Ying-Yao Chen

Kinetic evidence for human liver and stomach aldehyde dehydrogenase-3 representing an unique class of isozymes.

Substrate and coenzyme specificities of human liver and stomach aldehyde dehydrogenase (ALDH) isozymes were compared by staining with various aldehydes including propionaldehyde, heptaldehyde, decaldehyde, 2-furaldehyde, succinic semialdehyde, and glutamic γ-semialdehyde and with NAD+ or NADP+ on agarose isoelectric focusing gels. ALDH3 isozyme was isolated from a liver via carboxymethyl-Sephadex and blue Sepharose chromatographies and its kinetic constants for various substrates and coenzymes were determined. Consistent with the previously proposed genetic model for human ALDH3 isozymes (Yinet al., Biochem. Genet. 26:343, 1988), a single liver form and multiple stomach forms exhibited similar kinetic properties, which were strikingly distinct from those of ALDH1, ALDH2, and ALDH4 (glutamic γ-semialdehyde dehydrogenase). A set of activity assays using various substrates, coenzymes, and an inhibitor to distinguish ALDH1, ALDH2, ALDH3, and ALDH4 is presented. As previously reported in ALDH1 and ALDH2, a higher catalytic efficiency (Vmax/Km) for oxidation of long-chain aliphatic aldehydes was found in ALDH3, suggesting that these enzymes have a hydrophobic barrel-shape substrate binding pocket. Since theKm value for acetaldehyde for liver ALDH3, 83 mM, is very much higher than those of ALDH1 and ALDH2, ALDH3 thus represents an unique class of human ALDH isozymes and it appears not to be involved in ethanol metabolism.

Three-Combination Probiotics Therapy in Children With Salmonella and Rotavirus Gastroenteritis

Goals: Quantitative Vesikari scales and qualitative severe diarrhea (Vesikari scale ≥11) assessments were used to grade the Salmonella-induced and rotavirus-induced gastroenteritis severity. A significant reduction in severe diarrhea (Vesikari score ≥11) was used to evaluate the efficacy of three-combination probiotics (BIO-THREE). Background: Several studies have shown that rotavirus and Salmonella infections are the leading causes of infectious gastroenteritis. Although probiotics have been effective in some studies, the use of 3-combination formulation probiotics is rare. Study: This single-center, open-label, randomized, controlled trial included 159 patients (age range, 3 mo to 14 y) hospitalized with infectious gastroenteritis between February 2009 and October 2010. Results: Patients were grouped according to the pathogen identified (48, Salmonella; 42, rotavirus; and 69, unknown origin). The total diarrhea duration was significantly shorter for children who received BIO-THREE (P<0.0001). After BIO-THREE administration, there were significantly less intervention group patients with severe diarrhea at intervention day 3. Vesikari scale or diarrhea frequency results did not reveal significant differences between groups (except for day 5 in patients with rotavirus), and there were no significant changes in other clinical parameters or the length of hospital stay. Conclusions: Seven-day BIO-THREE administration demonstrated high efficacy and safety in infants and children with severe gastroenteritis. The incidence of severe gastroenteritis was significantly reduced in the rotavirus origin and BIO-THREE intervention groups.

Multiple Clinical Manifestations and Diagnostic Challenges of Incontinentia Pigmenti—12 Years' Experience in 1 Medical Center

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.

Pulmonary hemorrhage in very low‐birthweight infants: Risk factors and management

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.

Supraclavicular versus Infraclavicular Subclavian Vein Catheterization in Infants

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 Fishers 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.

Reappraisal of the Prostaglandin E1 Dose for Early Newborns with Patent Ductus Arteriosus-Dependent Pulmonary Circulation

OBJECTIVES The usual initial dose of prostaglandin E1 (PGE1) for ductal-dependent congenital heart disease (CHD) is 50-100 ng/kg/minute. The aim of this study was to review our experience of a low initial dose of PGE1 treatment in early newborns with congenital heart disease and patent ductus arteriosus (PDA)-dependent pulmonary flow. METHODS We reviewed the clinical data of 33 newborns with CHD and PDA-dependent pulmonary circulation who were admitted from January 2005 to December 2010. Clinical parameters were collected, including, PGE1 dosage, oxygenation condition, vital signs, and other related clinical parameters during admission. Echocardiography was employed to assess the status of the PDA as clinically indicated. RESULTS Thirty-three newborns, including 17 males and 16 females, with CHD and PDA-dependent pulmonary circulation were enrolled in the study. Their mean age was 2.9 ± 5.1 (within the range of 1-26) days with a median of 1.0 day. Among the 33 cases, 25 were diagnosed with pulmonary atresia and eight with critical pulmonary stenosis. Twenty-five of our patients were treated with the initial low-dosage regimen of 20.0 ± 7.4 ng/kg/minute in our neonatal intensive care unit. None of these 25 patients with had significant apnea necessitating intubation and none had hypotension, fever, convulsion or cortical hyperostosis. Three of the eight patients who were treated with high-dose PGE1 (39 ± 13.2 ng/kg/minute) before referral to our unit had apnea and intubation after PGE1 use. All patients had adequate PDA patency with a low maintenance dose of 10.5 ± 5.3 ng/kg/minute before operation under our protocol. CONCLUSION In our experience, adequate PDA flows in early newborns with CHD and PDA-dependent pulmonary circulation could be achieved at a much lower dose than recommended in the literature. The lower dose of PGE1 also causes much fewer complications, such as apnea, fever, and hypotension. For early newborns with CHD and PDA-dependent pulmonary circulation, treatment with a lower initial dose of PGE1 of 20 ng/kg/minute and a maintenance dose of 10 ng/kg/minute is recommended.

Subclavian Central Venous Catheterization in Infants with Body Weight Less Than 10 kg

Central venous access is an important aspect of medical treatment in intensive care units. We frequently require central venous catheterization (CVC) for total parenteral nutrition (TPN), intravenous antibiotics, multiple transfusions, and chemotherapy. The primary aim of this study is to demonstrate that percutaneous central venous catheterization in patients with body weight (BW) less than 10 kg can be conducted by the subclavian vein rather than the traditional femoral vein. Between January 1998 and December 2003, we performed 70 subclavian vein catheterizations (SVCs) in 46 patients with BW less than 10 kg in the pediatric intensive care unit (PICU) of a tertiary medical center. We divided patients according to their body weight into two groups, BW less than 5 kg and BW between 5 and 10 kg. We found SVC had a high total success rate, 92.9% (65/70), for the whole group. Success rate was 83.3% (15/18) for the BW less than 5 kg group and 96.2% (50/52) for the BW 5-10 kg group. In this study we found percutaneous subclavian venous catheterization in children with BW below 10 kg to be a relatively safe procedure with low risk of complication and no risk of mortality.

Urinary tract infection in infants: a single-center clinical analysis in southern Taiwan.

BACKGROUND This 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. METHODS The 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. RESULTS A 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<0.001) and higher C-reactive protein level (p<0.05). CONCLUSIONS E coli was the most common pathogen in the present cohort, and the top three pathogens were similar to those found in general pediatric population in Taiwan. VUR was the most common genitourinary tract anomaly in this age group. Positive DMSA was well correlated with VUR and higher C-reactive protein level.

Combined Methylmalonic Aciduria and Homocystinuria cblC Type of a Taiwanese Infant With c.609G>A and c.567dupT Mutations in the MMACHC Gene

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.

Perinatal factors in patent ductus arteriosus in very low-birthweight infants

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.