A mother brings her 3 year-old son to the urgent care clinic. She reports that since this morning, her boy has developed a high fever, joint pains, itching, ankle swelling and erythematous plaques. He is otherwise playful and active. He was prescribed cefaclor 12 days ago by his pediatrician for an ear infection. She stopped cefaclor 2 days ago but wonders why the rash is not going away. On physical examination, the patient has a maculopapular rash on his trunk. Pharyngeal examination is normal. There are no mucosal lesions. He has submandibular lymphadenopathy. Heart and lung examinations are normal.  Of the following, which is the most likely diagnosis? 

A 22 year-old African immigrant male came to the emergency room reporting fever, chills, sweating at night, cough, pain in the chest, blood in the sputum and weight loss. His medical history is significant for AIDS and cryptococcal meningitis. He was diagnosed with HIV four years ago. Two months ago he was treated in this hospital for cryptococcal meningitis with IV infusions of amphotericin B and flucytosine. He improved well and was discharged in good condition. At the time of his discharge, his  CD4+ cell count was 84 cells per microliter and HIV-1 viral load was 316232  RNA copies/mL.   Two weeks after his discharge, he was started on highly active antiretroviral therapy (HAART) for HIV. He reports that he felt ‘great’ since his discharge from the hospital till this new sickness came up.   His vital signs were, Temp = 101.2 F, O2 saturation 95%, BP 90/60 mm Hg, RR 17/minute, Pulse 94/minute.  Some of the laboratory results are as follows:  CD4 count is 1200 cells/mm3 HIV-1 viral load of 4800 RNA copies/mL Nucleic Acid Amplification Test is positive for mycobacterium tuberculosis in the sputum. Chest X ray showed bilateral lower lung zone infiltrates and mediastinal lymphadenopathy.        On hearing these reports, the patient stated that while there are lots of people with tuberculosis in his native Senegal, he never thought he would get it.  Which of the following was a risk factor for immune reconstitution inflammatory 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. After receiving treatment for cryptococcal meningitis, a HIV patient expressed interest to receive highly active antiretroviral therapy (HAART). Of the following, which describes the best way to reduce the risk of 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 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 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?

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). What is the most likely diagnosis in this patient?