Search PubMed⌕ Search

Biomedical subjects

E Hachulla

Publications and source records attributed to E Hachulla.

At least 181 records · Page 10Linked to original sources
PubMed1995

[Neurologic manifestations associated with antiphospholipid antibodies. Or what remains of neurolupus?].

Antiphospholipid antibody is associated with a clinical syndrome of vascular thrombosis, thrombocytopenia, recurrent fetal loss and livedo reticularis, whether or not a clinical diagnosis of systemic lupus erythematosus (SLE) coexists. Central nervous system involvement in SLE is multifactorial, thrombotic events, antineuronal antibodies, hypertension, infection, side effects of drugs etc. Antiphospholipid antibodies may play a role in focal neurological manifestations in SLE. In the absence of SLE, different neurological symptoms are well associated with antiphospholipid antibodies including stroke, seizures, dementia, migraine, ocular ischemia, chorea, transverse myelopathy, cerebral phlebitis. Other association are more controversal like Guillain Barré syndrome, motor neuron disease, communicating hydrocephalus. In all patients with antiphospholipid antibodies with neurological involvement, cerebral MRI may be performed with an echocardiographic study because a possible association with Libman and Sacks endocarditis, valve dysfunction or cardiac thrombus source of cerebral ischemia.

Antibodies, Antiphospholipid↗
PubMed1995

[Familial sarcoidosis: 3 cases in the same family].

Familial sarcoidosis is uncommon, representing less than 5% of the cases. We reported three cases of sarcoidosis in the same family (two sisters and a brother) with different clinical presentation in spite of the presence in each patient of HLA B8 DR3. These HLA phenotype may be related with favourable sarcoidosis. Until the two sisters had articular and benign lymph nodes diseases, the brother had multivisceral sarcoidosis involving the liver, the lung and the mediastinum. Corticosteroid were used during 6 month with favourable evolution. There is 16 years between the three sarcoidosis history but the disease appeared till the third decade. The two sisters and the brother did not live in the same city. These suggest than genetic predisposition is more effective than environmental basis.

Adult↗
PubMed1995

[Non-infectious inflammatory fasciitis: a borderline syndrome].

Inflammatory fasciitis without infection include different entity like eosinophilic fasciitis, the syndrome of eosinophilia-myalgia after tryptophan ingestion, toxic oil syndrome, exposure to trichlorethylene, phenylketonuria skin changes, the syndrome of palmar fasciitis, fasciitis in chronic graft-versus-host disease and fasciitis secondary to an adjacence process. The diagnosis of all these scleroderma-like skin changes is sometimes not easy because the clinical and sometimes the histopathological changes are bordeline manifestations with scleroderma. The most characteristic markers for non infectious fasciitis is eosinophilia and the infiltration of the fascia with eosinophilis, but it may be unremarkable or absent, only frequently present at the onset of the disease. Anamnesis is most important to guide the diagnosis. The eosinophils, but not only, may play a major role in the pathogenesis of this entity.

Eosinophilia↗
PubMed1995

[Digital arteritis, thrombosis and hypereosinophilic syndrome: an uncommon complication].

Digital necrosis has been exceptionally described in association with the hypereosinophilic syndrome. We report a case of a smoking patient with idiopathic hypereosinophilic syndrome who developed digital arteritis with necrosis. A radial artery biopsy confirmed the vasculitis with inflammatory infiltrates which were predominantly eosinophilic granulocytes. The eosinophils may have an active role in the development of the vasculitis.

Adult↗
PubMed1995

[HLA DRB1 polymorphism in rhizomelic pseudo-polyarthritis and Horton disease].

Some studies have suggested that distribution of HLA DRB1 alleles in polymyalgia rheumatica (PMR) resembles that found in giant cell arteritis (GCA). However these data are controversial. OBJECTIVE--To evaluate in French native patients whether PMR immunogenetically resembles GCA in determining HLA DRB1 alleles. PATIENTS AND METHODS--Fourty-five patients were included in the study. Twenty-one patients with PMR alone (Bird's criteria) and 24 with GCA (ACR criteria). In 11 patients, GCA was associated with PMR. HLA DRB1 genotype was determined by PCR-RFLP analysis. Statistical analysis was performed by the chi 2 test and determination of the odds ratio (OR). Two hundred and thirty-three unselected normal healthy subjects served as controls. RESULTS--A significant increased prevalence of HLA DR1 was observed in patient with PMR alone and an absence of DR7 (0% vs 10.3%, p = 0.02, OR = 0.1). An increased incidence of DR4 and particularly *0401 allele was only found in patients with GCA (OR = 2.4). No patient with isolated PMR had DR7 genotype compared with 25% in GCA (p < 0.001, OR = 0.03). A comparative study between isolated PMR versus GCA showed a significant increased in DR1 and DR3 alleles in isolated PMR and a significant increased prevalence of DR4 and DRB1 *0701 allele in GCA. CONCLUSION--The present study emphasizes the absence of similarity in HLA DRB1 allele distribution between PMR and GCA. The association of DR7 in patient with GCA seems characteristic in French native patients.

Aged↗
PubMed1995

Factors associated with Pneumocystis carinii pneumonia in Wegener's granulomatosis.

OBJECTIVE: To determine the factors associated with the occurrence of Pneumocystis carinii pneumonia (PCP) in Wegener's granulomatosis (WG). METHODS: We retrospectively compared a group of 12 patients with WG and PCP (PCP group), with 32 WG patients without PCP followed over the same period in the same centres (control group). RESULTS: The mean delay of onset of PCP after the start of the immunosuppressive therapy was 127 (SD 128) days. Before treatment, the clinical and biological features of the two groups were similar, except for the mean lymphocyte count which was lower in the PCP group than in the control group (1060/mm3 v 1426/mm3; p = 0.04). During treatment, both groups were lymphopenic. There was a significant difference between the lowest absolute lymphocyte count in each group (244/mm3 in the PCP group v 738/mm3 in the control group; p = 0.001). During the first three months of treatment, the lymphocyte count was less than 600/mm3 at least once in 10 of the 12 patients in the PCP group and in 11 of the 32 patients in the control group (p < 0.01). The mean cumulative dose of cyclophosphamide was greater in the PCP group than in the control group at the end of both the second (1.55 mg/kg/day v 0.99 mg/kg/day; p = 0.05) and the third (1.67 mg/kg/day v 0.97 mg/kg/day; p = 0.03) months. However, in multivariate analysis, the only two factors independently and significantly associated with the occurrence of PCP were the pretreatment lymphocyte count (p = 0.018) and the lymphocyte count three months after the start of the immunosuppressive treatment (p = 0.014). CONCLUSIONS: The severity of lymphocytopenia before and during immunosuppressive treatment is the factor best associated with PCP in WG.

Female↗
PubMed1995

Participation of cryoglobulinaemia in the severe peripheral neuropathies of primary Sjögren's syndrome.

A peripheral neuropathy is encountered in 10 to 50% of patients with primary Sjögren's syndrome (pSS). The pathogenesis of this complication remains unknown, but severe cases of peripheral neuropathies seem to result from a vasculitis. Since about 20% of pSS are associated with a cryoglobulinaemia, we initiated a retrospective study to assess the participation of cryoglobulinaemia in the severe peripheral neuropathies (SPN) of pSS. We found 6 cases of SPN (presence of pain and/or muscle weakness) in a group of 115 pSS (5%): 3 polyneuropathies and 3 mononeuritis multiplex. In all cases, the involvement was sensoromotor and axonal. Four patients had a biopsy-documented cutaneous vasculitis, and 2 of them had also a muscular lymphocytic vasculitis. A cryoglobulinaemia was detected in sera of 5 patients. Although the research was negative in the remaining patient, the presence of an IgM monoclonal gammapathy and of an hypo-complementaemia suggested also the presence of a cryoglobulinaemia. Complement was activated in the 6 cases. Treatment was aggressive: oral corticosteroid (6 cases), methylprednisolone pulses (4 cases), chloraminophen (1 case), plasma exchanges (2 cases), intravenous immunoglobulins (1 case). A stabilization or a partial regression of the neuropathies were observed in 1 case and 5 cases, respectively. This was associated with a reduction of cryoglobulin level and complement activation in 5 cases and 6 cases, respectively. This study suggests that SPN of pSS result from a cryoglobulin-mediated vasculitis.

Adult↗
PubMed1994

[Behçet disease. Comparative aspect of diagnostic criteria].

OBJECTIVES: Different diagnostic criteria for Behçet's disease are proposed in the literature. We compared the criteria proposed by Mason and Barnes with those proposed by the International Study Group and with those proposed by the Japanese committee. METHODS: This retrospective study included 41 patients with Behçet's disease according to the criteria set by Mason and Barnes. There were 16 patients of French origin and 25 patients of North African origin. RESULTS: Genital ulcerations were more frequent in the North African population (p < 0.007) and typical Behçet skin lesions were more frequent in the French population (p < 0.021). Comparing the clinical data among the 41 patients with Behçet's disease according to the Mason-Barnes criteria revealed that only 83% met the criteria set by the International Study Group and only 54% the criteria of the Japanese committee. For the International Study Group criteria, the requirement for recurrent buccal ulcerations with at least 3 episodes over a 12 month period (a major criteria) was the most limiting element. Likewise, genital ulcerations were not always recurrent. CONCLUSION: These findings confirm the variability of clinical manifestations of Behçet's disease with ethnic origin. Proposed diagnostic criteria vary depending on the study committee and should be used as diagnostic guidelines rather than as criteria required for positive diagnosis.

Adult↗
PubMed1994

Percutaneous vertebroplasty with acrylic cement in the treatment of a Langerhans cell vertebral histiocytosis.

A 25-year-old man developed multiple eosinophilic granuloma of bone including vertebral and sacral localization. Radiotherapy was initially administered. One year later, a relapse occurred in another vertebrae which was previously irradiated. Percutaneous vertebroplasty was, for the first time to our knowledge performed, in this indication, with a good clinical result with follow-up now for one year. We emphasize that such treatment is permissible only in symptomatic, progressive lesions, with threatened decompensation of spinal stability. This technique should be used only on an adult.

Adult↗
PubMed1994

[Cardiac complications in adult onset Still disease: from pericarditis to tamponade as manifestations].

Pericarditis is a common manifestation of adult still's diseases (ASD), observed in 20% to 3% cases. Pericardial tamponade is more seldom, less than ten cases have been reported in the literature. We reported two observations of patients with ASD and pericardial tamponade above a series of 18 patients with ASD, in one case revealed the disease. The first patient was a 32-year-old women and had fever, arthritis, high white blood cell count and developed pericardial tamponade. The evolution was favourable with bolus of methylprednisolone. In the second case, cardiac tamponade occurred several years after the onset of the disease. Surgical drainage was first required (800 ml) because of inefficacity of oral prednisone. A dramatic improvement was observed after bolus of methylprednisolone. In conclusion pericardial tamponade is a seldom but sometimes the first manifestation of ASD and may required in first intention bolus of methylprednisolone.

Adolescent↗
PubMed1994

[Sensitivity and specificity of antineutrophil cytoplasmic antibodies in systemic vasculitis].

The diagnostic value of c-ANCA as a specific marker of systemic vasculitis (particularly Wegener's granulomatosis) is well established. The prognostic value of c-ANCA for determining disease activity is controversial. We have prospectively studied in ten patients with systemic vasculitis over a mean period of 34 months (extreme 2-61 months). All patients had c-ANCA at the moment of the diagnosis: four patients had high titer of c-ANCA all over the period study; three clinical and biological exacerbations of the disease was observed without variation of the c-ANCA titer. In four patients c-ANCA disappeared within 6 months after the beginning of the treatment correlated with disease activity. Sometimes a rise of c-ANCA titer was observed with or without disease activity. In one case c-ANCA titer had a serrated evolution. The sensitivity and the specificity of the c-ANCA for disease activity in the ten studied patients were respectively 1 and 0.28. In patients with systemic vasculitis and c-ANCA at the time of the diagnosis, variation in c-ANCA titer alone is of limited prognostic value for predicting disease course.

Adolescent↗
PubMed1994

[Follow-up study, using dynamic transcutaneous oximetry in 17 patients with stage II Leriche and Fontaine occlusive arterial disease of the lower limbs].

We evaluated the value of dynamic transcutaneous oxygen pressure measurement (TcPO2) in 17 patients with stage II occlusive arterial disease of the lower limbs treated with exercise only. We studied 17 patients (15 men, two women) with an average age of 63 years (range 39-80 years). Claudication perimeter and dynamic TcPO2 were evaluated before and after 6 month walking exercise and tabac stopping. Four different sites of TcPO2 were studied: precordium (reference probe), thigh, calf and foot in the dorsal recumbent position after 30 minutes rest, during a standardised exercise stress test at 50 watts and during the recovery phase. The results were expressed as ratio of tissue oxygenation (RTO): thigh, calf or foot TcPO2/precordial TcPO2 x 100 in order to take into account the patients cardiorespiratory status and adaptation to exercise. Claudication perimeter was 255 m +/- 221 before 6 months exercise and 835 m +/- 539 after (P < 0.01). The duration of significative ischemia was significantly reduced after 6 months exercise (P = 0.02 calf, P < 0.01 foot). Dynamic transcutaneous oxymetry would therefore seem to be a useful method of assessing stage II occlusive peripheral arterial disease and the topography of tissue hypoxia. It could be valuable in orientating treatment and the first method to provide and objective evaluation of the efficacy of medical or surgical treatment.

Adult↗
PubMed1994

[Predictive signs of cancers in dermatomyositis. Study of 29 cases].

Dermatomyositis (DM) is characterized by an inflammatory myopathy and typical cutaneous lesions. In a retrospective study, 29 adult cases without overlap syndrome were reviewed. Our aim was to define predictive signs of cancer. Ten (four women, six men) of the 29 patients (35%) had DM associated with malignancy. The diagnosis of cancer was made concomitantly in six cases, more than year after (two cases), more than year prior to (two cases) the occurrence of DM. No extensive diagnosis procedures were necessary for identifying the tumors. A paraneoplasic evolution was certain for any patient, probable for six, improbable for fourth. For the group with malignancy: age was higher (P = 0.01), albumin lower (P = 0.04), C reactive protein higher (P = 0.0061). These parameters seem to have predictive value for the presence of malignancy.

Adult↗