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D Launay

Publications and source records attributed to D Launay.

36 records · Page 2Linked to original sources
PubMed2002

[Unusual association: hepatic peliosis and Crohn's disease].

INTRODUCTION: Hepatobiliary disorders occurs in about 10 to 30% of inflammatory bowel diseases. Fatty liver and gallstones are preferentially described with Crohn's disease. Conversely, peliosis hepatis has been only exceptionally reported in association with inflammatory bowel diseases. We describe here a case of peliosis hepatis complicating a misdiagnosed Crohn's disease. EXEGESIS: A 24-year-old woman was referred because of a hepatomegaly and a deterioration of the general state. The patient complained about diarrhea for 3 months and about an episode of bloody stools. There was an aphthous-like lesion on the tongue. X-ray pull-through revealed a typical aspect of terminal ileitis as seen in Crohn's disease. The liver was heterogeneous. Liver biopsy revealed a peliosis hepatis. No other possible cause of peliosis hepatis was found. Therefore, in this case, peliosis hepatis, revealed by the hepatomegaly, was associated with Crohn's disease. CONCLUSION: Association between Crohn's disease and peliosis hepatis has only exceptionally been described. A priori, this complication is not a pejorative feature in inflammatory bowel disease. The pathophysiological significance of this association remains unknown.

Adult↗
PubMed2002

[Systemic sclerosis and pregnancy].

PURPOSE: Pregnancy in a patient with systemic sclerosis (SSc) may pose a double problem to the medical team: influence of SSc on pregnancy and consequences of pregnancy to SSc manifestations. CURRENT KNOWLEDGE AND KEY POINTS: Concepts have evolved. SSc was considered for a long time not only as not very propitious for pregnancy but also as a strict contraindication for procreation because risks for the mother and the baby were thought to be major. Currently, fertility is thought to be normal. Miscarriages and small-for-gestation age infants rate do not seem to be higher in SSc. Maternal and perinatal mortality is also not higher in SSc without severe visceral manifestations, i.e. without either pulmonary hypertension, or cardiac or respiratory insufficiency. Conversely, there is a significantly higher frequency of premature infants in SSc. As regards influence of pregnancy on SSc, the greatest fear is the occurrence of renal crisis, which may be life threatening for both mother and child. Each elevation of blood pressure, even if this increase is mild, should be considered as potentially very serious. However, pregnancy itself does not seem to increase the risk of renal crisis. Consequences of pregnancy to SSc manifestations are various but usually mild. FUTURE PROSPECTS AND PROJECTS: SSc is not a strict contraindication for pregnancy only if severe organ involvement, diffuse subset of SSc or recent onset of the disease has been ruled out. Physicians should be aware of specific problems, which SSc is possibly posing during pregnancy. Finally, it has been recently suggested that pregnancies could be involved in the pathogenesis of SSc through persisting microchimerism of fetal origin.

Acute Disease↗
PubMed2001

[DRESS (Drug Rash with Eosinophilia and Systemic Symptoms) syndrome after sulfasalazine and carmazepine: report of two cases].

INTRODUCTION: To better individualize drug hypersensitivity reaction, Bocquet et al. have recently called this adverse drug reaction DRESS (Drug Rash with Eosinophilia and Systemic Symptoms). EXEGESIS: We report two cases of DRESS and highlight the main characteristics of this syndrome. Two patients presented severe febrile skin eruption following drug intake (carbamazepine or sulfazalazine), with hypereosinophilia and hepatitis. All symptoms resolved after drug withdrawal and corticosteroid therapy. DRESS syndrome is an idiosyncratic reaction characterised by febrile eruption, occurring 2 to 6 weeks after the beginning of the treatment, accompanied by systemic symptoms and biological abnormalities (hypereosinophilia, hepatitis). Some complications can occur. This syndrome can be fatal. Numerous drugs can be responsible for this reaction to medication. The physiopathology has not yet been elucidated, and the treatment is not codified, but the triggering agent must immediately be stopped. Corticotherapy is sometimes used. CONCLUSION: It is important to recognize this entity recently named DRESS syndrome because it can mimic other pathologies, is potentially serious, and because withdrawal of the incriminating drug is imperative.

Adolescent↗
PubMed2001

[Pulmonary hypertension screening in systemic scleroderma: a cohort study of 67 patients].

PURPOSE: Pulmonary hypertension is a severe complication of systemic sclerosis and has emerged as a major cause of morbidity and mortality in this condition. Treatment is all the more efficient as pulmonary hypertension is early diagnosed. A good knowledge of the clinical, biological and functional features of pulmonary hypertension in systemic sclerosis is therefore necessary to suspect and to diagnose pulmonary hypertension as early as possible. METHODS: Sixty seven patients with systemic sclerosis were retrospectively studied. We compared clinical, immunological, functional (spirometry) and morphological (pulmonary fibrosis) features according to the presence (n = 25) and the characteristic of pulmonary hypertension (isolated or secondary) or the absence (n = 42) of pulmonary hypertension, assessed by Doppler echocardiography. RESULTS: CREST syndrome (calcinosis, Raynaud's phenomenon, oesophageal involvement, sclerodactyly and telangiectasia) was more frequent in patients with isolated pulmonary hypertension than in patients without PH (72.7% vs 28.5%, P < 0.05; odds-ratio [OR] = 6.6) and dyspnea was more severe (P < 0.001; OR = 11.4). The age at time of pulmonary hypertension diagnosis was higher in patients with secondary pulmonary hypertension than in patients with isolated from (median: 62.5 years (range: 32-35) vs 53 years (range: 37-85), P < 0.05). Patients with isolated pulmonary hypertension had anticardiolipin antibodies more frequently than patients without pulmonary hypertension (72.7% vs 35.7%, P < 0.05). Isolated reduction of diffusing capacity was preferentially observed among patients with isolated pulmonary hypertension than among those without pulmonary hypertension. A linear relation between systolic pulmonary artery pressure values and diffusing capacity values (r = 0.72, P < 0.01) was found. Isolated reduction of diffusing capacity was more frequent in patients with isolated pulmonary hypertension than in patients without pulmonary hypertension (63.6% vs 14.3%, P < 0.001; OR = 10.5). CONCLUSION: The severity of pulmonary hypertension in systemic sclerosis justifies a systematic screening by Doppler echocardiography and diffusing capacity measurement. Our results allow us to better define the characteristics of sclerodermic patients with isolated or secondary pulmonary hypertension. The search for pulmonary hypertension should be repeated with time and clinicians should be particularly vigilant in the case of a patient presenting these characteristics.

Adult↗
PubMed2001

Scleromyxedema (lichen myxedematosus) associated with dermatomyositis.

A 41-year-old white man is described with papules of the lower and upper back, the neck and the upper chest, a marked deposition of mucin in the upper reticular dermis, and an IgG lambda monoclonal gammopathy strongly evocative of scleromyxedema (lichen myxedematosus). Additionally, he developed intense myalgia, muscle weakness and rhabdomyolysis, which were associated with heliotrope erythema, photosensitivity and an erythematous rash of the dorsum of the hands with Gottron's papules. Muscle biopsy revealed an inflammatory myositis, and dermatomyositis was diagnosed. The association of dermatomyositis and secondary mucinosis, or muscle involvement in primary papular mucinosis are not rare. However, the association between scleromyxedema and dermatomyositis has only exceptionally been reported.

Adult↗
PubMed2001

Relationship between parity and clinical and biological features in patients with systemic sclerosis.

OBJECTIVE: To assess the influence of parity on the clinical and biological features of systemic sclerosis (SSc). METHODS: We recorded the following clinical and biological data of 100 consecutive women with SSc: age, disease duration before diagnosis, cutaneous extension of sclerosis according to LeRoy's classification, pulmonary involvement, and antinuclear antibodies. We compared these features to the number and sex of children who were born before SSc onset. Date of birth of the first children was systematically recorded. RESULTS: Patients with limited SSc had more children before SSc onset than patients with diffuse SSc (2.4 +/- 1.8 vs 1.7 +/- 1.5; p < 0.05). The interval between first birth and SSc onset was shorter for patients with limited SSc than for patients with diffuse SSc (11.0 +/- 9.9 vs 23.5 +/- 14.5 yrs; p < 0.01). Patients with pulmonary fibrosis had more children than patients without pulmonary fibrosis (2.5 +/- 1.9 vs 2.0 +/- 1.6; p < 0.05). Age at first birth was significantly higher when the child was a girl than a boy (26.8 +/- 7.5 vs 22.9 +/- 5.3 yrs; p < 0.05). The interval between the first birth and SSc onset was shorter when the child was a girl than a boy (16.2 +/- 9.6 vs 25.4 +/- 13.4 yrs; p < 0.05). CONCLUSION: Pregnancy related microchimerism could be preferentially associated with limited SSc and pulmonary fibrosis. Microchimerism may be facilitated in cases in which the fetus is female.

Adult↗
PubMed2001

Aerosolized iloprost in CREST syndrome related pulmonary hypertension.

OBJECTIVE: To assess the outcome of patients with CREST syndrome associated severe pulmonary hypertension treated by aerosolized iloprost in a noncomparative study. METHODS: Five patients with CREST syndrome associated severe pulmonary hypertension were treated with 100 microg/day of aerosolized iloprost. New York Heart Association functional class and exercise tolerance (6 min walk test) were assessed at baseline, after one month, and then every 6 months. A right heart catheterization was performed at baseline in all but one patient. Systolic pulmonary artery pressure (PAP) was measured with Doppler echocardiography after one month and every 6 months. RESULTS: The mean followup was 13.2 +/- 8.8 months (median 6, range 6-24). Subjective quality of life improved in all patients. NYHA functional class decreased from Class III to II in 3 patients, from Class III to I in one patient, and from Class IV to III in one patient. At 6 months, the distance walked in 6 min had increased from 352 +/- 48 to 437 +/- 56 m (p = 0.06). At one month the mean systolic PAP was 58 +/- 13 vs 81 +/- 9 mm Hg at baseline (p = 0.04). At 6 months the mean systolic PAP was 57 +/- 13 mm Hg (p = 0.06). The improvement of both clinical and hemodynamic status was maintained in the 2 patients treated for 2 years. Neither adverse effects nor need to increase the daily dose of iloprost were observed. One patient died of right heart failure and one patient did not experience any improvement of exercise tolerance and hemodynamics. CONCLUSION: Aerosolized iloprost might be potentially useful as treatment for CREST syndrome associated pulmonary hypertension. However, patients who could benefit from this treatment will probably have to undergo careful criteria selection.

Administration, Inhalation↗
PubMed2001

[Hemorrhagic fever with renal syndrome].

Puumala hantavirus is the most common hantavirus infection in Western Europe. The causative agent, Puumala virus, is a member of the Hantavirus genus in the Bunyaviridae family. The natural hosts of hantaviruses are chronically, but asymptomatic infected rodents, which transmit the virus to human in their excretions. Puumala virus is carried by the bank vole, clethrionomys glareolus. Hemorrhagic fever with renal syndrome (HFRS) caused by Puumala virus in France or Belgium is very similar to the previously described Nephropathia epidemica in Scandinavia. In most severe cases, the disease is clinically characterized by high fever of abrupt onset, headache, loin or abdominal pains, nausea and vomiting, and occasionally acute and transient myopia. Renal involvement results in transient proteinuria and hematuria and acute renal failure. Except for interstitial hemorrhage in the outer medulla, the renal histopathologic findings are unspecific and include prominent changes in the interstitium with interstitial oedema and inflammatory infiltrates. Thrombocytopenia, mild elevation of liver enzymes, and leukocytosis are typical laboratory findings. Spontaneous complete recovery is the rule. Laboratory diagnosis is primarily based on serology such as indirect immunofluorescence or capture enzyme--linked immunosorbent assays which detect IgM antibodies and an increased level of IgG antibodies against Puumala virus. Viral antigen may be demonstrated in the cytoplasm of renal tubular epithelial cells.

Acute Kidney Injury↗
PubMed2000

[Digital necrosis in a patient with anorexia nervosa. Association of vasculopathy and radial artery injury].

BACKGROUND: Patients with anorexia nervosa can develop distal vasculopathy sometimes leading to severe Raynaud's phenomenon or acrocyanosis. We report a cas of anorexia nervosa-related vasculopathy associated with iatrogenic injury to the radial artery that led to digital necrosis. CASE REPORT: An 18-year-old woman, with a history of severe anorexia nervosa of 5 years duration and who acknowledged regular use of tobacco and cannabis, was hospitalized for necrosis of the left index and thumb that had occurred shortly after left radial artery puncture for blood gas analysis. Acrocyanosis of the 4 limbs had been present since the onset of anorexia nervosa. Arteriography of the upper limbs showed major spasm of the left radial and cubital arteries and thromboses in the left interdigital arteries of the left index and thumb. The distal portions of the arteries were then on the left and on the right. The nectrotic lesions healed after intravenous administration of ilomedine and interruption of tobacco and cannabis. Acrocyanosis of the four limbs persisted. DISCUSSION: We report a case of digital necrosis occurring after arterial puncture for blood gas analysis in a patient with a vascular bed weakened by tobacco and cannabis intoxication but also by anorexia nervosa-related vasculopathy. This observation underlines the potentially dangerous nature of invasive intravascular procedures in this context. Indications for vessel puncture must be assessed with prudence.

Adolescent↗
PubMed1999

[Influence of age on the clinical and biological characteristics of systemic scleroderma].

PURPOSE: The present study was aimed at assessing the influence of age on clinical and biological features of systemic sclerosis. METHODS: This retrospective study included 151 consecutive patients with systemic sclerosis. The median age at diagnosis was 50.0 years (range: 10-84 years). Patients were divided into two groups according to their age (lower than 50.0 years of age: 73 patients, equal to or above 50 years of age: 78 patients). The following features were compared between the two groups: gender, disease duration, extent of skin sclerosis, Crest syndrome, lung fibrosis, secondary Sjögren's syndrome, antinuclear, anticentromere, and anti-Scl70 antibodies. RESULTS: The disease duration was significantly higher in patients over 50 years of age (7.1 +/- 6.8 years vs 5.5 +/- 5.0 years, P < 0.05). Crest syndrome, secondary Sjögren's syndrome and anticentromere antibodies were significantly more common in patients over 50 years of age (17/73 vs 30/78, P < 10(-2); 9/73 vs 20/78, P < 10(-2), and 19/73 vs 31/78, P < 0.05; respectively). Anti-Scl70 antibodies were significantly more common in patients under 50 years of age (17/73 vs 10/78, P < 10(-2)). No significant difference was found in regard to the other features. CONCLUSION: The clinical and biological patterns of systemic sclerosis are different according to the age at disease onset. Crest syndrome including anticentromere antibodies and Sjögren's syndrome is more common in elderly patients, while anti- Scl-70 antibodies are more common in younger patients. This suggests the involvement of various mechanisms in the pathogenesis of systemic sclerosis, and that these mechanisms may depend on the age.

Adolescent↗
PubMed1998

[Primary biliary cirrhosis and systemic scleroderma (Reynolds syndrome): apropos of 8 new cases. The contribution of accessory salivary gland biopsy].

PURPOSE: We report eight new cases of the association primary biliary cirrhosis--systemic sclerosis (Reynolds' syndrome) and study the contribution of labial salivary gland biopsy to the disease diagnosis. METHODS: We retrospectively collected clinical and biological data as well as results of labial salivary gland biopsies in eight patients with Reynolds' syndrome. RESULTS: The eight patients were female. Systemic sclerosis corresponded to a CREST syndrome in three patients, while in two other patients two features of the CREST syndrome were observed. Anticentromere antibodies were detected in these five cases. The last three patients had systemic sclerosis with bilateral pulmonary fibrosis in two cases. All patients presented with Sjögren's syndrome. Labial salivary gland biopsies performed in six patients showed in five cases an inflammatory infiltrate and focal sialadenitis typically associated with Sjögren's syndrome. In four cases, organic microangiopathy and fibrosis were suggestive of systemic sclerosis, and in four other cases, the presence of a pericanalicular infiltrate of lymphocytes and necrosis of the excretory ducts epithelial cells suggested the existence of primary biliary cirrhosis. In three patients, all histological lesions were found in the same labial salivary gland biopsy. CONCLUSION: Coexistence of histological lesions on the same labial salivary gland suggestive of primary biliary cirrhosis, systemic sclerosis and Sjögren's syndrome has never been described previously. The diagnostic value of labial salivary gland biopsy has to be assessed in this context.

Adult↗
PubMed1998

[Bilateral spontaneous perirenal hematoma: an unusual complication of polyarteritis nodosa].

INTRODUCTION: Spontaneous perirenal hematoma is a serious and unusual complication of polyarteritis nodosa. Its treatment is not standardized. A review of the literature shows that therapeutic arterial embolization is an alternative to nephrectomy. EXEGESIS: We report a new case of spontaneous bilateral perirenal hematoma due to a rupture of a renal aneurysm in a 68-year-old patient presenting with polyarteritis nodosa. The patient had no hypertension. Renal arteriography helped guide the diagnosis, and selective embolization was performed to treat the left hematoma. The right hematoma only required symptomatic treatment. CONCLUSION: Arteriography with selective embolization appears to be a good alternative to nephrectomy in spontaneous perirenal hematoma complicating polyarteritis nodosa. However, in this case, the right hematoma required neither arteriography nor nephrectomy. This raises the questions of the necessity of selective embolization when the clinical status remains satisfying with a symptomatic treatment.

Aged↗
PubMed1990

Three-dimensional electron microscopy of entire cells.

The digital processing of serial electron-microscope sections containing laser-induced topographical references allows a three-dimensional (3-D) reconstruction of entire cells at a depth resolution of 40-60 nm by the use of novel image analysis methods. The images are directly processed by a video-camera placed under the electron microscope in TEM mode or by the electron counting device in STEM mode. The deformations associated with the cutting of embedded cells are back-calculated by new computer algorithms developed for image analysis and treatment. They correct the artefacts caused by serial sectioning and automatically reconstruct the third dimension of the cells. Used in such a way, our data provide definitive information on the 3-D architecture of cells. This computer-assisted 3-D analysis represents a new tool for the documentation and analysis of cell ultrastructure and for morphometric studies. Furthermore, it is now possible for the observer to view the contents of the reconstructed tissue volume in a variety of different ways using computer-aided display techniques.

Cell Line↗