Chang Gung Children's Hospital Research Articles

Comparable Outcomes Between Adolescent/Young Adults and Children With Acute Myeloid Leukemia Following Allogeneic Hematopoietic Cell Transplantation: A Single-Center Experience

The cytogenetics of acute myeloid leukemia (AML) increases exponentially with age. Adolescent and young adult (AYA) patients have specific psychosocial and other challenges, influencing their ability to access appropriate treatment. Therefore, in allogeneic hematopoietic stem cell transplantation (allo-HSCT) for AML, inferior outcomes would be observed in AYA patients compared to children. We defined the age range of AYA patients as 15 to 29 years. Sixty-three patients who underwent allo-HSCT from 1998 to 2020 at Chang Gung Children Hospital were enrolled in this study. Overall survival was the time duration from HSCT to death from any cause. Disease-free survival was the time duration from HSCT to the last follow-up or first event (failure to achieve complete remission, relapse, secondary malignancy, or death from any cause). Thirty-seven (59%) patients were <15 years of age during allo-HSCT, and 26 (41%) were 15 to 29 years of age. The median age during allo-HSCT was 6.3 years for those <15 years of age compared with 15.7 years for AYA patients. The median follow-up period was 2.2 years after hematopoietic stem cell transplantation for patients <15 years old and 3.8 years after hematopoietic stem cell transplantation for AYA patients. Univariate analysis revealed no significant difference in the 5-year overall survival or disease-free survival among all patients. Several distinct AML subtypes could be amenable to treatment deintensification and targeted therapies. Furthermore, we found that children and AYA patients who underwent allo-HSCT for AML had similar survival.

Clinical significance of the fucosyltransferase 2 (FUT2) secretor status in children hospitalized with acute gastroenteritis in Taiwan

The FUT2 gene is a histo-blood group antigen (HBGA) that determines the susceptibility to Norovirus (NoV) infection. This study investigated the clinical significance of the FUT2 gene profile and HBGA expression in NoV infection. Fecal specimens were collected from children in Chang-Gung Children's Hospital with acute gastroenteritis (AGE). The medical records were reviewed for clinical data. The viral etiology of gastroenteritis was validated using molecular methods. Genomic DNA was isolated from saliva or whole blood with the Puregene B Kit, according to the manufacturers' instructions. Single-nucleotide polymorphisms (SNPs) were determined by real-time PCR assays. FUT2 gene DNA was examined in 98 children with AGE. NoV was detected by RT-PCR in 44 patients (44.8%), while 54 (55.2%) had non-NoV AGE. Of the 44 NoV patients, 38 (86.3%) were secretors (no G428A mutation) and six (13.7%) were non-secretors (G428A mutation). Of the 54 non-NoV AGE patients, 28 (51.9%) were secretors and 20 (48.1%) were non-secretors. NoV-infected patients who were secretors had more frequent vomiting (P<0.001), longer duration of diarrhea (P<0.001), and greater overall disease severity score (P<0.001) compared with non-secretors. Non-NoV infection secretor AGE patients had a longer duration of diarrhea (P<0.001) than non-secretors. FUT2 secretor status affects NoV AGE in children. Secretor patients have prolonged diarrhea, more frequent vomiting, more severe disease, and greater infection transmissibility than non-secretors.

Pathological and Incidental Findings in 403 Taiwanese Girls With Central Precocious Puberty at Initial Diagnosis.

Background: Whether girls with central precocious puberty (CPP) should undergo routine brain magnetic resonance imaging (MRI) to identify potential intracranial pathologies is controversial.Aims: To evaluate the brain MRI results of girls with CPP and identify the clinical and endocrine predictors of brain abnormalities.Methods: This retrospective study obtained data from pediatric endocrine clinics at Chang Gung Children's Hospital. From 1997 and 2017, 403 girls were consecutively diagnosed with CPP. After the exclusion of patients with a history of central nervous system (CNS) insults or associated neuropsychiatric symptom or signs, we studied the prevalence of brain abnormalities in 251 girls with CPP who received detailed MRI examinations of the hypothalamus and pituitary area. We also recorded the demographic data of the participants, including the onset of puberty; initial pubertal status; height; weight; uterus and ovary sizes; and basal luteinizing hormone (LH), follicle-stimulating hormone (FSH), estradiol levels, and the response to GnRH stimulation test.Results: Among the 251 girls with CPP, we observed no brain alterations in 190 (75.70%), abnormalities in the hypothalamic-pituitary (H-P) area in 54 (21.51%), and abnormalities in the non-H-P area in 7 (2.79%). Among the 54 patients that had H-P lesions, we noted pathological findings related to CPP (hypothalamic hamartoma) in only one (0.4%) girl aged below 6 years old. We also identified incidental findings in the other girls with H-P lesions, including non-functioning pituitary microadenomas (12.35%), pituitary pars intermedia cysts (4.38%), Rathke's pouch cysts (1.99%), pituitary hypoplasia (1.59%), and pineal gland cysts (0.8%). The patients that had non-H-P lesions were found to have arachnoid cysts (1.59%), Chiari I malformation (0.4%), prepontine nodule (0.4%), and choroidal fissure cyst (0.4%). Of all the patients with brain lesions, 45 (73.77%) underwent regular MRI follow-up. While none of the H-P and non-H-P lesions showed progression, 19.67% of these regressed during the follow-up. None of the participants exhibited other hormonal abnormalities or underwent surgery.Conclusion: The prevalence of true pathological brain lesions related to CPP in girls without prior symptoms or signs of CNS lesions was low (0.4%). None of the girls with intracranial lesions required further intervention besides the GnRH agonist treatment. These data question the routine use of brain MRI in all girls with CPP, especially in those who are healthy without neurologic symptoms.

Therapeutic hypothermia for pediatric refractory status epilepticus May Ameliorate post-status epilepticus epilepsy

BackgroundTo compare the clinical characteristics and outcomes of pediatric patients with refractory status epilepticus (RSE) and super-refractory status epilepticus (SRSE) who received therapeutic hypothermia (TH) plus anticonvulsants or anticonvulsants alone. MethodsTwo-medical referral centers, retrospective cohort study. Pediatric Intensive Care Unit (PICU) at Taoyuan Chang Gung Children's hospital and Kaohsiung Chang Gung Memorial Hospital. We reviewed the medical records of 23 patients with RSE/SRSE who were admitted to PICU from January 2014 to December 2017. Of these, 11 patients received TH (TH group) and 12 patients did not (control group). ResultsThe selective endpoints were RSE/SRSE duration, length of PICU stay, and Glasgow Outcome Scale (GOS) score. We applied TH using the Artic Sun® temperature management system (target temperature, 34–35 °C; duration, 48–72 h). Of the 11 patients who received TH, 7 had febrile infection-related epilepsy syndrome (FIRSE), one had Dravet syndrome, and three had traumatic brain injury. The TH group had significantly shortern seizure durations than did the control group (hrs; median (IQR) 24(40) vs. 96(90), p < 0.05). Two patients in the TH group died of pulmonary embolism and extreme brain edema. The length of PICU stay was similar between the groups (days; median (IQR) 30(42) v.s 30.5(30.25)). The TH group had significantly better long-term outcomes than did the control group (GOS score, median (IQR) 4(2) v.s 3 (0.75), p = 0.01∗). The TH group had a significantly lower incidence of later chronic refractory epilepsy than did the control group (TH v.s non-TH, 5/11 (45%) v.s. 12/12(100%), p < 0.01). ConclusionsTH effectively reduced the seizure burden in patients with RSE/SRSE. Our findings support that for patients with RSE/SRSE, TH shortens the seizure duration, ultimately reducing the occurrence of post-status epilepticus epilepsy and improving patients’ long-term survival.

Divergence of group a rotavirus with genetic variations before and after introduction of rotavirus vaccines in northern Taiwan.

Despite the development of vaccines in 2006, rotavirus is still a major cause of acute gastroenteritis worldwide. This study was performed to analyze the presence of circulating rotaviruses before and after the introduction of rotavirus vaccines to allow phylogenetic comparisons of vaccine strains in northern Taiwan.Rotavirus genotyping and sequencing of rotavirus VP7 and VP4 PCR products were performed by Reverse Transcriptase Polymerase Chain Reaction and DNA autosequencing. Phylogenies were constructed by the neighbor-joining and maximum-likelihood methods using CLUSTAL W software included in the MEGA software package (version 6.0).Between April 2004 and December 2012, a total of 101 rotavirus specimens from pediatric patients with acute gastroenteritis hospitalized in Chang Gung Children's Hospital were amplified, and their VP4 and VP7 sequences were determined. These 101 specimens consisted of 55 pre-vaccine strains (G1 [13, 23.6%], G2 [12, 21.8%], G3 [16, 29.1%], and G9 [14, 25.5%]) and 46 post-vaccine strains (G1 [25, 54.3%], G2 [12, 26.1%], G3 [5, 10.9%], and G9 [4, 8.7%]). The most common combination of the G and P types was G2P[4], accounting for 36% cases, followed by G9P[8] (25%), G1P[8] (20%), G3P[4] (15%), G3P[8] (10%), G1P[4] (5%), and G2P[8] (5%). Phylogenetic analysis showed that only the G1 and P[8] genotypes clustered in the same lineages with the rotavirus vaccine strains.Based on our results, the inclusion of G9, modified G2 and G3 with target lineages, and the combination G2P[4] and G9P[8] in the rotavirus vaccines in Taiwan is warranted as a vaccination strategy.

Molecular epidemiology and clinical characteristics of norovirus gastroenteritis with seizures in children in Taiwan, 2006-2015.

This study examined the characteristics of norovirus (NoV) gastroenteritis associated with convulsions in children and its molecular epidemiology. From July 2006 through December 2015, NoV infection was confirmed by the genome detection using reverse transcriptase polymerase chain reaction. Viral genotyping with strain validation was achieved using sequence analyses with Basic Local Alignment Search Tool genome identification. The patients’ clinical features were assessed retrospectively, focusing on convulsive disorders. The diagnosis of encephalitis followed the International Encephalitis Consortium. Seizures occurred in 52 (20.9%) of 249 NoV infections. GII.4 Den_Haag_2006b (n = 22, 42.3%) and GII.4 Sydney 2012 (n = 10, 19.2%) were major variants correlated with convulsions. Patient with convulsions tend to have GII.4 genotype infection (P < .001), short vomiting (≤2 days) (P < .001), and no fever (P = .002). Compared to GII.4 Den_Haag_2006b, the GII.4 Sydney 2012-associated convulsions had similar manifestations except without significant winter preponderance (P = .049). The NoV infection with convulsions had less febrile course, specific genotype (GII.4) infections, and with shorter symptom of vomiting. Continuous surveillance is important for uncommon disease associated with emerging NoV strain infections. The prevention of NoV diseases requires the development of vaccines targeting highly virulent variants.

Long-term impact of suboptimal rotavirus vaccines on acute gastroenteritis in hospitalized children in Northern Taiwan.

Rotavirus vaccines were launched in Taiwan since early 2006. Our study was aimed to figure out long-term extended molecular epidemiology in acute gastroenteritis (AGE) in hospitalized young children after rotavirus vaccination in Taiwan. During the 10-year period from January 2007 to December 2016, fecal samples from children under 5 years old with AGE hospitalized in Chang Gung Children's Hospital (CGCH) were examined for enteric pathogens and they were divided into two time intervals: early post-vaccine (Jan. 2007 to Dec. 2011; EPV) and late post-vaccine (Jan. 2012 to Dec. 2016; LPV). In total, 837 patients with AGE were enrolled with complete study. In the EPV period, 106 (26.7%) rotavirus and 65 (16.4%) norovirus infections were identified as major pathogens. In the LPV period, 79 (17.9%) rotavirus and 98 (22.2%) norovirus infections were diagnosed. Statistical analyses showed a significantly decreased prevalence of rotavirus infection (P=0.002) and a significantly increased prevalence of norovirus (P=0.034) and enteric bacterial infections (P<0.001). A substantial decrease of rotavirus G1 (P=0.079) in the LPV period and norovirus GII.4 prevailed through the decade. In Taiwan, under a suboptimal rotavirus vaccination policy, there was a marked decrease in the rate of rotavirus AGE of hospitalized young children. Significantly increased norovirus infection has replaced rotavirus as the leading cause. Expansion of rotavirus vaccine coverage, development of a norovirus prevention strategy, and sustained bacterial infection control are important for AGE containment in children in Taiwan.

Prevalence and independent risk factors for hearing impairment among very low birth weight infants

BackgroundAlthough we've made big strides in perinatal and neonatal care, auditory handicap remains a serious complication in those who were born very premature. ObjectivesThe aim was to determine the prevalence and analyze possible risk factors of hearing impairment in very-low-birth-weight (VLBW) infants. Materials and methodsThis was a retrospective study by reviewing medical records of all VLBW infants (BW ≤ 1500 g) admitted to NICU of Chang Gung Children's Hospital over 2 years period from Jan. 2010 to 2011. Brainstem auditory evoked potentials (BAEP) hearing screening was performed at 3 months postnatal corrective age and repeated if failed the 1st time, then refer to ENT doctor if BAEP confirmed abnormal. All VLBW infants examined for hearing impairment were included and data were retrieved retrospectively and analyzed for neonatal risk factors using logistic regression. ResultsOver the period, 309 VLBW infants were screened. Prevalence of uni- or bilateral hearing impairment was 3.9% (12/309; 95% CI 2.6–4.1). The mean corrective age on diagnosed of hearing impairment was 2.9 ± 1.1 (range 1–5) months. Mean gestational age was 27.9 weeks (SD 1.4) and mean birth weight was 1028 g (SD 180). By univariant analysis for hearing impairment, severe birth asphyxia, craniofacial anomalies, ventilator dependence, patent ductus arteriosus ligation, and use of postnatal ototoxins yielded good prediction of hearing impairment in this population. However, using multivariate analysis revealed that the only independent risk factors for hearing impairment were ototoxins (OR: 3.62; CI: 1.67–7.82), PDA ligation (OR: 4.96; CI: 2.34–10.52), craniofacial anomalies (OR: 3.42; CI: 1.70–6.88)and assisted prolonged use of oxygen at gestational age of >36 weeks (OR: 5.94; CI: 2.61–13.54). ConclusionThe incidence of hearing impairment among VLBW infants was 3.9%. Prolonged supplemental oxygen use is a marker for predicting hearing impairment; this requires detailed analysis of the pathophysiologic features, to reduce the prevalence of hearing impairment.

Primary spinal tumors in childhood: A single institution 15 year experience.

Background:Pediatric primary spinal tumors (PST) are fairly uncommon, with little available data regarding incidence and outcomes.Materials and Methods:We conducted a retrospective review of the 22 consecutive patients less than 18 years old diagnosed with PST between March 1997 and May 2011 and treated at Chang Gung Children Hospital. All patients had undergone magnetic resonance imaging (MRI) for pre-operative evaluation and operations for PST. The extent of tumor removal was assessed by surgical report by the neurosurgeon or by post-operative MRI if available.Results:Ten of them had intramedullary tumors and 12 had intradural extramedullary tumors. All patients were treated with surgery to the primary site. A total of 15 patients underwent gross total tumor resection and seven patients received post-operative radiotherapy. Five patients received adjuvant chemotherapy for their primary tumor. Fourteen patients (64%) survived from study entry without tumor progression.Conclusions:PST encompassed a diverse group of pathologic entities that differ markedly based on the location and age of the children. Total resection of pediatric PST in children could be performed with acceptable risk and satisfactory long-term results.

Oligodendrogliomas in Children: Clinical Experiences With 20 Patients

Oligodendrogliomas occurring rarely in children are incompletely characterized. The purpose of this study was to identify prognostic factors affecting the local control and survival in the management of children with oligodendrogliomas. We retrospectively analyzed clinical data on 20 pediatric patients with oligodendrogliomas treated at Chang Gung Children's Hospital between 1994 and 2014. There were 12 males and 8 females with a median age of 9.2 years at diagnosis (range, 3 mo to 18 y). Eighteen (90%) tumors were located in the cerebral hemispheres, 10 cases were located on the right, 8 on the left. One was located in the third ventricle and 1 in the thoracic spine. Presenting symptoms included seizures (n=7), headache (n=5), visual field defects (n=3), limb weakness (n=2), vomiting (n =1), back pain (n=1), and increased head circumference (n=1). All patients underwent craniotomy: 8 gross total resections, 8 subtotal resections, and 4 biopsies. Nine of the patients had pure oligodendroglioma and 11 had anaplastic oligodendroglioma (WHO grade III or IV). Ten children had adjuvant therapy including radiation (n=7), chemotherapy (n=1) or both (n=2). With the median follow-up of 5.3 years (range, 1.2 to 14.7 y), the 5-year overall survival and disease-free survival rates were 78.9% with 65.0%, respectively. Total tumor resection offers better overall survival regardless of the histologic grading. Our data demonstrate that patients with less than gross total resections are at increased risk for progression and may benefit from more aggressive therapy.

Decreased ADAMTS 13 Activity is Associated With Disease Severity and Outcome in Pediatric Severe Sepsis.

Decreased ADAMTS 13 activity has been reported in severe sepsis and in sepsis-induced disseminated intravascular coagulation. This study aimed to investigate the role of ADAMTS 13 in different pediatric sepsis syndromes and evaluate its relationship with disease severity and outcome.We prospectively collected cases of sepsis treated in a pediatric intensive care unit, between July 2012 and June 2014 in Chang Gung Children's Hospital in Taoyuan, Taiwan. Clinical characteristics and ADAMTS-13 activity were analyzed.All sepsis syndromes had decreased ADAMTS 13 activity on days 1 and 3 of admission compared to healthy controls. Patients with septic shock had significantly decreased ADAMTS 13 activity on days 1 and 3 compared to those with sepsis and severe sepsis. There was a significant negative correlation between ADAMTS 13 activity on day 1 and day 1 PRISM-II, PELOD, P-MOD, and DIC scores. Patients with mortality had significantly decreased ADAMTS 13 activity on day 1 than survivors, but not on day 3.Different pediatric sepsis syndromes have varying degrees of decreased ADAMTS 13 activity. ADAMTS 13 activity is strongly negatively correlated with disease severity of pediatric sepsis syndrome, whereas decreased ADAMTS 13 activity on day 1 is associated with increased risk of mortality.

High prevalence and antimicrobial resistance of urinary tract infection isolates in febrile young children without localizing signs in Taiwan.

Antimicrobial susceptibility and prevalence of pediatric urinary tract infection (UTI) is very useful for pediatricians in selecting effective antibiotics in time to improve outcomes in patients. This study aimed to determine the prevalence rate, bacterial distribution, and antimicrobial susceptibility of UTI in febrile young children at a teaching hospital in northern Taiwan. From January 2011 to December 2011, all urinary isolates from suspected cases of UTI in febrile young children aged from 1 day to 36 months visiting the Pediatric Emergency Room of Chang Gung Children's Hospital, Taoyuan, Taiwan were identified by conventional methods. Antibiotic susceptibility was determined according to the Clinical and Laboratory Standards Institute. A total of 5470 (78%) from 7009 eligible children were enrolled in the study, and 619 (11.3%) had a diagnosis of UTI. The most prevalent bacterium was Escherichia coli (68%) followed by Klebsiella pneumoniae (8.1%) and Proteus mirabilis (6.8%). Ampicillin, piperacillin, and trimethoprim-sulfamethoxazole (TMP-SMX) showed a higher resistance rate in the three predominant bacteria. All tested bacteria showed higher resistance to ampicillin (79.3%) and TMP-SMX (44.1%), and lower resistance to cefazolin (17.7%) and gentamicin (13.0%). Fourteen percent of the isolates produced extended spectrum β-lactamase (ESBL), among which 93.33% were E. coli isolates. The overall prevalence of UTI in this study was higher than previously reported in febrile children. Higher antimicrobial resistance was found in ampicillin and TMP-SMX. Among commonly used antibiotics, cefazolin and gentamicin are recommended to treat UTI in febrile children aged < 3 years without localizing signs.

Transcranial Doppler ultrasound in therapeutic hypothermia for children after resuscitation

Aim of the studyThe aim of the present study was to assess the cerebral flow in children receiving therapeutic hypothermia after resuscitation. The prognostic value of transcranial Doppler findings was correlated with the clinical outcomes in these children. MethodsA retrospective cohort study was conducted at the paediatric intensive care unit of Chang Gung Children's Hospital between January 2011 and December 2012. All children from 1 month to 18 years of age who received therapeutic hypothermia after resuscitation were eligible. Serial transcranial Doppler examinations were performed and the findings were reviewed. ResultsSeventeen children met the eligibility criteria for this study. Fourteen patients (82.3%) were asphyxial in aetiology, and 12 (70.5%) of these cases occurred outside of the hospital. Eight patients (47.1%) had a Paediatric Cerebral Performance Score of 1 or 2 at 3 months after the events. Reversal diastolic or undetectable flow patterns during therapeutic hypothermia were associated with unfavourable prognosis. Normal mean flow velocity in the rewarming phase and normal pulsatility index in the hypothermia and rewarming phases were associated with favourable outcome. ConclusionThe transcranial Doppler examinations provided additional information for cerebral perfusion during therapeutic hypothermia, which may in the future be used to guide changes to hypothermia management. Mean cerebral blood flow velocity and pulsatility index by transcranial Doppler sonography can serve as a prognostic factor for children who receive therapeutic hypothermia after resuscitation.

Macrolide-resistant Mycoplasma pneumoniae in children in Taiwan

The aim of this study was to estimate the prevalence of macrolide-resistant Mycoplasma pneumoniae in Taiwan and to compare the clinical courses of pediatric patients with macrolide-resistant (MR) M. pneumoniae and macrolide-susceptible (MS) M. pneumoniae infection. Patients were among the children admitted to Chang Gung Children’s Hospital with mycoplasmal pneumonia between February and December 2011. Detection for macrolide resistance was performed after informed consent was obtained. We retrospectively reviewed medical records and compared the clinical courses of two groups of patients of 73 children enrolled into our study. The rate of macrolide resistance in M. pneumoniae was 12.3 %. Longer hospital stay was observed in the MR patients than MS patients [median, 7 days vs. 5 days (P = 0.019)]. Clinical features or radiographic or laboratory findings are not helpful to differentiate MR from MS mycoplasmal pneumonia. Early diagnosis of MR mycoplasmal pneumonia is crucial for the best management of these patients and obviates the need for extensive etiological searches of these nonresponding cases.

Viral etiology of bronchiolitis among pediatric inpatients in northern Taiwan with emphasis on newly identified respiratory viruses

PurposeViral etiology of bronchiolitis in children in Taiwan has been fragmentary. We conducted a prospective study to figure out the viral epidemiology of bronchiolitis in Taiwan.Materials and methodsFrom January 2009 to March 2011, a total of 113 children with bronchiolitis, aged <2 years, hospitalized in Chang Gung Children’s Hospital were randomly selected for viral etiology investigation. Nasopharyngeal aspirates were obtained from each case and sent for viral detection by tissue culture, antigen test, and polymerase chain reaction.ResultsA total of 120 viruses were detected from 113 children. Positive viral etiology was identified in 86 (76%) children. Mixed viral pathogens were found in 28 cases (25%). Respiratory syncytial virus (RSV) was the most common pathogen and was identified in 43.4% of the cases. Human bocavirus (hBoV) was the second most common identified virus (in 19.5%), followed by human metapneumovirus (hMPV), rhinovirus, influenza viruses, and coronavirus OC43. In terms of clinical characteristics, no significant difference was found among the children with bronchiolitis either caused by different single or mixed viral infection.ConclusionRSV was the most common etiologic agent for children with bronchiolitis in Taiwan. Newly identified viruses, including hMPV and hBoV, were also among the common causative agents. Clinical characteristics were not significantly different among the children with bronchiolitis caused by different viruses.

The Impact of Patent Ductus Arteriosus in Neonates with Late Onset Sepsis: A Retrospective Matched-Case Control Study

Late-onset sepsis (LOS) in neonates with patent ductus arteriosus (PDA) may predispose them to more complicated hospital courses. The objective of this study was to determine the incidence, the distribution of pathogens, and the clinical features of LOS in neonates with PDA and analyze their outcomes. The medical records were reviewed retrospectively of infants with PDA and LOS who were hospitalized in NICUs of Chang Gung Children's Hospital between January 2003 and December 2009. The clinical features of these infants were compared with a group of gestational age and birth body weight-matched neonates with LOS during the same period. During this period, 224 neonates were found to have at least one event of blood-culture proven LOS and 79 (35.3%) were documented to have PDA. Although most LOS episodes (85/104, 81.7%) in neonates with PDA occurred after closure of PDA, neonates with PDA had a significantly higher rate of bronchopulmonary dysplasia (81.0% vs. 61.0%, p=0.002) and a relatively higher rate of recurrent sepsis (25.3% vs. 15.2%, p=0.079) than those without PDA. Longer durations of ventilator support and hospital stay were also noted in neonates with PDA as compared to those without (p=0.001 and 0.005, respectively). In neonates with LOS, the presence of PDA, even though it is aggressively treated with indomethacin or surgical intervention, may still contribute to the complexity of hospitalization. Close monitoring and aggressive treatments are warranted in these neonates with PDA.

Clinical Variants of Guillain-Barré Syndrome in Children

Guillain-Barré syndrome is characterized by acute progressive weakness, areflexia, and maximal motor disability that occur within 4 weeks of onset. Various clinical subtypes have been described since the original description of the syndrome. This study aimed to identify characteristics of clinical variants of Guillain-Barré syndrome through retrospective review of cases in Chang Gung Children's Hospital from 2000-2010. Forty-three Guillain-Barré syndrome patients were evaluated based on clinical presentations and an electrodiagnostic study. The most frequent variant of Guillain-Barré syndrome was demyelinating polyneuropathy (67.4%), followed by acute axonal neuropathy (7.0%), Miller Fisher syndrome (7.0%), Bickerstaff brainstem encephalitis (7.0%), pharyngo-cervical-brachial variant (4.7%), and polyneuritis cranialis (4.7%). Follow-up revealed that 35 recovered satisfactorily, eight were persistently disabled, and none died during hospitalization. At the earliest stage, differentiating clinical variants from typical Guillain-Barré syndrome was difficult. Children with clinical variants of Guillain-Barré syndrome are more likely to manifest rapid onset from disease onset to nadir, increasing the severity of disability, cranial nerve involvement, urine incontinence, respiratory impairment, and need for ventilator support than in typical Guillain-Barré syndrome.

Analysis of Incidence and Risk Factors of Retinopathy of Prematurity Among Very-low-birth-weight Infants in North Taiwan

Although significant advances have been made in perinatal care, retinopathy of prematurity (ROP) remains a serious complication in prematurely born individuals. There have been limited studies on ROP in Taiwan, and most of those existing reports are outdated. This retrospective study included 252 very-low-birth-weight (VLBW) infants admitted to the neonatal intensive care unit of Chang Gung Children's Hospital over a 2-year period between July 2005 and June 2007. All infants were examined for ROP according to the guidelines published by the American Academy of Pediatrics. The relationship between clinical risk factors and the development of ROP was analyzed. Of the 252 VLBW infants, 216 met the screening criteria. Of the 216, 99 (45.8%) had ROP. Compared with neonates born at 29 weeks of gestational age (GA) or later, those very premature infants of ≤25 weeks' and 26-28 weeks' GA had increased odds ratios (OR) of 8.49 and 3.19, respectively, for the development of severe ROP. No ROP was detected in infants of greater than 33 weeks' GA. The simultaneous presence of a low GA, low birth weight (LBW), lower Apgar scores, hypotension, patent ductus arteriosus, septicemia, intraventricular hemorrhage, ventilator dependence, and use of postnatal steroids was associated with severe ROP. Using multiple logistic regression analyses for ROP, only maternal preeclampsia [OR, 2.52; confidence interval (CI), 1.32-4.7]; duration of mechanical ventilation (OR, 1.06; CI, 1.04-1.08); and LBW (OR, 2.62; CI, 1.370-3.375) predicted the development of threshold ROP. The incidence of ROP among VLBW infants was 45.8%; 19.0% had severe ROP. Infants of lower GAs and/or with LBW, whose mother had preeclampsia or who had a long duration of mechanical ventilation are at risk for the development of threshold ROP.

Analysis of Incidence and Risk Factors of Retinopathy of Prematurity among Very-Low-Birth-Weight Infants in North Taiwan

Background: Although significant advances have been made in perinatal care, retinopathy of prematurity (ROP) remains a serious complication in prematurely born individuals. There have been limited studies on ROP in Taiwan, and most of those existing reports are out-dated.Methods: This retrospective study included 252 very-low-birth-weight (VLBW) infants admitted to the neonatal intensive care unit (NICU) of Chang Gung Children's Hospital over a 2-year period between July 2005 and June 2007. All infants were examined for ROP according to the guidelines published by the American Academy of Pediatrics (AAP). The relationship between clinical risk factors and the development of ROP was analyzed.Results: Of the 252 VLBW infants, 216 met the screening criteria. Of the 216, 99 (45.8%) had ROP. The simultaneous presence of a low GA, low birth weight (BW), lower Apgar scores, hypotension, patent ductus arteriosus (PDA), septicemia, intraventricular hemorrhage (IVH), ventilator dependence, and use of postnatal steroids was associated with severe ROP. Using multiple logistic regression analyses for ROP, only maternal preeclampsia [odds ratio (OR), 2.52; confidence interval (CI), 1.32-4.7], duration of mechanical ventilation (OR, 1.06; CI, 1.04-1.08), and LBW (OR, 2.62; CI, 1.370-3.375) predicted the development of threshold ROP.Conclusion: The incidence of ROP among VLBW infants was 45.8%; 19.0% had severe ROP. Infants of lower GAs and/or with low BW, whose mother had preeclampsia, or who had a long duration of mechanical ventilation are at risk for the development of threshold ROP.

Necrotizing Pneumococcal Pneumonia With Bronchopleural Fistula Among Children in Taiwan

Severe necrotizing pneumococcal pneumonia may progress to the development of bronchopleural fistula (BPF). The purpose of this study was to describe the clinical courses and identify risk factors for the development of bronchopleural fistula in children with pneumococcal pneumonia. Histopathologic features of children receiving surgical resections of the lung because of BPF were analyzed to explore the pathogenesis of destructive lung disease caused by Streptococcus pneumoniae. A total of 112 cases of culture-proven pneumococcal pneumonia were identified between January 2001 and March 2010 at Chang Gung Children's Hospital. The medical charts of all cases of culture-proven pneumococcal pneumonia were reviewed. Pneumococcal pneumonia in 18 children (18/112, 16.1%) was complicated by BPF. As compared with children without BPF, children with BPF had significantly lower white blood cell counts at admission (P = 0.03) and significantly longer durations of fever and hospitalization (P < 0.001). Multivariate analysis revealed that acute respiratory failure (odds ratio = 8.9; 95% confidence interval = 2.6-30.9; P = 0.001) and serotype 19A infection (odds ratio = 5.0; 95% confidence interval = 1.2-22.1; P = 0.03) were risk factors for the development of BPF. Histopathologic analyses were available for 12 children who underwent surgical resections of the lung. Coagulative necrosis with pulmonary infarction was found in 11 of the 12 cases. Serotype 19A was strongly associated with BPF. Vaccines containing this serotype will be important for prevention.