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The Newborn Infant Parasympathetic Evaluation (NIPE) index is an instrument that enables continuous, fast and objective assessment of neonatal discomfort. The aim of the study was to analyse changes in NIPE values after performance of blood draws and the factors involved in this variation.We conducted a prospective observational study. We included infants admitted to the neonatal intensive care unit between June and December 2021 who underwent blood draws. We recorded demographic data, aspects related to the procedure, the NIPE index and the heart rate at baseline and 1, 2, 3, 4, 5, 10 and 15 min after the procedure.The study included 86 records for 49 patients. In the first 4 min after the procedure, there was a significant decrease in the NIPE index, with a maximum decrease of 22.8% relative to baseline and the nadir at 2.79 min. The decrease in NIPE values was greater in infants born preterm, male, with lower 5-min Apgar scores and following procedures that had been performed previously, after caesarean section or in the morning. There were no differences when the blood draw was obtained during kangaroo care. The correlation between the NIPE index and the heart rate was weak.After a painful procedure, such as a blood draw, the NIPE monitor showed a significant decrease in the first 4 min, which was more pronounced in preterm infants, in repeated procedures or after caesarean delivery. The NIPE index could help identify infants experiencing acute procedural pain, complementing clinical rating scales.

Corticosteroids have had a central role in the treatment of nephrotic syndrome. The management of these patients who become dependent to steroids is complex, involving different immunosuppressive drugs patterns. The monoclonal antibody anti CD20, Rituximab, is likely to have beneficial effects in cases of steroid-dependent nephrotic syndrome patients with no easy resolution, even when we cannot make a statement about the specific role in the impact. We bring our personal experience in pediatric patients treated with this medication during the last years, to provide a thorough overview and useful information about the role of Rituximab in this pathology.Retrospective study in patients with steroid-dependent idiopathic nephrotic syndrome controlled in the division of Pediatric Nephrology of a Spanish tertiary hospital in those patients who had received at least one treatment cycle of Rituximab, at any moment along the evolution of the disease.The study involved 8 patients. All of them previously received immunosuppressive therapy. The Rituximab were administered as an intravenous infusion, in a dose of 375 mg/mThe favorable results reported after rituximab treatment in our patients seems to confirm the effectiveness of this drug in the steroid-dependent nephrotic syndrome, making that therapeutic option into consideration and legitimating the use of the drug in complex cases involving pediatric patients. Even so, it seems recommendable to design pertinent studies to clarify, among others, the optimum regimen of the treatment (dose, interval and cycles), clinical repercussion and potential adverse effects in long terms.

Resumen Introduccion La corticoterapia continua siendo la piedra angular en el tratamiento del sindrome nefrotico. El manejo de los pacientes que desarrollan dependencia a esteroides es complejo, implicando distintas pautas de farmacos inmunosupresores. El rituximab, anticuerpo monoclonal anti-CD20, parece tener efectos beneficiosos en pacientes con sindrome nefrotico cortico-dependiente de dificil manejo clinico, si bien aun no esta bien definido su papel en esta entidad. Con el fin de aportar informacion util sobre el papel del rituximab en esta patologia, presentamos nuestra experiencia personal en pacientes pediatricos tratados con este farmaco en los ultimos anos. Materiales y metodos Estudio retrospectivo en pacientes con sindrome nefrotico idiopatico cortico-dependiente controlados en la Seccion de Nefrologia Pediatrica de un hospital terciario espanol, y que habian recibido, al menos, un ciclo de tratamiento con rituximab durante cualquier momento de la evolucion de la enfermedad. Resultados Se incluyeron en el estudio ocho pacientes. Todos habian recibido terapia inmunosupresora previamente. El rituximab se administro por via intravenosa a una dosis de 375 mg/m2, y todas las dosis se administraron en situacion de remision de la enfermedad. Se confirmo la deplecion completa de linfocitos B (CD 19%), tras la primera dosis de rituximab en todos, excepto en uno, en el que el recuento fue del 1%. El periodo de deplecion fue de 10,3 meses (mediana; rango 6,5-16 meses), y solamente en uno de los pacientes se registro una recaida de la enfermedad durante este periodo. Tras el inicio del tratamiento, se observo una reduccion en el numero de recaidas (3,6 recaidas/ano en el ano previo al inicio del tratamiento vs. 0,1 recaidas/ano durante el primer ano posrituximab). La supervivencia libre de recaida en el primer ano fue del 83,3% en los pacientes, con mas de una recaida (75% de los pacientes), sin existir recaidas tras el inicio del tratamiento en dos pacientes. En un 87,5% de los pacientes se pudieron retirar uno o mas farmacos tras el primer ciclo de rituximab. Tras el tratamiento con rituximab, se consiguio una reduccion del 96,5% de las dosis de corticoides administrados (28,5 mg/m2/dia en los tres meses pretratamiento vs. 1 mg/m2/dia en los tres ultimos meses de seguimiento en los pacientes tratados). Tras el periodo de seguimiento posrituximab (mediana 46,5 meses, rango 5-97 meses), no se observaron efectos adversos significativos atribuibles al farmaco. Conclusiones Los resultados favorables reportados tras el tratamiento con rituximab en nuestros pacientes parecen confirmar la eficacia del farmaco en el sindrome nefrotico cortico-dependiente y lo hacen considerar como una opcion terapeutica valida en casos de dificil manejo clinico en la edad pediatrica. Aun asi, parece recomendable disenar estudios apropiados que traten de aclarar, entre otros, el regimen optimo de tratamiento (dosis, intervalo y numero de ciclos), repercusion clinica y potenciales efectos adversos a largo plazo.

Background: Unplanned extubations are safety events relatively frequent in the neonatal intensive care units (NICU). This study aimed to describe the frequency and characteristics of unplanned extubations in a NICU. Methods: We conducted a retrospective observational study of unplanned extubations in the NICU of a tertiary regional referral hospital. We reviewed medical records for data collection and performed the statistical analysis, comparing the cases of unplanned extubations with those in which it did not occur among all the cases that received intubation and invasive neonatal mechanical ventilation. Results: A total of 958 newborns were admitted to the NICU, of which 174 required assistance with invasive mechanical ventilation (18.1%) and 28 experienced unplanned extubations (16.1%): 25 patients with one episode, one with two episodes, and two with three episodes. The rate was 2.93 unplanned extubations for every 100 days of invasive mechanical ventilation in 5 years, with a significant decrease in the last three years (p = 0.0158). We found a statistically significant correlation between a weight < 1500 g and unplanned extubation in the multivariate analysis, although sedation appears to affect its interaction. Conclusions: Unplanned extubations are a relatively frequent problem, although with a tendency to decrease in recent years. The weight of the patients at birth and sedation during ventilation are important factors in this safety problem.

Background Neonatal sepsis is a condition with high mortality and morbidity that contributes to high rates of antibiotic therapy at birth. In addition, very low birth weight newborns (VLBWN) are particularly vulnerable. Interleukin 6 (IL-6) seems to be an early and effective marker that could help a better selection of patients to be treated. This study aimed to evaluate the use of antibiotics in the first 72 hours of life in VLBW infants before and after using IL-6 as an infection marker. Also, we wanted to analyze the differences in morbidity and mortality during admission and other factors associated with the decision to start antibiotic treatment. Methods We conducted a cohort retrospective study. We included VLBWN born in our hospital or admitted before 72 hours of life in two two-year periods (2007-2008 and 2011-2012). Results Antibiotics use during the first 72 hours of life was analyzed as the primary variable, which was reduced by 20% on the second period (p = 0.002). Regarding the analysis of secondary variables, we found no significant differences in mortality during hospital admission and the incidence of nosocomial sepsis, enterocolitis, or invasive fungal infection. The multivariate analysis indicated extreme prematurity and the study group as the most strongly related factors to the start of antibiotic therapy. Conclusions IL-6 was a useful marker of infection to reduce the use of antibiotic therapy in VLBW infants without increasing mortality.

espanolIntroduccion: cuando no se dispone de leche materna propia, la leche materna donada es la mejor alternativa para asegurar los requerimientos nutricionales de los recien nacidos prematuros o enfermos. Objetivos: conocer el contenido de macronutrientes de la leche materna donada y como varian estos en los distintos periodos de la lactancia. Material y metodos: se analizo de forma retrospectiva la composicion nutricional de 822,423 litros de leche materna donada, procedentes de 160 donantes del banco de leche materna de un hospital de tercer nivel entre el 1 de febrero de 2017 y el 31 de diciembre de 2019. Resultados: el contenido en lipidos y carbohidratos se mantiene constante a lo largo de la lactancia; sin embargo, el contenido proteico disminuye inicialmente para luego volver a aumentar a partir del segundo ano de lactancia. Conclusiones: los bancos de leche materna deben conocer las variaciones del contenido en macronutrientes de la leche donada para optimizar la nutricion del recien nacido prematuro. EnglishIntroduction: when own mother´s breast milk is unavailable, donor human milk is the best option to guarantee the nutritional requirements of preterm or ill newborns. Objectives: to analyze the macronutrient composition of donor breast milk and its evolution over lactation periods. Material and methods: we retrospectively analyzed the nutritional composition of 822.423 L of donor breast milk from 160 donors at the human milk bank of a third-level hospital between February 1, 2017 and December 31, 2019. Results: lipid and carbohydrate composition remains constant throughout lactation. However, protein content decreases initially to increase again starting in the second year of lactation. Conclusions: donor human milk banks should be aware of the variations in macronutrient composition donor human milk shows in order to optimize nutrition for preterm newborns.

X-linked hypophosphatemia (XLH) causes significant burden in pediatric patients in spite of maintained treatment with phosphate supplements and vitamin D derivatives. Administration of burosumab has shown promising results in clinical trial but studies assessing its effect in the everyday practice are missing. With this aim, we analyzed the response to one-year treatment with burosumab, injected subcutaneously at 0.8 mg/kg every 2 weeks, in five children (three females) aged from 6 to 16 years, with genetically confirmed XLH. Patients were being treated with phosphate and vitamin D analogs until the beginning of burosumab treatment. In all children, burosumab administration led to normalization of serum phosphate in association with marked increase of tubular reabsorption of phosphate and reduction of elevated serum alkaline phosphatase levels. Baseline height of patients, from -3.56 to -0.46 SD, increased in the three prepubertal children (+0.84, +0.89, and +0.16 SD) during burosumab treatment. Growth improvement was associated with reduction in body mass index (-1.75, -1.47, and -0.17 SD, respectively), suggesting a salutary effect of burosumab on physical activity and body composition. Burosumab was well-tolerated, mild local pain at the injection site and transient and mild headache following the initial doses of burosumab being the only reported undesirable side effects. No patient exhibited hyperphosphatemia, progression of nephrocalcinosis, worsening of metabolic control or developed hyperparathyroidism. Mild elevation of serum PTH present at the beginning of treatment in one patient 4 was not modified by burosumab administration. These results indicate that in the clinical setting, beyond the strict conditions and follow-up of clinical trials, burosumab treatment for 1 year exerts positive effects in pediatric patients with XLH without major adverse events.