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?
Of the following, which will play the final role in the perforation of bacterial cell membranes in the complement cascade?
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?
Which of the following is the most common autoimmune disorder in patients with C1, C4, or C2 deficiency?
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?
A 4 month old male is brought to your office by his parents for a wellness check. They are concerned about his repeated bouts of eczema and recurrent infections. He has a complicated medical history significant for bloody diarrhea and hard to control epistaxis during the second month of his life. Your physical examination found a feeble baby with eczema, dry blood in the perinatal region and splenomegaly. You ordered some routine labs for this patient. Later that evening a technician from the laboratory called you to inform you of some ‘red flag’ lab results, which are a platelet count of 60000/ platelets/µL with reduced sized platelets in clumps. Concerned about the possibility of Wiskott Aldrich syndrome (WAS), you ordered a genetic test and it revealed a genetic defect on the short arm of the X-chromosome. In WAS patients, what is the most common finding at the time of diagnosis?
A 4 month old male is brought to your office by his parents for a wellness check. They are concerned about his repeated bouts of eczema and recurrent infections. He has a complicated medical history significant for bloody diarrhea and hard to control epistaxis during the second month of his life. Your physical examination found a feeble baby with eczema, dry blood in the perinatal region and splenomegaly. You ordered some routine labs for this patient. Later that evening a technician from the laboratory called you to inform you of some ‘red flag’ lab results, which are a platelet count of 60000/ platelets/µL with reduced sized platelets in clumps. Concerned about the possibility of Wiskott Aldrich syndrome (WAS), you ordered a genetic test and it revealed a genetic defect on the short arm of the X-chromosome. What is the most frequent cause of death in WAS?
A 4 month old male is brought to your office by his parents for a wellness check. They are concerned about his repeated bouts of eczema and recurrent infections. He has a complicated medical history significant for bloody diarrhea and hard to control epistaxis during the second month of his life. Your physical examination found a feeble baby with eczema, dry blood in the perinatal region and splenomegaly. You ordered some routine labs for this patient. Later that evening a technician from the laboratory called you to inform you of some ‘red flag’ lab results, which are a platelet count of 60000/ platelets/µL with reduced sized platelets in clumps. Concerned about the possibility of an immunodeficiency disorder you ordered a genetic test, which revealed a genetic defect on the short arm of the X-chromosome. You called the parents to explain the lab results in the light of your clinical findings. How do you explain the pathogenesis of this disease to his parents?
