bluuebunny/crossref_metadata_2025 huggingface dataset:
Diagnostic test accuracy of an automated device as a screening tool for fall risk assessment in community-residing elderly
Authors: Castellini Greta , Gianola Silvia , Stucovitz Elena , Tramacere Irene , Banfi Giuseppe , Moja Lorenzo
Published: September 2019

We aimed to determine the accuracy and failure of OAK device, an automated screening, for the assessment of fall risk in a prospective cohort of healthy adults aged over 65 years. The algorithm for fall risk assessment of the centers for disease control and prevention (CDC) was used as reference standard. Of the 183 individuals recruited, the CDC algorithm classified 80 as being at moderate/high risk and 103 at low risk of falling. OAK device failure incidence was 4.9% (confidence interval [CI] upper limit 7.7%), below the preset threshold for futility-early termination of the study (i.e., not above 15%). The OAK device showed a sensitivity of 84% and a specificity of 67% (receiver operating characteristic [ROC] area 82%; 95% confidence interval [CI] 76–88%), not reaching the preplanned target sensitivity (not lower than 85%). Diagnostic accuracy was not far from the sensitivity levels similar to those obtained with other fall risk assessment. However, some limitations can be considered.

ClinicalTrials.gov identifier: NCT02655796.
Therapeutic efficacy of zolpidem combined with cognitive-behavioral therapy on primary insomnia
Authors: Song Ying , Liang Bing
Published: September 2019

Background:

In this study, we intend to assess the efficacy of zolpidem combined with cognitive-behavioral therapy (CBT) for patients with primary insomnia (PI).

Methods:

A predefined search strategy will be used to search for associated literature from inception to the July 1, 2019: PubMed, EMBASE, Cochrane Library, Scopus, Web of Science, Google Scholar, Chinese Biomedical Literature Database, and China National Knowledge Infrastructure with no language limitation. In addition, we will also retrieve reference lists of included studies and relevant reviews, as well as the conference proceedings. All randomized controlled trials related to the zolpidem and CBT for PI will be included. Two authors will perform study selection, data collection, and study quality, respectively. We will also apply RevMan 5.3 software for statistical analysis.

Results:

This study will provide a comprehensive overview of the available evidence of the benefits and safety of zolpidem and CBT for PI. Primary outcomes are sleep quality and severity of sleep disorders. Secondary outcomes consist of sleep-onset latency, total sleep duration, sleep efficiency, and frequency and adverse events.

Conclusion:

The results of this study will inform clinical and policy decisions regarding the benefits and harm of zolpidem and CBT for patients with PI.

PROSPERO registration number:

PROSPERO CRD42019142796.
Primary repair for injury of medial collateral ligament during total-knee arthroplasty
Authors: Jin Cheng , Zhao Jia-Yi , Santoso Asep , Song Eun-Kyoo , Chan Chee-Ken , Jin Quan-He , Ko Jee-Wook , Seon Jong-Keun
Published: September 2019

The aim of this study was to determine whether primary repair for intraoperative injury of the medial collateral ligament (MCL) can achieve satisfactory clinical results when compared to the clinical results of patients with no MCL injury. Simultaneously, we sought to determine the differences between 2 methods of primary repair (anchor suture and staple) in terms of their clinical outcomes.

In our institute, 3897 total-knee arthroplasties (TKAs) were performed between 2003 and 2014. Sixty-five patients who suffered an MCL injury during the TKA procedure and in whom the injury was repaired with a suture anchor or staple (suture anchor: 36 vs staple: 29) were studied. A matched group of 65 patients without an MCL injury was selected to serve as the control group. Subjective feelings of instability and functional outcomes were assessed using the knee society (KS) score and the Western Ontario & McMaster Universities Osteoarthritis Index (WOMAC). Objective stability was evaluated by the measurement of opening angles in extension and at 30° of knee flexion on valgus stress radiographs. The clinical outcomes and stability results were compared between the suture anchor and staple methods.

The KS and WOMAC scores in patients who received primary repair of MCL injury during TKA improved from 50.6 ± 13.1 to 87.3 ± 7.3 (P < .001) and 65.9 ± 14.4 to 17.7 ± 6.6 (P < .001), respectively. However, there were no statistically significant differences in the KS (P = .84) and WOMAC (P = .71) scores when comparing the group that received primary repair to the control group. Radiographic stability also showed no differences between the repair and control groups in extension and at 30° of flexion (P = .48 and P = .11, respectively). In the subgroups, there were no significant differences between the suture anchor and staple repair methods in terms of stability and clinical outcomes.

Primary repair of an MCL injury during TKA may have clinical outcomes comparable to that in the no MCL injury group. Both staple and suture anchor repair methods could provide excellent clinical and stability outcomes in these types of cases, although a further cohort study is required to validate our results.
Management of giant benign fibrous histiocytoma in the spinal region with pleural involvement
Authors: Liu Shuzhong , Zhou Xi , Song An , Huo Zhen , Yao Siyuan , Wang Yipeng , Liu Yong
Published: September 2019

Rationale:

Benign fibrous histiocytoma with pleural involvement in spinal region is a highly unusual disease with no standard curative managements yet. The objective of this study is to report an extremely rare case of a giant benign fibrous histiocytoma with pleural involvement in spinal region successfully operated by posterior spinal surgery. The management of these unique cases has yet to be well-documented.

Patient concerns:

A 23-year-old man presented with a 2-month history of continuous and progressive back pain. A giant, expanding lesion of the T7 vertebral and paraspinal region with pleural involvement was identified.

Diagnoses:

Computed tomography scan and magnetic resonance imaging of spine showed expanding lesion of the T7 vertebral and paraspinal region involving the right thoracic cavity, which presented as a solid tumor. Postoperative pathology confirmed the diagnosis of thoracic benign fibrous histiocytoma.

Interventions:

The patient underwent thoracic spinal canal decompression, complete tumor resection, pleural defect repair, and T4 to T10 internal fixation procedure via a posterior approach.

Outcomes:

The patient's symptom improved significantly after the surgery, and the postoperative period was uneventful at the 2-year follow-up visit. There were no complications associated with the operation during the follow-up period.

Lessons:

In summary, the tumor's clinical features, imaging results, and pathological characteristics are unique. Combined efforts of specialists from orthopedics, thoracic surgery, neurosurgery, pathology, and medical oncology led to the successful diagnosis and management of this patient. Giant benign fibrous histiocytoma with pleural involvement in spinal region, although rare, should be part of the differential diagnosis when the patient presents with back pain and radiculopathy. We recommend the posterior or 1-stage anterior–posterior combined approach for complete resection of the giant thoracic benign fibrous histiocytoma when the tumor has caused severe symptoms or neurological deficits.
Renal and pulmonary thrombotic microangiopathy triggered by proteasome-inhibitor therapy in patient with smoldering myeloma
Authors: Cassol Clarissa A. , Williams Michael P.A. , Caza Tiffany N. , Rodriguez Sophia
Published: September 2019

Rationale:

Thrombotic microangiopathy (TMA) is a group of clinical syndromes characterized by excessive platelet activation and endothelial injury that leads to acute or chronic microvascular obliteration by intimal mucoid and fibrous thickening, with or without associated thrombi. It frequently involves the kidney but may involve any organ or system at variable frequencies depending on the underlying etiology. Among its numerous causes, drug toxicities and complement regulation abnormalities stand out as some of the most common. A more recently described association is with monoclonal gammopathy. Lung involvement by TMA is infrequent, but has been described in Cobalamin C deficiency and post stem-cell transplantation TMA.

Patient concerns:

This is the case of a patient with smoldering myeloma who received proteasome-inhibitor therapy due to retinopathy and developed acute renal failure within one week of therapy initiation.

Diagnoses:

A renal biopsy showed thrombotic microangiopathy. At the time, mild pulmonary hypertension was also noted and presumed to be idiopathic.

Interventions:

Given the known association of proteasome-inhibitor therapy with thrombotic microangiopathy, Bortezomib was discontinued and dialysis was initiated.

Outcomes:

Drug withdrawal failed to prevent disease progression and development of end-stage renal disease, as well as severe pulmonary hypertension that eventually lead to the patient's death.

Lessons:

To our knowledge, this is the first reported case of pulmonary involvement by TMA associated with monoclonal gammopathy which appears to have been triggered by proteasome-inhibitor therapy. Clinicians should be aware of this possibility to allow for more prompt recognition of pulmonary hypertension as a potential manifestation of monoclonal gammopathy-associated TMA, especially in patients also receiving proteasome-inhibitors, so that treatment aiming to slow disease progression can be instituted.
Analysis of the effects of low-level laser therapy on muscle fatigue of the biceps brachii muscle of healthy individuals and spastic individuals
Authors: da Silva Bruno Procopio , Souza Gabriela Aparecida da Silveira , Filho Alexandre Alves do Nascimento , Pinto Ana Paula , Guimarães Carolina Lobo , Pereira Aline Priscila Campos , Neves Marcele Florêncio das , Martins Patrícia Sardinha Leonardo Lopes , Lima Fernanda Pupio Silva , Lopes-Martins Rodrigo Alvaro Brandão , Lima Mário Oliveira
Published: September 2019

Background:

Muscular fatigue is caused by biochemical alterations that modify the mechanics of muscle contraction, resulting in negative changes in the performance of the contraction. Several resources are studied to mitigate this situation among which we can cite low-level laser therapy (LLLT). The effects of LLLT are being studied in healthy subjects with fibromyalgia and who are athletes, and currently the studies are being performed in spastic muscles with poststroke individuals. The aim will be to evaluate the effects of LLLT on the fatigue of the biceps brachii muscle of healthy individuals and individuals with spastic hemiparesis.

Methods:

A cross-sectional, comparative, randomized, placebo, double-blind clinical trial will be divided into 2 phases: phase I shall consist of 30 healthy subjects and phase II of 30 poststroke individuals. The study will consist of 3 groups (control group, placebo group, and LLLT group), and all individuals will pass through all groups, following the randomization criteria. The protocol consists of the application of LLLT in the biceps brachii muscle on the dominant side in healthy individuals and in the hemiparetic side of poststroke individuals, and, subsequently, 3 maximal isometric voluntary contractions (MIVCs) will be performed for 50 seconds in the dynamometer, with an interval of 50 seconds between them. Pain intensity will be evaluated by means of the visual analog scale, and the myoelectric activity by means of surface electromyography associated with the evaluation of muscular strength by means of the dynamometer. The local temperature will be evaluated by infrared thermography and blood lactate concentration through the lactimeter, which will be measured at 4 different times, before the application of the laser (basal), and 3, 15, and 25 minutes after the MIVC.
Clinical outcomes of discordant exercise electrocardiographic and echocardiographic findings compared with concordant findings in patients with chest pain and no history of coronary artery disease
Authors: Hwang Hui-Jeong , Sohn Il Suk , Park Chang-Bum , Jin Eun-Sun , Cho Jin-Man , Kim Chong-Jin
Published: September 2019

The aim of this study was to evaluate comparative clinical outcomes of discordant electrocardiographic (ECG) and echocardiographic (Echo) findings compared with concordant findings during treadmill exercise echocardiography in patients with chest pain and no history of coronary artery disease (CAD).

A total of 1725 consecutive patients who underwent treadmill echocardiography with chest pain and no history of CAD were screened. The patients were classified into 4 groups: ECG–/Echo– (negative ECG and Echo), ECG+/Echo– (positive ECG and negative Echo), ECG–/Echo+, and ECG+/Echo+. Concomitant CAD was determined using coronary angiography or coronary computed tomography. Major adverse cardiac events (MACEs) were defined as a composite of coronary revascularization, acute myocardial infarction, and death.

MACEs were similar between ECG–/Echo– and ECG+/Echo– groups. Compared with ECG+/Echo– group, ECG–/Echo+ group had more MACEs (adjusted hazard ratio [HR] adjusted by clinical risk factors [95% confidence interval {CI}], 3.57 [1.75–7.29], P < .001). Compared with ECG+/Echo+ group, ECG–/Echo+ group had lower prevalence of concomitant CAD and fewer MACEs (HR, 0.49 [0.29–0.81], P = .006).

Positive exercise Echo alone during treadmill exercise echocardiography had worse clinical outcomes than positive ECG alone, and the latter had similar outcomes to both negative ECG and Echo. Positive exercise Echo alone also had better clinical outcomes than both positive ECG and Echo. Therefore, exercise Echo findings might be superior for predicting clinical outcomes compared with exercise ECG findings. Additional consideration of ECG findings on positive exercise Echo will also facilitate better prediction of clinical outcomes.
The impact of pre-existing shoulder diseases and traumatic injuries of the shoulder on adhesive capsulitis in adult population
Authors: Tzeng Chung-Yuh , Chiang Hsiu-Yin , Huang Chun-Che , Lin Wei-Szu , Hsiao Tzu-Hung , Lin Ching-Heng
Published: September 2019

Adhesive capsulitis (AC) is a common chronic disorder for adult patients; however, whether a history of pre-existing shoulder diseases may affect the development of AC is still not fully understood. We aimed to investigate the incidence and prevalence of AC and to assess the association of pre-existing shoulder diseases and traumatic injuries of the shoulder with the development of AC in adults.

This retrospective population-based cohort and nested case-control study used data from the National Health Institute Research Database of Taiwan. A total of 24,414 patients aged 20 years or older and with a diagnosis of AC were identified between 2000 and 2013. We calculated the incidence of AC for each year during the study period. In addition, these AC patients were matched with controls (n = 97,656) in a ratio of 1:4 based on age, gender, and index date. Univariate and multivariate logistic regression models were performed to identify variables associated with AC.

Females and patients aged 50 to 69 years had higher age-gender standardized incidence and prevalence of AC than their counterparts. Multivariate analyses showed that after adjusting for relevant covariates, pre-existing shoulder diseases of calcific tendinitis (odds ratio [OR] = 8.74, 95% confidence interval [CI] = 5.66–13.5), biceps tendinitis (OR = 7.93, 95% CI = 5.33–11.79), rotator cuff syndrome (OR = 6, 95% CI = 5.26–6.85), osteoarthritis (OR = 4.27, 95% CI = 3.44–5.3), and impingement syndrome (OR = 3.13, 95% CI = 2.64–3.71), as well as fracture (OR = 4.51, 95% CI = 3.82–5.34) and dislocation (OR = 3.57, 95% CI = 2.35–5.45) of the shoulder were significantly associated with AC risk.

Higher odds of AC were observed among patients with pre-existing shoulder conditions. This study highlights the need to consider differences in AC risk among patients with various types of shoulder diseases and traumatic injuries of the shoulder.
Effectiveness of Fuyuan Xingnao Decoction for patients with diabetes mellitus combined cerebral infarction
Authors: Jiang Chao , Wang Ting , Ma Zhen , Fang Bang-jiang
Published: September 2019

Background:

Previous study has reported that Fuyuan Xingnao Decoction (FYXND) can be utilized for the treatment of patients with diabetes mellitus (DM) combined cerebral infarction (CI) effectively.

Methods:

We will search from the following databases of MEDLINE, EMBASE, Cochrane Library, PsycINFO, Global Health, Web of Science, Allied and Complementary Medicine Database, Chinese Biomedical Literature Database, and China National Knowledge Infrastructure. All databases will be searched from the inception to the present without language limitation. Two independent authors will perform literature selection, information collection, and methodological quality assessment. Statistical analysis will be carried out using RevMan 5.3 software.

Results:

This study will provide accurate results on the effectiveness and safety of FYXND on DM and CI through primary and secondary outcomes. The primary outcome is neurological deficit. The secondary outcomes consist of fasting blood glucose, hemoglobin Alc, fasting insulin, quality of life, and adverse effects.

Conclusions:

This well-designed study will establish high quality evidence of the effectiveness and safety of FYXND for DM and CI to facilitate the clinical practice and guideline development.
Mechanisms of action of molecules with anti-TNF-alpha activity on intestinal barrier inflammation
Authors: Lima Mayara Santa Rosa , Lima Vanessa Cristina Oliveira de , Piuvezam Grasiela , Azevedo Kesley Pablo Morais de , Maciel Bruna Leal Lima , Morais Ana Heloneida de Araújo
Published: September 2019

Background:

Tumor necrosis factor-alpha (TNF-alpha), among cytokines that mediate the inflammatory process, plays an important role in diseases involving the loss of intestinal barrier integrity. Several molecules with anti-TNF-alpha activity have been studied aiming to develop new therapies. The purpose of this paper is to describe the systematic review protocol of experimental studies that determine mechanisms of action of molecules with anti-TNF-alpha activity on intestinal barrier inflammation.

Methods:

This protocol is guided by the Preferred Reporting Items for Systematic Reviews and Meta-Analyzes Protocols (PRISMA-P). The databases to be searched are PubMed, EMBASE, Scopus, ScienceDirect, and Web of Science. Experimental studies in rats or mice that assessed the activity of anti-TNF-alpha molecules in models of intestinal barrier inflammation will be included in the systematic review. Studies characteristics, experimental model, and main results will be described and the bias risk assessment will be performed. Two independent reviewers will perform study selection, data extraction, and methodological quality assessment. A narrative synthesis will be made for the included studies. Also, if sufficient data is available, a meta-analysis will be conducted.I2statistics will be used to assess heterogeneity.

Results:

The present protocol will assist in producing a systematic review that identifies the mechanisms underlying the reduction of TNF-alpha in intestinal barrier inflammation models.

Conclusion:

The systematic review may contribute to the theoretical basis of research on new molecules with anti-TNF-alpha potential and, consequently, in the development of new therapies employed in humans.

PROSPERO registration number:

CRD42019131862.
Latent profile analysis of walking, sitting, grip strength, and perceived body shape and their association with mental health in older Korean adults with hypertension
Authors: Park Saengryeol , Castaneda-Gameros Diana , Oh In-Hwan
Published: September 2019

Evidence suggests that mental health is associated with multiple factors such as physical activity, sedentary behavior, and physical function in older adults. The present study used latent profile analysis to identify classes of older adults based on their health profile among a representative sample of Korean older adults with hypertension. Differences in mental health between these classes were also examined.

Seven hundred and sixty seven participants (mean age = 70.23, SD = 6.08; men 45.6%) were included in the analysis.

There were 3 latent classes (class 1: a physically inactive lifestyle with low physical function and body perception; class 2: a physically moderate lifestyle with moderate physical function and low body perception; class 3: a physically active lifestyle with high physical function and body perception). According to class comparisons, older adults in class 3 had significantly lower anxiety/depression levels than classes 1 and 2. Older adults in class 3 had significantly lower stress levels than class 1.

It is possible that among older adults, having a positive attitude of one's body shape may also be important for improving anxiety/depression along with having a physically active lifestyle and maintaining physical function.
Acupotomy for knee osteoarthritis
Authors: Xu Qinguang , Qiu Hanjun , Zhu Zhixiong , Wang Yueyi , Yang Shirong , Si Zhuyi , Shen Dong , Wang Xuezong , Di Yi , Lu Chaojie , Kang Xu , Wang Xiang
Published: September 2019

Background:

Knee osteoarthritis (KOA) is the most common form of arthritis, leading to pain disability in seniors and increased health care utilization. Acupotomy has been widely used to treat KOA. But its efficiency has not been scientifically and methodically evaluated. The aim of this study is to evaluate the efficacy and safety of acupotomy for the treatment of patients with KOA.

Methods:

Relevant studies will be searched from the databases of PubMed, EMBASE, Cochrane Library, China Knowledge Resource Integrated Database, Weipu Database for Chinese Technical Periodicals, SinoMed, and Wanfang Database from their inception to June 10, 2019. Two researchers will independently select studies, collect data, and assess the methodology quality by the Cochrane risk of bias tool.

Results:

The systematic review will provide high-quality evidence to assess the efficacy and safety of acupotomy for KOA by pain, stiffness, and dysfunction of knee joint, and quality of life, as well as adverse events.

Conclusion:

The systematic review will provide evidence to assess the effectiveness and safety of acupotomy therapy for KOA patients.

PROSPERO registration number:

PROSPERO CRD42019132082
Chronic hepatitis C virus infection
Authors: Li Xu , Wang Le , Gao Pujun
Published: September 2019

We investigated associations between inflammatory marker levels and hepatitis C virus (HCV)-related compensated liver cirrhosis risk in patients with chronic hepatitis C (CHC) infection in China. We used a case-control design and data from the records of 110 Chinese patients with CHC and cirrhosis for the study; 458 CHC patients who did not have a diagnosis of cirrhosis were matched to the case group by age and sex characteristics. We also investigated fatty liver disease risk factors. The group of patients with CHC infection and cirrhosis had lower platelet-to-lymphocyte ratio (PLR) values (60.63 [44.09, 89.31]) compared with the control group patients (80.24 [57.85, 111.08]). The results indicated that the group of patients with cirrhosis had higher 4-factor fibrosis index and aspartate aminotransferase (AST)-to-platelet ratio index (APRI) values compared with the group of patients with CHC-only (1.66 [0.98, 2.60] vs 0.71 [0.45, 1.17], respectively; P < .001 and 2.12 [0.97, 4.25] vs 0.99 [0.51, 2.01], respectively; P < .001). Compared with the control group, the AST/alanine aminotransferase ratio (AAR) values in the group of patients with cirrhosis were significantly higher (P < .001). Logistic regression analysis that included model adjustment for demographic characteristics and other factors that could affect cirrhosis risk revealed that greater 1/PLR values were associated with an increased odds of having cirrhosis (adjusted odds ratio [AOR], 95% confidence interval [CI] 0.991 [0.985–0.996]); APRI and AAR values were also independent predictors of the presence of compensated cirrhosis. We found that compared with the patients with CHC-only, the triglyceride, cholesterol, and low-density lipoprotein cholesterol levels in the patients with both CHC and fatty liver disease were significantly higher. The multivariate analysis of the risk of fatty liver development in patients with CHC infection found that cholesterol level was a statistically significant risk factor (AOR [95% CI] 1.380 [1.089–1.750], P = .008). Increased 1/PLR, APRI, and AAR values were associated with increased risks for development of cirrhosis in this population of Chinese patients with CHC infection. Higher cholesterol levels increased the risk of development of fatty liver disease in patients with CHC.
The impact of medical insurance on medical expenses for older Chinese
Authors: Zeng Yanbing , Luo Jiecheng , Ou Long , Yuan Manqiong , Zhou Zi , Han Yaofeng , Fang Ya
Published: September 2019

With the deepening population aging process in China, the medical expenses of older adults has become a widespread concerned. Medical insurance is a major source of Chinese medical financing and payment. The study aims to understand the current status of medical expenses for older adults and explore the effect of different types of health insurance on medical expenses in China.

The data came from the Chinese Longitudinal Health Longevity Survey (CLHLS) in 2014. The Kruskal–Wallis test and general multivariate linear regression model were applied to analyze the current situation and to explore how medical insurance as the main payment impacts medical expenses.

A total of 4376 older participants were included in this study. The median of medical expenses of a total was 1500 Yuan per year. The proportions of participants who had the urban employee-based basic medical insurance (UE-BMI), the urban residents basic medical insurance (UR-BMI), the new rural cooperative medical insurance scheme (NCMS), and the commercial medical insurance were 10.8%, 8.4%, 72.7%, and 0.9%, respectively. 34.8% of older adults paid the health care service via the NCMS and 11.9% paid via the UE-BMI. Participating in the NCMS and UR-BMI are significantly related to the level of the medical fees of older adults. UE-BMI, UR-BMI, and NCMS as main payment eased the pressure of medical expenses.

The influence of different types of medical insurances as main payments on the medical expenses of older adults is varied. Implementation of medical insurance should be taken to further relieve the medical expenses of older adults.
Evolution of tyrosinemia type 1 disease in patients treated with nitisinone in Spain
Authors: Couce María Luz , Sánchez-Pintos Paula , Aldámiz-Echevarría Luís , Vitoria Isidro , Navas Victor , Martín-Hernández Elena , García-Volpe Camila , Pintos Guillem , Peña-Quintana Luis , Hernández Tomás , Gil David , Sánchez-Valverde Félix , Bueno María , Roca Iria , López-Ruzafa Encarna , Díaz-Fernández Carmen
Published: September 2019

Treatment with nitisinone (NTBC) has brought about a drastic improvement in the treatment and prognosis of hereditary tyrosinemia type I (HT1). We conducted a retrospective observational multicentric study in Spanish HT1 patients treated with NTBC to assess clinical and biochemical long-term evolution.

We evaluated 52 patients, 7 adults and 45 children, treated with NTBC considering: age at diagnosis, diagnosis by clinical symptoms, or by newborn screening (NBS); phenotype (acute/subacute/chronic), mutational analysis; symptoms at diagnosis and clinical course; biochemical markers; doses of NTBC; treatment adherence; anthropometric evolution; and neurocognitive outcome.

The average follow-up period was 6.1 ± 4.9 and 10.6 ± 5.4 years in patients with early and late diagnosis respectively. All patients received NTBC from diagnosis with an average dose of 0.82 mg/kg/d. All NBS-patients (n = 8) were asymptomatic at diagnosis except 1 case with acute liver failure, and all remain free of liver and renal disease in follow-up. Liver and renal affectation was markedly more frequent at diagnosis in patients with late diagnosis (P < .001 and .03, respectively), with ulterior positive hepatic and renal course in 86.4% and 93.2% of no-NBS patients, although 1 patient with good metabolic control developed hepatocarcinoma.

Despite a satisfactory global nutritional evolution, 46.1% of patients showed overweight/obesity. Interestingly lower body mass index was observed in patients with good dietary adherence (20.40 ± 4.43 vs 24.30 ± 6.10; P = .08) and those with good pharmacological adherence (21.19 ± 4.68 vs 28.58 ± 213.79).

intellectual quotient was ≥85 in all NBS- and 68.75% of late diagnosis cases evaluated, 15% of which need pedagogical support, and 6.8% (3/44) showed school failure.

Among the 12 variants identified in fumarylacetoacetate hydrolase gene, 1 of them novel (H63D), the most prevalent in Spanish population is c.554–1 G>T.

After NTBC treatment a reduction in tyrosine and alpha-fetoprotein levels was observed in all the study groups, significant for alpha-fetoprotein in no NBS-group (P = .03), especially in subacute/chronic forms (P = .018).

This series confirms that NTBC treatment had clearly improved the prognosis and quality of life of HT1 patients, but it also shows frequent cognitive dysfunctions and learning difficulties in medium-term follow-up, and, in a novel way, a high percentage of overweight/obesity.
Cognitive and psychological outcomes of pediatric allogeneic hematopoietic stem cell transplantation survivors in a single center in China
Authors: Luo Yanhui , Yang Peiyi , Yang Yuting , He Peiling , Qin Maoquan , Wang Bin , Zhu Guanghua , Jia Chenguang , Yan Yan , Zhou Yuchen , Wang Ruixin , Wang Aihua , Zhou Xuan , Peng Xu
Published: September 2019

To investigate the cognitive and psychological outcomes of pediatric allogeneic HSCT survivors in China.

A total of 135 3 to 18 years old children and adolescents who underwent allo-HSCT and survived at least 3 months post-HSCT were recruited and completed the assessments. Cognitive and psychological functions were assessed via age-appropriate standardized measures. Clinical information was extracted from the medical records.

Forty one 3 to 6 years old children completed Psychological Questionnaires for 3 to 6 years Children. The scores of 21(51.2%) children in cognitive development dimension, 18(43.9%) in motor development dimension, 16(39.0%) in language development and social development dimension, 15(36.6%) in emotion and will dimension and 14(34.1%) in living habits dimension were less than the standard. Fifty six 8 to 16 years old children and adolescents completed the Depression Self-rating Scale for Children and 9 (16.1%) of these met the criteria of depression. Sixty nine 7 to 16 years old children and adolescents completed the screening for Child Anxiety Related Disorders and 7 (10.1%) of these met the criteria of anxiety, especially social phobia and school phobia. Eighty nine 6 to 18 years old children and adolescents completed the Symptom Checklist-90 and 43.8% to 77.5% of these experienced mild symptoms like obsession-compulsion (77.5%), hostility (64%), and interpersonal sensitivity (60.7%). Children treated with total body irradiation (TBI) showed more cognitive impairments like motor deficits than those without TBI. Also older children and adolescents had more symptoms like psychoticism.

These findings demonstrated cognitive and psychological late effects of pediatric allo-HSCT survivors in a single center in China and highlighted that the survivors conditioned with TBI had more cognitive impairments and older children and adolescents had more symptoms. Early intervention in these children and adolescents might minimize the cognitive losses and psychological effects.
Immune infiltration in nasopharyngeal carcinoma based on gene expression
Authors: Luo Meng-Si , Huang Guan-Jiang , Liu Bao-Xinzi
Published: September 2019

Immune infiltration of nasopharyngeal carcinoma (NPC) is closely associated with the patients’ prognosis. However, previous studies have not interpreted the difference of infiltrating immune cells in NPC.

We comprehensively analyzed the tumor-infiltrating immune cells present in NPC for the first time, which was based on a scientific deconvolution algorithm (CIBERSORT) and the gene expression data of GSE64634. The fractions of 22 immune cells were assessed to reveal the associations between normal samples and NPC samples.

Profiles of immune infiltration vary significantly between normal samples and NPC samples, and the variation could characterize the individual differences. NPC samples contained a higher proportion for M1 macrophages, whereas memory B cells and CD4 memory resting T cells were relatively lower.

Our data suggest that the differences in the infiltrating immune cells in NPC and these differences would probably facilitate patient consultation and individualized treatment.
Effectiveness of rehabilitation training combined acupuncture for the treatment of neurogenic bladder secondary to spinal cord injury
Authors: Yang Gui-fen , Sun Di , Wang Xin-hua , Chong Li , Luo Fang , Fang Cheng-bing
Published: September 2019

Background:

This study will aim to assess the effectiveness of the rehabilitation training (RT) combined acupuncture for the treatment of patients with neurogenic bladder (NB) secondary to the spinal cord injury (SCI).

Methods:

We will conduct a comprehensive literature search from the following databases from the inceptions to the present with no language limitation: PUBMED, EMBASE, Cochrane Library, SinoMed, Web of Science, Allied and Complementary Medicine Database, VIP, WANGFANG, Chinese Biomedical Literature Database, and China National Knowledge Infrastructure. Additionally, we will also search gray literature, including dissertations and conference proceedings. RevMan V.5.3 software will be used for the study selection, assessment of bias of bias, and data synthesis.

Results:

This study will synthesize the available evidence of RT combined with acupuncture for NB secondary to SCI, including episodes of urinary incontinence, urinary retention, urinary tract infection, bladder overactivity, quality of life, and adverse events.

Conclusion:

This study will determine whether RT combined acupuncture is an effective and safety therapy for NB secondary to SCI.

Systematic review registration:

PROSPERO CRD42019146127.
Tigecycline treatment in a liver transplant infant with carbapenem-resistant Escherichia coli infection
Authors: Yang Mei , Gao Hengmiao , Wang Xiaoling , Qian Suyun
Published: September 2019

Introduction:

During the past decade, the rate of carbapenem resistance among Enterobacteriaceae, mostly in Escherichia coli and Klebsiella pneumoniae, has significantly increased worldwide. It is a great challenge for the choice of drug treatment especially in children.

Tigecycline is the first drug in the glycylcycline class of antibiotics. For children, the China Food and Drug Administration and US Food and Drug Administration postulated that tigecycline is not recommended. It must be used only as salvage therapy for life-threatening infections in critically ill children who have no alternative treatment options.

Patient Concerns:

A male pediatric case of 4.5 months was blood stream infection after liver transplantation. The blood cultures obtained grew Gram-negative rods, which reportedly grew a strain of extended-spectrum β-lactamase and carbapenemases-producing Escherichia coli within 10 hours. All bacterial isolates were found to be resistant to all antimicrobial agents except aminoglycosides and tigecycline.

Diagnoses:

Complicated intra-abdominal infection, central line-associated blood stream infection.

Interventions:

The blood stream infection with carbapenem-resistant Escherichia coli after liver transplantation was cured by tigecycline.

Outcomes:

The patient's condition continued to improve, then transferred to general ward.

Conclusion:

The following report, to our knowledge, is the youngest liver transplantation patient who used tigecycline treatment around the world. It provides reference and experience for the use of tigecycline in infants with severe infections.
Case report of 49,XXXXY syndrome with cleft palate, diabetes, hypothyroidism, and cataracts
Authors: Wei Limin , Liu Yi , Sun Sufen , Tang Yong , Chen Shuchun , Song Guangyao
Published: September 2019

Rationale:

The karyotype 49,XXXXY is a rare form of Klinefelter syndrome usually presenting with ambiguous genitalia, facial dysmorphism, mental retardation, and a combination of cardiac, skeletal, and other malformations.

Patient concerns:

We describe a 19-year-old man, whose chromosomal analysis of peripheral blood revealed a karyotype of 49,XXXXY. His mental development and motor ability were significantly delayed. At the age of 19, he had failed to develop secondary sexual characteristics. His random blood glucose level was 19.61 mmol/L, and he showed dry mouth, polydipsia, and polyuria. He had a characteristic facial appearance with prognathism, widened nasal bridge, and strabismus. His bilateral elbow rotation was limited. He had atrophic testes with micropenis. Ophthalmic examination revealed a polar cataract in both eyes.

Diagnosis:

He was diagnosed with Klinefelter syndrome associated with cleft palate, hypothyroidism, cataracts, diabetes, and other anomalies.

Interventions:

After the initial diagnosis, the patient received intensive insulin therapy to correct hyperglycemia, and he received calcium and vitamin D supplements. The patient also received testosterone and thyroid hormone replacement therapy for primary hypogonadism.

Outcomes:

The patient was discharged 12 days after receiving treatment; meanwhile, there were no clinical symptoms of dry mouth, polyuria and polyuria, and his blood glucose level was controlled.

Lessons:

The combination of cleft palate, hypothyroidism, cataracts, diabetes, and osteoporosis in 49,XXXXY syndrome has not yet been reported. Early treatment and appropriate care can significantly improve the patient's quality of life and prevent serious consequences.
Laryngeal granuloma occurring after surgery for laryngeal cancer treated by surgical removal and immediate post-operative radiotherapy
Authors: Wu Jingyi , Jiang Tongchao , Wu Yu , Ding Lijuan , Dong Lihua
Published: September 2019

Rational:

Laryngeal granulomas are benign lesion that rarely occurs after surgery of laryngeal cancer. Until now there has not been standard treatment for it.

Patient concern:

The patient was diagnosed with laryngeal neoplasm one and half a month ago. Endoscopic low-temperature plasma knife in the radical excision of left vocal cord was performed under the general anesthesia. Postoperative histopathological examination confirmed left vocal cord tumor was highly differentiated invasive squamous cell carcinoma (SCC). Then the patient suffered unexplained intermittent dyspnea which persisted nearly 1 month after the surgery. Laryngoscope examination showed granulation formation on the glottis.

Diagnoses:

The patient was diagnosed with laryngeal granuloma 1 month after the surgery of laryngeal cancer.

Interventions:

The patient received resection of the laryngeal mass, and pathological examination confirmed the granuloma. Postoperative radiotherapy (RT) was performed within 24 hours after surgery.

Outcomes:

The patient was followed up for 3 years after surgery and the laryngeal granuloma and laryngeal cancer did not recur during follow-up. The symptoms of intermittent dyspnea disappeared and a satisfactory outcome was achieved.

Lessons:

Usually for primary laryngeal granulomas, surgical treatment alone is not enough, because it is easy to relapse. RT within 24 hours after operation can significantly reduce the recurrence of laryngeal granuloma.
Adjunctive granisetron therapy in patients with sepsis or septic shock (GRANTISS)
Authors: Guan Jianbin , Guo Yuexun , Chang Ping , Gan Jianwei , Zhou Jian , Wang Hua , Cen Zhongran , Tang Ying , Liu Zhanguo , Chen Peng
Published: September 2019

Introduction:

The incidence, mortality, and treatment costs of sepsis are high and, thus, present a major challenge for critical care medicine. Our previous studies suggest that intestinal metabolite granisetron has a potential therapeutic effect on sepsis. Granisetron is a clinically widely used antiemetic, which is safe, inexpensive, and reliable. However, its value in the treatment of sepsis remains unclear. This study aims to explore the efficacy and safety of granisetron in the treatment of sepsis.

Methods and analysis:

A single-center, single-blind, randomized, controlled clinical trial will be conducted on 154 patients with sepsis. Patients who meet sepsis 3.0 diagnostic criteria, aged ≥18 and ≤80 years, with PCT ≥ 2 ng/mL will be recruited. Patients will be randomized to receive intravenous granisetron 3 mg every 8 hours (n = 77) or an equal volume of normal saline (n = 77) for a treatment period of 4 days or to ICU discharge. The primary outcome is 28-day all-cause mortality. Secondary outcome measures include requirements for organ function support, changes of organ function, changes in infection biomarkers, changes in inflammatory and immune biomarkers, and the proportion of new organ failure. Adverse events and serious adverse events also will be observed closely.

Ethics and dissemination:

The study was approved by the Clinical Ethics Committee of Zhujiang Hospital of Southern Medical University (2018-ZZJHZX-009). The trial results will be disseminated at national and international conferences and through peer-reviewed journal.

Trial registration:

NCT03924518.URL: www.clinicaltrials.gov.

Protocol date:

1 May 2019. version 2.1.
Continuous femoral nerve block is more effective than continuous adductor canal block for treating pain after total knee arthroplasty
Authors: Borys Michał , Domagała Michał , Wencław Krzysztof , Jarczyńska-Domagała Joanna , Czuczwar Mirosław
Published: September 2019

Objectives:

Previous studies comparing adductor canal block (ACB) with femoral nerve block (FNB) are inconclusive with regard to patient-controlled analgesia (PCA) induced by opioids. Moreover, some postoperative pain severity results differ between previous randomized controlled trials (RCTs). The primary aim of the current study was to compare total intravenous morphine consumption administered via PCA during the first postoperative day in continuous FNB and ACB groups after total knee arthroplasty (TKA). Secondary aims included evaluation of postoperative pain via a visual analog scale, degree of knee extension, quadriceps muscle strength, and ability to sit, stand upright, and walk.

Methods:

The study was a RCT. Inclusion criteria were presence of gonarthrosis, age >18 and <75 years, and scheduled for TKA under single-shot spinal anesthesia.

Results:

A number of morphine uses was lower in the FNB group than in the ACB group (14, range 12–15 vs 20, range 18–22;P = .0001), and they perceived less severe pain at the 8th (P = .00003) and 24th hours. However, ACB was significantly superior with regard to most of the other parameters pertaining to mobility, including muscle strength at the 8th and 24th hours, degree of knee extension at the 8th hour, sitting at the 8th hour, standing upright at the 24th hour, and walking at the 24th and 48th hours.

Discussion:

FNB was associated with the perception of less severe pain after TKAs. However, ACB was associated with earlier mobility rehabilitation.
Dexamethasone-sparing regimen is an effective and safe alternative in overall antiemetic protection
Authors: Gu Yan-Lin , Xie Jia-ming , Ren Jie , Cao Hua , Wei Jin-rong , Chen Chao , Shao Le-Ning , Jiang Guo-Qin
Published: September 2019

Objective:

We performed a meta-analyisis to evaluate the efficacy of maintenance dexamethasone against acute or delayed chemotherapy-induced nausea and vomiting (CINV) in patients receiving moderately or highly emetic risk chemotherapy regimen.

Methods:

PubMed, Embase, and Cochrane Library were searched for eligible studies. Data comparing maintenance dexamethasone with single-dose dexamethasone during the acute, delayed, and overall phase of CINV were extracted. Overall risk ratio (RR) was used to estimate the efficacy and adverse effects.

Results:

Nine studies were included. In delayed phase, maintenance dexamethasone has similar efficacy to single-dose dexamethasone for no emetic episodes (RR, 1.06; 95% confidence interval [CI], 1.00–1.14), complete response (RR, 1.04; 95% CI, 0.98–1.11), complete control (RR, 1.07; 95% CI, 0.98–1.16), and total control (RR, 1.06; 95% CI, 0.91–1.23). In overall phase, maintenance dexamethasone has similar efficacy to single-dose dexamethasone for no emetic episodes (RR, 1.02; 95% CI, 0.94–1.11), complete response (RR, 1.02; 95% CI, 0.95 -1.09), complete control (RR, 1.03; 95% CI, 0.94–1.13), total control (RR, 1.05; 95% CI, 0.90–1.23), and no rescue medication (RR, 1.07; 95% CI, 0.97–1.19). Maintenance dexamethasone was only superior to single-dose dexamethasone for no rescue medication during delayed phase (RR, 1.10; 95% CI, 1.01–1.21, P = .034). The incidence of hiccup was observed higher in maintenance dexamethasone group (RR = 3.16, 95% CI, 1.12–8.92).

Conclusion:

The single-dose dexamethasone regimen offers high and similar overall control of symptoms as the maintenance dexamethasone regimen in this population. Multiple-day dexamethasone was suitable for patients who used rescue medication during the delayed phase.
Orbital abscess treated by ultrasound-guided fine needle aspiration and catheter drainage
Authors: Wang Yan , Zhang Jie , Dong Lei , Jiang Hong , Song Xicheng
Published: September 2019

Rationale:

Periorbital cellulitis or an orbital abscess caused by acute sinusitis is a serious acute infectious disease. If not treated in time, serious complications may occur.

Patient concerns:

A 16-year-old girl with a history of right-sided proptosis, periorbital swelling, chemosis, hypophasis, restricted ocular movement in the upward direction, and diminution of vision was referred to our institution. The clinic, computed tomography (CT) and magnetic resonance imaging (MRI) examination indicate right orbital abscess in the upper quadrant and sinusitis.

Diagnoses:

She was diagnosed with orbital abscess, acute sinusitis.

Interventions:

She underwent medical management, transnasal endoscopic surgery and then ultrasound-guided fine needle aspiration (FNA) and catheter drainage.

Outcomes:

She was completely cured without any complications or sequelae.

Lessons:

Performance of surgical drainage in a timely manner and administration of effective antibiotic treatment according to bacterial culture can reduce the complications of orbital abscesses. Ultrasound-guided FNA and catheter drainage is a safe, simple, and effective method for the treatment of orbital abscess.