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Introduction La pemphigoide de la grossesse (PG) place le clinicien en situation difficile, en raison du risque, non previsible, de complications gravidiques (CG) associees. Ces CG surviennent, selon la litterature, avec une frequence de 35 % qui justifie la recherche de marqueurs predictifs. L’objectif de ce travail etait d’etudier la relation entre les CG et le taux d’AC anti-BPAG2, outil diagnostique couramment utilise en pratique clinique. Materiel et methodes Cette etude retrospective multicentrique, menee entre 2009 et 2020, a inclus les PG avec AC anti-BPAG2 positifs au diagnostic (kit ELISA Euroimmun). Les PG du post-partum et les patientes avec dossier obstetrical introuvable ou mal renseigne ont ete exclues. Resultats Cent dix PG survenues au 1er (7 %), 2e (48 %) ou 3e (45 %) trimestre de la grossesse chez 107 patientes, d’âge moyen 33 ± 6 ans ont ete inclues. Des lesions bulleuses etaient presentes dans 83/110, 76 % des cas ( 50 bulles/j : 4 %). Quarante-deux PG sur 110 (38 %) se sont compliquees de : retard de croissance intra uterin (RCIU) n = 18, prematurite n = 26, hypotrophie fœtale n = 17. La survenue de ces CG n’etait pas associee aux comorbidites (diabete, HTA, infection) ni au traitement par corticoides systemiques mais au jeune âge gestationnel (p = 0,004) et a la presence de bulles, independamment de leur nombre (p = 0,015). Le taux d’AC anti-BPAG2 en tant que variable continue etait associe a la presence de bulles (p = 0,018) mais pas aux CG, considerees dans leur ensemble. Apres identification du taux d’AC anti-BPAG2 > 150 UI comme le plus discriminant (courbe ROC), l’analyse uni- et multivariee montrait que ce taux constituait un facteur de risque de RCIU (p = 0,016 ; OR 5,25) avec une valeur predictive positive de 30 % et une valeur predictive negative de 91 %. Discussion Cette etude represente, a notre connaissance, la plus grande serie de la litterature. Nos resultats sont concordants avec les donnees precedemment publiees et confirment notamment la relation entre : CG et presence de bulles ou jeune âge gestationnel, independamment des comorbidites et de la corticotherapie systemique. Les resultats observes ont une implication directe pour le clinicien dans sa pratique et sont etayes par un rationnel physiopathologique, en lien avec la presence placentaire de BPAG2. Conclusion Un taux d’AC anti-BPAG2 > 150 UI constitue un facteur de risque de RCIU et justifie une surveillance adaptee des patientes.

Introduction Les patients arrivent parfois en consultation d’allergologie, certains de l’allergene « coupable » de leurs symptomes. Le clinicien doit etre attentif au discours du patient mais aussi rester systematique. Methodes Un manutentionnaire de 48 ans, sans antecedent notable, consultait pour un eczema severe du visage et des mains, rythme par le travail, qu’il attribuait aux produits issus de la petrochimie qui etaient utilises dans son entreprise. Un eczema du visage, de la nuque et des faces dorsales des mains etait note a l’examen clinique. L’etude du poste de travail de ce cariste mettait en evidence la manipulation de blocs de polymeres bleus et blancs et de palettes avec des gants de manutention. Resultats Une exploration allergologique comprenant une batterie standard, les ajouts du GERDA, caoutchoucs et methacrylates, des tests semi-ouverts avec des morceaux de polymere, et les 2 faces d’un gant etait realisee : les tests positifs a 72 H etaient le chrome, la colophane et la face externe du gant usage. Apres reprise de l’interrogatoire, une allergie aux poussieres de bois de palette etait evoquee chez ce patient manipulant les palettes avec ses gants de manutention. Une seconde exploration allergologique avec des tests semi-ouverts a un gant non usage et aux poussieres de bois etait realisee : seul le test aux poussieres de bois humidifiees etait positif. L’analyse de ces poussieres par la CARSAT confirmait la presence de colophane. Le diagnostic de dermite aeroportee et manuportee a la colophane contenue dans les poussieres de bois etait retenu. Une franche amelioration etait constatee la reaffectation du salarie dans un autre secteur. Discussion L’utilisation de la colophane, contenue dans les bois de pin ou sapin, est tres repandue pour ces proprietes collante, emulsifiante, et decapante. La prevalence de l’eczema de contact a la colophane est estimee entre 0,7 et 3 % selon les etudes. Cet allergene est parfois retrouve dans des professions inattendues du fait de son utilisation ubiquitaire. Conclusion Ce cas illustre l’importance d’un raisonnement systematique dans l’exploration des dermatoses professionnelles.

Resume La dehiscence d’un canal semicirculaire (CSC) est un syndrome congenital affectant principalement le canal superieur et, moins frequemment, le CSC posterieur au sein de l’os temporal. Ce syndrome a d’abord ete decrit par Minor et al. en 1998. La prevalence de l’anomalie est d’environ 0,5 % dans la population generale. Le symptome le plus caracteristique est le phenomene de Tullio, associant en particulier vertiges et nystagmus. La surdite (le plus souvent de transmission) complete volontiers l’ensemble du tableau. La dehiscence peut aussi etre totalement asymptomatique et represente alors un incidentalome sur des examens radiologiques. L’evaluation ORL inclut l’audiometrie et les potentiels evoques vestibulaires myogeniques (PEVM). Les symptomes cliniques s’expliquent par la creation d’une troisieme fenetre au sein de l’oreille interne. Le diagnostic est confirme par la tomodensitometrie du rocher en haute resolution qui utilise les reconstructions multiplanaires (MPR) dans le plan du canal (plan de Poschl pour le CSC superieur) et les reconstructions en trois dimensions (3D). L’IRM est moins sensible au depistage de cette entite. Le principal diagnostic differentiel est l’otospongiose. Cette correlation clinicoradiologique a des applications therapeutiques, meme si la chirurgie n’est indiquee qu’en cas de symptomes vestibulaires invalidants.

Introduction Les antiseptiques locaux largement utilises en medecine pour la desinfection cutanee peuvent donner des eczemas de contact. Ces reactions localisees ne menent malheureusement pas toujours a des tests allergologiques. Methodes Un patient de 85 ans, etiquete allergique a la Betadine® dermique (povidone iodee), avait realise une desinfection par Biseptine® (chlorhexidine, alcool benzylique, chlorure de benzalkonium) avant un geste chirurgical. Lors de l’intervention, il etait constate un eczema diffus, aux zones d’application de l’antiseptique. Une batterie standard europeenne, les ajouts du GERDA, des patchs tests a la Betadine®, a la Biseptine® et a la chlorhexidine etaient realises. Les lectures a 48 h revelaient : Fragance mix I : ++, Hydroperoxyde de linalol a 1 % : ++, baume du Perou : +++, alcool benzylique a 10 % : +++, chlorure de Benzalkonium : + Biseptine® : + Betadine® : + Les patchs tests etaient negatifs pour la chlorhexidine. Le diagnostic d’allergie de contact a l’alcool benzylique et au chlorure de benzalkonium, contenus dans la Biseptine®, etait confirme. La chlorhexidine seule pouvait etre utilisee chez lui et devait etre son antiseptique de reference. Discussion Les eczemas de contact aux antiseptiques sont courants mais probablement sous-estimes. Ils concernent plus frequemment le principe actif (chlorexidine) mais les allergies aux excipients sont possibles d’ou l’importance de realiser des tests epicutanes. A. Barbaud [1] rapportait dans une etude observationnelle menee sur deux ans parmi les patients ayant presente une allergie de contact aux antiseptiques : 27 % d’allergie a l’hexamidine, 22 % au Septivon®, 19 % a la chlorhexidine, 19 % aux antiseptiques a base de povidone iodee, 4 % aux mercuriels, entre autres. La sensibilisation concernait l’excipient dans 30 % des cas, les molecules antiseptiques dans 70 % des cas. Conclusion Ce cas illustre l’importance de realiser des tests epicutanes : ils ont permis chez notre patient polysensibilise de trouver un antiseptique de reference.

Until recently, Bardet-Biedl syndrome was considered as a classic autosomal recessive condition. The disorder is defined by the association of the following clinical features: retinitis pigmentosa, polydactyly, obesity, hypogonadism, and possible mental retardation. This syndrome leads to multiple handicaps (visual impairment, complications of obesity, kidney failure, endocrine dysfunction). This condition, apparently clearly defined from a clinical point of view, appears to be genetically heterogenous. To date, six different genes have been identified: BBS1, BBS2, BBS4, BBS6, BBS7 and BBS8. Interestingly, this condition has recently been linked to a failure of cellular ciliogenesis. Moreover, this disorder is characterized by an additional degree of complexity, as it is the first example of triallelic inheritance described in human beings. However, this new finding appears to be less frequent than expected in this syndrome.

Summary. Despite the introduction of platelet additive solutions for the preparation of pooled platelet components, only a few studies of limited scope have evaluated the clinical efficacy of platelets stored in these solutions. The current report presents an analysis of data to evaluate the response to the transfusion of pooled buffy-coat components suspended in storage solution with reduced (35%) plasma content in comparison with 100% plasma products. During the euroSPRITE clinical trial of platelet components treated with a pathogen inactivation process, control treatment group platelet components were prepared in 100% allogeneic donor plasma (plasma control) or in platelet additive solution (T-Sol™) mixed with plasma (T-Sol control). Control group thrombocytopenic patients received either plasma control or T-Sol control platelet components. One-hour and 24-h platelet count increments (CIs) and corrected count increments (CCIs) were analysed for these two types of preparation. In addition, haemostatic assessments were conducted for each transfusion. One-hour and 24-h mean platelet CIs and post-transfusion haemostatic scores were not significantly different for patients receiving platelet components suspended in 100% plasma and T-Sol plasma mixtures. Pooled buffy-coat platelet components prepared in reduced plasma content mixtures provided therapeutic platelet CIs with effective haemostasis.

RESUME La demographie decroissante des ophtalmologistes va provoquer un changement a court terme du mode d’exercice de la profession. Les besoins de soins — glaucome, diabete, degenerescence maculaire liee a l’âge — ne sont deja pas suffisamment assures, pas plus que le depistage des troubles visuels de l’enfant et cette deficience va s’aggraver dans l’avenir du fait de l’allongement de la duree de la vie. Pour pallier ces difficultes une partie des actes techniques doit etre transferee aux orthoptistes, collaborateurs naturels des ophtalmologistes, sous la responsabilite de ceux-ci. L’Academie nationale de medecine recommande, outre l’augmentation du nombre d’ophtalmologistes en formation et l’accroissement de celui des orthoptistes, la collaboration etroite entre ces deux professions et la creation de reseaux de soins.

We report on the magnetic properties of 3 nm cobalt clusters embedded in gold matrix using magnetic investigations. From the analysis of the mFC, mZFC and m(H) curves, we determine precisely the Co magnetic diameter Probability Density Function (PDF) in highly diluted samples. We show that even with a 3% vol. concentrations of magnetic particles, the interactions between particles must necessarily be taken into account. For this purpose, we first determine the individual properties of particles in highly diluted samples (1%vol.). In a second step, we increase the concentration in order to retrieve the magnetic interactions signature. Exchange like interactions can be modeled by the formation of magnetic dimers, thus modifying the PDF.

Acknowledgments: This study was supported by a grant from the Belgian Red Cross. The authors thank Prof. W. Duquet for valuable help with the statistical analysis of the results. Abstract: We investigated correlations between soluble HLA-DR (sHLA-DR) molecules and several clinical, biological and genetic parameters associated with rheumatoid arthritis (RA) disease activity. Serum sHLA-DR concentrations were determined in 146 samples from 89 RA patients by an ELISA format, using an antibody combination of mouse and rat monoclonal anti-human HLA-DR antibodies. The mean sHLA-DR serum level in RA patients was significantly increased with 277±19 ng/ml compared to 142±13 ng/ml of 80 healthy controls (P

Our objective was to create a system for the supply of platelet concentrates (PCs) based on leukocyte-filtered PCs suspended in a storage medium (PAS-II) including: (1) pooled buffy-coat-derived (BC) PCs and (2) "split" apheresis PCs. The same standards were intended for the two preparations with regard to composition and blood cell counts. In preliminary studies, similar in vitro data were found for leukocyte-filtered and non-filtered reference BC PCs. Slightly inferior in vitro results than for BC PCs restricted the shelf-life of apheresis PCs to 5 days compared to 7 days for BC PCs. With the present platelet supply system in use as a routine service for one year, the experience was very satisfactory, meeting the demands for PCs very efficiently and resulting in a low out-dating rate (5%).

The true incidence of bedside transfusion errors, i.e. those happening when blood products have left the blood bank, is underestimated because published figures rely on reporting of clinically relevant events or on indirect methods. The SAnGUIS project assessing blood practice in a prospective and randomized fashion for 6 elective surgical procedures gave the opportunity to trace all transfused units and to identify steps at risk during blood delivery in surgery. We considered transfusion of a wrong unit as a major error and poor execution or documentation as a recording error. Over 15 months, 808 patients out of 1,448 were transfused with 3,485 units. A total of 165 errors were found after blood products had left the blood banks. Seven were misidentifications (0.74% of patients, 0.2% of units). Eight other major errors occurred in 4 (0.5%) patients. Major errors occurred during nonemergency situations, in wards or intensive care units. The remaining ('recording') 150 errors consisted of misrecordings (61), mislabellings (6), or failures to document transfusions in the medical records (83). All errors were uneventful except one misidentification which induced a transient, yet unreported, reaction. The 'descending' inquiry method used for this study showed that most errors pass unnoticed and are therefore not reported. Measurement of error rates may constitute an important quality indicator. Retrospective information of this survey to the concerned staff people provided an impetus to take adequate measures to reduce these bedside errors.

The polymorphic variable number of tandem repeats in the 5′ upstream region of the human insulin gene is a well-known non-human leukocyte antigen locus contributing to genetic susceptibility to IDDM. Controversy exists about the question as to whether INS susceptibility haplotypes are or are not preferentially inherited together with HLA-DR4 haplotypes. We investigated whether genetic interaction between INS and the HLA complex can be better defined using DQ genotypic and phenotypic markers in addition to DR serology. The 5′ INS 1/1 genotype was positively associated with IDDM both in non-DR4 subjects (relative risk = 4.3; 95% confidence interval, 1.6–11.5) and DR4 subjects (relative risk = 4.2; 95% confidence interval, 1.9–9.0). Further subdivision of IDDM patients and matched control subjects according to HLA-DQA1 and HLA-DQB1 genotype or phenotype also failed to show any association between 5′ INS and HLA class II genes in diabetic patients. The 5′ INS and HLA class II polymorphisms therefore provide independent risk markers, which may both contribute to the genetic screening of a high-risk population among nondiabetic individuals.

Background and Objectives The INTERCEPT Blood System for Platelets utilizes amotosalen–HCl (S-59) in combination with ultraviolet A (UVA) light to inactivate viruses, bacteria, protozoa and leucocytes that may contaminate platelet concentrates (PCs). To facilitate implementation of this technique into routine blood bank manufacturing procedures, this study evaluated the impact of different time settings of photochemical treatment on in vitro platelet function. Materials and Methods Platelets derived from apheresis (6·5–7·0 × 1011 platelets) were resuspended in 240 ml of autologous plasma and 360 ml of platelet additive solution (PAS III) and split into two equal-sized PC units. Whereas one unit was not treated, the other was treated with 150 µm amotosalen and 3 J/cm2 UVA light followed by a compound adsorption device (CAD) step for reduction of residual amotosalen and photoproducts. In a first series of experiments (arm A, n = 7), PC units were photochemically treated after an overnight storage period of 16–23 h followed by a CAD step of 4 h. In a second series (arm B, n = 8), photochemical treatment occurred after a short storage time of 4 h with a subsequent CAD step of 16 h. Platelet function was evaluated by assaying blood gas analysis, glucose and lactate concentration, lactate dehydrogenase (LDH), hypotonic shock response (HSR) and the expression of CD62p, over a period of 7 days. Results Neither of the photochemical treatment procedures showed differences for pH, pCO2, pO2, HCO3, glucose consumption or platelet activation until the end of day 7. Increased lactate values detected for the treated units of arm A at the end of the storage period were independent from the PCT time setting. Conclusions Photochemical pathogen inactivation with different initial resting periods between 4 and 23 h, and different CAD steps of 4 and 16 h, had no influence on the platelet in vitro function during 7 days of storage.

The analysis of the FOXL2 gene (3q23) in a series of two families and two sporadic cases affected with Blepharophimosis-Ptosis-Epicanthus Inversus Syndrome (BPES) is presented. This study detected two novel FOXL2 mutations (missence and nonsens mutations) and confirmed the recurrence of a previously described duplication. Magnetic Resonance Imaging (MRI) of the orbit, in one family, showed absence or hypotrophy of the eyelid superior levator muscle suggesting a possible role of FOXL2 in the development of this extra-ocular muscle.

Deferoxamine is a specific chelating agent of trivalent anions: iron ion and aluminum ion. The main prescriptions for this treatment are primary non-curable by blood letting hemochromatosis, secondary hemosiderosis, and aluminum intoxication associated with chronic kidney failure. Since the early 1980s, ocular toxicity has been documented in several publications.We recorded three clinical observations of patients presenting symptoms of an ocular toxicity caused by deferoxamine. The prescription of this treatment related to the presence of secondary hemosiderosis (a case of primitive myelofibrosis and a case of chronic myelomonocytic leukemia treated by blood transfusion) and an aluminum intoxication affecting a patient with chronic kidney failure. All three patients presented a gradual loss of visual acuity. The following were predominantly observed at the fundus examination which showed pigmentary anomalies near the macula such as mottling and dispersion affecting the electrophysiological studies. The termination of the treatment did not result in an improvement in the symptomatology.Considering the latest literature on the subject, the indications as well as the pharmaceutical properties of deferoxamine, the ophthalmological symptoms of this intoxication, the additional investigations and the anatomicopathological analyses are restated, together with the current pathogenical hypothesis.Deferoxamine can cause ocular toxicity resulting in severe and permanent lesions of the retinal pigment epithelium. The occurrence of disorders of the fundus and visual acuity requires, before and during the treatment, regular ophthalmological monitoring combined with electrophysiological explorations. This allows early treatment of the hematological or kidney disorder.

Following the eclipse of 11 August 1999, the ophthalmological clinic of Strasbourg cared for 4 patients who suffered from sun damage. The multifocal electroretinogram (ERG) carried out on our first patient enabled us to not only confirm the existence of a residual maculopathy as shown by the static visual field, but also, and above all to quantify the foveolar deficit. Moreover, with the other 3 patients, the multifocal ERG enabled us to detect a foveolar deficit completely overlooked by the other usual tests. In light of our clinical study, we can assert that the multifocal ERG has yet to be equaled by any other test for analysis of macular function.

Helinx™ technology uses the psoralen S-59 and ultraviolet A (UVA) light to inactivate a broad spectrum of bacteria, viruses, and leukocytes in platelet concentrates (PC). In a paired study we investigated the effect of the Helinx™ technology on platelet function over a storage period of 5 days. Double-dose apheresis PC (7.0 x 1011 platelets) resuspended in 35% plasma/65% platelet additive solution (PAS III) were collected (n = 8) and split into two equal size PC. One PC was not treated (Control), the other PC was treated with 150 µM S-59 and 3 Joules/cm2 UVA on Day 1 (Test).

We investigated correlations between soluble HLA-DR (sHLA-DR) molecules and several clinical, biological and genetic parameters associated with rheumatoid arthritis (RA) disease activity. Serum sHLA-DR concentrations were determined in 146 samples from 89 RA patients by an ELISA format, using an antibody combination of mouse and rat monoclonal anti-human HLA-DR antibodies. The mean sHLA-DR serum level in RA patients was significantly increased with 277+/-19 ng/ml compared to 142+/-13 ng/ml of 80 healthy controls (P0.001). In ascending order of significance, correlations were found between serum sHLA-DR and EULAR swelling and pain scores, Waaler-Rose, RA factor, ESR and CRP (P=0.025 to P0.001). High sHLA-DR levels were defined above 374 ng/ml that was the 95% confidence interval of the controls. Thirty-seven blood samples (25%) in 31 RA patients were above this level. The EULAR pain and swelling scores, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and RA factor were higher (P=0.044 to P0.001) at the moment of high sHLA-DR concentrations, compared to the lower concentrations. Higher disease activity was further found in groups of RA patients respectively heterozygous or homozygous for the disease-associated epitope (Q)R/KRAA within the HLA-DRB1 chain, compared to the group without this epitope (P0.017 for part of the results). Likewise, sHLA-DR was respectively 169+/-17 (no disease associated epitope), 324+/-34 (heterozygous) and 442+/-69 ng/ml (homozygous for the disease-associated epitope on HLA-DRB1 alleles) (P0.017). In conclusion, this study shows significant correlations between serum sHLA-DR levels and RA disease activity parameters, as well as increased sHLA-DR in patients with disease-associated epitope on HLA-DRB1 alleles.

Opto-ciliary shunts are compensatory bypass channels from the retinal venous system to the choroidal veins in case of long standing central retinal vein occlusion. Indocyanine green angiography with the Scanning Laser Ophtalmoscope may be helpful to differentiate acquired optociliary shunts from retinal or choroidal arteriovenous malformations.

We relate the case of a 15 years old girl who suffered a sudden and severe bilateral lowering of the eyesight, on the occasion of a dermatomyositis. The evolution, over a year, showed a remission of the systematic appearances but there was a limited recovery of the eyesight, the diffuse retinal lesions are the sign of a more extensive vascular problem, found in case of paediatric dermatomyositis.

Foetal blood sampling was performed at 35 weeks of gestation due to abnormal foetal ultrasound findings. There was apparent monosomy 21 (45,XX,-21) in all mitoses analyzed. The infant died at 37 weeks during delivery. Examination disclosed facial anomalies, clubfeet, hypoplasia of the left urogenital tract, agenesis of corpus callosum, ventricular dilatation, and heterotopias. Reevaluation of the karyotype showed an unbalanced translocation t(1;21) (q44;q22.11) which resulted from a maternal balanced translocation. These findings were confirmed by fluorescence in situ hybridization and molecular studies with chromosome 21 specific markers. The latter showed a proximal deletion of the maternally derived chromosome 21 including all loci from centromere down to the D21S210 locus. This case illustrates the need for complementary cytogenetic and molecular investigations in cases of apparent monosomy 21.

SANGUIS was multicentric European study involving more than 7,000 patients in 43 teaching hospitals during a one year period. The goal of the study was to describe current transfusion practice for elective surgery in adult. The present paper summarizes the data collected on the 1,193 patients enrolled in Belgium. It also introduces the final report of the SANGUIS study in Belgium, which will be published as a special issue of the Acta Chirurgica Belgica.

Four cases of inadvertent intracapsular lens delivery secondary to zonular lysis occurring during planned manual extracapsular cataract extraction were reported. Theoretical and practical considerations were presented based on these observations and literature review. These considerations would suggest relaxing incisions when capsulorhexis and planned extracapsular cataract extraction are associated.

The true incidence of bedside transfusion errors, i.e. those happening when blood products have left the blood bank, is underestimated because published figures rely on reporting of clinically relevant events or on indirect methods. The SAnGUIS project assessing blood practice in a prospective and randomized fashion for 6 elective surgical procedures gave the opportunity to trace all transfused units and to identify steps at risk during blood delivery in surgery. We considered transfusion of a wrong unit as a major error and poor execution or documentation as a recording error. Over 15 months, 808 patients out of 1,448 were transfused with 3,485 units. A total of 165 errors were found after blood products had left the blood banks. Seven were misidentifications (0.74% of patients, 0.2% of units). Eight other major errors occurred in 4 (0.5%) patients. Major errors occurred during nonemergency situations, in wards or intensive care units. The remaining ('recording') 150 errors consisted of misrecordings (61), mislabellings (6), or failures to document transfusions in the medical records (83). All errors were uneventful except one misidentification which induced a transient, yet unreported, reaction. The 'descending' inquiry method used for this study showed that most errors pass unnoticed and are therefore not reported. Measurement of error rates may constitute an important quality indicator. Retrospective information of this survey to the concerned staff people provided an impetus to take adequate measures to reduce these bedside errors.

A case of a stellate hemorrhage of the macula occurring in a young man is reported. Analysis of this rare clinical entity allowed us to link it to combined occlusion of the central retinal vein and cilioretinal artery. With regard to this case and to a review of the literature, clinical and angiofluorographic features of this syndrome are recalled. The usually good prognosis of this disease was not observed in our case. Finally, the controversial pathophysiological data are discussed.

After reviewing the various etio-pathogenic hypotheses for pterygium in which the discontinuity of the lacrymal film at the corneo-limbic junction appears to play an essential role, the authors discuss the current therapeutic choices. The value and place of lamellar keratoplasty overlapping the limbus are discussed.

The authors report a case of simultaneously occurring neurofibroma and schwannoma of the sciatic nerve and discuss the complementary aspects of MR and US. The schwannoma was well-defined and showed distal enhancement on sonographic evaluation, whereas the neurofibroma was ill-defined; both tumors were hypoechoic. T1- and T2-weighted MR images revealed similar signal characteristics of the two tumors, but intense enhancement following administration of gadolinium-DTPA distinguished the schwannoma from the neurofibroma.

An unusual myopathy with extensive myofibrillar degeneration related to the Z disk is reported. Focal Z disk disappearance, accumulation of numerous rods and cytoplasmic bodies and some rimmed vacuoles represented the major pathologic features of this sporadic muscular disorder, sharing common clinical and electrophysiological features with inclusion body myositis. This diagnosis was excluded because all nuclei appeared normal and 15-18 nm filaments were not observed in cytoplasm or in the vicinity of membranous whorls. Intramuscular innervation was studied by vital staining with methylene blue: the terminal innervation ratio was normal, indicating a myopathic disorder. The relationship between the present disorder and cytoplasmic bodies and nemaline myopathies is discussed.

Moebius syndrome is unusual and associates facial diplegia with a failure of lateral movements, and seems to be of nuclear or supranuclear origin, appearing during foetal life. Association with somatic malformations are frequent such as those of Poland syndrome. Surgery's envisaged when palsy of lateral movements is associated with deviation of the eyes.

A 63 year old woman developed progressive shortness of breath, pulmonary hypertension, and respiratory failure and died from pulmonary fibrosis 45 years after thoracic fistulography with Thorotrast. Bouts of acute respiratory failure occurred with features of noncardiogenic pulmonary oedema. Lung tissue obtained by biopsy and at necropsy showed abundant radioactive particles of thorium dioxide in the lungs. The particles were congregated in the walls of blood vessels and in perivascular fibrous zones, consistent with a causal role of Thorotrast in the development of lung fibrosis. It is suggested that the fibrosis was due to the combined effects of alpha radiation on the interstitial perivascular zones and of recurrent pulmonary oedema due to endothelial damage.

The authors report 4 cases of recurrent vitreous hemorrhage related to retinal breaks without detachment, bridged by a retinal vessel. Clinical and evolutive features of this type of breaks are emphasized according to a review of available literature. Fluoroscopic evaluation of vascular permeability appears as a promising prognostic tool. Management should be directed to the prevention of both retinal detachment and vitreous hemorrhage recurrence. Cryoapplication and scleral buckling is far more effective than photocoagulation.

A patient undergoing hemodialysis was treated intravenously with desferrioxamine (3 g) for an aluminum encephalopathy. He presented a sudden xanthopsia with visual loss. Fundus examination showed diffuse macular irregular pigmentary disturbances. We discuss current knowledge and possible pathogenesis of these observations published since 1983. Awareness of the side effects of desferrioxamine implies an ophthalmologic follow up similar as chloroquine.