Association between serum albumin levels at admission and clinical outcomes in pediatric intensive care units: a multi-center study.

Background: Albumin, a vital component in regulating human blood oncotic pressure, plays an important role in the prediction of prognosis in pediatric patients.Previous research identified significant differences in serum albumin levels of healthy and critically ill children.

Methods: The present study aims to investigate the correlation between albumin levels measured during pediatric intensive care unit(PICU) admission and clinical outcomes.

A Novel Variant of the PIK3R1 Gene Mutation Associated With SHORT Syndrome and Agammaglobulinemia.

Primary immunodeficiencies are disorders of the immune system often caused by mutations of genes required for lymphocyte development. Phosphoinositide-3-kinase regulatory subunit 1 (PIK3R1) gene mutations are associated with SHORT syndrome, a rare multisystem disease. The name stands for Short stature, Hyperextensibility, Ocular depression, Rieger anomaly and Teething delay.

A Unique Mutation in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gene Causing Cystic Fibrosis in a Pakistani Child: A Case Highlighting the Need for More Awareness.

Cystic fibrosis (CF) is a recessively inherited disease most commonly seen in Caucasians. The mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene are responsible for the condition, and to date, more than 2000 mutations have been published in the literature. The most common mutation worldwide is F508del.

Effect of intravenous lipid therapy in critically ill pediatric patients with calcium channel blocker toxicity.

Background: Overdose with calcium-channel blockers (CCBs) still maintain their importance with a high lethality rate after exposure. We report the intravenous lipid emulsion therapy (ILE) therapy in our CCB overdose patients.

Methods: We retrospectively analyzed the records of 6 patients with CCB intoxication from Batman Training and Research Hospital PICU between March 2021 and September 2022.

A rare presentation of childhood interstitial lung disease attributed to KDM3B gene mutation: a case report.

Childhood Interstitial Lung Disease (chILD) encompasses various respiratory conditions affecting children's lung airspaces and tissues, with diverse causes. One rare cause involves structural vascular changes. We describe a case of a 10-year-old boy diagnosed with chILD who exhibited specific dysmorphic features, developmental delay, and intellectual disability.

Indications, Efficacy, and Complications of Pediatric Bronchoscopy: A Retrospective Study at a Tertiary Center.

Background Bronchoscopy is an essential procedure for evaluating, diagnosing, and treating pediatric respiratory diseases. In this study, we demonstrate the indications and contraindications of bronchoscopy done in a tertiary referral hospital, Al Qassimi Woman's and Children's Hospital (AQWCH) in Sharjah, United Arab Emirates (UAE), in order to achieve better service. This study aims to evaluate patients' characteristics, diagnostic and therapeutic indications, and complications of bronchoscopy.

Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C) in a Middle Eastern patient cohort.

Objective: This is a comprehensive characteristic study of Kawasaki disease (KD) and Multi system inflammatory syndrome in children (MIS-C) in the Middle East that creates a formula to differentiate between the two.

Methods: We conducted a descriptive comparative study of KD and MIS-C in the United Arab Emirates. Retrospective MIS-C and KD cohorts were recruited between January 2017 until August 2021.

A Case of Coexistent Sarcoidosis and Tuberculosis: A Diagnostic Dilemma.

Sarcoidosis and tuberculosis (TB) are chronic granulomatous diseases with similar radiological, clinical, and histopathological presentations. Although rare, both conditions can coexist together. Case reports of concomitant incidence have been published in the literature.

Beyond the Local Basic Panel: Full CFTR Gene Analysis Identifies Novel CF Mutation Missed on Standard Testing in an Arabic Child.

Cystic fibrosis (CF) is an autosomal recessive disease caused by different mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. It is the most common inherited disorder in the Caucasian population, with around 2000 mutations identified for the CFTR gene. The precise prevalence of CF in Arab countries remains unknown, with the prevalence of F508 del found to be a common type with other endemic mutations.

A Retrospective Chart Review of Pediatric Complicated Community-Acquired Pneumonia: An Experience in the Al Qassimi Women and Children Hospital.

Background Community-acquired pneumonia (CAP) is one of the most common global health issues. Even though many vaccinations and new diagnostic tools are available, CAP has a higher mortality rate, especially in children less than five years of age. Complicated CAP (CCAP) in a healthy child is a severe disease characterized by a combination of local complications, such as parapneumonic effusion (PPE), empyema (EMP), necrotizing pneumonia (NP), abscess, pneumothorax, and bronchopleural fistula, and systemic complications, such as bacteremia, metastatic infection, multiorgan failure, acute respiratory distress syndrome, disseminated intravascular coagulation, and, rarely, death.

Nonspecific Interstitial Pneumonitis in a Child Associated With Hashimoto Thyroiditis.

Interstitial lung disease (ILD) is a rare disease defined as a specific type of chronic fibrosing interstitial pneumonitis whose effects are limited to the lung. Nonspecific interstitial pneumonia (NSIP) was defined as a histopathological form that can be seen in the presence of large different clinical and radiological features. The exact role of thyroid hormone in the pathogenetic mechanism of idiopathic interstitial pneumonitis (IIP) is unclear.

Bayesian Dynamic Borrowing of Historical Information with Applications to the Analysis of Large-Scale Assessments.

The purpose of this paper is to demonstrate and evaluate the use of Bayesian dynamic borrowing (Viele et al, in Pharm Stat 13:41-54, 2014) as a means of systematically utilizing historical information with specific applications to large-scale educational assessments. Dynamic borrowing via Bayesian hierarchical models is a special case of a general framework of historical borrowing where the degree of borrowing depends on the heterogeneity among historical data and current data. A joint prior distribution over the historical and current data sets is specified with the degree of heterogeneity across the data sets controlled by the variance of the joint distribution.

Are platelet indices promising ratios for predicting pediatric septic shock prognosis?

Objectives: The aim of the present study was to determine the prognostic value of thrombocytopenia, platelet indices (MPV/PLT and PDW/PLT) in children with septic shock.

Background: Septic shock is one of the major causes of mortality among children worldwide.

Methods: A retrospective analysis was made of children admitted to the pediatric intensive care unit between November 2010 and December 2019.

Metastatic colorectal cancer in both sides of Aegean sea: practice patterns and outcome.

Objective: The successful results obtained in trials may not necessarily translate into prolonged survival of metastatic colorectal cancer (mCRC) patients in real life. This multinational registry study aimed to evaluate the real-life data effecting the survival of patients with mCRC.

Methods: This is a multinational, retrospective registry study.

Childhood Interstitial Lung Disease Masquerading as Post COVID-19 Respiratory Distress.

Childhood interstitial lung diseases (chILD) are a set of illnesses affecting the bronchoalveolar spaces and the cellular compartment of the lungs. In the neonatal period, they are mainly classified under disorders of development, growth, surfactant dysfunction, and others of unknown causes distinctive in infancy. One of the most common causes is the deficiency of triphosphate binding cassette transporter A3 (ABCA3) protein.

Pulmonary Arterial Hypertension as a Fatal Complication of Neurofibromatosis Type 1 in a Neonate: A Diagnostic Dilemma.

Neurofibromatosis type 1 (NF1), or von Recklinghausen disease, is a genetically transmitted autosomal dominant disease, with a prevalence of one per 4000 live births. Pulmonary arterial hypertension (PAH) is a rare but potentially life-threatening complication of NF1. There are no confirmatory data about the congenital association between PAH and NF1.

Efficacy and Safety of Trastuzumab Emtansine in Her2 Positive Metastatic Breast Cancer: Real-World Experience.

Aim: The aim of this study is to evaluate the efficacy and toxicity of trastuzumab emtansine (T-DM1) in cases with metastatic breast cancer (mBC) in different lines of treatment.

Method: Retrospective analysis of T-DM1 results of human epidermal growth factor receptor 2 (Her2) positive 414 cases with mBC from 31 centers in Turkey.

Findings: Except 2, all of the cases were female with a median age of 47.

An Approach to Addressing Multiple Imputation Model Uncertainty Using Bayesian Model Averaging.

This paper considers the problem of imputation model uncertainty in the context of missing data problems. We argue that so-called "Bayesianly proper" approaches to multiple imputation, although correctly accounting for uncertainty in imputation model parameters, ignore the uncertainty in the imputation model itself. We address imputation model uncertainty by implementing Bayesian model averaging as part of the imputation process.

Bilateral Pulmonary Langerhans's Cell Histiocytosis is Surgical Challenge in Children: A Case Report.

 Pulmonary Langerhans's cell histiocytosis (PLCH) is a rare cause of interstitial lung disease in children and more than half of the cases are bilateral. Persistent respiratory distress due to spontaneous pneumothorax (SP) in bilateral PLCH may refractory to conservative treatment and posed a great challenge to surgical modalities. A 3-year-old boy with SP due to bilateral PLCH is presented to discuss the surgical options of recurrent and refractory PLCH cases in children.