A 3 year-old male is brought to your office for the evaluation of recurrent infections and persistent hunger, and not gaining weight. His mother reports that his medical history is significant for recurrent skin abscesses with staphylococcus aureus. Recently he started to complain about abdominal pain that starts one to two hours after a meal. When he vomits, the ingested food is ejected forcefully falling several feet away. Physical examination findings include several abscesses over both upper extremities, tenderness over the epigastric area.      You ordered an ultrasound of his abdomen which confirmed what you suspected, pyloric stenosis. Then you thought, “but why is this boy getting recurrent skin abscesses?” Incrementally, you ordered some laboratory tests to investigate this boy’s condition. Results came as follows CBC: anemia, leucocytosis, and elevated ESR  Skin abscess culture: Positive for Staphylococcus aureus, Burkholderia cepacia and Serratia marcescens  Dihydrorhodamine (DHR) test: Positive  Nitroblue tetrazolium test: Positive  You suspect chronic granulomatous disease in this patient. In most patients, this disorder is inherited as 

A 3 year-old male is brought to your office for the evaluation of recurrent infections and persistent hunger, and not gaining weight. His mother reports that his medical history is significant for recurrent skin abscesses with staphylococcus aureus. Recently he started to complain about abdominal pain that starts one to two hours after a meal. When he vomits, the ingested food is ejected forcefully falling several feet away. Physical examination findings include several abscesses over both upper extremities, tenderness over the epigastric area.      You ordered an ultrasound of his abdomen which confirmed what you suspected, pyloric stenosis. Then you thought, “but why is this boy getting recurrent skin abscesses?” Incrementally, you ordered some laboratory tests to investigate this boy’s condition. Results came as follows CBC: anemia, leucocytosis, and elevated ESR  Skin abscess culture: Positive for Staphylococcus aureus, Burkholderia cepacia and Serratia marcescens  Dihydrorhodamine (DHR) test: Positive  Nitroblue tetrazolium test: Positive  Which of the following best describes the pathogenesis of the disease affecting this patient?

A 3 year-old male is brought to your office for the evaluation of recurrent infections and persistent hunger, and not gaining weight. His mother reports that his medical history is significant for recurrent skin abscesses with staphylococcus aureus. Recently he started to complain about abdominal pain that starts one to two hours after a meal. When he vomits, the ingested food is ejected forcefully falling several feet away. Physical examination findings include several abscesses over both upper extremities, tenderness over the epigastric area.      You ordered an ultrasound of his abdomen which confirmed what you suspected, pyloric stenosis. Then you thought, “but why is this boy getting recurrent skin abscesses?” Incrementally, you ordered some laboratory tests to investigate this boy’s condition. Results came as follows CBC: anemia, leucocytosis, and elevated ESR  Skin abscess culture: Positive for Staphylococcus aureus, Burkholderia cepacia and Serratia marcescens  Dihydrorhodamine (DHR) test: Positive  Nitroblue tetrazolium test: Positive  You suspected a granulomatous disease in this boy. Which of the following is a granulomatous disease? 

A 10 year-old boy is brought to your office because his mother is concerned about his frequent bouts of sinusitis, pneumonia and skin infections. He has beautiful blue eyes but is unusually sensitive to bright light. Mother reports that there are some relatives with blue eyes and albinism in her extended family. Physical examination is significant for silvery hair,  albinism, hepatosplenomegaly, and ecchymosis. Laboratory tests show neutropenia, thrombocytopenia, elevated bleeding time and hypergammaglobulinemia. Pathology report says there are peroxidase-positive giant granules in the neutrophils  on peripheral smear. What is the most likely diagnosis in this patient? 

A 10 year-old boy is brought to your office because his mother is concerned about his frequent bouts of sinusitis, pneumonia and skin infections. He has beautiful blue eyes but is unusually sensitive to bright light. Mother reports that there are some relatives with blue eyes and albinism in her extended family. Physical examination is significant for silvery hair,  albinism, hepatosplenomegaly, and ecchymosis. Laboratory tests show neutropenia, thrombocytopenia, elevated bleeding time and hypergammaglobulinemia. Pathology report says there are peroxidase-positive giant granules in the neutrophils  on peripheral smear. You suspected Chédiak-Higashi syndrome in this boy. Of the following, which leukocyte morphology is characteristic of Chediak-Higashi syndrome? 

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? 

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?