bluuebunny/crossref_metadata_2025_split huggingface dataset:
Treat the congenital pseudarthrosis of the Tibia with Ilizarov technology
Authors: Kong Ling Dong , Cheng Han Xiong , Nie Tao 
Published: December 2018

Abstract

Rationale:
According to previously published studies, treating pediatric patients with congenital pseudarthrosis of the tibia (CPT) is challenging. More effective methods are needed to treat this patient population.

Patient concerns:
A 7-year-old girl with congenital pseudarthrosis of the distal tibia, previously misdiagnosed as a fracture, underwent plate fixation. Unfortunately, the steel plate broke after 3 months, which led to ankle angle deformity.

Diagnoses:
Congenital pseudarthrosis of the tibia.

Intervention:
Treat the congenital pseudarthrosis of the tibia with Ilizarov technology.

Outcomes:
lower limb force recovery, bone union, and lower-limb-length equalization were achieved. Congenital pseudarthrosis of the tibia were cured.

Lessons:
The Ilizarov technique is a safe, effective, and practical treatment option for patients with CPT.
Serum amyloid A1 (SAA1) gene polymorphisms in Japanese patients with adult-onset Still's disease
Authors: Yashiro Makiko , Furukawa Hiroshi , Asano Tomoyuki , Sato Shuzo , Kobayashi Hiroko , Watanabe Hiroshi , Suzuki Eiji , Nakamura Tadashi , Koga Tomohiro , Shimizu Toshimasa , Umeda Masataka , Nonaka Fumiaki , Ueki Yukitaka , Eguchi Katsumi , Kawakami Atsushi , Migita Kiyoshi 
Published: December 2018

Abstract
Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder in which inflammasome activation plays a pathophysiological role. In view of the inflammatory nature of AOSD, we investigated whether serum amyloid A (SAA) gene polymorphisms affect the susceptibility of patients with AOSD.
Eighty-seven Japanese patients with AOSD and 200 healthy Japanese subjects were recruited in this study. The genotypes of the -13C/T SNP in the 5′-flanking region of the SAA1 gene (rs12218) and two SNPs within exon 3 of SAA1 (2995C/T and 3010C/T polymorphisms) were determined using polymerase chain reaction fragment length polymorphism (PCR-RFLP) assay in all subjects. In AOSD patients, exons 1, 2, 3, and 10 of the MEFV gene were also genotyped by direct sequencing.
The frequency of the SAA1.3 allele was increased in AOSD patients compared with that in healthy subjects (43.1% versus 37.5%), but the difference was not significant. The −13T allele was more frequently observed in AOSD patients than in healthy subjects (50.6% versus 41.0%, P = .0336). AOSD patients with the −13T allele had been treated with immunosuppressants more frequently than those without this allele. MEFV mutations were detected in 49 patients with AOSD (49/87, 57.3%). AOSD patients with MEFV variants frequently exhibit macrophage activation syndrome, but the difference was not significant (34.7% versus 18.4%, P = .081). Also, there was no significant difference in SAA1 -13C/T allele frequency between AOSD patients with and without MEFV mutations.
Our data shows a significant association between T allele of rs12218 and AOSD in Japanese population.
Comparison of oncological benefits of deep neuromuscular block in obese patients with gastric cancer (DEBLOQS_GC study)
Authors: Lee Yoontaek , Ha Donghwan , An Liang , Jang You-Jin , Huh Hyub , Lee Chang Min , Kim Yeon-Hee , Kim Jong-Han , Park Seong-Heum , Mok Young-Jae , Lee Il Ok , Kwon Oh Kyoung , Kwak Kyung Hwa , Min Jae Seok , Kim Eun Jin , Choi Sung Il , Yi Jae Woo , Jeong Oh , Jung Mi Ran , Bae Hong Bum , Park Joong-Min , Jung Yong Hoon , Kim Jin-Jo , Kim Dal Ah , Park Sungsoo 
Published: December 2018

Abstract

Purpose:
Many studies have demonstrated the advantage of maintaining intraoperative deep neuromuscular block (NMB) with sugammadex. This trial is designed to evaluate the impact of muscle relaxation during laparoscopic subtotal gastrectomy on the oncological benefits, particularly in obese patients with gastric cancer.

Materials and methods:
This is a double-blind, randomized controlled multicenter prospective trial. Patients with clinical stage I–II gastric cancer with a body mass index of 25 and over, who undergo laparoscopic subtotal gastrectomy will be eligible for trial inclusion. The patients will be randomized into a deep NMB group or a moderate NMB group with a 1:1 ratio. A total of 196 patients (98 per group) are required. The primary endpoint is the number of harvested lymph nodes, which is a critical index of the quality of surgery in gastric cancer treatment. The secondary endpoints are surgeon's surgical condition score, patient's sedation score, and surgical outcomes including peak inspiratory pressure, operation time, postoperative pain, and morbidity.

Discussion:
This is the first study that compares deep NMB with moderate NMB during laparoscopic gastrectomy in obese patients with gastric cancer. We hope to show the oncologic benefits of deep NMB compared with moderate NMB during subtotal gastrectomy.

Trial registration number:
ClinicalTrials.gov (NCT03196791), date of registration: October 10, 2017.
New radiological findings and radiculomegaly in oculofaciocardiodental syndrome with a novel BCOR mutation
Authors: Kato Joji , Kushima Kazuhiko , Kushima Fumikazu 
Published: December 2018

Abstract

Rationale:
Oculofaciocardiodental syndrome (OFCD) patients who show radiculomegaly are very rare. We treated a new OFCD patient orthodontically, and performed longitudinal observation for 30 years. New findings, termed calcified-dental-papillae (CDPs) beneath open-apices (OAs) of developing radiculomegalies, pulp-stone-like-calcifications (PSLCs) and the process of radiculomegaly development were observed. A novel mutation of BCL-6 interacting corepressor (BCOR) was identified. Cone-beam-computed-tomography (CBCT) images of the radiculomegalies clarified their morphology.

Patient concerns:
A female patient and her parents were referred to orthodontic clinic for alignment of the teeth.

Diagnosis:
A CDP that harbored bulbous-round-calcified-tissue in the dental papilla beneath the OA of a developing radiculomegaly was found radiographically. PSLCs were observed in the dental pulp. Genetic analysis revealed a novel mutation c.265G>A on Exon 4 and diagnosed as OFCD. CBCT images confirmed round-calcified-tissue and PSLC and that the length of an affected canine was 38.0 mm and calculated as +14.8SD. These novel findings were not observed in lateral incisors and molars.

Interventions:
Observation was performed for 29 years and 3 months including orthodontic treatment for 2 years and 9 months.

Outcome:
Longitudinal follow-up for 26 years and 7 months after the treatment revealed that the development of radiculomegaly every few months or years, CDPs beneath OAs and PSLCs were observed. CDPs, PSLCs, and OAs were associated with radiculomegaly. The patient and the affected teeth including aligned teeth showed no particular change after the completion of the radiculomegaly. CBCT images showed bulbous-calcified-tissue and PSLCs in the mature dental pulp associated with radiculomegaly.

Lessons:
The radiographical findings of CDP, OA and PSLC help early diagnose of OFCD and have importance for initiating orthodontic treatment until radiculomegaly completion.
Arterial involvement in Erdheim–Chester disease
Authors: Villatoro-Villar Michel , Bold Michael S. , Warrington Kenneth J. , Crowson Cynthia S. , Goyal Gaurav , Shah Mithun , Go Ronald S. , Koster Matthew J. 
Published: December 2018

Abstract
Erdheim-Chester disease (ECD) is a rare histiocytosis of the “L” (Langerhans) group with multisystem involvement that can affect the large and medium-sized arteries mimicking vasculitis. Aortic involvement is common but the frequency and outcome of aortic branch vessel abnormalities are less well described.
Patients with ECD were retrospectively identified. Images containing information of arterial involvement within 6 months of diagnosis were considered baseline and compared to last follow-up studies. Two physicians independently reviewed the studies to evaluate for presence of abnormalities attributable to ECD. Age and sex-adjusted logistic regression models were used to examine associations between patient characteristics and vessel involvement at baseline.
Among a cohort of 64 patients with ECD, 63 had baseline imaging of vascular structures. ECD involvement of at least 1 segment of the aorta was observed in 56%. Abnormalities were also observed in aortic arch branches (26%), visceral branch arteries (40%), iliofemoral arteries (31%), coronary (5%), and pulmonary (3%) arteries. Perinephric fibrosis was strongly associated with the identification of abnormalities in the thoracic aorta (OR 4.92 [1.54, 15.75]; P
= .007), abdominal aorta (OR 7.57 [2.28, 25.07]; P = .001) and visceral branch arteries (OR 6.05 [1.52, 24.03]; P = .01) but not pelvic/lower extremity arteries. Complete normalization of arterial abnormalities at follow-up was only observed in 9% or less of arterial segments involved at baseline.
Aortic and aortic branch vessel abnormalities are frequently observed in patients with ECD and are often asymptomatic. Partial and/or complete resolution of arterial findings is uncommon.
Asymmetric pattern in generalized myasthenia gravis
Authors: Carvalho Thiago Pereira de , Vianna Clara Louise , Andrade Felipe Freitas , Alvarenga Rafaela Abreu , Sales Liz de Oliveira Moura , Rodrigues Rosana Souza , Rosado-de-Castro Paulo Henrique 
Published: December 2018

Abstract

Rationale:
Myasthenia gravis (MG) is an uncommon autoimmune disease mediated by antibodies that attack the postsynaptic acetylcholine receptors (AchRs) at the neuromuscular junction, causing fluctuating muscle weakness, aggravated with use, and relieved with rest.

Patient concerns:
A 32-year-old woman with a diagnosis of MG based on clinical findings, eletroneuromyography, and brain magnetic resonance imaging (MRI) was admitted to our hospital with dysphagia, dysphonia, diplopia, and intense weakness, associated with a 2-day viral gastroenteritis.

Diagnoses:
Physical examination revealed globally reduced deep tendon reflexes, and tetraparesis, with muscle strength grade 4 in the left limbs and grade 2 in the right limbs. Autoantibody dosing against AchR was elevated and computed tomography scan of the thorax revealed a thymic remnant.

Interventions:
Pyridostigmine and human immunoglobulin were infused.

Outcomes:
After 7 days of the reintroduction of pyridostigmine and human immunoglobulin infusion, the patient developed complete resolution of symptoms, being discharged from the hospital. Her symptoms are still well controlled 6 months later. The patient was evaluated by a thoracic surgeon and is awaiting elective thymectomy.

Lessons:
Although our patient presented all the diagnostic criteria of MG, the markedly asymmetric limb weakness presented in previous history and clinical examination of admission was an unusual and unexpected presentation, especially considering the pathophysiology of the disease. MRI of brain without abnormalities was fundamental to rule out another associated etiology. Our review of the literature revealed just 1 case report of MG with similar presentation. This clinical manifestation becomes valuable because it contributes to the list of unexpected presentation that should motivate its suspicion.
Minimally invasive surgery for inflammatory myofibroblastic tumor of the urinary bladder
Authors: Xu He , He Ben , Tu Xiang , Bao Yige , Yang Lu , Zhuo Hui , Wei Qiang 
Published: December 2018

Abstract

Rationale:
Inflammatory myofibroblastic tumors of the urinary bladder (IMTUB) is exceptionally rare. Currently, no standardized treatment has been established for IMTUBs.

Patient concerns:
Herein we report three cases presenting with hematuria and anemia. A 25-year-old man experienced painless gross hematuria for 2 days and the hemoglobin level continuously dropped to 88 g/L; a 72-year-old man complaining of gross hematuria for seven days; and a 33-year-old woman presenting with gross hematuria, urgency, and frequency for the duration of 20 days, with a hemoglobin level of 61 g/L.

Diagnosis:
Ultrasonography, contrast-enhanced computed tomography (CT) scan and magnetic resonance image (MRI) indicated masses of different sizes on the walls of the urinary bladders. Diagnostic transurethral resection of bladder tumor (TURBT) was performed which revealed the diagnosis of IMTUB.

Interventions:
In our cases, we removed the tumors completely with a minimally invasive approach. The first patient received TURBT only. The other patients underwent further laparoscopic and robot-assisted laparoscopic partial cystectomy respectively for the incomplete resection of tumor by diagnostic TURBT.

Outcomes:
Histology of the resected specimen had proliferation of spindle cells with inflammation consistent with IMTUB. Immunohistochemical staining revealed that the tumor cells were positive for anaplastic lymphoma kinase (ALK), Vimentin and Ki-67 (20%–40%), negative for smooth muscle actin (SMA), S-100 and desmin confirming the diagnosis of IMTUB. Follow-up cystoscopy and CT or MRI (mean follow-up period: two years) did not detect any local recurrence or distant metastasis.

Lessons:
Bladder-sparing treatment by TURBT or partial cystectomy remains the main mode of treatment for IMTUB. Laparoscopic and robot-assisted laparoscopic approach is safe and may yield satisfactory oncological and functional results. Regular follow-up protocol is necessary after operation.
Clinical and prognostic analyses of 110 patients with N3 nasopharyngeal carcinoma
Authors: Chen Jing , Liu Tongxin , Sun Quanquan , Hu Fujun 
Published: December 2018

Abstract

Objective:
To analyze the clinical outcome and prognostic factors of N3 nasopharyngeal carcinomas (NPCs), provide a basis for rational treatment and improve the cure rate.

Methods:
A total of 110 patients with a pathologically confirmed diagnosis of N3 (NPC 2008 stage in China) NPC from our hospital were retrospectively included in the study conducted from April 2007 to July 2011. All patients received intensity-modulated radiation therapy. Some of these patients received various chemotherapies. The doses of the planning gross primary tumor and retropharyngeal lymph node volume, high-risk planning tumor volume, low-risk planning tumor volume, and gross tumor volume of neck lymph nodes were 6000 to 7600, 5400 to 6600, 5000 to 6000, and 6000 to 6996 cGy, respectively. The Kaplan–Meier analysis and logrank test were carried out to calculate and compare the survival rates of the patients, and the Statistical Package for the Social Sciences software version 17.0 was used for all analyses. Meanwhile, the Cox model was used to analyze the prognostic factors.

Results:
In this study, the 1-, 3-, and 5-year overall survival rates of the patients were 92.63%, 83.16%, and 70.53%, respectively. Based on the univariate analysis, T stage (P = .043) and chemotherapy (P
= .003) were significant factors for survival. In the multivariate analysis, only chemotherapy influenced survival (Table 1

JOURNAL/medi/04.03/00005792-201812070-00076/table1/v/2024-05-24T194536Z/r/image-tiff

). Recent toxicity included radioactive oral mucosa inflammation and skin injury, and difficulty opening the mouth and hearing loss were considered late adverse reactions. None of the patients died during treatment.

Conclusions:
Patients with N3 NPC are at high risk of distant metastasis, and their 5-year survival rate is poor. The more important prognostic factors were T stage and chemotherapy. Patients with N3 NPC should be treated with combined chemotherapy and radiotherapy.
Emergence of daptomycin non-susceptible coagulase-negative Staphylococci in patients with cardiovascular device infections
Authors: Hagiya Hideharu , Sugawara Yo , Kimura Keigo , Hamaguchi Shigeto , Nishi Isao , Hayashi Masahiro , Akeda Yukihiro , Tomono Kazunori 
Published: December 2018

Abstract

Rationale:
Daptomycin (DAP) is a key drug for treating severe Staphylococcus infections. The emergence of DAP non-susceptible Staphylococcus aureus has been widely recognized in clinical situations, although the clinical status of DAP non-susceptible coagulase-negative Staphylococcus (CoNS) infections is unclear. We encountered 2 cases of cardiovascular device infections that were associated with DAP non-susceptible CoNS.

Patient concerns:
The first case involved a 60-year-old woman with a pump pocket infection in a left ventricular assist device. DAP non-susceptible Staphylococcus capitis subsp. ureolyticus was isolated from a blood culture after treatment using vancomycin (10 days) and DAP (6 days). The second case involved a 71-year-old man with an aortic graft infection. DAP non-susceptible S capitis subsp. ureolyticus was detected in pus after treatment using vancomycin (2 weeks) and DAP (1 week) without complete removal and debridement.

Diagnosis:
Cardiovascular device infections caused by DAP non-susceptible CoNS.

Interventions and outcomes:
Whole genome sequencing of these strains revealed multiple mutations in genes that are related to DAP-non-susceptibility in S aureus, which created amino acid substitutions in mprF, dltAB, dltD, rpoC, yycG, cls2, pgsA, and vraSR. To the very best of our knowledge, the substitution patterns were not identical to those previously reported in DAP non-susceptibile S aureus.

Lessons:
Clinicians should be cautious regarding the emergence of DAP non-susceptible CoNS, especially in cases with implanted prosthetic devices, inadequate debridement, and prior usage of vancomycin and DAP. Further studies are needed to understand the relevance of these genetic changes and DAP-non-susceptibility in CoNS strains.
Association between Helicobacter pylori infection and Sjögren syndrome
Authors: Chen Qianqian , Zhou Xiaoying , Tan Wenfeng , Zhang Miaojia 
Published: December 2018

Abstract

Background:

Helicobacter pylori has been proved as a risk factor of many diseases. There are some researches trying to find connection between H. pylori and Sjögren syndrome (SS). However, the conclusions of these studies are controversial. We conducted this meta-analysis to evaluate the association between H. pylori and SS.

Methods:
We searched PubMed and Embase databases for researches which include the data of H. pylori infection rate in SS and control groups. A fixed-effects model was used to analyze the risk odds ratio (OR) with 95% confidence intervals (CIs) according to the heterogeneity across the selected studies.

Results:
Nine studies with 1958 participants including 619 patients with SS met the inclusion criteria. The total infection rate of H. pylori was 53.83% (1054/1958). We found that the patients with SS had a significantly higher H. pylori infection rate than control groups (OR = 1.19, 95% CI: 1.01–1.41, P = .033). Subgroup analysis demonstrated a significantly higher H. pylori infection rate in patients with primary SS than controls (OR = 1.24, 95% CI: 1.03–1.50, P = .026).

Conclusion:
This meta-analysis is the 1st meta-analysis about the association between H. pylori and SS. The pooled data suggested a significantly higher H. pylori infection rate in patients with SS. More prospective or multicenter retrospective researches could be conducted in the future.
Factors associated with preschool workers’ willingness to continue working
Authors: Tayama Jun , Yoshida Yuri , Iwanaga Ryoichiro , Tokunaga Akiko , Tanaka Goro , Imamura Akira , Shimazu Akihito , Shirabe Susumu 
Published: December 2018

Abstract
In industrialized countries, the turnover rate of preschool workers is extremely high and now represents a social problem. Consequently, it has become important to promote stable early care and educational environments for this population. Several factors related to working environments are known to affect turnover intention; however, the specific factors related to turnover intention among preschool workers have not yet been identified. Considering this, the objective of the present study was to determine factors associated with preschool workers’ willingness to continue working.
The participants of this study were 1137 preschool workers based in Nagasaki Prefecture, Japan. Multivariate logistic regression analysis was used to analyze the participants’ data, with willingness to continue working for 5 years or more set as the dependent variable.
Analysis of the results for all subjects clearly showed that male sex, older age, good mental health, high number of social supports, and good work engagement have a positive effect on willingness to continue working. Further, stratifying the participants in terms of age revealed that for preschool workers under 39 years, male sex, good mental health, high number of social supports, and good work engagement act positively in regard to willingness to continue working. Meanwhile, for those over 40 years, it was found that permanent employment and good work engagement act positively in this regard. Thus, work engagement was the only common factor between preschool workers under 39 and over 40.
The findings of this cross-sectional study demonstrate that the factors associated with willingness to continue working among preschool workers differ between younger and older professionals. These findings underline the importance of considering age categories when attempting to maximize such professionals’ willingness to continue working.
Nocardial spinal epidural abscess with lumbar disc herniation
Authors: Ma Fei , Kang Min , Liao Ye Hui , Lee Guang Zhou , Tang Qiang , Tang Chao , Ding Yin Huan , Zhong De Jun 
Published: December 2018

Abstract

Rationale
Nocardial spinal epidural abscess is rare. The diagnosis is often difficult to make and, if delayed, poses a high risk of long-term disability. Nocardial spinal epidural abscess with severe lumbar disc herniation has not previously been reported.

Patient concerns
A 50-year-old man presented with progressive lumbago and leg pain for 6 weeks after receiving acupuncture therapy, and then the patient suddenly occurred urine retention after walking.

Diagnoses
Clinical examination revealed sign of cauda equina syndrome. Magnetic resonance imaging (MRI) revealed a Lumbar(L)4 to L5 disc herniation, L3 to Sacrum(S)1 epidural abscess, and L2 to S1 paravertebral abscess. The causative organism was Nocardia farcinica.

Interventions
An urgent paravertebral abscess debridement and right L4 to L5 laminectomy were performed. Simultaneously, the disc tissue protruding into the spinal canal was removed, as well as irrigation and drainage. And antimicrobial treatment was continued for 12 months.

Outcomes
Fortunately, the patient was able to walk with a cane and urinate autonomously without a catheter, although this remained difficult 7 days after surgery. After 1 year of treatment, the patient has recovered completely and returned to work.

Lessons
Nocardial spinal epidural abscess with severe lumbar disc herniation is extremely rare. Pain from spinal degenerative diseases often masks the early symptoms of spinal infection. It's worth noting that invasive treatment of spine is a way of causing spinal nocardial infection.