Audiological Follow-up Results after Newborn Hearing Screening Program

ObjectivesTo investigate the validity of newborn hearing screening protocol using automated auditory brainstem response (AABR) with a confirmation method using click auditory brainstem response (ABR) and to evaluate changes in hearing status of infants with confirmed congenital hearing loss.MethodsNeonates in the well-baby nursery were screened by staged AABR. Subjects whose final AABR result was "refer" were tested by diagnostic click ABR and 226 Hz tympanometry within 3 months of age. Changes in hearing status of subjects with confirmed hearing loss were analyzed by follow-up ABR at 3-6 month intervals.ResultsOf the 12,193 healthy babies born during this period, 10,879 (89.22%) were screened by AABR. Of 10,879 neonates screened by AABR, 148 (1.36%) were "referred"; of these, 45 subjects showed ABR thresholds over 30 dB nHL in at least one ear. Thirty-four subjects underwent serial follow-up ABR tests, with 11 (32.4%) found to have normal ABR thresholds. Most subjects with mild to moderate hearing loss were found to be normal before 1 year of age, whereas all infants with severe or profound hearing loss were identified as having congenital hearing loss.ConclusionThe referral rate and the positive predictive value of our protocol were acceptable. We have also found here that substantial temporary hearing loss can be included in the first confirmative diagnosis. Temporary hearing loss of our study on follow-up give emphasis to need of further differentiation using the testing for bone conduction and middle ear status

Validity of the Korean Developmental Screening Test for very-low-birth-weight infants

Purpose The importance of the neurodevelopmental outcomes of very-low-birth-weight (VLBW) infants has been emphasized as their mortality rate has markedly improved. This study aimed to assess the validity of the Korean Developmental Screening Test (K-DST), a developmental screening tool approved by the Korean Society of Pediatrics, for the timely diagnosis of neurodevelopmental delay in VLBW infants. Methods Subjects included VLBW infants enrolled in the Korean Neonatal Network database between January 2012 and December 2014. The collected data were analyzed for sensitivity, specificity, positive predictive value, and negative predictive value (NPV) in the K-DST compared to those in the Bayley Scales of Infant Development-II for VLBW infants. Results A total of 173 patients were enrolled. Their mean gestational age and mean birth weight were 27.5±2.8 weeks and 980.5±272.1 g, respectively. The frequency of failed psychomotor developmental index (PDI) <85 was similar to that in at least one domain of K-DST <1 standard deviation. Failure in more than one K-DST domain compared with a mental developmental index (MDI) <85 showed a sensitivity and NPV of 73.2% and 75.0%, respectively. Failure in more than one K-DST domain compared with PDI <85 showed a sensitivity and NPV of 60.3% and 71.6%, respectively. Each K-DST domain had a stronger correlation with predicting a failing MDI <85 than a failing PDI <85 (P<0.05). Conclusion K-DST could be a useful screening tool for predicting mental developmental delay in VLBW infants and referring them for neurodevelopmental assessments

Scoring Method for Early Prediction of Neonatal Chronic Lung Disease Using Modified Respiratory Parameters

In our previous study, we have demonstrated that peak inspiratory pressure over birth weight (PIP/kg) and mean airway pressure over birth weight (MAP/kg) were more significant risk factors for the development of neonatal chronic lung disease (CLD) than PIP and MAP. We aimed to develop a scoring method using the modified respiratory variables (SMUMRV) to predict CLD at early postnatal period. From 1997 to 1999, a retrospective review was performed for 197 infants <1,500 g for the development of the SMUMRV based on statistical analysis. From 2000 to 2001, calculated scores on day 4, 7 and 10 of life were obtained prospectively for 107 infants <1,500 g. Predictive values and the area under the receiver operator characteristic curve (AUC) were determined and compared with the result of the previous regression model. Gestational age, birth weight, 5 min Apgar score, PIP/kg at 12 hr of age, fractional inspired oxygen (FiO2), MAP/kg, modified oxygenation index and ventilatory mode were selected as parameters of SMUMRV. No significant differences of AUCs were found between the SMUMRV and the Yoder model. It is likely that our scoring method provides reliable values for predicting the development of CLD in very low birth weight infants

A Case of Campomelic Dysplasia without Sex Reversal

Campomelic dysplasia (CD; OMIM #114290), a rare form of congenital short-limbed dwarfism, is due to mutations in SOX9, a member of the SOX (SRY-related HMG box) gene family. Multiparous mother at 38 weeks' gestation delivered a 3,272 g baby boy with characteristic phenotypes including bowing of the lower limbs, a narrow thoracic cage, 11 pairs of ribs, hypoplastic scapulae, macrocephaly, flattened supraorbital ridges and nasal bridge, cleft palate, and micrognathia. He underwent a tracheostomy at the age of three months for severe laryngomalacia after a number of repeated hospitalizations due to respiratory problems and died at the age of four months from progressive respiratory failure. He was diagnosed as having CD based on a novel frameshift mutation (p.Gln458ArgfsX12) in the SOX9 gene, the mutation which has not yet been reported in Korea

Longitudinal Study on Trace Mineral Compositions (Selenium, Zinc, Copper, Manganese) in Korean Human Preterm Milk

We measured selenium, zinc, copper and manganese concentrations in the human milk of Korean mothers who gave birth to preterm infants, and compared these measurements with the recommended daily intakes. The samples of human milk were collected postpartum at week-1, -2, -4, -6, -8, and -12, from 67 mothers who gave birth to preterm infants (< 34 weeks, or birth weight < 1.8 kg). All samples were analyzed using atomic absorption spectrophotometry. The concentrations of selenium were 11.8 ± 0.5, 11.4 ± 0.8, 12.7 ± 0.9, 11.4 ± 0.8, 10.8 ± 0.9, and 10.5 ± 1.3 µg/L, zinc were 7.8 ± 0.5, 9.1 ± 0.8, 7.2 ± 0.9, 8.0 ± 0.8, 7.4 ± 0.9, and 6.6 ± 1.2 mg/L, copper were 506 ± 23.6, 489 ± 29.4, 384 ± 33.6, 356 ± 32.9, 303 ± 35.0, and 301 ± 48.0 µg/L and manganese were 133 ± 4.0, 127 ± 6.0, 125 ± 6.0, 123 ± 6.0, 127 ± 6.0, and 108 ± 9.0 µg/L at week-1, -2, -4, -6, -8, and -12, respectively. The concentrations of selenium and zinc meet the daily requirements but that of copper is low and of manganese exceeds daily requirements recommended by the American Academy of Pediatrics, Committee on Nutrition

Prevention of human milk-acquired cytomegalovirus infection in very-low-birth-weight infants

Abstract Background The aim of the study was to determine the rate of cytomegalovirus virolactia in the human milk (HM) of mothers of VLBW infants, compare the CMV infection rates and the changes in CMV DNA viral load and nutrient profile among different HM preparation methods. Methods A prospective randomized controlled study was performed in infants with gestational age < 32 weeks or birth-weight < 1500 g admitted to neonatal intensive care unit of Asan Medical Center and Haeundae Paik Hospital who were given mother’s own milk. Enrolled infants were randomized into three groups according to the HM preparation methods: freezing-thawing (FT), FT + low-temperature Holder pasteurization (FT + LP), and FT + high-temperature short-term pasteurization (FT + HP). Urine CMV culture and PCR were obtained at birth and at 4, 8, and 12 weeks. HM CMV culture and PCR were obtained at birth and at 3, 6, 9, and 12 weeks. Changes in macronutrients in HM was obtained at 4 ~ 6 weeks. Results Of 564 infants, 217 mothers (38.5%) produced CMV PCR positive milk. After exclusion, a total of 125 infants were randomized into the FT (n = 41), FT + LP (n = 42), and FT + HP (n = 42) groups, whose rate of HM-acquired CMV infection was 4.9% (n = 2), 9.5% (n = 4), and 2.4% (n = 1), respectively. Out of seven CMV infected infants, two infants fed with FT + LP HM developed CMV infection- associated symptoms. Ages at diagnoses were earlier (28.5 days after birth) and at younger post conceptional age (< 32 weeks) in comparison to infants with asymptomatic CMV infection. CMV DNA viral load significantly decreased after pasturizations, especially in FT + HP group. Conclusions HM-acquired symptomatic CMV infection rate is low and its impact on clinical course was not serious in our VLBW infants. However, evidences showing poor neurodevelopmental outcome in later life, we need to generate a guideline to protect VLBW infant form HM transmitted CMV infection. Based on our small sized study, we did not find any superiority in pasteurizing HM with frequently used LP in comparison to frozen or HP HM. More research is needed to determine the method and duration of pasteurization to reduce the HM-acquired CMV infection