bluuebunny/crossref_metadata_2025 huggingface dataset:
A comparative study of sialyloligosaccharides isolated from sialidosis and galactosialidosis urine
Authors: van Pelt J. , Kamerling J. P. , Bakker H. D. , Vliegenthart J. F. G.
Published: June 2005

Sialic acid‐containing carbohydrates were isolated from sialidosis urine by a combination of gel‐filtration on Bio‐Gel P‐6 and medium‐pressure anion‐exchange chromatography on Mono Q. The Mono Q fractions were subjected to 500‐MHz1H‐NMR spectroscopy, sugar analysis and analytical HPLC on Lichrosorb‐NH2. These methods indicated the presence of variousN‐acetyllactosamine type sialyloligosaccharides differing from each other in branching pattern and sialic acid linkage types. Among the structures were fully and partially sialylated mono‐, di‐, tri‐ and tetra‐antennary compounds. A comparison with the results from galactosialidosis urine indicated that essentially the same carbohydrates were present in both urines, but that the relative amounts of the various sialyloligosaccharides differ to some extent. Sialidosis urinary oligosaccharides contained relatively more α2–6 linked sialic acid than oligosaccharides from galactosialidosis urine. It could be concluded that the additionalβ‐galactosidase deficiency in galactosialidosis did not influence the nature of the excreted material and that the sialidase deficiency determined completely the defective catabolism of glycoproteins in both sialidosis and galactosialidosis.
Phosphorylaseb kinase deficiency in glycogenosis type VIII: Differentiation of different phenotypes and heterozygotes by erythrocyte enzyme assay
Authors: Besley G. T. N.
Published: June 2005

Erythrocytes provide a useful sample in which to study phosphorylaseb kinase defects in families affected with GSD VIII.
Biochemical characterization of patients and prenatal diagnosis of sialic acid storage disease for three families
Authors: Clements P. R. , Taylor J. A. , Hopwood J. J.
Published: June 2005

Modifications of the assay method of Aminoff (1961) for the determination of sialic acid levels in urine, amniotic fluid, cultured cell homogenates and tissue homogenates, which reduce the interference from proteins by precipitation and from interfering chromogens by second derivative spectroscopy are described.

Biochemical profiles of patients from three families with different clinical forms of sialic acid storage disease were made using data obtained with the new method. A family with two patients with the clinically severe, early‐onset form is described, in which a 9‐fold elevation of sialic acid can be detected in the skin fibroblasts and a 12‐fold elevation in the urine. A patient from the second family is described with very severe clinical features and with 160‐fold and 16‐fold elevations of sialic acid in the urine and skin fibroblasts respectively. A patient from a third family is described with mild clinical features but with a 160‐fold and 6‐fold elevation of sialic acid in urine and skin fibroblasts respectively. The data obtained in this study are compared with data obtained in other laboratories on other patients.

The method was used to assess the levels of sialic acid present in amniotic cells and chorionic villus cells obtained prenatally from pregnancies in each of the three families. In one case, in which amniotic cells were elevated in sialic acid and were vacuolated, the pregnancy was terminated. Follow‐up studies confirmed the diagnosis. Pregnancies from the other two families were assessed to be not affected.
A sibship with a mild variant of Zellweger syndrome
Authors: Barth P. G. , Schutgens R. B. H. , Wanders R. J. A. , Heymans H. S. A. , Moser A. E. , Moser H. W. , Bleeker‐Wagemakers E. M. , Jansonius‐Schultheiss K. , Derix M. , Nelck G. F.
Published: June 2005

A mild variant of Zellweger (cerebro‐hepato‐renal) syndrome was diagnosed in male and female siblings aged 7 and 2 years. They had mild facial dysmorphia, moderate psychomotor retardation, tapetoretinal degeneration, sensorineural deafness and hepatomegaly. Ultrastructural examination of a liver biopsy in the younger patient revealed the absence of recognizable peroxisomes. In both patients plasma levels of pipecolic acid, phytanic acid, trihydroxycoprostanoic acid and dihydroxycoprostanoic acid were elevated. The very long chain fatty acid C26:0 and the C26:0/C22:0 fatty acid ratio were elevated in plasma, but less than in classical Zellweger syndrome. In cultured fibroblasts, deficient acyl‐CoA:dihydroxyacetone phosphate acyltransferase and increased concentrations of C26:0 as well as C26:1 very long chain fatty acids were found within the ranges previously established for patients with classical Zellweger syndrome. Particle‐bound catalase was absent in fibroblasts. Despite the relatively mild clinical expression the biochemical abnormalities found in these patients are the result of a general peroxisomal dysfunction similar to the changes in classical Zellweger syndrome.
Review: Normal and abnormal central nervous system GABA metabolism in childhood
Authors: Jaeken J. , Casaer P. , Haegele K. D. , Schechter P. J.
Published: June 2005

The metabolism and function of central nervous system GABA is briefly reviewed. Hereditary disorders of the GABA metabolism presenting in childhood are discussed with particular emphasis on the recently identified succinic semialdehyde dehydrogenase deficiency and GABA‐transaminase deficiency, and on diseases associated with low CSF GABA which await further unravelling. Low CSF GABA concentrations are not always associated with convulsions. A separate section is devoted to the CSF as a tool in the diagnosis of these disorders. Finally, we present a few diagnostic and therapeutic guidelines.
Variation of lysosomal enzyme activity with gestational age in chorionic villi
Authors: Fukuda M. , Tanaka A. , Isshiki G.
Published: June 2005

The activities of 14 lysosomal enzymes in chorionic villi at gestational ages of 6–12 weeks were assayed.

Arylsulphatases A and B, α‐glucosidase and β‐glucuronidase activities increased with advancing gestational age. When compared with the activity in cultured amniotic fluid cells, arylsulphatase A, β‐galactosidase, α‐glucosidase, heparan N‐sulphatase, α‐l‐iduronidase, α‐mannosidase, neuraminidase and sphingomyelinase showed significant differences. All except β‐glucuronidase showed lower activity in chorionic villi than in cultured amniotic fluid cells. Prenatal diagnosis using chorionic villi was possible except for α‐l‐iduronidase.

Storage at −20°C up to 42 days did not significantly affect activity. The results emphasize the importance of using fresh or frozen age‐matched control tissue for diagnosis.
Trace element excess in PKU diets?
Authors: Sievers E. , Oldigs H. ‐D. , Dörner K. , Schaub J.
Published: June 2005

Knowledge of trace element requirements of infants with phenylketonuria (PKU) fed a semisynthetic diet is limited. Three infants with PKU detected early were studied longitudinally in classical balance studies for 72 h, under domestic conditions, at the ages of 2, 5, 8, 12 and 16 weeks. Iron, copper and manganese concentrations in the diet and faeces were determined by atomic absorption spectroscopy. The median concentrations in the diet (4.8 mg Fe/L, 1.7 mg Cu/L, 0.43 mg Mn/L) exceed those in human milk. This is mainly due to supplementation of the amino acid preparation used. The increased intake led to a significantly higher daily retention of Cu and Mn from the PKU‐diet fed, with a median of 0.17 mg Cu/kg and 6.4 µg Mn/kg body weight; the median retention of Fe was 0.24 mg Fe/kg. Our results confirmed the doubts about the suitability of the present trace element supplementation in formula for infants with PKU during the first four months of life.
Long‐term effects of bone marrow transplantation on lysosomal enzyme replacement in β‐glucuronidase‐deficient mice
Authors: Bou‐Gharios G. , Adams G. , Pace P. , Olsen I.
Published: June 2005

This study uses bone marrow transplantation (BMT) between congenic strains of mice as an experimental model to examine enzyme replacement therapy of lysosomal storage diseases. Bone marrow cells from donor mice which have normal levels of the lysosomal enzyme β‐glucuronidase (Gus), which is heat‐stable, rapidly repopulated the haematopoietic compartment of irradiated recipient mice which have only low levels of a thermolabile form of this enzyme. Gus activity was found to increase progressively in the tissues of the recipients, including the liver, heart and skeletal muscle. Elevated levels were also observed in the kidney and brain. The increase in enzyme activity in the host tissues was not due to the presence of contaminating blood cells, but rather to the acquisition of new, heat‐stable enzyme from the donor bone marrow cells. High levels of Gus activity persisted for at least 72 weeks, showing the potential therapeutic value of BMT for enzyme deficiency diseases.
Bone marrow transplantation for Sanfilippo disease type B
Authors: Vellodi A. , Young E. , New M. , Pot‐Mees C. , Hugh‐Jones K.
Published: June 2005

Allogeneic bone marrow transplantation was performed on twins with Sanfilippo B disease. They were the first two patients with this disorder to undergo the procedure. There was definite evidence of engraftment as shown by conversion to donor blood group antigen and tissue type, and increased leukocyte α‐glucosaminidase activity. Nine years post transplant, neither twin is as handicapped as her untreated brothers were at the same age, although in one twin hyperactivity and behavioural problems, characteristic of the disorder, are present. Details of the twins' intellectual development and growth, their α‐glucosaminidase activity and urinary glycosaminoglycan excretion are reported.
Zellweger syndrome in a preterm, small for gestational age infant
Authors: Samsom J. F. , Jakobs C. , van de Klei‐van Moorsel J. , Smit L. M. E. , Schutgens R. B. H. , Wanders R. J. A.
Published: June 2005

A preterm (gestational age 34 weeks), small for gestational age infant (birth weight <P2,3) is described. Because of unexplained slightly disturbed liver function tests at age 2 months, extensive metabolic examinations were performed. Elevated blood levels of very long‐chain fatty acids, pipecolic acid and abnormal levels of bile acid intermediates were detected, suggesting a peroxisomal disorder. The plasmalogen content of erythrocytes was decreased. Morphologically distinct peroxisomes were absent in the liver. In fibroblasts an accumulation of very long‐chain fatty acids, decreased activity of acyl‐CoA: dihydroxyacetone phosphate acyltransferase and impairedde novo biosynthesis of plasmalogens was found.

In summary, a mild variant of the classical cerebro‐hepato‐renal syndrome of Zellweger was found without the characteristic clinical facial signs.
Serotonin and noradrenaline concentrations and serotonin uptake in platelets from hyperphenylalaninaemic patients
Authors: Giovannini M. , Valsasina R. , Longhi R. , Cesura A. M. , Galva M. D. , Riva E. , Bondiolotti G. P. , Picotti G. B.
Published: June 2005

In three untreated patients with phenylketonuria (PKU), three PKU and six hyperphenylalaninaemic (HPA) patients in good metabolic control, the kinetic constants of plateletin vitro uptake of [14C]serotonin (5HT) did not significantly differ from those in 12 control subjects matched for age. The platelet concentrations of endogenous 5HT and noradrenaline (NA), taken as long‐term indices of the amount of these amines circulating in plasma, were lower than normal in PKU and HPA patients, whether or not they were kept on a diet. However, a reduction in plasma NA concentrations at the moment of blood collection was seen only in untreated PKU patients. These data indicate that there may be a chronic inhibition of 5HT and possibly of NA synthesis even in PKU or HPA subjects in good metabolic control, with normal psychomotor development and only slightly raised plasma phenylalanine levels.
The importance of recognizing secondary carnitine deficiency in organic acidaemias: Case report in glutaric acidaemia type II
Authors: Mandel H. , Africk D. , Blitzer M. , Shapira E.
Published: June 2005

Secondary carnitine deficiency in a patient with glutaric acidaemia type II, due to deficient ETF‐dehydrogenase activity, is described. The patient responded clinically to a pharmacological dose of riboflavin and a restricted protein diet. In the second year of her life she developed more frequent and severe exacerbations during intercurrent infections from which she did not fully recover. Hypotonia and marked ataxia persisted. Plasma carnitine was entirely complexed as acylcarnitine with no free carnitine detected. Retrospective evaluation of several frozen urine specimens obtained since the age of 10 months revealed undetectable free carnitine with elevated acylcarnitine levels. Marked clinical improvement was observed followingL‐carnitine supplementation. The hypotonia and ataxia disappeared. The frequency and the severity of the exacerbations were noticeably decreased. The role ofL‐carnitine in preventing the accumulation of acyl‐CoA compounds in inborn errors of organic acid metabolism is further emphasized by this patient. The necessity to evaluate free carnitine, acylcarnitine and acyl/free ratio in the assessment, follow‐up and management of patients with inborn errors of organic acid metabolism is discussed.
Liver pathology and immunocytochemistry in congenital peroxisomal diseases: a review
Authors: Roels F. , Espeel M. , De Craemer D.
Published: June 2005

Diagnostic and pathogenetic investigations of peroxisomal disorders should include the study of the macroscopic and microscopic pathology of the liver, in addition to careful clinical observations, skeletal X‐ray and brain CT scan, assays of very long‐chain fatty acids and bile acid intermediates, and selected enzyme activities. This review of the literature also contains novel observations about the following syndromes: cerebro‐hepato‐renal (Zellweger) syndrome, X‐linked and neonatal adrenoleukodystrophies (ALD, NALD), NALD‐like syndromes, infantile phytanic acid storage, classical Refsum disease, rhizomelic and other forms of chondrodysplasia punctata (XD, XR, AR), hyperpipecolic acidaemia, primary hyperoxaluria I, pseudo‐Zellweger and Zellweger‐like syndromes, and single enzyme deficiencies. Microscopic data include catalase staining and morphometry of peroxisomes, immunolocalization ofβ‐oxidation enzymes, detection of trilamellar, polarizing inclusions in PAS‐positive macrophages, fibrosis and iron storage. Peroxisomal enlargement appears to be related to functional deficit inβ‐oxidation disorders as well as in rhizomelic chondrodysplasia punctata. Because normal peroxisomal localization of activeβ‐oxidation enzymes can accompany a C26β‐oxidation deficit, other mechanisms such as impaired transport of metabolites should be investigated. ‘Ghost’‐like organelles are shown in the liver of an infantile Refsum patient and in an NALD‐like case; immuno‐gold labelling of membrane proteins did not reveal ghosts in Zellweger livers.
Apparent absence of glycogen branching enzyme activity in phosphofructokinase deficiency
Authors: Barash V. , Lilling S. , Fischer R. , Argov Z.
Published: June 2005

A 30‐year‐old woman with clinical features and biochemical findings of muscle phosphofructokinase deficiency was found to have a very low level of α‐1,4‐glucan:α‐1,4‐glucan‐6‐transglucosylase (branching enzyme, EC 2.4.1.18) activity in muscle. In contrast, branching enzyme activity in the leukocytes was in the range of control values. After sedimentation of the glycogen from muscle homogenates by centrifugation at 105 000 g, branching enzyme activity in muscle of the patient was similar to that of control subjects. This patient illustrates the possibility of falsely diagnosing branching enzyme deficiency when muscle glycogen content is elevated. It is likely that such an artefact may also cause a false positive diagnosis of branching enzyme deficiency in other metabolic diseases associated with glycogen accumulation.
The use of recombinant DNA techniques for the diagnosis of familial hypercholesterolaemia
Authors: Humphries S. , Taylor R. , Jeenah M. , Seed M.
Published: June 2005

In the UK, about 5% of patients with familial hypercholesterolaemia have a detectable deletion or rearrangement of part of the LDL‐receptor gene. This results in the detection of shorter or abnormal sized fragments of the LDL‐receptor gene in a Southern blot hybridization. This can be used to follow the inheritance of the defective gene, and for diagnosis in the families of these individuals. In the families of the rest of the patients, diagnosis may be possible using linked restriction fragment length polymorphisms (RFLPs) detected with the LDL‐receptor probe. There are now ten common RFLPs of the LDL‐receptor gene, with variable sites in the 3′ half of the gene. Over 80% of patients are heterozygous for at least one of these RFLPs, and therefore potentially informative for DNA diagnosis. For a foetus at risk of homozygous familial hypercholesterolaemia, antenatal diagnosis may also be possible using these methods. However, family studies require samples to be available from affected or unaffected relatives of the patient, and this limits the applicability of the tests. For some mutations, the base pair change causing the defect in the LDL‐receptor itself creates or destroys a site for a restriction enzyme. Such ‘mutation‐specific’ RFLPs could be used for population screening, but so far have only been reported for the familial hypercholesterolaemia mutation that is common in Lebanon. In the future it may be possible to develop mutation‐specific oligonucleotide probes for the diagnosis of familial hypercholesterolaemia. These would be appropriate for population screening or screening patients with hyperlipidaemia. This information may be useful if different mutations require different therapeutic strategies.
Thyroid structure and function in bovine β‐mannosidosis
Authors: Lovell K. L. , Jones M. Z. , Patterson J. , Abbitt B. , Castenson P.
Published: June 2005

In bovine β‐mannosidosis, the thyroid in the affected newborn shows marked cytoplasmic vacuolation. There is an associated reduction in the serum concentrations of thyroxine and tri‐iodothyronine.
Mild form of methylmalonic aciduria misdiagnosed as propionic acidaemia during a ketotic crisis
Authors: Tavares de Almeida I. , Duran M. , Silva M. F. B. , Portela R. , Cabral A. , Tasso T. , Eusébio F. , Silveira C.
Published: June 2005

A patient with methylmalonic acidaemia was initially misdiagnosed on the basis of an organic acid profile as having propionic acidaemia during a period of ketosis
Vitamins: An evolutionary perspective
Authors: Scriver C. R.
Published: June 2005

Access to vitamins and the genetic endowment to utilize them maintain vitamin‐dependent metabolic homeostasis in heterotrophs. Whereas the extent of adverse nutritional experiences has declined in modern human societies, phenocopies of deficiency diseases persist; accordingly, they have high heritability. The “vitamin‐responsive hereditary metabolic diseases” identify DNA that specifies conserved apoenzyme domains interacting with coenzyme and the cellular processes providing access to coenzymes. Could heterozygosity at those loci also be a determinant of disease (or health) under certain circumstances?
Prospective ascertainment of complete and partial serum biotinidase deficiency in the newborn
Authors: Dunkel G. , Scriver C. R. , Clow C. L. , Melançon S. , Lemieux B. , Grenier A. , Laberge C.
Published: June 2005

We screened 163 000 newborn filter‐paper blood samples for serum biotinidase deficiency (McKusick 25326) and found 15 probands: three had complete deficiency (incidence 18.4 cases per million live births, 95% confidence interval 4–54 cases per million); the others had partial deficiency. The positive predictive value of the test for either form of biotinidase deficiency was 9.86%. We found seasonal variation in biotinidase activity in filter‐paper blood samples. The cost per test was Can .27 (1987 dollar value) and per case of complete deficiency ascertained, $15 500. Family studies indicated that complete serum biotinidase deficiency is a homozygous phenotype and partial deficiency is the heterozygous form. Homozygous cases were treated with biotin and have shown no clinical manifestations (55 patient‐months of observation). None of the heterozygotes (n=42, age 3 months – 62 years) has clinical manifestations. The number of heterozygotes found by screening was much less than predicted probably because the screening test detects mainly the samples with very low (outlier) biotinidase activity. The variant allele(s) for biotinidase deficiency was more common in French Canadians than in other ethnic groups in Quebec; there was no evidence of regional clustering or founder effect.
Correction of sphingomyelinase deficiency in Niemann‐Pick type C fibroblasts by removal of lipoprotein fraction from culture media
Authors: Thomas G. H. , Tuck‐Muller C. M. , Miller C. S. , Reynolds L. W.
Published: June 2005

The average sphingomyelinase activity of fibroblasts obtained from 12 Niemann‐Pick type C patients was 37.9% of that of normal fibroblasts (27.2versus 72nmol(mg protein)−1h−1) when the cells were cultured in minimum essential medium containing 13% fetal bovine serum. Following replacement of the above medium with medium in which the lipoprotein fraction had been removed from the fetal bovine serum, the sphingomyelinase activity rose over a 7‐day period from about 1/3 of normal to normal or above. Upon reintroduction of medium containing 10% fetal bovine serum which had not been extracted, the sphingomyelinase activity of the Niemann‐Pick type C cells again fell within 48h to 30% of the normal controls. In contrast, cell lines from patients with either Niemann‐Pick Type A or B were not influenced by the presence or the absence of lipoprotein, i.e. lacked sphingomyelinase activity under all culture conditions examined. Histochemical staining with filipin showed an inverse relationship between the sphingomyelinase activity and intracellular, free, unesterified, cholesterol level. Moreover, immunochemical staining with an antibody against a lysosomal membrane protein provided direct evidence that the accumulation of unesterified cholesterol in cells cultured in regular (non‐extracted) medium occurred within lysosomes and/or related organelles.
Deoxyribose‐5‐phosphate aldolase deficiency — A harmless inborn error of metabolism
Authors: Chappel A. , Scholem R. D. , Brown G. K. , Truscott R. M. , Cotton R. G. H. , Haan E. A. , Danks D. M.
Published: June 2005

Evidence is presented that a deficiency of 2‐deoxyribose‐5‐phosphate aldolase was present in a previously described patient who excreted metabolites of 2‐deoxyribose in his urine. Minor clinical abnormalities present did not appear related to this disorder.
The iduronate sulphatase activities of cells and tissue fluids from patients with Hunter syndrome and normal controls
Authors: Dean M. F.
Published: June 2005

The substrateO‐(α‐L‐idopyranosyuronic acid‐2‐sulphate)‐(1 → 4)‐2,5‐anhydro‐D‐(3H‐1) mannitol‐6‐sulphate was used at a final concentration of 50 mmol/l to measure theα‐L‐idurono‐2‐sulphate sulphatase activities of cell extracts, serum and amniotic fluid. Activities were measured after dialysis against water, to avoid the inhibitory effect of sodium chloride and the reaction products separated by ion‐exchange chromatography on ECTEOLA cellulose. The enzyme present in normal serum had an apparentKm of 0.12 mmol/l. The mean activities of normal serum, fibroblasts and leukocytes were 0.61, 16.63 and 18.75 nmol/mg protein per hour respectively, while corresponding ‘Hunter’ tissues contained between 1% and 3% of normal activity. Cultured normal amniotic cells had a mean activity of 8.46 nmol/mg protein per hour, while those cultured from ‘Hunter’ patients contained about 6% of normal activity. Hunter disease can thus be readily diagnosed in a wide variety of tissues. Samples from three obligate heterozygotes were found to have iduronate sulphatase activities amounting to 41%, 57% and 63% of normal values.
Direct alteration of a gene in the human genome
Authors: Smithies O.
Published: June 2005

Direct alteration of a gene in the human genome requires an understanding of the role of the gene in metabolism. A gene may need to be introduced into a specific tissue or alternatively it may be possible to use accessible tissue such as bone marrow. The level of gene expression required also needs to be known as does the position in the genome into which the gene is to be inserted. Insertion of DNA needs to be of high efficiency and accuracy. Various methods are available including virus, the use of inert adjuvant, microinjection and electroporation. The procedure with the most potential for accuracy is the use of specially designed plasmids. The example of the use of such a plasmid in achieving target modification of the β‐globin gene is given. The method has high accuracy but low efficiency.
Endolymphatic potassium of the chicken vestibule during embryonic development
Authors: Masetto Sergio , Zucca Giampiero , Bottà Luisa , Valli Paolo
Published: June 2005

The endolymph fills the lumen of the inner ear membranous labyrinth. Its ionic composition is unique in vertebrates as an extracellular fluid for its high‐K+/low‐Na+ concentration. The endolymph is actively secreted by specialized cells located in the vestibular and cochlear epithelia. We have investigated the early phases of endolymph secretion by measuring the endolymphatic K+ concentration in the chicken vestibular system during pre‐hatching development. Measurements were done by inserting K+‐selective microelectrodes in chicken embryo ampullae dissected at different developmental stages from embryonic day 9 up to embryonic day 21 (day of hatching). We found that the K+ concentration is low (<10 mM/L) up to embryonic day 11, afterward it increases steeply to reach a plateau level of about 140 mM/L at embryonic day 19–21. We have developed a short‐term in vitro model of endolymph secretion by culturing vestibular ampullae dissected from embryonic day 11 chicken embryos for a few days. The preparation reproduced a double compartment system where the luminal K+ concentration increased along with the days of culturing. This model could be important for (1) investigating the development of cellular mechanisms contributing to endolymph homeostasis and (2) testing compounds that influence those mechanisms.
Immunochemical recognition of gelatinous zooplankton:
an application to identify the origin of the ‘barrel’: made
by the pelagic amphipod, Phronima sedentaria
Authors: Nishikawa Jun , Suzuki Yuzuru Suzuki , Nishida Shuhei
Published: June 2005

A first trial to recognize gelatinous zooplankton using an immunochemical method was carried out. Polyclonal antibodies (rabbit IgG) raised against three cnidarians and four pelagic tunicates were purified and tested for their specificity against various sympatric zooplankton and micronekton. While antibodies to cnidarians were species-specific or at least distinguishable by means of the patterns of the bands appearing, antibodies to the pelagic tunicates cross-reacted with the antigen from the other tunicate species although these did not cross-react with other animal taxa. These results suggest that the present cnidarian antibodies can be applied to the predator identification at the species level. Antibodies to pelagic tunicates would also be effective tools for basic scanning at higher taxonomic levels, i.e. pelagic tunicates or other animals. The antibodies were applied to the identification of the ‘barrel’ of Phronima, indicating that the ‘barrels’ originated from tunicates but not from cnidarians. The present study indicates that the immunochemical method using polyclonal antibodies is a powerful tool for the detection of gelatinous zooplankton.
John Adams ‘On the Transmigration of Souls’
Authors: Quinn Peter
Published: June 2005

JOHN ADAMS: On the Transmigration of Souls. New York Choral Artists, Brooklyn Youth Chorus, New York Philharmonic c. Lorin Maazel. Nonesuch 7559 79816 2.
Cyril Scott's orchestral music
Authors: Babcock David
Published: June 2005

CYRIL SCOTT: Symphony No. 3 The Muses; Piano Concerto No. 2; Neptune. Howard Shelley (pno), Huddersfield Choral Society, BBC Philharmonic, c. Martyn Brabbins. Chandos CHAN 10211.
Genes and environment interact to determine the fitness costs of resistance to
Bacillus thuringiensis
Authors: Raymond Ben , Sayyed Ali H , Wright Denis J
Published: June 2005

Genes which provide resistance to novel challenges such as pesticides, toxins or pathogens often impose fitness costs on individuals with a resistant phenotype. Studies of resistance to
Bacillus thuringiensis
and its insecticidal Cry toxins indicate that fitness costs may be variable and cryptic. Using two field populations (Karak and Serd4) of the diamondback moth,
Plutella xylostella
, we tested the hypothesis that the costs associated with resistance to the
B. thuringiensis
toxin Cry1Ac would be evident when insects were grown under poor environmental conditions, namely limited or poor quality resources. On a poor quality resource, a cultivar of
Brassica oleracea
var.
capitata
with varietal resistance to
P. xylostella
, only one resistant population, Karak, showed reduced fitness. Conversely, when we limited a high quality resource,
Brassica pekinensis
, by imposing larval competition, only resistant Serd4 insects had reduced survival at high larval densities. Furthermore, Cry1Ac resistance in Serd4 insects declined when reared at high larval densities while resistance at low densities fluctuated but did not decline significantly. These results confirm the hypothesis that resistance costs can appear under stressful conditions and demonstrate that the fitness cost of resistance to
Bacillus thuringiensis
can depend on the particular interaction between genes and the environment.
Cultivating knowledge sharing through the relationship management maturity model
Authors: Martin Valerie A. , Hatzakis Tally , Lycett Mark , Macredie Robert
Published: June 2005

Purpose

The purpose of this paper is to present the development of the relationship management maturity model (RMMM), the output of an initiative aimed at bridging the gap between business units and the IT organisation. It does this through improving and assessing knowledge sharing between business and IT staff in Finco, a large financial services organisation.

Design/methodology/approach

The objectives were achieved by undertaking ethnographic research with the relationship managers (RMs) as they carried out their activities, and developing the RMMM by visualizing the development of a community of practice (CoP) between business and IT.

Findings

The RMMM demonstrates a learning mechanism to bridge the business/IT gap through an interpretive approach to knowledge sharing by defining knowledge sharing processes between business and IT and defining the tasks of the relationship managers as facilitators of knowledge sharing.

Research limitations/implications

More research is necessary to determine whether the RMMM is a useful tool on which Finco can base the development of RM over the next few years.

Practical implications

The RMMM acts as a practical knowledge management tool, and will act as a future reference for the RMs as they attempt to further develop the business/IT relationship.

Originality/value

The findings provide an initial endorsement of the knowledge sharing perspective to understand the business/IT relationship. Also, the RMMM can be used to identify problematic issues and develop processes to address them.
Towards a theory of the integration of the Other in representation
Authors: Martin‐Vallas François
Published: June 2005

Abstract:  In this article1, the author tries to uncover the elements of a theoretical model which would take into account the psychic transformations necessary to facilitate the emergence of representation. Toward this end, he firstly relies on Jung's notion of the archetype and Freud's idea of hallucinatory wish fulfilment, which he reconsiders in the light of the writings of Fordham (de‐integration and re‐integration of the primary self), and of Jean Laplanche (primary seduction), and linking it to the model based on chaos theory as developed in physics. He thus concludes that under the influence of primary seduction, the archetype is able to become a veritable, strange psychic attractor, enabling the determining factor of the instinctual axis of the archetype to open up to the possibility of symbolization, a necessary underlying feature for the occurrence of subjectivity. He ends his argument with a brief clinical vignette which illustrates the effect of openness to the psychic unknown constituted by primary seduction in the transference.
Symbols and symbolization in clinical practice and in Elisabeth Marton's film My Name was Sabina Spielrein
Authors: Gibeault Alain
Published: June 2005

Abstract:  If symbolization can be defined, in a general way, as the operation of substitution by which something can represent something else for someone, the Freudian discovery of the unconscious introduced the idea of a process of work whereby the subject is differentiated from the object. As a consequence, the vicissitudes of symbolization are related to the vicissitudes of the drive.

The clinical case of a psychotic patient treated in individual psychoanalytic psychodrama shows the possibility of overcoming violence and destructivity thanks to the work of representation and symbolization of the denied and split psychic movements. Thanks to the third party function of the leader and of the analytic setting, the individual psychoanalytical psychodrama favours psychic change. Elisabeth Marton's film illustrates this issue in so far as the destructive passion between Sabina Spielrein and Jung was partly transformed into a creative work with Freud's role as a third party.
Winnicott's splitting headache: considering the gap between Jungian and object relations concepts
Authors: Morey Jeffrey Rubin
Published: June 2005

Abstract:  This paper examines some difficulties inherent in the process of integration of potentially divergent theoretical positions. It is noted that there is an increasing tendency toward integration of Jungian theory with various models from psychoanalysis and other scientific disciplines. This trend can be understood as relating to the larger post‐modern current operating in the scientific and cultural communities. As an element of the Zeitgeist, theory integration can proceed with a lack of critical stance on the process itself. An argument is developed on theoretical grounds for urging caution in the implementation of theory integration. The integrative process is examined using dream material from D. W. Winnicott in conjunction with his review of Memories, Dreams, Reflections. Through this discussion, some implications are examined as to the viability of linking Jungian and developmental models.
What does the child analyst bring to Jungian thought?
Authors: Allain‐Dupré Brigitte
Published: June 2005

Abstract:  The author wants to show the influence that the historical acknowledgement of child therapy at the Jerusalem IAAP Congress in 1983 has had today on the Jungian world, especially on the clinical approach to their patients by analysts working only with adult patients. If her conclusions do not allow her to dissociate the strong influence on psychoanalysis of contemporary research on attachment theory and mother‐child relationship from a specific Jungian child therapists’ perspective, she points out, through three examples from Jungian literature, how the need for a metapsychology of development and the study of primary and personal aspects of the patient's life are explicit in the work and research of analysts working with adults.
Sandplay in Jungian analysis: matter and symbolic integration1
Authors: Castellana Franco , Donfrancesco Antonietta
Published: June 2005

Abstract:  Jung described a way of thinking, tied to sensations and feelings, that is a thought connected to the body. Again in The Archetypes and the Collective Unconscious, Jung (1954) speaks of the symbol as a ‘live body’, corpus et anima. If the mind cannot credit itself with the discomfort that manifests through the body, this is a sign of a separation between mind and body. A consequence of all this is the literalization of the discomfort which makes it impossible to have a symbolic dimension.

Therapy activates a process in which the patient can move from a stage of separation to a possible connection between mind and body, resulting in changes in the level of communication and of awareness. The mind opens itself to symbolization and the body becomes a field for a common language. From our reflections we have come to ascertain that we can speak of analysis only when an imaginary space is achieved, an intermediate space between the patient and the analyst, a space that is created from the intertwining of the symbolic capability of the patient with that of the analyst.

Focusing our attention on the use of sandplay in analytic therapy, we know that one puts in the sand box objects that are marks of our psyche, visible traits that contain actions, corporeal movements and feelings. When one focuses on the overall representation built, one can go beyond the literal image and the analyst, keeping alive the image through his symbolic capability, opens the possibility of a dialogue with the symbolic dimension. The imagination as a symbolic thought is the humus that allows the analyst to maintain the intermediate symbolic level open by activating a symbolic process within the dialectic of the analysis.
Reverie: between thought and prayer
Authors: Mathew Marilyn
Published: June 2005

Abstract:  Taking inspiration from a quotation by Lao Tzu, this paper considers ‘reverie’ as a dynamic process of containment as well as a state of mind. Reverie as a quality of thought akin to prayer is explored in the relationship between self and other. In infant observation, the paper contemplates the development of a baby's capacity to reach beyond the personal world to an archetypal experience of being held, where the creation of an internal space in mind could indicate the beginnings of spiritual awareness. ‘Maternal/analytic reverie’ views the passage of unconscious communication between patient and analyst as the result of a ‘window’ opening in the meniscus of the analyst's self. ‘Paternal/supervisory reverie’ reflects on the impact on a patient of an extra containing mind of a supervisor. ‘Archetypal reverie’ considers the transforming impact of numinous dreams on the psyche, and reflects on the power of prayer. Drawing on clinical examples from these four areas, the aim is to demonstrate the usefulness of the ‘void within’ and the perceptive function of emptiness in the ‘doors and windows’ of our mind.
LONG‐TERM EFFECTS OF UNPAID OVERTIME
Authors: Pannenberg Markus
Published: June 2005

Why do people work unpaid overtime? Using data from the German Socio‐Economic Panel, we show that remarkable long‐term labour earnings gains are associated with unpaid overtime in West Germany. A descriptive analysis suggests that over a 10‐year period workers with unpaid overtime experience on average at least a 10 percentage points higher increase in real labour earnings than their co‐workers. Applying panel data models this result generally holds. Furthermore, we find some evidence for gender specific differences with respect to the effects of unpaid overtime worked. Our results point to the importance of investment in current working hours beyond the standard work week to enhance real earnings prospects.
GENETIC ENDOWMENTS, PARENTAL AND CHILD HEALTH IN RURAL ETHIOPIA
Authors: Kebede Bereket
Published: June 2005

The determinants of long‐term child health in Ethiopia – as measured by height‐for‐age z‐scores – are examined controlling for community, household and individual level heterogeneity. The influence of parental health and the role of genetics are analysed. The height of parents is highly significant but no significant correlation with per capita expenditures is found. Food prices, birth order, sex and age of children, number of siblings of the mother, years of marriage and altitude are important determinants. Deprivations in later years are more important than during pre‐ or neo‐natal periods. Genetic inheritance seems to explain the correlations between child and parental health.
THE MISSING LINK BETWEEN INFLATION UNCERTAINTY AND INTEREST RATES
Authors: Berument Hakan , Kilinc Zubeyir , Ozlale Umit
Published: June 2005

In the literature, there is no consensus about the direction of the effects of inflation uncertainty on interest rates. This paper states that such a result may stem from differentiation in the sources of the uncertainties and analyzes the effects of different types of inflation uncertainties on a set of interest rates for the UK within an interest rate rule framework. Three types of inflation uncertainties – impulse uncertainty, structural uncertainty and steady‐state uncertainty – are derived by using a time‐varying parameter model with a Generalized Autoregressive Conditional Heteroskedasticity specification. It is shown that the impulse uncertainty is positively and the structural uncertainty is negatively correlated with the interest rates. Moreover, these two uncertainties are important to explain short‐term interest rates for the period of inflation targeting era. However, this time, the impulse uncertainty is negatively and the structural uncertainty is positively correlated with the overnight interbank interest rates, which is consistent with the general characteristic of the inflation targeting regimes. Lastly, the evidence concerning the effect of the steady‐state inflation uncertainty on interest rates is not conclusive.
SHOULD CENTRAL BANKS CARE ABOUT REGIONAL IMBALANCES?
Authors: Røisland Øistein
Published: June 2005

Regional imbalances may arise both as a result of asymmetric shocks and of divergent responses to symmetric shocks, such as monetary policy shocks. This paper analyses flexible inflation targeting when regional imbalances are included in the loss function. By adding regional imbalances, the time‐inconsistency problem in monetary policy becomes more complex. The paper analyses alternative institutional arrangements designed to improve the discretionary equilibrium. Even in the absence of an over‐ambitious output target, it is shown that the outcome of monetary policy is improved if the central bank places less weight on regional imbalances than the political authorities.
ARMS EXPORT CONTROLS, SUBSIDIES AND THE WTO EXEMPTION
Authors: Del Carmen García‐Alonso María , Levine Paul
Published: June 2005

Owing to the World Trade Organization (WTO) exemption that allows governments to subsidize arms exports, the arms trade is one of the few remaining areas of trade where we observe export subsidies. This paper examines the effect of arms controls, in the form of licensing delays, on the incentives to subsidize arms exports and conversely the effect of the WTO arms trade exemption on the incentives to break arms control agreements. Our main result is that arms controls and free trade commitments re‐enforce each other. Licensing delays reduce the incentive to subsidise and free trade without subsidies reduces the benefits of a unilateral abrogation of arms controls. Transparency actually worsens the Nash inefficiencies at play in that incomplete information leads to lower subsidies and lower arms exports.
Efficacy of famotidine and omeprazole in healing symptoms of non‐erosive gastro‐oesophageal reflux disease: randomized‐controlled study of gastro‐oesophageal reflux disease
Authors: Wada T. , Sasaki M. , Kataoka H. , Tanida S. , Itoh K. , Ogasawara N. , Oshima T. , Togawa S. , Kubota E. , Yamada T. , Mori Y. , Fujita F. , Ohara H. , Nakao H. , Sobue S. , Joh T. , Itoh M.
Published: June 2005

Background : The epidemiology and pathophysiology of non‐erosive gastro‐oesophageal reflux disease differs from erosive gastro‐oesophageal reflux disease. There is a possibility that non‐erosive gastro‐oesophageal reflux disease treatment requires a different regimen/approach but it is not yet acknowledged.

Aim : To investigate the efficacy of famotidine and omeprazole in the treatment of gastro‐oesophageal reflux disease, especially non‐erosive gastro‐oesophageal reflux disease.

Patients and methods : A randomized, open‐label trial was conducted. Fifty‐four gastro‐oesophageal reflux disease patients were assigned to treatment with famotidine at a dosage of 20 mg twice daily; or omeprazole, 20 mg once daily, for a period of 8 weeks. The Short Form‐36 Health Survey and Gastrointestinal Symptom Rating Scale administered at baseline and after 8 weeks of treatment as well as a symptom questionnaire were conducted daily.

Results : Short Form‐36 revealed that gastro‐oesophageal reflux disease has severe impact on health‐related quality of life. Thirty‐nine subjects (77%) were endoscopically diagnosed as non‐erosive gastro‐oesophageal reflux disease. The mean Gastrointestinal Symptom Rating Scale abdominal pain, and indigestion score of non‐erosive gastro‐oesophageal reflux disease significantly improved in famotidine‐treated patients (P < 0.05), but not in the omeprazole. There was no significant change regarding improved heartburn symptoms of non‐erosive gastro‐oesophageal reflux disease between treatments in the daytime or night‐time.

Conclusion : Famotidine and omeprazole were both effective in improving symptoms of gastro‐oesophageal reflux disease, particularly non‐erosive gastro‐oesophageal reflux disease.
Famotidine vs. omeprazole: a prospective randomized multicentre trial to determine efficacy in non‐erosive gastro‐oesophageal reflux disease
Authors: Fujiwara Y. , Higuchi K. , Nebiki H. , Chono S. , Uno H. , Kitada K. , Satoh H. , Nakagawa K. , Kobayashi K. , Tominaga K. , Watanabe T. , Oshitani N. , Arakawa T.
Published: June 2005

Background : Several studies in Western countries showed that proton‐pump inhibitors are superior to histamine2‐receptor antagonists or placebo in the treatment of non‐erosive gastro‐oesophageal reflux disease. The efficacy of acid‐suppressive drugs for non‐erosive gastro‐oesophageal reflux disease in Japan, in which the prevalence of Helicobacter pylori infection is higher compared with Western countries, is unknown.

Aim : To compare the efficacy of famotidine and omeprazole in Japanese patients with non‐erosive gastro‐oesophageal reflux disease by a prospective randomized multicentre trial.

Methods : A total of 98 patients received either famotidine 20 mg b.d. (n = 48) or omeprazole once daily (n = 50). Frequency of gastro‐oesophageal reflux disease symptoms and health‐related quality of life were evaluated at baseline and after 4 weeks of treatment. Complete relief was defined as no gastro‐oesophageal reflux disease symptoms during the 7‐day interval in week 4.

Results : Complete relief was achieved in 23 (48%) of patients receiving famotidine and 28 (56%) of patients treated with omeprazole. In the famotidine group, complete relief rate in H. pylori‐negative patients was significantly lower than H. pylori‐positive patients (35% vs. 64%). Both famotidine and omeprazole improved most scales of health‐related quality of life. Omeprazole significantly improved reflux score irrespective of H. pylori infection while famotidine significantly improved reflux score in H. pylori‐positive patients but not in H. pylori‐negative patients.

Conclusions : Omeprazole is more effective than famotidine for the control of gastro‐oesophageal reflux disease symptoms in H. pylori‐negative patients, while similar efficacy is observed in H. pylori‐positive patients with non‐erosive gastro‐oesophageal reflux disease.