bluuebunny/crossref_metadata_2025 huggingface dataset:
Household composition and food expenditures in China
Authors: Gould Brian W.
Published: October 2002

With China admitted to the World Trade Organization there is the potential for dramatic increases in U.S. agricultural exports. The present analysis uses household‐level data to identify important determinants of expenditures on food for at‐home consumption by households in three Chinese urban provinces. Besides the role of household income, we examine the impact of household member age/gender composition on expenditures via the estimation of endogenously determined adult equivalents. Results from the analysis show that overall food at‐home expenditures are income inelastic, some commodities exhibit elastic responses to changes in their own price and household composition has a significant impact on food choice. [JEL category: D120]. © 2002 Wiley Periodicals, Inc.
A patent analysis of global food and beverage firms: The persistence of innovation
Authors: Alfranca Oscar , Rama Ruth , von Tunzelmann Nicholas
Published: October 2002

We explore whether current innovation has an enduring effect on future innovative activity in large, global food and beverage (F&B) companies. We analyze a sample of 16,698 patents granted in the United States over the period 1977 to 1994 to 103 F&B firms selected from the world's largest F&B multinationals. We test whether patent time series are trend stationary or difference stationary in order to detect how large the autoregressive parameter is and how enduring the impact of past innovation in these companies is. We conclude that the patent series are not consistent with the random walk model. The null hypothesis of a unit root can be rejected at the 5% level when a constant and a time trend are considered. Both utility and design patent series are stationary around a constant and a time trend. Moreover, there is a permanent component in the patent time series. Thus, global F&B firms show a stable pattern of technological accumulation in which “success breeds success.” “Old” innovators are the ones to foster both important changes and new ways of packaging products among F&B multinationals. The effect of past innovation is almost permanent. By contrast, other potential stimuli to technological change have only transitory effects on innovation. Patterns of technological accumulation vary in specific F&B industries. Past experience in design is important in highly processed foods and beverages, but not in agribusinesses and basic foodstuffs. Patterns of technological accumulation are similar in both smaller multinationals/newcomers and large, established multinationals. [EconLit citations : O330, F230, L660] © 2002 Wiley Periodicals, Inc.
Cholecystitis as the presenting manifestation of acute myeloid leukemia
Authors: Bloom Stuart H. , Coad James E. , Greeno Edward W. , Ashrani Aneel A. , Hammerschmidt Dale E.
Published: October 2002

We report a case of acute leukemia, which presented as cholecystitis in a previously healthy middle‐aged adult. Leukemic involvement of the gastrointestinal tract is a well‐known clinicopathological entity. However, leukemic infiltration of the gall bladder wall is a rare occurrence. It has only been previously described in the setting of relapsed disease and, to our knowledge, has never been reported as the primary manifestation of de novo acute leukemia. The patient in question was treated with standard induction and consolidation chemotherapy and remains in complete remission 19 months after his presentation with symptoms consistent with cholecystitis. Am. J. Hematol. 70:254–256, 2002. © 2002 Wiley‐Liss, Inc.
Fatal asthma from powdering shark cartilage and review of fatal occupational asthma literature
Authors: Ortega Hector G. , Kreiss Kathleen , Schill Donald P. , Weissman David N.
Published: October 2002

Background

Work‐related asthma (WRA) is the most common work‐associated respiratory disease in developed countries.

Method

We report shark cartilage dust as a new potential cause of occupational asthma (OA) in the context of other fatal OA case reports.

Results

A 38‐year‐old white male worked for 8 years in a facility which primarily granulated and powdered various plastics. Sixteen months prior to his death, the plant began grinding shark cartilage. After 10 months of exposure, he reported chest symptoms at work in association with exposure to shark cartilage dust and a physician diagnosed asthma. Six months later, he complained of shortness of breath at work and died from autopsy‐confirmed asthma. The latency from onset of exposure to symptoms and from symptom onset to death was shorter than 10 previously reported OA fatalities.

Conclusion

Recognition of occupational causes and triggers of asthma and removal of affected individuals from these exposures is critical and can prevent progression to irreversible or even fatal asthma. Am. J. Ind. Med. 42: 50–54, 2002. © 2002 Wiley‐Liss, Inc.
Prevalence of musculoskeletal symptoms and carpal tunnel syndrome among dental hygienists
Authors: Anton Dan , Rosecrance John , Merlino Linda , Cook Thomas
Published: October 2002

Background

Carpal tunnel syndrome (CTS) and other work‐related musculoskeletal disorders (MSDs) are common problems in general industry as well as dental hygiene. The purpose of this study is to determine the prevalence of CTS and other MSDs among dental hygienists. No previous studies involving dental hygienists have combined nerve conduction studies (NCS) and symptoms in their CTS case definitions.

Methods

Dental hygienists (N = 95) completed a symptom and job factors survey, hand symptom diagram, and underwent NCS across the wrist.

Results

At least one MSD was reported by 93%. Prevalence of CTS was 8.4% utilizing a case definition of symptoms and NCS, but 42% if defined by symptoms alone. Age, BMI, and the number of patients treated per day were significant factors associated with CTS.

Conclusions

Dental hygienists are exposed to occupational factors that increase the risk of CTS. A case definition that does not include NCS overestimates CTS prevalence. Am. J. Ind. Med. 42:248–257, 2002. © 2002 Wiley‐Liss, Inc.
Facilitating informed choice in prenatal testing: How well are we doing?
Authors: Marteau Theresa M. , Dormandy Elizabeth
Published: October 2002

There is a consensus that prenatal testing services need to provide the information and support necessary for women to make informed choices about prenatal testing. Informed choices are those based on relevant information that reflect the decision‐maker's values. To date, most research has focused on the information provided to women deciding whether to undergo tests. This has highlighted the poor quality of information provided to many women. There is agreement on the need to provide information on three key aspects of any test: the condition for which testing is being offered, characteristics of the test, and the implications of testing. Very little research has been conducted on decisions after the diagnosis of a fetal abnormality and how information and emotional and decisional support are and should be provided. Research is now needed in four key areas: first, on the optimal ways of organizing services to facilitate choices that are not only based on relevant information, but also reflect the decision‐maker's values; second, on the most effective ways of framing information needed for the different decisions involved in prenatal testing; third, on the most effective media in which to deliver information; and, fourth, to identify aspects of counseling that facilitate informed choices following diagnoses of fetal abnormality. If we value women's ability to make informed choices about prenatal tests as highly as we value reliable laboratory tests, evidence‐based quality standards need to be developed for the information and support women are given at all stages of the process of prenatal testing. © 2001 Wiley‐Liss, Inc.
Association study between two variants in the DOPA decarboxylase gene in bipolar and unipolar affective disorder
Authors: Jahnes Esther , Müller Daniel J. , Schulze Thomas G. , Windemuth Christine , Cichon Sven , Ohlraun Stephanie , Fangerau Heiner , Held Tilo , Maier Wolfgang , Propping Peter , Nöthen Markus M. , Rietschel Marcella
Published: October 2002

Irregularities of dopaminergic and serotonergic neurotransmission have been implicated in a variety of neuropsychiatric disorders. DOPA decarboxylase (DDC), also known as aromatic L‐amino acid decarboxylase, is an enzyme involved directly in the synthesis of dopamine and serotonin and indirectly in the synthesis of noradrenaline. Therefore, the DDC gene can be considered as a candidate gene for affective disorders. Recently, two novel variants were reported in the DDC gene: a 1‐bp deletion in the promoter and a 4‐bp deletion in the untranslated exon 1. Subsequently, an association case–control study including 112 English patients and 80 Danish patients with bipolar affective disorder (BPAD) revealed a significant association with the 1‐bp deletion. This finding prompted us to analyze whether this effect was also present in a larger and ethnically homogeneous sample of 228 unrelated German patients with BPAD (208 patients with BP I disorder, 20 patients with BP II disorder), 183 unrelated patients with unipolar affective disorder (UPAD), and 234 healthy control subjects. For both BPAD and UPAD we could not detect a genetic association with either variant. Thus, our results do not support an involvement of the 1‐bp or 4‐bp deletion within the DDC gene in the etiology of affective disorders. © 2002 Wiley‐Liss, Inc.
Molecular properties of HLA‐DQ alleles conferring susceptibility to or protection from insulin‐dependent diabetes mellitus: Keys to the fate of islet β‐cells
Authors: Moustakas Antonis K. , Papadopoulos George K.
Published: October 2002

The major histocompatibility complex Class II alleles, HLA‐DQ, and the related HLA‐DR, are the chief genetic elements of human type 1 diabetes. These genes code for polymorphic heterodimeric proteins, whose chief function is to trap peptide antigens in the endosome and present them on the surface of antigen‐presenting cells (dendritic cells, B lymphocytes, monocytes/macrophages) to CD4+T helper cells. A systematic investigation of the molecular properties of HLA‐DQ alleles linked to susceptibility or resistance to type 1 diabetes has shown that these properties segregate along lines of susceptibility or resistance. A correlation of these features with the function of each particular segment of the HLA‐DQ molecule yields interesting insights into the possible pathways leading to type 1 diabetes. There remain, however, areas to be clarified, including mechanisms by which dominant protection is conferred by certain alleles, the interplay between HLA‐DQ and the related locus HLA‐DR, that also shows autoantigen‐specific reactivity, and the cross‐Class help delivered to CD8+T cells, the final effectors in pancreatic β‐cell destruction. Clarification of these issues may lead to ways to prevent diabetes in predisposed individuals already exhibiting the genetic and immunological characteristics, and perhaps a cure in those with the disease, by means of transplantation, and measures for prevention of disease recurrence. © 2002 Wiley‐Liss, Inc.
Leukocyte adhesion deficiency (LAD) type II/carbohydrate deficient glycoprotein (CDG) IIc founder effect and genotype/phenotype correlation
Authors: Etzioni Amos , Sturla Laura , Antonellis Anthony , Green Eric D. , Gershoni‐Baruch Ruth , Berninsone Patricia M. , Hirschberg Carlos B. , Tonetti Michela
Published: October 2002

Leukocyte adhesion deficiency (LAD) type II is a rare autosomal recessive syndrome characterized by recurrent infections, typical dysmorphic features, the Bombay blood phenotype and severe growth and psychomotor retardation. It is attributed to a general absence of fucosylated glycans on the cell surface. Three Arab Israeli patients and one Turkish child have been reported so far. The primary defect in a specific GDP‐L‐fucose transporter of the Golgi apparatus has been disclosed recently. All three children reported by us are homozygous for one single founder mutation, different from that reported in the Turkish child. The amount of mRNA of the GDP‐L‐fucose transporter in cells from Arab patients and their parents are comparable to controls. Genotype/phenotype correlation studies show that the two different mutations are distinguished by differences in response to fucose supplementation and in the clinical phenotypes. © 2002 Wiley‐Liss, Inc.
SNPs in the CpG island of NAP1L2: A possible link between DNA methylation and neural tube defects?
Authors: Rogner Ute Christine , Danoy Patrick , Matsuda Fumihiko , Moore Gudrun Elizabeth , Stanier Philip , Avner Philip
Published: October 2002

Deletion of the murine X‐linked Nap1l2 gene causes lethality from midgestation onwards. The affected embryos exhibit neural tube defects (NTDs) closely resembling spina bifida and anencephaly in humans. X‐linked familial and spontaneous cases of NTD were analyzed for sequence alterations in the human NAP1L2. No differences were found in the familial cases. However, a number of single nucleotide polymorphisms (SNPs) within the 5′ region of NAP1L2 were identified both in cases of spontaneous NTD and in normal controls. Most of these SNPs lead to the replacement of guanidines or cytosines within a CpG island that is conserved between the human and the mouse promoter regions. Demethylation in vitro activates Nap1l2 transcriptional activity, suggesting the importance of the CpG island in regulating the activity of the Nap1l2/NAP1L2 genes, and the potential importance of the polymorphisms in modifying their transcriptional activity. NAP1L2/Nap1l2 expression may therefore depend on the genetic‐environmental factors that are frequently associated with NTDs. © 2002 Wiley‐Liss, Inc.
Report of a child with aortic aneurysm, orofacial clefting, hemangioma, upper sternal defect, and Marfanoid features: Possible PHACE syndrome
Authors: Slavotinek Anne M. , Dubovsky Elizabeth , Dietz Harry C. , Lacbawan Felicitas
Published: October 2002

We report a female patient who had a scalp hemangioma, a cleft uvula, an upper sternal defect, pectus excavatum, arachnodactyly, pes planus, and joint hypermobility. She had rupture of an aortic aneurysm after minor trauma at 11 years of age. At 17 years of age, elective repair of a dilated, ectatic aorta was complicated by cerebral ischemia. Other vascular abnormalities in the proband included an aneurysm of the left subclavian artery, atresia of the right carotid artery, and calcified cerebral aneurysms. We believe that the proband's physical anomalies are best described by the PHACE (posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities) phenotypic spectrum. This spectrum of physical anomalies also includes sternal clefting and hemagiomas as part of the sternal malformation/vascular dysplasia (SM/VD) association, as found in our patient, and the acronym PHACES has also been used. We consider that the PHACE phenotypic spectrum is likely to be broader than previously recognized and includes orofacial clefting and aortic dilatation and rupture. Our patient also had skeletal anomalies that lead to consideration of Marfan syndrome as a diagnosis. It should be recognized that there is clinical overlap between PHACE syndrome and Marfan syndrome when aortic dilatation is present. We would also like to emphasize the minor nature of the cutaneous findings in our patient despite her severe vascular complications. This is in contrast to previous reports of large or multiple hemangiomas in PHACE syndrome. Published 2002 Wiley‐Liss, Inc.
Balanced X chromosome inactivation patterns in the Rett syndrome brain
Authors: Shahbazian Mona D. , Sun Yaling , Zoghbi Huda Y.
Published: October 2002

In Rett syndrome (RTT), an X‐linked disorder essentially limited to females, neurological development goes awry. Causing this disarray in neuronal function is a mutated form of a protein known as methyl‐CpG‐binding protein 2 (MeCP2). Because the MECP2 gene is subject to X chromosome inactivation (XCI) in females, a number of studies have addressed whether the percentage of cells inactivating the normal vs. mutant chromosome in heterozygous females influences the phenotypic outcome of MECP2 mutations. Because most of these studies measured XCI in peripheral blood, however, interpretation of the results requires the assumption that XCI patterns in blood are representative of those in the brain, the primarily affected tissue. Here, we have analyzed the MECP2 sequence and XCI status in 13 brains of RTT patients. Mutations were identified in nine of the cases, with eight of these representing C to T transitions at CpG dinucleotides, and one being a novel frameshift mutation (765delA). Patterns of XCI were balanced in 10 of 10 cases for which the assay was informative. As previous studies have shown that a majority of RTT patients have balanced XCI patterns in peripheral blood, our results suggest that the pattern in blood is an accurate indicator of XCI patterns in the brain for a majority of cases, but there are some notable exceptions that this study may help explain. Given the correlation between balanced XCI and classic RTT, these results suggest that a certain percentage of neurons expressing the mutant MECP2 gene may be required for RTT to become manifest. © 2002 Wiley‐Liss, Inc.
NovelOCTN2mutations: No genotype–phenotype correlations: Early carnitine therapy prevents cardiomyopathy
Authors: Lamhonwah Anne‐Marie , Olpin Simon E. , Pollitt Rodney J. , Vianey‐Saban Christine , Divry Priscille , Guffon Nathalie , Besley Guy T. N. , Onizuka Russell , De Meirleir Linda J. , Cvitanovic‐Sojat Ljerka , Baric Ivo , Dionisi‐Vici Carlo , Fumic Ksenija , Maradin Miljenka , Tein Ingrid
Published: October 2002

Primary systemic carnitine deficiency or carnitine uptake defect (OMIM 212140) is a potentially lethal, autosomal recessive disorder characterized by progressive infantile‐onset cardiomyopathy, weakness, and recurrent hypoglycemic hypoketotic encephalopathy, which is highly responsive toL‐carnitine therapy. Molecular analysis of theSLC22A5(OCTN2) gene, encoding the high‐affinity carnitine transporter, was done in 11 affected individuals by direct nucleotide sequencing of polymerase chain reaction products from all 10 exons. Carnitine uptake (at Km of 5 μM) in cultured skin fibroblasts ranged from 1% to 20% of normal controls. Eleven mutations (delF23, N32S, and one 11‐bp duplication in exon 1; R169W in exon 3; a donor splice mutation [IVS3+1 G > A] in intron 3; frameshift mutations in exons 5 and 6; Y401X in exon 7; T440M, T468R and S470F in exon 8) are described. There was no correlation between residual uptake and severity of clinical presentation, suggesting that the wide phenotypic variability is likely related to exogenous stressors exacerbating carnitine deficiency. Most importantly, strict compliance with carnitine from birth appears to prevent the phenotype. © 2002 Wiley‐Liss, Inc.
Cytochrome c oxidase deficiency
Authors: Shoubridge Eric A.
Published: October 2002

Cytochrome c oxidase (COX) is the terminal enzyme of the mitochondrial respiratory chain, catalyzing the transfer of electrons from reduced cytochrome c to molecular oxygen. It is composed of 13 structural subunits, three of which are encoded in mtDNA and form the catalytic core of the enzyme. In addition to these structural subunits, a large number of accessory factors are necessary for the assembly and maintenance of the active holoenzyme complex. Most isolated COX deficiencies are inherited as autosomal recessive disorders; mutations in the mtDNA‐encoded COX subunit genes are relatively rare. These mutations are associated with a wide spectrum of clinical phenotypes ranging from isolated myopathy to multisystem disease, with onset from late childhood to adulthood. Autosomal recessive COX deficiencies generally have a very early age of onset and a fatal outcome. Several clinical presentations have been described including Leigh Syndrome, hypertrophic cardiomyopathy and myopathy, and fatal infantile lactic acidosis. Surprisingly, mutations in the nuclear‐encoded structural COX subunits have not been found in association with any of these phenotypes. Mutations have, however, been identified in several COX assembly factors: SURF1 (Leigh Syndrome), SCO2 (hypertrophic cardiomyopathy), SCO1 (hepatic failure, ketoacidotic coma), and COX10 (encephalopathy, tubulopathy). As all of these assembly factors are ubiquitously expressed, the molecular basis for the different clinical presentations remains unexplained. Although the genetic defects in the majority of patients with COX deficiency are unknown, it is likely that most will be solved in the near future using functional complementation techniques. © 2001 Wiley‐Liss, Inc.
Clinical spectrum and diagnosis of mitochondrial disorders
Authors: Munnich Arnold , Rustin Pierre
Published: October 2002

Respiratory chain deficiencies have long been regarded as neuromuscular diseases mostly originating from mutations in the mitochondrial DNA. Actually, oxidative phosphorylation, i.e., adenosine triphosphate (ATP) synthesis‐coupled electron transfer from substrate to oxygen through the respiratory chain, does not only occur in the neuromuscular system. For this reason, a respiratory chain deficiency can theoretically give rise to any symptom, in any organ or tissue, at any age and with any mode of inheritance, owing to the dual genetic origin of respiratory chain enzymes (nuclear DNA and mitochondrial DNA). In recent years, it has become increasingly clear that genetic defects of oxidative phosphorylation account for a large variety of clinical symptoms in both childhood and adulthood. Diagnosis of a respiratory chain deficiency is difficult initially when only one symptom is present, and easier when additional, seemingly unrelated, symptoms are observed. The clinical heterogeneity is echoed by the genetic heterogeneity illustrated by the increasing number of nuclear genes that have been shown to be involved in these diseases. In the absence of clear‐cut genotype‐phenotype correlations and in front of the large number of possibly involved genes, biochemical analyses are still the cornerstone of the diagnosis of this condition. © 2001 Wiley‐Liss, Inc.
Mitochondrial DNA mutations in human disease
Authors: DiMauro Salvatore , Schon Eric A.
Published: October 2002

The small, maternally inherited mitochondrial DNA (mtDNA) has turned out to be a Pandora's box of pathogenic mutations: 13 years into the era of “molecular mitochondrial medicine,” more than 100 pathogenic point mutations and innumerable rearrangements have been associated with a striking variety of multisystemic as well as tissue‐specific human diseases. After reviewing the principles of mitochondrial genetics, we consider disorders due to mutations in genes affecting mitochondrial protein synthesis and disorders due to mutations in protein‐coding genes. In contrast to the remarkable progress in our understanding of etiology, pathogenesis is only partially explained by the rules of mitochondrial genetics and remains largely unclear. We review recent progress in prenatal diagnosis, epidemiology, and in the development of animal models harboring mtDNA mutations. © 2001 Wiley‐Liss, Inc.
Respiratory chain complex I deficiency
Authors: Triepels R.H. , Van Den Heuvel L.P. , Trijbels J.M. , Smeitink J.A.
Published: October 2002

Oxidative phosphorylation disorders make a contribution of 1 per 10,000 live births in man, of which isolated complex I deficiency is frequently the cause. Complex I, or NADH:ubiquinone oxidoreductase, is the largest multi‐protein enzyme complex of the mitochondrial electron transfer chain. In complex I deficiency, various clinical phenotypes have been recognized, often resulting in multi‐system disorders with a fatal outcome at a young age. Recent advances in complex I deficiency, regarding clinical, biochemical, and molecular aspects are described. However, the genetic causes of about 60% of complex I deficiency remain unclear. As a consequence, further research will be needed to clarify the genetic defects in the remaining cases. Novel strategies in which interesting non‐structural nuclear‐encoded disease‐causing genes may be found, as well as the molecular genetic composition of human complex I, are presented. © 2001 Wiley‐Liss, Inc.
Button osteoma: Its etiology and pathophysiology
Authors: Eshed Vered , Latimer Bruce , Greenwald Charles M. , Jellema Lyman M. , Rothschild Bruce M. , Wish‐Baratz Susanne , Hershkovitz Israel
Published: October 2002

The present study investigates a circumscribed bony overgrowth on the cranial vault, known as button osteoma (BtO) and referred to here as button lesion (BtL). We discuss its anthropological implications. Data on its histology, location, and population distribution (by age, race, and gender) are provided.

Microscopically, BtL is composed of well‐organized dense lamellated bone which is poorly vascularized and with very few osteocytes. It forms a dome‐shaped roof over an underlying diploeized area which includes the ectocranial table.

The frequency of BtL is similar in modern (37.6%) and archaeological (41.1%) populations, in blacks, whites, males, and females, and correlates with age. It is rare in nonhuman primates. Fifty‐five percent of the human skulls studied by us had BtL only on the parietal, 23.6% on the frontal, and 3.6% on the occipital bones. Fifteen percent had BtL on both the frontal and parietal bones. No lateral preference was found. Most skulls with BtL (64.1%) had only one lesion, 20.4% had two BtL, and 15.4% demonstrated multiple BtL. The average number of button osteomas on an affected skull was 1.97. The frequency of large osteomas (0.5–1.0 cm) was similar in young and old age groups.

The demographic characteristics of BtL, mainly its high frequency among ancient and modern populations, its independence of sex and race, its scarcity in other primates, and the fact that its macro‐ and microstruture are indicative of an hamartoma (and not an osteoma or exostosis) suggest an evolutionary background to the phenomenon. Am J Phys Anthropol 118:217–230, 2002. © 2002 Wiley‐Liss, Inc.
The prevalence of narcolepsy among Chinese in Hong Kong
Authors: Wing Yun‐Kwok , Li Raymond Hiu‐Yeung , Lam Ching‐Wan , Ho Crover Kwok‐Wah , Fong Samson Yuk‐Yat , Leung Tony
Published: October 2002

Narcolepsy is a lifelong, crippling sleep disorder. Although the discovery of the hypocretin system has been a breakthough in genetics, the epidemiological aspects of narcolepsy remain elusive. Ethnic predisposition was suggested to partially account for the 2,500‐fold difference in the reported prevalence rates of narcolepsy between Japanese (0.59%) and Israeli Jews (0.00023%). We carried out a general population study, conducting a random telephone survey with a structured questionnaire, which included a validated screening instrument (a Chinese version of the Ullanlinna Narcolepsy Scale). It was followed by clinical‐polysomnographic‐HLA confirmation of the subjects determined to be positive for narcolepsy based on the questionnaire. Of 9,851 subjects interviewed, 28 subjects (0.28%, 58% female) were screened positive. Ninety percent had a second detailed interview, 64% had HLA typing, and over half of them had a sleep assessment. Only three subjects were found to have genuine narcolepsy. The most common nonnarcolepsy diagnoses were sleep apnea syndrome and sleep‐wake schedule disorder. The prevalence rate of narcolepsy in Southern (Hong Kong) Chinese was found to be 0.034% (95% confidence interval = 0.010–0.117%). All available narcoleptic subjects were HLA DRB1‐1501 positive and 50% were DQB1‐0602 positive. The prevalence rate of narcolepsy among Chinese is comparable to the rates for other populations in studies with stringent epidemiological designs, suggesting that major cross‐ethnic differences in the prevalence rates of narcolepsy previously reported likely resulted from methodological limitations.
Seizure‐associated hippocampal volume loss: A longitudinal magnetic resonance study of temporal lobe epilepsy
Authors: Briellmann Regula S. , Berkovic Samuel F. , Syngeniotis Ari , King Mark A. , Jackson Graeme D.
Published: October 2002

This longitudinal quantitative magnetic resonance imaging study of 24 patients with mild temporal lobe epilepsy shows an ipsilateral hippocampal volume decrease of 9% (range, −30 to +0.5%; p = 0.002, paired t test) over a period of 3.5 ± 0.7 years. The hippocampal volume loss was correlated to the number of generalized seizures between the scans (p = 0.0007, r = 0.6), suggesting seizure‐associated hippocampal damage.
Electrophysiological findings in X‐linked myopathy with excessive autophagy
Authors: Jääskeläinen Satu K. , Juel Vern C. , Udd Bjarne , Villanova Marcello , Liguori Rocco , Minassian Berge A. , Falck Björn , Niemi Pekka , Kalimo Hannu
Published: October 2002

We report electrophysiological features and magnetic resonance imaging muscle findings in 4 patients and 1 female carrier of X‐linked myopathy with excessive autophagy. Motor units were polyphasic with high mean amplitude and normal duration. The thigh muscles were most severely involved, but myotonic discharges were abundant in both clinically affected and unaffected muscles. Along with the clinicopathological features, these electrophysiological findings distinguish X‐linked myopathy with excessive autophagy from other limb‐girdle myopathies.
Sensory training for patients with focal hand dystonia
Authors: Zeuner Kirsten E. , Bara‐Jimenez William , Noguchi Patricia S. , Goldstein Susanne R. , Dambrosia James M. , Hallett Mark
Published: October 2002

Some patients with focal hand dystonia have impaired sensory perception. Abnormal sensory processing may lead to problems with fine motor control. For patients with focal hand dystonia who demonstrate sensory dysfunction, sensory training may reverse sensory impairment and dystonic symptoms. We studied the efficacy of learning to read braille as a method of sensory training for patients with focal hand dystonia. Sensory spatial discrimination was evaluated in 10 patients who had focal hand dystonia and 10 age‐ and gender‐matched controls with a spatial acuity test (JVP domes were used in this test). Clinical dystonia evaluation included the Fahn dystonia scale and time needed to write a standard paragraph. Each individual was trained in braille reading at the grade 1 level for 8 weeks, between 30 and 60 minutes daily, and was monitored closely to ensure that reading was done regularly. Both controls and patients demonstrated improvement on the spatial acuity test. Patients showed a significant mean difference from baseline to 8 weeks on the Fahn dystonia scale. Sixty percent of the patients shortened the time they needed to write a standard paragraph. Improved sensory perception correlated positively with improvement on the Fahn dystonia scale. We conclude that training in braille reading improves deficits in spatial discrimination and decreases disability in patients with focal hand dystonia.
Angiostrongylus cantonensis infection mimicking a spinal cord tumor
Authors: Petjom Supinda , Chaiwun Benjaporn , Settakorn Jongkolnee , Visrutaratna Pannee , Rangdaeng Samreung , Thorner Paul S.
Published: October 2002

Angiostrongylus cantonensis is the most common cause of eosinophilic meningitis and meningoencephalitis. Almost all cases are self‐limiting and are diagnosed by cerebrospinal fluid eosinophilia and enzyme‐linked immunosorbent assay; pathology reports are restricted to postmortem samples from lethal cases. We report on what we believe is the first case of A. cantonensis infection diagnosed by biopsy in a living patient. The spinal cord was biopsied because of the unusual clinical presentation of a myelopathy without meningeal symptoms, together with a mass lesion that was clinically and radiologically diagnosed as a spinal cord tumor.
Preferential loss of paternal 19q, but not 1p, alleles in oligodendrogliomas
Authors: Sanson Marc , Leuraud Pascal , Marie Yannick , Delattre Jean‐Yves , Hoang‐Xuan Khê
Published: October 2002

Portions of chromosomes 1p and 19q, which are frequently deleted in oligodendrogliomas, are subject to genomic imprinting, suggesting that the putative tumor suppressor genes could be monoallelically expressed. The parental origins of 1p and 19q allele losses were determined in 6 cases of pure oligodendroglioma. An equilibrated parental loss (3 maternal and 3 paternal) was found for 1p deletions. In contrast, 19q deletions always occurred on the paternal copy (p = 0.015). In this setting, a cloning strategy based on a search for homozygous deletion or mutation of the remaining allele would be appropriate for identifying the tumor suppressor gene located on 1p but inappropriate for identifying the presumably monoallelically expressed tumor suppressor gene located on 19q.
Selective loss of cholinergic sudomotor fibers causes anhidrosis in Ross syndrome
Authors: Sommer Claudia , Lindenlaub Thies , Zillikens Detlef , Toyka Klaus V. , Naumann Markus
Published: October 2002

Ross syndrome consists of segmental hyperhidrosis with widespread anhidrosis, Adie syndrome, and areflexia. The cause of this disorder is unknown. Selective degeneration of cholinergic fibers or of neural crest–derived structures has been suggested. We present clinical and skin biopsy data of 4 patients, providing evidence of reduced cholinergic sweat gland innervation in hypohidrotic skin by morphometric analysis. These findings indicate a selective degenerative process of the cholinergic sudomotor neurons.
Normokalemic periodic paralysis revisited: Does it exist?
Authors: Chinnery Patrick F. , Walls Timothy J. , Hanna Michael G. , Bates David , Fawcett Peter R. W.
Published: October 2002

Normokalemic periodic paralysis (normoKPP) is well established in the literature, but there are doubts as to whether it exists as a discrete entity. Retrospective clinical and molecular analysis has confirmed suspicions that most normoKPP families actually have a variant of hyperkalemic periodic paralysis (hyperKPP) due to a mutation of the muscle‐specific sodium channel gene (SCN4A). However, the original normoKPP family described by Poskanzer and Kerr (Poskanzer DC, Kerr DNS. A third type of periodic paralysis, with normokalemia and favourable response to sodium chloride. Am J Med 1961;31:328–342) has remained unchallenged. We identified the Met1592Val mutation of SCN4A in an affected descendent of this original normoKPP family. This is the final piece in the puzzle: normoKPP is actually a variant of hyperKPP and is not a distinct disorder.
Novel antibodies to synuclein show abundant striatal pathology in Lewy body diseases
Authors: Duda John E. , Giasson Benoit I. , Mabon Meghann E. , Lee Virginia M.‐Y. , Trojanowski John Q.
Published: October 2002

Intracytoplasmic inclusions composed of α‐synuclein (α‐syn) are characteristic of neurodegenerative Lewy body disorders. Using novel monoclonal antibodies raised against altered α‐syn, we uncovered an unprecedented and extensive burden of α‐syn pathology in the striatum of Lewy body disorders. The highest density of striatal pathology was observed in patients with a combination of Alzheimer's disease and dementia with Lewy bodies or pure dementia with Lewy bodies, and these α‐syn aggregates may contribute to the parkinsonism seen in these disorders.
Swelling kinetics of a hydrogel of poly(ethylene glycol) and poly(acrylamide‐co‐styrene)
Authors: Bajpai A. K. , Shrivastava Mudita
Published: October 2002

A hydrogel incorporating the hydrophilic polymer poly(ethylene glycol) and a copolymer of acrylamide and styrene was synthesized, and the dynamics of the water‐sorption process were studied. The effects of the composition of the hydrogel and the temperature of the swelling medium were investigated with respect to the water‐sorption characteristics of the hydrogel, and the kinetic parameters, including the swelling exponent and diffusion constant, were evaluated. The hydrogel was also judged for the antithrombogenic property of its surface. The experimental findings were explained on the basis of the core–shell polymeric structure of the hydrogel. © 2002 Wiley Periodicals, Inc. J Appl Polym Sci 85: 1419–1428, 2002
Grafting of cyclodextrins onto polypropylene nonwoven fabrics for the manufacture of reactive filters. III. Study of the sorption properties
Authors: Martel Bernard , Le Thuaut Philippe , Bertini Sabrina , Crini Grégorio , Bacquet Maryse , Torri Giangiacomo , Morcellet Michel
Published: October 2002

Polypropylene nonwoven supports carrying α‐, β‐, and γ‐cyclodextrins (CDs) were synthezised. The synthesis consisted of grafting glycidyl methacrylate (GMA) onto the fibers and reacting CDs with epoxide groups in the second step. This article concerns the sorption capacity of these supports toward organic pollutant models of 2‐naphtol, 4‐nitrophenol, pentachlorophenol, and (2,4‐dichlorophenoxy) acetic acid in static (batch) or dynamic (filtration) conditions. The results confirmed that the grafting of CDs onto the supports gave them their specific sorption properties. Besides, it was observed that the best performances were attained when the GMA add‐on was in the range of 76–113 % wt and the CD content was 87–118 μmol/g. We also evidenced that the interactions between the organic substrates and the filter occurred through specific interactions with CDs (host–guest inclusion compounds) and that to a lesser extent, nonspecific sorption phenomena also occurred. The data showed that the affinity between the pollutants with three different grafted CDs was not in agreement with the complex formation constants measured in homogeneous systems. Furthermore, the slow kinetics of exchange between the liquid and solid phases was a limiting factor that should prevent the use of the filters at elevated flow rates. At last, the great advantage of these filters is that they could undergo several cycles of use, thanks to intermediate regeneration steps with water–organic solvent mixtures. © 2002 Wiley Periodicals, Inc. J Appl Polym Sci 85: 1771–1778, 2002
Grafting of maleic anhydride onto polypropylene by reactive extrusion
Authors: Bettini S. H. P. , Agnelli J. A. M.
Published: October 2002

Maleic anhydride grafting onto polypropylene was conducted in a twin‐screw extruder according to an experimental design in which the maleic anhydride and peroxide concentrations were varied. The modified polypropylene was characterized by FTIR spectroscopy, melt‐flow index measurements, size‐exclusion chromatography, differential scanning calorimetry, and nuclear magnetic resonance. The results showed that only the independent variable peroxide concentration influenced the amount of reacted maleic anhydride, whereas the two variables studied influenced the molecular weight of the grafted polypropylene. © 2002 Wiley Periodicals, Inc. J Appl Polym Sci 85: 2706–2717, 2002
Preparation and properties of chemical cellulose from ascidian tunic and their regenerated cellulose fibers
Authors: Koo Young‐Seok , Wang Young‐Soo , You Sang‐Hoon , Kim Han‐Do
Published: October 2002

Chemical cellulose (dissolving pulp) was prepared from ascidian tunic by modified paper‐pulp process (prehydrolysis with acidic aqueous solution of H2SO4, digestion with alkali aqueous solution of NaOH/Na2S, bleaching with aqueous NaOCl solution, and washing with acetone/water). The α‐ cellulose content and the degree of polymerization (DPw) of the chemical cellulose was about 98 wt % and 918, respectively. The Japanese Industrial Standard (JIS) whiteness of the chemical cellulose was about 98%. From the X‐ray diffraction patterns and 13C‐NMR spectrum, it was found that the chemical cellulose obtained here has cellulose Iβ crystal structure. A new regenerated cellulose fiber was prepared from the chemical cellulose by dry–wet spinning using N‐methylmorpholine‐ N‐oxide (NMMO)/water (87/13 wt %) as solvent. The new regenerated cellulose fiber prepared in this study has a higher ratio of wet‐to‐dry strength (<0.97) than commercially regenerated cellulose fibers. © 2002 Wiley Periodicals, Inc. J Appl Polym Sci 85: 1634–1643, 2002.
LDPE/carbon black conductive composites: Influence of radiation crosslinking on PTC and NTC properties
Authors: Xie Hongfeng , Deng Pengyang , Dong Lisong , Sun Jiazhen
Published: October 2002

The electrical resistivity of low‐density polyethylene/carbon black composites irradiated by 60Co γ‐rays was investigated as a function of temperature. The experimental results obtained by scanning electron microscopy, solvent extraction techniques, and pressure‐specific volume‐temperature analysis techniques showed that the positive temperature coefficient (PTC) and negative temperature coefficient (NTC) effects of the composites were influenced by the irradiation dose, network forming (gel), and soluble fractions (sol). The NTC effect was effectively eliminated when the radiation dose reached 400 kGy. The results showed that the elimination of the NTC effect was related to the difference in the thermal expansion of the gel and sol regions. The thermal expansion of the sol played an important role in both increasing the PTC intensity and decreasing the NTC intensity at 400 kGy. © 2002 Wiley Periodicals, Inc. J Appl Polym Sci 85: 2742–2749, 2002
Functionalization of sisal fibers and high‐density polyethylene by cold plasma treatment
Authors: Martin Adriana R. , Manolache S. , Denes Ferencz S. , Mattoso Luiz H. C.
Published: October 2002

Chopped sisal fibers and finely powdered high‐density polyethylene were surface functionalized using dichlorosilane (DS) under radio frequency (RF)‐plasma conditions and characterized by electron spectroscopy for chemical analysis (ESCA) and fluorescence labeling techniques. A high‐capacity (10 L), rotating, 13.56 MHz, electrodeless plasma installation, specially designed to allow the uniform surface modification of powdery and particulate matter of irregular shape, was used. A three‐factor fractional experimental design was employed to evaluate the effect of RF‐power, pressure, and reaction time on the ESCA‐based relative atomic composition of plasma‐treated samples. It was demonstrated that SiHxCly functionalities are present on plasma‐exposed surfaces and these functionalization reactions can be controlled by selecting proper plasma parameters. © 2002 Wiley Periodicals, Inc. J Appl Polym Sci 85: 2145–2154, 2002
NMR study of poly(vinylpyrrolidone)/poly(ethylene oxide) blends
Authors: Diniz Teresinha M. F. F. , Tavares Maria I. B.
Published: October 2002

Development of polymeric blends has become very important for polymer industries because they have been shown to be successful and versatile alternatives to obtain new polymers. In this work binary blends formed by poly(vinylpyrrolidone) (PVP) and poly(ethylene oxide) (PEO) were studied by solution and solid‐state NMR to determine their physical interaction, homogeneity, and compatibility for use as membranes to separate water/alcohol. The NMR results allowed us to acquire information on the microstructure and molecular dynamic behavior of polymer blends. From the NMR solution it was possible to evaluate the microstructure: PVP presented a preferential syndiotactic distribution sequence and PEO presented two regions, one crystalline and the other amorphous. Considering the solid‐state NMR results it was possible to evaluate the molecular dynamics and all binary blends, showing that PEO behaves as a plasticizer; some intermolecular interaction was also observed. An important point was to evaluate the microstructure of the carbonyl PVP using cross polarization/magic‐angle spinning (CP/MAS) and CP/MAS/dipolar decoupling that was not observed before. © 2002 Wiley Periodicals, Inc. J Appl Polym Sci 85: 2820–2823, 2002
Morphological parameters in varieties of silk fibers determined by small‐angle X‐ray scattering
Authors: Londono J. David , Annadurai V. , Gopalkrishne Urs R. , Somashekar R.
Published: October 2002

The small‐angle X‐ray scattering intensity data recorded from silk fibers were compared with the simulated data obtained from a linear paracrystalline model. For this purpose, an exponential distribution function for the amorphous and crystalline phase lengths was used. There are significant changes in phase lengths because of amino acid compositional changes in different families of silk fibers. © 2002 Wiley Periodicals, Inc. J Appl Polym Sci 85: 2382–2388, 2002
Synthesis of bismaleimide resin containing the poly(ethylene glycol) side chain: Curing behavior and thermal properties
Authors: Chian K. S. , Du X. Y. , Goy H. A. , Feng J. L. , Yi S. , Yue C. Y.
Published: October 2002

Two maleimido end‐capped poly(ethylene glycol) (m‐PEG) of different molecular weights were synthesized and blended at various proportions with bismaleimide resin (4,4′‐bismaleimido diphenylmethane) (BDM). The curing behavior and the thermal properties of the m‐PEG/BDM blends were studied and presented here. It was found that the addition of m‐PEG enhanced the processability of the BDM resin significantly. The processing window of the BDM resin was increased from approximately 20 to 80°C. The addition of m‐PEG modified resins, however, resulted not only in the reduction in the thermal stability of the blended BDM resin but also elevation of the coefficients of thermal expansion. The changes in thermal/mechanical properties of the blends were found to be proportional to the amounts of m‐PEG incorporated. It was observed that the curing behavior, and thermal and mechanical properties, of the blends were independent of the molecular weight of the PEG segment. © 2002 Wiley Periodicals, Inc. J Appl Polym Sci 85: 2935–2945, 2002
PVA/PAA thermo‐induced hydrogel fiber: Preparation and pH‐sensitive behavior in electrolyte solution
Authors: Fei Jianqi , Zhang Zipeng , Zhong Leilan , Gu Lixia
Published: October 2002

The preparation of thermo‐induced hydrogel fibers composed of poly(vinyl alcohol) and poly(acrylic acid) is presented. The hydrogel fiber was prepared by extruding the spinning dope from in situ polymerization of acrylic acid in the presence of PVA into a coagulating bath of saturated ammonium sulfate aqueous solution. The network was formed by thermally heating the dried fibers under vacuum. The final hydrogel fibers exhibit pH‐sensitive behavior and show a hysteresis loop in the pH range from 3.0 to 12.0. The pH value, at which the swelling elongation ratio of the fiber had a jump, shifted to a lower value with increasing PAA content within the network. Increasing the heating temperature and time for the fibers, decreased the swelling elongation ratio, and the jump point pH shifted to higher pH value. The oscillatory swelling/contracting behavior of the hydrogel fiber exhibited a good reversible pH‐responsive property. Transmission Electron Microscopy (TEM) showed that PVA and PAA have good compatibility and give a relative independent interpenetrating network. Scanning Electron Microscopy (SEM) showed that in the surface of the fibers there were microholes and ditches due to some diffusion of PAA into the coagulating solution. © 2002 Wiley Periodicals, Inc. J Appl Polym Sci 85: 2423–2430, 2002