A 24 year-old male came to the emergency room complaining of fever, headache, nausea, vomiting, blurred vision, photophobia, lethargy, altered mental status and stiff neck. His past medical history is significant for HIV positivity and asthma. Physical examination revealed an irritable and restless patient with fever, altered mental status, stiff neck, nystagmus, papilledema, and cranial nerve deficits. Laboratory tests revealed elevated WBC with lymphocyte predominance, HIV-1 viral load of 302,551 RNA copies/mL and a CD4+ counts <100 cells/mm3. CSF culture came positive for cryptococcus antigen. Patient was diagnosed with cryptococcal meningitis and was started on amphotericin B and fluconazole. He showed good response to the treatment. His symptoms improved rapidly. Realizing that his life-threatening cryptococcal infection was due to his AIDS , he wanted to get started on highly reactive antiretroviral therapy (HAART) . Upon his discharge from the hospital, he was started on HAART. Two weeks passed uneventfully. Then, one evening, he returned to the emergency room complaining of fever, lymphadenitis, myalgia, cough, and chest pain. Physical examination is remarkable for lymphadenopathy, mucocutaneous ulcers in the mouth, bilateral rales on chest auscultation and hepatosplenomegaly. Vital signs: Temp: 100.6F, Oxygen Sat = 94, BP 100/70; Pulse = 97/min; RR = 22/min Some of the laboratory results are as follows: CD4 count is 800 cells/mm3 HIV-1 viral load of 4800 RNA copies/mL CSF positive for cryptococcus antigen Urine assay is positive for histoplasmosis Chest X ray showed apical infiltrates and nodules When the patient was told about his test results, he said he never heard about histoplasmosis and never had a sickness from it. Of the following, which is a complication of IRIS in this patient? 

A 24 year-old male came to the emergency room complaining of fever, headache, nausea, vomiting, blurred vision, photophobia, lethargy, altered mental status and stiff neck. His past medical history is significant for HIV positivity and asthma. Physical examination revealed an irritable and restless patient with fever, altered mental status, stiff neck, nystagmus, papilledema, and cranial nerve deficits. Laboratory tests revealed elevated WBC with lymphocyte predominance, HIV-1 viral load of 302,551 RNA copies/mL and a CD4+ counts <100 cells/mm3. CSF culture came positive for cryptococcus antigen. Patient was diagnosed with cryptococcal meningitis and was started on amphotericin B and fluconazole. He showed good response to the treatment. His symptoms improved rapidly. Realizing that his life-threatening cryptococcal infection was due to his AIDS , he wanted to get started on highly reactive antiretroviral therapy (HAART) . Upon his discharge from the hospital, he was started on HAART. Two weeks passed uneventfully. Then, one evening, he returned to the emergency room complaining of fever, lymphadenitis, myalgia, cough, and chest pain. Physical examination is remarkable for lymphadenopathy, mucocutaneous ulcers in the mouth, bilateral rales on chest auscultation and hepatosplenomegaly. Vital signs: Temp: 100.6F, Oxygen Sat = 94, BP 100/70; Pulse = 97/min; RR = 22/min Some of the laboratory results are as follows: CD4 count is 800 cells/mm3 HIV-1 viral load of 4800 RNA copies/mL CSF positive for cryptococcus antigen Urine assay is positive for histoplasmosis Chest X ray showed apical infiltrates and nodules When the patient was told about his test results, he said he never heard about histoplasmosis and never had a sickness from it. What is the definitive test to diagnose immune reconstitution syndrome in this patient?

A 24 year-old male came to the emergency room complaining of fever, headache, nausea, vomiting, blurred vision, photophobia, lethargy, altered mental status and stiff neck. His past medical history is significant for HIV positivity and asthma. Physical examination revealed an irritable and restless patient with fever, altered mental status, stiff neck, nystagmus, papilledema, and cranial nerve deficits. Laboratory tests revealed elevated WBC with lymphocyte predominance, HIV-1 viral load of 302,551 RNA copies/mL and a CD4+ counts <100 cells/mm3. CSF culture came positive for cryptococcus antigen. Patient was diagnosed with cryptococcal meningitis and was started on amphotericin B and fluconazole. He showed good response to the treatment. His symptoms improved rapidly. Realizing that his life-threatening cryptococcal infection was due to his AIDS , he wanted to get started on highly reactive antiretroviral therapy (HAART) . Upon his discharge from the hospital, he was started on HAART. Two weeks passed uneventfully. Then, one evening, he returned to the emergency room complaining of fever, lymphadenitis, myalgia, cough, and chest pain. Physical examination is remarkable for lymphadenopathy, mucocutaneous ulcers in the mouth, bilateral rales on chest auscultation and hepatosplenomegaly. Vital signs: Temp: 100.6F, Oxygen Sat = 94, BP 100/70; Pulse = 97/min; RR = 22/min Some of the laboratory results are as follows: CD4 count is 800 cells/mm3 HIV-1 viral load of 4800 RNA copies/mL CSF positive for cryptococcus antigen Urine assay is positive for histoplasmosis Chest X ray showed apical infiltrates and nodules When the patient was told about his test results, he said he never heard about histoplasmosis and never had a sickness from it. Of the following, which is the best course of management for this patient?

A 24 year-old male came to the emergency room complaining of fever, headache, nausea, vomiting, blurred vision, photophobia, lethargy, altered mental status and stiff neck. His past medical history is significant for HIV positivity and asthma. Physical examination revealed an irritable and restless patient with fever, altered mental status, stiff neck, nystagmus, papilledema, and cranial nerve deficits. Laboratory tests revealed elevated WBC with lymphocyte predominance, HIV-1 viral load of 302,551 RNA copies/mL and a CD4+ counts <100 cells/mm3. CSF culture came positive for cryptococcus antigen. Patient was diagnosed with cryptococcal meningitis and was started on amphotericin B and fluconazole. He showed good response to the treatment. His symptoms improved rapidly. Realizing that his life-threatening cryptococcal infection was due to his AIDS , he wanted to get started on highly reactive antiretroviral therapy (HAART) . Upon his discharge from the hospital, he was started on HAART. Two weeks passed uneventfully.      Then, one evening, he returned to the emergency room complaining of  fever, lymphadenitis, myalgia, cough, and chest pain. Physical examination is remarkable for lymphadenopathy, mucocutaneous ulcers in the mouth, bilateral rales on chest auscultation and hepatosplenomegaly.  Vital signs: Temp: 100.6F,  Oxygen Sat = 94, BP 100/70; Pulse = 97/min; RR = 22/min  Some of the laboratory results are as follows:  CD4 count is 800 cells/mm3 HIV-1 viral load of 4800 RNA copies/mL CSF positive for cryptococcus antigen  Urine assay is positive for histoplasmosis  Chest X ray showed apical infiltrates and nodules     When the patient was told about his test results, he said he never heard about histoplasmosis and never had a sickness from it. What is the most likely explanation for his emergency visit? 

A mother brought her 6 month-old male infant to your office for the establishment of care. She reports that he had four bouts of pneumonia and ‘countless’ skin infections in the last six months of his life. His past medical history is significant for delayed separation of his umbilicus after birth. You did not find any abnormalities in your physical examination. Her report of ‘countless’ skin infections prompted you to investigate further.You ordered some routine labs which is significant for a  WBC count 30000/microliter. Later, in consultation with a hematologist, you ordered a flow cytometry analysis, which showed absence of functional β2-integrins (CD11/CD18). You wondered why this baby has such a high WBC count. Of the following, which conditions show primary neutrophilia? 

A mother brought her 6 month-old male infant to your office for the establishment of care. She reports that he had four bouts of pneumonia and ‘countless’ skin infections in the last six months of his life. His past medical history is significant for delayed separation of his umbilicus after birth. You did not find any abnormalities in your physical examination. Her report of ‘countless’ skin infections prompted you to investigate further.You ordered some routine labs which is significant for a WBC count 30000/microliter. Later, in consultation with a hematologist, you ordered a flow cytometry analysis, which showed absence of functional β2-integrins (CD11/CD18). You suspected leukocyte adhesion deficiency in this baby. Which of the following is the best treatment for patients with Leukocyte Adhesion Deficiency Type I?

A mother brought her 6 month-old male infant to your office for the establishment of care. She reports that he had four bouts of pneumonia and ‘countless’ skin infections in the last six months of his life. His past medical history is significant for delayed separation of his umbilicus after birth. You did not find any abnormalities in your physical examination. Her report of ‘countless’ skin infections prompted you to investigate further.You ordered some routine labs which is significant for a WBC count 30000/microliter. Later, in consultation with a hematologist, you ordered a flow cytometry analysis, which showed absence of functional β2-integrins (CD11/CD18). You suspected leukocyte adhesion deficiency in this baby. Of the following, which is the best explanation of prognosis in a patient with this disorder?

A 4 year-old male was brought to the emergency room with severe bleeding from nose. He had a complicated medical history significant for delayed umbilical stump detachment,  intracerebral hemorrhage at birth, recurrent infections, misshapen brittle bones with several fractures in the past and poor wound healing with no pus formation. Blood analysis revealed leukocytosis and an autosomal recessive condition with  mutations of FERMT3 gene, which encodes for Kindlin-3 protein. Bleeding stopped after the administration of red blood cell transfusions and recombinant Factor VIIa. What is the most likely diagnosis in this patient? 

A dad brought his 3 year-old male son for a quick evaluation of toothache. His son is complaining of rapidly worsening pain around his teeth and gums for the last 3 days. The boy’s past medical history is significant for recurrent bacterial infections, impaired physical and mental growth. In the physical examination, you noticed a child who looks small for his age with unusual facial features. Laboratory workup showed marked leukocytosis, defective expression of selectin ligands, a rare Bombay (hh) blood type at the ABO locus and the absence  of sialyl Lewis X expression (CD15a). He responded well to antibiotics and oral fucose supplements. Which of the following is the most likely diagnosis in this patient? 

A mother brought her 6 month-old male infant to your office for the establishment of care. She reports that he had four bouts of pneumonia and ‘countless’ skin infections in the last six months of his life. His past medical history is significant for delayed separation of his umbilicus after birth. You did not find any abnormalities in your physical examination. Her report of ‘countless’ skin infections prompted you to investigate further.You ordered some routine labs which is significant for a WBC count 30000/microliter. You suspect leukocyte adhesion deficiency in this baby. Of the following, which is the pathological hallmark of leukocyte adhesion deficiency?