[Socio-demographic characteristics and health status of patients at a free-of-charge outpatient clinic in Paris]. / Analyse du profil socio-démographique et médical des patients de 5 permanences d'accès aux soins de santé (PASS) parisiennes.

PURPOSE: In the context of the French National Health Service, a free access to healthcare facilities (the PASS: "permanence d'accès aux soins de santé") has been implanted in 2000 for patients without health insurance or those dealing with financial hardship. There is few data about socio-demographic characteristics of the patients using these services. The objective of this study was to provide descriptive data about socio-demographic characteristics and motivation of those patients who use these clinics. METHODS: This descriptive cross-sectional study was conducted between April an May 2008, in 5 PASS clinics from academic tertiary hospitals in Paris. Descriptive data on patient were collected by general practitioners at the end of their consultations. RESULTS: This study included 581 patients. The mean age was 42 years, and 65% of patients were males. Only 50.9% declared a salary income and 38.5% had a health insurance. Half of the patients were homeless, and 80% were migrants. The main reasons to visit these health facilities were direct access (no appointment needed), being in financial difficulty and having a medical record in the same hospital. Half of the patients had one chronic disease at least, while only a third of them saw regularly a physician. A total of 834 diseases were found among the 581 patients, including 411 chronic diseases, and 17% of the patients had a psychologic or a psychiatric disorder. Prognosis was divided in three grades: good, low and poor. Almost a half of the patients were considered by the doctor as having a low or a poor prognosis if they would not receive a therapy. CONCLUSIONS: The findings of this study suggest that the PASS carry out their mission: most of the patients frequenting these facilities live under poor conditions and are in poor health status compared to the patients having access to conventional outpatient services.

The association of systemic lupus erythematosus and myasthenia gravis: a series of 17 cases, with a special focus on hydroxychloroquine use and a review of the literature.

The coexistence of systemic lupus erythematosus (SLE) and myasthenia gravis (MG) is rarely reported, and most of the published studies are case reports. Hydroxychloroquine, an antimalarial agent, is an essential treatment in patients with SLE but special caution is recommended when used in MG patients. We retrospectively analyzed the clinical features, laboratory findings, and outcome of 17 patients with both diseases with a special focus regarding hydroxychloroquine use and with a review of the literature. All patients were women. The mean age at MG onset and SLE diagnosis was 34.5 [14-64] and 37.8 [18-72] years, respectively. The presenting symptoms of MG were limb weakness (94%), ocular (88%) and bulbar involvement (53%). Autoantibodies against the acetylcholine receptor were positive in 94% of cases. The main manifestations of SLE included arthritis (88%), cytopenias (53%) and skin rash (41%). Treatment of SLE required hydroxychloroquine (94%), steroids (47%) and immunosuppressive drugs (18%). Among eight patients (47%) who developed MG after initiation of hydroxychloroquine, the question of induction of MG by hydroxychloroquine was raised in one patient. On the other hand, an exacerbation of myasthenic symptoms was only seen in one of the eight patients who received hydroxychloroquine after the diagnosis of MG. Including our cases, we reviewed a total of 70 patients with SLE and MG. Compared with a large series of 1,000 unselected SLE patients, those with associated MG were older, had lower incidence of cutaneous, renal, and neurological manifestations, and higher frequency of anticardiolipin antibodies and lupus anticoagulant. In conclusion, the clinical pattern of patients with SLE and MG seems to be characterized by a less severe course of SLE and higher frequency of antiphospholipid antibodies. Hydroxychloroquine treatment appears to be safe in this setting.

Systemic mastocytosis and bone involvement in a cohort of 75 patients.

OBJECTIVES: To investigate bone involvement in a large cohort of systemic mastocytosis (SM) patients, and evaluate the efficacy of bisphosphonate therapy. PATIENTS AND METHODS: From 2000 to 2004, 75 patients with SM according to WHO criteria underwent skeletal x-rays and bone mineral density (BMD) assessment. Sequential BMD assessments were performed in nine patients treated with bisphosphonate (mean follow-up 65 months). RESULTS: 37 patients (49%) had bone involvement according to both x-rays and BMD evaluations: osteoporosis (23 patients, 31%, mean lumbar spine T score: -3 SD), with vertebral fracture (13 patients, 17%), axial skeleton osteosclerosis (six patients, 8%), mixed patterns (three patients), osteopenia with pre-existing fractures (four patients) and focal osteolytic lesion (one patient). Blood count abnormalities were associated with osteosclerosis (p=0.005). In nine patients with osteoporosis and bisphosphonate therapy, mean lumbar spine BMD increased from 0.83 to 0.92 g/cm(2) (+11.1%; ie, +2.05% per year) without recurrence of vertebral fracture. CONCLUSION: Half of adult patients with SM have bone involvement. Osteoporosis is the most prevalent bone manifestation in SM (31%). Bisphosphonate therapy seems efficient to improve lumbar spine BMD during SM-related osteoporosis. Spine x-ray and BMD should be performed in all SM patients to detect those who may benefit from anti-osteoporotic therapy.

[Loss of consciousness in a child due to loperamide]. / Perte de connaissance chez une jeune enfant secondaire à la prise de lopéramide.

Loperamide is an antidiarrheal peripheral opiate agonist, with rare neurological secondary effects. We report the case of a 26-month-old child who had impaired consciousness under treatment by loperamide, and was treated successfully with naloxone. Limitations of the use of loperamide in young children are underlined. Naloxone may be used in case of impaired consciousness, for a diagnostic as well as therapeutic purpose.

[Treatment of systemic mastocytosis]. / Traitement des mastocytoses systémiques.

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.

[Unusual good prognosis for X-linked myotubular myopathy]. / Forme inhabituelle de bon pronostic de la myopathie myotubulaire liée à l'X.

Recessive X-linked myotubular myopathy has recently been shown to be linked to the mutation of a gene located in the Xq28 region. Evolution is used to be considered as fatal but mild forms or forms with a better prognosis have been recorded since. We report a case in a patient, whose parents were warned of fatal outcome once the diagnosis was made during the neonatal period. The reaction of the parents was to avoid any relationship with medical care. Ten years later the patient was seen in relatively good health thus proving that the evolution was more favourable than anticipated.

[Organization of patient management in level II centers in the Paris area: a prospective survey]. / Organisation de la prise en charge dans les centres de niveau II en région parisienne: enquête prospective.

Perinatal asphyxia is a common emergency for both obstetricians and pediatricians. A prospective study was conducted in 14 maternity hospitals (type II centres) in the Paris suburbs in order to assess pediatric activity and neonatal morbidity associated with supposed perinatal asphyxia in term newborns. Pediatricians were called in at birth very frequently: 1/20 deliveries. Intubation and/or resuscitation procedures were needed in 20% of cases and 20% of infants were referred to a neonatal unit for birth asphyxia or associated pathology. Moderate encephalopathy was observed in 1.5% of all term newborns who needed medical intervention for supposed birth asphyxia.

Deflazacort versus prednisone in patients with giant cell arteritis: effects on bone mass loss.

OBJECTIVE: To compare bone mass loss due to deflazacort versus prednisone in longterm treatment of patients with giant cell arteritis (GCA) in a randomized double blind comparative trial. METHODS: Seventy-four patients were included in a prospective multicenter study. Half received deflazacort (DFZ) and the other half prednisone (PR) for a minimum of 12 months. Calcium and vitamin D supplements were also provided to all subjects. Our intent was (1) to evaluate bone mineral density, using dual energy x-ray absorptiometry, at baseline and comparatively at 3, 6, and 12 mo; vertebral fractures by Meunier score and size variations after 12 mo treatments were also analyzed; (2) to assess calcium/phosphate metabolism modifications in both groups at baseline and after 12 mo. RESULTS: No significant difference was observed between the 2 groups in terms of treatment efficacy. Patients taking PR were slightly older on average versus the DFZ group (74 vs 70 yrs). Bone mass loss between entry and month 12 was not statistically different in the PR group (-0.026 +/- 0.007 g/cm2) compared to the DFZ group (-0.03 +/- 0.005 g/cm2). No significant difference was found in Meunier score variations (0.77 and 1.18 in the PR and DFZ groups, respectively; p = 0.3), nor in vertebral size variations (-0.4 and -0.2 in the PR and DFZ groups, respectively; p = 0.4). There was no difference in calcium/phosphate metabolism evaluations at month 12. CONCLUSION: In older patients taking longterm glucocorticoids who are at risk of osteoporosis, deflazacort did not result in less bone loss than prednisone.

[An unknown cause of prolonged fever: apropos of 6 cases of chronic aortic dissection]. / Une cause méconnue de fièvre prolongée: à propos de six cas de dissection aortique chronique.

PURPOSE: Aortic arch dissection may be sometimes misdiagnosed due to the lack of mild to moderate chest pain. Definite diagnosis is often made while dissection has already occurred more than 15 days ago, being thereafter considered as chronic. Aortic dissection may then present as a prolonged febrile illness with fever and/or inflammation as main symptoms, with little or no pain. METHODS: We retrospectively reviewed cases of chronic aortic dissections seen in a department of internal medicine and a department of neurology between 1975 and 1992. RESULTS: We report six cases of patients presenting with aortic dissection and describe their outcome and treatments after the diagnosis was made based on either thoracic computerized tomography or trans-esophageal echocardiography evidence. Four patients had surgical aortic arch repair while one patient was treated with beta-blockers. CONCLUSION: Chronic aortic dissection has rarely been reported to cause fever or increased sedimentation rate. Treatment has to be discussed between medical and surgical teams involved in the therapeutical management of these unusual patients.

[Sarcoidosis and pregnancy. A retrospective study of 11 cases]. / Sarcoïdose et grossess. Etude retrospective de 11 cas.

PURPOSE: To analyze the evolutive profile of sarcoidosis together with reciprocal interactions between pregnancy and sarcoidosis. METHODS: All events that occurred during pregnancy in 11 women presenting with sarcoidosis were analyzed. Histological confirmation was obtained for the 11 cases. For all pregnancies were analyzed the course of both sarcoidosis and pregnancy, and the influence of pregnancy on the disease evolution. RESULTS: Among 33 pregnancies, 23 led to the birth of healthy fetuses (five spontaneous abortions, four voluntary abortions, and one therapeutic abortion). The major event was fetal hypotrophy in six cases. Three of them occurred during pregnancy in prednisone-treated patients with active sarcoidosis. No relapse of cured sarcoidosis or further evolution of sarcoidosis that was inactivated as of the beginning of pregnancy were observed. The course of active sarcoidosis varied, as improvement (one case), worsening (two cases) and stabilization (two cases) were observed. During the first year of follow-up after delivery, four relapses and, in two cases, preliminary signs of the disease were observed. CONCLUSION: Apart from the hypothetical but not definite risk of hypotrophy, no negative interaction between sarcoidosis and pregnancy could be established. Pregnancy does not seem to interfere with the course of sarcoidosis. Considering the risk of relapse after delivery, pregnant women presenting with sarcoidosis should benefit from clinical and radiological follow-up.

Cryocrystalglobulinemia as a cause of systemic vasculopathy and widespread erosive arthropathy.

This report describes the case of woman who was admitted to the hospital for highly destructive axial and peripheral arthropathy in association with acute malignant hypertension and skin purpura. Type I IgGkappa serum cryoglobulinemia was identified and was classified as a monoclonal gammopathy of unknown significance. Cryoglobulin was shown to crystallize in the serum and synovium fluid and was responsible for both granulomatous microcrystalline synovial inflammation and occlusive vasculopathy in the kidneys and skin. Cryocrystalglobulinemia pathogenicity and therapeutic implications are discussed.

[Pleuropulmonary manifestations of systemic vasculitis]. / Manifestations pleuropulmonaires des vascularites systémiques.

Pleuropulmonary manifestations of systemic vasculitis are common, polymorphic and of ambiguous significance: the same pulmonary lesion may reveal a specific manifestation of vasculitis as well as a therapy-induced complication, especially infection which may favor per se a flare-up. Two questions will be successively studied: what are the pleuropulmonary characteristics of Wegener's granulomatosis, Churg-Strauss syndrome, periarteritis nodosa, Behçet's disease, Takayasu's disease and temporal arteritis? What are the major adverse effects that may occur in the course of a treated systemic vasculitis?

[Osteoporosis induced either by unfractionated heparin or by low molecular weight heparin]. / Ostéoporose induite soit par l'héparine non fractionnée soit par l'héparine de bas poids moléculaire.

Since the first suggestion of heparin-induced osteoporosis in 1963, a large body of work has been published. The mechanism can be explained by the effect of heparin as a cofactor for physiological stimulators of osteoclasts. Low-molecular weight heparins are an interesting alternative to non-fractionate heparin as the risk is reduced with an equivalent anticoagulation activity. Prevention is based on screening for patients at risk, preferential use of low-molecular weight heparins, early switch to anti-vitamin K and vitamin and calcium supplementation in pregnant women an elderly subjects. Curative treatment combines vitamin-calcium supplementation with inhibitors of bone resorption.