[Pediatric telecardiology experience in the COVID-19 pandemic context].

Introduction: The COVID-19 pandemic has caused a global impact on public health services. Using new strategies through telehealth for the management of patients with congenital heart disease was the challenge.

Objective: To describe the experience in telecardiology, and the strategies implemented during the pandemic.

[Argentinean Consensus on the Diagnosis and Treatment of Niemann- Pick Disease Type C].

Niemann-Pick type C (NPC) is a disorder of the lysosomal metabolism due to biallelic pathogenic variants in NPC1 or NPC2. Intracellular deposit of lipids, mainly unesterified cholesterol, gives rise to oxidative damage in several tissues, mainly neurons, spleen and liver. This, in turn, is associated with a myriad of neurological / psychiatric and visceral symptoms, with wide variability in age of presentation, from prenatal / neonatal to adult-onset forms of the disease.

Outcomes of Infants and Young Children With Relapsed Medulloblastoma After Initial Craniospinal Irradiation-Sparing Approaches: An International Cohort Study.

Purpose: Infant and young childhood medulloblastoma (iMB) is usually treated without craniospinal irradiation (CSI) to avoid neurocognitive late effects. Unfortunately, many children relapse. The purpose of this study was to assess salvage strategies and prognostic features of patients with iMB who relapse after CSI-sparing therapy.

Pseudomonas putida bacteremia in pediatric patients: A case series study.

Introduction: Bacteria of the genus Pseudomonas act as opportunistic pathogens. Pseudomonas putida has been considered a pathogen of low virulence and susceptible to multiple antibiotics, but in recent years resistant strains have emerged. The objective of this study is to describe the clinical characteristics, evolution and antibiotic resistance of P.

Clinical and molecular characteristics of pediatric low-grade glioma complicated with ventriculo-peritoneal shunt related ascites.

Introduction: Ventriculo-peritoneal shunt (VPS) related ascites is a rare complication of pediatric low grade gliomas (pLGG). Physiopathology of this complication is not fully understood and there is paucity of data regarding the molecular profile of pLGG gliomas complicating with ascites and the optimal management of this unusual event.

Methods: International multi-institutional retrospective analysis of patients diagnosed with BRAF altered pLGG and ascites arising as a complication of VPS.

[Targeted therapies with BRAF inhibitors for pediatric low- and high-grade gliomas with BRAFv600e mutation. Prof. Dr. Juan P. Garrahan Hospital experience].

The BRAFV600E point mutation plays a key role in the tumorigenesis of many gliomas. Inhibiting its product is part of the innovative therapies emerging in recent years. Knowing the role of these treatments is essential.

Management of ventriculomegaly in pediatric patients with syndromic craniosynostosis: a single center experience.

Introduction: Management of ventriculomegaly in pediatric patients with syndromic craniosynostosis (SC) requires understanding the underlying mechanisms that cause increased intracranial pressure (ICP) and the role of cerebrospinal fluid (CSF) in cranial vault expansion in order to select the best treatment option for each individual patient.

Methods: A total of 33 pediatric patients with SC requiring craniofacial surgery were retrospectively evaluated. Cases of nonsyndromic craniosynostosis and shunt-induced craniosynostosis were excluded.

[Hypercalcemia in a pediatric patient with ovarian dysgerminoma: A case report].

Hypercalcemia associated with malignant tumors is a rare entity in pediatrics (0.5-1.3 % of pediatric cancers).

[Mycobacterium bovis infection in an adolescent with prolonged febrile syndrome].

Bovine tuberculosis is an infectious, zoonotic disease, caused by Mycobacterium bovis. The bovine is the primary host. Other species can be infected (pigs, goats, horses, etc).

Telehealth for the Care of Complex Chronic Pediatric Patients in Latin America and the Caribbean: Narrative Review.

Purpose Of Review: This article is a review of the most significant productions on the care of pediatric patients with complex and/or rare chronic diseases in the field of telehealth, produced in Latin America and the Caribbean. It inquires about accessibility to health, its efficiency for the care of chronic and rare diseases, its acceptability/resistance by users (health teams and patients), and the standardization-evaluation of telecare processes.

Recent Findings: The formation of reference networks for patients with chronic and rare diseases.

[Recurrent pancreatitis in children: Case series].

Recurrent pancreatitis (RP) occurs in children between 15- 35 % of the cases. To determine the etiology and outcome of RP in children a descriptive, cross-sectional cohort study was conducted in children under 18 years of age with RP seen at Hospital Garrahan between 2008-2016. Of 10 patients with RP, 90 % were girls.

[Autoimmune pancreatitis in children. Clinical case].

Recurrent pancreatitis occurs in children between 15 and 35 % of the cases. It is defined as two or more separate episodes of acute pancreatitis with normalization of the pancreatic enzymes between episodes. One of the causes is autoimmune pancreatitis.

High prevalence of BRAF in patients with cholestasis, sclerosing cholangitis or liver fibrosis secondary to Langerhans cell histiocytosis.

Targeted therapies with MAPK inhibitors have proven to modulate the clinical manifestations of patients with Langerhans cell histiocytosis (LCH). We explored the presence of BRAF mutation in our cohort of patients with LCH and cholestasis, sclerosing cholangitis, or liver fibrosis that presented resistance to chemotherapy. The BRAF mutation was detected either in the diagnosis (skin and bone) or liver biopsy in our cohort of 13 patients.

Adherence to oral antineoplastic agents in pediatric oncology. A multicenter study.

Objective: To determine the adherence to oral maintenance medication among pediatric cancer patients and know their beliefs about medications.

Population And Methods: Information was obtained from parents, adolescents, and oncologists from six public children's hospitals of Argentina during 2018 and 2019. Questionnaires on adherence (Simplified Medication Adherence Questionnaire) and beliefs about medication (Beliefs About Medicines Questionnaire) were administered.

[Infantile intestinal fibrosarcoma. Case report with digestive bleeding in an infant].

Infantile fibrosarcoma is a rare tumor, belonging to the nonrhabdomyosarcoma, soft tissue sarcoma. It is mostly presented in infants, most commonly involving the extremities. We report a 5-month-old boy, presenting with digestive bleeding since the age of 3 months, initially diagnosed as cow's milk allergy, with a torpid evolution and anemia.

[National survey of pediatric residences in Argentina].

Introduction: Currently, there is no complete registry of pediatric residences in Argentina.

Objectives: To identify all pediatric residences in Argentina. To establish a situation diagnosis and a unified registry.

[Recommendations for the diagnosis and treatment of persistent postsurgical symptoms in Hirschsprung disease].

Hirschsprung disease is characterized by the lack of migration of intrinsic parasympathetic ganglia from neural crest and consequently absence of them at varying length of the bowel, resulting in functional obstruction. The incidence is 1 per 5000 births. After surgery, short term and long term comorbidity commonly occurs.

Outcomes of BRAF V600E Pediatric Gliomas Treated With Targeted BRAF Inhibition.

Purpose: Children with pediatric gliomas harboring a BRAF V600E mutation have poor outcomes with current chemoradiotherapy strategies. Our aim was to study the role of targeted BRAF inhibition in these tumors.

Patients And Methods: We collected clinical, imaging, molecular, and outcome information from patients with BRAF V600E-mutated glioma treated with BRAF inhibition across 29 centers from multiple countries.

Ten years of the neonatal respiratory extracorporeal membrane oxygenation program in an Argentinian public hospital.

Introduction: Extracorporeal membrane oxygenation (ECMO) is a highly invasive rescue treatment for critically-ill patients. The objective of the study was to show the outcomes of patients treated with ECMO in the neonatal intensive care unit of Hospital de Pediatria Juan P. Garrahan in the first 10 years of the program, and to analyze the risk factors associated with mortality on ECMO.

Pediatric residency program accreditation process by the Council for the Accreditation of Education Institutions.

Introduction: Postgraduate medical education as part of a residency system is the best model for specialist training. The accreditation of residency programs entails a harmonization and standardization process. It defines a common and reproducible basis to ensure high-quality training and universal curricular guidelines.

Congenital diaphragmatic hernia: postnatal predictors of mortality.

Introduction: Congenital diaphragmatic hernia (CDH) prevalence is low while its associated morbidity and mortality rates are high. Postnatal prognostic factors on the first day of life are useful for predicting the outcome.

Objectives: To determine the mortality predictive ability of postnatal echocardiographic, clinical, and biochemical factors among newborn infants with CDH in their first day of life.

[Serious neurological compromise due to vitamin B12 deficiency in infants of vegan and vegetarian mothers].

Vitamin B12 deficiency is one of the most serious complications of vegetarianism and its variants. Infants born to vegan mothers are at greater risk of serious deficiency, being more vulnerable to their effects. B12 deficiency is not usually suspected by the pediatrician in healthy infants with neurological symptoms.

[Multidisciplinary teamwork in the clinical application of the ketogenic diet].

The ketogenic diet is an established, effective and well-tolerated treatment in refractory epilepsy. This paper provides practical information on the administration of ketogenic diet. It goes over the standardized protocols and the new ones that allow to broaden the scope regarding the clinical management of the ketogenic diet.