Biomedical subjects
Publications and source records attributed to E Hachulla.
Twelve cases of Pneumocystis pneumonia in patients with Wegener's granulomatosis were compared with a control-group of 32 patients. Our study showed that the dose of cyclophosphamide received in the first three months and a profound lymphocytopenia were strongly predictive of a risk of Pneumocystis carinii pneumonia in these patients.
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Arteriopathies of occupational diseases predominate in the upper limbs and are usually expressed as Raynaud's phenomenon ("white finger"), sometimes complicated by digital gangrene. Hand-arm vibration disease, hypothenar hammer syndrome, exposure to silica even without silicosis and vinyl chloride intoxication are the principal causes of occupational diseases acknowledged as such by the laws. In a personal series of 60 patients with complicated upper limb arteriopathies, 25% had a recognized occupational disease. Since December 1992, Erasmus' syndrome and systemic scleroderma due to silica without silicosis have been accepted in France as being occupational diseases. The therapeutic problems are discussed, notably the surgical indications in hypothenar hammer syndrome, the possible medical treatments of finger necrosis occurring in Erasmus' syndrome and, in particular, the role played by spinal cord neurostimulation.
Sarcoid-like necrotizing granulomatosis is an exceptional condition characterized by lesions of epithelioid granulomatosis and signs of vasculitis. It usually involves the lungs and has a favourable prognosis. We report the case of a young woman with a systemic form of the disease which affected not only the lung, but also the eye and the central nervous system. Under corticosteroid therapy the pulmonary lesions and the biological inflammatory syndrome disappeared, but the brain lesions followed a course of their own despite the addition to steroid treatment of an immunosuppressive therapy. This case highlights the superiority of MRI over CT of the brain in the diagnosis and monitoring of brain lesions: CT became normal after corticosteroid therapy was initiated, where as the neurological signs became worse and the brain lesions progressed on MR images.
We describe a patient with polyarteritis nodosa who developed a large retroperitoneal hemorrhage from a ruptured suprarenal capsule artery aneurysm. Platinum coils were embolized selectively during arteriography and successfully controlled the aneurysmal bleeding. The vasculitis was controlled ten weeks later but our patient developed anemia and computerized tomography scan revealed a large retrogastric hemorrhage. A further arteriogram confirmed the presence of a ruptured gastric coronary artery aneurysm. A second embolization was performed with complete success. Selective embolization of small arteries with coils should be considered a good alternative to surgery in patients with polyarteritis nodosa and hemorrhage from a ruptured aneurysm; arteriography may be considered a potent diagnostic and therapeutic tool in the management of the treatment of polyarteritis nodosa.
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1. Using crossed immunoaffinity electrophoresis with free concanavalin A in the first dimension, we studied the microheterogeneity of alpha 1-antichymotrypsin due to various glycoforms in sera from patients with various liver diseases and after liver transplantation. 2. Studies by isoelectric focusing and immunoblotting and by crossed immunoelectrophoresis without concanavalin A in the first dimension allowed us to show that there is no dramatic variation in electrophoretic heterogeneity of alpha 1-antichymotrypsin in the serum of patients with liver diseases or after liver transplantation when compared with that of normal subjects. Therefore the heterogeneity observed in crossed immunoaffinity electrophoresis is due to various interactions with concanavalin A. 3. The results were expressed as the ratio of concanavalin A non-reactive glycoforms plus concanavalin A weakly reactive glycoforms to concanavalin A reactive glycoforms, called R alpha 1-ACT. R alpha 1-ACT was significantly higher in patients with cirrhosis (n = 53) when compared with normal subjects (n = 30). The median R alpha 1-ACT was 1 (range 0.72-1.25) in normal subjects. It was 1.6 (range 1.18-3.02), 1.45 (range 0.65-4.12) and 2.24 (range 1.03-19) in cirrhosis of Child's grade A, B and C, respectively. There was a dramatic decrease in glycoforms with mostly biantennary glycans in some patients with Child's grade C cirrhosis. Serum levels of alpha 1-antichymotrypsin were lower than normal only in some patients with Child's grade C cirrhosis. 4. Among the patients with acute viral hepatitis studied (n = 17), five were studied longitudinally.(ABSTRACT TRUNCATED AT 250 WORDS)
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alpha 1-Antichymotrypsin purified from normal human serum was separated by affinity chromatography into th ree microheterogeneous forms on a concanavalin-A-Sepharose column: a pass-through (peak 1), a retarded (peak 2) and a bound form (peaks 3 + 4). For each form the asparagine-linked carbohydrate chains were liberated as oligosaccharides by hydrazinolysis, submitted to reduction with NaBH4 after re-N-acetylation and further separated by affinity chromatography on a concanavalin-A-Sepharose column. The complete primary structure of the glycans was determined by high-resolution 1H-NMR spectroscopy. The results indicated the presence of disialyl diantennary and of trisialyl triantennary type glycanic structures, the latter being accompanied by traces of disialylated triantennary oligosaccharide. The N-glycanase was used for the deglycosylation of the unfractionated alpha 1-antichymotrypsin; the successive removal of the N-linked complex-type oligosaccharide side chains of alpha 1-antichymotrypsin was studied in the presence of detergents. From these experiments it is concluded that alpha 1-antichymotrypsin carries four oligosaccharide side chains. Moreover our results show that the peak 1 contains four triantennary glycans, the peak 2 three triantennary and one diantennary glycans while the bound peaks 3 + 4 possess, on average, about one triantennary and three diantennary glycans per molecule. Since we showed that the peak 4 contains mostly diantennary glycans, it can be deduced that in peak 3 there are molecules carrying two triantennary and two diantennary glycans and others carrying one triantennary and three diantennary glycans.
Cholesterol crystal embolization is an often fatal disorder in the elderly. Clinical manifestations consist of skin lesions arterial hypertension and renal failure. In some cases the clinical picture is suggestive of vasculitis. The most frequent predisposing factors are operative and radiological vascular procedures and the use of anticoagulants. The diagnosis can be confirmed by skin, muscle or kidney biopsy. Data concerning management are scarce and contradictory. A review of the literature has revealed some controversy as to how and when cholesterol crystal embolization should be treated, and controlled studies are lacking. We discuss the use of various drugs such as anticoagulants, antiplatelet agents and corticosteroids. In practice, the usual treatment is symptomatic and includes therapy of the peripheral vascular disease, adequate control of blood pressure and appropriate management of renal insufficiency. The most effective measure is prevention.
alpha 1-antichymotrypsin, purified or in whole serum, exhibits microheterogeneous forms when studied by crossed immunoaffinoelectrophoresis with free concanavalin A (Con A) in the first dimension. alpha 1-antichymotrypsin purified from the serum of a single healthy donor was separated into three forms by affinity chromatography on a Con A-Sepharose 4B column: a Con A non-reactive form, a Con A weakly reactive form and a Con A reactive form. Some of their physico-chemical properties are compared. The complete primary structure of the glycans of each form was determined by high resolution 1H-NMR spectroscopy. The results indicated the presence of diantennary and triantennary type glycanic structures which occur frequently in serum glycoproteins. From deglycosylation experiments it is concluded that alpha 1-antichymotrypsin carries four oligosaccharide side chains. The Con A non-reactive form contains four triantennary glycans, the weakly reactive form contains three triantennary and one diantennary glycans and the Con A reactive form possesses on average one triantennary and three diantennary glycans. Significant variations in the relative ratios of the microheterogeneous forms were detected in various inflammatory syndromes. There is an increased proportion of Con A non-reactive form in patients developing a systemic disease (systemic lupus erythematosus, rheumatoid arthritis, temporal arteritis). In contrast, the proportion of Con A non-reactive form decreased in patients with acute septic inflammation whereas no variation appeared in patients with metastatic breast cancer. We also studied the variations of alpha 1-antichymotrypsin microheterogeneity in sera from patients with giant-cell arteritis and/or polymyalgia rheumatica before and during treatment with glucocorticoids.(ABSTRACT TRUNCATED AT 250 WORDS)
This enzyme-linked immunosorbent assay procedure for quantifying serum amyloid P (SAP) in human plasma makes use of affinity-purified polyclonal antibodies to SAP in a "sandwich"-type format. The procedure is sensitive, reproducible, simple, and easily automatable. Results correlate well with those by a rocket immunoelectrophoresis method performed with the same antibodies. Sera from apparently normal individuals had a mean SAP content of 44.17 mg/L and increased with age.
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While an inhibitory effect on natural killer (NK) cell activity was demonstrated with partially purified alpha 1 Achy, neither highly purified alpha 1 Achy from two healthy donors nor from one patient with giant-cell arteritis, which carries more highly branched glycans, inhibited the NK cytotoxicity. Our purification procedure, based on immunoaffinity chromatography and gel filtration, was not in question since the pure alpha 1-proteinase inhibitor (alpha 1PI) prepared in our laboratory by using a similar procedure continued to inhibit the NK cytotoxicity. If an inhibitory effect not related to antiprotease activity occurs with alpha 1PI, it is surprising that it is not shared by alpha 1 Achy which, like alpha 1PI, belongs to the serpin family and which possesses a strong structural homology with alpha 1PI. Our finding that alpha 1PI is able to affect human NK cytotoxicity while alpha 1 Achy (even with more highly branched glycans) is unable to suggests that events controlling NK activity may involve other enzymes than chymotrypsin-like enzymes.
1. Using crossed immunoaffinity electrophoresis with free concanavalin A in the first dimension, we studied the glycan microheterogeneity of alpha 1-antichymotrypsin in sera from patients with giant-cell arteritis and/or polymyalgia rheumatica, and its variation in the serum of several of these patients during induction of disease remission by prednisone therapy and possible further recurrence of giant-cell arteritis and/or polymyalgia rheumatica. 2. In the serum of patients with active disease we observed increased proportions of concanavalin A nonreactive and concanavalin A weakly reactive fractions. The results were expressed as the ratio of concanavalin A non-reactive fraction plus concanavalin A weakly reactive fraction to concanavalin A reactive fraction, called R alpha 1-ACT. An R alpha 1-ACT higher than 1.8 (upper normal value) was found in 30/31 sera from patients with active disease (sensitivity 97%) and in 2/22 sera from patients with inactive disease (specificity 91%). 3. The erythrocyte sedimentation rate and the serum C-reactive protein level, common markers of biological inflammation, are usually elevated in active giant-cell arteritis and/or polymyalgia rheumatica. The two parameters are commonly used to guide the therapy during the course of the disease, but they have no specificity. An erythrocyte sedimentation rate of greater than 30 mm/h was found in 30/31 sera from patients with active disease (sensitivity 97%) and in 5/22 sera from patients with inactive disease (specificity 77%).(ABSTRACT TRUNCATED AT 250 WORDS)
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In crossed immunoaffinoelectrophoresis with free concanavalin A in the first dimension, human serum alpha 1-antichymotrypsin, purified or in whole serum, exhibited four peaks in presence of 0.02 M alpha-methyl-D-glucoside added to the second-dimensional gel. alpha 1-Antichymotrypsin purified from the serum of a single healthy donor was separated by affinity chromatography into three fractions on a laboratory-prepared concanavalin A-Sepharose 4B column: a pass-through fraction, a retarded fraction and a bound fraction, eluted from the column on addition of sugar to the buffer. These three fractions were analyzed by crossed immunoaffinoelectrophoresis and sodium dodecyl sulfate-polyacrylamide gel electrophoresis and isoelectric focusing before and after desialylation. The results of electrophoresis as well as chemical analyses indicate that these microheterogeneous forms carry glycans with decreasing degrees of branching from the concanavalin A-pass-through form to the concanavalin A-bound form. This approach represents a first step towards the elucidation of the molecular basis of the microheterogeneity of alpha 1-antichymotrypsin.