Résumé
The aim of this study is to evaluate the efficacity of immunosuppressive therapy in steroid - resistant idiopathic nephrotic syndrome. 30 children present to the renal biopsy a focal and segmental glomerulosclerosis [HSF 18 cases], minimal change disease [LGM: 9 cases] and diffuse mesangial proliferation [PMD: 3 cases]. 9 of 11 patients did not respond to cyclophosphamid. 28 patients received the cyclosporine - prednisone combinaison with a cyclosensibility in 23 patients [80 per cent]. The mycophenolate mofetil [MMF] was used in 7 patients, and 3 were in remission. In this study 7 patients [23 per cent] develop a chronic renal failure. the cyclophosphamid has not beneficial effects. The cyclosporine is an effective therapeutic agent. MMF has emerged as an important new therapeutic option for the treatment of SNCR and HSF
Sujets)
Humains , Mâle , Femelle , Syndrome néphrotique/congénital , Néphrose/étiologie , Enfant , Néphrose/traitement médicamenteux , Immunosuppresseurs , Stéroïdes , Résistance aux substances , Cyclophosphamide , Ciclosporine , Acide mycophénolique/analogues et dérivés , Glomérulonéphrite segmentaire et focaleRésumé
The acute ethmoiditis in children is a severe bacterial infection which may spread to the orbital or endocranial spaces. The authors report 5 cases, 4 cases with periorbital cellulitis, and a case revealed by fever and convulsions. The CT scam confirm the diagnosis. Two infants have an alone ehtmoiditis, three children have an ethmoiditis associated with paranasal sinusitis, and complicated with an intracranial infection in a case. Bacteria were isolated from only one child [staphylococcus]. The evolution were favorable in all cases, with antimicrobial therapy. The authors insist on the CT imaging in emergency, and the early and effective antimicrobial therapy
Résumé
Galloway - Mowat syndrome is a rare autosomal recessive disorder characterized by early onset nephrotic syndrome and central nervous system anomalies. We report two boys, born to consanguineous parents, who presented respectively at 32 months and 9 years of age a steroid-resistant nephrotic syndrome. The kidney biopsy showed, in the 2 cases, a focal and segmental glomerulosclerosis. At presentation they have a microcephaly, a mental retardation. A brain magnetic resonance imaging scan showed cortical atrophy, mega citerne, and white matter anomality in a case. One boy died at 3,5 years of age from end-stage renal failure, the other had 12 year old and on chronic hemodialysis. The particularity of the two cases is the nephrotic syndrome onset after 2 years of age