A 7 year-old male comes to your clinic with a large blister on his left foot. They come and disappear after a few days. Occasionally, he also develops swollen lips, swollen tongue, colicky abdominal pain with these blisters. Blisters are not itchy but slightly painful. These episodes come every few months and last for 1-2 days. Patient reports that one of his uncles has a similar disorder. Laboratory tests revealed low C4 complement level and low C1 esterase inhibitor level. What is the most likely diagnosis in this patient?
Of the following, which is shown to be effective in the treatment of ACE-inhibitor induced angioedema?
An 87 year-old female was brought to the hospital with sudden confusion, trouble speaking, trouble walking, dizziness, loss of balance and severe headache for the last 3 hours. She was diagnosed with acute ischemic stroke and was started on tissue plasminogen activator (tPA) IV infusion. After a few minutes, the patient started to develop a swollen face, swollen tongue, swollen lips, low blood pressure, a weak, rapid pulse and difficulty breathing. Suspecting anaphylaxis, you administered epinephrine, antihistamines and steroids. However, she was not responding to these measures and her distress only worsened with time. Ninety minutes later, you checked her serum tryptase level, which is normal. What is the next best action in her management?
A 48 yo female came to your urgent care clinic complaining of stress, swollen face, swollen lips, hoarseness of voice, some difficulty breathing, episodes of abdominal pain, and watery diarrhea for the last one week. Her current medications include lisinopril for hypertension and paroxetine for anxiety and obsessive compulsive disorder. She has been taking lisinopril 10 mg daily for over 10 years and paroxetine 20 mg daily for over 6 years. Her vital signs were Temperature 97.7 F, Respiratory rate 19/minute, Pulse 75/minute, BP 118/78 mm Hg. Her physical examination is significant for swollen lips, swollen face and tenderness in all quadrants of abdomen with palpation. Suspecting angioedema, you ordered serum C4 level, which gave a result of 4 mg/dl (normal 12 – 38 mg/dl). Her symptoms subsided over the next few days. Going forward, how would you manage her high blood pressure?
Of the following, which is the best cost-effective screening test to diagnose hereditary angioedema in a patient with symptoms and signs suggestive of hereditary angioedema?
A 22 year-old white female came to your office for the evaluation of greenish vaginal discharge. She also reports burning while urinating, an urge to urinate more frequently, sharp pain in the lower abdomen and bloody spotting between periods. After examining her and running laboratory tests, you diagnosed her with Neisseria gonorrhoeae infection. As you discuss the treatment plan for this condition, the patient frustratingly informs you that this is the fifth attack of gonorrhea in the last 8 months. After further laboratory tests, you diagnosed her with complement deficiency. A patient is diagnosed with complement deficiency. She is planning a trip to Greece in four weeks and would like to take your advice about travel vaccinations. Of the following live vaccines, which is contraindicated in this patient?
A patient showed normal resistance to encapsulated bacteria and increased susceptibility to Neisseria gonorrhea and Neisseria meningitidis. Deficiency in which of the following pathways of the complement system explains his condition?
A 30 year-old female came to your office for a tuberculin skin test for a foster child physical examination. Before she can take care of a foster child in her home, she should get a physical examination and a tuberculin skin test. She reports that she got a BCG vaccine when she was a child. She has a complicated medical history significant for HIV positivity five years ago. She also reports she got some discomfort with a PPD test 7 years ago. How do you administer a TB skin test to this patient?
A 55 year-old white male and his wife arrived in your office. He does not like to see doctors and the last time he went to a doctor’s office was when he was a baby. This time his wife insisted that he must see a doctor because his symptoms have only been getting worse for the past year. He reports he has had slight fever, fatigue, loss of appetite, weight loss, nose bleedings, sinus pains, painful gums, hoarseness of voice, repeated ‘ear infections’, ‘all my joints hurt’ joint pains, cough, bloody sputum, chest pains, ‘urine looks foamy and bloody’. He remembered having had a few tick bites 4 months ago while taking a hike in the nearby mountain. His wife insists on getting a Lyme test and prescribing doxycycline immediately. On physical examination, you noticed perforation of the nasal septum, collapse of the nasal bridge, swollen gums, right-sided facial palsy, tender, warm, swollen joints and palpable purpura on both upper and lower extremities. Suspecting a serious systemic disorder involving joints, you consulted a rheumatologist and together you ordered different laboratory and imaging tests for this patient. A chest computed tomography (CT) scan showed a cavitary lesion measuring 40 mm x 80 mm in the left lower lung lobe, subglottic tracheal stenosis and diffuse alveolar hemorrhage. Rheumatoid Factor……..Positive White blood cell count…..12,600/mm3 Erythrocyte sedimentation rate…..60 mm/hr Sputum cultures for Mycobacterium tuberculosis…..negative Anti-neutrophil cytoplasmic antibody (c-ANCA), Myeloperoxidase antibody……18 AU/ml and PR-3 antibody 28 AU/ml Lung biopsy showed non-caseating granulomatous inflammation and vasculitis Borrelia burgdorferi antibody enzyme immunoassay………0.7 Kidney biopsy: Necrotizing granuloma, vasculitis, multinucleated giant cells, palisading histiocytes, clusters of neutrophils within the blood vessel wall, coalescence of neutrophilic microabscesses, relatively sparse immunoglobulin and complement depositions, segmental necrotizing glomerulonephritis with multiple crescents Urinalysis…….Glucose – negative Blood – positive Protein – positive ECG…. showed diffuse ST-segment elevation in most leads On treatment, his symptoms responded well to two infusions of rituximab. What is the most likely diagnosis?