H. Dhouib

Nasopharyngeal carcinoma affects preferentially young adults and is characterized by a high rate of node and visceral metastases that explains a part of therapeutic failure. We report a case of thyroid metastasis of a nasopharyngeal carcinoma. A 50-year-old patient had been treated of a nasopharyngeal carcinoma (T4 N2M0). Four years after a concomitant chemoradiotherapy, he presented with a compressive mass in the thyroid loge associated to cervical nodes. He underwent a total thyroidectomy and the definitive result concluded to an carcinome indifférencié de type nasopharyngé (UCNT) thyroid metastasis. A new concomitant chemoradiotherapy was delivered and consequently the tumor disappeared. Secondly, several node recurrences occurred. Metastasis of UCNT in thyroid gland is exceptional. Several problems are related to its aetiopathogenesis and to its treatment that is not systematized in the literature. The prognosis like for all metastatic forms of nasopharyngeal cancer is unfavorable.

... H. DHOUIB, M. MNEJJA, W. BOUAYED, A.CHAKROUN, T. BOUDAWARA*, I. CHARFEDDINE, M. FRIKHA**, J. DAOUED***, A. GHORBEL, ... La première patiente, atteinte d'un mélanome malindu cavum (T2aN2Mo) (UICCC 97), a bénéficié d'un évidement ganglionnaire cervical ...

ObjectifThe incidence of aspergillosis has increased during the past decade with the widespread use of aggressive chemotherapy and immunosuppressive agents. The lung is the most common primary site of infection. Thyroid involvement is rare, usually asymptomatic, and difficult to make an antemortem diagnosis. Our aim is to discuss the epidemiological characteristics, the diagnosis and the histogenesis of this unusual thyroid

Chronic diseases affecting the inner ear and the retina cause severe impairments to our communication systems. In more than half of the cases, Usher syndrome (USH) is the origin of these double defects. Patients with USH type II (USH2) have retinitis pigmentosa (RP) that develops during puberty, moderate to severe hearing impairment with downsloping pure-tone audiogram, and normal vestibular function. Four loci and three genes are known for USH2. In this study, we proposed to localize the gene responsible for USH2 in a consanguineous family of Tunisian origin. Affected members underwent detailed ocular and audiologic characterization. One Tunisian family with USH2 and 45 healthy controls unrelated to the family were recruited. Two affected and six unaffected family members attended our study. DNA samples of eight family members were genotyped with polymorphic markers. Two-point and multipoint LOD scores were calculated using Genehunter software v2.1. Sequencing was used to investiga...

Abstract Introduction  Primitive melanomas of the nasosinusal mucosa are rare. They only represent 0.5 to 2% of melanoma and usually cause unilateral nasal obstruction and nasal bleeding. The diagnosis is histological, achieved via immunohistochemistry analysis. Their prognosis remains poor in spite of surgical treatment associated with post-surgical chemoradiotherapy. Our objective is to discuss the epidemiological, clinical and pathological data relating to

ABSTRACT In this paper we propose P/S-CoM+, a formal approach that helps architects to model correct publish/subscribe architectural styles (PSAS). We extend P/S-CoM formal approach, that focuses on the correct by design of PSAS structure, by behavioral aspect. We specify communication schemas including the state of component types and ensuring no loss of events. We formally model behavioral properties namely delivery semantics, ordering and persistence of notifications. We set up a formal method for composing communication schemas and building correct PSAS. We develop an ECLIPSE plugin P/S-CoM'SD that assists architects in graphically conceiving the desired PSAS. We describe the dynamic behavior of component types by specifying interaction rules. All the specifications are coded in Z notation and proved under Z-Eves theorem prover.

... OBSERVATION 1 T. Mariem, âgée de 13 ans, sans antécédents patholo-giques notables, a consulté au service d'ophtalmologie pour ... OBSERVATION 2 B .Sofiane âgé de 13 ans sans antécédents pathologiques particuliers, a consulté pour un œdème palpébral supérieur ...

Juvenile xanthogranuloma (JXG) is a cutaneous non-Langerhans cell histiocytosis affecting infants. We report the first case of JXG in the parotid gland of an adult. A 52 year-old man consulted for a painful swelling of the left parotid region, progressively increasing. On physical examination, the mass was 4 cm in diameter. A second 1.5 cm diameter tumor was found in the right parotid region. MRI revealed masses with heterogeneous hypodense on T1-weighted images and with hyper dense on T2 weighted images associated with heterogeneous enhancement after gadolinium injection. The superficial and deep lobes of the parotid gland were involved. Per-operative left-side exploration revealed a lipomatous degeneration of the parotid gland and facial nerve. A lower polar parotidectomy was performed. The histological examination identified a parotid xanthogranuloma. The JXG is usually revealed by cutaneous lesions. In fact, the morphological, immunohistochemical, and ultrastructural features of this affection lead to its diagnosis. Extra-cutaneous localizations are less frequent. The differential diagnosis is X histiocytosis.

... Hepatogastroenterology 1992;39:127–31. 11)Donald G. Crescenzo.Zenker's Diverticulum in the Elderly: Is Operation Justified? Ann Thorac Surg 1998;66:347–50. ... Surg Clin North Am 1983;63:815–24. 14) Aggerholm K, Illum P. Surgical treatment of Zenker's diverticulum. ...

Autosomal recessive retinitis pigmentosa (ARRP) is a genetically heterogeneous disorder. ARRP could be associated with extraocular manifestations that define specific syndromes such as Usher syndrome (USH) characterized by retinal degeneration and congenital hearing loss (HL). The USH type II (USH2) associates RP and mild-to-moderate HL with preserved vestibular function. At least three genes USH2A, the very large G-protein-coupled receptor, GPR98, and DFNB31 are responsible for USH2 syndrome. Here, we report on the segregation of non-syndromic ARRP and USH2 syndrome in a consanguineous Tunisian family, which was previously used to define USH2B locus. With regard to the co-occurrence of these two different pathologies, clinical and genetic reanalysis of the extended family showed (i) phenotypic heterogeneity within USH2 patients and (ii) excluded linkage to USH2B locus. Indeed, linkage analysis disclosed the cosegregation of the USH2 phenotype with the USH2C locus markers, D5S428 and D5S618, whereas the ARRP perfectly segregates with PDE6B flanking markers D4S3360 and D4S2930. Molecular analysis revealed two new missense mutations, p.Y6044C and p.W807R, occurring in GPR98 and PDE6B genes, respectively. In conclusion, our results show that the USH2B locus at chromosome 3p23-24.2 does not exist, and we therefore withdraw this locus designation. The combination of molecular findings for GPR98 and PDE6B genes enable us to explain the phenotypic heterogeneity and particularly the severe ocular affection first observed in one USH2 patient. This report presents an illustration of how consanguinity could increase familial clustering of multiple hereditary diseases within the same family.

Basosquamous carcinoma is a rare entity that essentially affects the head and neck region in male patients. The authors present the clinical signs and progression as well as the therapeutic consequences of this disease through two observations. CASE REPORT 1: A 41-year-old man presented with basosquamous carcinoma of the right temporoparietal region treated initially with surgery alone. Five years later, he was operated on for a local and lymph node recurrence followed by radiation therapy, stabilizing the disease for 4 years; subsequently a second recurrence with metastasis to the chest area occurred. The patient died 10 years after the onset of his disease of diffuse pneumopathy with severe septicemia. CASE REPORT 2: A 71-year-old man presented retroauricular basosquamous carcinoma at first treated with wide resection, but the surgical limits were invaded. He developed local recurrences treated with surgical resection until total petrosectomy, but the surgical limits were always invaded. Radiotherapy was delivered. Seven years after the end of treatment, he developed a local recurrence invading the brain, which was deemed untreatable. Basosquamous carcinoma is characterized by its severe aggression and its tendency to recur. Treatment is essentially surgical. Radiotherapy is an adjuvant for the cases with high risk of recurrence. The role of chemotherapy is not yet proved.