[Show abstract][Hide abstract] ABSTRACT: 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.
PLoS ONE 01/2014; 9(6):e100981. · 3.53 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Background
This study compared the current trend in survival rates and morbidity for very low birth weight (VLBW) infants in five Medical Training Centers of Prematurity for the Premature Baby Foundation of Taiwan (PBFT), with the outcomes from the USA, National Institute of Child Health and Human Development Neonatal Research Network (NICHD NRN), the Canadian Neonatal Network (CNN), and the Neonatal Research Network of Japan (NRNJ).
The survival rates of VLBW infants according to gestational age (GA) and major morbidities were compared between networks (Taiwan, USA, Canada, and Japan). Taiwanese data for VLBW infants of GA ≤28 weeks between 2007 and 2012 were obtained from the “PBFT Annual Conferences of Premature Care” reports defining survival rate as neonates that survived to the time of discharge. Major morbidities included severe neurological injury (Grade 3 or 4 intraventricular hemorrhage or periventricular leukomalacia), bronchopulmonary dysplasia, severe retinopathy of prematurity, necrotizing enterocolitis, late-onset sepsis, and patent ductus arteriosus.
The survival rates of VLBW infants of GA ≤28 weeks from the PBFT (Taiwan), NICHD NRN (USA), CNN (Canada), and NRNJ (Japan) were 77% (1323/1718), 72% (6859/9575), 82% (2353/2872), and 89% (4489/5069), respectively. The annual survival rates in Taiwan from 2007 to 2012 were 72%, 76%, 76%, 74%, 77%, and 78%, respectively. When GA from ≤23 weeks to 28 weeks was assessed in Taiwan, the survival rates of VLBW infants according to each week were 22%, 50%, 70%, 80%, 88%, and 92%, respectively. The survival rate, especially at lower GAs, was highest in the NRNJ (Japan). The major difference between Taiwan and Japan was attributed to the lower survival rates at lower GA (≤26 weeks) in Taiwan. Japan had the lowest rates of major morbidities among the four countries.
The survival rate of VLBW infants has improved over the past 6 years in Taiwan. It is higher than the USA, but lower than Canada and Japan. However, the results from Taiwan are from five Medical Training Centers for the PBFT rather than from a population-based study. It is crucial to have a nationwide neonatal research network to develop new practical approaches for VLBW infants in Taiwan.
[Show abstract][Hide abstract] ABSTRACT: We describe a neonate with VACTERL association and right-sided congenital diaphragmatic hernia (CDH). Such coexistence is rare. The lack of symptoms during the early neonatal period, the absence of bowel loops herniated into the right thoracic cavity, and an unfinished surgery led to clinical and radiological diagnostic difficulties. Respiratory distress occurred when the patient was 2 months old. Chest radiology plain film revealed typical findings of right-sided CDH. The diagnosis was confirmed after surgical exploration.
[Show abstract][Hide abstract] ABSTRACT: The objective of this study was to examine the relationships of Doppler cerebral blood flow velocity (CBFV) asymmetry measures with developmental outcomes in term infants. Doppler CBFV parameters (peak systolic velocity [PSV] and mean velocity [MV]) of the bilateral middle cerebral arteries of 52 healthy term infants were prospectively examined on postnatal days 1-5, and then their motor, cognitive and language development was evaluated with the Bayley Scales of Infant and Toddler Development, Third Edition, at 6, 12, 18 and 24 months of age. The left CBFV asymmetry measure (PSV or MV) was calculated by subtracting the right-side value from the left-side value. Left CBFV asymmetry measures were significantly positively related to motor scores at 6 (r = 0.3-0.32, p < 0.05) and 12 (r = 0.35, p < 0.05) months of age, but were not related to cognitive or language outcome. Thus, the leftward hemodynamic status of the middle cerebral arteries, as measured by cranial Doppler ultrasound in the neonatal period, predicts early motor outcome in term infants.
Ultrasound in medicine & biology 02/2013; · 2.46 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: 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.
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.
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.
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.
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.
[Show abstract][Hide abstract] ABSTRACT: Congenital central hypoventilation syndrome (CCHS) is a rare condition. The main characteristic is respiratory insufficiency during sleep. Patients who have CCHS need varying degrees of ventilation support during sleep, or even all day long, according to its severity. We report a term baby with repeated extubation failure, CCHS and Hirschsprung's disease diagnosed at 1 month of age. This patient was discharged at 5 months old with a home ventilator and reached normal developmental milestones.
[Show abstract][Hide abstract] ABSTRACT: De Barsy syndrome (DBS) is characterized by progeroid features, ophthalmological abnormalities, intrauterine growth retardation, and cutis laxa. Recently, PYCR1 mutations were identified in cutis laxa with progeroid features. Herein, we report on a DBS patient born to a nonconsanguineous Chinese family. The exceptional observation of congenital glaucoma, aortic root dilatation, and idiopathic hypertrophic pyloric stenosis in this patient widened the range of symptoms that have been noted in DBS. Mutation analysis of PYCR1 revealed compound heterozygous PYCR1 mutations, including a p.P115fsX7 null mutation allele and a second allele with two missense mutations in cis: p.G248E and p.G297R. The effect of mutation results in a reduction of PYCR1 mRNA expression and PYCR1 protein expression in skin fibroblasts from the patient. The findings presented here suggest a mutation screening of PYCR1 and cardiovascular survey in patients with DBS.
American Journal of Medical Genetics Part A 11/2011; 155A(12):3095-9. · 2.30 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Renal calcification in preterm infants has been described frequently. The etiologic factors have not yet been fully clarified. The objective of this study was to evaluate the incidence of and risk factors for renal calcification in our population.
We retrospectively reviewed the charts of very low birth weight preterm infants during a 1-year period. Renal ultrasound scans were performed at term or before discharge and at a corrected age of 1 year.
Six infants (6%) had renal calcification at term or before discharge compared with 96 who did not. Factors significantly associated with renal calcification included gestational age (26 weeks vs. 29 weeks, p=0.006), birth weight (851 g vs. 1141 g, p=0.004), duration of mechanical ventilation (69 days vs. 29 days, p=0.002), length of intensive care (72 days vs. 41 days, p=0.013), furosemide therapy (33% vs. 3%, p=0.027), and dexamethasone therapy (50% vs. 2% p=0.001). Birth weight and dexamethasone therapy had significant independent association after stepwise logistic regression analysis. Sex, oliguria, acidosis, duration of oxygen therapy, length of hospital stay, nutrition status, and nephrotoxic drugs did not differ between the two groups. Three of the six infants had spontaneous remission of renal calcification, whereas two patients without the finding in neonatal stage had renal calcification at a corrected age of 1 year.
The incidence of renal calcification in very low birth weight infants in this study was relatively low, and the calcification was transient in one-half of the infants. Extremely premature, sick infants requiring long-term ventilation, and those receiving furosemide or dexamethasone were more likely to have renal calcification. Clinicians should be aware that renal calcification may develop beyond the neonatal stage.
[Show abstract][Hide abstract] ABSTRACT: 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.
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).
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.
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.
[Show abstract][Hide abstract] ABSTRACT: Congenital Bochdalek diaphragmatic hernia (CDH) is easily recognized if it is present shortly after birth. However, cases of delayed CDH presentation are more subtle and therefore less easily diagnosed.
We retrospectively analyzed 85 patients who were under 18 and diagnosed with CDH between June 1987 and May 2007. Those diagnosed before 1 month of age were categorized as having early-presenting CDH and those diagnosed after 1 month were categorized as having late-presenting CDH.
Of the 85 cases, 68 (80%) were early-presenting and 17 (20%) were late-presenting CDH. Respiratory symptoms were more common in early-presenting CDH, while gastrointestinal complaints were more dominant in the late-presenting group, particularly in patients with left diaphragmatic defects. Late-presenting CDH, which was more likely to be associated with chronic gastrointestinal symptoms, was associated with a lower mortality than acute early-presenting CDH with respiratory symptoms, despite the more prompt diagnosis of the latter. The small bowel was the organ most commonly herniated into the thorax. Congenital heart disease was the most commonly associated malformation, but the presence of associated anomalies did not increase mortality.
Early-presenting CDH shows acute symptoms, is readily diagnosed, and requires prompt intervention to prevent death. In contrast, late-presenting CDH shows more subtle symptoms and is more difficult to diagnose. Although the late-presenting CHD has better prognosis, it still requires surgery intervention to alleviate the symptoms. Clinicians must maintain a high index of suspicion for CDH, regardless of its presentation.
[Show abstract][Hide abstract] ABSTRACT: Breast milk is the main source of postnatal human cytomegalovirus (HCMV) infection. The aim of this study was to assess the relationship between HCMV load in breast milk and viral transmission to very low birth weight (VLBW) infants.
Breast-fed VLBW infants who were born to HCMV-seropositive mothers and who were managed in a neonatal intensive care unit were enrolled in the study. Blood from mothers and infants was tested for HCMV antibodies after birth. Breast milk was collected for viral culture and HCMV load measurement. Urine from the babies was obtained for HCMV-DNA detection. Symptoms of HCMV infection were recorded and evaluated by neonatologists.
Of the 23 evaluated mothers during a 1-year period, 19 were HCMV seropositive; 17 of the women had detectable HCMV-DNA in their breast milk whey. Of the 23 infants born to the 19 seropositive mothers, 8 infants of 8 mothers had HCMV-DNA detected in the urine, indicating that they were infected, even though the breast milk was always frozen prior to feeding. Three infected infants had symptoms. At 4 weeks after delivery, the median viral load in breast milk from mothers of the 8 infected infants was significantly higher than that from mothers of the 15 noninfected infants (P = 0.04). HCMV was detectable in breast milk for a significantly longer period in mothers of infected infants (7.5 vs. 2.6 weeks P = 0.03).
High HCMV load and prolonged virus excretion in breast milk are maternal risk factors for viral transmission to VLBW infants.
[Show abstract][Hide abstract] ABSTRACT: Scrub typhus is a clinically important endemic disease in Taiwan. The aims of this study were to analyze the clinical manifestations, laboratory data and complications of pediatric scrub typhus in eastern Taiwan.
We searched medical records for all patients with scrub typhus who were hospitalized between 1992 and 2002 at the Taitung branch of Mackay Memorial Hospital, Taiwan. Records of children under the age of 18 with a confirmed diagnosis were selected for retrospective review.
During the study period, 145 patients fulfilled the diagnostic criteria for scrub typhus, of whom 106 (73%) were adults and 39 (27%) were children. The mean age of the children was 7.6+/-4.6 years. The most common clinical manifestations of pediatric scrub typhus were fever (n=39; 100%), cough (n=28; 72%), anorexia (72%), eschar (69%), chill (67%) and lymphadenopathy (64%). The most common complications were hepatic dysfunction (77%) and pneumonitis (54%). Three children (8%) required intensive care, but the overall survival rate was 97%. One child died with multi-organ failure within 8 hours after admission.
Scrub typhus should be considered in children with fever and hepatic dysfunction, particularly in those with a history of environmental exposure in an endemic area for scrub typhus. The presence of an eschar offers an important diagnostic clue, but not for all cases. Children with scrub typhus may develop serious complications and may even die if appropriate treatment is not given. Doxycycline is an effective antibiotic for pediatric scrub typhus in Taiwan.
[Show abstract][Hide abstract] ABSTRACT: Congenital candidiasis presents with a variety of clinical features. We report two neonates with congenital candidiasis characterized by diffuse erythematous papules associated with pneumonia and respiratory distress. Candida pseudohyphae were identifiable in skin scrapings. Systemic cultures were negative, but urine and sputum cultures grew Candida albicans. After prompt systemic antifungal therapy, the infants were discharged from hospital with no overt complications. This report highlights the presence of characteristic skin lesions associated with candidal infection, occurring within 24 hours of birth. This is an important observation which could help in the early diagnosis of congenital candidal infection.
[Show abstract][Hide abstract] ABSTRACT: This study examined the developmental and clinical outcomes in very-low-birthweight (VLBW; < or =1500g) infants with and without bronchopulmonary dysplasia (BPD) throughout infancy, and assessed if BPD predicted poor developmental outcome beyond the effects of other risk factors. One hundred and three VLBW infants (53 males, 50 females; mean gestational age 28wks [SD 2] birthweight 1041g [SD 261]) were graded for severity of BPD according to the American National Institutes of Health (NIH) consensus definition. Neuro-development was assessed using the Neonatal Neurobehavioral Examination-Chinese version, at 36 and 39 weeks' postmenstrual age, and the 2nd edition of the Bayley Scales of Infant Development at 6 and 12 months' corrected age. Clinical outcome was measured by means of rehospitalization for pulmonary causes and treatment with pulmonary medications. Compared with infants without BPD, infants with BPD had higher rates of clinical morbidity, and those with severe BPD further exhibited higher incidences of developmental delay throughout infancy. BPD predicts poor 1-year developmental and clinical outcomes in VLBW infants for which effects are well correlated to the NIH consensus definition.
[Show abstract][Hide abstract] ABSTRACT: 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.
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.
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.
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.
Pediatrics International 01/2008; 49(6):903-7. · 0.88 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: 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).
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.
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%).
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.
Pediatrics International 06/2007; 49(3):380-6. · 0.88 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: 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.
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.
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).
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.
Pediatrics International 06/2007; 49(3):375-9. · 0.88 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The aim of this study was to investigate the neurodevelopmental outcome in very low birth weight infants with postnatal subependymal cysts. During a 3-year period, postnatal subependymal cysts were diagnosed by serial cranial ultrasound in 21 very low birth weight infants born prior to 33 weeks' gestation. These infants and 116 healthy very low birth weight controls were evaluated with the Bayley Scales of Infant Development at 2 years of age. Preterm infants with postnatal subependymal cysts had a significantly lower Psychomotor Development Index (P = .034) and were more likely than the normal group to have motor developmental delay (Psychomotor Development Index <70) (P = .013). The findings indicate that postnatal subependymal cyst is a significant risk factor for impaired motor development in very low birth weight infants (odds ratio 5.73, 95% confidence interval 1.57-20.97).
Journal of Child Neurology 05/2007; 22(4):402-5. · 1.39 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: To evaluate the use of cesarean delivery in Taiwan by comparing local clinical indications with those in international cohorts.
In-patient claims from the National Health Insurance (NHI) in Taiwan were analyzed. Indications for cesarean delivery were evaluated with primary diagnosis codes and procedure codes from the NHI dataset. To produce a stable numerator for cesarean section, 3 years (1998-2000) of claims for cesarean delivery were abstracted and annualized.
Rates ranged between 27.3% and 28.7% for primary cesarean delivery and were below 5% for vaginal birth after a cesarean section (VBAC). Compared with rates in other countries, rates for overall and primary cesarean section as well as for VBAC were significantly higher in medical centers in Taiwan (P<0.001). However, the clinics contributed the most to the difference in both overall and primary cesarean rates. The most common indication for cesarean section was prior cesarean section (43.3%-45.5%), followed by malpresentation (19.6%-23.4%). The proportion of fetuses with malpresentation delivered by cesarean section in Taiwan was 7.9%, almost twice the upper limit expected for all pregnancies as indicated in international studies.
It is important to use appropriately documented data and to compare them with international data when monitoring local obstetric practices. The disproportionately high cesarean delivery rates in Taiwan may hold major lessons for the many countries contemplating or having universal health insurance coverage with a similar mix of providers.
International Journal of Gynecology & Obstetrics 01/2007; 96(1):57-61. · 1.84 Impact Factor