Plasma phenylalanine concentrations are associated with hepatic iron content in a murine model for phenylketonuria

Individuals with phenylketonuria (PKU) have been reported to have altered trace mineral status. In this study, we evaluated in a murine PKU model whether protein level and level of phenylalanine (PHE) restriction could modulate iron, copper, and zinc status. Fifty-four male weanling PKU and control mice were assigned to receive for 56 days an elemental low or normal protein diet; PKU mice also were assigned to receive PHE restriction (treated) or no restriction (untreated). PHE-restricted mice consumed a prescribed dietary PHE to maintain plasma PHE concentrations between 120 and 480 micromol/L. PHE-unrestricted and control mice received equal amounts of dietary PHE. Intestinal and hepatic copper, iron, and zinc were measured at day 56 and fecal minerals measured at baseline and day 56. Mean plasma PHE concentrations were significantly greater in PKU PHE-unrestricted versus PKU PHE-restricted mice and control mice. Mean intestinal weights when normalized for body weight were significantly greater in PKU mice versus control mice. PKU PHE-unrestricted mice had significantly lower hepatic copper and zinc than PKU PHE-restricted mice, and significantly greater hepatic iron than control and PKU PHE-restricted mice. PKU PHE-unrestricted mice on a low protein diet had hepatic iron concentrations about 1.5 times that of the other mice. Fecal iron concentrations in all mice were significantly greater at day 56 than at baseline. No animal group effects or protein level effects were found for fecal copper, iron, or zinc contents. We conclude that hyperphenylalaninemia alters the metabolism of iron, copper, and zinc in PKU mice.     

Gastric myeloid metaplasia: a case report and review of the literature

We report a case of gastric myeloid metaplasia in an 89- year-old woman with agnogenic myeloid metaplasia. The lesions were fortuitously discovered on upper endoscopy. The antral mucosa was thickened and polypoid, and on histologic examination contained immature granulocytes, megakaryocytes, and a few erythroblasts without desmoplastic stromal reaction. The granulocytes were positive for CD15, CD68, and myeloperoxidase on immunohistochemistry, and the megakaryocytes showed positive reactivity for factor VIII. Gastric myeloid metaplasia is a very rare event, and to our knowledge only 6 cases have been reported in the literature to date. It usually occurs in patients with advanced myeloproliferative syndrome. Gastric myeloid metaplasia often has a pseudotumoral appearance, leading to digestive symptoms. Histologic diagnosis is straightforward when trilinear hematopoietic elements are identified in gastric biopsies. Immunohistochemistry with anti-factor VIII antibody can be useful to confirm the presence of megakaryocytes.     

Development and Evaluation of a Western Blot Kit for Diagnosis of Human Trichinellosis

We evaluated industrially prepared Western blot strips designed to avoid the cross-reactions observed with indirect immunofluorescence and enzyme-linked immunosorbent assays used for the serodiagnosis of trichinellosis. The antigen preparations were crude extracts of Trichinella spiralis. The Western blot profile characteristic of trichinellosis was characterized by comparing 60 sera from patients infected by Trichinella to 11 sera from healthy subjects, 51 sera from patients with other proven parasitic diseases (cysticercosis, schistosomiasis, strongyloidosis, fascioliasis, toxocariasis, liver amebiasis, anisakiasis, filariasis, toxoplasmosis, hydatidosis, or malaria), and 23 sera from patients with autoantibodies. Specific 43- to 44-kDa and 64-kDa bands were obtained with all of the sera from 51 patients with acute trichinellosis, in 4 out of 9 patients at the early stages of the disease, and in only 1 control patient, who had suspected anisakiasis and in whom trichinellosis could not be ruled out by muscle biopsy.     

Are HLA-DR or TAP Genes Genetic Markers of Severity in Ulcerative Colitis?

The pathogeny of ulcerative colitis (UC) is not yet elucidated, but some arguments suggest the implication of genetic factors. Among the candidate genes, those encoding for HLA class II genotypes have been extensively studied in UC; however, discordant data may be imputable to heterogeneity, characterized by immunological markers such as atypical ANCA (p-ANCA), or to inclusion of more or less intractable UC. The aim of our study is to evaluate the interest of HLA class II and TAP genetic markers to identify different clinical forms of UC, according to p-ANCA status. Unrelated patients with a history of UC (n=91) and healthy control subjects with no personal or family history of inflammatory bowel diseases (IBD) (n=200) were included. HLA-DRB103 was less frequent in UC patients than in healthy controls (8% vs 28%,PC<0.03). No association was found with any TAP genotypes. Moreover, there was no association with the HLA-DR2 specificity, either in the entire group of UC patients (38% vs 28%) or in the p-ANCA-positive subgroup of patients (30%). The most consistent finding in the present study is that some genetic markers may characterize intractability in UC patients. HLA-DR2 was associated with poor prognosis, regardless of p-ANCA status. In HLA-DR2 and non-HLA-DR2 groups, colectomy was done in 55% and 27% of patients, respectively (PC<0.05). Furthermore, in non-HLA-DR2 patients, p-ANCA could be of interest to characterize those with more severe prognosis. Our results confirm the interest of genetic studies to define UC genetic susceptibility, taking into account intractability of the disease. They do not support the hypothesis that p-ANCA is a subclinical marker of genetic susceptibility to UC.     

Interstitial deletion of band q12 of chromosome 5

A six-months-old girl is presented with psychomotor retardation and multiple congenital malformations. The karyotype done on peripheral blood lymphocytes and skin fibroblasts was found to be 46,XX del(5)(411q13). The parents are consanguineous. Their karyotypes were normal.
The genes for Arylsulphatase B and Hexosaminidase B are not located in band 5q12.     

Monomères acryliques à fonction carbonate cyclique, 2 Modification chimique de copolymères à groupements carbonate cyclique lateraux

Acrylic monomers with a cyclic carbonate function, their synthesis having been described previously, are used to synthesize copolymers with pendent cyclic carbonate side groups. Their chemical modification by ring opening addition reactions provides a convenient method for preparing functional polymers. The study of reactions with amines, on model compounds and on polymers, shows the possibility of obtaining a macromolecular network with crosslinking groups of carbamate type.     

Role of depth in eye distance measurements: Comparison of single and stereo‐photogrammetry

Photogrammetry has been used as an alternative to direct measurements to obtain facial distances for a variety of anthropometric applications. Taking measurements from photographs is less intrusive to subjects and reduces screening time, but measurements from single frontal photographs neglect depth information and may be inadequate for screening purposes. This study examined the role of depth in measurements of palpebral fissure length, interpupillary distance, inner canthal distance, and outer canthal distance using single‐ and stereo‐photogrammetry; an operator selected landmarks on single and stereo digital photographs displayed on a computer monitor. Depth was not found to make a significant contribution to eye distances in an idealized system where the real‐world coordinates of points on the eye were known from three‐dimensional calibration of stereo photographs. However, the differences found between measurements taken from single frontal photographs and those from stereo‐photogrammetry indicated that measurements from single photographs are prone to errors due to misalignment of the camera, the face, and the calibration instrument during image acquisition; if single photographs are to be used, the placement of these components should be carefully monitored. Am. J. Hum. Biol. 15:573–578, 2003. © 2003 Wiley‐Liss, Inc.     

Ascending aortic stenosis selectively increases action potential-induced Ca2+ influx in epicardial myocytes of the rat left ventricle

A decrease of the transient outward potassium current (Ito) has been observed in cardiac hypertrophy and contributes to the altered shape of the action potential (AP) of hypertrophied ventricular myocytes. Since the shape and duration of the ventricular AP are important determinants of the Ca2+ influx during the AP (QCa), we investigated the effect of ascending aortic stenosis (AS) on QCa in endo- and epicardial myocytes of the left ventricular free wall using the AP voltage-clamp technique. In sham-operated animals, QCa was significantly larger in endocardial compared to epicardial myocytes (803 +/- 65 fC pF(-1), n = 27 vs. 167 +/- 32 fC pF(-1), n = 38, P < 0.001). Ascending aortic stenosis significantly increased QCa in epicardial myocytes (368 +/- 54 fC pF(-1), n = 42, P < 0.05), but did not alter QCa in endocardial myocytes (696 +/- 65 fC pF(-1), n = 26). Peak and current-voltage relation of the AP-induced Ca2+ current were unaffected by AS. However, the time course of the current-voltage relation was significantly prolonged in epicardial myocytes of AS animals. Model calculations revealed that the increase in QCa can be ascribed to a prolonged opening of the activation gate, whereas an increase in inactivation prevents an excessive increase in QCa. In conclusion, AS significantly increased AP-induced Ca2+ influx in epicardial but not in endocardial myocytes of the rat left ventricle.     

Induction of Apoptosis by Equine Arteritis Virus Infection

Equine arteritis virus (EAV) is the etiological agent of equine viral arteritis, a contagious viral disease of equids. EAV is the prototype virus of the arteriviruses, a group of small enveloped viruses with positive single-stranded RNA genomes. Because apoptosis or programmed cell death is believed to play an important role in the biogenesis of several cytopathogenic viruses, we examined whether EAV was able to induce cell apoptosis in vitro. To do this, Vero cells were infected with EAV at a multiplicity of infection of 0.1 tissue culture infectious dose (TCID₅₀) per cell, and analyzed at various time intervals for the appearance of apoptotic signs. Fragmentation of chromosomal DNA into nucleosomal oligomers and caspase activation were observed in the infected cells at the time (e.g. 24 h postinfection) where a noticeable cytopathic effect was observed. The kinetics of the DNA fragmentation correlated with that of the production of progeny virus, so that viral multiplication was not interrupted by the apoptotic cell damage. All these data provide evidence that EAV is able to induce apoptotic cell death in vitro.