Alpha heavy chain disease. A Tunisian case

Alpha heavy chain disease is a rare affection in the West and reported mainly in developing countries with the improvement of hygienic conditions, the disease become rare in Tunisia, the last case was reported in 1991. The aim of the study is to report a new Tunisian case and to describe clinical, endoscopical and histological characteristics of the disease. The patient in a 37 years old male, presented with chronic diarrhoea associated with malabsorption syndrome. The diagnosis of alpha heavy chain disease was confirmed by histological examination of the resected intestine after surgery for intestinal obstruction. The patient received chemotherapy. The follow up is of two months.     

Effect of fatty acids on expression of endothelial leukocyte adhesion molecules

Summary.Background: With respect to linoleic acid both beneficial and proatherogenic effects have been described. However, the effect on expression of cell adhesion molecules on human coronary artery endothelial cells (HCAEC) is not yet established. The aim of the experiments was to evaluate the influence of linoleic acid in comparison with palmitic acid regarding the cytokine-induced expression of endothelial leukocyte adhesion molecules (intercellular cell adhesion molecule-1 ICAM-1, vascular cell adhesion molecule-1 VCAM-1, E-selectin).Methods: HCAEC were cultured in microvascular endothelial cell growth medium. In the experiments, the cells were preincubated with linoleic acid and palmitic acid, respectively (10 µmol/l, 2 days) or under control conditions, after which interleukin- 1 (IL-1, 10 ng/ml in the test medium) was added for 1 day. The monoclonal antibodies used were fluorescein isothiocyanate (FITC)- labeled anti-ICAM-1, FITC-labeled anti-VCAM-1, and FITC-labeled anti-E-selectin. Expression was analyzed by flow cytofluorimetry. Next, to examine the effects of fatty acids on adhesion of monocytes to endothelial cells, adhesion experiments with the monocytic U 937 cell line were performed.Results and conclusions: IL-1 increased ICAM-1,VCAM-1, and E-selectin expression compared to controls. Incubation with IL-1 together with linoleic acid reduced the expression of ICAM-1 and VCAM-1 in contrast to palmitic acid. Furthermore, in the presence of linoleic acid a tendency of diminished adhesion of monocytes is seen.The results indicate that a reduced expression of cell adhesion molecules may be relevant to the antiatherogenic effects of linoleic acid. This is in contrast to the properties of palmitic acid.     

Inhalation of ultrafine particles alters blood leukocyte expression of adhesion molecules in humans

Ultrafine particles (UFPs; aerodynamic diameter < 100 nm) may contribute to the respiratory and cardiovascular morbidity and mortality associated with particulate air pollution. We tested the hypothesis that inhalation of carbon UFPs has vascular effects in healthy and asthmatic subjects, detectable as alterations in blood leukocyte expression of adhesion molecules. Healthy subjects inhaled filtered air and freshly generated elemental carbon particles (count median diameter approximately 25nm, geometric standard deviation approximately 1.6), for 2 hr, in three separate protocols: 10 microg/m3 at rest, 10 and 25 microg/m3 with exercise, and 50 microg/m3 with exercise. In a fourth protocol, subjects with asthma inhaled air and 10 microg/m3 UFPs with exercise. Peripheral venous blood was obtained before and at intervals after exposure, and leukocyte expression of surface markers was quantitated using multiparameter flow cytometry. In healthy subjects, particle exposure with exercise reduced expression of adhesion molecules CD54 and CD18 on monocytes and CD18 and CD49d on granulocytes. There were also concentration-related reductions in blood monocytes, basophils, and eosinophils and increased lymphocyte expression of the activation marker CD25. In subjects with asthma, exposure with exercise to 10 microg/m3 UFPs reduced expression of CD11b on monocytes and eosinophils and CD54 on granulocytes. Particle exposure also reduced the percentage of CD4+ T cells, basophils, and eosinophils. Inhalation of elemental carbon UFPs alters peripheral blood leukocyte distribution and expression of adhesion molecules, in a pattern consistent with increased retention of leukocytes in the pulmonary vascular bed.     

Wolfram syndrome. A new case report

The WOLFRAM syndrome (SW) is a rare hereditary disorder described for the first time in 1938 as the coexistence of a diabetes mellitus and an optic atrophy to which join frequently a diabetes insipidus and a bilateral deafness. Several genetic studies are current to determine transmission mechanisms, physiopathology of the disorder to update a curative therapy. On the occasion of a new case report we remind the main characteristics of this syndrome. The SW is a rare neurodegenerative disorder characterized by the coexistence of diabetes mellitus, diabetes insipidus, optic atrophy and deafness. Several other manifestations are frequently associated: neurological, urologic and endocrinous abnormalities as well as growth troubles. Only diabetes mellitus and optic atrophy are compulsory for the diagnosis. This Syndrome is transmitted to the autosomic recessive mode. Mitochondrial heredity is in the course of study. The systematic practice of a cerebral IRM during a SW allows finding several malformative abnormalities of which the most typical is the agenesis of the post-pituitary gland. The prognosis of the affection is essentially conditioned by the evoluting complications of the uropathy and the diabetes mellitus. The treatment is symptomatic. Genetic therapy is in.     

Reference ranges of 9 plasmatic proteins in Tunisian population based on the new IFCC reference material CRM 470

The interpretation of biological exam results requires the knowledge of physiological variation factors and reference values for every parameter. Following the preparation and the diffusion by the International Federation of Clinical Chemistry (IFCC) of a new reference material for the dosage of plasmatic proteins (CRM470) and in order to take part in the international effort of standardization of these dosages, we established reference ranges in the tunisian population for 9 plasmatic proteins: the Immunoglobulines G, A and M, complement factors C3 and C4, albumin, transferrin, haptoglobin and the a 1-glycoprotéin acid. Our sample of 211 healthy blood donors aged between 18 and 63 years. Reference limits 0.95 that we obtained are located within variation of most reference values lately found in the literature.     

Effect of glutamine on cell adhesion molecule expression and leukocyte transmigration in endothelial cells stimulated by preeclamptic plasma

OBJECTIVE: This study analyzed plasma glutamine (GLN) concentrations in women with preeclampsia. Also, in an in vitro study we evaluated whether GLN concentration was related to surface molecule expressions on endothelial cells (ECs) and polymorphonuclear neutrophils (PMNs) and the transendothelial migration of PMNs through ECs stimulated by preeclamptic plasma. METHODS: Blood samples were collected from 20 women with preeclampsia and 15 normal pregnant women for plasma GLN analysis. In the in vitro study, human umbilical vein endothelial cells and PMNs were treated with different concentrations (0, 300, 500, and 1000 microM) of GLN for 24 h. After that, we stimulated human umbilical vein endothelial cells for 3 h with plasma from patients with preeclampsia, and PMNs were allowed to transmigrate through ECs for 2 h. EC surface expressions of cellular adhesion molecules (CAMs) and integrin (CD11b) interleukin-8 (IL-8) receptor expressions on PMNs were measured by flow cytometry. The transendothelial migration of PMNs through ECs was also analyzed. RESULTS: Women with preeclampsia exhibited significantly lower plasma GLN concentrations than did normal pregnant women. The in vitro study showed that, compared with normal plasma, CAM expressions on human umbilical vein endothelial cells and PMNs were increased when preeclamptic plasma was stimulated. Among the groups with preeclamptic plasma stimulation, intracellular CAM-1 expression on ECs and CD11b and IL-8 receptor expressions on PMNs were lower with 500 and 1000 microM than with 300 microM of GLN. IL-8 production from ECs and PMNs was also lower with 500 and 1000 microM than with 300 microM of GLN. PMN transmigration was significantly higher with 300 microM of GLN than with the other GLN concentrations. CONCLUSIONS: Plasma GLN is depleted in women with preeclampsia. The result of this in vitro study showed that ECs and PMNs were activated after preeclamptic plasma stimulation. A low GLN concentration resulted in greater CAM expression and greater transendothelial migration of neutrophils. GLN administration at levels similar to or higher than physiologic concentrations decreased IL-8 and CAM expressions, and PMN transmigration decreased after stimulation with preeclamptic plasma.     

Intrauterine undernutrition in rats interferes with leukocyte migration, decreasing adhesion molecule expression in leukocytes and endothelial cells

Experimental and epidemiologic data have shown that malnutrition predisposes individuals to infections. Immune responses are compromised, particularly in undernourished children. Therefore, we investigated the migratory capacity of leukocytes, using the intravital microscopy technique, in male Wistar rats (8-9 wk of age) that were undernourished in utero after their dams were fed 50% less food than the amount consumed by control dams. The number of leukocytes rolling along the venular endothelium, sticking after stimulation with leukotriene B4, tumor necrosis factor-alpha (TNF-alpha) or zymosan-activated plasma, or migrating after TNF-alpha stimulation was significantly reduced in the undernourished rat offspring. Compared with nourished rat offspring, undernourished offspring had significantly reduced numbers of circulating leukocytes, higher blood pressure, and higher leukocyte rolling velocity (V(WBC)), as well as a higher ratio between V(WBC) and RBC velocity (V(RBC)). Endothelial P-selectin and intercellular adhesion molecule-1 (ICAM-1) expression, analyzed by immunohistochemistry, and basal leukocyte L-selectin expression, analyzed by flow cytometry, were significantly reduced in the undernourished rat offspring. Because the groups did not differ in leukocyte CD11/18 expression, endothelial expression of platelet-endothelial cell adhesion molecule-1, or venular blood flow velocity and, consequently, venular shear rate, we conclude that intrauterine undernutrition in rats reduces leukocyte migration, downregulates endothelial expression of P-selectin and ICAM-1, as well as leukocyte expression of L-selectin, while reducing leukocyte counts. The higher V(WBC) and V(WBC)/V(RBC) ratio may also play a role in this reduced leukocyte migration. Our data suggest that this phenomenon is involved in the increased predisposition to infections in undernourished subjects.     

Left paraduodenal internal hernia. A new case report

OBJECTIVE: Remind the clinical and radiological features of the internal left para duodenal hernia. MATERIALS AND METHOD: We report the case of a left para duodenal hernia in a 30 years old woman who suffers of an acute abdominal pain explored by ultra sonography, barium upper gastro intestinal radiography, computed tomography and MRI. RESULTS: CT shows a well limited intra abdominal mass composed of small bowel wrapped in a bag of peritoneum, and moving to the left the mesenteric axis CONCLUSION: Left internal para duodenal hernia is a rare phenomenon and is frequently non diagnosed. Spiral computed tomography allows us now, to make the diagnostic with a good sensitivity in the majority of cases.     

Effect of mifepristone on endometrial matrix metalloproteinase expression and leukocyte abundance in new medroxyprogesterone acetate users

PURPOSE: This double-blind, placebo-controlled study was conducted to evaluate the molecular mechanism of mifepristone controlling breakthrough bleeding (BTB) in new depot-medroxyprogesterone acetate (DMPA) users. METHOD: A total of 50 regularly cycling women who were new starters of DMPA were randomized to receive 50 mg of mifepristone or placebo once every 14 days for six cycles. Endometrial biopsies were obtained on each patient before, during and after treatment. Endometrial matrix metalloproteinase 1 (MMP-1) and MMP-9 protein and mRNA were determined by immunohistochemistry and real-time PCR, respectively. The number of T lymphocytes (CD3-positive) and mast cells (mast tryptase-positive) was evaluated by immunohistochemistry. RESULTS: MMP-1, MMP-9, CD3-positive and mast tryptase-positive cells increased following the DMPA treatment. Addition of mifepristone to DMPA-exposed endometrium for 1 week significantly decreased stromal MMP-9 expression and numbers of CD3-positive and mast tryptase-positive cells. CONCLUSION: The decreased rates of BTB in new users of DMPA by mifepristone are associated with decreased MMP-1 and MMP-9 expression and fewer mast and T cells.     

A new method of repair for quadriceps tendon ruptures. A case report

Rupture of the quadriceps tendon is an uncommon injury observed predominantly in subjects over 40 years old. Multiple surgical techniques have been employed to repair fresh and neglected ruptures; methods that make use of allograft and augmentation with tissues harvested from around the knee have been reported. We describe a case of surgical repair of a tendon-bone junction rupture in a 64-year-old patient by use of suture anchors to attach the tendon to bone and improve fixation of the soft tissue elements. Clinical diagnosis of rupture was confirmed radiographically and echographically. Surgical repair was performed within 24 hours of injury. Active movement of the knee started after 3 weeks and the patient was permitted to walk without weight-bearing with a knee cage. Three weeks later, he was permitted to walk with full weight-bearing unassisted by crutches; the knee cage was removed 6 weeks after surgery. At his most recent follow-up 24 months postoperative, quadriceps strength was equal to that of the controlateral knee and the patient has returned to sports and daily activities. The surgical method presented here provides a suture of the tendon ends without putting excess stress on the suture line during the period of early knee mobilization. Advantages over other techniques include reduced operative time, easy access to the implantation site, and better resistance of the suture material: the patient is thus able to initiate physical therapy earlier and more aggressively.     

Multiple lymphomatous polyposis of the gastrointestinal tract: a Tunisian case

Multiple lymphomatous polyposis is a distinctive primary gastrointestinal lymphoma which endoscopical, histopathological et immunophenotypical characteristics are well known. This lymphoma is rare and its prognosis is bad because of frequency of stage IV patients. We report the case of a 75-year-old male patient with multiple lymphomatous polyposis affecting the rectum, the colon and the stomach associated with an involvement of lymphadenopathies, bone marrow and liver. Treatment by chemotherapy was ineffective and patient dead after 3 sessions of CEOP protocol.