Glutaric Aciduria Type I Missed by Newborn Screening: Report of Four Cases from Three Families.

Glutaric aciduria type I (GA-1) is a rare autosomal-recessive disorder of the degradation of the amino acids lysine and tryptophan caused by mutations of the GCDH gene encoding glutaryl-CoA-dehydrogenase. Newborn screening (NBS) for this condition is based on elevated levels of glutarylcarnitine (C5DC) in dried blood spots (DBS). Here we report four cases from three families in whom a correctly performed NBS did not detect the condition. Glutarylcarnitine concentrations were either normal (slightly below) or slightly above the cut-off. Ratios to other acylcarnitines were also not persistently elevated. Therefore, three cases were defined as screen negative, and one case was defined as normal, after a normal control DBS sample. One patient was diagnosed after an acute encephalopathic crisis, and the other three patients had an insidious onset of the disease. GA-1 was genetically confirmed in all cases. Despite extensive efforts to increase sensitivity and specificity of NBS for GA-1, by adjusting cut-offs and introducing various ratios, the biological diversity still leads to false-negative NBS results for GA-1.

Correction: Spenger et al. Glutaric Aciduria Type I Missed by Newborn Screening: Report of Four Cases from Three Families. Int. J. Neonatal Screen. 2021, 7, 32.

There was an error in the original publication [...].

Phyllopertha horticola (Coleoptera: Scarabaeidae) larvae in eastern Austrian mountainous grasslands and the associated damage risk related to soil, topography and management.

The soil-dwelling larvae of several Scarabaeidae species (white grubs), like the cockchafer (Melolontha melolontha) and the garden chafer (Phyllopertha horticola), are serious pests in European cultivated grassland, reducing grass yield and destroying the turf by root-feeding. Nevertheless, the factors responsible for the development of large grub populations and the associated damage risk are poorly understood. The objectives of the study were to survey grub densities in grassland sites with different damage histories and find correlations with environmental and management variables. Data on grub densities were collected at 10 farms in the eastern Austrian Alps in September and October 2011. At each farm, one recently damaged site (high risk) and one site at which grub damage had never been observed by the farmers (undamaged site = low risk; each site: 500 m(2)) were sampled. All sites were dominated by P. horticola (99% of 1,422 collected individuals; maximum density 303 grubs/m(2)), which indicates that grub damage there is mainly caused by that species. Recently damaged sites tended to higher grub densities than undamaged sites. However, 3 out of 10 undamaged sites harbored high grub populations as well. Humus content together with the depth of the A-horizon significantly explained 38% of P. horticola grub density variance, with highest densities in deeper humus-rich soils. The risk of grub damage was positively connected to the humus content and negatively related to the cutting frequency. For the investigated mountainous grassland sites, these results suggest an important role of humus for the development of high grub densities and an effect of management intensity on grub damage.

Subsurface earthworm casts can be important soil microsites specifically influencing the growth of grassland plants.

Earthworms (Annelida: Oligochaeta) deposit several tons per hectare of casts enriched in nutrients and/or arbuscular mycorrhizal fungi (AMF) and create a spatial and temporal soil heterogeneity that can play a role in structuring plant communities. However, while we begin to understand the role of surface casts, it is still unclear to what extent plants utilize subsurface casts. We conducted a greenhouse experiment using large mesocosms (volume 45 l) to test whether (1) soil microsites consisting of earthworm casts with or without AMF (four Glomus taxa) affect the biomass production of 11 grassland plant species comprising the three functional groups grasses, forbs, and legumes, (2) different ecological groups of earthworms (soil dwellers-Aporrectodea caliginosa vs. vertical burrowers-Lumbricus terrestris) alter potential influences of soil microsites (i.e., four earthworms × two subsurface microsites × two AMF treatments). Soil microsites were artificially inserted in a 25-cm depth, and afterwards, plant species were sown in a regular pattern; the experiment ran for 6 months. Our results show that minute amounts of subsurface casts (0.89 g kg-1 soil) decreased the shoot and root production of forbs and legumes, but not that of grasses. The presence of earthworms reduced root biomass of grasses only. Our data also suggest that subsurface casts provide microsites from which root AMF colonization can start. Ecological groups of earthworms did not differ in their effects on plant production or AMF distribution. Taken together, these findings suggest that subsurface earthworm casts might play a role in structuring plant communities by specifically affecting the growth of certain functional groups of plants.