Iranian Journal of Pediatrics
Volume:31 Issue: 6, Dec 2021

COVID-19 results in an imbalance between procoagulant and anticoagulant homeostatic mechanisms that could be complicated with thrombotic events. In β-thalassemia patients, the presence of comorbidities, iron overload, adrenal hypofunction, splenectomy, and chronic hypercoagulable state might increase the susceptibility to COVID-19 and its severity.

 The search was conducted in PubMed, Web of Science, and Scopus databases for the key terms of β-thalassemia/thalassemia and COVID-19 until July 2021.

The survey of published observational studies (mostly multicenter and case reports) indicated a lower prevalence of COVID-19 in β-thalassemia patients compared with the general population, as well as mild to moderate COVID-19 in these patients, especially in those without comorbidity. β-Thalassemia children were susceptible to COVID-19 but with less severity compared to adults. There is no report of pulmonary embolism and thrombotic events in β-thalassemia patients with COVID-19; however, coagulation abnormality and pulmonary microembolism have been found in these patients.

Findings could be interpreted by the presence of high hemoglobin F (HbF) levels, the advantage of hydroxyurea (HU) therapy, splenectomy, and iron chelation therapy in these patients. However, due to the low sample size and studying mainly young patients, the results should be interpreted with caution, and it still needs more studies with a larger sample size to confirm these findings.

Morquio A, an autosomal recessive lysosomal storage disease, is caused by a defect in the enzyme N-acetyl-galactosamine-6-sulfatase. This leads to the accumulation of the glycosaminoglycans chondroitin-6-sulfate (C6S) and keratan sulfate (KS), resulting in various skeletal manifestations, multisystemic impairments, and significant morbidities.

This study aimed to evaluate the impact of the addition of elosulfase alfa to the hospital protocol on treating Iranian pediatricians with Morquio A syndrome.

A before and after study was conducted on ten patients with Morquio A syndrome diagnosis from 2019 to 2020. Elosulfase alfa was prescribed with the standard dose of 2 mg/kg/weekly IV infusion for 54 weeks. Then, growth indices, quality of life, and cardiopulmonary data were collected by research assistants using a pre-designed check. Data were entered in SPSS version 23. Quantitative variables were compared between the two periods using the Student's t-test, and qualitative variables were compared using the χ2 test or Fisher's exact test.

Ten pediatricians with MPS IV were included, seven of whom were female, with the mean age of 5.8 ± 2.3 years. The meantime for walking 6 m (P = 0.005), standing (P = 0.005), the stair climb test (P = 0.007), and quality of life (P = 0.015) had significant statistical difference before and after treatment by elosulfase alfa.

The addition of elosulfase alfa in patients with Morquio A syndrome was associated with a significant improvement outcome in the ‘after’ treatment period.

Respiratory severity score (RSS) is a simple, non-invasive respiratory failure assessment tool that its appropriateness for lung disease severity is well-established.

This study aimed to investigate the potential value of early-life RSS values in predicting mortality or severe bronchopulmonary dysplasia (BPD) in infants with extremely low birth weight (ELBW) undergoing invasive mechanical ventilation.

Following a retrospective approach, the current multi-center study intended to estimate the RSS and RSS/kg values in the first three days of life and time-weighted means of these values in ELBW babies who received invasive mechanical ventilation. Participants were categorized into two groups of survival without severe BPD and those with severe BPD or death. All RSS values and other risk factors were compared between groups. A multivariate logistic regression analysis was used to identify factors affecting severe BPD or death. The sensitivity and specificity of RSS values in predicting severe BPD or death were estimated using receiver operating characteristic (ROC) curves.

A total of 101 infants met the inclusion criteria. All RSS and RSS/kg values in the first three days of life were found to be significantly higher in the severe BPD or death group. The maximum area under the curve (AUC) in the ROC curves created for the respiratory severity score values was determined as RSS/kg mean with a cut-off value of 3.62 (85.3%), (P = 0.001). According to the multivariate logistic regression analysis, which included risk factors that may affect the development of severe BPD or mortality, both the mean RSS/kg score and the duration of invasive mechanical ventilation maintained statistically significant. [RSS/kg mean; OR = 2.28 (1.37 – 3.78), P = 0.001], [invasive MV duration; OR = 1.08 (95% CI, 1.03 – 1.14), P = 0.003].

This study demonstrated that high RSS/kg mean values in the early periods of life are valuable in predicting severe BPD or death in ELBW babies undergoing invasive mechanical ventilation. Furthermore, mean RSS had more predictive power than single RSS determinations, and incorporating body weight was associated with improved sensitivity of the score.

It is well-known that cigarette smoke contains chemical substances, including nicotine and carbon monoxide, which can have harmful effects on cardiovascular function.

This study aimed to investigate and compare lipid levels and carotid artery intima-media thickness (CAIMT) among neonates born to smoking and nonsmoking mothers.

This study was conducted on 40 term neonates (over 37 weeks of gestation) within the age range of 0 - 30 days born to smoking mothers (study group) and 34 term neonates born to nonsmoking mothers (control group). Both groups were assessed prospectively for triglyceride (TG), total cholesterol (TC), low-density lipoprotein cholesterol (LDL-C), high-density lipoprotein cholesterol (HDL-C), and CAIMT.

There was no difference in the mean age of mothers between control and study groups (29.0 ± 4.5 vs. 29.3 ± 5.7 years; P = 0.31). The CAIMT was observed as similar between the groups (0.35 ± 0.05 vs. 0.36 ± 0.05 mm in control and study groups, respectively; P = 0.665). Although HDL-C levels were significantly lower in the study group (48.5 ± 16.2 vs. 39.1 ± 17.8 mg/dL; P = 0.021), no difference was observed in LDL-C, TC, and TG levels between the two groups.

Based on the results, there were no differences in CAIMT and lipid profile other than low HDL levels between term neonates born to smoking mothers and neonates born to nonsmoking mothers. Atherosclerosis is a disease in which numerous factors play a role in the formation beginning in the fetal period and emerging with clinical findings in advanced age; therefore, it is required to perform further studies with longer follow-up and larger sample size to confirm the present study findings.

Sepsis still causes morbidity and mortality in children admitted to the pediatric intensive care unit (PICU). Sepsis induces myocardial dysfunction and causes a reversible decline in ejection fraction (EF) of ventricles. Many biomarkers have been described for diagnosing sepsis, including serum ferritin and C-reactive protein (CRP).

This study was conducted to assess the relationship of cardiac dysfunction evaluated using echocardiogram, ferritin, and CRP with negative outcomes of sepsis in the PICU.

A cross-sectional study was conducted on 80 patients aged between one month and six years who fulfilled the following criteria: (1) confirmed diagnosis of sepsis according to the American College of Critical Care Medicine; (2) receiving ventilation for 48 h and/or vasoactive medicines. The CRP and ferritin levels were recorded on the first day (D1) and third day (D3) of hospitalization in the PICU. Participants underwent an echocardiography study to investigate the ejection fraction on D1 and D3. All outcomes were evaluated.

Our results showed a highly statistically significant difference between D1 and D3 in ejection fraction (P = 0.001). The serum ferritin level and CRP enhanced significantly from D1 to D3 (P < 0.001). Low left ventricular ejection fraction, and high serum ferritin were associated with unfavorable outcomes (P values < 0.001 and 0.021, respectively), but there was no significant difference in the outcomes regarding CRP.

Cardiac dysfunction and high serum ferritin were associated with unfavorable outcomes in children with sepsis admitted to the PICU.

Biological inflammatory status in cancer patients is evaluated with various parameters. Limited studies have been conducted on this issue in children.

This study was done to evaluate the importance of the biological inflammatory status, including systemic inflammatory response index (SIRI), systemic immune-inflammation (SII) index, and hemoglobin-albumin-lymphocytes-platelets (HALP) score in children with cancer.

Demographic characteristics, complete blood count, and biochemical analysis of the patients on admission were recorded retrospectively. Since not meeting the necessary assumptions, the Mann-Whitney U test was used for the comparison of the two groups, and the Kruskal-Wallis test was used for the comparison of more than two groups

The SIRI of the patients was statistically higher than the control group (P < 0.0001). Considering lymphoma, central nervous system tumors, and solid tumors, there was a statistical difference between the groups in SII index and SIRI (P values: .0245, and .0060, respectively). Regarding inflammatory biomarkers, according to the extent of cancer (localized or advanced disease), it was found that SIRI was higher in patients with advanced disease (P = 0.0175). The patients who died had a statistically higher HALP score (P = 0.0472).

The inflammatory biomarkers can be used in childhood cancers to determine the extent of the disease and predict outcomes. However, in larger patient series, ideal values should be achieved by analyzing the receiver operating characteristic curves and the area under the curve.

The Coronavirus disease 2019 (COVID-19) pandemic has posed a severe threat to international health and the economy. Clinicians, the main staff involved in fighting against the pandemic, are under great pressure. However, relevant mental stress studies are lacking at present.

This study aimed to explore the mental stress level and its influencing factors among Chinese pediatricians under the outbreak of COVID-19, aiming to provide a certain theoretical basis for relevant psychological intervention among medical staff.

In this cross-sectional study, 352 in-service pediatricians were selected from nine hospitals in Jiangsu province, China, in February 2020. The online survey was performed to collect general information. Meanwhile, the Perceived Stress Scale (PSS-10), Self-rating Anxiety Scale (SAS), and Pittsburgh Sleep Quality Index (PSQI) scale were employed for assessment. Afterward, the stress level and influencing factors among pediatricians were analyzed through descriptive analysis, one-way analysis of variance (ANOVA), correlation analysis, and multiple linear regression analysis.

The mean score of PSS-10 showed moderate stress on the whole. Pediatricians from outpatient and emergency departments had significantly higher perceived stress than those from other departments. The perceived stress was significantly associated with age, educational level, professional title, work experience, and physical condition. Stepwise multiple linear regression showed that age, professional title, and physical condition had a linear relationship with the individual’s perceived stress. Besides, Pearson correlation analysis indicated that perceived stress was associated with anxiety level and sleep quality.

Under the outbreak of COVID-19, pediatricians suffer from relatively high levels of mental stress. The influencing factors include education, age, professional title, work experience, and physical condition. Typically, the anxiety level and sleep quality are correlated with the mental stress among pediatricians.

Duodenal ulcer perforations (DUP) are missed in the differential diagnosis of acute abdomen because they are less common in children than in adults. Delay in diagnosis may cause morbidity or even mortality. It was aimed to raise awareness about DUP in adolescent by comparing the data of adolescent cases treated in our clinic with the adult cases' data in the literature.

We reviewed the clinical characteristics of nine male patients with DUP, ages between 14 and 17 years, admitted to our clinic between January 2007 and June 2020 retrospectively. Literature data on DUP in adults were reviewed.

The obtained data were compared with the data of adult patients in the literature.

Patients were reported to have symptoms such as abdominal pain and vomiting that lasted for 1-30 days on average in 8 patients, and nonsteroidal anti-inflamatory drugs were used all patients except 2 patients. There was diffuse tenderness at the abdomen in all of the remaining patients and in 7 patients intraabdominal free air was observed. Perforation was repaired with omentoplasty in all patients. Unlike the adult population, DUP adolescents are more related to NSAID use rather than Helicobacter pylori infection and complicated surgical techniques were not required because the cases were generally not complicated.

Although it is rarely seen in adolescents and shows certain differences compared to adult patients, the anamnesis and physical examination of the patients should direct the physicians to the DUP. Differences from adult population should be considered in diagnosis and treatment.

The current study was done to identify key genes associated with Kawasaki disease (KD).

Three datasets were collected from Gene Expression Omnibus (GEO) database. Then, differentially expressed genes (DEGs) analysis, gene ontology (GO) annotation, and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway enrichment were performed. Quantitative real-time polymerase chain reaction (qRT-PCR) was used to determine the expression levels of DEGs in KD. Receiver operating characteristic (ROC) analysis was performed to assess the diagnostic value of DEGs.

In total, 2923 DEGs (1239 up- and 1684 down-regulated genes) were detected in KD. Ribosome, Leishmaniasis, and Tuberculosis significantly enriched KEGG pathways of DEGs. Six DEGs, including ADM, S100A12, ZNF438, MYD88, FCGR2A, and FCGR3B, were selected for qRT-PCR validation. Except for MYD88, the qRT-PCR results displayed similar expression patterns with that in our integrated analysis. ROC analysis revealed the diagnostic value of the six DEGs.

Our study was expected to provide clues toward understanding the pathophysiology of KD inflammation.