ERS/EBMT clinical practice guidelines on treatment of pulmonary chronic graft-versus-host disease in adults.

Chronic graft-versus-host disease (cGvHD) is a common complication after allogeneic haematopoietic stem cell transplantation, characterised by a broad disease spectrum that can affect virtually any organ. Although pulmonary cGvHD is a less common manifestation, it is of great concern due to its severity and poor prognosis. Optimal management of patients with pulmonary cGvHD is complicated and no standardised approach is available. The purpose of this joint European Respiratory Society (ERS) and European Society for Blood and Marrow Transplantation task force was to develop evidence-based recommendations regarding the treatment of pulmonary cGvHD phenotype bronchiolitis obliterans syndrome in adults. A multidisciplinary group representing specialists in haematology, respiratory medicine and methodology, as well as patient advocates, formulated eight PICO (patient, intervention, comparison, outcome) and two narrative questions. Following the ERS standardised methodology, we conducted systematic reviews to address these questions and used the Grading of Recommendations Assessment, Development and Evaluation approach to develop recommendations. The resulting guideline addresses common therapeutic options (inhalation therapy, fluticasone-azithromycin-montelukast, imatinib, ibrutinib, ruxolitinib, belumosudil, extracorporeal photopheresis and lung transplantation), as well as other aspects of general management, such as lung functional and radiological follow-up and pulmonary rehabilitation, for adults with pulmonary cGvHD phenotype bronchiolitis obliterans syndrome. These recommendations include important advancements that could be incorporated in the management of adults with pulmonary cGvHD, primarily aimed at improving and standardising treatment and improving outcomes.

Patient-Centered Digital Health Records and Their Effects on Health Outcomes: Systematic Review.

BACKGROUND: eHealth tools such as patient portals and personal health records, also known as patient-centered digital health records, can engage and empower individuals with chronic health conditions. Patients who are highly engaged in their care have improved disease knowledge, self-management skills, and clinical outcomes. OBJECTIVE: We aimed to systematically review the effects of patient-centered digital health records on clinical and patient-reported outcomes, health care utilization, and satisfaction among patients with chronic conditions and to assess the feasibility and acceptability of their use. METHODS: We searched MEDLINE, Cochrane, CINAHL, Embase, and PsycINFO databases between January 2000 and December 2021. PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines were followed. Eligible studies were those evaluating digital health records intended for nonhospitalized adult or pediatric patients with a chronic condition. Patients with a high disease burden were a subgroup of interest. Primary outcomes included clinical and patient-reported health outcomes and health care utilization. Secondary outcomes included satisfaction, feasibility, and acceptability. Joanna Briggs Institute critical appraisal tools were used for quality assessment. Two reviewers screened titles, abstracts, and full texts. Associations between health record use and outcomes were categorized as beneficial, neutral or clinically nonrelevant, or undesired. RESULTS: Of the 7716 unique publications examined, 81 (1%) met the eligibility criteria, with a total of 1,639,556 participants across all studies. The most commonly studied diseases included diabetes mellitus (37/81, 46%), cardiopulmonary conditions (21/81, 26%), and hematology-oncology conditions (14/81, 17%). One-third (24/81, 30%) of the studies were randomized controlled trials. Of the 81 studies that met the eligibility criteria, 16 (20%) were of high methodological quality. Reported outcomes varied across studies. The benefits of patient-centered digital health records were most frequently reported in the category health care utilization on the "use of recommended care services" (10/13, 77%), on the patient-reported outcomes "disease knowledge" (7/10, 70%), "patient engagement" (13/28, 56%), "treatment adherence" (10/18, 56%), and "self-management and self-efficacy" (10/19, 53%), and on the clinical outcome "laboratory parameters," including HbA1c and low-density lipoprotein (LDL; 16/33, 48%). Beneficial effects on "health-related quality of life" were seen in only 27% (4/15) of studies. Patient satisfaction (28/30, 93%), feasibility (15/19, 97%), and acceptability (23/26, 88%) were positively evaluated. More beneficial effects were reported for digital health records that predominantly focus on active features. Beneficial effects were less frequently observed among patients with a high disease burden and among high-quality studies. No unfavorable effects were observed. CONCLUSIONS: The use of patient-centered digital health records in nonhospitalized individuals with chronic health conditions is potentially associated with considerable beneficial effects on health care utilization, treatment adherence, and self-management or self-efficacy. However, for firm conclusions, more studies of high methodological quality are required. TRIAL REGISTRATION: PROSPERO (International Prospective Register of Systematic Reviews) CRD42020213285; https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=213285.

Language and Geographic Representation of Neurosurgical Journals: A Meta-Science Study.

OBJECTIVE: Medical journals have a role in promoting representation of neurosurgeons who speak primary languages other than English. We sought to characterize the language of publication and geographic origin of neurosurgical journals, delineate associations between impact factor (IF) and language and geographic variables, and describe steps to overcome language barriers to publishing. METHODS: Web of Science, Scopus, and Ulrich's Serial Analysis system were searched for neurosurgery journals. The journals were screened for relevance. Language of publication, country and World Health Organization region, World Bank income status and gross domestic product, and citation metrics were extracted. RESULTS: Of 867 journals, 74 neurosurgical journals were included. Common publication languages were English (52, 70.3%), Mandarin (5, 6.8%), and Spanish (4, 5.4%). Countries of publication for the greatest number of journals were the United States (23, 31.1%), United Kingdom (8, 10.8%), and China (6, 8.1%). Most journals originated from the Americas region (29, 39.2%), the European region (28, 37.8%), and from high-income countries (n = 54, 73.0%). Median IF was 1.55 (interquartile range [IQR] 0.89-2.40). Journals written in English (1.77 [IQR 1.00-2.87], P = 0.032) and from high-income countries (1.81 [IQR 1.0-2.70], P = 0.046) had highest median IF. When excluding outliers, there was a small but positive correlation between per capita gross domestic product and IF (ß = 0.021, P = 0.03, R2 = 0.097). CONCLUSIONS: Language concordance represents a substantial barrier to research equity in neurosurgery, limiting dissemination of ideas of merit that currently have inadequate outlets for readership. Initiatives aimed at increasing the accessibility of neurosurgical publishing to underrepresented authors are essential.

Visual Outcomes After Treatment for Sporadic Optic Pathway Gliomas in Pediatric Patients: A Systematic Review.

OBJECTIVE: Optic pathway gliomas (OPGs) typically occur in the first decade of life and 40%-50% are not associated with neurofibromatosis 1 (NF1) (sporadic). Management strategies are often patient specific because of the variable and unpredictable course. No study has summarized the effect of treatment strategies on visual outcomes in the subset of pediatric patients with sporadic OPG. METHODS: We conducted a systematic review to determine the nature of visual outcomes in pediatric patients with sporadic, non-NF1-associated OPG using the PubMed, Embase, Scopus, Cochrane, and CINAHL Plus databases. Visual outcomes were categorized as improved, unchanged, or deteriorated. RESULTS: Of 1316 results, 31 articles were included. Treatment indications are unknown with full clinical detail. A total of 45.2% (14/31) reported deteriorated outcomes after treatment, 35.5% (11/31) no change, and 19.4% (6/31) improvement. Of radiotherapy studies, 50.0% (4/8) found no change, 37.5% (3/8) deterioration, and 12.5% (1/8) improvement. Of chemotherapy studies, 35.7% (5/14) each showed improvement and deterioration, whereas 28.6% (4/14) showed no change. Of surgical studies, 62.5% (5/8) indicated deterioration, and 37/5% (3/8) indicated no change. The singular study examining observation reported deterioration in visual outcomes. Factors associated with poor visual outcomes included signs and symptoms of visual decline at presentation, involvement of the intraorbital optic nerve, and intracranial hypertension requiring surgery. Causality cannot be determined from systematic review. CONCLUSIONS: Most studies showed that vision in pediatric patients with sporadic OPG is stable to poor after observation, chemotherapy, radiotherapy, or surgery. Chemotherapy may be associated with most favorable visual outcomes.

Vision Outcomes for Pediatric Patients With Optic Pathway Gliomas Associated With Neurofibromatosis Type I: A Systematic Review of the Clinical Evidence.

Children with neurofibromatosis type I (NF1) have a higher predisposition for low-grade astrocytomas of the optic pathway, commonly referred to as optic pathway gliomas (OPGs). OPGs can result in visual deterioration. Treatment outcomes in OPG-NF1 management are often reported around tumor stabilization. We sought to compare vision outcomes associated with different OPG treatment strategies to inform about this important functional metric. A meta-analysis exploring the different modalities to treat children with OPG-NF1 was conducted following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines using multiple databases. Of the 113 articles identified in the search, 23 full text articles, representing 564 patients, were included for review. These articles included retrospective, prospective, and randomized controlled studies on observation (n=9), chemotherapy (n=19), radiation therapy (n=6), and surgery (n=7). Of the patients undergoing observation, 87% (60/69) demonstrated stable acuity. In the chemotherapy studies, 27.3% (72/264) demonstrated improved acuity/visual field and/or visual-evoked potential amplitudes, 39.4% (104/264) stable acuity, and 33.3% (88/264) deterioration. Both the radiation and surgical treatments reported worsening acuity at 90.9% (10/11) and 73.3% (11/15), respectively. Causal associations are not known. Indications for and timing of treatment choice warrant larger scale study to provide further understanding.

Hypocalcemia prevention and management after thyroidectomy in children: A systematic review.

INTRODUCTION: Hypocalcemia is the most common complication following thyroidectomy in children. Guidelines to manage post-thyroidectomy hypocalcemia are available for adults, but not children. The objective of this review was to identify practices related to hypocalcemia prevention and management in pediatric patients. METHODS: We identified studies examining the prevention and management of hypocalcemia in pediatric patients post-thyroidectomy within PubMed, EMBASE, Web of Science and Cochrane databases. Three independent reviewers screened citations and reviewed full-text papers. RESULTS: A total of 15 studies were included, representing 1552 patients. The overall study quality was weak with lack of randomization and inconsistent outcome reporting. The pooled incidence of hypocalcemia from the 15 studies was 35.5% for transient hypocalcemia and 4.2% for permanent hypocalcemia. All studies discussed post-operative hypocalcemia treatment, with most patients requiring admission for intra-venous calcium therapy. One study described a protocol discharging asymptomatic patients on calcitriol and calcium. Three studies discussed preoperative calcium supplementation in patients at risk of hypocalcemia. No studies examined routine use of calcium and/or vitamin D supplementation to prevent post-operative hypocalcemia. CONCLUSION: A significant number of children undergoing thyroidectomy develop hypocalcemia. Despite this high incidence, our systematic review demonstrates significant practice variation surrounding post-thyroidectomy hypocalcemia prevention and management in children. LEVEL OF EVIDENCE: III (systematic review of studies of which some were case-control studies (III) and some were case series (IV)).

Treatment of pediatric cerebral venous sinus thromboses: the role of anticoagulation.

BACKGROUND: Cerebral venous sinus thromboses (CVST) occur in children with a variety of etiologies. However, no standard treatment paradigm is established. We sought to identify what treatments have been applied, their outcomes, and the role of anticoagulation in pediatric patients with CVST. METHODS: A systematic review was conducted exploring all treatments of pediatric CVSTs using PubMed, Embase, Scopus, and Cochrane Library and Cochrane Central Register of Controlled Trials. Studies meeting inclusion criteria were reviewed in full and analyzed for study design, aim, population, interventions, and outcomes. RESULTS: Of 2946 resultant articles, 51 full-text articles were included. Management of infectious CVST included broad-spectrum antibiotics, surgery, and anticoagulation. Neoplastic and traumatic CVST treatment included anticoagulation. Treatment of CVSTs associated with metabolic abnormalities centered on correction of metabolic derangements, or supplementation where appropriate, and anticoagulation. Autoimmune, congenital, and thrombotic pathway CVSTs were treated with anticoagulation and treatment of the underlying disorder. Unfractionated heparin and low molecular weight heparin were most commonly used and seen to be effective and safe. Uncommonly, endovascular interventions including venous thrombectomy and intravenous injection of thrombolytic therapy were used with varying success. CONCLUSIONS: While conservative, medical, thombolytic, endovascular, and surgical treatment all have a role in in the treatment of pediatric CVSTs, anticoagulation is commonly applied and found to be safe and effective in pediatrics. Risks and benefits of anticoagulation must be considered on an individual basis as no randomized trials have established a standard of care. Based on our findings, we propose an approach to CVST treatment and look to future study aimed at more clearly delineating treatment dose, duration, and timing of re-evaluation in these patients.