Aim of the study. -To describe epidemiological features of an outbreak of flu A in hospitalized children and to evaluate the flu’s burden in pediatric and p ediatric emergencies departments. Population and methods. -...Aim of the study. -To describe epidemiological features of an outbreak of flu A in hospitalized children and to evaluate the flu’s burden in pediatric and p ediatric emergencies departments. Population and methods. -Multicenter prospect ive study in 2 pediatric university hospitals, in Marseille, France. Nasal swabs for flu A were performed in all the febrile children admitted during winter 200 3-2004. Pre and postoutbreak admissions in pediatric and pediatric emergencies departments were compared too. Results. -During the outbreak, 941 children were admitted and 605 were tested for flu A. Nasal swabs were positive in 111. Attac k’s rate was 11.8%. In these children, infants under 2 years were 66%. Respir atory forms were uncommon, although febrile seizures and digestive troubles were much frequent. The mean hospitalization duration was almost 4 days. Flu A outbr eak finally increased the pediatric and pediatric emergencies departments rates of admission, of 48%and 37%respectively. Conclusion. -Flu is frequently under estimated in children, especially in infants. Clinical forms are various. Child ’s vaccination is questionable in France.展开更多
Background. Pseudoxanthoma elasticum (PXE) is normally associated with mutations in the ABCC6 gene. A PXE phenotype without mutations in ABCC6 has been described in Greek and Italian patients presenting with beta thal...Background. Pseudoxanthoma elasticum (PXE) is normally associated with mutations in the ABCC6 gene. A PXE phenotype without mutations in ABCC6 has been described in Greek and Italian patients presenting with beta thalassemia. We attempted to determine the incidence of beta thalassemia in a cohort of French patients with PXE. Patients and methods. Fifty patients with PXE were included in the study. Laboratory examinations comprised hemoglobin electrophoresis, ABCC6 gene study and in some studies: mutation analysis, beta-globin gene. Results. No cases of beta thalassemia were diagnosed in this cohort of French patients with PXE. However, 20%of the latter exhibited a significant but isolated (i.e. without microcytic anemia) increase of hemoglobin A2 (HbA2). Statistical comparisons showed no difference in terms of geographical origin or severity of PXE between patients with high levels of HbA2 and those with normal levels of HbA2 other than the extent of cutaneous involvement. Study of the beta-globin gene displayed mutations only in the two patients with the highest recorded levels of HbA2. ABCC6 +beta-globin digenism was ruled out of the pathogenesis of PXE. Discussion. The PXE phenotype seen in some patients with beta thalassemia appears to be associated with epigenetic modification of ABCC6 transcription and depends specifically on the beta globin locus. Isolated increase in HbA2 is probably a laboratory marker for PXE. Here again, a functional epigenetic reaction between ABCC6 and the beta-globin locus was suspected. However, these reciprocal interactions are clearly unequal since the change in ABCC6 transcription occurring during the course of beta thalassaemia is responsible for a phenotype while increased HbA2 during the course of PXE has no clinical consequences.展开更多
文摘Aim of the study. -To describe epidemiological features of an outbreak of flu A in hospitalized children and to evaluate the flu’s burden in pediatric and p ediatric emergencies departments. Population and methods. -Multicenter prospect ive study in 2 pediatric university hospitals, in Marseille, France. Nasal swabs for flu A were performed in all the febrile children admitted during winter 200 3-2004. Pre and postoutbreak admissions in pediatric and pediatric emergencies departments were compared too. Results. -During the outbreak, 941 children were admitted and 605 were tested for flu A. Nasal swabs were positive in 111. Attac k’s rate was 11.8%. In these children, infants under 2 years were 66%. Respir atory forms were uncommon, although febrile seizures and digestive troubles were much frequent. The mean hospitalization duration was almost 4 days. Flu A outbr eak finally increased the pediatric and pediatric emergencies departments rates of admission, of 48%and 37%respectively. Conclusion. -Flu is frequently under estimated in children, especially in infants. Clinical forms are various. Child ’s vaccination is questionable in France.
文摘Background. Pseudoxanthoma elasticum (PXE) is normally associated with mutations in the ABCC6 gene. A PXE phenotype without mutations in ABCC6 has been described in Greek and Italian patients presenting with beta thalassemia. We attempted to determine the incidence of beta thalassemia in a cohort of French patients with PXE. Patients and methods. Fifty patients with PXE were included in the study. Laboratory examinations comprised hemoglobin electrophoresis, ABCC6 gene study and in some studies: mutation analysis, beta-globin gene. Results. No cases of beta thalassemia were diagnosed in this cohort of French patients with PXE. However, 20%of the latter exhibited a significant but isolated (i.e. without microcytic anemia) increase of hemoglobin A2 (HbA2). Statistical comparisons showed no difference in terms of geographical origin or severity of PXE between patients with high levels of HbA2 and those with normal levels of HbA2 other than the extent of cutaneous involvement. Study of the beta-globin gene displayed mutations only in the two patients with the highest recorded levels of HbA2. ABCC6 +beta-globin digenism was ruled out of the pathogenesis of PXE. Discussion. The PXE phenotype seen in some patients with beta thalassemia appears to be associated with epigenetic modification of ABCC6 transcription and depends specifically on the beta globin locus. Isolated increase in HbA2 is probably a laboratory marker for PXE. Here again, a functional epigenetic reaction between ABCC6 and the beta-globin locus was suspected. However, these reciprocal interactions are clearly unequal since the change in ABCC6 transcription occurring during the course of beta thalassaemia is responsible for a phenotype while increased HbA2 during the course of PXE has no clinical consequences.
