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Biomedical subjects

O Fain

Publications and source records attributed to O Fain.

At least 19 recordsLinked to original sources
PubMed2005

[Preeclampsia and antiphospholipid syndrome. Uterine artery Doppler evaluation].

INTRODUCTION: Women with persistently high resistance in uterine arteries have an increased risk of the subsequent development of preeclampsia. Doppler investigation provides a non-invasive method for the study of the uteroplacental blood flow. In pregnant women the antiphospholipid syndrome is associated to an increased risk of preeclampsia and complications related to uteroplacental insufficiency, and the role of uterine artery Doppler is discussed. CURRENT KNOWLEDGE AND KEY POINTS: In normal pregnancy, impedance to flow in the uterine arteries decreases with gestation, as the likely consequence of the physiologic change of spiral arteries into low-resistance vessels. In women with preeclampsia or related complications, the abnormal persistence of high resistance to flow in the uterine arteries correlates with maternal and neonatal outcome. In one study including patients with antiphospholipid syndrome, a high resistance index in the uterine arteries at 22-24 weeks gestation strongly predicted the subsequent development of preeclampsia. In another study including patients with lupus anticoagulant, persistent bilateral notches at 22-24 weeks gestation may identify preeclampsia and fetal growth restriction with a high sensitivity, specificity, positive and negative predictive value. The treatment may improve the uteroplacental blood flow and is a possible confounding factor which needs further evaluation. FUTURE PROSPECTS AND PROJECTS; In patients with antiphospholipid antibodies a higher impedance has been observed in the uterine artery, suggesting a possible vascular dysfunction precluding to impaired trophoblastic invasion and placental thrombosis, as probable mechanisms in the complications. If confirmed, these findings might have important implications for the management of these patients.

Adult↗
PubMed2005

[Systemic mastocytosis].

Systemic mastocytosis is characterized by abnormal mast cell proliferation in different organs. The 2001 consensus classification distinguishes in separate categories indolent systemic mastocytosis, systemic mastocytosis with concomitant blood disease, aggressive systemic mastocytosis and mast cell leukemia. Clinical manifestations are caused by tissue infiltration by proliferating mastocytes and by release of mediators. The principal organs affected are the skin, bones, digestive tract, liver, spleen and lymph nodes. Diagnosis of mastocytosis is based on appropriate stains (Giemsa, toluidine blue) and immunophenotype features (tryptase, CD117, also known as c-KIT and stem cell factor receptor). Serum tryptase levels reflect the total mast cell burden. Treatment must prevent release of mast cell mediators (histamine antagonists, cromolyn sodium, corticosteroids, or leukotriene-receptor inhibitors), limit bone involvement (bisphosphonates) and reduce the number of circulating mast cells (interferon, cladribine, or tyrosine kinase inhibitors). Enhanced understanding of the pathogenic mechanisms (mutation of c-kit and platelet-derived growth factor receptor alpha has led to the development of targeted treatments, including new inhibitors of tyrosine kinase and of nuclear factor Kappa B.

Benzamides↗
PubMed2004

Tolerance and short term efficacy of rituximab in 43 patients with systemic autoimmune diseases.

OBJECTIVE: To assess the tolerance and efficacy of rituximab in patients with various autoimmune diseases seen in daily rheumatological practice. METHODS: 866 rheumatology and internal medicine practitioners were contacted by e-mail to obtain the files of patients treated with rituximab for systemic autoimmune diseases. Patients with lymphoma were analysed if the evolution of the autoimmune disease could be evaluated. RESULTS: In all, 43 of 49 cases could be analysed, including 14 with rheumatoid arthritis (RA), 13 with systemic lupus erythematosus (SLE), six with primary Sjogren's syndrome (pSS), five with systemic vasculitis, and five with other autoimmune diseases. Rituximab was prescribed for lymphoma in two patients with RA and two with pSS. In the 39 other cases, rituximab was given because of the refractory character of the autoimmune disease. The mean follow up period was 8.3 months (range 2 to 26). There were 11 adverse events in 10 patients and treatment had to be discontinued in six. Efficacy was observed in 30 patients (70%): RA 11, SLE 9, pSS 5, vasculitis 2, antisynthetase syndromes 2, sarcoidosis 1. The mean decrease in corticosteroid intake was 9.5 mg/d (range 0 to 50) in responders. Seven patients experienced relapse after mean 8.1 months (5 to 15). Three patients died because of refractory autoimmune disease. CONCLUSIONS: Despite absence of marketing authorisation, rituximab is used to treat various refractory autoimmune diseases in daily rheumatological practice. This study showed good tolerance and short term clinical efficacy, with marked corticosteroid reduction in patients with SLE, pSS, vasculitis, and polymyositis.

Adult↗
PubMed2004

[Micronutrients status of Ile-de-France inhabitants. A literature review].

PURPOSE: A review of the literature about micronutrients status of Ile-de-France inhabitants. CURRENT KNOWLEDGE AND KEY POINTS: During the last decades, substantial changes in the French diet had an impact on micronutrients intake. Over this time, several studies conducted in Ile-de-France showed that a large part of its inhabitants is subject to an increased risk of mild to severe deficiency in one or more micronutrient(s). Teenagers and subjects over 70 years-old are groups at risk for vitamin D deficiency. During the 1980s, iron intake was insufficient in a majority of menstruating women and iron deficiency was common during pregnancy. Vitamin C deficiency is constantly present in homeless people and often associated with an insufficient intake in a number of micronutrients. FUTURE PROSPECTS AND PROJECTS: Identification of the groups at risk favours a better adequacy of preventive action on micronutrient deficiencies in this population. However, the efficacy of prevention is to be determined. Controlled trials of micronutrients' supplementation may be useful to value the beneficial effects of micronutrient supplementation on the incidence of cardiovascular diseases and cancer.

Adolescent↗
PubMed2004

[Acquired C1 inhibitor deficiency associated with lymphoproliferative disorders: four cases].

INTRODUCTION: Acquired C1 inhibitor deficiency is sometimes associated with lymphoproliferative disorders. EXEGESIS: We report four cases of acquired C1 inhibitor deficiency in association with lymphoproliferative disorders. Three of them were asymptomatic; one was associated with abdominal pain. Four women (median age, 66 years) presented either two non-Hodgkin lymphoma or two chronic lymphocytic leukaemia. C1 inhibitor deficiency was detected fortuitous (n = 1) or during investigation of arthralgia (n = 2), or Gougerot-Sjogren syndrome (n = 1). The deficit was acquired in all cases type I. Auto-immune disorders were associated with: Gougerot-Sjogren syndrome (n = 1), cryoglobulinemia (n = 2), IgM lambda monoclonal gammopathy (n = 1), Coombs positive test (n = 2), IgG anti-cardiolipine antibodies (n = 1). C1 inhibitor deficiency was not modified after lymphoproliferative disorders treatment (radiotherapy, splenic ablation) in two cases but patients were not in complete remission. C1 inhibitor raised normal level in one case, after five chemotherapy regimens, but decreased complement level and C4 split persist. CONCLUSION: Acquired C1 inhibitor deficiency associated with lymphoproliferative disorders is sometimes asymptomatic. Diagnosis could be delay in spite of clinical manifestations. Deficit correction is not constant after lymphoproliferative disorders treatment.

Aged↗
PubMed2004

European register of babies born to mothers with antiphospholipid syndrome.

This prospective multicentric register was initiated by the European Forum of Antiphospholipid Antibodies (APL) in 2003 after approval by local ethic committees. This register allows the investigation of infants after written informed parental consent. It collects mothers' clinical pattern of antiphospholipid syndrome (APS), course and outcome of pregnancy, treatment and immunological status. For the babies, clinical and immunological examinations are performed at birth; neurodevelopmental conditions followed up to five years. A re-evaluation of lupus anticoagulant (LA), anticardiolipin (ACL) or other antibodies will be done if they are positive at birth to follow their kinetics. A descriptive and a case control study of babies with versus without APL at birth will be possible after the inclusion of 300 cases.

Antiphospholipid Syndrome↗
PubMed2003

[Compression of the spinal cord revealing a seronegative rheumatoid arthritis].

INTRODUCTION: Craniocervical junction damages may result in a compression of the spinal cord. They may be caused by infectious, tumoral or inflammatory processes. Rheumatoid arthritis is probably among rheumatic diseases the most frequent cause of atlantoaxial arthritis. Nevertheless involvement of the craniocervical junction as the presenting symptom of rheumatoid arthritis is a very rare feature. EXEGESIS: We report the case of a 61 years old woman who presented with atlantoaxial involvement and spinal cord compression one year before the diagnosis of a seronegative rheumatoid arthritis. CONCLUSION: Symptomatic craniocervical junction damages may appear. Patients with damages of the craniocervical junction and negative investigations should be followed long-term; an underlying inflammatory disease may become evident after significant delay.

Arthritis, Rheumatoid↗
PubMed2003

[Treatment of systemic mastocytosis].

BACKGROUND: Systemic mastocytosis is a rare disease, characterized by mast cells proliferation in various organs. Two types of clinical manifestations can be distinguished: those related to mast cells mediators release and those related to tumoral proliferation involving different organs, these later defining aggressive systemic mastocytosis. Until recently, treatment was mainly symptomatic, without anti tumoral effect. RECENT FACTS: These last years, advances have been made in the understanding of the disease with the discovery of the c-kit oncogene mutation and the approach of the disease as a myeloproliferative disorder. PERSPECTIVES: Based on experiences acquired in the treatment of this kind of disorders, evaluation of new therapeutics, such as cladribine or combination of interferon-alpha and cytarabine is in progress. At least, tyrosine kinase inhibitors, a new family of molecules, are able of inhibiting some types of the mutated c-kit protein and one of them, imatinib mesylate, has shown a great efficacy in the treatment of gastro intestinal stromal tumors (GIST) which also involves the c-kit mutation. By analogy, treatment of patients with c-kit susceptible mutation might be treated with this molecule.

Adrenal Cortex Hormones↗
PubMed2003

Hyperthyroidism in two patients with Crohn disease and Takayasu arteritis.

Thyroid abnormalities and Takayasu arteritis (TA) have been reported separately in patients with Crohn disease (CD). We report two patients with hyperthyroidism, CD and Takayasu arteritis and discuss hypothetical mechanisms. Case 1. A thyrotoxic goiter was diagnosed in 1987 in a 34-year-old woman treated since 1969 for severe CD and TA. Iodine urinary excretion was 405 microg/mL (20-500). Anti-thyrotropin receptor (TRAK) and anti-thyroid antibodies were not detectable. The ultrasonography showed a nodule in the right lobe of the thyroid and two nodules in the left lobe. A 123I thyroid scan showed a multinodular goiter with no hot nodule. She was treated successfully with propylthiouracile until 1991, when a new episode of thyrotoxicosis led to a subtotal thyroidectomy. Case 2. Hyperthyroidism was diagnosed in February 2000 in a 49-year-old woman treated for CD and TA, both diagnosed in 1980. TRAK and anti-thyroid peroxydase antibodies were not detectable. The ultrasonography disclosed a normal thyroid volume with an inhomogeneous parenchymal structure and nodular images in both lobes. A 123I thyroid scan showed one hot nodule in the lower part of each lobe. A subtotal thyroidectomy was performed. The association of these three diseases may not be fortuitous, possibly explained by genetic predisposing factors and disease-related iodine deficiency both involving Nuclear Factor kappaB pathway.

Adult↗
PubMed2003

Questions about dexamethasone use for the prevention of anti-SSA related congenital heart block.

BACKGROUND: Mothers with anti-SSA/Ro antibodies who have had a previous fetus with congenital heart block (CHB) have a risk of recurrence estimated to be up to 16%. OBJECTIVE: To improve the management of these "high risk patients" by determining (a) whether or not prophylactic treatment is efficient; (b) whether or not fluorinated steroids (betametasone and dexamethasone) that do cross the placenta in an active form are safe for the fetus; and (c) which prophylactic treatment should be used. METHODS: Retrospective study performed on seven mothers sent to a university hospital owing to a past history of one (six mothers) or two children (one mother) with CHB. RESULTS: 13 subsequent pregnancies occurred. No CHB was observed. All four pregnancies in women treated with 10 mg/day prednisone were uneventful. Three pregnancies in women receiving no steroids resulted in two early spontaneous abortions and one live birth. The six pregnancies in women treated with dexamethasone (4-5 mg/day) ended in one early and one late spontaneous abortion, two stillbirths, and two live births with intrauterine growth restriction and mild adrenal insufficiency. A histological study of one stillbirth disclosed intrauterine growth restriction and marked adrenal hypoplasia. CONCLUSION: Adverse obstetric outcomes were often seen here and major concerns have been raised by paediatricians about the safety of fluorinated steroids, owing to the results of animals studies, retrospective data, and randomised trials. Because fluorinated steroids have not been shown to improve prophylactic treatment of CHB in pregnant women at high risk, their use is questionable.

Adrenal Insufficiency↗
PubMed2002

[Neonatal cutaneous lupus. Necessary interdisciplinary collaboration].

INTRODUCTION: Neonatal lupus erythematosus is a rare syndrome (affecting 5% of the children born of mothers with lupus), characterized essentially by cutaneous lesions and/or congenital auricular-ventricular heart block. It is due to the transplacental passage of maternal antibodies (anti-SSA or anti-SSB, or occasionally anti-U1RNP antibodies) into the fetal circulation. OBSERVATION: We report a case of neonatal lupus erythematosus, having appeared 4 weeks after birth. The 26 years old mother exhibited systemic lupus erythematosus concomitant to Gougerot-Sjögren's syndrome, with positive antinuclear factors (1/2560), native anti-DNA, anti-SSA and anti-SSB antibodies and anticardiolipin antibodies. During pregnancy, the mother had been treated with aspirin at the dose of 100 mg/day, followed by subcutaneous enoxaparin 0.4 ml/day, and combined with prednisone 10 mg/d and hydroxychloroquine 400 mg/day. Early and regular cardiac monitoring of the foetus was performed. The clinical examination and the electrocardiogram at birth were normal. Four weeks later, the infant presented with erythematous cutaneous lesions with atrophic center. No systemic treatment was initiated and the lesions partially regressed. CONCLUSION: Cutaneous lesions can also appear after the 4th week of life. It is important that the pediatricians clinically monitor all the children born to mothers exhibiting anti-SSA or anti-SSB antibodies, at least during the first 7 months of life.

Adult↗