bluuebunny/crossref_metadata_2025_split huggingface dataset:
Human Leukocyte Antigen Gene Testing and Carbamazepine-Induced Toxic Epidermal Necrolysis: A Study of Pediatric Practice
Authors: Asgarpour Jessica M.S. , Lam Lauren M. , Vogel Tina K. , Goez Helly R. , Fiorillo Loretta 
Published: September 2020

Background Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening drug-induced dermatologic conditions. SJS/TEN occurs in 1-10 per 10 000 patients taking carbamazepine (CBZ) (Pratt VM, McLeod HL, Rubinstein WS et al. Medical Genetics Summaries. National Center for Biotechnology Information US; 2018: 1-527). The development of SJS/TEN is associated with variable drug metabolism and presence of an at-risk HLA haplotype. HLA-B*15:02 and HLA-A*31:01 haplotypes can produce a hyperimmune response in the setting of CBZ use in patients of Asian and European descent, respectively (Schneider JA, Cohen PR. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A concise review with a comprehensive summary of therapeutic interventions emphasizing supportive measures. Adv Ther. 2017; 34:1235-1244). Objective The US Food and Drug Administration (FDA) and the Canadian pharmacogenomics Network for Drug Safety (CPNDS) recommend that patients with high-risk ethnic backgrounds should be genetic tested before initiating CBZ (Sukasem C, Chaichan C, Nakkrut T et al. Association between HLA-B Alleles and Carbamazepine-induced maculopapular exanthema and severe cutaneous reactions in Thai patients. Journal of Immunology Research. 2018; 1-11).We sought out to assess the awareness of this in prescribing practitioners and their standard of practice. Methods We created a 15-question survey and distributed to pediatric neurologists and pediatricians at the University of Alberta. We hypothesized that there was a discordance between the standard of practice and the recommendation by the FDA and CPNDS. Results The survey results indicated a lack of awareness of the at-risk ethnicities for CBZ-induced SJS/TEN. HLA gene testing was rarely done prior to initiation of CBZ in high-risk patients. In addition, there was a lack of awareness for standard of care for genetic testing in Canada and worldwide. Conclusions Our results demonstrate an evident gap between current prescriber practices and existing FDA and CPNDS recommendations to screen for HLA genotypes. We hope that this study captures the realistic potential to improve patient outcomes.
Neurological disorders in autism: A systematic review and meta-analysis
Authors: Pan Pei-Yin , Bölte Sven , Kaur Preet , Jamil Sadia , Jonsson Ulf 
Published: September 2020

The etiological significance of neurological disorders in autism is debated, but it is clear that they complicate support provision and clinical management, and can have negative impact on outcomes. This systematic review and meta-analysis explored the full range of co-occurring neurological disorders in autism. We estimated the odds of having neurological complications compared to the general population and other neurodevelopmental conditions, as well as the overall prevalence of different neurological disorders. Seventy-nine articles were eligible for the systematic review, including 28 case-control studies, 43 prevalence studies, and 8 cohort studies. Findings were heterogeneous across studies. Overall, autistic individuals were significantly more likely than the general population to exhibit epilepsy, macrocephaly, hydrocephalus, cerebral palsy, migraine/headache, and congenital abnormalities of the nervous system, with prevalence estimates ranging from 1.1% (0%–3.3%; hydrocephalus) to 14.2% (11.3%–17.2%; epilepsy). Epilepsy was also more common in autism than in attention-deficit/hyperactivity disorder (odds ratio [95% confidence interval] = 4.06 [2.81–5.88]). Findings indicate that awareness of neurological disorders and neurological check-ups are indicated in autism to ensure adequate physical health care and support. Prospective studies of neurological disorders in children diagnosed with or at risk of autism might further enhance our understanding of causal pathways. Lay abstract Neurological disorders, such as epilepsy and cerebral palsy, have been reported to occur among individuals with autism beyond chance and may have an impact on daily living across the lifespan. Although there has been research investigating neurological disorders in autism, the findings are not always conclusive. Previous summaries of existing studies have not evaluated the full range of neurological disorders. This study aimed to comprehensively explore the neurological problems appearing in autism to provide updated information that is needed for better healthcare and support in this population. We looked at already published studies focusing on risk or frequency of neurological disorders in autism. Our results suggest that individuals with autism are more likely than the general population to have a range of neurological disorders, including epilepsy, macrocephaly, hydrocephalus, cerebral palsy, migraine/headache, and inborn abnormalities of the nervous system. In order to provide individualized healthcare and support of high quality to individuals diagnosed with autism, health care professionals and other support providers need to be attentive to neurological complications. To further improve our understanding about the link between autism and neurological disorders, future research should follow the neurological health of children who are diagnosed with or are at increased likelihood of autism.
Acute Thrombosis of an Infrarenal Abdominal Aortic Aneurysm Presenting as Bilateral Critical Lower Limb Ischemia
Authors: Li Hai-Lei , Chan Yiu Che , Cui Dong-Zhe , Cheng Stephen W. 
Published: September 2020

Background: Intraluminal thrombosis in the aneurysm sac is commonly seen in abdominal aortic aneurysm (AAA). Complete acute thrombosis of an AAA is a rare and catastrophic event. Method: We report a patient with acute AAA thrombosis presenting as bilateral lower limbs critical ischemia. A79-year-old male with a past history of AAA presented with acute onset of bilateral leg pain, coolness, weakness and numbness. His lower extremity pulses including femorals were absent bilaterally on physical examination. Urgent computed tomography angiography showed an infrarenal AAA measuring 45 mm in diameter. The aneurysm sac and bilateral common iliac arteries were completely occluded. Emergency open repair of AAA and bilateral iliac thrombectomy were successfully performed under general anesthesia. Result: Bilateral femoral pulses were present and the neurological deficits were completely resolved after operation. Patient recovered well and was asymptomatic at 3-month follow-up. Conclusion: Acute thrombosis of aortic aneurysm could be an unusual cause of lower limb ischemia, prompt diagnosis followed by surgical revascularization is essential to avoid prolonged ischemia.
A Case-Control Study on the Therapeutic Effect of Mediastinoscope-Assisted and Thoracoscope-Assisted Esophagectomy
Authors: Guo Li , Zhao Qian , Wang Kairui , Zhao Duowen , Ye Xiaofeng , Li Tao 
Published: September 2020

Objective. To compare the clinical efficacies of mediastinoscope-assisted and thoracoscope-assisted esophagectomy. Materials and Methods. Seventy-six patients with esophageal cancer who underwent minimally invasive esophagectomy at the General Hospital of Ningxia Medical University between June 2015 and January 2019 were retrospectively evaluated. Among them, 28 patients underwent mediastinoscope-assisted transhiatal esophagectomy (MATHE), and 48 received thoracoscope-assisted transthoracic esophagectomy (TATTE). The perioperative clinical data and follow-up data of the 2 groups were compared. Results. All operations were successful in both groups. MATHE was favorable in terms of operation time, intraoperative blood loss, drainage volume 3 days after surgery, postoperative hospital stay, and hypoproteinemia ( P < .05). Lymph node dissections were less than those in the TATTE ( P < .05). No significant differences in long-term postoperative complications and survival rate were found between the 2 groups ( P > .05). Conclusion. MATHE has the advantages of minimal trauma, shorter operation time, less intraoperative blood loss, and faster recovery. More adequate tumor clearance in terms of lymph node dissection can be achieved with TATTE. However, the comparison of survival rates between the 2 groups is similar.
Does the Presence of Neuropathic Pain Influence the Response to Hyaluronic Acid in Patients with Knee Osteoarthritis?
Authors: Tiendrebeogo Evarice , Choueiri Magda , Chevalier Xavier , Conrozier Thierry , Eymard Florent 
Published: September 2020

Objectives Up to 50% of patients with symptomatic knee osteoarthritis (OA) present with neuropathic pain (NP) features. We assessed the impact of NP according to DN4 (Douleurs Neuropathiques 4 questions) score on the response to intra-articular (IA) hyaluronic acid (HA) injections and the effects of HA injections on NP. Materials and Methods We conducted a post hoc analysis from a multicenter, randomized, double-blind, noninferiority trial comparing the efficacy of 2 HA in symptomatic knee OA at 24 weeks. At baseline, demographic, anthropometric, radiologic data, and symptoms were recorded. The symptomatic effect of HA was assessed by VAS pain, patient global assessment (PGA), WOMAC, DN4, and OMERACT-OARSI response. Results A total of 187 patients were included. NP according to DN4 score was present in 20 patients (10.7%) at baseline. Most common positive DN4 items were tingling (36.9%) and burning (36.4%). NP was associated with WOMAC pain score ( P = 0.02). The presence of NP at baseline did not affect the symptomatic improvement after HA injections according to the VAS pain ( P = 0.71), PGA ( P = 050), WOMAC pain ( P = 0.89), WOMAC function ( P = 0.52), and rate of OMERACT-OARSI responders ( P = 0.21). The prevalence of patients with NP decreased by 50% ( n = 10) at 24 weeks after HA injections. Most improved DN4 items were itching (90%), hypoesthesia to pinprick (88%), and burning (50%). Conclusion In our study, NP was associated with pain severity, but did not influence the response to IA HA. On the other hand, HA injections reduced some NP features, especially itching, sting hypoesthesia, and burning.
Location Distribution of 2,087 Osteochondral Lesions of the Talus
Authors: van Diepen Pascal R. , Dahmen Jari , Altink J. Nienke , Stufkens Sjoerd A.S. , Kerkhoffs Gino M.M.J. 
Published: September 2020

Objective The primary aim of this study was to evaluate the exact location distribution in patients with osteochondral lesions of the talus (OLTs) using a 9-grid scheme. The secondary aim is to match lesion location to lesion size, arthroscopic or open operation, and trauma occurrence. Methods A systematic review was performed in the databases PubMed, EMBASE, and Cochrane. Search terms consisted of “talus” and “osteochondral lesion.” Two independent reviewers evaluated search results and conducted the quality assessment using the Methodological Index for Non-Randomized Studies (MINORS). Primary outcome measure was OLT location in the 9 zone-grid. Secondary outcome measures were OLT size in 9-zones, preoperative radiological modality use, demographic lesion size variables as well as open or arthroscopic treatment. Results Fifty-one articles with 2,087 OLTs were included. Heterogeneity concerning methodological nature was observed and methodological quality was low. The posteromedial (28%) and centromedial (31%) zones combined as one location was the location with the highest incidence of OLTs with a rate of 59%. Individual OLT size was reported for only 153 lesions (7%). Preoperative combination of X-ray and magnetic resonance imaging (MRI), and/or computed tomography (CT) was reported in 20 studies (43%). Trauma was reported in 78% of patients. Furthermore, 67% was treated arthroscopically and 76% received primary OLT treatment. Conclusion The majority of OLTs are located in the posteromedial and centromedial zone, while the largest OLTs were reported in the centrocentral zone. Further research is required to identify the prognostic impact of location occurrence on the outcomes following OLT treatment.
Malignancy risk in adults with growth hormone deficiency undergoing long-term treatment with biosimilar somatropin (Omnitrope®): data from the PATRO Adults study
Authors: Beck-Peccoz Paolo , Höybye Charlotte , Murray Robert D , Simsek Suat , Zabransky Markus , Zouater Hichem , Stalla Günter 
Published: September 2020

Background: To assess the safety (particularly the occurrence of malignancies) of growth hormone (GH) replacement (Omnitrope®) in adults with GH deficiency, using data from the ongoing PATRO Adults post-marketing surveillance study. Methods: PATRO Adults is being conducted in hospitals and specialized endocrinology clinics across Europe. All enrolled patients who receive ⩾1 dose of Omnitrope® are included in the safety population. Malignancies are listed as adverse events under the MedDRA System Organ Class ‘neoplasms, benign, malignant and unspecified (including cysts and polyps)’. Results: As of July 2018, 1293 patients had been enrolled in the study and 983 (76.0%) remained active in the study. Approximately half [ n = 637 (49.3%)] of the patients were GH treatment-naïve on study entry. The majority of enrolled patients had multiple pituitary hormone deficiency ( n = 1128, 87.2%). A total of 41 on-study malignancies were reported in 33 patients (2.6%; incidence rate 7.94 per 1000 patient-years). The most common cancers were basal cell carcinoma ( n = 13), prostate ( n = 6), breast, kidney and malignant melanoma (each n = 3). Treatment with Omnitrope® was discontinued following diagnosis of malignancy in 16 patients. The tumors occurred after a mean of 79.4 months of recombinant hormone GH (rhGH) treatment overall. Conclusion: Based on this snapshot of data from PATRO Adults, Omnitrope® treatment is tolerated in adult patients with GH deficiency in a real-life clinical practice setting. Our results do not generally support a carcinogenic effect of rhGH in adults with GH deficiency, although an increased risk of second new malignancies in patients with previous cancer cannot be excluded based on the current dataset.
Holmium laser lithotripsy with semirigid ureterorenoscopy in treatment of upper ureteral and renal pelvis stones under spinal anesthesia
Authors: Aras Bekir , Alkiş Okan , İvelik Halil İ , Sevim Mehmet , Başer Aykut 
Published: September 2020

Extracorporeal shockwave lithotripsy and especially flexible ureterorenoscopy (URS) are first-choice treatments of upper ureteral and renal pelvis stones. In our study, we aimed to demonstrate that the treatment of upper ureteral and renal pelvis stones smaller than 2 cm could be performed by semirigid URS under spinal anesthesia. The data of 292 patients were retrospectively analyzed. Stones were localized in upper ureter and renal pelvis in 191 (65.4%) and 101 (34.6%) patients, respectively. The stone-free rate was found to be 72.6%. The final stone-free rate was 95.2% after additional treatments administered to 80 patients with residues. While no peri-operative complication was observed, hematuria was observed in three patients during the post-operative period, high fever was observed in 27 patients and sepsis was observed in four patients. It is a well-known fact that flexible URS is the first-choice surgical method in the treatment of upper ureteral and renal pelvis stones. In the current study, we aimed to investigate whether laser lithotripsy with semirigid URS could also be a good and cheap alternative in clinical practice, and if upper urinary tract surgeries could be performed with spinal anesthesia especially in patients with a high risk of general anesthesia. Level of evidence:
Integrating a Multidisciplinary Pain Team and Chiropractic Care in a Community Health Center: An Observational Study of Managing Chronic Spinal Pain
Authors: Prater Christopher , Tepe Melissa , Battaglia Patrick 
Published: September 2020

Background Chronic spinal pain is one of the most common diseases in the United States. Underserved patients are most affected, and disproportionately may use opioid medications as they lack access to other therapies. It is therefore important to develop systems to treat spinal pain within the primary medical home. Methods We designed a prospective observational pilot study at a community health center to measure the effectiveness of two interventions among an underserved population: a multidisciplinary pain team and chiropractic care. Study outcomes were pain and functional disability measured by the Pain Disability Questionnaire (PDQ), and reduction of opioid dose at baseline and 6-12 months. Multivariate linear regression was used to determine associating factors for change in PDQ scores. Results Thirty-five individuals completed baseline and follow-up PDQs from August 2018 to May 2020. Overall, the mean baseline PDQ was 92.4 +/− 6.1 and the mean follow-up PDQ was 81.9 +/− 7.7, resulting in a mean improvement of −10.6 (95% CI 1.2 - −22.3, P = .08). Participants in the chiropractic team (mean change −25.0, P = .01) and those completing the study before COVID-19 (mean change = −22.6, P < .01) were found to have significantly greater improvement at follow-up. Conclusion This observational study within a community health center resulted in improvement in spinal pain and disability with chiropractic care versus a multidisciplinary pain team. Offering similar services in primary care may help to address pain and disability, and hopefully limit external referrals, advanced imaging, and opioid prescriptions.
Primary stability of a cementless modular revision hip stem in relation with the femoral defect size: A biomechanical study
Authors: Ellenrieder Martin , Souffrant Robert , Schulze Christian , Kluess Daniel , Mittelmeier Wolfram , Bader Rainer 
Published: September 2020

Purpose: Cementless modular fluted hip stems are commonly used in revision arthroplasty. Nevertheless, there is a wide spectrum of recommendations concerning the minimum bone stock required to enable osseous ingrowth and implant–bone micromotions <100 µm. This experimental study investigated the primary stability of a tapered cementless fluted revision stem depending on different types of bone defects. Methods: Implant–bone interface movements with a bimodular stem were examined under cyclic axial and torsional loading using composite femora. In four degrees of freedom, the implant subsidence and micromotions were captured with linear variable differential transformers for the intact femora and seven different defects ranging from Paprosky type I to type IIIB. Results: With a 7-cm length of intact diaphysis proximal to the isthmus (Paprosky IIIA), mean implant–bone micromotions of 66 µm occurred. An implant–bone contact zone of only 5 cm (Paprosky IIIA) resulted in micromotions notably over 100 µm and significantly increased subsidence ( p < 0.05). With a Paprosky IIIB defect (3 cm of intact diaphysis) rotational instability occurred in all specimens. Conclusions: Aside from critically increased interfacial micromotions (>100 µm), rotational instability emerged as a mechanism of fixation failure when the implant–bone contact zone was only 5 cm or less. Hence, future studies investigating the implant fixation in the case of femoral bone defects should consider both axial and torsional loading. With regard to the clinical application, our data suggest maintaining 7 cm of diaphyseal implant–bone contact for a safe anchorage of cementless fluted hip revision stems.
The LPI as an Effective Ground-level Strategy for the Deinstitutionalisation and Quality Alternative Care of Children
Authors: Sudesh G. A. Wasana 
Published: September 2020

Deinstitutionalisation and quality alternative care is a topic that is widely discussed in search of policies, strategies and good practices. This issue affects all children directly or indirectly. It is very pertinent to the South Asia region. Children who are in institutional care require deinstitutionalisation process that involves both prevention and a range of alternative care options that are community-based, family-based or family-like care. The Local Process Initiative (LPI), which was implemented in the Devinuwara Divisional Secretariat Division (DSD) in the Matara District of Sri Lanka, is an effective strategy that demonstrates deinstitutionalisation and quality alternative care. SOS Children’s Villages of Sri Lanka in seven locations including Devinuwara DSD since 2017 have carried out the LPI process successfully. It has generated positive outcomes especially in terms of strengthening the ground-level state mechanism for deinstitutionalisation and alternative care for children. The divisional secretary is the lead person in the ‘locational circle’ formed in this process, which composes of a range of field-level government officers involved in childcare and community development as they are the first point of contact for issues related to children and families. The government officers are considered the ‘change agents’, and their empowerment is at the centre of the LPI strategy and its success. From the LPI process in the Devinuwara DSD, it is expected to develop a model to avoid family separation. In here, it is expected to develop a ground-level deinstitutionalisation and a quality alternative care strategy which incorporate solutions from biological, psychological and sociological perspectives to build confidence of stakeholders on deinstitutionalisation and quality alternative care for children, and document evidence including success stories in order to support deinstitutionalisation and quality alternative care of children. This article offers an LPI process in the Devinuwara DSD as an effective strategy for the deinstitutionalisation and quality alternative care of children in South Asia.
Bordetella Holmesii: An Unusual Cause of Endogenous Endophthalmitis in a Patient With Sickle Cell Disease
Authors: Pegany Roma B. , George Roshan T. , Zhang Alice Yang 
Published: September 2020

Purpose: This case report describes a rare organism causing endogenous endophthalmitis in a patient with sickle cell disease. Methods: A case report was conducted. Results: A 41-year-old man with sickle cell disease presented with acute onset of blurry vision of the right eye. His visual acuity was counting fingers in the right eye and 20/20 in the left eye. He had ophthalmic findings of hypopyon and vitritis in the right eye, consistent with endophthalmitis. He was treated with intravitreal and systemic antibiotics. Vitreous cultures grew Bordetella holmesii. His visual acuity at follow-up visits improved to 20/40 in the setting of improved vitritis. Conclusions: This is the first case describing B holmesii, a rare causative organism of endogenous endophthalmitis, in a patient with sickle cell disease. More studies are needed to improve the early detection and treatment of this unusual organism.