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Biomedical subjects

E Hachulla

Publications and source records attributed to E Hachulla.

At least 163 records · Page 9Linked to original sources
PubMed1997

[Pseudoxanthoma elasticum: a rare cause of leg artery diseases in young adults].

Pseudoxanthoma elasticum (PXE) is a rare inherited connective tissue disorder which is characterized by elastic tissue degeneration involving the skin, eyes and arteries. We describe the case of a hypercholesterolemic 25 year-old woman with typical genetic dominant form of PXE revealed by distal limb artery involvement after a pregnancy. In young adults with arterial disease, a non atheromatous cause should always be sought for, even if risk factors for atherosclerosis are present.

Adult↗
PubMed1997

[Amyloidosis AL of the breast in an ileal carcinoid tumor].

An ileal tumor was detected in a patient with monoclonal plasmocytosis of the bone marrow (IgA Kappa). The pathological examination showed a carcinoid tumor with amyloid stroma. Immunotyping of the amyloidosis showed that it was stained with the antibody directed against light chain kappa, though the amyloidosis was located within a carcinoid tumor.

Amyloidosis↗
PubMed1996

[Glycoforms of alpha-1 antichymotrypsin in infectious process. Diagnostic value and follow-up].

OBJECTIVES: Three alpha 1-antichymotrypsin (alpha 1-ACT) glycans have been identified. We followed their levels during the septic processes in 24 patients in order to determine their diagnostic value in correlation with C-reactive peptide in patients with infection. METHODS: Sera were collected for assay on days 0, 3 and 6 after starting antibiotics. Erythrocyte sedimentation rate, alpha 1-ACT and C-reactive protein were determined. RESULTS: Erythrocyte sedimentation rate changed little while C-reactive protein fell sharply as in other inflammatory processes. Crossed immunoaffinoelectrophoresis showed a decreased formation of the Con-A non reactive fraction which disappeared rapidly after initiating antibiotics. Glycan microheterogeneity returned to normal at 6 days while C-reactive protein and alpha 1-ACT were still elevated. CONCLUSION: Synthesis and glycosylation of alpha 1-ACT are independent. Study of alpha 1-ACT glycan microheterogeneity may provide a useful test in the diagnosis of inflammatory processes of unknown origin and may be helpful in following-up patients with sepsis.

Adult↗
PubMed1996

Early expression of E-selectin, tumor necrosis factor alpha, and mast cell infiltration in the salivary glands of patients with systemic sclerosis.

OBJECTIVE: To assess the predictive value of early endothelial E-selectin and tumor necrosis factor alpha (TNF alpha) expression, as well as mast cell infiltration, in the subsequent progression to systemic sclerosis (SSc) in patients with Raynaud's phenomenon (RP) and abnormal nailfold capillaroscopic findings. METHODS: Clinical criteria were evaluated, and immunostaining was performed on lip biopsy samples from 22 patients with RP and abnormal capillaroscopic results. None of these patients initially fulfilled the American College of Rheumatology criteria for SSc. RESULTS: E-selectin, TNF alpha, and mast cell infiltration were found in 9, 10, and 8 of 11 patients, respectively, whose disease progressed to SSc, and in 0, 2, and 1 of 11 patients, respectively, whose disease did not progress to SSc (P < 0.001, P < 0.01, and P < 0.01, respectively). CONCLUSION: E-selectin, TNF alpha, and mast cell infiltration are detectable in the very early stages of SSc, prior to the onset of skin changes.

Adult↗
PubMed1996

[Benign angiopathy of the puerperium: a clinicoradiological case associated with ingestion of bromocriptine].

We report a case of benign cerebral angiopathy in a 37 year-old woman in the postpartum period occurring after bromocriptine therapy to suppress lactation. She presented with severe headache, hyperthermia and bradycardia. She had partial seizure. Angiography excluded cortical vein thrombosis but revealed multiple narrowing of intracerebral arteries. Bromocriptine was stopped. The patient totally recovered. A second angiography performed 3 months later was normal. Postpartum cerebral angiopathy is considered as rare but the prevalence is probably underestimated. Neurological features of this affection are multiple. Diagnosis is made by angiography performed in emergency and which showed narrowing of intracerebral arteries which are reversible. Prognosis is good. Reversible vasoconstriction may be due to acute arterial hypertension, sometimes triggered by ergot derivatives.

Adult↗
PubMed1996

[Initial manifestations and late diagnosis in chronic atrophic polychondritis: apropos of a series of 15 patients].

An auricular or nasal chondritis or a saddle nose deformity are the initial manifestation in half of cases of relapsing polychondritis; the other initial manifestations are various and less evocative; polyarthritis, laryngo-tracheal symptoms, episcleritis which delay the diagnosis. From 15 cases of relapsing polychondritis, the diagnosis time from the first symptom are studied; this one is long, about 3 years and 6 months (from 3 months to 17 years) in 13/15 of the cases even if the first manifestation is typical (external chondritis). In two cases only, the diagnosis was established after the first attack. This late of diagnosis had socioeconomical impact and exposed to severe complications like tracheal chondritis.

Adult↗
PubMed1996

[Sicca syndrome and hepatitis C virus infection: a Gougerot-Sjögren pseudo-syndrome?].

These last years, different virus have been incriminated in the etiopathogeny of the primary-Sjögren's syndrome and more particularly the hepatitis C virus. We have led a prospective study over 23 patients presenting a primary Sjögren's syndrome, searching for a sign of infection by the hepatitis C virus and over 23 patients presenting an active chronic hepatitis C virus searching for a Gougerot-Sjögren syndrome. The overcoming of the hepatitis C virus in the Sjögren group was 4.7% which was not significatively higher than in our sample of population. Parallelly, the search of the Sjögren's syndrome, in the hepatitis C virus group, found 4 patients (feminine, average age 48.5 years old) whose clinical board was compatible with this diagnostic. Antinuclear antibodies have not been found in any of the 23 patients. The lymphocytic typing of the infiltrate in the minor salivary glands biopsies showed a predominance of the CD8 lymphocytes in a proportion of 2/1, contrasting with what is observed in primary Sjögren's syndrome. We concluded that hepatitis C virus may be more associated with a chronic lymphocytic sialadenitis than an authentic primary Sjögren's syndrome.

Adult↗
PubMed1996

[Role of biopsy of the accessory salivary glands in Löfgren's syndrome and other forms of sarcoidosis].

The diagnosis of sarcoidosis requires histopathological analysis. Easy accessible site of biopsy is seldom. A systematic labial salivary gland biopsy was performed in 62 suspected sarcoidosis: 22 patients with Löfgren syndrome (group I), and 40 patients with systemic sarcoidosis (group II). Systematic bronchial biopsy was performed in eight patients of group I and ten patients of group II. If systematic biopsies were negative, direct biopsies were performed (lymph node, skin, kidney, liver). In group I, 8/22 labial salivary gland biopsies and 1/8 bronchial biopsies were positive; in group II, 17/40 labial salivary gland biopsies and 5/10 bronchial biopsies were positive. In the other patients, direct biopsies were positive: 27 lymph nodes, eight skin, eight hepatic, four kidney biopsies. In conclusion, labial salivary gland biopsy (even in the absence of sicca syndrome) is more reliable than systematic bronchial biopsies, particularly in Löfgren syndrome and may avoid in 30 to 50% of the cases more aggressive and dangerous biopsies such as liver, kidney or deep lymph nodes biopsies.

Adult↗
PubMed1996

Prospective and serial study of primary amyloidosis with serum amyloid P component scintigraphy: from diagnosis to prognosis.

OBJECTIVE: The purpose of this study was to assess the value of the serum amyloid P (SAP) component scintigraphy in patients with primary amyloidosis (AL). MATERIAL AND METHODS: Pure human SAP labeled with iodine-123 (123I-SAP) was given intravenously to 24 patients with biopsy-proven systemic amyloidosis (15 without multiple myeloma = group 1, and 9 with multiple myeloma = group 2) and to 6 patients with multiple myeloma without any clinical or biological signs of amyloidosis (group 3). Whole-body images as well as regional views and tissue retention levels were obtained after 24 hours. Our study was approved by the institutional review committee and all individuals gave informed consent and were prospectively studied (median 13 months, range 1 to 47 from the date of the scintigraphy to May 1995). RESULTS: Organ localization of 123I-SAP, indicating the presence of substantial visceral amyloid deposits, was observed in all patients in group 1 and 2. The organ uptake of 123I-SAP included the spleen (1 patient was splenectomized) in 20 of 23 cases (87%), the liver in 15 of 24 (60%), and the kidneys in 6 of 24 (25%). Myocardial 123I-SAP was never seen although 13 out of the 24 patients had clinical or echographic data for amyloidosis. Twenty-four hour tissue retention was significantly elevated in all patients (group 1 and group 2): 55.66% +/- 19.16% in group 1 and 34.37% +/- 24.92% in group 2, as compared with normal levels < 24%. The sensitivity of the technique was 79% when only organ uptake was considered but reached 100% when tissue retention was also considered. The 24-hour tissue retention might be correlated with the severity of the amyloidosis: mean survival in patients with tissue retention greater than 50% was 11.3 months versus 24.5 months in patients with levels less or equal to 50%. Five of the 6 patients with multiple myeloma without evidence of amyloidosis had abnormal 123I-SAP imaging and 24-hour tissue retention levels. In 2 of them, amyloidosis was secondly detected. In the 9 patients who had two scintigraphies, variations in 24-hour tissue retention values were in accordance with the clinical evaluation. CONCLUSIONS: Spleen and liver distribution of amyloidosis is mostly revealed by 123I-SAP scintigraphy in patients with AL amyloidosis. The uptake of 123I-SAP appeared in proportion to the quantity of amyloidosis present in different tissues, and the relative quantity of amyloid deposits in the myocardium, carpal tunnel, digestive tract, and kidneys was often small and seldom visualized by 123I-SAP scintigraphy. In contrast 24-hour tissue retention levels were abnormal in all cases of known AL amyloidosis. This may be a positive argument for the diagnosis of amyloidosis when histopathological tests are normal. Tissue retention levels appear important as they may be correlated with survival.

Aged↗
PubMed1996

Chronic pancreatitis: a complication of sarcoidosis?

Gastrointestinal localizations of sarcoidosis are rare. A total of 14 cases of pancreatic involvement in sarcoidosis has been reported previously. We report here a case of late onset idiopathic chronic pancreatitis diagnosed at the same time as sarcoidosis with liver, colonic, renal, mediastinal and neurologic involvement in a 56-year-old man. This case further suggests a possible relationship between idiopathic chronic pancreatitis and multisystem diseases.

Age of Onset↗
PubMed1996

Species specificity of anti-beta 2 glycoprotein I autoantibodies and its relevance to anticardiolipin antibody quantitation.

Some patients suspected of having antiphospholipid antibody syndrome (APS) were found to be positive for anti-beta 2 glycoprotein I (beta 2GPI) antibodies despite negative results for antibodies to cardiolipin (ACA). Since the major source of beta 2GPI in the ACA assay is animal (usually bovine) serum, we studied the influence on ACA quantitation of the species specificity of anti-beta 2GPI antibodies from patients with various autoimmune disorders, mostly systemic lupus erythematosus and primary APS. Ninety-seven sera were selected based on IgG (n = 76) or IgM (n = 64) positivity by ELISA using gamma-irradiated plates coated with human or bovine purified beta 2GPI. A higher proportion of IgM (43.7%) than IgG (7.9%) reacted to human, but not bovine, beta 2GPI. Furthermore, from the samples reactive to both proteins, the ratio of antibody level against bovine to that against human beta 2GPI was 1.08 +/- 0.58 for IgG and 0.58 +/- 0.3 for IgM (p < 10(-5)). IgG and IgM ACA were detected in 78 and 40 sera, respectively; concordance between the two ELISAs for ACA and anti-beta 2GPI antibodies was 94% for IgG and 75% for IgM. Out of 28 IgM showing recognition restricted to human beta 2GPI, 21 were missed by the ACA assay, possibly because of lower concentrations of beta 2GPI in those patients' sera. The antibody reactivity pattern towards human and bovine beta 2GPI of individual sera showed no variation with time and was related to the relative antibody avidity for each protein. A murine anti-human beta 2GPI monoclonal antibody, 9G1, that cross-reacts with bovine beta 2GPI, competed to a large extent with the patients' anti-beta 2GPI antibody binding sites whatever isotype involved or protein recognized. Therefore, anti-beta 2GPI antibodies of IgM isotype display a marked preference for human compared to bovine beta 2GPI responsible for frequent inconsistencies in the ACA assay.

Animals↗
PubMed1996

Treatment of mixed cryoglobulinemia with recombinant interferon alpha and adjuvant therapies. A prospective study on 20 patients.

In order to evaluate the efficacy of interferon alpha (IFNa) in mixed cryoglobulinemia (MC), a prospective multicenter clinical trial was conduced in April 1992. It consisted of treating 20 clinically symptomatic MC patients with IFNa for 26 weeks. Hepatitis C virus (HCV) infection was detected in 16 patients. A complete or partial clinical remission was obtained in 12 patients (60%). Eleven of these 12 responders (91.6%) experienced a clinical relapse less than 12 months after the end of therapy. Side effects were noted in 10 patients (50%). It was concluded that subcutaneously administered IFNa does not provide long-term remission.

Adrenal Cortex Hormones↗
PubMed1995

[Mycoplasma infection with Stevens-Johnson syndrome and antiphospholipid antibodies: apropos of 2 cases].

We report two cases of Mycoplasma pneumoniae infections associated with Stevens-Johnson syndrome and antiphospholipid antibodies. Such an association has been noted once in the literature. The relationship between antiphospholipid antibodies and Stevens-Johnson syndrome and others cutaneous manifestations of infections diseases is discussed. Though mainly described in systemic lupus erythematosus and autoimmune diseases, anticardiolipin antibodies and lupus anticoagulant have been found in many infectious disorders. But in the latter conditions, they have been considered by many authors as "non pathogenic" or "non prothrombotic" on epidemiologic and immunologic data. We suggest that antiphospholipid antibodies could possibly play a role in their pathogenesis especially as the mechanisms are not to date clearly understood.

Antibodies, Antiphospholipid↗