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To assess whether the number of extremely low birth weight (ELBW) infants treated annually in neonatal intensive care units (NICUs) in Taiwan affects the mortality and morbidity of this patient population. This retrospective cohort study included preterm infants with ELBW (≤1000 g). NICUs were divided into three subgroups according to the annual admissions of ELBW infants (low, ≤10; medium, 11–25; and high, >25). Perinatal characteristics, mortality, and short-term morbidities were compared between groups. A total of 1945 ELBW infants from 17 NICUs were analyzed (low-volume, n = 263; medium-volume, n = 420; and high-volume, n = 1262). After risk adjustments, infants from NICUs with low patient volumes were at a higher risk of death. The risk-adjusted odds ratios (aOR) for mortality were 0.61 (95% CI, 0.43–0.86) in the high-volume NICUs and 0.65 (95% CI, 0.43–0.98) in medium-volume NICUs, compared with infants admitted to low-volume NICUs. Infants in medium-volume NICUs had the lowest incidence of prenatal steroid exposure (58.1%, P < 0.001) and were associated with the highest risk of necrotizing enterocolitis (aOR, 2.35 [95% CI, 1.48–3.72]), severe intraventricular hemorrhage (aOR, 1.55 [95% CI, 1.01–2.28]), and bronchopulmonary dysplasia (aOR, 1.61 [95% CI, 1.10–2.35]). However, survival without major morbidity did not differ between the groups. The mortality risk was higher among ELBW infants admitted to NICUs with a low annual patient volume. This may emphasize the importance of systematically referring patients from these vulnerable populations to appropriate care settings. [ABSTRACT FROM AUTHOR]

Feeding problems are common problems seen in premature infants following their discharge from the NICU. However, the prevalence of feeding issues and failure to thrive among preterm infants in Taiwan is uncertain. All former studies of prevalence and identifications of feeding issues were from western countries. Those findings are therefore not representative. The objectives of this study were to understand the proportion of feeding problems and its trend in Taiwanese children who were born prematurely as well as to identify the medical and rehabilitation needs related to feeding difficulties in children with premature history. We investigated the prevalence of feeding problems in 2003-2006 using random subjects of 5% of the enrollees in the National Health Insurance program of Taiwan. Databases used in this study include "monthly claim summary for ambulatory care claims", "inpatient expenditures by admissions", "details of ambulatory care orders", etc. ICD-9 codes of preterm infants and related diagnoses were used as variables. According to this systematical sampling data, there is a rise in the percentage of children admitted to the hospital with low birth weight. High percentage of these children experienced feeding related issues/difficulties in their first 5 years of life. However, under-utilization of rehabilitation services were noted with these low birth weight children who had feeding problems. There is a high co-occurrence between low birth weight children and feeding problems in their first 5 years of life measured by frequency of hospital admissions or outpatient ambulatory care services requirement. It is recommended based on our results that children with low birth weight should be screened automatically for potential feeding difficulties during the hospital stay. Furthermore, feeding monitoring/management as well as parental education on potential risk for feeding difficulties should be incorporated in the early intervention program. (Contains 7 tables.)

The effects of early-life metal exposure on neurodevelopment in very low birth weight preterm (VLBMP) children (with a birth weight of <1500 g and a gestational age of <37 weeks) have not been clearly established. We aimed to investigate associations of childhood exposure to multiple metals and preterm low birth weight with neurodevelopment among children at 24 months of corrected age. VLBWP children (n = 65) and normal birth weight term (NBWT) children (n = 87) were enrolled from Mackay Memorial Hospital in Taiwan between December 2011 and April 2015. Lead (Pb), cadmium (Cd), arsenic (As), methylmercury (MeHg), and selenium (Se) concentrations in the hair and fingernails were analyzed as biomarkers for metal exposure. The Bayley Scale of Infant and Toddler Development, Third Edition, was used to determine neurodevelopment levels. VLBWP children had significantly lower scores in all development domains compared to NBWT children. We also investigated preliminary exposure levels of VLBWP children to metals as reference values for future epidemiological and clinical survey. Fingernails are a useful biomarker for metal exposure to evaluate the effects on neurological development. A multivariable regression analysis revealed that fingernail Cd concentrations were significantly negatively associated with cognition (β = −0.63, 95% confidence interval (CI): −1.17 to −0.08) and receptive language function (β = −0.43, 95% CI: −0.82 to −0.04) among VLBWP children. VLBWP children with a 10-μg/g increase in the As concentration in their nails had a 8.67-point lower composite score in cognitive ability and a 1.82-point lower score in gross-motor functions. Effects of preterm birth and postnatal exposure to Cd and As were associated with poorer cognitive, receptive language, and gross-motor abilities. VLBWP children are at risk for neurodevelopmental impairments when exposed to metals. Further large-scale studies are needed assess to the risk of neurodevelopmental impairments when vulnerable children are exposed to metal mixtures. [ABSTRACT FROM AUTHOR]

Ultrasound has been used to observe lung aeration and fluid clearance during the neonatal transition period, but there is no consensus regarding the optimal timing of lung ultrasound. We aimed to monitor the trend of the serial lung ultrasound score (LUS) and extended LUS (eLUS) throughout the neonatal transition period (≤1, 2, 4, 8, 24, and 48 h after birth), assess any correlation to the clinical presentation (using the Silverman Andersen Respiratory Severity Score (RSS)), and determine the optimal time of the ultrasound. We found both LUS and eLUS decreased significantly after 2 h of life and had similar statistical differences among the serial time points. Although both scores had a positive, moderate correlation to the RSS overall (Pearson correlation 0.499 [p < 0.001] between LUS and RSS, 0.504 [p < 0.001] between eLUS and RSS), the correlation was poor within 1 h of life (Pearson correlation 0.15 [p = 0.389] between LUS and RSS, 0.099 [p = 0.573] between eLUS and RSS). For better clinical correlation, the first lung ultrasound for the neonate may be performed at 2 h of life. Further research is warranted to explore the clinical value and limitations of earlier (≤1 h of life) lung ultrasound examinations. [ABSTRACT FROM AUTHOR]

Omphalocele and gastroschisis are the two most common congenital abdominal wall defects; however, no previous study has focused on gastrointestinal and hepatobiliary tract malformations in these two conditions. This study aimed to investigate the demographic characteristics, coexisting congenital gastrointestinal and hepatobiliary tract anomalies, hospital course, and outcomes of patients with gastroschisis and omphalocele. This is retrospective chart review of all patients admitted to one tertiary medical center in Taiwan between January 1, 2000 and June 30, 2020 with a diagnosis of gastroschisis or omphalocele. The medical records were reviewed to obtain demographic data regarding coexisting gastrointestinal and hepatobiliary tract anomalies and outcomes. Of the 51 patients included, 21 had gastroschisis and 30 had omphalocele. Gastroschisis was associated with a significantly younger maternal age and a higher incidence of small for gestational age. Of the 30 patients with omphalocele, twelve had associated gastrointestinal and hepatobiliary anomalies. Seven of the 21 patients with gastroschisis had gastrointestinal anomalies, and none had hepatobiliary anomalies. Among the omphalocele patients, three (10%) had documented malrotation, and one developed midgut volvulus. Among gastroschisis patients, four patients (19%) had malrotation, and two developed midgut volvulus. There were no statistically significant differences in postoperative complications or mortality rates between those with and without gastrointestinal/hepatobiliary tract anomalies. The diversity of coexisting gastrointestinal and hepatobiliary tract anomalies is higher in the omphalocele than in gastroschisis. In addition, we demonstrate that patients with gastroschisis or omphalocele have a higher rate of intestinal malrotation and midgut volvulus. [ABSTRACT FROM AUTHOR]

The present study included the first case of mucopolysaccharidosis (MPS) type VII in Taiwan. During pregnancy, the patient was diagnosed with hydrops fetalis and had ascites aspiration 4 times. In the following years, she presented gradually with chronic lung disease, developmental delay, short stature, dysmorphic features of coarse face, macroglossia and pigeon chest with scoliosis. Upon referral at age 4 years, she had corneal clouding, mild limitation of range of motion (ROM) and hepatosplenomegaly. X-ray showed paddle ribs and dysplastic vertebral bodies. MPS was suspected and urine glycosaminoglycans (GAGs) elevated were noted. The leukocyte enzymatic analyses for MPS I, MPS II, MPS IIIB, MPS IVA, and MPS VI were all normal. Afterward, the molecular analysis showed two heterozygous genetic variants of c.104C > A and c.1454C > T in trans in the GUSB gene (NM_000181.4) which were the causes for MPS VII. Then, we checked the leukocyte β-glucuronidase activity for MPS VII and showed extremely low, therefore confirmed the diagnosis. Clinicians should increase the awareness on the early signs of MPS to have a prompt diagnosis and offer the correct treatment like enzyme replacement therapy (ERT) as early as possible. [ABSTRACT FROM AUTHOR]

Nationwide group B Streptococcus agalactiae (GBS) antepartum screening was instituted in Taiwan in 2012. The impact of the policy on early-onset sepsis (EOS) has not been evaluated. This study aimed to examine the impact of the policy on the incidence of neonatal EOS. This was a retrospective study conducted at MacKay Children's Hospital. Patients with culture-proven neonatal EOS were enrolled and divided by birth year in relation to the implementation of GBS prevention policy: Epoch 1, 2001–2004 pre-GBS screening; Epoch 2, 2005–2011 elective GBS screening; and Epoch 3, 2012–2018 universal GBS screening. The pathogens and antimicrobial resistance patterns were reviewed and analyzed. The incidence was modeled using Poisson regression. A total of 128 neonates met the enrollment criteria. The observed incidence of EOS was 1.52‰. The incidence rates of EOS, GBS, and Escherichia coli (E. coli) sepsis were similar in Epoch 1 and Epoch 3. E. coli and non-Enterococcal group D Streptococcus (GDS) infection increased significantly in term infants, whereas the EOS-related mortality rate declined in preterm infants. Approximately 72% of the isolated E. coli were ampicillin-resistant, and the antimicrobial sensitivity remained unaltered during the studied period. The overall EOS incidence has not changed from 2001 to 2018. However, changes in the causative pathogens were observed in both term and preterm infants. Clinicians should be aware of this evolving epidemiology to provide prompt appropriate perinatal management. [ABSTRACT FROM AUTHOR]

Neurodevelopmental outcome in preterm infants has been of great importance in recent decades. We determined the prevalence of abnormal neurodevelopmental outcomes and associated risk factors of very-low-birth-weight (VLBW) preterm infants at 2 and 5 years of age. We conducted a population-based, prospective cohort study of VLBW preterm infants born between 2002 and 2009 in Taiwan. Sociodemographic, neonatal data, and neurological assessments at 2 and 5 years of age were obtained from the database of Taiwan Premature Infant Follow-up Network. Of the 6549 VLBW preterm infants included in the study, 5407 (82.56%) survived to discharge; 4105 and 1427 participated in follow-up assessments at age 2 and 5 years, respectively. At age 5 years, 76.87% (1097/1427), 12.05% (172/1427), and 8.76% (125/1427) of children had normal, borderline, and abnormal neurocognitive outcomes, separately. Among the enrolled children, 1385 were followed at both 2-year and 5-year-old. Among the 233 children with abnormal neurodevelopmental outcomes at age 5 years, nearly one-fifth (18.03%, 42/233) had normal or borderline neurodevelopmental outcomes at age 2 years. Among the 154 children with borderline neurodevelopmental outcomes at age 5 years, 71.43% (110/154) had normal or borderline neurodevelopmental outcomes at age 2 years. The risk factors significantly associated with disadvantageous (worsening or remaining unimproved) neurodevelopmental outcomes were lower gestational age, cystic periventricular leukomalacia, and paternal or maternal education ≤12 years. Almost one-fifth of VLBW preterm children with abnormal neurodevelopmental outcomes at age 5 years had normal or borderline neurologic and developmental assessments at age 2 years. For the high risk group such as VLBW preterm children, serial follow-up assessments beyond 2 years of age may be warranted and the eligibility of early intervention service should be revised by the government so proper and targeted intervention can be implemented at earlier age. [ABSTRACT FROM AUTHOR]

Objective: There is limited epidemiological data on the seasonality of respiratory syncytial virus (RSV) infection in subtropical climates, such as in Taiwan. This study aimed to assess RSV seasonality among children ≤24 months of age in Taiwan. We also assessed factors (gestational age [GA], chronologic age [CA], and bronchopulmonary dysplasia [BPD]) associated with RSV-associated hospitalization in preterm infants to confirm the appropriateness of the novel Taiwanese RSV prophylactic policy. Study Design: From January 2000 to August 2010, 3572 children aged ≤24-months were admitted to Taipei Mackay Memorial Hospital due to RSV infection. The monthly RSV-associated hospitalization rate among children aged ≤24 months was retrospectively reviewed. Among these children, 378 were born preterm. The associations between GA, CA, and BPD and the incidence of RSV-associated hospitalization in the preterm infants were assessed. Results: In children aged ≤24 months, the monthly distribution of RSV-associated hospitalization rates revealed a prolonged RSV season with a duration of 10 months. Infants with GAs ≤32 weeks and those who had BPD had the highest rates of RSV hospitalization (P<0.001). Preterm infants were most vulnerable to RSV infection within CA 9 months. Conclusions: Given that Taiwan has a prolonged (10-month) RSV season, the American Academy of Pediatrics' recommendations for RSV prophylaxis are not directly applicable. The current Taiwanese guidelines for RSV prophylaxis, which specify palivizumab injection (a total six doses until CA 8–9 months) for preterm infants (those born before 286/7 weeks GA or before 356/7 weeks GA with BPD), are appropriate. This prophylaxis strategy may be applicable to other countries/regions with subtropical climates. [ABSTRACT FROM AUTHOR]

Background: Respiratory syncytial virus (RSV) circulates year round in Taiwan. A novel six consecutive monthly doses of palivizumab for RSV prevention protocol has been approved for high risk preterm infants since December 2010. This study aimed to determine the clinical effectiveness and safety of this novel protocol for the prevention of RSV infection. Methods: From April 2011 to March 2013, we enrolled infants born at ≤28 weeks gestation and infants born at ≤35 weeks gestation with chronic lung disease (CLD) who received palivizumab prophylaxis as study group and followed up for 12 months. Historic control, those who were born and followed up between July 2000 and June 2008, were retrieved for propensity score matching. Primary endpoint was RSV-related hospitalization, and secondary endpoints included the length of hospital stay and intensive care unit (ICU) care. Results: We enrolled 127 infants (108 infants born at ≤28 weeks and 19 infants born at 29–35 weeks with CLD). They completed 6-dose palivizumab as scheduled. Among the study group, the RSV-related hospitalizations were 2 (1.6%) within 6 months and 5 (3.9%) within 12 months after discharge. We matched 127 infants in the control group with 127 infants in the study group by propensity score matching. The reduction of RSV-related hospitalization rates were 86% (10.2% vs 1.6%, p = 0.002) within 6 months after discharge and 78% (15.7% vs 3.9%, p = 0.004) within 12 months after discharge. Compared to the control group, the rate of ICU care significantly decreased from 7.1% to 0.8% (p = 0.024) within 6 months after discharge and from 7.9% to 0.8% (p = 0.014) within 12 months after discharge. Adverse events were recorded in 6.4% injections. Conclusions: Six monthly intramuscular administration of palivizumab is effective for prevention of RSV hospitalization in regions with no single seasonal peak of RSV infection such as Taiwan. [ABSTRACT FROM AUTHOR]

Background: The lack of good evidence for improved outcomes in children and young infants with febrile urinary tract infection (UTI) after aggressive treatment for vesicoureteral reflux (VUR) has raised doubts regarding the need for routine voiding cystourethrography (VCUG), and the appropriate imaging evaluation in these children remains controversial. Objectives: This prospective study aimed to determine whether abnormalities found on acute dimercaptosuccinic acid (DMSA) scan and ultrasound (US) can help indicate the necessity of voiding cystourethrography (VCUG) in young infants. Methods: For 3.5 years, all infants younger than 3 months presenting with first febrile UTI were prospectively studied. All infants were hospitalized and investigated using US (<3 days after admission), DMSA scan (<5 days after admission), and VCUG (7-10 days after antibiotic treatment) after diagnosis. The association among findings of US, DMSA scan, and VCUG were evaluated. Results: From 220 infants, there were abnormal results in 136 (61.8%) US and in 111 (50.5%) DMSA scans. By US, ten infants (4.5%) with abscess or structural abnormalities other than VUR were diagnosed. High-grade (III-V) VUR was present in 39 patients (17.7%). The sensitivities for high-grade VUR of renal US alone (76.9%) or DMSA scan alone (82.1%) were not as good as that of the 'OR rule' strategy, which had 92.3% sensitivity and 94.3% negative predictive value. Conclusions: To screen high-grade VUR in young infants with febrile UTI, US and acute DMSA scan could be performed first. VCUG is only indicated when abnormalities are apparent on either US or DMSA scan or both. [ABSTRACT FROM AUTHOR]

Background: There is no national data on group B streptococcus (GBS) infection in Taiwan. We investigated incidence of maternal GBS colonization and neonatal GBS infection rate and clinical pictures of neonatal GBS infection to estimate the value of intrapartum chemoprophylactic strategy in Taiwan. Methods: From January 2004 to June 2005, a prospective study to estimate maternal colonization rate by maternal rectovaginal culture at six hospitals was conducted. Neonatal GBS infection rate based on inborn infants was calculated retrospectively from January 2001 to June 2005; clinical pictures of infants diagnosed with invasive GBS disease were reviewed. Results: Maternal colonization rate of GBS was around 20% at hospital base, incidence of neonatal GBS infection was 1 per 1000 live births of infants born at hospitals. There were 221 infants with GBS infection: in 142, the disease occurred within 7 days of birth (early-onset disease, EOD), and in 79, it developed later (late-onset disease). Infantile EOD was more often seen in mothers with premature rupture of membrane, often accompanied by respiratory failure necessitating ventilator support. Infants with late-onset disease often manifested fever, leukopenia, thrombocytopenia, and meningitis. Fifteen infants died, mostly of EOD type (12 of 15). Risk factors of mortality included rescue at delivery room, leukopenia, thrombocytopenia, sepsis, respiratory distress, persistent hypertension of newborn, respiratory failure needing intensive respiratory support (intermittent mandatory ventilator and high frequency oscillatory ventilator), surfactant use, shock, and congenital heart diseases. Conclusions: We concluded that universal maternal rectovaginal culture of GBS with intrapartum antibiotic prophylaxis is an urgent call to reduce EOD and mortality because of GBS infection in neonates in Taiwan. [ABSTRACT FROM AUTHOR]

Background/Purpose: All children should have some developmental screening periodically throughout childhood, especially those who were born prematurely. There is limited information about the development of children with very low birth weight (VLBW; birth weight < 1500 g) beyond the preschool age in Taiwan. We evaluated intelligence quotient (IQ) and cognitive ability of prematurely born school-aged children in Taiwan. Methods: This was a multicenter study of VLBW and full-term children born between 1995 and 1997 at four hospitals in northern Taiwan. We used the Wechsler Intelligence Scale for Children, 3rd Edition (WISC-III), to assess these children. Demographic data were recorded including maternal and paternal age, education, birth weight, gestational age, and gender. Results: A total of 189 children (130 with VLBW born prematurely and 59 born at full term) were recruited. There were significant differences in performance IQ (PIQ; 90.16 ± 17.05 vs. 108.51 ± 15.65, p < 0.001), verbal IQ (VIQ; 97.43 ± 15.62 vs. 111.78 ± 13.65, p < 0.001), full-scale IQ (FSIQ; 93.14 ± 16.33 vs. 111.05 ± 14.81, p < 0.001), verbal comprehension index score (VCIS; 98.06 ± 15.53 vs. 112.47 ± 13.74, p < 0.001), perceptional organization index score (POIS; 92.39 ± 17.13 vs. 109.42 ± 14.87, p < 0.001) and freedom from distractibility index score (FDIS; 98.34 ± 17.71 vs. 110.53 ± 10.94, p = 0.008). There was no correlation between perinatal outcomes and FSIQ. Conclusion: Our results suggest that VLBW children have significantly lower PIQ, VIQ, FSIQ, VCIS, POIS and FDIS at primary school compared with full-term children. [Copyright &y& Elsevier]

Presents reports of various case studies conducted on congenital nasal pyriform aperture stenosis in Taiwan. Birth history and observations of the physical examination done on a four-month old boy; Analysis of the medical complications found in a 12-day old female baby at a hospital in Taiwan; Diagnosis of a six-day old boy hopitalized because of dyspnea; Discussion on the symptoms, diagnosis and treatment of the disease.

Background: Galloway-Mowat syndrome (GAMOS) is a rare autosomal recessive disease characterized by the combination of glomerulopathy with early-onset nephrotic syndrome and microcephaly with central nervous system anomalies. Given its clinical heterogeneity, GAMOS is believed to be a genetically heterogenous group of disorders. Recently, it has been reported that mutations in KEOPS-encoding genes, including the OSGEP gene, were responsible for GAMOS.Results: Overall, 6 patients from 5 different Taiwanese families were included in our study; the patients had an identical OSGEP gene mutation (c.740G > A transition) and all exhibited a uniform clinical phenotype with early-onset nephrotic syndrome, craniofacial and skeletal dysmorphism, primary microcephaly with pachygyria, and death before 2 years of age. We reviewed their clinical manifestations, the prenatal and postnatal presentations and ultrasound findings, results of imaging studies, associated anomalies, and outcome on follow-up. All individuals were found to have an "aged face" comprising peculiar facial dysmorphisms. Arachnodactyly or camptodactyly were noted in all patients. Neurological findings consisted of microcephaly, hypotonia, developmental delay, and seizures. Brain imaging studies all showed pachygyria and hypomyelination. All patients developed early-onset nephrotic syndrome. The proteinuria was steroid-resistant and eventually resulted in renal function impairment. Prenatal ultrasound findings included microcephaly, intrauterine growth restriction, and oligohydramnios. Fetal MRI in 2 patients confirmed the gyral and myelin abnormalities.Conclusions: Our study suggests that a careful review of the facial features can provide useful clues for an early and accurate diagnosis. Prenatal ultrasound findings, fetal MRI, genetic counseling, and mutation analysis may be useful for an early prenatal diagnosis. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the association of mode of delivery (MOD) with short‐term and neurodevelopmental outcomes at 2 years of corrected age (CA) in periviable singleton infants. Methods: This retrospective cohort study of the Taiwan Premature Infant Follow‐up Network database between 2010 and 2016 compared non‐anomalous singleton deliveries (cesarean delivery [CD] vs vaginal delivery [VD]) between 22 0/7 and 25 6/7 gestational weeks. Major morbidities, mortality, and neurodevelopmental outcomes were evaluated at 2‐year CA. Results: The CD and VD groups included 354 and 472 infants, respectively. The intraventricular hemorrhage (IVH) rate was lower in the CD group (54% vs 66%, P = 0.001), but severe IVH differed non‐significantly between groups (20% vs 26%, P = 0.057). In the small‐for‐gestational age subgroup, CD was associated with lower IVH (56% vs 84%, adjusted odds ratio [aOR] 0.17, 95% confidence interval [CI] 0.04–0.69) and better survival without neurodevelopmental impairment (29% vs 8%, aOR, 6.64, 95% CI 1.02–43.29) after controlling for potential confounders. Conclusion: The optimal MOD for periviable singleton birth and its impact are unclear. CD in periviable singleton births is associated with a decreased IVH risk, without improvement in severe IVH, mortality, or neurodevelopment at 2‐year CA. The small‐for‐gestational age subgroup may benefit from CD for better survival without neurodevelopmental impairment. Synopsis: Cesarean delivery in periviable singleton small‐for‐gestational‐age births is associated with better survival without neurodevelopmental impairment. [ABSTRACT FROM AUTHOR]

Preterm infants are at risk for emotional difficulties and behavioral problems. This study was aimed to investigate the effects of a clinic-based intervention program (CBIP) and a home-based intervention program (HBIP) compared with a usual care program (UCP) on emotion regulation to stress in preterm infants with very low birth weight (VLBW, birth weight <1500 g). A total of 178 VLBW preterm infants had been previously randomly assigned to receive one of three interventions (57 in CBIP, 63 in HBIP and 58 in UCP). The CBIP and HBIP contained identical child-, parent- and dyad-focused interventions that were provided to infants during hospitalization and were respectively delivered at clinics and at home at PMA 36-38 and 40 weeks, and 1, 2, 4, 6, 9, 12 months of corrected age. All infants were prospectively observed for behavioral reactivity and regulation in response to experimentally evoke stress evoked by a toy-behind-barrier procedure at 12, 18, and 24 months of corrected age. Their cognitive and language abilities, and mothers' responsiveness were also assessed at 12 months as potential covariates. Compared to the UCP-group infants, the HBIP-group infants exhibited shorter durations of visual orientation to a toy (adjusted difference [95% CI]=-1.60 [-3.07 to -0.13], p=0.03), and the CBIP-group infants exhibited shorter durations of avoidance (adjusted difference [95% CI]=-0.84 [-1.57 to -0.10], p=0.03) from 12 to 24 months of corrected age. The CBIP and HBIP showed no difference in the stress reactivity from the UCP, however. These results suggest that comprehensive interventions incorporating child-, parent- and dyad-focused services enhanced VLBW preterm infants' emotion regulation in response to stress at toddler age. [ABSTRACT FROM AUTHOR]

Highlights: [•] The BSID-II and Bayley-III were concurrently administered in this study. [•] The Bayley-III was reliable and generally contained similar constructs as the BSID-II. [•] The Bayley-III overestimated the developmental performance of Taiwanese infants. [•] Upward adjustments of the Bayley-III scores for definition of developmental delay were suggested. [Copyright &y& Elsevier]

Abstract: Introduction: Intervention studies of developmental care for preterm infants in Western societies have shown early but unsustainable effects on child outcomes, however only a limited of studies have examined if developmental care interventions produce similar effects in Eastern cultural contexts. Aims: To examine the effectiveness of in-hospital developmental care on neonatal morbidity, growth and development of preterm infants with very low birth weight (VLBW; birth weight<1500g) in Taiwan. Methods: One hundred and seventy-eight VLBW preterm infants were randomly assigned to the clinical trial during hospitalization at three hospitals in Taiwan; the control group received five sessions of standard child-focused developmental care and the intervention group received five sessions of child- and parent-focused developmental care. Sixty-two normal term infants were also included as a comparison group. Infants were examined for morbidity, growth and developmental outcomes at term age. Results: At study entry, more infants in the intervention group were twins or multiples than those in the control group (29% vs. 16%, p =0.05). After adjusting for birth set, the intervention group had lower incidences of stage II–III retinopathy (odds ratio [OR]=0.34 [95% confidence interval (CI): 0.15–0.79]; p =0.01) and feeding desaturation (OR=0.32 [95% CI: 0.10–1.00]; p =0.05) and had greater daily weight gains (difference=2.0g/day [95% CI: 0–4.0g/day]; p =0.05) as compared with the control group. However, the intervention and control groups did not differ in any of the neurodevelopmental measures. Conclusions: In-hospital developmental care has short-term benefits for Taiwanese VLBW preterm infants in reducing the risk of retinopathy and feeding desaturation as well as in enhancing weight gains at term age. [Copyright &y& Elsevier]

Summary: Objectives: Listeria monocytogenes is an important pathogen in neonates in Western countries, with a fatality rate of 20–30%. There is limited information on neonatal listeriosis in Eastern countries. The purpose of this study was to delineate the occurrence and clinical picture of neonatal listeriosis in Taiwan. Methods: A questionnaire-based survey of all of the 17 medical centers in Taiwan was performed, and a literature review of neonatal listeriosis as reported in Taiwan from 1990 to 2007 was made. Results: A total of 14 cases (10 male, four female) of neonatal listeriosis were identified, including 11 found from the survey of four medical centers and another three collected from the literature review. Three were found to have occurred prior to 2000 and 11 were found to have occurred after 2000. The age of onset was less than 3 days in all cases. L. monocytogenes was identified from blood in 13, cerebrospinal fluid in four, and gastric aspirate in two. Half of the cases (7/14) had involvement of the central nervous system with pleocytosis and hypoglycorrhachia in cerebrospinal fluid, and three of them even developed hydrocephalus. The mortality rate was 29%. Conclusions: Our findings suggest that listeriosis may emerge as an important health threat among newborn infants in Taiwan. [Copyright &y& Elsevier]

Background: Neonatal listeriosis is a major cause of mortality in newborn; however, there is limited information about this disease in Taiwan. The aim of our study was to identify the outcome determinants, clinical features, and incidence of pregnancy-associated listeriosis, which includes both neonatal and maternal listeriosis., Methods: We retrospectively analyzed the medical records of neonatal and maternal patients with pregnancy-associated listeriosis at two hospitals in Taiwan from January 2000 to December 2018. Listeriosis was indicated by positive Listeria monocytogenes culture., Results: Our study examined 18 neonates and 19 mothers. The neonatal and fetal death rate was 24%. All five cases of fetal losses or neonatal deaths occurred before 29 weeks of gestational age. The annual incidence of confirmed neonatal listeriosis increased significantly from 0.94/10,000 neonatal inpatients in 2000-2011 to 5.45/10,000 neonatal inpatients in 2012-2018 (p = 0.026). Clinical presentations of neonatal listeriosis included respiratory distress (85%), leukocytosis or leukopenia (77%), bandemia (69%), thrombocytopenia (77%), hypocalcemia (100%) and elevated C-reactive protein (CRP) levels (92%). Lower gestation correlated with a higher fatality rate (p = 0.002). Among the maternal cases investigated, 67% had a diagnosis of listeriosis, and 72% presented with fever. However, only 21% of the 19 mothers received complete antepartum ampicillin treatment., Conclusions: The incidence of neonatal listeriosis is increasing, especially in preterm neonates. Maternal listeriosis should be adequately treated with appropriate empirical antibiotics., (Copyright © 2019. Published by Elsevier B.V.)

To determine the natural history of patient with bilateral vocal fold immobility (BVFI), and to identify the factors or predictors associated with the tracheostomy and duration of cannulation for those who require tracheostomy.A retrospective review was carried out at a single tertiary referral center over a 23-year period of infants less than 1 year old who presented with BVFI. All information related to sex, etiology, gestational age at birth, vocal fold (VF) position at diagnosis, presence of concomitant airway disease, age at attainment of VF movement, age at decannulation, and current tracheostomy status were recorded to perform descriptive and comparative analyses.Forty-one patients were enrolled, and the tracheostomy rate was 80.5% (33/41). Those with a median VF position had a higher rate of tracheostomy in comparison with those with a paramedian VF position (P = .003). Among patients who underwent tracheostomy, 77.4% (24/31) were eventually decannulated. The median duration of cannulation was 4.4 years (range: 0.8 - 10.7 years). Those who were found to have attainment of VF movement at less than 1-year-old had a shorter duration of cannulation than others (mean: 1.9 years vs 5.2 years, P < .001). The mortality rate of those patients who received tracheostomy was 9.7% (3/31).Although a high tracheostomy rate was found in BVFI patients, most of them underwent decannulation. The earlier attainment of VF movement is achieved, the shorter duration of cannulation is required. This information is important for physicians while following up BVFI patients who have undergone tracheostomy.

VACTERL association is a non-random association of birth defects, which may include anomalies of the vertebral column, limbs, kidneys, and heart; anal atresia; tracheoesophageal fistula; and esophageal atresia. The presence of two or more of the defects establishes the diagnosis. The aim of our study is to describe the functional independence of children with VACTERL association and compare the results to unaffected children. These results will enable clinicians to provide more realistic prognostic information to parents and families. We used the WeeFIM questionnaire to assess the functional skills of 23 patients who had been diagnosed with VACTERL association at Mackay Memorial Hospital, Taipei, Taiwan, from June 1994 to June 2009. The total WeeFIM scores and sub-scores for three domains (self-care, mobility, and cognition) correlated significantly with age (P < 0.01). The scores were generally within the same range as those of unaffected Chinese children, although our subjects had slightly inferior scores on six items, including bowel, chair transfer, stairs, expression, social interaction, and problem solving. In conclusion, the daily functional skills of Taiwanese children with VACTERL association were similar to those of unaffected children., (Copyright © 2012 Wiley Periodicals, Inc.)

Background/purpose: Acute otitis media (AOM) is one of the most common diseases in children. Here, we describe the epidemiological and microbiological characteristics of AOM in Taiwanese children over a 10-year period., Methods: We retrospectively enrolled pediatric patients with culture-proven AOM who were treated at Mackay Memorial Hospital, Taipei between 1999-2008. The data include demographic characteristics, clinical history, and microbiological characteristics., Results: Six hundred and fourteen patients were included. The male:female ratio was 1.4 (p<0.001). Greater than three-fourths of the patients (476 [77.5%]) were < 5 years of age, and most patients were 1-2 years of age. The most common isolated pathogen was Streptococcus pneumoniae (419 patients [68.2%]), followed by nontypeable Haemophilus influenzae (NTHi; 118 patients [19.2%]). The distributions of age, gender, use of tympanocentesis, history of previous AOM, and use of antibiotic between patients infected with the two pathogens were not significantly different. However, the number of patients with AOM caused by S. pneumoniae, but not NTHi, decreased during the study period (p=0.004). Three hundred and eighty-seven children (63.0%) with AOM developed spontaneous otorrhea. Compared with patients who underwent tympanocentesis, those with spontaneous otorrhea were younger (27.0±16.4 vs. 31.1±15.2 months of age, p=0.004), more likely to have a previous history of AOM (p=0.019), and more likely to receive more antibiotics (p=0.012). The third most common pathogen was S. pyogenes (25 patients [4.1%]). S. pyogenes occurred more often in children > 5 years of age and was associated with spontaneous otorrhea (p<0.001)., Conclusion: S. pneumoniae and NTHi are common causes of culture-confirmed AOM in Taiwanese children. Although S. pyogenes is not as common, it usually causes AOM in children > 5 years of age and is associated with spontaneous otorrhea., (Copyright © 2012. Published by Elsevier B.V.)

Background: Early-onset sepsis (EOS) is the major cause of neonatal morbidity and mortality. Maternal group B Streptococcus (GBS) screening and intrapartum antibiotic prophylaxis (IAP) were implemented in our hospital in 2004. Our aim was to evaluate the effectiveness of the program and changes in pathogens and antibiotic susceptibility., Methods: The medical charts of mothers and infants with EOS between January 2001 and November 2008 were retrospectively reviewed. EOS was defined as sepsis occurring within 72 hours of birth. Data were pooled and compared for January 2001 through September 2004 (Period 1, without GBS screening) and October 2004 through November 2008 (Period 2, with GBS screening and IAP)., Results: The GBS screening rate increased from 10.11% in 2004 to 65% in 2008 and the IAP rate increased from 40% in 2004 to 90% in 2008. The most common EOS pathogen in Period 1 was GBS (45.4%), which decreased to 20% in Period 2 (p=0.081; trend p=0.009). The percentage of EOS because of Escherichia coli in Period 1 was 40.9% but increased to 70% in Period 2 (p=0.059). E coli EOS increased in extremely low birth weight premature babies weighing 500-1000g from Period 1 to Period 2 (p=0.031). The incidence of ampicillin-resistant E coli EOS was relatively high, but no significant change (88.9% vs. 92.9%) after implementation of GBS screening and IAP was noted., Conclusion: GBS screening plus IAP is effective in decreasing the incidence of GBS EOS; however, an increase in EOS caused by E coli was noted. Monitoring of pathogens causing EOS is important for effective treatment., (Copyright © 2011. Published by Elsevier B.V.)

Aim: The purpose of this study was to explore the paternal role of fathers of very low birth weight infants during their first year of life., Background: Following birth, very low birth weight infants often require extended hospitalisation and long-term care. The birth experience and the infant's hospitalisation often overwhelm parents and may hinder the development of their parental roles. The father is known to be an important supporting figure for the mother, but most studies in Taiwan have focussed on mothers., Design: A qualitative design using a grounded theory approach was employed in this study., Methods: In-depth interviews were conducted with a purposive sample of fathers during home visits. Each father was interviewed twice at one to three months and seven to nine months after his very low birth weight infant's discharge from the hospital. Data were analysed using constant comparison and repeated verification., Results: Twelve fathers were included in the study. During the first year of life of their very low birth weight infants, the fathers experienced 'protecting the wife-baby dyad', 'concentrating on the child's health and growth', and 'possessing a complete family'. Subcategories within each major category also emerged., Conclusion: In this study, Taiwanese fathers of very low birth weight infants learned different and broader dimensions of the paternal role and continually made adjustments to assure their family's integrity., Relevance to Clinical Practice: These findings can help nurses understand paternal perceptions and behaviours related to having a very low birth weight infant and in turn help fathers adjust to their role.

Background: Bacterial tracheitis may cause life-threatening airway obstruction., Methods: Records of patients admitted to the pediatric wards of Mackay Memorial Hospital between 1994 and 2005 with a diagnosis of bacterial tracheitis made on bronchoscopic visualization of thick membranous tracheal secretions were retrospectively reviewed., Results: A total of 40 patients (aged 1 month-8 years, 29 [73%] under 3 years old) were included. Cough, fever, dyspnea, and hoarseness were the commonest symptoms. Fourteen patients (21%) required intubation. The most frequently isolated bacteriae were alpha-hemolytic streptococcus (in 11, 38%), pseudomonas (5, 17%), and Staphylococcus aureus (4, 14%). Intubation was more frequent in patients seen between 1994 and 1999 compared with those seen later (8/12 early vs 9/28 late). In the early period alpha-hemolytic streptococcus (55%) and pseudomonas (36%) were isolated. In the later period the most frequently isolated bacteria was alpha-hemolytic streptococcus (28%), followed by S. aureus (22%). No patients died, but those with pseudomonas infection had more severe complications, including tracheal stenosis. The average hospital stay in the early period was 26.2 +/- 20.5 days versus 9.1 +/- 4.8 days in the late period. The corresponding lengths of stay in the intensive care unit were 10.5 +/- 11.5 days and 2.0 +/- 2.2 days., Conclusions: Bacterial tracheitis requiring hospitalization of children appeared to be milder in the second half of the study period. Pseudomonas tracheitis tends to have a severe course.

Objective: The goal was to investigate the efficacy of orally administered probiotics in preventing necrotizing enterocolitis for very low birth weight preterm infants., Methods: A prospective, blinded, randomized, multicenter controlled trial was conducted at 7 NICUs in Taiwan, to evaluate the beneficial effects of probiotics in necrotizing enterocolitis among very low birth weight infants (birth weight: <1500 g). Very low birth weight infants who survived to start enteral feeding were eligible and were assigned randomly to 2 groups after parental informed consent was obtained. Infants in the study group were given Bifidobacterium bifidum and Lactobacillus acidophilus, added to breast milk or mixed feeding (breast milk and formula), twice daily for 6 weeks. Infants in the control group were fed with breast milk or mixed feeding. The clinicians caring for the infants were blinded to the group assignment. The primary outcome measurement was death or necrotizing enterocolitis (Bell's stage >or=2)., Results: Four hundred thirty-four infants were enrolled, 217 in the study group and 217 in the control group. The incidence of death or necrotizing enterocolitis (stage >or=2) was significantly lower in the study group (4 of 217 infants vs 20 of 217 infants). The incidence of necrotizing enterocolitis (stage >or=2) was lower in the study group, compared with the control group (4 of 217 infants vs 14 of 217 infants). No adverse effect, such as sepsis, flatulence, or diarrhea, was noted., Conclusion: Probiotics, in the form of Bifidobacterium and Lactobacillus, fed enterally to very low birth weight preterm infants for 6 weeks reduced the incidence of death or necrotizing enterocolitis.

Introduction: Kawasaki disease (KD) is an acute febrile vasculitis of unknown etiology that mainly occurs in infants and children. Clinical and histopathologic findings suggest that vascular endothelial growth factor (VEGF) is involved in the coronary artery lesions (CALs) development in KD. This study hypothesized that specific VEGF gene polymorphisms and their haplotypes are associated with KD susceptibility and CAL development in Taiwanese children., Subjects and Methods: The VEGF -2578 A/C, -634 G/C, and +936 C/T single-nucleotide polymorphisms (SNPs) were genotyped in 156 children with KD and 672 ethnically matched healthy controls using the Pre-Developed TaqMan Allelic Discrimination Assay., Results: No significant differences in genotype, allele, carrier, and haplotype frequencies of the three SNPs were found between healthy controls and children with KD or between patients with and without CAL., Conclusion: Our data suggest that VEGF -2578 A/C, -634 G/C, and +936 C/T SNPs do not confer increased susceptibility to KD or to CAL development.

Objectives: To establish the normative data of the Bayley Scales of Infant Development-Second Edition (BSID-II) on Taiwanese infants from age 6 to 24 months and to explore the factors that relate to their mental and motor development., Methods: Five hundred and seven Taiwanese full-term infants were prospectively examined with the BSID-II at 6, 12, 18, and 24 months of age., Results: Taiwanese infants' Bayley mental and motor raw scores were lower than the United States norms from age 6 to 24 months, however, the discrepancy gradually declined with increasing age. Gender, intrauterine growth status, birth order, region of residence, maternal education, and paternal occupation were shown to have longitudinal associations with their mental and/or motor scores., Conclusions: Differences existed in the mental and motor development among Taiwanese and American infants. Our preliminary norms of the BSID-II may be more appropriate than the United States norms for Taiwanese children.

Although some previous studies have reported that genetic and immunologic factors play important roles in the pathogenesis of Kawasaki disease (KD), the etiologic factors of this enigmatical pediatric disease are still poorly understood. This study aims to investigate whether polymorphisms of the interleukin-4 gene (IL-4; -590 C/T in the promoter region and 8375 A/G in intron 3) are associated with KD and the development of coronary artery lesions (CALs) in Taiwanese children. Genomic DNA was extracted from whole-blood samples from 150 children with KD and 472 ethnically matched healthy control subjects. The IL-4 -590 C/T and 8375 A/G single nucleotide polymorphisms (SNPs) were genotyped by a real-time polymerase chain reaction system with the Pre-Developed TaqMan Allelic Discrimination Assay. No significant associations between IL-4 SNPs and susceptibility of KD with CALs were found. In addition, no evidence for associations between IL-4 SNPs and CAL development was found. These results suggest that IL-4 -590 C/T and 8375 A/G SNPs do not confer a relevant role in the susceptibility or CAL development of KD in Taiwanese children.

Background: Neonatal subgaleal hemorrhage (SGH) is a rare but potentially lethal medical emergency. The objective of the present retrospective study was to clarify the clinical presentation, management, and factors associated with morbidity and mortality., Methods: The records of all newborns from January 1995 to December 2004 were reviewed for evidence of SGH, based on the presence of a fluctuating hemorrhagic mass that crossed suture lines and extended toward the neck. Characteristics were compared between those with a poor or a good outcome., Results: Forty-two newborns with SGH were identified, 77% having had an instrumental delivery. The incidence was 0.6/1000 deliveries and 4.6/1000 vacuum-assisted deliveries. Thirteen patients (31%) had a poor outcome (five died, four had epilepsy, three with severe auditory dysfunction, two with cerebral palsy, and one with renal vein thrombosis). The group with the poor outcome had significantly more patients who had been transferred from other hospitals (P < 0.001). Those with a poor outcome had significantly more hypotension (P < 0.001) and seizures (P < 0.05). Laboratory findings associated with a poor outcome included anemia, coagulopathy, metabolic acidosis, and renal impairment. Other predictors of a poor prognosis included skull bone fracture, the need for pressors, blood transfusion, ventilator support, intensive care unit admission, and a longer hospital stay., Conclusions: SGH may be associated with serious complications and death. The condition must be recognized promptly and monitored closely. Those with poor prognostic factors should be referred for intensive care, treated aggressively, and followed in the long term.

Background: Prader-Willi syndrome (PWS) is a congenital disorder caused by absent expression of paternal genes in 15q11-13 affecting multiple systems. The information concerning the clinical features of this genetic disorder is incomplete in Taiwan., Methods: A retrospective analysis was carried out of 70 PWS patients (39 male, 31 females; age range, 1 month-22 years) seen in four major medical centers in Taiwan from January 1980 through June 2005. All cases were confirmed by methylation-specific polymerase chain reaction. The molecular characteristics, birth history, clinical presentation and laboratory studies were analyzed., Results: Complete genetic analysis was performed in 52 of the 70 patients with PWS. The abnormalities found included deletions in 45 (87%), maternal uniparental disomy (UPD) in five (10%), and a probable imprinting center deletion or an imprinting defect in two (4%). The average weight of the patients at birth was 2588 +/- 540 g. Bone age delay of >2 years and growth hormone (GH) deficiency were noted in 11/40 (28%) and 12/20 (60%), respectively. In the 18 in whom both bone age and GH were assessed, abnormalities of both were found in two (11%). In 2000, Taiwan instituted the Rare Diseases and Orphan Drugs Act and mandated a three-phase screening protocol for PWS. Of the 41 patients diagnosed with PWS before 2000, only four (10%) were diagnosed before the age of 3 months; in the 29 patients diagnosed after 2000, in 15 (52%) the syndrome was confirmed before 3 months of age (P < 0.001)., Conclusions: The present finding is in contrast to that of most previous reports that indicated a higher incidence of UPD in PWS. It is unclear whether this discrepancy in the incidence of UPD arises from under-diagnosis or because of ethnic differences, a question worthy of further study. The three-phase screening protocol has generated notable improvement in the early diagnosis of PWS in Taiwan.

Background: This study investigated the survival and natural history of trisomy 13 in a series of patients, comparing the management and outcome before and after the implementation of Taiwan's National Health Insurance program (NHI)., Methods: A total of 28 cases of trisomy 13 seen at Mackay Memorial Hospital, Taipei, Taiwan, from 1985 to 2004 were retrospectively reviewed. Survival and management before (12 cases) and after (16 cases) the implementation of National Health Insurance were compared, and structural defects, imaging findings, and cytogenetic results were analyzed. The cases that were diagnosed prenatally, and finally terminated, were excluded from this study. The diagnosis of trisomy 13 was based on the postnatal chromosome analysis., Results: All patients except one with trisomy 13 translocation died in their first year because of severe malformations of the cardiovascular or central nervous system. The median survival was 9 days. After implementation of National Health Insurance, survival with trisomy 13 was significantly longer than before (P < 0.05). The three most common structural defects were abnormal auricular helices or low-set ears (89%), cryptorchidism and abnormal scrotum of male (73%) and cleft lip and/or palate (71%). Using echocardiography, the most commonly detected heart defects were patent ductus arteriosus (68%), ventricular septal defect (50%) and atrial septal defect (50%), and eight cases (36%) had complex congenital heart defects. The most common brain lesion was lenticulostriate vasculopathy (22%), followed by holoprosencephaly (17%), brain edema (13%) and subependymal cyst (13%)., Conclusions: Early diagnosis and the survival patterns from the data collected should be used to inform parents and health-care professionals to assist in decision making. Although most patients with trisomy 13 die within the first weeks after birth, it is important to recognize that a few may survive the first year. When counseling families, the long-term survival prospects of trisomy 13 patients should be included.

Although some previous studies have reported that genetic and immunologic factors play important roles in the pathogenesis of Kawasaki disease (KD), the etiologic factors of this enigmatic pediatric disease are still poorly understood. The purpose of this study was to investigate whether polymorphisms of the human leukocyte antigen DRB1 (HLA-DRB1) gene are associated with KD and the development of coronary artery lesions (CAL) in Taiwanese children. Genomic DNA was extracted from whole blood samples from 145 children with KD and 331 healthy controls. The HLA-DRB1 gene was genotyped by polymerase chain reaction (PCR) and sequence-based typing assays. We found that the distribution of HLA-DRB1 allele families and alleles in children with KD did not differ from that in healthy controls. Stratified analysis did not demonstrate any association between particular HLA-DRB1 allele families or alleles and the development of CAL in children with KD. These findings suggest that susceptibility to KD and CAL is not associated with the HLA-DRB1 gene in a Taiwanese population. If immunogenetic determinants are involved in this disease and its complications in Taiwanese children, they must involve genes other than HLA-DRB1.

Background: The objective of the present retrospective study was to clarify the clinical course, major pathogens, and other infections associated with infected cephalohematoma., Methods: From January 1978 to December 2003, records of all newborns were reviewed for evidence of an infected cephalohematoma, based either on local signs of infection or a diagnostic tap that yielded pus. Patients were divided into two groups: those seen in the early period (1978-1990) and in the late period (1991-2003)., Results: Twenty-eight newborns with infected cephalohematoma were identified, 14 each in the early and late periods. The mean age at onset was 17.8 +/- 13.9 days. The most common local findings were erythema (79%), increasing size of the hematoma (68%), and a fluctuant mass (46%), while fever (64%), poor appetite (39%) and irritability (18%) were the common systemic signs. Common laboratory findings included leukocytosis (82%) and an elevated C-reactive protein (61%). Escherichia coli was the most common pathogen (16 patients, 57%) and was isolated significantly more frequently in the late period (early period: 36%, late period: 79%, P < 0.05). Staphylococcus aureus was the second common pathogen (five patients, 18%). Ten patients (36%) had other associated infection: eight had sepsis, three had meningitis, and one had osteomyelitis. In the early period three patients (11%) died., Conclusions: Clinicians should be aware that cephalohematoma is a potential site of infection. The incidence of associated system infection is high and may result in mortality. Appropriate diagnostic and therapeutic measures should be undertaken promptly if there are infectious signs.

Neonatal sepsis is a major cause of death in newborns despite sophisticated neonatal intensive care. This retrospective study reviewed the clinical characteristics of cases of culture-proven sepsis in a neonatal intensive care unit from January 1992 to December 2001. Patients were divided into those with onset of sepsis in the first 7 days of life (early-onset group) and those with onset after the seventh day of life (late-onset group). A total of 270 cases with 325 episodes of sepsis and 353 isolated pathogens were identified and included in the study. The male-to-female ratio was 1.4. The majority of cases of sepsis occurred in low birth weight (75.9%) and premature babies (76.7%). Late onset occurred in 71.9% of cases. Patients with late onset had a lower mortality rate than those with early onset (11.3% vs 28.9%). Coagulase-negative staphylococci (20.1%) was the most common organism isolated, but infection with Pseudomonas aeruginosa was associated with the highest morality rate (55.0%). Late-onset sepsis was significantly more common in very low birth weight and premature infants. The most frequently encountered pathogens in the early-onset group were group B streptococci (GBS) and Escherichia coli, while in the late-onset group, the organisms were coagulase-negative staphylococci and Enterobacteriaceae, including E. coli, Klebsiella pneumoniae, and Acinetobacter baumannii. GBS infection resulted in the highest mortality when the onset of sepsis was within the first 24 hours of life.