Evaluation of cerebral autoregulation of oxygen by NIRS method during postnatal transition period in term and late preterm newborns without resuscitation requirement.

BACKGROUND: Pulse oximetry is commonly used to monitor arterial oxygen saturation and heart rate during the transition period and reference intervals have been determined. However, the effect of the change in arterial oxygen saturation on tissue oxygenation does not seem to be the same. So, a non-invasive method for monitoring cerebral or regional tissue oxygenation will be potentially useful for vulnerable infants. This study aims to evaluate the effectiveness of cerebral autoregulation in the first 10 min after delivery in term and late preterm newborns without resuscitation requirement. METHODS: Cerebral tissue oxygen saturation was measured in the first 10 min after birth with near-infrared spectroscopy (NIRS) from the left forehead. Peripheral oxygen saturation was measured with pulse oximetry from the right hand and cerebral fractional tissue oxygen extraction was calculated. RESULTS: Nineteen late preterms and 20 term infants were included in the study. There was no statistically significant difference between median cerebral tissue oxygen saturation and cerebral fractional tissue oxygen extraction values of late preterm and term infants (p < 0.001). There was a strong inverse relationship between cerebral tissue oxygen saturation and cerebral fractional tissue oxygen extraction (p < 0.001). CONCLUSIONS: In late preterm infants similar to term infants, arterial oxygen saturation and cerebral tissue oxygen saturation increased with time, but inverse reduction of cerebral fractional tissue oxygen extraction showed the presence of an active autoregulation in the brain. This can be interpreted as the ability of the brain to protect itself from hypoxia by regulating oxygen uptake during normal fetal-neonatal transition process. A larger scale multi-center randomized control trial is now needed to further inform practice.

Neonatal Outcomes of Critical Congenital Heart Defects: A Multicenter Epidemiological Study of Turkish Neonatal Society : Neonatal Outcomes of CCHD.

Critical congenital heart disease (CCHD) is one of the leading causes of neonatal and infant mortality. We aimed to elucidate the epidemiology, spectrum, and outcome of neonatal CCHD in Türkiye. This was a multicenter epidemiological study of neonates with CCHD conducted from October 2021 to November 2022 at national tertiary health centers. Data from 488 neonatal CCHD patients from nine centers were entered into the Trials-Network online registry system during the study period. Transposition of great arteria was the most common neonatal CHD, accounting for 19.5% of all cases. Sixty-three (12.9%) patients had extra-cardiac congenital anomalies. A total of 325 patients underwent cardiac surgery. Aortic arch repair (29.5%), arterial switch (25.5%), and modified Blalock-Taussig shunt (13.2%). Overall, in-hospital mortality was 20.1% with postoperative mortality of 19.6%. Multivariate analysis showed that the need of prostaglandin E1 before intervention, higher VIS (> 17.5), the presence of major postoperative complications, and the need for early postoperative extracorporeal membrane oxygenation were the main risk factors for mortality. The mortality rate of CCHD in our country remains high, although it varies by health center. Further research needs to be conducted to determine long-term outcomes for this vulnerable population.

Effect of partially hydrolyzed synbiotic formula milk on weight gain of late preterm and term infants-a multicenter study.

Introduction: Data on the effectiveness of hydrolyzed infant formula containing both pre- and probiotics (synbiotic formula) on the growth of infants is still scarce. This retrospective study was designed to evaluate the effect of a partially hydrolyzed synbiotic formula on growth parameters and the possible occurrence of major gastrointestinal adverse events or morbidities in infants born via cesarean section (C-section) delivery. Methods: C-section-delivered term and late preterm infants who received either partially hydrolyzed synbiotic formula, standard formula, or maternal milk and followed at seven different hospitals from five different regions of Turkey, during a 1-year period with a minimum follow-up duration of 3 months were evaluated retrospectively. All the included infants were evaluated for their growth patterns and any kind of morbidity such as diarrhea, constipation, vomiting, infection, or history of hospitalization. Results: A total of 198 infants (73 in the human milk group, 61 in the standard formula group, and 64 in the partially hydrolyzed synbiotic formula group) reached the final analysis. The groups were similar regarding their demographic and perinatal characteristics. No difference was observed between the three groups regarding gastrointestinal major side effects. Growth velocities of the infants in the human milk and partially hydrolyzed synbiotic formula groups during the first month of life were similar whereas the weight gain of infants in the standard formula group was significantly less than these two groups (p < 0.001). Growth velocities were similar among the three groups between 1st and 3rd months of age. Discussion: A partially hydrolyzed synbiotic formula provided better weight gain in late-preterm and term infants who were delivered via C-section delivery compared to the standard formula during the first month of life. This weight gain was similar to the infants receiving exclusively human milk. This difference was not observed in length and head circumference gain. No difference was observed in any of the parameters during the 1st-3rd months of age. Specially formulated partially hydrolyzed synbiotic formulas may reverse at least some of the negative impacts of C-section delivery on the infant and help to provide better growth, especially during the early periods of life.

Evaluation and management of neonatal onset hyperinsulinemic hypoglycemia: a single neonatal center experience.

OBJECTIVES: To evaluate the clinical characteristics and treatment options of neonates requiring prolonged hospitalization due to persistent hyperinsulinemic hypoglycemia (HH). METHODS: This retrospective cohort study included infants >34 weeks of gestation at birth who were born in our hospital between 2018 and 2021, diagnosed with HH, and required diazoxide within the first 28 days of life. The baseline clinical characteristics, age at the time of diagnosis and treatment options in diazoxide resistance cases were recorded. Genetic mutation analysis, if performed, was also included. RESULTS: A total of 32 infants diagnosed with neonatal HH were followed up. Among the cohort, 25 infants were classified as having transient form of HH and seven infants were classified as having congenital hyperinsulinemic hypoglycemia (CHI). Thirty-one percent of the infants had no risk factors. The median birth weight was significantly higher in the CHI group, whereas no differences were found in other baseline characteristics. Patients diagnosed with CHI required higher glucose infusion rate, higher doses, and longer duration of diazoxide treatment than those in the transient HH group. Eight patients were resistant to diazoxide, and six of them required treatment with octreotide and finally sirolimus. Sirolimus prevented the need of pancreatectomy in five of six patients without causing major side effects. Homozygous mutations in the ABCC8 gene were found in four patients with CHI. CONCLUSIONS: The risk of persistent neonatal hyperinsulinism should be considered in hypoglycemic neonates particularly located in regions with high rates of consanguinity. Our study demonstrated sirolimus as an effective treatment option in avoiding pancreatectomy in severe cases.

Neurodevelopment of patients who received intravitreal bevacizumab or aflibercept for type 1 and aggressive retinopathy of prematurity.

PURPOSE: We aimed to compare the neurodevelopment of patients who received intravitreal bevacizumab (IVB) and intravitreal aflibercept (IVA) for type 1 retinopathy of prematurity (ROP) and aggressive ROP (A-ROP) using the Ages and Stages Questionnaire (ASQ-3) test. METHODS: Patients who underwent IVB (group 1), IVA (group 2), and patients who did not receive treatment (group 3) were included in the prospective-controlled study. The patients were grouped as low-intermediate-high risk according to the high-risk infant follow-up guide. With the ASQ-3 test, fine and gross motor movements, communication, problem solving, and personal-social developments of the patients were compared. RESULTS: The gender distribution, birth weight (BW), and neonatal risk category of the groups were similar. Gestational age (GA) of group 1 was found to be lower compared to group 3. There was no difference between the groups in the development of gross and fine motor movements, communication and problem solving. The personal and social development of group 1 and group 2 was found to be retarded compared to the control group. DISCUSSION: As a result, the detection of retardation in the personal and social areas in the treated patients, and the detection of retardation in other areas, although not significant, reveals the necessity of following the development of these patient groups and providing the necessary support in the areas where retardation is detected.

Comparison of Three Different Multiple Organ Dysfunction Scores for Predicting Mortality after Neonatal Cardiac Surgery.

Infants who undergo cardiac surgery frequently have complications that may advance to multiple organ failure and result in mortality. This study aims to compare three different multiple organ dysfunction scoring systems: the Neonatal Multiple Organ Dysfunction (NEOMOD) score, the modified NEOMOD score, and the Pediatric Logistic Organ Dysfunction-2 (PELOD-2) score in predicting postoperative 30-day mortality in neonates undergoing cardiac surgery. This retrospective cohort study was conducted between January 2019 and February 2021 in a single unit on neonates operated on due to congenital heart disease in the first 28 days of life. Patients who underwent off-pump surgeries were excluded from the study. The NEOMOD, modified NEOMOD, and PELOD-2 scores were calculated for each of the first 3 days following surgery. A total of 138 patients were included. All scores had satisfactory goodness-of-fit and at least good discriminative ability on each day. The modified NEOMOD score consistently demonstrated the best prediction among these three scores after the first day, reaching its peak performance on day 2 (area under curve: 0.824, CI: 0.75-0.89). Our findings suggest that NEOMOD and modified NEOMOD scores in the first 72 h could potentially serve as a predictor of mortality in this population.

Retrospective validation of the postnatal growth and retinopathy of prematurity (G-ROP) and Colorado retinopathy of prematurity (CO-ROP) models in a Turkish cohort.

BACKGROUND: The aim of this study was to investigate the effectiveness of the Postnatal Growth and Retinopathy of Prematurity (G-ROP) and Colorado Retinopathy of Prematurity (CO-ROP) models in predicting the risk of Retinopathy of Prematurity (ROP) in preterm infants at a tertiary ROP diagnostic and treatment center. METHODS: The G-ROP and CO-ROP models were applied to the study group using the data obtained. The sensitivity and specificity of both models were then calculated. RESULTS: One hundred and twenty-six infants were included in the study. When the G-ROP model was applied to the study group, the model`s sensitivity at detecting any stage ROP was 88.7%, while it was 93.3% for the treated group. The specificity of the model was 10.9% for any stage ROP, and 11.7% for the treated group. For the CO-ROP model in the same study group, the sensitivity at detecting any stage ROP was 87.3%, while it was 100% for the treated group. The CO-ROP model's specificity was 40% for any stage ROP, and 27.9% for the treated group. When cardiac pathology criteria were introduced to both models, the sensitivity of the G-ROP and CO-ROP models increased to 94.4% and 97.2%, respectively. CONCLUSIONS: It was found that the G-ROP and CO-ROP models are simple and effective models for predicting any degree of ROP development, but that they are unable to be 100% accurate. When the models were modified by introducing cardiac pathology criteria, it was observed that they began to produce more accurate results. Studies with larger groups are needed in order to assess the applicability of the modified criteria.

A novel marker for predicting type 1 retinopathy of prematurity: C-reactive protein/albumin ratio.

PURPOSE: We aimed to investigate whether the C-reactive protein (CRP) to albumin ratio (CAR) an inflammatory predictor can be used as a marker for the development of ROP. METHODS: Gestational age, birth weight, gender, neonatal, and maternal risk factors were recorded. The patients were divided into two groups: those who did not develop ROP (ROP -) and those who developed ROP (ROP +). The ROP + group was further separated into two groups: those who required treatment (ROP + T) and those who were not treated (ROP + NT). The following parameters were noted in the first postnatal week and at the end of the first postnatal month: CRP, albumin, CAR, white blood cell (WBC), neutrophil, lymphocyte, neutrophil-to-lymphocyte ratio (NLR), distribution red cell width (RDW), platelet (Plt), and RDW/platelet ratio. RESULTS: We evaluated 131 premature infants who met the inclusion criteria. There was no difference between the main groups in hemogram parameters and CAR at the postnatal first week. WBC count (p = 0.011), neutrophil count (p = 0.002), and NLR were high (p = 0.004) in the ROP + group at the end of the postnatal 1st month. The CAR level at the end of the first month was higher in the ROP + group (p = 0.027). CAR was similar between the ROP + T and ROP + NT groups (p = 0.112) in the postnatal first week but higher in the treatment-required group at the end of the first month (p < 0.01). CONCLUSION: High CAR and high NLR at the end of the postnatal first month can be used to predict the development of severe ROP.

The Effect of Continuous PEEP Administration during Surfactant Instillation on Cerebral Hemodynamics in Intubated Preterm Infants: A NIRS Study.

OBJECTIVE: There is an ongoing debate about the best and comfortable way to administer surfactant. We hypothesized that uninterrupted respiratory support and continuous PEEP implementation while instilling surfactant via endotracheal tube (ETT) with side port will result in higher regional cerebral tissue oxygenation (rcSO2) and the alterations in cerebral hemodynamics will be minimal. STUDY DESIGN: Preterm infants who required intubation in the delivery room and/or in the first 24 hours of life with gestational age <32 were enrolled. Patients were intubated either via conventional ETT or ETT with side port (Vygon) with appropriate sizes. Following neonatal intensive care unit admission a near-infrared spectroscopy (NIRS) probe was placed on the forehead and each infant was started to be monitored with NIRS. In conventional ETT group, patients separated from the ventilator while surfactant was instilled. In ETT with side port group, respiratory support was not interrupted during instillation. Heart rate, oxygen saturation, rcSO2, cerebral fractional tissue oxygen extraction (cFTOE), and blood pressures were recorded. RESULTS: A total of 46 infants were analyzed. Surfactant was instilled with conventional ETT in 23 and ETT with side port in 23 infants. Birth weights (1,037 ± 238 vs. 1,152 ± 277 g) and gestational ages (28 ± 2.3 vs. 29 ± 1.6 weeks) did not differ between groups. During instillation of surfactant, rcSO2 levels [61.5 (49-90) vs. 70 (48-85)] and cFTOE levels 0.28 (0.10-0.44) vs. 0.23 (0.03-0.44)] were similar (p = 0.58 and 0.82, respectively). CONCLUSION: Interruption of respiratory support during surfactant instillation did not significantly alter the cerebral tissue oxygenation. These results did not support our hypothesis and should be confirmed with further studies. KEY POINTS: · Monitoring intracerebral oxygenation changes during surfactant administration with NIRS is feasible.. · The surfactant administration method does not significantly alter the cerebral oxygenation.. · Surfactant administration itself rather than the method caused a transient drop in cerebral NIRS readings..

A Better Way for Ductal Stenting in Patients with Duct-Dependent Pulmonary Flow and Vertical and Tortuous Patent Ductus Arteriosus.

BACKGROUND: In this study, we aimed to compare the femoral route and the carotid artery route in terms of procedural success of ductal stent implantation in patients with ductdependent pulmonary blood flow. METHODS: The study included 51 patients with duct-dependent pulmonary circulation who underwent ductal stent implantation upon their admission to our clinic between July 2017 and March 2021. In total, 23 patients (group I) underwent ductal stent implantation via the femoral route, while the remaining 28 (group II) underwent the procedure via the carotid artery. The groups were compared in terms of procedural success, time, post-procedural blood pH, lactate levels, and complications. RESULTS: Duct morphology was observed in group 1 as follows: type 1 in 12 patients, type 3 in 8, type 2 in 2, and type 6 in 1 patient. In group 2, 26 patients had type 3, 1 had type 2, and 1 had type 6. The tortuosity index of the patients in group 1 was 1 in 8 patients, 2 in 8 patients, and 3 in 7 patients, while in group 2, it was 1 in 5 patients, 2 in 15 patients, and 3 in 8 patients. The success rate was 69.6% (16/26) in group I and 93.5% (29/31) in group II (P=.030). The cumulative success rate was 88.2% (45/51). The procedural durations were 78.2 ± 34.1 and 52.1 ± 22.0 minutes in group I and group II, respectively (P=.002). The mean blood pH values upon the completion of the procedure were 7.26 ± 0.1 and 7.33 ± 0.0 in group I and group II, respectively (P=.038). The mean post-procedural lactate levels were 2.8 mmol/L and 2.3 mmol/L in group I and group II, respectively (P=.038). The 2 groups did not show any differences in terms of procedural complications. CONCLUSION: The carotid artery route can be preferred, especially in vertical and tortuous ductus arteriosus, as it is associated with a high success rate and a short procedural time, as well as a better metabolic condition after the procedure.

Severe antenatal intraventricular hemorrhage in a newborn with WASP pathogenic variant.

Wiskott-Aldrich syndrome (WAS) is a rare X-linked immunodeficiency disorder caused by abnormal expression of the Wiskott-Aldrich syndrome protein due to WAS gene mutation, usually characterized by microthrombocytopenia, eczema, hematological malignancies, recurrent infections, and a high risk of autoimmune complications. In this report, we present a family presenting with severe intrauterine cranial hemorrhage. The family has novel c.1377_1378dup (p.Pro460Hisfs*12) variant of WAS gene. The severe and early onset clinic in the family seems to be related to location of the variant on VCA domain of the WAS protein.

Comparison of Assist/Control Ventilation with and without Volume Guarantee in Term or Near-Term Infants.

OBJECTIVES: This study aimed to compare the effects of volume guarantee (VG) combined with assist/control (AC) ventilation to AC alone on hypocarbia episodes and extubation success in infants born at or near term. METHODS: In this prospective cohort study, infants >34 weeks of gestation at birth, who were born in our hospital supported by synchronized, time-cycled, pressure limited, assist/control ventilation (AC) or assist-controlled VG mechanical ventilation (AC + VG) were included. After admission, infants received either AC or VG + AC using by Leoni Plus ventilator. The ventilation mode was left to the clinician. In the AC group, peak airway pressure was set clinically. In the VG + AC group, desired tidal volume was set at 5 mL/kg, with the ventilator adjusting peak inspiratory pressure to deliver this volume. The study was completed once the patient extubated. RESULTS: There were 35 patients in each group. Incidence of hypocarbia was lower in the VG + AC compared with AC (%17.1 and 22.8%, respectively) but statistically not significant. Out-of-range partial pressure of carbon dioxide (PCO2) levels were lower in the VG + AC group and it reached borderline statistical significance (p = 0.06). The median extubation time was 70 (42-110) hours in the VG + AC group, 89.5 (48.5-115.5) hours in the AC group, and it did not differ between groups (p = 0.47). CONCLUSION: We found combining AC and VG ventilation compared with AC ventilation alone yielded similar hypocarbia episodes and extubation time for infants of >34 gestational weeks with borderline significance lower out-of-range PCO2 incidence. KEY POINTS: · Underlying lung pathology requiring mechanical ventilation support in term infant is heterogeneous.. · VG ventilation compared with conventional modes yielded similar hypocarbia episodes in term infants.. · Combining VG ventilation lead to borderline significance lower out-of-range PCO2 incidence..

Ventilator-delivered nasal intermittent positive pressure ventilation versus nasal biphasic positive airway pressure following extubation in infants ≤1250 g birth weight: a randomized trial.

BACKGROUND AND OBJECTIVE: To determine the effectiveness of ventilator-delivered nasal intermittent positive pressure ventilation and nasal biphasic positive airway pressure (n-BiPAP) after first extubation attempt in infants ≤1250-g birthweight. METHODS: This randomized controlled study included mechanically ventilated preterm infants of ≤1250-g birthweight who were randomly assigned to ventilator-delivered NIPPV or n-BiPAP at first extubation within 2 weeks of age. The primary outcome (extubation failure within 96 h following extubation) and secondary outcomes were compared. RESULTS: Extubation failure occurred in 22 of 74 infants in n-BiPAP group and 34 of 75 infants in NIPPV group (OR 0.51, 95% CI: 0.26-1.002; p = .05). Duration of invasive and noninvasive ventilation were found to be similar between groups. Also, there were no significant differences among groups for intraventricular hemorrhage, medically/surgically treated patent ductus arteriosus, necrotizing enterocolitis, BPD or death. CONCLUSION: Sustained extubation in infants ≤1250-g birthweight and <2 weeks age did not differ between ventilator-delivered NIPPV and n-BiPAP. TRIAL REGISTRATION: Clinical Trials.gov under identifier NCT02842190.

Is arterial switch operation possible with neonatology-focused intensive care unit modality.

BACKGROUND: In this study, we aimed to examine the feasibility of arterial switch operation and its perioperative management with neonatology-focused intensive care modality in a region of Turkey where the birth rate and the number of asylum seekers who had to leave their country due to regional conflicts are high. METHODS: Between December 2017 and June 2020, a total of 57 patients (48 males, 9 females; median age: 12.2 days; range, 2 to 50 days) who were diagnosed with transposition of the great arteries in our clinic and underwent arterial switch operation were retrospectively analyzed. All patients were followed by the neonatologist in the neonatal intensive care unit during the preoperative and postoperative period. RESULTS: Thirty-eight (66.7%) patients had intact ventricular septum, 16 (28.1%) had ventricular septal defect, two (3.5%) had coarctation of the aorta, and one (1.7%) had Taussig-Bing anomaly. Coronary artery anomaly was present in 14 (24.5%) patients. The most common complications in the intensive care unit were renal failure requiring peritoneal dialysis in seven (12.3%) patients, supraventricular tachyarrhythmia in six (10.5%) patients, and eight (14%) patients left their chests open. The median length of stay in intensive care unit was 13.8 (range, 9 to 25) days and the median length of hospital stay was 24.5 (range, 16 to 47) days. The overall mortality rate for all patients was 12.3% (n=7). The median follow-up was 8.2 months. A pulmonary valve peak Doppler gradient of ≥36 mmHg was detected in five patients (8.7%) who were followed, and these patients were monitored by providing medical treatment. None of the patients needed reoperation or reintervention. CONCLUSION: We believe that arterial switch operation, one of the complex neonatal cardiac surgery, can be performed with an acceptable mortality and morbidity rate with the use of neonatology-focused intensive care modality, which is supported by pediatric cardiology and pediatric cardiac surgery.

Is Nasal Septum-Tragus Length Measurement Appropriate for Endotracheal Tube Intubation Depth in Neonates? A Randomized Controlled Study.

OBJECTIVE: Endotracheal intubation is a frequent procedure performed in neonates with respiratory distress. Clinicians use different methods to estimate the intubation insertion depth, but, unfortunately, the improper insertion results are very high. In this study, we aimed to compare the two different methods (Tochen's formula = weight in kilograms + 6 cm; and nasal septum-tragus length [NTL] + 1 cm) used to determine the endotracheal tube (ETT) insertion depth. STUDY DESIGN: Infants who had intubation indications were enrolled in this study. The intubation tube was fixed using the Tochen formula (Tochen group) or the NTL + 1 cm formula (NTL group). After intubation, the chest radiograph was evaluated (above T1, proper place, and below T2). RESULTS: A total of 167 infants (22-42 weeks of gestational age) were included in the study. The proper tube placement rate in both groups was similar (32.4 vs. 30.4% for infants < 34 weeks of gestational age and 56.8 vs. 45.0% in infants > 34 weeks of gestational age). The ETT was frequently placed below T2 at a higher rate in infants with a gestational age of <34 weeks, especially in the NTL group (46% in the Tochen group and 60.7% in the NTL group). CONCLUSION: The NTL + 1 cm formula led to a higher rate of ETT placement below T2, especially in infants with a birth weight of <1,500 g. Therefore, more studies are needed to determine the optimal ETT insertion depth.

Neonatal diabetes due to homozygous INS gene promoter mutations: Highly variable phenotype, remission and early relapse during the first 3 years of life.

Neonatal diabetes mellitus (NDM) is a rare form of monogenic diabetes presenting within the first 6 months of life. INS gene promoter mutations have been shown to cause both remitting/relapsing and permanent NDM. We, herein, present three interesting patients with INS gene promoter mutations. Two cousins with an identical homozygous c.-331C > G mutation presented with NDM. The first cousin had nonremitting diabetes and still requires multidose insulin injections at the current age of 6.1 years. However, the other cousin's diabetes remitted at the age of 9 months, and she is still in remission at the age of 3 years with no medication or dietary intervention required (latest HbA1c was 4.9%). The third patient had NDM also due to a homozygous INS promoter c.-331C>A mutation. Her diabetes remitted at the age of 2 months and relapsed at the age of 2.6 years with severe diabetic ketoacidosis (DKA). Distinct clinical phenotype and relapse with severe DKA in one of the three cases suggest that INS promotor mutations can cause a heterogeneous phenotype and even cases exhibiting remission can relapse unpredictably. Therefore, as the age of relapse is unpredictable, close follow-up and family education on diabetes symptoms are essential for cases with remitting/relapsing diabetes due to INS gene mutations.

Dolor y variabilidad de la frecuencia cardíaca en recién nacidos prematuros que recibieron surfactante: un estudio piloto / Neonatal pain and heart rate variability in preterm infants treated with surfactant: a pilot study

Objetivo. Evaluar la percepción del dolor de recién nacidos prematuros a quienes se les administró surfactante mediante diferentes técnicas, utilizando la variabilidad de la frecuencia cardíaca (VFC).Métodos. Se aleatorizó a los recién nacidos que requirieron tratamiento con surfactante por SDR a los grupos INSURE o MIST. El análisis de la VFC se realizó con la tecnología NIPE para evaluar el componente parasimpático del sistema nervioso autónomo de los recién nacidos. Se registró la VFC antes, durante y después de administrar el surfactante. La evaluación del dolor se determinó con la escala PIPP. Resultados. Se incluyó a 14 recién nacidos en el estudio. Los grupos tenían características demográficas similares. Los puntajes de la escala PIPP no difirieron entre los grupos INSURE y MIST (p = 0,05). Se observó una diferencia estadísticamente significativa en la mediana de la VFC durante la administración del surfactante entre los grupos INSURE y MIST (52 frente a 56, p = 0,03). El análisis de la VFC fue similar entre los grupos antes y después de administrar el surfactante.Conclusión. La administración de surfactante mediante la técnica MIST podría ser más cómoda para los recién nacidos prematuros con SDR. No obstante, es necesario realizar otros estudios con series más importantes.

Objective. We aimed to assess the pain perception of preterm infants treated with different surfactant administration techniques by using heart rate variability (HRV).Methods. Preterm infants who required surfactant therapy for RDS were randomized to INSURE or MIST groups. HRV analysis was performed by Newborn Infant Parasympathetic Evaluation monitor. HRV was recorded before, during and after surfactant administration. Pain assessment was determined by Premature Infant Pain Profile (PIPP) score.Results. Fourteen infants were enrolled in the study. Demographic characteristics of the groups were similar. PIPP scores did not differ between INSURE and MIST groups (p = 0.05). Statistically significant difference in median HRV during surfactant administration was observed between INSURE and MIST groups (52 vs. 56, p = 0.03). HRV analysis was similar between groups before and after surfactant administration. Conclusion. Surfactant administration with MIST technique might be more comfortable for preterm infants with RDS. However further studies with larger series are needed.

Neonatal pain and heart rate variability in preterm infants treated with surfactant: a pilot study. / Dolor y variabilidad de la frecuencia cardíaca en recién nacidos prematuros que recibieron surfactante: un estudio piloto.

OBJECTIVE: We aimed to assess the pain perception of preterm infants treated with different surfactant administration techniques by using heart rate variability (HRV). METHODS: Preterm infants who required surfactant therapy for RDS were randomized to INSURE or MIST groups. HRV analysis was performed by Newborn Infant Parasympathetic Evaluation monitor. HRV was recorded before, during and after surfactant administration. Pain assessment was determined by Premature Infant Pain Profile (PIPP) score. RESULTS: Fourteen infants were enrolled in the study. Demographic characteristics of the groups were similar. PIPP scores did not differ between INSURE and MIST groups (p = 0.05). Statistically significant difference in median HRV during surfactant administration was observed between INSURE and MIST groups (52 vs. 56, p = 0.03). HRV analysis was similar between groups before and after surfactant administration. CONCLUSION: Surfactant administration with MIST technique might be more comfortable for preterm infants with RDS. However further studies with larger series are needed.

Objetivo. Evaluar la percepción del dolor de recién nacidos prematuros a quienes se les administró surfactante mediante diferentes técnicas, utilizando la variabilidad de la frecuencia cardíaca (VFC). Métodos. Se aleatorizó a los recién nacidos que requirieron tratamiento con surfactante por SDR a los grupos INSURE o MIST. El análisis de la VFC se realizó con la tecnología NIPE para evaluar el componente parasimpático del sistema nervioso autónomo de los recién nacidos. Se registró la VFC antes, durante y después de administrar el surfactante. La evaluación del dolor se determinó con la escala PIPP. Resultados. Se incluyó a 14 recién nacidos en el estudio. Los grupos tenían características demográficas similares. Los puntajes de la escala PIPP no difirieron entre los grupos INSURE y MIST (p = 0,05). Se observó una diferencia estadísticamente significativa en la mediana de la VFC durante la administración del surfactante entre los grupos INSURE y MIST (52 frente a 56, p = 0,03). El análisis de la VFC fue similar entre los grupos antes y después de administrar el surfactante. Conclusión. La administración de surfactante mediante la técnica MIST podría ser más cómoda para los recién nacidos prematuros con SDR. No obstante, es necesario realizar otros estudios con series más importantes.

Miopatía nemalínica tratada con L-tirosina para aliviar los síntomas en un recién nacido / Nemaline rod myopathy treated with L-tyrosine to relieve symptoms in a neonate

La miopatía nemalínica es un trastorno heterogéneo definido por la presencia de estructuras con forma de bastones, conocidas como cuerpos nemalínicos (o bastones de nemalina). El diagnóstico se funda en la debilidad muscular, además de la visualización de cuerpos nemalínicos en la biopsia muscular. La miopatía nemalínica no tiene cura. Las estrategias terapéuticas para este trastorno son sintomáticas y empíricas. En este artículo, presentamos el caso de una recién nacida con insuficiencia respiratoria grave y debilidad muscular generalizada, a la que se le diagnosticó miopatía nemalínica a través de la biopsia muscular. La paciente tuvo una notable disminución de la sialorrea y una mejora de los movimientos espontáneos después del tratamiento con L-tirosina. Este caso se presenta para destacar la importancia de la biopsia muscular en el diagnóstico diferencial de la hipotonía grave durante el período neonatal y el posible beneficio del aporte suplementario de L-tirosina para disminuir la sialorrea y restaurar la fuerza muscular.

Nemaline myopathy (NM) is a heterogeneous disorder defined by the presence of rod-shaped structures known as nemaline bodies or rods. The diagnosis is based on muscle weakness, combined with visualization of nemaline bodies on muscle biopsy. There is no curative treatment for nemaline myopathy. Therapeutic strategies for this condition are symptomatic and empirical. Herein, we present a newborn with severe respiratory failure and generalized muscle weakness, who was diagnosed as NM by muscle biopsy. The patient experienced remarkable decrease in sialorrhea and improvement of spontaneous movements after L-tyrosine treatment. This case is presented to emphasize the importance of muscle biopsy in the differential diagnosis of severe hypotonia during neonatal period and a possible benefit of L-tyrosine supplementation for decreasing sialorrhea and restoring muscle strength.