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Introduction: Cystic fibrosis (CF) is a potentially severe disease. The development of new therapies with cystic fibrosis transmembrane conductance regulator (CFTR) modulators has been a great advance in the management of this condition because they improve the function of the faulty CFTR protein rather than palliate its consequences. CFTR modulator therapy improves pancreatic and lung function and, therefore, quality of life, with greater benefits the sooner treatment is started. For this reason, the use of these therapies is being approved for increasingly younger patients. Only two cases of pregnant women taking CFTR modulators therapy with CF fetuses have been reported, suggesting that it could resolve meconium ileus (MI) prenatally, and delay/prevent other consequences of CF. Case Presentation: We report a case of a healthy pregnant patient who underwent CFTR modulator therapy with elexacaftor-tezacaftor-ivacaftor (ETI) in order to treat her fetus with CF (F508del homozygous CFTR mutation) and MI. Ultrasound findings suggestive of MI were observed at 24 weeks. Both parents were tested for CFTR mutations, and both were carriers of the F508del CFTR mutation. The fetus was diagnosed with CF by amniocentesis at 26+2 weeks. Maternal ETI therapy was initiated at 31+1 weeks and no dilated bowel was observed at 39 weeks. There were no signs of bowel obstruction after birth. Maternal ETI treatment was continued during breastfeeding, with normal liver function. Immunoreactive trypsinogen in the newborn was 58.1 ng/mL, sweat chloride test was 80 mmol/l, and fecal elastase on the second day of life was 58 μg/g. Discussion/Conclusion: Prenatal ETI treatment, as well as during breastfeeding, could solve, prevent and/or delay CF complications.

Purpose: We aimed to describe the outcomes, focusing on the hearing and neurological development, of children born to mothers with SARS-CoV-2 infection during pregnancy, and to evaluate the persistence of maternal antibodies in the first months of life. Methods:An observational, prospective study at a tertiary hospital in Madrid (Spain) on infants born to mothers infected with SARS CoV-2 during pregnancy between March and September 2020. A follow-up visit at 1-3 months of age with a physical and neurological examination, cranial ultrasound (cUS), SARS-CoV-2 RT-PCR on NPS and SARS-CoV-2 serology were performed. Hearing was evaluated at birth through the automated auditory brainstem response and at six months of age through the auditory steady-state response. A neurodevelopmental examination using the Bayley-III scale was performed at 12 months of age. Results:Of 95 infants studied, neurological examination was normal in all of them at the follow-up visit, as was the cUS in 81/85 (95%) infants, with only mild abnormalities in four of them. Serology was positive in 47/95 (50%) infants, which was not associated with symptoms or severity of maternal infection. No hearing loss was detected and neurodevelopment was normal in 96% of the children (median Z score: 0). Conclusions: In this cohort, the majority of infants born to mothers with SARS-CoV-2 infection during pregnancy were healthy children with a normal cUS, no hearing loss and normal neurodevelopment in the first year of life. Only half of the infants had a positive serological result during the follow-up.

In utero transmission of severe acute respiratory syndrome coronavirus 2 infection is a point of debate. We report a case of severe acute respiratory syndrome coronavirus 2 vertical transmission from asymptomatic mother, with molecular detection in mother's blood at delivery and neonatal nasopharyngeal swabs at 5 and 28 hours of life and later IgG seroconversion. The newborn was asymptomatic.

Background and Objectives: Therapeutic interventions to improve the efficacy of whole-body cooling for hypoxic-ischemic encephalopathy (HIE) are desirable. Topiramate has been effective in reducing brain damage in experimental studies. However, in the clinical setting information is limited to a small number of feasibility trials. We launched a randomized controlled double-blinded topiramate/placebo multicenter trial with the primary objective being to reduce the antiepileptic activity in cooled neonates with HIE and assess if brain damage would be reduced as a consequence. Study Design: Neonates were randomly assigned to topiramate or placebo at the initiation of hypothermia. Topiramate was administered via a nasogastric tube. Brain electric activity was continuously monitored. Topiramate pharmacokinetics, energy-related and Krebs’ cycle intermediates, and lipid peroxidation biomarkers were determined using liquid chromatography-mass spectrometry and MRI for assessing brain damage. Results: Out of 180 eligible patients 110 were randomized, 57 (51.8%) to topiramate and 53 (48.2%) to placebo. No differences in the perinatal or postnatal variables were found. The topiramate group exhibited less seizure burden in the first 24 h of hypothermia (topiramate, n = 14 [25.9%] vs. placebo, n = 22 [42%]); needed less additional medication, and had lower mortality (topiramate, n = 5 [9.2%] vs. placebo, n = 10 [19.2%]); however, these results did not achieve statistical significance. Topiramate achieved a therapeutic range in 37.5 and 75.5% of the patients at 24 and 48 h, respectively. A significant association between serum topiramate levels and seizure activity (p < 0.016) was established. No differences for oxidative stress, energy-related metabolites, or MRI were found. Conclusions: Topiramate reduced seizures in patients achieving therapeutic levels in the first hours after treatment initiation; however, they represented only a part of the study population. Our results warrant further studies with higher loading and maintenance dosing of topiramate.

In 2017, a worldwide survey was conducted on compliance with the practices promoted by Neo-BFHI (Baby-friendly Hospital Initiative expansion to neonatal wards).To present the results of the Spanish wards that participated in the global survey and compare them with those obtained internationally.Cross-sectional study through a survey on compliance with the Neo-BFHI ("Three basic principles", "Ten steps adapted to neonatal wards" and "the compliance with the International Code of Marketing of Breast-milk Substitutes" and subsequent relevant World Health Assembly resolutions). Compliance was calculated as the mean in each indicator and a final mean score for each neonatal unit. For the partial and final scores for each country and at the international level, the median was used. All scores ranged between 0 and 100.The response rate in Spain was 90%. The range of the national mean for neonatal wards were from 37 to 99, with no differences in the final score according to the level of care. The global score for Spain (72) is below the international median (77) and this also occurs in 8 of 14 items. The neonatal wards from BFHI designated hospitals, obtained a significantly higher mean global score, and in 9 of 14 items than the non-accredited ones.Both international and national results indicate an improvement in breast feeding practices in neonatal units. The benefits of the BFHI accreditation of maternity reach neonatal wards. Spain has several key points below the international score.

What is known and objective Tocilizumab is an IL‐6 receptor inhibitor agent which has been proposed as a candidate to stop the inflammatory phase of infection by the severe acute respiratory syndrome coronavirus‐2 (SARS‐CoV‐2). However, safety data of tocilizumab in pregnant women and their newborn are scarce. We aimed to describe maternal and neonatal safety outcomes associated with tocilizumab treatment in pregnant women with severe COVID‐19. Methods This is a retrospective study of severe COVID‐19 pregnant women, treated with tocilizumab in two Spanish hospitals between 1 March and 31 April 2020. Demographics, medical history, clinical and radiologic findings, treatment information and laboratory data of mothers and their newborns were collected from electronic medical records. Results and discussion A total of 12 pregnant women were identified to have received tocilizumab during pregnancy in the two hospitals. Median gestational age at admission was 27.7 weeks (interquartile range, 18.0–36.4). Most of them received lopinavir/ritonavir, azithromycin and hydroxychloroquine, two patients received corticosteroids and one received interferon beta 1B. All 12 pregnancies resulted in live births. Somatometric values were normal for all newborns, and evolution at 14 and 28 days was favourable for all of them. Hepatotoxicity was observed in 2 patients, which improved or resolved at discharge. Cytomegalovirus reactivation was detected in another patient who had also received corticosteroids for 15 days, causing a congenital infection in her newborn. Both hepatotoxicity and viral reactivation adverse events were classified as possibly related to tocilizumab administration according to Naranjo's causality algorithm. What is new and conclusions It does not appear that tocilizumab has detrimental effects for the mother and newborn. Close monitoring of infections should be considered, especially if other immunosuppressive agents are used., Evolution of cytomegalovirus viral load and serology tests in one pregnant patient, in which viral reactivation was detected. She received a single dose of tocilizumab and methylprednisolone therapy for 15 days. Baseline IgM antibodies and amniotic fluid polymerase‐chain‐reaction were negative, but they became positive 6 days after tocilizumab infusion and viral load was detected on day 13.

Objective This study aimed to assess the applicability of the insertion of small diameter catheters through the femoral vein in extremely low-birth-weight (ELBW) infants.Study Design All femoral small diameter catheters (Silastic or femoral arterial catheter [FAC]) inserted in ELBW infants in a tertiary level neonatal intensive care unit were retrospectively reviewed. Success rate, dwelling time, and percutaneously inserted central venous catheter–related complications were recorded.Results Thirteen small diameter catheters were inserted in seven ELBW infants. Mean gestational age at birth was 25+3 weeks (standard deviation [SD] ± 2.12) and mean birth weight was 686 g (SD ± 204.9). Mean weight at the first time of insertion was 1,044 g (SD ± 376.3). In two occasions, a FAC was used instead of a Silastic. In most cases (11/13, 84.6%), the patient was intubated prior to the procedure. The mean dwelling time was 16.7 days (SD ± 9.8). Most of the inserted small diameter catheters were removed electively (8/12, 66.7%), except for one episode of clinical sepsis from coagulase-negative Staphylococcus and three cases of accidental line extravasation. No other complications were reported. The success rate was 92.3%.Conclusion Femoral venous catheterization using small diameter catheters in ELBW infants may be promising when other routes have been exhausted. Our results support that it is a feasible technique that can be performed at the bedside with successful results when conducted by experienced personnel.