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Biomedical subjects

A Ferrandez

Publications and source records attributed to A Ferrandez.

18 recordsLinked to original sources

Antibodies to carbonic anhydrase and IgG4 levels in idiopathic chronic pancreatitis: relevance for diagnosis of autoimmune pancreatitis.

BACKGROUND: Increased serum antibodies against carbonic anhydrase II (CA-II Ab) or IgG4 levels have been reported in cases of autoimmune chronic pancreatitis (ACP). AIM: To assess the relevance of serum CA-II Ab and IgG4 levels for the diagnosis of ACP in idiopathic CP (ICP) versus alcoholic CP and Sjogren's syndrome (SS). SUBJECTS: This was a multicentre study involving 227 subjects divided into four groups: ICP (n = 54), normal controls (n = 54, paired by age and sex with ICP patients), alcoholic CP (n = 86), and SS (n = 33). METHODS: CA-II Ab was measured by ELISA and confirmed by western blotting. A score of easy clinical use with major clinical, morphological, and biochemical parameters for the diagnosis of ACP was applied. RESULTS: The percentage of patients with increased serum CA-II Ab was higher in the ICP group (28%) than in controls (1.9%) and in patients with alcoholic CP (10.5%), but lower than in patients with SS (64%). The proportion with elevated IgG4 levels was higher in the ICP group (15%) compared with controls (1.9%) and SS (0%) but not significantly different from alcoholic CP (8%). Most ICP patients (7/8) with high IgG4 levels exhibited increased CA-II Ab and a compatible ACP score. A definitive diagnosis of ACP by histological analysis was associated with other autoimmune disorders, an increase in both serum IgG4 and CA-II Ab levels, and IgG4 positive plasma cells. CONCLUSIONS: The increase in serum IgG4 levels was strongly associated with elevated CA-II Ab levels, manifestations compatible with ACP, and lymphoplasmacytic infiltration when surgical specimens were available.

Adolescent↗

Soluble intercellular adhesion molecule-1 (s-ICAM-1/s-CD54) in diffuse large B-cell lymphoma: association with clinical characteristics and outcome.

BACKGROUND: High serum levels of soluble intercellular adhesion molecule-1(s-ICAM-1/s-CD54) have been associated with adverse clinical features and poor outcome in chronic lymphocytic leukemia, Hodgkin's disease and non-Hodgkin's lymphoma, but their value in the different subtypes of non-Hodgkin's lymphoma has not been well addressed. PATIENTS AND METHODS: Our aim was to study the serum levels of s-ICAM-1 in diffuse large B-cell lymphoma (DLBCL) and to correlate them with clinical characteristics and outcome. We analyzed the serum levels of s-ICAM-1 in a series of 55 patients with DLBCL diagnosed in a single institution. s-ICAM-1 levels were quantified by an immunoenzymatic assay. Median age was 62 years (range 22-96); 29 (53%) were male. Twenty-eight (51%) presented with advanced clinical stage (III/IV), 32 (58%) had extranodal involvement, 28 (51%) had high serum lactate dehydrogenase (LDH) and 23 (43%) had high beta2-microglobulin levels. All patients received anthracycline-containing regimens. Correlation between clinical variables and s-ICAM-1 levels were tested with the Mann-Whitney U-test and survival was plotted by the Kaplan-Meier method, and curves compared with the log-rank test. RESULTS: Serum levels of s-ICAM-1 were significantly increased in patients with DLBCL compared with normal controls (589 +/- 487 versus 279 +/- 65 ng/ml, respectively; P <0.001). Higher levels of s-ICAM-1 were present in patients with B symptoms, advanced stage and increased LDH and beta2-microglobulin. s-ICAM-1 levels also correlated with achievement of a complete response. Patients with s-ICAM-1 over 668 ng/ml had a shorter time to treatment failure (TTF) (3-year TTF, 59% versus 20%, respectively; P = 0.01) and overall survival (OS) (3-year OS, 58% versus 22%, respectively; P = 0.04) than the remainders. When only low and low-intermediate risk patients in the international prognostic index score were considered, those with s-ICAM-1 over 668 ng/ml also had worse TTF and OS. CONCLUSIONS: In DLBCL, s-ICAM-1 levels correlated with high tumor burden and lymphoma dissemination and may contribute to assessment of prognosis.

Adult↗

Numerical models of auto-regulation and blood flow in the cerebral circulation.

A two-dimensional time-dependent computational fluid dynamics model of the Circle of Willis has been developed. To simulate, not only the peripheral resistance of the cerebrovascular tree but also its auto-regulation function, a new "active" boundary condition has been defined and developed using control theory to provide a model of the feedback mechanism. The model was then used to simulate different common abnormalities of the Circle of Willis while a pressure drop, simulating a rapid compression of the right internal carotid artery, was imposed. Test results using a simple tube compared excellently with experiment. The total time-dependent flux for each efferent artery was tabulated and showed the important relationship between geometrical variations in the Circle of Willis and the auto-regulation of blood flow by vascular vaso-dilation and contraction. From this study, it was found that the worst case seemed to be that of a missing or dysfunctional right A1 segment of the anterior cerebral artery. The use of valid physiological models of the peripheral resistance allows for more realistic models of the blood flow in the Circle whilst allowing an easy extension to 3D patient specific simulations.

Blood Flow Velocity↗

Identification of two subgroups of mantle cell leukemia with distinct clinical and biological features.

INTRODUCTION: Mantle cell leukemia (MCLeu) has been considered as a leukemic form of mantle cell lymphoma (MCL). However, the presence of certain features rarely observed in MCL, such as transformation to prolymphocytic leukemia (PLL) or indolent clinical course, suggests that MCLeu may represent a distinct disorder. METHODS: Seven cases of MCLeu with t(11;14)(q13;q32) and BCL1-IGH gene rearrangement were ascertained among 140 newly diagnosed chronic B-cell lymphoproliferative disorders with leukemic expression. Comparative genomic hybridization, FISH for specific gene loci, and immunological studies were preformed in them. RESULTS: In comparison with CLL, MCLeu cases had low immunological scores < or =2 with respect to B-CLL (P<0.0001). Expression of CD38 was absent in 43% of MCLeu and in 44% of B-CLL. Comparative genomic hybridization analysis identified genomic imbalances in 86% of MCLeu with a similar pattern than in MCL: gains of 3q, 8q involving MYC gene and 15q, and losses of 6q, 9p, 13q and 17p affecting P53 gene. Differently from MCL and CLL, genomic loss of 8p was frequently detected in MCLeu (83%). Although clinical presentation of MCLeu was indistinguishable from CLL, all patients but one had disease progression within three years. According to the immunologic and genomic profiles, two distinct subgroups of MCLeu were defined: one related to PLL, showing CD38-, deletion of P53, and MYC amplification and another which corresponds to a leukemic form of classical MCL, presenting with CD38+ and normal P53 and MYC status. CONCLUSION: MCLeu and MCL are closely related disorders, as they show similar genomic and molecular patterns. However, the deletion of the short arm of chromosome 8 may represent a specific marker for MCLeu. Two distinct subgroups of MCLeu may also be distinguished according to the immunologic and genomic cell profiles.

ADP-ribosyl Cyclase↗

Molecular characterization of PadA, a phenylacetaldehyde dehydrogenase from Escherichia coli.

The padA gene encoding the phenylacetaldehyde dehydrogenase involved in the catabolism of 2-phenylethylamine in Escherichia coli has been cloned, sequenced, and located at 31.0 min on the chromosome. The deduced PadA polypeptide contains 499 amino acid residues with a predicted molecular mass of 53.7 kDa, and its primary structure reveals significant similarity with that of members of the aldehyde dehydrogenase superfamily. By engineering optimal transcription and translation elements, a high expression of the padA gene has been achieved. The active PadA enzyme is a homodimer that prefers NAD+ over NADP+ as coenzyme. The enzyme efficiently oxidizes only phenylacetaldehyde-like aromatic aldehydes, and has a weak esterase activity with p-nitrophenol. The padA gene constitutes a new catabolic tool for designing DNA cassettes to expand the abilities of microorganisms to degrade toxic aromatic compounds.

Aldehyde Oxidoreductases↗

Composite resin inlays: a study of marginal adaptation.

Ten mesio-occlusodistal composite resin inlays were fabricated by the indirect method and cemented in extracted posterior teeth. The restorations were subjected to thermocycling and subsequently placed in a 50% silver nitrate solution. Specimens were prepared and inspected under a stereoscopic microscope and a scanning electron microscope. The results showed a total absence of microleakage at the margins terminating in enamel and a slight degree of microleakage in only two margins terminating in cementum. This corresponded with the general absence of defects and gaps that was observed in the scanning electron microscope. The exception was in the two specimens that exhibited dye penetration; these were found to have material in the marginal areas.

Bicuspid↗

P450XXI (steroid 21-hydroxylase) gene deletions are not found in family studies of congenital adrenal hyperplasia.

Congenital adrenal hyperplasia (CAH) is a common genetic disorder due to defective 21-hydroxylation of steroid hormones. The human P450XXIA2 gene encodes cytochrome P450c21 [steroid 21-monooxygenase (steroid 21-hydroxylase), EC 1.14.99.10], which mediates 21-hydroxylation. The P450XXIA2 gene may be distinguished from the duplicated P450XXIA1 pseudogene by cleavage with the restriction endonuclease Taq I, with the XXIA2 gene characterized by a 3.7-kilobase (kb) fragment and the XXIA1 pseudogene characterized by a 3.2-kb fragment. Restriction endonuclease mapping by several laboratories has suggested that deletion of the P450XXIA2 gene occurs in about 25% of patients with CAH, as their genomic DNA lacks detectable 3.7-kb Taq I fragments. We have cloned human P450c21 cDNA and used it to study genomic DNA prepared from 51 persons in 10 families, each of which includes 2 or more persons with CAH. After Taq I digestion, apparent deletions are seen in 7 of the 20 alleles of the probands; using EcoRI, apparent deletions are seen in 9 of the 20 alleles. However, the apparently deleted alleles seen with Taq I do not coincide with those seen with EcoRI. Furthermore, studies with Bgl II, EcoRI, Kpn I, and Xba I yield normal patterns with at least two enzymes in all cases. Since all probands yielded normal patterns with at least two of the five enzymes used, we conclude that the P450XXIA2 gene "deletions" widely reported in CAH patients probably represent gene conversions, unequal crossovers, or polymorphisms rather than simple gene deletions.

Adrenal Hyperplasia, Congenital↗

Pubertal growth in patients with androgen insensitivity: indirect evidence for the importance of estrogens in pubertal growth of girls.

Spontaneous pubertal growth was studied in eight patients with the syndrome of androgen insensitivity to obtain information on the growth-promoting action of estrogens. In one additional patient (who had a gonadectomy before puberty), the effect of exogenous estrogens was studied. Mean age at peak height velocity (12.7 years) was closer to that in normal girls than to that in normal boys. Mean peak height velocity (7.4 cm/yr) was as in normal girls (7.3 cm/yr), but was lower than in normal boys (9.3 cm/yr). Bone age corresponded better to male standards. Mean adult height (172.3 cm) was lower than in normal men (-0.6 SD), but higher than in normal women (+1.4 SD). In the patient who had a gonadectomy, estrogen replacement caused a higher peak height velocity (12 cm/yr), but lower adult height (160.5 cm) than in the patients with intact gonads who received no treatments. We conclude that in normal girls, the pubertal growth spurt also results from the action of estrogens rather than of adrenal androgens. To ensure normal pubertal growth, physiologic estrogen replacement in hypogonadal females should be started at a bone age of about 11 years, and should not be delayed in the hope of achieving a greater mature height.

Adolescent↗

A note on modulations and structuring of locomotion in children and adults.

The aim of this study was to investigate the modulations of locomotion induced by a rhythmic cognitive task (counting one's steps). Subjects (6- and 8-year-olds and adults) were requested to walk freely, and then to walk while counting their steps. Here a decrease in cadence values was observed in children only, with quasi-total repercussions on velocity at the age of 6 only. The spatiotemporal structuring of locomotion described here is already present at 6 years of age and is not altered in the step-counting situation: strong links were observed between cadence and velocity, and between stride length and velocity, and weak links between cadence and stride length.

Journal Article↗

[Hypothyroidism caused by isolated TSH deficiency].

Hypothyroidism secondary to isolated thyrotropin (TSH) deficiency was confirmed by low TSH levels during TRF test. Other anterior pituitary hormones were normal. Replacement therapy with thyroxine improved the child's growth but not the intellectual ability. The diagnostic difficulties and the importance of early treatment are emphasised.

Child↗

Testosterone treatment of excessively tall boys.

Twenty-nine tall boys with a mean height prediction of 198 cm were treated for serious psychosocial reasons with high doses of a long-acting testosterone preparation (500 mg/m2/month). Their ages at the start of treatment ranged from 9.8 to 16.9 years, and the mean duration of treatment was 1.2 years. Bone age was assessed according to the Tanner-Whitehouse II (RUS) method, and height predictions were calculated using the age-specific regression equations of Tanner and colleagues. On the basis of bone age at the start of treatment, three groups were formed (bone age 12.1 to 14, 14.1 to 15, and greater than 15 years), and the results were assessed separately. In the whole series, adult height was reduced by 5.4 cm; the best results (8cm) were achieved in the youngest bone age group. Under treatment, bone maturation was accelerated (1.8 years per year) and growth velocity increased (youngest bone age group) or was normal (older bone age groups). Testicular volume remained prepubertal in young patients and decreased in older ones. After discontinuation of treatment, testicular volume and sperm count became normal again after a mean period of 1.5 years, but in a few cases recovery was slower. It is concluded that adult height in tall boys may be effectively reduced by testosterone, that the results are best if treatment is started in early puberty, and that the suppressing effects on pituitary and testicular function are fully reversible. Since the indication for treatment is a psychosocial one, the patients should be carefully selected, taking into account not only growth but also psychological and familial factors.

Adolescent↗

Estrogen treatment of excessively tall girls.

40 excessively tall girls with a mean height prediction of 182 cm were treated for serious psychosocial reasons continuously with ethinylestradiol 0.3 mg daily and with norethisterone 10 mg daily for 5-7 days every 4th week. Their age at the start of treatment ranged from 9.4 to 13.8 years, and the mean duration of treatment was 1.7 years. Bone age was assessed according to the TANNER-WHITEHOUSE-II (RUS) method, and height predictions were calculated using the age-specific regression equations of TANNER et al. Results were compared with those in 9 untreated control cases. Mean reduction of predicted height in all patients was 4.6 +/- 2.4 cm, and the most marked reduction was found in the youngest group of patients. However, even in patients after menarche and with a bone age above 14 years at start of treatment, there was still a considerable reduction of adult height. Bone maturation was accelerated by treatment (1.4 years per year), and the most marked acceleration was observed during the first 6 months (1.7 years per year). Growth velocity was normal or slightly subnormal during the first 6 months and very low thereafter. During treatment, there was a considerable weight gain which was, in part, lost again when treatment was discontinued. Posttherapeutic amenorrhea was infrequent, and normal and regular menstruations reappeared in the majority of the patients about 1 month after discontinuation. It is concluded that estrogen treatment in high doses is effective in reducing adult stature in girls and that somatic and psychological side effects are minimal when treatment is started after onset of spontaneous puberty. Although early treatment tends to give better results, beneficial results may still be obtained in older patients.

Adolescent↗

[Evolution of mortality amongst the youth of Navarra: 1985-1995].

Recent publications show that mortality rates amongst young people and adolescents in some industrialised countries have increased in recent years. The addition of new diseases such as AIDS, which principally affect the young population, to those inevitable deaths brought about through causes such as traffic accidents has increased interest in this public health problem. The number of deaths and the adjusted global mortality rates amongst men and women of 15-34 years did not increase in Navarra in the 1985-1995 period. These are situated around 70 per 100,000. Changes have taken place in the pattern of causes, similar to those observed in other industrialised areas, with an increase of deaths through overdose and AIDS, and a decline in mortality due to traffic accidents in recent years. Traffic accidents were the first cause of death amongst youths until 1993. From this year onwards deaths from AIDS became the first cause amongst women, while amongst men the number of deaths from AIDS is equal to those caused by traffic accidents.

English Abstract↗

[The effect of 2 bleaching agents on the enamel surface. An in-vitro study].

We present a study "in vitro" of the effect of bleaching agents on dental surfaces using the "Walking bleaching technique". We found that hydrogen peroxide bleached more quickly than carbamide although, after a period of six weeks, the results were the same as far as whitening was concerned. In the scanning electron microscope we observed significantly different changes in each case. Carbamide caused a regular and uniform opening of the enamel prisms of the surface while hydrogen peroxide produced more severe superficial destruction with the appearance of patterning similar to the acid etching, and the presence of some crystalline areas emerging from the body of the prisms.

Carbamide Peroxide↗