bluuebunny/crossref_metadata_2025 huggingface dataset:
Neonatal salt‐wasting and 11 β‐hydroxylase deficiency in a child carrying a homozygous deletion hybrid CYP11B2 (aldosterone synthase)–CYP11B1 (11 β‐hydroxylase)
Authors: Ezquieta B , Luzuriaga Cristina
Published: August 2004

This article reports the case of a boy diagnosed at 1.8 years of age with congenital adrenal hyperplasia due to 11 β‐hydroxylase deficiency. The patient showed salt‐wasting episodes during the neonatal period. On molecular analysis, a homozygous deletion hybrid (CYP11B2–CYP11B1) involving the CYP11B locus at 8q24.3 was found. Southern blot analysis showed the break point of the chimera gene to be located before intron 5; sequence analysis identified it at exon 4 between codons 202 and 248. This CYP11B2(5′)/B1(3′) hybrid should lack aldosterone synthase activity (due to the CYP11B1 residues at exons 5 and 6), and the enzyme it codes for should not be promoted by adrenocorticotropic hormone (ACTH) (CYP11B2 promoter sequences). The patient phenotype – neonatal salt‐wasting and 11 β‐hydroxylase deficiency – is in agreement with this hybrid structure. This is the first time a homozygous deletion hybrid generated by unequal crossover has been described in exon 4. This genetic lesion appears to be the reciprocal product from the recombination event that causes glucocorticoid‐remediable aldosteronism, a duplication dominant allele (CYP11B2–CYP11B1/B2–CYP11B1) coding for additional aldosterone synthase activity regulated by ACTH. The clinical presentation of the condition in this patient contributes to the in vivo understanding of the regulation of this complex locus in which two ‘duplicated’ genes have evolved different regulatory and enzymatic activities involved in mineralocorticoid and glucocorticoid synthesis in the adrenal glands. The fact that this allele was first predicted and has now been documented clinically and molecularly in vivo is particularly noteworthy.
Identification of somatic and germline mosaicism for a keratin 5 mutation in epidermolysis bullosa simplex in a family of which the proband was previously regarded as a sporadic case
Authors: Nagao‐Watanabe M , Fukao T , Matsui E , Kaneko H , Inoue R , Kawamoto N , Kasahara K , Nagai M , Ichiki Y , Kitajima Y , Kondo N
Published: August 2004

Epidermolysis bullosa simplex (EBS) is an autosomal‐dominant inherited blistering skin disease characterized by intraepidermal blistering due to mechanical stress‐induced degeneration of basal keratinocytes. EBS is caused by mutations in either keratin 5 or keratin 14, the major keratins expressed in the basal layer of the epidermis. We experienced a unique EBS‐affected family. The proband had a heterozygous 1649delG mutation in the keratin 5 gene and had been reported as a case of de novo mutation, because the mutations were not detected in the parents' DNA from blood samples. However, the proband's younger sister was revealed to have the same disease at birth and we found the same mutation in her. We reinvestigated the familial segregation of the 1649delG mutation and it was shown that the mother's DNA from hair bulb and buccal cell samples had the 1649delG mutation heterozygously, but her DNA from blood samples did not. A careful check on the mother's history disclosed that she had migratory circinate pigmentation in her skin in childhood, which means maternal somatic and germline mosaicism. The demonstration of somatic and gonadal mosaicism in the keratin 5 gene is important for accurate genetic counselling of families with sporadic cases of EBS.
Non‐pharmacologic treatment strategies in gastroesophageal reflux disease
Authors: SOLLANO JOSE D
Published: August 2004

Abstract  In the West, gastroesophageal reflux disease (GERD) is a common and well‐recognized disease. Lately, it has been described as an emerging problem in the East as well. While it is not a rapidly fatal illness, it causes a myriad of disturbing symptoms that remarkably reduce the patients’ quality of life (QOL). The economic impact that results from multiple consultations, diagnostic investigations, and administration of a variety of treatment regimens, including surgery, is enormous.

The operative management for GERD is fundoplication, for example Toupet (270 degree wrap of the distal esophagus) and Nissen (360 degree wrap of the distal esophagus). These surgical procedures are aimed at permanently controlling acid reflux by reconstructing the gastroesophageal junction. Currently, the ease, aesthetic advantages, and the comparable outcomes achieved by minimally invasive laparoscopic fundoplication have rekindled interest in the operative alternatives of GERD management. Fundoplication controls or diminishes considerably the severity of the symptoms associated with GERD. However, appearance of new symptoms i.e. dysphagia, ‘gas–bloat syndrome’, etc. as postoperative events have been reported.

Recently, several innovative endoluminal treatment modalities have been introduced, namely; endoscopic plicator/suturing devices, bulking injections, and radiofrequency treatment. They are focused on enhancing the performance of a malfunctioning lower esophageal sphincter. While results of several case series reflect substantial improvements in GERD‐HRQL scores, lack of long‐term durability data is a major concern when recommending these novel, relatively simple, peroral techniques to a long suffering patient. It is clear that these therapies are still evolving and long‐term outcomes of properly designed comparative efficacy trials are awaited.
Endoanal and endorectal ultrasound: applications in colorectal surgery
Authors: Rieger Nicholas , Tjandra Joe , Solomon Michael
Published: August 2004

Endoanal and endorectal ultrasound have an important role in colorectal surgery. They can be applied in the management of faecal incontinence, rectal tumours and inflammatory perianal conditions. In faecal incontinence, anal ultrasound will confirm the presence or absence of sphincter defects. This will direct any operative intervention such as direct sphincter repair. Ultrasound in rectal cancer allows staging of the tumour by assessing the depth of invasion through the bowel wall and involvement of mesenteric nodes. Such staging might influence the choice of operation and determine which patients might benefit from preoperative chemotherapy and radiotherapy. Ultrasound has a particular role in recurrent and complex anal fistula and perianal sepsis. Preoperative and perioperative planning with accurate delineation of fistula tracts, extensions and sphincter involvement might help prevent recurrence and impaired continence from sphincter damage after surgery. Correct interpretation of ultrasound images requires training and experience so that the results can be properly correlated with the clinical situation.
Stoichiometry of actual vs. potential predator–prey interactions: insights into nitrogen limitation for arthropod predators
Authors: Fagan William F. , Denno Robert F.
Published: August 2004

Literature‐compiled data sets demonstrate wide interspecific variation in nitrogen content among terrestrial arthropods and raise the possibility of nitrogen (N) limitation for predatory species. It remains unclear, however, whether the same disparities between N supply and demand that appear in literature compilations also exist in particular ecological communities. To address this uncertainty, we compared arthropod predator–prey stoichiometries derived from a compiled database with those from a natural Spartina saltmarsh community. Separate assessments of potential N‐limitation were made for arthropod predators feeding on herbivores and for intraguild predators feeding on intraguild prey. Relative to the compiled database, saltmarsh consumer–resource interactions exhibited increased disparity between N‐content of herbivores and N‐demand by predators. The high N content of saltmarsh arachnids relative to predatory insects at large may contribute to the supply‐demand disparity. Whether N‐limitation of terrestrial arthropod predators is widespread in the marsh, and in nature in general, depends sensitively on the predatory species’ gross growth efficiencies for N and carbon. Obtaining hard empirical data for these efficiency parameters should be a research goal.
A bidirectional gene trap construct suitable for T‐DNA and Ds‐mediated insertional mutagenesis in rice (Oryza sativa L.)
Authors: Eamens Andrew L. , Blanchard Chris L. , Dennis Elizabeth S. , Upadhyaya Narayana M.
Published: August 2004

A construct suitable for genome‐wide transfer‐DNA (T‐DNA) and subsequent transposon‐based (Ds) gene trapping has been developed for use in rice (Oryza sativa). This T‐DNA/Ds construct contains: Ds terminal sequences immediately inside T‐DNA borders for subsequent Ds mobilization; promoterless green fluorescent protein (sgfpS65T) and β‐glucuronidase (uidA) reporter genes, each fused to an intron (from Arabidopsis GPA1 gene) to enable bidirectional gene trapping by T‐DNA or Ds; an ampicillin resistance gene (bla) and a bacterial origin of replication (ori) to serve as the plasmid rescue system; an intron‐containing hygromycin phosphotransferase gene (hph) as a selectable marker or Ds tracer; and an intron‐containing barnase gene in the binary vector backbone (VB) to select against transformants carrying unwanted VB sequences. More than a threefold increase over previously reported reporter gene‐based gene trapping efficiencies was observed in primary T‐DNA/Ds transformant rice lines, returning an overall reporter gene expression frequency of 23%. Of the plant organs tested, 3.3–7.4% expressed either reporter at varying degrees of organ or tissue specificity. Approximately 70% of the right border (RB) flanking sequence tags (FSTs) retained 1–6 bp of the RB repeat and 30% of the left border (LB) FSTs retained 5–23 bp of the LB repeat. The remaining FSTs carried deletions of 2–84 bp inside the RB or 1–97 bp inside the LB. Transposition of Ds from the original T‐DNA was evident in T‐DNA/Ds callus lines super‐transformed with a transposase gene (Ac) construct, as indicated by gene trap reporter activity and rescue of new FSTs in the resulting double transformant lines.
Bt rice harbouring cry genes controlled by a constitutive or wound‐inducible promoter: protection and transgene expression under Mediterranean field conditions
Authors: Breitler Jean Christophe , Vassal Jean Michel , Del Mar Catala Maria , Meynard Donaldo , Marfà Victoria , Melé Enric , Royer Monique , Murillo Isabel , San Segundo Blanca , Guiderdoni Emmanuel , Messeguer Joaquima
Published: August 2004

Seven homozygous transgenic lines of two European commercial cultivars of rice (Ariete (A) and Senia (S)), harbouring the cry1B or cry1Aa Bacillus thuringiensis (Bt) δ‐endotoxin genes, were field evaluated for protection from striped stem borer (SSB) (Chilo suppressalis) damage during the 2001 and 2002 summer crop seasons in the Delta de l’Ebre region, Spain. The plant codon‐optimized toxin gene was placed under the control of the promoter of either the constitutive ubi1 gene or the wound‐inducible mpi gene from maize. Stable, high‐level, insecticidal protein accumulation was observed throughout root, leaf and seed tissues of field‐grown plants harbouring the cry1B (lines A64.1, A33.1, A3.4 and S98.9) or cry1Aa (lines S05.1 and A19.14) genes under the control of the ubi1 promoter. Conversely, no toxin was detected in unwounded vegetative tissues of the A9.1 line harbouring the cry1B gene controlled by the mpi promoter, indicating that natural environmental stresses did not trigger the activity of the wound‐inducible promoter. However, the toxin accumulated at 0.2% total soluble proteins in A9.1 sheath tissue exhibiting brown lesions resulting from SSB damage. The agronomical traits and performance of the transgenic lines were generally comparable with parental controls, except in the two lines accumulating Cry1Aa, which exhibited a high frequency of plants non‐true to type. Natural infestation was assisted with manual infestations of L2/L3 SSB larvae in border control plants surrounding the experimental plots, which served as a reservoir for the second‐cycle SSB population. The observation of damage (brown lesions and dead hearts) during the crop season and dissection of plants at harvest stage revealed a range of protection amongst the transgenic lines, which was highly consistent with the level of toxin accumulation and with previous experience in greenhouse assays. Lines A3.4 and S05.1 were found to exhibit stable and full protection against SSB attacks, mediated by the accumulation of Cry1B and Cry1Aa toxin, respectively, which was comparable with that afforded by the spraying of chemical insecticides on control plants. The wound‐induced A9.1 line exhibited a satisfactory level of protection, with a notably low level of penetration of SSB larvae in the stems, but higher external symptoms than constitutive lines, probably due to the time lag to benefit from the protective effect of Cry1B.
Engineered multidomain cysteine protease inhibitors yield resistance against western flower thrips (Frankliniella occidentalis) in greenhouse trials
Authors: Outchkourov Nikolay S. , De Kogel Willem Jan , Wiegers Gerrie L. , Abrahamson Magnus , Jongsma Maarten A.
Published: August 2004

Western flower thrips, Frankliniella occidentalis (Pergande) (Thysanoptera: Thripidae), cause very large economic damage on a variety of field and greenhouse crops. In this study, plant resistance against thrips was introduced into transgenic potato plants through the expression of novel, custom‐made, multidomain protease inhibitors. Representative classes of inhibitors of cysteine and aspartic proteases [kininogen domain 3 (K), stefin A (A), cystatin C (C), potato cystatin (P) and equistatin (EIM)] were fused into reading frames consisting of four (K‐A‐C‐P) to five (EIM‐K‐A‐C‐P) proteins, and were shown to fold into functional inhibitors in the yeast Pichia pastoris. The multidomain proteins were expressed in potato and found to be more resistant to degradation by plant proteases than the individual domains. In a time span of 14–16 days, transgenic potato plants expressing EIMKACP and KACP at a similar concentration reduced the number of larvae and adults to less than 20% of the control. Leaf damage on protected plants was minimal. Engineered multidomain cysteine protease inhibitors thus provide a novel way of controlling western flower thrips in greenhouse and field crops, and open up possibilities for novel insect resistance applications in transgenic crops.
Procedural Texture Matching and Transformation
Authors: Bourque Eric , Dudek Gregory
Published: August 2004

We present a technique for creating a smoothly varying sequence of procedural textures that interpolates between arbitrary input samples of texture. This texture transformation uses a library of procedural shaders and selects the correct shaders and associated parameters to accomplish the task.

In general, selecting a procedural texture from a library, or finding the correct parameters to produce a smooth texture transition can be complex and time consuming. We propose a strategy for automating this process. While superficially this problem appears intractable for both humans and computational systems, its natural characteristics make a computational solution feasible. We present an algorithm and experimental results demonstrating this approach.

Transformation between two textures can then be achieved procedurally, while enforcing perceptual similarity constraints between adjacent texture frames. We describe a technique for efficiently sampling the parameter domain of a shader based on a texture similarity function to create a smooth path through its texture range. In the case of evolving between several shaders, a method is described to obtain the best jump‐points which can be used to connect different shaders smoothly in texture space. Several examples of the technique are shown, and future directions as well as potential problems are discussed.

Categories and Subject Descriptors (according to ACM CCS): I.3.7 [Computer Graphics]: Texture
GPU‐Based Nonlinear Ray Tracing
Authors: Weiskopf Daniel , Schafhitzel Tobias , Ertl Thomas
Published: August 2004

In this paper, we present a mapping of nonlinear ray tracing to the GPU which avoids any data transfer back to main memory. The rendering process consists of the following parts: ray setup according to the camera parameters, ray integration, ray‐object intersection, and local illumination. Bent rays are approximated by polygonal lines that are represented by textures. Ray integration is based on an iterative numerical solution of ordinary differential equations whose initial values are determined during ray setup. To improve the rendering performance, we propose acceleration techniques such as early ray termination and adaptive ray integration. Finally, we discuss a variety of applications that range from the visualization of dynamical systems to the general relativistic visualization in astrophysics and the rendering of the continuous refraction in media with varying density.

Categories and Subject Descriptors (according to ACM CCS): I.3.3 [Computer Graphics]: Picture/Image Generation I.3.7 [Computer Graphics]: Three‐Dimensional Graphics and Realism
Welfare through Work: An Audit of Occupational Social Provision at the Turn of the New Century
Authors: Farnsworth Kevin
Published: August 2004

Occupational welfare has been a relatively neglected area in both theoretical and empirical studies of the welfare state despite its importance to overall levels of social provision. Surprisingly, there has not yet been a comprehensive examination of British occupational social provision, as opposed to non‐wage benefits more generally or specific provision such as pensions, housing or childcare. This neglect can be explained both by the perception that occupational welfare plays a relatively insignificant role in contemporary welfare states and by a general lack of clarity regarding its definition and scope, factors which have added to the difficulties surrounding its conceptualization and measurement. Despite the lack of attention it has received, however, recent pressures have propelled the issue higher up the social policy agenda, increasing the need for a clearer conception of what constitutes occupational social provision and a more comprehensive assessment of its contemporary significance. This paper seeks to shed some light on to these areas by drawing on comparative and UK data in order to carry out an audit of occupational social provision.
Stressful major life events are associated with a higher frequency of cutaneous sensory symptoms: an empirical study of non‐clinical subjects
Authors: Gupta MA , Gupta AK
Published: August 2004

Background  Psychological stress resulting from stressful major life events is known to exacerbate a wide range of skin disorders.

Objective  To examine the relationship between stressful major life events and dermatological symptoms among a non‐clinical sample.

Design  A cross‐sectional survey.

Setting  Community‐based subjects from London, Ontario, Canada were recruited from the local university, schools and churches.

Participants  Out of 600 consecutive, consenting volunteers 316 subjects [73 men and 243 women; age 38.7 ± 14.8 (mean ± SD) years; marital status 54% married; race 94%‘white’] completed the survey for this study. The exclusion criterion was a history of a major dermatological or medical disorder.

Main outcome measure  The number of major life events experienced over the previous 6 months measured using the Social Readjustment Rating Scale (SRRS) of Holmes and Rahe, and the frequency and severity of a range of cutaneous symptoms (‘burning’, ‘crawling sensation’, ‘tingling’, ‘pricking’ or ‘pins and needles’, ‘pain’, ‘tenderness’ of skin, ‘numbness’, ‘moderate to severe itching’, and ‘easy bruising’) that the subject may have experienced over the previous month.

Results  The most frequently reported body region affected was the scalp (59.5%) and the most frequently reported symptom was itching (69.3%). The total number of major life events experienced over the previous 6 months correlated with the severity of the individual cutaneous symptoms (0.22 ≤ Pearson r ≤ 0.41, P < 0.001) and with the total cutaneous symptom severity score (sum of all cutaneous severity ratings) (Pearson r = 0.40, P < 0.001). This correlation remained significant after the possible confounding effect of psychological factors on cutaneous symptoms was partialled out statistically (partial r = 0.19, P = 0.001).

Discussion  We observed a direct correlation between the number of major life events experienced over the previous 6 months and cutaneous symptoms experienced over the previous 1 month by non‐clinical subjects. The correlation remained significant after the effect of psychological factors was partialled out, suggesting that this relationship holds even if the subject does not acknowledge psychological distress in reaction to the major life event.
Textile dermatitis in patients with contact sensitization in Israel: a 4‐year prospective study
Authors: Lazarov A
Published: August 2004

Background  The exact incidence of textile dermatitis is unknown because of the lack of controlled epidemiological studies. Nevertheless, the increasing frequency of contact dermatitis to clothing has been demonstrated, thus indicating the importance of further investigations in this field.

Objective  To analyse the results from a 4‐year prospective study of the clinical and aetiological features of contact dermatitis to textiles in Israel. We also aimed to assess the frequency and relevance of sensitization to textile dyes and resins in these patients.

Methods  Six hundred and forty‐four patients (441 female and 203 male), referred for the investigation of contact dermatitis, and suspected of having textile allergic contact dermatitis (ACD), were studied. All patients were patch tested with the standard series (TRUE Tests), textile colour and finish series (TCFS) clothing extracts and pieces of garment in some cases. Readings were performed on days 2, 3 and in many patients also on day 7.

Results  Eighty‐three patients (12.9%) had an allergic reactions to a dye and/or resin allergen. Of them, 43 (51.8%) had positive patch tests to the textile dye allergens, 28 (33.7%) to the formaldehyde and textile finish resins and 12 (14.4%) to allergens from both groups. The highest incidence of sensitization from the dye group allergens was due to Disperse Blue (DB) 124 (30.6%), DB 106 (27.0%) and DB 85 (8.1%) and from the resin group to melamine formaldehyde and ethyleneurea melamine formaldehyde (20.7% each) and urea formaldehyde (18.3%). Present relevance of the patch tests was found in 81.4% of the cases. Concomitant sensitization with allergens from the standard series included nickel sulphate, potassium dichromate, formaldehyde, rubber additives and others. Although chronic dermatitis was the typical clinical presentation, less frequent forms such as purpuric, hyperpigmented and papulopustular lesions and atypical forms such as erythema multiforme‐like, nummular‐like lesions, lichenification and erythrodermia were observed in 24.4% of the cases. The atypical manifestations were provoked by sensitization to dye allergens and never to resins. Along with the typical distribution in areas of friction on the trunk and extremities, less frequent areas including the hands, face, genital area and the soles were affected too.

Conclusions  In view of the increasing frequency of contact dermatitis to clothing, the clinical assessment should include awareness of the classical as well as the unusual and atypical clinical forms and locations of ACD to textiles, for they are not infrequent. Although dyes and among them DB 106 and DB 124 are the most frequent allergens inducing textile dermatitis, concomitant testing with allergens from the textile dyes and resin groups is recommended when investigating patients with textile dermatitis.
Cutaneous tuberculosis in Indian children: the importance of screening for involvement of internal organs
Authors: Pandhi D , Reddy BSN , Chowdhary S , Khurana N
Published: August 2004

Aims and objectives  Resurgence of skin tuberculosis especially with drug‐resistant strains has been well documented in recent years, but this problem has not received much attention in the paediatric age group. Hence, we carried out the present study to analyse the clinical and therapeutic aspects of cutaneous tuberculosis in children.

Materials and methods  A detailed clinical examination, investigations, such as haemogram, serology for HIV, Mantoux test, chest X‐ray, cytology, culture and histopathology were carried out in all children. They were treated with antitubercular therapy (WHO regimen), and the clinical response was followed up.

Results  Of 142 patients with cutaneous tuberculosis, 68 were children (40 females, 28 males). These children were aged from 9 months to 14 years. The duration of the disease varied from 1 month to 6 years. Family history of tuberculosis was present in 28 (41.2%) of the patients. Scrofuloderma was the most common presentation encountered in 30 (44.1%) patients with preferential involvement of the cervical (56.2%) and inguinal (20%) regions. Fifteen (22.1%) patients had lupus vulgaris, of which the keratotic type was the most common (46.7%), 16 had lichen scrofulosorum, three had tuberculosis verrucosa cutis, and four had more than one type of tuberculosis. Involvement of the lung in 14 (20.6%), bone in seven (10.2%), and both in four (5.9%) was found. Histopathology corroborated the clinical diagnosis in 54 (80.6%), culture was positive in six (8.8%). Fifty (73.5%) patients completed the treatment with an excellent response, no multidrug resistant cases were seen.

Conclusions  Cutaneous tuberculosis in children continues to be an important cause of morbidity, there is a high likelihood of internal involvement, especially in patients with scrofuloderma. A search is required for more sensitive, economic diagnostic tools. Response to treatment at 4 weeks often helps in substantiating the diagnosis of tuberculosis in doubtful cases.
Atrophic dermatofibroma
Authors: Ohnishi T , Sasaki M , Nakai K , Watanabe S
Published: August 2004

Atrophic dermatofibroma, a newly proposed entity in recent times, is thought to be a specific variant of dermatofibroma. We report a typical case of atrophic dermatofibroma on the thigh of a 69‐year‐old female. The lesion consisted clinically of a light brown, intracutaneous nodule with a central crateriform depression, and histologically of fibrohistiocytic components in the thinning dermis. On elastica van Gieson stain, loss of elastic fibres and dense accumulation of elastic fibres around medium‐sized vessels were observed in the lesion.
Angiolymphoid hyperplasia with eosinophilia of the nail bed and bone: successful treatment with radiation therapy
Authors: Conill C , Toscas I , Mascaró JM , Vilalta A , Mascaró JM
Published: August 2004

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare entity characterized by the presence of one or several papules or nodules in the skin. Histopathology of ALHE shows a marked proliferation of blood vessels with distinctive large endothelial cells and variable inflammatory infiltrates with eosinophils. We report a 32‐year‐old Caucasian woman with multiple nodules involving the skin, subcutaneous tissue and bone of the distal phalanx of the fingers that were treated successfully with orthovoltage radiation therapy (40 Gy/20 fractions) and without any side‐effects after 9 years of follow‐up.
Fixed drug eruption from piroxicam
Authors: Cuerda Galindo E , Goday Buján JJ , García Silva JM , Martínez W , Verea Hernando M , Fonseca E
Published: August 2004

The pathogenetic mechanism of fixed drug eruption (FDE) is still unknown. One of the most common causes of FDE is the use of nonsteroidal antiinflammatory drugs (NSAIDs). Oxicams are in the NSAID group and piroxicam is one of the most used of these drugs. FDE caused by piroxicam is rare but a few cases have been reported. Patch tests are useful for diagnosing some cases of FDE; they give variable results on previously affected skin while no reaction appears on unaffected skin. Some cases of cross‐sensitivity among piroxicam and other substances have been reported. We report two new cases of FDE due to piroxicam with negative patch test on normal skin and positive results on affected skin.
Necrolytic migratory erythema: a cutaneous clue to glucagonoma syndrome
Authors: Remes‐Troche JM , García‐de‐Acevedo B , Zuñiga‐Varga J , Avila‐Funes A , Orozco‐Topete R
Published: August 2004

Necrolytic migratory erythema (NME) is a cutaneous manifestation of the glucagonoma syndrome. We present a case with a pancreatic glucagon‐secreting tumour, skin eruption and a good response to treatment.
Two case reports of cutaneous adverse reactions following hepatitis B vaccine: lichen planus and granuloma annulare
Authors: Criado PR , De Oliveira Ramos R , Vasconcellos C , Jardim Criado RF , Valente NYS
Published: August 2004

We report two cases of adverse cutaneous reactions following hepatitis B vaccination. The first case occurred 3 weeks after the first dose of hepatitis B vaccine in a 16‐year‐old white girl with the onset of lichen planus lesions on her thighs and abdomen. After the second dose a disseminated lichen planus developed within 2 weeks. The second case concerns to the development of papular and patch granuloma annulare in a 58‐year‐old white woman 2 months after the second dose of hepatitis B vaccine. To the best of our knowledge, only a few paediatric and adult cases of lichen planus as a complication of hepatitis B vaccination have been reported in medical literature so far. This is the second case of granuloma annulare following hepatitis B vaccine. Our report, similar to earlier papers, appears to support the onset of lichen planus and granuloma annulare as a possible rare complication of hepatitis B immunization.
The Van der Woude syndrome: a case report and review of the literature
Authors: Dissemond J , Haberer D , Franckson T , Hillen U
Published: August 2004

The Van der Woude syndrome is a rare autosomal dominant developmental malformation usually associated with bilateral lower lip pits. These congenital lip pits appear clinically as a malformation in the vermilion border of the lip, with or without excretion. As a genetic defect has been identified as a microdeletion of chromosome bands 1q32–q41, genetic counselling of patients may be considered. A nonsense mutation in the interferon regulatory factor‐6 (IRF‐6) is discussed as a pathogenic relevant factor. Therapeutic intervention is generally not necessary, although surgical excision is especially indicated in patients with recurrent inflammation. Physicians should be aware of the Van der Woude syndrome because it has been reported to be associated with a variety of malformations or other congenital disorders.
Vesicular pityriasis rosea: response to erythromycin treatment
Authors: Miranda SB , Lupi O , Lucas E
Published: August 2004

Pityriasis rosea (PR) is a relatively common disease although its aetiology has not yet been identified. It occurs worldwide and there is no racial susceptibility factor. It usually affects teenagers and young adults between 10 and 35 years of age. Typical PR is much easier to diagnose than the rare atypical forms. We report a rare case of vesicular PR in a black woman who had vesicular lesions limited to her palms and soles in addition to regular typical lesions. We devised an efficient oral erythromycin treatment for this patient.
Supported Employment: a Route to Social Networks
Authors: Forrester‐Jones Rachel , Jones Samantha , Heason Sophie , Di'Terlizzi Michele
Published: August 2004

Background  Evidence suggests that social networks mediate social functioning, self‐esteem, mental health and quality of life. This paper presents findings concerning changes in the social lives, skills, behaviour and life experiences of a group of people with intellectual disabilities (n = 18), who gained support from an employment agency to find paid work.

Method  The composition and quality of individuals’ social networks were mapped using a Social Network Guide. Changes in skills, behaviour and life experiences were assessed using standardized measures.

Results  The social network size of participants increased over time, with most social contacts being drawn from community contexts. This linked to improvements in life experiences, particularly in relation to leisure activities. Some improvements in adaptive behaviour were also found over time but no significant relationship between social network size and changes in adaptive behaviour were evident.

Conclusions  Whilst work will not guarantee social relationships, it can help maintain network size and provides a good opportunity for people with intellectual disabilities to meet others who are not associated with intellectual disability services.
The Boys from Bothaville, or the Rise and Fall of King Maize: A South African Story
Authors: BERNSTEIN HENRY
Published: August 2004

This paper tells the story, for the first time, of a maverick maize farmers’ association in South Africa during the period of apartheid. NAMPO (National Maize Producers’ Organization), that grew out of SAMPI (South African Maize Producers’ Institute), ultimately achieved a unique, if short‐lived, breach in the normal operations of ‘organized agriculture’: a set of relations and practices that bound together white farmers, the National Party and the state. The paper provides an account of SAMPI/NAMPO's project of ‘King Maize’ and an explanation of its fall after a brief period of victory from 1981 to 1985. This explanation draws on broader patterns of agrarian change in contemporary capitalism combined with the fracturing of the original agrarian bloc of apartheid in the 1980s, marking the end of a ‘second moment’ of South Africa's version of a Prussian path of capitalist development.
Whole‐brain glutamate metabolism evaluated by steady‐state kinetics using a double‐isotope procedure: effects of gabapentin
Authors: Xu Y. , Öz G. , LaNoue K. F. , Keiger C. J. , Berkich D. A. , Gruetter R. , Hutson S. H.
Published: August 2004

Cerebral rates of anaplerosis are known to be significant, yet the rates measured in vivo have been debated. In order to track glutamate metabolism in brain glutamatergic neurons and brain glia, for the first time unrestrained awake rats were continuously infused with a combination of H14CO3– and [1−13C]glucose in over 50 infusions ranging from 5 to 60 min. In whole‐brain extracts from these animals, the appearance of 14C in brain glutamate and glutamine and appearance of 13C in the C‐4 position of glutamate and glutamine were measured as a function of time. The rate of total neuronal glutamate turnover, the anaplerotic rate of synthesis of glutamine and glutamate from H14CO3–, flux through the glutamate/glutamine cycle, and a minimum estimate of whole‐brain anaplerosis was obtained. The rate of synthesis of 14C‐glutamate from H14CO3– was 1.29 ± 0.11 nmoles/min/mg protein, whereas the rate of synthesis of 14C‐glutamine was 1.48 ± 0.10 nmoles/min/mg protein compared to total glutamate turnover of 9.39 ± 0.73 nmoles/min/mg protein. From the turnover rate of glutamine, an upper limit for flux through the glutamate/glutamine cycle was estimated at 4.6 nmoles/min/mg protein. Synthesis of glutamine from H14CO3– was substantial, amounting to 32% of the glutamate/glutamine cycle. These rates were not significantly affected by a single injection of 100 mg/kg of the antiepileptic drug gabapentin. In contrast, acute administration of gabapentin significantly lowered incorporation of H14CO3– into glutamate and glutamine in excised rat retinas, suggesting metabolic effects of gabapentin may require chronic treatment and/or are restricted to brain areas enriched in target enzymes such as the cytosolic branched chain aminotransferase. We conclude that the brain has a high anaplerotic activity and that the combination of two tracers with different precursors affords unique insights into the compartmentation of cerebral metabolism.
Quantitative Analytical Methods in Translation Research
Authors: Dawson Jeffrey D.
Published: August 2004

In research involving quality of health care, an intervention is often attempted at one or more institutions. This article presents the advantages and disadvantages of various study designs that might be considered for such institution‐based research, looking at the issues of comparison groups, replication, blocking, and contamination. Three examples illustrate the importance of accommodating the lack of independence or clustering that occurs among subjects within an institution, and show how these accommodations for clustering can sometimes be made by applying basic statistical techniques to institution‐level outcomes, whereas at other times more sophisticated approaches are necessary.
Improving Care to Patients with Ischemic Heart Disease: Experiences in a Single Network of the Veterans Health Administration
Authors: Pineros Sandra L. , Sales Anne E. , Li Yu‐Fang , Sharp Nancy D.
Published: August 2004

Background: Ischemic heart disease (IHD) is the leading cause of death in the United States. Lowering serum cholesterol levels reduces coronary events and mortality; this effect is most evident in patients with preexisting IHD.

Aims: The primary aim of this article is to describe a set of interventions that were piloted in a single, regional Veterans Integrated Service Network (VISN) to promote secondary prevention among patients with IHD and to explore the effect of those interventions on patient outcomes.

Methods: An observational, before‐and‐after study of clinical interventions to improve lipid guideline compliance in VISN 20 (the Veterans Administration Northwest Network) was conducted. A total of 2,467 patients with established coronary artery disease from three medical facilities in VISN 20 were included. Each medical facility chose different interventions to lower low‐density lipoprotein cholesterol (LDL‐c) levels in their patients. One facility chose a paper point‐of‐care reminder, a second chose a lipid clinic, and a third chose audit/feedback to clinicians in addition to a patient‐education component. Data came from a relational database that mirrors the clinical information system at each site. Outcomes included the proportion of patients who had their LDL‐c measured, the proportion of patients who had lipid‐lowering agents prescribed, and the proportion of patients at LDL‐c goal of lower than 100 mg/dL measured before, during, and after the intervention period.

Results: Statistically significant improvements were observed within sites after the interventions were implemented.

Implications for Practice: Interventions that focused on secondary prevention in this high‐risk group were moderately successful in changing practice. Tailoring interventions to the needs of a specific site of care is feasible and may add to the likelihood of succeeding.

Conclusion: Overall, the three facilities improved in lipid measurement and management for patients with coronary artery disease.
Advancing Quality of Urinary Incontinence Evaluation and Treatment in Nursing Homes Through Translational Research
Authors: Watson Nancy M.
Published: August 2004

Aim: This article presents the development of ongoing research to improve the evaluation and treatment of urinary incontinence (UI) in nursing homes through “A Model for Use of the Urinary Incontinence Guideline in US Nursing Homes” under the initiative Translating Research into Practice (TRIP II).

Background: The previous research provided the definition of the clinical practices needing improvement, the relative size of the population needing care, and an estimate of the manpower required. The model proposed nurse practitioners (NPs) as consultants to nursing homes to perform the basic incontinence evaluation and treatment, thereby (1) improving incontinence evaluation and treatment, (2) reducing UI, (3) reducing complications, and (4) increasing cost savings attributable to UI.

Approach: A quasi‐experimental research design with pre‐ and post‐comparison was selected utilizing four control and four experimental NP caseloads followed for 16 weeks post‐onset (n = 320).

Findings: Findings are not yet available from this study, which is ongoing.

Discussion: The real‐world challenges presented by this translational research and their solutions are discussed.
Translation Research: Where Do We Go from Here?
Authors: Fraser Irene
Published: August 2004

Aim: Where do we go from here? This article draws on other articles in this supplement, the dialogue from the meeting that generated it, and other sources to identify steps to advance translation research, and in particular to achieve broader translation and use of evidence.

Implications: To move from the growing accumulation of individual successes in translation to broader scale translation and implementation of evidence, those who use research and those who do research will need to do four things: (1) increase synergy and synchronization across studies, through cross‐disease studies, adoption of a common and precise language to describe interventions, addressing issues of customization of interventions, and finding commonality in quality and outcomes measures; (2) take account of organizational factors that can shape the impact of interventions, and also move to the implementation of organizational evidence; (3) address environmental factors such as reimbursement and market competition; and (4) take findings to a larger scale through national demonstrations, efforts of national agents of change, challenge or partnership grants, or use of provider‐based networks.
An Artificial Myocardium Assist System: Electrohydraulic Ventricular Actuation Improves Myocardial Tissue Perfusion in Goats
Authors: Wang Qingtian , Yambe Tomoyuki , Shiraishi Yasuyuki , Duan Xudong , Nitta Shinichi , Tabayashi Kouichi , Umezu Mitsuo
Published: August 2004

Abstract:  Artificial hearts and ventricular assist devices have been widely used clinically to assist patients with severe heart failure. Unfortunately, direct contact between the device and the patient's blood leads to thromboembolic events, and then the need for anticoagulation and infections contribute significantly to complication and mortality. Compressing the dysfunctional heart from its epicardial surface, a nonblood‐contacting method of direct mechanical ventricular actuation could provide ventricular support, pulsatile blood flow, and avoid interactions between blood and the surface of the artificial assistance system. An ElectroHydraulic Artificial Myocardial (EHAM) assist system that might assist heart muscle contraction has been developed. The purpose of this study is to determine the efficiency of the EHAM system in perfusing myocardial tissue in an acute animal experiment.

Method:  Healthy adult goats (n = 8) were used in acute animal experiments. A left lateral thoracotomy was done and the chest was opened through the 4th and 5th rib resection. Hemodynamic parameters were continuously monitored including ECG, aortic blood pressure, left ventricular pressure, and pulmonary artery pressure. Myocardial tissue perfusion was measured by using an Omega flow laser fiber attached on the surface of the heart.

Results:  All the animals achieved significantly increased blood pressure, pulmonary artery flow, and myocardial tissue perfusion during the EHAM compression compared with the nondriving (pre‐assisted) mode.

Conclusions:  The EHAM system can effectively improve myocardial tissue perfusion and increase blood pressure thus demonstrating a potential for treating failing cardiac performance.
Experience with Expanded Polytetrafluoroethylene (ePTFE Gore‐Tex) Surgical Membrane for Coronary Artery Grafting: Does ePTFE Surgical Membrane Predispose to Postoperative Mediastinitis?
Authors: Kajiwara Hirokazu , Hamada Toshiyuki , Ichikawa Yukio , Ishi Masanori , Yamazaki Ichiya
Published: August 2004

Abstract:  The advantage of completely closing the pericardium after a coronary artery bypass grafting is the avoidance of injury of the heart and grafts during a re‐operation. However, it would obviously be counterproductive to close the pericardium with a substitute that is predisposed to infection. This study was designed to evaluate the safety of ePTFE surgical membrane in comparison to native pericardium or autologous tissue. Between January 1992 to March 2003, 695 coronary artery bypass graftings were performed. The hearts and grafts were covered with ePTFE surgical membrane (474 cases: ePTFE group), or autologous pericardium and/or other autologous tissue (221 cases: non‐ePTFE group). Often, a bilateral dissection of the internal thoracic artery was performed, which lengthened the surgery, the cardiopulmonary bypass, and the aortic clamp, in the ePTFE group. But there was no difference between the ePTFE group (2.1%) and the non‐ePTFE group (3.2%) in the development of postoperative mediastinitis. There was also no difference between the two groups in the organism type of the infection. Methicillin resistant Staphylococcus aureus (MRSA) is the most common organism cultured from sternal wound infections; there were five cases in the ePTFE group, and four cases in the non‐ePTFE group. In the ePTFE group, the hospital mortality due to postoperative mediastinitis was zero, and there was also no significant difference between the ePTFE group and the non‐ePTFE group in time from the drainage operation to discharge; 74.3 days in the ePTFE group, and 81.0 days in the non‐ePTFE group. The clinical use of ePTFE surgical membrane for a coronary artery bypass grafting does not appear to be a risk factor for mediasitinitis.
Artificial Oxygen Carriers as Red Blood Cell Substitutes: A Selected Review and Current Status
Authors: Kim Hae Won , Greenburg A. Gerson
Published: August 2004

Abstract:  Two distinct approaches are being explored in red blood cell substitute (RCS) development: hemoglobin‐based oxygen carriers (HBOCs) and perfluorocarbon‐based oxygen carriers (PFBOCs). HBOCs are based on intra‐ and/or intermolecularly “engineered” human or animal hemoglobins (Hbs), optimized for O2 delivery and longer intravascular circulation. Some are currently being evaluated in Phase II/III clinical studies. PFBOCs are aqueous emulsions of perfluorocarbon derivatives that dissolve relatively large amounts of O2. A PFBOC based on a 60% (wt/vol) emulsion of perfluorooctyl bromide has been evaluated in Phase II/III clinical trials. Although current PFBOC products generally require patients to breathe O2 enriched air, they render certain advantages since they are totally synthetic. This article provides a short review of the basic principles, approaches, and current status of RCS development. Results of preclinical and clinical studies including recent Phase II/III clinical studies are discussed.
Is There a Role for Blood Substitutes in Civilian Medicine: A Drug for Emergency Shock Cases?
Authors: Nosé Yukihiko
Published: August 2004

Abstract:  The oxygen carried inside plasma performs differently than the oxygen carried inside red cells. Only 0.13–0.3 mL of oxygen in 100 mL of blood is available inside plasma while 14–19 mL of oxygen is carried inside red cells. Thus, less than 5–8 mL of oxygen is available in the plasma of the entire body. When a patient develops hypovolemic shock, red cells are bypassed and are not perfused directly inside the tissues. However, plasma should reach such hypoxic tissues. Thus, an infusion of oxygen‐carrying macromolecules in plasma with a hemoglobin concentration of only 6% and P50 value of 24 mm Hg should be therapeutically effective even if less than 100 mL of stabilized hemoglobin solution (conjugated hemoglobin of 90 000 Da with a molecular size of less than 10 nm or 0.01 µm) are infused under shock conditions. The basic physiology of oxygen‐carrying macromolecules is described in detail, which is different from the oxygen carried inside the red cells and inside encapsulated oxygen‐carrying particles (typically 250 nm or 0.25 µm). Thus, the oxygen‐carrying macromolecues are extremely effective in the treatment of shock patients. In emergency cases, after the bleeding is controlled, a small infusion volume of oxygen‐carrying macromolecules will supply sufficient oxygen to the hypoxic tissues and immediately improve the blood pressure of shock patients.