Cranial dermal sinuses in pediatrics: Presentation, diagnosis, complications, and management. Experience at a tertiary care children's hospital.

Introduction: A cranial dermal sinus is a tubular tract resulting from the incomplete separation of the epithelial ectoderm from the neuroectoderm which may lead to infectious complications. There have been isolated reports of this condition.

Objective: A cranial dermal sinus is a tubular tract resulting from the incomplete separation of the epithelial ectoderm from the neuroectoderm which may lead to infectious complications.

HIV-1 subtype F integrase polymorphisms external to the catalytic core domain contribute to severe loss of replication capacity in context of the integrase inhibitor resistance mutation Q148H.

Background: In prior studies, HIV-1 BF recombinants with subtype F integrases failed to develop resistance to raltegravir through the Q148H mutational pathway. We aimed to determine the role of subtype-specific polymorphisms in integrase on drug susceptibility, viral replication and integration.

Methods: Integrase sequences were retrieved from the Los Alamos Database or obtained from the Garrahan HIV cohort.

[Medicinal cannabis: beyond the myth, just another (herbal) drug].

This editorial briefly explores some of the distinctions between drugs and pharmaceuticals, alongside definitions regarding Cannabis sativa and its use in the context of a newly legal industry. Furthermore, medical cannabis is considered with regard to its toxicity, its social uses, its pharmacological activity, its legal paradoxes, and the scientific revolution sparked by the study of the endocannabinoid system, which has gained a great deal of relevance for modern physiology and pharmacology.

Making Visible HTLV Infection in a Non-endemic Area of Argentina.

In Argentina, the human T-cell lymphotropic virus type 1 (HTLV-1) infection has been documented mainly among blood banks with a prevalence of ~0.02-0.046% for Buenos Aires city, 0.

Impact of Deletions in the Prognosis of Childhood Acute Lymphoblastic Leukemia in Argentina.

An association of deletions in the IKZF1 gene (IKZF1del) with poor prognosis in acute lymphoblastic leukemia (ALL) has been demonstrated. Additional deletions in other genes (IKZF1plus) define different IKZF1del subsets. We analyzed the influence of IKZF1del and/or IKZF1plus in the survival of children with ALL.

Neuromuscular lordoscoliosis: an unusual response to post-operative halo-gravity traction.

Purpose: To report the results of prolonged post-operative halo-gravity traction in a patient in whom the surgery had to be interrupted unexpectedly and for whom subsequently specific clinical circumstances contraindicated completion of the surgical procedure.

Methods: The patient was a 15-year-old male with severe cervico-dorsolumbar lordoscoliosis who was being studied for associated diffuse axonal injury. He performed halo-gravity traction for 12 weeks.

[Experience in pediatric patients with COVID-19 during the first pandemic wave].

Coronavirus 2 infection has spread rapidly throughout the world. Most of the current publications describe different behavior between an adult and pediatric population, this last one is associated with less clinical severity. The purpose of this study was to analyze the process of care, the epidemiological and clinical features, the evolution and the use of resources in pediatric patients with SARS-Cov-2 infection, treated during the first pandemic wave, at the beginning of 2020.

Multisystem Inflammatory Syndrome Temporally Related to COVID-19 in Children From Latin America and the Caribbean Region: A Systematic Review With a Meta-Analysis of Data From Regional Surveillance Systems.

Background: With the emergence of the COVID-19 pandemic, increasing numbers of cases of the multisystem inflammatory syndrome in children (MIS-C) have been reported worldwide; however, it is unclear whether this syndrome has a differential pattern in children from Latin America and the Caribbean (LAC). We conducted a systematic review and meta-analysis to analyze the epidemiological, clinical, and outcome characteristics of patients with MIS-C in LAC countries.

Methods: A systematic literature search was conducted in the main electronic databases and scientific meetings from March 1, 2020, to June 30, 2021.

[Reinfection by SARS-CoV-2 in a child with history of multisystem inflammatory syndrome: a case report].

The multisystem inflammatory syndrome in children temporally related to COVID-19 (MIS-C) is a rare disease in pediatrics, which emerges related to the SARS-CoV-2 pandemic and was initially described in May 2020. Given the short time of evolution of this disease, little is known about the pathophysiology, prognosis, and the possibility of recurrence. We present a clinical case of a 12-year-old patient who presented symptoms compatible with MIS-C in January 2021, with good subsequent clinical evolution.

Use of sulthiame as add-on therapy in children with myoclonic atonic epilepsy: A study of 35 patients.

Purpose: The aim of this retrospective study was to evaluate efficacy and tolerability of sulthiame (STM) as add-on treatment in 35 patients with myoclonic atonic epilepsy (MAE) resistant to other antiseizure medications (ASMs) and/or non-pharmacological treatment.

Methods: Patients were selected according to the diagnostic definition of MAE and were resistant to at least four previous to ASM, alone or in combination. Neurologic examinations, brain magnetic resonance imaging, and repeated prolonged electroencephalography (EEG) or video-EEG studies as well as neurometabolic studies were performed in all cases.

HIV-1 pretreatment drug resistance in vertically infected children is associated with poor virological response to protease inhibitor (PI)-based first-line antiretroviral therapy (ART): results from a cohort study in Argentina.

Background: Increasing evidence from adult cohorts suggests an important role of HIV-1 pretreatment drug resistance (PDR) in ART failure, in spite of treatment being fully active according to baseline genotyping tests. Whether this is also true for children is unknown.

Methods: Virological and immunological parameters were longitudinally assessed in a group of 39 HIV-1 vertically infected children starting first-line lopinavir/ritonavir-based ART at a median of 5.

Molecular and functional study of pediatric patients with Niemann-Pick C in Argentina.

Niemann-Pick type C (NP-C) is a rare, autosomal recessive disorder. At least 95% of all the cases with this disease are due to mutations in the NPC1 gene. The clinical signs and symptoms of NP-C are classified into visceral, neurological and psychiatric.

Electroclinical Features and Long-term Seizure Outcome in Patients With Eyelid Myoclonia With Absences.

Background And Objectives: Eyelid myoclonia (EM) with absences (EMA) is a generalized epilepsy syndrome with a prognosis and clinical characteristics that are still partially undefined. We investigated electroclinical endophenotypes and long-term seizure outcome in a large cohort of patients with EMA.

Methods: In this multicenter retrospective study, patients with EMA with ≥5 years of follow-up were included.

Congenital Adrenal Hyperplasia and Ehlers-Danlos Syndrome.

Congenital adrenal hyperplasia (CAH) secondary to 21-hydroxylase deficiency is an autosomal recessive disorder. The 21-hydroxylase enzyme P450c21 is encoded by the gene located on chromosome 6p21.33 within the HLA major histocompatibility complex.

Vigabatrin-associated brain abnormalities on MRI and other neurological symptoms in patients with West syndrome.

Objectives: Report a series of children with West syndrome (WS) treated with vigabatrin (VGB) who developed characteristic MRI alterations. In the majority, these adverse events were asymptomatic; however, some of the patients developed movement disorders and acute encephalopathy.

Methods: This is a retrospective analysis of our epilepsy clinical and EEG database of 288 patients with WS seen between 2014 and 2020.

Spontaneous remission in juvenile myasthenia gravis: A cohort of 13 cases and review of the literature.

We describe the clinical course of patients with juvenile myasthenia gravis who experienced spontaneous remission and review the literature. This is a retrospective study of 13 patients with spontaneous remission from a cohort of 133 patients younger than 18-years-old. We compared several variables with potential prognostic value in patients with and without spontaneous remission.

Panayiotopoulos syndrome: Unusual clinical manifestations.

Purpose: We retrospectively analyzed the electroclinical features, treatment, and outcome of patients with Panayiotopoulos syndrome (PS) who presented with unusual clinical manifestations.

Method: A retrospective, descriptive, multicenter study was conducted evaluating 44 patients with PS who had seizures with an unusual semiology. Data from patients with PS seen at eight Argentine centers between April 2000 and April 2019 were collected.

Outcomes of SARS-CoV-2-Positive Youths Tested in Emergency Departments: The Global PERN-COVID-19 Study.

Importance: Severe outcomes among youths with SARS-CoV-2 infections are poorly characterized.

Objective: To estimate the proportion of children with severe outcomes within 14 days of testing positive for SARS-CoV-2 in an emergency department (ED).

Design, Setting, And Participants: This prospective cohort study with 14-day follow-up enrolled participants between March 2020 and June 2021.

Long-term use of cannabidiol-enriched medical cannabis in a prospective cohort of children with drug-resistant developmental and epileptic encephalopathy.

Objective: We report our findings regarding effectiveness, safety, and tolerability of cannabidiol (CBD)-enriched medical cannabis as add-on therapy in children with drug-resistant epileptic encephalopathies (DEEs) after a median follow-up of 20 months.

Methods: A prospective cohort study was conducted to assess effectiveness, safety, and tolerability of CBD-enriched medical cannabis oil added to standard antiseizure medications in children with drug-resistant DEE seen at a single center.

Results: Between October 2018 and March 2020, 59 patients were enrolled.

Impact of achondroplasia on Latin American patients: a systematic review and meta-analysis of observational studies.

Background: Achondroplasia (ACH), the most common form of disproportionate short stature, is caused by a pathogenic variant in the fibroblast growth factor receptor 3 gene. Recent advances in drug therapy for ACH have highlighted the importance of elucidating the natural history and socioeconomic burden of this condition. Recognition that there are many potential issues for the patient with ACH is the first step in planning cost-effective interventions in Latin America (LATAM), a vast geographic territory comprising countries with multicultural characteristics and wide socioeconomic differences.

AmpR is a dual regulator in Stenotrophomonas maltophilia with a positive role in β-lactam resistance and a negative role in virulence, biofilm and DSF production.

Stenotrophomonas maltophilia intrinsic resistance to β-lactams is mediated by two chromosomal β-lactamases, L1 and L2, whose induction depends on AmpR. Its quorum sensing (QS) signal, the diffusible signal factor (DSF), has a positive role in biofilm production, virulence and induction of β-lactamases. We hypothesized that AmpR has a role in virulence, biofilm production and QS system.

Teleconsultation during a pandemic. Experience of developmental pediatricians at Hospital de Pediatría "Prof. Dr. Juan P. Garrahan".

Teleconsultation is one of the components of telemedicine. Developmental pediatricians provide assessments and followup to children with developmental challenges. During the pandemic, due to movement and travel restrictions and the importance of continued care, pediatricians started working remotely with virtual consultations.

Rare tumors in pediatrics. First report in Argentina.

Introduction: Collaborative clinical trials have enlarged the knowledge base about pediatric tumors; however, this is not the case for rare tumors (RT).

Objective: To describe the prevalence, clinical characteristics, and course of RT in pediatric patients diagnosed at Hospital Garrahan.

Material And Methods: Descriptive, retrospective study of patients aged 0-18 years diagnosed with a RT and admitted between January 2007 and December 2017.