Annales De Medecine Interne[JOURNAL]
Sun
200 papers
[Fabry's disease and hypoparathyroidism].
Misery L, Gregoire M, Prieur F … +7 more , Froissart R, Guffon N, Maitre S, Fond L, Denis L, Perrot JL, Cambazard F
Ann Med Interne (Paris) · 2002 Jun · PMID 12218896
INTRODUCTION: Fabry's disease is due to alpha-galactosidase deficiency. This rare lysosomal storage disease is transmitted by recessive X-linked heredity. Sphingolipids (galactosyl-glucosyl-ceramide) accumulate in many o...
INTRODUCTION: Fabry's disease is due to alpha-galactosidase deficiency. This rare lysosomal storage disease is transmitted by recessive X-linked heredity. Sphingolipids (galactosyl-glucosyl-ceramide) accumulate in many organs. CASE REPORT: A 19-year-old man with known hypoparathyroidism presented with telangiectasia and angiokeratomas on the buttocks, the hips, the hands and around the navel. For many years, he suffered from paroxysmal pain in the hands and feet. From childhood, he had complained of diffuse abdominal pain, associated with diarrhea. Ophthalmological slit lamp fundus examination showed corneal telangiectasia and cornea verticella. There was no kidney or heart involvement. The diagnosis of Fabry's disease was confirmed by very low levels of alpha-galactosidase. DISCUSSION: We did not find any other association of hypoparathyroidism and Fabry's disease in the literature. Hypoparathyroidism is not a manifestation of Fabry's disease. Idiopathic hypoparathyroidism is very rare and a genetic origin is known. This disease can be recessive X-linked. A co-transmission of idiopathic hypoparathyroidism and Fabry's disease is probable in our patient.
[Sjogren-Larsson syndrome: two cases with delayed diagnosis].
Misery L, Antoine JC, Touraine R … +5 more , Wanders R, Maitre S, Has C, Perrot JL, Cambazard F
Ann Med Interne (Paris) · 2002 Jun · PMID 12218895
INTRODUCTION: Sjögren-Larsson syndrome (SJS) is an autosomal-recessive disorder. Patients suffer from congenital ichtyosis, mental retardation and symmetric spastic paralysis. Ichtyosis is usually pronounced and associat...
INTRODUCTION: Sjögren-Larsson syndrome (SJS) is an autosomal-recessive disorder. Patients suffer from congenital ichtyosis, mental retardation and symmetric spastic paralysis. Ichtyosis is usually pronounced and associated with erythroderma. Neurological manifestations occur usually between 4 and 13 months of age. This genetic disease is due to fatty acid aldehyde dehydrogenase (FALDH) deficiency, leading to an accumulation of long-chain alcohols. The gene has been mapped to chromosome 17. CASE REPORTS: A 52-year-old woman was hospitalized because of a severe erythroderma with ichtyosis. She suffered from epilepsy, spastic diplegia and mental retardation (Little disease has been diagnosed). The association of spastic paraparesia and ichtyosiform erythroderma suggested the diagnosis of SJS. This was confirmed by the very low level of FALDH activity. A 27-year-old patient was hospitalized for the recent onset paraparesia. Erythematous patches were observed on arm pits and buttocks. The diagnosis of SJS was not confirmed by FALDH assay. DISCUSSION: Diagnosis of Sjögren-Larsson syndrome is a very rare disease in France. It is useful to evoke the diagnosis when spastic paraparesia is associated with these unusual cutaneous signs.
[Rectal endocrine intermediate-cell carcinoma].
Béchade D, Desramé J, Berthelet O … +4 more , Lecoules S, Coutant G, Arborio M, Algayres JP
Ann Med Interne (Paris) · 2002 Jun · PMID 12218894
An 82-year-old patient complained of diarrhea due to a rectal endocrine intermediate-cell carcinoma. Histology displayed a neuron-specific enolase and CD56 immunoreactive tumor. Hepatic metastases developed rapidly and t...
An 82-year-old patient complained of diarrhea due to a rectal endocrine intermediate-cell carcinoma. Histology displayed a neuron-specific enolase and CD56 immunoreactive tumor. Hepatic metastases developed rapidly and the tumor was briefly reactive to radiotherapy and chemotherapy. These tumors are rare and have a poor prognosis. We focus on the recent classification of gastrointestinal endocrine tumors.
[Long-term treatments for systemic sclerosis: what are the perspectives?].
Mouthon L, Agard C, Garcia De La Peña-Lefebvre P … +1 more , Guillevin L
Ann Med Interne (Paris) · 2002 Jun · PMID 12218893
Systemic sclerosis (SSc) is responsible for fibrosis of the dermis and other organs as well as vascular abnormalities. While the pathogenesis of SSc is continually being better understood, there is still no single therap...
Systemic sclerosis (SSc) is responsible for fibrosis of the dermis and other organs as well as vascular abnormalities. While the pathogenesis of SSc is continually being better understood, there is still no single therapeutic agent that has been shown to increase survival in a prospective randomized trial. Traditional medications such as colchicine and D-penicillamine are disappointing in clinical practice, and the latter one failed to clearly show benefit when tested in a prospective placebo controlled trial comparing conventional high dose versus low dose. Conversely, new disease modifying agents are emerging such as cyclophosphamide (CYC) in interstitial pulmonary disease and stem cell autograft after high dose CYC therapy in patients who develop visceral involvement in the three first years of evolution of the disease. Organ specific therapy may show dramatic benefit, such as angiotensin converting enzyme inhibitors in renal crisis and epoprostenol in primary pulmonary hypertension. We will try to review disease modifying agents available in SSc and emphasize new therapeutic agents that are currently being evaluated, including vasodilators, anti-inflammatory, anti-fibrosing agents and immunosuppressive molecules.
[Digestive manifestations in systemic sclerosis].
Attar A
Ann Med Interne (Paris) · 2002 Jun · PMID 12218892
Gastrointestinal involvement occurs in most patients with systemic sclerosis. Pathology is characterized by vasculopathy, resulting in tissue ischemia, progressive dysfunction and fibrosis. In its diffuse and visceral pa...
Gastrointestinal involvement occurs in most patients with systemic sclerosis. Pathology is characterized by vasculopathy, resulting in tissue ischemia, progressive dysfunction and fibrosis. In its diffuse and visceral pattern, digestive manifestations may involve most of the intestinal tract and are the most frequent before renal, cardiac and pulmonary involvement. Whatever the visceral extension, about 80% of patients have digestive manifestations including gastroesophageal reflux, abnormalities of intestinal motility leading to chronic intestinal pseudo-obstruction and small bowel bacterial overgrowth and malnutrition. Long-term treatment of reflux with high-dose proton pump inhibitors appears safe and effective for symptom relief and may prevent recurrence of esophagitis and stricture. Prokinetic agents effective in pseudoobstruction include metoclopramide, domperidone, octreotide, and erythromycin.
[Pulmonary hypertension associated with systemic sclerosis].
Sanchez O, Nunes H, Sitbon O … +3 more , Garcia G, Simonneau G, Humbert M
Ann Med Interne (Paris) · 2002 Jun · PMID 12218891
Pulmonary hypertension is a rare but well known life-threatening complication of scleroderma and particularly in its limited variant, the CREST syndrome (Calcification, Raynaud phenomenon, Esophageal dysmotility, Sclerod...
Pulmonary hypertension is a rare but well known life-threatening complication of scleroderma and particularly in its limited variant, the CREST syndrome (Calcification, Raynaud phenomenon, Esophageal dysmotility, Sclerodactily, Telangiectasia). The aim of this article is to analyze the available literature and to report the experience of a center for pulmonary vascular diseases. Dyspnea is the main symptom and is frequently severe. Echocardiography is an excellent tool to detect pulmonary hypertension. However, right-heart catheterization is necessary to confirm the diagnosis of pulmonary hypertension and to test vasoreactivity with a potent vasodilator such as nitric oxide. Hemodynamic parameters are less severe in patients with connective tissue diseases perhaps because of an earlier diagnosis. A significant lower proportion of patients presents an acute vasodilator response suggesting an early constitution of irreversible pulmonary vascular lesions. Continuous intravenous epoprostenol therapy seems to be less effective as compared with patients with primitive pulmonary hypertension and does not improve survival. In our experience, immunosuppressive therapy does not improve hemodynamic or clinical data. Novel therapies including oral, sub-cutaneous or inhaled stable prostacyclin analogues and endothelin receptor antagonists are currently evaluated in large placebo-controlled trials.
[Cardiovascular involvement in systemic sclerosis].
Constans J, Skopinski S, Barcat D … +1 more , Conri C
Ann Med Interne (Paris) · 2002 Jun · PMID 12218890
Heart involvement is frequent in systemic sclerosis. Cardiomyopathy is the main localization but its diagnosis is often late. Cardiac echography or radionuclide imaging show early involvement of the myocardium while show...
Heart involvement is frequent in systemic sclerosis. Cardiomyopathy is the main localization but its diagnosis is often late. Cardiac echography or radionuclide imaging show early involvement of the myocardium while showing alterations of diastolic function of the left ventricle or perfusion defects. The pathogenesis of this cardiomyopathy is supposed to be related to myocardial ischemia in relation with vasospasm, or with organic lesions of small arteries or coronary microcirculation. Pericarditis rarely is of clinical significance. Pulmonary hypertension concerns patients with proximal and advanced systemic sclerosis or limited forms such as CREST. It can be efficiently diagnosed by doppler echography but its therapy is difficult and its prognosis is poor. Epoprostenol in continuous venous infusion seems to be efficient but the accessibility to this therapy is difficult. While the involvement of middle-sized arteries of the hands is common, systemic sclerosis seems to be associated to an increased frequency of large-sized arteries disease.
[Psychiatric manifestations of systemic sclerosis].
Baubet T, Brunet M, Garcia De La Peña-Lefebvre P … +4 more , Taïeb O, Moro MR, Guillevin L, Mouthon L
Ann Med Interne (Paris) · 2002 Jun · PMID 12218889
Systemic sclerosis (SSc) is a rare conjuctive tissue disorder that is characterized by fibrosis of the skin and internal organs as well as vascular obliteration phenomenons. SSc is responsible for increased morbidity and...
Systemic sclerosis (SSc) is a rare conjuctive tissue disorder that is characterized by fibrosis of the skin and internal organs as well as vascular obliteration phenomenons. SSc is responsible for increased morbidity and mortality, and skin fibrosis may be responsible for major body changes. These phenomenons may contribute to the occurrence of psychological disturbances such as anxiety and depression, whereas the incidence of psychotic symptoms is very low in SSc patients. However, the prevalence of psychiatric symptoms during the course of SSc has not been evaluated yet with standardized evaluation scales, and an important breakthrough can be made in understanding the underlying mechanisms of psychiatric manifestations, in order to improve therapeutic management and quality of life.
[Immune-based therapies and HIV infection].
Weiss L
Ann Med Interne (Paris) · 2002 Jun · PMID 12218888
The aim of immune-based therapies in HIV infection is to enhance numbers and function of CD4 and CD8 T lymphocytes especially specific anti-HIV cellular immune responses in order to allow immune control of viral replicat...
The aim of immune-based therapies in HIV infection is to enhance numbers and function of CD4 and CD8 T lymphocytes especially specific anti-HIV cellular immune responses in order to allow immune control of viral replication. Interleukin-2 (IL-2) has been extensively studied in phase II trials. Intermittent administration of subcutaneous IL-2 results in substantial increases in CD4 cell counts in most HIV-patients. Two large-scale phase III international studies are addressing the key remaining question of the clinical benefit associated with CD4 cell expansions in HIV-infected patients receiving IL-2. Other cytokines including interferon-alpha are currently under investigation in phase II trials. The goal of therapeutic immunizations is to enhance and broaden the immune system's ability to recognize HIV. Several vaccine candidates, also tested as immunogens for preventive vaccines, are currently studied in phase II trials in patients exhibiting viral suppression under highly active antiretroviral therapy. It has been demonstrated in rhesus monkeys'models that therapeutic immunizations can result in the induction of strong CD4 and CD8 responses associated with viral control and prevention of clinical AIDS, following challenge with a highly pathogenic strain of chimeric SIV-HIV. The clinical efficacy of immune-based strategies remains to be demonstrated in randomized controlled clinical trials. Participation of immune system to control of viral replication may allow simplification of antiretroviral treatment which results in long-term adverse effects having impact in life quality of persons living with HIV.
Association between first injection risk behaviors and hepatitis C seropositivity among injecting drug users.
Vidal-Trecan GM, Varescon-Pousson I, Gagnière B … +3 more , Tcherny- Lessenot S, Madariaga E, Boissonnas A
Ann Med Interne (Paris) · 2002 Jun · PMID 12218887
HCV infection is rapidly acquired after drug addicts first inject drug intravenously. The risk behaviors accompanying the first intravenous substance injection are not well known. We used in 1997 a structured questionnai...
HCV infection is rapidly acquired after drug addicts first inject drug intravenously. The risk behaviors accompanying the first intravenous substance injection are not well known. We used in 1997 a structured questionnaire to investigate the relationships between risk behaviors at the first injection and current reported HCV status. We interviewed 151 injecting drug users from four treatment centers and one prison in Paris. Risk markers for reported HCV seroconversion were explored by use of logistic regression models. One hundred and forty-three injecting drug users (95%) agreed to participate in the study. At the first injection, 50% shared preparation equipment; 22% borrowed and 26% lent injecting equipment. At the time of the study, 46% reported that they were HCV-positive. Sharing preparation equipment (odds ratio=3.1; 95% confidence interval: 1.2-7.8) and lending injection equipment (odds ratio=3.0; 95% confidence interval: 1.1-8.5) during the first injection were independently associated with reported HCV seropositivity. The high-risk behaviors accompanying the first intravenous injection of drugs justifies the implementation of specific prevention measures, aimed at young drug users who have not started to inject.
[Masculine anorexia nervosa: realities and perspectives].
Chambry J, Corcos M, Guilbaud O … +1 more , Jeammet P
Ann Med Interne (Paris) · 2002 May · PMID 12218886
Since its description by Morton in 1694, masculine anorexia nervosa has been the subject of much debate. For many, two questions remain unanswered: does anorexia nervosa, as described in girls, exist in boys? - if so, is...
Since its description by Morton in 1694, masculine anorexia nervosa has been the subject of much debate. For many, two questions remain unanswered: does anorexia nervosa, as described in girls, exist in boys? - if so, is it the same disease? We analyzed the data in the literature which demonstrate a lower incidence than in the female population, although estimates are probable low due to underdiagnosis. The behavioral aspects suggest a similarity between masculine and feminine anorexia nervosa although the pure restrictive forms of anorexia are more rare in boys. There are however a few differences. Affected boys, according to Crips and Burns (1990), are heavier than girls at onset of the disorder but present a lower body weight during certain periods of the disease. Excessive physical activity is more frequent as is excessive intellectual involvement (Margo, 1987). The problem of amenorrhea, on/off periods, is not present in the male form. Testosterone and sexual function decline gradually, in parallel with the state of malnutrition (Anersen, 1990). The patient does not have particular difficulty discussing sexual relations but does exhibit a poor level of experience and mental representations. Contact with the opposite sex is rare and the fantastic life is generally very limited. The frequency of homosexual behavior would lie between 25% (Herzog, 1984) and 58% (Schneider and Agras, 1987), which is higher than in the female anorexia population (Herzog, 1984). This observation raises the question concerning the relationship between masculine mental anorexia nervosa and fragile sexual identity.
[Alexithymia and alcohol dependence].
Taieb O, Corcos M, Loas G … +9 more , Speranza M, Guilbaud O, Perez-Diaz F, Halfon O, Lang F, Bizouard P, Venisse JL, Flament M, Jeammet P
Ann Med Interne (Paris) · 2002 May · PMID 12218885
Alexithymia is a term introduced by Sifneos in 1973 to describe, initially in psychosomatic patients, the inability to recognize and to express emotions. Since 1973, alexithymia has been also described in alcohol depende...
Alexithymia is a term introduced by Sifneos in 1973 to describe, initially in psychosomatic patients, the inability to recognize and to express emotions. Since 1973, alexithymia has been also described in alcohol dependence. A review of these studies is proposed with the results of our study from the INSERM network "Dependence". Prevalence of alexithymia is high in alcoholic patients, between 40 and 60%. Some authors have found that alexithymia predicted poor outcome in these patients. The distinction between primary and secondary alexithymia and the relationships between depression and alexithymia are complex. The psychodynamic hypothesis that alexithymia could hold a central position secondary to early dysfunction in the construction of the psychic and somatic self can be put forward. In some circumstances, regression to this position, even after transient addictive filling-in, could lead to essential depression and to psychosomatic disease.
About the article of M. Pierrot et al.: "Intoxications by hallucinogenic mushrooms".
Webster P
Ann Med Interne (Paris) · 2002 May · PMID 12218884
Abstract loading — click title to view on PubMed.
Cardiovascular complications of cocaine use: recent points on cocaethylene toxicity.
Lepere B, Charbit B
Ann Med Interne (Paris) · 2002 May · PMID 12218883
Abstract loading — click title to view on PubMed.
[Is double diagnosis appropriate? A case of schizophrenia and heroine addiction].
Swartebroeckx M
Ann Med Interne (Paris) · 2002 May · PMID 12218882
We present a clinical case of a psychotic drug abuser. The diagnostic hypothesis was paranoid schizophrenia. This case clearly demonstrates the diagnostic duel between schizophrenia and heroine addiction. Based on this c...
We present a clinical case of a psychotic drug abuser. The diagnostic hypothesis was paranoid schizophrenia. This case clearly demonstrates the diagnostic duel between schizophrenia and heroine addiction. Based on this case, we hypothesize that use of drugs in such a patient fulfils an appeasing function rather than pathological burial. It would be interesting to determine the specificity of how this type of patient uses drugs, beyond the apparent double incidence of two diagnoses, in the mode of decompensation and chronic progression of the disease, which would be particularly useful for developing a treatment strategy.
[Behavioral and cognitive therapy to break the smoking habit. Review of the literature].
Le Foll B, Aubin HJ, Lagrue G
Ann Med Interne (Paris) · 2002 May · PMID 12218881
Nicotine addiction is a chronic disease characterized by frequent relapse. Pharmacological and psychological factors are involved and must be specifically addressed in addicts under treatment. Physicians are familiar wit...
Nicotine addiction is a chronic disease characterized by frequent relapse. Pharmacological and psychological factors are involved and must be specifically addressed in addicts under treatment. Physicians are familiar with pharmacological treatment with nicotine replacement therapy and bupropion, but not with psychological approaches such as behavioral and cognitive therapy. Various techniques have been evaluated during smoking cessation trials: aversive therapy, contracts, social support, stimulus control, relaxation, diet and nicotine fading. Such approaches have been completed with cognitive strategies and therapeutic programs often use motivational interviews, skills training and relapse prevention strategies. This article reviews these techniques and presents the results of a recent meta-analysis evaluating their efficacy. These results confirm the efficacy of behavioral and cognitive therapy in smoking cessation.
[Acute complications in cocaine users].
Guerot E, Sanchez O, Diehl JL … +1 more , Fagon JY
Ann Med Interne (Paris) · 2002 May · PMID 12218880
Cocaine use has increased considerably during the last twenty years and several related complications can be identified. Clinical features of cocaine intoxication are variable, but predominantly involve cardiovascular ev...
Cocaine use has increased considerably during the last twenty years and several related complications can be identified. Clinical features of cocaine intoxication are variable, but predominantly involve cardiovascular events. Chest pain is the most main complaint; myocardial ischemia must be ruled out. Other cardiovascular manifestations are left ventricular dysfunction, arrhythmia, endocarditis and aortic dissection. Non-cardiac complications include neurological (seizures, stroke, cerebral hemorrhage), respiratory (asthma, interstitial pneumonitis, pulmonary edema), renal (acute renal failure, rhabdomyolysis) and obstetrical disorders. Detection of cocaine in the urine provides the diagnosis. Symptomatic treatment is generally given, combining conventional treatment of the complication and broad use of benzodiazepines.
Economic and social effects of high-dose buprenorphine substitution therapy. Six-month results.
Lavignasse P, Lowenstein W, Batel P … +7 more , Constant MV, Jourdain JJ, Kopp P, Reynaud-Maurupt C, Riff B, Videau B, Mucchielli A
Ann Med Interne (Paris) · 2002 May · PMID 12218879
The purpose of this study was to analyze the impact of high-dose buprenorphine substitution therapy in opiate-dependent patients in terms of use of psychoactive substances, associated risks, social integration, and the s...
The purpose of this study was to analyze the impact of high-dose buprenorphine substitution therapy in opiate-dependent patients in terms of use of psychoactive substances, associated risks, social integration, and the social cost generated by the use of these substances. This was a longitudinal quantitative survey carried out in 1083 patients who were evaluated at three times: at the beginning of substitution therapy (D0), at 6 months and then at 12 months follow up (M6, M12). Data were collected with an anonymous self-administered questionnaire, completed in the presence of an investigating physician. Results demonstrated that patients treated with high-dose buprenorphine for 6 months, consumed fewer psychoactive drugs (heroin, cocaine, benzodiazepines) and had fewer associated risks. Additionally, several criteria involved in social integration showed improvement; morbidity and mortality decreased after the first 6 months of substitution therapy. These improvements were followed by a reduction in the social cost of drug use generated by the group of patients considered. These initial results require confirmation in the final analysis of the study taking into account the 12-month follow up.
[Frequency of re-incarcerations in the same detention center: role of substitution therapy. A preliminary retrospective analysis].
Levasseur L, Marzo JN, Ross N … +1 more , Blatier C
Ann Med Interne (Paris) · 2002 May · PMID 12218878
A retrospective study was carried out using 3 606 medical files of nine detention centers in France, over a three-month period (May to July 1997). The files were analyzed to determine, age, type of addiction and subseque...
A retrospective study was carried out using 3 606 medical files of nine detention centers in France, over a three-month period (May to July 1997). The files were analyzed to determine, age, type of addiction and subsequent type of therapy proposed: methadone, high-dose buprenorphine or abstinence. A comparison was then made to determine whether or not there exists a statistical relationship between the type of therapy given in prison for drug abuse and subsequent recurrent use during the following three and a half years, until December 2000.
[Drug users attending at hospital emergency rooms. Changes in sociodemographic features, health care, referral attitudes and impact of substitution therapy, between 1993 and 1998].
Espinoza P, Mathy F, Massari V … +2 more , Nuss P, Caron C
Ann Med Interne (Paris) · 2002 May · PMID 12218877
The purpose of this work was to assess changes observed between 1993 and 1998 relative to drug users seen at hospital emergency rooms. We retrospectively reviewed our medical files for two periods, 1993-1995 and 1996-199...
The purpose of this work was to assess changes observed between 1993 and 1998 relative to drug users seen at hospital emergency rooms. We retrospectively reviewed our medical files for two periods, 1993-1995 and 1996-1998, i.e. before and after routine institution of substitution therapy with buprenorphine and methadone. A predefined grid was used to select files. All patients whose file indicated drug use, irrespective of the reason for consultation, were eligible for inclusion. Overall demographic features were: mean age 29 years, men 74%, known address 60%, indication of medical coverage 29%, institutional transportation 70%, heroine abusers 53%, injecting drug users 65%, associated acute alcohol intoxication 27%. There was a significant decline in the incidence of drug abusers during the second period (2.42% versus 3.44%) with no difference for age, sex, or area of residence. Documentation of medical coverage improved. There was no change in the hour of arrival, more than 50% arrived outside regular hospital hours but a higher proportion arrived during the weekend, indicating some access difficulty. Our results are in agreement with the OFDT data on product use: reduction in the use of heroine and significant increase in the use of cocaine, alcohol consumption remaining unchanged. Among the 63 patients seen during the second period, 31.7% stated they were taking substitution therapy, indicating this population has access to treatment. There was no statistical difference for diagnosis, but certain trends were observed: acute intoxication with loss of consciousness leading to emergency squad transfer to the emergency room remained the most common situation, rate of trauma was unchanged at approximately 10%, as was the rate of post-IV abscesses; there was a 3-fold reduction in request for drugs and a 2-fold rise in public intoxication. These data help better define management conditions for drug users attending emergency rooms.
About
- Frequency
- Sun
- Papers found
- 200
- RSS feed
- Subscribe