Ilias Chatziioannidis

Haemostatic profile of healthy premature small for gestational age neonates

BACKGROUND The pathogenetic profile of premature Small for Gestational Age (SGA) neonates is strongly related to their haemostatic equilibrium, which is inadequately understood. OBJECTIVE To evaluate coagulation and fibrinolysis in premature SGA neonates before intervening with Vitamin K administration. STUDY DESIGN We performed a comparison of coagulation, natural inhibitors and fibrinolysis between SGA and Appropriate for Gestational Age (AGA) infants born prematurely [gestational age (G.A.) <37 weeks]. Study population consisted of 139 preterm newborns, 68 of whom were SGA (25 males and 43 females), while 71 were AGA (37 males and 34 females) that consisted the control group. Blood samples were obtained within 30 minutes following birth and before the administration of vitamin K. Investigation included: PT, INR, APTT, fibrinogen, coagulation factors II, V, VII, VIII, IX, X, XI, XII, von Willebrand factor, protein C and free protein S, antithrombin (AT), APCR, tPA and PAI-1. The independent t-test and the Mann-Whitney U test were used to compare the differences between the values of haemostatic parameters. RESULTS Premature SGA infants presented significantly lower levels of fibrinogen (p<0.029) and higher levels of VIIIc factor, APCR, tPA and PAI-1 (p<0.041, 0.017, 0.021 and 0.019 respectively). The two groups had similar demographic characteristics (except from birth weight), without significant differences in the values of other haemostatic parameters. CONCLUSIONS Despite the statistically significant differentiation in the levels of fibrinogen, VIIIc factor, APCR, tPA and PAI-1, the rest of haemostatic parameters have similar values between SGA and AGA preterms.

Intraventricular Hemorrhage and Platelet Indices in Extremely Premature Neonates.

Intraventricular hemorrhage (IVH) is a multifactorial disorder, the most important risk factors of which are prematurity and low birth weight. Disturbances in cerebral blood flow, inherent fragility of the germinal matrix vasculature, and platelet/coagulation disturbances are the 3 major pathogenic mechanisms. In this context, we investigated the role of platelet indices and several maternal and neonatal characteristics in the development of IVH through a retrospective cohort analysis of 130 extremely premature neonates, 24% of whom presented with severe IVH. There was a significant difference in platelet counts between the IVH and the control group on the first day of life (P=0.046). Presence of IVH was linked with lower birth weight (P=0.006) and lower gestational age (P=0.001). Platelet count on the first day of life was positively correlated with survival (P=0.001) and, along with platelet mass, was indicative of the worst IVH grade recorded for each neonate (P=0.002 and 0.007, respectively). Prolonged prothrombin time was also correlated with IVH (P<0.001), but factor analysis supported no prominent role. Maternal medications seem to play a minor role as well. In conclusion, IVH in extremely premature infants cannot be solely explained by platelet parameters, and further studies are required to determine the relationships between IVH, platelet indices, and outcomes.

Recurrent giant cell fibroblastoma: Malignancy predisposition in Kabuki syndrome revisited

Kabuki syndrome is a genetic condition characterized by distinctive facial phenotype, mental retardation, and internal organ malformations. Mutations of the epigenetic genes KMT2D and KDM6A cause dysregulation of certain developmental genes and account for the multiple congenital anomalies of the syndrome. Eight cases of malignancies have been reported in young patients with Kabuki syndrome although a causative association to the syndrome has not been established. We report a case of a 12‐year‐old girl with Kabuki syndrome who developed a tumor on the right side of her neck. A relapsing tumor 19 months after initial excision, proved to be giant cell fibroblastoma. Τhis is the first report of giant cell fibroblastoma ‐a rare tumor of childhood‐ in a patient with Kabuki syndrome.

Platelet Mass Predicts Intracranial Hemorrhage in Neonates With Gram-negative Sepsis

Neonatal sepsis due to gram-negative bacteria is associated with severe hemorrhagic conditions, such as intracranial hemorrhage (ICH). The aim of the study was to investigate the significance of platelet (PLT) count and platelet mass (PM) in predicting promptly neonatal ICH. Demographics, species, PLT, PM, ICH, and outcome for neonates with gram-negative sepsis for the period 2005 to 2012 were retrospectively recorded. Eighty-four infants were enrolled with median gestational age 30 weeks, median birthweight 1481.5 g, and median age at sepsis diagnosis 23 days. The most frequently isolated bacteria were Enterobacter spp. (38.1%). ICH occurred in 16 neonates (19%), whereas the mortality rate was 25% (21 neonates). The median PLT count and PM at days 1, 2, and 3 after diagnosis of gram-negative sepsis was significantly associated with the presence of ICH. Regression analysis revealed the cutoff predictive value of 355 fL/nL for the PM at day 3 (area under the curve: 75, sensitivity 90%, P=0.002). PM levels could play an important role in predicting the occurrence of ICH in high-risk neonates.

Prevalence, causes and mental health impact of workplace bullying in the Neonatal Intensive Care Unit environment

Objectives The aim of this study is to examine the prevalence, to report barriers and mental health impact of bullying behaviours and to analyse whether psychological support at work could affect victims of bullying in the healthcare workplace. Design Self-administered questionnaire survey. Setting 20 in total neonatal intensive care units in 17 hospitals in Greece. Participants 398 healthcare professionals (doctors, nurses). Main outcome measures The questionnaire included information on demographic data, Negative Act Questionnaire-Revised (NAQ-R) behaviour scale, data on sources of bullying, perpetrators profile, causal factors, actions taken and reasons for not reporting bullying, psychological support and 12-item General Health Questionnaire (GHQ-12) scores to investigate psychological distress. Results Prevalence of bullying measured by the NAQ-R was 53.1% for doctors and 53.6% for nurses. Victims of bullying differed from non-bullied in terms of gender and job experience, among demographic data. Crude NAQ-R score was found higher for female, young and inexperienced employees. Of those respondents who experienced bullying 44.9% self-labelled themselves as victims. Witnessing bullying of others was found 83.2%. Perpetrators were mainly females 45–64 years old, most likely being a supervisor/senior colleague. Common reasons for not reporting bullying was self-dealing and fear of consequences. Bullying was attributed to personality trait and management. Those who were bullied, self-labelled as a victim and witnessed bullying of others had higher GHQ-12 score. Moreover, psychological support at work had a favour effect on victims of bullying. Conclusions Prevalence of bullying and witnessing were found extremely high, while half of victims did not consider themselves as sufferers. The mental health impact on victims and witnesses was severe and support at work was necessary to ensure good mental health status among employees.

Focusing on patient safety in the Neonatal Intensive Care Unit environment

Patient safety in the Neonatal Intensive Care Unit (NICU) environment is an under-researched area, but recently seems to get high priority on the healthcare quality agenda worldwide. NICU, as a highly sensitive and technological driven environment, signals the importance for awareness in causation of mistakes and accidents. Adverse events and near misses that comprise the majority of human errors, cause morbidity often with devastating results, even death. Likewise in other organizations, errors causes are multiple and complex. Other high reliability organizations, such as air force and nuclear industry, offer examples of how standardized/homogenized work and removal of systems weaknesses can minimize errors. It is widely accepted that medical errors can be explained based on personal and/or system approach. The impact/effect of medical errors can be reduced when thorough/causative identification approach is followed by detailed analysis of consequences and prevention measures. NICU’s medical and nursing staff should be familiar with patient safety language, implement best practices, and support safety culture, maximizing efforts for reducing errors. Furthermore, top management commitment and support in developing patient safety culture is essential in order to assure the achievement of the desirable organizational safety outcomes. The aim of the paper is to review patient safety issues in the NICU environment, focusing on development and implementation of strategies, enhancing high quality standards for health care.

Newborns as potential organ donors: a new perspective?

Demand for organ donation is increasing over the years in all age groups leading search for alternative sources. Donation pool expansion in newborns necessitates new approaches specifically for brain and circulatory death definition. The “dead donor rule” (the donor must be dead before organ removal starts) remains the cornerstone for organ procurement. Brain death definition and time determination of circulatory death need to be uniformly accepted by the scientific community. Technical advances, uniform protocols for death determination and new proceedings in surgical field can maximize donation pool along with establishment of end-of-life care standard procedures and persistent support of the family. Newborns can serve as multivisceral donors in the near future to meet the increasing demand for organs in this age group.

Corrigendum to “Netherton Syndrome in a Neonate with Possible Growth Hormone Deficiency and Transient Hyperaldosteronism”

In the paper titled “Netherton Syndrome in a Neonate with Possible Growth Hormone Deficiency and Transient Hyperaldosteronism,” [1] the first and last names of all the authors were reversed. The correct names in order (first name, last name) have been shown above.

Comparison of 24 months neurodevelopmental outcome in twins and singletons ≤ 34 weeks gestation at birth

The aim of this study was to screen neurodevelopmental impairment of preterm twins born at less than 34 weeks of gestation, compare them with the outcome of preterm singletons, and to determine potential neonatal factors adversely related to motor and cognitive outcome. Twins of 25-34 weeks gestation were included in the study. In total, 46 twins were matched with 46 singletons and were followed prospectively to 24 months corrected age. Obstetrical and neonatal data were recorded. All infants were assessed using the Bayley Scales of Infant and Toddler Development III For all morbidities, a significant difference could not be demonstrated. At 24 month follow up there was no significant difference in the cognitive outcome for the twins compared to singletons [98.6 (± 10.4) vs 97.8 (± 9.7), respectively]. There was also no significant difference in the motor outcome for the twins compared to singletons [94.8 (± 12.4) vs 98.1 (± 9.6.), respectively]. For the twins, we found a link between pre-eclampsia and abnormal cognitive (p = 0.012) and motor (p = 0.030) results. With the number of twins steadily increasing, close developmental monitoring and probably early intervention services are needed to determine future directions for research.

Cerebral sinus thrombosis in an infant with Prader-Willi syndrome and literature review

A full-term male neonate from a first pregnancy of two clinical non-consanguineous parents was born at 40 weeks of gestation with cesarean section. He was admitted at 2 hours of life to our III level Neonatal Intensive Care Unit due to generalized hypotonia, presenting at birth. Cerebral ultrasound showed a temporal bilateral aspecific alteration of the parenchimal echogenicity, whereas a magnetic resonance imaging/venography revealed an extensive cerebral sinus thrombus. Extensive diagnostic studies for prothrombotic disorders showed negative results, even if there was an alterated haemostatic screening. Persistency of hypotonia led us to investigate Prader-Willi syndrome among others. Methylation analysis confirmed the diagnosis. This is the third report associating cerebral venous thrombosis and Prader-Willi syndrome, confirming sinus thrombosis as a possible presentation of this syndrome. A review of the literature is provided in order to disclose possible similarities and differences in Prader-Willi syndrome patients with cerebral sinovenus thrombosis.