Neonatal Presentation of Severe Hemophilia A: An Original Case Report and a Literature Review.

: We report the case of a neonate diagnosed with severe hemophilia A (HA) and conduct a literature review of cases of severe HA presenting at the neonatal age to help define the clinical diagnostic findings and existing differences between the sporadic and familial onset of this condition. : A 6-day-old newborn presented with worsening pallor, inappetence, and hyporeactivity for 48 h. The diagnosis was severe hemophilia A (HA), leading to an unfavorable outcome.

Do Different Amounts of Exogenous Surfactant Differently Influence Cerebrovascular Instability in a Consecutive Group of Preterm Babies? Preliminary Results from a Single-Center Experience.

Background: Thirty years ago, the first attempt by Saliba and colleagues was made to reduce the negative effects (hypercarbia) of exogenous surfactant (ES) by slowing its administration. Sixteen years later, we observed the first less invasive surfactant administration (LISA) attempt by Kribs and colleagues. Many studies, since that time, have tried to minimize the invasiveness of ES and subsequent cerebral blood flow perturbations through studies using near-infrared spectroscopy (NIRS).

Ventilatory Support, Extubation, and Cerebral Perfusion Changes in Pre-Term Neonates: A Near Infrared Spectroscopy Study.

Early extubation is considered to be beneficial for pre-term neonates. On the other hand, premature extubation can cause lung derecruitment, compromised gas exchange, and need for reintubation, which may be associated with severe brain injury caused by sudden cerebral blood flow changes. We used near infrared spectroscopy (NIRS) to investigate changes in cerebral oxygenation (rScO) and fractional tissue oxygen extraction (+) after extubation in pre-term infants.

Optimizing haemoglobin measurements in VLBW newborns: Insights from a comparative retrospective study.

Introduction: Haemoglobin levels assessment is a crucial part of neonatal intensive care practice, the painful experience of repeated heel pricks and venepunctures blood sampling may negatively affect neonatal clinical course. To date the reliability of haemoglobin levels obtained by point-of-care testing (POCT) analysis if compared to standard blood cell count remains controversial.

Materials And Methods: Retrospective study conducted on all inborn premature infants (gestational age < 32 weeks) admitted to NICU of the IRCCS Giannina Gaslini Institute during the period May 2021-April 2023.

Disruption of Cerebellar Granular Layer as a Consequence of Germinal Matrix Intraventricular Hemorrhage in Extreme Prematurity: An Acute Direct Mechanism Too?

Cerebellum is an important brain structure for the future development of motor, cognitive, and behavioral abilities in children. This structure undergoes its most significant growth during the third trimester of pregnancy. Prematurity gathers several risk factors for cerebellar impairment and underdevelopment, and among them is ventricular dilatation following germinal matrix intraventricular hemorrhage (GMH-IVH).

RiNeo MR: A mixed reality simulator for newborn life support training.

Neonatal resuscitation is an uncommon, albeit critical task that is more likely to succeed if performed properly and promptly. In this context, simulation is an appropriate way for training and assessing the abilities of all medical staff involved in delivery room care. Recent studies have shown that learning is enhanced if the simulation experience is realistic and engaging.

Point-of-care brain ultrasound and transcranial doppler or color-coded doppler in critically ill neonates and children.

Unlabelled: Point-of-care brain ultrasound and transcranial doppler or color-coded doppler is being increasingly used as an essential diagnostic and monitoring tool at the bedside of critically ill neonates and children. Brain ultrasound has already established as a cornerstone of daily practice in the management of the critically ill newborn for diagnosis and follow-up of the most common brain diseases, considering the easiness to insonate the brain through transfontanellar window. In critically ill children, doppler based techniques are used to assess cerebral hemodynamics in acute brain injury and recommended for screening patients suffering from sickle cell disease at risk for stroke.

Precocious Puberty Diagnoses Spike, COVID-19 Pandemic, and Body Mass Index: Findings From a 4-year Study.

Context: Since the COVID-19 outbreak, the number of girls with suspected precocious puberty has increased.

Objective: To compare the incidence of idiopathic central precocious puberty (ICPP) during COVID-19 with that of the previous 4 years.

Methods: Anthropometric, biochemical, and radiological parameters were collected between January 2016 and June 2021 from 133 girls who met the Rapidly Progressive ICPP criteria (RP-ICPP).

Clinical, Endocrine and Neuroimaging Findings in Girls With Central Precocious Puberty.

Context: The etiology of central precocious puberty (CPP) includes a spectrum of conditions. Girls younger than age 6 years with CPP should undergo cranial magnetic resonance imaging (MRI), but it remains controversial whether all girls who develop CPP between the ages of 6 and 8 years require neuroimaging examination.

Objective: To investigate the frequency of brain MRI abnormalities in girls diagnosed with CPP and the relationship between maternal factors, their age at presentation, clinical signs and symptoms, hormonal profiles, and neuroimaging findings.

RiNeo MR: A mixed-reality tool for newborn life support training.

The first minute of life, the Golden Minute, has been defined as a critical window in which fundamental physiological processes occur for establishing spontaneous ventilation in a newborn. Resuscitation is more likely to succeed if it is performed properly and at the right time. In this scenario, simulation is an appropriate tool for training and evaluating the abilities of all staff working in the delivery room, as well as students.

Endocrine Outcomes In Central Diabetes Insipidus: the Predictive Value of Neuroimaging "Mismatch Pattern".

Context: The etiology of central diabetes insipidus (CDI) in children is often unknown. Clinical and radiological features at disease onset do not allow discrimination between idiopathic forms and other conditions or to predict anterior pituitary dysfunction.

Objective: To evaluate the evolution of pituitary stalk (PS) thickening and the pattern of contrast-enhancement in relation with etiological diagnosis and pituitary function.