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Sample records for self-improving collodion ichthyosis

  1. Genotypic and clinical spectrum of self-improving collodion ichthyosis: ALOX12B, ALOXE3, and TGM1 mutations in Scandinavian patients

    DEFF Research Database (Denmark)

    Vahlquist, Anders; Bygum, Anette; Gånemo, Agneta

    2010-01-01

    Infants born with autosomal recessive congenital ichthyosis (ARCI) are often encapsulated in a collodion membrane, which shows a lamellar or erythrodermic type of ichthyosis upon shedding. However, some babies show a nearly normal underlying skin after several weeks, a phenotype called "self...... scaling, palmar hyperlinearity with keratoderma, and a frequent appearance of red cheeks and anhidrosis. Thus, we propose replacing SHCB with the term "self-improving collodion ichthyosis" (SICI). In conclusion, ALOX12B mutations are the leading cause of SICI in Scandinavia, followed by ALOXE3 mutations...

  2. Genotypic and Clinical Spectrum of Self-Improving Collodion Ichthyosis: ALOX12B, ALOXE3, and TGM1 Mutations in Scandinavian Patients

    DEFF Research Database (Denmark)

    Vahlquist, Anders; Bygum, Anette; Gånemo, Agneta

    2009-01-01

    Infants born with autosomal recessive congenital ichthyosis (ARCI) are often encapsulated in a collodion membrane, which shows a lamellar or erythrodermic type of ichthyosis upon shedding. However, some babies show a nearly normal underlying skin after several weeks, a phenotype called "self...... scaling, palmar hyperlinearity with keratoderma, and a frequent appearance of red cheeks and anhidrosis. Thus, we propose replacing SHCB with the term "self-improving collodion ichthyosis" (SICI). In conclusion, ALOX12B mutations are the leading cause of SICI in Scandinavia, followed by ALOXE3 mutations...

  3. LAMELLAR ICHTHYOSIS (COLLODION BABY

    Directory of Open Access Journals (Sweden)

    Paramarta IGE

    2012-11-01

    Full Text Available The ichthyosis are a heterogeneous group of hereditary and acquired disorder of keratinization which affected the epidermis characterized by presence of visible scales on the skin surface in the absence of inflammation. It can occur as a disease limited to the skin or in association with abnormalities of other organ systems. Lamelar ihthyosis (LI is one of two mayor autosomal recessive ichthyosis with an incidence of approximately one in 300,000. The diagnosis is based on clinical and pathologic finding. Infection is the most common complication, while prognosis of LI is depends on severity and complication of the disease. A case of lamellar ichthyosis in 0 day Balinese female baby was reported. The skin of the body was thick, plate-like appearance, scaling on the entire body, some of the thick skin was ruptured on chest and extremities. There were eclabium on the mouth and ectropion on the eyes. Histopathology examination showed hyperkeratosis without perivascular infiltration lymphocyte. The baby was given breast feeding, antibiotic, hydrocortisone cream and olium olivarum. The prognosis of the baby is good.

  4. Two Cases of Autosomal Recessive Congenital Ichthyosis due to CYP4F22 Mutations: Expanding the Genotype of Self-Healing Collodion Baby

    NARCIS (Netherlands)

    Noguera-Morel, L.; Feito-Rodriguez, M.; Maldonado-Cid, P.; Garcia-Minaur, S.; Kamsteeg, E.J.; Gonzalez-Sarmiento, R.; Lucas-Laguna, R. De; Hernandez-Martin, A.; Torrelo, A.

    2016-01-01

    Collodion babies are born with a tight, shiny cast that sheds in a few weeks. After shedding, most patients will display features of autosomal recessive congenital ichthyosis (ARCI) later in life but in up to 10% of cases, the skin eventually becomes normal or only minimally involved, a phenotype

  5. Collodion Baby - a Case Report

    Directory of Open Access Journals (Sweden)

    Stojanović Vesna

    2014-12-01

    Full Text Available Colloidon baby describes a highly characteristic clinical entity in newborns encased in a yellowish translucent membrane resembling collodion. In most cases the condition either precedes the development of one of a variety of ichthyoses, the commonest of which are lamellar ichthyosis and non-bullous ichthyosiform erythroderma, or occasionally represents an initial phase of other ichthyoses such as ichthyosis vulgaris. In at least 10% of all cases of collodion baby, the condition is followed by a mild ichthyosis of lamellar type, so mild as to be considered more or less normal, so-called self-healing collodion baby or ‘lamellar ichthyosis of the newborn’. In this report we present a rare case of collodion baby in whom, after collodion membrane peeled-off, the skin retained normal appearance.

  6. Collodion baby

    Directory of Open Access Journals (Sweden)

    Dhaded S

    1992-01-01

    Full Text Available Collodion baby is a rare congenital disorder characterized by parchment like taught membrane covering the whole body. Other findings in our case include ectropion, eversion of lips, and flattening of nose and ears. Skin biopsy showed features consistant with the diagnosis of collodion baby. The child was treated with supportive measures like antibiotics, intravenous fluids, humidification, and application of emollients.

  7. Expedient Treatment of a Collodion Baby

    Directory of Open Access Journals (Sweden)

    Michael Chung

    2011-01-01

    Full Text Available Only ~270 cases of collodion babies have been reported in the literature since 1892. As the name suggests, the term “collodion baby” refers to a phenotype that can be characterized by a yellow, shiny, tight parchment-like membrane stretched over the skin. Although the collodion membrane is only an evanescent condition of the newborn, neonatal complications can occur in 45% of all collodion babies, leading to a mortality rate of ~11% in the first few weeks of life. Most children born as collodion babies will spontaneously desquamate within 2 weeks, but may be as long as 3 months. Eventually, these children develop signs of one of several types of ichthyosis, which gives the skin the appearance of “fish scales.” We report a unique case of a Caucasian male that was born as a Collodion baby at the University of Kentucky Children's Hospital in Lexington, Kentucky. Although the impairment of the skin barrier function put the patient at risk for a number of complications, he improved significantly after being treated with emollients and antibiotics. In contrast to previous findings, we found that skin emollients were beneficial and did not increase the risk of infection.

  8. Collodion Baby with TGM1 gene mutation

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    Sharma D

    2015-09-01

    Full Text Available Deepak Sharma,1 Basudev Gupta,2 Sweta Shastri,3 Aakash Pandita,1 Smita Pawar4 1Department of Neonatology, Fernandez Hospital, Hyderguda, Hyderabad, Andhra Pradesh, 2Department of Pediatrics, Civil Hospital, Palwal, Haryana, 3Department of Pathology, NKP Salve Medical College, Nagpur, Maharashtra, 4Department of Obstetrics and Gynaecology, Fernandez Hospital, Hyderguda, Hyderabad, Andhra Pradesh, IndiaAbstract: Collodion baby (CB is normally diagnosed at the time of birth and refers to a newborn infant that is delivered with a lambskin-like membrane encompassing the total body surface. CB is not a specific disease entity, but is a common phenotype in conditions like harlequin ichthyosis, lamellar ichthyosis, nonbullous congenital ichthyosiform erythroderma, and trichothiodystrophy. We report a CB that was brought to our department and later diagnosed to have TGM1 gene c.984+1G>A mutation. However, it could not be ascertained whether the infant had lamellar ichthyosis or congenital ichthyosiform erythroderma (both having the same mutation. The infant was lost to follow-up.Keywords: cellophane membrane, c.984+1G>A mutation, lamellar ichthyosis, nonbullous congenital ichthyosiform erythroderma, parchment membrane, TGM1 gene

  9. Lamellar ichthyosis

    Science.gov (United States)

    ... the palms and soles is thickened Treatment Collodion babies usually need to stay in the neonatal intensive care unit (NICU). They are placed in a high-humidity incubator. They will need extra feedings. Moisturizers need to ...

  10. Ichthyosis hystrix

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    Surajit Nayak

    2013-01-01

    Full Text Available The present report describes the condition in a three day old male child with bilateral ,linear, hyperpigmented and hyperkeratotic verrucous plaques and patchy alopecia over scalpe without any nail and skeletal abnormalities. It was suggestive of ichthyosis hystrix type of epidermal nevus,and is being reported in view of the rarity of this condition.

  11. Ichthyosis vulgaris

    DEFF Research Database (Denmark)

    Thyssen, J P; Godoy-Gijon, E; Elias, P M

    2013-01-01

    in this review article, FLG mutations are observed in approximately 7·7% of Europeans and 3·0% of Asians, but appear to be infrequent in darker-skinned populations. This clinical review article provides an overview of ichthyosis vulgaris epidemiology, related disorders and pathomechanisms. Not only does......-deficient skin, and epidemiological studies have found higher levels of hand eczema, irritant contact dermatitis, nickel sensitization and serum vitamin D levels. When relevant, individuals should be informed about an increased risk of developing dermatitis when repeatedly or continuously exposed to nickel...... or irritants. Moreover, with our current knowledge, individuals with ichthyosis vulgaris should be protected against neonatal exposure to cats to prevent atopic dermatitis and should abstain from smoking to prevent asthma. Finally, they should be advised against excessive exposure to factors that decrease skin...

  12. Otologic Manifestations of Autosomal Recessive Congenital Ichthyosis in Children.

    Science.gov (United States)

    Martín-Santiago, A; Rodríguez-Pascual, M; Knöpfel, N; Hernández-Martín, Á

    2015-11-01

    Few studies have investigated ear involvement in nonsyndromic autosomal recessive congenital ichthyosis (ARCI). To assess the type and frequency of otologic manifestations of ARCI in patients under follow-up at the pediatric dermatology department of our hospital. We prospectively studied the presence of ear pain, ear itching, tinnitus, otitis, cerumen impaction, accumulation of epithelial debris, and hearing loss. Daily hygiene measures, topical treatments, medical-surgical interventions, and frequency of visits to an ear, nose, and throat (ENT) specialist were noted in the patients' medical records. Ear examination and hearing tests were performed in all cases. Ten patients were studied: 2 had a self-healing collodion baby phenotype and 8 had ichthyosis. There was mention of otologic manifestations in the records of all 8 patients with ichthyosis (100%); 6 of these patients (75%) had abnormalities in the external auditory canal examination and 2 (25%) had conductive hearing loss. Our findings are limited by the small number of patients studied, all of whom were younger than 19 years. The involvement of both dermatologists and ENT specialists in the management of patients with ichthyosis is crucial to ensure the application of the best therapeutic and preventive measures. More studies are needed to assess the prevalence and impact on quality of life of ear involvement in patients with ichthyosis and to determine the optimal interval between ENT visits for these patients. Copyright © 2015 Elsevier España, S.L.U. and AEDV. All rights reserved.

  13. A Rare Case Report of a Collodion Baby with Severe Hypernatremia

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    Ahmad Farhat

    2017-06-01

    Full Text Available Background: Collodion baby is a rare condition (i.e., 1:300000 birth, which is referred to a neonate covered with a tight and shiny membrane desquamating within two weeks. This condition takes place as a result of the epidermal cornification disorder. Given the impairment of the skin barrier function, these neonates are at the risk of several complications, including hypernatremic dehydration, hypothermia, skin infections, fissures, conjunctivitis, sepsis, dehydration, and constrictive bands of the extremities resulting in vascular compromise and edema. This condition has a high mortality rate; accordingly, the majority of the collodion babies die within the first few weeks of birth due to the secondary complications depending on the type of mutations. Although the collodion membrane is a transient condition, it can lead to a number of complications in 45% of the newborns and result in 11% mortality rate in the first few weeks of the neonatal life. However, in the recent years, the mortality rates have been declined owing to the introduction of systemic retinoids to the clinical practice and the advanced care methods, which are used in the intensive care units. Harlequin ichthyosis has been associated with the mutation in the ABCA12 gene; therefore, genetic counseling and mutation screening of this gene should be considered.Case report: Herein, we reported a case of a collodion baby with favorable evolution, who developed hypernatremia and suffered from some complications having aroused from the condition.Conclusion: Infection prevention and supportive care of collodion babies mainly with incubator, serum therapy, and feeding support can be effective in preventing complications.

  14. Scanning electron microscopy of the collodion membrane from a self-healing collodion baby*

    Science.gov (United States)

    de Almeida Jr., Hiram Larangeira; Isaacsson, Henrique; Guarenti, Isabelle Maffei; Silva, Ricardo Marques e; de Castro, Luis Antônio Suita

    2015-01-01

    Abstract Self-healing collodion baby is a well-established subtype of this condition. We examined a male newborn, who was covered by a collodion membrane. The shed membrane was examined with scanning electron microscopy. The outer surface showed a very compact keratin without the normal elimination of corneocytes. The lateral view of the specimen revealed a very thick, horny layer. The inner surface showed the structure of lower corneocytes with polygonal contour. With higher magnifications villous projections were seen in the cell membrane. PMID:26375232

  15. Ichthyosis uteri with leiomyoma.

    Science.gov (United States)

    Kanno, Kiyoshi; Kusakabe, Takashi; Takata, Megumi; Suzuki, Kazuo; Oowada, Makoto; Suzuki, Hiroshi

    2016-11-01

    A 58-year-old, postmenopausal, multiparous woman presented with a chief complaint of abnormal vaginal bleeding. Endometrial cytology was evaluated twice, revealing only squamous epithelial cells both times. Degenerated leiomyoma or uterine sarcoma was suspected from imaging findings, and total abdominal hysterectomy and bilateral salpingo-oophorectomy were therefore performed. However, histopathological examination revealed no signs of malignancy, and the patient was diagnosed as having ichthyosis uteri with uterine leiomyoma. No koilocytosis was evident, and immunostaining for p16 was also negative. Ichthyosis uteri is an extremely rare disease of unknown origin in which squamous metaplasia of the endometrium occurs across a wide area. Although regarded as a benign condition, cases have been reported in which the underlying condition was squamous cell carcinoma or endometrial adenocarcinoma. If ichthyosis uteri is present, a comprehensive approach is required, and the possibility of uterine malignancy should be considered. However, there may be no direct association between the malignant lesions and ichthyosis uteri. © 2016 Japan Society of Obstetrics and Gynecology.

  16. Ichthyosis prematurity syndrome: a well-defined congenital ichthyosis subtype

    DEFF Research Database (Denmark)

    Bygum, Anette; Westermark, Per; Brandrup, Flemming

    2008-01-01

    Ichthyosis prematurity syndrome is a rare syndrome characterized by the clinical triad of premature birth, thick caseous desquamating epidermis, and neonatal asphyxia. We describe two siblings with ichthyosis prematurity syndrome. The index patient was born at gestational week 34. Immediately aft...... in the stratum corneum and stratum granulosum. Diagnosing this syndrome is important to reassure parents, obstetricians, and pediatricians about its benign course after complications in the perinatal period....

  17. Acquired ichthyosis with hoffman's syndrome

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    Sathyanarayana B

    2003-01-01

    Full Text Available A middle aged man presented with features of acquired ichthyosis with Hoffman's syndrome. Laboratory tests support hypothyodism. Myoedema and hypertrophy of muscles were present. Patient was previously treated for Pellagra.

  18. Lamellar ichthyosis maps to chromosome 14q11

    Energy Technology Data Exchange (ETDEWEB)

    Russell, L.J.; Compton, J.G.; Bale, S.J. [and others

    1994-09-01

    Lamellar ichthyosis (LI) is a serious skin disorder inherited as an autosomal recessive trait and characterized by large, brown plate-like scales covering the body. Skin involvement is apparent at birth, often as a collodion membrane. Scarring alopecia, ectropion, and secondary hypohidrosis are frequent. We used a panel of candidates genes that are expressed in the epidermis to study seven multiplex Caucasian families in the U.S. and six inbred (multiplex and simplex) families in Egypt. We find no recombination (Z=9.11 at {theta}=0) in either set of families with transglutaminse 1 (TGM1), the gene encoding the enzyme responsible for cross-linking proteins to the cell envelope in the upper-most layer of the epidermis. In addition, striking homozygosity is observed in the inbred families for markers neighboring TGM1, defining a 9.3 cM candidate region which is bounded by MYH7 and D14S275. This is the first report of linkage in LI and suggests that further study of the TGM1 gene may identify the underlying pathogenesis of this severe, disfiguring disorder. Linkage-based genetic counseling and prenatal diagnosis is now available for informative at-risk families.

  19. Congenital lamellar ichthyosis, a case report.

    OpenAIRE

    Joaquín Saavedra D.; María José Sierralta S.; Cristian Saavedra D; Vanesa Rivera C; Francisco Cerda C.

    2014-01-01

    ABSTRACT INTRODUCTION: Lamellar Ichthyosis is a rare skin diseases belonging to the Group of the so-called genodermatoses. It is a form of congenital ichthyosis evident at birth. CASE REPORT: Male neonate, born at 36 weeks of gestation via cesarian section, appropriate for gestational age and Apgar Score 8. Nonconsanguineous parents. Affected brother with Ichthyosis lamellar. Is hospitalized in the Neonatal Intermediary Care Unit of the Hospital of San Fernando due to ...

  20. Dermatoglyphics in Ichthyosis and Alopecia Areata

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    K C Verma

    1981-01-01

    Full Text Available A total of 80 cases comprising of 40 controls and twenty each of ichthyosis and alopecia areata were studied for dermatoglyphic patterns and ridge count. Statistically significant increased incidence of whorl pattern was observed in female cases of autosomal dominant and sex linked ichthyosis. Significant change in the distribution of patterns was found in female cases of alopecia areata. -

  1. Collodion baby: A report of 4 cases | Obu | Nigerian Journal of ...

    African Journals Online (AJOL)

    Introduction: The term collodion baby refers to a clinical entity noted in newborns who are enmeshed by a translucent, cornified substance like sheets of uniform texture so called (collodion membrane) which gives the whole body surface a varnished appearance. Although, some other diseases and conditions may lead to ...

  2. Collodion-reinforcement and plasma-cleaning of target foils

    Science.gov (United States)

    Stoner, John O.

    2002-03-01

    The preparation of evaporated target foils can often be facilitated by use of collodion coatings either on the substrate sides or on the exterior surfaces of the foils. Later, such coatings must usually be removed. Cleaning of a foil is necessary if thin layers of adhesives have crept onto the foil. Removal and/or cleaning can often be done satisfactorily with an oxygen plasma. Apparatus and procedures used for this are described. Foils that were cleaned successfully, and some that were incompatible with the cleaning process are listed.

  3. Collodion-reinforcement and plasma-cleaning of target foils

    International Nuclear Information System (INIS)

    Stoner, John O.

    2002-01-01

    The preparation of evaporated target foils can often be facilitated by use of collodion coatings either on the substrate sides or on the exterior surfaces of the foils. Later, such coatings must usually be removed. Cleaning of a foil is necessary if thin layers of adhesives have crept onto the foil. Removal and/or cleaning can often be done satisfactorily with an oxygen plasma. Apparatus and procedures used for this are described. Foils that were cleaned successfully, and some that were incompatible with the cleaning process are listed

  4. Spontaneous subconjunctival abscess in congenital lamellar ichthyosis

    Directory of Open Access Journals (Sweden)

    Shivanand C Bubanale

    2018-01-01

    Full Text Available Congenital lamellar ichthyosis is an autosomal recessive, heterogeneous disorder presenting at birth with generalized skin involvement. The most common ophthalmic manifestation noted is bilateral ectropion of the lower eyelids. A 1-month-old female neonate, the second born of a nonconsanguineous marriage, presented with 4 days' history of redness, discharge, and swelling in the right eye. There was severe right upper eyelid ectropion, conjunctival injection, chemosis, a subconjunctival mass on the temporal bulbar conjunctiva spontaneously draining pus and corneal haze. The anterior chamber, iris, lens and fundus appeared normal. Congenital lamellar ichthyosis was suspected because of scaling and excessive dryness of the entire body. The occurrence of a spontaneous subconjunctival abscess is not known in lamellar ichthyosis. We thus report the management of a rare case of unilateral upper eyelid ectropion, subconjunctival abscess with orbital cellulitis in congenital lamellar ichthyosis.

  5. Generalized and Naevoid Epidermolytic Ichthyosis in Denmark

    DEFF Research Database (Denmark)

    Bygum, Anette; Virtanen, Marie; Brandrup, Flemming

    2012-01-01

    A Danish-Swedish collaboration was established to identify and classify a Danish cohort of patients with epidermolytic ichthyosis, also known as epidermolytic hyperkeratosis. Patients were recruited from 5 dermatology departments in Denmark, and data were obtained using a structured questionnaire...... and a systematic examination together with photographs, histopathological descriptions and blood samples for mutational analysis. Sixteen patients from 12 families with generalized or naevoid epidermolytic ichthyosis and ichthyosis bullosa of Siemens were identified. Five families had mutations in K1 and 6...... families had mutations in K10. Nine patients had been treated with systemic retinoids (etretinate, acitretin, isotretinoin or alitretinoin), but only 3 patients had acceptable treatment responses and chose to continue therapy. In conclusion epidermolytic ichthyosis is a rare disease with a prevalence...

  6. Ichthyosis

    Science.gov (United States)

    ... this research; and the dissemination of information on research progress in these diseases. Contact Us NIAMS Archive Viewers and Players Social Media Moderation Policy FOIA Privacy Statement Accessibility Disclaimer Digital Strategy ...

  7. Modern concepts of pathogenesis of ichthyosis

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    Світлана Володимирівна Дмитренко

    2015-06-01

    Full Text Available The modern concepts of ichthyosis are rather ambiguous and need more precise definition. The modern conception of pathogenesis of ichthysosis is offered and considered in this article.Aim. An aim is to analyze received data of our researches about molecular disturbances of keratin on the background of ichthyosis and the current data on the pathogenesis of disease.Materials and methods. An analysis of the results of research in 70 patients with ichthyosis by the methods of the flow cytometry, immunohistochemistry and by immunologic methods is presented in an article.Results. Authors revealed molecular, immunologic and immunohistochemical changes that realizes the disturbance of keratinization on the background of this disease. The model of pathogenesis of the various manifestations of gene mutations that causes ichthyosis is proposed and it can be taken into account when elaborating the new directions of therapy.Conclusions. Gene mutations that cause ichthyosis realizes on the background of disturbance of the cell cycle causing cornification and disturb the local and general immune reactions that summarily lead to the clinical presentations of disease. 

  8. [Ichthyosis and social stigma in Burkina Faso].

    Science.gov (United States)

    Korsaga/Somé, N; Salissou, L; Tapsoba, G P; Ouédraogo, M S; Traoré, F; Doulla, M; Barro/Traoré, F; Niamba, P; Traoré, A

    2016-01-01

    Through the story of two families presenting ichthyosis, we report the support and social integration difficulties inherent in these genetic diseases. Family No. 1: a 38-year-old shepherd and his wife of 25 years both had lamellar ichthyosis that had been present continually since childhood. They had had 2 stillborn infants as well as a live newborn that were all presenting lamellar ichthyosis. Family No. 2: a 45-year-old housewife was seen at our consultation with her 3 youngest children aged 8 years, 6 years and 18 months. According to the mother, at birth, all 3 children were covered with a membrane resembling plastic that crackled during movement, and they had red eyes. Examination of the 3 children revealed a clinical picture of lamellar ichthyosis with ectropion, malformed ears and brachydactyly. Although they presented delayed growth and weight development, psychomotor development was normal. There was no consanguinity between the parents. In both families, the visible nature of the dermatosis resulted in discrimination and ostracism. The precarious living conditions of the parents and the high cost of treatment in an African setting resulted in degradation of quality of life with exacerbation of the difficulties of social integration, resulting in a lack of schooling and a bleak future for these children. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  9. Topical cholesterol in clofazimine induced ichthyosis

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    Pandey S

    1994-01-01

    Full Text Available Topical application of 10% cholesterol in petrolatum significantly (P< 0.05 controlled the development of ichthyosis in 62 patients taking 100 mg clofazimine daily for a period of 3 months. However, topical cholesterol application did not affect the lowering of serum cholesterol induced by oral clofazimine. Probable mechanism of action is being discussed.

  10. Ichthyosis vulgaris: the filaggrin mutation disease.

    Science.gov (United States)

    Thyssen, J P; Godoy-Gijon, E; Elias, P M

    2013-06-01

    Ichthyosis vulgaris is caused by loss-of-function mutations in the filaggrin gene (FLG) and is characterized clinically by xerosis, scaling, keratosis pilaris, palmar and plantar hyperlinearity, and a strong association with atopic disorders. According to the published studies presented in this review article, FLG mutations are observed in approximately 7·7% of Europeans and 3·0% of Asians, but appear to be infrequent in darker-skinned populations. This clinical review article provides an overview of ichthyosis vulgaris epidemiology, related disorders and pathomechanisms. Not only does ichthyosis vulgaris possess a wide clinical spectrum, recent studies suggest that carriers of FLG mutations may have a generally altered risk of developing common diseases, even beyond atopic disorders. Mechanistic studies have shown increased penetration of allergens and chemicals in filaggrin-deficient skin, and epidemiological studies have found higher levels of hand eczema, irritant contact dermatitis, nickel sensitization and serum vitamin D levels. When relevant, individuals should be informed about an increased risk of developing dermatitis when repeatedly or continuously exposed to nickel or irritants. Moreover, with our current knowledge, individuals with ichthyosis vulgaris should be protected against neonatal exposure to cats to prevent atopic dermatitis and should abstain from smoking to prevent asthma. Finally, they should be advised against excessive exposure to factors that decrease skin barrier functions and increase the risk of atopic dermatitis. © 2013 The Authors. BJD © 2013 British Association of Dermatologists.

  11. Whole-exome sequencing for diagnosis of hereditary ichthyosis.

    Science.gov (United States)

    Sitek, J C; Kulseth, M A; Rypdal, K B; Skodje, T; Sheng, Y; Retterstøl, L

    2018-02-14

    Hereditary ichthyosis constitutes a diverse group of cornification disorders. Identification of the molecular cause facilitates optimal patient care. We wanted to estimate the diagnostic yield of applying whole-exome sequencing (WES) in the routine genetic workup of inherited ichthyosis. During a 3-year-period, all ichthyosis patients, except X-linked and mild vulgar ichthyosis, consecutively admitted to a university hospital clinic were offered WES with subsequent analysis of ichthyosis-related genes as a first-line genetic investigation. Clinical and molecular data have been collected retrospectively. Genetic variants causative for the ichthyosis were identified in 27 of 34 investigated patients (79.4%). In all, 31 causative mutations across 13 genes were disclosed, including 12 novel variants. TGM1 was the most frequently mutated gene, accounting for 43.7% of patients suffering from autosomal recessive congenital ichthyosis (ARCI). Whole-exome sequencing appears an effective tool in disclosing the molecular cause of patients with hereditary ichthyosis seen in clinical practice and should be considered a first-tier genetic test in these patients. © 2018 European Academy of Dermatology and Venereology.

  12. Quality of life in Swedish children with congenital ichthyosis

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    Agneta Gånemo

    2010-05-01

    Full Text Available Congenital ichthyosis encompasses a large group of keratinizing disorders with widespread scaling and a variable degree of erythema. Little is known about the quality of life in children with congenital ichthyosis and the impact of the disease on their family. Fifteen children aged 5-16 years with lamellar ichthyosis, Netherton’s syndrome, epidermolytic hyperkeratosis or Harlequin ichthyosis, were investigated concerning the effect of their ichthyosis on their quality of life. This was measured with the established Children’s Dermatology Life Quality Index (CDLQI, and the Dermatitis Family Impact questionnaire (DFI modified by substituting the word ichthyosis for eczema. The questionnaires covered the preceding seven days and each had a maximum score of 30: the higher the score, the greater the Quality of Life impairment. The median score was 9.0 (range 2-19 for the CDLQI and 9.0 (range 3-21 for DFI. There was a significant correlation between the DFI and the CDLQI scores. The item in the CDLQI questionnaire that showed the highest score was “itchy, scratchy, sore or painful skin” and the most highly scored item in the DFI questionnaire was effect on “housework, e.g. washing, cleaning”; both items related to the children’s symptoms. The results of the study clearly establish that congenital ichthyosis impairs the quality of life of the affected children and their families.

  13. Clinico- Pathological Study Of Ichthyosis Vulgaris

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    Nandy Utpal

    1997-01-01

    Full Text Available A clinico- pathological study of 28 cases of ichthyosis vulgaris appeared with in the age of 5 years. The presence of the disease since birth was also found. While most (24 patients showed a diminution of severity in summer with an aggravation during winter, 4 patients followed the opposite seasonal pattern. Only in 4 patients, fine scales in the scalp were detected. One patient showed an affection of flexures. There was also a low occurrence of palmo- planter hyperkeratosis, follicular keratosis, fissuring of hands and feet and atopy.

  14. Harlequin Ichthyosis – A Case Report

    LENUS (Irish Health Repository)

    Ugezu, C H

    2017-07-01

    Harlequin Ichthyosis is a very rare genetic disorder affecting mainly the skin with severe morbidity and mortality. It affects both sexes with incidence of about 1 in 300,000 live births. Autosomal recessive inheritance has been inferred with mutation in ABCA 12 gene identified. Hence, genetic counseling and mutation screening of this gene should be considered in at-risk patients. Death usually occurred in the first 3 months of life due to sepsis, feeding problems and respiratory distress. With improved neonatal care and early introduction of retinoids, its survival rate has increased.

  15. [A case of skin autograft for skin ulcers in ichthyosis].

    Science.gov (United States)

    Li, Shiwei; Yang, Xiaodong; Liu, Lijun; Tang, Xueyang

    2017-10-28

    Ichthyosis refers to a group of skin diseases characterized by abnormal keratinization of the epidermis, resulting in dryness, roughness and scale of the skin. A girl with ichthyosis, who presented with skin ulcers and infection of the right dorsal foot, was admitted to our department. An autologous razor-thin skin grafting procedure was performed to repair the skin ulcers after debridement and vacuum sealing drain. After 8 months of follow-up, both the donor and recipient site healed well and there were no newly formed ulcers or infections. Although the skin quality of ichthyosis is poor, the lesion area can still be used as donor or recipient cite.

  16. A Case Report of Ichthyosis Lamellar Syndrome

    Directory of Open Access Journals (Sweden)

    Gh. Eshghi

    2014-04-01

    Full Text Available Introduction: Ichthyosis lamellar syndrome is a rare genodermatosis and in most families is inherited as an autosomal recessive trait because of transglutaminase-1 deficiency. Case Report: Our patient was a 6 year old girl and she was the result of consanguinity. She had large plate-like scales. The scales had mosaic-like pattern and erythroderma was absent. Tautness of her facial skin was associated with ectropion and eclabion and hypoplasia of auricular cartilages. She had scarring alopecia because of taut skin (specially at the periphery of scalp. She also had palmoplantar keratoderma and secondary nail dystrophy and thanked nails. Her parents also gave us the history of heat intolerance and it is because of interaepi-dermal constriction of sweat ducts. Our patient had the history of recurrent ear infections and it is because of accumulation of scales in the external ear. Conclusion: Our patient underwent a biopsy and based on our clinical findings her diagnosis was lamellar ichthyosis. (Sci J Hamadan Univ Med Sci 2014; 21 (1:76-79

  17. Lamellar ichthyosis (collodian baby with severe bilateral ectropion

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    Boparai M

    1988-01-01

    Full Text Available A case of lamellar ichthyosis (collodian baby, is being reported. Skin biopsy has confirmed the diagnosis. Severe bilateral ectropion of thee eyelids was the prominent feature. Management of such cases has been briefly discussed.

  18. Testis cancer. Ichthyosis constitutes a significant risk factor

    DEFF Research Database (Denmark)

    Lykkesfeldt, G; Bennett, P; Lykkesfeldt, A E

    1991-01-01

    Testis cancer and ichthyosis are both relatively rare diseases. Hence the finding of six individuals with both these conditions in a small population with testicular cancer is highly conspicuous and indicates some kind of connection among such persons. Despite the identical clinical appearances...... of their ichthyoses, three of the ichthyotic subjects had no measurable activity of the enzyme, steroid sulfatase (STS) in leucocytes, a distinct characteristic of recessive X-linked ichthyosis (RXLI). However, the remaining three subjects had normal STS activity, a strong indicator of autosomal dominant ichthyosis...... (ADI). The STS activity in patients with testicular cancer who do not have ichthyosis (N = 30) was also within the normal range. The patients with testicular cancer with no skin disease had elevated serum levels of 4-androstenedione (4-AD), follicle stimulating hormone (FSH), and luteinizing hormone...

  19. Ichthyosis vulgaris and pycnodysostosis: An unusual occurrence

    Directory of Open Access Journals (Sweden)

    Vinayak Y. Kshirsagar

    2012-11-01

    Full Text Available Pycnodysostosis is a rare autosomal recessive disorder whose generesponsible for this phenotype (CTSK, mapped to human chromosome1q21, code for the enzyme cathepsin K, a lysosomal cysteineprotease; with an estimated incidence of 1.7 per 1 million births. This clinical entity includes micromelic dwarfism, increased radiological bone density, dysplasia of the skull, acro-osteolysis, straightening of the mandibular angle and in some cases, dysplasia of the acromial end of the clavicle. Oral and maxillo-facial manifestations of this disease are very clear. Herein we reported a case of pycnodysostosis, showing short stature with widening of the sutures, unfused anterior and posterior fontanelles, crowding of teeth with dental caries and typical radiological features associated with ichthyosis vulgaris and palmoplantar keratoderma.

  20. Spectrum of Autosomal Recessive Congenital Ichthyosis in Scandinavia

    DEFF Research Database (Denmark)

    Hellström Pigg, Maritta; Bygum, Anette; Gånemo, Agneta

    2016-01-01

    Autosomal recessive congenital ichthyosis (ARCI) represents a heterogeneous group of rare disorders of cornification with 3 major subtypes: harlequin ichthyosis (HI), lamellar ichthyosis (LI) and congenital ichthyosiform erythroderma (CIE). A 4th subtype has also been proposed: pleomorphic...... ichthyosis (PI), characterized by marked skin changes at birth and subsequently mild symptoms. In nationwide screenings of suspected cases of ARCI in Denmark and Sweden, we identified 132 patients (age range 0.1-86 years) classified as HI (n = 7), LI (n = 70), CIE (n = 17) and PI (n = 38). At birth......-100%). A scoring (0-4) of ichthyosis/ery-thema past infancy showed widely different mean values in the subgroups: HI (3.2/3.1), LI (2.4/0.6), CIE (1.8/1.6), PI (1.1/0.3). Novel or recurrent mutations were found in 113 patients: TGM1 (n = 56), NIPAL4 (n = 15), ALOX12B (n = 15), ABCA12 (n = 8), ALOXE3 (n = 9), SLC27...

  1. X-linked ichthyosis along with epidermolysis bullosa

    Directory of Open Access Journals (Sweden)

    Shambulingappa Pallagatti

    2012-01-01

    Full Text Available Ichthyoses are a heterogenous group of hereditary keratinization disorders that share in common the accumulation & shedding of large amounts of hyperkeratotic epidermis.Early reports of ichthyosis in the Indian and Chinese literature date back to several hundred years. X-linked recessive ichthyosis (XLI is a common disorder of keratinization and affects males who inherit an X-chromosome having a steroid sulphatase genetic mutation.In the present communication we report a case of XLI and dystrophic epidermolysis bullosa in the same patient. To the best of our knowledge it has been reported only once before.

  2. Cervical intraepitheial neoplasm with ichthyosis uteri- A case report

    Directory of Open Access Journals (Sweden)

    Karishma Malla Vaidya

    2018-04-01

    Full Text Available A rare condition in which the entire surface of the endometrium is replaced by stratified squamous epithelium is called Ichthyosis uteri. Originally described as an endometrial response to iatrogenically-introduced caustic substances, similar changes have since been described in association with a variety of inflammatory conditions of the endometrium.Here we report a case of intraepithelial neoplasm III, with ichthyosis uteri. A 75-years-old female with hypetension, underwent total abdominal hysterectomy with bilateral salphingo-ophorectomy for watery discharge per vaginal since four months.

  3. Oral manifestations of lamellar ichthyosis: A rare case report

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    Keerthi K Nair

    2016-01-01

    Full Text Available The ichthyoses are a heterogeneous group of disorders with both inherited and acquired forms. Autosomal recessive congenital ichthyosis (ARCI is a heterogeneous group of disorders that present at birth with the generalized involvement of skin without other systemic manifestations. Lamellar itchthyosis (LI is a nonsyndromic itchthyosis, which comes under the umbrella of ARCI. Little is only known about the oral manifestations of this disorder. We report a case of LI with oral manifestations.

  4. Ichthyosis follicularis with alopecia and photophobia (IFAP syndrome

    Directory of Open Access Journals (Sweden)

    Rai Vandana

    2006-01-01

    Full Text Available A 12-year-old boy born of a nonconsanguineous marriage presented with dry rough skin and photophobia since birth. His growth and developmental milestones were normal and there was no history of any neurological problem, hearing deficit or scarring around the hair follicles. Cutaneous examination revealed diffuse thinning of scalp hair with loss of eyebrows and eyelashes and a sandpapery texture of the skin all over the body, suggestive of ichthyosis follicularis with alopecia and photophobia syndrome.

  5. A novel connexin 26 mutation in a patient diagnosed with keratitis-ichthyosis-deafness syndrome

    NARCIS (Netherlands)

    van Steensel, Maurice A. M.; van Geel, Michel; Nahuys, Marc; Smitt, J. Henk Sillevis; Steijlen, Peter M.

    2002-01-01

    Keratitis-ichthyosis-deafness syndrome is a rare disorder characterized by erythrokeratoderma, deafness, and keratitis. Scarring alopecia and squamous cell carcinoma can also occur. Most cases described so far were sporadic. Here we present evidence that keratitis-ichthyosis-deafness syndrome is

  6. A novel connexin 26 mutation in a patient diagnosed with keratitis-ichthyosis-deafness syndrome.

    NARCIS (Netherlands)

    Steensel, M.A.M. van; Geel, M. van; Nahuys, M.; Smitt, J.H.; Steijlen, P.M.

    2002-01-01

    Keratitis-ichthyosis-deafness syndrome is a rare disorder characterized by erythrokeratoderma, deafness, and keratitis. Scarring alopecia and squamous cell carcinoma can also occur. Most cases described so far were sporadic. Here we present evidence that keratitis-ichthyosis-deafness syndrome is

  7. Autosomal recessive ichthyosis with hypotrichosis syndrome: further delineation of the phenotype

    NARCIS (Netherlands)

    Avrahami, L.; Maas, S.; Pasmanik-Chor, M.; Rainshtein, L.; Magal, N.; Smitt, J. H. S.; van Marle, J.; Shohat, M.; Basel-Vanagaite, L.

    2008-01-01

    Autosomal recessive ichthyosis with hypotrichosis (ARIH) syndrome, which is characterized by congenital ichthyosis, abnormal hair and corneal involvement, has recently been shown in one consanguineous Israeli Arab family to be caused by a mutation in the ST14 gene, which encodes serine protease

  8. Ichthyosis associated with rickets in two Indian children

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    Dimple Kothari

    2013-01-01

    Full Text Available We wish to report two cases of rickets due to vitamin D deficiency secondary to underlying ichthyotic skin disorder. The first case is of an 8-year-old male with history of multiple fluid-filled lesions over the body that would rupture to heal with thickening and scaling of skin, suggestive of epidermolytic hyperkeratosis, and the second is of a 14-year-old female with thick, large, quadrilateral scales over the extremities and back clinically consistent with lamellar ichthyosis. Both showed improvement with parenteral vitamin D3 and oral calcium supplements in addition to topical emollients.

  9. Keratitis-Ichthyosis-Deafness syndrome: A rare congenital disorder

    Science.gov (United States)

    Shanker, Vinay; Gupta, Mudita; Prashar, Aditi

    2012-01-01

    Keratitis-Icthyosis-Deafness syndrome is a rare congenital disorder characterized by keratitis, ichthyosis, and deafness. We report a 13 year old female child who presented with diffuse alopecia of the scalp and body. There was erythrokeratoderma of face and discrete hyperkeratotic hyperpigmented papulo plaque lesions on the body. Patient also had reticulate hyperkeratosis of palms and soles. There was history of recurrent episodes of folliculitis over the scalp and body. There was no evidence of any malignancy. Eye involvement in the form of bilateral vascularising keratitis was present. There was bilateral mixed hearing loss. PMID:23130264

  10. Keratitis-Ichthyosis-Deafness syndrome: A rare congenital disorder

    Directory of Open Access Journals (Sweden)

    Vinay Shanker

    2012-01-01

    Full Text Available Keratitis-Icthyosis-Deafness syndrome is a rare congenital disorder characterized by keratitis, ichthyosis, and deafness. We report a 13 year old female child who presented with diffuse alopecia of the scalp and body. There was erythrokeratoderma of face and discrete hyperkeratotic hyperpigmented papulo plaque lesions on the body. Patient also had reticulate hyperkeratosis of palms and soles. There was history of recurrent episodes of folliculitis over the scalp and body. There was no evidence of any malignancy. Eye involvement in the form of bilateral vascularising keratitis was present. There was bilateral mixed hearing loss.

  11. Clinical Observation of a Child with KID (Keratitis-Ichthyosis-Deafness Syndrome

    Directory of Open Access Journals (Sweden)

    V.A. Klymenko

    2015-09-01

    Full Text Available A clinical case of keratitis-ichthyosis-deafness (KID syndrome in an infant is described. The article familia-rizes pediatricians and family doctors with difficulties in the diagnosis of this rare genetic disease in infants.

  12. X-linked ichthyosis associated with psychosis and behavioral abnormalities: a case report.

    Science.gov (United States)

    Malik, Amna; Amer, Ahmed Bait; Salama, Mohammed; Haddad, Bander; Alrifai, Muhammad T; Balwi, Mohammed Al; Davies, William; Eyaid, Wafaa

    2017-09-22

    X-linked ichthyosis is a dermatological condition caused by deficiency for the enzyme steroid sulfatase. Previously, X-linked ichthyosis/steroid sulfatase deficiency has been associated with developmental and neurological phenotypes. Here, we show for the first time, that X-linked ichthyosis may be comorbid with an additional psychiatric phenotype (psychosis). We report the case of an 11-year-old Saudi Arabian boy with X-linked ichthyosis associated with psychosis, mental retardation, autism spectrum disorder, inattentive attention deficit hyperactivity disorder, and epilepsy. Genetic analysis revealed a 1.68 Mb deletion encompassing STS in 95% of cells while biochemical analysis revealed correspondingly low steroid sulfatase activity consistent with a diagnosis of X-linked ichthyosis. The psychotic symptoms could be reasonably well controlled by administration of an atypical antipsychotic. This report describes a case of comorbid X-linked ichthyosis and psychosis (most closely corresponding to early-onset schizophrenia) for the first time, and suggests that deficiency for steroid sulfatase and contiguous genes may increase vulnerability to psychosis as well as other psychological disorders.

  13. Porphyria Cutanea Tarda Presenting with Scleroderma, Ichthyosis, Alopecia, and Vitiligo

    Directory of Open Access Journals (Sweden)

    Megan E. MacGillivray

    2018-05-01

    Full Text Available Porphyria cutanea tarda (PCT is a cutaneous porphyria that presents later in life with cutaneous findings in sun-exposed sites. We report a complex case of PCT in a 67-year-old woman with an unusual constellation of cutaneous findings: scleroderma, acquired ichthyosis, and nonscarring alopecia. Possible triggers for her PCT include tamoxifen treatment for breast cancer and carrier status of the hemochromatosis gene. High-dose chloroquine was used to successfully achieve clinical remission and normalize her uroporphyrins. While on chloroquine she developed extensive classic vitiligo. It is not clear if this is another feature of her complex and unusual PCT, or a consequence of her antimalarial therapy.

  14. Generalized morphea in a child with harlequin ichthyosis: a rare association.

    Science.gov (United States)

    Giacomin, Maria F A; França, Camila M P; Oliveira, Zilda N P; Machado, Maria C R; Sallum, Adriana M E; Silva, Clovis A

    2016-01-01

    Harlequin ichthyosis (HI) is a severe and rare hereditary congenital skin disorder characterized by excessive dryness, ectropion and eclabion. The association of ichthyosis with systemic sclerosis has been described in only three children. No patient with generalized morphea (GM) associated with harlequin ichthyosis was described. A 4-years and 6-months girl, diagnosed with harlequin ichthyosis based on diffuse cutaneous thickening, scaling, erythema, ectropion and eclabium since the first hours of birth was described. She was treated with acitretin (1.0mg/kg/day) and emollient cream. At 3 years and 9 months, she developed muscle contractures with pain on motion and limitation in elbows and knees, and diffuse sclerodermic plaques on the abdomen, back, suprapubic area and lower limbs. Skin biopsy showed rectified epidermis and mild hyperorthokeratosis, reticular dermis with perivascular and periadnexal infiltrates of lymphocytes and mononuclear cells, and reticular dermis and sweat gland sclerosis surrounded by a dense collagen tissue, compatible with scleroderma. The patient fulfilled the GM subtype criteria. Methotrexate and prednisone were introduced. At 4 years and 3 months, new scleroderma lesions occurred and azathioprine was associated with previous therapy, with no apparent changes after two months. A case of harlequin ichthyosis associated with a GM was reported. The treatment of these two conditions is a challenge and requires a multidisciplinary team. Copyright © 2014 Elsevier Editora Ltda. All rights reserved.

  15. Clinico-epidemiological study of congenital ichthyosis in a tertiary care center of Eastern India

    Directory of Open Access Journals (Sweden)

    Arghyaprasun Ghosh

    2017-01-01

    Full Text Available Background: Congenital ichthyoses comprises various specific genetic diseases and can range from mild to very severe presentation. Furthermore, these may be associated with various syndromes. There is scanty data regarding the demographic profile and clinical features of patients with congenital ichthyosis in India. Aims and Objectives: The aim is to evaluate the epidemiology and clinical characteristics of various types of congenital ichthyoses. Materials and Methods: The study was conducted for 1 year from April 2013 to March 2014. Patients were evaluated for epidemiological profile and clinical features. Results: During the study of 1 year, 106 patients of congenital ichthyoses were identified. The most common of the various ichthyoses was ichthyosis vulgaris, followed by lamellar ichthyosis, X-linked recessive ichthyosis. One case of Netherton syndrome and one of ichthyosis hystrix were also identified. Conclusion: Various types of congenital ichthyoses present with different clinical features which range from mild to severe. These present with significant psychological stress to both patients and their families. Furthermore, all these diseases have significant implications of transmission to their offspring.

  16. A Topic Diathesis In Hereditary Ichthyosis Patients Attending A Tertiary Health Care Center In Saudi Arabia

    Directory of Open Access Journals (Sweden)

    Al-Akloby Omar M Al-Amro

    2004-01-01

    Full Text Available The occurrence of atopic diathesis in hereditary ichthyosis (HI has not been documented in Saudi patients. The atopic manifestations in histopathologically confirmed HI patients attending the dermatology clinic of king Fahad Hospital of the University at Al-Khobar city, Saudi Arabia is discussed in this study. From the dermatology OPD logbook, all Saudi patients with confirmed HI seen between January 1990 to December 1995 were included in the study. The findings regarding atopic manifestations were extracted into data collection forms and analyzed. During the 5 year study period, 10,455 new cases were seen in our dermatology OPD. Of these, 61 had hereditary icthyosis, with 37 males and 24 females with a male to female ratio of 1.5:1. Thus, the frequency of HI among Saudi hospital attendees was 6 per 1000 new cases. The type of HI was ichthyosis vulgaris in 25 (41% patients, X-linked recessive ichthyosis in 11 (18%, lamellar ichthyosis in 4(7%, bullous ichthyosiform erythroderma in 2 (3% and nonbullous ichthyosiform erythroderma was seen in 19 (31%. Generalized pruritus was present in 49 (80% cases, atopic dermatitis in , elevated serum IgE level was noted in 27 and bronchial asthma in 3 cases. Dandruff was reported in 24 cases, keratosis pilaris in15, recurrent skin infection in 7. Combination of hereditary ichthyosis, generalized pruritus and high serum IGE level was reported in 27 (44.3% patient.

  17. Ichthyosis hystrix disease or verrucous epidermal nevus (a retrospective analysis of 20-year observation

    Directory of Open Access Journals (Sweden)

    V. D. El'kin

    2017-01-01

    Full Text Available We present a  clinical case of ichthyosis hystrix, a rare genetic ichthyosiform dermatosis. Specifics of the disease are related to the complexity of differential diagnosis and verification of the diagnosis, because clinical manifestation of ichthyosis hystrix is similar with that of verrucous epidermal nevus. Clinical particulars of both nosologies are characterized by bizarre and widespread skin lesions of spicular hyperkeratotic growth, located along Blaschko lines. The exclusive feature of the clinical case described is a  long-term (20 years clinical and laboratory monitoring of the patient. The literature review provides brief information on etiology, pathogenesis, clinical features, differential diagnosis, and pathomorphology of ichthyosis hystrix and verrucous epidermal nevus.

  18. Skin barrier properties in patients with recessive X-linked ichthyosis

    DEFF Research Database (Denmark)

    Johansen, J D; Ramsing, D; Vejlsgaard, G

    1995-01-01

    increased in controls compared to ichthyosis patients, when evaluated by TEWL. When evaluated by erythema index a statistically significant increase in redness was found in controls, but not in ichthyosis patients. Electrical capacitance, reflecting skin hydration, was significantly reduced in RXLI patients......Patients with X-linked recessive ichthyosis (RXLI) were studied as a model of the effect of disturbed epidermal lipid composition on skin barrier function. Thirteen patients with RXLI and 15 age- and sex-matched controls were patch-tested with sodium lauryl sulphate (SLS) 0.5% for 24 h. Basal skin...... properties and skin response to SLS were studied by measurement of transepidermal water loss (TEWL), electrical capacitance and erythema index. No statistically significant difference in basal TEWL was found between the two groups. The skin response to SLS was found to be statistically significantly...

  19. Ichthyosis bullosa of Siemens: Response to topical tazarotene

    Directory of Open Access Journals (Sweden)

    Rajiv S

    2006-01-01

    Full Text Available In 1937, Siemens described a Dutch family with superficial blistering, flexural hyperkeratosis, and characteristic mauserung appearance. Since then, less than 20 kindreds with this condition have been described in the English dermatologic literature. A 14-year-old boy presented with history of recurrent blistering and peeling of skin since the age of 1 month, predominantly seen over limbs and trunk, often associated with secondary infection. His mother also had similar symptoms from childhood. On examination, the child had typical mauserung peeling of the skin and dirty gray hyperkeratosis in a rippled pattern over flexures. Skin biopsy from the boy showed intracorneal blistering with epidermolytic hyperkeratosis in the upper spinous layers. The typical history and clinical features along with characteristic histological findings confirmed our diagnosis of ichthyosis bullosa of Siemens. It must be differentiated from other conditions with epidermolytic hyperkeratosis and skin peeling, such as bullous ichthyosiform erythroderma of Brocq and peeling skin syndrome. Our patient responded well to 0.05% topical tazarotene gel over four weeks.

  20. Bilateral lower extremity hyperkeratotic plaques: a case report of ichthyosis vulgaris

    Directory of Open Access Journals (Sweden)

    Leight H

    2015-09-01

    Full Text Available Hayley Leight, Zachary Zinn, Omid JalaliDepartment of Dermatology, West Virginia University, Morgantown, WV, USA Abstract: Here, we report a case of a middle-aged woman presenting with severe, long-standing, hyperkeratotic plaques of the lower extremities unrelieved by over-the-counter medications. Initial history and clinical findings were suggestive of an inherited ichthyosis. Ichthyoses are genetic disorders characterized by dry scaly skin and altered skin-barrier function. A diagnosis of ichthyosis vulgaris was confirmed by histopathology. Etiology, prevalence, and treatment options are discussed. Keywords: filaggrin gene, FLG, profilaggrin, keratohyalin granules, hyperkeratosis

  1. Acquired Ichthyosis Triggered by an Osseous Hemangiopericytoma: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Aikaterini Patsatsi

    2014-01-01

    Full Text Available Ichthyoses are a heterogeneous group of cutaneous keratinization disorders that can be congenital or acquired. Apart from neoplastic disorders, the acquired form of ichthyosis (AI has been associated with a variety of diseases including infections, autoimmune/inflammatory and endocrine/metabolic diseases as well as nutritional conditions, medications and others. However, malignancy accounts for half of the reported cases, most commonly including lymphoproliferative disorders. We present a case of AI as a paraneoplastic skin manifestation of a primary, osseous hemangiopericytoma (HP accompanied by multiple liver metastases. We also review the literature and discuss the necessity of investigating underlying diseases, especially malignancy, when adult-onset ichthyosis arises.

  2. Skin barrier properties in patients with recessive X-linked ichthyosis

    DEFF Research Database (Denmark)

    Johansen, J D; Ramsing, D; Vejlsgaard, G

    1995-01-01

    Patients with X-linked recessive ichthyosis (RXLI) were studied as a model of the effect of disturbed epidermal lipid composition on skin barrier function. Thirteen patients with RXLI and 15 age- and sex-matched controls were patch-tested with sodium lauryl sulphate (SLS) 0.5% for 24 h. Basal skin...

  3. Identification of Mutations in SDR9C7 in 6 Families with Autosomal Recessive Congenital Ichthyosis

    DEFF Research Database (Denmark)

    Hotz, A; Fagerberg, C; Vahlquist, A

    2018-01-01

    Autosomal recessive congenital ichthyosis (ARCI) is a heterogeneous group of disorders of keratinization. To date, ARCI has been associated with following genes: ABCA12, ALOX12B, ALOXE3, CERS3, CYP4F22, NIPAL4, TGM1, PNPLA1 and recently SDR9C7 and SULT2B1.(1-6) Furthermore, seven patients from...

  4. Short- and medium-term efficacy of specific hydrotherapy in inherited ichthyosis.

    Science.gov (United States)

    Bodemer, C; Bourrat, E; Mazereeuw-Hautier, J; Boralevi, F; Barbarot, S; Bessis, D; Blanchet-Bardon, C; Bourdon-Lanoy, E; Stalder, J-F; Ribet, V; Guerrero, D; Sibaud, V

    2011-11-01

    Management of inherited ichthyoses is symptomatic. Despite treatment, skin symptoms have a major impact on patients' quality of life (QoL). To assess the short- and medium-term efficacy of hydrotherapy on QoL and clinical symptoms of patients with inherited ichthyosis. In this 9-month prospective, open-label, multicentre study, 20 children and 24 adults with ichthyosis were enrolled in several French reference and competence centres, 2 months before undergoing a 3-week treatment with specific hydrotherapeutic management at Avène Hydrotherapy Centre. At baseline (2 months before hydrotherapy), beginning (D0) and end of hydrotherapy (D18), and 3 and 6 months later at the reference and competence centres, patients self-assessed QoL using the Dermatology Life Quality Index (DLQI) or its paediatric version (Children's DLQI), and investigators evaluated ichthyosis severity using a specific clinical ichthyosis score. The DLQI scores were significantly improved not only at the end of the hydrotherapy treatment (-56% vs. baseline; mean ± SD 3·59 ± 4·30 at D18 vs. 8·35 ± 5·71 at D0; P hydrotherapy (-26% vs. baseline; mean ± SD 5·21 ± 5·11 vs. 6·89 ± 5·38; P = 0·03) (primary criterion). Clinical symptoms were also significantly improved at all post-treatment visits, with a decrease of the mean clinical ichthyosis score by -38% between D0 and D18, by -30% at 3 months and by -31% at 6 months vs. baseline. A 3-week treatment at Avène Hydrotherapy Centre provided significant and persisting improvement of QoL and clinical symptoms in patients with inherited ichthyoses. © 2011 The Authors. BJD © 2011 British Association of Dermatologists.

  5. A novel mutation in the L12 domain of keratin 1 is associated with mild epidermolytic ichthyosis

    NARCIS (Netherlands)

    Bolling, M. C.; Bladergroen, R. S.; van Steensel, M. A. M.; Willemsen, M.; Jonkman, M. F.; van Geel, M.

    P>Background Epidermolytic ichthyosis (EI), previously termed bullous congenital ichthyosiform erythroderma or epidermolytic hyperkeratosis, is a clinically heterogeneous genodermatosis caused by mutations in the genes encoding the suprabasal keratins 1 and 10. Classical EI is clinically

  6. Paraneoplastic addisonian pigmentation and acquired ichthyosis as presenting features of multiple myeloma

    International Nuclear Information System (INIS)

    Dar, N.R.; Raza, N.

    2010-01-01

    Black brown hyperpigmentation of the mucosae, sun exposed skin, palmar creases and frictional sites (Addisonian pigmentation) is characteristic of Addison disease. However, it can also occur as a para neoplastic manifestation of tumours like bronchogenic carcinoma. Acquired ichthyosis starts later in life and can also be a para neoplastic presentation.We report a unique combination of para neoplastic Addisonian pigmentation and acquired ichthyosis as presenting features in a patient with undiagnosed multiple myeloma. To the best of our knowledge this combination of para neoplastic dermatosis has not been documented before in multiple myeloma. It is concluded that the presence of more than one suspicious dermatosis may be an indicator of being para neoplastic requiring necessary work-up. (author)

  7. AIF programs supporting industry self-improvement

    International Nuclear Information System (INIS)

    Szalay, R.A.; Bivens, A.C.

    1985-01-01

    The Atomic Industrial Forum (AIF) report ''Nuclear Power in America's Future'' identifies those actions that must be taken by both government and industry if the nuclear power option is to be preserved. This includes initiatives that: 1) reduce construction costs and lead times; 2) establish a national energy policy that reflects the role of electricity and the nuclear power component; 3) provide better financial arrangements; 4) reform licensing and regulation; 5) enhance the light water reactor design; 6) inform the public on the benefits and contributions of nuclear power; and 7) complete the fuel cycle. In attempting to solve nuclear power's problems, the AIF has programs that support and push these needed actions. Much of the initiative must be taken by industry to enhance the nuclear power option. This paper discusses some AIF initiatives and programs that support industry self-improvement

  8. A de novo variant in the ASPRV1 gene in a dog with ichthyosis.

    Science.gov (United States)

    Bauer, Anina; Waluk, Dominik P; Galichet, Arnaud; Timm, Katrin; Jagannathan, Vidhya; Sayar, Beyza S; Wiener, Dominique J; Dietschi, Elisabeth; Müller, Eliane J; Roosje, Petra; Welle, Monika M; Leeb, Tosso

    2017-03-01

    Ichthyoses are a heterogeneous group of inherited cornification disorders characterized by generalized dry skin, scaling and/or hyperkeratosis. Ichthyosis vulgaris is the most common form of ichthyosis in humans and caused by genetic variants in the FLG gene encoding filaggrin. Filaggrin is a key player in the formation of the stratum corneum, the uppermost layer of the epidermis and therefore crucial for barrier function. During terminal differentiation of keratinocytes, the precursor profilaggrin is cleaved by several proteases into filaggrin monomers and eventually processed into free amino acids contributing to the hydration of the cornified layer. We studied a German Shepherd dog with a novel form of ichthyosis. Comparing the genome sequence of the affected dog with 288 genomes from genetically diverse non-affected dogs we identified a private heterozygous variant in the ASPRV1 gene encoding "aspartic peptidase, retroviral-like 1", which is also known as skin aspartic protease (SASPase). The variant was absent in both parents and therefore due to a de novo mutation event. It was a missense variant, c.1052T>C, affecting a conserved residue close to an autoprocessing cleavage site, p.(Leu351Pro). ASPRV1 encodes a retroviral-like protease involved in profilaggrin-to-filaggrin processing. By immunofluorescence staining we showed that the filaggrin expression pattern was altered in the affected dog. Thus, our findings provide strong evidence that the identified de novo variant is causative for the ichthyosis in the affected dog and that ASPRV1 plays an essential role in skin barrier formation. ASPRV1 is thus a novel candidate gene for unexplained human forms of ichthyoses.

  9. A de novo variant in the ASPRV1 gene in a dog with ichthyosis.

    Directory of Open Access Journals (Sweden)

    Anina Bauer

    2017-03-01

    Full Text Available Ichthyoses are a heterogeneous group of inherited cornification disorders characterized by generalized dry skin, scaling and/or hyperkeratosis. Ichthyosis vulgaris is the most common form of ichthyosis in humans and caused by genetic variants in the FLG gene encoding filaggrin. Filaggrin is a key player in the formation of the stratum corneum, the uppermost layer of the epidermis and therefore crucial for barrier function. During terminal differentiation of keratinocytes, the precursor profilaggrin is cleaved by several proteases into filaggrin monomers and eventually processed into free amino acids contributing to the hydration of the cornified layer. We studied a German Shepherd dog with a novel form of ichthyosis. Comparing the genome sequence of the affected dog with 288 genomes from genetically diverse non-affected dogs we identified a private heterozygous variant in the ASPRV1 gene encoding "aspartic peptidase, retroviral-like 1", which is also known as skin aspartic protease (SASPase. The variant was absent in both parents and therefore due to a de novo mutation event. It was a missense variant, c.1052T>C, affecting a conserved residue close to an autoprocessing cleavage site, p.(Leu351Pro. ASPRV1 encodes a retroviral-like protease involved in profilaggrin-to-filaggrin processing. By immunofluorescence staining we showed that the filaggrin expression pattern was altered in the affected dog. Thus, our findings provide strong evidence that the identified de novo variant is causative for the ichthyosis in the affected dog and that ASPRV1 plays an essential role in skin barrier formation. ASPRV1 is thus a novel candidate gene for unexplained human forms of ichthyoses.

  10. Role of molecular testing in the multidisciplinary diagnostic approach of ichthyosis.

    Science.gov (United States)

    Diociaiuti, Andrea; El Hachem, May; Pisaneschi, Elisa; Giancristoforo, Simona; Genovese, Silvia; Sirleto, Pietro; Boldrini, Renata; Angioni, Adriano

    2016-01-13

    The term ichthyosis describes a generalized disorder of cornification characterized by scaling and/or hyperkeratosis of different skin regions. Mutations in a broad group of genes related to keratinocyte differentiation and epidermal barrier function have been demonstrated to play a causative role in disease development. Ichthyosis may be classified in syndromic or non-syndromic forms based on the occurrence or absence of extracutaneous signs. In this setting, the diagnosis of ichthyosis is an integrated multistep process requiring a multidisciplinary approach in order to formulate the appropriate diagnostic hypothesis and to address the genetic testing. Due to the complex features of the different ichthyoses and the high number of genes involved we have investigated a group of 64 patients, affected by syndromic and non-syndromic diseases, using Next Generation Sequencing as a new tool for the molecular diagnosis. Using this innovative molecular approach we were able to find pathogenic mutations in 53 out of 64 patients resulting in 82.8 % total detection rate. An interesting result from the analysis of the data is the high rate of novel sequence variations found compared to known mutations and the relevant rate of homozygous mutations. The possibility to analyze a large number of genes associated with various diseases allows to study cases with phenotypes not well-determined, giving the opportunity to make new genotype-phenotype correlation. In some cases there were discrepancies between clinical features and histology or electron microscopy and only molecular analysis allowed to definitively resolve the diagnostic dilemma. The genetic diagnosis of ichthyosis leads to a more accurate and effective genetic counseling, allowing correct evaluation of the risk of recurrence, particularly in families with consanguineous background.

  11. A Novel Mutation in the Transglutaminase-1 Gene in an Autosomal Recessive Congenital Ichthyosis Patient

    Directory of Open Access Journals (Sweden)

    D. Vaigundan

    2014-01-01

    Full Text Available Structure-function implication on a novel homozygous Trp250/Gly mutation of transglutaminase-1 (TGM1 observed in a patient of autosomal recessive congenital ichthyosis is invoked from a bioinformatics analysis. Structural consequences of this mutation are hypothesized in comparison to homologous enzyme human factor XIIIA accepted as valid in similar structural analysis and are projected as guidelines for future studies at an experimental level on TGM1 thus mutated.

  12. Dental Treatments under the General Anesthesia in a Child with Keratitis, Ichthyosis, and Deafness Syndrome

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    Sera Sımsek Derelioglu

    2013-01-01

    Full Text Available KID syndrome is a rare genodermatosis characterized by keratitis, ichthyosis, and sensorineural deafness. Although the dermatological, ophthalmologic, and sensorineural defects are emphasized in the literature, oral and dental evaluations are so superficial. In this case report, dental and oral symptoms of a three year and five months old boy with KID syndrome, suffering severe Early Childhood Caries (s-ECC and dental treatments done under General Anesthesia (GA were reported.

  13. Kallmann syndrome and ichthyosis: a case of contiguous gene deletion syndrome

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    Irene Berges-Raso

    2017-09-01

    Full Text Available Kallmann syndrome is a genetically heterogeneous form of hypogonadotropic hypogonadism caused by gonadotropin-releasing hormone deficiency and characterized by anosmia or hyposmia due to hypoplasia of the olfactory bulbs; osteoporosis and metabolic syndrome can develop due to longstanding untreated hypogonadism. Kallmann syndrome affects 1 in 10 000 men and 1 in 50 000 women. Defects in 17 genes, including KAL1, have been implicated. Kallmann syndrome can be associated with X-linked ichthyosis, a skin disorder characterized by early onset dark, dry, irregular scales affecting the limb and trunk, caused by a defect of the steroid sulfatase gene (STS. Both KAL1 and STS are located in the Xp22.3 region; therefore, deletions in this region cause a contiguous gene syndrome. We report the case of a 32-year-old man with ichthyosis referred for evaluation of excessive height (2.07 m and weight (BMI: 29.6 kg/m2, microgenitalia and absence of secondary sex characteristics. We diagnosed Kallmann syndrome with ichthyosis due to a deletion in Xp22.3, a rare phenomenon.

  14. X-linked ichthyosis: clinical and molecular findings in 35 Italian patients.

    Science.gov (United States)

    Diociaiuti, Andrea; Angioni, Adriano; Pisaneschi, Elisa; Alesi, Viola; Zambruno, Giovanna; Novelli, Antonio; El Hachem, May

    2018-04-19

    Recessive X-linked ichthyosis (XLI), the second most common ichthyosis, is caused by mutations in the STS gene encoding the steroid sulfatase enzyme. A complete deletion of the STS gene is found in 85-90% of cases. Rarely, larger deletions involving contiguous genes are detected in syndromic patients. We report the clinical and molecular genetic findings in a series of 35 consecutive Italian male patients. All patients underwent molecular testing by MLPA or aCGH, followed, in case of negative results, by next generation sequencing analysis. Neuropsychiatric, ophthalmological and pediatric evaluations were also performed. Our survey showed a frequent presence of disease manifestations at birth (42.8%). Fold and palmoplantar surfaces were involved in 18 (51%) and 7 (20%) patients, respectively. Fourteen patients (42%) presented neuropsychiatric symptoms, including attention deficit hyperactivity disorder and motor disabilities. In addition, two patients with mental retardation were shown to be affected by a contiguous gene syndrome. Twenty-seven patients had a complete STS deletion, one a partial deletion and 7 carried missense mutations, two of which previously unreported. In addition, a de novo STS deletion was identified in a sporadic case. The frequent presence of palmoplantar and fold involvement in XLI should be taken into account when considering the differential diagnosis with ichthyosis vulgaris. Our findings also underline the relevance of involving the neuropsychiatrist in the multidisciplinary management of XLI. Finally, we report for the first time a de novo mutation which shows that STS deletion can also occur in oogenesis. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  15. Ichthyosis Follicularis with Atrichia and Photophobia Syndrome: First Case Report in Thailand

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    Rattanavalai Nitiyarom

    2017-05-01

    Full Text Available We herein describe the first reported case of Ichthyosis Follicularis with Atrichia and Photophobia (IFAP syndrome in Thailand. A 6-year-old boy presented with a history of photophobia since 1 month of age. Then he developed widespread follicular hyperkeratotic papules and subtotal non-scarring alopecia by the age of 10 months and 5 years, respectively. Sparse eyelashes and nail dystrophy were also noted. No neurological abnormalities, systemic involvement, and hearing impairment were observed. The clinical manifestations were consistent with IFAP syndrome, although genetic testing did not confirm the diagnosis of this rare disorder.

  16. A rare case of acute intermittent porphyria with ichthyosis vulgaris in a young boy

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    Garima Agrawal Varshney

    2018-01-01

    Full Text Available Acute intermittent porphyria (AIP and ichthyosis vulgaris both are autosomal dominant disorders with incomplete penetrance caused by the deficiency of porphobilinogen deaminase enzyme and filaggrin protein, respectively. We report a rare case of a 9-year-old boy having two genetic diseases with an unclear association. An acute attack of AIP is characterized by gastrointestinal symptoms and neuropsychiatric manifestations. Although rare in the first decade of life, the presence of reddish urine with a typical presentation such as abdominal pain, hypertension, seizure, and paresthesias lead us to the diagnosis of AIP. The precipitating factor in the present case was prolonged fasting in Ramadan.

  17. THE CASE OF HERPETIC ECZEMA IN A CHILD WITH CONGENITAL ICHTHYOSIS AND ATOPIC DERMATITIS

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    A. S. Stadnikova

    2017-01-01

    Full Text Available The case of the development of herpetic eczema (Kaposi's eczema is presented against the background of congenital ichthyosis and atopic dermatitis. It has been shown that the presence of atopic diseases such as allergic rhinitis and pollinosis, sensitization to many common allergens, and a positive family history of atopic dermatitis are factors of a more severe course of Kaposi's eczema. The presented clinical observation of the child with Kaposi's eczema showed that early diagnosis and timely initiated complex  therapy are the determining factors of a favorable prognosis of the disease.

  18. Dysplastic Ichthyosis Uteri-like changes of the entire endometrium associated with a squamous cell carcinoma of the uterine cervix

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    Fadare Oluwole

    2006-05-01

    Full Text Available Abstract Ichthyosis uteri is an exceedingly rare condition in which the entire surface of the endometrium is replaced by stratified squamous epithelium. Originally described as an endometrial response to iatrogenically-introduced caustic substances, similar changes have since been described in association with a variety of inflammatory conditions of the endometrium. We describe herein a heretofore undescribed example of a moderately differentiated squamous cell carcinoma of the uterine cervix associated with extensive ichthyosis uteri-like changes of the entire adjacent endometrium. Additionally, the squamous epithelium of the latter also showed multifocal changes diagnostic of a low-grade squamous intraepithelial lesion. The potential genesis of this composite of findings is discussed, as is the neoplastic potential of ichthyosis uteri. It is concluded that a squamous cell carcinoma of the cervix extended proximally into the endometrium, and that there was a colonization of a pre-existing ichthyosis uteri by associated human papillomavirus. The possibility of significant cervical pathology should be considered when plaques of squamous epithelium with low grade dysplastic changes are identified in an endometrial biopsy or curettage.

  19. A Defect in NIPAL4 Is Associated with Autosomal Recessive Congenital Ichthyosis in American Bulldogs.

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    Margret L Casal

    Full Text Available Autosomal recessive congenital ichthyosis in the American bulldog is characterized by generalized scaling and erythema with adherent scale on the glabrous skin. We had previously linked this disorder to NIPAL4, which encodes the protein ichthyin. Sequencing of NIPAL4 revealed a homozygous single base deletion (CanFam3.1 canine reference genome sequence NC_06586.3 g.52737379del, the 157th base (cytosine in exon 6 of NIPAL4 as the most likely causative variant in affected dogs. This frameshift deletion results in a premature stop codon producing a truncated and defective NIPAL4 (ichthyin protein of 248 amino acids instead of the wild-type length of 404. Obligate carriers were confirmed to be heterozygous for this variant, and 150 clinically non-affected dogs of other breeds were homozygous for the wild-type gene. Among 800 American bulldogs tested, 34% of clinically healthy dogs were discovered to be heterozygous for the defective allele. More importantly, the development of this canine model of autosomal recessive congenital ichthyosis will provide insight into the development of new treatments across species.

  20. FATP4 missense and nonsense mutations cause similar features in Ichthyosis Prematurity Syndrome

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    Dahl Niklas

    2011-03-01

    Full Text Available Abstract Background Ichthyosis Prematurity Syndrome (IPS is an autosomal recessive disorder characterized by premature birth, non-scaly ichthyosis and atopic manifestations. The disease was recently shown to be caused by mutations in the gene encoding the fatty acid transport protein 4 (FATP4 and a specific reduction in the incorporation of very long chain fatty acids (VLCFA into cellular lipids. Findings We screened probands from five families segregating IPS for mutations in the FATP4 gene. Four probands were compound heterozygous for four different mutations of which three are novel. Four patients were heterozygous and one patient homozygous for the previously reported non-sense mutation p.C168X (c.504c > a. All patients had clinical characteristics of IPS and a similar clinical course. Conclusions Missense mutations and non-sense mutations in FATP4 are associated with similar clinical features suggesting that missense mutations have a severe impact on FATP4 function. The results broaden the mutational spectrum in FATP4 associated with IPS for molecular diagnosis of and further functional analysis of FATP4.

  1. Communication Styles of Interactive Tools for Self-Improvement

    OpenAIRE

    Niess, Jasmin; Diefenbach, Sarah

    2016-01-01

    Background Interactive products for self-improvement (e.g., online trainings to reduce stress, fitness gadgets) have become increasingly popular among consumers and healthcare providers. In line with the idea of positive computing, these tools aim to support their users on their way to improved well-being and human flourishing. As an interdisciplinary domain, the design of self-improvement technologies requires psychological, technological, and design expertise. One needs to know how to suppo...

  2. Threshold levels of 25-hydroxyvitamin D and parathyroid hormone for impaired bone health in children with congenital ichthyosis and type IV and V skin.

    Science.gov (United States)

    Sethuraman, G; Sreenivas, V; Yenamandra, V K; Gupta, N; Sharma, V K; Marwaha, R K; Bhari, N; Irshad, M; Kabra, M; Thulkar, S

    2015-01-01

    Patients with congenital ichthyosis, especially those with darker skin types, are at increased risk of developing vitamin D deficiency and rickets. The relationships between 25-hydroxyvitamin D [25(OH)D], parathyroid hormone (PTH) and bone health have not been studied previously, in ichthyosis. To determine the threshold levels of 25(OH)D and PTH for impaired bone health in children with congenital ichthyosis. In this cross-sectional study, 119 children with ichthyosis and 168 controls were recruited. Serum 25(OH)D, PTH, calcium, phosphate and alkaline phosphatase (ALP) were measured. Radiological screening for rickets was carried out only in children with ichthyosis. Forty-seven children with ichthyosis had either clinical or radiological evidence of rickets. The correlation between serum 25(OH)D and PTH showed that a serum level of 25(OH)D 8 ng mL(-1) was associated with a significant increase in PTH. The correlation between PTH and ALP showed that a serum PTH level of 75 pg mL(-1) was associated with a significant increase in ALP levels. Of the different clinical phenotypes of ichthyosis, both autosomal recessive congenital ichthyosis (ARCI) and epidermolytic ichthyosis (EI) were found to have significantly increased PTH, ALP and radiological rickets scores compared with common ichthyosis. Serum levels of 25(OH)D ≤ 8 ng mL(-1) and PTH ≥ 75 pg mL(-1) significantly increases the risk for development of rickets [odds ratio (OR) 2·8; 95% confidence interval (CI) 1·05-7·40; P = 0·04] in ichthyosis. Among the different types, patients with ARCI (OR 4·83; 95% CI 1·74-13·45; P < 0·01) and EI (OR 5·71; 95% CI 1·74-18·79; P < 0·01) are at an increased risk of developing rickets. © 2014 British Association of Dermatologists.

  3. Calpain 12 Function Revealed through the Study of an Atypical Case of Autosomal Recessive Congenital Ichthyosis.

    Science.gov (United States)

    Bochner, Ron; Samuelov, Liat; Sarig, Ofer; Li, Qiaoli; Adase, Christopher A; Isakov, Ofer; Malchin, Natalia; Vodo, Dan; Shayevitch, Ronna; Peled, Alon; Yu, Benjamin D; Fainberg, Gilad; Warshauer, Emily; Adir, Noam; Erez, Noam; Gat, Andrea; Gottlieb, Yehonatan; Rogers, Tova; Pavlovsky, Mor; Goldberg, Ilan; Shomron, Noam; Sandilands, Aileen; Campbell, Linda E; MacCallum, Stephanie; McLean, W H Irwin; Ast, Gil; Gallo, Richard L; Uitto, Jouni; Sprecher, Eli

    2017-02-01

    Congenital erythroderma is a rare and often life-threatening condition, which has been shown to result from mutations in several genes encoding important components of the epidermal differentiation program. Using whole exome sequencing, we identified in a child with congenital exfoliative erythroderma, hypotrichosis, severe nail dystrophy and failure to thrive, two heterozygous mutations in ABCA12 (c.2956C>T, p.R986W; c.5778+2T>C, p. G1900Mfs*16), a gene known to be associated with two forms of ichthyosis, autosomal recessive congenital ichthyosis, and harlequin ichthyosis. Because the patient displayed an atypical phenotype, including severe hair and nail manifestations, we scrutinized the exome sequencing data for additional potentially deleterious genetic variations in genes of relevance to the cornification process. Two mutations were identified in CAPN12, encoding a member of the calpain proteases: a paternal missense mutation (c.1511C>A; p.P504Q) and a maternal deletion due to activation of a cryptic splice site in exon 9 of the gene (c.1090_1129del; p.Val364Lysfs*11). The calpain 12 protein was found to be expressed in both the epidermis and hair follicle of normal skin, but its expression was dramatically reduced in the patient's skin. The downregulation of capn12 expression in zebrafish was associated with abnormal epidermal morphogenesis. Small interfering RNA knockdown of CAPN12 in three-dimensional human skin models was associated with acanthosis, disorganized epidermal architecture, and downregulation of several differentiation markers, including filaggrin. Accordingly, filaggrin expression was almost absent in the patient skin. Using ex vivo live imaging, small interfering RNA knockdown of calpain 12 in skin from K14-H2B GFP mice led to significant hair follicle catagen transformation compared with controls. In summary, our results indicate that calpain 12 plays an essential role during epidermal ontogenesis and normal hair follicle cycling and that

  4. Communication Styles of Interactive Tools for Self-Improvement.

    Science.gov (United States)

    Niess, Jasmin; Diefenbach, Sarah

    Interactive products for self-improvement (e.g., online trainings to reduce stress, fitness gadgets) have become increasingly popular among consumers and healthcare providers. In line with the idea of positive computing, these tools aim to support their users on their way to improved well-being and human flourishing. As an interdisciplinary domain, the design of self-improvement technologies requires psychological, technological, and design expertise. One needs to know how to support people in behavior change, and one needs to find ways to do this through technology design. However, as recent reviews show, the interlocking relationship between these disciplines is still improvable. Many existing technologies for self-improvement neglect psychological theory on behavior change, especially motivational factors are not sufficiently considered. To counteract this, we suggest a focus on the dialog and emerging communication between product and user, considering the self-improvement tool as an interactive coach and advisor. The present qualitative interview study (N = 18) explored the user experience of self-improvement technologies. A special focus was on the perceived dialog between tool and user, which we analyzed in terms of models from communication psychology. Our findings show that users are sensible to the way the product "speaks to them" and consider this as essential for their experience and successful change. Analysis revealed different communication styles of self-improvement tools (e.g., helpful-cooperative, rational-distanced, critical-aggressive), each linked to specific emotional consequences. These findings form one starting point for a more psychologically founded design of self-improvement technology. On a more general level, our approach aims to contribute to a better integration of psychological and technological knowledge, and in consequence, supporting users on their way to enhanced well-being.

  5. Treatment of ichthyosis lamellaris using a series of herbal skin care products family.

    Science.gov (United States)

    Tirant, M; Bayer, P; Hercogovấ, J; Fioranelli, M; Gianfaldoni, S; Chokoeva, A A; Tchernev, G; Wollina, U; Novotny, F; Roccia, M G; Maximov, G K; França, K; Lotti, T

    2016-01-01

    Lamellar ichthyosis (LI) is a genetically heterogeneous group of disorders of keratinization that are inherited in an autosomal recessive fashion, occurring in approximately 1 in 300,000 live births. The treatment of the large, dark, plate-like scales that characterize the classic manifestation of the disease are still a challenge. The aim of this study was to evaluate the efficacy and tolerability of Dr. Michaels® skin-care products for the management of LI. A multi-centre European prospective study was conducted, including 10 patients (3 female/7 male) with lamellar ichthyosis, aged 38-54 years old (mean age: 46). Each patient had been treated with emollients plus other different systemic therapies, such as corticosteroids, Cyclosporin A or retinoids in the past. All patients were treated with Dr Michaels® product family including both topical and oral herbal supplements. The topical treatments used were the cleansing gel, activator formula and ointment. The oral medications were PSC 200, PSC 400 and PSC 900. Within 3 weeks of initiation of treatment, there were improvements observed on the skin including a reduction in scaling, fissuring, and intensity in erythema and pruritus with thinning of the hyperkeratotic plate. After 12-15 weeks, most of the plates and scales had been removed to reveal a normalised skin colour. Evidence of hair, eyelash and eyebrow growth was observed. There was partial nail resolution with a reduction in subungual hyperkeratosis. No adverse reactions were observed. Our patients showed excellent symptomatic response to treatment within a 14-week period, follow-up by an on-going regular assessment on a quarterly basis. The results show that Dr Michaels® product family is an effective and safe treatment option for LI.

  6. Study on optimal conditions and adsorption kinetics of copper from water by collodion membrane cross-linked poly-γ-glutamic acid

    Energy Technology Data Exchange (ETDEWEB)

    Wu, Xiangting; Wang, Aiyin; Li, Guoxing; Dong, Xinjiao; Wu, Mingjiang [Wenzhou University, Wenzhou (China); Zheng, Xiaojie [Wenzhou Vocational College of Technology and Science, Wenzhou (China)

    2013-06-15

    Poly-γ-glutamic acid (γ-PGA) is a novel polyamino acid formed through microorganism fermentation and biosynthesis. In the present test, membrane (PGA-C) formation by γ-PGA and collodion was performed by using 0.1% glutaraldehyde as a cross-linking agent. A study was conducted on the PGA-C adsorption of Cu{sup 2+}, specifically the related adsorption equilibrium and kinetics, desorption and regeneration. The results show that with an initial solution pH=5.5 and at 318 K, the static adsorption isotherm behavior of PGA-C is in compliance with the Langmuir model and is beneficial to the adsorption of the metal. Meanwhile, with the reaction lasting for 30min, adsorption equilibrium was reached with a maximum adsorption capacity up to 7.431 mg/g. The entire reaction process follows the pseudo-second-order kinetics. By using PGA-C, good regeneration results were obtained after adsorption-generation-adsorption cycling with an HCl solution (0.1 mol/L) as regeneration liquid.

  7. Harlequin Ichthyosis

    Directory of Open Access Journals (Sweden)

    Hashemzadeh Ahmad

    2009-04-01

    Full Text Available It is an autosomal recessive, and occasionally autosomal dominant mutant extremely rare disorder with only 100 reported case in literature. This fatal disorder occur in both sexes and all races. In most circumstances the newborn die soon after birth Also it is known as harlequin fetus, alligator baby or keratosis diffusa fatalis."nBecause of its rarity, we report 2 cases of this disorder, here.

  8. Cochlear Implantation in Patients with Keratitis-Ichthyosis-Deafness Syndrome: A Report of Two Cases

    Directory of Open Access Journals (Sweden)

    Birgul Gumus

    2017-01-01

    Full Text Available Background. Keratitis-ichthyosis-deafness (KID syndrome is a syndrome which presents with hearing loss and visual and keratinization disorders. In such patients, hearing aids cannot be effectively used in the rehabilitation of hearing loss because of the frequent blockage of the external ear canal with epithelial debris and due to dry and tense skin of the external ear canal. Moreover, severe or profound hearing loss also limits the benefits gained from the conventional hearing aids. On the other hand, cochlear implantation is a method that has been used in limited cases in the literature. Case Report. This study presents the results of cochlear implantation applied in our clinic to two children who had been diagnosed with KID. Audiological assessments before and after the cochlear implant operation were performed using pure-tone audiometry, immittance audiometry, and auditory brainstem response (ABR, and the postoperative follow-up was conducted using pure-tone audiometry. Conclusion. Skin problems, visual disturbances, and other additional problems complicate the short-term and long-term rehabilitation after implantation in individuals with KID syndrome. Close monitoring should be exercised due to possible skin complications that may develop during the postoperative period. The families and rehabilitation teams should be warned about the possible visual disturbances and skin complications.

  9. A mouse model of harlequin ichthyosis delineates a key role for Abca12 in lipid homeostasis.

    Directory of Open Access Journals (Sweden)

    Ian Smyth

    2008-09-01

    Full Text Available Harlequin Ichthyosis (HI is a severe and often lethal hyperkeratotic skin disease caused by mutations in the ABCA12 transport protein. In keratinocytes, ABCA12 is thought to regulate the transfer of lipids into small intracellular trafficking vesicles known as lamellar bodies. However, the nature and scope of this regulation remains unclear. As part of an original recessive mouse ENU mutagenesis screen, we have identified and characterised an animal model of HI and showed that it displays many of the hallmarks of the disease including hyperkeratosis, loss of barrier function, and defects in lipid homeostasis. We have used this model to follow disease progression in utero and present evidence that loss of Abca12 function leads to premature differentiation of basal keratinocytes. A comprehensive analysis of lipid levels in mutant epidermis demonstrated profound defects in lipid homeostasis, illustrating for the first time the extent to which Abca12 plays a pivotal role in maintaining lipid balance in the skin. To further investigate the scope of Abca12's activity, we have utilised cells from the mutant mouse to ascribe direct transport functions to the protein and, in doing so, we demonstrate activities independent of its role in lamellar body function. These cells have severely impaired lipid efflux leading to intracellular accumulation of neutral lipids. Furthermore, we identify Abca12 as a mediator of Abca1-regulated cellular cholesterol efflux, a finding that may have significant implications for other diseases of lipid metabolism and homeostasis, including atherosclerosis.

  10. Novel mutations in the genes TGM1 and ALOXE3 underlying autosomal recessive congenital ichthyosis

    Science.gov (United States)

    Ullah, Rahim; Ansar, Muhammad; Durrani, Zaka Ullah; Lee, Kwanghyuk; Santos-Cortez, Regie Lyn P.; Muhammad, Dost; Ali, Mahboob; Zia, Muhammad; Ayub, Muhammad; Khan, Suliman; Smith, Josh D.; Nickerson, Deborah A.; Shendure, Jay; Bamshad, Michael; Leal, Suzanne M.; Ahmad, Wasim

    2016-01-01

    Background Ichthyoses are clinically characterized by scaling or hyperkeratosis of the skin or both. It can be an isolated condition limited to the skin or appear secondarily with involvement of other cutaneous or systemic abnormalities. Methods The present study investigated clinical and molecular characterization of three consanguineous families (A, B, C) segregating two different forms of autosomal recessive congenital ichthyosis (ARCI). Linkage in three consanguineous families (A, B, C) segregating two different forms of ARCI was searched by typing microsatellite and single nucleotide polymorphism marker analysis. Sequencing of the two genes TGM1 and ALOXE3 was performed by the dideoxy chain termination method. Results Genome-wide linkage analysis established linkage in family A to TGM1 gene on chromosome 14q11 and in families B and C to ALOXE3 gene on chromosome 17p13. Subsequently, sequencing of these genes using samples from affected family members led to the identification of three novel mutations: a missense variant p.Trp455Arg in TGM1 (family A); a nonsense variant p.Arg140* in ALOXE3 (family B); and a complex rearrangement in ALOXE3 (family C). Conclusion The present study further extends the spectrum of mutations in the two genes involved in causing ARCI. Characterizing the clinical spectrum resulting from mutations in the TGM1 and ALOXE3 genes will improve diagnosis and may direct clinical care of the family members. PMID:26578203

  11. Recessive mutations in ELOVL4 cause ichthyosis, intellectual disability, and spastic quadriplegia.

    Science.gov (United States)

    Aldahmesh, Mohammed A; Mohamed, Jawahir Y; Alkuraya, Hisham S; Verma, Ishwar C; Puri, Ratna D; Alaiya, Ayodele A; Rizzo, William B; Alkuraya, Fowzan S

    2011-12-09

    Very-long-chain fatty acids (VLCFAs) play important roles in membrane structure and cellular signaling, and their contribution to human health is increasingly recognized. Fatty acid elongases catalyze the first and rate-limiting step in VLCFA synthesis. Heterozygous mutations in ELOVL4, the gene encoding one of the elongases, are known to cause macular degeneration in humans and retinal abnormalities in mice. However, biallelic ELOVL4 mutations have not been observed in humans, and murine models with homozygous mutations die within hours of birth as a result of a defective epidermal water barrier. Here, we report on two human individuals with recessive ELOVL4 mutations revealed by a combination of autozygome analysis and exome sequencing. These individuals exhibit clinical features of ichthyosis, seizures, mental retardation, and spasticity-a constellation that resembles Sjögren-Larsson syndrome (SLS) but presents a more severe neurologic phenotype. Our findings identify recessive mutations in ELOVL4 as the cause of a neuro-ichthyotic disease and emphasize the importance of VLCFA synthesis in brain and cutaneous development. Copyright © 2011 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  12. A novel mutation in the connexin 26 gene (GJB2) in a child with clinical and histological features of keratitis-ichthyosis-deafness (KID) syndrome

    DEFF Research Database (Denmark)

    Koppelhus, Uffe; Tranebjaerg, L; Esberg, G

    2011-01-01

    Keratitis-ichthyosis-deafness (KID) syndrome is a rare congenital ectodermal disorder, caused by heterozygous missense mutation in GJB2, encoding the gap junction protein connexin 26. The commonest mutation is the p.Asp50Asn mutation, and only a few other mutations have been described to date....

  13. A homozygous FITM2 mutation causes a deafness-dystonia syndrome with motor regression and signs of ichthyosis and sensory neuropathy

    DEFF Research Database (Denmark)

    Zazo Seco, Celia; Castells-Nobau, Anna; Joo, Seol-Hee

    2017-01-01

    A consanguineous family from Pakistan was ascertained to have a novel deafness-dystonia syndrome with motor regression, ichthyosis-like features and signs of sensory neuropathy. By applying a combined strategy of linkage analysis and whole-exome sequencing in the presented family, a homozygous no...

  14. Ichthyosis follicularis, alopecia and photophobia syndrome (IFAP: report of the first case with ocular and cutaneous manifestations in Brazil with a favorable response to treatment

    Directory of Open Access Journals (Sweden)

    Luisa Moreira Höpker

    2011-02-01

    Full Text Available Ichthyosis follicular, alopecia, and photophobia (IFAP syndrome is a rare disease, with possible X-linked mode of inheritance. The patient presented with ocular findings of photophobia, corneal scarring and erosions, superficial and deep corneal vascularization and myopia. He was treated with artificial tears and punctal occlusion with small improvement of photophobia. After three months using systemic retinoid (Acitretina and posterior amniotic membrane transplantation in the left eye, there was a significant improvement of photophobia, corneal erosions and neuropsychomotor development.

  15. Thinspiration: Self-Improvement Versus Self-Evaluation Social Comparisons with Thin-Ideal Media Portrayals.

    Science.gov (United States)

    Knobloch-Westerwick, Silvia

    2015-01-01

    Much research has demonstrated negative impacts of idealized-body imagery exposure on body satisfaction. Yet, paradoxically, media with such imagery attract mass audiences. Few studies showed women's body satisfaction increased due to thin-ideal exposure. The kind of social comparison women engage in (self-evaluation vs. self-improvement) may explain these inconsistent findings and the paradoxical attraction to thin-ideal messages. Across 5 days, thin-ideal messages were presented to 51 women; self-evaluation and self-improvement social comparisons as well as body satisfaction were measured each day. A linear positive change in body satisfaction emerged. Greater self-improvement social comparisons increased this change, whereas greater self-evaluation social comparisons reduced it. Extent of both social comparison types changed during the prolonged exposure. A greater tendency to compare one's body with others' improved body satisfaction through self-improvement social comparisons and fostered weight-loss behaviors through self-evaluation social comparisons.

  16. Behavioural and Psychiatric Phenotypes in Men and Boys with X-Linked Ichthyosis: Evidence from a Worldwide Online Survey.

    Directory of Open Access Journals (Sweden)

    Sohini Chatterjee

    Full Text Available X-linked ichthyosis (XLI is a rare dermatological condition arising from deficiency for the enzyme steroid sulfatase (STS. Preliminary evidence in boys with XLI, and animal model studies, suggests that individuals lacking STS are at increased risk of developmental disorders and associated traits. However, the behavioural profile of children with XLI is poorly-characterised, and the behavioural profile of adults with XLI has not yet been documented at all.Using an online survey, advertised worldwide, we collected detailed self- or parent-reported information on behaviour in adult (n = 58 and younger (≤18yrs, n = 24 males with XLI for comparison to data from their non-affected brothers, and age/gender-matched previously-published normative data. The survey comprised demographic and background information (including any prior clinical diagnoses and validated questionnaires assaying phenotypes of particular interest (Adult ADHD Self-Report Scale v1.1, Barrett Impulsiveness Scale-11, adult and adolescent Autism Quotient, Kessler Psychological Distress Scales, and Disruptive Behaviour Disorder Rating Scale.Individuals with XLI generally exhibited normal sensory function. Boys with XLI were at increased risk of developmental disorder, whilst adults with the condition were at increased risk of both developmental and mood disorders. Both adult and younger XLI groups scored significantly more highly than male general population norms on measures of inattention, impulsivity, autism-related traits, psychological distress and disruptive behavioural traits.These findings indicate that both adult and younger males with XLI exhibit personality profiles that are distinct from those of males within the general population, and suggest that individuals with XLI may be at heightened risk of psychopathology. The data are consistent with the notion that STS is important in neurodevelopment and ongoing brain function, and with previous work suggesting high rates of

  17. Defects in Stratum Corneum Desquamation Are the Predominant Effect of Impaired ABCA12 Function in a Novel Mouse Model of Harlequin Ichthyosis.

    Directory of Open Access Journals (Sweden)

    Lei Zhang

    Full Text Available Harlequin Ichthyosis is a severe skin disease caused by mutations in the human gene encoding ABCA12. Here, we characterize a novel mutation in intron 29 of the mouse Abca12 gene that leads to the loss of a 5' splice donor site and truncation of the Abca12 RNA transcript. Homozygous mutants of this smooth skin or smsk allele die perinatally with shiny translucent skin, typical of animal models of Harlequin Ichthyosis. Characterization of smsk mutant skin showed that the delivery of glucosylceramides and CORNEODESMOSIN was defective, while ultrastructural analysis revealed abnormal lamellar bodies and the absence of lipid lamellae in smsk epidermis. Unexpectedly, mutant stratum corneum remained intact when subjected to harsh chemical dissociation procedures. Moreover, both KALLIKREIN 5 and -7 were drastically decreased, with retention of desmoplakin in mutant SC. In cultured wild type keratinocytes, both KALLIKREIN 5 and -7 colocalized with ceramide metabolites following calcium-induced differentiation. Reducing the intracellular levels of glucosylceramide with a glucosylceramide synthase inhibitor resulted in decreased secretion of KALLIKREIN proteases by wild type keratinocytes, but not by smsk mutant keratinocytes. Together, these findings suggest an essential role for ABCA12 in transferring not only lipids, which are required for the formation of multilamellar structures in the stratum corneum, but also proteolytic enzymes that are required for normal desquamation. Smsk mutant mice recapitulate many of the pathological features of HI and can be used to explore novel topical therapies against a potentially lethal and debilitating neonatal disease.

  18. A Self-Improving School System and Its Potential for Reducing Inequality

    Science.gov (United States)

    Hargreaves, David H.

    2014-01-01

    After a brief review of some milestones in the story of how schools contribute to inequalities in student achievement, more recent work on how experience of collaboration between schools can help to narrow the gap is shown to underpin the new concept of a self-improving school system. The main focus is then on the principal features of a…

  19. When does self-improvement undermine materialistic tendencies, and when does it strengthen them?

    OpenAIRE

    Anna Maria Zawadzka

    2017-01-01

    Background In view of the fact that materialism may be caused by feelings of insecurity and low self-esteem, this paper attempts to answer the question of what the role of self-improvement is in the development of materialistic tendencies. Participants and procedure Two experiments were carried out (n = 144, n = 126). Half of the participants were exposed to self-threat (failure), which boosts materialistic tendencies, and the others were exposed to self-enhancement (succe...

  20. Collodion baby: A report of 4 cases

    African Journals Online (AJOL)

    owner

    2012-12-28

    Dec 28, 2012 ... Netherton, Kid and Bid as well as erythrokeratoderma variabilis of Mendes da Costa ... the third consecutive child of the mother with similar presentation at birth. ... Blood culture results are negative. By the 10th day there was ...

  1. Ethnicity moderates the outcomes of self-enhancement and self-improvement themes in expressive writing.

    Science.gov (United States)

    Tsai, William; Lau, Anna S; Niles, Andrea N; Coello, Jordan; Lieberman, Matthew D; Ko, Ahra C; Hur, Christopher; Stanton, Annette L

    2015-10-01

    The current study examined whether writing content related to self-enhancing (viz., downward social comparison and situational attributions) and self-improving (viz., upward social comparison and persistence) motivations were differentially related to expressive writing outcomes among 17 Asian American and 17 European American participants. Content analysis of the essays revealed no significant cultural group differences in the likelihood of engaging in self-enhancing versus self-improving reflections on negative personal experiences. However, cultural group differences were apparent in the relation between self-motivation processes and changes in anxiety and depressive symptoms at 3-month follow-up. Among European Americans, writing that reflected downward social comparison predicted positive outcomes, whereas persistence writing themes were related to poorer outcomes. For Asian Americans, writing about persistence was related to positive outcomes, whereas downward social comparison and situational attributions predicted poorer outcomes. Findings provide evidence suggesting culturally distinct mechanisms for the effects of expressive disclosure. (PsycINFO Database Record (c) 2015 APA, all rights reserved).

  2. Boost Your Body: Self-Improvement Magazine Messages Increase Body Satisfaction in Young Adults.

    Science.gov (United States)

    Veldhuis, Jolanda; Konijn, Elly A; Knobloch-Westerwick, Silvia

    2017-02-01

    The verbal messages that contextualize exposure to idealized body imagery may moderate media users' body satisfaction. Such contextualizing verbal messages often take the form of social comparison motives in fashion magazines, while body dissatisfaction is an important mechanism underlying various body image-related health issues like depression and unbalanced weight status. Hence, the present study applied social comparison motives as induced through magazine cover messages. Hypotheses were tested in an experimental design with social comparison motives (self-improvement vs. self-evaluation vs. control) and recipient gender as between-subjects factors and body satisfaction as within-subjects factor (N = 150). Results showed that self-improvement messages accompanying ideal body media models increased body satisfaction, compared to control messages and baseline measures. In contrast, the self-evaluation messages did not impact body satisfaction. Results imply that inconsistencies regarding effects from exposure to idealized body imagery are explained by the context in which media images are portrayed, evoking differential social comparison motives. Moreover, the findings imply that health communication interventions can use verbal messages on body improvement as helpful tools, if they draw on social comparison motives effectively.

  3. Keratitis-Ichthyosis-Deafness syndrome-associated Cx26 mutants produce nonfunctional gap junctions but hyperactive hemichannels when co-expressed with wild type Cx43

    Science.gov (United States)

    García, Isaac E.; Maripillán, Jaime; Jara, Oscar; Ceriani, Ricardo; Palacios-Muñoz, Angelina; Ramachandran, Jayalakshimi; Olivero, Pablo; Pérez-Acle, Tomás; González, Carlos; Sáez, Juan C.; Contreras, Jorge E.; Martínez, Agustín D.

    2015-01-01

    Mutations in Cx26 gene are found in most cases of human genetic deafness. Some mutations produce syndromic deafness associated with skin disorders, like Keratitis Ichthyosis Deafness syndrome (KID). Because in the human skin Cx26 is co-expressed with other connexins, like Cx43 and Cx30, and since KID syndrome is inherited as autosomal dominant condition, it is possible that KID mutations change the way Cx26 interacts with other co-expressed connexins. Indeed, some Cx26 syndromic mutations showed gap junction dominant negative effect when co-expressed with wild type connexins, including Cx26 and Cx43. The nature of these interactions and the consequences on hemichannels and gap junction channels functions remain unknown. In this study we demonstrate that syndromic mutations at the N-terminus segment of Cx26, change connexin oligomerization compatibility, allowing aberrant interactions with Cx43. Strikingly, heteromeric oligomer formed by Cx43/Cx26 (syndromic mutants) show exacerbated hemichannel activity, but nonfunctional gap junction channels; this also occurs for those Cx26 KID mutants that do not show functional homomeric hemichannels. Heterologous expression of these hyperactive heteromeric hemichannels increases cell membrane permeability, favoring ATP release and Ca2+ overload. The functional paradox produced by oligomerization of Cx43 and Cx26 KID mutants could underlie the severe syndromic phenotype in human skin. PMID:25625422

  4. A homozygous FITM2 mutation causes a deafness-dystonia syndrome with motor regression and signs of ichthyosis and sensory neuropathy

    Directory of Open Access Journals (Sweden)

    Celia Zazo Seco

    2017-02-01

    Full Text Available A consanguineous family from Pakistan was ascertained to have a novel deafness-dystonia syndrome with motor regression, ichthyosis-like features and signs of sensory neuropathy. By applying a combined strategy of linkage analysis and whole-exome sequencing in the presented family, a homozygous nonsense mutation, c.4G>T (p.Glu2*, in FITM2 was identified. FITM2 and its paralog FITM1 constitute an evolutionary conserved protein family involved in partitioning of triglycerides into cellular lipid droplets. Despite the role of FITM2 in neutral lipid storage and metabolism, no indications for lipodystrophy were observed in the affected individuals. In order to obtain independent evidence for the involvement of FITM2 in the human pathology, downregulation of the single Fitm ortholog, CG10671, in Drosophila melanogaster was pursued using RNA interference. Characteristics of the syndrome, including progressive locomotor impairment, hearing loss and disturbed sensory functions, were recapitulated in Drosophila, which supports the causative nature of the FITM2 mutation. Mutation-based genetic counseling can now be provided to the family and insight is obtained into the potential impact of genetic variation in FITM2.

  5. Analysis of TGM1, ALOX12B, ALOXE3, NIPAL4 and CYP4F22 in autosomal recessive congenital ichthyosis from Galicia (NW Spain): evidence of founder effects.

    Science.gov (United States)

    Rodríguez-Pazos, L; Ginarte, M; Fachal, L; Toribio, J; Carracedo, A; Vega, A

    2011-10-01

      Mutations in six genes have been identified in autosomal recessive congenital ichthyosis (ARCI). To date, few studies have analysed the spectrum of these mutations in specific populations. We have studied the characteristics of patients with ARCI in Galicia (NW Spain). Methods  We recruited patients by contacting all dermatology departments of Galicia and the Spanish patient organization for ichthyosis. TGM1, ALOX12B, ALOXE3, NIPAL4 and CYP4F22 were analysed in the patients and their relatives. We identified 23 patients with ARCI and estimated a prevalence of 1 : 122 000. Twenty of the patients were studied. Seventeen of them were clinically categorized as having lamellar ichthyosis (LI) and three as having congenital ichthyosiform erythroderma (CIE). TGM1 and ALOXE3 mutations were identified in 12/16 (75%) probands whereas no ALOX12B, NIPAL4 and CYP4F22 mutations were found. TGM1 mutations were found in 11/13 (85%) of LI probands. ALOXE3 mutations were identified in a single patient with CIE. Remarkably, mutations p.Arg760X, p.Asp408ValfsX21 and c.984+1G>A of TGM1 were present in six, four and two families, accounting for 41%, 23% and 14% of all TGM1 mutant alleles, respectively. The high percentage of patients with the same TGM1 mutations, together with the high number of homozygous probands (64%), indicates the existence of a strong founder effect in our population. © 2011 The Authors. BJD © 2011 British Association of Dermatologists 2011.

  6. Abca12-mediated lipid transport and Snap29-dependent trafficking of lamellar granules are crucial for epidermal morphogenesis in a zebrafish model of ichthyosis

    Directory of Open Access Journals (Sweden)

    Qiaoli Li

    2011-11-01

    Zebrafish (Danio rerio can serve as a model system to study heritable skin diseases. The skin is rapidly developed during the first 5–6 days of embryonic growth, accompanied by expression of skin-specific genes. Transmission electron microscopy (TEM of wild-type zebrafish at day 5 reveals a two-cell-layer epidermis separated from the underlying collagenous stroma by a basement membrane with fully developed hemidesmosomes. Scanning electron microscopy (SEM reveals an ordered surface contour of keratinocytes with discrete microridges. To gain insight into epidermal morphogenesis, we have employed morpholino-mediated knockdown of the abca12 and snap29 genes, which are crucial for secretion of lipids and intracellular trafficking of lamellar granules, respectively. Morpholinos, when placed on exon-intron junctions, were >90% effective in preventing the corresponding gene expression when injected into one- to four-cell-stage embryos. By day 3, TEM of abca12 morphants showed accumulation of lipid-containing electron-dense lamellar granules, whereas snap29 morphants showed the presence of apparently empty vesicles in the epidermis. Evaluation of epidermal morphogenesis by SEM revealed similar perturbations in both cases in the microridge architecture and the development of spicule-like protrusions on the surface of keratinocytes. These morphological findings are akin to epidermal changes in harlequin ichthyosis and CEDNIK syndrome, autosomal recessive keratinization disorders due to mutations in the ABCA12 and SNAP29 genes, respectively. The results indicate that interference of independent pathways involving lipid transport in the epidermis can result in phenotypically similar perturbations in epidermal morphogenesis, and that these fish mutants can serve as a model to study the pathomechanisms of these keratinization disorders.

  7. Aberrant distribution patterns of corneodesmosomal components of tape-stripped corneocytes in atopic dermatitis and related skin conditions (ichthyosis vulgaris, Netherton syndrome and peeling skin syndrome type B).

    Science.gov (United States)

    Igawa, Satomi; Kishibe, Mari; Honma, Masaru; Murakami, Masamoto; Mizuno, Yuki; Suga, Yasushi; Seishima, Mariko; Ohguchi, Yuka; Akiyama, Masashi; Hirose, Kenji; Ishida-Yamamoto, Akemi; Iizuka, Hajime

    2013-10-01

    Atopic dermatitis (AD), Netherton syndrome (NS) and peeling skin syndrome type B (PSS) may show some clinical phenotypic overlap. Corneodesmosomes are crucial for maintaining stratum corneum integrity and the components' localization can be visualized by immunostaining tape-stripped corneocytes. In normal skin, they are detected at the cell periphery. To determine whether AD, NS, PSS and ichthyosis vulgaris (IV) have differences in the corneodesmosomal components' distribution and corneocytes surface areas. Corneocytes were tape-stripped from a control group (n=12) and a disease group (37 AD cases, 3 IV cases, 4 NS cases, and 3 PSS cases), and analyzed with immunofluorescent microscopy. The distribution patterns of corneodesmosomal components: desmoglein 1, corneodesmosin, and desmocollin 1 were classified into four types: peripheral, sparse diffuse, dense diffuse and partial diffuse. Corneocyte surface areas were also measured. The corneodesmosome staining patterns were abnormal in the disease group. Other than in the 3 PSS cases, all three components showed similar patterns in each category. In lesional AD skin, the dense diffuse pattern was prominent. A high rate of the partial diffuse pattern, loss of linear cell-cell contacts, and irregular stripping manners were unique to NS. Only in PSS was corneodesmosin staining virtually absent. The corneocyte surface areas correlated significantly with the rate of combined sparse and dense diffuse patterns of desmoglein 1. This method may be used to assess abnormally differentiated corneocytes in AD and other diseases tested. In PSS samples, tape stripping analysis may serve as a non-invasive diagnostic test. Copyright © 2013 Japanese Society for Investigative Dermatology. Published by Elsevier Ireland Ltd. All rights reserved.

  8. Social Comparison in Coping With Occupational Uncertainty: Self-Improvement, Self-Enhancement, and the Regional Context.

    Science.gov (United States)

    Pavlova, Maria K; Lechner, Clemens M; Silbereisen, Rainer K

    2018-04-01

    Taking into account the regional context, we investigated whether social comparison in coping with occupational uncertainty served self-improvement (i.e., adaptive coping) or self-enhancement (i.e., subjective well-being). Respondents were 620 German adults aged 16 to 43, 59% female, who participated in three yearly follow-ups of a larger survey. The number of observations was 1,309 for contemporaneous and 1,079 for longitudinal analyses. Participants reported on perceived occupational uncertainty (e.g., risk of losing a job and difficulties with career planning), strategies for coping with it, and whether, and in which direction, they made social comparisons in coping with occupational uncertainty. Making social comparisons (vs. not) was associated with higher goal engagement and lower goal disengagement. Upward (as opposed to downward) comparison prospectively predicted higher goal engagement. Under high regional unemployment, upward comparison prospectively predicted lower goal disengagement, whereas making social comparisons was contemporaneously associated with higher subjective well-being. Higher regional unemployment rates predicted more frequent comparison, whereas comparison direction was predicted only by situational variables, especially personal control over the outcomes. When operationalized as a conscious mental action and put in the context of coping with occupational uncertainty, social comparison serves primarily self-improvement. © 2017 Wiley Periodicals, Inc.

  9. Self-esteem, readiness for self-improvement and life satisfaction in Indian and Polish female students

    Directory of Open Access Journals (Sweden)

    Zawadzka Anna Maria

    2016-06-01

    Full Text Available The study examines the question of how personal self-esteem, collective self-esteem and readiness for self-improvement are linked to satisfaction with life in women from countries differing with regard to level of collectivism. Our study participants were Polish (less collectivistic and Indian (more collectivistic female students. The obtained results indicate that personal self-esteem plays a very important role in satisfaction with life of women from the two countries. However, collective self-esteem is not directly related to satisfaction with life among women from the two cultures analyzed. Structural Equation Modeling showed that: a in the Indian group readiness for self-improvement is more important for satisfaction with life than in the Polish group and it is significantly related to satisfaction with life through collectivistic self-esteem, b the direct influence of both personal and collective self-esteem on satisfaction with life is more significant in the Polish group than in the Indian group.

  10. Divergent consequences of success and failure in japan and north america: an investigation of self-improving motivations and malleable selves.

    Science.gov (United States)

    Heine, S J; Lehman, D R; Ide, E; Leung, C; Kitayama, S; Takata, T; Matsumoto, H

    2001-10-01

    Self-enhancing and self-improving motivations were investigated across cultures. Replicating past research, North Americans who failed on a task persisted less on a follow-up task than those who succeeded. In contrast, Japanese who failed persisted more than those who succeeded. The Japanese pattern is evidence for a self-improving orientation: Failures highlight where corrective efforts are needed. Japanese who failed also enhanced the importance and the diagnosticity of the task compared with those who succeeded, whereas North Americans did the opposite. Study 2 revealed that self-improving motivations are specific to the tasks on which one receives feedback. Study 3 unpackaged the cultural differences by demonstrating that they are due, at least in part, to divergent lay theories regarding the utility of effort. Study 4 addressed the problem of comparing cultures on subjective Likert scales and replicated the findings with a different measure.

  11. A clinical nurse specialist-led intervention to enhance medication adherence using the plan-do-check-act cycle for continuous self-improvement.

    Science.gov (United States)

    Russell, Cynthia L

    2010-01-01

    A clinical nurse specialist-led intervention to improve medication adherence in chronically ill adults using renal transplant recipients as an exemplar population is proposed. Meta-analyses and systematic reviews of chronically ill and transplant patients indicate that patient-specific characteristics not only are poor and inconsistent predictors for medication nonadherence but also are not amenable to intervention. Adherence has not meaningfully improved, despite meta-analyses and systematic narrative reviews of randomized controlled trials (RCTs) dealing with medication nonadherence in acutely and chronically ill persons and RCTs dealing with transplant patients. Interventions with a superior potential to enhance medication adherence must be developed. Use of a clinical nurse specialist-led continuous self-improvement intervention with adult renal transplant recipients is proposed. Continuous self-improvement focuses on improving personal systems thinking and behavior using the plan-do-check-act process. Electronic medication monitoring reports, one of several objective measures of medication adherence, are used by the clinician to provide patient feedback during the check process on medication-taking patterns. Continuous self-improvement as an intervention holds promise in supporting patient self-management and diminishing the blame that clinicians place on patients for medication nonadherence. Using an objective measure of medication adherence such as an electronic monitoring report fosters collaborative patient-clinician discussions of daily medication-taking patterns. Through collaboration, ideas for improving medication taking can be explored. Changes can be followed and evaluated for effectiveness through the continuous self-improvement process. Future studies should include RCTs comparing educational and/or behavioral interventions to improve medication adherence.

  12. Lowering data retention voltage in static random access memory array by post fabrication self-improvement of cell stability by multiple stress application

    Science.gov (United States)

    Mizutani, Tomoko; Takeuchi, Kiyoshi; Saraya, Takuya; Kobayashi, Masaharu; Hiramoto, Toshiro

    2018-04-01

    We propose a new version of the post fabrication static random access memory (SRAM) self-improvement technique, which utilizes multiple stress application. It is demonstrated that, using a device matrix array (DMA) test element group (TEG) with intrinsic channel fully depleted (FD) silicon-on-thin-buried-oxide (SOTB) six-transistor (6T) SRAM cells fabricated by the 65 nm technology, the lowering of data retention voltage (DRV) is more effectively achieved than using the previously proposed single stress technique.

  13. The role of self-improvement and self-evaluation motives in social comparisons with idealised female bodies in the media.

    Science.gov (United States)

    Halliwell, Emma; Dittmar, Helga

    2005-09-01

    This study investigates the effect of social comparisons with media models on women's body image based on either self-evaluation or self-improvement motives. Ninety-eight women, for whom appearance was a relevant comparison dimension, viewed advertisements that did, or did not, feature idealised models, after being prompted to engage in self-evaluation or self-improvement comparisons. The results indicate that, when focusing on self-evaluation, comparisons with thin models are associated with higher body-focused anxiety than viewing no model advertisements. In contrast, when focusing on self-improvement, comparisons with thin models are not associated with higher body-focused anxiety than viewing no models. Furthermore, women's general tendency to engage in social comparisons moderated the effects of self-evaluative comparisons with models, so that women who did not habitually engage in social comparisons were most strongly affected. It is suggested that motive for social comparison may explain previous inconsistencies in the experimental exposure literature and warrants more careful attention in future research.

  14. Ichthyosis vulgaris met populatiespecifieke filaggrine (FLG) mutaties

    NARCIS (Netherlands)

    Clabbers, J.M.K.; Dodemont, S.R.P.; van Geel, M.; Steijlen, P.M.

    2017-01-01

    Filaggrin (‘filament-aggregating protein’) plays an essential role in the terminal differentiation of the epidermis. It forms an important barrier function of the skin preventing transepidermal water loss and penetration of allergens into the skin. The human profilaggrin gene consists of three

  15. Striving for self-improvement

    DEFF Research Database (Denmark)

    Pedersen, Inge Kryger

    2017-01-01

    The notion of medical enhancement technologies has drawn attention to optimization techniques within the health area. However, this notion has evolved at the level of governmental programmes, with very little attention directed towards people’s own practices. Using a social scientific body...

  16. More Fragmented, and yet More Networked: Analysing the Responses of Two Local Authorities in England to the Coalition's "Self-Improving School-Led System" Reforms

    Science.gov (United States)

    Greany, Toby

    2015-01-01

    This paper explores school reform in England under the Conservative-led Coalition government, elected in 2010, through a focus on the changing roles and status of Local Authorities (LAs). The Coalition's stated aim was the development of a "self-improving, school-led" system in which LAs should become "champions for children."…

  17. Collodion baby: A report of 4 cases | Obu | Nigerian Journal of ...

    African Journals Online (AJOL)

    Nigerian Journal of Paediatrics. Journal Home · ABOUT THIS JOURNAL · Advanced Search · Current Issue · Archives · Journal Home > Vol 40, No 3 (2013) >. Log in or Register to get access to full text downloads. Username, Password, Remember me, or Register · Download this PDF file. The PDF file you selected should ...

  18. Adult and Christian Self-Improvement Literature

    Science.gov (United States)

    Rowland, Michael L.

    2006-01-01

    Many adults search for spiritual guidance and direction and persist in seeking answers to life in today's fast-paced world. With burgeoning economic challenges, political corruption, war in Iraq, poverty, health care concerns, environmental concerns, rising fuel costs, violence, racism, and oppression, many adults seek solace and greater…

  19. Self-Improving CNC Milling Machine

    OpenAIRE

    Spilling, Torjus

    2014-01-01

    This thesis is a study of the ability of a CNC milling machine to create parts for itself, and an evaluation of whether or not the machine is able to improve itself by creating new machine parts. This will be explored by using off-the-shelf parts to build an initial machine, using 3D printing/rapid prototyping to create any special parts needed for the initial build. After an initial working machine is completed, the design of the machine parts will be adjusted so that the machine can start p...

  20. Congenital Ichthyosis in a Nigerian preterm neonate: A case report ...

    African Journals Online (AJOL)

    2016-01-12

    Jan 12, 2016 ... Leaving Certificate married to two wives. The mother ... laboratory screening revealed the following: PCV - ... as the facilities for genetic analysis were not. 100 ... using lubrication with bland petrolatum as a universally.

  1. Congenital Ichthyosis in a Nigerian preterm neonate: A case report ...

    African Journals Online (AJOL)

    The infant was managed using stringent thermoregulation, optimal hydration, use of topical emollient and antibiotics. Although, the skin disorder resolved within fifteen days of treatment, the infant thereafter developed inguinoscrotal hernia and hydrocephalus necessitating surgical consult. The case is reported to highlight ...

  2. Autosomal recessive ichthyosis with limb reduction defect: A simple ...

    African Journals Online (AJOL)

    Rabah M. Shawky

    2015-10-01

    Oct 1, 2015 ... Our patient is a 13 year old girl, the first in birth order of first cousin parents, (Fig. 1). She was born at term by cesarean sec- tion of uncomplicated pregnancy. The patient was born in a yellow, tight and shiny sheath that started to desquamate after two to three weeks and replaced by rounded dark scaly ...

  3. HARLEQUIN ICHTHYOSIS IN AN AFRICAN CHILD: CASE REPORT

    African Journals Online (AJOL)

    2010-09-09

    Sep 9, 2010 ... cytoplasm in the stratum cornea is congested with abnormal lipid containing ... drugs and counselling needs of the parents. Despite limited resources we ... administration of retinoids and psychosocial support for the family.

  4. Superación profesional en la Atención Primaria de Salud: una estrategia didáctica propiciadora de estilos de vida saludables Professional self-improvement in the Primary Health care: a didactic strategy for healthy lifestyles

    Directory of Open Access Journals (Sweden)

    Aglae Cáceres Diéguez

    2011-01-01

    Full Text Available Se persigue exponer una estrategia didáctica general de superación profesional de posgrado, formativa y desarrolladora, a través del método de investigación-acción, con la finalidad de propiciar transformaciones favorables de estilos de vida de la población cubana y se explica cómo implementarla. Habiendo sido adecuada a las necesidades formativas de los participantes para conseguir lo anterior mediante la apropiación y aplicación social de los conocimientos como integradores de habilidades, se encamina a fomentar actitudes positivas - a través de convicciones- para modificar comportamientos inadecuados, crear mecanismos para lograr una salud responsable, formar o consolidar valores, así como viabilizar el desarrollo intelectual y social para perfeccionar el modo de actuación profesional. Se sustenta en la dinámica de una concepción didáctica por medio de una intervención de superación dirigida a los profesionales de la atención primaria para transformar los estilos de vida de la población que puedan ser mejorados. Tanto las acciones educativas y socioculturales como las intervenciones de salud social se fundamentan en la integración de elementos planteados por reconocidos autores, entre los cuales figuran: Rojas, Fuillerat, Ferrer, Rodríguez Ojea y Castell Florit.It is our objective to expose a general didactic strategy of developing post degree professional self improvement, through the investigation-action method, with the purpose of propitiating favorable transformations of the Cuban population's lifestyles and it is explained how to implement it. After being adapted to the formation needs of the participants to obtain this goal by means of the appropriation and social application of knowledge as a skill unifier, it is aimed at creating positive attitudes-through convictions-at modifying inadequate behaviors, at creating mechanisms to achieve a considerable health, at forming or consolidating values, as well as making

  5. Memory-Modulation: Self-Improvement or Self-Depletion?

    Science.gov (United States)

    Lavazza, Andrea

    2018-01-01

    Autobiographical memory is fundamental to the process of self-construction. Therefore, the possibility of modifying autobiographical memories, in particular with memory-modulation and memory-erasing, is a very important topic both from the theoretical and from the practical point of view. The aim of this paper is to illustrate the state of the art of some of the most promising areas of memory-modulation and memory-erasing, considering how they can affect the self and the overall balance of the "self and autobiographical memory" system. Indeed, different conceptualizations of the self and of personal identity in relation to autobiographical memory are what makes memory-modulation and memory-erasing more or less desirable. Because of the current limitations (both practical and ethical) to interventions on memory, I can only sketch some hypotheses. However, it can be argued that the choice to mitigate painful memories (or edit memories for other reasons) is somehow problematic, from an ethical point of view, according to some of the theories of the self and personal identity in relation to autobiographical memory, in particular for the so-called narrative theories of personal identity, chosen here as the main case of study. Other conceptualizations of the "self and autobiographical memory" system, namely the constructivist theories, do not have this sort of critical concerns. However, many theories rely on normative (and not empirical) conceptions of the self: for them, the actions aimed at mitigating or removing specific (negative) memories can be seen either as an improvement or as a depletion or impairment of the self.

  6. Memory-Modulation: Self-Improvement or Self-Depletion?

    Directory of Open Access Journals (Sweden)

    Andrea Lavazza

    2018-04-01

    Full Text Available Autobiographical memory is fundamental to the process of self-construction. Therefore, the possibility of modifying autobiographical memories, in particular with memory-modulation and memory-erasing, is a very important topic both from the theoretical and from the practical point of view. The aim of this paper is to illustrate the state of the art of some of the most promising areas of memory-modulation and memory-erasing, considering how they can affect the self and the overall balance of the “self and autobiographical memory” system. Indeed, different conceptualizations of the self and of personal identity in relation to autobiographical memory are what makes memory-modulation and memory-erasing more or less desirable. Because of the current limitations (both practical and ethical to interventions on memory, I can only sketch some hypotheses. However, it can be argued that the choice to mitigate painful memories (or edit memories for other reasons is somehow problematic, from an ethical point of view, according to some of the theories of the self and personal identity in relation to autobiographical memory, in particular for the so-called narrative theories of personal identity, chosen here as the main case of study. Other conceptualizations of the “self and autobiographical memory” system, namely the constructivist theories, do not have this sort of critical concerns. However, many theories rely on normative (and not empirical conceptions of the self: for them, the actions aimed at mitigating or removing specific (negative memories can be seen either as an improvement or as a depletion or impairment of the self.

  7. The human cystatin M/E gene (CST6): exclusion candidate gene for harlequin ichthyosis.

    NARCIS (Netherlands)

    Zeeuwen, P.L.J.M.; Dale, B.A.; Jongh, G.J. de; Vlijmen-Willems, I.M.J.J. van; Fleckman, P.; Kimball, J.R.; Stephens, K.; Schalkwijk, J.

    2003-01-01

    Cystatin M/E is a recently discovered cysteine proteinase inhibitor whose expression is largely confined to cutaneous epithelia. In human skin it is expressed in sweat glands, hair follicles, and stratum granulosum of the epidermis where it presumably acts as a substrate for transglutaminase. Very

  8. The case of ichthyosis follicularis, alopecia and photophobia syndrome with retinal detachment

    Directory of Open Access Journals (Sweden)

    Bengü Nisa Akay

    2014-06-01

    Full Text Available Ichtiyosis follicularis, alopecia and photophobia (IFAP syndrome is a rare congenital ectodermal syndrome with X-linked inheritance. It occurs as a result of missense mutation in chromosome Xp22.11-Xp22.13 locus of MBTPS2 gene. It usually affects men and family history is always negative. Ichtiyosis follicularis and alopecia starts with birth. Photophobia and eye symptoms begin in early infancy or childhood. Other manifestations of the syndrome include short stature, mental retardation and seizures. There are no spesific histopathological findings specific for ichtyosis follicularis. A 29 years old male patient was admitted to outpatient clinic. Dermatological examination revealed keratosis pilaris localized to scalp, extremities and anterolateral of the body. Patient had xerosis, diffuse alopecia and prominent folicular appereance. Eye examination revealed cataracts and vision loss. These findings led us to IFAP syndrome diagnosis. The patient is presented for the rarity of the syndrome in the literature.

  9. Ny klassifikation og molekylærgenetisk viden om arvelig iktyose

    DEFF Research Database (Denmark)

    Andersen, Rikke Elkjær; Hertz, Jens Michael; Bygum, Anette

    2014-01-01

    A new classification of inherited ichthyoses is presented based on clinical features, genetic background and pathophysiology. Ichthyoses are disorders of cornification and may be part of syndromes. Ichthyosis vulgaris, X-linked ichthyosis, autosomal recessive congenital ichthyosis and syndrome...

  10. Genetics Home Reference: Chanarin-Dorfman syndrome

    Science.gov (United States)

    ... disease with ichthyosis Triglyceride storage disease with ichthyosis triglyceride storage disease with impaired long-chain fatty acid ... Lipid Metabolism Disorders Genetic and Rare Diseases Information Center (1 ...

  11. Autogenic Feedback Training (Body Fortran) with Biofeedback and the Computer for Self-Improvement and Change.

    Science.gov (United States)

    Cassel, Russell N.; Sumintardja, Elmira Nasrudin

    1983-01-01

    Describes autogenic feedback training, which provides the basis whereby an individual is able to improve on well being through use of a technique described as "body fortran," implying that you program self as one programs a computer. Necessary requisites are described including relaxation training and the management of stress. (JAC)

  12. Self-improvement and cooperation: How exchange relationships promote mastery-approach driven individuals’ job outcomes

    NARCIS (Netherlands)

    Poortvliet, P.M.; Giebels, E.

    2012-01-01

    In the present research we argue that mastery-approach goals may be beneficial in social achievement contexts because these goals lead to constructive exchange relationship building. An examination of three methodologically complementary studies revealed that mastery-approach goals lead to more

  13. Data-Driven Hint Generation in Vast Solution Spaces: A Self-Improving Python Programming Tutor

    Science.gov (United States)

    Rivers, Kelly; Koedinger, Kenneth R.

    2017-01-01

    To provide personalized help to students who are working on code-writing problems, we introduce a data-driven tutoring system, ITAP (Intelligent Teaching Assistant for Programming). ITAP uses state abstraction, path construction, and state reification to automatically generate personalized hints for students, even when given states that have not…

  14. Genetics Home Reference: Stormorken syndrome

    Science.gov (United States)

    ... ichthyosis), headaches, and difficulty with reading and spelling (dyslexia). Related Information What does it mean if a ... new syndrome: thrombocytopathia, muscle fatigue, asplenia, miosis, migraine, dyslexia and ichthyosis. Clin Genet. 1985 Nov;28(5): ...

  15. Disease: H00741 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H00741 Ichthyosis prematurity syndrome Ichthyosis prematurity syndrome is a form o...onnevig J, Johnsson M, Sitek JC, Blaas HG, Hausser I, Johansen FE, Jahnsen FL ... TITLE ... Ichthyosis prematurity...e cause the ichthyosis prematurity syndrome. ... JOURNAL ... Am J Hum Genet 85:248-53 (2009) DOI:10.1016/j.ajhg.2009.06.021 ...Dahl N, Fischer J ... TITLE ... Mutations in the fatty acid transport protein 4 gen

  16. Presentación de un caso de hemidisplasia congénita con ictiosis eritrodérmica Report of a congenital hemidysplasia with erythrodermic ichthyosis

    Directory of Open Access Journals (Sweden)

    Andrés A. Morilla Guzmán

    2008-09-01

    Full Text Available La hemidisplasia congénita con ictiosis y defectos de las extremidades es una enfermedad infrecuente, hereditaria, monogénica, que se transmite como un rasgo dominante ligado al cromosoma X. Se presenta el caso de una paciente con este diagnóstico clínico neonatal, que presentaba eritrodermia ictiosiforme en el hemicuerpo derecho, acompañada de hipomelia del miembro superior e inferior derechos, defectos óseos en miembros afectados y columna vertebral, agenesia renal unilateral, cardiopatía congénita de tipo comunicación interventricular conoventricular y arteria umbilical única. Se realizaron las interconsultas necesarias, estudios sonográficos y radiológicos para completar el diagnóstico y se ofreció asesoramiento genético y seguimiento del caso según las complicaciones reportadas en la literatura médica y los hallazgos clínicos de la paciente.Congenital hemidysplasia with icthyosis and limb defects is a hereditary, monogenic and infrequent disease transmitted as a dominant trait linked to the X chromosome. The case of a female patient with this neonatal clinical diagnosis showing ichthyosiform erythroderma on the right hemibody, accompanied with hypomelia of the right upper and lower limbs, bone defects in the affected limbs and spinal column, unilateral renal agenesia, congenital heart disease with inter- and conoventricular communication, and a unique umbilical artery was reported. The necessary inter-consultations were arranged and sonographic and radiological studies were conducted to complete the diagnosis. Genetic counselling was given and the case was followed up according to the complications reported in medical literature, and to the clinical findings of the patient.

  17. Inability of keratinocytes lacking their specific transglutaminase to form cross-linked envelopes: Absence of envelopes as a simple diagnostic test for lamellar ichthyosis

    OpenAIRE

    Jeon, Saewha; Djian, Philippe; Green, Howard

    1998-01-01

    Epidermal keratinocytes, late in their terminal differentiation, form cross-linked envelopes resistant to ionic detergent and reducing agent. Because the cross-linking process is catalyzed by the keratinocyte transglutaminase, the absence of active transglutaminase should result in failure of the keratinocyte to form a cross-linked envelope. Three keratinocyte strains bearing mutations in the keratinocyte transglutaminase were examined: two contained no detectable transglutaminase mRNA and no...

  18. The geography teacher's set of appliances - `GEOGRAPHY nEtQUIPMENT' - Self improved school equipment used in teaching geography

    Science.gov (United States)

    Pajtok-Tari, I.

    2009-04-01

    The multimedia application and the use of Internet are becoming more and more common at schools and at homes due to the widespread of computers. The multimedia programs offer a great help for geography teachers because with their use all the visual aids are not needed in the classroom. They mix the advantages of blacboards, slides, displays, overhead projectors and VCR-s. At the same time offering other opportunities which could not be provided by the aids mentioned above because of their limits. Using a projector connected to a computer students can see the visual aids prepared by the teacher projected. Their use is justified because student's books cannot contain all the increasing amount of knowledge. Success is guaranteed because students are sensitive to new approaches. Digitalizing the material and finding it on the internet that way preparing a colourful, varied geography lesson is a time-consuming process. Being the methodologist and didactic information technologist at the Geography Department of Eszterházy Károly College I have been working for years on facilitating the work of my students, colleagues and my own activity using varied visual aids and types of equipment as preparation for the geography lesson. I have created an electronic set of appliances using the Dreamweaver MX program (‘GEOGRAPHY nEtQUIPMENT', from the 1st September 2006 on the Internet), it can be a real help for the teacher in each teaching situation. The ‘GEOGRAPHY nEtQUIPMENT' is a multimedia, Internet service which can be loaded free, the teacher gets into a virtual office clicking to the different pieces (drawer, shelf, wall map, globe, laptop, Tv set etc.) the teacher can continue with the necessary school equipment. Such equipment like: lesson plans for the lessons using digital technology, photos, video clips, animation, illustrations, pieces of music, maps, collection of minerals, database, diagrams, charts, bibliography, student's books, geography lexicons, magazines, geography games, important web pages (SDT, Google Earth) etc. Multimedia offers a new and efficient procedure of spreading information. It creates new possibilities in forming the teaching - learning environment. It integrates more mediums that way providing more efficient visualized possibilities for the teacher. The use of multimedia systems is advantageous because at the same time they have effect on more senses. The presentation of the content is more interesting, pleasing, so it is motivating. The renewal of schools can be achieved by those teachers who are able to recognize and use the modern technics, tecnology, for this reason teachers should integrate them in their activities. Hopefully the Net set of appliances makes this easier. The Net set of appliances can be shaped by the teacher. It can be loaded with his own materials (photos, videos, tests, charts, drafts, etc.). This way teachers may help their students to be familiar with the faster and faster developing information society so satisfying one of the most common goals of teaching geography, according to it: giving a coherent, comprehensive image of our world on a scientific basis, systematic knowledge, so creating a firm base for their further self- studies and the clever reception and appreciation of information.

  19. The ‘Ilhas’ of Porto and ‘self-improvement urbanism’: the inhabitants as engine of urban transformation

    NARCIS (Netherlands)

    Holz Amorim de Sena, N.H.; Doevendans, P.; Rousseau, S.

    2014-01-01

    An ‘Ilha’ in Porto is a typology of low cost housing which spread within the city in the 19th Century as a result of the industrial development. The extremely small dwellings (around 4x4m) were established within backyards of middle class houses, connected to the street through a narrow corridor.

  20. THE VALUE OF SELF-IMPROVEMENT IN THE PROFESSIONAL COMPETITIVENESS OF GRADUATES IN THE CONDITIONS OF INFORMATIZATION

    Directory of Open Access Journals (Sweden)

    Е Ю Усенкова

    2016-12-01

    Full Text Available The article presents a synthesis of the experience of higher education teachers about methods of teaching, stimulating professional and cultural self-development of students, the conclusions and practical recommendations of scientists on the issues of self-perfection, which is one of the main conditions of competitiveness of modern University graduates.

  1. Self-improving Inference System to Support the Intelligence Preparation of the Battlefield: Requirements, State of the Art, and Prototypes

    Science.gov (United States)

    2014-12-01

    droit du Canada), telle que représentée par le ministre de la Défense nationale, 2014 Abstract …….. This report presents the results of...documents coming from the international military community (doctrine, manuals) as well as from the academia (scientific papers). From this analysis, a...References ..... [1] US Military, “US Joint Intelligence Preparation of the Operational Environment”, Joint Publication 2-01.3, 16 June

  2. Atypical Presentation of Sjögren-Larsson Syndrome

    Directory of Open Access Journals (Sweden)

    D. Papathemeli

    2017-01-01

    Full Text Available Sjögren-Larsson syndrome is a rare neurocutaneous disorder characterized by ichthyosis, spastic diplegia or tetraplegia, and intellectual disability. Herein, we describe a case of a Greek patient with ichthyosis and spasticity of the legs but with normal intelligence (IQ 95. This syndrome should be suspected when a child presents with ichthyosis and spastic diplegia or tetraplegia, even if intelligence is normal.

  3. Download

    African Journals Online (AJOL)

    rme

    X-Linked ichthyosis on the clinical features or pedigree analysis of the family in Egypt and the ... molecular studies to reach the proper diagnosis of X-linked ichthyosis. Key Words: .... case using G-banding technique.17,18,19,20 karyotyping.

  4. A Rare Cause of Fatty Liver and Elevated Aminotransferase Levels: Chanarin-Dorfman Syndrome: A Case Report

    Directory of Open Access Journals (Sweden)

    Özdal Ersoy

    2011-01-01

    Full Text Available Chanarin-Dorfman syndrome is a rare, inherited metabolic disorder of neutral lipid storage characterized by ichthyosis, lipid vacuoles in leukocytes, and involvement of several internal organs, mostly the liver. Since the initial case was reported by Dorfman in 1974, nearly 50 cases have been reported, and the majority were from Middle East countries. Here, we report a 20-year-old patient with ichthyosis from Turkey, diagnosed as Chanarin-Dorfman syndrome presented with asypmtomatic elevated transaminases and hepatosteatosis, and also briefly review the updated clinical implications and management of this rarely seen syndrome. Prompt diagnosis of this syndrome avoids further unnecessary investigations in patients with ichthyosis

  5. HARLEQUIN ICTHYOSIS: A RARE CASE REPORT

    Directory of Open Access Journals (Sweden)

    Pradipprava Paria

    2016-01-01

    Full Text Available Harlequin ichthyosis is the most severe form of congenital ichthyosis. It is a rare autosomal recessive disorder (1:300,000. The vast majority of affected individuals are due to mutation in the ABCA12 gene, which cause a deficiency of the epidermal lipid transporter, resulting in hyperkeratosis and abnormal barrier function of skin. Infants are very susceptible to metabolic abnormalitites and infections. They usually do not survive for very long. we report here a case of a new born with harlequin ichthyosis of consaguinious parentage who had a history of similar birth previously.

  6. Genotype-Phenotype Correlations Emerging from the Identification of Missense Mutations in MBTPS2

    NARCIS (Netherlands)

    Bornholdt, D.; Atkinson, T.P.; Bouadjar, B.; Catteau, B.; Cox, H.; Silva, D. De; Fischer, J.; Gunasekera, C.N.; Hadj-Rabia, S.; Happle, R.; Holder-Espinasse, M.; Kaminski, E.; Konig, A.; Megarbane, A.; Megarbane, H.; Neidel, U.; Oeffner, F.; Oji, V.; Theos, A.; Traupe, H.; Vahlquist, A.; Bon, B.W. van; Virtanen, M.; Grzeschik, K.H.

    2013-01-01

    Missense mutations affecting membrane-bound transcription factor protease site 2 (MBTPS2) have been associated with Ichthyosis Follicularis with Atrichia and Photophobia (IFAP) syndrome with or without BRESHECK syndrome, with keratosis follicularis spinulosa decalvans, and Olmsted syndrome. This

  7. Dorfman-Chanarin syndrome: a rare neutral lipid storage disease.

    Science.gov (United States)

    Mitra, Souvik; Samanta, Moumita; Sarkar, Mihir; Chatterjee, Sukanta

    2010-01-01

    Dorfman-Chanarin syndrome is a rare neutral lipid storage disorder characterized by ichthyosis, lipid vacuolations in peripheral leucocytes, and multisystem involvement. It is an autosomal recessive disorder caused by mutations in the CGI-58 gene. A total of 42 cases have been reported worldwide till February 2009 out of which 4 have been previously reported from India. We report a case of a 20-month-old male with congenital ichthyosis, organomegaly, and bilateral cryptorchidism. Examination of the peripheral smear revealed lipid vacuoles in the leucocytes consistent with Jordan's anomaly, which was confirmed by transmission electron microscopy. Liver biopsy revealed micronodular cirrhosis with macrovesicular steatosis while skin biopsy showed ichthyosis vulgaris. Dorfman-Chanarin syndrome was diagnosed on the basis of clinical and laboratory criteria with certain unreported manifestations. Dietary modifications were instituted and followed up after 1 year with promising results. This emphasizes the importance of neonatal screening for lipid vacuolations in peripheral blood in all cases of congenital ichthyosis.

  8. Sjogren-Larsson syndrome: Report of two cases

    Directory of Open Access Journals (Sweden)

    Uppal Monica

    2004-03-01

    Full Text Available Two cases of Sjogren-Larsson syndrome are discussed along with a review of the literature. Both the patients had generalized ichthyosis, spastic paraplegia, mental retardation and ophthalmologic examination showing glistening foveal and parafoveal dots.

  9. Genodermatoses in paediatric age group

    Directory of Open Access Journals (Sweden)

    Kumar Sunil

    1996-01-01

    Full Text Available Pattern of genodermatoses in paediatric age group was studied. The relative incidence of genodermatoses in paediatric dermatology out patient department was 0.62%. The commonest genodermatoses observed was ichthyosis.

  10. Dorfman-Chanarin syndrome: A rare neutral lipid storage disease

    OpenAIRE

    Mitra Souvik; Samanta Moumita; Sarkar Mihir; Chatterjee Sukanta

    2010-01-01

    Dorfman-Chanarin syndrome is a rare neutral lipid storage disorder characterized by ichthyosis, lipid vacuolations in peripheral leucocytes, and multisystem involvement. It is an autosomal recessive disorder caused by mutations in the CGI-58 gene. A total of 42 cases have been reported worldwide till February 2009 out of which 4 have been previously reported from India. We report a case of a 20-month-old male with congenital ichthyosis, organomegaly, and bilateral cryptorchidism. Examination ...

  11. A practical approach to ichthyoses with systemic manifestations.

    Science.gov (United States)

    Saral, S; Vural, A; Wollenberg, A; Ruzicka, T

    2017-06-01

    Inherited ichthyoses are rare disorders in terms of patient numbers, but abundant in terms of clinical-genetic subtypes. These disorders are often associated with severe systemic manifestations, in addition to significant medical, cosmetic and social problems. There are 17 subtypes of syndromic ichthyosis identified so far and most patients with these syndromes are living in countries with high consanguinity rates. Frequently, clinicians cannot make a definitive diagnosis and patients are not managed properly owing to the rarity and complexity of these disorders. These difficulties make this group of ichthyosis and the patients living with them 'orphan'. After skin and skin appendages, nervous system is the most frequently involved system in ichthyosis syndromes. Thus, association of ichthyosis with neurological symptoms provides an important clue for diagnosis. In this article, we aim to increase clinicians' comprehension of ichthyosis syndromes by providing a symptomatology-based approach based on this observation. Additionally, we provide a review of ichthyosis syndromes, with special emphasis on neurological symptoms, hoping to attract interest to this complicated field. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  12. Untitled

    Indian Academy of Sciences (India)

    Many substances show in colloidal solution, a double refraction of the same sign as in the amorphous solid state. Umlaufº has found that the sols of cherry gum and tragacanth thave positive and those of collodion, gelatine and gum arabic negative double refraction under high mechanical stress. Ambronn” found that the dry.

  13. Vanakaamera fotograafide tegus nädalavahetus Hollandis / Kaisa Keizars, Tarmo Virves

    Index Scriptorium Estoniae

    Keizars, Kaisa

    2013-01-01

    Rahvusvaheline üritus European Collodion Weekend 4.-5. maini Hollandis Veldhovenis, kus vanakaameraid kasutavad fotograafid valmistasid märgplaadi tehnikas ambrotüüpe ja ferrotüüpe. Eestlastest osalesid Tarmo Virves, Kaisa Keizars, Käty Tarkpea, Andres Palm

  14. Preventing Cauliflower Ear with a Modified Tie-Through Technique.

    Science.gov (United States)

    Dimeff, Robert J.; Hough, David O.

    1989-01-01

    Describes a quick, simple tie-through suture technique (in which a collodion packing is secured to the auricle with two buttons) for preventing cauliflower ear following external ear trauma in wrestlers and boxers. The technique ensures constant compression; multiple treatments for fluid reaccumulation are rarely necessary. (SM)

  15. Who Told you that you were Analogue?

    OpenAIRE

    Brown, A

    2017-01-01

    Before breakfast, my social media newsfeed drew my attention to two features on the wet collodion process: one article about lumen prints; two articles about World Cyanotype Day; and a video about Daguerrotypes. Somewhere, an algorithm is channelling digital representations of early photographic techniques towards me. \\ud Welcome to the future.

  16. Failure of the Nernst-Einstein equation to correlate electrical resistances and rates of ionic self-exchange across certain fixed charge membranes.

    Science.gov (United States)

    Gottlieb, M H; Sollner, K

    1968-05-01

    The electrical resistances and rates of self-exchange of univalent critical ions across several types of collodion matrix membranes of high ionic selectivity were studied over a wide range of conditions. The relationship which was observed between these quantities with membranes of a certain type, namely those activated with poly-2-vinyl-N-methyl pyridinium bromide, cannot be explained on the basis of current concepts of the movement of ions across ion exchange membranes. Rates of self-exchange across these membranes were several times greater than those calculated from the electrical resistances of the membranes on the basis of an expression derived by the use of the Nernst-Einstein equation. The magnitude of the discrepancy was greatest at low concentrations of the ambient electrolyte solution and was independent of the species of both critical and noncritical ions. The data obtained with other types of collodion matrix membranes were, at least approximately, in agreement with the predictions based on the Nernst-Einstein equation. Self-exchange rates across the anion permeable protamine collodion membranes, and across the cation permeable polystyrene sulfonic acid collodion membranes, were about 20% less than those calculated from the electrical resistances. The direction and magnitude of these differences, also observed by other investigators, are qualitatively understood as an electroosmotic effect. With cation permeable membranes prepared by the oxidation of preformed collodion membranes, almost exact agreement was obtained between measured and calculated self-exchange rates; the cause of the apparent absence of an electroosmotic effect with these membranes is unknown.

  17. TARGETED PRINCIPLE COMPONENT ANALYSIS: A NEW MOTION ARTIFACT CORRECTION APPROACH FOR NEAR-INFRARED SPECTROSCOPY.

    Science.gov (United States)

    Yücel, Meryem A; Selb, Juliette; Cooper, Robert J; Boas, David A

    2014-03-01

    As near-infrared spectroscopy (NIRS) broadens its application area to different age and disease groups, motion artifacts in the NIRS signal due to subject movement is becoming an important challenge. Motion artifacts generally produce signal fluctuations that are larger than physiological NIRS signals, thus it is crucial to correct for them before obtaining an estimate of stimulus evoked hemodynamic responses. There are various methods for correction such as principle component analysis (PCA), wavelet-based filtering and spline interpolation. Here, we introduce a new approach to motion artifact correction, targeted principle component analysis (tPCA), which incorporates a PCA filter only on the segments of data identified as motion artifacts. It is expected that this will overcome the issues of filtering desired signals that plagues standard PCA filtering of entire data sets. We compared the new approach with the most effective motion artifact correction algorithms on a set of data acquired simultaneously with a collodion-fixed probe (low motion artifact content) and a standard Velcro probe (high motion artifact content). Our results show that tPCA gives statistically better results in recovering hemodynamic response function (HRF) as compared to wavelet-based filtering and spline interpolation for the Velcro probe. It results in a significant reduction in mean-squared error (MSE) and significant enhancement in Pearson's correlation coefficient to the true HRF. The collodion-fixed fiber probe with no motion correction performed better than the Velcro probe corrected for motion artifacts in terms of MSE and Pearson's correlation coefficient. Thus, if the experimental study permits, the use of a collodion-fixed fiber probe may be desirable. If the use of a collodion-fixed probe is not feasible, then we suggest the use of tPCA in the processing of motion artifact contaminated data.

  18. TARGETED PRINCIPLE COMPONENT ANALYSIS: A NEW MOTION ARTIFACT CORRECTION APPROACH FOR NEAR-INFRARED SPECTROSCOPY

    Science.gov (United States)

    YÜCEL, MERYEM A.; SELB, JULIETTE; COOPER, ROBERT J.; BOAS, DAVID A.

    2014-01-01

    As near-infrared spectroscopy (NIRS) broadens its application area to different age and disease groups, motion artifacts in the NIRS signal due to subject movement is becoming an important challenge. Motion artifacts generally produce signal fluctuations that are larger than physiological NIRS signals, thus it is crucial to correct for them before obtaining an estimate of stimulus evoked hemodynamic responses. There are various methods for correction such as principle component analysis (PCA), wavelet-based filtering and spline interpolation. Here, we introduce a new approach to motion artifact correction, targeted principle component analysis (tPCA), which incorporates a PCA filter only on the segments of data identified as motion artifacts. It is expected that this will overcome the issues of filtering desired signals that plagues standard PCA filtering of entire data sets. We compared the new approach with the most effective motion artifact correction algorithms on a set of data acquired simultaneously with a collodion-fixed probe (low motion artifact content) and a standard Velcro probe (high motion artifact content). Our results show that tPCA gives statistically better results in recovering hemodynamic response function (HRF) as compared to wavelet-based filtering and spline interpolation for the Velcro probe. It results in a significant reduction in mean-squared error (MSE) and significant enhancement in Pearson’s correlation coefficient to the true HRF. The collodion-fixed fiber probe with no motion correction performed better than the Velcro probe corrected for motion artifacts in terms of MSE and Pearson’s correlation coefficient. Thus, if the experimental study permits, the use of a collodion-fixed fiber probe may be desirable. If the use of a collodion-fixed probe is not feasible, then we suggest the use of tPCA in the processing of motion artifact contaminated data. PMID:25360181

  19. Controlled long-term release of small peptide hormones using a new microporous polypropylene polymer: its application for vasopressin in the Brattleboro rat and potential perinatal use

    International Nuclear Information System (INIS)

    Kruisbrink, J.; Boer, G.J.

    1984-01-01

    Based on drug release by microporous hollow fibers and the recent introduction of microporous polymers, a new technique was developed for controlled delivery of peptides. Small-diameter microporous polypropylene tubing, lumen-loaded with microgram quantities of vasopressin, and coated with collodion, releases vasopressin after in vitro immersion slowly (1-100 ng/d) and constantly for months. The mechanism of pseudo-zero-order delivery is based on high adsorption of vasopressin, keeping the void volume concentration of dissolved vasopressin constant, which is consequently a constant driving force of outward diffusion. The collodion coating prevents the entry of proteinaceous compounds which would result in rapid desorption of vasopressin. The present delivery module provides a lasting release for other peptides as well (lysine-vasopressin, oxytocin, alpha-melanocyte-stimulating hormone and, to a lesser extent, Met-enkephalin). The microporous polymer-collodion device is biocompatible and, loaded with vasopressin, successfully alleviates the diabetes insipidus of Brattleboro rats deficient for vasopressin. Subcutaneous implantation normalized diuresis for a period of 60 d and constant urine vasopressin excretion is observed. When the commercially available osmotic minipump is too large for implantation, the small size of the present controlled-delivery system allows peptide treatment of young and immature laboratory rats, even if located in utero

  20. Revertant Mosaicism in Heritable Skin Diseases - Mechanisms of Natural Gene Therapy

    NARCIS (Netherlands)

    Pasmooij, Anna M. G.; Jonkman, Marcel F.; Uitto, Jouni

    Revertant mosaicism (RM) refers to the co-existence of cells carrying disease-causing mutations with cells in which the inherited mutation is genetically corrected by a spontaneous event. It has been discovered in an increasing number of heritable skin diseases: ichthyosis with confetti and

  1. CASE REPORT Radiographic diagnosis of a rare case of ...

    African Journals Online (AJOL)

    parts of the body, particularly the eyes, teeth and fingers, as the ... A 16-year-old girl presented to the hospital with complaints of weakness in her lower limbs, ... and cleft palate. ... in ODDD.7 Keratitis-ichthyosis-deafness (KID) syndrome is the most ... syndrome is of crucial importance in prevention and treatment of the.

  2. A mutation in the XPB/ERCC3 DNA repair transcription gene, associated with trichothiodystrophy.

    NARCIS (Netherlands)

    G. Weeda (Geert); E. Eveno; I. Donker (Ingrid); W. Vermeulen (Wim); O. Chevalier-Lagente (Odile); A. Taieb; A. Stary; J.H.J. Hoeijmakers (Jan); M. Mezzina; A. Sarasin

    1997-01-01

    textabstractTrichothiodystrophy (TTD) is a rare, autosomal recessive disorder characterized by sulfur-deficient brittle hair and nails, mental retardation, impaired sexual development, and ichthyosis. Photosensitivity has been reported in approximately 50% of the cases, but no skin cancer is

  3. Monitoring of fatty aldehyde dehydrogenase by formation of pyrenedecanoic acid from pyrenedecanal

    NARCIS (Netherlands)

    Keller, Markus A.; Watschinger, Katrin; Golderer, Georg; Maglione, Manuel; Sarg, Bettina; Lindner, Herbert H.; Werner-Felmayer, Gabriele; Terrinoni, Alessandro; Wanders, Ronald J. A.; Werner, Ernst R.

    2010-01-01

    Fatty aldehyde dehydrogenase (EC 1.2.1.48) converts long-chain fatty aldehydes to the corresponding acids. Deficiency in this enzyme causes the Sjogren Larsson Syndrome, a rare inherited disorder characterized by ichthyosis, spasticity, and mental retardation. Using a fluorescent aldehyde,

  4. MRI of a very rare hereditary ectodermal dysplasia: PIBI(D)S

    International Nuclear Information System (INIS)

    Peserico, A.; Bertoli, P.; Battistella, P.A.

    1992-01-01

    PIBI(D)S is a acronym for a very rare autosomal recessive syndrome consisting of photosensitivity, mild non-congenital ichthyosis, brittle cystine-deficient hair, impaired intelligence, occasionally decreased fertility and short stature. We report a 12-year-old female patient affected by PIBI(D)S with previously unreported MRI findings of central nervous system dysmyelination. (orig.)

  5. HID and KID syndromes are associated with the same connexin 26 mutation.

    NARCIS (Netherlands)

    Geel, M. van; Steensel, M.A.M. van; Kuster, W.; Hennies, H.C.; Happle, R.H.G.; Steijlen, P.M.; Konig, A.C.

    2002-01-01

    BACKGROUND: Keratitis-ichthyosis-deafness (KID) syndrome is a debilitating ectodermal dysplasia that predisposes patients to develop squamous cell carcinomas in addition to leading to profound sensory deafness and erythrokeratoderma. We recently demonstrated that KID can be caused by a specific

  6. Fulltext PDF

    Indian Academy of Sciences (India)

    Unknown

    lethal disorder called hydrops-ectopic calcification-'moth eaten' (HEM) or Greenberg skeletal dys- plasia. The healthy mother of the affected fetus had hypolobulated granulocyte nuclei and abnormal chromatin structure thus confirming her PHA status. In a third paper, Shultz et al (2003) reported that the mouse ichthyosis ...

  7. Connexin 26 ( GJB2 ) mutation in KID syndrome: An Egyptian patient

    African Journals Online (AJOL)

    Keratitis ± ichthyosis ± deafness (KID) syndrome is a rare disorder characterized by the occurrence of localized erythematous scaly skin lesions, severe bilateral keratitis, and sensorineural deafness. Other ocular manifestations include corneal epithelial defects and scarring, which cause progressive decline of visual acuity ...

  8. Genetics Home Reference: Sjögren-Larsson syndrome

    Science.gov (United States)

    ... Email Facebook Twitter Home Health Conditions Sjögren-Larsson syndrome Sjögren-Larsson syndrome Printable PDF Open All Close All ... FALDH deficiency fatty aldehyde dehydrogenase deficiency ichthyosis oligophrenia syndrome Sjogren-Larsson syndrome SLS Related Information How are genetic ...

  9. A novel assay for the prenatal diagnosis of Sjögren-Larsson syndrome

    NARCIS (Netherlands)

    van den Brink, D. M.; van Miert, J. M.; Wanders, R. J. A.

    2005-01-01

    Sjögren-Larsson syndrome (SLS) is a metabolic disorder characterized by ichthyosis, mental retardation and spastic diplegia or tetraplegia. The biochemical defect has been identified as a deficiency of fatty aldehyde dehydrogenase (FALDH), which is part of an enzyme complex that converts fatty

  10. MRI of a very rare hereditary ectodermal dysplasia: PIBI(D)S

    Energy Technology Data Exchange (ETDEWEB)

    Peserico, A.; Bertoli, P. (Ist. di Clinica Dermosifilopatica, Padua Univ. (Italy)); Battistella, P.A. (Dipt. di Pediatria, Padua Univ. (Italy))

    1992-08-01

    PIBI(D)S is a acronym for a very rare autosomal recessive syndrome consisting of photosensitivity, mild non-congenital ichthyosis, brittle cystine-deficient hair, impaired intelligence, occasionally decreased fertility and short stature. We report a 12-year-old female patient affected by PIBI(D)S with previously unreported MRI findings of central nervous system dysmyelination. (orig.).

  11. Filaggringenmutationer er hypige og öger risikoen for hudsygdom

    DEFF Research Database (Denmark)

    Meyer, Martin Willy; Thyssen, Jacob Pontoppidan

    2011-01-01

    Filaggrin proteins are important for the epidermal differentiation and skin barrier function. About 10% of Europeans carry loss-of-function mutations within the filaggrin gene complex. Mutations are strongly associated with ichthyosis vulgaris and atopic dermatitis as well as dry and fissured skin...

  12. Dorfman-Chanarin syndrome: A rare neutral lipid storage disease

    Directory of Open Access Journals (Sweden)

    Mitra Souvik

    2010-10-01

    Full Text Available Dorfman-Chanarin syndrome is a rare neutral lipid storage disorder characterized by ichthyosis, lipid vacuolations in peripheral leucocytes, and multisystem involvement. It is an autosomal recessive disorder caused by mutations in the CGI-58 gene. A total of 42 cases have been reported worldwide till February 2009 out of which 4 have been previously reported from India. We report a case of a 20-month-old male with congenital ichthyosis, organomegaly, and bilateral cryptorchidism. Examination of the peripheral smear revealed lipid vacuoles in the leucocytes consistent with Jordan′s anomaly, which was confirmed by transmission electron microscopy. Liver biopsy revealed micronodular cirrhosis with macrovesicular steatosis while skin biopsy showed ichthyosis vulgaris. Dorfman-Chanarin syndrome was diagnosed on the basis of clinical and laboratory criteria with certain unreported manifestations. Dietary modifications were instituted and followed up after 1 year with promising results. This emphasizes the importance of neonatal screening for lipid vacuolations in peripheral blood in all cases of congenital ichthyosis.

  13. Filaggrin loss-of-function mutations, atopic dermatitis and risk of actinic keratosis

    DEFF Research Database (Denmark)

    Andersen, Y M F; Egeberg, A; Balslev, E

    2017-01-01

    BACKGROUND: Common loss-of-function mutations in filaggrin gene (FLG) represent a strong genetic risk factor for atopic dermatitis (AD). Homozygous mutation carriers typically display ichthyosis vulgaris (IV) and many have concomitant AD. Previously, homozygous, but not heterozygous, filaggrin ge...

  14. Congenital anomalies in black South African liveborn neonates at ...

    African Journals Online (AJOL)

    hypospadias the commonest urogenital anomalies (Table VI). The incidence of cleft lip/palate was only 0,23 per 1 000. Iivebirths (Table V). The integumentary conditions seen included congenital ichthyosis, epidermolysis bullosa, both disorders of. Mendelian inheritance, an unclassifiable bullous dermatitis and a naevus.

  15. Detection of steroid sulfatase gene deletion (STS) in Egyptian males ...

    African Journals Online (AJOL)

    Patients and Methods: We performed this study on Egyptian males complaining of X-linked ichthyosis who were subjected to clinical examination, pedigree analysis of the family, cytogenetic studies using G-banding technique and fluorescent in situ hybridization (FISH) using locus specific probe for stereoid sulfatase (STS) ...

  16. [Synthesis and physico-chemical properties of lonazolac-Ca, a new antiphlogistic/antirheumatic agent].

    Science.gov (United States)

    Rainer, G; Krüger, U; Klemm, K

    1981-01-01

    Calcium-[3-(p-chlorophenyl)-1-phenylpyrazole-4]-acetate (Lonazolac-Ca, active principle of Irritren) is a new antiinflammatory/antirheumatic agent whose synthesis and physico-chemical properties are described. The physical parameters measured (pKa, partition coefficient P, saturation concentration Cs, surface activity, protein binding) are held against the corresponding values of indomethacin, diclofenac, and phenylbutazone. The size of the permeability coefficient PM of the passive transport through artificial phospholipid collodion membranes as well as the invasion curves calculated from PM indicate a good absorption of lonazolac in man.

  17. Final Report for X-ray Diffraction Sample Preparation Method Development

    Energy Technology Data Exchange (ETDEWEB)

    Ely, T. M. [Hanford Site (HNF), Richland, WA (United States); Meznarich, H. K. [Hanford Site (HNF), Richland, WA (United States); Valero, T. [Hanford Site (HNF), Richland, WA (United States)

    2018-01-30

    WRPS-1500790, “X-ray Diffraction Saltcake Sample Preparation Method Development Plan/Procedure,” was originally prepared with the intent of improving the specimen preparation methodology used to generate saltcake specimens suitable for XRD-based solid phase characterization. At the time that this test plan document was originally developed, packed powder in cavity supports with collodion binder was the established XRD specimen preparation method. An alternate specimen preparation method less vulnerable, if not completely invulnerable to preferred orientation effects, was desired as a replacement for the method.

  18. Importance of Skin Changes in the Differential Diagnosis of Congenital Muscular Dystrophies

    Directory of Open Access Journals (Sweden)

    Uluç Yis

    2016-01-01

    Full Text Available Megaconial congenital muscular dystrophy (OMIM 602541 is characterized with early-onset hypotonia, muscle wasting, proximal weakness, cardiomyopathy, mildly elevated serum creatine kinase (CK levels, and mild-to-moderate intellectual disability. We report two siblings in a consanguineous family admitted for psychomotor delay. Physical examination revealed proximal muscle weakness, contractures in the knee of elder sibling, diffuse mild generalized muscle atrophy, and dry skin with ichthyosis together with multiple nummular eczema in both siblings. Serum CK values were elevated up to 500 U/L. For genetic work-up, we performed whole exome sequencing (WES after Nimblegen enrichment on the Illumina platform. The WES revealed a novel homozygous missense mutation in the Choline Kinase-Beta (CHKB gene c.1031G>A (p.R344Q in exon 9. Ichthyosis-like skin changes with intense pruritus and nummular eczema may lead to clinical diagnosis in cases with megaconial congenital muscular dystrophy.

  19. Chanarin Dorfman Syndrome: A Case Report

    Directory of Open Access Journals (Sweden)

    Yasemin Ozkale

    2015-09-01

    Full Text Available Chanarin Dorfman Syndrome is a multisystem inherited metabolic disorder associated with congenital ichthyosis and accumulation of lipid droplets in varios types of cells. Observation of lipid vacuoles in neutrophils (Jordan's anomaly in peirpheral blood smears in patients with ichthyosis is diagnostic for Chanarin Dorfman Syndrome. Since the initial case was reported by Dorfman in 1974, nearly 50 cases have been reported in the literature, and the majority were from Middle East countries. In this report we presented a 5 year old patient who admitted to our hospital for creatine kinase elevation and diagnosed as Chanarin Dorfman Syndrome with clinical and laboratory findings. [Cukurova Med J 2015; 40(3.000: 614-618

  20. Juvenile folliculotropic and ichthyosiform mycosis fungoides.

    LENUS (Irish Health Repository)

    Ryan, C

    2012-02-01

    Ichthyosiform mycosis fungoides (MF) is a recently recognized clinical variant of MF, which appears as dry scaling patches and plaques, or as a generalized eruption. Acquired ichthyosis is well recognized as a paraneoplastic cutaneous presentation of malignancy, especially in lymphoproliferative disorders. In contrast, the ichthyosiform eruption in ichthyotic MF is attributable to infiltration of the skin by tumour cells. We report the case of a 15-year-old boy who presented with a 5-year history of enlarging pruritic plaques on the forehead and back, patchy alopecia and generalized ichthyosis. Histology of the forehead and back showed a dense, lymphocytic, folliculocentric and perivascular infiltrate of predominantly CD4-positive T cells consistent with folliculotropic MF. Histological examination of biopsies from ichthyotic skin found similar features. Our patient had a histological diagnosis at the age of 15 years, making him the youngest reported patient with either folliculotropic MF or ichthyotic MF.

  1. A novel recessive mutation in the gene ELOVL4 causes a neuro-ichthyotic disorder with variable expressivity

    Science.gov (United States)

    2014-01-01

    Background A rare neuro-ichthyotic disorder characterized by ichthyosis, spastic quadriplegia and intellectual disability and caused by recessive mutations in ELOVL4, encoding elongase-4 protein has recently been described. The objective of the study was to search for sequence variants in the gene ELOVL4 in three affected individuals of a consanguineous Pakistani family exhibiting features of neuro-ichthyotic disorder. Methods Linkage in the family was searched by genotyping microsatellite markers linked to the gene ELOVL4, mapped at chromosome 6p14.1. Exons and splice junction sites of the gene ELOVL4 were polymerase chain reaction amplified and sequenced in an automated DNA sequencer. Results DNA sequence analysis revealed a novel homozygous nonsense mutation (c.78C > G; p.Tyr26*). Conclusions Our report further confirms the recently described ELOVL4-related neuro-ichthyosis and shows that the neurological phenotype can be absent in some individuals. PMID:24571530

  2. Perinatal-lethal Gaucher disease presenting as hydrops fetalis.

    Science.gov (United States)

    BenHamida, Emira; Ayadi, Imene; Ouertani, Ines; Chammem, Maroua; Bezzine, Ahlem; BenTmime, Riadh; Attia, Leila; Mrad, Ridha; Marrakchi, Zahra

    2015-01-01

    Perinatal-lethal Gaucher disease is very rare and is considered a variant of type 2 Gaucher disease that occurs in the neonatal period. The most distinct features of perinatal-lethal Gaucher disease are non-immune hydrops fetalis. Less common signs of the disease are hepatosplenomegaly, ichthyosis and arthrogryposis. We report a case of Gaucher's disease (type 2) diagnosed in a newborn who presented with Hydrops Fetalis.

  3. Hyperkeratosis of the nipple and areola.

    Science.gov (United States)

    Kuhlman, D S; Hodge, S J; Owen, L G

    1985-10-01

    Hyperkeratosis of the nipple and areola is a rare condition. We report two cases of hyperkeratosis of the nipple and areola occurring in men with no underlying endocrinopathy or synthetic estrogenic drug therapy. Both patients demonstrated prompt resolution of the hyperkeratosis of the nipples with a keratolytic gel. Because our cases were not associated with ichthyosis or epidermal nevus, they best fit into the category of nevoid hyperkeratosis of the nipples.

  4. Opposed-phase MR imaging of lipid storage myopathy in a case of Chanarin-Dorfman disease

    International Nuclear Information System (INIS)

    Gaeta, Michele; Celona, Antonio; Racchiusa, Sergio; Mazziotti, Silvio; Minutoli, Fabio; Toscano, Antonio; Musumeci, Olimpia

    2008-01-01

    Chanarin-Dorfman disease (CDD) is a rare genetic disorder characterized by ichthyosis, myopathy, central nervous system disturbances, and intracellular lipid storage in muscle fibers, hepatocytes, and granulocytes. We describe skeletal muscle magnetic resonance imaging findings in a case of CDD, outlining the potential role of GE T1-weighted opposed-phase sequence (chemical shift imaging) in the evaluation of lipid storage myopathies. (orig.)

  5. Tay's syndrome: MRI

    Energy Technology Data Exchange (ETDEWEB)

    Porto, L.; Reichel, P.; Lanfermann, H.; Zanella, F.E. [Institut of Neuroradiology, Johann Wolfgang Goethe-University, Frankfurt/Main (Germany); Weis, R. [Neuropediatric Department, Johann Wolfgang Goethe-University, Frankfurt/Main (Germany); Schulz, C. [Dermatology Department, Johann Wolfgang Goethe-University, Frankfurt/Main (Germany)

    2000-11-01

    Tay's syndrome is a trichothiodystrophy associated with congenital ichthyosis. We report the findings on MRI and spectroscopy in a young girl with sparse, short, ruffled hair, dry skin and delayed milestones. T2-weighted images showed prominent diffuse confluent increase in signal symmetrically in all the supratentorial white matter. These findings are similar to those in a previously described case, and consistent with dysmyelination. Spectroscopy showed increased myoinositol and decreased choline. (orig.)

  6. Cerebellar and cerebral atrophy in trichothiodystrophy

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Hye-Kyung; Sargent, Michael A.; Poskitt, Kenneth J. [British Columbia Children' s Hospital, Department of Radiology, Vancouver, BC (Canada); Prendiville, Julie S. [British Columbia Children' s Hospital, Division of Paediatric Dermatology, Department of Paediatrics, Vancouver, BC (Canada)

    2005-10-01

    Trichothiodystrophy is a rare neuroectodermal disorder of autosomal recessive inheritance that is characterized by brittle hair, nail dysplasia, ichthyosis, mental retardation, and gonadal failure. We describe a female patient whose cranial MRI revealed almost total lack of myelination in the supratentorial white matter, which is similar to the previously described cases. In addition, there was progressive cerebellar and cerebral atrophy, which has not been well documented in association with trichothiodystrophy. (orig.)

  7. Ichtryosis Linearis Circumflexa

    Directory of Open Access Journals (Sweden)

    A J Kanwar

    1987-01-01

    Full Text Available A case of inchthosis linearis circumflexa occurring in the same family having classical migratory polycyclic skin lesion and flexural hyperkeratosis since infancy of ichthyosis linearis circumflexa multiple serpiginous polycyclic acteristic double edged scales. The patient had unusual features like pruritus and thickening of the plams and soles. One younger sib of the patient appeared to have lamellar ichtyosis. The response to therapy was not satisfactory.

  8. Opposed-phase MR imaging of lipid storage myopathy in a case of Chanarin-Dorfman disease

    Energy Technology Data Exchange (ETDEWEB)

    Gaeta, Michele; Celona, Antonio; Racchiusa, Sergio; Mazziotti, Silvio [University of Messina, Department of Radiological Sciences, Messina (Italy); Minutoli, Fabio [University of Messina, Department of Radiological Sciences, Messina (Italy); A.O.U. ' ' Policlinico G. Martino' ' , Dipartimento di Scienze Radiologiche, Messina (Italy); Toscano, Antonio; Musumeci, Olimpia [University of Messina, Department of Neurosciences, Psychiatry and Anaesthesiology, Messina (Italy)

    2008-11-15

    Chanarin-Dorfman disease (CDD) is a rare genetic disorder characterized by ichthyosis, myopathy, central nervous system disturbances, and intracellular lipid storage in muscle fibers, hepatocytes, and granulocytes. We describe skeletal muscle magnetic resonance imaging findings in a case of CDD, outlining the potential role of GE T1-weighted opposed-phase sequence (chemical shift imaging) in the evaluation of lipid storage myopathies. (orig.)

  9. Netherton′s Syndrome

    Directory of Open Access Journals (Sweden)

    M L Khatri

    1989-01-01

    Full Text Available A 6 year old Libyan boy had diffuse erythema at birth and later developed pruritic, maculo-papular, papular, circinat c, double-edge, scaly lesions, suggestive of ichthyosis linearis circumflexa (ILC.Hisscalp hair were brittle and sparse with partial patchy alopecia, showing change of trichorrhexis invaginata, these -associations being characteristic of Netherton′s syndrome. The boy had slightly stunted growth; a feature which has not been recorded in previously reported cases.

  10. Acral peeling skin syndrome associated with a novel CSTA gene mutation.

    Science.gov (United States)

    Muttardi, K; Nitoiu, D; Kelsell, D P; O'Toole, E A; Batta, K

    2016-06-01

    Acral peeling skin syndrome (APSS) is a rare autosomal recessive condition, characterized by asymptomatic peeling of the skin of the hands and feet, often linked to mutations in the gene TGM5. However, more recently recessive loss of function mutations in CSTA, encoding cystatin A, have been linked with APSS and exfoliative ichthyosis. We describe the clinical features in two sisters with APSS, associated with a novel large homozygous deletion encompassing exon 1 of CSTA. © 2015 British Association of Dermatologists.

  11. Chanarin-Dorfman Syndrome with Multi-System Involvement in Two Siblings

    Directory of Open Access Journals (Sweden)

    Seçil Arslansoyu Çamlar

    2013-03-01

    Full Text Available Chanarin-Dorfman syndrome (CDS is a very rare autosomal recessive inherited neutral lipid metabolism disorder associated with congenital ichthyosis and multi-system involvement. Observation of lipid vacuoles in neutrophils (Jordan’s anomaly in peripheral blood smears in patients with ichthyosiform erythroderma is diagnostic. Herein we present 2 siblings with CDS that were referred to Dokuz Eylul University School of Medicine Department of Pediatrics due to ichthyosis. They had hepatomegaly, cataract, growth retardation, and sensorineural hearing loss. Some lipid vacuoles in neutrophils were noted in peripheral blood smear evaluation. Genetic analysis showed homozygous N209X mutation in both patients. They were put on a low-fat high-carbohydrate diet supplemented with medium-chain fatty acids. During 6 months of follow-up, no improvement was observed in both patients. In conclusion, although CDS is a rare lipid storage disease, it should always be a consideration in patients with congenital ichthyosis, especially those with extracutaneous symptoms or signs. The diagnosis of CDS is made based on a very simple test-peripheral blood smear.

  12. An improved FT-TIMS method of measuring uranium isotope ratios in the uranium-bearing particles

    International Nuclear Information System (INIS)

    Chen, Yan; Wang, Fan; Zhao, Yong-Gang; Li, Li-Li; Zhang, Yan; Shen, Yan; Chang, Zhi-Yuan; Guo, Shi-Lun; Wang, Xiao-Ming; Cui, Jian-Yong; Liu, Yu-Ang

    2015-01-01

    An improved method of Fission Track technique combined with Thermal Ionization Mass Spectrometry (FT-TIMS) was established in order to determine isotope ratio of uranium-bearing particle. Working standard of uranium oxide particles with a defined diameter and isotopic composition were prepared and used to review the method. Results showed an excellent agreement with certified values. The developed method was used to analyze isotope ratio of single uranium-bearing particle in swipe samples successfully. The analysis results of uranium-bearing particles in swipe samples accorded with the operation history of the origin. - Highlights: • The developed method was successfully applied in the analysis of real swipe sample. • Uranium-bearing particles were confined in the middle of track detector. • The fission tracks of collodion film and PC film could be confirmed each other. • The thickness of collodion film should be no more than about 60 μm. • The method could avoid losing uranium-bearing particles in the etching step.

  13. Elucidation of the Synthetic Mechanism of Acylceramide, an Essential Lipid for Skin Barrier Function.

    Science.gov (United States)

    Ohno, Yusuke

    2017-01-01

    The primary function of the skin is to act as a permeability barrier that prevents water loss from inside the body and external invasion such as by pathogens, harmful substances, and allergens. Lipids play a critical role in skin barrier formation by forming multi-lamellar structures in the stratum corneum, the outermost cell layer of the epidermis. Ceramide, the backbone of sphingolipids, accounts for more than 50% of the stratum corneum lipids. Acylceramides are epidermis-specific ceramide species essential for skin barrier formation. Decreases in acylceramide levels and changes in ceramide composition and chain-length are associated with such cutaneous disorders as ichthyosis, atopic dermatitis, and psoriasis. Acylceramide consists of a long-chain base and an amide-linked ultra-long-chain fatty acid (ULCFA, 28-36 carbon chain), which is ω-hydroxylated and esterified with linoleic acid. Although the molecular mechanism by which acylceramide is generated has not been fully understood for decades, we recently identified two genes, CYP4F22 and PNPLA1, involved in acylceramide synthesis and elucidated the entire biosynthetic pathway of acylceramide: the synthesis of ULCFA by ELOVL1 and ELOVL4, ω-hydroxylation of the ULCFA by CYP4F22, amide-bond formation with a long-chain base by CERS3, and transacylation of linoleic acid from triacylglycerol to ω-hydroxyceramide by PNPLA1 to generate acylceramide. CYP4F22 and PNPLA1 are the causative genes of ichthyosis. We demonstrated that mutations of CYP4F22 or PNPLA1 markedly reduced acylceramide production. Our recent findings provide important insights into the molecular mechanisms of skin barrier formation and of ichthyosis pathogenesis.

  14. Expecting the most unexpected – a harlequin baby! A case report and literature analysis

    Directory of Open Access Journals (Sweden)

    Sundaramoorthy M. Srinivasan

    2012-10-01

    Full Text Available Twenty eight years old primi gave birth to an amazing live baby by vaginal delivery – a Harlequin. The child was born with massive, diamond-shaped scales which limit the child’s movements. The term harlequin comes from the baby’s facial expression and diamond-shaped pattern of the scales like the 17th century entertainers, harlequins. As the skin cracks at places of movement the protective function of the skin is lost. Till now the prenatal diagnosis was based on biopsy of fetal skin carried out in an advanced stage of pregnancy. The most important indication of fetal cutaneous biopsy is the diagnosis of genodermatosis and hereditary diseases including Harlequin ichthyosis. It is predictable that for Harlequin Ichthyosis the prenatal test based on DNA will replace the fetal biopsy made before tenth week of gestation by chorionic villus sampling, or even before, with non-invasive analysis of the DNA of fetal cells in maternal circulation. Advances in neonatal intensive care along with scaling being made easier by the use of systemic retinoids has led to improved survival and the use of the name “Harlequin baby” to “Harlequin fetus”, “World’s Largest Baby” or “World’s Smallest Baby”. The mortality rate for harlequin ichthyosis is high. With neonatal intensive care and the advent of retinoid therapy, some babies have survived the newborn period. They are still at risk of dying from systemic infection. It’s the world’s most unconquired medical challenge.

  15. Physical and visual state of 100-year-old Lippman color photographs

    Science.gov (United States)

    Alschuler, William R.

    1998-02-01

    In 1891 Gabriel Lippmann demonstrated a photographic process which records full color images on black and white emulsions. It is closely related to Denisyuk's reflection holography technique, which he named after Lippmann. After a historical introduction I will speak on the physical state and visual appearance of Lippmann-process color images produced at the turn of the century by Lippmann and a limited number of other practitioners. Images made on albumin, collodion and gelatine will be discussed, based on my examination of more than 400 images held in public and private collections. Examples of old original images (and some new work) will be shown. It is possible to conclude that, if properly excited, such images will be stable in their beautiful rendition of color and extremely high resolution almost indefinitely.

  16. THE COLLOIDAL BEHAVIOR OF EDESTIN

    Science.gov (United States)

    Hitchcock, David I.

    1922-01-01

    1. It has been shown by titration experiments that the globulin edestin behaves like an amphoteric electrolyte, reacting stoichiometrically with acids and bases. 2. The potential difference developed between a solution of edestin chloride or acetate separated by a collodion membrane from an acid solution free from protein was found to be influenced by salt concentration and hydrogen ion concentration in the way predicted by Donnan's theory of membrane equilibrium. 3. The osmotic pressure of such edestin-acid salt solutions was found to be influenced by salt concentration and by hydrogen ion concentration in the same way as is the potential difference. 4. The colloidal behavior of edestin is thus completely analogous to that observed by Loeb with gelatin, casein, and egg albumin, and may be explained by Loeb's theory of colloidal behavior, which is based on the idea that proteins react stoichiometrically as amphoteric electrolytes and on Donnan's theory of membrane equilibrium. PMID:19871959

  17. Ichthyosiform scaling secondary to megavoltage radiotherapy

    International Nuclear Information System (INIS)

    Ross, E.V.

    1991-01-01

    Acquired ichthyosis is a rare dermatosis associated with a number of malignancies. Side effects seen on the skin secondary to megavoltage radiotherapy are uncommon but may include fine dry desquamation and tanning. The authors present a case of ichthyosiform scaling limited to the radiation fields in a patient treated for brain metastases of a primary small cell lung carcinoma. The reader is reminded that side effects of megavoltage treatment do occur on the skin. A brief review of these effects is included. 5 references

  18. Despite the hair failing, nails thrive…

    Directory of Open Access Journals (Sweden)

    Samipa Samir Mukherjee

    2017-01-01

    Full Text Available Ectodermal dysplasias are defined as a group of congenital, nonprogressive, developmental syndromes with primary disorders in at least two ectoderm-derived structures namely eccrine glands, hair, nail and teeth. Pure hair and nail affecting ectodemal dysplasias are exceedingly rare in occurance and have been known to be associated with multiple abnormalities, such as keratoderma or ichthyosis, skeletal and hematological abnormalities, cardiac irregularities, mental or psychomotor retardation and cataracts. We herein report a case of hypotrichosis with trichorrhexis nodosa and twenty nail dystrophy- a hypohidrotic ectodermal dysplasia variant.

  19. Xeroderma Pigmentosum-Trichothiodystrophy overlap patient with novel XPD/ERCC2 mutation

    DEFF Research Database (Denmark)

    Kralund, Henrik H; Ousager, Lilian; Jaspers, Nicolaas G

    2013-01-01

    outcome from many of these mutations. We demonstrate a patient, believed to represent an overlap between XP and TTD/CS. In addition to other organ dysfunctions, the young man presented with Photosensitivity, Ichthyosis, Brittle hair, Impaired physical and mental development, Decreased fertility and Short...... appearance also suggested XP, but fibroblast cultures only demonstrated x2 UV-sensitivity with expected NER and TFIIH-activity decrease. Genetic sequencing of the XPD/ERCC2 gene established the patient as heterozygote compound with a novel, N-terminal Y18H mutation and a known C-terminal (TTD) mutation, A725...

  20. Hepatic hypervitaminosis A: a familial observation.

    Science.gov (United States)

    Sarles, J; Scheiner, C; Sarran, M; Giraud, F

    1990-01-01

    Four siblings with hepatic fibrosis are described. The liver damage in these patients was secondary to chronic ingestion of massive doses of vitamin A for congenital ichthyosis. Although the extrahepatic manifestations were helpful in the diagnosis of hypervitaminosis A, the distinctive features of hepatic histopathology were confirmatory. The plasma concentrations of vitamin A and retinol-binding protein were misleading. The recovery from the liver damage in these patients was slow despite a complete withdrawal of the vitamin A intake. These cases show the importance of hepatic vitamin A assessment in the diagnosis of hepatic fibrosis.

  1. A designated centre for people with disabilities operated by North West Parents and Friends Association, Sligo

    LENUS (Irish Health Repository)

    Ugezu, C H

    2017-07-01

    Harlequin Ichthyosis is a very rare genetic disorder affecting mainly the skin with severe morbidity and mortality. It affects both sexes with incidence of about 1 in 300,000 live births. Autosomal recessive inheritance has been inferred with mutation in ABCA 12 gene identified. Hence, genetic counseling and mutation screening of this gene should be considered in at-risk patients. Death usually occurred in the first 3 months of life due to sepsis, feeding problems and respiratory distress. With improved neonatal care and early introduction of retinoids, its survival rate has increased.

  2. Central osteosclerosis with trichothiodystrophy

    Energy Technology Data Exchange (ETDEWEB)

    Wakeling, Emma L.; Brady, Angela F. [North West Thames Regional Genetics Service, Kennedy-Galton Centre, Level 8 V, North West London Hospitals NHS Trust, Watford Road, HAI 3UJ, Harrow, Middlesex (United Kingdom); Cruwys, Michele [Department of Paediatrics, Hillingdon Hospital, Hillingdon, Middlesex (United Kingdom); Suri, Mohnish [Clinical Genetics Service, City Hospital, Nottingham (United Kingdom); Aylett, Sarah E. [Neurosciences Unit, Great Ormond Street Hospital for Children NHS Trust, London (United Kingdom); Hall, Christine [Department of Radiology, Great Ormond Street Hospital for Children NHS Trust, London (United Kingdom)

    2004-07-01

    Trichothiodystrophy (TTD) is a rare, autosomal recessive, multisystem disorder associated with defects in nucleotide excision repair. We report a 7-year-old boy with TTD due to mutation in the XPD gene. The patient has classic features of this condition, including brittle, sulphur-deficient hair, ichthyosis, growth retardation and developmental delay. In addition, he has radiological evidence of progressive central osteosclerosis. Although similar radiological findings have previously been reported in a small number of patients, this association is not widely recognised. We review the radiological findings in this and other similar cases and discuss the natural history of these bony changes. (orig.)

  3. Congenital upper eyelids ectropion in Down’s syndrome

    Directory of Open Access Journals (Sweden)

    Corredor-Osorio, Rafael

    2017-02-01

    Full Text Available Congenital bilateral ectropion of the upper eyelids is a rare, benign condition reported in ophthalmic literature. It is more frequently associated with Down’s syndrome, ichthyosis, and sporadic cases in newborns from black population. We report three cases of congenital bilateral upper eyelid ectropion associated with Down’s syndrome. Management of these patients usually requires medial and lateral canthoplasties, full-thickness pentagonal resection of the upper eyelids and placement of skin grafts. We present herein the evolution of one of these patients and we will discuss the mechanism of the eyelid ectropion and its treatment.

  4. A Turkish family with Sjögren-Larsson syndrome caused by a novel ALDH3A2 mutation

    Directory of Open Access Journals (Sweden)

    Faruk Incecik

    2013-01-01

    Full Text Available Sjögren-Larsson syndrome (SLS is an inherited neurocutaneous disorder caused by mutations in the aldehyde dehydrogenase family 3 member A2 (ALDH3A2 gene that encodes fatty aldehyde dehydrogenase. Affected patients display ichthyosis, mental retardation, and spastic diplegia. More than 70 mutations in ALDH3A2 have been discovered in SLS patients. We diagnosed two brothers age of 12 and 20 years with characteristic features of this rare syndrome. Magnetic resonance imaging showed demyelinating disease in both of them. We described a novel homozygous, c. 835 T > A (p.Y279N mutation in exon 6 in two patients.

  5. Estudio molecular en dos hermanas afectadas por ictiosis congénita autosómica recesiva : descripción de una nueva mutación causal en TGM1

    OpenAIRE

    Moreno Saboya, Meyid Bernardo

    2016-01-01

    Se describe la variante homocigota c.320-2A>G de TGM1 en dos hermanas con ictiosis congénita autosómica recesiva. El clonaje de los transcritos generados por esta variante permitió identificar tres mecanismos moleculares de splicing alternativos. The variant c.320-2A> G of TGM1 is described in two sisters with autosomal recessive congenital ichthyosis. The cloning of the transcripts generated by this variant allowed the identification of three alternative molecular splicing mechanisms. ...

  6. Severe steatohepatitis in a patient with a rare neutral lipid storage disorder due to ABHD5 mutation.

    Science.gov (United States)

    Ronchetti, Anna; Prati, Daniele; Pezzotta, Maria Grazia; Tavian, Daniela; Colombo, Roberto; Callea, Francesco; Colli, Agostino

    2008-09-01

    Fatty liver disease is mainly caused by alcohol consumption, excessive body weight, dyslipidemia and impaired glucose tolerance, but inherited disorders can sometimes be involved. We report the case of a 40-year-old woman with steatohepatitis and severe portal hypertension, associated with ichthyosis, cataract and hypoacusia. The clinical, pathological and genetic findings were consistent with a diagnosis of Chanarin-Dorfman syndrome (CDS), a rare autosomal recessive inherited neutral lipid storage disorder, and genetic analysis showed that a novel ABHD5 mutation is responsible.

  7. Novel duplication mutation in the patatin domain of adipose triglyceride lipase (PNPLA2) in neutral lipid storage disease with severe myopathy.

    Science.gov (United States)

    Akiyama, Masashi; Sakai, Kaori; Ogawa, Masaya; McMillan, James R; Sawamura, Daisuke; Shimizu, Hiroshi

    2007-12-01

    Recently, mutations in PNPLA2 encoding adipose triglyceride lipase (ATGL) were reported to underlie a neutral lipid storage disease (NLSD) subgroup characterized by mild myopathy and the absence of ichthyosis. In the present study a novel homozygous PNPLA2 mutation c.475_478dupCTCC (p.Gln160ProfsX19) in the patatin domain, the ATGL active site, was detected in a woman with NLSD and severe myopathy. The present results suggest that a premature truncation mutation in the patatin domain causes NLSD with severe myopathy.

  8. State of hydration and electrical conductance of ichthyotic skin

    OpenAIRE

    A B Gupta; Manisha Bhattacharya; B Haldar

    1990-01-01

    Dry skin of twelve subjects suffering from ichthyosis vulgaris and the efficacy of a moisturiser-Cotaryl were quantitatively assessed by measuring the skin surface hydration and high frequency (3.5 MHz) electrical conductance of skin. The state of hydration and conductance of ichthyotic skin were 86.9 + 24.6 and 11.0 + 5.7 micro-mho respectively, being much less-compared to 132. 0 + 5.3 and 72.5 + 54.0 micro-mho ofnormal subjects. The moisturiser increased the state of hydration and also the ...

  9. Report of the unannounced inspection at Louth County Hospital, Dundalk, Co Louth

    LENUS (Irish Health Repository)

    Ugezu, C H

    2017-07-01

    Harlequin Ichthyosis is a very rare genetic disorder affecting mainly the skin with severe morbidity and mortality. It affects both sexes with incidence of about 1 in 300,000 live births. Autosomal recessive inheritance has been inferred with mutation in ABCA 12 gene identified. Hence, genetic counseling and mutation screening of this gene should be considered in at-risk patients. Death usually occurred in the first 3 months of life due to sepsis, feeding problems and respiratory distress. With improved neonatal care and early introduction of retinoids, its survival rate has increased.

  10. Epicuticular wax on cherry laurel (Prunus laurocerasus) leaves does not constitute the cuticular transpiration barrier.

    Science.gov (United States)

    Zeisler, Viktoria; Schreiber, Lukas

    2016-01-01

    Epicuticular wax of cherry laurel does not contribute to the formation of the cuticular transpiration barrier, which must be established by intracuticular wax. Barrier properties of cuticles are established by cuticular wax deposited on the outer surface of the cuticle (epicuticular wax) and in the cutin polymer (intracuticular wax). It is still an open question to what extent epi- and/or intracuticular waxes contribute to the formation of the transpiration barrier. Epicuticular wax was mechanically removed from the surfaces of isolated cuticles and intact leaf disks of cherry laurel (Prunus laurocerasus L.) by stripping with different polymers (collodion, cellulose acetate and gum arabic). Scanning electron microscopy showed that two consecutive treatments with all three polymers were sufficient to completely remove epicuticular wax since wax platelets disappeared and cuticle surfaces appeared smooth. Waxes in consecutive polymer strips and wax remaining in the cuticle after treatment with the polymers were determined by gas chromatography. This confirmed that two treatments of the polymers were sufficient for selectively removing epicuticular wax. Water permeability of isolated cuticles and cuticles covering intact leaf disks was measured using (3)H-labelled water before and after selectively removing epicuticular wax. Cellulose acetate and its solvent acetone led to a significant increase of cuticular permeability, indicating that the organic solvent acetone affected the cuticular transpiration barrier. However, permeability did not change after two subsequent treatments with collodion and gum arabic or after treatment with the corresponding solvents (diethyl ether:ethanol or water). Thus, in the case of P. laurocerasus the epicuticular wax does not significantly contribute to the formation of the cuticular transpiration barrier, which evidently must be established by the intracuticular wax.

  11. Current developments in laser ablation-inductively coupled plasma-mass spectrometry for use in geology, forensics, and nuclear nonproliferation research

    Energy Technology Data Exchange (ETDEWEB)

    Messerly, Joshua D. [Iowa State Univ., Ames, IA (United States)

    2008-08-26

    ensemble. This would cause the analysis to be skewed. The use of a gelatin substrate allows the ablation a particle ensemble without disturbing other particles or the gelatin surface. A method to trap and ablate particles on filter paper using collodion was also investigated. The laser was used to dig through the collodion layer and into the particle ensemble. Both of these methods fix particles to allow spatial resolution of the particle ensembles. The use of vanillic acid as a possible enhancement to ablation was also studied. A vanillic acid coating of the particles fixed on top of the gelatin substrate was not found to have any positive effect on either signal intensity or precision. The mixing of vanillic acid in the collodion solution used to coat the filter paper increased ablation signal intensity by a factor of 4 to 5. There was little effect on precision, though. The collodion on filter paper method and the gelatin method of resolving particles have shown themselves to be possible tools in fighting proliferation of nuclear weapons and material. Future applications of LA-ICP-MS are only limited by the imagination of the investigator. Any material that can be ablated and aerosolized is a potential material for analysis by LA-ICP-MS. Improvements in aerosol transport, ablation chamber design, and laser focusing can make possible the ablation and analysis of very small amounts of material. This may perhaps lead to more possible uses in forensics. A similar method to the one used in Chapter 3 could perhaps be used to match drug residue to the place of origin. Perhaps a link could be made based on the elements leached from the soil by plants used to make drugs. This may have a specific pattern based on where the plant was grown. Synthetic drugs are produced in clandestine laboratories that are often times very dirty. The dust, debris, and unique materials in the lab environment could create enough variance to perhaps match drugs produced there to samples obtained off

  12. A patient case highlighting the myriad of cutaneous adverse effects of prolonged use of hydroxyurea.

    Science.gov (United States)

    Neill, Brett; Ryser, Ted; Neill, John; Aires, Daniel; Rajpara, Anand

    2017-11-15

    Hydroxyurea is an antimetabolite primarily used to treat myeloproliferative disorders, and chronic treatment is associated with many cutaneous adverse effects ranging in severity from ichthyosis to aggressive nonmelanoma skin cancer. We report a 67-year-oldman with a history of polycythemia vera who was referred for management of progressively worsening dorsal hand lesions. The patient presented withhyperpigmentation, ichthyosis, plantar keratoderma, dermatomyositis-like eruptions, two squamous cell carcinomas, and actinic keratoses. The adversereactions observed were acknowledged to be related to chronic hydroxyurea use. The patient underwent Mohs excision of the squamous cell carcinomas and thehydroxyurea was promptly discontinued; subsequent cutaneous improvement of the dermatomyositislike lesions ensued. Another clinically suspicious aggressive squamous cell carcinoma was suspected and the patient was referred to the plastic surgery department for complete excision because of the size of the lesion. The patient remains on periodic dermatology follow up. We report a case that exemplifies the cutaneous adverse effects of chronic hydroxyurea therapy. Although many cases improve after drug discontinuation, strict photoprotection and ongoing surveillance are indicated given the recently proposed premalignant potential of dermatomyositis-like eruptions and the aggressive nature of hydroxyurea-induced nonmelanoma skin cancer.

  13. Symptomatic lipid storage in carriers for the PNPLA2 gene.

    Science.gov (United States)

    Janssen, Mirian C H; van Engelen, Baziel; Kapusta, Livia; Lammens, Martin; van Dijk, Martin; Fischer, Judith; van der Graaf, Marinette; Wevers, Ron A; Fahrleitner, Manuela; Zimmermann, Robert; Morava, Eva

    2013-08-01

    Neutral lipid storage disease comprises a heterogeneous group of inherited disorders characterized by severe accumulation of cytoplasmic triglyceride droplets in several tissues and neutrophils. A novel type of autosomal recessive lipid myopathy due to PNPLA2 mutations was recently described with associated cardiac disease, myopathy and frequent infections, but without ichthyosis. Here we describe the clinical and biochemical characteristics of a long surviving patient and report on four carrier family members with diverse clinical involvement. Interestingly, heterozygous patients show neutral lipid storage in muscle and in the keratocytes of the skin, Jordans' bodies, mild myopathy and frequent infections. Biochemical analysis of fibroblasts obtained from patients revealed increased triglyceride storage and reduced lipid droplet-associated triglyceride hydrolase activity. Together, our data implicate that the wild-type allele cannot fully compensate for the mutated dysfunctional allele of PNPLA2 leading to triglyceride accumulation in muscle and mild myopathy in PNPLA2 mutation carriers. The presence of neutral lipid droplets in the skin in PNPLA2 mutation carriers strengthens the link between NLSD and other neutral lipid storage diseases with ichthyosis.

  14. Você conhece esta síndrome? Do you know this syndrome?

    Directory of Open Access Journals (Sweden)

    Marcela Duarte Villela Benez

    2010-12-01

    Full Text Available Relatamos um caso típico, em um paciente masculino de 20 anos, da síndrome de Sjögren-Larsson, que é uma doença neurocutânea, autossômica recessiva e incapacitante, caracterizada por ictiose congênita, plegia espástica e retardo mental. É causada pela deficiência da enzima aldeído graxo desidrogenase. Não tem cura, porém a maioria dos pacientes sobrevive até a idade adulta. O tratamento deve ser multidisciplinar e a terapia dermatológica tem o objetivo de aliviar o prurido persistente e a ictiose.We report a typical case of Sjogren-Larsson syndrome in a male patient, aged 20. The Sjogren-Larsson syndrome is a neurocutaneous, autosomal recessive and disabling condition, characterized by congenital ichthyosis, spastic paraplegia and mental retardation. It is caused by deficiency of the microsomal enzyme fatty aldehyde dehydrogenase. It has no cure, but most patients survive up to an adult age. Treatment should be multidisciplinary and dermatological therapy aims at relieving the persistent itching and ichthyosis.

  15. Cutaneous manifestations of non-Hodgkin's lymphoma.

    Science.gov (United States)

    Kumar, S S; Kuruvilla, M; Pai, G S; Dinesh, M

    2003-01-01

    Thirty-two confirmed cases of non -Hodgkin's lymphoma (NHL) were examined for cutaneous manifestations for a period of 2 years from November 1998 in KMC Hospital Attavar, Mangalore. Cutaneous manifestations in the study group were compared to a control group of 32 patients. Specific infiltrates were present in all (5/5) CTCL patients and one out of twenty-seven patients with low grade NHL. Morphologically they presented as papules, plaques, nodules and erythroderma. Infective conditions seen in the study group were superficial fungal (7/32) and viral infections (2/ 32). Non-infective conditions were acquired ichthyosis (10/32), generalised pruritus (5/32), insect bite reaction (1/32) and drug eruption (1/32). When compared to control patients only acquired ichthyosis and generalised pruritus were found to be statistically significant. The study group also showed changes due to chemotherapy like diffuse alopecia (24/29), bluish pigmentation of proximal part of nail (4/29), localised pigmentation of palms and soles (1 /29), diffuse pigmentation at injection site (1 /29), pigmentation at scar site (1 /29) and stomatitis (4/29).

  16. Neu-Laxova syndrome in an appropriate for gestational age newborn

    Directory of Open Access Journals (Sweden)

    Dilli Dilek

    2008-01-01

    Full Text Available Neu-Laxova syndrome is a rare lethal congenital disorder involving multiple systems. Intrauterine growth retardation, ichthyosis, microcephaly, abnormal facial findings, and limb contractures are its key features. We present a case of Neu-Laxova syndrome in a male appropriate for gestational age (AGA newborn with characteristic features including ichthyosis, microcephaly, severe ectropion, rudimentary ears, eclabion, limb contractures, and hypoplastic genitalia. The patient was born at 38 weeks of gestation to consanguinous Turkish parents. The mother was a 20-year-old primi gravida with lack of prenatal follow-up. Therefore, the case was diagnosed postnatally, and he died 5 days later. Because of the autosomal recessive inheritance of Neu-Laxova syndrome, in countries with high rates of consanguineous marriage, such as Turkey, physicians have to know this syndrome, and serial prenatal ultrasound examinations with genetic counseling should be performed on pregnant women at high risk. To the best of our knowledge, this is the first case described in an AGA newborn.

  17. Metabolism of very long-chain Fatty acids: genes and pathophysiology.

    Science.gov (United States)

    Sassa, Takayuki; Kihara, Akio

    2014-02-01

    Fatty acids (FAs) are highly diverse in terms of carbon (C) chain-length and number of double bonds. FAs with C>20 are called very long-chain fatty acids (VLCFAs). VLCFAs are found not only as constituents of cellular lipids such as sphingolipids and glycerophospholipids but also as precursors of lipid mediators. Our understanding on the function of VLCFAs is growing in parallel with the identification of enzymes involved in VLCFA synthesis or degradation. A variety of inherited diseases, such as ichthyosis, macular degeneration, myopathy, mental retardation, and demyelination, are caused by mutations in the genes encoding VLCFA metabolizing enzymes. In this review, we describe mammalian VLCFAs by highlighting their tissue distribution and metabolic pathways, and we discuss responsible genes and enzymes with reference to their roles in pathophysiology.

  18. Four Gaucher disease type II patients with three novel mutations: a single centre experience from Turkey.

    Science.gov (United States)

    Bulut, Fatma Derya; Kör, Deniz; Şeker-Yılmaz, Berna; Hergüner, Özlem; Ceylaner, Serdar; Özkınay, Ferda; Kılavuz, Sebile; Önenli-Mungan, Neslihan

    2018-04-14

    Gaucher disease is the most common lysosomal storage disorder due to glucosylceramidase enzyme deficiency. There are three subtypes of the disease. Neurological involvement accompanies visceral and haematological findings only in type II and type III Gaucher patients. Type II is the acute progressive neuronopathic form which is the most severe and rare subtype. Clinical findings are recognized prenatally or in the first months of life and followed by death within the first two years of age. Among our 81 Gaucher patients, we identified 4 (4,9%) type II patients in our metabolic centre. This rate is significantly higher than the rate reported in the literature (Gaucher patients with three novel mutations and one perinatal lethal form with generalized ichthyosis which is a very rare disorder. Additionally, we would like to highlight the phenotypic heterogeneity not only between the subtypes, also even in the same type.

  19. Filaggrin gene polymorphism associated with Epstein-Barr virus-associated tumors in China.

    Science.gov (United States)

    Yang, Yang; Liu, Wen; Zhao, Zhenzhen; Zhang, Yan; Xiao, Hua; Luo, Bing

    2017-08-01

    Mutations of filaggrin gene (FLG) have been identified as the cause of ichthyosis vulgaris, while recently FLG mutations were found to be associated with gastric cancer. This study aimed to investigate the association of filaggrin polymorphism with Epstein-Barr virus-associated tumors in China. A total of 200 patients with three types of tumors and 117 normal control samples were genotyped at three common FLG mutation loci (rs3126085, K4671X, R501X) by using Sequenom MassARRAY technique. The χ 2 test was used to evaluate the relationship between the mutation and the three kinds of tumors. A two-sided P value of Epstein-Barr virus (EBV)-associated gastric carcinoma (EBVaGC) and EBV-negative gastric carcinoma (EBVnGC), respectively. Furthermore, allele distributions in EBVaGC and EBVnGC were verified to be different in both SNP loci.

  20. A man with nevoid hyperkeratosis of the areola.

    Science.gov (United States)

    English, J C; Coots, N V

    1996-05-01

    Nevoid hyperkeratosis of the nipple and areola is a unique clinical entity and a diagnosis of exclusion. The patient presenting with nipple/areolar hyperkeratosis must be examined carefully for other underlying cutaneous diseases such as epidermal nevi, ichthyosis, acanthosis nigricans, Darier's disease, cutaneous T-cell lymphoma, and other chronic skin dermatitides that may be responsible for the changes. If no other clinical findings are evident, the diagnosis can be made. Although the disorder is more common in women of childbearing age, men may show nevoid changes after estrogen therapy or idiopathically. The use of topical 12 percent lactic acid lotion (Lac-Hydrin) resolves the skin changes over a six-month period.

  1. Management of dental trauma in a child with Xeroderma Pigmentosa

    Directory of Open Access Journals (Sweden)

    Nidhi Agarwal

    2018-01-01

    Full Text Available Xeroderma Pigmentosa is a rare dermatological autosomal recessive disorder that manifests itself early in life as severe sunburn usually after a short exposure to sunlight. The prime characteristic features include photosensitivity, hyperpigmentation and ichthyosis in sun exposed areas, and an increase in the risk of basocellular and squamous cell carcinomas and melanomas of the skin and eyes. The case report highlights the preventive treatment options along with all necessary precautions that should be taken to protect the patient from any iatrogenic inadvertent exposures that may be deleterious to his present state. The purpose of the report is also to discuss the important role of dental professionals when dealing with debilitating medical conditions.

  2. Atopic eczema and the filaggrin story.

    Science.gov (United States)

    Brown, Sara J; Irvine, Alan D

    2008-06-01

    The discovery that null mutations in the filaggrin gene (FLG) are associated with atopic eczema represents the single most significant breakthrough in understanding the genetic basis of this complex disorder. The association has been replicated in multiple independent studies during the past 2 years with the use of various methodologies, from populations in Europe, the United States, and Japan. Filaggrin plays a key role in epidermal barrier function, and its association with atopic eczema emphasizes the importance of barrier dysfunction in eczema pathogenesis. This review aims to summarize the current state of knowledge regarding the role of FLG mutations in ichthyosis vulgaris, atopic eczema, and other skin disorders, with an emphasis on potential clinical applications. Further research is needed to clarify the precise role of filaggrin in skin and systemic atopic disease, to pave the way for novel therapeutic interventions.

  3. CONGENITAL EYELID EVERSION - A CASE REPORT WITH REVIEW OF LITERATURE

    Directory of Open Access Journals (Sweden)

    Chinmayee

    2015-03-01

    Full Text Available INTRODUCTION: Congenital eversion of the eyelids, a condition usually seen shortly after birth is a rare condition affecting one or both eyelids of the eye. Many etiologies have been described but no specific cause has been identified. We report a case of congenital eve rsion of eyelids which was managed conservatively with spontaneous correction seen in 6 days. KEYMESSAGE: Congenital eversion of the eyelids is a rare easily identifiable and potentially treatable condition. Decision of surgical intervention has to be take n with care and must be individualized for each case. Congenital eversion must be differentiated from congenital ectropion due to causes like ichthyosis as the treatment widely differs. All ophthalmologists need to be aware of this condition as it can be e asily managed on an outpatient basis

  4. Dorfman-Chanarin syndrome

    Directory of Open Access Journals (Sweden)

    Gandhi Vijay

    2007-01-01

    Full Text Available A four-year-old girl was brought to the dermatology outpatient department with scaling all over the body since birth. She had history of episodic vomiting and abdominal distension. A dermatological diagnosis of lamellar ichthyosis was made. Abdominal examination revealed a nontender hepatomegaly, fatty liver on ultrasonography and deranged liver function tests. Peripheral blood smear showed lipid vacuoles in the granulocytes consistent with Jordans′ anomaly. Similar lipid vacuoles were seen in the basal layer in skin biopsy. An inflammatory infiltrate, moderate fibrosis in the portal tract and diffuse severe fatty change in hepatocytes were seen in liver biopsy. The patient was diagnosed as a case of Dorfman-Chanarin syndrome.

  5. Biofeedback, cognitive-behavioral methods, and hypnosis in dermatology: is it all in your mind?

    Science.gov (United States)

    Shenefelt, Philip D

    2003-01-01

    Biofeedback can improve cutaneous problems that have an autonomic nervous system component. Examples include biofeedback of galvanic skin resistance (GSR) for hyperhidrosis and biofeedback of skin temperature for Raynaud's disease. Hypnosis may enhance the effects obtained by biofeedback. Cognitive-behavioral methods may resolve dysfunctional thought patterns (cognitive) or actions (behavioral) that damage the skin or interfere with dermatologic therapy. Responsive diseases include acne excoriée, atopic dermatitis, factitious cheilitis, hyperhidrosis, lichen simplex chronicus, needle phobia, neurodermatitis, onychotillomania, prurigo nodularis, trichotillomania, and urticaria. Hypnosis can facilitate aversive therapy and enhance desensitization and other cognitive-behavioral methods. Hypnosis may improve or resolve numerous dermatoses. Examples include acne excoriée, alopecia areata, atopic dermatitis, congenital ichthyosiform erythroderma, dyshidrotic dermatitis, erythromelalgia, furuncles, glossodynia, herpes simplex, hyperhidrosis, ichthyosis vulgaris, lichen planus, neurodermatitis, nummular dermatitis, postherpetic neuralgia, pruritus, psoriasis, rosacea, trichotillomania, urticaria, verruca vulgaris, and vitiligo. Hypnosis can also reduce the anxiety and pain associated with dermatologic procedures.

  6. Effect of a new moisturizing lotion on immediate and cumulative skin hydration: Two randomized, intra-individual, vehicle- and comparator-controlled studies.

    Science.gov (United States)

    Nogueira, Alessandra; Sidou, Farzaneh; Brocard, Sylvie

    2011-08-01

    Moisturizers increase skin hydration and can serve as adjunctive care in dermatologic conditions such as xerosis, psoriasis vulgaris, atopic dermatitis and ichthyosis, in which dry skin is implicated. A non-irritating hydrating lotion (CDA lotion) was recently developed. We assessed the effect of CDA lotion on skin hydration in two randomized, evaluator-blind and intra-individual comparison studies. After a single application, CDA lotion induced significantly greater hydration than the non-treated control for at least 24 hours (p skin hydration up to 3 days after treatment cessation (p skin dryness score up to 7 days after treatment cessation (p hydration effects of CDA lotion were also compared to those of several currently available moisturizing products. In summary, application of CDA lotion increases skin hydration and alleviates the condition of skin dryness.

  7. [The role of psychological factors and psychiatric disorders in skin diseases].

    Science.gov (United States)

    Kieć-Swierczyńska, Marta; Dudek, Bohdan; Krecisz, Beata; Swierczyńska-Machura, Dominika; Dudek, Wojciech; Garnczarek, Adrianna; Turczyn, Katarzyna

    2006-01-01

    In this paper, the relation between psychological factors and psychiatric disorders in patients with skin diseases is discussed. On the one hand psychological factors (stress, negative emotions) can influence the generation and aggravation of skin disorders (urticaria, atopic dermatitis, vitiligo), on the other hand psychological disorders can result in some skin diseases (psoriasis, atopic dermatitis). In the majority of cases the quality of life is poorly estimated by patients with skin problems. Psychodermatology is divided into three categories according to the relationship between skin diseases and mental disorders: 1) psychophysiologic disorders caused by skin diseases triggering different emotional states (stress), but not directly combined with mental disorders (psoriasis, eczema); 2) primary psychiatric disorders responsible for self-induced skin disorders (trichotillomania); and 3) secondary psychiatric disorders caused by disfiguring skin (ichthyosis, acne conglobata, vitiligo), which can lead to states of fear, depression or suicidal thoughts.

  8. Loss of corneodesmosin leads to severe skin barrier defect, pruritus, and atopy: unraveling the peeling skin disease.

    Science.gov (United States)

    Oji, Vinzenz; Eckl, Katja-Martina; Aufenvenne, Karin; Nätebus, Marc; Tarinski, Tatjana; Ackermann, Katharina; Seller, Natalia; Metze, Dieter; Nürnberg, Gudrun; Fölster-Holst, Regina; Schäfer-Korting, Monika; Hausser, Ingrid; Traupe, Heiko; Hennies, Hans Christian

    2010-08-13

    Generalized peeling skin disease is an autosomal-recessive ichthyosiform erythroderma characterized by lifelong patchy peeling of the skin. After genome-wide linkage analysis, we have identified a homozygous nonsense mutation in CDSN in a large consanguineous family with generalized peeling skin, pruritus, and food allergies, which leads to a complete loss of corneodesmosin. In contrast to hypotrichosis simplex, which can be associated with specific dominant CDSN mutations, peeling skin disease is characterized by a complete loss of CDSN expression. The skin phenotype is consistent with a recent murine Cdsn knockout model. Using three-dimensional human skin models, we demonstrate that lack of corneodesmosin causes an epidermal barrier defect supposed to account for the predisposition to atopic diseases, and we confirm the role of corneodesmosin as a decisive epidermal adhesion molecule. Therefore, peeling skin disease will represent a new model disorder for atopic diseases, similarly to Netherton syndrome and ichthyosis vulgaris in the recent past.

  9. Acral peeling skin syndrome resulting from a homozygous nonsense mutation in the CSTA gene encoding cystatin A.

    Science.gov (United States)

    Krunic, Aleksandar L; Stone, Kristina L; Simpson, Michael A; McGrath, John A

    2013-01-01

    Acral peeling skin syndrome (APSS) is a clinically and genetically heterogeneous disorder. We used whole-exome sequencing to identify the molecular basis of APSS in a consanguineous Jordanian-American pedigree. We identified a homozygous nonsense mutation (p.Lys22X) in the CSTA gene, encoding cystatin A, that was confirmed using Sanger sequencing. Cystatin A is a protease inhibitor found in the cornified cell envelope, and loss-of-function mutations have previously been reported in two cases of exfoliative ichthyosis. Our study expands the molecular pathology of APSS and demonstrates the value of next-generation sequencing in the genetic characterization of inherited skin diseases. © 2013 Wiley Periodicals, Inc.

  10. Netherton syndrome in one Chinese adult with a novel mutation in the SPINK5 gene and immunohistochemical studies of LEKTI

    Directory of Open Access Journals (Sweden)

    Zhang Xi-Bao

    2012-01-01

    Full Text Available Background : Netherton syndrome (NS is a severe autosomal recessive ichthyosis. It is characterized by congenital ichthyosiform erythroderma, trichorrhexis invaginata, ichthyosis linearis circumflexa, atopic diathesis, and frequent bacterial infections. The disease is caused by mutations in the SPINK5 (serine protease inhibitor Kazal-type 5 gene, a new type of serine protease inhibitor involved in the regulation of skin barrier formation and immunity. We report one Chinese adult with NS. The patient had typical manifestation of NS except for trichorrhexis invaginata with an atopic diathesis and recurrent staphylococcal infections since birth. Aims: To evaluate the gene mutation and of its product activity of SPINK5 gene in confirmation of the diagnosis of one Chinese adult with NS. Materials and Methods: To screen mutations in the SPINK5 gene, 33 exons and flanking intron boundaries of SPINK5 were amplified with polymerase chain reaction (PCR and used for direct sequencing. In addition, immunohistochemical staining of LEKTI (lymphoepithelial Kazal-type-related inhibitor with specific antibody was used to confirm the diagnosis of NS. The results were compared with that of healthy individuals (twenty-five blood samples. Results: A G318A mutation was found at exon 5 of patient′s SPINK5 gene which is a novel missense mutation. The PCR amplification products with mutation-specific primer were obtained only from the DNA of the patients and their mother, but not from their father and 25 healthy individuals. Immunohistochemical studies indicated there was no LEKTI expression in NS patient′s skin and there was a strong LEKTI expression in the normal human skin. Conclusion: In this report, we describe heterozygous mutation in the SPINK5 gene and expression of LEKTI in one Chinese with NS. The results indicate that defective expression of LEKTI in the epidermis and mutations of SPINK5 gene are reliable for diagnostic feature of NS with atypical

  11. Magnetic Resonance Spectroscopy in Sjögren-Larsson Syndrome

    Directory of Open Access Journals (Sweden)

    Fesih Aktar

    2016-06-01

    Full Text Available Sjögren-Larsson syndrome (SLS is a rare neurocutane­ous disease showing an autosomal recessive transmis­sion due to a lack of fatty acid aldehyde dehydrogenase. Spastic diplegia or triplegia, mental retardation and con­genital lamellar ichthyosis are the major findings of the disease. The syndrome may be accompanied by various eye and teeth features, skeletal system anomaly, speak­ing defects, hypertelorism and epilepsy. A 9-month male patient has been hospitalized for convulsion and flaking on body. The patient history showed that flaking skin thickening and peeling was started at the birth, and he suffered a right-side focused seizure when he was three month-old and he was treated with phenobarbital and car­bamazepine upon the epilepsy diagnosis. Wide ichthyo­sis, hypertelorism and bilateral simian line were observed in the physical examination. Bilateral punctuate lesions in cornea, pigment epithelial atrophy in the right eye and esotropia in the left eye have been determined during the eye examination. An epiteliform anomaly has been ob­served in the left hemisphere by electroencephalography. In brain magnetic resonance imaging (MRI, an increase in cerebral-cerebellar brain parenchyma and T1-T2 relax­ation time and in the signal in corpus callosum (delayed myelination have been determined. With the observa­tion of the white matter in centrum semi oval using brain MRI spectroscopy, signs of a sphingolipid peak at 1.3 ppm have been observed. An SLS diagnosis has been proposed upon clinical and laboratory observations. We want to emphasize on the fact that in epilepsy cases with ichthyosis, SLS should be considered.

  12. STUDY ON NONINFECTIOUS DERMATOSES IN PAEDIATRIC AGE

    Directory of Open Access Journals (Sweden)

    Ananthi Mahalingam

    2017-09-01

    Full Text Available BACKGROUND Paediatric dermatology is a unique subspecialty in that child is not a miniature adult. Paediatric dermatoses differ from that of the adults in clinical presentation, treatment and prognosis. Various studies from India have shown infections and infestations to be the most common paediatric dermatoses. This study was planned to determine the epidemiological pattern of common noninfectious dermatoses in our paediatric patients as no such data are available from this part of the country. A cross-sectional study was undertaken to study the prevalence of the noninfectious dermatoses in all the new paediatric patients attending the Skin Outpatient Department (OPD at Villupuram Medical College over a period of three years. MATERIALS AND METHODS A total number of 550 children in the age group ranging from newborn to 12 years with noninfectious dermatoses attending the OPD for the first time were enrolled in the study. RESULTS Physiological changes of skin was the most common dermatoses in the newborn age group, while eczema was the most common dermatoses in infants, preschool and school going children. In the infants, eczema was followed by pigmentary disorders, mongolian spots, vascular nevi, ichthyosis, epidermolysis bullosa, alopecia areata and papular urticaria in the order of prevalence. Among preschool going children, eczema was followed by papular urticaria, papulosquamous disorders, pigmentary disorders, hair disorders, nevi, drug reactions, keratinisation disorders, urticaria, etc. In the school going age group, eczema was followed by papulosquamous disorders, pigmentary disorders, papular urticaria, nutritional disorders, ichthyosis, nevi, miliaria, drug reaction, hair disorders, photodermatoses, urticaria, collagen vascular disease and vascular nevi in the order of prevalence. CONCLUSION Eczema, papulosquamous disorders, papular urticaria, pigmentary disorders seem to be the most common noninfectious dermatoses in children. However

  13. Rediscovering Ducos du Hauron's Color Photography through a Review of His Three-Color Printing Processes and Synchrotron Microanalysis of His Prints.

    Science.gov (United States)

    Cotte, Marine; Fabris, Tiphaine; Langlois, Juliette; Bellot-Gurlet, Ludovic; Ploye, Françoise; Coural, Natalie; Boust, Clotilde; Gandolfo, Jean-Paul; Galifot, Thomas; Susini, Jean

    2018-06-18

    Louis Ducos du Huron (1837-1920) dedicated his entire life to the elaboration of physical-chemical processes for color photography. This study aimed at highlighting his unique contribution to three-color printing through 1) an in-depth review of the many protocols he published and 2) the synchrotron-based IR and X-ray microanalysis of fragments sampled in three artworks. Ducos du Hauron's method relied on the preparation and assembly of three monochromes (red, blue, yellow). This study brings to light complex multistep recipes based on photochemistry (carbon print), organic, and inorganic chemistry. The various ingredients involved (e.g., pigments, dichromate gelatin, collodion, resin) were identified and localized through their spectroscopic signature, confirming the relevance of synchrotron spectromicroscopy for the characterization of historical photographs. The impressive correlation between texts and chemical analyses calls for a wider application to the history of photography. © 2018 Wiley-VCH Verlag GmbH & Co. KGaA, Weinheim.

  14. Determination of the chemical properties of residues retained in individual cloud droplets by XRF microprobe at SPring-8

    International Nuclear Information System (INIS)

    Ma, C.-J.; Tohno, S.; Kasahara, M.; Hayakawa, S.

    2004-01-01

    To determine the chemical properties of residue retained in individual cloud droplets is primarily important for the understanding of rainout mechanism and aerosol modification in droplet. The sampling of individual cloud droplets were carried out on the summit of Mt. Taiko located in Tango peninsula, Kyoto prefecture, during Asian dust storm event in March of 2002. XRF microprobe system equipped at SPring-8, BL-37XU was applied to the subsequent quantification analysis of ultra trace elements in residues of individual cloud droplets. It was possible to form the replicas of separated individual cloud droplets on the thin collodion film. The two dimensional XRF maps for the residues in individual cloud droplets were clearly drawn by scanning of micro-beam. Also, XRF spectra of trace elements in residues were well resolved. From the XRF spectra for individual residues, the chemical mixed state of residues could be assumed. The chemical forms of Fe (Fe +++ ) and Zn (Zn + ) could be clearly characterized by their K-edge micro-XANES spectra. By comparison of Z/Si mass ratios of residues in cloud droplets and those of the original sands collected in desert areas in China, the aging of ambient dust particles and their in cloud modification were indirectly assumed

  15. On the ex situ and in situ doping of refractory Ta by pulsed laser ablation

    Energy Technology Data Exchange (ETDEWEB)

    Lin, Y.L.; Lin, S.S.; Shen, P. [National Sun Yat-sen University, Department of Materials and Optoelectronic Science, Kaohsiung, Taiwan (China); Chen, S. [I-Shou University, Department of Mechanical and Automation Engineering, Kaohsiung, Taiwan (China)

    2017-06-15

    Ex situ and in situ doping processes of refractory Ta were accomplished by pulsed laser ablation of bulk Ta in vacuum with C-O-H supply from C-coated collodion film and within tetraethyl orthosilicate (TEOS), respectively. Analytical electron microscopic observations of the resultant particulates and condensates indicated that the former process involved interdiffusion in the condensed state to form two kinds of core-shell structures, i.e., C-O-H doped Ta with α-type (bcc) core and lamellar shell and O-H doped γ-TaC{sub 1-x} with amorphous Ta-C-O-H surrounding, respectively, for potential wear and lubrication applications at high temperatures. In situ TEOS mediation, however, enhanced solute trapping to form Si-C-O-H overdoped α-Ta with significant tetragonality (c/a ratio up to 1.27) and Si-O-H doped γ-TaC{sub 1-x} with {111}{sub γ} twin/fault and occasional epitaxial relationship [ anti 123]{sub α}//[ anti 112]{sub γ}; (121){sub α}//(anti 11 anti 1){sub γ} due to particle coalescence. Such Si-C-O-H mediated Ta particles may have potential optocatalytic applications. (orig.)

  16. THE COLLOIDAL BEHAVIOR OF SERUM GLOBULIN

    Science.gov (United States)

    Hitchcock, David I.

    1922-01-01

    1. The globulin prepared from ox serum by dilution and precipitation with carbon dioxide has been found, by electrometric titration experiments, to behave like an amphoteric electrolyte, reacting stoichiometrically with acids and bases. 2. The potential difference developed between a solution of globulin chloride, phosphate, or acetate and a solution of the corresponding acid, free from protein, separated from the globulin by a collodion membrane, was found to be influenced by hydrogen ion concentration and salt concentration in the way predicted by Donnan's theory of membrane equilibrium. In experiments with sodium globulinate and sodium hydroxide it was found that the potential difference could be similarly explained. 3. The osmotic pressure of such solutions could be qualitatively accounted for by the Donnan theory, but exhibited a discrepancy which is explicable by analogy with certain experiments of Loeb on gelatin. 4. The application of Loeb's theory of colloidal behavior, which had previously been found to hold in the case of gelatin, casein, egg albumin, and edestin, has thus been extended to another protein, serum globulin. PMID:19871977

  17. Sex difference in human fingertip recognition of micron-level randomness as unpleasant.

    Science.gov (United States)

    Nakatani, M; Kawasoe, T; Denda, M

    2011-08-01

    We investigated sex difference in evaluation, using the human fingertip, of the tactile impressions of three different micron-scale patterns laser-engraved on plastic plates. There were two ordered (periodical) patterns consisting of ripples on a scale of a few micrometres and one pseudo-random (non-periodical) pattern; these patterns were considered to mimic the surface geometry of healthy and damaged human hair, respectively. In the first experiment, 10 women and 10 men ran a fingertip over each surface and determined which of the three plates felt most unpleasant. All 10 female participants reported the random pattern, but not the ordered patterns, as unpleasant, whereas the majority of the male participants did not. In the second experiment, 9 of 10 female participants continued to report the pseudo-random pattern as unpleasant even after their fingertip had been coated with a collodion membrane. In the third experiment, participants were asked to evaluate the magnitude of the tactile impression for each pattern. The results again indicated that female participants tend to report a greater magnitude of unpleasantness than male participants. Our findings indicate that the female participants could readily detect microgeometric surface characteristics and that they evaluated the random pattern as more unpleasant. Possible physical and perceptual mechanisms involved are discussed. © 2011 The Authors. ICS © 2011 Society of Cosmetic Scientists and the Société Française de Cosmétologie.

  18. Application of Monte Carlo method in study of the padronization for radionuclides with complex disintegration scheme in 4πβ-γ coincidence System

    International Nuclear Information System (INIS)

    Takeda, Mauro Noriaki

    2006-01-01

    The present work described a new methodology for modelling the behaviour of the activity in a 4πβ-γ coincidence system. The detection efficiency for electrons in the proportional counter and gamma radiation in the NaI(Tl) detector was calculated using the Monte Carlo program MCNP4C. Another Monte Carlo code was developed which follows the path in the disintegration scheme from the initial state of the precursor radionuclide, until the ground state of the daughter nucleus. Every step of the disintegration scheme is sorted by random numbers taking into account the probabilities of all β - branches, electronic capture branches, transitions probabilities and internal conversion coefficients. Once the final state was reached beta, electronic capture events and gamma transitions are accounted for the three spectra: beta, gamma and coincidence variation in the beta efficiency was performed simulating energy cut off or use of absorbers (Collodion). The selected radionuclides for simulation were: 134 Cs, 72 Ga which disintegrate by β - transition, 133 Ba which disintegrates by electronic capture and 35 S which is a beta pure emitter. For the latter, the Efficiency Tracing technique was simulated. The extrapolation curves obtained by Monte Carlo were filled by the Least Square Method with the experimental points and the results were compared to the Linear Extrapolation method. (author)

  19. Application of Monte Carlo method in study of the padronization for radionuclides with complex disintegration scheme in 4{pi}{beta}-{gamma} coincidence System; Aplicacao do metodo de Monte Carlo no estudo da padronizacao de radionuclideos com esquema de desintegracao complexos em sistema de coincidencias 4{pi}{beta}-{gamma}

    Energy Technology Data Exchange (ETDEWEB)

    Takeda, Mauro Noriaki

    2006-07-01

    The present work described a new methodology for modelling the behaviour of the activity in a 4{pi}{beta}-{gamma} coincidence system. The detection efficiency for electrons in the proportional counter and gamma radiation in the NaI(Tl) detector was calculated using the Monte Carlo program MCNP4C. Another Monte Carlo code was developed which follows the path in the disintegration scheme from the initial state of the precursor radionuclide, until the ground state of the daughter nucleus. Every step of the disintegration scheme is sorted by random numbers taking into account the probabilities of all {beta}{sup -} branches, electronic capture branches, transitions probabilities and internal conversion coefficients. Once the final state was reached beta, electronic capture events and gamma transitions are accounted for the three spectra: beta, gamma and coincidence variation in the beta efficiency was performed simulating energy cut off or use of absorbers (Collodion). The selected radionuclides for simulation were: {sup 134}Cs, {sup 72}Ga which disintegrate by {beta}{sup -} transition, {sup 133}Ba which disintegrates by electronic capture and {sup 35}S which is a beta pure emitter. For the latter, the Efficiency Tracing technique was simulated. The extrapolation curves obtained by Monte Carlo were filled by the Least Square Method with the experimental points and the results were compared to the Linear Extrapolation method. (author)

  20. Standardization of 201Tl and 55Fe radionuclides in a 4 (PC)-NaI(Tl) coincidence system

    International Nuclear Information System (INIS)

    Pires, Carlos Augusto

    2008-01-01

    In the present work the procedure for the standardization of radionuclides using the 4π(PC)-NaI(Tl) coincidence system was developed. The radionuclides selected were 201 Tl, used in nuclear medicine, and 55 Fe primary standard source, used for x-ray spectrometers calibration. The 4π(PC)-NaI(Tl) is composed of a 4 proportional counter operated at 0.1MPa coupled to two NaI(Tl) crystals. The 201 Tl decays by electron capture process followed by a prompt gamma-ray. The disintegration rate was determined by extrapolation technique using two methods: electronic discrimination and external absorbers. The radioactive sources were prepared in a 20 μg cm -2 thick Collodion film. The conventional electronic system was used. The observed events were registered by the TAC method. The 55 Fe decays by electron capture process to the ground state of 55 Mn, emitting x rays with around 6 keV. The standardization was obtained by the tracing method. This technique was applied using two radionuclides, which decay by electron capture process followed by a prompt gamma-ray, namely 51 Cr and 54 Mn, as tracers. Measurements with 1 and 2 aluminum foils, each 150 g cm-2 thick were carried out. The activity was obtained by extrapolation for zero thickness Al foil. The uncertainties were treated by means of matrix covariance methodology and takes into account all correlations involved. (author)

  1. Cesium 137 concentration in food items common to the Filipino dietary

    International Nuclear Information System (INIS)

    Cruz, B. de la; Marzan, A.M.; Balboa, B.C.; Barruel, D.; Paradero, R.

    1976-03-01

    Radioactivity from environmental contamination reaches the human population primarily in food. This study aims to determine the level of Cs-137 in various items common to the Filipine diet and to estimate the average dose commitment of the average Filipino resulting from the radionuclides. The method used was wet ashing with the addition of HNO 2 to disintegrate the organic material in the sample. Wet ashing was achieved using lower concentrations of hydrogen peroxide. The white residue obtained was mounted on a planchet using 1% collodion solution. Identification and further resolution of the gamma-emitting Cs-137 was made with the use of a Ge-Li detector attached to a multi channel analyzer. Gross beta activity of ash samples were likewise determined using a G-M counter. Shown in tabulated form are the ash content, gross beta and Cs-137 concentrations in fish samples collected from various fishing regions of the country. The values ranged from nil to 10.6 pCi per kg sample with a mean value of 5.6 pCi/kg

  2. Fundamental and methodological investigations for the improvement of elemental analysis by inductively coupled plasma mass soectrometry

    Energy Technology Data Exchange (ETDEWEB)

    Ebert, Christopher Hysjulien [Ames Lab., Ames, IA (United States)

    2012-01-01

    This dissertation describes a variety of studies meant to improve the analytical performance of inductively coupled plasma mass spectrometry (ICP-MS) and laser ablation (LA) ICP-MS. The emission behavior of individual droplets and LA generated particles in an ICP is studied using a high-speed, high frame rate digital camera. Phenomena are observed during the ablation of silicate glass that would cause elemental fractionation during analysis by ICP-MS. Preliminary work for ICP torch developments specifically tailored for the improvement of LA sample introduction are presented. An abnormal scarcity of metal-argon polyatomic ions (MAr{sup +}) is observed during ICP-MS analysis. Evidence shows that MAr{sup +} ions are dissociated by collisions with background gas in a shockwave near the tip of the skimmer cone. Method development towards the improvement of LA-ICP-MS for environmental monitoring is described. A method is developed to trap small particles in a collodion matrix and analyze each particle individually by LA-ICP-MS.

  3. Marketing to Nurses through an Incentive Program.

    Science.gov (United States)

    Campbell, Jeanne Phillips; Williams, Trudy

    1983-01-01

    Describes the Incentive Career Mobility Plan, a program for improving employee morale and retention by rewarding self-improvement. Discusses its use by nurse administrators for marketing their institutions to current and potential employees. (JOW)

  4. Autonomous search

    CERN Document Server

    Hamadi, Youssef; Saubion, Frédéric

    2012-01-01

    Autonomous combinatorial search (AS) represents a new field in combinatorial problem solving. Its major standpoint and originality is that it considers that problem solvers must be capable of self-improvement operations. This is the first book dedicated to AS.

  5. Prospect on general software of Monte Carlo method

    International Nuclear Information System (INIS)

    Pei Lucheng

    1992-01-01

    This is a short paper on the prospect of Monte Carlo general software. The content consists of cluster sampling method, zero variance technique, self-improved method, and vectorized Monte Carlo method

  6. Links between Conflict Management Research and Practice

    Science.gov (United States)

    Roloff, Michael E.

    2009-01-01

    This paper explicates the implications of my research on conflict management for self improvement and for practitioners who work to improve the conflict management of others. I also note how my experiences with practitioners have informed my research.

  7. the characteristics of a good teacher and how to become one

    African Journals Online (AJOL)

    MAL HAMZA

    techniques, commitment, and desire to make a difference ... eyes as well as their own. By asking ..... field. You should attend conferences, seminars, workshops, and induction courses for self-improvement. ... Curriculum and Instruction for Near.

  8. Advances in understanding the genetic basis of inherited single gene skin barrier disorders: new clues to key genes that may be involved in the pathogenesis of atopic dermatitis Avanços no entendimento da base genética de doenças hereditárias monogênicas da barreira epidérmica: novas pistas para os principais genes que podem estar envolvidos na patogênese da dermatite atópica

    Directory of Open Access Journals (Sweden)

    Joey E Lai-Cheong

    2006-12-01

    Full Text Available Increasing knowledge of genomic DNA sequences and genetic databases has led to the characterization of the molecular basis of several inherited skin disorders. In this review we summarize some of the major recent discoveries that have been made in defining the pathogenic mutations that cause inherited disorders of the skin barrier leading to skin scaling or increased transepidermal water loss in either rare disorders (Netherton’s syndrome or harlequin ichthyosis or more common genodermatoses (ichthyosis vulgaris. These molecular breakthroughs have led to more accurate diagnoses, better genetic counselling and, where appropriate, the feasibility of DNA-based prenatal diagnosis, as well as the possibility of developing newer forms of treatment, including gene or protein therapy. Identifying the molecular basis of these conditions, especially ichthyosis vulgaris, has also provided dramatic new insight into the genetic abnormalities in the common disorder, atopic dermatitis. Thus research on the relatively rare single gene inherited skin disorders not only has benefits for patients and their families with these uncommon conditions but also has the potential to yield fresh and significant new information about very common skin diseases.O maior conhecimento sobre as de sequências genômicas de DNA e as bases de dados genéticas levou à caracterização da base molecular de várias doenças hereditárias de pele. Nesta revisão resumimos algumas das descobertas recentes mais importantes quanto à definição das mutações patogênicas que causam as doenças hereditárias da barreira cutânea, levando a descamação ou aumento da perda hídrica transepidérmica, seja em doenças raras, (síndrome de Netherton ou ictiose em Arlequim ou genodermatoses mais comuns (ictiose vulgar. Estas descobertas moleculares têm conduzido a diagnósticos mais acurados, melhor aconselhamento genético e, quando apropriado, à possibilidade de diagnóstico pr

  9. Factors affecting dermatological manifestations in patients with end stage renal disease

    International Nuclear Information System (INIS)

    Anees, M.; Gull, S.; Nazeer, A.

    2018-01-01

    To determine skin changes in patients of End Stage Renal Disease (ESRD) on maintenance hemodialysis (MHD) and factors affecting these changes. Study Design:Cross-sectional observational study. Place and Duration of Study:Nephrology Department, Mayo Hospital, Lahore in collaboration with Dermatology Department, King Edward Medical University, Lahore, from October 2015 to January 2016. Methodology:Two hundred patients who were undergoing MHD for more than three months were included in the study. Patients' demographic data, laboratory reports and dialysis records were noted in a predesigned questionnaire. Skin examination was carried out by consultant dermatologist after patient's permission. Results:Among 200 patients included in study, 105 were males and rest of them were females. Major causes of ESRD were Diabetes Mellitus (n=83, 41.5%, followed by Hypertension (n=80, 40%), Nephrolithiasis (n=15, 7.5%) and Chronic glomerulonephritis (n=5, 2.5%). At least one cutaneous finding was present in every patient. Common skin findings observed were pigmentation (86%), xerosis (83%), pallor (79%), pruritus (69%), acquired ichthyosis (50.5%), and bacterial skin infections (18.5%). Among them, nail manifestations were half-and-half nails (52%), nychomycosis (30.5%), onycholysis (20.5%), subungual hyperkeratosis (23.5%), and Mee's lines (7.5). Among hair changes were sparse scalp hair (38.5%), brittle and lustreless hair (28%). The factors contributing to skin changes were patient's age, cause of ESRD, anti HCV positivity, high urea and creatinine levels, duration and frequency of hemodialysis, hemoglobin levels, calcium phosphate product and socioeconomic status. Some skin manifestations were interrelated with each other like xerosis with pruritus (p<0.001), pruritus with bacterial infection (p<0.022), acquired Ichthyosis (p=0.008) and hair changes (p=0.035). Conclusion:ESRD patients on hemodialysis develop various skin changes during the course of disease

  10. Oil refinery dusts: morphological and size analysis by TEM.

    Science.gov (United States)

    Sielicki, Przemysław; Janik, Helena; Guzman, Agnieszka; Broniszewski, Mieczysław; Namieśnik, Jacek

    2011-03-01

    The objectives of this work were to develop a means of sampling atmospheric dusts on the premises of an oil refinery for electron microscopic study to carry out preliminary morphological analyses and to compare these dusts with those collected at sites beyond the refinery limits. Carbon and collodion membranes were used as a support for collection of dust particles straight on transmission electron microscopy (TEM) grids. Micrographs of the dust particles were taken at magnifications from ×4,000 to ×80,000 with a Tesla BS500 transmission electron microscope. Four parameters were defined on the basis of the micrographs: surface area, Feret diameter, circumference, and shape coefficient. The micrographs and literature data were used to classify the atmospheric dusts into six groups: particles with an irregular shape and rounded edges; particles with an irregular shape and sharp edges; soot and its aggregates; spherical particles; singly occurring, ultrafine dust particles; and particles not allocated to any of the previous five groups. The types of dusts found in all the samples were similar, although differences did exist between the various morphological parameters. Dust particles with the largest Feret diameter were present in sample 3 (mean, 0.739 μm)-these were collected near the refinery's effluent treatment plant. The particles with the smallest diameter were found in the sample that had been intended to be a reference sample for the remaining results (mean, 0.326 μm). The dust particles collected in the refinery had larger mean Feret diameters, even 100% larger, than those collected beyond it. Particles with diameters from 0.1 to 0.2 μm made up the most numerous group in all the samples collected in the refinery.

  11. Are Polyatomic Interferences, Cross Contamination, Mixing-Effect, etc., Obstacles for the Use of Laser Ablation-ICP-MS Coupling as an Operational Technique for Uranium Isotope Ratio Particle Analysis?

    International Nuclear Information System (INIS)

    Donard, A.; Pointurier, F.; Pecheyran, C.

    2015-01-01

    Analysis of ''environmental samples'', which consists in dust collected with cotton clothes wiped by inspectors on surfaces inside declared nuclear facilities, is a key tool for safeguards. Although two methods (fission tracks-TIMS and SIMS) are already used routinely to determine the isotopic composition of uranium particles, the laser ablationinductively coupled plasma mass spectrometry (LA-ICP-MS) coupling has been proven to be an interesting option thanks to its rapidity, high sensitivity and high signal/noise ratio. At CEA and UPPA, feasibility of particle analysis using a nanosecond LA device and a quadrupole ICP-MS has been demonstrated. However, despite the obvious potential of LA-ICP-MS for particle analysis, the effect of many phenomena which may bias isotope ratio measurements or lead to false detections must be investigated. Actually, environmental samples contain many types of non-uranium particles (organic debris, iron oxides, etc.) that can form molecular interferences and induce the risk of isotopic measurement bias, especially for minor isotopes (234U, 236U). The influence of these polyatomic interferences on the measurements will be discussed. Moreover, different uranium isotopic compositions can be found in the same sample. Therefore, risks of memory effect and of particle-toparticle cross-contamination by the deposition of ablation debris around the crater have also been investigated. This study has been conducted by using a femtosecond laser ablation device coupled to a high sensitivity sector field ICP-MS. Particles were fixed onto the discs with collodion and were located thanks to their fission tracks so that micrometric particles can be analyzed separately. All uranium isotope ratios were measured. Results are compared with the ones obtained with the fission tracks-TIMS technique on other deposition discs from the same sample. Performance of the method in terms of accuracy, precision, and detection limits are estimated

  12. Hemodynamic Response to Interictal Epileptiform Discharges Addressed by Personalized EEG-fNIRS Recordings

    Science.gov (United States)

    Pellegrino, Giovanni; Machado, Alexis; von Ellenrieder, Nicolas; Watanabe, Satsuki; Hall, Jeffery A.; Lina, Jean-Marc; Kobayashi, Eliane; Grova, Christophe

    2016-01-01

    Objective: We aimed at studying the hemodynamic response (HR) to Interictal Epileptic Discharges (IEDs) using patient-specific and prolonged simultaneous ElectroEncephaloGraphy (EEG) and functional Near InfraRed Spectroscopy (fNIRS) recordings. Methods: The epileptic generator was localized using Magnetoencephalography source imaging. fNIRS montage was tailored for each patient, using an algorithm to optimize the sensitivity to the epileptic generator. Optodes were glued using collodion to achieve prolonged acquisition with high quality signal. fNIRS data analysis was handled with no a priori constraint on HR time course, averaging fNIRS signals to similar IEDs. Cluster-permutation analysis was performed on 3D reconstructed fNIRS data to identify significant spatio-temporal HR clusters. Standard (GLM with fixed HRF) and cluster-permutation EEG-fMRI analyses were performed for comparison purposes. Results: fNIRS detected HR to IEDs for 8/9 patients. It mainly consisted oxy-hemoglobin increases (seven patients), followed by oxy-hemoglobin decreases (six patients). HR was lateralized in six patients and lasted from 8.5 to 30 s. Standard EEG-fMRI analysis detected an HR in 4/9 patients (4/9 without enough IEDs, 1/9 unreliable result). The cluster-permutation EEG-fMRI analysis restricted to the region investigated by fNIRS showed additional strong and non-canonical BOLD responses starting earlier than the IEDs and lasting up to 30 s. Conclusions: (i) EEG-fNIRS is suitable to detect the HR to IEDs and can outperform EEG-fMRI because of prolonged recordings and greater chance to detect IEDs; (ii) cluster-permutation analysis unveils additional HR features underestimated when imposing a canonical HR function (iii) the HR is often bilateral and lasts up to 30 s. PMID:27047325

  13. Hemodynamic response to Interictal Epileptiform Discharges addressed by personalized EEG-fNIRS recordings

    Directory of Open Access Journals (Sweden)

    Giovanni ePellegrino

    2016-03-01

    Full Text Available Objective: We aimed at studying the hemodynamic response (HR to Interictal Epileptic Discharges (IEDs using patient-specific and prolonged simultaneous ElectroEncephaloGraphy (EEG and functional Near InfraRed Spectroscopy (fNIRS recordings. Methods: The epileptic generator was localized using Magnetoencephalography source imaging. fNIRS montage was tailored for each patient, using an algorithm to optimize the sensitivity to the epileptic generator. Optodes were glued using collodion to achieve prolonged acquisition with high quality signal. fNIRS data analysis was handled with no a priori constraint on HR time course, averaging fNIRS signals to similar IEDs. Cluster-permutation analysis was performed on 3D reconstructed fNIRS data to identify significant spatio-temporal HR clusters. Standard (GLM with fixed HRF and cluster-permutation EEG-fMRI analyses were performed for comparison purposes. Results: fNIRS detected HR to IEDs for 8/9 patients. It mainly consisted oxy-hemoglobin increases (7 patients, followed by oxy-hemoglobin decreases (6 patients. HR was lateralized in 6 patients and lasted from 8.5 to 30s. Standard EEG-fMRI analysis detected an HR in 4/9 patients (4/9 without enough IEDs, 1/9 unreliable result. The cluster-permutation EEG-fMRI analysis restricted to the region investigated by fNIRS showed additional strong and non-canonical BOLD responses starting earlier than the IEDs and lasting up to 30s. Conclusions: i EEG-fNIRS is suitable to detect the HR to IEDs and can outperform EEG-fMRI because of prolonged recordings and greater chance to detect IEDs; ii cluster-permutation analysis unveils additional HR features underestimated when imposing a canonical HR function iii the HR is often bilateral and lasts up to 30s.

  14. Noninvasive scalp recording of cortical auditory evoked potentials in the alert macaque monkey.

    Science.gov (United States)

    Itoh, Kosuke; Nejime, Masafumi; Konoike, Naho; Nakada, Tsutomu; Nakamura, Katsuki

    2015-09-01

    Scalp-recorded evoked potentials (EP) provide researchers and clinicians with irreplaceable means for recording stimulus-related neural activities in the human brain, due to its high temporal resolution, handiness, and, perhaps more importantly, non-invasiveness. This work recorded the scalp cortical auditory EP (CAEP) in unanesthetized monkeys by using methods that are essentially identical to those applied to humans. Young adult rhesus monkeys (Macaca mulatta, 5-7 years old) were seated in a monkey chair, and their head movements were partially restricted by polystyrene blocks and tension poles placed around their head. Individual electrodes were fixated on their scalp using collodion according to the 10-20 system. Pure tone stimuli were presented while electroencephalograms were recorded from up to nineteen channels, including an electrooculogram channel. In all monkeys (n = 3), the recorded CAEP comprised a series of positive and negative deflections, labeled here as macaque P1 (mP1), macaque N1 (mN1), macaque P2 (mP2), and macaque N2 (mN2), and these transient responses to sound onset were followed by a sustained potential that continued for the duration of the sound, labeled the macaque sustained potential (mSP). mP1, mN2 and mSP were the prominent responses, and they had maximal amplitudes over frontal/central midline electrode sites, consistent with generators in auditory cortices. The study represents the first noninvasive scalp recording of CAEP in alert rhesus monkeys, to our knowledge. Copyright © 2015 Elsevier B.V. All rights reserved.

  15. Lewis M. Rutherfurd and the First Photograph of Solar Granulation

    Science.gov (United States)

    Harvey, J. W.; Briggs, John W.; Prosser, Sian

    2017-08-01

    A major astronomical controversy of the mid-19th century was discordant descriptions of the small scale structure of the solar surface. Visual observers contradicted each other by describing the surface as consisting of “corrugations”, “willow leaves”, “rice grains”, “cumuli”, “thatch”, “granules”, etc. Early photographs of the solar surface were not good enough to settle the controversy. The French astronomer Jules Janssen is credited with the first 1876 photographs that clearly showed what we now call solar granulation (1876, CRAS 82, 1363). Upon seeing these images, New Yorker Lewis M. Rutherfurd (1878, MNRAS 38, 410) praised the high quality of Janssen’s images but asserted that he had also photographed granulation as early as 1871 using collodion wet plates. He sent copies of his best photograph to the Royal Astronomical Society to support his assertion. Curious about his claim, Briggs and Harvey set up Rutherfurd’s 13-inch achromatic refractor on Kitt Peak and found that it easily showed well-resolved solar granulation, so his claim might well have been justified. But without his plates we could not confirm the claim. For 140 years the copies of Rutherfurd’s best solar photograph remained in the archives of the Royal Astronomical Society and were recently discovered by Prosser (RAS Photographs A3/001B and A3/002). By coincidence a few days later, Briggs found the original August 11, 1871 plate. Despite poor condition these photographs show solar granulation. There are at least two other possible early claimants (Reade; Vogel) but their plates are almost certainly lost. Rutherfurd was a master of astronomical instrumentation and photography. He was reticent about his work, letting results speak for themselves, so it is satisfying to find that he was justified in making his claim of priority.

  16. Oil refinery dusts: morphological and size analysis by TEM

    Energy Technology Data Exchange (ETDEWEB)

    Sielicki, Przemyslaw; Janik, Helena; Guzman, Agnieszka [Gdansk University of Technology, Department of Polymer Technology, Chemical Faculty, Gdansk (Poland); Broniszewski, Mieczyslaw [Environmental Protection Office, Lotos Group, Gdansk (Poland); Namiesnik, Jacek [Gdansk University of Technology, Department of Analytical Chemistry, Chemical Faculty, Gdansk (Poland)

    2011-03-15

    The objectives of this work were to develop a means of sampling atmospheric dusts on the premises of an oil refinery for electron microscopic study to carry out preliminary morphological analyses and to compare these dusts with those collected at sites beyond the refinery limits. Carbon and collodion membranes were used as a support for collection of dust particles straight on transmission electron microscopy (TEM) grids. Micrographs of the dust particles were taken at magnifications from x 4,000 to x 80,000 with a Tesla BS500 transmission electron microscope. Four parameters were defined on the basis of the micrographs: surface area, Feret diameter, circumference, and shape coefficient. The micrographs and literature data were used to classify the atmospheric dusts into six groups: particles with an irregular shape and rounded edges; particles with an irregular shape and sharp edges; soot and its aggregates; spherical particles; singly occurring, ultrafine dust particles; and particles not allocated to any of the previous five groups. The types of dusts found in all the samples were similar, although differences did exist between the various morphological parameters. Dust particles with the largest Feret diameter were present in sample 3 (mean, 0.739 {mu}m) - these were collected near the refinery's effluent treatment plant. The particles with the smallest diameter were found in the sample that had been intended to be a reference sample for the remaining results (mean, 0.326 {mu}m). The dust particles collected in the refinery had larger mean Feret diameters, even 100% larger, than those collected beyond it. Particles with diameters from 0.1 to 0.2 {mu}m made up the most numerous group in all the samples collected in the refinery. (orig.)

  17. Molecular studies of deletions at the human steroid sulfatase locus

    International Nuclear Information System (INIS)

    Shapiro, L.J.; Yen, P.; Pomerantz, D.; Martin, E.; Rolewic, L.; Mohandas, T.

    1989-01-01

    The human steroid sulfatase gene (STS) is located on the distal X chromosome short arm close to the pseudoautosomal region but in a segment of DNA that is unique to the X chromosome. In contrast to most X chromosome-encoded genes, STS expression is not extinguished during the process of X chromosome inactivation. Deficiency of STS activity produced the syndrome of X chromosome-linked ichthyosis, which is one of the most common inborn errors of metabolism in man. Approximately 90% of STS - individuals have large deletions at the STS locus. The authors and others have found that the end points of such deletions are heterogeneous in their location. One recently ascertained subject was observed to have a 40-kilobase deletion that is entirely intragenic, permitting the cloning and sequencing of the deletion junction. Studies of this patient and of other X chromosome sequences in other subjects permit some insight into the mechanism(s) responsible for generating frequent deletions on the short arm of the X chromosome

  18. Gaucher disease in sheep.

    Science.gov (United States)

    Karageorgos, Litsa; Lancaster, Malcolm J; Nimmo, Judith S; Hopwood, John J

    2011-02-01

    Gaucher disease, an autosomal recessive lysosomal storage disorder caused by mutations in the β-glucocerebrosidase gene, was recently discovered in sheep on a "Southdown" sheep stud in Victoria, Australia. Clinical signs include neuropathy, thickened leathery skin, and ichthyosis, with lambs unable to stand from birth. Affected lambs were found to be deficient in glucocerebrosidase activity, and mutational analysis found them to be homozygous for the missense mutations c.1142G>A (p.C381Y) and c.1400C>T (p.P467L). In addition, four silent mutations were detected (c.777C>A [p.Y259Y], c1203A>G [p.Q401Q], c.1335T>C [p.I445I], c.1464C>G [p.L488L]). The human equivalent [C342Y] to the C381Y mutation leads to an acute neuronopathic phenotype in patients. Identification of an acute neuronopathic form of Gaucher disease in sheep provides a large animal model that will enable studies of pathology and evaluation of therapies to treat this common lysosomal storage disorder.

  19. Severe X-linked chondrodysplasia punctata in nine new female fetuses.

    Science.gov (United States)

    Lefebvre, Mathilde; Dufernez, Fabienne; Bruel, Ange-Line; Gonzales, Marie; Aral, Bernard; Saint-Onge, Judith; Gigot, Nadège; Desir, Julie; Daelemans, Caroline; Jossic, Frédérique; Schmitt, Sébastien; Mangione, Raphaele; Pelluard, Fanny; Vincent-Delorme, Catherine; Labaune, Jean-Marc; Bigi, Nicole; D'Olne, Dominique; Delezoide, Anne-Lise; Toutain, Annick; Blesson, Sophie; Cormier-Daire, Valérie; Thevenon, Julien; El Chehadeh, Salima; Masurel-Paulet, Alice; Joyé, Nicole; Vibert-Guigue, Claude; Rigonnot, Luc; Rousseau, Thierry; Vabres, Pierre; Hervé, Philippe; Lamazière, Antonin; Rivière, Jean-Baptiste; Faivre, Laurence; Laurent, Nicole; Thauvin-Robinet, Christel

    2015-07-01

    Conradi-Hünermann-Happle [X-linked dominant chondrodysplasia punctata 2 (CDPX2)] syndrome is a rare X-linked dominant skeletal dysplasia usually lethal in men while affected women show wide clinical heterogeneity. Different EBP mutations have been reported. Severe female cases have rarely been reported, with only six antenatal presentations. To better characterize the phenotype in female fetuses, we included nine antenatally diagnosed cases of women with EBP mutations. All cases were de novo except for two fetuses with an affected mother and one case of germinal mosaicism. The mean age at diagnosis was 22 weeks of gestation. The ultrasound features mainly included bone abnormalities: shortening (8/9 cases) and bowing of the long bones (5/9), punctuate epiphysis (7/9) and an irregular aspect of the spine (5/9). Postnatal X-rays and examination showed ichthyosis (8/9) and epiphyseal stippling (9/9), with frequent asymmetric short and bowed long bones. The X-inactivation pattern of the familial case revealed skewed X-inactivation in the mildly symptomatic mother and random X-inactivation in the severe fetal case. Differently affected skin samples of the same fetus revealed different patterns of X-inactivation. Prenatal detection of asymmetric shortening and bowing of the long bones and cartilage stippling should raise the possibility of CPDX2 in female fetuses, especially because the majority of such cases involve de novo mutations. © 2015 John Wiley & Sons, Ltd.

  20. Você conhece esta síndrome? Do you know this syndrome?

    Directory of Open Access Journals (Sweden)

    Francisco Paschoal

    2011-08-01

    Full Text Available A síndrome de KID é uma displasia ectodérmica congênita rara que afeta a pele, o epitélio da córnea e o ouvido interno. Clinicamente, observam-se placas de eritroqueratodermia na face e pregas, geralmente presentes desde o nascimento, a surdez neurossensorial severa e bilateral, e a vascularização córnea associado à queratite de evolução progressiva à qual surge após as alterações cutâneas e auditivas na puberdade. Face ao quadro surdez, às infecções cutâneas, ao risco de cegueira e à degeneração maligna, o diagnóstico precoce da síndrome é fundamental, bem como o seguimento clínico periódico e o aconselhamento genéticoKeratitis-ichthyosis-deafness (KID syndrome is a rare congenital ectodermal dysplasia affecting skin, the corneal epithelium and inner ear. Clinical signs consist of erythrokeratodermal plaques on the face and skin folds, usually present from birth, as well as severe and bilateral sensorineural hearing loss and corneal vascularization associated with slow-progressing keratitis which follows skin and hearing changes at puberty. In view of symptoms of deafness, blindness, skin infections and the risk of malignant degeneration, early diagnosis of the syndrome is essential, together with clinical follow-up and genetic counseling

  1. Keratitis–ichthyosis–deafness syndrome: first affected family reported in the Middle East

    Directory of Open Access Journals (Sweden)

    Al Fahaad H

    2014-03-01

    Full Text Available Hamad Al FahaadDepartment of Dermatology, College of Medicine, Najran University, Najran, Saudi ArabiaIntroduction: Keratitis–ichthyosis–deafness (KID syndrome is a rare congenital multisystem disorder affecting certain tissues of ectodermal origin such as epidermis, cochlea, and cornea, leading mainly to palmoplantar hyperkeratosis, ichthyosiform scaling, deafness, and blindness. The author reports for the first time in the Middle East three family members suffering from KID syndrome in the southwestern part of Saudi Arabia.Case presentation: Three patients from one family (ages 26, 16, and 14 years of apparently normal parents, with the two eldest being females and the youngest being male. All three patients were referred from a peripheral hospital to our dermatology clinic due to recurrent cutaneous fungal infections on their trunk, forearms, legs, and nails. On full assessment, they also found to have nearly similar cutaneous problems manifested by palmoplantar hyperkeratosis, generalized ichthyosiform scaling, subungual hyperkeratosis, and nail dystrophies. All patients suffered from total hearing loss in both ears since childhood as confirmed by pure tune audiometry. However, there was no blindness in any case; blepharitis with marked photophobia was the only ocular complaint. All these features are classically suggestive of KID syndrome.Keywords: connexin 26, GJB2, ichthyosis, KID syndrome, palmoplantar hyperkeratosis

  2. Total skin electron therapy as treatment for epitheliotropic lymphoma in a dog.

    Science.gov (United States)

    Santoro, Domenico; Kubicek, Lyndsay; Lu, Bo; Craft, William; Conway, Julia

    2017-04-01

    Mycosis fungoides (MF) is an uncommon cutaneous neoplasm in dogs. Treatment options are limited. Total skin electron therapy (TSET) has been suggested as a possible therapy for canine MF. To describe the use of TSET as palliative treatment for MF in a dog. An adult dog, previously diagnosed with nonepidermolytic ichthyosis, was presented with generalized erythroderma, alopecia and erosions. Histopathology revealed a densely cellular, well-demarcated, unencapsulated infiltrate extending from the epidermis to the mid-dermis compatible with MF. The infiltrate exhibited epitheliotropism multifocally for the epidermis, infundibula and adnexa. Due to a lack of response to chemotherapy, TSET was elected. Six megavoltage electrons were delivered using a 21EX Varian linear accelerator. A dose of 6 Gy was delivered to the skin surface and a 100 cm skin to surface distance was used for dog setup. The treatment time for the cranial half treatment was 3 h. The treatment was divided in two sessions (cranial and caudal halves of the body) 15 days apart. Clinical and histopathological complete remission was achieved and the dog was kept in remission with no additional treatments for 19 months before relapse and development of Sézary syndrome. To the best of the authors' knowledge, this is the first case reporting the use of TSET for medically refractory canine MF with post treatment follow-up. This case suggests that the use of TSET may be an effective palliative treatment for canine MF. © 2017 ESVD and ACVD.

  3. Muscle MRI in neutral lipid storage disease (NLSD).

    Science.gov (United States)

    Garibaldi, Matteo; Tasca, Giorgio; Diaz-Manera, Jordi; Ottaviani, Pierfancesco; Laschena, Francesco; Pantoli, Donatella; Gerevini, Simonetta; Fiorillo, Chiara; Maggi, Lorenzo; Tasca, Elisabetta; D'Amico, Adele; Musumeci, Olimpia; Toscano, Antonio; Bruno, Claudio; Massa, Roberto; Angelini, Corrado; Bertini, Enrico; Antonini, Giovanni; Pennisi, Elena Maria

    2017-07-01

    Neutral lipid storage disease (NLSD) is a rare inherited disorder of lipid metabolism resulting in lipid droplets accumulation in different tissues. Skeletal muscle could be affected in both two different form of disease: NLSD with myopathy (NLSD-M) and NLSD with ichthyosis (NLSD-I). We present the muscle imaging data of 12 patients from the Italian Network for NLSD: ten patients presenting NLSD-M and two patients with NLSD-I. In NLSD-M gluteus minimus, semimembranosus, soleus and gastrocnemius medialis in the lower limbs and infraspinatus in the upper limbs were the most affected muscles. Gracilis, sartorius, subscapularis, pectoralis, triceps brachii and sternocleidomastoid were spared. Muscle involvement was not homogenous and characteristic "patchy" replacement was observed in at least one muscle in all the patients. Half of the patients showed one or more STIR positive muscles. In both NLSD-I cases muscle involvement was not observed by T1-TSE sequences, but one of them showed positive STIR images in more than one muscle in the leg. Our data provides evidence that muscle imaging can identify characteristic alterations in NLSD-M, characterized by a specific pattern of muscle involvement with "patchy" areas of fatty replacement. Larger cohorts are needed to assess if a distinct pattern of muscle involvement exists also for NLSD-I.

  4. Imaging mass spectrometry visualizes ceramides and the pathogenesis of dorfman-chanarin syndrome due to ceramide metabolic abnormality in the skin.

    Directory of Open Access Journals (Sweden)

    Naoko Goto-Inoue

    Full Text Available Imaging mass spectrometry (IMS is a useful cutting edge technology used to investigate the distribution of biomolecules such as drugs and metabolites, as well as to identify molecular species in tissues and cells without labeling. To protect against excess water loss that is essential for survival in a terrestrial environment, mammalian skin possesses a competent permeability barrier in the stratum corneum (SC, the outermost layer of the epidermis. The key lipids constituting this barrier in the SC are the ceramides (Cers comprising of a heterogeneous molecular species. Alterations in Cer composition have been reported in several skin diseases that display abnormalities in the epidermal permeability barrier function. Not only the amounts of different Cers, but also their localizations are critical for the barrier function. We have employed our new imaging system, capable of high-lateral-resolution IMS with an atmospheric-pressure ionization source, to directly visualize the distribution of Cers. Moreover, we show an ichthyotic disease pathogenesis due to abnormal Cer metabolism in Dorfman-Chanarin syndrome, a neutral lipid storage disorder with ichthyosis in human skin, demonstrating that IMS is a novel diagnostic approach for assessing lipid abnormalities in clinical setting, as well as for investigating physiological roles of lipids in cells/tissues.

  5. Novel mutation in Sjogren-Larsson syndrome is associated with divergent neurologic phenotypes.

    Science.gov (United States)

    Davis, Kathleen; Holden, Kenton R; S'Aulis, Dana; Amador, Claudia; Matheus, M Gisele; Rizzo, William B

    2013-10-01

    Sjögren-Larsson syndrome is an inherited disorder of lipid metabolism caused by mutations in the ALDH3A2 gene that codes for fatty aldehyde dehydrogenase, which results in accumulation of fatty aldehydes and alcohols and is characterized by ichthyosis, intellectual disability, and spastic diplegia/quadriplegia. The authors describe 2 unrelated Honduran patients who carried the same novel homozygous nonsense mutation (c.1309A>T, p.K437X) and ALDH3A2 DNA haplotype, but widely differed in disease severity. One patient exhibited spastic quadriplegia with unusual neuroregression, whereas the other patient had the usual static form of spastic diplegia with neurodevelopmental disabilities. Biochemical analyses showed a similar profound deficiency of fatty aldehyde dehydrogenase activity and impaired fatty alcohol metabolism in both patients' cultured fibroblasts. These results indicate that variation in the neurologic phenotype of Sjögren-Larsson syndrome is not strictly determined by the ALDH3A2 mutation or the biochemical defect as expressed in cultured fibroblasts, but by unidentified epigenetic/environmental factors, gene modifiers, or other mechanisms.

  6. Karl Gustaf Torsten Sjögren and the Sjögren-Larsson syndrome

    Directory of Open Access Journals (Sweden)

    Khalid Al Aboud

    2011-09-01

    Full Text Available Karl Gustaf Torsten Sjögren (1896-1974 a Swedish psychiatrist and geneticist, was a pioneer of modern Swedish psychiatry. Sjögren studied medicine at the University of Uppsala. From 1932 to 1935, he was Head Physician and Director of Lillehagen Hospital in Gothenburg, and between 1935- 1945, he was physician-in-chief at the psychiatric department of Sahlgrenska Hospital in Gothenburg. Sjögren was professor of psychiatry at Karolinska Institutet from 1945 to 1961 and was elected to the Royal Swedish Academy of Sciences in 1951. Sjögren was an expert of psychiatry for the World Health Organization. Among his many contributions to medicine, he is credited for describing several medical conditions, which were later named after him, including Graefe- Sjögren syndrome, Marinesco-Sjögren syndrome, and Sjögren-Larsson syndrome (SLS. During his work on juvenile amaurotic idiocy, Sjögren forged a collaboration with Tage K.L. Larsson, a statistics lecturer at the University of Lund. Their study on the combination of oligophrenia, congenital ichthyosis, and spastic disorders in 1957 established the clinical and genetic profile of a new disease entity, later known as Sjogren-Larsson syndrome (SLS.

  7. Heredopatia atática polineuritiforme Heredopathia atactica polyieuritiformis

    Directory of Open Access Journals (Sweden)

    A. Spina-França

    1968-06-01

    Full Text Available Registro de caso de paciente com quadro mórbido relacionável ao da heredopatia atática polineuritiforme (doença de Refsum. O diagnóstico é discutido frente aos aspectos clínicos, laboratoriais e histopatológicos encontrados e sua relação com os achados referidos na literatura como próprios a essa moléstia.The case of a patient with many of the clinical characteristics commonly referred to in Refsum's disease is reported. Ichthyosis was noted in the patient and in other members of the family. Cerebrospinal fluid proteins were normal. Serum copper and caeruloplasmin concentrations were hight, as well as transaminases. Histological findings common to neuritis interstitialis hypaertrophica were found on examination of a peripheric nerve segment obtained by biopsy. Studies on the occurrence of phytanic acid could not be carried out. The relationship between the case reported and Refsum's syndrome is discussed.

  8. Lamellar granule secretion starts before the establishment of tight junction barrier for paracellular tracers in mammalian epidermis.

    Directory of Open Access Journals (Sweden)

    Akemi Ishida-Yamamoto

    Full Text Available Defects in epidermal barrier function and/or vesicular transport underlie severe skin diseases including ichthyosis and atopic dermatitis. Tight junctions (TJs form a single layered network in simple epithelia. TJs are important for both barrier functions and vesicular transport. Epidermis is stratified epithelia and lamellar granules (LGs are secreted from the stratum granulosum (SG in a sequential manner. Previously, continuous TJs and paracellular permeability barriers were found in the second layer (SG2 of SG in mice, but their fate and correlation with LG secretion have been poorly understood. We studied epidermal TJ-related structures in humans and in mice and found occludin/ZO-1 immunoreactive multilayered networks spanning the first layer of SG (SG1 and SG2. Paracellular penetration tracer passed through some TJs in SG2, but not in SG1. LG secretion into the paracellular tracer positive spaces started below the level of TJs of SG1. Our study suggests that LG-secretion starts before the establishment of TJ barrier in the mammalian epidermis.

  9. Comèl-Netherton syndrome – case report

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    Izabela Błażewicz

    2014-11-01

    Full Text Available Introduction. Comèl-Netherton syndrome is a rare autosomal recessive genodermatosis characterized by congenital ichthyosiform erythroderma, ichthyosis linearis circumflexa, trichorrhexis invaginata, and atopic diathesis. Comèl-Netherton syndrome is caused by mutations of the SPINK5 gene, which encodes the serine protease inhibitor LEKTI. Objective. We present diagnostic and therapeutic difficulties in a patient with Comèl-Netherton syndrome. Case report. We present the case of a 3-year-old boy, in whom from the first day of life generalized ichthyosiform erythroderma and diffuse exfoliation of the skin were observed. The differential diagnosis included Omenn syndrome, and atopic and seborrheic dermatitis. Finally, based on the overall clinical picture and microscopic examination of the hair, which showed the presence of bamboo hair, Comèl-Netherton syndrome was diagnosed. Conclusions . Because of similarity to other erythroderma, diagnosis of Comèl-Netherton syndrome in the first months of life creates diagnostic problems. In older children, recurrent skin infections and atopic diathesis can cause therapeutic difficulties and require the cooperation of many specialists.

  10. Ichthyosiform mycosis fungoides with alopecia and atypical membranous nephropathy

    Directory of Open Access Journals (Sweden)

    Qiang Zhou

    2011-01-01

    Full Text Available We describe here a rare case of variant of mycosis fungoides (MF: ichthyosiform MF with alopecia and atypical membranous nephropathy. The diagnosis was made based on the following findings: generalized ichthyosis-like eruption, alopecia, enlarged superficial lymph nodes, proteinuria, and hematuria, the histological features of the skin biopsy from both ichthyotic and alopecic lesions with immunohistochemical staining, and the renal biopsy examination with immunofluorescence. The histological examination of ichthyotic and alopecic lesions displayed a predominant infiltration of atypical lymphocytes in the upper dermis with the characteristics of epidermotropism and folliculotropism. Immunohistochemical studies demonstrated that most infiltrated atypical lymphocytes were CD3, CD4, and CD45RO positive, whereas negative for CD5, CD7, CD20, CD30, and CD56. A renal biopsy examination revealed atypical membranous nephropathy with deposition of immunoglobulin G (IgG, IgM, IgA, C1q, and C3. In this case atypical membranous nephropathy was involved, which is very uncommon and has never been presented in the literature to date. Although ichthyosiform MF usually features a relatively favorable course, diffuse alopecia and the renal involvement in this case might indicate aggressive disease and poor prognosis.

  11. Skin changes in internal malignancy

    Directory of Open Access Journals (Sweden)

    Rajagopal Ravi

    2004-07-01

    Full Text Available BACKGROUND: Internal malignancies are accompanied by various skin changes which may be specific infiltrates or non-specific changes. This study is aimed at determining the frequency of such changes in malignant disease treatment center attendees in India. METHODS: A study of 300 confirmed cases of internal malignancy at a malignant disease treatment center was undertaken to evaluate these skin changes. Specific infiltrates were confirmed by histopathology. Statistical methods were employed to calculate significance in non-specific lesions by comparing with 300 controls not suffering from internal malignancy. RESULTS: Skin changes were present in 82 (27.3%. Cutaneous metastases were found in 19 (6.3%; non-contiguous in 5 (1.6%; contiguous in 14 (4.3%. Non-specific skin lesions numbered 74 (11.6% in 52 patients. Statistically significant non-specific skin changes were acquired ichthyosis, herpes zoster and generalized pruritus. CONCLUSION: Metastases usually occurred late in internal malignancy (17, 5.6% except in a case each of histiocytic lymphoma and non-Hodgkin′s lymphoma (2, 0.7% where the lesions preceded malignancy by 3 months and 1 month respectively. Contiguous nodules were a marker of relapse after surgery in 3 (1%.

  12. Molecular evaluation of a novel missense mutation & an insertional truncating mutation in SUMF1 gene

    Directory of Open Access Journals (Sweden)

    Udhaya H Kotecha

    2014-01-01

    Full Text Available Background & objectives: Multiple suphphatase deficiency (MSD is an autosomal recessive disorder affecting the post translational activation of all enzymes of the sulphatase family. To date, approximately 30 different mutations have been identified in the causative gene, sulfatase modifying factor 1 (SUMF1. We describe here the mutation analysis of a case of MSD. Methods: The proband was a four year old boy with developmental delay followed by neuroregression. He had coarse facies, appendicular hypertonia, truncal ataxia and ichthyosis limited to both lower limbs. Radiographs showed dysostosis multiplex. Clinical suspicion of MSD was confirmed by enzyme analysis of four enzymes of the sulphatase group. Results: The patient was compound heterozygote for a c.451A>G (p.K151E substitution in exon 3 and a single base insertion mutation (c.690_691 InsT in exon 5 in the SUMF1 gene. The bioinformatic analysis of the missense mutation revealed no apparent effect on the overall structure. However, the mutated 151-amino acid residue was found to be adjacent to the substrate binding and the active site residues, thereby affecting the substrate binding and/or catalytic activity, resulting in almost complete loss of enzyme function. Conclusions: The two mutations identified in the present case were novel. This is perhaps the first report of an insertion mutation in SUMF1 causing premature truncation of the protein.

  13. Keratosis Pilaris Revisited: Is It More Than Just a Follicular Keratosis?

    Science.gov (United States)

    Thomas, Mary; Khopkar, Uday Sharadchandra

    2012-01-01

    Background: Keratosis pilaris (KP) is characterized by keratinous plugs in the follicular orifices and varying degrees of perifollicular erythema. The most accepted theory of its pathogenesis proposes defective keratinization of the follicular epithelium resulting in a keratotic infundibular plug. We decided to test this hypothesis by doing dermoscopy of patients diagnosed clinically as keratosis pilaris. Materials and Methods: Patients with a clinical diagnosis of KP seen between September 2011 and December 2011 were included in the study. A clinical history was obtained and examination and dermoscopic evaluation were performed on the lesions of KP. Results: The age of the patients ranged from 6-38 years. Sixteen patients had history of atopy. Nine had concomitant ichthyosis vulgaris. All the 25 patients were found to have coiled hair shafts within the affected follicular infundibula. The hair shafts were extracted with the help of a sterile needle and were found to retain their coiled nature. Perifollicular erythema was seen in 11 patients; perifollicular scaling in 9. Conclusion: Based on our observations and previously documented histological data of KP, we infer that KP may not be a disorder of keratinization, but caused by the circular hair shaft which ruptures the follicular epithelium leading to inflammation and abnormal follicular keratinization. PMID:23766609

  14. Sebaceous Gland, Hair Shaft, and Epidermal Barrier Abnormalities in Keratosis Pilaris with and without Filaggrin Deficiency

    Science.gov (United States)

    Gruber, Robert; Sugarman, Jeffrey L.; Crumrine, Debra; Hupe, Melanie; Mauro, Theodora M.; Mauldin, Elizabeth A.; Thyssen, Jacob P.; Brandner, Johanna M.; Hennies, Hans-Christian; Schmuth, Matthias; Elias, Peter M.

    2016-01-01

    Although keratosis pilaris (KP) is common, its etiopathogenesis remains unknown. KP is associated clinically with ichthyosis vulgaris and atopic dermatitis and molecular genetically with filaggrin-null mutations. In 20 KP patients and 20 matched controls, we assessed the filaggrin and claudin 1 genotypes, the phenotypes by dermatoscopy, and the morphology by light and transmission electron microscopy. Thirty-five percent of KP patients displayed filaggrin mutations, demonstrating that filaggrin mutations only partially account for the KP phenotype. Major histologic and dermatoscopic findings of KP were hyperkeratosis, hypergranulosis, mild T helper cell type 1-dominant lymphocytic inflammation, plugging of follicular orifices, striking absence of sebaceous glands, and hair shaft abnormalities in KP lesions but not in unaffected skin sites. Changes in barrier function and abnormal paracellular permeability were found in both interfollicular and follicular stratum corneum of lesional KP, which correlated ultrastructurally with impaired extracellular lamellar bilayer maturation and organization. All these features were independent of filaggrin genotype. Moreover, ultrastructure of corneodesmosomes and tight junctions appeared normal, immunohistochemistry for claudin 1 showed no reduction in protein amounts, and molecular analysis of claudin 1 was unremarkable. Our findings suggest that absence of sebaceous glands is an early step in KP pathogenesis, resulting in downstream hair shaft and epithelial barrier abnormalities. PMID:25660180

  15. Dermatologic Diseases in Silk Workers

    Directory of Open Access Journals (Sweden)

    J S Pasricha

    1985-01-01

    Full Text Available A survey of 112 workers of a silk facory near Bangalore, for dermatologic diseases revealed (1 a characteristic wearing off of the medial halves of the distal free edges of the finger nail plates in 10 of the 15 cocoonsorters, (2 maceration of the palms in 58 workers of the boiling and reeling section, and (3 pitted keratolysis of the palms, in 42 workers, also from the boiling and reeling section. There was no clinical evidence of contact dermatitis, and patch tests with the silk thread from the cocoons in 25 workers showed a very mild reaction in 2 workers and a doubtful reaction in another two. In addition, one worker from the skeining section had crisscross superficial fissures on the finger tips caused by friction, two workers had paronychia ′of the fingers and four workers had dermatophytFNx01t fingers webs. As in the previous survey, these workers also had a high incidence of ichthyosis (92 workers and hyperketatosis of the palms (62 workers and soles (110 workers.

  16. PNPLA1 Deficiency in Mice and Humans Leads to a Defect in the Synthesis of Omega-O-Acylceramides

    Science.gov (United States)

    Grond, Susanne; Eichmann, Thomas O.; Dubrac, Sandrine; Kolb, Dagmar; Schmuth, Matthias; Fischer, Judith; Crumrine, Debra; Elias, Peter M.; Haemmerle, Guenter; Zechner, Rudolf; Lass, Achim; Radner, Franz P.W.

    2017-01-01

    Mutations in PNPLA1 have been identified as causative for autosomal recessive congenital ichthyosis in humans and dogs. So far, the underlying molecular mechanisms are unknown. In this study, we generated and characterized PNPLA1-deficient mice and found that PNPLA1 is crucial for epidermal sphingolipid synthesis. The absence of functional PNPLA1 in mice impaired the formation of omega-O-acylceramides and led to an accumulation of nonesterified omega-hydroxy-ceramides. As a consequence, PNPLA1-deficient mice lacked a functional corneocyte-bound lipid envelope leading to a severe skin barrier defect and premature death of newborn animals. Functional analyses of differentiated keratinocytes from a patient with mutated PNPLA1 demonstrated an identical defect in omega-O-acylceramide synthesis in human cells, indicating that PNPLA1 function is conserved among mammals and indispensable for normal skin physiology. Notably, topical application of epidermal lipids from wild-type onto Pnpla1-mutant mice promoted rebuilding of the corneocyte-bound lipid envelope, indicating that supplementation of ichthyotic skin with omega-O-acylceramides might be a therapeutic approach for the treatment of skin symptoms in individuals affected by omega-O-acylceramide deficiency. PMID:27751867

  17. Trichothiodystrophy, a human DNA repair disorder with heterogeneity in the cellular response to ultraviolet light

    International Nuclear Information System (INIS)

    Lehmann, A.R.; Arlett, C.F.; Broughton, B.C.

    1988-01-01

    Trichothiodystrophy (TTD) is an autosomal recessive disorder characterized by brittle hair with reduced sulfur content, ichthyosis, peculiar face, and mental and physical retardation. Some patients are photosensitive. A previous study by Stefanini et al. showed that cells from four photosensitive patients with TTD had a molecular defect in DNA repair, which was not complemented by cells from xeroderma pigmentosum, complementation group D. In a detailed molecular and cellular study of the effects of UV light on cells cultured from three further TTD patients who did not exhibit photosensitivity we have found an array of different responses. In cells from the first patient, survival, excision repair, and DNA and RNA synthesis following UV irradiation were all normal, whereas in cells from the second patient all these responses were similar to those of excision-defective xeroderma pigmentosum (group D) cells. With the third patient, cell survival measured by colony-forming ability was normal following UV irradiation, even though repair synthesis was only 50% of normal and RNA synthesis was severely reduced. The excision-repair defect in these cells was not complemented by other TTD cell strains. These cellular characteristics of patient 3 have not been described previously for any other cell line. The normal survival may be attributed to the finding that the deficiency in excision-repair is confined to early times after irradiation. Our results pose a number of questions about the relationship between the molecular defect in DNA repair and the clinical symptoms of xeroderma pigmentosum and TTD

  18. al mena: a comprehensive resource of human genetic variants integrating genomes and exomes from Arab, Middle Eastern and North African populations.

    Science.gov (United States)

    Koshy, Remya; Ranawat, Anop; Scaria, Vinod

    2017-10-01

    Middle East and North Africa (MENA) encompass very unique populations, with a rich history and encompasses characteristic ethnic, linguistic and genetic diversity. The genetic diversity of MENA region has been largely unknown. The recent availability of whole-exome and whole-genome sequences from the region has made it possible to collect population-specific allele frequencies. The integration of data sets from this region would provide insights into the landscape of genetic variants in this region. We integrated genetic variants from multiple data sets systematically, available from this region to create a compendium of over 26 million genetic variations. The variants were systematically annotated and their allele frequencies in the data sets were computed and available as a web interface which enables quick query. As a proof of principle for application of the compendium for genetic epidemiology, we analyzed the allele frequencies for variants in transglutaminase 1 (TGM1) gene, associated with autosomal recessive lamellar ichthyosis. Our analysis revealed that the carrier frequency of selected variants differed widely with significant interethnic differences. To the best of our knowledge, al mena is the first and most comprehensive repertoire of genetic variations from the Arab, Middle Eastern and North African region. We hope al mena would accelerate Precision Medicine in the region.

  19. Case Report: Sjogren-Larsson Syndrome: Two Cases from One Family

    Directory of Open Access Journals (Sweden)

    Parvaneh Karim-Zadeh

    2006-04-01

    Full Text Available Sjogren–Larsson Syndrome (SLS is an autosomal recessive disorder characterized by generalized Ichthyosis, mental retardation, spastic diplegia or tetraplegia and epilepsy. This is a rare syndrome that caused by mutation in the ALDH3A2 gene, on chromosome 17p11.2. That encodes fatty aldehyde dehydrogenase (FAIDH, an enzyme that catalyzes the oxidation of medium – long chain aldehydes derived from lipid metabolism. Neuroimaging (MRI shows retardation of myelination and a mild myelin deficit. Proton Magnetic Resonance Spectroscopy (MRS shows the peak of lipids that accumulate because of fatty alchohols. We report two cases that they are siblings from relative parents. The Brother is 4 years old and his sister is 3 years old. , The clinical findings are developmental delay, mental retardation, spastic Tetraplegia and refractory seizure. The most important finding in these two siblings was generalized Icthyosis. MRI showed hyper signality in white matter and MRS showed the peak of accumulated lipids that confirmed the diagnosis of "Sjogren-Larsson Syndrome".

  20. Generalized peeling skin syndrome: Case report and review of the literature.

    Science.gov (United States)

    Kharfi, Monia; Khaled, Aida; Ammar, Donia; Ezzine, Nadia; El Fekih, Nadia; Fazaa, Becima; Jaafoura, Hiabib; Kamoun, Mohamed Ridha

    2010-03-15

    Peeling skin syndrome (PSS) is a rare form of ichthyosis with a probable autosomal recessive inheritance that exhibits superficial, painless, continual, or seasonal cutaneous exfoliation. The syndrome generally appears at birth or in infancy. We report a case of generalized PSS and provide a literature review. A 34-year-old woman reported a lifelong history of generalized and painless peeling of the skin that worsened in summer. Her parents were third degree cousins. Her twin sister and her two cousins presented with the same condition. Physical examination showed widespread superficial sheets of variable size that could be easily removed without bleeding or pain. No underlying erythema was noted. Otherwise, the patient was in good health. Histological findings showed an epidermal cleavage within the stratum corneum. The generalized form of PSS is classified into 3 types, A, B, and C, according to the classification system of Traupe and Mevorah. We have tried to classify the cases of generalized PSS already reported in the literature into one of these three types. Thirteen reported cases probably presented PSS-type A. Sixteen patients are best described as PSS-type B. Two patients exhibit PSS-type C. Fifteen reported patients had an acral form of peeling skin syndrome. The classification of the eleven remaining patients was difficult to determine. Our patient presented clinical and histological features of generalized PSS-type A.

  1. STUDY OF SPORTS TEACHERS STUDENTS' SKILLS FOR SELF-ASSESSMENT OF THEIR PROFESSIONAL QUALITIES

    Directory of Open Access Journals (Sweden)

    Tsonkova Dimitrinka Georgieva

    2013-12-01

    Full Text Available Аn essential characteristic of the teacher’s profession is the constant process of self-improvement, which is impossible without existence of a specific personal position and criteria of the sport pedagogue. One of the trends for self-improvement are his personal and professional qualities. They are important because of their specific role – as a means of influence on the trained students in the educational process. Self-evaluation of the level of their growth is a regulator for the sport teacher’s conduct and activity, because it determines the genuine orientation for the level of his qualities,the satisfaction or dissatisfaction.

  2. Report of a Therapeutic Drama Program in a Federal Prison: Implications for Applied Communication.

    Science.gov (United States)

    Mettee, Dorothy L.

    Noting that new approaches must be found to decrease the numbers of inmates returning to United States prisons and to provide means for productive changes that will result in self-improvement and new options for inmates both inside and outside the prison after their release, a drama program was established in 1979 for inmates at the Federal…

  3. The Organization and Structure of Community Education Offerings in Community Colleges

    Science.gov (United States)

    Miller, Michael; Grover, Kenda S.; Kacirek, Kit

    2014-01-01

    One of the key services community colleges provide is community education, meaning those programs and activities that are often offered for leisure or self-improvement and not for credit. Programs of this nature are increasingly challenged to be self-financing, whether through user fees or externally funded grants. The current study explored 75…

  4. The unity of laws, principles and consistent patterns of social education

    Directory of Open Access Journals (Sweden)

    Volodymyr Kostiv

    2017-03-01

    Full Text Available The article reveals the essence of social, educational and synergetic laws of basiccultural identity development, laws and principles of social education of young generation.Key words: law, pattern, principle, public law of basic cultural identity development,pedagogical law of integrative qualities formation in children and youth, synergetic law ofself-improvement, integrative personal traits self-improvement.

  5. Continuous Personal Improvement.

    Science.gov (United States)

    Emiliani, M. L.

    1998-01-01

    Suggests that continuous improvement tools used in the workplace can be applied to self-improvement. Explains the use of such techniques as one-piece flow, kanban, visual controls, and total productive maintenance. Points out misapplications of these tools and describes the use of fishbone diagrams to diagnose problems. (SK)

  6. Developmental Education Evaluation Model.

    Science.gov (United States)

    Perry-Miller, Mitzi; And Others

    A developmental education evaluation model designed to be used at a multi-unit urban community college is described. The purpose of the design was to determine the cost effectiveness/worth of programs in order to initiate self-improvement. A needs assessment was conducted by interviewing and taping the responses of students, faculty, staff, and…

  7. Why and how people engage in social comparison while learning social skills in groups.

    NARCIS (Netherlands)

    Buunk, Abraham (Bram); Cohen-Schotanus, J; Nek, R.H.

    This study was conducted among 269 medical students who participated in educational training groups. Self-evaluation was the most important motive to engage in social comparison with other group members, followed by, respectively, self-enhancement and self-improvement. Upward comparisons (i.e., with

  8. Integration of Educational and Sports Technologies in Youth Wellness Tourism

    Science.gov (United States)

    Malyshev, Andrei A.; Khodasevich, Leonid S.; Maznichenko, Marina A.; Romanov, Sergei M.

    2016-01-01

    The article reveals the potential of the youth tourism, addressing a number of problems of higher education: formation of a general cultural competence, preservation and strengthening of students' health, enhancing educational progress, motivating the physical self-improvement, contributing to the formation of healthy lifestyle values and…

  9. Enhancing the Educational Subject: Cognitive Capitalism, Positive Psychology and Well-Being Training in Schools

    Science.gov (United States)

    Reveley, James

    2013-01-01

    Positive psychology is influencing educational policy and practice in Britain and North America. This article reveals how this psychological discourse and its offshoot school-based training programs, which stress happiness, self-improvement and well-being, align with an emergent socio-economic formation: cognitive capitalism. Three key points are…

  10. How American and Chinese Journalism Students Prioritize Values: A Comparative Approach

    Science.gov (United States)

    Yang, Jin; Arant, David

    2016-01-01

    This study found that Chinese journalism students and American journalism students are more different than similar in their value systems. Overall, American students give greater weight to social-interaction values and self-improvement values, and Chinese students give greater importance to morality-oriented values and competency-oriented values.…

  11. Motives for Social Comparison.

    Science.gov (United States)

    Helgeson, Vicki S.; Mickelson, Kristin D.

    1995-01-01

    A set of motive statements for social comparison was elicited from one group of subjects and then rated in terms of usefulness by a second group of subjects. Analysis of these statements revealed six motives in response to two different hypothetical scenarios: self-evaluation, common bond, self-improvement, self-enhancement, altruism, and…

  12. Business Administration Scale for Family Child Care, BAS

    Science.gov (United States)

    Talan, Teri N.; Bloom, Paula Jorde

    2009-01-01

    The "BAS for Family Child Care" is the first valid and reliable tool for measuring and improving the overall quality of business and professional practices in family child care settings. It is applicable for multiple uses, including program self-improvement, technical assistance and monitoring, training, research and evaluation, and public…

  13. Challenging the Concept of "informal" urbanisation in African Cities

    DEFF Research Database (Denmark)

    Eskemose Andersen, Jørgen

    2013-01-01

    that are pushed out or unable to capitalise on the rising property values. This paper draws on in-depth studies of the peri-urban settlements of Maputo and demonstrates that the ‘informal’ process is producing self-improving urban form and housing. The paper refers to a concrete current project (Maxaquene A...

  14. Honesty in Critically Reflective Essays: An Analysis of Student Practice

    Science.gov (United States)

    Maloney, Stephen; Tai, Joanna Hong-Meng; Lo, Kristin; Molloy, Elizabeth; Ilic, Dragan

    2013-01-01

    In health professional education, reflective practice is seen as a potential means for self-improvement from everyday clinical encounters. This study aims to examine the level of student honesty in critical reflection, and barriers and facilitators for students engaging in honest reflection. Third year physiotherapy students, completing summative…

  15. Theorycrafting the Classroom: Constructing the Introductory Technical Communication Course as a Game

    Science.gov (United States)

    Finseth, Carly

    2015-01-01

    When games are approached as a pedagogical methodology, the homologies between games and technical communication are highlighted: pedagogy that teaches people to play and succeed within certain confines; classroom assessment that provides meaningful feedback to encourage self-improvement; instructional design that incorporates gaming theory and…

  16. Brainwaves and psyches : A genealogy of an extended self

    NARCIS (Netherlands)

    Brenninkmeijer, Jonna

    This article presents an ethnographical and historical analysis of the mode of being that is constituted when people use neurofeedback (brainwave training) for self-improvement. I analyse how human brainwaves have been associated with the psyche since their first demonstration by the psychiatrist

  17. Leisure resourcefulness as a predictor of level of affluence and life satisfaction: having more or doing with less

    Science.gov (United States)

    Jerry L. Ricciardo

    2010-01-01

    This research examines the role of affluence in guiding lifestyle orientation in contemporary society. The term "affluenza" is used to denote a lifestyle of consumption and materialism to achieve life satisfaction. The counter to affluenza is quality of life as manifested in self-improvement, community centeredness, and environmental stewardship. Leisure...

  18. AIMING AT THE TOP - UPWARD SOCIAL-COMPARISON OF ABILITIES AFTER FAILURE

    NARCIS (Netherlands)

    YBEMA, JF; BUUNK, BP

    1993-01-01

    This study examined the effects of feedback on a task on information seeking and partner preferences as forms of social comparison. It was predicted that subjects who experienced failure and perceived control over future performance would, for reasons of self-improvement, choose more strongly upward

  19. Disciplining the Conduct of Young People in Compulsory Education Policy and Practice

    Science.gov (United States)

    Hodgson, David

    2018-01-01

    Disciplining and pastoral power are central to the strategies and practices of intervening in the lives of young people deemed at risk of disengaging from school, or not completing their compulsory education. As an expression of power concerned with young people's welfare and self-improvement, disciplining and pastoral practices push young people…

  20. A Review on Reading-Supported Values Education of 7th Grade Students (Case of Kars Province, Turkey)

    Science.gov (United States)

    Karahan, Berna Ürün; Atalay, Tazegül Demir

    2017-01-01

    As is known, reading is a skill the individual faces throughout his/her life. Reading, which is also one of the four basic language skills, allows an individual to establish a connection with life. So that, self-improvement of the individual, the process of understanding and interpreting what is happening around him/her, expressing himself/herself…

  1. Leisure Worlds: Situations, Motivations and Young People's Encounters with Offending and Victimization

    Science.gov (United States)

    Tanner, Julian; Asbridge, Mark; Wortley, Scot

    2015-01-01

    With information supplied by a large (n = 3393) sample of high school students from Toronto, this paper tests the assumption that three forms of leisure activity--peer, risky, and self-improving leisure--have a relatively independent impact upon patterns of offending and victimization. Although we find significant support for this proposition, we…

  2. Heterogeneous Roles and Heterogeneous Practices: Understanding the Adoption and Uses of Nonprofit Performance Evaluations

    Science.gov (United States)

    Eckerd, Adam; Moulton, Stephanie

    2011-01-01

    Evaluating the performance of nonprofit organizations has been of growing importance for the last several decades. Although there is much good that can come out of self-improvement, there is substantial heterogeneity within the sector that calls into question the usefulness of "across the board" evaluation tools. In this article, the authors…

  3. The Cultural Context of Academic Motives: A Comparison of Filipino and American College Students.

    Science.gov (United States)

    Church, A. Timothy; Katigbak, Marcia S.

    1992-01-01

    Comparing self-reported academic motives of 409 male and 511 female Filipino college students and 407 male and 506 female (plus 12 unidentified) U.S. college students indicates that Filipinos rank approval and self-improvement higher and U.S. students rank motives involving performance standards higher. Many gender differences in motives…

  4. Benchmarking for Higher Education.

    Science.gov (United States)

    Jackson, Norman, Ed.; Lund, Helen, Ed.

    The chapters in this collection explore the concept of benchmarking as it is being used and developed in higher education (HE). Case studies and reviews show how universities in the United Kingdom are using benchmarking to aid in self-regulation and self-improvement. The chapters are: (1) "Introduction to Benchmarking" (Norman Jackson…

  5. Utilities get more involved in Inpo [Institute of Nuclear Power Operations

    International Nuclear Information System (INIS)

    Varley, J.

    1984-01-01

    The Institute of Nuclear Power Operations (INPO) was established four years ago in a bid to foster self improvement and self regulation among the American nuclear power utilities. A significant increase in utility commitment and involvement in many of its programmes appears to have made 1983 a very good year for INPO. (U.K.)

  6. Asian Educational Discourse: Construction of Ontological Security

    Science.gov (United States)

    Khalina, Natalya V.; Kovaleva, Alla V.; Voronin, Maksim S.; Anikin, Denis V.; Valyulina, Ekaterina V.

    2018-01-01

    This article considers the problem of ontology security through Asian educational discourse, which is structurally determined by the process of moral self-improvement. Considered are trends in improving the management of educational system by developing the culture of quality, which is considered as the next stage of the Asian education systems…

  7. Online Training and Practice Manual for ERIC Data Base Searchers. 2nd Edition.

    Science.gov (United States)

    Markey, Karen; Cochrane, Pauline A.

    Revised to reflect changes in both DIALOG and ERIC, this second edition of a self-improvement manual for online searchers who wish to refine their skills presents three basic approaches to searching and provides illustrations from DIALOG's ERIC ONTAP database. The manual is divided into two parts: an 8-step model of the total search process which…

  8. Organizational factors

    International Nuclear Information System (INIS)

    Holy, J.

    1999-12-01

    The following organizational factors are considered with respect to the human factor and operating safety of nuclear power plants: external influences; objectives and strategy; positions and ways of management; allocation of resources; working with human resources; operators' training; coordination of work; knowledge of organization and management; proceduralization of the topic; labour organizing culture; self-improvement system; and communication. (P.A.)

  9. Exploring the Effect of Student Confusion in Massive Open Online Courses

    Science.gov (United States)

    Yang, Diyi; Kraut, Robert E.; Rose, Carolyn P.

    2016-01-01

    Although thousands of students enroll in Massive Open Online Courses (MOOCs) for learning and self-improvement, many get confused, harming learning and increasing dropout rates. In this paper, we quantify these effects in two large MOOCs. We first describe how we automatically estimate students' confusion by looking at their clicking behavior on…

  10. Sub-Saharan Africa Report No. 2639.

    Science.gov (United States)

    1982-06-14

    square maters. The restoration work carried out by Soki Paint, the entrepreneur , cost CPA Fr 1,801,108. Under the heading of self-improvement, the CSC has...by the Consumer Price Index, last sonth rose 1,6 percent to give an annual increse of 16,5 percent-- the largest since 1920. Mw inceas for prtil won

  11. Science Teachers' Conceptualizations and Implications for the Development of the Professional Development Programs

    Science.gov (United States)

    Kaymakamoglu, Sibel Ersel

    2017-01-01

    This study aimed to investigate the two primary school science teachers' conceptions of professional development, their perceptions of self-improvement and the factors influencing their professional development. In this investigation, a case study approach was adopted. The participant teachers were given a semi-structured interview and the data…

  12. Executive Headteachers: What's in a Name? Executive Summary

    Science.gov (United States)

    Theobald, Katy; Lord, Pippa

    2016-01-01

    Executive headteachers (EHTs) are becoming increasingly prevalent as the self-improving school system matures; there are over 620 EHTs in the school workforce today; and the number recorded in the School Workforce Census (SWC) has increased by 240 per cent between 2010 and 2014. The role is still evolving locally and nationally and, as EHTs take…

  13. Using Policy-Capturing to Measure Attitudes in Organizational Diagnosis.

    Science.gov (United States)

    Madden, Joseph M.

    1981-01-01

    Discusses an indirect method of attitude measurement, policy-capturing, that can be applied on an individual basis. In three experiments this method detected prejudicial attitudes toward females not detected with traditional methods. Can be used as a self-improvement diagnostic tool for developing awareness of behavior influences. (JAC)

  14. Networking: Addressing Urban Students' Self-Esteem.

    Science.gov (United States)

    Tobias, Randolf; Turner, Thomas M.

    1997-01-01

    Describes Network in the Schools (NIS), a project to enhance teens' academic achievement and self-esteem, which uses small group classroom discussions regarding self-affirmation, social concerns, self-improvement, and reflection, and meetings for group sharing and self-expression. Presents findings that the program results in enhanced parent…

  15. Elemental and Isotopic Analysis of Uranium Oxide an NIST Glass Standards by FEMTOSECOND-LA-ICP-MIC-MS

    International Nuclear Information System (INIS)

    Ebert, Chris; Zamzow, Daniel S.; McBay, Eddie H.; Bostick, Debra A.; Bajic, Stanley J.; Baldwin, David P.; Houk, R.S.

    2009-01-01

    analyzed as particle aggregates immobilized in a collodion substrate. The uranium oxide samples were nuclear reference materials (CRMs U0002, U005-A, 129-A, U015, U030-A, and U050) obtained from New Brunswick Laboratory-USDOE.

  16. Neural processes underlying cultural differences in cognitive persistence.

    Science.gov (United States)

    Telzer, Eva H; Qu, Yang; Lin, Lynda C

    2017-08-01

    Self-improvement motivation, which occurs when individuals seek to improve upon their competence by gaining new knowledge and improving upon their skills, is critical for cognitive, social, and educational adjustment. While many studies have delineated the neural mechanisms supporting extrinsic motivation induced by monetary rewards, less work has examined the neural processes that support intrinsically motivated behaviors, such as self-improvement motivation. Because cultural groups traditionally vary in terms of their self-improvement motivation, we examined cultural differences in the behavioral and neural processes underlying motivated behaviors during cognitive persistence in the absence of extrinsic rewards. In Study 1, 71 American (47 females, M=19.68 years) and 68 Chinese (38 females, M=19.37 years) students completed a behavioral cognitive control task that required cognitive persistence across time. In Study 2, 14 American and 15 Chinese students completed the same cognitive persistence task during an fMRI scan. Across both studies, American students showed significant declines in cognitive performance across time, whereas Chinese participants demonstrated effective cognitive persistence. These behavioral effects were explained by cultural differences in self-improvement motivation and paralleled by increasing activation and functional coupling between the inferior frontal gyrus (IFG) and ventral striatum (VS) across the task among Chinese participants, neural activation and coupling that remained low in American participants. These findings suggest a potential neural mechanism by which the VS and IFG work in concert to promote cognitive persistence in the absence of extrinsic rewards. Thus, frontostriatal circuitry may be a neurobiological signal representing intrinsic motivation for self-improvement that serves an adaptive function, increasing Chinese students' motivation to engage in cognitive persistence. Copyright © 2017 Elsevier Inc. All rights

  17. Revertant mutation releases confined lethal mutation, opening Pandora's box: a novel genetic pathogenesis.

    Directory of Open Access Journals (Sweden)

    Yasushi Ogawa

    2014-05-01

    Full Text Available When two mutations, one dominant pathogenic and the other "confining" nonsense, coexist in the same allele, theoretically, reversion of the latter may elicit a disease, like the opening of Pandora's box. However, cases of this hypothetical pathogenic mechanism have never been reported. We describe a lethal form of keratitis-ichthyosis-deafness (KID syndrome caused by the reversion of the GJB2 nonsense mutation p.Tyr136X that would otherwise have confined the effect of another dominant lethal mutation, p.Gly45Glu, in the same allele. The patient's mother had the identical misssense mutation which was confined by the nonsense mutation. The biological relationship between the parents and the child was confirmed by genotyping of 15 short tandem repeat loci. Haplotype analysis using 40 SNPs spanning the >39 kbp region surrounding the GJB2 gene and an extended SNP microarray analysis spanning 83,483 SNPs throughout chromosome 13 in the family showed that an allelic recombination event involving the maternal allele carrying the mutations generated the pathogenic allele unique to the patient, although the possibility of coincidental accumulation of spontaneous point mutations cannot be completely excluded. Previous reports and our mutation screening support that p.Gly45Glu is in complete linkage disequilibrium with p.Tyr136X in the Japanese population. Estimated from statisitics in the literature, there may be approximately 11,000 p.Gly45Glu carriers in the Japanese population who have this second-site confining mutation, which acts as natural genetic protection from the lethal disease. The reversion-triggered onset of the disesase shown in this study is a previously unreported genetic pathogenesis based on Mendelian inheritance.

  18. Efeitos colaterais cutâneos após uso prolongado de hidroxiuréia na Policitemia Vera Cutaneous effects after prolongaded use of hydroxyurea in Polycythemia Vera

    Directory of Open Access Journals (Sweden)

    Emmanuel Rodrigues de França

    2011-08-01

    Full Text Available A hidroxiureia é um derivado hidroxilado da ureia utilizado em diversas desordens hematológicas. Inúmeras alterações cutâneas, porém raras, são relatadas após seu uso prolongado. A patogênese das mesmas não está bem esclarecida, porém, sugere-se que a droga tenha uma ação tóxica direta sobre a pele. Descrevemos um homem de 75 anos, branco, com diagnóstico de Policitemia Vera que, ao longo de 11 anos de tratamento com hidroxiureia, evoluiu com várias lesões cutâneas: hiperpigmentação da pele, lesões atróficas em antebraços, melanoníquia longitudinal das 20 unhas, úlcera em antebraço direito, xerose cutânea, ictiose em pernas e carcinoma espinocelular no pavilhão auricular direito. Até o momento, os relatos na literatura descrevem pouca diversidade de lesões nos pacientes acometidosHydroxyurea is an hydroxylated urea derivative used in many myeloproliferative disorders. Many, but unusual cutaneous disorders are related after its prolonged use. Their pathogenesis is not clear, but it is suggested that there is direct toxicity of the drug on the skin. We described a white, 75-year old man with diagnosis of Polycythemia Vera that in 11 years of treatment developed many cutaneous lesions: skin hyperpigmentation, atrophic lesions on forearms, longitudinal melanonychia of 20 nails, right forearm ulcer, cutaneous xerosis, ichthyosis and auricular spinocellular carcinoma. At this moment, the literature reports describe little diversity of lesions in affected patients

  19. Familial X/Y Translocation Encompassing ARSE in Two Moroccan Siblings with Sensorineural Deafness.

    Science.gov (United States)

    Amasdl, Saadia; Smaili, Wiam; Natiq, Abdelhafid; Hassani, Amale; Sbiti, Aziza; Agadr, Aomar; Sanlaville, Damien; Sefiani, Abdelaziz

    2017-01-01

    Unbalanced translocations involving X and Y chromosomes are rare and associated with a contiguous gene syndrome. The clinical phenotype is heterogeneous including mainly short stature, chondrodysplasia punctata, ichthyosis, hypogonadism, and intellectual disability. Here, we report 2 brothers with peculiar gestalt, short stature, and hearing loss, who harbor an X/Y translocation. Physical examination, brainstem acoustic potential evaluation, bone age, hormonal assessment, and X-ray investigations were performed. Because of their dysmorphic features, karyotyping, FISH, and aCGH were carried out. The probands had short stature, hypertelorism, midface hypoplasia, sensorineural hearing loss, normal intelligence as well as slight radial and ulnar bowing with brachytelephalangy. R-banding identified a derivative X chromosome with an abnormally expanded short arm. The mother was detected as a carrier of the same aberrant X chromosome. aCGH disclosed a 3.1-Mb distal deletion of chromosome region Xp22.33pter. This interval encompasses several genes, especially the short stature homeobox (SHOX) and arylsulfatase (ARSE) genes. The final karyotype of the probands was: 46,Y,der(X),t(X;Y)(p22;q12).ish der(X)(DXYS129-,DXYS153-)mat.arr[hg19] Xp22.33(61091_2689408)×1mat,Xp22.33(2701273_3258404)×0mat,Yq11.222q12 (21412851_59310245)×2. Herein, we describe a Moroccan family with a maternally inherited X/Y translocation and discuss the genotype-phenotype correlations according to the deleted genes. © 2017 S. Karger AG, Basel.

  20. A STUDY ON PAEDIATRIC DERMATOSES IN CHILDREN ATTENDING OPD OF GVR HOSPITAL, KURNOOL

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    M. Bhuvaneswari

    2018-02-01

    Full Text Available BACKGROUND Paediatric dermatoses include various diseases of neonates such as sebaceous hyperplasia, milia, sucking blisters etc., cutaneous infections such as impetigo, folliculitis, furuncles etc., arthropod bites and infestations such as scabies, pediculosis, nutritional dermatoses such as acrodermatitis enteropathica, essential fatty acid deficiency, kwashiorkor etc., eczematous disorders such as atopic dermatitis, seborrhoeic dermatitis, etc., vesiculobullous disorders such as erythema multiforme, SJS/TEN, hypersensitivity disorders like urticaria, papular urticaria, angioedema etc., photosensitivity diseases like acute sunburn reactions, phototoxic reactions, porphyrias etc., epidermal diseases like psoriasis, lichen planus etc., keratinisation disorders like ichthyosis vulgaris, palmoplantar keratosis, etc., The epidemiological aspects of various childhood dermatoses have been the subject of study by various researchers for over a century, but the accurate incidence or prevalence could not be made out because: lack of properly designed guidelines for evaluation, inadequate and improper compilation, lack of parental awareness and knowledge about the problems, home remedies, treatment by quacks not being reported. MATERIALS AND METHODS It is a hospital based observational study, which was conducted at Govt. general hospital, Kurnool and GVR Hospital, Kurnool. The study included 14,730 children who attended the above departments, of them, 5775 children were males and 5995 children are females. The study was conducted during the period of 1 year from March 2015-June 2016. RESULTS Infections are the commonest (30.44% followed by Infestations (14.40%. Secondary Infections were the commonest bacterial infections (25.37%. Among the infestations Scabies was the most prevalent (77.76% CONCLUSION In our study, it was observed that 28-30% of the children aged below 12 years attending paediatric outpatient department, GVR Hospital and 30-40% of the

  1. Acitretin in pediatric dermatoses

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    Manjyot Gautam

    2016-01-01

    Full Text Available Acitretin, a synthetic retinoid and the active metabolite of etretinate has been increasingly used over the past two decades. It has proved effective in the treatment of many conditions associated with hyperkeratosis and dyskeratosis. A Google scholar search for the use of acitretin in pediatric dermatoses was done using the words “pediatric dermatoses,” “acitretin,” “etretinate,” “systemic retinoids,” “psoriasis,” “pityriasis rubra pilaris,” “ichthyoses,” “disorders of keratinization,” “Darier's disease,” “palmoplantar keratoderma,” “verrucae,” “lichen planus,” “lupus erythematosus,” and “lichen sclerosus.” All the articles were retrieved and classified into review articles, studies, double-blinded trials, and case reports. The final data were then analyzed and presented in a narrative fashion. It has been found that acitretin is useful in a number of pediatric dermatoses. It is preferred over other drugs in pustular psoriasis. Good results can be obtained in various disorders of keratinization, and it may even prove life-saving in conditions like harlequin ichthyosis. However, long-term maintenance therapy is required and exacerbations are known on discontinuing the drug. It can also be used as alternative therapy for many other pediatric dermatoses where the primary treatment has failed. Acitretin should be used even in children for the proper indications. However, proper clinical and laboratory surveillance has to be maintained in patients on long-term acitretin.

  2. Cutaneous adverse reactions of chemotherapy in cancer patients: A clinicoepidemiological study

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    Saumita Ghosh Biswal

    2018-01-01

    Full Text Available Background: The diagnosis of cutaneous adversities in the cancer patient is especially difficult, given the complexity of their illness and combination protocols used for the treatment. The present study was undertaken to know the spectrum of cutaneous adversities in patients undergoing chemotherapy and the drug(s most commonly associated with it. Materials and Methods: A total of 1000 patients with malignancies under chemotherapy in the oncology ward and outpatient department were screened in this observational study from January 2013 to February 2015. Relevant investigations for diagnosis of malignancies under chemotherapy and dermatological disorders were carried out. Results: Three hundred and eighty-four patients presented with cutaneous adversities of chemotherapy. The most common was anagen effluvium (78.6%, followed by xerosis (4.4%, thrombophlebitis (3.1%, generalised pruritus (2.9%, melanonychia (2.9%, hand-foot syndrome (2.6%, extravasation reactions (1.8%, flagellate dermatosis (1.3%, prurigo nodularis (0.8%, exfoliation (0.5%, ichthyosis (0.5%, papulopustular rash (0.3%, bullous photodermatitis (0.3%, and Sweet's syndrome (0.3%. Chemotherapeutic drugs were mostly given in combinations. Most common drugs to cause anagen effluvium were alkylating agents in combinations, hand-foot syndrome by taxanes (docetaxel, flagellate dermatoses by antitumour antibiotics (bleomycin, and exfoliation by antimetabolites (methotrexate. The limitation of this study was to imply a specific drug as the causation of the cutaneous adversities since the chemotherapy mostly consisted of combination protocols. Therefore, we have tried to associate the drug combination itself. Conclusion: Chemotherapeutic drugs produce a range of cutaneous adversities, certain specific adversities pertaining to drugs, and their combinations have been implicated which should be looked for and managed accordingly. Knowledge of the adverse effects of anticancer drugs will help

  3. Cutaneous lesions in new born

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    Sachdeva Meenakshi

    2002-11-01

    Full Text Available Five hundred unselected newborn babies delivered in the Department of Obstetrics and Gynaecology, Unit II of SGBT Hospital attached to Government Medical College, Amritsar during April 2000 to October 2000 were examined for cutaneous lesions daily for the first five days after birth. Different cutaneous lesions were seen in 474(94. 8% newborns. The physiological skin changes observed in order of frequency were Epstein pearls in 305(61%, Mongolian spot in 301(60. 2%, superficial cutaneous desquamation in 200(40%, icterus in 128(25. 6%, milia in 119(23. 8%, sebaceous gland hyperplasia in 107 (21. 4%, occipital alopecia in 94(18. 8%, lanugo in 72(14. 4%, peripheral cyanosis in 47(9. 4%, breast hypertrophy in 29(5. 8% and miniature puberty in 28(5. 6% newborns. Of the transient non-infective skin diseases, erythema toxicum neonatorum was observed most commonly in 105(21 %, followed by miliaria rubra in 103(20. 6% and acne neonatorum in 27(5. 4% newborns. The naevi and other developmental defects in the descending order were salmon patch in 69(13. 8%, congenital melanocytic noevi in 10(2%, accessory tragi in 3(0.6%, spina bifida in 2(0.4%, hydrocephalus in 1(0.2% and poliosis in 1(0.2% newborns. Cradle cap was the only dermatitis observed in 50(10% newborns. One (0.2% case each of Harlequin ichthyosis and labial cyst was seen.

  4. The skin landscape in diabetes mellitus. Focus on dermocosmetic management

    Directory of Open Access Journals (Sweden)

    Piérard GE

    2013-05-01

    Full Text Available Gérald E Piérard,1 Sophie Seité,2 Trinh Hermanns-Lê,3 Philippe Delvenne,3 André Scheen,4 Claudine Piérard-Franchimont3 1Laboratory of Skin Bioengineering and Imaging (LABIC, University of Liège, Liège, Belgium; 2La Roche-Posay Pharmaceutical Laboratories, Asnières, France; 3Department of Dermatopathology, Unilab Lg, Liège University Hospital, Liège, Belgium; 4Department of Diabetology, Nutrition and Metabolic Diseases, and Clinical Pharmacology Unit, Liège University Hospital, Liège, Belgium Background: Some relationships are established between diabetes mellitus (DM and a series of cutaneous disorders. Specific dermatoses are markers for undiagnosed DM. Other disorders represent supervening complications in an already treated DM patient. Objective: To review the information about dermocosmetic care products and their appropriate use in the management and prevention of dermatoses related to DM. Method: The peer-reviewed literature and empiric findings are covered. Owing to the limited clinical evidence available for the use of dermocosmetics, a review of the routine practices and common therapies in DM-related dermatoses was conducted. Results: Some DM-related dermatoses (acanthosis nigricans, pigmented purpuric dermatosis are markers of macrovascular complications. The same disorders and some others (xerosis, Dupuytren's disease have been found to be more frequently associated with microangiopathy. Other skin diseases (alopecia areata, vitiligo were found to be markers of autoimmunity, particularly in type 1 DM. Unsurprisingly, using dermocosmetics and appropriate skin care has shown objective improvements of some DM-related dermatoses, such effects improve the quality of life. The most common skin manifestations of DM fall along continuum between "dry skin," xerosis, and acquired ichthyosis, occurring predominately on the shins and feet. Dermocosmetic products improve the feeling of well-being for DM patients. Keywords: diabetes

  5. Spontaneous atopic dermatitis-like symptoms in a/a ma ft/ma ft/J flaky tail mice appear early after birth.

    Directory of Open Access Journals (Sweden)

    Magdalini Kypriotou

    Full Text Available Loss-of-function mutations in human profilaggrin gene have been identified as the cause of ichthyosis vulgaris (IV, and as a major predisposition factor for atopic dermatitis (AD. Similarly, flaky tail (a/a ma ft/ma ft/J mice were described as a model for IV, and shown to be predisposed to eczema. The aim of this study was to correlate the flaky tail mouse phenotype with human IV and AD, in order to dissect early molecular events leading to atopic dermatitis in mice and men, suffering from filaggrin deficiency. Thus, 5-days old flaky tail pups were analyzed histologically, expression of cytokines was measured in skin and signaling pathways were investigated by protein analysis. Human biopsies of IV and AD patients were analyzed histologically and by real time PCR assays. Our data show acanthosis and hyperproliferation in flaky tail epidermis, associated with increased IL1β and thymic stromal lymphopoietin (TSLP expression, and Th2-polarization. Consequently, NFκB and Stat pathways were activated, and IL6 mRNA levels were increased. Further, quantitative analysis of late epidermal differentiation markers revealed increased Small proline-rich protein 2A (Sprr2a synthesis. Th2-polarization and Sprr2a increase may result from high TSLP expression, as shown after analysis of 5-days old K14-TSLP tg mouse skin biopsies. Our findings in the flaky tail mouse correlate with data obtained from patient biopsies of AD, but not IV. We propose that proinflammatory cytokines are responsible for acanthosis in flaky tail epidermis, and together with the Th2-derived cytokines lead to morphological changes. Accordingly, the a/a ma ft/ma ft/J mouse model can be used as an appropriate model to study early AD onset associated with profilaggrin deficiency.

  6. A clinico-etiological study of dermatoses in pediatric age group in tertiary health care center in South Gujarat region

    Directory of Open Access Journals (Sweden)

    Sugat A Jawade

    2015-01-01

    Full Text Available Background: Dermatologic conditions have different presentation and management in pediatric age group from that in adult; this to be studied separately for statistical and population based analysis. Objective: To study the pattern of various dermatoses in infants and children in tertiary health care center in South Gujarat region. Materials and Methods: This is a prospective study; various dermatoses were studied in pediatric patients up to 14 years of age attending the Dermatology OPD of New Civil Hospital, Surat, Gujarat over a period of 12 months from June 2009 to June 2010. All patients were divided into four different study groups: 1 to 6 years and 7 to 14 years. Results: There were 596 boys and 425 girls in total 1021 study populations. Majority of the skin conditions in neonates were erythema toxicum neonatorum (12.97%, scabies (9.92%, mongolian spot (9.16%, and seborrheic dermatitis (7.63%. In > 1 month to 14 years age group of children among infectious disorder, children were found to be affected most by scabies (24.49%, impetigo (5.96%, pyoderma (5.62%, molluscum contagiosum (5.39%, tinea capitis (4.49%, leprosy (2.02%, and viral warts (1.35% while among non-infectious disorders, they were affected by atopic dermatitis (4.27%, pityriasis alba (4.16%, seborrheic dermatitis (3.60%, pityriasis rosea (3.15%, others (3.01%, phrynoderma (2.70%, lichen planus (2.58%, contact dermatitis (1.57% and ichthyosis (1.45%. Conclusion: There is a need to emphasize on training the management of common pediatric dermatoses to dermatologists, general practitioners and pediatricians for early treatment.

  7. Cell cycle- and cancer-associated gene networks activated by Dsg2: evidence of cystatin A deregulation and a potential role in cell-cell adhesion.

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    Abhilasha Gupta

    Full Text Available Cell-cell adhesion is paramount in providing and maintaining multicellular structure and signal transmission between cells. In the skin, disruption to desmosomal regulated intercellular connectivity may lead to disorders of keratinization and hyperproliferative disease including cancer. Recently we showed transgenic mice overexpressing desmoglein 2 (Dsg2 in the epidermis develop hyperplasia. Following microarray and gene network analysis, we demonstrate that Dsg2 caused a profound change in the transcriptome of keratinocytes in vivo and altered a number of genes important in epithelial dysplasia including: calcium-binding proteins (S100A8 and S100A9, members of the cyclin protein family, and the cysteine protease inhibitor cystatin A (CSTA. CSTA is deregulated in several skin cancers, including squamous cell carcinomas (SCC and loss of function mutations lead to recessive skin fragility disorders. The microarray results were confirmed by qPCR, immunoblotting, and immunohistochemistry. CSTA was detected at high level throughout the newborn mouse epidermis but dramatically decreased with development and was detected predominantly in the differentiated layers. In human keratinocytes, knockdown of Dsg2 by siRNA or shRNA reduced CSTA expression. Furthermore, siRNA knockdown of CSTA resulted in cytoplasmic localization of Dsg2, perturbed cytokeratin 14 staining and reduced levels of desmoplakin in response to mechanical stretching. Both knockdown of either Dsg2 or CSTA induced loss of cell adhesion in a dispase-based assay and the effect was synergistic. Our findings here offer a novel pathway of CSTA regulation involving Dsg2 and a potential crosstalk between Dsg2 and CSTA that modulates cell adhesion. These results further support the recent human genetic findings that loss of function mutations in the CSTA gene result in skin fragility due to impaired cell-cell adhesion: autosomal-recessive exfoliative ichthyosis or acral peeling skin syndrome.

  8. The Effect of Multiple Sulfatase Deficiency (MSD) on Dental Development: Can We Use the Teeth as an Early Diagnostic Tool?

    Science.gov (United States)

    Zilberman, Uri; Bibi, Haim

    2016-01-01

    Multiple sulfatase deficiency (MSD) is a rare autosomal recessive inborn error of metabolism due to reduced catalytic activity of the different sulfatase. Affected individuals show neurologic deterioration with mental retardation, skeletal anomalies, organomegaly, and skin changes as in X-linked ichthyosis. The only organ that was not examined in MSD patients is the dentition. To evaluate the effect of the metabolic error on dental development in a patient with the intermediate severe late-infantile form of MSD (S155P). Histological and chemical study were performed on three deciduous and five permanent teeth from MSD patient and pair-matched normal patients. Tooth germ size and enamel thickness were reduced in both deciduous and permanent MSD teeth, and the scalloping feature of the DEJ was missing in MSD teeth causing enamel to break off from the dentin. The mineral components in the enamel and dentin were different. The metabolic error insults the teeth in the stage of organogenesis in both the deciduous and permanent dentition. The end result is teeth with very sharp cusp tips, thin hypomineralized enamel, and exposed dentin due to the break off of enamel. These findings are different from all other types of MPS syndromes.Clinically the phenotype of intermediate severe late-infantile form of MSD appeared during the third year of life. In children of parents that are carriers, we can diagnose the disease as early as birth using X-ray radiograph of the anterior upper region or as early as 6-8 months when the first deciduous tooth erupt and consider very early treatment to ameliorate the symptoms.

  9. Impaired quality of life in patients with systemic sclerosis compared to the general population and chronic dermatoses.

    Science.gov (United States)

    Bretterklieber, Agnes; Painsi, Clemens; Avian, Alexander; Wutte, Nora; Aberer, Elisabeth

    2014-09-02

    Systemic sclerosis (SSc) is a rare and potentially life threatening autoimmune disorder. The burden of disease compared to other dermatoses is unknown. The purpose of this study was to assess both the quality of life in patients with SSc and the variables that are associated with poor quality of life. Forty-one patients with systemic sclerosis (29 limited, 2 diffuse, 10 undifferentiated forms) were assessed with respect to their health status and compared to published data for the normal population, SSc patients from other studies, and patients with chronic skin diseases. For the most part, our SSc patients had better outcomes in all 8 dimensions of the SF-36 than SSc patients from other studies, and poorer scores than the healthy population and those with occupational contact dermatitis, ichthyosis, non-melanoma skin cancer, contact dermatitis, atopic eczema, chronic nail disease, vitiligo, health care workers with work-related disease, and those with other chronic skin diseases, but significantly better scores for mental health than those with nail disease, vitiligo, and health-care workers. Patients with atopic dermatitis, psoriasis and pemphigus had significantly poorer mean scores in social function and mental health than SSc patients. Patients with pemphigus were also significantly impaired in their physical and emotional roles. Patients with systemic lupus erythematosus (SLE) had the significantly poorest mean scores for QoL in all 8 domains except bodily pain and emotional role. Besides SLE, SSc is one of the most severe chronic dermatologic diseases in terms of reduced QoL. Since SSc cannot be cured, treatment strategies should include therapeutic interventions such as psychotherapy, social support, physiotherapy, and spiritual care. Their beneficial effects could be studied in future.

  10. Xeroderma Pigmentosum-Trichothiodystrophy overlap patient with novel XPD/ERCC2 mutation

    Science.gov (United States)

    Kralund, Henrik H.; Ousager, Lilian; Jaspers, Nicolaas G.; Raams, Anja; Pedersen, Erling B.; Gade, Else; Bygum, Anette

    2013-01-01

    Xeroderma Pigmentosum (XP), Trichothiodystrophy (TTD) and Cockayne Syndrome (CS) are rare, recessive disorders caused by mutational defects in the Nucleotide Excision Repair (NER) pathway and/or disruption of basic cellular DNA transcription. To date, a multitude of mutations in the XPD/ERCC2 gene have been described, many of which give rise to NER- and DNA transcription related diseases, which share certain diagnostic features and few overlap patients have been described. Despite increasing understanding of the roles of XPD/ERCC2 in mammalian cells, there is still weak predictability of somatic outcome from many of these mutations. We demonstrate a patient, believed to represent an overlap between XP and TTD/CS. In addition to other organ dysfunctions, the young man presented with Photosensitivity, Ichthyosis, Brittle hair, Impaired physical and mental development, Decreased fertility and Short stature (PIBIDS) suggestive of TTD, but lacking the almost patognomonic “tiger tail” banding of the hair under polarized light. Additionally, he developed basal cell carcinoma aged 28, as well as adult onset kidney failure, features normally not associated with TTD but rather XP/CS. His freckled appearance also suggested XP, but fibroblast cultures only demonstrated x2 UV-sensitivity with expected NER and TFIIH-activity decrease. Genetic sequencing of the XPD/ERCC2 gene established the patient as heterozygote compound with a novel, N-terminal Y18H mutation and a known C-terminal (TTD) mutation, A725P. The possible interplay between gene products and the patient phenotype is discussed. PMID:25002996

  11. Homozygous deletion of six genes including corneodesmosin on chromosome 6p21.3 is associated with generalized peeling skin disease.

    Science.gov (United States)

    Teye, Kwesi; Hamada, Takahiro; Krol, Rafal P; Numata, Sanae; Ishii, Norito; Matsuda, Mitsuhiro; Ohata, Chika; Furumura, Minao; Hashimoto, Takashi

    2014-07-01

    Peeling skin syndrome (PSS) is a rare autosomal recessive form of ichthyosis showing skin exfoliation. PSS is divided into acral and generalized PSS, and the latter is further classified into non-inflammatory type (PSS type A) and inflammatory type (PSS type B). PSS type B is now called peeling skin disease (PSD). Different loss-of-function mutations in the corneodesmosin (CDSN) gene have been reported to cause PSD. The aim of this study was to determine genetic basis of disease in a 14-year-old Japanese patient with PSD. Immunohistochemical study showed lack of corneodesmosin (CDSN) in the skin, and standard PCR for genomic DNA failed to amplify CDSN product, suggesting CDSN defect. Multiplex ligation-dependent probe amplification and genomic quantitative real-time PCR analyses detected large homozygous deletion of 59,184bp extending from 40.6kb upstream to 13.2kb downstream of CDSN, which included 6 genes (TCF19, CCHCR1, PSORS1C2, PSORS1C1, CDSN and C6orf15). The continuous gene lost did not result in additional clinical features. Inverted repeats with 85% similarity flanking the deletion breakpoint were considered to mediate the deletion by non-homologous end joining or fork stalling and template switching/microhomology-mediated break-induced replication. Parents were clinically unaffected and were heterozygote carriers of the same deletion, which was absent in 284 ethnically matched control alleles. We also developed simple PCR method, which is useful for detection of this deletion. Although 5 other genes were also deleted, homozygous deletion of CDSN was considered to be responsible for this PSD. Copyright © 2014 Japanese Society for Investigative Dermatology. Published by Elsevier Ireland Ltd. All rights reserved.

  12. Applications of hydroxy acids: classification, mechanisms, and photoactivity

    Directory of Open Access Journals (Sweden)

    Andrija Kornhauser

    2010-11-01

    Full Text Available Andrija Kornhauser1, Sergio G Coelho2, Vincent J Hearing21US Food and Drug Administration [retired], Annandale, VA, USA; 2Laboratory of Cell Biology, National Cancer Institute, National Institutes of Health, Bethesda, MD, USAAbstract: Hydroxy acids (HAs represent a class of compounds which have been widely used in a number of cosmetic and therapeutic formulations in order to achieve a variety of beneficial effects for the skin. We review and discuss the most frequently used classes of these compounds, such as a-hydroxy acids, β-hydroxy acids, polyhydroxy acids, and bionic acids, and describe their applications as cosmetic and therapeutic agents. Special emphasis is devoted to the safety evaluation of these formulations, in particular on the effects of their prolonged use on sun-exposed skin. Furthermore, we summarize the very limited number of studies dealing with the modifications evoked by topical application of products containing HAs on photocarcinogenesis. In spite of the large number of reports on the cosmetic and clinical effects of HAs, their biological mechanism(s of action still require more clarification. Some of these mechanisms are discussed in this article along with important findings on the effect of HAs on melanogenesis and on tanning. We also emphasize the important contribution of cosmetic vehicles in these types of studies. Thus, HAs play an important role in cosmetic formulations, as well as in many dermatologic applications, such as in treating photoaging, acne, ichthyosis, rosacea, pigmentation disorders, and psoriasis.Keywords: hydroxy acids, glycolic acid, salicylic acid, UV, erythema, cosmetics

  13. MRI and ¹H-MRS findings of three patients with Sjögren-Larsson syndrome Síndrome de Sjögren-Larsson: achados à ressonância magnética e espectroscopia de prótons em três pacientes

    Directory of Open Access Journals (Sweden)

    Mauro Nakayama

    2006-06-01

    Full Text Available Sjögren-Larsson syndrome (SLS is a rare autosomal recessive neurocutaneous disorder caused by deficiency of the microsomal enzyme fatty aldehyde dehydrogenase. Patients present the classical triad of congenital ichthyosis, mental retardation and spastic di- or tetraplegia. Magnetic resonance imaging (MRI of the brain usually shows hypomyelination involving the periventricular white matter. Cerebral proton MR spectroscopy (¹H-MRS reveals a characteristic abnormal lipid peak. We report three cases of SLS from different families with the typical clinical triad. The MRI and ¹H-MRS findings are discussed.A síndrome de Sjögren-Larsson (SJL é distúrbio raro, autossômico recessivo, caracterizado pela tríade clássica de ictiose congênita, retardo mental e tetraplegia ou diplegia espástica. Trata-se de um erro inato do metabolismo dos lipídios, causado pela deficiência da enzima microssômica aldeído graxo desidrogenase. Os achados de imagem do encéfalo na SJL demonstram atrofia cerebral e alteração da substância branca. A espectroscopia de prótons, com poucos casos relatados, caracteriza-se pelo elevado pico de lipídios e redução de N-acetil-aspartato. Apresentamos três casos de SJL, com ênfase nos achados da ressonância magnética e da espectroscopia de prótons.

  14. Why envy outperforms admiration.

    Science.gov (United States)

    van de Ven, Niels; Zeelenberg, Marcel; Pieters, Rik

    2011-06-01

    Four studies tested the hypothesis that the emotion of benign envy, but not the emotions of admiration or malicious envy, motivates people to improve themselves. Studies 1 to 3 found that only benign envy was related to the motivation to study more (Study 1) and to actual performance on the Remote Associates Task (which measures intelligence and creativity; Studies 2 and 3). Study 4 found that an upward social comparison triggered benign envy and subsequent better performance only when people thought self-improvement was attainable. When participants thought self-improvement was hard, an upward social comparison led to more admiration and no motivation to do better. Implications of these findings for theories of social emotions such as envy, social comparisons, and for understanding the influence of role models are discussed.

  15. Modulinis mokymas profesinio rengimo sistemoje : samprata ir principai

    OpenAIRE

    Navikienė, Živilė

    2008-01-01

    Many documents reflect the concern how to make VET system not only more attractive but also more available. Modular training is considered to be among the prerequisites for more flexible VET system. Modular training provides opportunities to students and teachers for creativity development as well as promotes independent continuous self-improvement. VET modularisation can help persons not only to acquire lacking competencies but also return to education system and at the same time to become m...

  16. The Attitudes of Teacher Trainees Towards Life Knowledge and Social Studies Teaching Course

    OpenAIRE

    S. Gulec; S. Alkis

    2009-01-01

    In Turkey, Social Studies programme basically aims to raise active and productive citizens. This means that teachers are going to inject philosophy of life to the students by means of social studies course. In order to carry out this responsibility, teachers and teachers-to-be should be accustomed to comprehension and learning processes of children and adolescents. By continuous self-improvement, the teachers should try to get more information on methods, materials and tools that can be used ...

  17. The influence of organizational culture on building a learning organization

    OpenAIRE

    Piłat, Michał

    2011-01-01

    The organizations of the 21st century more and more often see their human resources as the most valuable asset they hold. This is why they create wide range of opportunities to develop and self-improve for their employees. Such approach is aimed at increasing involvement, higher effectiveness, innovativeness and thus constant improvement of competitiveness. However this is not possible without employee constant learning and their selfactualization. In this way the learni...

  18. Self-Expression Through Brand and Consumption Choices: Examining Cross-Cultural Differences

    OpenAIRE

    Takashima, Mirei

    2016-01-01

    In this dissertation, I investigated how the brand and consumption choices across cultures vary in meaning. In particular, I examined how self-expression through choice varies between the Western and East Asian cultural contexts due to the difference in how the self is viewed. Specifically, Westerners express self-consistency because they view themselves as independent and consistent regardless of the context. In contrast, East Asians express through self-improvement efforts because they view...

  19. Lives-long learning: the effects of reincarnation belief on everyday life in England1

    OpenAIRE

    Walter, Tony; Waterhouse, Helen

    2001-01-01

    A sizeable minority of Westerners who have no particular connection with Eastern or New Age religions nevertheless claim to believe in reincarnation. Does this belief affect their practical morality and how they think about suffering and injustice? An interview study conducted in England mapped the range of meanings such people give to reincarnation, and found: 1) Karma was widely referred to, but in the context of Western notions of self-improvement; there was little recognition of the possi...

  20. Bootstrapping pronunciation models

    CSIR Research Space (South Africa)

    Davel, M

    2006-07-01

    Full Text Available -scarce language. During the procedure known as ‘bootstrapping’, a model is improved iteratively via a controlled series of increments, at each stage using the previous model to generate the next. This self- improving circularity distinguishes bootstrapping...-to-phoneme rules (the second representation) can be used to identify possible errors that require re-verification. In contrast, during the bootstrapping of acoustic models for speech recognition, both representations are amenable to automated analysis...

  1. Framework of Six Sigma implementation analysis on SMEs in Malaysia for information technology services, products and processes

    OpenAIRE

    Wong, Whee Yen; ,; ,

    2015-01-01

    For the past two decades, the majority of Malaysia’s IT companies have been widely adopting a Quality Assurance (QA) approach as a basis for self-improvement and internal-assessment in IT project management. Quality Control (QC) is a comprehensive top-down observation approach used to fulfill requirements for quality outputs which focuses on the aspect of process outputs evaluation. However in the Malaysian context, QC and combination of QA and QC as a means of quality improvement approaches ...

  2. The Third NDT Technology Forum

    International Nuclear Information System (INIS)

    2005-04-01

    This reports the presentation for enactment of law on development of nondestructive testing and management, which includes meaning and content of enactment of NDT law like background, process, important content, and demand for cooperation. It has four presentations on nondestructive testing ; safety and well-being trend and industrial function of NDT, safety function in public facilities and diversity of technical application, train and education and direction and attitude of self-improvement and industrial function and requirement of inspection of company.

  3. Creation of Learning Motivation in the Process of Cognitive Learning in Teaching Music to 12-13 Years Old Pupils

    OpenAIRE

    Kepule, Iveta

    2015-01-01

    Creation of learning motivation is one of the main problems of the school. Motivation is the basis for self-improvement; its fulfillment facilitates development of the personality and encourages a pupil to study better in order to reach the set goal. If the teacher knows the reasons for the learning motivation of the pupils, he or she can select the methods and techniques, which could stimulate the inquiry process, and in addition to development of various skills and abilities would increase ...

  4. From cattle and coke to Charlie: meeting the challenge of self marketing and personal branding

    OpenAIRE

    Shepherd, Ifan D. H.

    2005-01-01

    Since the late 1990s, self marketing and personal branding have become increasingly popular as subjects of self-improvement books, Web sites and consultancy services, especially in the USA. To date, little of this interest appears to have permeated the discipline of marketing, either in terms of formal research, textbook contents or academic curricula. This paper examines the theoretical basis of self marketing and personal branding, identifies some of the conceptual, practical and ethical pr...

  5. Education and the doctrine of the Mean in Aristotle and in Confucius

    OpenAIRE

    Panos ELIOPOULOS

    2014-01-01

    Aristotle and Confucius elaborate their theories on the basis of a complex apprehen - sion of the ethical and political problem as one. The Greek and the Chinese philosopher focus on the importance of virtue, which signifies a passage from an initial understanding of communal life to a life with others that becomes self-fulfilling and facilitates self improvement and excellence. The individual goal is the same as the collective goal; this becomes the foundation o...

  6. The Dynamics of Terminal and Instrumental Values of Education on the Example of MGIMO Students

    Directory of Open Access Journals (Sweden)

    Alexander L. Temnitskiy

    2015-01-01

    Full Text Available Attitude towards derivable knowledge as a process of satisfaction of cognition needs and also as opportunity for developing flairs and making self-improvement can be called terminal. At the same time, the increase of pragmatism in society and in demands of employers for graduates doesn't stimulate students' interest in terminal values of education. Nowadays students become much more interested in receiving good marks and good exam results than in the matter of subject that they study; they become more attracted by an opportunity of having a good time in university and making helpful acquaintances than by a chance of making a professional self-determination. How spread is the instrumental students' attitude towards studying at the university? Is it possible that under the influence of this phenomenon the terminal values of education may be extruded? These and other questions are being examined on the base of materials of four recurrent sociological researches carried out among MGIMO students in 2001-2013. The main objects of analysis are the values of educational process, relations with fellow-students, essential qualities of lecturer's personality, ways of spending free time. It was found out that the terminal educational values predominate among MGIMO students. They study a lot, tensely and with enthusiasm, spending the modal part of time doing their homework, actively working on their self-improvement. It is revealed that intimate knowledge and self-improvement as 2 main terminal values in education assign multidirectional vectors towards different aspects of educational process.

  7. The Effect of Clove Bud, Nigella, Salix Alba and Olive Oil on Wart Treatment in Comparison with Conventional Treatment

    Directory of Open Access Journals (Sweden)

    roghaye Jebraili

    2007-10-01

    Full Text Available Jebraili R1, Rezaei K2, Matourianpour H3, Moradi L4, Meshkaat MH5, Tarrahi MJ6 1. Assistant professor, Department of Dermatology, Faculty of Medicine, Islamic Azad University of Tehran 2. Instructor, Department of Nursing, Faculty of Nursing and Midwifery, Lorestan University of Medical Sciences 3. Assistant Professor, Department of Dermatology, Faculty of Medicine, Lorestan University of Medical Sciences 4. BSc. Nurse, Haaj Seddiq Health and Treatment Center, Khorramabad 5. Assistant Professor, Department of Chemistry, Lorestan University 6. Instructor, Department of Epidemiology and Statistics, Faculty of Medicine, Lorestan University of Medical Sciences Abstract Background: Wart is a common and contagious viral disease of the skin caused by papilloma viruses which leads to aesthetic and psychological problems, and if occurred in touching and pressured parts of the body causes pain and inflammation. The aim of this study was to investigate the effect of Clove bud, Nigella, Salix alba and Olive oil which have wound disinfectant, anesthetic, analgesic and wound healing properties on wart treatment in comparison with the conventional treatment. Materials and methods: This randomized double blind controlled clinical trial was conducted on 291 female students selected form guidance and high schools in Khorramabad, Lorestan, Iran, during the year 2007. The diagnosis of wart was confirmed by a dermatologist according to the diagnosis criteria. The cases fulfilling the inclusion criteria were assigned in 3 study groups randomly. The first group was treated with the conventional treatment (Salicylic acid 16.7%, lactic acid 16.7% in Collodione body, the second group with herbal medicine without acid in olive oil, and the third group with herbal medicine alongside salicylic acid 1% and lactic acid 1% in olive oil. Each group was administered the drugs for 6 weeks. The effects of drugs on lesions were assessed after 4 and 6 weeks and compared among 3

  8. Some Nigerian plants of dermatologic importance.

    Science.gov (United States)

    Ajose, Frances O A

    2007-10-01

    According to the World Health Organization (WHO), 80% of the world's population uses medicinal plants in the treatment of diseases and, in African countries, this rate is much higher. In recent years, however, medicinal plants have represented a primary health source for the pharmaceutical industry. No less than 400 compounds derived from plants are currently used in the preparation of drugs, such as vincristine and vinblastine used in the treatment of cancer. Nigerians still depend largely on crude herbal remedies or traditional medicine. They also use wild plants for cosmetics and perfumery. Some of these herbal remedies have been observed to be effective in certain skin diseases. The data were obtained from history questionnaires completed by patients at the Dermatology Clinic, Lagos State University Teaching Hospital (LASUTH), Ikeja, Lagos, Nigeria, and from oral interviews with vendors and prescribers of herbal preparations at major markets at Lagos and Ijebu-Ode in south-west Nigeria, between July 2004 and July 2006. Photographs of plants were taken at private residences at Lagos, Ibadan, and Ijebu-Ode in south-west Nigeria. A literature search was conducted on 38 of the plants. The data are presented in tabular form. Sixty-five per cent of patients had applied some form of herbal remedy before attending our clinic. The reasons for consultation included relapses, unsustained relief, incomplete resolution, and post inflammatory hyperpigmentation. Lesions for which herbs were successfully applied included infantile eczema and seborrhoiec dermatitis, atopic eczema, impetigo, impetiginized eczema, tinea capitis, scabies, erythema multiforme, leg ulcers, localized vitiligo, and sexually transmitted diseases. Partial relief was achieved in dermatophytoses, ichthyosis, leprosy, and systemic lupus erythematosus (SLE). Some forms of alopecia, onychomycosis, and vitiligo, as well as allergic dermatoses, were not improved by herbal medicines. The preparation of the

  9. [Spontaneous models of human diseases in dogs: ichthyoses as an example].

    Science.gov (United States)

    André, Catherine; Grall, Anaïs; Guaguere, Éric; Thomas, Anne; Galibert, Francis

    2013-06-01

    Ichthyoses encompass a heterogeneous group of genodermatoses characterized by abnormal desquamation over the entire body due to defects of the terminal differentiation of keratinocytes and desquamation, which occur in the upper layer of the epidermis. Even though in humans more than 40 genes have already been identified, the genetic causes of several forms remain unknown and are difficult to identify in Humans. Strikingly, several purebred dogs are also affected by specific forms of ichthyoses. In the Golden retriever dog breed, an autosomal recessive form of ichthyosis, resembling human autosomal recessive congenital ichthyoses, has recently been diagnosed with a high incidence. We first characterized the disease occurring in the golden retriever breed and collected cases and controls. A genome-wide association study on 40 unrelated Golden retriever dogs, using the canine 49.000 SNPs (single nucleotide polymorphisms) array (Affymetrix v2), followed by statistical analyses and candidate gene sequencing, allowed to identify the causal mutation in the lipase coding PNPLA1 gene (patatin-like phospholipase domain-containing protein). Screening for alterations in the human ortholog gene in 10 autosomal recessive congenital ichthyoses families, for which no genetic cause has been identified thus far, allowed to identify two recessive mutations in the PNPLA1 protein in two families. This collaborative work between "human" and "canine" geneticists, practicians, histopathologists, biochemists and electron microscopy experts not only allowed to identify, in humans, an eighth gene for autosomal recessive congenital ichthyoses, but also allowed to highlight the function of this as-yet-unknown skin specific lipase in the lipid metabolism of the skin barrier. For veterinary medicine and breeding practices, a genetic test has been developed. These findings illustrate the importance of the discovery of relevant human orthologous canine genetic diseases, whose causes can be tracked

  10. Design and Characterization of a Human Monoclonal Antibody that Modulates Mutant Connexin 26 Hemichannels Implicated in Deafness and Skin Disorders

    Directory of Open Access Journals (Sweden)

    Liang Xu

    2017-09-01

    Full Text Available Background: Mutations leading to changes in properties, regulation, or expression of connexin-made channels have been implicated in 28 distinct human hereditary diseases. Eight of these result from variants of connexin 26 (Cx26, a protein critically involved in cell-cell signaling in the inner ear and skin. Lack of non-toxic drugs with defined mechanisms of action poses a serious obstacle to therapeutic interventions for diseases caused by mutant connexins. In particular, molecules that specifically modulate connexin hemichannel function without affecting gap junction channels are considered of primary importance for the study of connexin hemichannel role in physiological as well as pathological conditions. Monoclonal antibodies developed in the last three decades have become the most important class of therapeutic biologicals. Recombinant methods permit rapid selection and improvement of monoclonal antibodies from libraries with large diversity.Methods: By screening a combinatorial library of human single-chain fragment variable (scFv antibodies expressed in phage, we identified a candidate that binds an extracellular epitope of Cx26. We characterized antibody action using a variety of biochemical and biophysical assays in HeLa cells, organotypic cultures of mouse cochlea and human keratinocyte-derived cells.Results: We determined that the antibody is a remarkably efficient, non-toxic, and completely reversible inhibitor of hemichannels formed by connexin 26 and does not affect direct cell-cell communication via gap junction channels. Importantly, we also demonstrate that the antibody efficiently inhibits hyperative mutant Cx26 hemichannels implicated in autosomal dominant non-syndromic hearing impairment accompanied by keratitis and hystrix-like ichthyosis-deafness (KID/HID syndrome. We solved the crystal structure of the antibody, identified residues that are critical for binding and used molecular dynamics to uncover its mechanism of action

  11. Distribution of ELOVL4 in the Developing and Adult Mouse Brain

    Directory of Open Access Journals (Sweden)

    David M. Sherry

    2017-05-01

    Full Text Available ELOngation of Very Long chain fatty acids (ELOVL-4 is essential for the synthesis of very long chain-fatty acids (fatty acids with chain lengths ≥ 28 carbons. The functions of ELOVL4 and its very long-chain fatty acid products are poorly understood at present. However, mutations in ELOVL4 cause neurodevelopmental or neurodegenerative diseases that vary according to the mutation and inheritance pattern. Heterozygous inheritance of different ELOVL4 mutations causes Stargardt-like Macular Dystrophy or Spinocerebellar Ataxia type 34. Homozygous inheritance of ELOVL4 mutations causes more severe disease characterized by seizures, intellectual disability, ichthyosis, and premature death. To better understand ELOVL4 and very long chain fatty acid function in the brain, we examined ELOVL4 expression in the mouse brain between embryonic day 18 and postnatal day 60 by immunolabeling using ELOVL4 and other marker antibodies. ELOVL4 was widely expressed in a region- and cell type-specific manner, and was restricted to cell bodies, consistent with its known localization to endoplasmic reticulum. ELOVL4 labeling was most prominent in gray matter, although labeling also was present in some cells located in white matter. ELOVL4 was widely expressed in the developing brain by embryonic day 18 and was especially pronounced in regions underlying the lateral ventricles and other neurogenic regions. The basal ganglia in particular showed intense ELOVL4 labeling at this stage. In the postnatal brain, cerebral cortex, hippocampus, cerebellum, thalamus, hypothalamus, midbrain, pons, and medulla all showed prominent ELOVL4 labeling, although ELOVL4 distribution was not uniform across all cells or subnuclei within these regions. In contrast, the basal ganglia showed little ELOVL4 labeling in the postnatal brain. Double labeling studies showed that ELOVL4 was primarily expressed by neurons, although presumptive oligodendrocytes located in white matter tracts also showed

  12. Common FLG mutation K4671X not associated with atopic dermatitis in Han Chinese in a family association study.

    Directory of Open Access Journals (Sweden)

    Ruhong Cheng

    Full Text Available BACKGROUND: Filaggrin gene (FLG mutations have been identified as the cause of ichthyosis vulgaris (IV and major predisposing factors for atopic dermatitis (AD. The relationship among AD, IV and FLG mutations has not been clarified yet. Mutations 3321delA and K4671X, two of the most common mutations in Chinese patients, were both statistically associated with AD in case-control studies. MATERIALS AND METHODS: A group of 100 family trios (a total of 300 members with one affected AD proband and both parents were recruited and screened for three filaggrin null mutations (3222del4, 3321delA and K4671X. The subjects' manifestations of AD and IV were assessed by two experienced dermatologists and recorded in detail. The relationship of common mutations to AD were assessed using both case-control and family-based tests of association. Filaggrin expression was measured in skin of 3 subjects with K4671X heterozygote and the normal control using quantitative real-time RT-PCR and immunohistochemistry. RESULTS: Of 100 probands for AD, 22 were carriers for common FLG mutations and only 2 of them were from 40 none-IV family trios (5.00%, consistent with that of the healthy control group (3.99%, P>0.05. Significant statistical associations were revealed between AD and 3321delA (P<0.001, odds ratio 12.28, 95% confidence interval 3.35-44.98 as well as K4671X (P = 0.002, odds ratio 4.53, 95% confidence interval 1.77-11.60. The family-based approach revealed that 3321delA was over-transmitted to AD offspring from parents (T:U = 12∶1, P = 0.003 but failed to demonstrate transmission disequilibrium between K4671X and AD (T:U = 10∶8, P = 0.815. Moreover, compared to the normal control, filaggrin expression at both mRNA and protein levels in epidermis of subjects with K4671X(heter was not reduced. CONCLUSIONS: AD patients from none-IV family trios have low probability of carrying FLG mutations. The present family samples confirmed the

  13. A distal region of the human TGM1 promoter is required for expression in transgenic mice and cultured keratinocytes

    Directory of Open Access Journals (Sweden)

    Lu Ying

    2004-04-01

    Full Text Available Abstract Background TGM1(transglutaminase 1 is an enzyme that crosslinks the cornified envelope of mature keratinocytes. Appropriate expression of the TGM1 gene is crucial for proper keratinocyte function as inactivating mutations lead to the debilitating skin disease, lamellar ichthyosis. TGM1 is also expressed in squamous metaplasia, a consequence in some epithelia of vitamin A deficiency or toxic insult that can lead to neoplasia. An understanding of the regulation of this gene in normal and abnormal differentiation states may contribute to better disease diagnosis and treatment. Methods In vivo requirements for expression of the TGM1 gene were studied by fusing various lengths of promoter DNA to a reporter and injecting the DNA into mouse embryos to generate transgenic animals. Expression of the reporter was ascertained by Western blotting and immunohistochemistry. Further delineation of a transcriptionally important distal region was determined by transfections of progressively shortened or mutated promoter DNA into cultured keratinocytes. Results In vivo analysis of a reporter transgene driven by the TGM1 promoter revealed that 1.6 kilobases, but not 1.1 kilobases, of DNA was sufficient to confer tissue-specific and cell layer-specific expression. This same region was responsible for reporter expression in tissues undergoing squamous metaplasia as a response to vitamin A deprivation. Mutation of a distal promoter AP1 site or proximal promoter CRE site, both identified as important transcriptional elements in transfection assays, did not prevent appropriate expression. Further searching for transcriptional elements using electrophoretic mobility shift (EMSA and transfection assays in cultured keratinocytes identified two Sp1 elements in a transcriptionally active region between -1.6 and -1.4 kilobases. While mutation of either Sp1 site or the AP1 site singly had only a small effect, mutation of all three sites eliminated nearly all the

  14. Dominant ELOVL1 mutation causes neurological disorder with ichthyotic keratoderma, spasticity, hypomyelination and dysmorphic features.

    Science.gov (United States)

    Kutkowska-Kaźmierczak, Anna; Rydzanicz, Małgorzata; Chlebowski, Aleksander; Kłosowska-Kosicka, Kamila; Mika, Adriana; Gruchota, Jakub; Jurkiewicz, Elżbieta; Kowalewski, Cezary; Pollak, Agnieszka; Stradomska, Teresa Joanna; Kmieć, Tomasz; Jakubowski, Rafał; Gasperowicz, Piotr; Walczak, Anna; Śladowski, Dariusz; Jankowska-Steifer, Ewa; Korniszewski, Lech; Kosińska, Joanna; Obersztyn, Ewa; Nowak, Wieslaw; Śledziński, Tomasz; Dziembowski, Andrzej; Płoski, Rafał

    2018-06-01

    Ichthyosis and neurological involvement occur in relatively few known Mendelian disorders caused by mutations in genes relevant both for epidermis and neural function. To identify the cause of a similar phenotype of ichthyotic keratoderma, spasticity, mild hypomyelination (on MRI) and dysmorphic features (IKSHD) observed in two unrelated paediatric probands without family history of disease. Whole exome sequencing was performed in both patients. The functional effect of prioritised variant in ELOVL1 (very-long-chain fatty acids (VLCFAs) elongase) was analysed by VLCFA profiling by gas chromatography-mass spectrometry in stably transfected HEK2932 cells and in cultured patient's fibroblasts. Probands shared novel heterozygous ELOVL1 p.Ser165Phe mutation (de novo in one family, while in the other family, father could not be tested). In transfected cells p.Ser165Phe: (1) reduced levels of FAs C24:0-C28:0 and C26:1 with the most pronounced effect for C26:0 (P=7.8×10 -6  vs HEK293 cells with wild type (wt) construct, no difference vs naïve HEK293) and (2) increased levels of C20:0 and C22:0 (P=6.3×10 -7 , P=1.2×10 -5 , for C20:0 and C22:0, respectively, comparison vs HEK293 cells with wt construct; P=2.2×10 -7 , P=1.9×10 -4 , respectively, comparison vs naïve HEK293). In skin fibroblasts, there was decrease of C26:1 (P=0.014), C28:0 (P=0.001) and increase of C20:0 (P=0.033) in the patient versus controls. There was a strong correlation (r=0.92, P=0.008) between the FAs profile of patient's fibroblasts and that of p.Ser165Phe transfected HEK293 cells. Serum levels of C20:0-C26:0 FAs were normal, but the C24:0/C22:0 ratio was decreased. The ELOVL1 p.Ser165Phe mutation is a likely cause of IKSHD. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  15. Síndrome de Chanarin-Dorfman Chanarin-Dorfman Syndrome

    Directory of Open Access Journals (Sweden)

    Sheila de Oliveira Garcia

    2010-01-01

    Full Text Available A síndrome de Chanarin-Dorfman é uma doença rara autossômica recessiva, que pode estar associada à ictiose, caracteriza-se pela presença de gotículas lipídicas intracelulares em células dos mais variados tecidos, especialmente nos queratinócitos e granulócitos. Mutações no gene ABHD5, que codifica uma proteína da esterase/lipase/subfamília tioesterase, têm sido identificadas como o principal motivo da desordem. As manifestações extracutâneas são heterogêneas, tanto em intensidade quanto em características. O envolvimento sistêmico pode incluir hepatoesplenomegalia, cataratas de dupla face, retardo de crescimento, miopatia, ataxia e perda auditiva neurossensorial bilateral.(1 Desde os primeiros relatos da síndrome,(1 apenas 30 pacientes foram descritos na literatura, a maioria proveniente de países do Oriente Médio.(2 O diagnóstico é confirmado pela presença de gotículas lipídicas em granulócitos de sangue periférico.(3Chanarin-Dorfman syndrome is a rare autosomal recessive disease that may be associated with ichthyosis and is characterized by the presence of intracellular lipid droplets in the cells of many different tissues, in particular in keratinocytes and granulocytes. Mutations in the gene that encodes the ABHD5 protein of the esterase/lipase/thioesterase subfamily have been identified as the main cause of the disorder. The extracutaneous manifestations are heterogeneous, both in intensity and characteristics. Systemic involvement may include hepatosplenomegaly, double-sided cataracts, growth retardation, myopathy, ataxia and bilateral sensorineural hearing loss.(1 Since the first reports of the syndrome (1 only 30 patients have been described in the literature, mostly from Middle Eastern countries.(2 The diagnosis is confirmed by the presence of lipid droplets in granulocytes of peripheral blood.(3

  16. Immune defects in families and patients with xeroderma pigmentosum and trichothiodystrophy.

    Science.gov (United States)

    Mariani, E; Facchini, A; Honorati, M C; Lalli, E; Berardesca, E; Ghetti, P; Marinoni, S; Nuzzo, F; Astaldi Ricotti, G C; Stefanini, M

    1992-01-01

    Xeroderma pigmentosum (XP) is a rare autosomal recessive disease characterized by photosensitivity, a high incidence of cancer in sun-exposed portions of the skin and a reduced capacity to repair the u.v.-induced DNA damage. One of the XP mutations (XP-D) has also been identified in patients affected by trichothiodystrophy (TTD), a rare autosomal recessive disease characterized by brittle hair, mental and physical retardation, peculiar face and ichthyosis. However, in these patients there is no evidence of increased skin tumour incidence. Since an impairment of cell-mediated immunity has been proposed as a co-factor in the cancer proneness of XP patients, we investigated the involvement of immune defect(s) in five XP patients, five TTD patients, their parents, and 24 TTD relatives. We evaluated the phenotype of circulating lymphocytes, natural killer (NK) cell lytic activity, target cell binding of NK cells at single cell level and the effect of interferons (IFN) alpha and beta on NK cell activity. The relative proportion of CD3+ and CD4+ circulating lymphocytes was reduced in XP but not in TTD patients. NK cell lytic activity was decreased in XP patients and their mothers, but their fathers showed normal lytic activity. NK activity varied among TTD families: four out of five patients and their relatives presented low NK cell activity, and one family was normal. In TTD family members, NK activity increased after incubation with IFN-alpha or IFN-beta, but never reached normal values. In contrast, in XP patients and their mothers, the defect was almost completely corrected after in vitro incubation with IFN-alpha or IFN-beta. Our study indicates impaired NK lytic activity in the majority of TTD and XP patients and that this defect is present also in members of their families. In addition, XP patients present a low number of circulating T cells. These multiple abnormalities, together with DNA repair defects, could be related to the increased cancer risk in XP patients

  17. Unified definition of a class of Monte Carlo estimators

    International Nuclear Information System (INIS)

    Lux, I.

    1978-01-01

    A unified definition of a wide class of Monte Carlo reaction rate estimators is presented, since most commonly used estimators belong to that class. The definition is given through an integral transformation of an arbitrary estimator of the class. Since the transformation contains an arbitrary function, in principle an infinite number of new estimators can be defined on the basis of one known estimator. It is shown that the most common estimators belonging to the class, such as the track-length and expectation estimators, are special cases of transformation, corresponding to the simplest transformation kernels when transforming the usual collision estimator. A pair of new estimators is defined and their variances are compared to the variance of the expectation estimator. One of the new estimators, called the trexpectation estimator, seems to be appropriate for flux-integral estimation in moderator regions. The other one, which uses an intermediate estimation of the final result and is therefore called the self-improving estimator, always yields a lower variance than the expectation estimator. As is shown, this estimator approximates well to possibly the best estimator of the class. Numerical results are presented for the simplest geometries, and these results indicate that for absorbers that are not too strong, in practical cases the standard deviation of the self-improving estimator is less than that of the expectation estimator by more than 10%. The experiments also suggest that the self-improving estimator is always superior to the track-length estimator as well, i.e., that it is the best of all known estimators belonging to the class. In the Appendices, for simplified cases, approximate conditions are given for which the trexpectation and track-length estimators show a higher efficiency than the expectation estimator

  18. The value of humor in psycotherapy

    Directory of Open Access Journals (Sweden)

    Liliana Chazenbalk

    2015-09-01

    Full Text Available The main point of this work is to show how humor—when is used properly- has a positive effect on the patient ant the therapist’s self improving their alliance, supervision and team meetings. It’s also good for profesionals to include humor, recreation and rest in their job routines, because these ingredients let them be more effective in psychoterapy. The vast majority of psychological theories agree on its importance. The Cognitive Psychology believes that humor helps the patient evaluate reality in a different way, stops disfunctional thoughts, lets the expression of negative emotions and finds more adaptative patterns. 

  19. Annotated Bibliography of the Air Force Human Resources Laboratory Technical Reports - 1978

    Science.gov (United States)

    1980-06-01

    IF ACCESION for NTIS Wh ite SectionW DOC Buff Sectio 0I uNANNOUNED E JUSTIFICATION7 7p -sAir~ mPU -~~ _ ___ _- ANNTATD BBLIGRAHY F TE AR FRCEIIUMA...Efforts were made to develop the most economical composite from the standpoint of number of data items required while retaining the maximum amount of...decisions included such job aspects as work itself. promotion chances. economic securit , and self-improvement opportunities. Of the I2 factors with high

  20. Atheist spirituality: a follow on from New Atheism?

    OpenAIRE

    Teemu Taira

    2012-01-01

    Books about well-being, self-improvement, life management and spirituality have been popular for many years. It is not news to anybody that such topics sell. However, books on atheism have never become bestsellers until the early years of the twenty-first century. Now the so-called New Atheist books have altogether sold millions. It may sound surprising, but atheism sells. It may have been the idea of a publishers’ marketing department to put the two selling points together, but in recent yea...

  1. Expert system for quality control in the INIS database

    International Nuclear Information System (INIS)

    Todeschini, C.; Tolstenkov, A.

    1990-05-01

    An expert system developed to identify input items to INIS database with a high probability of containing errors is described. The system employs a Knowledge Base constructed by the interpretation of a large number of intellectual choices or expert decisions made by human indexers and incorporated in the INIS database. On the basis of the descriptor indexing, the system checks the correctness of the categorization. A notable feature of the system is its capability of self improvement by the continuous updating of the Knowledge Base. The expert system has also been found to be extremely useful in identifying documents with poor indexing. 3 refs, 9 figs

  2. Activity know-how and doctrine of QC circle

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1976-09-15

    This books introduces activity know-how of QC circle giving descriptions of basic of QC circle activities, introduction operation and development and mind of QC circle activities, method for beginning of QC circle activity like, way order, motivation of introduction of QC circle activity, propel method of QC circle activities, such as leadership, brain storming, and rule of QC circle activity, management and propel method for improvement, development of QC circle activities. It also deals with doctrine of basic of QC circle, purpose, self improvement and group activity.

  3. Transformative Possibilities: Politics and Cosmetic Surgery in the Bolivarian Revolution.

    Science.gov (United States)

    Gulbas, Lauren E

    2017-10-01

    I examine the intersection of politics and aesthetics in a public hospital in Caracas, Venezuela in the first years of the twenty-first century. Given Venezuela's long-standing embrace of physical enhancement and the contradictions of the medical values of cosmetic surgery with those of Bolivarian socialism, the changing surgical practices at a well-established public site offer a significant case for considering how different actors negotiate the dialectics of care. In the face of increasing resource shortages, negotiations of aesthetic care contributed to tensions in the clinical encounter as patients creatively pushed Bolivarian policies to support their pursuits of aesthetic self-improvement.

  4. Activity know-how and doctrine of QC circle

    International Nuclear Information System (INIS)

    1976-09-01

    This books introduces activity know-how of QC circle giving descriptions of basic of QC circle activities, introduction operation and development and mind of QC circle activities, method for beginning of QC circle activity like, way order, motivation of introduction of QC circle activity, propel method of QC circle activities, such as leadership, brain storming, and rule of QC circle activity, management and propel method for improvement, development of QC circle activities. It also deals with doctrine of basic of QC circle, purpose, self improvement and group activity.

  5. Common issues found in operating safety peer review of nuclear power plants

    International Nuclear Information System (INIS)

    Wu Meijing; Zhang Fengping

    2004-01-01

    The 3rd stage of the safety culture promotion in a Nuclear Power Plant (NPP) is characterized by establishing learning organization and continuous self-improvement. Peer Review was used as an effective tool by a lot of NPPs to improve the overall management and performance. This Paper provided the WANO Peer Review Methodology, the common issues found, the recommendation or suggestions to correct the area for improvement. It may be beneficial to other NPP which planning to have Peer Review or Self Evaluation. (authors)

  6. Performance success from self-assessments

    International Nuclear Information System (INIS)

    Hughes, G.

    1996-01-01

    This paper provides information on and successes of self-assessments completed at the Callaway plant. It stresses the vital needs and substantial benefits from these continual self-improvement efforts. The Callaway plant staff use a variety of methods and techniques to do self-assessments with the focus on performance outcomes. Self-assessments help Callaway focus on the priority issues and direct resources properly. Callaway's success has been due to this learning culture. The Callaway plant is a Westinghouse four-loop plant in the state of Missouri and has been operational since 1984

  7. Expert system for quality control in the INIS database

    Energy Technology Data Exchange (ETDEWEB)

    Todeschini, C; Tolstenkov, A [International Atomic Energy Agency, Vienna (Austria)

    1990-05-01

    An expert system developed to identify input items to INIS database with a high probability of containing errors is described. The system employs a Knowledge Base constructed by the interpretation of a large number of intellectual choices or expert decisions made by human indexers and incorporated in the INIS database. On the basis of the descriptor indexing, the system checks the correctness of the categorization. A notable feature of the system is its capability of self improvement by the continuous updating of the Knowledge Base. The expert system has also been found to be extremely useful in identifying documents with poor indexing. 3 refs, 9 figs.

  8. NRC's experiment with plant personnel training: the acid test of self-regulation

    International Nuclear Information System (INIS)

    Reynolds, N.S.

    1985-01-01

    In February 1985, the US Nuclear Regulatory Commission (NRC) initiated an experiment with a form of nuclear utility self-regulation. The commissioners unanimously endorsed the nuclear utility industry's commitment to achieve self-improvement voluntarily in the area of training and qualification of nuclear plant personnel, and accepted that commitment as a basis for deferring rulemaking. In taking this action, the Commission may have signaled a marked departure from the post-Three Mile Island (TMI) era of prescriptive (and occasionally pedantic) regulatory practices to a new era of increased cooperation with nuclear utilities

  9. Perceptions of an international interprofessional education experience

    DEFF Research Database (Denmark)

    Macauley, Kelly; Skov, Hanne; Lethonen, Krista

    2016-01-01

    , international teams and apply didactic knowledge to community problems using the innovation process. The purpose of this article is to describestudent perceptions across 4 years of programme implementation. Through analysis of closed-ended survey data, we found that students who participated in the programme......-ended questions revealed five themes: language, teamwork andcollaboration, cultural awareness, innovation process, and personal growth and self-improvement. The longterm,follow-up data indicate that these attributes can be transferred into a clinical context which haveimplications for improved collaboration...

  10. THE AUTONOMOUS MAINTENANCE IMPLEMENTATION DIRECTORY AS A STEP TOWARD THE INTELLIGENT QUALITY MANAGEMENT SYSTEM

    Directory of Open Access Journals (Sweden)

    Michał MOLENDA

    2016-10-01

    Full Text Available The article describes the effects of the improvement of the production process which one of the industrial enterprises obtained by implementing the method of Autonomous Maintenance (AM, which is one of the pillars of the concept of Total Productive Maintenance (TPM. AM method was presented as an aid to the formation of intelligent, self-improving procesess of the quality management system (QMS. The main part of this article is to present results of studies that have been conducted in one of the large industrial enterprises in Poland, manufacturing for the automotive industry. The aim of the study was to evaluate the effectiveness of the implementation of the AM method as a tool for self-improvement of industrial processes in the following company. The study was conducted in 2015. The gathering and comparison of data from the period of two years, ie. the year before and the year after the implementation of AM, helped to determine the effectiveness of AM in building intelligent quality management system.

  11. [Career consciousness among Japanese female students: relationships between vocational motivation and career exploration].

    Science.gov (United States)

    Adachi, Tomoko

    2008-04-01

    This questionnaire study investigated career consciousness among 348 Japanese female freshman and sophomore college students. Variables included career consciousness (belief in the idea of a best-fit vocation, passivity, and giving priority to personal interests), vocational motives (self-improvement, interpersonal, status), as well as career decision-making self-efficacy (self-appraisal, gathering occupational information) and career exploration (self and environmental exploration). The results of causal analyses showed that the belief in the idea of a best-fit vocation and passivity had effects on all three vocational motives, but giving priority to personal interests had an effect only on motivation for self-improvement. Analysis of covariance (ANCOVA) models were constructed with career exploration as dependent variables, career consciousness as fixed factors, and career decision-making self-efficacy as covariates. The results of the analyses showed that students who had high career decision-making self-efficacy engaged in both self exploration and environmental exploration activities with a higher frequency. Students with a greater passive tendency toward their career engaged in both self and environmental exploration with a lower frequency. Belief in the idea of a best-fit vocation was associated only with differences in frequency of self exploration. Giving priority to personal interests did not produce differences in career exploration activities.

  12. Measurement of self-evaluative motives: a shopping scenario.

    Science.gov (United States)

    Wajda, Theresa A; Kolbe, Richard; Hu, Michael Y; Cui, Annie Peng

    2008-08-01

    To develop measures of consumers' self-evaluative motives of Self-verification, Self-enhancement, and Self-improvement within the context of a mall shopping environment, an initial set of 49 items was generated by conducting three focus-group sessions. These items were subsequently converted into shopping-dependent motive statements. 250 undergraduate college students responded on a 7-point scale to each statement as these related to the acquisition of recent personal shopping goods. An exploratory factor analysis yielded five factors, accounting for 57.7% of the variance, three of which corresponded to the Self-verification motive (five items), Self-enhancement motive (three items), and Self-improvement motive (six items). These 14 items, along with 9 reconstructed items, yielded 23 items retained and subjected to additional testing. In a final round of data collection, 169 college students provided data for exploratory factor analysis. 11 items were used in confirmatory factor analysis. Analysis indicated that the 11-item scale adequately captured measures of the three self-evaluative motives. However, further data reduction produced a 9-item scale with marked improvement in statistical fit over the 11-item scale.

  13. Cutaneous manifestations of internal malignancies in a tertiary health care hospital of a developing country Manifestações cutâneas de doenças malignas em um hospital terciário de um país em desenvolvimento

    Directory of Open Access Journals (Sweden)

    Alex G Ortega-Loayza

    2010-10-01

    Full Text Available In a public hospital in Lima, Peru, 24 patients with 16 types of paraneoplastic dermatoses were identified by data collection. The most frequent dermatosis was dermatomyositis (four patients. The other dermatoses were malignant acanthosis nigricans, palmoplantar keratoderma, bullous dermatoses, lymphomatoid papulosis, edematous scarring vasculitic panniculitis, Norwegian scabies, primary systemic amyloidosis, necrolytic migratory erythema, infective dermatitis, pancreatic panniculitis, generalized pruritus, Lesser-Trelat syndrome, and acquired ichthyosis. Most of these paraneoplastic dermatoses were diagnosed before (45.8% or at the time of (38.5% the diagnosis of the underlying malignancy. The most frequent underlying malignancies were lymphoma, adenocarcinomas of the upper digestive tract, and malignant neoplasms of the pancreas. The average age of the patients was 47.0 ± 16.9 years and the length of the disease since diagnosis was 13.7 months. The mortality rate was 75%. Paraneoplastic dermatoses are rare dermatologic entities that are difficult to diagnose. Surveillance is also hampered when patients do not have easy access to health care centers due to financial and geographical issues. However, when identified, they might facilitate the early diagnosis of an associated tumor and contribute to increase the surveillance of patients.Em um hospital público em Lima, Peru, 24 pacientes com 16 tipos de dermatoses paraneoplásicas foram identificados por meio de coleta de dados. A dermatose mais frequente foi dermatomiosite (quatro pacientes. As outras dermatoses foram acantose maligna, queratodermia palmoplantar, dermatoses bolhosas, papulose linfomatóide, cicatriz edematosa, paniculite e vasculite, escabiose norueguesa, amiloidose sistêmica primária, eritema necrolítico migratório, dermatite infecciosa, paniculite pancreática, prurido generalizado, sinal de Leser-Trelat e ictiose adquirida. Grande parte dessas dermatoses foi

  14. Diseases associated with hidranitis suppurativa: part 2 of a series on hidradenitis.

    Science.gov (United States)

    Scheinfeld, Noah

    2013-06-15

    Hidradenitis suppurativa (HS), a pathologic follicular disease, impacts patients' lives profoundly and usually occurs in isolation. The diseases with the strongest association are obesity, depression, and pain. HS is associated with many diseases including acne conglobata (AC), dissecting cellulitis, pilonidal cysts, and obesity. Pyoderma fistulans sinifica (fox den disease) appears to be the same entity as Hurley Stage 2 of 3 HS. The rate of acne vulgaris in HS patients mirrors unaffected controls. The most common, albeit still uncommon, association is with seronegative, haplotype unlinked arthritis (most importantly B27), in particular spondolyarthritis. Crohn disease and HS occur together at a rate that varies from 0.6% to 38% in retrospective cases series. Ulcerative colitis occurred with HS in 14% of patients in one series. The next most common association is with pyoderma gangrenosum, but this association is likely under-reported. Synovitis-Acne-Pustulosis Hyperostosis-Osteitis (SAPHO) syndrome, which is rare, has more than 10 reports linking it to HS. Nine case reports have linked Dowling-Degos disease (DDD) to HS and two reports related HS to Fox-Fordyce disease (FF), but because both occur in the axilla this might be a mere coincidence. HS is rarely associated with ophthalmic pathology. Specifically, more than 5 reports link it to Keratitis-Ichthyosis-Deafness syndrome (KID); greater than10 cases link it to interstitial keratitis and 2 cases are linked to Behçet's disease. The presence of proteinuria and acute nephritis link HS to the kidney, especially since and reports have documented resolution of HS after renal transplant. Florid steatocystoma multiplex, Sjogren Syndrome, and HS have been linked and their reports likely underestimate their coincidence because all these entities involve occlusion (albeit by different mechanisms). Three reports link HS and amyloid, but both share some common genetic underpinnings and thus the coincidence of these

  15. [Current situation of acupuncture in New Zealand].

    Science.gov (United States)

    Li, Xiaoji; Hu, Youping

    2017-04-12

    The beginning of TCM acupuncture in New Zealand dates back to the middle of 19th century. After self-improvement for more than 100 years, TCM acupuncture has gained a considerable development. From the perspective of history and current situation, the development of acupuncture in New Zealand was elaborated in this article; in addition, the sustainable development of acupuncture was discussed from the perspective of education and training. In New Zealand, the TCM acupuncture and dry needling have played a dominant role in acupuncture treatments, which are practiced by TCM practitioners and physical therapists. The TCM acupuncture is widely applied in department of internal medicine, surgery, gynecology, and pediatrics, etc., while the dry needling is li-mited for traumatology and pain disorder. Therefore, including TCM acupuncture into the public medical and educational system in New Zealand should be an essential policy of Ministry of Health to provide welfare for the people.

  16. Intrinsic motivation towards sports in Singaporean students: the role of sport ability beliefs.

    Science.gov (United States)

    Wang, C K John; Biddle, Stuart J H

    2003-09-01

    This study investigated determinants of active lifestyles in Singaporean university students. Using confirmatory factor analysis, a measure of lay beliefs concerning athletic ability was confirmed. Other results confirmed hypotheses that beliefs reflecting that athletic ability can be developed over time (incremental beliefs) predict an achievement task (self-referenced) orientation, while beliefs reflecting that athletic ability is relatively stable (entity beliefs) predict an ego (other-person, comparative) orientation. Goal orientations directly affect perceived competence which, in turn, influence intrinsic motivation to be physically active. A task orientation had a direct link to intrinsic motivation. Results suggest that intrinsic motivation towards sport and physical activity might be enhanced through interventions that focus on self-referenced and self-improvement notions of ability as well as perceived competence.

  17. Domains of unprofessional behavior during medical school associated with future disciplinary action by a state medical board.

    Science.gov (United States)

    Teherani, Arianne; Hodgson, Carol S; Banach, Mary; Papadakis, Maxine A

    2005-10-01

    In a previous study, we showed that unprofessional behavior in medical school was associated with subsequent disciplinary action. This study expands on that work by identifying the domains of unprofessional behavior that are most problematic. In this retrospective case-control study, negative comments were extracted from student files for 68 case (disciplined) and 196 matched control (nondisciplined) physicians. Comments were analyzed qualitatively and subsequently quantified. The relationship between domains of behavior and disciplinary action was established through chi-square tests and multivariate analysis of variance. Three domains of unprofessional behavior emerged that were related significantly to later disciplinary outcome: (1) poor reliability and responsibility, (2) lack of self-improvement and adaptability, and (3) poor initiative and motivation. Three critical domains of professionalism associated with future disciplinary action have been defined. These findings could lead to focused remediation strategies and policy decisions.

  18. Demand for engineering manpower at US nuclear utilities, 1987-1997

    International Nuclear Information System (INIS)

    Poling, D.Y.

    1988-01-01

    The Institute of Nuclear Power Operations (INPO), organized in December 1979, is an independent undertaking in self-improvement by the US nuclear utilities. INPO has conducted manpower surveys each year since 1981. The survey is designed to determine current employment, vacancies, turnover, and other employment-related matters at the 54 US electric utilities that operate or are constructing nuclear power plants. It also provides 10-yr projections of nuclear manpower demand at the utilities and current and 1-yr projections of employment opportunities for new engineering and science graduates. It should be noted that the data reported in this paper do not include nonutility employment; utility employment constitutes approximately one-third of the civilian nuclear work force as reported by the US Department of Energy

  19. Tell me the gossip: the self-evaluative function of receiving gossip about others.

    Science.gov (United States)

    Martinescu, Elena; Janssen, Onne; Nijstad, Bernard A

    2014-12-01

    We investigate the self-evaluative function of competence-related gossip for individuals who receive it. Using the Self-Concept Enhancing Tactician (SCENT) model, we propose that individuals use evaluative information about others (i.e., gossip) to improve, promote, and protect themselves. Results of a critical incident study and an experimental study showed that positive gossip had higher self-improvement value than negative gossip, whereas negative gossip had higher self-promotion value and raised higher self-protection concerns than positive gossip. Self-promotion mediated the relationship between gossip valence and pride, while self-protection mediated the relationship between gossip valence and fear, although the latter mediated relationship emerged for receivers with mastery goals rather than performance goals. These results suggest that gossip serves self-evaluative functions for gossip receivers and triggers self-conscious emotions. © 2014 by the Society for Personality and Social Psychology, Inc.

  20. Defensiveness versus remediation: self-theories and modes of self-esteem maintenance.

    Science.gov (United States)

    Nussbaum, A David; Dweck, Carol S

    2008-05-01

    How people maintain and repair their self-esteem has been a topic of widespread interest. In this article, the authors ask, What determines whether people will use direct, remedial actions, or defensive actions? In three studies, they tested the hypothesis that a belief in fixed intelligence (entity theory) would produce defensiveness, whereas a belief in improvable intelligence (incremental theory) would foster remediation. In each study, participants assigned to the entity condition opted for defensive self-esteem repair (downward comparison in Studies 1 and 3; a tutorial on already mastered material in Study 2), but those in the incremental condition opted for self-improvement (upward comparison in Studies 1 and 3; a tutorial on unmastered material in Study 2). Experiment 3 also linked these strategies to self-esteem repair; remedial strategies were the most effective in recovering lost self-esteem for those in the incremental condition, whereas defensive strategies were most effective for those in the entity condition.

  1. Virtue ethics, positive psychology, and a new model of science and engineering ethics education.

    Science.gov (United States)

    Han, Hyemin

    2015-04-01

    This essay develops a new conceptual framework of science and engineering ethics education based on virtue ethics and positive psychology. Virtue ethicists and positive psychologists have argued that current rule-based moral philosophy, psychology, and education cannot effectively promote students' moral motivation for actual moral behavior and may even lead to negative outcomes, such as moral schizophrenia. They have suggested that their own theoretical framework of virtue ethics and positive psychology can contribute to the effective promotion of motivation for self-improvement by connecting the notion of morality and eudaimonic happiness. Thus this essay attempts to apply virtue ethics and positive psychology to science and engineering ethics education and to develop a new conceptual framework for more effective education. In addition to the conceptual-level work, this essay suggests two possible educational methods: moral modeling and involvement in actual moral activity in science and engineering ethics classes, based on the conceptual framework.

  2. Obstacles for physical education teachers in public schools: an unsustainable situation

    Directory of Open Access Journals (Sweden)

    Renata Osborne

    Full Text Available Abstract The study aimed to identify difficulties and aspirations of physical education teachers at public schools in Niterói, inspired by UNESCO's quality physical education goal. An action research containing quantitative and qualitative data was conducted. Thirty-five physical education teachers completed a questionnaire and seven teachers were interviewed. The results indicated that the major difficulties faced were low wages, precarious infrastructure and lack of materials. Physical education is devalued, the space allocated is inadequate, and it is treated as mere recreation. Teachers criticized the lack of commitment of some colleagues who work without planning. They also complained about undisciplined students and lack of interest from their families. They aspire to self-improvement, infrastructure improvements, and more support from school and families. Teachers who do not educate and lack of support from school and government are an unsustainable reality. A synergy of efforts should be implemented, based on a systems view.

  3. Using high-level construal and perceptions of changeability to promote self-change over self-protection motives in response to negative feedback.

    Science.gov (United States)

    Belding, Jennifer N; Naufel, Karen Z; Fujita, Kentaro

    2015-06-01

    Diagnostic negative information presents people with a motivational dilemma. Although negative feedback can provide useful information with which to guide future self-improvement efforts, it also presents short-term affective costs. We propose that construal level, jointly with the perceived changeability of the feedback domain, determines whether people choose to accept or dismiss such information. Whereas low-level construal promotes short-term self-protection motivation (promoting dismissal), high-level construal promotes long-term self-change motivation (promoting acceptance)--to the extent that change is perceived as possible. Four studies support this hypothesis and examine underlying cognitive and motivational mechanisms. The present work may provide an integrative theoretical framework for understanding when people will be open to and accept negative diagnostic information, and has important practical implications for promoting self-change efforts. © 2015 by the Society for Personality and Social Psychology, Inc.

  4. THE USE OF E-PORTFOLIO TO DEVELOP ENGLISH LANGUAGE LEARNERS' AUTONOMY AND INDEPENDENCE

    Directory of Open Access Journals (Sweden)

    Oksana I. Ivanova

    2017-09-01

    Full Text Available This paper discusses the findings of a research study concerning the use of e-portfolios to develop learners' autonomy and independence, from the perspectives of teachers and students participating in this study. The findings demonstrate many of the benefits of e-portfolio practice regarding learners’ increased sense of ownership, teacher and peer feedback, enriched learning experience at both individual and technological levels, enhanced opportunity for self-improvement and increased awareness of the learning process. Despite many positive aspects of e-portfolios use, the study reveals some challenges facing students, teachers and support staff, mainly connected with technical problems and Internet access, and the necessity for teachers and learners to change their roles to become more independent in the learning process. The author concludes that e-portfolio is a promising tool to stimulate and challenge students to become independent and self-regulated learners that should be implemented in English language learning at higher educational institutions.

  5. Participation in Leisure Activities by Children and Adolescents with Juvenile Idiopathic Arthritis.

    Science.gov (United States)

    Cavallo, Sabrina; Majnemer, Annette; Duffy, Ciarán M; Feldman, Debbie Ehrmann

    2015-09-01

    To describe leisure activities of children and adolescents with juvenile idiopathic arthritis (JIA) in terms of diversity, intensity, and enjoyment, and to identify potential determinants. One hundred seven children and adolescents aged 8-17 years diagnosed with JIA and their families participated in this cross-sectional study. Participants answered the Children's Assessment of Participation and Enjoyment, which measures involvement in leisure (recreation, active physical, social, skill-based, self-improvement). Disease characteristics and sociodemographic factors were abstracted from the child's medical file. In terms of intensity, individuals with JIA were more often engaged in informal rather than formal leisure activities [t(106) = 45.5, p leisure activities in children and adolescents with arthritis may allow healthcare professionals to assess children's health needs with more precision and promote a healthier lifestyle.

  6. Happiness and unhappiness in east and west: themes and variations.

    Science.gov (United States)

    Uchida, Yukiko; Kitayama, Shinobu

    2009-08-01

    Cultural folk models of happiness and unhappiness are likely to have important bearings on social cognition and social behavior. At present, however, little is known about the nature of these models. Here, the authors systematically analyzed American and Japanese participants' spontaneously produced descriptions of the two emotions and observed, as predicted, that whereas Americans associated positive hedonic experience of happiness with personal achievement, Japanese associated it with social harmony. Furthermore, Japanese were more likely than Americans to mention both social disruption and transcendental reappraisal as features of happiness. As also predicted, unlike happiness, descriptions of unhappiness included various culture-specific coping actions: Whereas Americans focused on externalizing behavior (e.g., anger and aggression), Japanese highlighted transcendental reappraisal and self-improvement. Implications for research on culture and emotion are discussed. 2009 APA, all rights reserved.

  7. THE IMPLEMENTATION OF GOOGLE SERVICES FOR TEACHING GENERATION Z STUDENTS

    Directory of Open Access Journals (Sweden)

    Ivanna I. Podik

    2017-09-01

    Full Text Available The article describes the features of modern students from the point of view of the Generations' Theory. It is established that the dependence of students Z on digital devices and the global Internet network affects the organization of the educational process and necessitates the introduction of electronic educational resources. The possibilities of using the G Suite for Education (Gmail, Google Drive, Google Forms, Google Documents, Google Spreadsheets, etc. in the learning process are analyzed, and the experience of their implementation in the study of the Bookkeeping has been described. It is argued that the use of information products by Google participants in the learning process provides an opportunity to raise the level of cognitive activity of future accountants, as well as a powerful motivational tool for person-oriented learning and self-improvement of students.

  8. The Digitisation of Welfare – a Strategy towards improving Citizens’ Self-care and Co-management of Welfare

    DEFF Research Database (Denmark)

    Thualagant, Nicole; From, Ditte-Marie

    2018-01-01

    (SKAD) analysis, this chapter scrutinizes central documents on the strategy of digital welfare. Our exploration provides a critical insight in the current digitisation of health care by illustrating how new virtues of citizenship are demanded in the digital era in relation to digital health......Government strategy of Digital Welfare 2016-2020 in regards to health and discusses how this strategy encourages self-measurement and self-improvement through discourses of improvement at both state and citizen levels. By illustrating how performativity is embedded in current conceptions of health, this chapter...... emphasize how strategies of digitisation lean on a bio-citizenship where individuals with poor health capacities become depended, not on a supporting welfare system, but paradoxically on own self-management skills in order to receive health services. Based on the sociology of knowledge approach to discourse...

  9. Maintaining Vitality: Pharmacists' Continuing Professional Education Decision-Making in the Upper Midwest.

    Science.gov (United States)

    Henkel, Paul Jacob; Marvanova, Marketa

    2018-02-01

    Continuing professional education (CPE) plays an important role in continuing professional development of pharmacists for providing quality pharmaceutical care but also to maintain professional and organizational vitality and meet changing community/population needs. The study objective was to describe and understand factors of importance in selection of CPE credit hours among Upper Midwest pharmacists. A cross-sectional study of licensed pharmacists ( n = 1239) in Iowa, Minnesota, Nebraska, North Dakota, and South Dakota included completion of a questionnaire on demographics and CPE decision-making. Factor analysis, t -test, and multivariate analyses were performed using Stata 10.1. Pharmacists placed greatest importance on maintaining licensure (mean = 2.72/3.00), personal interest (mean = 2.57), and self-improvement (mean = 2.42). Community/population need (mean = 1.83) was rated as slightly more important ( p market, but more importantly to ensure continued provision of quality pharmaceutical care and patient education.

  10. A Teenager’s reϐlection on formation of the value orientations in the family and at school

    Directory of Open Access Journals (Sweden)

    VILIJA GRINCEVIČIENĖ

    2017-10-01

    Full Text Available Family and school – the most important social institutions in which the young generation is prepared to live in tomorrow’s society. Analysis of the research revealed that in the period of age from 14 to 19 the most important values is considered as: empathy, perfection, justice, self-improvement, self-esteem, security, acceptance, courage, knowledge, creativity, competitiveness, friendship, cooperation, responsibility and altruism. Family, which successfully trains and educates such values as security, justice, altruism, empathy, self-esteem, enable the young generation to develop social competence. Th e main values conveyed in the school- competitiveness, education, cooperation, friendship, recognition. Looking at the situation from a range of competencies, a school distinguished by communication, learning to learn skills and personal development. A closer interaction between school and family accelerate the positive socialization process of young generation and guarantee the further development of the formation process of value orientations (personality development.

  11. Innovative conditions of professionally applied training for maritime-students.

    Directory of Open Access Journals (Sweden)

    Podlesny A.I.

    2011-01-01

    Full Text Available The author considers organizational and methodological terms of implementation of professional and applied physical training for maritime students subject to their motivation to physical self-perfection. The purpose of the research is to define organizational and pedagogical terms for professional and applied physical training of maritime students to improve their physical condition and special physical attainment. The applied methods were: anthropometric metrology, functional probes, tonometry, pulsometry, motion tests and mathematical analysis. 70 students of 17-18 years participated in the research. It was determined that organizational and pedagogical terms directed on acceleration of making necessary for students to self-improve physically, positively impact on development of special physical state that are fundamental for professional activities of maritime students.

  12. Self image and place of control

    Directory of Open Access Journals (Sweden)

    Marica Mircea Adrian

    2017-05-01

    Full Text Available The problematic of the self has a long history, being analyzed in the space of philosophical rationality since the Greek antiquity, being directed by the Socratic imperative of self-knowledge. Together with the establishment and development of social psychology, the last century, the researches on self-meet a special amplification. In the last decades, the thematic of self and its dimensions met a remarkable effervescent theoretics. The psychological researches extended on self-consciousness, self-esteem, self-handicapping, self-efficacy, self-improvement, self-enhancement, self-monitoring, self-focusing, self-disclosure, self-verification, etc. Our research aims to investigate the correlations that exist between the self-esteem and place of control, having in view certain variables such as age, sex, the level of studies graduated. The results obtained confirm in a great extent other researches mentioned in the paper.

  13. Health, physical education and physical development of students in historically and personally developing paradigm

    Directory of Open Access Journals (Sweden)

    S.I. Belykh

    2013-05-01

    Full Text Available It is considered the general health situation of the population of Europe. It is shown that in the last decade in the European Union there is a steady trend towards better health. It is noted that in the countries of the former Soviet Union, including Ukraine, the reverse process. Revealed the possibility of improved system of physical education students in improving indicators of physical development, functional status and health of students and the general population. It is noted that the intense physical activity of young people is only 3 - 4 minutes per day, and moderate and total - just over 1 hour. It is noted that the enhancement of the educational component of university discipline Physical Education is defined professional and personal characteristics of teachers of physical education departments, their willingness to self-improvement and development. The prospects for the use of personal-oriented campaign in the reform of university academic discipline Physical Education.

  14. Economic analysis of needs the training of human resources

    Directory of Open Access Journals (Sweden)

    Buha Vesna V.

    2015-01-01

    Full Text Available The issue of lifelong learning is linked to the voluntary and motivated to seek knowledge from personal or organizational reasons. The fact that an individual learns driven by personal career goals, desire for self improvement and motivation refers to the importance of lifelong learning for the entire social inclusion, sustainability, and competitiveness and employment. The common denominator of all the problems of investment in education is an issue of increasing allocations to the social issues, the pace that in this area manifest needs. Relative resource constraints requires that the investment in professional development taking place in accordance with the expected contribution to the creation of new value, increase productivity and social development of society as a whole. Respecting this request, in general, should provide maximum socio-economic effects with minimal investment. Precise measurement is achieved by tools of economic analysis: cost & benefit, economic sensitivity analysis, risk assessment.

  15. Film jako komunikat w terapii logopedycznej

    Directory of Open Access Journals (Sweden)

    Jagoda Cieszyńska-Rożek

    2017-07-01

    Full Text Available The widespread access to devices, which capture audiovisual information for a given amount of time, has served as form of communication between therapists (has served as a source of information for the therapy. In neurologopedic therapy (in speech therapy, a movie serves as a report regarding the level of cognitive functions and language acquisition. Additionally, it ensures that the therapist controls their statements and program the language. A movie allows the verification of therapy plans and the documentation of the therapy. Authors of this article emphasize this specific way of collecting data for the self-improvement of specialists. With this method, therapists can analyze their own reactions and behaviors, evaluate their effectiveness and relevance, and make revisions if necessary. Movies are also a valuable resource for parents. Narrative movies that describe the stories of individuals with developmental disorders are incredible source of information for the mass population.

  16. Intelligent use of knowledge in the EUM system

    International Nuclear Information System (INIS)

    Starynkevitch, B.

    1987-05-01

    A system accepting truely descriptive knowledge should receive (on build) meta-knowledge for using knowledge. The EUM system, in development, described in this paper, accepts such a knowledge. On the bottom level, it is a programming language (or a virtual machine) designed to be able to self-improve by introspection. Upper levels use meta-knowledge. EUM can access all objects its uses, it executes orders by using explicit methods. These can be meta-expertises. Hence, EUM can reason about its own operation, and cleverly use its knowledge and methods. Interaction with user is done by orders; thus it can be expertly driven. The initial inference engine is made of modules which can be replaced or completed by meta-expertises, of which some partial examples are given. Hence, it is possible to extend the knowledge langage, and the inference mechanism, by meta-expertises. In the same way, knowledge bases can be expertly compiled into orders [fr

  17. Factors affecting occupational therapists' decision to join their regional professional association: Facteurs influençant la décision des ergothérapeutes de joindre les rangs de leur association professionnelle régionale.

    Science.gov (United States)

    Reyes, Angelica N; Brown, Cary A

    2018-01-01

    Voluntary occupational therapy organizations fill an important role. However, recruitment and retention can be problematic. Little is known about factors influencing occupational therapists to join/maintain membership in professional associations. This study investigated factors influencing occupational therapists' decision to join/remain members of their association. An electronic survey was carried out and data were analyzed using SPSS software and manual categorization of open-ended comments. Two hundred and fifty-four therapists responded. Generation of new ideas, opportunities for professional development, self-improvement, maintenance of standards, improvement of the profession, and discounts on equipment/educational opportunities were significant factors in deciding to join the organization. The factors perceived as priorities varied in relation to participants' year of graduation. More-customized strategies, reflecting priorities that vary during a therapist's career path, may need to be employed to best address recruitment and retention across the range of therapists' needs and goals.

  18. Faculty development: if you build it, they will come.

    Science.gov (United States)

    Steinert, Yvonne; Macdonald, Mary Ellen; Boillat, Miriam; Elizov, Michelle; Meterissian, Sarkis; Razack, Saleem; Ouellet, Marie-Noel; McLeod, Peter J

    2010-09-01

    The goals of this study were three-fold: to explore the reasons why some clinical teachers regularly attend centralised faculty development activities; to compare their responses with those of colleagues who do not attend, and to learn how we can make faculty development programmes more pertinent to teachers' needs. In 2008-2009, we conducted focus groups with 23 clinical teachers who had participated in faculty development activities on a regular basis in order to ascertain their perceptions of faculty development, reasons for participation, and perceived barriers against involvement. Thematic analysis and research team consensus guided the data interpretation. Reasons for regular participation included the perceptions that: faculty development enables personal and professional growth; learning and self-improvement are valued; workshop topics are viewed as relevant to teachers' needs; the opportunity to network with colleagues is appreciated, and initial positive experiences promote ongoing involvement. Barriers against participation mirrored those cited by non-attendees in an earlier study (e.g. volume of work, lack of time, logistical factors), but did not prevent participation. Suggestions for increasing participation included introducing a 'buddy system' for junior faculty members, an orientation workshop for new staff, and increased role-modelling and mentorship. The conceptualisation of faculty development as a means to achieve specific objectives and the desire for relevant programming that addresses current needs (i.e., expectancies), together with an appreciation of learning, self-improvement and networking with colleagues (i.e., values), were highlighted as reasons for participation by regular attendees. Medical educators should consider these 'lessons learned' in the design and delivery of faculty development offerings. They should also continue to explore the notion of faculty development as a social practice and the application of motivational

  19. Prudence, pleasure, and cognitive ageing: Configurations of the uses and users of brain training games within UK media, 2005-2015.

    Science.gov (United States)

    Pickersgill, Martyn; Broer, Tineke; Cunningham-Burley, Sarah; Deary, Ian

    2017-08-01

    The use of 'brain training' games is often regarded as relating to wider ideals of self-improvement and youthfulness. Hence, use is intertwined with discourses of 'active' ageing. This paper analyzes how the use and users of brain training games were configured in the UK media, from 2005 to 2015, and examines how notions of active ageing relate to these representations. Game users were rarely constructed solely as gamers, and were more often presented as prudent individuals focused on a serious goal. This configuration related to assumed and enjoined motivations for brain training; specifically, users were commonly framed as seeking to enhance cognition and limit/delay cognitive decline. Scientific evidence about brain training was often deployed to explain how games might work; sometimes, however, it was used to undermine the utility of games and assert the significance and cognitive health-benefits of other activities. A minority of texts explicitly critiqued ideals of self-improvement, arguing that game playing was important for its own sake. Yet, even the pleasure associated with gaming was occasionally instrumentalized as a mechanism for ensuring prudent life choices. The analysis casts fresh light on how debates around health, ageing, and science correspond to configurations of technology uses and users. It presents evidence of the widespread cultural circulation of enjoiners regarding self-care and healthy ageing within British society. However, the paper also provides indications of the limits to such imperatives: discourses of pleasure co-exist with and perhaps supplant logics of prudence in (accounts of) practices ostensibly aimed at ageing 'well'. Copyright © 2017 The Authors. Published by Elsevier Ltd.. All rights reserved.

  20. FORMATION OF STUDENTS' MOTIVATION FOR PHYSICAL CULTURE AND SPORTS IN HIGHER EDUCATION

    Directory of Open Access Journals (Sweden)

    Е. A. Chelnokova

    2018-01-01

    Full Text Available Introduction: The appeal to the research topic is caused by the actual problem: in the pedagogical practice of the higher school, the physical culture and health-improving activities in the majority do not contribute to the formation of independent, motivated and sustainable self-improvement activities by students with the help of physical culture. The traditional system of physical education is not characterized by efficiency. The formation of motivation for physical education is one of the most important problems of the modern university.Materials and methods: to assess the value potential of physical culture and sports, we set the task to identify: the attitude of students to physical culture and health, what is the social significance of physical culture, the systematic nature of physical culture and sports, the objective and subjective factors that contribute to increasing interest in physical culture and Sport as a value, the reasons that interfere with attending classes. To solve this problem, we developed a questionnaire and conducted anonymous questioning of students.Results: Having analyzed the data obtained as a result of the questionnaire, we came to the conclusion that students objectively assess their health, which many are not satisfied with; most students are not engaged in physical culture and sports, referring mainly to the lack of free time; students are ready to engage in physical culture in sections for the selected sport, to attend self-educational activities. Students mostly respond positively to the lessons in the discipline "Physical Culture", but a considerable number of students attend these classes only for the sake of credit.Discussion and Conclusions: physical education, based on sports-oriented principles, includes classes in sports sections by sport: volleyball, basketball, aerobics, badminton, football, table tennis, kettlebell lifting, sports dancing, swimming, athletics, gymnastics, and for general physical

  1. More than just dancing: experiences of people with Parkinson's disease in a therapeutic dance program.

    Science.gov (United States)

    Bognar, Stephanie; DeFaria, Anne Marie; O'Dwyer, Casey; Pankiw, Elana; Simic Bogler, Jennifer; Teixeira, Suzanne; Nyhof-Young, Joyce; Evans, Cathy

    2017-06-01

    To understand why individuals with Parkinson's disease (PD) participate in a community-based therapeutic dance program and to explore its influence on perceived physical, social and emotional well-being of participants. A qualitative descriptive design was employed using one-on-one semi-structured interviews. Individuals with PD who participated in the Dancing with Parkinson's program were recruited from two locations. Interviews were audio-recorded, transcribed, de-identified and then placed into NVivo 10 software for analysis. A content analysis approach was used with an inductive analysis method to generate a coding scheme. Group discussion facilitated development of overarching themes. Ten participants' responses revealed that the dance program allows for self-improvement and regaining identity through disease self-management. Positive influences of socialization arose through the class, decreasing isolation and improving quality of life. Participants communicate through music and dance to enhance connection with others. Dancing with Parkinson's classes allow for re-development of the social self, which can increase sense of enjoyment in life. Dance programs provide opportunities for social interaction, non-verbal communication and self-improvement, reestablishing self-identity and a sense of usefulness. This study provides unique insight into the experience of participating in a dance program from the perspective of individuals with PD. Implications for rehabilitation Dance is emerging as a strategy to address the physical and psychosocial effects of Parkinson's disease (PD), but little is known regarding participants' perceptions of community-based therapeutic dance programs for PD. This study found that Dancing with Parkinson's (DWP) facilitated an improvement in social participation, resulting in decreased isolation and improved quality of life. Participation in the DWP program can facilitate a positive change in perspective and attitude toward a PD

  2. Road to excellence through industry self-management

    International Nuclear Information System (INIS)

    Miller, J.H. Jr.

    1985-01-01

    In March 1984, nuclear utilities in the US took the initiative to make regulations in management and people-related areas unnecessary, as such regulations would interfere with our ability to manage ourselves. Such an initiative is the Nuclear Utility Management and Human Resources Committee (NUMARC), which was formed because the authors believe the electric utility industry is best capable of managing itself. NUMARC works with the US Nuclear Regulatory Commission (NRC) and its staff to develop a new approach to improving management and people-related areas of nuclear plant operation. In a recent NRC policy statement on training and qualification, the NRC recognized that the industry, through the initiatives of NUMARC and the Institute of Nuclear Power Operations (INPO), has made progress in developing programs to improve nuclear utility training and personnel qualification. It also recognized the importance of the industry's initiative and encouraged further self-improvement. The NRC stated it will refrain from new rulemaking in the area of training for a minimum of 2 yr, while monitoring industry efforts

  3. F. M. DOSTOYEVSKY AND L. N. TOLSTOY ABOUT SPIRITUALITY

    Directory of Open Access Journals (Sweden)

    S. V. Strutsenko

    2014-01-01

    Full Text Available The paper is devoted to the urgent issue of moral personality formation in the framework of the modern education system. Both the society and education specialists are concerned about the unsatisfactory moral education of the growing generation. For solving the problem of moral revival, the author considers the Russian pedagogical heritage - in particular, philosophical and publicistic works of F. M. Dostoyevsky and L. N. Tolstoy. The paper provides the modern definition of spirituality as the intellectual essence of the human being, based both on the deep knowledge of nature, society, human individuality and humanistic or religious values outweighing the selfish needs. In author’s opinion, the pedagogical concepts developed by Dostoyevsky and Tolstoy are still relevant, being aimed at developing a free personality and addressing the universal human values. The ideas and concepts of the 19th century’s outstanding thinkers perfectly correspond with the currently proclaimed educational content: they define the man as the highest society value; direct all the principles and parenting practices of spiritual and ethic education at creativity development, self-development, and self-improvement; and acknowledge the student’s right of individuality.

  4. F. M. DOSTOYEVSKY AND L. N. TOLSTOY ABOUT SPIRITUALITY

    Directory of Open Access Journals (Sweden)

    S. V. Strutsenko

    2014-01-01

    Full Text Available The paper is devoted to the urgent issue of moral personality formation in the framework of the modern education system. Both the society and education specialists are concerned about the unsatisfactory moral education of the growing generation. For solving the problem of moral revival, the author considers the Russian pedagogical heritage – particularly, the philosophical and publicistic works of F. M. Dostoyevsky and L. N. Tolstoy. The paper provides the modern definition of spirituality as the intellectual essence of the human being, based both on the deep knowledge of nature, society, human individuality and humanistic or religious values outweighing the selfish needs. In author’s opinion, the pedagogical concepts developed by Dostoyevsky and Tolstoy are still relevant, being aimed at developing the free personality and addressing the universal human values. The ideas and concepts of the 19th century’s outstanding thinkers perfectly correspond with the currently proclaimed educational content: they define the man as the highest society value; direct all the principles and parenting practices of spiritual and ethic education at creativity development, self-development, and self-improvement; and acknowledge a student’s individuality.

  5. Physical Culture as an integral part of general culture of medical students

    Directory of Open Access Journals (Sweden)

    N. V. Sivas

    2015-01-01

    Full Text Available The problem of education of the individuality through the culture with the help of development of value potential in physical culture is discussed in the article. Improving the efficiency of education of medical students is becoming the leading aim of high school, which is connected with the development of culture of thinking, imagination, feelings and human creativity. Development of human motor capabilities is inseparable from the development of his personal qualities in physical education. One of the most important tasks of the educational process at high school is providing the motivation of a healthy lifestyle, motivation for physical culture and sports. Promotion of a healthy lifestyle should go through the activation of incentive mechanisms and a number of other phenomena of the individual's inner world. Efficiency of this approach is that it provides activity of a person in questions connected with preservation of individual and public health. The article tells us about the need to develop programs that can promote future professionals to form healthy and productive lifestyle, sustained motivation to permanent physical self-improvement. The problem can be successfully solved in the process of learning such course as «Physical education».

  6. A model of pathways to artificial superintelligence catastrophe for risk and decision analysis

    Science.gov (United States)

    Barrett, Anthony M.; Baum, Seth D.

    2017-03-01

    An artificial superintelligence (ASI) is an artificial intelligence that is significantly more intelligent than humans in all respects. Whilst ASI does not currently exist, some scholars propose that it could be created sometime in the future, and furthermore that its creation could cause a severe global catastrophe, possibly even resulting in human extinction. Given the high stakes, it is important to analyze ASI risk and factor the risk into decisions related to ASI research and development. This paper presents a graphical model of major pathways to ASI catastrophe, focusing on ASI created via recursive self-improvement. The model uses the established risk and decision analysis modelling paradigms of fault trees and influence diagrams in order to depict combinations of events and conditions that could lead to AI catastrophe, as well as intervention options that could decrease risks. The events and conditions include select aspects of the ASI itself as well as the human process of ASI research, development and management. Model structure is derived from published literature on ASI risk. The model offers a foundation for rigorous quantitative evaluation and decision-making on the long-term risk of ASI catastrophe.

  7. ANALISIS KRITERIA PERSONAL BRANDING SELEBGRAM NON SELEBRITI (STUDI DESKRIPTIF KUALITATIF AKUN INSTAGRAM @LIPPIELUST

    Directory of Open Access Journals (Sweden)

    Dita Rachmawati

    2018-03-01

    Full Text Available ABSTRACTSocial media nowadays has become tool of marketing and promotion of goods, services, and personallike Instagram. Being a celebgram should have a strong Personal Branding, has uniqueness, thedifference from other celebgram in order to have a long existence to be celebgram on social mediaInstagram. Rissa is a non-celebrity celebgram. Rissa’s known as an endorser for beauty project thisleap how to work together on a project professional with many brands in the country and abroad.Rissa became a celebgram with a very specific focus that is more beauty that leads to lipstick. This study analyze about how Personal Branding criteria non celebrity celebgram. The method used is descriptive qualitative with indicator  11  Authentic Personal Branding criterion according toRampersad. In this study, Rissa’s Personal Branding has a personality as which  is character, values,vision that suits personal ambition, moral code and behavior, consistent, focused on one field,acknowledged and experienced, unique, connected to the audience, owns good relationship withpartner work and always make self improvement in Instagram account @ lippielust.Keywords - Personal Branding, Social Media, Instagram, Celebgram, Rissa Stellar

  8. Cleft lip and palate treatment of 530 children over a decade in a single centre.

    Science.gov (United States)

    Vlastos, I M; Koudoumnakis, E; Houlakis, M; Nasika, M; Griva, M; Stylogianni, E

    2009-07-01

    We sought to evaluate the process of care and the outcomes of cleft lip and palate operations carried by a multidisciplinary team at a centre of craniofacial anomalies with a high patients' volume. A retrospective review of all cleft lips and/or palates cases treated in the centre from 1995 to 2007 was performed. Direct and long term complication rates, clinical, audiologic, speech intelligibility and dental arch assessments were analyzed. A total of 530 children have been operated this period in the centre (64 isolated cleft lip closures). A detailed presentation of the outcomes is performed in relation to the various types of cleft lip and palates. The majority of parents (70%) reported very good or excellent results 2-5 years after the lip closure with the Millard technique, although those with bilateral clefts were significantly less satisfied (Ppalate and otitis media with effusion were self-improved 2-8 months after palate reconstruction and 83.3% of children treated with the two flaps palatoplasty technique had a rather high or very high intelligibility score. Muscles' retropositioning had a significant effect on intelligibility (P=0.04). Children with cleft lips and palates have a variety of conditions and functional limitations even after the surgical correction of their problem that need to be evaluated and treated by several specialists. The treatment protocol utilized by the multidisciplinary team of our centre is efficient with a relative low percentage of complications and unfavorable results.

  9. Special aspects of motivation of the structural subdivisions of the state emergency service of Ukraine in terms of physical self-culture

    Directory of Open Access Journals (Sweden)

    Stetsenko A.I.

    2015-03-01

    Full Text Available Purpose: to determine the motivation of employees of structural subdivisions of the State Emergency Service of Ukraine to improve their level of professional competence by means of physical training. Material: questionnaire survey of 130 rescue workers aged 25 to 40 years. Results: the main motives of rescue team personnel for physical culture and sports activities are gain in physical health and professional competence, while performing rescue missions. It was established that, when on duty, most of the firefighters and rescue workers are not engaged in physical exercise at all; household chores and poor state of health in case of men prevent rescue team employees from doing exercises outside of working hours. It was found that fire-rescue specialists give preference to the development of muscle strength during professional physical trainings and would like to perform power exercises. Conclusions: the low level of motivation of current fire-rescue workers for physical self-improvement requires optimization of control over professional physical education in departments of the State Emergency Service of Ukraine.

  10. Life Goals Over Time Among Homeless Adults in Permanent Supportive Housing.

    Science.gov (United States)

    Wenzel, S L; Rhoades, H; Moore, H; Lahey, J; Henwood, B; La Motte-Kerr, W; Bird, M

    2018-03-14

    Permanent supportive housing (PSH) is a widely-accepted solution to the challenge of chronic homelessness. While housing support and retention, physical health, and healthcare continue to be important for formerly homeless persons in PSH, "higher-order" and humanistic needs such as thriving have received less attention and as a result are less well understood in this population. One important indicator of thriving is the ability to establish and articulate life goals. This study utilizes longitudinal data from 421 formerly homeless adults prior to their move into PSH, and at 3-, 6- and 12-months after move-in (369 respondents completed all four interviews), to examine what life goals are articulated by this population and how those goals change over time. Prior to housing, most respondents articulated housing attainment as their primary life goal, whereas at follow-up interviews health goals, housing relocation, and financial goals became more prevalent. Aspirational goals (e.g., independence, self-improvement, artistic pursuits) were also common, but demonstrated a decrease over time in housing. Relationship goals remained common and consistent over time. Findings indicate that housing is a necessary, but perhaps not sufficient, step for improving thriving among formerly homeless adults. Implications for practice and future research are discussed. © Society for Community Research and Action 2018.

  11. The "zing factor"-how do faculty describe the best pediatrics residents?

    Science.gov (United States)

    Rosenbluth, Glenn; O'Brien, Bridget; Asher, Emily M; Cho, Christine S

    2014-03-01

    Faculty in graduate medical education programs may not have uniform approaches to differentiating the quality of residents, and reviews of evaluations suggest that faculty use different standards when assessing residents. Standards for assessing residents also do not consistently map to items on evaluation forms. One way to improve assessment is to reach consensus on the traits and behaviors that are (or should be) present in the best residents. A trained interviewer conducted semistructured interviews with faculty affiliated with 2 pediatrics residency programs until content saturation was achieved. Interviewees were asked to describe specific traits present in residents they identify as the best. Interviews were recorded and transcribed. We used an iterative, inductive approach to generate a coding scheme and identify common themes. From 23 interviews, we identified 7 thematic categories of traits and behaviors: personality, energy, professionalism, team behaviors, self-improvement behaviors, patient-interaction behaviors, and medical knowledge and clinical skills (including a subcategory, knowledge integration). Most faculty interviewees focused on traits like passion, enthusiasm, maturity, and reliability. Examination score or intelligence was mentioned less frequently than traits and behaviors categorized under personality and professionalism. Faculty identified many traits and behaviors in the residents they define as the best. The thematic categories had incomplete overlap with Accreditation Council for Graduate Medical Education (ACGME) and CanMEDS competencies. This research highlights the ongoing need to review our assessment strategies, and may have implications for the ACGME Milestone Project.

  12. The influence of Academic Sports Associations on the development of a sports career

    Directory of Open Access Journals (Sweden)

    Tomasz Łosień

    2017-10-01

    Full Text Available Introduction: Young people who continue their education at universities do not have to give up their physical activity. The existence of Academic Sports Unions allows you to continue and develop your sporting career. A significant number of students regularly participating in AZS classes have a chance to develop their sporting career. The possibility of obtaining a sports scholarship is an additional motivation for students to pursue their own scientific and sporting goals. Sport through the process of self-improvement introduces a specific discipline to everyday life, teaches regularity, diligence and good organization of time.The aim of the study: 1 Did you start your education at a university by limiting or giving up sports?, 2 Do universities and AZS help develop a sports career? 3 Did the students of AZS influence the development of their sports career? Material and methods: 204 students participated in the study, an original questionnaire consisting of 26 questions was used. The questions concerned, among others: forms of physical activity and training experience, the impact of undertaking education at a university on the development of a sports career. Results and conclusions: Over 80% of respondents did not give up their sport before starting their studies. Over 50% of respondents believe that AZS and universities support the development of young athletes, giving the opportunity to get better and better results.

  13. Positive technology–A powerful partnership between positive psychology and interactive technology. A discussion of potential and challenges.

    Directory of Open Access Journals (Sweden)

    Sarah Diefenbach

    2017-11-01

    Full Text Available Under the umbrella term "positive computing" concepts of positive psychology are transferred to the domain of human-computer interaction (HCI. In an interdisciplinary community psychologist, computer scientists, designers and others are exploring promising ways how to utilize interactive technology to support wellbeing and human flourishing. Along with this, the recent popularity of smartphone apps aiming at the improvement of health behavior, mindfulness and positive routines, suggests the general acceptance of technology as a facilitator of personal development. Given this, there generally seems a high potential for a technology mediated trigger of positive behavior change, especially in context of positive psychology and resource oriented approaches such as solution-focused coaching. At the same time, there is still a lack of well-founded approaches to design such technology which consider its responsible role as an "interactive coach" and systematically integrate the needed expertise of different disciplines. The present article discusses the general potential and particular challenges to support the goals of positive psychology and human desire for self-improvement through interactive technology and highlights critical steps for a successful partnership between both.

  14. Control System Architectures, Technologies and Concepts for Near Term and Future Human Exploration of Space

    Science.gov (United States)

    Boulanger, Richard; Overland, David

    2004-01-01

    Technologies that facilitate the design and control of complex, hybrid, and resource-constrained systems are examined. This paper focuses on design methodologies, and system architectures, not on specific control methods that may be applied to life support subsystems. Honeywell and Boeing have estimated that 60-80Y0 of the effort in developing complex control systems is software development, and only 20-40% is control system development. It has also been shown that large software projects have failure rates of as high as 50-65%. Concepts discussed include the Unified Modeling Language (UML) and design patterns with the goal of creating a self-improving, self-documenting system design process. Successful architectures for control must not only facilitate hardware to software integration, but must also reconcile continuously changing software with much less frequently changing hardware. These architectures rely on software modules or components to facilitate change. Architecting such systems for change leverages the interfaces between these modules or components.

  15. Psychological educational features of professional reflection levels in students of the teacher-training specialties

    Directory of Open Access Journals (Sweden)

    Asya A. Bekhoeva

    2015-03-01

    Full Text Available Shaping professional reflection of future teachers is of particular importance in the context of the modernization of the Russian education. However, despite the deep reflection of a problem in Russian pedagogical science the characteristics of development levels of pedagogical reflection among future teacher remains largely fragmented. The paper deals with professional and pedagogical reflection as a process of perceiving essential features of educational process by a teacher, summarizes the main theoretical and methodological approaches to this issue. The research is aimed to identify and describe levels of professional and pedagogical reflection among students. The research is divided in several stages: the stage of theoretical allocation of substantial components of professional pedagogical reflection, the stage of selecting proper research tools, ascertaining stage, and concluding stage. The conceptual basis of the research is to identify the main components that determine the following features of professional and pedagogical reflection: motivational, creative, emotional volitional, communicative, monitoring and evaluative. Based on the empirical results the levels of professional and pedagogical reflection of the students of the teacher-training specialties are identified. The first level is characterized by weak professional reflection and undifferentiated consciousness, self-awareness and self-esteem in the normal course of activities, the second level is associated with certain reflective activity and organization and is characterized by steady demand for professional and personal self-improvement. The indicator of the third level is high development of all components of professional reflection.

  16. A theoretical perspective to inform assessment and treatment strategies for animal hoarders.

    Science.gov (United States)

    Patronek, Gary J; Nathanson, Jane N

    2009-04-01

    Animal hoarding is a poorly understood, maladaptive, destructive behavior whose etiology and pathology are only beginning to emerge. We compare and contrast animal hoarding to the compulsive hoarding of objects and proceed to draw upon attachment theory, the literature of personality disorder and trauma, and our own clinical experience to propose a developmental trajectory. Throughout life, there is a persistent struggle to form a functional attachment style and achieve positive social integration. For some people, particularly those affected by a dysfunctional primary attachment experience in childhood, a protective, comforting relationship with animals may form an indelible imprint. In adulthood, when human attachment has been chronically problematic, compulsive caregiving of animals can become the primary means of maintaining or building a sense of self. Improving assessment and treatment of animal hoarders requires attention to contributing psychosocial conditions, while taking into account the centrality of the animals to the hoarder's identity, self-esteem and sense of control. It is our hope that the information presented will provide a basis upon which clinicians can focus their own counseling style, assessment, and methods of treatment.

  17. The Impact of Social Support and Attachment Style on Quality of Life and Readiness to Change in a Sample of Individuals Receiving Medication-Assisted Treatment for Opioid Dependence.

    Science.gov (United States)

    Cavaiola, Alan A; Fulmer, Barbara A; Stout, David

    2015-01-01

    A basic principle within the addictions treatment field is that social support is a vital ingredient in the recovery process. This study examines the nature of social support in a sample of opioid-dependent men and women who are currently being treated in a medication-assisted treatment program (methadone). This research examines the types of social support behaviors that the opioid-dependent individuals consider helpful and explores whether attachment style (i.e., secure, ambivalent, or anxious attachment) was a determining factor in whether social support was perceived as helpful. The dependent variables included readiness to change addictive behaviors and abstinence from other mood-altering drugs. Participants (N = 159) completed a demographic questionnaire, the Significant Others Scale, the Experiences in Close Relationships Scale, the Multidimensional Scale of Perceived Social Support Assessment, the Readiness to Change Scale, and an Attachment Style Questionnaire. The demographic questionnaire included subjective ratings of self-improvement. Social support predicted perceived improvement in all of the areas examined (e.g., health, family/social relationships) and abstinence; however, attachment style did not predict improvement or with readiness to change. Social support is an important factor in one's recovery from substance use disorders. Yet attachment style (i.e., anxious, avoidant, or secure) did not predict abstinence or overall improvement in functioning.

  18. "Personal mission statement": An analysis of medical students' and general practitioners' reflections on personal beliefs, values and goals in life.

    Science.gov (United States)

    Chew, B H; Lee, P Y; Ismail, I Z

    2014-01-01

    Personal mission in life can determine the motivation, happiness, career advancement and fulfilment in life of the medical students (MSs) along with improvement in professional/clinical performance of the family physicians. This study explored the personal beliefs, values and goals in the lives of MSs and general practitioners (GPs). Fourth-year MSs at the Universiti Putra Malaysia and GPs who participated in a 2-hour session on 'Ethics in Family Medicine' in 2012 were invited. All the participants submitted the post-session written reflections about their personal missions in life. The written reflections were analysed using thematic analysis. A total of 87 MSs and 31 GPs submitted their written reflections. The authors identified 17 categories from the reflections contained by four themes-good vs. smart doctor, professional improvement vs. self-improvement, self-fulfilment and expressed motivation. The most common categories were "to be a good doctor" (97/330) and "professional improvement" (65/330). Many MSs had expressed motivation and wanted to be a smart doctor as compared to the GPs, whereas a larger number of GPs wished to have a fulfilled life and be a good doctor through professional improvement. The difference between the two student groups might indicate different levels of maturity and life experiences. Medical teachers should engage students more effectively in orientating them towards the essential values needed in medical practice.

  19. Cost-based industrial enterprise human capital management

    Directory of Open Access Journals (Sweden)

    Glukhov Sergei

    2017-01-01

    Full Text Available The paper focuses on importance of human capital in development of industrial enterprises, issues of their management and methods of achieving balance between interests of owners and employees. Difference between such concepts as human and labor potential as well as human and working capital is clarified. The special attention is paid to the fact that an effective system of motivation and incentivation of labor is to serve as means of rapprochement of human and labor capital. The authors point out the limited scope of the traditional forms of labor motivation and incentivation mechanisms and highlight the complexity of their encouragement for collective work results. The authors suggest using the cost-based approach to workforce management, which is based on assessment of market and intrinsic value of human capital in view of investment and quality characteristics. The proposed approach is attended by methodological support and operational calculations. The study states that the suggested human capital management model can be introduced into practice, as well as substantiates the necessity for boosting the performance of industrial enterprises and their cost by increasing production, stimulating the staff for selfrealization and self-improvement by bringing the owners’ corporate interests closer to the interests of the employees.

  20. [Action tendencies of respect-related emotions: Focus on emotion episodes in Japanese university students].

    Science.gov (United States)

    Muto, Sera

    2016-06-01

    This study examined the action tendencies of respect-related emotions in Japanese university students. Participants (n = 405) randomly received a questionnaire about one of six respect-related emotions: (a) keiai (respect mingled with mild love); (b) shinsui (idolatry worship, and adoration); (c) ifu (awe mingled with fear); (d) kanshin (admiration); (e) kyotan (wonder); and (f) sonkei (respect proper) and were asked to recall a situation they felt the emotion. Next, they rated how much they felt like doing the respect-related (intrapersonal or interpersonal) actions in the situation. Statistical analysis revealed several action tendencies of respect-related emotions, however, the degree of each differed between the prototypical episodes of the emotions (a)-(e). The action tendency pattern of sonkei was most similar to that of keiai, therefore keiai could be considered as the prototypical feeling of sonkei in university students. Furthermore, almost all the respect-related emotions tended to strongly motivate willingness for self-correction and improvement. These findings suggest that respect-related emotions play an important role in self-improvement and building good relationships with superiors, at least in late adolescence.

  1. Keefektifan Layanan Bimbingan Kelompok dengan Media Film dalam Meningkatkan Self Esteem

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    Kamaludin Reza Sauqi

    2017-08-01

    Full Text Available The purpose from this research is to see the effectiveness of group guidance service within increase child self esteem at Yayasan Setara, Semarang. This type of research is experimental research with pre experimental design with a kind of one group pre test and post test design. Subject of this research is 5 grade student of PL Servatius Elementary School. Collecting data technique in this research using a psychological scale and collecting data tool using likert scale with 42 number of declaration point that has been tested before. Data analysis methods is using with wilcoxon test. Research has done to see self esteem before group guidance with film media given show that self esteem criteria in middle level (67,91%. Child self esteem after group guidance with film media given counted to high criteria (80,43%. Self esteem increased 12,52% after group guidance with film media given. Highest increase occurred in self improvement indicator with 16% percentage. Lowest increase indicator with 10,67% is self adjustment indicator. Wilcoxon test result tcount= 55 and ttable= 8, tcount> ttable, so Ho rejected and Ha accepted.

  2. Principles and practice of external digital photography in ophthalmology

    Directory of Open Access Journals (Sweden)

    Bipasha Mukherjee

    2012-01-01

    Full Text Available It is mandatory to incorporate clinical photography in an ophthalmic practice. Patient photographs are routinely used in teaching, presentations, documenting surgical outcomes and marketing. Standardized clinical photographs are part of an armamentarium for any ophthalmologist interested in enhancing his or her practice. Unfortunately, many clinicians still avoid taking patient photographs for want of basic knowledge or inclination. The ubiquitous presence of the digital camera and digital technology has made it extremely easy and affordable to take high-quality images. It is not compulsory to employ a professional photographer or invest in expensive equipment any longer for this purpose. Any ophthalmologist should be able to take clinical photographs in his/her office settings with minimal technical skill. The purpose of this article is to provide an ophthalmic surgeon with guidelines to achieve standardized photographic views for specific procedures, to achieve consistency, to help in pre-operative planning and to produce accurate pre-operative and post-operative comparisons, which will aid in self-improvement, patient education, medicolegal documentation and publications. This review also discusses editing, storage, patient consent, medicolegal issues and importance of maintenance of patient confidentiality.

  3. Nursing: building self-esteem in neurotic disorders in the elderly

    Directory of Open Access Journals (Sweden)

    Giovanna Enríquez Gutiérrez

    2014-04-01

    Full Text Available The prevailing cultural model is simplistic in that it envisions the development of life in terms of beginning, fullness and decay. During aging, older adults can witness their physical and mental deterioration, change society qualifies as poor and dependent, the appreciation of low self-esteem in them arises. Given the above, this research has as main purpose to promote the participation of the elderly with neurotic conditions for such that population fits your problem, regardless of its nature, and increase their self-esteem. The objective is to provide comprehensive support to older adults and their caregivers to strengthen in order to promote self-esteem of people with neurotic condition to maintain personal autonomy; apart seeks to provide a comprehensive and continuously support for dependents. The planning and development is based on the explanation of the disease, how it affects the balance of your system and how to achieve this need and how the parties of the client system interact inharmony with the entire system. The purpose is to show how you can achieve optimal care of these people in collaboration with the family, helping in the development of their environment, accepting the changes, and adapt and incorporate new circumstances by enriching their self- improvement to restore their well-being and promoteevery area of your life.

  4. [Evaluation of the Initial Stage Career Exploration Inventory (ISCEI)].

    Science.gov (United States)

    Adachi, Tomoko

    2010-06-01

    The Initial Stage Career Exploration Inventory (ISCEI) was designed to assess career exploration among students in the early stage of making career decisions. The reliability, validity, and applicability of the ISCEI were investigated. In Study 1, responses on the ISCEI from student participants (n = 294 : 69 men, 225 women) were factor analyzed. The results suggested a 3-factor structure consisting of "self-understanding," "information gathering" and "learning from others." Comparison between the ISCEI and self-improvement motive, vocational decisions, and career decision-making self-efficacy scales from the Career Exploration Survey (CES) indicated that the ISCEI had sufficient construct validity. Study 2 investigated the applicability of the ISCEI. The responses of student participants (n = 859 : 451 men, 408 women) on the ISCEI indicated high "self-understanding," neutral "information gathering," and comparatively low "learning from others" scores, which were similarly related to the CES as in Study 1. These findings indicate that the ISCEI can be used as a tool for understanding career exploration among students in the initial stage of making career decision.

  5. Description and Early Outcomes of a Comprehensive Curriculum Redesign at the Northwestern University Feinberg School of Medicine.

    Science.gov (United States)

    Heiman, Heather L; O'Brien, Celia L; Curry, Raymond H; Green, Marianne M; Baker, James F; Kushner, Robert F; Thomas, John X; Corbridge, Thomas C; Corcoran, Julia F; Hauser, Joshua M; Garcia, Patricia M

    2017-09-26

    In 2012, the Northwestern University Feinberg School of Medicine launched a redesigned curriculum addressing the four primary recommendations in the 2010 Carnegie Foundation for the Advancement of Teaching report on reforming medical education. This new curriculum provides a more standardized evaluation of students' competency achievement through a robust portfolio review process coupled with standard evaluations of medical knowledge and clinical skills. It individualizes learning processes through curriculum flexibility, enabling students to take electives earlier and complete clerkships in their preferred order. The new curriculum is integrated both horizontally and vertically, combining disciplines within organ-based modules and deliberately linking elements (science in medicine, clinical medicine, health and society, professional development) and threads (medical decision making, quality and safety, teamwork and leadership, lifestyle medicine, advocacy and equity) across the three phases that replaced the traditional four-year timeline. It encourages students to conduct research in an area of interest and commit to lifelong learning and self-improvement. The curriculum formalizes the process of professional identity formation and requires students to reflect on their experiences with the informal and hidden curricula, which strongly shape their identities.The authors describe the new curriculum structure, explain their approach to each Carnegie report recommendation, describe early outcomes and challenges, and propose areas for further work. Early data from the first cohort to progress through the curriculum show unchanged United States Medical Licensing Examination Step 1 and 2 scores, enhanced student research engagement and career exploration, and improved student confidence in the patient care and professional development domains.

  6. A Qualitative Study to Examine Feasibility and Design of an Online Social Networking Intervention to Increase Physical Activity in Teenage Girls.

    Science.gov (United States)

    Van Kessel, Gisela; Kavanagh, Madeleine; Maher, Carol

    2016-01-01

    Online social networks present wide-reaching and flexible platforms through which to deliver health interventions to targeted populations. This study used a social marketing approach to explore teenage girls' perceptions of physical activity and the potential use of online social networks to receive a physical activity intervention. Six focus groups were conducted with 19 Australian teenage girls (ages 13 to 18 years) with varying levels of physical activity and socioeconomic status. A semi-structured format was used, with groups discussion transcribed verbatim. Content analysis identified emergent themes, with triangulation and memos used to ensure accuracy. Physical activity was most appealing when it emphasised sport, exercise and fitness, along with opportunities for socialisation with friends and self-improvement. Participants were receptive to delivery of a physical activity intervention via online social networks, with Facebook the most widely reported site. Participants commonly accessed online social networks via mobile devices and particularly smartphones. Undesirable features included promotion of physical activity in terms of walking; use of cartoon imagery; use of humour; and promotion of the intervention via schools, each of which were considered "uncool". Participants noted that their parents were likely to be supportive of them using an online social networking physical activity intervention, particularly if not promoted as a weight loss intervention. This study identified key features likely to increase the feasibility and retention of an online social networking physical activity intervention for teenage girls. Guidelines for the design of interventions for teenage girls are provided for future applications.

  7. Mudanças na concepção de escrita de jovens e adultos em processo de letramento

    Directory of Open Access Journals (Sweden)

    Sylvia Bueno Terzi

    2005-01-01

    Full Text Available Este trabalho tem por objetivo a análise das mudanças na concepção de escrita de jovens e adultos não escolarizados, decorrentes da participação em um curso de alfabetização de cinco meses. Os dados mostram que a maior relevância atribuída pelos sujeitos às práticas de letramento está relacionada à escrita como meio de resolução de problemas, de crescimento e satisfação pessoal e de participação social e cidadania.This paper dicusses the changes in the conception of the written language as expressed by unschooled adolescents and adults who participated in a five-month literacy course. The data show that before the course the students considered literacy as a means for solving daily practical problems. After the course, they regarded literacy as a tool for self-improvement, social interaction and citizenship

  8. Stigmatization and denormalization as public health policies: some Kantian thoughts.

    Science.gov (United States)

    Dean, Richard

    2014-10-01

    The stigmatization of some groups of people, whether for some characteristic they possess or some behavior they engage in, will initially strike most of us as wrong. For many years, academic work in public health, which focused mainly on the stigmatization of HIV-positive individuals, reinforced this natural reaction to stigmatization, by pointing out the negative health effects of stigmatization. But more recently, the apparent success of anti-smoking campaigns which employ stigmatization of smokers has raised questions about whether stigmatization may sometimes be justified, because of its positive effects on public health. Discussion of the issue so far has focused on consequences, and on some Kantian considerations regarding the status of the stigmatized. In this article, I argue that further Kantian considerations regarding the treatment of the general public (the potential stigmatizers) also count against any public health policy involving stigmatization. Attempts to encourage stigmatization are likely to fail to appeal to the rational decision-making abilities of the general public, and the creation of stigmatized groups (even if they are stigmatized for their voluntary behavior) is an obstacle to the self-improvement of members of the general public. © 2013 John Wiley & Sons Ltd.

  9. Innovative method for training students to develop enterprising, decision-making and cooperation skills through complex computernetworks team-design

    Directory of Open Access Journals (Sweden)

    Mătăsaru Petre-Daniel

    2017-01-01

    Full Text Available Our paper presents a short overview of the educational methods used in the learning process in telecommunication domain, targeting skills and competences students acquire and develop during the semester and focusing on the ones that are most valuable and appreciated on the labor market after graduation. Our research of the market shows that besides basic engineering knowledge, skills like initiative, team-playing, business analysis, decision-making, marketing and creative project presentation are considered very valuable assets by HR recruiters. We implement and test an improved method that combines classic techniques with the use of modern digital tools, emphasizing on specific tasks that coach the student how to deal with real markets, extract valuable data through analysis, design up-to-date computer-networks, make correlated decisions based also on economic arguments, team working and assume responsibilities. This is achieved through a real-case study and project themes for computer-networks that involve real situations with technical and budgetary challenges, market analysis and research through online facilities and dealing with specialized software for network design and simulation with a creative presentation. The presented approach is intended to prepare faculty staff to implement innovative and self-improving teaching methods in engineering educational process and thus make an educational reform happen [1].

  10. Improving STEM Student Learning Outcomes with GIS

    Science.gov (United States)

    Montgomery, W. W.

    2013-12-01

    Longitudinal data collection initiated a decade ago as part of a successful NSF-CCLI grant proposal has resulted in a large - and growing - sample (200+) of students who report on their perceptions of self-improvement in Technology, Critical Thinking, and Quantitative Reasoning proficiencies upon completion of an introductory (200-level) GIS course at New Jersey City University, a Hispanic-Serving and Minority Institution in Jersey City, NJ. Results from student satisfaction surveys indicate that, not surprisingly, 80% of respondents report improved confidence in Technology Literacy. Critical Thinking proficiency is judged to be significantly improved by 60% of respondents. On the other hand, Quantitative Reasoning proficiency confidence is improved in only 30% of students. This latter finding has prompted the instructor to search for more easily recognizable (to the student) ways of embedding quantitative reasoning into the course, as it is obvious to any GIS professional that there is an enormous amount of quantitative reasoning associated with this technology. A second post-course questionnaire asks students to rate themselves in these STEM proficiency areas using rubrics. Results mirror those from the self-satisfaction surveys. On a 5-point Likkert scale, students tend to see themselves improving about one letter grade on average in each proficiency area. The self-evaluation rubrics are reviewed by the instructor and are judged to be accurate for about 75% of the respondents.

  11. The Social Context of "Do-It-Yourself" Brain Stimulation: Neurohackers, Biohackers, and Lifehackers.

    Science.gov (United States)

    Wexler, Anna

    2017-01-01

    The "do-it-yourself" (DIY) brain stimulation movement began in earnest in late 2011, when lay individuals began building stimulation devices and applying low levels of electricity to their heads for self-improvement purposes. To date, scholarship on the home use of brain stimulation has focused on characterizing the practices of users via quantitative and qualitative studies, and on analyzing related ethical and regulatory issues. In this perspective piece, however, I take the opposite approach: rather than viewing the home use of brain stimulation on its own, I argue that it must be understood within the context of other DIY and citizen science movements. Seen in this light, the home use of brain stimulation is only a small part of the "neurohacking" movement, which is comprised of individuals attempting to optimize their brains to achieve enhanced performance. Neurohacking itself is an offshoot of the "life hacking" (or "quantified self") movement, in which individuals self-track minute aspects of their daily lives in order to enhance productivity or performance. Additionally, the home or DIY use of brain stimulation is in many ways parallel to the DIY Biology (or "biohacking") movement, which seeks to democratize tools of scientific experimentation. Here, I describe the place of the home use of brain stimulation with regard to neurohackers, lifehackers, and biohackers, and suggest that a policy approach for the home use of brain stimulation should have an appreciation both of individual motivations as well as the broader social context of the movement itself.

  12. The ways of improvement of content and pedagogical conditions of preparation of master of preschool education in the context of development of creative potential

    Directory of Open Access Journals (Sweden)

    Oleksandra Iemchyk

    2017-04-01

    Full Text Available The necessity of changes in the process of professional preparation of master of preschool education, which is caused by insufficient preparation of graduates to solve problems in this field, low level of creative potential and, as the consequence, unavailability to realization creative pedagogical activity is outlined in the article. The main ways of improvement of content and pedagogical conditions of development of creative potential of master of preschool education in the process of professional preparation are presented: the improvement of content of scientific theoretical preparation of master, studying of progressive pedagogical experience; learning of professional pedagogical disciplines with the reliance to creative tasks with problem search nature; implementation to the educational process of magistracy of institution of higher education on the specialty “Preschool education” of special course “The foundations of development of creative potential of pedagogue”; attraction of students to the creative pedagogical practice; organization and management of students creative process from the side of teachers. There are also identified the perspectives of solution of the problem of development of creative abilities of personality of pedagogue during the professional preparation in the institution of higher education. The implementation of these ways to the educational process will contribute the increasing the readiness of graduates of institutions of higher education to realize pedagogical activity on the creative level, selfdevelopment and self-improvement of their personality.Key words: creative potential of master of preschool education, the professional preparation, the ways of improvement of content of professional preparation.

  13. Designing a leadership development program for surgeons.

    Science.gov (United States)

    Jaffe, Gregory A; Pradarelli, Jason C; Lemak, Christy Harris; Mulholland, Michael W; Dimick, Justin B

    2016-01-01

    Although numerous leadership development programs (LDPs) exist in health care, no programs have been specifically designed to meet the needs of surgeons. This study aimed to elicit practicing surgeons' motivations and desired goals for leadership training to design an evidence-based LDP in surgery. At a large academic health center, we conducted semistructured interviews with 24 surgical faculty members who voluntarily applied and were selected for participation in a newly created LDP. Transcriptions of the interviews were analyzed using analyst triangulation and thematic coding to extract major themes regarding surgeons' motivations and perceived needs for leadership knowledge and skills. Themes from interview responses were then used to design the program curriculum specifically to meet the leadership needs of surgical faculty. Three major themes emerged regarding surgeons' motivations for seeking leadership training: (1) Recognizing key gaps in their formal preparation for leadership roles; (2) Exhibiting an appetite for personal self-improvement; and (3) Seeking leadership guidance for career advancement. Participants' interviews revealed four specific domains of knowledge and skills that they indicated as desired takeaways from a LDP: (1) leadership and communication; (2) team building; (3) business acumen/finance; and (4) greater understanding of the health care context. Interviews with surgical faculty members identified gaps in prior leadership training and demonstrated concrete motivations and specific goals for participating in a formal leadership program. A LDP that is specifically tailored to address the needs of surgical faculty may benefit surgeons at a personal and institutional level. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. Development strategy and targets of CGNPG

    International Nuclear Information System (INIS)

    Zan Yunlong

    2002-01-01

    The development of nuclear power industry in Guangdong results from the steady implementation of a catch-up strategy aimed at the advanced world level in the nuclear power industry. China Guangdong Nuclear Power (Holding) Co., Ltd. (CGNPC) started from Daya Bay Nuclear Power Station (GNPS). In the form of joint venture, GNPS has obtained sophisticated technology, management expertise and human resources both at home and abroad, and has successfully completed the learning curve from importing, digesting, absorbing to innovating and self-improving. Under the principle of maintaining continuous nuclear power development by reinvesting the returns on the operating nuclear power stations, the second nuclear power project, Ling Ao Nuclear Power Station (LNPS) is progressing well and preparation for the third nuclear power project is now in full swing. With a rolling-on development mechanism being established, Daya Bay has become the cradle for nuclear power development in Guangdong. In the 21 st century, CGNPC is facing new challenges and opportunity. CGNPC will uphold the principle of maintaining continuous nuclear power development by reinvesting the returns on the operating nuclear power stations, brace itself for the market competition and explore sustained development of nuclear power in China by pursuing constant innovation in technology, management, system and concept. The strategy framework for future development of CGNPC is defined as follows: - to establish three-dimension strategic targets; - to pursue two-step development with the year 2015 as the dividing point; - to promote concerted development of nuclear power, associated industries and supporting services

  15. Postsecular Instruments of Acculturation. Czesław Miłosz’s Works from the Second American Stay

    Directory of Open Access Journals (Sweden)

    Jarzyńska Karina

    2017-08-01

    Full Text Available The article raises the question about the ways in which religious tradition can become an ally in the process of acculturation while serving the modern subject both as a springboard for innovative, creative work and as a tool of self-improvement. Czesław Miłosz’s selected works from his second stay in the United States (1961-1980 are analysed from the postsecular perspective which recognises religion as a full-fledged actor in the process of modern transformations that may broaden the field of artistic choice but remains vulnerable to artistic resemantizations or even profanations (Agamben. Such an analysis allows us to interpret the poem From the Rising of the Sun as a form of reconciliation of Miłosz’s American and Lithuanian experience (as well as of maturity and childhood, centre and periphery, modern and pre-modern cultural formation through textual practices inspired by his private Liturgy of the Hours. In this light, the translations of the Books of the Bible on which Miłosz worked, his novel The Mountains of Parnassus, as well as his essays from Visions from San Francisco Bay emerge as instruments of shaping the communal identity with the use of pre-existing rituals, which are, nonetheless, also negotiated in the act of writing.

  16. Image of God, religion, spirituality, and life changes in breast cancer survivors: a qualitative approach.

    Science.gov (United States)

    Schreiber, Judith A; Edward, Jean

    2015-04-01

    Religion and spirituality are much studied coping mechanisms; however, their relationship to changes in behaviors, relationships, and goals is unclear. This study explored the impact of a breast cancer diagnosis on religion/faith and changes in behaviors, relationship, or goals. In this qualitative study, women, who participated in a larger, quantitative study, completed written responses to questions regarding the role of religion/faith in their lives, the impact of their diagnosis on their image of God and on faith/religious beliefs, and any changes in behaviors, relationships, or life goals were examined. Based on previous findings noting differences in psychological outcomes based on a higher (HE) or lesser (LE) engaged view of God, 28 (14 HE; 14 LE) women were included in the analysis. Awareness of life and its fleeting nature was common to all. Ensuing behaviors varied from a need to focus on self-improvement-egocentrism (LE)-to a need to focus on using their experiences to help others-altruism (HE). Study results suggest that seemingly small, but highly meaningful, differences based on one's worldview result in considerably different attitudinal and behavioral outcomes.

  17. A longitudinal experimental study comparing the effectiveness of happiness-enhancing strategies in Anglo Americans and Asian Americans.

    Science.gov (United States)

    Boehm, Julia K; Lyubomirsky, Sonja; Sheldon, Kennon M

    2011-11-01

    Growing evidence suggests that well-being interventions can be effective. However, it is unclear whether happiness-increasing practices are equally effective for individuals from different cultural backgrounds. To investigate this question, Anglo Americans and predominantly foreign-born Asian Americans were randomly assigned to express optimism, convey gratitude, or list their past experiences (control group). Multilevel analyses indicated that participants in the optimism and gratitude conditions reported enhanced life satisfaction relative to those in the control condition. However, Anglo Americans in the treatment conditions demonstrated larger increases in life satisfaction relative to Asian Americans, while both cultural groups in the control condition showed the least improvement. These results are consistent with the idea that the value individualist cultures place on self-improvement and personal agency bolsters the efforts of Anglo Americans to become more satisfied, whereas collectivist cultures' de-emphasis of self-focus and individual goals interferes with the efforts of Asian Americans to pursue enhanced well-being.

  18. Monosymptomatic nocturnal enuresis

    Directory of Open Access Journals (Sweden)

    Bertan Karaboğa

    2012-03-01

    Full Text Available Enuresis Nocturna is the most common urologic problemin childhood. There is not a consensus about terminology.Terminology identified by The International Children’sContinence Society (ICCS is recommended. Bed-wettingat night during sleep (incontinence in children above 5years of age who don’t have congenital or acquired centralnervous system defect is defined as enuresis nocturna.There are two groups monosymptomatic (simpleand non-monosymptomatic (complicated. Monosymptomaticenuresis nocturna (MNE has no symptoms otherthan bed-wetting at night during sleep. Various theoriesconcerning etiology of MNE has been suggested; one ormore of genetic, hormonal, bladder associated and sleepdisorders are stated to play a role. Self-improvement canbe achieved each year by 15% increasing maturity. Underpinning treatment and in addition to this unique treatmentmust be done by considering the factors in the pathophysiology.The success of the treatment and roadmapto be followed must be arrange with child and family. Thepurpose of this eclectic is; evaluation of correct diagnosis,differential diagnosis, patient follow-up and treatment optionsof the cases applicant with nocturnal enuresis basedon the current knowledge of ICCS and Turkey EnuresisTreatment Guide.

  19. Influence of transition features on gender relations in Serbia

    Directory of Open Access Journals (Sweden)

    Mićunović Natalija

    2006-01-01

    Full Text Available The importance of male dominance in the scientific community is no strange to us all. Science, as a source of respected and influential information is a staunchly guarded male domain for millennia. What is specific for our time and place (the umber global zing beginning of the third millennium and a country in transition from post communism to yet to be determined brand of capitalism is the nervousness with which female presence is accepted. It is also the time of great changes in the axis of power, and the struggle for control is very aggressive. What is even more so in Serbia and Montenegro one of the pieces of ruins of the late SFRJ, is that the dysfunction of the war and sanctions brought about more inequality than before. And science is a field of professional achievement with slow and uncertain rewards, vast investment of time, effort and funds, for a career that is beneficial more to vanity than to the bank account. Women are seen as frivolous when they devote a huge amount of work and other resources to self-improvement and personal achievement and not to the comfort of the immediate family.

  20. Exploring Critical Alternatives for Youth Development through Lifestyle Sport: Surfing and Community Development in Aotearoa/New Zealand

    Directory of Open Access Journals (Sweden)

    Belinda Wheaton

    2017-12-01

    Full Text Available While competition-based team sports remain dominant in community and sport-for-development programs, researchers are exploring the value of alternative, less “sportized” activities such as lifestyle/action sports. In this paper, we explore the ways in which surfing is being used in development programs in Aotearoa/New Zealand, examining the perceived social benefits and impact. Our methods involved: (a mapping the range of surfing projects; and (b 8 in-depth interviews with program personnel. Widespread conviction in the positive developmental benefits of surfing was evident, and that surfing had a “special” capacity to reform or heal those who participate in it. However, the ways in which individuals’ self-developments were promoted appear to be following the traditional sport/youth development path. They focus on policies aimed at improved life chances, equipping youth with the tools for self-improvement and self-management, inculcating self-governance and self-reliance. However, a counter narrative co-existed, highlighting surfing as a freeing experience, which, rather than restoring social order, works to instigate a personal transformation or awakening. Despite the range of challenges presented by surfing as a tool for positive development, surfing presents a potentially “critical alternative” which if sport-for-development programs are to be a form of social change, we should remain open to exploring.

  1. Asian educational discourse: construction of ontological security

    Directory of Open Access Journals (Sweden)

    Natalya V. Khalina

    2018-03-01

    Full Text Available This article considers the problem of ontology security through Asian educational discourse, which is structurally determined by the process of moral self-improvement. Considered are trends in improving the management of educational system by developing the culture of quality, which is considered as the next stage of the Asian education systems development after the “quality of education” stage. We suggest an approach for assessing the vitality of educational process and its product based on monitoring trainees’ aptitudes system and school capabilities in developing and maintaining this system. In this study we refer to the concept of vitality and viability when describing the general theory of viability in connection with the core principles of Asian educational discourse. We outline main trends in the development of modern educational system in Asian university given the process of globalization and its impact on educational reforms in the Asia-Pacific region. Thus, the category of education quality in Asian system of higher education and narrative monitoring of Chinese students’ cognitive structures viability at Altai State University are introduced.

  2. Transformational Leadership in the City of Yogyakarta During Herry Zudianto’s Era

    Directory of Open Access Journals (Sweden)

    Erni Zuhriyati

    2014-08-01

    Full Text Available Political and governmental transformation that took place after the 1998 reformation brought the necessity for the bureaucracy to make self improvement. Bureaucratic performance in providing public services became the main focus. In this framework, leadership is the significant factor in performing a series of government innovation. This study aims to describe the implementation of transformational leadership to create an innovative government in Yogyakarta during Herry Zudianto’s Era. The category of this study is a qualitative research. The data used is primary data that was obtained by interview, questionnaire, and observation, and secondary data obtained with the technique of data collection documentation. The results showed that Herry Zudianto has the criteria of transformative leadership in doing government innovation with characteristics of the four dimensions, namely idealized influence, inspirational motivation, intellectual stimulation and individualized consideration. Transformational leadership by contextual style according to the demands of the contemporary socio-politics has implications for the occurrence of a change of two organizational entities, namely organizational culture and innovation in policy implementation.

  3. Positive and Negative Impacts of Cross-border M&A

    Institute of Scientific and Technical Information of China (English)

    裴长洪; 林江

    2007-01-01

    Mergers and acquisitions of Chinese enterprises by foreign investors have moved onto the public radar in recent years.To date,the M&A frenzy has drawn widespread attention,with a mixed reaction from proponents and opponents.Proponents consider such mergers and acquisitions conducive to realizing strategic readjustment of the national economic structure,optimizing resource allocation and improving the corporate governance structure.Opponents,however,are concerned that foreign mergers and acquisitions may jeopardize China’s industrial security and erode the executive power of the central government in undertaking industrial development planning.Are the benefits of M&A outweighed by the costs,or vice versa? The focus column of this edition features two articles which debate this issue from opposing viewpoints.In the article"Positive and Negative Impacts of Cross-border M&A",the authors consider foreign M(?)A to be a new way of boosting the level of foreign investment utilization,and advocate China taking full advantage of this approach.The authors of the article"Self-Improvement Or Self-Mutilation",meanwhile,hold foreign M&A to blame for state-owned asset erosion,and insist that China should oppose mergers and acquisitions of key state- owned enterprises by foreign investors at fire-sale prices.

  4. Perception of Shame in Otolaryngology-Head and Neck Surgery Training.

    Science.gov (United States)

    McMains, Kevin Christopher; Peel, Jennifer; Weitzel, Erik K; Der-Torossian, Hirak; Couch, Marion

    2015-11-01

    This survey was developed to assess the prevalence and effects of the perception of shame in otolaryngology-head and neck surgery residency training in the United States. Survey. US otolaryngology training programs. Faculty and trainees in US otolaryngology training programs. A 14-item survey to assess the prevalence of the experience of shame and the attitudes toward use of shame in otolaryngology residency training was sent to all otolaryngology-head and neck surgery program directors for distribution among their respective faculty and resident cohorts. A total of 267 responses were received (women, 24.7%; men, 75.3%): 42.7% of respondents were trainees; 7.0% of trainees thought that shame was a necessary/effective tool, compared with 11.4% of faculty; 50% of respondents felt that they had been personally shamed during residency; and 69.9% of respondents had witnessed another trainee being shamed during residency training. Trainees were most commonly shamed in the operating room (78.4%). Otolaryngology faculty members did the shaming 95.1% of the time. Although shaming prompted internal reflection/self-improvement in 57.4% of trainees, it also caused loss of self-confidence in 52.5%. Trainees who had been shamed were more likely to view shame as an appropriate educational tool (P Otolaryngology—Head and Neck Surgery Foundation 2015.

  5. An information technology framework for strengthening telehealthcare service delivery.

    Science.gov (United States)

    Chen, Li-Chin; Chen, Chi-Wen; Weng, Yung-Ching; Shang, Rung-Ji; Yu, Hui-Chu; Chung, Yufang; Lai, Feipei

    2012-10-01

    Telehealthcare has been used to provide healthcare service, and information technology infrastructure appears to be essential while providing telehealthcare service. Insufficiencies have been identified, such as lack of integration, need of accommodation of diverse biometric sensors, and accessing diverse networks as different houses have varying facilities, which challenge the promotion of telehealthcare. This study designs an information technology framework to strengthen telehealthcare delivery. The proposed framework consists of a system architecture design and a network transmission design. The aim of the framework is to integrate data from existing information systems, to adopt medical informatics standards, to integrate diverse biometric sensors, and to provide different data transmission networks to support a patient's house network despite the facilities. The proposed framework has been evaluated with a case study of two telehealthcare programs, with and without the adoption of the framework. The proposed framework facilitates the functionality of the program and enables steady patient enrollments. The overall patient participations are increased, and the patient outcomes appear positive. The attitudes toward the service and self-improvement also are positive. The findings of this study add up to the construction of a telehealthcare system. Implementing the proposed framework further assists the functionality of the service and enhances the availability of the service and patient acceptances.

  6. Nonlinear Control of an Active Magnetic Bearing System Achieved Using a Fuzzy Control with Radial Basis Function Neural Network

    Directory of Open Access Journals (Sweden)

    Seng-Chi Chen

    2014-01-01

    Full Text Available Studies on active magnetic bearing (AMB systems are increasing in popularity and practical applications. Magnetic bearings cause less noise, friction, and vibration than the conventional mechanical bearings; however, the control of AMB systems requires further investigation. The magnetic force has a highly nonlinear relation to the control current and the air gap. This paper proposes an intelligent control method for positioning an AMB system that uses a neural fuzzy controller (NFC. The mathematical model of an AMB system comprises identification followed by collection of information from this system. A fuzzy logic controller (FLC, the parameters of which are adjusted using a radial basis function neural network (RBFNN, is applied to the unbalanced vibration in an AMB system. The AMB system exhibited a satisfactory control performance, with low overshoot, and produced improved transient and steady-state responses under various operating conditions. The NFC has been verified on a prototype AMB system. The proposed controller can be feasibly applied to AMB systems exposed to various external disturbances; demonstrating the effectiveness of the NFC with self-learning and self-improving capacities is proven.

  7. Long-term outcomes of Class III treatment with mandibular cervical headgear followed by fixed appliances.

    Science.gov (United States)

    Baccetti, Tiziano; Rey, Diego; Oberti, Giovanni; Stahl, Franka; McNamara, James A

    2009-09-01

    To evaluate the stability of the outcomes of mandibular cervical headgear (MCH) and fixed appliance-treated Class III patients at a long-term posttreatment (5 years) observation, compared with well-matched untreated Class III controls, following a previous report on the short-term outcomes of this protocol. The treated group consisted of 20 patients with dentoskeletal Class III malocclusions treated with a two-phase protocol consisting of MCH and fixed appliances, while the control group comprised 18 untreated subjects with similar dentoskeletal Class III malocclusion. Lateral cephalograms of both patients and controls were analyzed at two time points: posttreatment (PT), after two-phase treatment; and long term (LT). All patients were at a postpubertal stage of skeletal maturity at PT, and they showed CS6 at LT, thus revealing completion of pubertal craniofacial growth. In the long term, the treatment group showed significantly smaller values for mandibular length (Co-Gn), SNB angle, maxillomandibular differential, and molar relation. When compared with the controls, the treated patients exhibited also greater values for ANB angle, Wits appraisal, and overjet at LT. No significant difference between the two groups was found for the changes occurring from PT to LT. Favorable dentoskeletal outcomes induced by MCH and fixed appliances remained stable in the long term; untreated Class III malocclusion did not show any tendency toward self-improvement during the postpubertal interval.

  8. Performance indicators for nuclear medicine and industrial radiographers. Final report

    International Nuclear Information System (INIS)

    Hill, T.D.

    1997-01-01

    HCA--Assessment Experts (HCA) was retained under contract to provide evidence that a behaviourally based approach to the development of performance indicators for radioisotope users could be successfully designed, implemented and rapidly delivered to a pilot sample. Moreover, HCA believed that it was uniquely qualified to not only achieve this success, but to show further that we could instill the motivation for self-improvement in the AECB inspection ratings of Licensees and Permit Holders. In the space of about ten weeks. HCA was able to deliver a comprehensive set of web-based tools for performance indicators. Not only did we deliver these tools, but we also included such supplemental information as relevant legislation, regulations. Inspectors' preferences and recommendations, among others, so as to foster a learning component of the performance indicators tools. The call for the continuation of this work is based on two sources. The response from participants to this project was very favourable - participants want these tools. Secondly, our research and experience have shown (and the larger body of empirical research also shows) that this is the type of performance feedback and communication that participants appreciate the most, and is the most predictive of successful compliance and improvement in the future. (author)

  9. KONTRIBUSI PENGGUNAAN JURNAL BELAJAR PADA PEMBELAJARAN MATAKULIAH PERMAINAN BOLABASKET

    Directory of Open Access Journals (Sweden)

    Fitria Dwi Andriyani

    2017-02-01

    THE CONTRIBUTION OF THE STUDENT’S JOURNAL TOWARD THE INSTRUCTIONAL PROCESS OF BASKETBALL GAMES COURSE   Abstract: The purpose of this study was to investigate the contribution of the student’s journal toward the instructional process of Basketball Games Course. This research was a descriptive research and conducted by applied the student’s journal after the learning process. The population of the research was the students of Class C Prodi PJKR FIK UNY which took part on the Basketball Games course on year 2014/2015. There were 46 students participated on this study. The research instruments on this study were the questions list of student’s journal and questionnaire of writing journal experience. The result revealed that the participants recognized the positive influence of the using of the student’s journal (100%. There wete 95.65% participants who have a positive opinion and feeling toward the journal writing. In addition, participants stated that the student’s journal facilitates to perform self reflection, memorize the material, realize the ability improvement, elevate the ability to understand the material, motivate for self improvement, and increase the knowledge. Our discussions conclude that the student’s journal writing has a positive and meaningful value toward the learning process. However, it needs a specific strategy to implement the journal so that it can give a real benefit for teachers and students.   Keywords: student’s journal, instructional, college students

  10. Prioritize Improvement Opportunities Identified In Self-Assessment Using Multi-Criteria Fuzzy Group Decision

    Directory of Open Access Journals (Sweden)

    Dr.Ghassem Faraj Pour

    2015-05-01

    Full Text Available ABSTRACT Efforts to improve the quality are one of the prerequisites for the success of individual companies and for the competitiveness of all whole companies. In the field of improvement and excellence business excellence models answer to the question that what the better organization is what goals and concepts they follow and according to what standards they behave. The EFQM excellence model can be transition from multiplicity to unity of different existing models. The most important approaches of these models are self-assessment and identifying improvement areas in an organization. On the other side organizations which are at lower level of total quality management will encounter so many areas to improve when using this model and implementing of self-improvement. Choosing the most important key problems are always the main challenges and because of resource constraints and strategic goals organizations have to prioritize identified improvement opportunities. This paper introduces a model for prioritizing and choosing the most significant improvement opportunities using the organization Business Excellence team members and because the analysis and decision making atmosphere for excellence team members is not generally complete with accurate information it seems using of fuzzy decision can be very helpful.

  11. From Retreat Center to Clinic to Boardroom? Perils and Promises of the Modern Mindfulness Movement

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    Norman A. S. Farb

    2014-11-01

    Full Text Available From its venerable Buddhist roots, mindfulness training (MT has spread rapidly across the globe in the past few decades due to its strong salutary claim, i.e., the notion that meditation practice is an efficacious means for self-improvement. However, concerns have arisen that the appropriation of MT techniques from classical Buddhist tradition into modern secular practice has diluted the benefits of these practices. The “great danger” to the movement is that inadequately adapted MT techniques, combined with unreasonable inflation of expectations regarding MT’s benefits, may undermine MT’s true potential to effect positive change in the world. And yet, these concerns can be mitigated by consideration of the salutary claim as a persistent “quality check” on MT efficacy. It is argued that scientific investigation can take an important role in delineating the necessary characteristics for fulfilling mindfulness’ salutary claim, as well as identifying contraindicated techniques and risk factors for training. By accepting that we cannot control the spread of MT into commercial domains, researchers may still work to distinguish “right” from “wrong” mindfulness through empirical study. In this way, modern science may help to realize the salutary claim and even contribute to classical Buddhist conceptions of mindfulness, advancing our understanding of how best to promote well-being.

  12. Current status of yoga in mental health services.

    Science.gov (United States)

    Varambally, Shivarama; Gangadhar, B N

    2016-06-01

    Yoga (derived from 'yuj' which means to yoke together or unite) has been used for millennia as a tool for self-improvement, with the ultimate goal of uniting the individual consciousness with the universal. The physical elements of yoga, although seen as necessary in the path to achieve the goal, they were not considered as the endpoint for a practitioner. Sage Patanjali, who codified the practices into an eight-limbed model (Ashtanga yoga) in the Patanjali Yoga Sutras, makes it clear that the target of yoga is primarily the mind. However, in the modern world, yoga practices have become immensely popular as aids to improve health. Yoga-based practices are being extensively used as therapeutic ingredients, alone or as adjuncts to other therapies in a variety of disorders, both physical and mental. There is now strong evidence to suggest that yoga-based interventions are beneficial in several lifestyle disorders. Recent research has also shown significant benefits in mental disorders such as depression, anxiety, and psychosis. This paper discusses the place of yoga as one of the therapeutic strategies in the holistic approach to mental disorders, and the challenges inherent to research in this area.

  13. Health education needs of intimate partner violence survivors: Perspectives from female survivors and social service providers.

    Science.gov (United States)

    Ferranti, Dina; Lorenzo, Dalia; Munoz-Rojas, Derby; Gonzalez-Guarda, Rosa M

    2018-03-01

    To explore the health education needs and learning preferences of female intimate partner violence (IPV) survivors in a social service agency located in South Florida, United States. An exploratory two-phase sequential mixed-methods study was completed through semistructured interviews with social service providers (n = 10), followed by a survey with predominately female IPV survivors (n = 122, 98.4%). Data obtained from interviews with social service providers were analyzed through conventional thematic content analysis. Data from interviews were used in developing a health survey completed by IPV survivors and analyzed utilizing descriptive statistics, chi-square tests and t tests. Three themes emerged from interviews including multidimensional health needs, navigating barriers to health care, and self-improvement specific to survivors of intimate partner violence. Survey results indicated that depression and self-esteem were the health education needs of highest priority. Demographic characteristics, including age and language use, were significantly associated to preferred methods of learning, p education needs. Current study findings can inform public health nurses in developing interventions or health-based programs for female IPV survivors in social service agency settings. © 2017 Wiley Periodicals, Inc.

  14. The origin of the aesthetic elements in traditional forms of Far-eastern martial arts

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    Aleksandar Filipović

    2016-02-01

    Full Text Available In light of the fact that Far-eastern martial arts have to be viewed as an inseparable part of the cultural traditions of China and Japan, their aesthetic character is tied to traditional cultural concepts which dominate these cultures, such as the concept of the unity of opposites, better known as yin and yang; the concept of the Road (the Tao as a symbol of continuing self-improvement; the concept of Chi or Qui energy which permeates the cosmos as well as individual beings, etc. These concepts owe their existence and development first and foremost to the religious and philosophical systems which shaped and influenced all segments of these two great cultures, and not just the martial tradition. On the other hand, performance arts weren’t spared this same influence. Performance arts and martial arts of the Far East had an intertwined history and strongly influenced each other. The aim of this paper will be to give an outline of the basic concepts connected to the aesthetics of the martial arts of China and Japan and point out the similar roots in other segments of cultural tradition.

  15. Principles and practice of external digital photography in ophthalmology

    Science.gov (United States)

    Mukherjee, Bipasha; Nair, Akshay Gopinathan

    2012-01-01

    It is mandatory to incorporate clinical photography in an ophthalmic practice. Patient photographs are routinely used in teaching, presentations, documenting surgical outcomes and marketing. Standardized clinical photographs are part of an armamentarium for any ophthalmologist interested in enhancing his or her practice. Unfortunately, many clinicians still avoid taking patient photographs for want of basic knowledge or inclination. The ubiquitous presence of the digital camera and digital technology has made it extremely easy and affordable to take high-quality images. It is not compulsory to employ a professional photographer or invest in expensive equipment any longer for this purpose. Any ophthalmologist should be able to take clinical photographs in his/her office settings with minimal technical skill. The purpose of this article is to provide an ophthalmic surgeon with guidelines to achieve standardized photographic views for specific procedures, to achieve consistency, to help in pre-operative planning and to produce accurate pre-operative and post-operative comparisons, which will aid in self-improvement, patient education, medicolegal documentation and publications. This review also discusses editing, storage, patient consent, medicolegal issues and importance of maintenance of patient confidentiality. PMID:22446907

  16. Undulations on the surface of elongated bubbles in confined gas-liquid flows

    Science.gov (United States)

    Magnini, M.; Ferrari, A.; Thome, J. R.; Stone, H. A.

    2017-08-01

    A systematic analysis is presented of the undulations appearing on the surface of long bubbles in confined gas-liquid flows. CFD simulations of the flow are performed with a self-improved version of the open-source solver ESI OpenFOAM (release 2.3.1), for Ca =0.002 -0.1 and Re =0.1 -1000 , where Ca =μ U /σ and Re =2 ρ U R /μ , with μ and ρ being, respectively, the viscosity and density of the liquid, σ the surface tension, U the bubble velocity, and R the tube radius. A model, based on an extension of the classical axisymmetric Bretherton theory, accounting for inertia and for the curvature of the tube's wall, is adopted to better understand the CFD results. The thickness of the liquid film, and the wavelength and decay rate of the undulations extracted from the CFD simulations, agree well with those obtained with the theoretical model. Inertial effects appear when the Weber number of the flow We =Ca Re =O (10-1) and are manifest by a larger number of undulation crests that become evident on the surface of the rear meniscus of the bubble. This study demonstrates that the necessary bubble length for a flat liquid film region to exist between the rear and front menisci rapidly increases above 10 R when Ca >0.01 and the value of the Reynolds number approaches 1000.

  17. Dynamics of the liquid film around elongated bubbles rising in vertical capillaries

    Science.gov (United States)

    Magnini, Mirco; Khodaparast, Sepideh; Matar, Omar K.; Stone, Howard A.; Thome, John R.

    2017-11-01

    We performed a theoretical, numerical and experimental study on elongated bubbles rising in vertical tubes in co-current liquid flows. The flow conditions were characterized by capillary, Reynolds and Bond numbers within the range of Ca = 0.005 - 0.1 , Re = 1 - 2000 and Bo = 0 - 20 . Direct numerical simulations of the two-phase flows are run with a self-improved version of OpenFOAM, implementing a coupled Level Set and Volume of Fluid method. A theoretical model based on an extension of the traditional Bretherton theory, accounting for inertia and the gravity force, is developed to obtain predictions of the profiles of the front and rear menisci of the bubble, liquid film thickness and bubble velocity. Different from the traditional theory for bubbles rising in a stagnant liquid, the gravity force impacts the flow already when Bo < 4 . Gravity effects speed up the bubble compared to the Bo = 0 case, making the liquid film thicker and reducing the amplitude of the undulation on the surface of the bubble near its tail. Gravity effects are more apparent in the visco-capillary regime, i.e. when the Reynolds number is below 1.

  18. The Autonomous Maintenance Implementation Directory as a Step Toward the Intelligent Quality Management System

    Science.gov (United States)

    Molenda, Michał

    2016-12-01

    The article describes the effects of the improvement of the production process which one of the industrial enterprises obtained by implementing the method of Autonomous Maintenance (AM), which is one of the pillars of the concept of Total Productive Maintenance (TPM). AM method was presented as an aid to the formation of intelligent, self-improving procesess of the quality management system (QMS). The main part of this article is to present results of studies that have been conducted in one of the large industrial enterprises in Poland, manufacturing for the automotive industry. The aim of the study was to evaluate the effectiveness of the implementation of the AM method as a tool for selfimprovement of industrial processes in the following company. The study was conducted in 2015. The gathering and comparison of data from the period of two years, ie. the year before and the year after the implementation of AM, helped to determine the effectiveness of AM in building intelligent quality management system.

  19. A Qualitative Study to Examine Feasibility and Design of an Online Social Networking Intervention to Increase Physical Activity in Teenage Girls.

    Directory of Open Access Journals (Sweden)

    Gisela Van Kessel

    Full Text Available Online social networks present wide-reaching and flexible platforms through which to deliver health interventions to targeted populations. This study used a social marketing approach to explore teenage girls' perceptions of physical activity and the potential use of online social networks to receive a physical activity intervention.Six focus groups were conducted with 19 Australian teenage girls (ages 13 to 18 years with varying levels of physical activity and socioeconomic status. A semi-structured format was used, with groups discussion transcribed verbatim. Content analysis identified emergent themes, with triangulation and memos used to ensure accuracy.Physical activity was most appealing when it emphasised sport, exercise and fitness, along with opportunities for socialisation with friends and self-improvement. Participants were receptive to delivery of a physical activity intervention via online social networks, with Facebook the most widely reported site. Participants commonly accessed online social networks via mobile devices and particularly smartphones. Undesirable features included promotion of physical activity in terms of walking; use of cartoon imagery; use of humour; and promotion of the intervention via schools, each of which were considered "uncool". Participants noted that their parents were likely to be supportive of them using an online social networking physical activity intervention, particularly if not promoted as a weight loss intervention.This study identified key features likely to increase the feasibility and retention of an online social networking physical activity intervention for teenage girls. Guidelines for the design of interventions for teenage girls are provided for future applications.

  20. An acoustic energy framework for predicting combustion-driven acoustic instabilities in premixed gas-turbines

    Science.gov (United States)

    Ibrahim, Zuhair M. A.

    The purpose of this study was to discover and assess student financial services delivered to students enrolled at East Tennessee State University. The research was undertaken for institutional self-improvement. The research explored changes that have occurred in student financial services in the dynamic higher education market. The research revealed universities pursued best practices for the delivery of student financial services through expanded employee knowledge, restructured organizations, and integrated information technologies. The research was conducted during October and November, 2006. The data were gathered from an online student survey of student financial services. The areas researched included: the Bursar office, the Financial Aid office, and online services. The results of the data analysis revealed problems with the students' perceived quality of existing financial services and the additional services students desire. The research focused on student perceptions of the quality of financial services by age and gender classifications and response categories. Although no statistically significant difference was found between the age-gender classifications on the perception of the quality of the financial services studied, the research adds to our understanding of student financial services at East Tennessee State University. Recommendation for continued research included annual surveys of segmented student populations that include ethnicity, age, gender, and educational level. The research would be used for continuous improvement efforts and student relationship management. Also additional research was recommended for employee learning in relation to the institution's mission, goals, and values.

  1. Spiritual practices of taoism

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    Yulia L. Butko

    2016-01-01

    Full Text Available In the article the spiritual practices of Taoism. Established that the spiritual path in Taoism is the main ultimate goal - achieving eternal, indestructible personality transformation through meditation and the reduction of lower mental strength to their upper classes. To achieve this, the Taoist practices, special practices that include, along with the meditative contemplation technique classes and various gymnastics, breathing exercises and the like, and (for a significant period of its history - Laboratory (“foreign” alchemy. Among the spiritual practices of Taoism is the main meditation that has little to do with certain external techniques. Taoist meditation leads people to unity, the only person that connects with the cosmos and society. The author concluded that the path of self-improvement Taoist, under the guidance of a teacher, is a series of distinct stages, gaining purely individual instruction. Spiritual practices like Taoist, were widely known in other religious and philosophical systems. However, the semantics of Taoist practices are significantly different, as well as their function in the structure of religious practices in general.

  2. An integrated approach for improving occupational health and safety management: the voluntary protection program in Taiwan.

    Science.gov (United States)

    Su, Teh-Sheng; Tsai, Way-Yi; Yu, Yi-Chun

    2005-05-01

    A voluntary compliance program for occupational health and safety management, Voluntary Protection Programs (VPP), was implemented with a strategy of cooperation and encouragement in Taiwan. Due to limitations on increasing the human forces of inspection, a regulatory-based guideline addressing the essence of Occupational Health and Safety Management Systems (OHSMS) was promulgated, which combined the resources of third parties and insurance providers to accredit a self-improving worksite with the benefits of waived general inspection and a merit contributing to insurance premium payment reduction. A designated institute accepts enterprise's applications, performs document review and organizes the onsite inspection. A final review committee of Council of Labor Affairs (CLA) confers a two-year certificate on an approved site. After ten years, the efforts have shown a dramatic reduction of occupational injuries and illness in the total number of 724 worksites granted certification. VPP worksites, in comparison with all industries, had 49% lower frequency rate in the past three years. The severity rate reduction was 80% in the same period. The characteristics of Taiwan VPP program and international occupational safety and health management programs are provided. A Plan-Do-Check-Act management cycle was employed for pursuing continual improvements to the culture fostered. The use of a quantitative measurement for assessing the performance of enterprises' occupational safety and health management showed the efficiency of the rating. The results demonstrate that an employer voluntary protection program is a promising strategy for a developing country.

  3. Mass sports of students and cadets which server military contract in positions of officers

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    Anastasia Bondar

    2015-12-01

    Full Text Available Purpose: study of features of organization of mass sports of students and cadets which server military contract in positions of officers. Material and Methods: analysis of literary sources and documents, analysis of the systems, questioning (questionnaire, methods of the mathematical processing of data. In a questionnaire 97 respondents – 29 cadets of a 5 course and 68 students of 4 courses of military-legal faculty of the Yaroslav Mudryi National law university. Results: 69% cadets and only 35% students are engaged in the different types of motive activity in free time, here 48% cadets and 43% students elect the playing types of sport (football, volley-ball, basket-ball, 65,5% cadets and 48,5% students go in for sports for self-perfection and self-realization, here 45% cadets and 32% students plan necessarily to prolong to be engaged in the select type of sport upon termination of studies in higher educational establishment. Conclusions: the Study 4th Year Students And Students Of The 5th Year Of Military Faculty Of Law Has Shown That The Students Serious About Their Chosen Profession And Understand The Importance Of Physical Fitness In A Future Service, They Are Able To Objectively Assess The Level Of Their Physical Readiness And Are Ready For Further Self-Improvement

  4. Superhuman AI for heads-up no-limit poker: Libratus beats top professionals.

    Science.gov (United States)

    Brown, Noam; Sandholm, Tuomas

    2018-01-26

    No-limit Texas hold'em is the most popular form of poker. Despite artificial intelligence (AI) successes in perfect-information games, the private information and massive game tree have made no-limit poker difficult to tackle. We present Libratus, an AI that, in a 120,000-hand competition, defeated four top human specialist professionals in heads-up no-limit Texas hold'em, the leading benchmark and long-standing challenge problem in imperfect-information game solving. Our game-theoretic approach features application-independent techniques: an algorithm for computing a blueprint for the overall strategy, an algorithm that fleshes out the details of the strategy for subgames that are reached during play, and a self-improver algorithm that fixes potential weaknesses that opponents have identified in the blueprint strategy. Copyright © 2018, The Authors, some rights reserved; exclusive licensee American Association for the Advancement of Science. No claim to original U.S. Government Works.

  5. Source memory that encoding was self-referential: the influence of stimulus characteristics.

    Science.gov (United States)

    Durbin, Kelly A; Mitchell, Karen J; Johnson, Marcia K

    2017-10-01

    Decades of research suggest that encoding information with respect to the self improves memory (self-reference effect, SRE) for items (item SRE). The current study focused on how processing information in reference to the self affects source memory for whether an item was self-referentially processed (a source SRE). Participants self-referentially or non-self-referentially encoded words (Experiment 1) or pictures (Experiment 2) that varied in valence (positive, negative, neutral). Relative to non-self-referential processing, self-referential processing enhanced item recognition for all stimulus types (an item SRE), but it only enhanced source memory for positive words (a source SRE). In fact, source memory for negative and neutral pictures was worse for items processed self-referentially than non-self-referentially. Together, the results suggest that item SRE and source SRE (e.g., remembering an item was encoded self-referentially) are not necessarily the same across stimulus types (e.g., words, pictures; positive, negative). While an item SRE may depend on the overall likelihood the item generates any association, the enhancing effects of self-referential processing on source memory for self-referential encoding may depend on how embedded a stimulus becomes in one's self-schema, and that depends, in part, on the stimulus' valence and format. Self-relevance ratings during encoding provide converging evidence for this interpretation.

  6. Factors Relating to Self-Efficacy Among Psychiatric Nurses.

    Science.gov (United States)

    Yada, Hironori; Kobayashi, Mako; Odachi, Ryo; Yamane, Toshie

    This study aimed to clarify the factors related to self-efficacy experienced by psychiatric nurses. Analysis of qualitative descriptive data from a free self-description questionnaire administered to 16 psychiatric nurses working in psychiatric hospitals revealed 24 codes across the following 8 categories as factors that increase self-efficacy: A1. possibility of practical use in nursing, A2. nursing judgment, A3. improvement of psychiatric symptoms, A4. the patients presenting a positive attitude, A5. building a relationship of trust with the patients, A6. building a relationship of trust with other nurses, A7. work progressing according to plan and A8. team medical practice. Twenty-five codes across the following 10 categories were identified as factors that decrease self-efficacy: B1. lack of communication, B2. uncertainty in caregiving, B3. recurrence of psychiatric symptoms, B4. feeling overpowered by a patient, B5. sense of being too busy to work adequately, B6. difficulty in bringing about self-improvement, B7. sense of loss regarding one's role as a nurse, B8. lack of physical strength, B9. mechanical performance of nursing and B10. fluctuating view of nursing due to mistakes. These factors require intervention for psychiatric nurses' self-efficacy.

  7. Licensee performance evaluation. Phase 1 technical report, November 1977--August 1978

    International Nuclear Information System (INIS)

    Chakoff, H.E.; Speaker, D.M.; Thompson, S.R.; Cohen, S.C.

    1978-10-01

    The report documents a methodology that NRC may use to evaluate the regulatory performance of its licensees. The purpose of such an evaluation is to permit NRC to identify those licensees whose regulatory performance is marginal. The methodology proposed in this report emphasizes the detailed analysis of the content and trends in Licensee Event Reports (LERs) submitted by licensees. In principle, the methodology can be applied to any class of NRC licensees; however, performance data is relatively sparse for licensee classes other than operating power reactors. Case studies applying the methodology to the examination of three operating power reactor licensees in NRC's Region III suggests that the methodology may permit NRC to identify repetitive, or casually-linked, events that can be attributed to licensee human error. This information will allow NRC to deal with licensees and regulatory problems in a uniform, consistent, and timely manner; permit more effective and efficient use of NRC inspection resources; and provide better feedback to licensees to facilitate their self-improvement efforts. The three case studies tend to confirm previously suspected differences in the regulatory performance of the three licensees

  8. Development of Sustainability Assessment Tool for Malaysian hydropower industry: A case study

    Science.gov (United States)

    Turan, Faiz Mohd; Johan, Kartina; Abu Sofian, Muhammad Irfan

    2018-04-01

    This research deals with the development of sustainability assessment tools as a medium to assess the performance of a hydropower project compliances towards sustainability practice. Since the increasing needs of implementing sustainability practice, developed countries are utilizing sustainability tools to achieve sustainable development goals. Its inception within ASEAN countries including Malaysia is still low. The problem with most tools developed from other countries is that it is not very comprehensive as well as its implementation factors are not suitable for the local environment that is not quantified. Hence, there is a need to develop a suitable sustainable assessment tool for the Malaysian hydropower industry to comply with the sustainable development goals as a bridging gap between the governor and the practitioner. The steps of achieving this goal is separated into several parts. The first part is to identify sustainable parameters from established tools as a model for comparison to enhance new parameters. The second stage is to convert equivalent quantification value from the model to the new developed tools. The last stage is to develop software program as a mean of gaining energy company feedback with systematic sustainable reporting from the surveyor so as to be able to integrate sustainability assessment, monitoring and reporting for self-improved reporting.

  9. Topical tacrolimus for atopic dermatitis.

    Science.gov (United States)

    Cury Martins, Jade; Martins, Ciro; Aoki, Valeria; Gois, Aecio F T; Ishii, Henrique A; da Silva, Edina M K

    2015-07-01

    .98, 1 study, n = 139, low-quality evidence), but the effects were equivocal when evaluating BSA. In the comparison of tacrolimus 0.03% with moderate-to-potent corticosteroids, no difference was found in most of the outcomes measured (including physician's and participant's assessment and also for the secondary outcomes), but in two studies, a marginal benefit favouring the corticosteroid group was found for the EASI and BSA scores.Burning was more frequent in those using calcineurin inhibitors than those using corticosteroid tacrolimus 0.03% (RR 2.48, 95% CI 1.96 to 3.14, 5 studies, 1883 participants, high-quality evidence), but no difference was found for skin infections. Symptoms observed were mild and transient. The comparison between the two calcineurin inhibitors (pimecrolimus and tacrolimus) showed the same overall incidence of adverse events, but with a small difference in the frequency of local effects.Serious adverse events were rare; occurred in both the tacrolimus and corticosteroid groups; and in most cases, were considered to be unrelated to the treatment. No cases of lymphoma were noted in the included studies nor in the non-comparative studies. Cases were only noted in spontaneous reports, cohorts, and case-control studies. Systemic absorption was rarely detectable, only in low levels, and this decreased with time. Exception is made for diseases with severe barrier defects, such as Netherton's syndrome, lamellar ichthyosis, and a few others, with case reports of a higher absorption. We evaluated clinical trials; case reports; and in vivo, in vitro, and animal studies; and didn't find any evidence that topical tacrolimus could cause skin atrophy. Tacrolimus 0.1% was better than low-potency corticosteroids, pimecrolimus 1%, and tacrolimus 0.03%. Results were equivocal when comparing both dose formulations to moderate-to-potent corticosteroids. Tacrolimus 0.03% was superior to mild corticosteroids and pimecrolimus. Both tacrolimus formulations seemed to be

  10. Identifying emotional intelligence skills of Turkish clinical nurses according to sociodemographic and professional variables.

    Science.gov (United States)

    Kahraman, Nilgün; Hiçdurmaz, Duygu

    2016-04-01

    This study aimed to identify the emotional intelligence skills of Turkish clinical nurses according to sociodemographic and professional variables. Emotional intelligence is "the ability of a person to comprehend self-emotions, to show empathy towards the feelings of others, and to control self-emotions in a way that enriches life." Nurses with a higher emotional intelligence level offer more efficient and professional care, and they accomplish more in their social and professional lives. We designed a descriptive cross-sectional study. The Introductory Information Form and the Bar-On emotional intelligence Inventory were used to collect data between 20th June and 20th August 2012. The study was conducted with 312 nurses from 37 hospitals located within the borders of the metropolitan municipality in Ankara. There were no significant differences between emotional intelligence scores of the nurses according to demographic variables such as age, gender, marital status, having children. Thus, sociodemographic factors did not appear to be key factors, but some professional variables did. Higher total emotional intelligence scores were observed in those who had 10 years or longer experience, who found oneself successful in professional life, who stated that emotional intelligence is an improvable skill and who previously received self-improvement training. Interpersonal skills were higher in those with a graduate degree and in nurses working in polyclinics and paediatric units. These findings indicate which groups require improvement in emotional intelligence skills and which skills need improvement. Additionally, these results provide knowledge and create awareness about emotional intelligence skills of nurses and the distribution of these skills according to sociodemographic and professional variables. Implementation of emotional intelligence improvement programmes targeting the determined clinical nursing groups by nursing administrations can help the increase in

  11. Diversity of activity participation determines bone mineral content in the lower limbs of pre-pubertal children with developmental coordination disorder.

    Science.gov (United States)

    Fong, S S M; Vackova, D; Choi, A W M; Cheng, Y T Y; Yam, T T T; Guo, X

    2018-04-01

    This study examined the relationships between activity participation and bone mineralization in children with developmental coordination disorder. Limited participation in physical, recreational, social, and skill-based and self-improvement activities contributed to lower bone mineral content. For improved bone health, these children should participate in a variety of activities, not only physical activities. Limited activity participation in children with developmental coordination disorder (DCD) may have a negative impact on bone mineral accrual. The objectives of this study were to compare bone mineralization and activity participation patterns of pre-pubertal children with DCD and those with typical development, and to determine the association between activity participation patterns and bone mineralization in children with DCD. Fifty-two children with DCD (mean age = 7.51 years) and 61 children with typical development (mean age = 7.22 years) participated in the study. Appendicular and total body (less head) bone mineral content (BMC) and bone mineral density (BMD) were evaluated by a whole-body dual-energy X-ray absorptiometry scan. Activity participation patterns were assessed using the Children's Assessment of Participation and Enjoyment (CAPE) questionnaire. Children with DCD had lower appendicular and total body BMCs and BMDs than children with typical development overall (p accounting for the effects of age, sex, height, lean mass, and fat mass, the total activity diversity score remained independently associated with leg BMC in children with DCD, explaining 5.1% of the variance (p = 0.030). However, the physical activity diversity score was no longer associated with leg BMC (p = 0.090). Diversity of activity participation and bone mineralization were lower in pre-pubertal children with DCD. Decreased total activity participation diversity was a contributing factor to lower BMC in the legs of children with DCD.

  12. Recurrent themes in the history of the home use of electrical stimulation: Transcranial direct current stimulation (tDCS) and the medical battery (1870-1920).

    Science.gov (United States)

    Wexler, Anna

    In recent years, neuroscientists and ethicists have warned of the dangers of the unsupervised home use of transcranial direct current stimulation (tDCS), in which individuals stimulate their own brains with low levels of electricity for self-improvement purposes. Although the home use of tDCS is often referred to as a novel phenomenon, in reality the late nineteenth and early twentieth century saw a proliferation of electrical stimulation devices for home use. In particular, the use of an object known as the medical battery bears a number of striking similarities to the modern-day use of tDCS. This article reviews a number of features thought to be unique to the present day home use of brain stimulation, with a particular focus on analogies between tDCS and the medical battery. Archival research was conducted at the Bakken Museum and at the American Medical Association's Historical Health Fraud Archives. Many of the features characterizing the contemporary home use tDCS-a do-it-yourself (DIY) movement, anti-medical establishment themes, conflicts between lay and professional usage-are a repetition of themes that occurred a century ago with regard to the medical battery. A number of features, however, seem to be unique to the present, such as the dominant discourse about risk and safety, the division between cranial and non-cranial stimulation, and utilization for cognitive enhancement purposes. Viewed in the long durée, the contemporary use of electrical stimulation at home is not a novel phenomenon, but rather the latest wave in a series of ongoing attempts by lay individuals to utilize electricity for therapeutic purposes. Copyright © 2016 Elsevier Inc. All rights reserved.

  13. ABORDĂRI TEORETICE ŞI IMPLICAŢII PRACTICE ÎN CONSILIEREA FAMILIEI CU PREADOLESCENŢI

    Directory of Open Access Journals (Sweden)

    Olga RĂILEANU

    2017-07-01

    Full Text Available Consilierea psihopedagogică a familiei cu preadolescenţi vizează un set de acţiuni speciale desfăşurate sistematic, realizate de consilier în baza abordărilor ştiinţifice şi a experienţelor avansate din domeniul consilierii psihopedagogice. În funcţie de situaţia concretă, de specificul abordărilor şi tendinţelor ştiinţifice privind formarea competenţelor parentale ale adultului şi orientarea preadolescentului spre armonizarea relaţiilor cu părinţii, consilierul îşi va individualiza inter­venţia. Prin consilierea familiei devine posibilă cultivarea membrilor ei în direcţia formării eficienţei personale în dezvol­tarea şi autoperfecţionarea personalităţii sale.THEORETICAL APPROACHES AND PRACTICAL IMPLICATIONS IN FAMILY COUNSELING WITH PRETEENSPsycho-pedagogical counseling with preteens target a range of actions carried out systematic pedagogical counselor based on scientific approaches and experiences in pedagogical counseling. Depending on the concrete situation, the specific of scientific approaches and tendencies on parental adult skills training and orientation towards harmonization preteen relationships with parents, the counselor will individualize the intervention. By providing counseling of the family members becomes possible to grow them in shaping the effectiveness of self-improvement and personal development of his personality.

  14. An investigation of motivational variables in CrossFit facilities.

    Science.gov (United States)

    Partridge, Julie A; Knapp, Bobbi A; Massengale, Brittany D

    2014-06-01

    CrossFit is a growing fitness trend in the United States; however, little systematic research has addressed specific motivational principles within this unique exercise environment. The purpose of the study was to explore the influence of gender and membership time on perceptions of motivational climate and goals within the CrossFit environment. Specifically, people may set goals related to self-improvement (i.e., mastery) or focus on their performance in comparison to others (i.e., performance). Motivational climate refers to an individual's perception of being encouraged to focus on either mastery or performance goals from CrossFit trainers. A total of 144 members (88 females; 56 males) completed questionnaires to assess participants' perceptions of CrossFit goal structures and perceptions of the motivational climate encouraged by the trainer within their CrossFit box. Results indicated a significant main effect for gender on preferred goals (p ≤ 0.05), with males reporting higher levels of performance approach goals and females reporting higher levels of master avoidance goals. Participants who reported shorter membership times were found to have significantly higher mastery-related goals than individuals who reported longer membership times (p ≤ 0.05). The results from the study suggest that practitioners should consider how perceptions of the motivational climate and goals in group-based exercise settings such as CrossFit may vary based on demographic variables, and that these differences may impact how to most effectively motivate, encourage, and instruct group members, particularly with regard to helping members set goals that most effectively address their approach to the CrossFit regimen.

  15. The Social Context of “Do-It-Yourself” Brain Stimulation: Neurohackers, Biohackers, and Lifehackers

    Directory of Open Access Journals (Sweden)

    Anna Wexler

    2017-05-01

    Full Text Available The “do-it-yourself” (DIY brain stimulation movement began in earnest in late 2011, when lay individuals began building stimulation devices and applying low levels of electricity to their heads for self-improvement purposes. To date, scholarship on the home use of brain stimulation has focused on characterizing the practices of users via quantitative and qualitative studies, and on analyzing related ethical and regulatory issues. In this perspective piece, however, I take the opposite approach: rather than viewing the home use of brain stimulation on its own, I argue that it must be understood within the context of other DIY and citizen science movements. Seen in this light, the home use of brain stimulation is only a small part of the “neurohacking” movement, which is comprised of individuals attempting to optimize their brains to achieve enhanced performance. Neurohacking itself is an offshoot of the “life hacking” (or “quantified self” movement, in which individuals self-track minute aspects of their daily lives in order to enhance productivity or performance. Additionally, the home or DIY use of brain stimulation is in many ways parallel to the DIY Biology (or “biohacking” movement, which seeks to democratize tools of scientific experimentation. Here, I describe the place of the home use of brain stimulation with regard to neurohackers, lifehackers, and biohackers, and suggest that a policy approach for the home use of brain stimulation should have an appreciation both of individual motivations as well as the broader social context of the movement itself.

  16. Contradiction between emphasizing creativity in theory and stifling it in practice

    Directory of Open Access Journals (Sweden)

    Milivojević Tatjana

    2017-01-01

    Full Text Available The word 'creativity', like other frequently used and popular words, is at risk of becoming a vague platitude or a slogan devoid of its conceptual determination. The knowledge and understanding of the phenomenon of creativity are not proportional to how much it is affirmed and promoted now days. This is particularly noticeable in work organizations of all kinds. Besides the subjective factors such as the employers' and managers' superficial or inadequate knowledge of what creativity really is, there are objective factors which put pressure on and hinder the creative impulses and processes. Economic, financial and market imperatives such as: fierce competition, short-term goals and deadlines, acceleration of work processes, rationalization, productivism, overall quantification (only measurable parameters and results being taken into account, excessive regulations, tight control systems and the like, contribute to the design of organizations which crush creativity. Some quite widespread management practices and behaviors, especially the so-called intrusive or micromanagement, are often a reaction to these constraints. As for the employees, they internalize all these pressures into strong inner imperatives of (overachievement. The imposed standard of a constantly high level of motivation, excellence and self-improvement is unsustainable in the long run. The guiding management principle of performing more and better for less (time, resources, energy, leads to the exhaustion of mental and other psychological resources needed for creative ideas to emerge and develop. Creativity involves complex, subtle and dynamic psychosocial mechanisms. Too often the managerial practices and organizational cultures are detrimental to the well known most powerful source of creativity: the intrinsic motivation. Briefly, this article deals with the paradox which lies in the fact that, in theory, so much emphasis is put on creativity and innovation as indispensable

  17. High altitude medicine education in China: exploring a new medical education reform.

    Science.gov (United States)

    Luo, Yongjun; Luo, Rong; Li, Weiming; Huang, Jianjun; Zhou, Qiquan; Gao, Yuqi

    2012-03-01

    China has the largest plateau in the world, which includes the whole of Tibet, part of Qinghai, Xinjiang, Yunnan, and Sichuan. The plateau area is about 257.2×10(4) km(2), which accounts for about 26.8% of the total area of China. According to data collected in 2006, approximately twelve million people were living at high altitudes, between 2200 to 5200 m high, on the Qinghai-Tibetan Plateau. Therefore, there is a need for medical workers who are trained to treat individuals living at high altitudes. To train undergraduates in high altitude medicine, the College of High Altitude Military Medicine was set up at the Third Military Medical University (TMMU) in Chongqing in 1999. This is the only school to teach high altitude medicine in China. Students at TMMU study natural and social sciences, basic medical sciences, clinical medical sciences, and high altitude medicine. In their 5(th) year, students work as interns at the General Hospital of Tibet Military Command in Lhasa for 3 months, where they receive on-site teaching. The method of on-site teaching is an innovative approach for training in high altitude medicine for undergraduates. Three improvements were implemented during the on-site teaching component of the training program: (1) standardization of the learning progress; (2) integration of formal knowledge with clinical experience; and (3) coaching students to develop habits of inquiry and to engage in ongoing self-improvement to set the stage for lifelong learning. Since the establishment of the innovative training methods in 2001, six classes of high altitude medicine undergraduates, who received on-site teaching, have graduated and achieved encouraging results. This evidence shows that on-site teaching needs to be used more widely in high altitude medicine education.

  18. Measuring social accountability in health professional education: development and international pilot testing of an evaluation framework.

    Science.gov (United States)

    Larkins, Sarah L; Preston, Robyn; Matte, Marie C; Lindemann, Iris C; Samson, Rex; Tandinco, Filedito D; Buso, David; Ross, Simone J; Pálsdóttir, Björg; Neusy, André-Jacques

    2013-01-01

    Health professional schools are responsible for producing graduates with competencies and attitudes to address health inequities and respond to priority health needs. Health professional schools striving towards social accountability founded the Training for Health Equity Network (THEnet). This article describes the development of THEnet evaluation framework for socially accountable health professional education, presents the framework to be used as a tool by other schools and discusses the findings of pilot implementation at five schools. The framework was designed collaboratively and built on Boelen and Woollard's conceptualization, production and usability model. It includes key components, linked to aspirational statements, indicators and suggested measurement tools. Five schools completed pilot implementation, involving workshops, document/data review and focus group discussions with faculty, students and community members. Three sections of the framework consider: How does our school work?; What do we do? and What difference do we make? Pilot testing proved that the evaluation framework was acceptable and feasible across contexts and produced findings useful at school level and to compare schools. The framework is designed as a formative exercise to help schools take a critical look at their performance and progress towards social accountability. Initiatives to implement the framework more widely are underway. The framework effectively aids in identifying strengths, weaknesses and gaps, with a view to schools striving for continuous self-improvement. THEnet evaluation framework is applicable and useful across contexts. It is possible and desirable to assess progress towards social accountability in health professional schools and this is an important step in producing health professionals with knowledge, attitudes, and skills to meet the challenges of priority health needs of underserved populations.

  19. SYNTHESIS OF ARTS AS A FACTOR OF TEENAGE CREATIVE EDUCATION

    Directory of Open Access Journals (Sweden)

    Liudmila Onofrichuk

    2017-03-01

    Full Text Available The article presents the method of teenage creative education by means of musical and theatrical arts at secondary comprehensive school. Showing school musical puppet theater «Fantasy» (secondary school No.12, Vinnytsia the author highlights the ways of pupils’ artistic and creative education during the study of the synthesis of the arts (music, singing, dance and recitation. The conditions affecting successful solution of the problem have been determined. Аmong them the author defines educational activities of a competent teacher who is capable to find out creative innovative solutions. The necessity and importance of using effective methods and techniques in terms of musical and theatrical activities for the development of pupils’ emotional sensitivity and overall creative development have been grounded. During music lessons, pupils successfully master creative abilities and skills (artistic speech, drama, puppet games, dancing, find innovate solutions to practical problems, interpret the original artistic images. Creative combinations of various forms and methods of work, rehearsals, spectacles, concert performances – promote the development of creativity, intensify artistic and performing activities of pupils. The awareness of the character’s motives is the impetus for creating the right stage feeling about reality and naturalness of stage action. It is noted that the art of musical theater helps them not only to acquire art knowledge and skills, but also strive for self-realization and self-improvement, better understanding of themselves and other people, awareness of the beauty of the life. The educational value of the theatrical activity lies in the understanding by teenagers their own attitude to the behavior of characters, developing the abilities to judge them critically, empathize and find alternatives for acquiring creative experience in future life situations.

  20. Human factors and safety issues associated with actinide retrieval from spent light water reactor fuel assemblies

    International Nuclear Information System (INIS)

    Spelt, P.F.

    1992-01-01

    A major problem in environmental restoration and waste management is the disposition of used fuel assemblies from the many light water reactors in the United States, which present a radiation hazard to those whose job is to dispose of them, with a similar threat to the general environment associated with long-term storage in fuel repositories around the country. Actinides resident in the fuel pins as a result of their use in reactor cores constitute a significant component of this hazard. Recently, the Department of Energy has initiated an Actinide Recycle Program to study the feasibility of using pyrochemical (molten salt) processes to recover actinides from the spent fuel assemblies of commercial reactors. This project concerns the application of robotics technology to the operation and maintenance functions of a plant whose objective is to recover actinides from spent fuel assemblies, and to dispose of the resulting hardware and chemical components from this process. Such a procedure involves a number of safety and human factors issues. The purpose of the project is to explore the use of robotics and artificial intelligence to facilitate accomplishment of the program goals while maintaining the safety of the humans doing the work and the integrity of the environment. This project will result in a graphic simulation on a Silicon Graphics workstation as a proof of principle demonstration of the feasibility of using robotics along with an intelligent operator interface. A major component of the operator-system interface is a hybrid artificial intelligence system developed at Oak Ridge National Laboratory, which combines artificial neural networks and an expert system into a hybrid, self-improving computer-based system interface. 10 refs

  1. Парадоксы навигационной карты (модели звёздного неба для дальних космических путешествий

    Directory of Open Access Journals (Sweden)

    Литвинчук Н. И.

    2015-02-01

    Full Text Available The man penetrates deeper into the mysteries of matter, space and time in the evolutionary development. He has only started the practical development of near space, but he already begins to travel into its depth in his dreams. One of the attributes of long-duration space travel is navigation charts (models of the Universe considering an exact arrangement of cosmic bodies and their gravitationalfields in space. For drawing up of such charts of the starry sky from the Earth it is necessary to consider a number of paradoxes which arise at the present system understanding of the Universe, such as its virtuality realities of the Big Bang, maximum flying speed, etc. Today we can fly away to the star worlds, which have not already been, and on the way to meet the worlds which have not been visible from the Earth yet. In the starry sky we see the story of stars removed from us for light years. Flight to these objects is the movement from their stories to reality.Training of their crews and staff will be not less important problem by the spaceship. Firstly, they will be trained on the Earth and then only in the flying using a simple self-improved system of training relevant staff.Today scientists and researchers did a huge groundwork in the field of space and the mass movement of enthusiasts has already started the implementation. It is pleasant, that the number of such enthusiasts already leads to practical qualitative changes, and it becomes the fact: different kind of space travel which is fast approaching reality.

  2. SELF-REGULATORY ABILITIES IN PROFESSIONAL ACTIVITY

    Directory of Open Access Journals (Sweden)

    G V Ozhiganova

    2016-12-01

    Full Text Available The self-regulation is considered by the author as a general ability of the person. The levels of self-regulation relating to any professional activity, and corresponding to these levels self-regulatory capacities are distinguished: 1 psychophysiological - the ability for self-regulation of emotional and psycho- physiological states; 2 socio-psychological - the ability for self-regulation in the process of social interaction; 3 psychological (the ability to regulate activities; the capacity for personal self-control;spiritual - the highest capacity for self-regulation due to the higher values and meanings of existence. Self-regulation at the highest spiritual level is considered in this research in connection with the actualization of higher self-regulatory capacities, leading to self-realization of the person including professional activity. Processes, levels, components of self-regulation, associated with different conditions of professional activities (for example, in extreme situations, as well as with different types of professions (teachers, sales managers, etc. are described. A particular attention is given to self- regulation in the teaching activities: levels, techniques of teachers’ self-regulatory skills are presented; the importance of teachers’ personal self-regulation is emphasized, because it determines self-development, self-improvement and self-fulfillment in their chosen profession, and is associated with the manifestation of higher self-regulatory capacities. It is noted that in the process of professional activities different levels and types of self-regulation are demanded. The self-regulation in professional activities is carried out due to various self-regulatory capabilities - from simple to complex, including the highest.

  3. The Social Context of “Do-It-Yourself” Brain Stimulation: Neurohackers, Biohackers, and Lifehackers

    Science.gov (United States)

    Wexler, Anna

    2017-01-01

    The “do-it-yourself” (DIY) brain stimulation movement began in earnest in late 2011, when lay individuals began building stimulation devices and applying low levels of electricity to their heads for self-improvement purposes. To date, scholarship on the home use of brain stimulation has focused on characterizing the practices of users via quantitative and qualitative studies, and on analyzing related ethical and regulatory issues. In this perspective piece, however, I take the opposite approach: rather than viewing the home use of brain stimulation on its own, I argue that it must be understood within the context of other DIY and citizen science movements. Seen in this light, the home use of brain stimulation is only a small part of the “neurohacking” movement, which is comprised of individuals attempting to optimize their brains to achieve enhanced performance. Neurohacking itself is an offshoot of the “life hacking” (or “quantified self”) movement, in which individuals self-track minute aspects of their daily lives in order to enhance productivity or performance. Additionally, the home or DIY use of brain stimulation is in many ways parallel to the DIY Biology (or “biohacking”) movement, which seeks to democratize tools of scientific experimentation. Here, I describe the place of the home use of brain stimulation with regard to neurohackers, lifehackers, and biohackers, and suggest that a policy approach for the home use of brain stimulation should have an appreciation both of individual motivations as well as the broader social context of the movement itself. PMID:28539877

  4. Working Memory, Reasoning, and Task Switching Training: Transfer Effects, Limitations, and Great Expectations?

    Science.gov (United States)

    Baniqued, Pauline L; Allen, Courtney M; Kranz, Michael B; Johnson, Kathryn; Sipolins, Aldis; Dickens, Charles; Ward, Nathan; Geyer, Alexandra; Kramer, Arthur F

    2015-01-01

    Although some studies have shown that cognitive training can produce improvements to untrained cognitive domains (far transfer), many others fail to show these effects, especially when it comes to improving fluid intelligence. The current study was designed to overcome several limitations of previous training studies by incorporating training expectancy assessments, an active control group, and "Mind Frontiers," a video game-based mobile program comprised of six adaptive, cognitively demanding training tasks that have been found to lead to increased scores in fluid intelligence (Gf) tests. We hypothesize that such integrated training may lead to broad improvements in cognitive abilities by targeting aspects of working memory, executive function, reasoning, and problem solving. Ninety participants completed 20 hour-and-a-half long training sessions over four to five weeks, 45 of whom played Mind Frontiers and 45 of whom completed visual search and change detection tasks (active control). After training, the Mind Frontiers group improved in working memory n-back tests, a composite measure of perceptual speed, and a composite measure of reaction time in reasoning tests. No training-related improvements were found in reasoning accuracy or other working memory tests, nor in composite measures of episodic memory, selective attention, divided attention, and multi-tasking. Perceived self-improvement in the tested abilities did not differ between groups. A general expectancy difference in problem-solving was observed between groups, but this perceived benefit did not correlate with training-related improvement. In summary, although these findings provide modest evidence regarding the efficacy of an integrated cognitive training program, more research is needed to determine the utility of Mind Frontiers as a cognitive training tool.

  5. Atheist spirituality: a follow on from New Atheism?

    Directory of Open Access Journals (Sweden)

    Teemu Taira

    2012-01-01

    Full Text Available Books about well-being, self-improvement, life management and spirituality have been popular for many years. It is not news to anybody that such topics sell. However, books on atheism have never become bestsellers until the early years of the twenty-first century. Now the so-called New Atheist books have altogether sold millions. It may sound surprising, but atheism sells. It may have been the idea of a publishers’ marketing department to put the two selling points together, but in recent years a number of books about atheist spirituality, spiritual atheism and atheist self-help have been published. That has been one aspect of the increased visibility of atheism and spirituality in public discourse. Atheist discourse which is combined with ‘spirituality’ might be called ‘post-secular’ as it does not fit easily into the neat binary classification between religious and non-religious secular. This article examines this hybrid area in atheist discourse in relation to three aspects: monotheism, spirituality and meditation. Atheist discourse situates itself against monotheism, but some spokespersons combine atheism with spirituality and meditation. This works as an example of a wider and recent trend in society where a blurring of the earlier normative boundaries between religion and non-religion has become fairly common, not necessarily in terms of beliefs, but of practices. Even though there is a long tradition of non-theistic and atheistic readings of Buddhism, for example, they have rarely been combined with an explicit criticism of monotheistic traditions and atheist consciousness-raising.

  6. THE ROLE OF TERTIARY EDUCATION IN THE ARCHITECTURE OF ECONOMIC DEVELOPMENT

    Directory of Open Access Journals (Sweden)

    Daniela Mihaela NEAMȚU

    2016-06-01

    Full Text Available In the current transformation of nations where all economic, social, political and civic pillars, experienced a new dynamic in trying to adapt to present conditions, contribution of higher education is becoming more evident in Romanian society. Universities have often been perceived as the key institutions in the processes of social change and development. The most explicit role universities have been assigned consisted of highly skilled labor productivity and conducting research processes to meet perceived economic needs of society. Higher education not only contributes to the formation of skilled workers, but also creates a workforce eager to acquire new knowledge that contributes to growth and social and economic development. Higher education creates new attitudes, makes vision changes necessary for the socialization of individuals, for modernization and transformation of societies. The need to draw attention to the importance of the human element brings to the fore the need for human resource development from the perspective of learning and lifelong self-improvement. Establishing the relationship between higher education and economic growth was based on an analysis of the link between them for a longer period of time, over 40 years, in the period 1971-2013 for the countries UK, Poland, Sweden, Korea South and Romania, using participation rates in tertiary education (School Enrollment Ratio, tertiary -% gross as an indicator for assessing the changes in higher education and the Gross Domestic Product per capita (expressed in dollars per capita as an indicator of economic growth. Choosing the five countries included in the research is justified by the investments made in education correlated with economic growth related to these countries.

  7. Improving maintenance medication adherence in adult inflammatory bowel disease patients: a pilot study

    Directory of Open Access Journals (Sweden)

    Michelle L. Matteson-Kome

    2014-01-01

    Full Text Available Medication nonadherence in inflammatory bowel disease (IBD may lead to suboptimal control of the disease, decreased quality of life, and poor outcomes. This pilot study evaluated the feasibility, intervention mechanism, and potential effectiveness of a three-month continuous self-improvement (CSI intervention to enhance medication adherence (MA in adult nonadherent IBD patients. Adult IBD patients taking a daily or twice-daily dosed maintenance medication were screened electronically for two months to determine baseline MA levels. Nonadherent IBD participants were randomized to the CSI or the attention control (AC intervention and monitored for three months. The CSI intervention consisted of a data evaluation and system refinement process in which system changes were identified and implemented. The AC group was given educational information regarding IBD disease process, extra-intestinal manifestations of IBD, and medical therapy. Demographic statistics, change scores for within and between- group differences, and effect size estimates were calculated. Nine nonadherent participants (medication adherence score <0.85 were eligible for randomization. The intervention was found feasible and acceptable. Although no statistically significant improvement in MA was found (P=0.14, adherence improved in 3 of 4 of the CSI group and 1 of 2 in the attention control group. The effect size calculation of 1.9 will determine the sample size for future study. The results of this pilot study showed the intervention was feasible and had a positive effect on MA change score and adherence levels. A larger fully powered study is needed to test of the effectiveness of this innovative intervention.

  8. The other side of the brain: The politics of split-brain research in the 1970s-1980s.

    Science.gov (United States)

    Staub, Michael E

    2016-11-01

    In the course of the 1970s and 1980s, theories derived from neuropsychological research on the bisected brain came rapidly to achieve the status of common sense in the United States and Canada, inflecting all manner of popular and academic discussion. These theories often posited that the right hemisphere was the seat of creative expression, whereas the left hemisphere housed rationality and language. This article analyzes the political and cultural implications of theories about the split brain. Gender relations, educational reform, management theory, race relations, and countercultural concepts about self-expression all quickly came to be viewed through the lens of left-brain/right-brain neuropsychological research. Yet these theories were often contradictory. On the one hand, some psychophysiological experiments premised that the brain was inherently plastic in nature, and thus self-improvement techniques (like mindfulness meditation) could be practiced to unfurl the right hemisphere's intuitive potentialities. On the other hand, other psychophysiological experiments concluded that Native Americans as well as African Americans and persons from "the East" appeared inherently to possess more highly developed right-brain talents, and therefore suffered in the context of a left-hemisphere-dominated Western society. In both instances, psychologists put neuroscientific research to political and social use. This article thus connects a story from the annals of the neurosciences to the history of psychological experimentation. It analyzes the critical impact that speculative ideas about the split brain were to have not only on the post-1960s history of psychology but also on what soon emerged after the 1990s as the social neuroscience revolution. (PsycINFO Database Record (c) 2016 APA, all rights reserved).

  9. PHILOSOPHICAL AND ANTHROPOLOGICAL IMPORTANCE OF DEVELOPMENT OF ARTIFICIALLY CREATED INTELLIGENT SYSTEMS

    Directory of Open Access Journals (Sweden)

    Yu. D. Gensitskiy

    2015-12-01

    Full Text Available Purpose. Understanding the philosophical and anthropological importance of the development the artificial intelligence systems requires the analysis of the socio and anthropological content of intercomputer problems of interaction in the context of media philosophical praxis, anthropological maintenance of intellect nature, considering the specifics of the concept of artificial intelligence systems in the environment of M2M development of socio-cognitive practices of intercomputer interaction of social and humanitarian potential. Methodology. The implementation target is seen in the use of scientific and theoretical basis of the media philosophical, philosophical anthropology, the media philosophical approach to understanding society, science and technology, the use of publications on selected topics of research. Scientific novelty. The concept of artificial intelligence systems in the aspect of social and humanitarian potential of their formation and development in the environment of M2M was considered. The problems of machine learning as technology transformation M2M were analysed. The anthropological threats to the development of artificially created intelligent systems were defined. Conclusions. From the global risks point of view, one of the most critical circumstances due to the artificial intelligent system can strengthen its intelligence very quickly. The obvious reason for suspecting such an opportunity – a recursive self-improvement. Such system becomes smarter, including the intelligent writing of internal cognitive function, that the ability to rewrite their existing cognitive function to make it work better. This will make such systems more intelligent, and smarter in terms of the processing itself. The success of artificial intelligence may be the beginning of the end of the human race. Almost any technology falling into malicious hands reveals the potential for harm, but when it comes to artificial intelligent system, there is a

  10. The substantive core of introduction of the saving production into educational process of the higher technical educational institution

    Directory of Open Access Journals (Sweden)

    Світлана Станіславівна Єрмакова

    2016-03-01

    of “retractive” production of knowledge and its permanent self-improvement

  11. Awareness of one’s own weaknesses is the first step on the path to leadership excellence

    Directory of Open Access Journals (Sweden)

    Vesna Janković

    2012-06-01

    Full Text Available RQ: Comparison of personal characteristics of management with the EFQM model of management.Purpose: The purpose of this research study is to determine the similarities and differences of management characteristics on the basis of comparing personal analysis characteristics and the model of management excellence. The aim is to improve the personal style of management and to come closer to excellence.Method: A qualitative method of comparative analysis was used.Results: The results indicated slight deviations that were not perceived by the author as weaknesses but opportunities for improvements. At the same time the results indicated the qualities of a good leader that were already known and upon which these were built upon and developed.Organization: The results are intended to be used by the author’s employer and by the author herself taking on new assignments and responsibilities; transferring from managing a small group of employees to managing a whole business unit and units within the company that are not completely familiar to the author. The research is also an instrument that aids superiors with directing and monitoring the author’s work. To other readers it brings a different view on the aspect of management and the notion of analysis and self-criticism as well as the constant search for improvement.Society: Comparison can be used in the wider environment. Originality: A different view on the problems of management byanalyzing oneself; a constant search for self-improvement on a personal level as well as in one’s own career.Limitations: The research is a basis for comparison, finding improvement, advancement, and digression in the next analysis of personality.

  12. [A review for a traditional Chinese medical journal Shou Shi Yi Bao].

    Science.gov (United States)

    Xiao, Mei-Hua; Sun, Wen-Zhong

    2009-06-01

    Shou Shi Yi Bao was a journal of traditional Chinese medicine (TCM) during the period from 1935 to 1937, and was originated by Chen Huan-yun, a TCM physician in Suzhou. It is mainly to transmit the knowledge of TCM and to promote the epidemic prevention capacity of local public. The editorial characteristics and historical value of the journal were explored in initial background, staff writers, editorial policies, contents and the Editor Chen's medical ideas. Shou Shi Yi Bao was supported by many famous TCM physicians, although the journal was originated from the civil society. It was an academic TCM journal with perfect practicability for orientating to the public and highlighting the academic spirit. Chen Huan-yun was a resolute defender of TCM, and had many opinions on clinical practice and lots of scientific suggestions on TCM development. Shou Shi Yi Bao reflected the main characteristics of TCM journals in 1930s. The journal was one of the important documents to study the TCM history during the period of the Republic of China in Jiangsu Province, and it also set a stage for the struggle between TCM and Western medicine at that time. The documentary information of the journal has literature and history values in reflecting the historical process of TCM self-improvement. The success of the journal was due to not only the broken-up sectarian bias and cooperation of the TCM practitioners but also the preponderant geographic and cultural circumstances of Suzhou as well as Chen Huan-yun's profound knowledge in traditional Chinese culture and medicine.

  13. Working Memory, Reasoning, and Task Switching Training: Transfer Effects, Limitations, and Great Expectations?

    Science.gov (United States)

    Baniqued, Pauline L.; Ward, Nathan; Geyer, Alexandra; Kramer, Arthur F.

    2015-01-01

    Although some studies have shown that cognitive training can produce improvements to untrained cognitive domains (far transfer), many others fail to show these effects, especially when it comes to improving fluid intelligence. The current study was designed to overcome several limitations of previous training studies by incorporating training expectancy assessments, an active control group, and “Mind Frontiers,” a video game-based mobile program comprised of six adaptive, cognitively demanding training tasks that have been found to lead to increased scores in fluid intelligence (Gf) tests. We hypothesize that such integrated training may lead to broad improvements in cognitive abilities by targeting aspects of working memory, executive function, reasoning, and problem solving. Ninety participants completed 20 hour-and-a-half long training sessions over four to five weeks, 45 of whom played Mind Frontiers and 45 of whom completed visual search and change detection tasks (active control). After training, the Mind Frontiers group improved in working memory n-back tests, a composite measure of perceptual speed, and a composite measure of reaction time in reasoning tests. No training-related improvements were found in reasoning accuracy or other working memory tests, nor in composite measures of episodic memory, selective attention, divided attention, and multi-tasking. Perceived self-improvement in the tested abilities did not differ between groups. A general expectancy difference in problem-solving was observed between groups, but this perceived benefit did not correlate with training-related improvement. In summary, although these findings provide modest evidence regarding the efficacy of an integrated cognitive training program, more research is needed to determine the utility of Mind Frontiers as a cognitive training tool. PMID:26555341

  14. A Study of Nora and Eliza from the Perspective of Feminism%女性主义视角下娜拉与伊莉莎的对比思考

    Institute of Scientific and Technical Information of China (English)

    卢青青

    2012-01-01

    易卜生笔下《玩偶之家》中的娜拉和萧伯纳《格梅利翁》中的伊莉莎生活背景迥然不同,但她们有着极其相似的经历,她们都有很强的觉醒意识,是追求独立自主、平等和谐的女性代言人。从女性主义视角走进娜拉和伊莉莎的世界,探索在男权社会中女性的人生和命运,以寻求现代女性在家庭、社会中如何关注个体发展,形成自己的独立人格,实现真正的自强自立。%The protagonists Nora in A Doll ' s House and Eliza in Pygmalion have quite different living background, but they possess similar experience. Both of them, having strong self-awareness, are the spokespersons who pursue self-independent, free and harmony life. From the perspective of feminism, it goes into the world of Nora and Eliza to explore the life and of the females in the situation of patriarchy. From the, modern women may have a better and right judgment of how to pay attention to their individual development and their family and social roles, by which they can develop their own independent personalities and truly achieve self-improvement and self-support and self-reliance in modern society.

  15. From Self-Assessment to Frustration, A Small Step Towards Autonomy in Robotic Navigation.

    Directory of Open Access Journals (Sweden)

    Adrien eJauffret

    2013-10-01

    Full Text Available Autonomy and self-improvement capabilities are still challenging in the fields of robotics and machine learning. Allowing a robot to autonomously navigate in wide and unknown environments not only requires a repertoire of robust strategies to cope with miscellaneous situations, but also needs mechanisms of self-assessment for guiding learning and for monitoring strategies. Monitoring strategies requires feedbacks on the behavior’s quality, from a given fitness system in order to take correct decisions.In this work, we focus on how a second-order controller can be used to (1 manage behaviors according to the situation and (2 seek for human interactions to improve skills. Following an incremental and constructivist approach, we present a generic neural architecture, based on an online novelty detection algorithm that may be able to self-evaluate any sensory-motor strategies. This architecture learns contingencies between sensations and actions, giving the expected sensation from the previous perception. Prediction error, coming from surprising events, provides a measure of the quality of the underlying sensory-motor contingencies. We show how a simple second-order controller (emotional system based on the prediction progress allows the system to regulate its behavior to solve complex navigation tasks and also succeeds in asking for help if it detects dead-lock situations.We propose that this model could be a key structure toward self-assessment and autonomy. We made several experiments that can account for such properties for two different strategies (road following and place cells based navigation in different situations.

  16. HIGHER EDUCATION IN THE CONTEXT OF FORMATION OF PROFESSIONAL THINKING OF STUDENTS

    Directory of Open Access Journals (Sweden)

    Irina V. Taraskina

    2016-03-01

    defend them, regardless of his chosen profession, to confidently engage in social relations, achieving results with minimal time and cost, capable to self-improvement, self-change, active adaptation in the labour market.

  17. Assistance provided by physical therapists from primary health care to patients after stroke

    Directory of Open Access Journals (Sweden)

    Christina Danielli Coelho de Morais Faria

    Full Text Available Abstract Introduction: Motor impairments, which are prevalent in stroke subjects, require physical therapy (PT rehabilitation. In primary care in the Brazilian Public Health System, PT are part of the Núcleo de Apoio à Saúde da Família (NASF. Objective: To describe the PT assistance provided to stroke patients in a primary healthcare center. Methods: The records of all stroke patients (n = 44; 69.23 ± 13.12 years identified by the health professionals were analyzed. Using keyword recognition, frequency analysis of the services offered by the PT was performed. Subjects were classified according to the Modified Rankin Scale. Results: In the 44 records, 45.5% had a description of the assistance provided by any professional of the NASF and 36.4% of the PT care. PT care was provided at the subject’s home (94.2% and at the healthcare center (5.8%. The PT practices were identified as: orientation (93.8%, evaluation (87.5%, exercises (50%, follow-up (37.5%, referral to another service or to undergrad PT students (18.8%, and referral to other NASF professionals (12.5%. Most of the subjects were classified as having mild/moderate disability. Conclusion: The minority of records had registration of attendance by the NASF PT. The majority of the sessions occurred at the subject’s home, which reveals a practice focused on individual care. The orientation was common, which illustrates that in primary care there is a focus on empowerment for health self-improvement. Follow-up was not common, despite clinical guidelines state that stroke subjects should be monitored at least once a year by the rehabilitation team.

  18. Enhancing Clients' Communication Regarding Goals for Using Psychiatric Medications.

    Science.gov (United States)

    Deegan, Patricia E; Carpenter-Song, Elizabeth; Drake, Robert E; Naslund, John A; Luciano, Alison; Hutchison, Shari L

    2017-08-01

    Discordance between psychiatric care providers' and clients' goals for medication treatment is prevalent and is a barrier to person-centered care. Power statements-short self-advocacy statements prepared by clients in response to a two-part template-offer a novel approach to help clients clarify and communicate their personal goals for using psychiatric medications. This study described the power statement method and examined a sample of power statements to understand clients' goals for medication treatment. More than 17,000 adults with serious mental illness at 69 public mental health clinics had the option to develop power statements by using a Web application located in the clinic waiting areas. A database query determined the percentage of clients who entered power statements into the Web application. The authors examined textual data from a random sample of 300 power statements by using content analysis. Nearly 14,000 (79%) clients developed power statements. Of the 277 statements in the sample deemed appropriate for content analysis, 272 statements had responses to the first part of the template and 230 had responses to the second part. Clients wanted psychiatric medications to help control symptoms in the service of improving functioning. Common goals for taking psychiatric medications (N=230 statements) were to enhance relationships (51%), well-being (32%), self-sufficiency (23%), employment (19%), hobbies (15%), and self-improvement (10%). People with serious mental illness typically viewed medications as a means to pursue meaningful life goals. Power statements appear to be a simple and scalable technique to enhance clients' communication of their goals for psychiatric medication treatment.

  19. Reducing the Digital Divide among Children Who Received Desktop or Hybrid Computers for the Home

    Directory of Open Access Journals (Sweden)

    Gila Cohen Zilka

    2016-06-01

    Full Text Available Researchers and policy makers have been exploring ways to reduce the digital divide. Parameters commonly used to examine the digital divide worldwide, as well as in this study, are: (a the digital divide in the accessibility and mobility of the ICT infrastructure and of the content infrastructure (e.g., sites used in school; and (b the digital divide in literacy skills. In the present study we examined the degree of effectiveness of receiving a desktop or hybrid computer for the home in reducing the digital divide among children of low socio-economic status aged 8-12 from various localities across Israel. The sample consisted of 1,248 respondents assessed in two measurements. As part of the mixed-method study, 128 children were also interviewed. Findings indicate that after the children received desktop or hybrid computers, changes occurred in their frequency of access, mobility, and computer literacy. Differences were found between the groups: hybrid computers reduce disparities and promote work with the computer and surfing the Internet more than do desktop computers. Narrowing the digital divide for this age group has many implications for the acquisition of skills and study habits, and consequently, for the realization of individual potential. The children spoke about self improvement as a result of exposure to the digital environment, about a sense of empowerment and of improvement in their advantage in the social fabric. Many children expressed a desire to continue their education and expand their knowledge of computer applications, the use of software, of games, and more. Therefore, if there is no computer in the home and it is necessary to decide between a desktop and a hybrid computer, a hybrid computer is preferable.

  20. Fathers’ involvement in child’s upbringing vs. young people’s idea of the father’s role in the family [Oddziaływania wychowawcze ojców a poglądy młodzieży na temat roli ojca w rodzinie

    Directory of Open Access Journals (Sweden)

    Anna MARZEC-TARASIŃSKA

    2017-11-01

    Full Text Available As a result of ongoing social, economic, and cultural changes the role of the father in his child’s upbringing is becoming more and more significant. Therefore a growing interest in the characteristics of the father’s role in the family and his educational competences can be observed. J. Witczak5 believes that father’s involvement in the upbringing process is not only crucial but practically irreplaceable. The father provides his child with the stimuli and models necessary for social and moral development and it is either impossible for the mother to provide these stimuli and models or she is able to do it only in a limited scope. Having analyzed the fathers’ involvement in the child’s development process, the difference between maternal and paternal love should be emphasised. K. Pospiszyl6 states that although mothers and fathers love their children in a different way, both types of love are essential. According to T. Sosnowski7 mood lability is typical of mothers. Fathers tend to be better-balanced in their feelings and more consistent in their behaviour, which is of high significance when it comes to the effectiveness of influencing the child’s behaviour. Consistency is absolutely fundamental for providing the child with emotional stability. Paternal love constitutes a strong encouragement to further development and provides directions concerning the child’s behaviour. E. Fromm8 claims that it is the mother's role to provide children with the feeling of life security, and the father’s role is to teach children and to direct them in a way that enables them to adjust to the community they live in. It is the father who poses requirements and shapes the child’s personality traits so that it is able to overcome life’s difficulties. Paternal love provides inspiration and encouragement for the child to further work on self-improvement.