Cutis verticis gyrata associated with congenital heart disease.

Cutis verticis gyrata (CVG) is a very rare benign disorder characterised by convoluted folds and deep furrows of the scalp that mimic cerebral sulci and gyri. Associations with other pathologies as neuropsychiatric and/or ophthalmologic disorders, secondary cases to inflammatory or neoplastic processes, as well as cases associated to genetic disorders as Turner's syndrome have been reported, but there is no literature describing an association with a congenital structural heart defect and no other underlying condition. We report a case of primary CVG in a 3-week-old female infant associated with an echocardiographic diagnosis of cor triatriatum. Other systemic examination findings and investigations were unremarkable, and the patient has normal neurodevelopment at 1 year old. Aside from the neuropsychiatric and ophthalmologic pathologies commonly associated with primary non-essential CVG, it should be noted that isolated congenital cardiac lesions are also possible, so as to increase our index of suspicion in patients with the disorder.

Foetal Haemoglobin as a Marker of Bone Marrow Suppression Secondary to Anti-Kell Alloimmunisation.

Anti-Kell alloimmunisation is a potentially severe minor blood group type incompatibility, not only as a cause of haemolytic disease of the foetus and newborn, but also due to the destruction of red blood cells (RBC) and mature form in the bone marrow with the subsequent hyporegenerative anaemia. In severe cases and when the foetus shows signs of anaemia, an intrauterine transfusion (IUT) may be necessary. When repeated, this treatment can suppress erythropoiesis and worsen the anaemia. We report the case of a newborn who required four IUTs plus an additional RBC transfusion at one month of life due to late onset anaemia. The identification of an adult haemoglobin profile with a complete absence of foetal haemoglobin in the patient's newborn screening samples at 2 and 10 days of life warned us of a possible late anaemia. The newborn was successfully treated with transfusion, oral supplements and subcutaneous erythropoietin. A blood sample taken at 4 months of life showed the expected haemoglobin profile for that age with a foetal haemoglobin of 17.7%. This case illustrates the importance of a close follow-up of these patients, as well as the usefulness of the haemoglobin profile screening as a tool for anaemia assessment.

Lung Ultrasound Scores Progress Differently in Extreme and Very Preterm Infants after Birth: A Multicentre Prospective Study.

INTRODUCTION: The lung ultrasound score (LUS) has been suggested to predict moderate-severe bronchopulmonary dysplasia (msBPD) in preterm infants. We aimed to assess LUS evolution after birth in preterm infants and the effect of gestational age. METHODS: This multicentre prospective observational study was performed with newborns born before 33 weeks of gestation. We created two groups: group 1 (23-27 weeks) and group 2 (28-32 weeks). We compared LUSs between the groups from birth until 36 weeks of postmenstrual age, and we estimated the LUS evolution in each group with a linear multilevel mixed-effects regression model. The effects of the need for surfactant or an msBPD diagnosis were also studied. RESULTS: We included 339 patients: 122 (36%) in group 1 and 217 (64%) in group 2. The infants in group 1 showed a steady progression in the LUS from birth until 4 weeks of age and a subsequent decrease; the infants in group 2 showed a progressive decrease in the LUS throughout the study. This progression varied significantly in the first weeks of life in infants who required surfactant at birth and after the first week of life in the patients diagnosed with msBPD. DISCUSSION/CONCLUSIONS: Extremely preterm infants showed persistently high LUSs during the first weeks of life, regardless of the progression to msBPD. In this group, the infants who did not require surfactant at birth exhibited an increase in their LUSs after the first week until their values were equal to the remaining infants in their group.

Assessing the impact of the COVID-19 pandemic on parental satisfaction in two European neonatal intensive care units.

BACKGROUND: Neonatal units across the world have altered their policies to prevent the spread of infection during the COVID-19 pandemic. Our aim was to report parental experience in two European neonatal units during the pandemic. METHODS: Parents of infants admitted to each neonatal unit were asked to complete a questionnaire regarding their experience during the COVID-19 pandemic. At King's College Hospital, UK (KCH), data were collected prospectively between June 2020 and August 2020 (first wave). At the Hospital Clínic Barcelona (HCM), data were collected retrospectively from parents whose infants were admitted between September 2020 and February 2021 (second and third wave). RESULTS: A total of 74 questionnaires were completed (38 from KCH and 36 from HCM). The parents reported that they were fully involved or involved in the care of their infants in 34 (89.4%) responses in KCH and 33 (91.6%) responses in HCM. Quality time spent with infants during the pandemic was more negatively affected at KCH compared with HCM (n=24 (63.2%) vs n=12 (33.3%)). Parents felt either satisfied or very satisfied with the updates from the clinical care team in 30 (79.0%) responses at KCH and 30 (83.4%) responses in HCM. The parents felt that the restrictions negatively affected breast feeding in six (15.8%) responses at KCH and two (5.6%) responses in HCM. Travelling to the hospital was reported overall to be sometimes difficult (39.2%); this did not differ between the two units (14 (36.8%) respondents at KCH and 15 (41.6%) from HCM). Furthermore, the self-reported amount of time spent giving kangaroo care also did not differ between the two countries. CONCLUSION: Restrictive policies implemented due to the COVID-19 pandemic had a negative impact on the perception of quality of time spent by parents with their newborns admitted to neonatal units.

Lung Ultrasound in Bronchopulmonary Dysplasia: Patterns and Predictors in Very Preterm Infants.

INTRODUCTION: Lung ultrasound (LUS) is useful for respiratory management in very preterm infants (VPI), but little is known about the echographic patterns in bronchopulmonary dysplasia (BPD), the relation between the image findings, and the severity of the disease and its long-term outcomes. We aimed to describe LUS patterns in BPD and analyze the accuracy of LUS to predict the need for respiratory support at 36 weeks postmenstrual age (PMA) in VPI. METHODS: Preterm infants ≤30.6 weeks of gestational age were recruited. LUS was performed at admission, at 7th, and 28th day of life (DOL) with a standardized protocol (6 zones: anterior, lateral, and posterior fields). Clinical data, respiratory outcomes, and image findings were recorded. RESULTS: Eighty-nine patients were studied. Infants with BPD had significantly higher LUS score at admission, at 7th, and 28th DOL. Patients with BPD exhibited more consolidations and pleural line abnormalities at 7th and 28th DOL than those without BPD (p < 0.001), regardless of the definition used for BPD. LUS at 7th DOL predicted NICHD 2001-BPD with R2 = 0.522; AUC = 0.87 (0.79-0.94), p < 0.001, and Jensen 2019-BPD with R2 = 0.315 (AUC = 0.80 [0.70-0.90], p < 0.001). A model including mechanical ventilation >5 days, oxygen therapy for 7 days and LUS score at 7th DOL accurately predicted the need for respiratory support at 36 weeks PMA (R2 = 0.655, p < 0.001) with an AUC = 0.90 (0.84-0.97), p < 0.001. CONCLUSION: LUS score, pleural line abnormalities, and consolidations can be useful to diagnose BPD in VPI and to predict its severity after the first week of life.

Feasibility of umbilical cord blood as a source of red blood cell transfusion in preterm infants.

BACKGROUND: Preterm infants born earlier than 32 weeks of gestational age (GA) often need red blood cell (RBC) transfusions, which have been associated with an increased incidence of complications of prematurity, due to changes in tissue oxygenation. Transfusion of umbilical cord blood (UCB) could be beneficial for this group. The aims of this study were: (i) to determine the RBC transfusion needs in infants <32 weeks in Hospital Clinic of Barcelona; (ii) to identify the target GA group that would benefit most from UCB transfusion; and (iii) to assess the current availability of UCB as a potential source of RBC transfusion for these premature infants in our tertiary referral blood bank. MATERIAL AND METHODS: A retrospective observational study was performed on infants born at <32 weeks GA, divided into two groups: (i) extremely low gestational age neonates (ELGAN) (from 230 to 276 weeks) and (ii) very preterm neonates (VPN) (from 280 to 316 weeks). Their complications and transfusion rates were compared. Processing and availability of UCB samples in the reference blood bank were assessed. RESULTS: Overall, 1,651 infants <32 weeks GA were admitted in the study period. While 12.5% of VPN received at least one RBC transfusion, the percentage increased to 60% among the ELGAN. Retinopathy of prematurity and bronchopulmonary dysplasia were diagnosed more frequently in the ELGAN group (p<0.001) than in the VPN group. The annual average volume of RBC transfusion in our study group was 1.35 L (95% CI: 1.07-1.64). The reference blood bank was able to produce 16 L (95% CI: 14-18) of UCB-RBC per year. CONCLUSION: Considering the data obtained about RBC transfusion needs and morbidities, the ELGAN group has been identified as the target group that would benefit most from UCB-RBC transfusions. We have demonstrated that our blood bank is able to produce enough RBC from UCB. Randomised control trials are warranted to study the potential benefits of UCB compared to adult blood for RBC transfusions.