Immunocompromised Patients and Disease
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Questions and Answers

Which condition is most likely associated with recurrent pneumonias in a child with chronic granulomatous disease?

  • Zygomycosis (correct)
  • Influenza
  • Sepsis
  • Group B Streptococcus (GBS)

What is a significant laboratory finding in a child suspected of having chronic granulomatous disease?

  • Increased susceptibility to infections (correct)
  • Decreased white blood cell count
  • Elevated blood glucose levels
  • Normal immune function

Which of the following organisms is typically responsible for infections in chronic granulomatous disease patients?

  • Staphylococcus aureus
  • Streptococcus pneumoniae
  • E. coli
  • Aspergillus species (correct)

Which symptom is least likely to be associated with severe sepsis in children?

<p>Increased platelet count (A)</p> Signup and view all the answers

In a child with suspected chronic granulomatous disease, which of the following findings would indicate a potential lung infection?

<p>Presence of diffuse rhonchi (B)</p> Signup and view all the answers

What is the most important clinical feature in diagnosing chronic granulomatous disease in children?

<p>Recurrent bacterial infections (C)</p> Signup and view all the answers

Which pathogen poses the greatest risk to a child diagnosed with chronic granulomatous disease?

<p>Burkholderia cepacia (C)</p> Signup and view all the answers

In the context of chronic granulomatous disease, which treatment option is least effective?

<p>Antiviral medications (D)</p> Signup and view all the answers

What type of infection is frequently associated with Staphylococcus aureus in patients with chronic granulomatous disease (CGD)?

<p>Liver abscesses (A)</p> Signup and view all the answers

Which organism is known for causing severe lung destruction and is often associated with high fever in patients with CGD?

<p>Nocardia (C)</p> Signup and view all the answers

In the context of CGD, which of the following respiratory infections typically does NOT lead to fever?

<p>Fungal pneumonia (D)</p> Signup and view all the answers

What is a common gastrointestinal manifestation that affects a significant portion of patients with chronic granulomatous disease?

<p>Diarrhea (D)</p> Signup and view all the answers

Which condition, if present, warrants testing for chronic granulomatous disease in infants?

<p>Any infection with atypical organisms (C)</p> Signup and view all the answers

Which organism is specifically indicated as a common cause of osteomyelitis in infants with CGD?

<p>Serratia marcescens (C)</p> Signup and view all the answers

What is a classic physical examination finding in children experiencing severe infections affecting CGD?

<p>Finger clubbing (A)</p> Signup and view all the answers

Which symptom is NOT typically associated with the pneumonia seen in CGD patients infected by Aspergillus?

<p>Fever (B)</p> Signup and view all the answers

What is a common feature of chronic granulomatous disease (CGD)?

<p>Impaired ability to produce hydrogen peroxide (C)</p> Signup and view all the answers

Which pathogen is NOT typically associated with chronic granulomatous disease?

<p>Mycobacterium tuberculosis (B)</p> Signup and view all the answers

Which of the following statement accurately describes a consequence of impaired phagocyte function in CGD?

<p>Increased risk of granuloma formation (C)</p> Signup and view all the answers

What is the estimated prevalence of chronic granulomatous disease in the US?

<p>1 in 250,000 live births (C)</p> Signup and view all the answers

Which immunodeficiency is characterized by a defect in leukocyte adhesion?

<p>Leukocyte adhesion deficiency Type 1 (LAD1) (A)</p> Signup and view all the answers

In patients with phagocyte dysfunction, which of the following types of infections is most likely to be recurrent?

<p>Respiratory tract infections (B)</p> Signup and view all the answers

What additional health issue is often observed in children with chronic granulomatous disease?

<p>Failure to thrive (C)</p> Signup and view all the answers

Which of the following is NOT a recommended focus for managing chronic granulomatous disease?

<p>Immunosuppressive therapy (D)</p> Signup and view all the answers

Flashcards

Primary immunodeficiencies

A group of disorders that affect the immune system, causing a weakened ability to fight infections. These deficiencies can affect different parts of the immune system, including phagocytes, complement, B cells, and T cells.

Phagocyte function defects

Defects in the ability of phagocytes (white blood cells that engulf and destroy pathogens) to function properly. This can lead to severe infections caused by common pathogens.

Chronic granulomatous disease (CGD)

A rare hereditary disease where neutrophils (a type of phagocyte) are unable to kill ingested pathogens, leading to the formation of granulomas in various organs.

LAD1 (Leukocyte adhesion deficiency Type 1)

A type of phagocyte defect caused by a mutation in the CD18 gene, preventing phagocytes from migrating to infection sites and engulfing bacteria.

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What causes Chronic Granulomatous Disease (CGD)?

Mutations in genes responsible for producing NADPH oxidase, an enzyme crucial for generating reactive oxygen species (like hydrogen peroxide) needed to kill pathogens.

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What are common infections associated with CGD?

CGD patients are prone to infections by specific bacteria, including Staphylococcus aureus, Burkholderia cepacia complex, Serratia marcescens, and Nocardia sp.

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What is the prevalence of CGD?

CGD is a rare disease, with a prevalence of approximately 1 in 250,000 live births in the US and UK.

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Are children with CGD healthy at birth?

Yes, children with CGD are typically healthy at birth but often develop recurrent infections later in life.

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Recurrent Pneumonias

Frequent occurrences of lung infections, often indicating an underlying immune system problem.

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Clubbing

A widening and curving of the fingertips, often associated with chronic lung diseases.

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Splenomegaly

Enlargement of the spleen, an organ that filters blood and plays a role in immunity.

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Lymphadenopathy

Swollen lymph nodes, which are small bean-shaped organs in the immune system.

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Cervical, Axillary, Preauricular

Locations of the swollen lymph nodes - neck (cervical), armpit (axillary), and ear (preauricular).

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Febrile

Having a fever, a sign of the body fighting infection.

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Tachycardia

Fast heart rate, a sign of the body working harder to compensate for the illness.

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CGD Infection in Infancy

Common infections in infants with Chronic Granulomatous Disease (CGD) are skin infections like impetigo and cellulitis, and bone infections like osteomyelitis, often caused by Serratia marcescens.

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Suspect CGD in Infants

Any infant with an infection caused by organisms like Serratia marcescens, Staphylococcus aureus, or Aspergillus should be tested for CGD.

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Aspergillus Pneumonia

Aspergillus pneumonia in CGD patients may not cause fever, unlike bacterial pneumonias. Nocardia, another common pathogen, causes severe fever and lung damage.

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CGD and Gastrointestinal Issues

Around 40-50% of patients with CGD experience gastrointestinal problems.

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Case Study Symptoms

The 12-year-old boy presented with fever, chills, sweats, productive cough, nausea, and vomiting. He had a history of recurrent pneumonias since the age of 5.

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Physical Examination Findings

The patient had finger clubbing, splenomegaly, and significant lymphadenopathy in various areas of his body.

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Case Study Diagnosis

Blood cultures revealed Staphylococcus aureus, chest X-ray and CT scan showed multiple bilateral abscesses in both lungs.

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Study Notes

Paediatric Infections (Neonatal)

  • Neonatal deaths: Worldwide, more than 40% of deaths occur within the neonatal period. That is, 3.1 million newborn deaths each year. The majority of these deaths occur in low-income countries.
  • Listeria monocytogenes: This bacterium is a significant cause of neonatal infections. It's a foodborne zoonosis that can pass from pregnant women to their fetuses and newborns, potentially causing septicemia, meningitis, and other severe complications. Risky foods include raw sprouts, soft cheese, smoked fish, and raw milk.
  • Whooping cough (Pertussis): Caused by Bordetella pertussis, this highly contagious respiratory infection can be fatal, particularly in infants under 4 months old. It is spread through respiratory droplets and is characterized by distinctive paroxysmal coughing episodes. Vaccination is a crucial preventative measure. Pregnant women should get vaccinated. Babies, people with a weak immune system and those with waning immunity are at risk.
  • Neonatal Infections: Common causes include E. coli, Staphylococcus aureus, Streptococcus agalactiae (Group B Strep), Listeria monocytogenes, and Haemophilus influenzae.
  • Risk factors in the mother for early-onset infections include: invasive group B strep infection, prelabour rupture of membranes, preterm birth, intrapartum fever.
  • Clinical indicators in the baby for possible early-onset infections include: altered behavior, feeding difficulties, abnormal heart rate, signs of respiratory distress, jaundice, need for mechanical ventilation, persistent fetal circulation, temperature abnormalities, signs of shock & unexplained bleeding, oliguria, altered glucose (hypo/hyperglycemia).
  • Treatment of possible infections: Antibiotics such as penicillin or gentamicin should be administered rapidly - ideally within one hour of making the decision to treat.

Geriatric Infectious Diseases

 - Geriatric population demographics: The elderly population (defined in this context as individuals >65 years old) is rapidly increasing globally. This trend has significant implications for healthcare systems and infectious disease management.

  • Increased risk factors: Factors like lifestyle, pre-existing health conditions (e.g., diabetes, heart disease and organ dysfunction) and diminished immunity contribute to higher susceptibility of the elderly to infections.
  • Healthcare-associated infections (HCAIs): These infections are a significant public health concern particularly amongst older adults who are often in long-term care facilities (LTCFs). Relocation between nursing homes is a crucial factor in the transfer and spread of antimicrobial resistance (AMR).
  • Common infections: Common infections include respiratory and gastrointestinal infections. Nosocomial infections (occur within hospital setting), UTIs, hospital acquired pneumonia and bacteremia are frequent.
  • Pneumococcal diseases: This highly contagious bacterial disease is a significant risk factor for the elderly, frequently leading to hospitalization and increased morbidity and mortality, in part, due to the consequences of complications from bacteraemia and meningitis. A vaccine is available.
  • Influenza: Influenza (flu), especially strains A and B, are seasonal illnesses that pose a significant health risk for the elderly, due to their increased susceptibility to complications such as pneumonia, cardiac respiratory failure, and exacerbation of pre-existing conditions.Vaccination is recommended.
  • Infections relating to chronic diseases associated with compromised immunity in the elderly, such as diabetes, is an important consideration—the physiological dysfunctions can affect susceptibility to infections.
  • Mucormycosis (Zygomycosis): A potentially serious fungal infection is a risk for the elderly. Infections often related to compromised immunity.

Infections in Individuals with Primary Immunodeficiencies

  • Primary immunodeficiencies: Hereditary or congenital deficiencies in the immune system.
  • Defects in phagocyte function: Severe infections due to common pathogens and frequent granulomas (collections of immune cells).
  • Complement deficiencies: Rheumatoid disorders, lupus and scleroderma,
  • B-cell immunodeficiencies: Recurrent respiratory tract infections and chronic gastrointestinal infections. 
  • T-cell immunodeficiencies (Combined immunodeficiencies): T-cell immunodeficiencies (typically combined with B dysfunctions) can also cause failure to thrive and oral thrush. 
  • Chronic granulomatous disease (CGD): A hereditary disease in which neutrophil granulocytes are unable to destroy certain types of ingested pathogens. Consequently it leads to potentially life-threatening infections by a wide range of microbes such as Staphylococcus aureus, Burkholderia cepacia complex, Serratia marcescens, Nocardia sp., and Aspergillus.

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