Introduction. Degos’disease or atrophic malignant papulosis is defined by porcelaine white cutaneous lesions with atrophic scarring, often associated with severe and fatal systemic involvement (visceral and neurologi...Introduction. Degos’disease or atrophic malignant papulosis is defined by porcelaine white cutaneous lesions with atrophic scarring, often associated with severe and fatal systemic involvement (visceral and neurological). Benign forms are rare or under-reported and the familial forms are exceptional. It is a very rare disease, only two hundred cases have been reported in the literature with a sex ratio of 3M/1F. The pathogenesis of Degos’disease remains controversial. The exceptional observation of familial form raises the question of a genetic predisposition of this disease or an infectious aetiology with a low virus. Observation. A 41 year-old woman was known to have Degos’disease for 26 years with only cutaneous manifestations. One of her two sons developed atrophic cutaneous lesions at the age of 20. In both patients, no thrombotic or immunological abnormalities were found. The karyotype was performed with normal results. Discussion. Degos’disease or malignant atrophic papulosis can have a long lasting benign evolution. Our patient, who had presented a benign form for 26 years, had the longest evolution ever documented in literature. We cannot be sure that her son will have a benign course of his Degos’disease because the diagnosis is recent and because the systemic involvement can appear after many years of evolution. Inthefamilialforms,fromourstudyandthe31casespreviously described in the literature, with ten different families, the course of the disease seems to be less severe than in sporadic forms. Among these familial forms of Degos’disease, only 4 patients presented a malignant form, which in one case did not prove a relationship between the death and the Degos’disease. Are the sporadic forms with only skin involvement less frequently reported? Has the familial form of Degos’disease the same course as a very severe common sporadic form?展开更多
Despite the emergence of new antiepileptic drugs, 10 to 20%of children with e pilepsy, half of whom have localization related epilepsy, remain refractory to drug treatment. Careful syndromic identification is essentia...Despite the emergence of new antiepileptic drugs, 10 to 20%of children with e pilepsy, half of whom have localization related epilepsy, remain refractory to drug treatment. Careful syndromic identification is essential before retaining t he diagnosis of intractable childhood epilepsy in order to optimize treatment an d avoid iatrogenic worsening. The use of appropriate associations of new antiepi leptic drugs should lead to better control in some situations, but further studi es are still necessary. A significant number of children with medically intracta ble lo calization related epilepsy may benefit from surgical treatment. Becaus e of the cognitive consequences of epilepsy in children, the question of the app ropriate time for surgery is still debated; the current trend is for early surge ry in children. For many authors, intractability can be assessed after 18 months of evolution, and retained when seizures persist at a frequency of one or more a month despite more than two correctly administered antiepileptic drugs. In cas e of epileptogenic encephalopathy, time to surgery may be shorter. Early predict ive criteria of intractability have been identified by several cohort studies an d include the presence of frequent seizures at disease onset, status epilepticus , with the prevalence of certain etiologies such as encephalitis or neuronal mig ration disorders. Conversely, some children may develop late intractability afte r an early benign course; the identification or early predictive criteria is sti ll unclear in this situation.展开更多
A modified plastic strain energy as hardening state parameter for dense sand was proposed, based on the results front a series of drained plane strain tests on saturated dense Japanese Toyoura sand with precise stress...A modified plastic strain energy as hardening state parameter for dense sand was proposed, based on the results front a series of drained plane strain tests on saturated dense Japanese Toyoura sand with precise stress and strain measurements along many stress paths. In addition, a unique hardening function between the plastic strain energy and the instantaneous stress path was also presented, which was independent of stress history. The proposed state parameter and hardening function was directly verified by the simple numerical integration method. It is shown that the proposed hardening function is independent of stress history and stress path and is appropriate to be used as the hardening rule in constitutive modeling for dense sand, and it is also capable of simulating the effects on the de-formation characteristics of stress history and stress path for dense sand.展开更多
文摘Introduction. Degos’disease or atrophic malignant papulosis is defined by porcelaine white cutaneous lesions with atrophic scarring, often associated with severe and fatal systemic involvement (visceral and neurological). Benign forms are rare or under-reported and the familial forms are exceptional. It is a very rare disease, only two hundred cases have been reported in the literature with a sex ratio of 3M/1F. The pathogenesis of Degos’disease remains controversial. The exceptional observation of familial form raises the question of a genetic predisposition of this disease or an infectious aetiology with a low virus. Observation. A 41 year-old woman was known to have Degos’disease for 26 years with only cutaneous manifestations. One of her two sons developed atrophic cutaneous lesions at the age of 20. In both patients, no thrombotic or immunological abnormalities were found. The karyotype was performed with normal results. Discussion. Degos’disease or malignant atrophic papulosis can have a long lasting benign evolution. Our patient, who had presented a benign form for 26 years, had the longest evolution ever documented in literature. We cannot be sure that her son will have a benign course of his Degos’disease because the diagnosis is recent and because the systemic involvement can appear after many years of evolution. Inthefamilialforms,fromourstudyandthe31casespreviously described in the literature, with ten different families, the course of the disease seems to be less severe than in sporadic forms. Among these familial forms of Degos’disease, only 4 patients presented a malignant form, which in one case did not prove a relationship between the death and the Degos’disease. Are the sporadic forms with only skin involvement less frequently reported? Has the familial form of Degos’disease the same course as a very severe common sporadic form?
文摘Despite the emergence of new antiepileptic drugs, 10 to 20%of children with e pilepsy, half of whom have localization related epilepsy, remain refractory to drug treatment. Careful syndromic identification is essential before retaining t he diagnosis of intractable childhood epilepsy in order to optimize treatment an d avoid iatrogenic worsening. The use of appropriate associations of new antiepi leptic drugs should lead to better control in some situations, but further studi es are still necessary. A significant number of children with medically intracta ble lo calization related epilepsy may benefit from surgical treatment. Becaus e of the cognitive consequences of epilepsy in children, the question of the app ropriate time for surgery is still debated; the current trend is for early surge ry in children. For many authors, intractability can be assessed after 18 months of evolution, and retained when seizures persist at a frequency of one or more a month despite more than two correctly administered antiepileptic drugs. In cas e of epileptogenic encephalopathy, time to surgery may be shorter. Early predict ive criteria of intractability have been identified by several cohort studies an d include the presence of frequent seizures at disease onset, status epilepticus , with the prevalence of certain etiologies such as encephalitis or neuronal mig ration disorders. Conversely, some children may develop late intractability afte r an early benign course; the identification or early predictive criteria is sti ll unclear in this situation.
文摘A modified plastic strain energy as hardening state parameter for dense sand was proposed, based on the results front a series of drained plane strain tests on saturated dense Japanese Toyoura sand with precise stress and strain measurements along many stress paths. In addition, a unique hardening function between the plastic strain energy and the instantaneous stress path was also presented, which was independent of stress history. The proposed state parameter and hardening function was directly verified by the simple numerical integration method. It is shown that the proposed hardening function is independent of stress history and stress path and is appropriate to be used as the hardening rule in constitutive modeling for dense sand, and it is also capable of simulating the effects on the de-formation characteristics of stress history and stress path for dense sand.
