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1.
Gene ; 910: 148337, 2024 Jun 05.
Artigo em Inglês | MEDLINE | ID: mdl-38432533

RESUMO

Bronchopulmonary dysplasia (BPD) is a serious chronic lung disease affecting extremely preterm infants. While mitochondrial dysfunction has been investigated in various medical conditions, limited research has explored mitochondrial DNA (mtDNA) gene mutations, specifically in BPD. This study aimed to evaluate mitochondrial mtDNA gene mutations in extremely preterm infants with BPD. In this prospective observational study, we enrolled a cohort of extremely preterm infants diagnosed with BPD. Clinical data were collected to provide comprehensive patient profiles. Peripheral blood mononuclear cells were isolated from whole-blood samples obtained within a defined timeframe. Subsequently, mtDNA extraction and sequencing using next-generation sequencing technology were performed to identify mtDNA gene mutations. Among the cohort of ten extremely preterm infants with BPD, mtDNA sequencing revealed the presence of mutations in seven patients, resulting in a total of twenty-one point mutations. Notably, many of these mutations were identified in loci associated with critical components of the respiratory chain complexes, vital for proper mitochondrial function and cellular energy production. This pilot study provides evidence of mtDNA point mutations in a subset of extremely preterm infants with BPD. These findings suggest a potential association between mitochondrial dysfunction and the pathogenesis of BPD. Further extensive investigations are warranted to unravel the mechanisms underlying mtDNA mutations in BPD.


Assuntos
Displasia Broncopulmonar , Doenças Mitocondriais , Lactente , Humanos , Recém-Nascido , Lactente Extremamente Prematuro , Displasia Broncopulmonar/genética , Leucócitos Mononucleares , Projetos Piloto , Mutação , DNA Mitocondrial/genética
2.
Neonatology ; 121(1): 74-80, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-37866355

RESUMO

INTRODUCTION: This study aimed to investigate the outcomes of infants at 18-24 months born in the Korean Neonatal Network with a birth weight <500 g. METHODS: The anthropometric and neurodevelopmental data of infants with a birth weight <500 g at a gestational age of ≥22 weeks who were registered in the Korean Neonatal Network 2013-2017 and followed up at a corrected age of 18-24 months were reviewed. Neurodevelopmental impairment was defined as the presence of any of the following: (1) cerebral palsy; (2) severe visual impairment; (3) hearing impairment; or (4) cognitive impairment. Cognitive impairment was defined as (1) a Bayley Scales of Infant Development-II Mental Development Index score <70; and (2) Bayley Scales of Infant and Toddler Development-III Cognitive and Language Composite scores <85. Cognitive testing was performed for infants with suspected problems upon clinician's referral to developmental specialists. RESULTS: At a median corrected age of 20 months, 26/52 (50%) of included infants had neurodevelopmental impairment. Cerebral palsy, severe visual impairment, wearing of glasses, hearing impairment, and cognitive impairment occurred in 22%, 0%, 8%, 5%, and 57% of the included infants, respectively. The proportions of infants with <2 standard deviations of weight, length, and head circumference were 54%, 52%, and 56%, respectively. The majority (70%) of infants were rehospitalized, and the most common cause was respiratory problems. CONCLUSION: Half of infants with a birth weight <500 g in Korea may exhibit neurodevelopmental impairment and growth retardation at a corrected age of 18-24 months. Multidisciplinary follow-up along with continuous rehabilitation will be needed to improve neurological and physical development in this special population.


Assuntos
Paralisia Cerebral , Perda Auditiva , Recém-Nascido , Lactente , Feminino , Humanos , Pré-Escolar , Criança , Peso ao Nascer , Estudos de Coortes , Paralisia Cerebral/diagnóstico , Paralisia Cerebral/epidemiologia , Paralisia Cerebral/etiologia , Perda Auditiva/epidemiologia , Perda Auditiva/complicações , Transtornos da Visão/epidemiologia , República da Coreia/epidemiologia , Deficiências do Desenvolvimento/diagnóstico , Deficiências do Desenvolvimento/epidemiologia , Deficiências do Desenvolvimento/etiologia
3.
BMC Pediatr ; 23(1): 244, 2023 05 18.
Artigo em Inglês | MEDLINE | ID: mdl-37202724

RESUMO

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.


Assuntos
Infecções por Citomegalovirus , Leite Humano , Recém-Nascido , Lactente , Feminino , Humanos , Estudos Prospectivos , Infecções por Citomegalovirus/diagnóstico , Infecções por Citomegalovirus/prevenção & controle , Recém-Nascido de muito Baixo Peso , Citomegalovirus/genética
4.
Clin Exp Pediatr ; 65(11): 540-546, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-35914772

RESUMO

BACKGROUND: Although the benefits of breastfeeding are broadly acknowledged with the efforts of the government and several medical societies, the rate of exclusive breastfeeding through 6 months is lower in Korea than in developed countries. PURPOSE: This study aimed to investigate pediatricians' perceptions of breastfeeding barriers and the current breastfeeding counseling environment and propose government policies to encourage breastfeeding in Korea. METHODS: Fourteen survey questions were developed during meetings of Korean Society of Breastfeeding Medicine experts. The Korean Pediatric Society emailed a structured questionnaire to domestic pediatricians registered as official members of the Korean Pediatric Society on May 4, 2021, and June 3, 2021. This study examined the survey responses received from 168 pediatricians. RESULTS: The 168 respondents included 62 professors, 53 paid doctors, and 53 private physicians. Breastfeeding was recommended by 146 Korean pediatricians (86.9%). However, only 99 responders (59%) currently provide breastfeeding counseling in hospitals. Most respondents stated providing less than 15 minutes of breastfeeding counseling time in the clinic. Moreover, 89.88% of the respondents responded that they would participate in breastfeeding counseling education if an appropriate breastfeeding counseling program was newly established. CONCLUSION: This study showed that, although Korean pediatricians had a positive attitude toward breastfeeding, limited counseling was provided for parents. Along with policy support to improve the medical environment through the establishment of an appropriate breastfeeding counseling program, high-quality counseling and an increased breastfeeding rate are expected.

5.
Clin Exp Pediatr ; 65(7): 350-357, 2022 07.
Artigo em Inglês | MEDLINE | ID: mdl-34886592

RESUMO

BACKGROUND: Due to increases in the number of infants born with younger gestational age (GA) and lower birth weight, the incidence of neonatal sepsis is increasing. PURPOSE: We investigated the changes in the prevalence of bacterial pathogens, their antimicrobial susceptibility, and sepsis-related mortality during 20 years at a neonatal intensive care unit. METHODS: The study period was divided into two 10-year phases (1998-2007 vs. 2008-2017). Medical records were reviewed to gather data on demographics, causative microbial pathogens, incidence of multidrug-resistant organisms, antimicrobial susceptibility, and rates of sepsis-related mortality. RESULTS: In both study phases, the most common pathogens for neonatal sepsis were coagulase-negative Staphylococcus (CoNS) (28.6%) and Enterobacter cloacae (16.1%) for early-onset sepsis (EOS, ≤72 hours after birth) and CoNS (54.7%) and Staphylococcus aureus (12.9%) for late-onset sepsis (LOS, >72 hours after birth). CoNS and S. aureus showed 100% sensitivity to vancomycin in both phases. The susceptibility of S. aureus to oxacillin increased from 19.2% to 57.9% in phase II than phase I. K. pneumonia and E. cloacae showed increases in its susceptibility to gentamicin, cefotaxime and ceftriaxone in phase II than phase I. In both phases, the most common pathogens that caused sepsis-related death were K. pneumoniae (18.2%) and Pseudomonas aeruginosa (13.6%). Sepsis-related mortality rate was higher in infants with GA <37 weeks than those with GA over 37 weeks (P=0.016). In addition, the mortality rate of neonatal sepsis caused by gram-negative bacteria was significantly higher than that caused by gram-positive bacteria (P<0.001). CONCLUSIONS: CoNS was the most common pathogen for EOS and LOS. While we found significant changes in antimicrobial sensitivities over time. GA below 37 weeks and gram-negative organisms are associated with mortality rate.

6.
BMC Pediatr ; 21(1): 460, 2021 10 20.
Artigo em Inglês | MEDLINE | ID: mdl-34666738

RESUMO

BACKGROUND: Right-sided congenital diaphragmatic hernia (RCDH) is relatively rare compared with left-sided congenital diaphragmatic hernia (LCDH). Clinical data of RCDH, especially with respect to antenatal prediction of neonatal outcome, are lacking. The aim of this study was to report the treatment outcomes of patients with antenatally diagnosed RCDH and to evaluate the predictability of observed-to-expected lung area-to-head circumference ratio (O/E LHR) for perinatal outcomes, focused on mortality or extracorporeal membrane oxygenation (ECMO) requirement. METHODS: We retrospectively reviewed the medical records of newborn infants with isolated RCDH. We analyzed and compared the clinical and prenatal characteristics including the fetal lung volume, which was measured as the O/E LHR, between the survivors and the non-survivors. RESULTS: A total of 26 (66.7%) of 39 patients with isolated RCDH survived to discharge. The O/E LHR was significantly greater in survivors (64.7 ± 21.2) than in non-survivors (40.5 ± 23.4) (P =.027). It was greater in survivors without ECMO requirement (68.3 ± 15.1) than non-survivors or those with ECMO requirement (46.3 ± 19.4; P = .010). The best O/E LHR cut-off value for predicting mortality in isolated RCDH was 50. CONCLUSIONS: The findings in this study suggest that O/E LHR, a well-characterized prognostic indicator in LCDH, could be applied to a fetus with antenatally diagnosed RCDH. A large cohort study is required to verify the association between O/E LHR values and the graded severity of RCDH.


Assuntos
Hérnias Diafragmáticas Congênitas , Feminino , Idade Gestacional , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Hérnias Diafragmáticas Congênitas/terapia , Humanos , Lactente , Recém-Nascido , Pulmão/diagnóstico por imagem , Gravidez , Prognóstico , Estudos Retrospectivos , Ultrassonografia Pré-Natal
7.
J Korean Med Sci ; 36(31): e206, 2021 Aug 09.
Artigo em Inglês | MEDLINE | ID: mdl-34402234

RESUMO

BACKGROUND: This study aimed to investigate the survival and morbidities of infants in the Korean Neonatal Network (KNN) with birth weight (BW) < 500 g. METHODS: The demographic and clinical data of 208 live-born infants with a BW < 500 g at a gestational age of ≥ 22 weeks who were treated in the neonatal intensive care units of the KNN between 2013 and 2017 were reviewed. RESULTS: The survival rate of the infants was 28%, with a median gestational age and BW of 243/7 weeks (range, 220/7-336/7) and 440 g (range, 220-499), respectively. Multivariable Cox proportional hazards analysis demonstrated that survival to discharge was associated with longer gestation, higher BW, female sex, singleton gestation, use of any antenatal corticosteroids, and higher Apgar scores at 5 minutes. The overall survival rates were significantly different between the BW categories of < 400 g and 400-499 g. However, there was no significant difference in the incidence of any morbidity between the BW groups. Half of the deaths of infants with BW < 500 g occurred within a week of life, mainly due to cardiopulmonary and neurologic causes. The major causes of death in infants after 1 week of age were infection and gastrointestinal disease. Among the surviving infants, 79% had moderate to severe bronchopulmonary dysplasia, 21% underwent surgical ligation of patent ductus arteriosus, 12% had severe intraventricular hemorrhage (grade III-IV), 38% had sepsis, 9% had necrotizing enterocolitis (stage ≥ 2), and 47% underwent laser treatment for retinopathy of prematurity. The median length of hospital stay was 132 days (range, 69-291), and 53% required assistive devices at discharge. CONCLUSION: Despite recent advances in neonatal intensive care, the survival and morbidity rates of infants with BW < 500 g need further improvement.


Assuntos
Peso ao Nascer , Doenças do Prematuro/mortalidade , Terapia Intensiva Neonatal/estatística & dados numéricos , Displasia Broncopulmonar/epidemiologia , Enterocolite Necrosante/epidemiologia , Feminino , Humanos , Lactente , Mortalidade Infantil , Lactente Extremamente Prematuro , Recém-Nascido , Recém-Nascido Prematuro , Recém-Nascido de muito Baixo Peso , Infecções/epidemiologia , Masculino , Morbidade , Gravidez , Resultado da Gravidez/epidemiologia , República da Coreia/epidemiologia , Taxa de Sobrevida
8.
Neonatology ; 118(2): 180-186, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33756484

RESUMO

PURPOSE: The agreement between axillary temperature (AT) and rectal temperature (RT) measurements has not been well established in preterm infants. Therefore, our study aimed to evaluate the agreement between AT and RT measurements in very preterm infants. METHODS: Preterm infants <32 weeks of gestational age were prospectively included. The infants' body temperature (BT) was measured twice a day from day 1 to day 6. A paired t-test and the Bland-Altman method were used to analyze the difference between the AT and RT. A linear regression model was used to explore the effects of environmental factors on the differences of BT between the axillary and rectal measurements and to calibrate the RT according to the AT. RESULTS: Eighty infants each underwent 6 paired axillary and rectal measurements. The gestational age varied from 22 to 31 weeks (mean 28 weeks). The birth weight varied from 302 to 1,770 g (mean 1,025 g). The AT was significantly lower than the RT. The difference between the RT and AT significantly increased with increasing RT. The AT and RT demonstrated poor agreement overall; however, the RT can be estimated using the AT with the following equation: RT = -4.033 + 1.116 × AT. Environmental factors, including the incubator temperature, incubator humidity, phototherapy, and application of invasive mechanical ventilation did not affect the differences between the AT and RT measurements. CONCLUSION: AT measurements cannot be interchangeably used with RT measurements in very preterm infants.


Assuntos
Temperatura Corporal , Recém-Nascido Prematuro , Axila , Humanos , Lactente , Recém-Nascido , Recém-Nascido de muito Baixo Peso , Temperatura
9.
BMC Pediatr ; 21(1): 120, 2021 03 11.
Artigo em Inglês | MEDLINE | ID: mdl-33706730

RESUMO

BACKGROUND: To evaluate the long-term functional and structural pulmonary development in children with repaired congenital diaphragmatic hernia (CDH) and to identify the associated perinatal-neonatal risk factors. METHODS: Children with repaired CDH through corrective surgery who were born at gestational age ≥ 35 weeks were included in this analysis. Those who were followed for at least 5 years were subjected to spirometry and chest computed tomography for evaluation of their functional and structural growth. Main bronchus diameters and lung volumes (total, left/right) were measured. According to total lung volume (TLV) relative to body surface area, children were grouped into TLV ≥ 50 group and TLV < 50 group and the associations with perinatal-neonatal factors were analyzed. RESULTS: Of the 28 children (mean age, 6.2 ± 0.2 years) with left-sided CDH, 7 (25%) had abnormal pulmonary function, of whom 6 (87%) showed restrictive patterns. All pulmonary functions except FEF25-75% were worse than those in matched healthy control group. Worse pulmonary function was significantly associated with small head and abdominal circumferences at birth. The mean TLV was 1339.1 ± 363.9 mL and LLV/TLV was 47.9 ± 2.5 mL. Children with abnormal pulmonary function were more likely to have smaller lung volumes. In multivariate analysis, abdominal circumference at birth was significantly associated with abnormal lung volume. CONCLUSIONS: A quarter of children with repaired CDH showed abnormal pulmonary function. Small abdominal circumference at birth was associated with abnormal pulmonary function and lower TLV. .


Assuntos
Hérnias Diafragmáticas Congênitas , Criança , Feminino , Idade Gestacional , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Hérnias Diafragmáticas Congênitas/cirurgia , Humanos , Lactente , Recém-Nascido , Pulmão/diagnóstico por imagem , Medidas de Volume Pulmonar , Gravidez
10.
Pediatrics ; 147(1)2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33310907

RESUMO

BACKGROUND AND OBJECTIVES: The existing prediction formulas for in-hospital mortality of very low birth weight (VLBW) infants were mostly developed in the 1990s or 2000s and thus may not reflect the recently improved levels of neonatal care. We conducted this study to build a model for predicting the in-hospital mortality using perinatal factors available soon after birth. METHODS: We gathered data on VLBW infants from the Korean Neonatal Network, a nationwide, prospective, Web-based registry that enrolled patients from 2013 to 2017. Perinatal variables that were significantly associated with mortality in univariate logistic regression or those with apparent clinical importance were included in the multivariable logistic regression model. The final formula was constructed by considering the collinearity, parsimony, goodness of fit, and clinical interpretation. RESULTS: A total of 9248 VLBW infants were analyzed, including 1105 (11.9%) who died during hospitalization. The mean gestational age was 29.0 ± 2.9 weeks and the mean birth weight was 1096 ± 280 g. Significant variables used in the final equation included polyhydramnios, oligohydramnios, gestational age, Apgar score at 1 minute, intubation at birth, birth weight, and base excess. In internal validation, the area under the curve (AUC) for the prediction of in-hospital mortality was 0.870 and the optimism-corrected AUC was 0.867. The prediction equation revealed good discrimination and calibration in the external validation as well (AUC: 0.876). CONCLUSIONS: The newly developed Korean Neonatal Network prediction formula for in-hospital mortality could be a useful tool in counseling by providing a reliable prediction for the in-hospital mortality of VLBW infants.


Assuntos
Regras de Decisão Clínica , Mortalidade Hospitalar , Recém-Nascido Prematuro , Recém-Nascido de muito Baixo Peso , Mortalidade Perinatal , Área Sob a Curva , Peso ao Nascer , Feminino , Idade Gestacional , Humanos , Recém-Nascido , Modelos Logísticos , Masculino , Sistema de Registros , Reprodutibilidade dos Testes , República da Coreia/epidemiologia , Estudos Retrospectivos , Medição de Risco , Fatores de Risco
11.
Transl Pediatr ; 10(11): 3091-3095, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34976775

RESUMO

In this study, we present the case of a 900 g, male infant born at 27+5 weeks, who was placed on high frequency oscillatory ventilation (HFOV) until repair of a left congenital diaphragmatic hernia (CDH) at 39 days of life (DOL). To date, this is the smallest infant with repair of the left CDH reported in the literature. After birth, he passed the cardiopulmonary stabilization phase and successfully underwent delayed surgery; in the process, he received ventilator assistance through HFOV. He weighed 1,660 gm at the time of surgery. We performed the thoracoscopic primary closure of the diaphragmatic defect. He was extubated on post-operation day (POD) 7 and discharged from hospital on POD 36 with 0.1 L/min supplemental oxygen via nasal cannula. He is being followed for growth and development and there has been no recurrence at the surgical site at 24 months of corrected age. In this case, high mean airway pressure (MAP) was required based on the patient's weight to achieve adequate recruitment of the left lung, and the patient was diagnosed with mental developmental delay on Bayley Scales of Infant Development-II. Thus, we suggest that the postnatal course and long-term outcomes for extremely low birth weight (ELBW) and preterm infants with left CDH is different from that for full-term babies. Therefore, future research should focus on preterm infants with left CDH.

12.
Korean Circ J ; 50(12): 1113-1123, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33258320

RESUMO

BACKGROUND AND OBJECTIVES: This study aimed to provide morbidity and mortality information on very low birth weight (VLBW) infants with congenital heart disease (CHD-VLBWs). METHODS: The study used a 10-year cohort of VLBW infants from a single institution. CHD was classified according to International Classification of Diseases, Version 9, Clinical Modification. Mortality and neonatal outcomes were assessed by comparing the CHD-VLBWs with gestational age- and birth weight-matched controls. RESULTS: The prevalence of CHD-VLBWs was 7.5% (79/1,050), mean gestational age was 31.1±3.2 weeks, and mean birth weight was 1,126.2±268.3 g; 50.6% of the infants were small for the gestational age. The CHD-VLBWs more commonly had bronchopulmonary dysplasia (BPD), and the longer they were exposed to oxygen, the more frequently they developed BPD. Those with cyanotic heart disease developed severe BPD more frequently. Necrotizing enterocolitis (NEC) occurred frequently in the CHD-VLBWs and was not associated with their feeding patterns. CHD-VLBWs had a higher mortality rate; prematurity-related diseases were the leading cause of death before surgery, while heart-related problems were the leading cause of death after surgery. We found no significant difference in mortality from prematurity-related disease between the CHD-VLBWs and controls. In the subgroup analysis of CHD, the cyanotic CHD group had a higher incidence of BPD and higher mortality rate than the acyanotic CHD group. CONCLUSIONS: CHD-VLBWs showed higher BPD, NEC, and mortality rates than those without CHD. There was also a higher incidence of BPD and mortality in VLBW infants with cyanotic CHD than in those with acyanotic CHD.

13.
BMC Nephrol ; 21(1): 437, 2020 10 19.
Artigo em Inglês | MEDLINE | ID: mdl-33076845

RESUMO

BACKGROUND: Peritoneal dialysis (PD) has been used occasionally in extremely-low-birth-weight (ELBW) infants with acute kidney injury (AKI). This study aimed to evaluate the clinical characteristics and outcomes of ELBW infants with AKI treated with PD. METHODS: In this retrospective cohort study, the medical records of ELBW infants with AKI, who underwent PD from January 2008 to February 2018, were reviewed. A PD catheter (7.5-9.0 Fr) or central venous catheter (4 Fr) was used for the peritoneal access. Treatment with PD solutions (2.5 or 4.25%) was started at 10 mL/kg, which was increased to 20-30 mL/kg for 60-120 min/cycle continuing for 24 h. RESULTS: Twelve ELBW infants (seven male and five female infants) were treated, and their mean (±SD) gestational age and birth weight were 27.2 (±3.3) weeks and 706.5 (±220.5) g, respectively. Two patients had severe perinatal asphyxia (5-min Apgar score ≤ 3). The most important indication for starting PD was AKI due to sepsis. The average (±SD) duration of PD was 9.4 (± 7.7) days. The potassium levels in the ELBW infants with hyperkalemia decreased from 6.8 to 5.0 mg/mL after 9.3 (± 4.4) days. The most common complication of PD was mechanical dysfunction of the catheters, such as dialysate leakage (75%). Two patients were successful weaned off PD. The mortality rate of the infants treated with PD was 91.7%. CONCLUSIONS: In this series, the mortality rate of ELBW infants with AKI treated with PD was relatively high because of their incompletely developed organ systems. Therefore, the use of PD should be carefully considered for the treatment of ELBW infants with AKI in terms of decisions regarding resuscitation.


Assuntos
Injúria Renal Aguda/terapia , Recém-Nascido de Peso Extremamente Baixo ao Nascer , Diálise Peritoneal , Injúria Renal Aguda/mortalidade , Feminino , Humanos , Hiperpotassemia/mortalidade , Lactente , Recém-Nascido , Recém-Nascido Prematuro , Masculino , Insuficiência de Múltiplos Órgãos/mortalidade , Diálise Peritoneal/efeitos adversos , Diálise Peritoneal/mortalidade , Prognóstico , Estudos Retrospectivos
14.
Medicine (Baltimore) ; 99(19): e20113, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32384486

RESUMO

Autosomal recessive polycystic kidney disease (ARPKD) is the most common inherited childhood-onset renal disease, with underlying ciliopathy, and varies widely in clinical severity. The aim of this study was to describe the most severe form of ARPKD, with a fatal clinical course, and its association with mutations in polycystic kidney and hepatic disease 1 (fibrocystin) (PKHD1). Clinical, imaging, pathological, and molecular genetic findings were reviewed in patients prenatally affected with ARPKD and their families.Five unrelated Korean families, including 9 patients, were analyzed. Among the 9 patients, 2 fetuses died in utero, 6 patients did not survive longer than a few days, and 1 patient survived for 5 months with ventilator support and renal replacement therapy. A total of 6 truncating mutations (all nonsense) and 4 missense mutations were detected in a compound heterozygous state, including 4 novel mutations. The most severe phenotypes were shared among all affected patients in each family, irrespective of mutation types.Our data suggest a strong genotype-phenotype relationship in ARPKD, with minimal intra-familial heterogeneity. These findings are important for informing future reproductive planning in affected families.


Assuntos
Rim Policístico Autossômico Recessivo/genética , Rim Policístico Autossômico Recessivo/mortalidade , Receptores de Superfície Celular/genética , Feto Abortado , Feminino , Genótipo , Humanos , Recém-Nascido , Masculino , Mutação , Fenótipo , Rim Policístico Autossômico Recessivo/patologia , República da Coreia , Índice de Gravidade de Doença
15.
Sci Rep ; 10(1): 4430, 2020 03 10.
Artigo em Inglês | MEDLINE | ID: mdl-32157119

RESUMO

Plasma B-type natriuretic peptide (BNP) is a useful marker for diagnosis of hemodynamically significant PDA (hsPDA) and serial BNP measurement is also valuable for monitoring treatment response. This retrospective study was performed to evaluate whether plasma BNP level can predict treatment response to ibuprofen in preterm infants born at <30 weeks of gestation with hsPDA. Plasma BNP was measured before (baseline) and 12 to 24 h after (post-treatment) completion of the first (IBU1) and second (IBU2) course of ibuprofen. We compared the BNP levels of responders (closed or insignificant PDA) with those of non-responders (hsPDA requiring further pharmacologic or surgical closure) to each course of ibuprofen. The treatment response rates for IBU1 (n = 92) and IBU2 (n = 19) were 74% and 26%, respectively. In IBU1, non-responders had lower gestational age and birth weight than responders (both, P = 0.004), while in IBU2, non-responders had lower birth weight (P = 0.014) and platelet counts (P = 0.005) than responders; however, baseline BNP levels did not differ significantly between responders and non-responders in either IBU1 (median 1,434 vs. 1,750 pg/mL) or IBU2 (415 vs. 596 pg/mL). Post-treatment BNP was a useful marker for monitoring treatment efficacy of IBU1 and IBU2 for hsPDA with a cut-off value of 331 pg/mL (P < 0.001) and 423 pg/mL(P < 0.010), respectively. We did not identify a cut-off baseline BNP level that could predict treatment response to ibuprofen in preterm infants with hsPDA.


Assuntos
Anti-Inflamatórios não Esteroides/uso terapêutico , Biomarcadores/sangue , Peso ao Nascer , Permeabilidade do Canal Arterial/sangue , Permeabilidade do Canal Arterial/tratamento farmacológico , Ibuprofeno/uso terapêutico , Recém-Nascido Prematuro , Permeabilidade do Canal Arterial/patologia , Feminino , Idade Gestacional , Hemodinâmica , Humanos , Recém-Nascido , Masculino , Estudos Retrospectivos
16.
Transfus Apher Sci ; 59(3): 102730, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31948914

RESUMO

BACKGROUND: Exchange transfusion (ET) is an established, efficacious, and reliable practice for severe neonatal hyperbilirubinemia, hemolytic disease of the newborn, and neonatal sepsis. This study assessed the indications and clinical outcomes of ET performed in a tertiary hospital in Korea. MATERIALS AND METHODS: We studied 64 ET sessions performed on 23 neonates between March 1999 and March 2018. ET was performed based on estimated double volume exchange transfusion using fresh red blood cells and fresh frozen plasma. Patients' clinical information, including demographic data and ET indication, and laboratory data were collected pre- and post-ET. RESULTS: The most common ET indication was hyperbilirubinemia with hemolytic anemia due to non-ABO maternal blood group discrepancies. In three preterm babies, ETs were performed for severe anemia, leukocytosis, and hyperkalemia cases. Before ET, the patients showed slightly high WBC counts, low hemoglobin levels, and low platelet counts. After ET, blood examination revealed normal WBC counts, increased hemoglobin levels, and decreased platelet counts (all P < 0.001). Bilirubin levels decreased immediately after ET (P < 0.001). Electrolyte and C-reactive protein levels showed no significant changes after ETs. Adverse events occurred in 11 (47.8 %) patients; the most common were hypoxemia and hypotension. One infant experienced cardiorespiratory arrest due to hypercalcemia and was successfully resuscitated. No one died within 24 h of ET. However, five infants showed hyperbilirubinemia aggravation. CONCLUSIONS: ET is an effective treatment modality for leukocytosis and hyperbilirubinemia with low mortality but involves common adverse events post-ET. This report provides an overview of current ET practices in Korea.


Assuntos
Anemia/terapia , Transfusão Total/métodos , Hiperbilirrubinemia Neonatal/terapia , Transfusão Total/efeitos adversos , Feminino , Humanos , Recém-Nascido , Masculino , República da Coreia
17.
J Clin Ultrasound ; 48(2): 89-96, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31609460

RESUMO

PURPOSE: To evaluate the prenatal sonographic predictive markers of the outcome in fetuses with bronchopulmonary sequestration (BPS). METHODS: BPS size and diameter of the feeding artery (FA) were measured prenatally and postnatally. Velocity of the FA and the left ventricular-modified myocardial performance index (LV mod-MPI) were also evaluated prenatally. RESULTS: Forty-seven women were included in the study. Mean gestational age, mass size, diameter and velocity of the FA, and LV mod-MPI at prenatal diagnosis were 23.5 ± 2.2 weeks, 3.6 ± 8.3 cm, 2.3 ± 0.6 mm, 46.6 ± 15.4 cm/s, and 0.46 ± 0.06, respectively. Mean mass diameter and FA diameter measured on postnatal CT examinations were 3.8 ± 1.0 cm and 2.3 ± 0.7 mm, respectively. Five patients had respiratory symptoms after birth. Twenty children (43%) underwent or were scheduled to undergo mass excision, and the remaining 27 (57%) were doing well without any intervention. There was no neonatal death. LV mod-MPI at diagnosis, the FA diameter after birth and the serial change in the FA size were significantly associated with postnatal mass excision. CONCLUSION: The FA diameter and LV mod-MPI may be additional markers for predicting whether fetuses with BPS should undergo mass excision in early childhood or conservative care.


Assuntos
Sequestro Broncopulmonar/diagnóstico por imagem , Sequestro Broncopulmonar/embriologia , Ultrassonografia Pré-Natal/métodos , Adulto , Feminino , Humanos , Gravidez
18.
Pediatr Neonatol ; 60(6): 648-653, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-30962158

RESUMO

BACKGROUND: The aim of our study was to measure the basal serum cortisol concentration immediately after birth and to determine its association with perinatal factors and clinical outcomes in very low birth weight (VLBW) infants. METHODS: Basal serum cortisol level was obtained within one hour after birth in inborn VLBW infants. The association between the basal serum cortisol level and perinatal and clinical outcomes was analyzed by comparing the groups with high versus low cortisol levels. RESULTS: In total, 80 infants were included. The median concentration of basal serum cortisol was 167 nmol/L with an interquartile range of 98-298 nmol/L. The basal serum cortisol concentration positively correlated with elapsed time from the last betamethasone dose. Low serum cortisol concentration was associated with antenatal corticosteroid therapy, low lactic acid level, and low leukocyte count at birth. Basal serum cortisol level was not associated with mortality and neonatal morbidities including hypotension and severe grade intraventricular hemorrhage. CONCLUSION: Both maternal corticosteroid therapy and perinatal distress may affect the basal serum cortisol concentration in VLBW infants early after birth.


Assuntos
Hidrocortisona/sangue , Recém-Nascido de muito Baixo Peso/sangue , Feminino , Humanos , Recém-Nascido , Masculino , Gravidez
19.
Korean J Pediatr ; 62(5): 187-192, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30999730

RESUMO

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.

20.
J Korean Med Sci ; 33(48): e309, 2018 Nov 26.
Artigo em Inglês | MEDLINE | ID: mdl-30473651

RESUMO

BACKGROUND: The aim of this study was to observe long-term outcomes of very low birth weight infants (VLBWIs) born between 2013 and 2014 in Korea, especially focusing on neurodevelopmental outcomes. METHODS: The data were collected from Korean Neonatal Network (KNN) registry from 43 and 54 participating units in 2013 and 2014, respectively. A standardized electronic case report form containing 30 items related to long-term follow up was used after data validation. RESULTS: Of 2,660 VLBWI, the mean gestational age and birth weight were 291/7 ± 26/7 weeks and 1,093 ± 268 g in 2013 and 292/7 ± 26/7 weeks and 1,125 ± 261 g in 2014, respectively. The post-discharge mortality rate was 1.2%-1.5%. Weight < 50th percentile was 46.5% in 2013 and 66.1% in 2014. The overall prevalence of cerebral palsy among the follow up infants was 6.2% in 2013 and 6.6% in 2014. The Bayley Scales of Infant Developmental Outcomes version II showed 14%-25% of infants had developmental delay and 3%-8% of infants in Bayley version III. For the Korean developmental screening test for infants and children, the area "Further evaluation needed" was 5%-12%. Blindness in both eyes was reported to be 0.2%-0.3%. For hearing impairment, 0.8%-1.9% showed bilateral hearing loss. Almost 50% were readmitted to hospital with respiratory illness as a leading cause. CONCLUSION: The overall prevalence of long-term outcomes was not largely different among the VLBWI born between 2013 and 2014. This study is the first large national data study of long-term outcomes.


Assuntos
Doenças do Prematuro/epidemiologia , Recém-Nascido de muito Baixo Peso , Peso ao Nascer , Cegueira/diagnóstico , Cegueira/epidemiologia , Paralisia Cerebral/diagnóstico , Paralisia Cerebral/epidemiologia , Bases de Dados Factuais , Deficiências do Desenvolvimento/diagnóstico , Deficiências do Desenvolvimento/epidemiologia , Idade Gestacional , Perda Auditiva/diagnóstico , Perda Auditiva/epidemiologia , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Doenças do Prematuro/diagnóstico , Prevalência , Sistema de Registros , República da Coreia/epidemiologia
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