Respiratory prehabilitation in pediatric anesthesia in children with muscular and neurologic disease.

Children with neuromuscular, chronic neurologic, and chest wall diseases are at increased risk of postoperative respiratory complications including atelectasis, pneumonia, and respiratory failure with the possible need for reintubation or even tracheostomy. These complications negatively impact patient outcomes, including increased healthcare resource utilization and increased surgical mortality. In these children, the existing respiratory reserve is often inadequate to withstand the stresses brought on during anesthesia and surgery.

Organic visual loss measured by kinetic perimetry and retinal electrophysiology in children with functional amblyopia.

Purpose: To demonstrate an organic (retinal) amblyogenic defect in functional amblyopes not responding to treatment.

Methods: Twenty-four children (Mean age: 5.9 ± 1.

Age and sex determine conversion from immediate-release to extended-release tacrolimus in a multi-center cohort of Canadian pediatric renal transplant recipients.

ER-Tac, taken once per day, is associated with improved adherence. This study examined the potential patient and clinical factors that influence clinicians to convert pediatric patients from immediate-release to ER-Tac. This prospective multi-center observational study followed Canadian pediatric kidney transplant recipients up to 5 years post-transplant.

Towards defining the surgical workforce for children: a geospatial analysis in Brazil.

Objectives: The optimal size of the health workforce for children's surgical care around the world remains poorly defined. The goal of this study was to characterise the surgical workforce for children across Brazil, and to identify associations between the surgical workforce and measures of childhood health.

Design: This study is an ecological, cross-sectional analysis using data from the Brazil public health system ().

Evidences Suggesting that Distinct Immunological and Cellular Responses to Light Damage Distinguishes Juvenile and Adult Rat Retinas.

To unravel the mechanisms behind the higher resistance to light damage of juvenile (JR) versus adult (AR) rats, Sprague Dawley rats were exposed to a bright luminous environment of 10, 000 lux. The light-induced retinopathy (LIR) was assessed with histology, electroretinography and immunohistochemistry (IHC). In JR, 2 days of exposure induced the typical LIR, while >3 days added little LIR.

PPARα-mediated peroxisome induction compensates PPARγ-deficiency in bronchiolar club cells.

Despite the important functions of PPARγ in various cell types of the lung, PPARγ-deficiency in club cells induces only mild emphysema. Peroxisomes are distributed in a similar way as PPARγ in the lung and are mainly enriched in club and AECII cells. To date, the effects of PPARγ-deficiency on the overall peroxisomal compartment and its metabolic alterations in pulmonary club cells are unknown.

The impact of erdosteine on cisplatin-induced ototoxicity: a proteomics approach.

Cisplatin is a commonly used chemotherapeutic agent and causes serious side effects, including progressive and irreversible hearing loss. No treatment is currently available for cisplatin-induced ototoxicity. We have previously demonstrated that erdosteine, a potent antioxidant, partially protected the cochlea against cisplatin toxicity in vivo.

Growth charts for individuals with rhizomelic chondrodysplasia punctata.

Rhizomelic chondrodysplasia punctata (RCDP) is a class of peroxisomal disorders characterized by defective plasmalogen biosynthesis. There are multiple recognized types of RCDP, all of which have autosomal recessive inheritance, and their associated genes are known: RCDP type 1 with PEX7, RCDP type 2 with GNPAT, RCDP type 3 with AGPS, RCDP type 4 with FAR1, and RCDP type 5 with PEX5. Among other medical/developmental issues, plasmalogen deficiency has a direct effect on bone growth and results in postnatal growth failure, the severity of which corresponds to the degree of plasmalogen deficiency.

Sildenafil Improves Functional and Structural Outcome of Retinal Injury Following Term Neonatal Hypoxia-Ischemia.

Purpose: The purpose of this study was to investigate the effects of sildenafil on retinal injury following neonatal hypoxia-ischemia (HI) at term-equivalent age in rat pups.

Methods: Hypoxia-ischemia was induced in male Long-Evans rat pups at postnatal day 10 (P10) by a left common carotid ligation followed by a 2-hour exposure to 8% oxygen. Sham-operated rats served as the control group.

Witnessing the first sign of retinitis pigmentosa onset in the allegedly normal eye of a case of unilateral RP: a 30-year follow-up.

Purpose: A patient initially presented with constricted visual field, attenuated retinal vasculature, pigmentary clumping and reduced ERG in OS only, suggestive of unilateral retinitis pigmentosa (RP). This patient was subsequently seen on eight occasions (over three decades), and, with time, the initially normal eye (OD) gradually showed signs of RP-like degeneration. The purpose of this study was to evaluate which clinical modality (visual field, funduscopy or electroretinography) could have first predicted this fate.

Promoting an ethic of engagement in pediatric palliative care research.

Background: This paper defends the ethical and empirical significance of direct engagement with terminally ill children and adolescents in PPC research on health-related quality of life. Clinical trials and other forms of health research have resulted in tremendous progress for improving clinical outcomes among children and adolescents diagnosed with a life-threatening illness. Less attention has been paid, however, to engaging this patient population directly in studies aimed at optimizing health-related quality of life in PPC.

Nuclear bioavailability of the glucocorticoid receptor in a pediatric asthma cohort with variable corticosteroid responsiveness.

Background: Despite the overall effectiveness of glucocorticoids (GCs) in the treatment of asthma, a large proportion of patients do not fully respond to this medication. The objective of the present study was to investigate the potential molecular mechanisms responsible for corticosteroid insensitivity in pediatric asthma.

Methods: Asthmatic children were classified as good (GSR) or poor corticosteroid responders (PSR) based on the changes in pulmonary function following GC treatment.

Visual Impairments Following Term Neonatal Encephalopathy: Do Retinal Impairments Also Play a Role?

Purpose: We investigated the effects of term neonatal encephalopathy on retinal function and structure.

Methods: A rat model of term neonatal hypoxic-ischemic (HI) encephalopathy (Vannucci model) was used. Hypoxia-ischemia was induced by a left common carotid ligation followed by a 2-hour period of hypoxia (8% oxygen) in Long-Evans rat pups at postnatal day 10 (P10).

WNT/β-Catenin Signaling Is Required for Integration of CD24+ Renal Progenitor Cells into Glycerol-Damaged Adult Renal Tubules.

During development, nephron progenitor cells (NPC) are induced to differentiate by WNT9b signals from the ureteric bud. Although nephrogenesis ends in the perinatal period, acute kidney injury (AKI) elicits repopulation of damaged nephrons. Interestingly, embryonic NPC infused into adult mice with AKI are incorporated into regenerating tubules.

Extracellular vesicles, tissue factor, cancer and thrombosis - discussion themes of the ISEV 2014 Educational Day.

Although the association between cancer and venous thromboembolism (VTE) has long been known, the mechanisms are poorly understood. Circulating tissue factor-bearing extracellular vesicles have been proposed as a possible explanation for the increased risk of VTE observed in some types of cancer. The International Society for Extracellular Vesicles (ISEV) and International Society on Thrombosis and Haemostasis (ISTH) held a joint Educational Day in April 2014 to discuss the latest developments in this field.

Anthracycline-containing chemotherapy causes long-term impairment of mitochondrial respiration and increased reactive oxygen species release in skeletal muscle.

Anticancer treatments for childhood acute lymphoblastic leukaemia (ALL) are highly effective but are now implicated in causing impaired muscle function in long-term survivors. However, no comprehensive assessment of skeletal muscle mitochondrial functions in long-term survivors has been performed and the presence of persistent chemotherapy-induced skeletal muscle mitochondrial dysfunction remains a strong possibility. Non-tumour-bearing mice were treated with two drugs that have been used frequently in ALL treatment (doxorubicin and dexamethasone) for up to 4 cycles at 3-week intervals and euthanized 3 months after the 4th cycle.

Disease specific therapies in leukodystrophies and leukoencephalopathies.

Leukodystrophies are a heterogeneous, often progressive group of disorders manifesting a wide range of symptoms and complications. Most of these disorders have historically had no etiologic or disease specific therapeutic approaches. Recently, a greater understanding of the pathologic mechanisms associated with leukodystrophies has allowed clinicians and researchers to prioritize treatment strategies and advance research in therapies for specific disorders, some of which are on the verge of pilot or Phase I/II clinical trials.

Point tenderness at 1 of 5 locations and limited elbow extension identify significant injury in children with acute elbow trauma: a study of diagnostic accuracy.

Objective: Our goal was to determine whether the combination of tenderness at 1 of 5 commonly fractured sites and elbow extension accurately predicts the presence of acute elbow fractures or isolated effusions in children.

Methods: A prospective cohort study was performed using a convenience sample of patients. Children 0 to 18 years old with acute elbow injuries without elbow deformities or radial head subluxations were prospectively enrolled.

A Review of Secondary Photoreceptor Degenerations in Systemic Disease.

Photoreceptor neuronal degenerations are common and incurable causes of human blindness with one in 2000 affected. Approximately, half of all patients are associated with known mutations in more than 200 disease genes. Most retinal degenerations are restricted to the retina (primary retinal degeneration) but photoreceptor degeneration can also be found in a wide variety of systemic and syndromic diseases.

Wilms tumor suppressor, WT1, suppresses epigenetic silencing of the β-catenin gene.

The mammalian kidney is derived from progenitor cells in intermediate mesoderm. During embryogenesis, progenitor cells expressing the Wilms tumor suppressor gene, WT1, are induced to differentiate in response to WNT signals from the ureteric bud. In hereditary Wilms tumors, clonal loss of WT1 precludes the β-catenin pathway response and leads to precancerous nephrogenic rests.

The mortality risk with graft function has decreased among children receiving a first kidney transplant in the United States.

Mortality has decreased in children with end-stage kidney disease. Decreases in mortality during dialysis and improved graft survival contributed to this improvement. However, it is unknown whether rates of death with graft function have also improved.