bluuebunny/crossref_metadata_2025_split huggingface dataset:
Hearing Loss in Mucopolysaccharidosis
Authors: Bicalho Cibele Gomes , de Araújo Leão Emília Katiane Embiruçu , de Andrade Álvaro Muiños , Acosta Angelina Xavier 
Published: September 2020

Abstract
Introduction Mucopolysaccharidosis (MPS) is a set of rare diseases caused by deficiency of lysosomal enzymes that lead to the accumulation of glycosaminoglycans (GAG) in tissues and organs, which, in turn, is responsible for the multisystemic clinical, chronic, and progressive symptoms.
Objective To describe the profile of the otorhinolaryngological clinical examination and audiology tests of patients with MPS disease.
Methods The present study is a case series. The evaluation was performed, initially, in 24 patients with MPS types I, II, IIIA, IV and VI.
Results The most common hearing complaint was hearing loss, which was confirmed by audiology tests in almost 100% of the patients, most of whom presented conductive hearing loss.
Conclusions It is important to evaluate the complaints, physical examination, and audiology tests in patients with MPS. The otorhinolaryngologistshould be part of the group of professionals that follows these patients to better monitor their hearing and provide early hearing rehabilitation.
Atlantoaxial Rotatory Dislocation: Delayed Diagnose Will Result in More Invasive Treatment Options
Authors: Pitzen Tobias , Ruf Michael , Meyer Carolin , Drumm Joerg 
Published: September 2020

Abstract
Purpose The atlantoaxial joint represents the most mobile joint complex within the spine, secured by ligaments and capsules. Integrity of the atlantoaxial joint is crucial with respect to the mobility of the head and the upper spine. Atlantoaxial rotatory dislocation is the most common type of injury within this joint in children and is characterized by a typical position of the head (cock robin position). Nevertheless, this type of injury is frequently overlooked. The purpose of the current study was threefold. First, the characteristics of the patients with atlantoaxial dislocation were identified. Next, we checked if the time to treatment did influence the type of treatment. Finally, we checked if the age of the child at the time of treatment influenced the type of treatment.
Methods Forty-four children, who were treated consecutively due to atlantoaxial dislocation at a single spine center between September 1993 and October 2018, are analyzed retrospectively regarding age, sex, symptoms, etiology, time to diagnosis, time to treatment, and outcome.
Results Forty-four children (30 girls, mean age 8.9 years) were included in the study. The cock robin head position was found in all of them, but neurological deficits were not found in any of them. In 21 patients, dislocation was caused by previous infection (Grisel's syndrome), whereas in 19 patients, dislocation was due to minor trauma. In 4 cases, etiology remained unknown. Mean time to sufficient treatment was 178 days. Eighteen patients received closed reduction and immobilization after 57 days at mean. Open reduction followed by temporary fixation was done in 12 patients after a mean time gap of 188 days. Bony atlantoaxial fusion was necessary in 14 children, who were diagnosed after 319 days on average. Invasiveness of treatment was dependent on the time delay between development of dislocation and treatment; a significant difference was found between invasiveness of treatment and time to treatment (Kruskal–Wallis test, p < 0,05). Moreover, older children were treated significantly more often with fusion than younger ones (χ
2, p = 0,002).
Conclusion Young girls are predisposed to incur an atlantoaxial rotatory dislocation, which usually occurs due to minor trauma or infection. The cock robin position is characteristic, but neurological deficits are not common. There is a need for early and sufficient treatment because delayed treatment necessitates more invasive treatment, thus leading to a complete loss of function of the most mobile joint within the spine. Finally, older children are predisposed to more invasive treatment strategies.
Bayes Theorem and Protopathic Bias: Methodological Concerns When Addressing the Impact of Fetal Heart Rate Patterns on the Cesarean Section Rate
Authors: Balayla Jacques , Lasry Ariane , Gil Yaron , Volodarsky-Perel Alexander 
Published: September 2020

AbstractOver the last 30 years, the caesarean section rate has reached global epidemic proportions. This trend is driven by multiple factors, an important one of which is the use and inconsistent interpretation of the electronic fetal monitoring (EFM) system. Despite its introduction in the 1960s, the EFM has not definitively improved neonatal outcomes, yet it has since significantly contributed to a seven-fold increase in the caesarean section rate. As we attempt to reduce the caesarean rates in the developed world, we should consider focusing on areas that have garnered little attention in the literature, such as physician sensitization to the poor predictive power of the EFM and the research method biases that are involved in studying the abnormal heart rate patterns—umbilical cord pH relationship. Herein, we apply Bayes theorem to different clinical scenarios to illustrate the poor predictive power of the EFM, as well as shed light on the principle of protopathic bias, which affects the classification of research outcomes among studies addressing the effects of the EFM on caesarean rates. We propose and discuss potential solutions to the aforementioned considerations, which include the re-examination of guidelines with which we interpret fetal heart rate patterns and the development of noninvasive technologies that evaluate fetal pH in real time.
Ultrasound-Guided Insertion of the Ventricular Catheter in Ventriculoperitoneal Shunt Surgery: Evaluation of Accuracy and Feasibility in a Prospective Cohort
Authors: Leu Severina , Kamenova Maria , Mariani Luigi , Soleman Jehuda 
Published: September 2020

Abstract
Objective Ventriculoperitoneal shunt (VPS) placement is one of the most frequent neurosurgical procedures. The position of the proximal catheter is important for shunt survival. Shunt placement is done either without image guidance (“freehand”) according to anatomical landmarks or by use of various image-guided techniques. Studies evaluating ultrasound-guided (US-G) VPS placement are sparse. We evaluate the accuracy and feasibility of US-G VPS placement, and compare it to freehand VPS placement.
Methods We prospectively collected data of consecutive patients undergoing US-G VPS placement. Thereafter, the US cohort was compared with a cohort of patients in whom VPS was inserted using the freehand technique (freehand cohort). Primary outcome was accuracy of catheter positioning, and secondary outcomes were postoperative improvement in Evans' index (EI), rates of shunt dysfunction and revision surgery, perioperative complications, as well as operation, and anesthesia times.
Results We included 15 patients undergoing US-G VPS insertion. Rates of optimally placed shunts were higher in the US cohort (67 vs. 49%, p = 0.28), whereas there were no malpositioned VPS (0%) in the US cohort, compared with 10 (5.8%) in the freehand cohort (p = 0.422). None of the factors in the univariate analysis showed significant association with nonoptimal (NOC) VPS placement in the US cohort. The mean EI improvement was significantly better in the US cohort than in the freehand cohort (0.043 vs. 0.014, p = 0.035).
Conclusion Based on our preliminary results, US-G VPS placement seems to be feasible, safe, and increases the rate of optimally placed catheters.
Intraoperative MRI-guided Resection in Pediatric Brain Tumor Surgery: A Meta-analysis of Extent of Resection and Safety Outcomes
Authors: Wach Johannes , Banat Mohammad , Borger Valeri , Vatter Hartmut , Haberl Hannes , Sarikaya-Seiwert Sevgi 
Published: September 2020

Abstract
Background The objective of this meta-analysis was to analyze the impact of intraoperative magnetic resonance imaging (iMRI) on pediatric brain tumor surgery with regard to the frequency of histopathologic entities, additional resections secondary to iMRI, rate of gross total resections (GTR) in glioma surgery, extent of resection (EoR) in supra- and infratentorial compartment, surgical site infections (SSIs), and neurologic outcome after surgery.
Methods MEDLINE/PubMed Service was searched for the terms “intraoperative MRI,” “pediatric,” “brain,” “tumor,” “glioma,” and “surgery.” The review produced 126 potential publications; 11 fulfilled the inclusion criteria, including 584 patients treated with iMRI-guided resections. Studies reporting about patients <18 years, setup of iMRI, surgical workflow, and extent of resection of iMRI-guided glioma resections were included.
Results IMRI-guided surgery is mainly used for pediatric low-grade gliomas. The mean rate of GTR in low- and high-grade gliomas was 78.5% (207/254; 95% confidence interval [CI]: 64.6–89.7, p < 0.001). The mean rate of GTR in iMRI-assisted low-grade glioma surgery was 74.3% (35/47; 95% CI: 61.1–85.5, p = 0.759). The rate of SSI in surgery assisted by iMRI was 1.6% (6/482; 95% CI: 0.7–2.9). New onset of transient postoperative neurologic deficits were observed in 37 (33.0%) of 112 patients.
Conclusion IMRI-guided surgery seems to improve the EoR in pediatric glioma surgery. The rate of SSI and the frequency of new neurologic deficits after IMRI-guided surgery are within the normal range of pediatric neuro-oncologic surgery.
Duration of the Third Stage of Labor and Estimated Blood Loss in Twin Vaginal Deliveries
Authors: Whittington Julie R. , Pagan Megan , Daugherty Kristen , Cummings Kelly , Ounpraseuth Songthip T. , Eads Lauren , Magann Everett F. 
Published: September 2020

Objective The main aim of this study was to characterize the duration of the third stage of labor and estimated blood loss in twin vaginal deliveries.
Study Design This was a retrospective case–control study. The data was collected from deliveries at the University of Arkansas for Medical Sciences in Little Rock, Arkansas, from January 2013 to June 2017. Women were identified who had twin gestation, were delivered vaginally, and whose maternal age was greater than 18 years old. Women were excluded if they had an intrauterine fetal demise, delivered either/both fetuses via cesarean, history of a previous cesarean or a fetus with a congenital anomaly. If a subject met criteria to be included in the study, the next normal singleton vaginal delivery was used as the control subject.
Results There were 132 singleton vaginal deliveries and 133 twin vaginal deliveries analyzed. There was no significant difference in the length of the third stage of labor between twin and singleton vaginal deliveries except in the 95th percentile of the distribution. Mothers delivering twins had an increase in third-stage duration by 7.618 minutes (95% confidence interval [CI]: 0.73, 14.50; p = 0.03) compared with those who delivered singletons. The twin group had a higher estimated blood loss than singleton deliveries. The increase in blood loss in the twin group was 149.02 mL (95% CI: 100.2, 197.8), 257.01 mL (95% CI: 117.9, 396.1), and 381.53 mL (95% CI: 201.1, 562.1) at the 50th, 90th, and 95th percentiles, respectively. When the third stage of labor was at the 90th percentile or less in twin pregnancy (14 minutes), estimated blood loss was less than 1000 mL.
Conclusion Twin pregnancy is a known risk factor for postpartum hemorrhage. As the duration of the third stage prolongs, the risk for hemorrhage also increases. We recommend delivery of the placenta in twin pregnancies by 15 minutes to reduce this risk.
Key Points
Interdisciplinary Approach for the Medical Management of Gestational Gigantomastia
Authors: Cabrera Camila , Radolec Mackenzy , Prescott Angela , De La Cruz Carolyn , Beck Stacy 
Published: September 2020

Abstract
Background Gestational gigantomastia is a rare and debilitating condition that is thought to result from hormone hypersensitivity. Several definitions have been proposed using breast weight and change in body mass index, but the breast growth is best summarized as rapid, diffuse, and excessive.
Case We report a case of a 31-year-old woman with a history of infertility and cystic fibrosis that developed pathologic breast growth during hormonal preparation for in vitro fertilization. Her serum laboratories were unremarkable, and she was medically managed until 31 weeks of gestation. After delivery, she experienced rapid decrease in breast size and was followed by plastic surgery with plan to allow spontaneous regression with interval breast reduction
Conclusion We highlight a successful interdisciplinary medical management approach, which helped to avoid a morbid, intrapartum breast reduction.
Late Presentation of Uterine Rupture Following Vaginal Birth After Cesarean Delivery: A Case Report
Authors: Choi Lindsey A. , Chung Ariel A. , Pierce Brian 
Published: September 2020

Abstract
Background A trial of labor after cesarean delivery is associated with uterine rupture rates of 0.5 to 0.9%, which can have devastating neonatal and maternal consequences. While uterine rupture typically occurs during labor, it can clinically manifest after delivery.
Case A 23-year-old multiparous female presented in labor at term. Her obstetrical history was significant for a prior low transverse cesarean delivery. She had an uncomplicated labor course and spontaneous vaginal delivery.Immediately after delivery, she complained of severe right shoulder and left lower quadrant pain. Bedside ultrasound revealed a 10-cm, complex, adnexal mass adjacent to the uterus without free fluid. She was hemodynamically stable and appeared clinically well. On repeat ultrasound, the mass was unchanged; however, the patient now had free intraperitoneal fluid along the liver edge.Emergent laparotomy revealed a uterine rupture along her prior hysterotomy with extension into the right uterine artery. A 10-cm broad ligament hematoma ruptured posteriorly resulting in a 1-L hemoperitoneum. She received multiple blood products intraoperatively and recovered well postpartum.
Conclusion Delivery after trial of labor after cesarean delivery usually decreases acuity; however, these patients remain at risk for significant complications. Clinicians should continue to assess patients in the immediate postpartum period and proceed with surgical intervention if necessary.
COVID-19 Infection and Its Influence in Otorhinolaryngology-Head and Neck Surgery
Authors: Parilli-Troconis Daniella , Baptista Peter , Marcano-Lozada Marcel , Goncalves Stefania , Shahal David , Chiossone-Kerdel Juan Armando 
Published: September 2020

Abstract
Introduction The novel coronavirus disease 2019 pandemic has rapidly spread worldwide, challenging healthcare resources and communities to an unprecedent degree. Simultaneously, the amount of clinical and scientific information released has overwhelmed journal platforms.
Objectives This review aims to summarize the available diagnostic tools and current guidelines to safely assist patients while limiting the exposure of otolaryngologists during this pandemic.
Data Synthesis Key articles were retrieved from the following databases: PubMed, Lancet, Springer Nature, BioMed Central, JAMA network and MEDLINE, as well as updated documents from the Spanish Ministry of Health, World Health Organization, Centers for Disease Control and Prevention, Spanish Association of Surgeons, ENT-UK, American College of Surgeons, and American Academy of Otolaryngology-Head and Neck Surgery. The terms used for the search were: COVID-19, Test COVID, Surgery in COVID, 2019-nCoV, ‘coronavirus’, and SARS-CoV-2. A total of 10,245 papers were retrieved. The inclusion criteria for the review included: COVID-19 testing (n = 531), society guidelines for otolaryngology-head and neck surgery patient care in the outpatient clinic (n = 10) and surgical (n = 18) settings. Studies not related to COVID-19 diagnosis were excluded.
Conclusion Healthcare institutions around the world are outlining their own protocols regarding laboratory testing and personnel protective equipment usage based upon medical societies recommendations during the COVID-19 pandemic. We have summarized the available laboratory tests and their respective sensitivity and specificity. Moreover, clinical guidelines from different societies were reviewed and summarized to facilitate guidance for otolaryngologists in the operating room and in the clinical settings.
Noninvasive Self-monitoring of Blood Glucose at Your Fingertips, Literally!: Smartphone-Based Photoplethysmography
Authors: Mazzu-Nascimento Thiago , Leal Ângela Merice de Oliveira , Nogueira-de-Almeida Carlos Alberto , de Avó Lucimar Retto da Silva , Carrilho Emanuel , Silva Diego Furtado 
Published: September 2020

AbstractDiabetes is a chronic disease and one of the major public health problems worldwide. It is a multifactorial disease, caused by genetic factors and lifestyle habits. Brazil had ∼ 16.8 million individuals living with diabetes in 2019 and is expected to reach 26 million people by 2045. There are global increasing needs for the development of noninvasive diagnostic methods and use of mobile health, mainly in face of the pandemic caused by the coronavirus disease 2019 (COVID-19). For daily glycemic control, diabetic patients use a portable glucometer for glycemic self-monitoring and need to prick their fingertips three or more times a day, generating a huge discomfort throughout their lives. Our goal here is to present a review with very recent emerging studies in the field of noninvasive diagnosis and to emphasize that smartphone-based photoplethysmography (spPPG), powered by artificial intelligence, might be a trend to self-monitor blood glucose levels. In photoplethysmography, a light source travels through the tissue, interacts with the interstitium and with cells and molecules present in the blood. Reflection of light occurs as it passes through the biological tissues and a photodetector can capture these interactions. When using a smartphone, the built-in flashlight is a white light-emitting LED and the camera works as a photodetector. The higher the concentration of circulating glucose, the greater the absorbance and, consequently, the lesser the reflected light intensity will be. Due to these optical phenomena, the signal intensity captured will be inversely proportional to the blood glucose level. Furthermore, we highlight the microvascular changes in the progression of diabetes that can interfere in the signals captured by the photodetector using spPPG, due to the decrease of peripheral blood perfusion, which can be confused with high blood glucose levels. It is necessary to create strategies to filter or reduce the impact of these vascular changes in the blood glucose level analysis. Deep learning strategies can help the machine to solve these challenges, allowing an accurate blood glucose level and interstitial glucose prediction.
Giant Lipomas of the Hand. Our Experience
Authors: Balvís-Balvís Patricia , Yañez-Calvo Javier , Castro-Menéndez Manuel , Ferreirós-Conde María José 
Published: September 2020

Abstract
Objective Giant lipomas are benign tumors larger than 5 cm in size that are very uncommun in the hand, with the extant literature limited to case reports and small case series. The aim of the present study is to describe our experience with giant lipomas at the level of the hand, reviewing the most important aspects in relation to their diagnosis and treatment.
Material and Methods We present 6 patients treated in our service with giant lipomas of the hand between 2007 and 2015. Four cases only presented difficulty in grasping and mobilizing the hand due to the large size of the lipoma. Two cases were accompanied by a clinical feature of compression of the median nerve in relation to its location within the carpal tunnel.
Results All patients underwent surgery, and a complete excision of the lipoma was performed. The functional results have been satisfactory in all cases.
Conclusions Giant lipomas of the hand are infrequent tumors of slow growth, generally asymptomatic, although they can cause a compressive pathology due to the great size that they reach. Magnetic resonance imaging is an especially useful test to locate and accurately determine the size of the lesion in view of its surgical excision. After surgery, it is important to make a differential diagnosis with low-grade liposarcomas through an anatomopathological study, since both, macroscopically, have similar characteristics.
Wiedemann–Steiner Syndrome: A Rare Differential Diagnosis of Neurodevelopmental Delay and Dysmorphic Features
Authors: Carman Kursat Bora , Kaplan Emre , Aslan Cefa Nil , Kocagil Sinem , Cilinigr Oguz , Yarar Coskun 
Published: September 2020

AbstractWiedemann–Steiner syndrome (WSS) is a rare genetic disorder characterized by dysmorphic features, neurodevelopmental delay, growth retardation, and hypertrichosis cubiti. It is caused by pathogenic variants in the KMT2A gene. Here, we report a child with WSS presented with neurodevelopmental delay. Genetic analysis revealed a heterozygous c.2312dupC (p.Ser774Valfs*11) variant at the KMT2A gene that was classified as pathogenic in dbSNP (rs1057518649). To the best of our knowledge, this is the first patient of WSS from Turkey. This case draws attention to the diagnosis of WSS in children with neurodevelopmental delay.
Fetal Macrocephaly: A Novel Sonographic Finding in Congenital Myotonic Dystrophy
Authors: Shinar Shiri , Balakumar Parry , Shah Vibhuti , Chong Karen , Uster Tami , Chitayat David 
Published: September 2020

Abstract
Objective Sonographic clues to the diagnosis of congenital myotonic dystrophy (CDM) are limited, particularly in the absence of family history of myotonic dystrophy (DM). We reviewed cases of CDM for unique prenatal findings.
Study Design A single-center case series of fetuses with CMD with characteristic prenatal findings confirmed postnatally.
Results Four fetuses with pre- or postnatally diagnosed CDM presented with macrocephaly in utero. While head measurements were appropriate for gestational age until midgestation, third-trimester head circumference and biparietal diameter were both >2 standard deviation (SD) above the mean in all. Abdominal and femur measurements were otherwise appropriate for gestation. Postnatally, the occipitofrontal circumference was >2 SD above the mean in all, confirming the diagnosis of macrocephaly.
Conclusion CDM should be included in the differential diagnosis of third-trimester macrocephaly, especially in the presence of additional sonographic clues and when maternal medical history and physical examination are suggestive of DM.