Prognostic role of bone erosion in orbital RMS: a report from the European Pediatric Soft Tissue Sarcoma Study Group (EpSSG).

Background: Orbital rhabdomyosarcoma (RMS) is often limited to the orbital cavity and has a favorable prognosis. In some cases, the tumor can erode the orbital bone and behave as a parameningeal RMS (PM-RMS); thus, it is treated more intensively. However, the current protocols do not provide any guidance on how to consider different grades of bone erosion (BE) that can vary widely, hampering a uniform classification and the subsequent treatment assignment.

Nephrocalcinosis tendency does not worsen under burosumab treatment for X-linked hypophosphatemic rickets: a multicenter pediatric study.

Background: X-linked hypophosphatemic rickets (XLH) is associated with uninhibited FGF23 activity, which leads to phosphaturia, hypophosphatemia and depressed active vitamin D (1,25OH2D) levels. Conventional treatment with phosphate supplements and vitamin D analogs may lead to hypercalciuria (HC), nephrocalcinosis (NC) and hyperparathyroidism. We investigated the effects of burosumab treatment, an anti-FGF23 monoclonal antibody recently approved for XLH, on these complications.

PDSS1 mutations-associated steroid-resistant nephrotic syndrome: case report and review of literature.

PDSS1 mutations hamper Coenzyme Q10 biosynthesis and cause a rare multisystem mitochondrial disease characterized by diverse clinical features and limited treatment options. To date, renal involvement has been reported in only one patient. We report a new female patient with compound heterozygous PDSS1 mutations and the clinical outcome following a trial of Coenzyme Q10 therapy.

Upadacitinib for Induction of Remission in Pediatric Ulcerative Colitis: An International Multi‑center Study.

Background And Aims: Data on upadacitinib therapy in children with ulcerative colitis (UC) or unclassified inflammatory bowel disease (IBD-U) are scarce. We aimed to evaluate the effectiveness and safety of upadacitinib as an induction therapy in pediatric UC or IBD-U.

Methods: In this multicenter retrospective study, children treated with upadacitinib for induction of remission of active UC or IBD-U from 30 centers worldwide were enrolled.

Prognostic Factors Associated With Increased Mortality in Pediatric Veno-Occlusive Disease Following Hematopoietic Cell Transplantation.

Background: Hepatic veno-occlusive disease (VOD) is a life-threatening complication of hematopoietic cell transplantation (HCT) and is categorized as a transplant-related, systemic endothelial disease. Severe VOD can lead to multi-organ dysfunction (MOF) and is associated with a high mortality rate.

Objective: To evaluate the incidence of VOD in children after HCT and analyze the outcomes and risk factors associated with increased mortality.

Parameningeal Rhabdomyosarcoma: Results of the European Pediatric Soft Tissue Sarcoma Study Group RMS 2005 Study.

Background: Parameningeal (PM) site is an unfavorable characteristic in rhabdomyosarcoma (RMS). We described the treatment and outcome for patients with PM RMS and investigated the prognostic value of risk factors. We scored PM site by originating site and by highest risk extension.

[CEREBROSPINAL FLUID (CSF) LEAK FOLLOWING COVID-19 NASAL SWAB: A CASE REPORT].

Coronavirus 19 (Covid-19) emerged as a global pandemic with over 12,900 deaths in Israel alone. On average, more than 40,000 tests to detect the virus daily were performed in Israel. According to the US Centers for Disease Control and Prevention (CDC), the recommended methods for testing are nasopharyngeal swab, mid-turbinate, or anterior nasal swab specimen.

Factors associated with early mortality in pediatric tracheostomy placement: a retrospective cohort study.

Unlabelled: To investigate the factors associated with early mortality and complications in children who receive a tracheostomy placement. This was a retrospective study at a tertiary referral center. Children who underwent tracheostomy between 2012 and 2023 was included.

BCL11A intellectual developmental disorder: defining the clinical spectrum and genotype-phenotype correlations.

An increasing number of individuals with intellectual developmental disorder (IDD) and heterozygous variants in BCL11A are identified, yet our knowledge of manifestations and mutational spectrum is lacking. To address this, we performed detailed analysis of 42 individuals with BCL11A-related IDD (BCL11A-IDD, a.k.

An international survey on retinopathy of prematurity practice patterns during the COVID-19 pandemic and lessons for future management.

Purpose: To assess retinopathy of prematurity (ROP) practice patterns during the coronavirus (COVID-19) pandemic.

Methods: A survey on ROP practice patterns during the COVID-19 pandemic was sent to the American Academy of Ophthalmic Executives, the International Pediatric Ophthalmology and Strabismus Council members, and to various national societies on May 19, 2020. The survey closed on the 31st of June 2020.

Exploring nurse mentors' job crafting: A longitudinal study on missed nursing care across student supervision.

Aim: To compare the job crafting strategies nurse mentors use when mentoring students versus during periods of respite and to assess the effectiveness of these strategies in reducing instances of missed nursing care (MNC).

Background: Nurses who serve as mentors may have to train a group of nursing students on top of their routine nursing duties. The corresponding high workloads and limited resources may force them to decide which nursing care to delay or omit (i.

Dynamics of gas exchange and heart rate signal entropy in standard cardiopulmonary exercise testing during critical periods of growth and development.

Standard cardiopulmonary exercise testing (CPET) produces a rich dataset but its current analysis is often limited to a few derived variables such as maximal or peak oxygen uptake (V̇O). We tested whether breath-by-breath CPET data could be used to determine sample entropy (SampEn) in 81 healthy children and adolescents (age 7-18 years old, equal sex distribution). To overcome challenges of the relatively small time-series CPET data size and its nonstationarity, we developed a Python algorithm for short-duration physiological signals.

An Integrative Pediatric Oncology Program Addressing Parents' Quality of Life-Related Concerns.

Background: Parents of children with cancer face bio-psycho-social-spiritual concerns which can significantly reduce quality of life (QoL). We examined the impact of an integrative oncology (IO) intervention on QoL-related concerns among parents of children in a pediatric hematology-oncology department.

Measures: The study was prospective, controlled, nonrandomized and patient-preferenced.

Impact of COVID-19 infection on lung function and nutritional status amongst individuals with cystic fibrosis: A global cohort study.

Background: Factors associated with severe COVID-19 infection have been identified; however, the impact of infection on longer-term outcomes is unclear. The objective of this study was to examine the impact of COVID-19 infection on the trajectory of lung function and nutritional status in people with cystic fibrosis (pwCF).

Methods: This is a retrospective global cohort study of pwCF who had confirmed COVID-19 infection diagnosed between January 1, 2020 and December 31, 2021.

Profiles of parental coping with paediatric cancer and their associations with parental illness adaptation.

Aims: To identify profiles of coping in parents of children with cancer and their underlying factors and to examine which profile(s) are associated with illness adaptation.

Design: A cross-sectional study utilizing surveys among parents of children with cancer (n = 89).

Methods: Questionnaires included socio-demographics, ways of coping, parenting stress, depression, post-traumatic symptoms, illness adjustment and quality of life.

National study reveals gram negative bacteremia on contemporary pediatric AML protocol.

Since bacteremia complicates childhood Acute myeloid leukemia (AML) patients, we assessed bacteremia rates in Israeli children with AML. All chemotherapy courses of patients enrolled in NOPHO-DBH-2012 AML protocol were included. Down syndrome, acute promyelocytic leukemia were excluded.

Anesthetic approach to pregnant patients with malaria: a narrative review of the literature.

Introduction: Anesthesiologists play an important role in the management of labor and delivery during acute malaria infection. The peripartum anesthesia considerations for such cases remain unclear.

Findings: Important peripartum considerations include the severity of thrombocytopenia and coagulopathy, hemodynamic status and cardiac disease, and the likelihood of central nervous system (CNS) involvement.

Localized incompletely resected standard risk rhabdomyosarcoma in children and adolescents: Results from the European Paediatric Soft Tissue Sarcoma Study Group RMS 2005 trial.

Background: The authors report the prospective evaluation of reduced dose alkylator chemotherapy combined with radiotherapy for European Pediatric Soft Tissue Sarcoma Study Group (EpSSG) standard risk nonalveolar rhabdomyosarcoma (NA-RMS).

Patients And Methods: Localized node negative Intergroup Rhabdomyosarcoma Study (IRS) II/III NA-RMS at favorable sites (subgroup C), <25 years old, received five cycles of ifosfamide, vincristine, and dactinomycin (IVA) chemotherapy (30 g/m ifosfamide) and four cycles of vincristine and dactinomycin (if receiving radiotherapy), or nine cycles of IVA (54 g/m ifosfamide) ± radiotherapy. Delayed primary tumor excision was considered for IRS III tumors.

Monitoring and integrated care coordination of patients with alpha-mannosidosis: A global Delphi consensus study.

Introduction: Current literature lacks consensus on initial assessments and routine follow-up care of patients with alpha-mannosidosis (AM). A Delphi panel was conducted to generate and validate recommendations on best practices for initial assessment, routine follow-up care, and integrated care coordination of patients with AM.

Methods: A modified Delphi method involving 3 rounds of online surveys was used.

Insights into Central Congenital Hypothyroidism: A Multicenter Retrospective Analysis.

Context: Central congenital hypothyroidism (CCH) is a thyroid hormone deficiency at birth caused by inadequate pituitary stimulation of the thyroid gland. Although primary CH has been studied extensively, studies on CCH are sparse.

Objectives: To assess the prevalence of CCH in Israel and describe its clinical features, neonatal screening results, and outcomes.