The usefulness of universal ultrasound before hospital discharge for early diagnosing hip dysplasia in Korean neonates with breech presentation in a single center: A retrospective study.

Developmental dysplasia of the hip (DDH) is a broad-spectrum disorder. Early diagnosis and treatment are important for improved prognosis and a lower risk of long-term complications. Selecting high-risk infants is important for the early diagnosis of DDH using ultrasonography; however, there are no standard international guidelines. This study aimed to identify the usefulness of universal ultrasound before hospital discharge in breech-born neonates and proposes selective ultrasound for high-risk patients. A retrospective chart review was conducted to identify breech-born neonates who underwent hip ultrasonography before discharge for the detection of DDH between 2019 and 2023. Patients were categorized into DDH and non-DDH groups according to the ultrasound results. We compared sex, gestational age, birth weight, first-born status, twin pregnancy, associated anomalies, presence of symptoms, physical examination results, and timing of the first hip ultrasound. The medical records of the mothers were reviewed to identify the amount of amniotic fluid and duration of breech presentation. This study included 102 patients, of whom 62 and 40 were assigned to the non-DDH and DDH groups, respectively. Congenital anomalies, positive symptoms, and positive physical examination results were significant risk factors. However, female sex, first-born status, and oligohydramnios were not statistically significant. The duration of breech presentation during pregnancy was not significant. Additionally, the risk of Pavlik harnesses was higher in patients who underwent a positive physical examination. Universal ultrasonography before discharge is not recommended for the early diagnosis of DDH in all breech-born neonates because of the high rate of overdiagnosis. We recommend that ultrasonography be performed in patients with congenital anomalies, except for foot problems, or in those with a positive physical examination conducted by trained specialists.

Risk factors and clinical outcomes of extubation failure in very early preterm infants: a single-center cohort study.

BACKGROUND: Early extubation success (ES) in preterm infants may reduce various mechanical ventilation-associated complications; however, extubation failure (EF) can cause adverse short- and long-term outcomes. Therefore, the present study aimed to identify differences in risk factors and clinical outcomes between ES and EF in very early preterm infants. METHODS: This retrospective study was conducted between January 2017 and December 2021. Premature infants born at 32 weeks' gestational age in whom extubation had failed at least once were assigned to the EF group. Successfully extubated patients with a similar gestational age and birth weight as those in the EF group were assigned to the ES group. EF was defined as the need for re-intubation within 120 h of extubation. Various variables were compared between groups. RESULTS: The EF rate in this study was 18.6% (24/129), and approximately 80% of patients with EF required re-intubation within 90.17 h. In the ES group, there was less use of inotropes within 7 days of life (12 [63.2%] vs. 22 [91.7%], p = 0.022), a lower respiratory severity score (RSS) at 1 and 4 weeks (1.72 vs. 2.5, p = 0.026; 1.73 vs. 2.92, p = 0.010), and a faster time to reach full feeding (18.7 vs. 29.7, p = 0.020). There was a higher severity of bronchopulmonary dysplasia BPD (3 [15.8%] vs. 14 [58.3%], p = 0.018), longer duration of oxygen supply (66.5 vs. 92.9, p = 0.042), and higher corrected age at discharge (39.6 vs. 42.5, p = 0.043) in the EF group. The cutoff value, sensitivity, and specificity of the respiratory severity score (RSS) at 1 week were 1.98, 0.71, and 0.42, respectively, and the cutoff value, sensitivity, and specificity of RSS at 4 weeks were 2.22, 0.67, and 0.47, respectively. CONCLUSIONS: EF caused adverse short-term outcomes such as a higher BPD severity and longer hospital stay. Therefore, extubation in very early preterm infants should be carefully evaluated. Using inotropes, feeding, and RSS at 1 week of age can help predict extubation success.

Clinical and Laboratory Findings of Nosocomial Sepsis in Extremely Low Birth Weight Infants According to Causative Organisms.

BACKGROUND: nosocomial sepsis remains a significant source of morbidity and mortality in extremely low birth weight (ELBW) infants. Early and accurate diagnosis is very important, but it is difficult due to the similarities in clinical manifestation between the causative microorganisms. We tried to identify the differences between causative microorganisms in clinical and laboratory findings and to help choose antibiotics, when sepsis was suspected in ELBW infants. METHODS: a retrospective study was conducted on preterm infants, born at less than 28 weeks of gestation, with a birth weight of less than 1000 g between January 2009 and December 2019. Clinical and laboratory findings of suspected sepsis, after the first 72 h of life, were assessed. We classified them into four groups according to blood culture results (gram positive, gram negative, fungal, and negative culture groups) and compared them. RESULTS: a total of 158 patients were included after using the exclusion criteria, with 45 (29%) in the gram positive group, 35 (22%) in the gram negative group, 27 (17%) in the fungal group, and 51 (32%) in the negative culture group. There were no significant differences in mean gestational age, birth weight, and neonatal morbidities, except for the age of onset, which was earlier in the fungal group than other groups. White blood cell (WBC) counts were the highest in the gram negative group and the lowest in the fungal group. The mean platelet counts were the lowest in the fungal group. C-reactive protein (CRP) levels were the highest in the gram negative group, while glucose was the highest in the fungal group. CONCLUSIONS: in conclusion, we showed that there are some differences in laboratory findings, according to causative microorganisms in the nosocomial sepsis of ELBW infants. Increased WBC and CRP were associated with gram negative infection, while decreased platelet and glucose level were associated with fungal infection. These data may be helpful for choosing empirical antibiotics when sepsis is suspected.

Chromosomal Microarray in Children With Developmental Delay: The Experience of a Tertiary Center in Korea.

Background and Objectives: Chromosomal microarray (CMA) is a first-tier genetic test for children with developmental delay (DD), intellectual disability (ID), autism spectrum disorders (ASDs), and multiple congenital anomalies (MCA). In this study, we report our experiences with the use of CMA in Korean children with unexplained DD/ID. Methods: We performed CMA in a cohort of 308 children with DD/ID between January 2010 and September 2020. We also retrospectively reviewed their medical records. The Affymetrix CytoScan 750 K array with an average resolution of 100 kb was used to perform CMA. Results: Comorbid neurodevelopmental disorders were ASD (37 patients; 12.0%), epilepsy (34 patients; 11.0%), and attention deficit hyperactivity disorders (12 patients; 3.9%). The diagnostic yield was 18.5%. Among the 221 copy number variants (CNVs) identified, 70 CNVs (57 patients; 18.5%) were pathogenic. Deletion CNVs were more common among pathogenic CNVs (PCNVs) than in non-PCNVs (P < 0.001). The size difference between PCNVs and non-PCNVs was not significant (P = 0.023). The number of included genes within CNV intervals was significantly higher in PCNVs (average 8.6; 0-347) than in non-PCNVs (average 47.5; 1-386) (P < 0.001). Short stature and hearing difficulty were also more common in the PCNV group than in the non-PCNV group (P = 0.010 and 0.070, respectively). Conclusion: This study provides additional evidence for the usefulness of CMA in genetic testing of children with DD/ID in Korea. The pathogenicity of CNVs correlated with the number of included genes within the CNV interval and deletion type of the CNVs, but not with CNV size.

Using lactate dehydrogenase to predict the severity of respiratory distress in term newborn infants with no perinatal asphyxia.

BACKGROUND: We aimed to establish whether knowledge of lactate dehydrogenase (LDH) levels on day 1, as well as the change in these levels in the first three days, could be of clinical benefit in the diagnosis and/or prediction of severity of respiratory distress syndrome (RDS) and transient tachypnea of the newborn (TTN). METHODS: A retrospective study was conducted on 275 term infants (35 with RDS and 240 with TTN) admitted to the neonatal intensive care unit from January 2014 to June 2019. LDH levels were measured on admission and after three days. RESULTS: Both RDS and TTN groups had elevated LDH levels during admission. LDH levels were significantly higher in the RDS group than in the TTN group on both days. LDH levels in both groups significantly correlated with both the duration of respiratory support required, as well as the number of hospital days. We used these outcomes as a measure of severity of these conditions. CONCLUSIONS: In patients with respiratory distress, it may not be clinically useful to use LDH levels on day 1 to differentiate between RDS and TTN, despite the statistically significant differences, because of the overlapping values. However, LDH levels on day 1 and day 3 may predict the degree and duration of the required respiratory support for both RDS and TTN groups.

Genetic Evaluation of Congenital Hypothyroidism with Gland in situ Using Targeted Exome Sequencing.

OBJECTIVE: To analyze the genetic causes of congenital hypothyroidism through the targeted exome sequencing of pediatric patients with congenital hypothyroidism with thyroid gland in situ. METHOD: The study population included 20 patients diagnosed with congenital hypothyroidism with thyroid gland in situ at the Pediatric Endocrinology Clinic of Pusan National University Hospital. Targeted exome sequencing was performed on eight causative genes, including thyroid stimulating hormone receptor (TSHR), mutation in which can cause hypothyroidism with a small or normal sized thyroid gland, and thyroglobulin (TG), thyroid peroxidase (TPO), dual oxidase 2 (DUOX2), dual oxidase maturation factor 2 (DUOXA2), iodotyrosine deiodinase (IYD), solute carrier family 26 member 4 (SLC26A4), and solute carrier family 5 member 5 (SLC5A5), mutations in which are known to cause thyroid dyshormonogenesis. RESULTS: Permanent, subclinical, and transient hypothyroidism were diagnosed in 15 (75%), three (15%), and two (10%) patients, respectively. Genetic mutations were identified in 16 patients (80% positivity rate). Targeted exome sequencing of eight genes identified 24 variants in these patients: 11 DUOX2 variants in eight patients; six TSHR variants in five patients; five TG variants in three patients; and two DUOXA2 variants in two patients. Of these 24 variants, 10 (41.6%) were novel. No variants were identified in TPO, IYD, SLC5A5, or SLC26A4. Two patients displayed triallelic (digenic) mutations (in TG and TSHR in one patient and DUOX2 and TSHR in the other). No variants were identified in three patients with permanent hypothyroidism and one patient with transient hypothyroidism. Genetic variations that could explain the congenital hypothyroidism phenotypes were identified in 12/15 cases (80%). CONCLUSIONS: Targeted exome sequencing identified the genetic causes of congenital hypothyroidism with thyroid gland in situ in 80% of the patients studied, with DUOX2 and TSHR mutations being the most common. As many of the identified variants were novel, additional studies on the genetic causes of congenital hypothyroidism are warranted.

Association between vitamin D deficiency at one month of age and bronchopulmonary dysplasia.

ABSTRACT: Vitamin D deficiency is common and increases the likelihood of neonatal morbidities in preterm infants. This study assessed vitamin D levels at 1 month of age after 4 weeks of vitamin D supplementation and determined the association between vitamin D levels and neonatal morbidities.This retrospective study included preterm infants with birth weight <1500 g or gestational age <32 weeks born in our hospital between January 2018 and December 2019. They were administered 400 IU of oral vitamin D supplementation after birth according to our policy. The infants were then divided into sufficient (≥20 ng/mL) and deficient (<20 ng/mL) groups according to their serum vitamin D levels at 1 month of age.The vitamin D deficient and sufficient groups included 49 and 41 patients, respectively. The mean gestational age and birth weight. GHT in the vitamin D deficient group were 29.1 ±â€Š2.1 weeks and 1216.1 ±â€Š308.1 g, respectively, and 30.0 ±â€Š1.7 weeks and 1387.6 ±â€Š350.8 g, respectively, in the sufficient group. No significant differences were observed between the 2 groups in demographic and clinical outcomes except for bronchopulmonary dysplasia (BPD), which occurred significantly more often in the vitamin D-deficient group (odds ratio 2.21; 95% confidence interval, 1.85-2.78; P = .02).The results of our study suggest that vitamin D deficiency at 1 month of age is associated with BPD in preterm infants.

Extracerebral choroid plexus papilloma in the pharynx with airway obstruction in a newborn: a case report.

BACKGROUND: Choroid plexus papillomas (CPPs) are rare, usually benign, neoplasms originating in the central nervous system. In this study, we present the first case of a giant airway-obstructing CPP in the pharynx of a newborn. CASE PRESENTATION: A cystic mass located in the pharynx was noted in a fetus at the 29th week of gestation. Elective cesarean section was performed at the 38th week of gestation with successful intubation and ex utero intrapartum treatment. On computed tomography, there was a huge airway-obstructing cystic mass in the choana and pharynx. Elective surgery with total excision was performed, and histological examination confirmed the diagnosis of CPP. CONCLUSION: We report the first case of an extracerebral airway-obstructing CPP in the pharynx of a newborn. Radiologic examinations are not enough for the diagnosis of CPPs, and complete excision of the tumor with histological confirmation is indispensable for accurate diagnosis and treatment.

Predictive value of an early amplitude-integrated electroencephalogram for short-term neurologic outcomes in preterm infants.

BACKGROUND AND OBJECTIVES: The aim of this study was to compare serial scores of amplitude-integrated electroencephalography (aEEG) in preterm infants with favorable neurologic outcome compared with those with unfavorable neurologic outcome and to evaluate whether aEEG in the early days of life has predictive value for short-term neurologic outcome in preterm infants. METHODS: This prospective observational study included infants born at ≤32 weeks of gestational age and ≤1,500 g of birth weight. On the basis of brain ultrasonography findings, the infants were divided into two groups (favorable and unfavorable outcome group) at 36 weeks of corrected age or at discharge. aEEG was performed at 12-14 h (day-1), 46-48 h (day-2), 70-72 h (day-3), and 1 week (day-7) of life. The aEEG recordings were analyzed using the criteria described by Burdjalov et al.23 and the serial scores of aEEG were compared between the two groups. RESULTS: Thirty five infants were enrolled and 18 infants and 17 infants were identified into both groups, respectively. Infants in the favorable outcome group showed high scores in almost all parameters and the score of all parameters increased over time. However, the scores of all components decreased in day-2 compared with those of day 1 in the unfavorable outcome group. The total score less than 3 of day-2 has predictive value of 70.6% of sensitivity and 72.2% of specificity for unfavorable outcome. CONCLUSION: We found that aEEG is a useful predictor for short-term neurologic outcome in preterm infants.

Identification of a novel variant in the PHEX gene using targeted gene panel sequencing in a 24-month-old boy with hypophosphatemic rickets.

Familial hypophosphatemic rickets (FHR) is a disorder characterized by phosphate wasting and hypophosphatemia due to defects in renal phosphate transport regulation. There are 4 known inherited forms of FHR that differ in their molecular causes. Very few studies have been conducted that focused on the molecular analysis of FHR in Koreans. Eighteen mutations of the PHEX gene have been identified to this date in Korea. Herein, we report the clinical case of a 24-month-old boy presenting with bowed legs and short stature. The biochemical profile showed hypophosphatemia with decreased tubular reabsorption of phosphate. Several family members were identified with short stature and genu varum. Therefore, he was diagnosed with FHR. To identify the molecular causes of FHR, we performed targeted gene panel sequencing and found a novel hemizygous missense variant, c.1949T>C (p.Leu650Pro), in the PHEX gene. This variant was also detected in the boy's mother who exhibited genu varum and short stature.

Meconium peritonitis resulting from different etiologies in siblings: a case report.

BACKGROUND: Meconium peritonitis is defined as aseptic chemical inflammation caused by intrauterine bowel perforation. The underlying causes of bowel perforation include intestinal atresia, midgut volvulus, intussusception, congenital bands, and meconium ileus. CASE PRESENTATION: Siblings with prenatally diagnosed meconium peritonitis of different etiologies were found. The elder sister was born at 36 + 6 weeks gestation with a birth weight of 3110 g. She was diagnosed with meconium peritonitis caused by ileal atresia. Two years later, the younger brother was born at 34 + 3 weeks gestation with a birth weight of 2850 g. He was diagnosed with meconium peritonitis caused by midgut volvulus. CONCLUSIONS: Among the previously reported cases of meconium peritonitis, familial occurance of meconium peritonitis is extremely rare. We present a case of prenatally diagnosed meconium peritonitis in siblings to promote further understanding of its etiology and clinical course.

An A627V-activating mutation in the thyroid-stimulating hormone receptor gene in familial nonautoimmune hyperthyroidism.

Nonautoimmune hyperthyroidism is a very rare cause of congenital hyperthyroidism that is usually caused by an activating mutation in the thyroid-stimulating hormone receptor (TSHR) gene. In this report, we describe a case of nonautoimmune hyperthyroidism in a patient with TSHR mutation. Our patient was the younger of a set of twins born at 36 weeks and 6 days of gestation. The patient was noted to be more irritable than the older twin at 80 days of age, and the mother was taking methimazole for Graves' disease that had been diagnosed 12 years prior. Therefore, a thyroid function test was conducted for the patient. The results revealed subclinical hyperthyroidism, and tests of antithyroglobulin antibody, antithyroid peroxidase antibody, and anti-thyroid-stimulating hormone (TSH) receptor antibody were all negative. During follow-up, at around 4 months of age, free T4 increased to 2.89 ng/dL, and TSH was still low at 0.01 µIU/mL; therefore, 3 mg/day of methimazole was initiated. Whole-exome sequencing showed a heterozygous variant of c.1800C>T (p.Ala627Val) in the TSHR gene. Testing in the family confirmed an identical variant in the patient's mother, leading to diagnosis of familial nonautoimmune hyperthyroidism inherited in an autosomal dominant pattern. This is the second report of A627V confirmed as a germline variant.

Utility of Magnetic Resonance Imaging (MRI) in Children With Strabismus.

PURPOSE: Magnetic resonance imaging (MRI) of the brain can provide valuable information about structural abnormalities in strabismus. The aim of this study was to evaluate the utility of MRI in this regard and to identify risk factors for abnormal MRI results in children with strabismus. METHODS: A retrospective analysis of children <18 years of age presenting with strabismus, who underwent brain MRI at Pusan National University Hospital (Busan, Korea) between January 2012 and March 2017, was performed. Clinical characteristics, MRI results, and ophthalmologic findings were reviewed. Findings were classified as normal or abnormal according to MRI results. Additionally, patients were divided according to age to compare characteristics of infantile and childhood strabismus. RESULTS: A total of 90 patients (47 [52.2%] male, 43 [47.8%] female; mean age, 2.19 ± 0.53 years) were enrolled. Of those, 64 (71.1%) presented with normal and 26 (28.9%) with abnormal MRI results. The age at presentation was lower and abnormal findings on fundus examination were more common in the abnormal MRI group (P = .002 and P = .008, respectively). Among the patients, 46 (51.1%) had infantile strabismus and 44 (48.9%) had childhood strabismus. Global developmental delays, speech delays, and MRI abnormalities were more common in patients with infantile than in those with childhood strabismus. Ptosis and headaches were more common in patients with childhood strabismus (P = .025, P = .025, respectively). CONCLUSION: Brain MRI was helpful for accurate diagnosis and treatment of strabismus in younger children, those with abnormal findings on fundus examination, and infants with developmental, especially speech, delays.

Clinical characterization of anti-GQ1b antibody syndrome in Korean children.

Anti-GQ1b antibody syndrome encompasses Miller Fisher syndrome and its related disorders. We retrospectively identified 11 pediatric patients (5.4-18 years old) with anti-GQ1b antibody syndrome. Diagnoses of patients included acute ophthalmoparesis (n = 6), classical Miller Fisher syndrome (n = 2), Miller Fisher syndrome/Guillain-Barré syndrome (n = 1), acute ataxic neuropathy (n = 1), and pharyngeal-cervical-brachial weakness (n = 1). Nine patients (81.8%) fully recovered. Maturational change in GQ1b antigen expression and the accessibility of anti-GQ1b antibodies might be the cause of the difference of clinical manifestations in children with anti-GQ1b antibody syndrome.

Comparison of conservative therapy and steroid therapy for Bell's palsy in children.

PURPOSE: Bell's palsy is characterized by sudden onset of unilateral facial weakness. The use of corticosteroids for childhood Bell's palsy is controversial. This study aimed to identify clinical characteristics, etiology, and laboratory findings in childhood Bell's palsy, and to evaluate the efficacy of corticosteroid treatment. METHODS: We conducted a retrospective analysis of children under 19 years of age treated for Bell's palsy between January 2009 and June 2017, and followed up for over 1 month. Clinical characteristics, neuroimaging data, laboratory findings, treatments, and outcomes were reviewed. Patients with Bell's palsy were divided into groups with (group 1) and without (group 2) corticosteroid treatment. Differences in onset age, sex, laterality, infection and vaccination history, degree of facial nerve palsy, and prognosis after treatment between the groups were analyzed. RESULTS: One hundred patients were included. Mean age at presentation was 7.4±5.62 years. A total of 73 patients (73%) received corticosteroids with or without intravenous antiviral agents, and 27 (27%) received only supportive treatment. There was no significant difference in the severity, laboratory findings, or neuroimaging findings between the groups. Significant improvement was observed in 68 (93.2%) and 26 patients (96.3%) in groups 1 and 2, respectively; this rate was not significantly different between the groups (P=0.48). CONCLUSION: Childhood Bell's palsy showed good prognosis with or without corticosteroid treatment; there was no difference in prognosis between treated and untreated groups. Steroid therapy in childhood Bell's palsy may not significantly improve outcomes.

Chylous Ascites in an Infant with Thanatophoric Dysplasia Type I with FGFR3 Mutation Surviving Five Months.

BACKGROUND: Thanatophoric dysplasia (TD) results from sporadic de novo mutations in the FGFR3 gene. Upon confirming intrauterine diagnosis of this perinatal disease, pregnancy termination is recommended. There is limited information on the natural history of longer-term survivors with type 1 TD. CASE REPORT: A full-term neonate was confirmed via postnatal genetic testing to have type 1 TD. At 28 days, chylous ascites developed. Medium-chain triglyceride use improved the ascites. Cerebral ventriculomegaly worsened throughout life. Death due to respiratory failure occurred at age 5 months. CONCLUSION: The chylous ascites in this child with type 1 TD and survival past the neonatal stage suggests that type 1 TD may be accompanied by abnormalities of the lymphatic channels. Moreover, ventriculomegaly can be progressive.

The clinical characteristics and neurodevelopmental outcome of preterm infants with persistent periventricular echogenicity.

PURPOSE: Periventricular echogenicity (PVE) presents as diffuse echo dense lesions of the periventricular white matter on cranial ultrasonography. Beyond two weeks of life, it is considered as prolonged or persistent PVE. The aim of our study was to investigate the clinical characteristics of preterm infants with persistent PVE beyond 2 weeks after birth and to determine whether these infants had an adverse neurodevelopmental outcome. METHODS: The medical records of preterm infants who were born at < 34 weeks of gestation and admitted to Pusan National University Hospital between 2009 and 2014 were reviewed. A total of 28 preterm infants with persistent PVE were enrolled. Sixty compatible infants closely matched for gestational age and birth weight to infants with PVE were selected as the control group. Clinical data, including maternal, perinatal and neonatal characteristics, were analyzed. We compared the Bayley Scales of Infant Development-III at 12 months' corrected age. RESULTS: The mean gestational age and birth weight were 31 + 3 (range, 29 + 2-33 + 6) weeks and 1523 (range, 911-2210) g, respectively, in the persistent PVE group. In the control group, the mean gestational age was 31 + 4 (range, 29 + 2-33 + 6) weeks and the mean birth weight was 1537 (range, 840-2100) g. There was no significant difference between the persistent PVE group and the control group, except for a significantly higher incidence of late sepsis in the persistent PVE group (p = 0.001). The results of Bayley test at 12 months of corrected age were available for 24 infants in the persistent PVE group and for 26 infants in the control group. A motor score of 86 (range, 78-95) versus 88 (range, 79-100), a language composite score of 88 (range, 78-97) versus 89 (range, 80-105), and a cognitive score of 90 (range, 81-100) versus 92 (range, 85-105) were observed in the persistent PVE group and the control group, respectively. No difference was detected in any scores between the two groups. CONCLUSION: The clinical characteristics and neurodevelopmental outcomes of preterm infants with persistent PVE were not different from those of infants with normal findings. Our study supports the concept that persistent PVE without cystic change may be a benign finding.

Extended Red Blood Cell Genotyping to Investigate Immunohematology Problems

No abstract available.

Endovascular stenting of tracheoinnominate fistula after tracheostomy in a 14-year-old boy.

Tracheoinnominate artery fistula is a rare, fatal complication of tracheostomy, and prompt diagnosis and management are imperative. We report the case of tracheoinnominate artery fistula after tracheostomy in a 14-year-old boy with a history of severe periventricular leukomalacia, hydrocephalus, cerebral palsy, and epilepsy. The tracheoinnominate artery fistula was successfully treated with a stent graft insertion via the right common femoral artery. Endovascular repair of the tracheoinnominate artery fistula via stent grafting is a safe, effective, and minimally invasive treatment for patients in poor clinical conditions and is an alternative to traditional open surgical treatment.

Establishing reference values for amplitude-integrated electroencephalography in preterms below 35 weeks of gestational age: A prospective observational cohort study.

Characteristic patterns in amplitude-integrated electroencephalography (aEEG) develop with gestational age (GA), and so can be used to evaluate brain maturation in premature infants. Reference aEEG values in normal preterm infants have not been identified and data are scarce. We aimed to validate a currently available aEEG scoring system. We also investigated the development of aEEG activity during the first week after birth, determining reference values in preterm infants with no abnormal cranial ultrasound findings. We prospectively studied aEEG and cranial ultrasounds in infants with a GA of < 35 weeks. We conducted aEEG at 12-14 hours, 46-48 hours, 70-72 hours, and 1 week after birth. The aEEG recordings were evaluated using Burdjalov criteria, scored by two independent neonatologists. Thirty-four infants were enrolled and completed the 1-week evaluation. GA ranged from 24 to 35 weeks and birth weights varied between 570 and 2,100 g. We analyzed 134 aEEG tracings, with a mean difference between raters of -0.05. Total scores, summed from scores for each assessed variable, increased gradually with advancing gestational and postnatal age. However, highest scores were not attained until 35 weeks' gestational age. There was high inter-rater agreement for aEEG scoring, and we could ascertain some approximate reference values for aEEG development in preterm infants at varying GA. To establish standardized aEEG reference criteria, further studies in larger cohorts of premature infants should be performed over longer periods.