Impact of SARS-CoV-2 infection on pain crisis and acute chest syndrome in patients with sickle cell anemia: A retrospective multi-cohort study based on US national data from 2020 to 2022.

COVID-19 infection has been a significant contributor to global morbidity and mortality, especially among those patients with chronic diseases. The Centers for Disease Control and Prevention have classified sickle cell disease (SCD) as a condition that increases the risk of severe illness from COVID-19 infection. A retrospective study was conducted using the TRiNetX health research network database to identify SCA patients ( HbSS, Sbeta-thalassemia zero) who had SARS-CoV-2 infection over 2 years; these were compared with similar patients who did not have the infection in terms of demographics, pain control, and laboratory parameters COVID-19 illness impacts [ain crises and ACS, and prior vaccination against influenza and COVID-19 may represent a protective factor for developing pain crises.

Hematologic manifestations of coronavirus disease 2019 in children: Case-series report and a review.

On 11 March 2020, coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus (SARS-CoV-2) was declared as a pandemic by the World Health Organization (WHO). As the COVID-19 pandemic has ravaged worldwide, children have not been unaffected. Information gleaned from adult experience with the disease has aided in disease detection and treatment strategies in children. Numerous cases have been described in adult literature about hematologic manifestations of COVID-19. This case series aims to report several hematologic presentations in patients with COVID-19 and multisystem inflammatory syndrome in children (MIS-C, an immune-mediated reaction leading to severe COVID-19 illness) with and without a primary hematologic disorder.

Corrigendum: Effect of COVID-19 pandemic on well child care and vaccination.

[This corrects the article DOI: 10.3389/fped.2022.873482.].

Effect of COVID-19 Pandemic on Well Child Care and Vaccination.

Objectives: The COVID-19 virus is highly contagious primarily via aerosol transmission and has a high mortality rate. On March 13, 2020, the United States declared a national emergency in response to the COVID-19 pandemic. This study aims to enumerate the effect of the pandemic on vaccination rates during the COVID-19 lockdown and the aftermath in pediatric patients aged 6weeks-6 years. Study Design: A retrospective review of medical records was performed of missed well childcare visits at MetroHealth from March 1, 2020 to June 30, 2020. The sample size of 400 children aged 6 weeks to 6 years were randomly selected. Demographic data, number of calls made to attempt, scheduled WCC, no show rates for clinic appointments, number of missed WCC, location of MH facility, insurance type, vaccination status prior to the pandemic were collected. Statistical analysis was performed with SPSS software (IBM Corp. Released 2020. IBM SPSS Statistics for Windows, Version 27.0. Armonk, NY: IBM Corp). Results: From this descriptive study, we found that 43.5% of patients were not up to date on their childhood vaccination. The mean age was 24.38 months (SD 20.15). There were slightly more males (52.8%) in the study than females (47.3%) and most children were of African American descent. More than 50% of patients missed a scheduled well child appointment and 27% had a missed at least two consecutive appointments. Conclusion: The COVID-19 pandemic has no doubt made a significant mark on health care; the effects would be both immediate and delayed, with vulnerable population being the most impacted. There is an urgent need to prevent a large-scale health disaster of catastrophic potential that could occur if an effective vaccination strategy is not implemented rapidly.

Sickle cell disease: A primary care update.

Sickle cell disease (SCD) is the most common hemoglobinopathy in the United States and causes significant disease-related morbidity including multiorgan damage, chronic anemia, and debilitating pain crises. Primary care physicians play a key role in the medical home model of care for adults with SCD. This review focuses on current recommendations for health maintenance and provides a brief summary of disease complications and current updates.

Paediatric haematologists' attitudes regarding haematopoietic cell transplantation as treatment for sickle cell disease.

Beginning early in childhood, patients with sickle cell disease [SCD; a group of genetic haemoglobin disorders characterized by the sickle or HbS mutation (HBB E7V)] are at risk of life-threatening and debilitating health events. Despite the high morbidity and mortality of this disease, haematopoietic cell transplantation (HCT), a curative therapy for SCD, remains underutilized. A variety of factors, including the limited availability of suitable donors, play a role in this trend, but do not fully explain the low frequency with which this therapy is employed. The objective of this study was to identify paediatric haematologists' attitudes about HCT as a treatment option for SCD, and to describe the impact of these attitudes on their practices of discussing HCT with families of children affected by this disease. A nationwide survey of paediatric haematologists in the United States was conducted between February and May 2016. Two hundred and eighty-seven surveys were included in the final analysis (response rate 20%). On average, respondents reported informing 42% of families about HCT as a treatment option (N = 248, 95% confidence interval: 38-46). Clinician attitudes about the cost and safety of HCT were associated with practices of discussing this therapy with families. These findings suggest that clinician attitudes and referral practices may play a role in the underutilization of this therapy in the SCD population.

Central Venous Line Associated Deep Vein Thrombosis in Hospitalized Children.

An increase in the incidence of deep vein thrombosis (DVT) has been reported in pediatric patients over the past decade. The presence of central venous line (CVL) is a major contributing risk factor with conflicting data on the relative risk of DVT with various types of central lines. We aimed to assess the incidence of and identify potential risk factors for DVT overall and with different types of CVL individually. A retrospective chart review of pediatric patients with a CVL placed at Cleveland Clinic Children's from 2011 to 2016 was conducted. Data collected included demographics, potential risk factors, CVL characteristics and related thrombotic events. The study cohort consisted of 376 CVLs in 325 patients between 0 and 26 years of age. There were 1.6 thrombi per 10,000 line-days (95% confidence interval: 1.0, 2.5), and the overall incidence of DVT was 5.1%. The incidence of DVT was highest with tunneled catheters (5/16=31%) versus with peripherally inserted central catheters (4/111=3.6%) or with ports (10/249=4%, P<0.001), and whereas there were overarching significant risk factors for CVL-associated thrombi, these risk factors differed in significance when analyzed by the CVL type. The study supports the need for continued improvement in pediatric hospital practices for early identification of patients at a higher thrombosis risk.

Parent and Guardian Knowledge of Hematopoietic Cell Transplantation as a Treatment Option for Sickle Cell Disease.

Beginning early in childhood, patients with sickle cell disease (SCD) are at risk of life-threatening and debilitating health events. Despite the high morbidity and mortality of this disease, hematopoietic cell transplantation (HCT), a curative treatment for SCD, remains underutilized. In the literature there is a paucity of data concerning medical decision maker (MDM) awareness of HCT as a treatment option for SCD. The objective of this study was to estimate the proportion of parents/guardians of children with SCD who are aware of HCT as a treatment option, and to identify the demographic factors associated with knowledge of this therapy's curative potential. Between November 2015 and December 2016, 327 parents/guardians were surveyed across 4 clinical sites in 3 Midwestern US cities. Although 82% of parents/guardians had heard of HCT in the past and 78% were aware of the therapy's curative potential, nearly half indicated that they did not know whether HCT could specifically cure their child of the disease. Respondents who had discussed HCT with their child's physician had 5 times higher odds of being aware of HCT's curative potential than those who had not. These findings suggest that additional efforts to enhance MDM knowledge of HCT as well as shared decision making in the use of this therapy, is warranted.

HbS-Sicilian (뫧)0-Thalassemia: A Rare Variant of Sickle Cell.

Sickle cell disease (SCD) is caused by a mutation in the sixth codon of the ß-globin gene on chromosome 11, which leads to a single amino acid substitution (glutamine to valine). Sickle-(δß)0-thalassemia is a rare variant of sickle cell disease (delta-beta thalassemia occurring in association with sickle hemoglobin, HbS), sparsely reported in literature, and has been associated with symptomatology necessitating careful monitoring and follow-up. We describe a patient who presented with a newborn screen reported as "FS" and a negative family history for sickle cell disease and sickle cell trait. Subsequent gene sequencing studies demonstrated the presence of Sickle-(δß)0-thalassemia. Clinical course has remained relatively stable for this patient now at 18 months of age without any SCD related symptomatology or complications. As this is a rare variant of SCD with potential complications, it is important to establish diagnosis towards planning comprehensive care.

Small molecules, LLL12 and FLLL32, inhibit STAT3 and exhibit potent growth suppressive activity in osteosarcoma cells and tumor growth in mice.

Constitutive activation of Signal Transducers and Activators of Transcription 3 (STAT3) is frequently detected in osteosarcoma, and hence, may serve as a therapeutic target. In order to target STAT3, we tested two new STAT3 inhibitors, LLL12 and FLLL32. LLL12 and FLLL32 inhibit STAT3 phosphorylation and STAT3 downstream targets. LLL12 and FLLL32 also inhibit IL-6 induced STAT3 phosphorylation. The inhibition of STAT3 by LLL12 and FLLL32 resulted in the induction of apoptosis, reduction of plating efficiency, and migration in osteosarcoma cells. Furthermore, LLL12 and FLLL32 inhibited SJSA osteosarcoma cells and OS-33 tumor growth in murine xenografts. These results provide evidence that constitutive STAT3 signaling is required for osteosarcoma survival and migration in vitro and tumor growth in vivo. Blocking persistent STAT3 signaling by LLL12 and FLLL32 may be a novel therapeutic approach for osteosarcoma.

Bilateral burkitt lymphoma of the ovaries: a report of a case in a child with williams syndrome.

A 10-year-old female with Williams Syndrome (WS) presented with a two-month history of fatigue, weight loss, and bilateral ovarian masses. Histologic, immunophenotypic, and cytogenetic studies confirmed the diagnosis of Burkitt lymphoma (BL). While there is no established association between the two disorders, this is the third case in the literature of Burkitt lymphoma in a patient with Williams Syndrome.

Hepatoblastoma and prune belly syndrome: a potential association.

Prune belly syndrome (PBS) is a congenital anomaly characterized by the clinical triad of lax abdominal musculature, bilateral cryptorchidism, and abnormalities of the kidney and urinary tract. Previous reports of malignancy in patients with PBS have been limited to germ cell tumors. Hepatoblastoma (HBL) is the most common hepatic malignancy of childhood, affecting approximately 100 children each year in the USA. We describe a set of 4 pediatric patients with PBS and HBL. All individuals were born after 2002. These subjects lacked genetic, natal, or environmental factors known to confer risk of HBL. The occurrence of PBS and HBL in these patients constitutes a novel potential association.