An unusual case of neonatal cholestasis.

Septo-optic dysplasia (SOD), otherwise called De Morsier syndrome, is a developmental anomaly of mid-line brain structures and includes optic nerve hypoplasia, absence of the septum pellucidum and hypothalamo-pituitary abnormalities). In literature an association between optic nerve hypoplasia and neonatal cholestasis is described. We report the case of a female infant with persistent cholestasis, low weight gain and onset of nystagmus that appeared at one month and a half of life. Ophthalmology evaluation showed left optic nerve hypoplasia. MRI scan of the brain demonstrated a thin left optic nerve, an ectoptic posterior pituitary gland, no visible infundibulum and lack of septum pellucidum. Endocrinological investigation showed GH and ACTH deficiency. We discuss about diagnosis and pathogenesis of De Morsier syndrome with a brief review of the literature.

Efficacy and safety of continuous intravenous infusion of remifentanil in preterm infants undergoing laser therapy in retinopathy of prematurity: clinical experience.

BACKGROUND: Preterm infants often require surgery. As experimental evidence suggests that premature infants may experience pain and this could even result in fatal complications, the anaesthesiologist must face problems related to lowbirth weight, high risk of hypothermia, concomitant pulmonary disease and metabolic and receptor immaturity. Recently remifentanil has been considered an optimal analgesic drug in a preterm infant undergoing mechanical ventilation and frequent surgical manoeuvres, but no clinical studies have been reported in the literature. The aim of our study was to evaluate the efficacy of a continuous intravenous infusion of remifentanil in premature infants undergoing laser therapy for retinopathy of prematurity (ROP). METHODS: Six premature infants with ROP were scheduled for laser therapy. The procedure was performed in the neonatal intensive care unit. Transcutaneous carbon dioxide, pulse oximetry, respiratory rate, ECG and noninvasive blood pressure were continuously monitored. Infusion of remifentanil started with a dose of 0.75-1 microg x kg-1x min-1, 1 h before surgery. A midazolam bolus dose (0.20 mg x kg-1) was administered and the remifentanil infusion was increased to 3-5 microg x kg-1x min-1 taking into account haemodynamic and respiratory changes or spontaneous movements. RESULTS: Increased dosage was necessary only for 10 min during the procedure. No changes in temperature and ventilatory settings were observed and after 2 h from the surgical procedure the preterm infants were back to their preoperative status. CONCLUSIONS: A continuous infusion of remifentanil allowed optimal control of surgical stress and a return to preoperative status and ventilatory settings without side-effects.

Patterns of expression of cell wall related genes in sugarcane

A busca por genes relacionados ao metabolismo da parede celular no banco de dados SUCEST-FAPESP resultou em 459 potenciais genes ("clusters") que correspondem a 3283 clones ("reads"), o que corresponde a cerca de 1.1 por cento do número total de clones. Foram construídos mapas de superfície para correlacionar os genes com as bibliotecas dos diferentes tecidos. Foram encontradas correlações positivas ou neutras entre os genes em uma mesma biblioteca. Os genes relacionados à síntese de celulose (família CesA) foram os de maior expressäo na planta, embora a maior expressäo esteja associada à raiz e colmo. Entre as hidrolases, ß-1,3-glucanases, xiloglucano endo-ß-transglicosilase, ß-glucosidase e endo-ß-mananase foram os genes com o maior número de clones. Análise de correlaçäo (por mapas de superfície) revelou que a expressäo dos genes relacionados à biossíntese parece estar associada à hidrólise de hemicelulose, enquanto as hidrolases de pectina estäo relacionadas principalmente às hidrolases de xiloglucano. O padräo de expressäo de genes relacionados à parede celular, baseado no número de "reads" por "cluster" refletiu bem as características fisiológicas esperadas para cada tecido. Este é o primeiro trabalho que fornece uma visäo geral do metabolismo de parede celular através da expressäo dos genes em vários tecidos ao mesmo tempo. Por exemplo, inflorescências em desenvolvimento se comportaram de forma semelhante a tecidos meristemáticos e à regiäo de transiçäo folha-raiz. Estes dados serviräo tanto para pesquisas em fisiologia e desenvolvimento de cana, como também para o desenvolvimento de processos industriais.

Scleral buckling surgery in stage 4 retinopathy of prematurity.

BACKGROUND: Technical advances in neonatal intensive care have significantly increased the number of very low birth-weight babies that survive the perinatal period. Some of these infants develop severe retinopathy of prematurity that may lead to retinal detachment. METHODS: Between November 1988 and January 1994, 28 eyes from 15 preterm babies underwent scleral buckling for stage 4 retinopathy of prematurity at a mean age of 4.2 months. Cryotherapy was performed preoperatively on 12 eyes and intraoperatively in the remaining 16 eyes. The mean follow-up period was 35 months. RESULTS: Scleral buckling produced retinal reattachment in 13 eyes (46.4%). Severe myopia (-5 D to -15 D) was found in all 13 of these eyes, 12 also presented convergent strabismus. Mean visual acuity, measured in 6 eyes from children over the age of 3 years was 20/40. In 7/28 eyes of the younger children of this group we found a fix and follow the light capability. No light perception was detected in 11/28 eyes; in the remaining 4/28 eyes there was only light perception. Scleral buckling failed to prevent the progression to stage 5 in 15 eyes (53.6%). Additional surgery was excluded for 9 of these eyes based on ultrasonography findings; the other 6 eyes underwent vitrectomy, which led to macular reattachment in 4 cases. CONCLUSIONS: Clinical experience shows that scleral buckling is not always capable of preventing progression of the disease to stage 5. Furthermore, even when the anatomic results of this procedure are good, the functional outcome is often complicated by severe visual impairment.

Identification of the preferential gaze position through elevation of visual fatigue in a selected group of VDU operators. A preliminary study.

A selected group of VDU workers were tested in an attempt to achieve objective evidence of visual fatigue and to identify a preferential monitor/gaze position during a particular work setting. Each subject was scored in a two-phase test. The highest mean scores were achieved when the monitor was lowered with respect to the primary/gaze position; the scores obtained with the monitor in primary or upward gaze position were significantly lower (p < 0.001). The downward gaze position should be considered more comfortable for both innervational and anatomical factors, as stated in previous studies of ocular mobility. A reduction of vertical saccades could also play a role in lessening visual fatigue in this gaze position.