Childhood Disintegrative Disorder.

ERIC Educational Resources Information Center

Malhotra, Savita; Gupta, Nitin

1999-01-01

This article reviews what is known about childhood distintegrative disorder (CDD), a clinical syndrome characterized by disintegration of mental functions and regression of acquired language and intellectual functions after a period (usually 3-4 years) of normal development. It reviews the condition's epidemiology, onset and progression,…

Acquired Aplastic Anemia: What Have We Learned and What Is in the Horizon?

PubMed

Savaşan, Süreyya

2018-06-01

Acquired aplastic anemia (aAA) characterized by peripheral pancytopenia and bone marrow aplasia is a rare and serious disorder. Differential diagnosis includes constitutional bone marrow failure syndromes and myelodysplastic disorders. Autoimmune reaction to altered hematopoietic stem cells highlights the underlying mechanism. Matched related donor allogeneic hematopoietic stem cell transplantation is the ideal pediatric treatment; alternative approaches include immunosuppressive therapy and use of eltrombopag. Progression to clonal disorders can occur. Recently, alternative donor hematopoietic stem cell transplantation outcomes have significantly improved. Despite advances, aAA continues to be a challenge for hematologists. Copyright © 2018 Elsevier Inc. All rights reserved.

Role of Radiologic Imaging in Genetic and Acquired Neuromuscular Disorders.

PubMed

Ortolan, Paolo; Zanato, Riccardo; Coran, Alessandro; Beltrame, Valeria; Stramare, Roberto

2015-03-11

Great technologic and clinical progress have been made in the last two decades in identifying genetic defects of several neuromuscular diseases, as Spinal Muscular Atrophy, genetic muscular dystrophies and other genetic myopathies. The diagnosis is usually challenging, due to great variability in genetic abnormalities and clinical phenotypes and the poor specificity of complementary analyses, i.e., serum creatine kinase (CK) and electrophysiology. Muscle biopsy represents the gold standard for the diagnosis of genetic neuromuscular diseases, but clinical imaging of muscle tissue is an important diagnostic tool to identify and quantifyies muscle damage. Radiologic imaging is, indeed, increasingly used as a diagnostic tool to describe patterns and the extent of muscle involvement, thanks to modern techniques that enable to definethe definition of degrees of muscle atrophy and changes in connective tissue. They usually grade the severity of the disease process with greater accuracy than clinical scores. Clinical imaging is more than complementary to perform muscle biopsy, especially as ultrasound scans are often mandatory to identify the muscle to be biopsied. We will here detail and provideWe will herein provide detailed examples of the radiologic methods that can be used in genetic and acquired neuromuscular disorders, stressing pros and cons. Muscle Imaging, MRI, CT, genetic muscle disorders, myopathies, dystrophies.

Role of Radiologic Imaging in Genetic and Acquired Neuromuscular Disorders

PubMed Central

Zanato, Riccardo; Coran, Alessandro; Beltrame, Valeria; Stramare, Roberto

2015-01-01

Great technologic and clinical progress have been made in the last two decades in identifying genetic defects of several neuromuscular diseases, as Spinal Muscular Atrophy, genetic muscular dystrophies and other genetic myopathies. The diagnosis is usually challenging, due to great variability in genetic abnormalities and clinical phenotypes and the poor specificity of complementary analyses, i.e., serum creatine kinase (CK) and electrophysiology. Muscle biopsy represents the gold standard for the diagnosis of genetic neuromuscular diseases, but clinical imaging of muscle tissue is an important diagnostic tool to identify and quantifyies muscle damage. Radiologic imaging is, indeed, increasingly used as a diagnostic tool to describe patterns and the extent of muscle involvement, thanks to modern techniques that enable to definethe definition of degrees of muscle atrophy and changes in connective tissue. They usually grade the severity of the disease process with greater accuracy than clinical scores. Clinical imaging is more than complementary to perform muscle biopsy, especially as ultrasound scans are often mandatory to identify the muscle to be biopsied. We will here detail and provideWe will herein provide detailed examples of the radiologic methods that can be used in genetic and acquired neuromuscular disorders, stressing pros and cons. Key Words: Muscle Imaging, MRI, CT, genetic muscle disorders, myopathies, dystrophies PMID:26913153

Microbe-Induced Inflammatory Signals Triggering Acquired Bone Marrow Failure Syndromes.

PubMed

Espinoza, J Luis; Kotecha, Ritesh; Nakao, Shinji

2017-01-01

Acquired bone marrow failure syndromes encompass a unique set of disorders characterized by a reduction in the effective production of mature cells by the bone marrow (BM). In the majority of cases, these syndromes are the result of the immune-mediated destruction of hematopoietic stem cells or their progenitors at various stages of differentiation. Microbial infection has also been associated with hematopoietic stem cell injury and may lead to associated transient or persistent BM failure, and recent evidence has highlighted the potential impact of commensal microbes and their metabolites on hematopoiesis. We summarize the interactions between microorganisms and the host immune system and emphasize how they may impact the development of acquired BM failure.

Microbe-Induced Inflammatory Signals Triggering Acquired Bone Marrow Failure Syndromes

PubMed Central

Espinoza, J. Luis; Kotecha, Ritesh; Nakao, Shinji

2017-01-01

Acquired bone marrow failure syndromes encompass a unique set of disorders characterized by a reduction in the effective production of mature cells by the bone marrow (BM). In the majority of cases, these syndromes are the result of the immune-mediated destruction of hematopoietic stem cells or their progenitors at various stages of differentiation. Microbial infection has also been associated with hematopoietic stem cell injury and may lead to associated transient or persistent BM failure, and recent evidence has highlighted the potential impact of commensal microbes and their metabolites on hematopoiesis. We summarize the interactions between microorganisms and the host immune system and emphasize how they may impact the development of acquired BM failure. PMID:28286502

Presentation of an acquired urea cycle disorder post liver transplantation.

PubMed

Ghabril, Marwan; Nguyen, Justin; Kramer, David; Genco, Trina; Mai, Martin; Rosser, Barry G

2007-12-01

The liver's role as the largest organ of metabolism and the unique and often critical function of liver-specific enzyme pathways imply a greater risk to the recipient of acquiring a donor metabolic disease with liver transplants versus other solid organ transplants. With clinical consequences rarely reported, the frequency of solid organ transplant transfer of metabolic disease is not known. Ornithine transcarbamylase deficiency (OTCD), although rare, is the most common of the urea cycle disorders (UCDs). Because of phenotypic heterogeneity, OTCD may go undiagnosed into adulthood. With over 5000 liver transplant procedures annually in the United States, the likelihood of unknowingly transmitting OTCD through liver transplantation is very low. We describe the clinical course of a liver transplant recipient presenting with acute hyperammonemia and encephalopathy after receiving a liver graft form a donor with unrecognized OTCD. Copyright (c) 2007 AASLD.

"… Trial and error …": Speech-language pathologists' perspectives of working with Indigenous Australian adults with acquired communication disorders.

PubMed

Cochrane, Frances Clare; Brown, Louise; Siyambalapitiya, Samantha; Plant, Christopher

2016-10-01

This study explored speech-language pathologists' (SLPs) perspectives about factors that influence clinical management of Aboriginal and Torres Strait Islander adults with acquired communication disorders (e.g. aphasia, motor speech disorders). Using a qualitative phenomenological approach, seven SLPs working in North Queensland, Australia with experience working with this population participated in semi-structured in-depth interviews. Qualitative content analysis was used to identify categories and overarching themes within the data. Four categories, in relation to barriers and facilitators, were identified from participants' responses: (1) The Practice Context; (2) Working Together; (3) Client Factors; and (4) Speech-Language Pathologist Factors. Three overarching themes were also found to influence effective speech pathology services: (1) Aboriginal and Torres Strait Islander Cultural Practices; (2) Information and Communication; and (3) Time. This study identified many complex and inter-related factors which influenced SLPs' effective clinical management of this caseload. The findings suggest that SLPs should employ a flexible, holistic and collaborative approach in order to facilitate effective clinical management with Aboriginal and Torres Strait Islander people with acquired communication disorders.

The inhibition of acquired fear.

PubMed

Izquierdo, Iván; Cammarota, Martín; Vianna, Mónica M R; Bevilaqua, Lía R M

2004-01-01

A conditioned stimulus (CS) associated with a fearsome unconditioned stimulus (US) generates learned fear. Acquired fear is at the root of a variety of mental disorders, among which phobias, generalized anxiety, the posttraumatic stress disorder (PTSD) and some forms of depression. The simplest way to inhibit learned fear is to extinguish it, which is usually done by repeatedly presenting the CS alone, so that a new association, CS-"no US", will eventually overcome the previously acquired CS-US association. Extinction was first described by Pavlov as a form of "internal inhibition" and was recommended by Freud and Ferenczi in the 1920s (who called it "habituation") as the treatment of choice for phobic disorders. It is used with success till this day, often in association with anxiolytic drugs. Extinction has since then been applied, also successfully and also often in association with anxiolytics, to the treatment of panic, generalized anxiety disorders and, more recently, PTSD. Extinction of learned fear involves gene expression, protein synthesis, N-methyl-D-aspartate (NMDA) receptors and signaling pathways in the hippocampus and the amygdala at the time of the first CS-no US association. It can be enhanced by increasing the exposure to the "no US" component at the time of behavioral testing, to the point of causing the complete uninstallment of the original fear response. Some theorists have recently proposed that reiteration of the CS alone may induce a reconsolidation of the learned behavior instead of its extinction. Reconsolidation would preserve the original memory from the labilization induced by its retrieval. If true, this would of course be disastrous for the psychotherapy of fear-motivated disorders. Here we show that neither the CS nor retrieval cause anything remotely like reconsolidation, but just extinction. In fact, our findings indicate that the reconsolidation hypothesis is essentially incorrect, at least for the form of contextual fear most

Brief Report: Childhood Disintegrative Disorder as a Likely Manifestation of Vitamin B12 Deficiency

ERIC Educational Resources Information Center

Malhotra, Savita; Subodh, B. N.; Parakh, Preeti; Lahariya, Sanjay

2013-01-01

Childhood disintegrative disorder is a rare disorder, characterized by regression of acquired skills after a period of normal development. The case of childhood disintegrative disorder presented here was found to have vitamin B12 deficiency and hyperhomocysteinemia on extensive evaluation to find a probable cause for regression. This case…

Post-traumatic seizure disorder following acquired brain injury.

PubMed

Teasell, Robert; Bayona, Nestor; Lippert, Corbin; Villamere, James; Hellings, Chelsea

2007-02-01

The present study aimed to evaluate the effectiveness of prophylactic anticonvulsant pharmacological strategies for the prevention of seizure disorders following acquired brain injury (ABI) to provide guidance for clinical practice based on the best available evidence. A systematic review of the literature from 1980-2005 was conducted focusing on treatment interventions available for post-traumatic seizures following ABI. The evidence for the efficacy of a given intervention was ranked as strong (supported by at least two randomized controlled trials (RCTs), moderate (supported by a single RCT), or limited (supported by other types of studies in the absence of RCTs). Based on a previous meta-analysis and the findings of this review, there is strong evidence that prophylactic anticonvulsant therapy decreases the occurrence of early seizures but only within the first week post-injury. Moreover, the evidence indicates that prophylactic anticonvulsant therapy does not decrease the incidence of seizure onset more than one week post-injury. In children, there is moderate evidence that prophylactic phenytoin does not reduce the incidence of early or late seizures. The efficacy of anticonvulsants after the development of seizures has not been specifically studied in ABI. Prophylactic anti-convulsants are effective in reducing seizures in the first week post-injury in adults. However, they do not reduce the occurrence of seizures after the first week.

Regression in autistic spectrum disorders.

PubMed

Stefanatos, Gerry A

2008-12-01

A significant proportion of children diagnosed with Autistic Spectrum Disorder experience a developmental regression characterized by a loss of previously-acquired skills. This may involve a loss of speech or social responsitivity, but often entails both. This paper critically reviews the phenomena of regression in autistic spectrum disorders, highlighting the characteristics of regression, age of onset, temporal course, and long-term outcome. Important considerations for diagnosis are discussed and multiple etiological factors currently hypothesized to underlie the phenomenon are reviewed. It is argued that regressive autistic spectrum disorders can be conceptualized on a spectrum with other regressive disorders that may share common pathophysiological features. The implications of this viewpoint are discussed.

Comparison of a therapeutic-only versus prophylactic platelet transfusion policy for people with congenital or acquired bone marrow failure disorders

PubMed Central

Ashraf, Asma; Hadjinicolaou, Andreas V; Doree, Carolyn; Hopewell, Sally; Trivella, Marialena; Estcourt, Lise J

2016-01-01

This is the protocol for a review and there is no abstract. The objectives are as follows: To compare a therapeutic-only versus prophylactic platelet transfusion policy for people with myelodysplasia, inherited or acquired aplastic anaemia, and other congenital bone marrow failure disorders. PMID:27660553

[Acquired perforating dermatosis in the patient with chronic kidney disease – case report and literature review].

PubMed

Steć, Anna; Paluch-Oleś, Jolanta; Korolczuk, Agnieszka; Magdalena Grzebalska, Agnieszka; Kozioł-Montewka, Maria; Książek, Andrzej

Acquired perforating dermatosis (APD) represents a heterogenous group of skin disorders characterized histopathologically by transepithelial elimination (TEE) of dermal structures. APD is manifested clinically as multi-localized, papulo-nodular skin lesions accompanied by a refractory pruritus. APD typically coexists with long-term disorders, most often diabetic kidney disease (DKD). The paper presents a case of a 56-year-old male patient with chronic kidney disease (CKD) and concomitant acquired reactive perforating collagenosis (ARPC), which is a subtype of APD. Etiological theories of ARPC as well as current diagnostic and treatment principles in dermatosis were described. On the basis of the presented case report and the literature review attention was paid to diagnostic difficulties associated with APD. The assumption was made that APD can be an underdiagnosed disease and thus it is not treated correctly. According to the authors’ opinion, this is an important circumstance to popularize the knowledge about APD.

Associative Learning Through Acquired Salience.

PubMed

Treviño, Mario

2015-01-01

Most associative learning studies describe the salience of stimuli as a fixed learning-rate parameter. Presumptive saliency signals, however, have also been linked to motivational and attentional processes. An interesting possibility, therefore, is that discriminative stimuli could also acquire salience as they become powerful predictors of outcomes. To explore this idea, we first characterized and extracted the learning curves from mice trained with discriminative images offering varying degrees of structural similarity. Next, we fitted a linear model of associative learning coupled to a series of mathematical representations for stimulus salience. We found that the best prediction, from the set of tested models, was one in which the visual salience depended on stimulus similarity and a non-linear function of the associative strength. Therefore, these analytic results support the idea that the net salience of a stimulus depends both on the items' effective salience and the motivational state of the subject that learns about it. Moreover, this dual salience model can explain why learning about a stimulus not only depends on the effective salience during acquisition but also on the specific learning trajectory that was used to reach this state. Our mathematical description could be instrumental for understanding aberrant salience acquisition under stressful situations and in neuropsychiatric disorders like schizophrenia, obsessive-compulsive disorder, and addiction.

Comorbidity between obsessive-compulsive disorder and body dysmorphic disorder: prevalence, explanatory theories, and clinical characterization

PubMed Central

Frías, Álvaro; Palma, Carol; Farriols, Núria; González, Laura

2015-01-01

Background With the advent of the fifth edition of Diagnostic and Statistical Manual of Mental Disorders, body dysmorphic disorder (BDD) has been subsumed into the obsessive-compulsive disorders and related disorders (OCDRD) category. Objective We aimed to determine the empirical evidence regarding the potential relationship between BDD and obsessive-compulsive disorder (OCD) based on the prevalence data, etiopathogenic pathways, and clinical characterization of patients with both disorders. Method A comprehensive search of databases (PubMed and PsycINFO) was performed. Published manuscripts between 1985 and May 2015 were identified. Overall, 53 studies fulfilled inclusion criteria. Results Lifetime comorbidity rates of BDD–OCD are almost three times higher in samples with a primary diagnosis of BDD than those with primary OCD (27.5% vs 10.4%). However, other mental disorders, such as social phobia or major mood depression, are more likely among both types of psychiatric samples. Empirical evidence regarding the etiopathogenic pathways for BDD–OCD comorbidity is still inconclusive, whether concerning common shared features or one disorder as a risk factor for the other. Specifically, current findings concerning third variables show more divergences than similarities when comparing both disorders. Preliminary data on the clinical characterization of the patients with BDD and OCD indicate that the deleterious clinical impact of BDD in OCD patients is greater than vice versa. Conclusion Despite the recent inclusion of BDD within the OCDRD, data from comparative studies between BDD and OCD need further evidence for supporting this nosological approach. To better define this issue, comparative studies between BDD, OCD, and social phobia should be carried out. PMID:26345330

Aetiological profile of acquired anaemia in a paediatric tertiary care setting.

PubMed

Bibi, Saima; Gilani, Syed Yasir Hussain; Shah, Syed Raza Ali; Bibi, Shawana

2011-01-01

Anaemia is the commonest haematological disorder frequently faced by clinicians worldwide. The multi-factorial aetiology of the disorder warrants a comprehensive search for the different causes as management plans differ for different disorders. The objective of this study was to identify the different acquired causes of anaemia in our paediatric population. The study was conducted at the Department of Paediatrics, Ayub Teaching Hospital from April 2009 to April 2010. It was a cross-sectional study. A total of 110 patients were included in the study who presented with anaemia secondary to acquired aetiologies and were assessed clinically using general physical and systemic examination. The salient clinical and laboratory data was retrieved in designed protocol. Out of a total of 110 patients, 61 (55.5%) were male and 49 (44.5%) were female. Mean age of the participants was 48 months. Nutritional anaemia comprising iron deficiency anaemia and megaloblastic anaemia was the leading cause being present in 49 (44.5%) patients followed by Visceral Leishmaniasis in 28 (25.5%) patients. Mean haemoglobin was 4.36 g/dl. Anaemia secondary to acquired causes is a disorder with grave consequences ranging from cognitive and psychomotor dysfunction to mortality in severe cases. Identification of the different acquired causes is important in preventing the disorder by guiding appropriate interventions.

Multiple myeloma associated with acquired cutis laxa.

PubMed

Cho, S Y; Maguire, R F

1980-08-01

Acquired cutis laxa is a rare disorder characterized by diffuse laxity of the skin and loss of connective tissue support with involvement of the lungs, gastrointestinal tract, pelvic organs, and aorta. The case report presented herein describes a forty-six year old woman with multiple myeloma and cutis laxa. Her history included several severe allergic reactions and the gradual development of lax skin, loss of connective tissue support throughout the body, and emphysema. At autopsy, multiple myeloma, diffuse laxity of the skin, and panacinar emphysema were found. The amount of elastic fiber in the skin, lungs, and aorta was decreased and showed abnormal fragmentation. Results of direct immunofluorescence study demonstrated IgG bound to dermal elastic fibers. Speculation regarding an immunologic etiology of the elastic tissue abnormality is presented herein.

An update on acquired nystagmus.

PubMed

Rucker, Janet C

2008-01-01

Proper evaluation and treatment of acquired nystagmus requires accurate characterization of nystagmus type and visual effects. This review addresses important historical and examination features of nystagmus and current concepts of pathogenesis and treatment of gaze-evoked nystagmus, nystagmus due to vision loss, acquired pendular nystagmus, peripheral and central vestibular nystagmus, and periodic alternating nystagmus.

Incidence and Long-term Outcomes of the HIV-Neuroretinal Disorder in Patients with the Acquired Immunodeficiency Syndrome

PubMed Central

Jabs, Douglas A.; Drye, Lea; Van Natta, Mark L.; Thorne, Jennifer E.; Holland, Gary N.

2014-01-01

Objectives Patients with the acquired immunodeficiency syndrome (AIDS) have an abnormality of retina/optic nerve function, manifested as decreased contrast sensitivity (in the absence of ocular opportunistic infections or media opacity), abnormalities on automated perimetry, and loss of retinal nerve fiber layer, even among those with good visual acuity, termed the HIV-neuroretinal disorder. The objectives of this study were to determine the prevalence, incidence, risk factors for, and outcomes of HIV-neuroretinal disorder. Design Prospective cohort study Participants 1822 patients with AIDS without ocular infections or media opacities. Methods Patients with HIV-neuroretinal disorder were identified by a contrast sensitivity < 1.50 log units in either eye in the absence of ocular opportunistic infections or media opacity. Main outcome measures Incidence of HIV-neuroretinal disorder, mortality, visual impairment (visual acuity 20/50 or worse), and blindness (20/200 or worse) on logarithmic visual acuity charts. Results Sixteen percent of participants had HIV-neuroretinal disorder at enrollment. The estimated cumulative incidence by 20 years after AIDS diagnosis was 51% (95% confidence interval [CI] 46%–55%). HIV-neuroretinal disorder was more common in women and African American persons. Risk factors for it included hepatitis C infection, low CD4+ T cells, and detectable HIV RNA in the blood. Patients with HIV neuroretinal disorder had a 70% excess mortality vs. those without it, even after adjusting for CD4+ T cells and HIV load (hazard ratio=1.7, 95% CI= 1.3–2.1, P<0.0001). Patients with HIV-neuroretinal disorder had increased risks of bilateral visual impairment (hazard ratio=6.5, 95% CI=2.6–10.6, P<0.0001) and blindness (hazard ratio=5.9, 95% CI=2.8–13.7, P=0.01) vs. those without HIV neuroretinal disorder. Conclusions HIV-neuroretinal disorder is a common finding among patients with AIDS, and it is associated with an increased mortality and an increased

Associative Learning Through Acquired Salience

PubMed Central

Treviño, Mario

2016-01-01

Most associative learning studies describe the salience of stimuli as a fixed learning-rate parameter. Presumptive saliency signals, however, have also been linked to motivational and attentional processes. An interesting possibility, therefore, is that discriminative stimuli could also acquire salience as they become powerful predictors of outcomes. To explore this idea, we first characterized and extracted the learning curves from mice trained with discriminative images offering varying degrees of structural similarity. Next, we fitted a linear model of associative learning coupled to a series of mathematical representations for stimulus salience. We found that the best prediction, from the set of tested models, was one in which the visual salience depended on stimulus similarity and a non-linear function of the associative strength. Therefore, these analytic results support the idea that the net salience of a stimulus depends both on the items' effective salience and the motivational state of the subject that learns about it. Moreover, this dual salience model can explain why learning about a stimulus not only depends on the effective salience during acquisition but also on the specific learning trajectory that was used to reach this state. Our mathematical description could be instrumental for understanding aberrant salience acquisition under stressful situations and in neuropsychiatric disorders like schizophrenia, obsessive-compulsive disorder, and addiction. PMID:26793078

Acquired and congenital disorders of sung performance: A review.

PubMed Central

Berkowska, Magdalena; Dalla Bella, Simone

2009-01-01

Many believe that the majority of people are unable to carry a tune. Yet, this widespread idea underestimates the singing abilities of the layman. Most occasional singers can sing in tune and in time, provided that they perform at a slow tempo. Here we characterize proficient singing in the general population and identify its neuronal underpinnings by reviewing behavioral and neuroimaging studies. In addition, poor singing resulting from a brain injury or neurogenetic disorder (i.e., tone deafness or congenital amusia) is examined. Different lines of evidence converge in indicating that poor singing is not a monolithic deficit. A variety of poor-singing "phenotypes" are described, with or without concurrent perceptual deficits. In addition, particular attention is paid to the dissociations between specific abilities in poor singers (e.g., production of absolute vs. relative pitch, pitch vs. time accuracy). Such diversity of impairments in poor singers can be traced to different faulty mechanisms within the vocal sensorimotor loop, such as pitch perception and sensorimotor integration. PMID:20523851

Predictors of Outcome following Acquired Brain Injury in Children

ERIC Educational Resources Information Center

Johnson, Abigail R.; DeMatt, Ellen; Salorio, Cynthia F.

2009-01-01

Acquired brain injury (ABI) in children and adolescents can result from multiple causes, including trauma, central nervous system infections, noninfectious disorders (epilepsy, hypoxia/ischemia, genetic/metabolic disorders), tumors, and vascular abnormalities. Prediction of outcomes is important, to target interventions, allocate resources,…

Disorders of Platelet Function

PubMed Central

Huebsch, Lothar B.; Harker, Laurence A.

1981-01-01

Platelets play an important role in hemostasis, and alterations in platelet function may be the cause of abnormal bleeding in a wide variety of congenital and acquired clinical disorders. Platelet dysfunction may be classified as disorders of (1) substrate connective tissue, (2) adhesion, (3) aggregation and (4) platelet-release reaction. The congenital defects of platelet function, although uncommon, have provided important insights into platelet physiology and pathophysiology and, as a group, are less common, better characterized and more readily classified than the acquired defects. The severity of bleeding resulting from platelet dysfunction varies greatly and is substantially increased when another defect of hemostasis coexists. A disorder of platelet function is suspected on the basis of the history and physical examination and is confirmed by the finding of a prolonged bleeding time in the presence of an adequate number of platelets. A specific diagnosis often requires measurements of the factor VIII and von Willebrand factor complex and other tests of platelet function. Some of these tests may be available only in specialized laboratories. Therapy for bleeding episodes resulting from platelet dysfunction is directed at (1) removing or treating the underlying cause of the platelet disorder; (2) replacing the missing plasma cofactors needed to support normal platelet function (such as by the transfusion of cryoprecipitate in patients with von Willebrand disease, and (3) transfusing functional platelets in the form of platelet concentrates in patients with disorders of intrinsic platelet dysfunction. ImagesFigure 1.Figure 2.Figure 3. PMID:7013276

Additional Evidence Is Needed to Recommend Acquiring a Dog to Families of Children with Autism Spectrum Disorder: A Response to Wright and Colleagues

ERIC Educational Resources Information Center

Crossman, Molly K.; Kazdin, Alan E.

2016-01-01

Caregivers of children with autism spectrum disorder are vulnerable to overstated benefits of interventions, and such overstatements are common with interventions involving animals. This response to Wright, Hall, Hames, Hardmin, Mills, the Paws Team, and Mills' (2015) article, "Acquiring a Pet Dog Significantly Reduces Stress of Primary…

Acquired aplastic anemia.

PubMed

Keohane, Elaine M

2004-01-01

Acquired aplastic anemia (AA) is a disorder characterized by a profound deficit of hematopoietic stem and progenitor cells, bone marrow hypocellularity, and peripheral blood pancytopenia. It primarily affects children, young adults, and those over 60 years of age. The majority of cases are idiopathic; however, idiosyncratic reactions to some drugs, chemicals, and viruses have been implicated in its etiology. An autoimmune T-cell reaction likely causes the stem cell depletion, but the precise mechanism, as well as the eliciting and target antigens, is unknown. Symptoms vary from severe life-threatening cytopenias to moderate or non-severe disease that does not require transfusion support. The peripheral blood typically exhibits pancytopenia, reticulocytopenia, and normocytic or macrocytic erythrocytes. The bone marrow is hypocellular and may exhibit dysplasia of the erythrocyte precursors. First line treatment for severe AA consists of hematopoietic stem cell transplantation in young patients with HLA identical siblings, while immunosuppression therapy is used for older patients and for those of any age who lack a HLA matched donor. Patients with AA have an increased risk of developing paroxysmal nocturnal hemoglobinuria (PNH), myelodysplastic syndrome (MDS), or acute leukemia. Further elucidation of the pathophysiology of this disease will result in a better understanding of the interrelationship among AA, PNH, and MDS, and may lead to novel targeted therapies.

Interventions for eye movement disorders due to acquired brain injury.

PubMed

Rowe, Fiona J; Hanna, Kerry; Evans, Jennifer R; Noonan, Carmel P; Garcia-Finana, Marta; Dodridge, Caroline S; Howard, Claire; Jarvis, Kathryn A; MacDiarmid, Sonia L; Maan, Tallat; North, Lorraine; Rodgers, Helen

2018-03-05

Acquired brain injury can cause eye movement disorders which may include: strabismus, gaze deficits and nystagmus, causing visual symptoms of double, blurred or 'juddery' vision and reading difficulties. A wide range of interventions exist that have potential to alleviate or ameliorate these symptoms. There is a need to evaluate the effectiveness of these interventions and the timing of their implementation. We aimed to assess the effectiveness of any intervention and determine the effect of timing of intervention in the treatment of strabismus, gaze deficits and nystagmus due to acquired brain injury. We considered restitutive, substitutive, compensatory or pharmacological interventions separately and compared them to control, placebo, alternative treatment or no treatment for improving ocular alignment or motility (or both). We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (containing the Cochrane Eyes and Vision Trials Register) (2017, Issue 5), MEDLINE Ovid, Embase Ovid, CINAHL EBSCO, AMED Ovid, PsycINFO Ovid, Dissertations & Theses (PQDT) database, PsycBITE (Psychological Database for Brain Impairment Treatment Efficacy), ISRCTN registry, ClinicalTrials.gov, Health Services Research Projects in Progress (HSRProj), National Eye Institute Clinical Studies Database and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP). The databases were last searched on 26 June 2017. No date or language restrictions were used in the electronic searches for trials. We manually searched the Australian Orthoptic Journal, British and Irish Orthoptic Journal, and ESA, ISA and IOA conference proceedings. We contacted researchers active in this field for information about further published or unpublished studies. We included randomised controlled trials (RCTs) of any intervention for ocular alignment or motility deficits (or both) due to acquired brain injury. Two review authors independently selected studies and

[Acquired angioedema – clinical characteristic of the patients diagnosed in 2012-2016 with acquired C1 inhibitor deficiency].

PubMed

Stobiecki, Marcin; Czarnobilska, Ewa; Obtułowicz, Krystyna

Acquired angioedema is a rare disease caused by a deficiency of C1 esterase inhibitor with recurrent swelling symptoms. It may occur in the course of lymphoproliferative disorders or autoimmune diseases. Symptoms resemble hereditary angioedema, and the only differentiating features is negative family history, late onset of symptoms and accompanying lymphoproliferative disorder. The aim of the study was to analyze the cases of acquired angioedema. The retrospective analysis of 341 patients from the registry of patients with C1 inhibitor deficiency. Results: We identified 4 patients among 119 with HAE (3.57%) diagnosed in this same period of time 2012-2016 who fulfilled the criteria of acquired edema. In two cases the primary reason of angioedema was lymphoproliferive disease, in two monoclonal gammapathy of unknown reason. We analyzed also the results of laboratory tests C4, C1 inhibitor, C1q. In all cases the face was dominated localization. After the treatment of primary lymphoproliferive disease, in two cases, we observed total remission of angioedema. Only one patient with gammapathy require treatment with C1 inhibitor during the attacks. In these case we observed both plasma deriver, and recombinant C1 inhibitor were effective.

Isolated acquired factor VII deficiency: review of the literature.

PubMed

Mulliez, Sylvie M N; Devreese, Katrien M J

2016-04-01

Isolated acquired factor VII (FVII) deficiency is a rare haemorrhagic disorder. We report what is currently known about the pathogenesis, clinical features, diagnosis, treatment and prognosis of acquired FVII deficiency. We performed a literature search and included all articles published between 1980 and August 2015. Acquired FVII deficiency has been reported in 42 patients. There are well-established clinical diseases associated with acquired FVII deficiency, most notably infections, malignancy and haematological stem cell transplantation. The exact pathogenesis of the diseases is still unknown, but different pathophysiological hypotheses have been suggested. The clinical manifestation of acquired FVII deficiency varies greatly in severity; asymptomatic course as well as severe life-threatening bleeding diathesis and fatal bleedings have been described.

Multifocal Motor Neuropathy, Multifocal Acquired Demyelinating Sensory and Motor Neuropathy and Other Chronic Acquired Demyelinating Polyneuropathy Variants

PubMed Central

Barohn, Richard J.; Katz, Jonathan

2014-01-01

Chronic acquired demyelinating neuropathies (CADP) are an important group of immune neuromuscular disorders affecting myelin. These are distinct from chronic inflammatory demyelinating polyneuropathy (CIDP). Classically, CIDP is characterized by proximal and distal weakness, large fiber sensory loss, elevated cerebrospinal fluid (CSF) protein content, demyelinating changes nerve conduction studies or nerve biopsy, and response to immunomodulating treatment. In this chapter we discuss CADP with emphasis on multifocal motor neuropathy (MMN), multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), distal acquired demyelinating symmetric (DADS) neuropathy and conclude with less common variants. While each of these entities has distinctive laboratory and electrodiagnostic features that aid in their diagnosis, clinical characteristics are of paramount importance in diagnosing specific conditions and determining the most appropriate therapies. Unlike CIDP, MMN is typically asymmetric and affects only the motor nerve fibers. MMN is a rare disease that presents chronically, over several years of progression affecting the arms are more commonly than the legs. Men are more likely than women to develop MMN. MADSAM should be suspected in patients who have weakness and loss of sensation in primarily one arm or leg which progresses slowly over several months to years. It is important in patient with multifocal demyelinating clinical presentation to distinguish MMN from MADSAM since corticosteroids are not effective in MMN where the mainstay of therapy is intravenous gammaglobulin (IVIg). DADS can be subdivided into DADS-M (associated woth M-protein) and DADS-I which is idioapthic. While DADS-I patients respond somewhat to immunotherapy, DADS-M patients present with distal predominant sensorimotor demyelinating neuropathy phenotype and are notoriously refractory to immunotherapies regardless of antibodies to myelin-associated glycoprotein (MAG). Our knowledge

Histological characterization of regression in acquired immunodeficiency syndrome-related Kaposi's sarcoma.

PubMed

Pantanowitz, Liron; Dezube, Bruce J; Pinkus, Geraldine S; Tahan, Steven R

2004-01-01

Kaposi's sarcoma (KS) is an angioproliferative lesion that may regress or progress. Progression is related to spindle cell proliferation and the expression of human herpes virus-8 latency genes, including latent nuclear antigen-1 (LNA-1), cyclin-D1, and bcl-2. KS regression has not been well characterized histologically. Therefore, this study was undertaken to characterize the histopathology of pharmacologically induced regressed cutaneous KS. Skin punch biopsies from eight patients with acquired immunodeficiency syndrome (AIDS)-related KS, that regressed following chemotherapy with paclitaxel or the angiogenesis inhibitor Col-3, were investigated by light microscopy. Comparative immunophenotyping on pre- and post-treatment specimens for CD31, LNA-1, cyclin-D1, bcl-2, and CD117 (c-kit) was performed. Clinical and histologic features of regression were similar for paclitaxel and Col-3 treatment. On clinical examination, lesions flattened, became smaller, and lost their purple-red appearance, resulting in an orange-brown macule. Histological regression was divided into partial (n = 3) and complete (n = 5) regression. Partially regressed lesions had a significant reduction of spindle cells in the dermal interstitium, with residual spindle cells arranged around superficial and mid-dermal capillaries. Complete regression was characterized by an absence of detectable spindle cells, with a slight increase in capillaries of the superficial plexus. All regressed samples exhibited a prominent, superficial, perivascular, lymphocytic infiltrate and abundant dermal hemosiderin-laden macrophages. This clinicopathologic picture resembled the findings of pigmented purpura. CD31 staining correlated with the reduction of spindle cells. Regression was accompanied by a quantitative and qualitative decrease in LNA-1 and cyclin-D1 immunoreactivity, but no change in bcl-2 or c-kit expression. Pharmacologically induced regression of AIDS-related cutaneous KS is characterized by a complete

The use of electropalatography in the assessment and treatment of acquired motor speech disorders in adults: current knowledge and future directions.

PubMed

McAuliffe, Megan J; Ward, Elizabeth C

2006-01-01

Electropalatography (EPG) has been employed to measure speech articulation since the mid-1970s. This technique has predominately been used in experimental phonetic research and in the diagnosis and treatment of articulation disorders in children. However, there is a growing body of research employing EPG to diagnose and treat articulatory impairment associated with acquired motor speech disorder (MSD) in adults. The purpose of this paper was to (1) review the findings of studies pertaining to the assessment and treatment of MSDs in adults using EPG, (2) highlight current methodologies employed, and (3) discuss the potential limitations of EPG in the assessment and treatment of MSDs and examine directions for future applied research and treatment studies.

Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2).

PubMed

Knoebl, P; Marco, P; Baudo, F; Collins, P; Huth-Kühne, A; Nemes, L; Pellegrini, F; Tengborn, L; Lévesque, H

2012-04-01

Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies against coagulation factor VIII and characterized by spontaneous hemorrhage in patients with no previous family or personal history of bleeding. Although data on several AHA cohorts have been collected, limited information is available on the optimal management of AHA. The European Acquired Hemophilia Registry (EACH2) was established to generate a prospective, large-scale, pan-European database on demographics, diagnosis, underlying disorders, bleeding characteristics, treatment and outcome of AHA patients. Five hundred and one (266 male, 235 female) patients from 117 centers and 13 European countries were included in the registry between 2003 and 2008. In 467 cases, hemostasis investigations and AHA diagnosis were triggered by a bleeding event. At diagnosis, patients were a median of 73.9 years. AHA was idiopathic in 51.9%; malignancy or autoimmune diseases were associated with 11.8% and 11.6% of cases. Fifty-seven per cent of the non-pregnancy-related cases were male. Four hundred and seventy-four bleeding episodes were reported at presentation, and hemostatic therapy initiated in 70.5% of patients. Delayed diagnosis significantly impacted treatment initiation in 33.5%. Four hundred and seventy-seven patients underwent immunosuppression, and 72.6% achieved complete remission. Representing the largest collection of consecutive AHA cases to date, EACH2 facilitates the analysis of a variety of open questions in AHA. © 2012 International Society on Thrombosis and Haemostasis.

Acquired Brachial Cutaneous Dyschromatosis in a Middle Aged Male

PubMed Central

Choi, Min Jung; Byun, Ji Yeon; Choi, Hae Young

2018-01-01

Acquired brachial cutaneous dyschromatosis (ABCD) is an acquired disorder of pigmentary change that presents as chronic, asymptomatic, geographic-shaped, gray-brown patches, consisting of mixed hyper and hypopigmented macules on the dorsal aspect of the forearms. We report a case of a 40-year-old male who presented with asymptomatic, multiple brown-colored macules on the outer aspects of both arms. He had no history of hypertension and had never taken angiotensin converting enzyme inhibitors. He also denied chronic sun exposure history. Histologic examination demonstrated epidermal atrophy, increased basal layer pigmentation, and several telangiectatic vessels in the upper dermis. Solar elastosis was not remarkable. The patient's clinical and histopathologic features were consistent with a diagnosis of ABCD. Poikiloderma of Civatte, melasma, acquired bilateral telangiectatic macules and other pigmentary disorders should be considered in the differential diagnosis of ABCD. Herein, we report a case of ABCD in a middle-aged male without hypertension and medication. PMID:29853750

Intensive care unit-acquired weakness.

PubMed

Griffiths, Richard D; Hall, Jesse B

2010-03-01

Severe weakness is being recognized as a complication that impacts significantly on the pace and degree of recovery and return to former functional status of patients who survive the organ failures that mandate life-support therapies such as mechanical ventilation. Despite the apparent importance of this problem, much remains to be understood about its incidence, causes, prevention, and treatment. Review from literature and an expert round-table. The Brussels Round Table Conference in 2009 convened more than 20 experts in the fields of intensive care, neurology, and muscle physiology to review current understandings of intensive care unit-acquired weakness and to improve clinical outcome. Formal electrophysiological evaluation of patients with intensive care unit-acquired weakness can identify peripheral neuropathies, myopathies, and combinations of these disorders, although the correlation of these findings to weakness measurable at the bedside is not always precise. For routine clinical purposes, bedside assessment of neuromuscular function can be performed but is often confounded by complicating factors such as sedative and analgesic administration. Risk factors for development of intensive care unit-acquired weakness include bed rest itself, sepsis, and corticosteroid exposure. A strong association exists between weakness and long-term ventilator dependence; weakness is a major determinant of patient outcomes after surviving acute respiratory failure and may be present for months, or indefinitely, in the convalescence phase of critical illness. Although much has been learned about the physiology and cell and molecular biology of skeletal and diaphragm dysfunction under conditions of aging, exercise, disuse, and sepsis, the application of these understandings to the bedside requires more study in both bench models and patients. Although a trend toward greater immobilization and sedation of patients has characterized the past several decades of intensive care

Functional characterization of rare FOXP2 variants in neurodevelopmental disorder.

PubMed

Estruch, Sara B; Graham, Sarah A; Chinnappa, Swathi M; Deriziotis, Pelagia; Fisher, Simon E

2016-01-01

Heterozygous disruption of FOXP2 causes a rare form of speech and language impairment. Screens of the FOXP2 sequence in individuals with speech/language-related disorders have identified several rare protein-altering variants, but their phenotypic relevance is often unclear. FOXP2 encodes a transcription factor with a forkhead box DNA-binding domain, but little is known about the functions of protein regions outside this domain. We performed detailed functional analyses of seven rare FOXP2 variants found in affected cases, including three which have not been previously characterized, testing intracellular localization, transcriptional regulation, dimerization, and interaction with other proteins. To shed further light on molecular functions of FOXP2, we characterized the interaction between this transcription factor and co-repressor proteins of the C-terminal binding protein (CTBP) family. Finally, we analysed the functional significance of the polyglutamine tracts in FOXP2, since tract length variations have been reported in cases of neurodevelopmental disorder. We confirmed etiological roles of multiple FOXP2 variants. Of three variants that have been suggested to cause speech/language disorder, but never before been characterized, only one showed functional effects. For the other two, we found no effects on protein function in any assays, suggesting that they are incidental to the phenotype. We identified a CTBP-binding region within the N-terminal portion of FOXP2. This region includes two amino acid substitutions that occurred on the human lineage following the split from chimpanzees. However, we did not observe any effects of these amino acid changes on CTBP binding or other core aspects of FOXP2 function. Finally, we found that FOXP2 variants with reduced polyglutamine tracts did not exhibit altered behaviour in cellular assays, indicating that such tracts are non-essential for core aspects of FOXP2 function, and that tract variation is unlikely to be a

Acquired Auditory Verbal Agnosia and Seizures in Childhood

ERIC Educational Resources Information Center

Cooper, Judith A.; Ferry, Peggy C.

1978-01-01

The paper presents a review of cases of children with acquired aphasia with convulsive disorder and discusses clinical features of three additional children in whom the specific syndrome of auditory verbal agnosia was identified. (Author/CL)

Acquired Aplastic Anemia in Children

PubMed Central

Hartung, Helge D.; Olson, Timothy S.; Bessler, Monica

2013-01-01

SYNOPSIS This article provides a practice-based and concise review of the etiology, diagnosis, and management of acquired aplastic anemia in children. Bone marrow transplantation, immunosuppressive therapy, and supportive care are discussed in detail. The aim is to provide the clinician with a better understanding of the disease and to offer guidelines for the management of children with this uncommon yet serious disorder. PMID:24237973

[How to characterize and treat sleep complaints in bipolar disorders?

PubMed

Geoffroy, P A; Micoulaud Franchi, J-A; Lopez, R; Poirot, I; Brion, A; Royant-Parola, S; Etain, B

2017-08-01

Sleep complaints are very common in bipolar disorders (BD) both during acute phases (manic and depressive episodes) and remission (about 80 % of patients with remitted BD have poor sleep quality). Sleep complaints during remission are of particular importance since they are associated with more mood relapses and worse outcomes. In this context, this review discusses the characterization and treatment of sleep complaints in BD. We examined the international scientific literature in June 2016 and performed a literature search with PubMed electronic database using the following headings: "bipolar disorder" and ("sleep" or "insomnia" or "hypersomnia" or "circadian" or "apnoea" or "apnea" or "restless legs"). Patients with BD suffer from sleep and circadian rhythm abnormalities during major depressive episodes (insomnia or hypersomnia, nightmares, nocturnal and/or early awakenings, non-restorative sleep) and manic episodes (insomnia, decreased need for sleep without fatigue), but also some of these abnormalities may persist during remission. These remission phases are characterized by a reduced quality and quantity of sleep, with a longer sleep duration, increased sleep latency, a lengthening of the wake time after sleep onset (WASO), a decrease of sleep efficiency, and greater variability in sleep/wake rhythms. Patients also present frequent sleep comorbidities: chronic insomnia, sleepiness, sleep phase delay syndrome, obstructive sleep apnea/hypopnea syndrome (OSAHS), and restless legs syndrome (RLS). These disorders are insufficiently diagnosed and treated whereas they are associated with mood relapses, treatment resistance, affect cognitive global functioning, reduce the quality of life, and contribute to weight gain or metabolic syndrome. Sleep and circadian rhythm abnormalities have been also associated with suicidal behaviors. Therefore, a clinical exploration with characterization of these abnormalities and disorders is essential. This exploration should be

Acquired dyslexia in a Turkish-English speaker.

PubMed

Raman, Ilhan; Weekes, Brendan S

2005-06-01

The Turkish script is characterised by completely transparent bidirectional mappings between orthography and phonology. To date, there has been no reported evidence of acquired dyslexia in Turkish speakers leading to the naïve view that reading and writing problems in Turkish are probably rare. We examined the extent to which phonological impairment and orthographic transparency influence reading disorders in a native Turkish speaker. BRB is a bilingual Turkish-English speaker with deep dysphasia accompanied by acquired dyslexia in both languages. The main findings are an effect of imageability on reading in Turkish coincident with surface dyslexia in English and preserved nonword reading. BRB's acquired dyslexia suggests that damage to phonological representations might have a consequence for learning to read in Turkish. We argue that BRB's acquired dyslexia has a common locus in chronic underactivation of phonological representations in Turkish and English. Despite a common locus, reading problems manifest themselves differently according to properties of the script and the type of task.

[PECULIARITIES OF COMMUNITY-ACQUIRED PNEUMONIA IN CHILDREN WITH NEUROLOGICAL PATHOLOGY].

PubMed

Zubarenko, O; Kopiyka, G; Kravchenko, T; Koval, L; Gurienko, K

2017-06-01

Neurological disorders in children highly affect the course of pneumonia, its outcome and the development of possible complications. The aim of the study was to reveal clinical and paraclinical features of community-acquired pneumonia in younger children with neurologic pathology infantile cerebral palsy. Under observation were 37 children with community-acquired pneumonia aged 1 to 3 years that suffered from spastic forms of infantile cerebral palsy. The comparison group consisted of 30 children with community-acquired pneumonia without any concomitant neurological pathology. The age of the children in the comparison and study groups was the same. The results of the study show that the presence of infantile cerebral palsy allow to relate the child to the risk group of respiratory pathology development. The course of community-acquired pneumonia in children affected by infantile cerebral palsy is characterized by rapid progression of symptoms and severity of the condition, and the clinical picture also has a number of characteristic features. Thus, cough, local physical data, classical laboratory signs of inflammation in the form of leukocytosis with neutrophil shift were noticed significantly less often in children with infantile cerebral palsy. The debut of the disease was often accompanied by bronchial obstruction, the inflammatory process was localized in the lower parts of the lungs and often matched the side of the neurologically affected part of the body. Children with cerebral palsy required a longer hospital-stay and a prolonged course of antibiotic therapy. Therefore, the risk of pneumonia in children with infantile cerebral palsy should be taken into account at the primary stage of medical care for the creation of preventive programs.

"Surgical" abdomen in a patient with chronic lymphocytic leukemia: a case of acquired angioedema.

PubMed

Jung, Moonjung; Rice, Lawrence

2011-12-01

Acquired angioedema (AAE), an acquired deficiency of C1esterase inhibitor, is a medically treatable condition which can cause severe abdominal pain mimicking an acute surgical abdomen. This disorder is strongly associated with chronic lymphocytic leukemia (CLL) and other indolent lymphoplasmacytic disorders. We describe a patient with known CLL who developed incapacitating, recurrent severe abdominal pains, culminating in partial bowel resection. Signs, symptoms, laboratory and pathologic findings demonstrated AAE. Wider appreciation of the possibility of AAE, particularly in patients with lymphoproliferative disorders, could lead to preventive therapy and spare unnecessary surgery. This is more important now that more effective medical therapies are available.

Dnmt2 mediates intergenerational transmission of paternally acquired metabolic disorders through sperm small non-coding RNAs.

PubMed

Zhang, Yunfang; Zhang, Xudong; Shi, Junchao; Tuorto, Francesca; Li, Xin; Liu, Yusheng; Liebers, Reinhard; Zhang, Liwen; Qu, Yongcun; Qian, Jingjing; Pahima, Maya; Liu, Ying; Yan, Menghong; Cao, Zhonghong; Lei, Xiaohua; Cao, Yujing; Peng, Hongying; Liu, Shichao; Wang, Yue; Zheng, Huili; Woolsey, Rebekah; Quilici, David; Zhai, Qiwei; Li, Lei; Zhou, Tong; Yan, Wei; Lyko, Frank; Zhang, Ying; Zhou, Qi; Duan, Enkui; Chen, Qi

2018-05-01

The discovery of RNAs (for example, messenger RNAs, non-coding RNAs) in sperm has opened the possibility that sperm may function by delivering additional paternal information aside from solely providing the DNA 1 . Increasing evidence now suggests that sperm small non-coding RNAs (sncRNAs) can mediate intergenerational transmission of paternally acquired phenotypes, including mental stress 2,3 and metabolic disorders 4-6 . How sperm sncRNAs encode paternal information remains unclear, but the mechanism may involve RNA modifications. Here we show that deletion of a mouse tRNA methyltransferase, DNMT2, abolished sperm sncRNA-mediated transmission of high-fat-diet-induced metabolic disorders to offspring. Dnmt2 deletion prevented the elevation of RNA modifications (m 5 C, m 2 G) in sperm 30-40 nt RNA fractions that are induced by a high-fat diet. Also, Dnmt2 deletion altered the sperm small RNA expression profile, including levels of tRNA-derived small RNAs and rRNA-derived small RNAs, which might be essential in composing a sperm RNA 'coding signature' that is needed for paternal epigenetic memory. Finally, we show that Dnmt2-mediated m 5 C contributes to the secondary structure and biological properties of sncRNAs, implicating sperm RNA modifications as an additional layer of paternal hereditary information.

Increased Small-World Network Topology Following Deployment-Acquired Traumatic Brain Injury Associated with the Development of Post-Traumatic Stress Disorder.

PubMed

Rowland, Jared A; Stapleton-Kotloski, Jennifer R; Dobbins, Dorothy L; Rogers, Emily; Godwin, Dwayne W; Taber, Katherine H

2018-05-01

Cross-sectional and longitudinal studies in active duty and veteran cohorts have both demonstrated that deployment-acquired traumatic brain injury (TBI) is an independent risk factor for developing post-traumatic stress disorder (PTSD), beyond confounds such as combat exposure, physical injury, predeployment TBI, and pre-deployment psychiatric symptoms. This study investigated how resting-state brain networks differ between individuals who developed PTSD and those who did not following deployment-acquired TBI. Participants included postdeployment veterans with deployment-acquired TBI history both with and without current PTSD diagnosis. Graph metrics, including small-worldness, clustering coefficient, and modularity, were calculated from individually constructed whole-brain networks based on 5-min eyes-open resting-state magnetoencephalography (MEG) recordings. Analyses were adjusted for age and premorbid IQ. Results demonstrated that participants with current PTSD displayed higher levels of small-worldness, F(1,12) = 5.364, p < 0.039, partial eta squared = 0.309, and Cohen's d = 0.972, and clustering coefficient, F(1, 12) = 12.204, p < 0.004, partial eta squared = 0.504, and Cohen's d = 0.905, than participants without current PTSD. There were no between-group differences in modularity or the number of modules present. These findings are consistent with a hyperconnectivity hypothesis of the effect of TBI history on functional networks rather than a disconnection hypothesis, demonstrating increased levels of clustering coefficient rather than a decrease as might be expected; however, these results do not account for potential changes in brain structure. These results demonstrate the potential pathological sequelae of changes in functional brain networks following deployment-acquired TBI and represent potential neurobiological changes associated with deployment-acquired TBI that may increase the risk of subsequently developing PTSD.

Inherited and acquired disorders of myelin: the underling myelin pathology

PubMed Central

Duncan, Ian D.; Radcliff, Abigail B.

2016-01-01

Remyelination is a major therapeutic goal in human myelin disorders, serving to restore function to demyelinated axons and providing neuroprotection. The target disorders that might be amenable to the promotion of this repair process are diverse and increasing in number. They range primarily from those of genetic, inflammatory to toxic origin. In order to apply remyelinating strategies to these disorders, it is essential to know whether the myelin damage results from a primary attack on myelin or the oligodendrocyte or both, and whether indeed these lead to myelin breakdown and demyelination. In some disorders, myelin sheath abnormalities are prominent but demyelination does not occur. This review explores the range of human and animal disorders where myelin pathology exists and focusses on defining the myelin changes in each and their cause, to help define whether they are targets for myelin repair therapy. PMID:27068622

Neuromuscular disorders in the intensive care unit.

PubMed

Marinelli, William A; Leatherman, James W

2002-10-01

Neuromuscular disorders encountered in the ICU can be categorized as muscular diseases that lead to ICU admission and those that are acquired in the ICU. This article discusses three neuromuscular disorders can lead to ICU admission and have a putative immune-mediated pathogenesis: the Guillian-Barré syndrome, myasthenia gravis, and dermatomyositis/polymyositis. It also reviews critical care polyneuropathy and ICU acquired myopathy, two disorders that, alone or in combination, are responsible for nearly all cases of severe ICU acquired muscle weakness.

Additional Evidence is Needed to Recommend Acquiring a Dog to Families of Children with Autism Spectrum Disorder: A Response to Wright and Colleagues.

PubMed

Crossman, Molly K; Kazdin, Alan E

2016-01-01

Caregivers of children with autism spectrum disorder are vulnerable to overstated benefits of interventions, and such overstatements are common with interventions involving animals. This response to Wright, Hall, Hames, Hardmin, Mills, the Paws Team, and Mills' (2015) article, "Acquiring a Pet Dog Significantly Reduces Stress of Primary Careers for Children with Autism Spectrum Disorder: A Prospective Case Control Study," details why that study's conclusions are premature. Specific limitations of the study are detailed, including overstatements of the supportive literature, problems with the design, and mismatch between the findings and conclusions. The purpose is not to challenge the benefits of pet ownership, but to point out that those benefits have not yet been established.

Acquired Hemophilia A in an advanced age patient of hispanic origin: a case report.

PubMed

Rivera Cora, Nalyssa I; Irizarry Delgado, Freddie; Merle Ramírez, Santa M; Vera Quiñones, Jorge

2017-09-04

Acquired Hemophilia A (AHA) is a rare hematological disorder that exhibits an incidence of approximately 1.5 cases per million patients a year. It is characterized by the development of autoantibodies against circulating Factor VIII coagulation proteins which, in turn, which in turn lead to potentially life-threatening hemorrhagic episodes. The incidence of AHA increases with age; with 80% of the affected patient population encompassing men and women that are 65 years or older. Some of the challenges that are highlighted in managing this disorder relate to the delayed diagnosis of this condition due to the rarity of the latter, the difficulty in establishing reliable hemostasis, and the secondary complications that are found when using immunosuppressive and hemostatic treatments in tandem with the elderly population afflicted with this disease. A 90-year-old female of Hispanic origin presented with a 2-week history of generalized weakness, dizziness, shortness of breath and extensive purpuric formations that involved the left arm towards the lateral aspect of the thorax with the inclusion of a small right lateral neck hematoma formation. Upon initial laboratory screening, a hemoglobin level of 7.9, a hematocrit level of 21.9 and a PTT value of 70.9 were discovered. Despite conventional hemostatic treatment approaches, the patient did not show marked improvement of the laboratory values. Ongoing specialized laboratory reports, combined with the clinical presentation of the patient, led to the diagnosis of Acquired Hemophilia A. Treatment with recombinant porcine Factor VIII was initiated, which led to rapid improvement of clinical symptoms and laboratory values. The patient was discharged with current treatment plan and emergent follow/up with a hematologist was scheduled. Acquired Hemophilia A is an elusive bleeding disorder that has been seldom encountered in the demographics of Puerto Rico. The prompt detection of this diagnosis based on the clinical

Experiences and expectations of return-to-work programs for nurses and midwives who have acquired a musculoskeletal disorder in the workplace: a qualitative systemic review protocol.

PubMed

Weckert, Christine; Stern, Cindy; Porritt, Kylie

2017-05-01

The objective of this systematic review is to identify and synthesize the best available evidence on the experiences and expectations of being involved in a program that aims to return nurses and midwives, who have acquired a musculoskeletal disorder (MSD) in the workplace, to work.The specific review questions are.

Connectionist neuropsychology: uncovering ultimate causes of acquired dyslexia.

PubMed

Woollams, Anna M

2014-01-01

Acquired dyslexia offers a unique window on to the nature of the cognitive and neural architecture supporting skilled reading. This paper provides an integrative overview of recent empirical and computational work on acquired dyslexia within the context of the primary systems framework as implemented in connectionist neuropsychological models. This view proposes that damage to general visual, phonological or semantic processing abilities are the root causes of different forms of acquired dyslexia. Recent case-series behavioural evidence concerning pure alexia, phonological dyslexia and surface dyslexia that supports this perspective is presented. Lesion simulations of these findings within connectionist models of reading demonstrate the viability of this approach. The commitment of such models to learnt representations allows them to capture key aspects of performance in each type of acquired dyslexia, particularly the associated non-reading deficits, the role of relearning and the influence of individual differences in the premorbid state of the reading system. Identification of these factors not only advances our understanding of acquired dyslexia and the mechanisms of normal reading but they are also relevant to the complex interactions underpinning developmental reading disorders.

Characterizing somatization, hypochondriasis, and hysteria in the borderline personality disorder.

PubMed

Snyder, S; Pitts, W M

1986-03-01

Somatization, hypochondriasis, and hysteria have often been considered as associated features of the borderline personality disorder. This study was designed to characterize these three syndromes in the borderline patient. Inpatients with DSM-III borderline personality disorder were compared with controls with dysthymic disorder. Scales and items from standardized rating instruments which measured the three syndromes were scored and compared between groups. Although the hysteria-obvious and hypochondriasis scales of the MMPI and the Hamilton Depression Scale item measuring hypochondriasis were elevated in the borderline group, there were no significant differences between groups. Scores of dysthymic patients significantly exceeded those of borderline patients on four of five MMPI codetypes measuring the three syndromes. Findings are discussed in light of previous psychodynamic, empirical, and research literature.

Acquired Thrombotic Thrombocytopenic Purpura in a Patient with Pernicious Anemia.

PubMed

Pandey, Ramesh Kumar; Dahal, Sumit; Fadlalla, Kamal Fadlalla El Jack; Bhagat, Shambhu; Bhattarai, Bikash

2017-01-01

Introduction . Acquired thrombotic thrombocytopenic purpura (TTP) has been associated with different autoimmune disorders. However, its association with pernicious anemia is rarely reported. Case Report . A 46-year-old male presented with blood in sputum and urine for one day. The vitals were stable. The physical examination was significant for icterus. Lab tests' results revealed leukocytosis, macrocytic anemia, severe thrombocytopenia, renal dysfunction, and unconjugated hyperbilirubinemia. He had an elevated LDH, low haptoglobin levels with many schistocytes, nucleated RBCs, and reticulocytes on peripheral smear. Low ADAMTS13 activity (<10%) with elevated ADAMTS13 antibody clinched the diagnosis of severe acquired TTP, and plasmapheresis was started. There was an initial improvement in his hematological markers, which were however not sustained on discontinuation of plasmapheresis. For his refractory TTP, he was resumed on daily plasmapheresis and Rituximab was started. Furthermore, the initial serum Vitamin B12 and reticulocyte index were low in the presence of anti-intrinsic factor antibody. So with the concomitant diagnosis of pernicious anemia, Vitamin B12 was supplemented. The rest of the immunological workups were negative. Subsequently, his symptoms resolved and his hematological parameters improved. Discussion . While pernicious anemia can masquerade as TTP, an actual association between the two can also occur and needs further evaluation and characterization.

Acquired Thrombotic Thrombocytopenic Purpura in a Patient with Pernicious Anemia

PubMed Central

Bhagat, Shambhu

2017-01-01

Introduction. Acquired thrombotic thrombocytopenic purpura (TTP) has been associated with different autoimmune disorders. However, its association with pernicious anemia is rarely reported. Case Report. A 46-year-old male presented with blood in sputum and urine for one day. The vitals were stable. The physical examination was significant for icterus. Lab tests' results revealed leukocytosis, macrocytic anemia, severe thrombocytopenia, renal dysfunction, and unconjugated hyperbilirubinemia. He had an elevated LDH, low haptoglobin levels with many schistocytes, nucleated RBCs, and reticulocytes on peripheral smear. Low ADAMTS13 activity (<10%) with elevated ADAMTS13 antibody clinched the diagnosis of severe acquired TTP, and plasmapheresis was started. There was an initial improvement in his hematological markers, which were however not sustained on discontinuation of plasmapheresis. For his refractory TTP, he was resumed on daily plasmapheresis and Rituximab was started. Furthermore, the initial serum Vitamin B12 and reticulocyte index were low in the presence of anti-intrinsic factor antibody. So with the concomitant diagnosis of pernicious anemia, Vitamin B12 was supplemented. The rest of the immunological workups were negative. Subsequently, his symptoms resolved and his hematological parameters improved. Discussion. While pernicious anemia can masquerade as TTP, an actual association between the two can also occur and needs further evaluation and characterization. PMID:28473932

Myasthenia gravis and related disorders: Pathology and molecular pathogenesis.

PubMed

Ha, James C; Richman, David P

2015-04-01

Disorders affecting the presynaptic, synaptic, and postsynaptic portions of the neuromuscular junction arise from various mechanisms in children and adults, including acquired autoimmune or toxic processes as well as genetic mutations. Disorders include autoimmune myasthenia gravis associated with acetylcholine receptor, muscle specific kinase or Lrp4 antibodies, Lambert-Eaton myasthenic syndrome, nerve terminal hyperexcitability syndromes, Guillain Barré syndrome, botulism, organophosphate poisoning and a number of congenital myasthenic syndromes. This review focuses on the various molecular and pathophysiological mechanisms of these disorders, characterization of which has been crucial to the development of treatment strategies specific for each pathogenic mechanism. In the future, further understanding of the underlying processes may lead to more effective and targeted therapies of these disorders. This article is part of a Special Issue entitled: Neuromuscular Diseases: Pathology and Molecular Pathogenesis. Copyright © 2014 Elsevier B.V. All rights reserved.

[Comparative evaluation of the effectiveness of various treatment modalities for accommodation disorders and acquired progressive myopia].

PubMed

Tarutta, E P; Tarasova, N A

2015-01-01

To evaluate the effectiveness of non-surgical treatment of accommodation disorders and progressive myopia in children. A total of 190 patients (380 eyes) with myopia aged from 6 to 18 years (10.79±0.18 years on average) were enrolled and divided into 9 groups depending on the treatment prescribed. Comparative evaluation of different hardware-based treatment modalities for progressive myopia allowed to work out their optimal combination: "Visotronic", "MACDEL 09", and magnetophoresis of Taufon 4%. Such courses, provided twice a year, were associated with optimization of accommodative response and 1.9-2.8 times reduction of the rate of myopia progression. On the contrary, pleoptic therapy showed a negative effect on accommodative tonus and the rate of progression of acquired myopia. Comparative evaluation of different hardware-based treatment modalities for progressive myopia and accommodation disorders allowed to work out their optimal combination: "Visotronic", "MACDEL 09" and magnetophoresis of Taufon 4%. This treatment, provided twice a year, allows to increase accommodative reserves and volume, improve objective accommodative response, and reduce accommodative hypertonus as well as the rate of myopia progression (1.9-2.8 times over a 1.5-year period). Under pleoptic therapy (specialized software, near field speckles, color pulse therapy, Ambliokor device), both accommodative tonus and the rate of myopia progression increased (1.3-1.5 and 1.6 times correspondingly).

Alcohol Use Disorders and Community-Acquired Pneumococcal Pneumonia: Associated Mortality, Prolonged Hospital Stay and Increased Hospital Spending.

PubMed

Gili-Miner, Miguel; López-Méndez, Julio; Béjar-Prado, Luis; Ramírez-Ramírez, Gloria; Vilches-Arenas, Ángel; Sala-Turrens, José

2015-11-01

The aim of this study was to investigate the impact of alcohol use disorders (AUD) on community-acquired pneumococcal pneumonia (CAPP) admissions, in terms of in-hospital mortality, prolonged stay and increased hospital spending. Retrospective observational study of a sample of CAPP patients from the minimum basic datasets of 87 Spanish hospitals during 2008-2010. Mortality, length of hospital stay and additional spending attributable to AUD were calculated after multivariate covariance analysis for variables such as age and sex, type of hospital, addictions and comorbidities. Among 16,202 non-elective admissions for CAPP in patients aged 18-74years, 2,685 had AUD. Patients admitted with CAPP and AUD were predominantly men with a higher prevalence of tobacco or drug use disorders and higher Charlson comorbidity index. Patients with CAPP and AUD had notably higher in-hospital mortality (50.8%; CI95%: 44.3-54.3%), prolonged length of stay (2.3days; CI95%: 2.0-2.7days) and increased costs (1,869.2€; CI95%: 1,498.6-2,239.8€). According to the results of this study, AUD in CAPP patients was associated with increased in-hospital mortality, length of hospital stay and hospital spending. Copyright © 2014 SEPAR. Published by Elsevier Espana. All rights reserved.

Coagulation disorders in pregnancy: acquired and inherited thrombophilias.

PubMed

Benedetto, Chiara; Marozio, Luca; Tavella, Anna Maria; Salton, Loredana; Grivon, Sara; Di Giampaolo, Francesca

2010-09-01

Both acquired and inherited thrombophilias are associated with an increased risk of pregnancy-related venous thromboembolism (VTE) as well as with adverse pregnancy outcome. However, the extension of attributable risk for each thrombophilia and outcome is still a question of debate. Thrombophilias have been investigated in connection with VTE and pregnancy complications such as: recurrent and nonrecurrent early pregnancy loss, late fetal death, placental abruption, fetal growth restriction, and preeclampsia. This review discusses the evidence of association between thrombophilias and pregnancy outcome together with issues as to clinical management and preventive strategies. © 2010 New York Academy of Sciences.

Connectionist neuropsychology: uncovering ultimate causes of acquired dyslexia

PubMed Central

Woollams, Anna M.

2014-01-01

Acquired dyslexia offers a unique window on to the nature of the cognitive and neural architecture supporting skilled reading. This paper provides an integrative overview of recent empirical and computational work on acquired dyslexia within the context of the primary systems framework as implemented in connectionist neuropsychological models. This view proposes that damage to general visual, phonological or semantic processing abilities are the root causes of different forms of acquired dyslexia. Recent case-series behavioural evidence concerning pure alexia, phonological dyslexia and surface dyslexia that supports this perspective is presented. Lesion simulations of these findings within connectionist models of reading demonstrate the viability of this approach. The commitment of such models to learnt representations allows them to capture key aspects of performance in each type of acquired dyslexia, particularly the associated non-reading deficits, the role of relearning and the influence of individual differences in the premorbid state of the reading system. Identification of these factors not only advances our understanding of acquired dyslexia and the mechanisms of normal reading but they are also relevant to the complex interactions underpinning developmental reading disorders. PMID:24324241

Persistent genital arousal disorder: characterization, etiology, and management.

PubMed

Facelle, Thomas M; Sadeghi-Nejad, Hossein; Goldmeier, David

2013-02-01

Persistent genital arousal disorder (PGAD) is a potentially debilitating disorder of unwanted genital sensation and arousal that is generally spontaneous and unrelenting. Since its first description in 2001, many potential etiologies and management strategies have been suggested. To review the literature on PGAD, identify possible causes of the disorder, and provide approaches to the assessment and treatment of the disorder based on the authors' experience and recent literature. PubMed searches through July 2012 were conducted to identify articles relevant to persistent sexual arousal syndrome and PGAD. Expert opinion was based on review of the medical literature related to this subject matter. PGAD is characterized by persistent sensations of genital arousal in the absence of sexual stimulation or emotion, which are considered unwanted and cause the patient at least moderate distress. The proposed etiologies of PGAD are plentiful and may involve a range of psychologic, pharmacologic, neurologic, and vascular causes. PGAD has been associated with other conditions including overactive bladder and restless leg syndrome. Assessment should include a through history and physical exam and tailored radiologic studies. Treatment should be aimed at reversible causes, whether physiologic or pharmacologic. All patients should be considered for cognitive therapy including mindfullness meditation and acceptance therapy. PGAD likely represents a range of conditions manifesting in unwanted genital sensations. Successful treatment requires a multidisciplinary approach and consideration of all reversible causes as well as cognitive therapy. © 2012 International Society for Sexual Medicine.

Role of Investigating Thrombophilic Disorders in Young Stroke

PubMed Central

Ng, Kay W. P.; Loh, Pei K.; Sharma, Vijay K.

2011-01-01

Our knowledge about various inherited and acquired causes of thrombophilic disorders has increased significantly during the past decade. Technology for various diagnostic tests for these rare disorders has matched the rapid advances in our understanding about the thrombophilic disorders. Inherited thrombophilic disorders predispose young patients for various venous or arterial thrombotic and thromboembolic episodes. Our understanding has also improved about various gene-gene and gene-environment interactions and their impact on the resultant heterogenous clinical manifestations. We describe various thrombophilic disorders, their diagnostic tests, pathogenic potential in isolation or with other concurrent inherited/acquired defects and possible therapeutic and prophylactic strategies. Better understanding, optimal diagnostic and screening protocols are expected to improve the diagnostic yield and help to reduce morbidity, disability, and mortality in relatively younger patients harbouring these inherited and acquired thrombophilic disorders. PMID:21331344

Immunoglobulin G4-related acquired hemophilia: A case report

PubMed Central

Li, Xiaoyan; Duan, Wei; Zhu, Xiang; Xu, Jianying

2016-01-01

Acquired hemophilia A (AHA) is a relatively rare and life-threatening bleeding disorder whose pathogenesis is not completely understood. The present study reports a rare case of immunogubulin (IgG)4-related AHA with multisystemic involvement. A 55-year old male patient presented with symptoms of bronchial asthma and multiple subdermal hematomas. Chest computed tomography showed multiple diffuse nodular lesions with thickening of bronchovascular bundles, and scattered high-density spots in both lung lobes. Laboratory investigations showed increased activated partial prothrombin time (120.0 sec), a markedly decreased factor VIII (FVIII) activity (0.5%), a high-titer of FVIII inhibitor (27.2 Bethesda units/ml) and a marked increase in serum IgG4 (>4.03 g/l) level. Left inguinal lymph node biopsy revealed capsular thickening with marked lymphoplasmacytic infiltration, occlusive phlebitis and irregular fibrosis. Immunostaining revealed numerous IgG4-positive plasma cells (>100 cells/human plasma fibronectin) in the nodular lesions, with an IgG4/IgG ratio of >40%. The symptoms were markedly alleviated following corticosteroid therapy. The current study presents the first reported case of a rare IgG4-related AHA that presented with unusual clinical features and multisystemic involvement. The patient responded well to corticosteroid therapy. Documentation of such rare cases will help in characterizing the pathogenesis, and prompt recognition and timely treatment of this rare disorder. PMID:28105131

Central hypothyroidism - a neglected thyroid disorder.

PubMed

Beck-Peccoz, Paolo; Rodari, Giulia; Giavoli, Claudia; Lania, Andrea

2017-10-01

Central hypothyroidism is a rare and heterogeneous disorder that is characterized by a defect in thyroid hormone secretion in an otherwise normal thyroid gland due to insufficient stimulation by TSH. The disease results from the abnormal function of the pituitary gland, the hypothalamus, or both. Moreover, central hypothyroidism can be isolated or combined with other pituitary hormone deficiencies, which are mostly acquired and are rarely congenital. The clinical manifestations of central hypothyroidism are usually milder than those observed in primary hypothyroidism. Obtaining a positive diagnosis for central hypothyroidism can be difficult from both a clinical and a biochemical perspective. The diagnosis of central hypothyroidism is based on low circulating levels of free T 4 in the presence of low to normal TSH concentrations. The correct diagnosis of both acquired (also termed sporadic) and congenital (also termed genetic) central hypothyroidism can be hindered by methodological interference in free T 4 or TSH measurements; routine utilization of total T 4 or T 3 measurements; concurrent systemic illness that is characterized by low levels of free T 4 and normal TSH concentrations; the use of the sole TSH-reflex strategy, which is the measurement of the sole level of TSH, without free T 4 , if levels of TSH are in the normal range; and the diagnosis of congenital hypothyroidism based on TSH analysis without the concomitant measurement of serum levels of T 4 . In this Review, we discuss current knowledge of the causes of central hypothyroidism, emphasizing possible pitfalls in the diagnosis and treatment of this disorder.

[The use of growth hormone to treat endocrine-metabolic disturbances in acquired immunodeficiency syndrome (AIDS) patients].

PubMed

Spinola-Castro, Angela Maria; Siviero-Miachon, Adriana A; da Silva, Marcos Tadeu Nolasco; Guerra-Junior, Gil

2008-07-01

Acquired Immunodeficiency Syndrome (Aids) was initially related to HIV-associated wasting syndrome, and its metabolic disturbances to altered body composition. After Highly Active Antiretroviral Therapy (HAART) was started, malnutrition has declined and HIV-associated lipodystrophy syndrome has emerged as an important metabolic disorder. Aids is also characterized by hormonal disturbances, principally in growth hormone/insulin-like growth factor 1 (GH/IGF-1) axis. The use of recombinant human GH (hrGH) was formerly indicated to treat wasting syndrome, in order to increase lean body mass. Even though the use of hrGH in lipodystrophy syndrome has been considered, the decrease in insulin sensitivity is a limitation for its use, which has not been officially approved yet. Diversity in therapeutic regimen is another limitation to its use in Aids patients. The present study has reviewed the main HIV-related endocrine-metabolic disorders as well as the use of hrGH in such conditions.

Integrating eating disorder-specific risk factors into the acquired preparedness model of dysregulated eating: A moderated mediation analysis.

PubMed

Racine, Sarah E; Martin, Shelby J

2017-01-01

Tests of the acquired preparedness model demonstrate that the personality trait of negative urgency (i.e., the tendency to act impulsively when distressed) predicts the expectation that eating alleviates negative affect, and this eating expectancy subsequently predicts dysregulated eating. Although recent data indicate that eating disorder-specific risk factors (i.e., appearance pressures, thin-ideal internalization, body dissatisfaction, dietary restraint) strengthen negative urgency-dysregulated eating associations, it is unclear whether these risk factors impact associations directly or indirectly (i.e., through eating expectancies). The current study used latent moderated structural equation modeling to test moderated mediation hypotheses in a sample of 313 female college students. Eating expectancies mediated the association between negative urgency and dysregulated eating, and the indirect effect of negative urgency on dysregulated eating through eating expectancies was conditional on level of each eating disorder risk factor. Appearance pressures, thin-ideal internalization, body dissatisfaction, and dietary restraint significantly moderated the association between eating expectancies and dysregulated eating, while only dietary restraint moderated the direct effect of negative urgency on dysregulated eating. Findings suggest that the development of high-risk eating expectancies among individuals with negative urgency, combined with sociocultural pressures for thinness and their consequences, is associated with the greatest risk for dysregulated eating. Copyright © 2016 Elsevier Ltd. All rights reserved.

Creatine Transporter Deficiency: Screening of Males with Neurodevelopmental Disorders and Neurocognitive Characterization of a Case.

PubMed

Thurm, Audrey; Himelstein, Daniel; DʼSouza, Precilla; Rennert, Owen; Jiang, Susanqi; Olatunji, Damilola; Longo, Nicola; Pasquali, Marzia; Swedo, Susan; Salomons, Gajja S; Carrillo, Nuria

2016-05-01

Creatine transporter deficiency (CTD) is an X-linked, neurometabolic disorder associated with intellectual disability that is characterized by brain creatine (Cr) deficiency and caused by mutations in SLC6A8, the Cr transporter 1 protein gene. CTD is identified by elevated urine creatine/creatinine (Cr/Crn) ratio or reduced Cr peak on brain magnetic resonance spectroscopy; the diagnosis is confirmed by decreased Cr uptake in cultured fibroblasts, and/or identification of a mutation in the SLC6A8 gene. Prevalence studies suggest this disorder may be underdiagnosed. We sought to identify cases from a well-characterized cohort of children diagnosed with neurodevelopmental disorders. Urine screening for CTD was performed on a cohort of 46 males with autism spectrum disorder (ASD) and 9 males with a history of non-ASD developmental delay (DD) classified with intellectual disability. We identified 1 patient with CTD in the cohort based on abnormal urine Cr/Crn, and confirmed the diagnosis by the identification of a novel frameshift mutation in the SLC6A8 gene. This patient presented without ASD but with intellectual disability, and was characterized by a nonspecific phenotype of early language delay and DD that persisted into moderate-to-severe intellectual disability, consistent with previous descriptions of CTD. Identification of patients with CTD is possible by measuring urine Cr and Crn levels and the current case adds to the growing literature of neurocognitive deficits associated with the disorder that affect cognition, language and behavior in childhood.

Comparison of a therapeutic-only versus prophylactic platelet transfusion policy for people with congenital or acquired bone marrow failure disorders.

PubMed

Malouf, Reem; Ashraf, Asma; Hadjinicolaou, Andreas V; Doree, Carolyn; Hopewell, Sally; Estcourt, Lise J

2018-05-14

Bone marrow disorders encompass a group of diseases characterised by reduced production of red cells, white cells, and platelets, or defects in their function, or both. The most common bone marrow disorder is myelodysplastic syndrome. Thrombocytopenia, a low platelet count, commonly occurs in people with bone marrow failure. Platetet transfusions are routinely used in people with thrombocytopenia secondary to bone marrow failure disorders to treat or prevent bleeding. Myelodysplastic syndrome is currently the most common reason for receiving a platelet transfusion in some Western countries. To determine whether a therapeutic-only platelet transfusion policy (transfusion given when patient is bleeding) is as effective and safe as a prophylactic platelet transfusion policy (transfusion given to prevent bleeding according to a prespecified platelet threshold) in people with congenital or acquired bone marrow failure disorders. We searched for randomised controlled trials (RCTs), non-RCTs, and controlled before-after studies (CBAs) in the Cochrane Central Register of Controlled Trials (CENTRAL) (the Cochrane Library 2017, Issue 9), Ovid MEDLINE (from 1946), Ovid Embase (from 1974), PubMed (e-publications only), the Transfusion Evidence Library (from 1950), and ongoing trial databases to 12 October 2017. We included RCTs, non-RCTs, and CBAs that involved the transfusion of platelet concentrates (prepared either from individual units of whole blood or by apheresis any dose, frequency, or transfusion trigger) and given to treat or prevent bleeding among people with congenital or acquired bone marrow failure disorders.We excluded uncontrolled studies, cross-sectional studies, and case-control studies. We excluded cluster-RCTs, non-randomised cluster trials, and CBAs with fewer than two intervention sites and two control sites due to the risk of confounding. We included all people with long-term bone marrow failure disorders that require platelet transfusions, including

Acquired high titre factor VIII inhibitor with underlying polyarteritis nodosa.

PubMed

Snowden, J A; Hutchings, M; Spearing, R; Patton, W N

1997-05-01

We here present the case of a 70-year-old woman referred to our unit for investigation of bleeding. Investigations confirmed a high titre acquired Factor VIII inhibitor. In association there was relapse of systemic illness associated with anti-neutrophil cytoplasmic antibodies (atypical pattern) for which she had been treated five years previously. Immunosuppression was attempted, but it failed to have an impact both on the inhibitor titre and on the underlying disorder. The patient died from multi-organ failure and massive chest hemorrhage. Post-mortem showed necrotizing vasculitis of medium sized vessels at several sites, including the kidney, consistent with a diagnosis of polyarteritis nodosa. Although it is well recognised that Factor VIII inhibitors are found in conjunction with autoimmune disorders, this case is significant in that it is the first associated with histologically proven polyarteritis nodosa type vasculitis. The case illustrates the difficulties in the investigation and management of patients with acquired high titre Factor VIII inhibitors.

SGCE mutations cause psychiatric disorders: clinical and genetic characterization

PubMed Central

Peall, Kathryn J.; Smith, Daniel J.; Kurian, Manju A.; Wardle, Mark; Waite, Adrian J.; Hedderly, Tammy; Lin, Jean-Pierre; Smith, Martin; Whone, Alan; Pall, Hardev; White, Cathy; Lux, Andrew; Jardine, Philip; Bajaj, Narinder; Lynch, Bryan; Kirov, George; O’Riordan, Sean; Samuel, Michael; Lynch, Timothy; King, Mary D.; Chinnery, Patrick F.; Warner, Thomas T.; Blake, Derek J.; Owen, Michael J.; Morris, Huw R.

2014-01-01

Myoclonus dystonia syndrome is a childhood onset hyperkinetic movement disorder characterized by predominant alcohol responsive upper body myoclonus and dystonia. A proportion of cases are due to mutations in the maternally imprinted SGCE gene. Previous studies have suggested that patients with SGCE mutations may have an increased rate of psychiatric disorders. We established a cohort of patients with myoclonus dystonia syndrome and SGCE mutations to determine the extent to which psychiatric disorders form part of the disease phenotype. In all, 89 patients with clinically suspected myoclonus dystonia syndrome were recruited from the UK and Ireland. SGCE was analysed using direct sequencing and for copy number variants. In those patients where no mutation was found TOR1A (GAG deletion), GCH1, THAP1 and NKX2-1 were also sequenced. SGCE mutation positive cases were systematically assessed using standardized psychiatric interviews and questionnaires and compared with a disability-matched control group of patients with alcohol responsive tremor. Nineteen (21%) probands had a SGCE mutation, five of which were novel. Recruitment of family members increased the affected SGCE mutation positive group to 27 of whom 21 (77%) had psychiatric symptoms. Obsessive–compulsive disorder was eight times more likely (P < 0.001) in mutation positive cases, compulsivity being the predominant feature (P < 0.001). Generalized anxiety disorder (P = 0.003) and alcohol dependence (P = 0.02) were five times more likely in mutation positive cases than tremor controls. SGCE mutations are associated with a specific psychiatric phenotype consisting of compulsivity, anxiety and alcoholism in addition to the characteristic motor phenotype. SGCE mutations are likely to have a pleiotropic effect in causing both motor and specific psychiatric symptoms. PMID:23365103

Quantification and Systematic Characterization of Stuttering-Like Disfluencies in Acquired Apraxia of Speech.

PubMed

Bailey, Dallin J; Blomgren, Michael; DeLong, Catharine; Berggren, Kiera; Wambaugh, Julie L

2017-06-22

The purpose of this article is to quantify and describe stuttering-like disfluencies in speakers with acquired apraxia of speech (AOS), utilizing the Lidcombe Behavioural Data Language (LBDL). Additional purposes include measuring test-retest reliability and examining the effect of speech sample type on disfluency rates. Two types of speech samples were elicited from 20 persons with AOS and aphasia: repetition of mono- and multisyllabic words from a protocol for assessing AOS (Duffy, 2013), and connected speech tasks (Nicholas & Brookshire, 1993). Sampling was repeated at 1 and 4 weeks following initial sampling. Stuttering-like disfluencies were coded using the LBDL, which is a taxonomy that focuses on motoric aspects of stuttering. Disfluency rates ranged from 0% to 13.1% for the connected speech task and from 0% to 17% for the word repetition task. There was no significant effect of speech sampling time on disfluency rate in the connected speech task, but there was a significant effect of time for the word repetition task. There was no significant effect of speech sample type. Speakers demonstrated both major types of stuttering-like disfluencies as categorized by the LBDL (fixed postures and repeated movements). Connected speech samples yielded more reliable tallies over repeated measurements. Suggestions are made for modifying the LBDL for use in AOS in order to further add to systematic descriptions of motoric disfluencies in this disorder.

Rethinking responsibility in offenders with acquired paedophilia: punishment or treatment?

PubMed

Gilbert, Frédéric; Focquaert, Farah

2015-01-01

This article reviews the current neurobiological literature on the aetiology of developmental and acquired paedophilia and examines what the consequences could be in terms of responsibility and treatment for the latter. Addressing the question of responsibility and punishment of offenders with acquired paedophilia from a neurobiological perspective is controversial. Consequently it is essential to avoid hasty conclusions based strictly on neurobiological abnormality justifications. This study establishes a distinction between developmental and acquired paedophilia. The article investigates whether offenders who fulfil the diagnosis of acquired paedophilia should be held fully responsible, particularly in cases where the offender's conduct appears to result from volitionally controlled behaviour that is seemingly incompatible with a neurological cause. Moreover, the article explores how responsibility can be compromised when offenders with acquired paedophilia have (partially) preserved moral knowledge despite their sexual disorder. The article then examines the option of offering mandatory treatment as an alternative to imprisonment for offenders with acquired paedophilia. Furthermore, the article addresses the ethical issues related to offering any form of quasi-coercive treatment as a condition of release. This study concludes that decisions to fully or partially excuse an individual who fulfil the diagnosis of acquired paedophilia should take all relevant information into account, both neurobiological and other environmental evidence, and should proceed on a careful case by case analysis before sentencing or offering treatment. Copyright © 2015 Elsevier Ltd. All rights reserved.

Brain disorders and the biological role of music

PubMed Central

Clark, Camilla N.; Downey, Laura E.

2015-01-01

Despite its evident universality and high social value, the ultimate biological role of music and its connection to brain disorders remain poorly understood. Recent findings from basic neuroscience have shed fresh light on these old problems. New insights provided by clinical neuroscience concerning the effects of brain disorders promise to be particularly valuable in uncovering the underlying cognitive and neural architecture of music and for assessing candidate accounts of the biological role of music. Here we advance a new model of the biological role of music in human evolution and the link to brain disorders, drawing on diverse lines of evidence derived from comparative ethology, cognitive neuropsychology and neuroimaging studies in the normal and the disordered brain. We propose that music evolved from the call signals of our hominid ancestors as a means mentally to rehearse and predict potentially costly, affectively laden social routines in surrogate, coded, low-cost form: essentially, a mechanism for transforming emotional mental states efficiently and adaptively into social signals. This biological role of music has its legacy today in the disordered processing of music and mental states that characterizes certain developmental and acquired clinical syndromes of brain network disintegration. PMID:24847111

Characterizing Aggressive Behavior with the Impulsive/Premeditated Aggression Scale among Adolescents with Conduct Disorder

PubMed Central

Mathias, Charles W.; Stanford, Matthew S.; Marsh, Dawn M.; Frick, Paul J.; Moeller, F. Gerard; Swann, Alan C.; Dougherty, Donald M.

2007-01-01

This study extends the use of the Impulsive/Premeditated Aggression Scale for subtyping aggressive behavior among adolescents with Conduct Disorder. Of the Conduct Disorder symptoms, aggression has the strongest prognostic and treatment implications. While aggression is a complex construct, convergent evidence supports a dichotomy of impulsive and premeditated aggressive subtypes that are qualitatively different from one another in terms of phenomenology and neurobiology. Previous attempts at measuring subtypes of aggression in children and adults are not clearly generalizable to adolescents. Sixty-six adolescents completed a questionnaire for characterizing aggression (Impulsive/Premeditated Aggression Scale), along with standard measures of personality and general functioning. Principal components analysis demonstrated two stable factors of aggression with good internal consistency and construct validity. Compared to the premeditated aggression factor, the impulsive aggression factor was associated with a broader range of personality, thought, emotional, and social problems. As in the adult and child literature, characterization of aggressive behavior into two subtypes appears to be relevant to understanding individual differences among adolescents with Conduct Disorder. PMID:17383014

Statistically Characterizing Intra- and Inter-Individual Variability in Children with Developmental Coordination Disorder

ERIC Educational Resources Information Center

King, Bradley R.; Harring, Jeffrey R.; Oliveira, Marcio A.; Clark, Jane E.

2011-01-01

Previous research investigating children with Developmental Coordination Disorder (DCD) has consistently reported increased intra- and inter-individual variability during motor skill performance. Statistically characterizing this variability is not only critical for the analysis and interpretation of behavioral data, but also may facilitate our…

The Acquired Preparedness Model of Risk for Bulimic Symptom Development

PubMed Central

Combs, Jessica L.; Smith, Gregory T.; Flory, Kate; Simmons, Jean R.; Hill, Kelly K.

2010-01-01

The authors applied person-environment transaction theory to test the acquired preparedness model of eating disorder risk. The model holds that (a) middle school girls high in the trait of ineffectiveness are differentially prepared to acquire high risk expectancies for reinforcement from dieting/thinness; (b) those expectancies predict subsequent binge eating and purging; and (c) the influence of the disposition of ineffectiveness on binge eating and purging is mediated by dieting/thinness expectancies. In a three-wave longitudinal study of 394 middle school girls, they found support for the model. Seventh grade girls’ scores on ineffectiveness predicted their subsequent endorsement of high risk dieting/thinness expectancies, which in turn predicted subsequent increases in binge eating and purging. Statistical tests of mediation supported the hypothesis that the prospective relation between ineffectiveness and binge eating was mediated by dieting/thinness expectancies, as was the prospective relation between ineffectiveness and purging. This application of a basic science theory to eating disorder risk appears fruitful, and the findings suggest the importance of early interventions that address both disposition and learning. PMID:20853933

ADAMTS13 Autoantibodies Cloned from Patients with Acquired Thrombotic Thrombocytopenic Purpura: 1. Structural and functional characterization in vitro

PubMed Central

Ostertag, Eric M.; Kacir, Stephen; Thiboutot, Michelle; Gulendran, Gayathri; Zheng, X. Long; Cines, Douglas B.; Siegel, Don L.

2016-01-01

BACKGROUND Acquired thrombotic thrombocytopenia purpura (TTP) is a life-threatening illness caused by autoantibodies that decrease the activity of ADAMTS13, the von Willebrand Factor cleaving protease. Despite efficacy of plasma exchange, mortality remains high and relapse is common. Improved therapies may come from understanding the diversity of pathogenic autoantibodies on a molecular/genetic level. Cloning comprehensive repertoires of patient autoantibodies can provide the necessary tools for studying immunobiology of disease and developing animal models. STUDY DESIGN AND METHODS Anti-ADAMTS13 antibodies were cloned from four patients with acquired TTP using phage display and characterized with respect to genetic origin, inhibition of ADAMTS13 proteolytic activity, and epitope specificity. Anti-idiotypic antisera raised to a subset of autoantibodies enabled comparison of their relatedness to each other and to polyclonal IgG in patient plasma. RESULTS Fifty-one unique antibodies were isolated comprising epitope specificities resembling the diversity found in circulating patient IgG. Antibodies directed to both the amino terminal domains and those requiring the ADAMTS13 cysteine-rich/spacer region for binding inhibited proteolytic activity, while those solely targeting carboxy-terminal domains were non-inhibitory. Anti-idiotypic antisera raised to a subset of antibody clones crossreacted with and reduced the inhibitory activity of polyclonal IgG from a set of unrelated patients. CONCLUSIONS Anti-ADAMTS13 autoantibodies isolated by repertoire cloning display the diversity of epitope specificities found in patient plasma and provide tools for developing animal models of acquired TTP. Shared idiotypes of inhibitory clones with circulating IgG from multiple patients suggest common features of pathogenic autoantibodies that could be exploited for developing more targeted therapies. PMID:27040144

Ultrasound of Inherited vs. Acquired Demyelinating Polyneuropathies

PubMed Central

Zaidman, Craig M.; Harms, Matthew B.; Pestronk, Alan

2013-01-01

Introduction We compared features of nerve enlargement in inherited and acquired demyelinating neuropathies using ultrasound. Methods We measured median and ulnar nerve cross-sectional areas in proximal and distal regions in 128 children and adults with inherited (Charcot-Marie Tooth-1 (CMT-1) (n=35)) and acquired (Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) (n=55), Guillaine-Barre Syndrome (GBS) (n=21) and Multifocal Motor Neuropathy (MMN) (n=17)) demyelinating neuropathies. We classified nerve enlargement by degree and number of regions affected. We defined patterns of nerve enlargement as: none- no enlargement; mild-nerves enlarged but never more than twice normal; regional- nerves normal at at least one region and enlarged more than twice normal at atleast one region; diffuse- nerves enlarged at all four regions with atleast one region more than twice normal size. Results Nerve enlargement was commonly diffuse (89%) and generally more than twice normal size in CMT-1, but not (p<0.001) in acquired disorders which mostly had either no, mild or regional nerve enlargement (CIDP (64%), GBS (95%), and MMN (100%)). In CIDP, subjects treated within three months of disease onset had less nerve enlargement than those treated later. Discussion Ultrasound identified patterns of diffuse nerve enlargement can be used to screen patients suspected of having CMT-1. Normal, mildly, or regionally enlarged nerves in demyelinating polyneuropathy suggests an acquired etiology. Early treatment in CIDP may impede nerve enlargement. PMID:24101129

Characterizing adult attention-deficit/hyperactivity-disorder and comorbid borderline personality disorder: ADHD symptoms, psychopathology, cognitive functioning and psychosocial factors.

PubMed

O'Malley, G K; McHugh, L; Mac Giollabhui, N; Bramham, J

2016-01-01

To characterize adults with comorbid attention-deficit/hyperactivity-disorder (ADHD) and borderline personality disorder (BPD) with regard to ADHD symptoms, psychopathology, cognitive functioning and psychosocial factors. A between-group design compared a group of individuals diagnosed with ADHD (n=40) with a group diagnosed with BPD and who also met the criteria for ADHD (ADHD+BPD) (n=20). Significant differences were observed for both childhood and current impulsivity symptoms, whereby ADHD+BPD exhibited increased impulsivity; no differences on self-report and cognitive measures of impulsivity were reported. The ADHD+BPD group scored significantly higher on measures of depression, anxiety and numerous other axis I and II conditions. The ADHD+BPD group scored significantly lower on most measures of intellectual functioning and attention, however largely not on those relating to response inhibition. Furthermore, group differences were observed for psychosocial factors, including education, substance use and criminal record. Comorbid ADHD and BPD is characterized by more symptoms of impulsivity, additional psychopathology, comparatively lower intellectual and attentional functioning and increased psychosocial difficulties. Copyright © 2015. Published by Elsevier Masson SAS.

Interventions for treating acute bleeding episodes in people with acquired hemophilia A.

PubMed

Zeng, Yan; Zhou, Ruiqing; Duan, Xin; Long, Dan; Yang, Songtao

2014-08-28

Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies to coagulation factor VIII (FVIII). In most cases, bleeding episodes are spontaneous and severe at presentation. The optimal hemostatic therapy is controversial. To determine the efficacy of hemostatic therapies for acute bleeds in people with acquired hemophilia A; and to compare different forms of therapy for these bleeds. We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library 2014, Issue 4) and MEDLINE (Ovid) (1948 to 30 April 2014). We searched the conference proceedings of the: American Society of Hematology; European Hematology Association; International Society on Thrombosis and Haemostasis (ISTH); and the European Association for Haemophilia and Allied Disorders (EAHAD) (from 2000 to 30 April 2014). In addition to this we searched clinical trials registers. All randomised controlled trials and quasi-randomised trials of hemostatic therapies for people with acquired hemophilia A, with no restrictions on gender, age or ethnicity. No trials matching the selection criteria were eligible for inclusion. No trials matching the selection criteria were eligible for inclusion. No randomised clinical trials of hemostatic therapies for acquired hemophilia A were found. Thus, we are not able to draw any conclusions or make any recommendations on the optimal hemostatic therapies for acquired hemophilia A based on the highest quality of evidence. GIven that carrying out randomized controlled trials in this field is a complex task, the authors suggest that, while planning randomised controlled trials in which patients can be enrolled, clinicians treating the disease continue to base their choices on alternative, lower quality sources of evidence, which hopefully, in the future, will also be appraised and incorporated in a Cochrane Review.

An Acquired Deficit of Audiovisual Speech Processing

ERIC Educational Resources Information Center

Hamilton, Roy H.; Shenton, Jeffrey T.; Coslett, H. Branch

2006-01-01

We report a 53-year-old patient (AWF) who has an acquired deficit of audiovisual speech integration, characterized by a perceived temporal mismatch between speech sounds and the sight of moving lips. AWF was less accurate on an auditory digit span task with vision of a speaker's face as compared to a condition in which no visual information from…

[Ejaculatory disorders except premature ejaculation, orgasmic disorders].

PubMed

Rigot, J-M; Marcelli, F; Giuliano, F

2013-07-01

Disorders of ejaculation and orgasm apart from premature ejaculation are pretty uncommon. Medical literature was reviewed and combined with expert opinion of the authors. The semiology of these disorders is essential: aspermia, hypospermia, retrograde ejaculation, delayed or absent ejaculation with or without orgasm. Whether this is a lifelong or acquired condition, it is essential to assess the side-effects of medications i.e. psychotropic drugs, including antidepressant, neuroleptics, tramadol, alphablockers: tamsulosin and silodosin must always be surveyed. The management is often difficult, especially with a parenthood perspective. The management of lifelong disorders must rely on psychosexual therapies. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

International recommendations on the diagnosis and treatment of patients with acquired hemophilia A

PubMed Central

Huth-Kühne, Angela; Baudo, Francesco; Collins, Peter; Ingerslev, Jørgen; Kessler, Craig M.; Lévesque, Hervé; Castellano, Maria Eva Mingot; Shima, Midori; St-Louis, Jean

2009-01-01

Acquired hemophilia A (AHA) is a rare bleeding disorder characterized by autoantibodies directed against circulating coagulation factor (F) VIII. Typically, patients with no prior history of a bleeding disorder present with spontaneous bleeding and an isolated prolonged aPTT. AHA may, however, present without any bleeding symptoms, therefore an isolated prolonged aPTT should always be investigated further irrespective of the clinical findings. Control of acute bleeding is the first priority, and we recommend first-line therapy with bypassing agents such as recombinant activated FVII or activated prothrombin complex concentrate. Once the diagnosis has been achieved, immediate autoantibody eradication to reduce subsequent bleeding risk should be performed. We recommend initial treatment with corticosteroids or combination therapy with corticosteroids and cyclophosphamide and suggest second-line therapy with rituximab if first-line therapy fails or is contraindicated. In contrast to congenital hemophilia, no comparative studies exist to support treatment recommendations for patients with AHA, therefore treatment guidance must rely on the expertise and clinical experience of specialists in the field. The aim of this document is to provide a set of international practice guidelines based on our collective clinical experience in treating patients with AHA and contribute to improved care for this patient group. PMID:19336751

Cross-sectional imaging of congenital and acquired abnormalities of the portal venous system

PubMed Central

Özbayrak, Mustafa; Tatlı, Servet

2016-01-01

Knowing the normal anatomy, variations, congenital and acquired pathologies of the portal venous system are important, especially when planning liver surgery and percutaneous interventional procedures. The portal venous system pathologies can be congenital such as agenesis of portal vein (PV) or can be involved by other hepatic disorders such as cirrhosis and malignancies. In this article, we present normal anatomy, variations, and acquired pathologies involving the portal venous system as seen on computed tomography (CT) and magnetic resonance imaging (MRI). PMID:27731302

Cholestasis caused by panhypopituitarism and acquired cytomegalovirus infection in a 2-month-old male infant

PubMed Central

Chan, U; Chan, Wai-Tao; Ting, Wei-Hsin; Ho, Che-Sheng; Liu, Hsi-Che; Lee, Hung-Chang

2017-01-01

Abstract Rationale: Septo-optic dysplasia (SOD) is a rare congenital disorder that may cause jaundice in infants. However, it is usually prone to neglect and misdiagnosis in infants with cholestasis because endocrine disorder such as panhypopituitarism is rare in the cause of infantile cholestasis. We report a case of SOD concurrent with acquired cytomegalovirus (CMV) infection, who presented with prolonged jaundice as the first clinical sign. Patient concerns: The patient was a 2-month-old male infant who presented with cholestasis, combined with fever and panhypopituitarism. Diagnoses: He was diagnosed with SOD and acquired CMV infection. Interventions: He was treated with hormone replacement therapy and ganciclovir. Outcomes: After correction of the pituitary hormone deficiency and ganciclovir treatment, significant improvements of cholestasis, retinal lesions, and growth rate were seen in our patient. Lessons: Although an endocrine disorder such as panhypopituitarism is rare in the cause of neonatal or infantile cholestasis, we must keep this reason in mind. PMID:28445302

Brain disorders and the biological role of music.

PubMed

Clark, Camilla N; Downey, Laura E; Warren, Jason D

2015-03-01

Despite its evident universality and high social value, the ultimate biological role of music and its connection to brain disorders remain poorly understood. Recent findings from basic neuroscience have shed fresh light on these old problems. New insights provided by clinical neuroscience concerning the effects of brain disorders promise to be particularly valuable in uncovering the underlying cognitive and neural architecture of music and for assessing candidate accounts of the biological role of music. Here we advance a new model of the biological role of music in human evolution and the link to brain disorders, drawing on diverse lines of evidence derived from comparative ethology, cognitive neuropsychology and neuroimaging studies in the normal and the disordered brain. We propose that music evolved from the call signals of our hominid ancestors as a means mentally to rehearse and predict potentially costly, affectively laden social routines in surrogate, coded, low-cost form: essentially, a mechanism for transforming emotional mental states efficiently and adaptively into social signals. This biological role of music has its legacy today in the disordered processing of music and mental states that characterizes certain developmental and acquired clinical syndromes of brain network disintegration. © The Author (2014). Published by Oxford University Press.

Motor learning characterization in people with autism spectrum disorder: A systematic review

PubMed Central

de Moraes, Íbis Ariana Peña; Massetti, Thais; Crocetta, Tânia Brusque; da Silva, Talita Dias; de Menezes, Lilian Del Ciello; Monteiro, Carlos Bandeira de Mello; Magalhães, Fernando Henrique

2017-01-01

ABSTRACT Autism Spectrum Disorder (ASD) is a neurodevelopmental disorder primarily characterized by deficits in social interaction, communication and implicit skill learning. OBJECTIVE: To analyse the results of research on "motor learning" and the means used for measuring "autistic disorder". METHODS: A systematic literature search was done using Medline/PubMed, Web of Science, BVS (virtual health library), and PsycINFO. We included articles that contained the keywords "autism" and "motor learning". The variables considered were the methodological aspects; results presented, and the methodological quality of the studies. RESULTS: A total of 42 studies were identified; 33 articles were excluded because they did not meet the inclusion criteria. Data were extracted from nine eligible studies and summarized. CONCLUSION: We concluded that although individuals with ASD showed performance difficulties in different memory and motor learning tasks, acquisition of skills still takes place in this population; however, this skill acquisition is related to heterogeneous events, occurring without the awareness of the individual. PMID:29213525

Epidemiology of Autism Spectrum Disorder in Portugal: Prevalence, Clinical Characterization, and Medical Conditions

ERIC Educational Resources Information Center

Oliveira, Guiomar; Ataide, Assuncao; Marques, Carla; Miguel, Teresa S.; Coutinho, Ana Margarida; Mota-Vieira, Luisa; Goncalves, Esmeralda; Lopes, Nazare Mendes; Rodrigues, Vitor; Carmona da Mota, Henrique; Vicente, Astrid Moura

2007-01-01

The objective of this study was to estimate the prevalence of autistic spectrum disorder (ASD) and identify its clinical characterization, and medical conditions in a paediatric population in Portugal. A school survey was conducted in elementary schools, targeting 332 808 school-aged children in the mainland and 10 910 in the Azores islands.…

Characterization and Factors Associated with Sleep Quality in Adolescents with Bipolar I Disorder

ERIC Educational Resources Information Center

Roybal, Donna J.; Chang, Kiki D.; Chen, Michael C.; Howe, Meghan E.; Gotlib, Ian H.; Singh, Manpreet K.

2011-01-01

Sleep disturbance is an early marker for bipolar disorder (BD) onset in youth. We characterized sleep quality in adolescents experiencing mania within the last 6-12 months. We examined the association between mood and sleep in 27 adolescents with BD and 24 matched healthy controls (HC). Subjects were assessed by parent and teen report of sleep, a…

Management of Acquired Atresia of the External Auditory Canal.

PubMed

Bajin, Münir Demir; Yılmaz, Taner; Günaydın, Rıza Önder; Kuşçu, Oğuz; Sözen, Tevfik; Jafarov, Shamkal

2015-08-01

The aim was to evaluate surgical techniques and their relationship to postoperative success rate and hearing outcomes in acquired atresia of the external auditory canal. In this article, 24 patients with acquired atresia of the external auditory canal were retrospectively evaluated regarding their canal status, hearing, and postoperative success. Acquired stenosis occurs more commonly in males with a male: female ratio of 2-3:1; it seems to be a disorder affecting young adults. Previous ear surgery (13 patients, 54.2%) and external ear trauma (11 patients, 45.8%) were the main etiological factors of acquired ear canal stenosis. Mastoidectomy (12/13) and traffic accidents (8/11) comprise the majority of these etiological factors. Endaural incision is performed in 79.2% and postauricular incision for 20.8% of cases during the operation. As types of surgical approach, transcanal (70.8%), transmastoid (20.8%), and combined (8.4%) approaches are chosen. The atretic plate is generally located at the bony-cartilaginous junction (37.5%) and in the cartilaginous canal (33.3%); the bony canal is involved in a few cases only. Preserved healthy canal skin, split- or full-thickness skin grafts, or pre- or postauricular skin flaps are used to line the ear canal, but preserved healthy canal skin is preferred. The results of surgery are generally satisfactory, and complications are few if surgical principles are followed.

Neurological Correlates of Reward Responding in Adolescents With and Without Externalizing Behavior Disorders

PubMed Central

Gatzke-Kopp, Lisa M.; Beauchaine, Theodore P.; Shannon, Katherine E.; Chipman, Jane; Fleming, Andrew P.; Crowell, Sheila E.; Liang, Olivia; Aylward, Elizabeth; Johnson, L. Clark

2009-01-01

Opposing theories of striatal hyper- and hypodopaminergic functioning have been suggested in the pathophysiology of externalizing behavior disorders. To test these competing theories, the authors used functional MRI to evaluate neural activity during a simple reward task in 12- to 16-year-old boys with attention-deficit/hyperactivity disorder and/or conduct disorder (n = 19) and in controls with no psychiatric condition (n = 11). The task proceeded in blocks during which participants received either (a) monetary incentives for correct responses or (b) no rewards for correct responses. Controls exhibited striatal activation only during reward, shifting to anterior cingulate activation during nonreward. In contrast, externalizing adolescents exhibited striatal activation during both reward and nonreward. Externalizing psychopathology appears to be characterized by deficits in processing the omission of predicted reward, which may render behaviors that are acquired through environmental contingencies difficult to extinguish when those contingencies change. PMID:19222326

Recombinant factor VIIa (eptacog alfa): a review of its use in congenital hemophilia with inhibitors, acquired hemophilia, and other congenital bleeding disorders.

PubMed

Croom, Katherine F; McCormack, Paul L

2008-01-01

Recombinant factor VIIa (NovoSeven; also known as recombinant activated factor VII or eptacog alfa) is structurally similar to human plasma-derived coagulation factor VIIa, but is manufactured using DNA biotechnology. Recombinant factor VIIa interacts with thrombin-activated platelets to produce a thrombin burst leading to accelerated fibrin clot formation localized to the site of vascular injury. It is approved in many countries for use as an intravenous hemostatic agent in patients with congenital hemophilia with inhibitors, and also for acquired hemophilia, factor VII deficiency, and Glanzmann thrombasthenia in some countries. Studies have shown it to be effective and generally well tolerated when used intravenously to treat bleeding episodes or provide hemostatic cover during surgery in patients with congenital hemophilia with inhibitors, acquired hemophilia, factor VII deficiency or Glanzmann thrombasthenia. Based on available data, its efficacy in terms of patient-assessed response may be similar to that of activated prothrombin complex concentrate (aPCC), but treatment with a single 270 microg/kg dose of recombinant factor VIIa might reduce the need for rescue therapy compared with aPCC. Recombinant factor VIIa is not immunogenic in patients with hemophilia, does not produce an anamnestic response in hemophilia patients with inhibitors, and has very low thrombogenicity. It is recommended in guidelines as the treatment of choice for bleeds in patients with hemophilia B with high-responding inhibitors and for patients with factor VII deficiency, and is also a first-line therapeutic option for high-responder hemophilia A patients with inhibitors and those with acquired hemophilia. Cost data from pharmacoeconomic analyses support its use in hemophilia patients with inhibitors. Thus, recombinant factor VIIa is a valuable treatment option for patients with these rare, but potentially serious, bleeding disorders.

Intensive Care Unit–Acquired Weakness: Implications for Physical Therapist Management

PubMed Central

Moss, Marc; Quan, Dianna; Schenkman, Margaret

2012-01-01

Patients admitted to the intensive care unit (ICU) can develop a condition referred to as “ICU-acquired weakness.” This condition is characterized by profound weakness that is greater than might be expected to result from prolonged bed rest. Intensive care unit–acquired weakness often is accompanied by dysfunction of multiple organ systems. Individuals with ICU-acquired weakness typically have significant activity limitations, often requiring physical assistance for even the most basic activities associated with bed mobility. Many of these individuals have activity limitations months to years after hospitalization. The purpose of this article is to review evidence that guides physical rehabilitation of people with ICU-acquired weakness. Included are diagnostic criteria, medical management, and prognostic indicators, as well as criteria for beginning physical rehabilitation, with an emphasis on patient safety. Data are presented indicating that rehabilitation can be implemented with very few adverse effects. Evidence is provided for appropriate measurement approaches and for physical intervention strategies. Finally, some of the key issues are summarized that should be investigated to determine the best intervention guidelines for individuals with ICU-acquired weakness. PMID:22282769

Male acquired hypogonadotropic hypogonadism: diagnosis and treatment.

PubMed

Salenave, Sylvie; Trabado, Sévérine; Maione, Luigi; Brailly-Tabard, Sylvie; Young, Jacques

2012-04-01

Acquired hypogonadotropic hypogonadism (AHH), contrary to congenital hypogonadotropic hypogonadism (CHH) is characterized by postnatal onset of disorders that damage or alter the function of gonadotropin-releasing hormone (GnRH) neurons and/or pituitary gonadotroph cells. AHH thus prevents the establishment of gonadotropin secretion at puberty, or its post-pubertal maintenance. Thus, postnatal AHH may prevent the onset of puberty or appear during pubertal development, but it usually emerges after the normal age of puberty. Although pituitary tumors, particularly prolactinoma, are the most common cause, sellar tumors or cyst of the hypothalamus or infundibulum, infiltrative, vascular, iron overload and other disorders may also cause AHH. Pituitary surgery and head trauma or cranial/pituitary radiation therapy are also usual causes of AHH. The clinical manifestations of AHH depend on age of onset, the degree of gonadotropin deficiency, the rapidity of its onset and the association to other pituitary function deficiencies or excess. Men with AHH have less stamina, decreased libido, erectile dysfunction and strength, and a worsened sense of well being leading to degraded quality of life. The physical examination is usually normal if hypogonadism is of recent onset. Diminished facial, body hair and muscle mass, fine facial wrinkles, gynecomastia, and hypotrophic testes are observed in long-standing and complete AHH. Spermatogenesis is impaired and the volume of ejaculate is decreased only when gonadotropins and testosterone levels are very low. Men with AHH may have normal or low serum LH and FSH concentrations, but normal gonadotropin values are inappropriate when associated with low serum testosterone. In the majority of AHH patients, serum inhibin B is "normal". The decrease of this sertolian hormone indicates a long-standing and severe gonadotropin deficiency. Symptoms, usually associated with significant testosterone deficiency in men with AHH, improve with

Electroencephalographic characterization of subgroups of children with learning disorders

PubMed Central

Roca-Stappung, Milene; Bosch-Bayard, Jorge; Harmony, Thalía; Ricardo-Garcell, Josefina

2017-01-01

Electroencephalographic alterations have been reported in subjects with learning disorders, but there is no consensus on what characterizes their electroencephalogram findings. Our objective was to determine if there were subgroups within a group of scholars with not otherwise specified learning disorders and if they had specific electroencephalographic patterns. Eighty-five subjects (31 female, 8–11 years) who scored low in at least two subscales -reading, writing and arithmetic- of the Infant Neuropsychological Evaluation were included. Electroencephalograms were recorded in 19 leads during rest with eyes closed; absolute power was obtained every 0.39 Hz. Three subgroups were formed according to children’s performance: Group 1 (G1, higher scores than Group 2 in reading speed and reading and writing accuracy), Group 2 (G2, better performance than G1 in composition) and Group 3 (G3, lower scores than Groups 1 and 2 in the three subscales). G3 had higher absolute power in frequencies in the delta and theta range at left frontotemporal sites than G1 and G2. G2 had higher absolute power within alpha frequencies than G3 and G1 at the left occipital site. G3 had higher absolute power in frequencies in the beta range than G1 in parietotemporal areas and than G2 in left frontopolar and temporal sites. G1 had higher absolute power within beta frequencies than G2 in the left frontopolar site. G3 had lower gamma absolute power values than the other groups in the left hemisphere, and gamma activity was higher in G1 than in G2 in frontopolar and temporal areas. This group of children with learning disorders is very heterogeneous. Three subgroups were found with different cognitive profiles, as well as a different electroencephalographic pattern. It is important to consider these differences when planning interventions for children with learning disorders. PMID:28708890

Basic Reading Skills in High-Functioning Swedish Children with Autism Spectrum Disorders or Attention Disorder

ERIC Educational Resources Information Center

Asberg, Jakob; Dahlgren, SvenOlof; Dahlgren Sandberg, Annika

2008-01-01

High-functioning children with autism spectrum disorders (ASD) have been reported to have an early success in reading. Children with attention disorders such as DAMP or ADHD, on the other hand, often struggle acquiring reading skills. The primary aim of the study was two-fold: (a) to compare reading performance of children with ASD, DAMP and…

Cholestasis caused by panhypopituitarism and acquired cytomegalovirus infection in a 2-month-old male infant: A case report.

PubMed

Chan, U; Chan, Wai-Tao; Ting, Wei-Hsin; Ho, Che-Sheng; Liu, Hsi-Che; Lee, Hung-Chang

2017-04-01

Septo-optic dysplasia (SOD) is a rare congenital disorder that may cause jaundice in infants. However, it is usually prone to neglect and misdiagnosis in infants with cholestasis because endocrine disorder such as panhypopituitarism is rare in the cause of infantile cholestasis. We report a case of SOD concurrent with acquired cytomegalovirus (CMV) infection, who presented with prolonged jaundice as the first clinical sign. The patient was a 2-month-old male infant who presented with cholestasis, combined with fever and panhypopituitarism. He was diagnosed with SOD and acquired CMV infection. He was treated with hormone replacement therapy and ganciclovir. After correction of the pituitary hormone deficiency and ganciclovir treatment, significant improvements of cholestasis, retinal lesions, and growth rate were seen in our patient. Although an endocrine disorder such as panhypopituitarism is rare in the cause of neonatal or infantile cholestasis, we must keep this reason in mind.

Differences in microbiological profile between community-acquired, healthcare-associated and hospital-acquired infections.

PubMed

Cardoso, Teresa; Ribeiro, Orquídea; Aragão, Irene; Costa-Pereira, Altamiro; Sarmento, António

2013-01-01

Microbiological profiles were analysed and compared for intra-abdominal, urinary, respiratory and bloodstream infections according to place of acquisition: community-acquired, with a separate analysis of healthcare-associated, and hospital-acquired. Prospective cohort study performed at a university tertiary care hospital over 1 year. Inclusion criteria were meeting the Centers for Disease Control definition of intra-abdominal, urinary, respiratory and bloodstream infections. A total of 1035 patients were included in the study. More than 25% of intra-abdominal infections were polymicrobial; multi-drug resistant gram-negatives were 38% in community-acquired, 50% in healthcare-associated and 57% in hospital-acquired. E. coli was the most prevalent among urinary infections: 69% in community-acquired, 56% in healthcare-associated and 26% in hospital-acquired; ESBL producers' pathogens were 10% in healthcare-associated and 3% in community-acquired and hospital-acquired. In respiratory infections Streptococcus pneumoniae was the most prevalent in community-acquired (54%) and MRSA in healthcare-associated (24%) and hospital-acquired (24%). A significant association was found between MRSA respiratory infection and hospitalization in the previous year (adjusted OR = 6.3), previous instrumentation (adjusted OR = 4.3) and previous antibiotic therapy (adjusted OR = 5.7); no cases were documented among patients without risk factors. Hospital mortality rate was 10% in community-acquired, 14% in healthcare-associated and 19% in hospital-acquired infection. This study shows that healthcare-associated has a different microbiologic profile than those from community or hospital acquired for the four main focus of infection. Knowledge of this fact is important because the existing guidelines for community-acquired are not entirely applicable for this group of patients.

Risk factors for acquired myasthenia gravis in dogs: 1,154 cases (1991-1995).

PubMed

Shelton, G D; Schule, A; Kass, P H

1997-12-01

To determine frequency of initial clinical signs and risk factors for acquired myasthenia gravis (MG) in dogs. Retrospective study. 1,154 dogs residing within the United States from 1991 to 1995 with a confirmed diagnosis of acquired MG and 7,176 dogs with other neuromuscular disorders, including generalized weakness, megaesophagus, and dysphagia (control group). Records were retrieved from a database containing results of serum samples tested for acetylcholine receptor antibodies. Signalment, breed, age, state of origin, and month of onset of clinical signs were obtained. An antibody titer > 0.6 nmol/L was diagnostic for acquired MG. Unconditional logistic regression was used for statistical analysis. In comparison with mixed-breed dogs, dogs with the highest risk of acquired MG were Akitas, terrier group, Scottish Terriers, German Shorthaired Pointers, and Chihuahuas. Rottweilers, Doberman Pinschers, Dalmatians, and Jack Russell Terriers had low relative risks. Sexually intact males and dogs less than 1 year old had some protection from risk. Generalized weakness with megaesophagus and megaesophagus alone were the most common initial clinical signs. Breed predispositions for acquired MG were demonstrated. Age and sex were contributing factors. Although most dogs had generalized clinical signs, a substantial proportion of dogs had focal signs.

Prospective longitudinal evaluation of the effect of deployment-acquired traumatic brain injury on posttraumatic stress and related disorders: results from the Army Study to Assess Risk and Resilience in Servicemembers (Army STARRS).

PubMed

Stein, Murray B; Kessler, Ronald C; Heeringa, Steven G; Jain, Sonia; Campbell-Sills, Laura; Colpe, Lisa J; Fullerton, Carol S; Nock, Matthew K; Sampson, Nancy A; Schoenbaum, Michael; Sun, Xiaoying; Thomas, Michael L; Ursano, Robert J

2015-11-01

Traumatic brain injury (TBI) is increasingly recognized as a risk factor for deleterious mental health and functional outcomes. The purpose of this study was to examine the strength and specificity of the association between deployment-acquired TBI and subsequent posttraumatic stress and related disorders among U.S. Army personnel. A prospective, longitudinal survey of soldiers in three Brigade Combat Teams was conducted 1-2 months prior to an average 10-month deployment to Afghanistan (T0), upon redeployment to the United States (T1), approximately 3 months later (T2), and approximately 9 months later (T3). Outcomes of interest were 30-day prevalence postdeployment of posttraumatic stress disorder (PTSD), major depressive episode, generalized anxiety disorder, and suicidality, as well as presence and severity of postdeployment PTSD symptoms. Complete information was available for 4,645 soldiers. Approximately one in five soldiers reported exposure to mild (18.0%) or more-than-mild (1.2%) TBI(s) during the index deployment. Even after adjusting for other risk factors (e.g., predeployment mental health status, severity of deployment stress, prior TBI history), deployment-acquired TBI was associated with elevated adjusted odds of PTSD and generalized anxiety disorder at T2 and T3 and of major depressive episode at T2. Suicidality risk at T2 appeared similarly elevated, but this association did not reach statistical significance. The findings highlight the importance of surveillance efforts to identify soldiers who have sustained TBIs and are therefore at risk for an array of postdeployment adverse mental health outcomes, including but not limited to PTSD. The mechanism(s) accounting for these associations need to be elucidated to inform development of effective preventive and early intervention programs.

Acquired reactive perforating collagenosis and pseudoporphyric bullous dermatosis in a hemodialysis patient.

PubMed

Sabanis, Nikos; Paschou, Eleni; Gavriilaki, Eleni; Kalaitzoglou, Asterios; Papanikolaou, Dimitrios; Vasileiou, Sotiris; Amplianitis, Ioannis

2016-07-01

Hemodialysis patients present with a broad spectrum of specific and nonspecific skin disorders, which rarely coexist. We report an exceptional case of a hemodialysis patient that developed acquired reactive perforating collagenosis and pseudoporphyric bullous dermatosis on the basis of common skin disorders which include hyperpigmentation, pruritus, xerosis cutis, and Linsday's nails. Interestingly, our patient presented with two unusual but distinctive cutaneous dermopathies on the background of other commonly seen skin alterations. The patient was successfully treated with allopurinol and N-acetylcysteine. Avoidance of potentially triggering factors such as alcohol, sunlight exposure and certain medication was recommended. Thus, increasing clinical awareness, assiduous investigation and early treatment of skin disorders are required to improve the prognosis and quality of life in this patient population. © 2016 International Society for Hemodialysis.

Foodborne listeriosis acquired in hospitals.

PubMed

Silk, Benjamin J; McCoy, Morgan H; Iwamoto, Martha; Griffin, Patricia M

2014-08-15

Listeriosis is characterized by bacteremia or meningitis. We searched for listeriosis case series and outbreak investigations published in English by 2013, and assessed the strength of evidence for foodborne acquisition among patients who ate hospital food. We identified 30 reports from 13 countries. Among the case series, the median proportion of cases considered to be hospital-acquired was 25% (range, 9%-67%). The median number of outbreak-related illnesses considered to be hospital-acquired was 4.0 (range, 2-16). All patients were immunosuppressed in 18 of 24 (75%) reports with available data. Eight outbreak reports with strong evidence for foodborne acquisition in a hospital implicated sandwiches (3 reports), butter, precut celery, Camembert cheese, sausage, and tuna salad (1 report each). Foodborne acquisition of listeriosis among hospitalized patients is well documented internationally. The number of listeriosis cases could be reduced substantially by establishing hospital policies for safe food preparation for immunocompromised patients and by not serving them higher-risk foods. Published by Oxford University Press on behalf of the Infectious Diseases Society of America 2014. This work is written by (a) US Government employee(s) and is in the public domain in the US.

Neural correlates of apathy in patients with neurodegenerative disorders, acquired brain injury, and psychiatric disorders.

PubMed

Kos, Claire; van Tol, Marie-José; Marsman, Jan-Bernard C; Knegtering, Henderikus; Aleman, André

2016-10-01

Apathy can be described as a loss of goal-directed purposeful behavior and is common in a variety of neurological and psychiatric disorders. Although previous studies investigated associations between abnormal brain functioning and apathy, it is unclear whether the neural basis of apathy is similar across different pathological conditions. The purpose of this systematic review was to provide an extensive overview of the neuroimaging literature on apathy including studies of various patient populations, and evaluate whether the current state of affairs suggest disorder specific or shared neural correlates of apathy. Results suggest that abnormalities within fronto-striatal circuits are most consistently associated with apathy across the different pathological conditions. Of note, abnormalities within the inferior parietal cortex were also linked to apathy, a region previously not included in neuroanatomical models of apathy. The variance in brain regions implicated in apathy may suggest that different routes towards apathy are possible. Future research should investigate possible alterations in different processes underlying goal-directed behavior, ranging from intention and goal-selection to action planning and execution. Copyright © 2016. Published by Elsevier Ltd.

Mitochondrial transfer RNA(Phe) mutation associated with a progressive neurodegenerative disorder characterized by psychiatric disturbance, dementia, and akinesia-rigidity.

PubMed

Young, Tim M; Blakely, Emma L; Swalwell, Helen; Carter, Janet E; Kartsounis, Luke D; O'Donovan, Dominic G; Turnbull, Douglass M; Taylor, Robert W; de Silva, Rajith N

2010-11-01

Mitochondrial diseases are characterized by wide phenotypic and genetic variability, but presentations in adults with akinetic rigidity and hyperkinetic movement disorders are rare. To describe clinically a subject with progressive neurodegeneration characterized by psychosis, dementia, and akinesia-rigidity, and to associate this phenotype with a novel mitochondrial transfer RNA(Phe) (tRNA(Phe)) (MTTF) mutation. Case description and detailed laboratory investigations of a 57-year-old woman at a university teaching hospital and a specialist mitochondrial diagnostic laboratory. Histopathological findings indicated that an underlying mitochondrial abnormality was responsible for the subject's progressive neurological disorder, with mitochondrial genome sequencing revealing a novel m.586G>A MTTF mutation. The clinical phenotypes associated with mitochondrial disorders may include akinesia-rigidity and psychosis. Our findings further broaden the spectrum of neurological disease associated with mitochondrial tRNA(Phe) mutations.

Anxiety Disorders among US Immigrants: The Role of Immigrant Background and Social-Psychological Factors.

PubMed

Szaflarski, Magdalena; Cubbins, Lisa A; Meganathan, Karthikeyan

2017-04-01

This study used the National Epidemiological Survey on Alcohol and Related Conditions, a longitudinal adult sample, to estimate the rates of prevalent, acquired, and persisting anxiety disorders by nativity and racial-ethnic origin while adjusting for acculturation, stress, social ties, and sociodemographics. Prevalent and acquired anxiety disorders were less likely among foreign-born than US-born, except Puerto-Rican- and Mexican-born who had higher risks. Persisting cases were similar between foreign-born and US-born, except Asian/Pacific Islanders who had lower risk. Stress and preference for socializing outside one's racial-ethnic group were associated with higher while close ties were associated with lower rates of acquired/persisting anxiety disorders.

FDG PET/CT findings in acquired perforating dermatosis.

PubMed

Shinmura, Akiko; Abe, Koichiro; Baba, Shingo; Isoda, Takuro; Maruoka, Yasuhiro; Yasukawa, Fumiko; Kiryu, Hiromaro; Sasaki, Masayuki; Furue, Masutaka; Honda, Hiroshi

2012-10-01

Acquired perforating dermatosis (APD) is an uncommon cutaneous perforating disorder. We report a patient on hemodialysis who developed skin eruption and jaundice. He underwent FDG PET/CT under suspicion of biliary malignancies. PET/CT showed no significant abnormal uptake except of multiple FDG-avid nodules in the skin. The eruption he had was histopathologically diagnosed as APD by skin biopsy. His case suggests that APD should be considered as a differential diagnosis when multiple cutaneous FDG accumulations are found in a patient on hemodialysis. To the best of our knowledge, this is the first report showing the FDG PET/CT findings of APD.

Case against Diagnosing Developmental Language Disorder by Default: A Single Case Study of Acquired Aphasia Associated with Convulsive Disorder

ERIC Educational Resources Information Center

Marinac, Julie V.; Harper, Laura

2009-01-01

The aim of this article is to inform the diagnostic knowledge base for professionals working in the field of language disorders when classic symptoms, characteristics and sequences are not found. The information reveals the risk of diagnosis with a developmental language disorder (DLD) by default when no underlying cause can be readily identified.…

17 CFR 210.8-06 - Real estate operations acquired or to be acquired.

Code of Federal Regulations, 2012 CFR

2012-04-01

... 17 Commodity and Securities Exchanges 2 2012-04-01 2012-04-01 false Real estate operations acquired or to be acquired. 210.8-06 Section 210.8-06 Commodity and Securities Exchanges SECURITIES AND...-06 Real estate operations acquired or to be acquired. If, during the period for which income...

17 CFR 210.8-06 - Real estate operations acquired or to be acquired.

Code of Federal Regulations, 2013 CFR

2013-04-01

... 17 Commodity and Securities Exchanges 2 2013-04-01 2013-04-01 false Real estate operations acquired or to be acquired. 210.8-06 Section 210.8-06 Commodity and Securities Exchanges SECURITIES AND...-06 Real estate operations acquired or to be acquired. If, during the period for which income...

17 CFR 210.8-06 - Real estate operations acquired or to be acquired.

Code of Federal Regulations, 2014 CFR

2014-04-01

... 17 Commodity and Securities Exchanges 3 2014-04-01 2014-04-01 false Real estate operations acquired or to be acquired. 210.8-06 Section 210.8-06 Commodity and Securities Exchanges SECURITIES AND...-06 Real estate operations acquired or to be acquired. If, during the period for which income...

Acquired hemophilia A: a review of recent data and new therapeutic options.

PubMed

Franchini, Massimo; Vaglio, Stefania; Marano, Giuseppe; Mengoli, Carlo; Gentili, Sara; Pupella, Simonetta; Liumbruno, Giancarlo Maria

2017-10-01

Acquired hemophilia A (AHA) is a rare, but potentially life-threatening, bleeding disorder caused by an autoantibody against factor VIII that interferes with its coagulant function. We performed a narrative review focusing on the diagnostic aspects of AHA and on the current treatment strategies with particular regard to new data and therapeutic developments. The management of this severe hemorrhagic disorder is based on the control of bleeding with the use of bypassing agents and on the utilization of a variety of immunosuppressant agents with the goal of eliminating the autoantibody permanently. The optimal management of AHA should be multidisciplinary and requires a close collaboration between physicians from various specialties.

Characterizing Time Irreversibility in Disordered Fermionic Systems by the Effect of Local Perturbations

NASA Astrophysics Data System (ADS)

Vardhan, Shreya; De Tomasi, Giuseppe; Heyl, Markus; Heller, Eric J.; Pollmann, Frank

2017-07-01

We study the effects of local perturbations on the dynamics of disordered fermionic systems in order to characterize time irreversibility. We focus on three different systems: the noninteracting Anderson and Aubry-André-Harper (AAH) models and the interacting spinless disordered t -V chain. First, we consider the effect on the full many-body wave functions by measuring the Loschmidt echo (LE). We show that in the extended or ergodic phase the LE decays exponentially fast with time, while in the localized phase the decay is algebraic. We demonstrate that the exponent of the decay of the LE in the localized phase diverges proportionally to the single-particle localization length as we approach the metal-insulator transition in the AAH model. Second, we probe different phases of disordered systems by studying the time expectation value of local observables evolved with two Hamiltonians that differ by a spatially local perturbation. Remarkably, we find that many-body localized systems could lose memory of the initial state in the long-time limit, in contrast to the noninteracting localized phase where some memory is always preserved.

17 CFR 210.8-06 - Real estate operations acquired or to be acquired.

Code of Federal Regulations, 2011 CFR

2011-04-01

... 17 Commodity and Securities Exchanges 2 2011-04-01 2011-04-01 false Real estate operations acquired or to be acquired. 210.8-06 Section 210.8-06 Commodity and Securities Exchanges SECURITIES AND... Statements of Smaller Reporting Companies § 210.8-06 Real estate operations acquired or to be acquired. If...

Epidemiological and clinical characterization following a first psychotic episode in major depressive disorder: comparisons with schizophrenia and bipolar I disorder in the Cavan-Monaghan First Episode Psychosis Study (CAMFEPS).

PubMed

Owoeye, Olabisi; Kingston, Tara; Scully, Paul J; Baldwin, Patrizia; Browne, David; Kinsella, Anthony; Russell, Vincent; O'Callaghan, Eadbhard; Waddington, John L

2013-07-01

While recent research on psychotic illness has focussed on the nosological, clinical, and biological relationships between schizophrenia and bipolar disorder, little attention has been directed to the most common other psychotic diagnosis, major depressive disorder with psychotic features (MDDP). As this diagnostic category captures the confluence between dimensions of psychotic and affective psychopathology, it is of unappreciated heuristic potential to inform on the nature of psychotic illness. Therefore, the epidemiology and clinical characteristics of MDDP were compared with those of schizophrenia and bipolar disorder within the Cavan-Monaghan First Episode Psychosis Study (n = 370). Epidemiologically, the first psychotic episode of MDDP (n = 77) was uniformly distributed across the adult life span, while schizophrenia (n = 73) and bipolar disorder (n = 73) were primarily disorders of young adulthood; the incidence of MDDP, like bipolar disorder, did not differ between the sexes, while the incidence of schizophrenia was more common in males than in females. Clinically, MDDP was characterized by negative symptoms, executive dysfunction, neurological soft signs (NSS), premorbid intellectual function, premorbid adjustment, and quality of life similar to those for schizophrenia, while bipolar disorder was characterized by less prominent negative symptoms, executive dysfunction and NSS, and better quality of life. These findings suggest that what we currently categorize as MDDP may be more closely aligned with other psychotic diagnoses than has been considered previously. They indicate that differences in how psychosis is manifested vis-à-vis depression and mania may be quantitative rather than qualitative and occur within a dimensional space, rather than validating categorical distinctions.

Using Spoken Language Benchmarks to Characterize the Expressive Language Skills of Young Children With Autism Spectrum Disorders

PubMed Central

Weismer, Susan Ellis

2015-01-01

Purpose Spoken language benchmarks proposed by Tager-Flusberg et al. (2009) were used to characterize communication profiles of toddlers with autism spectrum disorders and to investigate if there were differences in variables hypothesized to influence language development at different benchmark levels. Method The communication abilities of a large sample of toddlers with autism spectrum disorders (N = 105) were characterized in terms of spoken language benchmarks. The toddlers were grouped according to these benchmarks to investigate whether there were differences in selected variables across benchmark groups at a mean age of 2.5 years. Results The majority of children in the sample presented with uneven communication profiles with relative strengths in phonology and significant weaknesses in pragmatics. When children were grouped according to one expressive language domain, across-group differences were observed in response to joint attention and gestures but not cognition or restricted and repetitive behaviors. Conclusion The spoken language benchmarks are useful for characterizing early communication profiles and investigating features that influence expressive language growth. PMID:26254475

Brain activation patterns in women with acquired hypoactive sexual desire disorder and women with normal sexual function: a cross-sectional pilot study.

PubMed

Woodard, Terri L; Nowak, Nicole T; Balon, Richard; Tancer, Manuel; Diamond, Michael P

2013-10-01

To examine and compare brain activation patterns of premenopausal women with normal sexual function and those with hypoactive sexual desire disorder (HSDD) during viewing of validated sexually explicit film clips. Cross-sectional pilot study. University-based clinical research center. Premenopausal women. None. Areas of brain activation during viewing of sexually explicit film clips. Women with normal sexual function showed significantly greater activation of the right thalamus, left insula, left precentral gyrus, and left parahippocampal gyrus in comparison with women with HSDD, who exhibited greater activation of the right medial frontal gyrus and left precuneus regions. Women with HSDD may have alterations in activation of limbic and cortical structures responsible for acquiring, encoding, and retrieving memory, the processing and memory of emotional reactions, and areas responsible for heightened attention to one's own physical state. Copyright © 2013 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.

Brain activation patterns in women with acquired hypoactive sexual desire disorder and women with normal sexual function: a cross-sectional pilot study

PubMed Central

Woodard, Terri L.; Nowak, Nicole T.; Balon, Richard; Tancer, Manuel; Diamond, Michael P.

2013-01-01

Objective To examine and compare brain activation patterns of premenopausal women with normal sexual function and those with hypoactive sexual desire disorder (HSDD) during viewing of validated sexually explicit film clips. Design Cross-sectional pilot study. Setting University-based clinical research center. Patient(s) Premenopausal women. Intervention(s) None. Main Outcome Measure(s) Areas of brain activation during viewing of sexually explicit film clips. Result(s) Women with normal sexual function showed significantly greater activation of the right thalamus, left insula, left precentral gyrus, and left parahippocampal gyrus in comparison with women with HSDD, who exhibited greater activation of the right medial frontal gyrus and left precuneus regions. Conclusion(s) Women with HSDD may have alterations in activation of limbic and cortical structures responsible for acquiring, encoding, and retrieving memory, the processing and memory of emotional reactions, and areas responsible for heightened attention to one’s own physical state. PMID:23830149

[Chronologic analysis of clonal evolution in acquired aplastic anemia and sMDS].

PubMed

Yoshizato, Tetsuichi

2016-04-01

Acquired aplastic anemia (AA) is a prototype of idiopathic bone marrow failure, which is caused by immune-mediated destruction of hematopoietic progenitors but is also characterized by frequent evolution to clonal myeloid disorders, such as myelodysplastic syndromes or acute myeloid leukemia. However, the chronological behavior of the clonality and its link to myelodysplastic syndrome or acute myeloid leukemia has not been fully explored. To define the clonality and its chronological behavior in AA, we performed targeted sequencing (N=439) in cases with AA. Somatic mutations were detected in 1/3 of our cases. Mutations were most frequently found in DNMT3A, followed by BCOR, PIGA and ASXL1. The prevalence of mutations increased with age. The clone sizes in DNMT3A and ASXL1 were prone to increase, whereas those of BCOR and PIGA were more likely to decrease or remain stable. Mutations in PIGA, BCOR and BCORL1 correlated with a better response to immunosuppressive therapy and more favorable survival. On the other hand, other mutations were associated with worse outcomes. The chronological dynamics of clonality showed marked variability and were not necessarily associated with prognosis.

Bulimia Nervosa/Purging Disorder.

PubMed

Castillo, Marigold; Weiselberg, Eric

2017-04-01

Bulimia nervosa was first described in 1979 by British psychiatrist Gerald Russell as a "chronic phase of anorexia nervosa" in which patients overeat and then use compensatory mechanisms, such as self-induced vomiting, laxatives, or prolonged periods of starvation. The characterization of bulimia nervosa continues to evolve with the introduction of the DSM-5 in 2013. In this article, the epidemiology and risk factors of bulimia nervosa are identified and reviewed, along with the medical complications and psychiatric comorbidities. The evaluation of a patient with suspected bulimia nervosa is addressed, with an emphasis on acquiring a complete and thorough history as well as discovering any comorbidities that are present. Management of the patient involves both medical interventions and behavioral counseling in order to address physical, psychological, and social needs. Lastly, a new diagnosis introduced in the DSM-5, purging disorder, is described and discussed. Copyright © 2017 Mosby, Inc. All rights reserved.

Anxiety Disorders among US Immigrants: The Role of Immigrant Background and Social-Psychological Factors

PubMed Central

Szaflarski, Magdalena; Cubbins, Lisa A.; Meganathan, Karthikeyan

2017-01-01

This study used the National Epidemiological Survey on Alcohol and Related Conditions, a longitudinal adult sample, to estimate the rates of prevalent, acquired, and persisting anxiety disorders by nativity and racial-ethnic origin while adjusting for acculturation, stress, social ties, and sociodemographics. Prevalent and acquired anxiety disorders were less likely among foreign-born than US-born, except Puerto-Rican- and Mexican-born who had higher risks. Persisting cases were similar between foreign-born and US-born, except Asian/Pacific Islanders’ lower risk. Stress and preference for socializing outside one’s racial-ethnic group were associated with higher while close ties were associated with lower rates of acquired/persisting anxiety disorders. PMID:28379742

Characterizing the continuously acquired cardiovascular time series during hemodialysis, using median hybrid filter preprocessing noise reduction.

PubMed

Wilson, Scott; Bowyer, Andrea; Harrap, Stephen B

2015-01-01

The clinical characterization of cardiovascular dynamics during hemodialysis (HD) has important pathophysiological implications in terms of diagnostic, cardiovascular risk assessment, and treatment efficacy perspectives. Currently the diagnosis of significant intradialytic systolic blood pressure (SBP) changes among HD patients is imprecise and opportunistic, reliant upon the presence of hypotensive symptoms in conjunction with coincident but isolated noninvasive brachial cuff blood pressure (NIBP) readings. Considering hemodynamic variables as a time series makes a continuous recording approach more desirable than intermittent measures; however, in the clinical environment, the data signal is susceptible to corruption due to both impulsive and Gaussian-type noise. Signal preprocessing is an attractive solution to this problem. Prospectively collected continuous noninvasive SBP data over the short-break intradialytic period in ten patients was preprocessed using a novel median hybrid filter (MHF) algorithm and compared with 50 time-coincident pairs of intradialytic NIBP measures from routine HD practice. The median hybrid preprocessing technique for continuously acquired cardiovascular data yielded a dynamic regression without significant noise and artifact, suitable for high-level profiling of time-dependent SBP behavior. Signal accuracy is highly comparable with standard NIBP measurement, with the added clinical benefit of dynamic real-time hemodynamic information.

Trichotillomania, stereotypic movement disorder, and related disorders.

PubMed

Stein, Dan J; Garner, Joseph P; Keuthen, Nancy J; Franklin, Martin E; Walkup, John T; Woods, Douglas W

2007-08-01

Trichotillomania is currently classified as an impulse control disorder not otherwise classified, whereas body-focused behaviors other than hair-pulling may be diagnosed as stereotypic movement disorder. A number of disorders characterized by repetitive, body-focused behaviors (eg, skin-picking) are prevalent and disabling and may have phenomenological and psychobiological overlap. Such disorders deserve greater recognition in the official nosology, and there would seem to be clinical utility in classifying them in the same diagnostic category.

Characterizing Community-Based Mental Health Services for Children with Autism Spectrum Disorders and Disruptive Behavior Problems

ERIC Educational Resources Information Center

Brookman-Frazee, Lauren I.; Taylor, Robin; Garland, Ann F.

2010-01-01

This study describes the characteristics of children with autism spectrum disorders (ASD) with disruptive behavior problems served in community-based mental health clinics, characterizes psychotherapy process and outcome, and examines differences between children with ASD and a non-ASD comparison group. Results indicate that children with ASD…

Role of TGF-β signaling in inherited and acquired myopathies

PubMed Central

2011-01-01

The transforming growth factor-beta (TGF-β) superfamily consists of a variety of cytokines expressed in many different cell types including skeletal muscle. Members of this superfamily that are of particular importance in skeletal muscle are TGF-β1, mitogen-activated protein kinases (MAPKs), and myostatin. These signaling molecules play important roles in skeletal muscle homeostasis and in a variety of inherited and acquired neuromuscular disorders. Expression of these molecules is linked to normal processes in skeletal muscle such as growth, differentiation, regeneration, and stress response. However, chronic elevation of TGF-β1, MAPKs, and myostatin is linked to various features of muscle pathology, including impaired regeneration and atrophy. In this review, we focus on the aberrant signaling of TGF-β in various disorders such as Marfan syndrome, muscular dystrophies, sarcopenia, and critical illness myopathy. We also discuss how the inhibition of several members of the TGF-β signaling pathway has been implicated in ameliorating disease phenotypes, opening up novel therapeutic avenues for a large group of neuromuscular disorders. PMID:21798096

Panic attacks and hoarding disorder: an initial investigation.

PubMed

Raines, Amanda M; Oglesby, Mary E; Short, Nicole A; Albanese, Brian J; Schmidt, Norman B

2014-08-01

Panic attacks (PAs) defined as a discrete period of intense fear or discomfort, occur in the context of numerous anxiety and mood related disorders. Research has suggested that PAs serve as a significant indicator and prognostic factor for overall symptom severity, course, and comorbidity within various conditions. Consequently, a PA specifier is now applicable to all DSM-5 disorders. Despite these clinical and nosological implications, no research to date has examined associations between PAs and hoarding disorder. The current investigation evaluated relationships between PA endorsement and hoarding severity within a sample of 32 patients with hoarding disorder. Findings suggested a high rate of panic history among those with hoarding disorder (56%). Hoarders with co-occurring PAs, compared to those without PAs, evidenced significantly higher symptom severity. Moreover, PAs continued to significantly predict hoarding severity even after controlling for relevant covariates. When examining the specific relationships among PAs and hoarding symptoms (i.e., acquiring, difficulty discarding, and clutter), the endorsement of PAs was associated with increased acquiring and difficulty discarding symptoms. These findings add considerably to a growing body of literature on hoarding disorder. Implications for the assessment and treatment of PAs that co-occur with hoarding disorder are discussed. Copyright © 2014 Elsevier Inc. All rights reserved.

Postmodern Stress Disorder (PMSD): A Possible New Disorder.

PubMed

Eiser, Arnold R

2015-11-01

The murder of cardiovascular surgeon, Michael Davidson, MD, suggests the existence of a new disorder, postmodern stress disorder. This disorder is characterized by repetitive exposure to digital images of violence in a variety of electronic media, including films, television, video games, music videos, and other online sources. This disorder appears to be a variant of posttraumatic stress disorder, and shares with it excessive stimulation of the amygdala and loss of the normal inhibitory inputs from the orbitofrontal cingulate cortical gyrus. In postmodern stress disorder, repetitive digital microtraumas appear to have an effect similar to that of macrotraumas of warfare or civilian assaults. Other elements of the disorder include the development of fixed ideas of bullying or public shaming, access to weapons, and loss of impulse control. This syndrome could explain a number of previously inexplicable murders/suicides. Violence against health care professionals is a profound concern for the medical profession, as are assaults on nonclinicians. The recommendation is made to change forensic procedures to include obtaining historic information concerning the use of digital media during investigations of violent crimes and murders so that the disorder may be further characterized. Gaining an understanding of this disorder will require a multidisciplinary approach to this life-threatening public health problem. Research should also focus on the development and evaluation of possible antidotes to postmodern toxicities. Copyright © 2015 Elsevier Inc. All rights reserved.

WAYS OF ACQUIRING FLYING PHOBIA.

PubMed

Schindler, Bettina; Vriends, Noortje; Margraf, Jürgen; Stieglitz, Rolf-Dieter

2016-02-01

The few studies that have explored how flying phobia is acquired have produced contradictory results. We hypothesized that classical conditioning plays a role in acquiring flying phobia and investigated if vicarious (model) learning, informational learning through media, and experiencing stressful life events at the time of onset of phobia also play a role. Thirty patients with flying phobia and thirty healthy controls matched on age, sex, and education were interviewed with the Mini-DIPS, the short German version of the Anxiety Disorders Interview Schedule (DSM-IV diagnostic criteria) and the Fear-of-Flying History Interview. Fifty Percent of patients with flying phobia and 53% of healthy controls reported frightening events in the air. There was no significant difference between the two samples. Thus there were not more classical conditioning events for patients with flying phobia. There also was no significant difference between the two samples for vicarious (model) learning: 37% of flying phobia patients and 23% of healthy controls felt influenced by model learning. The influence of informational learning through media was significantly higher for the clinical sample (70%) than for the control group (37%). Patients with flying phobia experienced significantly more stressful life events in the period of their frightening flight experience (60%) than healthy controls (19%). Frightening experiences while flying are quite common, but not everybody develops a flying phobia. Stressful life events and other factors might enhance conditionability. Informational learning through negative media reports probably reinforces the development of flying phobia. Clinical implications are discussed. © 2015 Wiley Periodicals, Inc.

Protein A sepharose immunoadsorption: immunological and haemostatic effects in two cases of acquired haemophilia.

PubMed

Guillet, B; Kriaa, F; Huysse, M G; Proulle, V; George, C; Tchernia, G; D'Oiron, R; Laurian, Y; Charpentier, B; Lambert, T; Dreyfus, M

2001-09-01

Acquired haemophilia is a life-threatening disorder caused by circulating auto-antibodies that inhibit factor VIII coagulant activity (FBIII:C). Immunoadsorption on protein A sepharose (IA-PA) was performed in two bleeding patients with acquired haemophilia: we observed a dramatic and quick decrease in the anti-FVIII:C inhibitor titre leading to a normal, albeit transient, haemostatic status. In one case, IA-PA was the only procedure which succeeded in stopping massive haemorrhage. In the second case, IA-PA reinforced the haemostatic effect of recombinant activated factor VII by increasing the endogenous plasma factor VIII level. The efficacy of IA-PA was sustained with immunosuppressive treatment introduced, respectively, 10 and 15 d before the IA-PA procedures. Our experience with IA-PA suggests that this extracorporeal anti-FVIII:C removal procedure is a valuable therapeutic tool for acquired haemophilia and can alleviate life-threatening haemorrhages.

Fear conditioning and stimulus generalization in patients with social anxiety disorder.

PubMed

Ahrens, Lea M; Pauli, Paul; Reif, Andreas; Mühlberger, Andreas; Langs, Gernot; Aalderink, Tim; Wieser, Matthias J

2016-12-01

Although overgeneralization seems to be a hallmark of several anxiety disorders, this until now has not been investigated in social anxiety disorder (SAD). Therefore, we examined fear generalization in 26 SAD patients and 29 healthy controls (HC) using two faces as conditioned stimuli (CS+, CS-), and a loud scream and a fearful face as unconditioned stimulus (US). Generalization was tested by presenting both CS and four morphs of the two faces (generalization stimuli [GSs]), while ratings, heart rate (HR) and skin conductance responses (SCR) were recorded. Results revealed that SAD patients rated all stimuli as less pleasant and more arousing compared to HC. Moreover, ratings and SCR indicated that both groups generalized their acquired fear from the CS+ to GSs. Remarkably, only SAD patients showed generalization in HR responses (fear bradycardia). Overall, SAD seems not to be characterized by strong overgeneralization but discrepancies in fear responses to both conditioned and generalized threat stimuli. Copyright © 2016 Elsevier Ltd. All rights reserved.

Characterization of Motor Control in Handwriting Difficulties in Children with or without Developmental Coordination Disorder

ERIC Educational Resources Information Center

Chang, Shao-Hsia; Yu, Nan-Ying

2010-01-01

Aim: The purpose of this study was to characterize handwriting deficits in children with developmental coordination disorder (DCD) using computerized movement analyses. Method: Seventy-two children (40 females, 32 males; mean age 7y, SD 7mo; range 6y 2mo to 7y 11mo) with handwriting deficits (33 with DCD, 39 without DCD); and 22 age- and…

Ensemble characterization of an intrinsically disordered FG-Nup peptide and its F>A mutant in DMSO-d6.

PubMed

Reid, Korey M; Sunanda, Punnepalli; Raghothama, S; Krishnan, V V

2017-11-01

Intrinsically disordered proteins (IDP) lack a well-defined 3D-structure under physiological conditions, yet, the inherent disorder represented by an ensemble of conformation plays a critical role in many cellular and regulatory processes. Nucleoporins, or Nups, are the proteins found in the nuclear pore complex (NPC). The central pore of the NPC is occupied by Nups, which have phenylalanine-glycine domain repeats and are intrinsically disordered, and therefore are termed FG-Nups. These FG-domain repeats exhibit differing cohesiveness character and differ from least (FG) to most (GLFG) cohesive. The designed FG-Nup is a 25 AA model peptide containing a noncohesive FG-motif flanked by two cohesive GLFG-motifs (WT peptide). Complete NMR-based ensemble characterization of this peptide along with a control peptide with an F>A substitution (MU peptide) are discussed. Ensemble characterization of the NMR-determined models suggests that both the peptides do not have consistent secondary structures and continue to be disordered. Nonetheless, the role of cohesive elements mediated by the GLFG motifs is evident in the WT ensemble of structures that are more compact than the MU peptide. The approach presented here allows an alternate way to investigate the specific roles of distinct amino acid motifs that translate into the long-range organization of the ensemble of structures and in general on the nature of IDPs. © 2017 Wiley Periodicals, Inc.

Salt-bridge networks within globular and disordered proteins: characterizing trends for designable interactions.

PubMed

Basu, Sankar; Mukharjee, Debasish

2017-07-01

There has been considerable debate about the contribution of salt bridges to the stabilization of protein folds, in spite of their participation in crucial protein functions. Salt bridges appear to contribute to the activity-stability trade-off within proteins by bringing high-entropy charged amino acids into close contacts during the course of their functions. The current study analyzes the modes of association of salt bridges (in terms of networks) within globular proteins and at protein-protein interfaces. While the most common and trivial type of salt bridge is the isolated salt bridge, bifurcated salt bridge appears to be a distinct salt-bridge motif having a special topology and geometry. Bifurcated salt bridges are found ubiquitously in proteins and interprotein complexes. Interesting and attractive examples presenting different modes of interaction are highlighted. Bifurcated salt bridges appear to function as molecular clips that are used to stitch together large surface contours at interacting protein interfaces. The present work also emphasizes the key role of salt-bridge-mediated interactions in the partial folding of proteins containing long stretches of disordered regions. Salt-bridge-mediated interactions seem to be pivotal to the promotion of "disorder-to-order" transitions in small disordered protein fragments and their stabilization upon binding. The results obtained in this work should help to guide efforts to elucidate the modus operandi of these partially disordered proteins, and to conceptualize how these proteins manage to maintain the required amount of disorder even in their bound forms. This work could also potentially facilitate explorations of geometrically specific designable salt bridges through the characterization of composite salt-bridge networks. Graphical abstract ᅟ.

A Pilot Study of the DBT Coach: An Interactive Mobile Phone Application for Individuals with Borderline Personality Disorder and Substance Use Disorder

ERIC Educational Resources Information Center

Rizvi, Shireen L.; Dimeff, Linda A.; Skutch, Julie; Carroll, David; Linehan, Marsha M.

2011-01-01

Dialectical behavior therapy (DBT) has received strong empirical support and is practiced widely as a treatment for borderline personality disorder (BPD) and BPD with comorbid substance use disorders (BPD-SUD). Therapeutic success in DBT requires that individuals generalize newly acquired skills to their natural environment. However, there have…

12 CFR 583.1 - Acquire.

Code of Federal Regulations, 2010 CFR

2010-01-01

... 12 Banks and Banking 5 2010-01-01 2010-01-01 false Acquire. 583.1 Section 583.1 Banks and Banking OFFICE OF THRIFT SUPERVISION, DEPARTMENT OF THE TREASURY DEFINITIONS FOR REGULATIONS AFFECTING SAVINGS AND LOAN HOLDING COMPANIES § 583.1 Acquire. The term acquire means to acquire, directly or indirectly...

Mechanical characterization of disordered and anisotropic cellular monolayers

NASA Astrophysics Data System (ADS)

Nestor-Bergmann, Alexander; Johns, Emma; Woolner, Sarah; Jensen, Oliver E.

2018-05-01

We consider a cellular monolayer, described using a vertex-based model, for which cells form a spatially disordered array of convex polygons that tile the plane. Equilibrium cell configurations are assumed to minimize a global energy defined in terms of cell areas and perimeters; energy is dissipated via dynamic area and length changes, as well as cell neighbor exchanges. The model captures our observations of an epithelium from a Xenopus embryo showing that uniaxial stretching induces spatial ordering, with cells under net tension (compression) tending to align with (against) the direction of stretch, but with the stress remaining heterogeneous at the single-cell level. We use the vertex model to derive the linearized relation between tissue-level stress, strain, and strain rate about a deformed base state, which can be used to characterize the tissue's anisotropic mechanical properties; expressions for viscoelastic tissue moduli are given as direct sums over cells. When the base state is isotropic, the model predicts that tissue properties can be tuned to a regime with high elastic shear resistance but low resistance to area changes, or vice versa.

A J-modulated protonless NMR experiment characterizes the conformational ensemble of the intrinsically disordered protein WIP.

PubMed

Rozentur-Shkop, Eva; Goobes, Gil; Chill, Jordan H

2016-12-01

Intrinsically disordered proteins (IDPs) are multi-conformational polypeptides that lack a single stable three-dimensional structure. It has become increasingly clear that the versatile IDPs play key roles in a multitude of biological processes, and, given their flexible nature, NMR is a leading method to investigate IDP behavior on the molecular level. Here we present an IDP-tailored J-modulated experiment designed to monitor changes in the conformational ensemble characteristic of IDPs by accurately measuring backbone one- and two-bond J( 15 N, 13 Cα) couplings. This concept was realized using a unidirectional (H)NCO 13 C-detected experiment suitable for poor spectral dispersion and optimized for maximum coverage of amino acid types. To demonstrate the utility of this approach we applied it to the disordered actin-binding N-terminal domain of WASp interacting protein (WIP), a ubiquitous key modulator of cytoskeletal changes in a range of biological systems. One- and two-bond J( 15 N, 13 Cα) couplings were acquired for WIP residues 2-65 at various temperatures, and in denaturing and crowding environments. Under native conditions fitted J-couplings identified in the WIP conformational ensemble a propensity for extended conformation at residues 16-23 and 45-60, and a helical tendency at residues 28-42. These findings are consistent with a previous study of the based upon chemical shift and RDC data and confirm that the WIP 2-65 conformational ensemble is biased towards the structure assumed by this fragment in its actin-bound form. The effects of environmental changes upon this ensemble were readily apparent in the J-coupling data, which reflected a significant decrease in structural propensity at higher temperatures, in the presence of 8 M urea, and under the influence of a bacterial cell lysate. The latter suggests that crowding can cause protein unfolding through protein-protein interactions that stabilize the unfolded state. We conclude that J-couplings are a

Characterization of Movement Disorder Phenomenology in Genetically Proven, Familial Frontotemporal Lobar Degeneration: A Systematic Review and Meta-Analysis.

PubMed

Gasca-Salas, Carmen; Masellis, Mario; Khoo, Edwin; Shah, Binit B; Fisman, David; Lang, Anthony E; Kleiner-Fisman, Galit

2016-01-01

Mutations in granulin (PGRN) and tau (MAPT), and hexanucleotide repeat expansions near the C9orf72 genes are the most prevalent genetic causes of frontotemporal lobar degeneration. Although behavior, language and movement presentations are common, the relationship between genetic subgroup and movement disorder phenomenology is unclear. We conducted a systematic review and meta-analysis of the literature characterizing the spectrum and prevalence of movement disorders in genetic frontotemporal lobar degeneration. Electronic databases were searched using terms related to frontotemporal lobar degeneration and movement disorders. Articles were included when cases had a proven genetic cause. Study-specific prevalence estimates for clinical features were transformed using Freeman-Tukey arcsine transformation, allowing for pooled estimates of prevalence to be generated using random-effects models. The mean age at onset was earlier in those with MAPT mutations compared to PGRN (p<0.001) and C9orf72 (p = 0.024). 66.5% of subjects had an initial non-movement presentation that was most likely a behavioral syndrome (35.7%). At any point during the disease, parkinsonism was the most common movement syndrome reported in 79.8% followed by progressive supranuclear palsy (PSPS) and corticobasal (CBS) syndromes in 12.2% and 10.7%, respectively. The prevalence of movement disorder as initial presentation was higher in MAPT subjects (35.8%) compared to PGRN subjects (10.1). In those with a non-movement presentation, language disorder was more common in PGRN subjects (18.7%) compared to MAPT subjects (5.4%). This represents the first systematic review and meta-analysis of the occurrence of movement disorder phenomenology in genetic frontotemporal lobar degeneration. Standardized prospective collection of clinical information in conjunction with genetic characterization will be crucial for accurate clinico-genetic correlation.

Characterization of Movement Disorder Phenomenology in Genetically Proven, Familial Frontotemporal Lobar Degeneration: A Systematic Review and Meta-Analysis

PubMed Central

Gasca-Salas, Carmen; Masellis, Mario; Khoo, Edwin; Shah, Binit B.; Fisman, David; Lang, Anthony E.; Kleiner-Fisman, Galit

2016-01-01

Background Mutations in granulin (PGRN) and tau (MAPT), and hexanucleotide repeat expansions near the C9orf72 genes are the most prevalent genetic causes of frontotemporal lobar degeneration. Although behavior, language and movement presentations are common, the relationship between genetic subgroup and movement disorder phenomenology is unclear. Objective We conducted a systematic review and meta-analysis of the literature characterizing the spectrum and prevalence of movement disorders in genetic frontotemporal lobar degeneration. Methods Electronic databases were searched using terms related to frontotemporal lobar degeneration and movement disorders. Articles were included when cases had a proven genetic cause. Study-specific prevalence estimates for clinical features were transformed using Freeman-Tukey arcsine transformation, allowing for pooled estimates of prevalence to be generated using random-effects models. Results The mean age at onset was earlier in those with MAPT mutations compared to PGRN (p<0.001) and C9orf72 (p = 0.024). 66.5% of subjects had an initial non-movement presentation that was most likely a behavioral syndrome (35.7%). At any point during the disease, parkinsonism was the most common movement syndrome reported in 79.8% followed by progressive supranuclear palsy (PSPS) and corticobasal (CBS) syndromes in 12.2% and 10.7%, respectively. The prevalence of movement disorder as initial presentation was higher in MAPT subjects (35.8%) compared to PGRN subjects (10.1). In those with a non-movement presentation, language disorder was more common in PGRN subjects (18.7%) compared to MAPT subjects (5.4%). Summary This represents the first systematic review and meta-analysis of the occurrence of movement disorder phenomenology in genetic frontotemporal lobar degeneration. Standardized prospective collection of clinical information in conjunction with genetic characterization will be crucial for accurate clinico-genetic correlation. PMID:27100392

Acquired neuropathies.

PubMed

Lozeron, Pierre; Trocello, Jean-Marc; Kubis, Nathalie

2013-09-01

Acquired neuropathies represent most of the neuropathies encountered in clinical practice. Hundreds of causes have been identified even though up to 41% of patients are still classified as idiopathic (Rajabally and Shah in J Neurol 258:1431-1436, 1). Routine evaluation relies on comprehensive medical history taking, clinical examination, nerve conduction studies and laboratory tests. Other investigations such as nerve biopsy or nerve or muscle imaging are performed in specific settings. This review focuses on recent advances in acquired neuropathies.

Investigations of the pathogenesis of acquired pendular nystagmus

NASA Technical Reports Server (NTRS)

Averbuch-Heller, L.; Zivotofsky, A. Z.; Das, V. E.; DiScenna, A. O.; Leigh, R. J.

1995-01-01

We investigated the pathogenesis of acquired pendular nystagmus (APN) in six patients, three of whom had multiple sclerosis. First, we tested the hypothesis that the oscillations of APN are due to a delay in visual feedback secondary, for example, to demyelination of the optic nerves. We manipulated the latency to onset of visually guided eye movements using an electronic technique that induces sinusoidal oscillations in normal subjects. This manipulation did not change the characteristics of the APN, but did superimpose lower-frequency oscillations similar to those induced in normal subjects. These results are consistent with current models for smooth (non-saccadic) eye movements, which predict that prolongation of visual feedback could not account for the high-frequency oscillations that often characterize APN. Secondly, we attempted to determine whether an increase in the gain of the visually-enhanced vestibulo-ocular reflex (VOR), produced by viewing a near target, was accompanied by a commensurate increase in the amplitude of APN. Increases in horizontal or vertical VOR gain during near viewing occurred in four patients, but only two of them showed a parallel increase in APN amplitude. On the other hand, APN amplitude decreased during viewing of the near target in the two patients who showed no change in VOR gain. Taken together, these data suggest that neither delayed visual feedback nor a disorder of central vestibular mechanisms is primarily responsible for APN. More likely, these ocular oscillations are produced by abnormalities of internal feedback circuits, such as the reciprocal connections between brainstem nuclei and cerebellum.

Acquisition of CS-US contingencies during Pavlovian fear conditioning and extinction in social anxiety disorder and posttraumatic stress disorder.

PubMed

Rabinak, Christine A; Mori, Shoko; Lyons, Maryssa; Milad, Mohammed R; Phan, K Luan

2017-01-01

Fear-based disorders, like social anxiety disorder (SAD) and posttraumatic stress disorder (PTSD), are characterized by an exaggerated fear response and avoidance to trigger cues, suggesting a transdiagnostic mechanism of psychopathology. Current theories suggest that abnormalities in conditioned fear is a primary contributor to the pathophysiology of these disorders. The primary goal of this study was to compare acquisition of conditioned stimulus (CS) and aversive unconditioned stimulus (US) contingencies during fear learning and extinction in individuals with SAD and PTSD. In a standard Pavlovian fear conditioning-extinction paradigm we measured subjective US expectancy ratings to different CSs in patients with SAD (n=16) compared to patients with PTSD (n=13) and healthy controls (n=15) RESULTS: Both patient groups (SAD, PTSD) acquired differential conditioning between a CS that predicted US (CS+) and a CS that never predicted the US (CS-), however, both groups reported an increased expectancy that the US would occur following the CS-. Additionally, the PTSD group overestimated that the US would occur in general. Neither patient group showed evidence of successful extinction of the CS+-US contingency nor differentiated their expectation of US occurrence between the CS+ and CS- during extinction learning. Group sample sizes were small and we did not include a trauma-exposed group without PTSD CONCLUSIONS: Both SAD and PTSD generalize expectations of an aversive outcome across CSs, even when a CS never signals an aversive outcome and PTSD may tend to over-expect threat. Fear learning and extinction abnormalities may be a core feature underlying shared symptoms across fear-based disorders. Copyright © 2016 Elsevier B.V. All rights reserved.

Naturally acquired antibodies against Clostridium perfringens epsilon toxin in goats.

PubMed

Veschi, Josir Laine A; Bruzzone, Octavio A; Losada-Eaton, Daniela M; Dutra, Iveraldo S; Fernandez-Miyakawa, Mariano E

2008-09-15

Clostridium perfringens type D-producing epsilon toxin is a common cause of death in sheep and goats worldwide. Although anti-epsilon toxin serum antibodies have been detected in healthy non-vaccinated sheep, the information regarding naturally acquired antibodies in ruminants is scanty. The objective of the present report was to characterize the development of naturally acquired antibodies against C. perfringens epsilon toxin in goats. The levels of anti-epsilon toxin antibodies in blood serum of goat kids from two different herds were examined continuously for 14 months. Goats were not vaccinated against any clostridial disease and received heterologous colostrums from cows that were not vaccinated against any clostridial disease. During the survey one of these flocks suffered an unexpectedly severe C. perfringens type D enterotoxemia outbreak. The results showed that natural acquired antibodies against C. perfringens epsilon toxin can appear as early as 6 weeks in young goats and increase with the age without evidence of clinical disease. The enterotoxemia outbreak was coincident with a significant increase in the level of anti-epsilon toxin antibodies.

Evidence to Practice Commentary: New Evidence in Developmental Coordination Disorder (DCD)

ERIC Educational Resources Information Center

Novak, Iona

2013-01-01

Developmental coordination disorder (DCD) is frequently under-recognized, but in fact, it occurs in as many as 5-6% of children. DCD is a disorder of motor coordination that is not explained by intellectual disability or any congenital or acquired neurological disorder. Families seek physical and occupational therapy (OT) to ameliorate a child…

Hypercalcemic Disorders in Children

PubMed Central

Stokes, Victoria J; Nielsen, Morten F; Hannan, Fadil M

2017-01-01

ABSTRACT Hypercalcemia is defined as a serum calcium concentration that is greater than two standard deviations above the normal mean, which in children may vary with age and sex, reflecting changes in the normal physiology at each developmental stage. Hypercalcemic disorders in children may present with hypotonia, poor feeding, vomiting, constipation, abdominal pain, lethargy, polyuria, dehydration, failure to thrive, and seizures. In severe cases renal failure, pancreatitis and reduced consciousness may also occur and older children and adolescents may present with psychiatric symptoms. The causes of hypercalcemia in children can be classified as parathyroid hormone (PTH)‐dependent or PTH‐independent, and may be congenital or acquired. PTH‐independent hypercalcemia, ie, hypercalcemia associated with a suppressed PTH, is commoner in children than PTH‐dependent hypercalcemia. Acquired causes of PTH‐independent hypercalcemia in children include hypervitaminosis; granulomatous disorders, and endocrinopathies. Congenital syndromes associated with PTH‐independent hypercalcemia include idiopathic infantile hypercalcemia (IIH), William's syndrome, and inborn errors of metabolism. PTH‐dependent hypercalcemia is usually caused by parathyroid tumors, which may give rise to primary hyperparathyroidism (PHPT) or tertiary hyperparathyroidism, which usually arises in association with chronic renal failure and in the treatment of hypophosphatemic rickets. Acquired causes of PTH‐dependent hypercalcemia in neonates include maternal hypocalcemia and extracorporeal membrane oxygenation. PHPT usually occurs as an isolated nonsyndromic and nonhereditary endocrinopathy, but may also occur as a hereditary hypercalcemic disorder such as familial hypocalciuric hypercalcemia, neonatal severe primary hyperparathyroidism, and familial isolated primary hyperparathyroidism, and less commonly, as part of inherited complex syndromic disorders such as multiple endocrine neoplasia

Hypercalcemic Disorders in Children.

PubMed

Stokes, Victoria J; Nielsen, Morten F; Hannan, Fadil M; Thakker, Rajesh V

2017-11-01

Hypercalcemia is defined as a serum calcium concentration that is greater than two standard deviations above the normal mean, which in children may vary with age and sex, reflecting changes in the normal physiology at each developmental stage. Hypercalcemic disorders in children may present with hypotonia, poor feeding, vomiting, constipation, abdominal pain, lethargy, polyuria, dehydration, failure to thrive, and seizures. In severe cases renal failure, pancreatitis and reduced consciousness may also occur and older children and adolescents may present with psychiatric symptoms. The causes of hypercalcemia in children can be classified as parathyroid hormone (PTH)-dependent or PTH-independent, and may be congenital or acquired. PTH-independent hypercalcemia, ie, hypercalcemia associated with a suppressed PTH, is commoner in children than PTH-dependent hypercalcemia. Acquired causes of PTH-independent hypercalcemia in children include hypervitaminosis; granulomatous disorders, and endocrinopathies. Congenital syndromes associated with PTH-independent hypercalcemia include idiopathic infantile hypercalcemia (IIH), William's syndrome, and inborn errors of metabolism. PTH-dependent hypercalcemia is usually caused by parathyroid tumors, which may give rise to primary hyperparathyroidism (PHPT) or tertiary hyperparathyroidism, which usually arises in association with chronic renal failure and in the treatment of hypophosphatemic rickets. Acquired causes of PTH-dependent hypercalcemia in neonates include maternal hypocalcemia and extracorporeal membrane oxygenation. PHPT usually occurs as an isolated nonsyndromic and nonhereditary endocrinopathy, but may also occur as a hereditary hypercalcemic disorder such as familial hypocalciuric hypercalcemia, neonatal severe primary hyperparathyroidism, and familial isolated primary hyperparathyroidism, and less commonly, as part of inherited complex syndromic disorders such as multiple endocrine neoplasia (MEN). Advances in

The relationship between neuroticism, major depressive disorder and comorbid disorders in Chinese women

PubMed Central

Xia, Jing; He, Qiang; Li, Yihan; Xie, Dong; Zhu, Suoyu; Chen, Jing; Shen, Yuan; Zhang, Ning; Wei, Yan; Chen, Chunfeng; Shen, Jianhua; Zhang, Yan; Gao, Chengge; Li, Youhui; Ding, Jihong; Shen, Wenwu; Wang, Qian; Cao, Meiyue; Liu, Tiebang; Zhang, Jinbei; Duan, Huijun; Bao, Cheng; Ma, Ping; Zhou, Cong; Luo, Yanfang; Zhang, Fengzhi; Liu, Ying; Li, Yi; Jin, Guixing; Zhang, Yutang; Liang, Wei; Chen, Yunchun; Zhao, Changyin; Li, Haiyan; Chen, Yiping; Shi, Shenxun; Kendler, Kenneth S.; Flint, Jonathan; Wang, Xumei

2011-01-01

Objective The personality trait of neuroticism is a risk factor for major depressive disorder (MDD), but this relationship has not been demonstrated in clinical samples from Asia. Methods We examined a large-scale clinical study of Chinese Han women with recurrent major depression and community-acquired controls. Results Elevated levels of neuroticism increased the risk for lifetime MDD (with an odds ratio of 1.37 per SD), contributed to the comorbidity of MDD with anxiety disorders, and predicted the onset and severity of MDD. Our findings largely replicate those obtained in clinical populations in Europe and US but differ in two ways: we did not find a relationship between melancholia and neuroticism; we found lower mean scores for neuroticism (3.6 in our community control sample). Limitations Our findings do not apply to MDD in community-acquired samples and may be limited to Han Chinese women. It is not possible to determine whether the association between neuroticism and MDD reflects a causal relationship. Conclusions Neuroticism acts as a risk factor for MDD in Chinese women, as it does in the West and may particularly predispose to comorbidity with anxiety disorders. Cultural factors may have an important effect on its measurement. PMID:21824661

Fabrication and characterization of disordered polymer optical fibers for transverse Anderson localization of light.

PubMed

Karbasi, Salman; Frazier, Ryan J; Mirr, Craig R; Koch, Karl W; Mafi, Arash

2013-07-29

We develop and characterize a disordered polymer optical fiber that uses transverse Anderson localization as a novel waveguiding mechanism. The developed polymer optical fiber is composed of 80,000 strands of poly (methyl methacrylate) (PMMA) and polystyrene (PS) that are randomly mixed and drawn into a square cross section optical fiber with a side width of 250 μm. Initially, each strand is 200 μm in diameter and 8-inches long. During the mixing process of the original fiber strands, the fibers cross over each other; however, a large draw ratio guarantees that the refractive index profile is invariant along the length of the fiber for several tens of centimeters. The large refractive index difference of 0.1 between the disordered sites results in a small localized beam radius that is comparable to the beam radius of conventional optical fibers. The input light is launched from a standard single mode optical fiber using the butt-coupling method and the near-field output beam from the disordered fiber is imaged using a 40X objective and a CCD camera. The output beam diameter agrees well with the expected results from the numerical simulations. The disordered optical fiber presented in this work is the first device-level implementation of 2D Anderson localization, and can potentially be used for image transport and short-haul optical communication systems.

Fabrication and Characterization of Disordered Polymer Optical Fibers for Transverse Anderson Localization of Light

PubMed Central

Karbasi, Salman; Frazier, Ryan J.; Mirr, Craig R.; Koch, Karl W.; Mafi, Arash

2013-01-01

We develop and characterize a disordered polymer optical fiber that uses transverse Anderson localization as a novel waveguiding mechanism. The developed polymer optical fiber is composed of 80,000 strands of poly (methyl methacrylate) (PMMA) and polystyrene (PS) that are randomly mixed and drawn into a square cross section optical fiber with a side width of 250 μm. Initially, each strand is 200 μm in diameter and 8-inches long. During the mixing process of the original fiber strands, the fibers cross over each other; however, a large draw ratio guarantees that the refractive index profile is invariant along the length of the fiber for several tens of centimeters. The large refractive index difference of 0.1 between the disordered sites results in a small localized beam radius that is comparable to the beam radius of conventional optical fibers. The input light is launched from a standard single mode optical fiber using the butt-coupling method and the near-field output beam from the disordered fiber is imaged using a 40X objective and a CCD camera. The output beam diameter agrees well with the expected results from the numerical simulations. The disordered optical fiber presented in this work is the first device-level implementation of 2D Anderson localization, and can potentially be used for image transport and short-haul optical communication systems. PMID:23929276

Peripheral neuropathy in genetically characterized patients with mitochondrial disorders: A study from south India.

PubMed

Bindu, Parayil Sankaran; Govindaraju, Chikanna; Sonam, Kothari; Nagappa, Madhu; Chiplunkar, Shwetha; Kumar, Rakesh; Gayathri, Narayanappa; Bharath, M M Srinivas; Arvinda, Hanumanthapura R; Sinha, Sanjib; Khan, Nahid Akthar; Govindaraj, Periyasamy; Nunia, Vandana; Paramasivam, Arumugam; Thangaraj, Kumarasamy; Taly, Arun B

2016-03-01

There are relatively few studies, which focus on peripheral neuropathy in large cohorts of genetically characterized patients with mitochondrial disorders. This study sought to analyze the pattern of peripheral neuropathy in a cohort of patients with mitochondrial disorders. The study subjects were derived from a cohort of 52 patients with a genetic diagnosis of mitochondrial disorders seen over a period of 8 years (2006-2013). All patients underwent nerve conduction studies and those patients with abnormalities suggestive of peripheral neuropathy were included in the study. Their phenotypic features, genotype, pattern of peripheral neuropathy and nerve conduction abnormalities were analyzed retrospectively. The study cohort included 18 patients (age range: 18 months-50 years, M:F- 1.2:1).The genotype included mitochondrial DNA point mutations (n=11), SURF1 mutations (n=4) and POLG1(n=3). Axonal neuropathy was noted in 12 patients (sensori-motor:n=4; sensory:n=4; motor:n=4) and demyelinating neuropathy in 6. Phenotype-genotype correlations revealed predominant axonal neuropathy in mtDNA point mutations and demyelinating neuropathy in SURF1. Patients with POLG related disorders had both sensory ataxic neuropathy and axonal neuropathy. A careful analysis of the family history, clinical presentation, biochemical, histochemical and structural analysis may help to bring out the mitochondrial etiology in patients with peripheral neuropathy and may facilitate targeted gene testing. Presence of demyelinating neuropathy in Leigh's syndrome may suggest underlying SURF1 mutations. Sensory ataxic neuropathy with other mitochondrial signatures should raise the possibility of POLG related disorder. Copyright © 2015. Published by Elsevier B.V.

Dysthymic Disorder

PubMed Central

Sansone, Lori A.

2009-01-01

This ongoing column is dedicated to the challenging clinical interface between psychiatry and primary care—two fields that are inexorably linked. Dysthymic disorder is a smoldering mood disturbance characterized by a long duration (at least two years in adults) as well as transient periods of normal mood. The disorder is fairly common in the US general population (3–6%) as well as in primary care (7%) and mental health settings (up to one-third of psychiatric outpatients). While the etiology of dysthymia remains unknown, there appears to be a genetic susceptibility, which may manifest in the presence of various psychosocial stressors. While the Diagnostic and Statistical Manual of Mental Disorders diagnostic criteria are fairly clear, the disorder can be easily under-recognized for a variety of reasons. Treatment may include pharmacotherapy and psychotherapy, although the overall treatment course is oftentimes characterized by protracted symptoms and relapses. PMID:19724735

Diagnosis and treatment of chronic acquired demyelinating polyneuropathies.

PubMed

Latov, Norman

2014-08-01

Chronic neuropathies are operationally classified as primarily demyelinating or axonal, on the basis of electrodiagnostic or pathological criteria. Demyelinating neuropathies are further classified as hereditary or acquired-this distinction is important, because the acquired neuropathies are immune-mediated and, thus, amenable to treatment. The acquired chronic demyelinating neuropathies include chronic inflammatory demyelinating polyneuropathy (CIDP), neuropathy associated with monoclonal IgM antibodies to myelin-associated glycoprotein (MAG; anti-MAG neuropathy), multifocal motor neuropathy (MMN), and POEMS syndrome. They have characteristic--though overlapping--clinical presentations, are mediated by distinct immune mechanisms, and respond to different therapies. CIDP is the default diagnosis if the neuropathy is demyelinating and no other cause is found. Anti-MAG neuropathy is diagnosed on the basis of the presence of anti-MAG antibodies, MMN is characterized by multifocal weakness and motor conduction blocks, and POEMS syndrome is associated with IgG or IgA λ-type monoclonal gammopathy and osteosclerotic myeloma. The correct diagnosis, however, can be difficult to make in patients with atypical or overlapping presentations, or nondefinitive laboratory studies. First-line treatments include intravenous immunoglobulin (IVIg), corticosteroids or plasmapheresis for CIDP; IVIg for MMN; rituximab for anti-MAG neuropathy; and irradiation or chemotherapy for POEMS syndrome. A correct diagnosis is required for choosing the appropriate treatment, with the aim of preventing progressive neuropathy.

A novel paraneoplastic syndrome with acquired lipodystrophy and chronic inflammatory demyelinating polyneuropathy in an adolescent male with craniopharyngioma.

PubMed

Lockemer, Hillary Elizabeth; Sumpter, Kathryn Maria; Cope-Yokoyama, Sandy; Garg, Abhimanyu

2018-03-28

Acquired lipodystrophy, craniopharyngioma and chronic inflammatory demyelinating polyneuropathy (CIDP) are individually rare disorders, and have never before been reported in a single patient. A 15-year-7 month old Caucasian male presented with lower extremity weakness, frequent falls and abnormal fat distribution occurring over the previous 1 year. He was diagnosed with CIDP, craniopharyngioma and acquired lipodystrophy. The patient underwent tumor debulking and cranial irradiation for the craniopharyngioma, and received monthly intravenous immunoglobulin for the CIDP. The patient initially had some resolution of the lipodystrophy phenotype, but subsequently the abnormal fat distribution recurred and the patient developed additional systemic abnormalities, including mild pancytopenia and hepatic fibrosis. Our patient represents a novel association of acquired lipodystrophy, craniopharyngioma, and CIDP, possibly due to an as yet unidentified paraneoplastic autoantibody.

An extra X does not prevent acquired hemophilia - Pregnancy-associated acquired hemophilia A.

PubMed

Barg, Assaf A; Livnat, Tami; Kenet, Gili

2017-03-01

Acquired hemophilia A (AHA) is a severe bleeding disorder caused by autoantibodies against clotting factor VIII (FVIII). With an estimated annual incidence of 1.3 to 1.5 per million, AHA is a rare disease. An extremely rare form of AHA has been described among women in the peripartum period, and may present with peripartum hemorrhage. Notably, although hemorrhagic symptoms commonly present 1-4 months around delivery, they may occur up to 1 year after parturition. When caring for a mother with AHA it is important to note that Factor VIII inhibitor may be transferred via the placenta from the mother to the fetus. Hence the newborn may also be affected. It is important to increase the awareness of Gynecologists for clinical symptoms and laboratory signs of AHA in order to avoid delayed diagnosis. Treatment may involve use of bypass agents to control hemorrhage, despite the risk of thrombosis, while immunomodulation (with increasing role for Rituximab) may be required to eradicate the inhibiting antibodies. Our review will evaluate the epidemiology, diagnosis, clinical course and treatment of peripartum AHA, focusing upon mother and infant care. © 2017 Elsevier Ltd. All rights reserved.

Pulmonary disease at autopsy in patients with the acquired immunodeficiency syndrome.

PubMed

Wallace, J M; Hannah, J B

1988-08-01

To characterize the postmortem pulmonary disease and analyze the effectiveness of antemortem diagnosis, we examined the clinical records and autopsy material from 54 patients who died of the acquired immunodeficiency syndrome. At autopsy, all patients had pulmonary disease. One or more specific diagnoses were made in 53, including opportunistic infection, nonopportunistic infection, and Kaposi's sarcoma. Multiple postmortem pulmonary diagnoses were established in 37. Respiratory failure was the most common cause of death. Of the 97 pulmonary disorders discovered at autopsy, only 31 were diagnosed before death. The frequency with which infections were diagnosed during life varied according to the organism, and was significantly higher for Pneumocystis carinii than for cytomegalovirus or bacterial agents. Pulmonary Kaposi's sarcoma was diagnosed in only 7% of patients with autopsy documentation. The yield of diagnostic procedures also varied according to the disease present. Sputum culture was relatively effective in detecting Cryptococcus neoformans and Mycobacterium avium-intracellulare, fiber-optic bronchoscopy was extremely useful for diagnosing P Carinii, and one or more diagnoses were provided in 4 of 7 patients who underwent thoracotomy, but significant disease including cytomegalovirus infection and pulmonary Kaposi's sarcoma was frequently missed. Although the spectrum of lung disease found at autopsy is similar to that observed during life, the frequency of some pathologic processes including cytomegalovirus infection and Kaposi's sarcoma may be underrepresented in antemortem series.

Pulmonary Disease at Autopsy in Patients With the Acquired Immunodeficiency Syndrome

PubMed Central

Wallace, Jeanne M.; Hannah, James B.

1988-01-01

To characterize the postmortem pulmonary disease and analyze the effectiveness of antemortem diagnosis, we examined the clinical records and autopsy material from 54 patients who died of the acquired immunodeficiency syndrome. At autopsy, all patients had pulmonary disease. One or more specific diagnoses were made in 53, including opportunistic infection, nonopportunistic infection, and Kaposi's sarcoma. Multiple postmortem pulmonary diagnoses were established in 37. Respiratory failure was the most common cause of death. Of the 97 pulmonary disorders discovered at autopsy, only 31 were diagnosed before death. The frequency with which infections were diagnosed during life varied according to the organism, and was significantly higher for Pneumocystis carinii than for cytomegalovirus or bacterial agents. Pulmonary Kaposi's sarcoma was diagnosed in only 7% of patients with autopsy documentation. The yield of diagnostic procedures also varied according to the disease present. Sputum culture was relatively effective in detecting Cryptococcus neoformans and Mycobacterium avium-intracellulare, fiber-optic bronchoscopy was extremely useful for diagnosing P Carinii, and one or more diagnoses were provided in 4 of 7 patients who underwent thoracotomy, but significant disease including cytomegalovirus infection and pulmonary Kaposi's sarcoma was frequently missed. Although the spectrum of lung disease found at autopsy is similar to that observed during life, the frequency of some pathologic processes including cytomegalovirus infection and Kaposi's sarcoma may be underrepresented in antemortem series. PMID:3266812

AIDS: acquired immunodeficiency syndrome.

PubMed Central

Gilmore, N. J.; Beaulieu, R.; Steben, M.; Laverdière, M.

1983-01-01

Acquired immunodeficiency syndrome, or AIDS, is a new illness that occurs in previously healthy individuals. It is characterized by immunodeficiency, opportunistic infections and unusual malignant diseases. Life-threatening single or multiple infections with viruses, mycobacteria, fungi or protozoa are common. A rare neoplasm, Kaposi's sarcoma, has developed in approximately one third of patients with AIDS. More than 800 cases of AIDS have been reported in North America, over 24 of them in Canada. The majority of patients are male homosexuals, although AIDS has also developed in abusers of intravenously administered drugs, Haitian immigrants, individuals with hemophilia, recipients of blood transfusions, prostitutes, and infants, spouses and partners of patients with AIDS. The cause of AIDS is unknown, but the features are consistent with an infectious process. Early diagnosis can be difficult owing to the nonspecific symptoms and signs of the infections and malignant diseases. Therefore, vigilance by physicians is of utmost importance. PMID:6342737

AIDS: acquired immunodeficiency syndrome *

PubMed Central

Gilmore, N.J.; Beaulieu, R.; Steben, M.; Laverdière, M.

1992-01-01

Acquired immunodeficiency syndrome, or AIDS, is a new illness that occurs in previously healthy individuals. It is characterized by immunodeficiency, opportunistic infections and unusual malignant diseases. Life-threatening single or multiple infections with viruses, mycobacteria, fungi or protozoa are common. A rare neoplasm, Kaposi's sarcoma, has developed in approximately one third of patients with AIDS. More than 800 cases of AIDS have been reported in North America, over 24 of them in Canada. The majority of patients are male homosexuals, although AIDS has also developed in abusers of intravenously administered drugs, Haitian immigrants, individuals with hemophilia, recipients of blood transfusions, prostitutes, and infants, spouses and partners of patients with AIDS. The cause of AIDS is unknown, but the features are consistent with an infectious process. Early diagnosis can be difficult owing to the nonspecific symptoms and signs of the infections and malignant diseases. Therefore, vigilance by physicians is of the utmost importance. PMID:1544049

Functional speech disorders: clinical manifestations, diagnosis, and management.

PubMed

Duffy, J R

2016-01-01

Acquired psychogenic or functional speech disorders are a subtype of functional neurologic disorders. They can mimic organic speech disorders and, although any aspect of speech production can be affected, they manifest most often as dysphonia, stuttering, or prosodic abnormalities. This chapter reviews the prevalence of functional speech disorders, the spectrum of their primary clinical characteristics, and the clues that help distinguish them from organic neurologic diseases affecting the sensorimotor networks involved in speech production. Diagnosis of a speech disorder as functional can be supported by sometimes rapidly achieved positive outcomes of symptomatic speech therapy. The general principles of such therapy are reviewed. © 2016 Elsevier B.V. All rights reserved.

Acquired color vision deficiency.

PubMed

Simunovic, Matthew P

2016-01-01

Acquired color vision deficiency occurs as the result of ocular, neurologic, or systemic disease. A wide array of conditions may affect color vision, ranging from diseases of the ocular media through to pathology of the visual cortex. Traditionally, acquired color vision deficiency is considered a separate entity from congenital color vision deficiency, although emerging clinical and molecular genetic data would suggest a degree of overlap. We review the pathophysiology of acquired color vision deficiency, the data on its prevalence, theories for the preponderance of acquired S-mechanism (or tritan) deficiency, and discuss tests of color vision. We also briefly review the types of color vision deficiencies encountered in ocular disease, with an emphasis placed on larger or more detailed clinical investigations. Copyright © 2016 Elsevier Inc. All rights reserved.

Molecular Characterization of Acquired Tolerance of Tumor Cells to Picropodophyllin (PPP)

PubMed Central

Hashemi, Jamileh; Worrall, Claire; Vasilcanu, Daiana; Fryknäs, Mårten; Sulaiman, Luqman; Karimi, Mohsen; Weng, Wen-Hui; Lui, Weng-Onn; Rudduck, Christina; Axelson, Magnus; Jernberg-Wiklund, Helena; Girnita, Leonard; Larsson, Olle; Larsson, Catharina

2011-01-01

Background Picropodophyllin (PPP) is a promising novel anti-neoplastic agent that efficiently kills tumor cells in vitro and causes tumor regression and increased survival in vivo. We have previously reported that PPP treatment induced moderate tolerance in two out of 10 cell lines only, and here report the acquired genomic and expression alterations associated with PPP selection over 1.5 years of treatment. Methodology/Principal Findings Copy number alterations monitored using metaphase and array-based comparative genomic hybridization analyses revealed largely overlapping alterations in parental and maximally tolerant cells. Gain/ amplification of the MYC and PVT1 loci in 8q24.21 were verified on the chromosome level. Abnormalities observed in connection to PPP treatment included regular gains and losses, as well as homozygous losses in 10q24.1-q24.2 and 12p12.3-p13.2 in one of the lines and amplification at 5q11.2 in the other. Abnormalities observed in both tolerant derivatives include amplification/gain of 5q11.2, gain of 11q12.1-q14.3 and gain of 13q33.3-qter. Using Nexus software analysis we combined the array-CGH data with data from gene expression profilings and identified genes that were altered in both inputs. A subset of genes identified as downregulated (ALDH1A3, ANXA1, TLR4 and RAB5A) or upregulated (COX6A1, NFIX, ME1, MAPK and TAP2) were validated by siRNA in the tolerant or parental cells to alter sensitivity to PPP and confirmed to alter sensitivity to PPP in further cell lines. Conclusions Long-term PPP selection lead to altered gene expression in PPP tolerant cells with increase as well as decrease of genes involved in cell death such as PTEN and BCL2. In addition, acquired genomic copy number alterations were observed that were often reflected by altered mRNA expression levels for genes in the same regions. PMID:21423728

Molecular characterization of acquired tolerance of tumor cells to picropodophyllin (PPP).

PubMed

Hashemi, Jamileh; Worrall, Claire; Vasilcanu, Daiana; Fryknäs, Mårten; Sulaiman, Luqman; Karimi, Mohsen; Weng, Wen-Hui; Lui, Weng-Onn; Rudduck, Christina; Axelson, Magnus; Jernberg-Wiklund, Helena; Girnita, Leonard; Larsson, Olle; Larsson, Catharina

2011-03-14

Picropodophyllin (PPP) is a promising novel anti-neoplastic agent that efficiently kills tumor cells in vitro and causes tumor regression and increased survival in vivo. We have previously reported that PPP treatment induced moderate tolerance in two out of 10 cell lines only, and here report the acquired genomic and expression alterations associated with PPP selection over 1.5 years of treatment. Copy number alterations monitored using metaphase and array-based comparative genomic hybridization analyses revealed largely overlapping alterations in parental and maximally tolerant cells. Gain/amplification of the MYC and PVT1 loci in 8q24.21 were verified on the chromosome level. Abnormalities observed in connection to PPP treatment included regular gains and losses, as well as homozygous losses in 10q24.1-q24.2 and 12p12.3-p13.2 in one of the lines and amplification at 5q11.2 in the other. Abnormalities observed in both tolerant derivatives include amplification/gain of 5q11.2, gain of 11q12.1-q14.3 and gain of 13q33.3-qter. Using Nexus software analysis we combined the array-CGH data with data from gene expression profilings and identified genes that were altered in both inputs. A subset of genes identified as downregulated (ALDH1A3, ANXA1, TLR4 and RAB5A) or upregulated (COX6A1, NFIX, ME1, MAPK and TAP2) were validated by siRNA in the tolerant or parental cells to alter sensitivity to PPP and confirmed to alter sensitivity to PPP in further cell lines. Long-term PPP selection lead to altered gene expression in PPP tolerant cells with increase as well as decrease of genes involved in cell death such as PTEN and BCL2. In addition, acquired genomic copy number alterations were observed that were often reflected by altered mRNA expression levels for genes in the same regions.

The Role of Intrinsic Brain Functional Connectivity in Vulnerability and Resilience to Bipolar Disorder.

PubMed

Doucet, Gaelle E; Bassett, Danielle S; Yao, Nailin; Glahn, David C; Frangou, Sophia

2017-12-01

Bipolar disorder is a heritable disorder characterized by mood dysregulation associated with brain functional dysconnectivity. Previous research has focused on the detection of risk- and disease-associated dysconnectivity in individuals with bipolar disorder and their first-degree relatives. The present study seeks to identify adaptive brain connectivity features associated with resilience, defined here as avoidance of illness or delayed illness onset in unaffected siblings of patients with bipolar disorder. Graph theoretical methods were used to examine global and regional brain network topology in head-motion-corrected resting-state functional MRI data acquired from 78 patients with bipolar disorder, 64 unaffected siblings, and 41 healthy volunteers. Global network properties were preserved in patients and their siblings while both groups showed reductions in the cohesiveness of the sensorimotor network. In the patient group, these sensorimotor network abnormalities were coupled with reduced integration of core default mode network regions in the ventromedial cortex and hippocampus. Conversely, integration of the default mode network was increased in the sibling group compared with both the patient group and the healthy volunteer group. The authors found that trait-related vulnerability to bipolar disorder was associated with reduced resting-state cohesiveness of the sensorimotor network in patients with bipolar disorder. However, integration of the default mode network emerged as a key feature differentiating disease expression and resilience between the patients and their siblings. This is indicative of the presence of neural mechanisms that may promote resilience, or at least delay illness onset.

AIDS: Acquired Immune Deficiency Syndrome, Information and Procedural Guidelines for Providing Services to Persons with AIDS/HTLV-III.

ERIC Educational Resources Information Center

Montana State Dept. of Health and Environmental Sciences, Helena.

This manual presents information about the disease, Acquired Immune Deficiency Syndrome (AIDS), and guidelines for service delivery to Montana residents who have been diagnosed with AIDS or related disorders. The first section describes the disease's causes, symptoms, and transmission; risk factors; high-risk populations; prevention suggestions;…

Origins and consequences of technology acquirement by independent-living seniors: towards an integrative model.

PubMed

Peek, S T M; Luijkx, K G; Vrijhoef, H J M; Nieboer, M E; Aarts, S; van der Voort, C S; Rijnaard, M D; Wouters, E J M

2017-08-22

Living independently can be challenging for seniors. Technologies are expected to help older adults age in place, yet little empirical research is available on how seniors develop a need for technologies, how they acquire these technologies, and how these subsequently affect their lives. Aging is complex, dynamic and personal. But how does this translate to seniors' adoption and acceptance of technology? To better understand origins and consequences of technology acquirement by independent-living seniors, an explorative longitudinal qualitative field study was set up. Home visits were made to 33 community-dwelling seniors living in the Netherlands, on three occasions (2012-2014). Semi-structured interviews were conducted on the timeline of acquirements, and people and factors involved in acquirements. Additionally, participants were interviewed on experiences in using technologies since acquirement. Thematic analysis was employed to analyze interview transcripts, using a realist approach to better understand the contexts, mechanisms and outcomes of technology acquirements. Findings were accumulated in a new conceptual model: The Cycle of Technology Acquirement by Independent-Living Seniors (C-TAILS), which provides an integrative perspective on why and how technologies are acquired, and why these may or may not prove to be appropriate and effective, considering an independent-living senior's needs and circumstances at a given point in time. We found that externally driven and purely desire-driven acquirements led to a higher risk of suboptimal use and low levels of need satisfaction. Technology acquirement by independent-living seniors may be best characterized as a heterogeneous process with many different origins, pathways and consequences. Furthermore, technologies that are acquired in ways that are not congruent with seniors' personal needs and circumstances run a higher risk of proving to be ineffective or inappropriate. Yet, these needs and circumstances are

Developmental disorders: what can be learned from cognitive neuropsychology?

PubMed

Castles, Anne; Kohnen, Saskia; Nickels, Lyndsey; Brock, Jon

2014-01-01

The discipline of cognitive neuropsychology has been important for informing theories of cognition and describing the nature of acquired cognitive disorders, but its applicability in a developmental context has been questioned. Here, we revisit this issue, asking whether the cognitive neuropsychological approach can be helpful for exploring the nature and causes of developmental disorders and, if so, how. We outline the key features of the cognitive neuropsychological approach, and then consider how some of the major challenges to this approach from a developmental perspective might be met. In doing so, we distinguish between challenges to the methods of cognitive neuropsychology and those facing its deeper conceptual underpinnings. We conclude that the detailed investigation of patterns of both associations and dissociations, and across both developmental and acquired cases, can assist in describing the cognitive deficits within developmental disorders and in delineating possible causal pathways to their acquisition.

Developmental disorders: what can be learned from cognitive neuropsychology?

PubMed Central

Castles, Anne; Kohnen, Saskia; Nickels, Lyndsey; Brock, Jon

2014-01-01

The discipline of cognitive neuropsychology has been important for informing theories of cognition and describing the nature of acquired cognitive disorders, but its applicability in a developmental context has been questioned. Here, we revisit this issue, asking whether the cognitive neuropsychological approach can be helpful for exploring the nature and causes of developmental disorders and, if so, how. We outline the key features of the cognitive neuropsychological approach, and then consider how some of the major challenges to this approach from a developmental perspective might be met. In doing so, we distinguish between challenges to the methods of cognitive neuropsychology and those facing its deeper conceptual underpinnings. We conclude that the detailed investigation of patterns of both associations and dissociations, and across both developmental and acquired cases, can assist in describing the cognitive deficits within developmental disorders and in delineating possible causal pathways to their acquisition. PMID:24324246

Cognitive Rehabilitation of Episodic Memory Disorders: From Theory to Practice

PubMed Central

Ptak, Radek; der Linden, Martial Van; Schnider, Armin

2010-01-01

Memory disorders are among the most frequent and most debilitating cognitive impairments following acquired brain damage. Cognitive remediation strategies attempt to restore lost memory capacity, provide compensatory techniques or teach the use of external memory aids. Memory rehabilitation has strongly been influenced by memory theory, and the interaction between both has stimulated the development of techniques such as spaced retrieval, vanishing cues or errorless learning. These techniques partly rely on implicit memory and therefore enable even patients with dense amnesia to acquire new information. However, knowledge acquired in this way is often strongly domain-specific and inflexible. In addition, individual patients with amnesia respond differently to distinct interventions. The factors underlying these differences have not yet been identified. Behavioral management of memory failures therefore often relies on a careful description of environmental factors and measurement of associated behavioral disorders such as unawareness of memory failures. The current evidence suggests that patients with less severe disorders benefit from self-management techniques and mnemonics whereas rehabilitation of severely amnesic patients should focus on behavior management, the transmission of domain-specific knowledge through implicit memory processes and the compensation for memory deficits with memory aids. PMID:20700383

Development of the Aboriginal Communication Assessment After Brain Injury (ACAABI): A screening tool for identifying acquired communication disorders in Aboriginal Australians.

PubMed

Armstrong, Elizabeth M; Ciccone, Natalie; Hersh, Deborah; Katzenellebogen, Judith; Coffin, Juli; Thompson, Sandra; Flicker, Leon; Hayward, Colleen; Woods, Deborah; McAllister, Meaghan

2017-06-01

Acquired communication disorders (ACD), following stroke and traumatic brain injury, may not be correctly identified in Aboriginal Australians due to a lack of linguistically and culturally appropriate assessment tools. Within this paper we explore key issues that were considered in the development of the Aboriginal Communication Assessment After Brain Injury (ACAABI) - a screening tool designed to assess the presence of ACD in Aboriginal populations. A literature review and consultation with key stakeholders were undertaken to explore directions needed to develop a new tool, based on existing tools and recommendations for future developments. The literature searches revealed no existing screening tool for ACD in these populations, but identified tools in the areas of cognition and social-emotional wellbeing. Articles retrieved described details of the content and style of these tools, with recommendations for the development and administration of a new tool. The findings from the interview and focus group views were consistent with the approach recommended in the literature. There is a need for a screening tool for ACD to be developed but any tool must be informed by knowledge of Aboriginal language, culture and community input in order to be acceptable and valid.

[Eating disorders].

PubMed

Miyake, Yoshie; Okamoto, Yuri; Jinnin, Ran; Shishida, Kazuhiro; Okamoto, Yasumasa

2015-02-01

Eating disorders are characterized by aberrant patterns of eating behavior, including such symptoms as extreme restriction of food intake or binge eating, and severe disturbances in the perception of body shape and weight, as well as a drive for thinness and obsessive fears of becoming fat. Eating disorder is an important cause for physical and psychosocial morbidity in young women. Patients with eating disorders have a deficit in the cognitive process and functional abnormalities in the brain system. Recently, brain-imaging techniques have been used to identify specific brain areas that function abnormally in patients with eating disorders. We have discussed the clinical and cognitive aspects of eating disorders and summarized neuroimaging studies of eating disorders.

Characterizing outcome preferences in patients with psychotic disorders: a discrete choice conjoint experiment.

PubMed

Zipursky, Robert B; Cunningham, Charles E; Stewart, Bailey; Rimas, Heather; Cole, Emily; Vaz, Stephanie McDermid

2017-07-01

The majority of individuals with schizophrenia will achieve a remission of psychotic symptoms, but few will meet criteria for recovery. Little is known about what outcomes are important to patients. We carried out a discrete choice experiment to characterize the outcome preferences of patients with psychotic disorders. Participants (N=300) were recruited from two clinics specializing in psychotic disorders. Twelve outcomes were each defined at three levels and incorporated into a computerized survey with 15 choice tasks. Utility values and importance scores were calculated for each outcome level. Latent class analysis was carried out to determine whether participants were distributed into segments with different preferences. Multinomial logistic regression was used to identify predictors of segment membership. Latent class analysis revealed three segments of respondents. The first segment (48%), which we labeled "Achievement-focused," preferred to have a full-time job, to live independently, to be in a long-term relationship, and to have no psychotic symptoms. The second segment (29%), labeled "Stability-focused," preferred to not have a job, to live independently, and to have some ongoing psychotic symptoms. The third segment (23%), labeled "Health-focused," preferred to not have a job, to live in supervised housing, and to have no psychotic symptoms. Segment membership was predicted by education, socioeconomic status, psychotic symptom severity, and work status. This study has revealed that patients with psychotic disorders are distributed between segments with different outcome preferences. New approaches to improve outcomes for patients with psychotic disorders should be informed by a greater understanding of patient preferences and priorities. Copyright © 2016 Elsevier B.V. All rights reserved.

Branched-chain amino acid metabolism: from rare Mendelian diseases to more common disorders

PubMed Central

Burrage, Lindsay C.; Nagamani, Sandesh C.S.; Campeau, Philippe M.; Lee, Brendan H.

2014-01-01

Branched-chain amino acid (BCAA) metabolism plays a central role in the pathophysiology of both rare inborn errors of metabolism and the more common multifactorial diseases. Although deficiency of the branched-chain ketoacid dehydrogenase (BCKDC) and associated elevations in the BCAAs and their ketoacids have been recognized as the cause of maple syrup urine disease (MSUD) for decades, treatment options for this disorder have been limited to dietary interventions. In recent years, the discovery of improved leucine tolerance after liver transplantation has resulted in a new therapeutic strategy for this disorder. Likewise, targeting the regulation of the BCKDC activity may be an alternative potential treatment strategy for MSUD. The regulation of the BCKDC by the branched-chain ketoacid dehydrogenase kinase has also been implicated in a new inborn error of metabolism characterized by autism, intellectual disability and seizures. Finally, there is a growing body of literature implicating BCAA metabolism in more common disorders such as the metabolic syndrome, cancer and hepatic disease. This review surveys the knowledge acquired on the topic over the past 50 years and focuses on recent developments in the field of BCAA metabolism. PMID:24651065

Classification of Systemic and Localized Sweating Disorders.

PubMed

Ohshima, Yuichiro; Tamada, Yasuhiko

2016-01-01

Hyperhidrosis can be subdivided into generalized hyperhidrosis, with increased sweating over the entire body, and focal hyperhidrosis, in which the excessive sweating is restricted to specific parts of the body. Generalized hyperhidrosis may be either primary (idiopathic) or secondary. Secondary generalized hyperhidrosis may be caused by infections such as tuberculosis, hyperthyroidism, endocrine and metabolic disturbances such as pheochromocytoma, neurological disorders, or drugs. Focal hyperhidrosis may also be primary (idiopathic) or secondary. Frey's syndrome is one form of secondary focal hyperhidrosis that occurs during eating together with reddening of the area in front of the ear following parotid gland surgery or injury. Primary focal hyperhidrosis is particularly common on the palms and soles of the feet, in the axilla, and on the head. Anhidrosis may be either congenital/genetic or acquired. Some of the most typical forms of congenital/genetic anhidrosis include hypohidrotic ectodermal dysplasia, congenital insensitivity to pain and anhidrosis, and Fabry disease. Acquired anhidrosis is classified as secondary anhidrosis, which may be due to an underlying disorder such as a neurological disorder, an endocrine or metabolic disturbance, or the effect of drugs, or idiopathic anhidrosis for which the pathology, cause, and mechanism are unknown. Idiopathic anhidrosis is classified into acquired idiopathic generalized anhidrosis (AIGA), idiopathic segmental anhidrosis, and Ross syndrome. AIGA is divided into three categories according to differences in the site of disturbance: (1) sudomotor neuropathy, (2) idiopathic pure sudomotor failure, and (3) sweat gland failure. © 2016 S. Karger AG, Basel.

Characterization of in vivo-acquired resistance to macrolides of Mycoplasma gallisepticum strains isolated from poultry

PubMed Central

2011-01-01

The macrolide class of antibiotics, including tylosin and tilmicosin, is widely used in the veterinary field for prophylaxis and treatment of mycoplasmosis. In vitro susceptibility testing of 50 strains of M. gallisepticum isolated in Israel during the period 1997-2010 revealed that acquired resistance to tylosin as well as to tilmicosin was present in 50% of them. Moreover, 72% (13/18) of the strains isolated from clinical samples since 2006 showed acquired resistance to enrofloxacin, tylosin and tilmicosin. Molecular typing of the field isolates, performed by gene-target sequencing (GTS), detected 13 molecular types (I-XIII). Type II was the predominant type prior to 2006 whereas type X, first detected in 2008, is currently prevalent. All ten type X strains were resistant to both fluoroquinolones and macrolides, suggesting selective pressure leading to clonal dissemination of resistance. However, this was not a unique event since resistant strains with other GTS molecular types were also found. Concurrently, the molecular basis for macrolide resistance in M. gallisepticum was identified. Our results revealed a clear-cut correlation between single point mutations A2058G or A2059G in domain V of the gene encoding 23S rRNA (rrnA, MGA_01) and acquired macrolide resistance in M. gallisepticum. Indeed, all isolates with MIC ≥ 0.63 μg/mL to tylosin and with MIC ≥ 1.25 μg/mL to tilmicosin possess one of these mutations, suggesting an essential role in decreased susceptibility of M. gallisepticum to 16-membered macrolides. PMID:21810258

Patient Characterization Protocols for Psychophysiological Studies of Traumatic Brain Injury and Post-TBI Psychiatric Disorders

PubMed Central

Rapp, Paul E.; Rosenberg, Brenna M.; Keyser, David O.; Nathan, Dominic; Toruno, Kevin M.; Cellucci, Christopher J.; Albano, Alfonso M.; Wylie, Scott A.; Gibson, Douglas; Gilpin, Adele M. K.; Bashore, Theodore R.

2013-01-01

Psychophysiological investigations of traumatic brain injury (TBI) are being conducted for several reasons, including the objective of learning more about the underlying physiological mechanisms of the pathological processes that can be initiated by a head injury. Additional goals include the development of objective physiologically based measures that can be used to monitor the response to treatment and to identify minimally symptomatic individuals who are at risk of delayed-onset neuropsychiatric disorders following injury. Research programs studying TBI search for relationships between psychophysiological measures, particularly ERP (event-related potential) component properties (e.g., timing, amplitude, scalp distribution), and a participant’s clinical condition. Moreover, the complex relationships between brain injury and psychiatric disorders are receiving increased research attention, and ERP technologies are making contributions to this effort. This review has two objectives supporting such research efforts. The first is to review evidence indicating that TBI is a significant risk factor for post-injury neuropsychiatric disorders. The second objective is to introduce ERP researchers who are not familiar with neuropsychiatric assessment to the instruments that are available for characterizing TBI, post-concussion syndrome, and psychiatric disorders. Specific recommendations within this very large literature are made. We have proceeded on the assumption that, as is typically the case in an ERP laboratory, the investigators are not clinically qualified and that they will not have access to participant medical records. PMID:23885250

Characterization of Esophageal Motility Disorders in Children Presenting With Dysphagia Using High-Resolution Manometry.

PubMed

Edeani, Francis; Malik, Adeel; Kaul, Ajay

2017-03-01

The Chicago classification was based on metrics derived from studies in asymptomatic adult subjects. Our objectives were to characterize esophageal motility disorders in children and to determine whether the spectrum of manometric findings is similar between the pediatric and adult populations. Studies have suggested that the metrics utilized in manometric diagnosis depend on age, size, and manometric assembly. This would imply that a different set of metrics should be used for the pediatric population. There are no standardized and generally accepted metrics for use in the pediatric population, though there have been attempts to establish metrics specific to this population. Overall, we found that the distribution of esophageal motility disorders in children was like that described in adults using the Chicago classification. This analysis will serve as a prequel to follow-up studies exploring the individual metrics for variability among patients, with the objective of establishing novel metrics for the pediatric population.

Automaticity in Anxiety Disorders and Major Depressive Disorder

PubMed Central

Teachman, Bethany A.; Joormann, Jutta; Steinman, Shari; Gotlib, Ian H.

2012-01-01

In this paper we examine the nature of automatic cognitive processing in anxiety disorders and Major Depressive Disorder (MDD). Rather than viewing automaticity as a unitary construct, we follow a social cognition perspective (Bargh, 1994) that argues for four theoretically independent features of automaticity: unconscious (processing of emotional stimuli occurs outside awareness), efficient (processing emotional meaning uses minimal attentional resources), unintentional (no goal is needed to engage in processing emotional meaning), and uncontrollable (limited ability to avoid, alter or terminate processing emotional stimuli). Our review of the literature suggests that most anxiety disorders are characterized by uncontrollable, and likely also unconscious and unintentional, biased processing of threat-relevant information. In contrast, MDD is most clearly typified by uncontrollable, but not unconscious or unintentional, processing of negative information. For the anxiety disorders and for MDD, there is not sufficient evidence to draw firm conclusions about efficiency of processing, though early indications are that neither anxiety disorders nor MDD are characterized by this feature. Clinical and theoretical implications of these findings are discussed and directions for future research are offered. In particular, it is clear that paradigms that more directly delineate the different features of automaticity are required to gain a more comprehensive and systematic understanding of the importance of automatic processing in emotion dysregulation. PMID:22858684

Genetic disorders of thyroid metabolism and brain development

PubMed Central

Kurian, Manju A; Jungbluth, Heinz

2014-01-01

Normal thyroid metabolism is essential for human development, including the formation and functioning of the central and peripheral nervous system. Disorders of thyroid metabolism are increasingly recognized within the spectrum of paediatric neurological disorders. Both hypothyroid and hyperthyroid disease states (resulting from genetic and acquired aetiologies) can lead to characteristic neurological syndromes, with cognitive delay, extrapyramidal movement disorders, neuropsychiatric symptoms, and neuromuscular manifestations. In this review, the neurological manifestations of genetic disorders of thyroid metabolism are outlined, with particular focus on Allan-Herndon-Dudley syndrome and benign hereditary chorea. We report in detail the clinical features, major neurological and neuropsychiatric manifestations, molecular genetic findings, disease mechanisms, and therapeutic strategies for these emerging genetic ‘brain-thyroid’ disorders. PMID:24665922

Association of acquired thrombotic thrombocytopaenic purpura in a patient with pernicious anaemia

PubMed Central

Podder, Sidhertha; Cervates, Jose; Dey, Bimalangshu R

2015-01-01

Pernicious anaemia is an autoimmune disease caused by intrinsic factor antibody; it leads to vitamin B12 deficiency and is marked by ineffective erythropoiesis. Haematological features reveal macrocytosis, hyperchromasia and hypersegmented neutrophils. Schistocytes are typically seen in microangiopathy, such as in thrombotic thrombocytopaenic purpura (TTP)/haemolytic uraemic syndrome or disseminated intravascular haemolysis (DIC). We report a case of a patient with severe anaemia who presented to the emergency room. Peripheral smear revealed macrocytosis, hypersegmented neutrophils and marked schistocytosis. The patient also had high reticulocyte count with high serum lactate dehydrogenase, elevated D-dimer, low fibrinogen and low haptoglobin. Vitamin B12 level came back low and the presence of intrinsic factor antibody confirmed pernicious anaemia. ADAMTS13 level was noted to be mildly reduced, which raised the suspicion of the association of acquired TTP with pernicious anaemia. Acquired TTP is another autoimmune disorder and its association with pernicious anaemia needs further evaluation. PMID:26464409

Association of acquired thrombotic thrombocytopaenic purpura in a patient with pernicious anaemia.

PubMed

Podder, Sidhertha; Cervates, Jose; Dey, Bimalangshu R

2015-10-13

Pernicious anaemia is an autoimmune disease caused by intrinsic factor antibody; it leads to vitamin B12 deficiency and is marked by ineffective erythropoiesis. Haematological features reveal macrocytosis, hyperchromasia and hypersegmented neutrophils. Schistocytes are typically seen in microangiopathy, such as in thrombotic thrombocytopaenic purpura (TTP)/haemolytic uraemic syndrome or disseminated intravascular haemolysis (DIC). We report a case of a patient with severe anaemia who presented to the emergency room. Peripheral smear revealed macrocytosis, hypersegmented neutrophils and marked schistocytosis. The patient also had high reticulocyte count with high serum lactate dehydrogenase, elevated D-dimer, low fibrinogen and low haptoglobin. Vitamin B12 level came back low and the presence of intrinsic factor antibody confirmed pernicious anaemia. ADAMTS13 level was noted to be mildly reduced, which raised the suspicion of the association of acquired TTP with pernicious anaemia. Acquired TTP is another autoimmune disorder and its association with pernicious anaemia needs further evaluation. 2015 BMJ Publishing Group Ltd.

Magnetic resonance imaging depiction of acquired Dyke-Davidoff-Masson syndrome with crossed cerebro-cerebellar diaschisis: Report of two cases.

PubMed

Gupta, Ranjana; Joshi, Sandeep; Mittal, Amit; Luthra, Ishita; Mittal, Puneet; Verma, Vibha

2015-01-01

Acquired Dyke-Davidoff-Masson syndrome, also known as hemispheric atrophy, is characterized by loss of volume of one cerebral hemisphere from an insult in early life. Crossed cerebellar diaschisis refers to dysfunction/atrophy of cerebellar hemisphere which is secondary to contralateral supratentorial insult. We describe magnetic resonance imaging findings in two cases of acquired Dyke-Davidoff-Masson syndrome with crossed cerebro-cerebellar diaschisis.

Audiological and electrophysiological evaluation of children with acquired immunodeficiency syndrome (AIDS).

PubMed

Matas, Carla Gentile; Leite, Renata Aparecida; Magliaro, Fernanda Cristina Leite; Gonçalves, Isabela Crivellaro

2006-08-01

We examined the peripheral auditory system and the auditory brainstem pathway of children with Acquired Immunodeficiency Syndrome (AIDS). One hundred and one children, 51 with AIDS diagnosis and 50 normal children were evaluated. Audiological assessment included immittance measures, pure tone and speech audiometry and auditory brainstem response (ABR). The children with AIDS more frequently had abnormal results than did their matched controls, presenting either peripheral or auditory brainstem impairment. We suggest that AIDS be considered a risk factor for peripheral and/or auditory brainstem disorders. Further research should be carried out to investigate the auditory effects of HIV infection along the auditory pathway.

How trait anxiety, interpretation bias and memory affect acquired fear in children learning about new animals.

PubMed

Field, Zoë C; Field, Andy P

2013-06-01

Cognitive models of vulnerability to anxiety propose that information processing biases such as interpretation bias play a part in the etiology and maintenance of anxiety disorders. However, at present little is known about the role of memory in information processing accounts of child anxiety. The current study investigates the relationships between interpretation biases, memory and fear responses when learning about new stimuli. Children (aged 8-11 years) were presented with ambiguous information regarding a novel animal, and their fear, interpretation bias, and memory for the information was measured. The main findings were: (1) trait anxiety and interpretation bias significantly predicted acquired fear; (2) interpretation bias did not significantly mediate the relationship between trait anxiety and acquired fear; (3) interpretation bias appeared to be a more important predictor of acquired fear than trait anxiety per se; and (4) the relationship between interpretation bias and acquired fear was not mediated by the number of negative memories but was mediated by the number of positive and false-positive memories. The findings suggest that information processing models of child anxiety need to explain the role of positive memory in the formation of fear responses.

Tinea pedis acquired in mosques?

PubMed

Veraldi, Stefano; Esposito, Luigi; Gorani, Alberto

2018-03-30

Interdigital tinea pedis is a mycotic infection of the feet which occurs mainly in adult males. The transmission is due to repeated contacts with the soil. In Italy, Trichophyton rubrum, T. mentagrophytes (including T. mentagrophytes var. interdigitale) and Epidermophyton floccosum are the most frequently involved species. Interdigital tinea pedis is characterized clinically by maceration, erythema, vesicles, scales, crusts, erosions and ulcers located at the 2 nd , 3 rd and 4 th interdigital folds. Interdigital tinea pedis may be asymptomatic; however, when erosions and ulcers occur, the patients may complain of pruritus, burning sensation and pain. Superinfections, caused by Candida albicans and Gram-negative bacteria, are not rare. 1 We present four cases of interdigital tinea pedis which was probably acquired in Turkish mosques and holy Muslim places. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

Comorbidity in Hoarding Disorder

PubMed Central

Frost, Randy O.; Steketee, Gail; Tolin, David F.

2011-01-01

Hoarding Disorder (HD) is currently under consideration for inclusion as a distinct disorder in DSM-5 (1). Few studies have examined comorbidity patterns in people who hoard, and the ones that have suffer from serious methodological shortcomings including drawing from populations already diagnosed with obsessive compulsive disorder (OCD), using outdated definitions of hoarding, and relying on inadequate assessments of hoarding. The present study is the first large-scale study (n=217) of comorbidity in a sample of people meeting recently proposed criteria for hoarding disorder (1) and relying on validated assessment procedures. The HD sample was compared to 96 participants meeting criteria for OCD without HD. High comorbidity rates were observed for major depressive disorder (MDD) as well as acquisition-related impulse control disorders (compulsive buying, kleptomania, and acquiring free things). Fewer than 20% of HD participants met criteria for OCD, and the rate of OCD in HD was higher for men than women. Rates of MDD and acquisition-related impulse control disorders were higher among HD than OCD participants. No specific anxiety disorder was more frequent in HD, but social phobia was more frequent among men with HD than among men with OCD. Inattentive ADHD was diagnosed in 28% of HD participants and was significantly more frequent than among OCD participants (3%). These findings form important base rates for developing research and treatments for hoarding disorder. PMID:21770000

Characterizing Communicative Development in Children Referred for Autism Spectrum Disorders Using the MacArthur-Bates Communicative Development Inventory (CDI)

ERIC Educational Resources Information Center

Luyster, Rhiannon; Lopez, Kristina; Lord, Catherine

2007-01-01

Characterizing early communicative development in children with Autism Spectrum Disorders (ASD) is valuable for understanding profiles of ability in this population. The current investigation was modeled on Charman, Drew, Baird & Baird (2003b). Analyses explored parent report of early vocabulary, non-verbal communication, functional object use and…

Perioperative management of patients with congenital or acquired disorders of the QT interval.

PubMed

O'Hare, M; Maldonado, Y; Munro, J; Ackerman, M J; Ramakrishna, H; Sorajja, D

2018-04-01

QT prolongation can be attributable to various causes that can be categorised as acquired or congenital. Arrhythmias related to QT prolongation can result in clinical presentations, such as syncope and sudden cardiac death. The perioperative period presents a number of issues that may affect a patient's risk of developing polymorphic ventricular tachycardia or torsades de pointes. Although most patients may have an unremarkable perioperative course, some may have complications; this review article aims to help clinicians avoid potential complications, and to help them address treatment for perioperative issues that may occur. Copyright © 2018 British Journal of Anaesthesia. Published by Elsevier Ltd. All rights reserved.

OrbView-3 Initial On-Orbit Characterization

NASA Technical Reports Server (NTRS)

Ross, Kent; Blonski, Slawomir; Holekamp, Kara; Pagnutti, Mary; Zanoni, Vicki; Carver, David; Fendley, Debbie; Smith, Charles

2004-01-01

NASA at Stennis Space Center (SSC) established a Space Act Agreement with Orbital Sciences Corporation (OSC) and ORBIMAGE Inc. to collaborate on the characterization of the OrbView-3 system and its imagery products and to develop characterization techniques further. In accordance with the agreement, NASA performed an independent radiometric, spatial, and geopositional accuracy assessment of OrbView-3 imagery acquired before completion of the system's initial on-orbit checkout. OSC acquired OrbView-3 imagery over SSC from July 2003 through January 2004, and NASA collected ground reference information coincident with many of these acquisitions. After evaluating all acquisitions, NASA deemed two multispectral images and five panchromatic images useful for characterization. NASA then performed radiometric, spatial, and geopositional characterizations.

Acquired hyperpigmentations*

PubMed Central

Cestari, Tania Ferreira; Dantas, Lia Pinheiro; Boza, Juliana Catucci

2014-01-01

Cutaneous hyperpigmentations are frequent complaints, motivating around 8.5% of all dermatological consultations in our country. They can be congenital, with different patterns of inheritance, or acquired in consequence of skin problems, systemic diseases or secondary to environmental factors. The vast majority of them are linked to alterations on the pigment melanin, induced by different mechanisms. This review will focus on the major acquired hyperpigmentations associated with increased melanin, reviewing their mechanisms of action and possible preventive measures. Particularly prominent aspects of diagnosis and therapy will be emphasized, with focus on melasma, post-inflammatory hyperpigmentation, periorbital pigmentation, dermatosis papulosa nigra, phytophotodermatoses, flagellate dermatosis, erythema dyschromicum perstans, cervical poikiloderma (Poikiloderma of Civatte), acanthosis nigricans, cutaneous amyloidosis and reticulated confluent dermatitis PMID:24626644

Acquired pulmonary artery stenosis in four dogs.

PubMed

Scansen, Brian A; Schober, Karsten E; Bonagura, John D; Smeak, Daniel D

2008-04-15

4 dogs with acquired pulmonary artery stenosis (PAS) were examined for various clinical signs. One was a mixed-breed dog with congenital valvular PAS that subsequently developed peripheral PAS, one was a Golden Retriever with pulmonary valve fibrosarcoma, one was a Pembroke Welsh Corgi in which the left pulmonary artery had inadvertently been ligated during surgery for correction of patent ductus arteriosus, and one was a Boston Terrier with a heart-base mass compressing the pulmonary arteries. All 4 dogs were evaluated with 2-dimensional and Doppler echocardiography to characterize the nature and severity of the stenoses; other diagnostic tests were also performed. The mixed-breed dog with valvular and peripheral PAS was euthanized, surgical resection of the pulmonic valve mass was performed in the Golden Retriever, corrective surgery was performed on the Pembroke Welsh Corgi with left pulmonary artery ligation, and the Boston Terrier with the heart-base mass was managed medically. Acquired PAS in dogs may manifest as a clinically silent heart murmur, syncope, or right-sided heart failure. The diagnosis is made on the basis of imaging findings, particularly results of 2-dimensional and Doppler echocardiography. Treatment may include surgical, interventional, or medical modalities and is targeted at resolving the inciting cause.

Acquired Amino Acid Deficiencies: A Focus on Arginine and Glutamine.

PubMed

Morris, Claudia R; Hamilton-Reeves, Jill; Martindale, Robert G; Sarav, Menaka; Ochoa Gautier, Juan B

2017-04-01

Nonessential amino acids are synthesized de novo and therefore not diet dependent. In contrast, essential amino acids must be obtained through nutrition since they cannot be synthesized internally. Several nonessential amino acids may become essential under conditions of stress and catabolic states when the capacity of endogenous amino acid synthesis is exceeded. Arginine and glutamine are 2 such conditionally essential amino acids and are the focus of this review. Low arginine bioavailability plays a pivotal role in the pathogenesis of a growing number of varied diseases, including sickle cell disease, thalassemia, malaria, acute asthma, cystic fibrosis, pulmonary hypertension, cardiovascular disease, certain cancers, and trauma, among others. Catabolism of arginine by arginase enzymes is the most common cause of an acquired arginine deficiency syndrome, frequently contributing to endothelial dysfunction and/or T-cell dysfunction, depending on the clinical scenario and disease state. Glutamine, an arginine precursor, is one of the most abundant amino acids in the body and, like arginine, becomes deficient in several conditions of stress, including critical illness, trauma, infection, cancer, and gastrointestinal disorders. At-risk populations are discussed together with therapeutic options that target these specific acquired amino acid deficiencies.

[Acquired caustic vagina stenosis: Surgical outcomes of 21 cases].

PubMed

Séni, K; Horo, A G; Koffi, A; Aka, K E; Fomba, M; Koné, M

2016-03-01

To document epidemiology, causes, anatomical varieties and surgical management outcomes of caustic acquired vagina stenosis. Retrospective study of 21 patients involved from 1996 to 2012 at the department of obstetrics and gynecology of Yopougon's teaching hospital in Abidjan, Côte d'Ivoire. Vaginal stenosis due to genital malformation, vaginal irradiation of pelvic tumours, repair of bladder and vaginal fistulae or intersexual disorders were excluded. The prevalence was 0.14/1000 admissions, mean age was 32.1 years, mean gravidity was 1.76 and mean parity was 1.1. Vaginal stenosis seat and extent were variable and 100 % had a caustic origin by use of traditional medicine vaginal pessaire. Of the patients, 95.2 % underwent surgical treatment followed by several dilations sessions with glass dilators. Successful surgical outcome allowing coitus was achieved in 47.6 % of cases with 52.4 % failure. Acquired caustic vagina stenosis are frequently occurring and are a public health problem related to ignorance. Health education and establishment of expert centers with exchange of experiences in West Africa should be able to improve surgical outcomes. Copyright © 2016. Published by Elsevier SAS.

17 CFR 210.8-06 - Real estate operations acquired or to be acquired.

Code of Federal Regulations, 2010 CFR

2010-04-01

... EXCHANGE COMMISSION FORM AND CONTENT OF AND REQUIREMENTS FOR FINANCIAL STATEMENTS, SECURITIES ACT OF 1933..., INVESTMENT ADVISERS ACT OF 1940, AND ENERGY POLICY AND CONSERVATION ACT OF 1975 Article 8 Financial Statements of Smaller Reporting Companies § 210.8-06 Real estate operations acquired or to be acquired. If...

Evolution of language assessment in patients with acquired neurological disorders in Brazil

PubMed Central

Parente, Maria Alice de Mattos Pimenta; Baradel, Roberta Roque; Fonseca, Rochele Paz; Pereira, Natalie; Carthery-Goulart, Maria Teresa

2014-01-01

The objective of this paper was to describe the evolution of language assessments in patients with acquired neurological diseases over a period of around 45 years from 1970, when interdisciplinarity in Neuropsychology first began in Brazil, to the present day. The first twenty years of data was based on memories of Speech Pathology University Professors who were in charge of teaching aphasia. We then show the contributions of Linguistics, Cognitive Psychology, as well as Psycholinguistic and Psychometric criteria, to language evaluation. Finally, the current panorama of adaptations and creations of validated and standardized instruments is given, based on a search of the databases Pubmed, Scopus and Lilacs. Our closing remarks highlight the diversity in evaluation approaches and the recent tendency of language evaluations linked to new technologies such as brain imaging and computational analysis. PMID:29213904

Intracranial hemorrhage after blunt head trauma in children with bleeding disorders.

PubMed

Lee, Lois K; Dayan, Peter S; Gerardi, Michael J; Borgialli, Dominic A; Badawy, Mohamed K; Callahan, James M; Lillis, Kathleen A; Stanley, Rachel M; Gorelick, Marc H; Dong, Li; Zuspan, Sally Jo; Holmes, James F; Kuppermann, Nathan

2011-06-01

To determine computerized tomography (CT) use and prevalence of traumatic intracranial hemorrhage (ICH) in children with and without congenital and acquired bleeding disorders. We compared CT use and ICH prevalence in children with and without bleeding disorders in a multicenter cohort study of 43 904 children <18 years old with blunt head trauma evaluated in 25 emergency departments. A total of 230 children had bleeding disorders; all had Glasgow Coma Scale (GCS) scores of 14 to 15. These children had higher CT rates than children without bleeding disorders and GCS scores of 14 to 15 (risk ratio, 2.29; 95% CI, 2.15 to 2.44). Of the children who underwent imaging with CT, 2 of 186 children with bleeding disorders had ICH (1.1%; 95% CI, 0.1 to 3.8) , compared with 655 of 14 969 children without bleeding disorders (4.4%; 95% CI, 4.1-4.7; rate ratio, 0.25; 95% CI, 0.06 to 0.98). Both children with bleeding disorders and ICHs had symptoms; none of the children required neurosurgery. In children with head trauma, CTs are obtained twice as often in children with bleeding disorders, although ICHs occurred in only 1.1%, and these patients had symptoms. Routine CT imaging after head trauma may not be required in children without symptoms who have congenital and acquired bleeding disorders. Copyright © 2011 Mosby, Inc. All rights reserved.

Meige's Syndrome: Rare Neurological Disorder Presenting as Conversion Disorder.

PubMed

Debadatta, Mohapatra; Mishra, Ajay K

2013-07-01

Meige's syndrome is a rare neurological syndrome characterized by oromandibular dystonia and blepharospasm. Its pathophysiology is not clearly determined. A 35-year-old female presented to psychiatric department with blepharospasm and oromandibular dystonia with clinical provisional diagnosis of psychiatric disorder (Conversion Disorder). After thorough physical examination including detailed neurological exam and psychiatric evaluation no formal medical or psychiatric diagnosis could be made. The other differential diagnoses of extra pyramidal symptom, tardive dyskinesia, conversion disorder, anxiety disorder were ruled out by formal diagnostic criteria. Consequently with suspicion of Meige's syndrome she was referred to the department of Neurology and the diagnosis was confirmed. Hence, Meige's syndrome could be misdiagnosed as a psychiatric disorder such as conversion disorder or anxiety disorder because clinical features of Meige's syndrome are highly variable and affected by psychological factors and also can be inhibited voluntarily to some extent.

Comorbidity of Autism Spectrum Disorders and Emotional/behavioral Disorders: Towards Improved Diagnostic Procedures, Instructional Programming, and Personnel Preparation

ERIC Educational Resources Information Center

Clinton, Elias

2016-01-01

An emotional/behavioral disorder is a mental health disability characterized by intensive internalized behaviors (e.g., anxiety, depression) and/or externalized behaviors (e.g., physical aggression, verbal aggression). Autism is a neurodevelopmental disorder characterized by deficits in social communication and repetitive behaviors (i.e., stereo…

7 CFR 926.10 - Acquire.

Code of Federal Regulations, 2011 CFR

2011-01-01

... REQUIREMENTS APPLICABLE TO CRANBERRIES NOT SUBJECT TO THE CRANBERRY MARKETING ORDER § 926.10 Acquire. Acquire means to obtain cranberries by any means whatsoever for the purpose of handling cranberries. Effective...

7 CFR 926.10 - Acquire.

Code of Federal Regulations, 2014 CFR

2014-01-01

... REQUIREMENTS APPLICABLE TO CRANBERRIES NOT SUBJECT TO THE CRANBERRY MARKETING ORDER § 926.10 Acquire. Acquire means to obtain cranberries by any means whatsoever for the purpose of handling cranberries. Effective...

7 CFR 926.10 - Acquire.

Code of Federal Regulations, 2012 CFR

2012-01-01

... REQUIREMENTS APPLICABLE TO CRANBERRIES NOT SUBJECT TO THE CRANBERRY MARKETING ORDER § 926.10 Acquire. Acquire means to obtain cranberries by any means whatsoever for the purpose of handling cranberries. Effective...

7 CFR 926.10 - Acquire.

Code of Federal Regulations, 2013 CFR

2013-01-01

... REQUIREMENTS APPLICABLE TO CRANBERRIES NOT SUBJECT TO THE CRANBERRY MARKETING ORDER § 926.10 Acquire. Acquire means to obtain cranberries by any means whatsoever for the purpose of handling cranberries. Effective...

Swallowing Disorders in Severe Brain Injury in the Arousal Phase.

PubMed

Bremare, A; Rapin, A; Veber, B; Beuret-Blanquart, F; Verin, E

2016-08-01

The objective of this study was to determine the clinical characteristics of swallowing disorders in severe brain injury in the arousal phase after coma. Between December 1, 2013 and June 30, 2014, eleven patients with severe acquired brain injury who were admitted to rehabilitation center (Male 81.8 %; 40.7 ± 14.6 years) were included in the study. Evaluation of swallowing included a functional examination, clinical functional swallowing test, and naso-endoscopic swallowing test. All patients had swallowing disorders at admission. The first functional swallowing test showed oral (77.8 %) and pharyngeal (66.7 %) food bolus transport disorders; and alterations in airway protection mechanisms (80 %). Swallowing test under endoscopic control showed a disorder in swallowing coordination in 55.6 % of patients tested. Seven (63.6 %) patients resumed oral feeding within an average of 6 weeks after admission to rehabilitation center and 14 weeks after acquired brain injury. Six (85.7 %) of these seven patients continued to require modified solid and liquid textures. Swallowing disorders are a major concern in severe brain injury in the arousal phase. Early bedside assessment of swallowing is essential for detection of swallowing disorders to propose appropriate medical rehabilitation care to these patients in a state of altered consciousness.

The ketogenic diet is effective for refractory epilepsy associated with acquired structural epileptic encephalopathy.

PubMed

Villaluz, Mel Michel; Lomax, Lysa Boissé; Jadhav, Trupti; Cross, J Helen; Scheffer, Ingrid E

2018-07-01

Ketogenic diet therapies have proven efficacy for refractory epilepsy. There are many reports of their use in the genetic developmental and epileptic encephalopathies; however, little attention has been paid as to whether the diet is also effective in individuals with an acquired structural aetiology. We observed remarkable efficacy of the diet in two patients with hypoxic-ischaemic encephalopathy. We then analysed our cases with refractory structural epilepsies of acquired origin to characterize their response to the ketogenic diet. The classical ketogenic diet was implemented with dietary ratios of 3:1 to 4.4:1. Seizure frequency at 1 month, 3 months, 6 months, 1 year, and 2 years was ascertained. A responder was defined as greater than 50% seizure reduction compared to baseline. Seven of the nine patients were responders at 3 months. Somewhat surprisingly we found that the ketogenic diet was effective in patients with a developmental and epileptic encephalopathy due to an acquired structural aetiology. This cohort may not be routinely considered for the ketogenic diet because of their structural and acquired, rather than genetic, basis. The ketogenic diet should be considered early in the management of patients with acquired structural encephalopathies as it can improve seizure control with the potential to improve developmental outcome. The ketogenic diet was effective in children with epilepsy associated with an acquired structural aetiology. © 2018 Mac Keith Press.

Characterizing Objective Quality of Life and Normative Outcomes in Adults with Autism Spectrum Disorder: An Exploratory Latent Class Analysis

ERIC Educational Resources Information Center

Bishop-Fitzpatrick, Lauren; Hong, Jinkuk; Smith, Leann E.; Makuch, Renee A.; Greenberg, Jan S.; Mailick, Marsha R.

2016-01-01

This study aims to extend the definition of quality of life (QoL) for adults with autism spectrum disorder (ASD, n = 180, ages 23-60) by: (1) characterizing the heterogeneity of normative outcomes (employment, independent living, social engagement) and objective QoL (physical health, neighborhood quality, family contact, mental health issues); and…

Thyroid-related neurological disorders and complications in children.

PubMed

Nandi-Munshi, Debika; Taplin, Craig E

2015-04-01

Thyroid hormones exert critical roles throughout the body and play an important and permissive role in neuroendocrine, neurological, and neuromuscular function. We performed a PubMed search through June 2014 with search terms including "hypothyroidism," "hyperthyroidism," "neurological complications," "neuropathy," "myopathy," "congenital hypothyroidism," and "encephalopathy." Relevant publications reviewed included case series, individual case reports, systematic reviews, retrospective analyses, and randomized controlled trials. The neurological outcomes of congenital hypothyroidism were reviewed, along with the clinical features of associated neuromuscular syndromes of both hypothyroidism and hyperthyroidism, including other autoimmune conditions. Evidence for, and pathophysiological controversies surrounding, Hashimoto encephalopathy was also reviewed. The establishment of widespread newborn screening programs has been highly successful in attenuating or preventing early and irreversible neurological harm resulting from congenital thyroid hormone deficiency, but some children continue to display neuromuscular, sensory, and cognitive defects in later life. Acquired disorders of thyroid function such as Hashimoto thyroiditis and Graves' disease are associated with a spectrum of central nervous system and/or neuromuscular dysfunction. However, considerable variation in clinical phenotype is described, and much of our knowledge of the role of thyroid disease in childhood neurological disorders is derived from adult case series. Early and aggressive normalization of thyroxine levels in newborn infants with congenital hypothyroidism is important in minimizing neurological sequelae, but maternal thyroid hormone sources are also critically important to the early developing brain. A spectrum of neurological disorders has been reported in older children with acquired thyroid disease, but the frequency with which these occur remains poorly defined in the literature, and

Association Between Childhood to Adolescent Attention Deficit/Hyperactivity Disorder Symptom Trajectories and Late Adolescent Disordered Eating.

PubMed

Yilmaz, Zeynep; Javaras, Kristin N; Baker, Jessica H; Thornton, Laura M; Lichtenstein, Paul; Bulik, Cynthia M; Larsson, Henrik

2017-08-01

Disordered eating is more prevalent among adolescents with attention deficit/hyperactivity disorder. Both inattention and hyperactivity/impulsivity symptoms show strong associations with disordered eating, but few investigations of these associations have been longitudinal. Thus, we examined the effect of childhood to adolescent inattention and hyperactivity/impulsivity symptom trajectories on late adolescent disordered eating. We used growth mixture modeling to identify distinct inattention and hyperactivity/impulsivity symptom trajectories (called "classes") across three time points (ages 8-9, 13-14, and 16-17 years) in the Swedish Twin study of CHild and Adolescent Development. The resulting classes were used to predict Eating Disorder Inventory-2 Bulimia, Drive for Thinness, and Body Dissatisfaction subscales at age 16-17 years, with adjustment for sex and body mass index at age 16-17 years. The combined inattention and hyperactivity/impulsivity symptom trajectory classes included: a "low symptom" class characterized by low inattention and hyperactivity/impulsivity throughout childhood/adolescence; a "predominantly inattention" class characterized by elevated inattention, but not hyperactivity/impulsivity, throughout childhood/adolescence; a "predominantly hyp/imp" class characterized by elevated hyperactivity/impulsivity, but not inattention, throughout childhood/adolescence; and a "both inattention and hyp/imp" class characterized by elevated inattention and hyperactivity/impulsivity throughout childhood/adolescence. After adjusting for sex and body mass index or sex and anxiety/depression symptoms, the "both inattention and hyp/imp" (vs. "low symptom") class predicted significantly higher Eating Disorder Inventory-2 subscale scores during late adolescence. Increased vigilance for disordered eating among children who have both inattention and hyperactivity/impulsivity symptoms throughout childhood and adolescence could aid in early identification of eating

Using an emotional saccade task to characterize executive functioning and emotion processing in attention-deficit hyperactivity disorder and bipolar disorder.

PubMed

Yep, Rachel; Soncin, Stephen; Brien, Donald C; Coe, Brian C; Marin, Alina; Munoz, Douglas P

2018-04-23

Despite distinct diagnostic criteria, attention-deficit hyperactivity disorder (ADHD) and bipolar disorder (BD) share cognitive and emotion processing deficits that complicate diagnoses. The goal of this study was to use an emotional saccade task to characterize executive functioning and emotion processing in adult ADHD and BD. Participants (21 control, 20 ADHD, 20 BD) performed an interleaved pro/antisaccade task (look toward vs. look away from a visual target, respectively) in which the sex of emotional face stimuli acted as the cue to perform either the pro- or antisaccade. Both patient groups made more direction (erroneous prosaccades on antisaccade trials) and anticipatory (saccades made before cue processing) errors than controls. Controls exhibited lower microsaccade rates preceding correct anti- vs. prosaccade initiation, but this task-related modulation was absent in both patient groups. Regarding emotion processing, the ADHD group performed worse than controls on neutral face trials, while the BD group performed worse than controls on trials presenting faces of all valence. These findings support the role of fronto-striatal circuitry in mediating response inhibition deficits in both ADHD and BD, and suggest that such deficits are exacerbated in BD during emotion processing, presumably via dysregulated limbic system circuitry involving the anterior cingulate and orbitofrontal cortex. Copyright © 2018 Elsevier Inc. All rights reserved.

Numeracy Skills in Patients With Degenerative Disorders and Focal Brain Lesions

PubMed Central

Cappelletti, Marinella; Butterworth, Brian; Kopelman, Michael

2012-01-01

Objective: To characterize the numerical profile of patients with acquired brain disorders. Method: We investigated numeracy skills in 76 participants—40 healthy controls and 36 patients with neurodegenerative disorders (Alzheimer dementia, frontotemporal dementia, semantic dementia, progressive aphasia) and with focal brain lesions affecting parietal, frontal, and temporal areas as in herpes simplex encephalitis (HSE). All patients were tested with the same comprehensive battery of paper-and-pencil and computerized tasks assessing numerical abilities and calculation. Degenerative and HSE patients also performed nonnumerical semantic tasks. Results: Our results, based on nonparametric group statistics as well as on the analysis of individual patients, and all highly significant, show that: (a) all patients, including those with parietal lesions—a key brain area for numeracy processing—had intact processing of number quantity; (b) patients with impaired semantic knowledge had much better preserved numerical knowledge; and (c) most patients showed impaired calculation skills, with the exception of most semantic dementia and HSE patients. Conclusion: Our results allow us, for the first time, to characterize the numeracy skills in patients with a variety of neurological conditions and to suggest that the pattern of numerical performance can vary considerably across different neurological populations. Moreover, the selective sparing of calculation skills in most semantic dementia and HSE suggest that numerical abilities are an independent component of the semantic system. Finally, our data suggest that, besides the parietal areas, other brain regions might be critical to the understanding and processing of numerical concepts. PMID:22122516

Acquired simulated brown syndrome following surgical repair of medial orbital wall fracture.

PubMed

Hwang, Jong-uk; Lim, Hyun Taek

2005-03-01

Simulated Brown syndrome is a term applied to a myriad of disorders that cause a Brown syndrome-like motility. We encountered a case of acquired simulated Brown syndrome in a 41-year-old man following surgical repair of fractures of both medial orbital walls. He suffered from diplopia in primary gaze, associated with hypotropia of the affected eye. We performed an ipsilateral recession of the left inferior rectus muscle as a single-stage intraoperative adjustment procedure under topical anesthesia, rather than the direct approach to the superior oblique tendon. Postoperatively, the patient was asymptomatic in all diagnostic gaze positions.

[Gender as a key effect modifier of the relationship between physician work stressors and the acquired cardiovascular disorders].

PubMed

Nedić, Olesja; Belkić, Karen; Filipović, Danka; Jocić, Neda

2008-01-01

To assess gender as an effect-modifier vis-â-vis exposure to work stressors and the acquired cardiovascular disorders (ACVDC). The Occupational Stress Index for physicians was used in a case-control study (>90% participation rate) among physicians in Novi Sad (Cases: 50 males and 51 females with ACVD, Referents: 46 males and 6l females without ACVD). Patterns of disease manifestation, sociodemography, risk factors, Minnesota Multiphasic Personality Inventory (MMPI) tendency and exposure to work stressors differed in male compared to female physicians with ACVD. Accounting for these covariates, the males with ACVD had significantly higher general-level threat avoidance vigilance scores (more witnessed accidents at work and expert-witnessing in court (p < 0.0l)). Female physicians with ACVD had higher general-level underload (more fixed pay) and strictness (more limited decision-making concerning work-schedules and institutional policies) (p < 0.05). In stratified analysis, the relationship between working conditions and ACVD was most apparent among the women physicians. Compared to those without ACVD, female physicians with ACVD showed higher extrinsic time-pressure (speed-up (p < 0.01)), threat of job loss (p < 0.05), and elements of high demand (heterogeneous information, complex and heterogeneous task performance) (p < 0.05)), after accounting for covariates. Females in the surgical care specialties with ACVD were exposed to significantly higher demands, speed-up, and need for constant high attention than their female colleagues without ACVD, after accounting for covariates. Gender is a key effect-modifier of the relationship between exposure to work stressors and ACVD among physicians. Intervention studies should consider gender-specific work stressors, as well as those specific to physicians.

Life after acquired thrombotic thrombocytopenic purpura: morbidity, mortality, and risks during pregnancy.

PubMed

Vesely, S K

2015-06-01

Patients who have recovered from their acute episode of acquired ADAMTS13-deficient thrombotic thrombocytopenic purpura (TTP) were once thought to have complete recovery except for risk of relapse. Data from previous publications from the Oklahoma TTP-hemolytic uremic syndrome (HUS) Registry are summarized. Patients have decreased cognitive function and increased prevalence of hypertension, systemic lupus erythematosus, major depression, and albuminuria as compared to the expected values from the US population. The proportion of patients that died during the follow-up period was greater than expected based on the US population reference population. Among women who had a pregnancy following recovery from TTP, relapse during pregnancy or postpartum is uncommon, but the occurrence of preeclampsia may be increased. Thirteen of 16 pregnancies in these women resulted in healthy children. Increased morbidity and mortality in TTP patients following recovery suggest that TTP may be more of a chronic disorder than a disorder with acute episodes and complete recovery. © 2015 International Society on Thrombosis and Haemostasis.

Mesenchymal Stem Cell Benefits Observed in Bone Marrow Failure and Acquired Aplastic Anemia

PubMed Central

Gonzaga, Vivian Fonseca; Lisboa, Gustavo Sabino; Frare, Eduardo Osório

2017-01-01

Acquired aplastic anemia (AA) is a type of bone marrow failure (BMF) syndrome characterized by partial or total bone marrow (BM) destruction resulting in peripheral blood (PB) pancytopenia, which is the reduction in the number of red blood cells (RBC) and white blood cells (WBC), as well as platelets (PLT). The first-line treatment option of AA is given by hematopoietic stem cell (HSCs) transplant and/or immunosuppressive (IS) drug administration. Some patients did not respond to the treatment and remain pancytopenic following IS drugs. The studies are in progress to test the efficacy of adoptive cellular therapies as mesenchymal stem cells (MSCs), which confer low immunogenicity and are reliable allogeneic transplants in refractory severe aplastic anemia (SAA) cases. Moreover, bone marrow stromal cells (BMSC) constitute an essential component of the hematopoietic niche, responsible for stimulating and enhancing the proliferation of HSCs by secreting regulatory molecules and cytokines, providing stimulus to natural BM microenvironment for hematopoiesis. This review summarizes scientific evidences of the hematopoiesis improvements after MSC transplant, observed in acquired AA/BMF animal models as well as in patients with acquired AA. Additionally, we discuss the direct and indirect contribution of MSCs to the pathogenesis of acquired AA. PMID:29333168

Neural Correlates of Phonological Processing in Speech Sound Disorder: A Functional Magnetic Resonance Imaging Study

ERIC Educational Resources Information Center

Tkach, Jean A.; Chen, Xu; Freebairn, Lisa A.; Schmithorst, Vincent J.; Holland, Scott K.; Lewis, Barbara A.

2011-01-01

Speech sound disorders (SSD) are the largest group of communication disorders observed in children. One explanation for these disorders is that children with SSD fail to form stable phonological representations when acquiring the speech sound system of their language due to poor phonological memory (PM). The goal of this study was to examine PM in…

Brain structure–function associations in multi-generational families genetically enriched for bipolar disorder

PubMed Central

Schür, Remmelt; Sjouwerman, Rachel; Service, Susan K.; Araya, Carmen; Araya, Xinia; Bejarano, Julio; Knowles, Emma; Gomez-Makhinson, Juliana; Lopez, Maria C.; Aldana, Ileana; Teshiba, Terri M.; Abaryan, Zvart; Al-Sharif, Noor B.; Navarro, Linda; Tishler, Todd A.; Altshuler, Lori; Bartzokis, George; Escobar, Javier I.; Glahn, David C.; Thompson, Paul M.; Lopez-Jaramillo, Carlos; Macaya, Gabriel; Molina, Julio; Reus, Victor I.; Sabatti, Chiara; Cantor, Rita M.; Freimer, Nelson B.; Bearden, Carrie E.

2015-01-01

Recent theories regarding the pathophysiology of bipolar disorder suggest contributions of both neurodevelopmental and neurodegenerative processes. While structural neuroimaging studies indicate disease-associated neuroanatomical alterations, the behavioural correlates of these alterations have not been well characterized. Here, we investigated multi-generational families genetically enriched for bipolar disorder to: (i) characterize neurobehavioural correlates of neuroanatomical measures implicated in the pathophysiology of bipolar disorder; (ii) identify brain–behaviour associations that differ between diagnostic groups; (iii) identify neurocognitive traits that show evidence of accelerated ageing specifically in subjects with bipolar disorder; and (iv) identify brain–behaviour correlations that differ across the age span. Structural neuroimages and multi-dimensional assessments of temperament and neurocognition were acquired from 527 (153 bipolar disorder and 374 non-bipolar disorder) adults aged 18–87 years in 26 families with heavy genetic loading for bipolar disorder. We used linear regression models to identify significant brain–behaviour associations and test whether brain–behaviour relationships differed: (i) between diagnostic groups; and (ii) as a function of age. We found that total cortical and ventricular volume had the greatest number of significant behavioural associations, and included correlations with measures from multiple cognitive domains, particularly declarative and working memory and executive function. Cortical thickness measures, in contrast, showed more specific associations with declarative memory, letter fluency and processing speed tasks. While the majority of brain–behaviour relationships were similar across diagnostic groups, increased cortical thickness in ventrolateral prefrontal and parietal cortical regions was associated with better declarative memory only in bipolar disorder subjects, and not in non

Characterizing the experience of agitation in patients with bipolar disorder and schizophrenia.

PubMed

Roberts, Jenna; Gracia Canales, Alfredo; Blanthorn-Hazell, Sophee; Craciun Boldeanu, Anca; Judge, Davneet

2018-04-16

Agitation is a common manifestation of bipolar disorder and schizophrenia which includes symptoms ranging from inner tension and unease to violence and aggression. Much of the existing literature has focused on agitation in the acute setting, with the patient experience poorly defined. Thus, the aim of this study was to characterize agitation and its management from a patient perspective, with the focus on those who reside in the community. Surveys were completed across Germany, Spain and the UK by 583 community dwelling patients with schizophrenia or bipolar disorder who experienced episodes of agitation. Patients were recruited via either their physician or through patient support groups. The survey captured information on demographics, disease characteristics, frequency of agitation episodes and different pre-defined severity levels ranging from mild to severe, symptoms experienced during an episode, awareness of agitation and coping strategies employed by the patient. Statistics were descriptive in nature. The most commonly reported symptoms during an episode of agitation were feeling uneasy (n = 373, 64%), restless (n = 368, 63%) or nervous (n = 368, 63%). Patients experienced an average of 22.4 (SD 57.2) mild, 15.4 (SD 61.2) moderate, 6.8 (SD 63.3) moderate-intense and 2.9 (SD 24.4) severe episodes within the last 12 months; on average 2.7 (SD 6.8) required hospital attendance. Half of patients (n = 313) had attended hospital due to agitation. In total, 71% of patients (n = 412) were aware they were becoming agitated either always or sometimes and 61% of patients (n = 347) were aware of agitation triggers either always or sometimes. The majority of patients reported being able to sometimes control their agitation (56%, n = 329) but 16% (n = 94) stated that there is typically nothing they can do. To cope with episodes 55% (n = 125) of schizophrenia patients and 66% (n = 234) of bipolar disorder patients reported taking

Implementation and clinical characteristics of a posttraumatic stress disorder brain collection.

PubMed

Mighdoll, Michelle I; Deep-Soboslay, Amy; Bharadwaj, Rahul A; Cotoia, John A; Benedek, David M; Hyde, Thomas M; Kleinman, Joel E

2018-01-01

A postmortem human brain collection to study posttraumatic stress disorder (PTSD) is critical for uncovering the molecular mechanisms that contribute to this psychiatric disorder. We describe here the PTSD brain collection at the Lieber Institute for Brain Development in Baltimore, Maryland, consisting of postmortem brain donations acquired between 2012 and 2017. Thus far, 87 brains from individuals meeting DSM-5 criteria for PTSD were collected after consent was obtained from legal next-of-kin, and subsequently clinically characterized for molecular studies. PTSD brain donors had high rates of comorbid diagnoses, including depression (62.1%), substance abuse (74.7%), drug-related death (69.0%), and suicide completion (17.2%). PTSD cases were subdivided into two categories: combat-related PTSD (n = 24) and noncombat/domestic PTSD (n = 63). The major differences between the combat-related and domestic PTSD cohorts were sex, drug-related death, and the prevalence of bipolar disorder (BPD) comorbidity. The combat-related group was entirely male, with only one BPD subject (4.2%), and had significantly fewer drug-related deaths (45.8%) in contrast to the domestic group (31.8% male, 36.5% bipolar, and 77.8% drug-related deaths). Medical examiners' offices, particularly in areas with higher military populations, are an excellent source for PTSD brain donations of both combat-related and domestic PTSD. © 2017 Wiley Periodicals, Inc.

Neuropsychiatric disorders in Cushing's syndrome

PubMed Central

Pivonello, Rosario; Simeoli, Chiara; De Martino, Maria Cristina; Cozzolino, Alessia; De Leo, Monica; Iacuaniello, Davide; Pivonello, Claudia; Negri, Mariarosaria; Pellecchia, Maria Teresa; Iasevoli, Felice; Colao, Annamaria

2015-01-01

Endogenous Cushing's syndrome (CS), a rare endocrine disorder characterized by cortisol hypersecretion, is associated with psychiatric and neurocognitive disorders. Major depression, mania, anxiety, and neurocognitive impairment are the most important clinical abnormalities. Moreover, patients most often complain of impairment in quality of life, interference with family life, social, and work performance. Surprisingly, after hypercortisolism resolution, despite the improvement of the overall prevalence of psychiatric and neurocognitive disorders, the brain volume loss at least partially persists and it should be noted that some patients may still display depression, anxiety, panic disorders, and neurocognitive impairment. This brief review aimed at describing the prevalence of psychiatric and neurocognitive disorders and their characterization both during the active and remission phases of CS. The last section of this review is dedicated to quality of life, impaired during active CS and only partially resolved after resolution of hypercortisolism. PMID:25941467

[Acquired dyslexias and dysgraphias under the prism of cognitive neuropsychology: a model for the Spanish language].

PubMed

Böhm, P; Diéguez-Vide, F; Peña-Casanova, J; Tainturier, M J; Lecours, A R

2000-02-01

The present paper discusses the different clinical manifestations of acquired disorders of reading and writing from a neurocognitive viewpoint. Based on a specific functional architecture of reading and writing--a cognitive model; presented as well--the different syndromes of acquired dyslexias and dysgraphias, that have been described in the specialized literature during the last 25 years, will be reviewed. The different pathologies are distributed along three different functional axes: a plurimodal component, including the semantic system, for the description of peripheric disorders of reading and writing; a lexical block which is justified by the findings in patients with surface dyslexia/dysgraphia; and a third, sublexical component, in order to illustrate the different functional impairments in phonological dyslexia/dysgraphia. Following the description of syndromes due to selective "functional lesions", we discuss deep dyslexia/dysgraphia, a syndrome due to multiple functional lesions. All of the syndromes will be justified and discussed with respect to the different components of the functional architecture presented and are based on cases of the literature and personal observations. Concluding remarks will evaluate the new insights gained by the presented functional arquitecture in relation to other cognitive models for the analysis of reading aloud and writing to dictation of single words.

Clumsiness in Children: Developmental Coordination Disorder.

ERIC Educational Resources Information Center

Fox, Mervyn A.

1998-01-01

Explores the diagnostic criteria of developmental coordination disorder, a condition that is characterized by motor awkwardness and has a strong association with psychiatric disorders and learning disabilities. Delineates the nature of developmental coordination disorder and discusses its treatment through occupational therapy and cognitive…

7 CFR 926.10 - Acquire.

Code of Federal Regulations, 2010 CFR

2010-01-01

... of the Department of Agriculture (Continued) AGRICULTURAL MARKETING SERVICE (Marketing Agreements and... REQUIREMENTS APPLICABLE TO CRANBERRIES NOT SUBJECT TO THE CRANBERRY MARKETING ORDER § 926.10 Acquire. Acquire means to obtain cranberries by any means whatsoever for the purpose of handling cranberries. Effective...

Acquired word deafness, and the temporal grain of sound representation in the primary auditory cortex.

PubMed

Phillips, D P; Farmer, M E

1990-11-15

This paper explores the nature of the processing disorder which underlies the speech discrimination deficit in the syndrome of acquired word deafness following from pathology to the primary auditory cortex. A critical examination of the evidence on this disorder revealed the following. First, the most profound forms of the condition are expressed not only in an isolation of the cerebral linguistic processor from auditory input, but in a failure of even the perceptual elaboration of the relevant sounds. Second, in agreement with earlier studies, we conclude that the perceptual dimension disturbed in word deafness is a temporal one. We argue, however, that it is not a generalized disorder of auditory temporal processing, but one which is largely restricted to the processing of sounds with temporal content in the milliseconds to tens-of-milliseconds time frame. The perceptual elaboration of sounds with temporal content outside that range, in either direction, may survive the disorder. Third, we present neurophysiological evidence that the primary auditory cortex has a special role in the representation of auditory events in that time frame, but not in the representation of auditory events with temporal grains outside that range.

Community-acquired pneumonia.

PubMed

Falguera, M; Ramírez, M F

2015-11-01

This article not only reviews the essential aspects of community-acquired pneumonia for daily clinical practice, but also highlights the controversial issues and provides the newest available information. Community-acquired pneumonia is considered in a broad sense, without excluding certain variants that, in recent years, a number of authors have managed to delineate, such as healthcare-associated pneumonia. The latter form is nothing more than the same disease that affects more frail patients, with a greater number of risk factors, both sharing an overall common approach. Copyright © 2015 Elsevier España, S.L.U. y Sociedad Española de Medicina Interna (SEMI). All rights reserved.

Optimal management of common acquired melanocytic nevi (moles): current perspectives

PubMed Central

Sardana, Kabir; Chakravarty, Payal; Goel, Khushbu

2014-01-01

Although common acquired melanocytic nevi are largely benign, they are probably one of the most common indications for cosmetic surgery encountered by dermatologists. With recent advances, noninvasive tools can largely determine the potential for malignancy, although they cannot supplant histology. Although surgical shave excision with its myriad modifications has been in vogue for decades, the lack of an adequate histological sample, the largely blind nature of the procedure, and the possibility of recurrence are persisting issues. Pigment-specific lasers were initially used in the Q-switched mode, which was based on the thermal relaxation time of the melanocyte (size 7 μm; 1 μsec), which is not the primary target in melanocytic nevus. The cluster of nevus cells (100 μm) probably lends itself to treatment with a millisecond laser rather than a nanosecond laser. Thus, normal mode pigment-specific lasers and pulsed ablative lasers (CO2/erbium [Er]:yttrium aluminum garnet [YAG]) are more suited to treat acquired melanocytic nevi. The complexities of treating this disorder can be overcome by following a structured approach by using lasers that achieve the appropriate depth to treat the three subtypes of nevi: junctional, compound, and dermal. Thus, junctional nevi respond to Q-switched/normal mode pigment lasers, where for the compound and dermal nevi, pulsed ablative laser (CO2/Er:YAG) may be needed. If surgical excision is employed, a wide margin and proper depth must be ensured, which is skill dependent. A lifelong follow-up for recurrence and melanoma is warranted in predisposed individuals, although melanoma is decidedly uncommon in most acquired melanocytic nevi, even though histological markers may be seen on evaluation. PMID:24672253

Dysphagia associated with cervical spine and postural disorders.

PubMed

Papadopoulou, Soultana; Exarchakos, Georgios; Beris, Alexander; Ploumis, Avraam

2013-12-01

Difficulties with swallowing may be both persistent and life threatening for the majority of those who experience it irrespective of age, gender, and race. The purpose of this review is to define oropharyngeal dysphagia and describe its relationship to cervical spine disorders and postural disturbances due to either congenital or acquired disorders. The etiology and diagnosis of dysphagia are analyzed, focusing on cervical spine pathology associated with dysphagia as severe cervical spine disorders and postural disturbances largely have been held accountable for deglutition disorders. Scoliosis, kyphosis–lordosis, and osteophytes are the primary focus of this review in an attempt to elucidate the link between cervical spine disorders and dysphagia. It is important for physicians to be knowledgeable about what triggers oropharyngeal dysphagia in cases of cervical spine and postural disorders. Moreover, the optimum treatment for dysphagia, including the use of therapeutic maneuvers during deglutition, neck exercises, and surgical treatment, is discussed.

Differentiating hypochondriasis from panic disorder.

PubMed

Hiller, Wolfgang; Leibbrand, Rolf; Rief, Winfried; Fichter, Manfred M

2005-01-01

Hypochondriasis and panic disorder are both characterized by prevalent health anxieties and illness beliefs. Therefore, the question as to whether they represent distinct nosological entities has been raised. This study examines how clinical characteristics can be used to differentiate both disorders, taking the possibility of mixed symptomatologies (comorbidity) into account. We compared 46 patients with hypochondriasis, 45 with panic disorder, and 21 with comorbid hypochondriasis plus panic disorder. While panic patients had more comorbidity with agoraphobia, hypochondriasis was more closely associated with somatization. Patients with panic disorder were less pathological than hypochondriacal patients on all subscales of the Whiteley Index (WI) and the Illness Attitude Scales (IAS) except for illness behavior. These differences were independent of somatization. Patients with hypochondriasis plus panic had higher levels of anxiety, more somatization, more general psychopathology and a trend towards increased health care utilization. Clinicians were able to distinguish between patient groups based upon the tendency of hypochondriacal patients to demand unnecessary medical treatments. These results confirm that hypochondriasis and panic disorder are distinguishable clinical conditions, characterized by generally more psychopathology and distress in hypochondriasis.

Hospital-acquired pneumonia

MedlinePlus

... pneumonia; HCAP Patient Instructions Pneumonia in adults - discharge Images Hospital-acquired pneumonia Respiratory system References Chastre J, Luyt C-E. Ventilator-associated pneumonia. In: Broaddus ...

Sleep Patterns in Children with Attention-Deficit/Hyperactivity Disorder, Tic Disorder, and Comorbidity

ERIC Educational Resources Information Center

Kirov, Roumen; Kinkelbur, Joerg; Banaschewski, Tobias; Rothenberger, Aribert

2007-01-01

Background: In children, attention-deficit/hyperactivity disorder (ADHD), tic disorder (TD), and their coexistence (ADHD + TD comorbidity) are very common and clinically important. Associated sleep patterns and their clinical role are still insufficiently investigated. This study aimed at characterizing these sleep patterns in children with ADHD,…

New Low-Temperature Magnetic Data Acquired on Synthetic Lepidocrocite

NASA Astrophysics Data System (ADS)

Guyodo, Y.; Bonville, P.; Ona-Nguema, G.; Carvallo, C.; Wang, Y.; Morin, G.

2007-12-01

Lepidocrocite (γ-FeOOH) is an iron oxyhydroxide commonly found in the environment, which is assumed to be antiferromagnetic with a small ferromagnetic-like behavior and a Néel temperature of about 50K (e.g., Hirt et al., 2002, JGR, 107, 10.1029/2001JB000242). It is currently used as starting material in bio- reduction experiments leading to the formation of Fe(II)-bearing minerals such as green rusts, magnetite, and siderite (e.g., Ona-Nguema et al., 2002, Environ. Sci. Technol., 36, 16-20). Both initial and resulting materials are being characterized using various techniques including low-temperature magnetic methods. At this meeting, results obtained on the initial synthetic lepidocrocite samples will be presented, which describe an unusual magnetic behavior. In particular, field cooled and zero field cooled induced magnetization curves (obtained using a 5mT magnetic induction) merge at a temperature around 150K (well above 50K). Below this temperature, the difference between the two curves can be qualified as a remanent magnetization, acquired during cooling of the sample in the presence of a magnetic field. As a consequence, some ferromagnetic-like behavior persists at temperatures above the admitted Néel temperature. The cooling/warming cycle of the room temperature remanent magnetization (acquired using a 2.5T magnetic induction) also indicates that some remanence can be acquired well above that temperature. Other types of measurement have been performed in order to better constrain the low-temperature magnetic behavior of these samples, in particular using a high-field VSM.

Inherited and acquired thrombophilia in Indian women experiencing unexplained recurrent pregnancy loss.

PubMed

Patil, Rucha; Ghosh, Kanjaksha; Vora, Sonal; Shetty, Shrimati

2015-10-01

The most frequently hypothesized cause of unexplained recurrent pregnancy loss (RPL) refers to a defective maternal haemostatic response leading to uteroplacental thrombosis. Approximately 20% women suffering from pregnancy loss (PL) are associated with autoimmune disorders and more than 50% remain idiopathic after common traditional investigations. The present study aims to investigate the prevalence of different genetic and acquired thrombophilia markers in a large series of Indian women with RPL. Such studies will help analyze the markers which pose maximum risk and help in the appropriate treatment in subsequent pregnancies. The study comprised of 587 women with no apparent etiological causes of RPL and 115 healthy women controls. p values were calculated with two tailed Fisher's exact test; statistical significance was assumed at p<0.05, 95% confidence interval. Relative risks were also calculated. Among genetic thrombophilia, the risk of PL was highest with protein S deficiency (16%, p=0.006) followed by plasminogen activator inhibitor-1 4G/4G (23%, p=0.007) polymorphism. Among acquired markers, the risk of PL was the highest in women with anti-cardiolipin antibodies (24%, p=0.0001), followed by anti-annexin V antibodies (23%, p=0.0009) and lupus anticoagulants (8%, p=0.02). Thrombophilia, inherited and acquired, is an important contributing factor in unexplained RPL and should be screened in the order of its prevalence. Copyright © 2015 Elsevier Inc. All rights reserved.

Promoting Adaptive Behavior in Persons with Acquired Brain Injury, Extensive Motor and Communication Disabilities, and Consciousness Disorders

ERIC Educational Resources Information Center

Lancioni, Giulio E.; Singh, Nirbhay N.; O'Reilly, Mark F.; Sigafoos, Jeff; Belardinelli, Marta Olivetti; Buonocunto, Francesca; Sacco, Valentina; Navarro, Jorge; Lanzilotti, Crocifissa; De Tommaso, Marina; Megna, Marisa; Badagliacca, Francesco

2012-01-01

These two studies extended the evidence on the use of technology-based intervention packages to promote adaptive behavior in persons with acquired brain injury and multiple disabilities. Study I involved five participants in a minimally conscious state who were provided with intervention packages based on specific arrangements of optic, tilt, or…

Managing Severe Community-Acquired Pneumonia Due to Community Methicillin-Resistant Staphylococcus aureus (MRSA).

PubMed

Kwong, Jason C; Chua, Kyra; Charles, Patrick G P

2012-06-01

Community-associated methicillin-resistant Staphylococcus aureus (MRSA) is a rare, but significant cause of community-acquired pneumonia (CAP). A number of virulence determinants have been implicated in the development of severe community MRSA pneumonia, characterized by multilobar cavitating necrosis in patients without usual risk-factors for pneumonia. Optimal management is uncertain, and is extrapolated from anecdotal experiences with small case series, randomized studies of hospital-acquired pneumonia, and laboratory investigations using in vitro experiments and animal models of MRSA pneumonia. Adequate clinical suspicion, early diagnosis and administration of appropriate antibiotics are necessary for best patient outcomes, although some patients will still do badly even with early anti-MRSA therapy. Vancomycin or linezolid have been recommended as first-line therapy, possibly in combination with other antibiotics. Newer antibiotics such as ceftaroline are still being evaluated.

Beyond restoration to transformation: positive outcomes in the rehabilitation of acquired brain injury.

PubMed

McGrath, Joanna

2004-11-01

This paper compares the situation of the person with acquired brain injury to that of the people of Israel in the sixth century BCE (before the current era) during the period of exile in Babylon. Both situations are characterized by traumatic multiple losses, and a struggle to regain a sense of identity: personal, national or spiritual. Evidence from the literature on both brain injury rehabilitation and from the Hebrew Scriptures indicates that models of restoration of function and transformation of suffering have been applied to both situations. The relative strengths and weaknesses of these models are considered, and it is argued that models of transformation of suffering have much to offer, especially in the longer term psychotherapeutic rehabilitation of people with acquired brain injury, when restoration of function has reached its limits.

Intact implicit statistical learning in borderline personality disorder.

PubMed

Unoka, Zsolt; Vizin, Gabriella; Bjelik, Anna; Radics, Dóra; Nemeth, Dezso; Janacsek, Karolina

2017-09-01

Wide-spread neuropsychological deficits have been identified in borderline personality disorder (BPD). Previous research found impairments in decision making, declarative memory, working memory and executive functions; however, no studies have focused on implicit learning in BPD yet. The aim of our study was to investigate implicit statistical learning by comparing learning performance of 19 BPD patients and 19 healthy, age-, education- and gender-matched controls on a probabilistic sequence learning task. Moreover, we also tested whether participants retain the acquired knowledge after a delay period. To this end, participants were retested on a shorter version of the same task 24h after the learning phase. We found intact implicit statistical learning as well as retention of the acquired knowledge in this personality disorder. BPD patients seem to be able to extract and represent regularities implicitly, which is in line with the notion that implicit learning is less susceptible to illness compared to the more explicit processes. Copyright © 2017 Elsevier Ireland Ltd. All rights reserved.

Children Acquire Emotion Categories Gradually

ERIC Educational Resources Information Center

Widen, Sherri C.; Russell, James A.

2008-01-01

Some accounts imply that basic-level emotion categories are acquired early and quickly, whereas others imply that they are acquired later and more gradually. Our study examined this question for fear, happiness, sadness, and anger in the context of children's categorization of emotional facial expressions. Children (N=168, 2-5 years) first labeled…

Ensuring the profitability of acquired physician practices.

PubMed

Ortiz, J P

1997-01-01

Healthcare organizations are aggressively acquiring physician group practices to create primary care networks and broaden their managed care market penetration. However, few are realizing a positive return on investment after acquisition. The odds that acquired practices will be profitable can be improved if healthcare organizations plan carefully by establishing separate acquiring entities, setting clear goals for the practices, forming skilled management teams with strong physician leadership to manage the acquired practices, and carefully structuring their physician incentive compensation plans.

The efficacy of rituximab in the treatment of inhibitor-associated hemostatic disorders.

PubMed

Franchini, Massimo; Veneri, Dino; Lippi, Giuseppe; Stenner, Rachel

2006-08-01

Rituximab is a chimeric anti-CD20 monoclonal antibody active against normal and malignant B cells which has proven to be effective in the therapy of CD-20 positive lymphomas. Its B-cell cytotoxic action has also been exploited in many non-malignant autoimmune disorders in which it has been used with the aim of interfering with the production of pathologic antibodies. The present knowledge regarding the use of rituximab in antibody-associated disorders of hemostasis (i.e. idiopathic thrombocytopenic purpura, thrombotic thrombocytopenic purpura, acquired hemophilia A, congenital hemophilia with inhibitors, acquired inhibitors against coagulation factors) is presented briefly in this review. The results suggest that rituximab can be useful in the treatment of disorders of hemostasis associated with inhibitor formation. Although collectively the number of patients treated is now quite substantial, most of the data are drawn from isolated case reports or descriptions of small, uncontrolled series. Large, prospective, randomized trials are, therefore, needed to confirm the positive, preliminary results.

Bipolar disorders.

PubMed

Vieta, Eduard; Berk, Michael; Schulze, Thomas G; Carvalho, André F; Suppes, Trisha; Calabrese, Joseph R; Gao, Keming; Miskowiak, Kamilla W; Grande, Iria

2018-03-08

Bipolar disorders are chronic and recurrent disorders that affect >1% of the global population. Bipolar disorders are leading causes of disability in young people as they can lead to cognitive and functional impairment and increased mortality, particularly from suicide and cardiovascular disease. Psychiatric and nonpsychiatric medical comorbidities are common in patients and might also contribute to increased mortality. Bipolar disorders are some of the most heritable psychiatric disorders, although a model with gene-environment interactions is believed to best explain the aetiology. Early and accurate diagnosis is difficult in clinical practice as the onset of bipolar disorder is commonly characterized by nonspecific symptoms, mood lability or a depressive episode, which can be similar in presentation to unipolar depression. Moreover, patients and their families do not always understand the significance of their symptoms, especially with hypomanic or manic symptoms. As specific biomarkers for bipolar disorders are not yet available, careful clinical assessment remains the cornerstone of diagnosis. The detection of hypomanic symptoms and longtudinal clinical assessment are crucial to differentiate a bipolar disorder from other conditions. Optimal early treatment of patients with evidence-based medication (typically mood stabilizers and antipsychotics) and psychosocial strategies is necessary.

Cognitive and academic achievement changes associated with day hospital rehabilitation in children with acquired brain injury.

PubMed

Goldstein, Gerald; Mayfield, Joan; Thaler, Nicholas S; Walker, Jon; Allen, Daniel N

2018-01-01

An evaluation was made of the outcome of a day hospital rehabilitation program for children who experienced an acquired neurological illness, mainly traumatic brain injury. Participants were administered neuropsychological and academic evaluations upon entry to the program, immediately upon discharge and several months after discharge Repeated measures ANOVA results for variables selected from the Reynolds Intellectual Assessment and the Delis-Kaplan Executive Function System found that comparisons showed significant (≥p < .01) improvement occurred between the first and second assessment, generally with large effect sizes. There were some nonsignificant decrements in performance between the discharge and follow-up assessments. A correlational analysis showed that while the association between cognitive function and academic achievement was robust, correlation coefficients did not differ in strength before and following rehabilitation. The study demonstrates significant improvement in children with acquired neurological disorders following rehabilitation.

Aging Effects on Whole-Brain Functional Connectivity in Adults Free of Cognitive and Psychiatric Disorders.

PubMed

Ferreira, Luiz Kobuti; Regina, Ana Carolina Brocanello; Kovacevic, Natasa; Martin, Maria da Graça Morais; Santos, Pedro Paim; Carneiro, Camila de Godoi; Kerr, Daniel Shikanai; Amaro, Edson; McIntosh, Anthony Randal; Busatto, Geraldo F

2016-09-01

Aging is associated with decreased resting-state functional connectivity (RSFC) within the default mode network (DMN), but most functional imaging studies have restricted the analysis to specific brain regions or networks, a strategy not appropriate to describe system-wide changes. Moreover, few investigations have employed operational psychiatric interviewing procedures to select participants; this is an important limitation since mental disorders are prevalent and underdiagnosed and can be associated with RSFC abnormalities. In this study, resting-state fMRI was acquired from 59 adults free of cognitive and psychiatric disorders according to standardized criteria and based on extensive neuropsychological and clinical assessments. We tested for associations between age and whole-brain RSFC using Partial Least Squares, a multivariate technique. We found that normal aging is not only characterized by decreased RSFC within the DMN but also by ubiquitous increases in internetwork positive correlations and focal internetwork losses of anticorrelations (involving mainly connections between the DMN and the attentional networks). Our results reinforce the notion that the aging brain undergoes a dedifferentiation processes with loss of functional diversity. These findings advance the characterization of healthy aging effects on RSFC and highlight the importance of adopting a broad, system-wide perspective to analyze brain connectivity. © The Author 2015. Published by Oxford University Press. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com.

48 CFR 1845.502-70 - Contractor-acquired property.

Code of Federal Regulations, 2010 CFR

2010-10-01

... 48 Federal Acquisition Regulations System 6 2010-10-01 2010-10-01 true Contractor-acquired... Possession of Contractors 1845.502-70 Contractor-acquired property. All contractor-acquired property must be... contractor-acquired. (2) Submission of DD Form 1419, DOD Industrial Plant Requisition, or equivalent format...

Clinical assessment of decision-making capacity in acquired brain injury with personality change.

PubMed

Owen, Gareth S; Freyenhagen, Fabian; Martin, Wayne; David, Anthony S

2017-01-01

Assessment of decision-making capacity (DMC) can be difficult in acquired brain injury (ABI) particularly with the syndrome of organic personality disorder (OPD) (the "frontal lobe syndrome"). Clinical neuroscience may help but there are challenges translating its constructs to the decision-making abilities considered relevant by law and ethics. An in-depth interview study of DMC in OPD was undertaken. Six patients were purposefully sampled and rich interview data were acquired for scrutiny using interpretative phenomenological analysis. Interview data revealed that awareness of deficit and thinking about psychological states can be present. However, the awareness of deficit may not be "online" and effectively integrated into decision-making. Without this online awareness of deficit the ability to appreciate or use and weigh information in the process of deciding some matters appeared absent. We argue that the decision-making abilities discussed are: (1) necessary for DMC, (2) threatened by ABI , and (3) assessable at interview. Some advice for practically incorporating these abilities within assessments of DMC in patients with OPD is outlined.

[Minimal emotional dysfunction and first impression formation in personality disorders].

PubMed

Linden, M; Vilain, M

2011-01-01

"Minimal cerebral dysfunctions" are isolated impairments of basic mental functions, which are elements of complex functions like speech. The best described are cognitive dysfunctions such as reading and writing problems, dyscalculia, attention deficits, but also motor dysfunctions such as problems with articulation, hyperactivity or impulsivity. Personality disorders can be characterized by isolated emotional dysfunctions in relation to emotional adequacy, intensity and responsivity. For example, paranoid personality disorders can be characterized by continuous and inadequate distrust, as a disorder of emotional adequacy. Schizoid personality disorders can be characterized by low expressive emotionality, as a disorder of effect intensity, or dissocial personality disorders can be characterized by emotional non-responsivity. Minimal emotional dysfunctions cause interactional misunderstandings because of the psychology of "first impression formation". Studies have shown that in 100 ms persons build up complex and lasting emotional judgements about other persons. Therefore, minimal emotional dysfunctions result in interactional problems and adjustment disorders and in corresponding cognitive schemata.From the concept of minimal emotional dysfunctions specific psychotherapeutic interventions in respect to the patient-therapist relationship, the diagnostic process, the clarification of emotions and reality testing, and especially an understanding of personality disorders as impairment and "selection, optimization, and compensation" as a way of coping can be derived.

Mental disorders are not brain disorders.

PubMed

Banner, Natalie F

2013-06-01

As advances in neuroscience and genetics reveal complex associations between brain structures, functions and symptoms of mental disorders, there have been calls for psychiatric classifications to be reconfigured, to conceptualize mental disorders as disorders of the brain. In this paper, I argue that this view is mistaken, and that the level at which we identify mental disorders is, and should be, the person, not the brain. This is not to deny physicalism or argue that the mental realm is somehow distinct from the physical, but rather to suggest the things that are going 'wrong' in mental disorder are picked out at the person-level: they are characterized by breaches in epistemic, rational, evaluative, emotional, social and moral norms. However, as our scientific understanding of the brain becomes advanced, what makes an identified neurobiological difference in brain structure or functioning indicative of pathology is its association with these behaviours at the person-level. Instead of collapsing psychiatry into biomedicine, biomedicine may benefit from drawing closer to the expertise of psychiatry, as it is able to accommodate social, psychological and biological explanations while focusing on the person, within their environment. © 2013 John Wiley & Sons Ltd.

Acquired hepatocerebral degeneration: A case report

PubMed Central

Chen, Wei-Xing; Wang, Ping; Yan, Sen-Xiang; Li, You-Ming; Yu, Chao-Hui; Jiang, Ling-Ling

2005-01-01

AIM: Acquired hepatocerebral degeneration (AHD) is an exceptional type of hepatic encephalopathies (HE). It is characterized by neuropsychiatric and extrapyramidal symptomathology similar to that seen in hepatolenticular degeneration (Wilson’s disease). In this paper, we report a case of AHD with unusual presenting features. METHODS: A 28-year-old man with AHD was described and the literature was reviewed. RESULTS: The man had a history of HBV-related liver cirrhosis. He was admitted to our hospital with apathy, dysarthria, mild consciousness impairment and extrapyramidal symptoms after hematemesis. By review of the literature, cases with AHD often did not present consciousness impairment. So our case was once diagnosed incorrectly as Wilson’s disease. CONCLUSION: AHD is a rare syndrome and its variable clinical manifestations make it difficult to be diagnosed. But we believe that extensive examination and thorough understanding of the disease are beneficial to a correct diagnosis. Moreover, biocoene is effective in treating the case. PMID:15655841

Acquired perforating dermatosis: a report of 8 cases.

PubMed

González-Lara, L; Gómez-Bernal, S; Vázquez-López, F; Vivanco-Allende, B

2014-01-01

Acquired perforating dermatosis (APD) is an uncommon disease characterized by lesions exhibiting transepidermal elimination of collagen or elastic fibers. APD affects adults and is associated with systemic diseases, mainly diabetes mellitus and renal failure. We present 8 cases of APD. Seven patients had concomitant diabetes mellitus with or without chronic renal failure, and 1 had alcoholic cirrhosis. In the patients with chronic renal failure, the onset of APD coincided with transient worsening of renal function. The mean increase in creatinine concentrations above baseline was 1.14mg/dL. Acute deterioration of renal function may be involved in APD. Further studies are needed to investigate this association. Copyright © 2013 Elsevier España, S.L. y AEDV. All rights reserved.

Dysthymic disorder: forlorn and overlooked?

PubMed

Sansone, Randy A; Sansone, Lori A

2009-05-01

This ongoing column is dedicated to the challenging clinical interface between psychiatry and primary care-two fields that are inexorably linked.Dysthymic disorder is a smoldering mood disturbance characterized by a long duration (at least two years in adults) as well as transient periods of normal mood. The disorder is fairly common in the US general population (3-6%) as well as in primary care (7%) and mental health settings (up to one-third of psychiatric outpatients). While the etiology of dysthymia remains unknown, there appears to be a genetic susceptibility, which may manifest in the presence of various psychosocial stressors. While the Diagnostic and Statistical Manual of Mental Disorders diagnostic criteria are fairly clear, the disorder can be easily under-recognized for a variety of reasons. Treatment may include pharmacotherapy and psychotherapy, although the overall treatment course is oftentimes characterized by protracted symptoms and relapses.

Challenges and open issues in the management of acquired hemophilia A (AHA).

PubMed

Shetty, Shrimati D; Ghosh, Kanjaksha

2015-03-01

Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder caused by antibodies which neutralize the function of factor VIII (FVIII). The disease presents a complex clinical challenge to the treating Physicians and Hematologists. As the disease is associated with high mortality, prompt management is necessary. Early recognition, quick diagnosis and timely referral to a specialized center are important for better management of these patients. The different clinical manifestations, underlying pathology, inhibitor kinetics and the associated age related comorbidities do not allow extrapolation of the treatment protocols of congenital hemophilia to AHA. The basic strategies of the management of AHA patients involve maintaining hemostasis, suppression or eradication of antibodies, diagnosis and treatment of underlying pathology and avoid treatment related complications like thrombosis. The efficiency of hemostatic agents which are generally used to treat AHA is unpredictable. Due to the rarity of the disease, there are no randomized clinical trials on the management of this disorder and thus the expertise and experience of the treating Physicians' guide treatment strategies. Copyright © 2014 Elsevier Inc. All rights reserved.

Children with Anxiety Disorders: Use of a Cognitive Behavioral Therapy Model within a Social Milieu

ERIC Educational Resources Information Center

Kearny, Regina; Pawlukewicz, Justine; Guardino, Mary

2014-01-01

Because anxiety is the most common mental health disorder diagnosed in children, early intervention is crucial for fundamental coping. Although cognitive-behavioral therapy (CBT) is the preferred treatment method for this affective disorder, instruction for children needs to be specific for them to successfully acquire and implement essential CBT…

Diseases and disorders of muscle.

PubMed

Pearson, A M; Young, R B

1993-01-01

Muscle may suffer from a number of diseases or disorders, some being fatal to humans and animals. Their management or treatment depends on correct diagnosis. Although no single method may be used to identify all diseases, recognition depends on the following diagnostic procedures: (1) history and clinical examination, (2) blood biochemistry, (3) electromyography, (4) muscle biopsy, (5) nuclear magnetic resonance, (6) measurement of muscle cross-sectional area, (7) tests of muscle function, (8) provocation tests, and (9) studies on protein turnover. One or all of these procedures may prove helpful in diagnosis, but even then identification of the disorder may not be possible. Nevertheless, each of these procedures can provide useful information. Among the most common diseases in muscle are the muscular dystrophies, in which the newly identified muscle protein dystrophin is either absent or present at less than normal amounts in both Duchenne and Becker's muscular dystrophy. Although the identification of dystrophin represents a major breakthrough, treatment has not progressed to the experimental stage. Other major diseases of muscle include the inflammatory myopathies and neuropathies. Atrophy and hypertrophy of muscle and the relationship of aging, exercise, and fatigue all add to our understanding of the behavior of normal and abnormal muscle. Some other interesting related diseases and disorders of muscle include myasthenia gravis, muscular dysgenesis, and myclonus. Disorders of energy metabolism include those caused by abnormal glycolysis (Von Gierke's, Pompe's, Cori-Forbes, Andersen's, McArdle's, Hers', and Tauri's diseases) and by the acquired diseases of glycolysis (disorders of mitochondrial oxidation). Still other diseases associated with abnormal energy metabolism include lipid-related disorders (carnitine and carnitine palmitoyl-transferase deficiencies) and myotonic syndromes (myotonia congenita, paramyotonia congenita, hypokalemic and hyperkalemic

Combining neuropsychological and cognitive-behavioral approaches for treating psychological sequelae of acquired brain injury.

PubMed

Doering, Bettina; Exner, Cornelia

2011-03-01

Acquired brain injury (ABI) does not only result in physical and cognitive impairments, but may also entail behavioral-emotional difficulties and mental disorders. Although neuropsychological approaches target the rehabilitation of cognitive deficits, the treatment of emotional and behavioral sequelae has received less consideration. This review argues for the integration of cognitive-behavioral approaches into the rehabilitation process and examines respective recent research. Cognitive-behavioral interventions have been investigated in the treatment of behavioral disturbances and mental disorders after ABI. They have also been targeted at supporting adaptive coping with chronic injury-related impairments. Problem-solving approaches of cognitive behavioral therapy may work as meta-models or framework for the rehabilitative process. Unfortunately, most studies reviewed employed methodologically weak designs, which limit convincing conclusions. Still, positive intervention effects have been demonstrated concerning specific outcome measures. Whether these changes also translate into increased psychosocial functioning or quality of life remains unclear. Methodologically sound evidence for cognitive-behavioral interventions after ABI is limited, but preliminary results support the effectiveness of these interventions in the treatment of behavioral disorders and emotional disturbances after ABI. Integrating neuropsychological and cognitive-behavioral approaches may therefore prove beneficial to the rehabilitation process.

Integral Characterization of Defective BDNF/TrkB Signalling in Neurological and Psychiatric Disorders Leads the Way to New Therapies

PubMed Central

Tejeda, Gonzalo S.; Díaz-Guerra, Margarita

2017-01-01

Enhancement of brain-derived neurotrophic factor (BDNF) signalling has great potential in therapy for neurological and psychiatric disorders. This neurotrophin not only attenuates cell death but also promotes neuronal plasticity and function. However, an important challenge to this approach is the persistence of aberrant neurotrophic signalling due to a defective function of the BDNF high-affinity receptor, tropomyosin-related kinase B (TrkB), or downstream effectors. Such changes have been already described in several disorders, but their importance as pathological mechanisms has been frequently underestimated. This review highlights the relevance of an integrative characterization of aberrant BDNF/TrkB pathways for the rational design of therapies that by combining BDNF and TrkB targets could efficiently promote neurotrophic signalling. PMID:28134845

Mood disorders in eating disorder patients: Prevalence and chronology of ONSET.

PubMed

Godart, N; Radon, L; Curt, F; Duclos, J; Perdereau, F; Lang, F; Venisse, J L; Halfon, O; Bizouard, P; Loas, G; Corcos, M; Jeammet, Ph; Flament, M F

2015-10-01

In a clinical population, we estimated the frequency of mood disorders among 271 patients suffering from Anorexia Nervosa (AN) and Bulimia Nervosa (BN) in comparison to a control group matched for age and gender. The frequency of mood disorders was measured using the Mini International Neuropsychiatric Interview (MINI), DSM-IV version. Mood disorders were more frequent among eating disorder (ED) patients than among controls, with a global prevalence of the order of 80% for each ED group. The majority of the mood disorders comorbid with ED were depressive disorders (MDD and dysthymia). The relative chronology of onset of these disorders was equivocal, because mood disorders in some cases preceded and in others followed the onset of the eating disorders. Our sample was characterized by patients with severe ED and high comorbidities, and thus do not represent the entire population of AN or BN. This also may have resulted in an overestimation of prevalence. Mood disorders appear significantly more frequently in patients seeking care for ED than in controls. These results have implications for the assessment and treatment of ED patients, and for the aetio-pathogenesis of these disorders. Copyright © 2015 Elsevier B.V. All rights reserved.

Brain structure-function associations in multi-generational families genetically enriched for bipolar disorder.

PubMed

Fears, Scott C; Schür, Remmelt; Sjouwerman, Rachel; Service, Susan K; Araya, Carmen; Araya, Xinia; Bejarano, Julio; Knowles, Emma; Gomez-Makhinson, Juliana; Lopez, Maria C; Aldana, Ileana; Teshiba, Terri M; Abaryan, Zvart; Al-Sharif, Noor B; Navarro, Linda; Tishler, Todd A; Altshuler, Lori; Bartzokis, George; Escobar, Javier I; Glahn, David C; Thompson, Paul M; Lopez-Jaramillo, Carlos; Macaya, Gabriel; Molina, Julio; Reus, Victor I; Sabatti, Chiara; Cantor, Rita M; Freimer, Nelson B; Bearden, Carrie E

2015-07-01

Recent theories regarding the pathophysiology of bipolar disorder suggest contributions of both neurodevelopmental and neurodegenerative processes. While structural neuroimaging studies indicate disease-associated neuroanatomical alterations, the behavioural correlates of these alterations have not been well characterized. Here, we investigated multi-generational families genetically enriched for bipolar disorder to: (i) characterize neurobehavioural correlates of neuroanatomical measures implicated in the pathophysiology of bipolar disorder; (ii) identify brain-behaviour associations that differ between diagnostic groups; (iii) identify neurocognitive traits that show evidence of accelerated ageing specifically in subjects with bipolar disorder; and (iv) identify brain-behaviour correlations that differ across the age span. Structural neuroimages and multi-dimensional assessments of temperament and neurocognition were acquired from 527 (153 bipolar disorder and 374 non-bipolar disorder) adults aged 18-87 years in 26 families with heavy genetic loading for bipolar disorder. We used linear regression models to identify significant brain-behaviour associations and test whether brain-behaviour relationships differed: (i) between diagnostic groups; and (ii) as a function of age. We found that total cortical and ventricular volume had the greatest number of significant behavioural associations, and included correlations with measures from multiple cognitive domains, particularly declarative and working memory and executive function. Cortical thickness measures, in contrast, showed more specific associations with declarative memory, letter fluency and processing speed tasks. While the majority of brain-behaviour relationships were similar across diagnostic groups, increased cortical thickness in ventrolateral prefrontal and parietal cortical regions was associated with better declarative memory only in bipolar disorder subjects, and not in non-bipolar disorder family

Aligned nanofibrillar collagen scaffolds - Guiding lymphangiogenesis for treatment of acquired lymphedema.

PubMed

Hadamitzky, Catarina; Zaitseva, Tatiana S; Bazalova-Carter, Magdalena; Paukshto, Michael V; Hou, Luqia; Strassberg, Zachary; Ferguson, James; Matsuura, Yuka; Dash, Rajesh; Yang, Phillip C; Kretchetov, Shura; Vogt, Peter M; Rockson, Stanley G; Cooke, John P; Huang, Ngan F

2016-09-01

Secondary lymphedema is a common disorder associated with acquired functional impairment of the lymphatic system. The goal of this study was to evaluate the therapeutic efficacy of aligned nanofibrillar collagen scaffolds (BioBridge) positioned across the area of lymphatic obstruction in guiding lymphatic regeneration. In a porcine model of acquired lymphedema, animals were treated with BioBridge scaffolds, alone or in conjunction with autologous lymph node transfer as a source of endogenous lymphatic growth factor. They were compared with a surgical control group and a second control group in which the implanted BioBridge was supplemented with exogenous vascular endothelial growth factor-C (VEGF-C). Three months after implantation, immunofluorescence staining of lymphatic vessels demonstrated a significant increase in lymphatic collectors within close proximity to the scaffolds. To quantify the functional impact of scaffold implantation, bioimpedance was used as an early indicator of extracellular fluid accumulation. In comparison to the levels prior to implantation, the bioimpedance ratio was significantly improved only in the experimental BioBridge recipients with or without lymph node transfer, suggesting restoration of functional lymphatic drainage. These results further correlated with quantifiable lymphatic collectors, as visualized by contrast-enhanced computed tomography. They demonstrate the therapeutic potential of BioBridge scaffolds in secondary lymphedema. Copyright © 2016 Elsevier Ltd. All rights reserved.

Acquired Bilateral Longitudinal True Leukonychia in a 35-year-old Woman

PubMed Central

Mokhtari, Fatemeh; Mozafarpoor, Samaneh; Nouraei, Saeid; Nilforoushzadeh, Mohammad Ali

2016-01-01

Acquired bilateral longitudinal true leukonychia is a rare disorder. We present a case of a 35-year-old healthy woman presented with this unusual and rare manifestation. She mentioned a history of unprotected exposure to detergents and bleaching chemical agents. Considering her low zinc level, she was prescribed with zinc capsules and recommended to avoid chemical substances for 6 months. During bimonthly follow-up, her zinc level turned normal, and leukonychia subsequently disappeared. Bilateral longitudinal true leukonychia in the nails due to zinc deficiency and exposure to chemical substances has not been reported previously. Direct and indirect effects of chemical substances on matrix and the effect of zinc deficiency on healing process should be considered in these cases. PMID:27857831

Children with Comorbid Speech Sound Disorder and Specific Language Impairment Are at Increased Risk for Attention-Deficit/Hyperactivity Disorder

ERIC Educational Resources Information Center

McGrath, Lauren M.; Hutaff-Lee, Christa; Scott, Ashley; Boada, Richard; Shriberg, Lawrence D.; Pennington, Bruce F.

2008-01-01

This study focuses on the comorbidity between attention-deficit/hyperactivity disorder (ADHD) symptoms and speech sound disorder (SSD). SSD is a developmental disorder characterized by speech production errors that impact intelligibility. Previous research addressing this comorbidity has typically used heterogeneous groups of speech-language…

Longitudinal Study of Driver Licensing Rates among Adolescents and Young Adults with Autism Spectrum Disorder

ERIC Educational Resources Information Center

Curry, Allison E.; Yerys, Benjamin E.; Huang, Patty; Metzger, Kristi B.

2018-01-01

Driving may increase mobility and independence for adolescents with autism without intellectual disability (autism spectrum disorder); however, little is known about rates of licensure. To compare the proportion of adolescents with and without autism spectrum disorder who acquire a learner's permit and driver's license, as well as the rate at…

Acquired resistance mechanisms to tyrosine kinase inhibitors in lung cancer with activating epidermal growth factor receptor mutation--diversity, ductility, and destiny.

PubMed

Suda, Kenichi; Mizuuchi, Hiroshi; Maehara, Yoshihiko; Mitsudomi, Tetsuya

2012-12-01

Lung cancers that harbor somatic activating mutations in the gene for the epidermal growth factor receptor (EGFR) depend on mutant EGFR for their proliferation and survival; therefore, lung cancer patients with EGFR mutations often dramatically respond to orally available EGFR tyrosine kinase inhibitors (TKIs). However, emergence of acquired resistance is virtually inevitable, thus limiting improvement in patient outcomes. To elucidate and overcome this acquired resistance, multidisciplinary basic and clinical investigational approaches have been applied, using in vitro cell line models or samples obtained from lung cancer patients treated with EGFR-TKIs. These efforts have revealed several acquired resistance mechanisms and candidates, including EGFR secondary mutations (T790M and other rare mutations), MET amplification, PTEN downregulation, CRKL amplification, high-level HGF expression, FAS-NFκB pathway activation, epithelial-mesenchymal transition, and conversion to small cell lung cancer. Interestingly, cancer cells harbor potential destiny and ductility together in acquiring resistance to EGFR-TKIs, as shown in in vitro acquired resistance models. Molecular mechanisms of "reversible EGFR-TKI tolerance" that occur in early phase EGFR-TKI exposure have been identified in cell line models. Furthermore, others have reported molecular markers that can predict response to EGFR-TKIs in clinical settings. Deeper understanding of acquired resistance mechanisms to EGFR-TKIs, followed by the development of molecular target drugs that can overcome the resistance, might turn this fatal disease into a chronic disorder.

Neural Mechanisms of Emotion Regulation in Autism Spectrum Disorder

ERIC Educational Resources Information Center

Richey, J. Anthony; Damiano, Cara R.; Sabatino, Antoinette; Rittenberg, Alison; Petty, Chris; Bizzell, Josh; Voyvodic, James; Heller, Aaron S.; Coffman, Marika C.; Smoski, Moria; Davidson, Richard J.; Dichter, Gabriel S.

2015-01-01

Autism spectrum disorder (ASD) is characterized by high rates of comorbid internalizing and externalizing disorders. One mechanistic account of these comorbidities is that ASD is characterized by impaired emotion regulation (ER) that results in deficits modulating emotional responses. We assessed neural activation during cognitive reappraisal of…

Monitoring of anesthetic depth during surgical correction of acquired valvular disorders: single center, randomized trial.

PubMed

Lenkin, Andrey I; Zaharov, Viktor I; Lenkin, Pavel I; Smetkin, Alexey A; Bjertnaes, Lars J; Kirov, Mikhail Y

2014-04-01

The authors' primary objective was to test the hypothesis that Cerebral State Index (CSI)-guided control of anesthetic depth might reduce the consumption of anesthetics and shorten the duration of ICU and hospital stays after surgical correction of combined valve disorders. Single center, randomized trial. City Hospital Number 1 of Arkhangelsk, Russian Federation. Fifty adult patients with combined valve disorders requiring surgical correction. The patients were randomized into 2 groups. In the CSI group, anesthetic depth was monitored, and the rate of infusion of propofol was titrated to maintain the depth of anesthesia corresponding to a CSI of 40-60. In the control group, the depth of anesthesia was monitored clinically, and the dosage of propofol was administered according to the recommendations of the manufacturer. All patients received standard perioperative monitoring. Consumption of anesthetics and length of ICU and hospital stays were recorded. Preoperative patient characteristics did not differ significantly between the groups. In the CSI group, average intraoperative doses of midazolam and propofol were reduced by 41% and 19%, respectively (p<0.01). Maintenance of anesthesia guided by CSI shortened the time until fit for ICU discharge by 50% and reduced the lengths of ICU and postoperative hospital stays by 35% and 25%, respectively (p< 0.05). Monitoring of anesthetic depth reduces the requirements for midazolam and propofol, resulting in a faster recovery and a shorter postoperative ICU and hospital stay after surgical correction of combined valve disorders. Copyright © 2014 Elsevier Inc. All rights reserved.

Fanconi Anemia: A DNA repair disorder characterized by accelerated decline of the hematopoietic stem cell compartment and other features of aging.

PubMed

Brosh, Robert M; Bellani, Marina; Liu, Yie; Seidman, Michael M

2017-01-01

Fanconi Anemia (FA) is a rare autosomal genetic disorder characterized by progressive bone marrow failure (BMF), endocrine dysfunction, cancer, and other clinical features commonly associated with normal aging. The anemia stems directly from an accelerated decline of the hematopoietic stem cell compartment. Although FA is a complex heterogeneous disease linked to mutations in 19 currently identified genes, there has been much progress in understanding the molecular pathology involved. FA is broadly considered a DNA repair disorder and the FA gene products, together with other DNA repair factors, have been implicated in interstrand cross-link (ICL) repair. However, in addition to the defective DNA damage response, altered epigenetic regulation, and telomere defects, FA is also marked by elevated levels of inflammatory mediators in circulation, a hallmark of faster decline in not only other hereditary aging disorders but also normal aging. In this review, we offer a perspective of FA as a monogenic accelerated aging disorder, citing the latest evidence for its multi-factorial deficiencies underlying its unique clinical and cellular features. Published by Elsevier B.V.

Beyond Utterances: Distributed Cognition as a Framework for Studying Discourse in Adults with Acquired Brain Injury

PubMed Central

Duff, Melissa C.; Mutlu, Bilge; Byom, Lindsey; Turkstra, Lyn S.

2014-01-01

Considerable effort has been directed at understanding the nature of the communicative deficits observed in individuals with acquired brain injuries. Yet several theoretical, methodological, and clinical challenges remain. In this article, we examine distributed cognition as a framework for understanding interaction among communication partners, interaction of communication and cognition, and interaction with the environments and contexts of everyday language use. We review the basic principles of distributed cognition and the implications for applying this approach to the study of discourse in individuals with cognitive-communication disorders. We also review a range of protocols and findings from our research that highlight how the distributed cognition approach might offer a deeper understanding of communicative mechanisms and deficits in individuals with cognitive communication impairments. The advantages and implications of distributed cognition as a framework for studying discourse in adults with acquired brain injury are discussed. PMID:22362323

Reduced Gray Matter Volume in the Social Brain Network in Adults with Autism Spectrum Disorder

PubMed Central

Sato, Wataru; Kochiyama, Takanori; Uono, Shota; Yoshimura, Sayaka; Kubota, Yasutaka; Sawada, Reiko; Sakihama, Morimitsu; Toichi, Motomi

2017-01-01

Autism spectrum disorder (ASD) is a neurodevelopmental disorder characterized by behavioral impairment in social interactions. Although theoretical and empirical evidence suggests that impairment in the social brain network could be the neural underpinnings of ASD, previous structural magnetic resonance imaging (MRI) studies in adults with ASD have not provided clear support for this, possibly due to confounding factors, such as language impairments. To further explore this issue, we acquired structural MRI data and analyzed gray matter volume in adults with ASD (n = 36) who had no language impairments (diagnosed with Asperger’s disorder or pervasive developmental disorder not otherwise specified, with symptoms milder than those of Asperger’s disorder), had no comorbidity, and were not taking medications, and in age- and sex-matched typically developing (TD) controls (n = 36). Univariate voxel-based morphometry analyses revealed that regional gray matter volume was lower in the ASD than in the control group in several brain regions, including the right inferior occipital gyrus, left fusiform gyrus, right middle temporal gyrus, bilateral amygdala, right inferior frontal gyrus, right orbitofrontal cortex, and left dorsomedial prefrontal cortex. A multivariate approach using a partial least squares (PLS) method showed that these regions constituted a network that could be used to discriminate between the ASD and TD groups. A PLS discriminant analysis using information from these regions showed high accuracy, sensitivity, specificity, and precision (>80%) in discriminating between the groups. These results suggest that reduced gray matter volume in the social brain network represents the neural underpinnings of behavioral social malfunctioning in adults with ASD. PMID:28824399

Melanoma patient derived xenografts acquire distinct Vemurafenib resistance mechanisms

PubMed Central

Monsma, David J; Cherba, David M; Eugster, Emily E; Dylewski, Dawna L; Davidson, Paula T; Peterson, Chelsea A; Borgman, Andrew S; Winn, Mary E; Dykema, Karl J; Webb, Craig P; MacKeigan, Jeffrey P; Duesbery, Nicholas S; Nickoloff, Brian J; Monks, Noel R

2015-01-01

Variable clinical responses, tumor heterogeneity, and drug resistance reduce long-term survival outcomes for metastatic melanoma patients. To guide and accelerate drug development, we characterized tumor responses for five melanoma patient derived xenograft models treated with Vemurafenib. Three BRAFV600E models showed acquired drug resistance, one BRAFV600E model had a complete and durable response, and a BRAFV600V model was expectedly unresponsive. In progressing tumors, a variety of resistance mechanisms to BRAF inhibition were uncovered, including mutant BRAF alternative splicing, NRAS mutation, COT (MAP3K8) overexpression, and increased mutant BRAF gene amplification and copy number. The resistance mechanisms among the patient derived xenograft models were similar to the resistance pathways identified in clinical specimens from patients progressing on BRAF inhibitor therapy. In addition, there was both inter- and intra-patient heterogeneity in resistance mechanisms, accompanied by heterogeneous pERK expression immunostaining profiles. MEK monotherapy of Vemurafenib-resistant tumors caused toxicity and acquired drug resistance. However, tumors were eradicated when Vemurafenib was combined the MEK inhibitor. The diversity of drug responses among the xenograft models; the distinct mechanisms of resistance; and the ability to overcome resistance by the addition of a MEK inhibitor provide a scheduling rationale for clinical trials of next-generation drug combinations. PMID:26101714

Recombinant Intrinsically Disordered Proteins for NMR: Tips and Tricks.

PubMed

Calçada, Eduardo O; Korsak, Magdalena; Kozyreva, Tatiana

2015-01-01

The growing recognition of the several roles that intrinsically disordered proteins play in biology places an increasing importance on protein sample availability to allow the characterization of their structural and dynamic properties. The sample preparation is therefore the limiting step to allow any biophysical method being able to characterize the properties of an intrinsically disordered protein and to clarify the links between these properties and the associated biological functions. An increasing array of tools has been recruited to help prepare and characterize the structural and dynamic properties of disordered proteins. This chapter describes their sample preparation, covering the most common drawbacks/barriers usually found working in the laboratory bench. We want this chapter to be the bedside book of any scientist interested in preparing intrinsically disordered protein samples for further biophysical analysis.

Does metacognitive strategy instruction improve impaired receptive cognitive-communication skills following acquired brain injury?

PubMed

Copley, Anna; Smith, Kathleen; Savill, Katelyn; Finch, Emma

2015-01-01

To investigate if metacognitive strategy instruction (MSI) improves the receptive language skills of adults with cognitive-communication disorders secondary to acquired brain injury (ABI). An ABA intervention programme was implemented with eight adults with ABI, aged 25-70 years. The Measure of Cognitive-Linguistic Abilities (MCLA) was administered at baseline and following treatment. The treatment employed in this study involved three components: individual goal-based therapy, group remediation therapy using self-instruction and home practice. No receptive language sub-tests of the MCLA reached statistical significance. However, participants' raw score improvements in receptive language sub-tests indicated that MSI may be effective at remediating CCDs following ABI. Preliminary findings indicate that MSI may be effective in improving receptive language skills in adults with CCDs following ABI. Further research involving a more rigorous study, a larger sample size and a more reliable outcome measure is necessary and may provide statistically significant evidence for the effectiveness of MSI for remediating receptive language disorders.

Impaired holistic processing of unfamiliar individual faces in acquired prosopagnosia.

PubMed

Ramon, Meike; Busigny, Thomas; Rossion, Bruno

2010-03-01

Prosopagnosia is an impairment at individualizing faces that classically follows brain damage. Several studies have reported observations supporting an impairment of holistic/configural face processing in acquired prosopagnosia. However, this issue may require more compelling evidence as the cases reported were generally patients suffering from integrative visual agnosia, and the sensitivity of the paradigms used to measure holistic/configural face processing in normal individuals remains unclear. Here we tested a well-characterized case of acquired prosopagnosia (PS) with no object recognition impairment, in five behavioral experiments (whole/part and composite face paradigms with unfamiliar faces). In all experiments, for normal observers we found that processing of a given facial feature was affected by the location and identity of the other features in a whole face configuration. In contrast, the patient's results over these experiments indicate that she encodes local facial information independently of the other features embedded in the whole facial context. These observations and a survey of the literature indicate that abnormal holistic processing of the individual face may be a characteristic hallmark of prosopagnosia following brain damage, perhaps with various degrees of severity. Copyright (c) 2009 Elsevier Ltd. All rights reserved.

How to describe disordered structures

NASA Astrophysics Data System (ADS)

Nishio, Kengo; Miyazaki, Takehide

2016-04-01

Disordered structures such as liquids and glasses, grains and foams, galaxies, etc. are often represented as polyhedral tilings. Characterizing the associated polyhedral tiling is a promising strategy to understand the disordered structure. However, since a variety of polyhedra are arranged in complex ways, it is challenging to describe what polyhedra are tiled in what way. Here, to solve this problem, we create the theory of how the polyhedra are tiled. We first formulate an algorithm to convert a polyhedron into a codeword that instructs how to construct the polyhedron from its building-block polygons. By generalizing the method to polyhedral tilings, we describe the arrangements of polyhedra. Our theory allows us to characterize polyhedral tilings, and thereby paves the way to study from short- to long-range order of disordered structures in a systematic way.

Characterization of a novel chicken muscle disorder through differential gene expression and pathway analysis using RNA-sequencing.

PubMed

Mutryn, Marie F; Brannick, Erin M; Fu, Weixuan; Lee, William R; Abasht, Behnam

2015-05-21

Improvements in poultry production within the past 50 years have led to increased muscle yield and growth rate, which may be contributing to an increased rate and development of new muscle disorders in chickens. Previously reported muscle disorders and conditions are generally associated with poor meat quality traits and have a significant negative economic impact on the poultry industry. Recently, a novel myopathy phenotype has emerged which is characterized by palpably "hard" or tough breast muscle. The objective of this study is to identify the underlying biological mechanisms that contribute to this emerging muscle disorder colloquially referred to as "Wooden Breast", through the use of RNA-sequencing technology. We constructed cDNA libraries from five affected and six unaffected breast muscle samples from a line of commercial broiler chickens. After paired-end sequencing of samples using the Illumina Hiseq platform, we used Tophat to align the resulting sequence reads to the chicken reference genome and then used Cufflinks to find significant changes in gene transcript expression between each group. By comparing our gene list to previously published histology findings on this disorder and using Ingenuity Pathways Analysis (IPA®), we aim to develop a characteristic gene expression profile for this novel disorder through analyzing genes, gene families, and predicted biological pathways. Over 1500 genes were differentially expressed between affected and unaffected birds. There was an average of approximately 98 million reads per sample, across all samples. Results from the IPA analysis suggested "Diseases and Disorders" such as connective tissue disorders, "Molecular and Cellular Functions" such as cellular assembly and organization, cellular function and maintenance, and cellular movement, "Physiological System Development and Function" such as tissue development, and embryonic development, and "Top Canonical Pathways" such as, coagulation system, axonal

The Atlanta Motor Speech Disorders Corpus: Motivation, Development, and Utility.

PubMed

Laures-Gore, Jacqueline; Russell, Scott; Patel, Rupal; Frankel, Michael

2016-01-01

This paper describes the design and collection of a comprehensive spoken language dataset from speakers with motor speech disorders in Atlanta, Ga., USA. This collaborative project aimed to gather a spoken database consisting of nonmainstream American English speakers residing in the Southeastern US in order to provide a more diverse perspective of motor speech disorders. Ninety-nine adults with an acquired neurogenic disorder resulting in a motor speech disorder were recruited. Stimuli include isolated vowels, single words, sentences with contrastive focus, sentences with emotional content and prosody, sentences with acoustic and perceptual sensitivity to motor speech disorders, as well as 'The Caterpillar' and 'The Grandfather' passages. Utility of this data in understanding the potential interplay of dialect and dysarthria was demonstrated with a subset of the speech samples existing in the database. The Atlanta Motor Speech Disorders Corpus will enrich our understanding of motor speech disorders through the examination of speech from a diverse group of speakers. © 2016 S. Karger AG, Basel.

Large Multispectral and Albedo Panoramas Acquired by the Pancam Instruments on the Mars Exploration Rovers Spirit and Opportunity

NASA Technical Reports Server (NTRS)

Bell, J. F., III; Arneson, H. M.; Farrand, W. H.; Goetz, W.; Hayes, A. G.; Herkenhoff, K.; Johnson, M. J.; Johnson, J. R.; Joseph, J.; Kinch, K.

2005-01-01

Introduction. The panoramic camera (Pancam) multispectral, stereoscopic imaging systems on the Mars Exploration Rovers Spirit and Opportunity [1] have acquired and downlinked more than 45,000 images (35 Gbits of data) over more than 700 combined sols of operation on Mars as of early January 2005. A large subset of these images were acquired as part of 26 large multispectral and/or broadband "albedo" panoramas (15 on Spirit, 11 on Opportunity) covering large ranges of azimuth (12 spanning 360 ) and designed to characterize major regional color and albedo characteristics of the landing sites and various points along both rover traverses.

Protective Effects of Psychological Strengths Against Psychiatric Disorders Among Soldiers.

PubMed

Shrestha, Alice; Cornum, B G Rhonda; Vie, Loryana L; Scheier, Lawrence M; Lester, M A J Paul B; Seligman, Martin E P

2018-03-01

This study prospectively examined psychological strengths targeted in U.S. Army training programs as predictors of psychiatric diagnosis in active duty soldiers. At baseline, the cohort (140,584 soldiers) was without psychiatric disorder. Soldiers were then followed for 2 yr and classified as healthy, or acquiring a psychiatric diagnosis (adjustment disorder, anxiety disorder, depression, or post-traumatic stress disorder), or being prescribed psychotropic medication without a psychiatric diagnosis. Soldiers who remained healthy reported significantly higher strengths scores at baseline, compared with soldiers who were diagnosed with a psychiatric disorder. In addition, soldiers in the worst strengths decile were twice as likely to develop a psychiatric disorder, compared with soldiers in the top 50% on baseline strengths. Strengths afforded the greatest protection against depression. Offering tailored resilience training programs could help the Army steel vulnerable soldiers against the challenges of life, military training, and combat.

Addictive Disorders in Adolescents.

PubMed

Truong, Anh; Moukaddam, Nidal; Toledo, Alexander; Onigu-Otite, Edore

2017-09-01

Addictive disorders in youth represent a dynamic field characterized by shifting patterns of substance use and high rates of experimentation, while retaining the risky behaviors and negative outcomes associated with established drug classes. Youth/adolescents are also at the forefront of use of new technologies, and non-substance-related disorders are pertinent. These disorders present with similar pictures of impairment, and can be diagnosed following the same principles. An underlying mental disorder and the possibility of a dual diagnosis need to be assessed carefully, and optimal treatment includes psychosocial treatments with applicable pharmacologic management, the latter representing an expanding field. Copyright © 2017 Elsevier Inc. All rights reserved.

Multiscale roughness characterization from multiresolution remote sensing data acquired over the Asal-Ghoubbet rift, Republic of Djibouti

NASA Astrophysics Data System (ADS)

Labarre, Sébastien; Jacquemoud, Stéphane; Ferrari, Cécile; Delorme, Arthur; Rupnik, Ewelina; Derrien, Allan; Pierrot-Deseilligny, Marc; Grandin, Raphaël; Jalludin, Mohamed

2017-04-01

parameter that depicts well the ground truth, we applied two multiscale methods: fractal analysis and wavelet transform. The latter allows splitting the frequency band of a signal in several sub-bands, each of which corresponding to a spatial scale. By analyzing data acquired at Piton de la Fournaise Volcano, Réunion island, we showed that wavelet transform is a very powerful tool for characterizing roughness regimes over scales and that sub-centimeter surface features mostly explain the integrated roughness for meter-sized surfaces (Labarre et al., 2017, Icarus). This has to be confirmed on Djibouti terrains, for which we have a broader range of resolutions and larger areas.

Patterns of co-occurring addictions, posttraumatic stress disorder, and major depressive disorder in detoxification treatment seekers: Implications for improving detoxification treatment outcomes.

PubMed

Anderson, RaeAnn E; Hruska, Bryce; Boros, Alec P; Richardson, Christopher J; Delahanty, Douglas L

2018-03-01

Poly-substance use and psychiatric comorbidity are common among individuals receiving substance detoxification services. Posttraumatic stress disorder (PTSD) and major depressive disorder (MDD) are the most common co-occurring psychiatric disorders with substance use disorder (SUD). Current treatment favors a one-size-fits-all approach to treating addiction focusing on one substance or one comorbidity. Research examining patterns of substance use and comorbidities can inform efforts to effectively identify and differentially treat individuals with co-occurring conditions. Using latent class analysis, the current study identified four patterns of PTSD, MDD, and substance use among 375 addiction treatment seekers receiving medically supervised detoxification. The four identified classes were: 1) a PTSD-MDD-Poly SUD class characterized by PTSD and MDD occurring in the context of opioid, cannabis, and tobacco use disorders; 2) an MDD-Poly SUD class characterized by MDD and alcohol, opioid, tobacco, and cannabis use disorders; 3) an alcohol-tobacco class characterized by alcohol and tobacco use disorders; and 4) an opioid-tobacco use disorder class characterized by opioid and tobacco use disorders. The observed classes differed on gender and clinical characteristics including addiction severity, trauma history, and PTSD/MDD symptom severity. The observed classes likely require differing treatment approaches. For example, people in the PTSD-MDD-Poly SUD class would likely benefit from treatment approaches targeting anxiety sensitivity and distress tolerance, while the opioid-tobacco class would benefit from treatments that incorporate motivational interviewing. Appropriate matching of treatment to class could optimize treatment outcomes for polysubstance and comorbid psychiatric treatment seekers. These findings also underscore the importance of well-developed referral networks to optimize outpatient psychotherapy for detoxification treatment-seekers to enhance long

B-cell development and pneumococcal immunity in vertically acquired HIV infection.

PubMed

Eisen, Sarah; Hayden, Clare; Young, Carmel J; Gilson, Richard; Jungmann, Eva; Jacobsen, Marianne C; Poulsom, Hannah; Goldblatt, David; Klein, Nigel J; Baxendale, Helen E

2016-07-31

Many children with HIV infection now survive into adulthood. This study explored the impact of vertically acquired HIV in the era of antiretroviral therapy on the development of humoral immunity. Natural and vaccine-related immunity to pneumococcus and B-cell phenotype was characterized and compared in three groups of young adults: those with vertically-acquired infection, those with horizontally acquired infection and healthy controls. Serotype-specific pneumococcal (Pnc) immunoglobulin M and G concentrations before and up to 1 year post-Pnc polysaccharide (Pneumovax) immunization were determined, and opsonophagocytic activity was analysed. B-cell subpopulations and dynamic markers of B-cell signalling, turnover and susceptibility to apoptosis were evaluated by flow cytometry. HIV-infected patients showed impaired natural Pnc immunity and reduced humoral responses to immunization with Pneumovax; this was greatest in those viraemic at time of the study. Early-life viral control before the age of 10 years diminished these changes. Expanded populations of abnormally activated and immature B-cells were seen in both HIV-infected cohorts. Vertically infected patients were particularly vulnerable to reductions in marginal zone and switched memory populations. These aberrations were reduced in patients with early-life viral control. In children with HIV, damage to B-cell memory populations and impaired natural and vaccine immunity to pneumococcus is evident in early adult life. Sustained viral control from early childhood may help to limit this effect and optimize humoral immunity in adult life.

Neurocutaneous Disorders.

PubMed

Rosser, Tena

2018-02-01

This article presents an up-to-date summary of the genetic etiology, diagnostic criteria, clinical features, and current management recommendations for the most common neurocutaneous disorders encountered in clinical adult and pediatric neurology practices. The phakomatoses are a phenotypically and genetically diverse group of multisystem disorders that primarily affect the skin and central nervous system. A greater understanding of the genetic and biological underpinnings of numerous neurocutaneous disorders has led to better clinical characterization, more refined diagnostic criteria, and improved treatments in neurofibromatosis type 1, Legius syndrome, neurofibromatosis type 2, Noonan syndrome with multiple lentigines, tuberous sclerosis complex, Sturge-Weber syndrome, and incontinentia pigmenti. Neurologists require a basic knowledge of and familiarity with a wide variety of neurocutaneous disorders because of the frequent involvement of the central and peripheral nervous systems. A simple routine skin examination can often open a broad differential diagnosis and lead to improved patient care.

Differential Diagnosis of Speech Sound Disorder (Phonological Disorder): Audiological Assessment beyond the Pure-tone Audiogram.

PubMed

Iliadou, Vasiliki Vivian; Chermak, Gail D; Bamiou, Doris-Eva

2015-04-01

According to the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, diagnosis of speech sound disorder (SSD) requires a determination that it is not the result of other congenital or acquired conditions, including hearing loss or neurological conditions that may present with similar symptomatology. To examine peripheral and central auditory function for the purpose of determining whether a peripheral or central auditory disorder was an underlying factor or contributed to the child's SSD. Central auditory processing disorder clinic pediatric case reports. Three clinical cases are reviewed of children with diagnosed SSD who were referred for audiological evaluation by their speech-language pathologists as a result of slower than expected progress in therapy. Audiological testing revealed auditory deficits involving peripheral auditory function or the central auditory nervous system. These cases demonstrate the importance of increasing awareness among professionals of the need to fully evaluate the auditory system to identify auditory deficits that could contribute to a patient's speech sound (phonological) disorder. Audiological assessment in cases of suspected SSD should not be limited to pure-tone audiometry given its limitations in revealing the full range of peripheral and central auditory deficits, deficits which can compromise treatment of SSD. American Academy of Audiology.

Obsessive-compulsive spectrum disorders

PubMed Central

Allen, Andrea; King, Audrey; Hollander, Eric

2003-01-01

The obsessive-compulsive spectrum is an important concept referring to a number of disorders drawn from several diagnostic categories that share core obsessive-compulsive features. These disorders can be grouped by the focus of their symptoms: bodily preoccupation, impulse control, or neurological disorders. Although the disorders are clearly distinct from one another, they have intriguing similarities in phenomenology, etiology, pathophysiology, patient characteristics, and treatment response. In combination with the knowledge gained through many years of research on obsessive-compulsive disorder (OCD), the concept of a spectrum has generated much fruitful research on the spectrum disorders. It has become apparent that these disorders can also be viewed as being on a continuum of compulsivity to impulsivity, characterized by harm avoidance at the compulsive end and risk seeking at the impulsive end. The compulsive and impulsive disorders differ in systematic ways that are just beginning to be understood. Here, we review these concepts and several representative obsessive-compulsive spectrum disorders including both compulsive and impulsive disorders, as well as the three different symptom clusters: OCD, body dysmorphic disorder, pathological gambling, sexual compulsivity, and autism spectrum disorders. PMID:22033547

Clinical characteristics and treatment outcomes of patients with major depressive disorder and comorbid anxiety disorders - results from a European multicenter study.

PubMed

Dold, Markus; Bartova, Lucie; Souery, Daniel; Mendlewicz, Julien; Serretti, Alessandro; Porcelli, Stefano; Zohar, Joseph; Montgomery, Stuart; Kasper, Siegfried

2017-08-01

This naturalistic European multicenter study aimed to elucidate the association between major depressive disorder (MDD) and comorbid anxiety disorders. Demographic and clinical information of 1346 MDD patients were compared between those with and without concurrent anxiety disorders. The association between explanatory variables and the presence of comorbid anxiety disorders was examined using binary logistic regression analyses. 286 (21.2%) of the participants exhibited comorbid anxiety disorders, 10.8% generalized anxiety disorder (GAD), 8.3% panic disorder, 8.1% agoraphobia, and 3.3% social phobia. MDD patients with comorbid anxiety disorders were characterized by younger age (social phobia), outpatient status (agoraphobia), suicide risk (any anxiety disorder, panic disorder, agoraphobia, social phobia), higher depressive symptom severity (GAD), polypsychopharmacy (panic disorder, agoraphobia), and a higher proportion receiving augmentation treatment with benzodiazepines (any anxiety disorder, GAD, panic disorder, agoraphobia, social phobia) and pregabalin (any anxiety disorder, GAD, panic disorder). The results in terms of treatment response were conflicting (better response for panic disorder and poorer for GAD). The logistic regression analyses revealed younger age (any anxiety disorder, social phobia), outpatient status (agoraphobia), suicide risk (agoraphobia), severe depressive symptoms (any anxiety disorder, GAD, social phobia), poorer treatment response (GAD), and increased administration of benzodiazepines (any anxiety disorder, agoraphobia, social phobia) and pregabalin (any anxiety disorder, GAD, panic disorder) to be associated with comorbid anxiety disorders. Our findings suggest that the various anxiety disorders subtypes display divergent clinical characteristics and are associated with different variables. Especially comorbid GAD appears to be characterized by high symptom severity and poor treatment response. Copyright © 2017 Elsevier Ltd. All

Executive Functioning Differences between Adults with Attention Deficit Hyperactivity Disorder and Autistic Spectrum Disorder in Initiation, Planning and Strategy Formation

ERIC Educational Resources Information Center

Bramham, Jessica; Ambery, Fiona; Young, Susan; Morris, Robin; Russell, Ailsa; Xenitidis, Kiriakos; Asherson, Philip; Murphy, Declan

2009-01-01

Executive functioning deficits characterize the neuropsychological profiles of the childhood neurodevelopmental disorders of attention deficit hyperactivity disorder (ADHD) and autistic spectrum disorder (ASD). This study sought to determine whether similar impairments exist in adults with ADHD (N = 53) and ASD (N = 45) in comparison with a…

Disrupted lymphocyte homeostasis in hepatitis‐associated acquired aplastic anemia is associated with short telomeres

PubMed Central

Babushok, Daria V.; Grignon, Anne‐Laure; Li, Yimei; Atienza, Jamie; Xie, Hongbo M.; Lam, Ho‐Sun; Hartung, Helge; Bessler, Monica

2016-01-01

Hepatitis‐associated aplastic anemia (HAA) is a variant of acquired aplastic anemia (AA) in which immune‐mediated bone marrow failure (BMF) develops following an acute episode of seronegative hepatitis. Dyskeratosis congenita (DC) is an inherited BMF syndrome characterized by the presence of short telomeres, mucocutaneous abnormalities, and cancer predisposition. While both conditions may cause BMF and hepatic impairment, therapeutic approaches are distinct, making it imperative to establish the correct diagnosis. In clinical practice, lymphocyte telomere lengths (TL) are used as a first‐line screen to rule out inherited telomeropathies before initiating treatment for AA. To evaluate the reliability of TL in the HAA population, we performed a retrospective analysis of TL in 10 consecutively enrolled HAA patients compared to 19 patients with idiopathic AA (IAA). HAA patients had significantly shorter telomeres than IAA patients (P = 0.009), including four patients with TL at or below the 1st percentile for age‐matched controls. HAA patients had no clinical features of DC and did not carry disease‐causing mutations in known genes associated with inherited telomere disorders. Instead, short TLs were significantly correlated with severe lymphopenia and skewed lymphocyte subsets, features characteristic of HAA. Our results indicate the importance of caution in the interpretation of TL measurements in HAA, because, in this patient population, short telomeres have limited specificity. Am. J. Hematol. 91:243–247, 2016. © 2015 The Authors. American Journal of Hematology Published by Wiley Periodicals, Inc. PMID:26615915

Measurement Sets and Sites Commonly Used for Characterization

NASA Technical Reports Server (NTRS)

Pagnutti, Mary; Holekamp, Kara; Ryan, Robert; Sellers, Richard; Davis, Bruce; Zanoni, Vicki

2002-01-01

Scientists at NASA's Earth Science Applications Directorate are creating a well-characterized Verification & Validation (V&V) site at the Stennis Space Center. This site enables the in-flight characterization of remote sensing systems and the data they acquire. The data are predominantly acquired by commercial, high spatial resolution satellite systems, such as IKONOS and QuickBird 2, and airborne systems. The smaller scale of these newer high resolution remote sensing systems allows scientists to characterize the geometric, spatial, and radiometric data properties using a single V&V site. The targets and techniques used to characterize data from these newer systems can differ significantly from the techniques used to characterize data from the earlier, coarser spatial resolution systems. Scientists are also using the SSC V&V site to characterize thermal infrared systems and active LIDAR systems. SSC employs geodetic targets, edge targets, radiometric tarps, and thermal calibration ponds to characterize remote sensing data products. This paper presents a proposed set of required measurements for visible through long-wave infrared remote sensing systems and a description of the Stennis characterization. Other topics discussed include: 1) The use of ancillary atmospheric and solar measurements taken at SSC that support various characterizations; 2) Additional sites used for radiometric, geometric, and spatial characterization in the continental United States; 3) The need for a standardized technique to be adopted by CEOS and other organizations.

Are major dissociative disorders characterized by a qualitatively different kind of dissociation?

PubMed

Rodewald, Frauke; Dell, Paul F; Wilhelm-Gossling, Claudia; Gast, Ursula

2011-01-01

A total of 66 patients with a major dissociative disorder, 54 patients with nondissociative disorders, and 30 nonclinical controls were administered the Structured Clinical Interview for DSM-IV Dissociative Disorders-Revised, the Dissociative Experiences Scale, the Multidimensional Inventory of Dissociation, and the Symptom Checklist 90-Revised. Dissociative patients reported significantly more dissociative and nondissociative symptoms than did nondissociative patients and nonclinical controls. When general psychopathology was controlled, the dissociation scores of dissociative patients were still significantly higher than those of both other groups, whereas the dissociation scores of nondissociative patients and nonclinical controls no longer differed. These findings appear to be congruent with a typological model of dissociation that distinguishes between 2 qualitatively different kinds of dissociation. Specifically, the results of this study suggest that the dissociation that occurs in major dissociative disorders (i.e., dissociative identity disorder [DID] and dissociative disorder not otherwise specified, Type 1 [DDNOS-1]) is qualitatively different from the dissociation that occurs in persons who do not have a dissociative disorder. In contrast to previous research, the dissociation of persons who do not have a dissociative disorder is not limited to absorption; it covers a much wider range of phenomena. The authors hypothesize that different mechanisms produce the dissociation of persons with DID and DDNOS-1 as opposed to the dissociation of persons who do not have a dissociative disorder.

Clinical significance of acquired somatic mutations in aplastic anaemia.

PubMed

Marsh, J C W; Mufti, G J

2016-08-01

Aplastic anaemia (AA) is frequently associated with other disorders of clonal haemopoiesis such as paroxysmal nocturnal haemoglobinuria (PNH), myelodysplastic syndrome (MDS) and T-large granular lymphocytosis. Certain clones may escape the immune attack within the bone marrow environment and proliferate and attain a survival advantage over normal haemopoietic stem cells, such as trisomy 8, loss of heterozygosity of short arm of chromosome 6 and del13q clones. Recently acquired somatic mutations (SM), excluding PNH clones, have been reported in around 20-25 % of patients with AA, which predispose to a higher risk of later malignant transformation to MDS/acute myeloid leukaemia. Furthermore, certain SM, such as ASXL1 and DNMT3A are associated with poor survival following immunosuppressive therapy, whereas PIGA, BCOR/BCORL1 predict for good response and survival. Further detailed and serial analysis of the immune signature in AA is needed to understand the pathogenetic basis for the presence of clones with SM in a significant proportion of patients.

Cytomegalovirus Retinitis as a Presenting Feature of Multisystem Disorder: Dyskeratosis Congenita.

PubMed

Parchand, Swapnil; Barwad, Adarsh

2017-01-01

Cytomegalovirus (CMV) retinitis is an opportunistic infection commonly seen in disorders that affect the immune system of the body such as acquired immunodeficiency syndrome and hematological malignancies such as leukemia/lymphoma or organ transplantation. The occurrence of CMV retinitis in the absence of such condition should be thoroughly investigated, as it is a strong indicator of poor immune competence. We here report an interesting case of CMV retinitis as a presenting feature of rare multisystem disorde r "Dyskeratosis congenita."

How to describe disordered structures

PubMed Central

Nishio, Kengo; Miyazaki, Takehide

2016-01-01

Disordered structures such as liquids and glasses, grains and foams, galaxies, etc. are often represented as polyhedral tilings. Characterizing the associated polyhedral tiling is a promising strategy to understand the disordered structure. However, since a variety of polyhedra are arranged in complex ways, it is challenging to describe what polyhedra are tiled in what way. Here, to solve this problem, we create the theory of how the polyhedra are tiled. We first formulate an algorithm to convert a polyhedron into a codeword that instructs how to construct the polyhedron from its building-block polygons. By generalizing the method to polyhedral tilings, we describe the arrangements of polyhedra. Our theory allows us to characterize polyhedral tilings, and thereby paves the way to study from short- to long-range order of disordered structures in a systematic way. PMID:27064833

Advancing biomarker research: utilizing 'Big Data' approaches for the characterization and prevention of bipolar disorder.

PubMed

McIntyre, Roger S; Cha, Danielle S; Jerrell, Jeanette M; Swardfager, Walter; Kim, Rachael D; Costa, Leonardo G; Baskaran, Anusha; Soczynska, Joanna K; Woldeyohannes, Hanna O; Mansur, Rodrigo B; Brietzke, Elisa; Powell, Alissa M; Gallaugher, Ashley; Kudlow, Paul; Kaidanovich-Beilin, Oksana; Alsuwaidan, Mohammad

2014-08-01

To provide a strategic framework for the prevention of bipolar disorder (BD) that incorporates a 'Big Data' approach to risk assessment for BD. Computerized databases (e.g., Pubmed, PsychInfo, and MedlinePlus) were used to access English-language articles published between 1966 and 2012 with the search terms bipolar disorder, prodrome, 'Big Data', and biomarkers cross-referenced with genomics/genetics, transcriptomics, proteomics, metabolomics, inflammation, oxidative stress, neurotrophic factors, cytokines, cognition, neurocognition, and neuroimaging. Papers were selected from the initial search if the primary outcome(s) of interest was (were) categorized in any of the following domains: (i) 'omics' (e.g., genomics), (ii) molecular, (iii) neuroimaging, and (iv) neurocognitive. The current strategic approach to identifying individuals at risk for BD, with an emphasis on phenotypic information and family history, has insufficient predictive validity and is clinically inadequate. The heterogeneous clinical presentation of BD, as well as its pathoetiological complexity, suggests that it is unlikely that a single biomarker (or an exclusive biomarker approach) will sufficiently augment currently inadequate phenotypic-centric prediction models. We propose a 'Big Data'- bioinformatics approach that integrates vast and complex phenotypic, anamnestic, behavioral, family, and personal 'omics' profiling. Bioinformatic processing approaches, utilizing cloud- and grid-enabled computing, are now capable of analyzing data on the order of tera-, peta-, and exabytes, providing hitherto unheard of opportunities to fundamentally revolutionize how psychiatric disorders are predicted, prevented, and treated. High-throughput networks dedicated to research on, and the treatment of, BD, integrating both adult and younger populations, will be essential to sufficiently enroll adequate samples of individuals across the neurodevelopmental trajectory in studies to enable the characterization

Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry.

PubMed

Baudo, Francesco; Collins, Peter; Huth-Kühne, Angela; Lévesque, Hervé; Marco, Pascual; Nemes, László; Pellegrini, Fabio; Tengborn, Lilian; Knoebl, Paul

2012-07-05

Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies to coagulation FVIII. Bleeding episodes at presentation are spontaneous and severe in most cases. Optimal hemostatic therapy is controversial, and available data are from observational and retrospective studies only. The EACH2 registry, a multicenter, pan-European, Web-based database, reports current patient management. The aim was to assess the control of first bleeding episodes treated with a bypassing agent (rFVIIa or aPCC), FVIII, or DDAVP among 501 registered patients. Of 482 patients with one or more bleeding episodes, 144 (30%) received no treatment for bleeding; 31 were treated with symptomatic therapy only. Among 307 patients treated with a first-line hemostatic agent, 174 (56.7%) received rFVIIa, 63 (20.5%) aPCC, 56 (18.2%) FVIII, and 14 (4.6%) DDAVP. Bleeding was controlled in 269 of 338 (79.6%) patients treated with a first-line hemostatic agent or ancillary therapy alone. Propensity score matching was applied to allow unbiased comparison between treatment groups. Bleeding control was significantly higher in patients treated with bypassing agents versus FVIII/DDAVP (93.3% vs 68.3%; P = .003). Bleeding control was similar between rFVIIa and aPCC (93.0%; P = 1). Thrombotic events were reported in 3.6% of treated patients with a similar incidence between rFVIIa (2.9%) and aPCC (4.8%).

Interpersonal Stress Regulation and the Development of Anxiety Disorders: An Attachment-Based Developmental Framework

PubMed Central

Nolte, Tobias; Guiney, Jo; Fonagy, Peter; Mayes, Linda C.; Luyten, Patrick

2011-01-01

Anxiety disorders represent a common but often debilitating form of psychopathology in both children and adults. While there is a growing understanding of the etiology and maintenance of these disorders across various research domains, only recently have integrative accounts been proposed. While classical attachment history has been a traditional core construct in psychological models of anxiety, contemporary attachment theory has the potential to integrate neurobiological and behavioral findings within a multidisciplinary developmental framework. The current paper proposes a modern attachment theory-based developmental model grounded in relevant literature from multiple disciplines including social neuroscience, genetics, neuroendocrinology, and the study of family factors involved in the development of anxiety disorders. Recent accounts of stress regulation have highlighted the interplay between stress, anxiety, and activation of the attachment system. This interplay directly affects the development of social–cognitive and mentalizing capacities that are acquired in the interpersonal context of early attachment relationships. Early attachment experiences are conceptualized as the key organizer of a complex interplay between genetic, environmental, and epigenetic contributions to the development of anxiety disorders – a multifactorial etiology resulting from dysfunctional co-regulation of fear and stress states. These risk-conferring processes are characterized by hyperactivation strategies in the face of anxiety. The cumulative allostatic load and subsequent “wear and tear” effects associated with hyperactivation strategies converge on the neural pathways of anxiety and stress. Attachment experiences further influence the development of anxiety as potential moderators of risk factors, differentially impacting on genetic vulnerability and relevant neurobiological pathways. Implications for further research and potential treatments are outlined. PMID

Elderly persons with ICU-acquired weakness: the potential role for β-hydroxy-β-methylbutyrate (HMB) supplementation?

PubMed

Rahman, Adam; Wilund, Kenneth; Fitschen, Peter J; Jeejeebhoy, Khursheed; Agarwala, Ravi; Drover, John W; Mourtzakis, Marina

2014-07-01

Intensive care unit (ICU)-acquired weakness is common and characterized by muscle loss, weakness, and paralysis. It is associated with poor short-term outcomes, including increased mortality, but the consequences of reduced long-term outcomes, including decreased physical function and quality of life, can be just as devastating. ICU-acquired weakness is particularly relevant to elderly patients who are increasingly consuming ICU resources and are at increased risk for ICU-acquired weakness and complications, including mortality. Elderly patients often enter critical illness with reduced muscle mass and function and are also at increased risk for accelerated disuse atrophy with acute illness. Increasingly, intensivists and researchers are focusing on strategies and therapies aimed at improving long-term neuromuscular function. β-Hydroxy-β-methylbutyrate (HMB), an ergogenic supplement, has shown efficacy in elderly patients and certain clinical populations in counteracting muscle loss. The present review discusses ICU-acquired weakness, as well as the unique physiology of muscle loss and skeletal muscle function in elderly patients, and then summarizes the evidence for HMB in elderly patients and in clinical populations. We subsequently postulate on the potential role and strategies in studying HMB in elderly ICU patients to improve muscle mass and function. © 2013 American Society for Parenteral and Enteral Nutrition.

A multicenter qualitative study on preventing hospital-acquired urinary tract infection in US hospitals.

PubMed

Saint, Sanjay; Kowalski, Christine P; Forman, Jane; Damschroder, Laura; Hofer, Timothy P; Kaufman, Samuel R; Creswell, John W; Krein, Sarah L

2008-04-01

Although urinary tract infection (UTI) is the most common hospital-acquired infection, there is little information about why hospitals use or do not use a range of available preventive practices. We thus conducted a multicenter study to understand better how US hospitals approach the prevention of hospital-acquired UTI. This research is part of a larger study employing both quantitative and qualitative methods. The qualitative phase consisted of 38 semistructured phone interviews with key personnel at 14 purposefully sampled US hospitals and 39 in-person interviews at 5 of those 14 hospitals, to identify recurrent and unifying themes that characterize how hospitals have addressed hospital-acquired UTI. Four recurrent themes emerged from our study data. First, although preventing hospital-acquired UTI was a low priority for most hospitals, there was substantial recognition of the value of early removal of a urinary catheter for patients. Second, those hospitals that made UTI prevention a high priority also focused on noninfectious complications and had committed advocates, or "champions," who facilitated prevention activities. Third, hospital-specific pilot studies were important in deciding whether or not to use devices such as antimicrobial-impregnated catheters. Finally, external forces, such as public reporting, influenced UTI surveillance and infection prevention activities. Clinicians and policy makers can use our findings to develop initiatives that, for example, use a champion to promote the removal of unnecessary urinary catheters or exploit external forces, such public reporting, to enhance patient safety.

Sleep-related movement disorders.

PubMed

Merlino, Giovanni; Gigli, Gian Luigi

2012-06-01

Several movement disorders may occur during nocturnal rest disrupting sleep. A part of these complaints is characterized by relatively simple, non-purposeful and usually stereotyped movements. The last version of the International Classification of Sleep Disorders includes these clinical conditions (i.e. restless legs syndrome, periodic limb movement disorder, sleep-related leg cramps, sleep-related bruxism and sleep-related rhythmic movement disorder) under the category entitled sleep-related movement disorders. Moreover, apparently physiological movements (e.g. alternating leg muscle activation and excessive hypnic fragmentary myoclonus) can show a high frequency and severity impairing sleep quality. Clinical and, in specific cases, neurophysiological assessments are required to detect the presence of nocturnal movement complaints. Patients reporting poor sleep due to these abnormal movements should undergo non-pharmacological or pharmacological treatments.

Characterizing Factors of Employment Status in Persons With Major Depressive Disorder.

PubMed

Chen, Fang-Pei; Samet, Sharon; Gorroochurn, Prakash; O'Hara, Kathleen M

2016-09-01

Employment is fundamental to mental health recovery. The aim of this study is to construct a parsimonious profile indicating employment potential of people with major depressive disorder (MDD) to facilitate clinical assessment on employment participation. Data were drawn from Waves 1 (2001-2002) and 2 (2004-2005) of the National Epidemiologic Survey on Alcohol and Related Conditions. We included participants who had MDD at Wave 1 and were interviewed in both waves (N = 2,864). We conducted Classification and Regression Tree (CART) analysis to identify key characterizing factors of Wave 2 employment among 32 Wave 1 risk and protective factors. The results show that 82.1% of those employed at Wave 1 were likely to be employed at Wave 2. Among those unemployed at Wave 1, 51% of those motivated to work, measured by work-seeking behavior in the prior year, were likely to be employed at Wave 2. Among those unemployed and motivated to work, better functional mental health was associated with employment (>25.3 vs. ≤25.3). Results highlight the importance of motivation to work, shown in active work seeking, in facilitating employment despite clinical conditions. © The Author(s) 2015.

Community-acquired bacterial meningitis.

PubMed

van de Beek, Diederik; Brouwer, Matthijs; Hasbun, Rodrigo; Koedel, Uwe; Whitney, Cynthia G; Wijdicks, Eelco

2016-11-03

Meningitis is an inflammation of the meninges and subarachnoid space that can also involve the brain cortex and parenchyma. It can be acquired spontaneously in the community - community-acquired bacterial meningitis - or in the hospital as a complication of invasive procedures or head trauma (nosocomial bacterial meningitis). Despite advances in treatment and vaccinations, community-acquired bacterial meningitis remains one of the most important infectious diseases worldwide. Streptococcus pneumoniae and Neisseria meningitidis are the most common causative bacteria and are associated with high mortality and morbidity; vaccines targeting these organisms, which have designs similar to the successful vaccine that targets Haemophilus influenzae type b meningitis, are now being used in many routine vaccination programmes. Experimental and genetic association studies have increased our knowledge about the pathogenesis of bacterial meningitis. Early antibiotic treatment improves the outcome, but the growing emergence of drug resistance as well as shifts in the distribution of serotypes and groups are fuelling further development of new vaccines and treatment strategies. Corticosteroids were found to be beneficial in high-income countries depending on the bacterial species. Further improvements in the outcome are likely to come from dampening the host inflammatory response and implementing preventive measures, especially the development of new vaccines.

[Fegelers syndrome, acquired port-wine stain or acquired capillary malformation: three cases and a literature review].

PubMed

Freysz, M; Cribier, B; Lipsker, D

2013-05-01

Port-wine stains or capillary malformations are generally congenital. Very few cases of acquired port-wine stains in adults have been described, and these occur particularly after trauma. We report three cases of acquired port-wine stains and we performed a review of the literature using the keywords "port-wine stain", "capillary malformation", "angioma" and "acquired" in the Medline database PubMed. All relevant articles were included. Two male patients and one female patient consulted for one or more angiomatous lesions, located respectively on the upper rear part of the right thigh (case 1), the left leg (case 2) and the right side of the face, skull and chest (case 3). Each patient's skin biopsy was consistent with port-wine stain. The three patients asserted the acquired nature of the lesions: the male patients were respectively 17 and 38 years old, and the female patient was 11 years old. No causative factors were evident preceding the lesion, and there was no family history of port-wine stain. The topography was systematic in patients 2 and 3. The lesions were light red in patient 1, dark red in patient 2 and pale pink in patient 3. The remainder of the physical examination was unremarkable, except for benign angiokeratoma of the scrotum in case 1 and pigmented leucoderma-type macules in case 3. LITERATURE RESULTS: Sixty-six cases of acquired port-wine stains were reported in the literature. The average age was 25 years (3-69) with a sex-ratio of 0.88. Generally, no causative factor was given. However, trauma (30.5%), estrogenic impregnation (16.5%), and more rarely, medication, solar damage, frostbite, cluster headache, herpes zoster and acoustic neuroma were reported as causatives factors. Acquired port-wine stain is rare. Although often idiopathic, it can result from spinal trauma, which must be explored if suggested by the history. In our series, the clinical presentation suggested a latent congenital vascular malformation of late onset, in particular in

The Best Time to Acquire New Skills: Age-Related Differences in Implicit Sequence Learning across the Human Lifespan

ERIC Educational Resources Information Center

Janacsek, Karolina; Fiser, Jozsef; Nemeth, Dezso

2012-01-01

Implicit skill learning underlies obtaining not only motor, but also cognitive and social skills through the life of an individual. Yet, the ontogenetic changes in humans' implicit learning abilities have not yet been characterized, and, thus, their role in acquiring new knowledge efficiently during development is unknown. We investigated such…

[Innate immunity in neuroimmunological disorders].

PubMed

Miyake, Sachiko

2013-05-01

Exogeneous pathogen-associated molecular patterns and endogenous danger signals bind to pattern recognition receptors and activate innate immunity cells, leading to proinflammatory cytokine production and activation of acquired immue cells. These are important factors in the pathogenesis of autoimmune-mediated neuroimmunological disorders such as multiple sclerosis. Furthermore, recent advances in the study of innate immunity revealed that innate immunity is a major players in the pathogenesis of some neuroimmunological diseases such as Behçet's disease and herpes simplex virus encephalitis.

[Neural mechanism underlying autistic savant and acquired savant syndrome].

PubMed

Takahata, Keisuke; Kato, Motoichiro

2008-07-01

It is well known that the cases with savant syndrome, demonstrate outstanding mental capability despite coexisting severe mental disabilities. In many cases, savant skills are characterized by its domain-specificity, enhanced memory capability, and excessive focus on low-level perceptual processing. In addition, impaired integrative cognitive processing such as social cognition or executive function, restricted interest, and compulsive repetition of the same act are observed in savant individuals. All these are significantly relevant to the behavioral characteristics observed in individuals with autistic spectrum disorders (ASD). A neurocognitive model of savant syndrome should explain these cognitive features and the juxtaposition of outstanding talents with cognitive disabilities. In recent neuropsychological studies, Miller (1998) reported clinical cases of "acquired savant," i.e., patients who improved or newly acquired an artistic savant-like skill in the early stage of frontotemporal dementia (FTD). Although the relationship between an autistic savant and acquired savant remains to be elucidated, the advent of neuroimaging study of ASD and the clarification of FTD patients with savant-like skills may clarify the shared neural mechanisms of both types of talent. In this review, we classified current cognitive models of savant syndrome into the following 3 categories. (1) A hypermnesic model that suggests that savant skills develop from existing or dormant cognitive functions such as memory. However, recent findings obtained through neuropsychological examinations imply that savant individuals solve problems using a strategy that is fairly different from a non-autistic one. (2) A paradoxical functional facilitation model (Kapur, 1996) that offers possible explanations about how pathological states in the brain lead to development of prodigious skills. This model emphasizes the role of reciprocal inhibitory interaction among adjacent or distant cortical regions

Disorders of Thought Are Severe Mood Disorders: the Selective Attention Defect in Mania Challenges the Kraepelinian Dichotomy—A Review

PubMed Central

Raymond Lake, C.

2008-01-01

Kraepelin said severe mental illness was due to 2 diseases subsequently characterized as disorders of thought vs disorders of mood, ie, the Kraepelinian dichotomy. Schizophrenia, traditionally considered the disorder of thought, has been defined by the presence of hallucinations, delusions, catatonia, and disorganization. Tangentiality, derailment, loose associations, and thought blocking are typically considered pathognomonic of schizophrenia. By contrast, the mood disorders have been characterized only as disorders of the emotions, though both depression and mania, when severe, are now recognized to include the same psychotic features traditionally considered diagnostic of schizophrenia. This article addresses disordered thinking in mania in order to clarify the relationship between schizophrenia and psychotic mood disorders. Normally, the brain's selective attention mechanism filters and prioritizes incoming stimuli by excluding from consciousness extraneous, low-priority stimuli and grading the importance of more relevant data. Because this “filter/prioritizer” becomes defective in mania, tangential stimuli are processed without appropriate prioritization. Observed as distractibility, this symptom is an index of the breakdown in selective attention and the severity of mania, accounting for the signs and symptoms of psychotic thinking. The zone of rarity between schizophrenia and psychotic mood disorders is blurred because severe disorders of mood are also disorders of thought. This relationship calls into question the tenet that schizophrenia is a disease separate from psychotic mood disorders. Patients whose case histories are discussed herein gave their written informed consent to participate in this institutional human subjects committee–approved protocol. PMID:17515440

Obsessive-compulsive disorder, impulse control disorders and drug addiction: common features and potential treatments.

PubMed

Fontenelle, Leonardo F; Oostermeijer, Sanne; Harrison, Ben J; Pantelis, Christos; Yücel, Murat

2011-05-07

The basic concepts underlying compulsive, impulsive and addictive behaviours overlap, which may help explain why laymen use these expressions interchangeably. Although there has been a large research effort to better characterize and disentangle these behaviours, clinicians and scientists are still unable to clearly differentiate them. Accordingly, obsessive-compulsive disorder (OCD), impulse control disorders (ICD) and substance-related disorders (SUD) overlap on different levels, including phenomenology, co-morbidity, neurocircuitry, neurocognition, neurochemistry and family history. In this review we summarize these issues with particular emphasis on the role of the opioid system in the pathophysiology and treatment of OCD, ICD and SUD. We postulate that with progression and chronicity of OCD, the proportion of the OCD-related behaviours (e.g. checking, washing, ordering and hoarding, among others) that are driven by impulsive 'rash' processes increase as involvement of more ventral striatal circuits becomes prominent. In contrast, as SUD and ICD progress, the proportion of the SUD- and ICD-related behaviours that are driven by compulsive 'habitual' processes increase as involvement of more dorsal striatal circuits become prominent. We are not arguing that, with time, ICD becomes OCD or vice versa. Instead, we are proposing that these disorders may acquire qualities of the other with time. In other words, while patients with ICD/SUD may develop 'compulsive impulsions', patients with OCD may exhibit 'impulsive compulsions'. There are many potential implications of our model. Theoretically, OCD patients exhibiting impulsive or addictive features could be managed with drugs that address the quality of the underlying drives and the involvement of neural systems. For example, agents for the reduction or prevention of relapse of addiction (e.g. heavy drinking), which modulate the cortico-mesolimbic dopamine system through the opioid (e.g. buprenorphine and naltrexone

Acquired perforating dermatosis in a patient with chronic renal failure.

PubMed

Fernandes, Karen de Almeida Pinto; Lima, Lourenço de Azevedo; Guedes, Juliana Chaves Ruiz; Lima, Ricardo Barbosa; D'Acri, Antônio Macedo; Martins, Carlos José

2016-01-01

Perforating dermatoses are a group of skin diseases characterized by transepidermal elimination of dermal material. The disease is divided into two groups: the primary group and the secondary group. The classical or primary perforating dermatoses are subdivided into four types according to the eliminated dermal materials: Kyrle disease, perforating reactive collagenosis, elastosis perforans serpiginosa, and perforating folliculitis. The secondary form is known as acquired perforating dermatosis. The term was proposed in 1989 by Rapini to designate the perforating dermatoses affecting adult patients with systemic disease, regardless of the dermal materials eliminated. This report describes a case of the disease with elimination of collagen and elastic fibers in a patient with chronic renal failure.

Acquired partial lipodystrophy is associated with increased risk for developing metabolic abnormalities.

PubMed

Akinci, Baris; Koseoglu, Fatos Dilan; Onay, Huseyin; Yavuz, Sevgi; Altay, Canan; Simsir, Ilgin Yildirim; Ozisik, Secil; Demir, Leyla; Korkut, Meltem; Yilmaz, Nusret; Ozen, Samim; Akinci, Gulcin; Atik, Tahir; Calan, Mehmet; Secil, Mustafa; Comlekci, Abdurrahman; Demir, Tevfik

2015-09-01

Acquired partial lipodystrophy (APL) is a rare disorder characterized by progressive selective fat loss. In previous studies, metabolic abnormalities were reported to be relatively rare in APL, whilst they were quite common in other types of lipodystrophy syndromes. In this nationwide cohort study, we evaluated 21 Turkish patients with APL who were enrolled in a prospective follow-up protocol. Subjects were investigated for metabolic abnormalities. Fat distribution was assessed by whole body MRI. Hepatic steatosis was evaluated by ultrasound, MRI and MR spectroscopy. Patients with diabetes underwent a mix meal stimulated C-peptide/insulin test to investigate pancreatic beta cell functions. Leptin and adiponectin levels were measured. Fifteen individuals (71.4%) had at least one metabolic abnormality. Six patients (28.6%) had diabetes, 12 (57.1%) hypertrigylceridemia, 10 (47.6%) low HDL cholesterol, and 11 (52.4%) hepatic steatosis. Steatohepatitis was further confirmed in 2 patients with liver biopsy. Anti-GAD was negative in all APL patients with diabetes. APL patients with diabetes had lower leptin and adiponectin levels compared to patients with type 2 diabetes and healthy controls. However, contrary to what we observed in patients with congenital generalized lipodystrophy (CGL), we did not detect consistently very low leptin levels in APL patients. The mix meal test suggested that APL patients with diabetes had a significant amount of functional pancreatic beta cells, and their diabetes was apparently associated with insulin resistance. Our results show that APL is associated with increased risk for developing metabolic abnormalities. We suggest that close long-term follow-up is required to identify and manage metabolic abnormalities in APL. Copyright © 2015 Elsevier Inc. All rights reserved.

Characterization of Medication Use in a Multicenter Sample of Pediatric Inpatients with Autism Spectrum Disorder.

PubMed

Wink, Logan K; Pedapati, Ernest V; Adams, Ryan; Erickson, Craig A; Pedersen, Kahsi A; Morrow, Eric M; Kaplan, Desmond; Siegel, Matthew

2017-05-17

Nearly 11% of youth with Autism Spectrum Disorder (ASD) undergo psychiatric hospitalization, and 65% are treated with psychotropic medication. Here we characterize psychotropic medication usage in subjects enrolled in the Autism Inpatient Collection. Participant psychotropic medication usage rates topped 90% at admission and discharge, though there was a decline at 2-month follow-up. Antipsychotics, ADHD medications, and sleep aids were the most commonly reported classes of medications. The impact of age, gender, and non-verbal IQ on medication usage rates was minimal, though age and IQ may play a role in prescribing practices. Future work is indicated to explore medication usage trends, the impact of clinical factors on medication use rates, and the safety of psychotropic medications in youth with ASD.

Preschoolers Acquire General Knowledge by Sharing in Pretense

ERIC Educational Resources Information Center

Sutherland, Shelbie L.; Friedman, Ori

2012-01-01

Children acquire general knowledge about many kinds of things, but there are few known means by which this knowledge is acquired. In this article, it is proposed that children acquire generic knowledge by sharing in pretend play. In Experiment 1, twenty-two 3- to 4-year-olds watched pretense in which a puppet represented a "nerp" (an unfamiliar…

Desmosomes in acquired disease

PubMed Central

Stahley, Sara N.; Kowalczyk, Andrew P.

2015-01-01

Desmosomes are cell-cell junctions that mediate adhesion and couple the intermediate filament cytoskeleton to sites of cell-cell contact. This architectural arrangement functions to integrate adhesion and cytoskeletal elements of adjacent cells. The importance of this robust adhesion system is evident in numerous human diseases, both inherited and acquired, that occur when desmosome function is compromised. This review focuses on autoimmune and infectious diseases that impair desmosome function. In addition, we discuss emerging evidence that desmosomal genes are often misregulated in cancer. The emphasis of our discussion is placed on how human diseases inform our understanding of basic desmosome biology, and in turn, how fundamental advances in the cell biology of desmosomes may lead to new treatments for acquired diseases of the desmosome. PMID:25795143

Adults with Asperger Syndrome: A Childhood Disorder Grows Up

ERIC Educational Resources Information Center

Wilkinson, Lee A.

2007-01-01

Asperger syndrome is a chronic developmental disorder characterized by problems in social relatedness, empathic communication and understanding, and circumscribed interests. The inclusion of Asperger's Disorder (Asperger syndrome) in the "Diagnostic and Statistical Manual of Mental Disorders" (DSM-IV; American Psychiatric Association, 1994), has…

Conjunctival Primary Acquired Melanosis: Is It Time for a New Terminology?

PubMed

Jakobiec, Frederick A

2016-02-01

To review the diagnostic categories of a group of conditions referred to as "primary acquired melanosis." Literature review on the subject and proposal of an alternative diagnostic schema with histopathologic and immunohistochemical illustrations. Standard hematoxylin-eosin-stained sections and immunohistochemical stains for MART-1, HMB-45, microphthalmia-associated transcription factor (MiTF), and Ki-67 for calculating the proliferation index are illustrated. "Melanosis" is an inadequate and misleading term because it does not distinguish between conjunctival intraepithelial melanin overproduction ("hyperpigmentation") and intraepithelial melanocytic proliferation. It is recommended that "intraepithelial melanocytic proliferation" be adopted for histopathologic diagnosis. Atypical proliferations are characterized either by bloated dendritic melanocytes with enlarged cell components (dendrites, cell bodies, and nuclei) or by epithelioid melanocytes without dendrites. Atypical polygonal or epithelioid pagetoid cells may reach higher levels of the epithelium beyond the basal layer. Immunohistochemistry defines the degree of melanocytic proliferation or the cellular shape (dendritic or nondendritic) (MART-1, HMB-45) or identifies the melanocytic nuclei (MiTF). Intraepithelial melanocytic proliferation without atypia represents increased numbers of normal-appearing dendritic melanocytes (hyperplasia or early neoplasia) that generally remain confined to the basal/basement membrane region. Intraepithelial nonproliferative melanocytic pigmentation signifies the usually small number of conjunctival basal dendritic melanocytes that synthesize increased amounts of melanin that is transferred to surrounding keratinocytes. All pre- and postoperative biopsies of flat conjunctival melanocytic disorders should be evaluated immunohistochemically if there is any question regarding atypicality. This should lead to a clearer microscopic descriptive diagnosis that is predicated on an

Are Eating Disorders Related to Attention Deficit/Hyperactivity Disorder?

PubMed Central

Reinblatt, Shauna P.

2016-01-01

Opinion statement Attention deficit/hyperactivity disorder (ADHD) is a disorder characterized by impulsivity, hyperactivity, and inattention. Binge-eating behavior is often impulsive and is the hallmark of the two eating disorders, binge-eating disorder (BED) and bulimia nervosa (BN), both of which are associated with significant health impairment. Bingeing behavior is also seen in the binge purge subtype of anorexia nervosa. Individuals with AN of the binge purge subtypes, BN and BED, have been found to exhibit impulsive behaviors that are often not limited to binge eating alone. There is preliminary evidence linking ADHD to BN and to BED in both adults and children. The neurobiological mechanisms behind these associations are only beginning to emerge; however, they suggest that impulse control deficits may play a role in these eating disorders. Additionally, although they may not meet full criteria for one of these eating disorders, some adults and children with ADHD present with dysregulated, impulsive eating disorder behaviors and there is a growing association between ADHD, obesity, and binge-eating behavior in both children and adults. The relationship between ADHD and binge eating is novel, supported by growing evidence and worthy of further research. We will review the underlying neurobiological underpinnings, neuroimaging data, and possible psychopharmacological treatment options, which target both ADHD and binge-eating behaviors as well as future research and treatment directions. PMID:26949595

Corpus callosum demyelination associated with acquired stuttering.

PubMed

Decker, Barbara McElwee; Guitar, Barry; Solomon, Andrew

2018-04-21

Compared with developmental stuttering, adult onset acquired stuttering is rare. However, several case reports describe acquired stuttering and an association with callosal pathology. Interestingly, these cases share a neuroanatomical localisation also demonstrated in developmental stuttering. We present a case of adult onset acquired stuttering associated with inflammatory demyelination within the corpus callosum. This patient's disfluency improved after the initiation of immunomodulatory therapy. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Risk characterization of hospitalizations for mental illness and/or behavioral disorders with concurrent heat-related illness

PubMed Central

2017-01-01

Background Many studies have found significant associations between high ambient temperatures and increases in heat-related morbidity and mortality. Several studies have demonstrated that increases in heat-related hospitalizations are elevated among individuals with diagnosed mental illnesses and/or behavioral disorders (MBD). However, there are a limited number of studies regarding risk factors associated with specific mental illnesses that contribute, at least in part, to heat-related illnesses (HRI) in the United States. Objective To identify and characterize individual and environmental risk factors associated with MBD hospitalizations with a concurrent HRI diagnosis. Methods This study uses hospitalization data from the Nationwide Inpatient Sample (2001–2010). Descriptive analyses of primary and secondary diagnoses of MBDs with an HRI were examined. Risk ratios (RR) were calculated from multivariable models to identify risk factors for hospitalizations among patients with mental illnesses and/or behavioral disorders and HRI. Results Nondependent alcohol/drug abuse, dementia, and schizophrenia were among the disorders that were associated with increased frequency of HRI hospitalizations among MBD patients. Increased risk of MBD hospitalizations with HRI was observed for Males (RR, 3.06), African Americans (RR, 1.16), Native Americans (RR, 1.70), uninsured (RR, 1.92), and those 40 years and older, compared to MBD hospitalizations alone. Conclusions Previous studies outside the U.S. have found that dementia and schizophrenia are significant risk factors for HRI hospitalizations. Our results suggest that hospitalizations among substance abusers may also be an important risk factor associated with heat morbidity. Improved understanding of these relative risks could help inform future public health strategies. PMID:29036206

Developmental and benign movement disorders in childhood.

PubMed

Bonnet, Cecilia; Roubertie, Agathe; Doummar, Diane; Bahi-Buisson, Nadia; Cochen de Cock, Valérie; Roze, Emmanuel

2010-07-30

Developmental and benign movement disorders are a group of movement disorders with onset in the neonatal period, infancy, or childhood. They are characterized by the absence of associated neurological manifestations and by their favorable outcome, although developmental abnormalities can be occasionally observed. Knowledge of the clinical, neurophysiological, and pathogenetic aspects of these disorders is poor. Based on a comprehensive review of the literature and our practical experience, this article summarizes current knowledge in this area. We pay special attention to the recognition and management of these movement disorders in children. (c) 2010 Movement Disorder Society.

Spinal cord toxoplasmosis in human immunodeficiency virus infection/acquired immunodeficiency syndrome.

PubMed

García-García, Concepción; Castillo-Álvarez, Federico; Azcona-Gutiérrez, José M; Herraiz, María J; Ibarra, Valvanera; Oteo, José A

2015-05-01

Neurological complications in patients with human immunodeficiency virus infection/acquired immunodeficiency syndrome (HIV/AIDS) are still common, even in the era of highly active antiretroviral therapy. Opportunistic infections, immune reconstitution, the virus itself, antiretroviral drugs and neurocognitive disorders have to be considered when establishing the differential diagnosis. Toxoplasmic encephalitis remains the major cause of space-occupying lesions in the brain of patients with HIV/AIDS; however, spinal cord involvement has been reported infrequently. Here, we review spinal cord toxoplasmosis in HIV infection and illustrate the condition with a recent case from our hospital. We suggest that most patients with HIV/AIDS and myelitis with enhanced spine lesions, multiple brain lesions and positive serology for Toxoplasma gondii should receive immediate empirical treatment for toxoplasmosis, and a biopsy should be performed in those cases without clinical improvement or with deterioration.

Diminished medial prefrontal cortex activation during the recollection of stressful events is an acquired characteristic of PTSD.

PubMed

Dahlgren, M K; Laifer, L M; VanElzakker, M B; Offringa, R; Hughes, K C; Staples-Bradley, L K; Dubois, S J; Lasko, N B; Hinojosa, C A; Orr, S P; Pitman, R K; Shin, L M

2018-05-01

Previous research has shown relatively diminished medial prefrontal cortex activation and heightened psychophysiological responses during the recollection of personal events in post-traumatic stress disorder (PTSD), but the origin of these abnormalities is unknown. Twin studies provide the opportunity to determine whether such abnormalities reflect familial vulnerabilities, result from trauma exposure, or are acquired characteristics of PTSD. In this case-control twin study, 26 male identical twin pairs (12 PTSD; 14 non-PTSD) discordant for PTSD and combat exposure recalled and imagined trauma-unrelated stressful and neutral life events using a standard script-driven imagery paradigm during functional magnetic resonance imaging and concurrent skin conductance measurement. Diminished activation in the medial prefrontal cortex during Stressful v. Neutral script-driven imagery was observed in the individuals with PTSD, relative to other groups. Diminished medial prefrontal cortex activation during Stressful v. Neutral script-driven imagery may be an acquired characteristic of PTSD. If replicated, this finding could be used prospectively to inform diagnosis and the assessment of treatment response.

Combining land use data acquired from Landsat with soil map data

NASA Technical Reports Server (NTRS)

Westin, F. C.; Brandner, T. M.

1981-01-01

A method currently used to derive agrophysical units (APUs), i.e., geographical areas having definable/comparable agronomic and physical parameters which reflect a range in agricultural use and management, is discussed with reference to results obtained for South Dakota and an area in China. The method consists of combining agricultural land use data acquired from Landsat with soil map data. The resulting map units are soil associations characterized by cropland use intensity, and they can be used to identify major cropland areas and to develop a rating reflecting the relative potential of the soils in the delineated area for crop production, as well as to update small-scale soil maps.

Neurodevelopmental behavioral and cognitive disorders.

PubMed

Jeste, Shafali Spurling

2015-06-01

Neurodevelopmental disorders are a group of heterogeneous conditions characterized by a delay or disturbance in the acquisition of skills in a variety of developmental domains, including motor, social, language, and cognition. This article reviews the most commonly diagnosed neurodevelopmental disorders, which include attention deficit hyperactivity disorder (ADHD), autism spectrum disorder, global developmental delay, and intellectual disability and also provides updates on diagnosis, neurobiology, treatment, and issues surrounding the transition to adulthood. Although symptoms emerge at discrete points in childhood, these disorders result from abnormal brain maturation that likely precedes clinical impairment. As a result, research has focused on the identification of predictive biological and behavioral markers, with the ultimate goal of initiating treatments that may either alter developmental trajectories or lessen clinical severity. Advances in the methods used to identify genetic variants, from chromosomal microarray analysis to whole exome sequencing, have facilitated the characterization of many genetic mutations and syndromes that share common pathways to abnormal circuit formation and brain development. Not only do genetic discoveries enrich our understanding of mechanisms underlying atypical development, but they also allow us to identify more homogeneous subgroups within this spectrum of conditions. Impairments do continue into adulthood, with challenges in the transition to adulthood including the management of comorbidities and the provision of educational and vocational supports. Advances in our understanding of the neurobiology and developmental trajectories of these disorders will pave the way for tremendous advances in treatment. Mechanism-based therapies for genetic syndromes are being studied with the goal of expanding targeted treatments to nonsyndromic forms of neurodevelopmental disorders.

The influence of (central) auditory processing disorder in speech sound disorders.

PubMed

Barrozo, Tatiane Faria; Pagan-Neves, Luciana de Oliveira; Vilela, Nadia; Carvallo, Renata Mota Mamede; Wertzner, Haydée Fiszbein

2016-01-01

Considering the importance of auditory information for the acquisition and organization of phonological rules, the assessment of (central) auditory processing contributes to both the diagnosis and targeting of speech therapy in children with speech sound disorders. To study phonological measures and (central) auditory processing of children with speech sound disorder. Clinical and experimental study, with 21 subjects with speech sound disorder aged between 7.0 and 9.11 years, divided into two groups according to their (central) auditory processing disorder. The assessment comprised tests of phonology, speech inconsistency, and metalinguistic abilities. The group with (central) auditory processing disorder demonstrated greater severity of speech sound disorder. The cutoff value obtained for the process density index was the one that best characterized the occurrence of phonological processes for children above 7 years of age. The comparison among the tests evaluated between the two groups showed differences in some phonological and metalinguistic abilities. Children with an index value above 0.54 demonstrated strong tendencies towards presenting a (central) auditory processing disorder, and this measure was effective to indicate the need for evaluation in children with speech sound disorder. Copyright © 2015 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Disgust, Fear, and the Anxiety Disorders: A Critical Review

PubMed Central

Cisler, Josh M.; Olatunji, Bunmi O.; Lohr, Jeffrey M.

2010-01-01

Anxiety disorders have traditionally been conceptualized as reflecting the emotions of fear and anxiety. A developing program of research demonstrates a relation between disgust and three specific anxiety disorders: blood-injection-injury (BII) phobia, spider phobia, and contamination-related obsessive-compulsive disorder (OCD). This review serves three purposes. First, the authors review the response patterns predicted to be observed if the emotional response in these disorders involved disgust versus fear. The review suggests specific response patterns that characterize disgust and fear in the domains of heart rate, facial expression, neural activity, and cognitive processes. Second, the authors review extant research employing measures of these domains in spider phobia, BII phobia, and contamination-related OCD. The evidence suggests that both fear and disgust characterize each of these disorders, but the magnitude at which the emotions characterize the disorders may depend on the response domain measured. For example, disgust may be more involved in spider phobia in appraisals and facial expression, but less involved in neural correlates or heart rate domains. Third, the authors suggest guidelines for future research, including concurrent use of specific measures as well as examining whether the different emotions in different response domains respond to similar interventions (e.g., exposure). PMID:18977061

Screening tools for the identification of dementia for adults with age-related acquired hearing or vision impairment: a scoping review.

PubMed

Pye, Annie; Charalambous, Anna Pavlina; Leroi, Iracema; Thodi, Chrysoulla; Dawes, Piers

2017-11-01

Cognitive screening tests frequently rely on items being correctly heard or seen. We aimed to identify, describe, and evaluate the adaptation, validity, and availability of cognitive screening and assessment tools for dementia which have been developed or adapted for adults with acquired hearing and/or vision impairment. Electronic databases were searched using subject terms "hearing disorders" OR "vision disorders" AND "cognitive assessment," supplemented by exploring reference lists of included papers and via consultation with health professionals to identify additional literature. 1,551 papers were identified, of which 13 met inclusion criteria. Four papers related to tests adapted for hearing impairment; 11 papers related to tests adapted for vision impairment. Frequently adapted tests were the Mini-Mental State Examination (MMSE) and the Montreal Cognitive Assessment (MOCA). Adaptations for hearing impairment involved deleting or creating written versions for hearing-dependent items. Adaptations for vision impairment involved deleting vision-dependent items or spoken/tactile versions of visual tasks. No study reported validity of the test in relation to detection of dementia in people with hearing/vision impairment. Item deletion had a negative impact on the psychometric properties of the test. While attempts have been made to adapt cognitive tests for people with acquired hearing and/or vision impairment, the primary limitation of these adaptations is that their validity in accurately detecting dementia among those with acquired hearing or vision impairment is yet to be established. It is likely that the sensitivity and specificity of the adapted versions are poorer than the original, especially if the adaptation involved item deletion. One solution would involve item substitution in an alternative sensory modality followed by re-validation of the adapted test.

Childhood Disintegrative Disorder as a Complication of Chicken Pox.

PubMed

Verma, Jitendra Kumar; Mohapatra, Satyakam

2016-01-01

Childhood disintegrative disorder (CDD) is characterized by late onset (>3 years of age) of developmental delays in language, social function and motor skills. Commonly there is no antecedent physical disorder leading to childhood disintegrative disorder. The present case report describes a child who developed childhood disintegrative disorder at the age of 6 years after an episode of chicken pox.

[Delinquency by a juvenile with pervasive developmental disorder: a case in which interaction between disorder and family dysfunction played a key role].

PubMed

Kurumatani, Takahiro; Yamashita, Mahoko

2003-01-01

Pervasive Developmental Disorder (PDD) is characterized by peculiar psycho-behavioral patterns. Youths with PDD who commit delinquent acts often exhibit strange behavior beyond ordinary comprehension. In order to fully understand and identify PDD it is important to take a comprehensive psychosocial history. Here we report on a 19-year-old delinquent male from a juvenile detention home who was exhibiting PDD. Both the psycho-behavioral patterns of the subject and the dysfunctional family background were key factors in this case. The subject persistently stalked and harassed an elementary school girl despite repeated intervention by police and the girl's parents to stop doing so. This behavior culminated in the subject being arrested and put on probation. While on probation his behavior continued, leading to his arrest and confinement to a correctional facility for juveniles with medical needs. Several features that included sustained impairment in social interaction and repetitive patterns of behavior that are often identified in delinquents with PDD were found to exist in the subject and he was diagnosed with PDD (probably Asperger's disease). The subject presented with a superficial understanding of the consequences his behavior had on others and for himself. Also, impaired family function was found to be a major contributing factor to his delinquency. Together theses factors hindered the subject from acquiring appropriate social skills. The major responsibility for providing care and support of an individual with PDD is within the family. Failure of the family to undertake such responsibilities can lead to ineffective treatment even after a specialist has identified the disorder. Given the slow decline of the nuclear family in Japan, it is important to understand the role of the family in caring for a child with PDD. Also, public recognition of PDD and social support for individuals with the disorder is important.

Patterns of anxiety and personality disorder comorbidity.

PubMed

Skodol, A E; Oldham, J M; Hyler, S E; Stein, D J; Hollander, E; Gallaher, P E; Lopez, A E

1995-01-01

The purpose of this study was to examine patterns of comorbidity of DSM-III-R anxiety disorders and personality disorders (PD). Two-hundred subjects were independently interviewed with the Structured Clinical Interview for DSM-III-R (SCID) and the Personality Disorder Examination (PDE) face-to-face by two experienced clinicians. One-hundred and forty-six also completed the Personality Diagnositc Questionnaire-Revised (PDQ-R). Rates of personality disorder among patients with and without anxiety disorders were determined by each of the three instruments. Comorbidity between panic disorder social phobia, obsessive-compulsive disorder and simple phobia and a conservative estimate of individual Axis II disorders was examined. Results indicate that panic disorder, either current or lifetime, is associated with borderline, avoidant, and dependent personality disorders: social phobia is associated with avoidant personality disorder, and obsessive-compulsive disorder is associated with obsessive-compulsive and avoidant personality disorders. Anxiety disorders with personality disorders are characterized by chronicity and lower levels of functioning compared with anxiety disorders without personality disorders.

"Complex" Posttraumatic Stress Disorder/Disorders of Extreme Stress (CP/DES) in Sexually Abused Children: An Exloratory Study.

ERIC Educational Resources Information Center

Hall, Darlene Kordich

1999-01-01

Compares three groups of young sexually abused children on seven "Complex" Posttraumatic Stress Disorder/Disorders of Extreme Stress (CP/DES) indices. As cumulative number of types of trauma increased, the number of CP/DES symptoms rose. Results suggest that CP/DES also characterizes sexually abused children, especially those who have…

[PECULIARITIES OF THE ANALYSIS OF THE LEVEL OF PROINFLAMMATORY CYTOKINS IN THE COMMUNITY-ACQUIRED PNEUMONIA IN CHILDREN].

PubMed

Akhaeyva, A; Zhupenova, D; Kenzhetaeva, T; Kysabekova, A; Dzhabaeva, S

2017-11-01

The high specific gravity in the structure of morbidity in children of all age groups, complicated course, determines the urgency of studying the clinical and diagnostic aspects of community-acquired pneumonia. In recent years, interest has been growing in the study of the child's cytokine status. A number of studies indicate that cytokines regulate the severity and duration of the inflammatory process. In this regard, the study of the possibility of determining the level of proinflammatory cytokines (IL-6 , TNF-α) is of great practical importance for assessing the prognosis of community-acquired pneumonia in children. In a prospective cohort study, 90 children with community-acquired pneumonia aged between 5 and 14 years were treated under treatment in the department respiratory of the Children›s Hospital in Karaganda, of which 47% were girls (95% CI 31.51% - 56.33%) and boys 53% (CI 95% 34.91% - 59.88%). The control group included 20 healthy children. Analysis of the results of the study revealed an increase in the content of proinflammatory cytokines in the blood serum and urine on children with community-acquired pneumonia depending on the severity of the course. At the same time, the equivalence of the cytokine trends in serum and urine determines the possibility of noninvasive detection of cytokines, both for characterizing the inflammatory response of the organism as such and for predicting the development of community-acquired pneumonia, which is especially valuable in pediatric practice.

Heart Rate and Treatment Effect in Children with Disruptive Behavior Disorders

ERIC Educational Resources Information Center

Stadler, Christina; Grasmann, Dorte; Fegert, Jorg M.; Holtmann, Martin; Poustka, Fritz; Schmeck, Klaus

2008-01-01

Objective: To examine whether children with disruptive behavior disorders (DBDs; hyperkinetic conduct disorder, conduct disorder, hyperkinetic disorder) characterized by low heart rate profit less from an intensive cognitive behavioral intervention aimed at reducing impulsive, oppositional and aggressive behavior problems. Method: Basal heart rate…

Characterization of a novel cation transporter ATPase gene (ATP13A4) interrupted by 3q25-q29 inversion in an individual with language delay.

PubMed

Kwasnicka-Crawford, Dorota A; Carson, Andrew R; Roberts, Wendy; Summers, Anne M; Rehnström, Karola; Järvelä, Irma; Scherer, Stephen W

2005-08-01

Specific language impairment (SLI) is defined as failure to acquire normal language skills despite adequate intelligence and environmental stimulation. Although SLI disorders are often heritable, the genetic basis is likely to involve a number of risk factors. This study describes a 7-year-old girl carrying an inherited paracentric inversion of the long arm of chromosome 3 [46XX, inv(3)(q25.32-q29)] having clinically defined expressive and receptive language delay. Fluorescence in situ hybridization (FISH) with locus-specific bacterial artificial chromosome clones (BACs) as probes was used to characterize the inverted chromosome 3. The proximal and distal inversion breakpoint was found to reside between markers D3S3692/D3S1553 and D3S3590/D3S2305, respectively. ATP13A4, a novel gene coding for a cation-transporting P-type ATPase, was found to be disrupted by the distal breakpoint. The ATP13A4 gene was shown to comprise a 3591-bp transcript encompassing 30 exons spanning 152 kb of the genomic DNA. This study discusses the characterization of ATP13A4 and its possible involvement in speech-language disorder.

[Modern documentary research on disease menu of acupuncture-moxibustion for mental and behavioral disorder].

PubMed

Hu, You-Ping; Chen, Yong; Xing, Lin; Niu, Bai-Lu; Zhu, Feng-Juan; Han, Jing; Wang, Yu; Bian, Wei; Liu, Cong-Sheng; Wei, Li; Du, Yuan-Hao

2011-10-01

Dominant disease menu of mental and behavioral disorder of acupuncture therapy was summarized and obtained in this article. Literatures on clinical treatment of mental and behavioral disorder with acupuncture were picked up from CBMdisc and CNKI during 1978 to 2007. Types of mental and behavioral disorder and report frequency of each disease treated with acupuncture were counted. And dominant diseases which were favorable to be treated with acupuncture were acquired through analysis and inductive method. Twenty-nine diseases of mental and behavioral disorder are favorable to be treated with acupuncture which were mentioned in totally 1967 related documents. It is found that the number of reports of sleep disorder, depression, hysteria aphronesia, dementia and sexual disorder are ranked as the top five. It is concluded that the preponderant diseases of mental and behavioral disorder treated by acupuncture are dementia, withdrawal syndrome, mental retardation, obsessive-compulsive disorder, sleep disorder, gastrointestinal neurosis (gastrointestinal disorders), depression, alcoholism and globus hystericus.

Myelofibrosis and acquired hemophilia A: a case report.

PubMed

Wrobel, Marie; Comio, Emilie; Gay, Valerie; Baroudi, Noureddine; Meyer, Pascal; Chuniaud-Louche, Christine; Hacini, Maya; Pica, Gian Matteo

2016-05-07

Myelofibrosis and acquired hemophilia A is a rare association. To the best of our knowledge only one case of myelofibrosis and acquired hemophilia A has been previously described. A 66-year-old Caucasian man diagnosed with myelofibrosis evolving in acute myeloid leukemia was referred to us for postoperative bleeding. Hemostatic studies showed prolonged activated partial thromboplastin time, decreased factor VIII coagulation, and a high factor VIII inhibitor titer; these findings led to a diagnosis of acquired hemophilia A for which he was treated with methylprednisolone and recombinant activated factor VII on admission. Due to a lack of response he was subsequently treated with rituximab combined with activated prothrombin complex concentrates. Furthermore, he received azacytidine to treat the underlying hematological malignancies. Immunosuppressive rituximab therapy resolved acquired hemophilia A with marked efficacy. Rapid and accurate diagnosis, effective hemostatic therapy, and timely treatment for underlying disease are important in the management of acquired hemophilia A secondary to hematological malignancy.

Emotional Stimuli and Motor Conversion Disorder

ERIC Educational Resources Information Center

Voon, Valerie; Brezing, Christina; Gallea, Cecile; Ameli, Rezvan; Roelofs, Karin; LaFrance, W. Curt, Jr.; Hallett, Mark

2010-01-01

Conversion disorder is characterized by neurological signs and symptoms related to an underlying psychological issue. Amygdala activity to affective stimuli is well characterized in healthy volunteers with greater amygdala activity to both negative and positive stimuli relative to neutral stimuli, and greater activity to negative relative to…

Seasonal Affective Disorder

PubMed Central

Rohan, Kelly J.

2005-01-01

Seasonal affective disorder (SAD), characterized by fall/winter major depression with spring/summer remission, is a prevalent mental health problem. SAD etiology is not certain, but available models focus on neurotransmitters, hormones, circadian rhythm dysregulation, genetic polymorphisms, and psychological factors. Light therapy is established as the best available treatment for SAD. Alternative and/or supplementary approaches involving medications, cognitive-behavioral therapy, and exercise are currently being developed and evaluated. Given the complexity of the disorder, interdisciplinary research stands to make a significant contribution to advancing our understanding of SAD conceptualization and treatment. PMID:21179639

Prediction and diagnosis of apple fruit physiological disorders

USDA-ARS?s Scientific Manuscript database

Apple postharvest physiological disorders, characterized by peel or flesh necrosis, result in significant yearly financial losses in commercial operations. Stakeholders have identified the need for effective, consistent control measures for apple postharvest physiological disorders and the developme...

Epilepsy: A Spectrum Disorder

PubMed Central

Sirven, Joseph I.

2015-01-01

Epilepsy, a disorder of unprovoked seizures is a multifaceted disease affecting individuals of all ages with a particular predilection for the very young and old. In addition to seizures, many patients often report cognitive and psychiatric problems associated with both the seizures themselves and its therapy. Epilepsy has numerous etiologies both idiopathic and acquired with a wide range of therapeutic responses. Despite numerous treatments available to control repetitive seizures including medications, diets, immunotherapy, surgery, and neuromodulatory devices, a large percentage of patients continue to suffer the consequences of uncontrolled seizures, which include psychosocial stigma and death. PMID:26328931

Elevated expression of CX3C chemokine receptor 1 mediates recruitment of T cells into bone marrow of patients with acquired aplastic anaemia.

PubMed

Ren, J; Hou, X Y; Ma, S H; Zhang, F K; Zhen, J H; Sun, L; Sun, Y X; Hao, Y L; Cheng, Y F; Hou, M; Xu, C G; Zhang, M H; Peng, J

2014-11-01

Acquired aplastic anaemia (AA) is a T-cell-mediated, organ-specific autoimmune disease characterized by haematopoietic stem cell destruction in the bone marrow. The exact molecular mechanism of T-cell trafficking into the bone marrow is unclear in AA. Very late activation antigen-4 (VLA-4) and CX3C chemokine receptor 1 (CX3CR1) play active roles in many autoimmune diseases. Therefore, we investigated whether VLA-4 and CX3CR1 also contribute to T-cell migration into the bone marrow in acquired AA. Expression levels of CX3CR1 and VLA-4 and their ligands [fractalkine (CX3CL1) and vascular cell adhesion molecule-1 (VCAM-1)] were examined in 63 patients with AA and 21 healthy control subjects. T-cell chemotaxis and adhesion were analysed in 17 patients with severe AA. We also prospectively evaluated the expression pattern of CX3CR1 during treatment with antithymocyte globulin plus cyclosporine in 11 patients with severe AA. The proportion of peripheral and bone marrow CD4(+) and CD8(+) T cells expressing CX3CR1 and the level of CX3CL1 was increased in patients with AA. However, there was no significant difference in VLA-4 expression or VCAM-1 levels. Functional studies demonstrated that chemotaxis towards autologous bone marrow plasma or soluble CX3CL1 was significantly higher in T cells from AA patients and could be blocked by CX3CR1 inhibitors. CX3CR1-mediated T-cell adhesion was also upregulated in these patients. The expression of CX3CR1 was associated with the efficacy of immunosuppressive therapy. The present findings demonstrate that CX3CR1 plays a pivotal role in recruitment of T cells into the bone marrow in acquired AA and is a potential therapeutic target for treatment of this disorder. © 2014 The Association for the Publication of the Journal of Internal Medicine.

Immunochip analysis identifies novel susceptibility loci in the human leukocyte antigen region for acquired thrombotic thrombocytopenic purpura.

PubMed

Mancini, I; Ricaño-Ponce, I; Pappalardo, E; Cairo, A; Gorski, M M; Casoli, G; Ferrari, B; Alberti, M; Mikovic, D; Noris, M; Wijmenga, C; Peyvandi, F

2016-12-01

Essentials Genetic predisposition to acquired thrombotic thrombocytopenic purpura (aTTP) is mainly unknown. Genetic risk factors for aTTP were studied by Immunochip analysis and replication study. Human leukocyte antigen (HLA) variant rs6903608 conferred a 2.5-fold higher risk of developing aTTP. rs6903608 and HLA-DQB1*05:03 may explain most of the HLA association signal in aTTP. Click to hear Dr Cataland's presentation on acquired thrombotic thrombocytopenic purpura SUMMARY: Background Acquired thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy associated with the development of autoantibodies against the von Willebrand factor-cleaving protease ADAMTS-13. Similarly to what has been found for other autoimmune disorders, there is evidence of a genetic contribution, including the association of the human leukocyte antigen (HLA) class II complex with disease risk. Objective To identify novel genetic risk factors in acquired TTP. Patients/Methods We undertook a case-control genetic association study in 190 European-origin TTP patients and 1255 Italian healthy controls by using the Illumina Immunochip. Replication analysis in 88 Italian cases and 456 controls was performed with single-nucleotide polymorphism (SNP) TaqMan assays. Results and conclusion We identified one common variant (rs6903608) located within the HLA class II locus that was independently associated with acquired TTP at genome-wide significance and conferred a 2.6-fold increased risk of developing a TTP episode (95% confidence interval [CI] 2.02-3.27, P = 1.64 × 10 -14 ). We also found five non-HLA variants mapping to chromosomes 2, 6, 8 and X that were suggestively associated with the disease: rs9490550, rs115265285, rs5927472, rs7823314, and rs1334768 (nominal P-values ranging from 1.59 × 10 -5 to 7.60 × 10 -5 ). Replication analysis confirmed the association of HLA variant rs6903608 with acquired TTP (pooled P = 3.95 × 10 -19 ). Imputation of classic

14 CFR 1274.402 - Contractor acquired property.

Code of Federal Regulations, 2010 CFR

2010-01-01

... 14 Aeronautics and Space 5 2010-01-01 2010-01-01 false Contractor acquired property. 1274.402 Section 1274.402 Aeronautics and Space NATIONAL AERONAUTICS AND SPACE ADMINISTRATION COOPERATIVE AGREEMENTS WITH COMMERCIAL FIRMS Property § 1274.402 Contractor acquired property. As provided in § 1274.923...

New Described Dermatological Disorders

PubMed Central

Cevirgen Cemil, Bengu; Keseroglu, Havva Ozge; Kaya Akis, Havva

2014-01-01

Many advances in dermatology have been made in recent years. In the present review article, newly described disorders from the last six years are presented in detail. We divided these reports into different sections, including syndromes, autoinflammatory diseases, tumors, and unclassified disease. Syndromes included are “circumferential skin creases Kunze type” and “unusual type of pachyonychia congenita or a new syndrome”; autoinflammatory diseases include “chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome,” “pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH) syndrome,” and “pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PAPASH) syndrome”; tumors include “acquired reactive digital fibroma,” “onychocytic matricoma and onychocytic carcinoma,” “infundibulocystic nail bed squamous cell carcinoma,” and “acral histiocytic nodules”; unclassified disorders include “saurian papulosis,” “symmetrical acrokeratoderma,” “confetti-like macular atrophy,” and “skin spicules,” “erythema papulosa semicircularis recidivans.” PMID:25243162

Imagery Rescripting in Posttraumatic Stress Disorder

ERIC Educational Resources Information Center

Hackmann, Anne

2011-01-01

This article provides an overview of methods of working with imagery to change meanings and ameliorate posttraumatic stress disorder (PTSD). It opens with a description of phenomenology in this disorder, usually characterized by a small number of recurrent images of the trauma, each representing a moment that warned of a threat to the physical or…

Extreme Cognitions in Bipolar Spectrum Disorders: Associations with Personality Disorder Characteristics and Risk for Episode Recurrence

PubMed Central

Stange, Jonathan P.; Adams, Ashleigh Molz; O'Garro-Moore, Jared K.; Weiss, Rachel B.; Ong, Mian-Li; Walshaw, Patricia D.; Abramson, Lyn Y.; Alloy, Lauren B.

2014-01-01

Bipolar spectrum disorders (BSDs) are often characterized by cognitive inflexibility and affective extremities, including “extreme” or polarized thoughts and beliefs, which have been shown to predict a more severe course of illness. However, little research has evaluated factors that may be associated with extreme cognitions, such as personality disorders, which are often characterized by extreme, inflexible beliefs and also are associated with poor illness course in BSDs. The present study evaluated associations between BSDs, personality disorder characteristics, and extreme cognitions (polarized responses made on measures of attributional style and dysfunctional attitudes), as well as links between extreme cognitions and the occurrence of mood episodes, among euthymic young adults with BSDs (n = 83) and demographically-matched healthy controls (n = 89) followed prospectively for three years. The relationship between personality disorder characteristics and negative and positive extreme cognitions was stronger among BSD participants than among healthy controls, even after statistically accounting for general cognitive styles. Furthermore, extreme negative cognitions predicted the prospective onset of major depressive and hypomanic episodes. These results suggest that extreme cognitive styles are most common in individuals with BSDs and personality disorder characteristics, and they provide further evidence that extreme negative cognitions may confer risk for mood dysregulation. PMID:25645172

[Megaesophagus in the dog and cat].

PubMed

Mace, S; Shelton, G D; Eddlestone, S

2013-01-01

Megaesophagus is a disorder of the esophagus characterized by diffuse dilation and decreased peristalsis. It is classified into congenital and acquired forms. Gastrointestinal, endocrine, immune-mediated, neuromuscular, paraneoplastic, and toxic disorders have been associated with acquired megaesophagus. Common clinical signs of megaesophagus are regurgitation, weight loss, coughing, and halitosis. Most cases of megaesophagus can be diagnosed using thoracic radiography; however, diagnosing the underlying cause requires a thorough history and additional diagnostics. The treatment, management, and prognosis of megaesophagus vary greatly depending on the underlying cause.

Hyperkinetic Impulse Disorder in Children's Behavior Problems

ERIC Educational Resources Information Center

Laufer, Maurice W.; Denhoff, Eric; Solomons, Gerald

2011-01-01

A very common cause of children's behavior disorder disturbance is an entity described as the hyperkinetic impulse disorder. This is characterized by hyperactivity, short attention span and poor powers of concentration, irritability, impulsiveness, variability, and poor schoolwork. The existence of this complexity may lead to many psychological…

Integrative Response Therapy for Binge Eating Disorder

ERIC Educational Resources Information Center

Robinson, Athena

2013-01-01

Binge eating disorder (BED), a chronic condition characterized by eating disorder psychopathology and physical and social disability, represents a significant public health problem. Guided self-help (GSH) treatments for BED appear promising and may be more readily disseminable to mental health care providers, accessible to patients, and…

Characterizing cognitive heterogeneity on the schizophrenia-bipolar disorder spectrum.

PubMed

Van Rheenen, T E; Lewandowski, K E; Tan, E J; Ospina, L H; Ongur, D; Neill, E; Gurvich, C; Pantelis, C; Malhotra, A K; Rossell, S L; Burdick, K E

2017-07-01

Current group-average analysis suggests quantitative but not qualitative cognitive differences between schizophrenia (SZ) and bipolar disorder (BD). There is increasing recognition that cognitive within-group heterogeneity exists in both disorders, but it remains unclear as to whether between-group comparisons of performance in cognitive subgroups emerging from within each of these nosological categories uphold group-average findings. We addressed this by identifying cognitive subgroups in large samples of SZ and BD patients independently, and comparing their cognitive profiles. The utility of a cross-diagnostic clustering approach to understanding cognitive heterogeneity in these patients was also explored. Hierarchical clustering analyses were conducted using cognitive data from 1541 participants (SZ n = 564, BD n = 402, healthy control n = 575). Three qualitatively and quantitatively similar clusters emerged within each clinical group: a severely impaired cluster, a mild-moderately impaired cluster and a relatively intact cognitive cluster. A cross-diagnostic clustering solution also resulted in three subgroups and was superior in reducing cognitive heterogeneity compared with disorder clustering independently. Quantitative SZ-BD cognitive differences commonly seen using group averages did not hold when cognitive heterogeneity was factored into our sample. Members of each corresponding subgroup, irrespective of diagnosis, might be manifesting the outcome of differences in shared cognitive risk factors.

Autoimmunity and dysmetabolism of human acquired immunodeficiency syndrome.

PubMed

Huang, Yan-Mei; Hong, Xue-Zhi; Xu, Jia-Hua; Luo, Jiang-Xi; Mo, Han-You; Zhao, Hai-Lu

2016-06-01

Acquired immunodeficiency syndrome (AIDS) remains ill-defined by lists of symptoms, infections, tumors, and disorders in metabolism and immunity. Low CD4 cell count, severe loss of body weight, pneumocystis pneumonia, and Kaposi's sarcoma are the major disease indicators. Lines of evidence indicate that patients living with AIDS have both immunodeficiency and autoimmunity. Immunodeficiency is attributed to deficits in the skin- and mucosa-defined innate immunity, CD4 T cells and regulatory T cells, presumably relating human immunodeficiency virus (HIV) infection. The autoimmunity in AIDS is evident by: (1) overproduction of autoantibodies, (2) impaired response of CD4 cells and CD8 cells, (3) failure of clinical trials of HIV vaccines, and (4) therapeutic benefits of immunosuppression following solid organ transplantation and bone marrow transplantation in patients at risk of AIDS. Autoantibodies are generated in response to antigens such as debris and molecules de novo released from dead cells, infectious agents, and catabolic events. Disturbances in metabolic homeostasis occur at the interface of immunodeficiency and autoimmunity in the development of AIDS. Optimal treatments favor therapeutics targeting on the regulation of metabolism to restore immune homeostasis.

Zebra Fish Lacking Adaptive Immunity Acquire an Antiviral Alert State Characterized by Upregulated Gene Expression of Apoptosis, Multigene Families, and Interferon-Related Genes

PubMed Central

García-Valtanen, Pablo; Martínez-López, Alicia; López-Muñoz, Azucena; Bello-Perez, Melissa; Medina-Gali, Regla M.; Ortega-Villaizán, María del Mar; Varela, Monica; Figueras, Antonio; Mulero, Víctoriano; Novoa, Beatriz; Estepa, Amparo; Coll, Julio

2017-01-01

To investigate fish innate immunity, we have conducted organ and cell immune-related transcriptomic as well as immunohistologic analysis in mutant zebra fish (Danio rerio) lacking adaptive immunity (rag1−/−) at different developmental stages (egg, larvae, and adult), before and after infection with spring viremia carp virus (SVCV). The results revealed that, compared to immunocompetent zebra fish (rag1+/+), rag1−/− acquired increased resistance to SVCV with age, correlating with elevated transcript levels of immune genes in skin/fins and lymphoid organs (head kidney and spleen). Gene sets corresponding to apoptotic functions, immune-related multigene families, and interferon-related genes were constitutively upregulated in uninfected adult rag1−/− zebra fish. Overexpression of activated CASPASE-3 in different tissues before and after infection with SVCV further confirmed increased apoptotic function in rag1−/− zebra fish. Concurrently, staining of different tissue samples with a pan-leukocyte antibody marker showed abundant leukocyte infiltrations in SVCV-infected rag1−/− fish, coinciding with increased transcript expression of genes related to NK-cells and macrophages, suggesting that these genes played a key role in the enhanced immune response of rag1−/− zebra fish to SVCV lethal infection. Overall, we present evidence that indicates that rag1−/− zebra fish acquire an antiviral alert state while they reach adulthood in the absence of adaptive immunity. This antiviral state was characterized by (i) a more rapid response to viral infection, which resulted in increased survival, (ii) the involvement of NK-cell- and macrophage-mediated transcript responses rather than B- and/or T-cell dependent cells, and (iii) enhanced apoptosis, described here for the first time, as well as the similar modulation of multigene family/interferon-related genes previously associated to fish that survived lethal viral infections. From this and other studies

Posttraumatic Stress Disorder Symptoms Among Navy Recruits

DTIC Science & Technology

2005-10-18

of Family Violence, Northern Illinois University, 2005. 25. Baldwin CY: The Effects of Witnessing Violence and Post Traumatic Stress Disorder on...Individuals who enter the military with pre-existing mental health problems such as posttraumatic stress disorder ( PTSD ) are likely to be at heightened risk... Disorders .1 PTSD arises in response to an identified traumatic experience and is characterized by three types of symptoms, all of which must be

High-Throughput Characterization of Intrinsic Disorder in Proteins from the Protein Structure Initiative

PubMed Central

Johnson, Derrick E.; Xue, Bin; Sickmeier, Megan D.; Meng, Jingwei; Cortese, Marc S.; Oldfield, Christopher J.; Le Gall, Tanguy; Dunker, A. Keith; Uversky, Vladimir N.

2012-01-01

The identification of intrinsically disordered proteins (IDPs) among the targets that fail to form satisfactory crystal structures in the Protein Structure Initiative represent a key to reducing the costs and time for determining three-dimensional structures of proteins. To help in this endeavor, several Protein Structure Initiative Centers were asked to send samples of both crystallizable proteins and proteins that failed to crystallize. The abundance of intrinsic disorder in these proteins was evaluated via computational analysis using Predictors of Natural Disordered Regions (PONDR®) and the potential cleavage sites and corresponding fragments were determined. Then, the target proteins were analyzed for intrinsic disorder by their resistance to limited proteolysis. The rates of tryptic digestion of sample target proteins were compared to those of lysozyme/myoglobin, apo-myoglobin and α-casein as standards of ordered, partially disordered and completely disordered proteins, respectively. At the next stage, the protein samples were subjected to both far-UV and near-UV circular dichroism (CD) analysis. For most of the samples, a good agreement between CD data, predictions of disorder and the rates of limited tryptic digestion was established. Further experimentation is being performed on a smaller subset of these samples in order to obtain more detailed information on the ordered/disordered nature of the proteins. PMID:22651963

Characteristics of patients with hospital-acquired influenza A (H1N1)pdm09 virus admitted to the intensive care unit.

PubMed

Álvarez-Lerma, F; Marín-Corral, J; Vilà, C; Masclans, J R; Loeches, I M; Barbadillo, S; González de Molina, F J; Rodríguez, A

2017-02-01

Influenza A (H1N1)pdm09 virus infection acquired in the hospital and in critically ill patients admitted to the intensive care unit (ICU) has been poorly characterized. To assess the clinical impact of hospital-acquired infection with influenza A (H1N1)pdm09 virus in critically ill patients. Analysis of a prospective database of the Spanish registry (2009-2015) of patients with severe influenza A admitted to the ICU. Infection was defined as hospital-acquired when diagnosis and starting of treatment occurred from the seventh day of hospital stay with no suspicion on hospital admission, and community-acquired when diagnosis was established within the first 48 h of admission. Of 2421 patients with influenza A (H1N1)pdm09 infection, 224 (9.3%) were classified as hospital-acquired and 1103 (45.6%) as community-acquired (remaining cases unclassified). Intra-ICU mortality was higher in the hospital-acquired group (32.9% vs 18.8%, P < 0.001). Independent factors associated with mortality were hospital-acquired influenza A (H1N1)pdm09 infection (odds ratio: 1.63; 95% confidence interval: 1.37-1.99), APACHE II score on ICU admission (1.09; 1.06-1.11), underlying haematological disease (3.19; 1.78-5.73), and need of extrarenal depuration techniques (4.20; 2.61-6.77) and mechanical ventilation (4.34; 2.62-7.21). Influenza A (H1N1)pdm09 infection acquired in the hospital is an independent factor for death in critically ill patients admitted to the ICU. Copyright © 2017 The Authors. Published by Elsevier Ltd.. All rights reserved.

Acquired Cystic Kidney Disease

MedlinePlus

... care provider about when to begin screening. Eating, Diet, and Nutrition No specific diet will prevent or delay acquired ... Weight Management Liver Disease Urologic Diseases Endocrine Diseases Diet & Nutrition Blood Diseases Diagnostic Tests La información de la ...

[Molecular characterization and antimicrobial susceptibility pattern of extended-spectrum β-lactamase-producing Escherichia coli as cause of community acquired urinary tract infection].

PubMed

Galindo-Méndez, Mario

Background Community acquired urinary tract infections (CaUTI) caused by strains of extended-spectrum β-lactamases (ESBL) - producing Escherichia coli, mainly by strains carrying the blaCTX-M-15 gene, is a growing phenomenon worldwide. Aim To determine the antibiotic susceptibility pattern of ESBL-producing E. coli as cause of CaUTI and to identify their molecular pattern. Methods A descriptive study was performed in the city of Oaxaca, Mexico, from where 288 strains of CaUTI-producing strains of E. coli in adults with possible UTI were isolated. The CLSI criteria was followed to determine the antimicrobial susceptibility patterns, and their molecular characterization was performed by using PCR. Results 31.3% of E. coli strains isolated in our population were ESBL producers, which presented higher levels of antibiotic resistance than those of non-producers of these enzymes. 95.6% of the studied strains were carriers of the blaCTX-M gene. Conclusions One-third of the Ca-UTI caused by E. coli in our population are caused by ESBL-producing strains, which present high levels of resistance to the antibiotics widely used in our community. This situation considerably decreases the number of antibiotics available for an empiric treatment against these infections.

Diagnosis and treatment of impulse control disorders in patients with movement disorders

PubMed Central

Mestre, Tiago A.; Strafella, Antonio P.; Thomsen, Teri; Voon, Valerie

2013-01-01

Impulse control disorders are a psychiatric condition characterized by the failure to resist an impulsive act or behavior that may be harmful to self or others. In movement disorders, impulse control disorders are associated with dopaminergic treatment, notably dopamine agonists (DAs). Impulse control disorders have been studied extensively in Parkinson’s disease, but are also recognized in restless leg syndrome and atypical Parkinsonian syndromes. Epidemiological studies suggest younger age, male sex, greater novelty seeking, impulsivity, depression and premorbid impulse control disorders as the most consistent risk factors. Such patients may warrant special monitoring after starting treatment with a DA. Various individual screening tools are available for people without Parkinson’s disease. The Questionnaire for Impulsive-Compulsive Disorders in Parkinson’s Disease has been developed specifically for Parkinson’s disease. The best treatment for impulse control disorders is prevention. However, after the development of impulse control disorders, the mainstay intervention is to reduce or discontinue the offending anti-Parkinsonian medication. In refractory cases, other pharmacological interventions are available, including neuroleptics, antiepileptics, amantadine, antiandrogens, lithium and opioid antagonists. Unfortunately, their use is only supported by case reports, small case series or open-label clinical studies. Prospective, controlled studies are warranted. Ongoing investigations include naltrexone and nicotine. PMID:23634190

Diagnosis and treatment of impulse control disorders in patients with movement disorders.

PubMed

Mestre, Tiago A; Strafella, Antonio P; Thomsen, Teri; Voon, Valerie; Miyasaki, Janis

2013-05-01

Impulse control disorders are a psychiatric condition characterized by the failure to resist an impulsive act or behavior that may be harmful to self or others. In movement disorders, impulse control disorders are associated with dopaminergic treatment, notably dopamine agonists (DAs). Impulse control disorders have been studied extensively in Parkinson's disease, but are also recognized in restless leg syndrome and atypical Parkinsonian syndromes. Epidemiological studies suggest younger age, male sex, greater novelty seeking, impulsivity, depression and premorbid impulse control disorders as the most consistent risk factors. Such patients may warrant special monitoring after starting treatment with a DA. Various individual screening tools are available for people without Parkinson's disease. The Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease has been developed specifically for Parkinson's disease. The best treatment for impulse control disorders is prevention. However, after the development of impulse control disorders, the mainstay intervention is to reduce or discontinue the offending anti-Parkinsonian medication. In refractory cases, other pharmacological interventions are available, including neuroleptics, antiepileptics, amantadine, antiandrogens, lithium and opioid antagonists. Unfortunately, their use is only supported by case reports, small case series or open-label clinical studies. Prospective, controlled studies are warranted. Ongoing investigations include naltrexone and nicotine.

26 CFR 1.9002-6 - Acquiring corporation.

Code of Federal Regulations, 2012 CFR

2012-04-01

... 26 Internal Revenue 13 2012-04-01 2012-04-01 false Acquiring corporation. 1.9002-6 Section 1.9002... (CONTINUED) INCOME TAXES (CONTINUED) General Actuarial Valuations § 1.9002-6 Acquiring corporation. Section 5... a corporation by another corporation in a distribution or transfer described in section 381(a) of...

26 CFR 1.9002-6 - Acquiring corporation.

Code of Federal Regulations, 2014 CFR

2014-04-01

... 26 Internal Revenue 13 2014-04-01 2014-04-01 false Acquiring corporation. 1.9002-6 Section 1.9002... (CONTINUED) INCOME TAXES (CONTINUED) General Actuarial Valuations § 1.9002-6 Acquiring corporation. Section 5... a corporation by another corporation in a distribution or transfer described in section 381(a) of...

26 CFR 1.9002-6 - Acquiring corporation.

Code of Federal Regulations, 2013 CFR

2013-04-01

... 26 Internal Revenue 13 2013-04-01 2013-04-01 false Acquiring corporation. 1.9002-6 Section 1.9002... (CONTINUED) INCOME TAXES (CONTINUED) General Actuarial Valuations § 1.9002-6 Acquiring corporation. Section 5... a corporation by another corporation in a distribution or transfer described in section 381(a) of...

26 CFR 1.9002-6 - Acquiring corporation.

Code of Federal Regulations, 2011 CFR

2011-04-01

... 26 Internal Revenue 13 2011-04-01 2011-04-01 false Acquiring corporation. 1.9002-6 Section 1.9002... (CONTINUED) INCOME TAXES (CONTINUED) General Actuarial Valuations § 1.9002-6 Acquiring corporation. Section 5... a corporation by another corporation in a distribution or transfer described in section 381(a) of...

Specificity of interpersonal problems in generalized anxiety disorder versus other anxiety disorders and depression.

PubMed

Uhmann, Stefan; Beesdo-Baum, Katja; Becker, Eni S; Hoyer, Jürgen

2010-11-01

We examined the diagnostic specificity of interpersonal problems (IP) in generalized anxiety disorder (GAD). We expected generally higher interpersonal distress, and specifically higher levels of nonassertive, exploitable, overly nurturant, and intrusive behavior in n = 58 patients with Diagnostic and Statistical Manual of Mental Disorders, 4th Edition GAD compared with patients with post-traumatic stress disorder (n = 46), other anxiety disorders (n = 47), and unipolar depressive disorders (n = 47). IP were assessed with the Inventory of Interpersonal Problems. Specificity in the sense of heightened interpersonal distress for GAD was not supported in any of the aforementioned scales, neither for pure nor for comorbid GAD. This finding persisted after accounting for the degree of depressiveness (Beck Depression Inventory). GAD patients are rather not characterized by more self-ascribed IPs although they may worry more about interpersonal issues in general.

Landsat 8 on-orbit characterization and calibration system

USGS Publications Warehouse

Micijevic, Esad; Morfitt, Ron; Choate, Michael J.

2011-01-01

The Landsat Data Continuity Mission (LDCM) is planning to launch the Landsat 8 satellite in December 2012, which continues an uninterrupted record of consistently calibrated globally acquired multispectral images of the Earth started in 1972. The satellite will carry two imaging sensors: the Operational Land Imager (OLI) and the Thermal Infrared Sensor (TIRS). The OLI will provide visible, near-infrared and short-wave infrared data in nine spectral bands while the TIRS will acquire thermal infrared data in two bands. Both sensors have a pushbroom design and consequently, each has a large number of detectors to be characterized. Image and calibration data downlinked from the satellite will be processed by the U.S. Geological Survey (USGS) Earth Resources Observation and Science (EROS) Center using the Landsat 8 Image Assessment System (IAS), a component of the Ground System. In addition to extracting statistics from all Earth images acquired, the IAS will process and trend results from analysis of special calibration acquisitions, such as solar diffuser, lunar, shutter, night, lamp and blackbody data, and preselected calibration sites. The trended data will be systematically processed and analyzed, and calibration and characterization parameters will be updated using both automatic and customized manual tools. This paper describes the analysis tools and the system developed to monitor and characterize on-orbit performance and calibrate the Landsat 8 sensors and image data products.

Utilization of protein intrinsic disorder knowledge in structural proteomics

PubMed Central

Oldfield, Christopher J.; Xue, Bin; Van, Ya-Yue; Ulrich, Eldon L.; Markley, John L.; Dunker, A. Keith; Uversky, Vladimir N.

2014-01-01

Intrinsically disordered proteins (IDPs) and proteins with long disordered regions are highly abundant in various proteomes. Despite their lack of well-defined ordered structure, these proteins and regions are frequently involved in crucial biological processes. Although in recent years these proteins have attracted the attention of many researchers, IDPs represent a significant challenge for structural characterization since these proteins can impact many of the processes in the structure determination pipeline. Here we investigate the effects of IDPs on the structure determination process and the utility of disorder prediction in selecting and improving proteins for structural characterization. Examination of the extent of intrinsic disorder in existing crystal structures found that relatively few protein crystal structures contain extensive regions of intrinsic disorder. Although intrinsic disorder is not the only cause of crystallization failures and many structured proteins cannot be crystallized, filtering out highly disordered proteins from structure-determination target lists is still likely to be cost effective. Therefore it is desirable to avoid highly disordered proteins from structure-determination target lists and we show that disorder prediction can be applied effectively to enrich structure determination pipelines with proteins more likely to yield crystal structures. For structural investigation of specific proteins, disorder prediction can be used to improve targets for structure determination. Finally, a framework for considering intrinsic disorder in the structure determination pipeline is proposed. PMID:23232152

The alphabet of intrinsic disorder

PubMed Central

Theillet, Francois-Xavier; Kalmar, Lajos; Tompa, Peter; Han, Kyou-Hoon; Selenko, Philipp; Dunker, A. Keith; Daughdrill, Gary W.; Uversky, Vladimir N

2013-01-01

A significant fraction of every proteome is occupied by biologically active proteins that do not form unique three-dimensional structures. These intrinsically disordered proteins (IDPs) and IDP regions (IDPRs) have essential biological functions and are characterized by extensive structural plasticity. Such structural and functional behavior is encoded in the amino acid sequences of IDPs/IDPRs, which are enriched in disorder-promoting residues and depleted in order-promoting residues. In fact, amino acid residues can be arranged according to their disorder-promoting tendency to form an alphabet of intrinsic disorder that defines the structural complexity and diversity of IDPs/IDPRs. This review is the first in a series of publications dedicated to the roles that different amino acid residues play in defining the phenomenon of protein intrinsic disorder. We start with proline because data suggests that of the 20 common amino acid residues, this one is the most disorder-promoting. PMID:28516008

A systematic review of treatment intensity in speech disorders.

PubMed

Kaipa, Ramesh; Peterson, Abigail Marie

2016-12-01

Treatment intensity (sometimes referred to as "practice amount") has been well-investigated in learning non-speech tasks, but its role in treating speech disorders has not been largely analysed. This study reviewed the literature regarding treatment intensity in speech disorders. A systematic search was conducted in four databases using appropriate search terms. Seven articles from a total of 580 met the inclusion criteria. The speech disorders investigated included speech sound disorders, dysarthria, acquired apraxia of speech and childhood apraxia of speech. All seven studies were evaluated for their methodological quality, research phase and evidence level. Evidence level of reviewed studies ranged from moderate to strong. With regard to the research phase, only one study was considered to be phase III research, which corresponds to the controlled trial phase. The remaining studies were considered to be phase II research, which corresponds to the phase where magnitude of therapeutic effect is assessed. Results suggested that higher treatment intensity was favourable over lower treatment intensity of specific treatment technique(s) for treating childhood apraxia of speech and speech sound (phonological) disorders. Future research should incorporate randomised-controlled designs to establish optimal treatment intensity that is specific to each of the speech disorders.

Body dysmorphic disorder: history and curiosities.

PubMed

França, Katlein; Roccia, Maria Grazia; Castillo, David; ALHarbi, Mana; Tchernev, Georgi; Chokoeva, Anastasia; Lotti, Torello; Fioranelli, Massimo

2017-10-01

Body dysmorphic disorder is a chronic psychiatric disorder characterized by excessive preoccupation with an absent or minimal physical deformity. It causes severe distress and impairs normal functioning. In the last centuries, this disorder has been mentioned in the medical literature by important mental health practitioners by different names, such as "dysmorphophobia" or "dermatologic hypochondriasis". However, not until the last century was it included among the obsessive-compulsive disorders, although its classification has changed over time.Patients with body dysmorphic disorder constantly seek cosmetic treatments in order to improve their physical appearance, which more often deteriorates their mental condition. The high prevalence of psychiatric disorders in cosmetic medical practice has led in this field of study to the new science "cosmetic psychodermatology". This paper presents a summary of important facts about body dysmorphic disorder and its description throughout the history of medicine.

Mental Health Disorder Therapeutic Modalities Modified for the GMS.

PubMed

Sumneangsanor, Tipsuda; Vuthiarpa, Sararud; Somprasert, Chomchueun

2017-12-01

Mental health disorders can affect physical and psychological behaviors. The people of the Greater Mekong Subregion (GMS) have a high risk of mental health disorders, such as depression, stress, and substance abuse be-cause the people in this region are trafficked for forced sex work and various forms of forced labor. In these situations, vic-tims often endure violence and abuse from trafficking recruiters, employers, and other individuals. The purposes of this study were to identify the elements characterizing mental health disorders, especially in terms of depression, stress, and sub-stance abuse, and to identify the treatment modalities for mental health disorders in the GMS. The researcher undertook a comparative analysis of the literature, reviews of epidemiological studies and mental disorder therapies, and overviews of previous research studies, were used to generate a synthesis of the existing knowledge of the mental disorder therapeutic modalities. Regarding the search methods, the data from the electronic databases PubMed, PsycINFO, Dynamed and ScienceDirect were supplemented with a manual reference search covering relevant studies from 2005 to 2016. Thirty-one papers were included in the review of elements characterizing mental health disorders, especially in terms of depression, stress, and substance abuse, and to identify the treatment modalities for mental health disorders in the GMS. Nine papers defined characterizing mental health disorders, in terms of depression, stress, and substance abuse. Twenty-two papers showed the treatment modalities for mental health disorders that the treatment was effective, these in-cluded pharmacological treatments and psychological treatments, such as mindfulness-based cognitive therapy, biofeedback, and music therapy. Useful guidance can be provided for the prevention and treatment of mental health disorders, and for the care of people in the Greater Mekong Subregion. The finding of this review confirms the

Hydrogen-Exchange Mass Spectrometry for the Study of Intrinsic Disorder in Proteins

PubMed Central

Balasubramaniam, Deepa; Komives, Elizabeth A.

2012-01-01

Amide hydrogen/deuterium exchange detected by mass spectrometry (HXMS) is seeing wider use for the identification of intrinsically disordered parts of proteins. In this review, we discuss examples of how discovery of intrinsically disordered regions and their removal can aid in structure determination, biopharmaceutical quality control, the characterization of how posttranslational modifications affect weak structuring of disordered regions, the study of coupled folding and binding, and the characterization of amyloid formation. PMID:23099262

A Disorder Unique to Adolescence? The Kleine-Levin Syndrome.

ERIC Educational Resources Information Center

Cawthorn, Paul

1990-01-01

Describes Kleine-Levin syndrome, rare disorder characterized by excessive sleep and abnormal hunger. Notes that, in its pure form, disorder can only be diagnosed in adolescent males. Presents case study of 15-year-old male with disease. Presents evidence which suggests link between Kleine-Levin syndrome and cyclic affective disorders. (Author/ABL)

Mental Health Disorders. Adolescent Health Highlight. Publication #2013-1

ERIC Educational Resources Information Center

Murphey, David; Barry, Megan; Vaughn, Brigitte

2013-01-01

Mental disorders are diagnosable conditions characterized by changes in thinking, mood, or behavior (or some combination of these) that can cause a person to feel stressed out and impair his or her ability to function. These disorders are common in adolescence. This "Adolescent Health Highlight" presents the warning signs of mental disorders;…

[Peripheral nervous system and speech disorders].

PubMed

Ferri, Lluís

2014-02-24

Disorders affecting the lower motor neurons in childhood, with a congenital or acquired aetiology, give rise to difficulties in neuromotor response and, therefore, motor disorders affecting speech in a period that is especially critical for the development of language. The low incidence of this pathology, its comorbidity with other brain conditions and its uncertain prognosis make it a particularly interesting area of study. The purpose of this work is to review the motor disorders affecting speech in flaccid dysarthria, together with its functional evaluation and speech therapy interventions. The study aims to carry out the clinical characterisation of the disorders affecting verbal production of a peripheral origin, and more specifically flaccid dysarthria and its respiratory, phonatory, resonance, articulatory and prosodic manifestations. The analysis then goes on to outline the functional evaluation and lines of intervention for its treatment are proposed. The clinical manifestations of flaccid dysarthria are very heterogeneous and range from very slight difficulties in articulation to severe disorders that seriously limit the capacity for verbal expression. In most cases, a functional examination yields valuable findings for its identification and classification, for determining the need for complementary evaluations and for establishing the most suitable programme of speech therapy. The guided participation of the family and the interdisciplinary approach are factors that play a decisive role in improving these processes.

Biophysical characterization of the structural change of Nopp140, an intrinsically disordered protein, in the interaction with CK2α

DOE Office of Scientific and Technical Information (OSTI.GOV)

Na, Jung-Hyun; Biomedical Research Institute, Korea Institute of Science and Technology, Seoul 02792; Department of Chemistry and Nano Science, Ewha Womans University, Seoul 03760

2016-08-19

Nucleolar phosphoprotein 140 (Nopp140) is a nucleolar protein, more than 80% of which is disordered. Previous studies have shown that the C-terminal region of Nopp140 (residues 568–596) interacts with protein kinase CK2α, and inhibits the catalytic activity of CK2. Although the region of Nopp140 responsible for the interaction with CK2α was identified, the structural features and the effect of this interaction on the structure of Nopp140 have not been defined due to the difficulty of structural characterization of disordered protein. In this study, the disordered feature of Nopp140 and the effect of CK2α on the structure of Nopp140 were examinedmore » using single-molecule fluorescence resonance energy transfer (smFRET) and electron paramagnetic resonance (EPR). The interaction with CK2α was increased conformational rigidity of the CK2α-interacting region of Nopp140 (Nopp140C), suggesting that the disordered and flexible conformation of Nopp140C became more rigid conformation as it binds to CK2α. In addition, site specific spin labeling and EPR analysis confirmed that the residues 574–589 of Nopp140 are critical for binding to CK2α. Similar technical approaches can be applied to analyze the conformational changes in other IDPs during their interactions with binding partners. - Highlights: • Nopp140 is intrinsically disordered protein (IDP). • Conformation of Nopp140 became more rigid conformation due to interaction with CK2α. • smFRET and EPR could be applied to analyze the structural changes of IDPs.« less

26 CFR 1.9002-6 - Acquiring corporation.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 26 Internal Revenue 13 2010-04-01 2010-04-01 false Acquiring corporation. 1.9002-6 Section 1.9002... (CONTINUED) INCOME TAXES General Actuarial Valuations § 1.9002-6 Acquiring corporation. Section 5(d) of the... corporation by another corporation in a distribution or transfer described in section 381(a) of the Code the...

19 CFR 148.33 - Articles acquired abroad.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 19 Customs Duties 2 2010-04-01 2010-04-01 false Articles acquired abroad. 148.33 Section 148.33... Articles acquired abroad. (a) Exemption. Each returning resident is entitled to bring in free of duty and..., Harmonized Tariff Schedule of the United States (19 U.S.C. 1202), articles for his personal or household use...

19 CFR 148.33 - Articles acquired abroad.

Code of Federal Regulations, 2012 CFR

2012-04-01

... 19 Customs Duties 2 2012-04-01 2012-04-01 false Articles acquired abroad. 148.33 Section 148.33... Articles acquired abroad. (a) Exemption. Each returning resident is entitled to bring in free of duty and..., Harmonized Tariff Schedule of the United States (19 U.S.C. 1202), articles for his personal or household use...

19 CFR 148.33 - Articles acquired abroad.

Code of Federal Regulations, 2014 CFR

2014-04-01

... 19 Customs Duties 2 2014-04-01 2014-04-01 false Articles acquired abroad. 148.33 Section 148.33... Articles acquired abroad. (a) Exemption. Each returning resident is entitled to bring in free of duty and..., Harmonized Tariff Schedule of the United States (19 U.S.C. 1202), articles for his personal or household use...

19 CFR 148.33 - Articles acquired abroad.

Code of Federal Regulations, 2011 CFR

2011-04-01

... 19 Customs Duties 2 2011-04-01 2011-04-01 false Articles acquired abroad. 148.33 Section 148.33... Articles acquired abroad. (a) Exemption. Each returning resident is entitled to bring in free of duty and..., Harmonized Tariff Schedule of the United States (19 U.S.C. 1202), articles for his personal or household use...

19 CFR 148.33 - Articles acquired abroad.

Code of Federal Regulations, 2013 CFR

2013-04-01

... 19 Customs Duties 2 2013-04-01 2013-04-01 false Articles acquired abroad. 148.33 Section 148.33... Articles acquired abroad. (a) Exemption. Each returning resident is entitled to bring in free of duty and..., Harmonized Tariff Schedule of the United States (19 U.S.C. 1202), articles for his personal or household use...

Eating Disorders and Epigenetics.

PubMed

Thaler, Lea; Steiger, Howard

2017-01-01

Eating disorders (EDs) are characterized by intense preoccupation with shape and weight and maladaptive eating practices. The complex of symptoms that characterize EDs often arise through the activation of latent genetic potentials by environmental exposures, and epigenetic mechanisms are believed to link environmental exposures to gene expression. This chapter provides an overview of genetic factors acting in the etiology of EDs. It then provides a background to the hypothesis that epigenetic mechanisms link stresses such as obstetric complications and childhood abuse as well as effects of malnutrition to eating disorders (EDs). The chapter then summarizes the emerging body of literature on epigenetics and EDs-mainly studies on DNA methylation in samples of anorexia and bulimia. The available evidence base suggests that an epigenetically informed perspective contributes in valuable ways to the understanding of why people develop EDs.

Rehabilitation of Reading and Visual Exploration in Visual Field Disorders: Transfer or Specificity?

ERIC Educational Resources Information Center

Schuett, Susanne; Heywood, Charles A.; Kentridge, Robert W.; Dauner, Ruth; Zihl, Josef

2012-01-01

Reading and visual exploration impairments in unilateral homonymous visual field disorders are frequent and disabling consequences of acquired brain injury. Compensatory therapies have been developed, which allow patients to regain sufficient reading and visual exploration performance through systematic oculomotor training. However, it is still…

Myasthenia and related disorders of the neuromuscular junction.

PubMed

Spillane, Jennifer; Beeson, David J; Kullmann, Dimitri M

2010-08-01

Our understanding of transmission at the neuromuscular junction has increased greatly in recent years. We now recognise a wide variety of autoimmune and genetic diseases that affect this specialised synapse, causing muscle weakness and fatigue. These disorders greatly affect quality of life and rarely can be fatal. Myasthenia gravis is the most common disorder and is most commonly caused by autoantibodies targeting postsynaptic acetylcholine receptors. Antibodies to muscle-specific kinase (MuSK) are detected in a variable proportion of the remainder. Treatment is symptomatic and immunomodulatory. Lambert-Eaton myasthenic syndrome is caused by antibodies to presynaptic calcium channels, and approximately 50% of cases are paraneoplastic, most often related to small cell carcinoma of the lung. Botulism is an acquired disorder caused by neurotoxins produced by Clostridium botulinum, impairing acetylcholine release into the synaptic cleft. In addition, several rare congenital myasthenic syndromes have been identified, caused by inherited defects in presynaptic, synaptic basal lamina and postsynaptic proteins necessary for neuromuscular transmission. This review focuses on recent advances in the diagnosis and treatment of these disorders.

Premenstrual Dysphoric Disorder: Epidemiology and Treatment.

PubMed

Hantsoo, Liisa; Epperson, C Neill

2015-11-01

Recently designated as a disorder in the DSM-5, premenstrual dysphoric disorder (PMDD) presents an array of avenues for further research. PMDD's profile, characterized by cognitive-affective symptoms during the premenstruum, is unique from that of other affective disorders in its symptoms and cyclicity. Neurosteroids may be a key contributor to PMDD's clinical presentation and etiology, and represent a potential avenue for drug development. This review will present recent literature on potential contributors to PMDD's pathophysiology, including neurosteroids and stress, and explore potential treatment targets.

Premenstrual Dysphoric Disorder: Epidemiology and Treatment

PubMed Central

Hantsoo, Liisa; Epperson, C. Neill

2016-01-01

Recently designated as a disorder in the DSM-5, premenstrual dysphoric disorder (PMDD) presents an array of avenues for further research. PMDD's profile, characterized by cognitive–affective symptoms during the premenstruum, is unique from that of other affective disorders in its symptoms and cyclicity. Neurosteroids may be a key contributor to PMDD's clinical presentation and etiology, and represent a potential avenue for drug development. This review will present recent literature on potential contributors to PMDD's pathophysiology, including neurosteroids and stress, and explore potential treatment targets. PMID:26377947

A Primer on the Genetics of Comorbid Eating Disorders and Substance Use Disorders.

PubMed

Munn-Chernoff, Melissa A; Baker, Jessica H

2016-03-01

Eating disorders (EDs) and substance use disorders (SUDs) frequently co-occur; however, the reasons for this are unclear. We review the current literature on genetic risk for EDs and SUDs, as well as preliminary findings exploring whether these classes of disorders have overlapping genetic risk. Overall, genetic factors contribute to individual differences in liability to multiple EDs and SUDs. Although initial family studies concluded that no shared familial (which includes genetic) risk between EDs and SUDs exists, twin studies suggest a moderate proportion of shared variance is attributable to overlapping genetic factors, particularly for those EDs characterized by binge eating and/or inappropriate compensatory behaviours. No adoption or molecular genetic studies have examined shared genetic risk between these classes of disorders. Research investigating binge eating and inappropriate compensatory behaviours using emerging statistical genetic methods, as well as examining gene-environment interplay, will provide important clues into the aetiology of comorbid EDs and SUDs. Copyright © 2015 John Wiley & Sons, Ltd and Eating Disorders Association.

Questionnaires that screen for multiple sleep disorders.

PubMed

Klingman, Karen J; Jungquist, Carla R; Perlis, Michael L

2017-04-01

The goal of this review was to identify, describe, and evaluate the existing multiple sleep disorders screening questionnaires for their comprehensiveness, brevity, and psychometric quality. A systematic review was conducted using Medline/PubMed, cumulative index to nursing & allied health literature, health and psychosocial instruments and the "grey literature". Search terms were "sleep disorders, screening, questionnaires, and psychometrics". The scope of the search was limited to English language articles for adult age groups from 1989 through 2015. Of the n = 2812 articles identified, most were assessment or treatment guideline reviews, topical reviews, and/or empirical articles. Seven of the articles described multiple sleep disorders screening instruments. Of the identified instruments, two questionnaires (the Holland sleep Disorders questionnaire and sleep-50) were evaluated as comprehensive and one questionnaire (the global sleep assessment questionnaire [GSAQ]) was judged to be both comprehensive and efficient. The GSAQ was found to cover four of the six core intrinsic disorders, sleep insufficiency, and daytime sequela with 11 questions. Accordingly, the GSAQ is the most suitable for application as a general sleep disorders screener. Additional work is required to validate this instrument in the context of primary care. Finally, the future development of multiple sleep disorders screening questionnaires should not only cover all six intrinsic sleep disorders but also acquire some basic demographic information (age, sex, body mass index, presence/absence of bed partner, work status and shift) and some limited data regarding sleep sufficiency and the daytime consequences of sleep disturbance. Copyright © 2016 Elsevier Ltd. All rights reserved.

Attention Deficit Hyperactivity Disorder

PubMed Central

Matthews, Marguerite; Nigg, Joel T.

2014-01-01

Over the last two decades, there have been numerous technical and methodological advances available to clinicians and researchers to better understand attention deficit hyperactivity disorder (ADHD) and its etiology. Despite the growing body of literature investigating the disorder’s pathophysiology, ADHD remains a complex psychiatric disorder to characterize. This chapter will briefly review the literature on ADHD, with a focus on its history, the current genetic insights, neurophysiologic theories, and the use of neuroimaging to further understand the etiology. We address some of the major concerns that remain unclear about ADHD, including subtype instability, heterogeneity, and the underlying neural correlates that define the disorder. We highlight that the field of ADHD is rapidly evolving; the descriptions provided here will hopefully provide a sturdy foundation for which to build and improve our understanding of the disorder. PMID:24214656

Effectiveness of Modeling and Rehearsal to Teach Fire Safety Skills to Children with Autism

ERIC Educational Resources Information Center

Garcia, David

2013-01-01

Autism is a disorder that affects children at an alarming rate. One out of every 88 children is diagnosed with autism in the United States. The disorder is characterized by communication, social, and behavioral deficits. Children with autism often require specialized teaching methods to learn basic skills that most children acquire without…

19 CFR 148.38 - Sale of articles acquired abroad.

Code of Federal Regulations, 2011 CFR

2011-04-01

... 19 Customs Duties 2 2011-04-01 2011-04-01 false Sale of articles acquired abroad. 148.38 Section... § 148.38 Sale of articles acquired abroad. An article brought in under the $800 or $1,600 exemption for articles acquired abroad for personal or household use and subsequently sold is not dutiable or subject to...

19 CFR 148.38 - Sale of articles acquired abroad.

Code of Federal Regulations, 2012 CFR

2012-04-01

... 19 Customs Duties 2 2012-04-01 2012-04-01 false Sale of articles acquired abroad. 148.38 Section... § 148.38 Sale of articles acquired abroad. An article brought in under the $800 or $1,600 exemption for articles acquired abroad for personal or household use and subsequently sold is not dutiable or subject to...

19 CFR 148.38 - Sale of articles acquired abroad.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 19 Customs Duties 2 2010-04-01 2010-04-01 false Sale of articles acquired abroad. 148.38 Section... § 148.38 Sale of articles acquired abroad. An article brought in under the $800 or $1,600 exemption for articles acquired abroad for personal or household use and subsequently sold is not dutiable or subject to...

19 CFR 148.38 - Sale of articles acquired abroad.

Code of Federal Regulations, 2013 CFR

2013-04-01

... 19 Customs Duties 2 2013-04-01 2013-04-01 false Sale of articles acquired abroad. 148.38 Section... § 148.38 Sale of articles acquired abroad. An article brought in under the $800 or $1,600 exemption for articles acquired abroad for personal or household use and subsequently sold is not dutiable or subject to...

Clinical features of dog- and bat-acquired rabies in humans.

PubMed

Udow, Sean J; Marrie, Ruth Ann; Jackson, Alan C

2013-09-01

Clinical differences in rabies due to canine and bat rabies virus variants have been noted, but no detailed studies have been reported to support these observations. Using the Morbidity and Mortality Weekly Report and PubMed, we identified 142 case reports of rabies from North America, South America, Europe, Africa, and Asia. We systematically abstracted 126 selected data elements and compared clinical features and investigation results in dog- and bat-acquired cases of rabies. Survivors and cases acquired from aerosolized viral exposure or tissue/organ transplant were excluded (n = 20). Of 122 cases, 49 (40.2%) were dog-acquired and 54 (44.3%) were bat-acquired. Bat-acquired cases of rabies were more often misdiagnosed and lacked a bite history. Encephalopathy, hydrophobia, and aerophobia were more common in dog-acquired rabies. Abnormal cranial nerve, motor, and sensory examinations, tremor, myoclonus, local sensory symptoms, symptoms at the exposure site, and local symptoms in the absence of a bite or scratch were more common in patients with bat-acquired rabies, as was increased cerebrospinal fluid protein (P = .031). Patients with paralytic rabies had longer survival times than those with encephalitic rabies, and also had shorter incubation periods if they had received postexposure prophylaxis. Clinical differences in dog- and bat-acquired rabies may reflect differences in the route of viral spread of rabies virus variants in the nervous system, although certain variants could cause more severe dysfunction in neuronal subpopulations. Recognition that bat-acquired rabies may present with different clinical manifestations than dog-acquired rabies may help improve the early diagnosis of rabies.

[Rhythm disorders and cardiac crypto-malformations].

PubMed

Davy, J M; Raczka, F; Cung, T T; Combes, N; Bortone, A; Gaty, D

2005-12-01

Faced with a cardiac arrhythmia occuring in an apparently healthy heart, it is necessary to perform an anatomical investigation to detect any unsuspected anomalies. Congenital cardiopathy must certainly be excluded, as this is often responsible for rhythm disorders and/or cardiac conduction defects. Similarly, any acquired conditions, cardiomyopathy, or cardiac tumour must be sought. However, the possibility should always be considered of a minimal congenital malformation, which could be repsonsible for: any type of cardiac arrhythmia: rhythm disorder or conduction defect at the atrial, junctional or ventricular level, with a benign or serious prognosis. Unexpected therapeutic difficulties during radiofrequency ablation procedures or at implantation of pacemakers or defibrillators. Together with rhythm studies, the investigation of choice is high quality imaging, either the classic left or right angiography or the more modern cardiac CT or intracardiac mapping.

High prevalence of personality disorders among circadian rhythm sleep disorders (CRSD) patients.

PubMed

Dagan, Y; Sela, H; Omer, H; Hallis, D; Dar, R

1996-10-01

The purpose of this study was to examine systematically our previous clinical impression regarding the prevalence of personality disorders in patients suffering from circadian rhythm sleep disorders (CRSD). We hypothesized that, in a group of patients suffering from CRSD, there would be a higher frequency of personality disorders than in a group of healthy controls. The experimental group consisted of CRSD patients diagnosed according to a clinical interview and actigraphic recordings. The control group consisted of healthy volunteers in whom CRSD had been ruled out by means of a self-administered questionnaire. Both groups were assessed for personality disorders using the MCMI, a diagnostic tool based on Millon's biopsychosocial theory of personality and the PRQ-R, a diagnostic tool based on the DMS-III-R. Both tests provided clear and significant support for the hypothesis that individuals suffering from CRSD are characterized to a greater extent by personality disorders than a control group. No specific characteristic pattern or profile of personality disorders was clearly detected. Correct early diagnosis and treatment of CRSD may improve afflicted individuals' adaptive capabilities and perhaps even prevent the development of a personality disorder. This suggests how important a greater awareness of CRSD on the part of the professional community may be.

Non-speech oro-motor exercise use in acquired dysarthria management: regimes and rationales.

PubMed

Mackenzie, Catherine; Muir, Margaret; Allen, Carolyn

2010-01-01

Non-speech oro-motor exercises (NSOMExs) are described in speech and language therapy manuals and are thought to be much used in acquired dysarthria intervention, though there is no robust evidence of an influence on speech outcome. Opinions differ as to whether, and for which dysarthria presentations, NSOMExs are appropriate. The investigation sought to collect development-phase data, in accordance with the Medical Research Council (MRC) evaluation of complex interventions. The aims were to establish the extent of use of NSOMExs in acquired disorders, the exercise regimes in use for dysarthria, with which dysarthric populations, and the anticipated clinical outcomes. A further aim was to determine the influencing rationales where NSOMExs were or were not used in dysarthria intervention. Speech and language therapists throughout Scotland, Wales, and Northern Ireland, working with adult-acquired dysarthria, were identified by their service heads. They received postal questionnaires comprising 21 closed and two open questions, covering respondent biographics, use of NSOMExs, anticipated clinical outcomes, and practice influencing rationales. One hundred and ninety-one (56% response) completed questionnaires were returned. Eighty-one per cent of respondents used NSOMExs in dysarthria. There was no association with years of speech and language therapy experience. Those who used and those who did not use NSOMExs provided similar influencing rationales, including evidence from their own practice, and Higher Education Institute teaching. More experienced speech and language therapists were more likely than those more recently qualified to be guided by results from their own practice. Input from the attended Higher Education Institute was more influential for those less experienced than for those more experienced. Clinical outcome aims were not confined to speech, but also included improvements in movement, sensory awareness, appearance, emotional status, dysphagia and

Preschoolers acquire general knowledge by sharing in pretense.

PubMed

Sutherland, Shelbie L; Friedman, Ori

2012-01-01

Children acquire general knowledge about many kinds of things, but there are few known means by which this knowledge is acquired. In this article, it is proposed that children acquire generic knowledge by sharing in pretend play. In Experiment 1, twenty-two 3- to 4-year-olds watched pretense in which a puppet represented a "nerp" (an unfamiliar kind of animal). For instance, in one scenario, the nerp ate and disliked a carrot. When subsequently asked generic questions about real nerps, children's responses suggested that they had learned general facts (e.g., nerps dislike carrots). In Experiment 2, thirty-two 4- to 5-year-olds learned from scenarios lacking pretend speech or sound effects. The findings reveal a long overlooked means by which children can acquire generic knowledge. © 2012 The Authors. Child Development © 2012 Society for Research in Child Development, Inc.

Immunophenotypic characterization of bone marrow mast cells in mastocytosis and other mast cell disorders.

PubMed

Sánchez-Muñoz, Laura; Teodósio, Cristina; Morgado, José M; Escribano, Luis

2011-01-01

Mastocytosis is a term used to designate a heterogeneous group of disorders characterized by an abnormal proliferation and accumulation of mast cells (MCs) in one or multiple tissues including skin, bone marrow (BM), liver, spleen, and lymph nodes, among others. Recent advances in our understanding of mast cell biology and disease resulted in the identification of important differences in the expression of mast cell surface antigens between normal and neoplastic mast cells. Most notably, detection of aberrant expression of CD25 and CD2 on the surface of neoplastic mast cells but not on their normal counterparts lead to the inclusion of this immunophenotypic abnormality in the World Health Organization diagnostic criteria for systemic mastocytosis. Aberrant mast cell surface marker expression can be detected in the bone marrow aspirate by flow cytometry, even in patients lacking histopathologically detectable aggregates of mast cells in bone marrow biopsy sections. These aberrant immunophenotypic features are of great relevance for the assessment of tissue involvement in mastocytosis with consequences in the diagnosis, classification, and follow-up of the disease and in its differential diagnosis with other entities. In this chapter, we provide the reader with information for the objective and reproducible identification of pathologic MCs by using quantitative multiparametric flow cytometry, for their phenotypic characterization, and the criteria currently used for correct interpretation of the immunophenotypic results obtained. Copyright © 2011 Elsevier Inc. All rights reserved.

[Perioperative disorders of mental functions].

PubMed

Tonković, Dinko; Adam, Visnja Nesek; Kovacević, Marko; Bogović, Tajana Zah; Drvar, Zeljko; Baronica, Robert

2012-03-01

Mental disorders are characterized by disturbances of thought, perception, affect and behavior, which occur as a result of brain damage. Recognizing and treating these conditions is necessary not only for psychiatrists but for all physicians. Disorder of mental function is one of the most common associated conditions in intensive care unit (ICU) patients. However, disturbances of mental function often remain unrecognized. In ICU patients, different types of mental function disorders may develop. They range from sleep disorders, severe depression, anxiety, posttraumatic stress disorder (PTSD) to cognitive disorders including delirium. The causes of mental dysfunction in ICU patients can be divided into environmental and medical. Cognitive disorders are related to mental processes such as learning ability, memory, perception and problem solving. Cognitive disorders are usually not prominent in the early postoperative period and in many cases are discovered after hospital discharge because of difficulties in performing everyday activities at home or at work. The etiology of postoperative cognitive impairment is unclear. Older age, previous presence of cognitive dysfunction, severity of disease, and polypharmacy with more than four drugs are some of the risk factors identified. Delirium is a multifactorial disorder. It is an acute confusional state characterized by alteration of consciousness with reduced ability to focus, sustain, or shift attention. It is considered as the most common form of mental distress in ICU patients. Nearly 30% of all hospitalized patients pass through deliriant phase during their hospital stay. Delirium can last for several days to several weeks. Almost always it ends with complete withdrawal of psychopathological symptoms. Sometimes it can evolve into a chronic brain syndrome (dementia). The causes are often multifactorial and require a number of measures to ease the symptoms. Delirious patient is at risk of complications of immobility and

CD4+ T Cell Response Correlates with Naturally Acquired Antibodies against Plasmodium vivax Tryptophan-Rich Antigens

PubMed Central

Zeeshan, Mohammad; Tyagi, Kriti

2015-01-01

Tryptophan-rich proteins play important biological functions for the Plasmodium parasite. Plasmodium vivax contains remarkably large numbers of such proteins belonging to the “Pv-fam-a” family that need to be characterized. Earlier, we reported the presence of memory T cells and naturally acquired antibodies against 15 of these proteins in P. vivax malaria-exposed individuals (M. Zeeshan, H. Bora, and Y. D. Sharma, J Infect Dis 207:175–185, 2013, http://dx.doi.org/10.1093/infdis/jis650). Here, we sought to characterize and ascertain the cross talk between effector responses of T and B cells in malarial patients against all Pv-fam-a family proteins. Therefore, we expressed the remaining 21 of these proteins in Escherichia coli and studied the humoral and cellular immune responses based on the same parameters used in our previous study. Naturally acquired IgG antibodies were detected against all 21 antigens in P. vivax patient sera (37.7 to 94.4% seropositivity). These antigens were able to activate the lymphocytes of P. vivax-exposed individuals, and the activated CD4+ T lymphocytes produced higher levels of Th1 (interleukin-2 [IL-2] and gamma interferon [IFN-γ]) and Th2 (IL-4 and IL-10) cytokines than the healthy controls, but the response was Th2 biased. The combined results of present and previous studies seem to suggest a striking link between induction of the CD4+ T cell response and naturally acquired antibodies against all 36 proteins of the Pv-fam-a family, the majority of them having conserved sequences in the parasite population. Further work is required to utilize this information to develop immunotherapeutic treatments for this disease. PMID:25733522

Influence of the segmentation on the characterization of cerebral networks of structural damage for patients with disorders of consciousness

NASA Astrophysics Data System (ADS)

Martínez, Darwin; Mahalingam, Jamuna J.; Soddu, Andrea; Franco, Hugo; Lepore, Natasha; Laureys, Steven; Gómez, Francisco

2015-01-01

Disorders of consciousness (DOC) are a consequence of a variety of severe brain injuries. DOC commonly results in anatomical brain modifications, which can affect cortical and sub-cortical brain structures. Postmortem studies suggest that severity of brain damage correlates with level of impairment in DOC. In-vivo studies in neuroimaging mainly focus in alterations on single structures. Recent evidence suggests that rather than one, multiple brain regions can be simultaneously affected by this condition. In other words, DOC may be linked to an underlying cerebral network of structural damage. Recently, geometrical spatial relationships among key sub-cortical brain regions, such as left and right thalamus and brain stem, have been used for the characterization of this network. This approach is strongly supported on automatic segmentation processes, which aim to extract regions of interests without human intervention. Nevertheless, patients with DOC usually present massive structural brain changes. Therefore, segmentation methods may highly influence the characterization of the underlying cerebral network structure. In this work, we evaluate the level of characterization obtained by using the spatial relationships as descriptor of a sub-cortical cerebral network (left and right thalamus) in patients with DOC, when different segmentation approaches are used (FSL, Free-surfer and manual segmentation). Our results suggest that segmentation process may play a critical role for the construction of robust and reliable structural characterization of DOC conditions.

Characterization of the gag/fusion protein encoded by the defective Duplan retrovirus inducing murine acquired immunodeficiency syndrome.

PubMed Central

Huang, M; Jolicoeur, P

1990-01-01

Murine acquired immunodeficiency syndrome is induced by a defective retrovirus. Sequencing of this defective viral genome revealed a long open reading frame which encodes a putative gag/fusion protein, N-MA-p12-CA-NC-COOH, (D. C. Aziz, Z. Hanna, and P. Jolicoeur, Nature (London) 338:505-508, 1989). We raised a specific antibody to the unique p12 domain of this gag fusion precursor, Pr60gag. We found that Pr60gag was indeed encoded by the defective viral genome both in cell-free translation reticulocyte extracts and in infected mouse fibroblasts. Pr60gag was found to be myristylated, phosphorylated, and attached to the cell membrane, like other helper murine leukemia virus (MuLV) gag precursors. Pr60gag was not substantially cleaved within the nonproducer cells and was not released from these cells. However, in the presence of helper MuLV proteins, it formed phenotypically mixed particles. In these particles, Pr60gag was only partially cleaved. In helper MuLV-producing cells harboring the defective virus, a gag-related p40 intermediate was generated both intracellularly and extracellularly. In these cells, Pr60gag appeared to behave as a dominant negative mutant, interfering with proper cleavage of helper Pr65gag. Our data indicate that Pr60gag is a major (and possibly the only) gene product of the defective murine acquired immunodeficiency syndrome virus and is likely to harbor some determinants of pathogenicity of this virus. Images PMID:2243376

Computer Simulations of Intrinsically Disordered Proteins

NASA Astrophysics Data System (ADS)

Chong, Song-Ho; Chatterjee, Prathit; Ham, Sihyun

2017-05-01

The investigation of intrinsically disordered proteins (IDPs) is a new frontier in structural and molecular biology that requires a new paradigm to connect structural disorder to function. Molecular dynamics simulations and statistical thermodynamics potentially offer ideal tools for atomic-level characterizations and thermodynamic descriptions of this fascinating class of proteins that will complement experimental studies. However, IDPs display sensitivity to inaccuracies in the underlying molecular mechanics force fields. Thus, achieving an accurate structural characterization of IDPs via simulations is a challenge. It is also daunting to perform a configuration-space integration over heterogeneous structural ensembles sampled by IDPs to extract, in particular, protein configurational entropy. In this review, we summarize recent efforts devoted to the development of force fields and the critical evaluations of their performance when applied to IDPs. We also survey recent advances in computational methods for protein configurational entropy that aim to provide a thermodynamic link between structural disorder and protein activity.

Legionella pneumophila community-acquired pneumonia (CAP) in a post-splenectomy patient with myelodysplastic syndrome (MDS).

PubMed

Cunha, Burke A; Hage, Jean E

2012-01-01

Legionnaire's disease is a cause of community-acquired pneumonia (CAP) in normal hosts, but those with impaired cell-mediated immunity (CMI) and T-lymphocyte function are particularly predisposed to Legionella species CAP. Myelodysplastic syndrome (MDS) is a disorder of the elderly that is associated with impaired CMI. Cases of MDS or Legionella species CAP are rare. Splenectomized patients primarily have impaired humoral immunity and B-lymphocyte function, and, to a lesser extent, some decrease in CMI. For this reason, Legionnaire's disease has rarely been reported in splenectomized patients. We believe this to be the first reported case of Legionella pneumophila CAP in an asplenic patient with MDS. Copyright © 2012 Elsevier Inc. All rights reserved.

Case Study: Camptocormia, a Rare Conversion Disorder.

ERIC Educational Resources Information Center

Rajmohan, Velayudhan; Thomas, Biju; Sreekumar, Kumaran

2004-01-01

Camptocormia is a condition characterized by severe frontal flexion of the spinal cord and knees, with passive drooping of both arms. It occurs as a form of conversion disorder. Some cases are associated with behavioral problems. A case of camptocormia of 2-year duration in a south Indian adolescent girl with oppositional defiant disorder and…

Aquatic Toxicity Information Retrieval Data Base (ACQUIRE). Data file

DOE Office of Scientific and Technical Information (OSTI.GOV)

Not Available

The purpose of Acquire is to provide scientists and managers quick access to a comprehensive, systematic, computerized compilation of aquatic toxicity data. Scientific papers published both nationally and internationally on the toxicity of chemicals to aquatic organisms and plants are collected and reviewed for ACQUIRE. Independently compiled data files that meet ACQUIRE parameter and quality assurance criteria are also included. Selected toxicity test results and related testing information for any individual chemical from laboratory and field aquatic toxicity effects are included for tests with freshwater and marine organisms. The total number of data records in ACQUIRE is now over 105,300.more » This includes data from 6000 references, for 5200 chemicals and 2400 test species. A major data file, Acute Toxicity of Organic Chemicals (ATOC), has been incorporated into ACQUIRE. The ATOC file contains laboratory acute test data on 525 organic chemicals using juvenile fathead minnows.« less

Acquired perforating dermatosis in a patient with Poland syndrome.

PubMed

Fistarol, Susanna K; Itin, Peter H

2003-01-01

Acquired perforating dermatosis (APD) is characterized by umbilicated 1- to 10-mm-measuring papulonodules with a central adherent oystershell-like keratotic plug, typically on the dorsa of the hands, forearms and over the knees. APD is associated with systemic diseases, especially diabetes mellitus and/or renal failure. Histologically the lesions show transepidermal elimination of altered dermal components into a cup-shaped epidermal depression. We present a 69-year-old man with coexisting APD and Poland syndrome (PS), an association not yet described. PS (OMIM 173800) is a rare congenital anomaly consisting of unilateral partial or total absence of the greater pectoralis muscle and ipsilateral symbrachydactyly. Most cases of PS are sporadic as it was in our case. Our patient had, in addition, an untreated diabetic condition, hyperuricaemia, dilated cardiomyopathy and a very recent pulmonary embolism. He responded to therapy with allopurinol. Copyright 2003 S. Karger AG, Basel

Characterizing psychiatric comorbidity in children with autism spectrum disorder receiving publicly funded mental health services.

PubMed

Brookman-Frazee, Lauren; Stadnick, Nicole; Chlebowski, Colby; Baker-Ericzén, Mary; Ganger, William

2017-09-01

Publicly funded mental health programs play a significant role in serving children with autism spectrum disorder. Understanding patterns of psychiatric comorbidity for this population within mental health settings is important to implement appropriately tailored interventions. This study (1) describes patterns of psychiatric comorbidity in children with autism spectrum disorder who present to mental health services with challenging behaviors and (2) identifies child characteristics associated with comorbid conditions. Data are drawn from baseline assessments from 201 children with autism spectrum disorder who participated in a community effectiveness trial across 29 publicly funded mental health programs. Non-autism spectrum disorder diagnoses were assessed using an adapted Mini-International Neuropsychiatric Interview, parent version. Approximately 92% of children met criteria for at least one non-autism spectrum disorder diagnosis (78% attention deficit hyperactivity disorder, 58% oppositional defiant disorder, 56% anxiety, 30% mood). Logistic regression indicated that child gender and clinical characteristics were differentially associated with meeting criteria for attention deficit hyperactivity disorder, oppositional defiant disorder, an anxiety, or a mood disorder. Exploratory analyses supported a link between challenging behaviors and mood disorder symptoms and revealed high prevalence of these symptoms in this autism spectrum disorder population. Findings provide direction for tailoring intervention to address a broad range of clinical issues for youth with autism spectrum disorder served in mental health settings.

Robotic set-up to quantify hand-eye behavior in motor execution and learning of children with autism spectrum disorder.

PubMed

Casellato, Claudia; Gandolla, Marta; Crippa, Alessandro; Pedrocchi, Alessandra

2017-07-01

Autism spectrum disorder (ASD) is a multifaceted neurodevelopmental disorder characterized by a persistence of social and communication impairment, and restricted and repetitive behaviors. However, motor disorders have also been described, but not objectively assessed. Most studies showed inefficient eye-hand coordination and motor learning in children with ASD; in other experiments, mechanisms of acquisition of internal models in self-generated movements appeared to be normal in autism. In this framework, we have developed a robotic protocol, recording gaze and hand data during upper limb tasks, in which a haptic pen-like handle is moved along specific trajectories displayed on the screen. The protocol includes trials of reaching under a perturbing force field and catching moving targets, with or without visual availability of the whole path. We acquired 16 typically-developing scholar-age children and one child with ASD as a case study. Speed-accuracy tradeoff, motor performance, and gaze-hand spatial coordination have been evaluated. Compared to typically developing peers, in the force field sequence, the child with ASD showed an intact but delayed learning, and more variable gazehand patterns. In the catching trials, he showed less efficient movements, but an intact capability of exploiting the available a-priori plan. The proposed protocol represents a powerful tool, easily tunable, for quantitative (longitudinal) assessment, and for subject-tailored training in ASD.

Epidemiology of acquired aplastic anaemia in Pakistan.

PubMed

Ehsan, Ayesha; Shah, Shahida Amjad Riaz; Ibrahim, Tayyaba

2011-01-01

Acquired aplastic anaemia is a rare disease which results in morbidity and mortality at a young age. This study was carried out to determine the clinical presentation, haematological parameters and association factors of acquired aplastic anaemia in a cohort of Pakistani patients. This was a cross-sectional study conducted at Haematology Department, Shaikh Zayed Hospital, Lahore over 7 years from June 2000 to July 2007. Eighty-two patients of acquired aplastic anaemia were enrolled in the study by non-probability purposive sampling. Their diagnosis was confirmed by complete blood count, bone marrow aspirate and trephine biopsy. The cohort was classified on the basis of severity and the epidemiological, clinical and haematological parameters were analysed. Of the 82 enrolled patients of acquired aplastic anaemia, 49 (59.8%) were males and 33 (40.2%) were females. Mean age of the patients was 27.93 +/- 18.7 years with a range of 1-80 years. The male to female ratio was 1.48:1. Bone marrow cellularity was less than 25 % in 31 (38.0%) cases and between 25-30% in 51 (62%) of patients. Most of the cases were clinically severe aplastic anaemia (68%). In 62 (76%) of the cases no association factors predisposing to aplastic anaemia could be identified. Acquired Aplastic anaemia is a disease of all ages. In the second decade and the elderly predominantly severe clinical stages were seen. Males presented at a younger age while females presented at all ages with a somewhat similar incidence. No association factors of Aplastic Anaemia could be identified in majority of the patients.

Disordered models of acquired dyslexia

NASA Astrophysics Data System (ADS)

Virasoro, M. A.

We show that certain specific correlations in the probability of errors observed in dyslexic patients that are normally explained by introducing additional complexity in the model for the reading process are typical of any Neural Network system that has learned to deal with a quasiregular environment. On the other hand we show that in Neural Networks the more regular behavior does not become naturally the default behavior.

Evidence for a Multi-Dimensional Latent Structural Model of Externalizing Disorders

ERIC Educational Resources Information Center

Witkiewitz, Katie; King, Kevin; McMahon, Robert J.; Wu, Johnny; Luk, Jeremy; Bierman, Karen L.; Coie, John D.; Dodge, Kenneth A.; Greenberg, Mark T.; Lochman, John E.; Pinderhughes, Ellen E.

2013-01-01

Strong associations between conduct disorder (CD), antisocial personality disorder (ASPD) and substance use disorders (SUD) seem to reflect a general vulnerability to externalizing behaviors. Recent studies have characterized this vulnerability on a continuous scale, rather than as distinct categories, suggesting that the revision of the…

An International Survey of Brain Banking Operation and Characterization Practices.

PubMed

Palmer-Aronsten, Beatrix; Sheedy, Donna; McCrossin, Toni; Kril, Jillian

2016-12-01

Brain banks continue to make a major contribution to the study of neurological and psychiatric disorders. The current complexity and scope of research heighten the need for well-characterized cases and the demand for larger cohorts and necessitate strategies, such as the establishment of bank networks based in regional areas. While individual brain banks have developed protocols that meet researchers' needs within the confines of resources and funding, to further promote collaboration, standardization and scientific validity and understanding of the current protocols of participating banks are required. A survey was sent to brain banks, identified by an Internet search, to investigate operational protocols, case characterization, cohort management, data collection, standardization, and degree of collaboration between banks. The majority of the 24 banks that returned the survey have been established for more than 20 years, and most are affiliated with a regional network. While prospective donor programs were the primary source of donation, the data collected on donors varied. Longitudinal information assists case characterization and enhances the analysis capabilities of research. However, acquiring this information depended on the availability of qualified staff. Respondents indicated a high level of importance for standardization, but only 8 of 24 considered this occurred between banks. Standard diagnostic criteria were not achieved in the classification of controls, and some banks relied on the researcher to indicate the criteria for classification of controls. Although the capacity to collaborate with other banks was indicated by 16 of 24 banks, this occurred infrequently. Engagement of all brain banks to participate toward a consensus of diagnostic tools, especially for controls, will strengthen collaboration.

Characterization of fecal vancomycin-resistant enterococci with acquired and intrinsic resistance mechanisms in wild animals, Spain.

PubMed

Lozano, Carmen; Gonzalez-Barrio, David; Camacho, Maria Cruz; Lima-Barbero, Jose Francisco; de la Puente, Javier; Höfle, Ursula; Torres, Carmen

2016-11-01

The objectives were to evaluate the presence of vancomycin-resistant enterococci with acquired (VRE-a) and intrinsic (VRE-i) resistance mechanisms in fecal samples from different wild animals, and analyze their phenotypes and genotypes of antimicrobial resistance. A total of 348 cloacal/rectal samples from red-legged partridges (127), white storks (81), red kites (59), and wild boars (81) (June 2014/February 2015) were inoculated in Slanetz-Bartley agar supplemented with vancomycin (4 μg/mL). We investigated the susceptibility to 12 antimicrobials and the presence of 19 antimicrobial resistance and five virulence genes. In addition, we performed multilocus sequence typing, detection of IS16 and studied Tn1546 structure. One VRE-a isolate was identified in one wild boar. This isolate was identified as Enterococcus faecium, harbored vanA gene included into Tn1546 (truncated with IS1542/IS1216), and belonged to the new ST993. This isolate contained the erm(A), erm(B), tet(M), dfrG, and dfrK genes. Neither element IS16 nor the studied virulence genes were detected. Ninety-six VRE-i isolates were identified (89 Enterococcus gallinarum and seven Enterococcus casseliflavus), with the following prevalence: red kites (71.2 %), white storks (46.9 %), red-legged partridges (7.9 %), and wild boars (4.9 %). Most E. gallinarum isolates showed resistance to tetracycline (66.3 %) and/or erythromycin (46.1 %). High-level resistance to aminoglycosides was present among our VRE-i isolates: kanamycin (22.9 %), streptomycin (11.5 %), and gentamicin (9.4 %). In general, VRE-i isolates of red kites showed higher rates of resistance for non-glycopeptide agents than those of other animal species. The dissemination of acquired resistance mechanisms in natural environments could have implications in the global spread of resistance with public health implications.

Autism Spectrum Disorders (ASD) in Blind Children: Very High Prevalence, Potentially Better Outlook

ERIC Educational Resources Information Center

Jure, Rubin; Pogonza, Ramón; Rapin, Isabelle

2016-01-01

Autism spectrum disorders affected 19 of 38 unselected children at a school for the blind in Cordoba, Argentina. Autism was linked to total congenital blindness, not blindness' etiology, acquired or incomplete blindness, sex, overt brain damage, or socioeconomic status. Autism "recovery," had occurred in 4 verbal children. Congenital…

Key sleep neurologic disorders

PubMed Central

St. Louis, Erik K.

2014-01-01

Summary Sleep disorders are frequent comorbidities in neurologic patients. This review focuses on clinical aspects and prognosis of 3 neurologic sleep disorders: narcolepsy, restless legs syndrome/Willis-Ekbom disease (RLS/WED), and REM sleep behavior disorder (RBD). Narcolepsy causes pervasive, enduring excessive daytime sleepiness, adversely affecting patients' daily functioning. RLS/WED is characterized by an uncomfortable urge to move the legs before sleep, often evolving toward augmentation and resulting in daylong bothersome symptoms. RBD causes potentially injurious dream enactment behaviors that often signify future evolution of overt synucleinopathy neurodegeneration in as many as 81% of patients. Timely recognition, referral for polysomnography, and longitudinal follow-up of narcolepsy, RLS/WED, and RBD patients are imperatives for neurologists in providing quality comprehensive patient care. PMID:24605270

Pervasive Developmental Disorders. NICHCY Briefing Paper FS20.

ERIC Educational Resources Information Center

Tsai, Luke Y.

This briefing paper is intended to provide basic information about the diagnosis, educational programming, and special needs of children and youth with Pervasive Developmental Disorders (PDD), a group of neurological disorders usually evident by age 3 and characterized by impairments in social interaction, imaginative activity, and verbal and…

Language disorders in children with central nervous system injury

PubMed Central

Dennis, Maureen

2011-01-01

Children with injury to the central nervous system (CNS) exhibit a variety of language disorders that have been described by members of different disciplines, in different journals, using different descriptors and taxonomies. This paper is an overview of language deficits in children with CNS injury, whether congenital or acquired after a period of normal development. It first reviews the principal CNS conditions associated with language disorders in childhood. It then describes a functional taxonomy of language, with examples of the phenomenology and neurobiology of clinical deficits in children with CNS insults. Finally, it attempts to situate language in the broader realm of cognition and in current theoretical accounts of embodied cognition. PMID:20397297

Mental Health Disorder Therapeutic Modalities Modified for the GMS

PubMed Central

Sumneangsanor, Tipsuda; Vuthiarpa, Sararud; Somprasert, Chomchueun

2017-01-01

Background: Mental health disorders can affect physical and psychological behaviors. The people of the Greater Mekong Subregion (GMS) have a high risk of mental health disorders, such as depression, stress, and substance abuse be-cause the people in this region are trafficked for forced sex work and various forms of forced labor. In these situations, vic-tims often endure violence and abuse from trafficking recruiters, employers, and other individuals. The purposes of this study were to identify the elements characterizing mental health disorders, especially in terms of depression, stress, and sub-stance abuse, and to identify the treatment modalities for mental health disorders in the GMS. Methods: The researcher undertook a comparative analysis of the literature, reviews of epidemiological studies and mental disorder therapies, and overviews of previous research studies, were used to generate a synthesis of the existing knowledge of the mental disorder therapeutic modalities. Regarding the search methods, the data from the electronic databases PubMed, PsycINFO, Dynamed and ScienceDirect were supplemented with a manual reference search covering relevant studies from 2005 to 2016. Results: Thirty-one papers were included in the review of elements characterizing mental health disorders, especially in terms of depression, stress, and substance abuse, and to identify the treatment modalities for mental health disorders in the GMS. Nine papers defined characterizing mental health disorders, in terms of depression, stress, and substance abuse. Twenty-two papers showed the treatment modalities for mental health disorders that the treatment was effective, these in-cluded pharmacological treatments and psychological treatments, such as mindfulness-based cognitive therapy, biofeedback, and music therapy. Useful guidance can be provided for the prevention and treatment of mental health disorders, and for the care of people in the Greater Mekong Subregion. Conclusion: The

'All the better for not seeing you': effects of communicative context on the speech of an individual with acquired communication difficulties.

PubMed

Bruce, Carolyn; Braidwood, Ursula; Newton, Caroline

2013-01-01

Evidence shows that speakers adjust their speech depending on the demands of the listener. However, it is unclear whether people with acquired communication disorders can and do make similar adaptations. This study investigated the impact of different conversational settings on the intelligibility of a speaker with acquired communication difficulties. Twenty-eight assessors listened to recordings of the speaker reading aloud 40 words and 32 sentences to a listener who was either face-to-face or unseen. The speaker's ability to convey information was measured by the accuracy of assessors' orthographic transcriptions of the words and sentences. Assessors' scores were significantly higher in the unseen condition for the single word task particularly if they had heard the face-to-face condition first. Scores for the sentence task were significantly higher in the second presentation regardless of the condition. The results from this study suggest that therapy conducted in situations where the client is not able to see their conversation partner may encourage them to perform at a higher level and increase the clarity of their speech. Readers will be able to describe: (1) the range of conversational adjustments made by speakers without communication difficulties; (2) differences between these tasks in offering contextual information to the listener; and (3) the potential for using challenging communicative situations to improve the performance of adults with communication disorders. Copyright © 2013 Elsevier Inc. All rights reserved.

Gender identity disorder and schizophrenia: neurodevelopmental disorders with common causal mechanisms?

PubMed

Rajkumar, Ravi Philip

2014-01-01

Gender identity disorder (GID), recently renamed gender dysphoria (GD), is a rare condition characterized by an incongruity between gender identity and biological sex. Clinical evidence suggests that schizophrenia occurs in patients with GID at rates higher than in the general population and that patients with GID may have schizophrenia-like personality traits. Conversely, patients with schizophrenia may experience alterations in gender identity and gender role perception. Neurobiological research, including brain imaging and studies of finger length ratio and handedness, suggests that both these disorders are associated with altered cerebral sexual dimorphism and changes in cerebral lateralization. Various mechanisms, such as Toxoplasma infection, reduced levels of brain-derived neurotrophic factor (BDNF), early childhood adversity, and links with autism spectrum disorders, may account for some of this overlap. The implications of this association for further research are discussed.

Gender Identity Disorder and Schizophrenia: Neurodevelopmental Disorders with Common Causal Mechanisms?

PubMed Central

Rajkumar, Ravi Philip

2014-01-01

Gender identity disorder (GID), recently renamed gender dysphoria (GD), is a rare condition characterized by an incongruity between gender identity and biological sex. Clinical evidence suggests that schizophrenia occurs in patients with GID at rates higher than in the general population and that patients with GID may have schizophrenia-like personality traits. Conversely, patients with schizophrenia may experience alterations in gender identity and gender role perception. Neurobiological research, including brain imaging and studies of finger length ratio and handedness, suggests that both these disorders are associated with altered cerebral sexual dimorphism and changes in cerebral lateralization. Various mechanisms, such as Toxoplasma infection, reduced levels of brain-derived neurotrophic factor (BDNF), early childhood adversity, and links with autism spectrum disorders, may account for some of this overlap. The implications of this association for further research are discussed. PMID:25548672

[Motility disorders of the esophagus].

PubMed

Bruder, E; Rougemont, A-L; Furlano, R I; Schneider, J F; Mayr, J; Haecker, F-M; Beier, K; Schneider, J; Weber, P; Berberich, T; Cathomas, G; Meier-Ruge, W A

2013-03-01

Motility disorders of the esophagus comprise a heterogeneous spectrum of diseases. Primary malformations of the esophagus are now amenable to improved surgical and gastroenterological therapies; however, they often lead to persistent long-term esophageal dysmotility. Achalasia originates from impaired relaxation of the gastroesophageal sphincter apparatus. Systemic diseases may give rise to secondary disorders of esophageal motility. A number of visceral neuromuscular disorders show an esophageal manifestation but aganglionosis rarely extends into the esophagus. The growing group of myopathies includes metabolic and mitochondrial disorders with increasing levels of genetic characterization and incipient emergence of therapeutic strategies. Esophagitis with an infectious etiology causes severe dysmotility particularly in immunocompromised patients. Immunologically mediated inflammatory processes involving the esophagus are increasingly better understood. Finally, rare tumors and tumor-like lesions may impair esophageal motor function.

Attachment and Personality Disorders

ERIC Educational Resources Information Center

Sinha, Preeti; Sharan, Pratap

2007-01-01

Personality disorders (PDs) arise from core psychopathology of interpersonal relationships and understanding of self and others. The distorted representations of self and others, as well as unhealthy relationships that characterize persons with various PDs, indicate the possibility that persons with PDs have insecure attachment. Insecure…

Experimental characterization of the perceptron laser rangefinder

NASA Technical Reports Server (NTRS)

Kweon, I. S.; Hoffman, Regis; Krotkov, Eric

1991-01-01

In this report, we characterize experimentally a scanning laser rangefinder that employs active sensing to acquire three-dimensional images. We present experimental techniques applicable to a wide variety of laser scanners, and document the results of applying them to a device manufactured by Perceptron. Nominally, the sensor acquires data over a 60 deg x 60 deg field of view in 256 x 256 pixel images at 2 Hz. It digitizes both range and reflectance pixels to 12 bits, providing a maximum range of 40 m and a depth resolution of 1 cm. We present methods and results from experiments to measure geometric parameters including the field of view, angular scanning increments, and minimum sensing distance. We characterize qualitatively problems caused by implementation flaws, including internal reflections and range drift over time, and problems caused by inherent limitations of the rangefinding technology, including sensitivity to ambient light and surface material. We characterize statistically the precision and accuracy of the range measurements. We conclude that the performance of the Perceptron scanner does not compare favorably with the nominal performance, that scanner modifications are required, and that further experimentation must be conducted.

Psychiatric diagnosis and antiretroviral adherence among adolescent Medicaid beneficiaries diagnosed with human immunodeficiency virus/acquired immunodeficiency syndrome.

PubMed

Walkup, James; Akincigil, Ayse; Bilder, Scott; Rosato, Nancy Scotto; Crystal, Stephen

2009-05-01

Research on adults with human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) has suggested that psychiatric and substance abuse comorbidities are prevalent in this population, and that these may sometimes be associated with use of antiretroviral therapy (ART) and adherence. For adolescents with HIV/AIDS, much less is known about patterns of mental health comorbidity, and even fewer data are available that compare them to socioeconomically comparable youth without HIV/AIDS. Using medical and pharmacy data from 1999 to 2000 Medicaid claims (Medicaid Analytic Extract) from 4 states for beneficiaries aged 12 to 17 years, we identified 833 youth under care for HIV/AIDS meeting study criteria within the HIV/AIDS group, receipt of ART was less likely for youth who had diagnoses of substance abuse, conduct disorders, or emotional disorders than for others. Once ART was initiated, adherence did not significantly differ between adolescents living with a psychiatric condition, and those who were not, with the exception of an association between conduct disorder and lower adherence. Among those with HIV/AIDS, ART use and adherence were more common among youth with higher rates of service use, regardless of psychiatric status. Associations between race and adherence varied by gender: compared with their white counterparts, minority girls had lower, and minority boys had higher adherence.

The development of acquired immunity to tapeworms and progress towards active immunization, with special reference to Echinococcus spp

PubMed Central

Gemmell, M. A.; Soulsby, E. J. L.

1968-01-01

An assessment is made of the present state of knowledge on acquired immune responses developed by the intermediate and definitive hosts to tapeworm infections. From this evaluation, some gaps in knowledge and some of the problems associated with the development of practical immunization techniques are described. The principal conclusion reached is that absolute resistance to the larval stage can be acquired and resistance to a number of cestode species can be artificially induced in a number of hosts. Thus, research directed towards isolation and characterization of the functional antigens may lead to the development of vaccines for use in public health programmes, such as for the control of echinococcosis, as well as for improving the present status of meat hygiene in regions where cysticercosis, for example, exists. PMID:4883063

Exercise caution: Over-exercise is associated with suicidality among individuals with disordered eating

PubMed Central

Smith, April R.; Fink, Erin L.; Anestis, Michael D.; Ribeiro, Jessica D.; Gordon, Kathryn H.; Davis, Heather; Keel, Pamela K.; Bardone-Cone, Anna M.; Peterson, Carol B.; Klein, Marjorie H.; Crow, Scott; Mitchell, James E.; Crosby, Ross D.; Wonderlich, Stephen A.; le Grange, Daniel; Joiner, Thomas E.

2017-01-01

We conducted four studies to examine the relationship between over-exercise and suicidality. Study 1 investigated whether over-exercise predicted suicidal behavior after controlling for other eating disorder behaviors in a patient sample of 204 women (144 with Diagnostic and Statistical Manual of Mental Disorders, 4th Edition (DSM-IV) Bulimia Nervosa [BN]). Study 2 tested the prospective association between over-exercise and acquired capability for suicide (ACS) in a sample of 171 college students followed for 3–4 weeks. Study 3 investigated whether pain insensitivity accounted for the relationship between over-exercise and ACS in a new sample of 467 college students. Study 4 tested whether ACS accounted for the relationship between over-exercise and suicidal behavior in a sample of 512 college students. In Study 1, after controlling for key covariates, over-exercise was the only disordered eating variable that maintained a significant relationship with suicidal behavior. In Study 2, Time 1 over-exercise was the only disordered eating behavior that was associated with Time 2 ACS. In Study 3, pain insensitivity accounted for the relationship between over-exercise and ACS. In Study 4, ACS accounted for the relationship between over-exercise and suicidal behavior. Over-exercise appears to be associated with suicidal behavior, an association accounted for by pain insensitivity and the acquired capability for suicide; notably, this association was found across a series of four studies with different populations. PMID:23219104

Expressed emotion is not associated with disorder severity in first-episode mental disorder.

PubMed

Heikkilä, Jyrki; Karlsson, Hasse; Taiminen, Tero; Lauerma, Hannu; Ilonen, Tuula; Leinonen, Kirsi-Marja; Wallenius, Elina; Virtanen, Hilkka; Heinimaa, Markus; Koponen, Salla; Jalo, Päivi; Kaljonen, Anne; Salakangas, Raimo K R

2002-08-30

A family atmosphere characterized by expressed emotion (EE) is a robust predictor of clinical outcome of patients with schizophrenia and mood disorders. However, there is ongoing discussion as to whether EE is more a cause of clinical outcome or a parental reaction to disorder severity. This cross-sectional study examines a sample of 42 consecutive first-episode patients from a defined geographical area with severe mental disorders (schizophrenia-related disorders, psychotic mood disorders, and non-psychotic mood disorders). Their 42 relatives were interviewed, and the relationships between EE variables derived with the five-minute speech sample method (FMSS) and the patients' demographic, premorbid and clinical measures were analyzed. A high EE score was found in 40% of the relatives. High EE was associated with the interviewed relative's not being a spouse and the patient's being young and unmarried. It was not associated with premorbid characteristics, symptom dimensions or the diagnostic group of the patient. These results do not support the hypothesis that EE is a reaction to the clinical features of the patient. Instead, demographic factors may partly mediate the effect of EE on prognosis.

Perisylvian GABA levels in schizophrenia and bipolar disorder.

PubMed

Atagün, Murat İlhan; Şıkoğlu, Elif Muazzez; Soykan, Çağlar; Serdar Süleyman, Can; Ulusoy-Kaymak, Semra; Çayköylü, Ali; Algın, Oktay; Phillips, Mary Louise; Öngür, Dost; Moore, Constance Mary

2017-01-10

The aim of this study is to measure GABA levels of perisylvian cortices in schizophrenia and bipolar disorder patients, using proton magnetic resonance spectroscopy ( 1 H-MRS). Patients with schizophrenia (n=25), bipolar I disorder (BD-I; n=28) and bipolar II disorder (BD-II; n=20) were compared with healthy controls (n=30). 1 H-MRS data was acquired using a Siemens 3T whole body scanner to quantify right and left perisylvian structures' (including superior temporal lobes) GABA levels. Right perisylvian GABA values differed significantly between groups [χ 2 =9.62, df: 3, p=0.022]. GABA levels were significantly higher in the schizophrenia group compared with the healthy control group (p=0.002). Furthermore, Chlorpromazine equivalent doses of antipsychotics correlated with right hemisphere GABA levels (r 2 =0.68, p=0.006, n=33). GABA levels are elevated in the right hemisphere in patients with schizophrenia in comparison to bipolar disorder and healthy controls. The balance between excitatory and inhibitory controls over the cortical circuits may have direct relationship with GABAergic functions in auditory cortices. In addition, GABA levels may be altered by brain regions of interest, psychotropic medications, and clinical stage in schizophrenia and bipolar disorder. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Perisylvian GABA levels in schizophrenia and bipolar disorder

PubMed Central

ATAGÜN, Murat İlhan; ŞIKOĞLU, Elif Muazzez; SOYKAN, Çağlar; CAN, Serdar Süleyman; ULUSOY-KAYMAK, Semra; ÇAYKÖYLÜ, Ali; ALGIN, Oktay; PHILLIPS, Mary Louise; ÖNGÜR, Dost; MOORE, Constance Mary

2016-01-01

The aim of this study is to measure GABA levels of perisylvian cortices in schizophrenia and bipolar disorder patients, using proton magnetic resonance spectroscopy (1H-MRS). Patients with schizophrenia (n=25), bipolar I disorder (BD-I; n=28) and bipolar II disorder (BD-II; n=20) were compared with healthy controls (n=30). 1H-MRS data was acquired using a Siemens 3 Tesla whole body scanner to quantify right and left perisylvian structures’ (including superior temporal lobes) GABA levels. Right perisylvian GABA values differed significantly between groups [χ2=9.62, df: 3, p = 0.022]. GABA levels were significantly higher in the schizophrenia group compared with the healthy control group (p=0.002). Furthermore, Chlorpromazine equivalent doses of antipsychotics correlated with right hemisphere GABA levels (r2=0.68, p=0.006, n=33). GABA levels are elevated in the right hemisphere in patients with schizophrenia in comparison to bipolar disorder and healthy controls. The balance between excitatory and inhibitory controls over the cortical circuits may have direct relationship with GABAergic functions in auditory cortices. In addition, GABA levels may be altered by brain regions of interest, psychotropic medications, and clinical stage in schizophrenia and bipolar disorder. PMID:27890741

Depression or Endocrine Disorder?: What Mental Health Counselors Need to Know about Hypothyroidism.

ERIC Educational Resources Information Center

Stanley, Paula Helen

1997-01-01

Describes hypothyroidism, an endocrine disorder characterized by symptoms that resemble those of depression. Discusses features of the disorder, types and grades of hypothyroidism, causes, valuative techniques for the disorder, and implications of hypothyroidism in counseling and in treating patients suffering from this disorder. (RJM)

A review of compulsive buying disorder

PubMed Central

BLACK, DONALD W

2007-01-01

Compulsive buying disorder (CBD) is characterized by excessive shopping cognitions and buying behavior that leads to distress or impairment. Found worldwide, the disorder has a lifetime prevalence of 5.8% in the US general population. Most subjects studied clinically are women (~80%), though this gender difference may be artifactual. Subjects with CBD report a preoccupation with shopping, prepurchase tension or anxiety, and a sense of relief following the purchase. CBD is associated with significant psychiatric comorbidity, particularly mood and anxiety disorders, substance use disorders, eating disorders, and other disorders of impulse control. The majority of persons with CBD appear to meet criteria for an Axis II disorder, although there is no special "shopping" personality. Compulsive shopping tends to run in families, and these families are filled with mood and substance use disorders. There are no standard treatments. Psychopharmacologic treatment studies are being actively pursued, and group cognitive-behavioral models have been developed and are promising. Debtors Anonymous, simplicity circles, bibliotherapy, financial counseling, and marital therapy may also play a role in the management of CBD. PMID:17342214

Autism Spectrum Disorder Profile in Neurofibromatosis Type I

ERIC Educational Resources Information Center

Garg, Shruti; Plasschaert, Ellen; Descheemaeker, Mie-Jef; Huson, Susan; Borghgraef, Martine; Vogels, Annick; Evans, D. Gareth; Legius, Eric; Green, Jonathan

2015-01-01

Neurofibromatosis Type 1 (NF1) is a common autosomal dominant single-gene disorder, in which the co-occurrence of autism spectrum disorder (ASD) has attracted considerable research interest recently with prevalence estimates of 21-40%. However, detailed characterization of the ASD behavioral phenotype in NF1 is still lacking. This study…

Control methods for merging ALSM and ground-based laser point clouds acquired under forest canopies

NASA Astrophysics Data System (ADS)

Slatton, Kenneth C.; Coleman, Matt; Carter, William E.; Shrestha, Ramesh L.; Sartori, Michael

2004-12-01

Merging of point data acquired from ground-based and airborne scanning laser rangers has been demonstrated for cases in which a common set of targets can be readily located in both data sets. However, direct merging of point data was not generally possible if the two data sets did not share common targets. This is often the case for ranging measurements acquired in forest canopies, where airborne systems image the canopy crowns well, but receive a relatively sparse set of points from the ground and understory. Conversely, ground-based scans of the understory do not generally sample the upper canopy. An experiment was conducted to establish a viable procedure for acquiring and georeferencing laser ranging data underneath a forest canopy. Once georeferenced, the ground-based data points can be merged with airborne points even in cases where no natural targets are common to both data sets. Two ground-based laser scans are merged and georeferenced with a final absolute error in the target locations of less than 10cm. This is comparable to the accuracy of the georeferenced airborne data. Thus, merging of the georeferenced ground-based and airborne data should be feasible. The motivation for this investigation is to facilitate a thorough characterization of airborne laser ranging phenomenology over forested terrain as a function of vertical location in the canopy.

Late-onset obsessive compulsive disorder associated with possible gliomatosis cerebri.

PubMed

Kumar, Vineet; Chakrabarti, Subho; Modi, Manish; Sahoo, Manoj

2009-01-01

Onset of obsessive-compulsive disorder (OCD) after the age of 50 years is rare, and should alert the physician to possible "organic" causes of OCD. These include infections, degenerative disorders, brain injury and cerebrovascular lesions, principally involving the frontal lobes and basal ganglia. The current patient had obsessive images, anxiety, auditory hallucinations and seizures following (possible) gliomatosis cerebri, with onset around 69 years of age. The atypical presentation, lesions involving the cortical-basal ganglia-thalamic-cortical circuit and the association with neurological signs/symptoms, was characteristic. However, late-onset OCD has not been commonly reported with diffuse lesions, and the association with gliomatosis cerebri is not known. This patient's case illustrates the need for careful screening of older patients with recently acquired OCD, and for further systematic study of OCD in the broad range of neuropsychiatric disorders affecting the elderly.

Mental health trajectories from adolescence to adulthood: Language disorder and other childhood and adolescent risk factors.

PubMed

Bao, Lin; Brownlie, E B; Beitchman, Joseph H

2016-05-01

Longitudinal research on mental health development beyond adolescence among nonclinical populations is lacking. This study reports on psychiatric disorder trajectories from late adolescence to young adulthood in relation to childhood and adolescent risk factors. Participants were recruited for a prospective longitudinal study tracing a community sample of 5-year-old children with communication disorders and a matched control cohort to age 31. Psychiatric disorders were measured at ages 19, 25, and 31. Known predictors of psychopathology and two school-related factors specifically associated with language disorder (LD) were measured by self-reports and semistructured interviews. The LD cohort was uniquely characterized by a significantly decreasing disorder trajectory in early adulthood. Special education was associated with differential disorder trajectories between LD and control cohorts, whereas maltreatment history, specific learning disorder, family structure, and maternal psychological distress were associated with consistent trajectories between cohorts. From late adolescence to young adulthood, childhood LD was characterized by a developmentally limited course of psychiatric disorder; maltreatment was consistently characterized by an elevated risk of psychiatric disorder regardless of LD history, whereas special education was associated with significantly decreasing risk of psychiatric disorder only in the presence of LD.

Characterizing autism spectrum disorders by key biochemical pathways.

PubMed

Subramanian, Megha; Timmerman, Christina K; Schwartz, Joshua L; Pham, Daniel L; Meffert, Mollie K

2015-01-01

The genetic and phenotypic heterogeneity of autism spectrum disorders (ASD) presents a substantial challenge for diagnosis, classification, research, and treatment. Investigations into the underlying molecular etiology of ASD have often yielded mixed and at times opposing findings. Defining the molecular and biochemical underpinnings of heterogeneity in ASD is crucial to our understanding of the pathophysiological development of the disorder, and has the potential to assist in diagnosis and the rational design of clinical trials. In this review, we propose that genetically diverse forms of ASD may be usefully parsed into entities resulting from converse patterns of growth regulation at the molecular level, which lead to the correlates of general synaptic and neural overgrowth or undergrowth. Abnormal brain growth during development is a characteristic feature that has been observed both in children with autism and in mouse models of autism. We review evidence from syndromic and non-syndromic ASD to suggest that entities currently classified as autism may fundamentally differ by underlying pro- or anti-growth abnormalities in key biochemical pathways, giving rise to either excessive or reduced synaptic connectivity in affected brain regions. We posit that this classification strategy has the potential not only to aid research efforts, but also to ultimately facilitate early diagnosis and direct appropriate therapeutic interventions.

Characterizing autism spectrum disorders by key biochemical pathways

PubMed Central

Subramanian, Megha; Timmerman, Christina K.; Schwartz, Joshua L.; Pham, Daniel L.; Meffert, Mollie K.

2015-01-01

The genetic and phenotypic heterogeneity of autism spectrum disorders (ASD) presents a substantial challenge for diagnosis, classification, research, and treatment. Investigations into the underlying molecular etiology of ASD have often yielded mixed and at times opposing findings. Defining the molecular and biochemical underpinnings of heterogeneity in ASD is crucial to our understanding of the pathophysiological development of the disorder, and has the potential to assist in diagnosis and the rational design of clinical trials. In this review, we propose that genetically diverse forms of ASD may be usefully parsed into entities resulting from converse patterns of growth regulation at the molecular level, which lead to the correlates of general synaptic and neural overgrowth or undergrowth. Abnormal brain growth during development is a characteristic feature that has been observed both in children with autism and in mouse models of autism. We review evidence from syndromic and non-syndromic ASD to suggest that entities currently classified as autism may fundamentally differ by underlying pro- or anti-growth abnormalities in key biochemical pathways, giving rise to either excessive or reduced synaptic connectivity in affected brain regions. We posit that this classification strategy has the potential not only to aid research efforts, but also to ultimately facilitate early diagnosis and direct appropriate therapeutic interventions. PMID:26483618

Selective early-acquired fear memories undergo temporary suppression during adolescence

PubMed Central

Pattwell, Siobhan S.; Bath, Kevin G.; Casey, B. J.; Ninan, Ipe; Lee, Francis S.

2011-01-01

Highly conserved neural circuitry between rodents and humans has allowed for in-depth characterization of behavioral and molecular processes associated with emotional learning and memory. Despite increased prevalence of affective disorders in adolescent humans, few studies have characterized how associative-emotional learning changes during the transition through adolescence or identified mechanisms underlying such changes. By examining fear conditioning in mice, as they transitioned into and out of adolescence, we found that a suppression of contextual fear occurs during adolescence. Although contextual fear memories were not expressed during early adolescence, they could be retrieved and expressed as the mice transitioned out of adolescence. This temporary suppression of contextual fear was associated with blunted synaptic activity in the basal amygdala and decreased PI3K and MAPK signaling in the hippocampus. These findings reveal a unique form of brain plasticity in fear learning during early adolescence and may prove informative for understanding endogenous mechanisms to suppress unwanted fear memories. PMID:21220344

Ketamine as a Rapid Treatment for Post-Traumatic Stress Disorder

DTIC Science & Technology

2011-10-01

Post - traumatic stress disorder ( PTSD ) is a debilitating anxiety disorder characterized by intrusive re-experiences of the traumatic events...08-1-0602 TITLE: Ketamine as a Rapid Treatment for Post - Traumatic Stress Disorder PRINCIPAL INVESTIGATOR: Dennis Charney...dissociative effects of ketamine but not have any sustained anxiolytic and antidepressant effects. Forty individuals diagnosed with post - traumatic

[Diagnostics and antimicrobial therapy of severe community-acquired pneumonia].

PubMed

Sinopalnikov, A I; Zaitsev, A A

2015-04-01

In the current paper authors presented the latest information concerning etiology of severe community-acquired pneumonia. Most cases are caused by a relatively small number ofpathogenic bacterial and viral natures. The frequency of detection of various pathogens of severe community-acquired pneumonia may vary greatly depending on the region, season and clinical profile of patients, availability of relevant risk factors. Authors presented clinical characteristics of severe community-acquired pneumonia and comparative evaluation of a number of scales to assess the risk of adverse outcome of the disease. Diagnosis of severe community-acquired pneumonia includes the following: collecting of epidemiological history, identification of pneumonia, detection of sepsis and identification of multiple organ dysfunction syndrome, detection of acute respiratory failure, assessment of comorbidity. Authors gave recommendations concerning evaluation of the clinical manifestations of the disease, the use of instrumental and laboratory methods for diagnosis of severe community-acquired pneumonia. To select the mode of antimicrobial therapy is most important local monitoring antimicrobial resistance of pathogens. The main criteria for the effectiveness of treatment are to reduce body temperature, severe intoxication, respiratory and organ failure.

[The genetics of depressive disorders].

PubMed

Schulte-Körne, Gerd; Allgaier, Antje-Kathrin

2008-01-01

Among the most common severe psychiatric disorders worldwide, depressive disorders are a leading cause of morbidity, the onset usually occurring during childhood or adolescence. Symptomatology, prevalence, outcome and treatment differentiate depressive disorder nosologically as being either unipolar depression or bipolar disorder, which is characterized by one or more episodes of mania with or without episodes of depression. Genetic factors decisively influence the susceptibility to depressive disorders. Family studies and twin studies have been essential in defining the magnitude of familial risk and liability to heritability, particularly in the case of bipolar disorder. In recent years, linkage and association studies have made great strides towards identifying candidate genes. Particularly the s-allele of the serotonin transporter has been repeatedly confirmed to be a risk factor. Meta-analyses suggest, however, that the genetic contributions of the ascertained loci are relatively small. Along with genetic factors, environmental factors are heavily involved. Gene-environment action plays a pivotal role, particularly in unipolar depression. The genetic disposition seems to be modulated by a protective or pathogenic environment. Early-onset disorders must be further investigated in future as studies to date are somewhat limited.

Clonality of Bacterial Pathogens Causing Hospital-Acquired Pneumonia.

PubMed

Pudová, V; Htoutou Sedláková, M; Kolář, M

2016-09-01

Hospital-acquired pneumonia (HAP) is one of the most serious complications in patients staying in intensive care units. This multicenter study of Czech patients with HAP aimed at assessing the clonality of bacterial pathogens causing the condition. Bacterial isolates were compared using pulsed-field gel electrophoresis. Included in this study were 330 patients hospitalized between May 1, 2013 and December 31, 2014 at departments of anesthesiology and intensive care medicine of four big hospitals in the Czech Republic. A total of 531 bacterial isolates were obtained, of which 267 were classified as etiological agents causing HAP. Similarity or identity was assessed in 231 bacterial isolates most frequently obtained from HAP patients. Over the study period, no significant clonal spread was noted. Most isolates were unique strains, and the included HAP cases may therefore be characterized as mostly endogenous. Yet there were differences in species and potential identical isolates between the participating centers. In three hospitals, Gram-negative bacteria (Enterobacteriaceae and Pseudomonas aeruginosa) prevailed as etiological agents, and Staphylococcus aureus was most prevalent in the fourth center.

Autism Spectrum Disorder: Primary Care Principles.

PubMed

Sanchack, Kristian E; Thomas, Craig A

2016-12-15

Autism spectrum disorder is characterized by difficulty with social communication and restricted, repetitive patterns of behavior, interest, or activities. The Diagnostic and Statistical Manual of Mental Disorders, 5th ed., created an umbrella diagnosis that includes several previously separate conditions: autistic disorder, Asperger syndrome, childhood disintegrative disorder, and pervasive developmental disorder not otherwise specified. There is insufficient evidence to recommend screening for autism spectrum disorder in children 18 to 30 months of age in whom the disorder is not suspected; however, there is a growing body of evidence that early intensive behavioral intervention based on applied behavior analysis improves cognitive ability, language, and adaptive skills. Therefore, early identification of autism spectrum disorder is important, and experts recommend the use of a validated screening tool at 18- and 24-month well-child visits. Medications can be used as adjunctive treatment for maladaptive behaviors and comorbid psychiatric conditions, but there is no single medical therapy that is effective for all symptoms of autism spectrum disorder. Prognosis is heavily affected by the severity of diagnosis and the presence of intellectual disability. Children with optimal outcomes receive earlier, more intensive behavioral interventions and less pharmacologic treatment.

Analysis of ultrasound pulse-echo images for characterization of muscle disease

NASA Astrophysics Data System (ADS)

Leeman, Sidney; Heckmatt, John Z.

1996-04-01

This study aims to extract quantifiable indices characterizing ultrasound propagation and scattering in skeletal muscle, from data acquired using a real-time linear array scanner in a paediatric muscle clinic, in order to establish early diagnosis of Duchenne muscular dystrophy in young children, as well as to chart the progressive severity of the disease. Approximately 40 patients with gait disorders, aged between 1 and 11 years, were scanned with a real-time linear array ultrasound scanner, at 5 MHz. A control group consisted of approximately 50 boys, in the same age range, with no evidence or history of muscle disease. Results show that ultrasound quantitative methods can provide a tight clustering of normal data, and also provide a basis for charting the degree of change in diseased muscle. The most significant (quantitative) parameters derive from the frequency of the attenuation and the muscle echogenicity. The approach provides a discrimination method that is more sensitive than visual assessment of the corresponding image by even an experienced observer. There are also indications that the need for traumatic muscle biopsy may be obviated in some cases.

Extensive Drug Resistance Acquired During Treatment of Multidrug-Resistant Tuberculosis

PubMed Central

Cegielski, J. Peter; Dalton, Tracy; Yagui, Martin; Wattanaamornkiet, Wanpen; Volchenkov, Grigory V.; Via, Laura E.; Van Der Walt, Martie; Tupasi, Thelma; Smith, Sarah E.; Odendaal, Ronel; Leimane, Vaira; Kvasnovsky, Charlotte; Kuznetsova, Tatiana; Kurbatova, Ekaterina; Kummik, Tiina; Kuksa, Liga; Kliiman, Kai; Kiryanova, Elena V.; Kim, HeeJin; Kim, Chang-ki; Kazennyy, Boris Y.; Jou, Ruwen; Huang, Wei-Lun; Ershova, Julia; Erokhin, Vladislav V.; Diem, Lois; Contreras, Carmen; Cho, Sang Nae; Chernousova, Larisa N.; Chen, Michael P.; Caoili, Janice Campos; Bayona, Jaime; Akksilp, Somsak; Calahuanca, Gloria Yale; Wolfgang, Melanie; Viiklepp, Piret; Vasilieva, Irina A.; Taylor, Allison; Tan, Kathrine; Suarez, Carmen; Sture, Ingrida; Somova, Tatiana; Smirnova, Tatyana G.; Sigman, Erika; Skenders, Girts; Sitti, Wanlaya; Shamputa, Isdore C.; Riekstina, Vija; Pua, Kristine Rose; Therese, M.; Perez, C.; Park, Seungkyu; Norvaisha, Inga; Nemtsova, Evgenia S.; Min, Seonyeong; Metchock, Beverly; Levina, Klavdia; Lei, Yung-Chao; Lee, Jongseok; Larionova, Elena E.; Lancaster, Joey; Jeon, Doosoo; Jave, Oswaldo; Khorosheva, Tatiana; Hwang, Soo Hee; Huang, Angela Song-En; Gler, M. Tarcela; Dravniece, Gunta; Eum, Seokyong; Demikhova, Olga V.; Degtyareva, Irina; Danilovits, Manfred; Cirula, Anda; Cho, Eunjin; Cai, Ying; Brand, Jeanette; Bonilla, Cesar; Barry, Clifton E.; Asencios, Luis; Andreevskaya, Sofia N.; Akksilp, Rattanawadee

2014-01-01

Background. Increasing access to drugs for the treatment of multidrug-resistant (MDR) tuberculosis is crucial but could lead to increasing resistance to these same drugs. In 2000, the international Green Light Committee (GLC) initiative began to increase access while attempting to prevent acquired resistance. Methods. To assess the GLC's impact, we followed adults with pulmonary MDR tuberculosis from the start to the end of treatment with monthly sputum cultures, drug susceptibility testing, and genotyping. We compared the frequency and predictors of acquired resistance to second-line drugs (SLDs) in 9 countries that volunteered to participate, 5 countries that met GLC criteria, and 4 countries that did not apply to the GLC. Results. In total, 832 subjects were enrolled. Of those without baseline resistance to specific SLDs, 68 (8.9%) acquired extensively drug-resistant (XDR) tuberculosis, 79 (11.2%) acquired fluoroquinolone (FQ) resistance, and 56 (7.8%) acquired resistance to second-line injectable drugs (SLIs). The relative risk (95% confidence interval [CI]) of acquired resistance was lower at GLC-approved sites: 0.27 (.16–.47) for XDR tuberculosis, 0.28 (.17–.45) for FQ, and 0.15 (.06–.39) to 0.60 (.34–1.05) for 3 different SLIs. The risk increased as the number of potentially effective drugs decreased. Controlling for baseline drug resistance and differences between sites, the odds ratios (95% CIs) were 0.21 (.07–.62) for acquired XDR tuberculosis and 0.23 (.09–.59) for acquired FQ resistance. Conclusions. Treatment of MDR tuberculosis involves substantial risk of acquired resistance to SLDs, increasing as baseline drug resistance increases. The risk was significantly lower in programs documented by the GLC to meet specific standards. PMID:25057101

Brain Volumetric Correlates of Autism Spectrum Disorder Symptoms in Attention Deficit/Hyperactivity Disorder

PubMed Central

O’Dwyer, Laurence; Tanner, Colby; van Dongen, Eelco V.; Greven, Corina U.; Bralten, Janita; Zwiers, Marcel P.; Franke, Barbara; Oosterlaan, Jaap; Heslenfeld, Dirk; Hoekstra, Pieter; Hartman, Catharina A.; Rommelse, Nanda; Buitelaar, Jan K.

2014-01-01

Autism spectrum disorder (ASD) symptoms frequently occur in subjects with attention deficit/hyperactivity disorder (ADHD). While there is evidence that both ADHD and ASD have differential structural correlates, no study to date has investigated these structural correlates within a framework that robustly accounts for the phenotypic overlap between the two disorders. The presence of ASD symptoms was measured by the parent-reported Children’s Social and Behavioural Questionnaire (CSBQ) in ADHD subjects (n = 180), their unaffected siblings (n = 118) and healthy controls (n = 146). ADHD symptoms were assessed by a structured interview (K-SADS-PL) and the Conners’ ADHD questionnaires. Whole brain T1-weighted MPRAGE images were acquired and the structural MRI correlates of ASD symptom scores were analysed by modelling ASD symptom scores against white matter (WM) and grey matter (GM) volumes using mixed effects models which controlled for ADHD symptom levels. ASD symptoms were significantly elevated in ADHD subjects relative to both controls and unaffected siblings. ASD scores were predicted by the interaction between WM and GM volumes. Increasing ASD score was associated with greater GM volume. Equivocal results from previous structural studies in ADHD and ASD may be due to the fact that comorbidity has not been taken into account in studies to date. The current findings stress the need to account for issues of ASD comorbidity in ADHD. PMID:24979066

An Internet-Based Telerehabilitation System for the Assessment of Motor Speech Disorders: A Pilot Study

ERIC Educational Resources Information Center

Hill, Anne J.; Theodoros, Deborah G.; Russell, Trevor G.; Cahill, Louise M.; Ward, Elizabeth C.; Clark, Kathy M.

2006-01-01

Purpose: This pilot study explored the feasibility and effectiveness of an Internet-based telerehabilitation application for the assessment of motor speech disorders in adults with acquired neurological impairment. Method: Using a counterbalanced, repeated measures research design, 2 speech-language pathologists assessed 19 speakers with…

[Diagnosis and therapy of anxiety disorders].

PubMed

Wacker, H R

1997-07-01

Anxiety disorders may be encountered by the medical practitioner in the form of phobias, panic disorder or generalized anxiety disorder. A phobia is characterized by a strong, irrational fear of a given object or situation, often resulting in avoidance behavior. Phobic patients usually respond well to cognitive behavioral therapy. Panic disorder, which is distinguished by recurring, unexpected attacks of fear not bound to particular situations, may also be treated with cognitive behavioral therapy and/or with clomipramin, benzodiazepines or selective serotonin reuptake inhibitors. Patients with generalized anxiety disorder, the main symptom of which is a persistent, free-floating fear over a period of at least several months, may be helped through relaxation techniques, counseling and/or medication with low doses of sedating tricyclic compounds or short-term treatment with benzodiazepines. This article will describe anamnestic findings and the results of clinical examinations of patients with anxiety disorders. Factors to be considered in differential diagnosis will be discussed.

Microendophenotypes of psychiatric disorders: phenotypes of psychiatric disorders at the level of molecular dynamics, synapses, neurons, and neural circuits.

PubMed

Kida, S; Kato, T

2015-01-01

Psychiatric disorders are caused not only by genetic factors but also by complicated factors such as environmental ones. Moreover, environmental factors are rarely quantitated as biological and biochemical indicators, making it extremely difficult to understand the pathological conditions of psychiatric disorders as well as their underlying pathogenic mechanisms. Additionally, we have actually no other option but to perform biological studies on postmortem human brains that display features of psychiatric disorders, thereby resulting in a lack of experimental materials to characterize the basic biology of these disorders. From these backgrounds, animal, tissue, or cell models that can be used in basic research are indispensable to understand biologically the pathogenic mechanisms of psychiatric disorders. In this review, we discuss the importance of microendophenotypes of psychiatric disorders, i.e., phenotypes at the level of molecular dynamics, neurons, synapses, and neural circuits, as targets of basic research on these disorders.

MoRFpred, a computational tool for sequence-based prediction and characterization of short disorder-to-order transitioning binding regions in proteins

PubMed Central

Disfani, Fatemeh Miri; Hsu, Wei-Lun; Mizianty, Marcin J.; Oldfield, Christopher J.; Xue, Bin; Dunker, A. Keith; Uversky, Vladimir N.; Kurgan, Lukasz

2012-01-01

Motivation: Molecular recognition features (MoRFs) are short binding regions located within longer intrinsically disordered regions that bind to protein partners via disorder-to-order transitions. MoRFs are implicated in important processes including signaling and regulation. However, only a limited number of experimentally validated MoRFs is known, which motivates development of computational methods that predict MoRFs from protein chains. Results: We introduce a new MoRF predictor, MoRFpred, which identifies all MoRF types (α, β, coil and complex). We develop a comprehensive dataset of annotated MoRFs to build and empirically compare our method. MoRFpred utilizes a novel design in which annotations generated by sequence alignment are fused with predictions generated by a Support Vector Machine (SVM), which uses a custom designed set of sequence-derived features. The features provide information about evolutionary profiles, selected physiochemical properties of amino acids, and predicted disorder, solvent accessibility and B-factors. Empirical evaluation on several datasets shows that MoRFpred outperforms related methods: α-MoRF-Pred that predicts α-MoRFs and ANCHOR which finds disordered regions that become ordered when bound to a globular partner. We show that our predicted (new) MoRF regions have non-random sequence similarity with native MoRFs. We use this observation along with the fact that predictions with higher probability are more accurate to identify putative MoRF regions. We also identify a few sequence-derived hallmarks of MoRFs. They are characterized by dips in the disorder predictions and higher hydrophobicity and stability when compared to adjacent (in the chain) residues. Availability: http://biomine.ece.ualberta.ca/MoRFpred/; http://biomine.ece.ualberta.ca/MoRFpred/Supplement.pdf Contact: lkurgan@ece.ualberta.ca Supplementary information: Supplementary data are available at Bioinformatics online. PMID:22689782

The Possible Role of Transplacentally-Acquired Antibodies to Infectious Agents, With Molecular Mimicry to Nervous System Sialic Acid Epitopes, as Causes of Neuromental Disorders: Prevention and Vaccine Implications

PubMed Central

Nahmias, André J.; Nahmias, Susanne Beckman; Danielsson, Dan

2006-01-01

Proof of causality of most neuromental disorders (NMD's) is largely unavailable. Lessons from four-decade investigations of the epidemiology, immunology, pathogenesis, prevention and therapy of perinatal infectious agents, which invade directly the nervous system, have led us to propose a new indirect effect hypothesis: maternal transplacentally-acquired antibodies, to agents with epitope molecular mimicry with the developing nervous system, can cross the fetus/infant's blood–nervous system barriers to cause NMD's, clinically manifest years later.Further rationale is provided by relevant evolutionary/developmental (EVO–DEVO) considerations—applicable also to some vaccines. The hypothesis is being tested in: (a) older pregnancy studies with available maternal and newborn sera, and follow-up of the progeny for NMD's; and (b) NMD registry individuals linked to their stored newborn blood spots. Preliminary results support a possible role for schizophrenia of high-tittered antibodies to some agents (toxoplasma, influenza and herpes simplex type 2 virus).A model that includes likely genetic and postnatal influences is schematized and a list of putative agents and factors, based on varying rationales, is tabulated. In case pilot studies are confirmed, the identified agent(s) and antibodies would need to be tested in new prospectively enrolled pregnant women, so as to establish further risk factors leading to possible preventive modalities. PMID:17162360

Obsessive–compulsive disorder: subclassification based on co-morbidity

PubMed Central

Nestadt, G.; Di, C. Z.; Riddle, M. A.; Grados, M. A.; Greenberg, B. D.; Fyer, A. J.; McCracken, J. T.; Rauch, S. L.; Murphy, D. L.; Rasmussen, S. A.; Cullen, B.; Pinto, A.; Knowles, J. A.; Piacentini, J.; Pauls, D. L.; Bienvenu, O. J.; Wang, Y.; Liang, K. Y.; Samuels, J. F.; Roche, K. Bandeen

2011-01-01

Background Obsessive–compulsive disorder (OCD) is probably an etiologically heterogeneous condition. Many patients manifest other psychiatric syndromes. This study investigated the relationship between OCD and co-morbid conditions to identify subtypes. Method Seven hundred and six individuals with OCD were assessed in the OCD Collaborative Genetics Study (OCGS). Multi-level latent class analysis was conducted based on the presence of eight co-morbid psychiatric conditions [generalized anxiety disorder (GAD), major depression, panic disorder (PD), separation anxiety disorder (SAD), tics, mania, somatization disorders (Som) and grooming disorders (GrD)]. The relationship of the derived classes to specific clinical characteristics was investigated. Results Two and three classes of OCD syndromes emerge from the analyses. The two-class solution describes lesser and greater co-morbidity classes and the more descriptive three-class solution is characterized by: (1) an OCD simplex class, in which major depressive disorder (MDD) is the most frequent additional disorder; (2) an OCD co-morbid tic-related class, in which tics are prominent and affective syndromes are considerably rarer; and (3) an OCD co-morbid affective-related class in which PD and affective syndromes are highly represented. The OCD co-morbid tic-related class is predominantly male and characterized by high conscientiousness. The OCD co-morbid affective-related class is predominantly female, has a young age at onset, obsessive–compulsive personality disorder (OCPD) features, high scores on the ‘taboo’ factor of OCD symptoms, and low conscientiousness. Conclusions OCD can be classified into three classes based on co-morbidity. Membership within a class is differentially associated with other clinical characteristics. These classes, if replicated, should have important implications for research and clinical endeavors. PMID:19046474

Acquired factor VII deficiency associated with acute myeloid leukemia.

PubMed

Anoun, Soumaya; Lamchahab, Mouna; Oukkache, Bouchra; Qachouh, Maryam; Benchekroun, Said; Quessar, Asmaa

2015-04-01

Isolated acquired factor VII deficiency is a rare coagulopathy. It has been reported in 31 patients with malignancy, sepsis, postoperatively, aplastic anemia, and during bone marrow transplantation. We discuss, through a new case of acquired factor VII deficiency, the characteristics of this disease when it is associated with acute myeloid leukemia. Acquired factor VII deficiency in hematological diseases can be caused by intensive chemotherapy, infections, or hepatic dysfunction. The best treatment in developing countries remains corticosteroids associated with plasma exchange, frozen plasma, and antibiotics.

27 CFR 6.45 - Assistance in acquiring license.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 27 Alcohol, Tobacco Products and Firearms 1 2010-04-01 2010-04-01 false Assistance in acquiring license. 6.45 Section 6.45 Alcohol, Tobacco Products and Firearms ALCOHOL AND TOBACCO TAX AND TRADE BUREAU, DEPARTMENT OF THE TREASURY LIQUORS âTIED-HOUSEâ Unlawful Inducements Furnishing Things of Value § 6.45 Assistance in acquiring license. Any...

Effect of disorder on the optical properties of short period superlattices

NASA Technical Reports Server (NTRS)

Strozier, J. A.; Zhang, Y. A.; Horton, C.; Ignatiev, A.; Shih, H. D.

1993-01-01

The optical properties of disordered short period superlattices are studied using a one-dimensional tight-binding model. A difference vector and disorder structure factor are proposed to characterize the disordered superlattice. The density of states, participation number, and optical absorption coefficients for both ordered and disordered superlattices are calculated as a function of energy. The results show that introduction of disorder into an indirect band gap material enhances the optical transition near the indirect band edge.

Pediatric misophonia with comorbid obsessive-compulsive spectrum disorders.

PubMed

Webber, Troy A; Johnson, Patricia L; Storch, Eric A