Clinical Phenotype and Management of Severe Neurotoxicity Observed in Patients with Neuroblastoma Treated with Dinutuximab Beta in Clinical Trials.

Neurotoxicity is an off-tumour, on-target side effect of GD2-directed immunotherapy with monoclonal antibodies. Here, we report the frequency, management and outcome of patients enrolled in two prospective clinical trials who experienced severe neurotoxicity during immunotherapy with the anti-GD2 antibody dinutuximab beta (DB) administered as short-term infusion (HR-NBL1/SIOPEN study, randomisation R2, EudraCT 2006-001489-17) or as long-term infusion (HR-NBL1/SIOPEN study, randomisation R4, EudraCT 2006-001489-17 and LTI/SIOPEN study, EudraCT 2009-018077-31), either alone or with subcutaneous interleukin-2 (scIL-2). The total number of patients included in this analysis was 1102.

Incidence patterns of childhood non-Wilms renal tumors: Comparing data of the Nationwide Registry of Childhood Hematological Malignancies and Solid Tumors (NARECHEM-ST), Greece, and the Surveillance, Epidemiology, and End Results Program (SEER), USA.

Background: We used, for the first time, data registered in the Nationwide Registry for Childhood Hematological Malignancies and Solid Tumors (NARECHEM-ST)-Greece to estimate incidence/time trends of the rare childhood (0-14 years) non-Wilms tumors (non-WT), and compared the results of malignant non-WT to those from the Surveillance, Epidemiology, and End Results Program (SEER)-USA.

Methods: Fifty-five cases (n = 33 malignant-only) were extracted from NARECHEM-ST (2001-2020) and 332 malignant cases from SEER (1990-2017). To allow between-country comparisons, age-standardized incidence rates (AIR) of malignant-only non-WT were calculated, and temporal trends were evaluated using Poisson and joinpoint regressions.

Concomitant Existence of Bilateral Adrenal Adenomas. To Operate or Not?

Nowadays, through the wide use of both magnetic resonance imaging and computed tomography, the diagnosis of adrenal incidentaloma is becoming increasingly frequent. Pheochromocytomas are neuroendocrine tumors which produce catecholamine, and they are characterized by headaches, palpitations, sweating and hypertension. Aldosterone-secreting adrenal cortical adenomas can cause various metabolic and cardiovascular diseases due to aldosterone excess.

Characterization of Amino Acid Substitutions and Deletions in Kindlin-1 FERM Domain: Relevance for Precision Medicine.

Kindler epidermolysis bullosa is a genodermatosis that manifests with cutaneous and mucosal fragility and with photosensitivity. No cure is available to date. Kindlin-1, a deficient protein, binds to β-integrin and is required for its activation.

Neuroblastoma survivors at risk for developing subsequent neoplasms: A systematic review.

Neuroblastoma survivors have an increased risk of unfavorable long-term health outcomes, of which developing subsequent neoplasms is one of the most serious. We aimed to provide an overview of the current knowledge on the risk of subsequent neoplasms in neuroblastoma survivors. We conducted a systematic literature search in Medline/Pubmed (01-01-1945-13-01-2022) to identify studies that reported on ≥ 100 neuroblastoma survivors and assessed subsequent neoplasms as an outcome.

Allogeneic hematopoietic stem cell transplantation in infants is associated with significant morbidity and mortality.

Background: Infants are subjected to hematopoietic stem cell transplantation (HSCT) due to malignant and non-malignant diseases. However, specific data concerning the outcome and transplantation-related complications in infants, as a separate age group, are limited. Our aim was to evaluate the impact of infancy on the outcome, toxicity, and complications after HSCT.

Low- and High-Attenuation Lung Volume in Quantitative Chest CT in Children without Lung Disease.

In contrast to studies of adults with emphysema, application of fixed thresholds to determine low- and high-attenuation areas (air-trapping and parenchymal lung disease) in pediatric quantitative chest CT is problematic. We aimed to assess age effects on: (i) mean lung attenuation (full inspiration); and (ii) low and high attenuation thresholds (LAT and HAT) defined as mean attenuation and 1 SD below and above mean, respectively. Chest CTs from children aged 6-17 years without abnormalities were retrieved, and histograms of attenuation coefficients were analyzed.

Mitochondrial neurogastrointestinal encephalomyopathy: Clinical and biochemical impact of allogeneic stem cell transplantation in a Greek patient with one novel mutation.

We describe the case of a Greek female patient with the Classic form of the ultra- rare and fatal autosomal recessive disorder Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) and the impact of allogeneic hematopoietic stem cell transplantation on the biochemical and clinical aspects of the disease. The patient presented at the age of 15 years with severe gastrointestinal symptoms, cachexia, peripheral neuropathy and diffuse leukoencephalopathy. The diagnosis of MNGIE disease was established by the increased levels of thymidine and deoxyuridine in plasma and the complete deficiency of thymidine phosphorylase activity.

Treatment of alopecia totalis/universalis/focalis with vitamin D and analogs: Three case reports and a literature review.

Background: Alopecia areata (AA) is an inflammatory disease with autoimmune, environmental, and inherited components directed at the hair follicle, either limited to patchy hair loss over the scalp (Focalis, AF), total loss of scalp hair (Totalis, AT), or total loss of both scalp and body hair (Universalis, AU). Despite multiple treatment modalities, no therapy exists. Vitamin D deficiency in patients with AA/AT/AF influences disease severity and duration, inversely correlating with inflammation histologically.

Adolescent Perspectives About Online Hate Speech: Qualitative Analysis in the SELMA Project.

Objective: This paper aims to study the views, perceptions and representations of online hate speech among adolescents in the Greek cohort of the SELMA Project.

Methods: Qualitative research was conducted in focus groups of 36 Greek adolescents and the data were processed through thematic analysis method.

Results: The majority was unfamiliar with the term "hate speech" and confused it with cyberbullying.

Forced Oscillation Technique for Monitoring the Respiratory Status of Children with Cystic Fibrosis: A Systematic Review.

Spirometry is considered the gold standard method for monitoring lung function of patients with cystic fibrosis (CF) but it requires patients' cooperation and therefore it is not useful for the majority of preschool-aged children. Oscillometry is an alternative modality for lung function monitoring that requires minimal cooperation and can be applied in children as young as 3 years of age. Furthermore, it generates lesser aerosol compared to spirometry, an issue that is of considerable importance in the COVID-19 era.

Acute acalculous cholecystitis due to infectious causes.

Acute acalculous cholecystitis (AAC) is an inflammation of the gallbladder not associated with the presence of gallstones. It usually occurs in critically ill patients but it has also been implicated as a cause of cholecystitis in previously healthy individuals. In this subgroup of patients, infectious causes comprise the primary etiology.

Accuracy of the sleep clinical record for the diagnosis of pediatric moderate-to-severe obstructive sleep apnea syndrome.

Purpose: The sleep clinical record (SCR) has been used to diagnose obstructive sleep apnea syndrome (OSAS) in children when access to polysomnography (PSG) is limited. Our aim was to determine the best SCR score that could facilitate diagnosis of moderate-to-severe OSAS in children with snoring.

Methods: Healthy children with history of snoring, who were referred for PSG, were prospectively recruited.

Systematic Review and Meta-analysis of Histological Gastric Biopsy Aspects According to the Updated Sydney System in Children.

Objectives: A descriptive and comparative study of gastric histological aspects according to the updated Sydney classification (USC), obtained from Helicobacter pylori-positive versus H pylori-negative children referred for upper gastrointestinal endoscopy.

Methods: The Prisma method was used to perform a systematic review and meta-analysis. Selection criteria were based on following key words USC, H pylori, children, endoscopy, or biopsy.

Small bowel obstruction due to a congenital adhesion: a rare case report.

The exact incidence of small bowel obstruction (SBO) due to congenital adhesions remains unclear. Herein, we report a 59-year-old male who appeared in the emergency department with diffuse abdominal pain associated with vomiting. The patient reported no previous medical or surgical history.

Osteoid Osteoma: An Updated Review of Epidemiology, Pathogenesis, Clinical Presentation, Radiological Features, and Treatment Option.

Osteoid osteoma, the third most common benign bone tumor, usually occurs in the cortex of long bones. It consists of a radiolucent nidus surrounded by reactive osteosclerosis. Generally, osteoid osteoma affects young males.

Association of vitamin A status with lung function in children and adolescents with cystic fibrosis.

Importance: Vitamin A (or retinol) has considerable antioxidative and anti-inflammatory attributes and it may have protective effects on the respiratory health of patients with cystic fibrosis (CF). This issue, however, remains controversial.

Objective: The purpose of the present study was to investigate the relationship between serum retinol levels (SRL) and force expiratory volume in 1 second (FEV1) in patients with CF.

Impact of COVID-19 Lockdown on Pulmonary and Nutritional Status in Children and Young Adults With Cystic Fibrosis, in Greece.

During the first wave of the coronavirus pandemic in 2020, Greece adopted strict lockdown measures. We aimed to investigate the effects of lockdown and the resultant changes in the standard of care, on the lung function and somatic growth of cystic fibrosis (CF) patients. We analyzed data on body mass index and lung function of 103 CF patients 5.

Quantitation and evaluation of perinatal medium-chain and long-chain acylcarnitine blood concentrations in 12,000 full-term breastfed newborns.

Objectives: Medium-chain (MCA) and long-chain acylcarnitine (LCA) blood concentrations play a significant role in the fatty acid (FA) oxidation process, especially during the first days of life. Identification of their abnormal concentrations, expanded newborn screening, can lead to the diagnosis of FA oxidation disorders. This study aimed to demonstrate MCA and LCA concentrations in Dried Blood Spots (DBS) of full-term breastfed infants, in relation to their birth weight (BW) perinatally.

Randomized Trial of Two Induction Therapy Regimens for High-Risk Neuroblastoma: HR-NBL1.5 International Society of Pediatric Oncology European Neuroblastoma Group Study.

Purpose: Induction therapy is a critical component of the therapy of high-risk neuroblastoma. We aimed to assess if the Memorial Sloan Kettering Cancer Center (MSKCC) N5 induction regimen (MSKCC-N5) would improve metastatic complete response (mCR) rate and 3-year event-free survival (EFS) compared with rapid COJEC (rCOJEC; cisplatin [C], vincristine [O], carboplatin [J], etoposide [E], and cyclophosphamide [C]).

Patients And Methods: Patients (age 1-20 years) with stage 4 neuroblastoma or stage 4/4s aged < 1 year with amplification were eligible for random assignment to rCOJEC or MSKCC-N5.

Overall and event-free survival of childhood lymphoma in Greece: analysis of harmonized clinical data over a 24-year active registration period.

We assessed event-free (EFS) and overall (OS) survival in 676 incident cases of childhood Hodgkin (HL) and non-Hodgkin (NHL) lymphoma actively registered in Greece (1996-2019). HL-OS was 96% and NHL-OS 85%, whereas HL-EFS was 86% and NHL-EFS was 81%, notably similar to the respective OS rates (HL: 95%, NHL: 85%) in developed countries. For HL, older age at diagnosis, high maternal education and close proximity to treatment centers were linked to remarkably favorable outcomes.