Lon-Yen Tsao

Publications of Lon-Yen Tsao

• Group B streptococcal infection in Taiwan: maternal colonization and neonatal infection.

  Authors: Hsiu-Wen Yu, Hsiao-Chuan Lin, Peng-Hong Yang, Chyong-Hsin Hsu, Wu-Shiun Hsieh, Lon-Yen Tsao, Chao-Huei Chen, Hung-Chih Lin, Ya-Chun Tseng

  Pediatrics and neonatology. 08/2011; 52(4):190-5.

  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 neonatalThere 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. 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. 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. 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.

• New plasma separation glucose oxidase-based glucometer in monitoring of blood with different PO2 levels.

  Authors: Ming-Sheng Lee, Lon-Yen Tsao, Yu-Ching Tseng, Shiu-Jung Tsai, Mei-Li Tsai, Tony Huang, Han-Yao Chiu

  Pediatrics and neonatology. 02/2011; 52(1):24-9.

  The PalmLab glucometer is a newly designed plasma separation glucose oxidase (GO)-based glucometer. Past studies have shown that the accuracy of GO-based glucometers is compromised when measurementsThe PalmLab glucometer is a newly designed plasma separation glucose oxidase (GO)-based glucometer. Past studies have shown that the accuracy of GO-based glucometers is compromised when measurements are taken in patients with high PO(2) levels. We performed a two-arm study comparing the fitness of the PalmLab blood glucometer with that of a standard glucose analyzer in monitoring blood glucose levels in pediatric patients, especially when arterial partial pressure of oxygen (PO(2)) was high. In the first arm of the study, arterial blood samples from pediatric patients were measured by the PalmLab blood glucometer and the YSI 2302 Plus Glucose/Lactate analyzer. In the second arm of the study, venous blood samples from adult volunteers were spiked with glucose water to prepare three different levels of glucose (65, 150, and 300mg/dL) and then oxygenated to six levels of PO(2) (range, 40-400mmHg). The biases of the PalmLab glucometer were calculated. A total of 162 samples were collected in the first arm of the study. Results of linear regression showed that the coefficient of determination (R(2)) between PalmLab glucometer and standard glucose analyzer was 0.9864. Error grid analysis revealed that all the results were within Zone A (clinically accurate estimate zone). The biases between the two systems were low at different PO(2) levels. In the second arm of the study, the results were also unaffected by changes in PO(2). The PalmLab glucometer provides accurate results in samples with high PO(2) and is suitable for measuring arterial glucose levels in pediatric patients.

• Dose effects of chronically infused nitric oxide synthase inhibitor N(G)-nitro-L-arginine methyl ester on anabolic response and arginine metabolism in rats with subacute peritonitis.

  Authors: Chien-Chou Hsiao, Chien-Hsing Lee, Lon-Yen Tsao, Hui-Chen Lo

  Biological & pharmaceutical bulletin. 01/2011; 34(2):177-82.

  Nitric oxide synthase (NOS) inhibitors alleviate the adverse effects of nitric oxide (NO) overproduction that occurs during peritonitis, a clinical condition that is accompanied by arginineNitric oxide synthase (NOS) inhibitors alleviate the adverse effects of nitric oxide (NO) overproduction that occurs during peritonitis, a clinical condition that is accompanied by arginine deficiency. However, the variations in the disease severity and the dosage, route, and period of NOS inhibitor administration are debatable. Therefore, we investigated the dose effects of chronically infused NOS inhibitor, N(G)-nitro-L-arginine methyl ester (L-NAME) on the anabolism, inflammatory responses, and arginine metabolism in parenterally fed rats with cecal puncture-induced subacute peritonitis. Male Wistar rats were divided into 4 groups and were administered total parenteral nutrition solutions with 0, 5 (low dose), 25 (medium dose), or 50 (high dose) mg·kg(-1)·d(-1) of L-NAME for 7 d. Sham-operated rats administered total parenteral nutrition solution and normal healthy rats fed chow diet were also included. Our results showed that parenteral infusion significantly decreased body weight gain and plasma citrulline concentrations. In rats with subacute peritonitis, the parenteral infusion-induced increases in circulating white blood cells and NO were significantly decreased, whereas the decrease in serum albumin levels was significantly increased. Rats with subacute peritonitis that were administered chronic infusion of L-NAME had a significantly reduced nitrogen balance. In addition, rats administered the medium dose of L-NAME had significantly increased plasma arginine, ornithine, glutamate, and proline. In conclusion, chronic infusion of NOS inhibitors may not alter systemic NO homeostasis and inflammatory response but may facilitate the production of arginine-associated amino acids and nitrogen excretion in cases of subacute peritonitis.

• Changing clinical presentations and survival pattern in trisomy 18.

  Authors: Chien-Chou Hsiao, Lon-Yen Tsao, Hsiao-Neng Chen, Han-Yao Chiu, Weng-Cheng Chang

  Pediatrics and neonatology. 08/2009; 50(4):147-51.

  BACKGROUND: The clinical presentations and survival patterns of infants with trisomy 18 have changed with increasing utilization of prenatal ultrasound and amniocentesis, and improvements in neonatalBACKGROUND: The clinical presentations and survival patterns of infants with trisomy 18 have changed with increasing utilization of prenatal ultrasound and amniocentesis, and improvements in neonatal intensive care. METHODS: We obtained data on duration of survival, male to female ratio, and clinical details for patients with trisomy 18, and calculated the prevalence rate. RESULTS: We studied 31 consecutive trisomy 18 infants. The estimated prevalence was 1/4, 144. Eleven (35%) were premature infants, and 20 (65%) were full term. Mean birth weight was 1896 g. Median life expectancy was 12 days; 11 days for males and 14 days for females (p = 0.87). The short-term survival rates of 1 week, 4 weeks, and 6 months were 58%, 32%, and 10%, respectively. The long-term survival rates of 1 year, 2 years, and 3 years were 6%, 6%, and 3%, respectively. Families signed do-not-resuscitate consent forms for five male (50%) and 19 female infants (90%) (p = 0.043). CONCLUSION: All trisomy 18 infants in this study were preterm or full-term deliveries. Mean birth weight was lower than previously reported, and a high percentage of families signed do-not-resuscitate consent forms. Females did not survive longer than males, due to more females not being resuscitated. Most infants died in the first few weeks of life, but 3-6% of infants lived for 21 year. The possibility of long-term survival should be considered when counseling parents regarding trisomy 18.

• Outcomes in neonates with pulmonary atresia and intact ventricular septum underwent pulmonary valvulotomy and valvuloplasty using a flexible 2-French radiofrequency catheter.

  Authors: Meng-Luen Lee, Lon-Yen Tsao, Han-Yao Chiu, Ming Chen, Ing-Sh Chiu

  Yonsei medical journal. 05/2009; 50(2):245-51.

  PURPOSE: Outcomes in 6 neonates with pulmonary atresia and intact ventricular septum (PAIVS) undergoing radiofrequency pulmonary valvulotomy and valvuloplasty (RPVV) were reported to identify thePURPOSE: Outcomes in 6 neonates with pulmonary atresia and intact ventricular septum (PAIVS) undergoing radiofrequency pulmonary valvulotomy and valvuloplasty (RPVV) were reported to identify the factors favorable for RPVV as the treatment of choice. MATERIALS AND METHODS: From May 2000 to January 2008, 6 patients with PAIVS were included in this retrospective study. They were aged 1 day to 90 days old. Study modalities included review of recordings of presentations and profiles of chest radiography, electrocardiography, echocardiography, and cardiac catheterization with angiography. Hemodynamic profiles from the echocardiography and the cardiac catheterization were analyzed. RESULTS: Echocardiography showed severe tricuspid regurgitation, membranous atresia of the pulmonary valve, intact ventricular septum, patent ductus arteriosus, and hypoplastic right ventricle in 6 patients. The pulmonary valve annulus were 4.2 to 6.9 mm in diameters, and those of the tricuspid valve were 7.1 to 10.1 mm. Elevated serum level of cardiac enzymes were found in 1 patient with ventriculocoronary communication (VCC). At cardiac catheterization, the ratio of systolic pressure of the right ventricle to that of the left ventricle ranged from 1.43 to 2.33 before RPVV, and from 0.54 to 1.15 after RPVV (p=0.027). The pressure gradients ranged from 76 to 136 mmHg before RPVV, and from 15 to 39 mmHg after RPVV (p=0.028). The echocardiographic gradients ranged from 16 to 32 mmHg within 24 hours after RPVV, and from 15 to 50 mmHg at the follow-ups. CONCLUSION: RPVV can be a treatment of choice for neonates with PAIVS, if there is patent infundibulum, no right-ventricular dependent coronary circulation, and adequate tricuspid valve and pulmonary valve.

• Late-onset Enterobacter cloacae sepsis in very-low-birth-weight neonates: experience in a medical center.

  Authors: Hsiao-Neng Chen, Meng-Luen Lee, Wai-Kit Yu, Yue-Wen Lin, Lon-Yen Tsao

  Pediatrics and neonatology. 03/2009; 50(1):3-7.

  OBJECTIVE: The objective of this study was to review the early clinical profiles and outcomes of very-low-birth-weight (VLBW) neonates with late-onset sepsis caused by Enterobacter cloacae. METHODS:OBJECTIVE: The objective of this study was to review the early clinical profiles and outcomes of very-low-birth-weight (VLBW) neonates with late-onset sepsis caused by Enterobacter cloacae. METHODS: We reviewed the medical records of VLBW neonates whose blood and/or cerebral spinal fluid yielded E. cloacae after 3 days of hospitalization in our neonatal intensive care unit. RESULTS: From January 1997 to December 2006, a total of 29 episodes of E. cloacae infection occurred in 28 VLBW neonates. The onset of E. cloacae infection ranged from 4 to 70 days (27.4 +/- 9.6) days after birth. The most common symptoms and signs of sepsis were desaturation (58.6%), tachycardia (58.6%), apnea (55.2%), unstable body temperature (48.3%), and decreased activity (44.8%). The infected neonates had an average of four kinds of symptoms in each sepsis episode. The most common laboratory findings in VLBW neonates with E. cloacae sepsis were thrombocytopenia (65.5%), C-reactive protein > or = 1 mg/dL (55.2%), band-form neutrophils > or = 5% (41.4%), and leucopoenia (20.7%). Empirical intravenous antibiotic therapy comprising piperacillin (or piperacillin and tazobactam) and gentamicin (or amikacin) was successful in the early treatment of 24 episodes of E. cloacae sepsis in 25 patients. Three neonates (10.7%) died due to E. cloacae sepsis. Four neonates (14.3%) developed E. cloacae meningitis, and two of them developed brain abscesses. CONCLUSIONS: E. cloacae infection in VLBW neonates usually presents with nonspecific symptoms and signs. Early recognition of sepsis and empirical combination of piperacillin (or piperacillin and tazobactam) and gentamicin (or amikacin) may be useful for treatment of sepsis caused by this highly virulent pathogen.

• Early or late surgical ligation of medical refractory patent ductus arteriosus in premature infants.

  Authors: Chien-Chou Hsiao, Jen-Tien Wung, Lon-Yen Tsao, Weng-Cheng Chang

  Journal of the Formosan Medical Association = Taiwan yi zhi. 01/2009; 108(1):72-7.

  Optimal time to surgical ligation of patent ductus arteriosus (PDA) in very-low-birth-weight (< 1500 g) premature infants remains an area of controversy. We compared the outcomes of early or lateOptimal time to surgical ligation of patent ductus arteriosus (PDA) in very-low-birth-weight (< 1500 g) premature infants remains an area of controversy. We compared the outcomes of early or late ligation of medical refractory PDA in very-low-birth-weight premature infants. Fifty-six infants underwent surgical closure of PDA after failure of or having contraindications to medical treatment. Thirteen infants were in the early ligation (</= 14 days) and 43 in the late ligation (> 14 days) groups. Basic clinical features, major morbidity of prematurity and mortality were compared. Clinical features and major outcomes were similar. The early ligation group had earlier onset of symptomatic PDA (5.7 +/- 1.6 days vs. 8.1 +/- 3.6 days, p = 0.024), and fewer days of total parenteral nutrition (TPN) (39.6 +/- 13.9 days vs. 60.4 +/- 31.4 days, p = 0.025) and ventilator use (11.1 +/- 6.7 days vs. 18.6 +/- 10.5 days, p = 0.019). Early ligation of medical refractory PDA in very-low-birth-weight premature infants improves enteral feeding tolerance and reduces TPN and ventilator use, but long-term benefits need further investigation.

• Phenotype and genotype of two Taiwanese cystic fibrosis siblings and a survey of delta F508 in East Asians.

  Authors: Chao-Jen Lin, Shun-Ping Chang, Yu-Yuan Ke, Han-Yao Chiu, Lon-Yen Tsao, Ming Chen

  Pediatrics and neonatology. 01/2009; 49(6):240-4.

  BACKGROUND: Cystic fibrosis (CF) is considered to be a rare disease in Asians. We report two cases of CF in a 5-year-old girl and her newborn brother. They are of mixed parentage: a Taiwanese motherBACKGROUND: Cystic fibrosis (CF) is considered to be a rare disease in Asians. We report two cases of CF in a 5-year-old girl and her newborn brother. They are of mixed parentage: a Taiwanese mother and an Australian father. METHODS: A comprehensive mutational analysis of the cystic fibrosis transmembrane conductance regulator (CFTR) gene was completed. Literature was reviewed for delta F508 in East Asians. RESULTS: Two mutation sites were identified in the siblings. The carrier status of their parents and elder brother were also confirmed: heterozygous delta F508 mutation from the father; 13 TG repeats in the IVS8-5T from the mother. An update of delta F508 mutation reported in East Asian patients from various ethnicities is included; most of them were of mixed parentage. CONCLUSION: These two cases are the first report of cystic fibrosis associated with a delta F508 mutation in a Taiwanese patient attributable to a mutation most commonly seen in Caucasians. We found that the delta F508 mutation is of western origin. Asian patients are seldom found with this mutation unless they are of mixed parentage. Our findings provide further evidence that different ethnicities have their own set of CFTR mutations.

• Endoscopic surgery for nasal glioma mimicking encephalocele in infancy.

  Authors: Chung-Lun Wu, Lon-Yen Tsao, Albert D Yang, Mu-Kuan Chen

  Skull base : official journal of North American Skull Base Society ... [et al.]. 12/2008; 18(6):401-4.

  Intranasal gliomas are challenging for several reasons. Their diagnosis may not be immediately obvious at presentation. It is important to exclude an intracranial extension, and for this some haveIntranasal gliomas are challenging for several reasons. Their diagnosis may not be immediately obvious at presentation. It is important to exclude an intracranial extension, and for this some have suggested a craniotomy. We report a 9-day-old male infant in whom an intranasal glioma that mimicked an encephalocele was successfully excised by endoscopic approach after failure to excise it through a craniotomy. After more than a 5-year follow-up, the patient is still free from the disease. An endoscopic intranasal approach provides a safe and effective method for the management of nasal glioma and does not result in postoperative facial scaring or deformity. Endoscopic techniques provide excellent visualization and are preferable to the classic frontal craniotomy to excluding intracranial extension of nasal glioma.

• Oral probiotics prevent necrotizing enterocolitis in very low birth weight preterm infants: a multicenter, randomized, controlled trial.

  Authors: Hung-Chih Lin, Chyong-Hsin Hsu, Hsiu-Lin Chen, Mei-Yung Chung, Jen-Fu Hsu, Rey-in Lien, Lon-Yen Tsao, Chao-Huei Chen, Bai-Horng Su

  Pediatrics. 11/2008; 122(4):693-700.

  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,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.

• Heat-shock response protects peripheral blood mononuclear cells (PBMCs) from hydrogen peroxide-induced mitochondrial disturbance.

  Authors: Han-Yao Chiu, Lon-Yen Tsao, Rei-Cheng Yang

  Cell stress & chaperones. 10/2008;

  The present study was designed to investigate ex vivo the protective mechanisms of heat-shock response against H(2)O(2)-induced oxidative stress in peripheral blood mononuclear cells (PBMCs) of rats.The present study was designed to investigate ex vivo the protective mechanisms of heat-shock response against H(2)O(2)-induced oxidative stress in peripheral blood mononuclear cells (PBMCs) of rats. Twenty-four hours later, heat-shock treatment was executed in vivo; rat PBMCs were collected and treated with H(2)O(2). The accumulation of reactive oxygen species and the mitochondrial membrane potential were evaluated by intracellular fluorescent dHE and JC-1 dye staining, respectively, and expression of HSP72 and cytochrome c was detected by Western blot analysis. Cellular apoptosis was assayed by TUNEL staining and double staining of Annexin V and PI. The results showed that H(2)O(2)-induced oxidative stress leads to intracellular superoxide accumulation and collapse of the mitochondrial membrane potential in rat PBMCs. Moreover, cellular apoptosis was detected after H(2)O(2) treatment, and the release of mitochondrial cytochrome c from mitochondria to cytosol was significantly enhanced. Heat-shock pretreatment decreases the accumulation of intracellular superoxide in PBMCs during H(2)O(2)-induced oxidative stress. Moreover, heat-shock treatment prevents the collapse of the mitochondrial membrane potential and cytochrome c release from mitochondria during H(2)O(2)-induced oxidative stress. In conclusion, mitochondria are critical organelles of the protective effects of heat-shock treatment. Cellular apoptosis during H(2)O(2)-induced oxidative stress is decreased by heat-shock treatment through a decrease in superoxide induction and preservation of the mitochondrial membrane potential.

• Acute metabolic decompensation and sudden death in Barth syndrome: report of a family and a literature review.

  Authors: Ting-Yu Yen, Wuh-Liang Hwu, Yin-Hsiu Chien, Mei-Hwan Wu, Ming-Tai Lin, Lon-Yen Tsao, Wu-Shiun Hsieh, Ni-Chung Lee

  European journal of pediatrics. 09/2008; 167(8):941-4.

  Barth syndrome presents in infancy with hypotonia, dilated cardiomyopathy, and neutropenia. We report a patient whose family history included two males who had died suddenly at the age of 15 days andBarth syndrome presents in infancy with hypotonia, dilated cardiomyopathy, and neutropenia. We report a patient whose family history included two males who had died suddenly at the age of 15 days and 2 years, respectively. The index case presented with acute metabolic decompensation at 13 days of age. Within 8 h of presenting with metabolic acidosis (pH 7.13), lactic acidemia (18.5 mmol/l), hyperammonemia (375 mug/dl), hypoglycemia (25 mg/dl), and coagulopathy, the patient developed respiratory failure and required intubation. The diagnosis was established by the presence of left ventricular noncompaction and molecular analysis (c.C153G or Y51X mutation of the TAZ gene). The gene product, taffazin, is a homologue of the glycerolipid transferases involved in the phospholipid metabolism as tetralinoleoyl-cardiolipin, a component of the mitochondrial inner membrane. In conclusion, mutations in taffazin impair mitochondrial respiratory chain complexes, which may results in the acute metabolic decompensation and sudden death; cardiac transplantation is the only possibility at the present time.

• Exchange Transfusion Using Peripheral Vessels Is Safe and Effective in Newborn Infants.

  Authors: Hsiao-Neng Chen, Meng-Luen Lee, Lon-Yen Tsao

  Pediatrics. 09/2008;

  OBJECTIVES. The purpose of this study was to compare the efficiency and safety of exchange transfusion by using peripheral arteries and veins with that of conventional exchange transfusion via theOBJECTIVES. The purpose of this study was to compare the efficiency and safety of exchange transfusion by using peripheral arteries and veins with that of conventional exchange transfusion via the umbilical vein in treating neonatal pathologic hyperbilirubinemia. PATIENTS AND METHODS. We retrospectively reviewed the medical charts of all neonates who had undergone exchange transfusion at our institution from January 1995 to December 2006. Causes of jaundice, efficiency of exchange transfusion in lowering serum bilirubin concentrations, adverse events, and outcomes were recorded. Data were compared between neonates who had undergone exchange transfusion via the peripheral arteries and veins method and those who had undergone exchange transfusion via the umbilical vein method. Data were also compared between stable neonates (body weight > 1500 g without medical problems other than jaundice) and unstable neonates. RESULTS. A total of 123 exchange-transfusion procedures were performed in 102 neonates in the 12-year study period: 24 were performed via the umbilical vein method and 99 via the peripheral vessels method. A total of 87 procedures were performed in 75 stable neonates and 36 in 27 unstable neonates. There was no significant difference in reduction of serum bilirubin level from circulation or the duration of procedures between the 2 methods. Eight neonates died before discharge, but none of the deaths seem to have been attributable to the exchange-transfusion procedure. Severe adverse events occurred more commonly in the umbilical vein group than the peripheral arteries and veins group in the stable neonates. All of the severe and minor events resolved completely without noticeable sequelae before discharge. CONCLUSIONS. Exchange transfusion using peripheral arteries and veins is efficient and effective in reducing serum bilirubin from circulation and is associated with few adverse events. This method should be considered for all neonates requiring exchange transfusion for treatment of neonatal hyperbilirubinemia.

• A systematic classification of the congenital bronchopulmonary vascular malformations: dysmorphogeneses of the primitive foregut system and the primitive aortic arch system.

  Authors: Meng-Luen Lee, Hung-Chi Lue, Ing-Sh Chiu, Han-Yao Chiu, Lon-Yen Tsao, Ching-Yuan Cheng, Albert D Yang

  Yonsei medical journal. 03/2008; 49(1):90-102.

  PURPOSE: We reviewed the cases of 33 patients from our clinic and 142 patients from the literature with congenital bronchopulmonary vascular malformations (BPVM), systematically analyzed thePURPOSE: We reviewed the cases of 33 patients from our clinic and 142 patients from the literature with congenital bronchopulmonary vascular malformations (BPVM), systematically analyzed the bronchopulmonary airways, pulmonary arterial supplies, and pulmonary venous drainages, and classified these patients by pulmonary malinosculation (PM). MATERIALS AND METHODS: From January 1990 to January 2007, a total of 33 patients (17 men or boys and 16 women or girls), aged 1 day to 24 years (median, 2.5 months), with congenital BPVM were included in this study. Profiles of clinical manifestations, chest radiographs, echocardiographs, esophagographs, computer tomography (CT), magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), cardiac catheterizations with angiography, contrast bronchographs, bronchoscopies, chromosomal studies, surgeries, and autopsies of these patients were analyzed to confirm the diagnosis of congenital BPVM. A total of 142 cases from the literature were also reviewed and classified similarly. RESULTS: The malformations of our 33 patients can be classified as type A isolated bronchial PM in 13 patients, type B isolated arterial PM in three, type C isolated venous PM in two, type D mixed bronchoarterial PM in five, type F mixed arteriovenous PM in one, and type G mixed bronchoarteriovenous PM in nine. CONCLUSION: Dysmorphogeneses of the primitive foregut system and the primitive aortic arch system may lead to haphazard malinosculations of the airways, arteries, and veins of the lung. A systematic classification of patients with congenital BPVM is clinically feasible by assessing the three basic bronchovascular systems of the lung independently.

• Persistent fifth aortic arch associated with 22q11.2 deletion syndrome.

  Authors: Meng-Luen Lee, Hsiao-Neng Chen, Ming Chen, Lon-Yen Tsao, Bao-Tyan Wang, Mei-Hui Lee, Ing-Sh Chiu

  Journal of the Formosan Medical Association = Taiwan yi zhi. 05/2006; 105(4):284-9.

  BACKGROUND: Chromosome 22q11.2 deletion is frequently associated with conotruncal malformations and aortic arch anomalies. This study investigated the association of chromosome 22q11.2 deletion withBACKGROUND: Chromosome 22q11.2 deletion is frequently associated with conotruncal malformations and aortic arch anomalies. This study investigated the association of chromosome 22q11.2 deletion with clinical manifestations in four pediatric patients with persistent fifth aortic arch. METHODS: Four patients with persistent fifth aortic arch treated between July 1997 and June 2004 were included in this retrospective study. There were two girls and two boys, aged 2 days to 11.3 years, with persistent fifth aortic arch and cardiac conotruncal malformations. Chart recordings, plain chest films, two-dimensional and Doppler echocardiograms, cardiac catheterization with angiograms, surgical findings, and cytogenetic study were analyzed. RESULTS: Clinically, all four patients had the cardinal phenotypic features of 22q11.2 deletion syndrome, including cardiovascular malformations (conotruncal malformations and aortic arch anomalies), abnormal facies, thymic hypoplasia, canopy anomaly of the palate (high-arched palate, rather than cleft palate), and hypocalcemia (or hypoparathyroidism). All four patients were confirmed to have chromosome 22q11.2 deletion. CONCLUSION: Congenital conotruncal malformations, including tetralogy of Fallot with pulmonary atresia or stenosis, and aortic arch anomalies including a persistent fifth aortic arch or a right aortic arch, should lead to suspicion of chromosome 22q11.2 deletion when manifested together with any one of the other four cardinal phenotypic features.

• Microchip, reverse transcription-polymerase chain reaction and culture methods to detect enterovirus infection in pediatric patients.

  Authors: Lon-Yen Tsao, Chi-Yung Lin, Ya-Yan Yu, Bao-Tyan Wang

  Pediatrics international : official journal of the Japan Pediatric Society. 02/2006; 48(1):5-10.

  BACKGROUND: Enterovirus infection usually presents with mild and self-limited illness in children. However, Enterovirus type 71 can be characterized by neurotropism and may cause severe illness orBACKGROUND: Enterovirus infection usually presents with mild and self-limited illness in children. However, Enterovirus type 71 can be characterized by neurotropism and may cause severe illness or even sudden death. Early detection of the virus will allow a physician to provide intensive or aggressive intervention. The purpose of the present study was to compare sensitivity of two innovative laboratory methods, that is, the DR.EV microchip method (DR. Chip Biotechnology, Shin-Tsu, Taiwan) and the reverse transcription-polymerase chain reaction (RT-PCR) method following conventional virus culture in detecting enterovirus infection in pediatric patients with herpangina or hand-foot-mouth disease. METHODS: A total of 87 children (age range: 1-8 years) were enrolled because of typical clinical findings of herpangina and hand-foot-mouth disease. Two hundred children selected after a careful clinical history review and physical examinations, were included as controls. All of these children had at least throat swab and rectal swab specimens taken and tested for evidence of enterovirus infection by microchip, RT-PCR and virus culture methods. In addition, 21 patients also had cerebrospinal fluid (CSF) specimens taken to test for possible central nervous system involvement. RESULT: The test results obtained from the 200 healthy kindergarten children were all negative for enteroviral infection by these three methods. Among the 87 test patients, the positive rates for throat swab, rectal swab and CSF by DR.EV chip, RT-PCR and virus culture were 71%, 68%, and 45% (throat swab); 66%, 61%, and 33% (rectal swab); and 52%, 29%, and 5% (CSF), respectively. There was no significant difference in the positive rates between the DR.EV chip and the RT-PCR methods (P > 0.1) on all types of specimens. However, statistically significant differences in positive rates were noted between the DR.EV chip and the conventional virus culture methods on all types of specimens (P < 0.001). Sensitivity of the microchip, RT-PCR and virus culture methods, was 82%, 72%, and 53%, respectively. CONCLUSION: The DR.EV chip method yielded a statistically higher positive rate and faster test results than the conventional viral culture method.

• Interventional cardiac catheterization for the coarctation of the aortic arch and patent ductus arteriosus in a 3-day-old neonate with the Holt-Oram syndrome.

  Authors: Meng-Luen Lee, Lon-Yen Tsao, Yu-Mei Wang

  International journal of cardiology. 07/2005; 101(3):503-5.

• Absence of hypercoagulability in acute Kawasaki disease.

  Authors: Ming-Tsan Lin, Lon-Yen Tsao, Ming-Lin Cheng, Yu-Jun Chang, Han-Yao Chiu, Hsiao-Neng Chen, Su-Feng Kuo, Shwu-Jin Chiou

  Pediatrics international : official journal of the Japan Pediatric Society. 05/2005; 47(2):126-31.

  BACKGROUND: Kawasaki disease (KD) is a systemic vasculitis syndrome with the striking feature of cardiovascular involvement. Endothelial cell (EC) damage has been suggested to predispose individualsBACKGROUND: Kawasaki disease (KD) is a systemic vasculitis syndrome with the striking feature of cardiovascular involvement. Endothelial cell (EC) damage has been suggested to predispose individuals to the development of coronary vascular disorders. When EC are perturbed, prethrombotic complications ensue. The purpose of this study was to examine the clinical relevance of EC activation and hypercoagulability in the pathogenesis of KD and to determine if plasma levels of these markers are correlated with the development of coronary aneurysms. METHODS: EC function and coagulation status were assessed in 52 patients with acute KD, 20 febrile control subjects, and 20 healthy control subjects. Biological markers of EC and hypercoagulability were measured and included thrombomodulin, tissue factor, tissue factor pathway inhibitor, von Willebrand factor (vWF), coagulation factor VII (FVII), activated factor VII, prothrombin fragment 1 + 2 (F1 + 2), and D-dimer. RESULTS: Transient dilatation of coronary arteries was the most common complication (55.8%), and coronary aneurysm was noted in five patients (9.6%). Levels of vWF, FVII, F1 + 2 and D-dimer were higher in acute KD patients compared with healthy controls but not febrile controls. Markers of EC and hypercoagulability were not different between patients with cardiac complications and those without cardiac complications. Biological and immunological assays did not demonstrate the prethrombotic state in acute KD. CONCLUSIONS: Our results suggest that hypercoagulability does not occur during the acute stage of KD. Markers of EC damage and hypercoagulability are not predictive of coronary aneurysms in KD.

• Revisit on a distinctive chromosome 1p36 deletion syndrome: report of one case and review of the English literature.

  Authors: Meng-Luen Lee, Lon-Yen Tsao, Bao-Tyan Wang, Mei-Huei Lee, Ing-Sh Chiu

  International journal of cardiology. 10/2004; 96(3):477-80.

• Maternally inherited unbalanced translocation of chromosome 22 in a 5-day-old neonate with persistent fifth aortic arch and tetralogy of Fallot.

  Authors: Meng-Luen Lee, Lon-Yen Tsao, Bao-Tyan Wang, Mei-Huei Lee, Ing-Sh Chiu

  International journal of cardiology. 09/2003; 90(2-3):337-9.