Is 'oil pulling' a 'snake oil'? : a clinical trial

The traditional Ayurveda practice of ‘oil pulling’ has become a recent phenomenon and concerns about its efficacy have been raised. Objectives: (1) to determine awareness about the practice of ‘oil pulling’ among a group of young adults, and to determine variations in awareness with respect to socio-demographic factors, oral health behaviours (oral hygiene and dental attendance) and use of natural health products; (2) to determine the effectiveness of ‘oil pulling’ and conventional oral hygiene practice compared to the use of conventional oral hygiene practice alone in terms of oral hygiene and (3) to determine the effectiveness of ‘oil pulling’ and conventional oral hygiene practice compared to the use of conventional oral hygiene practice alone in terms of gingival health. Methods: Group members recruited seventy-four young adults to participate in a clinical trial over a two-month period comparing the effectiveness of (a) ‘oil pulling’ and conventional oral hygiene methods (toothbrush and toothpaste) versus (b) conventional oral hygiene methods alone. Oral hygiene was assessed using the Plaque Index - PI (Silness and Löe, 1964) and the proportion of sites with visible plaque (PVP). Gingival health was assessed using the Gingival Index – GI (Silness and Löe,1963) and the proportion of sites with gingival bleeding (PGB). Participants were block randomized in groups of four to a cross over clinical trial and assessments were conducted at one-month and two-months. Results: Approximately a quarter (28.4%, 21) of participants was aware of the practice of ‘oil pulling’. Awareness of the practice was associated with reported use of natural dental/oral health products (p0.05). There were observed significant differences in gingival health among both the test and control groups from baseline to one-month (p0.05). No significant differences were observed in oral health parameters from one-month to two-month among neither the test nor control groups (p>0.05). Conclusion: Awareness of the practice of ‘oil pulling’ is relatively common and is associated with use of natural dental/oral health products. Findings from the clinical trial failed to support the adjunct use of ‘oil pulling’ in addition to conventional oral hygiene practices.published_or_final_versio

Isolation and characterization of a human heart cDNA encoding a new member of the small heat shock protein family — HSPL27

AbstractA novel cDNA clone was isolated from a human adult heart cDNA library. This cDNA clone is similar to the small heat shock protein (smhsp) in both DNA and amino acid sequences, especially in the conserved region. Sequence analysis has shown that the putative novel smhsp, named 27 kDa heat-shock-protein-like protein (HSPL27) is a protein of 241 amino acids with a deduced molecular mass of 26.7 kDa and a deduced pI of 8.0. We have expressed the HSPL27 in E. coli and the expressed protein was found to be present in the soluble fraction of the bacterial cell lysate. Chromosomal mapping data shows that the HSPL27 gene is located at human chromosome 5q11.2

International Paediatric Mitochondrial Disease Scale

Objective: There is an urgent need for reliable and universally applicable outcome measures for children with mitochondrial diseases. In this study, we aimed to adapt the currently available Newcastle Paediatric Mitochondrial Disease Scale (NPMDS) to the International Paediatric Mitochondrial Disease Scale (IPMDS) during a Delphi-based process with input from international collaborators, patients and caretakers, as well as a pilot reliability study in eight patients. Subsequently, we aimed to test the feasibility, construct validity and reliability of the IPMDS in a multicentre study. Methods: A clinically, biochemically and genetically heterogeneous group of 17 patients (age 1.6–16 years) from five different expert centres from four different continents were evaluated in this study. Results: The feasibility of the IPMDS was good, as indicated by a low number of missing items (4 %) and the positive evaluation of patients, parents and users. Principal component analysis of our small sample identified three factors, which explained 57.9 % of the variance. Good construct validity was found using hypothesis testing. The overall interrater reliability was good [median intraclass correlation coefficient for agreement between raters (ICCagreement) 0.85; range 0.23–0.99). Conclusion: In conclusion, we suggest using the IPMDS for assessing natural history in children with mitochondrial diseases. These data should be used to further explore construct validity of the IPMDS and to set age limits. In parallel, responsiveness and the minimal clinically important difference should be studied to facilitate sample size calculations in future clinical trials

Assessment of intellectual impairment, health-related quality of life, and behavioral phenotype in patients with neurotransmitter related disorders: Data from the iNTD registry

Inherited disorders of neurotransmitter metabolism are a group of rare diseases, which are caused by impaired synthesis, transport, or degradation of neurotransmitters or cofactors and result in various degrees of delayed or impaired psychomotor development. To assess the effect of neurotransmitter deficiencies on intelligence, quality of life, and behavior, the data of 148 patients in the registry of the International Working Group on Neurotransmitter Related Disorders (iNTD) was evaluated using results from standardized age-adjusted tests and questionnaires. Patients with a primary disorder of monoamine metabolism had lower IQ scores (mean IQ 58, range 40-100) within the range of cognitive impairment (<70) compared to patients with a BH4 deficiency (mean IQ 84, range 40-129). Short attention span and distractibility were most frequently mentioned by parents, while patients reported most frequently anxiety and distractibility when asked for behavioral traits. In individuals with succinic semialdehyde dehydrogenase deficiency, self-stimulatory behaviors were commonly reported by parents, whereas in patients with dopamine transporter deficiency, DNAJC12 deficiency, and monoamine oxidase A deficiency, self-injurious or mutilating behaviors have commonly been observed. Phobic fears were increased in patients with 6-pyruvoyltetrahydropterin synthase deficiency, while individuals with sepiapterin reductase deficiency frequently experienced communication and sleep difficulties. Patients with BH4 deficiencies achieved significantly higher quality of life as compared to other groups. This analysis of the iNTD registry data highlights: (a) difference in IQ and subdomains of quality of life between BH4 deficiencies and primary neurotransmitter-related disorders and (b) previously underreported behavioral traits.Dietmar Hopp Stiftung (DE); Medical Faculty of the University of Heidelberg

Garip aileler

Fazlı Necip'in Yeni Asır'da tefrika edilen Garip Aileler adlı romanıArşivdeki eksikler nedeniyle tefrika yarım kalmıştır. Ancak tefrikanın tamamlandığı bilinmektedir

International Paediatric Mitochondrial Disease Scale

OBJECTIVE : There is an urgent need for reliable and universally applicable outcome measures for children with mitochondrial diseases. In this study, we aimed to adapt the currently available Newcastle Paediatric Mitochondrial Disease Scale (NPMDS) to the International Paediatric Mitochondrial Disease Scale (IPMDS) during a Delphi-based process with input from international collaborators, patients and caretakers, as well as a pilot reliability study in eight patients. Subsequently, we aimed to test the feasibility, construct validity and reliability of the IPMDS in a multicentre study. METHODS : A clinically, biochemically and genetically heterogeneous group of 17 patients (age 1.6–16 years) from five different expert centres from four different continents were evaluated in this study. RESULTS : The feasibility of the IPMDS was good, as indicated by a low number of missing items (4 %) and the positive evaluation of patients, parents and users. Principal component analysis of our small sample identified three factors, which explained 57.9 % of the variance. Good construct validity was found using hypothesis testing. The overall interrater reliability was good [median intraclass correlation coefficient for agreement between raters (ICCagreement) 0.85; range 0.23–0.99). CONCLUSION : In conclusion, we suggest using the IPMDS for assessing natural history in children with mitochondrial diseases. These data should be used to further explore construct validity of the IPMDS and to set age limits. In parallel, responsiveness and the minimal clinically important difference should be studied to facilitate sample size calculations in future clinical trials.The work of SK and JS was sponsored by ZonMW (The Netherlands Organization for Health Research and Development).http://link.springer.com/journal/10545am2017Paediatrics and Child Healt

ARX-associated infantile epileptic-dyskinetic encephalopathy with responsiveness to valproate for controlling seizures and reduced activity of muscle mitochondrial complex IV

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