bluuebunny/crossref_metadata_2025 huggingface dataset:
Identification of a 3-Deoxy-
d
-
manno
-Octulosonic Acid Biosynthetic Operon in
Moraxella catarrhalis
and Analysis of a KdsA-Deficient Isogenic Mutant
Authors: Luke Nicole R. , Allen Simon , Gibson Bradford W. , Campagnari Anthony A.
Published: October 2003

Lipooligosaccharide (LOS), a predominant surface-exposed component of the outer membrane, has been implicated as a virulence factor in the pathogenesis of
Moraxella catarrhalis
infections. However, the critical steps involved in the biosynthesis and assembly of
M. catarrhalis
LOS currently remain undefined. In this study, we used random transposon mutagenesis to identify a 3-deoxy-
d
-
manno
-octulosonic acid (KDO) biosynthetic operon in
M. catarrhalis
with the gene order
pyrG
-
kdsA
-
eno
. The lipid A-KDO molecule serves as the acceptor onto which a variety of glycosyl transferases sequentially add the core and branch oligosaccharide extensions for the LOS molecule. KdsA, the KDO-8-phosphate synthase, catalyzes the first step of KDO biosynthesis and is an essential enzyme in gram-negative enteric bacteria for maintenance of bacterial viability. We report the construction of an isogenic
M. catarrhalis kdsA
mutant in strain 7169 by allelic exchange. Our data indicate that an LOS molecule consisting only of lipid A and lacking KDO glycosylation is sufficient to sustain
M. catarrhalis
survival in vitro. In addition, comparative growth and susceptibility assays were performed to assess the sensitivity of 7169kdsA11 compared to that of the parental strain. The results of these studies demonstrate that the native LOS molecule is an important factor in maintaining the integrity of the outer membrane and suggest that LOS is a critical component involved in the ability of
M. catarrhalis
to resist the bactericidal activity of human sera.
Modulation of T-Cell Costimulation as Immunotherapy or Immunochemotherapy in Experimental Visceral Leishmaniasis
Authors: Murray Henry W. , Lu Cristina M. , Brooks Elaine B. , Fichtl Richard E. , DeVecchio Jennifer L. , Heinzel Frederick P.
Published: October 2003

CD40 ligand (CD40L)-deficient C57BL/6 mice failed to control intracellularLeishmania donovanivisceral infection, indicating that acquired resistance involves CD40-CD40L signaling and costimulation. Conversely, in wild-type C57BL/6 and BALB/c mice with established visceral infection, injection of agonist anti-CD40 monoclonal antibody (MAb) induced killing of ∼60% of parasites within liver macrophages, stimulated gamma interferon (IFN-γ) secretion, and enhanced mononuclear cell recruitment and tissue granuloma formation. Comparable parasite killing was also induced by MAb blockade (inhibition) of cytotoxic T lymphocyte antigen-4 (CTLA-4) which downregulates separate CD28-B7 T-cell costimulation. Optimal killing triggered by both anti-CD40 and anti-CTLA-4 required endogenous IFN-γ and involved interleukin 12. CD40L−/−mice also failed to respond to antileishmanial chemotherapy (antimony), while in normal animals, anti-CD40 and anti-CTLA-4 synergistically enhanced antimony-associated killing. CD40L-CD40 signaling regulates outcome and response to treatment of experimental visceral leishmaniasis, and MAb targeting of T-cell costimulatory pathways (CD40L-CD40 and CD28-B7) yields macrophage activation and immunotherapeutic and immunochemotherapeutic activity.
Phosphatidylcholine-Specific Phospholipase C and Sphingomyelinase Activities in Bacteria of theBacillus cereusGroup
Authors: Pomerantsev A. P. , Kalnin K. V. , Osorio M. , Leppla S. H.
Published: October 2003

Bacillus anthracisis nonhemolytic, even though it is closely related to the highly hemolyticBacillus cereus.Hemolysis byB. cereusresults largely from the action of phosphatidylcholine-specific phospholipase C (PC-PLC) and sphingomyelinase (SPH), encoded by theplcandsphgenes, respectively. InB. cereus, these genes are organized in an operon regulated by the global regulator PlcR.B. anthraciscontains a highly similar cereolysin operon, but it is transcriptionally silent because theB. anthracisPlcR is truncated at the C terminus. Here we report the cloning, expression, purification, and enzymatic characterization of PC-PLC and SPH fromB. cereusandB. anthracis.We also investigated the effects of expressing PlcR on the expression ofplcandsph. InB. cereus, PlcR was found to be a positive regulator ofplcbut a negative regulator ofsph. Replacement of theB. cereus plcRgene by its truncated orthologue fromB. anthraciseliminated the activities of both PC-PLC and SPH, whereas introduction intoB. anthracisof theB. cereus plcRgene with its own promoter did not activate cereolysin expression. Hemolytic activity was detected inB. anthracisstrains containing theB. cereus plcRgene on a multicopy plasmid under control of the strongB. anthracisprotective antigen gene promoter or in a strain carrying a multicopy plasmid containing the entireB. cereus plc-sphoperon. Slight hemolysis and PC-PLC activation were found when PlcR-producingB. anthracisstrains were grown under anaerobic-plus-CO2or especially under aerobic-plus-CO2conditions. Unmodified parentalB. anthracisstrains did not demonstrate obvious hemolysis under the same conditions.
Detection ofN-(3-Oxohexanoyl)-l-Homoserine Lactone in Mice Infected withYersinia enterocoliticaSerotype O8
Authors: Jacobi Christoph A. , Bach Alexandra , Eberl Leo , Steidle Anette , Heesemann Jürgen
Published: October 2003

Yersinia enterocoliticasynthesizesN-acyl-l-homoserine lactone (AHL) signal molecules via the LuxR-LuxI homologues YenR-YenI. In this study we checked two prototypes of mouse-virulentY.enterocoliticaserotype O8 strains WA-314 and 8081 for AHL production in vitro and in vivo (mouse infection model). We used thin-layer chromatography in combination with theEscherichia coliAHL biosensor to identify the AHL species produced. We detected only OHHL [N-(3-oxohexanoyl)-l-homoserine lactone] and not HHL (N-hexanoyl-l-homoserine lactone) produced byY.enterocoliticaO8 in culture supernatant or infected mouse tissue. This is the first report demonstrating AHL production by yersiniae during infection.
Legionella pneumophilaSuppresses Macrophage Interleukin-12 Production by Activating the p42/44 Mitogen-Activated Protein Kinase Cascade
Authors: Matsunaga Kazuto , Yamaguchi Hiroyuki , Klein Thomas W. , Friedman Herman , Yamamoto Yoshimasa
Published: October 2003

A possible involvement of the mitogen-activated protein (MAP) kinase cascade in the inhibition of macrophage interleukin-12 (IL-12) production byLegionella pneumophilainfection was examined. The results of MAP kinase inhibition by p42/44 and p38 MAP kinase inhibitors and of p42/44 MAP kinase activity assays indicate thatL. pneumophilainfection of macrophages causes a selective inhibition of lipopolysaccharide-induced IL-12 production by activating the p42/44 MAP kinase cascade. In addition, it was also revealed that the p38 MAP kinase may be important for the production of IL-12 but not for the inhibition caused byL. pneumophilainfection.
Risk Factors of Stroke Mortality: A 40-Year Follow-Up of the Corfu Cohort from the Seven-Countries Study
Authors: Panagiotakos Demosthenes B. , Chrysohoou Christina , Pitsavos Christos , Menotti Alessandro , Dontas Anastasios , Skoumas John , Stefanadis Christodoulos , Toutouzas Pavlos
Published: October 2003

The present work evaluates several risk factors for stroke mortality based on a 40-year follow-up of the Corfu cohort from the Seven-Countries Study. The population studied in this analysis consisted of 529 rural men (49.7 ± 5.7 years old) enrolled at 1961. The death rate at the end of follow-up was 87.1% (i.e. 461 deaths out of 529 participants). Of 529 cardiovascular-disease-free men at entry, 74 (14%) died because of hemorrhagic or thrombotic stroke. Age (HR = 1.11/year, p < 0.001), pulse pressure (HR = 1.16/5 mm Hg, p < 0.001), total serum cholesterol levels (HR = 0.81/10 mg/dl, p < 0.01) and presence of physical activity (HR = 0.59, p < 0.05), showed a statistically significant association with fatal stroke events. In conclusion, age, pulse pressure levels, physical activity (protective) and total serum cholesterol levels (protective) were significantly related with 40-year stroke mortality.
Parkinson’s Disease and CYP2D6 Polymorphism in Asian Populations: A Meta-Analysis
Authors: Persad Amanda S. , Stedeford Todd , Tanaka Seigo , Chen Liping , Banasik Marek
Published: October 2003

Polymorphism of CYP2D6 and its relationship with the development of Parkinson’s disease (PD) has been controversial. The distribution of the B-mutation of CYP2D6, a mutation that results in the absence of a functional protein, differs by ethnicity and accounts for less than 1% of the ‘poor metabolizer’ phenotype in Asians. Thus, a meta-analysis was conducted to determine if polymorphism, other than the B-mutation, within the CYP2D6 gene confers a greater susceptibility to PD outcome among Asian populations. Eleven studies were identified, two of which were excluded due to unavailability in the English language or availability of the same original data in more detail in another publication. None of the studies showed a statistically significant association between CYP2D6 polymorphism and PD (p < 0.05). The overall odds ratio was 0.84 (95% confidence interval 0.66–1.08). We conclude that among Asian populations, there is no convincing evidence of an association between CYP2D6 polymorphism and the risk of developing PD.
Multiple Sclerosis in Northern Sardinia, Italy: A Methodological Approach for Genetic Epidemiological Studies
Authors: Pugliatti Maura , Sotgiu Stefano , Sadovnick A. Dessa , Yee Irene M.L. , Sotgiu M. Alessandra , Poser Charles M. , Rosati Giulio
Published: October 2003

The etiopathogenesis of multiple sclerosis (MS) remains unclear. However, genetic factors are believed to be important in disease susceptibility. A methodological approach is presented for a population-based study aimed at investigating MS familial incidence and patterns of familial clustering in Northern Sardinia, Italy, with a reported MS prevalence of 150/100,000 population. Patients with MS since 1965 to the present and known to the MS Register for the province of Sassari will be asked to provide genealogical, comorbid and demographic information. Statistical analyses of the familial risk for MS will depend on the completeness of MS ‘age at onset’ data for affected individuals and age at the time family history is obtained (or age at death) for unaffected family members.
Economic Considerations for Health Insurance Coverage of Emerging Genetic Tests
Authors: Giacomini Mita , Miller Fiona , O’Brien Bernie J.
Published: October 2003

Public and private health insurance plans face the question of whether to cover emerging genetic tests for cancer and other diseases. This paper outlines issues in the economic evaluation of new genetic tests, illustrating key methodological issues and policy implications with findings from a comprehensive and systematic review of the 14 full economic evaluations published over the past 5 years that have addressed both the costs and consequences of molecular genetic tests. Key questions for framing an evaluation include: whose viewpoint matters, which costs and consequences are relevant, and to which clinical alternatives should new genetic tests be compared? While economic evaluation research can inform coverage decisions about genetic tests, the coverage decision-making process must also inform economic researchers about the aims, context, and value systems within which genetic tests will be covered and practised.
Validation of the Dutch Short Form of the State Scale of the Spielberger State-Trait Anxiety Inventory: Considerations for Usage in Screening Outcomes
Authors: van der Bij Akke K. , de Weerd Sabina , Cikot Rolf J.L.M. , Steegers Eric A.P. , Braspenning Jozé C.C.
Published: October 2003

Objective: To validate the six-item short form of the state scale of the Spielberger State-Trait Anxiety Inventory (STAI) for usage in screening outcomes in a Dutch population receiving preconception counseling. Methods: Men and women completed the 20-item full form of the STAI before (n = 310) and after preconception counseling (n = 147). Prorated scores of the six-item form were compared to the full form using Pearson’s correlation coefficients and paired t tests. Results: Cronbach’s α for the Dutch translation of the short-form of the STAI-state was 0.83. The short form highly correlates with the full form (r = 0.95). The short form was able to discriminate between different levels of anxiety and was sensitive to change. Conclusion: The Dutch translation of the short form of the STAI-state has good reliability and validity and was found to be useful as a quick tool to evaluate the effectiveness of screening programs on anxiety levels. We believe our results will be applicable to other populations, although this needs to be confirmed in other studies.
Community Diagnosis of Maternal Exposure to Risk Factors for Congenital Defects
Authors: de Lima Garcias Gilberto , Schüler-Faccini Lavínia
Published: October 2003

Objectives: This paper describes a community diagnosis of maternal exposure to risk factors for congenital defects in the population of Pelotas, RS, Brazil (400,000 inhabitants). The authors investigated biological and demographic factors (maternal age, alcohol ingestion and smoking), social and economic factors (family income and type of work), and welfare factors (prenatal care, illnesses during pregnancy, drug therapy, and vaccinations). These factors were matched up with the Decalogue for Prevention of Congenital Defects recommended by the Latin American Collaborative Study of Congenital Malformations. Methods: Data were collected in a population-based study in which mothers were interviewed in the hospitals of Pelotas, RS, during 1995. On each day, the first 3 women to give birth were interviewed in every city hospital. The sample resulted in a total of 3,219 interviewed women. Results and Conclusions: Low-income groups are most exposed. They present the following potential risk factors: (a) outside the ideal age range for pregnancy; (b) fewer prenatal care appointments; (c) no prevention against rubella through vaccinations, and (d) more cigarette smoking. In the high-income groups, the potential risk factors were: (a) drug therapy (more common) and (b) more women working outside the home. We did not find any difference among social classes concerning: (a) illnesses during pregnancy and (b) ingestion of alcohol. When a general risk score was used, this figure was higher in the group of women who were not within ideal age for pregnancy. However, there was no difference among social classes. This has led us to conclude that delayed childbearing is a global risk whereas each social class has its own risks.
Predictive Genetic Testing for Breast Cancer and Huntington’s Disease: The Opinions of Midwives and Nurses in Flanders
Authors: Welkenhuysen Myriam , Evers-Kiebooms Gerry
Published: October 2003

Objective: Investigate attitudes of midwives and nurses regarding predictive testing for hereditary breast cancer and Huntington’s disease. Methods: Mail questionnaires were sent to 119 midwives and 881 nurses. Results: The response rate was 58% for midwives, but only 33% for nurses. The attitude toward predictive testing in an asymptomatic adult was more favourable in case of hereditary breast cancer than in case of Huntington’s disease, mostly because prevention or therapy is unavailable for the latter condition. Prevention of breast cancer through prophylactic mastectomy elicited adverse reactions among 40% of the participants. Predictive testing for these diseases in an adolescent at the own request was also rated favourably while testing in children at the parents’ request was less acceptable. For both diseases, concealing carrier status information from the employer or the insurance company was judged as acceptable by the majority. Conclusion: Our findings contribute to the understanding of nurses’ and especially midwives’ attitudes to predictive testing, which may in turn serve as a basis for genetic education for these groups of health care professionals.
Smooth Muscle Cell Outgrowth from Coronary Atherectomy Specimens in vitro Is Associated with Less Time to Restenosis and Expression of a Key Transcription Factor KLF5/BTEB2
Authors: Sakamoto Hironosuke , Sakamaki Tetsuo , Kanda Tsugiyasu , Hoshino Yo-ichi , Sawada Yoshie , Sato Mahito , Sato Hiroko , Oyama Yuko , Nakano Akihiko , Takase Shin-ichi , Hasegawa Akira , Nagai Ryozo , Kurabayashi Masahiko
Published: October 2003

Atherectomy specimens offer an opportunity to study the biology of coronary artery lesions. We cultured smooth muscle cells (SMCs) from specimens obtained from 24 patients with coronary restenosis after angioplasty to study the relationship between activity of SMCs (in vitro outgrowth) and the time course of restenosis. We also examined expression of a Kruppel-like zinc-finger transcription factor 5 (KLF; also known as BTEB2 and IKLF), which is markedly induced in activated SMCs, in the same specimens. SMC outgrowth was observed in 9 of 24 specimens (37.5%). Restenosis occurred sooner (p < 0.01) in patients whose specimens showed outgrowth compared to those whose specimens showed no outgrowth. Immunostaining for KLF5 was more common in specimens with outgrowth (89 vs. 20%, p < 0.01). These data suggest that the number of activated SMCs in lesions may determine in vitro outgrowth and also affect the time to restenosis.
Visual Outcome in Macular Retinoblastoma Treated with Primary Chemotherapy
Authors: Balasubramanya R. , Pushker Neelam , Bajaj Mandeep S. , Rani Alka , Ghose Supriyo , Arya Laxman S.
Published: October 2003

In this prospective clinical study, we treated 8 patients (8 eyes, 11 tumors) of macular retinoblastoma with chemotherapy alone, with the aim of avoiding visual loss because of retinal damage by radiation or adjuvant therapy and to assess their final visual outcome. The mean basal dimension of the tumor was 5.0 ± 2.6 mm and the mean height was 2.9 ± 1.3 mm. The fovea was involved in 4 patients (50%) and foveal detachment was present in 3 patients (37.5%). All the patients required less than 6 cycles for tumor control. Four patients (50%) obtained visual acuities ranging from 20/25 to 20/200. Two patients (25%) could gain visual acuity of 20/20. The mean follow-up of patients was 3.24 ± 0.9 years (range 2–5). No patient developed recurrence or metastatic disease. In conclusion, recent trends for ocular salvage have favored chemoreduction followed by focal therapy for the treatment of retinoblastoma. This study supports the consideration of chemotherapy alone as the primary treatment in eyes with macular retinoblastomas, under close supervision, with satisfactory visual outcome.
Male-Pattern Baldness Is Common in Men with X-Linked Recessive Ichthyosis
Authors: Axt-Gadermann M. , Schlichting M. , Küster W.
Published: October 2003

Background: X-linked recessive ichthyosis (XRI) is a relatively common genetic disorder of keratinization caused by deficiency in steroid sulfatase (STS) activity. STS appears to play an important role in testosterone metabolism. Therefore it has been discussed that the presence of normally functioning STS may be a presupposition for the development of androgenetic alopecia (AGA). Methods: Patients with the diagnosis of XRI were sent questionnaires. Results and Conclusions: We reviewed 26 cases with XRI and noticed 11 patients with AGA in an advanced stage. The existence of two pathways for the steroid biosynthesis may be the explanation for a compensatory mechanism in XRI males. The Δ5 pathway depends on steroid sulfate activity, whereas the working Δ4 pathway produces AGA in XRI males.
Predominant Telangiectatic Erythema in Linear Atrophoderma of Moulin: Novel Variant or Separate Entity?
Authors: Utikal Jochen , Keil Darinka , Klemke Claus-Detlev , Bayerl Christiane , Goerdt Sergij
Published: October 2003

Linear atrophoderma of Moulin is a distinctive disease originally described in 1992 and characterized by acquired, mildly atrophic, non-sclerotic, slightly hyperpigmented lesions following the lines of Blaschko. Here, we describe a 15-year-old girl with a 13-year history and a 29-year-old male with a 6-year history of prominent linear telangiectatic erythema and mild atrophoderma following the lines of Blaschko that involved the right leg and hip, and both legs, the trunk and both arms, respectively. As pronounced telangiectatic erythema within lesions of atrophoderma of Moulin has not hitherto been described, we propose that the disease in our patients represents a novel variant of linear atrophoderma of Moulin. Due to considerable overlap, we do not favour the notion that our cases constitute an entity entirely separate from linear atrophoderma of Moulin.
Secondary Anetoderma Overlying Pilomatrixomas
Authors: Fujioka Masaki , Gozo Nishimura , Osamu Miyazato , Tsuneyuki Yamamoto , Takehisa Yosida
Published: October 2003

Five pilomatrixomas with anetodermic cutaneous changes are presented. Four large tumors revealed soft, atrophic, pink translucent skin covering a firm subcutaneous mass, and their appearance was similar to that of a keloid or hypertrophic scar. One small tumor revealed soft, slightly atrophic, reddened or purplish skin covering a firm subcutaneous mass: its clinical appearance was similar to that of a hematoma or minor infection. Histopathological examination of the skin showed atrophic and edematous changes in the dermis associated with diminished fragmented collagen and absent elastic tissue. It is supposed that the dermal atrophic appearance bears a relation to the loss of elastic fibers and the dermal edema is related to the leakage of lymphatic fluid. Both phenomena may be caused by continuous pressure from the outside, as these anetodermic pilomatrixomas were located where they were apt to be exposed to mechanical irritation.
Multiple Eruptive Dermatofibromas with CD34+ Cells in a Patient with Hypertriglyceridemia
Authors: Tsunemi Yuichiro , Ihn Hironobu , Hattori Naoko , Saeki Hidehisa , Tamaki Kunihiko
Published: October 2003

Multiple eruptive dermatofibromas (MEDF) are rare and have been thought to be associated with altered immunity. It has been reported that dermatofibromas (DFs) are positive for factor X IIIa but not for CD34. In this report, we describe a 34-year-old Japanese woman with MEDF and persistent hypertriglyceridemia. Histopathological examination revealed that several areas in larger nodules, showing higher cellularity and storiform growth pattern, had CD34+ cells. Our case and the review of previous reports suggest that hyperlipidemia, including hypertriglyceridemia, could induce MEDF and that highly cellular areas in DFs are sometimes positive for CD34.
Surgery for External Auditory Canal Exostoses: A Report of 182 Operations
Authors: Vasama Juha-Pekka
Published: October 2003

The pre-operative findings and operative outcome were evaluated from patient records in 136 patients (182 ears) who had surgery for external auditory canal (EAC) exostoses. Furthermore, 114 patients (84%) replied to a questionnaire inquiring about the symptoms and the operation’s impact on their quality of life. The main symptoms prior to the surgery were recurrent external otitis, hearing loss and wax retention. Interestingly, 23 patients (21%) were operated without any symptoms. After the operation, 75 patients (66%) had relief of their symptoms. In 33 patients (29%) the symptoms remained the same, and in 5 patients (4%) they became worse. The quality of life improved in 77 patients (68%); 19 patients (14%) had a complication due to the surgery, and 8 patients (6%) needed a re-operation. The surgery for EAC exostoses should be reserved only for symptomatic cases and only for experienced specialists in order to minimize complications and unnecessary operations.
Otoacoustic Emissions in Universal Hearing Screening: Which Day after Birth Should We Examine the Newborns?
Authors: Korres Stavros , Nikolopoulos Thomas , Ferekidis Eleftherios , Gotzamanoglou Zaphyria , Georgiou Anastasia , Balatsouras Dimitrios G.
Published: October 2003

Transiently evoked otoacoustic emissions (TEOAEs) have been widely used in universal newborn hearing screening programs. Although there is consensus with regard to the avoidance of early screening, especially during the first hours after birth, the optimum testing day is not yet unanimously accepted. The aim of the present study was to compare the ‘pass-refer’ results between 4 groups of newborns tested during the 4 postbirth days and determine the most appropriate day for assessing newborn hearing. Our results suggest that, although TEOAEs can be recorded in very high rates from the first 24 h of life, ‘refer’ scores are lower on the third and fourth days after birth. It may be thus concluded that the optimum time of assessing newborn hearing in universal hearing screening programs seems to be the third or fourth postbirth day, provided that other social or financial reasons do not suggest an earlier discharge from the hospital.
Argon Plasma Surgery for Treatment of Inferior Turbinate Hypertrophy: A Long-Term Follow-Up in 157 Patients
Authors: Ferri E. , Armato E. , Cavaleri S. , Capuzzo P. , Ianniello F.
Published: October 2003

Chronic nasal obstruction is a common disorder, mostly caused by hypertrophic inferior turbinates. In cases of nonresponse to conservative medical management, the surgical reduction of the inferior turbinates is often required. Surgical techniques for the reduction of hyperplastic nasal turbinates include monopolar or bipolar coagulation, partial or total reduction of the turbinates with a conchotome or scissors and laser surgery with Nd:YAG, CO2 or KTP. Argon plasma coagulation (APC) is a new, innovative technique in otorhinolaryngology which is based on high-frequency electrocoagulation with an argon gas source. The fast and short surgery times, the absence of hemorrhagic complications and, in rhinosurgery, the complete abstention from any kind of nasal packing are remarkable. Between March and November 2000, a group of 157 patients with symptoms of nasal obstruction due to hypertrophic inferior turbinates were treated using APC, at the Otorhinolaryngology Surgery Department of the Hospital of Dolo (Venice). The follow-up period was 24 months. Rhinomanometry was performed to objectively measure the nasal obstruction and to verify the postoperative improvement. The Student t test was used for statistical data. After 24 months, 87% (p < 0.001) of the patients reported to have a better nasal airflow than before the operation. None of the patients needed a nasal packing after APC surgery. The indication, advantages, complications, controversies and long-term results of the treatment with APC in rhinosurgery are reviewed and discussed.
Cochlear Implantation after Acoustic Tumour Resection in Neurofibromatosis Type 2: Impact of Intra- and Postoperative Neural Response Telemetry Monitoring
Authors: Nölle C. , Todt I. , Basta D. , Unterberg A. , Mautner V.F. , Ernst A.
Published: October 2003

The present paper reports about a 16-year-old male with neurofibromatosis type 2 (NF-2) of the Wishart type with bilateral deafness who had undergone cochlear implantation after resection of the acoustic neuroma (AN) of the same side. Neural response telemetry (NRT) recordings are essential in those patients during cochlear implantation where no stapedial reflexes can be electrically elicited due to the resection of the AN. In the present case, amplitude growth function and a type II pattern of the NRT waveforms could be well established. The comparison of the N1 response intra-operatively and after 2 years showed a decline in latency by 50% and an increase in absolute amplitude by 10 times at the same current level of electrical stimulation. This improved auditory nerve transduction suggested a change to a ‘faster’ encoding strategy to improve speech understanding. The change from SPEAK to ACE 18 months after the operation led to an increase in the open-set sentence recognition test from 52 to 88%. Thus, NRT recordings monitor the intra-operative success of electrode placement and help to assess the integrity of the auditory pathway. Moreover, they can reliably be used in programming the speech processor postoperatively as objective tool. In patients with NF-2, the restoration of hearing can be successfully achieved in several ways. The indications for hearing implants (auditory brain stem and cochlear implants) should be carefully considered with respect to the remaining, functional integrity of the auditory nerve and the technical possibilities to monitor the success of these procedures.
Standard Therapy of Crohn’s Disease
Authors: Miehsler Wolfgang , Gasche Christoph
Published: October 2003

Therapy of Crohn’s disease is either counteracting the underlying mechanisms of bowel inflammation or specifically targeting certain complications of disease, e.g. strictures, fistulae, bacterial overgrowth, or anemia. The therapeutic arsenal is well defined and has been tested for safety and efficacy. There is need and opportunity to adapt therapies to individual situations. This is made on the basis of scientific facts that have been collected from randomized controlled trials and on the basis of reasoned thinking that weighs the potential effect against potential side effects in individual cases. In clinical practice, the potency of lifestyle changes such as smoking cessation is still underestimated and not extensively exploited. As the understanding of genetic mutations, their gene function and phenotypic expression grows, we anticipate that future treatment can be tailored to certain genotypes or phenotypes of Crohn’s disease.
Preliminary Planning for Training Speech and Language Therapists in Uganda
Authors: Robinson Heather , Afako Ruth , Wickenden Mary , Hartley Sally
Published: October 2003

Country-specific issues affect both the implementation of services for people with communication disabilities and the training of specialists who provide these services. Uganda is a majority world country with an extremely limited specialist service, but is looking to expand it by developing training for specialist workers. This paper reports on the initial processes adopted in Uganda to develop such training. A workshop that brought together all the stakeholders identified key issues. The paper relates these issues to the International Association of Logopedics and Phoniatrics guidelines for education and training and makes suggestions for the way forward.
Antigenic Specificity of Serum Antibodies in Mice Fed Soy Protein
Authors: Christensen Hanne R. , Bruun Susanne W. , Frøkiær Hanne
Published: October 2003

Background: Soybean protein is used in a number of food products but unfortunately is also a common cause of food allergy. Upon ingestion of soy protein, healthy mice like other animals and humans generate a soy-specific antibody response in the absence of signs of illness. Not much is known about the relationship between the immunogenic proteins involved in this nondeleterious antibody response and the pathological response associated with food allergy.The objective of the present study was to characterize the antigenic specificity of the soy protein-specific antibody response generated in healthy mice ingesting soy protein. Methods: Blood from mice fed a soy-containing diet was analyzed using ELISA and immunoblot for antibody reactivity towards various soy protein fractions and pure soy proteins/subunits. Mice bred on a soy-free diet were used as controls. Results: The detectable antigenic specificity of the serum antibodies of soy-consuming mice comprised glycinin and β-conglycinin. Immunoblots with soy protein extract demonstrated antibody reactivity towards both the basic and the acidic chains of glycinin and the β-conglycinin subunits with an individual response pattern among mice. Moreover, antibody reactivity was found towards the native quaternary structure of glycinin. Conclusions: Mice ingesting soy protein generate an antibody response with reactivity towards glycinin and β-conglycinin. Antibody reactivity found towards the native quaternary structure of glycinin indicates an oral immunogenicity of the highly processing-resistant oligomerized glycinin.
Tolerability of Rofecoxib in Patients with Adverse Reactions to Nonsteroidal Anti-Inflammatory Drugs: A Study of 216 Patients and Literature Review
Authors: Perrone Maria Rosaria , Artesani Maria Cristina , Viola Marinella , Gaeta Francesco , Caringi Mario , Quaratino Donato , Romano Antonino
Published: October 2003

Background: Adverse reactions to nonsteroidal anti-inflammatory drugs (NSAIDs) are frequent, particularly among patients with chronic urticaria or asthma. The identification of an alternative safe and reliable drug is a common problem in clinical practice. Objective: To assess the tolerability of rofecoxib, a new NSAID that selectively inhibits the inducible isoform of cyclooxygenase, in a large group of NSAID-sensitive patients. Methods: We studied 216 patients (164 females and 52 males) who had suffered adverse reactions to one or more NSAIDs; 98 subjects (45.4%) had experienced reactions to only one NSAID (single hypersensitivity) and 118 subjects (54.6%) had reacted to multiple NSAIDs (multiple hypersensitivity). Cutaneous reactions were reported by 79.6% of the subjects, respiratory symptoms by 10.7%, cutaneous and respiratory symptoms by 8.3%, anaphylaxis by 1.4%. All the subjects underwent a single-blind, placebo-controlled oral challenge with divided therapeutic doses of rofecoxib (6.25 mg +18.75 mg 1 h later = cumulative dose of 25 mg). Results: No reactions to the placebo were observed; only 1 subject (0.46%) experienced an urticarial reaction, after the second dose of rofecoxib. Conclusions: Considering previous studies and our own data, rofecoxib was well tolerated by all of the 174 patients with exclusively NSAID-related respiratory symptoms. Rofecoxib also had a very low rate (1.6%) of cross-reactivity in the 600 patients with exclusively cutaneous reactions to NSAIDs.
Input and Output Characteristics of Collision Avoidance Behavior in the Frog Rana catesbeiana
Authors: Yamamoto Keisuke , Nakata Maki , Nakagawa Hideki
Published: October 2003

Input and output characteristics of collision avoidance behavior in the bullfrog were examined using computer graphics to model a looming stimulus. The means of time-to-collision of avoidance behavior in response to looming visual stimuli approaching at a velocity of either 2 or 4 m/s were significantly different (t(141) = 7.93, p < 0.05). On the other hand, mean threshold sizes of visual stimuli triggering avoidance behavior were not significantly different in either case (t(201) = 0.54, p > 0.05). Furthermore, we showed that the mean threshold sizes changed in a predictable manner depending on the distance between the displayed stimulus and the animal. These results strongly suggest that the frog displays collision avoidance behavior when the visual angle of a looming object reaches a constant value (about 20°). The mean maximum velocities of the avoidance behavior in response to the two visual stimuli were not significantly different (t(198) = 1.44, p > 0.05). However, we found that the frog could control the velocity depending on the location of an approaching object in its dorsal visual field. Finally, we demonstrated that habituation significantly affected the mean probability of avoidance behavior occurrence (ANOVA, at 2 m/s, F(2,15) = 14.25; at 4 m/s, F(2,15) = 13.35, p < 0.05), but not those of time-to-collision, threshold size and maximum velocity (ANOVA, at 2 m/s, F(2,13) = 0.07, F(2,14) = 0.46 and F(2,14) = 0.70, respectively; at 4 m/s, F(2,15) = 0.50, F(2,14) = 0.68 and F(2,14) = 0.41, respectively, p > 0.05). Thus, frog collision avoidance behavior seems to have an all or none – like property.
Retinal Topography of the Harp Seal Pagophilus groenlandicus
Authors: Mass Alla M. , Supin A.Y.
Published: October 2003

The total number, size, topographic distribution, and cell density of ganglion cells were studied in retinal wholemounts of the harp seal Pagophilus groenlandicus. Ganglion cell size varied from 10 to 60 µm. A distinct group were large ganglion cells more than 30–35 µm in diameter which were similar to α-cells known in terrestrial mammals. The number of α-cells constituted 5.3–5.9% of the total ganglion cell population. The cell size distribution was bimodal, with the second mode composed of α-cells. The topographic distribution of ganglion cells showed a definite area of high cell density similar to area centralis of terrestrial carnivores. This area was located in the temporal retinal quadrant, 7–8 mm (16–18°) from the optic disk. In this area, the peak cell densities in two wholemounts were 2,500 and 1,650 (mean 2,075) cells/mm2. With a posterior nodal distance of 25.5 mm (underwater), this density corresponded to 495 and 327 (mean 411) cells/deg2. These values predict a retinal resolution of 2.7–3.3′ (11.1–9.0 cycles/deg) in water and 3.6–4.4′ (8.3–6.8 cycles/deg) in air. Topographic distributions of α-cells was qualitatively similar to that of the total ganglion cell population, but the density of α-cells constituted only a few percent (mostly 3–7.5%) of the total ganglion cell density.
What We Have Learned about Cachexia in Gastrointestinal Cancer
Authors: Palesty J.A. , Dudrick S.J.
Published: October 2003

It is appreciated widely by clinicians that significant malnutrition accompanies malignant processes in approximately 50% of patients and eventually leads to severe wasting which accounts for approximately 30% of cancer-related deaths overall, 30–50% of deaths in patients with gastrointestinal tract cancers, and up to 80% of deaths in patients with advanced pancreatic cancer. The body wasting known as cancer cachexia is a complex syndrome characterized by progressive tissue depletion and decreased nutrient intake that is manifested clinically as inexplicable, recalcitrant anorexia and inexorable host weight loss. Decreased nutritional intake, increased metabolic expenditure and dysfunctional metabolic processes, including hormonal and cytokine-related abnormalities, all appear to play roles in the development of cancer cachexia. Although this condition of advanced protein-calorie malnutrition, sometimes described as the cancer anorexia-cachexia syndrome, is not entirely understood, it appears to be multifactorial, is a major cause of morbidity and mortality in cancer patients, and ultimately leads to death. Therapeutic interventions have met with little success, and, regardless of tremendous efforts throughout the decades, the exact nature of the mediators responsible for cancer cachexia remain elusive. The pathogenesis of cancer cachexia appears to be related to proinflammatory cytokines, alterations in the neuroendocrine axis and tumor-derived catabolic factors. Despite trials of conventional and/or aggressive nutritional support by a myriad of feeding techniques, patients with cancer cachexia have failed to gain consistent significant benefits in terms of weight gain, functional ability, quality of life or survival. Additionally, attempts to ameliorate the abnormal clinical and metabolic features of cancer cachexia with a variety of pharmacologic agents have met with only limited success. Either until cancer of the gastrointestinal tract can be cured or until it is possible to identify the exact causes and mechanisms of the cancer cachexia syndrome, the most realistic and practical options currently are directed toward minimizing adverse gastrointestinal side effects or complications of the malignant process and/or therapy, as well as increasing appetite, food intake and nutrient utilization in an effort to enhance quality of life and improve survival.
Nutritional Support in Acute Pancreatitis
Authors: Avgerinos Costas , Delis Spiros , Rizos Spyros , Dervenis Christos
Published: October 2003

Acute pancreatitis (AP), mainly the severe necrotizing type, results in extreme energy demands which might lead, if prolonged, to severe malnutrition. Besides that, starving during AP contributes to gut barrier dysfunction, the main cause of bacterial translocation and sepsis. The aim of nutritional support in AP is to prevent malnutrition and protect the gut by maintaining mucosal integrity. Traditionally, nutritional support during the acute phase of the disease has been provided through total parenteral nutrition (TPN) solutions. However, recent animal and human studies have identified new patterns of pancreatic secretion and hormonal stimulation during the course of AP, different from those assumed for years. Thus it has become feasible to use the natural enteral route for nutrition with potential benefits compared with TPN.
Serum Mineral Levels in Children with Intestinal Parasitic Infection
Authors: Olivares José L. , Fernández Ramona , Fleta Jesús , Rodríguez Gerardo , Clavel Antonio
Published: October 2003

Parasitic infections are highly prevalent in the general population. A relation between a parasitic infection and absorption of minerals is not an easy task. Serum levels of copper, zinc and magnesium were prospectively measured in 64 children with intestinal parasitic infection. Thirty-nine children with Enterobius vermicularis were treated with pyrantel pamoate and 25 children with Giardia lamblia with tinidazole and metronidazole. Three months after treatment, significant differences in serum copper, zinc and magnesium were seen in patients with E. vermicularis infection, and in serum magnesium levels in patients with G. lamblia. Although the pathogenic mechanism is not clear, these findings could reflect a deficiency related to malabsorption due to mucous affection. Early detection and treatment of intestinal parasitosis could avoid these serum mineral deficiencies.
Fructose Breath Hydrogen Test – Is It Really a Harmless Diagnostic Procedure?
Authors: Müller P. , Meier C. , Böhme H.J. , Richter T.
Published: October 2003

Usage of hydrogen breath tests has become one of the standard procedures in diagnosing chronic unspecific abdominal pain. These tests are said to be of sufficient specificity and sensitivity, are easily done, non-invasive and are more often practiced in outpatients. A 13-year-old boy is reported with chronic unspecific abdominal pain and growth retardation and so far misdiagnosed hereditary fructose intolerance (HFI), who developed life-threatening adverse effects during the fructose breath hydrogen test. It is concluded that the possibility of HFI should be excluded first by a carefully explored dietary history before the fructose breath test is performed under medical supervision. If there is any suspicion of HFI, a molecular genetic analysis should be preferred.
Ion Release from Copper Phosphate Cement and Influence on Streptococcus mutans Growth in vitro: A Comparative Study
Authors: Foley Jennifer , Blackwell Alison
Published: October 2003

The aim of this study was to compare the effects of a black copper cement (BCC), an established restorative material (a conventional glass ionomer cement) and two temporary restorative materials (a zinc phosphate and a zinc polycarboxylate cement) on the growth of Streptococcus mutans in vitro, and to correlate bacterial growth with ion release from each material. Test specimens were eluted in either 0.1 M lactic acid, pH 4, or 0.1 M sodium chloride, pH 7. At 2 days, 7 days, 28 days and 6 months, eluates were inoculated with S. mutans and bacterial growth was recorded. Metal ion (Cu2+, Zn2+ and Mg2+) and fluoride release were measured. At most immersion times, the different materials had a statistically significant inhibitory effect on bacterial growth compared to the respective control, at both pH levels. The inhibitory effect decreased with time and in most cases was associated with high levels of ion release at the beginning of the experimental period, followed by significantly lower levels. For BCC, there were statistically significant relationships between the median rates of growth of S. mutans in the presence of BCC eluates and the median values for release of copper and zinc, although not magnesium. Of the different materials, BCC demonstrated greatest antibacterial activity.
Evaluation of the Effectiveness of Laser Fluorescence in Monitoring in vitro Remineralization of Incipient Caries Lesions in Primary Teeth
Authors: Mendes Fausto Medeiros , Nicolau José , Duarte Danilo Antonio
Published: October 2003

The purpose of this investigation was to examine the effectiveness of laser fluorescence for monitoring in vitro remineralization of caries lesions. Seventeen natural enamel lesions in primary molars were each cut into two parts. One part was exposed, at room temperature, to a remineralizing solution (experimental) and the other part to water (control) for 28 days. Laser fluorescence readings were taken at the beginning of the experiment and during the experiment. At the end of the experiment, lesion depth in the experimental halves was significantly less than in the control halves (p < 0.01), suggesting that remineralization had occurred. However, the laser fluorescence readings showed no statistically significant changes during the experiment, either in the experimental or control halves. We conclude that laser fluorescence was not able to detect in vitro remineralization of natural incipient caries lesions of primary teeth.
Parental origin of the two additional haploid sets of chromosomes in an embryo with tetraploidy
Authors: Baumer A. , Dres D. , Basaran S. , Isçi H. , Dehgan T. , Schinzel A.
Published: October 2003

We report on the molecular investigations performed on an embryo with tetraploidy, karyotype 92,XXXY. The embryo was spontaneously aborted after eight weeks of gestation. Molecular analyses were performed in order to determine the parental origin and mode of formation of the two additional haploid sets of chromosomes. Microsatellite markers mapping to pericentromeric chromosome regions were used. Our results show a maternal origin of one additional set of chromosomes most likely due to the incorporation of the polar body of meiosis I and a paternal origin of the second additional set of chromosomes most likely due to dispermy. The karyotype 92,XXXY is rather unusual, indeed the vast majority of cases with tetraploidy have the karyotypes 92,XXXX or 92,XXYY. To the best of our knowledge this is the first case with 92,XXXY for which molecular investigations have been performed.
Chromosome banding in Amphibia
Authors: Schmid M. , Feichtinger W. , Steinlein C. , Visbal García R. , Fernández Badillo A.
Published: October 2003

Extensive cytogenetic analyses on a population of the leptodactylid frog Eleutherodactylus riveroi in northern Venezuela revealed the existence of multiple XXAA♂/XYAA♀/XAAY♀ sex chromosomes. The XAAY karyotype originated by a centric (Robertsonian) fusion between the original, free Y chromosome and an autosome. 46.2% of the male individuals in this population are carriers of this Y-autosome fusion. In male meiosis the XAAY sex chromosomes pair in the expected trivalent configuration. In the same population 53.8% of the male animals still possess the original, free XY sex chromosomes. E. riveroi is only the second vertebrate species discovered in which a derived Y-autosome fusion coexists with the ancestral free XY sex chromosomes. The free XY sex chromosomes, as well as the multiple XAY sex chromosomes are still in a very primitive (homomorphic) stage of differentiation. With no banding technique applied it is possible to distinguish the Y from the X. Various banding techniques and in situ hybridizations have been carried out to characterize the karyotypes. DNA flow cytometric measurements show that the genome size of E. riveroi resembles that of other Eleutherodactylus species. The cytogenetic data obtained in E. riveroi are compared with those of the sole other vertebrate known to possess the extremely rare, multiple XXAA♂/XYAA♀/XAAY♀ sex chromosomes. Surprisingly enough, this vertebrate again is a frog belonging to the genus Eleutherodactylus [E. (maussi) biporcatus] which lives exactly in the same habitat in northern Venezuela as does E. riveroi.