SSRI’s

Palliative management in end stage disease

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4
Q

Diabetes

A
Investigations:
BSLs
HBA1c
Blood pressure, lipids
Results of retinal screening
Urine protein creat ratio
Creatinine
Management:
Type 1
- education
- medical alert bracelet
- driving
- employer supportive?
- diabetes nurse
- BSL testing at least 4x a day
- insulin therapy (usually basal bolus)
- role of carbohydrate counting, insulin sensitivy factor and insulin pumps
- unwell advice - take insulin, liquid glucose, test for ketones, present early for medical attention
- hypoglycemic education
- exercise (reduce insulin or supplement with glucose)
- regular assessment for complications (see below)
- smoking cessation
- limit ETOH
- pregnancy planning
- mental health
- screen for other autoimmune disorders
- perioperative management

Type 2

  • education
  • weight loss
  • diabetes nurse
  • testing and control
  • diet advice/alcohol/smoking
  • diet controlled, metformin, sulphonylurea, insulin
  • newer GLP-1 analogues, DPPIV inhibitors
  • statin
  • aspirin controversial (can be considered in those with more than one risk factor)
Complications
Microvascular
- nephropathy
    - 3 monthly PCR and creat
    - blood pressure control (goal less than 130/80) ACE-I
    - assess for dialysis or transplant
    - PD better for diabetics
- retinopathy
    - opthalmology every 2 years
    - panphotocoagulation vs avastin
- peripheral neuropathy
    - annual review with monofilament
    - inspect feet daily
    - 4 monthly podiatry
    - careful nail care
    - footwear
    - early medical assessment of skin ulcers
- autonomic neuropathy
   - orthostatic advice
   - screen for erectile dysfunction
Macrovascular
- PVD/CVA/CVD

Targets:
HbA1c less than 53 indicates good control
Less than 40 in a patient on insulin indicates likely hypoglycemic episodes
ACCORD trial showed increase mortality in elderly with tight control

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5
Q

Maintaining independance

A

PT - structured excercise programme to maintain strength and balance, walking aids
OT - vocational OT, equipment around the house
Driving assessments
SW - assessments for supports
Driving support agencies - driving miss daisy
Maintain good relationship with GP, possibility of home visits
Support groups - information sharing
Engaging family
Stop smoking and reduce alcohol
Screening for depression
Blisterpack for medications

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6
Q

Osteoporosis

A

History of fractures and pain
Consider secondary causes: steroids, hypogonadism, thyroid disease, chronic heparin treatment, drugs (lithium, enzyme inducing anti-convulsants) malabsorbtion, malnutrition, connective tissue disease

Investigations
Bloods: ALP, Calcium, Vit D, PTH
DEXA scan - T score less than 2.5

Management:
Smoking cessation
Alcohol reduction
Excercise - weight bearing excercise
Calcium - ideally 2 servings of calcium per day
Vit D
Bisphosphonates
Other option: Teriparatide (T score less than -3), Denosumab, Raloxefine
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7
Q

COPD

A

Non-pharmacological

  • education
  • written action plan
  • nutrition
  • smoking cessation
  • vaccination (flu vaccine - annually, pneumovax 5 yearly)
  • pulmonary rehab (education/optimising medical management, PT, psychosocial support)
  • flying advice
  • home oxygen (paO2 less than 55, or less than 60 with pul hypertension)
  • home BiPap
  • lung volume reduction surgery
  • lung transplantation
  • advanced care planning/hospice/palliative care

Pharmacological
- no evidence for prophylaxic antibiotics
- some role for macrolide as anti-inflammatory
- Step 1: Short acting bronchodilator (b-agonist, anticholinergic)
- Step 2: Add long acting bronchodilator (b-agonist, anticholinergic)
- Step 3: Add inhaled glucocorticoid (indicated for severe COPD with exacerbations)
- Consider adding tiotropium for severe COPD
Treat exacerbations - steroids, oral antibiotics, NIV

Things that improve survival:
smoking cessation and oxygen
Things that improve QOL:
pulmonary rehab - also reduces hospitalisations
Things that reduce exacerbations:
inhaled corticosteroids
LABAs
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8
Q

Falls

A
Education
- home
- footwear
- alcohol use
- diet
Consider TaiChi for balance
MDT
- rehabilitation
- OT: environment, free from clutter, rails, ramp, lighting
- PT: transfers, education around excercise daily
- SW: home alarm, medicalert bracelet
Medically
- Vision 
- Hearing
- Continence
- Hip protectors
- Osteoporosis - Vit D, bisphosphonate
- Postural hypotension
- Medication review (greater than 4 medications = bad)
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9
Q

Long-term corticosteroid use

A
side effects: MANY!!!
immunosuppression
adrenal suppression
Cushing's syndrome
hyperglycaemia and diabetes
cardiovascular disease
fluid retention
osteoporosis (>5mg/day associated with sig reduction in BMD)
osteonecrosis 
cataracts and glaucoma
psychiatric disturbance
GI bleeding
myopathy
impaired wound healing

corticosteroids have both glucocorticoid and mineralocorticoid effect:
glucocorticoid - anti-inflammatory, immunosuppression, vasoconstrictive effects
mineralocorticoid - electrolyte and fluid balance

prior to starting long-term steroids

  • lowest dose for shortest time
  • consider steroid sparing agents where possible
  • assess for infection e.g. active shingles, Hepatitis, TB
  • assess BMD (DEXA) then 1 year F/U then 2-3 yearly
  • assess baseline cardiovascular risk including lipids, HbA1c

adrenals:
- screen for adrenal suppression with early morning cortisol if suspected, if low performed short synacthen (insulin tolerance test is definitive test)

eyes:
annual ophthalmology review if > 6/12 treatment

bones (see osteoporosis speil)
greatest bone loss in first 6-12 months of treatment
adequate dietary calcium intake
Vit D supplementation
all patients on >7.5mg/day of Pred for > 3/12 are at risk
in general those > 65 should be on bisphosphonate
also consider if 15 - insulin
* adjust diabetes medications and increase BSL monitoring if steroid dose changed

GI:
consider PPI for GI protection

Education:

  • increase dose when unwell
  • don’t stop suddenly
  • inform medical professionals of long-term steroid use
  • medic-alert bracelet
  • symptoms of development of hyperglycaemia
  • test BSLs more freq and contact GP/practice nurse if change in dose if diabetic

Lifestyle:
- regular exercise, limit sugar in diet - to prevent excessive weight gain

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10
Q

Transplant

A

Pre-transplant:

  • underlying disease
  • function prior to transplant (ie. hospital admissions, requirement for home O2, LVAD, dialysis)
  • work up for transplant (cardiovascular, respiratory, CMV, Tb etc.)

Transplant:

  • Type of donor (live, deceased, related, unrelated)
  • Match of donor
  • CMV status
  • immediate operative and post operative complications
  • Duration since transplant

Post transplant:

  • immunosuppression
  • prophylaxis (viral/bacterial/fungal)
  • ongoing screening (diabetes, hypertension, skin cancers, other malignancies)
  • monitoring of graft function
  • episodes of rejection
  • monitoring for disease recurrence
  • current graft function
  • social impact of transplant (e.g displacement from home)
  • follow up at transplant unit
  • understanding of survival of graft

Immunosuppression:

  • interactions
  • drug monitoring (levels, side effects)
  • infective complications
  • importance of compliance
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11
Q

Splenectomy

A

Either elective splenectomy, emergency splenectomy or hyposplenic due to underlying disease (e.g. coeliac disease)

Vaccination pre-splenectomy (or 2 weeks after emergency procedure):
2 weeks before
- meningicoccal
- pneumococcal
- Hib
- influenza
Splenectomy patients are able to have live vaccines

Management post-splenectomy:

  • notify all medical practitoners about hyposplenism
  • medic-alert bracelet
  • seek medical attention early if unwell
  • liaise with primary care practitioner with clear instructions for maintenance of vaccinations
  • give supply of amoxicillin to take if unwell and unable to immediately access medical care
  • daily antibiotics for first 2 years (at least) after splenectomy (highest risk period) - amoxicillin or penicillin V
  • avoid travelling to countries with malaria
  • advise of high risk of infection after animal bites
  • carry splenectomy card with you at all times

Follow up vaccines:
Meningococcal - every 5 years
Pneumococcal - every 5 years
Yearly influenza vaccine

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12
Q

Cirrhosis

A

Compensated cirrhosis

  • asymptomatic, no varices/ascites
  • median survival greater than 12 y

Decompensated cirrhosis

  • symptomatic
  • development of jaundice, ascites, variceal haemorrhage or hepatic encephalopathy
  • 1 year survival 61%

Management and monitoring of complications
General:
- vaccinations
- abstinence
- avoid hepatotoxins
- weight loss
- screen for HCC every 6 months with USS

Ascites (1 y survival = 50%)

  • SAAG greater than 11 = due to portal hypertension
  • salt restriction
  • diuretics; spironolactone better than frusemide
  • large volume paracentesis

Varices (mortality 20% with each bleed)

  • endoscopy at diagnosis of cirrhosis
  • no varices = repeat 2-3 y intervals
  • varices = risk assessment; small + red wale mark or large (greater than 5mm), then prophylaxis
  • primary prophylaxis: non selective beta blocker or endoscopic variceal ligation
  • secondary prophylaxis (prev haemorrhage): both non selective beta blocker + endoscopic variceal ligation
  • acute bleeding: non aggressive resus, terlipressin, antibiotics, endoscopic banding, balloon tamponade, TIPS (transjugular intrahepatic portosystemic shunt)

Spontaneous bacterial peritonitis (mortality 20% with each episode)

  • ascitic tap = PMN greater than 250, positive micro
  • ceftriaxone
  • recur in 70%; prophylaxis with norfloxacin

Hepatorenal syndrome

  • type 1 = acute onset with associated drop in BP, poor prognosis (median survival 2 weeks)
  • type 2 = slow progression of renal impairment (6 month survival)
  • consider terlipression
  • dialysis as bridge to transplant

Hepatic encephalopathy (1 y survival 40%)

  • treat precipitant: infection, bleeding, constipation, diarrhoea, drugs, metabolic derangement
  • lactulose
  • rifaximin
  • do not protein restrict (increases mortality)
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13
Q

Chronic kidney disease

A

General management

  • avoid nephrotoxins
  • target BP 130/80 or 125/75 if proteinuria
  • ACE-I or ARB for proteinuria
  • lifestyle modifications: alcohol, smoking, exercise
  • control glucose (target HbA1c less than 53)
  • reduce drug doses as appropriate
  • monitor GFR 3 monthly

Complications

  • fluid overload: need higher doses of diuretics
  • hyperkalaemia: low K diet, avoid drugs (NSAIDs)
  • CVD: leading cause of mortality
  • anaemia (seen in Stage 3-4 CKD, typically less than 30ml/min); consider EPO if Hb less than 100, must be iron replete (ferritin greater than 200), consider inflammation, hyperparathyroidism, aim Hb 100-120
  • haemostasis: DDAVP, cryoprecipitate, blood tx
  • uraemic peripheral neuropathy
  • Ca/PO4: dietary PO4 restriction, phosphate binders (calcium containing if hypocalcaemia), activated Vitamin D, consider cinacalcet and parathyroidectomy
  • calciphylaxis: wound care, high dose oxygen, sodium thiosulphate with dialysis, increased time on haemodialysis, stop warfarin and iron

Non-pharamacological

  • diet: get a dietician involved
  • low sodium and K+ diet
  • fluid restriction
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14
Q

Common drug interactions to remember in transplant patients

A

Drugs for infection:
Azole antifungals - inhibit CYP3A4 = increase CNI levels
Macrolides (except Azithromycin) also inhibit CYP3A4
Rifampicin induces 3A4 = lower drug levels

Hypertension:
Non-dihydropyridine calcium channel blockers (Diltiazem/Verapamil) inhibit 3A4 = increase CNI levels (can be used as a CNI sparing agent)

Antidepressants:
SSRIs have mild inhibiton of 3A4 - Sertraline and Ecitalopram considered best of antidepressants

Statin:
Metabolised through CYP3A4, CNIs inhibit this and risk of developing statin myopathy and liver failure
- use pravastatin (low potency steroid and monitor LFTs and CK)

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15
Q

Alcohol

A

Guidelines
Women - no more than 10 drinks per week, no more than 2 drinks per day
Men - no more than 15 drinks per week, no more than 3 every per day
2 alcohol free days per week

CAGE questions:
Are you Concerned about your drinking?
Do you get Angry if anyone criticizes your drinking?
Do you feel Guilty about drinking?
Do you need an drink early in the morning (Eye opener)?

If no signs of alcohol abuse or dependence then patient can still have “at risk drinking”

Signs of alcohol abuse:

  • risk of bodily harm
  • relationship affect
  • role interference (Work/family)
  • run-ins with the law

Signs of alcohol dependence:

  • craving alcohol
  • loss of control (inability to cease drinking)
  • withdrawal symptoms
  • tolerance

Brief intervention

  • state drinking is medically unsafe
  • ask willingness to reduce alcohol intake
  • negotiate goal (abstience is best)
  • offer support
  • screen often
Readiness to change:
Pre-contemplation
Contemplation
Planning
Action
Maintaince

Support

  • information
  • addiction specialist (if dependance)
  • support groups (AA)
  • counselling
  • medications (e.g. Disulfiram, Naltrexone)

Treat associated disorders (nicotine dependence, medical and psychiatric)

Medical manifestations of alcohol use:

  • atrial fibrillation
  • reflux
  • macrocytosis
  • peripheral neuropathies
  • chronic pancreatitis
  • chronic liver disease
  • hypertension
  • cardiomyopathy
  • malignancy
  • wernickes (ataxia, confusion, opthalmoplegia), Korsakoffs
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16
Q

Adolescents

A

HEADS assessment

HOME

  • living situation
  • health of family members
  • family relationships
  • domestic violence/substance abuse in household

EDUCATION/EMPLOYMENT

  • schooling, doing well?, relationship with teachers
  • future plans
  • bullying
  • work

ACTIVITIES

  • peers/friends
  • hobbies
  • interests
  • religion
  • sport
  • gang/involvement with police
  • driving

DRUGS

  • smoking/alcohol/other drugs
  • drink driving
  • payment for drugs

SEXUALITY

  • sexually active
  • relationship
  • contraception
  • STD/pregnancy/termination
  • abuse

SUICIDE AND DEPRESSION
- eating disorders

Main causes of morbidity/mortality:

  • unintentional injuries (e.g. MVA)
  • alcohol and drug abuse
  • unwanted pregnancy
  • STD
  • eating disorders
  • mood disorders
17
Q

Sexual dysfunction

A

Screen for associated disorders

  • diabetes
  • alcohol use
  • peripheral vascular disease
  • cardiovascular disease
  • hypogonadism
  • depression
  • spinal cord disease

Discuss affect on relationship
Psychological contribution
Medications that could be contributing

Modify risk factors
Offer counselling
Sildenafil (1 hour before sex, PDE5 inhibitors)
Vacum devices/rings etc.

18
Q

Domestic violence

A

Types of violence:
Physical, sexual, psychological, financial, neglect

Populations to consider:
Elderly, migrants, disabled

Assess for imminent danger - police
Support networks - womans refuge, youthline etc.
Providing education and resources
Counselling 
Anger management
Social work
19
Q

Depression

A

Screening (2 questions)
In the last month have you
- felt low, depressed or hopeless?
- had little interest or pleasure in doing things?

Management:
Assess risk (suicide) -> urgent psychiatric referral
Frequent follow up
Guided self help resources, internet
Web based CBT
Cognitive behavioral therapy/interpersonal therapy
Relaxation techniques
Excercise/diet/sleep hygiene/avoid alcohol

Drug therapy

  • SSRIs recommended first line
  • Fluoxetine for children and adolescents
  • Citalopram, E-citalopram, Sertraline for those on other medicaitons as lower risk of drug interactions

Follow up

  • monitor frequently (increased risk of suicide with initiation for therapy)
  • if no response at 4-6 weeks consider compliance, increasing dose or change to alternative drug
  • duration of therapy should be at least 6 months to reduce relapse
  • when stopping decrease dose gradually, remember washout period

Refer to psychiatry service if high risk, possible other diagnosis, failed treatment, recurrent depression

20
Q

Sleep

A

Evaluating sleep
- time to bed, time to sleep, awakenings, daytime sleeps

Assess psychological - anxiety, depression
Sleep environment
Physical (OSA, heart failure, pain, restless legs, urinary frequency)

Management:
Sleep diary

A - avoid health
S - sleep/sex only use of bed
L - leave laptops out of the bedroom
E - excercise reguarly, not close to sleep
E - early rising, avoid daytime naps
P - plan for bedtime, bath or warm drink

Relaxation therapy

Drug treatment

  • avoid if possible
  • melatonin (good for adjustment e.g jetlag)
  • short acting benzos for short amounts of time if required
21
Q

Ladies, babies, transplants and immunosuppression

A
  • Fertility can be restored from 1-2 months post transplant
  • Must use effective contraception
  • Ideal time for pregnancy post transplant is after 2 years when graft function and immunosuppression is STABLE
  • Mother, fetus and graft are all at risk

Pre-conception counselling
Regular visits with obstetrician, specialist, neonatologist in high risk clinic
Vaccinations UTD - Hep B, Influenza, Pneumococcus
*Rubella should be given prior to transplant as this is a live vaccine (if not already vaccinated)

Maternal complications:
- Most common is pregnancy-induced HTN which is strongly associated with pre-conception renal dysfunction

fetal complications:

  • higher rates of prematurity, IUGR, low birth weight, perinatal infections
  • similar rates of congenital anomalies to general pop

Factors associated with good pregnancy outcomes:

  • Good general health for about 2 years after Tx
  • No graft rejection in the last year
  • Good, stable graft function
  • No acute infections that might affect the foetus
  • Maintenance immunosuppression at stable doses
  • Patient compliance with treatment and follow-up
  • Well controlled BP
  • basically everything is stable and functioning well*

Delivery

  • Vaginal delivery preferred
  • Increased steroid dose when labouring due to stress
  • Antibiotic cover if any procedures required

Drugs safe in pregnancy:
-corticosteroids, Azathioprine, Cyclosporine
Not safe in Pregnancy:
- MMF, Sirolimus, MTX
- change to alternative at least 6/52 prior to conception
Prednisone safe in BF but others not

Other considerations:

  • referral to genetics if hereditary condition (e.g. PCKD) is the reason for transplant
  • check smears UTD in all ladies
22
Q

Initiating insulin

A

in type 2 diabetes insulin should be used in conjunctive with or following

  • diet and lifestyle management
  • initiation of Metformin
  • initiation of Sulphonylurea

consider in those with HbA1c > 65% (or if other measures are not achieving a previously agreed upon target)

patient must measure BSLs for a week prior to initiating insulin
- pre meals and 2 hours after

Isophane is usually first line (intermediate acting insulin) usually once a day initially, 8-10 units
If required BD then stop sulphonylurea
Long acting insulins - Glargine, are indicated when there are concerns re hypos

doses need to be titrated up weekly

Education:
administration, storage of insulin
BSL monitoring and meter use
must continue to exercise as insulin results in weight gain
managing hypos
- education re symptoms
- take BSL - eat 6 jellybeans
- re-check after 10-15 mins and repeat until BSL > 4 then eat a more complex snack

if employed as a vocational driver and taking insulin then should be referred to diabetes clinic
other drivers should know their BSL prior to driving and carry glucose in their car in case of hypos

involve diabetes CNS

23
Q

Coeliac disease

A
Consider in those with:
malabsorption
- steatorrhoea
- unexplained iron deficiency (Courts)
- osteoporosis/osteomalacia
Other autoimmune disease

Test: must be done whilst eating gluten
tissue transglutaminase
- IgA deficiency is tested for and an IgGtTG level performed instead
If equivocal can perform HLADQ2 and DQ8 - if negative then highly unlikely to have CD

If positive serology then confirm with small bowel Bx

  • must be eating gluten
  • villous atrophy evident
Management:
gluten-free diet
replace deficiencies
seek and treat osteoporosis
can re-scope on 3 months 

symptoms resolve in weeks
histology resolves in months
tTG Abs normalise in 3-6 months

Complications:
“your spleen disappears” - P. Roberts (splenic atrophy)
T cell lymphoma (consider if enlarged spleen)
slightly increased risk of bowel Ca
ulceration of small bowel
osteoporosis

Immunise as per splenectomy

  • pneumococcus
  • Hib
  • meningococcus
24
Q

IBD general principles

A

peak age of onset 20-40 years

Symptoms:
diarrhoea +/- blood, mucous
faecal urgency, tenesmus
abdo pain/cramps
fistula, abscesses
mouth ulcers
non-bowel manifestations:
joints: arthraligias, sacrolitilitis, Ank spons
skin: erythema nodosum, pyoderma gangrenous
liver disease: autoimmune, PSC
urinary: renal stones
malabsorption
anaemia
osteoporosis/osteomalcia

10-20% have FH

Ix:
FBC, U&E, LFTs, CRP
iron, B12, folate
coeliac serology (Ddx) - anti tTG ABs
stool cultures (Ddx infectious colitis)

colonoscopy
AXR if acute pain
CT/MRI enteroclysis

signs of severe disease:

  • Severe abdominal pain + tenderness
  • Severe diarrhoea (>8/day)+/- without bleeding
  • Dramatic weight loss
  • Fever or severe systemic illness

General management of IBD:

  • Initial management of relapse
  • Recognising complications
  • Providing ongoing medication and monitoring for adverse effects
  • Providing education and support - IBD support groups, IBD nurse, stoma nurse, dietician

goals:
bring active disease to remission
maintain remission

surveillance:
colonoscopy after 8 years of disease (not required if only proctitis)
then 2-3 yearly
* annually if UC associated with PSC

25
Q

Management of UC

A

corticosteroids:

  • used in acute flares and tapered off
  • no role in maintenance

5-ASA (Pentasa)
- if proctitis only can use enemas/suppositories
SEs: blood dyscrasias

Azathioprine or 6-mercaptopurine
- check TPMT level before starting
- monitor FBC weekly then 2 weekly then monthly
- monitor LFTs monthly
SEs: leukopaenia, pancreatitis, lymphoma
Anti-TNFs: Infliximab, Adalimumab
prior to starting:
- update imms
- pap smear
- Hep B and C serology
- CXR and Quantiferon gold
SEs: infusion reaction, infections, lymphomas incl hepatosplenic T cell lymphoma, demyelinating disease, CHF

Surgery: can be curative in UC - up to 40% will require
indications:
- Lack of response or intolerance to medications
- Acute complications e.g. toxic megacolon or haemorrhage
- Precancerous or cancerous changes in the colon
* can get pouchitis after surgery

Acute severe flare:

  • fluids, correct electrolytes and anaemia
  • antibiotics
  • IV steroids - if no improvement then salvage therapy with Infliximab or Cyclosporin or surgery

if raised ALP > 3 x ULN consider associated PSC
- associated with pANCA

26
Q

Crohn’s disease

A

5-ASA
- more effective for colonic disease

Budesonide
- ileocolic disease

Azathioprine or 6-MP or MTX
- steroid sparing agents to reduce relapse

MTX
before starting
- start folic acid
- baseline LFTs, Hep B and C serology, CXR
- avoid alcohol
monitoring:
- monthly LFTs for 3 months then 3 monthly
SEs: hepatotoxicity, bone marrow suppression, interstitial pneumonitis
- contraindicated in pregnancy

TNF-alpha inhibitors

  • heals fistulas well
  • refractory disease

Severe disease flare:
3 days IV corticosteroids then either salvage therapy with Infliximab or surgery

Surgery:

  • for complications such as fistula, abscesses, obstruction not responding to medical management
  • recurrence rate of disease is unchanged by surgery

note: ileoanal pouch formation reduces fertility

27
Q

Vasculitis

A
Granulomatosis with polyangiitis
- small-medium vessels
- URT, LRT and renal
- cANCA positive
GCA
- medium vessels
- assoc with PMR
- 60-70's
- headache, jaw claudication, visual loss
Polyarteritis nodosa
- medium vessels
- multiple systems - coronaries, mesenteric (abode pain), renal, mono neuritis multiplex
- assoc with Hep B
Chung-Strauss
- small vessels
- pulmonary-renal syndrome
- asthmatic phase -> eosinophilic -> vasculitic
- peripheral neuropathy, mononeuritis multiplex
- pANCA (50%)
- tissue Bx - eosinophilia
Microscopic polyarertitis
- small vessels
- pulmonary-renal syndrome
- pANCA
- renal biopsy confirms diagnosis

General principles of management:
Investigations:
ANCA - cANCA (anti PR3), pANCA (anti MPO)
ESR typically > 70
FBC - normocytic anaemia and neutrophilia common, eosinophilia in C-S
Renal function (GPA, PAN, MPA)
LFTs
Urine - casts, dysmorphic RBCs
CXR - GPA, C-S
Biopsy - most reliable for making Dx
exclude infection, malignancy or autoimmune disease

Treatment:
agressive immunosuppression to prevent permanent injury
- steroids
- Cyclophosphamide for renal or lung disease (GPA, PAN) - need monitoring of blood count, monitoring for haematuria (bladder cancer)

Issues to discuss around:

  • long-term steroids
  • osteoporosis
  • immunosuppression and infection risk
  • HTN, diabetes
  • cardiovascular risk increased
28
Q

Scleroderma

A

establish whether limited (to elbows +/- face) or diffuse (above elbows and involving chest/organs)

SKIN: Calcinoshs, Raynaud’s, Sclerodactyly, Telangiectasia
- ischaemia of digits
JOINTS: asthropathy in rheumatoid distribution, CTS
GI: dysphagia, heartburn, diarrhoea (malabsorption, bacterial overgrowth), incontinence
RENAL: HTN, CKD, renal crisis
RESP: ILD (diffuse), Pulm HTN (limited), pleurisy
CARDIO: pericarditis, arythmies, CHF
other: erectile dysfunction, hypothyroidism

ASSESS FUNCTION

Investigations:
ESR/CRP raised
FBC - anaemia chronic disease, bleeding oesophagi’s
Iron/B12/folate - malabsorption
Renal
Anti-Scl 70 - positive in a MINORITY of diffuse
- associated with ILD
Anti-centromere AB - 70% with limited (CREST)
Ix of malasoprtion and dysphagia
- OGD, oesophageal manometry
Monitoring for ILD and pulm HTN as early Mx prevents complications
- PFTs, HRCT for ILD
- Echo, R heart cath, 6 min walk test for pulm HTN

Treatment
Raynauds:
- smoking cessation
- CCB (Nifedipine), alpha blockers, topical nitrates
- iloprost infusions for ischaemia
Oesophageal symptoms
- PPI
Malabsorption/bacterial overgrowth
- antibiotics
ILD
- may improve with Cyclophosphamide (9 months)
Pericarditis/ inflammatory myopathy, early ILD - steroids
Renal
- ACE inhibitor to manage BP aggressively to prevent crisis
Pulm HTN
- phosphodiesterase 5 inhibitors - Sildenafil
- endothelia recepto antagonists - Bosenten
- Prostanoids - inhaled Iloprost

PT - hand exercises to improve mobility
OT - aids to asses function

29
Q

Scleroderma

A

establish whether limited (to elbows +/- face) or diffuse (above elbows and involving chest/organs)

SKIN: Calcinoshs, Raynaud’s, Sclerodactyly, Telangiectasia
- ischaemia of digits
JOINTS: asthropathy in rheumatoid distribution, CTS
GI: dysphagia, heartburn, diarrhoea (malabsorption, bacterial overgrowth), incontinence
RENAL: HTN, CKD, renal crisis
RESP: ILD (diffuse), Pulm HTN (limited), pleurisy
CARDIO: pericarditis, arythmies, CHF
other: erectile dysfunction, hypothyroidism

ASSESS FUNCTION

Investigations:
ESR/CRP raised
FBC - anaemia chronic disease, bleeding oesophagi’s
Iron/B12/folate - malabsorption
Renal
Anti-Scl 70 - positive in a MINORITY of diffuse
- associated with ILD
Anti-centromere AB - 70% with limited (CREST)
Ix of malasoprtion and dysphagia
- OGD, oesophageal manometry
Monitoring for ILD and pulm HTN as early Mx prevents complications
- PFTs, HRCT for ILD
- Echo, R heart cath, 6 min walk test for pulm HTN

Treatment
Raynauds:
- smoking cessation
- CCB (Nifedipine), alpha blockers, topical nitrates
- iloprost infusions for ischaemia
Oesophageal symptoms
- PPI
Malabsorption/bacterial overgrowth
- antibiotics
ILD
- may improve with Cyclophosphamide (9 months)
Pericarditis/ inflammatory myopathy, early ILD - steroids
Renal
- ACE inhibitor to manage BP aggressively to prevent crisis
Pulm HTN
- phosphodiesterase 5 inhibitors - Sildenafil
- endothelia recepto antagonists - Bosenten
- Prostanoids - inhaled Iloprost

30
Q

Lung transplant

A
Indications:
COPD (most common)
IPF
CF
Alpha1antitrypsin
Pulmonary hypertension

Median survival:
single lung - 4.5 years
double lung - 6.5 years

Causes of death:
Early - primary graft failure (ARDS, DAD)
Late - chronic allograft dysfunction caused by bronchiolitis obliterans (occurs in 50% at 5 years)
Infection
Cancer (skin, PTLD, colon, breast)

Lung function improves over 1-3 months then stabilises
Immunosuppression is typically 3 drug regime with CNI, anti-metabolite and prednisone

Prophylaxis

  • cotrimoxazole for PCP
  • voriconazole/amphotericin B for aspergillus/fungal (not universal)

Causes of SOB in transplant:

  • infection
  • bronchial stenosis
  • tracheobronchomalacia
  • anastomotic site infection
  • underlying lung disease
  • bronchiolitis obliterans (can use azithromycin to treat)
31
Q

Heart transplant

A

Median survival - 11 years

Causes of death:

  • Graft failure
  • Opportunistic infection
  • Acute allograft rejection
  • Cardiac allograft vasculopathy
  • Lymphoma/malignancy (most frequent after 5 years)

Immunosuppression
- typically 3 drug regime but can wean steroids

Surveillence
- annual angiography or dobutamine stress ECHO for first 5 years to assess for cardiac allograft vasculopathy
then ongoing depending on risk

To treat cardiac allograft vasculopathy

  • statin
  • diltiazem (controversial)
  • sirolimus
  • PCI (not usually amenable)
32
Q

Liver transplant

A

Survival: 70% at 5y

Immunosuppression:
Typically with steroid, anti-metabolite, and CNI but steroid can be weaned

Prophylaxis

  • Cotrim for PCP
  • Acyclovir for HSV, VSV
  • Hep B - immunoglobulin and antivirals

Monitoring
- monthly LFTs

Reasons for raised LFTs post transplant:

  • rejection
  • ischemic insult
  • biliary complications
  • infections (viral heptatitis and sepsis)
  • drug toxicites and hypersensitivites

Complications:

  • primary non function of graft
  • hepatic artery stenosis
  • portal vein stenosis
  • vena cava stenosis
  • anastamotic leak
  • biliary stenosis
  • infection
  • rejection (chronic bile duct destruction)

Diseases that can recur in graft

  • hepatitis B and C
  • cholestatic diseases (PSC, PBC)