Ictiosis congénita: manifestaciones oftalmológicas / Congenital ichthyosis: ophthalmological manifestations

Objetivos: Describir las manifestaciones oftalmológicas de la ictiosis congénita. Materiales y Métodos: Estudio retrospectivo de 8 pacientes con ictiosis lamelar congénita atendidos en el servicio de oftalmología del Hospital de Pediatría Prof. Dr. Juan P. Garrahan. Se analizaron: edad, sexo, posicionamiento de los párpados, la presencia de lagoftalmos, alteraciones de la superficie ocular y tratamientos tópicos y quirúrgicos instaurados para la patología ocular. Resultados: Se estudiaron 16 ojos de 8 pacientes, 7 de sexo masculino y 1 de sexo femenino. Cinco pacientes presentaron ictiosis lamelar clásica y 2 eritrodermia congénita ictiosiforme (EIC). La edad media de los pacientes fue de 30,6 meses (r= 15 días y 108 meses). Los hallazgos oftalmológicos observados fueron: queratitis y lagoftalmos en el 50% de los pacientes, ectropión en el 37,5% y absceso corneal en ambos ojos de 1 paciente. Todos los pacientes fueron tratados de primera instancia con lubricantes, 2 se trataron con eritromicina tópica ante la presencia de secreción y sólo 1 paciente requirió tratamiento con colirio fortificado y colocación de membrana amniótica. Conclusión: la ictiosis congénita produce alteraciones en los párpados y en la superficie corneal con alto riesgo de secuelas y pérdida visual. Los controles periódicos son indispensables para poder prevenir las lesiones y sus complicaciones (AU)

Aim: To describe the ophthalmological manifestations of congenital ichthyosis. Material and Methods: A retrospective study of eight patients with congenital lamellar ichthyosis was conducted at the Department of Ophthalmology of the Pediatric Hospital Prof. Dr. Juan P. Garrahan. Age, sex, eyelid position, presence of lagophthalmos, ocular surface alterations, as well as topical and surgical eye treatment were analyzed. Results: 16 eyes of 8 patients, 7 male and 1 female, were studied. Five patients had classical lamellar ichthyosis and two had congenital ichthyosiform erythroderma (CIE). Mean age of the patients was 30.6 months (r, 15 days to 108 months). Ophthalmological findings were: keratitis and lagophthalmos in 50% of patients, ectropion in 37.5%, and corneal abscess in both eyes in one patient. All patients were initialy treated with artificial tears, two were treated with topical erythromycin because of secretion, and only one patient needed treatment with fortified eye drops and placement of amniotic membrane. Conclusion: Congenital ichthyosis causes disorders of the eyelids and corneal damage with a high risk of sequelae and vision loss. Periodic controls are necessary to prevent the lesions and their complications (AU)

Different patterns of renal osteodystrophy in Iberoamerica.

The various forms of renal osteodystrophy are predominant hyperparathyroid bone disease, mixed uremic osteodystrophy, low turnover osteomalacia, and adynamic bone disease. The present study analyses a total number of 1,209 bone biopsies from 5 different countries (Brazil, Uruguay, Argentina, Portugal, and Spain). Low turnover osteomalacia and mixed uremic osteodystrophy were more common in Brazil, Uruguay, and Argentina than in Portugal and Spain whereas predominant hyperparathyroid bone disease was seen more often in Portugal and Spain. In all centers, independent of the aluminum staining technique used, the extent of aluminum deposited in bone was greater in patients presenting with low bone turnover, whether from low turnover osteomalacia or adynamic bone disease, than in the predominant hyperparathyroid bone disease. In summary, even though recent reports have indicated that, over the last decade, the incidence of aluminum-induced toxicity was reduced, aluminum still seems to be implicated in a great percentage of symptomatic low bone remodelling lesions in Iberoamerica.

Response to melatonin in brain areas implicated in the sexual cycle of the rat.

In the sexual cycle control of the female rats, the amygdala, hypothalamus and septal area, nervous structures of the limbic system as well as the posterior cortex (latero-occipital), play an important role. The pineal gland is closely related to the gonadal function via its hormone melatonin. Based on this theory, we have studied the effect of melatonin in female rats on oxidative metabolism during the estrus and diestrus phases of the sexual cycle of the aforementioned structures and of the anterior cortex (latero-frontal), which is considered to be a control tissue of sexual activity, and also on the ovary. It was observed that melatonin administered by intraperitoneal injection or produced by the body itself by stimulation of the pineal gland due to blindness, produces an inhibiting effect on the mentioned nervous structures which is also manifest in the ovary, decreasing oxidative metabolism.

Effects of ocular enucleation on the oxidative metabolism of CNS structures and ovarium in female rats.

In this paper we have studied oxygen uptake of the amygdala, hypothalamus, septal area, anterior cortex (latero-frontal), posterior cortex (latero-occipital) and ovarium in female rats in the oestrous and dioestrous phases with occular enculeation. Our results point to a statistically significant difference in the oestrous phase of the posterior cortex and ovarium levels, comparing control rats with the enucleated ones. In the dioestrous phase, the modifications that we have encountered are at the amygdala and ovarium. We point out the possible participation of the pineal gland in the control of the sexual cycle in in enucleated rats.