Burnout in doctors and nurses working in neonatal and pediatric intensive care units in a General Hospital

Aim: Health professionals working in intensive care units (ICU) are at high risk of developing the syndrome of professional burnout. The aim of the study was to explore the severity of professional burnout in doctors and nurses of neonatal (NICU) and pediatric ICUs (PICU) while identifying the factors associated with it.Study population and methods: Anonymous questionnaires were distributed to the nurses and doctors working in a NICU and PICU of a General Hospital. We utilized a 22-item questionnaire, the Maslach Burnout Inventory that evaluates three domains of burnout; emotional exhaustion (EE), depersonalization (DP), and personal accomplishment (PA).Results: The response rate was 58% (52/90). The average (SD) scores were 30.71 (11.5) for EE, 10.11 (5.9) for DP, and 33.37 (8.0) for PA. DP scores were significant higher in PICU than NICU (p = 0.032) and EE and DP scores were higher in nurses than doctors (p 0.013 and p 0.0001 for EE and DP, respectively). Employees who reported health issues had a significantly higher degree of EE (p = 0.044) and appeared less satisfied with their PA (p = 0.046). Multiple regression analysis confirmed that ICU type and professional capacity were independent predictors of burnout. The age, marital status and years of ICU work did not significantly affect the burnout severity.Conclusions: There is a significant degree of burnout in the personnel of Greek PICUs and NICUs which is affected by the professional status, type of ICU, and health issues of the employees involved. The state must implement the necessary interventions that will effectively minimize burnout in ICU personnel in order to prevent the unfavorable consequences on both staff members and inpatients

Accuracy of plasma interleukin-6 and C-reactive protein as markers of sepsis in preterm neonates with respiratory distress syndrome

Aim: To evaluate the diagnostic accuracy of plasma interleukin-6 (IL-6) and serum CRP in detecting blood culture proven sepsis in preterm neonates with respiratory distress syndrome (RDS).Patients and Methods: 140 preterm neonates, 62 with RDS and 78 without RDS, who developed clinical signs of sepsis were prospectively studied. Plasma IL-6 and serum CRP levels were measured. The ROC curves, the sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and likelihood ratios (LR) were calculated for IL-6 (> 20 pg/mL and > 80 pg/mL) and CRP (> 10 mg/L).Results: Positive blood cultures were found in 64 neonates (37 with RDS and 27 without RDS). Neonates with RDS had higher IL-6 (p 20 pg/mL was 0,93, 0,65 and 2,68, respectively, in neonates without RDS, and 0,92, 0,16 and 1,09, respectively, in neonates with RDS. The sensitivity, specificity and LR of CRP > 10mg/L were 0,86, 0,71 and 2,97, respectively, in neonates without RDS and 0,73, 0,68 and 2,28, respectively, in neonates with RDS.Conclusions: In neonates without RDS both the IL-6 and CRP are equally accurate markers in diagnosing sepsis, whereas in those with RDS the diagnostic value of IL-6 is limited

Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked Syndrome Associated With a Novel Mutation of FOXP3 Gene

The immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare, x-linked, recessive disorder characterized by dysfunction of the T regulatory (Treg) lymphocytes leading to autoimmune diseases. Herein we report a male patient with IPEX syndrome who presented with severe diarrhea, eczema, and malabsorption leading to failure to thrive and necessitating total parenteral nutrition, as well as with liver dysfunction. Laboratory investigation showed elevated liver enzymes that declined following treatment with glucocorticosteroids and immunosuppressive drugs, marked eosinophilia, increased total IgE, and decreased Treg cells. DNA analysis revealed that the patient himself was hemizygous and his mother heterozygous for the exon 10, c.1015C>T (p.Pro339Ser) mutation of the FOXP3 gene, which has not been previously reported. The current case indicates that mutations resulting in substitution of a certain amino-acid (i.e., proline 339) by different amino-acids are manifested with different IPEX phenotypes

Antimicrobial Peptides in Early-Life Host Defense, Perinatal Infections, and Necrotizing Enterocolitis—An Update

Host defense against early-life infections such as chorioamnionitis, neonatal sepsis, or necrotizing enterocolitis (NEC) relies primarily on innate immunity, in which antimicrobial peptides (AMPs) play a major role. AMPs that are important for the fetus and neonate include α and β defensins, cathelicidin LL-37, antiproteases (elafin, SLPI), and hepcidin. They can be produced by the fetus or neonate, the placenta, chorioamniotic membranes, recruited neutrophils, and milk-protein ingestion or proteolysis. They possess antimicrobial, immunomodulating, inflammation-regulating, and tissue-repairing properties. AMPs are expressed as early as the 13th week and increase progressively through gestation. Limited studies are available on AMP expression and levels in the fetus and neonate. Nevertheless, existing evidence supports the role of AMPs in pathogenesis of chorioamnionitis, neonatal sepsis, and NEC, and their association with disease severity. This suggests a potential role of AMPs in diagnosis, prevention, prognosis, and treatment of sepsis and NEC. Herein, we present an overview of the antimicrobial and immunomodulating properties of human AMPs, their sources in the intrauterine environment, fetus, and neonate, and their changes during pre- and post-natal infections and NEC. We also discuss emerging data regarding the potential utility of AMPs in early-life infections, as diagnostic or predictive biomarkers and as therapeutic alternatives or adjuncts to antibiotic therapy considering the increase of antibiotic resistance in neonatal intensive care units

A Novel Mutation of VPS33B Gene Associated with Incomplete Arthrogryposis-Renal Dysfunction-Cholestasis Phenotype

Arthrogryposis-renal dysfunction-cholestasis (ARC) syndrome is an autosomal recessive disorder caused by mutations of the VPS33B encoding the vacuolar protein sorting 33B (VPS33B), which is involved in the intracellular protein sorting and vesicular trafficking. We report a rare case of ARC syndrome without arthrogryposis caused by a novel mutation of VPS33B. A female patient of Greek origin presented on the 14th day of life with renal tubular acidosis, Fanconi syndrome, nephrogenic diabetes insipidus, and cholestasis with normal gamma-glutamyl transpeptidase, without arthrogryposis and dysmorphic features. She was born to apparently healthy, nonconsanguineous parents. Additional features included dry and scaling skin, generalized hypotonia, hypoplastic corpus callosum, neurodevelopmental delay, failure to thrive, short stature, recurrent febrile episodes with and without infections, and gastrointestinal bleeding. DNA testing revealed that the patient was homozygous for the novel c.1098_1099delTG (p.Glu367Alafs∗17) mutation of exon 14 of VPS33B gene (NM_018668) on chromosome 15q26.1, leading to a nonsense frameshift variant of VPS33B with premature termination of translation. Her parents were heterozygous for the same VPS33B mutation. The prognosis was predictably poor in the context of the intractable polyuria necessitating long-term parenteral fluid administration via indwelling central catheter leading to catheter-related sepsis, to which she eventually succumbed at the age of 7 months. This is the first published VPS33B mutation in an ARC patient of Greek origin. The current case adds to the spectrum of ARC-associated VPS33B mutations and provides evidence supporting the existence of incomplete ARC phenotype. Increased awareness and early genetic testing for ARC are suggested in cases with isolated cholestasis and/or renal tubular dysfunction, even in the absence of arthrogryposis

Modifications of Own Mothers’ Milk Fortification Protocol Affect Early Plasma IGF-I and Ghrelin Levels in Preterm Infants. A Randomized Clinical Trial

The aim was to investigate the effect of two own mother’s milk (OMM) fortification protocols on (a) IGF-I and ghrelin plasma levels at 35 post-conceptional weeks (PCW, T2) and whether this effect is maintained after elimination of the differences in OMM fortification, and (b) growth until 12 months corrected age. Forty-eight OMM-fed preterm infants (GA 24–32 weeks) were randomly allocated to the fixed-fortification (FF) group (n = 23) and the protein-targeting fortification (PTF) group (n = 25) targeting the recommended daily protein intake (PI). Plasma IGF-I and ghrelin were assessed at 35 (T2) and 40 (T3) PCW while growth was longitudinally assessed until 12 months corrected age. PTF group had lower IGF-I and higher ghrelin than FF group at T2, while receiving lower daily protein and energy amounts. PI correlated positively to T2-IGF-I and inversely to T3-ghrelin while energy intake (EI) correlated inversely to T2- and T3-ghrelin. Group and PI were independent predictors of adjusted T2-IGF-I, while group and EI were predictors of adjusted and T2-ghrelin. Growth parameter z-scores were comparable between groups up to 12 months corrected age. Modifications of OMM fortification have a transient effect on early plasma IGF-I and ghrelin levels in preterm infants in a way consistent with the previously recognized protein-energy/endocrine balance, indicating a potential programming effect

Early Postnatal Changes of Bone Turnover Biomarkers in Very Low-Birth-Weight Neonates-The Effect of Two Parenteral Lipid Emulsions with Different Polyunsaturated Fatty Acid Content: A Randomized Double-Blind Study

Background omega-3 polyunsaturated fatty acids (n-3 PUFAs) are reported to have beneficial effect on bone mineral density. This study aimed to evaluate early changes of bone turnover biomarkers in very low-birth-weight (VLBW) neonates and the effect of 2 parenteral lipid emulsions (PLEs) with different PUFA composition. Methods This is a randomized double-blind study with parallel design. VLBW neonates (n = 66) receiving parenteral nutrition (PN)>70% of daily energy requirements for >14 days were assigned into 2 groups that were prescribed soybean oil-based (n = 35) and n-3-enriched PLE (n = 31), respectively. Osteoprotegerin (OPG), soluble receptor activator of nuclear factor-kB ligand (sRANKL), osteocalcin (OC), interleukin-6 (enzyme-linked immunoblot assay kits), Ca, and P plasma levels were assessed before PLE implementation (T1) and on day 20 of life (T2). Results In the total population, sRANKL and OC significantly increased, whereas OPG and the OPG/sRANKL ratio decreased from T1 to T2. Within each group, T1-to-T2 changes of OC were significant in both groups, whereas those of OPG/sRANKL were significant only in the soybean-based group. Multiple regressions showed an independent effect of group allocation on OPG change. Significant associations were observed between PN duration and sRANKL change (negatively), n-6/n-3 and OC changes (positively), and OPG and sRANKL changes (positively). Conclusions A high bone-turnover rate in VLBW neonates with predominance of bone resorption is confirmed. The lower rate of OPG/sRANKL reduction in the n-3-enriched PLE group indicates that n-3 PUFA-enriched PLEs may help to attenuate early bone loss in VLBW neonates