[Hodgkin disease revealed by a nephrotic syndrome: A case report]. / Lymphome de Hodgkin révélé par un syndrome néphrotique : à propos d'un cas.

Pediatric nephrotic syndrome (NS) is most often idiopathic or primary but in rare cases, it can be secondary to neoplasia. We report on a case of steroid-resistant NS revealing as a paraneoplastic syndrome of Hodgkin disease (HD) in a 12-year-old boy. The onset of the NS can be earlier, later, or simultaneous to the HD. Treatment of the lymphoma allows the disappearance of the NS. In the case we observed, the diagnosis of HD was delayed because HD presented with an isolated, hilar adenopathy in the absence of retroperitoneal or peripheral locations. In children aged 10 years or more presenting with NS, steroid-resistant or otherwise, a possible paraneoplastic origin such as Hodgkin lymphoma should always be taken into consideration and eventually eliminated.

[Method for using inhalation chambers with facial masks in asthma. Evaluation in 60 children below four years of age]. / Technique d'utilisation de la chambre d'inhalation avec masque facial dans l'asthme. Evaluation chez 60 enfants âgés de moins de quatre ans.

UNLABELLED: Spacers with face masks are widely used for the treatment of asthma in young children. A poor inhalation technique may compromise the treatments efficiency. METHODS: The inhalation technique of spacers with face masks was evaluated in a prospective study of 60 children below four years of age. A checklist of 12 items was used, each one being coded by zero or one, and a total score < or = 12 points was calculated. RESULTS: Mean total score was 8.93 +/- 1.84 (extremes: 6-12). The canister was shaken before use in 48.3% of cases, one puff delivered when the child was breathing in 71.7% and the valve's mobility checked in 85%. The canister was shaken before the second puff in 13.3% of cases and two consecutive puffs individualized in 28%. DISCUSSION: The usual mistakes are lacking to shake the canister and consecutive puffs' individualization. Medical partners and families education should be reinforced.

[Paraplegia and medullary arteriovenous malformation. Role of surgery, corticosteroids and embolization]. / Paraplégie et malformation artérioveineuse médullaire. Place de la chirurgie, de la corticothérapie et de l'embolisation.

CASE REPORT: We report the case of a nine-year-old child with a cervical medullary arteriovenous malformation (AVM), revealed by total paraplegia, whose regression started during steroid therapy, before embolization. CONCLUSION: The frequency of AVM's spontaneous favorable outcome is unknown but should not be underestimated. It might be improved by steroids.

[Kikuchi-Fujimato disease]. / La maladie de Kikuchi-Fujimato.

UNLABELLED: Chronic adenopathies usually reveal lymphoma or infectious disease, particularly tuberculosis. CASE REPORT: We report a case of Kikuchi-Fujimato's disease revealed by cervical adenopathies, associated with fever and inflammatory syndrome. CONCLUSION: The diagnosis of Kikuchi-Fujimato's disease lies on histological examination. Resolution is faster with steroids.

[Hodgkin's disease limited to intrathoracic sites. Case report]. / Maladie de Hodgkin de localisation intrathoracique pure. A propos d'un cas.

UNLABELLED: Hodgkin's disease without peripheral lymphadenopathy or hepatosplenomegaly is exceptional. CASE REPORT: Hodgkin's disease was revealed by lung nodules, one of them cavitating, with mediastinal enlargement. Diagnosis was confirmed on a video-assisted pleuroscopic biopsy. CONCLUSION: Hodgkin's disease should be considered in case of mediastinal enlargement with lung nodules.

[Acute recurrent pancreatitis and heterozygous mutation of the cystic fibrosis gene: a case report]. / Pancréatite aiguë récidivante et mutation hétérozygote du gène CFTR. A propos d'un cas.

UNLABELLED: Recurrent pancreatitis is seldom associated with CFTR (Cystic Fibrosis Transmembrane Regulator) gene mutation. CASE REPORT: A 17-year-old boy presented with isolated idiopathic pancreatitis. CFTR gene mutations study revealed delta F508 heterozygous mutation. CONCLUSION: Mutations for the CFTR gene should be explored in case of recurrent pancreatitis.

[Acute respiratory insufficiency revealing myasthenia gravis: apropos of 3 cases]. / Insuffisance respiratoire aiguë révélatrice d'une myasthénie juvénile: à propos de trois cas.

BACKGROUND: Myasthenia gravis is usually revealed by a ptosis or a diplopia. A respiratory muscle weakness often occurs during the course but an acute respiratory failure as initial feature is unusual. CASE REPORTS: Three girls, aged 8, 10 and 14 years, were hospitalised in an intensive care unit, along a 15 year-period, for an acute respiratory distress. The first two children suffered from skeletal and bulbar muscle weakness. The third, admitted with the diagnosis of unexplained pneumonia, was complaining of skeletal and bulbar muscle weakness for the last 18 months. Myasthenia gravis was confirmed with electromyography, and detection of the acetylcholine-receptors antibodies in all three cases. CONCLUSION: Any unexplained acute respiratory distress must lead to search for skeletal and bulbar muscle weakness, specially after muscular exercise or at the end of day, manifestations which characterize myasthenia gravis.

[Treatment with hydroxyurea of polycythemia vera in a 11 year-old child]. / Traitement par hydroxyurée d'une polyglobulie primitive chez un enfant de 11 ans.

BACKGROUND: Polycythemia vera is a very rare disease in childhood; its treatment for this reason is not well established. CASE REPORT: An 11-year-old boy was admitted for polycythemia vera. The three classical major criteria for this condition were present and no cause for secondary polyglobulia could be recognized. The patient was given hydroxyurea. Three years later, he is well with hematocrit below 50%. CONCLUSIONS: Oral continuous myelosuppressive therapy with hydroxyurea may be proposed in children if there is no HLA-matched family member. Long-term follow-up is nevertheless necessary in order to detect relapse or side-effects of the treatment.