Evaluation of the safety profile and therapeutic efficacy of remdesivir in children with SARS-CoV-2 infection - a single-center, retrospective, cohort study.

Despite 3.5 years of the SARS-CoV-2 pandemic, we still lack effective drugs against COVID-19. The first and most widely used drug, remdesivir, has not yet been shown to be effective in adults. Even less is known about its effectiveness in children. Therefore, the aim of this retrospective study was to evaluate the safety and efficacy of remdesivir in pediatric patients with COVID-19 hospitalized in one medical center. The medical records of 328 children with COVID-19 were analyzed. Analysis was performed on the subgroups of children treated and not treated with remdesivir. Clinical data on general health, course of COVID-19 and treatment received were analyzed. Remdesivir was administered to 64 children, 16 to treat severe or critical illness and 48 because of the presence of risk factors to prevent progression to severe COVID-19. In children with severe COVID-19, remdesivir did not reduce the mortality rate. However, in patients with milder disease and risk factors, the drug significantly reduced the risk of progression to severe disease. Among adverse events, only mild aminotransferase elevations were observed in 4 patients, but none of these required discontinuation of treatment. CONCLUSIONS: Remdesivir is a safe treatment option for children with COVID-19. However, the efficacy of this therapy is still uncertain. It appears that in children with asymptomatic to moderate COVID-19 and risk factors for severe disease, remdesivir could be an effective method of prophylaxis. However, its efficacy in controlling severe disease is questionable and requires further study. WHAT IS KNOWN: • There are still no effective drugs to combat COVID-19, and the efficacy of the widely used remdesivir in adults is controversial. • All recommendations and guidelines on the use of remdesivir in the pediatric population are based mainly on clinical trials in adults. WHAT IS NEW: • Remdesivir is a safe treatment for COVID-19 in the pediatric population. • In children with asymptomatic to moderate COVID-19 and risk factors for severe disease, remdesivir could be an effective drug to prevent disease progression. However, its efficacy in treating severe disease in children needs further exploration.

Outcomes of Transannular Repair of Tetralogy of Fallot With a Contegra® Monocuspid Patch.

BACKGROUND: Surgical repair of tetralogy of Fallot (ToF) depends on the anatomical variations of the heart defect. A group of patients with a hypoplastic pulmonary valve annulus required a transannular patch. This study aimed to evaluate the early and late outcomes of ToF repair with a transannular Contegra® monocuspid patch in a single center. METHODS: A retrospective review of medical records was conducted. This study included 224 children with a median age of 13 months who underwent ToF repair with a Contegra® transannular patch in over 20 years of observation. The primary outcomes were hospital mortality and need for early reoperations. The secondary outcomes were late death and event-free survival. RESULTS: The hospital mortality in our group was 3.1%, whereas two patients required early reoperation. Three patients were excluded from the study because follow-up data were not available. In the remaining group of patients (212 patients), the median follow-up was 116 (range, 1-206) months. One patient died because of sudden cardiac arrest at home six months after surgery. Event-free survival was observed in 181 patients (85.4%), whereas the remaining 30 patients (14.1%) required graft replacement. The median time to reoperation was 99 (range, 4-183) months. CONCLUSIONS: Although surgical treatment of ToF has been performed for more than 60 years worldwide, the optimal approach in children with a hypoplastic pulmonary valve annulus remains debatable. Among options, the Contegra® monocuspid patch can be effectively used in transannular repair of ToF with good long-term results.

Surgical Outcomes of Total Anomalous Pulmonary Venous Connection Repair.

Background and Objectives: Over the years, surgical repair of total anomalous pulmonary venous connection (TAPVC) outcomes have improved, however, morbidity and mortality still remain significant. This study aims to assess the early and long-term outcomes of surgical treatment of TAPVC children, operated on between 2006 and 2016, in one pediatric center in Poland. Materials and Methods: Diagnostics, surgical treatment, and follow-up data from 83 patients were collected. In addition, survival and risk factor analyses, control echocardiographic, and electrocardiographic examinations were performed. Results: In the analyzed group (n = 83), there were seven hospital deaths (within 30 days after the operation) (8.4%) and nine late deaths (10.8%). The mean follow-up time was 5.5 years, and, for patients who survived, it was 7.1 years. The mean survival time in patients with completed follow-up (n = 70) was 10.3 years; the overall five-year survival rate was 78.4%. Independent mortality risk factors were type I TAPVC, single ventricle physiology, time from admission to operation, intensive care unit stay, postoperative hospital stay, and temporary kidney insufficiency requiring dialysis. Conclusions: The presence of single ventricle physiology and the supracardiac subtype of TAPVC might be negative prognostic factors, while normal heart physiology presents with good post-repair results. This study indicates that cardiac arrhythmias may occur. Morbidity and mortality, related to surgical TAPVC correction, still remain significant.

Clinical characteristics of Kawasaki disease in Polish children: A retrospective study.

BACKGROUND: Kawasaki disease (KD), an acute, generalized vasculitis, is associated with an increased risk of coronary heart disease and is the most common cause of acquired heart disease in childhood. The incidence of KD is increasing worldwide. AIMS: Our study aims to analyze KD's clinical course in children and to evaluate risk factors for persistent changes in coronary vessels after 6-8 weeks of treatment. METHODS: The retrospective analysis included patients with KD hospitalized in a single tertiary carehospital. The diagnosis, as well as treatment, were based on the current worldwide treatment standards. The clinical course, selected laboratory parameters, the treatment effect, and following cardiac complications were analyzed in different age groups. RESULTS: In the years 2006-2019, 140 patients aged from two months to 16 years: 52 girls and 88 boys, were diagnosed with KD. Coronary artery aneurysms (CAA) at weeks 6-8 of disease were found in 16% of patients. Boys and infants were more likely to develop aneurysms at weeks 6-8 of the disease (P = 0.045; P = 0.03; respectively). The CAA frequency was related to the atypical course (P = 0.02), late diagnosis (P = 0.04), presence of changes in the coronary arteries at the time of diag nosis (P<0.001), immunoglobulin resistance (P = 0.002), a lower hemoglobin concentraction (P<0.001), and a higher platelet count (P = 0.02). There were 28% of patients resistant to first-line time treatment. In this group, we found CAA in 31% of children. CONCLUSIONS: We found that late diagnosis, low hemoglobin level, high platelet count, CAA presence at diagnosis, atypical course of KD, and resistance to intravenous immunoglobulins are predictors of CAA after 6-8 weeks in KD patients.

Anti-inflammatory Treatment of Kawasaki Disease: Comparison of Current Guidelines and Perspectives.

Kawasaki disease (KD), an acute, generalized vasculitis, is associated with an increased risk of coronary heart disease and is the most common cause of acquired heart disease in childhood. The incidence of KD is increasing worldwide. There are numerous international treatment guidelines. Our study aims to perform the first one so far comparison of them. While the gold standard therapy remains still the same (intravenous immunoglobulins and aspirin), there is currently a lack of evidence for choosing optimal treatment for high-risk patients and refractory KD. In this review, we also discuss the treatment of complications of KD and Kawasaki-like phenotypes, present an anti-inflammatory treatment in the light of new scientific data, and present novel potential therapeutic targets for KD.

Parenteral nutrition mixtures prepared at home by trained parents are as safe as pharmacy-made mixtures: a 3-y prospective study.

OBJECTIVES: Home parenteral nutrition (HPN) prevents malnutrition in children with intestinal failure improving chances for the best possible physical development and quality of life. For organizational reasons, in Poland the majority of children on HPN receive nutrition mixtures prepared at home by their parents. The aim of this study was to evaluate whether this method influences the frequency of catheter-related bloodstream infections (CRBSIs). METHODS: The aim of this prospective study was to assess the incidence of CRBSIs from January 2008 to December 2010 in 181 children on HPN and to compare the incidence in two subgroups of these children: group Ph (pharmacy) consisting of 43 patients receiving mixtures prepared in the hospital pharmacy weekly, and group H (home)-138 patients receiving nutrition mixtures prepared daily at home by their caregivers. Central venous CRBSIs were diagnosed by the presence of clinical signs (such as fever, chills, malaise, vomiting) associated with positive blood culture from the central catheter. RESULTS: From 2008 to 2010 parenteral nutrition was administrated during 128 905 catheter-days in 181 patients, and CRBSIs occurred 129 times (1 episode per 1000 catheter-days) in 65 patients. There were 42 bloodstream infections in group Ph during 29 225 catheter-days (i.e., 1.43 per 1000 catheter-days), whereas 87 bloodstream infections occurred in group H during 100 775 catheter-days (i.e., 0.86 per 1000 catheter-days; P = 0.0516). CONCLUSIONS: Preparing parenteral nutrition mixtures at home by trained parents is a safe method of HPN with a slightly (however insignificantly) lower incidence of CRBSIs compared with hospital pharmacy-prepared parenteral nutrition mixtures.

Polish experience in nutrition support in children.

Organization of enteral nutrition programme in Poland has developed rapidly in the last years, however, the underdiagnosis and late diagnosis of malnutrition are still the major challenges. For those children who are unable to tolerate enteral diet, intravenous support is required. Main achievements in our parenteral nutrition programme (PN) consist in decreasing septic complication rate and introducting fish oil based emulsions for prevention of PN-related liver disease. The challenge to combat in the future comprises development of a network of nutritional centres covering the whole country, which will take care of patients requiring nutrition support. The Children's Memorial Health Institute in Warsaw will remain the reference centre for the management of the most complicated cases. The organization of postgraduate courses should lead to continuous reduction of nutrition related complications rate. The important problem remains the relatively low number of patients weaned off PN. The widespread introduction of intestine rehabilitation programme is essential to improve this issue.

[Congenital diarrhoea]. / Biegunka wrodzona.

Congenital diarrhoea of heterogenic etiology is a rare cause of chronic diarrhoea. Characteristic features are: onset in the first weeks of life, life-threatening severe dehydratation and electrolyte disorders leading to a necessity of long-term parenteral nutrition. The clinical onset may be delayed and the degree of diarrhoea may be modest, making the diagnosis difficult. The main causes of congenital diarrhoea such as intestine electrolytes, carbohydrates, lipid and protein transport disorders and congenital enzymatic deficiencies, enterocyte polarization disorders, hormonal, immunological, metabolic, genetic and congenital anatomic disorders are presented in the paper. Some of them, such as: microvillus inclusion disease, tufting enteropathy, intestinal anedocrynosis, IPEX syndrome (immunodysregulation polyendocrinopathy enteropathy X-linked syndrome) have been described recently. One of the basic investigations, when congenital diarrhea is suspected, is general examination of the stool, its electrolyte concentration and serum electrolytes and blood gas analysis. Often, small bowel biopsy with histological examination (with the use of electronic microscopy and PAS staining) is indicated. In some cases molecular examination is possible and indicated. In differential diagnosis other, more frequent causes of chronic diarrhea of infancy, have to be excluded. In most of the cases of congenital diarrhoea there is no casual treatment available - usually long-term parenteral nutrition is necessary.

[Prevention of osteopenia in premature infants]. / Zapobieganie osteopenii u wczesniaków.

Proposition of recommendations for prevention of osteopenia in premature infants is presented in this article. In parenteral nutrition in premature infants calcium and phosphorus should be supplemented early in a dose of 80-100 mg/kg/24 h (2-2.5 mmol/kg/24 h) and 43-63 mg/kg/24 h (1.4-2 mmol/kg/24 h) respectively. In enteral nutrition calcium and phosphorus should be supplemented in a dose 90-150 mg/kg/24 h (2.25-3.7 mmol/kg/24 h), and: 45-80 mg/kg/24 h (1.5-2.6 mmol/kg) respectively. Breast milk fortifier is recommended up to the corrected age of 40 Hbd and in case of growth retarded infants - up to 52 Hbd. Vitamin D should be supplemented in a dose of 400-800 IU, particularly in breast fed infants. Vitamin content in formula or fortifier must be taken into account. Active metabolites of vitamin D are not recommended. Physical activities, together with appropriate mineral, protein and energy intake may decrease the risk of osteopenia. Laboratory assessment of calcium and phosphorus homeostasis is recommended every 2 weeks, from 6(th) week of life.

[Treatment of gastro-esophageal reflux in children and premature infants]. / Leczenie refluksu zoladkowo-przelykowego u dzieci i noworodków urodzonych przedwczesnie.

Gastroesophageal reflux is a common problem in the paediatric population, which affects especially infants, neonates and preterm infants. Usually the course is benign, but it can also be a cause of severe complications and acute life threatening events. In this paper diagnostic methods are presented and a systematic literature review of treatment options is given. Current recommendations are proposed according to the results of this review.

[Immunological response (IgM) to Legionella pneumophila infection in children]. / Odpowiedz odpornosciowa w klasie IgM na zakazenie Legionella pneumophila u dzieci.

Most of the publications report cases of Legionella infection among adults. In our studies the level of IgM to Legionella pneumophila sg 1 was determined by ELISA method in serum samples of 144 children with symptoms of respiratory tract infections. Children were from 5 months to 18 years old. The significant level of IgM was found in 41 sera collected from 34 children aged 2 years and above. There were samples collected from 21 girls and 13 boys. The highest percentage of significant level of IgM was found in the age group 4-10 years (31.8%) and 10-14 years (31.7%). Among the youngest children (below 2 years) the equivocal results were found in 6 samples. The significant difference in IgM level was found between the children age groups. There was not found the significant differences in IgM level depending on gender. Obtained results indicated the importance of such kind of studies and necessity of adjusting cut-off values to age groups of children.