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Nina Hediger, Markus A Landolt, Carmen Diez-Fernandez, Martina Huemer, Johannes Häberle
Neonatal onset hyperammonemia in patients with urea cycle disorders (UCDs) is still associated with high morbidity and mortality. Current protocols consistently recommend emergency medical and dietary management. In case of increasing or persistent hyperammonemia, with continuous or progressive neurological signs, dialysis is performed, mostly as ultima ratio. It is presently unknown whether the currently defined ammonia threshold (e.g., at 500 μmol/L) to start dialysis is useful to improve clinical outcome...
March 8, 2018: Journal of Inherited Metabolic Disease
Brita Sedlmayr, Jennifer Schöffler, Hans-Ulrich Prokosch, Martin Sedlmayr
BACKGROUND: The use of a nationwide medication plan has been promoted as an effective strategy to improve patient safety in Germany. However, the medication plan only exists as a paper-based version, which is related to several problems, that could be circumvented by an electronic alternative. OBJECTIVE: The main objective of this study was to report on the development of a mobile interface concept to support the management of medication information. METHODS: The human-centered design (UCD) process was chosen...
March 5, 2018: Informatics for Health & Social Care
Guido Giunti, Vasiliki Mylonopoulou, Octavio Rivera Romero
BACKGROUND: Multiple sclerosis (MS) is one of the world's most common neurologic disorders. Fatigue is one of most common symptoms that persons with MS experience, having significant impact on their quality of life and limiting their activity levels. Self-management strategies are used to support them in the care of their health. Mobile health (mHealth) solutions are a way to offer persons with chronic conditions tools to successfully manage their symptoms and problems. Gamification is a current trend among mHealth apps used to create engaging user experiences and is suggested to be effective for behavioral change...
March 2, 2018: JMIR MHealth and UHealth
Alexandra Nagy, Aparna Bhaduri, Nahid Shahmarvand, Jahanbanoo Shahryari, James L Zehnder, Roger A Warnke, Tariq Mughal, Siraj Ali, Robert S Ohgami
Castleman disease (CD) is a rare lymphoproliferative disorder subclassified as unicentric CD (UCD) or multicentric CD (MCD) based on clinical features and the distribution of enlarged lymph nodes with characteristic histopathology. MCD can be further subtyped based on human herpes virus 8 (HHV8) infection into HHV8-associated MCD, HHV8- /idiopathic MCD (iMCD), and polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin change (POEMS)-associated MCD. In a subset of cases of UCD, an associated follicular dendritic cell sarcoma (FDCS) may be seen...
March 13, 2018: Blood Advances
Sofoklis Mitsos, Alexandros Stamatopoulos, Davide Patrini, Robert S George, David R Lawrence, Nikolaos Panagiotopoulos
INTRODUCTION: Castleman's disease is a rare benign lymphoproliferative disorder of unknown etiology. The disease occurs in two clinical forms with different prognoses, treatments and symptoms: a unicentric form (UCD), which is solitary, localized, and a multicentric form characterized by generalized lymphadenopathy and systemic symptoms. This article aims to review the current literature to consolidate the evidence surrounding the curative potential of surgical treatment to the unicentric type...
2018: Advances in Respiratory Medicine
Lichun Meng, Andreas Fix, Martin Wirth, Lasse Høgstedt, Peter Tidemand-Lichtenberg, Christian Pedersen, Peter John Rodrigo
We demonstrate a robust, compact, portable and efficient upconversion detector (UCD) for a differential absorption lidar (DIAL) system designed for range-resolved methane (CH4 ) atmospheric sensing. The UCD is built on an intracavity pump system that mixes a 1064 nm pump laser with the lidar backscatter signal at 1646 nm in a 25-mm long periodically poled lithium niobate crystal. The upconverted signal at 646 nm is detected by a photomultiplier tube (PMT). The UCD with a noise equivalent power around 127 fW/Hz1/2 outperforms a conventional InGaAs based avalanche photodetector when both are used for DIAL measurements...
February 19, 2018: Optics Express
Ramachandiran Nandhagopal, Fathiya Al-Murshedi, Mujahid Al-Busaidi, Amna Al-Busaidi
Primary hyperammonemic encephalopathy due to urea cycle disorders (UCD) typically manifests with episodic unresponsiveness and this clinical entity is not often included in the differential diagnosis of presumed non-convulsive status epilepticus (NCSE). However, this diagnostic consideration has therapeutic implications. In this report, we document the therapeutic importance of elucidating the specific cause of hyperammonemic encephalopathy that closely mimicked NCSE through 2 unique illustrative cases.
January 2018: Neurosciences: the Official Journal of the Pan Arab Union of Neurological Sciences
Jin-Peng Jiang, Xiao-Fei Shen, Jun-Feng Du, Wen-Xian Guan
The aim of the present study was to share the experience of a single institute in the diagnosis, use of accessory examinations and treatment strategies of Castleman's disease (CD). The present study analyzed 34 patients (13 males and 21 females) with CD who were hospitalized between January 2006 and September 2014. The patients were divided into two groups based on the anatomical distribution of the disease: Unicentric CD (UCD) and multicentric CD (MCD). Histological data was obtained from lymph node biopsies...
February 2018: Oncology Letters
Guido Giunti, Jan Kool, Octavio Rivera Romero, Enrique Dorronzoro Zubiete
BACKGROUND: Multiple sclerosis (MS) is one of the world's most common neurologic disorders, with symptoms such as fatigue, cognitive problems, and issues with mobility. Evidence suggests that physical activity (PA) helps people with MS reduce fatigue and improve quality of life. The use of mobile technologies for health has grown in recent years with little involvement from relevant stakeholders. User-centered design (UCD) is a design philosophy with the goal of creating solutions specific to the needs and tasks of the intended users...
February 9, 2018: JMIR MHealth and UHealth
Ajanta Barh, Peter Tidemand-Lichtenberg, Christian Pedersen
Low noise detection with state-of-the-art mid-infrared (MIR) detectors (e.g., PbS, PbSe, InSb, HgCdTe) is a primary challenge owing to the intrinsic thermal background radiation of the low bandgap detector material itself. However, researchers have employed frequency upconversion based detectors (UCD), operable at room temperature, as a promising alternative to traditional direct detection schemes. UCD allows for the use of a low noise silicon-CCD/camera to improve the SNR. Using UCD, the noise contributions from the nonlinear material itself should be evaluated in order to estimate the limits of the noise-equivalent power of an UCD system...
February 5, 2018: Optics Express
Yael Wilnai, Yair J Blumenfeld, Kristina Cusmano, Susan R Hintz, Deborah Alcorn, William E Benitz, William E Berquist, Jonathan A Bernstein, Ricardo O Castillo, Waldo Concepcion, Tina M Cowan, Kenneth L Cox, Deirdre J Lyell, Carlos O Esquivel, Margaret Homeyer, Louanne Hudgins, Melissa Hurwitz, Jonathan P Palma, Susan Schelley, Vishnu Priya Akula, Marshall L Summar, Gregory M Enns
PURPOSE OF STUDY: Patients with neonatal urea cycle defects (UCDs) typically experience severe hyperammonemia during the first days of life, which results in serious neurological injury or death. Long-term prognosis despite optimal pharmacological and dietary therapy is still poor. The combination of intravenous sodium phenylacetate and sodium benzoate (Ammonul®) can eliminate nitrogen waste independent of the urea cycle. We report attempts to improve outcomes for males with severe ornithine transcarbamylase deficiency (OTCD), a severe X-linked condition, via prenatal intravenous administration of Ammonul and arginine to heterozygous carrier females of OTCD during labor...
March 2018: Molecular Genetics and Metabolism
Charic D Farinango, Juan S Benavides, Jesús D Cerón, Diego M López, Rosa E Álvarez
Background: Previous studies have demonstrated the effectiveness of information and communication technologies to support healthy lifestyle interventions. In particular, personal health record systems (PHR-Ss) empower self-care, essential to support lifestyle changes. Approaches such as the user-centered design (UCD), which is already a standard within the software industry (ISO 9241-210:2010), provide specifications and guidelines to guarantee user acceptance and quality of eHealth systems...
2018: Journal of Multidisciplinary Healthcare
Catia Cavicchi, Chiara Chilleri, Antonella Fioravanti, Lorenzo Ferri, Francesco Ripandelli, Cinzia Costa, Paolo Calabresi, Paolo Prontera, Francesca Pochiero, Elisabetta Pasquini, Silvia Funghini, Giancarlo la Marca, Maria Alice Donati, Amelia Morrone
N-acetylglutamate synthase deficiency (NAGSD) is an extremely rare urea cycle disorder (UCD) with few adult cases so far described. Diagnosis of late-onset presentations is difficult and delayed treatment may increase the risk of severe hyperammonemia. We describe a 52-year-old woman with recurrent headaches who experienced an acute onset of NAGSD. As very few papers focus on headaches in UCDs, we also report a literature review of types and pathophysiologic mechanisms of UCD-related headaches. In our case, headaches had been present since puberty (3-4 days a week) and were often accompanied by nausea, vomiting, or behavioural changes...
January 24, 2018: International Journal of Molecular Sciences
Claire Acevedo, Meghan Sylvia, Eric Schaible, James L Graham, Kimber L Stanhope, Lionel N Metz, Bernd Gludovatz, Ann V Schwartz, Robert O Ritchie, Tamara N Alliston, Peter J Havel, Aaron J Fields
Adults with type 2 diabetes (T2D) have a higher fracture risk for a given bone quantity, but the mechanisms remain unclear. Using a rat model of polygenic obese T2D, we demonstrate that diabetes significantly reduces whole-bone strength for a given bone mass (micro-CT-derived BMC), and we quantify the roles of T2D-induced deficits in material properties versus bone structure, i.e., geometry and microarchitecture. Lumbar vertebrae and ulnae were harvested from 6-month-old lean Sprague-Dawley rats, obese Sprague-Dawley rats, and diabetic obese UCD-T2DM rats (diabetic for 69 ± 7 days; blood glucose >200 mg/dl)...
January 17, 2018: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
Kane E Deering, Anna C Callan, Richard L Prince, Wai H Lim, Peter L Thompson, Joshua R Lewis, Andrea L Hinwood, Amanda Devine
BACKGROUND: Cadmium has been associated with increased risk of cardiovascular disease (CVD) in observational studies, however there has been a limited focus on this relationship in women. OBJECTIVES: This study investigated the association of urinary cadmium (UCd) concentrations with CVD outcomes and all-cause mortality in elderly Western Australian (WA) women. METHODS: UCd excretion was measured at baseline in 1359 women, mean age 75.2 ± 2...
December 26, 2017: International Journal of Hygiene and Environmental Health
Alvaro Sobrinho, Leandro Dias da Silva, Angelo Perkusich, Maria Eliete Pinheiro, Paulo Cunha
BACKGROUND: The chronic kidney disease (CKD) is a worldwide critical problem, especially in developing countries. CKD patients usually begin their treatment in advanced stages, which requires dialysis and kidney transplantation, and consequently, affects mortality rates. This issue is faced by a mobile health (mHealth) application (app) that aims to assist the early diagnosis and self-monitoring of the disease progression. METHODS: A user-centered design (UCD) approach involving health professionals (nurse and nephrologists) and target users guided the development process of the app between 2012 and 2016...
January 12, 2018: BMC Medical Informatics and Decision Making
Connie Hung, Casey Bronec, Eleonora Napoli, James Graham, Kimber L Stanhope, Ilaria Marsilio, Maria Cecilia Giron, Peter J Havel, Cecilia Giulivi
Ileal interposition (IT) surgery delays the onset of diabetes in a rat model of type-2 diabetes (UCD-T2DM). Here, to gain a deeper understanding of the molecular events underlying the effects of IT surgery, we examined the changes in the proteome of 4 white adipose depots (retroperitoneal, mesenteric, inguinal, and epididymal), and plasma free fatty acid profile in pre-diabetic rats 1.5 months following IT or sham surgery. The IT-mediated changes were exerted mainly in mesenteric fat, and spanned from delayed adipocyte maturation to a neuroendocrine remodeling...
January 10, 2018: Biochemical Journal
Na Ren, Lei Ding, Erna Jia, Jinru Xue
BACKGROUND: Our case describe a rare recurrence case of Unicentric Castleman's disease (UCD) with hyaline vascular type 14 years after surgery. CASE PRESENTATION: A 35-year-old Chinese female was admitted to hospital with one and half months history chest distress and chest pain. Patient reports a history of thoracic operation for mediastinal mass 14 years ago, and it was diagnosed UCD with hyaline vascular type after postoperative pathological examination. At this time, the imaging examination showed a mediastinal mass once again...
January 4, 2018: BMC Surgery
Shangxia Pan, Lifeng Lin, Fan Zeng, Jianpeng Zhang, Guanghui Dong, Boyi Yang, You Jing, Shejun Chen, Gan Zhang, Zhiqiang Yu, Guoying Sheng, Huimin Ma
Exposure to metal(loid)s can lead to adverse effects on nervous system in children. However, little is known about the possible interaction effects of simultaneous exposure to multiple metal(loid)s on children's intelligence. In addition, relationship between blood lead concentrations (<100 μg/L) and the intelligence of children over 5 years needs further epidemiological evidence. We recruited 530 children aged 9-11 years, including 266 living in a town near an industrialized area and 264 from another town in the same city in South China as a reference...
December 20, 2017: Environmental Pollution
Rodrigo Poubel Vieira de Rezende, Ronaldo Altenburg Gismondi, Haim Cesar Maleh, Elisa Mendes de Miranda Coelho, Carol Sartori Vieira, Maria Luiza Garcia Rosa, Luis Otavio Mocarzel
The objective of this study was to assess the mortality profile related to SSc in the state of Rio de Janeiro, Brazil. We retrospectively examined all registered deaths in the region (2006-2015 period) in which the diagnosis of SSc was mentioned on any line of the death certificates (underlying cause of death [UCD], n = 223; non-UCD, n = 151). Besides the analysis of gender, age, and the causes of death, we also compared the mortality from UCDs between individuals whose death causes included SSc (cases) and those whose death causes did not include SSc (deceased controls)...
December 16, 2017: Clinical Rheumatology
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