Assessment of quality of life among parents of children with congenital heart disease using WHOQOL-BREF: a cross-sectional study from Northwest Saudi Arabia.

BACKGROUND AND AIMS: Health-related quality of life (HRQOL) has garnered increasing interest especially for health care providers and researchers. The study aims to evaluate the HRQOL in parents of congenital heart disease (CHD) children, and to clarify the effect of the disease severity on the outcome of the HRQOL perception. Also, to analyze the internal consistency of the Arabic version of the World Health Organization (WHO) QOL-BREEF tool in order to determine whether the tool had good validity for the target population. METHODS: A cross-sectional study. The HRQOL perception was evaluated using WHOQOL-BREF questionnaire, and the internal consistency of the tool was tested using Cronbach's alpha (α-C), RESULTS: The study sample consisted of 200 individuals, 120 parents of CHD children, compared to 80 parents of children with minor illnesses (mean age of participating parents = 35.1 ± 9.8 years). While evaluating the HRQOL, the group of parents of children with minor illnesses had higher scores than the total group of parents of CHD children in all domains, indicating a better HRQOL. Class-IV subgroup of parents of CHD children showed the most significant lower total score of domains between all classes (44.47 ± 12, p < 0.001). With respect to the internal consistency of the WHOQOL-BREF, estimation of α-C values were 0.84 points for the group of parents of CHD children, and 0.87 for the group of parents of children with minor illnesses. CONCLUSIONS: This short-term study emphasized that, HRQOL scores among parents of CHD children are compromised, and the severity of their children illness significantly affect the total score of domains in their HRQOL perception. Furthermore, the tool showed to be practical and efficient to evaluate the QOL of parents of CHD children in our population in future researches.

Cytokine Profiling among Children with Multisystem Inflammatory Syndrome versus Simple COVID-19 Infection: A Study from Northwest Saudi Arabia.

BACKGROUND: Multisystem Inflammatory Syndrome in Children (MIS-C) is a novel syndrome associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection with varying clinical features. This study aimed to analyze the expression profiles of cytokines in blood, report the important clinical characteristics, and correlate these with the short- and mid-term outcomes. METHODS: This cross-sectional study was conducted on hospitalized children with MIS-C from March 2021 to May 2022. Phenotypes were classified into two groups (A,B) according to the severity of the disease and the need for invasive respiratory support. Clinical features, laboratory parameters, and outcomes were reported. RESULTS: We identified 60 children with MIS-C (mean age of 7.4 ± 3.8 years) compared to 30 age- and sex-matched controls with simple COVID-19. The clinical manifestations of MIS-C patients were fever (100%), respiratory (83.3%), GIT (80%), and conjunctivitis (80%). Twenty-seven MIS-C children (45%) required PICU admission due to shock and needed mechanical ventilation. Anemia, lymphopenia, and elevated levels of inflammatory and tissue injury markers were observed in the MIS-C groups (mainly B). High cytokine levels (IL-1ß, IL-6, IFN-α, GM-CSF, and HMGB1) were observed acutely in the MIS-C children, and a persistent elevation of some cytokines were reported at midterm follow-up, especially in Group B. CONCLUSION: Robust inflammatory response to COVID-19 disease with elevated IL-1ß, IL-6, and GM-CSF levels might explain the severity and outcome of the clinical syndrome.

Stent or Shunt, What Could be Better for Children with Duct Dependent Pulmonary Circulation?

BACKGROUND: Systemic to pulmonary shunt (Shunt) is offered for children with duct dependent pulmonary circulation to augment pulmonary flow. Recently patent ductus arteriosus (PDA) stent (Stent) is widely used as an alternative method. We aimed to compare post intervention outcomes in children underwent either procedure. METHODS: Infants under 3 months who had an initial palliation by Shunt or Stent were retrospectively reviewed between 2008 and 2016, then followed till the second intervention or 1 year whichever earlier. RESULTS: 187 patients (110 Shunt and 77 Stent) were included. Initial weight and pulmonary artery (PA) branches size were similar between the groups. Shunt patients had more shock preoperatively and required more emergency intervention. Stent group showed less ICU stay 4 (1-8) vs 13 (7-23) days, p < 0.0001 and less positive pressure ventilation days 1 (0-2) vs 5.5 (3-11), p < 0.0001. However, Stent group had more symptomatic arterial and deep venous thromboses. In Stent patients the branch PAs growth was better and more homogeneous. At follow-up, no difference between groups regarding cumulative readmission days to hospital, hemoglobin levels and the weight percentile for age. Mortality was not different with a tendency to be higher in the Shunt group (13%) compared to the Stent group (5%), p 0.1. CONCLUSIONS: The implantation of PDA stent in patients with duct dependent pulmonary circulation results in a smoother ICU course and a shorter hospital stay, with higher risk of vascular injury. Shunt and Stent procedures have a good outcome for PA growth, somatic growth and survival.

Esophageal lung with rare associated vascular and anorectal malformations.

Esophageal lung is a rare communicating bronchopulmonary foregut malformation in which the main bronchus arising from the trachea is absent. The affected lung is usually hypoplastic and aerated via an anomalous airway originating from the esophagus. Other anomalies such as esophageal atresia with tracheoesophageal fistula or VACTERL (vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities) association can co-exist. The initial radiographic findings may be normal, but subsequent imaging usually shows progressive and recurrent lung collapse, probably because of recurrent aspiration through the anomalous airway and poor compliance of the affected lung during breathing. In this report, we describe a neonate with esophageal lung and rare associated anomalies, including anorectal malformation, pulmonary artery sling, and inferior vena cava interruption with azygous continuation. To our knowledge, this is the first report of esophageal lung with such associations.