[Familial idiopathic pulmonary fibrosis associated with autoimmune polyendocrinopathy and epidermodysplasia verruciformis]. / Fibrose pulmonaire idiopathique familiale associée à une polyendocrinopathie auto-immune et à une épidermodysplasie verruciforme.

Familial idiopathic pulmonary fibrosis (IPF) is a very rare and progressively fatal disease. Its pathogenesis is not fully understood and involves damage to alveolar epithelial cells of possibly immunological, microbiological or chemical origin, leading to fibrosing healing. A genetic predisposition has been demonstrated. The authors report the case of a female patient whose brother died at the age of 29 from IPF. She had epidermodysplasia verruciformis since childhood, with the absence of pubertal development. At the age of 31, she presented diffuse interstitial pneumonia. A lung biopsy confirmed the diagnosis of IPF. Endocrine explorations detected hypogonadotropic hypogonadism, primary hypothyroidism and magnetic resonance imaging revealed an empty sella turcica. The association of familial IPF, autoimmune polyendocrinopathy and genetic dermatosis caused by a cellular immune deficiency supports the hypothesis of an immune dysfunction in the pathogenesis of IPF.

[Mucormycosis and diabetes: three cases reported]. / Mucormycose et diabète: a propos de trois cas.

Mucormycosis is a rare and often fatal fungal infection. It mainly occurs when predisposing factors such as diabetic ketoacidosis are present. We report three cases of rhino-orbito-cerebral, rhino-orbital and pulmonary mucormycosis in patients with diabetic ketoacidosis. The diagnosis was reached after identification of hypae of the order mucorales using pathology and mycology examinations. A fatal issue occured in two cases and one case survived after adequate therapy.

[Lymphoepithelioma-like carcinoma of the uterine cervix: case report]. / Carcinome de type lymphoépithélial du col utérin. A propos d'un cas.

We report a case of a 79-year-old Tunisian woman who presented with post-coital bleeding. A polypoïd mass of the cervix was discovered. Histopathological examination of the specimen biopsy showed a squamous-cell carcinoma. The patient underwent radical resection. The definitive diagnosis of lymphoepithelioma-like carcinoma of the cervix was retained after histopathological and immunohistochemical examination. There was no local involvement and no metastasis. The lymphoepitheloma-like carcinoma of the uterine cervix is a rare entity with distinct morphological features and a usually good prognosis.

[Post-traumatic adenoid cystic carcinoma of the lacrimal gland]. / Carcinome adénoïde kystique post-traumatique de la glande lacrymale.

A young 17-year-old man was injured on the external orbital canthus and developed a tumefaction which remained stable. After 2 years the tumefaction with exophthalmia and visual troubles. Radiological investigations suggested two diagnosis: Organized hematoma or a lacrimal gland tumor. Surgical exploration found an apparently benign tumor but histologically it was a cystic adenoid carcinoma.

[Primary malignant non-Hodgkin's lymphoma of the salivary glands. An anatomo-clinical and immunohistochemical study of 2 cases and a review of the literature]. / Les lymphomes malins non hodgkiniens primitifs des glandes salivaires. Etude anatomo-clinique et immunohistochimique de 2 cas et revue de la littérature.

The authors reports two cases of primary non Hodgkin's lymphomas of the right submandibular gland in a 50 years old woman, and of a minor gland in another woman also 50 years old. The diagnosis was difficult for the second case, and was established on the immunochemistry study. The authors discuss the epidemiological, clinical, radiological, pathological features of this neoplasm and its management.

Diffuse bilateral glomerulocystic disease of the kidneys and multiple cardiac rhabdomyomas in a newborn. Relationship with tuberous sclerosis and review of the literature.

A case of glomerulocystic disease of the kidneys in a newborn is described. The kidneys were very large and diffusely cystic. Histologically, all portions of the nephron were involved and some of the cysts were lined by a prominent hyperplastic eosinophilic epithelium. This, together with the presence of numerous cardiac rhabdomyomas, a nodular haemangiomatous harmartoma and histiocytoid cell nests in the spleen were strongly suggestive of tuberous sclerosis. The relation between glomerulocystic kidney disease and tuberous sclerosis is discussed.

Serum type I collagen and N-terminal peptide of type III procollagen in chronic hepatitis. Relationship to liver histology and conventional liver tests.

The aim of this study was to compare serum N-terminal peptide of type III procollagen to aminotransferases and gamma-globulins as a marker for histological activity in patients with chronic hepatitis and to assess the role of type I collagen, a new serum marker, as a marker of fibrosis in these patients. Sixty patients with biopsy-proven chronic hepatitis were included in this study. Liver disease was virus B-related in 29, autoimmune in five, drug-induced in five, and of unknown etiology in 21. Each biopsy was independently assessed by two liver pathologists. Two histological scores, a score of activity and a score of fibrosis, were established. Serum N-terminal peptide of type III procollagen and type I collagen were assayed by liquid phase RIA. Significant correlations were noted between serum N-terminal peptide of type III procollagen and scores of activity (r = 0.70, p less than 10(-4)) and fibrosis (r = 0.45, p = 0.0005), and between serum type I collagen and scores of activity (r = 0.46, p = 0.0004) and fibrosis (r = 0.67, p less than 10(-4)). When the correlation between scores of activity and fibrosis (r = 0.52, p = 10(-4)) was considered by partial correlation, serum N-terminal peptide of type III procollagen was correlated with the score of activity (r = 0.63, p less than 10(-3)) but not with the score of fibrosis, and serum type I collagen was correlated with the score of fibrosis (r = 0.58, p less than 10(-3)), but not with the score of activity.(ABSTRACT TRUNCATED AT 250 WORDS)

Ureteral valves: report of a case diagnosed by antegrade pyelography.

Ureteral valves are an uncommon cause of upper urinary tract obstruction. The majority of cases are discovered only at surgery or autopsy. We report a case diagnosed by antegrade pyelography under sonographic guidance and we review the literature.

[Cavitary tumor lesions of the small intestine. Radio-ultrasonic diagnosis. Apropos of 4 cases]. / Les lésions tumorales cavitaires du grêle. Diagnostic radio-échographique. A propos de 4 observations.

Excavated lesions of small intestine are rare and generally of a malignant nature, the must frequent cause being metastases. In Tunisia these lesions are usually the aneurysmal type of lymphoma. The etiology in a series of four cases of excavated lesion of small intestine was not always lymphomatous. Radiologic and ultrasound semeiology are outlined and etiologies discussed. Scan images are not available since whole-body scanners are not in use in Tunisia.

[Mediterranean boutonneuse fever. Apropos of cases observed in Sousse]. / La fièvre boutonneuse méditerranéenne. A propos des cas observés à Sousse.

We report 5 confirmed cases of boutonneuse fever. All patients presented fever with a generalised maculopapular rash, and the tache noire at the site of the tick bite. Trimethoprim sulfamethoxazole failed to cure one patient. Treatment with oral oxytetracycline was effective in all cases.