Search PubMed⌕ Search

Biomedical subjects

J-C Piette

Publications and source records attributed to J-C Piette.

At least 37 records · Page 2Linked to original sources
PubMed2005

[Catastrophic antiphospholipid syndrome after ovulation induction].

INTRODUCTION: The catastrophic antiphospholipid syndrome (CAPS) is a rare expression of the antiphospholipid syndrome (APLS). The all predicting factors of happening of CAPS are not yet discovered. Some women presenting an APLS with infertility have recourse to ovulation induction (OI). EXEGESIS: After an OI, a woman is admitted for acute renal failure, hypertension, seizures, respiratory failure. History and immunologic tests are in favour of APLS. Renal biopsy highlights a thrombotic microangiopathy. Diagnosis of CAPS is made. Patient improves after respiratory assistance, parenteral treatment for hypertension and anticoagulant therapy. CONCLUSION: IO seems to be on of the factors which leads to CAPS. To avoid such consequences, it's essential to know the history and the immunological status of the patients having recourse to this treatment to take care of these risked pregnancies.

Acute Disease↗
PubMed2005

[Pleading to maintain hydroxychloroquine throughout Lupus pregnancies].

PURPOSE: The use of Hydroxychloroquine (HCQ) during pregnancy has remained controversial for a long time. However, it is generally agreed that pregnancy per se increases disease activity in patients with systemic lupus erythematosus (SLE) and that withdrawal of HCQ at the onset of pregnancy may result in exacerbation of SLE. Therefore, stopping HCQ at the onset of pregnancy may result in exacerbation of SLE which could be detrimental to both mother and fetus. CURRENT KNOWLEDGE AND KEY POINTS: The available data suggest that HCQ can be continued safely throughout pregnancy. After the first report by Parke of successful continuation of HCQ throughout gestation, more than 250 pregnancies resulting in live births have been reported and no increase in the rate of birth defects have been demonstrated. When studied, no retinal toxicity and ototoxicity have been found in the children. Data concerning lactation and HCQ treatment are rare. However, the amount of HCQ received by children through lactation seems very low. FUTURE PROSPECTS AND PROJECTS: For patients with SLE already taking HCQ, the benefits of continuing treatment with this medication throughout pregnancy seem to outweigh the hypothetical risks associated with its use. HCQ should probably be maintained throughout pregnancy in these patients with SLE and it does not seem necessary to advise against breastfeeding. Further studies with prospective follow-up of children exposed in utero to HCQ remain however needed to provide a definitive answer.

Antirheumatic Agents↗
PubMed2005

Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome.

OBJECTIVE: To describe the characteristics of patients with catastrophic antiphospholipid syndrome (APS) included in the International Registry of patients with this condition (CAPS registry) and to analyse the value of the recently proposed preliminary criteria for the classification of catastrophic APS. METHODS: A review of the first 220 patients included in the website based CAPS registry was undertaken and the preliminary criteria for their classification were tested; 175 unselected patients with systemic lupus erythematosus or APS, or both, acted as controls. RESULTS: The mean age of the patients was 38 (14) years (range 7 to 74), with a female preponderance (F/M, 153/67). The main clinical manifestations included renal involvement in 154 (70%), pulmonary in 146 (66%), cerebral in 133 (60%), cardiac in 115 (52%), and cutaneous in 104 (47%); 114 patients (52%) recovered after the catastrophic APS event (mortality 48%). Patients who received the combination of anticoagulation plus steroids plus plasma exchange or intravenous immunoglobulins had the best survival rate (63%, p = 0.09). Sufficient data could be analysed for application of the classification criteria in 176 patients. According to the preliminary criteria, 89 patients (51%) could be classified as having "definite" and 70 (40%) as having "probable" catastrophic APS, thus given a sensitivity of 90.3% with a specificity of 99.4%. Positive and negative predictive values were 99.4% and 91.1%, respectively. CONCLUSIONS: The preliminary criteria for the classification of catastrophic APS and the CAPS registry are useful tools for epidemiological studies.

Adolescent↗
PubMed2005

[Severe visual loss due to retinal artery occlusion associated with Susac's syndrome: a case report].

INTRODUCTION: Susac's syndrome consists of the clinical triad of cerebral microangiopathy, branch retinal artery occlusions, and hearing loss. The pathogenesis of the disease remains unknown. The severity of retinal vasculopathy remains variable. CASE REPORT: A 57-year-old Caucasian woman presented in 1999 for the diagnostic and therapeutic management of a severe and complete form of the disease. Fluorescein angiography and cerebral MRI were contributive in this case and confirmed the diagnosis. A severe unilateral visual loss occurred one month after the initiation of systemic corticosteroids. Funduscopy disclosed a temporal retinal artery occlusion with major ischemia. Therefore, cyclophosphamide and anticoagulants were added to corticosteroids and total visual recovery was achieved within 2 weeks without any relapse after a follow-up period of 54 months. CONCLUSION: Therapeutic management of Susac's syndrome is still controversial. Major immunosuppressive regimens are mandatory in the face of severe visual loss associated with central or branch retinal artery occlusions. The final prognosis of the disease seems favorable in the absence of prolonged and symptomatic retinal ischemia.

Blindness↗
PubMed2005

[Diagnostic and therapeutic management of Vogt-Koyanagi-Harada syndrome].

PURPOSE: To determine the most efficient diagnostic tools in Vogt-Koyanagi-Harada syndrome, taking into account the international diagnostic criteria, and to evaluate the therapeutic management of these patients. PATIENTS AND METHODS: This study examined patients with a suspicion of VKH syndrome who presented between January 2001 and March 2003, including ocular and extraocular evaluation of the disease at the time of diagnosis. Each patient was classified according to the 1978 international diagnostic criteria and the revised 2001 criteria. In most cases, intravenous steroid pulses were administered. Immunosuppressors were initiated when inflammation was not controlled with steroids. RESULTS: Twenty-two patients were included. The mean age was 33.5 years (range, 15-49 years). Posterior segment involvement, which was observed in 21 patients, depended on the stage of the disease. Anterior segment inflammation was associated in eleven cases. Neurologic symptoms, including meningitis, cerebrospinal fluid lymphocytic pleocytosis, tinnitus, or hearing loss were observed in 12 patients. Fourteen patients had dermatologic signs. Five patients who developed VKH syndrome did not meet the 1978 criteria, and three patients did not meet the 2001 revised criteria. In 19 cases, intraocular inflammation was controlled with corticosteroids. In three cases, corticosteroids could not be discontinued. These patients were treated with immunosuppressive molecules: azathioprine, cyclophosphamide, interferon alpha. At the end of the follow-up period, inflammation was controlled in all patients. DISCUSSION: Revision of the diagnostic criteria provides a more subtle diagnosis of VKH syndrome. However, it is difficult to consider the variability of clinical symptoms during the duration of disease. Corticosteroids must be used at appropriate dosages, followed by slow tapering over 6 months. This attitude seems to reduce the duration of ocular inflammation and decreases the frequency of recurrence. The use of immunomodulating drugs could be reduced by early and appropriate use of systemic steroids. Interferon alpha seems to be a promising alternative in corticoresistant or corticodependent forms of the disease, but further controlled studies are required.

Adolescent↗
PubMed2005

Genetic heterogeneity of the hypervariable region I of Hepatitis C virus and lymphoproliferative disorders.

B-cell lymphoproliferative disorders (BCLD) have been associated with chronic hepatitis C virus (HCV) infection. The HCV glycoprotein E2 (gpE2) hypervariable region I (HVR-I) may be a potential antigenic candidate to promote B-cell proliferation. The purpose of this study was to analyze the influence of HVR-I sequence variability in the development of BCLD. HVR-I sequences were studied in 29 chronically HCV-infected patients with (n=15) or without (n=14) BCLD. After PCR amplification of the gpE2 region, analysis of the 81 bp HVR-I encoding fragment was performed on 7-18 clones per patient. HVR-I sequence complexity was slightly lower in patients with BCLD (mean 0.347) than without (0.468) (P=0.2), though, sequence diversities were similar (0.0370 vs 0.0954, P=0.239). Phylogenetic analysis did not reveal any BCLD-associated clustering. In our population, neither the recently described insertion between positions 1 and 2 of HVR-I nor residues at positions 4 and 13 were particularly linked to BCLD. As previously described, we confirm the high degree of conservation of HVR-I residues T-2, G-6 and G-23 in our patients. Contrary to recent findings, our analysis based on multiple clones per patient analysis did not reveal any particular motif associated with BCLD.

Adult↗
PubMed2005

The HELLP syndrome in the antiphospholipid syndrome: retrospective study of 16 cases in 15 women.

OBJECTIVE: To study the characteristics of the haemolysis, elevated liver enzymes, low platelets (HELLP) syndrome in the antiphospholipid syndrome (APS) and its influence on the subsequent pregnancies. METHODS: This was a retrospective analysis of 16 episodes of HELLP complicating APS in 15 women. RESULTS: HELLP was complete in 10 cases and partial in six. It occurred during the second trimester in seven cases (the earliest at 18 weeks' gestation), the third trimester in seven cases, and the day following delivery in two cases. Pre-eclampsia was present in six cases and eclampsia in five. Outcome of pregnancies was: live birth (n = 8), stillbirth (n = 2) and fetal death (n = 6). APS was primary in nine women and secondary to systemic lupus erythematosus (SLE) in six. HELLP revealed primary APS in six cases. Seven women were not treated. Low dose aspirin was empirically prescribed in one woman whose APS had been undiagnosed despite a history of two fetal deaths. In the other women, therapy consisted of aspirin (n = 8), low molecular weight heparin with a dose varying between 3000 and 12 000 U daily (n = 5), and high dose immunoglobulin every 4 weeks (n = 2), hydroxychloroquine (n = 4), and prednisone (n = 6). Six women had seven subsequent pregnancies, 3-6 years after the complicated pregnancy. HELLP recurred at 33 weeks' gestation in one woman with SLE treated with prednisone, hydroxychloroquine, aspirin, and enoxaparin, and pregnancy ended in live birth. One woman became pregnant after in vitro fertilisation and embryo transfer, but pregnancy ended in fetal death despite prednisone, hydroxychloroquine, and enoxaparin. Four women had five uneventful pregnancies with 100 mg daily aspirin and heparin. CONCLUSIONS: APS may be revealed by HELLP. In APS, HELLP is associated with pre-eclampsia/eclampsia in most cases and seems to occur earlier than in the general population. Heparin plus aspirin may prevent obstetric complications in the subsequent pregnancies.

Adult↗
PubMed2005

Familial inflammatory inclusion body myositis.

OBJECTIVE: To compare familial inflammatory inclusion body myositis (IBM) with hereditary inclusion body myopathies and sporadic IBM. PATIENTS AND METHODS: Clinical, biological, MRI, and histological data were analysed in two siblings with inflammatory IBM and compared with those of patients with sporadic and hereditary IBM. RESULTS: Both patients had a clinical phenotype of sporadic IBM, which differs from hereditary myopathies because of late age of onset--respectively 65 and 66 years, and different pattern of muscular involvement--asymmetric, mainly distal but also involving quadriceps. MRI showed selective fatty infiltration and oedema in the extensor compartment of thigh muscles. The diagnosis of IBM was confirmed by muscle biopsy, showing muscle fibres containing numerous rimmed vacuoles, a characteristic shared by all types of IBM. In contrast with hereditary IBM, histological analysis also showed inflammatory mononuclear infiltrate invading non-necrotic fibres, ragged red and oxidase c negative fibres, and positive Congo red staining. Moreover, HLA class II typing disclosed DR beta 1 0301 haplotype, which is significantly related to sporadic but not to hereditary IBM. With steroid treatment and monthly intravenous immunoglobulins, the disease was stabilised in both patients at protracted follow up. CONCLUSION: Sporadic and familial inflammatory IBM share the same clinical, biological, MRI, and histological features.

Aged↗
PubMed2004

[Scurvy].

Explore the source record for details and available documents.

Adult↗
PubMed2003

[Chronic severe uveitis: classification, search for etiology and therapeutic approach].

PURPOSE: Severe chronic and refractory uveitis is a major diagnostic and therapeutic challenge for ophthalmologists and internists. Molecular tools, such as PCR but also new imaging techniques, have significantly changed the diagnostic approach during the last 10 years. Presumed and empirical diagnosis should be excluded in the face of atypical clinical presentations. CURRENT KNOWLEDGE AND KEY POINTS: A retrospective study based on 927 consecutive patients presenting with severe uveitis between 1991-1996, has recently defined the epidemiological characteristics and the visual outcome of this group of patients. An associated condition was determined in 67.5% of cases, divided in 4 different subgroups: infectious uveitis; uveitis associated with a systemic disease; eye-limited, presumed immune-mediated disorder and idiopathic eye-limited disorder. The management of patients with sight-threatening forms of uveitis is efficiently performed in collaboration with internists and depends on a complete diagnostic procedure and a well-adapted treatment. FUTURE PROSPECTS AND PROJECTS: Extensive work-up is mandatory when the therapeutic response seems atypical with resistance to corticosteroids and classical immunosuppressive drugs. Infectious uveitis should be excluded in severe and intractable forms of uveitis. Thereafter, new therapeutic strategies based on type I interferon and anti-TNF molecules can be proposed in order to decrease the potential risk of blindness in this young group of patients.

Chronic Disease↗
PubMed2003

[Bilateral internal carotid thrombosis: search for strangulation, even very ancient].

INTRODUCTION: We report 2 cases of bilateral carotid thrombosis secondary to repeated attempts of strangulation. EXEGESIS: The first case was discovered in a 31-years-old woman who complained of psychiatric manifestations, memory loss and aphasia. Subsequently she developed hemiplegia which worsened under low dose aspirin. The second case occurred in a 41-years-old woman who presented with sudden hemiplegia and aphasia. Diagnosis of bilateral carotid thrombosis based on angiocomputerized tomography or angioRMI data which suggested dissection. Thrombosis location was similar: bilateral, symmetric, close to the carotid bifurcation. Both women had atherosclerosis risk factors: current smoking, use of estrogen containing pill, dyslipidemia and/or familial cardiovascular history. Search for thrombophilia was negative. Both women had been victims of intimate partner violence with repeated attempts of strangulation, but they were stopped 8 and 6 years before the diagnosis of carotid thrombosis. Several private interviews were necessary before knowing the exact cause. CONCLUSION: Symmetrical bilateral thrombosis of internal carotids near the bifurcation should lead to evoke repeated attempts of strangulation, even if there are arterial risk factors, and especially if the patient is a middle-aged female. The therapy is not codified but prolonged anticoagulation is generally prescribed.

Adult↗