[Mandibular osteitis on a periapical granuloma mimicking an osteosarcoma: a case report]. / Ostéite mandibulaire sur un granulome péri-apical faisant évoquer un ostéosarcome. A propos d'un cas Clinique.

Introduction: Osteitis is an inflammatory disease of bone tissue. Its clinical presentation varies according to the site, presence or absence of pus, duration and etiology. It represents one of the endo-periodontal diseases complication. We report the case of a mandibular osteitis having led to confusion with an ostesarcoma. Observation: A 13-year-old boy was referred to our department for a right low swelling with suspicion of mandible osteosarcoma. The lesion's evolution was 6 weeks. With the local aggressiveness and the speed evolution, the first diagnosis proposed was a mandibular osteosarcoma. Based on the clinical and radiological data established, the diagnosis of mandibular osteitis on a periapical granuloma was made and the treatment consisted on the causal tooth extraction with curettage of the periapical lesion associated to the prescription of macrolide (ERY®). The clinical follow-up was 6 weeks, a recovery with cessation of clinical signs were noticed. Discussion: The differential diagnosis facing the clinical context is an osteitis on a periapical inflammatory cyst and especially mandibular osteosarcoma. For the therapy, it's a 3 components treatment (etiology, medical and surgical) to obtain a recovery.

The Antley-Bixler syndrome. A new case without radiohumeral synostosis.

The author report a new case of the syndrome described by Antley and Bixler. It concerns a female new-born who presents a craniosynostosis with brachycephaly, midface hypoplasia, unilateral choanal atresia, multiple joint contractures and arachnodactyly. There was no bowing of the femora and only the radius and the ulna were bowed. The absence of radiohumeral synostosis reported in all observations of the literature represents the main particularity of this observation.

[Wolman disease]. / La maladie de Wolman.

BACKGROUND: Wolman disease is a severe disease associated with hepatosplenomegaly and adrenal calcifications; it is nearly always fatal in the first year of life. CASE REPORT: A boy was born to consanguineous parents. His weight was 3,500 g, height 53 cm. Hepatomegaly was observed at the age of 26 days; he also had vomiting and watery stools with failure to thrive. Diagnosis of Wolman disease was suspected due to family history. Two sisters had died at the age of 3 months without precise diagnosis; both had abdominal distension, hepatosplenomegaly, anemia and inanition; CT scan showed calcifications of adrenal glands in one of them that had been attributed to adrenal hemorrhage. Investigations in our patient showed no adrenal calcification, hepatomegaly without splenomegaly, anemia (Hb: 8 g/100 ml). Liver biopsy showed enlarged and vacuolated parenchymal and Kupffer cells but the marrow did not contain foam cells. Acid lipase deficiency was demonstrated in cultured skin fibroblasts, permitting prenatal diagnosis in a further sib. CONCLUSION: This case of Wolman disease was the first seen in Tunisia; it was inherited as an autosomal recessive disorder; this patient, as two of his sisters, died during the first 6 months of life.

[Budd-Chiari syndrome in children. Apropos of 7 cases]. / Le syndrome de Budd-Chiari chez l'enfant. A propos de 7 observations.

Seven cases of Budd-Chiari syndrome are reported in children. The mode of onset was fulminant in one case with rapidly lethal liver failure, acute in 5 cases with rapid appearance of hepatomegaly and ascites and insidious in one case, with isolated hepatomegaly. Hepatomegaly, which is a constant sign, was present in the 7 patients. Ascites and collateral venous circulation were present in 6, splenomegaly in 2 and moderate jaundice in one only. Liver function tests, deeply abnormal in the patient with fulminant liver failure, was only slightly abnormal in the 6 others. Diagnosis was corroborated by ultrasonography, cavography, hepatic veins angiography and liver biopsy in 6 patients and by post mortem examination in the 7th. Etiologic investigations did not allow finding the cause of Budd-Chiari syndrome. However, this series can be distinguished by associated total villous atrophy in 3 cases, psoriasis in one, hepatitis B in one, hepatitis A and intestinal giardiasis in one. Portasystemic shunts were performed in 3 patients. One died in the immediate postoperative period, the 2 others are presently in good health with a 5 and 6 1/2 year-follow-up. One patient died rapidly from fulminant liver failure. Another, untreated, died 16 years after the onset of the disease, from an unknown cause. Two patients are lost to follow-up.

[Severe forms of liver involvement in visceral leishmaniasis. Apropos of 7 cases]. / Les formes sévères d'atteinte hépatique au cours de la leishmaniose viscérale. A propos de 7 cas.

In a series of 180 cases of Kala-azar, hepatic involvement was found in 16 patients. The authors report 7 cases of severe hepatitis with cytolysis, cholestasis and liver failure. These patients presented with high triglyceride, low cholesterol and low alpha-lipoprotein blood levels. The authors suggest that an activation of the mononuclear phagocyte system might explain these abnormalities.

[2 familial cases of homocystinuria one of which revealed by fatal hypertensive encephalopathy]. / A propos de deux cas familiaux d'homocystinurie dont l'un révélé par une encéphalopathie hypertensive mortelle.

Two cases in the same sibship are reported. The elder patient, who had posterior dislocation of the lens resulting in glaucoma and significant psychomotor retardation, died at the age of 13 with malignant arterial hypertension. Death was caused by thrombotic events (left carotid artery, coronary vessels, renal arteries and arterioles with fibrous endarteritis). The sister, aged 10, had psychomotor retardation and anomalies of both lenses. Chromatographic studies of serum and urine amino acids confirmed the diagnosis of homocystinuria. The form was pyridoxine-sensitive as shown by the results of therapy with pyridoxine and folates. We suggest that homocystinuria, although infrequent, should be routinely looked for in every child with a thrombotic event since a pyridoxine-folate combination is successful in half the cases, preventing the development of complications especially when initiated early.

[Hydatid cyst of the brain. Apropos of a series of 14 cases]. / Le kyste hydatique du cerveau. A propos d'une série de quatorze cas.

The authors report a series of 14 cases of cerebral hydatid cysts. This is a rare site (1.5% of all sites) which usually presents in the form of intracranial hypertension, sometimes associated with localizing signs, the diagnosis is no longer a problem since the development of computed tomography. Treatment is exclusively surgical and the prognosis depends on the degree of preoperative neurological impairment. Twenty-eight per cent of cases had postoperative neurological sequelae which stresses the importance of early diagnosis and treatment.

[The nephrotic syndrome in kala-azar]. / Syndrome néphrotique au cours du kala-azar.

Glomerular nephropathy is unusually known in kala-azar. We present 3 cases and we report a review on the clinical, biological and immunohistological aspects of this glomerulonephritis. The immunologic mechanisms of this parasitic nephropathy and its reversibility after specific treatment are characteristic.