Pulmonary function and body composition parameters of children with cystic fibrosis from a hight complexity institution between 2015 and 2018.

Background: An adequate nutritional status in patients with cystic fibrosis correlates with higher survival and better pulmonary function. Body mass index (BMI) is an anthropometric indicator with independent association with pulmonary function, and it can hide alterations of nutritional status in cystic fibrosis.

Objective: To determine the relationship of lung function with BMI and body composition parameters in children with cystic fibrosis in a Hight Complexity institution between 2015 and 2018.

Modified NEOMOD score as a neonatal mortality prediction tool in a medium-income country: A validation diagnostic test study.

Background And Aims: Multiple organ dysfunction (MOD) is a potentially reversible physiological disorder that involves two or more systems. Modified NEOMOD (Neonatal Multiple Organ Dysfunction score) scale could be a useful instrument to measure MOD and predict mortality. Our aim was to validate modified NEOMOD in patients from a neonatal intensive care unit (NICU) of a middle-income country.

Characterization of a group of children with eosinophilic esophagitis in Cali, Colombia.

Purpose: Eosinophilic esophagitis (EoE) is an immunologically mediated chronic disease of the gastrointestinal tract. The objective of this study was to clinically and demographically describe a child population with EoE diagnosed in a high-complexity hospital in Cali, Colombia.

Methods: A retrospective study was carried out.

Urinary KIM-1 is not correlated with gestational age among 5-year-old children born prematurely.

Background: Preterm birth is associated with decreased nephron endowment. Currently, there is no reliable non-invasive biomarker to identify or monitor decreased nephron number in at-risk patients. Urinary Kidney Injury Molecule-1 (KIM-1) is a biomarker of acute and chronic renal injury.

Systemic Bevacizumab for Recurrent Respiratory Papillomatosis: A Scoping Review from 2009 to 2022.

Background: Respiratory recurrent papillomatosis (RRP) is a fatal disease with no known cure. In severe RRP cases, systemic bevacizumab (SB) could be used as adjuvant therapy.

Objective: This study aims to determine the extent and type of evidence in relation to the clinical outcomes of RRP after SB treatment.

B-cell activating factor (BAFF) and its receptors' expression in pediatric nephrotic syndrome is associated with worse prognosis.

Aim: Immune pathogenesis of nephrotic syndrome (NS) is not completely understood. We aimed to evaluate the expression of B-cell activating factor (BAFF) and its receptors in renal samples from pediatric NS patients and its relationship with renal function survival.

Materials And Methods: We conducted an ambispective study on 33 patients with pediatric NS.

20 years of experience with the Fontan procedure: characteristics and clinical outcomes of children in a tertiary referral hospital.

Introduction: Without participating in a contractile chamber, the Fontan procedure seeks to create a separation of oxygenated and deoxygenated blood in patients with univentricular heart, reducing the risks of long-term hypoxemia and improving their survival. This study describes the clinical outcomes of children undergoing the Fontan procedure between 2000 and 2020 in a tertiary referral hospital care centre in southwestern Colombia.

Materials And Methods: A retrospective observational descriptive study.

Gigantomastia as a Cause of Pulmonary Hypertension.

Reduction mammaplasty is the gold standard treatment for gigantomastia. We report one female patient with juvenile gigantomastia associated with severe pulmonary hypertension where her pulmonary pressure decreased significantly after the surgery, improving her quality of life. A 22-year-old female patient with gigantomastia since 10 years old, tricuspid regurgitation, and pulmonary thromboembolism antecedent was admitted to the emergency department.

Demographic and clinical characterization of pediatric group patients with inborn errors of the immune system in a Colombian tertiary hospital.

Introduction: In recent decades, there has been a growing increase in the diagnosis of patients with inborn errors of the immune system, formerly known as primary immunodeficiency disorders (PIDs). Timely diagnosis remains a challenge due to low clinical suspicion and poor education on the subject. It is estimated that between 70% and 90% of these pathologies remain underdiagnosed in our environment.

Treatment of post-transplant recurrent FSGS in children using plasmapheresis and augmentation of immunosuppression.

Background: Up to 60% of pediatric renal transplant recipients with end-stage renal disease due to primary focal and segmental glomerulosclerosis (FSGS) may develop recurrent disease. Such recurrence is associated with poor prognosis if no remission is achieved. We report a single center experience with a protocol based on plasmapheresis and increased immunosuppression that resulted in a high long-lived remission rate.

Pediatric hemophagocytic lymphohistiocytosis: A rarely diagnosed entity in a developing country.

Background: Hemophagocytic lymphohistiocytosis (HLH) is an exaggerated inflammatory reaction secondary to a host's inadequate immune response causing a self-perpetuating loop of altered regulation. Signs and symptoms of HLH are compatible with other common diseases and are nonspecific. Underdiagnosis makes it difficult to estimate the real incidence of HLH, especially in developing countries.

Renal volume of five-year-old preterm children are not different than full-term controls.

Objective: In previous studies, smaller renal volumes were reported in prematurely born infants, however, these renal volumes were not corrected for body surface area, the main determinant of renal size. Given the rapid growth of the renal cortex after premature birth, the authors hypothesized that corrected volumes would not differ from healthy controls.

Methods: Ambispective cohort study with prospective follow-up of prematurely born babies in a large specialized center and retrospectively recruited healthy control group.

Skin manifestations in pediatric patients with primary immunodeficiency diseases (PIDs) in a tertiary care hospital in Colombia.

Background: The current literature describes the characteristics of some skin manifestations in the context of primary immunodeficiency diseases (PIDs), also known as inborn errors of the immune system. However, there are hardly any data on the epidemiological trends of skin manifestations and PIDs in Latin America (LA). We aimed to describe the characteristics of patients with skin manifestations and the diagnosis of a PID treated at a tertiary hospital in Colombia.

Low agreement between kidney volume and kidney length z-scores.

Background: Pediatric nephrologists use kidney length and kidney volume z-scores to longitudinally assess normal nephron endowment. However, most radiologists only report kidney length. Agreement between kidney length and kidney volume z-scores in children has been understudied.

Methylation Status of and in Small for Gestational Age Children with and without Catch-up Growth.

Objective: In small for gestational age (SGA) children, catch-up growth could be influenced by methylation of several genes involved in metabolism. Epigenetics may influence the development of metabolic diseases in adulthood. To compare the methylation of leptin (), glucagon-like peptide-2 receptor (), insulin receptor substrate-2 () in SGA patients with and without catch-up growth.

Prenatal Diagnosis of Pfeiffer Syndrome Patient with FGFR2 C.940-1G>C Variant: A Case Report.

Background: Pfeiffer syndrome (PS) is an autosomal dominant disorder caused by mutations in fibroblast growth factor receptor FGFR1 and FGFR2 genes, occurring in approximately 1:100,000 live births. PS has a wide range of clinical expression and severity, so early prenatal diagnosis is difficult and genetic counseling is desirable. We describe a PS newborn with her ultrasound and molecular studies.

[Ex-Utero Intrapartum Treatment for airway management in congenital giant neck masses].

Introduction: Congenital head and neck masses are associated with perinatal asphyxia and brain injury, increasing the risk of death. The EXIT (Ex Utero Intrapartum Treatment) technique con sists of ensuring the newborn's airway while is still receiving placental support. This technique has not been standardized in developing countries.

Renal length z-score for the detection of dysfunction in children with solitary functioning kidney.

Aim: To evaluate whether renal length z-scores predict renal dysfunction in children with a solitary functioning kidney (SFK).

Methods: In a single-centre retrospective cohort of children with SFK, we correlated body mass index z-scores, extracellular volume and lean body mass to renal length z-scores. We grouped these z-scores to other markers of renal dysfunction (proteinuria, hypertension, extracellular volume and abnormal estimated glomerular function rate [eGFR]) and analysed renal length z-score with multivariate analysis, receiver-operated characteristics (ROC) plots and Youden's index to determine an appropriate cut-off.

Lower prevalence of aortic dilatation among preemptive pediatric renal transplant recipients - A cross-sectional cohort study.

Background: Aortic dilatation is a cardiovascular complication in pediatric renal transplant recipients and may have an increased risk of aortic dissection, aortic rupture, and death. Studies failed to show an association between blood pressure and aortic dilatation; however, 24-hours ambulatory blood pressure monitoring (ABPM) was not performed. There was also no comparison between preemptive transplantation and dialysis.