Síndrome amelogénesis imperfecta-nefrocalcinosis: revisión bibliográfica / Amelogenesis imperfect-nephrocalcinosis syndrome: literature review

El Síndrome Amelogénesis Imperfecta-Nefrocalcinosis es una rara enfermedad caracterizada por presentar Amelogénesis Imperfecta (AI) tipo hipoplásico en su mayoría asociado a una enfermedad renal llamada: Nefrocalcinosis.La AI es una alteración de la estructura y apariencia del esmalte con un origen genético que afecta en su mayoría a todos los dientes. Puede presentarse aislada o asociada a síndromes. Se distinguen 3 tipos (hipoplásico, hipocalcificado o hipomadurado) y entre ellos existen 15 subtipos, basado en las manifestaciones clínicas y el modo en que se transmite. Entre los genes descritos están: AMELX, ENAM, FAM83H, KLK4 y MMP20. La nefrocalcinosis (NC) es una enfermedad comúnmente caracterizada por la precipitación de sales de calcio en el tejido renal. En los pacientes con este síndrome, el daño en la función renal es variable y puede demorarse hasta la adultez a pesar de la presencia típica de hiperecogenicidad en la niñez. La relación entre los defectos de esmalte y la NC aun es incierta, pudiendo ser de tipo medular o cortical, siendo la primera la más común. El objetivo de esta revisión bibliográfica es detallar las características y tipos de ambas entidades, así como describir los casos publicados en la literatura. La mayoría de los reportes que se hacen cumplen un patrón autosómico recesivo, generalmente por matrimonios consanguíneos, donde se presenta la NC de tipo medular y la AI es de tipo hipoplásico en su mayoría, que se caracteriza por ser un defecto cuantitativo de esmalte, donde clínicamente se observa ausencia del mismo...

Amelogenesis Imperfecta -Nephrocalcinosis Syndrome is a rare disease characterized by the presence of Amelogenesis Imperfecta (AI) mostly hypoplastic type associated with a kidney disease called: Nephrocalcinosis. The AI is an alteration of the structure and appearance of the enanel with a genetic origin that mostly affects all the teeth. There may be isolated or associated with syndromes. There are 3 types (hypoplastic, hypocalcification or hypomineralised) and among them, there are 15 subtypes based on clinical manifestations and the mode how it is transmitted. Among the genes described are: AMELX, ENAM, FAM83H, KLK4 and MMP20. Nephrocalcinosis (NC) is a disease commonly characterized by the precipitation of calcium salts in the renal tissue. In patients with this syndrome, impaired renal function is variable and may be delayed until adulthood despite the presence of typical hyperechogenicity in childhood. The relationship between enamel defects and NC is still uncertain and may be cortical or medullary type, being the most common the first one. The aim of this review is to describe the characteristics and types of both entities, and to describe the cases published in the literature. Most reports have an autosomal recessive pattern, usually by consanguineous marriages, which it is presents mostly the NC medullary type and hypoplastic type of AI, which is characterized like a quantitative defect of enamel, where clinically observed absence of enamel...

Calidad diagnóstica en pacientes con lesiones premalignas y malignas de cuello uterino: Ambulatorio Palo Negro, Estado Aragua. 2003-2005 / Diagnostic quality of cytology exams in patients with premalignant and malignant lesions of the uterine cervix: Consults in the Palo Negro, State Aragua. Outpatient clinic from 2003 through 2005

Con el propósito de estimar la calidad diagnóstica de las citologías de pacientes con lesiones premalignas y malignas de cuello uterino que acudieron a la consulta de patología de cuello Uterino en el Ambulatorio de Palo Negro durante el período 2003-2005, se realizó un estudio de concordancia, para lo cual se revisaron 60 reportes citológicos e histológicos; obteniendo así el porcentaje de concordancia citohistológico en una primera observación. Posteriormente se seleccionaron los casos no concordantes, siendo sometidas a una segunda revisión por dos especialistas del área, calculándose la concordancia intra e inter observador aplicando el índice de Kappa. El 85% de las muestras citológicas evaluadas fueron satisfactorias, con un 7,84% asociadas a inflamatorio severo. El 53.92% de anormalidades de las células escamosas estuvo representado por el LIE de Bajo Grado y un 39% por NIC I; la concordancia citohistológica para una primera revisión fue de 41,7% correspondiendo 54,76% a los NIC I, con un índice de Kappa de -0.0746, el cual es considerado como un índice pobre de concordancia e igualmente en la segunda observación predomino una fuerza de concordancia pobre a débil siendo necesario implementar medidas correctivas que mejoren los índices de concordancia citohistológica, para lograr diagnósticos citológicos mas confiables, y con ellos mejorar la calidad diagnóstica de las citologías.

With the objective of estimating the accuracy of diagnostic in smears perform on patients with premalignant and malignant Uterine Cervix injuries in the ambulatory of Palo Negro, during the period of 2003-2005, an agreement study was performed based on the review of 60 smear and histological reports; obtaining the percentage of cytohistological agreements on a first observation. Afterwards, all non-agree cases were selected and were submitted to a second review by two specialists in the field, calculating the agreement intra and inter observer by applying the Kappa index. 85% of the evaluated smear samples were satisfactory, with 7,84% been associated to severe inflammation. 53,9% of squamous cell anomalies was represented by a low level LIE and 39% by the NIC I. The cytohistological agreement for a first revision was 41,7% corresponding 54,76% to the NIC I, with a Kappa index of -0.0746, wich is considered a poor agreement index. Likewise, a poor and weak agreement predominated in a second observation as it was necessary to implement corrective measures to improve the index of cytohistological agreements to obtain more reliable and more accurate diagnostics, hence improving the quality of smear diagnostic.

The effect of interval from day of administration of bovine somatotropin (bST) to synchronization of ovulation and timed-insemination on conception rate of dairy cows with and without ovarian cysts.

The objective of this study is to evaluate the effect of the interval from the day of administration of bovine somatotropin (bST) to the day of initiation of synchronization of ovulation (Day 0 and 7) and timed-insemination (TAI) on conception rate (CR) of dairy cows with and without ovarian cysts, respectively. Lactating dairy cows (n = 359) were divided into two groups. Cows in Group 1 (n = 238, without ovarian cysts) were treated with 100 microg, i.m. GnRH on Day 0; 25 mg, i.m. PGF2a on Day 8. 100 mirog. i.m. GnRH on Day 10; and inseminated 16 h later without detection of estrus. Cows in Group 2 (n = 121, with ovarian cysts) were treated with 100 microg, i.m. GnRH on Day 0; 100 microg, i.m. GnRH on Day 7; 25 mg, i.m. PGF2alpha on Day 14, 100 ug, i.m. GnRH on Day 16; and inseminated 16 h later without detection of estrus. Between 60 and 63 days postpartum, all cows in the herd were given bST every 14 days for the duration of the study. Logistic regression was used to assess the risk of nonpregnancy associated with interval from bST treatment to Day 0 for cows without ovarian cysts. and both Day 0 and 7 for cows with ovarian cysts adjusting for parity and days in milk. The CR for cows in Group 1 was significantly higher when the interval from last treatment with bST to Day 0 was between 1 and 3 days (28%) compared to 4-6 days (14%). In addition, the risk of nonpregnancy was 2.19 times greater in cows 4-6 days after bST treatment compared to 1-3 days after adjusting for parity and days in milk. The CR for cows in Group 2 was not significantly different when the interval from last treatment with bST to both Day 0 and 7 was between 1 and 3, 4 and 6, and 7 and 14 days. In conclusion, the results of this study suggested bST treatment closer to Day 0 had a positive effect on CR of cows without ovarian cysts, but bST treatment closer to both Day 0 and 7 had no effect on CR of cows with ovarian cysts. This was interpreted to mean that bST had a beneficial effect on either, or both, the preovulatory follicle and the oocyte in dairy cows without ovarian cysts, but not in dairy cows with ovarian cysts.

[Colombians in Venezuela: myth and reality]. / Los Colombianos en Venezuela: mito y realidad.

PIP: Figures of Colombian immigrants in Venezuela in the period 1981-1986 as well as the development of their socio-demographic profile and their integration into the labor market are analyzed by means of ad hoc tables drawn from a Sample Home Survey by the Oficina Central de Estadisticas e Informatica and information from the civil record office. It is estimated that in 1987 there are only half a million Colombians in Venezuela and, contrary to what is generally assumed, not only has the afflux decreased (3/4 of the Colombians entered before 1981), but also a return migration can be observed. Colombian immigration is not homogeneous, but is made up of different groups as far as their professional skills and occupations are concerned. The number of Colombians engaged in household service and those living in rural areas has decreased, but not the number of active farm workers. A greater proportion of Colombians engaged in trade and sales activities is observed, but artisans and skilled labor are still the majority.^ieng

Effect of low oral doses of disopyramide and amiodarone on ventricular and atrial arrhythmias of chagasic patients with advanced myocardial damage.

Low-dose (7 mg/kg per day) disopyramide administration to arrhythmic chagasic patients decreased the frequency of ventricular extrasystoles in 4 of 17 patients (24%) and suppressed most complex ventricular arrhythmias in 12 of 15 patients (80%). This assessment was made from 72-h continuous Holter monitoring recorded during the course of this double blind, placebo-controlled randomized crossover study. Seven patients (41%) complained of anticholinergic side effects, but no contractile or conduction system depression was seen. Amiodarone (200 mg) given on a single blind, placebo-controlled basis to 9 of these patients reduced the frequency of ventricular extrasystoles in 6 of 9 patients (67%) and suppressed complex ventricular ectopy in 6 of 7 patients (85%). One patient was unable to tolerate this drug (11%). Both drugs seemed less effective in controlling supraventricular arrhythmias, although disopyramide eliminated paroxysms of supraventricular tachycardia in 9 of 13 (69%) and amiodarone in all 6 patients with this arrhythmia. Amiodarone appears to be a better antiarrhythmic drug for chagasic patients, due to its greater effectiveness and lower incidence of side effects.