bluuebunny/crossref_metadata_2025_split huggingface dataset:
Haematopoietic stem cell transplantation in paediatric rheumatic disease
Authors: Abinun Mario , Slatter Mary A. 
Published: July 2021

Purpose of review
A small proportion of children affected by rheumatic diseases suffer from severe, progressive disease, resistant to conventional antirheumatic therapies and to biologic agents interfering with inflammatory cytokines, costimulatory molecules expressed on immune system cells and intracellular signalling pathways. Adding to the poor prognosis is a high risk from significant morbidity and mortality associated with long-term treatment with multiple, often combined anti-inflammatory and immunosuppressive agents. Carefully selected patients from this unfortunate group may benefit from treatment with haematopoietic stem cell transplantation.

Recent findings
The majority of patients with severe paediatric rheumatic and autoinflammatory diseases treated with autologous and/or allogeneic haematopoietic stem cell transplantation achieved long-term remission. However, the incidence of disease relapse and transplant related morbidity and mortality is still significant.

Summary
Careful patient and donor selection, timing of the transplant earlier in the course of disease rather than the ‘last resort’ and choosing the most suitable conditioning regimen for each individual patient are the major factors favouring successful outcome. Close co-operation between the patients, their family, and involved medical teams is essential.
Prone-Positioning for Severe Acute Respiratory Distress Syndrome Requiring Extracorporeal Membrane Oxygenation
Authors: Petit Matthieu , Fetita Catalin , Gaudemer Augustin , Treluyer Ludovic , Lebreton Guillaume , Franchineau Guillaume , Hekimian Guillaume , Chommeloux Juliette , Pineton de Chambrun Marc , Brechot Nicolas , Luyt Charles-Edouard , Combes Alain , Schmidt Matthieu 
Published: July 2021

OBJECTIVES:
To determine the characteristics and outcomes of patients prone-positioned during extracorporeal membrane oxygenation for severe acute respiratory distress syndrome and lung CT pattern associated with improved respiratory system static compliance after that intervention.

DESIGN:
Retrospective, single-center study over 8 years.

Settings:
Twenty-six bed ICU in a tertiary center.

MEASUREMENTS AND MAIN RESULTS:
A propensity score–matched analysis compared patients with prone-positioning during extracorporeal membrane oxygenation and those without. An increase of the static compliance greater than or equal to 3 mL/cm H2O after 16 hours of prone-positioning defined prone-positioning responders. The primary outcome was the time to successful extracorporeal membrane oxygenation weaning within 90 days of postextracorporeal membrane oxygenation start, with death as a competing risk. Among 298 venovenous extracorporeal membrane oxygenation–treated adults with severe acute respiratory distress syndrome, 64 were prone-positioning extracorporeal membrane oxygenation. Although both propensity score–matched groups had similar extracorporeal membrane oxygenation durations, prone-positioning extracorporeal membrane oxygenation patients’ 90-day probability of being weaned-off extracorporeal membrane oxygenation and alive was higher (0.75 vs 0.54, p = 0.03; subdistribution hazard ratio [95% CI], 1.54 [1.05–2.58]) and 90-day mortality was lower (20% vs 42%, p < 0.01) than that for no prone-positioning extracorporeal membrane oxygenation patients. Extracorporeal membrane oxygenation–related complications were comparable for the two groups. Patients without improved static compliance had higher percentages of nonaerated or poorly aerated ventral and medial-ventral lung regions (p = 0.047).

CONCLUSIONS:
Prone-positioning during venovenous extracorporeal membrane oxygenation was safe and effective and was associated with a higher probability of surviving and being weaned-off extracorporeal membrane oxygenation at 90 days. Patients with greater normally aerated lung tissue in the ventral and medial-ventral regions on quantitative lung CT-scan performed before prone-positioning are more likely to improve their static compliance after that procedure during extracorporeal membrane oxygenation.
Prevalence and Clinical Impact of Reduced Coagulation Factor XII Activity in Patients Receiving Extracorporeal Membrane Oxygenation
Authors: Buchtele Nina , Schwameis Michael , Schellongowski Peter , Quehenberger Peter , Knöbl Paul , Traby Ludwig , Schmid Monika , Schoergenhofer Christian , Herkner Harald , Jilma Bernd , Staudinger Thomas 
Published: July 2021

OBJECTIVES:
Extracorporeal membrane oxygenation provides large surface exposure to human blood leading to coagulation activation. Only limited clinical data are available on contact activation and coagulation factor XII activity in extracorporeal membrane oxygenation patients.

DESIGN:
Prospective cohort study.

SETTING:
Three medical ICUs at the Medical University of Vienna.

PATIENTS:
Adult patients receiving venovenous or venoarterial extracorporeal membrane oxygenation.

INTERVENTIONS:
None.

MEASUREMENTS AND MAIN RESULTS:
The primary outcome was the change in coagulation factor XII activity in response to extracorporeal membrane oxygenation. Secondary outcomes included the prevalence of reduced coagulation factor XII activity (< 60%) among patients receiving extracorporeal membrane oxygenation and association of coagulation factor XII activity with thromboembolic and bleeding complications. An exploratory endpoint was the association of coagulation factor XII activity and activated partial thromboplastin time in heparinase-treated samples in vitro. Fifty-one patients with a total of 117 samples were included in the study between July 2018 and February 2020. Fifty patients (98%) had reduced coagulation factor XII activity at any timepoint during extracorporeal membrane oxygenation. Median coagulation factor XII activity during extracorporeal membrane oxygenation treatment was 30% (interquartile range, 21.5–41%) and increased after discontinuation (p = 0.047). Patients with thromboembolic complications had higher median coagulation factor XII activity during extracorporeal membrane oxygenation (34% vs 23%; p = 0.023). The odds of a thromboembolic event increased by 200% per tertile of median coagulation factor XII activity (crude odds ratio, 3.034; 95% CI, 1.21–7.63). No association with bleeding was observed. In heparinase-treated samples, coagulation factor XII activity correlated well with activated partial thromboplastin time (r = –0.789; p = 0.007).

CONCLUSIONS:
We observed a high prevalence of reduced coagulation factor XII activity in adult patients on extracorporeal membrane oxygenation, which may confound activated partial thromboplastin time measurements and limit its clinical usefulness for monitoring and titrating anticoagulation with unfractionated heparin. Lower coagulation factor XII activity was associated with less thromboembolic complications, which may highlight the potential of coagulation factor XII to serve as a target for anticoagulation in extracorporeal membrane oxygenation.
Non-IFNγ Whole Blood Cytokine Responses to Mycobacterium tuberculosis Antigens in HIV-exposed Infants
Authors: Anterasian Christine , Warr Alex J. , Lacourse Sylvia M. , Kinuthia John , Richardson Barbra A. , Nguyen Felicia K. , Matemo Daniel , Maleche-Obimbo Elizabeth , John Stewart Grace C. , Hawn Thomas R. 
Published: July 2021

Background:
HIV-exposed uninfected (HEU) infants have increased risk of tuberculosis (TB). Testing for Mycobacterium tuberculosis (Mtb) infection is limited by reduced Quantiferon (QFT) sensitivity in infants and tuberculin skin test (TST) cross-reactivity with Bacillus Calmette–Guérin vaccine. Our objective is to assess if non-IFNγ cytokine responses to Mtb-specific antigens have improved sensitivity in detecting Mtb infection in HEU infants compared with QFT.

Methods:
HEU infants were enrolled in a randomized clinical trial of isoniazid preventive therapy (IPT) to prevent Mtb infection in Kenya (N = 300) and assessed at 12 months postrandomization (14 months of age) by TST and QFT-Plus. Non-IFNγ cytokine secretion (IL2, TNF, IP10, N = 229) in QFT-Plus supernatants was measured using Luminex assay. Logistic regression was used to assess the effect of IPT on Mtb infection outcomes in HEU infants.

Results:
Three of 251 (1.2%) infants were QFT-Plus positive. Non-IFNγ Mtb antigen-specific responses were detected in 12 additional infants (12/229, 5.2%), all TST negative. IPT was not associated with Mtb infection defined as any Mtb antigen-specific cytokine response (odds ratio = 0.7, P = 0.54). Mtb antigen-specific IL2/IP10 responses had fair correlation (τ = 0.25). Otherwise, non-IFNγ cytokine responses had minimal correlation with QFT-Plus and no correlation with TST size.

Conclusions:
We detected non-IFNg Mtb antigen-specific T-cell responses in 14-month HEU infants. Non-IFNg cytokines may be more sensitive than IFNg in detecting infant Mtb infection. IPT during the first year of life was not associated with Mtb infection measured by IFNg, IL2, IP10 and TNF Mtb-specific responses.
Recent Experience: Corticosteroids as a First-line Therapy in Children With Multisystem Inflammatory Syndrome and COVID-19-related Myocardial Damage
Authors: Vukomanovic Vladislav , Krasic Stasa , Prijic Sergej , Ninic Sanja , Popovic Sasa , Petrovic Gordana , Ristic Snezana , Simic Radoje , Cerovic Ivana , Nesic Dejan 
Published: July 2021

Background:
Cardiovascular complications with myocarditis in multisystem inflammatory syndrome in children (MIS-C) associated with severe acute respiratory syndrome coronavirus 2 infection have been reported, but the optimal therapeutic strategy remains unknown.

Methods:
A retrospective cohort study included 19 patients with acute left ventricular systolic dysfunction associated with MIS-C, average years of age 13.2 ± 3.8, treated from April 2020 to April 2021.

Results:
Treatment failure (TF) was observed in 8 patients (in the intravenous immunoglobulin [IVIG] group 7/10; in the corticosteroid [CS] group 1/9). The independent risk factor for TF was IVIG treatment (odds ratio [OR] 18.6, 95% confidence interval [CI] 1.6–222.93, P = 0.02). Patients initially treated with CS became afebrile during in-hospital day 1 (1.5, interquartile range [IQR] 1–2), while IVIG-treated patients became afebrile on in-hospital day 4 (IQR 2–4.25), after CS was added. The C-reactive protein (CRP) significantly declined in CS-treated patients on day 2 (P = 0.01), while in the IVIG group, CRP decreased significantly on the fourth day (P = 0.04). Sodium and albumin levels were higher on third in-hospital day in the CS group than in the IVIG group (P = 0.015, P = 0.03). A significant improvement and normalization of ejection fraction (EF) during the first 3 days was observed only in the CS group (P = 0.005). ICU stays were shorter in the CS group (4, IQR 2–5.5) than in the IVIG group (IVIG group 7, IQR 6–8.5) (P = 0.002).

Conclusions:
Among children with MIS-C with cardiovascular involvement, treatment with CS was associated with faster normalization of LV EF, fever, laboratory analysis, and shorter ICU than IVIG-treated patients.
Cutaneous Manifestations of Sarcoidosis
Authors: Khawar Talha , Chatta Payush , Sandhu Sareen 
Published: July 2021

ABSTRACT
Sarcoidosis is a multisystem autoimmune disorder that is primarily characterized by its pulmonary manifestations. However, it is equally important to recognize the systemic nature of the disease including cutaneous manifestations, which are seen in a sizable proportion of patients with sarcoidosis. Dermatology nurses have a very important role to play in early recognition and diagnosis of this disease. This review article focuses on the multiple cutaneous manifestations of sarcoidosis, suggested clinical workup, and available treatment options. The aim of this review is to increase awareness of this disease among dermatology nurses to help facilitate early diagnosis and management.
Sunnybrook Facial Grading System: Intra-rater and Inter-rater Variabilities
Authors: Cabrol Camille , Elarouti Léa , Montava Anne-Laure , Jarze Sylvie , Mancini Julien , Lavieille Jean-Pierre , Barry Pauline , Montava Marion 
Published: July 2021

Objectives:
Evaluate intra-rater and inter-rater variabilities of the Sunnybrook Facial Grading System (SFGS) and explore potential factors of variability.

Study Design:
Prospective test of hypothesis.

Setting:
University tertiary referral center.

Participants/Methods:
Facial video recordings of 20 patients with variable degrees of peripheral facial palsy (PFP) were anonymized then presented to 31 independents raters in 2 trials. The raters were senior and junior professionals involved in the management of PFP: ENT specialists, physiotherapists, and speech therapists. The SFGS was used for grading paralysis.

Main Outcome Measure:
Intra-rater and inter-rater variabilities were estimated by intraclass correlation coefficient (ICC [95% confidence interval]) for the composite score and the three subscores of the SFGS. Factors of variability studied were: rater professions and rater experience (senior vs junior).

Results:
For the total population, the intra-rater ICC was 0.915[0.900–0.929] for the composite score considered to represent almost perfect repeatability. Repeatability was important for symmetry at rest (0.694[0.646–0.737]), almost perfect for voluntary movements (0.903[0.886–0.918]), and important to almost perfect for synkinesis (0.810[0.778–0.838]). The inter-rater ICC for the composite score was 0.847[0.755–0.923] indicating important to almost perfect agreement between all raters. Agreement between raters was important to almost perfect for voluntary movements (0.839[0.746–0.919]), but moderate to important for symmetry at rest (0.553[0.408–0.730]) and weak to important for synkinesis (0.476[0.333–0.666]). Some variability was found between raters groups; however, repeatability and agreement were good for all raters.

Conclusions:
The SFGS is a reproducible scale. It can be used with good reproducibility by both novices and experts, and by all professionals involved in the management of PFP.
Could Jinfukang alleviate the chemotherapy-related adverse effects in non-small cell lung cancer patients?
Authors: Zheng Xing , Wang Wenmin , Wang Gefei , Liu Shenghua 
Published: July 2021

Abstract

Background:
Lung cancer is the main cause of cancer-related death in the world, and non-small cell lung cancer (NSCLC) accounts for approximately 85% of all lung cancers. Cisplatin and its derivatives are the first-line chemotherapeutic drugs for patients with advanced lung cancer, but the chemotherapy-related adverse reactions greatly impact the quality of life (QOL) of patients and limit their use. Jinfukang is a commonly used traditional Chinese medicine preparation with anti-tumor effect in China, which has been approved by China Food and Drug Administration against NSCLC. At present, there is a lack of strict randomized controlled trials to study whether Jinfukang could alleviate the chemotherapy-related adverse effects in the treatment of advanced NSCLC. Therefore, we intend to perform a double-blind, placebo controlled, randomized trial to evaluate the effect of Jinfukang in alleviating the chemotherapy-related adverse effects of patients with advanced NSCLC.

Methods:
This is a prospective, double-blind, randomized, placebo controlled trial. According to the randomized control principle, 168 patients will be divided into treatment group and control group at 1:1 ratio. The patients in the two groups will be treated continuously for 3 cycles and followed up for 3 years. Outcome indicators include: the incidence of chemotherapy-related adverse effects, the progression-free survival (PFS), total effective rate, and QOL evaluation. We will use SPSS19.0 to analyze the results.

Conclusions:
This study will help to evaluate the effect of Jinfukang alleviating chemotherapy-related adverse effects in the treatment of advanced NSCLC.

Trial registration:
DOI 10.17605/OSF.IO/YWBSC
Acupuncture for the treatment of leptin resistance in obesity
Authors: Gao Tianjiao , Ma Dehui , Chen Shaotao , Zhang Xiaolin , Han Yiran , Liu Mingjun 
Published: July 2021

AbstractBackground:Recently, there has been a global increase in obesity and obesity-related diseases. The prevention and treatment of obesity have become one of the most significant public health challenges worldwide in the 21st century, and how to effectively curb the occurrence of obesity has become a major global concern. Numerous studies have shown that the majority of obese individuals do not respond to leptin, and instead demonstrate leptin resistance. Clinical studies have found that acupuncture is widely used in the clinical treatment of obesity in recent years, but whether it can improve leptin resistance has not been systematically reviewed. This study is aimed to investigate the effectiveness of acupuncture in obesity with leptin resistance (LR).Methods:We searched PubMed, Cochrane Library, Embase, China National Knowledge Infrastructure (CNKI), Wan Fang, Technology Journal Database (VIP), and China Biology Medicine disc (CBM). Chinese Clinical Trial Registry. The time was from the establishment of the database to March 19, 2021. RevMan 5.3 software was used to assess the quality and risk of the included studies.Results:This study will be conducted in terms of clinical efficacy, serum leptin content, and body weight change. The current evidence shows that the incidence of the disease is high and the comprehensive quality is high.Conclusion:The conclusion of this review will provide a basis for judging whether acupuncture therapy is effective in the treatment of leptin resistance in obesity.
Similar appearance of different multifocal carpal bone destructing disease entities in 3 patients
Authors: Lee Jun-Ku , Kwon Young Woo , Shim Jae Chan , Kang Yun Kyung , Cho Weon Min , Park Jong Woong , Han Soo-Hong 
Published: July 2021

Abstract

Rationale:
Several diseases feature tumors, or tumor-mimicking lesions, that further invade the bone and surrounding joints of the wrist region. Here, we describe 3 rare cases of multiple destructed carpal bones and adjacent joints in different disease entities confirmed via pathologic diagnosis.

Patient concerns:
All 3 cases were examined between January 2016 and December 2019. Three patients presented with similar clinical manifestations and radiographic features, with multiple osteolytic lesions in the carpal bones and metacarpal bone base.

Diagnoses:
The 3 cases were diagnosed as diffuse type tenosynovial giant cell tumor, calcifying aponeurotic fibroma, and rheumatoid arthritis.

Interventions:
Separate, experienced radiologist and pathologist took part in the interpretation and compartmentalization of radiographs and pathological findings, respectively. Even magnetic resonance imaging could not achieve a diagnosis; surgical excision was therefore required, with subsequent pathological assessment for treatment and final diagnosis.

Outcomes:
functional outcomes also differed among patients, poorest in rheumatoid arthritis patient.

lessons:
We report 3 rare disease entities, presenting with multifocal osteolytic lesions in the wrist. They all presented with similar clinical manifestations, and the final diagnoses were made via pathological evaluation. Compared with tenosynovial giant cell tumor and calcifying aponeurotic fibroma, rheumatoid arthritis had the poorest outcome.
The value of hemoglobin-to-red blood cell distribution width ratio (Hb/RDW), neutrophil-to-lymphocyte ratio (NLR), and platelet-to-lymphocyte ratio (PLR) for the diagnosis of nasopharyngeal cancer
Authors: Lin Zhongyuan , Zhang Xuan , Luo Yu , Chen Yanyun , Yuan Yulin 
Published: July 2021

Abstract
The clinical significance of hemoglobin-to-red blood cell distribution width (Hb/RDW) for the diagnosis of nasopharyngeal cancer (NPC) has not been reported yet. This study aimed to evaluate the value of preoperative Hb/RDW, neutrophil-to-lymphocyte ratio (NLR), and platelet-to-lymphocyte ratio (PLR) for the diagnosis of NPC.
A total of 180 NPC patients (NPC group) and 149 healthy subjects (control group) were recruited to assess the value of Hb/RDW, NLR, and PLR for the diagnosis of NPC.
It was noted that NLR and PLR were significantly higher in the NPC group than those in the control group (P < .001); however, Hb/RDW was lower in the NPC group compared with that in the control group (P < .001). NLR was also remarkably different between patients of stage I+II and those of stage III+IV (P = .043), and that was different in patients with lymph node metastases or not (P = .030). Besides, PLR was significantly different in patients with serosal invasion or not (P = .031). In receiver operating characteristic curve, compared with Hb/RDW alone (sensitivity, 66.67%; specificity, 85.23%), the sensitivity (67.78%, 72.78%) and specificity (89.62%, 90.6%) of Hb/RDW with NLR and PLR were both increased. Furthermore, Hb/RDW combined with NLR area under the ROC (AUC), 0.824; 95% confidence interval (CI): 0.779–0.864, P = .0080) or PLR (AUC: 0.851, 95% CI: 0.808–0.888, P = .0002) had a greater AUC value for the diagnosis of NPC compared with Hb/RDW alone (AUC: 0.781, 95% CI: 0.732–0.824).
Hb/RDW can be used as a valuable indicator for auxiliary diagnosis of NPC. Preoperative Hb/RDW combined with NLR or PLR is of great significance in the auxiliary diagnosis and pathological staging of NPC.
Effect of folic acid combined with pravastatin on arteriosclerosis in elderly hypertensive patients with lacunar infarction
Authors: Li Chunxia , Bu Xingpeng , Liu Yaru 
Published: July 2021

Abstract
This study aimed to assess the effect of folic acid combined with pravastatin on atherosclerosis-related indexes in elderly patients with hypertension complicated with lacunar cerebral infarction.
A total of 134 elderly hypertensive patients with lacunar cerebral infarction were randomly divided into 3 groups using the random number table method. Group A, the folic acid group, had 45 cases and received low-dose folic acid (0.8 mg/d) treatment on the basis of antihypertensive treatment. Group B, the pravastatin group, had 45 cases and received pravastatin (20 mg/d) treatment on the basis of antihypertensive treatment. Group C, the folic acid combined with the pravastatin group, had 44 cases. Members of this group received pravastatin (20 mg/d) and low-dose folic acid (0.8 mg/d) based on antihypertensive treatment. Levels of folic acid, homocysteine (Hcy), tumor necrosis factor alpha (TNF-a), matrix metallopeptidase 9 (MMP-9), cholesterol (TC), and low-density lipoprotein cholesterol (LDL-C) were measured by ELISA before treatment in all 3 groups. Carotid intima-media thickness (IMT) was measured using ultrasound, and systolic and diastolic blood pressure were measured with a mercury column. After 8 weeks of treatment, the levels of folic acid, Hcy, TNF-a, MMP-9, TC, LDL-C, and systolic and diastolic blood pressure were compared among the 3 groups. IMT levels were measured at 12 weeks of treatment.
After 8 weeks of treatment, compared with group B, patients in groups A and C had folic acid levels significantly higher than baseline levels, with significantly lower Hcy levels (both P < .05). Patients in group C presented significantly decreased TNF-a, MMP-9, TC, and LDL-C levels and systolic and diastolic blood pressure after 8 weeks of treatment, compared with those in groups A and B (both P < .05). These patients also showed significantly decreased IMT levels compared with those in the other groups (P < .05).
Low-dose folic acid combined with pravastatin in elderly patients with lacunar cerebral infarction can reduce the level of homocysteine, improve the degree of carotid atherosclerosis, protect vascular endothelium, and reduce blood lipids and blood pressure, presenting better benefits than pravastatin alone.