[Digestive manifestations of hyperoxaluria]

Primary hyperoxaluria is a rare hereditary disease caracterised by deposits of calcium oxalate in different tissues, essentially the kidney. The digestive localization was exceptional. We reported two childs who are aged respectively 13 and 16 years; they are admitted for end stage renal failure caused by primitive hyperoxaluria. The diagnosis was suspected because there were consanguinity, nephrolithiasis in the two cases and nephrocalcinosis in one. It is confirmed by increase oxalic and oxaluric levels. The hemodialysis was started. After some years, the two patients developed crystal deposition in her digestive wall

[Canavan disease or n-acetyl aspartic aciduria [one case]]

Canavan's disease [CD] or N-Acetylaspartic aciduria, is an autosomal recessive leukodystrophy characterized by spongy degeneration of brain. The disease is an inborn error of metabolism caused by aspartoacylase deficiency resulting from accumulation of N- acetylaspartic acid in the brain. The authors report a case in a ten-month old boy who presented with developmental delay and megalencephaly noticeable afterfour months of age. Magnetic resonance imaging of the brain show diffuse white matter degeneration. The diagnosis of CD was confirmed by nuclear magnetic resonance spectroscopy and gas chromatography-mass spectrometry

[Diabetes in chronic pancreatitis- about 22 cases]

The aim of our study was to determine the diabetes profile in chronic pancreatitis, through an analysis of 22 chronic pancreatitis cases seen over 14 years in the service of gastroenterology. There were 6 men and 2 women. The average age is 50 years, the age oscillating between 20 and 67 years. The diabetes revealed chronic pancreatitis in 5 cases. These patients were alcoholic. The diabetes appeared in average after 4 years of evolution. Seven patients had pancreatic calcifications. This diabetes was insulin dependent in 4 cases. Through the reported cases, a literature review is done in order to point on features of diabetes when occurring with chronic pancreatitis

[Purulent pleural effusion in children. About 47 cases]

The authors report retrospectively 47 cases of purulent pleural effusion, collaged in paediatrics department CHU Sahloul Sousse in 11 years [1994-2004]. It's about 30 boys and 17 girls. The symptomatology was dominated by fever, cough and dyspnea. The bacteria is identified in 18 cases: staphylococcus in 11 cases, streptococcus pneumonia in 3 cases and haemophilus in 3. The treatment was based in double antibiotherapy associated with kinetherapy in all cases; the drainage was required in 15 cases. The outcome is good in 38 cases; 7 cases of sequelle were noted. Two children were dead

Precocious puberty and hypothalamic hamartoma

Central precocious puberty is exceptionally due to hypothalamic hamartoma in girls under one year of age. A small girl hospitalized for exploration of precocious puberty started since the age of 7 months. Exploration of the hypothalamic-pituitary axis confirmed the central origin of this puberty. Magnetic resonance imaging [MRI] showed a small pediculate hypothalamic hamartoma [HH]. Evolution under triptorelin [recoil of 6 years] was favourable with notably the secondary sexual characters disappearance, the slowing of the speed of growth and the bony maturation. Central precocious puberty must make search HH even in girl. Its pathogenesis again controversial, seem to be in this age probably multifactorial. Our observation illustrates the efficiency of long-acting GnRH agonists during the central precocious puberty due to HH even in infantile form

Imaging features in disseminated BCG infection. Report of two family cases

The dissemined BCG infection is a rare and serious post BCG vaccinal complication, occuring mostly in patients with immunity disorder. We presented two family cases of dessimined BCG infection with an underling chronic septic granulomatosis. Diagnosis of BCG infection was established on imaging features of a multifocal involvement: osteoarticular, visceral, and lymphadenopathy. Actually, CT scan and MRI allow diagnosis extension and etiology of multifocal involve based on puncture and/or biopsy. Usually no bacteriological evidence of Bacillus Calmette Guerin is obtained on culture in 50% of cases

[Radioclinic aspects of pediatric lumbar disc herniation. Report of 12 cases]

12 cases of disc herniation in pediatric age group are reported. All patients were teenagers [mean 14 years and 6 months]. Trauma was noted in 50 % of cases. Diagnosis was made with 4 months delay. Severe back pain with sciatica associated with spinal stiffness and pseudo-scoliotic attitude were noted in 10 cases. Imaging modalities included CT Scan and myelography demonstrated the disc herniation and radicular compression in all cases. Congenital spinal abnormalities such narrowed central canal and lateral recess were noted in 3 cases and a listel avulsion in 3 cases. L4-L5 and L5-S1 levels are the most common sites involved. 9 children had surgery and 2 hexatrione chimionucleolysis resultats were excellent in 83 % of cases. Disc herniation is a rare pathology in pediatric age group and teenagers; however, frequency is increased because of the intensevly sport practice among young people. Its main characteristics were predominance of trauma, congenital abnormalities spinal, marked physical complaints, an easy CT scan diagnosis and better therapeutic results