Evaluation of QT Dispersion in Children with Breath Holding Spells

How to Cite This Article: Movahedian AH, Heidarzadeh Arani M, Motaharizad M, Mousavi GhA, Mosayebi Z. Evaluation of QT Dispersion in Children with Breath Holding Spells. Iran J Child Neurol. Winter 2016; 10(1):25-30.AbstractObjectiveBreath holding spells (BHS) are common involuntary reflexes in infancy and early childhood. Differential diagnosis should embrace Long QT Syndrome (LQTS) and paroxysmal abnormalities of rhythm. The aim of this study was to compare QT dispersion (QTd) in children with breath holding spells and normal controls.Materials & MethodsQT dispersion and Corrected QT(QTc) dispersion were measured in 12 lead surface electrocardiograms in 56 patients with BHS and compared with healthy children of the same age referred to the clinic for regular checkup visits.Results The most common type of BHS was cyanotic (83.9%). Seven patients (12.5%) had pallid and two patients (3.5%) had mixed spells. There was a history of breath holding spells in 33.9% of the children. QT dispersion was 61.6± 22.5 and 47.1±18.8 ms in patient and control groups, respectively. QTc dispersion (QTcd) was 104 ± 29.6 and 71.9 ±18.2 ms, respectively. There was a significant difference between patient and control groups in terms of QTd and QTcd (P<0.001).ConclusionQTd and QTcd were increased in children with BHS. Therefore, the evaluation of EKG for early diagnosis of rhythm abnormalities seems reasonable in these children

Atypical Ataxia Presentation in Variant Ataxia Telangiectasia: Iranian Case-Series and Review of the Literature

Ataxia-telangiectasia (AT) is a rare autosomal recessive neurodegenerative multisystem disorder. A minority of AT patients can present late-onset atypical presentations due to unknown mechanisms. The demographic, clinical, immunological and genetic data were collected by direct interview and examining the Iranian AT patients with late-onset manifestations. We also conducted a systematic literature review for reported atypical AT patients. We identified three Iranian AT patients (3/249, 1.2% of total registry) with later age at ataxia onset and slower neurologic progression despite elevated alpha-fetoprotein levels, history of respiratory infections, and immunological features of the syndrome. Of note, all patients developed autoimmunity in which a decrease of naïve T cells and regulatory T cells were observed. The literature searches also summarized data from 73 variant AT patients with atypical presentation indicating biallelic mild mutations mainly lead to an atypical phenotype with an increased risk of cancer. Variant AT patients present with milder phenotype or atypical form of classical symptoms causing under- or mis- diagnosis. Although missense mutations are more frequent, an atypical presentation can be associated with deleterious mutations due to unknown modifying factors

Comparison of the Results of Open Oral Food Challenge Test in Allergic Children with Positive ‎Skin Prick Test to Cow\'s Milk, Egg and Wheat

Background and Aim: Considering the need to perform a food challenge test in suspected food allergy cases, the purpose of this study was to investigate the results of the open oral food challenge (OFC) test in children with allergies and compare its results with their skin prick test (SPT). Materials and Methods: In this descriptive, cross-sectional, retrospective study, we reviewed the records of 63 allergic children with a positive SPT to cow milk, egg, or wheat, who had referred to the allergy and immunology subspecialty clinic in Kashan, Iran. The patients with positive SPT for the studied allergens, received complete allergen restriction diet for 3 months. After 3 months, open OFC test was performed according to the protocol. Data were analyzed using descriptive statistics and inferential statistics (Chi-square test to investigate the relationship between the response type in OFC and qualitative variables). Results: The mean age of the children was 6.46±3.14 years and more than half of them were girls. Most children were allergic to cow milk (54%). 25.4% of allergic children responded negatively to the open OFC test. 26.5%, 29.4%, and 16.7% of the children who were allergic to cow milk, egg, or wheat, respectively, responded negatively to the open OFC test, and these differences were not statistically significant. In all three food allergen groups, skin-mucosal symptoms were the most common finding which was indicative of a positive response to the open OFC test. Conclusion: The open OFC test results in children with a positive SPT to cow milk, egg or wheat indicated a relatively high failure rate of the test in the children. We recommend attention to food sensitivities in the evaluation of these patients. After confirmation of food sensitivities by existing methods; it is necessary to prevent consumption of these allergens and observe safety tips when dealing with these allergen

Implementation and effectiveness of continuous kangaroo mother care : a participatory action research protocol

BACKGROUND : The efficacy of continuous kangaroo mother care (C-KMC) in reducing neonatal mortality and morbidity among low birthweight and premature infants has been confirmed. Despite the recommendations of the World Health Organization, UNICEF, and the Ministry of Health of Iran to use C-KMC for eligible hospitalized neonates, this type of care is not performed due to implementation problems. This protocol aims to describe the design, implementation, and assessment of C-KMC in one tertiary hospital by means of participatory action research. METHODS : The objective of this study is to design and implement a C-KMC program for neonates that will be performed in two phases using a stages-of-change model. The first phase will be conducted in three consecutive activities of designing, implementing, and assessing the introduction of C-KMC. The second phase of the study has a before-and-after design to assess the effectiveness of C-KMC by comparing the length of preterm neonates’ stay in hospital and exclusive breastfeeding at discharge before and after implementing C-KMC. DISCUSSION : KMC is an important component of neonatal developmental care as part of family-centered care. Applying this type of care requires creating appropriate strategies, budget allocation, and clear and coordinated planning at different levels of the health system. The stages-of-change model is one of the appropriate approaches to the implementation of C-KMC.Tabriz University of Medical Scienceshttp://internationalbreastfeedingjournal.biomedcentral.comam2022Obstetrics and Gynaecolog

Financial challenges in the family physician programme in Iran: A systematic review of qualitative research

Introduction: The family physician programme (FPP) was implemented nearly two decades ago as a major health reform. Since the health system and FPP function in a rapidly changing social and economic environment, successful expansion of the programme requires a detailed analysis of its multiple major challenges, including the crucial aspect of its funding system. This systematic review aimed to assess the challenges in the FPP relative to its financing. Methods: All published articles related to the FPP in Iran were included in this study. In particular, original qualitative studies published in English or Persian from 2011 to 2021 were included. In January 2022, international credible scholarly databases and Persian databases were searched. All selected articles were carefully studied, and the data were extracted using the sample, phenomenon of interest, design, evaluation and research type technique. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses were used in preparing the study report. Results: Among 491 articles retrieved from the search strategy, 50 met the inclusion criteria after their titles and abstracts were screened. Twenty-nine studies were excluded after their full texts were reviewed. A total of 11 eligible empirical studies were finally included. Based on the results, six broad categories (budget and funding, insurance system, tariffs, payments, accountability and injustice) were identified as financial challenges. Conclusion: This study identified the challenges associated with financing among family physicians, and the results could provide guidance for policy-making in the expansion of the FPP

Comparison of Common Monogenic Defects in a Large Predominantly Antibody Deficiency Cohort

Background: Predominantly antibody deficiencies (PADs) are the most common primary immunodeficiencies, characterized by hypogammaglobulinemia and inability to generate effective antibody responses. Objective: We intended to report most common monogenic PADs and to investigate how patients with PAD who were primarily diagnosed as suffering from agammaglobulinemia, hyper-IgM (HIgM) syndrome, and common variable immunodeficiency (CVID) have different clinical and immunological findings. Methods: Stepwise next-generation sequencing and Sanger sequencing were performed for confirmation of the mutations in the patients clinically diagnosed as suffering from agammaglobulinemia, HIgM syndrome, and CVID. Results: Among 550 registered patients, the predominant genetic defects associated with agammaglobulinemia (48 Bruton's tyrosine kinase [BTK] and 6 μ heavy chain deficiencies), HIgM syndrome (21 CD40 ligand and 7 activation-induced cytidine deaminase deficiencies), and CVID (17 lipopolysaccharides-responsive beige-like anchor deficiency and 12 atypical Immunodeficiency, Centromeric instability, and Facial dysmorphism syndromes) were identified. Clinical disease severity was significantly higher in patients with μ heavy chain and CD40 ligand mutations compared with patients with BTK (P = .003) and activation-induced cytidine deaminase (P = .009) mutations. Paralysis following live polio vaccination was considerably higher in patients with μ heavy chain deficiency compared with BTK deficiency (P < .001). We found a genotype-phenotype correlation among patients with BTK mutations regarding clinical manifestation of meningitis and chronic diarrhea. Surprisingly, we noticed that first presentations in most patients with Immunodeficiency, Centromeric instability, and Facial dysmorphism were respiratory complications (P = .008), whereas first presentations in patients with lipopolysaccharides-responsive beige-like anchor deficiency were nonrespiratory complications (P = .008). Conclusions: This study highlights similarities and differences in the clinical and genetic spectrum of the most common PAD-associated gene defects. This comprehensive comparison will facilitate clinical decision making, and improve prognosis and targeted treatment

Comparison of Common Monogenic Defects in a Large Predominantly Antibody Deficiency Cohort

Predominantly antibody deficiencies (PADs) are the most common primary immunodeficiencies, characterized by hypogammaglobulinemia and inability to generate effective antibody responses