Blackboard Mock Exam 2018 Flashcards

Question

A 55 year old man with known carcinoma of the lungs, develops SOB over a few days. He has a large cardiac silhouette on his CXR but no pulmonary oedema. ``` What is the diagnosis? A. Atrial septal defect B. Dilated cardiomyopathy C. Infective endocarditits D. Mitral regurgitation E. Mitral stenosis F. Pulmonary fibrosis G. Pericarditis H. Conduction system disease I. Pericardial effusion J. Aortic valve disease K. Tuberculosis L. Hypertensive cardiomyopathy ```

Answer 1

I. Pericardial effusion This is a malignant effusion (one of the most likely to lead to tamponade) caused by lung cancer. Other prevalent malignant causes include breats cancer, lymphomas and leukaemias. This may also be the first sign of metastatic disease. The history of lung cancer here should make you suspicious. Other causes of a pericardial effusion include hypothyroidism (high protein content and accumulate very slowly due to capillary leak), cardiac causes such as CHF and dissection of the proximal aorta, trauma, radiation-related, uraemia, immune-mediated such as SLE, Dressler’s, amyloidosis and Wegener’s, infectious or idiopathic (which is generally assumed to be viral). Symptoms may coexist with those of pericarditis sometimes. ECG and CXR are indicated here. Most patients also get an echocardiogram whic is the preferred test to establish the diagnosis. On ECG there may be diffuse ST elevation and PR depression with epicardial inflammation. If the effusion is large enough there may be electrical alternans, which is beat-to-beat variation of the ventricular axis (find an image of this to cement it in your memory). The cardiac shadow on CXR is said to be ‘water-bottle shaped’. Pericardiocentesis may be necessary depending on the clinical case.

Answer 2

G. Gastric ulcer The patient has a bleeding peptic ulcer (the black tarry stools from the UGI bleed). Epigastric pain and tenderness related to eating a meal is typical of a peptic ulcer. 80% are duodenal and 20% are gastric. Ulcers may cause iron deficiency anaemia and associated symptoms may feature. Key risk factors are NSAID use, like in this patient, H. pylori infection, smoking and a family history of PUD. Zollinger-Ellison syndrome should be considered if there are multiple ulcers or ulcers refractory to treatment. Gastric ulcers classically cause pain which is exacerbated by eating and immediately relieved on vomiting. There is usually also weight loss due to a fear of food and its association with pain. Duodenal ulcers are classically made worse by hunger and are relieved by eating and the patient may wake at night with the pain. As a result, weight gain is typically a feature. In reality, it is difficult to differentiate the site of the ulcer based on these features. The most specific and sensitive test is an upper GI endoscopy which is initially ordered if the patient has ‘red flag’ symptoms, is >55 years of age or fails to respond to treatment. Duodenal ulcers rarely undergo malignant transformation so do not require a compulsory biopsy but gastric ulcers require biopsies to rule this out. In patients who are 55 or younger without ‘red flags’, testing for Helicobacter pylori (breath testing with radiolabelled urea or stool antigen testing) is necessary. Management is aimed at correcting the underlying cause such as discontinuing NSAIDs. H. pylori eradication should be started if the organism is present with triple therapy. Otherwise, a PPI is indicated.

Answer 3

H. Crohn's disease This patient gives a history of IBD. This could well be UC where the mainstay of treatment is with 5-ASA. A colonoscopy is required to assess the extent of disease and for a definitive diagnosis. Biopsy in CD will show transmural granulomatous inflammation. CD can affect the whole GIT but favours the TI (RLQ pain) and proximal colon and is macroscopically characterised by skip lesions. UC on the other hand is characterised by the presence of crypt abscesses, which is pathognomic. CD risk is increased 3-4 fold by smoking whereas smoking seems protective in UC. The mainstay of treatment in CD is with steroids and azathioprine to revent relapses and for those suffering side effects of steroid treatment. TNF-alpha inhibitors also have a role. Surgery in CD is only indicated in a small number of patients who bleed, for bowel perforation and cases of complete obstruction. The aim is to rest distal disease by temporarily diverting faecal flow.

Answer 4

F. Mallory-Weiss tear This occurs after a rise in abdominal pressure which induces a tear in the oesophageal mucosa, causing subsequent GI bleeding. It commonly presents with haematemesis after an episode of retching/vomiting/coughing/straining. Hence, risk factors include anything which can cause vomiting like heavy alcohol use, which is commonly the case in EMQs. Also, other conditions would include food poisoning like in this case, bowel obstruction, hyperemesis gravidarum, bulimia, the chronic cough of COPD, meningitis etc... you name it really. Classically, MWT presents with a small self limiting episode of haematemesis. Definitive diagnosis is made by OGD. Treatment is supportive because most cases are self limiting and emergency treatment is not offered unless the patient is showing signs of clinical instability. If the patient is actively bleeding, treatment will be with therapeutic endoscopy in most cases, and very very few cases will require more intervention such as angiography with embolisation.

Answer 5

E. Oesophageal malignancy Such levels of extreme weight loss over a short period of time with GI symptoms here points to GI malignancy which is bleeding. The only option on the list is oesophageal. EMQs normally mention dysphagia, which occurs when there is obstruction of more than 2/3 of the lumen and presence indicates locally advanced disease however this is absent here. There may additionally be odynophagia. Men are twice as likely to develop oesophageal cancer. GORD, Barrett’s oesophagus, FH, tobacco and alcohol are all risk factors. The two main types are squamous cell carcinoma and adenocarcinoma. Tumours in the upper 2/3 of the oesophagus are SCC whereas those that lie in the lower 1/3 are adenocarcinomas. The main test to order is an OGD with biopsy. Treatment is either surgical resection or with chemo or radiotherapy alongside endoscopic ablation with or without stenting and brachytherapy.

Answer 6

B. Duodenal ulcer The patient has a duodenal ulcer. Epigastric pain and tenderness related to eating a meal is typical of a peptic ulcer. 80% are duodenal and 20% are gastric. Ulcers may cause iron deficiency anaemia and associated symptoms may feature. Key risk factors are NSAID use, like in this patient, H. pylori infection, smoking and a family history of PUD. Zollinger-Ellison syndrome should be considered if there are multiple ulcers or ulcers refractory to treatment. Gastric ulcers classically cause pain which is exacerbated by eating and immediately relieved on vomiting. There is usually also weight loss due to a fear of food and its association with pain. Duodenal ulcers are classically made worse by hunger and are relieved by eating and the patient may wake at night with the pain. As a result, weight gain is typically a feature. In reality, it is difficult to differentiate the site of the ulcer based on these features. The most specific and sensitive test is an upper GI endoscopy which is initially ordered if the patient has ‘red flag’ symptoms, is >55 years of age or fails to respond to treatment. Duodenal ulcers rarely undergo malignant transformation so do not require a compulsory biopsy but gastric ulcers require biopsies to rule this out. In patients who are 55 or younger without ‘red flags’, testing for Helicobacter pylori (breath testing with radiolabelled urea or stool antigen testing) is necessary. Management is aimed at correcting the underlying cause such as discontinuing NSAIDs. H. pylori eradication should be started if the organism is present with triple therapy. Otherwise, a PPI is indicated.

Answer 7

K. Sarcoidosis Sarcoidosis is a chronic multisystem disease with an unknown aetiology but pulmonary involvement usually dominates. Hypercalcaemia occurs in these granulomatous conditions (also including TB and leprosy) as a result of tissue being able to 1-alpha-hydroxylate 25(OH) D leading to vitamin D (the 1,25-dihydroxyvitamin D3) excess. Erythema nodosum, tender erythematous nodules and lupus pernio, indurated plaques with discoloration on the face, are typical skin manifestations of sarcoidosis which this patient has. CXR will typically show bilateral hilar lymphadenopathy and CXR findings are used in the staging of disease. Additionally, serum calcium and ACE levels may be raised. A transbronchial biopsy is essential for diagnosis in most cases and shows the presence of non-caseating granulomas. Black people have a higher lifetime risk of sarcoidosis, as do those of Scandinavian origin. The mainstay of treatment for severe disease involves systemic corticosteroids.

Answer 8

B. Bronchiecstasis Bronchiectasis is permanent bronchi dilatation due to bronchial wall damage and loss of elasticity. It is often as a consequence of recurrent/severe infections (whooping cough in this case caused by Bordetella pertussis) and most present with chronic productive mucopurulent cough. The most common identifiable cause is CF. Many patients will have crackles on auscultation of the lungs and high-pitched inspiratory squeaks and pops are also commonly heard. SOB is present in the majority of patients, especially with exertion (and is a good correlate of the severity on CT) and more than half will have recurrent episodes of fever, which is also a sign of an acute exacerbation. Daily sputum production is present in two thirds of patients and bloody sputum in half. The most common identifiable cause is CF and clubbing is a sign of bronchiectasis. Chest CT is the diagnostic test. Diagnosis is aided by sputum analysis.

Answer 9

A. Tuberculosis It is important to have a high level of suspicion when evaluating patients with risk factors who present with suggestive symptoms. Night sweats, fever, weight loss, malaise, cough, haemoptysis and erythema nodosum are all suggestive. In the first half of the 20th century, tuberculosis accounted for over 90% of cases of erythema nodosum. Other key risk factors for pulmonary TB include exposure to infection and returning from or being born in a high-risk region such as Asia, Africa and Latin America (India in this case). The examination findings here are consistent with a parapneumonic effusion. If TB is suspected, the patient should be placed in isolation and a CXR obtained with 3 sputum samples cultured for AFB being the gold standard of diagnosis. Culture takes several weeks so sputum smears will be done before culture results are known. Interferon-gamma release assays (IGRAs) are now used by some hospitals to rapidly determine a patient’s TB status. All patients who have TB should be tested for HIV within 2 months of diagnosis.

Answer 10

H. Left ventricular failure LVF causes congestion in the pulmonary circulation so the symptoms are respiratory with evidence here of pulmonary oedema. As seen in this patient, there is SOB and there may also be orthopnoea. This is why you can ask patients in a cardiac history how many pillows they sleep with. PND can also occur as well as ‘cardiac asthma’. RVF leads to a backlog of blood and congestion of the systemic capillaries. This causes peripheral oedema and ascites and hepatomegaly may develop. Nocturia may be a symptom as fluid returns from the legs when the patient lies down flat. This patient does have peripheral oedema too so technically has CCF (congestive cardiac failure), but the best option on the list is LVF.

Answer 11

F. Streptococcus pneumoniae The rusty coloured sputum is hinting at a pneumococcal pneumonia.The patient has presented with common symptoms of fever and a productive cough. Examination findings are also consistent – have a think about what would actually be found while performing a respiratory examination on this patient. There may also be SOB, chills, rigors and pleuritic chest pain. The most specific and sensitive test is a CXR (PA and lateral) and initial treatment of a CAP is empirical with antibiotics. Often diagnosis is made solely on history and examination findings. Management is guided by the patient’s CURB-65 score.

Answer 12

A. Laryngitis Laryngitis, as the name would suggest, is inflammation of the larynx, which can lead to oedema of the true vocal folds. It has both infectious and noninfectious causes such as vocal strain. Symptoms of acute disease are most commonly hoarseness, generally over a period of less than a week, usually preceded by viral URTI and usually self limiting. The pain on swallowing and sore throat is common of URTIs. An exudate or cervical lymphadenitis would suggest bacterial infection instead. Treatment begins, as always, with ABC and airway assessment. Chronic laryngitis presents with hoarseness lasting more than 3 weeks and this needs investigating due to the fact that symptoms may be similar to cancer of larynx. Antibiotics are given in bacterial cases or otherwise voice rest and hydration is sufficient.

Answer 13

H. Angioedema Angio-oedema is swelling involving the deeper layers of the subdermis (occuring in association with urticaria in around 40% of cases). In this case it involves the face/neck and is dangerous – the risk being airway compromise and this requires rapid treatment with adrenaline. The food trigger here is obvious – this person has just consumed peanuts and this is allergic in nature, causing an IgE mediated reaction. Common triggers aside from nuts include eggs and shellfish although any food can be implicated. As mentioned, airway management and adrenaline is crucial here and you would also give antihistamines, IV corticosteroids and tell the patient to avoid the trigger.

Answer 14

I. Laryngeal nerve palsy This patient has just had neck surgery and the hoarseness here results from damage to the recurrent laryngeal nerve, which is a branch of the vagus nerve which supplies motor function and sensation to the larynx. This nerve runs posterior to the thyroid and results in hoarseness when damaged. Bilateral damage is even worse and the patient could have difficulty breathing and the complete inability to speak. The right recurrent laryngeal is more prone to damage as it is located relatively more medial than the left.

Answer 15

G. Carcinoma of the larynx Laryngeal cancer is frequently associated with two big risk factors: smoking and alcohol use. Major signs are persistent (>3 weeks) of hoarseness, dysphonia, sore throat, dysphagia, referred otalgia (seen here) and neck masses/adenopathy. These patients need to be evaluated by a specialist and as mentioned this can resemble chronic laryngitis in presentation. An MDT approach is taken to try and preserve the organ as much as possible, with salvage surgical resection offered in advanced disease.

Answer 16

E. Hypothyroidism This woman is in a hypothyroid state. The hoarseness may be a symptom of a goitre (enlarged thyroid). Worldwide, the most common cause is iodine deficiency. Other causes include Hashimoto’s or secondary and tertiary hypothyroidism. It can also result from viral de Quervain’s thyroiditis or postpartum thyroiditis. Symptoms include those mentioned as well as depression, bradycardia, sluggish reflexes, cold intolerance and muscle cramps. Diagnosis is based on measurement of TSH and thyroid hormones. Treatment is by replacement of T4 with or without T3 in combination. If the patient has normal T3 and T4 but mildly elevated TSH, this is described as subclinical hypothyroidism.

Answer 17

G. Abdominal ultra sound scan (USS) This is hepatic cirrhosis leading to clubbing. The haematemesis is secondary to oesophageal varices and he has signs of chronic liver disease. LFTs are non-specific however ultrasound can show signs of advanced cirrhosis. There may be liver surface nodularity (remember cirrhosis entails nodular regeneration), small liver and possible left/caudate lobe hypertrophy. Signs of portal hypertension may also be picked up such as ascites, splenomegaly and increased diameter of the hepatic portal vein. The most specific and sensitive test is a liver biopsy, which is not given as an option here. Additionally abdominal CT or MRI can also be done similar to USS, with similar findings.

Answer 18

M. CXR Possible mesothelioma. Possible pulmonary fibrosis. Either way, CXR is the way to go.

Answer 19

D. Echocardiogram The cause here is cardiac and likely Eisenmenger’s syndrome. This is clubbing due to congenital cyanotic heart disease and you would want to perform an echocardiogram here to look for structural heart defects.

Answer 20

H. Colonoscopy This is clubbing due to inflammatory bowel disease (Crohn’s or UC) and diagnosis of this would be made on colonoscopy. Biopsy in CD will show transmural granulomatous inflammation. CD can affect the whole GIT but favours the TI (RLQ pain) and proximal colon and is macroscopically characterised by skip lesions. UC on the other hand is characterised by the presence of crypt abscesses, which is pathognomic.

Answer 21

C. Bronchoscopy This is likely lung cancer and diagnosis relies on pathological confirmation from a tissue sample, often obtained from bronchoscopy. A CXR will of course be done in the first instance as the initial investigation but the best test on the list for diagnosis is a bronchoscopy.

Answer 22

H. Endomysial antibodies This is a common condition in the US and Europe. Coeliac disease most commonly presents with IDA, although it can also lead to a macrocytic anaemia with mainly folate deficiency (though B12 is also affected but hepatic stores last several years). The mouth ulcers are a sign of this. There are also GI symptoms resulting from malabsorption. It is an autoimmune condition (the presence of another autoimmune condition here is a risk factor) triggered by gluten peptides found in wheat, rye and barley. The ultimate best test is duodenal biopsy and histology to show intra-epithelial lymphocytes, villous atrophy and crypt hyperplasia. Macroscopic changes may be present but endoscopy is generally unhelpful. The test of choice before performing such an invasive confirmatory test is to look for elevated anti-gliadin antibodies. Anti-tissue transglutaminase is less accurate and endomysial antibody is more expensive and has lower sensitivity, though is the only option on this list specific for coeliac. It is worth knowing about the Schilling test as it is frequently examined. However, it is no longer routinely done in clinical practice. In this test, IM vitamin B12 is given to saturate stores. Then oral radiolabelled B12 is given and urine is collected over 24 hours. The amount excreted is lower in B12 malabsorption. If this is not corrected by IF the problem is with the ileum and not inadequate IF.

Answer 23

E. HIV test Molluscum contagiosum is an umbilicated, pearl like, smooth papules and at least a third of patients will also develop symptoms of local erythema, swelling or pruritis. HIV is a strong risk factor for MC. It is transmitted skin-to-skin or fomite in children and sexually transmitted in adulthood. Oral candidiasis is also an opportunistic infection associated with the immunocompromised state of HIV. Weight loss is also seen in HIV and may result from malnutrition, TB or HIV wasting syndrome. Diarrhoea is also common. An HIV test is needed in this patient and contact tracing will be necessary.

Answer 24

I. Sweat test CF is autosomal recessive and the mean age of death is around 40. There is currently no cure for this condition. The reccurent chest infections and greasy stools (fat malabsorption due to pancreatic insufficiency) should make you think of CF. A persistent cough which is productive would also raise suspicions. Additionally, you may find nasal polyps and hepatomegaly and/or splenomegaly and a congenital absence of the vas deferens in males. There may also be failure to thrive in infants. The most conclusive diagnostic test is the sweat test which is positive if sweat chloride is >60mmol/L. Serum IRT from a heel prick blood spot allows screening of newborns. CF is a genetic condition with abnormal salt and water transport due to mutations in the CFTR (an apical anion channel). Heterozygotes generally do not demonstrate disease.

Answer 25

A. ERCP This is chronic pancreatitis which is most commonly associated with chronic alcohol abuse. Features include the epigastric pain here, which classically radiates to the back, and steatorrhoea from malabsorption (pale, foul-smelling and difficult to flush stools). There may additionally be DM due to pancreatic failure and the patient may be malnourished. The diagnosis is based on findings and imaging – your options are USS which is less sensitive, or CT, which is more sensitive but involves radiation exposure. AXR is not a sensitive enough test. However, this question is looking for the best test which is ERCP, commonly considered the most accurate test with high sensitivity and specificity. It is limited in use though due to cost and the risk to the patient. Characteristically ERCP would show beading of the main pancreatic duct as well as irregularities in the side branches. Faecal elastase-1 is inaccurate for diagnosing mild to moderate pancreatic insufficiency, and anyway has unacceptably low sensitivity. There is no real definitive treatment, which is mainly symptomatic and the underlying and precipitating factors are treated – in this case, this man’s alcohol excess. Complications of chronic pancreatic imflammation include the development of pseudocysts, calficiation, DM and malabsorption.

Answer 26

M. Colonoscopy This is a history of inflammatory bowel disease. A colonoscopy is required to assess the extent of disease and for a definitive diagnosis. Biopsy in CD will show transmural granulomatous inflammation. CD can affect the whole GIT but favours the TI (RLQ pain) and proximal colon and is macroscopically characterised by skip lesions. UC on the other hand is characterised by the presence of crypt abscesses, which is pathognomic.

Answer 27

H. Primary biliary cirrhosis Primary biliary cirrhosis (PBC) is a chronic condition where the intrahepatic small bile ducts are progressively damaged (and eventually lost) occuring on a background of portal tract inflammation. Fibrosis develops, ultimately leading to cirrhosis (which is defined as fibrosis with nodular regeneration). It is widely believed to be autoimmune in aetiology as almost all patients have AMA (present here). The pointers in this question which would raise your suspicion, is xanthelasma around the eyes, pruritis in the absence of an obvious dermatological cause, fatigue and the features of liver disease typical of cirrhosis (obstructive jaundice) and splenomegaly as a feature of portal hypertension.

Answer 28

C. Gilbert's syndrome Gilbert’s occurs in an asymptomatic patient, often as an incidental finding or mild jaundice occuring in adolescence/young adult age. There is elevated unconjugated BR with other liver tests being normal. The blood smear is also normal with normal reticulocyte count, and normal Hb indicating that this is not due to haemolysis. It is a common syndrome and is not really a disease, more a physiological variant. No treatment is needed and this condition is due to decreased UDPGT activity leading to decreased conjugation of unconjugated bilirubin, leading to elevated levels. Positive FH is common as this condition is most likely transmitted in an autosomal recessive pattern.

Answer 29

G. Carcinoma of the pancreas Pancreatic cancer (of the head) typically presents with painless obstructive jaundice and weight loss and generally presents late. There is however epigastric pain in this case, which is a possible presentation. Whipple’s procedure or Traverso-Longmire procedure (pancreaticoduodenectomy) offers the only hope of a cure but only a small minority are elegible for these procedures. The first tests to order are an abdominal USS and LFTs. Note Courvoisier’s law: Jaundice and a palpable painless gallbladder is unlikely to be caused by gallstones. The tumour marker for pancreatic cancer is CA19-9 which is useful in preoperative staging.

Answer 30

A. Hepatitis A This is likely hepatitis A which is primarily transmitted via the faecal-oral route. After the virus is consumed and absorbed, it replicates in the liver and is excreted in the bile (to be re-transmitted). Transmission usually precedes symptoms by about 2 weeks and patients are non-infectious 1 week after onset of jaundice. The history can reveal risk factors such as living in an endemic area, contact with an infected person, homosexual sex or a known food-borne outbreak. This is classically, in EMQs, associated with shellfish which is harvested from sewage contaminated water. If the patient has other liver diseases such as HBV or HCV or cirrhosis then there is a higher risk of fulminant HAV infection. The clinical course of HAV consists of a pre-icteric phase, lasting 5-7 days, consisting characteristically of N&V, abdominal pain, fever, malaise and headache. Rarer symptoms may be present such as arthralgias and even severe thrombocytopenia and signs that may be found include splenomegaly, RUQ tenderness and tender hepatomegaly as well as bradycardia. The icteric phase is characterised by dark urine, pale stools, jaundice and pruritis. When jaundice comes on, the pre-icteric phase symptoms usually diminish, and jaundice typically peaks at 2 weeks. However, a fulminant course runs in <1% of patients with worsenining jaundice and encephalopathy. Serum transaminases may reach in excess of 10,000 units, although there is little correlation between the level and disease severity. ALT is typically higher than AST.

Answer 31

E. Haemolytic anaemia Africans have higher incidence of sickle cell anaemia. This is a presentation of bone pain here with dactylitis, consistent with hand-foot syndrome which can be what young infants and children present with (it is often a child’s first presentation of disease). The jaundice here is due to haemolysis and so while this is sickle cell anaemia, the options are trying to get you to think a bit about the best fit here which would be haemolytic anaemia. About 8% of black people carry the gene and the prevalence is high in sub-Saharan Africa. The condition is autosomal recessive and therefore occurs in 1 in 4 pregnancies where both parents carry the sickle gene. Sickling occurs when RBCs containing HbS become distorted into a crescent shape. Patients with sickle cell anaemia have no HbA at all. If both parents carry the sickle cell gene, there is a 1 in 4 chance of giving birth to a child with sickle cell anaemia. Sickle cell disease also includes other conditions such as HbS from one parent with another abnormal Hb or beta thalassaemia from the other parent such as HbS-Beta thal and HbSC. Treatment goals here include fluid replacement therapy, pain management and symptomatic control.

Answer 32

I. Extensive necrosis Paracetamol OD can occur after a single large OD or repeated ODs. Often, the patient is asymptomatic at initial presentation but if untreated may cause liver injury over the 2-4 days after ingestion, including fulminant liver failure. Massive hepatic necrosis will occur and will be the cause of fulminant liver failure. Paracetamol is the most frequent intentional OD drug in this country. The risk of liver damage is increased after taking drugs which induce CYP 450. Inducers include St John’s wort, barbiturates, phenytoin, tetracycline, chronic alcohol use and carbamazepine. A serum paracetamol level is important to order as early as possible, but at the earliest 4 hours post-ingestion.Treatment if indicated is with N-acetylcysteine with the level based on a paracetamol treatment graph.

Answer 33

H. Hepatocellular carcinoma Patients with cirrhosis, especially those with alcoholic liver disease, are at a high risk of developing HCC. Cirrhosis is irreversible so despite stopping drinking, he is still at risk of HCC (hepatoma). Patients with cirrhosis should be screened for HCC with serum AFP and USS at 6 month intervals.

Answer 34

F. Cholecystitis Cholecystitis is acute GB inflammation caused by an obstruction at the cystic duct. It occurs as a major complication of gallstones and classically presents with RUQ pain and fever. Gallstones in EMQs classically involves the Fs (Fat, Forty, Female, Fertile, Fair). USS is the definitive initial investigation. HIDA scanning and MRI may help if the diagnosis remains unclear. Treatment is with cholecystectomy.

Answer 35

A. Extensive cirrhosis This is a case of haematemesis secondary to oesophageal varices. Oesophageal varices are a direct result of portal hypertension, which occurs as a progressive complication of cirrhosis, which is what liver biopsy will show. Diagnosis and surveillance by endoscopy is an important part of management of this condition and in terms of prophylaxis against variceal bleeding before it has occured, non-selective beta blockers and/or endoscopic ligation can be used.

Answer 36

E. Hepatitis B Hepatitis B is a DNA virus which is transmitted percutaneously and permucosally. It is also a STI. HCV is an RNA virus and RNA-PCR will be positive. A brief bit about hepatitis B markers: HBsAb appears several weeks after HBsAg disappears and in most patients suggests a resolved infection and life-long immunity (it is also detectable and titres are measured in those immunised with the HBV vaccine). HBsAg on the other hand appears 2-10 weeks after exposure to HBV and usually, in self-limiting acute cases, becomes undetectable after 4-6 months of infection. Persistence for >6 months implies chronic infection. Core antibody (IgM) appears within weeks of acute infection and remains detectable for 4-8 months and can be the only way to diagnose acute infection during the period when surface antigen disappears but before surface antibody has appeared. Chronic infection is indicated by IgG core antibody. The best single test to screen household contacts of infected individuals to determine the need to vaccinate is still HBcAb. E antigen is a soluble viral protein in serum which is part of the early acute infection and disappears soon after peak ALT levels. Presence >3 months indicates chronic infection is likely. E antigen being present in those with surface antigen indicates greater infectivity and a high level of viral activity and replication.

Answer 37

E. Activated charcoal ``` This is an overdose of tricyclic antidepressants which are a class of drugs with a narrow therapeutic index and therefore become potent toxins in moderate doses to both the CNS and cardiovascular system. The main aim in treatment is to provide respiratory and cardiovascular support until the medicine has been fully metabolised and eliminated. GI decontamination should be considered in those presenting with early overdose (under 2 hours after ingestion) provided that the airway can be protected. There is no shown clear benefit to repeated doses of activated charcoal. The warm, dry skin is part of the anticholinergic effects (physostigmine should NOT be used to reverse this as it has been in rare cases been associated with asystole – would you rather have a patient who is flushed or flatlined?). Other anticholinergic effects include dilated pupils, urinary retention, decreased or absent bowel sounds and changes in mental status. Hypotension is common and is due to alpha 1 antagonism. Classic ECG changes are of sinus tachycardia progressing to wide complex tachycardia and ventricular arrhythmias (with increasing severity and intoxication). Condution problems and hypotension is improved with hypertonic sodium bicarbonate and if arrhythmias are present, treatment of these involves correcting the acidosis, hypoxia and electrolyte abnormalities. Anti-arrhythmics are generally avoided. If hypotension is refractory then a vasopressor can be used. BZDs can be used for any seizures. ```

Answer 38

A. N-acetylcysteine Paracetamol OD can occur after a single large OD or repeated ODs. Often, the patient is asymptomatic at initial presentation but if untreated may cause liver injury over the 2-4 days after ingestion, including fulminant liver failure. Paracetamol is the most frequent intentional OD drug in this country. The risk of liver damage is increased after taking drugs which induce CYP 450. Inducers include St John’s wort, barbiturates, phenytoin, tetracycline, chronic alcohol use and carbamazepine. A serum paracetamol level is important to order as early as possible, but at the earliest 4 hours post-ingestion.Treatment if indicated is with N-acetylcysteine with the level based on a paracetamol treatment graph.

Answer 39

H. IV-naloxone Signs of opiate OD include CNS depression, miosis (pinpoint pupils) and apnoea. Naloxone is indicated both therapeutically and diagnostically. If there is a response, then it is diagnostic. Another diagnosis should be sought if the patient is unresponsive. IV is the preferred route of administration although naloxone can be given IM or SC if IV access cannot be established. Ventilatory support is key with 100% oxygen.

Answer 40

D. IV-glucagon Glucagon stimulates adenyl cyclase which acts to increase intracellular cAMP and to therefore increase cytosolic calcium and cardiac contractility. Hypotension and bradyarrhythmias are the most common initial findings of beta blocker toxicity. If IV glucogon is not available then high-dose insulin can be used instead with co-administration of dextrose to maintain blood glucose levels.

Answer 41

B. IV-ethanol Antifreeze is ethylene glycol. It is a sweet-tasting, odourless and colourless liquid and the substance itself is non-toxic and initially causes inebriation. Toxicity appears within 12-24 hours and is due to metabolic acidosis and the formation of calcium oxalate from one of the metabolites. Oxalate deposits in the lungs, myocardium and kidneys leading to organ damage and renal failure, and hypocalcaemia may also occur due to the consumption of circulating calcium. Ethylene glycol is not absorbed by activated charcoal and gastric decontamination is pointless regardless of time since consumption. The first line treatment is fomepizole (4-methylpyrazole) which is a competitive inhibitor of alcohol dehydrogenase, an enzyme involved in catalysing the initial steps in metabolism of ethylene glycol and methanol into toxic metabolites. However, this is not on the list, and oral ethanol (loading dose or infusion) can be used in this case (have a think about why ethanol would work as an antidote, if you think back to how ethanol is metabolised). Dialysis may well be required.

Answer 42

C. Diagnostic laparotomy An abdominal and pelvic CT scan would normally be ordered in situations like this, with possible appendicitis, but it is assumed here that the doctors were thinking of pregnancy as a possibility so a sonogram was done instead, which turns out to be inconclusive. Now, in this situation, you could do an abdominal MRI (especially in early pregnancy) or go ahead with a CT scan anyway, but a diagnostic laparotomy is the best option here to diagnose and treat at the same time. The main differential here is either obstetric, such as a ruptured ectopic pregnancy, or acute appendicitis. You would have imagined they would have done the usual important tests like FBC and a urinary pregnancy test... but these results are not available, nor are they an option, but a prudent doctor would have ordered them in the diagnostic work up. However, given USS does not show a mass in the fallopian tubes, this may push you away from an ectopic pregnancy... however, USS is operator dependent, this is a female of childbearing age (this age is getting lower and lower in the UK) and the doctor has failed to obtain either serum or urine HCG levels or asked about any missed menstrual periods. This patient, should be made NBM with maintenance IV fluids like lactated Ringer’s, and have a laparotomy which can be both diagnostic and therapeutic. You can take the appendix out if this is the problem or deal with the ectopic, if that is the problem, or deal with whatever it could be.. say for instance, a rare Meckel’s diverticulitis.

Answer 43

D. CT scan This patient has renal colic which classically presents with severe flank pain radiating to the groin. Infection may complicate renal calculi. Microscopic haematuria is present in up to 90% of cases. Up to 85% of stones are visible on a plain KUB although urate stones are radiolucent. If the stone is radio-opaque, calcification will be seen within the urinary tract. In pregnancy though, a renal USS is first line. The IVP has now been replaced by the CT scan which is the new diagnostic standard. A non-contrast helical (or spiral) CT is preferred due to high sensitivity and specificity and acurately determines presence, site and size of stones. Stones are analysed after they are extracted or when they are expelled to check their composition. In reality the first test to order is urinalysis – it is quick and simple. You’d also always exclude ectopic pregnancy with a urine pregnancy test if this were a female of childbearing age, look for hypercalcaemia and hyperuricaemia and perform a FBC to look for infection.

Answer 44

L. Acute pancreatitis This patient has acute pancreatitis – the cause here being alcohol. He is describing mid-epigastric pain. This pain classically radiates around to the back, which in itself is almost diagnostic. Complicated haemorrhagic pancreatitis may exhibit Cullen’s sign, Grey-Turner’s sign and Fox’s sign. Make sure you know what these are and you are familiar with the causes of acute pancreatitis (GET SMASHED). Those caused by hypocalcaemia may also display Chvostek’s sign and Trousseau’s sign. Key to diagnosis is serum amylase or lipase levels which are massively elevated. Prognostic criteria are outlined in Ranson’s criteria applied on admission and after 48 hours, or the modified Glasgow score which you can find in your Oxford Handbook. An abdominal CT is however the most sensitive and specific study and findings may include enlargement of the pancreas with irregular contours, necrosis, pseudocysts and peripancreatic fat obliteration.

Answer 45

I. Oral antibiotics This patient obviously has diverticulitis and does not need further investigation. Symptomatic diverticulitis presents with fever, high WCC and LLQ pain. Risk factors for diverticular disease include low dietary fibre and advanced age. Oral antibiotic therapy and analgesia is indicated. If there is no improvement in 72 hours after oral antibiotics then IV antibiotics are indicated. Make sure you understand the differences in the terms: diverticulosis, diverticulitis and diverticular disease.

Answer 46

I. Ultrasound scan Abdominal ultrasound is ordered when a patient presents with biliary pain and is the single best test for cholelithiasis (though has a low sensitivity for choledocholithiasis). Note that cholelithiasis refers to stones in the gallbladder and choledocholithiasis refers to stones in the bile duct. If stones are found then this would give weight to a diagnosis of acute cholecystitis. There are symptoms here of obstructive jaundice due to gallstone obstruction of bile outflow. Serum amylase would also be ordered in any patient presenting with pain located in the epigastric region, to rule out acute pancreatitis. This has been done here. In this patient you would also order LFTs, FBC looking for evidence of inflammation. MRCP, ERCP and EUS can be considered if necessary.

Answer 47

K. IV naloxone Signs of opiate OD include CNS depression, miosis (pinpoint pupils) and apnoea. Naloxone is indicated both therapeutically and diagnostically. If there is a response, then it is diagnostic. Another diagnosis should be sought if the patient is unresponsive. IV is the preferred route of administration although naloxone can be given IM or SC if IV access cannot be established. Ventilatory support is key with 100% oxygen.

Answer 48

I. IV lorazepam This is a tonic-clonic, generalised seizure. It is characterised by LOC and widespread motor tonic contractions followed by clonic jerking movements. There will characteristically be a suppressed level of arousal following the event. This may either reflect a primary generalised episode or a focal seizure with secondary generalisation. The main aim of acute treatment is to terminate the seizure and to protect the airway. Management always starts with basic life-support (like every acute emergency) and your ABCs. IV access needs to be established (bloods sent to the lab too and serum glucose measured to test for reversable causes of seizure activity – thiamine should also be given to the patient if there is any concern about deficiency and hypoglycaemia, for instance in alcohol abuse). The following are needed: ECG, pulse oximetry, ABG. IV lorazepam is the preferred initial therapy, though rectal diazepam can be used if there is no IV access. If BZDs fail to stop the seizure then phenytoin or fosphenytoin can be tried. After the episode, MRI and EEG are essential in diagnosing an epilepsy syndrome. During the episode of generalised tonic-clonic activity, the EEG will show bilateral synchrony in the epileptiform activity. If this is a one-off seizure in which a provoking factor, such as electrolyte disturbance or hypoglycaemia, has been identified then there is no need for therapy for epilepsy. In unprovoked cases, this depends on history, examination, EEG and MRI. Treatment may not be needed the first time but after a second unprovoked instance, therapy is generally recommended.

Answer 49

G. Elevate legs This is a case of vasovagal syncope. Elevation of the legs will help move blood towards the head. The other options on the list are not necessary.

Answer 50

I. IV lorazepam This patient is in status epilepticus defined by 30 minutes or more of continuous seizure activity, or repetitive seizures with no intervening recovery of consciousness. SE can be either generalised convulsive or non-convulsive (simple or complex partial). In children, seizures of a shorter duration may also be considered to be status epilepticus. The initial treatment is as 2) and should start with BLS measures, continuous monitoring and benzodiazepines as first line therapy – with IV lorazepam. Securing the patient’s airwar may prove difficult due to the convulsions and neuromuscular blockade with a short-acting drug such as vecuronium may be necessary. Unresponsive cases can get anticonvulsants or phenobarbitone. It is worth noting that phenytoin infusions may lead to venous irritation and tissue damage if the undiluted drug is given through a small bore cannula. If SE persists, the next step to take is to intubate and start general anaesthesia. The best initial agents to use are midazolam and propofol though other options include pentobarbital and thiopental, the former being an active metabolite of the latter. GA should be tapered off after a minimum of 12 hours, and if the seziure recurs then the infusion should be restarted for another 12-24 hours.

Answer 51

F. IM benzylpenicillin This is suspected bacterial meningitis and in the community setting the patient should receive immediate IM benzylpenicillin before being transferred to hospital as an emergency case.

Answer 52

C. X-linked recessive Here is a list of the important conditions you should be aware of: Autosomal dominant: Achrondroplasia, APKD, dystrophia myotonica, familial hypercholesterolaemia, Huntington’s, Marfan’s, NF, tuberous sclerosis, osteogenesis imperfecta Autosomal recessive: CF, hereditary haemochromatosis, sickle cell disease, Wilson’s, Friedreich’s ataxia, CAH X-linked recessive: DMD, Fragile X syndrome, Haemophilia A and B, G6PDH deficiency, Alport’s You should be aware that Down’s is trisomy 21, Edwards’ is trisomy 18 and Patau’s is trisomy 13.

Answer 53

A. Autosomal recessive Here is a list of the important conditions you should be aware of: Autosomal dominant: Achrondroplasia, APKD, dystrophia myotonica, familial hypercholesterolaemia, Huntington’s, Marfan’s, NF, tuberous sclerosis, osteogenesis imperfecta Autosomal recessive: CF, hereditary haemochromatosis, sickle cell disease, Wilson’s, Friedreich’s ataxia, CAH X-linked recessive: DMD, Fragile X syndrome, Haemophilia A and B, G6PDH deficiency, Alport’s You should be aware that Down’s is trisomy 21, Edwards’ is trisomy 18 and Patau’s is trisomy 13.

Answer 54

G. Autosomal dominant Here is a list of the important conditions you should be aware of: Autosomal dominant: Achrondroplasia, APKD, dystrophia myotonica, familial hypercholesterolaemia, Huntington’s, Marfan’s, NF, tuberous sclerosis, osteogenesis imperfecta Autosomal recessive: CF, hereditary haemochromatosis, sickle cell disease, Wilson’s, Friedreich’s ataxia, CAH X-linked recessive: DMD, Fragile X syndrome, Haemophilia A and B, G6PDH deficiency, Alport’s You should be aware that Down’s is trisomy 21, Edwards’ is trisomy 18 and Patau’s is trisomy 13.

Answer 55

F. Chromosomal abnormality Here is a list of the important conditions you should be aware of: Autosomal dominant: Achrondroplasia, APKD, dystrophia myotonica, familial hypercholesterolaemia, Huntington’s, Marfan’s, NF, tuberous sclerosis, osteogenesis imperfecta Autosomal recessive: CF, hereditary haemochromatosis, sickle cell disease, Wilson’s, Friedreich’s ataxia, CAH X-linked recessive: DMD, Fragile X syndrome, Haemophilia A and B, G6PDH deficiency, Alport’s You should be aware that Down’s is trisomy 21, Edwards’ is trisomy 18 and Patau’s is trisomy 13.

Answer 56

B. None/ unknown Here is a list of the important conditions you should be aware of: Autosomal dominant: Achrondroplasia, APKD, dystrophia myotonica, familial hypercholesterolaemia, Huntington’s, Marfan’s, NF, tuberous sclerosis, osteogenesis imperfecta Autosomal recessive: CF, hereditary haemochromatosis, sickle cell disease, Wilson’s, Friedreich’s ataxia, CAH X-linked recessive: DMD, Fragile X syndrome, Haemophilia A and B, G6PDH deficiency, Alport’s You should be aware that Down’s is trisomy 21, Edwards’ is trisomy 18 and Patau’s is trisomy 13.

Answer 57

C. Acute urinary retention This is acute urinary retention caused by anticholinergics. Unwanted effects of this class of drugs include urinary retention, constipation, erectile dysfunction, CNS disturbance, cycloplegia, dry mouth and decreased sweating. Poisoning in severe cases can be treated with anticholinesterases such as physostigmine. This man will need to be catheterised first to relieve the urinary retention and prevent any further complications from occuring.

Answer 58

B. Labour Abdominal pain throughout pregnancy is common although reproductive organs share the name visceral innvervations as the lower ileum, sigmoid colon and rectum so it may be difficult to differentiate GI pain from gynaecological pain. This acute abdomen would require some consideration as the clinical picture may be distorted by the fact that pregnancy stretches the anterior abdominal wall so peritoneal signs are often different due to a lack of contact with the underlying inflammation, if an inflammatory process were to be present. The case described here could well be labour and the already overweight woman has simply been unaware of the fact she is pregnant (which although uncommon, can occur). The gross abdominal distension (which she may not attribute to anything abnormal if she is already fat) and symptoms are pointing to labour and there may also be uterine contractions. Blood or mucus on vaginal examination may be seen and you would expect the cervix to be soft, effaced and possibly dilated. However, this could be another complication of pregnancy or even a non-gynaecological condition and these need to also be considered during the diagnostic work-up. An ultrasound scan, which is safe to use in pregnancy, should be done here.

Answer 59

H. Bladder cancer Gross haematuria is the primary symptom of bladder cancer. Over 80% present with haematuria which is the primary presenting complaint in this condition. The gross haematuria is classically painless and present throughout the entire urinary stream, however while painful the rest of the symptoms fit. It is also worth noting that carcinoma in situ commonly presents with dysuria and frequency and can easily be confused with prostatitis. Risk factors include smoking, exposure to carcinogens such as the aromatic amines used in rubber and dye industries, age >55, pelvic radiation and Schistosomiasis resulting in SCC (related to chronic inflammation – so other risks also include UTI, stones etc). Bladder cancer is the most common cancer in Egypt, for the latter reason. Cystoscopy and urinary cytology are key in diagnosis. Low grade tumours are papillary and easy to see on cystoscopy whereas high grade tumours and carcinoma in situ are often difficult to visualise. Resection provides diagnosis and primary treatment in one step.

Answer 60

A. Urinary tract infection This young woman has presented with a UTI (dysuria, frequency, and foul smelling urine). Sexual activity the strongest risk factor for UTIs in women. It is diagnosed with a urine dipstick and microscopic analysis for bacteria, WBC and RBC and urine culture and antibiotic sensitivities. Antibiotic selection should be based on local guidelines or known sensitivities. Uncomplicated UTIs with no known antibiotic resistance can be treated with co-trimoxazole or nitrofurantoin. If there is resistance, a quinolone can be considered such as ciprofloxacin.

Answer 61

I. Urinary tract stones: ureteric colic/ stricture This patient has renal colic which classically presents with severe flank pain radiating to the groin. Microscopic haematuria is present in up to 90% of cases. Up to 85% of stones are visible on a plain KUB although urate stones are radiolucent. If the stone is radio-opaque, calcification will be seen within the urinary tract. In pregnancy, a renal USS is first line. The IVP has now been replaced by the CT scan which is the new diagnostic standard otherwise. A non-contrast helical (or spiral) CT is preferred due to high sensitivity and specificity and acurately determines presence, site and size of stones. Stones are analysed after they are extracted or when they are expelled to check their composition. It is worth noting that in all females of child bearing age, a urine pregnancy test is necessary to exclude an ectopic pregnancy.

Answer 62

J. Myocardial infarction This diabetic is having a silent MI without chest pain. All of the other symptoms are indicative such as vomiting, sweating and tachycardia. Silent MIs are more common in the elderly and those with DM probably due to autonomic neuropathy. If ECG shows STEMI, new LBBB or confirmed posterior MI then PCI/thrombolysis is indicated. It is worth noting that RV infarction is present in 40% of inferior infarcts so if ST elevation is seen in II, III and aVF, right sided ECG leads should be obtained. Cardiac biomarkers include CK-MB and troponin. Troponins rise 4-6 hrs after onset of infarction and peak at 18-24 hours and may persist for 7-10 days.

Answer 63

D. Meningitis From the list of options given, the only feasible option is meningitis. This history is not consistent with a migraine. Commonly in meningitis, there will be a headache, fever and nuchal rigidity. There may also be an altered mental status, confusion, photophobia and vomiting. Kernig’s sign is uncommon but is positive when attempts to extend the leg are met with resistance when the patient is supine with the thigh flexed to 90 degrees. Another uncommon sign is Brudzinski’s sign and a petechial/purpuric rash, typically associated with meningococcal meningitis. CT head should be considered before LP if there is any evidence of raised ICP. An LP will confirm the diagnosis with bacterial meningitis showing a low CSF glucose, elevated CSF protein and positive CSF culture/gram stain or meningococcal antigen.

Answer 64

G. Pancreatitis This patient has pancreatitis. He has vomited heavily and is describing mid-epigastric pain radiating around to the back. This description of the pain itself is almost diagnostic. This pain is classically relieved if the patient curls up and is worse with movement. Complicated haemorrhagic pancreatitis may exhibit Cullen’s sign, Grey-Turner’s sign and Fox’s sign. Make sure you know what these are and you are familiar with the causes of acute pancreatitis – it seems likely to be alcohol in this instance. Those caused by hypocalcaemia may display Chvostek’s sign and Trousseau’s sign. Key to diagnosis is serum amylase or lipase levels which are massively elevated. Prognostic criteria are outlined in Ranson’s criteria applied on admission and after 48 hours, or the modified Glasgow score which you can find in your Oxford Handbook. An abdominal CT is however the most sensitive and specific study and findings may include enlargement of the pancreas with irregular contours, necrosis, pseudocysts and peripancreatic fat obliteration.

Answer 65

E. Small bowel obstruction Bowel obstruction has several causes such as adhesions or cancer in the older patient. In this case the PMH of recent surgery suggests adhesions as a likely aetiology. The profuse vomiting, distended and colicky painful abdomen and increased tinkling high pitched bowel signs are all indicative. The proximal segment of bowel dilates and distal bowel collapses. Completely obstructed patients generally require surgery. If, on AXR, air is seen to be seeping past the obstruction then the obstruction is partial. As a standard, all patients should be made NBM and given supplemental oxygen, IV fluids and NG decompression (to reduce flow/gastric contents/air towards the obstruction), unless they are rushed off for an emergency laparotomy because, for example, they have complete SBO and are peritonitic. Small bowel vs. large bowel obstruction Small bowel obstruction tends to be more acute, tinkling bowel sounds can be heard, pain is central and colicky, , and is particularly linked to post-surgical adhesions Large bowel obstruction is more gradual, bowel sounds are diminished or normal, pain is localised

Answer 66

A. Labrynthitis Labrynthitis is an inflammatory condition affecting the labyrinth in the cochlea and the vestibular system of the inner ear. The most common cause is viral in origin although bacterial laryrinthitis occurs as a complication of otitis media or meningitis. The typical presentation is one including vertigo, imbalance and hearing loss. The typical description is of acute rotational vertigo which may last up to 7 hours. This is often associated with N&V. Risk factors include recent URTI or other viral infections such as VZV, CMV, mymps, measles, rubella and HIV. Treatment is typically symptomatic and involves the use of vestibular suppresants (benzodiazepines such as diazepam) and antiemetics (such as promethazine or metaclopramide). Labyrinthitis is the only condition on the list which would cause this constellation of symptoms this patient is describing. In reality, this presentation could of course be vestibular neuritis.

Answer 67

A. Endometriosis Endometriosis is a chronic inflammatory condition defined by endometrial stroma and glands located outside of the uterine cavity – the most common sites being the pelvic peritoneum and ovaries. It may present as an incidental finding in asymptomatic patients but more commonly it presents in women of reproductive age with chronic pelvic pain and/or subfertility. This woman has symptoms which make this diagnosis likely. There is dyspareunia which is pain during sexual intercourse, particularly on deep penetration, and may be caused by a distortion in the pelvic anatomy and rectovaginal involvement. There is also well documented genetic predisposition so a positive FH may be found. Additionally, nulliparous women are more likely to be diagnosed with endometriosis than parous women. The diagnosis can be confirmed on visualising the ectopic tissue directly and focused biopsies during laparoscopy but this is not generally necessary as clinical suspicion is enough to start treatment. Options for treatment include NSAIDs, COCPs, GnRH agonists, danazol or related androgens and surgical destruction of lesions. Those who present with subfertility may be considered for ovarian hyperstimulation and IVF.

Answer 68

A. Endometrial cancer This is a common malignancy and is usually an adenocarcinoma. Obesity is associated with an increased incidence of endometrial cancer and also poorer outcome. Risk factors to consider aside from obesity include HRT, tamoxifen use, age over 50, unopposed oestrogen and radiotherapy. FH and a history of other cancers are also risks. A good history is important to establish that PV bleeding does not have another obvious cause such as intercourse or that associated with HRT, and that is is unlikely to be related to another malignancy like cervical cancer. Examination tends to be challenging due to the prevalence of obesity in those with endometrial cancer. The presentation is typically with post-menopausal bleeding PV and often the disease is surgically curable. Diagnosis will need to be confirmed by biopsy and histology with histopathology showing adenocarcinoma.

Answer 69

F. Adenomyosis Adenomyosis, as you can probably figure out from the name, is the presence of ectopic glandular tissue in muscle. The first test to order is a pelvic USS which shows a normal or enlarged uterus. It is of minimal diagnostic value, especially if the clinical history and examination findings are suggestive, but can be used to rule out endometriosis, where the USS may show the presence of ovarian endometriomas. Examination may reveal an enlarged globular uterus and uterine tenderness on palpation, particularly during the menses. The history is usually of a parous woman, symptoms commonly occuring after childbirth, with heavy menstrual flow or an abnormal bleeding pattern being seen. Adenomyosis is a condition which can be diagnosed clinically, although if a costly MRI pelvis is done, then abnormal signal intensities within the myometrium can be seen.

Answer 70

D. Ectopic pregnancy Ectopic pregnancy usually presents between 6-8 weeks after the last normal menstrual period but it can present later on. The risk increases if the woman has had a previous ectopic, surgery on the tubes, genital infections, smokes or uses an IUD. The classic symptoms and signs are pain, vaginal bleeding and amenorrhoea. If the patient is haemodynamically unstable or there is cervical motion tenderness, this may indicate that a rupture has occured or is imminent. Rupture, which is a complication, can present with shock from blood loss and with unusual patterns of referred pain from the presence of intraperitoneal blood. The positive urine pregnancy test here confirms pregnancy. Once the patient is confirmed to be pregnant, a transvaginal USS is used to determine the location of the pregnancy. If an intrauterine gestation is visible on USS regardless of whether it is viable, then the chances of having an ectopic pregnancy are incredibly low. Occasionally, an ectopic pregnancy itself can be seen, either as a ‘doughnut sign’ (adnexal mass separate from two clearly seen ovaries) or ‘ring of fire’ (increased blood flood to the ectopic seen on colour Doppler). A transabdominal ultrasound is less sensitive than a TVUS. Treatment approaches can include expactant, medical (methotrexate) or surgical (salpingectomy, salpingostomy).

Answer 71

B. Pelvic inflammatory disease Pelvic inflammatory disease is an acute ascending infection of the female tract that is often associated with Neisseria gonorrhoeae or Chlamydia trachomatis. Key risk factors include prior infection with chlamydia or gonorrhoea or PID, young age of onset of sexual activity, unprotected sex with multiple partners and IUD use. Signs and symptoms vary and can include tenderness of the lower abdomen, adnexal tenderness and cervical motion tenderness. Fever and cervical or vaginal discharge may also be present. Complications include tubo-ovarian abscess and subsequent infertility or ectopic pregnancy due to scarred or obstructed fallopian tubes.

Answer 72

G. Caecal carcinoma The weight loss, fatigue and RIF discomfort point to caecal carcinoma. Right sided colorectal cancer tends to present with anaemic symptoms. Almost 90% are anaemic at diagnosis.

Answer 73

E. Haemorrhoids Haemorrhoids are vascular rich cushions in the anal canal and presents, typically, as painless bright PR bleeding or with sudden onset pain in the area associated with a palpable mass. Pruritus ani is common and there is often perianal pain or discomfort. Diagnosis is made visually. Grade 1 is limited to within the anal canal. Grade 2 protrudes but spontaneously reduces when the patient stops straining. Grade 3 protrudes and reduces fully on manual pressure. Grade 4 is irreducible. Treatment includes fibre, ligation, photocoagulation, sclerotherapy or surgical haemorrhoidectomy. Haemorrhoidectomy is the treatment of choice of choice for patients with grade 4 haemorrhoids or for any patient who has failed with more conservative treatment such as sclerotherapy.

Answer 74

J. Duodenal ulcer Haemorrhoids are vascular rich cushions in the anal canal and presents, typically, as painless bright PR bleeding or with sudden onset pain in the area associated with a palpable mass. Pruritus ani is common and there is often perianal pain or discomfort. Diagnosis is made visually. Grade 1 is limited to within the anal canal. Grade 2 protrudes but spontaneously reduces when the patient stops straining. Grade 3 protrudes and reduces fully on manual pressure. Grade 4 is irreducible. Treatment includes fibre, ligation, photocoagulation, sclerotherapy or surgical haemorrhoidectomy. Haemorrhoidectomy is the treatment of choice of choice for patients with grade 4 haemorrhoids or for any patient who has failed with more conservative treatment such as sclerotherapy.

Answer 75

D. Carcinoma of the rectum This a rectal carcinoma. Tenesmus, blood PR alongside weight loss are all highly suggestive. Treatment of rectal carcinoma involves surgical excision where possible. This can either be an anterior resection (tumours in the upper 1/3 of the rectum) or an abdominoperineal resection (if the tumour lies lower down). APER involves the formation of a permanent colostomy and has a high incidence of sexual and urinary dysfunction. Anterior resection involves a colo-anal anastamosis.

Answer 76

B. Inflammatory bowel disease This woman has inflammatory bowel disease, which by the history is probably more likely to be UC than CD, whereby the mainstay of treatment is with 5-ASA. A colonoscopy is required to assess the extent of disease and for a definitive diagnosis. Biopsy in CD will show transmural granulomatous inflammation. CD can affect the whole GIT but favours the TI and proximal colon and is macroscopically characterised by skip lesions. UC on the other hand is characterised by the presence of crypt abscesses, which is pathognomic. CD risk is increased 3-4 fold by smoking whereas smoking seems protective in UC. The mainstay of treatment in CD is with steroids and azathioprine to prevent relapses and for those suffering side effects of steroid treatment. TNF-alpha inhibitors also have a role. Surgery in CD is only indicated in a small number of patients who bleed, for bowel perforation and cases of complete obstruction. The aim is to rest distal disease by temporarily diverting faecal flow.

Answer 77

G. Addison's disease Hyperpigmentation in the palmar creases points towards Addison’s disease. Hyperpigmentation due to excess ACTH production can be mucosal or cutaneous and is more pronounced in the palms, knuckles and around scars. MSH is a byproduct of the production of ACTH from the cleavage of POMC. Anorexia, fatigue and weight loss is observed in all patients. Sodium is low and potassium elevated. Vomiting is present in 75% of patients and nausea is a common finding. Additionally, postural hypotension may be present. The presence of other autoimmune diseases is a risk factor for the development of Addison’s. Diagnosis of Addison’s can be made on an ACTH stimulation test (synacthen test) whereby serum cortisol remains low despite the administration of synthetic ACTH. In an emergency, treatment should not be delayed by diagnostic testing.

Answer 78

K. Glandular fever This is infectious mononucleosis or glandular fever and is caused by EBV. It is characterised by fever, pharyngitis and lymphadenopathy. Enlargement of the spleen begins in the first week and lasts 3-4 weeks, occuring in half of all cases. Risk factors for EBV transmission include kissing and sex. The fact her boyfriend has had similar symptoms should raise your suspicion. A FBC will show an atypical lymphocytosis. Confirmation of IM involves detection of the existence of heterophile antibodies using the Paul Bunnell monospot. A more accurate test is a serological test detecting EBV specific antibodies. Treatment is usually symptomatic but IM can carry rare but potentially life threatening complications.

Answer 79

F. Diabetes mellitus Polyuria, polydipsia, nocturia in a boy who is tired all the time should make you think of T1DM. Insulin is needed alongside dietary changes and exercise. Insulin regimes aim to mimic physiological insulin release with a basal-bolus dosing. There is an option between using a pump and having multiple daily injections. It is worth noting that there is a high incidence of diabulimia among young people with T1DM who give themselves less insulin than they need in order to lose weight (they lose weight, ‘look good’ but trash their bodies).

Answer 80

E. AIDS AIDS (acquired immunodeficiency syndrome) is caused by HIV, which is a retrovirus. To give you an indication of risk here, there is a risk of 50 infections per 10,000 exposed to an infected source in unprotected receptive anal intercourse. The risk with receptive vaginal intercourse is 10 infections per 10,000 exposures. Obviously, people have sex more often than the one off, as is human nature, so what seems like a small risk per sexual encounter adds up. IVDU needle sharing has a risk of 67 per 10,000, a needle-stick is 30 per 10,000 (equal to having receptive vaginal intercourse 3 times with an HIV positive man, so be careful on the wards, though this statistic does depend on factors like the size of the needle) and the risk associated with vertical transmission is associated with maternal viral load (the risk goes if you can suppress the viral load with anti-retrovirals). The thing to note is that the association with homosexuality is based on the increased risk of transmission from receptive anal compared to receptive vaginal, and if you happen to be a homosexual male and contract HIV, you’re unlikely to pass it on with vaginal intercourse. There should not be negative stigma attached to HIV. There are two types, HIV 1 which is the main virus responsible and HIV 2 which is restricted to parts of West Africa. Weight loss is common in HIV and if more than 10% body weight is lost or BMI reduces to 18.5, this is an indication of more severe immunocompromise. Weight loss in HIV may result from malnutrition, co-existent TB infection or HIV wasting syndrome, the latter being an AIDS defining illness. The purple lesions seen here are due to Kaposi’s sarcoma, which is a neoplasm derived from mesenchymal tissue, associated with HHV-8 infection. This is an AIDS defining infection. There are WHO (stage 1-4) and CDC criteria used in clinical staging of HIV. This patient needs to have a CD4 count, HBV and HCV screen, VDRL (syphilis), tuberculin skin test (TB) and CXR. HIV viral load will also be assessed. Prophylaxis and immunisations should be considered against infections such as hepatitis, influenza, PCP and TB. HAART needds to be initiated as he has developed AIDS. Classes of antiretrovirals include NRTIs, NNRTIs, protease inhibitors, fusion inhibitors and integrase inhibitors.

Answer 81

D. Hypothyroidism There is weight gain, cold intolerance, fatigue and constipation as well as bradycardia which all point to hypothyroidism. Worldwide, the most common cause is iodine deficiency. Other causes include Hashimoto’s or secondary and tertiary hypothyroidism. It can also result from viral de Quervain’s thyroiditis or postpartum thyroiditis. Symptoms include those mentioned as well as depression, bradycardia, sluggish reflexes, constipation, cold intolerance and muscle cramps. Diagnosis is based on measurement of TSH and thyroid hormones. Treatment is by replacement of T4 with or without T3 in combination. If the patient has normal T3 and T4 but mildly elevated TSH, this is described as subclinical hypothyroidism.

Answer 82

L. Sickle cell anaemia Africans have higher incidence of sickle cell anaemia. The jaundice here is due to haemolysis and accounts for the raised unconjugated bilirubin. The splenomegaly here is further suggestive. About 8% of black people carry the gene and the prevalence is high in sub-Saharan Africa. The condition is autosomal recessive and therefore occurs in 1 in 4 pregnancies where both parents carry the sickle gene. Sickling occurs when RBCs containing HbS become distorted into a crescent shape. Patients with sickle cell anaemia have no HbA at all. If both parents carry the sickle cell gene, there is a 1 in 4 chance of giving birth to a child with sickle cell anaemia. Sickle cell disease also includes other conditions such as HbS from one parent with another abnormal Hb or beta thalassaemia from the other parent such as HbS-Beta thal and HbSC. Treatment goals include fluid replacement therapy, pain management where necessary and symptomatic control.

Answer 83

D. Gilbert's syndrome Gilbert’s occurs in an asymptomatic patient, often as an incidental finding or mild jaundice occuring in adolescence/young adult age, or during times of physiological stress (this patient has a cold). There is elevated unconjugated BR with other liver tests being normal. The blood smear is also normal with normal reticulocyte count, and normal Hb indicating that this is not due to haemolysis. It is a common syndrome and is not really a disease, more a physiological variant. No treatment is needed and this condition is due to decreased UDPGT activity leading to decreased conjugation of unconjugated bilirubin, leading to elevated levels. Positive FH is common as this condition is most likely transmitted in an autosomal recessive pattern.

Answer 84

J. Hepatitis A IgM anti-hepatitis A virus is positive here which is highly sensitive and specific combined with the typical symptoms this student displays. IgM antibodies are detectable typically 5-10 days before symptom onset and remain raised for 4-6 months. It can be ordered alongside IgG anti-HAV and is a cheap and simple test. IgG rises soon after IgM and stays elevated for life so a positive IgG can mean prior infection or recent disease. Again, a cheap and simple to carry out test. Hepatitis A is primarily transmitted via the faecal-oral route. After the virus is consumed and absorbed, it replicates in the liver and is excreted in the bile (to be re-transmitted). Transmission usually precedes symptoms by about 2 weeks and patients are non-infectious 1 week after onset of jaundice. The history can reveal risk factors such as living in an endemic area, contact with an infected person, homosexual sex or a known food-borne outbreak. This is classically, in EMQs, associated with shellfish which is harvested from sewage contaminated water. If the patient has other liver diseases such as HBV or HCV or cirrhosis then there is a higher risk of fulminant HAV infection. The clinical course of HAV consists of a pre-icteric phase, lasting 5-7 days, consisting characteristically of N&V, abdominal pain, fever, malaise and headache. Rarer symptoms may be present such as arthralgias and even severe thrombocytopenia and signs that may be found include splenomegaly, RUQ tenderness and tender hepatomegaly as well as bradycardia. The icteric phase is characterised by dark urine, pale stools, jaundice and pruritis. When jaundice comes on, the pre-icteric phase symptoms usually diminish, and jaundice typically peaks at 2 weeks. However, a fulminant course runs in <1% of patients with worsenining jaundice and encephalopathy. Serum transaminases may reach in excess of 10,000 units, although there is little correlation between the level and disease severity. ALT is typically higher than AST.

Answer 85

E. Primary sclerosing cholangitis Primary sclerosing cholangitis is a cholestatic liver disease which causes bile duct destruction, cirrhosis and end-stage liver disease. It predominantly affects young and middle-aged men, often with underlying inflammatory bowel disease. There is an association of PSC with UC (typically) and IBD diagnosis tends to precede that of PSC with a mean time from onset of IBD to PSC of 9 years. This patient has evidence of obstructive jaundice and pruritis. The diagnosis involves laboratory tests in combination with cholangiography. There are no specific auto-antibodies specific to or diagnostic of PSC but a number of serum autoantibodies such as ANCA are often present in PSC patients. There is currently no effectively medical therapy and the only treatment option in those with advanced disease is to have a liver transplant. The leading cause of death in these patients is liver failure and cholangiocarcinoma. The latter is a relatively common complication and should be suspected in patients who present with rapidly progressing jaundice, weight loss or abdominal pain on a background of PSC.

Answer 86

M. Carcinoma of the head of the pancreas Pancreatic cancer (of the head) typically presents with painless obstructive jaundice and weight loss and generally presents late. Whipple’s procedure or Traverso-Longmire procedure (pancreaticoduodenectomy) offers the only hope of a cure but only a small minority are elegible for these procedures. The first tests to order are an abdominal USS and LFTs. Note Courvoisier’s law: Jaundice and a palpable painless gallbladder is unlikely to be caused by gallstones. The tumour marker for pancreatic cancer is CA19-9 which is useful in preoperative staging.

Answer 87

B. Nebulised salbutamol This patient is having an asthma attack. The best option here is nebulised salbutamol initially. Early systemic corticosteroids and supplemental oxygen should be considered and the patient’s status needs to be monitored regularly.

Answer 88

A. Oral clarithromycin This is a CAP which is confirmed by CXR shadowing, which would show airspace shadowing with air bronchograms. The history is also consistent with respiratory symptoms and pyrexia. The most sensitive test for CAP is a CXR. The treatment is initially empirical with antibiotics and management would be guided by this patient’s CURB-65 score. A macrolide is the first choice in adults with no contra-indications without a recent course of antibiotics or risk of drug resistance, but always check local prescribing policies. Azithromycin, clarithromycin or erythromycin are all valid choices here.

Answer 89

E. IV cefuroxime This is an acute infective exacerbation of COPD and the patient has already received nebulised salbutamol and corticosteroids. The next important thing to do is to give IV antibiotics. Inpatient therapy for severe infections can be with IV ceftriaxone with azithromycin. High risk patients should receive piperacillin and tazobactam, or meropenem, to cover for pseudomonas.

Answer 90

G. Diuretic This patient has developed pulmonary oedema which accounts for the history and examination findings. The CXR indicates the cause is heart failure. He will require diuretics and fluid restriction to deal with his overloaded state. Patients need to be sat upright to improve the SOB and IV access needs to be established. Oxygen, morphine, diuretics (frusemide or another loop diuretic) and nitrates will be given. Once stable, medical treatment of heart failure should be started which involves in the first instance, an ACE inhibitor followed by beta blockade.

Answer 91

D. Opiate Opiates are commonly prescribed for analgesia and can also be used as an anti-tussive in this case (suppresses ACh and NK release needed for cough activation and inteferes with serotonin receptors in the DRN). They are also abused for their euphoric effects mediated by their action on dopamine release at the nucleus accumbens. They have a host of side effects: GI effects (receptors present on the myenteric and submucosal plexus) of decreased gastric motility, emptying and increased gut water reabsorption... in short, constipation; Respiratory depression (desensitises central chemoreceptors to lessen the response to PaCO2); N&V by stimulating the CTZ; Pupillary constriction via the Edinger-Westphal nucleus; And causes symptoms like pruritis, urticaria, hypotension through histamine release via a direct effect on mast cells.

Answer 92

F. Methadone program This patient is a regular recreational opiate user and should be educated about the dangers, particularly of overdose. Needle sharing puts the patient at risk of infections such as HIV and HCV and advice should also be given on the use of clean needles or techniques on how to clean needles. This patient should be commended on wanting to take part in a detoxification programme for his chronic problem. Methadone is a drug which is used in opioid dependence as substitution therapy. Buprenorphine is another drug which can be used for this purpose and both are recommended in NICE guidelines. This medication can be started with a short period of stabilisation followed by either a withdrawal regimen or maintenance treatment, which reduces drug use and crime and improves health. Methadone is a long-acting opioid agonist, usually given as a single daily oral solution. It has a long half life and takes 3-10 days for plasma levels to reach a steady state. Buprenorphine is a partial opioid receptor agonist (i.e. it has both opioid agonist and antagonist properties) and some patients may prefer this drug as it is less sedating than methadone so may be more suitable for people who drive around, for instance. Adjunctive therapy may be needed to manage withdrawal symptoms. Loperamide can be used for diarrhoea, mebeverine for stomach cramps, paracetamol and NSAIDs for muscular pains and headaches, and metaclopramide or prochlorperazine for N&V. Lofexidine is an alpha-2 agonist which can be used to alleviate physical symptoms of opioid withdrawal. Remember that naloxone is an opioid-receptor antagonist used to reverse opioid overdose (those dependent can be given a supply in case they ‘accidentally’ overdose) and naltrexone is prescribed to prevent relapse in formerly dependent patients.

Answer 93

J. Dilsulfiram Disulfiram is used as an adjunct in the treatment of alcohol dependence. It leads to an unpleasant systemic reaction after taking even the smallest amount of alcohol as it causes acetaldehyde to accumulate in the body. Disulfiram blocks the metabolism of alcohol. It is only effective if taken daily and symptoms can occur within 10 minutes of ingesting any alcohol and include facial flushing, throbbing headache, palpitations, tachycardia, N&V and possible arrhythmias, hypotension and collapse with large doses of alcohol. These reactions can last several hours. Even the smallest amounts of alcohol in medications and even mouthwash can lead to a reaction. After stopping treatment, alcohol still should be avoided for at least a week. Other medications that can be used to prevent relapse and support abstinence include acamprosate (which stabilises glutamate and GABA systems) and naltrexone which is an opioid antagonist.

Answer 94

B. Physician advice This (likely medical) student is not dependent nor does he have any physical symptoms but is concerned and aware that his drinking is harmful. At this stage he needs advice on the dangers of excessive alcohol consumption which is the first line intervention for those with patterns of problematic alcohol use but are not dependent (as well as those with mild dependence). The consultations can consist of one or more sessions with a doctor during which feedback can be given about the patient’s alcohol use and the consequences in a supportive and empathic manner. The doctor and patient can work together to develop an action plan for cutting down or eliminating alcohol use and then assessing how effective this is with subsequent visits. Motivational interviewing is a technique which can also be deployed in this situation.

Answer 95

I. Inpatient detoxification with chlorodiazepoxide Chlordiazepoxide is a long-acting benzodiazepine which is used to attenuate alcohol withdrawal symptoms. People with severe dependence should undergo withdrawal in an inpatient setting – withdrawal in those who are severely dependent without medical support can lead to seizures, DT and death. A symptom triggered flexible regimen is typically used in hospital and continued assessment and monitoring is carried out for 24-48 hours, usually followed by a 5 day reducing dose schedule. When BZDs are contraindicated, carbamazepine can be used as an alternative. Clomethiazole can also be used in the acute withdrawal setting although BZDs are preferred. Patients with marked agitation or hallucinations, and those at risk of DT can be given antipsychotic drugs such as haloperidol as an adjunct. This patient should also be given parenteral thiamine (as Pabrinex) due to the risk of developing Wernicke’s encephalopathy from chronic alcohol abuse.

Answer 96

C. Alcoholics anonymous Alcoholics Anonymous is a help group where people share their experiences and help each other recover from alcoholism.

Answer 97

C. Anorexia nervosa Anorexia nervosa often occurs in women which is a key risk factor. Puberty/adolescence and obsessive personality traits are additional risks. There is a higher incidence reported in western cultures (presumably skinny models on TV, adverts etc...) and studies on immigrants moving to a western culture exhibited a higher incidence. There is also postulated to be a genetic contribution from twin studies.Those who in higher socioeconomic classes are more affected. BMI is usually <17.5 and the patient often has a fear of weight gain and will refuse intervention to gain weight. The patient often fails to acknowledge how thin they are and can indicate how some parts of her body are ‘big’. Amenorrhoea is also a common complaint. Often anorexics eat vegetarian or vegan diets and you should inquire about the patient’s eating habits.There are two subtypes of AN. Restrictive where the patient will diet and exercise or the bingeing/purging types where there is also calorie restriction but ocassional binges and purging afterwards. There is also a tendency to laxative, diuretic and enema abuse.

Answer 98

H. Hyperthyroidism TSH is the initial screening test and if suppressed, T4/T3 levels are measured. Treatment aims to normalise thyroid function and is achieved by radioactive iodine, anti-thyroid medications or with surgery. They are all effective and relatively safe options. Symptomatic therapy is given with beta blockers such as propranolol.

Answer 99

J. HIV HIV is a retrovirus and there are two types, HIV 1 which is the main virus responsible and HIV 2 which is restricted to parts of West Africa. Weight loss is common in HIV and if more than 10% body weight is lost of BMI reduces to 18.5, this is an indication of more severe immunocompromise. Weight loss in HIV may result from malnutrition, co-existent TB infection or HIV wasting syndrome, the latter being an AIDS defining illness. Generalised lymphadenopathy is also common and is characterised by the painless enlargement of 2 more more non-contiguous sites of >1cm for >3 months. Neutropenia is also seen due to CD4 deficiency and thrombocytopenia may also be seen along with an anaemic picture. There are WHO (stage 1-4) and CDC criteria used in clinical staging. This patient needs to have a CD4 count, HBV and HCV screen, VDRL (syphilis), tuberculin skin test (TB) and CXR. HIV viral load will also be assessed. Prophylaxis and immunisations should be considered against infections such as hepatitis, influenza, PCP and TB. When to initiate HAART depends on the clinical stage, CD4 and co-morbidities. This patient will need to be started on HAART. Classes of antiretrovirals include NRTIs, NNRTIs, protease inhibitors, fusion inhibitors and integrase inhibitors.

Answer 100

K. Malignancy This person likely has lung cancer which has resulted in the significant weight loss of 10kg. This is post-obstructive pneymonia which is common in lung cancer patients and is caused, most of the time, by a large and centrally obstructing tumour. It is essential to relieve this obstruction in this case and many techniques can be tried. There is also bone pain here in the lower spinal column which is due to metastases. The prognosis here is not good.

Answer 101

E. Malabsorption There is frequent loose stools here and abdominal pain. Combined with the IDA, this points to malabsorption. This could well be a presentation of coeliac disease – IDA is one of the most common clinical presentations and abdominal pain and diarrhoea are common. Coeliac disease is a systemic autoimmune condition triggered by dietary gluten peptides found in grains. It is a relatively common condition. The only treatment is a strict gluten-free diet for life.

Answer 102

F. Asterixis Asterixis is a flapping tremor, or a liver flap, which is a coarse tremor of the hand when the wrist is held extended. It is caused in this case by liver failure and can be a sign of hepatic encephalopathy. The signs found on examination point to decompensated chronic liver disease.

Answer 103

D. Sydenham's chorea Chorea features as part of the acute presentation in 5-10% of patients with rheumatic fever. It can also occur as an isolated event up to 6 months after the initial GABHS infection. It is named Sydenham chorea after the doctor who described St Vitus Dance in the 17th century. Choreiform movements can affect the whole body or just one side of the body, in which case it is referred to as hemi-chorea. The head is often involved with erratic facial movements that resemble grimaces, grins and growns, and the tongue may be affected to resemble a bag of worms when protruded, and protrusion cannot be maintained. In severe cases the patient may have an impaired ability to eat. Chorea disappears with sleep and is made worse by purposeful movements. When the patient is asked to grip the doctor’s hand, the patient will be unable to maintain grip and rhythmic squeezing occurs. There are two signs to look out for in these patients. The first is the spooning sign, which is a flexion at the wrist with finger extension when the hand is held extended. The pronator sign is the second which is when the palms turn outwards when held above the head. Both are consistent with chorea. Remember that the 5 major manifestations of acute rheumatic fever are carditis, polyarthritis, chorea, erythema marginatum and SC nodules – the most common of which are carditis and polyarthritis. Primary episodes occur mainly in children aged 5-14 and are rare in those over 30. The greatest burden of disease remains in the developing countries and in populations of people living in poverty.

Answer 104

E. Exaggerated physiological tremor The tremor seen in hyperthyroidism is usually fine and is a exaggerated physiological phenomenon. Treatment aims to normalise thyroid function and is achieved by radioactive iodine, antithyroid medications or with surgery. They are all effective and relatively safe options. Symptomatic therapy is given with beta blockers such as propranolol.

Answer 105

H. Cerebellar tremor A cerebellar tremor can be seen in MS, trauma or stroke. The history may feature complaints of incoordination and imbalance, a possible FH of cerebellar ataxia or PMH of MS, head trauma, stroke or cerebellar haemorrhage. The tremor itself is a coarse and irregular kinetic tremor which is generated proximally. There may also be abnormal finger-to-nose testing and heel-to-shin testing, dysdiadochokinesia, wide-based ataxic gait (like the patient is drunk) as well as dysarthria (speech problems). The first test to order is an MRI of the patient’s head which may show signs of cerebellar atrophy or may suggest changes of demyelination seen in MS or changes of stroke, trauma or haemorrhage. You may also want to perform other tests such as thyroid function tests to exclude hyperthyroidism.

Answer 106

B. MS MS is a demyelinating CNS condition which is characterised by 2 or more episodes of neurological dysfunction which are separated in both time and space. MS classically presents in white women aged 20-40 with temporary visual/sensory loss although any presentation can occur. MRI is a sensitive test but less specific than spinal MRI, however, spinal MRI is abnormal in fewer cases. Treatment aims at treating the attack, preventing future attacks and symptomatic treatment of problems like bladder dysfunction, pain and fatigue.

Answer 107

J. Cystic hygroma A cystic hygroma is a cystic lymphatic lesion which occurs as a congenital birth defect. It can arise anywhere but is classically found in the left posterior triangle of the neck and is the most common lymphangioma. There are large cyst like cavities in this lesion containing a watery fluid and as such it transilluminates. They are a benign lesion but can be disfiguring and is a condition which very rarely presents in adulthood, and tends to affect children. It can be seen as part of Noonan’s syndrome, which is an autosomal dominant disorder characteristically with short stature, chest deformity, congenital heart defects and unusual facial features.

Answer 108

H. Parotitis Parotitis is inflammation of one or both parotid glands. This is a form of sialadenitis, with the parotid glands here being enlarged and inflammed. Pain and dysphagia are common. Fever may also be noted in acute bacterial cases. Patients with acute bacterial sialadenitis may give a history of recent surgery or the use of medications such as antihistamines, antidepressants or anticholinergics, which may also lead to xerostomia. Stones in the glandular ducts (sialoliths) can result in obstructive sialadenitis, although there is no evidence from this case that this is the cause. Other causes include Sjogren’s syndrome which can cause an autoimmune sialadenitis. In terms of management, pus swabs should be taken from any exudate and imaging should be done to exclude an obstructing salivary gland stone or evolving abscess. Plain dental radiographs are the investigation of choice with CT imaging done if plain radiographs come back negative and a stone is suspected. USS is useful if ab abscess is suspected. FBC can be done to evaulate WCC and if a fever is present, blood cultures should be done to guide antibiotic therapy.

Answer 109

F. Thyroglossal cyst This midline neck swelling moves up on both swallowing and tongue protrusion making this a thyroglossal cyst. It is a cyst that forms from a remnant thyroglossal duct and can hence develop anywhere along the length of this embryological duct, which is a midline structure between the foramen caecum at the back of the tongue and the thyroid gland.

Answer 110

A. Pharyngeal pouch A pharyngeal pouch is also called a Zenker’s diverticulum (it is a false diverticulum – i.e. it does not involve all layers of the oesophageal wall). The dehiscence of Killian lies in the posterior laryngopharynx which is where the wall herniates through giving an outpouching just above the cricopharyngeal muscle. It may help to gave a look at a picture in an anatomy textbook. A pharyngeal pouch can cause symptoms of dysphagia and the sensation of a lump in the neck (which can frequently be misdiagnosed as globus hystericus). There may also be regurgitation of food, cough, halitosis and gurgling noises and the condition is associated with webs. There is usually no pain except in the presence of a carcinoma. Additionally, it may be asymptomatic. Examination may reveal a swelling in the neck which is usually found on the left side, in the lower part of the anterior triangle, which is soft and may gurgle on palpation (Boyce’s sign). A spasm of coughing may occur on palpation due to spilling of the contents into the larynx. A barium swallow will confirm the dagnosis. Surgical intervetion may be necessary.

Answer 111

C. Metastatic carcinoma The voice change here suggests a primary laryngeal carcinoma which we assume here has metastasized locally and to lymph nodes in the deep cervical chain. The hard nature of the lumps, the fact they are painless and firm suggests cervical metastases which are more common in supraglottic cancer (glottic cancer has a much lower rate of cervical metastases). Cervical lymphadenopathy is common and the size, location, mobility and degree of firmness indicate the degree of progression of laryngeal malignancy. Hoarseness, dysphonia, sore throat, dysphagia, referred otalgia, vocal cord lesions and persistent neck mass/adenopathy for >3 weeks are sentinel signs of laryngeal cancer, which is frequently associated with smoking and alcohol use. An MDT approach is taken with the aim of treatment with organ preservation, with salvage surgical resection offered in advanced stage disease. The rate of organ preservation has significantly improved in the last 30 or so years.

Answer 112

A. Left heart failure There are no expressed signs or symptoms of RVF here such as peripheral oedema, ascites, elevated JVP and hepatomegaly. Nocturia may be a symptom as fluid returns from the legs when the patient lies down flat. RVF leads to a backlog of blood and congestion of the systemic capillaries. LVF, on the other hand, causes congestion in the pulmonary circulation so the symptoms are respiratory. As seen in this patient, there is SOB and there may be the classic cough productive of frothy sputum – a sign of pulmonary oedema. On respiratory examination, pulmonary oedema due to LVF may give audible fine late inspiratory crepitations at the bases. There may also be orthopnoea. This is why you can ask patients in a cardiac history how many pillows they sleep with. PND can also occur as well as ‘cardiac asthma’. Initial investigations should include ECG, CXR, TTE and bloods including BNP levels. First line treatment is with an ACE inhibitor which reduces morbidity and mortality. Salt and fluid restriction is also beneficial. All patients with chronic heart failure will also receive a beta blocker such as carvedilol. Other adjuncts include spironolactone, diuretics, hydralazine and a nitrate, and digoxin. The NYHA classification criteria can be used based on symptoms to describe functional limitations and ranges from Class I to Class IV with symptoms occuring at rest. Many patients are asymptomatic for long periods of time because mild cardiac impairment is balanced by compensation.

Answer 113

B. Aortic dissection The tearing chest pain suggests aortic dissection. There may also be interscapular pain with dissection of the descending aorta. Dissecting aneurysms are either type A, which involves the ascending aorta, or type B. Type A dissections require urgent surgery whereas type B can be managed medically if it is not complicated by end organ ischaemia. BP differential between the 2 arms is a hallmark feature. Pulse differences may also be present in the lower limbs. There may also be the diastolic murmur of AR in proximal dissections. A CT scan is indicated as soon as a diagnosis of aortic dissection is suspected and should be from the chest to the pelvis to see the full extent of the dissecting aneurysm. What you will see is the intimal flap. MRI is more sensitive and specific but is more difficult to obtain acutely. Features of Marfan’s syndrome or Ehlers-Danlos syndrome may be present. Features include tall stature, arachnodactyly, pectus excavatum, hypermobile joints and narrow face. Marfan’s predisposes to both aneurysms and/or dissections, presumably due to weakness of the aortic wall. Type IV Ehlers-Danlos predisposes to both aneurysms and/or dissections Other strong risk factors include hypertension, atherosclerotic aneurysmal disease, bicuspid aortic valve, coarctation, smoking, FH and annulo-aortic ectasia.

Answer 114

G. Pericarditis Symptoms include a sharp and severe chest pain retrosternally which is worse on inspiration and when supine, relieved by sitting forwards. The classical finding on examination is a friction rub which is said to sound like ‘walking on snow’. There may be diffuse ST elevations on ECG, classically saddle-shaped, an effusion on echocardiography and blood results suggesting inflammation. Complications include tamponade and constrictive pericarditis. Prior viral infection is a risk factor with the most common pericardial infection being viral. Bacterial purulent pericarditis also occurs. The inflammation is due either to direct viral attack or immune mediated damage. Other risk factors include male gender, post-MI (both ‘early’ and Dressler’s), post-pericardiotomy syndrome, neoplasm from local tumour invasion, uraemia and autoimmune conditions such as RA and SLE.

Answer 115

J. MI This patient’s chest pain sounds like it is due to an MI. Chest pain is classically severe and heavy in nature (often described as crushing), located centrally with possible radiation to the left arm or jaw and lasts for >20 minutes. SOB due to pulmonary congestion and sweating due to high sympathetic output are also common symptoms. Risk factors incorporate the standard set of cardiovascular risks such as smoking, high BP, DM, obesity and dyslipidaemia. The fact the patient has GTN spray suggests a medical history of angina and cardiovascular risk factors. An ECG is indicated here. STEMI, new LBBB or confirmed posterior MI is an indication for PCI/thrombolysis. It is worth noting that RV infarction is present in 40% of inferior infarcts so in this case, right sided ECG leads should also be obtained.

Answer 116

I. Dressler's syndrome This is percarditis again, likely to be Dressler’s syndrome. This is believed to be an autoimmune process with myocardial neo-antigens implicated in the aetiology and occurs typically 2-3 weeks post-MI. Typical treatment is with aspirin. It tends to subside in a few days and raised ESR is a lab finding which can be seen. See above for more information on angina.

Answer 117

B. Aortic regurgitation Aortic regurgitation is the leakage of blood back into the LV in diastole. The collapsing pulse is also known as a water hammer or Corrigan’s pulse and describes the rapid rise and quick ‘collapse’ of the arterial pulse resulting in a wide pulse pressure. The murmur in AR is early diastolic in mild cases and increases to pansystolic in severe cases. Risk factors include a bicuspid valve, rheumatic fever, endocarditis, anklylosing spondylitis and Marfan’s. Other commonly seen signs in EMQs, although uncommon in clinical practice include Traube’s (pistol shot sounds over the femoral arteries when compressed), Quincke’s (subungal capillary pulsations), Duroziez’s (systolic and diastolic murmurs over the femoral arteries) and de Musset’s (head bobbing with each heart beat) sign. Other equally uncommon signs (there are many for AR) include Muller’s, Mayen’s, Lighthouse, Hill’s, Becker’s, Landolfi’s, Rosenbach’s, Gerhardt’s, Lincoln’s and Sherman’s sign. The visible carotid pulsation here is Corrigan’s sign, with the pulse characteristic as described previously. Occasionally although uncommon, an Austin Flint murmur may be heard which is a rumbling mid-diastolic murmur best heard at the apex, produced by the regurgitant jet hitting the LV endocardium. Its presence indicates severe AR and the absence of a loud S1 or an opening snap distinguishes this from the murmur of mitral stenosis.

Answer 118

H. Infective endocarditis Any patient presenting with fever and a new murmur should always make you think of bacterial endocarditis. The weakness here may be consistent with a stroke and systemic emboli may be the cause here. The classic new or worsening murmur is actually rare. As are splinter haemorrhages, which EMQs often mention. Other uncommon signs you may find include Janeway lesions (painless macular haemorrhagic plaques on the palms and soles) and Osler nodes (painful nodules on the pads of the fingers and toes). Roth spots may also be seen on fundoscopy. Three sets of blood cultures are required and this patient will have to go for an echocardiogram. The Duke criteria is used for diagnosis.

Answer 119

A. Aortic stenosis Aortic stenosis can present with chest pain, dyspnoea and syncope. It is characterised by a harsh ejection systolic murmur heard loudest at the right upper sternal edge at end expiration, which radiates up towards the carotids. The pulse pressure is narrow and there may be an associated slow-rising and plateau pulse. Doppler echo is vital for diagnosis and shows a pressure gradient across the narrowed valve orifice. 20% of cases are due to a congenital bicuspid valve. The most common cause of aortic stenosis in adults is calcification of normal trileaflet valves. Clinically stable patients may be considered for surgical repair or TAVR.

Answer 120

C. Innocent murmur This is a functional murmur which is not caused by a structural cardiac defect. They often reflect hyperdynamic states such as that seen in pregnancy. Functional murmurs tend to be systolic, occuring in an otherwise healthy individual with no symptoms. They are also characteristically position dependent and soft in nature. Ones that occur in children tend to disappear as the child grows. Benign paediatric murmurs include Still’s murmur.

Answer 121

F. Mitral regurgitation MR is loudest at the apex and radiates to the axilla and tends to be around grade 4. It is associated with a systolic thrill at the apex. TTE is the investigation of choice for diagnosis. Chronic MR is associated with a laterally displaced apex beat with LV dilatation. Mitral valve prolapse is a strong risk factor for development of MR. The alcohol history here is hinting at dilated cardiomyopathy as a possible cause.

Answer 122

I. Subarachnoid haemorrhage SAH (bleeding into the subarachnoid space) presents with sudden severe headache patients will often describe as the worst headache of their life, and can often be so bad that they feel like they’ve been kicked in the back of the back. Half of all patients lose consciousness and eye pain with exposure to light can also be seen. Altered mental status is common. SAH occurs most commonly in the 50-55 age group and affects women and black people more than men and white people. The most common cause of non-traumatic SAH is an aneurysm which ruptures. Conditions which predispose to aneurysm formation and SAH include adult PKD, Marfan’s, NF1 and Ehlers-Danlos. Cerebral aneurysms arise around the circle of Willis. A CT scan is indicated, and if unrevealing, this should be followed by an LP. Cerebral angiography can confirm the presence of aneurysms. The patient should be stabilised and this followed by surgical clipping or endovascular coil embolisation, the choice is subject to much current controversy sparked by relatively recent research. Complications can commonly occur and include rebleeding, hydrocephalus and vasospasm.

Answer 123

H. Meningitis This patient has meningitis. Universities are common sites of outbreaks due to crowding. Commonly there will be a headache, fever and nuchal rigidity. There may also be an altered mental status (this patient has LOC), confusion, photophobia and vomiting. Kernig’s sign is uncommon but is positive when attempts to extend the leg are met with resistance when the patient is supine with the thigh flexed to 90 degrees. Another uncommon sign is Brudzinski’s sign and the petechial/purpuric rash seen here, typically associated with meningococcal meningitis. CT head should be considered before LP if there is any evidence of raised ICP. An LP will confirm the diagnosis with bacterial meningitis showing a low CSF glucose, elevated CSF protein and positive CSF culture/gram stain or meningococcal antigen. Investigations should not delay treatment in this very urgent situation and antibiotics need to be promptly administered. IM benzylpenicillin can be given on the way to the hospital with empirical antibiotic therapy being started on arrival before the causative organism is identified. This can be, for example, with vancomycin and ceftriaxone (or meropenem, cefotaxime, chloramphenicol). Dexamethasone should also be given before the first dose of antibiotics and continued for 4 days as this has been shown to improve outcome and overall mortality in community-acquired bacterial meningitis (though NICE guidelines state it should not be given to those aged <3 months).

Answer 124

B. Normal pressure hydrocephalus This is normal pressure hydrocephalus – a diagnosis you should suspect in any patient who presents with a gait apraxia (the falls here from loss of balance) and cognitive impairment. There may also be urinary symptoms such as urgency, frequency or urge incontinence. These symptoms tend to be insidious in onset over months or years.There is not significantly raised CSF pressure though perhaps this should not be named ‘normal pressure’ as elevated CSF pressure may be seen but just not significantly so. CT head is the first test to order, although MRI head can also be done, and could be normal but could show mild to moderate ventricular enlargement, periventricular leukomalacia, cerebral infarction, relative preservation of the cortical gyri and sulci and reduced diameter of the corpus callosum and widened callosal angle. This diagnosis can be excluded if an obstructive lesion is seen. If you suspect Parkinson’s then a levodopa challenge should be ordered.

Answer 125

D. Wernicke's encephalopathy Wernicke’s is due to acute thiamine deficiency, which is a problem in alcoholics. Others at risk include those with AIDS, cancer and treatment with chemotherapy, malnutrition and GIT surgery, especially bariatric procedures. It is a clinically under-diagnosed condition. The classic EMQ triad is of mental change, ophthalmoplegia and gait dysfunction, which is actually only seen in 10% of cases. In reality, the manifestations are varied and a high index of suspicion is needed. Despite there, the manifestiations typically include altered consciousness, gait disorders and eye movement abnormalities. This is an emergency and treatment is with parenteral replacement of thiamine. This avoids permanent neurological damage including later development of Korsakoff’s psychosis, which is irreversible. Note that thiamine should be given before dextrose! Magnesium deficiency also needs to be corrected as it is a co-factor in the functioning of thiamine dependent enzymes.

Answer 126

K. Hepatic failure This patient has decompensated chronic liver disease (he is in liver failure) which has resulted in neurological symptoms associated with hepatic encephalopathy. The brain is exposed to ammonia which bypasses the liver by portosystemic shunting. It is a diagnosis of exclusion and tests will need to be conducted to rule out other potential causes of confusion. The findings of jaundice and fetor hepaticus (liver failure) are signs of liver disease. Think about the other signs you might see like spider naevi and palmar erythema. This patient may also have asterixis which is a coarse flapping tremor. HE is likely caused by a host of factors. This patient’s LFTs will be abnormal and she is likely to have coagulopathy too (PT will be elevated).

Answer 127

K. Myeloma This patient has multiple myeloma. This is characteristed by clonal proliferation of plasma cells in BM and commonly presents with bony pain and symptoms of anaemia. There may also be infections present in 10%. Elevated ESR agrees with this diagnosis. 30% have hypercalcaemia and half have renal impairment, which is associated with a worse prognosis. This explains the elevated urea and creatinine here. The diagnostic test is serum or urine electrophoresis looking for a paraprotein spike of IgG or IgA and light chain urinary excretion (Bence Jones proteins). Bone marrow examination and skeletal survey will also need to be conducted. Bone marrow analysis will help differentiate this from MGUS and solitary plasmacytoma. Bone changes include osteopenia, osteolytic lesions and fractures which this person’s XR indicates with the punched out lytic lesions. Younger patients may be candidates for high-dose chemotherapy and autologous transplantation.

Answer 128

F. Aplastic anaemia This is aplastic anaemia characterised here with the pancytopenia (which is common, but diagnosis requires 2 cytopenias out of 3) and the presentation with infections (neutropenia), fatigue (anaemia) and bruising (thrombocytopenia). Risk factors include paroxysmal noctural haemoglobinuria, hepatitis and NSAIDs. If macrocytosis is seen, this may suggest an inherited syndrome such as Fanconi’s anaemia. The reticulocyte count here rules out haemolytic anaemia. The definitive diagnosis is made on biopsy of bone marrow which shows a hypocellular marrow with no abnormal cell populations and no fibrosis. Which conditions would there be abnormal cell populations or fibrosis on bone marrow biopsy?

Answer 129

G. Chronic myeloid leukaemia This is CML which tends to present in the 30-60 age group. At presentation 1/3 may be asymptomatic though if symptomatic, it presents with symptoms including fever, weight loss and night sweats. There is myeloid stem cell proliferation and presents with raised neutrophils, metamyelocytes and basophils. The patient may also describe LUQ discomfort or fullness due to the feeling of a mass due to splenomegaly. There are also symptoms of anaemia here due to BM infiltration of leukaemic cells. Bruises are common and are either spontaneous or from minor trauma. All patients have raised WCC. CML is associated with the philadelphia chromosome characterised by t(9;22) of bcr-abl. There tends to be massive splenomegaly which is the most common physical finding on examination. This conditon may transform to AML or ALL in what is known as a ‘blast crisis’. CML responds to imatinib, which is an anti-bcr-abl antibody and gives long term remission in most patients.

Answer 130

A. Sickle cell anaemia The reticulocytosis here is as a result of the haemolytic anaemia this patient is suffering due to sickle cell anaemia. The bone pain in the chest and hip is an acute symptom of vaso-occlusive crises. It is generally unusual for someone to reach this age without being aware that they have sickle cell anaemia so in real life, this would be an uncommon way to present. The diagnosis should however be suspected due to the episodes of pain (vaso-occlusive crises) and the findings of anaemia with reticulocytosis. Sickling occurs when RBCs containing HbS become distorted into a crescent shape. Patients with sickle cell anaemia have no HbA at all. If both parents carry the sickle cell gene, there is a 1 in 4 chance of giving birth to a child with sickle cell anaemia.

Answer 131

D. Iron deficiency anaemia IDA is a microcytic hypochromic anaemia characterised by low serum iron, high TIBC and low transferrin saturation and serum ferritin. Bleeding is the principle cause of IDA. IDA is not an end diagnosis and has many causes and this is something that is important to bear in mind at all times. The cause here is from chronic blood loss from what would appear to be possible right sided bowel cancer. Worldwide, the most common cause is hookworm infection, and in the UK, menstrual losses in women. Causes of IDA can be broadly divided into 4 categories: decreased intake, increased loss, increased requirements (such as in pregnancy), and unknown.

Answer 132

E. Legionella pneumoniae Legionella is a gram negative rod. Legionella infecting the lungs is legionnaires’ disease or Legionella pneumonia whereas non-lung infection is known as Pontiac fever. This bacteria is found in aqueous environments such as lakes and almost all cases are from contaminated water systems, which relates to the risk factors of getting Legionella (recent water exposure like a hot tub). Smoking is also a risk factor. It can cause confusion as well as hyponatraemia, abdominal pain, diarrhoea and bradycardia. Legionella does not grow on routine culture media and diagnosis relies on urine antigen detection, serology or culture on special media.

Answer 133

K. Pseudomonas aeruginosa Pseudomonas has virulence factors for lung colonisation and is noted for its type III injection apparatus, which you may remember from your microbiology lectures. This organism is one of the main 5 organisms responsible for hospital acquired (or nosocomial) infections in the UK. Remember that a nosocomial infection is defined as one acquired >48 hours after admission. It is a gram negative bacilli causing hospital acquired pneumonia and UTI. It particularly affects immunocompromised hosts such as those on chemotherapy, those with cystic fibrosis, burns and wounds. Biofilms are antibiotic resistant. Key risks also include urinary catheters and central venous lines etc... I would not worry too much about MacConkey agar as lots of things grow on it. It is a culture medium designed to grow gram negative bacteria, so organisms ranging from E. coli, Klebsiella and Salmonella, to Proteus, Shigella and Enterobacter will all grow on it. This just rules out Streptococcus pneumoniae. Pseudomonas is also a special little organism which is not sensitive to many antibiotics originally used for gram negatives. You need to prescribe a drug here which has specific anti-pseudomonal activity such as ceftazidime (a third generation cephalosporin), tazocin, ciprofloxacin, imipenem or gentamicin (usually used with one of the others). This organism acquires resistance genes very quickly so two antibiotics are given. It is, as mentioned, inherently resistant to many drug classes. Some are even resistant to all antibiotics.

Answer 134

F. Streptococcus pyogenes The ‘strawberry’ tongue, or a red swollen tongue, is a sign of Scarlet fever (along with Kawasaki disease and toxic shock syndrome which is caused by bacteria such as staphylococcus aureus). Scarlet fever is caused by an exotoxin released by Streptococcus pyogenes. The history is characteristically a child <10 years old, usually in the autumn, winter seasons, maybe early spring, presenting with sore throat, fever, malaise and GI upset. Examination may reveal a fever, pharyngeal redness with possible exudate, a generalised sandpaper-like erythematous rash, linear petechial streaks (pastia lines) in skin folds and tender cervical lymphadenopathy. Of course, also, the red swollen tongue. You can request a pharyngeal swab but the diagnosis is generally clinical. You can also expect ASO titres, if done, to be positive.

Answer 135

A. Leptospira interrogans Leptospirosis is a zoonosis, which is transmitted by contact with urine of infected animals (also possible sources include blood and fluids). The history may reveal someone swimming in rat infested canal water, for instance, or in this case, a triathlon – which involves some swimming in perhaps not too clean water. Outbreaks of this are associated with flooding and natural disasters, as can be expected. Affected patients can present with an extensive spectrum of clinical manifestations ranging from subclinical illness in 90% to renal and hepatic failure and pulmonary haemorrhage. The important factor in diagnosis is a high index of suspicion based on epidemiological exposure. There is an acute phase with fever, headaches, myalgia and then an immune phase with additional pulmonary symptoms and potential organ damage (leading to the symptoms seen here such as jaundice). The rash is maculopapular and non-pruritic, lasting 1 or 2 days, present during the acute phase. It is rarely seen. Treatment is with benzylpenicillin or amoxicillion and/or doxycycline and supportive care. Those with severe disease carry a poor prognosis.

Answer 136

J. Camplyobacter jejuni This is what we colloquially refer to as ‘food poisoning’ – infective gastroenteritis. The most common bacterial cause in the UK is Campylobactor jejuni. This organism is also a major cause of traveller’s diarrhoea. The second most common cause of acute enteritis is an organism known as Campylobacter coli. There is also Campylobacter fetus which is a rare cause of extra-intestinal infection mainly in immunocompromised patients. Campylobacters are small, gram negative rods with a curved spiral shape. 11 Campylobacters are pathogenic to humans. Most Campylobacter jejuni infections come from infected poultry. The diarrhoea is usually self limiting and resolves in a week – bacteraemia is very rare. Fluid and electrolyte replacement is essential. Only a small number of patients will benefit from antimicrobials. Note that C. jejuni is a commonly identified organism in the aetiology of Guillain-Barre syndrome.

Answer 137

B. MEN II MEN (Multiple Endocrine Neoplasia) syndromes are hereditary autosomal dominant tumour syndromes with distinct patterns of organ involvement. At this stage, the purely simplified patterns (classification is actually more complicated) you need to be familiar with are: MEN1 consists of parathyroid adenomas, pancreatic tumours and pituitary adenomas. MEN2A consists of parathyroid, medullary thyroid cancer and phaeochromocytoma. And MEN2B or 3 consists of what is seen in 2A plus the addition of a marfanoid phenotype and ganglioneuromas (intestinal and visceral). Prophylactic thyroidectomy in childhood is indicated in MEN2. Medical management is aimed at controlling hormone hypersecretion. Surgery is often done on tumours. If you’re really interest, you can look MEN syndromes up in more detail. MEN1 is typically caused by mutations in the MEN1 gene whereas MEN2 is typically caused by mutations in the RET proto-oncogene.

Answer 138

J. Nelson's syndrome Nelson’s syndrome is the enlargement of a pituitary adenoma which occurs after bilateral adrenalectomy. Once you know this fact, the diagnosis is clear. Bilateral adrenalectomy is an operation which can be done for Cushing’s syndrome in order to completely eliminate the production of cortisol. However, this removes cortisol’s negative feedback response which allows any pre-existing pituitary adenoma to grow without negative feedback. As a result, this rapid enlargement of the pituitary adenoma has caused this man’s symptoms of increased pigmentation due to raised MSH (a by product of POMC cleavage to give ACTH), headaches and visual disturbances (due to the space-occupying lesion). This is now rare as the operation is now only used in extreme cases. Sometimes pituitary sugery will be performed.

Answer 139

G. Sheehan's syndrome Sheehan’s syndrome is panhypopituitarism which occurs due to necrosis of the pituitary gland following post-partum haemorrhage and hypovolaemia. Blood loss leads to a vasoconstrictor spasm of the hypophysial arteries leading to ischaemia and necrosis of the pituitary. This is a rare complication of pregnancy. Symptoms include those seen and are due to deficiency of a host of hormones. Treatment is aimed at replacing the missing hormones (hydrocortisone, thyroxine etc).

Answer 140

H. Pseudo-Cushing's syndrome Pseudo-Cushing’s syndrome is where a patient has all of the signs and symptoms and even abnormal hormone levels seen in Cushing’s syndrome however there is no problem to be found with the hypothalamo-pituitary-adrenal axis. It is hence idiopathic. Whole body CT here is normal so rules out causes from organs such as the lungs (ectopic ACTH), adrenals and MRI has also ruled out a pituitary cause.

Answer 141

E. Kallmann's syndrome Kallmann’s syndrome (hypogonadotrophic hypogonadism) is a cause of primary amenorrhoea. The history tends to be of delayed development of secondary sexual characterisitcs with anosmia. There is either a missing olfactory bulb or one which is not fully developed (this may be seen on MRI) so there may be a lack of a sense of smell or a sense of smell which is severely reduced (hyposmia seen here). It may be diagnostically challenging as it is difficult to distinguish pathological developmental delay from constitutional delay. Normal but pre-pubertal external and internal genitalia are seen, and normal final adult height if treated. You would expect serum FSH to be low (though FSH assays have very very very wide ranges) and LH if done. Low oestradiol can also be expected.

Answer 142

H. Guillan-Barre syndrome This previous history of influenza-like illness weeks before the onset of neurological symptoms indicates Guillain-Barre syndrome which is a demyelinating polyneuropathy. Classic neurology is a progressive symmetrical muscle weakness affecting lower extremities before upper extremities, and proximal muscles before distal muscles, accompanied by paraesthesias in the hands and feet which often precedes onset of weakness. The paralysis is typically flaccid with areflexia and progresses acutely over days, with an ensuing plateau phase followed by recovery. Two thirds of patients have a history of either prior influenza-like illness or gastroenteritis. Weak risks include immunisation, cancer and lymphoma, older age, HIV infection and male gender. Up to 30% will develop respiratory muscle weakness requiring ventilation so spirometry should be carried out at 6 hour intervals initially (and may show reduced vital capacity). AST and ALT may be elevated though the cause is unclear. LP is useful and the classic finding is of elevated CSF protein with normal cell count (known as albuminocytological dissociation). Treatment is with supportive and disease modifying treatment (plasma exchange or high dose Ig). GBS is classified by symptoms and variants exist such as Miller-Fisher syndrome, which occasionally crop up in EMQ books but will probably never crop up in clinical practice.

Answer 143

I. Milroy's syndrome Milroy’s is primary lymphoedema with a familial autosomal dominant pattern of inheritance. It often presents in infancy. There is unfortunately no cure and treatment involves compression, with surgery reserved for those refractive to conservative measures and/or with major morbidity.

Answer 144

B. Tietze's syndrome This is costochondritis, or Tietze’s syndrome (which describes constochondritis accompanied by chest wall swelling), which presents with insidious onset of anterior chest wall pain which is made worse by certain movements of the chest and deep inspiration. The key sign here is that there is pain when palpating the costochondral joints, particularly the 2nd to the 5th and the diagnosis is clinical. Tests are done to exclude other diagnoses here such as breast pathology. First line treatment is with NSAIDs. Oral NSAIDs are preferred in a primary care setting and a beneficial response confirms the diagnosis. If NSAIDs or local corticosteroid injection (usually performed by a specialist) fail to make the symptoms better than you should seek further investigations and consider a wider differential diagnosis which include conditions like pleuritis, ACS, PE, rib fracture and GORD.

Answer 145

E. Felty's syndrome Felty’s syndrome is a rare extra-articular manifestation of rhematoid arthritis characterised by persistent and idiopathic neutropenia and in some cases splenomegaly. It occurs in <1% of RA patients, often in the 5th to 7th decade of life, typically in white patients with long-standing seropositive and erosive disease. Treatment aims at trying to raise the neutrophil count and prevent recurrent infections, while controlling systemic RA. The cause of the neutropenia is multifactorial and due to an imbalance between production in BM and descruction in peripheral blood. Other causes of neutropenia need to be ruled out first so investigations like peripheral blood film are needed.

Answer 146

A. Osler-Weber-Rendu syndrome This man has the facial telangiectasia of OWR, also called hereditary haemorrhagic telangiectasia. This causes abnormal blod vessels pretty much everywhere which are prone to bleed. It is an autosomal dominant condition so a positive FH can often be found.

Answer 147

H. Migraine Migraine is a chronic condition, with genetic determinants, which usually presents in early to mid life. The typical migraine aura this patient describes (which can be visual, sensory or speech symptoms) which can occur during or before the headache, is pathognomic, but is not seen in the majority of patients. The aura can be positive phenomena (for example a patient seeing flashing lights) or negative phenomena (for example visual loss). Nausea, photophobia and disability (the headache gets in the way with the patient’s ability to function) accompanying a headache also suggest a migraine diagnosis. The headache of a migraine tends to be prolonged if untreated, and tends to be unilateral and pounding (but does not have to be). The OCP may be a trigger here. Tests aim to rule out other differentials, although if the history is compatible and neurological examination is unremarkable, further testing is not needed.

Answer 148

E. Subarachnoid haemorrhage SAH (bleeding into the subarachnoid space) presents with sudden severe headache patients will often describe as the worst headache of their life, and can often be so bad that they feel like they’ve been kicked in the back of the back. Half of all patients lose consciousness and eye pain with exposure to light can also be seen. Altered mental status is common. SAH occurs most commonly in the 50-55 age group and affects women and black people more than men and white people. The most common cause of non-traumatic SAH is an aneurysm which ruptures. Conditions which predispose to aneurysm formation and SAH include adult PKD, Marfan’s, NF1 and Ehlers-Danlos. Cerebral aneurysms arise around the circle of Willis. The family history of kidney disease here points to adult PKD. A CT scan is indicated, and if unrevealing, this should be followed by an LP. Cerebral angiography can confirm the presence of aneurysms. The patient should be stabilised and this followed by surgical clipping or endovascular coil embolisation, the choice is subject to much current controversy sparked by relatively recent research. Complications can commonly occur and include rebleeding, hydrocephalus and vasospasm.

Answer 149

C. Space-occupying lesion Raised ICP classically presents with symptoms which are worse in the morning. The headache can either awake the patient from sleep or is present on waking up and decreases after being awake for several hours. It is also made worse on exertion/Valsalva such as the coughing and bending over here. This patient will require a CT head to try to find the cause which could be a space-occupying lesion. This is also the cause of the papilloedema seen on fundoscopy. Raised ICP can also manifest with a CNVI palsy, which is a false localising sign.

Answer 150

A. Trigeminal neuralgia Trigeminal neuralgia occurs as episodes of severe unilateral pain in the distribution of CNV, usually lasting seconds, with no pain occuring between these episodes. Examination is often unremarkable. The pain is described as sharp, intense, stabbing or burning. It can be triggered commonly by actions such as eating, tooth brushing, cold and touch. Shaving and eating seem to be common in EMQs. Most people are asymptomatic between attacks although the severity of the pain makes these patients live in constant fear. TN is more common in MS and incidence increases with age. Post-herpetic TN is also possible. The mainstay of treatment is medical, with antiepileptics such as carbamazepine (which is the only medicine which is proven in RCTs and is therefore typically first line). If medical treatment fails, surgical options do exist such as microvascular decompression.

Answer 151

I. Cluster headache Cluster headache is characterised by attacks of severe pain localised to the unilateral orbital, supraorbital and/or temporal areas which lasts from 15 minutes to 3 hours, and occurs with a frequency ranging from once every other day to 8 times a day. These attacks can occur at the same time period of many weeks (known as the cluster period) accompanied by ipsilateral autonomic signs. The cause is hypothalamic activation with secondary trigeminal and autonomic activation (for instance, lacrimation, rhinorrhoea, nasal congestion, conjunctival injection and partial Horner’s i.e. ptosis and miosis). Cluster period attacks can be triggered by things like alcohol. Greater occipital nerve blockade often provides immediate relief until preventative medications take effect.

Answer 152

G. Pyoderma gangrenosum This is pyoderma gangrenosum, which presents with multiple lesions, most commonly affecting the lower extremity and is linked to UC although it can be seen less commonly in Crohn’s, and is also seen in conditions such as RA and the myeloid dyscrasias. These lesions start as tender papules or vesicles which develop into painful ulcers with a dusky purple edge and surrounding induration and erythema. The base may contain granulation tissue and lesions heal with atrophic scars.

Answer 153

B. Venous ulcer Venous ulcers occur on a background of deep venous insufficiency. There is oedema and a brown skin discolouration due to leaching of pigments and haemosiderin deposition. In addition there may be lipodermatosclerosis and an inflammatory response, which is seen as an eczema-like thickening and hardening of the skin. The skin can also be drawn tightly around the ankle. Ulceration usually follows trauma and is usually on the medial gaiter region. The base has granulation tissue and is sloughy in nature and there is a sloping edge to the ulcer. The shape is often irregular. Look up some photos to help you remember. Once significant arterial disease is excluded (ulcers can have mixed components), the mainstay of treatment is with compression bandaging, appropriate dressings and treatment of any infection with antibiotics. Maggots can also be used and varicose veins should be treated where possible to reduce recurrence. If the ulcer is not healing, a biopsy should be considered (Marjolin’s ulcer).

Answer 154

E. Sickle cell anaemia Adults with sickle cell anaemia may present with leg ulcers although it has to be said that it is very unusual for a person with sickle cell disease to reach adulthood without being aware of their diagnosis. The adult patient could present with unexplained haemolysis, possible intermittent episodes of pain due to vaso-occlusive crises, avascular necrosis and retinal haemorrhage too. The doppler derived ankle-brachial pressure index is normal here which points away from peripheral arterial disease, which in this relatively young person is unlikely in any case. The typical constellation of findings of venous ulcers are not seen (oedema, lipodermatosclerosis). In real medicine, as it is rare for sickle cell anaemia to present this late, I would consider other possibilities like pyoderma gangrenosum (which is arguably more likely in this case, given the location of the ulcer and age of the patient, so if this was your answer then you can give yourself a ‘real life medicine’ mark), but this is an EMQ (key point!) and the fact this lady is from the sub-Saharan Africa is a key fact. From an epidemiological perspective, the prevalence is 10-30% in sub-Saharan Africa. Between 25 and 30% of newborns in western Africa are carriers of sickle cell trait. This patient here will need to have their peripheral blood film reviewed following by haemoglobin electrophoresis and HPLC. Sickle solubility is a rapid test which can also be done but will not differentiate sickle trait from sickle disease. At the end of the day, remember that to do well in an EMQ, you need to think like an EMQ.

Answer 155

I. Squamous cell carcinoma SCC is a malignant tumour of keratinocytes arising in the epidermis of the skin. Risk factors include UV exposure, older age, immunosuppression, fair skin, HPV and exposure to ionising radiation. The skin changes here are characteristic. Most frequently however, SCC often presents with multiple actinic keratoses, with a change in the lesion such as bleeding, induration, change in size and shape or inflammation. Aks and SCCs lie on a spectrum so it may be difficult to clinically differentiate the two. Skin biopsy will confirm the diagnosis. This can either be a shave biopsy, punch biopsy, incisional biopsy or excisional biopsy depending on the size and location.

Answer 156

D. Arterial ulcer Arterial ulcers are deep and painful with a well defined edge, usually found on the shin or foot. They often have a pale base and a punched out appearance. The low ankle brachial pressure index is indicative too. There may be local changes such as cold peripheries, loss of hair, dusky cyanosis and toenail dystrophy. The surrounding skin is often white and shiny. It is typically most painful in bed and the pain is sometimes relieved by having the legs dependent. On examination, peripheral pulses may be absent or reduced. An angiogram with contrast will define the lesion and determine whether it can be improved by surgical intervention. Pain often increases when your legs are at rest and elevated. They can occur between the webs of toes so it is important to always check these in your peripheral vascular examination.

Answer 157

D. Porphyria There are many types of porphyria. This patient has acute intermittent porphyria, which is characterised by symptoms like the ones this patient describes – abdominal pain, peripheral motor neuropathy, mental symptoms like confusion. These symptoms, certainly in EMQs, can be trigged by the use of certain drugs which are known to provoke AIP attacks. In reality, the list of drugs is pretty vast and include most CYP450 inducers, but in EMQs, alcohol and the OCP are common. It is worth noting that alcohol also induces an enzyme called delta-aminolevulinic acid synthase, which can exacerbate AIP. It is probably not worth learning the pathways unless you plan on sitting USMLE. AIP is a genetic disorder where there is a partial deficiency of PBGD (the third enzyme in the haem biosynthetic pathway). Treatment of acute attacks involves IV haem arginate with adjunctive dextrose IV. The pain is thought to be neuropathic in origin. The patient may complain of red/browny urine due to increased urinary excretion of intermediates in the haem pathway.

Answer 158

E. Beri-beri Beriberi is vitamin B1 (thaimine) deficiency. Deficiency is the cause of several clinical syndromes including wet and dry beriberi and Wenicke’s encephalopathy. The presentation depends on the chronicity of B1 deficiency. Dry beriberi is a distal peripheral polyneuropathy characterised by parasthesia, reduced knee jerks and other tendon reflexes, and progressive severe weakness with muscle wasting secondary to chronic deficiency. Wet beriberi, which this person also has is secondary to either acute or chronic deficiency and is characterised by high-output heart failure with peripheral vasodilation, peripheral oedema and orthopnoea or low-output heart failure with lactic acidosis and peripheral cyanosis. The latter is also referred to as Shosin beriberi. Remember that Wernicke’s is an acute neuropsychiatric syndrome which classically presents with the triad of acute confusion, ataxia and ocular abnormalities (such as nystagmus and strabismus) secondary to acute B1 deficiency.

Answer 159

C. Diabetic amyotrophy Diabetic amyotrophy, more common in T2DM, is an uncommon peripheral diabetic neuropathic complaint. It presents with severe muscle weakness and pain with proximal thigh muscle atrophy. Weak knee flexion and quadriceps wasting is typical of diabetic amyotrophy.

Answer 160

A. HIV HIV is a retrovirus and there are two types, HIV 1 which is the main virus responsible and HIV 2 which is restricted to parts of West Africa. Strong risk factors include needle sharing with IVDU, unprotected receptive intercourse, needle stick injury and high maternal viral load (mother to child). Kaposi’s sarcoma may present as a pink or violaceous patch on the skin or in the mouth and it is an AIDS-defining condition. Peripheral neuropathy is common and may be related either to HIV or some other medicine or toxin (some HAART can cause PN). There are WHO (stage 1-4) and CDC criteria used in clinical staging. This patient needs to have a CD4 count, HBV and HCV screen, VDRL (syphilis), tuberculin skin test (TB) and CXR. HIV viral load will also be assessed. Prophylaxis and immunisations should be considered against infections such as hepatitis, influenza, PCP and TB. When to initiate HAART depends on the clinical stage, CD4 and co-morbidities. This patient will need to be started on HAART if he has not already. Classes of antiretrovirals include NRTIs, NNRTIs, protease inhibitors, fusion inhibitors and integrase inhibitors.

Answer 161

H. Vitamin B12 deficiency This is a macrocytic anaemia with elevated MCV. The classical peripheral blood smear would show megalocytes (RBC precursors whose numbers increase) and hypersegmented polymorphonuclear cells although the film may be normal in early deficiency. You would also want to order a serum B12 level in this patient and would expect the corrected reticulocyte index to be low due to decreased production (opposed to high in haemolytic anaemia due to increased production). This peripheral sensory neuropathy would likely be a loss of proprioception and vibration indicating degeneration of the dorsal column-medial lemniscus pathway. The patient may also have a positive Romberg as a result. Parasthesias are also common in B12 deficiency indicating peripheral neuropathy. Pallor and signs of frank anaemia are late signs. It is important to remember that hepatic B12 stores last for many years so B12 deficiency depends on chronic deficiency over a long period of time. In general, deficiency is caused by decreased intake, decreased gastric breakdown from food or malabsorption from the GIT. Serum B12 is a useful investigation to initially conduct and will guide further investigations and treatment. You should consider an underlying malabsorptive process such as coeliac or Crohn’s as a possible cause.

Answer 162

C. Neurogenic shock This is a thoracolumbar spine fracture. Neurogenic shock is not to be confused with spinal shock which is not circulatory in nature ( and is characterised by hypotonia or flaccidity that resolves within 24 hours). Neurogenic shock is a form of distributive shock due to spine or braintem injury and there is resulting failure of vasoregulation. As a result there is a fall in systemic vascular resistance with vasodilation, leading to low BP as blood pools in the extremeties where sympathetic tone is low. This is occasionally associated with bradycardia which is due to autonomic disruption.

Answer 163

A. Urinary retention This is clearly urinary retention. Urine output is gradually deteriorating in a step-wise manner and he is now anuric

Answer 164

F. Pulmonary oedema This patient has been fluid overloaded and has pulmonary oedema, causing her dyspnoea. She will require diuretics and fluid restriction to deal with his overloaded state. The findings of bibasal fine creptitions, heard at the end of expiration, is characteristic. Infiltrates may also be seen on CXR. Type 1 respiratory failure is hypoxic respiratory failure, which occurs when PaO2 is low. Type 2 respiratory failure is known also as hypercapnic respiratory failure and occurs when there is hypoxia associated with a high PaCO2. As a general note for your exams. Common causes of type 1 respiratory failure include pulmonary oedema, pneumonia and PE. Common causes of type 2 include COPD and respiratory muscle weakness.

Answer 165

B. Haemorrhagic shock This is a history of a ruptured AAA. He has key risk factors of obesity, smoking and T2DM. There is abdominal pain radiating to the back here and the low BP and compensatory tachycardia is due to the blood loss. This patient is in haemorrhagic shock. As this AAA has ruptured, this man will need urgent surgical repair, with of course standard resuscitation measures. The airway will needed to be managed with supplemental oxygen and ET intubation, a central venous catheter will need to be inserted, an arterial catheter and urinary catheter will also be needed for monitoring, and the target systolic BP is 50-70. Infusing too many gluids may increase the risk of death. The most effective form of surgical repair is an EVAR (endovascular AAA repair), anatomy permitting, otherwise traditional open repair is performed. Open repair has a mortality of 48%. Antibiotics will also be needed to cover bacteria to prevent graft infection. This will be prescribed in line with local protocols.

Answer 166

E. Percutaneous jejunostomy This patient needs nutritional support to prevent malnutrition and starvation. Enteral nutrition here is not possible as a chunk of the oesophagus has just been removed and we have to wait for an intact anastomosis. During surgery, a percutaneous jejunostomy can be placed to provide a temporary route of nutrition until oral feeding can resume. A surgeon at operation cannot place a percutaneous gastrostomy, and it is more suitable for prolonged feeding. Furthermore, in the immediate post-operative period, there may be gastric stasis so it is preferred to deliver the feed via a post-pyloric placement. If you have seen a PEG tube (percutaneous gastrostomy), you will know why it is not preferred. It is not a pleasant sight (and I don’t mean cosmetically – just have a look at the needle). Some younger people may ask for it to be converted to a button gastrostomy for cosmetic reasons. A PEG tube is placed laparoscopically. A percutaneous jejunostomy is an alternative to parenteral (IV) nutrition in this post-operative patient. Parenteral nutrition is really a last resort and this is not an indication for it. Only when the GIT is either unavailable or function is indequate should you consider it. This can be delivered via a venflon, PICC line or centrally. There are a host of complications, both nutritionally, related to the catheter e.g. infection, thrombosis and the effect on organ systems e.g. biliary disease. Note that you should also be aware of the phenomenon known as refeeding syndrome.

Answer 167

C. IgA nephropathy About 50% of those with IgA nephropathy will present with recurrent episodes of macroscopic haematuria after a URTI or gastroenteritis. About a third will have microscopic haematuria and mild proteinuria. Less than 10% present with nephrotic syndrome or acute rapidly progressive GN. Fever and myalgia are systemic findings. The oedema here may indicate nephrotic syndrome. Definitive diagnosis is made on renal biopsy. Light microscopy shows focal or diffuse mesangial proliferation and extracellular expansion, and IF shows diffuse mesangial IgA deposition in a granular pattern. ACE inhibitors are prescribed to reduce proteinuria, particularly in the setting of hypertension but they have not been shown to preserve renal function. Early treatment with corticosteroids has been shown to delay renal decline in those with moderate proteinuria. A urine dipstick would be the first test to order here.

Answer 168

I. Goodpasture's syndrome Goodpasture’s syndrome is defined by autoantibodies to the alpha-3 chain of type IV collagen which leads to progressive renal dysfunction. It is one of the few causes of pulmonary renal syndrome, which is characterised by pulmonary haemorrhage with rapidly progressive GN. Diagnosis is by early renal biopsy and serology. Around 30-50% of patients with anti-GBM disease will have a positive ANCA result, which changes subsequent management of this condition. Positive ANCA usually suggests a diagnosis of Wegener’s granulomatosis, Churg-Strauss or microscopic polyarteritis instead of Goodpasture’s. Note that MPO-ANCA is also known as pANCA and ANCA stands for anti-neutrophil cystoplasmic antibodies. Anti-GBM serology is a confirmatory diagnostic test in addition to renal biopsy and this can also be used to monitor response to treatment and to gauge when to stop plasmapheresis (i.e. when the antibody titre turns negative). Biopsy of the kidneys will show a crescentic GN and characteristic linear IgG staining on immunofluorescence. The renal function tests here are abnormal, which is the hallmark of Goodpasture’s syndrome. If it is diagnosed before renal dysfunction becomes severe then aggressive treatment can lead to an excellent prognosis. Plasma exchange can be used to remove preformed antibodies.

Answer 169

B. Post-streptococcal glomerulonephritis Nephritic syndrome is typically defined by acute kidney injury, hypertension and an active urinary sediment (RBCs and RBC casts). This is post-infectious glomerulonephritis caused by group A beta-haemolytic streptococcus with renal endothelial cell damage. Serological markers would expect to show antibodies to streptococcus and low complement and treatment here is with antibiotics. The high ASOT (antistreptolysin O antibody titres) indicates post-streptococcal GN. There may also be positive anti-Dnase and antihyaluronidase in post-streptococcal GN. As a note, causes of nephritic syndrome (with sub-nephrotic range proteinuria) include: IgA nephropathy, post-infectious GN and rapidly progressive GN. Nephrotic syndrome causes include minimal change disease, focal and segmental glomerulosclerosis, membranous nephropathy, deposition diseases and membranoproliferative GN.

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D. Henoch-Schonlein purpura Henoch-Schonlein purpura is the most common vasculitis in childhood and in all cases there is a rash of palpable purpura which are typically non-blanching. If there is no rash, then it is not HSP. They are normally 2-10mm in diameter and are due to the extravasation of blood into the skin. They can occur anywhere on the body but are usually concentrated on the lower extremities. Half of all patients have abdominal pain and arthralgias are commonly present (found in about 80%) and often associated with oedema. The joints most often affected are the knees and ankles. About half will show signs of renal disease such as proteinuria or haematuria. Risk factors for this condition include being male, age 3-15 and history of prior UTI. Complications can occur and the most common cause of death is renal failure. While serum IgA levels may be elevated, this is not a specific test for HSP.

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H. Retroperitoneal fibrosis Retroperitoneal fibrosis is a rare disorder where there is proliferation of fibrous tissue in the retroperitoneum which here has caused the ureters to become obstructed. This has led to oliguria, back pain and the palpable kidneys. The cause could be an effect of antihypertensives, or due to malignancy or some other cause. A CT scan is the test to order here and treatment depends on severity and can be either surgical or medical, the latter generally with glucocorticoids followed by DMARDs.

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H. Trousseau's sign Trousseau’s sign is carpal spasm when a blood pressure cuff is used for several minutes. Carpopedal spasm that occurs with hypocalcaemia is a painful spasm and could be the presenting sign. Chvostek’s sign is twitching of the perioral muscles in response to tapping over the facial nerve at the ear. If urgent replacement is necessary, calcium gluconate can be given IV. It is preferred over calcium chloride as it causes less tissue necrosis if it leaks out. It is worth noting that digoxin may be ineffective until serum calcium is restored to normal.

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E. Racoon eyes Basilar skull fractures have specific clinical features. Blood pooling from these fractures can cause periorbital bruising (raccoon eyes), brusing over the mastoid area (Battle’s sign) and bloody otorrhoea. There may also be CSF leak resulting in CSF otorrhoea or rhinorrhoea. A unilateral raccoon eye has an 85% positive predictive value for this diagnosis.

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D. Grey-Turner's sign Complicated haemorrhagic pancreatitis may exhibit Cullen’s sign, Grey-Turner’s sign and Fox’s sign. Grey-Turner’s sign refers to bruising of the flanks and can take 24-48 hours to occur. It is due to retroperitoneal haemorrhage.

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G. Murphy's sign A positive Murphy’s sign may commonly be seen in cholecystitis. This is where palpation of the right subcostal region reveals tenderness. During deep inspiration, the tenderness suddenly becomes worse and there is inspiratory arrest as a result. This sign can also be elicited during ultrasound examination when pressure is applied using the ultrasound probe.

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F. Quincke's sign Quincke’s sign is an uncommonly seen sign where there is subungal or lip capillary pulsations caused by the large stroke volume seen in AR. This is a peripheral sign associated with a bounding pulse and the systolic hypertension of chronic severe aortic regurgitation.

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Other specific antidotes to be aware of (check out TOXBASE if interested): • Calcium channel blocker OD can be treated with calcium chloride. Glucagon can be tried if calcium replacement alone is insufficient. • Sodium channel blocker toxicity can be treated with sodium bicarbonate depending on the QRS length. • Salicylate or phenobarbital OD can be treated with urine alkalinisation with IV sodium bicarbonate. Phenobarbital responds well to multidose charcoal. • Cholinesterase toxicity can be treated with IV atropine if there is symptomatic bradycardia. Severe cases can benefit also from IV pralidoxime. • Cyanide toxicity can be treated with hydroxocobalamin. • Warfarin toxicity can be treated with FFP/PCC and vitamin K. • Digoxin toxicity is treated with digoxin-specific antibody fragments (digoxin immune Fab). • Sulphonylurea OD can be treated with glucose infusions in combination with IV octreotide if hypoglycaemia is problematic. • Heavy metal toxicity can be treated with the appropriate chelating agent.

Blackboard Mock Exam 2018 Flashcards

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