Hereditary pancreatitis for the endoscopist

OpenAIRE

Patel, Milan R.; Eppolito, Amanda L.; Willingham, Field F.

2013-01-01

Hereditary pancreatitis shares a majority of clinical and morphologic features with chronic alcoholic pancreatitis, but may present at an earlier age. The term hereditary pancreatitis has primarily been associated with mutations in the serine protease 1 gene (PRSS1) which encodes for cationic trypsinogen. PRSS1 mutations account for approximately 68–81% of hereditary pancreatitis. Mutations in other genes, primarily serine protease inhibitor Kazal type 1 (SPINK1) and the cystic fibrosis trans...

Hereditary angioedema.

Science.gov (United States)

Bracho, Francisco A

2005-11-01

Hereditary angioedema is an autosomal-dominant deficiency of C1 inhibitor--a serpin inhibitor of kallikrein, C1r, C1s, factor XII, and plasmin. Quantitative or qualitative deficiency of C1 inhibitor leads to the generation of vasoactive mediators, most likely bradykinin. The clinical syndrome is repeated bouts of nonpruritic, nonpitting edema of the face, larynx, extermities, and intestinal viscera. Recently, investigators, physicians, and industry have demonstrated a renewed interest in the biology and treatment of hereditary angioedema. Investigators have generated a C1INH-/- mouse model that has demonstrated the importance of the contact activation system for hereditary angioedema-related vascular permeability. An interactive database of mutations is available electronically. Investigators have continued exploration into mRNA/protein levels. The proceedings of a recent workshop have been impressive in the scope and depth. Clinicians have produced consensus documents and expert reviews. The pharmaceutical industry has initiated clinical trails with novel agents. Hereditary angioedema is often misdiagnosed and poorly treated. Diagnosis requires careful medical and family history and the measurement of functional C1 inhibitor and C4 levels. Attenuated androgens, anti-fibrinolytics, and C1 inhibitor concentrates are used for long-term and preprocedure prophylaxis, but have significant drawbacks. C1 inhibitor concentrates and fresh frozen plasma are available for acute intervention. The mainstays of supportive care are airway monitoring, pain relief, hydration, and control of nausea. New agents such as recombinant C1 inhibitor, kallikrein inhibitors, and bradykinin inhibitors may offer safer and more tolerable treatments.

Dehydration in the Older Adult.

Science.gov (United States)

Miller, Hayley J

2015-09-01

Dehydration affects 20% to 30% of older adults. It has a greater negative outcome in this population than in younger adults and increases mortality, morbidity, and disability. Dehydration is often caused by water deprivation in older adults, although excess water loss may also be a cause. Traditional markers for dehydration do not take into consideration many of the physiological differences present in older adults. Clinical assessment of dehydration in older adults poses different findings, yet is not always diagnostic. Treatment of dehydration should focus on prevention and early diagnosis before it negatively effects health and gives rise to comorbidities. The current article discusses what has most thoroughly been studied; the best strategies and assessment tools for evaluation, diagnosis, and treatment of dehydration in older adults; and what needs to be researched further. [Journal of Gerontological Nursing, 41(9), 8-13.]. Copyright 2015, SLACK Incorporated.

Hereditary forms of breast cancer

International Nuclear Information System (INIS)

Bella, V.

2009-01-01

Breast cancer is the most common oncologic disease in the female population. Besides the sporadic occurrence it occurs in the familial and hereditary form. Persons with the occurrence of positive family anamnesis of breast cancer should be actively investigated. In the indicated cases it is necessary to send the woman to genetic examination. In case that the hereditary form of breast cancer is affirmed it is necessary to examine her family relatives. Women with the hereditary form of breast cancer occur in about 5 – 10 % portion from all women diagnosed with breast cancer. Nowadays we already know that 80 % of hereditary breast cancers are due to germ mutations in BRCA 1 and BRCA 2 gene. Persons with detected gene mutations must be dispensarized in the centres intended for it. (author)

Dehydration behaviour of hydrates

Energy Technology Data Exchange (ETDEWEB)

Dette, S.S.; Stelzer, T.; Jones, M.J.; Ulrich, J. [Martin-Luther-Universitaet Halle-Wittenberg, Zentrum fuer Ingenieurwissenschaften, Verfahrenstechnik/TVT, 06099 Halle (Germany)

2010-07-15

Immersing a crystalline solvate in a suitable anti-solvent can induce phase transformation to solvent-free solid phase. In certain cases the solvent-mediated phase transition results in the generation of hollow, tubular structures. Both the tube dimensions of sodium-2-keto-L-gulonate anhydrate (skga) and the dehydration kinetics of sodium-2-keto-L-gulonate monohydrate (skgm) can be modified by the antisolvent employed. An explanation for the variable dehydration behaviour of skgm in the antisolvents is presented here. Furthermore, other crystalline hydrates were dehydrated in dry methanol. Providing an operational window can be found, any hydrate material could possibly find use in the production of tubes (micro- or nanotubes for different applications). The experimental conditions selected (dry methanol as antisolvent, dehydration temperature at 25 C) for the dehydration did not lead to the anhydrate tube growth for all hydrates investigated. Based upon the results presented here a first hypothesis is presented to explain this effect. (copyright 2010 WILEY-VCH Verlag GmbH and Co. KGaA, Weinheim) (orig.)

BRCA1/2 associated hereditary breast cancer

Institute of Scientific and Technical Information of China (English)

Li-song TENG; Yi ZHENG; Hao-hao WANG

2008-01-01

Breast cancer is one of the leading causes of death in women today. Some of the patients are hereditary, with a large proportion characterized by mutation in BRCA1 and/or BRCA2 genes. In this review, we provide an overview of these two genes,focusing on their relationship with hereditary breast cancers. BRCA1/2 associated hereditary breast cancers have unique features that differ from the general breast cancers, including alterations in cellular molecules, pathological bases, biological behavior, and a different prevention strategy. But the outcome of BRCA1/2 associated hereditary breast cancers still remains controversial;further studies are needed to elucidate the nature of BRCA1/2 associated hereditary breast cancers.

Startle responses in hereditary hyperekplexia

NARCIS (Netherlands)

Tijssen, M. A.; Voorkamp, L. M.; Padberg, G. W.; van Dijk, J. G.

1997-01-01

BACKGROUND: Patients with hereditary hyperekplexia have excessive startle responses that are accompanied by transient stiffness and also continuous stiffness in infancy. A point of mutation has been identified for the major form of hereditary hyperekplexia in the gene encoding the alpha 1 subunit of

Startle responses in hereditary hyperekplexia

NARCIS (Netherlands)

Tijssen, MAJ; Voorkamp, LM; Padberg, GW; vanDijk, JG

Background: Patients with hereditary hyperekplexia have excessive startle responses that are accompanied by transient stiffness and also continuous stiffness in infancy. A point of mutation has been identified for the major form of hereditary hyperekplexia in the gene encoding the alpha 1 subunit of

Salmonella survival during thermal dehydration of fresh garlic and storage of dehydrated garlic products.

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Zhang, Hongmei; Qi, Yan; Wang, Lei; Zhang, Shaokang; Deng, Xiangyu

2017-12-18

Salmonella survival was characterized and modeled during thermal dehydration of fresh garlic and storage of dehydrated garlic products. In our experiments that simulated commercial dehydration processing at 80±5°C, moderate level of Salmonella contamination (4-5logCFU/g) on fresh garlic was reduced below the enumeration limit (1.7logCFU/g) after 4.5h of dehydration and not detectable by culture enrichment after 7h. With high level of contamination (7-8logCFU/g), the Salmonella population persisted at 3.6logCFU/g after 8h of processing. By increasing the dehydration temperature to 90±5°C, the moderate and high levels of initial Salmonella load on fresh garlic dropped below the enumeration limit after 1.5 and 3.75h of processing and became undetectable by culture enrichment after 2.5 and 6h, respectively. During the storage of dried garlic products, Salmonella was not able to grow under all tested combinations of temperature (25 and 35°C) and water activity (0.56-0.98) levels, suggesting active inhibition. Storage temperature played a primary role in determining Salmonella survival on dehydrated garlic flakes. Under a typical storage condition at 25°C and ambient relative humidity, Salmonella could persist over months with the population gradually declining (4.3 log reduction over 88days). Granular size of dehydrated garlic had an impact on Salmonella survival, with better survival of the pathogen observed in bigger granules. At the early stage of dehydrated garlic storage (until 7days), rising water activity appeared to initially promote but then inhibited Salmonella survival, resulting in a water activity threshold at 0.73 where Salmonella displayed strongest persistence. However, this phenomenon was less apparent during extended storage (after 14days). Copyright © 2017 Elsevier B.V. All rights reserved.

Water-loss dehydration and aging.

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Hooper, Lee; Bunn, Diane; Jimoh, Florence O; Fairweather-Tait, Susan J

2014-01-01

This review defines water-loss and salt-loss dehydration. For older people serum osmolality appears the most appropriate gold standard for diagnosis of water-loss dehydration, but clear signs of early dehydration have not been developed. In older adults, lower muscle mass, reduced kidney function, physical and cognitive disabilities, blunted thirst, and polypharmacy all increase dehydration risk. Cross-sectional studies suggest a water-loss dehydration prevalence of 20-30% in this population. Water-loss dehydration is associated with higher mortality, morbidity and disability in older people, but evidence is still needed that this relationship is causal. There are a variety of ways we may be able to help older people reduce their risk of dehydration by recognising that they are not drinking enough, and being helped to drink more. Strategies to increase fluid intake in residential care homes include identifying and overcoming individual and institutional barriers to drinking, such as being worried about not reaching the toilet in time, physical inability to make or to reach drinks, and reduced social drinking and drinking pleasure. Research needs are discussed, some of which will be addressed by the FP7-funded NU-AGE (New dietary strategies addressing the specific needs of elderly population for a healthy ageing in Europe) trial. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

Device to control the desalination and dehydration process for petroleum in an electro-dehydrator

Energy Technology Data Exchange (ETDEWEB)

Kusowskii, B I; Pawlow, N I; Matiitschenko, A P; Sacharow, S A; Kirilin, A F

1976-01-22

The invention deals with a device to control the desalination and dehydration processes for petroleum in electro-dehydrators. In the latter, the water-petroleum emulsion introduced is separated by an electric field into petroleum and salt water. Regulation of the dehydration and desalination process by means of the current difference of two circuits in the emulgator, in which this difference controls the controlling unit of a regulating valve, via which salt water and dirt remains are removed from the dehydrator, is known. This device, however, is not immune to disturbance, as when changing the water content of the emulsion, spark-overs may occur between one electrode and the surface of the separated salt water. This disturbance is removed according to the invention in that the abrupt changes of current prior to the spark-over cause a forcibly increased through-flow amount of discharging salt water and dirt remains for a certain time and thus prevent the full formation of an electric spark-over disturbing the operation of the dehydrator.

Dehydration

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... dehydration also may differ by age. Infant or young child Dry mouth and tongue No tears when ... Clinic does not endorse any of the third party products and services advertised. Advertising and sponsorship policy ...

Evaluation and Management of Dehydration in Children.

Science.gov (United States)

Santillanes, Genevieve; Rose, Emily

2018-05-01

The article discusses the evaluation of dehydration in children and reviews the literature on physical findings of dehydration. Pediatric dehydration is a common problem in emergency departments and wide practice variation in treatment exists. Dehydration can be treated with oral, nasogastric, subcutaneous, or intravenous fluids. Although oral rehydration is underutilized in the United States, most children with dehydration can be successfully rehydrated via the oral route. Selection of oral rehydration solution and techniques for successful oral rehydration are presented. Appropriate selection and rate of administration of intravenous fluids are also discussed for isonatremic, hyponatremic, and hypernatremic dehydration. Copyright © 2017 Elsevier Inc. All rights reserved.

Cellular characteristics of hereditary diseases of man

International Nuclear Information System (INIS)

Sasaki, Masao

1978-01-01

Hereditary diseases of which abnormal characters could be detected at cultured cell level were introduced, and tissue cultures of them were described. Characteristics such as reproduction, disorder, elimination, and repair of DNA in hereditary diseases with high cancer risk such as Bloom syndrome, etc. were investigated. Radiosensitivity of these hereditary diseases was also described, and factors of carcinogenesis were investigated. (Serizawa, K.)

Hereditary angioedema

Science.gov (United States)

... disease; HAE- Hereditary angioedema; Kallikrein inhibitor-HAE: bradykinin receptor antagonist-HAE; C1-inhibitors-HAE; Hives-HAE ... aunt, uncle, or grandparent. Dental procedures, sickness (including colds and the flu), and surgery may trigger HAE ...

The molecular classification of hereditary endocrine diseases.

Science.gov (United States)

Ye, Lei; Ning, Guang

2015-12-01

Hereditary endocrine diseases are an important group of diseases with great heterogeneity. The current classification for hereditary endocrine disease is mostly based upon anatomy, which is helpful for pathophysiological interpretation, but does not address the pathogenic variability associated with different underlying genetic causes. Identification of an endocrinopathy-associated genetic alteration provides evidence for differential diagnosis, discovery of non-classical disease, and the potential for earlier diagnosis and targeted therapy. Molecular diagnosis should be routinely applied when managing patients with suspicion of hereditary disease. To enhance the accurate diagnosis and treatment of patients with hereditary endocrine diseases, we propose categorization of endocrine diseases into three groups based upon the function of the mutant gene: cell differentiation, hormone synthesis and action, and tumorigenesis. Each category was further grouped according to the specific gene function. We believe that this format would facilitate practice of precision medicine in the field of hereditary endocrine diseases.

[Dehydration due to "mouth broken"].

Science.gov (United States)

Meijler, D P M; van Mossevelde, P W J; van Beek, R H T

2012-09-01

Two children were admitted to a medical centre due to dehydration after an oral injury and the extraction of a tooth. One child complained of "mouth broken". Dehydration is the most common water-electrolyte imbalance in children. Babies and young children are prone to dehydration due to their relatively large body surface area, the high percentage extracellular fluid, and the limited ability of the kidneys to conserve water. After the removal ofa tooth, after an oral trauma or in case of oral discomfort, a child is at greater risk of dehydration by reduced fluid and food intake due to oral pain and/or discomfort and anxiety to drink. In those cases, extra attention needs to be devoted to the intake of fluids.

Comparison of clinical and biochemical markers of dehydration with the clinical dehydration scale in children: a case comparison trial.

Science.gov (United States)

Tam, Ron K; Wong, Hubert; Plint, Amy; Lepage, Nathalie; Filler, Guido

2014-06-16

The clinical dehydration scale (CDS) is a quick, easy-to-use tool with 4 clinical items and a score of 1-8 that serves to classify dehydration in children with gastroenteritis as no, some or moderate/severe dehydration. Studies validating the CDS (Friedman JN) with a comparison group remain elusive. We hypothesized that the CDS correlates with a wide spectrum of established markers of dehydration, making it an appropriate and easy-to-use clinical tool. This study was designed as a prospective double-cohort trial in a single tertiary care center. Children with diarrhea and vomiting, who clinically required intravenous fluids for rehydration, were compared with minor trauma patients who required intravenous needling for conscious sedation. We compared the CDS with clinical and urinary markers (urinary electrolytes, proteins, ratios and fractional excretions) for dehydration in both groups using receiver operating characteristic (ROC) curves to determine the area under the curve (AUC). We enrolled 73 children (male = 36) in the dehydration group and 143 (male = 105) in the comparison group. Median age was 32 months (range 3-214) in the dehydration and 96 months (range 2.6-214 months, p dehydration group and 0 in the comparison group (p dehydrated group: difference in heart rate, diastolic blood pressure, urine sodium/potassium ratio, urine sodium, fractional sodium excretion, serum bicarbonate, and creatinine measurements. The best markers for dehydration were urine Na and serum bicarbonate (ROC AUC = 0.798 and 0.821, respectively). CDS was most closely correlated with serum bicarbonate (Pearson r = -0.3696, p = 0.002). Although serum bicarbonate is not the gold standard for dehydration, this study provides further evidence for the usefulness of the CDS as a dehydration marker in children. Registered at ClinicalTrials.gov (NCT00462527) on April 18, 2007.

The diagnostic accuracy of Clinical Dehydration Scale in identifying dehydration in children with acute gastroenteritis: a systematic review.

Science.gov (United States)

Falszewska, Anna; Dziechciarz, Piotr; Szajewska, Hania

2014-10-01

To systematically update diagnostic accuracy of the Clinical Dehydration Scale (CDS) in clinical recognition of dehydration in children with acute gastroenteritis. Six databases were searched for diagnostic accuracy studies in which population were children aged 1 to 36 months with acute gastroenteritis; index test was the CDS; and reference test was post-illness weight gain. Three studies involving 360 children were included. Limited evidence showed that in high-income countries the CDS provides strong diagnostic accuracy for ruling in moderate and severe (>6%) dehydration (positive likelihood ratio 5.2-6.6), but has limited value for ruling it out (negative likelihood ratio 0.4-0.55). In low-income countries, the CDS has limited value either for ruling moderate or severe dehydration in or out. In both settings, the CDS had limited value for ruling in or out dehydration dehydration 3% to 6%. The CDS can help assess moderate to severe dehydration in high-income settings. Given the limited data, the evidence should be viewed with caution. © The Author(s) 2014.

Splenic Involvement in Hereditary Hemorrhagic Telangiectasia

Directory of Open Access Journals (Sweden)

Susumu Takamatsu

2016-01-01

Full Text Available A 33-year-old man who presented with prolonged epigastric pain was referred to our hospital. He had experienced recurrent epistaxis and had a family history of hereditary hemorrhagic telangiectasia. Computed tomography and magnetic resonance imaging revealed splenomegaly and a 9 cm hypervascular mass in his spleen. Computed tomography also showed a pulmonary arteriovenous malformation and heterogeneous enhancement of the liver parenchyma, suggesting the presence of arteriosystemic shunts and telangiectases. Based on these findings, the patient was definitely diagnosed with hereditary hemorrhagic telangiectasia according to Curaçao criteria. He underwent splenectomy, and his symptoms disappeared after surgery. Pathological examination of the resected specimen revealed that the hypervascular lesion of the spleen was not a tumor but was composed of abnormal vessels associated with hereditary hemorrhagic telangiectasia. Symptomatic splenic involvement may be a rare manifestation of hereditary hemorrhagic telangiectasia but can be revealed by imaging modalities.

Genetics Home Reference: hereditary hemorrhagic telangiectasia

Science.gov (United States)

... Central OMIM: JUVENILE POLYPOSIS/HEREDITARY HEMORRHAGIC TELANGIECTASIA SYNDROME McDonald J, Bayrak-Toydemir P, Pyeritz RE. Hereditary hemorrhagic ... 10.1097/GIM.0b013e3182136d32. Review. Citation on PubMed McDonald J, Wooderchak-Donahue W, VanSant Webb C, Whitehead ...

Loss-of-function and gain-of-function phenotypes of stomatocytosis mutant RhAG F65S

Science.gov (United States)

Stewart, Andrew K.; Shmukler, Boris E.; Vandorpe, David H.; Rivera, Alicia; Heneghan, John F.; Li, Xiaojin; Hsu, Ann; Karpatkin, Margaret; O'Neill, Allison F.; Bauer, Daniel E.; Heeney, Matthew M.; John, Kathryn; Kuypers, Frans A.; Gallagher, Patrick G.; Lux, Samuel E.; Brugnara, Carlo; Westhoff, Connie M.

2011-01-01

Four patients with overhydrated cation leak stomatocytosis (OHSt) exhibited the heterozygous RhAG missense mutation F65S. OHSt erythrocytes were osmotically fragile, with elevated Na and decreased K contents and increased cation channel-like activity. Xenopus oocytes expressing wild-type RhAG and RhAG F65S exhibited increased ouabain and bumetanide-resistant uptake of Li+ and 86Rb+, with secondarily increased 86Rb+ influx sensitive to ouabain and to bumetanide. Increased RhAG-associated 14C-methylammonium (MA) influx was severely reduced in RhAG F65S-expressing oocytes. RhAG-associated influxes of Li+, 86Rb+, and 14C-MA were pharmacologically distinct, and Li+ uptakes associated with RhAG and RhAG F65S were differentially inhibited by NH4+ and Gd3+. RhAG-expressing oocytes were acidified and depolarized by 5 mM bath NH3/NH4+, but alkalinized and depolarized by subsequent bath exposure to 5 mM methylammonium chloride (MA/MA+). RhAG F65S-expressing oocytes exhibited near-wild-type responses to NH4Cl, but MA/MA+ elicited attenuated alkalinization and strong hyperpolarization. Expression of RhAG or RhAG F65S increased steady-state cation currents unaltered by bath Li+ substitution or bath addition of 5 mM NH4Cl or MA/MA+. These oocyte studies suggest that 1) RhAG expression increases oocyte transport of NH3/NH4+ and MA/MA+; 2) RhAG F65S exhibits gain-of-function phenotypes of increased cation conductance/permeability, and loss-of-function phenotypes of decreased and modified MA/MA+ transport, and decreased NH3/NH4+-associated depolarization; and 3) RhAG transports NH3/NH4+ and MA/MA+ by distinct mechanisms, and/or the substrates elicit distinct cellular responses. Thus, RhAG F65S is a loss-of-function mutation for amine transport. The altered oocyte intracellular pH, membrane potential, and currents associated with RhAG or RhAG F65S expression may reflect distinct transport mechanisms. PMID:21849667

THERAPY OF DIARRHEA AND DEHYDRATION IN CHILDREN

OpenAIRE

E.G. Tsymbalova; R.F. Tepaev

2011-01-01

Dehydration is one of the leading causes of mortality in children. The most frequent cause of dehydration in children is diarrhea syndrome. Timely differential diagnostics of etiological causes, assessment of severity, dehydration type are critical conditions of successful therapy of diarrhea syndrome. The article provides modern recommendations on diagnostics and treatment of dehydration depending on the type and severity, on correction of electrolyte abnormalities. The authors also highligh...

Assessment of extracellular dehydration using saliva osmolality.

Science.gov (United States)

Ely, Brett R; Cheuvront, Samuel N; Kenefick, Robert W; Spitz, Marissa G; Heavens, Kristen R; Walsh, Neil P; Sawka, Michael N

2014-01-01

When substantial solute losses accompany body water an isotonic hypovolemia (extracellular dehydration) results. The potential for using blood or urine to assess extracellular dehydration is generally poor, but saliva is not a simple ultra-filtrate of plasma and the autonomic regulation of salivary gland function suggests the possibility that saliva osmolality (Sosm) may afford detection of extracellular dehydration via the influence of volume-mediated factors. This study aimed to evaluate the assessment of extracellular dehydration using Sosm. In addition, two common saliva collection methods and their effects on Sosm were compared. Blood, urine, and saliva samples were collected in 24 healthy volunteers during paired euhydration and dehydration trials. Furosemide administration and 12 h fluid restriction were used to produce extracellular dehydration. Expectoration and salivette collection methods were compared in a separate group of eight euhydrated volunteers. All comparisons were made using paired t-tests. The diagnostic potential of body fluids was additionally evaluated. Dehydration (3.1 ± 0.5% loss of body mass) decreased PV (-0.49 ± 0.12 L; -15.12 ± 3.94% change), but Sosm changes were marginal ( 0.05). Extracelluar dehydration was not detectable using plasma, urine, or saliva measures. Salivette and expectoration sampling methods produced similar, consistent results for Sosm, suggesting no methodological influence on Sosm.

Dehydration survival of crop plants and its measurement.

Science.gov (United States)

Blum, Abraham; Tuberosa, Roberto

2018-01-08

Dehydration survival under drought stress is defined in this review as the transition from plant activity into a quiescent state of life preservation, which will be terminated by either recovery or death, depending on the stress regime and the plant's resilience. Dehydration survival is a popular phenotype by which functional genomics attempts to test gene function in drought resistance and survival. The available reports on phenotyping and genotyping of dehydration survival in genomic studies indicate that the measurement of this trait is often biased to the extent that misguided interpretations are likely to occur. This review briefly discusses the physiological basis of dehydration survival in resurrection plants and crop plants, and concludes that in phenotyping dehydration survival there is a need to distinguish between dehydration avoidance and dehydration tolerance (also termed desiccation tolerance) in affecting survival and recovery. Without this distinction, functional genomics studies of the trait might be biased. Survival due to dehydration avoidance is expressed by the capacity to maintain a relatively high plant water status as the plant is desiccated. Survival due to dehydration tolerance is expressed by delayed mortality (mortality at a relatively low plant water status) as affected by the resilience of plant metabolism. The common test of dehydration survival, using the relative recovery after a given number of stress days, is therefore insufficient because it is mainly driven by dehydration avoidance and so ignores a possible role for dehydration tolerance. Conceivable methods for more accurate phenotyping of the two components of dehydration survival are proposed and discussed. © The Author(s) 2018. Published by Oxford University Press on behalf of the Society for Experimental Biology. All rights reserved. For permissions, please email: journals.permissions@oup.com.

Hereditary neuropathies: systematization and diagnostics (clinical case of hereditary motor and sensor neuropathy of the IA type

Directory of Open Access Journals (Sweden)

Kolokolova A.M.

2016-09-01

Full Text Available Aim: to study the value of routine methods (clinical symptoms, electrophysiological findings and results of DNA analysis in diagnostics of hereditary motor sensory neuropathy type IA in outpatient clinics. Material and Methods. The review of foreign literature is represented. The phenotypic polymorphism, genetic heterogeneity and the difficulties of diagnostics are identified. A family with hereditary motor sensory neuropathy of lAtype is presented, which was diagnosed on the base of available methods in outpatient practice (clinical symptoms, genealogical method, electro-physiological findings and DNA analysis results. Results. Routine algorithm (consistent valuation of clinical symptoms, neurophysiologic findings and the results of DNA analysis helped to verify the diagnosis of hereditary motor sensory neuropathy of lAtype in outpatient practice after more than 20 years of the onset of the disease. Conclusion. The neurologists of outpatient clinics and other specialists must be informed about the availability of diagnostics of hereditary diseases of nervous system.

The dehydration of uranyl nitrate hexahydrate

International Nuclear Information System (INIS)

Badalov, A.; Kamalov, D.D.; Khamidov, B.O.; Mirsaidov, I.U.; Eshbekov, N.R.

2010-01-01

Present article is devoted to study of dehydration process of uranyl nitrate hexahydrate. The dehydration process of uranyl nitrate hexahydrate was studied by means of tensimeter method with membrane zero-manometer. The research was carried out under equilibrium conditions. It was defined that in studied temperature ranges (300-450 K) the dehydration process of UO_2(NO_3)_2 has a three stage character.

Genetics Home Reference: hereditary diffuse gastric cancer

Science.gov (United States)

... Health Conditions Hereditary diffuse gastric cancer Hereditary diffuse gastric cancer Printable PDF Open All Close All Enable Javascript ... Diffuse Gastric Cancer MedlinePlus Encyclopedia: Gastric Cancer National Cancer ... Option Overview General Information from MedlinePlus ( ...

Breastfeeding-associated hypernatraemic dehydration

Directory of Open Access Journals (Sweden)

Sónia Melo Gomes

2009-03-01

Full Text Available Objective: In the last few years there has been an increase in case reports of hypernatraemic dehydration in breastfed newborns. Insufficient intake has an important role in the pathophysiology of this condition. The aim of this study was to evaluate exclusively breastfed neonates admitted for hypernatraemic dehydration. Methods: Retrospective study of breastfed neonates diagnosed with hypernatraemic dehydration, between March 2002 and March 2008, in a level 1 maternity. Rresults: Nineteen cases were identified (0.44% of neonatal intermediate care hospitalizations, 53% of them were male. The annual distribution revealed a higher number of cases in 2008: 26.3% in only three months. Median birth weight was 3,000 g and the median gestational age was 38 weeks. Vaginal delivery was the most frequent form of birth (42%, and 79% of mothers were primiparas. Admissions were made through the emergency department in 68.4%. The main reasons for seeking medical attention were: poor oral intake (32%, weight loss (26%, and jaundice (26%. The median age at admission was four days. Percentage of weight loss: 6.7 to 40%, median was 11%. Dehydration signs were absent in 42% of the patients. Median Na+ values were 152 mEq/l. Jaundice was the most frequent comorbidity found (74%. Intravenous fluids were administered in 89% and acute neurological complications were found in 21%, there were no deaths. Cconclusions: Breastfeeding-associated hypernatraemic dehydration seems to be a consequence of breastfeeding difficulties in inexperienced mothers. Since many cases are paucisymptomatic, there should be a high level of suspicion, especially in those patients with jaundice.

Drug therapy for hereditary cancers

Directory of Open Access Journals (Sweden)

Imyanitov Evgeny N

2011-08-01

Full Text Available Abstract Tumors arising in patients with hereditary cancer syndromes may have distinct drug sensitivity as compared to their sporadic counterparts. Breast and ovarian neoplasms from BRCA1 or BRCA2 mutation carriers are characterized by deficient homologous recombination (HR of DNA, that makes them particularly sensitive to platinum compounds or inhibitors of poly (ADP-ribose polymerase (PARP. Outstandingly durable complete responses to high dose chemotherapy have been observed in several cases of BRCA-related metastatic breast cancer (BC. Multiple lines of evidence indicate that women with BRCA1-related BC may derive less benefit from taxane-based treatment than other categories of BC patients. There is virtually no reports directly assessing drug response in hereditary colorectal cancer (CRC patients; studies involving non-selected (i.e., both sporadic and hereditary CRC with high-level microsatellite instability (MSI-H suggest therapeutic advantage of irinotecan. Celecoxib has been approved for the treatment of familial adenomatous polyposis (FAP. Hereditary medullary thyroid cancers (MTC have been shown to be highly responsive to a multitargeted tyrosine kinase inhibitor vandetanib, which exerts specific activity towards mutated RET receptor. Given the rapidly improving accessibility of DNA analysis, it is foreseen that the potential predictive value of cancer-associated germ-line mutations will be increasingly considered in the future studies.

Genetic profiles distinguish different types of hereditary ovarian cancer

DEFF Research Database (Denmark)

Domanska, Katarina; Malander, Susanne; Staaf, Johan

2010-01-01

(HBOC) syndrome and the hereditary non-polyposis colorectal cancer (HNPCC) syndrome. Genome-wide array comparative genomic hybridization was applied to 12 HBOC associated tumors with BRCA1 mutations and 8 HNPCC associated tumors with mismatch repair gene mutations with 24 sporadic ovarian cancers......Heredity represents the strongest risk factor for ovarian cancer with disease predisposing mutations identified in 15% of the tumors. With the aim to identify genetic classifiers for hereditary ovarian cancer, we profiled hereditary ovarian cancers linked to the hereditary breast and ovarian cancer...... that HBOC and HNPCC associated ovarian cancer develop along distinct genetic pathways and genetic profiles can thus be applied to distinguish between different types of hereditary ovarian cancer....

Diagnostic accuracy of clinical dehydration scales in children.

Science.gov (United States)

Falszewska, Anna; Dziechciarz, Piotr; Szajewska, Hania

2017-08-01

The aim of this study was to evaluate the diagnostic accuracy of the Clinical Dehydration Scale (CDS), the World Health Organization (WHO) scale, and the Gorelick scale for dehydration assessment in children. A prospective, observational study was carried out between October 2014 and December 2016. Eligible participants were children aged 1 month to 5 years with acute diarrhea. After hospital admission, each patient's weight was recorded and the degree of dehydration based on three scales was assessed. The reference standard was the percentage weight change between the discharge and admission weights. The main outcomes were the sensitivity, specificity, positive likelihood ratio (LR), and negative LR. Of 128 children enrolled in the study, complete data were available from 118 patients for analysis. Most of children presented with no or mild dehydration. Only the CDS showed limited value in confirming a diagnosis of dehydration ≥6% (positive LR 3.9, 95% CI 1.1 to 9.1), with no value in ruling it out (negative LR 0.6, 95% CI 0.2 to 0.99). In our cohort, the CDS was of limited diagnostic value in ruling in severe dehydration in children with acute gastroenteritis. The WHO and Gorelick scales were not helpful in the assessment of dehydration. What is Known : • Treatment of acute gastroenteritis (AGE) is based on assessing and correcting the degree of dehydration. • Several scales combining various signs and symptoms have been developed, including the Clinical Dehydration Scale (CDS), and the World Health Organization (WHO) scale, and the Gorelick scale. None of these scales is internationally accepted for best accuracy in diagnosing dehydration in children. What is New: • The CDS was of limited diagnostic value in ruling in severe dehydration in children with AGE. • The WHO and Gorelick scales were not helpful in the assessment of dehydration.

Hereditary haemorrhagic telangiectasia

DEFF Research Database (Denmark)

Kjeldsen, A D; Vase, P; Green, A

1999-01-01

Hereditary haemorrhagic telangiectasia (HHT) is a dominantly inherited disease characterized by telangiectatic lesions. The disease manifestations are variable and include epistaxis, gastrointestinal bleeding, pulmonary arteriovenous malformations and cerebral arteriovenous malformations. Early...

Hereditary spherocytosis: Consequences of delayed diagnosis

Directory of Open Access Journals (Sweden)

Sarah C Steward

2014-08-01

Full Text Available Objective: To determine whether patients with undiagnosed hereditary spherocytosis hospitalized for transfusions might have avoided hospitalization via earlier diagnosis. Study design: Charts of all (N = 30 patients with hereditary spherocytosis seen in pediatric hematology at West Virginia University-Charleston were reviewed. Family and transfusion history and presence of neonatal jaundice were recorded. Complete blood count and reticulocyte values during infancy were available for 20 of 30 patients, while baseline steady-state values were available for all 30. Results: Transfusions were given to 22 patients; 12 of 14 with an aplastic crisis were undiagnosed. In 10 of 12, the severity of anemia led to hospitalization (3 to intensive care. All 10 had prior mean corpuscular hemoglobin concentration and/or red cell distribution width elevations and a history of neonatal jaundice; 7 of 10 had a positive family history. Conclusions: Undiagnosed hereditary spherocytosis may lead to inpatient transfusions for severe anemia. Earlier detection of hereditary spherocytosis is easily achievable and may reduce hospitalizations via closer monitoring.

Downhole dehydration - status report and implementation study; Downhole Dehydration - Statusbericht und Umsetzungsstudie

Energy Technology Data Exchange (ETDEWEB)

Jacobs, D.; Schmidt, D. [BEB Erdgas und Erdoel GmbH, Hannover (Germany)

1998-12-31

Downhole dehydration, i.e. in-situ separation of water and oil, is an interesting new technology. The contribution describes the technology and the results of a field experiment. (orig.) [Deutsch] Die Tail-End Foerderphase in der deutschen Erdoelproduktion, welche durch hohe Wasserhebekosten gekennzeichnet ist, erfordert zur Aufrechterhaltung der Wirtschaftlichkeit neue Gedankenansaetze. Ein aus wirtschaftlicher und technischer Sicht reizvoller Optimierungsgedanke ist die untertaegige Wasser/Oel Separation, auch Downhole Dehydration genannt. Unter Downhole Dehydration (DHD) versteht man also die untertaegige (teilweise) Separation des Lagerstaettenwassers vom Nassoel, kurz nachdem das Gemisch untertaegig in das Bohrloch eingetreten ist. Dabei wird das abgetrennte Lagerstaettenwasser untertage in einen geeigneten Horizont unmittelbar wieder injiziert und das Oel (wie bei der konventionellen Foerderung) zutage gepumpt, mit dem Ziel der Nutzung der daraus resultierenden Kosten- und Investitionsersparnis. Ziel dieses Vortrages ist es, einen kurzen Einblick in die erstmalig in Europa angewandte Technik zu geben und von den Erfahrungen des praktizierten Feldversuches zu berichten. (orig.)

Hereditary & familial colorectal cancer : Identification, characteristics, surveillance

NARCIS (Netherlands)

Kallenberg, F.G.J.

2017-01-01

Of all colorectal cancer (CRC) cases, 15-20% is related to familial or hereditary factors. Diagnosing familial and hereditary CRC syndromes is important for several reasons. One of these is that surveillance colonoscopies can reduce CRC incidence and mortality importantly. A complete family history

Brazilian guidelines for the diagnosis and treatment of hereditary angioedema.

Science.gov (United States)

Giavina-Bianchi, Pedro; França, Alfeu T; Grumach, Anete S; Motta, Abílio A; Fernandes, Fátima R; Campos, Regis A; Valle, Solange O; Rosário, Nelson A; Sole, Dirceu

2011-01-01

Hereditary angioedema is an autosomal dominant disease characterized by edema attacks with multiple organ involvement. It is caused by a quantitative or functional deficiency of the C1 inhibitor, which is a member of the serine protease inhibitor family. Hereditary angioedema is unknown to many health professionals and is therefore an underdiagnosed disease. The causes of death from hereditary angioedema include laryngeal edema with asphyxia. The estimated mortality rate in patients in whom the disease goes undetected and who are therefore incorrectly treated is 25-40%. In addition to edema of the glottis, hereditary angioedema often results in edema of the gastrointestinal tract, which can be incapacitating. Patients with hereditary angioedema may undergo unnecessary surgical interventions because the digestive tract can be the primary or only organ system involved, thus mimicking acute surgical abdomen. It is estimated that patients with hereditary angioedema experience some degree of disability 20-100 days per year. The Experts in Clinical Immunology and Allergy of the "Associação Brasileira de Alergia e Imunopatologia -ASBAI" developed these guidelines for the diagnosis, therapy, and management of hereditary angioedema.

Serum bicarbonate and dehydration severity in gastroenteritis

OpenAIRE

Narchi, H.

1998-01-01

The concentration of bicarbonate was measured in serum samples from 106 children with gastroenteritis and dehydration. A concentration less than 22 mmol/l was more common in children with severe dehydration, but the magnitude of bicarbonate reduction was not significantly different with increasing degrees of dehydration. Doctors should not rely on the serum bicarbonate concentration when assessing fluid deficit.



Fructokinase activity mediates dehydration-induced renal injury.

Science.gov (United States)

Roncal Jimenez, Carlos A; Ishimoto, Takuji; Lanaspa, Miguel A; Rivard, Christopher J; Nakagawa, Takahiko; Ejaz, A Ahsan; Cicerchi, Christina; Inaba, Shinichiro; Le, MyPhuong; Miyazaki, Makoto; Glaser, Jason; Correa-Rotter, Ricardo; González, Marvin A; Aragón, Aurora; Wesseling, Catharina; Sánchez-Lozada, Laura G; Johnson, Richard J

2014-08-01

The epidemic of chronic kidney disease in Nicaragua (Mesoamerican nephropathy) has been linked with recurrent dehydration. Here we tested whether recurrent dehydration may cause renal injury by activation of the polyol pathway, resulting in the generation of endogenous fructose in the kidney that might subsequently induce renal injury via metabolism by fructokinase. Wild-type and fructokinase-deficient mice were subjected to recurrent heat-induced dehydration. One group of each genotype was provided water throughout the day and the other group was hydrated at night, after the dehydration. Both groups received the same total hydration in 24 h. Wild-type mice that received delayed hydration developed renal injury, with elevated serum creatinine, increased urinary NGAL, proximal tubular injury, and renal inflammation and fibrosis. This was associated with activation of the polyol pathway, with increased renal cortical sorbitol and fructose levels. Fructokinase-knockout mice with delayed hydration were protected from renal injury. Thus, recurrent dehydration can induce renal injury via a fructokinase-dependent mechanism, likely from the generation of endogenous fructose via the polyol pathway. Access to sufficient water during the dehydration period can protect mice from developing renal injury. These studies provide a potential mechanism for Mesoamerican nephropathy.

Genetics Home Reference: hereditary sensory neuropathy type IA

Science.gov (United States)

... sensory neuropathy type IA Hereditary sensory neuropathy type IA Printable PDF Open All Close All Enable Javascript ... expand/collapse boxes. Description Hereditary sensory neuropathy type IA is a condition characterized by nerve abnormalities in ...

Ethanol dehydration on doped cadmium oxide

International Nuclear Information System (INIS)

Abd El-Salaam, K.M.

1975-01-01

The vapour phase catalytic dehydration of ethanol over Fe impregnated cadmium oxide was investigated between 200-450 0 C in atmospheric pressure. Electron transfer mechanisms involved in adsorption and catalytic dehydration reaction were investigated. The change in electrical conductivity of the catalyst resulting from calcination, adsorption and surface reaction processes were studied. Adsorption conductivity at low temperature ( 0 C) indicates that ethanol adsorbs as an electron donor. A mechanism of creation of interstitial Cd atoms responsible for the catalytic dehydration of ethanol on the catalyst surface was suggested. (orig.) [de

Electrolyte Concentrates Treat Dehydration

Science.gov (United States)

2009-01-01

Wellness Brands Inc. of Boulder, Colorado, exclusively licensed a unique electrolyte concentrate formula developed by Ames Research Center to treat and prevent dehydration in astronauts returning to Earth. Marketed as The Right Stuff, the company's NASA-derived formula is an ideal measure for athletes looking to combat dehydration and boost performance. Wellness Brands also plans to expand with products that make use of the formula's effective hydration properties to help treat conditions including heat stroke, altitude sickness, jet lag, and disease.

[Multicenter validation of the clinical dehydration scale for children].

Science.gov (United States)

Gravel, J; Manzano, S; Guimont, C; Lacroix, L; Gervaix, A; Bailey, B

2010-12-01

Dehydration is an important complication for sick children. The Clinical Dehydration Scale for children (CDS) measures dehydration based on 4 clinical signs: general appearance, eyes, saliva, and tears. To validate the association between the CDS and markers of dehydration in children aged 1 month to 5 years visiting emergency departments (EDs) for vomiting and/or diarrhea. An international prospective cohort study conducted in 3 university-affiliated EDs in 2009. Participants were a convenience sample of children aged 1-60 months presenting to the ED for acute vomiting and/or diarrhea. Following triage, a research nurse obtained informed consent and evaluated dehydration using the CDS. A few days after recovery, another research assistant weighed participants at home. The primary outcome was the percentage of dehydration calculated by the difference in weight at first evaluation and after recovery. Secondary outcomes included proportion of blood test measurements, intravenous use, hospitalization, and inter-rater agreement. During the study period, 264 children were recruited and data regarding weight and dehydration scores were complete for 219 (83%). According to the CDS, 88 had no dehydration, 159 some dehydration, and 15 moderate or severe dehydration. A Chi-square test showed a statistical association between CDS and weight gain, the occurrence of blood tests, intravenous rehydration, hospitalization, and abnormal plasmatic bicarbonate. Good inter-rater correlation was found among participants (linear weighted Kappa score of 0.65; (95% CI, 0.43-0.87). CDS categories correlate with markers of dehydration for young children complaining of vomiting and/or diarrhea in the ED. Copyright © 2010 Elsevier Masson SAS. All rights reserved.

Dehydration of aluminum chloride hexahydrate: a technical assessment

International Nuclear Information System (INIS)

Miller, R.L.

1984-04-01

Physical and chemical properties of aluminum chloride hexahydrate, ACH, and related compounds are noted as well as the relation of these properties to the problem of dehydration of ACH to form anhydrous aluminum chloride. Several approaches to the dehydration of ACH are discussed and a rationale or each approach is given: solvent extraction, ligand exchange, azeotropic distillation, chemical dehydration, and energy specific direct heating. While the thermodynamics of dehydration are frequently favorable, the rate of reaction and the mechanisms of reaction are more frequently unfavorable. However, each approach requires experimentation to test its effectiveness. The stability of the aluminum-oxygen bond in the hydrate is such that dehydration without decomposition to the oxide will be very difficult

Brazilian guidelines for the diagnosis and treatment of hereditary angioedema

Directory of Open Access Journals (Sweden)

Pedro Giavina-Bianchi

2011-01-01

Full Text Available Hereditary angioedema is an autosomal dominant disease characterized by edema attacks with multiple organ involvement. It is caused by a quantitative or functional deficiency of the C1 inhibitor, which is a member of the serine protease inhibitor family. Hereditary angioedema is unknown to many health professionals and is therefore an underdiagnosed disease. The causes of death from hereditary angioedema include laryngeal edema with asphyxia. The estimated mortality rate in patients in whom the disease goes undetected and who are therefore incorrectly treated is 25-40%. In addition to edema of the glottis, hereditary angioedema often results in edema of the gastrointestinal tract, which can be incapacitating. Patients with hereditary angioedema may undergo unnecessary surgical interventions because the digestive tract can be the primary or only organ system involved, thus mimicking acute surgical abdomen. It is estimated that patients with hereditary angioedema experience some degree of disability 20-100 days per year. The Experts in Clinical Immunology and Allergy of the "Associação Brasileira de Alergia e Imunopatologia -ASBAI" developed these guidelines for the diagnosis, therapy, and management of hereditary angioedema.

Thermodynamics of mercaptopurine dehydration.

Science.gov (United States)

Niazi, S

1978-04-01

The hydrate form of mercaptopurine was shown to undergo peritectic decomposition of its water molecule, localized dissolution, and dehydration around 125 degrees. The anhydrate form was prepared by a thermal method, whose effectiveness was confirmed by X-ray diffraction, NMR spectroscopy, and differential scanning calorimetry. The activation energy for mercaptopurine dehydration calculated by various methods ranged from 45.74 to 63.04 kcal/mole. The dehydration enthalpy was calculated to be 8.27 kcal/mole by differential scanning calorimetry. The solution enthalpy for the hydrate was calculated to be 4.85 kcal/mole from its saturation solubility and differential scanning calorimetry. Anhydrate solubility in water was calculated based on initial dissolution rate data since the anhydrate converts to hydrate in aqueous media. The high degree of stability against interconversion of the hydrate and anhydrate forms and the higher solubility of the anhydrate suggest that use of the anhydrate might improve mercaptopurine bioavailability.

Overexpression of LOV KELCH protein 2 confers dehydration tolerance and is associated with enhanced expression of dehydration-inducible genes in Arabidopsis thaliana.

Science.gov (United States)

Miyazaki, Yuji; Abe, Hiroshi; Takase, Tomoyuki; Kobayashi, Masatomo; Kiyosue, Tomohiro

2015-05-01

The overexpression of LKP2 confers dehydration tolerance in Arabidopsis thaliana ; this is likely due to enhanced expression of dehydration-inducible genes and reduced stomatal opening. LOV KELCH protein 2 (LKP2) modulates the circadian rhythm and flowering time in plants. In this study, we observed that LKP2 overexpression enhanced dehydration tolerance in Arabidopsis. Microarray analysis demonstrated that expression of water deprivation-responsive genes was higher in the absence of dehydration stress in transgenic Arabidopsis plants expressing green fluorescent protein-tagged LKP2 (GFP-LKP2) than in control transgenic plants expressing GFP. After dehydration followed by rehydration, GFP-LKP2 plants developed more leaves and roots and exhibited higher survival rates than control plants. In the absence of dehydration stress, four dehydration-inducible genes, namely DREB1A, DREB1B, DREB1C, and RD29A, were expressed in GFP-LKP2 plants, whereas they were not expressed or were expressed at low levels in control plants. Under dehydration stress, the expression of DREB2B and RD29A peaked faster in the GFP-LKP2 plants than in control plants. The stomatal aperture of GFP-LKP2 plants was smaller than that of control plants. These results suggest that the dehydration tolerance of GFP-LKP2 plants is caused by upregulation of DREB1A-C/CBF1-3 and their downstream targets; restricted stomatal opening in the absence of dehydration stress also appears to contribute to the phenotype. The rapid and high expression of DREB2B and its downstream target genes also likely accounts for some features of the GFP-LKP2 phenotype. Our results suggest that LKP2 can be used for biotechnological applications not only to adjust the flowering time control but also to enhance dehydration tolerance.

EAMJ Oct Hereditary.indd

African Journals Online (AJOL)

HEREDITARY GINGIVAL FIBROMATOSIS: REPORT OF FAMILY CASE SERIES. E. G. Wagaiyu ... Senior Lecturer, Department of Periodontology/Community and Preventive Dentistry, ... fibrous connective tissue held in chronically inflamed.

Dehydration related abdominal pain (drap)

International Nuclear Information System (INIS)

Shah, S.I.; Aurangzeb; Khan, I.; Bhatti, A.M.; Khan, A.A.

2004-01-01

Objective: To describe the frequency of dehydration as a medical cause of acute abdomen. Subjects and Methods: All the patients reporting with abdominal pain to the surgical outpatient department or the emergency department were reviewed in the study. The clinical findings in all these cases were studied along with the mode of their management and outcome. Results: Of all the patients presenting with abdominal pain, 3.3% (n=68) were suffering from dehydration related abdominal pain. They were predominantly males in a ratio of 8.7: 1, mostly in the 2nd and 3rd decades of their lives. All these cases were suffering from acute or chronic dehydration were provisionally diagnosed by general practitioners as 'acute abdomen' and referred for surgical consultation. Associated symptoms included vomiting in 42.6%, backache in 91.2%, headache in 95.6%, and pain in lower limbs in 97.1 % of the cases. 83.8% required indoor management with intravenous fluids. All the patients became asymptomatic with rehydration therapy. Conclusion: Dehydration is a possible cause of severe abdominal pain. There is a need to educate the general public about the benefits of adequate fluid intake. (author)

Learning about Hereditary Hemochromatosis

Science.gov (United States)

Skip to main content Learning About Hereditary Hemochromatosis Enter Search Term(s): Español Research Funding An Overview Bioinformatics Current Grants Education and Training Funding Extramural Research ...

Hereditary breast cancer

DEFF Research Database (Denmark)

Larsen, Martin J; Thomassen, Mads; Gerdes, Anne-Marie

2014-01-01

Pathogenic mutations in BRCA1 or BRCA2 are only detected in 25% of families with a strong history of breast cancer, though hereditary factors are expected to be involved in the remaining families with no recognized mutation. Molecular characterization is expected to provide new insight...... into the tumor biology to guide the search of new high-risk alleles and provide better classification of the growing number of BRCA1/2 variants of unknown significance (VUS). In this review, we provide an overview of hereditary breast cancer, its genetic background, and clinical implications, before focusing...... on the pathologically and molecular features associated with the disease. Recent transcriptome and genome profiling studies of tumor series from BRCA1/2 mutation carriers as well as familial non-BRCA1/2 will be discussed. Special attention is paid to its association with molecular breast cancer subtypes as well...

Irradiation of dehydrated vegetables

International Nuclear Information System (INIS)

Esterhuyse, A; Esterhuizen, T.

1985-01-01

The reason for radurization was to decreased the microbial count of dehydrated vegetables. The average absorbed irradiation dose range between 2kGy and 15kGy. The product catagories include a) Green vegetables b) White vegetables c) Powders of a) and b). The microbiological aspects were: Declining curves for the different products of T.P.C., Coliforms, E. Coli, Stap. areus, Yeast + Mold at different doses. The organoleptical aspects were: change in taste, flavour, texture, colour and moisture. The aim is the marketing of irradiated dehydrated vegetables national and international basis

Hypertension despite dehydration during severe pediatric diabetic ketoacidosis.

Science.gov (United States)

Deeter, Kristina H; Roberts, Joan S; Bradford, Heidi; Richards, Todd; Shaw, Dennis; Marro, Kenneth; Chiu, Harvey; Pihoker, Catherine; Lynn, Anne; Vavilala, Monica S

2011-06-01

Diabetic ketoacidosis (DKA) may result in both dehydration and cerebral edema but these processes may have opposing effects on blood pressure. We examined the relationship between dehydration and blood pressure in pediatric DKA. A retrospective review was performed at Seattle Children's Hospital, Seattle, WA. Participants were hospitalized children less than 18 yr. Intervention(s) or main exposure was to patients with DKA (venous pH 300 mg/dL, HCO(3) Dehydration was calculated as percent body weight lost at admission compared to discharge. Hypertension (systolic and/or diastolic blood pressure (DBP) percentile > 95%) was defined based on National Heart, Lung, and Blood Institute (NHLBI, 2004) nomograms and hypotension was defined as systolic blood pressure (SBP) dehydration. Despite dehydration, most children admitted with severe DKA had hypertension. © 2011 John Wiley & Sons A/S.

A Review of Hereditary Fructose Intolerance

Directory of Open Access Journals (Sweden)

Mogoş Tiberius

2016-03-01

Full Text Available Fructose intolerance is a metabolic disorder with hereditary determinism, clinically manifested on terms of fructose intake. Untreated, hereditary fructose intolerance may result in renal and hepatic failure. Unfortunately, there are no formal diagnostic and surveillance guidelines for this disease. If identified and treated before the occurrence of permanent organ damage, patients can improve their symptoms and self-rated health. Implementation and adherence to a strict fructose free diet is often difficult, but not impossible.

Pancreatic cancer risk in hereditary pancreatitis

Directory of Open Access Journals (Sweden)

Frank Ulrich Weiss

2014-02-01

Full Text Available Inflammation is part of the body’s immune response in order to remove harmful stimuli – like pathogens, irritants or damaged cells - and start the healing process. Recurrent or chronic inflammation on the other side seems a predisposing factor for carcinogenesis and has been found associated with cancer development. In chronic pancreatitis mutations of the cationic trypsinogen (PRSS1 gene have been identified as risk factors of the disease. Hereditary pancreatitis is a rare cause of chronic pancreatic inflammation with an early onset, mostly during childhood. Hereditary pancreatitis often starts with recurrent episodes of acute pancreatitis and the clinical phenotype is not very much different from other etiologies of the disease. The long-lasting inflammation however generates a tumor promoting environment and represents a major risk factor for tumor development This review will reflect our knowledge concerning the specific risk of hereditary pancreatitis patients to develop pancreatic cancer.

Hereditary Hearing Loss.

Science.gov (United States)

Tran, LenhAnh P.; Grundfast, Kenneth M.

1997-01-01

This article discusses inheritance patterns in hearing loss, epidemiology, clues to genetic causes, locating genes that cause hereditary disorders, genes related to hearing loss disorders in individuals with Usher syndrome, Waardenburg syndrome, Treacher-Collins syndrome, Branchio-oto-renal and Pendred syndromes, and the significance of finding…

Revisited diagnostics of hereditary epidermolysis bullosa

Directory of Open Access Journals (Sweden)

V. I. Albanova

2014-01-01

Full Text Available Hereditary epidermolysis bullosa is a big group of hereditary diseases with the main manifestations in the form of blisters on the skin and mucous coat after slight mechanical injuries. It is not always possible to diagnose this disease based on the clinical picture. The article discusses current laboratory diagnostics methods for hereditary epidermolysis bullosa including immunofluorescence antigen mapping (IFM, transmission electron microscopy (TEM and genetic analysis (molecular or DNA diagnostics as well as their advantages and disadvantages. TEM determines the micro splitting level and nature of ultrafine changes in the area of the dermoepidermal junction; at the same time, such tests need special expensive equipment. Substantial experience is also needed to analyze the resulting submicroscopic images. IFM determines whether expression of the affected protein related to the disease development is reduced or absent; however, invalid (false positive or false negative results can be obtained in patients with the reduced expression of the affected protein. Genetic analysis plays a key role for prenatal diagnostics. Therefore, to make an exact diagnosis of hereditary epidermolysis bullosa, it is expedient to apply IFM, TEM and genetic analysis. The need to set an exact diagnosis of the disease is related to the fact that the promising treatment methods being currently developed are aimed at treating patients with certain forms of the disease.

Microglassification™: a Novel Technique for Protein Dehydration

DEFF Research Database (Denmark)

Aniket; Gaul, David; Rickard, Deborah

2014-01-01

he dehydration of biologics is commonly employed to achieve solid-dose formulation and enhanced stability during long-term preservation. We have developed a novel process, MicroglassificationTM, which can rapidly and controllably dehydrate protein solutions into solid amorphous microspheres at room...... time and decreasing water activity. Image analysis at single particle and bulk scale showed the formation of solid BSA microspheres with a maximum protein concentration of 1147 ± 32 mg/mL. The native BSA samples were dehydrated to approximately 450 waters per BSA, which is well below monolayer coverage...... of 1282 waters per BSA. The secondary structure of MicroglassifiedTM BSA reverted to native-like conformation upon rehydration with only minor irreversible aggregation (2.7%). Results of the study establish the efficacy of the MicroglassificationTM for the successful dehydration of biologics....

Evidence-based management of epistaxis in hereditary haemorrhagic telangiectasia.

Science.gov (United States)

Syed, I; Sunkaraneni, V S

2015-05-01

There are currently no guidelines in the UK for the specific management of hereditary haemorrhagic telangiectasia related epistaxis. The authors aimed to review the literature and provide an algorithm for the management of hereditary haemorrhagic telangiectasia related epistaxis. The Medline and Embase databases were interrogated on 15 November 2013 using the search items 'hereditary haemorrhagic telangiectasia' (title), 'epistaxis' (title) and 'treatment' (title and abstract), and limiting the search to articles published in English. A total of 46 publications were identified, comprising 1 systematic review, 2 randomised, controlled trials, 27 case series, 9 case reports, 4 questionnaire studies and 3 in vitro studies. There is a lack of high-level evidence for the use of many of the available treatments for the specific management of epistaxis in hereditary haemorrhagic telangiectasia. Current management should be based on a multidisciplinary team approach involving both a hereditary haemorrhagic telangiectasia physician and an ENT surgeon, especially when systemic therapy is being considered. The suggested treatment algorithm considers that the severity of epistaxis merits intervention at different levels of the treatment ladder. The patient should be assessed using a reproducible validated assessment tool, for example an epistaxis severity score, to guide treatment. More research is required, particularly in the investigation of topical agents targeting the development and fragility of telangiectasiae in hereditary haemorrhagic telangiectasia.

Dehydration of core/shell fruits

OpenAIRE

Liu, Y.; Yang, Xiaosong; Cao, Y.; Wang, Z.; Chen, B.; Zhang, Jian J.; Zhang, H.

2015-01-01

Dehydrated core/shell fruits, such as jujubes, raisins and plums, show very complex buckles and wrinkles on their exocarp. It is a challenging task to model such complicated patterns and their evolution in a virtual environment even for professional animators. This paper presents a unified physically-based approach to simulate the morphological transformation for the core/shell fruits in the dehydration process. A finite element method (FEM), which is based on the multiplicative decomposition...

Observations on saliva osmolality during progressive dehydration and partial rehydration.

Science.gov (United States)

Taylor, Nigel A S; van den Heuvel, Anne M J; Kerry, Pete; McGhee, Sheena; Peoples, Gregory E; Brown, Marc A; Patterson, Mark J

2012-09-01

A need exists to identify dehydrated individuals under stressful settings beyond the laboratory. A predictive index based on changes in saliva osmolality has been proposed, and its efficacy and sensitivity was appraised across mass (water) losses from 1 to 7%. Twelve euhydrated males [serum osmolality: 286.1 mOsm kg(-1) H(2)O (SD 4.3)] completed three exercise- and heat-induced dehydration trials (35.6°C, 56% relative humidity): 7% dehydration (6.15 h), 3% dehydration (with 60% fluid replacement: 2.37 h), repeat 7% dehydration (5.27 h). Expectorated saliva osmolality, measured at baseline and at each 1% mass change, was used to predict instantaneous hydration state relative to mass losses of 3 and 6%. Saliva osmolality increased linearly with dehydration, although its basal osmolality and its rate of change varied among and within subjects across trials. Receiver operating characteristic curves indicated a good predictive power for saliva osmolality when used with two, single-threshold cutoffs to differentiate between hydrated and dehydrated individuals (area under curve: 3% cutoff = 0.868, 6% cutoff = 0.831). However, when analysed using a double-threshold detection technique (3 and 6%), as might be used in a field-based monitor, <50% of the osmolality data could correctly identify individuals who exceeded 3% dehydration. Indeed, within the 3-6% dehydration range, its sensitivity was 64%, while beyond 6% dehydration, this fell to 42%. Therefore, while expectorated saliva osmolality tracked mass losses within individuals, its large intra- and inter-individual variability limited its predictive power and sensitivity, rendering its utility questionable within a universal dehydration monitor.

Dehydration in children with diabetic ketoacidosis: a prospective study.

Science.gov (United States)

Sottosanti, Maria; Morrison, Gavin C; Singh, Ram N; Sharma, Ajay P; Fraser, Douglas D; Alawi, Khalid; Seabrook, Jamie A; Kornecki, Alik

2012-02-01

To investigate the association between the degree of patient dehydration on presentation with diabetic ketoacidosis (DKA) and clinical and laboratory parameters obtained on admission. Prospective descriptive study. A tertiary care children's hospital. Thirty-nine paediatric patients (1 month-16 years) presenting with 42 episodes of DKA. Clinical and biochemical variables were collected on admission. Dehydration was calculated by measuring acute changes in body weight during the period of illness. All patients were treated according to a previously established protocol. Magnitude of dehydration, defined as % loss of body weight (LBW), was determined by the difference in body weight obtained at presentation and at discharge. The relationship between the magnitude of dehydration and the clinical assessment and biochemical parameters was examined. The median (25th-75th centiles) magnitude of dehydration at presentation was 5.7% (3.8-8.3%) (mean ± SD 6.8 ± 5%). Neither the initial clinical assessment nor the comprehensive biochemical profile at admission correlated with the magnitude of dehydration. Despite considerable variation in the degree of dehydration and biochemical disequilibrium, all patients recovered from DKA within 24 h with a standardised therapeutic approach. Furthermore, the rapidity of patient recovery did not correlate with the magnitude of dehydration on presentation or the amount of fluid administered (median (25th-75th centiles) 48.8 ml/kg (38.5-60.3)) in the first 12 h. The magnitude of dehydration in DKA is not reflected by either clinical or biochemical parameters. These findings need confirmation in larger studies.

Hereditary angioedema: a bradykinin-mediated swelling disorder.

Science.gov (United States)

Björkqvist, Jenny; Sala-Cunill, Anna; Renné, Thomas

2013-03-01

Edema is tissue swelling and is a common symptom in a variety of diseases. Edema form due to accumulation of fluids, either through reduced drainage or increased vascular permeability. There are multiple vascular signalling pathways that regulate vessel permeability. An important mediator that increases vascular leak is the peptide hormone bradykinin, which is the principal agent in the swelling disorder hereditary angioedema. The disease is autosomal dominant inherited and presents clinically with recurrent episodes of acute swelling that can be life-threatening involving the skin, the oropharyngeal, laryngeal, and gastrointestinal mucosa. Three different types of hereditary angiodema exist in patients. The review summarises current knowledge on the pathophysiology of hereditary angiodema and focuses on recent experimental and pharmacological findings that have led to a better understanding and new treatments for the disease.

[Paediatric retinal detachment and hereditary vitreoretinal disorders].

Science.gov (United States)

Meier, P

2013-09-01

The number of retinal detachments in children is very low in comparison to the number in adults. One predisposing factor for development of paediatric retinal detachment is suffering from hereditary vitreoretinal degeneration (e.g., Stickler syndrome, Wagner syndrome, Kniest dysplasia, familial exudative vitreoretinopathy, congenital X-linked retinoschisis, Knobloch syndrome, incontinentia pigmenti, Norrie disease). Hereditary vitreoretinopathies are characterised by an abnormal-appearing vitreous gel with associated retinal changes. In most of these eyes further ocular abnormalities can be diagnosed. A group of hereditary disorders is associated with characteristic systemic abnormalities. Allied conditions should be considered in the clinical diagnosis. Vitreoretinopathies are the most common cause of inherited retinal detachment. In most eyes primary vitrectomy is necessary, and disease-specific surgical treatment is discussed. Georg Thieme Verlag KG Stuttgart · New York.

Genetics Home Reference: hereditary leiomyomatosis and renal cell cancer

Science.gov (United States)

... Home Health Conditions HLRCC Hereditary leiomyomatosis and renal cell cancer Printable PDF Open All Close All Enable Javascript ... expand/collapse boxes. Description Hereditary leiomyomatosis and renal cell cancer ( HLRCC ) is a disorder in which affected individuals ...

Organization and Running of the First Comprehensive Hereditary Cancer Clinic in India

Directory of Open Access Journals (Sweden)

Rajkumar T

2005-11-01

Full Text Available Abstract Hereditary cancers are thought to account for around 5% of cancers, particularly breast/ovarian and colorectal cancers. In India there is a paucity of data on hereditary cancers and the mutations in some of the common genes linked to hereditary cancers, such as BRCA1, BRCA2, hMSH2 and hMLH1. The country's first comprehensive hereditary cancer clinic was established in February 2002. The article describes the organization and running of the Clinic. It also discusses some of the social issues relevant to the given population in running the Hereditary Cancer Clinic.

Freeze dehydration of milk using microwave energy

International Nuclear Information System (INIS)

Souda, K.B.; Akyel, C.; Bilgen, E.

1989-01-01

This paper presents the results of experimental studies on heat and mass transfer during a microwave freeze dehydration process. An experimental system and procedure was developed to freeze dry milk. A 2500-W microwave system with an appropriate wave guide was set up and instrumented, and a procedure was experimentally developed to obtain milk powder first by freezing milk and then dehydrating it at low pressure using microwave energy. An unsteady-state analysis was used to derive a one-dimensional mathematical model of the freeze dehydration process in a microwave electromagnetic field

Computational Dehydration of Crystalline Hydrates Using Molecular Dynamics Simulations

DEFF Research Database (Denmark)

Larsen, Anders Støttrup; Rantanen, Jukka; Johansson, Kristoffer E

2017-01-01

Molecular dynamics (MD) simulations have evolved to an increasingly reliable and accessible technique and are today implemented in many areas of biomedical sciences. We present a generally applicable method to study dehydration of hydrates based on MD simulations and apply this approach...... to the dehydration of ampicillin trihydrate. The crystallographic unit cell of the trihydrate is used to construct the simulation cell containing 216 ampicillin and 648 water molecules. This system is dehydrated by removing water molecules during a 2200 ps simulation, and depending on the computational dehydration....... The structural changes could be followed in real time, and in addition, an intermediate amorphous phase was identified. The computationally identified dehydrated structure (anhydrate) was slightly different from the experimentally known anhydrate structure suggesting that the simulated computational structure...

A study on dehydration of rare earth chloride hydrate

International Nuclear Information System (INIS)

Cho, Yong Zun; Eun, Hee Chul; Son, Sung Mo; Lee, Tae Kyo; Hwang, Taek Sung

2012-01-01

The dehydration schemes of rare earth (La, Ce, Nd, Pr, Sm. Eu, Gd, Y) chloride hydrates was investigated by using a dehydration apparatus. To prevent the formation of the rare earth oxychlorides, the operation temperature was changed step by step (80→150→230 degree C) based on the TGA (thermo-gravimetric analysis) results of the rare earth chloride hydrates. A vacuum pump and preheated Ar gas were used to effectively remove the evaporated moisture and maintain an inert condition in the dehydration apparatus. The dehydration temperature of the rare earth chloride hydrate was increased when the atomic number of the rare earth nuclide was increased. The content of the moisture in the rare earth chloride hydrate was decreased below 10% in the dehydration apparatus.

Therapeutic Strategies for Hereditary Kidney Cancer.

Science.gov (United States)

Sidana, Abhinav; Srinivasan, Ramaprasad

2016-08-01

The study of hereditary forms of kidney cancer has vastly increased our understanding of metabolic and genetic pathways involved in the development of both inherited and sporadic kidney cancers. The recognition that diverse molecular events drive different forms of kidney cancers has led to the preclinical and clinical development of specific pathway-directed strategies tailored to treat distinct subgroups of kidney cancer. Here, we describe the molecular mechanisms underlying the pathogenesis of several different types of hereditary renal cancers, review their clinical characteristics, and summarize the treatment strategies for the management of these cancers.

Retention of nutrients in green leafy vegetables on dehydration.

Science.gov (United States)

Gupta, Sheetal; Gowri, B S; Lakshmi, A Jyothi; Prakash, Jamuna

2013-10-01

The objective of the study was to investigate the influence of dehydration on nutrient composition of Amaranthus gangeticus, Chenopodium album, Centella asiatica, Amaranthus tricolor and Trigonella foenum graecum. The green leafy vegetables (GLV) were steam blanched for 5 min after pretreatment and dried in an oven at 60 °C for 10-12 h. The fresh and dehydrated samples were analyzed for selected proximate constituents, vitamins, minerals, antinutrients and dialyzable minerals. Dehydration seems to have little effect on the proximate, mineral and antinutrient content of the GLV. Among the vitamins, retention of ascorbic acid was 1-14%, thiamine 22-71%, total carotene 49-73% and β-carotene 20-69% respectively, of their initial content. Dialyzable iron and calcium in the fresh vegetables ranged between 0.21-3.5 mg and 15.36-81.33 mg/100 g respectively, which reduced to 0.05-0.53 mg and 6.94-58.15 mg/100 g on dehydration. Dehydration seems to be the simplest convenient technology for preserving these sources of micronutrients, especially when they are abundantly available. Irrespective of the losses of vitamins that take place during dehydration, dehydrated GLV are a concentrated natural source of micronutrients and they can be used in product formulations. Value addition of traditional products with dehydrated GLV can be advocated as a feasible food-based approach to combat micronutrient malnutrition.

Attitude towards pre-implantation genetic diagnosis for hereditary cancer

NARCIS (Netherlands)

Lammens, Chantal; Bleiker, Eveline; Aaronson, Neil; Vriends, Annette; Ausems, Margreet; Jansweijer, Maaike; Wagner, Anja; Sijmons, Rolf; van den Ouweland, Ans; van der Luijt, Rob; Spruijt, Liesbeth; Gómez García, Encarna; Ruijs, Mariëlle; Verhoef, Senno

2009-01-01

The use of pre-implantation genetic diagnosis (PGD) for hereditary cancer is subject to on-going debate, particularly among professionals. This study evaluates the attitude towards PGD and attitude-associated characteristics of those concerned: family members with a hereditary cancer predisposition.

Squamous cell carcinoma complicating an hereditary epidermo-lysis bullosa

International Nuclear Information System (INIS)

Mseddi, M.; Turki, H.; Marrekchi, S.; Abdelmaksoud, W.; Masmoudi, A.; Bouassida, S.; Zahaf, A.

2004-01-01

The dystrophic form of hereditary epidermo-lysis bullosa is associated with an increased frequency of squamous cell carcinoma. We report a new case. An 18-year-old patient, carrying a Hallopeau Siemens hereditary epidermo-lysis bullosa, presented a subcutaneous nodular lesion, for 1 year that ulcerated and budded with inguinal lymphadenopathy. The histological study ted to the conclusion of a well differentiated squamous cell carcinoma. The patient was treated surgically. Tumor and metastatic lymph nodes were excised. A radiotherapy was decided but the postoperative course was fatal due to an infection and to a deterioration of her general condition. Squamous cell carcinoma frequently occurs on the cicatricial lesion of hereditary epidermo-lysis bullosa and usually affects males with recessive hereditary epidermo-lysis bullosa. Metastases are frequent, precocious and multiple. The treatment may be surgical. The particularities of our observation are the young age of patient and the localization. (author)

Hereditary Lymphedema of the Leg – A Case Report

Directory of Open Access Journals (Sweden)

Birgit Heinig

2017-07-01

Full Text Available Primary of hereditary lymphedema is a rare but progressive disease. It is yet not curable. We present a 48-year-old male patient with hereditary lymphedema of his left leg, that was realised by minor trauma (able twist when he was seven years old. He had never been treated for lymphedema but experienced multiple erysipelas during his life. After diagnostic procedures to exclude other causes of leg swelling, the diagnosis of hereditary lymphedema of the leg, stage III was confirmed. We initialized complex decongestive therapy. During two weeks of intensive treatment, the circumference of the left leg could be reduced by 10 cm. This case illustrates the "natural course" hereditary lymphedema. But it raises the hope that even after decades of ignorance, the patients benefits from complex decongestive treatment. Therapeutic nihilism is unnecessary and poses lymphedema patients to risks of infection and secondary malignancies like Stewart-Trewes syndrome.

The prevalence of depression in hereditary spastic paraplegia.

Science.gov (United States)

Vahter, L; Braschinsky, M; Haldre, S; Gross-Paju, K

2009-09-01

To evaluate the prevalence of depression and sensitivity and specificity of the single-item interview 'Are you depressed?' for people with hereditary spastic paraplegia in Estonia. Single-item interview 'Are you depressed?' was used as a screening question for depression; all participants then completed the Beck Depression Inventory. People with hereditary spastic paraplegia identified from the epidemiological database who agreed to participate in the study. Beck Depression Inventory, clinical interview. The epidemiological database consisted of 59 patients with clinically confirmed diagnosis of hereditary spastic paraplegia. Forty-eight of these consented to participate in the study. The Beck Depression Inventory score was higher than cut-off point in 58% (28/48) and lower in 42% (20/48). Of the study group, 44% (21/48) had mild, 13% (6/48) moderate and one person revealed severe depression. There was a statistically significant correlation between Beck Depression Inventory score and level of mobility; no other significant correlations with other measures were detected. Of the participants, 54% (26/48) had subjective complaints about depression and answered 'Yes' to the single-item interview 'Are you depressed?'. The sensitivity of the one-item interview in the hereditary spastic paraplegia group was 75% and specificity 75%. Our results show that mild depression is prevalent among people with hereditary spastic paraplegia. Although the single question may be helpful, it cannot be relied upon entirely when assessing a person for depression.

Diagnosing dehydration? Blend evidence with clinical observations.

Science.gov (United States)

Armstrong, Lawrence E; Kavouras, Stavros A; Walsh, Neil P; Roberts, William O

2016-11-01

The purpose of the review is to provide recommendations to improve clinical decision-making based on the strengths and weaknesses of commonly used hydration biomarkers and clinical assessment methods. There is widespread consensus regarding treatment, but not the diagnosis of dehydration. Even though it is generally accepted that a proper clinical diagnosis of dehydration can only be made biochemically rather than relying upon clinical signs and symptoms, no gold standard biochemical hydration index exists. Other than clinical biomarkers in blood (i.e., osmolality and blood urea nitrogen/creatinine) and in urine (i.e., osmolality and specific gravity), blood pressure assessment and clinical symptoms in the eye (i.e., tear production and palpitating pressure) and the mouth (i.e., thirst and mucous wetness) can provide important information for diagnosing dehydration. We conclude that clinical observations based on a combination of history, physical examination, laboratory values, and clinician experience remain the best approach to the diagnosis of dehydration.

Water-activity of dehydrated guava slices sweeteners

International Nuclear Information System (INIS)

Ayub, M.; Zeb, A.; Ullah, J.

2005-01-01

A study was carried out to investigate the individual and combined effect of caloric sweeteners (sucrose, glucose and fructose) and non-caloric sweeteners (saccharine, cyclamate and aspartame) along with antioxidants (citric acid and ascorbic acid) and chemical preservatives (potassium metabisulphite and potassium sorbate) on the water-activity (a/sub w/) of dehydrated guava slices. Different dilutions of caloric sweeteners (20, 30, 40 and 50 degree brix (bx) and non-caloric sweeteners (equivalent to sucrose sweetness) were used. Guava slices were osmotically dehydrated in these solutions and then dehydrated initially at 0 and then at 60 degree C to final moisture-content of 20-25%. Guava slices prepared with sucrose: glucose 7:3 potassium metabisulphite, ascorbic acid and citric acid produced best quality products, which have minimum (a/sub w/) and best overall sensory characteristics. The analysis showed that treatments and their various concentrations had a significant effect (p=0.05) on (a/sub w/) of dehydrated guava slices. (author)

Comparison of gas dehydration methods based on energy ...

African Journals Online (AJOL)

Comparison of gas dehydration methods based on energy consumption. ... PROMOTING ACCESS TO AFRICAN RESEARCH ... This study compares three conventional methods of natural gas (Associated Natural Gas) dehydration to carry out ...

Hereditary and non-hereditary microangiopathies in the young. An up-date.

Science.gov (United States)

Ringelstein, E Bernd; Kleffner, Ilka; Dittrich, Ralf; Kuhlenbäumer, Gregor; Ritter, Martin A

2010-12-15

In recent years, a considerable number of new sporadic or hereditary small artery diseases of the brain have been detected which preferably occur in younger age, below 45 years. Cerebral microangiopathies constitute an appreciable portion of all strokes. In middle aged patients, hereditary cerebral small vessel diseases have to be separated from sporadic degenerative cerebral microangiopathy which is mainly due to a high vascular risk load. Features of the following disorders and details how to differentiate them, are reviewed here, namely CADASIL, MELAS, AD-RVLC, HEMID, CARASIL, PADMAL, FABRY, COL4A1-related cerebral small vessel diseases and a Portuguese type of autosomal dominant cerebral small vessel disease (SVDB). The symptomatic overlap of the cerebral microangiopathies include also other distinctive non-hereditary diseases like posterior (reversible) encephalopathy and Susac's syndrome which are also described. Some of the microangiopathies described here are not only seen in the young but also in the elderly. The precise diagnosis has direct therapeutic implications in several of these entities. Cerebral microangiopathies cause recurring strokes and diffuse white matter lesions leading to a broad spectrum of gait disturbances and in most of these disorders cognitive impairment or even vascular dementia in the long term. Often, they also involve the eye, the inner ear or the kidney. Several typical imaging findings from illustrative cases are presented. The order in which these diseases are presented here is not dictated by an inner logic principle, because a genetically or pathophysiologically based classification system of all these entities does not exist yet. Some entities are well established and not unusual, whereas others have only been described in a few cases in total. Copyright © 2010 Elsevier B.V. All rights reserved.

Effects of Dehydration on Brain Functioning: A Life-Span Perspective.

Science.gov (United States)

Pross, Nathalie

2017-01-01

In the last 10 years, there has been an increase in the publication of literature dealing with the effects of mild dehydration on cognition in healthy adults. Fewer studies, leading to less consistent data, involved other age groups. In healthy young adults refraining from drinking or participating in dehydration protocols, it was found that mild dehydration had no impact on performance, whereas the mood was widely impaired. Several studies have also been conducted in young children either as observational studies or as interventional studies. Nevertheless, methodological differences in (de)hydration monitoring, in cognitive assessments, and in the age/brain maturation of study participants, often resulted in contradictory findings regarding the cognitive functions impacted by (de)hydration. Although not consistent, these data showed that not only mood but also performance tend to be impaired by dehydration in children. Even if older adults are likely to be more vulnerable to dehydration than younger adults, very few studies have been conducted in this regard in this population. The results show that, like it is in children, cognition tends to be impaired when the elderly are dehydrated. Taken together, these studies suggest that dehydration has greater detrimental effects in vulnerable populations. Recent imaging data suggest that the brain of children and elderly adults may have fewer resources to manage the effects of dehydration. Consequently, cognitive tasks may be more demanding for younger and older brains and performance more likely to be impaired in these populations, in comparison to young healthy subjects who have greater and more efficient resources. © 2017 The Author(s) Published by S. Karger AG, Basel.

Using Dehydrated Vegetables in Some Brown Bread Types

Directory of Open Access Journals (Sweden)

Simona Man

2013-11-01

Full Text Available Normal 0 false false false MicrosoftInternetExplorer4 Expanding the range of bakery products in terms of producing supplemented or dietetic products has been an increasingly important trend in contemporary baking. Bakery products as basic and popular food, could be used in the prevention of nutritive deficiencies of many important nutrients, by supplementing the products with biologically valuable ingredients. Such ingredients are dehydrated vegetables in the form of powder. For establishing the bread quality, a special importance shows it’s chemical composition, because the substances that enter in it’s constitution serve to obtaining the energy necessary to the human body. Beside the chemical composition, the bread quality and alimentary use, respectively, depends a large measure on a series of signs: flavor and taste, external appearance, crumb porosity and texture, breads’ volume. This paper belongs to a more complex study, which aims are obtaining some bread assortments with high nutritional value, and improving their sensorial and rheological features, by adding dehydrated vegetables at different levels 4% potato flakes, 2% dehydrated onion, 0.5% dehydrated garlic and 2% dehydrated leek.

Knowledge regarding basic concepts of hereditary cancers, and the ...

African Journals Online (AJOL)

Background. In families with hereditary cancer, at-risk individuals can benefit from genetic counselling and testing. General practitioners (GPs) are ideally placed to identify such families and refer them appropriately. Objective. To assess the practices, knowledge and attitudes of GPs regarding common hereditary cancers.

Effects of Dehydration on Fish Muscles at Chilled Temperature

Science.gov (United States)

Miki, Hidemasa; Seto, Fuminori; Nishimoto, Motomi; Nishimoto, Junichi

Recently,new method of removing water from fish fillet at low temperature using dehydration sheet have been reported. The present study is concerned with the factors to affect the quality during dehydration of horse mackerel muscle at low temperature. The rate of dehydration at -3 °C was about two times faster than that at 0 °C. The rate of denaturation of fish muscle protein was kept less than about 10 % (ATPase activity) of the undenaturated initial values after removing free water content. Present results suggest the practical possibility of the dehydration at -3 °C for keeping quality of fish flesh.

Uric acid and dehydration in children with gastroenteritis.

Science.gov (United States)

Kuge, Rie; Morikawa, Yoshihiko; Hasegawa, Yukihiro

2017-11-01

Clinical assessment of dehydration in children with acute gastroenteritis (AGE) is crucial for treatment. The gold standard for assessment is acute weight loss. Dehydration severity, as determined by weight loss, significantly correlates with serum urea nitrogen (UN) in children with AGE. The disadvantage of using serum UN as a marker of dehydration severity, however, is that the level can be affected by nutrition. Serum uric acid (UA) theoretically could serve as an alternative in this regard, but there have been no reports on the direct relationship between serum UA and weight loss. The aim of this study was therefore to assess the relationship between serum UA and weight loss in patients with AGE. This was a prospective observational study of children 1-71 months of age admitted to Tokyo Metropolitan Children's Medical Center with AGE between 1 December 2010 and 31 March 2015. Patients with symptoms of AGE ≥ 8 days, chronic disease, or incomplete data were excluded from the study. Pre-post-rehydration weight change was used as an alternative, gold standard marker for the assessment of dehydration severity. The study participants consisted of 97 children. Serum UA correlated significantly with weight change, and increased significantly along with dehydration severity. Serum UA can be considered as an adjunct to the clinical assessment of dehydration in children with AGE. © 2017 Japan Pediatric Society.

Whole transcriptome organisation in the dehydrated supraoptic nucleus

Directory of Open Access Journals (Sweden)

C.C.T. Hindmarch

2013-12-01

Full Text Available The supraoptic nucleus (SON is part of the central osmotic circuitry that synthesises the hormone vasopressin (Avp and transports it to terminals in the posterior lobe of the pituitary. Following osmotic stress such as dehydration, this tissue undergoes morphological, electrical and transcriptional changes to facilitate the appropriate regulation and release of Avp into the circulation where it conserves water at the level of the kidney. Here, the organisation of the whole transcriptome following dehydration is modelled to fit Zipf's law, a natural power law that holds true for all natural languages, that states if the frequency of word usage is plotted against its rank, then the log linear regression of this is -1. We have applied this model to our previously published euhydrated and dehydrated SON data to observe this trend and how it changes following dehydration. In accordance with other studies, our whole transcriptome data fit well with this model in the euhydrated SON microarrays, but interestingly, fit better in the dehydrated arrays. This trend was observed in a subset of differentially regulated genes and also following network reconstruction using a third-party database that mines public data. We make use of language as a metaphor that helps us philosophise about the role of the whole transcriptome in providing a suitable environment for the delivery of Avp following a survival threat like dehydration.

Fruits and vegetables dehydration

International Nuclear Information System (INIS)

De Ita, A; Flores, G; Franco, F

2015-01-01

Dehydration diagrams were determined by means of Differential Thermal Analysis, DTA, and Thermo Gravimetric Analysis, TGA, curves of several simultaneous fruits and vegetables, all under the same conditions. The greater mass loss is associated with water containing in the structure of the investigated materials at low temperature. In poblano chile water is lost in a single step. The banana shows a very sharply two stages, while jicama can be observed although with a little difficulty three stages. The major mass loss occurs in the poblano chile and the lower in banana. The velocity and temperature of dehydration vary within a small range for most materials investigated, except for banana and cactus how are very different

Fruits and vegetables dehydration

Science.gov (United States)

de Ita, A.; Flores, G.; Franco, F.

2015-01-01

Dehydration diagrams were determined by means of Differential Thermal Analysis, DTA, and Thermo Gravimetric Analysis, TGA, curves of several simultaneous fruits and vegetables, all under the same conditions. The greater mass loss is associated with water containing in the structure of the investigated materials at low temperature. In poblano chile water is lost in a single step. The banana shows a very sharply two stages, while jicama can be observed although with a little difficulty three stages. The major mass loss occurs in the poblano chile and the lower in banana. The velocity and temperature of dehydration vary within a small range for most materials investigated, except for banana and cactus how are very different.

Hereditary periodic fever syndromes

NARCIS (Netherlands)

McDermott, MF; Frenkel, J

Hereditary periodic fever syndromes are defined by recurrent attacks of generalised inflammation for which no infectious or auto-immune cause can be identified. For most of these disorders, the molecular basis has recently been elucidated. This has opened the prospect of novel therapeutic

Immobilisation increases yeast cells' resistance to dehydration-rehydration treatment.

Science.gov (United States)

Borovikova, Diana; Rozenfelde, Linda; Pavlovska, Ilona; Rapoport, Alexander

2014-08-20

This study was performed with the goal of revealing if the dehydration procedure used in our new immobilisation method noticeably decreases the viability of yeast cells in immobilised preparations. Various yeasts were used in this research: Saccharomyces cerevisiae cells that were rather sensitive to dehydration and had been aerobically grown in an ethanol-containing medium, a recombinant strain of S. cerevisiae grown in aerobic conditions which were completely non-resistant to dehydration and an anaerobically grown bakers' yeast strain S. cerevisiae, as well as a fairly resistant Pichia pastoris strain. Experiments performed showed that immobilisation of all these strains essentially increased their resistance to a dehydration-rehydration treatment. The increase of cells' viability (compared with control cells dehydrated in similar conditions) was from 30 to 60%. It is concluded that a new immobilisation method, which includes a dehydration stage, does not lead to an essential loss of yeast cell viability. Correspondingly, there is no risk of losing the biotechnological activities of immobilised preparations. The possibility of producing dry, active yeast preparations is shown, for those strains that are very sensitive to dehydration and which can be used in biotechnology in an immobilised form. Finally, the immobilisation approach can be used for the development of efficient methods for the storage of recombinant yeast strains. Copyright © 2014 Elsevier B.V. All rights reserved.

Diagnosis and management of dehydration in children.

Science.gov (United States)

Canavan, Amy; Arant, Billy S

2009-10-01

The most useful individual signs for identifying dehydration in children are prolonged capillary refill time, abnormal skin turgor, and abnormal respiratory pattern. However, clinical dehydration scales based on a combination of physical examination findings are better predictors than individual signs. Oral rehydration therapy is the preferred treatment of mild to moderate dehydration caused by diarrhea in children. Appropriate oral rehydration therapy is as effective as intravenous fluid in managing fluid and electrolyte losses and has many advantages. Goals of oral rehydration therapy are restoration of circulating blood volume, restoration of interstitial fluid volume, and maintenance of rehydration. When rehydration is achieved, a normal age-appropriate diet should be initiated.

Dehydration Comes on Fast and Can Be Fatal

Science.gov (United States)

... can be fatal Dehydration comes on fast and can be fatal During the hot summer months,the ... and keeping hydrated. “Dehydration is very dangerous. It can lead to an emergency visit, and it can ...

Dehydration kinetics of talc at 1 bar

Science.gov (United States)

Ganguly, J.; Bose, K.

1991-01-01

Experimental results on the dehydration kinetics of talc, which is likely to be a major potential resource for water and hydrogen in carbonaceous chondrites, is presented. The rate of dehydration of an essentially pure Mg-end member natural talc, (Mg(.99)Fe(.01))3Si4O10(OH)2, was studied by measuring in situ weight change under isothermal condition at 1 bar as a function of time in the temperature range 775 to 985 C. The grain size of the starting material was 0.7 to 1 micron. It was found that the data up to 50 to 60 percent dehydration can be fitted by an equation of the form alpha = exp(-Kt(exp n)), where alpha is the weight fraction of talc remaining, K is a rate constant and n is a numerical constant for a given temperature. For any set of isothermal data, there is a major change in the value of n for larger dehydration. For up to approximately 50 percent dehydration, all rate constants can be described by an Arrheniun relation with an activation energy of 432 (+/- 30) kJ/mol; n has a nearly constant value of 0.54 between 775 and 875 C, but increases almost linearly according to n = -10.77 + 0.012T C at T greater than or equal to 875 C.

Hereditary familial vestibular degenerative diseases.

NARCIS (Netherlands)

Sun, J.; Alphen, A.M. van; Wagenaar, M.; Huygen, P.L.M.; Hoogenraad, C.C.; Hasson, T.; Koekkoek, S.K.; Bohne, B.A.; Zeeuw, C.I. de

2001-01-01

Identification of genes involved in hereditary vestibular disease is growing at a remarkable pace. Mutant mouse technology can be an important tool for understanding the biological mechanism of human vestibular diseases.

GOHAL!: sustainable membranes for alcohol dehydration

DEFF Research Database (Denmark)

Boffa, Vittorio

2017-01-01

the pristine graphene oxide membrane. The enhanced water permeability along with the good water/ethanol selectivity makes the GO-HAL membranes promising devices for alcohol dehydration technologies. This study provides a new basis for the rational design of the future generation of GO-based membranes......Treat, H2020-MSCA-RISE-2014 (n. 64555). [1] V. Boffa et. al., Carbon-based building blocks for alcohol dehydration membranes with disorder-enhanced water permeability, Carbon, under review....

Disease expression in women with hereditary angioedema

DEFF Research Database (Denmark)

Bouillet, Laurence; Longhurst, Hilary; Boccon-Gibod, Isabelle

2008-01-01

OBJECTIVE: Fluctuations in sex hormones can trigger angioedema attacks in women with hereditary angioedema. Combined oral contraceptive therapies, as well as pregnancy, can induce severe attacks. The course of angioedema may be very variable in different women. STUDY DESIGN: Within the PREHAEAT p......-sensitive phenotype for some patients. CONCLUSION: The course of angioedema in women with C1 inhibitor deficiency is affected by physiologic hormonal changes; consequently, physicians should take these into account when advising on management.......OBJECTIVE: Fluctuations in sex hormones can trigger angioedema attacks in women with hereditary angioedema. Combined oral contraceptive therapies, as well as pregnancy, can induce severe attacks. The course of angioedema may be very variable in different women. STUDY DESIGN: Within the PREHAEAT...... project launched by the European Union, data on 150 postpubertal women with hereditary angioedema were collected in 8 countries, using a patient-based questionnaire. RESULTS: Puberty worsened the disease for 62%. Combined oral contraceptives worsened the disease for 79%, whereas progestogen-only pills...

Gastric tumours in hereditary cancer syndromes: clinical features, molecular biology and strategies for prevention.

Science.gov (United States)

Sereno, María; Aguayo, Cristina; Guillén Ponce, Carmen; Gómez-Raposo, César; Zambrana, Francisco; Gómez-López, Miriam; Casado, Enrique

2011-09-01

Gastric cancer is the major cause of cancer-related deaths worldwide. The majority of them are classified as sporadic, whereas the remaining 10% exhibit familial clustering. Hereditary diffuse gastric cancer (HDGC) syndrome is the most important condition that leads to hereditary gastric cancer. However, other hereditary cancer syndromes, such as hereditary non-polyposis colorectal cancer, familial adenomatous polyposis, Peutz-Jeghers syndrome, Li-Fraumeni syndrome and hereditary breast and ovarian cancer, entail a higher risk compared to the general population for developing this kind of neoplasia. In this review, we describe briefly the most important aspects related to clinical features, molecular biology and strategies for prevention in hereditary gastric associated to different cancer syndromes.

Rehydration Capacities and Rates for Various Porcine Tissues after Dehydration

Science.gov (United States)

Meyer, Jacob P.; McAvoy, Kieran E.; Jiang, Jack

2013-01-01

The biphasic effects of liquid on tissue biomechanics are well known in cartilage and vocal folds, yet not extensively in other tissue types. Past studies have shown that tissue dehydration significantly impacts biomechanical properties and that rehydration can restore these properties in certain tissue types. However, these studies failed to consider how temporal exposure to dehydrating or rehydrating agents may alter tissue rehydration capacity, as overexposure to dehydration may permanently prevent rehydration to the initial liquid volume. Select porcine tissues were dehydrated until they reached between 100% and 40% of their initial mass. Each sample was allowed to rehydrate for 5 hours in a 0.9% saline solution, and the percent change between the initial and rehydrated mass values was calculated. Spearman correlation tests indicated a greater loss in mass despite rehydration when tissues were previously exposed to greater levels of dehydration. Additionally, Pearson correlation tests indicated the total liquid mass of samples after complete rehydration decreased when previously exposed to higher levels of dehydration. Rehydration rates were found by dehydrating tissues to 40% of their initial mass followed by rehydration in a 0.9% saline solution for 60 minutes, with mass measurements occurring in 15 minute intervals. All tissues rehydrated nonlinearly, most increasing significantly in mass up to 30 minutes after initial soaking. This study suggests the ability for tissues to rehydrate is dependent on the level of initial dehydration exposure. In vitro rehydration experiments therefore require controlled dosage and temporal exposure to dehydrating and rehydrating agents to avoid incomplete rehydration, and caution should be taken when combining different tissue types in models of hydration. PMID:24023753

Open-heart surgery using a centrifugal pump: a case of hereditary spherocytosis.

Science.gov (United States)

Matsuzaki, Yuichi; Tomioka, Hideyuki; Saso, Masaki; Azuma, Takashi; Saito, Satoshi; Aomi, Shigeyuki; Yamazaki, Kenji

2016-08-26

Hereditary spherocytosis is a genetic, frequently familial hemolytic blood disease characterized by varying degrees of hemolytic anemia, splenomegaly, and jaundice. There are few reports on adult open-heart surgery for patients with hereditary spherocytosis. We report a rare case of an adult open-heart surgery associated with hereditary spherocytosis. A 63-year-old man was admitted for congestive heart failure due to bicuspid aortic valve, aortic valve regurgitation, and sinus of subaortic aneurysm. The family history, the microscopic findings of the blood smear, and the characteristic osmotic fragility confirmed the diagnosis of hereditary spherocytosis. Furthermore, splenectomy had not been undertaken preoperatively. The patient underwent a successful operation by means of a centrifugal pump. Haptoglobin was used during the cardiopulmonary bypass, and a biological valve was selected to prevent hemolysis. No significant hemolysis occurred intraoperatively or postoperatively. There are no previous reports of patients with hereditary spherocytosis, and bicuspid aortic valve. We have successfully performed an adult open-heart surgery using a centrifugal pump in an adult patient suffering from hereditary spherocytosis and bicuspid aortic valve.

In situ dehydration of yugawaralite

DEFF Research Database (Denmark)

Artioli, G.; Ståhl, Kenny; Cruciani, G.

2001-01-01

The structural response of the natural zeolite yugawaralite (CaAl2Si6O16. 4H(2)O) upon thermally induced dehydration has been studied by Rietveld analysis of temperature-resolved powder diffraction data collected in situ in the temperature range 315-791 K using synchrotron radiation. The room...... progressively disappearing as the dehydration proceeds. The yugawaralite structure reacts to the release of water molecules with small changes in the Ca-O bond distances and minor distortions of the tetrahedral framework up to about 695 K. Above this temperature the Ca coordination falls below 7 (four framework...

Methods for Human Dehydration Measurement

Science.gov (United States)

Trenz, Florian; Weigel, Robert; Hagelauer, Amelie

2018-03-01

The aim of this article is to give a broad overview of current methods for the identification and quantification of the human dehydration level. Starting off from most common clinical setups, including vital parameters and general patients' appearance, more quantifiable results from chemical laboratory and electromagnetic measurement methods will be reviewed. Different analysis methods throughout the electromagnetic spectrum, ranging from direct current (DC) conductivity measurements up to neutron activation analysis (NAA), are discussed on the base of published results. Finally, promising technologies, which allow for an integration of a dehydration assessment system in a compact and portable way, will be spotted.

Hereditary lymphedema of the leg – A Case Report

NARCIS (Netherlands)

Heinig, Birgit; Lotti, T.; Tchernev, Georgi; Wollina, Uwe

2017-01-01

Primary of hereditary lymphedema is a rare but progressive disease. It is yet not curable. We present a 48-year-old male patient with hereditary lymphedema of his left leg, that was realised by minor trauma (able twist) when he was seven years old. He had never been treated for lymphedema but

Comparison of Gas Dehydration Methods based on Energy ...

African Journals Online (AJOL)

PROF HORSFALL

Natural Gas) dehydration to carry out the dehydration process and suitability of use on the basis of energy ... pipelines and distribution for burning by end users, natural gas must ... A good account of energy and material balance will create a ...

Diagnosis and management of hereditary hemochromatosis.

Science.gov (United States)

Salgia, Reena J; Brown, Kimberly

2015-02-01

Hereditary hemochromatosis is a rare genetic disorder that can have significant clinical consequences. Hemochromatosis is associated with iron overload, and can initially be recognized through laboratory testing for serum ferritin and transferrin saturation. Genetic testing for the HFE mutation can be performed in patients with elevated iron indices and a suspicion for hemochromatosis or liver disease. The main pathway resulting in iron overload is through altered hepcidin levels. Treatment of patients with the clinical phenotype of hereditary hemochromatosis is commonly through phlebotomy for removal of excess iron stores. This article highlights the current information and data regarding the diagnosis and management of hemochromatosis. Copyright © 2015 Elsevier Inc. All rights reserved.

40 CFR 63.765 - Glycol dehydration unit process vent standards.

Science.gov (United States)

2010-07-01

... 40 Protection of Environment 10 2010-07-01 2010-07-01 false Glycol dehydration unit process vent... Facilities § 63.765 Glycol dehydration unit process vent standards. (a) This section applies to each glycol dehydration unit subject to this subpart with an actual annual average natural gas flowrate equal to or...

40 CFR 63.1275 - Glycol dehydration unit process vent standards.

Science.gov (United States)

2010-07-01

... 40 Protection of Environment 11 2010-07-01 2010-07-01 true Glycol dehydration unit process vent... Facilities § 63.1275 Glycol dehydration unit process vent standards. (a) This section applies to each glycol dehydration unit subject to this subpart with an actual annual average natural gas flowrate equal to or...

New treatments of hereditary blindness

DEFF Research Database (Denmark)

Bertelsen, Mette; Rosenberg, Thomas; Larsen, Michael

2013-01-01

Ongoing clinical trials are targeting several previously intractable hereditary causes of blindness of congenital, childhood or early adulthood onset, mainly in the optic nerve and retina. The intended stage of initiation of the new therapeutic approaches ranges from neonatal life and a structura......Ongoing clinical trials are targeting several previously intractable hereditary causes of blindness of congenital, childhood or early adulthood onset, mainly in the optic nerve and retina. The intended stage of initiation of the new therapeutic approaches ranges from neonatal life...... and a structurally intact retinal tissue to adult life with a complete loss of photoreceptors. It must be assumed that some of the trials will succeed in producing new therapies and action must be taken to refine and accelerate diagnostics and to preserve therapeutic potential in blind people....

Upstream petroleum industry glycol dehydrator benzene emissions status report

International Nuclear Information System (INIS)

1999-07-01

The population of dehydrators referred to are located in the Western Sedimentary Basin in northeast British Columbia, Alberta and Saskatchewan, and includes units installed at wellsites, compressor stations, gas plants, central crude oil treating facilities, and reservoir or salt cavern gas storage facilities. Benzene emissions from the still column vent on glycol dehydrators occur as a result of glycol's strong affinity for aromatic hydrocarbons, including benzene. A study was carried out to: 1) develop a list of oil and gas companies operating in Canada, 2) develop an equipment and benzene emissions inventory of glycol dehydrators, 3) develop a database in Microsoft Access format to gather and maintain inventory and emission data, 4) evaluate and validate at least 10% of the reported data, 5) develop a list of companies that manufacture dehydrators and incinerators to determine how many new dehydrators were sold for use in Canada in 1998, and 6) prepare a report summarizing findings and recommendations. The companies included in the survey were the oil and gas companies identified by the Nickels' Oil and Gas Index and others provided by CAPP, CGA, and SEPAC. The project was carried out to gather glycol dehydrator equipment and still column vent benzene emissions information. 8 refs

Hereditary breast cancer: from molecular pathology to tailored therapies.

Science.gov (United States)

Tan, D S P; Marchiò, C; Reis-Filho, J S

2008-10-01

Hereditary breast cancer accounts for up to 5-10% of all breast carcinomas. Recent studies have demonstrated that mutations in two high-penetrance genes, namely BRCA1 and BRCA2, are responsible for about 16% of the familial risk of breast cancer. Even though subsequent studies have failed to find another high-penetrance breast cancer susceptibility gene, several genes that confer a moderate to low risk of breast cancer development have been identified; moreover, hereditary breast cancer can be part of multiple cancer syndromes. In this review we will focus on the hereditary breast carcinomas caused by mutations in BRCA1, BRCA2, Fanconi anaemia (FANC) genes, CHK2 and ATM tumour suppressor genes. We describe the hallmark histological features of these carcinomas compared with non-hereditary breast cancers and show how an accurate histopathological diagnosis may help improve the identification of patients to be screened for mutations. Finally, novel therapeutic approaches to treat patients with BRCA1 and BRCA2 germ line mutations, including cross-linking agents and PARP inhibitors, are discussed.

Radioactive waste slurry dehydrating and drum filling device

International Nuclear Information System (INIS)

Ichihashi, Toshio; Abe, Kazuaki; Hasegawa, Akira

1981-01-01

Purpose: To obtain a device for simultaneously filling and dehydrating radioactive waste in a waste can without the necessity of a special device for dehydration. Constitution: This device includes a radioactive waste storage tank, a pump for supplying the waste from the tank to a can, a drain tube having a filter at the lower end and installed displaceable in the axial direction of the can, and a drain pump. The slurry stored in the radioactive waste storage tank is supplied by the pump to the can, and the feedwater in the slurry is removed by another pump through a drain pipe having a filter which does not pass solid content from the can. Accordingly, as the slurry is filled in the can, the feedwater contained therein is removed. Consequently, it can simultaneously dehydrate and fill the dehydrated waste in the can. (Yoshihara, H.)

Dehydration in the Elderly: A Review Focused on Economic Burden.

Science.gov (United States)

Frangeskou, M; Lopez-Valcarcel, B; Serra-Majem, L

2015-06-01

Dehydration is the most common fluid and electrolyte problem among elderly patients. It is reported to be widely prevalent and costly to individuals and to the health care system. The purpose of this review is to summarize the literature on the economic burden of dehydration in the elderly. A comprehensive search of several databases from database inception to November 2013, only in English language, was conducted. The databases included Pubmed and ISI Web of Science. The search terms «dehydration» / "hyponaremia" / "hypernatremia" AND «cost» AND «elderly» were used to search for comparative studies of the economic burden of dehydration. A total of 15 papers were identified. Dehydration in the elderly is an independent factor of higher health care expenditures. It is directly associated with an increase in hospital mortality, as well as with an increase in the utilization of ICU, short and long term care facilities, readmission rates and hospital resources, especially among those with moderate to severe hyponatremia. Dehydration represents a potential target for intervention to reduce healthcare expenditures and improve patients' quality of life.

Kinetic isotope effect in dehydration of ionic solids. II. The kinetics of dehydration of calcium oxalate monohydrate

International Nuclear Information System (INIS)

Manche, E.P.; Carroll, B.

1977-01-01

The kinetics of the isothermal dehydration of the protonated and deuterated monohydrate of calcium oxalate has been investigated at 120, 150, and 170 0 C. The rate of dehydration for these salts was found to be k/sub H//k/sub D/ = 1.025 +- 0.012. This result rules out the enormous kinetic isotope effect as given in the literature. An isotope effect of a few percent is not ruled out; this magnitude is in keeping with that found by Heinzinger in other dehydration processes. An estimated difference of about 150 cal/mol between the heat of desorption for H 2 O and D 2 O should have led to a ratio, k/sub h//k/sub D/ = 1.20. The smaller observed ratio has been explained on the basis of a compensation effect and may be considered an example of the Barclay--Butler correlation

What's the Big Sweat about Dehydration? (For Kids)

Science.gov (United States)

... First Aid & Safety Doctors & Hospitals Videos Recipes for Kids Kids site Sitio para niños How the Body Works ... for Educators Search English Español Dehydration KidsHealth / For Kids / Dehydration Print en español ¿Por qué es tan ...

Pes cavus and hereditary neuropathies: when a relationship should be suspected.

Science.gov (United States)

Piazza, S; Ricci, G; Caldarazzo Ienco, E; Carlesi, C; Volpi, L; Siciliano, G; Mancuso, M

2010-12-01

The hereditary peripheral neuropathies are a clinically and genetically heterogeneous group of diseases of the peripheral nervous system. Foot deformities, including the common pes cavus, but also hammer toes and twisting of the ankle, are frequently present in patients with hereditary peripheral neuropathy, and often represent one of the first signs of the disease. Pes cavus in hereditary peripheral neuropathies is caused by imbalance between the intrinsic muscles of the foot and the muscles of the leg. Accurate clinical evaluation in patients with pes cavus is necessary to exclude or confirm the presence of peripheral neuropathy. Hereditary peripheral neuropathies should be suspected in those cases with bilateral foot deformities, in the presence of family history for pes cavus and/or gait impairment, and in the presence of neurological symptoms or signs, such as distal muscle hypotrophy of limbs. Herein, we review the hereditary peripheral neuropathies in which pes cavus plays a key role as a "spy sign," discussing the clinical and molecular features of these disorders to highlight the importance of pes cavus as a helpful clinical sign in these rare diseases.

Dehydration and crystallization kinetics of zirconia-yttria gels

International Nuclear Information System (INIS)

Ramanathan, S.; Muraleedharan, R.V.; Roy, S.K.; Nayar, P.K.K.

1995-01-01

Zirconia and zirconia-yttria gels containing 4 and 8 mol% yttria were obtained by coprecipitation and drying at 373 K. The dehydration and crystallization behavior of the dried gels was studied by DSC, TG, and XRD. The gels undergo elimination of water over a wide temperature range of 373--673 K. The peak temperature of the endotherm corresponding to dehydration and the kinetic constants for the process were not influenced by the yttria content of the gel. The enthalpy of dehydration observed was in good agreement with the heat of vaporization data. The dehydration was followed by a sharp exothermic crystallization process. The peak temperature of the exotherm and the activation energy of the process increased with an increase in yttria content, while the enthalpy of crystallization showed a decrease. The ''glow effect'' reduced with increasing yttria content. Pure zirconia crystallizes in the tetragonal form while the zirconia containing 4 and 8 mol% yttria appears to crystallize in the cubic form

Survey of quality indicators in commercial dehydrated fruits.

Science.gov (United States)

Megías-Pérez, Roberto; Gamboa-Santos, Juliana; Soria, Ana Cristina; Villamiel, Mar; Montilla, Antonia

2014-05-01

Physical and chemical quality parameters (dry matter, aw, protein, carbohydrates, vitamin C, 2-furoylmethyl amino acids, rehydration ratio and leaching loss) have been determined in 30 commercial dehydrated fruits (strawberry, blueberry, raspberry, cranberry, cherry, apple, grapefruit, mango, kiwifruit, pineapple, melon, coconut, banana and papaya). For comparison purposes, strawberry samples processed in the laboratory by freeze-drying and by convective drying were used as control samples. Overall quality of dehydrated fruits seemed to be greatly dependent on processing conditions and, in a cluster analysis, samples which were presumably subjected to osmotic dehydration were separated from the rest of fruits. These samples presented the lowest concentration of vitamin C and the highest evolution of Maillard reaction, as evidenced by its high concentration of 2-furoylmethyl amino acids. This is the first study on the usefulness of this combination of chemical and physical indicators to assess the overall quality of commercial dehydrated fruits. Copyright © 2013 Elsevier Ltd. All rights reserved.

Review: Clinical aspects of hereditary DNA Mismatch repair gene mutations

NARCIS (Netherlands)

Sijmons, Rolf H.; Hofstra, Robert M. W.

Inherited mutations of the DNA Mismatch repair genes MLH1, MSH2, MSH6 and PMS2 can result in two hereditary tumor syndromes: the adult-onset autosomal dominant Lynch syndrome, previously referred to as Hereditary Non-Polyposis Colorectal Cancer (HNPCC) and the childhood-onset autosomal recessive

Dehydration of alcohols using solid acid catalysts

OpenAIRE

Cholerton, Mary

2014-01-01

Solid acid catalysts were prepared through silicon substitution into aluminophosphate frameworks. Silicon incorporation was confirmed using solid state nuclear magnetic resonance spectroscopy. The nature of the acid sites generated was determined using Fourier Transform infrared spectroscopy. These materials were tested as catalysts for the dehydration of ethanol to ethylene at low operating temperatures. The materials were active for dehydration of ethanol to ethylene with significant differ...

Studying of the dehydration process of uranyl nitrate hexahydrate (Unh)

International Nuclear Information System (INIS)

Badalov, A.; Kamalov, J.J.; Homidov, B.J.; Mirsaidov, I.U.; Eshbekov, N.R.

2005-01-01

By the tensimeteric method is studying the dehydration process of uranyl nitrate hexahydrate (Unh). It is shown, that the temperature interval 300-400 K in equilibrium conditions the dehydration process of Unh runs in three stages. According to the equations of dependence of saturated steam pressure from temperature, the thermodynamic characteristics of each stage of the dehydration process of Unh are calculated

Ethanol dehydration

OpenAIRE

Ana María Uyazán; Iván Dario Gil; J L Aguilar; Gerardo Rodríguez Niño; Luis Alfonso Caicedo

2004-01-01

This review outlines ethanol dehydration processes and their most important characteristics. It also deals with the main operating variables and some criteria used in designing the separation scheme. A differentiation is made between processes involving liquid steam balance in separation operations and those doing it by screening the difference in molecule size. The last part presents a comparison between the three main industrial processes, stressing their stengths and weaknesses from the op...

Rates of Hospitalization for Dehydration Following Hurricane Sandy in New Jersey.

Science.gov (United States)

Swerdel, Joel N; Rhoads, George G; Cosgrove, Nora M; Kostis, John B

2016-04-01

Hurricane Sandy, one of the most destructive natural disasters in New Jersey history, made landfall on October 29, 2012. Prolonged loss of electrical power and extensive infrastructure damage restricted access for many to food and water. We examined the rate of dehydration in New Jersey residents after Hurricane Sandy. We obtained data from 2008 to 2012 from the Myocardial Infarction Data Acquisition System (MIDAS), a repository of in-patient records from nonfederal New Jersey hospitals (N=517,355). Patients with dehydration had ICD-9-CM discharge diagnosis codes for dehydration, volume depletion, and/or hypovolemia. We used log-linear modeling to estimate the change in in-patient hospitalizations for dehydration comparing 2 weeks after Sandy with the same period in the previous 4 years (2008-2011). In-patient hospitalizations for dehydration were 66% higher after Sandy than in 2008-2011 (rate ratio [RR]: 1.66; 95% confidence interval [CI]: 1.50, 1.84). Hospitalizations for dehydration in patients over 65 years of age increased by nearly 80% after Sandy compared with 2008-2011 (RR: 1.79; 95% CI: 1.58, 2.02). Sandy was associated with a marked increase in hospitalizations for dehydration. Reducing the rate of dehydration following extreme weather events is an important public health concern that needs to be addressed, especially in those over 65 years of age.

[Consensus on clinical diagnosis, treatment and pedigree management of hereditary colorectal cancer in China].

Science.gov (United States)

2018-01-23

Hereditary colorectal cancer can be divded into two categories based on the presence or absence of polyps. The first category is characterized by the development of polyposis, which includes familial adenomatous polyposis (FAP); The second category is nonpolyposis colorectal cancer, which is represented by Lynch syndrome. "Consensus on clinical diagnosis, treatment and pedigree management of hereditary colorectal cancer in China" developed by the Genetics Group of the Committee of Colorectal Cancer, Chinese Anti-cancer Association, is composed of three sections, including hereditary nonpolyposis syndrome, polyposis syndrome as well as genetic evaluation of hereditary colorectal cancer. The consensus aims to provide recommendations on management of the respective hereditary syndromes in terms of definition, clinical and pathological features, diagnostic standards, treatment, and follow-ups. In addition to describing diagnostic and treatment strategies, prophylactic treatment as well as genetic screening and pedigree monitoring is highly recommended. Through the establishment of this expert consensus, we hope to promote better understanding of hereditary colorectal cancer for clinicians and encourage standardized treatment through multidisciplinery approaches, eventually improving clinical treatment and pedigree management of hereditary colorectal cancer in China.

Hereditary hemochromatosis: An opportunity for gene therapy

Directory of Open Access Journals (Sweden)

FERNANDO EZQUER

2006-01-01

Full Text Available Levels of body iron should be tightly controlled to prevent the formation of oxygen radicals, lipoperoxidation, genotoxicity, and the production of cytotoxic cytokines, which result in damage to a number of organs. Enterocytes in the intestinal villae are involved in the apical uptake of iron from the intestinal lumen; iron is further exported from the cells into the circulation. The apical divalent metal transporter-1 (DMT1 transports ferrous iron from the lumen into the cells, while the basolateral transporter ferroportin extrudes iron from the enterocytes into the circulation. Patients with hereditary hemochromatosis display an accelerated transepithelial uptake of iron, which leads to body iron accumulation that results in cirrhosis, hepatocellular carcinoma, pancreatitis, and cardiomyopathy. Hereditary hemochromatosis, a recessive genetic condition, is the most prevalent genetic disease in Caucasians, with a prevalence of one in 300 subjects. The majority of patients with hereditary hemochromatosis display mutations in the gene coding for HFE, a protein that normally acts as an inhibitor of transepithelial iron transport. We discuss the different control points in the homeostasis of iron and the different mutations that exist in patients with hereditary hemochromatosis. These control sites may be influenced by gene therapeutic approaches; one general therapy for hemochromatosis of different etiologies is the inhibition of DMT1 synthesis by antisense-generating genes, which has been shown to markedly inhibit apical iron uptake by intestinal epithelial cells. We further discuss the most promising strategies to develop gene vectors and deliver them into enterocytes

External validation and comparison of three pediatric clinical dehydration scales.

Science.gov (United States)

Jauregui, Joshua; Nelson, Daniel; Choo, Esther; Stearns, Branden; Levine, Adam C; Liebmann, Otto; Shah, Sachita P

2014-01-01

To prospectively validate three popular clinical dehydration scales and overall physician gestalt in children with vomiting or diarrhea relative to the criterion standard of percent weight change with rehydration. We prospectively enrolled a non-consecutive cohort of children ≤ 18 years of age with an acute episode of diarrhea or vomiting. Patient weight, clinical scale variables and physician clinical impression, or gestalt, were recorded before and after fluid resuscitation in the emergency department and upon hospital discharge. The percent weight change from presentation to discharge was used to calculate the degree of dehydration, with a weight change of ≥ 5% considered significant dehydration. Receiver operating characteristics (ROC) curves were constructed for each of the three clinical scales and physician gestalt. Sensitivity and specificity were calculated based on the best cut-points of the ROC curve. We approached 209 patients, and of those, 148 were enrolled and 113 patients had complete data for analysis. Of these, 10.6% had significant dehydration based on our criterion standard. The Clinical Dehydration Scale (CDS) and Gorelick scales both had an area under the ROC curve (AUC) statistically different from the reference line with AUCs of 0.72 (95% CI 0.60, 0.84) and 0.71 (95% CI 0.57, 0.85) respectively. The World Health Organization (WHO) scale and physician gestalt had AUCs of 0.61 (95% CI 0.45, 0.77) and 0.61 (0.44, 0.78) respectively, which were not statistically significant. The Gorelick scale and Clinical Dehydration Scale were fair predictors of dehydration in children with diarrhea or vomiting. The World Health Organization scale and physician gestalt were not helpful predictors of dehydration in our cohort.

Prognostic value of amplitude-integrated electroencephalography in neonates with hypernatremic dehydration.

Science.gov (United States)

Tekgunduz, Kadir Şerafettin; Caner, Ibrahim; Eras, Zeynep; Taştekin, Ayhan; Tan, Huseyin; Dinlen, Nurdan

2014-05-01

Hypernatremic dehydration in neonates is a condition that develops due to inadequate fluid intake and it may lead to cerebral damage. We aimed to determine whether there was an association between serum sodium levels on admission and aEEG patterns and prognosis, as well as any association between aEEG findings and survival rates and long-term prognosis. The present study included all term infants hospitalized for hypernatremic dehydration in between January 2010 and May 2011. Infants were monitored by aEEG. At 2 years of age, we performed a detailed evaluation to assess the impact of hypernatremic dehydration on the neurodevelopmental outcome. Twenty-one infants were admitted to the neonatal intensive care unit for hypernatremic dehydration. A correlation was found between increased serum sodium levels and aEEG abnormalities. Neurodevelopmental assessment was available for 17 of the 21 infants. The results revealed that hypernatremic dehydration did not adversely affect the long-term outcomes. The follow-up of newborns after discharge is key to determine the risks associated with hypernatremic dehydration. Our results suggest that hypernatremic dehydration had no impact on the long-term outcome. In addition, continuous aEEG monitoring could provide information regarding early prognosis and mortality.

Peculiarities of dehydration of calcium dodeca- and hexavanadates

International Nuclear Information System (INIS)

Koryakova, O.V.; Perelyaeva, L.A.; Zabolotskaya, E.V.; Medvedeva, N.I.; Ivakin, A.A.

1982-01-01

Infrared spectroscopic, electron paramagentic resonance, X-ray diffraction and thermogravimetric methods were used to study the process and dehydration products of CaV 12 O 31 x12H 2 O and CaV 6 O 16 x6H 2 O calcium dodeca-and hexavanadates. The dependence of chemical composition on dehydration temperature was established. It was revealed that investigated polyvanadates contain two types of water crystallization and coordinationally linked-one. Complete dehydration (300-400 deg C) leads to irreversible structural changes and formation of oxide vanadium bronze. The ''final'' product (t > 500 deg C) is the mixture of vanadium pentaoxide and calcium metavanadate

RECRUITMENT OF PATIENTS WITH HEREDITARY HAEMOCHROMATOSIS AS BLOOD DONORS

Directory of Open Access Journals (Sweden)

Marko Cukjati

2004-12-01

Full Text Available Background. Hereditary haemochromatosis is the most common inherited disorder in white persons with prevalence of about 1 in 200. Therapeutic phlebotomy is an effective treatment for the disease and prevents its sequele. In addition to their altruism, patients with hereditary haemochromatosis have also medical and monetary incentives for blood donation. Current guidelines do not allow haemochromatosis patients to donate blood. About two thirds of patients are eligible as blood donors and about two thirds of therapeutically drawn blood is suitable for transfusion. Therapeutically drawn blood could increase the blood supply by 1.5 to 30%.Conclusions. The number of states that already accept patients with hereditary haemochromatosis as blood donors is increasing. To avoid monetary incentives they offer free phlebotomies for all patients with hereditary haemochromatosis. There have been no reports about higher incidence of transfusion reactions. In Slovenia the number of therapeutic phlebotomy is increasing. We should evaluate the possibilities for recruitment of haemochromatosis patients as blood donors also in our country. It is necessary to modify regulatory restrictions and to ensure that there is no other incentives than altruism for blood donation.

Genetics and ionizing radiations. 1. Genetics and hereditary diseases

International Nuclear Information System (INIS)

Dutrillaux, B.

1980-01-01

The desoxyribonucleic acid (DNA) is the chemical vehicle of heredity. Each hereditary character is determined by a short segment of the DNA molecule called gene. Gene operations are governed by regulating systems. The DNA is located in the chromosomes, easily analysed by light microscopy. The chromosome number and form are fairly characteristic of a species. Ours has 46 chromosomes, i.e. 23 pairs. Anomalies of the hereditary stock can be qualitative: affecting one gene they are expressed by diversely serious diseases. They can be quantitative and bear on the lack or excess of a chromosome or a segment of chromosome; most often, resulting diseases are very serious; Downs's syndrome is a well-known example. The various modes of transmission of these hereditary characters are analysed. The change of a chromosome or a gene from a normal to an abnormal form is called a mutation. It occurs scarcely, but the effects of mutations accumulate. At birth, nearly 10% of children should have one abnormal hereditary character at least, however most of these characters do not induce a true disease. Anomalies are more frequent at conception, many abnormal embryos or foetuses being aliminated by miscarriages [fr

Dehydration processes using membranes with hydrophobic coating

Science.gov (United States)

Huang, Yu; Baker, Richard W; Aldajani, Tiem; Ly, Jennifer

2013-07-30

Processes for removing water from organic compounds, especially polar compounds such as alcohols. The processes include a membrane-based dehydration step, using a membrane that has a dioxole-based polymer selective layer or the like and a hydrophilic selective layer, and can operate even when the stream to be treated has a high water content, such as 10 wt % or more. The processes are particularly useful for dehydrating ethanol.

Production and evaluation of precooked dehydrated unripe banana ...

African Journals Online (AJOL)

No significant change in total aerobic counts or yeasts and moulds counts occurred in dehydrated banana slices packaged in 250 gauge polyethylene bags and stored at ambient temperature for 3 months. The slices were found to be high in starch (~68.5%) and minerals. When shallow fried, the dehydrated banana slices ...

Osmotic dehydration of fruit and berry raw materials in the food industry

Directory of Open Access Journals (Sweden)

N. A. Gribova

2017-01-01

Full Text Available Osmotic dehydration has recently received more attention as an effective method of preserving fruits and berries. Osmosis is a simple process that facilitates the processing of fruits and berries in order to preserve the original characteristics, namely nutritional value and organoleptic properties: color, aroma and texture. Osmotic dehydration has found wide application in the preservation of food products, as the activity of water in fruits and berries decreases, in some of them up to 90% of water is contained. The process of osmotic dehydration with the help of various agents is less energy-intensive than the process of drying or freezing, since it can be processed at ambient temperature. Osmotic dehydration has potential advantages in preserving the quality of food and in maintaining healthy food for the food industry. Treatment includes dehydration of fruits and berries by an osmotic agent followed by dehydration in dry or frozen apparatus where the moisture content decreases and the product becomes more stable. This process is a partial dewatering process to provide improved product quality compared to conventional drying processes or freezing. The purpose of studying osmotic dehydration is to identify the advantages and disadvantages in the treatment of osmotic agents. Various aspects of osmotic dehydration technology are considered, namely the solutions used, the characteristics of solutions, the effect of variable processes and the qualitative characteristics of osmo-dehydrated products. Factors of osmotic dehydration that depend on the osmotic agent, concentration of solute, temperature, time, size, shape and compactness of the material, mixing and the ratio of the solution to the samples.

Measured degree of dehydration in children and adolescents with type 1 diabetic ketoacidosis.

Science.gov (United States)

Ugale, Judith; Mata, Angela; Meert, Kathleen L; Sarnaik, Ashok P

2012-03-01

Successful management of diabetic ketoacidosis depends on adequate rehydration while avoiding cerebral edema. Our objectives are to 1) measure the degree of dehydration in children with type 1 diabetes mellitus and diabetic ketoacidosis based on change in body weight; and 2) investigate the relationships between measured degree of dehydration and clinically assessed degree of dehydration, severity of diabetic ketoacidosis, and routine serum laboratory values. Prospective observational study. University-affiliated tertiary care children's hospital. Sixty-six patients dehydration was based on the difference between admission and plateau weights. Clinical degree of dehydration was assessed by physical examination and severity of diabetic ketoacidosis was assessed by blood gas values as defined by international guidelines. Laboratory values obtained on admission included serum glucose, urea nitrogen, sodium, and osmolality. Median measured degree of dehydration was 5.2% (interquartile range, 3.1% to 7.8%). Fourteen (21%) patients were clinically assessed as mild dehydration, 49 (74%) as moderate, and three (5%) as severe. Patients clinically assessed as moderately dehydrated had a greater measured degree of dehydration (5.8%; interquartile range, 3.6% to 9.6%) than those assessed as mildly dehydrated (3.7%; interquartile range, 2.3% to 6.4%) or severely dehydrated (2.5%; interquartile range, 2.3% to 2.6%). Nine (14%) patients were assessed as mild diabetic ketoacidosis, 18 (27%) as moderate, and 39 (59%) as severe. Diabetic ketoacidosis severity groups did not differ in measured degree of dehydration. Variables independently associated with measured degree of dehydration included serum urea nitrogen and sodium concentration on admission. Hydration status in children with diabetic ketoacidosis cannot be accurately assessed by physical examination or blood gas values. Fluid therapy based on maintenance plus 6% deficit replacement is reasonable for most patients.

Dehydration kinetics of boehmite in the temperature range 723-873 K

Energy Technology Data Exchange (ETDEWEB)

Xu, Bingan, E-mail: bingan.xu@csiro.au [A.J. Parker Cooperative Research Centre for Integrated Hydrometallurgy Solutions, CSIRO Light Metals Flagship (Division of Process Science and Engineering), PO Box 7229, Karawara, WA 6152 (Australia); Smith, Peter [A.J. Parker Cooperative Research Centre for Integrated Hydrometallurgy Solutions, CSIRO Light Metals Flagship (Division of Process Science and Engineering), PO Box 7229, Karawara, WA 6152 (Australia)

2012-03-10

Highlights: Black-Right-Pointing-Pointer The dehydration of boehmite is a complex topotactic process. Black-Right-Pointing-Pointer The process is chemical control with a diffusion complication as the reaction progresses. Black-Right-Pointing-Pointer Al cation diffusion is the rate-limiting step. Black-Right-Pointing-Pointer The reaction rates predicted by the model-free method agree well with the measured values. - Abstract: The dehydration kinetics of boehmite prepared from the hydrothermal transformation of gibbsite has been comprehensively studied to clarify the mechanism of the dehydration process using a thermogravimetric technique under both isothermal and non-isothermal conditions. Selected residues were examined by XRD, SEM, TEM and selected area electron diffraction (SAED). Results indicate that the dehydration process is a topotactic reaction which brings about {gamma}-Al{sub 2}O{sub 3} with porous microstructure and poor crystallinity. The kinetic data were analysed using the model-free (isoconversional) methods. The results indicate that the activation energy of the dehydration process depends on the reaction extent, signifying the dehydration process is chemical controlled with a diffusion complication as the reaction extent increases. Prediction of the dehydration extent was accomplished using the kinetic parameter of the non-isothermal process and the isoconversional method. The predicted rate is in a reasonably good agreement with the measured values. Finally the mechanism of the dehydration process is discussed based on the auxiliary results from the physical examination of the reacted residues and the kinetic behaviour.

Hereditary spastic paraplegia with cerebellar ataxia

DEFF Research Database (Denmark)

Nielsen, J E; Johnsen, B; Koefoed, P

2004-01-01

Complex forms of hereditary spastic paraplegia (HSP) are rare and usually transmitted in an autosomal recessive pattern. A family of four generations with autosomal dominant hereditary spastic paraplegia (AD-HSP) and a complex phenotype with variably expressed co-existing ataxia, dysarthria......, unipolar depression, epilepsy, migraine, and cognitive impairment was investigated. Genetic linkage analysis and sequencing of the SPG4 gene was performed and electrophysiologic investigations were carried out in six individuals and positron emission tomography (PET) in one patient. The disease was linked...... in those individuals who were clinically affected by a complex phenotype consisting of HSP and cerebellar ataxia. Other features noted in this kindred including epilepsy, cognitive impairment, depression, and migraine did not segregate with the HSP phenotype or mutation, and therefore the significance...

Hereditary myopathies with early respiratory insufficiency in adults.

Science.gov (United States)

Naddaf, Elie; Milone, Margherita

2017-11-01

Hereditary myopathies with early respiratory insufficiency as a predominant feature of the clinical phenotype are uncommon and underestimated in adults. We reviewed the clinical and laboratory data of patients with hereditary myopathies who demonstrated early respiratory insufficiency before the need for ambulatory assistance. Only patients with disease-causing mutations or a specific histopathological diagnosis were included. Patients with cardiomyopathy were excluded. We identified 22 patients; half had isolated respiratory symptoms at onset. The diagnosis of the myopathy was often delayed, resulting in delayed ventilatory support. The most common myopathies were adult-onset Pompe disease, myofibrillar myopathy, multi-minicore disease, and myotonic dystrophy type 1. Single cases of laminopathy, MELAS (mitochondrial encephalomyopathy with lactic acidosis and strokelike events), centronuclear myopathy, and cytoplasmic body myopathy were identified. We highlighted the most common hereditary myopathies associated with early respiratory insufficiency as the predominant clinical feature, and underscored the importance of a timely diagnosis for patient care. Muscle Nerve 56: 881-886, 2017. © 2017 Wiley Periodicals, Inc.

External validation and comparison of three pediatric clinical dehydration scales.

Directory of Open Access Journals (Sweden)

Joshua Jauregui

Full Text Available OBJECTIVE: To prospectively validate three popular clinical dehydration scales and overall physician gestalt in children with vomiting or diarrhea relative to the criterion standard of percent weight change with rehydration. METHODS: We prospectively enrolled a non-consecutive cohort of children ≤ 18 years of age with an acute episode of diarrhea or vomiting. Patient weight, clinical scale variables and physician clinical impression, or gestalt, were recorded before and after fluid resuscitation in the emergency department and upon hospital discharge. The percent weight change from presentation to discharge was used to calculate the degree of dehydration, with a weight change of ≥ 5% considered significant dehydration. Receiver operating characteristics (ROC curves were constructed for each of the three clinical scales and physician gestalt. Sensitivity and specificity were calculated based on the best cut-points of the ROC curve. RESULTS: We approached 209 patients, and of those, 148 were enrolled and 113 patients had complete data for analysis. Of these, 10.6% had significant dehydration based on our criterion standard. The Clinical Dehydration Scale (CDS and Gorelick scales both had an area under the ROC curve (AUC statistically different from the reference line with AUCs of 0.72 (95% CI 0.60, 0.84 and 0.71 (95% CI 0.57, 0.85 respectively. The World Health Organization (WHO scale and physician gestalt had AUCs of 0.61 (95% CI 0.45, 0.77 and 0.61 (0.44, 0.78 respectively, which were not statistically significant. CONCLUSION: The Gorelick scale and Clinical Dehydration Scale were fair predictors of dehydration in children with diarrhea or vomiting. The World Health Organization scale and physician gestalt were not helpful predictors of dehydration in our cohort.

Which Frail Older People Are Dehydrated? The UK DRIE Study.

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Hooper, Lee; Bunn, Diane K; Downing, Alice; Jimoh, Florence O; Groves, Joyce; Free, Carol; Cowap, Vicky; Potter, John F; Hunter, Paul R; Shepstone, Lee

2016-10-01

Water-loss dehydration in older people is associated with increased mortality and disability. We aimed to assess the prevalence of dehydration in older people living in UK long-term care and associated cognitive, functional, and health characteristics. The Dehydration Recognition In our Elders (DRIE) cohort study included people aged 65 or older living in long-term care without heart or renal failure. In a cross-sectional baseline analysis, we assessed serum osmolality, previously suggested dehydration risk factors, general health, markers of continence, cognitive and functional health, nutrition status, and medications. Univariate linear regression was used to assess relationships between participant characteristics and serum osmolality, then associated characteristics entered into stepwise backwards multivariate linear regression. DRIE included 188 residents (mean age 86 years, 66% women) of whom 20% were dehydrated (serum osmolality >300 mOsm/kg). Linear and logistic regression suggested that renal, cognitive, and diabetic status were consistently associated with serum osmolality and odds of dehydration, while potassium-sparing diuretics, sex, number of recent health contacts, and bladder incontinence were sometimes associated. Thirst was not associated with hydration status. DRIE found high prevalence of dehydration in older people living in UK long-term care, reinforcing the proposed association between cognitive and renal function and hydration. Dehydration is associated with increased mortality and disability in older people, but trials to assess effects of interventions to support healthy fluid intakes in older people living in residential care are needed to enable us to formally assess causal direction and any health benefits of increasing fluid intakes. © The Author 2015. Published by Oxford University Press on behalf of The Gerontological Society of America. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Hereditary hemochromatosis

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Stephen A. Geller

2015-03-01

Full Text Available Hereditary hemochromatosis (HH is the most commonly identified autosomal recessive genetic disorder in the white population, characterized by increased intestinal iron absorption and secondary abnormal accumulation in parenchymal organs, not infrequently accompanied by functional impairment. This entity is associated with mutations of the HFE gene (located on the short arm of chromosome 6 at location 6p22.2; closely linked to the HLA-A3 locus, which encodes the HFE protein, a membrane protein thought to regulate iron absorption by affecting the interaction between transferrin receptor and transferrin.

Physicochemical characteristics of guava “Paluma” submitted to osmotic dehydration

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Roselene Ferreira Oliveira

2014-09-01

Full Text Available The aim of this work was to evaluate the conservation post process osmotic of guava stored temperature at 5oC. Guava (Psidium guajava L., red variety “Paluma” minimally processed by mild osmotic dehydration, were packaged in polyethylene terephthalate (PET and stored temperature at 5ºC. Non-treated guava, packed in PET trays, was used as control. The treatment used was osmotic dehydration in sucrose syrup at 60ºBrix and physicochemical determinations were pH, total soluble solids (TSS, total titratable acidity (TTA, reducing sugars (RS, total sugars (TS and parameters related to colour read (a*, chroma (c*, yellow (b*, luminosity (L* of the fresh and osmotically dehydrated guava slices. The dehydrated fruits lost about 34.45% of water, concentrating contents of soluble solids, total and reducing sugars, when compared to control samples. The pH value remained around 3.76 for the OD fruits and 3.87 for the fresh fruits. The colour of the dehydrated fruits was more intense than the control samples’. The guava slices osmotic dehydration had 21 days of shelf life, showed physicochemical characteristics significantly superior to the control samples’, having a stable and high quality product as a result.

Hereditary Transthyretin Amyloidosis in Eight Chinese Families

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Ling-Chao Meng

2015-01-01

Full Text Available Background: Mutations of transthyretin (TTR cause the most common type of autosomal-dominant hereditary systemic amyloidosis, which occurs worldwide. To date, more and more mutations in the TTR gene have been reported. Some variations in the clinical presentation are often observed in patients with the same mutation or the patients in the same family. The purpose of this study was to find out the clinicopathologic and genetic features of Chinese patients with hereditary TTR amyloidosis. Methods: Clinical and necessary examination materials were collected from nine patients of eight families with hereditary TTR amyloidosis at Peking University First Hospital from January 2007 to November 2014. Sural nerve biopsies were taken for eight patients and skin biopsies were taken in the calf/upper arm for two patients, for light and electron microscopy examination. The TTR genes from the nine patients were analyzed. Results: The onset age varied from 23 to 68 years. The main manifestations were paresthesia, proximal and/or distal weakness, autonomic dysfunction, cardiomyopathy, vitreous opacity, hearing loss, and glossohypertrophia. Nerve biopsy demonstrated severe loss of myelinated fibers in seven cases and amyloid deposits in three. One patient had skin amyloid deposits which were revealed from electron microscopic examination. Genetic analysis showed six kinds of mutations of TTR gene, including Val30Met, Phe33Leu, Ala36Pro, Val30Ala, Phe33Val, and Glu42Gly in exon 2. Conclusions: Since the pathological examinations of sural nerve were negative for amyloid deposition in most patients, the screening for TTR mutations should be performed in all the adult patients, who are clinically suspected with hereditary TTR amyloidosis.

Ozone fumigation for safety and quality of wine grapes in postharvest dehydration.

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Botondi, Rinaldo; De Sanctis, Federica; Moscatelli, Niccolò; Vettraino, Anna Maria; Catelli, Cesare; Mencarelli, Fabio

2015-12-01

This paper proposes postharvest ozone fumigation (as a method) to control microorganisms and evaluate the effect on polyphenols, anthocyanins, carotenoids and cell wall enzymes during the grape dehydration for wine production. Pignola grapes were ozone-treated (1.5 g/h) for 18 h (A=shock treatment), then dehydrated or ozone-treated (1.5 g/h) for 18 h and at 0.5 g/h for 4 h each day (B=long-term treatment) during dehydration. Treatment and dehydration were performed at 10 °C. No significant difference was found for total carotenoid, total phenolic and total anthocyanin contents after 18 h of O3 treatment. A significant decrease in phenolic and anthocyanin contents occurred during treatment B. Also carotenoids were affected by B ozone treatment. Pectin methylesterase (PME) and polygalacturonase (PG) activities were higher in A-treated grapes during dehydration. Finally, ozone reduced fungi and yeasts by 50%. Shock ozone fumigation (A treatment) before dehydration can be used to reduce the microbial count during dehydration without affecting polyphenol and carotenoid contents. Copyright © 2015 Elsevier Ltd. All rights reserved.

Voluntary dehydration among elementary school children residing in a hot arid environment.

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Bar-David, Y; Urkin, J; Landau, D; Bar-David, Z; Pilpel, D

2009-10-01

Voluntary dehydration is a condition where humans do not drink appropriately in the presence of an adequate fluid supply. This may adversely affect their physical and intellectual performance. The present study aimed to describe the prevalence of voluntary dehydration among elementary school children of different ethnicities and countries of birth. Four hundred and twenty-nine elementary school children, aged 8-10 years, from four subpopulations (Israeli-born Jewish and Bedouin-Arab children, and immigrant children who recently arrived to Israel from Eastern Europe and from Ethiopia) were studied. The level of dehydration was determined by noontime urine osmolality, from samples taken over 1 week in mid-summer. Urine osmolality dehydrated group was that of Israeli-born Jewish children, whereas the Bedouin-Arab children were the least dehydrated. A high proportion of children who reside in a hot and arid environment were found to be in a state of moderate to severe dehydration. Bedouin ethnicity was associated with better hydration, whereas Israeli-born Jews were most severely dehydrated. Educational intervention programmes promoting water intake should start in early childhood and continue throughout life.

Advances and challenges in hereditary cancer pharmacogenetics.

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Cascorbi, Ingolf; Werk, Anneke Nina

2017-01-01

Cancer pharmacogenetics usually considers tumor-specific targets. However, hereditary genetic variants may interfere with the pharmacokinetics of antimetabolites and other anti-cancer drugs, which may lead to severe adverse events. Areas covered: Here, the impact of hereditary genes considered in drug labels such as thiopurine S-methyltransferase (TPMT), UDP-glucuronosyltransferase 1A1 (UTG1A1) and dihydropyrimidine dehydrogenase (DPYD) are discussed with respect to guidelines of the Clinical Pharmacogenetics Implementation Consortium (CPIC). Moreover, the association between genetic variants of drug transporters with the clinical outcome is comprehensively discussed. Expert opinion: Precision therapy in the field of oncology is developing tremendously. There are a number of somatic tumor genetic markers that are indicative for treatment with anti-cancer drugs. By contrast, for some hereditary variants, recommendations have been developed. Although we have vast knowledge on the association between drug transporter variants and clinical outcome, the overall data is inconsistent and the predictability of the related phenotype is low. Further developments in research may lead to the discovery of rare, but functionally relevant single nucleotide polymorphisms and a better understanding of multiple genomic, epigenomic as well as phenotypic factors, contributing to drug response in malignancies.

Thermodynamics of dehydration process of uranyl nitrate pentahydrate of thorium

International Nuclear Information System (INIS)

Khamidov, F.A.; Mirsaidov, I.U.; Nazarov, K.M.; Nasriddinov, S.K.; Badalov, A.B.

2010-01-01

Present article is devoted to thermodynamics of dehydration process of uranyl nitrate pentahydrate of thorium. The results of researches of dehydration process of uranyl nitrate pentahydrate of thorium Th(NO_3)_4·5H_2O were considered. The researches were carried out by means of tensimeter method with membrane zero-manometer under equilibrium conditions and at 300-450 K temperature ranges. The thermodynamic characteristics of dehydration process of initial crystalline hydrate was defined.

Weathering of natural uranyl oxide hydrates: Schoepite polytypes and dehydration effects

International Nuclear Information System (INIS)

Finch, R.J.; Miller, M.L.; Ewing, R.C.

1992-01-01

Partial dehydration of schoepite, UO 3 x2H 2 O, is reported to produce three discrete schoepite polytypes with characteristic unit cell parameters, but this has not been confirmed. The loss of structural water from the schoepite interlayer results in progressive modification to the structure; expansion parallel to schoepite cleavage planes, and extensive fracturing. Dehydration of schoepite commences at grain boundaries and progresses inward until the entire grain is converted to dehydrated schoepite, UO 3 x0.8H 2 O. The volume decrease associated with dehydration results in expanded grain boundaries. These gaps can provide pathways for the access of groundwater, and uranyl silicates and uranyl carbonates have precipitated within these gaps, replacing both schoepite and dehydrated schoepite. Schoepite, however, is not observed to re-precipitate where in contact with dehydrated schoepite. Thus, while the formation of schoepite early during the corrosion of uraninite may be favored, schoepite is not a long-term solubility limiting phase for oxidized uranium in natural ground waters containing dissolved silica or carbonate. (orig.)

Effect of friction on vibrotactile sensation of normal and dehydrated skin.

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Chen, S; Ge, S; Tang, W; Zhang, J

2016-02-01

Vibrotactile sensation mediated is highly dependent on surface mechanical and frictional properties. Dehydration of skin could change these properties. To investigate the relationship between friction and vibrotactile sensation of normal and dehydrated skin. Vibrations were firstly measured during surface exploration using a biomimetic sensor. Piglet skin was used as human skin model to study frictional properties for both normal and dehydrated skin using an atomic force microscope on nanoscale and a pin-on-disk tribometer on macroscale. Effect of vibrational frequency on friction and vibrotactile perception was also observed on nano and macro scale for normal and dehydrated skin. The result indicated that dehydrated skin was less sensitive than normal skin. The coefficient of friction of dehydrated skin is smaller than that of normal skin on both nano and macro scale. The coefficient of friction increases as increasing scanning frequencies. There is a positive correlation between coefficient of friction and vibrotactile sensation on nanoscale and macroscale. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Intraocular pressure following 18 hours of systemic dehydration in ocular normotensive healthy subjects

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Faustina K. Idu

2015-07-01

Full Text Available Aim: To investigate the effect of dehydration on intraocular pressure (IOP. Subjects: The study population comprised 50 subjects (27 men and 23 women with a mean age of 42.84 ± 2.64 years. Method: The subjects dehydrated for 18 hours. IOP was assessed in both eyes using the Perkins handheld applanation tonometer. Baseline measurement of IOP was taken before the subjects dehydrated for 18 hours and repeated after dehydration. Blood samples were taken before and after dehydration for the determination of plasma osmolality and plasma antidiuretic hormone (ADH concentrations. Results: A statistically significant (p < 0.001 reduction in the mean weight of subjects occurred after dehydration. There was a significant reduction in IOP from 16.68 mmHg ± 0.32 mmHg to 13.08 mmHg ± 0.31 mmHg after dehydration (p < 0.001. Mean plasma osmolality showed a statistically significant increase (p < 0.05 from baseline of 290.02 mOsmol/kg ± 1.25 mOsmol/kg to 294.96 mOsmol/kg ± 1.33 mOsmol/kg after dehydration. There was also a significant increase (p < 0.05 in mean plasma ADH concentration from 5.36 pg/mL ± 0.21 pg/mL to 6.40 pg/mL ± 0.20 pg/mL after dehydration. There were no significant differences in both systolic and diastolic blood pressures before and after dehydration. Conclusion: Dehydration decreases IOP in healthy humans; this decrease probably results from an increase in plasma osmolality and plasma ADH concentrations.

Ethanol dehydration

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Ana María Uyazán

2004-09-01

Full Text Available This review outlines ethanol dehydration processes and their most important characteristics. It also deals with the main operating variables and some criteria used in designing the separation scheme. A differentiation is made between processes involving liquid steam balance in separation operations and those doing it by screening the difference in molecule size. The last part presents a comparison between the three main industrial processes, stressing their stengths and weaknesses from the operational, energy consumption and industrial services points of view.

Ultrasound assessment of severe dehydration in children with diarrhea and vomiting.

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Levine, Adam C; Shah, Sachita P; Umulisa, Irenee; Munyaneza, Richard B Mark; Dushimiyimana, Jean Marie; Stegmann, Katrina; Musavuli, Juvenal; Ngabitsinze, Protogene; Stulac, Sara; Epino, Henry M; Noble, Vicki E

2010-10-01

The objective of this study was to determine the test characteristics for two different ultrasound (US) measures of severe dehydration in children (aorta to inferior vena cava [IVC] ratio and IVC inspiratory collapse) and one clinical measure of severe dehydration (the World Health Organization [WHO] dehydration scale). The authors enrolled a prospective cohort of children presenting with diarrhea and/or vomiting to three rural Rwandan hospitals. Children were assessed clinically using the WHO scale and then underwent US of the IVC by a second clinician. All children were weighed on admission and then fluid-resuscitated according to standard hospital protocols. A percent weight change between admission and discharge of greater than 10% was considered the criterion standard for severe dehydration. Receiver operating characteristic (ROC) curves were created for each of the three tests of severe dehydration compared to the criterion standard. Children ranged in age from 1 month to 10 years; 29% of the children had severe dehydration according to the criterion standard. Of the three different measures of dehydration tested, only US assessment of the aorta/IVC ratio had an area under the ROC curve statistically different from the reference line. At its best cut-point, the aorta/IVC ratio had a sensitivity of 93% and specificity of 59%, compared with 93% and 35% for IVC inspiratory collapse and 73% and 43% for the WHO scale. Ultrasound of the aorta/IVC ratio can be used to identify severe dehydration in children presenting with acute diarrhea and may be helpful in guiding clinical management. © 2010 by the Society for Academic Emergency Medicine.

40 CFR 407.50 - Applicability; description of the dehydrated potato products subcategory.

Science.gov (United States)

2010-07-01

... dehydrated potato products subcategory. 407.50 Section 407.50 Protection of Environment ENVIRONMENTAL... PROCESSING POINT SOURCE CATEGORY Dehydrated Potato Products Subcategory § 407.50 Applicability; description of the dehydrated potato products subcategory. The provisions of this subpart are applicable to...

Dehydration and rehydration of a tuff vitrophyre

International Nuclear Information System (INIS)

Vaniman, D.; Bish, D., Chipera, S.

1993-01-01

The basal vitrophyre of the Topopah Spring Member of the Paintbrush Tuff at Yucca Mountain, Nevada, is a perlitic glass with 2.8 to 4.6% water. The dehydration of this vitrophyre was studied by thermogravimetric analysis (TGA) and by isothermal heating at 50 degrees, 100 degrees, 200 degrees, and 400 degrees C for 3.4 years followed by 1.1 years of rehydration at high controlled humidity (∼79% relative humidity). No crystallization of the glass was observed in long-term dehydration or rehydration; the only observed chemical alteration was loss of up to 60% of original fluorine. TGA studies show a characteristic two-stage dehydration of the vitrophyre, with two-thirds to three-fourths weight loss occurring most rapidly at temperatures ranging from 278 degrees to 346 degrees C in 10 degrees C/min heating experiments. The remaining water, about 1% in all of the vitrophyre samples studied regardless of total water content, is lost only on second-stage heating to temperatures above 650 degrees C. Long-term isothermal heating at ≤400 degrees C releases only the first-stage water. Loss of essentially all first-stage water occurred in less than 1 hour at 400 degrees C; proportionately lower losses were obtained at 200 degrees and 100 degrees C. Small (0.2%) water loss occurred in the 50 degrees C experiment. A time-temperature-dehydration diagram generated from the isothermal heating data shows a clustering of dehydration contours that are the equilibrium equivalent of the rapid first-stage water loss in dynamic TGA experiments. These dry-heating experiments provide an end-member characterization of glass transformations for comparison with water-saturated heating experiments in which glass alteration is prominent. 29 figs., 7 figs., 7 tabs

Genetics Home Reference: hereditary hypophosphatemic rickets

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... A characteristic of X-linked inheritance is that fathers cannot pass X-linked traits to their sons. ... families, hereditary hypophosphatemic rickets has had an autosomal dominant inheritance pattern, which means one copy of an ...

Hereditary History Preserving Bisimilarity Is Undecidable

DEFF Research Database (Denmark)

Jurdzinski, Marcin; Nielsen, Mogens

2000-01-01

History preserving bisimilarity (hp-bisimilarity) and hereditary history preserving bisimilarity (hhp-bisimilarity) are behavioural equivalences taking into account causal relationships between events of concurrent systems. Their prominent feature is being preserved under action refinement...

Research on terahertz properties of rat brain tissue sections during dehydration

Science.gov (United States)

Cui, Gangqiang; Liang, Jianfeng; Zhao, Hongwei; Zhao, Xianghui; Chang, Chao

2018-01-01

Biological tissue sections are always kept in a system purged with dry nitrogen for the measurement of terahertz spectrum. However, the injected nitrogen will cause dehydration of tissue sections, which will affect the accuracy of spectrum measurement. In this paper, terahertz time-domain spectrometer is used to measure the terahertz spectra of rat brain tissue sections during dehydration. The changes of terahertz properties, including terahertz transmittance, refractive index and extinction coefficient during dehydration are also analyzed. The amplitudes of terahertz time-domain spectra increase gradually during the dehydration process. Besides, the terahertz properties show obvious changes during the dehydration process. All the results indicate that the injected dry nitrogen has a significant effect on the terahertz spectra and properties of tissue sections. This study contributes to further research and application of terahertz technology in biomedical field.

New forms of -compactness with respect to hereditary classes

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Abdo Mohammed Qahis

2019-01-01

Full Text Available A hereditary class on a set X is a nonempty collection of subsets closed under heredity. The aim of this paper is to introduce and study strong forms of u-compactness in generalized topological spaces with respect to a hereditary class, called  SuH-compactness and S- SuH-compactness. Also several of their properties are presented. Finally some eects of various kinds of functions on them are studied.

Analysis of Genetic Mutations in a Cohort of Hereditary Optic Neuropathy in Shanghai, China.

Science.gov (United States)

Gan, Dekang; Li, Mengwei; Wu, Jihong; Sun, Xinghuai; Tian, Guohong

2017-01-01

To evaluate the clinical classification and characteristics of hereditary optic neuropathy patients in a single center in China. Retrospective case study. Patients diagnosed with hereditary optic neuropathy between January 2014 and December 2015 in the neuro-ophthalmology division in Shanghai Eye and ENT Hospital of Fudan University were recruited. Clinical features as well as visual field, brain/orbital MRI, and spectrum domain optical coherence tomography (SD-OCT) were analyzed. Eighty-two patients diagnosed by gene test were evaluated, including 66 males and 16 females. The mean age of the patients was 19.4 years (range, 5-46 years). A total of 158 eyes were analyzed, including 6 unilateral, 61 bilateral, and 15 sequential. The median duration of the disease was 0.5 year (range, 0.1-20 years). Genetic test identified 68 patients with Leber hereditary optic neuropathy, 9 with dominant optic neuropathy, and 2 with a Wolfram gene mutation. There was also one case of hereditary spastic paraplegia, spinocerebellar ataxia, and polymicrogyria with optic nerve atrophy, respectively. Leber hereditary optic neuropathy is the most common detected type of hereditary optic neuropathy in Shanghai, China. The detection of other autosomal mutations in hereditary optic neuropathy is limited by the currently available technique.

Clinical features of Hereditary Haemorrhagic Telangiectasia

NARCIS (Netherlands)

Hosman, A.E.

2017-01-01

Hereditary Haemorrhagic Telangiectasia (HHT), also known as Rendu-Osler-Weber disease (ROW), is an autosomal dominant disease with multi-systemic vascular dysplasia characterized by mucocutaneous telangiectasia, arteriovenous malformations and recurrent spontaneous epistaxis (nosebleeds). Most cases

Dehydration kinetics of boehmite in the temperature range 723–873 K

International Nuclear Information System (INIS)

Xu, Bingan; Smith, Peter

2012-01-01

Highlights: ► The dehydration of boehmite is a complex topotactic process. ► The process is chemical control with a diffusion complication as the reaction progresses. ► Al cation diffusion is the rate-limiting step. ► The reaction rates predicted by the model-free method agree well with the measured values. - Abstract: The dehydration kinetics of boehmite prepared from the hydrothermal transformation of gibbsite has been comprehensively studied to clarify the mechanism of the dehydration process using a thermogravimetric technique under both isothermal and non-isothermal conditions. Selected residues were examined by XRD, SEM, TEM and selected area electron diffraction (SAED). Results indicate that the dehydration process is a topotactic reaction which brings about γ-Al 2 O 3 with porous microstructure and poor crystallinity. The kinetic data were analysed using the model-free (isoconversional) methods. The results indicate that the activation energy of the dehydration process depends on the reaction extent, signifying the dehydration process is chemical controlled with a diffusion complication as the reaction extent increases. Prediction of the dehydration extent was accomplished using the kinetic parameter of the non-isothermal process and the isoconversional method. The predicted rate is in a reasonably good agreement with the measured values. Finally the mechanism of the dehydration process is discussed based on the auxiliary results from the physical examination of the reacted residues and the kinetic behaviour.

Dehydration and fluid volume kinetics before major open abdominal surgery.

Science.gov (United States)

Hahn, R G; Bahlmann, H; Nilsson, L

2014-11-01

Assessment of dehydration in the preoperative setting is of potential clinical value. The present study uses urine analysis and plasma volume kinetics, which have both been validated against induced changes in body water in volunteers, to study the incidence and severity of dehydration before open abdominal surgery begins. Thirty patients (mean age 64 years) had their urine analysed before major elective open abdominal surgery for colour, specific weight, osmolality and creatinine. The results were scored and the mean taken to represent a 'dehydration index'. Thereafter, the patients received an infusion of 5 ml/kg of Ringer's acetate intravenously for over 15 min. Blood was sampled for 70 min and the blood haemoglobin concentration used to estimate the plasma volume kinetics. Distribution of fluid occurred more slowly (P dehydrated as compared with euhydrated patients. The dehydration index indicated that the fluid deficit in these patients corresponded to 2.5% of the body weight, whereas the deficit in the others was 1%. In contrast, the 11 patients who later developed postoperative nausea and vomiting had a very short elimination half-life, only 9 min (median, P dehydration before major surgery was modest as evidenced both by urine sampling and volume kinetic analysis. © 2014 The Acta Anaesthesiologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.

Severe diarrhea-dehydration in infancy permanently alters auditory function.

Science.gov (United States)

Todd, N Wendell

2012-02-01

Of the myriad etiologies of sensorineural hearing impairment, metabolic stress is rarely considered. I posit that severe dehydration in conjunction with hypoxia, at least during infancy, prompts permanent changes in the cochlea. In a population-based prospective study of otitis media, children without otitis were found to have at age 4-8 years, worse auditory thresholds if as an infant had been hospitalized for diarrhea-dehydration. What is more, stapedius reflex thresholds tended to be lower in children who had been hospitalized for diarrhea-dehydration: that is, less acoustic energy for arousal or to be frightening. The hypothesis that the transient metabolic stress of dehydration with hypoxia prompts permanent sensorineural hearing impairment with reduced uncomfortable loudness thresholds, is both (1) consistent in an evolutionary sense with a subsequent survival advantage, and (2) subject to verification both by descriptive studies of children undergoing ECMO (ExtraCorporeal Membrane Oxygenation) or care for congenital diaphragmatic hernia, and by animal studies. Copyright © 2011 Elsevier Ltd. All rights reserved.

Epidemiology of Non-hereditary Angioedema

DEFF Research Database (Denmark)

Madsen, Flemming; Attermann, Jorn; Linneberg, Allan

2012-01-01

The prevalence of non-hereditary angioedema was investigated in a general population sample (n = 7,931) and in a sample of Danish patients (n = 7,433) tested for deficiency of functional complement C1 esterase inhibitor protein (functional C1 INH). The general population sample (44% response rate...

Comparison of clinical associations and laboratory abnormalities in children with moderate and severe dehydration.

Science.gov (United States)

Hayajneh, Wail A; Jdaitawi, Hussein; Al Shurman, Abdullah; Hayajneh, Yaseen A

2010-03-01

To search for possible early clinical associations and laboratory abnormalities in children with severe dehydration in northern Jordan. We prospectively evaluated 251 children with acute gastroenteritis. Dehydration assessment was done following a known clinical scheme. Probable clinical associations and laboratory abnormalities were examined against the preassigned dehydration status. Children with severe dehydration had significantly more hypernatremia and hyperkalemia, less isonatremia, and higher mean levels of urea, creatinine, and glucose (P dehydration. Historic clinical characteristics of patients did not correlate to dehydration degree. Serum urea, creatinine, sodium, potassium, and glucose were useful independently in augmenting clinical examination to diagnose the degree of dehydration status among children presenting with gastroenteritis. Serum urea performed the best among all. On the contrary, none of the examined historical clinical patterns could be correlated to the dehydration status. Larger and multicenter studies are needed to validate our results and to examine their impact on final outcomes.

Mathematical modeling of dehydration of 'Fuji' and 'Gala' apples slices using infrared

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Emílio de Souza Santos

2011-09-01

Full Text Available The objective of this work was to determine and model the infrared dehydration curves of apple slices - Fuji and Gala varieties. The slices were dehydrated until constant mass, in a prototype dryer with infrared heating source. The applied temperatures ranged from 50 to 100 °C. Due to the physical characteristics of the product, the dehydration curve was divided in two periods, constant and falling, separated by the critical moisture content. A linear model was used to describe the constant dehydration period. Empirical models traditionally used to model the drying behavior of agricultural products were fitted to the experimental data of the falling dehydration period. Critical moisture contents of 2.811 and 3.103 kgw kgs-1 were observed for the Fuji and Gala varieties, respectively. Based on the results, it was concluded that the constant dehydration rates presented a direct relationship with the temperature; thus, it was possible to fit a model that describes the moisture content variation in function of time and temperature. Among the tested models, which describe the falling dehydration period, the model proposed by Midilli presented the best fit for all studied conditions.

Dehydrated melon containing antioxidants and calcium from grape juice

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Hulda N. M. Chambi

2016-11-01

Full Text Available Background: Grape juice has a high antioxidant potential, capable of fighting oxidative processes in the body. The juice is mainly marketed in its concentrated form, which has a high content of glucose and fructose. The juice concentrate may then be used as an osmotic agent to dehydrated fruit with a relatively short shelf-life at room temperature, such as melon. The osmotic dehydration process can also be combined with conventional drying in order to further reduce the water activity (a w of the product. Finally, the antioxidant-rich melon meets the consumers’ demand for foods which contain ingredients that may impart health benefits. Results: Melon dehydrated by osmotic process at 200, 400 and 600 mbar, using grape juice concentrate (GJC, showed no significant differences in physical characteristics (a w , °Brix, and moisture content. Higher efficiency was observed when dehydration was performed at 200 mbar. After osmotic dehydration with GJC, both plasmolysis of the melon cells and an increase in intercellular spaces were observed by optical microscopy, with no negative impact on the mechanical properties (True stress, Hencky’s strain and deformability modulus. Calcium present in GJC was impregnated into the melon matrix, thus contributing with the mineral composition and mechanical properties of the final product. No significant differences were observed for the antioxidant capacity of melon dehydrated both with GJC and GJC followed by air-drying at 50 and 70°C. This demonstrates that it is possible to combine the two processes to obtain a product with intermediate moisture without decreasing its antioxidant capacity. The samples scored above the acceptable limit (>5 varying between like slightly to like moderately, resulting in a purchase intent with average scores between 3 (maybe/maybe not buy and 4 (probably would buy. Conclusions: A product with intermediate water activity, acidic, firm, high antioxidant capacity, rich in calcium

Organ specific proteomic dissection of Selaginella bryopteris undergoing dehydration and rehydration

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Farah eDeeba

2016-04-01

Full Text Available To explore molecular mechanisms underlying the physiological response of Selaginella bryopteris, a comprehensive proteome analysis was carried out in roots and fronds undergoing dehydration and rehydration. Plants were dehydrated for 7 days followed by 2 h and 24 h of rehydration. In roots out of 59 identified spots, 58 protein spots were found to be up-regulated during dehydration stress. The identified proteins were related to signaling, stress and defense, protein and nucleotide metabolism, carbohydrate and energy metabolism, storage and epigenetic control. Most of these proteins remained up-regulated on first rehydration, suggesting their role in recovery phase also. Among the 90 identified proteins in fronds, about 49% proteins were up-regulated during dehydration stress. Large number of ROS scavenging proteins was enhanced on dehydration. Many other proteins involved in energy, protein turnover and nucleotide metabolism, epigenetic control were also highly upregulated. Many photosynthesis related proteins were upregulated during stress. This would have helped plant to recover rapidly on rehydration. This study provides a comprehensive picture of different cellular responses elucidated by the proteome changes during dehydration and rehydration in roots and fronds as expected from a well-choreographed response from a resurrection plant.

Hospital Admissions for Malnutrition and Dehydration in Patients With Dementia.

Science.gov (United States)

Marshall, Katherine A; Burson, Rosanne; Gall, Kristyn; Saunders, Mitzi M

2016-01-01

Dehydration and malnutrition are commonly experienced by patients with dementia and can result in hospitalizations and decreased quality of life. The purpose of this study was to explore and describe retrospectively, the incidence and correlations of variables that may precede hospitalizations for dehydration/malnutrition in the community-dwelling patient with dementia. Data from the Outcome and Assessment Information Set (OASIS) Start of Care (SOC) on 44 patients served by a Michigan home care agency were retrieved for analysis. This study did not reveal any single or collection of variables that would predict risk for hospitalization for dehydration/malnutrition. With the lack of specific predictors of hospitalization related to dehydration and malnutrition, clinicians need to place high priority on risk-lowering strategies and preventive education for patients, family, and caregivers.

Thermal Dehydration Kinetics of Gypsum and Borogypsum under Non-isothermal Conditions

Institute of Scientific and Technical Information of China (English)

I.Y.Elbeyli; S.Piskin

2004-01-01

Thermal dehydration of gypsum and borogypsum was investigated under nonisothermal conditions in air by using simultaneous thermogravimetric-differential thermal analyzer. Nonisothermal experiments were carried out at various linear heating rates. Kinetics of dehydration in the temperature range of 373-503 K were evaluated from the DTA (differential thermal analysis)-TGA (thermogravimetric analysis) data by means of Coats-Redfern,Kissinger and Doyle Equations. Values of the activation energy and the pre-exponential factor of the dehydration were calculated. The results of thermal experiments and kinetic parameters indicated that borogypsum is similar to gypsum from dehydration mechanism point of view although it consists of boron and small amount of alkali metal oxides.

Genetics Home Reference: hereditary hemochromatosis

Science.gov (United States)

... Type 1 hemochromatosis results from mutations in the HFE gene, and type 2 hemochromatosis results from mutations in ... about the genes associated with hereditary hemochromatosis HAMP HFE HJV PNPLA3 SLC40A1 TFR2 Related Information What is a gene? What is a gene mutation and how do ...

Dehydration of δ-AlOOH in the lower mantle

Science.gov (United States)

Piet, H.; Shim, S. H.; Tappan, J.; Leinenweber, K. D.; Greenberg, E.; Prakapenka, V. B.

2017-12-01

Hydrous phase δ-AlOOH is an important candidate for water transport and storage in the Earth's deep mantle [1]. Knowing the conditions, under which it is stable and dehydrated, is therefore important for understanding the water transportation to the deep mantle or even to the core. A few experimental studies [1, 2] have shown that δ-AlOOH may be stable in cold descending slabs while it is dehydrated into a mixture of corundum and water under normal mantle conditions, up to 25 GPa. A subsequent study [3] reported the stability of δ-AlOOH in cold descending slabs to the core-mantle boundary conditions (2300 K at 135 GPa). However, the dehydration of δ-AlOOH has not bee directly observed in the experiments conducted at pressures above 25 GPa. We have synthesized δ-AlOOH from diaspore and Al(OH)3 in multi-anvil press at ASU. The sample was mixed with Au for coupling with near IR laser beams and loaded in diamond-anvil cells. We performed the laser-heated diamond anvil cell experiments at the 13IDD beamline of the Advanced Photon Source and ASU. At APS, we measured X-ray diffraction patterns at in situ high pressure and temperature. We observed the appearance of the corundum diffraction lines at 1700-2000 K and 55-90 GPa, indicating the dehydration of δ-AlOOH to Al2O3+ H2O. We found that the transition occurs over a broad range of temperature (500 K). We also observed that the dehydration of δ-AlOOH was accompanied by sudden change in laser coupling, most likely due to the release of fluids. The property change also helps us to determine the dehydration at ASU without in situ XRD. Our new experimental results indicate that δ-AlOOH would be stable in most subducting slabs in the deep mantle. However, because the dehydration occurs very close to the temperatures expected for the lower mantle, its stability is uncertain in the normal mantle. [1] Ohtani et al. 2001, Stability field of new hydrous phase, delta-AlOOH, Geophysical Research Letters 28, 3991-3993. [2

Hereditary pituitary hyperplasia with infantile gigantism.

Science.gov (United States)

Gläsker, Sven; Vortmeyer, Alexander O; Lafferty, Antony R A; Hofman, Paul L; Li, Jie; Weil, Robert J; Zhuang, Zhengping; Oldfield, Edward H

2011-12-01

We report hereditary pituitary hyperplasia. The objective of the study was to describe the results of the clinical and laboratory analysis of this rare instance of hereditary pituitary hyperplasia. The study is a retrospective analysis of three cases from one family. The study was conducted at the National Institutes of Health, a tertiary referral center. A mother and both her sons had very early-onset gigantism associated with high levels of serum GH and prolactin. The condition was treated by total hypophysectomy. We performed clinical, pathological, and molecular evaluations, including evaluation basal and provocative endocrine testing, neuroradiological assessment, and assessment of the pituitary tissue by microscopic evaluation, immunohistochemistry, and electron microscopy. All three family members had very early onset of gigantism associated with abnormally high serum levels of GH and prolactin. Serum GHRH levels were not elevated in either of the boys. The clinical, radiographic, surgical, and histological findings indicated mammosomatotroph hyperplasia. The pituitary gland of both boys revealed diffuse mammosomatotroph hyperplasia of the entire pituitary gland without evidence of adenoma. Prolactin and GH were secreted by the same cells within the same secretory granules. Western blot and immunohistochemistry demonstrated expression of GHRH in clusters of cells distributed throughout the hyperplastic pituitary of both boys. This hereditary condition seems to be a result of embryonic pituitary maldevelopment with retention and expansion of the mammosomatotrophs. The findings suggest that it is caused by paracrine or autocrine pituitary GHRH secretion during pituitary development.

Ibuprofen-associated acute kidney injury in dehydrated children with acute gastroenteritis.

Science.gov (United States)

Balestracci, Alejandro; Ezquer, Mauricio; Elmo, María Eugenia; Molini, Andrea; Thorel, Claudia; Torrents, Milagros; Toledo, Ismael

2015-10-01

Non-steroidal anti-inflammatory drugs (NSAIDs) induce acute kidney injury (AKI) in volume-depleted patients; however the prevalence of this complication is likely underestimated. We assessed the impact of ibuprofen exposure on renal function among dehydrated children with acute gastroenteritis (AGE) to further characterize NSAID-associated AKI. Over a 1-year period dehydrated children with AGE (n = 105) were prospectively enrolled and grouped as cases, presenting with AKI (n = 46) or controls, not presenting with AKI (n = 59). AKI was defined by pediatric RIFLE (pRIFLE) criteria. Among the children enrolled in the study, AKI prevalence was 44 %, and 34 (54 %) of the 63 patients who received ibuprofen developed renal impairment. Relative to the controls, children presenting with AKI were younger (median age 0.66 vs. 1.74 years; p dehydration, ibuprofen exposure remained an independent risk factor for AKI (p dehydrated children with AGE. Drug exposure increased the risk for developing AKI by more than twofold, independent of the magnitude of the dehydration.

Study of Trehalose Addition on Aroma Retention in Dehydrated Strawberry Puree

Directory of Open Access Journals (Sweden)

Draženka Komes

2003-01-01

Full Text Available In order to improve the quality of dehydrated fruit products, the influence of the addition of two sugars (sucrose and trehalose on the retention of aroma components during dehydration of strawberry puree was investigated. Manual headspace solid-phase microextraction (SPME, containing polydimethylsiloxane coated fibre (100 μm coupled with gas chromatography (GC-FID and GC-MS was used for the analysis of the aroma of strawberry puree dehydrated by using freeze drying and foam-mat drying. The analytes identified included esters, carbonyl compounds, terpenoids, several alcohols and acids. The results obtained in this study give further insight into the mechanisms concerning the application of trehalose as flavouring additive, due to its ability to retain and preserve the fruit volatiles responsible for the characteristic flavour of fresh fruits during dehydration processes. The best retention of aroma components in dehydrated strawberry puree was obtained by trehalose addition when combined with freeze drying.

Dehydration triggers differential microRNA expression in Xenopus laevis brain.

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Luu, Bryan E; Storey, Kenneth B

2015-11-15

African clawed frogs, Xenopus laevis, although primarily aquatic, have a high tolerance for dehydration, being capable of withstanding the loss of up to 32-35% of total water body water. Recent studies have shown that microRNAs play a role in the response to dehydration by the liver, kidney and ventral skin of X. laevis. MicroRNAs act by modulating the expression of mRNA transcripts, thereby affecting diverse biochemical pathways. In this study, 43 microRNAs were assessed in frog brains comparing control and dehydrated (31.2±0.83% of total body water lost) conditions. MicroRNAs of interest were measured using a modified protocol which employs polyadenylation of microRNAs prior to reverse transcription and qPCR. Twelve microRNAs that showed a significant decrease in expression (to 41-77% of control levels) in brains from dehydrated frogs (xla-miR-15a, -150, -181a, -191, -211, -218, -219b, -30c, -30e, -31, -34a, and -34b) were identified. Genomic analysis showed that the sequences of these dehydration-responsive microRNAs were highly conserved as compared with the comparable microRNAs of mice (91-100%). Suppression of these microRNAs implies that translation of the mRNA transcripts under their control could be enhanced in response to dehydration. Bioinformatic analysis using the DIANA miRPath program (v.2.0) predicted the top two KEGG pathways that these microRNAs collectively regulate: 1. Axon guidance, and 2. Long-term potentiation. Previous studies indicated that suppression of these microRNAs promotes neuroprotective pathways by increasing the expression of brain-derived neurotrophic factor and activating anti-apoptotic pathways. This suggests that similar actions may be triggered in X. laevis brains as a protective response to dehydration. Crown Copyright © 2015. Published by Elsevier B.V. All rights reserved.

Phase transitions on dehydration of the natural zeolite thomsonite

DEFF Research Database (Denmark)

Ståhl, Kenny

2001-01-01

The dehydration of the natural zeolite thomsonite, Na4CasAl20Si20O80 24H(2)O, has been studied using a combination of conventional and synchrotron X-ray powder diffraction. A preliminary in situ dehydration study revealed two distinct unit cell changes at approximately 540 and 570 K, respectively...

Straightforward uranium-catalyzed dehydration of primary amides to nitriles

International Nuclear Information System (INIS)

Enthaler, Stephan

2011-01-01

The efficient uranium-catalyzed dehydration of a variety of primary amides, using N-methyl-N-(trimethylsilyl)trifluoroacetamide (MSTFA) as a dehydration reagent, to the corresponding nitriles has been investigated. With this catalyst system, extraordinary catalyst activities and selectivities were feasible. (Copyright copyright 2011 WILEY-VCH Verlag GmbH and Co. KGaA, Weinheim)

Immunophenotyping of hereditary breast cancer

NARCIS (Netherlands)

van der Groep, P.

2009-01-01

Hereditary breast cancer runs in families where several family members in different generations are affected. Most of these breast cancers are caused by mutations in the high penetrance genes BRCA1 and BRCA2 which account for about 5% of all breast cancers. However, mutations in BRCA1 and BRCA2 may

Epidemiology of Non-hereditary Angioedema

DEFF Research Database (Denmark)

Madsen, Flemming; Attermann, Jørn; Linneberg, Allan

2012-01-01

The prevalence of non-hereditary angioedema was investigated in a general population sample (n¿=¿7,931) and in a sample of Danish patients (n¿=¿7,433) tested for deficiency of functional complement C1 esterase inhibitor protein (functional C1 INH). The general population sample (44% response rate...

The metabolomic signature of Leber's hereditary optic neuropathy reveals endoplasmic reticulum stress.

Science.gov (United States)

Chao de la Barca, Juan Manuel; Simard, Gilles; Amati-Bonneau, Patrizia; Safiedeen, Zainab; Prunier-Mirebeau, Delphine; Chupin, Stéphanie; Gadras, Cédric; Tessier, Lydie; Gueguen, Naïg; Chevrollier, Arnaud; Desquiret-Dumas, Valérie; Ferré, Marc; Bris, Céline; Kouassi Nzoughet, Judith; Bocca, Cinzia; Leruez, Stéphanie; Verny, Christophe; Miléa, Dan; Bonneau, Dominique; Lenaers, Guy; Martinez, M Carmen; Procaccio, Vincent; Reynier, Pascal

2016-11-01

Leber's hereditary optic neuropathy (MIM#535000), the commonest mitochondrial DNA-related disease, is caused by mutations affecting mitochondrial complex I. The clinical expression of the disorder, usually occurring in young adults, is typically characterized by subacute, usually sequential, bilateral visual loss, resulting from the degeneration of retinal ganglion cells. As the precise action of mitochondrial DNA mutations on the overall cell metabolism in Leber's hereditary optic neuropathy is unknown, we investigated the metabolomic profile of the disease. High performance liquid chromatography coupled with tandem mass spectrometry was used to quantify 188 metabolites in fibroblasts from 16 patients with Leber's hereditary optic neuropathy and eight healthy control subjects. Latent variable-based statistical methods were used to identify discriminating metabolites. One hundred and twenty-four of the metabolites were considered to be accurately quantified. A supervised orthogonal partial least squares discriminant analysis model separating patients with Leber's hereditary optic neuropathy from control subjects showed good predictive capability (Q 2cumulated = 0.57). Thirty-eight metabolites appeared to be the most significant variables, defining a Leber's hereditary optic neuropathy metabolic signature that revealed decreased concentrations of all proteinogenic amino acids, spermidine, putrescine, isovaleryl-carnitine, propionyl-carnitine and five sphingomyelin species, together with increased concentrations of 10 phosphatidylcholine species. This signature was not reproduced by the inhibition of complex I with rotenone or piericidin A in control fibroblasts. The importance of sphingomyelins and phosphatidylcholines in the Leber's hereditary optic neuropathy signature, together with the decreased amino acid pool, suggested an involvement of the endoplasmic reticulum. This was confirmed by the significantly increased phosphorylation of PERK and eIF2α, as well as

Proteome analysis of Physcomitrella patens exposed to progressive dehydration and rehydration

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Cui, Suxia; Hu, Jia; Guo, Shilei; Wang, Jie; Cheng, Yali; Dang, Xinxing; Wu, Lili; He, Yikun

2012-01-01

Physcomitrella patens is an extremely dehydration-tolerant moss. However, the molecular basis of its responses to loss of cellular water remains unclear. A comprehensive proteomic analysis of dehydration- and rehydration-responsive proteins has been conducted using quantitative two-dimensional difference in-gel electrophoresis (2D-DIGE), and traditional 2-D gel electrophoresis (2-DE) combined with MALDI TOF/TOF MS. Of the 216 differentially-expressed protein spots, 112 and 104 were dehydration- and rehydration-responsive proteins, respectively. The functional categories of the most differentially-expressed proteins were seed maturation, defence, protein synthesis and quality control, and energy production. Strikingly, most of the late embryogenesis abundant (LEA) proteins were expressed at a basal level under control conditions and their synthesis was strongly enhanced by dehydration, a pattern that was confirmed by RT-PCR. Actinoporins, phosphatidylethanolamine-binding protein, arabinogalactan protein, and phospholipase are the likely dominant players in the defence system. In addition, 24 proteins of unknown function were identified as novel dehydration- or rehydration-responsive proteins. Our data indicate that Physcomitrella adopts a rapid protein response mechanism to cope with dehydration in its leafy-shoot and basal expression levels of desiccation-tolerant proteins are rapidly upgraded at high levels under stress. This mechanism appears similar to that seen in angiosperm seeds. PMID:21994173

Hereditary rickets. How genetic alterations explain the biochemical and clinical phenotypes.

Science.gov (United States)

Papadopoulou, Anna; Gole, Evaggelia; Nicolaidou, Polyxeni

2013-12-01

The reemergence of vitamin D deficiency in the industrialized countries resurrects the "threat" of nutritional rickets, especially among pediatric populations, a fact that may lead to underdiagnosis of hereditary rickets. Today, hereditary rickets may be subdivided into two main groups according to their biochemical profile: the one associated with defects in vitamin D synthesis and action and the second associated with abnormal phosphorus metabolism. The classification of the patients in a particular group of hereditary rickets is determinative of the treatment to follow. This review, through the recent advances on vitamin D and P metabolism, discusses the molecular and biochemical defects associated to each group of inherited rickets, as well as the clinical phenotypes and the recommended therapeutic approaches.

Economic burden associated with hospital postadmission dehydration.

Science.gov (United States)

Pash, Elizabeth; Parikh, Niraj; Hashemi, Lobat

2014-11-01

Development of dehydration after hospital admission can be a measure of quality care, but evidence describing the incidence, economic burden, and outcomes of dehydration in hospitalized patients is lacking. The objective of this study was to compare costs and resource utilization of U.S. patients experiencing postadmission dehydration (PAD) with those who do not in a hospital setting. All adult inpatient discharges, excluding those with suspected dehydration present on admission (International Classification of Diseases, Ninth Revision, Clinical Modification [ICD-9-CM] codes for dehydration: 276.0, 276.1, 276.5), were identified from the Premier database using ICD-9-CM codes. PAD and no-PAD (NPAD) groups were matched on propensity score adjusting for demographics (age, sex, race, medical, elective patients), patient severity (All Patient Refined Diagnosis-Related Groups severity scores), and hospital characteristics (geographic location, bed size, teaching and urban hospital). Costs, length of stay (LOS), and incidence of mortality and catheter-associated urinary tract infection (CAUTI) were compared between groups using the t test for continuous variables and the χ(2) test for categorical variables. In total, 86,398 (2.1%) of all the selected patients experienced PAD. Postmatching mean total costs were significantly higher for the PAD group compared with the NPAD group ($33,945 vs $22,380; P < .0001). Departmental costs were also significantly higher for the PAD group (all P < .0001). Compared with the NPAD group, the PAD group had a higher mean LOS (12.9 vs 8.2 days), a higher incidence of CAUTI (0.6% vs 0.5%), and higher in-hospital mortality (8.6% vs 7.8%) (all P < .05). The results for subgroup analysis also showed significantly higher total cost and longer LOS days for patients with PAD (all P < .05). The economic burden associated with hospital PAD in medical and surgical patients was substantial. © 2014 American Society for Parenteral and Enteral

Quantitative Study for the Surface Dehydration of Vocal Folds Based on High-Speed Imaging.

Science.gov (United States)

Li, Lin; Zhang, Yu; Maytag, Allison L; Jiang, Jack J

2015-07-01

From the perspective of the glottal area and mucosal wave, quantitatively estimate the differences of vocal fold on laryngeal activity during phonation at three different dehydration levels. Controlled three sets of tests. A dehydration experiment for 10 excised canine larynges was conducted at 16 cm H2O. According to the dehydration cycle time (H), dehydration levels were divided into three degrees (0% H, 50% H, 75% H). The glottal area and mucosal wave under three dehydration levels were extracted from high-speed images and digital videokymography (DKG) image sequences. Direct and non-direct amplitude components were derived from glottal areas. The amplitude and frequency of mucosal wave were calculated from DKG image sequences. These parameters in condition of three dehydration levels were compared for statistical analysis. The results showed a significant difference in direct (P = 0.001; P = 0.005) and non-direct (P = 0.005; P = 0.016) components of glottal areas between every two different dehydration levels. Considering the right-upper, right-lower, left-upper, and left-lower of vocal fold, the amplitudes of mucosal waves consistently decreased with increasing of dehydration levels. But, there was no significant difference in frequency. Surface dehydration could give rise to complex variation of vocal fold on tissues and vibratory mechanism, which should need analyzing from multiple perspectives. The results suggested that the combination of glottal area and mucosal wave could be better to research the change of vocal fold at different dehydrations. It would become a better crucial research tool for the clinical treatment of dehydration-induced laryngeal pathologies. Copyright © 2015 The Voice Foundation. Published by Elsevier Inc. All rights reserved.

Hereditary noetherian prime rings and idealizers

CERN Document Server

Levy, Lawrence S

2011-01-01

The direct sum behaviour of its projective modules is a fundamental property of any ring. Hereditary Noetherian prime rings are perhaps the only noncommutative Noetherian rings for which this direct sum behaviour (for both finitely and infinitely generated projective modules) is well-understood, yet highly nontrivial. This book surveys material previously available only in the research literature. It provides a re-worked and simplified account, with improved clarity, fresh insights and many original results about finite length modules, injective modules and projective modules. It culminates in the authors' surprisingly complete structure theorem for projective modules which involves two independent additive invariants: genus and Steinitz class. Several applications demonstrate its utility. The theory, extending the well-known module theory of commutative Dedekind domains and of hereditary orders, develops via a detailed study of simple modules. This relies upon the substantial account of idealizer subrings wh...

Hereditary Ovarian Cancer: Not Only BRCA 1 and 2 Genes

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Angela Toss

2015-01-01

Full Text Available More than one-fifth of ovarian tumors have hereditary susceptibility and, in about 65–85% of these cases, the genetic abnormality is a germline mutation in BRCA genes. Nevertheless, several other suppressor genes and oncogenes have been associated with hereditary ovarian cancers, including the mismatch repair (MMR genes in Lynch syndrome, the tumor suppressor gene, TP53, in the Li-Fraumeni syndrome, and several other genes involved in the double-strand breaks repair system, such as CHEK2, RAD51, BRIP1, and PALB2. The study of genetic discriminators and deregulated pathways involved in hereditary ovarian syndromes is relevant for the future development of molecular diagnostic strategies and targeted therapeutic approaches. The recent development and implementation of next-generation sequencing technologies have provided the opportunity to simultaneously analyze multiple cancer susceptibility genes, reduce the delay and costs, and optimize the molecular diagnosis of hereditary tumors. Particularly, the identification of mutations in ovarian cancer susceptibility genes in healthy women may result in a more personalized cancer risk management with tailored clinical and radiological surveillance, chemopreventive approaches, and/or prophylactic surgeries. On the other hand, for ovarian cancer patients, the identification of mutations may provide potential targets for biologic agents and guide treatment decision-making.

Could Neonatal Hypernatremia Dehydration Influence Hearing Status?

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Hassan Boskabadi

2014-01-01

Full Text Available Introduction: Neonatal hypernatremia dehydration (NHD is a dangerous condition in neonates, which is accompanied by acute complications (renal failure, cerebral edema, and cerebral hemorrhage and chronic complications (developmental delay. Children begin learning language from birth, and hearing impairment interferes with this process. We assessed the hearing status of infants with hypernatremia dehydration.   Materials and Methods: In a case-control study in 110 infants presenting at the Ghaem Hospital (Mashhad, Iran between 2007 and 2011, we examined the incidence of hearing impairment in infants suffering from hypernatremia dehydration (serum sodium >150 mEq/L in comparison with infants with normal sodium level (serum sodium ≤150 mEq/L.   Results: Three of 110 cases examined in the study group showed a transient hearing impairment. A mean serum sodium level of 173mg/dl was reported among hearing-impaired infants.   Conclusion:  Transient hearing impairment was higher in infants with hypernatremia; although this difference was not significant (P>0.05. Hearing impairment was observed in cases of severe hypernatremia.  

Quality of frozen fruit bars manufactured through infrared pre-dehydration

Science.gov (United States)

In this study, frozen restructured whole apple and strawberry bars were manufactured by partial dehydration, using infrared (IR) heating, followed by restructuring and freezing. The objective of this investigation was to determine the effect of IR partial dehydration on the quality of restructured f...

Effects of tissue fixation and dehydration on tendon collagen nanostructure.

Science.gov (United States)

Turunen, Mikael J; Khayyeri, Hanifeh; Guizar-Sicairos, Manuel; Isaksson, Hanna

2017-09-01

Collagen is the most prominent protein in biological tissues. Tissue fixation is often required for preservation or sectioning of the tissue. This may affect collagen nanostructure and potentially provide incorrect information when analyzed after fixation. We aimed to unravel the effect of 1) ethanol and formalin fixation and 2) 24h air-dehydration on the organization and structure of collagen fibers at the nano-scale using small and wide angle X-ray scattering. Samples were divided into 4 groups: ethanol fixed, formalin fixed, and two untreated sample groups. Samples were allowed to air-dehydrate in handmade Kapton pockets during the measurements (24h) except for one untreated group. Ethanol fixation affected the collagen organization and nanostructure substantially and during 24h of dehydration dramatic changes were evident. Formalin fixation had minor effects on the collagen organization but after 12h of air-dehydration the spatial variation increased substantially, not evident in the untreated samples. Generally, collagen shrinkage and loss of alignment was evident in all samples during 24h of dehydration but the changes were subtle in all groups except the ethanol fixed samples. This study shows that tissue fixation needs to be chosen carefully in order to preserve the features of interest in the tissue. Copyright © 2017 Elsevier Inc. All rights reserved.

Preoperative dehydration increases risk of postoperative acute renal failure in colon and rectal surgery.

Science.gov (United States)

Moghadamyeghaneh, Zhobin; Phelan, Michael J; Carmichael, Joseph C; Mills, Steven D; Pigazzi, Alessio; Nguyen, Ninh T; Stamos, Michael J

2014-12-01

There is limited data regarding the effects of preoperative dehydration on postoperative renal function. We sought to identify associations between hydration status before operation and postoperative acute renal failure (ARF) in patients undergoing colorectal resection. The NSQIP database was used to examine the data of patients undergoing colorectal resection from 2005 to 2011. We used preoperative blood urea nitrogen (BUN)/creatinine ratio >20 as a marker of relative dehydration. Multivariate analysis using logistic regression was performed to quantify the association of BUN/Cr ratio with ARF. We sampled 27,860 patients who underwent colorectal resection. Patients with dehydration had higher risk of ARF compared to patients with BUN/Cr Dehydration was associated with an increase in mortality of the affected patients (AOR, 2.19; P dehydrated patients. Open colorectal procedures (AOR, 2.67; P = 0.01) and total colectomy procedure (AOR, 1.62; P Dehydration before operation is a common condition in colorectal surgery (incidence of 27.7 %). Preoperative dehydration is associated with increased rates of postoperative ARF, MI, and cardiac arrest. Hydrotherapy of patients with dehydration may decrease postoperative complications in colorectal surgery.

WATER TEMPERATURE, VOLUNTARY DRINKING AND FLUID BALANCE IN DEHYDRATED TAEKWONDO ATHLETES

Directory of Open Access Journals (Sweden)

Saeed Khamnei

2011-12-01

Full Text Available Voluntary drinking is one of the major determiners of rehydration, especially as regards exercise or workout in the heat. The present study undertakes to search for the effect of voluntary intake of water with different temperatures on fluid balance in Taekwondo athletes. Six young healthy male Taekwondo athletes were dehydrated by moderate exercise in a chamber with ambient temperature at 38-40°C and relative humidity between 20-30%. On four separate days they were allowed to drink ad libitum plane water with the four temperatures of 5, 16, 26, and 58°C, after dehydration. The volume of voluntary drinking and weight change was measured; then the primary percentage of dehydration, sweat loss, fluid deficit and involuntary dehydration were calculated. Voluntary drinking of water proved to be statistically different in the presented temperatures. Water at 16°C involved the greatest intake, while fluid deficit and involuntary dehydration were the lowest. Intake of water in the 5°C trial significantly correlated with the subject's plasma osmolality change after dehydration, yet it showed no significant correlation with weight loss. In conclusion, by way of achieving more voluntary intake of water and better fluid state, recommending cool water (~16°C for athletes is in order. Unlike the publicly held view, drinking cold water (~5°C does not improve voluntary drinking and hydration status.

RANCANG BANGUN ALAT DEHYDRATOR BIOETANOL UNTUK MENGHASILKAN FUEL GRADE ETHANOL (FGE

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Rochmad Winarso

2015-11-01

Full Text Available ABSTRAK Bioethanol merupakan salah satu bahan bakar alternatif dengan sumber bahan baru yang dapat diperbarui. Bioethanol dapat menjadi bahan bakar alternatif bila mempunyai konsentrasi lebih dari 99% yang dikenal dengan nama Fuel Grade Ethanol. Proses pembuatan Fuel Grade Ethanol menggunakan metode pemisahan lanjut diantaranya adalah dengan metode distilasi azeotrop, pervorasi membran, dan adsorbsi. Tujuan dari penelitian ini adalah melakukan pengujian mesin dehydrator bioethanol yang bekerja dengan metode absorbsi. Mesin dehydrator ini menggunakan zeolit syntetis dengan ukuran 3 A yang sebelumnya telah dikembangkan. Pengembangan ini dilakukan melalui tiga tahapan, yaitu: (1 Tahap perancangan (desain alat dehydrator biorthanol; (2 Tahap pembuatan alat dehydrator berdasarkan spesifikasi yang telah ditetapkan ; (3 Pengujian alat dehydrator yang berorientasi hasil yaitu bioetanol minimal berkadar sekitar 99%. Mesin destilator bioetanol yang telah dikembangkan mempunya spesifikasi sebagai berikut: dimensi tangki bahan baku tingginya adalah 250 mm dengan diameter 300 mm, Bagian tabung I terbuat dari pipa stainless steel dengan diameter 100 mm dan tinggi 600 mm. Tebal dari pipa tersebut adalah 2 mm. Tabung II terbuat dari stainless steel yang mempunyai diamater 100 mm dan tinggi 300 mm dengan ketebalan 2 mm. Kondensor berdiameter 100 mm dan tinggi 600 mm. Hasil penelitian ini menunjukkan bahwa mesin yang sudah dikembangkan ini mampu menghasilkan Fuel Grade Ethanol dengan kadar lebih dari 99%. Kata kunci: dehydrator, bioethanol, fuel grade ethanol, bahan bakar alternatif.

MRI in Leber's hereditary optic neuropathy

DEFF Research Database (Denmark)

Matthews, Lucy; Enzinger, Christian; Fazekas, Franz

2015-01-01

BACKGROUND: Leber's hereditary optic neuropathy (LHON) and a multiple sclerosis (MS)-like illness appear to coexist 50 times more frequently than would be expected by chance. This association of LHON and MS (LMS) raises an important question about whether there could be a common pathophysiological...

Hereditary Gigantism-the biblical giant Goliath and his brothers.

Science.gov (United States)

Donnelly, Deirdre E; Morrison, Patrick J

2014-05-01

The biblical giant Goliath has an identifiable family tree suggestive of autosomal dominant inheritance. We suggest that he had a hereditary pituitary disorder possibly due to the AIP gene, causing early onset and familial acromegaly or gigantism. We comment on the evidence within the scriptures for his other relatives including a relative with six digits and speculate on possible causes of the six digits. Recognition of a hereditary pituitary disorder in the biblical Goliath and his family sheds additional information on his and other family members' battles with David and his relatives.

(1)H nuclear magnetic resonance (NMR) as a tool to measure dehydration in mice.

Science.gov (United States)

Li, Matthew; Vassiliou, Christophoros C; Colucci, Lina A; Cima, Michael J

2015-08-01

Dehydration is a prevalent pathology, where loss of bodily water can result in variable symptoms. Symptoms can range from simple thirst to dire scenarios involving loss of consciousness. Clinical methods exist that assess dehydration from qualitative weight changes to more quantitative osmolality measurements. These methods are imprecise, invasive, and/or easily confounded, despite being practiced clinically. We investigate a non-invasive, non-imaging (1)H NMR method of assessing dehydration that attempts to address issues with existing clinical methods. Dehydration was achieved by exposing mice (n = 16) to a thermally elevated environment (37 °C) for up to 7.5 h (0.11-13% weight loss). Whole body NMR measurements were made using a Bruker LF50 BCA-Analyzer before and after dehydration. Physical lean tissue, adipose, and free water compartment approximations had NMR values extracted from relaxation data through a multi-exponential fitting method. Changes in before/after NMR values were compared with clinically practiced metrics of weight loss (percent dehydration) as well as blood and urine osmolality. A linear correlation between tissue relaxometry and both animal percent dehydration and urine osmolality was observed in lean tissue, but not adipose or free fluids. Calculated R(2) values for percent dehydration were 0.8619 (lean, P dehydration in live animals. Copyright © 2015 John Wiley & Sons, Ltd.

Time-resolved x-ray diffraction analysis of the experimental dehydration of serpentine at high pressure

International Nuclear Information System (INIS)

Inoue, Toru; Yoshimi, Isamu; Yamada, Akihiro; Kikegawa, Takumi

2009-01-01

Time-resolved, in situ X-ray diffraction analysis was used to determine the dehydration rate and kinetics of serpentine during experimental dehydration at high pressures. The capsule used comprises a diamond sleeve fitted with Au or Pt lids in order to provide high-quality, time-resolved X-ray diffraction data. Antigorite quickly dehydrated to enstatite + forsterite + fluid within 2 h at 650degC below ∼6 GPa. Avrami modeling of the results and SEM observations of the partially dehydrated sample revealed that the nucleation rate was quite high for enstatite but low for forsterite, showing incubation periods of ∼10 min before appearing. The crystallization of these minerals is controlled largely by the composition of the fluid generated from serpentine dehydration. The dehydration boundary determined below 6 GPa in the present study is consistent with the results of previous phase equilibrium studies. This study indicates that serpentine in a subducting slab dehydrates rapidly below 6 GPa when the slab intersects the dehydration boundary conditions. (author)

Descriptive Epidemiology, Molecular Biology and Genetics of Hereditary Prostate Cancer in Denmark

DEFF Research Database (Denmark)

Bentzon, Diem Nguyen

2012-01-01

A search for markers that can differentiate indolent prostate cancers from more aggressive forms. Assessment of clinical differences between hereditary and sporadicc prostate cancer.......A search for markers that can differentiate indolent prostate cancers from more aggressive forms. Assessment of clinical differences between hereditary and sporadicc prostate cancer....

Association Between Dehydration and Fever During the First Week of Life.

Science.gov (United States)

Boutin, Ariane; Carceller, Ana; Desjardins, Marie Pier; Sanchez, Marisol; Gravel, Jocelyn

2017-12-01

Trying to differentiate serious bacterial infection (SBI) from a self-limiting illness in febrile infants seen in the pediatric emergency department (PED) is a significant challenge. The aim of the study was to determine the prevalence of dehydration and its relationship to SBI in febrile full-term newborns under 1 week of age seen in a PED. A retrospective observational study was carried out on all children younger than 8 days of age with fever who presented to a single, tertiary care, PED from January 2009 to April 2014. Dehydration was defined as plasma sodium >150 mmol/L or >10% loss of birth weight. SBI was defined by the presence of a positive culture in the blood, urine, cerebrospinal fluid; osteoarticular infection; bacterial enteritis; or pneumonia. The primary analysis was the proportion of children with dehydration. A secondary analysis compared proportion of infection according to hydration status. Of the 895 children under 8 days of age who visited the PED, 69 consulted for fever. Seven patients were excluded because they were transferred from another hospital. Sixty-two eligible patients were included in the final analysis. Of these, 17 (27%) were dehydrated according to our definition. Only 2 patients had an SBI while 2 others had a final diagnosis of viral myocarditis and encephalitis, respectively. None of the 4 children with serious infection fulfilled our definition of dehydration, and all had a plasma sodium level lower than 145 mmol/L. Dehydration is frequently associated with fever in infants younger than 8 days of age seen in a PED. Early identification of dehydration may be useful in limiting the aggressive intervention in some of these infants.

Studying thermal dehydration of double nickel alkali metal pyrophosphates

International Nuclear Information System (INIS)

Bykanova, T.A.; Lavrov, A.V.; AN SSSR, Moscow. Inst. Obshchej i Neorganicheskoj Khimii)

1978-01-01

The methods of thermogravimetry, paper chromatography, infrared spectroscopy and X-ray phase analysis were used in studying the process of thermal dehydration of pyrophosphates of the M 2 Ni 3 (P 2 O 7 ) 2 xnH 2 O type (where n=6, 10; M=Na, K, Rb, Cs, NH 4 ). The dehydration of Cs 2 Ni 3 (P 2 O 7 ) 2 x10H 2 O proceeds in a single stage (endothermal effect at 210 deg C). The exothermal effects at 730 and 690 deg C correspond to the crystallization of the amorphous dehydration products. It has been established that binary pyrophosphates of nickel with alkali metals decompose when heated into Ni 3 (PO 4 ) 2 +MPO 4

Dehydration-responsive miRNAs in foxtail millet: genome-wide identification, characterization and expression profiling.

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Yadav, Amita; Khan, Yusuf; Prasad, Manoj

2016-03-01

A set of novel and known dehydration-responsive miRNAs have been identified in foxtail millet. These findings provide new insights into understanding the functional role of miRNAs and their respective targets in regulating plant response to dehydration stress. MicroRNAs perform significant regulatory roles in growth, development and stress response of plants. Though the miRNA-mediated gene regulatory networks under dehydration stress remain largely unexplored in plant including foxtail millet (Setaria italica), which is a natural abiotic stress tolerant crop. To find out the dehydration-responsive miRNAs at the global level, four small RNA libraries were constructed from control and dehydration stress treated seedlings of two foxtail millet cultivars showing contrasting tolerance behavior towards dehydration stress. Using Illumina sequencing technology, 55 known and 136 novel miRNAs were identified, representing 22 and 48 miRNA families, respectively. Eighteen known and 33 novel miRNAs were differentially expressed during dehydration stress. After the stress treatment, 32 dehydration-responsive miRNAs were up-regulated in tolerant cultivar and 22 miRNAs were down-regulated in sensitive cultivar, suggesting that miRNA-mediated molecular regulation might play important roles in providing contrasting characteristics to these cultivars. Predicted targets of identified miRNAs were found to encode various transcription factors and functional enzymes, indicating their involvement in broad spectrum regulatory functions and biological processes. Further, differential expression patterns of seven known miRNAs were validated by northern blot and expression of ten novel dehydration-responsive miRNAs were confirmed by SL-qRT PCR. Differential expression behavior of five miRNA-target genes was verified under dehydration stress treatment and two of them also validated by RLM RACE. Overall, the present study highlights the importance of dehydration stress-associated post

Thermal dehydration kinetics of phosphogypsum

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López, F. A.

2015-09-01

Full Text Available Phsophogypsum is a by-product from the processing phosphate rock. Before the use of it in cement industry such as setting regulator is necessary a study of dehydration reaction of phosphogypsum to avoid the false setting during the milling. The aim is to study the thermal behavior of two different phosphogypsum sources (Spain and Tunisia under non-isothermal conditions in argon atmosphere by using Thermo-Gravimetriy, Differential Thermal Analysis (TG-DTA and Differential Scanning Calorimetry (DSC. DSC experiments were carried out at temperatures ranging from ambient to 350 °C at different heating rates. The temperatures of conversion from gypsum to hemihydrate and anhydrite states and heat of dehydration were determined. Various methods were used to analyze the DSC data for reaction kinetics determination. The activation energy and frequency factor were calculated for dehydration of phosphogypsum. Activation energy values of the main dehydration reaction of phosphogypsum were calculated to be approximately 61–118 kJ/mol.El fosfoyeso es un subproducto procedente del procesado de la roca fosfato. Una de las posibles vías de reutilización y revalorización es su uso como regulador del fraguado en la industria cementera. Debido a los posibles problemas de falso fraguado asociado a los procesos de deshidratación que tienen lugar durante la molienda del cemento, esta investigación estudió el comportamiento térmico, bajo condiciones no-isotérmicas en atmósfera de argón, de dos fosfoyesos, mediante TG-DTA y DSC. Los ensayos de DSC se realizaron hasta los 350 °C a diferentes velocidades de calentamiento. La temperatura de conversión del yeso a las formas de hemihidrato y anhidrita y el calor de hidratación fueron determinados. Las cinéticas de reacción fueron obtenidas analizando los datos de DSC mediante varios métodos. Se calculó la energía de activación y el factor de frecuencia para las reacciones de deshidratación del

2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema

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Bowen Tom

2010-07-01

Full Text Available Abstract Background We published the Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema (HAE; C1 inhibitor [C1-INH] deficiency and updated this as Hereditary angioedema: a current state-of-the-art review: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema. Objective To update the International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema (circa 2010. Methods The Canadian Hereditary Angioedema Network (CHAEN/Réseau Canadien d'angioédème héréditaire (RCAH http://www.haecanada.com and cosponsors University of Calgary and the Canadian Society of Allergy and Clinical Immunology (with an unrestricted educational grant from CSL Behring held our third Conference May 15th to 16th, 2010 in Toronto Canada to update our consensus approach. The Consensus document was reviewed at the meeting and then circulated for review. Results This manuscript is the 2010 International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema that resulted from that conference. Conclusions Consensus approach is only an interim guide to a complex disorder such as HAE and should be replaced as soon as possible with large phase III and IV clinical trials, meta analyses, and using data base registry validation of approaches including quality of life and cost benefit analyses, followed by large head-to-head clinical trials and then evidence-based guidelines and standards for HAE disease management.

Value of point-of-care ketones in assessing dehydration and acidosis in children with gastroenteritis.

Science.gov (United States)

Levy, Jason A; Waltzman, Mark; Monuteaux, Michael C; Bachur, Richard G

2013-11-01

Children with gastroenteritis often develop dehydration with metabolic acidosis. Serum ketones are frequently elevated in this population. The goal was to determine the relationship between initial serum ketone concentration and both the degree of dehydration and the magnitude of acidosis. This was a secondary analysis of a prospective trial of crystalloid administration for rapid rehydration. Children 6 months to 6 years of age with gastroenteritis and dehydration were enrolled. A point-of-care serum ketone (beta-hydroxybutyrate) concentration was obtained at the time of study enrollment. The relationship between initial serum ketone concentration and a prospectively assigned and previously validated clinical dehydration score, and serum bicarbonate concentration, was analyzed. A total of 188 patients were enrolled. The median serum ketone concentration was elevated at 3.1 mmol/L (interquartile range [IQR] = 1.2 to 4.6 mmol/L), and the median dehydration score was consistent with moderate dehydration. A significant positive relationship was found between serum ketone concentration and the clinical dehydration score (Spearman's rho = 0.22, p = 0.003). Patients with moderate dehydration had a higher median serum ketone concentration than those with mild dehydration (3.6 mmol/L vs. 1.4 mmol/L, p = 0.007). Additionally, the serum ketone concentration was inversely correlated with serum bicarbonate concentration (ρ = -0.26, p Children with gastroenteritis and dehydration have elevated serum ketone concentrations that correlate with both degree of dehydration and magnitude of metabolic acidosis. Point-of-care serum ketone measurement may be a useful tool to inform management decisions at the point of triage or in the initial evaluation of children with gastroenteritis and dehydration. © 2013 by the Society for Academic Emergency Medicine.

Genetics 101 --The Hereditary Material of Life

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... of this page please turn Javascript on. Feature: Genetics 101 Genetics 101 — The Hereditary Material of Life Past Issues / Summer 2013 Table of Contents Genetics is the study of heredity, the process in ...

External validation of the clinical dehydration scale for children with acute gastroenteritis.

Science.gov (United States)

Bailey, Benoit; Gravel, Jocelyn; Goldman, Ran D; Friedman, Jeremy N; Parkin, Patricia C

2010-06-01

The objective was to validate the clinical dehydration scale (CDS) for children with gastroenteritis in a different pediatric emergency department (ED) from where it was initially derived and validated. A prospective cohort study was performed in a tertiary care pediatric ED over a 1-year period. A sample of triage nurses were trained in applying the CDS. The CDS consists of four clinical characteristics (general appearance, eyes, mucous membranes, and tears), each of which are scored 0, 1, or 2 for a total score of 0 to 8, with 0 representing no dehydration; 1 to 4, some dehydration; and 5 to 8, moderate/severe dehydration. Children 1 month to 5 years of age with vomiting and/or diarrhea who had the CDS documented at triage and a final diagnosis of gastroenteritis, gastritis, or enteritis were enrolled. Exclusion criteria included a chronic disease, treatment with intravenous (IV) rehydration within the previous 24 hours, visit to the ED for the same illness in the 7 days prior to arrival, and diarrhea of more than 10 days' duration. The primary outcome was the length of stay (LOS) in the ED from the time of seeing a physician to discharge, analyzed with a Kruskal-Wallis test. From April 2008 to March 2009, 150 patients with a mean (+/-SD) age of 22 (+/-14) months (range = 4 months to 4 years) were enrolled. Fifty-six patients had no dehydration, 74 had some dehydration, and 20 had moderate/severe dehydration. The median LOS in the ED after being seen by a physician was significantly longer as children appeared more dehydrated according to the CDS: 54 minutes (interquartile range [IQR] = 26-175 minutes), 128 minutes (IQR = 25-334 minutes), and 425 minutes (IQR = 218-673 minutes) for the no, some, and moderate/severe dehydration groups, respectively (p children with gastroenteritis in a different pediatric center than the original one where it was developed. It is a good predictor of LOS in the ED after being seen by a physician. (c) 2010 by the Society for

Cancer risk in families with hereditary nonpolyposis colorectal cancer diagnosed by mutation analysis

NARCIS (Netherlands)

Vasen, H. F.; Wijnen, J. T.; Menko, F. H.; Kleibeuker, J. H.; Taal, B. G.; Griffioen, G.; Nagengast, F. M.; Meijers-Heijboer, E. H.; Bertario, L.; Varesco, L.; Bisgaard, M. L.; Mohr, J.; Fodde, R.; Khan, P. M.

1996-01-01

Hereditary nonpolyposis colorectal cancer is characterized by early-onset colorectal cancer and the occurrence of various other cancers. The recent isolation of four mismatch repair genes responsible for hereditary nonpolyposis colorectal cancer allows for the identification of carriers within

Cancer risk in families with hereditary nonpolyposis colorectal cancer diagnosed by mutation analysis

NARCIS (Netherlands)

Vasen, HFA; Wijnen, JT; Menko, FH; Kleibeuker, JH; Taal, BG; Griffioen, G; Nagengast, FM; MeijersHeijboer, EH; Bertario, L; Varesco, L; Bisgaard, ML; Mohr, J; Fodde, R; Khan, PM

Background & Aims: Hereditary nonpolyposis colorectal cancer is characterized by early-onset colorectal cancer and the occurrence of various other cancers, The recent isolation of four mismatch repair genes responsible for hereditary nonpolyposis colorectal cancer allows for the identification of

The Usefulness of Clinical and Laboratory Parameters for Predicting Severity of Dehydration in Children with Acute Gastroenteritis

Science.gov (United States)

Hoxha, Teuta Faik; Azemi, Mehmedali; Avdiu, Muharrem; Ismaili-jaha, Vlora; Grajqevci, Violeta; Petrela, Ela

2014-01-01

ABSTRACT Background: An accurate assessment of the degree of dehydration in infants and children is important for proper decision-making and treatment. This emphasizes the need for laboratory tests to improve the accuracy of clinical assessment of dehydration. The aim of this study was to assess the relationship between clinical and laboratory parameters in the assessment of dehydration. Methods: We evaluated prospectively 200 children aged 1 month to 5 years who presented with diarrhea, vomiting or both. Dehydration assessment was done following a known clinical scheme. Results: We enrolled in the study 200 children (57.5% were male). The mean age was 15.62±9.03 months, with more than half those studied being under 24 months old. Overall, 46.5% (93) had mild dehydration, 34% (68) had moderate dehydration, 5.5% (11) had severe dehydration whereas, 14% (28) had no dehydration. Patients historical clinical variables in all dehydration groups did not differ significantly regarding age, sex, fever, frequency of vomiting, duration of diarrhea and vomiting, while there was a trend toward severe dehydration in children with more frequent diarrhea (p=0.004). Serum urea and creatinine cannot discriminate between mild and moderate dehydration but they showed a good specificity for severe dehydration of 99% and 100% respectively. Serum bicarbonates and base excess decreased significantly with a degree of dehydration and can discriminate between all dehydration groups (P<0.001). Conclusion: Blood gases were useful to diagnose the degree of dehydration status among children presenting with acute gastroenteritis. Serum urea and creatinine were the most specific tests for severe dehydration diagnosis. Historical clinical patterns apart from frequency of diarrhea did not correlate with dehydration status. Further studies are needed to validate our results. PMID:25568559

Fresh or dehydrated spineless cactus in diets for lambs

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Solano Felipe Just de Andrade

2016-05-01

Full Text Available The objective was to evaluate the effect of partial or total replacement of Tifton 85 hay with fresh or dehydrated spineless cactus on performance and body weight components of sheep. Thirty hair lambs, with 19 kg ± 0.35 kg initial weight, were randomly divided into five treatments, namely: Tifton 85 Hay (HA; Tifton 85 Hay + fresh spineless cactus (HAFC; Tifton 85 Hay + dehydrated spineless cactus (HADC; fresh spineless cactus (FC; dehydrated spineless cactus (DC. In addition to the spineless cactus and hay, the complete mixture contained soybean meal, mineral salt and limestone in all treatments. Animals were confined for 75 days and then slaughtered. The weight of body components was recorded. Dry matter intake, total digestible nutrients intake and voluntary water intake were higher (p 0.05 by treatments. The cold carcass weight, rib eye area, weight and yield of the skin, liver, kidney and large intestine were higher (p < 0.05 for the DC and HADC treatments. Partial or total replacement of Tifton 85 hay with fresh or dehydrated spineless cactus, in complete diets for sheep, increases the weight of the cold carcass and non-carcass components.

A fatal case of hypernatraemic dehydration in a neonate.

Science.gov (United States)

Staub, Eveline; Wilkins, Barry

2012-09-01

Problems with lactation can result in hypernatraemic dehydration in the neonate, with potentially severe adverse consequences. This is illustrated in this fatal case of a 10 day old neonate who presented with excessive hypernatraemic dehydration due to insufficient breast milk intake, resulting in cerebral sinus vein thrombosis with cerebral haemorrhage and infarction. Differential diagnosis included excessive sodium intake (through inappropriately mixed formula or house remedies or through hyperaldosteronism) and high water deficit (renal or gastrointestinal losses, nephrogenic or central diabetes insipidus), all of which were ruled out by specific investigations or history. No evidence was found for inborn error of metabolism. The dehydration in this baby, however, was accentuated by trans-epidermal water loss due to an ichthyosiform skin condition. This first ever reported Australian fatality from neonatal hypernatraemic dehydration supports the concern of health care professionals over rising incidences of this entity in exclusively breastfed infants, and should encourage endorsement of improved monitoring of weight loss in newborns and breastfeeding support for their mothers. © 2012 The Authors. Journal of Paediatrics and Child Health © 2012 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

Chemical clearing and dehydration of GFP expressing mouse brains.

Science.gov (United States)

Becker, Klaus; Jährling, Nina; Saghafi, Saiedeh; Weiler, Reto; Dodt, Hans-Ulrich

2012-01-01

Generally, chemical tissue clearing is performed by a solution consisting of two parts benzyl benzoate and one part benzyl alcohol. However, prolonged exposure to this mixture markedly reduces the fluorescence of GFP expressing specimens, so that one has to compromise between clearing quality and fluorescence preservation. This can be a severe drawback when working with specimens exhibiting low GFP expression rates. Thus, we screened for a substitute and found that dibenzyl ether (phenylmethoxymethylbenzene, CAS 103-50-4) can be applied as a more GFP-friendly clearing medium. Clearing with dibenzyl ether provides improved tissue transparency and strikingly improved fluorescence intensity in GFP expressing mouse brains and other samples as mouse spinal cords, or embryos. Chemical clearing, staining, and embedding of biological samples mostly requires careful foregoing tissue dehydration. The commonly applied tissue dehydration medium is ethanol, which also can markedly impair GFP fluorescence. Screening for a substitute also for ethanol we found that tetrahydrofuran (CAS 109-99-9) is a more GFP-friendly dehydration medium than ethanol, providing better tissue transparency obtained by successive clearing. Combined, tetrahydrofuran and dibenzyl ether allow dehydration and chemical clearing of even delicate samples for UM, confocal microscopy, and other microscopy techniques.

Dehydration and hydration behavior of metal-salt-modified materials for chemical heat pumps

International Nuclear Information System (INIS)

Ishitobi, Hirokazu; Uruma, Keirei; Takeuchi, Masato; Ryu, Junichi; Kato, Yukitaka

2013-01-01

Lithium chloride (LiCl) modified magnesium hydroxide (Mg(OH) 2 ) is a potential new material for chemical heat pumps. However, there is insufficient information concerning its dehydration and hydration behavior. In this study, the dehydration and hydration reactions, corresponding to the heat storage and the heat output operations, respectively, of authentic Mg(OH) 2 and LiCl-modified Mg(OH) 2 were investigated by thermogravimetric methods and near infrared spectroscopy. The dehydration of authentic Mg(OH) 2 proceeded as a one-step reaction. In contrast, the dehydration of LiCl-modified Mg(OH) 2 occurred in two steps. The dehydration reaction rates were increased by LiCl modification of the Mg(OH) 2 surface, while the activation energy for the first-order dehydration reaction was lowered. The mechanism for the hydration reaction of magnesium oxide (MgO) was different to that for the hydration of LiCl-modified MgO. This difference was explained by the effect of the LiCl on the MgO particle surface. - Highlights: ► LiCl-modified Mg(OH) 2 is a candidate material for chemical heat pumps. ► The dehydration reaction of LiCl-modified Mg(OH) 2 is a two-step reaction. ► The dehydration reaction of Mg(OH) 2 was enhanced by LiCl modification. ► The hydration mechanisms of authentic MgO and LiCl-modified MgO were different.

Relationship of Renal Function Tests and Electrolyte Levels with Severity of Dehydration in Acute Diarrhea.

Science.gov (United States)

Gauchan, E; Malla, K K

2015-01-01

Acute diarrheal illness constitutes a major cause of morbidity and mortality in children in developing countries. Most of the complications of diarrhea occur due to excessive fluid and electrolyte loss; adverse complications are seen more with increasing severity of dehydration. This study was conducted to identify the relation of renal function and electrolyte abnormalities in children with varying severity of dehydration. This study was carried out in Manipal Teaching Hospital, Pokhara, Nepal over duration of one year. The aims were to find out the association of renal function and electrolyte disturbances with type of diarrhea, severity of dehydration and their relation to outcome. All children more than one month and less than 15 years with acute diarrhea were included in the study. Data were entered and analyzed by SPSS version 19. Statistical analysis applied was Chi-square test. A p-value of Dehydration was associated more with Acute Watery Diarrhea than with Invasive Diarrhea. Renal function and electrolyte abnormalities were seen more in Acute Watery Diarrhea with increasing levels of blood urea, serum creatinine and abnormal levels of serum sodium seen with increased severity of dehydration. Abnormalities in renal function and electrolytes correlated significantly with severity of dehydration. The outcome of patients correlated with severity of dehydration with mortality occurring in 18.1% of patients with Severe dehydration, 0.8% of Some dehydration with no mortality in the No dehydration group.

Management of acute attacks of hereditary angioedema: potential role of icatibant

Directory of Open Access Journals (Sweden)

Hilary J Longhurst

2010-09-01

Full Text Available Hilary J LonghurstDepartment of Immunology, Barts and The London NHS Trust, London, UKAbstract: Icatibant (Firazyr® is a novel subcutaneous treatment recently licensed in the European Union for acute hereditary angioedema. Hereditary angioedema, resulting from inherited partial C1 inhibitor deficiency, is a disabling condition characterized by intermittent episodes of bradykinin-mediated angioedema. Icatibant blocks bradykinin B2 receptors, attenutating the episode. Randomized double-blind, placebo-controlled trials of icatibant, showed significant superiority over oral tranexamic acid in 74 European patients and a trend to improvement in a similar US trial comparing icatibant with placebo in 55 patients. Outcomes for several endpoints did not reach significance in the US trial, perhaps because of low participant numbers and confounding factors: a further trial is planned. Open label studies have shown benefit in multiple treatments for attacks at all sites. Approximately 10% of patients require a second dose for re-emergent symptoms, usually 10 to 27 hours after the initial treatment. Its subcutaneous route of administration, good tolerability and novel mode of action make icatibant a promising addition to the limited repertoire of treatments for hereditary angioedema.Keywords: hereditary angioedema, bradykinin, icatibant, C1 inhibitor deficiency

Apple's dehydration by the irradiation pretreatment

International Nuclear Information System (INIS)

Fu Junjie; Chao Yan; Shen Weiqiao; Wang Jun

2001-01-01

"6"0 Co γ-ray irradiation was used as a pre-treatment method to dry the apple. The aim of this study was to discover the effect factor concerning the change of the apple cell structure which would affect the speed of drhydration and the relation between the speed of hot air dehydration and the irradiation dose. The results demonstrated that with the increasing of irradiation dose. The damage of apple's vacuole membrane increased. The positive correlation was shown in slice thickness and dehydration rate, the relation of the irradiation dose and the temperature of hot air was negatively correlated. The optimum of pre-treatment was gained for slice thickness, the irradiation dose and dry temperature of hot air. (authors)

The usefulness of clinical and laboratory parameters for predicting severity of dehydration in children with acute gastroenteritis.

Science.gov (United States)

Hoxha, Teuta Faik; Azemi, Mehmedali; Avdiu, Muharrem; Ismaili-Jaha, Vlora; Grajqevci, Violeta; Petrela, Ela

2014-10-01

An accurate assessment of the degree of dehydration in infants and children is important for proper decision-making and treatment. This emphasizes the need for laboratory tests to improve the accuracy of clinical assessment of dehydration. The aim of this study was to assess the relationship between clinical and laboratory parameters in the assessment of dehydration. We evaluated prospectively 200 children aged 1 month to 5 years who presented with diarrhea, vomiting or both. Dehydration assessment was done following a known clinical scheme. We enrolled in the study 200 children (57.5% were male). The mean age was 15.62±9.03 months, with more than half those studied being under 24 months old. Overall, 46.5% (93) had mild dehydration, 34% (68) had moderate dehydration, 5.5% (11) had severe dehydration whereas, 14% (28) had no dehydration. Patients historical clinical variables in all dehydration groups did not differ significantly regarding age, sex, fever, frequency of vomiting, duration of diarrhea and vomiting, while there was a trend toward severe dehydration in children with more frequent diarrhea (p=0.004). Serum urea and creatinine cannot discriminate between mild and moderate dehydration but they showed a good specificity for severe dehydration of 99% and 100% respectively. Serum bicarbonates and base excess decreased significantly with a degree of dehydration and can discriminate between all dehydration groups (Pdehydration status among children presenting with acute gastroenteritis. Serum urea and creatinine were the most specific tests for severe dehydration diagnosis. Historical clinical patterns apart from frequency of diarrhea did not correlate with dehydration status. Further studies are needed to validate our results.

The independent influences of heat strain and dehydration upon cognition.

Science.gov (United States)

van den Heuvel, Anne M J; Haberley, Benjamin J; Hoyle, David J R; Taylor, Nigel A S; Croft, Rodney J

2017-05-01

Many researchers have addressed the potential effects of hyperthermia and dehydration on cognition, often revealing contradictory outcomes. A possible reason for this inconsistency is that experiments may have been inadequately designed for such effects. In this study, the impact of hyperthermia, dehydration and their combination on cognition were evaluated in eight young males, after accounting for a range of experimental limitations. Passive heating and thermal clamping at two mean body temperatures (36.5, 38.5 °C) were performed under three hydration states (euhydrated, 3 and 5% dehydrated) to assess their effects on difficulty-matched working memory and visual perception tasks, and on a difficulty manipulated perceptual task. Data were analysed according to signal detection theory to isolate changes in response sensitivity, bias and speed. Neither moderate hyperthermia (P = 0.141) nor dehydration (P > 0.604) modified response sensitivity, nor did they significantly interact (P > 0.698). Therefore, the ability to distinguish correct from incorrect responses was unaffected. Nevertheless, hyperthermia, but not dehydration (P = 0.301), reduced the response bias (-0.08 versus 2.2 [normothermia]; P = 0.010) and reaction time (mean reduction 49 ms; P < 0.001), eliciting more liberal and faster responses (P = 0.010). Response bias was reduced for the memory relative to the perceptual task (P = 0.037), and this effect was enhanced during hyperthermia (P = 0.031). These observations imply that, once potentially confounding influences were controlled, moderate hyperthermia, significant dehydration and their combined effects had insufficient impact to impair cognition within the memory and perceptual domains tested. Nonetheless, moderate hyperthermia elicited more liberal and rapid responses.

Neuromyelitis optica antibody in Leber hereditary optic neuropathy: case report

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Luciano Mesquita Simão

2012-08-01

Full Text Available Neuromyelitis optica antibody (or aquaporin-4 antibody is a well stablished serum marker associated to high-risk neuromyelitis optica syndrome that presents as an inflammatory demyelinating disease characterized by the occurrence of bilateral and simultaneous optic neuritis without complete visual recovery or it occurs as an isolated episode of transverse myelitis accompanied by longitudinally extensive spinal cord lesions. On the other hand, Leber hereditary optic neuropathy is a primarily hereditary disorder that affects all tissues of the body and its clinical presentation is tissue-specific for the optic nerve and, eventually, it might reach the spinal cord. Overlapping clinical features of neuromyelitis optica and Leber hereditary optic neuropathy may suggest common target organ diseases. The case report described herein emphasizes the coexistence of serum markers of both diseases, and suggests that further investigation of this challenging clinical presentation is warranted to confirm or rule out this association.

Surveillance for hereditary nonpolyposis colorectal cancer: a long-term study on 114 families.

NARCIS (Netherlands)

Vos tot Nederveen Cappel, W.H. de; Nagengast, F.M.; Griffioen, G.; Menko, F.H.; Taal, B.G.; Kleibeuker, J.H.; Vasen, H.F.

2002-01-01

PURPOSE: Hereditary nonpolyposis colorectal cancer is caused by germline mutations in DNA mismatch repair genes. Mutation carriers have a 60 to 85 percent risk of developing colorectal cancer. In the Netherlands hereditary nonpolyposis colorectal cancer families are monitored in an intensive

Surveillance for hereditary nonpolyposis colorectal cancer - A long-term study on 114 families

NARCIS (Netherlands)

Cappel, WHDTN; Nagengast, FM; Griffioen, G; Menko, FH; Taal, BG; Kleibeuker, JH; Vasen, HF

2002-01-01

PURPOSE: Hereditary nonpolyposis colorectal cancer is caused by germline mutations in DNA mismatch repair genes. Mutation carriers have a 60 to 85 percent risk of developing colorectal cancer. In the Netherlands hereditary nonpolyposis colorectal cancer families are monitored in an intensive

Effects of irradiation on decontamination and nutrients of dehydrated longan

International Nuclear Information System (INIS)

Zhu Jiating; Feng Min; Tang Yuxin; Lin Jiabin; Yang Ping; Wang Dening

2011-01-01

The dehydrated longan were irradiated at the doses of 2, 4, 6, 8 and 10 kGy and the effects of irradiation on nutrients contents and decontamination were studied. Results showed that dehydrated longan were irradiated at 6 kGy, the number of total bacterial count, mold and coliform bacteria accorded with national standards. There were no significant influence on contents of crude fat, ash, Fe, P, Na and V B2 , but the contents of protein, crude fiber, carbohydrate, moisture, Ca, K and V C of irradiated deghdrated longan were significantly different with control. 6-10 kGy irradiation could meet the commercial demands of dehydrated longan decontamination. (authors)

Recent Advances on Bioethanol Dehydration using Zeolite Membrane

Science.gov (United States)

Makertihartha, I. G. B. N.; Dharmawijaya, P. T.; Wenten, I. G.

2017-07-01

Renewable energy has gained increasing attention throughout the world. Bioethanol has the potential to replace existing fossil fuel usage without much modification in existing facilities. Bioethanol which generally produced from fermentation route produces low ethanol concentration. However, fuel grade ethanol requires low water content to avoid engine stall. Dehydration process has been increasingly important in fuel grade ethanol production. Among all dehydration processes, pervaporation is considered as the most promising technology. Zeolite possesses high potential in pervaporation of bioethanol into fuel grade ethanol. Zeolite membrane can either remove organic (ethanol) from aqueous mixture or water from the mixture, depending on the framework used. Hydrophilic zeolite membrane, e.g. LTA, can easily remove water from the mixture leaving high ethanol concentration. On the other hand, hydrophobic zeolite membrane, e.g. silicate-1, can remove ethanol from aqueous solution. This review presents the concept of bioethanol dehydration using zeolite membrane. Special attention is given to the performance of selected pathway related to framework selection.

The tumour spectrum in hereditary non-polyposis colorectal cancer: a study of 24 kindreds in the Netherlands

NARCIS (Netherlands)

Vasen, H. F.; Offerhaus, G. J.; den Hartog Jager, F. C.; Menko, F. H.; Nagengast, F. M.; Griffioen, G.; van Hogezand, R. B.; Heintz, A. P.

1990-01-01

The hereditary colonic cancer syndrome without polyposis, hereditary non-polyposis colorectal cancer (HNPCC), is usually divided into 2 main categories: hereditary site-specific colorectal cancer (Lynch syndrome I) and colorectal cancer in association with other forms of cancer (Lynch syndrome II).

Methods to increase the rate of mass transfer during osmotic dehydration of foods.

Science.gov (United States)

Chwastek, Anna

2014-01-01

Traditional methods of food preservation such as freezing, freeze drying (lyophilization), vacuum drying, convection drying are often supplemented by new technologies that enable obtaining of high quality products. Osmotic dehydration is more and more often used during processing of fruits and vegetables. This method allows maintaining good organoleptic and functional properties in the finished product. Obtaining the desired degree of dehydration or saturation of the material with an osmoactive substance often requires  elongation of time or use of high temperatures. In recent years much attention was devoted to techniques aimed at increasing the mass transfer between the dehydrated material and the hypertonic solution. The work reviews the literature focused on methods of streamlining the process of osmotic dehydration which include the use of: ultrasound, high hydrostatic pressure, vacuum osmotic dehydration and pulsed electric field.

Dehydration-induced modulation of κ-opioid inhibition of vasopressin neurone activity

Science.gov (United States)

Scott, Victoria; Bishop, Valerie R; Leng, Gareth; Brown, Colin H

2009-01-01

Dehydration increases vasopressin (antidiuretic hormone) secretion from the posterior pituitary gland to reduce water loss in the urine. Vasopressin secretion is determined by action potential firing in vasopressin neurones, which can exhibit continuous, phasic (alternating periods of activity and silence), or irregular activity. Autocrine κ-opioid inhibition contributes to the generation of activity patterning of vasopressin neurones under basal conditions and so we used in vivo extracellular single unit recording to test the hypothesis that changes in autocrine κ-opioid inhibition drive changes in activity patterning of vasopressin neurones during dehydration. Dehydration increased the firing rate of rat vasopressin neurones displaying continuous activity (from 7.1 ± 0.5 to 9.0 ± 0.6 spikes s−1) and phasic activity (from 4.2 ± 0.7 to 7.8 ± 0.9 spikes s−1), but not those displaying irregular activity. The dehydration-induced increase in phasic activity was via an increase in intraburst firing rate. The selective κ-opioid receptor antagonist nor-binaltorphimine increased the firing rate of phasic neurones in non-dehydrated rats (from 3.4 ± 0.8 to 5.3 ± 0.6 spikes s−1) and dehydrated rats (from 6.4 ± 0.5 to 9.1 ± 1.2 spikes s−1), indicating that κ-opioid feedback inhibition of phasic bursts is maintained during dehydration. In a separate series of experiments, prodynorphin mRNA expression was increased in vasopressin neurones of hyperosmotic rats, compared to hypo-osmotic rats. Hence, it appears that dynorphin expression in vasopressin neurones undergoes dynamic changes in proportion to the required secretion of vasopressin so that, even under stimulated conditions, autocrine feedback inhibition of vasopressin neurones prevents over-excitation. PMID:19822541

Validation of the clinical dehydration scale for children with acute gastroenteritis.

Science.gov (United States)

Goldman, Ran D; Friedman, Jeremy N; Parkin, Patricia C

2008-09-01

We previously created a clinical dehydration scale. Our objective was to validate the clinical dehydration scale with a new cohort of patients with acute gastroenteritis who were assessed in a tertiary emergency department in a developed country. A prospective observational study was performed in an emergency department at a large pediatric tertiary center in Canada. Children 1 month to 5 years of age with symptoms of acute gastroenteritis who were assessed in the emergency department were enrolled consecutively during a 4-month period. The main outcome measures were length of stay, proportion of children receiving intravenous fluid rehydration, and proportions of children with abnormal serum pH values or bicarbonate levels. A total of 205 children were enrolled, with a mean age of 22.4 +/- 14.9 months; 103 (50%) were male. The distribution of severity categories was as follows: no dehydration (score of 0), n = 117 (57%); some dehydration (score of 1-4), n = 83 (41%); moderate/severe dehydration (score of 5-8), n = 5 (2%). The 3 dehydration categories were significantly different with respect to the validation hypotheses (length of stay, mean +/- SD: none, 245 +/- 181 minutes; some, 397 +/- 302 minutes; moderate/severe, 501 +/- 389 minutes; treatment with intravenous fluids: none, n =17, 15%; some, n = 41, 49%; moderate/severe, n = 4, 80%; number of vomiting episodes in the 7 days before the emergency department visit: none, 8.4 +/- 7.7 episodes; some, 13 +/- 10.7 episodes; moderate/severe, 30.2 +/- 14.8 episodes). The clinical dehydration scale and the 3 severity categories were valid for a prospectively enrolled cohort of patients who were assessed in our tertiary emergency department. The scoring system was valuable in predicting a longer length of stay and the need for intravenous fluid rehydration for children with symptoms of acute gastroenteritis.

Dehydration of Glucose to 5-Hydroxymethylfurfural Using Nb-doped Tungstite

KAUST Repository

Yue, Chaochao

2016-08-05

Dehydration of glucose to 5-hydroxymethylfurfural (HMF) remains a significant problem in the context of the valorization of lignocellulosic biomass. Hydrolysis of WCl6 and NbCl5 leads to precipitation of Nb-containing tungstite (WO3H2O) at low Nb content and mixtures of tungstite and niobic acid at higher Nb content. Tungstite is a promising catalyst for the dehydration of glucose to HMF. Compared with Nb2O5, fewer by-products are formed because of the low BrOnsted acidity of the (mixed) oxides. In water, an optimum yield of HMF was obtained for Nb-W oxides with low Nb content owing to balanced Lewis and BrOnsted acidity. In THF/water, the strong Lewis acidity and weak BrOnsted acidity caused the reaction to proceed through isomerization to fructose and dehydration of fructose to a partially dehydrated intermediate, which was identified by LC-ESI-MS. The addition of HCl to the reaction mixture resulted in rapid dehydration of this intermediate to HMF. The HMF yield obtained in this way was approximately 56% for all tungstite catalysts. Density functional theory calculations show that the Lewis acid centers on the tungstite surface can isomerize glucose into fructose. Substitution of W by Nb lowers the overall activation barrier for glucose isomerization by stabilizing the deprotonated glucose adsorbate.

[The progress and prospect of application of genetic testing technology-based gene detection technology in the diagnosis and treatment of hereditary cancer].

Science.gov (United States)

He, J X; Jiang, Y F

2017-08-06

Hereditary cancer is caused by specific pathogenic gene mutations. Early detection and early intervention are the most effective ways to prevent and control hereditary cancer. High-throughput sequencing based genetic testing technology (NGS) breaks through the restrictions of pedigree analysis, provide a convenient and efficient method to detect and diagnose hereditary cancer. Here, we introduce the mechanism of hereditary cancer, summarize, discuss and prospect the application of NGS and other genetic tests in the diagnosis of hereditary retinoblastoma, hereditary breast and ovarian cancer syndrome, hereditary colorectal cancer and other complex and rare hereditary tumors.

2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema

DEFF Research Database (Denmark)

Bowen, Tom; Cicardi, Marco; Farkas, Henriette

2010-01-01

ABSTRACT: BACKGROUND: We published the Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema (HAE; C1 inhibitor [C1-INH] deficiency) and updated this as Hereditary angioedema: a current state-of-the-art review: Canadian Hungarian 2007 ...

Estimation of EuroQol 5-Dimensions health status utility values in hereditary angioedema

DEFF Research Database (Denmark)

Aygören-Pürsün, Emel; Bygum, Anette; Beusterien, Kathleen

2016-01-01

OBJECTIVE: To estimate health status utility (preference) weights for hereditary angioedema (HAE) during an attack and between attacks using data from the Hereditary Angioedema Burden of Illness Study in Europe (HAE-BOIS-Europe) survey. Utility measures quantitatively describe the net impact of a...

The prevalence of primary hereditary hemochromatosis in central Anatolia.

Science.gov (United States)

Karaca, Halit; Güven, Kadri; Önal, Müge; Gürsoy, Şebnem; Başkol, Mevlüt; Özkul, Yusuf

2013-01-01

Hereditary hemochromatosis is an autosomal recessive disorder associated with the HFE genes. Early identification and diagnosis is important as end stage organ damage may occur if treatment is delayed.. This study aimed to identify the prevalence of hereditary hemochromatosis in Kayseri and surroundings known as Central Anatolia. 2304 participants (1220 males, 1084 females) who were older then the age of 17 were included in the study conducted between December 2005 and December 2006 in Kayseri, Turkey. Transferin saturation was measured from overnight fasting blood samples. Serum iron, total iron binding capacity, and transferin saturation were measured. Serum ferritin levels and hereditary hemochromatosis genetic analysis were also performed after an overnight fasting blood samples from participants whose transferin saturation results were more than 50% in man and more than 45% in women. The homozygote C282Y mutation and heterozygote C282Y mutation prevalences were found as 0.08% (1/1220) and 0.08% (1/1220) in male participants, respectively. The heterozygote H63D mutation prevalence was found in 0.09% (1/1084) of female participants. Calculated prevalences in general population are as follows; The homozygote C282Y mutation prevalence is 0.043% (1/2304), the heterozygote C282Y mutation prevalence is 0.043% (1/2304) and the heterozygote H63D mutation prevalence is 0.043% (1/2304). The prevalence of hereditary hemochromatosis in Central Anatolia is 0.043% (1/2304). Because of the relatively low frequency, population screening studies are not cost-effective.

Intragenic Duplication A Novel Mutational Mechanism in Hereditary Pancreatitis

DEFF Research Database (Denmark)

Joergensen, M. T.; Geisz, A.; Brusgaard, K.

2011-01-01

OBJECTIVES: In a hereditary pancreatitis family from Denmark, we identified a novel intragenic duplication of 9 nucleotides in exon-2 of the human cationic trypsinogen (PRSS1) gene (c.63_71dup) which at the amino-acid level resulted in the insertion of 3 amino acids within the activation peptide...... pancreatitis. The accelerated activation of p.K23_I24insIDK by cathepsin B is a unique biochemical property not found in any other pancreatitis-associated trypsinogen mutant. In contrast, the robust autoactivation of the novel mutant confirms the notion that increased autoactivation is a disease......-relevant mechanism in hereditary pancreatitis....

Dysphonia and vocal fold telangiectasia in hereditary hemorrhagic telangiectasia.

Science.gov (United States)

Chang, Joseph; Yung, Katherine C

2014-11-01

This case report is the first documentation of dysphonia and vocal fold telangiectasia as a complication of hereditary hemorrhagic telangiectasia (HHT). Case report of a 40-year-old man with HHT presenting with 2 years of worsening hoarseness. Hoarseness corresponded with a period of anticoagulation. Endoscopy revealed vocal fold scarring, vocal fold telangiectasias, and plica ventricular is suggestive of previous submucosal vocal fold hemorrhage and subsequent counterproductive compensation with ventricular phonation. Hereditary hemorrhagic telangiectasia may present as dysphonia with vocal fold telangiectasias and place patients at risk of vocal fold hemorrhage. © The Author(s) 2014.

Effective hepatitis A virus inactivation during low-heat dehydration of contaminated green onions.

Science.gov (United States)

Laird, David T; Sun, Yan; Reineke, Karl F; Shieh, Y Carol

2011-08-01

Preserving fruits and vegetables by dehydration is common; however, information is limited concerning viral survival on the produce during the process. This work demonstrated the effects of low heat dehydration on inactivating hepatitis A virus (HAV) on contaminated green onions. Inoculated and uninoculated onion samples were dehydrated at target temperatures of 45-65 °C for 20 h. HAV from artificially contaminated onions (fresh or dehydrated) was eluted by shaking at 145 rpm at 20 °C for 20 min with 3% beef extract, pH 8, and followed by 0.2 μM-membrane filtration before plaque assay and quantitative reverse transcription-polymerase chain reaction (qRT-PCR) analysis. Dilutions of the filtrates were made for obtaining countable plaques on FRhK-4 cell monolayers in 6-well plates, and also for eliminating inhibitors in qRT-PCR. Average water activity of the onions after 20 h-dehydration was 0.227, regardless of temperature used (47.9 °C or 65.1 °C). Eight dehydration trials resulted in a linear relationship between HAV inactivation and dehydration temperature, with HAV log reduction = 0.1372x(°C) - 5.5572, r(2) = 0.88. Therefore, the 20 h-heating at 47.8, 55.1, and 62.4 °C reduced infectious HAV in onions by 1, 2, and 3 logs respectively, the Z value being 7.3 °C. It was concluded that low heat dehydration using 62.5 °C or above could effectively inactivate HAV on contaminated onions by >3 logs. Published by Elsevier Ltd.

An organ-specific role for ethylene in rose petal expansion during dehydration and rehydration

Science.gov (United States)

Liu, Daofeng; Liu, Xiaojing; Meng, Yonglu; Sun, Cuihui; Tang, Hongshu; Jiang, Yudong; Khan, Muhammad Ali; Xue, Jingqi; Ma, Nan; Gao, Junping

2013-01-01

Dehydration is a major factor resulting in huge loss from cut flowers during transportation. In the present study, dehydration inhibited petal cell expansion and resulted in irregular flowers in cut roses, mimicking ethylene-treated flowers. Among the five floral organs, dehydration substantially elevated ethylene production in the sepals, whilst rehydration caused rapid and elevated ethylene levels in the gynoecia and sepals. Among the five ethylene biosynthetic enzyme genes (RhACS1–5), expression of RhACS1 and RhACS2 was induced by dehydration and rehydration in the two floral organs. Silencing both RhACS1 and RhACS2 significantly suppressed dehydration- and rehydration-induced ethylene in the sepals and gynoecia. This weakened the inhibitory effect of dehydration on petal cell expansion. β-glucuronidase activity driven by both the RhACS1 and RhACS2 promoters was dramatically induced in the sepals, pistil, and stamens, but not in the petals of transgenic Arabidopsis. This further supports the organ-specific induction of these two genes. Among the five rose ethylene receptor genes (RhETR1–5), expression of RhETR3 was predominantly induced by dehydration and rehydration in the petals. RhETR3 silencing clearly aggravated the inhibitory effect of dehydration on petal cell expansion. However, no significant difference in the effect between RhETR3-silenced flowers and RhETR-genes-silenced flowers was observed. Furthermore, RhETR-genes silencing extensively altered the expression of 21 cell expansion-related downstream genes in response to ethylene. These results suggest that induction of ethylene biosynthesis by dehydration proceeds in an organ-specific manner, indicating that ethylene can function as a mediator in dehydration-caused inhibition of cell expansion in rose petals. PMID:23599274

Transcriptional and physiological data reveal the dehydration memory behavior in switchgrass (Panicum virgatum L.).

Science.gov (United States)

Zhang, Chao; Peng, Xi; Guo, Xiaofeng; Tang, Gaijuan; Sun, Fengli; Liu, Shudong; Xi, Yajun

2018-01-01

Switchgrass ( Panicum virgatum L.) is a model biofuel plant because of its high biomass, cellulose-richness, easy degradation to ethanol, and the availability of extensive genomic information. However, a little is currently known about the molecular responses of switchgrass plants to dehydration stress, especially multiple dehydration stresses. Studies on the transcriptional profiles of 35-day-old tissue culture plants revealed 741 dehydration memory genes. Gene Ontology and pathway analysis showed that these genes were enriched in phenylpropanoid biosynthesis, starch and sucrose metabolism, and plant hormone signal transduction. Further analysis of specific pathways combined with physiological data suggested that switchgrass improved its dehydration resistance by changing various aspects of its responses to secondary dehydration stress (D2), including the regulation of abscisic acid (ABA) and jasmonic acid (JA) biosynthesis and signal transduction, the biosynthesis of osmolytes (l-proline, stachyose and trehalose), energy metabolism (i.e., metabolic process relating to photosynthetic systems, glycolysis, and the TCA cycle), and lignin biosynthesis. The transcriptional data and chemical substance assays showed that ABA was significantly accumulated during both primary (D1) and secondary (D2) dehydration stresses, whereas JA accumulated during D1 but became significantly less abundant during D2. This suggests the existence of a complicated signaling network of plant hormones in response to repeated dehydration stresses. A homology analysis focusing on switchgrass, maize, and Arabidopsis revealed the conservation and species-specific distribution of dehydration memory genes. The molecular responses of switchgrass plants to successive dehydration stresses have been systematically characterized, revealing a previously unknown transcriptional memory behavior. These results provide new insights into the mechanisms of dehydration stress responses in plants. The genes and

Hereditary breast and ovarian cancer

DEFF Research Database (Denmark)

Nielsen, Finn Cilius; Hansen, Thomas van Overeem; Sørensen, Claus Storgaard

2016-01-01

Genetic abnormalities in the DNA repair genes BRCA1 and BRCA2 predispose to hereditary breast and ovarian cancer (HBOC). However, only approximately 25% of cases of HBOC can be ascribed to BRCA1 and BRCA2 mutations. Recently, exome sequencing has uncovered substantial locus heterogeneity among...... of putative causal variants and the clinical application of new HBOC genes in cancer risk management and treatment decision-making....

Mechanisms by Which Dehydration May Lead to Chronic Kidney Disease.

Science.gov (United States)

Roncal-Jimenez, C; Lanaspa, M A; Jensen, T; Sanchez-Lozada, L G; Johnson, R J

2015-01-01

Dehydration, a condition that characterizes excessive loss of body water, is well known to be associated with acute renal dysfunction; however, it has largely been considered reversible and to be associated with no long-term effects on the kidney. Recently, an epidemic of chronic kidney disease has emerged in Central America in which the major risk factor seems to be recurrent heat-associated dehydration. This has led to studies investigating whether recurrent dehydration may lead to permanent kidney damage. Three major potential mechanisms have been identified, including the effects of vasopressin on the kidney, the activation of the aldose reductase-fructokinase pathway, and the effects of chronic hyperuricemia. The discovery of these pathways has also led to the recognition that mild dehydration may be a risk factor in progression of all types of chronic kidney diseases. Furthermore, there is some evidence that increasing hydration, particularly with water, may actually prevent CKD. Thus, a whole new area of investigation is developing that focuses on the role of water and osmolarity and their influence on kidney function and health. © 2015 S. Karger AG, Basel.

Carotid Flow Time Test Performance for the Detection of Dehydration in Children With Diarrhea.

Science.gov (United States)

Mackenzie, David C; Nasrin, Sabiha; Atika, Bita; Modi, Payal; Alam, Nur H; Levine, Adam C

2017-11-09

Unstructured clinical assessments of dehydration in children are inaccurate. Point-of-care ultrasound is a noninvasive diagnostic tool that can help evaluate the volume status; the corrected carotid artery flow time has been shown to predict volume depletion in adults. We sought to determine the ability of the corrected carotid artery flow time to identify dehydration in a population of children presenting with acute diarrhea in Dhaka, Bangladesh. Children presenting with acute diarrhea were recruited and rehydrated according to hospital protocols. The corrected carotid artery flow time was measured at the time of presentation. The percentage of weight change with rehydration was used to categorize each child's dehydration as severe (>9%), some (3%-9%), or none (dehydration. Linear regression was used to model the relationship between the corrected carotid artery flow time and percentage of dehydration. A total of 350 children (0-60 months) were enrolled. The mean corrected carotid artery flow time was 326 milliseconds (interquartile range, 295-351 milliseconds). The area under the receiver operating characteristic curve for the detection of severe dehydration was 0.51 (95% confidence interval, 0.42, 0.61). Linear regression modeling showed a weak association between the flow time and dehydration. The corrected carotid artery flow time was a poor predictor of severe dehydration in this population of children with diarrhea. © 2017 by the American Institute of Ultrasound in Medicine.

Improvements in the order, isotropy and electron density of glypican-1 crystals by controlled dehydration

Energy Technology Data Exchange (ETDEWEB)

Awad, Wael [Lund University, Box 124, 221 00 Lund (Sweden); Cairo University, Cairo (Egypt); Svensson Birkedal, Gabriel [Lund University, Biomedical Center A13, 221 84 Lund (Sweden); Thunnissen, Marjolein M. G. M. [Lund University, Box 124, 221 00 Lund (Sweden); Lund University, Box 188, 221 00 Lund (Sweden); Mani, Katrin [Lund University, Biomedical Center A13, 221 84 Lund (Sweden); Logan, Derek T., E-mail: derek.logan@biochemistry.lu.se [Lund University, Box 124, 221 00 Lund (Sweden)

2013-12-01

The anisotropy of crystals of glypican-1 was significantly reduced by controlled dehydration using the HC1 device, allowing the building of previously disordered parts of the structure. The use of controlled dehydration for improvement of protein crystal diffraction quality is increasing in popularity, although there are still relatively few documented examples of success. A study has been carried out to establish whether controlled dehydration could be used to improve the anisotropy of crystals of the core protein of the human proteoglycan glypican-1. Crystals were subjected to controlled dehydration using the HC1 device. The optimal protocol for dehydration was developed by careful investigation of the following parameters: dehydration rate, final relative humidity and total incubation time T{sub inc}. Of these, the most important was shown to be T{sub inc}. After dehydration using the optimal protocol the crystals showed significantly reduced anisotropy and improved electron density, allowing the building of previously disordered parts of the structure.

Approximate Mathematical Modeling of Osmotic Dehydration of Cone-Shaped Fruits and Vegetables in Hypertonic Solutions

Directory of Open Access Journals (Sweden)

Mohammad Sirousazar

2017-07-01

Full Text Available Water loss kinetics in osmotic dehydration of cone-shaped fruits and vegetables was modeled on the basis of diffusion mechanism, using the Fick’s second law. The model was developed by taking into account the influences of the fruit geometrical characteristics, initial water content of fruit, water diffusion coefficient in fruit, and the water concentration in hypertonic solution. Based on the obtained model, it was shown that the water diffusion coefficient and the initial water concentration of fruit have direct effects on the dehydration rate and also inverse influence on the dehydration duration. The geometrical parameters of fruit and water concentration in hypertonic solution showed direct effect on the dehydration duration as well as inverse effect on the dehydration rate. The presented model seems to be useful tool to predict the dehydration kinetics of cone-shaped fruit during osmotic dehydration process and to optimize the process prior to perform the experiments.

Methods for assessing the effects of dehydration on cognitive function.

Science.gov (United States)

Lieberman, Harris R

2012-11-01

Studying the effects of dehydration on cognitive function presents a variety of unique and difficult challenges to investigators. These challenges, which are addressed in this article, can be divided into three general categories: 1) choosing an appropriate method of generating a consistent level of dehydration; 2) determining and effectively employing appropriate and sensitive measures of cognitive state; and 3) adequately controlling the many confounding factors that interfere with assessment of cognitive function. The design and conduct of studies on the effects of dehydration on cognitive function should carefully consider various methodological issues, and investigators should carefully weigh the benefits and disadvantages of particular methods and procedures. © 2012 International Life Sciences Institute.

mPGES-1-derived PGE2 mediates dehydration natriuresis

Science.gov (United States)

Jia, Zhanjun; Liu, Gang; Sun, Ying; Kakizoe, Yutaka; Guan, Guangju; Zhang, Aihua; Zhou, Shu-Feng

2013-01-01

PGE2 is a natriuretic factor whose production is elevated after water deprivation (WD) but its role in dehydration natriuresis is not well-defined. The goal of the present study was to investigate the role of microsomal prostaglandin E synthase-1 (mPGES-1) in dehydration natriuresis. After 24-h WD, wild-type (WT) mice exhibited a significant increase in 24-h urinary Na+ excretion accompanied with normal plasma Na+ concentration and osmolality. In contrast, WD-induced elevation of urinary Na+ excretion was completely abolished in mPGES-1 knockout (KO) mice in parallel with increased plasma Na+ concentration and a trend increase in plasma osmolality. WD induced a 1.8-fold increase in urinary PGE2 output and a 1.6-fold increase in PGE2 content in the renal medulla of WT mice, both of which were completely abolished by mPGES-1 deletion. Similar patterns of changes were observed for urinary nitrate/nitrite and cGMP. The natriuresis in dehydrated WT mice was associated with a significant downregulation of renal medullary epithelial Na channel-α mRNA and protein, contrasting to unaltered expressions in dehydrated KO mice. By quantitative RT-PCR, WD increased the endothelial nitric oxide synthase (eNOS), inducible NOS, and neuronal NOS expressions in the renal medulla of WT mice by 3.9-, 1.48-, and 2.6-fold, respectively, all of which were significantly blocked in mPGES-1 KO mice. The regulation of eNOS expression was further confirmed by immunoblotting. Taken together, our results suggest that mPGES-1-derived PGE2 contributes to dehydration natriuresis likely via NO/cGMP. PMID:23171554

Thermodynamic characteristics of dehydration of hexahydrates of erbium, thulium and lutetium chlorides

International Nuclear Information System (INIS)

Ukraintseva, Eh.A.; Sokolova, N.P.; Logvinenko, V.A.

1991-01-01

Temperature dependence of water vapour equilibrium pressure over the compounds of ErCl 3 ·6H-2O, TmCl 3 ·6H 2 O and LuCl 3 ·6H 2 O is studied by membrane method within the temperature range of 309-403 K. Dehydration process stoichiometry is determined thermogravimetrically under quasi-equilibrium conditions. All three compounds split off three molecules at the first stage of dehydration. ErCl 3 ·6H 2 O and TmCl 2 ·6H 2 O are very similar to terbium and disprosium chloride hexahydrates by vapour pressure value and dehydration enthalpy; enthalpy of the first dehydration stage is of the same character as those of nedymium, gadolinium and holmium chloride haxahydrates

Chemical clearing and dehydration of GFP expressing mouse brains.

Directory of Open Access Journals (Sweden)

Klaus Becker

Full Text Available Generally, chemical tissue clearing is performed by a solution consisting of two parts benzyl benzoate and one part benzyl alcohol. However, prolonged exposure to this mixture markedly reduces the fluorescence of GFP expressing specimens, so that one has to compromise between clearing quality and fluorescence preservation. This can be a severe drawback when working with specimens exhibiting low GFP expression rates. Thus, we screened for a substitute and found that dibenzyl ether (phenylmethoxymethylbenzene, CAS 103-50-4 can be applied as a more GFP-friendly clearing medium. Clearing with dibenzyl ether provides improved tissue transparency and strikingly improved fluorescence intensity in GFP expressing mouse brains and other samples as mouse spinal cords, or embryos. Chemical clearing, staining, and embedding of biological samples mostly requires careful foregoing tissue dehydration. The commonly applied tissue dehydration medium is ethanol, which also can markedly impair GFP fluorescence. Screening for a substitute also for ethanol we found that tetrahydrofuran (CAS 109-99-9 is a more GFP-friendly dehydration medium than ethanol, providing better tissue transparency obtained by successive clearing. Combined, tetrahydrofuran and dibenzyl ether allow dehydration and chemical clearing of even delicate samples for UM, confocal microscopy, and other microscopy techniques.

Gene expression changes governing extreme dehydration tolerance in an Antarctic insect

Science.gov (United States)

Teets, Nicholas M.; Peyton, Justin T.; Colinet, Herve; Renault, David; Kelley, Joanna L.; Kawarasaki, Yuta; Lee, Richard E.; Denlinger, David L.

2012-01-01

Among terrestrial organisms, arthropods are especially susceptible to dehydration, given their small body size and high surface area to volume ratio. This challenge is particularly acute for polar arthropods that face near-constant desiccating conditions, as water is frozen and thus unavailable for much of the year. The molecular mechanisms that govern extreme dehydration tolerance in insects remain largely undefined. In this study, we used RNA sequencing to quantify transcriptional mechanisms of extreme dehydration tolerance in the Antarctic midge, Belgica antarctica, the world’s southernmost insect and only insect endemic to Antarctica. Larvae of B. antarctica are remarkably tolerant of dehydration, surviving losses up to 70% of their body water. Gene expression changes in response to dehydration indicated up-regulation of cellular recycling pathways including the ubiquitin-mediated proteasome and autophagy, with concurrent down-regulation of genes involved in general metabolism and ATP production. Metabolomics results revealed shifts in metabolite pools that correlated closely with changes in gene expression, indicating that coordinated changes in gene expression and metabolism are a critical component of the dehydration response. Finally, using comparative genomics, we compared our gene expression results with a transcriptomic dataset for the Arctic collembolan, Megaphorura arctica. Although B. antarctica and M. arctica are adapted to similar environments, our analysis indicated very little overlap in expression profiles between these two arthropods. Whereas several orthologous genes showed similar expression patterns, transcriptional changes were largely species specific, indicating these polar arthropods have developed distinct transcriptional mechanisms to cope with similar desiccating conditions. PMID:23197828

Hereditary chronic pancreatitis

Directory of Open Access Journals (Sweden)

Mössner Joachim

2007-01-01

Full Text Available Abstract Hereditary chronic pancreatitis (HCP is a very rare form of early onset chronic pancreatitis. With the exception of the young age at diagnosis and a slower progression, the clinical course, morphological features and laboratory findings of HCP do not differ from those of patients with alcoholic chronic pancreatitis. As well, diagnostic criteria and treatment of HCP resemble that of chronic pancreatitis of other causes. The clinical presentation is highly variable and includes chronic abdominal pain, impairment of endocrine and exocrine pancreatic function, nausea and vomiting, maldigestion, diabetes, pseudocysts, bile duct and duodenal obstruction, and rarely pancreatic cancer. Fortunately, most patients have a mild disease. Mutations in the PRSS1 gene, encoding cationic trypsinogen, play a causative role in chronic pancreatitis. It has been shown that the PRSS1 mutations increase autocatalytic conversion of trypsinogen to active trypsin, and thus probably cause premature, intrapancreatic trypsinogen activation disturbing the intrapancreatic balance of proteases and their inhibitors. Other genes, such as the anionic trypsinogen (PRSS2, the serine protease inhibitor, Kazal type 1 (SPINK1 and the cystic fibrosis transmembrane conductance regulator (CFTR have been found to be associated with chronic pancreatitis (idiopathic and hereditary as well. Genetic testing should only be performed in carefully selected patients by direct DNA sequencing and antenatal diagnosis should not be encouraged. Treatment focuses on enzyme and nutritional supplementation, pain management, pancreatic diabetes, and local organ complications, such as pseudocysts, bile duct or duodenal obstruction. The disease course and prognosis of patients with HCP is unpredictable. Pancreatic cancer risk is elevated. Therefore, HCP patients should strongly avoid environmental risk factors for pancreatic cancer.

Major and minor form of hereditary hyperekplexia

NARCIS (Netherlands)

Tijssen, MAJ; Vergouwe, MN; van Dijk, JG; Rees, M; Frants, RR; Brown, P

Hyperekplexia is a hereditary neurological disorder characterized by excessive startle responses. Within the disorder two clinical forms can be distinguished. The major form is characterized by continuous generalized stiffness in the first year of life and an exaggerated startle reflex, accompanied

Hereditary hemorrhagic telangiectasia clinical and molecular genetics

NARCIS (Netherlands)

Letteboer, T.G.W.

2010-01-01

Hereditary hemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber (ROW) syndrome is an autosomal dominant disease characterized by vascular malformations in multiple organ systems. HHT has an age-related penetrance and variable clinical expression. The clinical symptoms are caused by direct

Dehydration Kinetics of Chlorite at High Temperatures and Geophysical Implications

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Shen, K.; Wang, D.; Liu, T.

2017-12-01

A significant amount of water is released from hydrous phases in subduction zones and the brought into the earth's interior. The resulting flux may trigger earthquakes and arc magmatism. Chlorite is one of the most important hydrous minerals with a high water content of 13.0 wt.% in the deep subduction zones, and the dehydration of chlorites are thought to be associated with many anomalies geophysical observations. To understand the nature of the geology and geophysical phenomenon, further research on the dehydration of chlorite should be carried out. Here we report the new results on dehydration kinetics of chlorite at high temperatures. We investigated the dehydration kinetics of chlorite using thermogravimetric analyses (TGA) and X-ray diffraction. The dehydration experiments were conducted with heating rates of 15, 20, 25 K/min up to 1466K. The fitted TGA data results indicate that the probable dehydroxylation mechanism of chlorite is a three-dimensional diffusion reaction with the Fick's second law. The results reveal that the dehydroxylation reaction can be divided into two stages corresponding to the hydroxyls in the two different layers: the first stage between 853 K and 973 K is related to the dehydroxylation of the interlayer hydroxide with the activation energy (Ea) of 159 kJ/ mol and pre-exponential factor (D0) value of 1.53x10-5 m2/s; the second stage between 973 K and 1093 K with an Ea value of 189 kJ/mol and D0 of 2.1x10-5 m2/s is due to the dehydroxylation of the `talc' layer. The mineral reactions and products were observed by high-temperature X-ray diffraction. There are metastable phases during reactions and product phases exhibited a topotactic relationship. The dehydroxylation reaction of chlorite is controlled by an inhomogeneous mechanism. We determine that the fluid production rates of chlorite are 2.7x10-4s-1, 4.5x10-4s-1, 7.3x10-4s-1, 1.2x10-3s-1, 1.7x10-4s-1, at 863 K, 883 K, 903 K, 923 K, 943 K for isothermal dehydration reaction. Our

Dehydration and acute weight gain in mixed martial arts fighters before competition.

Science.gov (United States)

Jetton, Adam M; Lawrence, Marcus M; Meucci, Marco; Haines, Tracie L; Collier, Scott R; Morris, David M; Utter, Alan C

2013-05-01

The purpose of this study was to characterize the magnitude of acute weight gain (AWG) and dehydration in mixed martial arts (MMA) fighters before competition. Urinary measures of hydration status and body mass were determined approximately 24 hours before and then again approximately 2 hours before competition in 40 MMA fighters (mean ± SE, age: 25.2 ± 0.65 years, height: 1.77 ± 0.01 m, body mass: 75.8 ± 1.5 kg). The AWG was defined as the amount of body weight the fighters gained in the approximately 22-hour period between the official weigh-in and the actual competition. On average, the MMA fighters gained 3.40 ± 2.2 kg or 4.4% of their body weight in the approximately 22-hour period before competition. Urine specific gravity significantly decreased (p 1.021 immediately before competition indicating significant or serious dehydration. The MMA fighters undergo significant dehydration and fluctuations in body mass (4.4% avg.) in the 24-hour period before competition. Urinary measures of hydration status indicate that a significant proportion of MMA fighters are not successfully rehydrating before competition and subsequently are competing in a dehydrated state. Weight management guidelines to prevent acute dehydration in MMA fighters are warranted to prevent unnecessary adverse health events secondary to dehydration.

Osmotic dehydration of Braeburn variety apples in the production of sustainable food products

Science.gov (United States)

Ciurzyńska, Agnieszka; Cichowska, Joanna; Kowalska, Hanna; Czajkowska, Kinga; Lenart, Andrzej

2018-01-01

The aim of this work was to investigate the effects of osmotic dehydration conditions on the properties of osmotically pre-treated dried apples. The scope of research included analysing the most important mass exchange coefficients, i.e. water loss, solid gain, reduced water content and water activity, as well as colour changes of the obtained dried product. In the study, apples were osmotically dehydrated in one of two 60% solutions: sucrose or sucrose with an addition of chokeberry juice concentrate, for 30 and 120 min, in temperatures of 40 and 60°C. Ultrasound was also used during the first 30 min of the dehydration process. After osmotic pre-treatment, apples were subjected to innovative convective drying with the puffing effect, and to freeze-drying. Temperature and dehydration time increased the effectiveness of mass exchange during osmotic dehydration. The addition of chokeberry juice concentrate to standard sucrose solution and the use of ultrasound did not change the value of solid gain and reduced water content. Water activity of the dried apple tissue was not significantly changed after osmotic dehydration, while changes in colour were significant.

A New Freezing Method Using Pre-Dehydration by Microwave-Vacuum Drying

Science.gov (United States)

Tsuruta, Takaharu; Hamidi, Nurkholis

Partial dehydration by microwave-vacuum drying has been applied to tuna and strawberry in order to reduce cell-damages caused by the formation of large ice-crystals during freezing. The samples were subjected to microwave vacuum drying at pressure of 5 kPa and temperature less than 27°C to remove small amount of water prior to freezing. The tuna were cooled by using the freezing chamber at temperature -50°C or -150°C, while the strawberries were frozen at temperature -30°C or -80°C, respectively. The temperature transients in tuna showed that removing some water before freezing made the freezing time shorter. The observations of ice crystal clearly indicated that rapid cooling and pre-dehydration prior to freezing were effective in minimizing the size of ice crystal. It is also understood that the formation of large ice crystals has a close relation to the cell damages. After thawing, the observation of microstructure was done on the tuna and strawberry halves. The pre-dehydrated samples showed a better structure than the un-dehydrated one. It is concluded that the pre-dehydration by microwave-vacuum drying is one promising method for the cryo-preservation of foods.

Transcriptome sequencing and whole genome expression profiling of chrysanthemum under dehydration stress

Science.gov (United States)

2013-01-01

Background Chrysanthemum is one of the most important ornamental crops in the world and drought stress seriously limits its production and distribution. In order to generate a functional genomics resource and obtain a deeper understanding of the molecular mechanisms regarding chrysanthemum responses to dehydration stress, we performed large-scale transcriptome sequencing of chrysanthemum plants under dehydration stress using the Illumina sequencing technology. Results Two cDNA libraries constructed from mRNAs of control and dehydration-treated seedlings were sequenced by Illumina technology. A total of more than 100 million reads were generated and de novo assembled into 98,180 unique transcripts which were further extensively annotated by comparing their sequencing to different protein databases. Biochemical pathways were predicted from these transcript sequences. Furthermore, we performed gene expression profiling analysis upon dehydration treatment in chrysanthemum and identified 8,558 dehydration-responsive unique transcripts, including 307 transcription factors and 229 protein kinases and many well-known stress responsive genes. Gene ontology (GO) term enrichment and biochemical pathway analyses showed that dehydration stress caused changes in hormone response, secondary and amino acid metabolism, and light and photoperiod response. These findings suggest that drought tolerance of chrysanthemum plants may be related to the regulation of hormone biosynthesis and signaling, reduction of oxidative damage, stabilization of cell proteins and structures, and maintenance of energy and carbon supply. Conclusions Our transcriptome sequences can provide a valuable resource for chrysanthemum breeding and research and novel insights into chrysanthemum responses to dehydration stress and offer candidate genes or markers that can be used to guide future studies attempting to breed drought tolerant chrysanthemum cultivars. PMID:24074255

Hereditary Kidney Cancer Syndromes and Surgical Management of the Small Renal Mass.

Science.gov (United States)

Nguyen, Kevin A; Syed, Jamil S; Shuch, Brian

2017-05-01

The management of patients with hereditary kidney cancers presents unique challenges to clinicians. In addition to an earlier age of onset compared with patients with sporadic kidney cancer, those with hereditary kidney cancer syndromes often present with bilateral and/or multifocal renal tumors and are at risk for multiple de novo lesions. This population of patients may also present with extrarenal manifestations, which adds an additional layer of complexity. Physicians who manage these patients should be familiar with the underlying clinical characteristics of each hereditary kidney cancer syndrome and the suggested surgical approaches and recommendations of genetic testing for at-risk individuals. Copyright © 2016 Elsevier Inc. All rights reserved.

Fetal MRI of hereditary multiple intestinal atresia with postnatal correlation

International Nuclear Information System (INIS)

Githu, Tangayi; Merrow, Arnold C.; Lee, Jason K.; Garrison, Aaron P.; Brown, Rebeccah L.

2014-01-01

Hereditary multiple intestinal atresia (HMIA) is an extremely uncommon cause of congenital bowel obstruction. The morbidity and mortality of this disease differ significantly from those of isolated intestinal atresias and non-hereditary forms of multiple intestinal atresia. Most notably, despite successful operative repairs of the atresias found in this disease, HMIA maintains a 100% lethality rate from continued post-operative intestinal failure and an associated severe immunodeficiency. We present a case of HMIA evaluated with fetal MRI and subsequently diagnosed by a combination of corroborative postnatal imaging with surgical exploration and pathological examination. (orig.)

Fetal MRI of hereditary multiple intestinal atresia with postnatal correlation

Energy Technology Data Exchange (ETDEWEB)

Githu, Tangayi [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Radiology of Huntsville, P.C., Huntsville, AL (United States); Merrow, Arnold C.; Lee, Jason K. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Garrison, Aaron P. [Cincinnati Children' s Hospital Medical Center, Department of Surgical Services, Division of Pediatric General and Thoracic Surgery, Cincinnati, OH (United States); Akron Children' s Hospital, Pediatric Surgery, Akron, OH (United States); Brown, Rebeccah L. [Cincinnati Children' s Hospital Medical Center, Department of Surgical Services, Division of Pediatric General and Thoracic Surgery, Cincinnati, OH (United States)

2014-03-15

Hereditary multiple intestinal atresia (HMIA) is an extremely uncommon cause of congenital bowel obstruction. The morbidity and mortality of this disease differ significantly from those of isolated intestinal atresias and non-hereditary forms of multiple intestinal atresia. Most notably, despite successful operative repairs of the atresias found in this disease, HMIA maintains a 100% lethality rate from continued post-operative intestinal failure and an associated severe immunodeficiency. We present a case of HMIA evaluated with fetal MRI and subsequently diagnosed by a combination of corroborative postnatal imaging with surgical exploration and pathological examination. (orig.)

Recent development in osmotic dehydration of fruit and vegetables: a review.

Science.gov (United States)

Chandra, Suresh; Kumari, Durvesh

2015-01-01

Osmotic dehydration of fruits and vegetables is achieved by placing the solid/semi solid, whole or in pieces, in a hypertonic solution (sugar and/or salt) with a simultaneous counter diffusion of solutes from the osmotic solution into the tissues. Osmotic dehydration is recommended as a processing method to obtain better quality of food products. Partial dehydration allows structural, nutritional, sensory, and other functional properties of the raw material to be modified. However, the food industry uptake of osmotic dehydration of foods has not been extensive as expected due to the poor understanding of the counter current flow phenomena associated with it. However, these flows are in a dynamic equilibrium with each other and significantly influence the final product in terms of preservation, nutrition, and organoleptic properties. The demand of healthy, natural, nutritious, and tasty processed food products continuously increases, not only for finished products, but also for ingredient to be included in complex foods such as ice cream, cereals, dairy, confectionaries, and bakery products.

DEHYDRATION OF LOW WATER CONTENT ETHANOL

Science.gov (United States)

Pervaporation has emerged as an economically viable alternative technology for the dehydration of organic solvents, removal of organic compounds from water and organic/organic separations. Development of a membrane system with suitable flux and selectivity characteristics plays a...

Mild dehydration modifies the cerebrovascular response to the cold pressor test.

Science.gov (United States)

Perry, Blake G; Bear, Tracey L K; Lucas, Samuel J E; Mündel, Toby

2016-01-01

The cold pressor test (CPT) is widely used in clinical practice and physiological research. It is characterized by a robust autonomic response, with associated increases in heart rate (HR), mean arterial pressure (MAP) and mean middle cerebral artery blood flow velocity (MCAv(mean)). Hydration status is not commonly reported when conducting this test, yet blood viscosity alone can modulate MCAv(mean), potentially modifying the MCAv(mean) response to the CPT. We investigated the effect of mild dehydration on the physiological response to the CPT in 10 healthy men (mean ± SD: age 28 ± 5 years; body mass 83 ± 5 kg). All participants completed two CPTs, cold water (0°C) immersion of both feet for 90 s, with the order of the euhydration and dehydration trials counterbalanced. Beat-to-beat MCAv, MAP, HR and breath-by-breath partial pressure of end-tidal CO2 (P(ET,CO2)) were measured continuously. Participants' pain perception was measured 1 min into the CPT using a visual analog scale (no pain = 0; maximal pain = 10). Dehydration significantly elevated plasma osmolality and urine specific gravity and reduced body mass (all P 0.05). After 90 s of immersion, the change in MCAv(mean) from baseline was less in the dehydration compared with the euhydration trial (change 0 ± 5 versus 7 ± 7 cm s(-1), P = 0.01), as was P(ET,CO2) (change -3 ± 2 versus 0 ± 3 mmHg, P = 0.02). Dehydration was associated with greater relative pain sensation during the CPT (7.0 ± 1.3 vs 5.8 ± 1.8, P = 0.02). Our results demonstrate that mild dehydration can modify the cerebrovascular response to the CPT, with dehydration increasing perceived pain, lowering P ET ,CO2 and, ultimately, blunting the MCAv(mean) response. © 2015 The Authors. Experimental Physiology © 2015 The Physiological Society.

Dehydration kinetics of a natural montmorillonite

International Nuclear Information System (INIS)

Andreotti, G.; Loria, A.; Mazzega, E.; Valeri, S.

1978-01-01

The dehydration mass loss of samples of montmorillonite (a layered hydrated silicate) was measured in an environment at constant temperature and at constant undersatured water vapour content. The hydration curves were analyzed by assuming the model of thermally activated, monoenergetic and first-order process compatible with the experimental results. Two dehydration kinetics with activation energies (0.21+-0.02) eV and (0.34+-0.02) eV were found that can be related to the existence of two kinds of water (structured and nonstructured) in the hydrated silicate interlayers. The possibility of an icelike structure of a fraction of the adsorbed molecules is briefly discussed. The experimental results show, furthermore, that in the investigated hydration range (up to about 10% in mass) the fraction of structured water has a maximum when the hydration is about 6%. (author)

Dehydration is an independent predictor of discharge outcome and admission cost in acute ischaemic stroke.

Science.gov (United States)

Liu, C-H; Lin, S-C; Lin, J-R; Yang, J-T; Chang, Y-J; Chang, C-H; Chang, T-Y; Huang, K-L; Ryu, S-J; Lee, T-H

2014-09-01

Our aim was to investigate the influence of admission dehydration on the discharge outcome in acute ischaemic and hemorrhagic stroke. Between January 2009 and December 2011, 4311 ischaemic and 1371 hemorrhagic stroke patients from the stroke registry of Chang Gung healthcare system were analyzed. The eligible patients were identified according to inclusion/exclusion criteria. In total, 2570 acute ischaemic and 573 acute hemorrhagic stroke patients were finally recruited. According to the blood urea nitrogen (BUN) to creatinine (Cr) ratio (BUN/Cr), these patients were divided into dehydrated (BUN/Cr ≥ 15) and non-dehydrated (BUN/Cr dehydration had higher infection rates (P = 0.006), worse discharge BI (62.8 ± 37.4 vs. 73.4 ± 32.4, P dehydration. However, acute hemorrhagic stroke with or without admission dehydration showd no difference in admission costs (P = 0.618) and discharge outcomes (BI, P = 0.058; mRS, P = 0.058). Admission dehydration is associated with worse discharge outcomes and higher admission costs in acute ischaemic stroke but not in hemorrhagic stroke. © 2014 The Author(s) European Journal of Neurology © 2014 EAN.

Use of bedside ultrasound to assess degree of dehydration in children with gastroenteritis.

Science.gov (United States)

Chen, Lei; Hsiao, Allen; Langhan, Melissa; Riera, Antonio; Santucci, Karen A

2010-10-01

Prospectively identifying children with significant dehydration from gastroenteritis is difficult in acute care settings. Previous work by our group has shown that bedside ultrasound (US) measurement of the inferior vena cava (IVC) and the aorta (Ao) diameter ratio is correlated with intravascular volume. This study was designed to validate the use of this method in the prospective identification of children with dehydration by investigating whether the IVC/Ao ratio correlated with dehydration in children with acute gastroenteritis. Another objective was to investigate the interrater reliability of the IVC/Ao measurements. A prospective observational study was carried out in a pediatric emergency department (PED) between November 2007 and June 2009. Children with acute gastroenteritis were enrolled as subjects. A pair of investigators obtained transverse images of the IVC and Ao using bedside US. The ratio of IVC and Ao diameters (IVC/Ao) was calculated. Subjects were asked to return after resolution of symptoms. The difference between the convalescent weight and ill weight was used to calculate the degree of dehydration. Greater than or equal to 5% difference was judged to be significant. Linear regression was performed with dehydration as the dependent variable and the IVC/Ao as the independent variable. Pearson's correlation coefficient was calculated to assess the degree of agreement between observers. A total of 112 subjects were enrolled. Seventy-one subjects (63%) completed follow-up. Twenty-eight subjects (39%) had significant dehydration. The linear regression model resulted in an R² value of 0.21 (p dehydration. Forty-eight paired measurements of IVC/Ao ratios were made. The Pearson correlation coefficient was 0.76.   In this pilot study the ratio of IVC to Ao diameters, as measured by bedside US, was a marginally accurate measurement of acute weight loss in children with dehydration from gastroenteritis. The technique demonstrated good interrater

Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

DEFF Research Database (Denmark)

Andersen, Michelle Fog; Bygum, Anette

2015-01-01

however not always have to be caused by angioedema but can relate to other concomitant disorders. In this report we are focusing on misdiagnosis in a patient with known hereditary angioedema, whose bleeding episode caused by idiopathic thrombocytopenic purpura was mistaken for an acute attack...

Hydrophobic Hyflon® AD/PVDF membranes for butanol dehydration via pervaporation

KAUST Repository

Jalal, Taghreed; Bettahalli Narasimha, Murthy Srivatsa; Le, Ngoc Lieu; Nunes, Suzana Pereira

2015-01-01

Novel hydrophobic Hyflon® AD /PVDF membranes were developed and investigated for n-butanol dehydration via pervaporation. The coating protocols for thin defect-free Hyflon® AD selective layer on the PVDF support was optimized. Water and n-butanol transport was measured, analyzing the effect of operating conditions. The water flux through the newly developed membranes was higher than 150 g/m2.h with selectivity for water higher than 99 wt %. The focus was on the use of Hyflon® AD as the selective layer for n-butanol dehydration. The membrane application can be extended to other solvents, supporting an effective and simple method for dehydration with hydrophobic membranes.

Hydrophobic Hyflon® AD/PVDF membranes for butanol dehydration via pervaporation

KAUST Repository

Jalal, Taghreed

2015-10-21

Novel hydrophobic Hyflon® AD /PVDF membranes were developed and investigated for n-butanol dehydration via pervaporation. The coating protocols for thin defect-free Hyflon® AD selective layer on the PVDF support was optimized. Water and n-butanol transport was measured, analyzing the effect of operating conditions. The water flux through the newly developed membranes was higher than 150 g/m2.h with selectivity for water higher than 99 wt %. The focus was on the use of Hyflon® AD as the selective layer for n-butanol dehydration. The membrane application can be extended to other solvents, supporting an effective and simple method for dehydration with hydrophobic membranes.

Hereditary Angioedema in Childhood

DEFF Research Database (Denmark)

Kjaer, Line; Bygum, Anette

2012-01-01

  Hereditary angioedema (HAE) is a rare inherited disease that is often difficult to diagnose. We report a case of a 9-year-old boy with a spontaneous mutation causing HAE, diagnosed after a life-threatening episode of angioedema of the head and upper respiratory tract after a 5-year history of r...... of recurrent skin swellings and abdominal pain leading to several hospital admissions. The aim of this report is to direct focus on this rare disease, which can be treated effectively, to diminish morbidity and mortality of children suffering from undiagnosed HAE....

Dehydration enhances pain-evoked activation in the human brain compared with rehydration.

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Ogino, Yuichi; Kakeda, Takahiro; Nakamura, Koji; Saito, Shigeru

2014-06-01

Negative effects of dehydration on the human brain and cognitive function have been reported. In this study, we examined the effects of dehydration on pain thresholds and cortical activations in response to pain, compared with rehydration with an oral rehydration solution (ORS) by functional magnetic resonance imaging. Five healthy adult men were subjected to dehydration and rehydration on 2 different days. The condition on the first day was randomly assigned to each subject. They completed a 40-minute exercise protocol using a walking machine after 12 hours of fasting under both conditions. For rehydration, the subjects consumed up to 3000 mL ORS starting from the night before the test day. After exercise, a painful stimulus (cold pressor test) was applied to the subjects' medial forearm in a magnetic resonance imaging scanning gantry, and pain-evoked brain activation was analyzed. On the rehydration day, each of the subjects consumed an average of 2040 mL (range; 1800-2500 mL) ORS. Physiological data revealed that subjects when dehydrated lost more weight from exercise than subjects when rehydrated had a larger heart rate increase, a higher tympanic temperature, and a higher urine osmolality. Subjective data revealed that the subjects reported significantly stronger thirst while dehydrated than while rehydrated with ORS, although the levels of hunger and anxiety and mood did not significantly differ between conditions. The cold pressor test robustly activated the pain-related neural network, notably the anterior cingulate cortex, insula, and thalamus. Such activations in the dehydrated subjects were greater than those in the rehydrated subjects in terms of peak and cluster, accompanied by a decrease in pain threshold (P = 0.001). Our findings suggest that dehydration brings about increased brain activity related to painful stimuli together with enhanced thirst, whereas rehydration with ORS alleviates thirst and decreases brain activity related to painful stimuli.

The management of children with gastroenteritis and dehydration in the emergency department.

Science.gov (United States)

Colletti, James E; Brown, Kathleen M; Sharieff, Ghazala Q; Barata, Isabel A; Ishimine, Paul

2010-06-01

Acute gastroenteritis is characterized by diarrhea, which may be accompanied by nausea, vomiting, fever, and abdominal pain. To review the evidence on the assessment of dehydration, methods of rehydration, and the utility of antiemetics in the child presenting with acute gastroenteritis. The evidence suggests that the three most useful predictors of 5% or more dehydration are abnormal capillary refill, abnormal skin turgor, and abnormal respiratory pattern. Studies are conflicting on whether blood urea nitrogen (BUN) or BUN/creatinine ratio correlates with dehydration, but several studies found that low serum bicarbonate combined with certain clinical parameters predicts dehydration. In most studies, oral or nasogastric rehydration with an oral rehydration solution was equally efficacious as intravenous (i.v.) rehydration. Many experts discourage the routine use of antiemetics in young children. However, children receiving ondensetron are less likely to vomit, have greater oral intake, and are less likely to be treated by intravenous rehydration. Mean length of Emergency Department (ED) stay is also less, and very few serious side effects have been reported. In the ED, dehydration is evaluated by synthesizing the historical and physical examination, and obtaining laboratory data points in select patients. No single laboratory value has been found to be accurate in predicting the degree of dehydration and this is not routinely recommended. The evidence suggests that the majority of children with mild to moderate dehydration can be treated successfully with oral rehydration therapy. Ondansetron (orally or intravenously) may be effective in decreasing the rate of vomiting, improving the success rate of oral hydration, preventing the need for i.v. hydration, and preventing the need for hospital admission in those receiving i.v. hydration. Copyright 2010. Published by Elsevier Inc.

Egg clutch dehydration induces early hatching in red-eyed treefrogs, Agalychnis callidryas

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María José Salica

2017-07-01

Full Text Available Terrestrial eggs have evolved repeatedly in tropical anurans exposing embryos to the new threat of dehydration. Red-eyed treefrogs, Agalychnis callidryas, lay eggs on plants over water. Maternally provided water allows shaded eggs in humid sites to develop to hatching without rainfall, but unshaded eggs and those in less humid sites can die from dehydration. Hatching responses of amphibian eggs to dry conditions are known from two lineages with independent origins of terrestrial eggs. Here, we experimentally tested for dehydration-induced early hatching in another lineage (Agalychnis callidryas, Phyllomedusidae, representing a third independent origin of terrestrial eggs. We also investigated how dehydration affected egg and clutch structure, and egg mortality. We collected clutches from a pond in Gamboa, Panama, and randomly allocated them to wet or dry treatments at age 1 day. Embryos hatched earlier from dry clutches than from wet clutches, accelerating hatching by ∼11%. Clutch thickness and egg diameter were affected by dehydration, diverging between treatments over time. Meanwhile, mortality in dry clutches was six-fold higher than in control clutches. With this study, early hatching responses to escape mortality from egg dehydration are now known from three anuran lineages with independent origins of terrestrial eggs, suggesting they may be widespread. Further studies are needed to understand how terrestrial amphibian eggs can respond to, or will be affected by, rapid changes in climate over the next decades.

Treatment of mild to moderate dehydration in children with oral rehydration therapy.

Science.gov (United States)

Diggins, Kristene C

2008-08-01

To review current literature on the effectiveness of oral rehydration therapy (ORT) in the treatment of mild to moderate dehydration in children. Recommendations from American Academy of Pediatrics (AAP), World Health Organization (WHO), selected research articles (2000-2006), and Internet sources. Dehydration is a common diagnosis in pediatric primary care. The literature indicates that dehydration is more often treated with intravenous (IV) therapy when ORT would be equally effective. ORT is an effective treatment for children with mild to moderate dehydration. ORT could be used more frequently rather than IV rehydration therapy. The use of ORT versus traditional methods of IV hydration matches the nursing philosophy of holistic care by enhancing client comfort and autonomy. Current practice in the treatment of mild to moderate dehydration in children does not match both AAP and WHO guidelines, which are based on evidence supporting ORT effectiveness. Treatment with ORT allows children more flexibility to be treated at home and thus decreases hospital stay. Evidence shows that the time required to initiate ORT is actually quicker than IV therapy and allows for a less stressful therapy that can be performed in the home.

Dehydration at admission increased the need for dialysis in hemolytic uremic syndrome children.

Science.gov (United States)

Balestracci, Alejandro; Martin, Sandra Mariel; Toledo, Ismael; Alvarado, Caupolican; Wainsztein, Raquel Eva

2012-08-01

Oligoanuric forms of postdiarrheal hemolytic uremic syndrome (D+ HUS) usually have more severe acute stage and higher risk of chronic sequelae than nonoligoanuric forms. During the diarrheal phase, gastrointestinal losses could lead to dehydration with pre-renal injury enhancing the risk of oligoanuric D+ HUS. Furthermore, it had been shown that intravenous volume expansion during the prodromal phase could decrease the frequency of oligoanuric renal failure. Thus, we performed this retrospective study to determine whether dehydration on admission is associated with increased need for dialysis in D+ HUS patients. Data from 137 children was reviewed, which were divided into two groups according to their hydration status at admission: normohydrated (n = 86) and dehydrated (n = 51). Laboratory parameters of the dehydrated patients reflected expected deteriorations (higher urea, higher hematocrit and lower sodium, bicarbonate, and pH) than normohydrated ones. Likewise, the dehydrated group had a higher rate of vomiting and need for dialysis (70.6 versus 40.7 %, p = 0.0007). Our data suggests that dehydration at hospital admission might represent a concomitant factor aggravating the intrinsic renal disease in D+ HUS patients increasing the need for dialysis. Therefore, the early recognition of patients at risk of D+ HUS is encouraged to guarantee a well-hydrated status.

Gastric emptying of liquids in rats dehydrated by water deprivation

Directory of Open Access Journals (Sweden)

Baracat E.C.E.

1997-01-01

Full Text Available The gastric emptying of liquids was investigated in male Wistar rats (8 to 10 weeks old, 210-300 g dehydrated by water deprivation. In this model of dehydration, weight loss, hematocrit and plasma density were significantly higher in the dehydrated animals than in the control groups after 48 and 72 h of water deprivation (P<0.05. Three test meals (saline (N = 10, water (N = 10 and a WHO rehydrating solution containing in one liter 90 mEq sodium, 20 mEq potassium, 80 mEq chloride and 30 mEq citrate (N = 10 were used to study gastric emptying following water deprivation for 24, 48 and 72 h. After 72 h, gastric emptying of the water (39.4% retention and rehydrating solution (49.2% retention test meals was significantly retarded compared to the corresponding control groups (P<0.05, Mann-Whitney test. The 72-h period of deprivation was used to study the recovery from dehydration, and water was supplied for 60 or 120 min after 67 h of deprivation. Body weight loss, hematocrit and plasma density tended to return to normal when water was offered for 120 min. In the animals supplied with water for 60 min, there was a recovery in the gastric emptying of water while the gastric emptying of the rehydrating solution was still retarded (53.1% retention; P<0.02, Kruskal-Wallis test. In the group supplied with water for 120 min, the gastric emptying of the rehydrating (51.7% retention and gluco-saline (46.0% retention solutions tended to be retarded (P = 0.04, Kruskal-Wallis test. In this model of dehydration caused by water deprivation, with little alteration in the body electrolyte content, gastric emptying of the rehydrating solution was retarded after rehydration with water. We conclude that the mechanisms whereby receptors in the duodenal mucosa can modify gastric motility are altered during dehydration caused by water deprivation

Modern approaches to the treatment of dehydration in children and adults

Directory of Open Access Journals (Sweden)

S.O. Kramariov

2017-10-01

Full Text Available Dehydration is the cause of many lethal outcomes, primarily due to diarrhea. The significant achievement that made it possible to save millions of human lives was the creation of oral rehydration salt (ORS. ORS for the treatment of dehydration in infectious diarrhea was created at the International Centre for Diarrhoeal Diseases Research, Bangladesh in the late 60s of the XX century for the treatment of cholera. Subsequently, for the treatment of non-cholera diarrhea, an improved ORS formula was developed. According to the World Health Organization, as compared with a standard ORS solution, a solution that has reduced osmolarity can decrease the need for intravenous infusion by 33 %. In addition, this solution also reduces the incidence of vomiting by 30 % and stool frequency by 20 %. 68.5 % of children with respiratory diseases, who were admitted to an infectious hospital, had signs of dehydration. In common with diarrhea, dehydration in respiratory diseases is one of the indicators of severity of the patient’s condition. Oral rehydration is currently used in different countries of the world in 99 % of children suffering from diarrhea. Timely and adequate rehydration therapy is a primary and most important part in the treatment of dehydration in both children and adults. Early use of adequate rehydration therapy is a prerequisite for the rapid and successful treatment of the disease.

Renal AA amyloidosis in a patient with hereditary complete complement C4 deficiency

Directory of Open Access Journals (Sweden)

Imed Helal

2011-01-01

Full Text Available Hereditary complete C4 deficiency has until now been reported in 30 cases only. A disturbed clearance of immune- complexes probably predisposes these individuals to systemic lupus erythematosus, other immune- complex diseases and recurrent microbial infections. We present here a 20- year- old female with hereditary complete C4 deficiency. Renal biopsy demonstrated renal AA amyloidosis. This unique case further substantiates that deficiency of classical pathway components predisposes to the development of recurrent microbial infections and that the patients may develop AA amyloidosis. Furthermore, in clinical practice, the nephrotic syndrome occurring in a patient with hereditary complete complement C4 deficiency should lead to the suspicion of renal AA amyloidosis.

The molecular basis of hereditary enamel defects in humans.

Science.gov (United States)

Wright, J T; Carrion, I A; Morris, C

2015-01-01

The formation of human enamel is highly regulated at the molecular level and involves thousands of genes. Requisites for development of this highly mineralized tissue include cell differentiation; production of a unique extracellular matrix; processing of the extracellular matrix; altering of cell function during different stages of enamel formation; cell movement and attachment; regulation of ion and protein movement; and regulation of hydration, pH, and other conditions of the microenvironment, to name just a few. Not surprising, there is a plethora of hereditary conditions with an enamel phenotype. The objective of this review was to identify the hereditary conditions listed on Online Mendelian Inheritance in Man (OMIM) that have an associated enamel phenotype and whether a causative gene has been identified. The OMIM database was searched with the terms amelogenesis, enamel, dental, and tooth, and all results were screened by 2 individuals to determine if an enamel phenotype was identified. Gene and gene product function was reviewed on OMIM and from publications identified in PubMed. The search strategy revealed 91 conditions listed in OMIM as having an enamel phenotype, and of those, 71 have a known molecular etiology or linked genetic loci. The purported protein function of those conditions with a known genetic basis included enzymes, regulatory proteins, extracellular matrix proteins, transcription factors, and transmembrane proteins. The most common enamel phenotype was a deficient amount of enamel, or enamel hypoplasia, with hypomineralization defects being reported less frequently. Knowing these molecular defects allows an initial cataloging of molecular pathways that lead to hereditary enamel defects in humans. This knowledge provides insight into the diverse molecular pathways involved in enamel formation and can be useful when searching for the genetic etiology of hereditary conditions that involve enamel. © International & American Associations for

The Molecular Basis of Hereditary Enamel Defects in Humans

Science.gov (United States)

Carrion, I.A.; Morris, C.

2015-01-01

The formation of human enamel is highly regulated at the molecular level and involves thousands of genes. Requisites for development of this highly mineralized tissue include cell differentiation; production of a unique extracellular matrix; processing of the extracellular matrix; altering of cell function during different stages of enamel formation; cell movement and attachment; regulation of ion and protein movement; and regulation of hydration, pH, and other conditions of the microenvironment, to name just a few. Not surprising, there is a plethora of hereditary conditions with an enamel phenotype. The objective of this review was to identify the hereditary conditions listed on Online Mendelian Inheritance in Man (OMIM) that have an associated enamel phenotype and whether a causative gene has been identified. The OMIM database was searched with the terms amelogenesis, enamel, dental, and tooth, and all results were screened by 2 individuals to determine if an enamel phenotype was identified. Gene and gene product function was reviewed on OMIM and from publications identified in PubMed. The search strategy revealed 91 conditions listed in OMIM as having an enamel phenotype, and of those, 71 have a known molecular etiology or linked genetic loci. The purported protein function of those conditions with a known genetic basis included enzymes, regulatory proteins, extracellular matrix proteins, transcription factors, and transmembrane proteins. The most common enamel phenotype was a deficient amount of enamel, or enamel hypoplasia, with hypomineralization defects being reported less frequently. Knowing these molecular defects allows an initial cataloging of molecular pathways that lead to hereditary enamel defects in humans. This knowledge provides insight into the diverse molecular pathways involved in enamel formation and can be useful when searching for the genetic etiology of hereditary conditions that involve enamel. PMID:25389004

Germ line mutations of mismatch repair genes in hereditary nonpolyposis colorectal cancer patients with small bowel cancer: International Society for Gastrointestinal Hereditary Tumours Collaborative Study

DEFF Research Database (Denmark)

Park, Jae-Gahb; Kim, Duck-Woo; Hong, Chang Won

2006-01-01

PURPOSE: The aim of study was to determine the clinical characteristics and mutational profiles of the mismatch repair genes in hereditary nonpolyposis colorectal cancer (HNPCC) patients with small bowel cancer (SBC). EXPERIMENTAL DESIGN: A questionnaire was mailed to 55 members of the Internatio......PURPOSE: The aim of study was to determine the clinical characteristics and mutational profiles of the mismatch repair genes in hereditary nonpolyposis colorectal cancer (HNPCC) patients with small bowel cancer (SBC). EXPERIMENTAL DESIGN: A questionnaire was mailed to 55 members...... of the International Society for Gastrointestinal Hereditary Tumours, requesting information regarding patients with HNPCC-associated SBC and germ line mismatch repair gene mutations. RESULTS: The study population consisted of 85 HNPCC patients with identified mismatch repair gene mutations and SBCs. SBC was the first...... HNPCC-associated malignancy in 14 of 41 (34.1%) patients for whom a personal history of HNPCC-associated cancers was available. The study population harbored 69 different germ line mismatch repair gene mutations, including 31 mutations in MLH1, 34 in MSH2, 3 in MSH6, and 1 in PMS2. We compared...

Psychological distress in women at risk for hereditary breast cancer: the role of family communication and perceived social support.

Science.gov (United States)

den Heijer, Mariska; Seynaeve, Caroline; Vanheusden, Kathleen; Duivenvoorden, Hugo J; Bartels, Carina C M; Menke-Pluymers, Marian B E; Tibben, Aad

2011-12-01

Hereditary breast cancer has a profound impact on individual family members and on their mutual communication and interactions. The way at-risk women cope with the threat of hereditary breast cancer may depend on the quality of family communication about hereditary breast cancer and on the perceived social support from family and friends. To examine the associations of family communication and social support with long-term psychological distress in a group of women at risk for hereditary breast cancer, who opted either for regular breast surveillance or prophylactic surgery. The study cohort consisted of 222 women at risk for hereditary breast cancer, who previously participated in a study on the psychological consequences of either regular breast cancer surveillance or prophylactic surgery. General and breast cancer specific distress, hereditary cancer-related family communication, perceived social support, and demographics were assessed. Using structural equation modelling, we found that open communication about hereditary cancer within the family was associated with less general and breast cancer specific distress. In addition, perceived support from family and friends was indirectly associated with less general and breast cancer-specific distress through open communication within the family. These findings indicate that family communication and perceived social support from friends and family are of paramount importance in the long-term adaptation to being at risk for hereditary breast cancer. Attention for these issues needs to be incorporated in the care of women at risk for hereditary breast cancer. Copyright © 2010 John Wiley & Sons, Ltd.

X-ray criteria of the differential diagnosis of hereditary tubulopathies in children

International Nuclear Information System (INIS)

Bosin, V.Yu.; Kondrina, V.V.; Mulyk, T.E.; Verbitskaya, A.I.

1995-01-01

In search for x-ray signs of skeletal involvement specific for each type of hereditary tubulopathies Vitamin D-resistant rickets, Renal tubular acidosis, Toni-Debre-Fanconi disease, the authors analyze the results of clinical and X-ray examinations of 144 children aged 2 to 16. Study demonstrated the possibility and high reliability of X-ray differential diagnosis of various forms of hereditary tubulopathies in children. 5 refs., 8 figs., 2 tabs

Spinal Exostosis in a Boy with Multiple Hereditary Exostoses

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Ali Al Kaissi

2013-01-01

Full Text Available We report on a 13-year-old boy who presented with multiple hereditary exostosis and had development of back pain, associated with neurological deficits, and was found to have exostoses in the spinal canal. Spine radiograph showed a cauliflower-like abnormality of multiple exostoses of the posterior arch (pedicle of the thoracic vertebrae (T3–5. Reformatted CT scanning revealed the simultaneous development of intra- and extraspinal osteochondromatosis of T3–5. The spinal cord was compressed by the intraspinal exostosis. Our patient was surgically treated for intraspinal exostoses and showed cessation of neurological deficits. We report what might be a rare association of spinal cord compression in a patient with multiple hereditary exostoses.

Sugar beet molasses: Properties and applications in osmotic dehydration of fruits and vegetables

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Šarić Ljubiša Ć.

2016-01-01

Full Text Available Molasses is an important by-product of sugar beet or sugar cane refining industry and it was one of the first sweeteners used in human nutrition. Sugar cane molasses has unique characteristics that can make it suitable for application in food industry, especially in confectionery and bakery products. On the other hand, sugar beet molasses has not had greater application in the human diet, primarily because of its strong smell and taste of the beet, which makes it unattractive for consumption. Since recent investigations showed that sugar beet molasses can be used as a hypertonic solution in osmotic dehydration of different materials of plant and animal origin, the objective of this work was to review recently studied sugar beet molasses in terms of its applications in osmotic dehydrations of fruits and vegetables. Previous studies showed that sugar beet molasses is an excellent medium for osmotic dehydration of fruits and vegetables (apple, carrot, plum, etc. primarily due to a high content of dry matter (80%, w/w and specific nutrient content. An important advantage of using sugar beet molasses as a hypertonic solution is an enrichment of the dehydrated material in minerals and vitamins, which penetrate from molasses into the plant tissue. Concentration of sugar beet molasses solution and immersion time had the biggest influence on the process of osmotic dehydration of fruit and vegetables, while the temperature of the solution was the least influential parameter. The effect of immersion time on the kinetics of osmotic dehydration in sugar beet molasses increases with an increase in concentration of hypertonic solution. Fruit and vegetables dehydrated in sugar beet molasses had a higher dry matter content compared to samples treated in sucrose solutions. Besides, application of sugar beet molasses in osmotic dehydration of fruits and vegetables had some other advantages such as lower cost of molasses compared to sugar and its liquid aggregate

Fatal cerebral edema and intracranial hemorrhage associated with hypernatremic dehydration

International Nuclear Information System (INIS)

Mocharla, R.; Schexnayder, S.M.; Glasier, C.M.

1997-01-01

We report neuroimaging findings of intracranial hemorrhage and cerebral edema in an infant with obtundation and seizures, initially suspected to be secondary to non-accidental trauma but finally attributed to hypernatremic dehydration. Neuroimaging findings due to hypernatremic dehydration have not been previously described in the radiologic literature. Hypernatremia should be included in the differential diagnosis of intracranial hemorrhage in the infant without evidence of nonaccidental trauma. (orig.). With 1 fig

Inactivation of Enterobacter sakazakii of dehydrated infant formula by gamma-irradiation

International Nuclear Information System (INIS)

Lee, Ju-Woon; Oh, Sang-Hee; Byun, Eui-Baek; Kim, Jae-Hun; Kim, Jang-Ho; Woon, Jae-Ho; Byun, Myung-Woo

2007-01-01

Enterobacter sakazakii has been implicated as a causal organism in a severe form of neonatal meningitis, with reported mortality rates of 20%. The population at greatest risk is immunocompromised infants of any age. Dried infant formula has been identified as a potential source of the organism in both outbreaks and sporadic cases. The objective of this study was to investigate theirradiation effect of the inactivation on E. sakazakii (ATCC 29544) of a dehydrated infant formula. The D 10 -values were 0.22-0.27 and 0.76 kGy for broth and dehydrated infant formula, respectively. The irradiation at 5.0 kGy was able to completely eliminate the E. sakazakii inoculated at 8.0 to 9.0 log CFU g -1 onto a dehydrated infant formula. There was no regrowth for all samples during the time they were stored at 10 deg. C for 6 h after rehydration. The present results indicated that a gamma-irradiation could potentially be used to inactivate E. sakazakii in a dehydrated powdered infant formula

Inactivation of Enterobacter sakazakii of dehydrated infant formula by gamma-irradiation

Energy Technology Data Exchange (ETDEWEB)

Lee, Ju-Woon; Oh, Sang-Hee [Radiation Application Research Division, Advanced Radiation Technology Institute, Korea Atomic Energy Research Institute, Jeongeup 580 185 (Korea, Republic of); Byun, Eui-Baek [Radiation Application Research Division, Advanced Radiation Technology Institute, Korea Atomic Energy Research Institute, Jeongeup 580 185 (Korea, Republic of); Graduate School of Biotechnology, Korea University, Yeongi 339-700 (Korea, Republic of); Kim, Jae-Hun; Kim, Jang-Ho [Radiation Application Research Division, Advanced Radiation Technology Institute, Korea Atomic Energy Research Institute, Jeongeup 580 185 (Korea, Republic of); Woon, Jae-Ho [Livestock Products standard Division, National Veterinary Research and Quarantine Service, Anyang 430-824 (Korea, Republic of); Byun, Myung-Woo [Radiation Application Research Division, Advanced Radiation Technology Institute, Korea Atomic Energy Research Institute, Jeongeup 580 185 (Korea, Republic of)], E-mail: mwbyun@kaeri.re.kr

2007-11-15

Enterobacter sakazakii has been implicated as a causal organism in a severe form of neonatal meningitis, with reported mortality rates of 20%. The population at greatest risk is immunocompromised infants of any age. Dried infant formula has been identified as a potential source of the organism in both outbreaks and sporadic cases. The objective of this study was to investigate theirradiation effect of the inactivation on E. sakazakii (ATCC 29544) of a dehydrated infant formula. The D{sub 10}-values were 0.22-0.27 and 0.76 kGy for broth and dehydrated infant formula, respectively. The irradiation at 5.0 kGy was able to completely eliminate the E. sakazakii inoculated at 8.0 to 9.0 log CFU g{sup -1} onto a dehydrated infant formula. There was no regrowth for all samples during the time they were stored at 10 deg. C for 6 h after rehydration. The present results indicated that a gamma-irradiation could potentially be used to inactivate E. sakazakii in a dehydrated powdered infant formula.

Inactivation of Enterobacter sakazakii of dehydrated infant formula by gamma-irradiation

Science.gov (United States)

Lee, Ju-Woon; Oh, Sang-Hee; Byun, Eui-Baek; Kim, Jae-Hun; Kim, Jang-Ho; Woon, Jae-Ho; Byun, Myung-Woo

2007-11-01

Enterobacter sakazakii has been implicated as a causal organism in a severe form of neonatal meningitis, with reported mortality rates of 20%. The population at greatest risk is immunocompromised infants of any age. Dried infant formula has been identified as a potential source of the organism in both outbreaks and sporadic cases. The objective of this study was to investigate theirradiation effect of the inactivation on E. sakazakii (ATCC 29544) of a dehydrated infant formula. The D10-values were 0.22-0.27 and 0.76 kGy for broth and dehydrated infant formula, respectively. The irradiation at 5.0 kGy was able to completely eliminate the E. sakazakii inoculated at 8.0 to 9.0 log CFU g -1 onto a dehydrated infant formula. There was no regrowth for all samples during the time they were stored at 10 °C for 6 h after rehydration. The present results indicated that a gamma-irradiation could potentially be used to inactivate E. sakazakii in a dehydrated powdered infant formula.

Performance of clinical signs in the diagnosis of dehydration in children with acute gastroenteritis.

Science.gov (United States)

Hoxha, Teuta; Xhelili, Luan; Azemi, Mehmedali; Avdiu, Muharrem; Ismaili-Jaha, Vlora; Efendija-Beqa, Urata; Grajcevci-Uka, Violeta

2015-02-01

Acute evaluation and treatment of children presenting with dehydration represent one of the most common situation in the pediatric emergency department. To identify dehydration in infants and children before treatment, a number of symptoms and clinical signs have been evaluated. The aim of the study was to describe the performance of clinical signs in detecting dehydration in children. Two hundred children aged 1 month to 5 year were involved in our prospective study. The clinical assessment consisted of the ten clinical signs of dehydration, including those recommended by WHO (World Health Organization), heart rate, and capillary refill time. Two hundred patients with diarrhea were enrolled in the study. The mean age was 15.62±9.03 months and 57.5% were male. Of these 121 had a fluid deficit of dehydration were found to have the following respective gains in percent weight at the end of illness: 2.44±0.3, 6.05± 1.01 and, 10.66± 0.28, respectively. All clinical signs were found more frequently with increasing amounts of dehydration(pdehydration (deficit dehydration (deficit 5% to 9%) was 6.5 and among those with severe dehydration (deficit >10%) the median was 9 (p<0.0001, Kruskal-Wallis test). Using stepwise linear regression and a p value of <0.05 for entry into the model, a four-variable model including sunken eyes, skin elasticity, week radial pulse, and general appearance was derived. None of the 10 findings studied, is sufficiently accurate to be used in isolation. When considered together, sunken eyes, decreased skin turgor, weak pulse and general appearance provide the best explanatory power of the physical signs considered.

Lea protein expression during cold-induced dehydration in the Arctic collembola Megaphorura arctica

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Popović Ž.D.

2011-01-01

Full Text Available The Arctic springtail Megaphorura arctica (Tullberg, 1876 employs a strategy known as cryoprotective dehydration to survive winter temperatures as low as -25°C. During cryoprotective dehydration, water is lost from the animal to ice in its surroundings as a result of the difference in vapour pressure between the animal’s supercooled body fluids and ice (Worland et al., 1998; Holmstrup and Somme, 1998. This mechanism ensures that as the habitat temperature falls, the concentration of solutes remains high enough to prevent freezing (Holmstrup et al., 2002. In M. arctica, accumulation of trehalose, a cryo/anhydro protectant, occurs in parallel with dehydration. Recent studies have identified a number of genes and cellular processes involved in cryoprotective dehydration in M. arctica (Clark et al., 2007; Clark et al., 2009; Purać et al., 2011. One of them includes late embryogenesis abundant (LEA proteins. This study, together with that of Bahrndorff et al. (2008, suggests that LEA proteins may be involved in protective dehydration in this species.

Sonographic aorta/IVC cross-sectional area index for evaluation of dehydration in children.

Science.gov (United States)

Kwon, Hyuksool; Jung, Jae Yun; Lee, Jin Hee; Kwak, Young Ho; Kim, Do Kyun; Jung, Jin Hee; Chang, Ik Wan; Kim, Kyuseok

2016-09-01

Current studies have not found sufficient evidence to encourage the use of ultrasound for assessing dehydration in children. We introduce a new sonographic parameter, the "aorta/inferior vena cava (IVC) cross-sectional area index" (Ao/IVCA) measured just inferior to the xiphoid process, for the effective evaluation of dehydration in children. This is a prospective, observational study. We enrolled children who presented to the pediatric emergency department (PED) between May 2014 and January 2015. We measured the maximum diameter of the aorta from inner wall to inner wall, and the long and short axis diameters of IVC using a convex array transducer. Ao/IVCA was calculated and compared with aorta/IVC maximal diameter index (Ao/IVCD) and the clinical dehydration scale (CDS). A total of 34 children were enrolled. We found a statistically significant correlation between Ao/IVCA and CDS (R(2) = 0.30; P dehydration. The diagnostic performance of Ao/IVCA for dehydration might be higher than that of the method that uses the maximum diameter of IVC and the aorta. Copyright © 2016 Elsevier Inc. All rights reserved.

Ventricular assist device implantation in a young patient with non-compaction cardiomyopathy and hereditary spherocytosis.

Science.gov (United States)

Huenges, Katharina; Panholzer, Bernd; Cremer, Jochen; Haneya, Assad

2018-04-01

A case of a 15-year-old female patient with acute heart failure due to non-compaction cardiomyopathy and hereditary anaemia (hereditary spherocytic elliptocytosis) requiring ventricular assist device implantation as a bridge to transplantation is presented. The possible effects of mechanical stress on erythrocytes potentially induced by mechanical circulatory support remains unclear, but it may lead to haemolytic crisis in patients suffering from hereditary anaemia. In our case, ventricular assist device therapy was feasible, and haematological complications did not occur within 6 weeks of bridging our patient to heart transplantation.

Dehydration stress memory genes of Zea mays; comparison with Arabidopsis thaliana

Science.gov (United States)

2014-01-01

Background Pre-exposing plants to diverse abiotic stresses may alter their physiological and transcriptional responses to a subsequent stress, suggesting a form of “stress memory”. Arabidopsis thaliana plants that have experienced multiple exposures to dehydration stress display transcriptional behavior suggesting “memory” from an earlier stress. Genes that respond to a first stress by up-regulating or down-regulating their transcription but in a subsequent stress provide a significantly different response define the ‘memory genes’ category. Genes responding similarly to each stress form the ‘non-memory’ category. It is unknown whether such memory responses exists in other Angiosperm lineages and whether memory is an evolutionarily conserved response to repeated dehydration stresses. Results Here, we determine the transcriptional responses of maize (Zea mays L.) plants that have experienced repeated exposures to dehydration stress in comparison with plants encountering the stress for the first time. Four distinct transcription memory response patterns similar to those displayed by A. thaliana were revealed. The most important contribution is the evidence that monocot and eudicot plants, two lineages that have diverged 140 to 200 M years ago, display similar abilities to ‘remember’ a dehydration stress and to modify their transcriptional responses, accordingly. The highly sensitive RNA-Seq analyses allowed to identify genes that function similarly in the two lineages, as well as genes that function in species-specific ways. Memory transcription patterns indicate that the transcriptional behavior of responding genes under repeated stresses is different from the behavior during an initial dehydration stress, suggesting that stress memory is a complex phenotype resulting from coordinated responses of multiple signaling pathways. Conclusions Structurally related genes displaying the same memory responses in the two species would suggest conservation

Effects of dehydration methods on quality characteristics of yellow passion fruit co-products.

Science.gov (United States)

Silva, Neiton C; Duarte, Claudio R; Barrozo, Marcos As

2017-11-01

The production and processing of fruits generate a large amount of residues, which are usually disposed of or under-used, representing losses of raw material and energy. The present paper investigates the effect of four dehydration techniques (convective, infrared, microwave and freeze-drying) on yellow passion fruit (Passiflora edulis f. flavicarpa) co-products and the influence of the main variables on moisture removal and bioactive compounds. The compounds analyzed were total phenolics, total flavonoids, ascorbic acid and pectin. The content of phenolics and flavonoids increased after dehydration in all techniques investigated and the process temperatures directly affected the ascorbic acid content. Microwave dehydration showed the best results for most bioactive compounds analyzed, if performed in suitable process conditions. However, the highest levels of pectin content were obtained by freeze-drying and convective dehydration. This study reinforces the importance of the adequate use of passion fruit co-products due to the high levels of bioactive compounds in this material. Microwave dehydration presented the best results, which indicates the potential use of this technique for a better exploitation of fruit co-products. Larger quantities of pectin were extracted from samples dehydrated through methodologies with long-time process and low temperatures, such as convective drying and freeze-drying. © 2017 Society of Chemical Industry. © 2017 Society of Chemical Industry.

Proteomics approach to identify dehydration responsive nuclear proteins from chickpea (Cicer arietinum L.).

Science.gov (United States)

Pandey, Aarti; Chakraborty, Subhra; Datta, Asis; Chakraborty, Niranjan

2008-01-01

Dehydration or water-deficit is one of the most important environmental stress factors that greatly influences plant growth and development and limits crop productivity. Plants respond and adapt to such stress by altering their cellular metabolism and activating various defense machineries. Mechanisms that operate signal perception, transduction, and downstream regulatory events provide valuable information about the underlying pathways involved in environmental stress responses. The nuclear proteins constitute a highly organized, complex network that plays diverse roles during cellular development and other physiological processes. To gain a better understanding of dehydration response in plants, we have developed a comparative nuclear proteome in a food legume, chickpea (Cicer arietinum L.). Three-week-old chickpea seedlings were subjected to progressive dehydration by withdrawing water and the changes in the nuclear proteome were examined using two-dimensional gel electrophoresis. Approximately 205 protein spots were found to be differentially regulated under dehydration. Mass spectrometry analysis allowed the identification of 147 differentially expressed proteins, presumably involved in a variety of functions including gene transcription and replication, molecular chaperones, cell signaling, and chromatin remodeling. The dehydration responsive nuclear proteome of chickpea revealed a coordinated response, which involves both the regulatory as well as the functional proteins. This study, for the first time, provides an insight into the complex metabolic network operating in the nucleus during dehydration.

DEHYDRATION OF CHEESE BY HOT AIR, MICROWAVE AND FREEZE-DRYING

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ANA RITA C. PINHO

2017-12-01

Full Text Available The objective of this work was to study the dehydration of skim cheese through different methods, in particular by hot air, microwave and freeze-drying, in order to assess which of these methods would be more suitable for the development of a new product (cheese snack. For the three processes of dehydration, several temperatures, powers and times were used, respectively. The drying time was optimized to allow the water activity of the final product to be between 0.3 and 0.4. The color and texture of the product obtained by the three processes were evaluated, and the nutritional analysis (protein, lipids, ash of the product dried by hot air at 52 ºC and by microwave at 750 W and 850 W was performed. The sensory analysis of the microwave dehydrated products was also carried out. The results obtained revealed that the temperature played a relevant role in the drying time and the hardness of the product. In the dehydration by microwave, the power of 850 W resulted in a lower drying time and a better color preservation, but in a high hardness of the samples. Among the three processes studied, the microwave drying was the fastest for the water removal from the cheese.

Charts for weight loss to detect hypernatremic dehydration and prevent formula supplementing.

Science.gov (United States)

van Dommelen, Paula; Boer, Suzanne; Unal, Sevim; van Wouwe, Jacobus P

2014-06-01

Most breast-fed newborns get the milk they need. However, very rarely milk intake is insufficient mostly as a result of poor breastfeeding techniques. Dramatic weight loss and hypernatremic dehydration may occur. Our aim was to construct charts for weight loss. A case-control study was performed. Charts with standard deviation score (SDS) lines for weight loss in the first month were constructed for 2,359 healthy breast-fed term newborns and 271 cases with breastfeeding-associated hypernatremic dehydration with serum sodium level > 149 mEq/L. Day 0 was defined as the day of birth. Many cases with (or who will develop) hypernatremic dehydration (84%; +1 SDS line) fell below the -1 SDS line at day 3, the -2 SDS line at day 4, and the -2.5 SDS line at day 5 in the chart of the healthy breast-fed newborns. Weight loss of cases with permanent residual symptoms was far below the -2.5 SDS. Already at an early age, weight loss differs between healthy breast-fed newborns and those with hypernatremic dehydration. Charts for weight loss are, therefore, useful tools to detect early, or prevent newborns from developing, breastfeeding-associated hypernatremic dehydration, and also to prevent unnecessary formula supplementing. © 2014 Wiley Periodicals, Inc.

The value of body weight measurement to assess dehydration in children.

Science.gov (United States)

Pruvost, Isabelle; Dubos, François; Chazard, Emmanuel; Hue, Valérie; Duhamel, Alain; Martinot, Alain

2013-01-01

Dehydration secondary to gastroenteritis is one of the most common reasons for office visits and hospital admissions. The indicator most commonly used to estimate dehydration status is acute weight loss. Post-illness weight gain is considered as the gold-standard to determine the true level of dehydration and is widely used to estimate weight loss in research. To determine the value of post-illness weight gain as a gold standard for acute dehydration, we conducted a prospective cohort study in which 293 children, aged 1 month to 2 years, with acute diarrhea were followed for 7 days during a 3-year period. The main outcome measures were an accurate pre-illness weight (if available within 8 days before the diarrhea), post-illness weight, and theoretical weight (predicted from the child's individual growth chart). Post-illness weight was measured for 231 (79%) and both theoretical and post-illness weights were obtained for 111 (39%). Only 62 (21%) had an accurate pre-illness weight. The correlation between post-illness and theoretical weight was excellent (0.978), but bootstrapped linear regression analysis showed that post-illness weight underestimated theoretical weight by 0.48 kg (95% CI: 0.06-0.79, pdehydration according to post-illness weight (21%) was significantly lower than the prevalence estimated by either theoretical weight (60%) or clinical assessment (66%, pdehydration. The performance of dehydration signs or scales determined by using post-illness weight as a gold standard has to be reconsidered.

Pavlodar city children's some hereditary diseases

International Nuclear Information System (INIS)

Shajmardanova, B. Kh.; Gorbach, S.V.

1997-01-01

Territory of the Pavlodar region directly adjoining to the Semipalatinsk test site is unique object for study of many year tests consequences on population health. Health worsening caused by small doses of radiation on artificial pollution background is defined. Purpose of the work is Pavlodar city children's some hereditary diseases (Downs syndrome, crack of upper lip and/or palate, hemophilia) under study of frequency dynamic of statistical data within period from 1980 by 1995. It is defined: a) tendency to growth Downs syndrome frequency has been distinctly observed beginning of the 1982; b) it is noted Downs syndrome frequency growth stabilization within period from 1988 by 1991; c) among children with Downs syndrome is distinguished low viability; d) there is rather higher correlation rate of Downs syndrome and congenial heart threshold against average statistical index; e) character of frequencies changes of crack of upper lip and/or palate has tendency to growth; f) it is defined that boys predominate among children with this disease; g) congenial crack of soft palate have being revealed as solitary thresholds of development; h) genealogy analysis of hemophilia sick reveals, that it has only hereditary character. 8 refs

Molecular biology of hereditary diabetes insipidus.

Science.gov (United States)

Fujiwara, T Mary; Bichet, Daniel G

2005-10-01

The identification, characterization, and mutational analysis of three different genes-the arginine vasopressin gene (AVP), the arginine vasopressin receptor 2 gene (AVPR2), and the vasopressin-sensitive water channel gene (aquaporin 2 [AQP2])-provide the basis for understanding of three different hereditary forms of "pure" diabetes insipidus: Neurohypophyseal diabetes insipidus, X-linked nephrogenic diabetes insipidus (NDI), and non-X-linked NDI, respectively. It is clinically useful to distinguish two types of hereditary NDI: A "pure" type characterized by loss of water only and a complex type characterized by loss of water and ions. Patients who have congenital NDI and bear mutations in the AVPR2 or AQP2 genes have a "pure" NDI phenotype with loss of water but normal conservation of sodium, potassium, chloride, and calcium. Patients who bear inactivating mutations in genes (SLC12A1, KCNJ1, CLCNKB, CLCNKA and CLCNKB in combination, or BSND) that encode the membrane proteins of the thick ascending limb of the loop of Henle have a complex polyuro-polydipsic syndrome with loss of water, sodium, chloride, calcium, magnesium, and potassium. These advances provide diagnostic and clinical tools for physicians who care for these patients.

Dehydration Process of Hofmann-Type Layered Solids

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Edilso Reguera

2013-04-01

Full Text Available In the present work the dehydration process of layered solids with formula unit M(H2O2[Ni(CN4]·nH2O, M = Ni, Co, Mn; n = 1, 2, 4 is studied using modulated thermogravimetry. The results show that water molecules need to overcome an energetic barrier (activation energy between 63 and 500 kJ/mol in order to diffuse through the interlayer region. The related kinetic parameters show a dependence on the water partial pressure. On the other hand, X-ray diffraction results provide evidence that the dehydration process is accompanied by framework collapse, limiting the structural reversibility, except for heating below 80 °C where the ordered structure remains. Removal of water molecules from the interlayer region disrupts the long-range structural order of the solid.

Analysis of dehydration and strength in elite badminton players.

Science.gov (United States)

Abián-Vicén, Javier; Del Coso, Juan; González-Millán, Cristina; Salinero, Juan José; Abián, Pablo

2012-01-01

The negative effects of dehydration on aerobic activities are well established. However, it is unknown how dehydration affects intermittent sports performance. The purpose of this study was to identify the level of dehydration in elite badminton players and its relation to muscle strength and power production. Seventy matches from the National Spanish badminton championship were analyzed (46 men's singles and 24 women's singles). Before and after each match, jump height and power production were determined during a countermovement jump on a force platform. Participants' body weight and a urine sample were also obtained before and after each match. The amount of liquid that the players drank during the match was also calculated by weighing their individual drinking bottles. Sweat rate during the game was 1.14 ± 0.46 l/h in men and 1.02 ± 0.64 l/h in women. The players rehydrated at a rate of 1.10 ± 0.55 l/h and 1.01 ± 0.44 l/h in the male and female groups respectively. Thus, the dehydration attained during the game was only 0.37 ± 0.50% in men and 0.32 ± 0.83% in women. No differences were found in any of the parameters analyzed during the vertical jump (men: from 31.82 ± 5.29 to 32.90 ± 4.49 W/kg; p>0.05, women: from 26.36 ± 4.73 to 27.25 ± 4.44 W/kg; p>0.05). Post-exercise urine samples revealed proteinuria (60.9% of cases in men and 66.7% in women), leukocyturia (men = 43.5% and women = 50.0%) and erythrocyturia (men = 50.0% and women = 21.7%). Despite a moderate sweat rate, badminton players adequately hydrated during a game and thus the dehydration attained was low. The badminton match did not cause muscle fatigue but it significantly increased the prevalence of proteinuria, leukocyturia and erythrocyturia.

Analysis of dehydration and strength in elite badminton players.

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Javier Abián-Vicén

Full Text Available BACKGROUND: The negative effects of dehydration on aerobic activities are well established. However, it is unknown how dehydration affects intermittent sports performance. The purpose of this study was to identify the level of dehydration in elite badminton players and its relation to muscle strength and power production. METHODOLOGY: Seventy matches from the National Spanish badminton championship were analyzed (46 men's singles and 24 women's singles. Before and after each match, jump height and power production were determined during a countermovement jump on a force platform. Participants' body weight and a urine sample were also obtained before and after each match. The amount of liquid that the players drank during the match was also calculated by weighing their individual drinking bottles. RESULTS AND DISCUSSION: Sweat rate during the game was 1.14 ± 0.46 l/h in men and 1.02 ± 0.64 l/h in women. The players rehydrated at a rate of 1.10 ± 0.55 l/h and 1.01 ± 0.44 l/h in the male and female groups respectively. Thus, the dehydration attained during the game was only 0.37 ± 0.50% in men and 0.32 ± 0.83% in women. No differences were found in any of the parameters analyzed during the vertical jump (men: from 31.82 ± 5.29 to 32.90 ± 4.49 W/kg; p>0.05, women: from 26.36 ± 4.73 to 27.25 ± 4.44 W/kg; p>0.05. Post-exercise urine samples revealed proteinuria (60.9% of cases in men and 66.7% in women, leukocyturia (men = 43.5% and women = 50.0% and erythrocyturia (men = 50.0% and women = 21.7%. CONCLUSIONS: Despite a moderate sweat rate, badminton players adequately hydrated during a game and thus the dehydration attained was low. The badminton match did not cause muscle fatigue but it significantly increased the prevalence of proteinuria, leukocyturia and erythrocyturia.

Dehydration-driven topotaxy in subduction zones

Science.gov (United States)

Padrón-Navarta, José Alberto; Tommasi, Andréa; Garrido, Carlos J.

2014-05-01

Mineral replacement reactions play a fundamental role in the chemistry and the strength of the lithosphere. When externally or internally derived fluids are present, interface-coupled dissolution-precipitation is the driving mechanism for such reactions [1]. One of the microstructural features of this process is a 3D arrangement of crystallographic axes across internal interfaces (topotaxy) between reactant and product phases. Dehydration reactions are a special case of mineral replacement reaction that generates a transient fluid-filled porosity. Among others, the dehydration serpentinite is of special relevance in subduction zones because of the amount of fluids involved (potentially up to 13 wt.%). Two topotatic relationships between olivine and antigorite (the serpentine mineral stable at high temperature and pressure) have been reported in partially hydrated mantle wedge xenoliths [2]. Therefore, if precursor antigorite serpentine has a strong crystallographic preferred orientation (CPO) its dehydration might result in prograde peridotite with a strong inherited CPO. However for predicting the importance of topotactic reactions for seismic anisotropy of subduction zones we also need to consider the crystallization orthopyroxene + chlorite in the prograde reaction and, more importantly, the fact that this dehydration reaction produces a transient porosity of ca. 20 % vol. that results in local fluctuations of strain during compaction and fluid migration. We address this issue by a microstructural comparison between the CPO developed in olivine, orthopyroxene and chlorite during high-pressure antigorite dehydration in piston cylinder experiments (at 750ºC and 20 kbar and 1000ºC and 30 kbar, 168 h) and that recorded in natural samples (Cerro del Almirez, Betic Cordillera, Spain). Experimentally developed CPOs are strong. Prograde minerals show a significant inheritance of the former antigorite foliation. Topotactic relations are dominated by (001)atg//(100)ol

Clinicoelectrophysiologic and magnetoresonance and tomographic investigation of hereditary and congenital diseases of the brain

International Nuclear Information System (INIS)

Kamalov, I.I.; Pikuza, O.I.; Idrisova, L.G.; Uryvskij, V.I.

1996-01-01

The combined investigation of hereditary and congenital diseases of the brain using magnetoresonance tomography is performed. The hereditary and congenital diseases of the brain accompanied by disorders of liquoroconductive tracts with medullary substance lesion are revealed. The investigation results provide timely development of the treatment tactics and rehabilitation of sick children. Refs. 3

Evaluation of the quality of hot air dehydrated onion coming from gamma radiated bulbs

International Nuclear Information System (INIS)

Elman, L.; Pezzutti, A.; Croci, C.A.

2003-01-01

The purpose of this work was to evaluate the quality of hot air dehydrated onion, as regards physical and chemical characteristics, coming from the regional product that was gamma irradiated for sprout inhibition. We worked with the onion variety Valenciana Sintetica 14. Radio inhibition was made 30 days post harvest with gamma radiation from a 60 Co source at the Centro Atomico Ezeiza-CNEA, using a dose of 60 Gy. The skin of the bulbs was manually removed and the bulbs were cut in pieces 3 mm thick and between 1 and 3 cm long. The material was dehydrated in a rotating dryer with forced air circulation at 60 C degrees, between 0.8 and 1.7 m/s air speed and at ambient relative humidity. Dehydration was made 80 days after post-irradiation. The quality of the dehydrated onion was evaluated by the following physical- chemical analysis: total solids content, pungency (indirectly measured by pyruvic acid content assessment), color, pH, carbon hydrates and sensorial analysis. All analytical determinations were made in triplicate. The results obtained showed there are no significant changes between the averages of the physical-chemical properties of the control dehydrated samples and those coming from the radio-inhibited raw matter. According to the sensorial analysis, only the color of dehydrated onion was affected by the radio inhibition process. However, and according to the panel members comment, the greatest browning degree observed in ionizing radiation treated onion seemed to result more attractive to them. It may be concluded that radio inhibited regional onion can be useful as raw matter for hot air dehydrated product. It must be remarked that its use would extend the product use by dehydration plants, thus implying an increase of their processing capacity with the corresponding financial benefit. (author)

HYPERGLYCEMIA IN GRADE III AND GRADE IV MALNUTRITION WITH DEHYDRATING GASTROENTERITIS

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Mallesh

2015-07-01

Full Text Available OBJECTIVE OF THE STUDY: To study the incidence of hyperglycemia in grade III AND IV malnutrition with no signs and some signs of dehydration. SETTING: Tertiary care center located in north Karnataka. DESIGN: Prospective observational study involving 21 children. METHODS: All the children with grade III and grade IV malnutrition presenting with diarrhea of less than 14 days duration, having no signs to some signs of dehydration. RESULTS: Hyperglycemia was observed in three of 21 children hypoglycemia was observed in six children. CONCLUSION: Although hypoglycemia is common finding in moderate and severely malnourished children with dehydrating gastroenteritis, hyperglycemia should also be suspected and treated. Long term follow up of these children is needed involving larger number of people in multicenter studies.

Dehydration/hydration of granular beds for thermal storage applications: a combined NMR and temperature study

NARCIS (Netherlands)

Donkers, P.A.J.; Pel, L.; Adan, O.C.G.

For heat/cold storage systems a granular bed of salt hydrates is studied during dehydration/hydration. The water density in these beds are measured with help of NMR. Diffusion based dehydration of a granular bed of Na2SO4·10H2O is shown to be internally limited as larger grains dehydrate faster than

Effect of dehydration on the development of collaterals in acute middle cerebral artery occlusion.

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Chang, S-W; Huang, Y-C; Lin, L-C; Yang, J-T; Weng, H-H; Tsai, Y-H; Lee, T-H

2016-03-01

Recent large series studies have demonstrated that dehydration is common amongst stroke subjects and is associated with poor outcome. However, the effects of hydration status on the development of collaterals have never been discussed. In this study, the hypothesis that hydration status is an important factor for developing collaterals after acute middle cerebral artery (MCA) infarction was tested. Eighty-seven patients with acute infarction due to occlusion of the MCA were enrolled. Two collateral markers, posterior cerebral artery (PCA) laterality and fluid-attenuated inversion recovery hyperintense vessels (HVs) were assessed from magnetic resonance imaging. Dehydration status was defined by a nitrogen to creatinine ratio ≧ of 15. The associations between dehydration status and the development of collaterals were estimated. Sixty-one of 87 patients (70.1%) were identified as dehydrated. The development of PCA laterality and HVs shows a significant difference between dehydrated and euhydrated patients. A serum nitrogen to creatinine ratio Dehydration remained an independent negative predictor for the development of PCA laterality and HVs in the multivariate analysis. Hydration status is associated with the development of collateral flow after acute MCA occlusion. This preliminary study provides an imaging clue that hydration status and early hydration therapy could be important for acute stroke management. © 2016 EAN.

ATG18 and FAB1 are involved in dehydration stress tolerance in Saccharomyces cerevisiae.

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López-Martínez, Gema; Margalef-Català, Mar; Salinas, Francisco; Liti, Gianni; Cordero-Otero, Ricardo

2015-01-01

Recently, different dehydration-based technologies have been evaluated for the purpose of cell and tissue preservation. Although some early results have been promising, they have not satisfied the requirements for large-scale applications. The long experience of using quantitative trait loci (QTLs) with the yeast Saccharomyces cerevisiae has proven to be a good model organism for studying the link between complex phenotypes and DNA variations. Here, we use QTL analysis as a tool for identifying the specific yeast traits involved in dehydration stress tolerance. Three hybrids obtained from stable haploids and sequenced in the Saccharomyces Genome Resequencing Project showed intermediate dehydration tolerance in most cases. The dehydration resistance trait of 96 segregants from each hybrid was quantified. A smooth, continuous distribution of the anhydrobiosis tolerance trait was found, suggesting that this trait is determined by multiple QTLs. Therefore, we carried out a QTL analysis to identify the determinants of this dehydration tolerance trait at the genomic level. Among the genes identified after reciprocal hemizygosity assays, RSM22, ATG18 and DBR1 had not been referenced in previous studies. We report new phenotypes for these genes using a previously validated test. Finally, our data illustrates the power of this approach in the investigation of the complex cell dehydration phenotype.

ATG18 and FAB1 are involved in dehydration stress tolerance in Saccharomyces cerevisiae.

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Gema López-Martínez

Full Text Available Recently, different dehydration-based technologies have been evaluated for the purpose of cell and tissue preservation. Although some early results have been promising, they have not satisfied the requirements for large-scale applications. The long experience of using quantitative trait loci (QTLs with the yeast Saccharomyces cerevisiae has proven to be a good model organism for studying the link between complex phenotypes and DNA variations. Here, we use QTL analysis as a tool for identifying the specific yeast traits involved in dehydration stress tolerance. Three hybrids obtained from stable haploids and sequenced in the Saccharomyces Genome Resequencing Project showed intermediate dehydration tolerance in most cases. The dehydration resistance trait of 96 segregants from each hybrid was quantified. A smooth, continuous distribution of the anhydrobiosis tolerance trait was found, suggesting that this trait is determined by multiple QTLs. Therefore, we carried out a QTL analysis to identify the determinants of this dehydration tolerance trait at the genomic level. Among the genes identified after reciprocal hemizygosity assays, RSM22, ATG18 and DBR1 had not been referenced in previous studies. We report new phenotypes for these genes using a previously validated test. Finally, our data illustrates the power of this approach in the investigation of the complex cell dehydration phenotype.

Assessing Dehydration Employing End-Tidal Carbon Dioxide in Children With Vomiting and Diarrhea.

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Freedman, Stephen B; Johnson, David W; Nettel-Aguirre, Alberto; Mikrogianakis, Angelo; Williamson-Urquhart, Sarah; Monfries, Nicholas; Cheng, Adam

2017-05-23

Serum bicarbonate reflects dehydration severity in children with gastroenteritis. Previous work in children receiving intravenous rehydration has correlated end-tidal carbon dioxide (EtCO2) with serum bicarbonate. We evaluated whether EtCO2 predicts weight change in children with vomiting and/or diarrhea. A prospective cohort study was conducted. Eligible children were 3 months to 10 years old and presented for emergency department (ED) care because of vomiting and/or diarrhea. End-tidal carbon dioxide measurements were performed after triage. The diagnostic standard was weight change determined from serial measurements after symptom resolution. A receiver operating characteristic curve was constructed to identify a cut-point to predict 5% or more dehydration. In total, 195 children were enrolled. Among the 169 (87%) with EtCO2 measurements, the median (interquartile range [IQR]) was 30.4 (27.8 to 33.1). One hundred fifty-eight had repeat weights performed at home; the median (IQR) weight change from ED presentation to well weight was +0.06 (-0.14 to +0.30) or +0.72% (-1.2% to +2.1%). Sixteen percent (25/158) had 3% or more and 4% (6/158) had 5% or more weight gain (ie, percent dehydration). One hundred sixteen (60%) completed home follow-up and had acceptable EtCO2 recordings. Receiver operating curve analysis revealed an area under the curve of 0.34 (95% confidence interval, 0.06 to 0.62) for EtCO2 as a predictor of 5% or more dehydration. The limited accuracy of EtCO2 measurement to predict 5% or more dehydration precludes its use as a tool to assess dehydration severity in children. End-tidal carbon dioxide monitoring does not have the ability to identify those children with 5% or more dehydration in a cohort of children with vomiting and/or diarrhea presenting for ED care.

Dehydration kinetics of talc and 10 Å phase: Consequences for subduction zone seismicity

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Chollet, Mélanie; Daniel, Isabelle; Koga, Kenneth T.; Petitgirard, Sylvain; Morard, Guillaume

2009-06-01

The process of dehydration embrittlement is usually proposed as an explanation for the presence of intermediate-depth earthquakes in subduction zones. It assumes that the release of water by hydrous mineral breakdown is fast enough to provoke brittle failure. We performed high-pressure, high-temperature, dehydration experiments of talc and 10 Å phase coupled with in situ measurement of reaction kinetics using synchrotron X-ray diffraction. Newly developed, X-ray transparent, pressure-sealed, titanium capsule ensured a closed thermochemical environment. From isothermal kinetics data fitted to the Avrami's equation and from the texture of reaction products, we conclude that dehydration rates of these minerals are limited by diffusion. Predicted minimum rates of fluid release range from 10 - 4 to 9 × 10 - 6 m 3fluid m - 3 rock s - 1 , and are fast enough to provoke hydraulic rupture since Maxwell relaxation rate of rocks relevant of subduction zones are slower than the rate of fluid release. These rates are comparable between talc, 10 Å phase and antigorite also [Perrillat, J.-P., Daniel, I., Koga, K.T., Reynard, B., Cardon, H., Crichton, W.A., 2005. Kinetics of antigorite dehydration: a real-time X-ray diffraction study. Earth Planet. Sci. Lett. 236, 899-913]. Consequently, we suggest that the dehydration of hydrous minerals may eventually be fast enough to trigger the intermediate-depth earthquakes, and that the deepest among intermediate-depth earthquakes may actually locate the limits for dehydration of hydrous minerals in the downgoing lithosphere.

Dehydration of Older Patients in Institutional Care and the Home Environment.

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Lešnik, Amadeus; Piko, Nejc; Železnik, Danica; Bevc, Sebastjan

2017-11-01

Dehydration in older adults is an important clinical problem associated with more comorbidities, longer hospital stays, and higher mortality rates. However, in daily clinical practice, no single gold standard marker of hydration status in older adults is available. The aim of the current study was to define the fluid balance status in older adults residing in institutional care or the home. Four hundred ten patients (192 from institutional care and 218 from home care) 65 and older from the region of lower Styria (Slovenia) were included in the study. Serum osmolality, electrolytes, and blood urea nitrogen to creatinine (BUN:Cr) ratio were used to identify dehydration. Statistically significant differences were found between groups in serum osmolality and BUN:Cr ratio. Moreover, dehydration (defined as increased serum osmolality) was significantly more common in patients in institutional care than home care (51% versus 41.3%, respectively). The results confirm that dehydration is a common clinical problem in older adults, especially in those from institutional care. Although many methods of determining hydration status in older adults have been proposed, no gold standard exists, making hydration evaluation difficult in this population. [Res Gerontol Nurs. 2017; 10(6):260-266.]. Copyright 2017, SLACK Incorporated.

Neonatal dehydrering (tørstefeber) hos nyfødte børn

DEFF Research Database (Denmark)

Zachariassen, Gitte; Juvonen, Pekka

2002-01-01

The aim was to identify characteristics in infants suffering from dehydration caused by breast-feeding malnutrition over a period of 21 months in South Denmark.......The aim was to identify characteristics in infants suffering from dehydration caused by breast-feeding malnutrition over a period of 21 months in South Denmark....

Gastrointestinal bleeding in patients with hereditary hemorrhagic telangiectasia

DEFF Research Database (Denmark)

Kjeldsen, A D; Kjeldsen, J

2000-01-01

Gastrointestinal bleeding occurs in a number of patients with hereditary hemorrhagic telangiectasia (HHT) and may lead to a high transfusion need. The aim of this study was to estimate the occurrence and severity of gastrointestinal bleeding in a geographically well defined HHT population....

Sulindac treatment in hereditary non-pollyposis colorectal cancer

NARCIS (Netherlands)

Rijcken, Fleur E. M.; Hollema, Harry; van der Zee, Ate G. J.; van der Sluis, Tineke; Ek, Wytske Boersma-van; Kleibeuker, Jan H.

Non-steroidal anti-inflammatory drugs, e.g. sulindac have been extensively studied for chemoprevention in familial adenomatous polyposis, but not in hereditary non-polyposis colorectal cancer (HNPCC). We evaluated these effects in HNPCC using surrogate end-points for cancer risk. In a randomised

HIGH PERMEABILITY MEMBRANES FOR THE DEHYDRATION OF LOW WATER CONTENT ETHANOL BY PERVAPORATION

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Energy efficient dehydration of low water content ethanol is a challenge for the sustainable production of fuel-grade ethanol. Pervaporative membrane dehydration using a recently developed hydrophilic polymer membrane formulation consisting of a cross-linked mixture of poly(allyl...

Diagnosing clinically significant dehydration in children with acute gastroenteritis using noninvasive methods: a meta-analysis.

Science.gov (United States)

Freedman, Stephen B; Vandermeer, Ben; Milne, Andrea; Hartling, Lisa

2015-04-01

To determine the most accurate, noninvasive method of assessing dehydration. The following data sources were searched: electronic databases, gray literature, scientific meetings, reference lists, and authors of unpublished studies. Eligible studies were comparative outpatient evaluations that used an accepted reference standard and were conducted in developed countries in children aged Dehydration Scale (CDS), the "Gorelick" score, and unstructured physician assessment were evaluated in 3, 2, and 5 studies, respectively. Bedside ultrasound, capillary digital videography, and urinary measurements were each evaluated in one study. The CDS had a positive likelihood ratio (LR) range of 1.87-11.79 and a negative LR range of 0.30-0.71 to predict 6% dehydration. When combined with the 4-item Gorelick Score, the positive LR was 1.93 (95% CI 1.07-3.49) and negative LR was of 0.40 (95% CI 0.24-0.68). Unstructured dehydration assessment had a pooled positive LR of 2.13 (95% CI 1.33-3.44) and negative LR of 0.48 (95% CI 0.28-0.82) to detect ≥ 5% dehydration. Overall, the clinical scales evaluated provide some improved diagnostic accuracy. However, test characteristics indicate that their ability to identify children both with and without dehydration is suboptimal. Current evidence does not support the routine use of ultrasound or urinalysis to determine dehydration severity. Copyright © 2015 Elsevier Inc. All rights reserved.

Improving the diffraction of apoA-IV crystals through extreme dehydration

International Nuclear Information System (INIS)

Deng, Xiaodi; Davidson, W. Sean; Thompson, Thomas B.

2011-01-01

Apolipoprotein A-IV crystals consisted of a long unit-cell edge (540 Å) with a high mosaic spread, making them intractable for X-ray diffraction analysis. Extreme dehydration in 60% PEG 3350 was utilized as a post-crystallization treatment as well a screening method to significantly sharpen the mosaic spread and increase the overall resolution of diffraction. Apolipoproteins are the protein component of high-density lipoproteins (HDL), which are necessary for mobilizing lipid-like molecules throughout the body. Apolipoproteins undergo self-association, especially at higher concentrations, making them difficult to crystallize. Here, the crystallization and diffraction of the core fragment of apolipoprotein A-IV (apoA-IV), consisting of residues 64–335, is presented. ApoA-IV 64–335 crystallized readily in a variety of hexagonal (P6) morphologies with similar unit-cell parameters, all containing a long axis of nearly 550 Å in length. Preliminary diffraction experiments with the different crystal morphologies all resulted in limited streaky diffraction to 3.5 Å resolution. Crystal dehydration was applied to the different morphologies with variable success and was also used as a quality indicator of crystal-growth conditions. The results show that the morphologies that withstood the most extreme dehydration conditions showed the greatest improvement in diffraction. One morphology in particular was able to withstand dehydration in 60% PEG 3350 for over 12 h, which resulted in well defined intensities to 2.7 Å resolution. These results suggest that the approach of integrating dehydration with variation in crystal-growth conditions might be a general technique to optimize diffraction

Survey of Clinical Evidence in Newborns with Hypernatremic Dehydration

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Hasan Boskabadi

2016-01-01

Full Text Available Background & objectives: Weight loss and hypernatremic dehydration is a common problem in infants with inadequate nutrition. Determination of risk factors and clinical characteristics of these neonates may help to prevent or early detection of hypernatremia. This study designed to determine the prevalence of clinical signs and risk factors for hypernatremic dehydration. Methods: This study is a sectional descriptive study that conducted on 418 newborns during 1385 to 1393 AHS that referred to Ghaem hospital in Mashhad. The hypernatremic group consisted of 197 neonates with serum sodium level higher than 150 mg/dl and 221 isonatremic newborns with sodium level less than 150 mmol/L were included as controls. Both groups were compared in terms of clinical symptoms of dehydration and maternal risk factors. All data were recorded and coded. Data analysis performed using paired T test and chi square tests by SPSS 16 software. Results: In the present study, 2.8% of infants were hypernatremic. The most common symptoms were neonatal jaundice (62.2%, hyperthermia (25.8%, lethargy (23.6%, agitation (20.8 % and mucous membranes dryness (17.2%. Problems in pregnancy (p=0.002, inappropriate feeding techniques (p=0.000, the first lactation duration (p=0.022 and frequency per day (p=0.000 had statistically significant difference between case and control groups. Conclusion: Prevalence of hypernatremic dehydrationis considerable in our study and identifying the risk factors may help to reduce its incidence or exacerbation. Weight loss, especially in newborns having jaundice, hyperthermia and irritability, may be helpful in the early diagnosis of hypernatremic dehydration

Airway Surface Dehydration Aggravates Cigarette Smoke-Induced Hallmarks of COPD in Mice.

Science.gov (United States)

Seys, Leen J M; Verhamme, Fien M; Dupont, Lisa L; Desauter, Elke; Duerr, Julia; Seyhan Agircan, Ayca; Conickx, Griet; Joos, Guy F; Brusselle, Guy G; Mall, Marcus A; Bracke, Ken R

2015-01-01

Airway surface dehydration, caused by an imbalance between secretion and absorption of ions and fluid across the epithelium and/or increased epithelial mucin secretion, impairs mucociliary clearance. Recent evidence suggests that this mechanism may be implicated in chronic obstructive pulmonary disease (COPD). However, the role of airway surface dehydration in the pathogenesis of cigarette smoke (CS)-induced COPD remains unknown. We aimed to investigate in vivo the effect of airway surface dehydration on several CS-induced hallmarks of COPD in mice with airway-specific overexpression of the β-subunit of the epithelial Na⁺ channel (βENaC). βENaC-Tg mice and wild-type (WT) littermates were exposed to air or CS for 4 or 8 weeks. Pathological hallmarks of COPD, including goblet cell metaplasia, mucin expression, pulmonary inflammation, lymphoid follicles, emphysema and airway wall remodelling were determined and lung function was measured. Airway surface dehydration in βENaC-Tg mice aggravated CS-induced airway inflammation, mucin expression and destruction of alveolar walls and accelerated the formation of pulmonary lymphoid follicles. Moreover, lung function measurements demonstrated an increased compliance and total lung capacity and a lower resistance and hysteresis in βENaC-Tg mice, compared to WT mice. CS exposure further altered lung function measurements. We conclude that airway surface dehydration is a risk factor that aggravates CS-induced hallmarks of COPD.

Management of acute attacks of hereditary angioedema: potential role of icatibant.

Science.gov (United States)

Longhurst, Hilary J

2010-09-07

Icatibant (Firazyr(®)) is a novel subcutaneous treatment recently licensed in the European Union for acute hereditary angioedema. Hereditary angioedema, resulting from inherited partial C1 inhibitor deficiency, is a disabling condition characterized by intermittent episodes of bradykinin-mediated angioedema. Icatibant blocks bradykinin B2 receptors, attenutating the episode. Randomized double-blind, placebo-controlled trials of icatibant, showed significant superiority over oral tranexamic acid in 74 European patients and a trend to improvement in a similar US trial comparing icatibant with placebo in 55 patients. Outcomes for several endpoints did not reach significance in the US trial, perhaps because of low participant numbers and confounding factors: a further trial is planned. Open label studies have shown benefit in multiple treatments for attacks at all sites. Approximately 10% of patients require a second dose for re-emergent symptoms, usually 10 to 27 hours after the initial treatment. Its subcutaneous route of administration, good tolerability and novel mode of action make icatibant a promising addition to the limited repertoire of treatments for hereditary angioedema.

Management of acute attacks of hereditary angioedema: potential role of icatibant

Science.gov (United States)

Longhurst, Hilary J

2010-01-01

Icatibant (Firazyr®) is a novel subcutaneous treatment recently licensed in the European Union for acute hereditary angioedema. Hereditary angioedema, resulting from inherited partial C1 inhibitor deficiency, is a disabling condition characterized by intermittent episodes of bradykinin-mediated angioedema. Icatibant blocks bradykinin B2 receptors, attenutating the episode. Randomized double-blind, placebo-controlled trials of icatibant, showed significant superiority over oral tranexamic acid in 74 European patients and a trend to improvement in a similar US trial comparing icatibant with placebo in 55 patients. Outcomes for several endpoints did not reach significance in the US trial, perhaps because of low participant numbers and confounding factors: a further trial is planned. Open label studies have shown benefit in multiple treatments for attacks at all sites. Approximately 10% of patients require a second dose for re-emergent symptoms, usually 10 to 27 hours after the initial treatment. Its subcutaneous route of administration, good tolerability and novel mode of action make icatibant a promising addition to the limited repertoire of treatments for hereditary angioedema. PMID:20859548

Hereditary Colorectal Cancer (CRC Program in Latvia

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Irmejs Arvids

2003-12-01

Full Text Available Abstract Introduction The aim of the study is to evaluate the incidence and phenotype - genotype characteristics of hereditary colorectal cancer syndromes in Latvia in order to develop the basis of clinical management for patients and their relatives affected by these syndromes. Materials and methods From 02/1999-09/2002 in several hospitals in Latvia cancer family histories were collected from 865 patients with CRC. In families suspected of having a history consistent with a hereditary colorectal cancer syndrome, DNA testing for MLH1, MSH2 and MSH6 genes was performed. In addition immunohistochemical (IH examination of the normal and cancer tissue from large bowel tumors for MSH2 and MSH6 protein expression was performed prior to DNA analysis. Results From the 865 CRC cases only 3 (0.35% pedigrees fulfilled the Amsterdam II criteria of Hereditary Nonpolyposis Colorectal Cancer (HNPCC and 15 cases (1.73% were suspected of HNPCC. In 69 cases (8% with a cancer family aggregation (CFA were identified. Thus far 27 IH analyses have been performed and in 3 cancers homogenous lack of MSH2 or MSH6 protein expression was found. In one of these cases a mutation in MSH6 was identified. In 18 patients suspected of HNPCC or of matching the Amsterdam II criteria, denaturing high performance liquid chromatography (DHPLC followed by DNA sequencing of any heteroduplexes of the 35 exons comprising both MLH1 and MSH2 was performed revealing 3 mutations. For all of kindreds diagnosed definitively or with a high probability of being an HNPCC family appropriate recommendations concerning prophylactic measures, surveillance and treatment were provided in written form. Conclusions Existing pedigree/clinical data suggest that in Latvia the frequency of HNPCC is around 2% of consecutive colorectal cancer patients. It is crucial that genetic counseling is an integral part of cancer family syndrome management.

Remedial technology for contaminated natural gas dehydrator sites

International Nuclear Information System (INIS)

Prosen, B.J.; Korreck, W.M.; Armstrong, J.M.

1991-01-01

Ground water and soil contamination at many of Michigan's oil and gas well sites has been attributed to natural gas dehydration processes. Since water was once thought to be the only by-product from the dehydration process, condensate from the process was discharged directly to the ground for several years. This condensate was later found to contain benzene, toluene, ethyl-benzene, and xylenes (BTEX), and the process of discharging condensate to the ground was stopped. Many oil and gas well sites had become impacted from the process during this time. Although condensate is no longer discharged to the ground, soil and water contamination still remains at many of these sites. In the last few years, the Michigan Department of Natural Resources has targeted over 90 well sites for assessment of contamination associated with gas dehydration. The results of many of these assessments indicate that soil and ground water have been impacted, and the State of Michigan has mandated cleanup of these sites. Remedial technologies which have been used to contain and/or clean up the sites include excavation and product removal, soil venting, purge and treat, and enhanced biodegradation. This paper is a discussion of the technology, implementation, and results from each of these methods

EPR study on gamma-irradiated fruits dehydrated via osmosis

International Nuclear Information System (INIS)

Yordanov, N.D.; Aleksieva, K.

2007-01-01

The shape and time stability of the electron paramagnetic resonance (EPR) spectra of non- and γ-irradiated papaya, melon, cherry and fig samples dehydrated via osmosis are reported. It is shown that non-irradiated samples are generally EPR silent whereas γ-irradiated exhibit 'sugar-like' EPR spectra. The recorded EPR spectra are monitored for a period of 7 months after irradiation (stored at low humidity and in the dark). The results suggest longer period of unambiguous identification of the radiation processing of osmose dehydrated fruits. Therefore, the Protocol EN 13708,2001 issued by CEN is fully applicable for the studied fruit samples

EPR study on gamma-irradiated fruits dehydrated via osmosis

Science.gov (United States)

Yordanov, N. D.; Aleksieva, K.

2007-06-01

The shape and time stability of the electron paramagnetic resonance (EPR) spectra of non- and γ-irradiated papaya, melon, cherry and fig samples dehydrated via osmosis are reported. It is shown that non-irradiated samples are generally EPR silent whereas γ-irradiated exhibit "sugar-like" EPR spectra. The recorded EPR spectra are monitored for a period of 7 months after irradiation (stored at low humidity and in the dark). The results suggest longer period of unambiguous identification of the radiation processing of osmose dehydrated fruits. Therefore, the Protocol EN 13708,2001 issued by CEN is fully applicable for the studied fruit samples.

Cellular Pathways of Hereditary Spastic Paraplegia*

OpenAIRE

Blackstone, Craig

2012-01-01

Human voluntary movement is controlled by the pyramidal motor system, a long CNS pathway comprising corticospinal and lower motor neurons. Hereditary spastic paraplegias (HSPs) are a large, genetically diverse group of inherited neurologic disorders characterized by a length-dependent distal axonopathy of the corticospinal tracts, resulting in lower limb spasticity and weakness. A range of studies are converging on alterations in the shaping of organelles, particularly the endoplasmic reticul...

Physicochemical characteristics of guava “Paluma” submitted to osmotic dehydration

OpenAIRE

Roselene Ferreira Oliveira; Lia Mara Moterlle; Edmar Clemente

2014-01-01

The aim of this work was to evaluate the conservation post process osmotic of guava stored temperature at 5oC. Guava (Psidium guajava L.), red variety “Paluma” minimally processed by mild osmotic dehydration, were packaged in polyethylene terephthalate (PET) and stored temperature at 5ºC. Non-treated guava, packed in PET trays, was used as control. The treatment used was osmotic dehydration in sucrose syrup at 60ºBrix and physicochemical determinations were pH, total soluble solids (TSS), tot...

Evaluation of WHO Criteria to Determine Degree of Dehydration in Children with Acute Diarrhea

OpenAIRE

Sari, Suprawita; Supriatmo; Margaretha, S. L.; Nafianti, S.; Hasibuan, B.; Sinuhaji, A. B.

2005-01-01

Objective To evaluate the diagnostic accuracy and agreement between the 1980 and 1990 WHO criteria for determining the degree of dehydration in children with acute diarrhea. Methods This prospective study was conducted in two hospitals from October 2002 to February 2003. Clinical signs of dehydration all patients were recorded. The degree of dehydration based on the 1980 and 1990 WHO criteria was determined and compared with fluid deficit measured by the difference of body weight on admiss...

Performance of Clinical Signs in the Diagnosis of Dehydration in Children with Acute Gastroenteritis

OpenAIRE

Hoxha, Teuta; Xhelili, Luan; Azemi, Mehmedali; Avdiu, Muharrem; Ismaili-Jaha, Vlora; Efendija-Beqa, Urata; Grajcevci-Uka, Violeta

2015-01-01

Background: Acute evaluation and treatment of children presenting with dehydration represent one of the most common situation in the pediatric emergency department. To identify dehydration in infants and children before treatment, a number of symptoms and clinical signs have been evaluated. The aim of the study was to describe the performance of clinical signs in detecting dehydration in children. Methods: Two hundred children aged 1 month to 5 year were involved in our prospective study. The...

Hereditary iron and copper deposition

DEFF Research Database (Denmark)

Aaseth, Jan; Flaten, Trond Peder; Andersen, Ole

2007-01-01

Hereditary deposition of iron (primary haemochromatosis) or copper (Wilson's disease) are autosomal recessive metabolic disease characterized by progressive liver pathology and subsequent involvement of various other organs. The prevalence of primary haemochromatosis is approximately 0.5%, about......, they may be inadequate in patients diagnosed so late that extensive body deposits of metal have been developed. The main research needs in this field are to further clarify molecular mechanisms of disease progression and to develop new chelators that are more effective and less toxic than those presently...

Hereditary hemochromatosis: genetic complexity and new diagnostic approaches.

NARCIS (Netherlands)

Swinkels, D.W.; Janssen, M.C.H.; Bergmans, J.; Marx, J.J.M.

2006-01-01

Since the discovery of the hemochromatosis gene (HFE) in 1996, several novel gene defects have been detected, explaining the mechanism and diversity of iron-overload diseases. At least 4 main types of hereditary hemochromatosis (HH) have been identified. Surprisingly, genes involved in HH encode for

Biphasic single-reactor process for dehydration of xylose and hydrogenation of produced furfural

NARCIS (Netherlands)

Ordomskiy, V.; Schouten, J.C.; Schaaf, van der J.; Nijhuis, T.A.

2013-01-01

The processes of xylose dehydration and the consecutive furfural hydrogenation have been combined in a single biphasic reactor. The dehydration was studied over Amberlyst-15 and the hydrogenation over a hydrophobic Ru/C catalyst. 1-Butanol, 2-methyltetrahydrofuran and cyclohexane were used as

Red cell 2,3-diphosphoglycerate levels in children with hereditary haemolytic anaemias.

Science.gov (United States)

Haidas, S; Zannos-Mariolea, L; Matsaniotis, N

1975-12-01

The role of red cell 2,3-diphosphoglycerate (2,3-DPG) in increasing the availability of haemoglobin oxygen in neonatal jaundice and hereditary haemolytic anaemias was investigated. Measurements of 2,3-DPG were carried out on 58 normal children and six normal adults, 18 full-term newborns with neonatal jaundice and 57 cases (51 children and six adults) with hereditary haemolytic anaemias. In normal children and adults, with a mean haemoglobin of 12.69 g/dl, mean 2,3-DPG was 14.90 mumol/g Hb. In jaundiced newborns with a mean haemoglobin of 16.04 g/dl mean 2,3-DPG levels were 14.51 mumol/g Hb, i.e. normal. 2,3-DPG levels were increased in patients with beta-thalassaemia major, alpha-thalassaemia, sickle-cell disease, favism, hereditary spherocytosis and in heterozygotes for beta-thalassaemia with increased haemoglobin F. In heterozygotes for beta-thalassaemia with increased haemoglobin A2 only and in sickle cell trait 2,3-DPG levels were normal.

Isothermal dehydration of thin films of water and sugar solutions

Energy Technology Data Exchange (ETDEWEB)

Heyd, R. [Centre de Recherche sur la Matière Divisée, University of Orleans and CNRS, rue de la Férollerie 1B, 45071 Orléans Cedex 2 (France); Rampino, A. [Centre de Recherche sur la Matière Divisée, University of Orleans and CNRS, rue de la Férollerie 1B, 45071 Orléans Cedex 2 (France); Laboratory of Physical and Macromolecular Chemistry, University of Trieste, Via Giorgieri 1, 34127 Trieste (Italy); Bellich, B.; Elisei, E. [Laboratory of Physical and Macromolecular Chemistry, University of Trieste, Via Giorgieri 1, 34127 Trieste (Italy); Cesàro, A. [Laboratory of Physical and Macromolecular Chemistry, University of Trieste, Via Giorgieri 1, 34127 Trieste (Italy); Elettra Sincrotrone Trieste, Area Science Park, I-34149 Trieste (Italy); Saboungi, M.-L. [Centre de Recherche sur la Matière Divisée, University of Orleans and CNRS, rue de la Férollerie 1B, 45071 Orléans Cedex 2 (France); Institut de Minéralogie, de Physique des Matériaux, et de Cosmochimie (IMPMC), Sorbonne Univ-UPMC, Univ Paris 06, UMR CNRS 7590, Museum National d’Histoire Naturelle, IRD UMR 206, 4 Place Jussieu, F-75005 Paris (France)

2014-03-28

The process of quasi-isothermal dehydration of thin films of pure water and aqueous sugar solutions is investigated with a dual experimental and theoretical approach. A nanoporous paper disk with a homogeneous internal structure was used as a substrate. This experimental set-up makes it possible to gather thermodynamic data under well-defined conditions, develop a numerical model, and extract needed information about the dehydration process, in particular the water activity. It is found that the temperature evolution of the pure water film is not strictly isothermal during the drying process, possibly due to the influence of water diffusion through the cellulose web of the substrate. The role of sugar is clearly detectable and its influence on the dehydration process can be identified. At the end of the drying process, trehalose molecules slow down the diffusion of water molecules through the substrate in a more pronounced way than do the glucose molecules.

Dehydrating process experiment on spent ion-exchange resin sludge by Funda Filter

International Nuclear Information System (INIS)

Hasegawa, Tatsuo; Ishino, Kazuyuki

1977-01-01

In nuclear power plants, Funda Filters are employed to dehydrate spent powdery ion-exchange resin sludge. The Funda Filter is very effective for eliminating small rust components contained in spent powdery resin slurry; however, in the drying process, the complete drying of spent powdery resin is very difficult because the filter cake of resin on the horizontal filter leaf is likely to crack and let out steam and hot air through the cracks. This paper deals with the results of experiments conducted to clarify the detailed phenomena of dehydration so the above problem could be solved. The above experiments were made on the precoating and drying of granular ion-exchange resin slurry that had not yet been put to practical use. The experiments were composed of one fundamental and one operational stage. In the fundamental experiment, the dehydration properties and dehydration mechanism of resins were made clear, and the most effective operational method was established through the operational experiments conducted using large-scale Funda Filter test equipment under various conditions. (auth.)

Lesion dehydration rate changes with the surface layer thickness during enamel remineralization

Science.gov (United States)

Chang, Nai-Yuan N.; Jew, Jamison M.; Fried, Daniel

2018-02-01

A transparent highly mineralized outer surface zone is formed on caries lesions during remineralization that reduces the permeability to water and plaque generated acids. However, it has not been established how thick the surface zone should be to inhibit the penetration of these fluids. Near-IR (NIR) reflectance coupled with dehydration can be used to measure changes in the fluid permeability of lesions in enamel and dentin. Based on our previous studies, we postulate that there is a strong correlation between the surface layer thickness and the rate of dehydration. In this study, the rates of dehydration for simulated lesions in enamel with varying remineralization durations were measured. Reflectance imaging at NIR wavelengths from 1400-2300 nm, which coincides with higher water absorption and manifests the greatest sensitivity to contrast changes during dehydration measurements, was used to image simulated enamel lesions. The results suggest that the relationship between surface zone thickness and lesion permeability is highly non-linear, and that a small increase in the surface layer thickness may lead to a significant decrease in permeability.

chronic dehydration affects hydroelectrolytic equilibrium and adrenal

African Journals Online (AJOL)

A. Dekar-Madoui

2017-05-01

May 1, 2017 ... response but rather an index of metabolic strategy probably for metabolic ... [11] Dai WJ, Yao T. Effects of dehydration and salt-loading on ... [12] Kondo N, Arima H, Banno R, Kuwahara S, Sato I, Oiso Y. Osmoregulation of.

Hereditary angioedema in women

Directory of Open Access Journals (Sweden)

Bouillet Laurence

2010-07-01

Full Text Available Abstract Women with hereditary angioedema (HAE are more likely to be symptomatic that men. Hormonal factors (puberty, contraception, pregnancy,.... play a significant role in the precipitation or worsening of the condition in women. So, combined contraceptive pills are not indicated and progestogen pill must be preferred. During pregnancy, attack rate can increase (38-48% of women. C1Inhibitor concentrate and tranexamic acid can be used during pregnancy. Attenuated androgens for long term prophylaxis are effective but side effects appear more often in female patients. These side effects are dose dependant and can be attenuated by titrating the dose down the lowest effective level.

Comparison of Attitudes Regarding Preimplantation Genetic Diagnosis Among Patients with Hereditary Cancer Syndromes

Science.gov (United States)

Rich, Thereasa A.; Liu, Mei; Etzel, Carol J.; Bannon, Sarah A.; Mork, Maureen E.; Ready, Kaylene; Saraiya, Devki S.; Grubbs, Elizabeth G.; Perrier, Nancy D.; Lu, Karen H.; Arun, Banu K.; Woodard, Terri L.; Schover, Leslie R.; Litton, Jennifer K.

2014-01-01

Introduction Preimplantation Genetic Diagnosis (PGD) allows couples to avoid having a child with an inherited condition, potentially reducing cancer burden in families with a hereditary cancer predisposition. This study investigated awareness and acceptance of PGD among patients with hereditary cancer syndromes. Methods Questionnaires were mailed to 984 adults with hereditary breast and ovarian cancer, Lynch syndrome, familial adenomatous polyposis, or multiple endocrine neoplasia type 1 or 2. Associations between clinical, demographic, and psychosocial factors and awareness and acceptance of PGD were examined. Results Of 370 respondents (38% return rate), 28% felt their syndrome impacted family planning, 24% were aware of PGD, 72% felt that PGD should be offered, 43% would consider using PGD, and 29% were uncertain. Family experience and syndrome-specific characteristics, such as disease severity, quality of life and availability of medical interventions as well as gender, family planning stage, and religiosity impact perceptions of the acceptability of PGD, though a high level of uncertainty exists. Conclusion Hereditary cancer patients' opinions about the acceptability of PGD are similar to those of genetics and ethical experts. Patients should be told about PGD given that most had not heard of PGD, but feel that PGD should be offered. PMID:24072553

Could Ossification of the Achilles Tendon Have a Hereditary Component?

Directory of Open Access Journals (Sweden)

Chawki Cortbaoui

2013-01-01

Full Text Available Ossification of the Achilles tendon (OTA is an unusual clinical condition. It is characterized by the presence of an ossified mass within the fibrocartilaginous substance of the Achilles tendon. The etiology of the ossification of the Achilles tendon is unknown. Review of the literature suggests that its etiology is multifactorial. The major contributing factors are trauma and surgery with other minor causes such as systemic diseases, metabolic conditions, and infections. To our knowledge, no previous reports suggest any genetic/hereditary predisposition in OAT. We report 3 siblings who have OAT with no history of any of the aforementioned predisposing factors. Could OAT have a hereditary component as one of its etiologies?

Accuracy of Inferior Vena Cava Ultrasound for Predicting Dehydration in Children with Acute Diarrhea in Resource-Limited Settings.

Science.gov (United States)

Modi, Payal; Glavis-Bloom, Justin; Nasrin, Sabiha; Guy, Allysia; Chowa, Erika P; Dvor, Nathan; Dworkis, Daniel A; Oh, Michael; Silvestri, David M; Strasberg, Stephen; Rege, Soham; Noble, Vicki E; Alam, Nur H; Levine, Adam C

2016-01-01

Although dehydration from diarrhea is a leading cause of morbidity and mortality in children under five, existing methods of assessing dehydration status in children have limited accuracy. To assess the accuracy of point-of-care ultrasound measurement of the aorta-to-IVC ratio as a predictor of dehydration in children. A prospective cohort study of children under five years with acute diarrhea was conducted in the rehydration unit of the International Centre for Diarrhoeal Disease Research, Bangladesh (icddr,b). Ultrasound measurements of aorta-to-IVC ratio and dehydrated weight were obtained on patient arrival. Percent weight change was monitored during rehydration to classify children as having "some dehydration" with weight change 3-9% or "severe dehydration" with weight change > 9%. Logistic regression analysis and Receiver-Operator Characteristic (ROC) curves were used to evaluate the accuracy of aorta-to-IVC ratio as a predictor of dehydration severity. 850 children were enrolled, of which 771 were included in the final analysis. Aorta to IVC ratio was a significant predictor of the percent dehydration in children with acute diarrhea, with each 1-point increase in the aorta to IVC ratio predicting a 1.1% increase in the percent dehydration of the child. However, the area under the ROC curve (0.60), sensitivity (67%), and specificity (49%), for predicting severe dehydration were all poor. Point-of-care ultrasound of the aorta-to-IVC ratio was statistically associated with volume status, but was not accurate enough to be used as an independent screening tool for dehydration in children under five years presenting with acute diarrhea in a resource-limited setting.

Accuracy of Inferior Vena Cava Ultrasound for Predicting Dehydration in Children with Acute Diarrhea in Resource-Limited Settings.

Directory of Open Access Journals (Sweden)

Payal Modi

Full Text Available Although dehydration from diarrhea is a leading cause of morbidity and mortality in children under five, existing methods of assessing dehydration status in children have limited accuracy.To assess the accuracy of point-of-care ultrasound measurement of the aorta-to-IVC ratio as a predictor of dehydration in children.A prospective cohort study of children under five years with acute diarrhea was conducted in the rehydration unit of the International Centre for Diarrhoeal Disease Research, Bangladesh (icddr,b. Ultrasound measurements of aorta-to-IVC ratio and dehydrated weight were obtained on patient arrival. Percent weight change was monitored during rehydration to classify children as having "some dehydration" with weight change 3-9% or "severe dehydration" with weight change > 9%. Logistic regression analysis and Receiver-Operator Characteristic (ROC curves were used to evaluate the accuracy of aorta-to-IVC ratio as a predictor of dehydration severity.850 children were enrolled, of which 771 were included in the final analysis. Aorta to IVC ratio was a significant predictor of the percent dehydration in children with acute diarrhea, with each 1-point increase in the aorta to IVC ratio predicting a 1.1% increase in the percent dehydration of the child. However, the area under the ROC curve (0.60, sensitivity (67%, and specificity (49%, for predicting severe dehydration were all poor.Point-of-care ultrasound of the aorta-to-IVC ratio was statistically associated with volume status, but was not accurate enough to be used as an independent screening tool for dehydration in children under five years presenting with acute diarrhea in a resource-limited setting.

The diagnostic accuracy of multi-frequency bioelectrical impedance analysis in diagnosing dehydration after stroke

Science.gov (United States)

Kafri, Mohannad W.; Myint, Phyo Kyaw; Doherty, Danielle; Wilson, Alexander Hugh; Potter, John F.; Hooper, Lee

2013-01-01

Background Non-invasive methods for detecting water-loss dehydration following acute stroke would be clinically useful. We evaluated the diagnostic accuracy of multi-frequency bioelectrical impedance analysis (MF-BIA) against reference standards serum osmolality and osmolarity. Material/Methods Patients admitted to an acute stroke unit were recruited. Blood samples for electrolytes and osmolality were taken within 20 minutes of MF-BIA. Total body water (TBW%), intracellular (ICW%) and extracellular water (ECW%), as percentages of total body weight, were calculated by MF-BIA equipment and from impedance measures using published equations for older people. These were compared to hydration status (based on serum osmolality and calculated osmolarity). The most promising Receiver Operating Characteristics curves were plotted. Results 27 stroke patients were recruited (mean age 71.3, SD10.7). Only a TBW% cut-off at 46% was consistent with current dehydration (serum osmolality >300 mOsm/kg) and TBW% at 47% impending dehydration (calculated osmolarity ≥295–300 mOsm/L) with sensitivity and specificity both >60%. Even here diagnostic accuracy of MF-BIA was poor, a third of those with dehydration were wrongly classified as hydrated and a third classified as dehydrated were well hydrated. Secondary analyses assessing diagnostic accuracy of TBW% for men and women separately, and using TBW as a percentage of lean body mass showed some promise, but did not provide diagnostically accurate measures across the population. Conclusions MF-BIA appears ineffective at diagnosing water-loss dehydration after stroke and cannot be recommended as a test for dehydration, but separating assessment by sex, and using TBW as a percentage of lean body weight may warrant further investigation. PMID:23839255

High Prevalence of Dehydration and Inadequate Nutritional Knowledge Among University and Club Level Athletes.

Science.gov (United States)

Magee, Pamela Jane; Gallagher, Alison M; McCormack, Jacqueline M

2017-04-01

Although dehydration of ≥ 2% body weight (BW) loss significantly impairs endurance performance, dehydration remains prevalent among athletes and may be owing to a lack of knowledge in relation to fluid requirements. The aim of this study was to assess the hydration status of university/club level athletes (n = 430) from a range of sports/activities (army officer cadet training; bootcamp training; cycling; Gaelic Athletic Association camogie, football and hurling; golf; hockey; netball; rugby; running (sprinting and endurance); Shotokan karate and soccer) immediately before and after training/competition and to assess their nutritional knowledge. Urine specific gravity (USG) was measured immediately before and after exercise and BW loss during exercise was assessed. Nutritional knowledge was assessed using a validated questionnaire. 31.9% of athletes commenced exercise in a dehydrated state (USG >1.020) with 43.6% of participants dehydrated posttraining/competition. Dehydration was particularly prevalent (>40% of cohort) among karateka, female netball players, army officer cadets, and golfers. Golfers that commenced a competitive 18 hole round dehydrated took a significantly higher number of strokes to complete the round in comparison with their euhydrated counterparts (79.5 ± 2.1 vs. 75.7 ± 3.9 strokes, p = .049). Nutritional knowledge was poor among participants (median total score [IQR]; 52.9% [46.0, 59.8]), albeit athletes who were euhydrated at the start of exercise had a higher overall score in comparison with dehydrated athletes (55.2% vs. 50.6%, p = .001). Findings from the current study, therefore, have significant implications for the education of athletes in relation to their individual fluid requirements around exercise.

Hereditary neuromuscular diseases

Energy Technology Data Exchange (ETDEWEB)

Oezsarlak, O. E-mail: ozkan.ozsarlak@uza.be; Schepens, E.; Parizel, P.M.; Goethem, J.W. van; Vanhoenacker, F.; Schepper, A.M. de; Martin, J.J

2001-12-01

This article presents the actual classification of neuromuscular diseases based on present expansion of our knowledge and understanding due to genetic developments. It summarizes the genetic and clinical presentations of each disorder together with CT findings, which we studied in a large group of patients with neuromuscular diseases. The muscular dystrophies as the largest and most common group of hereditary muscle diseases will be highlighted by giving detailed information about the role of CT and MRI in the differential diagnosis. The radiological features of neuromuscular diseases are atrophy, hypertrophy, pseudohypertrophy and fatty infiltration of muscles on a selective basis. Although the patterns and distribution of involvement are characteristic in some of the diseases, the definition of the type of disease based on CT scan only is not always possible.

DEHYDRATION IN CHILDREN WITH ACUTE DIARRHEA

Directory of Open Access Journals (Sweden)

S. V. Khaliullina

2014-01-01

Full Text Available The article is focused on the main issues of the regulation of water-electrolyte metabolism in children, possible variants of its disorder in acute diarrhoeal diseases. The clinical features of dehydration depending on the severity and qualitative component of losses are described, recommendations on laboratory diagnosis and treatment are provided. 

Pig skin apposite dehydrated by lyophilization

International Nuclear Information System (INIS)

Reyes F, M.L.; Gonzalez V, C.; Flores A, M.; Peralta R, J.; Reboyo B, D.; Rodriguez U, M.D.

2007-01-01

Taking like base a work carried out in 2001 in the Radio sterilized Tissue Bank (BTR) in which lyophilized apposite of pig skin were obtained at laboratory scale, this work is presented that had as purpose to process pig skin to produce temporary covers of skin (apposite) dehydrated by lyophilization to commercial scale. (Author)

Reducing the negative vocal effects of superficial laryngeal dehydration with humidification.

Science.gov (United States)

Levendoski, Elizabeth Erickson; Sundarrajan, Anusha; Sivasankar, M Preeti

2014-07-01

Environmental humidification is a simple, cost-effective method believed to reduce superficial laryngeal drying. This study sought to validate this belief by investigating whether humidification treatment would reduce the negative effects of superficial laryngeal dehydration on phonation threshold pressure (PTP). Phonation threshold pressure data analysis may be vulnerable to bias because of lack of investigator blinding. Consequently, this study investigated the extent of PTP analysis reliability between unblinded and blinded investigators. Healthy male and female adults were assigned to a vocal fatigue (n = 20) or control group (n = 20) based on their responses to a questionnaire. PTP was assessed after 2 hours of mouth breathing in low humidity (dehydration challenge), following a 5-minute break in ambient humidity, and after 2 hours of mouth breathing in high humidity (humidification). PTP significantly increased following the laryngeal dehydration challenge. After humidification, PTP returned toward baseline. These effects were observed in both subject groups. PTP measurements were highly correlated between the unblinded and blinded investigator. Humidification may be an effective approach to decrease the detrimental voice effects of superficial laryngeal dehydration. These data lay the foundation for future investigations aimed at preventing and treating the negative voice changes associated with chronic, surface laryngeal drying.

Hereditary effects in eccentric compact binary inspirals to third post-Newtonian order

Science.gov (United States)

Loutrel, Nicholas; Yunes, Nicolás

2017-02-01

While there has been much success in understanding the orbital dynamics and gravitational wave emission of eccentric compact binaries in the post-Newtonian formalism, some problems still remain. The largest of these concerns hereditary effects: non-linear phenomena related to the scattering off of the background curved spacetime (tails) and to the generation of gravitational waves by gravitational waves (memory). Currently, these hereditary effects are only known numerically for arbitrary eccentricity through infinite sums of Bessel functions, with closed-form, analytic results only available in the small eccentricity limit. We here calculate, for the first time, closed-form, analytic expressions for all hereditary effects to third post-Newtonian order in binaries with arbitrary eccentricity. For the tails, we first asymptotically expand all Bessel functions in high eccentricity and find a superasymptotic series for each enhancement factor, accurate to better than 10-3 relative to post-Newtonian numerical calculations at all eccentricities. We further improve the small-eccentricity behavior of the superasymptotic series by generating hyperasymptotic expressions for each enhancement factor, typically accurate to better than 10-8 at all eccentricities. For the memory, we discuss its computation within the context of an osculating approximation of the binary’s orbit and the difficulties that arise. Our closed-form analytic expressions for the hereditary fluxes allow us to numerically compute orbital phases that are identical to those found using an infinite sum of Bessel functions to double numerical precision.

Hereditary effects in eccentric compact binary inspirals to third post-Newtonian order

International Nuclear Information System (INIS)

Loutrel, Nicholas; Yunes, Nicolás

2017-01-01

While there has been much success in understanding the orbital dynamics and gravitational wave emission of eccentric compact binaries in the post-Newtonian formalism, some problems still remain. The largest of these concerns hereditary effects: non-linear phenomena related to the scattering off of the background curved spacetime (tails) and to the generation of gravitational waves by gravitational waves (memory). Currently, these hereditary effects are only known numerically for arbitrary eccentricity through infinite sums of Bessel functions, with closed-form, analytic results only available in the small eccentricity limit. We here calculate, for the first time, closed-form, analytic expressions for all hereditary effects to third post-Newtonian order in binaries with arbitrary eccentricity. For the tails, we first asymptotically expand all Bessel functions in high eccentricity and find a superasymptotic series for each enhancement factor, accurate to better than 10 −3 relative to post-Newtonian numerical calculations at all eccentricities. We further improve the small-eccentricity behavior of the superasymptotic series by generating hyperasymptotic expressions for each enhancement factor, typically accurate to better than 10 −8 at all eccentricities. For the memory, we discuss its computation within the context of an osculating approximation of the binary’s orbit and the difficulties that arise. Our closed-form analytic expressions for the hereditary fluxes allow us to numerically compute orbital phases that are identical to those found using an infinite sum of Bessel functions to double numerical precision. (paper)

EPR study on gamma-irradiated fruits dehydrated via osmosis

Energy Technology Data Exchange (ETDEWEB)

Yordanov, N.D. [Laboratory EPR, Institute of Catalysis, Bulgarian Academy of Sciences, 1113 Sofia (Bulgaria)]. E-mail: ndyepr@bas.bg; Aleksieva, K. [Laboratory EPR, Institute of Catalysis, Bulgarian Academy of Sciences, 1113 Sofia (Bulgaria)

2007-06-15

The shape and time stability of the electron paramagnetic resonance (EPR) spectra of non- and {gamma}-irradiated papaya, melon, cherry and fig samples dehydrated via osmosis are reported. It is shown that non-irradiated samples are generally EPR silent whereas {gamma}-irradiated exhibit 'sugar-like' EPR spectra. The recorded EPR spectra are monitored for a period of 7 months after irradiation (stored at low humidity and in the dark). The results suggest longer period of unambiguous identification of the radiation processing of osmose dehydrated fruits. Therefore, the Protocol EN 13708,2001 issued by CEN is fully applicable for the studied fruit samples.

Hopefulness predicts resilience after hereditary colorectal cancer genetic testing: a prospective outcome trajectories study

OpenAIRE

Chu Annie TW; Bonanno George A; Ho Judy WC; Ho Samuel MY; Chan Emily MS

2010-01-01

Abstract Background - Genetic testing for hereditary colorectal cancer (HCRC) had significant psychological consequences for test recipients. This prospective longitudinal study investigated the factors that predict psychological resilience in adults undergoing genetic testing for HCRC. Methods - A longitudinal study was carried out from April 2003 to August 2006 on Hong Kong Chinese HCRC family members who were recruited and offered genetic testing by the Hereditary Gastrointestinal Cancer R...

The lateral neostriatum is necessary for compensatory ingestive behaviour after intravascular dehydration in female rats.

Science.gov (United States)

Lelos, M J; Harrison, D J; Rosser, A E; Dunnett, S B

2013-12-01

Aberrant striatal function results in an array of physiological symptoms, including impaired consummatory and regulatory behaviours, which can lead to weight loss and dehydration. It was hypothesised, therefore, that cell loss in the neostriatum may contribute to altered fluid intake by regulating physiological signals related to dehydration status. To test this theory, rats with lesions of the lateral neostriatum and sham controls underwent a series of physiological challenges, including the experimental induction of intracellular and intravascular dehydration. No baseline differences in prandial or non-prandial drinking were observed, nor were differences in locomotor activity evident between groups. Furthermore, intracellular dehydration increased water intake in lesion rats in a manner comparable to sham rats. Interestingly, a specific impairment was evident in lesion rats after subcutaneous injection of poly-ethylene glycol was used to induce intravascular dehydration, such that lesion rats failed to adapt their water intake to this physiological change. The results suggest that the striatal lesions resulted in regulatory dysfunction by impairing motivational control over compensatory ingestive behaviour after intravascular hydration, while the physiological signals related to dehydration remain intact. Loss of these cells in neurodegenerative disorders, such Huntington's disease, may contribute to regulatory changes evident in the course of the disease. Copyright © 2013 Elsevier Ltd. All rights reserved.

Autosomal dominant hereditary ataxia in Sri Lanka

OpenAIRE

Sumathipala, Dulika S; Abeysekera, Gayan S; Jayasekara, Rohan W; Tallaksen, Chantal ME; Dissanayake, Vajira HW

2013-01-01

Background Spinocerebellar ataxias (SCA) are a group of hereditary neurodegenerative disorders. Prevalence of SCA subtypes differ worldwide. Autosomal dominant ataxias are the commonest types of inherited ataxias seen in Sri Lanka. The aim of the study is to determine the genetic etiology of patients with autosomal dominant ataxia in Sri Lanka and to describe the clinical features of each genetic subtype. Methods ...

Thermal dehydration kinetics of phosphogypsum

OpenAIRE

López Gómez, Félix Antonio; Tayibi, Hanan; García-Díaz, Irene; Alguacil, Francisco José

2015-01-01

Phsophogypsum is a by-product from the processing phosphate rock. Before the use of it in cement industry such as setting regulator is necessary a study of dehydration reaction of phosphogypsum to avoid the false setting during the milling. The aim is to study the thermal behavior of two different phosphogypsum sources (Spain and Tunisia) under non-isothermal conditions in argon atmosphere by using Thermo-Gravimetriy, Differential Thermal Analysis (TG-DTA) and Differential Scanning Calori...

A comprehensive custom panel design for routine hereditary cancer testing: preserving control, improving diagnostics and revealing a complex variation landscape.

Science.gov (United States)

Castellanos, Elisabeth; Gel, Bernat; Rosas, Inma; Tornero, Eva; Santín, Sheila; Pluvinet, Raquel; Velasco, Juan; Sumoy, Lauro; Del Valle, Jesús; Perucho, Manuel; Blanco, Ignacio; Navarro, Matilde; Brunet, Joan; Pineda, Marta; Feliubadaló, Lidia; Capellá, Gabi; Lázaro, Conxi; Serra, Eduard

2017-01-04

We wanted to implement an NGS strategy to globally analyze hereditary cancer with diagnostic quality while retaining the same degree of understanding and control we had in pre-NGS strategies. To do this, we developed the I2HCP panel, a custom bait library covering 122 hereditary cancer genes. We improved bait design, tested different NGS platforms and created a clinically driven custom data analysis pipeline. The I2HCP panel was developed using a training set of hereditary colorectal cancer, hereditary breast and ovarian cancer and neurofibromatosis patients and reached an accuracy, analytical sensitivity and specificity greater than 99%, which was maintained in a validation set. I2HCP changed our diagnostic approach, involving clinicians and a genetic diagnostics team from panel design to reporting. The new strategy improved diagnostic sensitivity, solved uncertain clinical diagnoses and identified mutations in new genes. We assessed the genetic variation in the complete set of hereditary cancer genes, revealing a complex variation landscape that coexists with the disease-causing mutation. We developed, validated and implemented a custom NGS-based strategy for hereditary cancer diagnostics that improved our previous workflows. Additionally, the existence of a rich genetic variation in hereditary cancer genes favors the use of this panel to investigate their role in cancer risk.

Clinical and laboratory assessment of dehydration severity in children with acute gastroenteritis.

Science.gov (United States)

Parkin, Patricia C; Macarthur, Colin; Khambalia, Amina; Goldman, Ran D; Friedman, Jeremy N

2010-03-01

To evaluate clinical and laboratory assessment of dehydration severity in children, 1 to 36 months, with acute gastroenteritis. Clinical and laboratory measures and weight change following rehydration were collected for enrolled children. Pediatric emergency department. Likelihood ratio (LR+) and 95% confidence interval (CI): for a clinical score of 0, the LR+ was 2.2 (95% CI = 0.9-5.3); for a clinical score of 1 to 4, the LR+ was 1.3 (95% CI = 0.90-1.74); for a clinical score of 5 to 8, the LR+ was 5.2 (95% CI = 2.2-12.8); for a venous pH Dehydration Scale and laboratory measures into clinical decision-making algorithms to assess dehydration severity in children with acute gastroenteritis.

[Molecular genetic diagnostics and screening of hereditary hemochromatosis].

Science.gov (United States)

Zlocha, J; Kovács, L; Pozgayová, S; Kupcová, V; Durínová, S

2006-06-01

Hereditary hemochromatosis is considered one of the most common hereditary diseases in population of Caucasian origin. In recent years, a candidate gene for HLA-linked hemochromatosis, HFE, has been cloned, and a single G-to-A mutation resulting in a cysteine-to-tyrosine substitution (C282Y) has been identified in up to 80% of study patients with type 1 hereditary hemochromatosis. The purpose of the paper was to confirm the importance of genetic testing for HFE mutations in making the diagnosis of hemochromatosis and find out a suitable diagnostic algorithm for the indication of this form of diagnostics in patients suspected of hereditary hemochromatosis. The examination of C282Y mutation was conducted in 500 subjects. The most frequent indications for DNA analysis were hepatopathy of unknown ethiology, liver cirrhosis, diabetes mellitus, bronze skin pigmentation in connection with high serum iron concentration, elevated transferrin saturation and elevated serum ferritin levels. In our group of patients, 29 homozygotes and 75 heterozygotes for C282Y mutation were identified, 10 patients carried both C282Y and H63D mutations of HFE gene (compound heterozygotes), whereas in 386 subjects the mutation was not found. The genotype-phenotype correlation showed that 22 homozygotes had liver affection proved by imaging and/or histologic methods. Except the liver disorders, the most common symptoms of these patients were type 2 diabetes mellitus or glucose tolerance disorder (10 patients), arthritis or joint pain (9 patients) and cardiovascular disorders, such as cardiomyopathy (2 patients). Bronze skin pigmentation was present in 9 homozygotes. Transferin saturation values were significantly higher in homozygotes for C282Y mutation as compared to C282Y heterozygotes (p diagnostics of this severe, but in early recognition curable disease. Early detection and phlebotomy treatment prior to the onset of cirrhosis can reduce morbidity and normalize life expectancy. It is readily

Use of osmotic dehydration to improve fruits and vegetables quality during processing.

Science.gov (United States)

Maftoonazad, Neda

2010-11-01

Osmotic treatment describes a preparation step to further processing of foods involving simultaneous transient moisture loss and solids gain when immersing in osmotic solutions, resulting in partial drying and improving the overall quality of food products. The different aspects of the osmotic dehydration (OD) technology namely the solutes employed, solutions characteristics used, process variables influence, as well as, the quality characteristics of the osmodehydrated products will be discussed in this review. As the process is carried out at mild temperatures and the moisture is removed by a liquid diffusion process, phase change that would be present in the other drying processes will be avoided, resulting in high quality products and may also lead to substantial energy savings. To optimize this process, modeling of the mass transfer phenomenon can improve high product quality. Several techniques such as microwave heating, vacuum, high pressure, pulsed electric field, etc. may be employed during or after osmotic treatment to enhance performance of the osmotic dehydration. Moreover new technologies used in osmotic dehydration will be discussed. Patents on osmotic dehydration of fruits and vegetables are also discussed in this article.

Nocturnal lagophthalmos: never seen before in hypernatraemic dehydration.

Science.gov (United States)

Rai, Birendra; Moka, Sudha; Sharif, Farhana

2014-04-11

We present two cases in which a 10-month-old male infant and another 15-month-old female child presented with symptoms of sleeping with their eyes wide open (lagophthalmos) with features of gastroenteritis (GE) and dehydration. The first child had been seen and discharged the previous day from the paediatric emergency department (ED) with a diagnosis of GE. He presented the following day with sleeping discomfort with his eyes wide open and ongoing symptoms of GE. The second child presented to the ED with features of GE. She was found to be sleeping in the ED with her eyes wide open. Investigations of both children revealed hypernatraemic dehydration. Correction of the electrolyte imbalance in both cases over a period of 48 h led to the resolution of symptoms.

Multiple Hereditary Osteochondromatosis: A Case Report

OpenAIRE

K???kesmen, ?i?dem; ?zen, Bu?ra; Ak?am, Mustafa

2007-01-01

Objectives Common carious lesions owing to vomiting are not widespread in children. In this case, we aimed to report an 11-years-old male patient with common carious lesions due to repeated vomitings, chewing and eating difficulty and retarded growth with Multiple Hereditary Osteochondromatosis (MHO). Case Report An 11-years-old boy was referred to Department of Pediatric Dentistry in Faculty of Dentistry because of eating difficulty owing to common carious lesions. It was seen that the patie...

Pancreatic cancer risk in hereditary pancreatitis

OpenAIRE

Weiss, Frank U.

2014-01-01

Inflammation is part of the body’s immune response in order to remove harmful stimuli – like pathogens, irritants or damaged cells - and start the healing process. Recurrent or chronic inflammation on the other side seems a predisposing factor for carcinogenesis and has been found associated with cancer development. In chronic pancreatitis mutations of the cationic trypsinogen (PRSS1) gene have been identified as risk factors of the disease. Hereditary pancreatitis is a rare cause of chronic...

Pulmonary hypertension and right heart failure due to severe hypernatremic dehydration.

Science.gov (United States)

Chiwane, Saurabh; Ahmed, Tageldin M; Bauerfeld, Christian P; Chauhan, Monika

2017-07-01

Neonates are at risk of developing hypernatremic dehydration and its associated complications, such as stroke, dural sinus thrombosis and renal vein thrombosis. Pulmonary hypertension has not been described as a complication of hypernatremia. We report a case of a seven-day-old neonate with severe hypernatremic dehydration who went on to develop pulmonary hypertension and right heart failure needing extracorporeal membrane oxygenation (ECMO). Normal or high anion gap metabolic acidosis commonly accompanies hypernatremic dehydration. The presence of acidosis and/or hypoxia can delay the normal drop in pulmonary vascular resistance (PVR) after birth, causing pulmonary hypertension and right ventricular failure. A high index of suspicion is paramount to diagnose pulmonary hypertension and aggressive correction of the acidosis and hypoxia is needed. In the presence of severe right ventricular failure, ECMO can be used as a bridge to recovery while underlying metabolic derangements are being corrected.

Memory responses of jasmonic acid-associated Arabidopsis genes to a repeated dehydration stress.

Science.gov (United States)

Liu, Ning; Staswick, Paul E; Avramova, Zoya

2016-11-01

Dehydration stress activates numerous genes co-regulated by diverse signaling pathways. Upon repeated exposures, however, a subset of these genes does not respond maintaining instead transcription at their initial pre-stressed levels ('revised-response' genes). Most of these genes are involved in jasmonic acid (JA) biosynthesis, JA-signaling and JA-mediated stress responses. How these JA-associated genes are regulated to provide different responses to similar dehydration stresses is an enigma. Here, we investigate molecular mechanisms that contribute to this transcriptional behavior. The memory-mechanism is stress-specific: one exposure to dehydration stress or to abscisic acid (ABA) is required to prevent transcription in the second. Both ABA-mediated and JA-mediated pathways are critical for the activation of these genes, but the two signaling pathways interact differently during a single or multiple encounters with dehydration stress. Synthesis of JA during the first (S1) but not the second dehydration stress (S2) accounts for the altered transcriptional responses. We propose a model for these memory responses, wherein lack of MYC2 and of JA synthesis in S2 is responsible for the lack of expression of downstream genes. The similar length of the memory displayed by different memory-type genes suggests biological relevance for transcriptional memory as a gene-regulating mechanism during recurring bouts of drought. © 2016 John Wiley & Sons Ltd.

Heat stress and dehydration in adapting for performance: Good, bad, both, or neither?

Science.gov (United States)

Akerman, Ashley Paul; Tipton, Michael; Minson, Christopher T; Cotter, James David

2016-01-01

Physiological systems respond acutely to stress to minimize homeostatic disturbance, and typically adapt to chronic stress to enhance tolerance to that or a related stressor. It is legitimate to ask whether dehydration is a valuable stressor in stimulating adaptation per se . While hypoxia has had long-standing interest by athletes and researchers as an ergogenic aid, heat and nutritional stressors have had little interest until the past decade. Heat and dehydration are highly interlinked in their causation and the physiological strain they induce, so their individual roles in adaptation are difficult to delineate. The effectiveness of heat acclimation as an ergogenic aid remains unclear for team sport and endurance athletes despite several recent studies on this topic. Very few studies have examined the potential ergogenic (or ergolytic) adaptations to ecologically-valid dehydration as a stressor in its own right, despite longstanding evidence of relevant fluid-regulatory adaptations from short-term hypohydration. Transient and self-limiting dehydration (e.g., as constrained by thirst), as with most forms of stress, might have a time and a place in physiological or behavioral adaptations independently or by exacerbating other stressors (esp. heat); it cannot be dismissed without the appropriate evidence. The present review did not identify such evidence. Future research should identify how the magnitude and timing of dehydration might augment or interfere with the adaptive processes in behaviorally constrained versus unconstrained humans.

The Influence of the Osmotic Dehydration Process on Physicochemical Properties of Osmotic Solution.

Science.gov (United States)

Lech, Krzysztof; Michalska, Anna; Wojdyło, Aneta; Nowicka, Paulina; Figiel, Adam

2017-12-16

The osmotic dehydration (OD) process consists of the removal of water from a material during which the solids from the osmotic solution are transported to the material by osmosis. This process is commonly performed in sucrose and salt solutions. Taking into account that a relatively high consumption of those substances might have a negative effect on human health, attempts have been made to search for alternatives that can be used for osmotic dehydration. One of these is an application of chokeberry juice with proven beneficial properties to human health. This study aimed to evaluate the physicochemical properties of the OD solution (chokeberry juice concentrate) before and after the osmotic dehydration of carrot and zucchini. The total polyphenolics content, antioxidant capacity (ABTS, FRAP), dynamic viscosity, density, and water activity were examined in relation to the juice concentration used for the osmotic solution before and after the OD process. During the osmotic dehydration process, the concentration of the chokeberry juice decreased. Compounds with lower molecular weight and lower antioxidant capacity present in concentrated chokeberry juice had a stronger influence on the exchange of compounds during the OD process in carrot and zucchini. The water activity of the osmotic solution increased after the osmotic dehydration process. It was concluded that the osmotic solution after the OD process might be successfully re-used as a product with high quality for i.e. juice production.

Development and characterisation of hybrid polysaccharide membranes for dehydration processes.

Science.gov (United States)

Meireles, Inês T; Huertas, Rosa M; Torres, Cristiana A V; Coelhoso, Isabel M; Crespo, João G

2018-07-01

The purpose of this work is the development and characterisation of new hybrid polysaccharide (FucoPol) membranes. These membranes were prepared by incorporation of a SiO 2 network homogeneously dispersed by using a sol-gel method with GPTMS as a crosslinker silica precursor. They were further crosslinked with CaCl 2 for reinforcement of mechanical properties and improvement of their permeation performance. They were characterised in terms of their structural, mechanical and thermal properties. They presented a dense and homogeneous structure, resistant to deformation, with a Tg of 43 °C and a thermal decomposition between 240 and 251 °C. The hybrid FucoPol membranes were tested for ethanol dehydration by pervaporation and also for nitrogen dehydration. They exhibited high water selectivity values, similar to PERVAP ® 4101, however they lost their stability when exposed to solutions of 10.0 wt.% water in ethanol. In contrast, these membranes were stable when applied in N 2 dehydration, leading to reproducible performance and very high water selectivities. Copyright © 2018 Elsevier Ltd. All rights reserved.

Dehydration Behaviour of Borax Pentahydrate to Anhydrous Borax by Multi-Stage Heating in a Fluidized

OpenAIRE

ŞAHİN, Ömer

2002-01-01

In order to optimize the anhydrous borax process in a fluidized bed calcinator, it is absolutely necessary to study the dehydration behaviour of borax pentahydrate. It was found that the basic feature of the dehydration of borax pentahydrate to anhydrous borax is concerned with the conditions employed. The bulk density and sodium borate content of the end product were determined to be a function of number of stages of temperature of the dehydration period in the fluidized bed. It...

Permeability of gypsum samples dehydrated in air

Science.gov (United States)

Milsch, Harald; Priegnitz, Mike; Blöcher, Guido

2011-09-01

We report on changes in rock permeability induced by devolatilization reactions using gypsum as a reference analog material. Cylindrical samples of natural alabaster were dehydrated in air (dry) for up to 800 h at ambient pressure and temperatures between 378 and 423 K. Subsequently, the reaction kinetics, so induced changes in porosity, and the concurrent evolution of sample permeability were constrained. Weighing the heated samples in predefined time intervals yielded the reaction progress where the stoichiometric mass balance indicated an ultimate and complete dehydration to anhydrite regardless of temperature. Porosity showed to continuously increase with reaction progress from approximately 2% to 30%, whilst the initial bulk volume remained unchanged. Within these limits permeability significantly increased with porosity by almost three orders of magnitude from approximately 7 × 10-19 m2 to 3 × 10-16 m2. We show that - when mechanical and hydraulic feedbacks can be excluded - permeability, reaction progress, and porosity are related unequivocally.

Dehydration of alcohols over oxide catalysts: γ-eliminations -- stereospecificity and selectivity

International Nuclear Information System (INIS)

Siddhan, S.; Narayanan, K.

1979-01-01

The effect of alkali impregnation on alumina catalysts has been investigated by a physicochemical study of pure and modified alumina catalyst samples. The stereospecificity and selectivity of dehyration reactions, as well as the incidence of γ-elimination, have been studied by passing suitable substrates over catalyst samples. There was a change in the acidity-basicity balance in the sodium-impregnated alumina samples vis a vis pure alumina, while the surface area virtually remained constant. A higher propensity for γ-elimination was noticed with increases in basicity of the catalyst. 1-Olefin formation was found to be larger in more basic alumina- and thoria-catalyzed dehydration reactions. Thoria was strikingly unique in its capacity to dehydrate only alcohols, which have at least one β-hydrogen atom. Neopentyl alcohol could not be dehydrated even under drastic conditions. The modes of elimination in the case of alumina and thoria have been shown to be anti and syn, respectively, from the results of the dehydration studies with threo-3-methyl-2-pentanol. Studies of alcohols with proper β-substituents revealed that the cis preference is not universal in all catalytic eliminations but, in fact, depends on the mode of elimination. While cis-preference was noticed in alumina-catalyzed anti eliminations, trans-olefin was formed to a major amount in thoria-catalyzed syn-elimination processes. 9 figures, 13 tables

Current problems in haematology. 2: Hereditary spherocytosis.

OpenAIRE

Smedley, J C; Bellingham, A J

1991-01-01

Hereditary spherocytosis is a relatively common haematological disorder and will be encountered by all haematologists. The abundance of new information, dealing principally with molecular and genetic aspects of pathophysiology, is beginning to have implications for its investigation and management. While these advances have not yet exerted a large influence at therapeutic level, the promise of such advents as prenatal diagnosis make this an exciting field to watch.

Multimodality imaging features of hereditary multiple exostoses

OpenAIRE

Kok, H K; Fitzgerald, L; Campbell, N; Lyburn, I D; Munk, P L; Buckley, O; Torreggiani, W C

2013-01-01

Hereditary multiple exostoses (HME) or diaphyseal aclasis is an inherited disorder characterised by the formation of multiple osteochondromas, which are cartilage-capped osseous outgrowths, and the development of associated osseous deformities. Individuals with HME may be asymptomatic or develop clinical symptoms, which prompt imaging studies. Different modalities ranging from plain radiographs to cross-sectional and nuclear medicine imaging studies can be helpful in the diagnosis and detecti...

DEHYDRATION IN SOCCER PLAYERS AFTER A MATCH IN THE HEAT

Directory of Open Access Journals (Sweden)

J.C.B. Marins

2011-11-01

Full Text Available We investigated the level of dehydration after a match in 20 soccer players (mean ± SD, 17.9 ± 1.3 years old, height 1.75 ± 0.05 m, body mass 70.71± 7.65 kg from two teams that participate in a Brazilian Championship game performed at a temperature of 29 ± 1.1 C and a relative humidity of 64 ± 4.2%. Body mass, urine specific gravity and urinary protein were measured before and after the match, and self-perception measurements were performed during the match. Body mass loss was 1.00 ± 0.39 kg, corresponding to a dehydration percentage of 1.35 ± 0.87%. The mean sweating rate during the match was 866 ± 319 ml · h[sup]-1[/sup] and total fluid intake was 1265.00 ± 505.45 ml. The sweating rate and the quantity of ingested fluids correlated positively (r = 0.98; P<0.05. Protein occurred in the urine in 18 soccer players. The players showed no perception of thirst and considered themselves as comfortable during the match. At the end of the match the soccer players replaced 57.7 ± 15% of the water loss and presented a condition of significant to severe dehydration based on the post-match urine specific gravity data (1.027 ± 6 g · ml[sup]-1[/sup]. The results of this study demonstrate that most of the soccer players began the match with some degree of dehydration that worsened during the match.

Non-isothermal dehydration kinetic study of aspartame hemihydrate using DSC, TGA and DSC-FTIR microspectroscopy

Directory of Open Access Journals (Sweden)

Wei-hsien Hsieh

2018-05-01

Full Text Available Three thermal analytical techniques such as differential scanning calorimetry (DSC, thermal gravimetric analysis (TGA using five heating rates, and DSC-Fourier Transform Infrared (DSC-FTIR microspectroscopy using one heating rate, were used to determine the thermal characteristics and the dehydration process of aspartame (APM hemihydrate in the solid state. The intramolecular cyclization process of APM anhydrate was also examined. One exothermic and four endothermic peaks were observed in the DSC thermogram of APM hemihydrate, in which the exothermic peak was due to the crystallization of some amorphous APM caused by dehydration process from hemihydrate to anhydride. While four endothermic peaks were corresponded to the evaporation of absorbed water, the dehydration of hemihydrate, the diketopiperazines (DKP formation via intramolecular cyclization, and the melting of DKP, respectively. The weight loss measured in TGA curve of APM hemihydrate was associated with these endothermic peaks in the DSC thermogram. According to the Flynn–Wall–Ozawa (FWO model, the activation energy of dehydration process within 100–150 °C was about 218 ± 11 kJ/mol determined by TGA technique. Both the dehydration and DKP formation processes for solid-state APM hemihydrate were markedly evidenced from the thermal-responsive changes in several specific FTIR bands by a single-step DSC-FTIR microspectroscopy. Keywords: Aspartame (APM hemihydrate, DSC/TGA, DSC-FTIR, Dehydration, Activation energy, DKP formation

Catalytic dehydration of ethanol using transition metal oxide catalysts.

Science.gov (United States)

Zaki, T

2005-04-15

The aim of this work is to study catalytic ethanol dehydration using different prepared catalysts, which include Fe(2)O(3), Mn(2)O(3), and calcined physical mixtures of both ferric and manganese oxides with alumina and/or silica gel. The physicochemical properties of these catalysts were investigated via X-ray powder diffraction (XRD), acidity measurement, and nitrogen adsorption-desorption at -196 degrees C. The catalytic activities of such catalysts were tested through conversion of ethanol at 200-500 degrees C using a catalytic flow system operated under atmospheric pressure. The results obtained indicated that the dehydration reaction on the catalyst relies on surface acidity, whereas the ethylene production selectivity depends on the catalyst chemical constituents.

Hereditary Portfolio Optimization with Taxes and Fixed Plus Proportional Transaction Costs—Part II

Directory of Open Access Journals (Sweden)

Mou-Hsiung Chang

2007-01-01

Full Text Available This paper is the continuation of the paper entitled “Hereditary portfolio optimization with taxes and fixed plus proportional transaction costs I” that treats an infinite-time horizon hereditary portfolio optimization problem in a market that consists of one savings account and one stock account. Within the solvency region, the investor is allowed to consume from the savings account and can make transactions between the two assets subject to paying capital-gain taxes as well as a fixed plus proportional transaction cost. The investor is to seek an optimal consumption-trading strategy in order to maximize the expected utility from the total discounted consumption. The portfolio optimization problem is formulated as an infinite dimensional stochastic classical impulse control problem due to the hereditary nature of the stock price dynamics and inventories. This paper contains the verification theorem for the optimal strategy. It also proves that the value function is a viscosity solution of the QVHJBI.

Surgical treatment of hereditary lens subluxations.

Science.gov (United States)

Ozdek, Sengul; Sari, Ayca; Bilgihan, Kamil; Akata, Fikret; Hasanreisoglu, Berati

2002-01-01

To evaluate the effectiveness and results of pars plana vitreolensectomy approach with transscleral fixation of intraocular lens in hereditary lens subluxations. Fifteen eyes of 9 consecutive patients with a mean age of 12.8+/-6.2 years (6-26 years) with hereditary lens subluxation were operated on and the results were evaluated in a prospective study. Surgery was considered if best spectacle corrected visual acuity (BSCVA) was less than 20/70. All eyes underwent a 2-port pars plana vitreolensectomy and transscleral fixation of an intraocular lens (IOL). The mean follow-up period was 12.6+/-7.5 months (6-22 months). There was no major intraoperative complication. Preoperatively, 8 eyes (53.3%) had a BSCVA of counting fingers (CF) and 7 eyes (46.6%) had a BSCVA of 20/200 to 20/70. Postoperatively, 14 eyes (93.3%) had a BSCVA of 20/50 or better. None of the patients had IOL decentration or intraocular pressure (IOP) increase during the follow-up period. There was a macular hole formation in 1 eye postoperatively. The early results of pars plana vitreolensectomy with IOL implantation using scleral fixation technique had shown that it not only promises a rapid visual rehabilitation but it is also a relatively safe method. More serious complications, however, may occur in the long term.

Motor activation in SPG4-linked hereditary spastic paraplegia

DEFF Research Database (Denmark)

Scheuer, KH; Nielsen, JE; Krabbe, Katja

2006-01-01

OBJECTIVE: The aim of this study was to investigate the extent of motor cortical functional reorganisation in patients with SPG4-linked hereditary spastic paraplegia by exploring cortical motor activation related to movements of clinically affected (lower) and unaffected (upper) limbs. METHODS: T...

An ABC of the Warning Signs of Hereditary Angioedema

DEFF Research Database (Denmark)

Grumach, Anete Sevciovic; Ferraroni, Natasha; Olivares, Maria Margarita

2017-01-01

Hereditary angioedema (HAE) with C1 inhibitor deficiency is a genetic disorder that clinically manifests with attacks of angioedema in the subcutaneous and submucosal tissues, mainly in the extremities, abdomen, and upper airway. During attacks, vascular permeability is increased due to increased...

Biochemical degradation and physical migration of polyphenolic compounds in osmotic dehydrated blueberries with pulsed electric field and thermal pretreatments.

Science.gov (United States)

Yu, Yuanshan; Jin, Tony Z; Fan, Xuetong; Wu, Jijun

2018-01-15

Fresh blueberries were pretreated by pulsed electric fields (PEF) or thermal pretreatment and then were subject to osmotic dehydration. The changes in contents of anthocyanins, predominantly phenolic acids and flavonols, total phenolics, polyphenol oxidase (PPO) activity and antioxidant activity in the blueberry samples during pretreatment and osmotic dehydration were investigated. Biochemical degradation and physical migration of these nutritive compounds from fruits to osmotic solutions were observed during the pretreatments and osmotic dehydration. PEF pretreated samples had the least degradation loss but the most migration loss of these compounds compared to thermally pretreated and control samples. Higher rates of water loss and solid gain during osmotic dehydration were also obtained by PEF pretreatment, reducing the dehydration time from 130 to 48h. PEF pretreated and dehydrated fruits showed superior appearance to thermally pretreated and control samples. Therefore, PEF pretreatment is a preferred technology that balances nutritive quality, appearance, and dehydration rate. Published by Elsevier Ltd.

Comparative transcriptome analysis of differentially expressed genes in foxtail millet (Setaria italica L.) during dehydration stress.

Science.gov (United States)

Lata, Charu; Sahu, Pranav Pankaj; Prasad, Manoj

2010-03-19

Dehydration stress is one of the most important abiotic stresses that adversely influence crop growth and productivity. With the aim to understand the molecular mechanisms underlying dehydration stress tolerance in foxtail millet (Setaria italica L.), a drought tolerant crop, we examined its transcriptome changes at two time points (early and late) of dehydration stress. Two suppression subtractive hybridization (SSH) forward libraries were constructed from 21-day old seedlings of tolerant cv. Prasad at 0.5 and 6h PEG-induced dehydration stress. A total of 327 unique ESTs were identified from both libraries and were classified into 11 different categories according to their putative functions. The plant response against dehydration stress was complex, representing major transcripts involved in metabolism, stress, signaling, transcription regulation, translation and proteolysis. By Reverse Northern (RN) technique we identified the differential expression pattern of 327 transcripts, 86 (about 26%) of which showed > or = 1.7-fold induction. Further the obtained results were validated by quantitative real-time PCR (qRT-PCR) to have a comparative expression profiling of randomly chosen 9 up-regulated transcripts (> or =2.5 fold induction) between cv. Prasad (tolerant) and cv. Lepakshi (sensitive) upon dehydration stress. These transcripts showed a differential expression pattern in both cultivars at different time points of stress treatment as analyzed by qRT-PCR. The possible relationship of the identified transcripts with dehydration tolerance mechanism is discussed. Copyright 2010 Elsevier Inc. All rights reserved.

Dehydration Stress Contributes to the Enhancement of Plant Defense Response and Mite Performance on Barley

Directory of Open Access Journals (Sweden)

M. E. Santamaria

2018-04-01

Full Text Available Under natural conditions, plants suffer different stresses simultaneously or in a sequential way. At present, the combined effect of biotic and abiotic stressors is one of the most important threats to crop production. Understanding how plants deal with the panoply of potential stresses affecting them is crucial to develop biotechnological tools to protect plants. As well as for drought stress, the economic importance of the spider mite on agriculture is expected to increase due to climate change. Barley is a host of the polyphagous spider mite Tetranychus urticae and drought produces important yield losses. To obtain insights on the combined effect of drought and mite stresses on the defensive response of this cereal, we have analyzed the transcriptomic responses of barley plants subjected to dehydration (water-deficit treatment, spider mite attack, or to the combined dehydration-spider mite stress. The expression patterns of mite-induced responsive genes included many jasmonic acid responsive genes and were quickly induced. In contrast, genes related to dehydration tolerance were later up-regulated. Besides, a higher up-regulation of mite-induced defenses was showed by the combined dehydration and mite treatment than by the individual mite stress. On the other hand, the performance of the mite in dehydration stressed and well-watered plants was tested. Despite the stronger defensive response in plants that suffer dehydration and mite stresses, the spider mite demonstrates a better performance under dehydration condition than in well-watered plants. These results highlight the complexity of the regulatory events leading to the response to a combination of stresses and emphasize the difficulties to predict their consequences on crop production.

Dehydration Stress Contributes to the Enhancement of Plant Defense Response and Mite Performance on Barley

Science.gov (United States)

Santamaria, M. E.; Diaz, Isabel; Martinez, Manuel

2018-01-01

Under natural conditions, plants suffer different stresses simultaneously or in a sequential way. At present, the combined effect of biotic and abiotic stressors is one of the most important threats to crop production. Understanding how plants deal with the panoply of potential stresses affecting them is crucial to develop biotechnological tools to protect plants. As well as for drought stress, the economic importance of the spider mite on agriculture is expected to increase due to climate change. Barley is a host of the polyphagous spider mite Tetranychus urticae and drought produces important yield losses. To obtain insights on the combined effect of drought and mite stresses on the defensive response of this cereal, we have analyzed the transcriptomic responses of barley plants subjected to dehydration (water-deficit) treatment, spider mite attack, or to the combined dehydration-spider mite stress. The expression patterns of mite-induced responsive genes included many jasmonic acid responsive genes and were quickly induced. In contrast, genes related to dehydration tolerance were later up-regulated. Besides, a higher up-regulation of mite-induced defenses was showed by the combined dehydration and mite treatment than by the individual mite stress. On the other hand, the performance of the mite in dehydration stressed and well-watered plants was tested. Despite the stronger defensive response in plants that suffer dehydration and mite stresses, the spider mite demonstrates a better performance under dehydration condition than in well-watered plants. These results highlight the complexity of the regulatory events leading to the response to a combination of stresses and emphasize the difficulties to predict their consequences on crop production. PMID:29681917

Associations Between Dehydration, Cognitive Impairment, and Frailty in Older Hospitalized Patients: An Exploratory Study.

Science.gov (United States)

McCrow, Judy; Morton, Margaret; Travers, Catherine; Harvey, Keren; Eeles, Eamonn

2016-05-01

HOW TO OBTAIN CONTACT HOURS BY READING THIS ARTICLE INSTRUCTIONS 1.2 contact hours will be awarded by Villanova University College of Nursing upon successful completion of this activity. A contact hour is a unit of measurement that denotes 60 minutes of an organized learning activity. This is a learner-based activity. Villanova University College of Nursing does not require submission of your answers to the quiz. A contact hour certificate will be awarded once you register, pay the registration fee, and complete the evaluation form online at http://goo.gl/gMfXaf. To obtain contact hours you must: 1. Read the article, "Associations Between Dehydration, Cognitive Impairment, and Frailty in Older Hospitalized Patients: An Exploratory Study" found on pages 19-27, carefully noting any tables and other illustrative materials that are included to enhance your knowledge and understanding of the content. Be sure to keep track of the amount of time (number of minutes) you spend reading the article and completing the quiz. 2. Read and answer each question on the quiz. After completing all of the questions, compare your answers to those provided within this issue. If you have incorrect answers, return to the article for further study. 3. Go to the Villanova website listed above to register for contact hour credit. You will be asked to provide your name; contact information; and a VISA, MasterCard, or Discover card number for payment of the $20.00 fee. Once you complete the online evaluation, a certificate will be automatically generated. This activity is valid for continuing education credit until April 30, 2019. CONTACT HOURS This activity is co-provided by Villanova University College of Nursing and SLACK Incorporated. Villanova University College of Nursing is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center's Commission on Accreditation. ACTIVITY OBJECTIVES 1. Describe the incidence of dehydration in older hospitalized

Iron in hereditary retinal degeneration: PIXE microanalysis Preliminary results

International Nuclear Information System (INIS)

Sergeant, C.; Gouget, B.; Llabador, Y.; Simonoff, M.; Yefimova, M.; Courtois, Y.; Jeanny, J.C.

1999-01-01

Several types of hereditary retinal degeneration with progressive alteration of photoreceptors exist in men and animals. Recent immunohistochemical results have shown strong degradation of transferrin, the protein responsible for iron transport, in retinas of rats with hereditary retinal degeneration. Freeze-dried thin sections of rat retinas from different stages of the disease, and respective coeval control sections, have been analyzed using nuclear microprobe. In this first part of the study, the rat retinas at post-natal stages of 35 and 45 days have been analyzed. The sample preparation and the post-irradiation staining to determine precisely the retinal layers involved are described. Preliminary results of element distributions (K, Ca, Fe) in the rat retina layers are discussed. A very high content of calcium in the choriocapillaris of dystrophic rat retinas was observed. Preliminary results on iron distribution in the rat retina layers are presented

Ethical, social and counselling issues in hereditary cancer susceptibility.

Science.gov (United States)

Garber, J E; Patenaude, A F

1995-01-01

Genetic testing for hereditary susceptibility to disease is new. Much has been learned from experience with Huntington's disease and other non-malignant conditions. There are some differences in the case of predisposition testing for cancer: there is often the perception that cancer is preventable and sometimes curable, in contrast to other hereditary conditions. Testing raises many issues new to the medical community and to the public as well. There is great concern that the explosive technology be used responsibly, so that the potential benefits of genetic knowledge are not eclipsed by the risks to autonomy, privacy and justice. Practical concerns about insurability and discrimination may inhibit some at risk individuals from taking advantage of this powerful technology. There has been considerable effort already in the UK, Europe and the USA at the research and social levels to create protection for individuals found to carry genetic susceptibility to disease.

NOVEL POLYMERIC MEMBRANE FOR DEHYDRATION OF ORGANIC SOLVENTS

Science.gov (United States)

Pervaporation has emerged as an economically viable alternative technology for dehydration of organic solvents, removal of organic compounds and organic/organic separations. Development of a membrane system with suitable flux and selectivity characteristics plays a critical role...

Hetero-azeotropic distillation: combining fungal dehydration and lipid extraction.

Science.gov (United States)

Tough, A J; Isabella, B L; Beattie, J E; Herbert, R A

2000-01-01

A low-cost single-stage laboratory process combining fungal dehydration and lipid extraction was compared with a traditional two-stage method employing freeze-drying and subsequent mechanical disruption in the presence of solvent. The ability of a number of organic solvents to form hetero-azeotropes with water was exploited. Chloroform, cyclohexane and hexane were assessed in their abilities to both dry and extract lipid from the oleaginous phycomycete Mortierella alpina (ATCC 32222). Drying rate and lipid extraction were maximised under conditions that prevented fungal agglomeration. The total processing time was limited by the rate of dehydration rather than by the rate of lipid extraction. In all cases azeotropic distillation facilitated a greater rate of dehydration than was possible with freeze-drying. A consequent reduction in overall processing time was observed. Uniquely, both the solvent used and the mode of mixing employed controlled the morphology of the aggregates formed during distillation. In combination with mild mixing chloroform discouraged agglomeration whereas cyclohexane and hexane promoted aggregation. Successful lipid extraction was dependent on the use of dry biomass rather than on the application of heat to effect distillation. Neither the application of heat nor the solvent employed had any significant effect on the lipid composition of the extracted oil.

Preventing and treating dehydration in the elderly during periods of illness and warm weather

NARCIS (Netherlands)

Schols, J.M.G.A.; Groot, de C.P.G.M.; Cammen, van der T.J.M.; Olde Rikkert, M.G.M.

2009-01-01

Objective: Translate the available knowledge on ageing and dehydration into main messages for clinical practice. Main points: Older people are more susceptible to dehydration than younger people. This is partly due to lack of thirst sensation and changes in the water and sodium balance that

Preventing and treating dehydration in the elderly during periods of illness and warm weather.

NARCIS (Netherlands)

Schols, J.M.; Groot, C.P.G.M. de; Cammen, T.J.M. van der; Olde Rikkert, M.G.M.

2009-01-01

OBJECTIVE: Translate the available knowledge on ageing and dehydration into main messages for clinical practice. MAIN POINTS: Older people are more susceptible to dehydration than younger people. This is partly due to lack of thirst sensation and changes in the water and sodium balance that

Iron-Catalyzed Dehydration of Aldoximes to Nitriles Requiring Neither Other Reagents Nor Nitrile Media.

Science.gov (United States)

Hyodo, Kengo; Kitagawa, Saki; Yamazaki, Masayuki; Uchida, Kingo

2016-05-06

The dehydration of aldoximes is an environmentally benign reaction affording the desired nitrile and water as a by-product. However, most of the reported catalytic dehydration reactions of aldoximes require a solvent containing nitrile to synthesize the corresponding nitrile compounds. Inspired by recent reports on the enzymatic synthesis under nitrile-free conditions, we here describe that a simple iron salt catalyzes the dehydration of aldoximes requiring neither other reagents nor nitrile media. Our method can be applied to the one-pot synthesis of nitiriles from aldehydes. © 2016 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

Heat integrated ethanol dehydration flowsheets

Energy Technology Data Exchange (ETDEWEB)

Hutahaean, L.S.; Shen, W.H.; Brunt, V. Van [Univ. of South Carolina, Columbia, SC (United States)

1995-04-01

zA theoretical evaluation of heat-integrated heterogeneous-azeotropic ethanol-water distillation flowsheets is presented. Simulations of two column flowsheets using several different hydrocarbon entrainers reveal a region of potential heat integration and substantial reduction in operating energy. In this paper, methods for comparing hydrocarbon entrainers are shown. Two aspects of entrainers are related to operating and capital costs. The binary azeotropic composition of the entrainer-ethanol mixture is related to the energy requirements of the flowsheet. A temperature difference in the azeotrophic column is related to the size of the column and overall process staging requirements. Although the hydrophobicity of an entrainer is essential for specification of staging in the dehydration column, no substantial increase in operating energy results from an entrainer that has a higher water content. Likewise, liquid-liquid equilibria between several entrainer-ethanol-water mixtures have no substantial effect on either staging or operation. Rather, increasing the alcohol content of the entrainer-ethanol azeotrope limits its recovery in the dehydration column, and increases the recycle and reflux streams. These effects both contribute to increasing the separation energy requirements and reducing the region of potential heat integration. A cost comparison with a multieffect extractive distillation flowsheet reveals that the costs are comparable; however, the extractive distillation flowsheet is more cost effective as operating costs increase.

Comprehensive Evaluation of dehydration impact on ocular tissue during Ramadan fasting

Directory of Open Access Journals (Sweden)

Javad Heravian

2015-05-01

Full Text Available Purpose: The present study aimed to review the effect of dehydration during Ramadan fasting on the health and ocular parameters leading to changes in eye function. Methods: Articles included in the study were taken from Pub Med, Ovid, Web of Science and Google Scholar up to 2014.Related articles were also obtained from scientific journals in fasting and eye. Results: Dehydration and nutrition changes in Ramadan cause an increase in tear osmolarity, ocular aberration, anterior chamber depth, IOL measurement, central corneal thickness, retinal and choroidal thickness. And also a decrease in IOP, tear secretion, and vitreous thickness. Conclusion: Besides many research in relation to effect of dehydration impact on ocular parameters during Ramadan fasting, although the findings reveal it is associated with significant changes on ocular parameters. It seems requisite to have comprehensive study in "fasting and ocular parameters” which will be helpful in making decision and giving plan to the patients.

Dehydration Influences Mood and Cognition: A Plausible Hypothesis?

Science.gov (United States)

Benton, David

2011-01-01

The hypothesis was considered that a low fluid intake disrupts cognition and mood. Most research has been carried out on young fit adults, who typically have exercised, often in heat. The results of these studies are inconsistent, preventing any conclusion. Even if the findings had been consistent, confounding variables such as fatigue and increased temperature make it unwise to extrapolate these findings. Thus in young adults there is little evidence that under normal living conditions dehydration disrupts cognition, although this may simply reflect a lack of relevant evidence. There remains the possibility that particular populations are at high risk of dehydration. It is known that renal function declines in many older individuals and thirst mechanisms become less effective. Although there are a few reports that more dehydrated older adults perform cognitive tasks less well, the body of information is limited and there have been little attempt to improve functioning by increasing hydration status. Although children are another potentially vulnerable group that have also been subject to little study, they are the group that has produced the only consistent findings in this area. Four intervention studies have found improved performance in children aged 7 to 9 years. In these studies children, eating and drinking as normal, have been tested on occasions when they have and not have consumed a drink. After a drink both memory and attention have been found to be improved. PMID:22254111

Hereditary hemochromatosis and risk of ischemic heart disease

DEFF Research Database (Denmark)

Ellervik, Christina; Tybjaerg-Hansen, Anne; Grande, Peer

2005-01-01

BACKGROUND: We tested the hypothesis that the hereditary hemochromatosis genotypes C282Y/C282Y, C282Y/H63D, or C282Y/wild-type are risk factors for ischemic heart disease (IHD) and myocardial infarction (MI). METHODS AND RESULTS: We performed a prospective study of 9178 individuals from the Danish...

Suspected Perinatal Depression Revealed to be Hereditary Diffuse Leukoencephalopathy with Spheroids.

Science.gov (United States)

Blume, Josefine; Weissert, Robert

2017-01-01

Early motor symptoms of neurodegenerative diseases often appear in combination with psychiatric symptoms, such as depression or personality changes, and are in danger of being misdiagnosed as psychogenic in young patients. We present the case of a 32-year-old woman who presented with rapid-onset depression, followed by a hypokinetic movement disorder and cognitive decline during pregnancy. Genetic testing revealed a mutation in the colony-stimulating factor 1 receptor gene, which led to the diagnosis of hereditary diffuse leukoencephalopathy with spheroids. Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is probably an under-recognized disease. HDLS should be considered in patients with rapidly progressing parkinsonian symptoms and dementia accompanied by white matter lesions.

Suspected Perinatal Depression Revealed to be Hereditary Diffuse Leukoencephalopathy with Spheroids

Directory of Open Access Journals (Sweden)

Josefine Blume

2017-01-01

Full Text Available Early motor symptoms of neurodegenerative diseases often appear in combination with psychiatric symptoms, such as depression or personality changes, and are in danger of being misdiagnosed as psychogenic in young patients. We present the case of a 32-year-old woman who presented with rapid-onset depression, followed by a hypokinetic movement disorder and cognitive decline during pregnancy. Genetic testing revealed a mutation in the colony-stimulating factor 1 receptor gene, which led to the diagnosis of hereditary diffuse leukoencephalopathy with spheroids. Hereditary diffuse leukoencephalopathy with spheroids (HDLS is probably an under-recognized disease. HDLS should be considered in patients with rapidly progressing parkinsonian symptoms and dementia accompanied by white matter lesions.

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.

Experimental Evaluation of Mechanical Dehydration of Nigerian ...

African Journals Online (AJOL)

However the quality of the products produced at the higher temperatures is somewhat lower than that of 500C. The results indicate a need for further investigation of the fermentation process. dehydration at lower temperatures than 50°C, and the quantitative analysis of the theobromine content of the finished product.

Leber's hereditary optic neuropathy and vitamin B12 deficiency

NARCIS (Netherlands)

Pott, Jan Willem R.; Wong, Kwok H.

2006-01-01

Background: Leber's hereditary optic neuropathy (LHON) is a maternally inherited optic neuropathy caused by mutations in mitochondrial DNA (mtDNA). It is also believed that several epigenetic factors have an influence on the development of LHON. Methods: A case series was observed. Results: Three

On the many faces of Leber hereditary optic neuropathy

NARCIS (Netherlands)

Oostra, RJ; Tijmes, NT; Cobben, JM; Bolhuis, PA; vanNesselrooij, BPM; Houtman, WA; deKokNazaruk, MM; BleekerWagemakers, EM

Leber hereditary optic neuropathy (LHON) is a maternally inherited disorder, associated with mutations in the mitochondrial DNA, which is notorious for its aspecific presentations. Two pedigrees are described with cases that are atypical for LHON with respect to sex, age of onset, interval between

On the many faces of Leber hereditary optic neuropathy

NARCIS (Netherlands)

Oostra, R. J.; Tijmes, N. T.; Cobben, J. M.; Bolhuis, P. A.; van Nesselrooij, B. P.; Houtman, W. A.; de Kok-Nazaruk, M. M.; Bleeker-Wagemakers, E. M.

1997-01-01

Leber hereditary optic neuropathy (LHON) is a maternally inherited disorder, associated with mutations in the mitochondrial DNA, which is notorious for its aspecific presentations. Two pedigrees are described with cases that are atypical for LHON with respect to sex, age of onset, interval between

Study of glycolytic intermediates in hereditary elliptocytosis with thalassemia

Directory of Open Access Journals (Sweden)

Pavri Roshan

1977-01-01

Full Text Available Glycolytic intermediates like ATP, DPG and GSH have been studied in a family with. hereditary elliptocytosis and thalassemia. Results indicate a fall in ATP with a concomitant rise in DPG in the Patient. Findings are discussed in relation to other data.

Construction and application of EST library from Setaria italica in response to dehydration stress.

Science.gov (United States)

Zhang, Jinpeng; Liu, Tingsong; Fu, Junjie; Zhu, Yun; Jia, Jinping; Zheng, Jun; Zhao, Yinhe; Zhang, Ying; Wang, Guoying

2007-07-01

Foxtail millet is a gramineous crop with low water requirement. Despite its high water use efficiency, less attention has been paid to the molecular genetics of foxtail millet. This article reports the construction of subtracted cDNA libraries from foxtail millet seedlings under dehydration stress and the expression profile analysis of 1947 UniESTs from the subtracted cDNA libraries by a cDNA microarray. The results showed that 95 and 57 ESTs were upregulated by dehydration stress, respectively, in roots and shoots of seedlings and that 10 and 27 ESTs were downregulated, respectively, in roots and shoots. The expression profile analysis showed that genes induced in foxtail millet roots were different from those in shoots during dehydration stress and that the early response to dehydration stress in foxtail millet roots was the activation of the glycolysis metabolism. Moreover, protein degradation pathway may also play a pivotal role in drought-tolerant responses of foxtail millet. Finally, Northern blot analysis validated well the cDNA microarray data.

Plasma water as a diagnostic tool in the assessment of dehydration in children with acute gastroenteritis.

Science.gov (United States)

Plaisier, Annemarie; Maingay-de Groof, Femke; Mast-Harwig, Roechama; Kalkman, Patricia M J; Wulkan, Remi W; Verwers, Renee; Neele, Marjolein; Hop, Wim C J; Groeneweg, Michael

2010-07-01

Acute gastroenteritis is common in childhood. The estimation of the degree of dehydration is essential for management of acute gastroenteritis. Plasma water was assessed as a diagnostic tool in children with acute gastroenteritis and dehydration admitted to hospital. In a prospective cohort study, 101 patients presenting at the emergency department with dehydration were included. Clinical assessment, routine laboratory tests, and plasma water measurement were performed. Plasma water was measured as a percentage of water content using dry weight method. During admission, patients were rehydrated in 12 h. Weight gain at the end of the rehydration period and 2 weeks thereafter was used to determine the percentage of weight loss as a gold standard for the severity of dehydration. Clinical assessment of dehydration was not significantly associated with the percentage of weight loss. Blood urea nitrogen (r = 0.3, p = 0.03), base excess (r =-0.31, p = 0.03), and serum bicarbonate (r = 0.32, p = 0.02) were significantly correlated with the percentage of weight loss. Plasma water did not correlate with the percentage of weight loss. On the basis of the presented data, plasma water should not be used as a diagnostic tool in the assessment of dehydration in children with acute gastroenteritis.

Dehydration in the tropical tropopause layer estimated from the water vapor match

Directory of Open Access Journals (Sweden)

Y. Inai

2013-09-01

Full Text Available We apply the match technique, whereby the same air mass is observed more than once and such cases are termed a "match", to study the dehydration process associated with horizontal advection in the tropical tropopause layer (TTL over the western Pacific. The matches are obtained from profile data taken by the Soundings of Ozone and Water in the Equatorial Region (SOWER campaign network observations using isentropic trajectories calculated from European Centre for Medium-Range Weather Forecasts (ECMWF operational analyses. For the matches identified, extensive screening procedures are performed to verify the representativeness of the air parcel and the validity of the isentropic treatment, and to check for possible water injection by deep convection, consistency between the sonde data and analysis field referring to the ozone conservation. Among the matches that passed the screening tests, we identified some cases corresponding to the first quantitative value of dehydration associated with horizontal advection in the TTL. The statistical features of dehydration for the air parcels advected in the lower TTL are derived from the matches. The threshold of nucleation is estimated to be 146 ± 1% (1σ in relative humidity with respect to ice (RHice, while dehydration seems to continue until RHice reaches about 75 ± 23% (1σ in the altitude region from 350 to 360 K. The efficiency of dehydration expressed by the relaxation time required for the supersaturated air parcel to approach saturation is empirically determined from the matches. A relaxation time of approximately one hour reproduces the second water vapor observation reasonably well, given the first observed water vapor amount and the history of the saturation mixing ratio during advection in the lower TTL.

Hereditary hemorrhagic telangiectasia: genetics and molecular diagnostics in a new era

Directory of Open Access Journals (Sweden)

Jamie eMcDonald

2015-01-01

Full Text Available Hereditary hemorrhagic telangiectasia (HHT is a vascular dysplasia characterized by telangiectases and arteriovenous malformations (AVMs in particular locations described in consensus clinical diagnostic criteria published in 2000. Two genes in the transforming growth factor-beta (TGF-β signaling pathway, ENG and ACVRL1, were discovered almost two decades ago, and mutations in these genes have been reported to cause up to 85% of HHT. In our experience, approximately 96% of individuals with HHT have a mutation in these two genes, when published (Curaçao diagnostic criteria for HHT are strictly applied. More recently, two additional genes in the same pathway, SMAD4 and GDF2, have been identified in a much smaller number of patients with a similar or overlapping phenotype to HHT. Yet families still exist with compelling evidence of a hereditary telangiectasia disorder, but no identifiable mutation in a known gene. Recent availability of whole exome and genome testing has created new opportunities to facilitate gene discovery, identify genetic modifiers to explain clinical variability, and potentially define an increased spectrum of hereditary telangiectasia disorders. An expanded approach to molecular diagnostics for inherited telangiectasia disorders that incorporates a multi-gene next generation sequencing (NGS HHT panel is proposed.

Hydrous mineral dehydration around heat-generating nuclear waste in bedded salt formations.

Science.gov (United States)

Jordan, Amy B; Boukhalfa, Hakim; Caporuscio, Florie A; Robinson, Bruce A; Stauffer, Philip H

2015-06-02

Heat-generating nuclear waste disposal in bedded salt during the first two years after waste emplacement is explored using numerical simulations tied to experiments of hydrous mineral dehydration. Heating impure salt samples to temperatures of 265 °C can release over 20% by mass of hydrous minerals as water. Three steps in a series of dehydration reactions are measured (65, 110, and 265 °C), and water loss associated with each step is averaged from experimental data into a water source model. Simulations using this dehydration model are used to predict temperature, moisture, and porosity after heating by 750-W waste canisters, assuming hydrous mineral mass fractions from 0 to 10%. The formation of a three-phase heat pipe (with counter-circulation of vapor and brine) occurs as water vapor is driven away from the heat source, condenses, and flows back toward the heat source, leading to changes in porosity, permeability, temperature, saturation, and thermal conductivity of the backfill salt surrounding the waste canisters. Heat pipe formation depends on temperature, moisture availability, and mobility. In certain cases, dehydration of hydrous minerals provides sufficient extra moisture to push the system into a sustained heat pipe, where simulations neglecting this process do not.

Mechanisms and prevention of plant tissue collapse during dehydration: a critical review.

Science.gov (United States)

Prothon, Frédéric; Ahrné, Lilia; Sjöholm, Ingegerd

2003-01-01

The appearance and functional properties are primordial in the quality assessment of semifinished fruit and vegetable products. These properties are often associated with shrunken, shriveled, darkened materials of poor rehydration ability after been subjected to air-drying--the most used drying method in the food industry. Fruits and vegetables are cellular tissues containing gas-filled pores that tend to collapse when subjected to dehydration. Collapse is an overall term that has different meanings and scale-settings in the literature depending on whether the author is a plant physiologist, a food technologist, a chemical engineer, or a material scientist. Some clarifications are given in this particular but wide field. The purpose of this work was to make a state-of-the-art contribution to the structural and textural effects of different types of dehydration on edible plant products and give a basis for preventing this phenomenon. The plant tissue is described, and the primordial role of the cell wall in keeping the structural integrity is emphasized. Water and its functionality at macro and micro levels of the cellular tissue are reviewed as well as its transport during dehydration. The effects of both dehydration and rehydration are described in detail, and the term "textural collapse" is proposed as an alternative to structural collapse.

Effect of process variables on the osmotic dehydration of star-fruit slices

Directory of Open Access Journals (Sweden)

Camila Dalben Madeira Campos

2012-06-01

Full Text Available The objective of this work was to study the effect of blanching and the influence of temperature, solution concentration, and the initial fruit:solution ratio on the osmotic dehydration of star-fruit slices. For blanching, different concentrations of citric and ascorbic acids were studied. The samples immersed in 0.75% citric acid presented little variation in color in relation to the fresh star-fruit. Osmotic dehydration was carried out in an incubator with orbital shaking, controlled temperature, and constant shaking at 120 rpm. The influence of process variables was studied in trials defined by a complete 23 central composite design. In general, water loss and solids gain were positively influenced by temperature and by solution concentration. Nevertheless, lower temperatures reduced water loss throughout the osmotic dehydration process. An increase in the amount of dehydrating solution (initial fruit:solution ratio slightly influenced the evaluated responses. The process carried out at 50 ºC with a solution concentration of 50% resulted in a product with lower solids gain and greater water loss. Under these conditions, blanching minimized the effect of the osmotic treatment on star-fruit browning, and therefore the blanched fruits showed little variation in color in relation to the fresh fruit.

Hereditary colorectal cancer diagnostics

DEFF Research Database (Denmark)

Klarskov, Louise; Holck, Susanne; Bernstein, Inge

2012-01-01

BackgroundThe hereditary non-polyposis colorectal cancer (HNPCC) subset of tumours can broadly be divided into tumours caused by an underlying mismatch-repair gene mutation, referred to as Lynch syndrome, and those that develop in families with similar patterns of heredity but without disease......-predisposing germline mismatch repair mutations, referred to as familial colorectal cancer type X (FCCTX). Recognition of HNPCC-associated colorectal cancers is central since surveillance programmes effectively reduce morbidity and mortality. The characteristic morphological features linked to Lynch syndrome can aid...... in the identification of this subset, whereas the possibility to use morphological features as an indicator of FCCTX is uncertain.Objective and methodsTo perform a detailed morphological evaluation of HNPCC-associated colorectal cancers and demonstrate significant differences between tumours associated with FCCTX...

Dehydration of 2-Methyl-1-Cyclohexanol: New Findings from a Popular Undergraduate Laboratory Experiment

Science.gov (United States)

Friesen, J. Brent; Schretzman, Robert

2011-01-01

The mineral acid-catalyzed dehydration of 2-methyl-1-cyclohexanol has been a popular laboratory exercise in second-year organic chemistry for several decades. The dehydration experiment is often performed by organic chemistry students to illustrate Zaitsev's rule. However, sensitive analytical techniques reveal that the results do not entirely…

Combined pulmonary involvement in hereditary lysozyme amyloidosis with associated pulmonary sarcoidosis: a case report.

Science.gov (United States)

McCarthy, Cormac; Deegan, Alexander P; Garvey, John F; McDonnell, Timothy J

2013-12-17

Sarcoidosis is a multisystem inflammatory disorder of unknown cause which can affect any organ system. Autosomal dominant lysozyme amyloidosis is a very rare form of hereditary amyloidosis. The Arg64 variant is extraordinarily rare with each family showing a particular pattern of organ involvement, however while Sicca syndrome, gastrointestinal involvement and renal failure are common, lymph node involvement is very rare. In this case report we describe the first reported case of sarcoidosis in association with hereditary lysozyme amyloidosis.

Dehydration-induced release of vasopressin involves activation of hypothalamic histaminergic neurons.

Science.gov (United States)

Kjaer, A; Knigge, U; Rouleau, A; Garbarg, M; Warberg, J

1994-08-01

The hypothalamic neurotransmitter histamine (HA) induces arginine vasopressin (AVP) release when administered centrally. We studied and characterized this effect of HA with respect to receptor involvement. In addition, we studied the possible role of hypothalamic histaminergic neurons in the mediation of a physiological stimulus (dehydration) for AVP secretion. Intracerebroventricular administration of HA, the H1-receptor agonists 2(3-bromophenyl)HA and 2-thiazolylethylamine, or the H2-receptor agonists amthamine or 4-methyl-HA stimulated AVP secretion. The stimulatory action of HA on AVP was inhibited by pretreatment with the H1-receptor antagonist mepyramine or the H2-receptor antagonist cimetidine. Twenty-four hours of dehydration elevated the plasma osmolality from 298 +/- 3 to 310 +/- 3 mmol/liter and increased the plasma AVP concentration 4-fold. The hypothalamic content of HA and its metabolite tele-methyl-HA was elevated in response to dehydration, indicating an increased synthesis and release of hypothalamic HA. Dehydration-induced AVP secretion was lowered when neuronal HA synthesis was inhibited by the administration of (S) alpha-fluoromethylhistidine or when the animals were pretreated with the H3-receptor agonist R(alpha)methylhistamine, which inhibits the release and synthesis of HA, the H1-receptor antagonists mepyramine and cetirizine, or the H2-receptor antagonists cimetidine and ranitidine. We conclude that HA, via activation of both H1- and H2-receptors, stimulates AVP release and that HA is a physiological regulator of AVP secretion.

Hereditary arrhythmias and cardiomyopathies: decision-making about genetic testing.

Science.gov (United States)

Louis, Clauden; Calamaro, Emily; Vinocur, Jeffrey M

2018-01-01

The modern field of clinical genetics has advanced beyond the traditional teachings familiar to most practicing cardiologists. Increased understanding of the roles of genetic testing may improve uptake and appropriateness of use. Clinical genetics has become integral to the management of patients with hereditary arrhythmia and cardiomyopathy diagnoses. Depending on the condition, genetic testing may be useful for diagnosis, prognosis, treatment, family screening, and reproductive planning. However, genetic testing is a powerful tool with potential for underuse, overuse, and misuse. In the absence of a substantial body of literature on how these guidelines are applied in clinical practice, we use a case-based approach to highlight key lessons and pitfalls. Importantly, in many scenarios genetic testing has become the standard of care supported by numerous class I recommendations; genetic counselors can improve accessibility to and appropriate use and application of testing. Optimal management of hereditary arrhythmias and cardiomyopathies incorporates genetic testing, applied as per consensus guidelines, with involvement of a multidisciplinary team.

Dehydration accelerates reductions in cerebral blood flow during prolonged exercise in the heat without compromising brain metabolism.

Science.gov (United States)

Trangmar, Steven J; Chiesa, Scott T; Llodio, Iñaki; Garcia, Benjamin; Kalsi, Kameljit K; Secher, Niels H; González-Alonso, José

2015-11-01

Dehydration hastens the decline in cerebral blood flow (CBF) during incremental exercise, whereas the cerebral metabolic rate for O2 (CMRO2 ) is preserved. It remains unknown whether CMRO2 is also maintained during prolonged exercise in the heat and whether an eventual decline in CBF is coupled to fatigue. Two studies were undertaken. In study 1, 10 male cyclists cycled in the heat for ∼2 h with (control) and without fluid replacement (dehydration) while internal and external carotid artery blood flow and core and blood temperature were obtained. Arterial and internal jugular venous blood samples were assessed with dehydration to evaluate CMRO2 . In study 2, in 8 male subjects, middle cerebral artery blood velocity was measured during prolonged exercise to exhaustion in both dehydrated and euhydrated states. After a rise at the onset of exercise, internal carotid artery flow declined to baseline with progressive dehydration (P dehydration accelerates the decline in CBF without affecting CMRO2 and also restricts extracranial perfusion. Thus, fatigue is related to a reduction in CBF and extracranial perfusion rather than CMRO2 . Copyright © 2015 the American Physiological Society.

Comparative study of the loss of vitamin C in chalarina (Casimiroa edulis by four methods of dehydration

Directory of Open Access Journals (Sweden)

Judith Castañeda

2010-03-01

Full Text Available The aim of this study was to compare the loss of chalarina vitamin C after applying four methods of dehydration: vacuum osmotic dehydration combined with convective drying, convective drying, osmotic dehydration combined with vacuum freeze-drying, and freeze-drying. The fresh chalarina was fitted into cubes of 1 cm; we determined the vitamin C content and other chemical properties of the pulp. The powders were characterized: vitamin C, moisture content, aw, density, angle of flow and compressibility. Fresh chalarina pulp has a vitamin C content of 29.75 mg/100g. Osmotic dehydration and vacuum reduced to 15.75 mg/100g this content; combined with convective drying is reached 9.31 mg/100g; if combined with freeze-drying is obtained 14.98 mg/100g. Applying convective drying was obtained only 7.05 mg of vitaminaC/100g chalarina powder, while by applying only freeze-drying was obtained 23.63 mg/100g. We conclude that the powder obtained by vacuum osmotic dehydration - convective drying has better physical and chemical characteristics of powders obtained with other methods of dehydration.

Recommendations regarding splenectomy in hereditary hemolytic anemias

Science.gov (United States)

Iolascon, Achille; Andolfo, Immacolata; Barcellini, Wilma; Corcione, Francesco; Garçon, Loïc; De Franceschi, Lucia; Pignata, Claudio; Graziadei, Giovanna; Pospisilova, Dagmar; Rees, David C.; de Montalembert, Mariane; Rivella, Stefano; Gambale, Antonella; Russo, Roberta; Ribeiro, Leticia; Vives-Corrons, Jules; Martinez, Patricia Aguilar; Kattamis, Antonis; Gulbis, Beatrice; Cappellini, Maria Domenica; Roberts, Irene; Tamary, Hannah

2017-01-01

Hereditary hemolytic anemias are a group of disorders with a variety of causes, including red cell membrane defects, red blood cell enzyme disorders, congenital dyserythropoietic anemias, thalassemia syndromes and hemoglobinopathies. As damaged red blood cells passing through the red pulp of the spleen are removed by splenic macrophages, splenectomy is one possible therapeutic approach to the management of severely affected patients. However, except for hereditary spherocytosis for which the effectiveness of splenectomy has been well documented, the efficacy of splenectomy in other anemias within this group has yet to be determined and there are concerns regarding short- and long-term infectious and thrombotic complications. In light of the priorities identified by the European Hematology Association Roadmap we generated specific recommendations for each disorder, except thalassemia syndromes for which there are other, recent guidelines. Our recommendations are intended to enable clinicians to achieve better informed decisions on disease management by splenectomy, on the type of splenectomy and the possible consequences. As no randomized clinical trials, case control or cohort studies regarding splenectomy in these disorders were found in the literature, recommendations for each disease were based on expert opinion and were subsequently critically revised and modified by the Splenectomy in Rare Anemias Study Group, which includes hematologists caring for both adults and children. PMID:28550188

Development and validation of a 36-gene sequencing assay for hereditary cancer risk assessment

Directory of Open Access Journals (Sweden)

Valentina S. Vysotskaia

2017-02-01

Full Text Available The past two decades have brought many important advances in our understanding of the hereditary susceptibility to cancer. Numerous studies have provided convincing evidence that identification of germline mutations associated with hereditary cancer syndromes can lead to reductions in morbidity and mortality through targeted risk management options. Additionally, advances in gene sequencing technology now permit the development of multigene hereditary cancer testing panels. Here, we describe the 2016 revision of the Counsyl Inherited Cancer Screen for detecting single-nucleotide variants (SNVs, short insertions and deletions (indels, and copy number variants (CNVs in 36 genes associated with an elevated risk for breast, ovarian, colorectal, gastric, endometrial, pancreatic, thyroid, prostate, melanoma, and neuroendocrine cancers. To determine test accuracy and reproducibility, we performed a rigorous analytical validation across 341 samples, including 118 cell lines and 223 patient samples. The screen achieved 100% test sensitivity across different mutation types, with high specificity and 100% concordance with conventional Sanger sequencing and multiplex ligation-dependent probe amplification (MLPA. We also demonstrated the screen’s high intra-run and inter-run reproducibility and robust performance on blood and saliva specimens. Furthermore, we showed that pathogenic Alu element insertions can be accurately detected by our test. Overall, the validation in our clinical laboratory demonstrated the analytical performance required for collecting and reporting genetic information related to risk of developing hereditary cancers.

Hepatitis C infection in patients with hereditary bleeding disorders: epidemiology, natural history, and management.

Science.gov (United States)

Papadopoulos, Nikolaos; Argiana, Vasiliki; Deutsch, Melanie

2018-01-01

Hereditary bleeding disorders include a group of diseases with abnormalities of coagulation. Prior to 1990, infection with hepatitis C virus (HCV) was mainly transmitted via pooled plasma products as a treatment for hereditary bleeding disorders. Anti-HCV positivity in these patients may be as high as >70% in some areas, while some of them have also been coinfected with human immunodeficiency virus. Since about 20% of HCV-infected patients clear the infection naturally, chronic HCV infection represents a significant health problem in this group of patients. Mortality due to chronic HCV infection is estimated to be >10 times higher in patients with hemophilia than in the general population, and is mainly due to liver cirrhosis and hepatocellular carcinoma. The antiviral treatment of HCV in patients with hereditary bleeding disorders is not different from that of any other infected patients. Nevertheless, many patients with hereditary bleeding disorders have declined (Peg)interferon-based treatment because of side effects. In recent years, multiple orally administrated direct-acting antivirals (DAAs) have been approved for HCV treatment. Unfortunately, there is not much experience from treating these patients with DAA regimens, as major studies and real-life data did not include adequate numbers of patients with inherited hemorrhagic disorders. However, the available data indicate that DAAs have an excellent safety profile with a sustained virological response rate of >90%.

Identification of Cis-Acting Promoter Elements in Cold- and Dehydration-Induced Transcriptional Pathways in Arabidopsis, Rice, and Soybean

Science.gov (United States)

Maruyama, Kyonoshin; Todaka, Daisuke; Mizoi, Junya; Yoshida, Takuya; Kidokoro, Satoshi; Matsukura, Satoko; Takasaki, Hironori; Sakurai, Tetsuya; Yamamoto, Yoshiharu Y.; Yoshiwara, Kyouko; Kojima, Mikiko; Sakakibara, Hitoshi; Shinozaki, Kazuo; Yamaguchi-Shinozaki, Kazuko

2012-01-01

The genomes of three plants, Arabidopsis (Arabidopsis thaliana), rice (Oryza sativa), and soybean (Glycine max), have been sequenced, and their many genes and promoters have been predicted. In Arabidopsis, cis-acting promoter elements involved in cold- and dehydration-responsive gene expression have been extensively analysed; however, the characteristics of such cis-acting promoter sequences in cold- and dehydration-inducible genes of rice and soybean remain to be clarified. In this study, we performed microarray analyses using the three species, and compared characteristics of identified cold- and dehydration-inducible genes. Transcription profiles of the cold- and dehydration-responsive genes were similar among these three species, showing representative upregulated (dehydrin/LEA) and downregulated (photosynthesis-related) genes. All (46 = 4096) hexamer sequences in the promoters of the three species were investigated, revealing the frequency of conserved sequences in cold- and dehydration-inducible promoters. A core sequence of the abscisic acid-responsive element (ABRE) was the most conserved in dehydration-inducible promoters of all three species, suggesting that transcriptional regulation for dehydration-inducible genes is similar among these three species, with the ABRE-dependent transcriptional pathway. In contrast, for cold-inducible promoters, the conserved hexamer sequences were diversified among these three species, suggesting the existence of diverse transcriptional regulatory pathways for cold-inducible genes among the species. PMID:22184637

Carbon-based building blocks for alcohol dehydration

DEFF Research Database (Denmark)

Boffa, Vittorio; Mallon, Peter E.; Magnacca, Giuliana

A biopolymer (HAL), extracted from organic compost with yield ~20%, was used to fabricate composite HAL-graphene oxide (GO) membranes. Upon thermal stabilization, HAL brings high disorder in the membrane structure, thus increasing water permeability. This feature together with the good water/etha....../ethanol perm-selectivity make GO-HAL membranes promising devices for alcohol dehydration....

Methods for dehydration of sugars and sugar alcohols

Science.gov (United States)

Holladay, Johnathan E [Kennewick, WA; Hu, Jianli [Kennewick, WA; Zhang, Xinjie [Burlington, MA; Wang, Yong [Richland, WA

2010-08-10

The invention includes a method of dehydration of a sugar using a dehydration catalyst and a co-catalyst within a reactor. A sugar is introduced and H.sub.2 is flowed through the reactor at a pressure of less than or equal to about 300 psig to convert at least some of the sugar into an anhydrosugar product. The invention includes a process for producing isosorbide. A starting material comprising sorbitol is flowed into a reactor. H.sub.2 is counter flowed through the reactor. The starting material is exposed to a catalyst in the presence of a co-catalyst which comprises at least one metal. The exposing is conducted at a hydrogen pressure of less than or equal to 300 psig within the reactor and the hydrogen removes at least some of any water present during the exposing and inhibits formation of colored byproducts.

Sporadic diffuse segmental interstitial cell of Cajal hyperplasia harbouring two gastric gastrointestinal stromal tumours (GIST mimicking hereditary GIST syndromes

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Mafalda Costa Neves

2015-01-01

Conclusion: We describe a diffuse form of sporadic ICC hyperplasia harbouring multifocal GISTs, mimicking diffuse ICC hyperplasia in hereditary GIST syndromes. Detection of somatic c-KIT exon 11 mutation ruled out a hereditary disorder.

Severe fatigue and reduced quality of life in children with hereditary motor and sensory neuropathy 1A

NARCIS (Netherlands)

Jagersma, Elbrich; Jeukens-Visser, Martine; van Paassen, Barbara W.; Meester-Delver, Anke; Nollet, Frans

2013-01-01

Severe fatigue and low quality of life are reported by a majority of adult patients with hereditary motor and sensory neuropathy 1A. In children with hereditary motor and sensory neuropathy 1A, the prevalence and impact of fatigue have not been studied yet. In this questionnaire survey, 55 Dutch

A RhABF2/Ferritin module affects rose (Rosa hybrida) petal dehydration tolerance and senescence by modulating iron levels.

Science.gov (United States)

Liu, Jitao; Fan, Youwei; Zou, Jing; Fang, Yiqun; Wang, Linghao; Wang, Meng; Jiang, Xinqiang; Liu, Yiqing; Gao, Junping; Zhang, Changqing

2017-12-01

Plants often develop the capacity to tolerate moderate and reversible environmental stresses, such as drought, and to re-establish normal development once the stress has been removed. An example of this phenomenon is provided by cut rose (Rosa hybrida) flowers, which experience typical reversible dehydration stresses during post-harvest handling after harvesting at the bud stages. The molecular mechanisms involved in rose flower dehydration tolerance are not known, however. Here, we characterized a dehydration- and abscisic acid (ABA)-induced ferritin gene (RhFer1). Dehydration-induced free ferrous iron (Fe 2+ ) is preferentially sequestered by RhFer1 and not transported outside of the petal cells, to restrict oxidative stresses during dehydration. Free Fe 2+ accumulation resulted in more serious oxidative stresses and the induction of genes encoding antioxidant enzyme in RhFer1-silenced petals, and poorer dehydration tolerance was observed compared with tobacco rattle virus (TRV) controls. We also determined that RhABF2, an AREB/ABF transcription factor involved in the ABA signaling pathway, can activate RhFer1 expression by directly binding to its promoter. The silencing of RhABF2 decreased dehydration tolerance and disrupted Fe homeostasis in rose petals during dehydration, as did the silencing of RhFer1. Although both RhFer1 and Fe transporter genes are induced during flower natural senescence in plants, the silencing of RhABF2 or RhFer1 accelerates the petal senescence processes. These results suggest that the regulatory module RhABF2/RhFer1 contributes to the maintenance of Fe levels and enhances dehydration tolerance through the action of RhFer1 locally sequestering free Fe 2+ under dehydration conditions, and plays synergistic roles with transporter genes during flower senescence. © 2017 The Authors The Plant Journal © 2017 John Wiley & Sons Ltd.

Comparing the Accuracy of the Three Dehydration Scales in Children with Acute Diarrhea in a Developing Country of Kosovo.

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Hoxha, Teuta; Xhelili, Luan; Azemi, Mehmedali; Avdiu, Muharrem; Ismaili-Jaha, Vlora; Efendija-Beqa, Urata; Grajcevci-Uka, Violeta

2015-06-01

Although diarrhea is a preventable disease, it remains the second leading cause of death (after pneumonia) among children aged under five years worldwide. The World Health Organization (WHO) scale, the Gorelick scale, and the Clinical Dehydration Scale (CDS) were created to estimate dehydration status using clinical signs. The purpose of this study is to determine whether these clinical scales can accurately assess dehydration status of children in a developing country of Kosovo. Children aged 1 month to 5 years with a history of acute diarrhea were enrolled in the study. After recording the data about the patients historical features the treating physician recorded the physical examination findings consistent with each clinical score. Receiver operating characteristic (ROC) curves were constructed to evaluate the performance of the three scales, compared to the gold standard, percent weight change with rehydration. Sensitivity, specificity and likelihood ratios were calculated using the best cut-off points of the ROC curves. We enrolled 230 children, and 200 children met eligibility criteria. The WHO scale for predicting significant dehydration (≥5 percent weight change) had an area under the curve (AUC) of 0.71 (95% : CI= 0.65-0.77). The Gorelick scales 4- and 10-point for predicting significant dehydration, had an area under the curve of 0.71 (95% : CI=0.63- 0.78) and 0.74 (95% : CI= 0.68-0.81) respectively. Only the CDS for predicting the significant dehydration above ≥6% percent weight change, did not have an area under the curve statistically different from the reference line with an AUC of 0.54 (95% CI = 0.45- 0.63). The WHO dehydration scale and Gorelick scales were fair predictors of dehydration in children with diarrhea. Only the Clinical Dehydration Scale was found not to be a helpful predictor of dehydration in our study cohort.

Comparing the Accuracy of the Three Dehydration Scales in Children with Acute Diarrhea in a Developing Country of Kosovo

Science.gov (United States)

Hoxha, Teuta; Xhelili, Luan; Azemi, Mehmedali; Avdiu, Muharrem; Ismaili-Jaha, Vlora; Efendija-Beqa, Urata; Grajcevci-Uka, Violeta

2015-01-01

Background. Although diarrhea is a preventable disease, it remains the second leading cause of death (after pneumonia) among children aged under five years worldwide. The World Health Organization (WHO) scale, the Gorelick scale, and the Clinical Dehydration Scale (CDS) were created to estimate dehydration status using clinical signs. The purpose of this study is to determine whether these clinical scales can accurately assess dehydration status of children in a developing country of Kosovo. Methodology. Children aged 1 month to 5 years with a history of acute diarrhea were enrolled in the study. After recording the data about the patients historical features the treating physician recorded the physical examination findings consistent with each clinical score. Receiver operating characteristic (ROC) curves were constructed to evaluate the performance of the three scales, compared to the gold standard, percent weight change with rehydration. Sensitivity, specificity and likelihood ratios were calculated using the best cut-off points of the ROC curves. Results. We enrolled 230 children, and 200 children met eligibility criteria. The WHO scale for predicting significant dehydration (≥5 percent weight change) had an area under the curve (AUC) of 0.71 (95% : CI= 0.65-0.77). The Gorelick scales 4- and 10-point for predicting significant dehydration, had an area under the curve of 0.71 (95% : CI=0.63- 0.78) and 0.74 (95% : CI= 0.68-0.81) respectively. Only the CDS for predicting the significant dehydration above ≥6% percent weight change, did not have an area under the curve statistically different from the reference line with an AUC of 0.54 (95% CI = 0.45- 0.63). Conclusion. The WHO dehydration scale and Gorelick scales were fair predictors of dehydration in children with diarrhea. Only the Clinical Dehydration Scale was found not to be a helpful predictor of dehydration in our study cohort. PMID:26244042

Hereditary chronic pancreatitis in a patient with type 1 diabetes mellitus

Directory of Open Access Journals (Sweden)

Varalakshmi Muthukrishnan

2018-01-01

Full Text Available In this case report, we present a young patient with type 1 diabetes who had hereditary chronic pancreatitis. We evaluated him for the cause of pancreatitis, but it was inconclusive and finally the genetic testing was done for him, which revealed heterozygous missense mutation in exon 3 of the PRSS1 gene (protease serine 1 gene on chromosome 7. Hence, we were able to make the diagnosis of hereditary chronic pancreatitis. Chronic pancreatitis secondary to any cause can lead to permanent diabetes, which is typically difficult to control. However, in this case, the episodes of recurrent pancreatitis were present after the onset of type 1 diabetes as compared to the usual presentation of diabetes after the advancement of chronic pancreatitis.

Recurrent IVF failure and hereditary thrombophilia.

Science.gov (United States)

Safdarian, Leila; Najmi, Zahra; Aleyasin, Ashraf; Aghahosseini, Marzieh; Rashidi, Mandana; Asadollah, Sara

2014-07-01

The largest percentage of failed invitro fertilization (IVF (cycles, are due to lack of implantation. As hereditary thrombophilia can cause in placentation failure, it may have a role in recurrent IVF failure. Aim of this case-control study was to determine whether hereditary thrombophilia is more prevalent in women with recurrent IVF failures. Case group comprised 96 infertile women, with a history of recurrent IVF failure. Control group was comprised of 95 healthy women with proven fertility who had conceived spontaneously. All participants were assessed for the presence of inherited thrombophilias including: factor V Leiden, methilen tetrahydrofolate reductase (MTHFR) mutation, prothrombin mutation, homocystein level, protein S and C deficiency, antithrombin III (AT-III) deficiency and plasminogen activator inhibitor-1 (PAI-1) mutation. Presence of thrombophilia was compared between groups. Having at least one thrombophilia known as a risk factor for recurrent IVF failure (95% CI=1.74-5.70, OR=3.15, p=0.00). Mutation of factor V Leiden (95% CI=1.26-10.27, OR=3.06, P=0.01) and homozygote form of MTHFR mutation (95% CI=1.55-97.86, OR=12.33, p=0.05) were also risk factors for recurrent IVF failure. However, we could not find significant difference in other inherited thrombophilia's. Inherited thrombophilia is more prevalent in women with recurrent IVF failure compared with healthy women. Having at least one thrombophilia, mutation of factor V Leiden and homozygote form of MTHFR mutation were risk factors for recurrent IVF failure.

Sex differences and plasticity in dehydration tolerance: insight from a tropical liverwort

OpenAIRE

Marks, Rose A.; Burton, James F.; McLetchie, D. Nicholas

2016-01-01

Background and Aims Adaptations allowing plants to cope with drying are particularly relevant in the light of predicted climate change. Dehydration tolerance (DhT, also dehydration-tolerant) is one such adaptation enabling tissue to survive substantial drying. A great deal of work has been conducted on highly DhT species. However, bryophytes showing less intense and variable DhT are understudied, despite the potential for these species to provide an informative link between highly tolerant an...

Osmotic dehydration of fruit and berry raw materials in the food industry

OpenAIRE

N. A. Gribova; L. G. Eliseeva

2017-01-01

Osmotic dehydration has recently received more attention as an effective method of preserving fruits and berries. Osmosis is a simple process that facilitates the processing of fruits and berries in order to preserve the original characteristics, namely nutritional value and organoleptic properties: color, aroma and texture. Osmotic dehydration has found wide application in the preservation of food products, as the activity of water in fruits and berries decreases, in some of them up to 90% o...

Mass transfer kinetics during osmotic dehydration of pomegranate arils.

Science.gov (United States)

Mundada, Manoj; Hathan, Bahadur Singh; Maske, Swati

2011-01-01

The mass transfer kinetics during osmotic dehydration of pomegranate arils in osmotic solution of sucrose was studied to increase palatability and shelf life of arils. The freezing of the whole pomegranate at -18 °C was carried out prior to osmotic dehydration to increase the permeability of the outer cellular layer of the arils. The osmotic solution concentrations used were 40, 50, 60°Bx, osmotic solution temperatures were 35, 45, 55 °C. The fruit to solution ratio was kept 1:4 (w/w) during all the experiments and the process duration varied from 0 to 240 min. Azuara model and Peleg model were the best fitted as compared to other models for water loss and solute gain of pomegranate arils, respectively. Generalized Exponential Model had an excellent fit for water loss ratio and solute gain ratio of pomegranate arils. Effective moisture diffusivity of water as well as solute was estimated using the analytical solution of Fick's law of diffusion. For above conditions of osmotic dehydration, average effective diffusivity of water loss and solute gain varied from 2.718 × 10(-10) to 5.124 × 10(-10) m(2)/s and 1.471 × 10(-10) to 5.147 × 10(-10) m(2)/s, respectively. The final product was successfully utilized in some nutritional formulations such as ice cream and bakery products.

Mania associated with complicated hereditary spastic paraparesis

OpenAIRE

Raghavendra B Nayak; Govind S Bhogale; Nanasaheb M Patil; Aditya A Pandurangi

2011-01-01

Hereditary spastic paraparesis (HSP) is an inherited group of neurological disorders with progressive lower limb spasticity. HSP can be clinically grouped into pure and complicated forms. Pure HSP is one without any associated neurological/psychiatric comorbidity. Depression is the most common psychiatric comorbidity. Presence of mania or bipolar affective illness with HSP is a rare phenomenon. We report a case of a 17-year-old boy who presented with classical features of HSP with complaints ...

The effects of dehydration, moderate alcohol consumption, and rehydration on cognitive functions.

Science.gov (United States)

Irwin, Christopher; Leveritt, Michael; Shum, David; Desbrow, Ben

2013-05-01

This study investigated the impact of mild-moderate dehydration on alcohol-induced deteriorations in cognitive functions. Sixteen healthy males participated in a single-blind, placebo-controlled cross-over design study involving 4 experimental trials (separated by ≥7 d). In each trial, participants were dehydrated by 2.5% body mass through exercise. After 1 h recovery in a thermo-neutral environment (22 ± 2 °C, 60-70% relative humidity) 4 tasks from the Cambridge Neuropsychological Test Automated Battery (CANTAB) were administered to the participants (test 1). In two of the trials, participants were provided with water equivalent to either 50% or 150% body mass loss and given salt (NaCl) capsules (50 mmol/L). A set volume of alcohol or placebo was then consumed in each trial, incorporating the conditions: dehydration-placebo (DP), dehydration-alcohol (DA), partial rehydration-alcohol (PA), and full rehydration-alcohol (FA). The same 4 CANTAB tasks were then re-administered (test 2). Subjective ratings of mood and estimates of alcohol intoxication and driving impairment were also recorded in each trial. Alcohol consumption caused deterioration on 3 of the 4 CANTAB measures (viz., choice reaction time, executive function and response inhibition). This reduction in performance was exacerbated when participants were dehydrated compared to trials where full rehydration occurred. Subjective ratings of impairment and intoxication were not significantly different between any of the trials where alcohol was consumed; however ratings for alcohol trials were significantly higher than in the placebo trial. These findings suggest that rehydration after exercise that causes fluid loss can attenuate alcohol-related deterioration of cognitive functions. This may pose implications for post match fluid replacement if a moderate amount of alcohol is also consumed. Copyright © 2013 Elsevier Inc. All rights reserved.

Friedreich's ataxia mimicking hereditary motor and sensory neuropathy.

Science.gov (United States)

Panas, Marios; Kalfakis, Nikolaos; Karadima, Georgia; Davaki, Panagiota; Vassilopoulos, Demetris

2002-11-01

Four patients from three unrelated families, with clinical and electrophysiological findings compatible with the diagnosis of hereditary motor and sensory neuropathy, are presented. The molecular analysis showed that the affected individuals were homozygous for the mutation in the X25 gene, characteristic of Friedreich's ataxia. These patients seem to represent a form of Friedreich's ataxia mimicking Charcot-Marie-Tooth disease.

Hereditary spastic paraplegia.

Science.gov (United States)

Blackstone, Craig

2018-01-01

The hereditary spastic paraplegias (HSPs) are a heterogeneous group of neurologic disorders with the common feature of prominent lower-extremity spasticity, resulting from a length-dependent axonopathy of corticospinal upper motor neurons. The HSPs exist not only in "pure" forms but also in "complex" forms that are associated with additional neurologic and extraneurologic features. The HSPs are among the most genetically diverse neurologic disorders, with well over 70 distinct genetic loci, for which about 60 mutated genes have already been identified. Numerous studies elucidating the molecular pathogenesis underlying HSPs have highlighted the importance of basic cellular functions - especially membrane trafficking, mitochondrial function, organelle shaping and biogenesis, axon transport, and lipid/cholesterol metabolism - in axon development and maintenance. An encouragingly small number of converging cellular pathogenic themes have been identified for the most common HSPs, and some of these pathways present compelling targets for future therapies. Copyright © 2018 Elsevier B.V. All rights reserved.

Evaluation of neutrophil gelatinase-associated lipocalin in pediatric patients with acute rotavirus gastroenteritis and dehydration.

Science.gov (United States)

Çelik, Tanju; Altekin, Emel; İşgüder, Rana; Kenesari, Yasin; Duman, Murat; Arslan, Nur

2013-09-03

Dehydration caused by acute rotavirus gastroenteritis is a frequent finding in pediatric patients. The most important treatment modality in these patients is recognising and treating dehydration, electrolyte imbalance and acute kidney injury. Neutrophil gelatinase-associated lipocalin (NGAL) is used widely as a biomarker for the diagnosis of acute or chronic renal injury in numerous clinical studies. It is recognized as an early marker of acute renal failure before the elevation of routine biochemical tests such as creatinine. The aim of this study is to investigate the plasma and urine NGAL concentrations in mildly or moderately dehydrated patients with acute rotavirus gastroenteritis. A total of 30 patients (13 girls, mean age 62.5 ± 46.2 months) with diarrhea and mild/moderate dehydration and 35 healthy controls (17 girls, mean age 81.1 ± 41.8 months) were enrolled in the study. Plasma and urine NGAL levels of the two groups were compared. The mean age, gender and serum creatinine levels of the patients and healthy controls were similar. The mean plasma and urine NGAL levels of the patients were significantly higher than controls (plasma: 118.6 ± 81.2 vs. 66.5 ± 11.3, p = 0.001 and urine: 17.7 ± 17.5 vs. 10.6 ± 7.9, p = 0.035, respectively). Mildly or moderately dehydrated children have higher plasma and urine NGAL levels compared to control subjects. Plasma and/or urine NGAL levels can be used for the early prediction of renal impairment in children with mild or moderate dehydration.

Mechanistic basis of an epistatic interaction reducing age at onset in hereditary spastic paraplegia.

Science.gov (United States)

Newton, Timothy; Allison, Rachel; Edgar, James R; Lumb, Jennifer H; Rodger, Catherine E; Manna, Paul T; Rizo, Tania; Kohl, Zacharias; Nygren, Anders O H; Arning, Larissa; Schüle, Rebecca; Depienne, Christel; Goldberg, Lisa; Frahm, Christiane; Stevanin, Giovanni; Durr, Alexandra; Schöls, Ludger; Winner, Beate; Beetz, Christian; Reid, Evan

2018-05-01

Many genetic neurological disorders exhibit variable expression within affected families, often exemplified by variations in disease age at onset. Epistatic effects (i.e. effects of modifier genes on the disease gene) may underlie this variation, but the mechanistic basis for such epistatic interactions is rarely understood. Here we report a novel epistatic interaction between SPAST and the contiguous gene DPY30, which modifies age at onset in hereditary spastic paraplegia, a genetic axonopathy. We found that patients with hereditary spastic paraplegia caused by genomic deletions of SPAST that extended into DPY30 had a significantly younger age at onset. We show that, like spastin, the protein encoded by SPAST, the DPY30 protein controls endosomal tubule fission, traffic of mannose 6-phosphate receptors from endosomes to the Golgi, and lysosomal ultrastructural morphology. We propose that additive effects on this pathway explain the reduced age at onset of hereditary spastic paraplegia in patients who are haploinsufficient for both genes.

Brain abscesses and hereditary hemorrhagic telangiectasia

International Nuclear Information System (INIS)

Vives, Daniel A.; Bauni, Carlos E.; Mendoza, Monica E.

2003-01-01

Rendu-Osler-Weber disease or Hereditary Hemorrhagic Telangiectasia (HHT) is a generalized familial angiodysplastic disorder. The neurological manifestations of this entity are due to Central Nervous System vascular lesions or to complications of other visceral lesions such as pulmonary arteriovenous fistulae. This report describes two patients (males, 40 and 61 years old), with brain abscesses associated with HHT. The CT, MRI and Angiographic findings as well as the therapeutic approach are analyzed. Patients with brain abscess of unknown origin must be evaluated for the presence of lung vascular malformation in association with HHT. (author)

Bone scintigraphy in hereditary multiple exostoses

International Nuclear Information System (INIS)

Epstein, D.A.; Levin, E.J.

1978-01-01

Two adult patients with multiple hereditary exostoses, a skeletal disorder with recognized malignant potential, each demonstrated increased /sup 99m/Tc diphosphonate uptake in an exostosis in which renewed growth had begun. None of the other multiple exostoses in either patient showed abnormal uptake. Histologic study of the lesions demonstrated chondrosarcoma in one case and benign osteochondroma in the second. Although bone scintigraphy nonspecifically identifies bone growth rather than malignant degeneration, it is more useful than radiographic bone survey in the periodic surveillance of adult patients with this disorder

Evolution of microstructure and elastic wave velocities in dehydrated gypsum samples

Science.gov (United States)

Milsch, Harald; Priegnitz, Mike

2012-12-01

We report on changes in P and S-wave velocities and rock microstructure induced by devolatilization reactions using gypsum as a reference analog material. Cylindrical samples of natural alabaster were dehydrated in air, at ambient pressure, and temperatures between 378 and 423 K. Dehydration did not proceed homogeneously but via a reaction front moving sample inwards separating an outer highly porous rim from the remaining gypsum which, above approximately 393 (±5) K, concurrently decomposed into hemihydrate. Overall porosity was observed to continuously increase with reaction progress from approximately 2% for fully hydrated samples to 30% for completely dehydrated ones. Concurrently, P and S-wave velocities linearly decreased with porosity from 5.2 and 2.7 km/s to 1.0 and 0.7 km/s, respectively. It is concluded that a linearized empirical Raymer-type model extended by a critical porosity term and based on the respective time dependent mineral and pore volumes reasonably replicates the P and S-wave data in relation to reaction progress and porosity.

The neuropathology of hereditary congenital facial palsy vs Mobius syndrome.

NARCIS (Netherlands)

Verzijl, H.T.F.M.; Zwaag, B. van der; Lammens, M.M.Y.; Donkelaar, H.J. ten; Padberg, G.W.A.M.

2005-01-01

OBJECTIVE: To characterize the neuropathology of hereditary congenital facial palsy. METHODS: The authors compared brainstem pathology of three members of one family with autosomal dominant congenital facial palsy to that in three age-matched controls. The neuropathologic findings of the familial

Theoretical investigation of the selective dehydration and dehydrogenation of ethanol catalyzed by small molecules.

Science.gov (United States)

Wang, Yanqun; Tang, Yizhen; Shao, Youxiang

2017-09-01

Catalytic dehydration and dehydrogenation reactions of ethanol have been investigated systematically using the ab initio quantum chemistry methods The catalysts include water, hydrogen peroxide, formic acid, phosphoric acid, hydrogen fluoride, ammonia, and ethanol itself. Moreover, a few clusters of water and ethanol were considered to simulate the catalytic mechanisms in supercritical water and supercritical ethanol. The barriers for both dehydration and dehydrogenation can be reduced significantly in the presence of the catalysts. It is revealed that the selectivity of the catalytic dehydration and dehydrogenation depends on the acidity and basicity of the catalysts and the sizes of the clusters. The acidic catalyst prefers dehydration while the basic catalysts tend to promote dehydrogenation more effectively. The calculated water-dimer catalysis mechanism supports the experimental results of the selective oxidation of ethanol in the supercritical water. It is suggested that the solvent- and catalyst-free self-oxidation of the supercritical ethanol could be an important mechanism for the selective dehydrogenation of ethanol on the theoretical point of view. Copyright © 2017 Elsevier Inc. All rights reserved.

Intragenic duplication: a novel mutational mechanism in hereditary pancreatitis

DEFF Research Database (Denmark)

Joergensen, Maiken T; Geisz, Andrea; Brusgaard, Klaus

2011-01-01

In a hereditary pancreatitis family from Denmark, we identified a novel intragenic duplication of 9 nucleotides in exon-2 of the human cationic trypsinogen (PRSS1) gene (c.63_71dup) which at the amino-acid level resulted in the insertion of 3 amino acids within the activation peptide of cationic...

Severe Hypernatremic Dehydration and Lower Limb Gangrene in an Infant Exposed to Lamotrigine, Aripiprazole, and Sertraline in Breast Milk.

Science.gov (United States)

Morin, Caroline; Chevalier, Isabelle

Hypernatremic dehydration is well described in exclusively breastfed neonates, although life-threatening complications are rarely reported. The present article describes a case of severe hypernatremic dehydration in a previously healthy term neonate. Other published cases of severe complications of hypernatremic dehydration are discussed. The exclusively breastfed neonate described had severe hypernatremic dehydration because of inadequate milk intake, with disseminated intravascular coagulation and right lower limb gangrene that required amputation of all five toes and surgical debridement of the metatarsals. The usual etiology of hypernatremic dehydration in this age group is insufficient breast milk intake. Here, the infant's mother was treated for bipolar disorder with lamotrigine 250 mg orally once daily, aripiprazole 15 mg orally once daily, and sertraline 100 mg orally once daily. Awareness of these complications should prompt close follow-up of the infant with poor weight gain. The role of maternal medication as a risk factor for hypernatremic dehydration among exclusively breastfed infants needs to be further explored.

Efficiency of Osmotic Dehydration of Apples in Polyols Solutions.

Science.gov (United States)

Cichowska, Joanna; Żubernik, Joanna; Czyżewski, Jakub; Kowalska, Hanna; Witrowa-Rajchert, Dorota

2018-02-17

The present study aimed to evaluate the influence of selected compounds from the polyol group, as well as other saccharides, on the osmotic dehydration process of apples. The following alternative solutions were examined: erythritol, xylitol, maltitol, inulin and oligofructose. Efficiency of the osmotic dehydration process was evaluated based on the kinetics of the process, and through comparison of the results obtained during the application of a sucrose solution. This innovative research utilizes alternative solutions in osmotic pretreatment, which until now, have not been commonly used in fruit processing by researchers worldwide. Results indicate that erythritol and xylitol show stronger or similar efficiency to sucrose; however, the use of inulin, as well as oligofructose, was not satisfactory due to the insufficient, small osmotic driving forces of the process, and the low values of mass transfer parameters.

Comparison of three dehydration scales showed that they were of limited or no value for assessing small children with acute diarrhoea.

Science.gov (United States)

Pomorska, Dominika; Dziechciarz, Piotr; Mduma, Esto; Gidion, Joshua; Falszewska, Anna; Szajewska, Hania

2018-02-20

We explored the diagnostic accuracy of the clinical dehydration scale (CDS), the World Health Organization (WHO) scale and the Gorelick scale for assessing dehydration in children admitted to a Tanzanian referral hospital. This was a prospective, observational study, carried out from April 2015 to January 2017 on children aged one month to five years admitted to the hospital with acute diarrhoea lasting less than five days. Before rehydration therapy, each patient's weight was recorded and the degree of dehydration was assessed based on the three scales. The reference standard was the percentage weight change between admission and discharge. The main outcomes were the sensitivity, specificity and positive and negative likelihood ratios (LRs) of the scales. Data from 124 eligible patients were available. The CDS showed limited value for ruling in cases with some dehydration (LR 1.9, 95% confidence interval 1.1-2.8), but was of no value in assessing no and moderate to severe dehydration. The WHO and Gorelick scales were of no value in evaluating any degree of dehydration. The WHO and Gorelick dehydration scales were no use for assessing dehydration in small children, and the CDS was of limited use for predicting cases with some dehydration. ©2018 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.

Android Mobile Informatics Application for some Hereditary Diseases and Disorders (AMAHD: A complementary framework for medical practitioners and patients

Directory of Open Access Journals (Sweden)

Olugbenga Oluwagbemi

Full Text Available Hereditary diseases and disorders constitute a public health problem. Many people in rural communities of developing countries of the world are particularly ignorant about the cause, modes of transmissions and the treatment plans for such diseases. In some cases, some people lack essential knowledge between common and rare hereditary diseases.It is therefore appropriate and essential to develop a mobile application that will act as an educative resource and a good knowledge base for common and rare hereditary diseases.The aim of this research is to develop AMAHD (Android Mobile Informatics Application for some Hereditary Diseases and Disorders.The objectives of this research are to create an android mobile application that will act as a reference point and provide useful information about various hereditary diseases to medical personnel and professionals; provide additional educational resource to biological and bioinformatics researchers in different higher institutions; and provide a pedagogical, diagnostic and complementary foundational learning tool for African research students in biosciences, bioinformatics, and all other categories of students that currently engage in multidisciplinary research in the aspect of hereditary diseases.Essential data was sourced from relevant literature. We developed AMAHD through an integration of programming languages in Java and XML (Extended Markup Language. SQLite was used to implement the database. We developed a Logical Disjunction Rule-based Algorithm (LDRA for the AMAHD’s diagnosis module.A comparative analysis between existing commercial hereditary mobile applications and AMAHD was conducted and the results presented. A world-wide online survey (spanning Africa, Asia, Europe, America and Australia was conducted to sample the opinion of individuals across the globe on the classification of hereditary diseases as either rare or common, within their respective regions. In addition, an evaluation of

Dehydration and performance on clinical concussion measures in collegiate wrestlers.

Science.gov (United States)

Weber, Amanda Friedline; Mihalik, Jason P; Register-Mihalik, Johna K; Mays, Sally; Prentice, William E; Guskiewicz, Kevin M

2013-01-01

The effects of dehydration induced by wrestling-related weight-cutting tactics on clinical concussion outcomes, such as neurocognitive function, balance performance, and symptoms, have not been adequately studied. To evaluate the effects of dehydration on the outcome of clinical concussion measures in National Collegiate Athletic Association Division I collegiate wrestlers. Repeated-measures design. Clinical research laboratory. Thirty-two Division I healthy collegiate male wrestlers (age = 20.0 ± 1.4 years; height = 175.0 ± 7.5 cm; baseline mass = 79.2 ± 12.6 kg). Participants completed preseason concussion baseline testing in early September. Weight and urine samples were also collected at this time. All participants reported to prewrestling practice and postwrestling practice for the same test battery and protocol in mid-October. They had begun practicing weight-cutting tactics a day before prepractice and postpractice testing. Differences between these measures permitted us to evaluate how dehydration and weight-cutting tactics affected concussion measures. Sport Concussion Assessment Tool 2 (SCAT2), Balance Error Scoring System, Graded Symptom Checklist, and Simple Reaction Time scores. The Simple Reaction Time was measured using the Automated Neuropsychological Assessment Metrics. The SCAT2 measurements were lower at prepractice (P = .002) and postpractice (P < .001) when compared with baseline. The BESS error scores were higher at postpractice when compared with baseline (P = .015). The GSC severity scores were higher at prepractice (P = .011) and postpractice (P < .001) than at baseline and at postpractice when than at prepractice (P = .003). The number of Graded Symptom Checklist symptoms reported was also higher at prepractice (P = .036) and postpractice (P < .001) when compared with baseline, and at postpractice when compared with prepractice (P = .003). Our results suggest that it is important for wrestlers to be evaluated in a euhydrated state to

Dehydration of isobutanol and the elimination of water from fuel alcohols.

Science.gov (United States)

Rosado-Reyes, Claudette M; Tsang, Wing; Alecu, Ionut M; Merchant, Shamel S; Green, William H

2013-08-08

Rate coefficients for the dehydration of isobutanol have been determined experimentally from comparative rate single pulse shock tube measurements and calculated via multistructural transition state theory (MS-TST). They are represented by the Arrhenius expression, k(isobutanol → isobutene + H2O)(experimental) = 7.2 × 10(13) exp(-35300 K/T) s(-1). The theoretical work leads to the high pressure rate expression, k(isobutanol → isobutene + H2O)(theory) = 3.5 × 10(13) exp(-35400 K/T) s(-1). Results are thus within a factor of 2 of each other. The experimental results cover the temperature range 1090-1240 K and pressure range 1.5-6 atm, with no discernible pressure effects. Analysis of these results, in combination with earlier single pulse shock tube work, made it possible to derive the governing factors that control the rate coefficients for alcohol dehydration in general. Alcohol dehydration rate constants depend on the location of the hydroxyl group (primary, secondary, and tertiary) and the number of available H-atoms adjacent to the OH group for water elimination. The position of the H-atoms in the hydrocarbon backbone appears to be unimportant except for highly substituted molecules. From these correlations, we have derived k(isopropanol → propene + H2O) = 7.2 × 10(13) exp(-33000 K/T) s(-1). Comparison of experimental determination with theoretical calculations for this dehydration, and those for ethanol show deviations of the same magnitude as for isobutanol. Systematic differences between experiments and theoretical calculations are common.

Coinheritance of hereditary spherocytosis and reversibility of cirrhosis in a young female patient with hereditary hemochromatosis.

Science.gov (United States)

Höblinger, A; Erdmann, C; Strassburg, C P; Sauerbruch, T; Lammert, F

2009-04-16

Here we report a 33-years-old woman with hereditary spherocytosis and hemochromatosis due to homozygosity for the C282Y mutation of the HFE gene. The coinheritance of both conditions led to severe iron overload and liver cirrhosis at young age. The patient was treated by repeated phlebotomy, and reversibility of cirrhosis was documented by transient elastography. This report discusses the pathophysiology of iron accumulation in patients with hemolytic anemia combined with HFE C282Y homozygosity. The case indicates that patients with hematological disorders characterized by increased erythropoetic activity should be screened for HFE mutations.

Stroke from systemic vascular disorders in Saudi children: The devastating role of hypernatremic dehydration

International Nuclear Information System (INIS)

Salih, Mustafa A.; Al-Jarallah, Ahmed A.; Zahraa, Jihad N.; Alorainy, Ibrahim A.; Hassan, Hamdy H.

2006-01-01

Systemic vascular disorders, leading to childhood stroke, include volume depletion or systemic hypotension and hypernatremic dehydration. We describe 3 cases of stroke following systemic vascular disorders. These were diagnosed during a prospective and retrospective study on childhood stroke, which included 104 patients. Post-gastroenteritis hypernatremic dehydration is an important, potentially preventable, cause of stroke in Saudi children. (author)

The mechanism of the dehydration reaction of solid HIO 4. 2H 2O and the role of radiation

International Nuclear Information System (INIS)

Takriti, S.

1998-01-01

A thermal and kinetic study of dehydration reaction of periodic acid dehydrate has been carried out with isothermal and physical measurements. Isothermal dehydration curves have been studied in the temperature ranges between 333 0 K respectively. Both dehydration processes involve the loss two molecules of water in the studied temperature ranges. Thermodynamic and kinetic parameters have been calculated using different physical models proposed. The data analysis illustrated that activation energy is about 1 eV for the first step and 1.2 eV for second

Empirically Derived Dehydration Scoring and Decision Tree Models for Children With Diarrhea: Assessment and Internal Validation in a Prospective Cohort Study in Dhaka, Bangladesh.

Science.gov (United States)

Levine, Adam C; Glavis-Bloom, Justin; Modi, Payal; Nasrin, Sabiha; Rege, Soham; Chu, Chieh; Schmid, Christopher H; Alam, Nur H

2015-08-18

Diarrhea remains one of the most common and most deadly conditions affecting children worldwide. Accurately assessing dehydration status is critical to determining treatment course, yet no clinical diagnostic models for dehydration have been empirically derived and validated for use in resource-limited settings. In the Dehydration: Assessing Kids Accurately (DHAKA) prospective cohort study, a random sample of children under 5 with acute diarrhea was enrolled between February and June 2014 in Bangladesh. Local nurses assessed children for clinical signs of dehydration on arrival, and then serial weights were obtained as subjects were rehydrated. For each child, the percent weight change with rehydration was used to classify subjects with severe dehydration (>9% weight change), some dehydration (3-9%), or no dehydration (Dehydration Score and DHAKA Dehydration Tree, respectively. Models were assessed for their accuracy using the area under their receiver operating characteristic curve (AUC) and for their reliability through repeat clinical exams. Bootstrapping was used to internally validate the models. A total of 850 children were enrolled, with 771 included in the final analysis. Of the 771 children included in the analysis, 11% were classified with severe dehydration, 45% with some dehydration, and 44% with no dehydration. Both the DHAKA Dehydration Score and DHAKA Dehydration Tree had significant AUCs of 0.79 (95% CI = 0.74, 0.84) and 0.76 (95% CI = 0.71, 0.80), respectively, for the diagnosis of severe dehydration. Additionally, the DHAKA Dehydration Score and DHAKA Dehydration Tree had significant positive likelihood ratios of 2.0 (95% CI = 1.8, 2.3) and 2.5 (95% CI = 2.1, 2.8), respectively, and significant negative likelihood ratios of 0.23 (95% CI = 0.13, 0.40) and 0.28 (95% CI = 0.18, 0.44), respectively, for the diagnosis of severe dehydration. Both models demonstrated 90% agreement between independent raters and good

Influences of dehydration on clinical features of radiological pneumonia in children attending an urban diarrhoea treatment centre in Bangladesh.

Science.gov (United States)

Chisti, M J; Salam, M A; Bardhan, P K; Ahad, R; La Vincente, S; Duke, T

2010-01-01

As the signs of dehydration often overlap with those of pneumonia, it may be difficult for health workers in resource-poor settings to make a clinical diagnosis of pneumonia in children with dehydration. This issue has received very little attention. To compare the clinical features of pneumonia in children with and without dehydration caused by diarrhoea. All children aged 2-59 months with diarrhoea and radiologically confirmed pneumonia admitted to the Special Care Ward (SCW) of Dhaka Hospital, ICDDR,B between September and December 2007 were enrolled for the study. Children with dehydration (67 cases) and those without (101 controls) were compared. Cases presented less frequently with fast breathing (60% vs 88%, pchildren with dehydration, especially when there is severe malnutrition.

Methods to increase the rate of mass transfer during osmotic dehydration of foods

OpenAIRE

Anna Chwastek

2014-01-01

Traditional methods of food preservation such as freezing, freeze drying (lyophilization), vacuum drying, convection drying are often supplemented by new technologies that enable obtaining of high quality products. Osmotic dehydration is more and more often used during processing of fruits and vegetables. This method allows maintaining good organoleptic and functional properties in the finished product. Obtaining the desired degree of dehydration or saturation of the material with an osmoacti...

The Usefulness of Clinical and Laboratory Parameters for Predicting Severity of Dehydration in Children with Acute Gastroenteritis

OpenAIRE

Hoxha, Teuta Faik; Azemi, Mehmedali; Avdiu, Muharrem; Ismaili-jaha, Vlora; Grajqevci, Violeta; Petrela, Ela

2014-01-01

ABSTRACT Background: An accurate assessment of the degree of dehydration in infants and children is important for proper decision-making and treatment. This emphasizes the need for laboratory tests to improve the accuracy of clinical assessment of dehydration. The aim of this study was to assess the relationship between clinical and laboratory parameters in the assessment of dehydration. Methods: We evaluated prospectively 200 children aged 1 month to 5 years who presented with diarrhea, vomi...

Presence and dehydration of ikaite, calcium carbonate hexahydrate, in frozen shrimp shell.

Science.gov (United States)

Mikkelsen, A; Andersen, A B; Engelsen, S B; Hansen, H C; Larsen, O; Skibsted, L H

1999-03-01

Ikaite, calcium carbonate hexahydrate, has by means of X-ray diffraction analyses of frozen samples been identified as the mineral component of the white spots formed in the shell of frozen shrimp during storage. When the shrimp thaw and the shell material is dried and kept at room temperature, ikaite rapidly transforms into a mixture of anhydrous calcium carbonate forms. X-ray diffraction analyses and Raman spectra of synthetic ikaite as well as the dehydration product confirm the assignments, and the rate constant for dehydration is approximately 7 x 10(-)(4) s(-)(1) at ambient temperature. Differential scanning calorimetry showed that dehydration of synthetic ikaite is an entropy-driven, athermal process and confirms that a single first-order reaction is rate-determining. Ikaite is found to be stable in aqueous solution at temperatures below 5 degrees C and in the shell of frozen shrimps but decomposes on thawing to form anhydrous calcium carbonates.

Pervaporation : membranes and models for the dehydration of ethanol

NARCIS (Netherlands)

Spitzen, Johannes Wilhelmus Franciscus

1988-01-01

In this thesis the dehydration of ethanol/water mixtures by pervaporation using homogeneous membranes is studied. Both the general transport mechanism as well as the development of highly selective membranes for ethanol/water separation are investigated.

[Hereditary motor and sensory Lom-neuropathy--first Hungarian case report].

Science.gov (United States)

Szabó, Antal; Siska, Eva; Molnár, Mária Judit

2007-01-20

Hereditary motor and sensory neuropathy-Lom is an autosomal recessive disorder of the peripheral nervous system, which occurs only in the european Roma population. The symptoms start in the first decade with slowly progressive gait disturbance, weakness and wasting of distal upper extremity muscles, joint deformities and hearing loss develop later in the second and third decades. This disorder is caused by a homozygous missense mutation of the NDRG1 gene, located in the 8q24 region. The Schwann cell dysfunction is most probably caused by altered lipid metabolism as a consequence of the NDRG1 mutation. Molecular genetic testing can be a first diagnostic step among roma individuals showing a Lom neuropathy phenotype, making evaluation of such patients and also genetic counselling faster and easier. Screening for hereditary neuromuscular disorders in this genetically isolated community may become an important public health issue in the near future.

Orthodontic treatment for a patient with hereditary angiodema: a case report.

Science.gov (United States)

Waldon, Kate; Barber, Sophy Kathleen; Spencer, Richard James

2015-05-01

Hereditary angiodema (HAE), also known as C1 esterase inhibitor deficiency, causes sufferers to experience episodic subcutaneous and submucosal oedema. These episodes can be triggered by dental treatment and manifest as life-threatening oedematous swelling in the head and neck region. This case report reviews an adolescent with hereditary angiodema whose malocclusion required orthodontic intervention. Due to her complex and unpredictable reaction to dental treatment, various options were explored before determining the appropriate care pathway for this patient. Trial placement of a sectional fixed appliance tested the tissue reaction prior to comprehensive treatment including extractions and fixed orthodontic appliances. This report demonstrates successful interdisciplinary management facilitating orthodontic care in a patient with HAE. © 2014 BSPD, IAPD and John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Hereditary non-polyposis colorectal cancer/Lynch syndrome in three dimensions.

Science.gov (United States)

Kravochuck, Sara E; Church, James M

2017-12-01

Hereditary non-polyposis colorectal cancer (HNPCC) is defined by family history, and Lynch syndrome (LS) is defined genetically. However, universal tumour testing is now increasingly used to screen for patients with defective mismatch repair. This mixing of the results of family history, tumour testing and germline testing produces multiple permutations and combinations that can foster confusion. We wanted to clarify hereditary colorectal cancer using the three dimensions of classification: family history, tumour testing and germline testing. Family history (Amsterdam I or II criteria versus not Amsterdam criteria) was used to define patients and families with HNPCC. Tumour testing and germline testing were then performed to sub-classify patients and families. The permutations of these classifications are applied to our registry. There were 234 HNPCC families: 129 had LS of which 55 were three-dimensional Lynch (family history, tumour testing and germline testing), 66 were two-dimensional Lynch and eight were one-dimensional Lynch. A total of 10 families had tumour Lynch (tumours with microsatellite instability or loss of expression of a mismatch repair protein but an Amsterdam-negative family and negative germline testing), five were Lynch like (Amsterdam-positive family, tumours with microsatellite instability or loss of expression of a mismatch repair protein on immunohistochemistry but negative germline testing), 26 were familial colorectal cancer type X and 95 were HNPCC. Hereditary colorectal cancer can be confusing. Sorting families in three dimensions can clarify the confusion and may direct further testing and, ultimately, surveillance. © 2016 Royal Australasian College of Surgeons.

Characterization of the Promoter Region of an Arabidopsis Gene for 9-cis-Epoxycarotenoid Dioxygenase Involved in Dehydration-Inducible Transcription

Science.gov (United States)

Behnam, Babak; Iuchi, Satoshi; Fujita, Miki; Fujita, Yasunari; Takasaki, Hironori; Osakabe, Yuriko; Yamaguchi-Shinozaki, Kazuko; Kobayashi, Masatomo; Shinozaki, Kazuo

2013-01-01

Plants respond to dehydration stress and tolerate water-deficit status through complex physiological and cellular processes. Many genes are induced by water deficit. Abscisic acid (ABA) plays important roles in tolerance to dehydration stress by inducing many stress genes. ABA is synthesized de novo in response to dehydration. Most of the genes involved in ABA biosynthesis have been identified, and they are expressed mainly in leaf vascular tissues. Of the products of such genes, 9-cis-epoxycarotenoid dioxygenase (NCED) is a key enzyme in ABA biosynthesis. One of the five NCED genes in Arabidopsis, AtNCED3, is significantly induced by dehydration. To understand the regulatory mechanism of the early stages of the dehydration stress response, it is important to analyse the transcriptional regulatory systems of AtNCED3. In the present study, we found that an overlapping G-box recognition sequence (5′-CACGTG-3′) at −2248 bp from the transcriptional start site of AtNCED3 is an important cis-acting element in the induction of the dehydration response. We discuss the possible transcriptional regulatory system of dehydration-responsive AtNCED3 expression, and how this may control the level of ABA under water-deficit conditions. PMID:23604098

Clinical and genetic characteristics of Chinese hereditary nonpolyposis colorectal cancer families

OpenAIRE

Wang, Xu-Lin; Yuan, Ying; Zhang, Su-Zhan; Cai, Shan-Rong; Huang, Yan-Qin; Jiang, Qiang; Zheng, Shu

2006-01-01

AIM: To analyze the clinical characteristics of Chinese hereditary nonpolyposis colorectal cancer (HNPCC) families and to screen the germline mutations of human mismatch repair genes hMLH1 and hMSH2 in the probands.

Comparative study of the loss of vitamin C in chalarina (Casimiroa edulis) by four methods of dehydration

OpenAIRE

Judith Castañeda; Hubert Arteaga; Raúl Siche; Gilbert Rodriguez

2010-01-01

The aim of this study was to compare the loss of chalarina vitamin C after applying four methods of dehydration: vacuum osmotic dehydration combined with convective drying, convective drying, osmotic dehydration combined with vacuum freeze-drying, and freeze-drying. The fresh chalarina was fitted into cubes of 1 cm; we determined the vitamin C content and other chemical properties of the pulp. The powders were characterized: vitamin C, moisture content, aw, density, angle of flow and compress...

RB1 mutations and second primary malignancies after hereditary retinoblastoma

NARCIS (Netherlands)

Dommering, Charlotte J.; Marees, Tamara; van der Hout, Annemarie H.; Imhof, Saskia M.; Meijers-Heijboer, Hanne; Ringens, Peter J.; van Leeuwen, Flora E.; Moll, Annette C.

2012-01-01

Survivors of hereditary retinoblastoma have a high risk of second primary malignancies, but it has not been investigated whether specific RB1 germline mutations are associated with greater risk of second primary malignancies in a large cohort. We conducted a retrospective cohort study of 199

RB1 mutations and second primary malignancies after hereditary retinoblastoma

NARCIS (Netherlands)

Dommering, Charlotte J.; Marees, Tamara; van der Hout, Annemarie H.; Imhof, Saskia M.; Meijers-Heijboer, Hanne; Ringens, Peter J.; van Leeuwen, Flora E.; Moll, Annette C.

Survivors of hereditary retinoblastoma have a high risk of second primary malignancies, but it has not been investigated whether specific RB1 germline mutations are associated with greater risk of second primary malignancies in a large cohort. We conducted a retrospective cohort study of 199

Icatibant, a new bradykinin-receptor antagonist, in hereditary angioedema.

Science.gov (United States)

Cicardi, Marco; Banerji, Aleena; Bracho, Francisco; Malbrán, Alejandro; Rosenkranz, Bernd; Riedl, Marc; Bork, Konrad; Lumry, William; Aberer, Werner; Bier, Henning; Bas, Murat; Greve, Jens; Hoffmann, Thomas K; Farkas, Henriette; Reshef, Avner; Ritchie, Bruce; Yang, William; Grabbe, Jürgen; Kivity, Shmuel; Kreuz, Wolfhart; Levy, Robyn J; Luger, Thomas; Obtulowicz, Krystyna; Schmid-Grendelmeier, Peter; Bull, Christian; Sitkauskiene, Brigita; Smith, William B; Toubi, Elias; Werner, Sonja; Anné, Suresh; Björkander, Janne; Bouillet, Laurence; Cillari, Enrico; Hurewitz, David; Jacobson, Kraig W; Katelaris, Constance H; Maurer, Marcus; Merk, Hans; Bernstein, Jonathan A; Feighery, Conleth; Floccard, Bernard; Gleich, Gerald; Hébert, Jacques; Kaatz, Martin; Keith, Paul; Kirkpatrick, Charles H; Langton, David; Martin, Ludovic; Pichler, Christiane; Resnick, David; Wombolt, Duane; Fernández Romero, Diego S; Zanichelli, Andrea; Arcoleo, Francesco; Knolle, Jochen; Kravec, Irina; Dong, Liying; Zimmermann, Jens; Rosen, Kimberly; Fan, Wing-Tze

2010-08-05

Hereditary angioedema is characterized by recurrent attacks of angioedema of the skin, larynx, and gastrointestinal tract. Bradykinin is the key mediator of symptoms. Icatibant is a selective bradykinin B2 receptor antagonist. In two double-blind, randomized, multicenter trials, we evaluated the effect of icatibant in patients with hereditary angioedema presenting with cutaneous or abdominal attacks. In the For Angioedema Subcutaneous Treatment (FAST) 1 trial, patients received either icatibant or placebo; in FAST-2, patients received either icatibant or oral tranexamic acid, at a dose of 3 g daily for 2 days. Icatibant was given once, subcutaneously, at a dose of 30 mg. The primary end point was the median time to clinically significant relief of symptoms. A total of 56 and 74 patients underwent randomization in the FAST-1 and FAST-2 trials, respectively. The primary end point was reached in 2.5 hours with icatibant versus 4.6 hours with placebo in the FAST-1 trial (P=0.14) and in 2.0 hours with icatibant versus 12.0 hours with tranexamic acid in the FAST-2 trial (P<0.001). In the FAST-1 study, 3 recipients of icatibant and 13 recipients of placebo needed treatment with rescue medication. The median time to first improvement of symptoms, as assessed by patients and by investigators, was significantly shorter with icatibant in both trials. No icatibant-related serious adverse events were reported. In patients with hereditary angioedema having acute attacks, we found a significant benefit of icatibant as compared with tranexamic acid in one trial and a nonsignificant benefit of icatibant as compared with placebo in the other trial with regard to the primary end point. The early use of rescue medication may have obscured the benefit of icatibant in the placebo trial. (Funded by Jerini; ClinicalTrials.gov numbers, NCT00097695 and NCT00500656.)

Reliability of Urinary Dehydration Markers in Elite Youth Boxers.

Science.gov (United States)

Zubac, Damir; Cular, Drazen; Marusic, Uros

2018-03-01

To determine the reliability and diagnostic accuracy of noninvasive urinary dehydration markers in field-based settings on a day-to-day basis in elite adolescent amateur boxers. Sixty-nine urine samples were collected daily from 23 athletes (17.3 ± 1.9 y) during their weight-stable phase and analyzed by field and laboratory measures of hydration status. Urine osmolality (U OSM ), urine specific gravity (U SG ), total protein content (T PC ), and body-mass stability were evaluated to determine fluid balance and hydration status. Overall macronutrient and water intake were determined using dietary records. According to their anthropometric characteristics, athletes were assigned into 2 groups: lightweight (L WB ) and heavyweight (H WB ) boxers. Data presented on U OSM demonstrated a uniform increment by 11.2% ± 12.8% (L WB ) and 19.9% ± 22.7% (H WB ) (P dehydration markers. Poor correlations were found between U SG and T PC metabolites (r = .27, P = .211). Urinary dehydration markers (both U SG and U OSM ) exhibit high variability and seem to be unreliable diagnostic tools to track actual body-weight loss in real-life settings. The ad libitum fluid intake was apparently inadequate to match acute fluid loss during and after intense preparation. The applicability of a single-time-point hydration-status assessment concept may preclude accurate assessment of actual body-weight deficits in youth boxers.

Solid-vapor interactions: Influence of environmental conditions on the dehydration of carbamazepine dihydrate

OpenAIRE

Surana, Rahul; Pyne, Abira; Suryanarayanan, Raj

2003-01-01

The goal of this research was a phenomenological study of the effect of environmental factors on the dehydration behavior of carbamazepine dihydrate. Dehydration experiments were performed in an automated vapor sorption apparatus under a variety of conditions, and weight loss was monitored as a function of time. In addition to lattice water, carbamazepine dihydrate contained a significant amount of physically bound water. Based on the kinetics of water loss, it was possible to differentiate b...

Prophylactic Therapy for Hereditary Angioedema.

Science.gov (United States)

Longhurst, Hilary; Zinser, Emily

2017-08-01

Long-term prophylaxis is needed in many patients with hereditary angioedema and poses many challenges. Attenuated androgens are effective in many but are limited by side effect profiles. There is less evidence for efficacy of tranexamic acid and progestagens; however, the small side effect profile makes tranexamic acid an option for prophylaxis in children and progestagens an option for women. C1 inhibitor is beneficial, but at present requires intravenous delivery and may need dose titration for maximum efficacy. Short-term prophylaxis should be considered for all procedures. New therapies are promising in overcoming many problems encountered with current options for long-term prophylaxis. Copyright © 2017 Elsevier Inc. All rights reserved.

DEHYDRATION OF EDIBLE MUSHROOMS (PLEUROTUS OSTREATUS)

OpenAIRE

Salas de la Torre, N.; Bazán, D.; Osorio, A.; Cornejo, O.; Carrero, E.

2014-01-01

The edible mushroom Pleurotus ostreatus have been subjected to thermal, chemical and thermal-chemical treatment. The results show that the chemical treatment produces a more effective enzymatic inactivation compared to the other two treatments. Also, the experimental study of fungi dehydration carried out at 55 ° C reveals that the critical moisture content is 10.4 kg water / kg dry solids, the equilibrium moisture is 0.22 kg water / kg of solid . Los hongos comestibles Pleurotus ostreatus...

Efficiency of Osmotic Dehydration of Apples in Polyols Solutions

Directory of Open Access Journals (Sweden)

Joanna Cichowska

2018-02-01

Full Text Available The present study aimed to evaluate the influence of selected compounds from the polyol group, as well as other saccharides, on the osmotic dehydration process of apples. The following alternative solutions were examined: erythritol, xylitol, maltitol, inulin and oligofructose. Efficiency of the osmotic dehydration process was evaluated based on the kinetics of the process, and through comparison of the results obtained during the application of a sucrose solution. This innovative research utilizes alternative solutions in osmotic pretreatment, which until now, have not been commonly used in fruit processing by researchers worldwide. Results indicate that erythritol and xylitol show stronger or similar efficiency to sucrose; however, the use of inulin, as well as oligofructose, was not satisfactory due to the insufficient, small osmotic driving forces of the process, and the low values of mass transfer parameters.

Increasing fluid intake and reducing dehydration risk in older people living in long-term care: a systematic review.

Science.gov (United States)

Bunn, Diane; Jimoh, Florence; Wilsher, Stephanie Howard; Hooper, Lee

2015-02-01

To assess the efficacy of interventions and environmental factors on increasing fluid intake or reducing dehydration risk in older people living in long-term care facilities. Systematic review of intervention and observational studies. Thirteen electronic databases were searched from inception until September 2013 in all languages. References of included papers and reviews were checked. Intervention and observational studies investigating modifiable factors to increase fluid intake and/or reduce dehydration risk in older people (≥65 years) living in long-term care facilities who could drink orally. Two reviewers independently screened, selected, abstracted data, and assessed risk of bias from included studies; narrative synthesis was performed. A total of 4328 titles and abstracts were identified, 325 full-text articles were obtained and 23 were included in the review. Nineteen intervention and 4 observational studies from 7 countries investigated factors at the resident, institutional, or policy level. Overall, the studies were at high risk of bias due to selection and attrition bias and lack of valid outcome measures of fluid intake and dehydration assessment. Reported findings from 6 of the 9 intervention studies investigating the effect of multicomponent strategies on fluid intake or dehydration described a positive effect. Components included greater choice and availability of beverages, increased staff awareness, and increased staff assistance with drinking and toileting. Implementation of the US Resident Assessment Instrument reduced dehydration prevalence from 3% to 1%, P = .01. Two smaller studies reported positive effects: one on fluid intake in 9 men with Alzheimer disease using high-contrast red cups, the other involved supplementing 13 mildly dehydrated residents with oral hydration solution over 5 days to reduce dehydration. Modifications to the dining environment, advice to residents, presentation of beverages, and mode of delivery (straw vs beaker

Dehydration treatment practices among pediatrics-trained and non-pediatrics trained emergency physicians.

Science.gov (United States)

Nunez, Jeranil; Liu, Deborah R; Nager, Alan L

2012-04-01

We sought to survey emergency physicians in the United States regarding the management of pediatric dehydration secondary to acute gastroenteritis. We hypothesized that responses from physicians with dedicated pediatric training (PT), that is, board certification in pediatrics or pediatric emergency medicine, would differ from responses of physicians with no dedicated pediatric training (non-PT). An anonymous survey was mailed to randomly selected members of the American College of Emergency Physicians and sent electronically to enrollees of Brown University pediatric emergency medicine listserv. The survey consisted of 17 multiple-choice questions based on a clinical scenario depicting a 2-year-old with acute gastroenteritis and moderate dehydration. Questions asked related to treatment preferences, practice setting, and training information. One thousand sixty-nine surveys were received: 997 surveys were used for data analysis, including 269 PT physicians and 721 non-PT physicians. Seventy-nine percent of PT physicians correctly classified the scenario patient as moderately dehydrated versus 71% of non-PT physicians (P = 0.063). Among those who correctly classified the patient, 121 PT physicians (58%) and 350 non-PT physicians (68%) would initially hydrate the patient with intravenous fluids. Pediatrics-trained physicians were more likely to initially choose oral or nasogastric hydration compared with non-PT physicians (P = 0.0127). Pediatrics-trained physicians were less likely to perform laboratory testing compared with the non-PT group (n = 92, 45%, vs n = 337, 66%; P dehydrated children, significantly more PT physicians, compared with non-PT physicians, follow established guidelines.

Treatment of Hereditary Hemorrhagic Telangiectasia-Related Epistaxis.

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Sautter, Nathan B; Smith, Timothy L

2016-06-01

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease with an incidence of 1:5000. Recurrent, spontaneous epistaxis is the most common presenting symptom. Severity of epistaxis varies widely, from mild, self-limited nosebleeds to severe, life-threatening nasal hemorrhage. Treatment of HHT-related epistaxis presents a challenge to the otolaryngologist due to the recurrent, persistent nature of epistaxis often requiring multiple treatments. Treatment modalities range from conservative topical therapies to more aggressive surgical treatments. Copyright © 2016 Elsevier Inc. All rights reserved.

Coping with dehydration: sympathetic activation and regulation of glutamatergic transmission in the hypothalamic PVN

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Bardgett, Megan E.; Chen, Qing-Hui; Guo, Qing; Calderon, Alfredo S.; Andrade, Mary Ann

2014-01-01

Autonomic and endocrine profiles of chronic hypertension and heart failure resemble those of acute dehydration. Importantly, all of these conditions are associated with exaggerated sympathetic nerve activity (SNA) driven by glutamatergic activation of the hypothalamic paraventricular nucleus (PVN). Here, studies sought to gain insight into mechanisms of disease by determining the role of PVN ionotropic glutamate receptors in supporting SNA and mean arterial pressure (MAP) during dehydration and by elucidating mechanisms regulating receptor activity. Blockade of PVN N-methyl-d-aspartate (NMDA) receptors reduced (P dehydrated (DH) (48 h water deprivation) rats, but had no effect in euhydrated (EH) controls. Blockade of PVN α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptors had no effect in either group. NMDA in PVN caused dose-dependent increases of renal SNA and MAP in both groups, but the maximum agonist evoked response (Emax) of the renal SNA response was greater (P dehydration increases excitatory NMDA receptor tone in PVN. Reduced glial-mediated glutamate uptake was identified as a key contributing factor. Defective glutamate uptake in PVN could therefore be an important, but as yet unexplored, mechanism driving sympathetic hyperactivity in chronic cardiovascular diseases. PMID:24671240

Investigation into structure and dehydration dynamic of gallic acid monohydrate: A Raman spectroscopic study.

Science.gov (United States)

Cai, Qiang; Xue, Jiadan; Wang, Qiqi; Du, Yong

2018-05-02

The dehydration process of gallic acid monohydrate was carried out by heating method and characterized using Raman spectroscopic technique. Density functional theory calculation with B3LYP function is applied to simulate optimized structures and vibrational frequencies of anhydrous gallic acid and its corresponding monohydrated form. Different vibrational modes are assigned by comparison between experimental and theoretical Raman spectra of above two polymorphs. Raman spectra show that vibrational modes of the monohydrate are distinctively different from those of anhydrous one. Meanwhile, the dynamic information about dehydration process of gallic acid monohydrate could also be observed and monitored directly with the help of Raman spectral analysis. The decay rate of the characteristic band from gallic acid monohydrate and the growth rate of anhydrous one are pretty consistent with each other. It indicates that there is no intermediate present during the dehydration process of gallic acid monohydrate. The results could offer us benchmark works for identifying both anhydrous and hydrated pharmaceutical compounds, characterizing their corresponding molecular conformation within various crystalline forms, and also providing useful information about the process of dehydration dynamic at the microscopic molecular level. Copyright © 2018 Elsevier B.V. All rights reserved.

Genes for hereditary sensory and autonomic neuropathies : a genotype-phenotype correlation

NARCIS (Netherlands)

Rotthier, Annelies; Baets, Jonathan; De Vriendt, Els; Jacobs, An; Auer-Grumbach, Michaela; Levy, Nicolas; Bonello-Palot, Nathalie; Kilic, Sara Sebnem; Weis, Joachim; Nascimento, Andres; Swinkels, Marielle; Kruyt, Moyo C.; Jordanova, Albena; De Jonghe, Peter; Timmerman, Vincent

2009-01-01

Hereditary sensory and autonomic neuropathies (HSAN) are clinically and genetically heterogeneous disorders characterized by axonal atrophy and degeneration, exclusively or predominantly affecting the sensory and autonomic neurons. So far, disease-associated mutations have been identified in seven