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Pleural empyema is rarely seen in the newborn period. Here we report the first case of exclusive and successful conservative management of pleural empyema in an extremely low birth weight infant. Chest drainage or percutaneous aspiration was not feasible. Noninvasive treatment might represent an acceptable option in premature infants. [ABSTRACT FROM AUTHOR]

Background Intraventricular hemorrhage (IVH) is one of the most formidable neurological complications in premature infants. Low grade IVH is often regarded as a low risk factor for long-term neurological impairment. Methods We retrospectively investigated, if there was an association of low IVH grade and adverse neurological outcome in survivors at 20–24 months of corrected age. Results 89 infants, median gestational age 27 + 4, were included in the study. In total 55 children with IVH grade I ( n = 34) or grade II ( n = 21), plus 34 children with IVH grade III ( n = 13) and IVH grad IV ( n = 21) were included. In infants with grade I and II IVH median mental developmental index (MDI) and psychomotor developmental index (PDI) were 90 and 88, respectively. In total 18 children (33%) had a MDI one standard deviation below the mean (< 85, < 1SD). 9 out of these 18 had an IVH grade I. 17 infants (31%) had a PDI < 1 SD. An MDI/PDI < 2SD was only found in 7 (13%)/6 (11%) children, respectively. Regression analysis showed that IVH grade ( p < 0,001) and gestational age ( p = 0,008) were significant independent risk factors for an adverse motoric outcome. Conclusion Our collective revealed that an IVH grade I was not always associated with a good outcome. Interpretation and prediction of long outcome prognosis after IVH grade I or II requires careful consideration as it might be associated with increased disability. [ABSTRACT FROM AUTHOR]

To safeguard the cell from the accumulation of potentially harmful metabolic intermediates, specific repair mechanisms have evolved. APOA1BP , now renamed NAXE , encodes an epimerase essential in the cellular metabolite repair for NADHX and NADPHX. The enzyme catalyzes the epimerization of NAD(P)HX, thereby avoiding the accumulation of toxic metabolites. The clinical importance of the NAD(P)HX repair system has been unknown. Exome sequencing revealed pathogenic biallelic mutations in NAXE in children from four families with (sub-) acute-onset ataxia, cerebellar edema, spinal myelopathy, and skin lesions. Lactate was elevated in cerebrospinal fluid of all affected individuals. Disease onset was during the second year of life and clinical signs as well as episodes of deterioration were triggered by febrile infections. Disease course was rapidly progressive, leading to coma, global brain atrophy, and finally to death in all affected individuals. NAXE levels were undetectable in fibroblasts from affected individuals of two families. In these fibroblasts we measured highly elevated concentrations of the toxic metabolite cyclic-NADHX, confirming a deficiency of the mitochondrial NAD(P)HX repair system. Finally, NAD or nicotinic acid (vitamin B3) supplementation might have therapeutic implications for this fatal disorder. [ABSTRACT FROM AUTHOR]