PACES Viva Questions Flashcards

Question

Describe the use of non-invasive ventilation (NIV) in the treatment of acute exacerbations of COPD.

Answer 1

Bi-level positive airway pressure (BiPAP) provides pressure support via a facemask with higher inspiratory positive airway pressure (IPAP) than expiratory positive airway pressure (EPAP) - Ventilators differ in delivering either a selected volume or pressure and can be timed or patient-triggered, with back-up rates if the patient fails to breathe. - NIV improves oxygenation, increases the removal of CO2, increases functional residual capacity, increases tidal volume, improves lung and chest wall mechanics and decreases respiratory effort. Compared to conventional medical care NIV has been shown to: - Decrease the need for endotracheal intubation - improve pH and respiratory rate - Result in fewer complications (principally ventilator associated pneumonias) - Result in shorter lengths of hospital stay - Reduce mortality Indications: - NIV should be considered in patients who despite maximal medical treatment and controlled oxygen therapy have a respiratory acidosis (pH less than 7.35) - patients should be able to consent to treatment and have potential for recovery to a quality of life that is acceptable to the patient. - relative exlusion criteria include: life-threatening hypoxaemia, severe comorbidity, confusion, facial injuries, vomiting, fixed upper airway obstruction, inability to protect airway, undrained pneumothorax, haemodynamic instability, bowel obstruction or upper gastrointestinal surgery. - in patients in whom endotracheal intubation is inappropriate there is flexibility for these guidelines, for example an unconscious patient secondary to COPD induced hypercapnia. Commencing NIV: - At the time of commencing NIV a decision regarding the most appropriate ceiling of care for the patient should be documented (e.g. should they be intubated and mechanically ventilated if NIV fails) and a resuscitation decision should be made. - Initial pressures recommended are 12-16cmH2O and EPAP 4-5cmH2O with oxygen adjusted to reach target saturation’s 88-92%. Pressures are adjusted depending on patient tolerability and clinical response. Patients should be closely monitored with repeat blood gases 1 hour after commencing of NIV or following change in settings.

Answer 2

- CPAP maintains these same pressure support throughout the breathing cycle, splinting open the upper airways, recruiting collapsed alveoli (Thus improving lung compliance) and reducing ventilation/perfusion mismatching. - It is used for the treatment of acute pulmonary oedema, obstructive sleep apnoea and Type 1 respiratory failure.

Answer 3

It is an acute, rapidly progressive lung fibrosis also known as acute interstitial pneumonia (AIP). It may respond to steroids but has a poor overall outcome (6-month survival 22%)

Answer 4

There are 3 main types of CT Scanning: High-resolution Computed tomography (HRCT: 1-1.5mSv): - involves taking very thin sections (1mm) with high spatial frequency re-constructions with ‘skipped’ areas between the thin sections (1mm every 10mm therefore produces sampling of the lung but not continuous imaging) - Images obtained in inspiration and expiration and additionally whilst supine and prone(to exclude dependant change seen on scans which can be mistaken for pathology/disease) - Most useful for assessment of interstitial processes such as fibrosis or emphysema and endbronical changes with ‘Tree in bud’ appearances. Dynamic (Standard CT:8mSv): - involves taking thicker sections (10mm) which are then used for reconstruction (Provides continuous images in contrast to HRCT). - intravenous contrast may be used as it clarifies anatomy and boundaries of the great vessels and improves assessment of the mediastinum and hilar regions for lymphadenopathy. - most useful or assessment of airspace disease (pneumonia or malignancy) and mediastinal pathology (Lymph nodes in staging) Computed Tomography pulmonary angiogram (CTPA: 8mSv): - involves very thin sections (Can be variable e.g. 0.5-0.625mm) in a continuous fashion to obtain images of the pulmonary arteries using multi-detector CT scanning (MDCT) - Main diagnostic investigations to detect pulmonary embolism.

Answer 5

Treatments options include the following: - Corticosteroids (factors contributing to lack of response include male sex, severity of symptoms or worse lung function on presentation, neutrophilia on BAL or predominant honeycombing on CT) - Immumnosuppresives (Cyclophosphamide, azathioprine, methotrexate, and cyclosporine) - Anti-oxidant (N-acetylcysteine in conjunction with corticosteroids and Azathioprine) - Supplemental Oxygen (PaO2 less than 7.3kPa or 8kPa with cor pulmonale) - Lung transplantation (60% 1 year survival)

Answer 6

Agents acting as anti-fibrotic have been suggested for use in the management of pulmonary fibrosis. Evidence for their use is limited and therefore they are rarely used in clinical practise e.g. pirfenidone. Other drugs that may be of use include bosentan and warfarin due to their role in pulmonary hypertension.

Answer 7

The median survival is 2.8 years. 5-year survival 50% (60% in steroid responders and 25% in non-responders). Favourable prognostic factors include the NSIP subtype.

Answer 8

Hypersensitivity Pneumonitis Sarcoidosis Organising pneumonia Lymphocytic interstitial pneumonia (LIP)

Answer 9

There are 5 main respiratory manifestations of rheumatoid arthritis. It is however to important to note that nowadays rheumatoid arthritis related lung disease is seen much less frequently due to earlier and more aggressive treatment regimes: Pleural effusions: - males more than females - Usually asymptomatic; respiratory symptoms will only occur if effusion large enough - Characteristically low glucose and rheumatoid factor +Ve effusions. Pulmonary nodules: - May be seen on radiography and precede arthritis onset. - usually asymptomatic but may become infected, cavitation, or cause haemoptysis. - predilection for upper lobe location Fibrotic lung disease: - 2% of all cases become symptomatic, however subclinical disease is common with a reduced DLCO on PFTs and UIP pattern on HRCT. - Can result directly from disease or secondary to treatment (Methotrexate/gold). If secondary to treatment, changes may regress on discontinuation of the offending drug. Caplan Syndrome: coal worker pneumoconiosis and rheumatoid arthritis Obliterative Bronchiolitis: - Rare manifestation involving small airways obstruction which progress to necrotising bronchiolitis - previously occurred in those treated with gold or penicillamine.

Answer 10

Maddrey’s discriminators function test was described by Maddrey Et al in 1978 to predict prognosis in alcoholic hepatitis: - It is calculated by 4.6 x INR + Serum Bilirubin - during the episode a score over 32 carries a 50% mortality rate and is an indication for treatment with steroids, where as over 90% of patient less than 32 will survive. The Mayo End stage liver disease (MELD) score has been applied to alcoholic hepatitis. This is a composite score deceived from serum bilirubin, creatinine and INR and predicts survival probability over 90 days. In one study, patients with a score less than 11 had a 30-day survival of 96% whilst the 30-day survival in those with a. Score over 11 was 45%. The Glasgow alcoholic hepatitis score on day 1 has an overall accuracy of 81% when predicting 28-day outcome.

Answer 11

- The mainstays of management are supportive and include: abstinence from alcohol, adequate nutrition, and treatment of any inter current infections. - liver biopsy may be indicated to confirm diagnosis - Maddreys Discriminant function score over 32 is an indication for treatment with steroids. 40mg oral prednisolone is frequently used. Other drugs which may be useful include pentoxifylline

Answer 12

- hepatic steatosis, accumulation of fst in liver cells, This is not related to the total alcohol exposure. - alcoholic hepatitis, acute inflammation and hepatocyte necrosis - hepatic cirrhosis, fibrosis of liver tissue - while cirrhosis is irreversible and If progressive can lead to liver failure , both steatosis and hepatitis are potentially reversible following abstinence from alcohol

Answer 13

Hepatic amyloidosis is characteristically associated with loss of the clotting factors IX and X. In addition, infiltration with amyloid protein contributes to vascular fragility. There is significant risk of bleeding if percutaneous liver biopsy is performed and spontaneous hepatic rupture has been described

Answer 14

Complications of CLD include: - Portal Hypertension - Haemorrhage, usually GI ( gastric ulcers and erosions still occur more frequently than variceal bleeding - Ascites - Spontaneous Bacterial peritonitis - hepatic encephalopathy - hepatorenal syndrome - hepatopulmonary syndrome

Answer 15

- the liver can compensate for a significant amount of hepatocyte injury but can decompensate as a result of ongoing liver injury (continued alcohol intake or untreated chronic active viral hepatitis) or additional stress (e.g. GI bleeding, large salt intake, sepsis, constipation, dehydration) - clinically, decompensated CLD will present with a combination of ascites, encephalopathy, hepatorenal or hepatopulmonary syndrome in an acute setting

Answer 16

Grade 1: Mild Confusion, euphoria or depression; Short attention span and impaired mental tasks such as addition, disordered sleep patterns Grade 2: drowsiness, lethargy, mild disorientation mainly for time but also sometimes for place, inappropriate behaviour and personality change, impaired mental tasks such as subtraction Grade 3: somnolent but rousable, usually with verbal stimuli sufficing. Grossly confused and disorientated , speech may be incomprehensible amnesia often present Grade 4: comatose, no response to verbal or painful stimuli

Answer 17

The most commonly used grading system for grading the severity of cirrhosis is the modified Child-Pugh system which scores clinical and biochemical parameters out of three to obtain a composite score. ``` Encephalopathy: none, stage 1-2, stage 3-4 Bilirubin: less than 34, 34-50, over 50 INR: less than 1.7, 1.7 -2.3, over 2.3 Ascites: None, Mild, Severe Albumin: over 35, 28-35, less than 28 ``` Note that PSC and PBC are characterised by higher levels of bilirubin. Therefore in these conditions the criteria for bilirubin are modified to less than 68, 68-170 and over 170 Severity is classified according to the Child Pugh score into Class A (5-6) Class B (7-9) or class C (10-15) corresponding to 1-year survivals of 100%, 80%, 50% respectively.

Answer 18

Jaundice can be classified as: - Prehepatic due to excessive breakdown of red blood cells e.g. autoimmune haemolytic anaemia, malaria, hereditary haemoglobinopathies - hepatic due to hepatocyte injury, e.g. acute viral hepatitis, paracetamol, alcohol, hypoxic/ischaemic - Post-hepatic due to obstruction to the normal flow of bile e.g. gallstone obstruction, pancreatic head malignancies

Answer 19

Hepatorenal syndrome results from inadequate hepatic breakdown of vasoactive substances, leading to excessive vasoconstriction. It can devolop rapidly (type 1) or slowly (type 2) and mimics pre renal renal impairment. As the kidneys attempt to conserve maximum salt and water in response to perceived hypovolaemia, the patient produces low volumes of highly concentrated (high osmolar) urine that is low in sodium. It is usually a diagnosis of exclusion, having given a fluid challenge and ruled out obstruction, sepsis and nephrotoxic Drugs. The kidneys of patients with hepatorenal syndrome are usually normal and in the acute setting may be salvaged. In general, if the EGFR is less than 30 in the pre-morbid state, combined liver and kidney transplantation is usually undertaken in favour of single liver engraftment as peri operative injury and postoperative immunosuppressive usually leads to loss of the remaining kidney function

Answer 20

- a non salt added diet should be started ( 90mmol or 5.2g salt/day) - spironolactone from 100-400mg/day, in all patients able to tolerate the drug without excessive hyperkalaemia - loop diuretics should be initiated next usually furosemide from 40-160mg/day - if these measures fail or if the patient has significant symptoms related to the ascites therapeutic paracentesis should be carried out. - radiological procedures such as transjugular intra-hepatic portosystemic shunt procedure may help to relieve portal hypertension but the risk of encephalopathy is increased as nitrogenous waste effectively bypass the liver with a resultant increased concentration in the systemic circulation. - surgical measures (portosystemic and peritoneovenous shunts) are much less frequently used these days but still play a role in recurrent ascites. - liver transplantation is a curative procedure for end-stage liver disease when medical management fails

Answer 21

Stage 1 Investigations: - Urine: Glucose, Protein - Haematology: FBC, ESR, Vitamin B12 and folate levels - Biochemistry: fasting blood glucose level;”, HbA1c, renal function, liver function, TFTs Stage 2 Investigations: - the most important stage 2 investigation is neurophysiological investigation. About 80% of symmetrical peripheral neuropathies are axonal. - electrophysiological investigation (EMG and NCS) for assessment of distal and proximal nerve stimulation - Serum electrophoresis, serum ACE - immunology: ANA, Antiextractable nuclear antigens (Anti-Ro, Anti-La) ANCAs - CXR Stage 3 Investigations: - Urine: Bence Jones Protein - Biochemistry: oral glucose tolerance test - CSF: Cells, Protein, Immunoglobulin oligoclonal bands - Immunology: HIV antibodies, antineuronal antibodies ( Anti-Hu, Anti-Yo) antigliadin antibodies, antiganglioside antibodies, antimyelin associated glycoprotein antibodies - Tests dor Sjögren’s syndrome (Schirmers rest, rose Bengal test, salivary flow rate, labial gland biopsy - search for carcinoma, lymphoma, or solitary myeloma, skeletal survey, CTCAP, PET - molecular genetic tests: peripheral nerve myelin protein 22 gene duplication (the commonest cause of Charcot-Marie-tooth disease type 1) or deletion (hereditary neuropathy with liability to pressure palsies) connexin 32 mutation (X Linked Charcot-Marie-Tooth disease) PO gene mutation (another cause of Charcot-Marie-Tooth disease type 1)

Answer 22

These would include: Neuromuscular junction disorders: - Myasthenia gravis( look for complex ophthalmoplegia, ptosis, fatiguability) - Lambert-Eaton myasthenic syndrome (associated with SCLC- Cachexia, post titanic potentiation Distal Myopathies; - Myotonic dystrophy (frontal balding, facial muscle wasting, cataracts, learning disability, cardiac pacemaker, percussion myotonia) - inclusion body myositis (wasting and weakness of finger and wrist flexors (scalloping) knee extensors and ankle dorsiflexors. Weakness of the wrist and finger flexors insistem disproportionate to that of extensor counterparts. Hence loss of finger dexterity and grip strength may be a presenting or prominent feature

Answer 23

Aortic Dissection. This is the most feared complication as the mortality is at least 50%whereas the mortality of elective aortic root replacement is 5%.

Answer 24

The major objective of follow-up is to avoid acute aortic dissection, a yearly echo should be done to monitor the aortic root, and if there is a family history of aortic dissection or the aorta has started to dilate, this should be more frequently.

Answer 25

Autosominal dominant with complete penetrance, but very variable clinical expression. Even within a given family the manifestions can be quite variable between families there is great variation due to different mutations in the fibirillin gene. Ring present,

Answer 26

ADPKD is phenotypically inherited in an autosomally dominant manner. In fact, the mutated gene is recessive to the normal allele and cysts form only in renal tubular cells where a stochastic event has lead to a mutation of the normal allele (in the same way as it the case with childhood retinoblastoma). The usual screening test, for those who wish to have it, is an ultrasound scan of the kidneys. However as renal cysts develop with ageing, a negative ultrasound below the age of 30 does not exclude the disease. When to screen a patient is a question of considerable debate. As there is no specific treatment for ADPKD, it can be argued that regular B checks and treatment is preferable to ultrasonic confirmation of the conditions. Others argue that the diagnosis of ADPKD should be established in everyone who is of child bearing age for the purposes of genetic counselling and for the exclusions of berry aneurysms which if sufficiently large can be treated angiograpically and reduce long term risk of subarachnoid haemorrhage. The ultrasonic criteria for a diagnosis of ADPKD is as are as follows: - 15-39 years of age: 3 or more cysts i the kidneys (unilaterally or bilaterally) - 40-59 years of age: 2 or more cysts in each kidney - over 60 years: 4 or more cysts in each kidney Remember there are two types of ADPKD type 1 and type 2 the latter a milder phenotype.

Answer 27

``` Cysts in other organs Intracranial berry aneurysms Polycythaemia (Excess EPO) Hypertension (Excess Renin) Cardiac Valve disease ( Usually mitral valve prolapse but can also cause MR, TR, AR). Diverticular disease Aortic Aneurysm or thoracic coarctation Abdominal wall hernias ```

Answer 28

Common sites, Kidneys, Liver, Pancreases, Aracnoid cysts. Uncommon sites: Spleen, thyroid, lungs, ovaries, testes, seminal vesicles, bladder, broad ligament, uterus.

Answer 29

Infected cysts Haemorrhage into a cyst. The following conditions occur with higher frequency than the general population in patients with ADPKD and also cause abdominal pain. - diverticulitis/diverticular perforation - nephrolithaisis - strangulated/incarcerated hernia - Ruptured AAA - Tuberous Sclerosus complex - The gene for ADPKD type 2 is very close to the gene for TSC here certain mutations can cause both

Answer 30

PBC is asymptomatic in many patients and is diagnosed incidentally e.g. a cholecystectomy. In those who present with symptoms, pruritis and fatigue are the commonest presenting features. Some patients experience RUQ Pain. Impaired bile production leads to impaired absorption of fat soluble vitamins and easy brushing in some patients.

Answer 31

The female:male ratio in PBC is approximately 9:1. AMAs are directed against the mitochondrial enzyme complex M2. The antigen itself is the pyruvate dehydrogenase complex E2 found on the inner mitochondrial membrane. AMA is positive in 90% of patients wit PBC. In many cases disease progression is so slow that lifespan is unaffected and PBC may diagnosed incidentally e.g. at cholecystectomy. However, symptomatic patients are unlikely to survive more than 2 years

Answer 32

The most common differential diagnoses for PBC are autoimmune hepatitis and primary sclerosing cholangitis. PBC can occasionally be mistaken for Wilson’s disease as Kayser-Fleischer rings may occur as a result of impaired copper excretion. Granulomas in the liver may suggest cholestatic sarcoidosis but AMAs are usually absent in this condition

Answer 33

General measures include avoidance of alcohol and supplementation of the diet with the fat soluble vitamins (A,D,E,K). Ursodeoxycholic acid reduced cholestasis and improves liver function tests. However, it has little effect on symptoms or prognosis. Pruritis is generally assumed to be caused by circulating bile acids and may be helped by the anion exchange resin Cholestyramine. There is, however, evidence that the itch may have a central component. In advanced cases, a liver transplant may be successful. Functional decline, uncontrolled itching, hepatic encephalopathy, bleeding varices, and recurrent infections are all indications for transplantation.

Answer 34

PBC was first described by Thomas Addison about 100years before it was officially given the name primary biliary cirrhosis.

Answer 35

Alcoholic liver disease

Answer 36

Yes, this is now accepted as an indication for liver transplantation.

Answer 37

There are many estimates but the overal survival is approximately 60% over 15 years.

Answer 38

In the context of liver failure, the king’s College Hospital (KCH) criteria identify patients at particular high risk of death who should be discussed with a liver transplant unit. The KCH criteria divide patients into those with paracetamol-induced acute liver failure and those with non-paracetamol induced liver failure: Paracetamol Induced: - Arterial Lactate over 3.5 4 hours after resuscitation. - Or pH less than 7.3 or arterial lactate over 3 12 hours after resuscitation. - Or any 3 of the following: INR over 6.5, Crea over 300, Encephalopathy III or IV. Non-paracetamol induced: - Arterial Lactate over 3.5 4 hours after resuscitation - or INR over 6.5 - Or any 3 of the following: Age less than 10 or over 40, Serum bili above 300 umol/l, jaundice for 7 days, aetiology being a drug reaction.

Answer 39

Current immunosuppressive regimens use corticosteroids in combination with a calcineurin inhibitor such as Tacrolimus or ciclosporin and an antiproliferative agent such as mycophenolate mofetil. The first patient to receive a liver transplant and survive to 1 year was performed by Dr Thomas Starzl in 1967. However it was not until the introduction of ciclosporin in the early 1980s that long term survival became possible.

Answer 40

A number of devices exist to try and bridge the gap between liver failure and recovery or transplantation. Probably the best know n is the molecular adsorbents recirculating system (MARS), which consists of 2 circuits. In the first the patient’s blood is exposed to albumin via a semipermeable membrane, allowing toxins such as ammonia, bile acids, and bilirubin to be bound. The second circuit serves to remove these bound toxins from the albumin. Newer support systems include ECLAD (Extracorporeal liver assist devices) and Demetriou, which incorporates hepatoblastoma cell lines attached to semipermeable membrane columns. Heterotopic liver transplantation is the implantation of a lobe of allogeneic liver while leaving the native liver in situ. This is carried out when these is potential for the native liver to recover. The diner liver lobe is rejected with time, leaving the recovered, functioning liver.

Answer 41

Carcinoma of the stomach or pancreas Abdominal Aortic Anuerysm (Pulsatile) Lymphoma Caudate lobe of liver.

Answer 42

``` Crohn’s disease Caecal Carcinoma Ileocaecal mass including amoebic abscess, ileocaecal TB, appendicular mass, ideal carcinoid and lymphoma Ovarian Tumour (Benign or malignant) Renal Transplant ```

Answer 43

``` Sigmoid Carcinoma Diverticular mass/abscess Faecal Mass Ovarian Tumour (Benign or malignant) Renal Transplant ```

Answer 44

Diabetes mellitus ADPKD Chronic Glomerulonephritis Hypertension in Afro-Caribbean patients (NB the causative association between hypertension and ESRF in other ethnic groups is not very well established)

Answer 45

Patients with Alport’s syndrome have a defect in type IV collagen, so a new transplant exposes them to antigens they have not encountered before. The subsequent antibody response against this antigen may result in some patients developing anti-glomerular basement membrane disease (the antibody responsible for Goodpasture’s disease)

Answer 46

Cytomegalovirus Pneumocystis Jiroveci Epstein-Barr Virus (particularly in patients with post-transplant lymphoproliferative disease) BK virus (A common polyomarvirus which lies dormant in the renal tract after primary infection and can be reactivated by immunsuppresion. Complications o BK virus in transplants patients include ureteric stenosis and interstitial nephritis). JC Virus (another polyomavirus which causes progressive multifocal leukoencephalopathy) is being reported more and more in chronically immunosuppressed renal transplant recipients)

Answer 47

Good blood supply There is sufficient space for the kidney Proximity to bladder for ureteric anastomosis Easy access to perform renal biopsies or nephrostomies (in the event of a ureteric stricture) without having to puncture the peritoneum or risk abdominal viscera Note tat a kidney can be transplanted on either side of the pelvis depending on the quality of the vascular supply and venous drainage. As many patients with ESRF have diabetes, vasculopathy is common therefore an assessment of arterial supply during transplant work-up is important.

Answer 48

The Commonest cause of death in all patients with renal disease is cardiovascular. Accelerated cardiovascular disease (CVD) is a feature of all patients with renal disease as a result of both generic (Hypertenson, hyperlipidaemia, chronic inflammaton) and renal-specific (calcium, phosphate, PTH abnormalities, chronic anaemia, chronic calcineurin inhibitor therapy) risk factors. After CVD, the commonest cause of death are malignancy and infections. Both related to chronic immunosuppression.

Answer 49

Common: Cirrhosis with Portal Hypertension, Malignancy, Cardiac failure, Nephrotic syndrome Uncommon: Budd-Chiari syndrome, Portal Vein thrombosis, constrictive pericarditis, malabsorption syndromes, peritoneal mesothelioma, TB Peritonitis, Myoxedema, Ovarian disease.

Answer 50

Relative renal hypoperfusion causes increased renin from the juxtaglomerular apparatus. Renin activates aldosterone. Deficient hepatic metabolism reduces aldosterone and ADH breakdown. Hypoalbuminaemia decreased oncotic pressure. Third spacing of volume exacerbates renal hypoperfusion. The combination of salt and water retention from secondary hyperaldosteronism and high ADH) with high portal pressure causes net ultrafiltration of fluid into the abdominal cavity resulting in ascites.

Answer 51

Use the serum: ascites albumin gradients (SAAG). A low gradient (less than 11g/L) indicates loss of protein into the ascites and is a sign of exudate where as a high gradient (over 11g/L) indicates a transudate

Answer 52

Autosomal recessive disease due to a defect in the CFTR gene (Cystic Fibrosis Transmembrane Conductance Regulator Protein) Located on chromosome 7. Over 1250 mutations. Frequency of mutations is 1 in2000-3000 Live births in Caucasians. CFTR protein located in all exocrine tissues. Defective CFTR protein prevents chloride moving out of cells, this in turn leads to Na Hyperabsorption occuring to keep intracellular electrochemical balance. There is osmotic pull of water into the cells. Dehydration of extra cellular surfaces and thick viscous secretion easily amenable to colonisation and infection.

Answer 53

CF is a multisystem disease with lung disease being the major cause of mortality and morbidity. Gastrointestinal: - Pancreatic enzyme insufficiency with malabsorption of fat and protein. - Diabetes (Cystic fibrosis- related diabetes CFRD) - Meconium Ileus in newborns and distal intestinal obstruction syndrome (DIOS) - Focal biliary cirrhosis leading to or with portal hypertension - Cholelithiasis Reproductive: Male subfertility (over 95% infertility) due to defective sperm transport. Spermatogenesis is normal. Musculoskeletal: Osteoporosis ENT: Sinus disease and nasal polyposis

Answer 54

``` Haemophilus Influenza (Early Childhood) Staph Aureus (Early Childhood) Pseudomonas (Late Childhood) Burkholderia cepacia ‘complex’ (less common but important) this is associated with accelerated decline in pulmonary function an decreased survival and its presence may be a contraindication to lung transplantation in some centres. ```

Answer 55

Mean survival 32 years and increasing, many patients live into their 40’s and lead active lives.

Answer 56

Pulmonary Complications: - Recurrent infection - Haemoptysis - empyema/abscess - Cor pulmonale Extra pulmonary complications: - anaemia - metastatic infection e.g. cerebral abscess - secondary amyloidosis

Answer 57

Connective tissue disease e.g. RA Chronic Sinusitis IBD Marfan’s syndrome

Answer 58

Pseudomonas, Haemophiluis and Streptococcus ( Less commonly)

Answer 59

Normal pulse character, normal 2nd heart sound, frequently in an elderly person note this overlaps with mild AS.

Answer 60

Endocarditis Heart Failure AV Block due to invasion of calcium from the valve ring into the his-purkinje system Embolic Events

Answer 61

In severe AS one sees slow rising pulse, absent 2nd heart sound, possibly wit evidence of heart failure clinically. Raised Mean Valve Gradient and reduced Aortic valve area are associated with more severe AS.

Answer 62

Coarctation of the aorta with bicuspid AS(Remember to check for radio-femoral delay) Other valvular disease, particular mitral in rheumatic disease Angiodysplasia of the colon and anaemia (Heyede’s Syndrome)

Answer 63

Symptomatic AS. This is particular and urgently the case if the symptom is syncope, in which case the median survival is 4 months. Asymptomatic AS where the gradient is 40mmHg or more and any of the following also apply: - LV EF less than 45% - Hypotension in exercise - VT - LVH more than 15mm - Valve Area less than 0.6cm2/m2

Answer 64

``` Sarcoidosis Streptococcal Throat Infection Streptomycin, Sulphonamides Oral Contraceptive Pill Pregnancy TB Inflammatory Bowel Disease Lymphoma Idiopathic ```

Answer 65

Autosomal dominant condition affecting Chromosome 15 causing a Defect in Fibrillin protein

Answer 66

Paget’s Disease Pseudoxanthoma Elasticum Ehlers-Danlos Syndrome

Answer 67

Inherited: myotonic Dustrophy, Muscular Dystrophy Endocrine: Cushing’s Syndrome, hyperparathyroidism, thyrotoxicosis, diabetic amyotrophy Inflammatory: polymyositis, rheumatoid arthritis Metabolic: osteomalacia Malignancy: paraneoplastic, lambert eaten myasthenic syndrome Drugs: Alcohol, Steroids

Answer 68

The Big Three C’s: Cirrhosis (alcoholic) Carcinoma (HCC or Metastases) Congestive Cardic Failure Plus Three I’s: Infectious: HBV and HCV, EBV Immune: PBC, PSC, AIH Infiltrative: Amyloid, Sarcoid, Myeloproliferative

Answer 69

PBC: IgM Antimitochondrial Subtype M2 PSC: ANA Anti-SMA AIH: - Type 1 ANA Anti SMA ( adults and children) - Type 2 Liver/Kidney Microsomal Type 1 (LKM1) Antibodies ( children) - Type 3 soluble liver-Kidney antigen (middle aged adults)

Answer 70

Spleen over 8cm Myeloproliferative: CML myelofibrosis Infective: Malaria, Visceral Leishmaniasis (kala-azar) Infiltrative: Gaucher’s (lysosomal Storage disorder)

Answer 71

Spleen 4-8cm Myeloproliferative/lymphoproliferative disorders Infiltrative: Amyloid and Gaucher’s, Sarcoid

Answer 72

Spleen less than 4cm, splenic tip Myeloproliferative / lymphoproliferative Portal Hypertension Infective: EBV, IE, Hepatitis, Brucellosis Haemolytic Anaemia Thyrotoxicosis

Answer 73

Autosomal Dominant: ADPKD1 on chromosome 16 (85%) ADPKD2 on chromosome 4 (15%)

Answer 74

Pleural Protein less than 25g/l = Transudate Pleural Protein over 35g/L = exudate Pleural protein 25-35g/L = Lights Criteria Lights Criteria: 1 of the following indicates Exudate - pleural fluid protein to Serum protein ratio over 0.5 - pleural LDH to Serum LDH ratio over 0.6 - pleural LDH is 2/3 over serum upper value of normal - serum albumin-pleural albumin is less than 1.2g/dL

Answer 75

``` Berylliosis, siliocosis (pneuoconiosis) Radiation Extrinsic allergic alveolitis Ankylosing spondylitis Sarcoidosis Tuberculosis ```

Answer 76

Diagnostic criteria for IE patient requires 2 major, 1 major and 2 minor or 5 minor criteria Major Criteria: Typical organism in two blood cultures Echo: abscess, large vegetation dehiscence ``` Minor Criteria: Pyrexia over 38 Echo suggestive Predisposed e.g. previous or prosthetic valve or IVDU Embolia phenomena Vasculitic phenomena raised CRP ESR Atypical organism on blood culture ```

Answer 77

``` Medical Failure to antibiotics Fungal resistant time antifúngicos Cardiac failure Aortic root abscess Heart block Severe valvular incompetence Septic emboli ```

Answer 78

Corrigans: visible vigorous neck pulsatstion Quinckes: nail bed capillary pulsation De Mussets: head nodding Duroziez’s: diastolic murmur proximal to femoral artery compression Traubes: Pistol shot sound over the femoral arteries

Answer 79

Pulmonary Stenosis Ventricular Septal Defect Overriding Aorta Right Ventricular Hypertrophy (Often corrected in childhood old midline sternotomy)

Answer 80

Standing from squatting position and valsalva manourvre reduces LV volume Enhances Mitral Valve Prolapse and HOCM

Answer 81

Myopathic facies: long thin expressionless Wasting of facial muscles and sternocleidmastoid Bilateral ptosis Frontal balding Dysarthria Myotonia Distal muscle wasting and weakness Percussion myotonia

Answer 82

DM1: CTG trinucleotide repeat within DPMK gene on chromosome 19 DM2: CCTG tetranucleotide repeat within ZNF9 gene in chromosome 3 (presents later)

Answer 83

Unilateral: Third nerve palsy Hotness syndrome ``` Bilateral: Myotonic Dystrophy Myasthenia Gravis Congenital Senile Kearns-Sayre syndrome Bilateral horners ```

Answer 84

DANISH ``` Dysdiadochokinesis Ataxia Nystagmus Intentional Tremor Scanning Dysarthria Hypotonia/Hyporeflexia ```

Answer 85

PASTRIES ``` Paraneoplastic Syndrome Alcoholic Cerebellar Denegeration Sclerosis (MS) Tumour (posterior fossa SOL) Rare (Friedrichs and ataxic telangiectasia) Iatrogenic (phenytoin toxicity) Endocrine (hypothyroidism) Stroke ( brain stem vascular event) ```

Answer 86

Impairment of adduction of ipsilateral side with contralateral abduction nystagmus. Normal convergence. Due to a lesion medial longitudinal fasciculus

Answer 87

Worsening symptoms after a hot bath the or exercise, occurs in MS

Answer 88

TACS: Hemiplegia Homonomous hemianopia Higher cortical dysfunction e.g. dysphasia, dyspraxia, neglect PACS: 2/3 of the above LACS: Pure hemisensory or hemimotor loss

Answer 89

Occlusion of posterior inferior cerebellar artery Ipsilateral cerebellar signs Ipsilateral horners syndrome Ipsilateral trigeminal Pain and temperature loss Ipsilateral palatal paralysis and reduced gag reflex Contralateral pain and temperature sensory loss of limbs

Answer 90

Common: MS Spinal cord compression/ Cervical myelopathy MND (no sensory signs) ``` Other: Anterior spinal artery thrombosis Syingomyelia Transverse myelitis Tropical Spastic paraparesis Hereditary spastic paraplegia Subacute degeneration of cord ( absent reflexes upgoing plantars) Friedrichs ataxia Parasagittal falx meningioma ```

Answer 91

``` L2/L3 hip flexion L3/L4 knee extension (Knee Jerk) L4/L5 foot dorso Flexion L5 extension 1st toe L5/S1 knee flexion hip extension S1/S2 foot plantar flexion (Ankle Jerk) ```

Answer 92

Inversion is spared in common peroneal

Answer 93

Symptoms arising from lesions affecting pyramidal system (corticospinal and corticobulbar tracts) typically cause UMN with extensors affected predominately in upper limbs and flexors in lower limbs

Answer 94

``` C4/C5 shoulder abduction C5/C6 elbow supination (Biceps and Supinator Jerk) C6 Wrist Extension C7/C8 elbow extension (Triceps Jerk) T1 finger adduction ```

Answer 95

Types + Features: -Amyotrophic lateral sclerosis: affects motor cortex and anterior horn cells. weakness, UMN + LMN signs worse prognosis if bulbar onset, increased age, decreased FVC - progressive bulbar palsy: only affect cranial nerves IX-XII - progressive muscular atrophy: anterior horn cell only thus no UMN signs, affects distal muscle groups before proximal. - Primary lateral sclerosis: los of betz cells in motor cortex, thus mainly UMN signs, spastic leg weakness and pseudobulbar palsy

Answer 96

``` Diabetes Mellitus Alcohol Drugs e.g. isoniazid and vincristine Vitamin deficiency b1, b12 CKD HIV ```

Answer 97

GBS and Botulism Lead toxicity Porphyria Hereditary sensory motor neuropathy (Charcot Marie Tooth)

Answer 98

WARDS PLC ``` Wegner's granulomatosis (Granulomatosis with Polyangitis) Amyloidosis/ AIDS Rheumatoid Arthritis /SLE Diabetes Mellius Sarcoidosis Polyarteritis Nodosa/ Churg Strauss Leprosy Carcinomatosis ```

Answer 99

``` GBS CIDP HSMN I Amiodarone Paraproteinaemia ```

Answer 100

``` Alcohol Diabetes B12 deficiency HSMN II Vasculitis ```

Answer 101

Autosomal recessive Main: Young Adult in wheelchair or ataxic gait Pes cavus Bilateral cerebellar ataxia Posterior column signs ( loss of vibration sense and joint position) Leg wasting with absent reflexes and upgoing plantars ``` Other: Kyphscoliosis Optic atrophy High arched palate Sensorineural deafness HOCM murmur ```

Answer 102

``` Friedrichs ataxia Subacute degeneration of cord Motor neurone disease Taboparesis Comia medullaris lesion ```

Answer 103

In facial nerve palsy the eyeball rolls upwards on attempted eye closure

Answer 104

``` Bells Ramsay Hunt Syndrome Mononeuropathy (diabetes, Sarcoid Lyme) Tumour/trauma MS/ Stroke ```

Answer 105

``` GBS Sarcoidosis Myasthenia gravis Bilateral bells Lyme disease ```

Answer 106

Sympathetic loss to face/eye PEAS Ptosis Enophthalmos Anhydrosis Small Pupil (miosis)

Answer 107

Moderately dilated pupil they had poor response to light and a sluggish response to accommodation Associated with diminished or absent ankle and knee jerks Benign condition

Answer 108

Small irregular pupil they accommodated but doesn’t react to light

Answer 109

Complete Ptosis Dilated pupil (unless medical) Down and out eye position. Test the trochlear nerve on looking Nasally the eye will intort indicating the trochlear is working

Answer 110

Mononeuritis Multiplex Midbrain Infarction e.g. Weber’s Midbrain demyelination (MS) Migraine

Answer 111

Posterior communicating artery aneurysm (painful) Cavernous sinus pathology e.g. thrombosis, tumour or fistula Cerebral Uncus herniation

Answer 112

Private Vehicle: - First unprovoked seizure - 6 Months - epilepsy - 1 year fit free - ACS - 1 month untreated 1 week if stented - Stroke/TIA 1 month if no persistent deficient - IDDM - DVLA notification, no visual impairment and aware of hypoglycaemia HGV: - First unprovoked seizure - 5 years - epilepsy - 10 years fit free off meds - ACS - 6 weeks if symptom free and no inducible ischaemia - Stroke/TIA 1 year if no persistent deficient - IDDM - banned

Answer 113

Anti Thyroid Peroxidase Antibodies: associated with Graves and hashimotos thyroiditis Anti Thyroglobulin Antibodies associated with thyroid cancer and Hashimoto’s thyroiditis Anti Thyroid Stimulating Hormone Receptors associated with Graves

Answer 114

In pregnancy there is an increase in the levels of thyroxine-binding globulin. This causes an increase in the total levels of thyroxine but does not affect the free thyroxine level. Thyrotoxicosis: - Untreated thyrotoxicosis increases the risk of foetal loss, maternal heart failure and premature labour. - Graves' disease is the most common causes of thyrotoxicosis in pregnancy. It is also recognised that activation of the TSH receptor by HCG may also occur - often termed transient gestational hyperthyroidism.. HCG levels will fall in second and third trimester - Propylthiouracil has traditionally been the anti-thyroid drug of choice. However most guidelines recommend its use in the first trimester after this time switch to carbimazole as propylthiouracil is associated wit increased risk of maternal liver toxicity - Maternal free thyroxine levels should be kept in the upper third of normal reference range to avoid foetal hypothyroidism. - thyrotrophin receptor stimulating antibodies should be checked at 30-36 weeks gestation to help determine risk of neonatal thyroid problems. - block and replace regimes should not be used in pregnancy and radio iodine is contradindicated.

Answer 115

Renal: Renal parenchyma disease e.g. Vasculitis Renaovascular disease e.g. RAS Chronic Renal Disease of any aetiology ``` Endocrine: Cushing s syndrome Hyperaldosteronsim (Conns) Adrenal Hyperplasia Pheochromocytoma Acromegaly Thyroid disease ``` Cardio respiratory: Coarctation of aorta Obstructive sleep apnoea ``` Drug Induced: Ciclosporin NSAIDs COCP Decongestants Steroids ```

Answer 116

Marfan’s Syndrome Homocystinuria MEN2b

Answer 117

MEN1: 3P’s Parathyroid (Primary Hyperparathyroidism) Pituitary adenomas Pancreatic lesions (insulinoma glucagonoma) MEN2a: 2 P’s Medullary Thyroid Cancer Parathyroid (Primary Hyperparathyroidism) Phaeochromocytoma ``` MEN2b: 1 P’s Medullary Thyroid Cancer Phaeochromocytoma Marfanoid Habitus Neuromas ```

PACES Viva Questions Flashcards

(141 cards)