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No guts, absolutely no fame: underestimating the benefits of delivering youngsters with mechanistic details.
In craniomaxillofacial surgery we often deal with hypoplastic mandibles and mandibular asymmetries, the correction of which is critical to obtaining acceptable aesthetic results. In all of them we find common skeletal problems once growth has finished, such as an inclined occlusal plane and facial asymmetry with a stable dental occlusion. Simultaneous maxillomandibular distraction, which involves a Le Fort I osteotomy and a mandibular osteotomy with intermaxillary fixation during the period of active distraction, is an excellent technique to solve these problems. Virtual surgical planning, stereolithographic models, and surgical guides are supportive tools for obtaining excellent results. In this paper we present our experience with five cases of hypoplastic mandibles and mandibular asymmetries of different aetiologies. In all patients we achieved a considerable improvement in their physical appearance in the distance between the lateral canthus and oral commissure, the height of the mandibular ramus, the inclination of the occlusal plane, and the medial position of the chin. The benefits of virtual surgical planning in terms of choosing the optimal vector and the amount of distraction make it a promising technological tool to achieve excellent outcomes. Oromandibular dystonia (OMD) is characterised by sustained or repetitive involuntary movements of the jaw, face, and tongue. People with the condition may present to their dentist, general practitioner, or a secondary care specialist with non-specific symptoms including jaw or facial pain, bruxism, subluxations or dislocations of the jaw; fractured teeth or dental restorations, or both; or jaw tremor. Many clinicians are not aware of the disorder and this can lead to delayed diagnoses, unnecessary complications, and inappropriate treatment. OMD is an important diagnosis not to miss because referral for specialist management can provide good long-term results. To aid early, accurate diagnosis, this paper focuses on the key clinical features of the disorder and its dental and medical mimics. The aim of this review was to critically investigate and assess the evidence relating to the use and efficacy of botulinum toxin (BTX) in the management of temporomandibular joint disorders (TMD) and masticatory myofascial pain. A comprehensive search was conducted of PubMed, Scopus, Embase, and Cochrane CENTRAL, to find relevant studies from the last 30 years up to the end of July 2018. Seven were identified. Three showed a significant reduction in pain between the BTX and placebo groups and one showed a clinical, but not a significant, difference. In one that compared BTX with another novel treatment, myofascial pain reduced equally in both groups, and in the remaining two there was no significant difference in pain reduction between the BTX and control groups. Of the four studies that assessed mouth opening, two reported that BTX had resulted in a slight improvement; one reported no improvement, and the other a worsening of the condition. A meta-analysis was not possible because of the considerable variation in the studies' designs, the heterogeneity between the groups, and the different assessment tools used. Despite showing benefits, consensus on the therapeutic benefit of BTX in the management of myofascial TMD is lacking. Further randomised controlled trials with larger sample sizes, minimal bias, and longer follow-up periods are now needed. Infants born very preterm have a variable baseline risk of bronchopulmonary dysplasia (BPD). Using the example of evidence-based drug therapies to prevent BPD, we designed a visual aid that displays the "number needed to treat" with CIs for caffeine, vitamin A, and hydrocortisone over a range of baseline risks. OBJECTIVE To assess the importance of genetic and nongenetic risk factors contributing to hepatic fat accumulation in a multiethnic population of youth. STUDY DESIGN We investigated the relationship between genetic factors and hepatic fat fraction (HFF) in 347 children aged 12.5-19.5 years. We examined 5 single nucleotide polymorphisms previously associated with HFF and a weighted genetic risk score (GRS) and examined how these associations varied with ethnicity (Hispanic vs non-Hispanic white) and body mass index (BMI) category. We also compared how much variation in HFF was explained by genetic factors vs cardiometabolic factors (BMI z-score and the Homeostasis Model of Insulin Resistance) or diet. RESULTS PNPLA3 rs738409 and the GRS were each associated with HFF among Hispanic (β = 0.39; 95% CI, 0.16-0.62; P = .001; and β = 0.20; 95% CI, 0.05-0.34; P = .007, respectively) but not non-Hispanic white (β = 0.04; 95% CI, -0.18 to 0.26; P = .696; and β = 0.03; 95% CI, -0.09 to 0.14; P = .651, respectively) youth. Cardiometabolic risk factors explained more of the variation in HFF than genetic risk factors among non-lean Hispanic individuals (27.2% for cardiometabolic markers vs 6.4% for rs738409 and 4.3% for the GRS), and genetic risk factors were more important among lean individuals (2.7% for cardiometabolic markers vs 12.6% for rs738409 and 4.4% for the GRS). CONCLUSIONS Poor cardiometabolic health may be more important than genetic factors when predicting HFF in overweight and obese young populations. Genetic risk is an important contributor to pediatric HFF among lean Hispanics, but further studies are necessary to elucidate the strength of the association between genetic risk and HFF in non-Hispanic white youth. Published by Elsevier Inc.OBJECTIVE To examine disparities in the diagnosis of leukodystrophies including geographic factors and access to specialty centers. STUDY DESIGN Retrospective cohort study of pediatric patients admitted to Pediatric Health Information System hospitals. Patients with leukodystrophy were identified with International Classification of Diseases, Tenth Revision, Clinical Modification diagnostic codes for any of 4 leukodystrophies (X-linked adrenoleukodystrophy, Hurler disease, Krabbe disease, and metachromatic leukodystrophy). We used 3-level hierarchical generalized logistic modeling to predict diagnosis of a leukodystrophy based on distance traveled for hospital, neighborhood composition, urban/rural context, and access to specialty center. RESULTS We identified 501 patients with leukodystrophy. Patients seen at a leukodystrophy center of excellence hospital were 1.73 times more likely to be diagnosed than patients at non-center of excellence hospitals. Patients who traveled farther were more likely to be diagnosed than those who traveled shorter. Patients living in a Health Professionals Shortage Area zip code were 0.86 times less likely to be diagnosed than those living in a non-Health Professionals Shortage Area zip code. CONCLUSIONS Geographic factors affect the diagnosis of leukodystrophies in pediatric patients, particularly in regard to access to a center with expertise in leukodystrophies. Our findings suggest a need for improving access to pediatric specialists and possibly deploying specialists or diagnostic testing more broadly. OBJECTIVE To characterize current youth perspectives of prescription pain medication. STUDY DESIGN In total, 1047 youths aged 14-24 years were recruited by targeted social media advertisements to match national demographic benchmarks. Youths were queried by open-ended text message prompts about exposure and access to prescription pain medication, perceived safety of prescribed and nonprescribed medication, and associations with the word "opioid." Responses were analyzed inductively for emerging themes and frequencies. RESULTS Among 745 respondents (71.2% response rate), 439 identified as female (59.3%), 561 as white (75.8%), and mean age was 18.3 ± 3.2 years. Previous exposure to prescription pain medication was reported by 377 respondents (52.0%), most commonly related to dentistry (32.8%), surgery (19.2%), and injury (12.0%). Nonmedical sources of access to prescription pain medication were identified by 256 respondents (36.9%) and medical sources other than their doctor by an additional 111 respondents (16.0%). Three additional themes emerged from youth responses (1) prescribed medication was thought to be safer than nonprescribed medication, based on trust in doctors; (2) risks of addiction and overdose were thought to be greater for nonprescribed medication; (3) respondents had a widely ranging understanding of the word "opioid," from historical to current events, medical to illicit substances, and personal to public associations. CONCLUSIONS Although youths are aware of the opioid crisis, they perceive less risk of prescription pain medication prescribed by a doctor, than from other sources. Policies should target education to youth in clinical and nonclinical settings, highlighting the risks of addiction and overdose with all opioids. A mixed methods approach was used to examine perspectives of 114 primary care providers regarding barriers and needs for support in caring for children with autism. The most common barriers related to lack of knowledge and resources for diagnosing and treating children with autism, and inadequate visit time and reimbursement. CONTEXT There is a critical need for effective bladder-sparing therapies for bacillus Calmette-Guérin (BCG)-unresponsive non-muscle-invasive bladder cancer (NMIBC). Owing to the current lack of effective agents that can be used as a control, the US Food and Drug Administration began to accept single-arm trials for patients with carcinoma in situ (CIS), using complete response rate (CRR) and duration of response as the primary endpoints to support marketing applications. Despite the ensuing growth of clinical trials in this space, no consensus exists on a clinically relevant benchmark for CRR. OBJECTIVE To elucidate the CRR and recurrence-free rate (RFR) using bladder-sparing agents after BCG failure in order to provide a frame of reference for future clinical trial results. EVIDENCE ACQUISITION We performed a systematic review of clinical trials utilizing bladder-sparing therapeutics for NMIBC recurring after intravesical BCG (PROSPERO CRD42019130553). The search was performed in MEDLINE, EMBASE, and Cochranetoxicities. CONCLUSIONS We demonstrate that, to date, bladder-sparing therapies achieved modest efficacy in patients with NMIBC after BCG. Results from the current study will serve as a frame of reference for emerging trial results in the BCG-unresponsive space. PATIENT SUMMARY In this study, we found that bladder-sparing therapies achieved modest efficacy in patients with non-muscle-invasive bladder cancer after bacillus Calmette-Guérin (BCG). These results will serve to inform future clinical trial results for salvage agents used to treat BCG-unresponsive bladder cancer. BACKGROUND Transcatheter closure of patent ductus arteriosus (PDA) is an effective alternative to surgical ligation in preterm infants. However, data on device deformation and risk of left pulmonary artery (LPA) obstruction remain scant. This study describes the outcomes and complications of transcatheter closure of PDA in preterm infants weighing less then 2500 g. METHODS Amplatzer Piccolo Occluder and Amplatzer Vascular Plug were used. Echocardiography was repeated at prespecified intervals. The device waist and length were assessed through lateral fluoroscopy immediately and at least 3 months after deployment. RESULTS Fourteen infants were prospectively enrolled (mean procedural weight 1335 g, procedural age 24 days), and all procedures were successful. There was no obstruction of adjacent vessels immediately after deployment. At follow-up, three infants developed aortic coarctation, all of which resolved gradually. Obstruction of the LPA occurred in eight infants, with five being severe cases. this website Compared with the shape immediately after deployment, the devices became significantly more flattened and lengthened at follow-up in patients with LPA obstruction.
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