Recommendations on vaccination in children and adolescents with inborn errors of immunity according to the expanded Colombian immunization program.

In this manuscript, we carried out an exhaustive analysis of the global recommendations for immunization in inborn errors of immunity patients. We examined the mechanisms of action and types of vaccines, and we described the vaccines included in the Colombian immunization program together with the specific guidelines for immunization in patients with the most frequent inborn errors of immunity in Colombia. These recommendations were adjusted according to the severity and subclassifications of each immunodeficiency, considering variations in the immune response to offer evidencebased recommendations for vaccination in children with these conditions.

From phenotypic to molecular diagnosis: Insights from a clinical immunology service focused on inborn errors of immunity in Colombia.

Introduction: Inborn errors of immunity include a broad spectrum of genetic diseases, in which a specific gene mutation might alter the entire emphasis and approach for an individual patient.

Objective: To conduct a comprehensive analysis of the correlation between phenotypic and molecular diagnoses in patients with confirmed inborn errors of immunity at a tertiary hospital in Cali, Colombia.

Materials And Methods: We conducted a retrospective study in which we sequentially evaluated all available institutional medical records with a diagnosis of inborn errors of immunity.

The Influence of Sex, Fitness, BMI, and Cardiovascular Risk Factors on Cerebral Blood Velocity Responsiveness to Graded Exercise Testing in Middle-Aged Adults.

Mean middle cerebral artery velocity (MCAv) and the pulsatility index (PI), at rest and in response to exercise, are important markers of cerebrovascular health status in middle-aged adults, when vascular decline assumes substantial relevance. Thus, this study aimed to describe and compare the responses of MCAv and PI to incremental exercise. Two hundred and forty-eight volunteers (50-58 years, 55% women) completed a ramp test on a cycle-ergometer.

Clustering of obesogenic behaviours amongst 140 052 South American adolescents: a harmonized meta-analysis of national health surveys.

Background: Physical activity, sedentary behaviour, and diet synergically coexist (in different ways) within adolescents' lifestyles. Thus, understanding obesogenic behaviour patterns within populations is critical to promoting adolescents' health. This study investigates clustering patterns of adolescents' obesogenic behaviours across South American countries.

Factors associated with sarcopenia in a population of non-disabled older patients.

Background And Aim: To evaluate the association between sarcopenia and metabolic syndrome.

Patients And Methods: A case-control study.

Setting: Geriatric Care Clinic of Metepec (México).

Characteristics, outcomes and treatment patterns in acute myeloid leukemia patients 60 years or older in Colombia: a RENEHOC-PETHEMA study.

There is a limited information available on the clinical characteristics, treatment patterns and outcomes on older patients diagnosed with Acute Myeloid Leukemia (AML) in Latin-America. This multicenter retrospective study analyzed 269 patients over 60 years of age diagnosed with AML in Colombia, using data from RENEHOC-PETHEMA registry, from 2009 to 2023. The median age at diagnosis was 70 years (Range:60-98), 55% were men, 61% had an ECOG < 2, and 75.

Hearing loss in patients with Morquio A syndrome: A scoping review.

Background: Hearing impairment is a prevalent clinical feature in Morquio syndrome (mucopolysaccharidosis IVA or MPS IVA) patients, often presenting in diverse forms: conductive, sensorineural, or a combination known as mixed hearing loss. The mixed form entails a blend of both conductive and sensorineural elements, typically exhibiting a progressive trajectory. This scoping review aimed to comprehensively analyze available evidence pertaining to the pathophysiology, classification, epidemiology, and clinical management of hearing loss in individuals with MPS IVA.

Effectiveness of Manual Bronchial Clearance Techniques in the Treatment of Bronchiolitis.

Background: Bronchiolitis is a seasonal viral infection of the respiratory tract that causes numerous childhood hospitalizations annually. Treatments vary based on severity, with mild cases requiring fluids and moderate to severe cases involving hospitalization with oxygen therapy, bronchodilators, and chest physiotherapy. Manual bronchial clearance techniques differ between Anglo-Saxon and European schools, and their effectiveness remains a subject of debate.

Chronic Use of Benzodiazepine in Older Adults and Its Relationship with Dementia: A Systematic Review and Meta-Analysis.

Learning Objective: After participating in this CME activity, the psychiatrist should be better able to:• Explain current understanding of the relationship between chronic benzodiazepine use and dementia.

Background: Chronic use of benzodiazepines (BZ) for managing conditions such as anxiety disorders, depression, sleep disorders, and other chronic diseases is widespread; yet, there is considerable controversy regarding its potential links to dementia risk. This systematic review and meta-analysis aims to clarify this relationship by synthesizing and analyzing the available evidence to provide a clearer understanding of whether prolonged BZ use contributes to developing dementia.

Structural inequality linked to brain volume and network dynamics in aging and dementia across the Americas.

Structural inequality, the uneven distribution of resources and opportunities, influences health outcomes. However, the biological embedding of structural inequality in aging and dementia, especially among underrepresented populations, is unclear. We examined the association between structural inequality (country-level and state-level Gini indices) and brain volume and connectivity in 2,135 healthy controls, and individuals with Alzheimer's disease and frontotemporal lobe degeneration from Latin America and the United States.

Increased Early-Mortality in Children With Solid Tumors During the COVID-19 Pandemic in a Middle-Income Country.

Background: Measures to control COVID-19 transmission disrupted childhood cancer care. Data on the effects of the COVID-19 pandemic on childhood cancer mortality are lacking. This study describes the impact of the pandemic on childhood cancer early-mortality (≤ 24 months).

Normal echocardiographic findings in healthy pregnant women: A narrative review of the literature.

During pregnancy, significant physiological changes occur that result in cardiac remodeling and altered functional performance, though these are generally reversible postpartum. Pregnancy increases the cardiovascular system's demand, requiring substantial adaptations such as elevated cardiac output (CO), plasma volume, stroke volume (SV), and heart rate (HR), alongside a reduction in systemic vascular resistance (SVR) and mean arterial pressure. These adaptations, essential to meet the hemodynamic needs of both the mother and fetus, often differ from standard echocardiographic measurements used to evaluate cardiac function, making interpretation challenging.

Retinal Racemosal Hemangioma Associated with Intracranial Vascular Malformation (Wyburn -Mason Syndrome) - Case Report.

Purpose: To describe a case of Wyburn-Mason syndrome in a young child with initially declined treatment leading to delayed intervention.

Methodology: Case report.

Results: We report a 6-year-old boy with mild periocular trauma following a fall, who was incidentally found to have a retinal arteriovenous malformation (AVM) associated with a brain AVM, indicative of Wyburn-Mason Syndrome.

Diagnosis, treatment, and follow-up of patients with hypophosphatasia.

Introduction: Hypophosphatasia is a rare inherited systemic metabolic disorder, with an estimated prevalence in the severe forms of the disease of 1/100.000-1/300.000, that affects the typical architecture of bone, leading to defective mineralization during growth and remodeling.

International Society on Thrombosis and Haemostasis Clinical Practice Guideline for Treatment of Congenital Haemophilia-A Critical Appraisal.

Introduction: Evidence-based clinical practice guidelines drive optimal patient care and facilitate access to high-quality treatment. Creating guidelines for rare diseases such as haemophilia, where evidence does not often come from randomized controlled trials but from non-randomized and well-designed observational studies and real-world data, is challenging. The methodology used for assessing available evidence should consider this critical fact.

International cross-sectional survey on current and updated definitions of intra-abdominal hypertension and abdominal compartment syndrome.

Background: The Abdominal Compartment Society (WSACS) established consensus definitions and recommendations for the management of intra-abdominal hypertension (IAH) and abdominal compartment syndrome (ACS) in 2006, and they were last updated in 2013. The WSACS conducted an international survey between 2022 and 2023 to seek the agreement of healthcare practitioners (HCPs) worldwide on current and new candidate statements that may be used for future guidelines.

Methods: A self-administered, online cross-sectional survey was conducted under the auspices of the WSACS to assess the level of agreement among HCPs over current and new candidate statements.

Interobserver variation in the Parkland scale. Are we seeing the same thing?

Objective: The aim of this study was to analyze the reliability of agreement between surgeons when using the Parkland Grading Scale for Acute Cholecystitis (PGS-AC).

Methods: A total of 43 images taken out of videos of laparoscopic cholecystectomies (LCs) were collected, they were used to frame an online questionnaire that was sent to 18 surgeons and resident doctors who classified the images according to the Parkland scale criteria, followed by the evaluation of concordance between observers applying the Fleiss κ test.

Results: A global Fleiss' κ value of 0.

No sex differences in systemic metabolic responses to acute sprint interval training performed after an oral 75-g glucose load in adults with excess adiposity.

Background & Aims: Research exploring sex-based variations in responses to acute sprint interval training (SIT) remains limited. This study aimed to examine the impact of biological sex on the systemic metabolic response to SIT. We hypothesized that acute metabolic responses to SIT would differ between males and females.

Phenotype-Genotype Correlation in Morquio A Syndrome: Protocol for a Meta-Analysis.

Background: Mucopolysaccharidosis type IVA (MPS IVA), also known as Morquio A syndrome, is a rare lysosomal storage disease characterized by autosomal recessive inheritance of mutations in the N-acetylgalactosamine-6-sulfatase (GALNS) gene. This leads to a deficiency of the GALNS enzyme, causing the accumulation of glycosaminoglycans in tissues. Morquio A syndrome primarily affects the skeletal system and joints but can also impact various organs, resulting in symptoms such as hearing and vision loss, respiratory issues, spinal cord compression, heart diseases, and hepatomegaly.

Concordance between clinical diagnoses and autopsies anatomopathological findings at a tertiary-level university hospital.

Introduction: A clinical autopsy is a fundamental diagnostic tool for confirming the diagnosis of diseases of public health interest. However, the clinical-pathological concordance has not been evaluated.

Objective: To determine the concordance between clinical diagnoses and anatomopathological findings from autopsies conducted at a tertiary-level hospital institution.

Diagnostic Accuracy of Optic Nerve Sheath Diameter Measurement by Ultrasonography for Noninvasive Estimation of Intracranial Hypertension in Traumatic Brain Injury: A Systematic Review and Meta-Analysis.

Background And Objectives: Intracranial hypertension (IH) is associated with an unfavorable outcome in traumatic brain injury (TBI), and management strategies guided by intracranial pressure monitoring improve prognosis. Owing to the limitations of using invasive devices, measurement of optic nerve sheath diameter (ONSD) by ultrasonography is an alternative noninvasive method. However, its accuracy has not been validated in patients with TBI, so we aim to determine the diagnostic accuracy of measuring ONSD by ultrasonography in patients with TBI to estimate IH, compared with invasive monitoring.

Using different anthropometric indices of obesity as predictors for type 2 diabetes mellitus in older adults.

Background And Aims: There is some evidence that anthropometric measurements can be associated with the risk of type 2 diabetes mellitus (T2DM). Nevertheless, there is no comprehensive consensus on which anthropometric index is the best for evaluating the risk and predicting T2DM, especially in older adults. For this reason, we compared the performance of six indices for detecting T2DM in a population of Colombian older adults.