The impact of combining educational program with the improving of infrastructure to diagnose on early detection of primary immunodeficiencies in children

Boyarchuk, Oksana; Volokha, Alla; Hariyan, Tetyana; Kinash, M. I.; Volianska, L.A.; Birchenko, Inna; Habor, H. H.; Korda, Mychaylo

doi:10.1007/s12026-019-09103-w

Cited by 16 publications

(12 citation statements)

References 14 publications

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“…[14] Since there is currently no neonatal screening for PIDs in Ukraine, defining of these symptoms is very important for a timely diagnosis of these diseases. [18] This study was limited to pool of patients followed up in one regional center.…”

Section: Discussionmentioning

confidence: 99%

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

Boyarchuk¹,

Dmytrash²

2019

Turk J Immunol

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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.

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Section: Discussionmentioning

confidence: 99%

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

Boyarchuk¹,

Dmytrash²

2019

Turk J Immunol

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“…Therefore, we aimed to improve the knowledge of warning and specific signs of PIDs. Particular attention was paid to the diagnosis of Nijmegen breakage syndrome and Di George syndrome, which are the most common in Ternopil region [12][13][14]. In this study, we see a significant increase in the percentage of correct answers (by 27.5%) in this block of questions.…”

Section: Discussionmentioning

confidence: 50%

“…Our previous study has shown that implementation of an educational program resulted in the increase of the number of detected PIDs immediately afterwards almost threefold, and compared to the two years prior to the awareness campaign (2015-2016) almost fivefold (2 cases per year before versus 9.5 cases per year during the awareness campaign) [14]. The number of children with suspected PIDs referred by pediatric sub specialists has almost doubled in the second year of the program (41 vs. 22) [14]. This result is consistent with those of previous studies measuring the impact of physician education and public awareness campaigns in Jeffrey Modell Centers Network of 39 countries and 120 cities worldwide [7].…”

Section: Plos Onementioning

confidence: 94%

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

et al. 2020

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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.

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“…Physician education can be an effective tool for increasing awareness about Lyme disease and the potential outcomes of treatment [19,20]. The improvement of doctors' knowledge about JHR will help to enhance alertness and to avoid unnecessary prescriptions and concerns.…”

Section: Discussionmentioning

confidence: 99%

The Jarisch-Herxheimer reaction associated with doxycycline in a patient with Lyme arthritis

Nykytyuk¹,

Boyarchuk²,

Klymnyuk³

et al. 2020

Reumatologia

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Objectives: The objective of this article was to present the clinical peculiarity of the Jarisch-Herxheimer reaction (JHR) during antibiotic treatment of Lyme arthritis. Material and methods: Case study presentation as a basis for discussion, literature search of PubMed database particularly in the subject of combination of the JHR and Lyme borreliosis using the combination of words "Jarisch-Herxheimer reaction" and "Lyme borreliosis", "Lyme arthritis", "antibiotic treatment", "generalized inflammation", "cytokine production", "children", "increasing awareness"; discussion of the problem based on a clinical case and cited articles. Results: The authors present a case of Lyme arthritis in a 13-year-old boy, as well as a discussion of clinical features of this complication of Lyme disease treatment as the Jarisch-Herxheimer reaction. On the 7 th day of doxycycline treatment the patient's condition deteriorated: a low-grade fever occurred, and severe arthralgias with intense hip, ankle and cervical spine pain and myalgias developed. Erythrocyte sedimentation rate and C-reactive protein were elevated. The Jarisch-Herxheimer reaction was diagnosed. This complication was characterized by severity, long duration and marked signs of inflammation. Conclusions: In the reviewed literature the Jarisch-Herxheimer reaction in patients with Lyme borreliosis is described more often in adults, with mild course and short duration of the disease. Based on the presented clinical picture this severe complication may also be associated with long duration and marked signs of inflammation. The authors suggest that informing health professionals about occurrence of the Jarisch-Herxheimer reaction should help physicians to distinguish it from allergic reactions or other conditions and improve treatment outcomes.

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The impact of combining educational program with the improving of infrastructure to diagnose on early detection of primary immunodeficiencies in children

Cited by 16 publications

References 14 publications

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

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

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

The Jarisch-Herxheimer reaction associated with doxycycline in a patient with Lyme arthritis

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