An extensive hemimelic polyostotic fibrous dysplasia: a case report.

We report a new case of polyostotic fibrous dysplasia (FD). A 26-year-old woman was referred to our department complaining of pain in her left arm. She had suffered for tow fractures in left leg and arm previously. Plain radiographs showed osteolytics lesions at the left humerus and radius. Histological examination of the surgical specimens showed FD. She has beneficed with zoledronic acid perfusion.

Assessing human genetic diversity in Tunisian Berber populations by Alu insertion polymorphisms.

BACKGROUND: The most recent Alu insertions reveal different series of characteristics such as stability that make them particularly suitable genetic markers for human biological studies. AIM: Six human-specific Alu insertion polymorphisms were typed in two Tunisian Berber populations with the aim of analysing the genetic diversity of these two communities and the genetic relationships between this region of North Africa and other populations. SUBJECTS AND METHODS: Forty-seven Berbers from Sejnane and 33 from Takrouna were sampled. Alu insertion polymorphism was analysed using PCR with loci specific primers. RESULTS: A similar level of gene diversity was detected in Sejnane and Takrouna populations. PC results revealed genetic affinities between these two populations and some Eurasian populations (Germany, Genova and Syria). In contrast, there is a differentiation between these two Berber communities and North African and Iberian populations. CONCLUSION: The results of this study confirm the heterogeneity of Berbers in North Africa, which suggests their diverse origins. In the case of Sejnane and Takrouna populations, these results are in line with an ancient Euro Mediterranean background that has already been studied by archaeologists, particularly for the population of Sejnane.

[Parotid mucormycosis: A new case and review of the literature]. / Mucormycose parotidienne : une nouvelle observation et revue de la littérature.

INTRODUCTION: Mucormycosis is a rare, devastating, fungal infection, which disproportionately affects non-controlled diabetic patients, notably during ketoacidosis. The authors report the case of cervical mucormycoses with a particularly favorable evolution in diabetic woman. REPORT: A 54-year-old woman, type 2 diabetic, had presented a left lateral cervical mass. The diagnosis was confirmed by histological examination. She was treated with Amphotericin B with favorable evolution. CONCLUSION: The mucormycose is a rare infection. The treatment is medical and surgical. The prognosis is severe with an overall mortality rate of 40%.

Rhinocerebral mucormycosis: five cases and a literature review.

OBJECTIVE: In this retrospective study, we describe our experience in the diagnosis and management of rhinocerebral mucormycosis (RCM), a rapidly lethal fungal infection. METHODOLOGY: Between 1997 and 2007, five patients hospitalized for suspicion of RCM. Computed tomography was performed in all cases, and diagnosis was confirmed after anatomopathological or mycological examination. All patients underwent medical and surgical treatment. Follow-up was clinical and radiological with a mean period of 17 months. RESULTS: All patients were diabetic. Exophthalmia, rhinorrhea, and ophthalmoplegia were the most frequent symptoms observed. One patient had loss of visual acuity and another exhibited peripheral facial palsy. One patient had extensive hemifacial cutaneous necrosis. Nasal endoscopy revealed black necrotic lesions in one case, and another patient had a tumefaction localised in the left middle meatus. Necrotic lesions were most often found in the orbit, the maxillary and the ethmoidal sinuses on computed tomography (four cases for each site). One patient had thrombophlebitis of the cavernous sinus, and another had an intracranial extension. All patients were administered ordinary insulin and intravenous amphotericin B. Surgical debridement of the nasal cavity and the involved sinuses was performed through lateral rhinotomy (four cases) or endoscopy (one case). Unilateral orbital exenteration was associated in two cases. Progression was favourable in four cases; one patient died from sepsis despite aggressive treatment. CONCLUSION: Early diagnosis is crucial for the management of RCM. Treatment of underlying disorders, use of intravenous amphotericin B, and aggressive surgical intervention are key in reducing morbidity and mortality rates.

Influence of airflow rate and substrate nature on heterogeneous struvite precipitation.

In wastewater treatment plants a hard scale consisting of struvite crystals can be formed, in pipes and recirculation pumps, during anaerobic digestion of wastewater. This study was conducted to evaluate the effect of airflow rate and substrate nature on nucleation type, induction period and supersaturation coefficient during struvite precipitation. A crystallization reactor similar to that designed for calcium carbonate precipitation was used. The pH of synthetic wastewater solution was increased by air bubbling. Experimental results indicated that the airflow increased heterogeneous precipitation of struvite. The susceptibility to scale formation was more important on polyamide and polyvinyl chloride than on stainless steel. In all cases, X-ray diffraction and infrared spectroscopy showed that the precipitated solid phase was solely struvite. No difference in crystal morphology was observed. However, at similar experimental conditions, the particle size of struvite was higher for stainless-steel material than that for plastic materials.

Expression of ACC oxidase antisense gene inhibits ripening of cantaloupe melon fruits.

The plant hormone ethylene plays a major role in the ripening of climacteric fruit. We have generated transgenic cantaloupe Charentais melons expressing an antisense ACC oxidase gene; ACC oxidase catalyzes the last step of ethylene biosynthesis. Ethylene production of transgenic fruit was < 1% of control untransformed fruit, and the ripening process was blocked both on and off the vine. The antisense phenotype could be reversed by exogenous ethylene treatment. Analysis of antisense ACC oxidase melons indicated that the ripening process includes ethylene-dependent and ethylene-independent pathways. Because the transgenic line we generated displays extended storage life and improved quality, it has a promising potential for commercial development.

[Skeletal fluorosis: a case report]. / Fluorose osseuse: à propos d'un cas.

The authors report a case of skeletal fluorosis described in a 60 years old man living in south-west Tunisian. The main clinical sign is a severe functional legs disability. Radiographic examination has shown a diffused osteocondensation, with cervical spine degenerative discopathy, a pelvis bilateral osteoarthritis and an interosseous membrane ossification of forearms and legs. The skeletal fluorosis diagnosis has been confirmed by high serum and urinary fluoride levels.

Paranasal sinus osteomas: Diagnosis and treatment.

INTRODUCTION: Osteoma is the most common benign tumor of the nose and paranasal sinuses. It is a slow-growing bony tumor, often asymptomatic, occurring mainly in frontal and ethmoid sinuses. Theories regarding the origin of osteomas are still discussed. The aims of the study were to describe diagnosis circumstances in our series and to set out our respective indications for open and endoscopic approaches in the treatment of nasosinusal osteomas. PATIENTS AND METHODS: A retrospective study was conducted on the files of all the patients treated for a paranasal sinus osteoma in our department between 1990 and 2013. Diagnosis circumstances and kind of treatment were collected and analyzed. RESULTS: The files of 45 patients (mean age: 49.2; sex-ratio: 1.19) could be collected. The most common symptom was headache found in all patients. The most common location was the frontal sinus (30 cases). Thirty-nine open procedures were performed. Four osteomas were removed under endoscopic assistance. In one case, a combined approach has been used. Overall complication rate was 11.1%. Symptoms improved in all patients. Two recurrences were observed. DISCUSSION: Surgical indications in paranasal sinus osteomas are theorically well codified. However, approaches remain controversial. In our experience, the preferred approach was the open one. Endoscopic techniques, when indicated, are more challenging and need sophisticated instrumentation and a long learning curve.

[Pure gonadal dysgenesis with 46 XY karyotyping (Swyer's syndrome) with gonadoblastoma, dysgerminoma and embryonal carcinoma]. / Dysgénésie gonadique pure à caryotype 46 XY (syndrome de Swyer) avec gonadoblastome, dysgerminome et carcinome embryonnaire.

We report the clinical and pathologic findings in a 22-year-old woman with XY gonadal dysgenesis (Swyer's syndrome), who had bilateral gonadoblastoma associated on the right side with a dysgerminoma and an embryonal carcinoma. Swyer's syndrome is a distinct type of pure gonadal dysgenesis characterized by a 46 XY karyotype. It shows an abnormality in testicular differentiation. The patients are phenotypic females without stigmas of Turner syndrome. They have also elevated gonadotropins and hypoplastic gonads without germ-cells. The tumor that usually develops in Swyer's syndrome is gonadoblastoma. This tumor arises on dysgenesic gonads with a Y chromosome. Although gonadoblastoma is considered benign, the risk of malignant germ cell development is high. This means that these dysgenesic gonads should be removed surgically as soon as Swyer's syndrome is established.

[Primitive choriocarcinoma of the cervix. Apropos of a case]. / Choriocarcinome primitif du col de l'utérus. A propos d'un cas.

We report a case history of primitive chorioncarcinoma of the cervix in a woman of 48 years of age. The diagnosis of chorioncarcinoma of the cervix was strongly suggested in the hysterectomy specimen that was obtained during an emergency operation. In fact it consisted of a polypoid mass near the cervix which showed as severe haemorrhage, so that there was no chance of taking a biopsy specimen. The history that the patient gave as well as her clinical and biological findings made it possible to diagnose the case definitively. This trophoblastic tumour resulting from pregnancy is extremely are in the cervix. 60 cases have been published up to now in the world literature. The rareness of this tumour in this site makes it worth while to try to find out the aetiology and pathology of the condition. It is to be distinguished from other chorioncarcinomata in other sites.

[Pelvic hydatic echinococcosis in women in the maternity ward at the Habib Thameur University Hospital. Apropos of 5 cases (1978-1982)]. / L'échinococcose hydatique pelvienne chez la femme à la maternité du C.H.U. Habib Thameur. A propos de 5 cas (1978-1982).

We report 5 cases of pelvic hydatic cysts in women seen between 1978 and 1982. Hydatic disease is endemic in Tunisia, in spite of attempts to eliminate it, and these attempts are being improved. The plague persists because of certain inbuilt customs and because sheep are normally raised by small-scale production methods. The incidence of pelvic hydatic cyst disease is 2% of pelvic tumours seen in our maternity department over 5 years. The diagnosis was made easily preoperatively in only 2 cases, and it was in those 2 cases because of a previous history of hydatid disease. Ultrasound and electrosyneretic testing for hydatid cyst confirm the diagnosis. In the other cases the diagnosis was only made during the operation. In only one case had hysterosalpingography shown an impression on the right side of the uterus and a displaced right tube. The treatment must be surgical; and the outlook is good, except in one case where there was a recurrence. Post-operative follow-up consists of pelvic ultrasound and electro-syneresis as well as a general supervision of the patient's general state of health. At present we are accustomed to thinking of hydatid cysts when confronted with a pelvic tumour in a woman who comes from a region where the condition is endemic, whether she has or does not have any previous history of hydatid disease. The diagnosis is confirmed by ultrasound and electrosyneresis. Prophylaxis is always worthwhile.

["In utero" diagnosis of congenital fragilitas ossium (osteogenesis imperfecta) (author's transl)]. / Diagnostic "in utéro" d'une fragilité osseuse congénitale.

The authors report a case of congenital fragilitas ossium diagnosed "in utero". The radiography of the fetus in the uterus showed bony abnormalities suggestive of Porak and Durante's disease. This early diagnosis is often useful because it will help to prevent trauma before and during delivery which is a principal cause of fetal death.

[Prenatal echographic diagnosis of conjoined twins after 20 weeks of amenorrhea]. / Diagnostic échographique anténatal d'un monstre double à 20 semaines d'aménorrhée.

Conjoined twins are a rare entity in human teratology. Because early diagnosis of conjoined twins is so difficult as to be almost impossible it is usually only made in labour. Recent progress in the use of ultrasound as a means of diagnosis and also advances in neonatal surgery have stimulated a renewed interest in this malformation. A recent case at the University Hospital Centre of Habib Thameur in Tunis made it possible for us to diagnose the condition after 20 weeks of amenorrhoea and to terminate the pregnancy.

[Mitochondrial DNA polymorphism reveals a genetic differentiation between ethnic groups in the population of Jerba]. / Le polymorphisme de l'ADN mitochondrial revèle une différenciation génétique entre certains groupes ethniques de la population de Jerba.

Jerba is an island situated in the South-East of Tunisia were some ethnic groups (Arabs, Berbers, Blacks, Jewishs and others) cohabit for centuries. The religion and cultural differences have represented an obstacle to a mixture between these groups. In order to evaluate the genetic differentiation between the muslim groups (Arabs, Berbers and Blacks), we have analysed the polymorphism of a mitochondrial DNA coding region. The cytochrome oxydase coding region (COII) was amplified by PCR in 57 Arabs, 42 Berbers and 16 Blacks. The amplified products were analysed by Restriction Fragment Length Polymorphism (RFLP). Genetic distances were calculated by using the AMOVA program. The values of these distances were significantly different between Arabs and Blacks, and between Berbers and Blacks but not between Arabs and Berbers. So That, to refine the evaluation of genetic diversity between Arabs and Berbers, we have analysed the polymorphism of a second mitochondrial coding region which encodes for the fifth unit of NADH deshydrogenase (ND5). Eleven haplotypes were defined from the resulting data of mitochondrial COII and ND5 polymorphism and a significant genetic distance between Arabs and Berbers was computed.

[Management of ocular and orbital complications in acute sinusitis]. / Prise en charge des complications oculo-orbitaires des sinusites aiguës.

PURPOSE: Describe the clinical presentations of orbital complications of acute sinusitis and discuss therapeutic approaches. METHODS: Retrospective study of 29 cases of acute sinusitis with orbital extension hospitalized over a period of 12years (2000 to 2012). RESULTS: There were 23 men and 6 women. The mean age was 15.75years. The average time until consultation was 7.68 days. Sinusitis was ethmoido-maxillary in 20 cases, ethmoidal in 6 cases, and frontal in 3 cases. Orbital extension was grouped according to the Chandler classification: stage I (3 cases), stage II (3 cases), stage III (15 cases), stage IV (8 cases). Antibiotic therapy was prescribed in all cases. Surgery was performed in 22 cases. Bacterial cultures revealed streptococcus (2 cases), aspergillus fumigatus (1 case) and were negative in 8 cases. The outcome was favorable in 27 cases. In two cases, the outcome was unfavorable with associated intracranial complications. DISCUSSION: Oculoorbital extension of acute sinusitis is a diagnostic and therapeutic emergency. Paranasal sinus CT is essential to confirm orbital extension and identify the causative sinus. Intravenous antibiotic therapy is the main treatment. The roles of corticosteroid and heparin therapy remain controversial. Surgery is indicated in the case of abscess or high visual risk. Endoscopic endonasal surgery appears to have a double role, in diagnosis and treatment.

[Orbital complications of sinusitis]. / Les complications orbitaires des sinusites.

PURPOSE: Orbital complications of sinusitis are rare but serious, with potential functional and even fatal consequences. The purpose of this work is to study the epidemiological, clinical and paraclinical features of the orbital complications of sinusitis, as well as the various treatment modalities and clinical course. PATIENTS AND METHODS: We report the results of a retrospective study of 34 cases of orbital complications of sinusitis, collected between 1998 and 2009. RESULTS: Mean age was 19 years 3 months, with a clear male predominance. No predisposing factors were found. All patients presented with periorbital edema. In addition to periorbital edema, ophthalmological examination also revealed: proptosis in 19 patients, decreased visual acuity in eight patients and ocular motility disturbances in 10 patients. All patients underwent emergent facial CT, two patients underwent orbital ultrasound, and one underwent magnetic resonance imaging. Initial treatment was based on empiric intravenous antibiotic therapy. Orbital drainage was performed in 20 patients. Sinus drainage was performed in 16 patients (14 cases during the acute phase) with a middle meatotomy in all cases. The initial response was favorable in 97.5% of cases. One patient had a recurrence. Mean follow-up was 11 months. CONCLUSION: Orbital complications of sinusitis are a diagnostic and therapeutic emergency. Imaging helps guide management. Directed treatment and early management can improve mortality and morbidity.

[Clinical aspects of brain metastases from nasopharyngeal carcinoma]. / Aspects anatomocliniques des métastases cérébrales des carcinomes nasopharyngés.

Metastasis to the central nervous system, either through a hematogenous route or through the cerebrospinal fluid, is extremely rare in nasopharynx cancer. We aim to expose clinical aspects, therapeutic features and prognosis of nasopharyngeal carcinoma with brain metastases. We retrospectively reviewed the medical history of about 420 patients with nasopharyngeal carcinoma treated during 17 years at the university hospital of Sfax (Tunisia). Among them, three patients had brain metastasis. We excluded patients with direct extension to the brain. Tumours of the nasopharynx were locally advanced. The first patient had brain metastases at the initial diagnosis. The two other patients had brain metastases at 10 and 16 months during the follow-up. Ocular signs were the symptoms. Lesions were unique in two patients. Synchronous bone metastases were recorded in the three cases. All patients had whole brain radiation therapy and palliative chemotherapy. All patients had a progression of the disease and died. Brain metastases in nasopharynx cancer represent a rare event. Prognosis is poor, depending on age, surgical excision and synchronous metastases. Survival does not exceed 6 months.