Staphylococcal necrotizing pneumonia producing the Panton Valentine leukotoxin (PVL) has been described for many years. The french reference center for staphylococcal toxaemia defined it with precision in 1999. A 10- ...Staphylococcal necrotizing pneumonia producing the Panton Valentine leukotoxin (PVL) has been described for many years. The french reference center for staphylococcal toxaemia defined it with precision in 1999. A 10- year- old child, died in 36 hours from respiratory distress and shock. Staphylococcal pneumonia was suspected then confirmed: S. Aureus producing PVL was isolated in lung, blood and articulations.展开更多
Vascular Ehlers-Danlos syndrome(EDS) is at high risk of death by arterial rupture. Case report. -A 13-year-old boy with vascular EDS and aortic dissection was admitted in pediatric emergency care unit. The children di...Vascular Ehlers-Danlos syndrome(EDS) is at high risk of death by arterial rupture. Case report. -A 13-year-old boy with vascular EDS and aortic dissection was admitted in pediatric emergency care unit. The children died after surgery by massive hemorrhage and prothetic rupture. Discussion. -The precocity of this vascular accident EDS is uncommon. It occured despite clinical and echocardiographic follow-up. The death of the children confirmed the difficulties in surgery of vascular manifestation, even if appropriate and rapid management was already done. Conclusion. -Patients with vascular EDS must be identified because of its lethal complications. Friability of the vessels makes surgical treatment difficult. A trial using beta blockers therapy is ongoing.展开更多
Congenital galactosaemia reveals usually in the second and third weeks of life with a severe liver dysfunction.We report on a case of congenital galactosaemia with, on the one hand, an early onset liver failure, witho...Congenital galactosaemia reveals usually in the second and third weeks of life with a severe liver dysfunction.We report on a case of congenital galactosaemia with, on the one hand, an early onset liver failure, without any free interval, and on the other hand, an hemophagocytic syndrome as a severe secondary outbreak with pulmonary haemorrhage.Appropriate diet led to normalisation of liver function.Hemophagocytosis, probably linked to an associated Klebsiella Pneumoniae sepsis, had a favourable outcome after antibiotic and corticosteroid therapy.展开更多
文摘Staphylococcal necrotizing pneumonia producing the Panton Valentine leukotoxin (PVL) has been described for many years. The french reference center for staphylococcal toxaemia defined it with precision in 1999. A 10- year- old child, died in 36 hours from respiratory distress and shock. Staphylococcal pneumonia was suspected then confirmed: S. Aureus producing PVL was isolated in lung, blood and articulations.
文摘Vascular Ehlers-Danlos syndrome(EDS) is at high risk of death by arterial rupture. Case report. -A 13-year-old boy with vascular EDS and aortic dissection was admitted in pediatric emergency care unit. The children died after surgery by massive hemorrhage and prothetic rupture. Discussion. -The precocity of this vascular accident EDS is uncommon. It occured despite clinical and echocardiographic follow-up. The death of the children confirmed the difficulties in surgery of vascular manifestation, even if appropriate and rapid management was already done. Conclusion. -Patients with vascular EDS must be identified because of its lethal complications. Friability of the vessels makes surgical treatment difficult. A trial using beta blockers therapy is ongoing.
文摘Congenital galactosaemia reveals usually in the second and third weeks of life with a severe liver dysfunction.We report on a case of congenital galactosaemia with, on the one hand, an early onset liver failure, without any free interval, and on the other hand, an hemophagocytic syndrome as a severe secondary outbreak with pulmonary haemorrhage.Appropriate diet led to normalisation of liver function.Hemophagocytosis, probably linked to an associated Klebsiella Pneumoniae sepsis, had a favourable outcome after antibiotic and corticosteroid therapy.
