The Medical Awareness Concerning Primary Immunodeficiency Diseases (PID) in the City of São Paulo, Brazil

Dantas, Eber; Aranda, Carolina Sánchez; Nobre, Fernanda Aimée; Essiben, Félix; Fahl, Kristine; Mazzucchelli, Juliana Themudo Lessa; Friedenbach, D.L.; Nudelman, Victor; Condino‐Neto, Antônio; Lin, Li; Chaves, J.D.; Costa-Carvalho, B. T.

doi:10.1016/j.jaci.2011.12.648

Cited by 4 publications

(11 citation statements)

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“…Brazilian studies showed that 77% of physicians did not know the 10 warning signs for primary immunodeficiency and only 40.3% had further examined patients who frequently use antibiotics for PID [8,9]. The indicator for knowledge obtained in this study had a mean of 48.08% ±16.06.…”

Section: Discussionmentioning

confidence: 73%

Physicians’ awareness concerning primary immunodeficiencies in the Ternopil Region of Ukraine

Boyarchuk¹,

Lewandowicz-Uszyńska²,

Kinash³

et al. 2018

polp

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Introduction: Primary immunodeficiencies (PID) are genetic defects of the immune system that result in chronic, serious, and often life-threatening infections if they are not diagnosed and treated. Worldwide, from 70 to 90% of PID sufferers remain undiagnosed because of poor awareness. Early diagnosis is very important for adequate prevention and management of PID infectious complications and may improve the life quality of patients with PID. Aim of the study: The aim of this study was to assess the awareness of physicians of different specialties about the signs of primary immunodeficiency in children and adults. Material and methods: A survey among physicians of different specialties on awareness of PID was conducted. The study involved physicians of the Ternopil region of Ukraine. Of 103 participants 42 were paediatricians, 25 general practitioners/family physicians, eight internists, 13 paediatric surgical specialists, and 15 physicians of paediatric sub-specialties. The survey consisted of a questionnaire containing 25 questions. Results: Sixty-one (59.2%) physicians gave more than 50% of correct answers. The percentage of correct answers to the questions about warning signs of PID in children was 66.1% among paediatricians and 64% among general practitioners, in adults-66.7% and 72%, respectively. The lowest knowledge of PID was about the specific signs of PID, more often concerning verification of Nijmegen breakage syndrome, ataxia-telangiectasia, and DiGeorge syndrome. Conclusions: This study has revealed poor awareness about PID among physicians in the Ternopil region of Ukraine. There was no significant difference in the percentages of correct answers among the first-contact physicians (paediatricians, general practitioners/family physicians) and paediatric sub-specialists. There is a significant need for educational programs to improve physicians' knowledge on PID. Implementation of the model of combined physician education and public awareness may improve detection of PID in children in the early stages.

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Section: Discussionmentioning

confidence: 73%

Physicians’ awareness concerning primary immunodeficiencies in the Ternopil Region of Ukraine

Boyarchuk¹,

Lewandowicz-Uszyńska²,

Kinash³

et al. 2018

polp

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“…Twenty-seven of 100 patients had IgG hypogammaglobinemia (median: 598 mg/dL, IQ range: 459-654), 23 of 100 had IgM hypogammaglobinemia (median: 38 mg/dL, IQ range: 25-43), and 6 of 100 had IgA hypogammaglobinemia (median: 36 mg/dL, IQ range: 18-50). The median hospital length of stay for patients with IgG hypogammaglobinemia was significantly higher when compared to patients with normal IgG levels (five days, IQ range [3][4][5][6][7][8][9][10] vs three days, IQ range [2][3][4][5], P = .0085). Fourteen patients underwent further immune evaluation, resulting in one diagnosis of multiple myeloma, three patients diagnosed with specific antibody deficiency, and one patient diagnosed with selective IgA deficiency.…”

Section: Resultsmentioning

confidence: 93%

“…Primary immunodeficiency (PIDD) is estimated to affect one in 1,800 individuals in the United States 4 . The National Institutes of Health (NIH) estimates that only one out of three individuals with PIDD are appropriately diagnosed.…”

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confidence: 99%

Screening for Humoral Immunodeficiency in Patients with Community‐Acquired Pneumonia

Vadamalai

Sanche-Tejera

Bress

et al. 2018

Journal of Hospital Medicine

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BACKGROUND Immunodeficiency is an underrecognized risk factor for infections, such as community‐acquired pneumonia (CAP). OBJECTIVE We evaluated patients admitted with CAP for humoral immunodeficiency. DESIGN Prospective cohort study SETTING Inpatients PATIENTS, INTERVENTION, AND MEASUREMENTS We enrolled 100 consecutive patients admitted with a diagnosis of CAP from February 2017 to April 2017. Serum IgG, IgM, IgA, and IgE levels were obtained within the first 24 hours of admission. CURB‐65 score and length of hospital stay were calculated. The Wilcoxon rank‐sum test, Kruskal‐Wallis test, and simple linear regression analysis were used in data analysis. RESULTS The prevalence of hypogammaglobinemia in patients with CAP was 38% (95% CI: 28.47% to 48.25%). Twenty‐seven of 100 patients had IgG hypogammaglobinemia (median: 598 mg/dL, IQ range: 459‐654), 23 of 100 had IgM hypogammaglobinemia (median: 38 mg/dL, IQ range: 25‐43), and 6 of 100 had IgA hypogammaglobinemia (median: 36 mg/dL, IQ range: 18‐50). The median hospital length of stay for patients with IgG hypogammaglobinemia was significantly higher when compared to patients with normal IgG levels (five days, IQ range [3‐10] vs three days, IQ range [2‐5], P = .0085). Fourteen patients underwent further immune evaluation, resulting in one diagnosis of multiple myeloma, three patients diagnosed with specific antibody deficiency, and one patient diagnosed with selective IgA deficiency. CONCLUSION There is a high prevalence of hypogammaglobinemia in patients hospitalized with CAP, with IgG and IgM being the most commonly affected classes. IgG hypogammaglobinemia was associated with an increased length of hospitalization. Screening immunoglobulin levels in CAP patients may also uncover underlying humoral immunodeficiency or immunoproliferative disorders.

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“…There has been an increasing concern regarding costs of medical assistance for patients with different chronic diseases, including primary immunodeficiencies (PID) (1-7), which are not so rare and often underdiagnosed. In PID, several studies have shown that costs are often associated with delayed diagnosis (8)(9)(10)(11)(12)(13)(14). This generates a series of deleterious events for the patient, their families and the health system, especially regarding hospitalizations due to infection (8)(9)(10)(11)(12)(13)(14).…”

mentioning

confidence: 99%

“…In PID, several studies have shown that costs are often associated with delayed diagnosis (8)(9)(10)(11)(12)(13)(14). This generates a series of deleterious events for the patient, their families and the health system, especially regarding hospitalizations due to infection (8)(9)(10)(11)(12)(13)(14). The ideal diagnostic approach for PIDs includes extensive immunophenotyping of peripheral lymphocyte subsets, functional and molecular tests, and should be performed as early as possible (15,16).…”

mentioning

confidence: 99%

Managing costs in primary immunodeficiency: minimal immunophenotyping and three national references

Dias

Silva

Cunha

et al. 2019

APMIS

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Dias ALA, da Silva RG, Cunha FGP, Morcillo AM, Lorand-Metze I, Vilela MMS, Riccetto AGL. Managing costs in primary immunodeficiency: minimal immunophenotyping and three national references. APMIS 2019; 127: 228-235.Our aim was to evaluate the cost-effectiveness of a minimal lymphocyte subset quantification (LSQ) by flow cytometry as the first screening in children with clinically suspected primary immunodeficiency (PID). Two hundred sixty-eight Brazilian patients (0-21 years old) were studied. They were divided by clinical and phenotypical features into those fulfilling criteria for PID (PID phenotype) according to the 2017 International Union of Immunological Societies (IUIS) classification and those not fulfilling these criteria (non-PID phenotype). We evaluated how many patients had values below the 10th percentile for five lymphocyte subsets in peripheral blood, (suggestive of PID) according to reference values for Brazil, Italy and USA. Three lymphocyte subsets (T CD3/CD4, B CD19 and NK CD16/CD56) had p-value < 0.05 and Odds Ratio (OR) indicating a risk at least two times higher for the diagnosis of a PID phenotype. The application of Kappa coefficient (k) on Brazilian vs Italian and Brazilian vs US data sets resulted in k compatible with strong or excellent level of agreement between the three classification systems. The authors conclude that a number of CD3 + / CD4 + , CD19 + and CD16 + /CD56 + (NK) cells in peripheral blood <10th percentile represented a significant risk for the diagnosis of PID in this cohort. Natural killer (NK) deficiency is quite rare and has a very specific clinical profile. So, the analysis of these cells could be requested only in some cases, saving even more costs. The minimal immunophenotyping, with quantification of T CD4 + , CD19 + and in some cases CD16 + /CD56 + cells, may be a useful tool for the first screening of PID, saving costs, especially in developing countries.

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The Medical Awareness Concerning Primary Immunodeficiency Diseases (PID) in the City of São Paulo, Brazil

Cited by 4 publications

References 0 publications

Physicians’ awareness concerning primary immunodeficiencies in the Ternopil Region of Ukraine

Physicians’ awareness concerning primary immunodeficiencies in the Ternopil Region of Ukraine

Screening for Humoral Immunodeficiency in Patients with Community‐Acquired Pneumonia

Managing costs in primary immunodeficiency: minimal immunophenotyping and three national references

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