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Dunnigan syndrome, or Familial Partial Lipodystrophy type 2 (FPLD2; ORPHA 2348), is a rare autosomal dominant disorder due to pathogenic variants of the LMNA gene. The objective of the French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins), is to provide health professionals with a guide to optimal management and care of patients with FPLD2, based on a critical literature review and multidisciplinary expert consensus. The PNDS, written by members of the French National Reference Center for Rare Diseases of Insulin Secretion and Insulin Sensitivity (PRISIS), is available on the French Health Authority website (in French). Dunnigan syndrome is characterized by a partial atrophy of the subcutaneous adipose tissue and by an insulin resistance syndrome, associated with a risk of metabolic, cardiovascular and muscular complications. Its prevalence, assessed at 1/100.000 in Europe, is probably considerably underestimated. Thorough clinical examination is key to diagnosis. Biochemical testing frequently shows hyperinsulinemia, abnormal glucose tolerance and hypertriglyceridemia. Elevated hepatic transaminases (hepatic steatosis) and creatine phosphokinase, and hyperandrogenism in women, are common. Molecular analysis of the LMNA gene confirms diagnosis and allows for family investigations. Regular screening and multidisciplinary monitoring of the associated complications are necessary. Diabetes frequently develops from puberty onwards. Hypertriglyceridemia may lead to acute pancreatitis. Early atherosclerosis and cardiomyopathy should be monitored. In women, polycystic ovary syndrome is common. Overall, the management of patients with Dunnigan syndrome requires the collaboration of several health care providers. The attending physician, in conjunction with the national care network, will ensure that the patient receives optimal care through regular follow-up and screening. The various elements of this PNDS are described to provide such a support. [ABSTRACT FROM AUTHOR]

Pinning of dislocations at nanosized obstacles like precipitates, voids, and bubbles is a crucial mechanism in the context of phenomena like hardening and creep. The interaction between such an obstacle and a dislocation is often studied at fundamental level by means of analytical tools, atomistic simulations, and finite element methods. Nevertheless, the information extracted from such studies cannot be utilized to its maximum extent on account of insufficient information about the underlying statistics of this process comprising a large number of dislocations and obstacles in a system. Here, we propose a new statistical approach, where the statistics of pinning of dislocations by idealized spherical obstacles is explored by taking into account the generalized size-distribution of the obstacles along with the dislocation density within a three-dimensional framework. Starting with a minimal set of material parameters, the framework employs the method of geometrical statistics with a few simple assumptions compatible with the real physical scenario. The application of this approach, in combination with the knowledge of fundamental dislocationobstacle interactions, has successfully been demonstrated for dislocation pinning at nanovoids in neutron irradiated type 316-stainless steel in regard to the non-conservative motion of dislocations. An interesting phenomenon of transition from rare pinning to multiple pinning regimes with increasing irradiation temperature is revealed [ABSTRACT FROM AUTHOR]

Purpose: Testotoxicosis is an autosomal dominant form of limited gonadotropin-independent precocious puberty in boys. It is caused by a heterozygous constitutively activating mutation of the LHCGR gene encoding the luteinizing/hormone receptor (LHR). Some twenty mutations of the LHCGR gene have been reported. Most of them are constitutive mutations isolated from blood leukocyte DNA, although others are somatic, found only in testicular tumoural tissue. In all the previously reported cases of these somatic mutations, the tumour, whether a nodular Leydig cell adenoma or hyperplasia, was easily visible on testicular ultrasonography. The aim of this study was to describe an unusual presentation of a patient with the clinical and hormonal characteristics of testotoxicosis but no well-circumscribed lesion at testicular ultrasonography. Materials and Methods: Molecular analysis of the LHCGR gene was performed by direct sequencing of DNA extracted from peripheral leucocytes and testicular biopsy. Results: Molecular analysis didn't find any LHR mutation in blood, whereas it revealed for the first time a somatic D578H mutation in testicular tissue despite no evidence of a nodular aspect at testis ultrasonography. Conclusions: This observation underlines the need to look for a somatic LHCGR gene mutation from the testicular biopsies of all boys with testotoxicosis with no constitutive LHCGR gene mutation identified from blood DNA, even in the absence of circumscribed testicular lesion at ultrasonography. In addition, based on the known link between LHR mutations and testicular tumourigenesis, yearly ultrasound monitoring of the testes should be considered for these patients. [ABSTRACT FROM AUTHOR]

Presents a surface analysis of bismuth-based cuprates superconductors through secondary ion mass spectrometry and X-ray photoelectron spectroscopy. Properties of cuprates superconductors; Discussion on kinetic energy distributions for secondary negative ions; Explanation for the emission of small clusters in bismuth-based cuprates.

Presents a study which examined the superconducting compound YBa[sub2]Cu[sub3]O[sub7-x] and oxides of copper by secondary-ion-mass spectrometry (SIMS). Experimental procedures; Results; Conclusions.

Microcephalic primordial dwarfisms are a group of rare Mendelian disorders characterized by severe growth retardation and microcephaly. The molecular basis is heterogeneous, with disease‐causing genes implicated in different cellular functions. Recently, 2 patients were reported with the same homozygous variant in the WDR4 gene, coding for an enzyme responsible for the m7G46 post transcriptional modification of tRNA. We report here 2 sisters harboring compound heterozygous variants of WDR4. Their phenotype differs from that of the first 2 described patients: they both have a severe microcephaly but only one of the 2 sisters had a head circumference at birth below −2 SD, their intellectual deficiency is less severe, and they have a growth hormone deficiency and a partial hypogonadotropic hypogonadotropism. One of the 2 variants is a frameshift mutation, and the other one is a missense occurring in the same nucleotide affected by the first reported pathogenic variant, which could therefore be a mutational hot spot. The description of these 2 sisters allow us to confirm that biallelic variants in the WDR4 gene can lead to a specific phenotype, characterized by severe growth retardation and microcephaly. [ABSTRACT FROM AUTHOR]

Aim: The RePPOP Aquitaine network, which was established in south-west France to prevent and treat paediatric obesity, has developed a multidisciplinary approach based on multicomponent lifestyle interventions and family-based actions. This study assessed the impact of its care management programme and investigated the factors associated with better outcomes.Methods: The impact of the RePPOP care management programme was assessed by changes in the body mass index (BMI) Z score, between baseline and the end-of-care management. We focused on 982 overweight or obese children (59.9% girls) with a mean age of 10.64 years and a range of 2.4-17.9 years. A multivariate analysis examined the independent factors associated with better outcomes.Results: At the end-of-care management programme, 75.5% of children had decreased their BMI Z score. Initial characteristics significantly associated with better outcomes were as follows: the age at baseline between five years and 15 years of age, playing sport at a club, being followed up by RePPOP for longer than 10 months, no parental obesity and no academic difficulties.Conclusion: This study confirmed that multidisciplinary treatment had a significant positive effect on paediatric obesity and that social and individual factors affected the efficiency of the care management. [ABSTRACT FROM AUTHOR]

We study the measure of complexity in solid Argon system from the time series data of kinetic energy of single Argon atoms at different equilibrated temperatures. To account the inherent multi-scale dependence of the complexity, the multi-scale entropy of the time series of kinetic energy of individual Argon atoms are computed at different equilibrated temperatures. The multi-scale entropy study reveals that the dynamics of an atom becomes more complex at higher temperatures and the result corroborates well with the variation of the pair correlation function of the atoms in the solid Argon crystal. Also, we repeat the multi-scale entropy analysis for synthetically generated Levy noise time series and for time series data obtained from the outcomes of exponential decay with noise $$\,d x(t)=-x(t)\,dt+\sigma \,dB(t)$$ (Langevin equation). Our study establishes that the scale dependence of sample entropy for time series of kinetic energy of individual atoms in solid Argon system has similar tendency as that of Levy noise time series and the outcomes of exponential decay with noise (Langevin equation). [ABSTRACT FROM AUTHOR]

In this letter we show that the diffusion kinetics of kinetic energy among the atoms in non-equilibrium crystalline systems follows a universal scaling relation and obeys Levy-walk properties. This scaling relation is found to be valid for systems no matter how far they are driven out of equilibrium. [ABSTRACT FROM AUTHOR]

A simplified instrumentation with nominal circuitry has been developed to measure in- and out-of-phase complex magnetic susceptibility of bulk magnetic samples. A unique shape of the absolute coil is implemented to improve the magnetic field homogeneity inside the coil. Complex susceptibility of nano-composite is measured by an innovative phase detection circuit. In this paper, we also discuss in detail the principle, design, and the performance of the instrumentation. This susceptometer is also suitable for liquid samples, viz., ferro-fluids, blood samples, etc. [ABSTRACT FROM PUBLISHER]

An experimental study is presented on the effect of microstructure change on the Portevin-Le Chatelier (PLC) effect of Al-2.5 pct Mg alloy. Tensile tests are performed on as-received and heat-treated [at 673 K (400 °C) for 16 hours] samples for a wide range of strain rates. The serrations observed in the stress-time curves are investigated on statistical analysis point of view. The microstructures of the samples are characterized by optical metallography, X-ray diffraction, and electrical resistivity measurements. It is found that the excess vacancies generated due to heat treatment have an effect on the PLC phenomenon, which leads to a decrease in the strain rate sensitivity and an increase in the number of stress drop occurrences per unit time. The microstructural parameters like domain size and dislocation density have no appreciable effect on the PLC effect as far as the statistical behaviors of the serrations are considered. [ABSTRACT FROM AUTHOR]

In this study, samples of zircaloy-2 were irradiated with oxygen ion beam (O5+ ions with 116 MeV energy) to fluence varying from 1013 to 1015 ion/cm2. The irradiated samples were observed under a transmission electron microscope to identify the irradiation-induced defects and phase transformations. Microstructural investigations on these samples showed the presence of interstitial defects and ZrO2 precipitates in the α (hcp) matrix. The alignment of defects appeared to facilitate the formation of the ZrO2 phase in the irradiated samples. Orientation relationship between the oxide and the matrix phase was determined and a possible mechanism for the formation of the ZrO2 phase based on the entrapment of the oxygen ions has been proposed. [ABSTRACT FROM AUTHOR]

The Portevin-Le Chatelier (PLC) effect is a kind of plastic instability observed in many dilute alloys when deformed at certain ranges of strain rate and temperature. In this paper we present a comprehensive statistical analysis of the observed experimental data obtained during PLC effect and establish that the occurrence probability of the stress drops in the dynamical process responsible for PLC effect is Poisson in nature. [ABSTRACT FROM AUTHOR]

Zr-1Nb samples were irradiated with 116 MeV O ions at different doses ranging from 5 × 10 to 8 × 10 O/m. X-ray diffraction line profile analysis was performed to characterize the microstructural parameters of these samples. Average domain size, microstrain and dislocation density were estimated as a function of dose. An anomaly was observed in the values of these parameters at a dose of 2 × 10 O/m. Positron annihilation spectroscopy was used to determine the existence and nature of vacancy clusters in the samples. Isochronal annealing was carried out for a sample to study the evolution of defect clusters. [ABSTRACT FROM AUTHOR]

Tensile tests were carried out by deforming polycrystalline samples of Al-2.5 pct Mg alloy at four different temperatures in an intermediate strain rate regime of 2 × 10 s to 2 × 10 s. The Portevin-Le Chatelier (PLC) effect was observed throughout the strain rate and temperature region. The mean cumulative stress drop magnitude and the mean reloading time exhibit an increasing trend with temperature, which is attributed to the enhanced solute diffusion at higher temperature. The observed stress-time series data were analyzed using the nonlinear dynamical methods. From the analyses, we established the presence of deterministic chaos in the PLC effect throughout the temperature regime. The dynamics goes to higher dimension at a sufficiently high temperature of 425 K (152 °C), but the complexity of the dynamics is not affected by the temperature. [ABSTRACT FROM AUTHOR]

We have observed prominent change in the velocity of dislocation in thin films due to the proximity of free surfaces using molecular dynamics simulations. A simple model has been introduced and the numerical implementation of our model is in accord with the simulation results. Such surface effects have been attributed to the change in lattice resistance to dislocation motion. [ABSTRACT FROM AUTHOR]

The complexity of the Portevin-Le Chatelier effect in Al-2.5%Mg polycrystalline samples subjected to uniaxial tensile tests is quantified. Multiscale entropy analysis is carried out on the stress time series data observed during jerky flow to quantify the complexity of the distinct spatiotemporal dynamical regimes. It is shown that for the static type C band, the entropy is very low for all the scales compared to the hopping type B and the propagating type A bands. The results are interpreted considering the time and length scales relevant to the effect. [ABSTRACT FROM AUTHOR]

Tensile tests have been carried out by deforming polycrystalline samples of substitutional Al-2.5%Mg alloy at room temperature at a range of strain rates. The Portevin-Le Chatelier (PLC) effect was observed. From an analysis of the experimental stress versus time series data we have inferred that the dynamics of the PLC effect in a local finite time is controlled by a finite number of degrees of freedom and this effective dimension becomes reduced with increasing strain. [ABSTRACT FROM AUTHOR]

This overview reports some of the research works carried out by us in recent past on the application of artificial neural network (ANN) based modeling in alloy design and thermomechanical processing of austenitic stainless steels. Different ANN models were created in order to simulate various correlations and phenomena in austenitic stainless steels. These include: prediction of mechanical properties of alloy D9 from its alloy content, modeling constitutive flow behavior of austenitic stainless steels, and prediction of torsional flow behavior of type 304L stainless steel. Attempt has been made to explain the simulated results by relevant fundamental metallurgical phenomena. [ABSTRACT FROM AUTHOR]

Determination of lattice misfit and microstructural parameters of the coherent precipitates in Ni based alloy Inconel-625 is a challenging problem as the diffraction peaks of the precipitate and the matrix are completely overlapping. X-ray Diffraction Line Profile Analysis (XRDLPA) has been used to characterize the bulk samples of Inconel 625 at different heat-treated conditions to determine the lattice parameters of the parent phase and also the coherent precipitates by the separation of the overlapping peaks. The lattice misfits of the coherent precipitates with the matrix and their microstructural parameters like size and strain have also been determined. [ABSTRACT FROM AUTHOR]

The heart beat data recorded from samples before and during meditation are analyzed using two different scaling analysis methods. These analyses revealed that meditation severely affects the long range correlation of heart beat of a normal heart. Moreover, it is found that meditation induces periodic behavior in the heart beat. The complexity of the heart rate variability is quantified using multiscale entropy analysis and recurrence analysis. The heart beat during meditation is found to be more complex. [ABSTRACT FROM AUTHOR]

In this article an artificial neural network based system to predict weld bead geometry using features derived from the infrared thermal video of a welding process is proposed. The multilayer perceptron and radial basis function networks are used in the prediction model and an online feature selection technique prioritises the features used in the prediction model. The efficacy of the system is demonstrated with a number of welding experiments and using the leave one out cross-validation experiments. [ABSTRACT FROM AUTHOR]

Molecular dynamics simulation study based on the EAM potential is carried out to investigate the effect of pressure on the rapid solidification of Aluminum. The radial distribution function is used to characterize the structure of the Al solidified under different pressures. It is indicated that a high pressure leads to strong crystallization tendency during cooling. [ABSTRACT FROM AUTHOR]

Zirconium-based alloys have been irradiated with 11 and 15 MeV proton and 116 MeV oxygen ions at different doses. The changes in the microstructure have been studied for the ion-irradiated alloys as a function of dose using X-ray diffraction line profile analysis (XRDLPA) based on the whole powder pattern fitting technique. It was observed that the microstructural parameters such as domain size, microstrain within the domain, and dislocation density did not change significantly with the increase in dose for proton-irradiated samples. A clear change was noticed in these microstructural parameters as a function of dose for oxygen-irradiated samples. There was a drastic decrease in domain size at a dose of 1 × 1017 O5+/m2, but these values reached a plateau with increasing dose. The values of microstrain and dislocation density increased significantly with the dose of irradiation. [ABSTRACT FROM AUTHOR]

Two sets of amorphous carbon materials prepared at different routes are irradiated with swift (145 MeV) heavy ion (Ne6+). The structural parameters like the extent of local ordering along the c and a axis i.e., Lc and La, the average spacing of the crystallographic planes (002) i.e., d002 and the fraction of the amorphous phase of the unirradiated and the irradiated samples are estimated by X-ray diffraction technique. The fraction of the amorphous phase is generally found to increase with the irradiation dose for both sets of the samples. The estimated d002 values are found to be almost unaffected by irradiation but Lc and La are decreased slightly with irradiation. The estimated values of Lc and La corroborate with the increase of disorder in both sets of the samples with the increasing dose of irradiation. [ABSTRACT FROM AUTHOR]

The use of graphite as a moderator in a low temperature thermal nuclear reactor is restricted due to accumulation of energy caused by displacement of atoms by neutrons and high energetic particles. Thermal transients may lead to a release of stored energy that may raise the temperature of the fuel clad above the design limit. Disordered carbon is thought to be an alternative choice for this purpose. Two types of disordered carbon composites, namely, CB (made up of 15 wt. % carbon black dispersed in carbonized phenolic resin) and PAN (made up of 20 vol. % chopped polyacrylonitrile carbon fibre dispersed in carbonized phenolic resin matrix) have been irradiated with 145 MeV Ne6+ ions at three fluence levels of 1.0×1013, 5.0×1013 and 1.5×1014 Ne6+/cm2, respectively. The XRD patterns revealed that both the samples remained disordered even after irradiation. The maximum release of stored energy for CB was 212 J/g and that of PAN was 906 J/g. For CB, the release of stored energy was a first order reaction with activation energy of 2.79 eV and a frequency factor of 3.72×1028 per second. 13% of the defects got annealed by heating up to 700 °C. PAN showed a third-order release rate with activation energy of 1.69 eV and a frequency factor of 1.77×1014 per second. 56% of the total defects got annealed by heating it up to 700 °C. CB seems to be the better choice than PAN as it showed less energy release with a slower rate. [ABSTRACT FROM AUTHOR]

The time series data of the monthly rainfall records (for the time period 1871–2002) in All India and different regions of India are analyzed. It is found that the distributions of the rainfall intensity exhibit perfect power law behavior. The scaling analysis revealed two distinct scaling regions in the rainfall time series. [ABSTRACT FROM AUTHOR]

The plastic deformation of dilute alloys is often accompanied by plastic instabilities due to dynamic strain aging and dislocation interaction. The repeated breakaway of dislocations from and their recapture by solute atoms leads to stress serrations and localized strain in the strain controlled tensile tests, known as the Portevin-Le Chatelier (PLC) effect. In this present work, we analyze the stress time series data of the observed PLC effect in the constant strain rate tensile tests on Al-2.5%Mg alloy for a wide range of strain rates at room temperature. The scaling behavior of the PLC effect was studied using two complementary scaling analysis methods: the finite variance scaling method and the diffusion entropy analysis. From these analyses we could establish that in the entire span of strain rates, PLC effect showed Levy walk property. Moreover, the multiscale entropy analysis is carried out on the stress time series data observed during the PLC effect to quantify the complexity of the distinct spatiotemporal dynamical regimes. It is shown that for the static type C band, the entropy is very low for all the scales compared to the hopping type B and the propagating type A bands. The results are interpreted considering the time and length scales relevant to the effect. [ABSTRACT FROM AUTHOR]

Microstructural parameters such as the average domain size, effective domain size at a particular crystallographic direction, and microstrain within the domains of titanium and Ti-5 pct Ta-2 pct Nb, irradiated with 116 MeV O5+ ion, have been characterized as a function of dose by X-ray diffraction line-profile analysis (XRDLPA) using different model-based approaches. The dislocation density and stacking-fault probabilities have also been estimated from the analysis. The analysis revealed that there was a significant decrease of the average domain size with dose as compared to the unirradiated sample. The estimated values of dislocation density increased significantly for the irradiated samples and were found to be an order of magnitude more as compared to the unirradiated one. However, the dislocation density became saturated with an increase in dose. The deformation (stacking-fault) probabilities were found to be negligible even with the increase in dose of irradiation. [ABSTRACT FROM AUTHOR]

X-ray diffraction (XRD) intensity data from five different alloys of aluminum-zirconium-lithium and aluminum-magnesium-lithium, having extensive application in aircraft industries, were measured with a PHILIPS PW 1730 X-ray diffractometer. The microstructural parameters like coherent domain size, microstrain within the domain, and stacking faults were evaluated in the deformed lattice of the alloys by applying Fourier line-shape analysis. It has been observed in the deformed lattice that deformation faults, both intrinsic and extrinsic, are absent, whereas the twin fault has significant presence in the lattice and shows systematic changes with the increase of lithium content. [ABSTRACT FROM AUTHOR]

Commercial aluminum rolling mills operate under very restricted thermomechanical conditions determined from experience and plant trials. In this paper we report results for four-stand tandem mill rolling simulations within and beyond the thermomechanical conditions typical of a rolling mill by plane strain compression (PSC) testing to assess the effect of deformed conditions on the texture of the hot deformed aluminum strip after annealing. A neural network modeling study was then initiated to find a predictive relationship between the observed texture and the thermomechanical parameters of strain, strain rate, and temperature. The model suggested that temperature is the prime variable that influences texture. Such models can be used to evaluate optimal strategies for the control of process parameters of a four-stand tandem mill. [ABSTRACT FROM AUTHOR]

We report the case of a 20-year-old female patient with Beckwith-Wiedemann syndrome presenting with high blood pressure and bilateral adrenal pheochromocytoma successfully removed with laparoscopy in the same time. To our knowledge, the present case is the first observation of a bilateral pheochromocytoma occurring in the Beckwith-Wiedemann syndrome. It provides further support for a genetic anomaly in this condition. Our case also indicates the interest of laparoscopy for the surgical treatment of adrenal pheochromocytoma, even in bilateral tumors. [ABSTRACT FROM AUTHOR]

X-ray diffraction intensity data from three compositions of zirconium-base alloys, viz. ZIRCALOY-2, ZIRLO, and Zr-25 pct Nb, having extensive application in nuclear industry were recorded using a PHILIPS PW 1730 X-ray diffractometer. Detailed studies using the recorded diffractometer data, keeping in view the recent trends in the powder diffraction analysis, were carried out with a view to evaluating the microstructural paramaters, including domain size, microstrain, faulting probability, and dislocation density. While faulting appears to be absent in all alloys, the average domain size and root-mean-square (rms) strain appear to be smaller compared to pure zirconium. However, both of these parameters, i.e., average domain size and rms strain tend to increase with the addition of Nb, as in ZIRLO, but display a diminishing trend with higher content of Nb, as is evident in the Zr-2.5 pct Nb alloy. [ABSTRACT FROM AUTHOR]

For the first time the concept of fractal geometry is introduced to characterize discrete ultrasonic time domain signals from a single crystal, a polycrystalline metal, and an alloy. These signals possess unique fractal dimensions and remain invariant with the change in sampling rate of signal capturing. The fractal dimension of these signals were evaluated by both the box counting method and the power spectrum method. The similarity and difference in the fractal dimension of these signals obtained from the above two methods have been discussed. © 1995 Acoustical Society of America. [ABSTRACT FROM AUTHOR]

Since the beginning of the present century students of Indian history have been making strenuous efforts to collect such materials as would help them to reconstruct the early history of Bengal. But so far they have not succeeded in ascertaining definitely even the dates of those kings of the Sena Dynasty who governed dominions of large extent and took rank among the great powers. The discovery of the era with which is associated the name of Laksmana Sena Deva induced several well-known archaeologists to bring its initial date to bear on the history of Bengal. From the scanty data which were then available the late Professor Kielhorn after much laborious calculation definitely settled that the Lakṣmaṇa Samvat or La-Sam began in A.D. 1119–20. According to him the La-Sam was an ordinary Southern (Kārttikādi) year with Amānta scheme of lunar fortnights; and the first date of the era was October 7, A.D. 1119. As this date has not been made use of in reconstructing the chronology of the Sena kings, it may be accepted for the present; and time will show whether the conclusion of the learned doctor is right or wrong. But the assertion of the historians that the initial date of the Lakṣmaṇa Sena era synchronizes with the commencement of Lakṣmaṇa Sena's reign is quite untenable and can never be accepted as true. In Indian history there is no era which does not commemorate some epoch-making event which affected the people of the country in general. And ordinary succession to the throne in its normal course, as was the case with Lakṣmaṇa Sena Deva, does not justify the inauguration of an era in place of the usual regnal years to which the people in those days were accustomed. [ABSTRACT FROM PUBLISHER]

Plastic flow behaviour of low carbon steel has been studied at room temperature during tensile deformation by varying the initial strain rate of 3.3 x 10-4 s-1 to a final strain rate ranging from 1.33 x 10-3 s-1 to 2 x 10-3 s-1 at a fixed engineering strain of 12%. Haasen plot revealed that the mobile dislocation density remained almost invariant at the juncture where there was a sudden increase in stress with a change in strain rate and the plastic flow was solely dependent on the velocity of mobile dislocations. In that critical regime, the variation of stress with time was fitted with a Boltzmann type Sigmoid function. The increase in stress was found to increase with final strain rate and the time elapsed in attaining these stress values showed a decreasing trend. Both of these parameters saturated asymptotically at a higher final strain rate. [ABSTRACT FROM AUTHOR]

Unlabelled: We report the cases of two sibs of North African origin with AAAS gene mutation characterized by the heterogeneity of their phenotype. While an 8-y-old boy presented with acute adrenal insufficiency and mental retardation, the diagnosis was suggested by the clinical history of his 6-y-old sister who had symptomatic achalasia and chronic adrenal failure.Conclusion: Our observations corroborate the phenotypic heterogeneity reported in triple A syndrome, and underline the possibility of a variable intra-familial expression. [ABSTRACT FROM AUTHOR]