Microcirculatory assessment of red blood cell transfusion in children with severe anemia

Background: Pediatric hematology patients frequently receive red blood all transfusions for severe anemia. Our goal was to assess the effect of blood transfusion on the microcirculation and thus provide information on the quality of tissue perfusion. Methods and patients: The sublingual microcirculation was visualized with Sidestream-Darkfield Imaging in 20 anemic (Hb: 7.2 g/dL, 95% CI 6.6-7.9) children receiving red blood cell transfusions and in age matched healthy non-anemic controls. Functional vessel density (FVD) was determined with a semiautomatic program. Results: Immediately after transfusion FVD increased (13.4 versus 15 mm/mm2) and RBC velocity (696 (598-792) versus 628 (549-707) μm/s) decreased but FVD was always significantly lower and RBC velocity was always higher than in the age matched control group (FVD 17 mm/mm2; RBC velocity: 486 (441-530) μm/s). FVD at baseline was lower in patients with infections but with a larger increase after transfusion compared to anemic children without infections (∆FVD 3.4 versus ∆FVD 1.3 mm/mm2). Hemoglobin levels and capillary density correlated well. We did see a larger rise in FVD with transfusion of RBCs aged < 12 days. Conclusion: Whereas conventional monitoring methods may not be able to assess the effect of therapies aimed to improve tissue perfusion, SDF imaging can demonstrate improvements after transfusion but also continuous differences to non-anemic controls. In particular, the microcirculation of anemic oncology patients with infection improves after transfusion. Transfusion thresholds might need to be set higher in in such patients and fresh RBCs < 12 day of storage should be used

Microcirculatory assessment of red blood cell transfusion in children with severe anemia

Background: Pediatric hematology patients frequently receive red blood all transfusions for severe anemia. Our goal was to assess the effect of blood transfusion on the microcirculation and thus provide information on the quality of tissue perfusion. Methods and patients: The sublingual microcirculation was visualized with Sidestream-Darkfield Imaging in 20 anemic (Hb: 7.2 g/dL, 95% CI 6.6-7.9) children receiving red blood cell transfusions and in age matched healthy non-anemic controls. Functional vessel density (FVD) was determined with a semiautomatic program. Results: Immediately after transfusion FVD increased (13.4 versus 15 mm/mm2) and RBC velocity (696 (598-792) versus 628 (549-707) μm/s) decreased but FVD was always significantly lower and RBC velocity was always higher than in the age matched control group (FVD 17 mm/mm2; RBC velocity: 486 (441-530) μm/s). FVD at baseline was lower in patients with infections but with a larger increase after transfusion compared to anemic children without infections (∆FVD 3.4 versus ∆FVD 1.3 mm/mm2). Hemoglobin levels and capillary density correlated well. We did see a larger rise in FVD with transfusion of RBCs aged < 12 days. Conclusion: Whereas conventional monitoring methods may not be able to assess the effect of therapies aimed to improve tissue perfusion, SDF imaging can demonstrate improvements after transfusion but also continuous differences to non-anemic controls. In particular, the microcirculation of anemic oncology patients with infection improves after transfusion. Transfusion thresholds might need to be set higher in in such patients and fresh RBCs < 12 day of storage should be used

Fecal Microbiota Nutrient Utilization Potential Suggests Mucins as Drivers for Initial Gut Colonization of Mother-Child-Shared Bacteria

publishedVersio

Severe persistent pulmonary hypertension of the newborn and dysmorphic features in neonate with a deletion involving TWIST1 and PHF14: a case report

Abstract Background Persistent pulmonary hypertension is a well-known disease of the newborn that in most cases responds well to treatment with nitric oxide and treatment of any underlying causes. Genetic causes of persistent pulmonary hypertension of the newborn are rare. The TWIST1 gene is involved in morphogenetics, and deletions are known to cause Saethre-Chotzen syndrome. Deletions of PHF14 have never been reported in neonates, but animal studies have shown a link between severe defects in lung development and deletions of this gene. There have not, to the best of our knowledge, been any publications of a link between the genes TWIST1 and PHF14 and persistent pulmonary hypertension of the newborn, making this a novel finding. Case presentation We describe a white male neonate born at term to non-consanguineous white parents; he presented with dysmorphic features and a therapy-refractory persistent pulmonary hypertension. Array-based comparative genomic hybridization revealed the presence of a 14.7 Mb interstitial deletion on chromosome 7, encompassing the genes TWIST1 and PHF14. Conclusions The TWIST1 gene can explain our patient’s dysmorphic features. His severe persistent pulmonary hypertension has, however, not been described before in conjunction with the TWIST1 gene, but could be explained by involvement of PHF14, consistent with findings in animal experiments showing lethal respiratory failure with depletion of PHF14. These findings are novel and of importance for the clinical management and diagnostic workup of neonates with severe persistent pulmonary hypertension of the newborn and dysmorphic features

De novo species identification using 16S rRNA gene nanopore sequencing

Nanopore sequencing is rapidly becoming more popular for use in various microbiota-based applications. Major limitations of current approaches are that they do not enable de novo species identification and that they cannot be used to verify species assignments. This severely limits applicability of the nanopore sequencing technology in taxonomic applications. Here, we demonstrate the possibility of de novo species identification and verification using hexamer frequencies in combination with k-means clustering for nanopore sequencing data. The approach was tested on the human infant gut microbiota of 3-month-old infants. Using the hexamer k-means approach we identified two new low abundant species associated with vaginal delivery. In addition, we confirmed both the vaginal delivery association for two previously identified species and the overall high levels of bifidobacteria. Taxonomic assignments were further verified by mock community analyses. Therefore, we believe our de novo species identification approach will have widespread application in analyzing microbial communities in the future

Skin emollient and early complementary feeding to prevent infant atopic dermatitis (PreventADALL): a factorial, multicentre, cluster-randomised trial

acceptedVersio

Predicting skin barrier dysfunction and atopic dermatitis in early infancy

Dry skin is associated with increased transepidermal water loss (TEWL), which has been found to precede atopic dermatitis (AD) in childhood. Objective We aimed to identify parental, prenatal, and perinatal predictive factors of dry skin, high TEWL, and AD at 3 months of age, and to determine if dry skin or high TEWL at 3 months can predict AD at 6 months. Methods From the Preventing Atopic Dermatitis and Allergies in children prospective birth cohort study, we included 1150 mother-child pairs. Dry skin, TEWL, and eczema were assessed at 3- and 6-month investigations. Eczema, used as a proxy for AD, was defined as the presence of eczematous lesions, excluding differential diagnoses to AD. High TEWL was defined as TEWL >90th percentile, equaling 11.3 g/m2/h. Potential predictive factors were recorded from electronic questionnaires at 18- and 34-week pregnancy and obstetric charts. Results Significant predictive factors (P 38 gestational weeks and paternal age >37 years; for high TEWL, male sex, birth during winter season, and maternal allergic disease; and for eczema, elective caesarean section, multiparity, and maternal allergic diseases. Dry skin without eczema at 3 months was predictive for eczema at 6 months (adjusted odds ratio: 1.92, 95% confidence interval: 1.21-3.05; P = .005), whereas high TEWL at 3 months was not. Conclusion In early infancy, distinct parental- and pregnancy-related factors were predictive for dry skin, high TEWL, and AD. Dry skin at 3 months of age was predictive for AD 3 months later