Hirschsprung disease (HSCR, aganglionic megacolon) represents the main genetic cause of functional intestinal obstruction with an incidence of 1/5000 live births. This developmental disorder is a ...neurocristopathy and is characterised by the absence of the enteric ganglia along a variable length of the intestine. In the last decades, the development of surgical approaches has importantly decreased mortality and morbidity which allowed the emergence of familial cases. Isolated HSCR appears to be a non-Mendelian malformation with low, sex-dependent penetrance, and variable expression according to the length of the aganglionic segment. While all Mendelian modes of inheritance have been described in syndromic HSCR, isolated HSCR stands as a model for genetic disorders with complex patterns of inheritance. The tyrosine kinase receptor RET is the major gene with both rare coding sequence mutations and/or a frequent variant located in an enhancer element predisposing to the disease. Hitherto, 10 genes and five loci have been found to be involved in HSCR development.
This study aimed to evaluate post-adenoidectomy quality of life in children with refractory chronic rhinosinusitis.
A prospective interventional study of children aged 4-12 years with chronic ...refractory rhinosinusitis was conducted. A total of 60 children completed follow up. Nasal endoscopy and non-contrast computed tomography of the paranasal sinuses were performed, and both symptoms and their effects on patient quality of life pre- and post-adenoidectomy were evaluated.
The most frequent symptoms were nasal obstruction, cough, fever and fatigue, which were experienced by 100 per cent, 90 per cent, 85 per cent and 81.7 per cent of children, respectively. Nasal endoscopy showed oedema and discharge were present in all children. A statistically significant post-operative improvement in sinus and nasal quality of life was seen in 53 children (88.3 per cent).
Adenoidectomy is a simple, first-line surgical procedure for managing paediatric chronic rhinosinusitis refractory to maximal medical therapy and leads to an improved quality of life.
Abstract
Purpose
. Commercial electron FLASH platforms deliver ultra-high dose rate doses at discrete combinations of pulse parameters including pulse width (PW), pulse repetition frequency (PRF) and ...number of pulses (
N
), which dictate unique combinations of dose and dose rates. Additionally, collimation, source to surface distance, and airgaps also vary the dose per pulse (DPP). Currently, obtaining pulse parameters for the desired dose and dose rate is a cumbersome manual process involving creating, updating, and looking up values in large spreadsheets for every treatment configuration. This work presents a pulse parameter optimizer application to match intended dose and dose rate precisely and efficiently.
Methods
. Dose and dose rate calculation methods have been described for a commercial electron FLASH platform. A constrained optimization for the dose and dose rate cost function was modelled as a mixed integer problem in MATLAB (The MathWorks Inc., Version9.13.0 R2022b, Natick, Massachusetts). The beam and machine data required for the application were acquired using GafChromic film and alternating current current transformers (ACCTs). Variables for optimization included DPP for every collimator, PW and PRF measured using ACCT and airgap factors.
Results
. Using PW, PRF,
N
and airgap factors as parameters, a software was created to optimize dose and dose rate, reaching the closest match if exact dose and dose rates are not achievable. Optimization took 20 s or less to converge to results. This software was validated for accuracy of dose calculation and precision in matching prescribed dose and dose rate.
Conclusion
. A pulse parameter optimization application was built for a commercial electron FLASH platform to increase efficiency in dose, dose rate, and pulse parameter prescription process. Automating this process reduces safety concerns associated with manual look up and calculation of these parameters, especially when many subjects at different doses and dose rates are to be safely managed.
Background
Honey reduced post‐tonsillectomy pain, but its effects on awakening at night, inflammation and healing of the tonsillar fossa were controversial.
Objectives
This systematic review and ...meta‐analysis of randomised controlled trials (RCTs) evaluated the effect of oral honey on pain, consumption of painkillers, awakening at night, healing of tonsillar fossa and adverse effects in children after tonsillectomy.
Methods
A search of MEDLINE, EMBASE, Scopus, CINAHL and Cochrane Collaboration Library databases was performed without any restriction of publication year. The end date of search was 30 June 2016. The search was supplemented by search from Google, hand search of cross‐references of selected articles and reviews, and contacting the authors of different studies. The inclusion criteria were RCTs comparing the effect of honey with control on different outcomes, in children after tonsillectomy.
Results
Our search generated 64 studies, and eight RCTs met our inclusion criteria. The methodological quality of RCTs was poor. Compared to control, honey significantly decreased postoperative pain from day 1 to day 7 (P = 0.05 to <0.0001); consumption of painkillers from days 1 to 5 (P = 0.03 to 0.003) and on day 10 (P = 0.002); and number of awakening at night due to pain on days 2 and 4 after tonsillectomy (P = 0.0001, 0.004). The healing of tonsillar fossa was significantly greater with honey compared to control on days 3–4 (P = 0.02) and days ≥9 (P = 0.01) after tonsillectomy. The adverse effects were not significantly different between honey and control groups. The Grading of Recommendation, Assessment, Development and Evaluation (GRADE) of the evidence for different outcomes varied from ‘low’ to ‘very low’.
Conclusions
Honey improved pain, requirement of painkillers and awakening at night due to pain in children after tonsillectomy. There was little improvement in healing of tonsillar fossa. The GRADE of the evidence varied from ‘low’ to ‘very low’. A good‐quality, placebo‐controlled RCT of different doses and durations of administration of honey is required to evaluate its clear efficacy and safety in children after tonsillectomy.
To study the role of mometasone furoate aqueous nasal spray for the management of adenoidal hypertrophy in children with more than 50 per cent obstruction, and to assess its impact on change in ...quality of life.
A prospective, randomised, double-blind, interventional placebo-controlled study was conducted. A total of 100 children aged 2-12 years completed treatment and follow up. The symptoms and degree of obstruction were evaluated by nasopharyngoscopy conducted pre-treatment and 24 weeks post-treatment. Subjects received mometasone furoate nasal spray at a daily dose of 200 µg for 8 weeks, followed by a dose of 200 µg on alternate days for 16 weeks. RESULTS were compared with those of a matched control group who were given saline nasal spray.
With mometasone treatment, there was an 89.8 per cent reduction in clinical symptom score, and the degree of obstruction dropped from 87 to 72 per cent (p < 0.0001). A statistically significant change in quality of life scores was seen in patients treated with the mometasone nasal spray (score change of 37.47) as compared with those given saline nasal spray (score change of 11.25) (p = 0.0001).
Mometasone nasal spray appears to be effective in treating children with obstructive adenoids.
Abstract Lymphangioma circumscriptum is an uncommon congenital skin disorder occurring commonly in limbs and genitals, and is extremely rare in tongue. Although complete surgical excision is the most ...widely used treatment, more conservative procedures such as sclerotherapy are being increasingly used for treatment of lymphangiomas. We present a series of two cases of lymphangioma circumscriptum of tongue which were treated successfully with intralesional bleomycin injection.
For the small band of investigators who study the genetics of Hirschsprung disease, or congenital aganglionosis, there was exciting news as 1994 drew to a close. In a series of papers published in ...Cell, Masashi Yanagisawa's (1,2) and may group (3) proved that the endothelin-induced signaling pathway was crucial for the development of enteric ganglia and that this pathway was genetically compromised in Hirschsprung disease (HSCR). Hosoda et al. (2) demonstrated that a null mutation created by targeted disruption of the endothelin-B receptor gene (Ednrb) in the mouse produced an autosomal recessive phenotype of white spotting and megacolon. They also showed that an identical phenotype called piebald-lethal, which maps to mouse chromosome 14, was due to a mutation in the Ednrb gene as well. In the human, earlier genetic mapping and linkage disequilibrium studies in a large, inbred Mennonite kindred segregating HSCR had already identified a susceptibility locus on human chromosome 13q22 (4). Puffenberger et al. (3) mapped the human endothelin-B receptor gene to this locus and demonstrated that a EDNRB missense mutation, which substituted cysteine for tryptophan at residue 276, segregated with HSCR in this kindred.
PDF
