bluuebunny/crossref_metadata_2025_split huggingface dataset:
Hereditary hemorrhagic telangiectasia (HHT): a practical guide to management
Authors: Hammill Adrienne M. , Wusik Katie , Kasthuri Raj S. 
Published: December 2021

AbstractHereditary hemorrhagic telangiectasia (HHT), the second most common inherited bleeding disorder, is associated with the development of malformed blood vessels. Abnormal blood vessels may be small and cutaneous or mucosal (telangiectasia), with frequent complications of bleeding, or large and visceral (arteriovenous malformations [AVMs]), with additional risks that can lead to significant morbidity and even mortality. HHT can present in many different ways and can be difficult to recognize, particularly in younger patients in the absence of a known family history of disease or epistaxis, its most common manifestation. HHT is commonly diagnosed using the established Curaçao clinical criteria, which include (1) family history, (2) recurrent epistaxis, (3) telangiectasia, and (4) visceral AVMs. Fulfillment of 3 or more criteria provides a definite diagnosis of HHT, whereas 2 criteria constitute a possible diagnosis of HHT. However, these criteria are insufficient in children to rule out disease due to the age-dependent development of some of these criteria. Genetic testing, when positive, can provide definitive diagnosis of HHT in all age groups. Clinical course is often complicated by significant epistaxis and/or gastrointestinal bleeding, leading to anemia in half of adult patients with HHT. The management paradigm has recently shifted from surgical approaches to medical treatments aimed at control of chronic bleeding, such as antifibrinolytic and antiangiogenic agents, combined with aggressive iron replacement with intravenous iron. Guidelines for management of HHT, including screening and treatment, were determined by expert consensus and originally published in 2009 with updates and new guidelines in 2020.
Chronic thromboembolic pulmonary hypertension: anticoagulation and beyond
Authors: Martin Karlyn A. , Cuttica Michael J. 
Published: December 2021

Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication in pulmonary embolism (PE) survivors, characterized by chronic vascular occlusion and pulmonary hypertension. The identification and diagnosis of CTEPH requires a stepwise approach, starting with symptom evaluation, functional evaluation, screening imaging, and progressing to interventional hemodynamic assessment. On the backbone of anticoagulation, CTEPH management necessitates a multidisciplinary approach. Surgical pulmonary thromboendarterectomy (PTE) is the only potentially curative option. In nonoperable disease or residual disease after PTE, interventional balloon pulmonary angioplasty and/or pulmonary-vasodilator therapies can be offered, in collaboration with interventional and vascular pulmonary colleagues. As it is a disease that can cause high morbidity and mortality, CTEPH requires a high index of suspicion to diagnose and treat in patients following PE.
Infectious complications and vaccines
Authors: Ljungman Per 
Published: December 2021

Abstract
Infections are a major cause of morbidity and can result in mortality in long-term survivors after allogeneic hematopoietic cell transplantation. Chronic graft-versus-host disease and delayed immune reconstitution are recognized risk factors. Different strategies must be utilized depending on the individual patient's situation but include prolonged antimicrobial prophylaxis and vaccination. Some important infections due to pathogens preventable by vaccination are pneumococci, influenza, varicella-zoster virus, and SARS-CoV-2. Despite the fact that such recommendations have been in place for decades, implementation of these recommendations has been reported to be poor.
Advances in the management of α-thalassemia major: reasons to be optimistic
Authors: Horvei Paulina , MacKenzie Tippi , Kharbanda Sandhya 
Published: December 2021

Abstract
α-Thalassemia major (ATM) is a severe disease resulting from deletions in all 4 copies of the α-globin gene. Although it is usually fatal before birth, the advent of in utero transfusions has enabled survival of a growing number of children. Postnatal therapy consists of chronic transfusions or stem cell transplantation, similar to patients with β-thalassemia major. In this review, we discuss the experience with postnatal stem cell transplantation in patients with ATM, as well as the ongoing phase 1 clinical trial of in utero stem cell transplantation for this condition.
How to recognize and manage COVID-19-associated coagulopathy
Authors: Gerber Gloria F. , Chaturvedi Shruti 
Published: December 2021

AbstractCOVID-19 is frequently associated with abnormalities on coagulation testing and a coagulopathy driven by inflammation, intravascular coagulation activation, and microvascular thrombosis. Elevated D-dimer is the most common finding and is a predictor of adverse outcomes including thrombosis, critical illness, and death. Although COVID-19-associated coagulopathy has some similarities to disseminated intravascular coagulation, the platelet count is usually preserved, coagulation times are usually normal or minimally prolonged, and thrombosis is more common than bleeding, at least in noncritically ill patients. Bleeding is uncommon but may be a significant problem in critically ill patients, including those who may develop a consumptive coagulopathy with frank disseminated intravascular coagulation and those on extracorporeal membrane oxygenation. Blood product support to correct coagulopathy is reserved for bleeding patients or those requiring invasive procedures. Current recommendations suggest that all hospitalized patients should receive at least a prophylactic dose of anticoagulation. Results from a multiplatform randomized clinical trial suggest that therapeutically dosed anticoagulation may improve outcomes, including the need for organ support and mortality in moderately ill patients but not in those requiring critical care. The results of ongoing trials evaluating the impact of different antithrombotic strategies (therapeutic agents and intensity) on COVID-19 outcomes are eagerly awaited and are expected to have important implications for patient management. We also discuss COVID-19 vaccine-associated cytopenias and bleeding as well as vaccine-induced thrombotic thrombocytopenia, in which thrombosis is associated with thrombocytopenia, elevated D-dimer, and, frequently, hypofibrinogenemia.
COVID-19 and thrombosis: searching for evidence
Authors: Thilagar Bright , Beidoun Mohamad , Rhoades Ruben , Kaatz Scott 
Published: December 2021

Abstract
Early in the pandemic, COVID-19-related increases in rates of venous and arterial thromboembolism were seen. Many observational studies suggested a benefit of prophylactic anticoagulation for hospitalized patients using various dosing strategies. Randomized trials were initiated to compare the efficacy of these different options in acutely ill and critically ill inpatients as the concept of immune-mediated inflammatory microthrombosis emerged. We present a case-based review of how we approach thromboembolic prophylaxis in COVID-19 and briefly discuss the epidemiology, the pathophysiology, and the rare occurrence of vaccine-induced thrombotic thrombocytopenia.
Advances in MGUS diagnosis, risk stratification, and management: introducing myeloma-defining genomic events
Authors: Landgren Ola 
Published: December 2021

Abstract
In the 1960s, Dr Jan Waldenström argued that patients who had monoclonal proteins without any symptoms or evidence of end-organ damage represented a benign monoclonal gammopathy. In 1978, Dr Robert Kyle introduced the concept of “monoclonal gammopathy of undetermined significance” (MGUS) given that, at diagnosis, it was not possible with available methods (ie, serum protein electrophoresis to define the concentration of M-proteins and microscopy to determine the plasma cell percentage in bone marrow aspirates) to determine which patients would ultimately progress to multiple myeloma. The application of low-input whole-genome sequencing (WGS) technology has circumvented previous problems related to volume of clonal plasma cells and contamination by normal plasma cells and allowed for the interrogation of the WGS landscape of MGUS. As discussed in this chapter, the distribution of genetic events reveals striking differences and the existence of 2 biologically and clinically distinct entities of asymptomatic monoclonal gammopathies. Thus, we already have genomic tools to identify “myeloma-defining genomic events,” and consequently, it is reasonable to consider updating our preferred terminologies. When the clinical field is ready to move forward, we should be able to consolidate current terminologies—from current 7 clinical categories: low-risk MGUS, intermediate-risk MGUS, high-risk MGUS, low-risk smoldering myeloma, intermediate-risk smoldering myeloma, high-risk smoldering myeloma, and multiple myeloma—to future 3 genomic-based categories: monoclonal gammopathy, early detection of multiple myeloma (in which myeloma-defining genomic events already have been acquired), and multiple myeloma (patients who are already progressing and clinically defined cases). Ongoing investigations will continue to advance the field.
Evidence-Based Minireview: Molecular precision and clinical uncertainty: should molecular profiling be routinely used to guide risk stratification in MDS?
Authors: Richardson Daniel R. , DeZern Amy E. 
Published: December 2021

Abstract
This is a focused clinical vignette and review of the literature in MDS to discuss the application of molecular sequencing for risk stratification in MDS. The authors utilize an exemplar patient case and explain the advantages and disadvantages, based on available data, of routine use of this testing for MDS patients.
Spatial frequency analysis detects altered tissue organization following hamstring strain injury at time of injury but not return to sport
Authors: Crawford Scott K. , Wille Christa M. , Stiffler-Joachim Mikel R. , Lee Kenneth S. , Bashford Greg R. , Heiderscheit Bryan C. 
Published: December 2021

Abstract
Background
Hamstring strain injury (HSI) diagnosis is often corroborated using ultrasound. Spatial frequency analysis (SFA) is a quantitative ultrasound method that has proven useful in characterizing altered tissue organization. The purpose of this study was to determine changes in muscular tissue organization using SFA following HSI.

Methods
Ultrasound B-mode images were captured at time of injury (TOI) and return to sport (RTS) in collegiate athletes who sustained an HSI. Spatial frequency parameters extracted from two-dimensional Fourier Transforms in user-defined regions of interest (ROI) were analyzed. Separate ROIs encompassed injured and adjacent tissue within the same image of the injured limb and mirrored locations in the contralateral limb at TOI. The ROIs for RTS images were drawn to correspond to the injury-matched location determined from TOI imaging. Peak spatial frequency radius (PSFR) and the fascicular banded pattern relative to image background (Mmax%) were compared between injured and adjacent portions within the same image with separate paired t-tests. Within-image differences of SFA parameters in the injured limb were calculated and compared between TOI and RTS with Wilcoxon rank sum tests.

Results
Within the injured limb at TOI, PSFR differences in injured and healthy regions did not strictly meet statistical significance (p = 0.06), while Mmax% was different between regions (p < 0.001). No differences were observed between regions in the contralateral limb at TOI (PSFR, p = 0.16; Mmax%, p = 0.30). Significant within-image differences in PSFR (p = 0.03) and Mmax% (p = 0.04) at RTS were detected relative to TOI.

Conclusions
These findings are a first step in determining the usefulness of SFA in muscle injury characterization and provide quantitative assessment of both fascicular disruption and edema presence in acute HSI.
Catastrophic health expenditure and its determinants in households with lung cancer patients in China: a retrospective cohort study
Authors: Sun Cheng-yao , Shi Ju-fang , Fu Wen-qi , Zhang Xin , Liu Guo-xiang , Chen Wan-qing , He Jie 
Published: December 2021

Abstract
Background
Numerous studies have examined catastrophic health expenditures (CHE) worldwide, mostly focusing on general or common chronic populations, rather than particularly vulnerable groups. This study assessed the medical expenditure and compensation of lung cancer, and explored the extent and influencing factors of CHE among households with lung cancer patients in China.

Methods
During 2018–2019, a hospital-based multicenter retrospective survey was conducted in seven provinces/municipalities across China as a part of the Cancer Screening Program of Urban China. CHE was measured according to the proportion of out-of-pocket (OOP) health payments of households on non-food expenditures. Chi-square tests and logistic regression analysis was adjusted to determine the factors that significantly influenced the likelihood of a household with lung cancer patient to incur in CHE.

Results
In total, 470 households with lung cancer patients were included in the analysis. Health insurance was shown to protect some households from the impact of CHE. Nonetheless, CHE incidence (78.1%) and intensity (14.02% for average distance and 22.56% for relative distance) were still relatively high among households with lung cancer patients. The incidence was lower in households covered by the Urban Employee Basic Medical Insurance (UEMBI) insurance, with higher income level and shorter disease course.

Conclusion
More attention is needed for CHE incidence among vulnerable populations in China. Households with lung cancer patients were shown to be more likely to develop CHE. Therefore, policy makers should focus on improving the financial protection and reducing the economic burden of this disease.
Same-sex behavior disclosure to health care providers associated with greater awareness of pre-exposure prophylaxis
Authors: Cao Wangnan , You Xinyi , Li Jinghua , Peng Liping , Gu Jing , Hao Chun , Hou Fengsu , Wei Dannuo , Deng Yang , Hao Yuantao , Mo Phoenix Kit-han 
Published: December 2021

Abstract
Background
This study aimed to determine whether the disclosure of same-sex behavior to health care providers (HCPs) is associated with higher rates of prior human immunodeficiency virus (HIV) testing experience and greater awareness of immediate antiretroviral therapy (ART), Undetectable = Untransmittable (U=U), and pre-exposure prophylaxis (PrEP) among men who have sex with men (MSM).

Methods
We conducted a cross-sectional survey among 689 adult males in Chengdu, China who self-reported having had anal intercourse with at least one man in the past 6 months. We measured same-sex behavior disclosure to three types of HCPs (hospital clinicians, community-based organization peer educators, and Center for Disease Control and Prevention public health specialists), and the awareness of immediate ART, U=U, and PrEP.

Results
Of the 689 enrolled participants, 31.4% had disclosed their same-sex behavior to some or all of the clinicians, 83.9% had done so to the peer educators, and 56.8% had done so to the public health specialists. Approximately four in five (82.1%) of the participants had ever been tested for HIV. The awareness rate was 84.8% for immediate ART, 20.2% for U=U, and 50.7% for PrEP. After controlling for significant background variables, same-sex behavior disclosure to clinicians was associated with greater awareness of PrEP (adjusted odds ratio [AOR] = 1.64; 95% confidence interval [CI]: 1.08–2.48), but similar findings were not reported regarding disclosure to peer educators or public health specialist. Same-sex behavior disclosure to any types of HCPs was not associated with HIV testing experience, and awareness of immediate ART or U=U.

Conclusions
The rates of same-sex behavior disclosure varied with different types of HCPs. Disclosure to clinicians was associated with greater awareness of PrEP, but not awareness of immediate ART or U=U.
An analysis of health inequalities depending on educational level using nationally representative survey data in Japan, 2019
Authors: Okui Tasuku 
Published: December 2021

Abstract
Background
In recent years, socioeconomic differences in health statuses and behaviors have not been investigated from the nationally representative survey data in Japan. In this study, we showed differences in representative health behaviors and statuses depending on educational level using a nationally representative survey data in Japan.

Methods
Aggregated (not individual level) data from the Comprehensive Survey of Living Conditions in 2019 were used to examine the association between educational level and outcome status of psychological distress (K6 scores > = 5), self-rated health, smoking, alcohol drinking, and cancer screening participation (stomach, lung, colorectal, breast, and uterine cancers). Data of 217,179 households in Japan were aggregated by the Ministry of Health, Labour, and Welfare in the survey, and the data of the estimated number of household members and persons corresponding to each response option for the questions in all of Japan were used. Five-year age groups from 20 to 24 to 80–84 years and over 84 years were analyzed, and the prevalence or participation rate by educational level were calculated. In addition, the age-standardized prevalence or participation rate according to educational level were also calculated by sex. Moreover, a Poisson regression model was applied for evaluating an association of educational level with the outcomes.

Results
As a result, a clear gradient by educational level was observed in almost all the age groups for the prevalence of psychological distress, poor self-rated health, and smoking and participation rates in cancer screening, and high educational level were associated with better health-related behaviors and statuses. Conversely, drinking prevalence was shown to be higher rather in highly educated people. In addition, a statistically significant association of educational level with all the outcomes was observed.

Conclusion
It was shown that disparities in health behaviors and statuses still persisted in recent years, and the findings suggested that further measures should be taken to tackle this disparity.
The role of suction drainage in the management of peri-operative bleeding in Total and Unicompartmental knee arthroplasty: a retrospective comparative study
Authors: Manta Nicola , Mangiavini Laura , Balbino Cristiana , Colombo Andrea , Pandini Elisa Giacomina , Pironti Pierluigi , Viganò Marco , D’Anchise Roberto 
Published: December 2021

Abstract
Background
Suction drainage is commonly applied after total knee arthroplasty (TKA) and unicompartmental knee arthroplasty (UKA) to reduce hematoma, swelling and to favor surgical wound healing. However, its efficacy remains controversial; thus, the purpose of this study is to evaluate drainage efficiency in the management of postoperative bleeding in TKA and UKA.

Methods
The cohort comprised 134 clinical records of patients affected by knee osteoarthritis (OA) who underwent either TKA or UKA. All the patients were subdivided into 2 groups: the first one with drainage and the second one without drainage (respectively 61 and 73 patients). For each group, hemoglobin levels in the preoperative, first, second and third postoperative day were collected. Postoperative complications such as swelling, bleeding from the surgical wound or the need for blood transfusion, were also recorded.

Results
Our results did not show any significant difference of hemoglobin levels in the first (p = 0.715), second (p = 0.203) and third post-operative day (p = 0.467) between the two groups. Moreover, no significant correlation between knee swelling or transfusion rate and the drainage was observed (p = 0.703 and p = 0.662 respectively). Besides, a significant correlation was found between bleeding from the surgical wound and the absence of drainage (p = 0.006).

Conclusions
The study demonstrates how the routine use of suction drainage does not provide substantial benefits in the postoperative blood loss management after TKA or UKA.

Trial registration
ClinicalTrials.gov NCT04508101, 09/08/2020, Retrospectively registered

Level of evidence
III
Recombinant growth hormone therapy in children with Turner Syndrome in Korea: a phase III Randomized Trial
Authors: Kim Jinsup , Kim Min-Sun , Suh Byung-Kyu , Ko Cheol Woo , Lee Kee-Hyoung , Yoo Han-Wook , Shin Choong Ho , Hwang Jin Soon , Kim Ho-Seong , Chung Woo Yeong , Kim Chan Jong , Han Heon-Seok , Jin Dong-Kyu 
Published: December 2021

Abstract
Background
Short stature is the most consistent characteristic feature of Turner syndrome (TS). To improve final heights of children with TS effectively, it is important to provide them with early and appropriate treatment using growth hormone (GH). The objective of this study was to assess the efficacy and safety of a new recombinant human GH, Growtropin®-II (DA-3002, Dong-A ST Co., Ltd) versus a comparator (Genotropin®, Pfizer Inc.) for Korean children with TS.

Methods
This open-label, active-controlled, parallel-group, randomized controlled phase III trial was conducted at 11 hospitals in Korea. Eligible patients (n = 58) were randomized to two groups: 1) DA-3002 group (administrated with DA-3002 at 0.14 IU [0.0450–0.050 mg] /kg/day); and 2) comparator group (administrated with the comparator at 0.14 IU [0.0450–0.050 mg] /kg/day).

Results
The change from baseline in annualized height velocity (HV) after a 52-week treatment period was 4.15 ± 0.30 cm/year in the DA-3002 group and 4.34 ± 0.29 cm/year in the comparator group. The lower bound of 95% two-sided confidence interval for group difference in the change of annualized HV (− 1.02) satisfied the non-inferiority margin (− 1.5). The change in height standard deviation score (HtSDS) at 52-week was 0.70 ± 0.23 for the DA-3002 group and 0.66 ± 0.39 for the comparator group, showing no significant (p = 0.685) difference between the two groups. The change of skeletal maturity defined as change in bone age/change in chronological age between the two groups was not significantly different (1.25 ± 0.58 for the DA-3002 group and 1.47 ± 0.45 for the comparator group, p = 0.134). Changes from baseline in serum insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein-3 (IGFBP-3) after 52 weeks of treatment did not differ significantly between the two groups (p = 0.565 and p = 0.388, respectively) either. The occurrence of adverse events was not statistically different between groups.

Conclusions
This study demonstrates that the efficacy and safety of GH treatment with DA-3002 in children with TS are comparable with those of the comparator. It is expected to analysis the long-term effect of DA-3002 on the increase of final adult height in children with TS and possible late-onset complications in the future.

Trial registration
The study was registered at ClinicalTrials.gov. ClinicalTrials.gov identifier: NCT01813630 (19/03/2013).