Investigation of primary immune deficiency after severe bacterial infection in children: A population-based study in western France.

BACKGROUND: Infectious diseases are still an important cause of morbidity and mortality in high-income countries and may preferentially affect predisposed children, especially immunocompromised children. We aimed to evaluate the frequency of recommended immunological tests in children with community-onset severe bacterial infection (COSBI) admitted to a pediatric intensive care unit. We also assessed the frequency and described the typology of diagnosed primary immune deficiency (PID). METHODS: We conducted a retrospective observational epidemiological study in six university hospitals in western France. All children from 1 month to 16 years of age admitted to hospital for bacterial meningitis, purpura fulminans, or meningococcal disease between August 2009 and January 2014 were included. We analyzed the frequency, type, and results of the immunological tests performed on children with meningitis, purpura fulminans, or a meningococcemia episode. RESULTS: Among the 143 children included (144 episodes), 84 (59%) and 60 (41%) had bacterial meningitis and purpura fulminans or meningococcemia, respectively: 72 (50%) had immunological tests and 8% had a complete immunological investigation as recommended. Among the 72 children examined for PID, 11 (15%) had at least one anomaly in the immunological test results. Two children had a diagnosis of PID (one with C2 deficit and the other with C8 deficit) and seven other children had possible PID. Thus, the prevalence of a definite or possible diagnosis of PID was 12% among the children examined. CONCLUSION: PID is rarely investigated after COSBI. We raise awareness of the need for immunological investigations after a severe infection requiring PICU admission.

[Malignant pertussis and exchange transfusion]. / Coqueluche maligne et exsanguino-transfusion.

CASE REPORT: Malignant pertussis is a critical clinical state associated with fatal outcome in 70% of cases. The severity criteria are a lung infection with pulmonary hypertension and hyperleukocytosis usually above 50 G/L. We report the case of a 2.5-month-old girl hospitalized with critical pertussis in a pediatric intensive care unit. She had acute respiratory distress syndrome with pulmonary hypertension complicated by a bacterial secondary infection with Enterobacter cloacae managed by high-frequency oscillatory ventilation associated with pulmonary vasodilatation therapy. In the absence of clinical improvement and before considering extracorporeal life support, exchange transfusion was performed at day 9 to reduce hyperleukocytosis at 70 G/L. Exchange transfusion was successfully performed with a reduction of leukocytes to under 40 G/L followed by steady improvement of pulmonary function. Weaning from mechanical ventilation and discharge took place at day 23 and 38, respectively. COMMENTS: Exchange transfusion should be considered in infants suffering from malignant pertussis with extreme leukocytosis before hemodynamic failure to improve the survival prognosis.

[Atrial chaotic tachycardia during a respiratory tract infection due to NL63 coronavirus]. / Tachycardie atriale chaotique au cours d'une infection respiratoire à coronavirus NL63.

We report the case of a 3-month-old boy hospitalized with acute bronchiolitis. Respiratory distress was associated with cardiogenic shock caused by chaotic atrial tachycardia. The cause of bronchiolitis was a coronavirus NL63 viral infection, confirmed in nasopharyngeal aspirations. The patient required intensive care including diuretics (furosemide), anti-arrhythmic drugs (amiodarone and digoxin), and inotropic drugs (milrinone and levosimendan) associated with mechanical ventilation. The outcome was favorable in 10 days and the sinusal cardiac rhythm was completely restored at discharge.

[Enzyme replacement therapy in a boy with infantile Pompe disease: cardiac follow-up]. / Enzymothérapie substitutive chez un nourrisson atteint de maladie de Pompe : évolution cardiologique.

Pompe disease is an autosomal recessive glycogen storage disorder caused by acid-alpha-glucosidase deficiency. The infantile form is usually fatal by 1 year of age in the absence of specific therapy. We report the cardiac follow-up of a 4-month-old boy treated with enzyme replacement therapy (ERT) for 8 months. The patient had no cardiac failure at the age of 1 year. Before starting ERT, ECG showed a shortened PR interval, with huge QRS complexes and biventricular hypertrophy; echocardiography demonstrated major hypertrophic cardiomyopathy. The QRS voltage (SV1+RV6) decreased from 13 to 2.9 mV after 32 weeks of ERT, suggesting a progressive reduction of cardiac hypertrophy and intracellular glycogen excess. The PR interval increased from 60 to 90 ms. A block of the right bundle branch appeared after 13 weeks of treatment. The indexed left ventricular mass decreased from 240 to 90 g/m2 after 30 weeks of ERT. The left ventricular ejection fraction decreased transitorily between the 5th and the 15 th weeks of treatment. In summary, ERT is an efficient therapeutic approach for the cardiomyopathy of infantile Pompe disease. However, the possible occurrence of a right bundle branch block and a transitory alteration in the ejection fraction highlight the importance of cardiac follow-up.

Plasma concentrations of prostaglandins E2 and F2a in asthmatic patients.

Plasma concentrations of prostaglandins E2 (PGE2) and F2a (PGF 2a) are studied in venous and arterial blood in 14 healthy subjects and 32 asthmatic patients. In the asthmatic patients we found: (1) a good correlation between PGE2 concentration in venous blood and the seriousness of the airway obstruction; (2) a good correlation between PGE2 and PGF2a in the arterial blood, but not in the venous blood; (3) a decrease in the arterial concentrations of PGE2 and PGF2a after fenoterol-induced bronchodilatation, but the variations of PGE2 and PGF2a still correlate. None of these results were obtained in normal subjects. It seems that the asthmatic allergic patient has a disorder of the metabolism of prostaglandins in the lungs; however, the results obtained do not allow us to say if it is a causal condition or a metabolic consequence of the bronchospasm. Concerning the venous blood, there could be an increase in the peripheral production of PGE2 which might result from the hypoxemia following airway obstruction.

[Prostaglandin biosynthesis in psoriatic epidermis (author's transl)]. / Biosynthèse des prostaglandines dans l'épiderme psoriasique.

The biosynthesis of prostaglandins E2 (PGE2) and F2 alpha (PGF2) from exogenous arachidonic acid (AA) was investigated in 12 involved and uninvolved psoriatic epidermis and in 6 normal epidermis. PGE2 and PGF2 were determined by radio-immuno-assay after addition of 25 microgram of AA and 1 hour incubation. The biosynthesis of PGE2 and PGF2 alpha was higher in psoriatic epidermis than in control epidermis but lower in involved than in uninvolved epidermis. The ratio PGE2/PGF2 alpha is decreased in involved epidermis when compared with uninvolved epidermis and with controls. These quantitative and qualitative disturbances of the PG metabolism in psoriatic epidermis might play an important role in the pathogenesis of the disease. They could be responsible for abnormal keratinocyte differentiation and proliferation, lipid membrane abnormalities and abnormal immune response of psoriatic patients.