It is reported about a case of congenital afirinogenaemia on a newborn female. The treatment is described. Congenital afirinogenaemia is a rare autosomal recessive disorder. There are 250 cases in world literature, mostly with consanguineous parents. Bleeding is usually mild, so that treatment should not be prophylactic, because any blood product might cause complication, like AIDS. Only acute haemorrhage should be treated with fresh frozen plasma or fibrinogen concentrates.

Attention-deficit/hyperactivity disorder may have substantial impact on family life, peer interactions, and quality of life. Stimulants are recommended as first-line pharmacotherapy for ADHD. OROS(®) MPH (Concerta(®)) is a long-acting preparation with duration of effect for up to 12 h. In this 8-week, prospective, open-label, non-interventional trial the impact of therapy with OROS(®) MPH on functioning in four different areas of life (school, recreation, family life, and peer interaction), severity of disease, and quality of life (QoL) as well as tolerability were investigated under daily routine care. 306 patients, aged 10.2±2.3 years, were either transitioned to OROS(®) MPH from short-acting, immediate-release MPH (-IR) preparations (n=231; 75%), or treatment was initiated with OROS(®) MPH in MPH-naïve patients (n =75; 25%). In both groups, therapy with OROS(®) MPH was associated with significant improvements in daily functioning, severity of disease, and QoL. Adverse events (AE) were documented in 160 patients (52.3%). In 95 patients (31.0%) a causal relationship was assessed as at least possible. Four serious AEs were reported in 2 patients and rated as doubtfully related to study medication. Most frequent AEs (≥5% of patients) were insomnia, anorexia, ineffectiveness of medication, and headache. In 12.1% of patients AE led to discontinuation of study participation. Considering the limitations of this non-interventional study, the results refer to the importance of a therapy that covers not only school-time, but also takes other areas of life into account. Initiating treatment with long-acting preparations, such as OROS(®) MPH in MPH-naïve patients might be a feasible option.

Zusammenfassung Bedingt durch den hohen Stoffwechselumsatz immunkompetenter Zellen wird die Infektanf?lligkeit des kindlichen Organismus entscheidend von der Mikron?hrstoffzufuhr bestimmt. In einer nicht-interventionellen Beobachtungsstudie mit 542 Kindern ab 4 Jahren wurde deshalb gepr?ft, inwieweit die Applikation einer Mikron?hrstoffkombination, konzipiert f?r Kinder mit rezidivierenden Infektionskrankheiten, mit einem klinisch-immunologischen Nutzen verbunden ist. Im Beobachtungsverlauf verbesserten sich in der Gruppe mit Ern?hrungsmedizin (EG) alle untersuchten Parameter deutlich st?rker als in der Kontrollgruppe; der Gruppenunterschied bei H?ufigkeit, Dauer und Intensit?t der Infekte war f?r alle dokumentierten Infektionsarten ? mit Ausnahme der Pneumonien ? signifikant (p < 0,0001, p < 0,05). Auch beim Patientenbefinden wurden in der EG in allen Kategorien (Immunsystem, Ern?hrungszustand, Konzentrationsf?higkeit, k?rperlicher Allgemeinzustand, subjektives Befinden und Appetit) statistisch signifikante Verbesserungen beobachtet (p < 0,0001).