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Biomedical subjects

C Stricker

Publications and source records attributed to C Stricker.

At least 19 recordsLinked to original sources
PubMed2002

Nonmyeloablative allogeneic stem cell transplantation for refractory Hodgkin's lymphoma complicated by interleukin-2 responsive progressive multifocal leukoencephalopathy.

Nonmyeloablative allogeneic stem cell transplantation (NMASCT) can be used to exploit the graft-versus-tumor (GVT) potential of allogeneic donor cells in the setting of reduced conditioning regimen toxicity. This approach is particularly attractive for patients who have received extensive prior therapy and are poor candidates for traditional allogeneic stem cell transplantation. However, toxicity in heavily pretreated patients remains uncertain. Additional immunosuppression in already immunocompromised patients may result in unexpected toxicity. We report a case of probable progressive multifocal leukoencephalopathy (PML) responsive to interleukin-2 (IL-2) following a NMASCT in a 29-year-old woman with relapsed Hodgkin's lymphoma. The patient developed severe neurological symptoms approximately 6 weeks following NMASCT associated with low CD4+ cell counts and magnetic resonance imaging (MRI) was consistent with PML. IL-2 therapy resulted in increasing CD4+ counts and progressive resolution of neurological symptoms. Disruption of IL-2 therapy led to neurological deterioration, which responded to reinstitution of IL-2 therapy. The patient's lymphoma initially progressed following NMASCT, but has responded to donor leukocyte infusions (DLI). This case reiterates the potent GVT potential of NMASCT in patients with Hodgkin's disease. However, it demonstrates the potential for severe complications related to immunosuppression, especially in heavily pretreated patients. The toxicity after NMASCT should not be understated and will need to be explored further.

Adult↗
PubMed2002

Further contributions to the genetic aspect of congenital sensorineural deafness in Dalmatians.

Sensorineural deafness is a common congenital disorder in Dalmatians and is genetically transmitted. Different modes of inheritance have been proposed and the objective of this study was to study these by segregation analyses using maximum likelihood procedures. Data from 33 complete Dalmatian families were collected and data from 56 single Dalmatians added. This resulted in a total of 575 dogs with 357 known phenotypes. All dogs were clinically evaluated and electrophysiologically tested with brainstem auditory evoked responses. The prevalence of deafness was 16.5% (9.4% unilaterally deaf, 7.1% bilaterally deaf). Females were 4.4% more affected than males but this difference was not significant. Within the same litter, different phenotypic expressions of deafness occurred, which suggested different expressions of the disease. In addition, two data sets were analysed: the first included normal, uni- and bilaterally deaf dogs, the second had normal and deaf Dalmatians. We found that a recessive allele at a single biallelic major locus fitted our data best, although an incomplete penetrance of the recessive homozygotes was observed.

Animals↗
PubMed2002

Central synaptic integration: linear after all?

Unitary synaptic currents in hippocampus show small variability. Experimental evidence suggests that the neuron is endowed with mechanisms to reduce location-dependent differences in amplitude and time course of synaptic events, contributing to small variability. These mechanisms may allow the neuron to count individual quanta and thereby linearize integration of unitary events.

Animals↗
PubMed2001

Transition from GABAergic to glycinergic synaptic transmission in newly formed spinal networks.

The role of glycinergic and GABAergic systems in mediating spontaneous synaptic transmission in newly formed neural networks was examined in motoneurons in the developing rat spinal cord. Properties of action potential-independent miniature inhibitory postsynaptic currents (mIPSCs) mediated by glycine and GABA(A) receptors (GlyR and GABA(A)R) were studied in spinal cord slices of 17- to 18-day-old embryos (E17-18) and 1- to 3-day-old postnatal rats (P1-3). mIPSC frequency and amplitude significantly increased after birth, while their decay time decreased. To determine the contribution of glycinergic and GABAergic synapses to those changes, GlyR- and GABA(A)R-mediated mIPSCs were isolated based on their pharmacological properties. Two populations of pharmacologically distinct mIPSCs were recorded in the presence of glycine or GABA(A) receptors antagonists: bicuculline-resistant, fast-decaying GlyR-mediated mIPSCs, and strychnine-resistant, slow-decaying GABA(A)R-mediated mIPSCs. The frequency of GABA(A)R-mediated mIPSCs was fourfold higher than that of GlyR-mediated mIPSCs at E17-18, indicating that GABAergic synaptic sites were functionally dominant at early stages of neural network formation. Properties of GABA(A)R-mediated mIPSC amplitude fluctuations changed from primarily unimodal skewed distribution at E17-18 to Gaussian mixtures with two to three discrete components at P1-3. A developmental shift from primarily long-duration GABAergic mIPSCs to short-duration glycinergic mIPSCs was evident after birth, when the frequency of GlyR-mediated mIPSCs increased 10-fold. This finding suggested that either the number of glycinergic synapses or the probability of vesicular glycine release increased during the period studied. The increased frequency of GlyR-mediated mIPSCs was associated with more than a twofold increase in their mean amplitude, and in the number of motoneurons in which mIPSC amplitude fluctuations were best fitted by multi-component Gaussian curves. A third subpopulation of mIPSCs was apparent in the absence of glycine and GABA(A) receptor antagonists: mIPSCs with both fast and slow decaying components. Based on their dual-component decay time and their suppression by either strychnine or bicuculline, we assumed that these were generated by the activation of co-localized postsynaptic glycine and GABA(A) receptors. The contribution of mixed glycine-GABA synaptic sites to the generation of mIPSCs did not change after birth. The developmental switch from predominantly long-duration GABAergic inhibitory synaptic currents to short-duration glycinergic currents might serve as a mechanism regulating neuronal excitation in the developing spinal networks.

Animals↗
PubMed2001

[Orthopedic treatment of labio-maxillo-palatal clefts: our approach].

Many authors use a preliminary orthopedic procedure before cleft lip and palate surgical closure in order to prevent possible bone distortion following the rupture of the muscle belts resulting from the cleft. Actually, this is generally not only an orthopedic treatment but rather a surgical orthopedic step which includes lip adhesion before the surgical closure of the clefts. Following the procedures proposed by Georgiade and Latham, we have used since 1996 a treatment based on traction applied with an elastic chain on splints attached by transmaxillary pins for certain types of clefts, namely unilateral complete clefts with endognathy of the small fragment, unilateral complete clefts larger than 7 mm, bilateral wide complete clefts with premaxilla protrusion, and bilateral wide complete clefts with collapsus and premaxillary protrusion. Technical procedures vary with the type of cleft. Standard procedures with or without jacks are used for the other types of complete clefts. These orthopedic procedures with elastic traction are performed between the 3rd and 6th week, before lip adhesion of the upper part of the lip (combined with release of skin and subcutaneous tissues from the underlying alar cartilage). A palatine plate with or without a jack, fitted most of the time with a spring for nostril support, is then inserted until surgical closure.

Bone Nails↗
PubMed2000

Dendrosomatic voltage and charge transfer in rat neocortical pyramidal cells in vitro.

Most excitatory synapses on neocortical pyramidal cells are located on dendrites, which are endowed with a variety of active conductances. The main origin for action potentials is thought to be at the initial segment of the axon, although local regenerative activity can be initiated in the dendrites. The transfer characteristics of synaptic voltage and charge along the dendrite to the soma remains largely unknown, although this is an essential determinant of neural input-output transformations. Here we perform dual whole-cell recordings from layer V pyramidal cells in slices from somatosensory cortex of juvenile rats. Steady-state and sinusoidal current injections are applied to characterize the voltage transfer characteristics of the apical dendrite under resting conditions. Furthermore, dendrosomatic charge and voltage transfer are determined by mimicking synapses via dynamic current-clamping. We find that around rest, the dendrite behaves like a linear cable. The cutoff frequency for somatopetal current transfer is around 4 Hz, i.e., synaptic inputs are heavily low-pass filtered. In agreement with linearity, transfer resistances are reciprocal in opposite directions, and the centroids of the synaptic time course are on the order of the membrane time constant. Transfer of excitatory postsynaptic potential (EPSP) charge, but not peak amplitude, is positively correlated with membrane potential. We conclude that the integrative properties of dendrites in infragranular neocortical pyramidal cells appear to be linear near resting membrane potential. However, at polarized potentials charge transferred is voltage-dependent with a loss of charge at hyperpolarized and a gain of charge at depolarized potentials.

Animals↗
PubMed2000

[Facial bone reconstruction. Review of particularities and procedures].

The authors report the particularities of the cranio-maxillofacial skeleton and the aims of the reconstruction procedures: osteogenesis, osteoconduction, osteoinduction. They review the various procedures, their abilities, some technical notes, their advantages and disadvantages. They analyse in order: classical bone autografts, pedicled bone flaps (arterio-osseous, fascio-osseous, myo-osseous flaps), free bone flaps, classical and pedicled periosteal transplantations. They also describe the mechanical and biological procedures of bone stimulation: bone distraction, guided bone regeneration, bone growth factors, and the biomaterials, especially coral and enosseous implants. They conclude that, in facial reconstruction, the best material is the autologous bone membranous and vascularized.

Bone Transplantation↗
PubMed1999

Analysis of NMDA-independent long-term potentiation induced at CA3-CA1 synapses in rat hippocampus in vitro.

1. Excitatory postsynaptic currents (EPSCs) were evoked at synapses formed by Schaffer collaterals/commissural (CA3) axons with CA1 pyramidal cells using the rat hippocampal slice preparation. Long-term potentiation (LTP) was induced at these synapses using a pairing protocol, with 50 microM d,l-APV present in the artificial cerebrospinal fluid (ACSF). 2. Quantal analysis of the amplitudes of the control and conditioned EPSCs showed that the enhancement of synaptic strength was due entirely to an increase in quantal content of the EPSC. No change occurred in the quantal current. 3. These results were compared with those obtained from a previous quantal analysis of LTP induced in normal ACSF, where both quantal current and quantal content increased. The results suggest that calcium entering via NMDA receptors initiates the signalling cascade that results in enhanced AMPA currents because it is adding to cytoplasmic calcium from other sources to reach a threshold for this signalling pathway, or because calcium entering via NMDA receptors specifically activates this signalling pathway.

Animals↗
PubMed1999

The L-gulono-gamma-lactone oxidase gene (GULO) which is a candidate for vitamin C deficiency in pigs maps to chromosome 14.

Vitamin C deficient pigs, when fed a diet lacking L-ascorbic acid (AscA), manifest deformity of the legs, multiple fractures, osteoporosis, growth retardation and haemorrhagic tendencies. This trait was shown by others to be controlled by a single autosomal recessive allele designated as od (osteogenic disorder). The inability of AscA biosynthesis in primates and guinea pigs that exhibit similar symptoms, when they are not supplemented with AscA in the food, was traced to the lack of L-gulono-gamma-lactone oxidase, which catalyzes the terminal step in the biosynthesis of AscA. The non-functional GULOP was mapped to human chromosome 8p21 that corresponds to an evolutionarily conserved segment on either porcine chromosome 4 (SSC4) or 14 (SSC14). We investigated linkage between OD and SSC4- and 14-specific microsatellite loci in order to map the OD locus. Twenty-seven informative meioses in families from one sire and three dams revealed linkage of od with microsatellites SW857 and S0089, located in the subcentromeric region of SSC14. We isolated part of the GULO gene of the pig by screening a porcine genomic library using a pig GULO cDNA as a probe, and mapped it to SSC14q14 by fluorescence in situ hybridization (FISH). Thus, the porcine GULO gene is both a good physiological and positional candidate gene for vitamin C deficiency in pigs.

Animals↗
PubMed1999

[Surgery of labio-maxillo-palatal clefts during humanitarian mission in the Philippines].

After describing the Philippines and the Comité de Soutien Enfance Philippines, a humanitarian association with a three-fold objective: to build, to educate and to treat, the authors present the organization and practical conduct of their missions abroad which have enabled them to treat almost 400 patients with cleft lip and palate between the ages of 3 months and 46 years.

Adolescent↗
PubMed1999

[The value of sialo-MRI in the study of salivary gland duct pathology].

Usual imaging diagnostic for salivary glands is sialography. Sialography is not stripped of disadvantages and failures. The MRI-sialography is an examination which is carried out without any injection of contrast's product (without catheterization or intravenous injection). It is thus noninvasive and painless. The complete study of salivary gland and its ducts is always possible and could not be blocked by local or loco-regional conditions. It allows exploration of several salivary glands in the same time. We think that the MRI-sialography must find its place in the diagnosis arsenal for salivary pathology in spite of its current handicaps represented by its cost and the difficulty of access to the apparatuses.

Contrast Media↗
PubMed1998

Long-term plasticity at excitatory synapses on aspinous interneurons in area CA1 lacks synaptic specificity.

The synaptic specificity of long-term potentiation (LTP) was examined at synapses formed on aspinous dendrites of interneurons whose somata were located in the pyramidal cell layer of hippocampal area CA1. Intracellular recordings from slices prepared from rats were used to monitor excitatory postsynaptic potentials (EPSPs) elicited by extracellular stimulation in stratum radiatum. Two synaptic inputs were evoked at 0.5 Hz by stimulating axons adjacent to stratum pyramidale and s. lacunosum-moleculare. After obtaining baseline recordings (>/=10 min), one of the EPSPs was conditioned. The protocol involved tetanic stimulation, sometimes combined with somatic depolarization. Low-frequency stimulation of the two pathways was then resumed and EPSPs were recorded for <30 min. We observed both homosynaptic and heterosynaptic changes in synaptic strength. LTP and long-term depression (LTD) were seen in both pathways and all possible combinations of changes in the two EPSPs were observed, including heterosynaptic LTP associated with either homosynaptic LTP or LTD. Intracellular 1,2-bis (2-aminophenoxy)-ethane-N,N,N',N'-tetraacetic acid (10 mM) abolished alterations in synaptic strength. When axons in s. radiatum synapse onto a spiny pyramidal cell, synaptic specificity of LTP is preserved. However the results obtained from aspinous interneurons show that synaptic specificity of LTP is lost. These results are consistent with the hypothesis that spines provide postsynaptic mechanism(s) for conferring specificity to LTP.

Action Potentials↗
PubMed1998

Evidence for autosomal recessive inheritance of a major gene for bovine dilated cardiomyopathy.

The objective of this study was to establish the mode of inheritance of bovine dilated cardiomyopathy (BDCMP). We analyzed a pedigree comprising 75 animals in three age classes and five diagnostic classes based on clinical and pathological findings using the Pedigree Analysis Package. Segregation analyses were performed under three models, a major gene model, a mixed model, and an environment model. Under each model three data sets were analyzed. In the first data set, only animals with clinically manifested BDCMP were considered affected; in the second data set, animals with no clinical findings but with strong pathological evidence were included in the group of affected animals; and in the third data set, this group was extended to include animals that were suspected of having BDCMP. For all three data sets, a recessive allele at a single biallelic major locus controlling the underlying liability fitted the data best. Based on Akaike's information criterion, the major gene model was the most efficient model in all data sets. We conclude that a single biallelic major locus is likely responsible for the disease.

Age Distribution↗
PubMed1998

Detection and utilization of single genes without DNA assays.

Quantitative traits are often assumed to be controlled by a large number of loci that each have a small effect. Under this assumption, the distribution of genotypic and phenotypic values can be adequately modeled by a multivariate normal distribution. Thus, most genetic analyses are based on mixed linear models. Evidence is accumulating, however, for the presence of loci that have large effects on traits of economic importance. If the genotypes for such loci can be observed without error, then--conditional on these observed genotypes--genotypic and phenotypic values follow a multivariate normal distribution, and data from very large pedigrees can be analyzed using a mixed linear model that includes the genotypic effects for these loci as fixed effects. However, when the major genotype is not observed, the genotypic and phenotypic values follow a mixture of multivariate normal distributions, and analyses based on fitting a mixed linear model may not be optimum, especially for populations undergoing selection and nonrandom mating. Several approaches are discussed for the genetic analysis of data when the major genotypes are not known.

Algorithms↗
PubMed1997

Effect, number and location of synapses made by single pyramidal cells onto aspiny interneurones of cat visual cortex.

1. Dual intracellular recordings were made from synaptically coupled pyramidal cell-to-interneurone pairs (n = 5) of the cat visual cortex in vitro. Pre- and postsynaptic neurones were labelled with biocytin, followed by correlated light and electron microscopic analysis to determine all sites of synaptic interaction. 2. Pyramidal neurones in layers II-III elicited monosynaptic EPSPs in three distinct classes of smooth dendritic local-circuit neurones, namely basket cells (n = 3), a dendrite-targeting cell (n = 1) and a double bouquet cell (n = 1). Unitary EPSPs in basket cells were mediated by one, two, and two synaptic junctions, whereas the pyramid-to-dendrite-targeting cell and pyramid-to-double bouquet cell interaction were mediated by five and seven synaptic junctions, respectively. Recurrent synaptic junctions were found on all somato-dendritic compartments, with a tendency to be clustered close to the soma on the double bouquet and dendrite-targeting cells. The latter interneurones were reciprocally connected with pyramidal cells. 3. Unitary EPSPs had an average peak amplitude of 1005 +/- 518 microV, fast rise times (10-90%; 0.67 +/- 0.25 ms) and were of short duration (at half-amplitude, 4.7 +/- 1.0 ms). Their decay was monoexponential (tau = 7.8 +/- 4.3 ms) at hyperpolarized membrane potentials and appeared to be shaped by passive membrane properties (tau = 9.2 +/- 8.5 ms). All parameters of concomitantly recorded spontaneous EPSPs were remarkably similar (mean amplitude, 981 +/- 433 microV; mean rise time, 0.68 +/- 0.18 ms; mean duration, 4.7 +/- 1.7 ms). 4. In all three pyramidal-to-basket cell pairs, closely timed (10-50 ms) pairs of presynaptic action potentials resulted in statistically significant paired-pulse depression, the mean of the averaged second EPSPs being 80 +/- 11% of the averaged conditioning event. The overall degree of paired-pulse modulation was relatively little affected by either the amplitude of the preceding event or the inter-event interval. 5. The probability density function of the peak amplitudes of the unitary EPSPs could be adequately fitted with a quantal model. Without quantal variance, however, the minimum number of components in the model, excluding the failures, exceeded the number of electron microscopically determined synaptic junctions for all five connections. In contrast, incorporating quantal variance gave a minimum number of components which was compatible with the number of synaptic junctions, and which fitted the data equally well as models incorporating additional components but no quantal variance. For this model with quantal variance with the minimum number of components the estimate of the quantal coefficient of variation ranged between 0.33 and 0.46, and the corresponding quantal sizes ranged between 260 and 657 microV. The peak EPSP amplitudes in two of the four connections with more than one synaptic junction could be adequately described by a uniform binomial model for transmitter release. 6. In conclusion, at least three distinct interneurone classes receive local excitatory pyramidal cell input which they relay to different compartments on their postsynaptic target neurones. The reliability of transmission is high, but the fast time course of the EPSPs constrains their temporal summation. Due to the relatively small amplitude of unitary EPSPs several convergent inputs will therefore be required to elicit suprathreshold responses.

Action Potentials↗
PubMed1997

Two alpha(1,2) fucosyltransferase genes on porcine chromosome 6q11 are closely linked to the blood group inhibitor (S) and Escherichia coli F18 receptor (ECF18R) loci.

The Escherichia coli F18 receptor locus (ECF18R) has been genetically mapped to the halothane linkage group on porcine Chromosome (Chr) 6. In an attempt to obtain candidate genes for this locus, we isolated 5 cosmids containing the alpha (1,2)fucosyltransferase genes FUT1, FUT2, and the pseudogene FUT2P from a porcine genomic library. Mapping by fluorescence in situ hybridization placed all these clones in band q11 of porcine Chr 6 (SSC6q11). Sequence analysis of the cosmids resulted in the characterization of an open reading frame (ORF), 1098 bp in length, that is 82.3% identical to the human FUT1 sequence; a second ORF, 1023 bp in length, 85% identical to the human FUT2 sequence; and a third FUT-like sequence thought to be a pseudogene. The FUT1 and FUT2 loci therefore seem to be the porcine equivalents of the human blood group H and Secretor loci. Direct sequencing of the two ORFs in swine being either susceptible or resistant to adhesion and colonization by F18 fimbriated Escherichia coli (ECF18) revealed two polymorphisms at bp 307 (M307) and bp 857 (M857) of the FUT1 ORF. Analysis of these mutations in 34 Swiss Landrace families with 221 progeny showed close linkage with the locus controlling resistance and susceptibility to E. coli F18 adhesion and colonization in the small intestine (ECF18R), and with the locus of the blood group inhibitor S. A high linkage disequilibrium of M307-ECF18R in Large White pigs makes the M307 mutation a good marker for marker-assisted selection of E. coli F18 adhesion-resistant animals in this breed. Whether the FUT1 or possibly the FUT2 gene products are involved in the synthesis of carbohydrate structures responsible for bacterial adhesion remains to be determined.

Adhesins, Escherichia coli↗
PubMed1997

[Unusual facial clefts].

After briefly review facial morphogenesis, the authors define facial clefts, distinguishing primary clefts, secondary clefts, and residual clefts. They discuss the uncertainties surrounding the embryology and clinical features of palpebral colobomas. The various pathogenetic concepts are analysed: amniotic hypothesis, vascular hypothesis, fusion defect. The various classifications of rare facial clefts are reviewed, with particular emphasis on Tessier's classification and the so-called Milan classification. The general principles of surgical treatment are described together with the various skeletal and soft tissues procedures.

Craniofacial Abnormalities↗