Infection Phenotypes Among Patients with Primary Antibody Deficiency Mined from a US Patient Registry

Pickett, Grant; Motazedi, Tina; Kutac, Carleigh; Cahill, Gina; Cunnigham-Rundles, Charlotte; Fuleihan, Ramsay; Sullivan, Kathleen E.; Rider, Nicholas L.

doi:10.1007/s10875-020-00916-1

Cited by 6 publications

(4 citation statements)

References 36 publications

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“…Although the majority of patients affected by PID become symptomatic early in life, their confirmatory diagnosis may be delayed. 14,15 Thus, it was not surprising that nearly two-thirds of our patients with PID were diagnosed after pneumonia recurrence. Based on our results, we therefore now emphasize the need for periodically reviewing the underlying causes of RP since the disease spectrum is changing over time.…”

Section: Discussionmentioning

confidence: 94%

“…The Canadian study also included a broader cohort of patients with immune disorders—including those with malignant neoplasms and HIV infection—while we focused on PID. Although the majority of patients affected by PID become symptomatic early in life, their confirmatory diagnosis may be delayed 14,15 . Thus, it was not surprising that nearly two‐thirds of our patients with PID were diagnosed after pneumonia recurrence.…”

Section: Discussionmentioning

confidence: 98%

“…Among 551 patients, 90.6% were younger than 6 years of age; 66.4% were male; and 18.6% were born premature. The median age at first episode of pneumonia was 5 (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19) months, and the median age at diagnosis of RP was 14 (8-37) months (Table 1). Overall, 483 (87.7%) patients were identified as having at least one underlying disorder, and the proportions of prematurity and low birth weight were significantly higher in patients with underlying diseases.…”

Section: Patient Characteristicsmentioning

confidence: 99%

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Underlying causes and outcomes of recurrent pneumonia in hospitalized children

Mei

Dai

Guo

et al. 2023

Pediatric Pulmonology

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Objectives: To describe the clinical characteristics and underlying causes of recurrent pneumonia (RP) among hospitalized children, and to identify risk factors associated with adverse outcomes. Methods:We reviewed the medical records of hospitalized children diagnosed with RP at the Children's Hospital of Fudan University from January 2016 to January 2021 and then described clinical characteristics and underlying causes. The associations between factors and adverse outcomes were assessed using logistic regression.Results: Of 551 children with RP, 483 (87.7%) manifested underlying causes, with recurrent aspiration (127, 23.0%), primary immunodeficiency (PID) (91, 16.5%), and congenital heart diseases (63, 11.4%) being the most common. Genetic defects were identified in about a quarter (158, 28.7%) of the patients. PID odds ratio (OR, 7.9; 95% confidence interval [CI], 2.8-22.8), primary ciliary dyskinesia (OR, 12.9; 95% CI, 3.0-54.8), bronchiolitis obliterans (OR, 7.0; 95% CI, 1.7-28.5), and a diagnosis of RP at an age of >3 years (OR, 3.4; 95% CI, 1.3-9.0) were risk factors for severe outcomes. Aspiration (OR, 2.9; 95% CI, 1.3-6.3) and an abnormal family history (OR, 3.3; 95% CI, 1.3-8.2) were risk factors for rehospitalization. Conclusions:The majority (87.7%) of hospitalized children with RP exhibited underlying causes, and genetic defects were common.

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Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 98%

Section: Patient Characteristicsmentioning

confidence: 99%

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Underlying causes and outcomes of recurrent pneumonia in hospitalized children

Mei

Dai

Guo

et al. 2023

Pediatric Pulmonology

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“…Except for one asymptomatic child, all symptomatic children suffered from recurrent infections, mostly of the respiratory tract, and to a lesser extent from allergy and autoimmunity (32 and 9%, respectively). Indeed, as shown for other PAD patients [ 6 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 ], in unPAD children, recurrent respiratory infections represent the clinical hallmark, particularly in the first decade of life [ 18 ]. Conversely, immune dysregulation may complicate the clinical course mostly in early and late adulthood [ 14 , 18 ].…”

Section: Discussionmentioning

confidence: 99%

The Evolutionary Scenario of Pediatric Unclassified Primary Antibody Deficiency to Adulthood

Sgrulletti

Costagliola

Giardino

et al. 2023

JCM

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Background: Unclassified primary antibody deficiency (unPAD) is a relatively novel inborn error of immunity (IEI) condition that can vary with time to more defined entities. Since long-term follow-up (FU) studies are scarce, we aimed to provide insight into the evolutionary clinical and immunological scenario of unPAD children to adulthood and identification of biomarkers of primary immune deficiency (PID) persistence. Methods: A total of 23 pediatric unPAD patients underwent clinical and immunological FU for a mean time of 14 years (range 3–32 years, median 16 years). Results: UnPAD diagnosis may change over time. At the last FU, 10/23 (44%) children matched the diagnosis of transient hypogammaglobulinemia of infancy and 13/23 (56%) suffered from a persistent PID. In detail, an unPAD condition was confirmed in 7/23 (30%) patients, whereas 3/23 (13%), 2/23 (9%), and 1/23 (4%) were reclassified as common variable immunodeficiency, selective IgA deficiency, and isolated IgM deficiency, respectively. Low IgA, low specific antibody response to pneumococcus, and lower respiratory tract infections at diagnosis were independently associated with IEI persistence. Conclusions: Long-term monitoring of unPAD patients is required to define their outcome and possible evolution towards a definitive IEI diagnosis.

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