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Autoinflammatory disorders are characterized by usually unprovoked recurrent episodes of features of inflammation caused by activation of the innate immune system. Many autoinflammatory disorders - the monogenetic defects in particular - are associated with alterations of inflammasomes. Inflammasomes are complex multimolecular structures, which respond to(More)
Two extremely low birth weight (ELBW) infants developed characteristic signs of kernicterus at 4 and 8 months corrected age despite only moderate neonatal hyperbilirubinemia (peak serum bilirubin <10 g/dl) and phototherapy being applied according to current guidelines. Both girls were from twin pregnancies and had fetal complications (donor in a twin-twin(More)
BACKGROUND Necrotizing enterocolitis (NEC) and focal intestinal perforation (FIP) are major causes of morbidity in infants with extremely low birth weight (ELBW). OBJECTIVE To evaluate the surgical procedures applied, and the survival and long-term outcome of ELBW infants with NEC and FIP in a single-center study. METHODS Inborn and outborn ELBW infants(More)
BACKGROUND Since 2008, follow-up examinations at 2 years of age with the standardized Bayley II test have become obligatory in Germany for all very low birth weight infants. AIM We already performed such examinations before 2006. Here, we compared our data and the completeness of our examinations before and after the introduction of the obligatory 2-year(More)
Methods A 9-year old boy, diagnosed with symptomatic MWS and evidence of a V198M mutation (father also V198M), only partially responsive to anakinra, participated in a phase III study of canakinumab in CAPS patients (N=166). Study treatment was 8-weekly administration of canakinumab by s.c. injection, 150 mg to adults and 2 mg/kg to patients ≤40 kg for up(More)