Updates on Langerhans cell histiocytosis and other histiocytosis in children: invited review-challenges and novelties in paediatric tumours.

Langerhans cell histiocytosis (LCH), juvenile xanthogranuloma (JXG) family lesions, and Rosai-Dorfman-Destombes disease (RDD) are now classified by the World Health Organization (WHO) under the heading of histiocytic/dendritic cell neoplasms. Each disease may manifest as a focal lesion, as multiple lesions, or as a widespread aggressive systemic disease with visceral organ involvement. Erdheim-Chester disease (ECD) is a rare systemic disease process of adults with limited cases in children.

Endovascular thrombectomy for childhood stroke (Save ChildS Pro): an international, multicentre, prospective registry study.

Background: Emerging evidence suggests that endovascular thrombectomy is beneficial for treatment of childhood stroke, but the safety and effectiveness of endovascular thrombectomy has not been compared with best medical treatment. We aimed to prospectively analyse functional outcomes of endovascular thrombectomy versus best medical treatment in children with intracranial arterial occlusion stroke.

Methods: In this prospective registry study, 45 centres in 12 countries across Asia and Australia, Europe, North America, and South America reported functional outcomes for children aged between 28 days and 18 years presenting with arterial ischaemic stroke caused by a large-vessel or medium-vessel occlusion who received either endovascular thrombectomy plus best medical practice or best medical treatment alone.

New Synergistic Combination Therapy of Mupirocin and α-Pinene Against Multidrug-Resistant Clinical Strains of Methicillin-Resistant Staphylococcus aureus.

Objective: Increased mupirocin use leads to mupirocin resistance and is associated with persistence of methicillin-resistant Staphylococcus aureus (MRSA) carriers, prolonged hospitalization, and significant economic burdens for health systems. The study aimed to investigate the antimicrobial activity of compounds of Salvia rosmarinus L. ("rosemary", formerly Rosmarinus officinalis), alone or in combination with mupirocin, against multidrug resistant MRSA using isolates obtained from pediatric patients.

How I do it: operative nuances of the Encephalo-Duro-Mio-Synangiosis (EDMS) technique for pediatric moyamoya disease and syndrome.

Background: Moyamoya disease, a progressive occlusive arteriopathy mainly affecting the supraclinoid internal carotid artery, leads to abnormal "Moyamoya vessels" and ischemic events in children due to decreased cerebral blood flow. Surgery, especially indirect revascularization, is suggested for pediatric Moyamoya cases.

Method: We present the Encephalo-Duro-Mio-Synangiosis (EDMS) technique, illustrated with figures and videos, based on 14 years' experience performing 71 surgeries by the senior author (SGJ) and the Moyamoya Interdisciplinary Workteam at "Prof.

Exome Sequencing Has a High Diagnostic Rate in Sporadic Congenital Hypopituitarism and Reveals Novel Candidate Genes.

Context: The pituitary gland is key for childhood growth, puberty, and metabolism. Pituitary dysfunction is associated with a spectrum of phenotypes, from mild to severe. Congenital hypopituitarism (CH) is the most commonly reported pediatric endocrine dysfunction, with an incidence of 1:4000, yet low rates of genetic diagnosis have been reported.

Human milk and breastfeeding during ketogenic diet therapy in infants with epilepsy: Clinical practice guideline.

Ketogenic diet therapy (KDT) is a safe and effective treatment for epilepsy and glucose transporter type 1 (GLUT1) deficiency syndrome in infancy. Complete weaning from breastfeeding is not required to implement KDT; however, breastfeeding remains uncommon. Barriers include feasibility concerns and lack of referrals to expert centres.

The diagnostic workup of children with the radiologically isolated syndrome differs by age and by sex.

Background: Cerebrospinal fluid (CSF) and spinal MRIs are often obtained in children with the radiologically isolated syndrome (RIS) for diagnosis and prognosis. Factors affecting the frequency and timing of these tests are unknown.

Objective: To determine whether age or sex were associated with (1) having CSF or spinal MRI obtained or (2) the timing of these tests.

Use of ketogenic dietary therapy for drug-resistant epilepsy in early infancy.

Objective: There is growing evidence that ketogenic dietary therapy (KDT) can be safely and efficiently used in young children, but little evidence exists on its use in newborns. Developmental and epileptic encephalopathies starting in the neonatal period or early infancy usually present a poor prognosis. The aim of this study was to evaluate effectiveness, safety, and survival of infants younger than 3 months of age with drug-resistant epilepsy in whom KDT was used.

[Optimization in the detection of bacteremia: Integrated use of FilmArray (BCID2) and MALDI-TOF].

The usefulness of the combined use of MALDI-TOF MS from a subculture with 3-5h of incubation and the BCID2 panel (FilmArray) for the identification of microorganisms from positive blood cultures and its importance in the adjustment of antimicrobial therapy was analyzed. Overall identification with BCID2 was 90.4% (142/157) and with Maldi-TOF MS 83.

Characteristics of Transport of Ill Pediatric Patients in the Emergency Department: A Latin America Multicenter Prospective Study.

Objective: The aim was to describe the characteristics of the transport system of critically ill pediatric patients in the emergency departments (EDs) in Latin America (LA).

Methodology: This is a prospective cross-sectional study in a 1-year period. Patients were recruited on days 1, 7, 14, 21, and 28 of each month in the EDs in LA.

Latitudes and attitudes: A multinational study of laparoscopic pyeloplasty in children.

Purpose: The Anderson-Hynes technique has been the treatment of choice for primary ureteropelvic junction obstruction in children. Laparoscopic approach has shown similar outcomes to open, with advantages of shorter hospital stay and less pain. We reviewed the experience of 11 geographically diverse, tertiary pediatric urology institutions focusing on the outcomes and complications of laparoscopic pyeloplasty.

Initial management of acute hyperammonemia in pediatrics.

Hyperammonemia is a medical emergency. There are no publications regarding the availability of resources, supplies, and knowledge necessary for the initial management of hyperammonemia by pediatricians in Argentina; however, according to the authors' experience, the necessary resources are not available all year round in a large portion of our territory. Based on such state of affairs, an international bibliographic review on this topic and the authors' experience, we developed a series of ecommendations for the initial pediatric management of this emergency, with the objective of reducing deficiencies, allowing adequate clinical suspicion leading to a timely diagnosis and emergency management and a rational use of pharmacological resources (some of which are costly) to reduce the morbidity and mortality associated with hyperammonemia.

Efficacy and safety of empagliflozin in glycogen storage disease type Ib: Data from an international questionnaire.

Purpose: This paper aims to report collective information on safety and efficacy of empagliflozin drug repurposing in individuals with glycogen storage disease type Ib (GSD Ib).

Methods: This is an international retrospective questionnaire study on the safety and efficacy of empagliflozin use for management of neutropenia/neutrophil dysfunction in patients with GSD Ib, conducted among the respective health care providers from 24 countries across the globe.

Results: Clinical data from 112 individuals with GSD Ib were evaluated, representing a total of 94 treatment years.

HAX1-dependent control of mitochondrial proteostasis governs neutrophil granulocyte differentiation.

The relevance of molecular mechanisms governing mitochondrial proteostasis to the differentiation and function of hematopoietic and immune cells is largely elusive. Through dissection of the network of proteins related to HCLS1-associated protein X-1, we defined a potentially novel functional CLPB/HAX1/(PRKD2)/HSP27 axis with critical importance for the differentiation of neutrophil granulocytes and, thus, elucidated molecular and metabolic mechanisms underlying congenital neutropenia in patients with HAX1 deficiency as well as bi- and monoallelic mutations in CLPB. As shown by stable isotope labeling by amino acids in cell culture (SILAC) proteomics, CLPB and HAX1 control the balance of mitochondrial protein synthesis and persistence crucial for proper mitochondrial function.

[Clinical guidelines for the managment of intestinal failure secondary to pediatric short bowel syndrome].

Intestinal failure secondary to short bowel syndrome in pediatrics, is a rare condition with high morbimortality. A follow up multidisciplinary team is necessary to minimize complications and optimize the intestinal rehabilitation. There are no gold standard guidelines for the management of this group of complex patients.

Role of minimally invasive surgery (MIS) in different sexual development (DSD).

Differences/disorders of sex development (DSD) are a heterogeneous group of congenital conditions in which the development of chromosomal, gonadal, or anatomical sex is atypical. Patients usually present during the newborn period but occasionally some cases remain unrecognized until later in infancy or even adolescence. Genital appearance, psychosocial support, sociocultural background, gender identity development, and genetic and biochemical analysis in addition to ethical and legal implications need to be considered when deciding on the appropriate treatment strategy.

[Perceptions and feelings of Argentine children regarding the COVID-19 quarantine].

The Subcommittee on the Rights of the Child and the Social Pediatrics Committee of the Sociedad Argentina de Pediatría carried out a fieldwork that allowed us to give a voice to our children and adolescents (NNA), during quarantine and social isolation mandatory imposed in our country by the global pandemic COVID-19. The objectives were to analyze the impact on their feelings, emotions and desires; evaluate and compare personal, family and social repercussions pre, intra and post quarantine; and identify support and containment needs. The results of the research allow us to affirm that children have been the most vulnerable and the least listened to.

End-tidal CO and transcutaneous CO : Are we ready to replace arterial CO in awake children?

Introduction: Arterial blood gas analysis (ABG) is the gold standard test for carbon dioxide measurement. End-tidal PCO (PetCO ) and transcutaneous PCO (PtcCO ) are noninvasive alternative methods.

Objective: To examine the use of PetCO and PtcCO as PaCO surrogates in awake children.

Long-acting Release Octreotide for Pediatric Upper Gastrointestinal Bleeding.

Introduction: Upper gastrointestinal bleeding (UGIB) secondary to portal hypertension (PHT), without endoscopic or surgical treatment options due to an ectopic or unidentified bleeding site or the patient's anatomic characteristics, is challenging in pediatric hepatology. The usual treatment in these cases includes intravenous Octreotide. Recently, the availability of long-acting release Octreo tide (OCT-LAR) for monthly intramuscular administration has become an interesting therapeutic alternative.

Feasibility of a pediatric long-term Home Ventilation Program in Argentina: 11 years' experience.

Background: Pediatric home ventilation (HV) has increased worldwide. A Home Ventilation Program (HVP) was started in the Pulmonary Department of the "Hospital de Pediatría Prof. Dr.

Pilomatrixomas in children: Report of 149 cases. A retrospective study at two children's hospitals.

A pilomatrixoma is a benign skin tumor common in children, which develops from the matrix cells of hair follicles. It presents as a nodule or tumor of approximately 0.5-3 cm in size, with calcium-like consistency, faceted edges, and blue erythematous overlying skin.

Extracorporeal membrane oxygenation in pediatric cardiovascular care: Experience of a center in Argentina.

Objective: To describe the results of extracorporeal membrane oxygenation in patients undergoing heart surgery and analyze the risk factors for morbidity and mortality.

Methods: Retrospective study conducted in cardiac patients under circulatory support. Outcome measures, diagnosis, surgery, Risk Adjustment for Congenital Heart Surgery (RACHS) score, implantation time, cannulation, length of support during stay, complications, survival, and follow-up were recorded.

Recommendations for the management of pediatric septic shock in the first hour (part two).

In 2016, the Surviving Sepsis Campaign and the National Institute for Health and Care Excellence (NICE) developed clinical practice guidelines for the management of pediatric septic shock. In 2017, the American College of Critical Care Medicine (ACCM) updated its recommendations for hemodynamic support of pediatric shock. Recognizing septic shock is critical, as well as an optimal, time-sensitive treatment.

Recommendations for the management of pediatric septic shock in the first hour (part one).

In the past two years, different organizations have updated their clinical practice guidelines for hemodynamic support in pediatric septic shock. The studies conducted in adults have questioned the initial management of sepsis in accordance to protocols based on achieving various goals. However, the usefulness of these protocols in children has been demonstrated.

Validation of the United Kingdom copy-number alteration classifier in 3239 children with B-cell precursor ALL.

Genetic abnormalities provide vital diagnostic and prognostic information in pediatric acute lymphoblastic leukemia (ALL) and are increasingly used to assign patients to risk groups. We recently proposed a novel classifier based on the copy-number alteration (CNA) profile of the 8 most commonly deleted genes in B-cell precursor ALL. This classifier defined 3 CNA subgroups in consecutive UK trials and was able to discriminate patients with intermediate-risk cytogenetics.