Case-based MCQ

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Primary biliary cholangitis (PBC; previously referred to as primary biliary cirrhosis) is a chronic inflammatory disease that leads to fibrous obliteration of intrahepatic bile ducts. The disease is much more common in females with a female to male ratio of 9:1 and a median age of onset around 50 years. Up to 25% of patients are usually diagnosed during routine blood evaluation. Ursodiol is the major medication that is used to slow down the progression of PBC. The earlier treatment is initiated, the most effective it is. Serum bilirubin (choice A) has been shown to be the best prognostic factor. Indeed, bilirubin enters in all the mathematical models that have been developed. When serum bilirubin is constantly above 34.2, 102.6 and 171 µmol/L, mean survival is 4.1, 2.1 and 1.4 years, respectively. Also, elevated alkaline phosphatase levels are associated with worse outcomes. 🔖 Key point: Serum bilirubin and alkaline phosphatase have shown to be good prognostic factors in patients with primary biliary cholangitis.

⏳ Case-based MCQ | #Case_460 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 A 43-year-old woman with unremarkable medical history and normal physical examination had elevated levels of ALP and GGT. The diagnosis of primary biliary cholangitis (PBC) was established and the patient was treated appropriately. However, five years later, she started complaining of fatigue and generalized pruritus. Her liver was 3 cm below the costal margin.

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Diabetic ketoacidosis (DKA) is one of the life-threatening acute complications of diabetes mellitus. In addition to hyperglycemia and ketonemia, DKA is associated with severe electrolyte disturbances and dehydration. Plasma anion gap (PAG) is the recommended test for monitoring of diabetic ketoacidosis (choice D). PAG estimates the concentration of all unmeasured anions contributing to acidosis in DKA. Although acetoacetate and beta-hydroxybutyrate are the anions responsible for the high PAG metabolic acidosis in this patient, contribution from other acids cannot be excluded. One such anion is lactate. If for any reason the patient develops hypoxia or ischemia, production of lactic acid and thus, of lactate will increase. Anion gap would thus, increase even if the production of ketoacids is curtailed by insulin therapy. ⚠ Blood pH (choice A) may be totally normal in some mixed acid-base disturbances. For example, if our patient develops hyperventilation due to severe pain and PaCO2 drops to 17mmHg, his pH would be 7.39 (normal range 7.35-7.45). Thus pH cannot be used to evaluate response to acid-base disturbances to treatment. ⚠ Normal serum bicarbonate (choice B) does not guarantee normal acid-base status. If our patient develops severe vomiting, an element of metabolic alkalosis will develop, and bicarbonate might rise to the normal range despite the persistence of metabolic acidosis. PAG will still remain high. Measurement of plasma bicarbonate level is thus, not suitable for the assessment of response to acidosis to treatment. ⚠ Serum ketone level (choice C), especially of beta-hydroxybutyrate, might be a good indicator for assessment of pure ketoacidosis. However, as outlined above, if other acids contribute to the acidosis, they will be missed if only ketone body level is used to monitor response to treatment. ⚠ For many reasons, pH of urine (choice E) is not suitable for monitoring the response to acidosis to treatment. Urine pH has a lower limit of 4.5, reflecting the maximum gradient against which hydrogen ions can be pumped in the distal part of the nephron. Regardless of the severity of acidosis, pH cannot drop below this lower limit. Additionally, urine pH does not reflect the total hydrogen ion carrying capacity of urine. Especially in acidosis, a large amount of hydrogen ion is carried in urine as ammonium (NH4). Third, urinary pH might also change in response to local factors in the kidney or urinary tract like infection. 🔖 Key point: Plasma anion gap is the recommended test for monitoring of diabetic ketoacidosis.

⏳ Case-based MCQ | #Case_460 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 A 20-year-old female was brought to the ED with confusion. The patient was not oriented in time, place, and person and had Glasgow Coma Score of 14. Pulse rate was weak and regular at 112 bpm, respiration was regular and deep at 30 bpm, blood pressure 105/60 mmHg and her body temperature was 36.9°C. The diagnosis of diabetic ketoacidosis was established. Treatment was initiated with the infusion of normal saline and then IV insulin and closely monitored. The patient started showing some improvement. 

Bell's palsy (idiopathic facial paralysis) is an unexplained weakness or paralysis of the facial nerve, the nerve that controls muscle movement on one side of the face. The condition causes drooping on the affected side, and patients may not be able to close the eye and may experience tearing, drooling and hypersensitive hearing. Although Bell's palsy is unsettling and inconvenient, it is typically not indicative of a serious health problem and in most cases completely resolves itself.  The condition can strike at any age, but young and middle-age adults seem to be the most vulnerable. Pregnant women and individuals with diabetes, influenza, a cold, or an upper respiratory infection seem to be at a greater risk. About eight percent of patients report a family history of Bell's palsy, but it's unclear if the disease has a genetic basis. While the exact cause of Bell's palsy is not known, many believe that in most cases it is triggered by an infection of the facial nerve by herpes simplex virus (HSV) (choice E). HSV infection has been discovered in up to seventy percent of patients diagnosed with Bell's palsy. ⚠ Other infectious causes of acute peripheral facial palsy include adenovirus, coxsackievirus, cytomegalovirus, Epstein-Barr virus (choice A), influenza B, mumps, and rubella virus. ⚠ Other diseases including Lyme disease and, rarely, HIV (choice C), may also cause sudden facial paralysis. Varicella-zoster virus, a related herpes virus and the cause of chickenpox and shingles, is another cause.  ⚠ Human papillomavirus (choice D) and Respiratory syncytial virus (choice E) are not associated with facial palsy.

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⏳ Case-based MCQ | #Case_458 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 A 41-year-old man presents to the ED with acute onset of a right-sided facial droop. He cannot close his right eye, and he also cannot move the right side of his face when asked to smile. He does admit that he had an upper respiratory infection recently. His speech is not impaired and he has normal motor and sensory exam of his extremities on both sides. His head CT is also normal.

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⏳ Case-based MCQ | #Case_458 | #answer 〰〰〰〰〰〰〰〰〰〰〰〰〰〰 ✅ B This patient presents with the triad of lower extremity edema, cyanosis, severe pain after 24 hours of experiencing blanching and edema; this is suggestive of the diagnosis of phlegmasia cerulea dolens (PCD), which usually follows phlegmasia alba dolens. This is a rare complication of massive deep vein thrombosis (DVT) that presents suddenly with complete venous occlusion leading to impaired arterial flow. Often the iliofemoral area is affected, and the impaired drainage disorder results in tissue ischemia. This serious condition can lead to venous gangrene of the extremity and compartment syndrome. It is an emergency that requires prompt diagnosis and effective treatment as the mortality rate is between 25% and 40%, while the risk of amputation could be as high as 50%. The most significant risk factors are malignancy, such as breast cancer (choice B), heart failure, heparin-induced thrombocytopenia, surgery, trauma, and third-trimester pregnancy. ⚠ Atherosclerosis (choice A) is the underlying disease in peripheral arterial disease, which could also lead to decreased dorsalis pedis pulse, but this patient's clinical presentation strongly suggests PCD. ⚠ Diabetes mellitus (choice C) is a risk factor for atherosclerosis, which could lead to decreased lower extremity arterial pulses, but it is not significantly associated with PCD. ⚠ Hyperthyroidism (choice D) is not a risk factor for DVT or PCD. ⚠ Thromboangiitis obliterans (choice E), also known as Buerger's disease, is a vasculitis affecting the lower extremities that has been associated with smoking. Pain, cyanosis, gangrene can all occur, making it similar to the PCD. Patients are usually younger than 45 years, and the blanching that preceded the patient's current condition steers us towards phlegmasia. 🔖 Key point: A triad of lower extremity edema, cyanosis, severe pain after 24 hours of experiencing blanching and edema is suggestive of the diagnosis of phlegmasia cerulea dolens, a rare complication of massive deep vein thrombosis that presents suddenly with complete venous occlusion leading to impaired arterial flow.