bluuebunny/crossref_metadata_2025 huggingface dataset:
Disturbed very long chain (C24–C26) fatty acid pattern in fibroblasts of patients with Zellweger's syndrome
Authors: Govaerts L. , Bakkeren J. , Monnens L. , Maas J. , Trijbels F. , Kleijer W.
Published: June 2005

The very long chain fatty acids in cultured fibroblasts from six patients with the cerebro‐hepato‐renal syndrome of Zellweger, from six of their parents, from three controls, and also in three amniotic fluid control cell lines were analysed by gas chromatography. Increased concentrations of hexacosanoic acid (C26:O) were consistently found in the Zellweger syndrome. Also the ratios C26:O/C22:O, C25:O/C22:O, and C24:O/C22:O were elevated. The very long chain fatty acid levels and ratios in fibroblasts from the patients' parents were within the normal range. Findings in amniotic fluid cell lines indicate the possibility of antenatal diagnosis for Zellweger's syndrome. The similarities between neonatal adrenoleukodystrophy and Zellweger's syndrome suggest the applicability of this technique also in neonatal adrenoleukodystrophy.
Inability of second trimester human amniotic fluid cell cultures to aromatize C19‐steroids
Authors: O'Shannessy D. J. , Renwick A. G. C.
Published: June 2005

Amniotic fluid (AF) and fibroblast (F) cell types, derived from human amniotic fluid by amniocentesis in the second trimester, were cultured using media of varying composition and their ability to transform C19‐steroids to oestrogens was investigated by fluorimetry, radioimmunoassay, tritium release and measurement of cytochrome P450. Although the cells were shown to be metabolically active by the presence of mitotic figures, the incorporation of tritiated leucine into protein and the production of the β‐subunit of hCG, no evidence of aromatization was obtained despite the inclusion in the medium of a number of steroids known to be transformed to oestrogens in severalin vivo andin vitro preparations. The addition of reported stimulants of aromatase activity, viz. hCG, FSH, diethylstilbestrol, prostaglandins E2 and F2α and dibutyryl cyclic AMP, was without effect.
Gaucher disease (type 1): Physical and kinetic properties of liposomal and soluble ‘acid’ β‐glucosidase
Authors: Carroll M.
Published: June 2005

‘Acid’ β‐glucosidase of human spleen, from either normal controls or patients with type 1 (adult) Gaucher disease, was incorporated into phosphatidylcholine liposomes. The non‐incorporated (soluble) Gaucherenzyme had a higher apparent molecular weight than had the corresponding control. Liposomal ‘acid’ β‐glucosidase prepared from Gaucher‐spleen was more thermostable than was the corresponding normal enzyme; it was also stimulated by acidic lipids to a much lesser extent. The results suggest that the genetic mutation in type 1 (adult) Gaucher disease has multiple effects on the glycoprotein form of ‘acid’ β‐glucosidase.
Comparison of ornithine metabolism in hyperornithinaemia‐hyperammonaemia‐homocitrullinuria syndrome, lysinuric protein intolerance and gyrate atrophy fibroblasts
Authors: Botschner J. , Smith D. W. , Simell O. , Scriver C. R.
Published: June 2005

We measuredl‐ornithine oxidation in cultured skin fibroblasts from seven patients with hyperornithinaemia‐hyperammonaemia‐homocitrullinuria (HHH) syndrome (McKusick 23897), and compared it with oxidation by ornithine aminotransferase deficient gyrate atrophy (McKusick 25887) cells and lysinuric protein intolerance (McKusick 22270) cells in which there is an ornithine transport abnormality at the plasma membrane. Net uptake of ornithine is not abnormal in intact HHH cells. Ornithine oxidation was depressed in HHH and gyrate atrophy cells but not in lysinuric protein intolerance cells; the latter finding suggests there is no significant mitochondrial defect in lysinuric protein intolerance cells. Since HHH cells have intact ornithine aminotransferase, impaired oxidation is compatible with deficient penetration of ornithine into mitochondria in this disease. We could not demonstrate a gene dosage effect in oxidation values.
The role of the blood‐brain barrier in the aetiology of permanent brain dysfunction in hyperphenylalaninaemia
Authors: Hommes F. A.
Published: June 2005

Calculations on the rate of entry of the neutral amino acids into the brain via the blood‐brain barrier show that a considerable decrease in this rate, particularly for tryptophan and tyrosine, takes place in histidinaemia and tyrosinaemia, type II. These conditions are, however, not associated with mental retardation. It is therefore concluded that effects at the blood‐brain barrier alone do not provide an adequate explanation for the aetiology of permanent brain dysfunction in hyperphenylalaninaemia.
Abnormal fatty acid composition of biotin‐responsive multiple carboxylase deficiency fibroblasts
Authors: Packman S. , Whitney S. C. , Fitch M. , Fleming S. E.
Published: June 2005

Clinical and biochemical correlations in the biotin‐responsive multiple carboxylase deficiencies have suggested that disordered lipogenesis plays a role in the pathogenesis of the disease. In particular, the activity of biotin‐dependent acetyl CoA carboxylase and thede novo synthesis of fatty acids are reduced in mutant fibroblasts. In the present work, we examine the biochemical consequences of these deficiencies, and document and characterize an abnormal fatty acid composition in holocarboxylase synthetase deficiency fibroblasts. Following growth in biotin‐restricted medium, the total fatty acid content of mutant cells is reduced. There were significant reductions in the percentage as 16:0, 18:0 and 20:3N9 fatty acids, with the proportion of longer‐chain fatty acids either increased or maintained at control levels. The cellular content of 16:0, 16:1, 18:0, 18:1 and 20:3N9 fatty acids was reduced, while that of the longer‐chain fatty acids was preserved at control levels in mutant cells deprived of biotin. We speculate that the components of the altered fatty acid pools may be disproportionately incorporated into complex lipids in mutant cells, with pathologic effects on the multiple carboxylase deficiency phenotype.
Cultured skin fibroblasts: Useful for diagnosis of Wilson's disease?
Authors: Van den Berg G. J. , Van den Hamer C. J. A. , Meijer R. J. , Hoogenraad T. U.
Published: June 2005

The copper content of and radiocopper uptake in fibroblast cultures were studied to evaluate their usefulness for the diagnosis of Wilson's disease. We used methods closely related to those described in the literature, and applied these to cell lines of six patients with Wilson's disease and 12 controls. The results were:

The copper content of the cytosol of skin fibroblasts derived from patients with Wilson's disease was lower than that of controls when the cells were grown in a medium with a low copper concentration (0.7 µmol L−1); increased copper concentration (157 µmol L−1 in the medium failed to demonstrate any difference between normal fibroblasts and those derived from patients with Wilson's disease.

Radiocopper uptake studies did not differentiate between normal fibroblasts and fibroblasts from patients with Wilson's disease.

We conclude that the cytosolic copper content of fibroblasts grown in a low copper medium is a potential diagnostic tool in Wilson's disease. At present not all controls can be distinguished from the Wilson cells; ways must be sought, therefore, of improving the technique.
Excretion of hippuric acid during sodium benzoate therapy in patients with hyperglycinaemia or hyperammonaemia
Authors: Barshop B. A. , Breuer J. , Holm J. , Leslie J. , Nyhan W. L.
Published: June 2005

In patients with non‐ketotic hyperglycinaemia and two patients with urea cycle disorders treated with varying doses of sodium benzoate there was a linear correlation between intake of benzoate and excretion of hippurate. Patients with non‐ketotic hyperglycinaemia excreted significantly more benzoate in the form of hippurate than patients with urea cycle disorders (74 ± 7.0vs 41 ± 3.6%). The plasma concentration of glycine decreased following benzoate treatment only in the patients with non‐ketotic hyperglycinaemia. The observed difference between the two groups in the excretion of hippurate seems to support the concept that glycine availability may be limiting in benzoate therapy for some patients.
Glutathionuria: γ‐Glutamyl transpeptidase deficiency
Authors: Wright Elaine C. , Stern J. , Ersser R. , Patrick A. D.
Published: June 2005

A mentally retarded young woman with severe behaviour problems was found to excrete large amounts of glutathione due to a generalized γ‐glutamyl transpeptidase deficiency. As in the only other case described in detail, plasma levels and renal reabsorption of the amino acids were normal. In the parents' urine, plasma and leukocytes, enzyme activity was normal but in their cultured fibroblasts it was below the minimum for the control range. An autosomal recessive mode of inheritance is suggested. The implications of these findings for the possible role of the γ‐glutamyl cycle in amino acid transport are briefly discussed.
Leigh's disease with decreased activities of pyruvate carboxylase and pyruvate decarboxylase
Authors: Van Biervliet J. P. G. M. , Duran M. , Wadman S. K. , Koster J. F. , van Rossum A.
Published: June 2005

In a patient with the clinical symptoms of Leigh's disease a partial deficiency of hepatic pyruvate carboxylase and pyruvate decarboxylase was found at necropsy. Cerebral pyruvate decarboxylase activity was also diminished. All enzyme activities were measured in total homogenates. The finding of typical necrotic lesions in the brain stem was consistent with the clinical diagnosis. During life moderate lactic acidaemia and no hypoglycaemia were observed, but an abnormal organic acid excretion pattern was present. The contribution of the enzyme defects to the aetiology of the disease is discussed.
Myelin and its abnormalities
Authors: McKhann G. M. , Poduslo S. E.
Published: June 2005

This review starts with a brief outline about what myelin is, what it does, and how it is formed. Attention is then turned to newer methods of studying myelinationin vitro. Finally, diseases which can affect myelin are commented upon and advances which have occurred over the last few years are pointed out.
Failure of protein loading tests to identify heterozygosity for ornithine carbamoyltransferase deficiency
Authors: Becroft D. M. O. , Barry D. M. J. , Webster D. R. , Simmonds H. A.
Published: June 2005

Protein loading tests for the diagnosis of heterozygous ornithine carbamoyltransferase deficiency were performed on two occasions on an asymptomatic woman whose daughter and two infant sons died of the disease. Neither loading test produced the expected increases in urinary orotic acid excretion and studies of other pyrimidine and purine metabolites in urine and plasma did not suggest that these would provide better discrimination from non‐carriers. The results probably reflect an extensive inactivation of the mutant X chromosome in liver cells and reinforce the need for caution in interpreting negative test results.
A treatment of non‐ketotic hyperglycinaemia
Authors: Schoos‐Barbette S. , Gerard J. , Francotte N. , Lambotte C.
Published: June 2005

The treatment comprising a special diet (without glycine, serine, and with a reduced amount of threonine), strychnine nitrate and ursodesoxycholic acid (UDCA) led to normoglycinaemia in this form of severe nonketotic glycine encephalopathy. Diet and treatment were well tolerated but without significant effect upon psychomotor development. This treatment should be more effective if administered before irreversible brain damage occurs, particularly in moderate and chronic forms of NKH.
Possible explanation for hyperglycinaemia in propionic acidaemia and methylmalonic acidaemia: Propionate and methylmalonate inhibit liver and brain mitochondrial glycine transport
Authors: Ugarte M. , Lopez‐Lahoya J. , Garcia M. L. , Benavides J. , Valdivieso F.
Published: June 2005

The effect of propionate and methylmalonate on the transport of glycine into rat liver and brain mitochondria was investigated. Both propionate and methylmalonate markedly inhibited mitochondrial glycine transport. These compounds also inhibited14CO2 production from [14C]glycine by isolated brain and liver mitochondria and glycine metabolism in rat brain cortex slices. These results are discussed with reference to hyperglycinaemia associated with propionic acidaemia and methylmalonic acidaemia and as a possible contributory factor to the pathological mechanisms in these conditions.
Aspartylglycosaminuria in Northern Norway in eight patients: Clinical heterogeneity and variations with the diet
Authors: Borud O. , Strömme J. H. , Lie S. O. , Torp K. H.
Published: June 2005

Urinary excretion of aspartylglycosamines was investigated in eight patients by semiquantitative thin‐layer chromatography, and bound glycosamines by a quantitative photometric method (Elson‐Morgan reaction). Each patient showed a fairly constant level, relative to the creatinine, of aspartylglycosamines in urine. The least retarded patient, aged 31, excreted about 350 mg/g creatinine, one‐third of that found in two severely retarded young patients, aged 4 and 7 years (1400 and 940 mg/g creatinine, respectively). Three days on a low‐protein diet did not change the aspartylglycosamine excretion in the patient showing the highest excretion rate.
Diagnosis of Gaucher's disease in cultured skin fibroblasts and leucocytes
Authors: Butterworth J. , Broadhead D. M.
Published: June 2005

Washing skin fibroblasts or leucocytes in 0.25 mol/l sucrose increases the activity ofβ‐glucosidase at acid pH. This effect is primarily due to removal of low levels of sodium chloride, which inhibit acidβ‐glucosidase. A secondary factor for skin fibroblasts is the removal of residual phosphate buffer pH 7.3 used to wash the cells following trypsinization. As theβ‐glucosidase activity of water‐lysed leucocytes is higher at acid pH than that of a saline suspension of leucocytes, the former are better for the diagnosis of Gaucher's disease. However, more reliable results still may be obtained by assay of this enzyme in cultured skin fibroblasts.
Glutaric aciduria type II: Biochemical investigation and treatment of a child diagnosed prenatally
Authors: Bennett M. J. , Curnock D. A. , Engel P. C. , Shaw L. , Gray R. G. F. , Hull D. , Patrick A. D. , Pollitt R. J.
Published: June 2005

Two sibs with the acute neonatal form of glutaric aciduria type II (deficientin vivo activity of multiple acyl‐CoA dehydrogenases) are described. In the second case diagnosis was made prenatally on the basis of reduced oxidation of palmitate by cultured amniotic fluid cells. With prompt intervention in the neonatal period and a carefully controlled diet later, this second case progressed well up to 4 months of age but died suddenly of cardiac failure, probably attributable to accumulation of fat. Neither patient showed any congenital morphological abnormality.

Cultured fibroblasts from the second case showed a marked defect in the oxidation of a range of substrates requiring acyl‐CoA dehydrogenases for their catabolism, but residual activity for some substrates was quite high. Large quantities of sarcosine were excreted in urine, again suggesting that the mutation leaves some residual dehydrogenation activity. Butyryl‐, octanoyl‐ and palmitoyl‐CoA dehydrogenases were present in essentially normal quantities in postmortem liver.
Transport and storage of metals
Authors: Laurie S. H.
Published: June 2005

Current understanding of the means by which the trace metals copper, iron and zinc are transported and stored in the human body is reviewed. Although metal‐related inherited metabolic diseases manifest themselves as deficiencies in metalloenzymes, these deficiencies in fact arise from disturbances in the absorption/rejection or transport or storage phases of the metal metabolism. The considerable information available on iron metabolism shows these phases to be closely interrelated with a number of feedback mechanisms operating as controls on the various metabolic stages. Copper and zinc have vastly different roles in their enzymes but, following their close coordination chemistry properties, have similar metabolic routes. Both make use of albumin for transport and of metallothionein at the absorption and hepatic‐binding stages. In contrast to iron, copper and zinc have no well‐defined storage system; instead they make use of body reserves distributed in various tissues. Our knowledge of the transport and storage systems of the trace metals is still very incomplete. More research is needed on the dynamics of movement of the trace metals between the various body tissues and fluids. Further advances will also come from the study of related metal inherited diseases in other animal species.
Mutations in humans and animals which affect copper metabolism
Authors: Camakaris J. , Phillips M. , Danks D. M. , Brown R. , Stevenson T.
Published: June 2005

Various inherited disorders of copper metabolism in man and animals are reviewed. Emphasis is placed on the use of cultured cells from mutants to determine the primary molecular defects and to acquire basic knowledge of normal copper metabolism. This allows better diagnostic tests and possible treatment of the disorders. Menkes' disease in humans and mottled mouse mutants are discussed in detail, as they illustrate these approaches.
Menkes' X‐linked disease: Prenatal diagnosis and carrier detection
Authors: Horn N.
Published: June 2005

Increased64Cu uptake into cultured cells is a biochemical marker for mutant cells in Menkes' disease (McKusick 30940). Using this marker selective prenatal diagnosis has been carried out in more than 80 at‐risk pregnancies. The64Cu uptake into cultures from affected male fetuses is, however, negatively correlated to the fetal age at amniocentesis. After the 18th week of gestation the risk of false negatives is significant.

Using copper uptake into uncloned cultures, a number of obligate and possible carriers showed significantly increased values, but the range of values of obligate carriers considerably overlapped those of the normal controls. All values of normal controls were within a limited range and values above the upper limit in females at risk must, therefore, be caused by mutant cells and establish the carrier diagnosis. However, the extreme skewing of the distribution towards normal values in obligate carriers indicates a strong selection against the mutant cell type and this will hamper the detection of all female carriers in risk families.

C‐banding heteromorphism of the X‐chromosome provides a supplementary carrier detection method. Linkage analysis in five Danish families demonstrated a close physical relationship between the gene for Menkes' disease and the centromere region. By comparative gene mapping (mouse/man) the most likely localization of the gene for Menkes' disease can be suggested to be in band q13 on the long arm of the human X‐chromosome. This regional assignment facilitates the choice of appropriate X‐specific DNA probes in search for linkage at the DNA level.
Inborn errors of cellular organelles: an overview
Authors: Tager J. M.
Published: June 2005

Metabolic processes in the cell are catalysed by enzymes and enzyme systems present in discrete intracellular compartments consisting of the cytosol and various intracellular organelles. Three well defined groups of genetic diseases in man can now be recognized in which the functions of an intracellular organelle are impaired: lysosomal storage diseases, mitochondrial disorders and peroxisomal diseases. Extensive studies carried out during the last decade on the biogenesis of intracellular organelles have contributed to an understanding of the molecular basis of the lesions leading to these three groups of genetic disorders. The results of the studies have stressed that such lesions can arise not only through mutations in the structural genes for the proteins in an organelle but also through mutations in the genes coding for components required for the specific transport and incorporation of proteins into organelles.
Effects of phenylalanine loading on protein synthesis in the fetal heart and brain of rat: an experimental approach to maternal phenylketonuria
Authors: Okano Y. , Chow I. Z. , Isshiki G. , Inoue A. , Oura T.
Published: June 2005

Pregnant rats were loaded withl‐phenylalanine, and the distributions of [14C]leucine and [14C]urea into fetal plasma and tissues were examined. Uptake of [14C]leucine into the supernatant and protein fractions of fetal plasma and tissues was low in the rats loaded with phenylalanine. In contrast, [14C]urea was distributed identically in both groups, indicating that maternal hyperphenylalaninemia did not affect blood flow across the placenta. Administration of phenylalanine andp‐chlorophenylalanine produced amino acid imbalance in fetal tissues. Along with these changes, polysomes of the affected fetal heart and brain disaggregated without changes in the ribonuclease activity. These results indicate that high phenylalanine levels in maternal plasma disturb the active transport of amino acids across the placenta, causing an amino acid imbalance and disaggregation of polysomes in fetal heart and brain. These changes may contribute to the congenital heart disease and mental retardation of maternal phenylketonuria.
Possible prenatal diagnosis of type III glycogenosis
Authors: Maire I. , Mathieu M.
Published: June 2005

This paper reports the first prenatal diagnosis in a pregnancy at risk for type III glycogenosis using a reliable test for amylo‐1,6‐glucosidase in cultured amniotic fluid cells. This test, based upon the different rates of glycogen and phosphorylase limit dextrin hydrolysis, was found to be more reliable in detecting a deficiency of amylo‐1,6‐glucosidase activity than either phosphorylase limit dextrin hydrolysis alone or [14C]glucose incorporation using leukocytes and cultured cells.
Amniotic tissue transplantation as a trial of treatment in some lysosomal storage diseases
Authors: Tylki‐Szymańska A. , Maciejko D. , Kidawa M. , Jabłońska‐Budaj U. , Czartoryska B.
Published: June 2005

This communication reports the clinical and biochemical results in six patients: four with mucopolysaccharidosis, one with GM1 gangliosidosis (Morquio B) and one with I‐cell disease, who were treated by amniotic tissue transplantation. The sole evident clinical result was the diminishing of corneal clouding in three cases. A slight increase of beta‐galactosidase activity in one patient's plasma was observed. The time of improvement was about 2 months after the transplantation and was transitory.
Cranial computerized tomography in dihydropteridine reductase deficiency
Authors: Longhi R. , Valsasina R. , Buttè C. , Paccanelli S. , Riva E. , Giovannini M.
Published: June 2005

Dihydropteridine reductase deficiency is a rare cause of hyperphenylalaninaemia, characterized by severe and progressive neurological impairment, despite early and accurate dietary control of plasma phenylalanine. We describe two girls, diagnosed at 17 and 14 months of age, respectively, and immediately treated withl‐dopa, 5‐hydroxytryptophan and carbidopa. In spite of an adequate dietary and pharmacological treatment, the clinical and neurological pictures progressively worsened. Repeated cranial computerized axial tomography scans showed degeneration of the white matter and, in one case, calcification of the basal ganglia. The possible association of this last finding with folate depletion is discussed.
Biochemical phenotyping of a single sibship with both cystinosis and fabry disease
Authors: Gahl W. A. , Adamson M. , Kaiser‐Kupfer I. , Ludwig I. H. , O'Connell H. J. , Cohen W. , Barranger J.
Published: June 2005

Two lysosomal storage diseases, cystinosis and Fabry disease, were diagnosed clinically in different members of a single sibship. The possibility that the affected sister and brother might have related disorders with disparate manifestations was pursued. The four principal family members were tested for heterozygote status with respect to serum and leukocyte α‐galactosidase A activity, urinary trihexosylceramide excretion, and the capacity to engage in cystine counter‐transport across leukocyte lysosome membranes. Results were consistent with classical autosomal recessive inheritance in the case of cystinosis and X‐linked inheritance for Fabry disease, confirming that this family represents an example of two rare disorders occurring in the same sibship.
An evaluation of protein requirements in methylmalonic acidaemia
Authors: Ney D. , Bay C. , Saudubray J. M. , Kelts D. G. , Kulovich S. , Sweetman L. , Nyhan W. L.
Published: June 2005

A 3‐month‐old girl and a 13‐month‐old boy with vitamin B12‐unresponsive methylmalonic acidaemia were studied to determine responses to varying levels of protein intake of growth, nitrogen balance and organic acid metabolism. A linear increase in the excretion of methylmalonic acid was observed in both patients above a critical level of protein intake. The inflection point was judged to reflect a ceiling above which amino acid intake exceeded requirements and catabolism was initiated. Below this point in each infant there was a plateau of minimal excretion of methylmalonic acid. Within this plateau level a reasonable rate of growth and metabolic stability were achieved at intakes between 0.70 and 0.75 and between 0.75 and 1.17 g protein kg−1, respectively, indicating that there is a range of protein tolerance and the importance of an individual approach to the provision of protein in patients with methylmalonic acidaemia. In the 3‐month‐old infant, nitrogen equilibrium was achieved at protein intakes above 0.6 g kg−1 and modest nitrogen retention was attained at a protein intake of 0.75 g kg−1, a level at which the excretion of methylmalonic acid was minimal and weight gain satisfactory. A protein intake of 1.25 g kg−1 was required to achieve a level of nitrogen retention often considered optimal for normal growth; however, this infant demonstrated an elevated excretion of methylmalonic acid and was close to clinical illness at this level of protein intake. The 13‐month‐old infant demonstrated a normal level of nitrogen retention, minimal excretion of methylmalonic acid, and a satisfactory rate of growth at protein intakes of 1.0–1.17 g kg−1. The values should prove useful guidelines for the management of infants requiring minimal intakes of protein. In studies carried out at 18–20 months of age, supplementation of the basic diet containing 0.75 g kg protein−1 with a mixture of amino acids not containing the precursors of methylmalonic acid was associated with increase of retention of nitrogen and increased concentrations of some essential amino acids in plasma, but effects on growth and the excretion of methylmalonic acid were not significant.
Groin Hernia Repair by Laparoscopic Techniques: Current Status and Controversies
Authors: Arregui Maurice E. , Young Susan B.
Published: June 2005

Laparoscopic hernia repair remains controversial, and its position in current hernia surgery remains in flux. In this article we attempt to put the laparoscopic approach in perspective by describing the rationale for its development. We summarize studies comparing it with open repairs, including recent publications, meta‐analyses, and systematic reviews; and we then contrast the data with recent findings of the United States Veterans Affairs Cooperative study 456. We discuss the current and future status of the laparoscopic approach to inguinal hernia repair and present an update of our own laparoscopic totally extraperitoneal technique without mesh fixation. From 1994 to 2004 we performed 314 hernia repairs on 224 patients with no intraoperative complications, no conversions to an open procedure, and no mortality. Thirty (14%) minor postoperative complications occurred. There were three herniated lipomas (preperitoneal fat) but no true peritoneal reherniations. We evaluate critical points of laparoscopic hernia repair including extensive preperitoneal dissection, mesh configuration, size and fixation, cost reduction, and the learning curve.
A View on Decoherence Via Master Equations
Authors: Rebolledo Rolando
Published: June 2005

This paper addresses the discussion on probabilistic features of the concept of decoherence as it appeared in quantum physics. Given a Lindblad-type generator of an open system dynamics, we derive applicable criteria to characterize decoherent behaviour.
Finite Temperature Dynamics of the Total State in an Open System Model
Authors: Strunz Walter T.
Published: June 2005

We determine the dynamics of the total state of a system and environment for an open system model, at finite temperature. Based on a partial Husimi representation, our framework describes the full dynamics very efficiently through equations in the Hilbert space of the open system only. We briefly review the zero-temperature case and present the corresponding new finite temperature theory, within the usual Born-Markov approximation. As we will show, from a reduced point of view, our approach amounts to the derivation of a stochastic Schrödinger equation description of the dynamics. We show how the reduced density operator evolves according to the expected (finite temperature) master equation of Lindblad form.
On Concurrence and Entanglement of Rank Two Channels
Authors: Uhlmann Armin
Published: June 2005

Concurrence and further entanglement quantifiers can be computed explicitly for channels of rank two if representable by just two Kraus operators. Almost all details are available for the subclass of rank two 1-qubit channels. There is a simple geometric picture beyond, explaining nicely the role of anti-linearity.
Species richness and composition of Curculionidae (Coleoptera) in a conifer plantation, secondary forest, and old‐growth forest in the central mountainous region of Japan
Authors: Ohsawa Masashi
Published: June 2005

Weevil (Curculionidae, Coleoptera) species richness and composition were investigated and compared among larch [Larix kaempferi (Lamb.) Carriére] plantations, secondary forests, and old‐growth forests in the central mountainous region of Japan. In addition, to assess the effects of forest‐management practices, namely thinning and long‐rotation logging schedules (long rotation), the weevil assemblages of recently thinned middle‐aged and long‐rotated larch plantations were compared with those of middle‐aged larch plantations. Malaise traps were set in 44 stands of these forest types, and weevils were separated and identified. Several environmental factors other than forest type were also examined. Weevil species richness and diversity indices [Shannon‐Wiener diversity index (H′) and Simpson's index of diversity (D)] were higher in the secondary forest than in the larch plantation. Because of its wide distribution and higher weevil species richness, the secondary forest contributed to maintaining weevil diversity in this region. Old‐growth forest had higher diversity indices (H′ and D) than did the larch plantation. The secondary forest had the highest number of species in total. Though the number of individuals was the highest in larch plantation, species richness, H′ and D of the plantation were generally low. Weevil community structure and species composition differed among the three forest types, but the difference in weevil composition between the larch plantation and the other two forest types was the largest. Forest type is probably the most important factor for determining the differences in weevil assemblage, and further, both dominant tree type (coniferous trees versus broad‐leaved trees) and the number of mature tree species seem to be important factors for weevil species composition. Among forest management practices, long rotation caused diversity indices (H′ and D) to increase while thinning appeared to cause only minor changes in the weevil assemblages. Because species richness and species composition of Curculionidae well reflected the differences in forest types and some other environmental factors investigated, this family seems suitable for diversity research in forests. Further research on biodiversity with the use of this family should, therefore, be expected.
NF‐κB activation but not PI3K/Akt is required for dexamethasone dependent protection against TNF‐α cytotoxicity in L929 cells
Authors: Mendoza-Milla Criselda , Rodríguez Catalina Machuca , Alarcón Emilio Córdova , Bernal Adriana Estrada , Toledo-Cuevas E. Mayra , Martínez Eduardo Martínez , Dehesa Alejandro Zentella
Published: June 2005

Tumor necrosis factor alpha (TNF‐α) is one of the best‐described cell death promoters. In murine L929 fibroblasts, dexamethasone inhibits TNF‐α‐induced cytotoxicity. Since phosphatidyl inositol 3 kinase (PI3K) and nuclear factor kappa B (NF‐κB) proteins regulate several survival pathways, we evaluated their participation in dexamethasone protection against TNF‐α cell death. We interfered with these pathways by overexpressing a negative dominant mutant of PI3K or a non‐degradable mutant of inhibitor of NF‐κB alpha (IκBα) (the cytoplasmic inhibitor of NF‐κB) in L929 cells. The mutant IκB, but not the mutant PI3K, abrogated dexamethasone‐mediated protection. The loss of dexamethasone protection was associated with a diminished accumulation in XIAP and c‐IAP proteins.
Abortion and Postabortion Care: Ethical, Legal, and Policy Issues in Developing Countries
Authors: Miller Suellen , Billings Deborah L.
Published: June 2005

This case study of a woman who wants to terminate her pregnancy but does not have access to safe services explores the technical, ethical, and legal effects of the Mexico City Policy (Global Gag Rule) on health care providers working in developing countries. This woman's self‐induced termination resulted in an incomplete abortion, and she sought care from a midwife. The current Mexico City Policy effectively limits a health care provider's ability to offer abortion services and counseling, even when these services are legal. The policy has an adverse impact on women's access to safe care. The provision of comprehensive postabortion care, not restricted by the Mexico City Policy, is the key to preventing abortion‐related morbidity and mortality.
Mothers, Midwives, and HIV/AIDS in Sub‐Saharan Africa
Authors: Raisler Jeanne , Cohn Jonathan
Published: June 2005

This article reviews clinical and program issues in the prevention of mother to child transmission (PMTCT) of HIV in sub‐Saharan Africa. Topics include prevention of infection, voluntary counseling and testing, prenatal care, labor and birth, postpartum, family planning, infant feeding, and the role of traditional birth attendants. Programs providing short‐course antiretroviral therapy to prevent infant infection are contrasted with comprehensive programs offering antiretroviral therapy and medical care to mothers, children, and families. Feminization of the epidemic is related to gender inequalities that facilitate the spread of HIV and make pregnant women an especially vulnerable group. Nurses and midwives are the primary health care providers for most of the population in sub‐Saharan Africa. They are the backbone of the new PMTCT programs and will be the largest group of health workers available to diagnose and treat opportunistic infections and dispense antiretroviral therapy. But they have received little training and support to provide AIDS care and treatment and are rarely consulted when plans are made about workforce issues and capacity development in the health sector. Clinical training, leadership skills, salary support, expansion of the nursing workforce, and development of expanded roles for nurses and midwives in AIDS care are needed to help them turn the tide of the epidemic.
Cryotherapy Treatment for Cervical Intraepithelial Neoplasia: Women's Experiences in Peru
Authors: Coffey Patricia S. , Bingham Allison , Winkler Jennifer L. , Bishop Amie , Sellors John W. , Lagos Gloria , Pastor Cesar Moron
Published: June 2005

Our objective was to examine cryotherapy experiences among women who received treatment for cervical intraepithelial neoplasia in a cervical cancer prevention project in rural Peru. The sample consisted of all women receiving cryotherapy during a 4‐month period (July through October 2001). Structured interviews were conducted to collect information about the adequacy of information provision, women's satisfaction with cryotherapy, their ability to comply with postcryotherapy recommendations and condom use, their experience with cryotherapy side effects, and their satisfaction with cryotherapy follow‐up. Of the 224 women who were interviewed, user satisfaction with cryotherapy treatment was generally good. A few women engaged in sex earlier than 30 days after treatment, primarily due to partner pressure to resume sex and the women's inability to successfully negotiate abstention from sex. These couples were not always able to use condoms. The percentage of women reporting vaginal discharge was within the range of responses reported in other studies. Cryotherapy appears to be acceptable to women in low‐resource settings such as Peru. J Midwifery Womens Heal
Barriers to and Facilitators for Newborn Resuscitation in Malawi, Africa
Authors: Bream Kent D.W. , Gennaro Susan , Kafulafula Ursula , Mbweza Ellen , Hehir David
Published: June 2005

Newborn resuscitation is a key component of efforts to reduce neonatal morbidity and mortality. This article reports the findings of focus groups conducted to explore the barriers and facilitators present for obstetric nurse providers in a central urban hospital in Malawi regarding the training and provision of neonatal resuscitation. All obstetric nurse providers in this setting participated in focus groups; these groups identified 4 themes: 1) confidence in their skills in assessing newborns and recognizing the need for resuscitation, 2) a lack of resources, 3) facilitators who would enable them to introduce resuscitation, and 4) solutions to the current problems. Resource shortages and barriers to providing care included availability of staff, equipment, and supplies; labor ward geography; ethical dilemmas; and the lack of standard protocols regarding newborn resuscitation. Facilitators were professional experience and the critical load of deliveries performed. Solutions to barriers included small resource additions as well as long‐term policy changes. With standard policy and protocols, experienced, confident nurses could overcome the barriers to providing newborn resuscitation. This group identified ways to change systems to decrease infant mortality, thereby improving the health and quality of life of women receiving care in Malawi.
Managing bipolar disorder during pregnancy and lactation: is there a safe and effective option?
Authors: Thomas Pierre , Severus W. Emanuel
Published: June 2005

Bipolar disorder is a serious psychiatric illness that usually emerges during adolescence or early adulthood, and patients are likely to experience recurrent episodes throughout their lives. The treatment of bipolar disorder is complicated by the difficulty in distinguishing between subtly different disease subtypes (bipolar I, bipolar II, rapid cycling and mixed episodes), each of which is associated with a different probability of treatment success. Furthermore, physicians are faced with an array of treatment options that includes mood stabilisers, antidepressants, and typical and atypical antipsychotics.
Bipolar disorder and personality disorder
Authors: Bowden Charles , Maier Wolfgang
Published: June 2005

Patients with bipolar disorders experience cyclical changes in mood that present as a range of different syndromes. In classical mania, patients experience episodes of euphoria, whereas in depressive episodes they suffer from depression. In hypomania, patients experience a milder form of mania, and in mixed mania, patients may experience both manic and depressive symptoms simultaneously, or alternate between them rapidly. Because of this wide range of symptoms, bipolar disorders can appear to overlap with other mental disorders, especially personality disorders.
Microzooplankton dynamics during the development of the spring bloom in the north-east Atlantic
Authors: Fileman E.S. , Leakey R.J.G.
Published: June 2005

Microzooplankton community composition, abundance, biomass and grazing impact were assessed, along with measurements of ciliate growth and mortality, during the onset of the spring bloom in the north-east Atlantic. The study was undertaken as part of the UK Biogeochemical Ocean Flux Study during 1 May to 15 June 1990. The microzooplankton community was composed of protozoans and metazoan developmental stages with respective mixed-layer depth integrated biomass values ranging from 127 to 638 and 74 to 394 mg C m−2. High numbers of aloricate ciliates (up to 35,000 cells l−1) dominated the microzooplankton community during early May prior to the onset of the spring bloom. Ciliate abundance then declined rapidly during mid-May with community growth rates ranging from −0·71 to 0·23 d−1. High abundances of metazoplankton (up to 400 l−1) were also recorded at this time and may have contributed to the decline in ciliate numbers. In late May and early June the protozoan community comprised a more even mix of dinoflagellates, tintinnids and aloricate ciliates. Phytoplankton mortality rates, measured using a dilution technique, ranged from 0·2 to 0·5 d−1. The microzooplankton consumed 8 to 44 μg C l−1 d−1, equivalent to between 16 and 40% of the chlorophyll biomass and 38 and 154% of primary production. These high rates of herbivory reflect the predominance of small (<5 μm in length) phytoplankton cells present throughout the first half of the study and support previous studies demonstrating the microzooplankton to be the main grazers of phytoplankton in the north-east Atlantic. However, there is also evidence that a disparity between predator and prey may have prevented a response by the microzooplankton to rapid increases in phytoplankton biomass and production during the spring bloom.
Summer mesozooplankton distribution in coastal waters of central Greece (eastern Mediterranean). I. Hydrology and group composition
Authors: Ramfos Alexis , Somarakis Stylianos , Koutsikopoulos Constantin , Fragopoulu Nina
Published: June 2005

Zooplankton and hydrography of the upper 200 m was studied in coastal and pelagic waters off central Greece (eastern Mediterranean) during July 1998. The spatial distribution of the main environmental (temperature, salinity, fluorescence) and zooplankton (abundance, dry-weight, dominant groups) features of the area was delimited over an intense grid of 124 stations. The complex topography of the surveyed area comprised an interesting field for the study of pelagic–coastal interaction and its influence on the taxonomic composition of zooplankton.

A typical summer thermal stratification was observed over the entire study area. Marked differences were found in surface-salinity between the eastern (Aegean Sea) and the western (Ionian Sea) part of the study area, which was attributed to the presence of less saline waters originating from the Black Sea. A deep fluorescence maximum was present in both coastal and pelagic stations. Zooplankton abundance and biomass showed an inshore–offshore gradient with coastal areas being significantly richer than pelagic areas. The taxonomic composition of zooplankton varied greatly both along the inshore–offshore gradient and among the different semi-enclosed Gulfs. Copepods were the dominant group in the pelagic waters comprising 67–78% in terms of mean relative abundance. In coastal areas, copepods were less important with cladocerans (especially the species Penilia avirostris) being the dominant group (44–51%). Appendicularians and chaetognaths comprised an important component of the zooplankton community over the entire surveyed area with mean relative abundance ranging from 2·5 to 14% and 0·6 to 5·8%, respectively.
Characterization of a Karenia papilionacea-like dinoflagellate from the South China Sea
Authors: Yeung P.K.K. , Hung V.K.L. , Chan F.K.C. , Wong J.T.Y.
Published: June 2005

The morphological characteristics of a new isolate of a dorso-ventrally flattened athecate dinoflagellate obtained from Hong Kong (HK) waters is described. Partial sequences of the large subunit ribosomal RNA gene were obtained and a high degree of similarity was observed with various species within the genus Karenia. Phylogenetic comparison suggested the Karenia sp. HK forms a clade with the newly described species K. papilionacea from New Zealand but not with authentic K. brevis from North America. Karenia sp. HK is the first K. brevis-like dinoflagellate characterized by molecular cladistics from the South China Sea and the results call for re-examination of the status of other K. brevis-like morphotypes described from the region.
A new species of Orbiniella (Polychaeta: Orbiniidae) from deep basins of Antarctica
Authors: Narayanaswamy Bhavani E. , Blake James A.
Published: June 2005

During the 2002 Antarctic Deep-sea Biodiversity (ANDEEP) programme to the Drake Passage, Weddell Sea Basin and South Sandwich Slope and trench, a new deep-water species of orbiniid polychaete was collected: Orbiniella andeepia sp. nov. Orbiniella andeepia appears to be most closely related to O. marionensis but differs in capillary setal structure, the type and number of acicular spines found in each podial lobe. Orbiniella andeepia is only the third deep-water species of Orbiniella to be discovered. It exhibits both a wide depth- and geographic-range within the Antarctic slope and abyssal sediments.
The complete larval development of Pilumnus limosus (Crustacea: Decapoda: Brachyura: Pilumnidae) described from laboratory reared material
Authors: García-Guerrero Marcelo U. , Hendrickx Michel E. , Cuesta José A. , Rodríguez Antonio
Published: June 2005

The pilumnid crab Pilumnus limosus is known from Gonzaga Bay, Baja California, Mexico, to Zorritos, Paita, Peru. Larvae from a female collected in the intertidal rocky shore in Mazatlán, south-eastern Gulf of California, Mexico, were reared in the laboratory from Zoea I to megalopa. A complete description and illustration of larval features is provided and a comparison with closely related species showing known larval development is presented. As in most species of this genus, P. limosus larval development consists of four zoeae. All features observed in P. limosus are similar to those of P. reticulatus and P. sayi. All those larvae have lateral processes on abdominal somites 2–5 (2 or 3 in other species); posteromarginal denticles on abdominal somites 2–6 (absent in other species of the genus).
Reproductive biology of the hairy crab Pilumnus vespertilio (Brachyura: Pilumnidae) in the East African region
Authors: Litulo Carlos
Published: June 2005

Reproductive patterns such as gonad maturation, production and release of gametes, fecundity and moulting play a major role in the continuity of populations and their adaptations to the environment they inhabit. The present study assesses the breeding cycle, moult activity and egg production in the hairy crab Pilumnus vespertilio, a typical inhabitant of intertidal pebble and rocky substrates of East African coasts. A total of 930 crabs was collected between January and December 2002 at Inhaca Island, southern Mozambique. Both oogenesis and spermatogenesis exhibit a continuous and synchronized pattern. Maturation of the female and male gonads occurs from early winter to mid summer and spawning is in summer. Moulting females showed two major peaks (January–March and October–December) whereas a single peak was recorded in males (October–December). Pilumnus vespertilio breeds continuously with high incidence in March and December and a strong decrease from April to July. Results revealed that both temperature and rainfall were positively associated with the relative abundance of egg-bearing females. The number of eggs carried by ovigerous females increased with size, ranging from 300 to 8000. The high reproductive activity of P. vespertilio may enhance larval survival and development, which guarantees juvenile recruitment in the study area.