Advancements in the Management of Pediatric and Adult Inflammatory Bowel Disease: A Systematic Review of Treatment Strategies and Long-Term Outcomes.

Inflammatory bowel disease (IBD), including Crohn's disease and ulcerative colitis (UC), remains a clinically complex condition in children and adults. This study is a systematic analysis of key developments in the treatment of inflammatory bowel diseases, as well as their efficacy and safety over time. Early diagnosis of pediatric IBD is very important since it affects growth and development in children.

Efficacy and Safety of Pharmacokinetically-Driven Dosing of Mycophenolate Mofetil for the Treatment of Pediatric Proliferative Lupus Nephritis-A Double-Blind Placebo Controlled Clinical Trial (The Pediatric Lupus Nephritis Mycophenolate Mofetil Study).

Background: The safety and efficacy of mycophenolate mofetil (MMF) for lupus nephritis (LN) treatment is established in adults and in some children. MMF is rapidly converted to the biologically active metabolite mycophenolic acid (MPA) whose pharmacokinetics (PK) is characterized by large inter- and intra-individual variability.

Methods/design: This randomized, double-blind, active comparator, controlled clinical trial of pediatric subjects with proliferative LN compares pharmacokinetically-guided precision-dosing of MMF (MMF, i.

Constitutively active Lyn kinase causes a cutaneous small vessel vasculitis and liver fibrosis syndrome.

Neutrophilic inflammation is a hallmark of many monogenic autoinflammatory diseases; pathomechanisms that regulate extravasation of damaging immune cells into surrounding tissues are poorly understood. Here we identified three unrelated boys with perinatal-onset of neutrophilic cutaneous small vessel vasculitis and systemic inflammation. Two patients developed liver fibrosis in their first year of life.

Development and validation of the first adaptive test of emotion perception in music.

The Musical Emotion Discrimination Task (MEDT) is a short, non-adaptive test of the ability to discriminate emotions in music. Test-takers hear two performances of the same melody, both played by the same performer but each trying to communicate a different basic emotion, and are asked to determine which one is "happier", for example. The goal of the current study was to construct a new version of the MEDT using a larger set of shorter, more diverse music clips and an adaptive framework to expand the ability range for which the test can deliver measurements.

Compromised skin barrier induced by prolonged face mask usage during the COVID-19 pandemic and its remedy with proper moisturization.

Background: Prolonged face mask usage, a daily practice for the public due to the COVID-19 pandemic, creates high levels of humidity underneath the mask, which may cause unexpected skin concerns.

Objective: To investigate the impact of repeated mask usage on the face by comparing skin properties inside and outside of the mask-covered areas.

Methods: A double-blinded, randomized, split-face clinical study was conducted with 21 healthy female participants who wore face masks at least 6 h every day for 1 week, with one side of their face treated with a moisturizer three times daily.

Health disparities in outcomes of pediatric systemic lupus erythematosus.

Healthcare disparities exist throughout the United States, and disparities in healthcare delivery are responsible for a substantial portion of preventable morbidity and mortality. SLE disproportionately affects racial and ethnic minoritized groups, including Blacks, Hispanics, and Asians/Pacific Islanders. Specifically, Black females have a 3 to 4-fold increased risk of developing SLE than White females.

Effects of a Powder Formulation of on Growth and Resistance of Two Cocoa Hybrids from Cameroon against (Causal Agent of Black Pod Disease).

In the present study we evaluated the efficacy of a bioformulation of for control of black pod disease in cocoa and enhancement of seedling growth. The formulation developed using talc powder and cassava starch as carriers showed high shelf-life of 1.07 × 10 CFU/g after six months storage at 4°C.

Survival of musical activities. When do young people stop making music?

Although making music is a popular leisure activity for children and adolescents, few stay musically engaged. Previous research has focused on finding reasons for quitting musical activities, pedagogical strategies to keep students engaged with music, and motivational factors of musical training. Nonetheless, we know very little about how the proportion of musically active children changes with age and what traits influence the survival of musical engagement.

Paediatric Liver Transplantation During COVID-19 Pandemic: Lessons Learned and Unanswered Questions.

COVID-19 pandemic has imposed many challenges on paediatric liver transplantation (PLT) services and has necessitated several adaptations in different stages of the process to ensure transplant centres can still deliver the proposed services in addition to protecting patients and staff against infection. This review article digs through the current literature to clarify the challenges imposed by SARS-CoV2 on PLT centres globally. It provides an overview of current practice as well as suggestions from experts in the field to overcome multiple obstacles.

Differences in rituximab use between pediatric rheumatologists and nephrologists for the treatment of refractory lupus nephritis and renal flare in childhood-onset SLE.

Background: Consensus treatment plans have been developed for induction therapy of newly diagnosed proliferative lupus nephritis (LN) in childhood-onset systemic lupus erythematosus. However, patients who do not respond to initial therapy, or who develop renal flare after remission, warrant escalation of treatment. Our objective was to assess current practices of pediatric nephrologists and rheumatologists in North America in treatment of refractory proliferative LN and flare.

International Consensus for the Dosing of Corticosteroids in Childhood-Onset Systemic Lupus Erythematosus With Proliferative Lupus Nephritis.

Objective: To develop a standardized steroid dosing regimen (SSR) for physicians treating childhood-onset systemic lupus erythematosus (SLE) complicated by lupus nephritis (LN), using consensus formation methodology.

Methods: Parameters influencing corticosteroid (CS) dosing were identified (step 1). Data from children with proliferative LN were used to generate patient profiles (step 2).

Formal neurocognitive function and anti-N-methyl-D-aspartate receptor antibodies in paediatric lupus.

Objective: SLE is a chronic multisystem autoimmune inflammatory disease impacting a number of organs, including the central nervous system (CNS). The pathophysiology of CNS lupus is multifactorial, making diagnosis problematic. Neurocognitive (NC) testing and specific biomarkers to identify the development of neuropsychiatric (NP) symptoms in lupus are needed.

What is the long-term outlook for young people following liver transplant? A single-centre retrospective analysis of physical and psychosocial outcomes.

LT is a successful treatment for end-stage liver disease. The long-term outcome of patients transplanted in childhood has not previously been widely reported. This project assessed the long-term impact of transplantation in patients surviving >15 years.

Bioremediation of di-(2-ethylhexyl) phthalate contaminated red soil by Gordonia terrae RL-JC02: Characterization, metabolic pathway and kinetics.

Di-(2-ethylhexyl) phthalate (DEHP) is the most widely used plasticizer and a representative endocrine disrupting chemical. The toxicological effects of DEHP on environmental and human health have been widely investigated. In this study, the DEHP-degrading bacterial strain RL-JC02 was isolated from red soil with long-term usage of plastic mulch, and it was identified as Gordonia terrae by 16S rRNA gene analysis coupled with physiological and biochemical characterization.

Distinct interferon signatures and cytokine patterns define additional systemic autoinflammatory diseases.

BACKGROUNDUndifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine dysregulation may identify diseases with available targeted treatments.METHODSSixty-six consecutively referred USAID patients underwent underwent screening for the presence of an interferon signature using a standardized type-I IFN-response-gene score (IRG-S), cytokine profiling, and genetic evaluation by next-generation sequencing.

Cardiac and Liver Disease in Children: Implications for Management Before and After Liver Transplantation.

There is close interaction between the functions of the liver and heart affecting the presentation, diagnosis, and outcome of acute and chronic cardiac and liver disease. Conditions affecting both organ systems should be considered when proposing transplantation because the interaction between cardiac disease and liver disease has implications for diagnosis, management, selection for transplantation, and, ultimately, for longterm outcomes after liver transplantation (LT). The combination of cardiac and liver disease is well recognized in adults but is less appreciated in pediatric patients.

Research priorities in childhood-onset lupus: results of a multidisciplinary prioritization exercise.

Background: Childhood-onset systemic erythematosus lupus (cSLE) is characterized by more severe disease, widespread organ involvement and higher mortality compared to adult-onset SLE. However, cSLE is largely underfunded to carry out necessary research to advance the field. Few commonly used SLE medications have been studied in children, and important knowledge gaps exist concerning epidemiology, genetics, pathophysiology and optimal treatments for cSLE.

Use of secukinumab in a pediatric patient leads to significant improvement in nail psoriasis and psoriatic arthritis.

Many pediatric psoriasis patients suffer from nail involvement and psoriatic arthritis. In adults, biologic agents have demonstrated success in treating refractory nail psoriasis and arthritis, but studies are limited in children. In this report, we present a pediatric patient with severe, recalcitrant nail and joint psoriasis, successfully treated with secukinumab.

Complete genome sequence of a phthalic acid esters degrading Mycobacterium sp. YC-RL4.

Mycobacterium sp. YC-RL4 is capable of utilizing a broad range of phthalic acid esters (PAEs) as sole source of carbon and energy for growth. The preliminary studies demonstrated its high degrading efficiency and good performance during the bioprocess with environmental samples.

Retrospective Evaluation of Topical Bimatoprost and Iris Pigmentation Change.

Background: Topical bimatoprost is a topical prostaglandin analog originally used to treat glaucoma and more recently used to cosmetically induce hypertrichosis of the eyelashes. Iris pigmentation change has been noted in the treatment of glaucoma but has not been assessed with the cosmetic periorbital application of bimatoprost.

Objective: To evaluate for iris pigmentation change with the long-term cosmetic use of topical bimatoprost.

Advances in the care of children with lupus nephritis.

The care of children with lupus nephritis (LN) has changed dramatically over the past 50 y. The majority of patients with childhood-onset systemic lupus erythematosus (cSLE) develop LN. In the 1960's, prognosis in children was worse than in adults; therapies were limited and toxic.

FROSTED BRANCH ANGIITIS IN METHIMAZOLE-INDUCED ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-POSITIVE VASCULITIS.

Purpose: To describe an unusual case of frosted branch angiitis that developed in a patient with acute onset systemic vasculitis possibly triggered by the antithyroid medication methimazole.

Methods: We conducted a thorough review of the medical records of a 16-year-old female patient who presented with frosted branch angiitis. During the initial hospital admission, the patient underwent an extensive systemic workup to determine the etiology of her disease and ophthalmologic testing including fundus photographs and fluorescein angiography.