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Biomedical subjects

D Rodriguez

Publications and source records attributed to D Rodriguez.

At least 19 recordsLinked to original sources
PubMed2002

[Neurofibromatosis 1: recommendations for management].

Twenty experts, members of a French medical network devoted to neurofibromatosis 1 have elaborated recommendations for the management of the disease. Bibliography was obtained through a Medline of articles from 1966 to 1999 for the terms neurofibromatosis, NF1, neurofibroma and from textbooks. A consensual document was written taking into account extracted data. An annual careful clinical examination is recommended except in cases with complications. Screening investigations are not recommended due to the rarity of complications, generally symptomatic and easily detected during the clinical follow-up. The only controversial exception might be magnetic resonance imaging for early detection of optic pathway gliomas in young children. A co-ordinated follow-up in specialised multidisciplinary centres, providing patients with a rational management, is recommended.

Adolescent↗
PubMed2001

The A17L gene product of vaccinia virus is exposed on the surface of IMV.

The p21 membrane protein of vaccinia virus (VV), encoded by the A17L gene, has been reported to localize on the inner of the two membranes of the intracellular mature virus (IMV). It has also been shown that p21 acts as a membrane anchor for the externally located fusion protein p14 (A27L gene). Since p14 is located on the surface of IMVs, it is hard to envision that p21 should be located only on the inner membrane. Our results from (i) immunoelectron microscopy, (ii) biotinylation, and (iii) protease treatment of purified IMVs showed that the N-terminus of p21 is exposed on the surface of virus particles, while the C-terminus is embedded in the membrane. Mono-specific antibodies to the N-terminus of p21 neutralize infection of VV while antibodies to the C-terminal domain do not. We suggest that p21 molecules are located both in the inner and in the outer membrane of IMV.

Animals↗
PubMed2001

Infantile Alexander disease: spectrum of GFAP mutations and genotype-phenotype correlation.

Heterozygous, de novo mutations in the glial fibrillary acidic protein (GFAP) gene have recently been reported in 12 patients affected by neuropathologically proved Alexander disease. We searched for GFAP mutations in a series of patients who had heterogeneous clinical symptoms but were candidates for Alexander disease on the basis of suggestive neuroimaging abnormalities. Missense, heterozygous, de novo GFAP mutations were found in exons 1 or 4 for 14 of the 15 patients analyzed, including patients without macrocephaly. Nine patients carried arginine mutations (four had R79H; four had R239C; and one had R239H) that have been described elsewhere, whereas the other five had one of four novel mutations, of which two affect arginine (2R88C and 1R88S) and two affect nonarginine residues (1L76F and 1N77Y). All mutations were located in the rod domain of GFAP, and there is a correlation between clinical severity and the affected amino acid. These results confirm that GFAP mutations are a reliable molecular marker for the diagnosis of infantile Alexander disease, and they also form a basis for the recommendation of GFAP analysis for prenatal diagnosis to detect potential cases of germinal mosaicism.

Adolescent↗
PubMed2001

Proteolytic exposure of a cryptic site within collagen type IV is required for angiogenesis and tumor growth in vivo.

Evidence is provided that proteolytic cleavage of collagen type IV results in the exposure of a functionally important cryptic site hidden within its triple helical structure. Exposure of this cryptic site was associated with angiogenic, but not quiescent, blood vessels and was required for angiogenesis in vivo. Exposure of the HUIV26 epitope was associated with a loss of alpha1beta1 integrin binding and the gain of alphavbeta3 binding. A monoclonal antibody (HUIV26) directed to this site disrupts integrin-dependent endothelial cell interactions and potently inhibits angiogenesis and tumor growth. Together, these studies suggest a novel mechanism by which proteolysis contributes to angiogenesis by exposing hidden regulatory elements within matrix-immobilized collagen type IV.

Animals↗
PubMed2001

Pontoneocerebellar hypoplasia: definition of MR features.

We report five patients with pontoneocerebellar hypoplasia. Because the issue of cerebellar malformations is a difficult subject, we tried to define criteria for diagnosis on MRI: a thin flat pons with disappearance of the anterior curve, a small cerebellum with predominant flattening of the hemispheres and shortened cerebellar fissures, in contrast to atrophy. The posterior fossa is not enlarged. We emphasize the probable late onset of the disease in fetal life because of the demonstration of the abnormalities at US during the last trimester of the pregnancy in one patient. Prenatal diagnosis is important because of possible autosomal recessive transmission.

Cerebellum↗
PubMed2001

Urinary retention following routine neurosurgical spine procedures.

BACKGROUND: The rate and duration of urinary retention after routine cervical and lumbar spine procedures were studied. METHODS: Preoperative, intraoperative, and postoperative factors related to urinary retention (age, sex, duration of operation, medications, Foley use, hospital stay, and cost) were analyzed in a retrospective review of 503 patients' charts. RESULTS: Urinary retention occurred 38% of the time following cervical and lumbar spine procedures. Advanced age and preoperative beta blockers contributed to a higher incidence of urinary retention. Preoperative anti-inflammatory medications and narcotic analgesics reduced the frequency of urinary retention. The duration of urinary retention was prolonged in older patients and patients who underwent intraoperative Foley catheterization. Urinary retention contributed to longer hospitalization and increased hospital costs. CONCLUSIONS: Transient urinary retention is a common complication of routine neurosurgical spine procedures that prolongs hospital stays and increases the costs of hospitalization.

Adult↗
PubMed2001

Mutations in GFAP, encoding glial fibrillary acidic protein, are associated with Alexander disease.

Alexander disease is a rare disorder of the central nervous system of unknown etiology. Infants with Alexander disease develop a leukoencephalopathy with macrocephaly, seizures and psychomotor retardation, leading to death usually within the first decade; patients with juvenile or adult forms typically experience ataxia, bulbar signs and spasticity, and a more slowly progressive course. The pathological hallmark of all forms of Alexander disease is the presence of Rosenthal fibers, cytoplasmic inclusions in astrocytes that contain the intermediate filament protein GFAP in association with small heat-shock proteins. We previously found that overexpression of human GFAP in astrocytes of transgenic mice is fatal and accompanied by the presence of inclusion bodies indistinguishable from human Rosenthal fibers. These results suggested that a primary alteration in GFAP may be responsible for Alexander disease. Sequence analysis of DNA samples from patients representing different Alexander disease phenotypes revealed that most cases are associated with non-conservative mutations in the coding region of GFAP. Alexander disease therefore represents the first example of a primary genetic disorder of astrocytes, one of the major cell types in the vertebrate CNS.

Adolescent↗
PubMed2001

Plastid-expressed 5-enolpyruvylshikimate-3-phosphate synthase genes provide high level glyphosate tolerance in tobacco.

Plastid transformation (transplastomic) technology has several potential advantages for biotechnological applications including the use of unmodified prokaryotic genes for engineering, potential high-level gene expression and gene containment due to maternal inheritance in most crop plants. However, the efficacy of a plastid-encoded trait may change depending on plastid number and tissue type. We report a feasibility study in tobacco plastids to achieve high-level herbicide resistance in both vegetative tissues and reproductive organs. We chose to test glyphosate resistance via over-expression in plastids of tolerant forms of 5-enolpyruvylshikimate-3-phosphate synthase (EPSPS). Immunological, enzymatic and whole-plant assays were used to prove the efficacy of three different prokaryotic (Achromobacter, Agrobacterium and Bacillus) EPSPS genes. Using the Agrobacterium strain CP4 EPSPS as a model we identified translational control sequences that direct a 10,000-fold range of protein accumulation (to >10% total soluble protein in leaves). Plastid-expressed EPSPS could provide very high levels of glyphosate resistance, although levels of resistance in vegetative and reproductive tissues differed depending on EPSPS accumulation levels, and correlated to the plastid abundance in these tissues. Paradoxically, higher levels of plastid-expressed EPSPS protein accumulation were apparently required for efficacy than from a similar nuclear-encoded gene. Nevertheless, the demonstration of high-level glyphosate tolerance in vegetative and reproductive organs using transplastomic technology provides a necessary step for transfer of this technology to other crop species.

3-Phosphoshikimate 1-Carboxyvinyltransferase↗
PubMed2001

Successful surgical relief of seizures associated with hamartoma of the floor of the fourth ventricle in children: report of two cases.

OBJECTIVE AND IMPORTANCE: To discuss the physiopathology and surgical handling of seizures due to hamartoma of the floor of the fourth ventricle in two children. CLINICAL PRESENTATION: Two girls aged 3 years at the time of their operations presented with seizures due to a lesion of the floor of the fourth ventricle. The seizures began within the first days of life and consisted of hemifacial contraction, then head deviation, blinking of the eyelids, and intermittent dysautonomic manifestations. The interictal neurological condition seemed normal in one patient and showed a slight development delay in the other. An ictal electroencephalogram showed slow waves in the posterior areas. A magnetic resonance imaging scan revealed a mass that remained unchanged on serial examinations bordering the fourth ventricle, with an isointense signal on T1-weighted sequences and high-intensity signals on T2-weighted sequences without gadolinium enhancement. An ictal single-photon emission computed tomographic scan showed hyperperfusion in the lesion in both girls. INTERVENTION: The operation consisted of resection and disconnection of the lesion. An electrical recording was obtained in one patient during the operation while she was anesthetized; the recording, made by means of a depth electrode with five contacts inside the lesion, indicated that repetitive theta rhythmic discharges were present. Neuropathology was consistent with a hamartoma. In both girls, the seizures disappeared after their operations, and antiepileptic drugs were withdrawn (follow-up periods, 8 and 3 yr, respectively). CONCLUSION: Considering the results of single-photon emission computed tomography, the intralesional electrical record, and the relief of seizures after the operation, we postulate that the seizures arose from inside the lesion. This particular kind of noncortical seizure is similar to gelastic seizure due to hypothalamic hamartoma.

Brain Diseases↗
PubMed2001

Suppression of asthma-like responses in different mouse strains by oral tolerance.

In this study we examined the effect of oral antigen (Ag) administration on the development of experimental asthma in different mouse strains. We selected BALB/c, BP2, CBA/Ca interleukin (IL)-5 transgenic, and BALB/c T-cell receptor-delta-deficient mouse strains because they exhibit different aspects of the asthma syndrome. Mice exposed to 1% ovalbumin (OVA), dissolved in the drinking water for 5 consecutive days, became unresponsive to subsequent immunogenic OVA challenges. This regimen of OVA administration induced Ag-specific unresponsiveness in all mouse strains tested, including gammadelta-deficient mice that are said to be resistant to tolerance induction. The Ag-specific unresponsiveness was characterized by reduced (almost absent) airway eosinophilic inflammation, airway hyperreactivity, and mucus production; also by low levels of T helper (Th) 2-type cytokines in bronchoalveolar lavage fluid, and decreased immunoglobulin (Ig) G1 and IgE OVA-specific antibody production. The unresponsive state was not associated with increased levels of the suppressive cytokines IL-10 and transforming growth factor (TGF)-beta or with immune deviation toward the Th1 pathway due to increased levels of interferon-gamma and IL-12. Moreover, treatment with anti- TGF-beta antibodies did not abrogate oral tolerance. Oral Ag administration was quite effective in suppressing the development of key features of asthma when initiated after primary immunization (Day 0) or after booster (Day 7), but not after challenge (Day 14) when it increased allergic responses. Collectively, our findings show for the first time the beneficial and detrimental effects of oral Ag administration on the development of experimental asthma.

Administration, Inhalation↗
PubMed2001

Efficacy and tolerance of adapalene cream 0.1% compared with its cream vehicle for the treatment of acne vulgaris.

Adapalene gel 0.1% is approved for use in the treatment of acne vulgaris. A new cream formulation, adapalene cream 0.1%, has been developed. Our objective was to evaluate the efficacy and tolerability of adapalene cream 0.1% in comparison with its cream vehicle, applied once daily for 12 weeks to patients with facial acne vulgaris. We used a 12-week, multicenter, randomized, double-blind, vehicle-controlled, comparative phase 3 study of adapalene cream 0.1% and cream vehicle. The study enrolled 237 patients (125 males and 112 females), aged 12 through 30 years, with mild-to-moderate acne vulgaris. Adapalene cream 0.1% demonstrated superior efficacy compared with its cream vehicle. Significantly lower numbers of total inflammatory and noninflammatory lesion counts were observed at the end of the study period in patients using adapalene cream 0.1% as opposed to those using cream vehicle (P<.05 compared with baseline, for all 3 parameters). Adapalene cream 0.1% caused more cutaneous side effects than the cream vehicle, but these were tolerated in most patients. In summary, the results of this study indicate that adapalene cream 0.1% demonstrates superior efficacy over cream vehicle for the treatment of acne vulgaris. Adapalene cream 0.1% also has excellent tolerability and is associated with a low incidence of cutaneous adverse events.

Acne Vulgaris↗
PubMed2000

Intramolecular

2-Propynyldiarylacetylenes undergo thermal intramolecular [4 + 2] cycloaddition to give benzo[b]fluorene derivatives in good yields. The hybridization of the tether connecting the reacting alkynes has a pronounced effect on the course of the reaction. Theoretical calculations and isotopic labeling studies support a mechanism which involves the generation of a cyclic allene intermediate that evolves to the final benzo[b]fluorene.

Journal Article↗
PubMed2000

Immunogenicity of Ty-VLP bearing a CD8(+) T cell epitope of the CS protein of P. yoelii: enhanced memory response by boosting with recombinant vaccinia virus.

We characterized the immunogenicity of the hybrid Ty-virus-like carrying the CD8(+) T cell epitope (SYVPSAEQI) of the circumsporozoite (CS) protein of Plasmodium yoelii (TyCS-VLP), a rodent malaria parasite. Balb/c mice were immunized with hybrid TyCS-VLP, and their CS-specific CD8(+) T cell response was quantitatively evaluated with the ELISPOT assay, based on the enumeration of epitope specific gamma-interferon secreting CD8(+) T cell. A single immunization with the TyCS-VLP by a variety of routes and doses indicated that the maximal response occurred in mice, which were immunized with 50 micrograms of these particles, administered via intramuscular. Combined immunization of mice with this TyCS-VLP followed by recombinant vaccinia virus expressing the entire P. yoelii CS protein (VacPyCS) or irradiated sporozoites, induced high levels of IFN-gamma-producing cells. The immunization regime, priming with TyCS-VLP and boosting with VacPyCS generated a potent protective immune response, which strongly inhibited P. yoelii liver stages development and protected 62% of the mice against a subsequent live P. yoelii sporozoite challenge.

Animals↗