L'incidence annuelle du syndrome de Guillain-Barre est de 1,5/100000 habitants La mortalite actuelle est estimee a environ 5 % d'apres des essais therapeutiques recents, bien conduits Dix pour cent des malades gardent des sequelles motrices tres invalidantes un an apres le debut des premiers signes neurologiques La prise en charge de ces malades necessite des equipes entrainees, multidisciplinaires, pouvant pratiquer l'ensemble des therapeutiques specifiques La corticotherapie per os'ou par voie intraveineuse est inefficace Les echanges plasmatiques sont le premier traitement dont l'efficacite a ete demontree par rapport a un groupe controle Les indications sont maintenant mieux connues Les formes benignes (marche possible) beneficient de 2 echanges plasmatiques; 2 echanges supplementaires sont realises en cas d'aggravation Dans les formes intermediaires (marche impossible) et les formes severes (recours a la ventilation mecanique), 4 echanges plasmatiques sont conseilles Il n'est pas utile d'augmenter leur nombre dans les formes severes ou en cas d'absence d'amelioration De fortes doses d'immunoglobulines donnees par voie intraveineuse (lq IV) [0,4 g/kg/j pendant 5 jours] sont aussi efficaces que les echanges plasmatiques dans les formes intermediaires et severes Dans ces formes, le choix entre Ig IV et echanges plasmatiques depend des contre-indications respectives de ces traitements et de leur faisabilite Les travaux en cours ont comme objectif de mieux preciser les indications respectives des echanges plasmatiques et des lq IV dans des formes de gravite differente, leur morbidite comparee, la dose optimale des lq IV

[Guillain-Barré syndrome].

Background The clinically appropriate range for oxygen saturation in preterm infants is unknown. Previous studies have shown that infants had reduced rates of retinopathy of prematurity when lower targets of oxygen saturation were used. Methods In three international randomized, controlled trials, we evaluated the effects of targeting an oxygen saturation of 85 to 89%, as compared with a range of 91 to 95%, on disability-free survival at 2 years in infants born before 28 weeks' gestation. Halfway through the trials, the oximeter-calibration algorithm was revised. Recruitment was stopped early when an interim analysis showed an increased rate of death at 36 weeks in the group with a lower oxygen saturation. We analyzed pooled data from patients and now report hospital-discharge outcomes. Results A total of 2448 infants were recruited. Among the 1187 infants whose treatment used the revised oximeter-calibration algorithm, the rate of death was significantly higher in the lower-target group than in the highe...

https://espace.library.uq.edu.au/view/UQ:319530/UQ319530_OA.pdf

Oxygen Saturation and Outcomes in Preterm Infants

Amblyopia and binocular vision.

PURPOSE. Pediatric glaucoma is a rare, potentially blinding condition, yet, in the United Kingdom, there is a paucity of contemporary epidemiologic and clinical data regarding this condition. The British Infantile and Childhood Glaucoma (BIG) Eye Study is the first national population-based study conducted to examine the incidence, detection patterns, current management, and intraocular pressure (IOP) control at 1 year in children with newly diagnosed glaucoma in the United Kingdom. METHODS. A prospective study was conducted wherein children in the United Kingdom and Republic of Ireland aged 16 years with newly diagnosed primary or secondary glaucoma, were identified by consultant ophthalmologists through active surveillance from December 2001 until November 2002. Eligible cases were re-evaluated 12 months after notification. RESULTS. Of the 99 eligible children with newly diagnosed glaucoma, 47 had primary and 52 secondary glaucoma. The annual incidence of diagnosis of primary congenital glaucoma (PCG) in Great Britain was 5.41 in 100,000 (1/18,500) live births and in the Republic of Ireland, 3.31 in 100,000 (1/ 30,200). The incidence of PCG in children of Pakistani origin was almost nine times that of Caucasians. IOP control of 21 mm Hg was achieved in 94% with medications (60% without medications) in cases of PCG and in 86% with medications (28% without medications) in cases of secondary glaucoma. CONCLUSIONS. The British annual incidence of PCG diagnosis is comparable to that reported for other similar populations. Ethnic minorities from South Asia are at significantly increased risk of PCG. Successful IOP control in PCG after surgery in Britain is comparable to that in the published literature. (Invest Ophthalmol Vis Sci. 2007;48:4100‐4106) DOI:10.1167/

The British Infantile and Childhood Glaucoma (BIG) Eye Study

http://academic.oup.com/milmed/article-pdf/132/2/162/24232928/milmed-132-2-162a.pdf

The Metabolic Basis of Inherited Disease

Sect.1 Normal Development: Embryology Postnatal Development * Sect.2 Practical Aspects of Pediatric Ophthalmology: Pediatric Eye Exam Pediatric Low-Vision Patient Breaking the News Pediatric Eye Trauma * Sect 3 Strabismus and Amblyopia: Anatomy and Physiology of the Extraocular Muscles Laws of Ocular Motility and Introduction to Strabismus Visual Development, Amblyopia and Sensory Adaptations Introduction to Strabismus and the Ocular Motor Exam Sensory Examination Esotropia Exotropia Oblique Overaction and A- and V-Patterns Vertical Strabismus Strabismus Syndromes Optical Pearls and Pitfalls: Tricks and Traps in Strabismus * Sect 4 Lids, Orbit and Lacrima: Lid Malformation Malposition and Lesions Lacrimal System Protopsis and Orbital Disease * Sect 5 Anterior Segment Disease: Conjunctivitis Tumors of the Conjunctiva Dysgenesis of the Anterior Segment Corneal Lesions Pediatric Iris Abnormalities Pediatric Lens Abnormalities * Sect 6 Glaucoma and Uveitis: Pediatric Glaucoma Pediatric Uveitis * Sect 7 Pediatric Retinal Disease: Heritable Disease of RPE, Bruch's Membrane and Choriocapillaris Retinitis Pigmentosa and Associated.

Pediatric Ophthalmology and Strabismus

Pediatric Eye Examination.- Anatomy and Physiology of Eye Movements.- Binocular Vision and Introduction to Strabismus.- Visual Development and Amblyopia.- The Ocular Motor Examination.- Sensory Aspects of Strabismus.- Esodeviations.- Exotropia.- Alphabet Patterns and Oblique Muscle Dysfunctions.- Complex Strabismus: Restriction, Paresis, Dissociated Strabismus, and Torticollis.- Strabismus Surgery.- Ocular Motility Disorders.- Optical Pearls and Pitfalls.

Handbook of pediatric strabismus and amblyopia

The purposes of this investigation were to determine: (a) if visual evoked potential (VEP) abnormalities could be identified in children with neurofibromatosis type 1 (NF1) with no evidence of optic pathway or brain neoplasias on MRI; and (b) if VEP abnormalities could be explained by the presence of hyperintense T2-weighted foci on MRI testing, known as unidentified bright objects (UBOs). To answer these questions, VEPs were recorded from 16 children with NF1 and compared to 13 normal subjects in the same age range tested with the same protocol. Pattern-reversal VEPs were recorded at four stimulus sizes both monocularly and binocularly, the latter to hemi-field stimuli. Flash VEPs were recorded in dark- and light-adapted conditions. VEP measurements and MRI readings for UBOs were conducted in a masked fashion. Ten of the 16 children with NF1 had abnormal VEPs to at least one of the four types of stimuli. Abnormalities included delayed responses (n=6), absent flash VEP P2 component (n=3), or both (n=1). Abnormalities of the P2 component of the dark-adapted flash VEP were the most common finding (n=7), although no single testing strategy was able to identify all children with abnormal VEPs. UBOs were present in all children, demonstrating that their presence does not fully account for VEP abnormalities in children with NF1. This study also demonstrates that VEP abnormalities are present also in the absence of neoplasias of the optic pathways or of the brain. Our results are suggestive of a primary abnormality of visual processing in children with NF1.

Visual evoked potentials in children with neurofibromatosis type 1.

Pediatric Visual Electrophysiology.- The Pediatric Low-Vision Patient.- Breaking the News: The Role of the Physician.- Heritable Disorders of RPE, Bruch's Membrane, and the Choriocapillaris.- Retinitis Pigmentosa and Associated Disorders.- Disorders of the Vitreous and Vitreoretinal Interface.- Retinal Vascular Disorders.- Nonvascular Hamartomas.- Retinoblastoma and Other Malignant Intraocular Tumors.- Retinopathy of Prematurity.- Infectious, Inflammatory, and Toxic Diseases of the Retina and Vitreous.- Myopia.- Patterns of Retinal Disease in Children.

Peter H. Spiegel

Papers

Pediatric Ophthalmology and Strabismus

Handbook of pediatric strabismus and amblyopia

Visual evoked potentials in children with neurofibromatosis type 1.

Handbook of pediatric retinal disease

Oftalmología pediátrica y estrabismo