Part 2 Flashcards

1
Q

Membranous Glomerulonephritis / Nephropathy Pathophysiology / Causes

A

Primary or Secondary
Immune complexes at basement membrane causing thickening and spiking

This causes Protein leak, hypoalb, oedema and hyperlipid (Nephrotic Syndrome)

Primary: Anti PLA2R Antibodies
Secondary: Infection (hep B, Malaria, Syphilis)
MALIGNANCY: Lung and Lymphoma
Autoimmune, SLE / Rheumatoid

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2
Q

Lung Ca that causes paraneoplastic syndromes? Which syndromes?

A

Small Cell Lung Ca (Central, advanced, smoking)

  • ACTH secreting -> Cushings Syndrome (hypokalaemia)
  • ADH secreting -> SIADH and hyponatraemia
  • Lambert Eaton -> Weakness (Calcium channel)
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3
Q

canagliflozin, dapagliflozin and empagliflozin: What are they and how do they work?

A

SGLT-2 inhibitors
Inhibit sodium-glucose co-transporter 2 (SGLT-2) in the renal proximal convoluted tubule
reduce glucose reabsorption + increase urinary glucose excretion

Causes normoglycaemic Ketoacidosis
Increased UTIs
Lower limb amputation

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4
Q

Left Varicocele?

A

Consider RCC
- Compression of Left Testicular Vein
RCC: Flank pain, Haematuria and abdominal mass

Seen in older men
Associated with Von Hippel Lindau (VHL mutation autosomal dominant) CNS and eye Haemangiomas

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5
Q

Stauffer syndrome

A

Paraneoplastic Hepatic Dysfunction associated with RCC

Thought to be IL 6 Mediated

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6
Q

RCC Paranoeplastic syndrome?

A

erythropoietin (polycythaemia), parathyroid hormone (hypercalcaemia), renin, ACTH

Stauffer syndrome

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7
Q

DDP4 Inhibitors?

A

Sitagliptin, Vitagliptin.
Work by increasing GLP 1 levels
GLP causes increased insulin, reduced glucagon.

DDP4 inhibitors therefore cause no weightgain or hypoglycaemia as not effecting insulin directly.

Cause Pancreatitis!

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8
Q

GPL 1 Mimics:

A

Exenatide or Liraglitide

Cause increase insulin and decrease glucagon

Nausea and Vomiting
Pancreatitis
Cause Weight Loss

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9
Q

Renal Tubular Acidosis Type 1

A

Due to Reduced H+ Secretion into the urine at the distal convoluted tubule

Leads to a normal anion gap acidosis with hypokalaemia
Associated with hypercalcaemia and kidney stones

HYPOKALAEMIA

Sjrogrens and Rheumatoid Arthritis and Kidney Transpolant are associated with it

Treated with oral sodium bicarbonate

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10
Q

Causes of Normal Anion Gap Acidosis

A

Addison’s
Bicarbonate Losses (Diarrhoea and RTA)
Chloride Excess (IV Fluids NaCl)
Drugs (acetazolamide)

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11
Q

Causes of Increased Anion Gap Acidosis

A

Ketoacidosis (Starvation, DKA)
Uraemia
Lactic acidosis (Metformin, sepsis, Trauma)
Toxins (salicylates, Methanol, Ethylene Glycol)

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12
Q

Renal Tubular Acidosis Type 2

A

Proximal Convoluted Tubule is unable to resorb Bicarbonate leading to increased losses. Is a global problem with PCT leading to reduced resobtion of glucose, phosphate, calcium, protein, potassium etc.

HYPOKALAEMIA

Caused by Drugs and Fanconi Syndrome
Drugs include Acetazolamide, Sulfonamides

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13
Q

Renal Tubular Acidosis Type 4

A

Distal Convoluted Tubule due to reduced Aldosterone action. (Addison’s)
Associated with Hyperkalaemia but normally normal sodium (lower end)

HYPERKALAEMIA

Because aldosterone triggers sodium resorption in exchange for potassium and hydrogen, there is reduced potassium excretion, causing hyperkalemia and reduced acid excretion.

Caused by Diabetes and hypoaldosteronism (steroid use)

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14
Q

Alports Syndrome?

A

Hereditory X linked recessive Disorder of Type IV Collagen leading to Ear, Eye and Kidney problems.
A cause of Renal Failure and may require transplant.

Leads to microscopic haematuria, proteinuria, progressive kidney dysfunction, bilateral sensorineural deafness, retinitis pigmentosa, Lenticonus (protrusion of lens into anterior chamber)

Renal biopsy - splitting of lamina densa.- Basket Weave

Anti GBM antibodies - Therefore if renal transplant fails may be described as Good pastures rather than graft rejection

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15
Q

Myasthenia Gravis

A

Bimodal distrubution 20-30 F, 50-70 M.
Progressive Weakness
Auto anitbodies to Acetyl Choline Post Synaptic Receptors.
Treated with acetylcolinesterase inhibitors like noestigmine.

Can be exacerbated into crisis by Aminoglycosides, Beta Blockers, Lithium, Penacillamine, antibiotics, Gentamicin.

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16
Q

ADEM (Acute Disseminated Encephalomylitis)

A

Autoimmune demylinating disease of the central nervous system. Following bacterial or viral infection 2days - 2 months. Measeles Mumps, Rubella common causes. Multifocal neurological involvement, starting as headache, nausea vomiting, then motor, sensory, ocular motor and brainstem dysfucntion

MRI- supra and infra tentorial demylination
Steroids and IVIG is the management

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17
Q

ADEM (Acute Disseminated Encephalomylitis)

A

Autoimmune demylinating disease of the central nervous system. Following bacterial or viral infection 2days - 2 months. Measeles Mumps, Rubella common causes. Multifocal neurological involvement, starting as headache, nausea vomiting, then motor, sensory, ocular motor and brainstem dysfucntion

MRI- supra and infra tentorial demylination
Steroids and IVIG is the management

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18
Q

Typhoid

A

CAused by Salmonella Typhoid (Para Typhoid - Salmonella Paratyphoid)

Faeco oral route enertic Fever

Features:
RElative Bradycardia
Rose spots (paratyphoid) - rash on trunk
Normally it is Constipation Not Diarrhoea

Complications
Osteomylitis, Meningitis, GI Bleed, Cholecystitis

Treated with Cefotaxime

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19
Q

Actinic Keratosis

A

Multiple, crusty lesions that are pink/brown in sun exposed areas
Topical Florouracil is treatment and sun avoidance
Imiquimod also shows promise
NSAIDS

Treatment:
5 -FU Topical Florouracil cream
NSAIDs
Imiquimod

Cryotherapy and curtarage

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20
Q

Serum Ascites Albumin Gradient

SAAG

A

SAAG is calculated from Ascitic fluid. Ascitic fluid albumin is taken away from the serum albumin value. If < 11 this indicates portal hypertension

If > 11 this is another cause for Ascites

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21
Q

Low SAAG < 11 causes of Ascites

A

Indicates portal hypertension

Cirrhosis
Alcoholic hepatitis
Cardiac ascites
Mixed ascites
Massive liver metastases
Fulminant hepatic failure
Budd-Chiari syndrome
Portal vein thrombosis
Veno-occlusive disease
Myxoedema
Fatty liver of pregnancy
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22
Q

High SAAG > 11 Causes of Ascites

A
Peritoneal carcinomatosis
Tuberculous peritonitis
Pancreatic ascites
Bowel obstruction
Biliary ascites
Postoperative lymphatic leak
Serositis in connective tissue diseases
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23
Q

Facioscapulohumeral Muscular Dystrophy?

A

Autosomal Dominant Mysculodystrophy leading to symtpoms in arm and face
Onset around 20 years old

Winging of the scapular is very characteristic

Progressive but good prognosis - mild symptoms

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24
Q

Transjugular intrahepatic porto-systemic shunt (TIPPS)

A

Indications:

  • Refractory Ascites
  • Variceal bleeds
  • hepatic pleural effusions

Is a Bypass of the liver - Portal (hypertensive) blood is stented straight into the hepatic vein (and then IVC) without going through the liver.

This reduces portal hypertension however toxins then no longer are going through the liver

Contraindications:
Severe And Progressive Liver Failure - Child-Pugh Score > 12
Uncontrolled hepatic encephalopathy (exacerbated as toxins now bypassing liver)
Right Sided Heart Failure (this increases venous return therefore exacerbating peripheral oedema)
Uncontrolled sepsis
Unrelieved Biliary Obstruction

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25
Transjugular intrahepatic porto-systemic shunt (TIPPS)
Indications: - Refractory Ascites - Variceal bleeds - hepatic pleural effusions Is a Bypass of the liver - Portal (hypertensive) blood is stented straight into the hepatic vein (and then IVC) without going through the liver. This reduces portal hypertension however toxins then no longer are going through the liver Contraindications: Severe And Progressive Liver Failure - Child-Pugh Score > 12 Uncontrolled hepatic encephalopathy (exacerbated as toxins now bypassing liver) Right Sided Heart Failure (this increases venous return therefore exacerbating peripheral oedema) Uncontrolled sepsis Unrelieved Biliary Obstruction
26
Churg Strauss Syndrome?
Eosphinophilic granulomatosis with polyangiitis EGPA ANCA (pANCA) associated medium vessel vasculitis - Asthma (first phase) - Eosphinophilia (lung and blood) (second stage) - Sinusitis (polyps) - Mononeuritis multiplex (vasculitic (3rd) Stage) - pANCA (60%) Leukotriene Receptor Antagonist may precipitate Disease Steroids and immunosupression
27
Granulomatosis with Polyangitis?
Wegener's Granulomatosis - Autoimmune necrotising granulomatous vasculitis of the upper and lower airways plus kidneys - Epistaxis, sinusitis, nasal crusting (upper airway) - Dyspnoea, haemoptysis (Lower Airway) - Rapidly Progressive Glomerulonephritis - Saddles-Shape Nose Deformity - Vasculitis Rash cANCA > 90% Managed with steroids and cyclophosphamide Plasma Exchange `
28
Hypernatraemia
Dehydration Osmotic Diuresis (Hyperosmolar Non Ketotic Diabetic State) Diabetes Insipidus Excess IV saline Correct with caution - can cause cerebral oedema therefore no greather than 0.5mmol/hour concentration correction Central pontine Myelinolysis is a neurological disorder caused by rapid correction of HYPOnatraemia Presents with Paraylsis, dysphagia and dysarthria.
29
Trigeminal Neuralgia?
Electric Shock like pain on light touch of the face. In the Trigeminal region. First line treatment is Carbemazepine. Consider diagnosis if: - Opthalmic region only - Optic Neuritis - Bilateral pain - History of MS in family - No response to treatment
30
Primary Biliary Cholangitis
This is an autoimmune disorder effecting liver in middleaged women. Leads to interlobular inflammation of the bile ducts causing cholestasis and leading to cirrhosis ``` Associated with Connective Tissue Disease: Sjogren's RA Systemic Sclerosis Thyroid disease ``` Anti Mitochondrial Antibodies are 98% Anti Smooth Muscle is 30% Increased risk of Cirrohsis Osteomalacia and Osteoperosis (due to decreased Vitamin D activation by the Liver) HEPATIC ADENOCARCINOMA risk 20 x Tx: Cholestyramine, Fat Soluble Vitamin Replacement, Ursodeoxycholic acid, Liver Transplant
31
Methotrexate Myelosuppresion?
Treat with Folinic Acid
32
Cocaine Toxicity Treatment?
Start With Benzos Then Verapamil for palpitations or tachycardia AVOID Beta Blocker
33
Dermatitis Herpetiformis?
Dermatitis herpetiformis is an autoimmune blistering skin disorder associated with coeliac disease. It is caused by deposition of IgA in the dermis. Itchy blistering on extensor surfaces Dapsone and Gluten Free Diet is management IgA deposits seen on immunofluorecence
34
Autoimmune Hepatitis
Autoimmune condition seen in young females associated with HLA B8 and DR3 Three types: - Type 1 = ANA and or Antismooth Muscle (SMA) - Type 2 = Anti-liver/kidney Microsomal type 1 antibodies (LKM1) ** CHILREN ONLY** - Type 3 = Soluble Liver-Kidney Antigen present * * ONLY MIDDLE AGE** Treated with Steroids and Liver Transplantation
35
hypertrophic pulmonary osteoarthropathy (HPOA)
Paraneoplastic syndrome with squamous cell and adenocarcinoma Lung cancer which leads to tender and swollen wrists. Associated with clubbing Bronchogenic carcinoma
36
Thymoma?
Presents in `60-70 year olds. Most common tumour of anterior mediastinum. Associated with myasthenia gravis (30-40% of patients with thymoma) red cell aplasia dermatomyositis also : SLE, SIADH
37
Anti Mitochondrial Antibody
Primary Biliary Cirrhosis
38
Anti- Liver/Kidney microsomal Type 1 Antibodies LKM1
Type 2 Autoimmune Hepatitis
39
Severe Cronhs disease treatment?
Steroids and azithromycin If still agressive go to biological agents quick Inflixamab is good for severe disease- also rectal / anal disease Methotrexate for perianal disease
40
Hyperosmolar Hyperglycaemic State?
Medical Emergency and tough to manage Hyperglycaemia causing osmotic diuresis, severe dehydration and electrolyte deficienceies. Elderly T2 DM is typical HSS has higher mortality vs DKA with complications like MI, Stroke, Thrombosis, Seizures, Cerebral oedema and central pontine myelinolysis. HSS takes days therefore dehydration is more severe Hyperglycaemia causes osmotic diuresis with associated loss of sodium and potassium Severe volume depletion causes raised serum osmolarity (> 320mosmol/kg) causing hyperviscosity. Severe electrolyte losses and volume depletion but hypertonicity causes preservation of intravascular volume Symtpoms: Fatigue, weakness, lethargy, N+V Lowered conciousness, headaches, papilloedema Hyperviscosity Hypotension, tachycardia, dehydration
41
How to Diagnose HHS
Hypovloaemia Marked Hyperglycaemia Without Ketonaemia or acidosis Significan raised serum osmolality (> 320) Calculate Osmolality with (2 * Na + K) + Glucose + Urea
42
Treatment of Whipple's Disease?
IV Cefotaxime for 4 weeks Then co -trimoxazole for 1 year Biopsy with PAS staining shows positive macrophages in duodenum
43
Calculate the Anion Gap
Na + K - Cl - HCO3
44
Ethylene Glycol Toxicity?
Stage 1: Alcohol intoxication: Confusion, slurred speech dizziness Stage 2: Metabolic Acidosis with High anion gap and HIGH OSMOLAR GAP Tachycardia and hypertension Stage 3: AKI
45
Treatment of local anaesthetic toxicity?
IV lipid emulsion History of perioral parathesia, followed by tachy cardia and wide QRS interval, followed by cardiac arrest ALL shortly after a procedure being started It is due to IV or excess administration. CNS over activity then depression as lidocain blocks inhibitory pathways then blocks both inhib and activating.
46
CF Managemet
``` REgular Physio and postural Drainage High Calorie Diet Vitamin Supplementation Pancreatic Enzyme Supplements Heart and Lung Transplant ``` IF Delta F508 Mutation Orkambi (Lumacaftor and Ivacaftor) combination therapy can be used Lumacaftor increases the number of CFTR proteins that are transported to the cell surface Ivacaftor is a potentiator of CFTR that is already on the cell surface, this increased the probability that the defective channel with be open
47
Superior vena Cava Obstruction?
``` Oncological emergency Compression of SVC commonly due to lung cancers or lymphoma SOB is most common symptom Then Swelling of face, neck and arms Headache worse in the morning Dstended neck veins ``` ``` Small cell Lung Ca, Lymphoma Kaposi's Sarcoma, Breast Ca Aortic Aneurysm Mediastinal fibrosis Goitre Thrombosis ``` Dexamethasone is management Acutely Stenting In the long run, small cell = radio and chemo Non small cell = radiotherapy
48
Waterhouse Friedrichsen Syndrome?
Adrenal Haemorrhage likely secondary to TB or Minigococcal infection. Adrenal insufficiency with changes seen on CT
49
Raynaud's Disease
Primary Raynaud's Disease - Young Women under 30 with bilateral symptoms - No over rashes or abnormal history Nifedipine 1st line IV prostacycline 2nd line
50
Strongyloidiasis
Sarvae from soil contaminated with faeces migrates in the blood to the lungs. It enters the bronchial tree, causing irritation, coughed up and swallowed. Then in the duodenum and jej it invades the bowel. Asymptomatic potentially but can have diarrhoea and abdominal pain and bloating but when immunosuppressed they cause auto infection and disseminated disease This is a Larva Currens picture Pruritic, linear, urticarial rash - Intense Pruritus as worms migrate through the skin - Asthmatics immunosuppression with steroids causes paradoxical worsening of symptoms If Larvae Migrate to the lungs pneumonitis similar to Loeffler's syndrome may be triggered IverMectin and Albendazole are Treatment
51
Heparin Iduced Thrombocytopenia
HIT Immune mediated - antibodies form against platelet factor 4 and heparin These bind to platelets on the surface Then reduce platelet levels but is a PROTHROMBOTIC state 50% reduction of platelets Thrombosis Skin Allergy Anticoagulants like lepirudin or danaparoid TAKES A WEEK TO OCCUR!
52
Kearns Sayre Syndrome?
Mitochondrial Disorder presents < 20 with progressive external opthalmoplegia. Ptosis develops with horizontal gaze issues. Patients have Pigmentary retinopathy Patients get tunnel vision and night blindness Cardiac conduction defects usually develop and can cause sudden cardiac death
53
Retinitis Pigmentosa
Peripheral retina pigmentation leading to tunnel vision - Nightblindness - Tunnel Vision - Fundoscopy shows black bone spicule shaped pigment Associated with - Refsum Disease: Cerebellar Ataxia, peripheral neuropathy, deafness, ichthyosis - Usher Syndrome - Abetalipoproteinemia - Lawrence-Moon-Biedl Syndrome - Kearns-Sayre Syndrome - Alport's Syndrome
54
Facioscapulohumeral Muscular Dystrophy?
Autosomal Dominant Mysculodystrophy leading to symtpoms in arm and face Onset around 20 years old
55
Trientine?
Copper Chelating used to treat Wilsons
56
Low Caeruloplasmin?
Wilsons
57
Kayser-Fleischer Rings?
Green brown deposits at the peripheries of the Iris | Due to deposits in Descement Membrane
58
ATP7B gene defect?
Chromosome 13 Wilson's disease Autosomal Recessive
59
Wilson's Disease?
Copper Accumulation due to defective Liver Deposits in Liver- causing failure Deposits all over body causing neurological signs (basal Ganglia), Kidney leading to Fanconi Syndrome and Type 2 RTA, Haemolysis and Blue Nails Cornea - Kayser-Fleischer Rings
60
Wilson's Disease Diagnosis?
``` Low Caeruloplasmin Low Total Copper (Paradoxical) High Free Serum Copper Kayser-Fleischer Rings on Slit Lamp Examination Increased 24 hour urinary collection ```
61
Present infection vs Past in Antibodies?
``` IgM = Ig Mow (NOW) IgG = Ig Gone (Past) ```
62
Cocaine Toxicity Treatment?
Start With Benzos Then Verapamil for palpitations or tachycardia AVOID Beta Blocker
63
Dermatitis Herpetiformis?
Dermatitis herpetiformis is an autoimmune blistering skin disorder associated with coeliac disease. It is caused by deposition of IgA in the dermis. Itchy blistering on extensor surfaces Dapsone and Gluten Free Diet is management IgA deposits seen on immunofluorecence
64
Management of Fibro Myalgia
explanation aerobic exercise: has the strongest evidence base cognitive behavioural therapy medication: pregabalin, duloxetine, amitriptyline When starting Medication, start a new one for 4 weeks. Then assess response. If no response, stop and trial a different medication.
65
hypertrophic pulmonary osteoarthropathy (HPOA)
Paraneoplastic syndrome with squamous cell and adenocarcinoma Lung cancer which leads to tender and swollen wrists. Associated with clubbing
66
Thymoma?
Presents in `60-70 year olds. Most common tumour of anterior mediastinum. Associated with myasthenia gravis (30-40% of patients with thymoma) red cell aplasia dermatomyositis also : SLE, SIADH
67
Erythema ab igne?
Caused by infra red radiation (open fire) | Causes squamous cell skin cancer
68
Tuberculosis Meningitis?
Longer Prodrome than bacterial meningitis May be a few weeks unwell with behaviour change Can get cranial nerve palsy (like malignant meningitis) Lymphocytes seen in CSF Low Low Glucose < 50% High Protein > 1 No organisms will be seen
69
Severe Cronhs disease treatment?
Steroids and azithromycin If still agressive go to biological agents quick Inflixamab is good for severe disease- also rectal / anal disease Methotrexate for perianal disease
70
Whipple's Disease?
Whipple's disease is a rare multi-system disorder caused by Tropheryma whippelii infection. It is more common in those who are HLA-B27 positive and in middle-aged men.
71
Whipple's Disease Symptoms?
Features malabsorption: diarrhoea, weight loss large-joint arthralgia lymphadenopathy skin: hyperpigmentation and photosensitivity pleurisy, pericarditis neurological symptoms (rare): ophthalmoplegia, dementia, seizures, ataxia, myoclonus
72
Treatment of Whipple's Disease?
IV Cefotaxime for 4 weeks Then co -trimoxazole for 1 year Biopsy with PAS staining shows positive macrophages in duodenum
73
TCA overdose?
Anticholinergic side effects first: Dry eyes, blurred vision, sinus tachycardia, nausea, dilated pupils Then develop: - Cardiovascular instability - ECG: sinus tachy, wide QRS and prolonged QT - Develop Metabolic Acidosis - Seizures / Coma Widening QRS > 100 = Seizure prone, 160 = Ventricular arrythmias
74
Management of TCA overdose?
IV bicarbonate - first line in hypotension and arrythmias Indications = wider QRS or Ventricular Arrythmia ``` Avoid class 1 a and class 1 c antiarrythmics - Flecanide as prolong depolarisation Class III drugs like amiodarone avoid due to prolonging the QT interval ``` IV lipid emulsion can be used Dialysis is ineffective
75
Paragonimus westermani?
``` This termatode (fluke) infection of the lung presents like TB Prolonged history of productive cough, brown in sputum ? HAemoptysis but not. Then fevers ++ ``` The patients bloods will reveal an eosinophilia and treatment is the Praziquantel
76
Superior vena Cava Obstruction?
``` Oncological emergency Compression of SVC commonly due to lung cancers or lymphoma SOB is most common symptom Then Swelling of face, neck and arms Headache worse in the morning Dstended neck veins ``` ``` Small cell Lung Ca, Lymphoma Kaposi's Sarcoma, Breast Ca Aortic Aneurysm Mediastinal fibrosis Goitre Thrombosis ``` Dexamethasone is management Acutely Stenting In the long run, small cell = radio and chemo Non small cell = radiotherapy
77
Optic Neuritis
Unilateral decreased visual activity over hours and days Poor colour vision - Ischihara plates reduced score Pain worse on eye movement Relative Afferent Pupillary Defect Central Scotoma IV steroids high dose initially Recovery is a month MRI - if > 3 white matter lesions - 50% 5 year risk of developing MS Causes inclulde MS DM Syphillis
78
Allergic Bronchopulmonary Aspergillosis?
Allergy to Aspergillus spores. Often in exam there is a history of bronchiectasis and Eosinophilia Features: Bronchoconstriction- wheeze, cough, ? prev diagnosis of asthma ? better with bronchodilators/ asthma treatment Bronchiectasis ``` Eosinophillia Flitting CXR changes RAST positive test to Aspergillus Postive IgG precipitins Raised IgE ``` Steroids Itraconazole is second line
79
Theophylline?
Like caffeine is naturally occuring methylxanthines Bronchodilator used in COPD and Asthma ? phosphodiesterase inhibitor increasing cAMP but not entirely known how it works Poisoning: - Acidosis - Hypokalaemia - Vomiting - Tachycardia - Arrythmias - Seizures - hyperglycaemia - Increased Myocardial Contractility Gastric Lavage < 1 hour Activated Charcoal Charcoal haemoperfusion > haemodyalisis
80
Coombs Test?
Direct Coombs Test- used to detec Immunohaemolytic Anaemia Take Blood of patient and add Coombs Reagent (Anti Auto Antibodies). This causes agglutination if there are auto antibodies present. Also used in haemolytic disease of the newborn Indirect Coombs Test Prenatal Screening of mother for Rhesus Antibodies Take Mother blood- take out the Rhesus Antibodies Then add Rhesus positive RBCs (to cause antibodies to attach to antigens) then add Coombs reagent (antiautoantibodies) This causes again RBC agglutination.
81
Autoimmune Haemolytic Anaemia?
RBC haemolysis secondary to auto antibodies targeting RBCs causing destruction Idiopathic Medication Underlying Disease Process RBCs usually 120 day lifetime. ``` Extrinsic type of haemolytic anaemia Haemolysis occurs in in liver/spleen therefore extra vascular Warm is more common type (> 37 degrees) Cold Rare (0-10degrees) ``` Warm = IgG IgG bind to Rh Antigen on RBC and cause antibody dependent cell mediated cytotoxicity. Macrophages, neuts, t cells and NK cells cause haemolysis Cold = IgM IgM bind to L I or P antigens. Then compliment pathway causes breakdown in the liver through Membrane attack complex (MAC). This can also occur in introvascularly causing raynaud's
82
Haemolysis signs
``` Increased Reticulocytes Normocytic anaemia Lactate Dehydrogenase levels raised Hb --> Unconjugated Bilirubin increased Haptoblobin levels decrease Haemosiderinuria - Damage kidneys and renal insufficiency ``` Direct Coombs test detects auto antibodies Warm C3D and IgG positive Cold C3D only
83
Causes of Warm Haemolytic anaemia?
``` Idiopathic Viral infections SLE Lymphomas Leukemia Penicillin and Cephalosporins ```
84
Cold Haemolytic anaemia causes?
Chronic - Leukaemia and Lymphomas Acute - Viral Pneumonia, Mycoplasma, Infectious Mononucleosis
85
Haemolytic Crisis
``` Bounding heart rate SOB Multiorgan failure Jaundice Fatigue Hepatosplenomegaly ```
86
Treatment of Autoimmune Haemolytic Anaemia?
Warm - Steroids and splenectomy | Cold - none required
87
Latent Autoimmune Diabetes of Adulthood?
Subtype of Diabetes in which patients present with phenotypic features of Type 2 DM whilest displaying the prescence of makrers of autimmunity ( Anti GAD antibodies) 30-40 year olds Oral hypoglycaemic agents are used B cell function may decline and may require progressive insulin
88
BTS guidelines for lung Nodules?
Nodule < 5mm or clearly benign or unsuitable for treatment - discharge Nodule 5-6mm CT in 1 year Nodule > 6-7mm - 3 months CT Nodule > 8 mm and Low Risk - 3 month CT Nodule > 8mm and high risk (via Brock Model) - CT PET IF CT PET shows high uptake - Biopsy
89
Secondary Pneumothorax
If patient > 50 and pneumothorax rim is > 2cm OR PATIENT IS SYMPTOMATIC = Chest Drain If patient > 50 and pneumothorax rim is 1-2cm - Aspirate If fails- still > 1 cm or symptomatic = Chest Drain If patient > 50 and pneumothorax rim is < 1 cm = admit and oxygen
90
Primary Pneumothorax
Primary Pneumothorax < 2 cm and not SOB = discharge If > 2cm or SOB = Aspirate (if fails - chest drain) Patients should be advised to stop smoking
91
Lights Criteria
Use when protein 25-30 > 30 is Exudate, < 30 is Transudate. Lights used for inbetween Lights: if one of the following true then likely Exudate Pleural fluid protein / Serum protein = >0.5 Pleural fluid LDH / Serum LDH = > 0.6 If Pleural fluid LDH is 66% of normal range of serum LDH
92
Viridans sterptococcus
Risk Factor is poor Dentition
93
Membranous Glomerulonephritis
Commonest type of glomerulonephritis 3rd most common cause of ESRF BM is thickened with subepithelial electron dense deposits. Creating a spike and dome appearance Idiopathic is due to antiphopholipase A 2 antibodies Drugs include Penicillamine, NSAIDs and Gold SLE Thyroiditis and rheumatoid Management: ACEi or ARB These reduce proteinurea and improve prognosis Then Immunosupression Steroids + Cyclophosphamides is often used Consider Anticoagulation
94
Prognosis of Membranous Glomerulonephritis
1/3 Remission 1/3 Protein urea 1/3 ESRF Good prognosis : -females Young presentation Asymptomatic
95
McArdle Disease?
Glycogen Storage Disease Myophosphorylase deficiency or glycogen storage Disease V Presents in adolescence and presents with exersis in tolerance, cramps and weakness. Unfortunately chronic fatigue is often a mysdiagnosis Autosomal Recessive - No venous blood lactate rise on exercise - Muscle biopsy shows elevated glycose concentration and muscle phosphorylase deficiency - elevated CK and Myoglobinuria Forarm muscle exercise testing or genetic testing is diagnositic Mx: Avoidance of low carb diets and low intensity aerobic exercise
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Gaucher disease
Lipid Storage Disease Genetic Disorder in which glucocerebroside accumulates in cells and organs Most common lipid storage disorder - Easy Bruising - Fatigue - Anaemia - Thrombocytopenia - Hepatosplenomegaly ASEPTIC necrosis of the femur Deficiency of glucocerebrosidase Leading to glucocerebroside accumulations in macrophages and therefore: spleen, liver, kidneys lungs brain and bone marrow Commoner in Ashkenazi Jews
97
Von Girkes Disease
Glycogen Storage Disorder Glucose - 6 - Phosphatase deficiency Hepatic Accumulation of Glycogen Leads to Hypoglcyaemia, Lactic Acidosis and Hepatomegaly
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Pompe's Disease
Glycogen Storage Disorder Lysosomal Alpha 1, 4 Glucosidase Deficiency Cardiac, Hepatic and Muscle Glycogen accumulation. Features include cardiomegaly
99
Cori Disease
Glycogen Storage Disorder Alpha 1, 6 Glucosidase Deficiency Hepatic and Cardiac Accumulation - leading to muscle hypotonia
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Dual immunomodulator and biological therapy in Crohns has increased risk compared to biological alone?
Increased risk of non melanoma skin cancer
101
Infliximab risks
``` Hypersensitivity CCF Aplastic Anaemia Reactivation of TB Skin cancer (in combination with azothiaprine) ```
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Hypokalaemic Periodic Paralysis
Rare Autosomal Dominant disorder with episodes of paraylsis typically at night Mutation in muscle voltage gated calcium channels Can be precipitated by carb meals History of exercise Life long Potassium replacement
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Indications for Plasma exchange in ANCA associated Vasculitis?
Severe Active Renal disease (Cr > 350) Pulmonary haemorrhage Concurrent Anti - GBM Auto Antibody disease
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Indications for plasma exchange?
Guillain Barre Syndrome Myasthenia Gravis Good Pastures syndrome ANCA positive vasculitis (pulm haem, acute renal failure) TTP/HUS Cryoglobulinaemia Hyperviscosity syndrome (2ndary to myleoma)
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Complications of plasma Exchnage?
``` Hypocalcaemia Metabolic Alkalosis Systemic Medication removal Coagulation factor depletion Immunoglobulin depletion ```
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Cryoglobulinaemia
This is the presence of circulating proteins which precipitate in the cold. Commonly associated with hepatitis C infection. Three types 1 - monoclonal (25%) 2 - Mixed mono and poly (usually rheumatoid factor) (25%) 3 - Polyclonal (usually rheumatoid factor ) (50%) Type 1: - IgG or IgM - Multiple Myeloma - Waldenstrom Macroglobulinaemia Type 2: mixed monoclonal or polyclonal (usually Rhematoid Factor) Associated: Hep C, Sjogren's, Lymphoma and Rehumatoid Arthritis Type 3: Polyclonal - usually with Rheumatoid factor RA and Sjogrens Symptoms: - Raynaud's only seen in type 1 Cutaenous- vascular purpura, distal ulceration and ulceration - Arthralgia - Renal involvement (diffuse Glomerulonephritis) Tests: Low complement (especially c4) High ESR Immunosuppression and plasmapheresis Can cause a peripheral neuropathy from small vessel vasculitis- sensory or sensory motor peripheral neuropathy
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Fabry's disease:
X-linked lysosomal storage disorder that causes a painful peripheral neuropathy due to deposition of glycosphingolipids within small sensory fibres. (nerve conduction normal as large fibres uneffected)
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Chronic inflammatory demyelinating polyradiculoneuropathy CIDP
This is prominently motor neuropathy often affecting proximal and distal muscles. There is sensory involvement with vibration and proprioception. Show Conduction slowing reflecting demyelination rather than amplitudes which suggests axonal loss
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Demylinating peripheral neuropathy (slowing of conduction on conduction studies)
Guillain Barre Syndrome CIDP (Chornic inflammatory Demyelinating polyradiculoneuropathy) Amiodarone Hereditary sensorimotor neuropathies type 1 Paraprotein neuropathy
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Axonal Pathology
``` Alcohol DM Vasculitis B12 Hereditary sensorimotor neuoropathy type 2 ```
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C5, 6
Deltoid Biceps Brachioradialis
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C5, C6, C 7
``` Serratus Anterior (paraylsis - winging of scapula) Triceps ```
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C8
Finger Flexors
114
Stills Disease
A Systemic Inflammatory condition of unkown aetiology but often thought to be secondary to an infectious trigger on the background of a genetic predisposition. Presents in young adults 15-25 yo peak. Fever and new non pruritic rash Basically - graham Nash with A rash Joint pain not swelling All negative screens GIVE NSAIDS unlike Graham Nash
115
How to diagnose Still Disease?
Yamaguchi criteria PAtients need all major criteria and 2 minor Major: Fever 39 degrees > 1 week Arthralgia > 2 weeks Non pruritic or maculopapular rash Lecuocytosis Minor: Sore throat, lymphadenopathy, hepatomegaly or splenomegaly, LFT derangement, ANA negative and rheumatoid factor negative
116
Stills Disease Fever, Rash and other key features
Tends to spike daily or twice daily Rash is salmon coloured on trunk or the soles, palms and face Knees and wrists are common joint involved Ferritin rises are characteristic as well
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Stills Disease Treatment?
NSAIDS NSAIDs for fever joint pain and serositis Then Steroids or D MArds
118
Superficial Thrombophlebitis
Inflammation associated with thrombosis of one of the superficial veins (usually long saphenous vein of the leg. 20% of superficial thrombophlebitis have underlying DVT and 4% will progress to DVT if untreated. Treatment with oral NSAIDs, stockings and Prophylactic dose Enoxaparin for 30 days as higher risk of DVT
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C-telopeptide
Sensitive marker of increased bone turnover observed in Pagets disease and useful in monitoring disease progression or treatment efficacy
120
Paget's disease
Increased but uncontrolled bone turnover Osteoclastic disorter Increased osteoclastic resorption and then increased osteoblastic activity UK - 5% Older man with pone pain and isolated raised ALP ALP raised Calcium and phosphate are normal PINP (procollagen type 1 N-terminal propeptide), serum C telopeptide, urinary N telopeptide and urinary hydroxyproline are raised These are all markers of bone turnover Indications for treatment: Bone Pain, Skull or long bone deformity, fracture, periarticular pagets Treated with bisphosphonates
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Complications of pagets
``` Deafness Bone Sarcoma 1% of patients Fractures Skull Thickening High Output Cardiac Failure ```
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Acute intermittent porphyria
Rare Autosomal Dominant condition with a defect in porphobilinogen deaminase - biosynthesis of heam. Toxic accumulation of dela aminolaevulinic acid and porphobillinogen Abdominal pain with neuropsychaiatric symptoms in a 20-40 year old female (Christie) Classical presentation is a combination of abdominal, neurological and psychiatric symptoms: - Abdominal pain and vomiting - Motor Neuropathy - Psychiatric - depression / acute agitation - Hypertension and Tachycardia
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Diagnosis of acute intermittent porphyria
Urine turns red on standing Raised urinary porphobilinogen Assay of red cells for pophobilinogen deaminase
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Typhus
Rickettsial Disease Can cause widespread vasculitis - Fever, Headahce - Blakc Eschar at site of inoculation - Maculopapular or vasculitic rash Deranged clotting, renal failure and DIC, low platelets Interstitial infiltrates
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Treatment of Rickettsia disease
Doxycycline | Chloramphenicol
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Prussian Blue
Used to treat Thallium Poisoning
127
Thallium Poinsoning symptoms?
``` Painful peripheral neuropathy Abdominal pain Diarrhoea and vomitting Ataxia Sepia tinge to vision Reduced visual acuity Cranial nerve palsy Minimal body hair / alopecia Glossitis Mood issues ``` Occupation: Electroplating Treated with Prussian Blue TRIAD: Fluctuant mood, painful distal parasthesia and Alopecia = Pathognomonic
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Indication to leave a chest drain in in infective pleural effusion?
pH < 7.2 | Purulent aspirate
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Kartagener's Syndrome
Primary Ciliary Dyskinesia - Bronchiectasis - Recurrent sinusitis - Otitis Media - Subfertility Dextrocardia / Sinus Invertus Patients are tested by putting sweet in the nose. If they don't tase after 20 mins then they have ciliary dyskinesia
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Bronchiectasis Causes:
Infective: - TB - Measles - Purtussis - Pneumonia CF Immune Deficiency: - IgA - Hypogammaglobulinaemia Allergic Bronchopulmonary Aspergillosis Ciliary Dyskinetic Syndromes Kartageener's syndrome Youngs syndrome Yellow Nail Syndrome
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Toxoplasmosis in HIV
Multiple Lesions Ring or nodular enchancement Thallium SPECT negative 50% of cerebral lesions in patients with HIV Treatment is with Sulfadiazinde and pyrimethamine
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Primary CNS Lymphoma
30% of cerebral lesions in patients with HIV EBV association Single homogenous enhancing lesion Treatment with steroids and methotrexate (chemo) and consideration for sugery
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Other Neurological differentials in HIV
- Toxoplasmosis - CNS lymphoma - Encephalitis: CMV or HIV itself Can also be HSV CT shows oedematous brain ``` - Cryptococcus Most common fungal infection of CNS - Headache, fever, seizures, focal deficit CSF - high opening pressure India Ink Test Positive ``` - Progressive Multifocal Leukoencephalopathy PML Widespread demyelination Due to JC virus - behavioural changes, speech, motor, visual impairment Single or multiple lesions on CT w/o mass effect and don't usually enhance MRI shows demylination Aids Dementia Complex - Caused by HIV virus itself - behavioural changes and motor impairment - CT cortical and subcortical atrophy
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Pneumothorax - when can you fly again?
1 week following resolution of chest x ray
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Inferior MI
II, III and AVF | Right Coronary Artery thrombus
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Anterior MI
V1-V4 | Left Anterior Descending
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Lateral MI
V5, 6 and I | Circuflex artery
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Malaria Falciparum
MOST SEVERE Malaria Causes Cytoadherence to block spleen blood flow and cause ischemic damage Need to decide if severe or not Then need to highlight any complications Severe: - Schizonts (last stage before lysis of RBC and increased viral load) - Parasitaemia > 2% - Hypoglycaemia - Acidosis - Temp 39 - Severe Anaemia - Any complications Complications: - Cerebral Malaria - seizures and coma - Acute Renal failure (blackwater fever) - Acute Respiratory Distress - Hypoglycamia - DIC SEVERE DISEASE: Treat with IV Artesunate > 10% parasite = EXCHANGE TRANSFUSION IF shock - then treat with antibiotics as well For non severe disease: artemisinin based combo therapys are recommended
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Sickle cell immunity to Malaria = how does it work?
No duffy antigen | Therefore Plasmodium Vivax cannot enter cells
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Other immunity to Malaira?
Thalassemia and G6PD increase likelyhood that infected RBC will dye from oxidative stress This gives a level of immunity
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P Malariae Fever?
Quartan Fever- 72 hours
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P vivax and P ovale fever?
Tertian Fever- 48 hours
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P Knowlesi
Fever every 24 hours
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P Falciparum
Fever varies - Malignant Tertian Fever
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Patients that don't benefit from Lung Reduction surgery in COPD?
Non Upper Lobe empysema | High Exercise Capacity
146
Microscopic Colitis
Occurs in association with PPI use History of chronic watery diarrhoea with increased frequency Statins and SSRIs can also cause it Colonoscopy and biopsy is the way to confirm the diagnosis
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Anti TTG antibodies
Non invasive confirmation of coeliac disease | Where negative, but still clinically coeliac, can go onto upper GI endoscopy and biopsy
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Faecal calprotectin
Screening test for active Crohn's and UC
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Facal Elastase
Used to evaluate pancreatic insufficiency
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Carcinoembryonic antigen?
Used as Tumor marker for colorectal cancer
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Inclusion Body Myositis
Cause of Myopathy Associated with cytoplasmic inclusions on mm biopsy Older males with proximal and distal muscles Quads fingers and wrist flexors Raised CK
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Drug Induced Lupus
Anti histone antibody positive ANA postiive 100% Anti dsDNA negative
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Drugs that cause Drug Induced Lupus
``` Procainamide Hydralazine Isoniazid Micocycline Phenytoin Penicillamine ```
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Chickenpox infection in adults
Primary infection with varicella zoster virus Shingles is the reactivation of the dormant virus in dorsal root ganglion ``` Highly infectious Respiratory spread Can be caught of shingles patient 4 days before rash, 5 days after day 1 of rash 10-21 day incubation ``` ``` Symptoms: - fever - itchy rash starting on head/trunk before spreading - Macular then vesicular Systemic upset ``` Tx: keep cool Trim nails 5 days off after rash started IVIG for immunocomprimised or newborns Common complication is bacterial infection NSAIDS increase this risk Can develop cellulitis Can also develop invasive group a strep infection This can be Nec Fasc - out of proportion pain for symptoms of erythematous rash Other complictions include - pneumonia - Encephalitis - Disseminated haemorrhagic chicken pox - Arthritis
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Rapidly progressive Glomerulonephritis
Term for rapid loss of renal function assciated with formation of epithelial crescents - Good Pastures Syndrome - Wegener's Granulomatosis - SLE - Microscopic polyarteritis PRESENTS AS NEPHRITIC SYNDROME - red cell casts, proteinuria, hypertension, oliguria Treated with immunosuppression and plasmapheresis Anti coagulation Type 1 - Anti-GBM antibody (Goodpasture Sydrome) - linear on immunofluorecence Type 2 - Immune complexes (post strep, Lupus, IgA, HSP) Granular on immunofluorecence Type 3 - Pauci - Immune (ANCA positive) No immunofluorecence
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Cardiac Amyloidosis
ECG shows low voltage complexes with poor R wave Progression in the chest leads This is common in Myeloma
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Progressive Multifocal Leukoencephalopathy
is an oppurtunistic infection by the JC virus that only occurs in patients who are immunocomprimised. Natalizumab has a 2.1 in 1000 risk of developing it. Multifocal demyelination Presents with sub acute behaviour change, speech, motor and visual impairment
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Multiple Slcerosis Treatment:
Reduce the frequency and duration of relapses - No cure Acute replase: High dose steroids - shorten the relapse but not recovery DMARDS beta interferon reduces relapse rate by 30% Criteria for beta interferon: - Relaps, Remiting disease: 2 r in past 2 years and able to walk 100m - Secondary progressive disease: 2 r in past 2 years and able to walk 10 m Glatiramer acetate: Immune decoy Natalizumab: alpha 4 and beta 1 integrin antagonsit found on leucocytes Fingolimod ``` Other issues: Fatigue: - Exclude anaemia / hypothyroid Treat with amantadine mindfullness CBT ``` Spasticity: Baclofen and Gabapentin Bladder Dysfunction - urgency, incontinence and overflow - Guidelines say get US first - If significant residual volume - self catheterise - if no signficant residual volume - anticholinergics Oscillopsia (visual fields oscillate) - Gabapentin
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If about to treat malaria what should you check?
G6PD deficiency | Primaquine, Cipro and sulphonamides cause haemolysis
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Lansoprazole interactions
hyponatraemia Hypomagnesmia Osteoperosis Microscopic colitis Increased C.Diff Reduced HIV antiretroviral efficiency (atazanavir, Eviplera) Reduced Methotrexate clearance (omeprazole and aspirin)
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Donepezil adverse effects
Is a cholinesterase inhibitor used to treat mild to moderate dementia It has vagotonic effects that lead to bradycardia and heart block
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Pharmacological management of dementia?
1st line is acetylcholinesterase inhibitors: Donepezil, glantamine and rivastigmine (as used in MG) This is for Mild to moderate Alzheimers 2nd Line is Memantine (NMDA receptor Antagonist) - Add on or monotherapy severe Alzheimers - start if intollerant of first line in moderate disease
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Hyperaldosteronism treatment
Primary Hyperaldosteronism Commonly caused by an adrenal adenoma (CONNS) However, bilateral adrenal hyperplasia is actually now 70% of cases Treat conns with surgery but BAH with spironolactone, so differentiating is imporant Signs and symptoms: - Hypertension (due to high Na) - Hypokalaemia (can present with muscle weakness) - Alkalosis Aldosterone/renin ratio is the first line investigation High aldosterone with low renin levels should be seen Then, high resolution CT with adrenal vein sampling to see if bilateral or unilateral sources of aldosterone excess If unilateral - surgery If bilateral adrenocortical hyperplasia - spironolactone
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Paraxysmal Noctural Hemoglobinurea
Dark Urine, Haemolytic Anaemia and Thrombosis Due to an acquired genetic mutation in the PIGA gene. This codes for GPI - the anchor protein to CD 55 and CD 59 These are both Complement Regulatory Proteins This means they help the complement system (alternative pathway that reacts to bacterial endotoxins and forms the MAC) to differentiate from self and non self CD 55 and CD 59 are found on RBC, Neutrophils and Platelets Without GPI then there is no CD 55 and CD 59 This causes unregulated complement activation and therefore Intrinsic, intravascular, Haemolysis There is haemolysis throughout the day Then at night urine is concentrated so then in the morning it looks like there is increased blood in urine hence the name Complications are thrombosis (commonest cause of death), Irone Def Anaemia (haemoglobin loss) and AML
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Diagnosis of PNH
HAM test positive | Flow Cytometry is gold standard
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Treating PNH
Iron, RBC transfusion Ecluzimab - binds to C5 and therefore stops MAC formation Stem cell transplant
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Ascarasis
Due to infection with roundworm Ascaris lumbricoides Infections begin in gut following ingestion, then penetrate duodenal wall to migrate to lungs, coughed up and swallowed, cycle begins again Diagnosis is made by identification of worm or eggs within faeces Treatment is with mebendazole May have loffler's syndrome then GI history Contrast seen in worm on bareum enema
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Myoxedmea Coma
Give IV T3 and T4 + Steroids Presents with hypothermia, low GCS, bradycardia, hypotension Need to rule out addisons as well
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Hypercalcaemia management
The initial management of hypercalcaemia is rehydration with normal saline, typically 3-4 litres/day. Following rehydration bisphosphonates may be used. They typically take 2-3 days to work with maximal effect being seen at 7 days Other options include: calcitonin - quicker effect than bisphosphonates steroids in sarcoidosis 1. Fluids 2. Bisphosphonates 3. CALCITONIN Steroids for Sarcoidosis Think about Furosemide in specialist circum Loop diuretics such as furosemide are sometimes used in hypercalcaemia, particularly in patients who cannot tolerate aggressive fluid rehydration. However, they should be used with caution as they may worsen electrolyte derangement and volume depletion.
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Indication for Mitral Valve Replacement
1. EF < 60% 2. Left ventricular end systolic diameter > 40mm (LV Dilation or Hypertrophy) 3. New onset of AF or 4. Pulmonary Hypertension > 20mmHg If not 6 monthly ECHO No need for prophylaxis (only in prostetic repair or replacement)
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Diagnosis of Diabetes?
If the patient is symptomatic: fasting glucose greater than or equal to 7.0 mmol/l random glucose greater than or equal to 11.1 mmol/l (or after 75g oral glucose tolerance test) This needs to be demonstrated twice in an aysmptomatic patient HbA1c of > 48mmol/L (6.5%)
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Diagnosis of Prediabetes?
Prediabetes is split into 2 dependent on the Diagnostic Test used or abnormal Impaired fasting Glucose: A fasting glucose greater than or equal to 6.1 but less than 7.0 mmol/l implies impaired fasting glucose (IFG) IFG: Fasting Glucose 6.1-7.0 mmol/L Impaired Glucose Tollerance OGTT 2-hour value greater than or equal to 7.8 mmol/l but less than 11.1 mmol/l
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HbA1c
> 48mmol/L = Diabetes (6.5%) If < 48mmol/L = need fasting glucose or OGTT as this doesn't exclude diabetes
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Radioiodine therapy following CT Contrast?
Wait 8 weeks before starting
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Haemangioblastomas
Cause a paraneoplastic syndrome where they release EPO This leads to a very high Hb Seen in Von Hipple Lindau syndrome Have a very good prognosis if they are ameanable to surgical resection
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Combined Pulmonary Fibrosis and Emphysema
CPFE is characterized by exertional dyspnoea, upper-lobe emphysema and lower-lobe fibrosis, preserved lung volume and severely reduced capacity of gas exchange. The preserved lung volumes are thought to arise because of the counterbalanced effects of hyperinflation from emphysema and the restrictive effects of pulmonary fibrosis. Both mechanisms lead to reduced gas exchange, hence the significantly reduced DLCO.
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Idiopathic Pulmonary Fibrosis
Ongoing repair process of having excess collagen / scar tissue in the interstial lung tissue Type 2 pneumocytes over preliforate and too much collagen is deposited by myofibroblasts Reduced myofibroblast apopotosis Reduced blood oxygenation as distance between alveolai and capillary is increased Excess collagen also causes increased lung stiffness, making ventilation more difficult This is restrictive lung disease, interstitial lung disease. - Reduced Total Lung Capacity - Reduced Force Vital Capacity - Reduced Force Expitory Volume in first second Cysts plus thick walls - honeycombing Progressive therefore symptoms progress overtime Clubbing Respiratory failure Investigation Chest CT Spirometry Treatment is supplimental oxygen and lung transplant Old age, Male, Tobacco smoker Life Expectancy from Diagnosis is 3-4 years
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LTOT in COPD
Assessment is done by measuring arterial blood gases on 2 occasions at least 3 weeks apart in patients with stable COPD on optimal management. Offer LTOT to patients with a pO2 of < 7.3 kPa or to those with a pO2 of 7.3 - 8 kPa and one of the following: secondary polycythaemia peripheral oedema pulmonary hypertension Don't offer to smokers who don't want to give up Assess risk of falls and fires
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Protein C deficiency
Protein C deficiency Protein C deficiency is an autosomal codominant condition which causes an increased risk of thrombosis Features venous thromboembolism skin necrosis following the commencement of warfarin: when warfarin is first started biosynthesis of protein C is reduced. This results in a temporary procoagulant state after initially starting warfarin, normally avoided by concurrent heparin administration. Thrombosis may occur in venules leading to skin necrosis
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Organophosphate insectiside poisoning
One of the effects of organophosphate poisoning is inhibition of acetylcholinesterase leading to upregulation of nicotinic and muscarinic cholinergic neurotransmission. In warfare, sarin gas is a highly toxic synthetic organophosphorus compound that has similar effects. ``` Features can be predicted by the accumulation of acetylcholine (mnemonic = SLUD) Salivation Lacrimation Urination Defecation/diarrhoea cardiovascular: hypotension, bradycardia also: small pupils, muscle fasciculation ``` Management atropine
181
Restrictive Cardiomyopathy?
Restrictive cardiomyopathy Causes amyloidosis (e.g. secondary to myeloma) - most common cause in UK haemochromatosis post-radiation fibrosis Loffler's syndrome: endomyocardial fibrosis with a prominent eosinophilic infiltrate endocardial fibroelastosis: thick fibroelastic tissue forms in the endocardium; most commonly seen in young children sarcoidosis scleroderma Pathophysiology primarily characterized by decreased compliance of the ventricular endomyocardium causes predominately diastolic dysfunction Features similar to constrictive pericarditis low-voltage ECG Features suggesting restrictive cardiomyopathy rather than constrictive pericarditis prominent apical pulse absence of pericardial calcification on CXR the heart may be enlarged ECG abnormalities e.g. bundle branch block, Q waves Investigations echocardiography cardiac MRI
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Hereditary Haemorrhagic Telangiectasia
HHT is autosomal dominant condition - Multiple Telangectasia over skin and mucous membranes 2/ 4 = possible 3/4 = Definite - Epistaxis - Telangiectases - Visceral Lesions (GI tract, or pulm, hepatic, or cerebral AVMs) - Family History (first degree)
183
Alpha Thalassemia Gene Mutation?
Autosomal Recessive Deletion of the Alpha Globin Genes on Chromosome 16 2 pairs of genes 1 defect = silent carrier 2 defect = thalaseamia minor 3 defect = HbH disease - Severe anaemia and hepatosplenomegaly 4 defect -= Bart's Hydrops Fetalis - incompatabile with life
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Alpha Thalassemia Diagnosis
- Hemoglobin Electropheresis (increased band at HbH) - Genetic Testing - Fetal Sampling - amniocentesis Have Haemolysis and Extravascular haemolysis Get Jaundice Hypoxia - Increased RBC production Bones to enlarge and Hepatospenomegaly
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Treatment of Thalassemia
SEVERE only - Blood TRansfusions - Iron Chelating agents Fetal : Intrauterine blood transfusions Bone Marrow Transplants
186
Beta Thalassemia
``` Point Mutation in Beta Globin Gene on Chromosome 11 Causes reduced (B+) or abscent Beta Globin (BO) ``` Autosomal Recessive therefore can have traight or mild disease if 1 or severe disease if genes from both parents: 1 x B+ = Minor 1 x BO = Minor 2 x B+ = Beta Thalassemia Intermedia 2 x BO = Beta Thalassemia Major
187
Adult Hb
HbA x2 and HbB x2
188
Fetal Hb
HbA x 2 and Hb gamma x 2
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Beta Thalasemia Symptoms
Minor - Asymptomatic Beta Thalassemia Major - Start at 6 months as Fetal hb still exists ``` Symptoms: Anaemia Jaundice Hepatosplenomegaly Haemochromatosis Growth Retardation ``` Frontal Bossing Chipmunck Facies Hair on End Skull x ray
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Diagnosis
``` Low Hb Low MCV (Very Low) Increased RDW (different sizes due to immature retic) Film: Microcytic and hypochormic Target cells ``` High Fe High Ferritin High Transferritin Sat Level Haemoglobin Electrophoresis Low HbA High HbF High HbA2 levels
191
Beta Thalassemia major Treatment
Blood Transfusions Iron Chelating Agents Splenectomy if hepatosplenomegaly
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Causes of Microcytic Anaemia?
Iron Def Thalassaemia Congenital Sideroblastic anaemia Lead Poisoning
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post-transplantation lymphoproliferative disorder (PTLD)
weight loss anaemia lymphadenopathy His high dose immunosuppression have precipitated infection with EBV and subsequent B cell proliferation. PTLD incidence is highest in the first year post-transplantation, which is the time when immunosuppression is the most intense. Treatment involves reducing his current immunosuppression therapy and giving additional chemotherapy.
194
Drug induced Pancreatitis
``` azathioprine, mesalazine*, didanosine, bendroflumethiazide, furosemide, pentamidine, steroids, sodium valproate ```
195
Tacrolimus
Tacrolimus is a macrolide used as an immunosuppressant to prevent transplant rejection. It has a very similar action to ciclosporin: Action of ciclosporin decreases clonal proliferation of T cells by reducing IL-2 release binds to cyclophilin forming a complex which inhibits calcineurin, a phosphotase that activates various transcription factors in T cells The action of tacrolimus differs in that it binds to a protein called FKBP rather than cyclophilin Tacrolimus is more potent than ciclosporin and hence the incidence of organ rejection is less. However, nephrotoxicity and impaired glucose tolerance is more common
196
Zinc Deficiency
Zinc deficiency causes characteristic skin rash, alopecia, taste impairment, glucose, intolerance and diarrhoea.
197
Vitamin A deficiency
Night Blindness
198
Vitamin E deficiency
Spinocerebellar Ataxia Mypoathy Anaemia
199
Selenium deficiency
seen in malabsorption and parenteral nutrition | Cardiac necrosis causing congestive cardiomyopathy
200
``` Niacin deficiency (vitamin b3) ```
PEllagra 4 ds Dermatitis dementia diarrhoea death Alopecia Glossitis Oedema
201
Abscence Seizures?
First line is Valproate and Ethosuzimide
202
Polyarteritis nodosa?
Medium sized arterial vasculitis with necrotizing inflammation leading to aneurysm formation jPAN more common in middle aged men with Hep B ``` Fever Malaise Arthralgia Weight Loss Mononeuritis multiplex and sensorimotor polyneuropathy Testicular Pain Livedo Reticularis Renal failure pANCA postiive in 30% Hep B positive in 30% ```
203
Livedo reticularis?
``` Homocystinuria Polyarteritis nodosa SLE Amantadine Cholesterol Embolisation Antiphospholipid syndrome ```
204
Homocystinuria
Rare autosomal recessive def of cystathionine beta synthase Elevated urine homocystein concentration - Fine Fair Hair - Marfan's like - Learning Difficulties - Seizures - Lens disolcation - VTE - Livedo reticularis Treatment is Pyridoxine (Vitamin B 6)
205
Pyridoxine?
Vitamin B 6
206
Cholesterol Embolisation
Cholesterol emboli may break off causing renal disease Normally after vasc surgery or angiography Eosinophilia Purpura Renal Failure Livedo reticularis
207
Amantidine
Increases dopamine release and inhibits its uptake at synapses Causes ataxia, Slurred speech, confusion, dizziness, livedo reticularis Used in PArkinsons, MS, fam spastic ataxia
208
Myelofibrosis?
Occurs when haemopoetic cells lines are replaced by fibroblast. This casues fibrosis and reduced function of the bone marrow Jak2 gene leads to increased trigger of JAK2 pathway causing increased fibrosis Then haemopoetic cells migrate to other sites to produce blood - liver spleen lungs This leads to thromboyctopenia, hepatosplenomegaly and pulmonary hypertension The patient will be pancytopenic as the extra medullary processes can't fully compensate for fibrotic marrow Diagnosis shows increased platelets Pancytopenia Tear shaped and immature blood cells (nucleated rbc etc) Treatment is with EPO and blood transfusion Ruxolitinib stops JAK2 pathway and reduces spleen size BMT
209
Beri Beri
B1 deficiency B1 is Thiamine Thiamine is a water soluble vitamin of the B complex group Important in the catabolism of sugars, aminoacids Clinical consequences are seen in brain and hear (lots of aerobic tissues) Causes of Thiamine deficiency Alcohol excess Malnutrition Wenicke's enecphalopathy - Nystagmus, opthalmoplegia and ataxia Korsakoff's Syndrome - Amnesia and confabulation Dry beriberi - peripheral neuropathy Wet Beriberi - dilated cardiomyopathy
210
Idarucizumab
Dabigatran Reveral in life threatening bleeding
211
Dabigatran indications / contraindications
Prevention of VTE (PE/DVT) Prevention of STroke in AF Contraindicated with Mechanical heart valve replacements
212
Omalizumab
Can be used in IgE high Asthma
213
Chicken pox Exposure in Immunocomprimised?
Varicella Zoster Immunoglobulin (VZIG) prophylaxis is recommended for people who fulfill all 3 of: - Exposure to chicken pox or herpes zoster (shingles) - Clinical condition that increases risk of severe disease: This would be Pegnant, neonate or immunosuppresed - No antibodies Therefore need an antibody test before going to IVIG
214
Lofgren's Syndrome?
Acute presentation of Sarcoidosis | Presents with a classic Triad of erythema nodosum, polyarthralgia and bilateral hilar lymphadenopathy
215
Sarcoidosis investigations
``` ACE - raised Calcium Raised IL 2 receptors Rasied CRP Hilar lymphadenopathy High Resolution CT - Pulmonary Nodules PET CT ``` Histology is gold standard - non caseating granulomas with epitheliod cells
216
Sarcoidosis prognosis
6 Months - 60% of people have spontaneous resolution Can cause pulmonary fibrosis and pulmonary hypertension
217
Stoke Adam's syndrome?
Sudden loss of conciousness secondary to intermittent complete heart block Wide inverted T wave Complete HEart Block History of sudden intermittent fainting
218
Paraquat poisoning
Multi organ damage including lungs, heart liver. Quite deadly Only real treatment is early use of activated charcoal or fullers earth Associated with Parkinsons Disese
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MEN | Multiple Endocrine Neoplasia
This is a genetic mutation that causes predisposition to development of adenomas in gland tissue 3 main patterns of disease described MEN 1 - (3 Ps) Pituitary, parathyroid and Pancreas Caused by the MEN 1 GENE MEN 2 is all caused by RET ONCO GENE MEN 2 a - 2 Ps Parathyroid Phaemochromocytoma Medullary Thyroid Cancer ``` Men 2 b - 1 P (2 Ms) Phaeochromocytoma Medullary Thyroid Cancer Neuromas MARFANS LIKE BODY ```
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Holmes Tremor?
Lesions in the Red Nucleus Most commonly due to a stroke in the area Irregular Low Frequesncy Tremor At Rest, POSTURAL AND INTENTION Levodopa or chronic thalamic stimulation
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Vemurafenib and Dabrafenib
Both BRAF inhibitors Used to increase survival in metastatic Melanoma Activate the MAPK pathway with keratinocytes and cause increased SCC often within the first three months of therapy
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Roflumilast
Recommended in COPD where there are > 2 exacerbations / year and FEV1 < 50% Phosphodiesterase - 4 inhibitor recommeneded
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CKD bone complications?
Low Vitamin D 1 alpha hydroxylation occurs in the Kidny High Phosphate - Kidneys normally excrete phosphate High phosphate - drags calcium from bones causing osteomalacia Low calcium due to lack of Vit D and High phosphate Secondary hyperparathyroidism
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Treatmen of CKD bone disease
Aim is to reduce phosphate and reduce parathyroid hormone levels Reduce dietary phosphate intake Phophate Binders Alfacalcidol and calcitriol (VIT D) Parathyroidectomy ``` Phosphate binders: Calcium Carbonate (issues in hypercalcaemia) ``` Sevelamer - Non calcium direct binder Reduces Uric acid and Lipid profile
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HOCM Treatment?
1st line Start with Betablocker Verapamil in asthmatics 2nd Line If failed to tollerate with just monotherapy now start DISOPYRAMIDE (negative ionotropic anti arrhythmic that reduces the LVOT - left ventricular outflow tract gradient and reduces mortality 3rd line Consider Surgical Myectomy, DDR pacemakers and alcohol ablation. ACEi should be avoided in HOCM as reducing afterload can worsen the LVOT gradient
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HOCM is an autosomal dominant disorder of muscle tissue in 0.2% population
``` Treatment is with Amiodarone Betablockers or verapamil Cardioverter defib Dual Chamber PAcemakers Endocarditis Prophylaxis ``` Avoid NItrates and ACE inhibitors
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Foster Kennedy Syndrome
Frontal lobe mass that leads to ipsilateral optic nerve compression and raised intracrainial pressure and secondary optic nerve atrophy ``` Reduced color vision Reduced visual acuity optic atrophy in the ipsilateral eye papilloedema in the contralateral eye central scotoma in the ipsilateral eye anosmia ```
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Restless leg syndrome?
Need to move legs Relieved by movement Worse at night Causing an impact on social or life Treatment with dopaine agonists or Gabapentin Second line is SSRI ``` there is a positive family history in 50% of patients with idiopathic RLS iron deficiency anaemia uraemia diabetes mellitus pregnancy ``` pramipexole, ropinirole, bromocriptine, levodopa-carbidopa, and rotigotine
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Aortic Stenosis
Clinical features of symptomatic disease chest pain dyspnoea syncope An ejection systolic murmur (ESM) is classically seen in aortic stenosis. This is decreased following the Valsalva manoeuvre. ``` Features of severe aortic stenosis narrow pulse pressure slow rising pulse delayed ESM soft/absent S2 S4 thrill duration of murmur left ventricular hypertrophy or failure ``` Causes of aortic stenosis degenerative calcification (most commo if asymptomatic but valvular gradient > 40 mmHg and with features such as left ventricular systolic dysfunction then consider surgery cardiovascular disease may coexist. For this reason an angiogram is often done prior to surgery so that the procedures can be combined balloon valvuloplasty is limited to patients with critical aortic stenosis who are not fit for valve replacement
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Spastic Paraparesis
Spastic paraparesis describes a upper motor neuron pattern of weakness in the lower limbs ``` Causes demyelination e.g. multiple sclerosis cord compression: trauma, tumour parasagittal meningioma tropical spastic paraparesis transverse myelitis e.g. HIV syringomyelia hereditary spastic paraplegia osteoarthritis of the cervical spine ```
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Amyloidosis
amyloidosis is a term which describes the extracellular deposition of an insoluble fibrillar protein termed amyloid amyloid is derived from many different precursor proteins the accumulation of amyloid fibrils leads to tissue/organ dysfunction Classification systemic or localized further characterised by precursor protein (e.g. AL in myeloma - A for Amyloid, L for immunoglobulin Light chain fragments) AA amyloidosis occurs secondary to a poorly controlled chronic inflammatory condition such as ankylosing spondylitis or rheumatoid arthritis. The key to treatment is to address the underlying condition. AL amyloidosis is not linked to another condition and results from abnormal plasma cells producing excessive amounts of light chains in a process similar to multiple myeloma. Diagnosis Congo red staining: apple-green birefringence serum amyloid precursor (SAP) scan biopsy of rectal tissue Can cause carpal tunnel
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Exercise induced wheat angioedema
Exercised induced anaphylaxis is now well described and is most associated with wheat ingestion. The clinical manifestations usually occur around 10 minutes after exercise and follow a sequence of pruritus, widespread urticaria and then subsequently respiratory distress and vascular collapse. The condition usually resolves on stopping exercise and is managed in the same manner as anaphylaxis. The patients can usually eat the causative food without problems so long as they do not exercise afterwards. The physiology of the condition remains slightly unknown, but it may be related to endorphin release during exercise. The endocrines cause excessive histamine release from mast cells in susceptible individuals.
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ST elevation without reciprocal depression?
The presence of ST elevation without reciprocal depression shortly following a myocardial infarction is suggestive of a left ventricle aneurysm. Left ventricle aneurysms predispose to both ventricular arrhythmias and cardiac thrombo-embolisms.
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tropical spastic paraparesis
HTLV-1 associated Myopathy HAM Transmitted through sexual contact / breastfeeding / sharing needles HTLV-I is a retrovirus endemic in southern Japan, equatorial Africa and South America. Presents with upper motor neurone signs and symptoms particularly in the LOWER Limb ``` PAtient with 1/5 Lower limbs Hyperreflexia Chronic Urinary Retention HTLV Antibody positive Steroids have minimal help but only treatment ```
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Progressive Supranuclear Palsy
Progressive supranuclear palsy Overview aka Steele-Richardson-Olszewski syndrome a 'Parkinson Plus' syndrome ``` Features impairment of vertical gaze (down gaze worse than up gaze - patients may complain of difficultly reading or descending stairs) parkinsonism falls slurring of speech cognitive impairment ``` Management poor response to L-dopa
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Cardiac Catheterisation and oxygen saturations
deoxygenated blood returns to the right side of the heart via the superior vena cava (SVC) and inferior vena cava (IVC). It has an oxygen saturation level of around 70%. The right atrium (RA), right ventricle (RV) and pulmonary artery (PA) normally have oxygen saturation levels of around 70% the lungs oxygenate the blood to a level of around 98-100%. The left atrium (LA), left ventricle (LV) and aorta should all therefore have oxygen saturation levels of 98-100% Dependent on where the lesion is ASD/VSD/ PDA you can figure out which blood will be mixed Eisenmenger syndrome is reveral of shunt (from R to left) PDA attaches the Aorta to the PA (therefore PA will have higher oxygen sats)
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DRESS Syndrome
Unexpected severe reaction to medication Skin, liver Kidneys Lungs and heart are involved 2 - 8 weeks after starting medication ``` Skin Rash (MORBILLIFORM) Inflammation - looks like sepsis ``` Eosinophilia Lymphadenopathy LFT derrangement Stop Drug Piriton/steroids for rash Supportive Care Drug Reaction with Eosinophillia and Systemic Symptoms
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HELP Syndrome
Haemolysis, Elevated Liver Enzymes and Low Platelets A serious manifestation of Pre Eclampsia HTN, Vomiting and Abdominal Pain
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Pre Eclampsia? Complications and RF
Pre Eclampsia is seen after 20 weeks and characterised by Pregnancy induced Hypertension with proteinuria (0.3g/24 hours) ``` Pre Eclampsia causes: - Prematurity IUGR Eclampsia Haemorrhage Cardiac Failure Multi-organ Failure ``` RF: HTN, CKD Autoimmune T1/2 Diabetes ``` OTher softer: - first preg > 40 BMI > 35 Muliple pregnancy Family history ```
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Severe Pre Eclampsia?
``` Hypertension > 170 Proteinuria HEadache Visual disturbance RUQ/Epigastric pain Hyperreflexia Platelet count < 100 ``` Essenitally - if you are symptomatic from HTN or have any HELP then it is severe
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Treatment of Pre Eclampsia?
Mod - High Risk Preg= Aspirin from 12 weeks 75mg OD Treat Blood pressure > 160 (oral labetalol) Nifedipine if asthmatic Delivery of the baby is ultimate treatment
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Pneumothorax diving?
Need pleuradhesis can't go until this
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Difference between osa and ohs
OSA is transient hypercapnea after sleeping | OHS is chronic hypercapnea in the daytime with increased bicarb (like COPD)
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Anteriror Interosseous Syndrome
Motor loss of thumb,, index and middle finger | No sensory loss( that would be median nerve palsy)
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Erythema Multiforme
Hypersensitivity reaction secondary to infections or drugs Thought to be spectrum of stevens johnson syndrome Target LEsions Back of the hands and feed Pruritis sometimes seen ``` HSV - 50% of cases ORF IDiopathic Mycoplasma Streptococcus Penicillin, sulphonamides, carbemazepine, allopurinol, NSAIDs, OCP , Nevirapine ``` SLE Sarcoidosis Malignancy
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WHich pathology recurrs in renal transplant the most?
MEmbranous Glomerulonephritis Most common cause of nephrotic sydrome in adults ? maybe because it effects some people twice??
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Poor Prognosis in aspestosis?
Clubbing
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Pleural Thickening/ Plaques
Plaques: Mainly cuased by Asbestos Previous rib fracture Thickening: Can be Benign or Malignant - Benign is caused by inflammation essentially: - recent inflam - recurrent pneumothoraces - Pleural empyema - Haemothorax - Asbestosis - Silicosis - Malignant: - Mesothelioma - Primary Pleural Lymphoma - Pleural Mets (Lung and Breast most Commonly)
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Mesothlioma
Malignant disease of the pleura. Crocidolite blue asbestos is the most dangerous form ``` Progressive SOB Chest Pain Pleural Effusions Weight loss Chest Wall pain Clubbing 30% present with painless pleural effsion ``` Malignancy of meothelial cells of the pleura Mets to contralateral lung and peritoneum R> L lung CXR - pleural effusion or thickening Pleural CT Biopsy nodules Send Cytology from pleural fluid Palliative treatmnet VERY POOR PROGNOSIS Asbestos also causes increase risk factor for lung cancers with synergistic (x the rf) effect with cigarrete smoking
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Lung Fibrosis
Upper and Lower Zone Most common in Lower zones (idiopathic pulmonary fibrosis and drugs) ``` - Upper: Hypersensitivity pneumonitis (extrinsic allergic alveolitis) Coal workers (pneumoconiosis Silicosis Sarcoidosis Ankylosing spondylitis Histiocytosis Tuberculosis ``` ``` lower zones - MORE COMMON Idiopathic Pulmonary fibrosis Connective tissue (SLE RA) Amiodarone, Bleomycin, Methotrexate Aspestosis ```
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Idiopathic pulmonary fibrosis
Chronic lung disease by progressive fibrosis of the intersitium of the lungs No underlying cause in idiopathic Seen in 50-70 year olds More common in men Progressive Exertional dyspnoea Fine End inspiratory crackles Dry Cough Clubbing Restrictive Spirometry FVC reduced, FEV1 Preserved FEV1 / FVC = Increased Impaired Gas Exchange - Reduced TLCO Interstitial shadowing - ground glass then going to honey combing ANA positive and RF positive in some cases Rehab LTOT and Lung Transplant Poor Prognosis
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Budd Chiari Syndrome Triad?
Abdominal Pain Painful hepatomegaly Ascites
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Budd Chiari Pathophysiology
Blocked hepatic veins (stop blood going into IVC) and cause post hepatic obstruction Lobular Necrosis in the sinusoids - cause nutmeg liver Portal hypertension (superior mesenteric and splenic vein) causes splenomegaly Shunts caused oesoph varices, caput medusa and haemorrhoids
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Causes of budd chiari
El Louise Mostly associated with hypercoaguability Polycythemia ruba vera is most common cause OCP Pregnancy Paroxysmal Nocturnal Haemaglobinuria (unkown) Ca Other secondary causes (from outside compression) - Tumour Think of Evie Jane
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Confirmed Meningococcal meningitis contacts?
Receive 7 days cipro / rifampicin think of adam This is only for meningococcal disesae Not for Pneumococcal meningitis
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Dubin Johnson Syndrome
Raised Conjugated Bilirubin Otherwise normal LFTs Benign autosomal recessive disorder with conjugated hyperbilirubinaemia that is conjugated and therefore in the urine. CMOAT Protein defect
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Nitrous Oxide and B12 Deficiency?
Nitrous Oxide abuse can exacerbate the B12 deficiency especially in at risk groups
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B12 low, Folate high?
Think small bowel bacterial overgrowth RF: - Noenates with congenital GI abnormalities - DM - Scleroderma (? Oeosphageal dysmotility) Looks like IBS - diarrhoea, bloating, abdo pain Hydrogen breath test - small bwoel aspiration and culture is used Correction of the underlying disorder normally fixes the problem Antibiotics )(Co amox or metronidazole) B12 is Low, Folate High
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Liddle's syndrome
Rare Autosomal Dominant condition Hypertension Hypokalaemia Acidosis Will have picture of hyperaldosteronism Then renin and aldosterone will be low (negative feedback as kidneys are perfused well - this is normal) Then diagnosis is Liddles They alter the sodium channels in the distal tubules causing increased sodium absorption Treatment is with amiloride or triamterene - potassium sparing diuretic
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Myotonic Dystrophy
Most common muscular dystrophy - delayed relaxation of muscles - Wasting and weakness - Cardiac conduction deficits - Cataracts - Characteristic FAcies DM1 and DM2 It is autosomal Dominiant Trinucleotide repeat DM1 - CTG at end of DMPK gene on 19 DM2 ZNF9 on Chromosome 3 DM1 - Chromosome 19 Distal weakness more prominent DM2 - ZNF9 chromosome 3 Proximal weakness more prominent Severe Congenital form not seen - Long Haggard faces - Frontal Balding - Bilateral Ptosis - Cataracts - Dysarthria ``` Myotonic - spasms of muscles Distal weakness Mild cognitive impairment DM Testicular atrophy Cardiac involvement - myopathy or heart block Dysphagia ``` Think of the young guy with cardiac arrest at Mansfield ICU
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Albumin Creatinine Ratio?
MEn < 3.5 = normal | Women < 2.5 = Normal
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Cysticercosis
Larval stage of a tpae Worm Taenia Solium Neurocysticercosis or Extraneural cysticercosis NCC causes seizures in endemic areas Ingestion of eggs in tapeworm carier stool Eggs hatch in small intestine, invade bowel wall and then disseminate via blood to brain, liver, muscle. Cysts located in the brain - neurocysticercosis and lead to hydrocephalus. Can be very protracted history Brain is swiss cheese appearance Bendazoles is the treatment
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Cyanide Poisoning
Most Common cause is fire Found in insecticides, photograph development and metal ``` Brick Red Skin Smell of Almods Hypoxia, Hypotension, headache, confusion Brady Cardia Lactic Acidosis Confusion DILATED PUPILS ``` 100% oxygen 4 threapies Hydroxocobalamin IV Sodium Nitrite IV Sodium Thiosulfate IV Amyl nitrite
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Organophosphate poisoning
Inhibition of acetylcholinesterase leading to increased nicotinic, muscarinic and cholinergic neurotransmittion ``` SLUD (parasympathetic) Salivation Lacrimation Urination Defication Bradycardia Small Pupils ``` Atropine Pralidoxime
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Guttate Psoriasis
Children and adolescents May be precipitated by streptococcal infection 2-4 weeks prior Tear Drop Papules with scaling on the top Most resolve on their own within 2-3 months UVB
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Guttate Psoriasis
Children and adolescents May be precipitated by streptococcal infection 2-4 weeks prior Tear Drop Papules with scaling on the top Most resolve on their own within 2-3 months UVB Lesions are small round or oval with scaly papules All over the body Trunk upper back
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Aminophylline
...
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Scan for Phaeochromcytoma?
MIBG Scan uses radioactive Iodine to find phaeochromocytoma tumour cells which is detected with the gamma camera
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Pentagastrin Stimulation test?
Test for medullary carcinoma of the thyroid (associated with MEN 2)
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24 hr urinary HIAA
Screening tool for carcinoid
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MRI renal angiography
Used for Renal Artery stenosis (> CT)
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GH, IGF1 and Gut hormones-
test for pancreatic malignancy associated with MEN 1
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MIBG
Scan for Phaeochromocytoma. | 10% of phaeochromocytoma is in the sympathetic chain (similar to neuroblastoma).
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Fish tank granuloma
Mycobacterium Marinum 3-4 week incubation and lesions are painful or painless Break in the skin then you get lumps Treatment with tetracyclines, fluoroquinolones, sulfonamides and macrolides
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Miller - Fisher Syndrome?
Opthalmoplegia, ataxia and areflexia = think miller fisher Vairant of Guillain Barre Syndrome Anti GQ1b Antibodies in 90 % Eye Muscles are typically first effected Descending paralysis normally Anti GQ1b
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guillain Barre Syndrome
Immune mediated demyelination of peripheral nervous system often triggered by infection (Campylobacter classically) Cross reaction of antibodies with gangliosides in the peripheral nervous system AntiGanglioside antibodies found in 25% of patients Anti GM1 Antibodies (25%)
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Anti GM1 Antibodies
Guillain Barre Syndrome
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Anti GQ1b Antibodies
Miller-Fisher Syndrome
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Anti Jo Antibodies
Polymyositis
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Anti Cholinesterase antibodies
Myasthenia Gravis
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Tuberculosis Management in resistant disesae
If resistant disease requires 5 drugs for 18-24 months If LFTs 5 x the normal limit then stop the medication
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Atrial Septal Primum Defect osition?
These are low down in the septum
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Atrial Septal Secundum Defect position?
These are high up in the septum
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Atrial Septal Secundum Defect position?
These are high up in the septum
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MS in pregnancy
Stop Interferon Beta Pregnancy improves rate of relapse during pregnancy, then increases for 6 months post partum, then goes back to normal There is no risk of progression to progressive disease
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Expanded Disability Status Scale
This is a tool used to gague impact of MS on patients life and measure severity of diease/ relapses
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Bergers disease Causes? Associated diseases?
Commonest cause of Glomerulonephritis worldwise Macroscopic Haematuria in young person with an URTI ``` Associated conditions: Alcoholic cirrhosis Coeliac Disease Dermatitis Herpitformis HSP ``` Mesangial Deposition of IgA immune complexes Considerable overlap with HSP Positive immunofluorescence for igA and C 3 Typical patient: Young male with recurrent episodes URTI Very rare progression to renal failure
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How to differentiate between post strep and IgA nephropathy
Post Strep Low Complement levels Proteinuria > Haematuria An interval between URTI and renal problems in post strep Berger's - Haematuria - 2 days post URTI 'Young males
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Treatment of IgA
Steroids have no help Endstage renal failure in 25% Good Prognosis is Frank Haematuira Bad is Male and Protein ++
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Long QT Syndrome
Inheritted condition with delayed repolarization of the ventrilces. Can lead to VT/ Torsade de pointes and sudden death. LQTS1 + 2 are commonest and are from slow rectifier potassium channel defects. Long QT1 - USually with exertional syncope, oten swimming Long QT2 - Emotional stress causes syncope Long QT3 - Events occur at night or at rest
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Drugs that cause prolonged QT
Amiodarone, Sotalol TCA/ SSRIs (citalopram) Methadone Chloroquine Erythromcyin Haloperidol Ondanestron
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Drugs that cause prolonged QT
Amiodarone, Sotalol TCA/ SSRIs (citalopram) Methadone Chloroquine Erythromcyin Haloperidol Ondanestron
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Jervell Lange Nielsen
Long QT + Deafness
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Ramano-Ward Syndrome
Long QT no Deafness
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Electrolyte causes of Long QT
Hypocalcaemia, kalaemia, magnesaemia Also Acute MI, myocarditis, hypothermia and SAH
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Anti Mictochondrial Antibodies?
Primarcy Biliary Cirrhosis IgM M2 Subtype Middle Age Females
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Anti Mictochondrial Antibodies?
Primarcy Biliary Cholangitis IgM M2 Subtype Middle Age Females Sjogren's Syndrome Rheumatoid Arthritis Systemic Slcerosis Thyroid Disease
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MEN 2a/b Thyroid cancer monitoring?
Calcitonin - This is the marker for medullary thyroid cancer
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Chromogranin A and 5-HIAA?
Neuroendocrine tumours and Carcinoid Tumours respectively
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Thyroid peroxidase antibody titre ?
Autoimmune thyroid conditions like hashimotos and Graves
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Thyroglobulin
Marker for papillary and follicular thyroid carcinoma
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Lichen Sclerosus
Inflammatory condition that usually affects the genitalia and is more common in elderly females. Leads to atrophy of the epidermis White Plaques Very itchy Steroids and emolients
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Lichen Sclerosus
Inflammatory condition that usually affects the genitalia and is more common in elderly females. Leads to atrophy of the epidermis White Plaques Very itchy Steroids and emolients
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Pemphigoid Gestationis
Pruiritic blistering lesions Larger and peri umbilical 2nd - 3rd trimester Oral Steroids
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Polymorphic eruption of pregnancy
Pruritic condition of the last trimester Abdominal striae Topical steroids - oral
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Rabies?
Acute encephalitis Agitation Hypersalivation Hydrophobia ``` Dog or animal bit in foreign country Need irrigation of the wound Need IVIG x 2 doses If immunised need 2 further doses of the vaccine If un immunised ```
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Rabies?
Acute encephalitis Agitation Hypersalivation Hydrophobia Dog or animal bit in foreign country Need irrigation of the wound If immunised need 2 further doses of the vaccine If un immunised Need IVIG doses in first 7 days Then need full vaccination course Use newer vaccinations over nerve based ones
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Rabies?
Acute encephalitis Agitation Hypersalivation Hydrophobia Dog or animal bit in foreign country Need irrigation of the wound If immunised need 2 further doses of the vaccine If un immunised Need IVIG doses in first 7 days Then need full vaccination course Use newer vaccinations over nerve based ones
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Saccharin test?
Ciliary dysmotility
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Low Immunoglobulins in respiratory case?
Think Bronchiectasis | IgA immuno deficiency and hypogammaglobulinaemia cause Bronchiectasis
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Low Immunoglobulins in respiratory case?
Think Bronchiectasis | IgA immuno deficiency and hypogammaglobulinaemia cause Bronchiectasis
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Alpha 1 antitripsin
Deficiency of a Protease inhibitor produced by the liver This is protective against neutrophil elactase Non smoking COPD - think alpha 1 antitrypsin Linked on 14 chromosome Allells are looked at on electropheresis Allells - M= normal, S= slow, Z = very slow PiMM is normal PiSS is 50% of normal alpha 1 anti tripsin speed PiZZ is 10% of normal alpha 1 antitripsin speed PiZZ normally manifest disease Mainly emphysema in lower lobes Also Liver cirrhosis in 1% Spirometry shows obstructive picture!
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Treatment of H Pylori
FIrst-line treatment over seven days: If no allergies: PPI, amoxicillin and clarithromycin or metronidazole If penicillin allergic: PPI, clarithromycin and metronidazole If penicillin allergic and previous clarithromycin exposure: PP, bismuth, metronidazole and tetracycline
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Acintomyces?
Actinomyces israelii chronic, progressive granulomatous disease caused by filamentous Gram-positive anaerobic bacteria Actinomycetaceae family. typically causes oral/facial abscesses with sulphur granules in sinus tracts may also cause an abdominal mass e.g. in the right iliac fossa The mass forms multiple sinus tracts Histological: Gram-positive organisms and sulphur granules.
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Acintomyces?
Actinomyces israelii chronic, progressive granulomatous disease caused by filamentous Gram-positive anaerobic bacteria Actinomycetaceae family. typically causes oral/facial abscesses with sulphur granules in sinus tracts may also cause an abdominal mass e.g. in the right iliac fossa The mass forms multiple sinus tracts Histological: Gram-positive organisms and sulphur granules. Long-term antibiotic therapy usually with penicillin May require surgery in necrosis or abscess formation
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Acintomyces?
Actinomyces israelii chronic, progressive granulomatous disease caused by filamentous Gram-positive anaerobic bacteria Actinomycetaceae family. typically causes oral/facial abscesses with sulphur granules in sinus tracts may also cause an abdominal mass e.g. in the right iliac fossa The mass forms multiple sinus tracts Histological: Gram-positive organisms and sulphur granules. Long-term antibiotic therapy usually with penicillin May require surgery in necrosis or abscess formation
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Norcardia?
Nocardia Basics typically causes pneumonia in immunocompromised patients may also cause brain abscesses
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Norcardia?
Nocardia Basics typically causes pneumonia in immunocompromised patients may also cause brain abscesses Sulphur granules seen
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Causes of Hypoglycaemia
Hypoglycaemia Causes insulinoma - increased ratio of proinsulin to insulin self-administration of insulin/sulphonylureas liver failure Addison's disease alcohol Other possible causes in children nesidioblastosis - beta cell hyperplasia
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C peptide
Is a mraker of endogenous insulin secretion Can be used to investigat insulinoma Fasted Insulin and C peptide levels (supervised) will be normal if no insulinoma These will be raised in insulinoma causing the hypoglycaemia
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C peptide
Is a mraker of endogenous insulin secretion Can be used to investigat insulinoma Fasted Insulin and C peptide levels (supervised) will be normal if no insulinoma These will be raised in insulinoma or sulphonyl urea poisoning causing the hypoglycaemia
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False Positive 5-HIAA urinary collection
``` Foods causing false positive 5-HIAA urinary collection results: Banana Avocado Aubergine Pineapple Plums Walnuts Tomatoes ``` ``` Drug Flouracil Caffeine Naproxen Paracetamol ```
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Drugs causing false negative 5-HIAA urinary collection
Aspirin, Levodopa, Methyldopa, ACTH
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Anaemia in CKD patients?`
Correct Iron Ferritin < 100 or Transferrin sats > 20% then give iron infusion Folowing this - give the EPO
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Hydrogen breath test?
Small BActerial Overgrowth Syndrome RF: DM, Scleroderma and Neonates with GI abnormalities Rifamixin Co amoxiclav Metronidazole High FOLATE Low B12
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Urea Breath Test
H. Pylori
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Denosumab
New treatment for osteoperosis Human MAB Stops RNAKL osteoclasts Can be given to prevent path fractures if boney mets are present If low eGFR - this is the preferred treatment of osteoperosis
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Pulmonary arterial pressure
Less than 20mmHg
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Bowen's disease
Intraepidermal SCC Elderly Ladies get it Red Scaly Patches Sun Exposed Areas like lower limb
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Brucellosis?
Gram positive cocci Associated with close contact with animals and unpasturised milk Treatment is Doxycycline and Rifampicin for 8 weeks Features non-specific: fever, malaise hepatosplenomegaly sacroilitis: spinal tenderness may be seen complications: osteomyelitis, infective endocarditis, meningoencephalitis, orchitis leukopenia often seen Diagnosis the Rose Bengal plate test can be used for screening but other tests are required to confirm the diagnosis Brucella serology is the best test for diagnosis blood and bone marrow cultures may be suitable in certain patients, but these tests are often negative
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Alkaptonuria
Autosomal recessive disesae of HDG deficiency Tyrosine metabolism therefore not possible then toxic build up of homogenistic acid Urine is black Pigmented sclera Then the acid builds up in cartilage and tissue Causes calcification of intervertabral discs- causing back pain Renal Stones Change of Diet High Dose Vitamin C Looks like bamboo spine
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Acute Interstitial Nephritis
``` 25% of all Drug induced AKI Causes drugs: the most common cause, particularly antibiotics penicillin rifampicin NSAIDs allopurinol furosemide systemic disease: SLE, sarcoidosis, and Sjögren's syndrome infection: Hanta virus , staphylococci ``` ``` Features fever, rash, arthralgia eosinophilia mild renal impairment hypertension ```
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Migraine prophylaxis?
2 or more attacks per month Topiramate or propanolol Propanolol in child bearing women (Topiramate is teratogenic) Toperamte in asthma 2nd line is acupuncture
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serum cystatin C
eGFR in high muscle mass or low muscle mass
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COPD severity?
Post Bronchodilator FEV1/FVC = < 0.7 + ``` Stages: FEV1 <80% predicted = 1 FEV1 50-80% of Predicted = 2 FEV1 30-50% of PRedicted = 3 FEV1 <30% of PRedicted = 4 ```
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Poorly controlled DM1 with high BMI and south indian?
Can have metformin to reduce the insulin side effects and improve glycaemic control
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CLL indications for treatment
Progressive marrow failure Massive >10cm or progressive lymphadenopathy Massive > 6cm or progressive splenomegaly Progressive Lymphocytosis (>50% increase in 2 months) Systemic symptoms Autoimmune cytopaenia
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Management of CLL
? For Treatment - ? indicated If not indicated - regular blood test Fludarabine, Cyclophosphamide and rituximab is initial treatment FCR Ibrutinib for those who failed prev FCR
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Ibrutinib
Chemo for those who fail initial Fludarabine, Cyclophosphamide and rituximab treatment for CLL
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FCR?
Fludarabine, Cyclophosphamide and rituximab for CLL
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Neuromyelitis optica
DEVIC's Disease Demyelinating condition associated with anti NMO antibodies - Aquaporin 4 antibodies. This presents with demyelination of the optic nerve and spinal cord. More severe corse than MS Particularly prevalent in asian populations Optic nerve and cervical spine 2/3 of the following 1. Spinal Cord Lesion involving 3 or more levels 2 Initially normal MRI brain 3. Aquaporin 4 positive serum antibody
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Uhthoff's phenomenon
Seen in MS - Worsening of symptoms associated with heat. This could be hot weather, exercise, hot bath or tubs or saunas
343
Hypertriglycerideaemia?
Causes pancreatitis Treated with Fibrates Statins do reduce triglyceride levels but they are manly indicated in mixed hyperlipidaemia
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Calciphylaxis
Rare complication of endstage renal failure. Depsoitions of calcium within arterioles causing microvascular occlusion and necrosis. This causes painful necrotic skin lesions. Hypercalcaemia, hyperphosphataemia and hyperparathyroidism Warfarin causes / exacerbates / is contra indicated in Calciphylaxis Treatment is trying to control cal phosph and pth levels and avoid calcium and warfarin.
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Treatment of pulmonary fibrosis?
Get a restrictive lung picture FEV1 normal, FVC decreased, FEV1/FVC increased Pulmonary rehab Pirfenidone (antifibrotic agent) is used in selected patients Nintendanib Also in certain circumstances
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Inferior MI and heart block
Inferior portions of ventricles supplied by the Right Coronary artery Also supplies the SAN and AVN Therefore heart block followin inferior MI is failry common and transient after PCI Escape rhythm comes from high up in conductive system so narrow QRS as goes down bundle of his
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Anterior MI and heart block
Heart block is uncommon but shows significant damage to ventricular myocardium and conductive system Escape rhythm therefore starts distally resulting in a broad complex ventricular escape rhythm - wide QRS
348
TTP
Pentad - Microangiopathic haemolytic anaeia - Thrombocytopenia - Fever - Neurological fluctuation - Renal Failure Microangiopathic haemolytic shown by schistocytes TTP is inherited or acquired Inhertied - Deficiency of ADAMTS13 Gene Therefore Deficient of the enzyme Acquired - Much more common An Inhibitor of ADAMTS13 Enzye - redcued activity ADAMTS13 normal function - Cleave vWF multimers into vWF monomers In TTP this is reduced therefore increased vWF multimers - Causing platelet microthrombi and decreased platelets - Microthrombi In acquired TTP it is IgG inhibition Coombes test negative ``` MCV normal Intravascular haemolysis: - LDH high - Haptoglobin low - Hemosiderin urea - Hyperbilirubinaemia - Platelets low ``` Treatment is with Plasma Exchange - new plasma to increase ADAMST13 Enzymes
349
Upshaw - Schulman Syndrome
Autosomal recessive Deficiency of ADAMTS13 Enzyme causing TTP
350
Causes of Acquired TTP
post-infection e.g. urinary, gastrointestinal pregnancy drugs: ciclosporin, oral contraceptive pill, penicillin, clopidogrel, aciclovir tumours SLE HIV
351
Colonoscopy screening in IBD patients for Colorectal cancer
Increased risk Surveillance is done on mild, mod, severe risk - Mild (EVERY 5 YEARS) - Extensive colitis with no active endoscopic/histological inflammation - OR left sided colitis - OR Crohn's colitis of <50% colon Mod (EVERY 3 YEARS) - Extensive colitis with mild active endoscopy/histological inflammation - OR post-inflammatory polyps - OR family history of colorectal cancer in a first degree relative aged 50 or over Severe (EVERY YEAR) - Extensive colitis with moderate/severe active endoscopic/histological inflammation - OR stricture in past 5 years - OR dysplasia in past 5 years declining surgery - OR primary sclerosing cholangitis / transplant for primary sclerosing cholangitis - OR family history of colorectal cancer in first degree relatives aged <50 years
352
``` Guillian Barre syndrome: weakness? Tone? Reflexes? Sensory symptoms? Pain? CSF? ```
Guillain-Barre syndrome describes an immune mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni) Weakness in the lower extremities are affected first, however it tends to affect proximal muscles earlier than the distal ones reflexes are reduced or absent sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs around 65% of patients experience back/leg pain in the initial stages of the illness Slightly raised protein on CSF
353
Bile Acid Malabsorption
Common in Crohns post terminal ileal resection Terminal ilium absorbs bile acids Without this can have chronic diarrhea Secondary causes are often seen in patients with ileal disease, such as with Crohn's. Other secondary causes include: cholecystectomy coeliac disease small intestinal bacterial overgrowth the test of choice is SeHCAT nuclear medicine test using a gamma-emitting selenium molecule in selenium homocholic acid taurine or tauroselcholic acid (SeHCAT) scans are done 7 days apart to assess the retention/loss of radiolabelled 75SeHCAT Management bile acid sequestrants e.g. cholestyramine
354
SeHCAT scan ?
Selenium scan for Bile Acid Malabsorption
355
Which antibiotic causes tendinopathy/ Rupture?
Ciprofloxacin | Levofloxacin
356
Staghorn / Struvite stones
Magnesium, ammonium phophate stones are formed in urinary amonnia high levels and alkaline urine > 7.2. Urea-releaseing bacteria hydrolyze the ammonium (Proteius, Klebsiella and Mycoplasma) Occurs in women more commonly Hypercalaemia is another risk factor
357
Numbers in SIADH
Na < 135 Serum Osm < 271 Urine Osm > 100 Euvolaemic patient
358
SIADH Causes
Malignancy (Small cell lung CA/ Prost/ Panc) Neuro (Stroke, SAH, Subdural, Infection) Infection (TB, Pneumonia) Drugs: Sulfonylurea, SSRI, Carbamazepine, Cyclophosphamide, Vincristine PEEP Porphyrias
359
Treatment of SIADH
Slow correction to avoid central pontine Myelinolysis Fluid REstriction Demeclocycline ADH (Vasopressin) antagonist
360
Multifocal motor Neuropathy with conduction Block
An Acquired immune mediated demylinating neuropathy MND like symptoms but responds to IVIG Typically it presents with aseymmetric bi brachial motor weakness wihtouth sensory or bulbar involvement Treatmnet is IVIG
361
TRALI - Transfusion related acute lung injury
Seen in plasma transfusions and cause of most morbidity in trasnfusions. Human Luecocyte antibodies / Neutrophil antibodies in the donor plasma lead to non cardiogenic pulmonary oedema due to increased vascular permeability. Hypoxia, Pulmonary infiltrates on Chest X ray, Fever, Hypotension Stop Transfuision Oxygen Supportive care
362
TB screening?
Performed with the Mantoux test Type 4 Hypersensitivity Reaction Interferon gamma blood test then used Mantoux test: 0.1ml of 1:1000 purified protein derivative is injected intradermally Read 3 days later If induation < 6mm - Negatvie - no significant hypersensitivity to tuberculin protein - PREVIOUSLY UNVACCINATED If Induration 6-15mm - Postivie- hypersensitive to tuberculin protein. NOT FOR BCG - PREVIOUS TB or PREVIOUS BCG 15 mm + = Strongly positive hypersensitive to tuberculin protine - SUGGESTIVE OF TB INFECTION
363
Ziehl Neelson Stain?
TB - stain
364
TB meningitis?
STart 4 x Antibiotics for TB | Then start CEF and Vanc and Dex as well to treat for Bacterial Meningitis too
365
Psoriasis triggered by which drugs?
``` Lithium Beta Blockers Antimalarials Non Steroidal anti inflammatory Ace I Tetracyclines and Penicillins ``` TRauma and Alcohol Strep = Guttate Psoriasis
366
Low Testosterone next test
FSH and LH If LH and FSH are High then it would suggest a Primary cause of hypogonadism If LH and FSH are low then suggests secondary If Primary: - Klinefelter's - Kallman - Childhood Mumps - Testosterone levels declining with age IF Secondary needs: - Prolactin - Morning Cortisol - MRI of Pituitary
367
Hypogonadism symptoms
``` Loss of libido Erectile Dysfunction Lethargy Decreased Muscle mass and Strength Reduced facial hair growth Impaired Glucose Tolerance ```
368
Tabes Dorsalis
``` Late Consequence of NEurosyphillis Slow degeneration (demyelination) of neural tracts of the dorsal root ganglia of the spinal cord ``` Trep Palladium 20 years after infection Radicular parethesia Thunder bold pain in limbs, back and face Broad based foot slapping gait - senosry ataxia Loss of reflexes in lower limbs Argyll Roberson Pupils - small pupil, loss of light reaction but preserved accomodation
369
Myeloma Staging
Done with B2 - microglobulin and albumin
370
Toulene Toxicity
Sniffing Glue Increases Dopamine- euphoria ``` Irritation of the eyes, nose and respiratory tract from inhalation. Ataxia Confusion Headache Euophoria ```
371
Melasma
Condition of hyperpigmented macules in sun exposed areas. | Found in women on Hormone therapy Pill or Pregnant
372
Malt Lymphoma
Gastric Malt Lymphoma H Pylori infection in 95% of cases Good Prognosis If low grade then 80% respond to H Pylori Eradication
373
Pre Renal Azotemia
Urine Na < 20 Urine Osm > 500 Serum urea: Creatinie ration Increased Urine: Plasma Urea > 10
374
Rabies Exposure?
Give IVIG Whatever the time between the exposer then the 5 dose schedule of Rabies Vaccination 0, 3, 7, 14, 28 days
375
Cyproheptadine?
5-HT2 Receptor Antagonist USed in Seretonin Syndrome Resistant to initial control with oral diazepam Following this with respiratory failure, coma, rigidity or severe hyperthermia - Intubate and Ventilate with Chlorpromazine
376
Pontine Haemorrhage
Reduced GCS, Paralysis, Bilateral Pin Point Pupils | First ORder Sympathetic tracts - bilateral horners syndrome
377
Midbrain haemorrhage/ Lesions
Fixed, Mid point pupils
378
Lateral medulla
Ipsilateral hornders syndrome and pin point pupil
379
Wallenberg Syndrome?
Lateral medullary syndrome: Medulla is supplied by vertibral artery and PICA Stroke leads to: - Spinothalamic damage- contralateral pain and temperature loss - Hypothalamic spinal damage - Ispilateral horners - Ipsilateral Ataxia (spinocerebellar tracts) Cranial nerve involvement: - Hoarse voice and dysphagia (cranial nerves IX - XII Vertigo (VIII) Facial parathesia - Cranial nerve V root is here too
380
Lupus
Anti Nuclear Antigens Antigen Antibody Complex then leads to build up in organs and damage Fever Joint Pain Rash in Woman Multi organ therefore hard to diagnose: 4 of these 11 - Malar Butterfly rash - Discoid Rash - Photosensitivity - Ulcers Pleuritis / Pericarditis Arthritis Kidney Involvement Neurological Disorders Anaemia, Thrombocytopenia, Leukopenia Antibodies: - ANA positive (very sensitive) - Anti Smith - Anti dsDNA - Anti Phopspholipid ``` Trigger UV radiation Estrogen Virus Bacteria Drugs ```
381
Anti Ro/ Anti La
Sjogren's syndrome | Anti Ro can cross placenta and cause congenital heart block which can reuquire pacing at birth
382
Anti CCP
Rhuematoid
383
Shingles Vaccine?
Available for people 70-79 Live Attenuated Immunosuppression therefore a contra indication Management of acute shingles is with oral acyclovir
384
Somogyi Effect
Take insulin before bed and wake up with high blood sugar levels (rebound high) Paradoxically you should lower the insulin before bed Too low overnight then get a rebound secretion of glucagon etc to increase blood sugars
385
Hepatic Encephalopathy
Treat Precipitating Cause Lactulose Rifamixin (used in bacterial overgrowth too) Lactulose promotes excretion of ammonia causing gut bacteria to metabolise it Antibiotics like rifamixin modulate gut flore so reduced ammonia production
386
Barrets oesophagus with low grade dysplasia
Treatment with radiofrequency ablation is usually well tollerated and successful Surveillance should be done endocsopically every 6 months for first year then annually after that If High grade dysplasia or intra mucosal carinoma, then every 3 months for first year, 6 months for sedond year and then annually
387
Ataxia Telangectasia
Autosomal recessive disorder with defect in the ATM gene Causes one of the inherited combined immunodeficiency disorders presenting with abnormal movements in childhood at age 2 years old Cerebellar Ataxia Telangectasia IgA deficiency Increased risk of Lymphoma na dluekaemia Hypogammaglobulinaemia
388
Chaga's disease
Trypanosoma Cruzi from South America 2 forms of Trypanosomiasis - African (sleeping sickness) and Chaga's American Trypanosomiasis is caused by T Cruzi Chagoma (erythematous nodule at site of infection) Perioorbital swelling (romana sign) Heart and GI tract effect chronically Myocarditis with dilated cardiomyopathy Gastointestinal is mega oesophagus and mega colon that causes constipation and dysphagia AZOLE for Treatment First line is Benznidazole
389
African sleeping sickness
Trypanosoa rhodesiense is by the Tsetse fly. More acute Chancre - nodule at site of infection Posterior cervical lympho nodes Then CNS involvement - Sleepyness, headaches, mood changes, meningo ecnephalitis IV pentamidine IV melarsoprol `
390
Drugs that cause lung fibrosis
``` Amiodarone Bleomycin, Bromocryptine Cabergoline DMARDS - Methotrexate, Sulfasalazine Nitrofurantoin ``` `
391
Posterior MI
R Wave TALL in V1 and V2
392
Caplan's Syndrome?
Interstitial lung disease in coal miners with RA. Muliple nodules on CXR
393
MODY
Maturity onset Diabetes of the Young Esentially this is T2 DM in < 25 year old person Autosomal dominant so there is lots of family history MODY 3 = 60% HNF1 alpha gene defect Increased risk of HCC MODY 2 = 20% of cases Due to glucokinase gene defect Treatment is with sulfonylureas C peptide is good marker of insulin production and can reule out Type 1 DM If C Peptide is high, then is T 2 as there is insulin production just not enough
394
Amiodarone and Thyroid Gland
1/6 people on amiodarone get thyroid problems Amiodarone induced hypothyroidism Amiodarone induced thyrotoxicosis is in 2 types Type 1 - Excess iodine causes excess hormone production. This has a Goitre and is treated with Carbimazole Type 2 - Amiodarone causes destructive thyroiditis. Destruction on US, no goitre and treated with STeroids
395
Reversible cerebrovascular vasoconstriction syndrome
Cause of Thunderclap headhache (max intensity within minute 10/10) with normal CT and LP (aka not SAH) Vasoconstriction after Cannabis, post partum or seretonin Throbbing headhace and seizures Beading on CT A Can cause bleeds and strokes
396
Posterior Reversible Encephalopathy syndrome
Cause of Thunderclap headahce with normal CT and LP (AKA Not SAH) Swelling of brain usually as a result of underlying cause. Headhace, changes in vision and seizures. High BP, CKD, Infection, Autoimmune disease and Pre Eclampsia are the usual causes
397
Thunderclap headache
``` SAH Cerebral Venous Sinus Thrombosis Artery Dissection Pituitary apoplexy Reversible Cerebral Vasoconstriction Posterior Reversible Leucoencephalopathy syndrome Hypertensive crisis ```
398
Pituitary apoplexy is bleeding into or impair blood supply to the pituitary
Caused by tumor most commonly but 80% not diagnosed previously Sudden headache, visual field defect MRI imaging May have normal CT
399
Lupus Nephritis?
Best Treatment for Lupus Nephritis is Mycophenolate Mofetil Start by treating with steroids and cyclophosphamide then switch to mycophenolate long term
400
Scabies failure of treatment
BAsically Scabies if treated properly should not have any burrow markes or tract marks. There may be pruritis up to 6 weeks after due to allergic reaction. However, if failed treatment with permethrin 5% and everything done properly - then treat with different insecticide. Like Malthion 0.5%
401
Achalasia
Management: - Nifedipine Heller Cariomyotomy Botox Baloon Dilation
402
Crohn's Disease
Start with steroids Induce remission Then start Azothiprine or Metocaptopurine Before starting this check TPMT If TPMT low then need to not give above and give Methotrexate instead If early severe disease or resistant disease consider infliximab Metrionidazole for peri anal disesae Mesalazine should be considered in previous surgery
403
Haemochromatosis screening?
Transferrin Saturation NOT FERRITIN - Acute phase reactant Trans ferritin Sats > 55% Raised ferritin and Iron Low TIBC Venesection is the first line treatment Following this Desferrioxamine is second line Aim Transsferrin sats < 50% and Serum ferritin below 50
404
Lung Cancer Associated with Paraneoplastic syndromes?
Small Cell Lung Cancer - Lamber-Eaton Myasthenic Syndorome Antibody against pre synaptic voltage gated calcium channel Causes weakness that gets better with repeated movements - Anti Hu Syndrome- Sensory neuo[athy and cerebellar syndrome - anti GAD Still persons syndrome Anti Ri Ocular opsoclonus- myoclonus
405
Anti Yo
Associated wtih ovarian and breast cancer | Cerebellar syndrome
406
Anti GAD
Breast, colorectal and small cell lung cancer | Stiff person's syndrome or diffuse hypertonia
407
Anti Ri
Ocular opsoclonus myoclonus | Breast and small cell lung cancer
408
Ritonavir
Anti HIV drug inhibits P450 system | May increase seretide levels or fluticasone therefore make you more likely to have adrenal insufficiency
409
p450 inducers
PC BRAS - induce (Fits, TB, DM, Alcohol) ``` Phenytoin Carbamazepine Barbiturates Rifampicin Alcohol (long term) Sulphonylureas (gliclazide)/ St John's Wart ``` These reduce the other drugs concentration metabolised by cyctocrome P450 aka Warfarin, Steroids, COCP
410
p450 inhibitors
O DEVICES ``` Omeprazole Disulfiram Erythromycin Valpropate Isoniazid Ciprofloxacin Ethanol (acutely) Sulfonamides (Sulfasalazine) ``` These increase the other drugs concentration metabolised by cytocrome p450 aka warfarin, steroids, cocp
411
Gottron's papulses?
Rough patches seen in dermatomyositis. They are seen on start of the extensor aspect of fingers. (MCP and PIP Joint) These are accompanied by heliotrope rash around the eyes This is either idiopathic dermatomyositis or secondary to malignancy- this is common
412
Erythema Gyratum Repens
Annular erthema Looks like dried red larva 'wood grain' Seen in malignancy Lung Cancer
413
Dermatomyositis
Symmetrical proximal mm weakness with skin lesions Inflammatory process Idiopathic or malignancy Ovarian, breast and lung cancer Polymositis is without skin involvement ``` Skin: Photosensitive Gottrons Heliotrope rash periorbitally Macular rash - back and shoulder Nail fold capillary dilatation ``` ``` Proximal mm weakness Resp mm weakness ILD Raynauds Dysphagia/ Dysphonia ``` ANA positive Anti Jo 1 Antibodies Anti Mi - 2 Antibodies Anti SRP antibodies
414
Cystinuria
Autosomal Recessive Disorder by the formation of recrrent renal stones COLA defect Semi opaque on x ray Need a cyanide nitroprusside test to diagnose Treatment with Hydration D pinicillamine Urinary Alkalinization (Bicarb)
415
Intrahepatic cholestasis of pregnancy
``` Seen in 2nd Trimester - 3rd trimester Itchy feet ALT < 3 x normal Bile Acids +++ (10-100x) Ursodeoxycholic acid and delivery (Induce at 37 weeks) ``` No Rash No Jaundice but raised bilirubin
416
Acute Fatty liver of Pregnancy
3rd Trimester or post partum ``` Abdominal Pain N +V Headache Jaundice HYPO Glycaemia Pre Eclampsia ``` ALT very raised Supportive treatment and delivery of the child
417
If New drug added in DM?
Aim for Reduction in HbA1c by 11% Aiming for HbA1c < 48 (6.5%) Add another drug is > 58mmol (7.5%)
418
Anti CCP?
Rheumatoid Arthritis
419
Amytrophic Lateral Sclerosis
Mixture of upper motor neurone and lower motor neurone features PSeudo-Bulbar ' Donald Duck ' speech - nasal, high pitch Tongue Fasciculations Treatment of MND: NEurological condition that can present with both upper and lower motor neuron signs. Resents > 40 Amyotrophic lateral sclerosis, progresive muscular atrophy and bulbar palsy Treatment Riluzole - stimulates glutamate receptors Prolongs life by 3 months Non invasive Ventilation BIPAP at night 7 months added on
420
Efavirenz toxicity
Neuropsychiatric toxicity Psychosis Is cp450 drug
421
Ethambutol Toxicity
Ethambutol - E - Eye Optic neuritis Colour vision loss first sign
422
Rifamipicin
Causes red secretions
423
Isoniazid
Peripheral neuropathy Pyridoxine Hydrochloride Vitamin B6 Used to prevent this and sideroblastic anaemia Also used with penicilamine D for prevention of neuropathy in wilsons patients
424
Nephrogenic Diabetes insipidus? Treatment? Diagnosis
Water Deprivation test - urine osm stays exactly the same throughout Treatment with Thiazides and Low Salt/ Protein Diet
425
Paraquat Overdose Testing
Urine Dithionate Testing Try to confirm or exclude exposure to paraquat Positive within 6 hours and remains for a few days Administer Fullers Eart before result is back
426
Dukes Cancer Staging?
Dukes' classification and 5-year survival rates Stage 5-year survival A - confined to mucosa and submucosa 95% in men and 100% in women B - extends through the muscularis propria > 80% in men and 90% in women C - regional lymph nodes involved 65% in men and 65% in women D - distant spread > 5% in men and 10% in women
427
Efaverinz side effects?
Anti HIV med thtat causes cognitive/neuro sypmtoms when starting in 50% of patient sin the first month
428
In Thyrotoxicosis which drugs should you stop?
Amiodarone | Aspirin- can make it owrse
429
Leprosy Treatment?
Management WHO-recommended triple therapy: rifampicin, dapsone and clofazimine RDC Rif, Dap, Clof Think hypopigment and sensation loss Alopecia Foreign travel Slow growing
430
Medullary Thyroid Cancer Tumour Marker?
Calcitonin
431
MElanoma Tumour marker?
s100 | `
432
Upgoing plantars and absent ankle jerks?
``` subacute combined degeration of the cord SCDC Montor Neurone Disease Friedrich's Ataxia Tabes Dorsalis Dual Peripheral with central pathology ```
433
Rheumatoid Arthritis Management?
``` Monotherapy Monitor CRP and DAS28 score to see response Flares - steroids Methotrexate is most widely used Sulfasalazine Hydroxychloroquine ``` If there is an inadequate response to 2 DMARDs (including Methotrexate) then start TNF alpha inhibitor: Entanercept (can cause demyelination and reactivation of TB) Infliximab (Reactivation of TB) Also can give Rituximab - Anti CD20 monoclonal antibody resulting in B cell depletion
434
Lung Cancer Management:
Non Small Cell - Radio therapy mainly 20% are suitable for surgery ``` ? Fit for surgery - Peripheral tumour No malignant effusion, vocal cord involvement, SVC obstruction FEV1 > 1.5 L for Lobectomy FEV1 > 2 L for pneumonectomy ``` Small Cell - Poor Prognosis - Treatment offered for 1 - 2 disease - Chemo and Radiotherapy usually 10 % 5 year survival from Lung Ca
435
Rotterdam Criteria for Diagnosis of PCOS?
Clinical or biochemical evidence of hyperadrogenism Evidence of oligo or an ovulation Presence of polycstic ovaries
436
Management of PCOS
Weight Reduction COCP Acne/ Hirsutism COCP Spironolactone Infertility Weight loss Clomifene Consider Metformin too
437
Thrombus visualised on Stroke but on going haemorrhagic transformation>
Start Heparin infusion This is quickest to reverse Neuro obs Stop if further deterioration ie re bleeds
438
Alcoholic Hallucinosis
Alcoholic Hallucinosis is a rare condition that occurs in intoxication or withdrawal of alcohol. Consciousness in tact Auditory Hallucinations heared Resolve in 6 months
439
Treatment of Glioblastoma?
Chemo-Radiotherapy | Surgical
440
Treatment of Infertility in Kallmann's syndrome?
HCG - stimulate the gonads FSH- Stimulate ovulation Then once ovulated- Harvest the Eggs and Start IVF
441
Treatment of Abscence Seizures?
Valproate and Ethosuximide Avoid CarbeMAZEapine Makes Abscence and Juvenille myoclonic epilespy worse.
442
Keratoacanthoma
Benign epithelial tumour Advancing age Smooth Dome Volcano
443
Anterior Uveitis
``` Acute onset Pain Pupil small and irregular Photophobia Blurred Vision Red Eye Hypopyon ``` ``` Ankylosing Spondylitis Reactive Arthritis Ulcerative Colitis Becet's Disease sarcoidosis ``` Atropine Steroids
444
Aortic Stenosis Clinical Findings
``` Narrow pulse pressure Slow Rising Pulse Delayed ESM Absent S2 S4 ESM decreased following valsava ```
445
Autoimmune Haemolytic Anaemia
Direct Coombs Test Warm - IgG positive SLE, CLL, Lymphoma, Methyldopa Cold- IgM positive Lymphoma, Mycoplasma, EBV
446
Waldenstrom's Macroglobulinaemia
``` Older MEn IgM Paraproteinaemia Weight loss Lethargy Hyperviscous Heptamosplenomegaly Lymphadenopathy Raynauds ```
447
AML
``` Myeloid failure RBCs, Platelets and Neutrophils low Plenomegaly Splenomegaly Infections Fatigue ``` ``` Acute Promyelocytic Leukaemia M3 t(15:17) PML and RAR alpha gene fusion Young people < 25 Good Probnosis ```
448
Thiazides electrolyte distrubance?
Hypercalcaemina
449
Inferior Quadrantanopia - homonymous hemianopia and bi temporal hemianopia?
PITS - homonoymous Parietal is Inferior Temporal is Superior Craniopharyngioma is inferior homonymous quadrantanopia
450
Superior Quadrantanopia- unilateral and bilateral?
Unilateral = Temporal lesion Bilateral = Pituitary
451
Hereditary Spherocytosis
Most common haemolytic anaemia Autosomal Dominant Biconcave disc is now sphere Failure to thrive Jaunic,e Glalstones Splenomegaly APLASTIC CRISIS precipitated by PArvovirsu infection Osmotic fragility test previously recommended but no longer Patients with FH, Typical features and lab tests don't require any other tests Cryoheaeolysis and EMA binding if needed Acute Hammolytic crisis - suporitve Transfusion Folate replacement and Splenectomy in the long run
452
Gilteman's Syndrome
Hypokalaemia and Normotension
453
Liddl'es Syndrome
Hpokalaemia and Hypertension
454
Bartter's Syndrome
Hypokalaemia and Normotension
455
Causes of Hypokcalaemia and Normotension
``` Diuretics Gilteman Bartter's RTA 1 + 2 Diarrhoea ```
456
Hypokalamiea and Hypertension
Cushings Liddle's Primary Hyperaldosteronism 11 beta hydroxlase deficiency
457
Hashimotos Thyroiditis
Chronic Autoimmune Thyroiditis Hypothyroidism after transiet hyperthyroidism Female Hypothyroid Goitre- firm non tender Anti thyroid peroxidase and anti thryoglobulin antibodies present Development of MALT lymphoma Other Autoimmune conditions common
458
Vitamin B 1?
Thiamine
459
Ropinirole?
Restless legs dsyndrome and Parkinsons
460
Riluzole
MND
461
Writer's Cramp/ Focal dystonia?
Flexion/Extension/rotation of Muscles of the hand. Suddenly | Relieved by stimulating another body part
462
Alendronate upper GI side effects?
Swap to Risedronate or Etidronate
463
Scleroderma Antibodies
scl 70 = Diffuse | Anti Centromere Antibodies = Limited
464
Enzyme Inhibitors
SICKFACES. COM ``` Sodium Valproate Isoniazid Cemetidine Ketaconazole Fluconazole Alochol Chloramphenicol Erythromycin Sulphonamides Ciprofloxacin Omeprazole Metronidazole ```
465
Scleroderma Antibodies
scl 70 = Diffuse | Anti Centromere Antibodies = Limited
466
Loiasis:
African Eye worm 2 symptms Calabar Swellings around hands and arms that move - angio edema Eye worm moving across the eye ball Treatment: Removal DEC - diethylcarbamazine or Albendazole
467
PPAR Gamma Agonist?
Pioglitazone USed in severe NAFLD with cirrhosis even in the abscene of diabetes
468
Non Alcoholic Fatty liver Disease
Steatosis - Fat in liver Steatohepatitis - Fat in liver with associated inflammation (ALT up) Progressive disease to fibrosis and cirrhosis Hepatic Manifestation of metabolic syndrome - Insulin resistence Non Alcoholic Steatohepatitis NASH is used to show changes similar to alcoholic hepatitis in the absence of alcohol abuse. ``` 4% population have NASH - Obesity T2DM High Cholesterol Sudden Weight loss ``` Increased echogenicity on ultrasound and ALT greater than AST Enhanced liver Fibrosis blood test to check. > 10.5 is suggestive of Advanced cirrhosis if ELF not avaialbe FIbroScan - Liver stiffness on US Biopsy is the next stage Weightlos and lifestyle measurements Pioglitazone
469
Deferiprone
Iron Chelating agent used in haemochromatosis treatment
470
In pregnancy management of Haemochromatosis?
Monitor Cardiac and liver function ``` Pregnancy will lower iron Monitor Ferritin If normal, observe If low treated iron deficiency If high phlebotomy ```
471
Polychondritis
Inflammatory process of cartilage Effects the ears and nose commonly with pain and inflammation No specific antibodies Can have scleritis, episcleritis and arthropathy as well
472
HIT confirm diagnosis
If > 50% in 7 days but another alternative diagnosis (sepsis) Then do Anti Platelet 4 antibodies if moderate or low positive Then final test is Seretonin release assay - this is highly specific
473
Carcinoid syndrome?
From a neurendocrine tumour that then secretes neurotransmitters Needs to be in the liver to have carcinoid syndrome Seretonin is one of the neurotransmitters secreted - Diarrhoea (increased motility) - Tricuspid Regurgitation (mid diastolic murmur) - due to fibrosis of the heart - Bronchoconstriction Histamine and bradykinin also released This leads to flushing (red Face) and Itching There is reduced trptophan and therefore Vitamin b3 levels drop This can cause Pellagra (diarrhoea, dermatitis, dementia Octreotide scan and CT can show the tumour site Urinary 5 hydroxyureindoleacetic acid 5HIAA Somatostatin analogls like ocretotide
474
HHV 5 treatment post kidney transplant?
HHV 5 is CMV Can be from latent activation, infection via transplanted organ or new infection IV ganciclovir is needed Withouth this Renal function likely to deteriorate
475
Zanamavir
Treatment of Influenza in Immuno comprimised | Oseltamivir is in Immunocompetant
476
HHV 5 treatment post kidney transplant?
HHV 5 is CMV Can be from latent activation, infection via transplanted organ or new infection IV ganciclovir is needed Withouth this Renal function likely to deteriorate
477
Zollinger Ellison Syndrome
Excess Gastrin Likley due to duodenal or gastic tumour secreting gastrin (can be pancreas) 30% from MEN 1 Multiple Ulcers Diahrroea Malabsorption Need Gastrin Level Then need to do a secretin stimulation test This will cause the gastrin to go up
478
Colonoscopy Risk Criteria
Low Risk - 5 years colonsocopy 1 or 2 adenomas < 10mm Intermediate risk - 3 years (x 2 ) 3 - 4 adenomas < 10mm or 1-2 Adenomas > 10mm High Risk - 1 year (then 3 years x 2) 5 + < 10mm 3 + > 10mm