Weaning Humidified High Flow Oxygen Therapy among Paediatric Patients: An Integrative Review of Literature.

PROBLEM: The paucity of up-to-date recommendations and evidence-based models, whether it is physician-initiated or initiated by other healthcare professionals, for humidified high flow oxygen therapy among children. ELIGIBILITY CRITERIA: The inclusion criteria included the following: 1) use of high flow oxygen therapy (≥15 L/min); 2) published studies from the year 2000 and onwards; 3) research article in a peer-reviewed journal; 4) studies conducted in a hospital setting involving paediatric patients <18 years old; 5) availability of full article online. SAMPLE: From March to April 2018, electronic databases such as PubMed, Cumulative Index of Nursing and Allied Health Literature, Excerpta Medica Database, Cochrane Library, Joanna Briggs Institute Library of Systematic Reviews, SCOPUS, Ovid, Informit, and Google Scholar were accessed. The systematic search initially yielded 41 studies. RESULTS: Eventually, three eligible studies were reviewed and appraised. Overarching themes were identified: 1) the lack of weaning standards; 2) the limited focus on young population in intensive care settings; and 3) the paucity of weaning models. CONCLUSION: The lack of studies suggested that this is a fertile area for research. In this light, this paper challenged researchers, clinicians, and experts to develop evidence-based standards and models of weaning towards efficient and better quality of care. IMPLICATION: This review may lead to the development of nurse-led or nurse-initiated weaning protocols to enable timely weaning intervention for children and thus reduce the need for prolonged oxygen use. Furthermore, this may also instigate an economic evaluation of a nurse-lead weaning against current models of medically lead weaning.

Investigation of molybdenum cofactor deficiency due to MOCS2 deficiency in a newborn baby.

BACKGROUND: Molybdenum cofactor deficiency (MOCD) is a severe autosomal recessive neonatal metabolic disease that causes seizures and death or severe brain damage. Symptoms, signs and cerebral images can resemble those attributed to intrapartum hypoxia. In humans, molybdenum cofactor (MOCO) has been found to participate in four metabolic reactions: aldehyde dehydrogenase (or oxidase), xanthine oxidoreductase (or oxidase) and sulfite oxidase, and some of the components of molybdenum cofactor synthesis participate in amidoxime reductase. A newborn girl developed refractory seizures, opisthotonus, exaggerated startle reflexes and vomiting on the second day of life. Treatment included intravenous fluid, glucose supplementation, empiric antibiotic therapy and anticonvulsant medication. Her encephalopathy progressed, and she was given palliative care and died aged 1 week. There were no dysmorphic features, including ectopia lentis but ultrasonography revealed a thin corpus callosum. OBJECTIVES: The aim of this study is to provide etiology, prognosis and genetic counseling. METHODS: Biochemical analysis of urine, blood, Sanger sequencing of leukocyte DNA, and analysis of the effect of the mutation on protein expression. RESULTS: Uric acid level was low in blood, and S-sulfo-L-cysteine and xanthine were elevated in urine. Compound Z was detected in urine. Two MOCS2 gene mutations were identified: c.501 + 2delT, which disrupts a conserved splice site sequence, and c.419C > T (pS140F). Protein expression studies confirmed that the p.S140F substitution was pathogenic. The parents were shown to be heterozygous carriers. CONCLUSIONS: Mutation analysis confirmed that the MOCD in this family could not be treated with cPMP infusion, and enabled prenatal diagnosis and termination of a subsequent affected pregnancy.

Randomized study of high-dose pulse calcitriol or placebo prior to radical prostatectomy.

BACKGROUND: Cancer chemoprevention trials require enormous resources due to the large numbers of patients and the years of follow-up needed to achieve sufficient statistical power. Examination of candidate prevention agents using biomarkers as surrogate end points has been proposed as a method to rapidly identify promising agents for prevention trials. Treatment of patients with candidate agents prior to scheduled biopsy or surgical resection of malignancy allows for direct examination of the treatment effects on tumor tissue. In this study, we selected this approach to test several hypotheses about the effect of calcitriol (1,25-dihydroxycholecalciferol), the active form of vitamin D, on early-stage human prostate cancer. METHODS: After selection of surgical treatment for histologically confirmed adenocarcinoma of the prostate, patients were randomized to either calcitriol 0.5 mug/kg or placebo weekly for 4 weeks. The expression levels of the vitamin D receptor (VDR), proliferating cell nuclear antigen, PTEN (MMAC1/TEP1), c-Myc, transforming growth factor (TGF) beta receptor type II (TGFbeta RII), and Bcl-2 were quantified using immunohistochemistry in the patients' prostate specimens post surgery. RESULTS: Thirty-seven of 39 prostate tumors were evaluable for molecular end points. VDR expression was reduced in patients treated with calcitriol (mean, 75.3% of cells) compared with those that received placebo (mean, 98.6%; P = 0.005). Calcitriol treatment did not result in a statistically significant change in the fraction of cells expressing TGFbeta RII, PTEN, or proliferating cell nuclear antigen. Bcl-2 and c-Myc expression was at the lower limits of detection in both the calcitriol group and the placebo group; therefore, we were unable to determine whether drug treatment induced a significant change in these biomarkers. CONCLUSIONS: High-dose calcitriol down-regulates VDR expression in human prostate cancer. Further study is needed to determine the biological consequences of VDR down-regulation in prostate cancer. This study shows that the use of the preprostatectomy model is feasible and can be used to test the effect of candidate chemopreventive agents on prostate cancer.