bluuebunny/crossref_metadata_2025_split huggingface dataset:
International Board Certified Lactation Consultants’ Practices Regarding Supplemental Feeding Methods for Breastfed Infants
Authors: Penny Frances , Judge Michelle , Brownell Elizabeth A. , McGrath Jacqueline M. 
Published: April 2019

Background: According to the Baby Friendly Hospital Initiative, when supplementary feeding occurs, mothers should be counseled on the use and risks of feeding bottles and teats. To help support this initiative it is important to understand the supplementation practices of Internationally Board Certified Lactation Consultants (IBCLC)®. Research aims: To determine (1) if the location of an IBCLC’s practice has any impact on supplemental feeding methods; (2) the preferred methods of and the main reasons for supplementation; (3) the level of an IBCLC’s confidence with supplemental feeding methods; (4) who is making supplemental feeding decisions; and (5) if there are geographical differences among supplementation choices and reasons for supplementation. Methods: An exploratory, descriptive, cross sectional survey of IBCLCs was conducted to generate data about their use of supplemental feeding methods. The survey was sent via email invitation through the International Board of Lactation Consultant’s Board of Examiners, with a response rate of 11.5% ( N = 2,308). Results: There was no standard method of supplementation among participants. Participants indicated that they were confident advising mothers on alternative feeding methods. Only 17.6% ( n = 406) of participants reported that the IBCLC was the caregiver who recommended the method of supplementation used. The majority of participants believed the Supplemental Feeding Tube Device SFTD) best preserves the breastfeeding relationship, and this was their preferred method of supplementation. However, the bottle was ranked as the number one method used in the United States, Australia, and Canada. The use of alternative feeding methods may be overwhelming to the mother. Conclusion: Supplementation by alternative feeding methods might help preserve the breastfeeding relationship and help reach the World Health Organization’s goal of increasing exclusive breastfeeding rates.
Chemerin and Dermcidin in Human Milk and Their Alteration in Gestational Diabetes
Authors: Ustebay Sefer , Baykus Yakup , Deniz Rulin , Ugur Kader , Yavuzkir Seyda , Yardim Meltem , Kalayci Mehmet , Çaglar Mete , Aydin Suleyman 
Published: April 2019

Background: Chemerin and dermcidin, which have antimicrobial properties, are molecules that are also related to insulin resistance and inflammation. Research aims: The aims were to determine the amounts of chemerin and dermcidin in the milk and blood of mothers with gestational diabetes, and to compare the amounts of chemerin and dermcidin in the milk and blood of mothers with and without diabetes. Methods: This was a two-group nonrandomized longitudinal study with a convenience sampling of mothers without gestational diabetes ( n = 27) and mothers with gestational diabetes ( n = 26). Human milk and blood samples were obtained from these mothers during colostrum, transitional, and mature milk periods. The amount of chemerin and dermcidin in these samples was measured by enzyme-linked immunosorbent assay. Results: The presence of chemerin and dermcidin was first detected in human milk. The amounts of chemerin and dermcidin in the blood of all the mothers were greater in the colostrum period and lowest in the mature period. The amount of chemerin and dermcidin in the milk of all the mothers was greater than that in the blood. The amounts of chemerin and dermcidin were significantly increased in both blood and human milk within the gestational age samples. Conclusions: Chemerin and dermcidin may contribute to the protection of infants from infections during infancy. Increased amounts of these molecules found within the gestational diabetes group may also prevent adverse maternal and fetal outcomes.
Improved biological insight and influence on management in indolent lymphoma. Talk 3: update on nodal and splenic marginal zone lymphoma
Authors: Thieblemont Catherine 
Published: April 2019

Abstract
Splenic marginal zone lymphoma (SMZL) and nodal marginal zone lymphoma (NMZL) are rare indolent chronic B-cell lymphomas. Prognosis is typically good with median survival around 10-15 years. Management is generally based on the presence of symptoms or high tumor burden. There are no standard treatments for these 2 entities, and therapeutic strategies are rapidly evolving. Clinical developments for these 2 entities are oriented by genomic studies, with largely overlapping mutational profiles involving the NOTCH, B-cell receptor (BcR) and nuclear factor κB (NF-κB) signaling, chromatin remodeling, and the cytoskeleton. Although new therapeutic options based on targeting signaling pathways and overcoming resistance are increasingly available, few specific prospective studies are performed for these rare subtypes, limiting the conclusions that can be drawn. Novel drugs targeting B-cell signaling have shown promise, including ibrutinib and copanlisib. The second-generation oral immunomodalator (IMiD) lenalidomide showed impressive results when combined with rituximab. Other potential solutions include targeting the NF-κB, JAK/STAT, BCL2, NOTCH, and Toll-like receptor signaling pathways; however, studies in these 2 MZL entities are yet to prove a definitive benefit. Molecular profiling is now a cornerstone of diagnostic, prognostic, and therapeutic strategies to offer patient- and disease-specific solutions. The development of a wider range of effective targeted therapies and prognostic biomarkers is keenly awaited and is expected to strongly affect the natural history of SMZL and NMZL.
Acquired ribosomopathies in leukemia and solid tumors
Authors: Vlachos Adrianna 
Published: April 2019

AbstractA mutation in the gene encoding the small subunit-associated ribosomal protein RPS19, leading to RPS19 haploinsufficiency, is one of the ribosomal protein gene defects responsible for the rare inherited bone marrow failure syndrome Diamond Blackfan anemia (DBA). Additional inherited and acquired defects in ribosomal proteins (RPs) continue to be identified and are the basis for a new class of diseases called the ribosomopathies. Acquired RPS14 haploinsufficiency has been found to be causative of the bone marrow failure found in 5q– myelodysplastic syndromes. Both under- and overexpression of RPs have also been implicated in several malignancies. This review will describe the somatic ribosomopathies that have been found to be associated with a variety of solid tumors as well as leukemia and will review cancers in which over- or underexpression of these proteins seem to be associated with outcome.
Genetics and prognosis of ALL in children vs adults
Authors: Roberts Kathryn G. 
Published: April 2019

Abstract
Acute lymphoblastic leukemia (ALL) is characterized by genetic alterations that block differentiation, promote proliferation of lymphoid precursor cells, and are important for risk stratification. Although ALL is less common in adolescents and young adults (AYAs) and adults than children, survival rates are inferior, and long-term prognosis for adults is poor. Thus, ALL remains a challenging disease to treat in the AYA and adult populations. A major contributing factor that influences prognosis in this population is the reduced prevalence of genetic subtypes associated with favorable outcome and a concomitant increase in subtypes associated with poor outcome. Recent advances in genomic profiling across the age spectrum continue to enhance our knowledge of the differences in disease biology between children and adults and are providing important insights into novel therapeutic targets. Philadelphia chromosome-like (Ph-like) ALL is one such subtype characterized by alterations that deregulate cytokine receptor or tyrosine kinase signaling and are amenable to inhibition with approved tyrosine kinase inhibitors. One of the greatest challenges now remaining is determining how to implement this breadth of genomic information into rapid and accurate diagnostic testing to facilitate the development of novel clinical trials that improve the outcome of AYAs and adults with ALL.
Inherited hemolytic anemia: a possessive beginner’s guide
Authors: Mohandas Narla 
Published: April 2019

Abstract
Significant advances have been made in diagnosis and clinical management of inherited red cell membrane disorders that result in hemolytic anemia. Membrane structural defects lead to hereditary spherocytosis (HS) and hereditary elliptocytosis (HE), whereas altered membrane transport function accounts for hereditary xerocytosis (HX) and hereditary overhydrated stomatocytosis (OHS). The degrees of membrane loss and resultant increases in cell sphericity determine the severity of anemia in HS and HE, and splenectomy leads to amelioration of anemia by increasing the circulatory red cell life span. Alterations in cell volume as a result of disordered membrane cation permeability account for reduced life span red cells in HX and OHS. Importantly, splenectomy is not beneficial in these 2 membrane transport disorders and is not recommended because it is ineffective and may lead to an increased risk of life-threatening thrombosis. Rational approaches are now available for the diagnosis and management of these inherited red cell disorders, and these will be discussed in this review.
A brief, but comprehensive, guide to clonal evolution in aplastic anemia
Authors: Babushok Daria V. 
Published: April 2019

Abstract
Acquired aplastic anemia (AA) is an immune-mediated bone marrow aplasia that is strongly associated with clonal hematopoiesis upon marrow recovery. More than 70% of AA patients develop somatic mutations in their hematopoietic cells. In contrast to other conditions linked to clonal hematopoiesis, such as myelodysplastic syndrome (MDS) or clonal hematopoiesis of indeterminate potential in the elderly, the top alterations in AA are closely related to its immune pathogenesis. Nearly 40% of AA patients carry somatic mutations in the PIGA gene manifested as clonal populations of cells with the paroxysmal nocturnal hemoglobinuria phenotype, and 17% of AA patients have loss of HLA class I alleles. It is estimated that between 20% and 35% of AA patients have somatic mutations associated with hematologic malignancies, most characteristically in the ASXL1, BCOR, and BCORL1 genes. Risk factors for evolution to MDS in AA include the duration of disease, acquisition of high-risk somatic mutations, and age at AA onset. Emerging data suggest that several HLA class I alleles not only predispose to the development of AA but may also predispose to clonal evolution in AA patients. Long-term prospective studies are needed to determine the true prognostic implications of clonal hematopoiesis in AA. This article provides a brief, but comprehensive, review of our current understanding of clonal evolution in AA and concludes with 3 cases that illustrate a practical approach for integrating results of next-generation molecular studies into the clinical care of AA patients in 2018.
Platform-Independent Classification System to Predict Molecular Subtypes of High-Grade Serous Ovarian Carcinoma
Authors: Shilpi Arunima , Kandpal Manoj , Ji Yanrong , Seagle Brandon L. , Shahabi Shohreh , Davuluri Ramana V. 
Published: April 2019

PURPOSEMolecular cancer subtyping is an important tool in predicting prognosis and developing novel precision medicine approaches. We developed a novel platform-independent gene expression–based classification system for molecular subtyping of patients with high-grade serous ovarian carcinoma (HGSOC).METHODSUnprocessed exon array (569 tumor and nine normal) and RNA sequencing (RNA-seq; 376 tumor) HGSOC data sets, with clinical annotations, were downloaded from the Genomic Data Commons portal. Sample clustering was performed by non-negative matrix factorization by using isoform-level expression estimates. The association between the subtypes and overall survival was evaluated by Cox proportional hazards regression model after adjusting for the covariates. A novel classification system was developed for HGSOC molecular subtyping. Robustness and generalizability of the gene signatures were validated using independent microarray and RNA-seq data sets.RESULTSSample clustering recaptured the four known The Cancer Genome Atlas molecular subtypes but switched the subtype for 22% of the cases, which resulted in significant ( P = .006) survival differences among the refined subgroups. After adjusting for covariate effects, the mesenchymal subgroup was found to be at an increased hazard for death compared with the immunoreactive subgroup. Both gene- and isoform-level signatures achieved more than 92% prediction accuracy when tested on independent samples profiled on the exon array platform. When the classifier was applied to RNA-seq data, the subtyping calls agreed with the predictions made from exon array data for 95% of the 279 samples profiled by both platforms.CONCLUSIONIsoform-level expression analysis successfully stratifies patients with HGSOC into groups with differing prognosis and has led to the development of robust, platform-independent gene signatures for HGSOC molecular subtyping. The association of the refined The Cancer Genome Atlas HGSOC subtypes with overall survival, independent of covariates, enhances the clinical annotation of the HGSOC cohort.
Advanced Epithelial Ovarian Cancer: Do More Options Mean Greater Benefits?
Authors: Bouberhan Sara , Shea Meghan , Cannistra Stephen A. 
Published: April 2019

The Oncology Grand Rounds series is designed to place original reports published in the Journal into clinical context. A case presentation is followed by a description of diagnostic and management challenges, a review of the relevant literature, and a summary of the authors’ suggested management approaches. The goal of this series is to help readers better understand how to apply the results of key studies, including those published in Journal of Clinical Oncology, to patients seen in their own clinical practice. A healthy 51-year-old woman presented with increasing abdominal and pelvic pain. Computed tomography imaging of the abdomen and pelvis showed an 11.6-cm pelvic mass, retroperitoneal lymphadenopathy, right hydronephrosis, and mesenteric tumor deposits ( Fig 1A ). A serum CA-125 was elevated at 1,149 U/mL. She underwent primary surgical cytoreduction including hysterectomy, bilateral salpingo-oophorectomy, appendectomy, resection of pelvic tumor, omentectomy, and low anterior resection with colorectal anastomosis. Intraoperatively, she was noted to have bilateral ovarian masses, pelvic and para-aortic lymphadenopathy, and a 4-cm omental tumor; in addition, both the uterus and rectosigmoid colon had adherent tumor deposits. All gross tumor was resected during the procedure. Final pathology confirmed high-grade serous carcinoma of ovarian origin ( Fig 1B ) that was determined to be stage IIIC as a result of upper abdominal involvement with greater than 2-cm tumor deposits, as well as retroperitoneal lymph node involvement. She underwent germline genetic testing, which did not identify a mutation in the BRCA1, BRCA2, BRIP1, RAD51C, or RAD51D genes. She presented for adjuvant chemotherapy after an optimal (R0) resection.
Clinical Features of Multiple Endocrine Neoplasia Type 4: Novel Pathogenic Variant and Review of Published Cases
Authors: Frederiksen Anja , Rossing Maria , Hermann Pernille , Ejersted Charlotte , Thakker Rajesh V , Frost Morten 
Published: April 2019

Abstract

Context
The clinical phenotype of multiple endocrine neoplasia type 4 (MEN4) is undefined due to a limited number of published cases. Knowledge on disease manifestation in MEN4 is essential for developing prevention programs and treatment.

Objective
To expand current knowledge of the MEN4 phenotype including assessment of penetrance.

Design
This is a case report and a brief review of previously published MEN4 cases.

Patients
We report a large Danish family with multiple cases of endocrine tumors that segregated with a pathogenic variant in the CDKN1B gene.

Main Outcome/Result
The medical history of the proband included primary hyperparathyroidism and Cushing disease. Genetic analysis identified a pathogenic variant in CDKN1B (c.121_122delTT, p.Leu41Asnfs*83). Among the family members, another 12 individuals were identified as carriers of the same variant, which segregated with development of endocrine tumors. Hypercalcemia due to primary hyperparathyroidism occurred in all 13 of the available carriers of the genetic variant, and 4 patients also had functioning or nonfunctioning pituitary adenomas, whereas 1 patient had a metastatic neuroendocrine tumor (carcinoid). Loss-of-heterozygosity was detected in two of five parathyroid adenomas, supporting that CDKN1B acts as a tumor suppressor gene. Thirty cases representing 16 different CDKN1B variants have previously been reported, and these cases presented primarily with primary hyperparathyroidism and functioning and nonfunctioning pituitary tumors.

Conclusion
Hypercalcemia due to primary hyperparathyroidism and pituitary tumors are common in MEN4. Gastrointestinal neuroendocrine tumors appear to be less prevalent in MEN4 than in MEN1.
Brain organoids: advances, applications and challenges
Authors: Qian Xuyu , Song Hongjun , Ming Guo-li 
Published: April 2019

ABSTRACT
Brain organoids are self-assembled three-dimensional aggregates generated from pluripotent stem cells with cell types and cytoarchitectures that resemble the embryonic human brain. As such, they have emerged as novel model systems that can be used to investigate human brain development and disorders. Although brain organoids mimic many key features of early human brain development at molecular, cellular, structural and functional levels, some aspects of brain development, such as the formation of distinct cortical neuronal layers, gyrification, and the establishment of complex neuronal circuitry, are not fully recapitulated. Here, we summarize recent advances in the development of brain organoid methodologies and discuss their applications in disease modeling. In addition, we compare current organoid systems to the embryonic human brain, highlighting features that currently can and cannot be recapitulated, and discuss perspectives for advancing current brain organoid technologies to expand their applications.
Aesthetic management of white lesions in the permanent dentition
Authors: Field Richard 
Published: April 2019

Tooth whitening to treat unaesthetic yellow/brown lesions has a strong evidence base and is a routine cosmetic procedure in many dental practices. The management of white lesions, however, is more challenging and is the subject of debate. As traditional whitening procedures may make white lesions more obvious in the short term, there is a risk that patients may be misinformed that limited treatment options are available, and they may be prescribed invasive cosmetic procedures instead. This paper aims to help clinicians accurately diagnose aetiological factors that produce white lesions and describes a hierarchy of treatment options which are both aesthetic and biologically respectful, focusing on the materials, equipment and clinical techniques that are available for the minimally invasive management of white lesions using resin infiltration. CPD/Clinical Relevance: This paper aims to help clinicians select the optimal materials, equipment and clinical techniques for the minimally invasive management of unaesthetic white enamel defects.
Resin Luting Materials
Authors: Burke F J Trevor 
Published: April 2019

Luting materials are a central component of indirect dentistry, with the most recently introduced types being resin-based materials. These may be classified into conventional resin luting materials, self-adhesive resin luting materials and ‘smart’ resin luting materials. Their physical properties have been found to be good, with no risk of dissolution in the dilute organic acids found in plaque, such as occurs with phosphate and glass ionomer cements. Uses of resin luting materials include the luting of any indirect restoration, but these materials have facilitated the development of the resin-retained bridge and aesthetic ceramic or composite restorations, notwithstanding the fact that they may provide, for the first time, truly adhesive luting of crowns and inlays. CPD/Clinical Relevance: Resin luting materials are appropriate for placement of all indirect restorations: the self-adhesive variants have simplified the use of such materials.
Don't forget the foal: the nursing requirements of hospitalised foals when the mare is the primary patient
Authors: Harniman Suzannah 
Published: April 2019

In the post-partum period, there are a range of factors that could result in the hospitalisation of the mare. To minimise the long-term impact that the hospitalisation period has on the physical and behavioural development of the foal veterinary practices should have a protocol in place to care for a foal even though the mare is the primary patient. When patients are admitted detailed information should be gathered from the owner about both the mare and the foal. This provides valuable information necessary to plan appropriate nursing care. Important factors that need to be considered when planning care include the nutritional needs of the foal, the development of the selective bond between the mare and the foal and the handling of them as patients.Where possible the owner should be encouraged to participate in the care of their mare and the foal during the hospitalisation period. This will help the owner feel more involved in the provision of care and have a better understanding of the mare and foal's care requirements post discharge. This is a technique used in human paediatric nursing and is known as parent participation in care.
Reviews
Authors: Guo Ting , Kimbrough Andrew , Duchan Joshua S. , Young Miriama , Tebbutt John 
Published: April 2019

The Sociolinguistics of Voice in Globalising China, Dong Jie (2017) London and New York: Routledge, 160 pp., ISBN 978-1-13879-880-9, h/bk, £115.00
Theatre & Voice, Konstantinos Thomaidis (2017) London: Palgrave, 89 pp., ISBN 978-1-13755-249-5, USD 17.99, £ 6.99
Karaoke Idols: Popular Music and the Performance of Identity, Kevin Brown (2015) Bristol: Intellect, xvii + 152 pp., ISBN 978-1-78320-444-1, p/bk, $36
Postopera: Reinventing the Voice-Body, Jelena Novak (2015) Ashgate Interdisciplinary Studies in Opera, Dorchester: Ashgate, 192 pp., ISBN 978-1-47244-103-4, h/bk, $120; ISBN 978-1-13850-496-7, p/bk, $49.95
The Voice and its Double: Media and Music in Northern Australia, Daniel Fisher (2016) Durham and London: Duke University Press, 320 pp., ISBN 978-0-82236-086-9, h/bk, $99.95; ISBN 978-0-82236-120-6, p/bk, $26.95
OHS Co-Founder Henry W Hofstetter (1914-2002)
Authors: Goss David A. 
Published: April 2019

Henry W Hofstetter, OD, PhD, had a long and distinguished career as an optometric educator, writer, researcher, administrator, and leader. This biographical sketch examines his life and career, and notes his role in fostering the study and understanding of optometry’s history, as exemplified by his work as co-founder of the Optometric Historical Society and long-time editor of its quarterly publication, Hindsight.