Individualized immunoglobulin treatment in pediatric patients with primary humoral immunodeficiency disease

Patel, Niraj

doi:10.1111/pai.12923

Cited by 8 publications

(9 citation statements)

References 51 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Pulmonary infection is the most common presentation of antibody deficiencies in children 12 . In the present study, 20/120 patients had low level of one immunoglobulin, and decreased IgA levels were associated with pneumonia.…”

Section: Discussionsupporting

confidence: 53%

Immune‐related factors associated with pneumonia in 127 children with coronavirus disease 2019 in Wuhan

Deng

Xiong

et al. 2020

Pediatric Pulmonology

View full text Add to dashboard Cite

Objective Information regarding the association of immune‐related factors with pneumonia in children with coronavirus disease 2019 (COVID‐19) is scarce. This study aims to summarize the immune‐related factors and their association with pneumonia in children with COVID‐19. Methods Children with COVID‐19 at Wuhan Children's Hospital from 28 January to 12 March 2020 were enrolled. Pneumonia due to causes other than COVID‐19 were excluded. The clinical and laboratory information including routine blood tests, blood biochemistry, lymphocyte subsets, immunoglobulins, cytokines, and inflammatory factors were analyzed retrospectively in 127 patients. Normal ranges and mean values of laboratory markers were applied as parameters for logistic regression analyses of their association with pneumonia. Results In nonintensive care unit patients, 48.8% and 22.4% of patients had increased levels of procalcitonin and hypersensitive C‐reactive protein (hs‐CRP) respectively. A total 12.6% and 18.1% of patients had decreased levels of immunoglobulin A (IgA) and interleukin 10 (IL‐10), respectively. Approximately 65.8% of patients had pneumonia. These patients had decreased levels of globulin (odds ratio [OR], 3.13; 95% confidence interval [CI] 1.41‐6.93; P = .005), IgA (OR, 4.00; 95% CI, 1.13‐14.18; P = .032), and increased levels of hs‐CRP (OR, 3.14; 95% CI, 1.34‐7.36; P = .008), procalcitonin (OR, 3.83; 95% CI, 2.03‐7.24; P < .001), IL‐10 (OR, 7.0; 95% CI, 1.59‐30.80; P = .010), and CD4+ CD25+ T lymphocyte less than 5.0% (OR, 1.93; 95% CI, 1.04‐3.61; P = 0.038). Conclusion Decreased IgA and CD4+ CD25+ T lymphocyte percentage, and increased hs‐CRP, procalcitonin, and IL‐10 were associated with pneumonia, suggesting that the immune‐related factors may participate in the pathogenesis of pneumonia in children with COVID‐19.

show abstract

Section: Discussionsupporting

confidence: 53%

Immune‐related factors associated with pneumonia in 127 children with coronavirus disease 2019 in Wuhan

Deng

Xiong

et al. 2020

Pediatric Pulmonology

View full text Add to dashboard Cite

show abstract

“…Several genes have been identified to be associated with the development of SCID. 11,12 However, in more than 15% of children with confirmed SCID, the associated genes are unknown. Mostly the inheritance of SCID is recessive autosomal, in which the both copies of particular defective genes from the parents is responsible for the development of disease.…”

Section: Discussionmentioning

confidence: 99%

Update: Primary immunodeficiency disorders among north Indian children

Deepak

Kumar²,

Saurabh³

et al. 2021

IJPO

View full text Add to dashboard Cite

Primary immunodeficiency disorders (PIDs) are a group of genetic abnormalities characterized by defectin one or more constituents of the immune system.This group of disorders are largely undiagnosed and unreported worldwide due to lack of awareness among the medical practitioners,parents as well as lack of state of art diagnostic facilities. Earlier we had reported the distribution pattern of various categories of PID in children of north India; in this report we are appending the data with current findings.In this retrospective study we pooled data from PIDs workup of 706 children with suspected PIDs, below the age of 18Yrs, in the period of May 2017 October 2019. The clinical assessment and presentation of these children was suggestive of PID. The peripheral blood of these children was used for flow cytometry based immunophenotyping of immune cells. PIDs were classified according to the International Union of Immunological Societies’ (IUIS) criteria.A total of 133 (18.38%) children were diagnosed with one or other form of PID with overall median age was 3.25 years (male: 2.3 and female: 4.2Yrs). Chronic infection, persistent diarrhea and retarded growth were the common warning signsin these patients. Combined humoral and cellular immunodeficiency was observed in 32%, phagocytic defect in 23%, antibody defect in 17%, dysregulated innate immunity in 19% and other well defined syndromes in 9% of total diagnosed PID children. Around 15.78% of PID cases were seen in coupleswithconsanguineous marriage, past family history of PID in 20.30% and families with sibling death of unknown cause in 24.06%. The cause of death of the sibling was not known. PID diagnosed children received prophylactic antibiotics and/or antifungals in addition tospecific therapy for the underlying immune deficiency.The field of PID remainsunexplored worldwide. The awareness in the developed countries is more than that of developing countries like India. The developing countries face several challenges in the diagnosis of PIDs such as awareness among patients and medical practitioners, mostly in the rural settings, lack of sufficient number of tertiary care centres, lack of equipped immunological laboratory to diagnose the disease.

show abstract

“…Optimization of IgG therapy can lead to a reduction in the frequency and severity of serious bacterial infections and end-organ damage with an improved health-related quality of life 9,[18][19][20][21][22][23][24][25][26][27][28] . SCIG is efficacious, can be administered at home, is associated with fewer systemic side effects than IVIG, and often requires no pre-medications 29 . The aims of this retrospective pilot study were to identify and evaluate children with recurrent bacterial sinopulmonary infections who had appropriate rise in pneumococcal antibody titers following immunization with PPSV23 but low response by avidity.…”

Section: Introductionmentioning

confidence: 99%

Comparison of Pneumococcal Avidity and Antibody Concentration in Children with Recurrent Infections: A Retrospective Pilot Study

Intner¹,

Altrich²,

Patel³

2020

J Immunol Sci

View full text Add to dashboard Cite

Measurement of pneumococcal antibody concentration is a frequently used parameter for functional antibody response to vaccination. Antibody concentration in response to vaccination and strength of antigen-antibody (avidity) interaction are both important measurements of functional antibody response. Both antibody concentration and avidity contribute to immunity against invasive pneumococcal disease. Higher avidity is correlated with increasing bactericidal activity and opsonophagocytosis. On the other hand, patients with lower pneumococcal avidity may be more likely to develop clinically significant pneumococcal sinopulmonary infections. Nine patients with recurrent bacterial respiratory infections were identified by retrospective chart review as having adequate pneumococcal antibody concentrations, but with low avidity for multiple serotypes following immunization with pneumococcal vaccine polyvalent (PPSV23). We assessed response with IgG replacement therapy in these patients. The mean number of serotypes with a normal antibody response (>1.3 mg/ml) among 9 children following immunization with pneumococcal vaccine polyvalent was 19.1 (range 12-22) of 23 serotypes while the mean number of serotypes with a normal avidity response (≥1.0) was 4.7 (range 2-7) of 23 serotypes. Flow cytometry was performed for 8 of the 9 patients prior to starting SCIG replacement therapy. 100% of the cohort experienced a significant decrease in yearly infection rate after starting immunoglobulin replacement. This is the first study to assess the clinical response to immune globulin replacement in patients with normal pneumococcal antibody response but poor pneumococcal avidity, and suggests that patients with poor pneumococcal avidity but apparent normal response by pneumococcal antibody following PPSV23 may benefit from IgG replacement therapy.

show abstract

Individualized immunoglobulin treatment in pediatric patients with primary humoral immunodeficiency disease

Cited by 8 publications

References 51 publications

Immune‐related factors associated with pneumonia in 127 children with coronavirus disease 2019 in Wuhan

Immune‐related factors associated with pneumonia in 127 children with coronavirus disease 2019 in Wuhan

Update: Primary immunodeficiency disorders among north Indian children

Comparison of Pneumococcal Avidity and Antibody Concentration in Children with Recurrent Infections: A Retrospective Pilot Study

Contact Info

Product

Resources

About