Aim:  Aim of the study is to investigate the frequency of and predictors for rehospitalization within the first 2 years of life among preterm infants. Methods:  All children born before 32 weeks of gestation in Northern Tyrol between January 2003 and July 2008 were prospectively enrolled. Data on rehospitalizations were obtained from hospital admission records. The association between candidate risk factors and readmission was analysed by means of logistic regression analysis. Results:  In the first and second years of life, 151 and 93 of 377 children (40.1% and 24.7%), respectively, were readmitted to one of the hospitals in Northern Tyrol. The most common causes of rehospitalization were respiratory disorders, accounting for 42.1% and 47.4% of total readmissions in the first and second years of life. Chronic lung disease (CLD), male sex and smoking in pregnancy were risk conditions relevant to readmission in the first year of life, but only CLD in the second year. Conclusion:  Infants born before 32 weeks of gestation have a high risk of rehospitalization with respiratory illness significantly contributing to postdischarge morbidity. Neonatal intensive care should aim to further improve respiratory health in preterm infants, and adequate follow‐up services must be offered.

This study identified distinct risk factors for adverse outcome in preterm infants of lower (<30 weeks) and higher (30-32 weeks) gestational age. In the lower gestational age group, neonatal risk predictors are most important. Antenatal steroids appear to decrease the risk for adverse outcome in both age groups.

BackgroundSince 1980, about 100 types of congenital disorders of glycosylation (CDG) have been reported representing an expanding group of inherited disorders. ALG8-CDG (= CDG-Ih) is one of the less frequently reported types of CDG, maybe due to its severe multi-organ involvement with coagulation disturbances, edema, massive gastrointestinal protein loosing enteropathy, cataracts, and often early death. We report three additional patients, provide an update on two previously reported, and summarize features of ten patients reported in literature.ResultsOf 15 ALG8-CDG patients, three were homozygous and 12 compound heterozygous. There were multiple prenatal abnormalities in 6/12 patients. In 13/15, there were symptoms at birth, 9/15 died within 12 months. Birth weight was appropriate in 11/12, only one was small for gestational age. Prematurity was reported in 7/12. Hydrops fetalis was noticed in 3, edemas in 11/13; gastrointestinal symptoms in 9/14; structural brain pathology, psychomental retardation, seizures, ataxia in 12/13, muscle hypotonia in 13/14. Common dysmorphic signs were: low set ears, macroglossia, hypertelorism, pes equinovarus, campto- and brachydactyly (13/15). In 10/11, there was coagulopathy, in 8/11 elevated transaminases; thrombocytopenia was present in 9/9. Eye involvement was reported in 9/14. CDG typical skin involvement was reported in 8/13.ConclusionIn ALG8-CDG, isoelectric focusing of transferrin in serum or plasma shows an abnormal sialotransferrin pattern. The diagnosis is confirmed by mutation analysis in ALG8; all patients reported so far had point mutations or small deletions. The prognosis is generally poor. Thus, a timely and correct diagnosis is important for counselling.

Aim:  To provide comprehensive data on potential sex differences in maternal and neonatal characteristics, short‐term morbidity and neurodevelopmental outcome within an entire geographically determined collective of infants born at a gestational age <32 weeks. Methods:  Between 2003 and 2008, we prospectively enrolled all infants born in Tyrol at <32 weeks of gestation; the association between sex, and a wide set of pre‐ and postnatal factors, post‐discharge morbidity and neurodevelopmental outcome was analysed. Results:  Girls less frequently suffered from early‐onset sepsis than boys (p = 0.030). After adjustment for multiple corrections (Bonferroni’s p = 0.003), no sex differences were seen within any maternal or neonatal parameter. Analysis of morbidity revealed a higher readmission rate in boys (p < 0.0001), which was primarily caused by a greater incidence of respiratory problems (p = 0.003). Boys did not show a greater adverse neurodevelopmental outcome at the age of 12 or 24 months. Conclusion:  Parents of boys should be prepared for a potentially higher frequency of readmission after initial discharge, but our data currently give no reason for parents of boys to be disproportionately anxious about their neurodevelopmental outcome. Whether boys also enjoy a rosy prognosis for developmental outcome at school age remains to be elucidated.