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Mucormycosis is an unusual systemic infection caused by multicellular fungi. Despite, few reports have been documented world widely, incidences of mucormycosis are recently increasing due to several predisposing factors such as diabetes, COVID-19 pandemic, and over prescription of corticosteroids. The clinical symptoms of this disease are often unfamiliar and vague. To the best of the authors’ knowledge, this is the first report that describes a case of clinical human mucormycosis during the COVID-19 pandemic in Libya. This report discusses also the risk factors and steps that can be applied to prevent occurrence of this disease. The patient is a 58-year-old Libyan female who presented with a history of COVID-19 infection, diabetes mellitus, hypertension, hypothyroidism, obesity, obstructive sleep apnea, and stable ischemic heart disease. She was complaining of loss of vision of the right eye, redness, and swelling all over the right face with scattered areas of blackish discoloration involving the inner third of the right eye and right face. Clinical examination showed bilateral basal and mid-lung fields fine crepitation, abdominal distension, severe chemosis, proptosis, restriction of all eye movement, aniscoria, upper lid ptosis and loss of corneal sensation of right eye. Computerized tomography (CT-Scan) of brain, para-nasal sinus, and orbit revealed right periorbital cellulitis, right eye proptosis, right ethmoidal, maxillary, sphenoidal sinusitis, and right cavernous sinus thrombosis without bone erosion. The histopathological report showed numerous broad branching aseptate hyphae and spores with acute cellulitis indicating an invasive fungal infection (mucormycosis). Mucormycosis is a rare fatal disease. Physician awareness and early management may significantly reduce mucormycosis severity.

Brucellosis in human is an uncommon disease in Libya and usually diagnosed by serological assays. The clinical symptoms of this disease are none specific which makes differential diagnosis difficult.The patient was a sixteen years old girl, lives in a rural area. She was suffering from intermittent fever for several days associated with chills, mild cough, and headache. Comprehensive physical examination revealed the patient was generally unwell, pallor and, had abdominal tenderness. Laboratory investigations revealed that the patient had pancytopenia and a marked increase in C-reactive protein, procalcitonin, lactate dehydrogenase, aspartate transaminase. Provisional diagnosis was acute leukemia or aplastic anemia. Slow growth of Gram-negative coccobacilli in aerobic blood culture was detected. It could not clearly be identified by phoenix bacterial identification system. It was provisionally misdiagnosed Haemophilus spp; while finally it was identified Brucella spp. Brucellosis may manifest in a delicate manner which makes its diagnosis very challenging. It has the ability to affect various systems in the body, displaying a confusion in differential diagnosis, and delay in medical care, thus raising the risk of complications. Unusual medical laboratory findings should not be overlooked as they could help in bringing up the right clinical diagnosis.

Although breast cancer and endometrial cancer are two frequent female cancers, finding synchronous primary cancers in the same patient is a comparatively uncommon occurrence. We present the case of a Libyan woman who developed synchronous breast cancer, endometrial cancer, and small lymphocytic lymphoma. For the previous six months, a 49-year-old female patient had a right breast mass. An ultrasound scan revealed an uneven doubtful growth in the right breast as well as swollen of the axillary lymph nodes. After a wide local excision, histopathology revealed that the patient had invasive ductal carcinoma of the breast with a positive resection margin, and he was admitted to the Surgery Department. No distal metastasis was seen on a computed tomography (CT) scan of the chest, abdomen, or pelvis, so the patient had a right mastectomy and axillary clearance. Residual invasive ductal carcinoma was found on histopathology and immunohistochemistry with positivity for the estrogen receptor and the progesterone receptor. Small lymphocytic lymphoma (SLL) affected the axillary lymph nodes, affirmed by immunohistochemical staining positive for CD20, CD5, CD23 and BCL-2 while negative for CD3 and Cyclin D1. Resection margins were free. Second cancers are characterized by being linked to SCL, and some researchers have described that the risk of second cancers is elevated in SCL patients. We represent a combined case of synchronous primary SCL with breast cancer and endometrial cancer in a woman which is a rare occurrence.

The terrible triad of the elbow (TTE) is a lesion associating a dislocation of the elbow, a fracture of the radial head, and a fracture of the coronoid process, with a high potential of complication. The treatment is based on the restoration of bone lesions and external capsular ligament repair. The systematic repair of the medial collateral ligament (MCL) is still debated in the literature. The aim of this study is to evaluate the clinical and functional results of the surgical treatment in a real-world series from Easter Morocco.This was a retrospective study of 6 cases of TTE operated by isolated external approach or combined approach (internal or anterior) of the elbow in the department of Traumatology-Orthopedics of the Mohammed VI University Hospital (Oujda Morocco), over a period of 7 years from 2013 to 2020. Radial head and coronoid process fractures were classified according to the Mason and Morrey-Regan classifications, respectively. The following clinical parameters were evaluated: Mayo Clinic Elbow Performance Score (MEPS), Quick Disabilities of the Arm, Shoulder and Hand (DASH), Visual Analog Scale (VAS), and arc of mobility in flexion-extension and prono-supination.6 male patients treated between May 2013 and December 2020 were included. The median follow-up was 48 months. All patients had frontal and lateral standard X-ray of over and under joints, and computed tomography (CT)-scan was delivered for 5 cases. The elbow dislocation was posterolateral in five cases, and posteromedial in only one patient. Radial head fractures were classified as type I in one case, type II in two cases, and type III in three cases. Coronoid fractures were type I in three cases, type II in one case, and type III in two cases. At the last follow up, the mean MEPS, Quick DASH, and VAS was 81, 28, 0.8, respectively. The mean arc of mobility in flexion was 120° and it was deficient by 20° in extension. In addition, the mean arc of mobility in protonation was 80°, while in supination it was 75°. Regarding complications, we noticed an instability of the elbow on valgus in a single case, elbow stiffness with heterotopic ossification in a single case, ulnar nerve damage in a single case during medial collateral ligament anchoring, and elbow hygroma in one single case.The surgical management of TTE can provide good and long-term functional results after restoration of the stabilization of bone structures and the lateral ligament complex, without the need to repair the medial collateral ligament.

Second-order rate constants (k1) for the σ-complexation of 4-nitrobenzofurazan 1 with four 4-X-substituted phenoxide anions 2a–2d (X = OCH3, CH3, H and Cl) were measured in aqueous solution at 20 °C. Using this series of phenoxide anions as a reference, the electrophilicity parameter (E) of this electrophile 1 has been evaluated according to Mayr’s approach. With the E value of –9.42, Mayr’s equation was found to correctly predict the rate constants for the reactions of 1 with hydroxide ion in H2O and a 1:1 ratio of H2O to CH3CN. However, the large βnuc value of 1.12 obtained in the present work is clearly consistent with a single electron transfer (SET) mechanism.

The nucleophilic addition reactions of N1-methyl-4-nitro-2,1,3-benzoselenadiazolium tetrafluoroborate 1 with aliphatic amines 2a–c (diethylamine 2a, dipropylamine 2b, and allylamine 2c) have been kinetically studied by UV–vis spectroscopy in acetonitrile solution at 20°C. The kinetic data have been analyzed, using the Mayr equation, allowing the quantification of the electrophilicity parameter (E) value of benzoselenadiazolium cation 1 (E = −14.72). The reliability of parameter E has been reasonably verified by comparison of calculated and experimental second-order rate constants for the reactions of cation 1 with other amines 2d–f (pyrrolidine 2d, piperidine 2e, and morpholine 2f) under the same conditions as those of the amines 2a–c. A linear Bronsted plot (R2 = 0.9945) with a βnuc value of 0.55 has been obtained for the reactions of 1 with the secondary amines employed in the present work. Interestingly, satisfactory correlation between the log values of measured and calculated rate constants with a slope very close to unity has been obtained and discussed.

The kinetics of the coupling of N1-methyl-4-nitro-2,1,3 benzothiadiazolium tetrafluoroborate 1 with a series of 4-X-substituted anilines 2a–f (X = OH, OMe, Me, H, Cl, and CN) have been investigated in acetonitrile at 20°C. The second-order rate constants result in a nonlinear Bronsted-type plot. The Hammett plot is also nonlinear, whereas the Yukawa–Tsuno plot exhibits an excellent linear correlation with ρ = –1.62 and r = 1.44. The large Bronsted (βnuc = 1.24) and Hammett (ρ = –5.16) values suggest that the reactions proceed trough a single electron transfer mechanism. The finding of satisfactory correlation between the log k1 of the reactions and the oxidation potentials (E°) of anilines 2a–d supports this mechanism. On the other hand, electrophilicity parameter E of benzothiadiazolium cation 1 as defined by the correlation log k20°C = s(E + N) has been determined and compared with the electrophilic reactivities of a large variety of electrophiles.

Le syndrome d’Allgrove ou triple A syndrome est un syndrome hereditaire rare, associant une alacrymie, une achalasie et une maladie d’Addison. Des signes neurologiques peuvent s’y associer. Recemment, des mutations du AAAS, un gene candidat localise sur le chromosome 12q13 ont ete identifiees. L’observation d’une famille dont les sept enfants avaient une alacrymie congenitale entrant dans le cadre du syndrome d’Allgrove est presentee ici. Quatre d’entre eux ont ete operes pour achalasie. Cinq presentaient des troubles neurologiques comme l’atteinte des nerfs crâniens, le syndrome pyramidal, le syndrome neurogene peripherique et une sclerose laterale amyotrophique. Deux enfants avaient les signes d’Addison. La physiopathologie, ainsi que les caracteristiques cliniques et genetiques de ce syndrome ont ete envisagees. Le syndrome d’Allgrove doit etre evoque devant toute alacrymie de l’enfant ou du sujet jeune.

Introduction La survenue de recidive de la retinochoroidite toxoplasmique est un des principaux problemes dans la prise en charge de la toxoplasmose oculaire. Par consequent, plusieurs etudes ont ete menees dans le but de determiner le meilleur protocole therapeutique permettant de pallier a ce probleme. Cette etude a pour objectif de demontrer l’innocuite et l’efficacite du protocole clindamycine-corticoide et d’evaluer le taux de recurrence avec ce traitement. Patients et methodes Cette etude portait sur 21 cas presentant une retinochoroidite toxoplasmique active. Les patients etaient recenses dans le service d’ophtalmologie de 1995 a 2000. Il s’agissait de 10 hommes et 11 femmes d’âge moyen 28 ans. Tous les patients avaient suivi le protocole clindamycine en sous-conjonctival, associe a une corticotherapie par voie orale a raison de 1 mg/kg/j. La surveillance au cours du suivi etait essentiellement clinique, basee sur l’evaluation de l’acuite visuelle et l’examen biomicroscopique du fond d’œil, completee par une angiographie retinienne en cas de besoin. Resultats Le suivi moyen etait de 22 mois, allant de 6 a 52 mois. Le traitement a base de clindamycine-corticoide avait permis d’obtenir une amelioration subjective de l’acuite visuelle au cours des 4 premiers jours dans 68,75 % des cas et une cicatrisation des foyers chorioretiniens dans tous les cas dans un delai moyen de 1,6 mois. Une recurrence etait observee dans uniquement 2 cas (9 %). Elle avait eu lieu au cours des 18 premiers mois. La tolerance de ces injections etait bonne. Nous avions recense uniquement un cas d’erosion corneo-conjonctivale et un cas de necrose conjonctivale rapidement resolutive. Conclusion Le protocole clindamycine-corticoide a permis d’obtenir de bons resultats avec une bonne tolerance. Il a permis egalement de reduire le taux de recurrence par rapport aux protocoles classiques (pyrimethamine-sulfadiazine, pyrimethamine-azithromycine) ou un taux de recurrence de 50 % a ete rapporte. Ces resultats devront etre confirmes lors de larges etudes randomisees.

Summary Background: The genus Malassezia (Pityrosporum), recognized as a member of microbiological flora of the skin in humans and warm-blooded animals, has been recently revised to include 10 Malassezia species. The aim of the study was the isolation, identification and analysis of Malassezia species distribution in skin of healthy volunteers and lesions of pityriasis versicolor (PV). Material and methods: Specimens were taken from 100 patients with PV and 30 healthy volunteers. Cultures were made in modified Dixon agar medium and the isolates were identified by morphological and physiological methods: macroscopy, microscopy, catalase, β-glucosidase and lipid assimilation tests. Results: Malassezia globosa was the predominant species in lesional skin of PV (65%). It was isolated alone in 47% of cases and associated in 18% with M. furfur (13%) or M. sympodialis (5%). In healthy skin M. globosa was found alone in 7.77% and associated in 15.54%, respectively, with M. furfur (4.44%), M. sympodialis (4.44%), M. restricta (3.33%) and M. slooffiae (1.11%). Conclusion: From these findings it was suggested that M. globosa presents the main species implicated in the pathogenicity of PV and M. furfur as the second agent of importance.

Introduction Le decollement de retine (DR) apres chirurgie refractive par laser Excimer presente une incidence non negligeable. A partir d’une etude multicentrique dans les Centres de Laser Excimer Tunisiens, nous avons analyse et discute son imputabilite dans la genese du DR. Materiel et methodes Quatre centres disposant de 9 Lasers Excimer ont participe a cette etude ; 22 700 yeux ont beneficie d’une chirurgie refractive durant la periode allant de decembre 1994 a janvier 2002. L’âge moyen des patients ayant presente un DR etait de 37 ans, la myopie moyenne etait de 13,5 D. Le delai moyen de survenue de DR etait de 20 mois. Resultats Quinze yeux de 13 patients ont developpe un DR rhegmatogene dont 2 etaient bilateraux. Parmi ces cas, 8 ont eu un Lasik, 6 une photokeractectomie refractive (PKR) et a cause d’une forte regression post PKR, un patient a ete retraite par Lasik. Le DR etait total ou subtotal dans 5 yeux, partiel superieur avec une dechirure temporale dans 6 yeux et nasale dans 3 yeux. Un cas avec un DR inferieur, 2 cas de dechirure geante et 1 cas de dechirure posterieure etaient egalement colliges. L’indication operatoire etait de mise pour 14 yeux. Une reapplication retinienne a ete obtenue dans 12 cas. L’acuite visuelle moyenne postoperatoire etait de 7/10 e . Discussion La survenue spontanee de DR rhegmatogene dans la population de myopes est estimee a 2,2 % ; elle est de l’ordre de 0,1 % dans une population d’emmetrope. Le laser Excimer pourrait majorer le risque chez le myope par son effet thermique induit par l’onde de choc et le traumatisme subi par l’anneau de succion lors du Lasik. Une revue de la litterature a mis en doute l’hypothese d’une relation de cause a effet. L’incidence du DR post Excimer est inferieure a 0,1 % et rejoint celle de la population emmetrope. Ce faible pourcentage serait explique par une selection des patients et la pratique d’un traitement preventif des lesions dangereuses de la peripherie retinienne. Si une chirurgie du DR est necessaire, la manipulation de la cornee doit etre minutieuse ; un cas de dehiscence du volet a ete mentionne dans la litterature. Conclusion La chirurgie refractive par Lasik ou PKR indiquee pour les myopes forts ne majore pas statistiquement le risque d’apparition de DR. La selection et le traitement preventif des lesions degeneratives en diminuent le risque.

But de l’etude Le but de ce travail est d’evaluer les particularites epidemiologiques, cliniques, et therapeutiques du decollement de retine post contusif. Materiels et methodes L’etude est menee de facon retrospective sur 48 yeux de 48 personnes hospitalisees pour decollements de retine post contusifs diagnostiques entre janvier 1990 et decembre 2000 au service d’Ophtalmologie du CHU Habib Bourguiba de Sfax. Les particularites epidemiologiques — âge, sexe et circonstances de traumatismes — et cliniques — etendue de decollement, dehiscences (type, siege et nombre), ainsi que les techniques chirurgicales ont ete precisees. Resultats L’âge moyen des patients etait de 39,3 ans, avec une nette predominance masculine (75 % des patients). Les agressions et les accidents domestiques representaient les causes les plus frequentes des traumatismes (83,2 %). Le decollement de retine est total dans 47,9 % des cas. Les dehiscences retrouvees etaient a type de desinsertion a l’ora serrata dans 22 %, trous atrophiques dans 33,9 %, dechirures retiniennes dans 40,6 % et dechirures geantes dans 4,2 % des cas. Ces dehiscences siegeaient electivement dans le quadrant temporal inferieur (40 %). Le traitement chirurgical a ete decide pour 41 patients, dont 38 ont beneficie d’une chirurgie classique, et 3 d’une chirurgie endoculaire. L’abstention therapeutique a ete decidee pour 2 patients, et 5 patients ont ete perdus de vue. Le succes anatomique a ete note dans 92,7 % des cas. Conclusion Il ressort de cette etude que le pronostic du decollement de retine post contusif reste favorable vu le jeune âge et l’absence de proliferation vitreo-retinienne avancee, mais ceci n’affecte en rien l’importance de la prevention.

Purpose To describe a previously unreported clinical entity of progressive extensive macular atrophy and pseudo-drusen appearance in middle age patients. Methods The database of the outpatient clinic for genetic sensory diseases was screened for patients over 40 years with uncharacterized macular dystrophy. Patients with extensive macular atrophy and pseudodrusen-like appearance were included. Clinical investigations included visual acuity, eye examination, color fundus imaging, OCT scan, autofluorescence, Farnsworth 15-HUE color testing, Goldman perimetry and ISCEV full-field ERG. Results Eighteen patients of 45 screened records matched the inclusion criteria. Bilateral polycyclic well-delineated chorioretinal atrophy extending to the temporal vascular arcades, with a larger vertical axis and without sparing of the fovea featured the macular lesion. The pseudodrusen-like appearance was widespread thoughout the posterior pole and the peripheral retina. In the extreme periphery, paving stone lesions were mostly located in the inferior quadrants. In contrast to age-related macular degeneration, a rapid progression of the atrophy was observed with an early involvement of the foveal zone, thus leading to a severe visual loss. All the patients except two were legally blind at the end of the follow-up. In all patients, the scotopic and photopic ERG responses were reduced. Conclusion The cause of Extensive Macular Atrophy with Pseudodrusen (EMAP) remains unknown. Extensive Macular Atrophy with pseudodrusen (EMAP) should be considered as a possible pattern of severe macular dystrophy occurring in the middle-aged adult.

Purpose To describe 3 families with Best’s disease with normal electro-oculogram (EOG) and without VMD2 mutations. Methods Evaluation of the patients included visual acuity, fundus and autofluorescence (Heidelberg Retinal Angiograph), Goldmann visual fields, optical coherence tomography (Zeiss, OCT3), full field (ISCEV protocol) and multifocal electroretinograms, and EOG (ISCEV protocol). The diagnosis of Best’s disease was based on autosomal dominant inheritance, typical yellowish, autofluorescent material in the central macula accumulating beneath the retinal pigment epithelium, and decrease of the EOG Arden ratio. Results Among the 1130 families with various retinal dystrophies followed up in Montpellier, 40 (3.5%) were found with vitelliform macular dystrophy. Best’s disease was observed in 20 of them while 13 families had adult macular vitelliform dystrophy and 7 had reticular dystrophy. In the group with Best’s disease, a normal EOG was recorded in 3 families. None of these 3 families carried mutations in VMD2 or RDS, and linkage to the VMD2 locus was excluded in one of them. Conclusion In patients with Best’s disease and VMD2 (bestrophin) mutations, the decreased response of the EOG is attributed to an abnormal transepithelial chloride transport. However, the observation of families with normal EOG and no VMD2 mutations suggests that other genes are responsible for Best’s disease which could not impair the ionic transport but yet lead to similar subretinal deposits.

The lipophilic yeast of the genus Malassezia are opportunistic microorganisms of the skin microflora but they can be agents of various dermatomycoses. The aim of this study was to perform molecular identification of the commonly isolated Malassezia species from various dermatomycoses in our region. Thirty strains of Malassezia were isolated from different dermatologic affections: pityriasis versicolor (17), dandruff (5), seborrheic dermatitis (4), onyxis (2), folliculitis (1) and blepharitis (1). These species were identified by their morphological features and biochemical characterisation. The molecular identification was achieved by amplification of the internal transcribed spacer region by simple PCR. PCR technique was used for molecular characterisation of four Malassezia species: Malassezia globosa (270 bp), Malassezia furfur (230 bp), Malassezia sympodialis (190 bp) and Malassezia restricta (320 bp). We have detected the association between M. furfur and M. sympodialis in 16% and confirmed presumptive identification in 70% of the cases. The phenotypic identification based on microscopic and physiological method is difficult and time consuming. The application of a simple PCR method provides a sensitive and rapid identification system for Malassezia species, which may be applied in epidemiological surveys and routine practice.

To assess the benefit of cochlear implant in children presenting an Usher type 1 syndrome (speech understanding, speech production intelligibility, academic performance) and to search any correlation between the phenotype and the genotype in this population.Retrospective case series analysis about 13 implanted Usher type I children. Cochlear implantation was performed from 1995 to 2005. Our population was divided in three groups: group 1 (implantation between 1 and 3 years of age); group 2 (implantation between 4 and 7 years of age) and group 3 (implantation between 14 and 17 years of age). Postoperative speech perception, speech production intelligibility and education settings were evaluated.Molecular genetic analysis was performed in 11 patients and pathogenic mutations were identified in all cases: (mutation in myosin 7A gene in 5 cases; mutation in cadherin 23 gene in 6 cases). Four new mutations 2 in the MYO7A gene and 2 in the CDH23 gene never reported before were found. Walking delay and hearing level were not statistically correlated with the genotype abnormalities found. The speech discrimination skills, the speech production intelligibility and the academic performance were better in the group 1 children than the group 2 children after cochlear implantation. All the children of group 1 but one were in mainstreaming education. Specific language impairment was identified in two children of group 1. The group 3 children could not achieve open-set perceptive tasks after implantation--only closed-set word test can be done and their speech production remained unintelligible after cochlear implantation.Molecular analysis of Usher type I syndrome can ascertain the diagnosis in spite of the genetic heterogeneity. In this study, clinical symptoms weren't correlated with genotypic mutations. Speech discrimination skills, speech production quality, and academic performance were correlated with the age at implant.

Introduction L’apparition d’une insuffisance respiratoire aigue (IRA) est un evenement grave dans l’histoire evolutive d’un cancer broncho-pulmonaire (KBP). Notre objectif etait de determiner les etiologies et les caracteristiques de l’IRA chez les patients porteurs d’un KBP. Methodes Il s’agit d’une etude retrospective faite sur une periode de 4 ans ; incluant 31 patients atteints de KBP et hospitalises pour une IRA, dans le service de pneumologie de l’hopital la Rabta (Tunis). Resultats Parmi 310 sujets atteints de KBP, 10 % ont presente une IRA. Dans 38 % des cas, l’IRA etait inaugurale. L’âge moyen etait de 60 ans. Une predominance masculine a ete notee (93 %). L’IRA etait en rapport avec l’evolutivite tumorale dans 31 %. Dans les autres cas, elle etait secondaire a une pneumopathie infectieuse (19 %), a une embolie pulmonaire (12 %), a une pleuresie (16 %), a un pneumothorax (3 %) et a une exacerbation de BPCO (19 %). Il s’agissait de CNPC dans 24 cas (24/215) et de CPC dans 7 cas (7/95) (p > 0,05). La maladie etait a un stade metastatique dans 58 %, localement avance dans 36 % et localise dans 6 % des cas. La plupart des patients ont recu des traitements symptomatiques, aspecifiques du KBP (oxygenotherapie, bronchodilatateurs, corticosteroides, opioides, anxiolytiques, anticoagulants…). Une bonne evolution de l’IRA a ete notee chez 64 % des cas, 29 % etaient decedes dans un tableau d’IRA. Conclusion Nous concluons que certaines causes d’IRA sont potentiellement curables chez les patients atteints de KBP. Le pronostic reste grave si l’IRA est liee a la progression du cancer et si elle n’est pas accessible a un traitement. Des traitements plus specifiques peuvent etre envisages dans des situations particulieres (prothese endobronchique, laser…) afin d’ameliorer le pronostic.

Acanthamoeba keratitis is a rare but severe corneal infection which, despite improvements in diagnosis and treatment, still culminates in prolonged morbidity and significant loss of visual acuity. We present the case report of the first identification of Acanthamoeba as a causative agent of keratitis in Tunisia. Case no 1: A 20-year-old girl, nearsighted corrected with soft contact lenses, suffering from a deep corneal inflammation and poor visual acuity The ophthalmological examination showed bilateral dendritiform epithelial keratitis. The illness did not respond to topical and general antibiotic treatment and developed bilateral corneal abscess. Microscopic examination and culture of samples from cornea scraping revealed the presence of trophozoit and cysts of Acanthamoeba associated with Fusarium oxysporum. As the treatment with local Ketoconazol and antibiotherapy didn't show any result, two transfixiant keratoplasty were carried out and treatment by Désomédine, PHMB (polyhexamethylene biguanide) and Voriconazol was started. After two months, the patient felt better, vision was also improved (2/10) and infiltrates became smaller Case no 2: A 19-year-old girl, nearsighted with soft contact lenses consulted for a bilateral corneal ulceration and poor vision (1/20). Trophozoit and cysts of Acanthamoeba were found in the contact lens solution. She was treated quickly with Désomédine. Visual acuity improved to 7/10 but the corneal ulceration left a residual opacity

Triple A or Allgrove syndrome is a rare autosomal recessive disease with alacrima, achalasia, and ACTH-resistant adrenal insufficiency. It is usually associated with neurological disorders. Recently, mutations in the AAAS, a candidate gene mapped to chromosome 12q13, were identified. We report a family with seven affected siblings. All of them have signs of alacrima, four were operated on for achalasia, five have neurological abnormalities including cranial nerve abnormalities, amyotrophic lateral sclerosis, pyramidal syndrome, distal motor neuropathy, and amyotrophy, and two have adrenal insufficiency. Triple A syndrome should be considered in any young patient with alacrima.

To evaluate the efficacy and safety of subconjunctival injections of clindamycin associated with oral corticotherapy in the treatment of toxoplasmic retinochoroiditis (TRC) and its effect on recurrence.This study investigated 21 patients (10 males and 11 females) who presented to an ophthalmological department from 1995 to 2000, aged 14-55 years (mean, 28 years), with unilateral TRC. They were treated with subconjunctival injections of clindamycin and systemic corticotherapy at a dose of 1 mg/kg/day. Clinical follow-up consisted of assessment of visual acuity and biomicroscopic fundus examination, completed by retinal angiography if necessary.We observed clinical improvement in 68.75% of patients within 4 days and in all patients healing of the chorioretinal area within 1.6 months. During the follow-up period ranging from 6 to 52 months (mean, 22 months), there have been two recurrences (9%) over the first 18 months. Tolerance of the injections was good. Serious side effects were noted in two patients: one case of conjunctival necrosis and one case of corneal and conjunctival erosion.In treating ocular toxoplasmosis, clindamycin appears to be a safe and effective substitute for the classic protocol (pyrimethamine-sulfadiazine, pyrimethamine-azithromycin), for which a 50% recurrence rate has been reported. It provides relatively good protection against recurrence and warrants testing in further controlled studies.

Refractive surgery by LASIK or photorefractive keratectomy (PRK) generaly aims at a myopic population that has a high probability of developing rhegmatogenous retinal detachment (RD). The authors report a multicenter study with 15 cases of RD appearing after refractive surgery by Excimer laser and discuss the role played by the techniques used.Five centers fitted with nine Excimer laser devices took part in this study. Of 22,700 eyes undergoing refractive myopic surgery during the period 1994-2002, 15 eyes developed rhegmatogenous RD. The average age of the patients with RD was 37 years. The average myopia was 13.5 D. RD occurred a mean of 20 months after refractive laser.Fifteen eyes of 13 patients developed a rhegmatogenous RD, two of which were bilateral. Eight of these cases had LASIK surgery and six had photorefractive keratectomy; one of the latter patients was retreated with LASIK because of substantial regression after PRK. RD was total or subtotal in five eyes, partial superior with a temporal tear in six eyes, and nasal in three eyes. One case with inferior RD, two cases with giant retinal tear and one case with posterior tear were also repaired. Fourteen eyes were suitable for operation. The retina was reattached in 12 cases. Mean postoperative visual acuity was 7/10.The occurrence of rhegmatogenous RD in the myopic population is estimated at 2.2%. It is estimated at 0.1% in the emmetropic population. The Excimer laser, through its thermic effects, shock wave, traumatism undergone by the suction ring at the time of LASIK surgery, could increase this risk in myopic patients. A review of the literature cast doubt on the cause and effect hypothesis. Personal and multicenter studies (including ours) show that the frequency rate of rhegmatogenous RD after Excimer laser is equivalent and even lower than that estimated with an emmetropic population. The low percentage of RD after Excimer surgery found in the literature as well as in our study (0.1%) may be explained by patient selection, the systematic monitoring of the peripheral fundus, and the prophylactic treatment of degenerative lesions by photocoagulation. In RD surgery, the cornea must be manipulated carefully, a case of flap dehiscence has been reported in the literature.Refractive surgery by LASIK or PRK for severe myopia increases the risk of RD. Systematically monitoring the peripheral fundus and preventive photocoagulation have mitigated its occurrence, and the risk incurred in the myopic population has fallen to the emmetropic population's rate. Nevertheless, candidates for LASIK or PRK surgery must be informed because severe myopia constitutes a non-negligible risk factor.

Analysis of the epidemiological, clinical and therapeutic characteristics of retinal detachment after ocular blunt trauma.We retrospectively reviewed 48 patients with isolated retinal detachment after ocular blunt trauma, studying their epidemiological characteristics (age, sex and trauma circumstances), their clinic details and the surgical techniques used.The average age of our patients was 39.3 Years; most were male (75%). Assault and home accidents were the most common cause of ocular trauma (83.2%). Total retinal detachment was noted in 47.9%. Of the retinal breaks found, dialysis at the ora serrata was observed in 22%, atrophic holes in 33.9%, retinal breaks in 40.6% and giant tears in 4.2%. Most of the breaks were situated at the lower temporal quadrant. Forty one out of the 48 patients were operated on, with surgical treatment consisting in episcleral surgery in 38 cases and vitreoretinal surgery in three cases. Complete anatomical success was achieved in 92.7%.This survey shows that the prognosis of retinal detachment after ocular blunt trauma remains favorable given the young age and the absence of advanced vitreoretinal proliferation, but this should not affect the importance of prevention.

Introduction L’association d’un diabete de type 1 a une thyreopathie auto-immune s’integre dans le cadre d’un syndrome appele polyendocrinopathie auto-immune (PEAI) de type IIIa selon la classification de Neufeld. Le but de notre etude est d’etudier les caracteristiques epidemiologiques, cliniques et immunologiques du diabete de type1 associe a une thyreopathie auto-immune au sein d’une polyendocrinopathie auto-immune. Patients et methodes Il s’agit d’une etude retrospective portant sur 60 cas de PEAI de type IIIa collige dans notre service durant une periode de 27 ans. Resultats L’âge moyen des patients au moment de l’association des deux endocrinopathies etait 39 ans. Le diabete etait decouvert a un âge moyen plus precoce (32 ans) que les thyreopathies auto-immunes (37,7 ans). Une predominance feminine nette etait marquee : Sex-ratio (F/H) : 3,8. Il s’agissait d’une association du diabete de type 1 et thyroidite de Hashimoto goitreuse dans 27 cas, 16 cas de thyroidite atrophique et de 17 cas de maladie de Basedow. D’autres maladies auto-immunes etaient associees : hypophysite, maladie cœliaque, anemie de Biermer, vitiligo chacun dans 2 cas. L’hepatite auto-immune, l’alopecie et le syndrome de Goujerot Sjogren etaient notes chacun dans 1 cas. Le delai d’apparition des maladies auto-immunes depend de l’ordre de leur survenue. En effet, l’intervalle du temps moyen qui separe le diabete et la thyreopathie (10,3 ans) etait plus long que l’intervalle du temps entre la thyreopathie et diabete (4,3 ans) avec une difference significative (p = 0,02). L’etude immunologique montrait une positivite des anticorps anti-thyroperoxydase dans 74,8 % des cas, anti-thyroglobuline dans 45,5 % des cas, anticorps anti-GAD dans 63,6 % des cas, tandis que les ICA etaient positifs dans 34 % des cas seulement. Conclusion En conclusion, le diabete type1 precede les maladies thyroidiennes auto-immunes ce qui offre l’occasion de depister des dysthyroidies devant tout diabete type1 declare.

Introduction La polyendocrinopathie auto-immune de type II (PEAI II) est definie, selon la classification de Neufeld, par l’association d’une maladie d’Addison a une maladie thyroidienne auto-immune et/ou un diabete. Elles repondent a un modele physiopathologique particulier que schematise le concept de maladies auto-immunes specifiques d’organes. Materiels et methodes Nous avons mene une etude retrospective de 39 cas de PEAI II, ainsi qu’une etude cas temoins du polymorphisme des genes HLA classe II, qui inclut 13 cas de patients ayant une PEAI II et de 120 temoins sains, afin d’identifier les alleles de predisposition. Resultats La maladie d’Addison etait decouverte a un âge moyen de 39 ans. Une predominance feminine etait notee. Les thyreopathies auto-immunes etaient les plus frequemment associees a la maladie d’Addison dans 82 % des cas : 16 cas de thyroidite de Hashimoto dans sa forme atrophique, 12 cas dans sa forme goitreuse et 4 cas de maladie de Basedow. Le diabete etait associe a la maladie d’Addison dans 25,6 % des cas. L’etude immunologique montrait une positivite des anticorps anti-thyroperoxydase dans 54 % des cas, anticorps anti-GAD dans 28,5 % des cas et les anticorps anti-cortex surrenalien dans 21,8 % des cas. L’etude du polymorphisme des genes HLA classe II, realisee pour 13 cas, montrait que l’allele DRB1*03 etait significativement associe aux PEAI type II ( p = 0,00004 ; RR = 3,48). Une association moins forte avec l’allele DRB1*04 etait retrouvee ( p = 0,05 ; RR = 1.8), devenant non significative apres correction de Bontferroni. De meme, une association significative de l’allele DQB1*0302 etait observee ( p = 0,002 ; RR = 2,27) dans notre population. On n’a pas note d’association negative d’un ou plusieurs alleles avec la PEAI II. Conclusion A travers cette etude, nous avons pu mettre en relief les caracteristiques epidemiologiques, cliniques et immunologiques de la maladie d’Addisson au cours de la PEAI et nous avons prouve l’association entre cette entite et le polymorphisme des genes du systeme HLA.

Introduction La maladie de Best est une dystrophie maculaire hereditaire caracterisee par une transmission autosomique dominante, la presence de materiel vitellin maculaire avec des aspects evolutifs connus et une diminution obligatoire du rapport d’Arden. Trois familles avec une maladie de Best respectant tous ces criteres sauf l’atteinte de l’electro-oculogramme sont ici etudiees. Materiels et Methodes La selection de ces familles a tenu compte des arbres genealogiques (maladie hereditaire a transmission dominante), des donnees cliniques (atteinte precoce, materiel vitellin caracteristique), des cliches en auto fluorescence (HRA2, materiel auto fluorescent) et de l’aspect en tomographie a coherence optique (depot hyper-reflectif sous la ligne de l’epithelium pigmentaire). Resultats Mille cent trente familles ont ete examinees dans ce centre. Quarante familles (3.5 %) ont une « dystrophie vitelliforme ». Vingt familles ont une maladie de Best, treize une dystrophie vitelliforme de l’adulte et sept une dystrophie reticulee. Parmi le groupe de maladie de Best, trois familles n’avaient pas d’alterations de l’electro-oculogramme. Ces trois familles n’ont pas de mutations dans le gene RDS. Il n’y a pas de mutation du gene VMD2 pour une de ces trois familles (resultats en attente pour les deux autres). Discussion Les anomalies de l’electro-oculogramme dans la maladie de Best liee a VMD2 seraient la traduction de modifications de transports ioniques de l’epithelium pigmentaire. Les alterations de la bestrophine (proteine transmembranaire de l’epithelium pigmentaire codee par VMD2) modifieraient ces transports et conduiraient egalement a l’accumulation de materiel (mecanisme inconnu). Ces trois familles ont probablement une maladie de Best avec une mutation sur un autre gene ayant pour seule consequence l’accumulation de materiel. Conclusion La maladie de Best n’est pas uniquement liee au gene VMD2. Un autre gene est probablement implique dans l’accumulation de materiel, ce sous-groupe de patients ayant un electro-oculogramme normal.

We report a case of duplication of the mouth, tongue, mandible, and cervical spine in a female neonate. Findings in this patient were somewhat different from those in previously reported cases. Clinical, radiological and therapeutic features in this localized duplication are reviewed. Embryologic hypotheses include defective midline development, such as in the split notochord syndrome, and development of totipotent cells from the first branchial arch.

Four cases of bilateral nephroblastoma out of a total number of 99 cases have been observed during a period of 18 years at the INSE of Tunis (4%). The frequency of associated anomalies, the familial incidence, the young age of the patients are underlined. The main therapeutic modalities are discussed. Based on the most recent studies published in the literature, the prognosis of bilateral nephroblastoma has been improved in recent years with a possibility of survival at 2 years exceeding two thirds of the cases.

A simplified panorama of the different formation phases of the upper respiratory system (larynx, trachea, main bronchi) is presented. Chronologic evolution of organogenesis in embryologic phase and fetal phase is emphasized. Post-natal growing is also described. A classification of malformations of the upper respiratory system is done, based on topographic data. The mechanism of genesis of the malformations remains of hypothetical value.

The authors analyse this case and those published in the literature (115 cases) and the relationship to Wilm's tumor. The histologic characteristics of this tumor and its benign course indicate surgical treatment without any adjuvant treatment.

This paper describes 62 cases of peptic complications due to gastro-oesophageal reflux in infants and children at the Children's Hospital in Tunis over a 20 years period. The age of the patients ranged from 6 months to 14 years with a majority of males. Failure to thrive, anemia and respiratory complications were the most common features. Upper gastro-intestinal series and endoscopy were performed in all patients and showed the presence of associated hiatus hernia in 53 patients, oesophagitis in 62 and oesophageal stricture in 33. All patients had a primary course of medical treatment. Fifty-three patients required Nissen's fundoplication. Thirty-one patients required oesophageal dilatation. A complete cure of the structures was achieved in all patients but 7. In the whole series, 4 children died.