Intracranial haemorrhage due to vitamin K deficiency associated with alpha-1-antitrypsin deficiency type PiZ.
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Biomedical subjects
Publications and source records attributed to C Vazquez.
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Ninety-three Spanish children suffering from coeliac disease and 103 control subjects from the same area were screened for the amount of alpha 1-antitrypsin (alpha 1AT) and for any electrophoretic variations in it. In this case-control study no significant differences were detected either in phenotype distribution or amount. The present results indicate that no genetic association exists between alpha 1AT and coeliac disease.
In the autolytic phase of growth Schizophyllum commune lost 62% of its dry weight in 70 days of incubation. The variations in the activity of some lytic enzymes were studied in the culture fluid and mycelial extracts during growth and autolysis of this fungus. The enzymes 1,3-beta-glucanase (exoglucanase), 1,3(4)-beta-glucanase (endoglucanase), alpha-amylase, and invertase behaved in the same way in culture fluid and mycelial extract, but their activities were much higher in the culture fluid. The enzyme activities increased during autolysis, but then decreased at the end of this period except in the case of alpha-amylase which remained high. It was only possible to detect 1,6-beta-glucanase, cellulase, and polygalacturonase activities at certain times during the autolytic phase of growth. The enzyme chitinase was not detected and 1,3-alpha-glucanase (S-glucanase) occurred in the mycelial extract at a higher concentration than in the culture fluid. A decrease in the activity of this enzyme in the mycelial extract and an increase in the culture fluid occurred during autolysis. The enzyme 1,3-alpha-glucanase exhibited two optima pH, one at 6.0 and the other at 8.0. The Km value for the latter was 0.02 M at pH 5.5 in borate-citrate-phosphate buffer.
A 38-year-old male with proven Takayasu's arteritis presented, in a routine investigation, with thrombocytopenia, prolonged whole blood clotting time and activated partial thromboplastin time. Further studies demonstrated low levels of factor IX caused by a circulating anticoagulant. Immunological studies revealed an IgG (with kappa chains predominance) nature of this. Corticoid therapy decreased but did not suppress the anticoagulant activity. Since similar coagulation abnormalities have been described in collagen diseases, this observation is in support of this etiology to be considered in Takayasu's arteritis.
A method for isolation of membrane and flagellar fractions from Trypanosoma cruzi epimastigotes, and descriptions of their ultrastructural characteristics and antigenic activity are presented. Electron-microscopic observation revealed that the membrane fraction resembled vesicles, as occurs frequently when the pressure-depressure method is used for cell disruption. The flagellar fraction revealed flagella and some membranes, both related and unrelated to the flagellar structure. Flagellar and membrane fractions had 5 and 3 precipitin lines respectively against anti-whole homogenate of T. cruzi, in double diffusion tests. One of the precipitin bands common to both fractions gave a reaction of identiy with one precipitin line of the microsomal fractions, that was not present in the mitochondrial, nuclear and cell sap fractions. Data from tests of protective activity activity obtained during a period of 3 years with whole homogenate and F and M fraction are also presented Protective activity against lethal challenge doses of trypomastigotes is strongly associated with the flagellar fraction.
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PURPOSE: To evaluate the usefulness of a new technique of transvascular renal biopsy with a flexible forceps in high-risk patients and compare its results with those reported for other techniques of renal biopsy. PATIENTS AND METHODS: Sixteen transfemoral renal biopsies were performed in 13 high-risk patients. Indications included severe bleeding disorders, combined renal-hepatic biopsy, morbid obesity, solitary kidney, and poor clinical condition. The technique was performed through the femoral vein; a 7-F Mullins introducer sheath was placed in a subcortical renal vein, and the biopsy was performed with a flexible biopsy forceps. RESULTS: Renal tissue was obtained in 15 of 16 procedures (93.7%) with at least one glomerulus in 14 of 16 (87.5%), and at least six glomeruli in 11 of 16 procedures (69%). Samples adequate for diagnosis were obtained in 13 of 16 procedures (81%). Mean number of glomeruli per procedure was 12.6 (range, 0-39). Aside from transient hematuria in two patients, no other complications were known to have occurred. CONCLUSIONS: Transfemoral renal biopsy is a safe technique for acquisition of renal samples in high-risk patients. The procedure can be performed at the same time as other vascular techniques. The histopathologic results, although worse than those obtained with the percutaneous approach, are better than those reported with other transvascular techniques.
The concentration of somatostatin was studied in controls as well as in plasma and jejunal mucosa from celiac children with and without jejunal villous atrophy. The binding of somatostatin to cytosolic fraction, isolated from jejunal mucosa from celiac patients and controls, was also investigated. Fasting plasma somatostatin concentrations were not significantly different among the various groups studied. However, the jejunal somatostatin content was significantly higher in the group of celiac children with subtotal villous atrophy as compared to control group and celiac children with normal villous architecture. The binding of somatostatin to cytosol of jejunal mucosa was significantly decreased in the group of celiac children with subtotal villous atrophy. The present results suggest that somatostatin may be involved in the pathophysiological processes of celiac disease.
In order to investigate if a plasma profile of gastrointestinal peptides reflects changes in jejunal mucosa in celiac disease, we studied basal and postprandial plasma levels of gastrin, secretin, somatostatin, vasoactive intestinal polypeptide (VIP), and neurotensin in children with untreated and treated celiac disease and in a control group of children. Basal and 30-min postprandial secretin concentrations were statistically significantly lower in untreated celiac children compared to both treated celiac (p less than 0.01 and p less than 0.05, respectively) and control children (p less than 0.001). Plasma secretin levels 30 min after a breakfast meal were also statistically significantly lower (p less than 0.001) in treated celiac children with respect to the control group. In both untreated and treated celiac groups, basal and postprandial plasma levels of somatostatin and VIP were statistically significantly decreased (p less than 0.001) compared to control children. Moreover, there was a significant rise in postprandial levels of neurotensin after a breakfast meal in untreated celiac children. On the contrary, there was no rise of neurotensin in healthy children. These findings seem to indicate that determination of plasma profile of gastrointestinal peptides in children with celiac disease may be useful in monitoring the development of this disease and, thus, the number of jejunal biopsies could be decreased.
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The association of cancer and Crohn's disease is well documented and comprises 2% of cases, but the onset of a malignant lymphoma during the course of the disease is a very rare finding (20 cases reported). A patient presented with a malignant plasmocytic lymphoma complicating an ileal Crohn's affection. Both clinical and radiological diagnosis were uncertain, most such cases being discovered perioperatively, confirmation of the diagnosis being provided by histology and immunohistology, which also allow classification of the lymphoma to allow adaptation of complementary postoperative treatment.
The decision on whether to operate or not abdominal aortic aneurysms (AAA) in elderly depends on the relative risk of the operation versus the natural course of the unoperated AAA. From January 1984 to December 996, 138 patients, aged 80 years and older, were referred to our department for an aneurysm of 40 mm or more (transverse diameter) of the infrarenal abdominal aorta (95 asymptomatic, 15 painful, and 28 ruptured AAA). For 58 patients with asymptomatic AAA, operation was denied at referral because of transverse diameter less than 50 mm (n = 21), patient refusal (n = 10) or unacceptable operative risk or poor general condition (n = 27). Thirty-four of these observed AAA were ultimately operated after a mean delay of 41 months because of aneurysm enlargement (n = 15), aneurysm tenderness (n = 6) or rupture (n = 13). Overall, 52 patients had immediate (n = 37) or delayed (n = 15) elective repair of their AAA, with an in-hospital mortality of 5.7%. Urgent operation was done for 21 patients with a painful AAA. Six patients died at hospital (28% mortality rate). Emergent surgery was applied to 41 patients with ruptured AAA (including 13 AAA who ruptured during surveillance). The operative mortality in this subgroup attained 68%. Follow-up for the 77 survivors and the 24 non-operative patients averaged 43 months. The 5-year survival (operative mortality included) is 47% for electively operated patients, 30% for urgently and 20% for emergently operated patients. For comparison, the 5-year survival of an age and sex matched Belgian population is 63%. For the 24 medically followed AAA, the 5-year survival was 33%. In six cases, the cause of death was rupture of the AAA. Of the 58 patients for whom operation was initially not considered, 19 (33%) presented AAA rupture (13 operated in emergency and 6 who never came to surgery). The operative outcome of AAA repair in octogenarians is less favourable than in the younger age group (3.6% mortality after elective repair, 44% after operation for AAA rupture, according to our institution data). The authors conclude that AAA surgery should not be denied to octogenarians on the basis of advanced age alone. They recommend a straightforward surgery for otherwise healthy octogenarians with AAA of 50 mm diameter, surveillance up to 60 mm for high-risk patients and no surgery for unfit, bedridden or demented patients.