Primary Immunodeficiency Diseases in Aguascalientes, Mexico: Results from an Educational Program

Álvarez-Cardona, Aristóteles; Espinosa‐Padilla, Sara Elva; Reyes, Saúl; Ventura-Juárez, Javier; López-Valdez, Jaime; Martínez-Medina, Lucila; Santillan-Artolozaga, Alberto; Cajero-Avelar, Adriana; Luna-Sosa, Alma R De; Torres-Bernal, Luis Fernando; Espinosa-Rosales, Francisco

doi:10.1007/s10875-016-0242-0

Cited by 10 publications

(7 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Among the children that were referred with suspected PID 12.7% of the cases were confirmed [14], which is consistent with the results of other study which showed a PID prevalence 9.9% among those referred for evaluation [15].…”

Section: Plos Onesupporting

confidence: 90%

“…The mean delay from initial symptoms to diagnosis in the patients from our center was 31.5 months (2.6 years), ranging from one to 156 months [16]. Other studies reported the mean delay from initial symptoms to a referral for medical advice at 3.7 years [15]; the mean, depending on a PID category, was from 6 to 56 months, and the range from 1 to 134 months [17]. In our previous study, the longest delay to a diagnosis was in a newly diagnosed 16 yearold patient with AT [16].…”

Section: Plos Onementioning

confidence: 68%

“…The effectiveness of implementing an educational program was demonstrated in a study from Mexico [15], which found improved diagnostic outcomes at the regional level. They estimated that 75 physicians need to be taught to get one PID patient referral [15]. Therefore, education is a long and painstaking process, but it is very necessary to improve the diagnosis of PIDs.…”

Section: Plos Onementioning

confidence: 99%

See 2 more Smart Citations

Evaluation of awareness about primary immunodeficiencies among physicians before and after implementation of the educational program: A longitudinal study

et al. 2020

View full text Add to dashboard Cite

Increasing physicians' awareness is one of the main ways to improve early diagnosis of rare diseases. A survey among physicians of different specialties to evaluate the knowledge about primary immunodeficiencies (PID) was conducted in 2016 and in 2019 -before and after the implementation of an educational program. We compare responses from 82 doctors who participated in the 2016 survey, and 67 doctors who have taken part in the survey in 2019: pediatricians, general practitioners / family physicians and physicians of pediatric sub specialties. The percentage of correct answers to all survey questions after the implementation of the educational program has significantly increased (79.0% in 2019 versus 58.3% in 2016, P<0.0001). This increase in the percentage of correct answers was noted among the surveyed doctors of all specialties. Particular progress was found among pediatricians, who have achieved more than 80% of correct answers. In 2019 the doctors demonstrated better knowledge on the warning signs of PID and specific features of Nijmegen breakage syndrome, DiGeorge syndrome and ataxia-telangiectasia syndrome. Thus, the implementation of an educational program improved physicians' awareness of PIDs, and will contribute to early detection of PIDs and their medical care.

show abstract

Section: Plos Onesupporting

confidence: 90%

Section: Plos Onementioning

confidence: 68%

Section: Plos Onementioning

confidence: 99%

See 1 more Smart Citation

Evaluation of awareness about primary immunodeficiencies among physicians before and after implementation of the educational program: A longitudinal study

et al. 2020

View full text Add to dashboard Cite

show abstract

“…[11,12] However, the studies in some PID centers also established the prevalence of CID among the cases. [2,13,14] Not all PID groups are represented in our regional PID registry. Defects in intrinsic and innate immunity, autoinflammatory disorders, and complement deficiencies were not reported.…”

Section: Discussionmentioning

confidence: 99%

Clinical Manifestations in the Patients with Primary Immunodeficiencies: Data from One Regional Center

Boyarchuk¹,

Dmytrash²

2019

Turk J Immunol

View full text Add to dashboard Cite

Introduction: Defining clinical signs is very important for timely diagnosis of diseases, as well as primary immunodeficiencies (PID). The aim of our study was to identify manifestations and their specific features in the patients of Ternopil region, Ukraine, in order to improve early detection of primary immunodeficiencies. Materials and Methods: This retrospective and prospective study involved 36 patients with primary immunodeficiencies, whose clinical symptoms were followed up from the disease onset. A prospective analysis was performed for 26 patients, while retrospective analysis based on the review of medical records was conducted for 10 patients. Results: Combined immunodeficiencies with associated or syndromic features such as Nijmegen breakage syndrome, 22q11.2 deletion syndrome, ataxia-teleangiectasia, were diagnosed the most frequently and followed by antibody deficiencies. The mean delay from initial symptoms to diagnosis was 31.5 months, and ranging from one to 156 months. Recurrent bacterial respiratory tract infections were evidenced in 61.1% of patients, recurrent viral respiratory infections-in 44.4% of patients. Skin infections were reported in 25.0% of all cases. Allergies were present in 11.1% and autoimmune diseases in 16.7% of all patients; malignancy was revealed in one case. Conclusions: Infections were the most prominent clinical manisfestation PIDs, although more than 30% of the children have had PID manifestations accompanied by autoimmune disorders, allergies, or malignancies. Taking into account regional features of the PID incidence, more attention should be given to the clinical signs of microcephaly, ataxia, teleangiectasia and congenital heart defects in PID diagnosis. Lymphopenia and characteristic dysmorphic features are also warning signs for PID in the neonatal period and early infancy.

show abstract

“…As the diagnostic delay is still high in our study cohort and similar cohorts around the world, even with different resource settings, we should improve our efforts in order to diminish it. The creation of an educational program for health personnel and general public, could be a promising strategy to achieve this goal, as has been recently shown by a group or researchers in Aguascalientes Mexico [18]. …”

Section: Discussionmentioning

confidence: 99%

Disease burden for patients with primary immunodeficiency diseases identified at reference hospitals in Guanajuato, Mexico

et al. 2017

View full text Add to dashboard Cite

BackgroundIn addition to the deleterious effect on health, there is considerable economic and psychosocial morbidity associated with primary immunodeficiency diseases (PID). Also, the cost of a late diagnosis frequently results in a heavy disease burden on the patient. The objective of this study was to collect and analyze data on patients with PID in the state of Guanajuato in Mexico, to indirectly estimate the burden of the disease.MethodsAn observational, longitudinal, and comparative study was conducted. A total of 44 patients were included and grouped according to the updated classification of PID.ResultsThe median time elapsed from the onset of symptoms to the reference and diagnosis by a tertiary hospital was of 2.17 (IQR = 6.44) years. Before diagnosis, the number of hospitalizations/year per patient was 0.86 (IQR = 2.28), the number of visit to emergency room/year per patient was 0.92 (IQR = 1.77), the number of doctor’s visits/year per patient was 15 (IQR = 11.25), whereas the school/work absence days per patient were reported in 52.72 (IQR = 56.35) days per year. After diagnosis, 20 patients (45.45%) received IVIG replacement therapy, and all of them presented a significant improvement (p <0.05) in all the mentioned variables. Characteristically, even when patients with PID received IVIG, there was still an important disease burden when comparing them against healthy controls. Complications secondary to PID were detected in 19 patients (43.18%). The reported overall mortality rate was 6.82% (n = 3).ConclusionsWe were able to indirectly estimate an important disease burden in patients with PID; which is considered to be preventable, at least in part, with effective interventions like health planning, research, collaboration with primary care providers, and generation of policies and practices, in order to improve the quality of life and care of families with PID.

show abstract

Primary Immunodeficiency Diseases in Aguascalientes, Mexico: Results from an Educational Program

Cited by 10 publications

References 18 publications

Evaluation of awareness about primary immunodeficiencies among physicians before and after implementation of the educational program: A longitudinal study

Evaluation of awareness about primary immunodeficiencies among physicians before and after implementation of the educational program: A longitudinal study

Clinical Manifestations in the Patients with Primary Immunodeficiencies: Data from One Regional Center

Disease burden for patients with primary immunodeficiency diseases identified at reference hospitals in Guanajuato, Mexico

Contact Info

Product

Resources

About