A Comprehensive Approach to the Management of Children and Adults with Chronic Granulomatous Disease

Thomsen, Isaac; Smith, M.; Holland, Steven M.; Creech, C. Buddy

doi:10.1016/j.jaip.2016.03.021

Cited by 56 publications

(57 citation statements)

References 27 publications

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“…Common variable immunodeficiency patients are not particularly prone to bacterial and/or parasitic infections primarily involving the liver (69). Similarly, the finding of opportunistic or unusual pathogens, such as Microsporidia or Cryptosporidia, is rare and might suggest investigating for a combined immunodeficiency, characterized by greater degrees of T-cell dysfunction (70).…”

Section: Infectionsmentioning

confidence: 99%

Heterogeneity of Liver Disease in Common Variable Immunodeficiency Disorders

et al. 2020

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Section: Infectionsmentioning

confidence: 99%

Heterogeneity of Liver Disease in Common Variable Immunodeficiency Disorders

et al. 2020

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“…TMP-SMX for the prevention of infections in patients with CGD is typically dosed at 5 mg/kg per day (based on the TMP component) up to 1 double-strength tablet daily [7]. Although TMP-SMX therapy is generally well tolerated, it does have a variety of potential toxicities.…”

Section: Prophylactic Antibiotic Therapymentioning

confidence: 99%

Prevention of Infectious Complications in Patients With Chronic Granulomatous Disease

Slack

Thomsen

2018

Journal of the Pediatric Infectious Diseases Society

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Chronic granulomatous disease (CGD) is a primary immunodeficiency that confers a markedly increased risk of bacterial and fungal infections caused by certain opportunistic pathogens. Current evidence supports the use of prophylactic antibacterial, antifungal, and immunomodulatory therapies designed to prevent serious or life-threatening infections in patients with CGD. In this review, we discuss current strategies for the prevention of infections in children and adults with CGD and the evidence that supports those strategies. In addition, we address current challenges and opportunities for future research in this important area.

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“…Infections typically involve the lung (pneumonia), lymph nodes (lymphadenitis), liver (abscess), bone (osteomyelitis), and skin (abscesses or cellulitis) while granulomas typically involve the genitourinary system (bladder) and gastrointestinal tract (often the pylorus initially, and later the esophagus, jejunum, ileum, cecum, rectum, and perirectal area) [5]. The current mortality rate is 2-5% per year [6].…”

Section: To the Editormentioning

confidence: 99%