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El síndrome de Bloch-Sulzberger o incontinencia pigmentaria es una genodermatosis infrecuente que afecta a los tejidos derivados del neuroectodermo. Esta patología herencia dominante, tiene penetrancia 100% y se encuentra ligada al cromosoma X. En pacientes género masculino suele ser letal, por lo la mayor parte casos reportados son mujeres diagnosticadas durante primera infancia. cuanto cuadro...

We report here a rare case of incontinentia pigmenti (IP) in a 10-year-old girl who developed Behçet's disease. IP was diagnosed in infancy and Behçet's disease was diagnosed at 10 years of age. The initial presentations of Behçet's disease were spiking fever and recurrent painful oral and genital ulcers that were refractory to antibiotics. After corticosteroid treatment, her fever subsided and...

REFERENCES ] Kubota Y, Toyoda E Kubota H. Face mask fitting for edentulous patients. Anesth Analg 1993; 76: 450. 2 McGee IP II, Vender IS. Nonintubation management of the airway. In: Benumof JL (Ed.). Clinical Procedures in Anesthesia and Intensive Care. Philadelphia: JB Lippineott Co., 1992; 89-114. 3 Fisher JA, Ananthanarayan C, Eddist G. Role of the laryngeal mask in airway management. Can J...

INTRODUCTION Incontinentia pigmenti or Bloch-Sulzberger syndrome is a rare X-linked dominant disease that mainly affects the skin, eyes, hair, central nervous system and teeth. The disease is predominant among women. Although dermatologic manifestations are among the most important aspects for the diagnosis of the syndrome, they are less damaging to the patient and do not require treatment. How...

Incontinentia Pigmenti is an uncommon X-linked genodermatosis, caused by mutations in the NEMO gene. It is a systemic disease that involves tissue of ectodermic and mesodermic origin, including cutaneous tissue, teeth, eyes and the central nervous system, amongst other organs. The Authors report a rare case of Incontinentia Pigmenti in a female newborn.

Incontinentia pigmenti is associated with various anomalies in 80% of cases. Among the most important are the ocular abnormalities and more particularly a retrolental mass with detachment of a dysplastic retina. At the basis of this manifestation are retinal vascular changes, characterised at first by ectatic tortuous veins and arteriovenous anastomoses as well as by aneurysmal-like dilatations.

A case of incontinentia pigmenti is reported with fundus changes in 1 eye. She had microaneurysms temporal to the macula, with an abnormal branch of inferior temporal vein. There was extensive retinitis proliferans in the upper temporal equatorial region, which showed leakage on fluorescein angiography.

This document describes a framework for IP Flow Information Export (IPFIX) Mediation. This framework extends the IPFIX reference model specified in RFC 5470 by defining the IPFIX Mediator components.