Stargardt Disease.

Abstract:

:Stargardt disease (STGD) is one of the most common macular dystrophies in young adults. It progresses slowly. Its prevalence is about 1:8000-10,000. Age of onset is a surrogate marker: The earlier the onset, the more severe the disease course. Onset usually occurs in childhood or early adolescence, at about 10-15 years of age. Vision is between about 20/70 and 20/200. The fundus shows a bull's eye pattern or beaten-bronze appearance, with or without yellowish flecks (fundus flavimaculatus). Fluorescein angiography may show dark choroid in about 80% of cases. On fundus autofluorescence (FAF), newer flecks appear hyperautofluorescent (hyperAF); older ones become progressively more hypoAF with time. Some flecks are surrounded by a ring of decreased AF. Peripapillary sparing is one the characteristics of Stargardt disease, but this area can be involved in about 2-7% of cases. The reason for this sparing is unclear; this area may be more resilient to the deleterious effect of ABCA4 gene mutation, and there might be a more favorable RPE photoreceptor ratio, resulting in less lipofuscin build-up, in the presence of a thicker overlying peripapillary retinal nerve fiber layer. Patients with Stargardt disease should avoid bright light and excessive vitamin A.

journal_name

Adv Exp Med Biol

authors

Tsang SH,Sharma T

doi

10.1007/978-3-319-95046-4_27

subject

Has Abstract

pub_date

2018-01-01 00:00:00

pages

139-151

eissn

0065-2598

issn

2214-8019

journal_volume

1085

pub_type

杂志文章,评审
  • Update on Comparative Biology of Elephants: Factors Affecting Reproduction, Health and Welfare.

    abstract::Asian (Elephas maximus) and African (Loxodonta africana) elephants serve as important keystone, umbrella and flagship species. Despite that, population numbers are declining, due mainly to poaching and habitat destruction. Understanding reproductive mechanisms is vital to effective management, particularly insurance p...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章,评审

    doi:10.1007/978-3-030-23633-5_9

    authors: Brown JL

    更新日期:2019-01-01 00:00:00

  • Heterogeneity of Human Mesenchymal Stromal/Stem Cells.

    abstract::Increasing evidence has shown that mesenchymal stem cells (MSCs) isolated from body tissues are heterogeneous while being examined in vitro and in vivo. Besides some common characteristics, MSCs derived from different tissues exhibit unique biological properties. In addition, the therapeutic effects of MSCs may vary w...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章,评审

    doi:10.1007/978-3-030-11096-3_10

    authors: Wang W,Han ZC

    更新日期:2019-01-01 00:00:00

  • Diagnosing Hepatorenal Tyrosinaemia in Europe: Newborn Mass Screening Versus Selective Screening.

    abstract::Hepatorenal tyrosinaemia (HT1) is a serious condition that used to be fatal before the advent of nitisinone (NTBC, Orfadine®) as a therapeutic option. We have recently shown that selective screening is inadequate as initial symptoms are often uncharacteristic which leads to a considerable delay in diagnosis and treatm...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章,评审

    doi:10.1007/978-3-319-55780-9_11

    authors: Das AM,Mayorandan S,Janzen N

    更新日期:2017-01-01 00:00:00

  • Acute Response to Cigarette Smoking Assessed in Exhaled Breath Condensate in Patients with Chronic Obstructive Pulmonary Disease and Healthy Smokers.

    abstract::The effect of acute exposure to cigarette smoke (CS) on the respiratory system has been less extensively studied than the long term effects of smoking. The aim of the present study was to evaluate the acute response to CS in smokers suffering from chronic obstructive pulmonary disease (COPD) and in healthy smokers. Ni...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章

    doi:10.1007/5584_2016_43

    authors: Maskey-Warzęchowska M,Nejman-Gryz P,Osinka K,Lis P,Malesa K,Górska K,Krenke R

    更新日期:2017-01-01 00:00:00

  • Kinins as mediators of human allergic reactions.

    abstract::We have demonstrated that kinins are generated following nasal challenge with allergen of allergic (5.6 +/- 0.17 ng/m-), but not nonallergic (0.04 +/- .02 ng/ml), individuals (n = 8 in each case). The presence of kinin was highly correlated with that of histamine and TAME-esterase activity and with clinical symptoms (...

    journal_title:Advances in experimental medicine and biology

    pub_type: 临床试验,杂志文章

    doi:10.1007/978-1-4757-0154-8_22

    authors: Proud D,Naclerio RM,Togias AG,Kagey-Sobotka A,Adkinson NF Jr,Norman PA,Lichtenstein LM

    更新日期:1986-01-01 00:00:00

  • Similarities and Differences in Psychology.

    abstract::Addiction is marked by repeating a certain behavior while ignoring the potential physical or mental consequences. Non-substance addiction provides an ideal model for researching the emergence and development of addiction's basic mechanism. Comparative studies of substance and non-substance addiction are helpful to rev...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章,评审

    doi:10.1007/978-981-10-5562-1_6

    authors: Chen Y,Sun Y,Ai SZ,Li JJ,Lu L,Shi J

    更新日期:2017-01-01 00:00:00

  • fMRI for monitoring dynamic changes in tissue oxygenation/blood flow: potential applications for tumor response to carbogen treatment.

    abstract::The ability to differentiate between well-oxygenated and poorly-oxygenated tumors may play an important role in selecting an optimal therapeutic regime for tumor treatment of the individual patient. We present preliminary results in the development of a dynamic functional MRI method for mapping tissue oxygenation and ...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章

    doi:10.1007/978-1-4615-0075-9_42

    authors: Zhong J,Kwok WC,Okunieff P

    更新日期:2003-01-01 00:00:00

  • Does health status influence acceptance of illness in patients with chronic respiratory diseases?

    abstract::The level of illness acceptance correlates positively with compliance to the doctor's recommendations, and negatively with the frequency and intensity of complications of chronic diseases. The purpose of this study was to determine the influence of the clinical condition on the level of illness acceptance, and to find...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章

    doi:10.1007/5584_2014_47

    authors: Kurpas D,Mroczek B,Brodowski J,Urban M,Nitsch-Osuch A

    更新日期:2015-01-01 00:00:00

  • Coronaviruses use discontinuous extension for synthesis of subgenome-length negative strands.

    abstract::We have developed a new model for coronavirus transcription, which we call discontinuous extension, to explain how subgenome-length negatives stands are derived directly from the genome. The current model called leader-primed transcription, which states that subgenomic mRNA is transcribed directly from genome-length n...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章,评审

    doi:10.1007/978-1-4615-1899-0_79

    authors: Sawicki SG,Sawicki DL

    更新日期:1995-01-01 00:00:00

  • Investigation of the Role of a Supplementation with Taurine on the Effects of Hypoglycemic-Hypotensive Therapy Against Diabetes-Induced Nephrotoxicity in Rats.

    abstract::This study has examined the role of supplementing a treatment of diabetic rats with captopril (CAP), metformin (MET) or CAP-MET with the antioxidant amino acid taurine (TAU) on biochemical indices of diabetes-induced metabolic changes, oxidative stress and nephropathy. To this end, groups of 6 male Sprague-Dawley rats...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章

    doi:10.1007/978-94-024-1079-2_32

    authors: Pandya K,Clark GJ,Lau-Cam CA

    更新日期:2017-01-01 00:00:00

  • Lung function at age 18-25 years: a comparison of different reference value systems.

    abstract::The anthropometrical data of the Caucasian population have significantly changed within the last five decades. The European Community for Coal and Steel (ECCS) assumes a plateau phase and recommends the entry of 25 years old for calculation of reference values in this age range. The question arises if the commonly use...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章

    doi:10.1007/978-94-007-4546-9_18

    authors: Marek E,Volke J,Smith HJ,Serbetci B,Kalhoff H,Kotschy-Lang N,Merget R,Marek W

    更新日期:2013-01-01 00:00:00

  • Host recognition by pathogenic fungi through plant flavonoids.

    abstract::A common characteristic among fungal pathogens of plants is that each specializes on a narrow range of specific plants as hosts. One adaptation to a specific host plant is the recognition of the host's chemicals which can be used to trigger genes or developmental pathways needed for pathogenesis. The production of cha...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章,评审

    doi:10.1007/978-1-4757-5235-9_2

    authors: Straney D,Khan R,Tan R,Bagga S

    更新日期:2002-01-01 00:00:00

  • Acute Myeloid Leukemia Stem Cell Heterogeneity and Its Clinical Relevance.

    abstract::The failure of complete remissions to reliably translate into cures in acute myeloid leukemia (AML) can be explained by the leukemia stem cell (LSC) paradigm, which hypothesizes that rare leukemia cells with stem cell features, including self-renewal capacity and drug resistance, are primarily responsible for both dis...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章,评审

    doi:10.1007/978-3-030-14366-4_9

    authors: Karantanos T,Jones RJ

    更新日期:2019-01-01 00:00:00

  • Fumonisin toxicity and metabolism studies at the USDA. Fumonisin toxicity and metabolism.

    abstract::Fumonisins are responsible for many of the toxic effects of the common corn fungus, Fusarium moniliforme. They are acute renal and liver toxins in rats, and have tumor promoting activity. Fumonisin B1 is poorly absorbed, rapidly excreted, and persists in small amounts in the liver and kidney. Fumonisins are specific i...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章,评审

    doi:10.1007/978-1-4899-1379-1_20

    authors: Norred WP,Voss KA,Riley RT,Plattner RD

    更新日期:1996-01-01 00:00:00

  • Immunotherapies in CLL.

    abstract::Chronic lymphocytic leukemia (CLL) is the most frequently diagnosed leukemia in the Western world, yet remains essentially incurable. Although initial chemotherapy response rates are high, patients invariably relapse and subsequently develop resistance to chemotherapy. For the moment, allogeneic hematopoietic stem cel...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章,评审

    doi:10.1007/978-1-4614-8051-8_11

    authors: Park JH,Brentjens RJ

    更新日期:2013-01-01 00:00:00

  • Stimulus-Secretion Coupling in Beta-Cells: From Basic to Bedside.

    abstract::Insulin secretion in humans is usually induced by mixed meals, which upon ingestion, increase the plasma concentration of glucose, fatty acids, amino acids, and incretins like glucagon-like peptide 1. Beta-cells can stay in the off-mode, ready-mode or on-mode; the mode-switching being determined by the open state prob...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章,评审

    doi:10.1007/978-3-030-12457-1_37

    authors: Islam MS

    更新日期:2020-01-01 00:00:00

  • A simple volume related model of arterial blood pressure generation.

    abstract::A single compartment model of the arterial circulation was used to generate an arterial blood pressure waveform from pre-determined stroke volume (SV) and arterial resistance (R). With fixed stroke volume and varying resistances blood pressure waveforms showed mean values proportional to resistance but amplitude lesse...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章

    doi:10.1007/978-0-387-74911-2_13

    authors: Wolff CB,Gooch BS,Douglas JS

    更新日期:2008-01-01 00:00:00

  • Dietary effects on gene expression in mammary tumorigenesis.

    abstract::Studies were undertaken to determine the effect a high fat diet has on the hormonally controlled transcription of the Mtv-1 locus in C3Hf mice. The expression of this locus in the initiating event in mammary tumor development in the C3Hf mouse. Mice were weaned at 21 days to either a high fat diet containing 23.5 perc...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章

    doi:10.1007/978-1-4899-0949-7_7

    authors: Etkind PR

    更新日期:1995-01-01 00:00:00

  • Is type 1 diabetes a disease of the gut immune system triggered by cow's milk insulin?

    abstract::The role of the gut immune system in the development of autoimmune type 1 diabetes is evaluated in this review with special emphasis in the hypothesis suggesting that dietary cow's milk insulin could trigger beta-cell autoimmunity when the mechanisms of oral tolerance are disturbed. ...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章,评审

    doi:10.1007/1-4020-3535-7_22

    authors: Vaarala O

    更新日期:2005-01-01 00:00:00

  • EEG Analysis of the Neurofeedback Training Effect in Algorithmic Thinking.

    abstract::Although significant advances have been made in understanding several cognitive states, the algorithmic thinking ability is yet to be analyzed in terms of neuroscience and brain imaging techniques. Studies on the effects of neurofeedback on learning disabilities especially mathematics disorders are limited. The object...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章

    doi:10.1007/978-3-319-56246-9_26

    authors: Plerou A,Vlamos P,Margetaki A

    更新日期:2017-01-01 00:00:00

  • Regulatory T Cells: Concept, Classification, Phenotype, and Biological Characteristics.

    abstract::Regulatory T cells (Treg) play an indispensable role in maintaining the body's immune nonresponse to self-antigens and suppressing the body's unwarranted and potentially harmful immune responses. Their absence, reduction, dysfunction, transformation, and instability can lead to numerous autoimmune diseases. There are ...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章

    doi:10.1007/978-981-15-6407-9_1

    authors: Du Y,Fang Q,Zheng SG

    更新日期:2021-01-01 00:00:00

  • Skeletal muscle and glioma oxygenation by carbogen inhalation in rats: a longitudinal study by EPR oximetry using single-probe implantable oxygen sensors.

    abstract::The feasibility of EPR oximetry using a single-probe implantable oxygen sensor (ImOS) was tested for repeated measurement of pO₂ in skeletal muscle and ectopic 9L tumors in rats. The ImOS (50 mm length) were constructed using nickel-chromium alloy wires, with lithium phthalocyanine (LiPc, oximetry probe) crystals load...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章

    doi:10.1007/978-1-4939-0620-8_13

    authors: Hou H,Khan N,Lariviere J,Hodge S,Chen EY,Jarvis LA,Eastman A,Williams BB,Kuppusamy P,Swartz HM

    更新日期:2014-01-01 00:00:00

  • Exercise and Organ Cross Talk.

    abstract::Chronic heart failure, diabetes, depression, and other chronic diseases are associated with high mortality rate and low cure rate. Exercise induces muscle contraction and secretes multiple myokines, which affects the signaling pathways in skeletal muscle tissues and regulate remote organ functions. Exercise is known t...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章,评审

    doi:10.1007/978-981-15-1792-1_4

    authors: Fan Z,Xu M

    更新日期:2020-01-01 00:00:00

  • Regulation of proteinase inhibitor genes in food plants.

    abstract::Proteinase inhibitor proteins in the families Solanaceae, Leguminosae and Graminae are stored in seeds and tubers and are also found to accumulate in leaves in response to pest attacks. The isolation of the proteinase Inhibitor I cDNA and its gene from tomato has provided information concerning proteinase inhibitor sy...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章

    doi:10.1007/978-1-4757-0022-0_17

    authors: Brown WE,Graham JS,Lee JS,Ryan CA

    更新日期:1986-01-01 00:00:00

  • Roles of Slit Ligands and Their Roundabout (Robo) Family of Receptors in Bone Remodeling.

    abstract::Slit guidance ligands (Slits) and their roundabout (Robo) family of receptors are well-known axon guidance molecules that were originally identified in Drosophila mutants with commissural axon pathfinding defects. However, Slit-Robo signaling has been shown to be involved in not only neurogenesis, but also the develop...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章

    doi:10.1007/5584_2020_586

    authors: Niimi T

    更新日期:2020-09-29 00:00:00

  • Characterizing multi-omic data in systems biology.

    abstract::In today's biology, studies have shifted to analyzing systems over discrete biochemical reactions and pathways. These studies depend on combining the results from scores of experimental methods that analyze DNA; mRNA; noncoding RNAs, DNA, RNA, and protein interactions; and the nucleotide modifications that form the ep...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章,评审

    doi:10.1007/978-1-4614-8778-4_2

    authors: Mason CE,Porter SG,Smith TM

    更新日期:2014-01-01 00:00:00

  • Developing Well-Annotated Species-Specific Protein Databases Using Comparative Proteogenomics.

    abstract::Proteomics is a mass spectrometry-based discipline that aims to analyze proteomes and their functions. Many proteomic studies require well-developed protein databases for reference. However, most proteomes are not well-annotated, aside from model organisms. Techniques like six-frame translation, ab initio gene predict...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章,评审

    doi:10.1007/978-3-030-15950-4_22

    authors: Dupree EJ,Crimmins BS,Holsen TM,Darie CC

    更新日期:2019-01-01 00:00:00

  • Photoactivated Adenylyl Cyclases: Fundamental Properties and Applications.

    abstract::Photoactivated adenylyl cyclase (PAC) was first discovered to be a sensor for photoavoidance in the flagellate Euglena gracilis. PAC is a flavoprotein that catalyzes the production of cAMP upon illumination with blue light, which enables us to optogenetically manipulate intracellular cAMP levels in various biological ...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章

    doi:10.1007/978-981-15-8763-4_7

    authors: Iseki M,Park SY

    更新日期:2021-01-01 00:00:00

  • Functional Analysis of Circular RNAs.

    abstract::Circular RNAs characterize a class of widespread and diverse endogenous RNAs which are non-coding RNAs that are made by back-splicing events and have covalently closed loops with no polyadenylated tails. Various indications specify that circular RNAs (circRNAs) are plentiful in the human transcriptome. However, their ...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章,评审

    doi:10.1007/978-981-13-1426-1_8

    authors: Shanmugapriya,Huda HA,Vijayarathna S,Oon CE,Chen Y,Kanwar JR,Ng ML,Sasidharan S

    更新日期:2018-01-01 00:00:00

  • Notch and Stem Cells.

    abstract::The Notch pathway is crucial in the regulation of stem cells biology. Notch-mediated signalling controls several aspects of tissue homeostasis in both embryonic and adult tissues, balancing stem cells maintenance and differentiation. Although the major elements of the pathway are well conserved throughout evolution, i...

    journal_title:Advances in experimental medicine and biology

    pub_type: 杂志文章,评审

    doi:10.1007/978-3-319-89512-3_12

    authors: Bigas A,Porcheri C

    更新日期:2018-01-01 00:00:00