[Congenital hepatic fibrosis in children: about 9 cases and review of the literature]

Congenital hepatic fibrosis is a recessive autosomic disease with two major risks: gastrointestinal haemorrhage caused by portal hypertension and cholangitis related to bacterial infection of dilated intrahepatic bile ducts. The aim of our study is to define epidemiological features, the presenting symptoms, the diagnosis, the evolution and the management of this disease. Between January 1990 and December 2000, we reported the cases of nine children with this disease at children hospital of Tunis.Three were male and six female. The mild age was three years and six months. Consanguinity was present in five cases and similar cases were found in six cases. The FHC was revealed by portal hypertension in five cases, angiocholitis in one case and by portal hypertension and angiocholitis in three cases. Liver biopsy was done in seven children. Ultrasound examination of the liver and kidney revealed caroli syndrome in five cases and polykystose renal in two cases The intravenous pyelography was performed in four cases showing precalicial canalicular ectasia in four cases. Eosophageal endoscopy had shown oesophageal varices in six patients. The follow up had shown that three patients had gastrointestinal bleeding, three had angiocholitis. One patient died with multivisceral failure. The treatment of acute bleeding has needed blood transfusion in four cases. Primary prevention of bleeding was done by endoscopic sclerosis alone in one case and associated to betablokers in two cases. Secondary prevention of varices bleeding was done by sclerotherapic in two cases, by beta blokers alone in one case and by betablokers associated to elastic ligation of oesophageal varices in one case

kyste hydatique du poumon chez l'enfant a propos de 54 cas

The purpose of this retrospective study is to clarify the clinical, radiological and evolutionary aspects of the hydatid cyst of the lung. Over a period of 9 years [January, 1983 - December, 2001], we brought together in the pediatric service B of Children's hospital of Tunis, 54 cases of hydatid cyst of the lung. The average age is of 7 years 3 months [extremes 2 and 14 years], and the sex- ratio of 1, 16. 61% of the children are of rural origin. Bronchpulmonary infection is the most frequent circumstance of discovery [72% of cases], hemoptysis is revealing in 37% of cases. Discovery is fortuitous in 7% of cases. The radiography of the chest is of a big diagnostic contribution. The radiological aspects obtained are: a homogeneous opaqueness [61%], a diverse opaqueness [26%], an image of lung abscess [22%], an aspect of floating membrane [4%], a pleural effusion [9%], and an opaqueness with growing gas [4%]. Chest echography was contributory in 77% of cases. 74% of the children were operated without complications. The others were lost

[Chronic septic granulomatosis in children revealed by invasive pulmonary aspergillosis]

Chronic granulomatosis disease [GCD] is a hereditary abnormality of phagocytic cells, frequently revealed by invasive pulmonary Aspergillosis. The authors report the case of 8 year old girl investigates for hypotrophia, recurrent lung disease and hemoptysia. She was treated for pulmonary tuberculosis because of biological inflammatory syndrome, right alveolar, opacity and positive tuberculin skin test. The course was marked by the development of a thoracic wall mass. Anti- aspergillus serology was positive; culture of the thoracic mass puncture and bronchoalveolar lavage isolated fumigatus Aspergillus. GCD was suggested and confirmed by the NBT test

Lymphome T primaire du pancreas chez l'enfant

Involvement of the gastrointestinal tract is frequently reported among the extranodaI sites of non-Hodgkin's lymphoma, but primary lymphoma of the pancreas is very rare. We report the case of an 11-year-old boy explored for epigastric pain with fever leading to the diagnosis of high-grade primary non Hodgkin's T-cell lymphoma originating from pancreas. This pancreatic lesion is documented by sonography, CT and MRI. This patient died quickly with severe infection

Premiere crise convulsive de l'enfant. Strategie d'exploration

We have curried out a retrospective study on 51 children aged between 2 years 3 month and 13 years in order to determine the findings and to define the indications of neuroimaging modalities in children with a first unprovoked seizure. Children who had neuroimaging studies were divided into two groups based on the results of neuroimaging normal or abnormal. We have compared the two groups according to each clinical and electroencephalographic parameters studied. Neuroimaging abnormalitie were found in 47% of cases. According to our results and literature review, we concluded that the imaging should be done if we have at least one of the criteria: an abnormal neurological examination after the seizure and focal slowing waves on the electroencephalogram

Osteoarthropathie hypertrophiante revelant un thymome malin chez un enfant. Apport de l'imagerie

Association of hypertrophic osteoarthropathy and malignant thymoma is very rare in children. Authors report a case of a 14 year old girl followed for rheumatic fever since she was 8 years old. She was explored for arthralgia. The exam showed Hippocratic fingers, soft tissues hypertrophy of lower limbs and dyspnoea. Plain radiography had demonstrated symmetrical ossifying periostisis in tubular bones of lower limbs evocating hypertrophic osteoarthropathy diagnosis. Chest radiography has demonstrated bilobate anterior mediastinal mass. Ultrasonography and MRI revealed a voluminous anterior mediastinal mass with cystic and fleshy components. Thymic adenocarcinoma diagnosis was made by surgical biopsy