Tuberculosis Disease in Immunocompromised Children and Adolescents: A Pediatric Tuberculosis Network European Trials Group Multicenter Case-control Study.

Background: In high-resource settings, the survival of children with immunocompromise (IC) has increased and immunosuppressive therapies are increasingly being used. This study aimed to determine the clinical characteristics, performance of diagnostic tools, and outcome of IC children with tuberculosis (TB) in Europe.

Methods: Multicenter, matched case-control study within the Pediatric Tuberculosis Network European Trials Group, capturing TB cases <18 years diagnosed 2000-2020.

Juvenile idiopathic arthritis in the young adult. Methodology, objectives, and initial data from the JUVENSER registry.

Objectives: To describe the methodology, objectives, and initial data of the registry of young adult patients diagnosed with Juvenile Idiopathic Arthritis (JIA), JUVENSER. The main objective of the project is to know the sociodemographic and clinical characteristics, and disease activity of patients with JIA reaching the transition to adulthood.

Material And Method: Longitudinal, prospective, multicentre study, including patients between 16 and 25 years old, with a diagnosis of JIA in any of its categories.

Neuroendoscopic septostomy in unilateral and bilateral ventricular hydrocephalus.

Purpose: This study describes the results of septostomy (SPT) in terms of success and analysis of follow-up in a series of pediatric patients diagnosed with unilateral ventricular hydrocephalus (UHV) and biventricular hydrocephalus (BVH).

Methods: A total of 29 pediatric patients diagnosed with UVH or BVH were included in this study. In UVH, a neuroendoscopic SPT was performed (sometimes accompanied by NEFPFMO).

Fibrinogen Deficiency with Thrombotic Manifestations.

Unlabelled: Fibrinogen deficiencies are very rare. Qualitative fibrinogen deficiencies (dysfibrinogenaemia and hypodysfibrinogenemia) are functional disorders that can present with both haemorrhagic symptoms and with thrombotic phenomena as unique and paradoxical manifestation. We present the case of a 77-year-old man being investigated for a partially thrombosed abdominal aortic aneurysm as well as an ischaemic stroke 20 years previously.

Endoscopic ultrasonography-guided celiac plexus neurolysis in patients with unresectable pancreatic cancer: An update.

Pancreatic cancer produces disabling abdominal pain, and the pain medical management for pancreatic cancer is often challenging because it mainly relies on the use of narcotics (major opioids). However, opioids often provide suboptimal pain relief, and the use of opioids can lead to patient tolerance and several side effects that considerably reduce the quality of life of pancreatic cancer patients. Endosonography-guided celiac plexus neurolysis (EUS-CPN) is an alternative for pain control in patients with nonsurgical pancreatic cancer; EUS-CPN consists of the injection of alcohol and a local anesthetic into the area of the celiac plexus to achieve chemical ablation of the nerve tissue.

Prenatal diagnosis of rhombencephalosynapsis: neuroimaging features and severity of vermian anomaly.

Objectives: To describe the prenatal neuroimaging spectrum of rhombencephalosynapsis (RES) and criteria for its classification according to the severity of vermian anomaly.

Methods: In this multicenter retrospective study of fetuses with RES between 2002 and 2020, the medical records and brain ultrasound and magnetic resonance images were evaluated comprehensively to determine the severity of the vermian anomaly and the presence of associated brain findings. RES was classified, according to the pattern of vermian agenesis and the extent of the fusion of the hemispheres, as complete RES (complete absence of the vermis) or partial RES (further classified according to the part of the vermis that was missing and, consequently, the region of hemispheric fusion, as anterior, posterior, severe or mixed RES).

Congenital dyserythropoietic anemia types Ib, II, and III: novel variants in the CDIN1 gene and functional study of a novel variant in the KIF23 gene.

Congenital dyserythropoietic anemias (CDA) are disorders characterized by ineffective erythropoiesis and morphological anomalies in erythrocytes and erythroblasts. The purpose of this study is to identify the gene variants in patients diagnosed with CDA. We analyzed five unrelated patients and two siblings with a targeted panel of genes to CDA: CDAN1, CDIN1, SEC23B, KIF23, KLF1, and GATA1 genes.

Tuberculosis in a Spanish cohort of children living with HIV: the CHOTIS study (Childhood HIV & TB study).

Tuberculosis (TB) is the leading opportunistic infection in children with human immunodeficiency virus (HIV), but is uncommon in low prevalence regions. We aim to describe the changing epidemiology and clinical presentation of TB-HIV co-infection in a cohort of HIV-infected children in Spain. Children diagnosed with TB between 1995 and 2016 in the paediatric HIV cohort were identified.

Healing criteria: How should an episode of benign paroxistic positional vertigo of posterior semicircular canal's resolution be defined? Prospective observational study.

Objectives: To compare the outcome of the Epley maneuver (EM) in benign paroxysmal positional vertigo of the posterior canal (CSP-BPPV) depending on the definition used for recovery.

Design: Multicenter observational prospective study.

Setting: Otoneurology Units of 5 tertiary reference hospitals.

Linear Unilateral Basaloid Follicular Hamartoma Following Blaschko's Lines: Two Case Reports and Review of the Literature.

Basaloid follicular hamartoma (BFH) is a rare follicular malformation characterized by variable clinical presentations and identical histopathologic features. We present the cases of a 3-month-old boy and an 8-year-old boy with linear unilateral BFH. To the best of our knowledge, only 14 cases of linear unilateral BFH have been described in the English-language literature.

Epidemiology of vestibular disorders in the otoneurology unit.

Introduction And Objectives: The set of symptoms relating to disorders of the balance system are common in the general population. However, there are few studies quantifying the frequency of onset of the various vestibular disorders that present in specialist otoneurology units in the Spanish population. The aim of this study is to establish the epidemiology of vestibular disorders, their form of presentation, and the expected care burden in a specialist otoneurology clinic.

Factors Associated with Stunting among Children Aged 0 to 59 Months from the Central Region of Mozambique.

The objective of this study was to identify the major socio-demographic, health, and environmental determinants of stunting among children aged 0-59 months from the Tete province (Mozambique) and offering useful information for future healthcare strategies and interventions. A case-control study was conducted among 282 (162 boys; 120 girls) children under five years of age from the central region of Mozambique between 1 May and 3 June 2014. Children with stunting (HAZ < -2 SD according to the WHO Child Growth Standards in 2006) were considered as cases and those who had a -score < -2 SD were considered as controls.

Cochlear Implant Evaluation: Prognosis Estimation by Data Mining System.

Objective: Prediction of speech recognition (SR) and quality of life (QoL) outcomes after cochlear implantation is one of the most important challenges for otologists. By sifting through very large amounts of data, data mining reveals trends, patterns, and relationships that might otherwise have remained undetected. There are identifiable pre-implantational factors that condition the cochlear implantation outcome.

Metabolic Syndrome in Children and Adolescents Living with HIV.

Background: Metabolic syndrome (MetS) is considered an independent risk factor for developing cardiovascular disease. It is well known that the prevalence of metabolic disorders have increased in pediatric HIV-infected children. The objective of this study is to assess the prevalence and characteristics of MetS in HIV-infected children and adolescents in Spain.

Relationship between vitamin D deficiency and visually evoked potentials in multiple sclerosis.

Objective: To evaluate the possible relationship between serum 25-OH vitamin D levels and visually evoked potentials (VEP) in patients with multiple sclerosis (MS), residents in the south zone of Gran Canaria.

Material And Methods: The study included 49 patients with MS, on whom 25-OH-vitamin D was determined, along with VEP, and a neurological examination to determine incapacity. Clinical variables, such as a history of optic neuritis were recorded.

Description of the novel HLA-DQB1*02:02:01:02 allele in a Spanish individual.

HLA-DQB1*02:02:01:02 has an intron sequences coming from a combination of DQB1*02:01:01 and DQB1*02:02:01:01.

Reduction in posterior semicircular canal gain by age in video head impulse testing. Observational study.

Introduction: and objectives: In-depth assessment of the vestibulo-ocular reflex with the new video systems available provides information on various previously-unidentified characteristics in patients with peripheral vestibular disorder. The aim of this work is to quantify how often pathological gain (uni- or bilateral) in the posterior semicircular canal (PSC) gain appears in video head-impulse testing (vHIT) with preservation of the gain in the rest of the semicircular canals, and to analyse the relation of this frequency with patient age and/or diagnosis.

Methods: This was a prospective observational study on adults referred to our unit between June 2012 and February 2014.

Audio-vestibular signs and symptoms in Chiari malformation type i. Case series and literature review.

Introduction: Chiari malformation is an alteration of the base of the skull with herniation through the foramen magnum of the brain stem and cerebellum. Although the most common presentation is occipital headache, the association of audio-vestibular symptoms is not rare. The aim of our study was to describe audio-vestibular signs and symptoms in Chiari malformation type i (CM-I).