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Background: To investigate the clinical features of Kikuchi-Fujimoto disease (KFD) in children, and place an emphasis on the therapeutic effects of hydroxychloroquine as monotherapy. Methods: We retrospectively reviewed the medical records of all children diagnosed with KFD during the period January 1992 to September 2016 at a tertiary medical center in Taiwan. Results: 40 patients were histopathologically confirmed as KFD, and the mean age of the patients was 13.9 ± 3.1 years. The male to female ratio was 1:1. The lymph node involvements were often cervical (95%) with features of unilateral predisposition (75%), polyadenopathy (84.4%) and tenderness (56.3%). Fever, cough, rhinorrhea, and tonsillitis were other common presentations. Laboratory findings included leukopenia (56.5%), monocytosis (63.6%), with positive results of EB-VCA IgG (88.9%), EB-VCA IgM (22.2%), EBEA IgG (22.2%) and EBNA IgG (88.9%). The univariate analyses of prolonged fever with lymphopenia, monocytosis, thrombocytopenia and necrotizing type in histopathology were disclosed as statistically significant (P

Background The relationship between hemoglobin and patent ductus arteriosus (PDA) has not been discussed before. The aim of this study was to assess the influence of hemoglobin and perinatal factors on PDA in very low-birthweight (VLBW) infants. Methods Using data from the Premature Baby Foundation of Taiwan (PBFA), the characteristics, perinatal factors, and hemoglobin of VLBW infants with and without PDA and treatment were compared. Results Between January 2008 and December 2010, 89 VLBW infants were admitted to hospital. The overall survival rate was 87.6%. Twelve infants were excluded. Compared to those without PDA, the 34 infants with PDA received oral indomethacin or surgery, had smaller mean gestational age (GA; 27.93 ± 1.818 weeks, P = 0.000003), lower mean birthweight (BW; 1031 ± 259 g, P = 0.0001), significantly lower Apgar score at 5 min, more severe respiratory distress syndrome (RDS; grade 3 or 4), greater use of surfactants, and lower mean hemoglobin (P = 0.018) after birth than those without PDA. Compared to those with indomethacin, the seven infants with surgery had lower mean GA (26.43 ± 1.718 weeks, P = 0.011), significantly lower Apgar scores at 1 min, but higher platelet count (P = 0.002) after birth, and more red blood cell transfusions (P = 0.039). Conclusions Smaller GA, lower BW, lower Apgar score at 5 min, more severe RDS, greater use of surfactants, and lower hemoglobin after birth place VLBW infants at greater risk of PDA. Proper prenatal care, and prevention of premature labor and delivery may be the most important preventative factors. The appropriate hemoglobin level for PDA closure requires further investigation.

BackgroundThis study summarized the epidemiology, etiology, and susceptibility of pathogens to antibiotics, and specific characteristics in infants aged less than 4 months diagnosed with urinary tract infection in the past decade in Taiwan.MethodsThe medical charts of patients aged less than 4 months admitted for urinary tract infection to Kaohsiung Veterans General Hospital between January 2001 and December 2009 were retrospectively reviewed.ResultsA total of 132 patients, with male predominance (68.9%), were enrolled. The top three pathogens were similar to those identified in previous studies in Taiwan. The most common pathogen, Escherichia coli (85.3%), was resistant to ampicillin (75.9%), followed by sulfamethoxazole/trimethoprim (31.7%), and cefazolin (28.5%). Dimercaptosuccinic acid (DMSA) renal scan revealed 34.5% positive findings, while the vesicoureteral reflux (VUR) rate was 37.8% by direct radionuclide voiding cystography and/or voiding cysto-urethrography. Positive DMSA findings significantly correlated with VUR (p

Background: Beginning 2007, the intratracheal route of epinephrine to end massive pulmonary hemorrhage (MPH) in very low-birthweight (VLBW) infants was modified at Kaohsiung Veterans General Hospital. The aim of the present study was to assess the change in outcomes for these infants, and to evaluate the risk factors of MPH. Methods: Using the database of the Premature Baby Foundation of Taiwan, the mortality, risk factors and characteristics of VLBW infants with or without MPH were compared between 2000–2006 and 2007–2010. Results: Between January 2000 and December 2010, 399 VLBW infants were admitted to Kaohsiung Veterans General Hospital. Mean birthweight (BW) was 1099.6 ± 272.7 g, and mean gestational age (GA) was 28.7 ± 2.9 weeks. The overall survival rate was 84.2%. Sixteen (4%) had MPH: 11 in the first group (2000–2006; 18.2% survival rate), and five in the second group (2007–2010; 80% survival rate; P= 0.0000002). Infants with MPH had lower mean BW (864.9 ± 301.4 g, P= 0.0004), smaller mean GA (26.1 ± 2.0 weeks, P= 0.0002), significantly lower Apgar scores at 1 and 5 min, higher severity of respiratory distress syndrome (RDS; grades 3 or 4), and greater use of surfactant than infants without MPH. They also had more intraventricular hemorrhage and higher mortality. Conclusions: Smaller GA, lower BW, lower Apgar scores at 1 and 5 min, severe RDS (grades 3 or 4), and use of surfactant place VLBW infants at greater risk of MPH. Proper prenatal care and preventing premature labor and delivery were the most important preventative factors. A quick, deep thrust of intratracheal epinephrine with a catheter may improve survival.

Background/Purpose Infective endocarditis caused by Staphylococcus lugdunensis is a rare disease. Since its first description in 1988, there have only been a few reports of this disease and the causative organism. These publications were primarily case reports and brief case series. We conducted a literature review to identify the nature of the disease and its risk factors. Methods We retrospectively reviewed the cases reported between 1988 and 2008 by searching the relevant literature using the keywords "endocarditis" and " Staphylococcus lugdunensis " in the Medline database. All cases included met the definition of the modified Duke criteria. Results For the period 1988-2008, 67 cases from 27 articles were reviewed. The mean age of individuals was 53.9 years. Left-sided valvular endocarditis represented 52 (82.5%) of cases and native-valve endocarditis was evident in 48 (78.7%) of cases. A large proportion (82%) of 50 S. lugdunensis strains were susceptible to penicillin. Valve replacement operations were performed in 42 (66.7%) patients and the mortality rate was 38.8%. Univariate analysis showed a higher mortality rate in patients aged more than 50 years, those treated before 1995, those treated with antibiotics alone, and those with growth not detected by echocar-diography. Medical treatment alone was the independent risk factor for mortality by multivariate analysis. Conclusion S. lugdunensis endocarditis led to substantial morbidity and mortality. Detailed microbiological identification, echocardiography evaluation, and valve replacement may improve the clinical outcome of individuals with S. lugdunensis endocarditis.

The utero-placental ischemia induced by pregnancy-induced hypertension (PIH) could lead to fetal hypoxia and proinflammatory cytokine release, which are associated with the development of neonatal necrotizing enterocolitis (NEC). However, a few studies have investigated the relationship between PIH and neonatal NEC and have produced controversial results. Therefore, we attempted to assess the relationship between PIH and the subsequent neonatal NEC risk and identify predictive risk factors. Patients with newly diagnosed PIH were recruited from the Taiwan National Health Insurance Research Database (NHIRD). For each participant, 4 age- and delivery-year-matched participants without PIH were randomly selected. A multivariable logistic regression was performed for the identification of the predictive risk factors for neonatal NEC. Among the 23.3 million individuals registered in the NHIRD, 29,013 patients with PIH and 116,052 matched controls were identified. For the multivariable analysis, maternal PIH was associated with an increased risk of subsequent neonatal NEC development (odds ratio [OR] 1.86, 95% confidence interval [CI] 1.08–3.21, P = .026). Furthermore, single parity (OR 2.06, 95% CI 1.12–3.77, P = .019), preterm birth (OR 5.97, 95% CI 3.49–10.20, P

Background Incontinentia pigmenti (IP) is a rare X-linked dominant disorder that involves ectodermal tissues of multiple systems. Previous reports are few in Taiwan. To contribute toward better understanding of IP, we describe and discuss the clinical features of cases that were diagnosed in a medical center during the past 12 years. Methods The medical records of all patients with IP between July 1995 and June 2007 were reviewed retrospectively. The demographics, physical findings, pathology reports, molecular study reports, eosinophil counts and outcome were recorded. Results A total of 4 patients, 3 female and 1 male neonate, who met the criteria for the diagnosis of IP were enrolled. Among these cases, 3 were not diagnosed with IP at initial presentation but were regarded to have infectious diseases. A definite family history of 3 consecutive generations was proved not only by clinical manifestations but also by molecular study in 1 patient. The patient also had retinal and vitreous body hemorrhage, which rapidly progressed to retinal detachment of the right eye in 2 months. Another patient presenting with stage III hyperpigmentation at birth had an extremely rare finding of left foot deformity. The male patient had unilateral and localized vesicular lesions over his left thigh. Conclusion Diagnosis of IP is difficult in the neonatal period. Referral to experienced specialists is necessary. Multiple clinical characteristics of IP and rapid progression of ophthalmologic manifestations can be demonstrated through our study. Furthermore, 3 of the 4 cases in our study are the very first reports in Taiwan.

Background Infective endocarditis (IE) due to Pseudomonas aeruginosa is rare and accounts for only about 3% of all patients with this disease. Most infections are associated with the use of intravenous drugs. Patients with P. aeruginosa -related IE who do not use intravenous drugs are extremely rare. We carried out a review of the literature to identify the nature and risk factors of this disease. Methods Patients with IE reported between 1993 and 2013 were reviewed by searching the Medline database using the keywords "endocarditis" and " Pseudomonas aeruginosa ". All of the patients included met the definition of the modified Duke criteria. Results Twenty-seven patients in 22 reports were reviewed. IE associated with health care accounted for 20 patients (74%). The mean age of the patients was 53.4 years and there was a predominance of men (81.5%). Native valve endocarditis was seen in 20 (74.1%) patients. Surgery for infection control was performed in 15 (55.6%) patients and the mortality rate in patients who underwent surgery was 33.3% (five patients). A relapse of IE after adequate treatment was seen in nine (33.3%) patients. The mortality rate in all 27 patients was 28.6% (2/7) for those with community-acquired IE and 40% (8/20) for those with IE associated with health care. Univariate analysis showed a higher mortality rate in patients aged >60 years and in those whose source of endocarditis was related to a prosthetic device. Conclusion P. aeruginosa endocarditis has substantial morbidity and mortality. It is characterized by easy relapse and is highly associated with prosthetic devices.

Patients with type 1 diabetes mellitus (T1DM) have an increased risk of other autoimmune disorders. The combination of Addison's disease with T1DM and/or autoimmune thyroid disease is known as autoimmune polyendocrinopathy type 2 (APS-2). 21-hydroxylase autoantibody (21OHAb) is considered as a valuable marker for identifying patients with autoimmune Addison's disease (AD); however, it is not available in some countries. Here we present a 5-year-old boy with newly diagnosed T1DM, who developed AD with adrenal crisis within only six months, and after 1-year treatment, the test of 21OHAb was negative. This was a rare and the first APS-2 case in Taiwan, because APS-2 affects female adults more often, but not boys. At diagnosis of T1DM, we suggest that checking diurnal cortisol and adrenocorticotropic hormone levels as a baseline evaluations, and if it is available, checking 21OHAb as well. If there is subtle evidence of AD, such as unexplained hypoglycemia or unreasonably reduced insulin requirements, adrenal functions must be studied as soon as possible, even in the 21OHAb-negative T1DM patients. Even if nothing is abnormal, the patient still needs an annual measurement.

We report the case of a newborn who suffered right atrial laceration during delivery. To our knowledge, there has been no previous report of blunt injury causing right atrial laceration with hemopericardium complicated by cardiac tamponade in the perinatal period.

Background Lower respiratory tract infections (LRTIs) play an important role in pediatric diseases; however, there are limited data about LRTIs in Southern Taiwan. This study aimed to investigate the clinical and epidemiological data of LRTIs in this area. Methods Children aged under 5 years who were hospitalized at a medical center in Southern Taiwan with acute LRTIs from July 2010 to October 2010 (summer) and from March 2011 to May 2011 (spring) were prospectively enrolled. Nasopharyngeal aspirates were obtained and sent for viral cultures, multiplex polymerase chain reaction (PCR), and traditional quick tests. The clinical features, laboratory data, and imaging findings were recorded and analyzed. Results A total of 90 children were enrolled, 70 of whom had detectable pathogens. The positive rate of conventional viral and bacterial cultures was 25.6%, which increased to 77.77% after combining with the two multiplex PCR methods. Adenovirus and enterovirus were the most common viral etiologies identified (26.5% of cases) and Streptococcus pneumoniae was the leading bacterial etiology (46.4%). The seasonal trend of viral infections in Southern Taiwan was different from Northern Taiwan. There were no differences in demographic data, severity of disease, or hospital stay between single and mixed infections. A similar result was found between nonpneumococcal and pneumococcal infections. Conclusion Viral infections were the main etiologies of LRTIs in young children. Multiplex PCR methods are rapid assays that can increase the diagnostic yield rate. Mixed infections do not seem to affect the severity of disease. Early detection may aid clinicians in appropriate decision-making and treatment.

Combined methylmalonic aciduria and homocystinuria, cobalamin (cbl)C type (cblC disease), the most common inborn error of vitamin B 12 , is a rare disorder of intracellular cbl metabolism because of mutations in the MMACHC gene located in chromosome region 1p34.1. It has become possible to establish phenotype–genotype correlations and to observe ethnicity-related trends. This article provides detailed clinical manifestations and outcomes of a Taiwanese infant boy with early-onset cblC disease, heterozygous for c.609G>A and c.567dupT mutations, although there is limited information about cases with c.609G>A or c.567dupT mutation in the literature. He had no significant clinical abnormality during his neonatal period, whereas elevated C3 level was noted at newborn screening. He presented later with life-threatening manifestations and failure to thrive, which resolved through our treatment, although delayed development was still noted at 6 months of age. To date, all reported cblC patients with the c.609G>A mutation have been East Asians. Therefore, we suggest that c.609G>A should be included in the initial mutation screening tests for a cblC patient in East Asian populations.

Background: Central venous catheterization is an important procedure for infant patients for a number of different purposes, including nutritional support, surgical operation, hemodynamic monitoring, and multiple lines for critical care medications. Subclavian vein catheterization (SVC) is one of the central vein catheterization techniques. SVC can be performed from 4 different locations: right supraclavicular (RSC), left supraclavicular (LSC), right infraclavicular (RIC), and left infraclavicular (LIC). The purpose of this study was to evaluate the relative effectiveness and complication risks of these 4 SVC locations in infants. Methods: In our pediatric intensive care unit, which is part of a tertiary medical center, a well-trained fellow doctor performed the following catheterizations: 21 RSC, 24 LSC, 24 RIC, and 22 LIC, for a total of 91 SVC operations in infants. The patients were placed in the Trendelenburg position. The site of puncture was decided by the operator. Statistical significance was analyzed according to Fisher's exact test and 2-sample t test. Results: The overall success rate was 90.1% (82 out of 91 operations). No statistically significant differences were noted among these 4 groups, either in the success or complication rate. There were 6 cases of arterial puncture (5 supraclavicular and 1 infraclavicular, p = 0.09), 2 cases of pneumothorax (1 RSC and 1 RIC), and 2 cases of malpositioned catheter (1 RSC and 1 RIC). There was no mortality. Conclusion: In our study, we found that there was no statistically significant difference among the 4 SVC locations in effectiveness of operation or in risk of complication. There was a tendency to damage the subclavian arteries through the supraclavicular route. (J Chin Med Assoc 2006;69(4):153-156)

To evaluate the screening performance of individual and combined use of clinical breast examination, ultrasonography and mammography in Chinese women, we conducted a biennial breast cancer screening program among 14,464 women aged 35 to 74 years old who lived in Qibao County, Minhang district of Shanghai, China, between May 2008 and Sept 2012. All participants were submitted to clinical breast examination, and then women with positive results and all women at age of 45-69 years old were preformed breast ultrasonography and mammography. The examination results were compared against pathological findings as the gold standard of reference. A total of 66 women were diagnosed with breast cancer in the two rounds of the screening, yielding an incident rate of 194 per 100,000 person-years. The sensitivity of clinical breast examination, ultrasonography and mammography alone were 61.4%, 53.7% and 67.3%, respectively. While mammography performed better in elder age groups and hormone receptor positive disease groups, ultrasonography had a higher sensitivity in younger age group and did not differ in sensitivity by estrogen receptor or progesterone receptor status. Combined use of the two imaging examinations increased the sensitivity in almost all age groups, but had a higher sensitivity in hormone receptor positive cancers than in those negative. Our results suggest that the Qibao modality is an effective strategy for breast cancer screening among Chinese women, especially for early detection of elder and hormone receptor positive breast cancer. Electronic supplementary material The online version of this article (doi:10.1186/2193-1801-2-276) contains supplementary material, which is available to authorized users.