Thromboprophylaxis for Coagulopathy Related to COVID-19 in Pediatrics: A Narrative Review.

In addition to harming the respiratory system, COVID-19 can affect multiple organs. Children may develop a specific complication of COVID-19 called multisystem inflammatory syndrome in children (MIS-C) which could influence the vascular system of children and cause multiple coagulopathies in the body. Information on the use of thromboprophylaxis in this condition was collected via the review of various articles. In general, different factors in immune system responses can trigger the initiation of thrombotic events. Studies have shown that starting anticoagulant prophylaxis, which contributes to decreased thrombotic events, is dependent on the patient's condition and D-dimer levels. However, further studies on pediatric populations are needed to establish the role of anticoagulants in children with this condition.

Six-minute walk test and factors affecting exercise capacity in children with cystic fibrosis.

PURPOSE: This study aimed to compare the result of the six-minute walk test (6MWT) in patients with cystic fibrosis (CF) aged < 20 years old and individuals without CF. METHODS: In this cross-sectional study, 50 children and adolescents with CF and 20 children and adolescents without CF underwent the 6MWT. Vital signs before and immediately after the 6MWT and six-minute walk distance (6MWD) were evaluated. RESULTS: The mean change in heart rate, percentage of peripheral oxygen saturation (SpO2%), systolic blood pressure, respiratory rate, and dyspnea severity during the 6MWT was significantly higher in patients with CF. In the case group, 6MWD was associated with regular chest physical therapy (CPT) and forced expiratory volume (FEV)> 80%. Patients with CF receiving regular CPT or mechanical vibration and with FEV in the first second > 80% showed better physical capacity during the 6MWT (smaller Sp02% decline and lower dyspnea perception). CONCLUSION: Children and adolescents with CF have lower physical capacity compared to individuals without CF. CPT and mechanical vibration could be used to increase physical capacity in this population.

Economic Burden of Asthma in Northwest Iran.

Background: The economic burden of asthma is a major public health concern. This study estimates the economic burden of asthma in Northwest of Iran. Methods: A longitudinal study was conducted between 2017 and 2018 in Tabriz (Iran) using the Persian version of the Work Productivity and Activity Impairment (WPAI) questionnaire. Direct and indirect costs associated with asthma were estimated based on the societal perspective, prevalence-based approach, and bottom-up method. Annual indirect costs were estimated using the human capital (HC) method. The structural equation model was used to evaluate the relationship between costs, sex, and asthma severity. Results: A total of 621 patients with asthma were enrolled in the study. Significant differences were found between female and male patients for the mean cost of radiology (P=0.006), laboratory (P=0.028), and diagnostic (P=0.017) tests at baseline, and for laboratory (P=0.012), and diagnostic (P=0.027) tests at one-year follow-up. The more severe asthma, the more significant the costs for annual physician office visits (P=0.040) and medications (P=0.013). As asthma severity increased, significantly higher expenditures were observed in women for days lost from work at baseline (P=0.009) and one-year follow-up (P=0.001), and in men for productivity loss at work due to impairment at baseline (P=0.045). A significant association between indirect costs and the cost of impairment-related lost productivity at work (ß=3.29, P<0.001), and between severe asthma and indirect costs (ß=32.36, P<0.001) was observed. Conclusion: High costs are incurred by Iranian asthma patients, especially because of impairment-related productivity loss at work associated with asthma exacerbation.

DOCK8-related Immunodeficiency Syndrome (DIDS): Report of Novel Mutations in Iranian Patients.

DOCK8 immunodeficiency syndrome (DIDS) is a rare autosomal recessive (AR) disorder characterized by elevated serum IgE levels, eosinophilia, recurrent cutaneous infections, severe eczema, and sinopulmonary and gastrointestinal infections. This syndrome is a multisystem disease that is associated with both immune deficiency and neurological complications. In this study, we describe the clinical characteristics of two Iranian patients with DOCK8 deficiency and propose possible mechanisms for this condition. By using whole exome sequencing (WES), we identified two novel mutations, namely c.3233_3234del AG (p.Q1078fs) in exon 6 and a large deletion with 94 kb (c.405-3231 deletion, p.K135fs), in these two patients. These variations are confirmed with Sanger sequencing and CGH array. Subsequent co-segregation analysis is performed to identify inheritance patterns. Both patients were homozygote and their parents were heterozygote for the variations. For further investigation, prediction tools were applied to identify the pathogenicity of the variations and also for modeling the truncated proteins. The patients did not show neurological abnormalities associated with a deficiency of the N terminal region of DOCK8. The absence of neurological complications in the first patient is justifiable due to the maintenance of the proline-rich region in DOCK8, but for the second patient with expanded deletion which is almost like null DOCK8 protein, it is not presumable, pointing to the fact that the C terminal region of the protein might have functions in the proliferation and migration neurons in the peripheral nervous system. Alternatively, it is possible that neurological abnormalities follow an age-dependent pattern, leading to the appearance of related symptoms later in life. Further multiple functional studies are needed to model different identified variants in animal models to confirm our results and suggest possible mechanisms associated with DOCK8 deficiency in this study.

New insight into clinical heterogeneity and inheritance diversity of FBLN5-related cutis laxa.

BACKGROUND: FBLN5-related cutis laxa (CL) is a rare disorder that involves elastic fiber-enriched tissues and is characterized by lax skin and variable systemic involvement such as pulmonary emphysema, arterial involvement, inguinal hernias, hollow viscus diverticula and pyloric stenosis. This type of CL follows mostly autosomal recessive (AR) and less commonly autosomal dominant patterns of inheritance. RESULTS: In this study, we detected a novel homozygous missense variant in exon 6 of FBLN5 gene (c.G544C, p.A182P) by using whole exome sequencing in a consanguineous Iranian family with two affected members. Our twin patients showed some of the clinical manifestation of FBLN5-related CL but they did not present pulmonary complications, gastrointestinal and genitourinary abnormalities. The notable thing about this monozygotic twin sisters is that only one of them showed ventricular septal defect, suggesting that this type of CL has intrafamilial variability. Co-segregation analysis showed the patients' parents and relatives were heterozygous for detected variation suggesting AR form of the CL. In silico prediction tools showed that this mutation is pathogenic and 3D modeling of the normal and mutant protein revealed relative structural alteration of fibulin-5 suggesting that the A182P can contribute to the CL phenotype via the combined effect of lack of protein function and partly misfolding-associated toxicity. CONCLUSION: We underlined the probable roles and functions of the involved domain of fibulin-5 and proposed some possible mechanisms involved in AR form of FBLN5-related CL. However, further functional studies and subsequent clinical and molecular investigations are needed to confirm our findings.

Validation of the Persian work productivity and activity impairment questionnaire in asthmatic patients.

BACKGROUND: The work productivity and activity impairment (WPAI) questionnaire is a fine linguistic validated tool to measure work productivity and activity impairment. Considering its capability, this study aimed to evaluate the validity of the Persian version of WPAI-AQ in asthmatics. METHODS: The standard forward-backward process was used to translate the English version of WPAI-AQ into Persian. The convergent validity and responsiveness were evaluated by analyzing the correlations between the Persian WPAI-AQ and the health outcomes, and its longitudinal change score with the change in SGRQ score, respectively. Additionally, the stability was estimated according to test-retest scores. RESULTS: There was a significant correlation between the Persian WPAI-AQ related outcomes and symptoms, activities, and impacts of disease (r = 0.41-0.89, p < 0.04). Desirable stability was observed by the test-retest analysis; 0.90 (95%CI: 0.89-0.95) for overall impairment, 0.90 (95%CI: 0.86-0.93) for work time missed, 0.72 (95%CI: 0.54-0.83) for activity impairment; 0.79 (95%CI: 0.71-0.86) for student class time missed, and 0.76 (95%CI: 0.66-0.81) for school impairment. Response to the change scores strongly supported the longitudinal responsiveness of the Persian WPAI-AQ (r = 0.37 to 0.63, p < 0.05). CONCLUSION: The Persian WPAI-AQ is a feasible valid tool to estimate work productivity and activity impairment in Persian-speaking asthmatic patients.

The Emergency Severity Index, version 4, for pediatric triage: a reliability study in Tabriz Children's Hospital, Tabriz, Iran.

BACKGROUND: The Emergency Severity Index (ESI) has earned reliability and validity in adult populations but has not been adequately evaluated in pediatric patients. The aim of this study was to assess the reliability of the ESI version 4 and inter-rater reliability measures to evaluate the performance of nurses in the emergency ward. METHODS: Raters were part of the same team of pediatric emergency medicine team, including pediatric emergency medicine (PEM) physicians and pediatric triage (PT) nurses. Reliability and agreement rates were measured using kappa statistics. The measurements were compared with the admission rates, readmissions to the PEM division, location of admission and death as outcomes. RESULTS: Initially, PT nurses rated 20 case scenarios. Further in a prospective cohort study, 1104 children were assigned ESI scores by both nurses and physicians. The ratings of case scenarios showed a kappa value of 0.84. In actual patients, ratings showed high concordance with the physicians' ratings with the kappa value of 0.82 being in a good agreement with the nurses' ratings. The main area of discordance was detected in level 4 where 48 cases were triaged in higher levels and 25 were triaged in lower levels. The analysis showed the likelihood of admission clearly increased as the ESI score decreased (p<0.0001). There was a significant correlation between the admission status and triage level in both PT nurses' and PEM physicians' ratings (Spearman coefficient=0.374, 0.407; p<0.0001). CONCLUSION: ESI scores assigned to the pediatric patients are reliable in the hands of experienced PT nurses and PEM physicians. The very good agreement between PT nurses and PEM physicians, demonstrated in this study, is essential in cooperative work in crowded referral emergency departments and helpful in challenging triage cases.

Nasopharyngeal bleeding due to leech bites in a 9-month-old infant.

Leeches are annelids, comprising the subclass Hirudinea. There are freshwater, terrestrial and marine leeches. Haemophagic leeches attach to their hosts and remain there until they become filled up with blood and then they fall off to digest. Leech bites can manifest various symptoms, which lead to anemia. Here is reported, a case of leech bites in a 9-month-old infant, with a long history of hematemesis, melena, epistaxis and anemia which demanded repeated transfusions. So, leech bites may be a differential diagnosis of anemia particularly in the endemic rural areas where villagers use non boiled water.

Relationship between endoscopic nodular gastritis and Helicobacter pylori infection in children.

INTRODUCTION: The specificity of relationship of endoscopic evidence of nodular gastritis with Helicobacter pylori infection is unclear. AIM: To assess the relationship of endoscopic nodular gastritis and H. pylori infection among children. METHODS: 124 children (median age 8.2 years, range 1-15) undergoing upper GI endoscopy for abdominal pain underwent urease test and histological examination of gastric mucosa to determine the presence and density of H. pylori infection, and presence and severity of gastritis. RESULTS: H. pylori infection was detected in 57 (46%) children. Endoscopic nodular gastritis was present in 46 of these 57 patients (81%) and in 24 of 67 (36%) H. pylori-negative patients (36%). The frequency of endoscopic nodular gastritis was related to increasing age (p< 0.0001), presence of H. pylori, grade of histologic gastritis, and H. pylori density (p< 0.0001). CONCLUSION: Endoscopic finding of nodular gastritis is associated with presence of H. pylori infection and active chronic gastritis in children.