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PURPOSE: Newborn screening (NBS) programs are important for appropriate management of susceptible neonates to prevent serious clinical problems. Neonates admitted to neonatal intensive care units (NICU) are at a potentially high risk of false-positive results, and repetitive NBS after total parenteral nutrition is completely off results in delayed diagnosis. Here, we present the usefulness of a targeted next-generation sequencing (TNGS) panel to complement NBS for early diagnosis in high-risk neonates. MATERIALS AND METHODS: The TNGS panel covered 198 genes associated with actionable genetic and metabolic diseases that are typically included in NBS programs in Korea using tandem mass spectrometry. The panel was applied to 48 infants admitted to the NICU of Severance Children's Hospital between May 2017 and September 2017. The infants were not selected for suspected metabolic disorders. RESULTS: A total of 13 variants classified as likely pathogenic or pathogenic were detected in 11 (22.9%) neonates, including six genes (DHCR7, PCBD1, GAA, ALDOB, ATP7B, and GBA) associated with metabolic diseases not covered in NBS. One of the 48 infants was diagnosed with an isobutyl-CoA dehydrogenase deficiency, and false positive results of tandem mass screening were confirmed in two infants using the TNGS panel. CONCLUSION: The implementation of TNGS in conjunction with conventional NBS can allow for better management of and earlier diagnosis in susceptible infants, thus preventing the development of critical conditions in these sick infants.
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Humanos , Lactante , Recién Nacido , Proteínas del Sistema Complemento , Diagnóstico Tardío , Diagnóstico , Diagnóstico Precoz , Unidades de Cuidado Intensivo Neonatal , Corea (Geográfico) , Tamizaje Masivo , Enfermedades Metabólicas , Errores Innatos del Metabolismo , Oxidorreductasas , Nutrición Parenteral Total , Espectrometría de Masas en TándemRESUMEN
PURPOSE: Congenital pulmonary airway malformation (CPAM)—a rare developmental anomaly—affects the lower respiratory tract in newborns. By comparing the reliability of diagnostic tools and identifying predictive factors for symptoms, we provide comprehensive clinical data for the proper management of CPAM. METHODS: We reviewed the medical records of 66 patients with prenatally diagnosed CPAM delivered at Severance Children's Hospital between January 2005 and July 2017. RESULTS: We enrolled 33 boys and 33 girls. Their mean gestational age and birth weight were 38.8 weeks and 3,050 g, respectively. Prenatal ultrasonography and postnatal radiography, lung ultrasonography, and chest computed tomography (CT) showed inconsistent findings. Chest CT showed superior sensitivity (100%) and positive predictive value (90%). Among the 66 patients, 59 had postnatally confirmed CPAM, three had pulmonary sequestration, one had cystic teratoma, and one had a normal lung. Of the 59 patients with CPAM, 21 (35%; mean age, 23.4 months) underwent surgery, including 15 who underwent video-assisted thoracoscopy. Twenty-five and 12 patients exhibited respiratory symptoms at birth and during infancy, respectively. Apgar scores and mediastinal shift on radiography were significantly associated with respiratory symptoms at birth. However, none of the factors could predict respiratory symptoms during infancy. CONCLUSION: Radiography or ultrasonography combined with chest CT can confirm an unclear or inconsistent lesion. Apgar scores and mediastinal shift on radiography can predict respiratory symptoms at birth. However, symptoms during infancy are not associated with prenatal and postnatal factors. Chest CT combined with periodic symptom monitoring is important for diagnosing and managing patients with prenatally diagnosed CPAM and to guide appropriate timing of surgery.
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Femenino , Humanos , Recién Nacido , Peso al Nacer , Secuestro Broncopulmonar , Malformación Adenomatoide Quística Congénita del Pulmón , Edad Gestacional , Pulmón , Registros Médicos , Parto , Radiografía , Sistema Respiratorio , Teratoma , Cirugía Torácica Asistida por Video , Toracoscopía , Tórax , Tomografía Computarizada por Rayos X , Ultrasonografía , Ultrasonografía PrenatalRESUMEN
Genitopatellar syndrome (GPS) is a rare disorder characterized by patellar hypoplasia, flexion contractures of the lower limbs, psychomotor retardation and genital and renal anomalies. We report the case of a female infant diagnosed with GPS to a KAT6B gene mutation, which was identified using whole exome sequencing.
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Femenino , Humanos , Lactante , Contractura , Exoma , Corea (Geográfico) , Extremidad InferiorRESUMEN
Pylorospasm is a cause of delayed gastric emptying in young infants. As in patients with hypertrophic pyloric stenosis, most pylorospasm patients present with projectile vomiting. However, unlike that in case of hypertrophic pyloric stenosis, no persistent pyloric stenotic lesions are present. As such, follow-up using serial gastrointestinal fluoroscopy or ultrasonography can be helpful in diagnosing patients with clinical signs of gastroparesis. Most cases can be treated conservatively, but some patients require pharmacologic treatment. Antispasmodics have been proposed as a treatment for pylorospasm, but their use in neonates and infants has rarely been reported. Herein, we present a case of pylorospasm diagnosed in the neonatal period and successfully treated with intravenous atropine.
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Humanos , Lactante , Recién Nacido , Atropina , Fluoroscopía , Estudios de Seguimiento , Vaciamiento Gástrico , Gastroparesia , Parasimpatolíticos , Estenosis Hipertrófica del Piloro , Píloro , Espasmo , Ultrasonografía , VómitosRESUMEN
PURPOSE: Hypothermia at admission is associated with increased mortality and morbidity in preterm infants. We performed a quality improvement (QI) effort to determine the impact of a decrease in admission hypothermia in preterm infants. METHODS: The study enrolled very low birth weight (VLBW) infants born at Gangnam Severance Hospital between January 2013 and December 2016. This multidisciplinary QI effort included the use of occlusive wraps, warm blankets, and caps; the delivery room temperature was maintained above 23.0℃, and a check-list was used for feedback. RESULTS: Among 259 preterm infants, the incidence of hypothermia (defined as body temperature <36.0℃) decreased significantly from 68% to 41%, and the mean body temperature on neonatal intensive care unit admission increased significantly from 35.5℃ to 36.0℃. In subgroup analysis of VLBW infants, admission hypothermia and neonatal outcomes were compared between the pre-QI (n=55) and post-QI groups (n=75). Body temperature on admission increased significantly from 35.4℃ to 35.9℃ and the number of infants with hypothermia decreased significantly from 71% to 45%. There were no cases of neonatal hyperthermia. The incidence of pulmonary hemorrhage was significantly decreased (P=0.017). Interaction analysis showed that birth weight and gestational age were not correlated with hypothermia following implementation of the protocol. CONCLUSION: Our study demonstrated a significant reduction in admission hypothermia following the introduction of a standardized protocol in our QI effort. This resulted in an effective reduction in the incidence of massive pulmonary hemorrhage.
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Humanos , Lactante , Recién Nacido , Peso al Nacer , Temperatura Corporal , Salas de Parto , Fiebre , Edad Gestacional , Hemorragia , Hipotermia , Incidencia , Recien Nacido Prematuro , Recién Nacido de muy Bajo Peso , Cuidado Intensivo Neonatal , Mortalidad , Qi , Mejoramiento de la CalidadRESUMEN
PURPOSE: Removal of CO₂ is much efficient during high-frequency oscillatory ventilation (HFOV) for preterm infants. However, an optimal carbon dioxide diffusion coefficient (DCO₂) and tidal volume (VT) have not yet been established due to much individual variance. This study aimed to analyze DCO₂ values, VT, and minute volume in very-low-birth-weight (VLBW) infants using HFOV and correlates with plasma CO₂ (pCO₂). MATERIALS AND METHODS: Daily respiratory mechanics and ventilator settings from twenty VLBW infants and their two hundred seventeen results of blood gas analysis were collected. Patients were treated with the Dräger Babylog VN500 ventilator (Drägerwerk Ag & Co.) in HFOV mode. The normocapnia was indicated as pCO₂ ranging from 45 mm Hg to 55 mm Hg. RESULTS: The measured VT was 1.7 mL/kg, minute volume was 0.7 mL/kg, and DCO₂ was 43.5 mL²/s. Mean results of the blood gas test were as follows: pH, 7.31; pCO₂, 52.6 mm Hg; and SpO₂, 90.5%. In normocapnic state, the mean VT was significantly higher than in hypercapnic state (2.1±0.5 mL/kg vs. 1.6±0.3 mL/kg), and the mean DCO₂ showed significant difference (68.4±32.7 mL²/s vs. 32.4±15.7 mL²/s). The DCO₂ was significantly correlated with the pCO₂ (p=0.024). In the receiver operating curve analysis, the estimated optimal cut-off point to predict the remaining normocapnic status was a VT of 1.75 mL/kg (sensitivity 73%, specificity 80%). CONCLUSION: In VLBW infants treated with HFOV, VT of 1.75 mL/kg is recommended for maintaining proper ventilation.
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Femenino , Humanos , Lactante , Recién Nacido , Masculino , Análisis de los Gases de la Sangre , Dióxido de Carbono/análisis , Ventilación de Alta Frecuencia , Hipercapnia/fisiopatología , Incidencia , Recién Nacido de muy Bajo Peso/fisiología , Curva ROC , Volumen de Ventilación PulmonarRESUMEN
PURPOSE: The aims of this study were to evaluate the safety and pharmacokinetics of levetiracetam (LEV) in neonates with seizures and to establish a population pharmacokinetics (PPK) model by using the software NONMEM. METHODS: A retrospective analysis of 18 neonatal patients with seizures, who were treated with LEV, including 151 serum samples, was performed. The mean loading dose was 20 mg/kg, followed by a mean maintenance dose of 29 mg/kg/day. RESULTS: Seventeen neonates (94%) had seizure cessation within 1 week and 16 (84%) remained seizure-free at 30 days under the LEV therapy. The mean serum concentration of LEV was 8.7 µg/mL. Eight samples (5%) were found above the therapeutic range. No serious adverse effects were detected. In the PPK analysis for Korean neonates, the half-life was 9.6 hours; clearance, 0.357 L/hr; and volume of distribution, 4.947 L, showing differences from those in adults. CONCLUSION: LEV is a safe and effective option for the treatment of neonatal seizures with careful therapeutic drug monitoring.
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Adulto , Humanos , Recién Nacido , Monitoreo de Drogas , Semivida , Farmacocinética , Estudios Retrospectivos , ConvulsionesRESUMEN
PURPOSE: The goal of nutritional support for very-low-birth-weight (VLBW) infants from birth to term is to match the in utero growth rates; however, this is rarely achieved. METHODS: We evaluated postdischarge growth patterns and growth failure in 81 Korean VLBW infants through a retrospective study. Weight and height were measured and calculated based on age percentile distribution every 3 months until age 24 months. Growth failure was defined as weight and height below the 10th percentile at 24 months. For the subgroup analysis, small-for-gestational age (SGA) and extremely low birth weight (ELBW) infants were evaluated. The growth patterns based on the Korean, World Health Organization (WHO), or Centers for Disease Control and Prevention (CDC) standard were serially compared over time. RESULTS: At postconception age (PCA) 40 weeks, 47 (58%) and 45 infants (55%) showed growth failure in terms of weight and height, respectively. At PCA 24 months, 20 infants (24%) showed growth failure for weight and 14 (18%) for height. Growth failure rates were higher for the SGA infants than for the appropriate-weight-for-gestational age infants at PCA 24 months (P=0.045 for weight and P=0.038 for height). Growth failure rates were higher for the ELBW infants than for the non-ELBW infants at PCA 24 months (P<0.001 for weight and P=0.003 for height). Significant differences were found among the WHO, CDC, and Korean standards (P<0.001). CONCLUSION: Advancements in neonatal care have improved the catch-up growth of VLBW infants, but this is insufficient. Careful observation and aggressive interventions, especially in SGA and ELBW infants, are needed.
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Humanos , Lactante , Recién Nacido , Edad Gestacional , Recien Nacido con Peso al Nacer Extremadamente Bajo , Recién Nacido de Bajo Peso , Recien Nacido Prematuro , Recién Nacido de muy Bajo Peso , Apoyo Nutricional , Parto , Anafilaxis Cutánea Pasiva , Estudios Retrospectivos , Organización Mundial de la SaludRESUMEN
PURPOSE: Caffeine shows wide interindividual pharmacokinetic (PK) variation, and therapeutic drug monitoring (TDM) may be needed. The PK profile of caffeine in Korean preterm neonates was investigated, and factors influencing the clearance of caffeine were analyzed. METHODS: Fifty-nine preterm neonates receiving caffeine for apnea of prematurity were enrolled in the study (gestational age, 29.5±2.2 weeks and birth weight [BW], 1,318±358 g). Caffeine (20 mg/kg) was intravenously administered to each neonate as a loading dose, followed by a maintenance dose of 5-10 mg/kg/d. A total of 190 serum concentrations were measured for population PK analysis and modeling using nonlinear mixed-effects model (NONMEM®) software. RESULTS: The mean serum concentration of caffeine was 15.4±4.5 mg/L (range 7.8-33.0 mg/L). High serum concentrations (>20 mg/L) were noted in 36 samples (29%). At the first measurement of serum caffeine, the mean postmenstrual age was 33.9±2.3 weeks, mean BW was 1,802±471 g, mean duration of treatment was 7.4±9.4 days, and mean sampling time after the last dose was 21.8±2.1 hours. In the population PK analysis, the clearance was 0.033 L/h and volume of distribution was 0.371 L. Typical clearance was calculated as 0.0293×(BW/70)1.33. Among the subjects receiving 5 mg/kg/d caffeine, the most significant risk factor associated with high serum concentrations (>20 mg/L) was low BW (P=0.024). CONCLUSION: BW was the only covariate that influenced caffeine clearance in preterm neonates. Preterm neonates with low BW should be carefully monitored for apnea and adverse reactions in addition to undergoing TDM.
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Humanos , Recién Nacido , Apnea , Peso al Nacer , Cafeína , Monitoreo de Drogas , Recien Nacido Prematuro , Farmacocinética , Factores de RiesgoRESUMEN
Protein C (PROC) deficiency is caused by mutations in the PROC gene on chromosome 2q14.3. Patients with PROC deficiency typically present distinguished purpura, intracerebral and intravascular coagulopathy, and ophthalmologic complications. Here, we report a rare severe form of PROC deficiency resulting from a compound heterozygosity in PROC. The patient was a 5-day-old female neonate born at 39 weeks of gestation with a birth weight of 2,960 g. She was transferred to our hospital with running a fever at 38.5℃ and with dark red patches on her feet. At admission, a complete blood count showed no specific findings, but levels of PROC and protein S were abnormally low (1% and 68%, respectively). Magnetic resonance imaging revealed intracerebral hemorrhaging and parenchymal damage with dysplasia of the brain. Ophthalmologic examination revealed vitreous hemorrhaging with retinal detachment. Genetic testing revealed a missense mutation (Arg211Trp) and a frameshift mutation (Gly239Serfs*8) in PROC, inherited from the father and mother, respectively. The patient recovered from purpura after undergoing ventriculoperitoneal shunting and treatment with fresh frozen plasma, warfarin sodium, and PROC concentrate. This is the first report of severe neonatal PROC deficiency with purpura fulminans, vitreous hemorrhage, and intracerebral hemorrhage confirmed via PROC genetic testing, which identified a rare compound heterozygosity of PROC.
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Femenino , Humanos , Recién Nacido , Embarazo , Peso al Nacer , Recuento de Células Sanguíneas , Encéfalo , Hemorragia Cerebral , Diagnóstico , Padre , Fiebre , Pie , Mutación del Sistema de Lectura , Pruebas Genéticas , Imagen por Resonancia Magnética , Madres , Mutación Missense , Plasma , Deficiencia de Proteína C , Proteína C , Proteína S , Púrpura , Púrpura Fulminante , Desprendimiento de Retina , Carrera , Derivación Ventriculoperitoneal , Hemorragia Vítrea , WarfarinaRESUMEN
The pulmonary interstitial emphysema (PIE) is a life-threatening illness in premature infants with mechanical ventilation. While most are managed conservatively, decompression would be necessary. Here, we report the first case of PIE treated by percutaneous catheter insertion in an extremely low birth weight (ELBW) infant in Korea. The patient, born with 660 g in 23+2 weeks of gestation, showed PIE in left lower lung on postnatal day 12. Percutaneous catheter insertion was performed on postnatal day 25. The size of PIE decreased, but didn't disappear completely. On postnatal day 42, we exchanged catheter and inserted additional catheter in pleural space. However, sudden desaturation and pneumothorax occurred on postnatal day 44. We changed catheter in pleural space, and pneumothorax and PIE improved. Finally, we successfully removed catheters, and weaned patient out. As in our case, percutaneous catheter insertion would be a useful option for ELBW infants with PIE.
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Humanos , Lactante , Recién Nacido , Embarazo , Catéteres , Catéteres de Permanencia , Descompresión , Enfisema , Recien Nacido con Peso al Nacer Extremadamente Bajo , Recién Nacido de Bajo Peso , Recien Nacido Prematuro , Corea (Geográfico) , Pulmón , Neumotórax , Enfisema Pulmonar , Respiración ArtificialRESUMEN
The pulmonary interstitial emphysema (PIE) is a life-threatening illness in premature infants with mechanical ventilation. While most are managed conservatively, decompression would be necessary. Here, we report the first case of PIE treated by percutaneous catheter insertion in an extremely low birth weight (ELBW) infant in Korea. The patient, born with 660 g in 23+2 weeks of gestation, showed PIE in left lower lung on postnatal day 12. Percutaneous catheter insertion was performed on postnatal day 25. The size of PIE decreased, but didn't disappear completely. On postnatal day 42, we exchanged catheter and inserted additional catheter in pleural space. However, sudden desaturation and pneumothorax occurred on postnatal day 44. We changed catheter in pleural space, and pneumothorax and PIE improved. Finally, we successfully removed catheters, and weaned patient out. As in our case, percutaneous catheter insertion would be a useful option for ELBW infants with PIE.
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Humanos , Lactante , Recién Nacido , Embarazo , Catéteres , Catéteres de Permanencia , Descompresión , Enfisema , Recien Nacido con Peso al Nacer Extremadamente Bajo , Recién Nacido de Bajo Peso , Recien Nacido Prematuro , Corea (Geográfico) , Pulmón , Neumotórax , Enfisema Pulmonar , Respiración ArtificialRESUMEN
Survival of very-low-birth-weight infants (VLBWI) depends on professional perinatal management that begins at delivery. Korean Neonatal Network data on neonatal resuscitation management and initial care of VLBWI of less than 33 weeks gestation born from January 2013 to June 2014 were reviewed to investigate the current practice of neonatal resuscitation in Korea. Antenatal data, perinatal data, and short-term morbidities were analyzed. Out of 2,132 neonates, 91.7% needed resuscitation at birth, chest compression was performed on only 104 infants (5.4%) and epinephrine was administered to 80 infants (4.1%). Infants who received cardiac compression and/or epinephrine administration at birth (DR-CPR) were significantly more acidotic (P or = grade 3 (OR, 2.71; 95% CI 1.57-4.68), periventricular leukomalacia (OR, 2.94; 95% CI 1.72-5.01), and necrotizing enterocolitis (OR, 2.12; 95% CI 1.15-3.91) compared with those infants who needed only PPV. Meticulous and aggressive management of infants who needed DR-CPR at birth and quality improvement of the delivery room management will result in reduced morbidities and early death for the vulnerable VLBWI.
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Humanos , Lactante , Recién Nacido , Puntaje de Apgar , Reanimación Cardiopulmonar , Bases de Datos Factuales , Salas de Parto , Enterocolitis Necrotizante/complicaciones , Epinefrina/administración & dosificación , Edad Gestacional , Hemorragia/complicaciones , Muerte del Lactante , Recién Nacido de muy Bajo Peso , Leucomalacia Periventricular/complicaciones , Modelos Logísticos , Oportunidad Relativa , Respiración con Presión Positiva , Estudios RetrospectivosRESUMEN
Congenital intracranial pial arteriovenous fistulas (AVFs) are rare cerebrovascular lesions. Their clinical manifestations tend to vary according to age, with pediatric populations being more likely to have symptoms like congestive cardiac failure and seizures because of arteriovenous shunting; hemorrhage is the major presentation in adult populations. Pediatric populations, especially newborn infants, seldom experience a hemorrhagic event. Here, we report two rare cases of neonates with congenital pial AVF presenting as intraventricular and subdural hemorrhage, respectively, which were treated with endovascular embolization.
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Adulto , Humanos , Lactante , Recién Nacido , Fístula Arteriovenosa , Encéfalo , Embolización Terapéutica , Estrógenos Conjugados (USP) , Insuficiencia Cardíaca , Hematoma Subdural , Hemorragia , Hemorragias Intracraneales , ConvulsionesRESUMEN
PURPOSE: Patent ductus arteriosus (PDA) is common in preterm infants, and about 30% of preterm infants undergo surgical ligation of the PDA. Cardiopulmonary instability, defined as hypotension and respiratory failure after PDA ligation, is reported to occur at a frequency of 40-50%. This study investigated the factors affecting cardiopulmonary instability after PDA ligation in preterm infants. METHODS: The medical records of 45 very low birth weight (VLBW) infants who underwent PDA ligation in the neonatal intensive care unit from January 2009 to December 2013 were analyzed retrospectively. PDA ligation was only performed when medical treatment for hemodynamically significant PDA failed or was contraindicated. The cases were categorized into the hemodynamic instability (n=20) and control (n=25) groups. RESULTS: Patients underwent ligation at the mean age of 14.3+/-13.3 days. There were no significant differences between groups in mortality or weaning from ventilation after PDA ligation. In the hemodynamic instability group, birth weight was significantly lower (P=0.046) and the pre-operation C-reactive protein (CRP) level was significantly higher (P=0.042) than in the control group. The use of high-frequency ventilation was higher in the hemodynamic instability group (P=0.033). There were no differences in use of inotropics, mean airway pressure at ligation, timing of ligation, or PDA size between groups. The birth weight and pre-operation CRP level at the time of ligation remained a significant risk factor for cardiopulmonary instability on multiple logistic regression analysis. CONCLUSION: In VLBW infants, lower birth weight and a higher pre-operation CRP level are related to unstable conditions after PDA ligation.
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Humanos , Lactante , Recién Nacido , Peso al Nacer , Proteína C-Reactiva , Conducto Arterioso Permeable , Hemodinámica , Ventilación de Alta Frecuencia , Hipotensión , Recien Nacido Prematuro , Recién Nacido de muy Bajo Peso , Cuidado Intensivo Neonatal , Ligadura , Modelos Logísticos , Registros Médicos , Mortalidad , Insuficiencia Respiratoria , Estudios Retrospectivos , Factores de Riesgo , Ventilación , DesteteRESUMEN
PURPOSE: This study aimed to analyze the risk factors for the development of rickets of prematurity in extremely low birth weight (ELBW) infants. METHODS: We retrospectively reviewed the data of 57 ELBW infants. Nineteen infants were diagnosed with rickets and 38 infants without rickets were recruited. On radiologic evaluation, 47% of infants had grade I, 37% had grade II, and 16% had grade III rickets. RESULTS: In ELBW infants with rickets, the durations of oxygen administration, mechanical ventilation, parenteral nutrition, and hospitalization were significantly longer compared to those of the control group. The number of days for achieving an enteral caloric intake of 80 kcal/kg/d and the number of days for regaining birth weight were significantly longer compared to those of the control group. Serial weight changes from birth weight during 8 weeks after birth was significantly lower in the rickets group than in the control group. Retinopathy of prematurity was significantly higher in the rickets group than in the control group. After adjustment for birth weight, rickets of prematurity was correlated with days for regaining birth weight (odds ratio [OR], 1.316; P=0.010), and with weight changes from birth weight at 4 weeks of age (OR, 0.964; P=0.033). CONCLUSION: In ELBW infants, the risk factors for rickets of prematurity were days for regaining birth weight from birth and the weight changes at 4 weeks of age. Early aggressive nutrition for regaining birth weight earlier may reduce the development of rickets of prematurity.
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Humanos , Lactante , Recién Nacido , Peso al Nacer , Cambios en el Peso Corporal , Ingestión de Energía , Nutrición Enteral , Hospitalización , Recién Nacido de Bajo Peso , Oxígeno , Nutrición Parenteral , Parto , Respiración Artificial , Retinopatía de la Prematuridad , Estudios Retrospectivos , Raquitismo , Factores de RiesgoRESUMEN
PURPOSE: This study aimed to identify the safety of Kangaroo Mother Care (KMC) and to analyze its medical and psychological effects. METHODS: Forty five preterm infants, admitted to Gangnam Severance Hospital NICU from October 2012 to September 2013, were recruited. KMC was performed for 1 hour daily until the discharge by either mother or father. The medical effects in KMC group were compared with historical control group. Psychological effects in KMC group were made a comparison with the non-KMC group. RESULTS: The mean gestational age was 31.3+/-3.5 weeks, and the mean birth weight was 1,080+/-250 g. KMC was started at the mean age of 18.3+/-8.4 days. No event of hypothermia, bradycardia, desaturation, or hypotension occurred. There was no event of death or sepsis. Four patients (9%) had apnea but recovered after the tactile stimulation. Duration of the hospitalization in KMC group was significantly shorter than control group (P=0.041). Spielberger's anxiety scale decreased from 49.7 to 48.5. Muller's maternal attachment inventory increased from 98.4 to 99.5. Reduction of the self-happiness score improved from 5% to 70%. The score for Spielberger's anxiety scale tended to be lower in the KMC group than in the control group without statistically significant. Muller's maternal attachment inventory score was significantly higher in the KMC group. CONCLUSION: KMC is a safe method to Korean preterm infants. Due to reduction of hospitalization and beneficial psychological effect, we believe that offering KMC to more preterm infants as a supplement therapy will be useful.
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Humanos , Recién Nacido , Ansiedad , Apnea , Peso al Nacer , Bradicardia , Padre , Edad Gestacional , Hospitalización , Hipotensión , Hipotermia , Recien Nacido Prematuro , Método Madre-Canguro , Corea (Geográfico) , Macropodidae , Madres , SepsisRESUMEN
PURPOSE: The vancomycin dosage regimen is regularly modified according to the patient's glomerular filtration rate (GFR). In the present study, we aimed to assess the usefulness of serum cystatin C (Cys-C) concentration, compared with serum creatinine (SCr) concentration, for predicting vancomycin clearance (CLvcm) in neonates. METHODS: We retrospectively analyzed the laboratory data of 50 term neonates who were admitted to the neonatal intensive care unit and received intravenous vancomycin, and assessed the pharmacokinetic profiles. Creatinine clearance (CLcr) and GFR based on Cys-C (GFRcys-c) were estimated using the Schwartz and Larsson formulas, respectively. RESULTS: The mean CLvcm (+/-standard deviation) was 74.52+/-31.17 L/hr, the volume of distribution of vancomycin was 0.67+/-0.14 L, and vancomycin half-life was 9.16+/-17.42 hours. The SCr was 0.46+/-0.25 mg/dL and serum Cys-C was 1.43+/-0.34 mg/L. The peak and trough concentrations of vancomycin were 24.65+/-14.84 and 8.10+/-5.35 mcg/mL, respectively. The calculated GFR based on serum creatinine concentration (GFR-Cr) and GFRcys-c were 70.2+/-9.45 and 63.6+/-30.18 mL/min, respectively. The correlation constant for CLvcm and the reciprocal of Cys-C (0.479, P=0.001) was significantly higher than that for CLvcm and the reciprocal of SCr (0.286, P=0.044). GFRcys-c was strongly correlated with CLvcm (P=0.001), and the correlation constant was significantly higher than that for CLvcm and CLcr (0.496, P=0.001). Linear regression analysis showed that only GFRcys-c was independently and positively correlated with CLvcm (F=41.9, P<0.001). CONCLUSION: The use of serum Cys-C as a marker of CLvcm could be beneficial for more reliable predictions of serum vancomycin concentrations, particularly in neonates.
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Humanos , Recién Nacido , Creatinina , Cistatina C , Tasa de Filtración Glomerular , Semivida , Cuidado Intensivo Neonatal , Modelos Lineales , Estudios Retrospectivos , VancomicinaRESUMEN
PURPOSE: The purpose of this study was to evaluate the efficacy and safety of Montelukast sodium in the prevention of bronchopulmonarydysplasia (BPD). METHODS: The Interventional study was designed as a multicenter, prospective, and randomized trial, with open labeled and parallel-experimental groups, 66 infants were enrolled and allocated to either the case group (n=30) or the control group (n=36) based on gestational age (GA). Infants in the case group were given Montelukast sodium (Singulair) based on their body weight (BW). Zero week was defined as the start time of the study. RESULTS: The incidence of moderate to severe BPD was not different between the groups (case group: 13 of 30 [43.3%] vs. control group: 19 of 36 [52.8%], P=0.912). Additionally, secondary outcomes such as ventilation index, mean airway pressure and resort to systemic steroids were not significantly different. There were no serious adverse drug reactions in either group, and furthermore the rate of occurrence of mild drug related-events were not significantly different (case group: 10 of 42 [23.8%] vs. control group: 6 of 48 (15.8%), P=0.414). CONCLUSION: Montelukast was not effective in reducing moderate or severe BPD. There were no significant adverse drug events associated with Montelukast treatment.
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Humanos , Lactante , Recién Nacido , Peso Corporal , Displasia Broncopulmonar , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos , Edad Gestacional , Colonias de Salud , Incidencia , Recien Nacido Prematuro , Antagonistas de Leucotrieno , Farmacocinética , Estudios Prospectivos , Sodio , Esteroides , VentilaciónRESUMEN
Autosomal recessive polycystic kidney disease (ARPKD) is a severe form of polycystic kidney disease that is characterized by enlarged kidneys and congenital hepatic fibrosis. The clinical spectrum of this condition shows wide variation. Approximately 30-50% of affected individuals die in the neonatal period, while others survive into adulthood. ARPKD is caused by mutations in the polycystic kidney and hepatic disease 1 (PKHD1) gene on chromosome 6p12, which consists of 86 exons variably assembled into many alternatively spliced transcripts. We report a case of a pathogenic PKHD1 frameshift mutation, c.889_931del43, which was identified using direct full sequencing, associated with enlarged cystic kidneys and dilatation of intrahepatic bile duct, as observed on imaging studies.