Case-based MCQ

⏳ Case-based MCQ | #Case_329 | #answer 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 ✅ A There is an association between type 1 diabetes and coeliac disease. The gold standard to diagnose coeliac disease is a jejunal/duodenal biopsy. In the past, small-bowel biopsies for diagnosis of coeliac disease were taken from the jejunum, but nowadays most gastroenterologists take endoscopic biopsies from the distal duodenum. Coeliac disease is caused by sensitivity to the protein gluten. Repeated exposure leads to villous atrophy which in turn causes malabsorption. 🟣Signs and symptoms • Chronic or intermittent diarrhoea • Stinking stools/steatorrhoea • Persistent or unexplained gastrointestinal symptoms including bloating, nauseaand vomiting • Fatigue • Recurrent abdominal pain, cramping or distension • Sudden or unexpected weight loss • Unexplained iron, vitamin B12 or folate deficiency. Note that the one of the most common presentation of coeliac disease is iron deficiency anaemia. Also,folate deficiency is more common than vitamin B12 deficiency in coeliac disease Complications • osteoporosis • T-cell lymphoma of small intestine (rare) 🟣Investigation Diagnosis is made by a combination of immunology and jejunal biopsy. Any test for coeliac disease is accurate only if a gluten-containing diet is eaten during the diagnostic process. The person should not start a gluten-free diet until diagnosis is confirmed. NICE issued guidelines on the investigation of coeliac disease in 2009. If patients arealready taking a gluten-free diet they should be asked, if possible, to reintroduce glutenfor at least 6 weeks prior to testing. 🟣Specific auto-antibodies • Tissue transglutaminase (TTG) antibodies (IgA) are first-choice according to NICE • Endomysial antibody (IgA) 🟣Jejunal/duodenal biopsy A biopsy is still needed to diagnose coeliac disease even if antibody test confirm the diagnosis of coeliac disease. • Villous atrophy • Crypt hyperplasia • Increase in intraepithelial lymphocytes 🟣Management • Gluten-free diet

⏳ Case-based MCQ | #Case_329 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 A 28 year old type 1 diabetic has intermittent diarrhoea and abdominal bloating over the last 6 months. He also complains of feeling tired all the time. His blood results show the following: Haemoglobin 135 g/L (130-180 g/L) Ferritin 30 ng/ml (20-300 ng/ml) Thyroid stimulating hormone (TSH) 2.5 mU/L (0.5-5.7 mU/L) Immunoglobulin A (IgA) tissue transglutaminase positive What is the SINGLE most appropriate next step in action? A. Duodenal biopsy B. Colonoscopy C. Sweat test D. Thyroid function test E. Advice patients to go on a gluten-free diet

⏳ Case-based MCQ | #Case_328 | #answer 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 ✅ C The patient described in the vignette has chronic granulomatous disease (CGD). Although the presentation of CGD is variable, the diagnosis should be suspected in patients with recurrent lymphadenitis, multiple-site osteomyelitis, cutaneous abscesses (confused with “acne”), hepatic abscesses, a family history of recurrent infections, or unusual infections with catalasepositive organisms such as Staphylococcus aureus or Aspergillus. In many of these cases, the patient is asymptomatic or with much fewer signs and symptoms (e.g., lack of fever) than would otherwise be expected given the infectious burden. The disease is caused by mutations in genes encoding proteins involved in generating a respiratory burst. One of these, phox91, is on the X chromosome, providing an X-linked pattern of inheritance as demonstrated by the family history. ⚠ Answer A is incorrect. Ataxia-telangiectasia is a T-lymphocyte disorder that manifests as oculocutaneous telangiectasias and progressive cerebellar ataxia. It is caused by a DNA repair defect resulting from a mutation in the ATM gene encoding a protein critical for repair of DNA breaks. Ataxia generally begins when the child learns to walk, and the telangiectasias generally become apparent between 3 and 6 years of age. ⚠ Answer B is incorrect. Chédiak-Higashi syndrome includes neutropenia, neuropathy, and oculocutaneous albinism. It is inherited in an autosomal-recessive manner. The disorder is caused by a defect in neutrophil chemotaxis resulting from mutations in the LYST gene required for sorting of lysosomal granule contents. Patients have albinism due to abnormal melanosomes and inappropriate delivery of melanin in the skin. ⚠ Answer D is incorrect. Hyper IgE syndrome (HIES) is characterized by recurrent bacterial infection and cavitating pneumonia. It is typically associated with impaired inflammation giving rise to so-called “cold abscesses.” Although abscesses are found in HIES, granulomas are generally not. Furthermore, HIES occurs in an autosomal dominant or autosomal recessive pattern of inheritance and not in the X-linked pattern suggested by the case history. ⚠ Answer E is incorrect. Wiskott-Aldrich syndrome is an X-linked disorder with B- and T-lymphocyte dysfunction. Affected patients typically present with eczema and thrombocytopenia. Although patients have abnormalities of their skin, these are heralded by petechiae (from the thrombocytopenia) and eczema. The disease results from mutations of the Wiskott-Aldrich syndrome protein (WASp) gene on the X chromosome. WASp is important for rearranging the actin cytoskeleton in immune cells and thus enables responsiveness.

⏳ Case-based MCQ | #Case_328 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 A 2-year-old boy is brought to the clinic for evaluation of “acne.” His mother describes a recent onset of pain in his left foot and in his right hand. She says he has been hospitalized once for lymphadenitis, but he has been otherwise healthy. His review of systems is otherwise negative. He as two maternal uncles who died in childhood from pneumonia. The child is afebrile, with a heart rate of 64/min, blood pressure of 108/70 mm Hg, and respiratory rate of 15/min. Physical examination reveals multiple scarred lesions on his skin, two of which are 1 cm in diameter and filled with purulent material. Pain is elicited by palpation of the purulent lesions, and they are somewhat warm to the touch. From which immunodeficiency disorder is this patient most likely suffering? (A) Ataxia-telangiectasia (B) Chédiak-Higashi syndrome (C) Chronic granulomatous disease (D) Hyper IgE syndrome (E) Wiskott-Aldrich syndrome

⏳ Case-based MCQ | #Case_327 | #answer 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 ✅ D This patient has developed a posttransplant lymphoproliferative disorder (PTLD). Any patient who is chronically immunosuppressed is at risk for development of lymphoproliferative malignancy, and a cardiac transplant patient is at intermediate risk (4.6% incidence) compared to kidney (1.0%) or liver (3.0%) transplant patients. PTLDs generally occur in extranodal locations, and may respond completely to reduction in immunosuppression; however, since immunosuppression is necessary for protection of grafted organs, radiotherapy is also often employed. In addition, most PTLDs are related to primary or reactivation infection with Epstein Barr virus. Some evidence demonstrates that the use of acyclovir during treatment for PTLD contributes to eradication of the virus and resolution of the malignancy caused by it. The majority of centers thus employ acyclovir as an adjuvant therapy to radiotherapy/surgery coupled with a reduction in immunosuppression. ⚠ Answer A is incorrect. The purpose of acyclovir in treatment of posttransplant lymphoproliferative disorder is to eliminate the Epstein-Barr virus that is causative in the development of the malignancy, not to eradicate the neoplastic cells themselves; this is left to the host immune system and radiation therapy, if employed. ⚠ Answer B is incorrect. Although severe infection with cytomegalovirus is a risk for any immunosuppressed patient, antiviral therapy is generally not employed in patients undergoing cancer therapy. Acyclovir prophylaxis is employed in some bone marrow transplant centers. ⚠ Answer C is incorrect. Radiation to mucous membranes will indeed cause inflammation and decreased barrier protection. This is generally not held to be an indication for antiviral prophylaxis, however. ⚠ Answer E is incorrect. If anything, decreasing the patient’s immunosuppressive regimen will assist her immune system in preventing viral infection

⏳ Case-based MCQ | #Case_327 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 A 32-year-old woman develops a progressive idiopathic cardiomyopathy and eventually undergoes a cardiac transplant. She is placed on a rigorous protocol for immunosuppression. Two years later she develops right-sided sinus fullness, and imaging reveals a mass filling the right paranasal sinus. She is treated with a reduction in immunosuppression and acyclovir. She achieves a full recovery. Why is acyclovir part of her therapeutic regimen? (A) Acyclovir has also been shown to have antineoplastic properties (B) Acyclovir is necessary for prophylaxis against cytomegalovirus in any immunosuppressed patient (C) Acyclovir will reduce the risk of infection due to radiation of the mucous membranes and subsequent breakdown (D) Eradication of Epstein-Barr virus may contribute to resolution of the paranasal sinus mass (E) Since the patient’s immunosuppressive regimen must be decreased, protection against viruses that might infect the patient’s new heart is particularly important

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⏳ Case-based MCQ | #Case_326 | #answer 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 ✅ C Oedema and fluid overload in third spaces, such as ascites, are managed with diuretics. The diuretic most useful in cirrhosis is spironolactone. This is because cirrhotics have intravascular volume depletion, which results in a high aldosterone state. Spironolactone is an aldosterone antagonist. Common indications of spironolactone - Ascites: patients with cirrhosis develop a secondary hyperaldosteronism. - Hypertension: used as one of the last treatment options (in a 'step 4' treatment) - Heart failure - Nephrotic syndrome

⏳ Case-based MCQ | #Case_326 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 A 35 year olf man with a history of alcohol abuse has oedema and ascites demonstrated by shifting dullness. Spider naevi is noted on his trunk. Paracentesis shows clear fluid. He has a temperature of 37.2 C, a pulse of 85 beats/minute, a blood pressure of 119/85 mmHg and a respiratory rate of 20 breaths/minute. What is the SINGLE most appropriate medication to start? A. Corticosteroid B. Azthioprine C. Spironolactone D. Cholestyramine E. Penicillamine

⏳ Case-based MCQ | #Case_325 | #answer 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 ✅ E The question here is written to test your understanding of the pain ladder. As the pain is not well controlled, we should move up on the pain ladder. The pain ladder consist of 3 steps: 1: Simple analgesia like paracetamol. Aspirin, NSAIDs +/- adjuvant therapy 2: Weak opiates: e.g codeine, tramadol, dihydrocodeine +/- adjuvant therapy 3. Strong opiates: morphine, fentanyl patches, diamorphine, oxycodone +/- adjuvant therapy. If patient is still in pain, you should never go back a step on the pain ladder, only go forward. Dihydrocodeine is similr to codeine in its analgesic effect and would not benefit the patient. There is no point in the patient taking 2 different types of opioids. If a patient is started on a strong opioid, the weaker opioids should be stopped. Oral oxycodone is twice the potency of oral morphine. It would not be suitable unless the patient has tried morphine for a few days first and has intolerable side effects like constipation

⏳ Case-based MCQ | #Case_325 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 A 52 year old man has a history of prostate cancer and bone metastasis. He has been taking regular codeine orally for his back pain for the past year. For the past three l months, he finds the back pain worsening. He often wakes up in the middle of the night due to the back pain. What is the SINGLE most appropriate management? A. Replace codeine with oral dihydrocodeine B. Add on buscopan to his regimen C.Replace codeine with oral oxycodone D.Add on oral morphine to his regimen E.Replace codeine with oral morphine

⏳ Case-based MCQ | #Case_324 | #answer 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 ✅ E Mikulicz’s syndrome is the benign persistent swelling of lacrimal and parotid (or submandibular) glands due to lymphocytic infiltration. When no specific cause is found it is called Mikulicz’s disease; and if secondary to disease like sarcoidosis or tuberculosis, it is termed as Mikulicz’s syndrome. In this case, where it is likely secondary to sarcoidosis given the patient’s chest X-ray findings it is termed Mikulicz’s syndrome. Mikulicz disease and syndrome has the same clinical picture and it comprises of a triad of: ▪Symmetrical enlargement of all salivary glands, ▪Narrowing of the palpebral fissures due to enlargement of the lacrimal glands, ▪Dryness of the mouth

⏳ Case-based MCQ | #Case_324 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 A 33 year old woman comes in with a 6 month history of painless bilateral swelling of the face and a mild grade fever. The swelling has been progressively increasing in size. She also complains of having worsening symptoms of dry mouth. On a routine chest X-ray, she is found to have bilateral perihilar lymphadenopathy. What is the SINGLE most likely diagnosis? A. Chronic sialadenitis B. Carcinoma of salivary gland C. Lofgren syndrome D. Adenoid cystic carcinoma E. Mikulicz’s syndrome

⏳ Case-based MCQ | #Case_323 | #answer 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 ✅ A Legionella pneumophila is the causative organism that causes Legionnaires' disease which is a severe, potentially fatal acute pneumonia acquired by droplet inhalation of water contaminated. L. pneumophila is found in natural water supplies and soil. It is also found in many recirculation and water supply systems. For the purpose of this exam, look out for hints like traveling, hotel stays, whirlpool spas, hot tubs as often they would put one of these hints in the question, if the examiners would like you to select Legionella pneumophila as the answer.

⏳ Case-based MCQ | #Case_323 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 A 33 year old man has mild headache and myalgia for 2 days followed by high fever, chills, rigors and a cough. His cough was initially dry but progressed to be productive. He has just returned from a conference in Greece where he mentions that he swam and used the hot tubs in the hotel. He has a temperature oef 38.1 C and is seen to be dyspnoeic. Chest X-ray shows patchy alveolar infiltrates. What is the SINGLE most likely organism which would have caused his symptoms? A. Legionella pneumophila B. Mycoplasma pneumoniae C. Staphylococcus aureus D. Streptococcus pneumoniae E. Klebsiella pneumoniae

⏳ Case-based MCQ | #Case_322 | #answer 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 ✅ C This is a diagnosis of spontaneous pneumothorax. Key clues for PLAB 1: young male playing a sport develops sudden shortness of breath. There usually would be clues “decreased breath sounds on one side”. Sometimes they may say a “tall” man or an “athlete”, as these would be the common presenters of spontaneous pneumothorax. Primary spontaneous pneumothoraces occur most commonly in tall thin men aged between 20 and 40. Cigarette is a major risk factor for pneumothorax. The mechanism is unclear; a smokinginduced influx of inflammatory cells may both break down elastic lung fibres (causing bulla formation) and cause small airways obstruction (increasing alveolar pressure and the likelihood of interstitial air leak) Chest X-ray is the diagnostic test in most cases, revealing a visible lung edge and absent lung markings peripherally. If patient is cyanosed, dyspneic, underlying lung disease – perform arterial blood gas.

⏳ Case-based MCQ | #Case_322 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 A 15 year old boy presents to the Emergency Department with a sudden onset of chest pain and increasing shortness of breath during a beach volleyball game. He has a medical history of asthma and is on a beta-2 agonist inhaler. On examination, there is no cyanosis but there are reduced breath sounds on the left side. Which of the following is the SINGLE most appropriate investigation? A. D-dimer B. CT chest C. Chest x-ray D. Peak flow meter E. Spirometry

⏳ Case-based MCQ | #Case_321 | #answer 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 ✅ C This is a diagnosis of intussusception. Note the PLAB 1 clues: the infant is between 5-12 months, child has been crying persistently (indication of abdominal pain), drawing the legs up to chest, currant jelly blood in stool, and sausage-shaped mass. This along with pyloric stenosis and malrotation with volvulus is a common paediatric surgical question. Know the differences between each. Intussusception Presentation: TRIAD of: 🔺 Abdominal pain 🔺 Currant jelly blood in stool 🔺 Sausage-shaped mass on palpation (often in the right upper quadrant) - Child is crying persistently - Drawing up of legs - May be vomiting if severe Diagnosis: - Abdominal ultrasound → may show doughnut or target sign - Bowel enema Treatment: - Air enema reduction or laparotomy Remember: “Red currant jelly” stools is pathognomonic for intussusception

⏳ Case-based MCQ | #Case_321 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 A 10 month old male infant presents with a 6 hour history of crying and passage of loose, bloody stool. On examination, the infant is irritable, with intermittent drawing up of his knees to his chest, and a temperature of 38.8 C. A currant jelly-coloured blood is seen in his stools. What is the SINGLE most likely diagnosis? A. Constipation B. Gastroenteritis C. Intussusception D. Meckel’s diverticulum E. Volvulus