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Biomedical subjects

Wu-Shiun Hsieh

Publications and source records attributed to Wu-Shiun Hsieh.

At least 19 recordsLinked to original sources

Chinese herbal medicines used in pregnancy: a population-based survey in Taiwan.

PURPOSE: To explore the use (prevalence and related factors) of Chinese herbal medicines used by pregnant women. METHODS: We used multistage stratified systematic sampling to recruit 2048 postpartum women from the Taiwan national birth register between November and December 2003. Subjects underwent a home interview 6 months after their deliveries. A structured questionnaire was successfully applied to 87% of the sampled population. RESULTS: At least one Chinese herbal medicine was used during pregnancy by 24.1% of the interviewed subjects. Pregnant women with threatened abortion appeared to use more Chinese herbal medicines than other pregnant women. CONCLUSION: Chinese herbal medicines are frequently used by pregnant women in Taiwan and are very frequently used by those with a threatened abortion.

Abortion, Threatened↗

Vestibular evoked myogenic potentials in newborns.

This study presents a novel method for recording vestibular evoked myogenic potential (VEMP) in newborns, used to investigate the maturation of sacculocollic reflex at birth. Twenty full-term newborns aged 2-5 days old were enrolled in this study. During natural sleep, each newborn underwent distortion product otoacoustic emission test, and VEMP test using the head rotation method. For comparison, 20 healthy adults also underwent VEMP test using the same method. Based on adult criteria, 40 newborn ears revealed normal VEMPs in 40%, prolonged VEMPs in 35%, and absent VEMPs in 25%, indicating that great variation exists in the maturation of the sacculocollic reflex at birth. Comparison of VEMP characteristics between healthy newborns and adults revealed nonsignificant difference in the response rate and the latency of n23. However, significant differences existed in the latency of p13, interpeak p13-n23 interval and p13-n23 amplitude between newborns and adults. In conclusion, VEMPs in newborns can be easily recorded by the head rotation method. Prolonged or absent VEMPs in newborns may reflect incomplete maturity of the sacculocollic reflex pathway, especially the myelination. A further large number of newborns receiving MRI scan for other reasons may undergo VEMP test to verify this hypothesis.

Acoustic Stimulation↗

Quantitative assay of deletion or duplication genotype by capillary electrophoresis system: Application in Prader-Willi syndrome and Duchenne muscular dystrophy.

BACKGROUND: Deletions and duplications involving large DNA segments result in underexpression or overexpression, depending on the changes in allele dose, and are known to cause many common disorders. Detection of allele dose variations in the human genome is increasingly important in medical genetic diagnosis. METHODS: We used multiplex quantitative PCR coupled with capillary electrophoresis for accurate allele dose determination. In cases of Prader-Willi syndrome (PWS), a total of 24 patients with PWS, as well as 205 control individuals from the general population, were analyzed by use of multiplex quantitative PCR to amplify the FGFR2 gene, the KRIT1 gene, and the SNRPN gene simultaneously. In cases of Duchenne muscular dystrophy (DMD), we optimized the multiplex quantitative PCR to amplify 38 exons to analyze the DMD gene for rapid diagnosis of 12 DMD-affected males, 12 obligate carriers from families, and 50 unaffected female controls. RESULTS: We were able to unambiguously diagnose the deletion genotype in PWS patients and identify all deletion or duplication genotypes and carrier status in DMD-affected cases with 100% sensitivity and specificity. CONCLUSIONS: This report describes a novel single assay that can rapidly quantify allele dose to provide accurate clinical genetic diagnosis. This technique offers a valuable alternative for the rapid detection of genomic deletions or duplications and decreases costs because it does not require expensive fluorescent reagents.

Autoantigens↗

Severe obstetric complications and birth characteristics in preterm or term delivery were accurately recalled by mothers.

OBJECTIVE: This study examined the accuracy of maternal recall of obstetric complications and birth characteristics and its determinants for both preterm and term deliveries 3-9 years ago. METHODS: In 101 preterm and 107 term deliveries at the National Taiwan University Hospital during 1995-2000, recall data were obtained by telephone interview with the mothers and were matched with medical records. RESULTS: Among 10 obstetric complications assessed, the accuracy of maternal recall could either have high sensitivity and high specificity (Cesarean section, gestational hypertension, and induced labor), low to moderate sensitivity and high specificity (pre-eclampsia, breech, and cord loops), or low sensitivity and low specificity (ante partum vaginal bleeding, edema, and proteinuria). The correlations between maternal recall and medical records for birth weight (r = .95) and gestational age (r =.93) in the preterm group were slightly higher than those in the term group (r = .89 and .83, respectively). Factors associated with higher recall accuracy included preterm delivery, first birth order, and lower total parity, but no factor consistently related to maternal accuracy for all obstetric complications and birth characteristics. CONCLUSION: The accuracy of maternal recall on obstetric complications varied depending on the nature of complications examined, whereas that on birth characteristics was high.

Birth Weight↗

Clinical manifestations and neurodevelopmental outcome following an event of accidental intramuscular injection of atracurium in newborns.

UNLABELLED: Pediatric populations are at risk for medication errors that may be associated with mortality and disability. The purpose of this study was to describe the clinical manifestations of seven newborns following an event of accidental intramuscular injection of atracurium and to assess the impact on neurodevelopmental outcome. This study enrolled seven newborns who were accidentally administered 10 mg of atracurium, equivalent to 2.6-3.3 mg/kg, in a local perinatal clinic. Accident reports and hospital records were reviewed to obtain the history and medical data for the event. The survivors were prospectively examined for their growth, health and neurodevelopment until 24 months of age. All newborns showed immediate apnea and cyanosis requiring resuscitation after atracurium injection and presented with respiratory failure and flaccid paralysis on arrival for emergency medical services. One newborn was asystolic despite resuscitation and died of multiple organ failure. Of the five survivors available for follow-up, all achieved favorable growth outcomes. However, four showed mild to significant delay in development; and two manifested mild hypomyelination of cerebral white matter on the brain magnetic resonance imaging. CONCLUSION: Newborns accidentally injected with high doses of atracurium are at risk of death and neurodevelopmental delay. The serious clinical manifestations, developmental delay and cerebral hypomyelination were most likely due to insufficient immediate respiratory assistance following atracurium injection.

Atracurium↗

Congenital hemangiopericytoma in a neonate.

Hemangiopericytoma is a rare malignant vascular tumor that usually occurs in adults. The occurrence of these tumors in infants, known as congenital or infantile hemangiopericytoma, is even rarer and their behavior may be more benign than the adult type. We describe a 1-day-old female neonate with congenital hemangiopericytoma, presenting with a right inguinal mass at birth. At the time of surgery, lymphangioma was suspected because of its appearance, fluid-filled multicystic content, and the high incidence of this disease in pediatric patients. Tumor excision was performed and hemangiopericytoma was diagnosed by histology. There was no tumor recurrence during 12 months of follow-up.

Abdominal Neoplasms↗

[Applying health beliefs to mothers who join in newborn hearing screening].

This study explores the potential of applying health belief models to studies of mothers who participate in newborn hearing screening (NHS) in order to establish a reference for the future research. The study was designed prospectively, with 653 effective samples enrolled between November 1991 and April 1992 and data collected using a self-constructed questionnaire. The return rate for the questionnaire was 68.6%. Main conclusions include: (1) the decision to participate in newborn hearing screening correlates in a significant manner with potential participant backgrounds and actions clues; (2) "knowledge of NHS" and "perceived benefits of action in NHS" were the most important factors in this study associated with NHS participation; (3) subjects who participated in the NHS demonstrated significantly higher scores in "knowledge of NHS" and "perceived benefits of action in NHS" than those who did not participate. Significant negative correlation was identified between education level and perceived barriers to joining NHS. Significant positive correlation was observed between education level and NHS knowledge. Also, a significant positive relationship was identified between "perceived benefits of action in NHS" and both "clues of inner action" and "knowledge of NHS". However, "perceived barriers to NHS action" was found to share no significant relationship with any of the others. A positive relationship was also identified between "perceived benefits of action in NHS" and both "clues of outer action" and "amount of information resources"; and (4) "clues of outer action", "NHS knowledge", and "perceived benefits of NHS action" were found to be the most important indicators affecting study subjects' decision regarding whether or not to join NHS.

Adult↗

Cutis marmorata telangiectatica congenita with gangrenous ulceration and hypovolaemic shock.

UNLABELLED: Cutis marmorata telangiectatica congenita (CMTC) is an uncommon sporadic congenital vascular anomaly characterised by persistent cutis marmorata, telangiectasia, and phlebectasia. The disease usually has a benign clinical course and over 50% of cases recover spontaneously without specific treatment. The occurrence of life-threatening complications in CMTC is rare. CONCLUSION: We report a neonate with life-threatening cutis marmorata telangiectatica congenita which was complicated by gangrenous ulceration, bleeding episodes, and hypovolaemic shock.

Capillaries↗

Quantitative analysis of SMN1 and SMN2 genes based on DHPLC: a highly efficient and reliable carrier-screening test.

Autosomal recessive spinal muscular atrophy (SMA) is a common, fatal neuromuscular disease caused by homozygous absence of the SMN1 gene in approximately 94% of patients. However, a highly homologous SMN2 gene exists in the same chromosome interval, centromeric to SMN1, and hampers detection of SMN1. We present a new, rapid, simple, and highly reliable method for detecting the SMN1 deletion/conversion and for determining the copy numbers of the SMN1 and SMN2 genes by DHPLC. We analyzed SMN1/SMN2 gene exon 7 deletion/conversion by DHPLC. A total of 25 patients with spinal muscular atrophy lacking the SMN1 gene as well as 309 control individuals from the general population and the family members of patients with SMA were analyzed. By DHPLC analysis, we could detect the SMA-affected cases efficiently just by recognizing an SMN2-only peak. Furthermore, after specific primer amplification and adjustment of the oven temperature, all of the SMA carriers with an SMN1/SMN2 ratio not equal to 1 could be identified unambiguously by this simple and efficient detection system. To calculate the total SMN1/SMN2 gene dosages further, we developed a specific multiplex competitive PCR protocol by simultaneously amplifying the CYBB gene (X-linked), the KRIT1 gene (on chromosome arm 7q), and the SMN1/SMN2 gene ratio by DHPLC. By applying this technique, we could successfully designate all of the genotypes with different SMN1/SMN2 gene copy numbers, including equal and unequal amounts of SMN1 and SMN2. We demonstrated that DHPLC is a fast and reliable tool for detection of carriers of SMA.

Chromatography, High Pressure Liquid↗

Vascular endothelial growth factor in preterm infants with respiratory distress syndrome.

Respiratory distress syndrome (RDS) secondary to surfactant deficiency is a common cause of morbidity and mortality in premature infants. Increasing evidence suggests that vascular endothelial growth factor (VEGF) may contribute to surfactant secretion and pulmonary maturation. However, differences in cord blood VEGF concentrations in infants with and without respiratory distress syndrome have not been reported. We hypothesized that premature infants with higher VEGF levels in cord blood had a lower risk of developing RDS. Cord blood samples were obtained from preterm infants born at 32 weeks of gestation or earlier. Infants were excluded if there was evidence of prenatal maternal infection or any infection within the first 3 days of life. Cord blood VEGF levels were measured using an enzyme-linked immunosorbent assay (ELISA). We found that neonates with clinically diagnosed RDS had a lower gestational age (GA), lower birth weight (BW), higher incidence of mechanical ventilation requirements, longer duration of mechanical ventilation, and lower Apgar scores at 1 and 5 min. Infants with RDS had significantly lower cord blood VEGF levels. GA, BW, premature rupture of membranes (PROM), and antenatal steroid treatment were not associated with changes in cord blood VEGF levels. The specificity of cord blood VEGF above 34 pg/ml for predicting the absence of RDS was 86%, the sensitivity was 53%, the positive predictive value was 84%, and the negative predictive value was 56%. Our data demonstrated that cord blood VEGF elevation was significantly correlated with an absence of RDS.

Apgar Score↗

Excess soluble fms-like tyrosine kinase 1 and low platelet counts in premature neonates of preeclamptic mothers.

OBJECTIVE: To investigate the relationship of neonatal platelet count and preeclampsia to levels of soluble fms-like tyrosine kinase 1 (sFlt-1), placental growth factor (PlGF), and vascular endothelial growth factor (VEGF) in the cord blood of preterm infants. METHODS: Sixty-nine preterm infants with a gestational age between 26 and 37 weeks at birth were enrolled. sFlt-1, PlGF, and VEGF levels in the cord blood of preterm neonates, with or without maternal preeclampsia, were measured using a standardized sandwich enzyme-linked immunosorbent assay method. RESULTS: Infants with maternal preeclampsia had higher cord blood sFlt-1 but lower PlGF and VEGF levels. There was a significantly positive relationship between neonatal platelet count and birth weight and a significantly negative relationship between neonatal platelet count and cord blood sFlt-1 levels. Multiple regression analysis revealed that only birth weight and cord blood sFlt-1 levels were independently related to neonatal platelet count, whereas maternal preeclampsia, gestational age (GA), and small for GA (SGA) were not related. Neonates with thrombocytopenia had higher cord blood sFlt-1 levels but lower birth weight. A significant correlation was also found between birth weight and cord blood sFlt-1 levels. Multiple regression with birth weight as the dependent variable found that only GA and cord blood sFlt-1 levels were independently related. Furthermore, infants with high cord blood sFlt-1 were more likely to have lower platelet count and maternal preeclampsia, be SGA, and have a trend toward lower birth weight. CONCLUSION: Excess sFlt-1 may play an important role in the development of maternal preeclampsia- induced neonatal thrombocytopenia, and SGA.

Birth Weight↗

Persistent pulmonary hypertension in a neonate with vein of Galen arteriovenous malformation.

Vein of Galen aneurysmal malformation (VGAM) often leads to death in the neonatal period, mainly due to congestive heart failure. Chronic and excessive pulmonary flow in utero and postnatally is attributed to large VGAM and right ventricular overload. We report a male neonate with VGAM complicated by severe heart failure and persistent pulmonary hypertension. Endovascular coil embolization of VGAM was performed, resulting in improvement of congestive heart failure; however, severe persistent pulmonary hypertension led to death 2 days after the embolization. Postmortem examination showed marked right and left ventricular hypertrophy and impressive muscular thickening of intra-alveolar arterioles. Neonatal embolization seems to be beneficial only in babies without suprasystemic pulmonary hypertension. Therefore, early delivery and repair of VGAM should be considered before the onset of persistent pulmonary hypertension.

Humans↗

Ursodeoxycholic acid (UDCA) therapy in very-low-birth-weight infants with parenteral nutrition-associated cholestasis.

OBJECTIVE: To determine the effect of ursodeoxycholic acid (UDCA) in very-low-birth-weight (VLBW) infants with parenteral nutrition-associated cholestasis (PNAC). STUDY DESIGN: A retrospective study of all VLBW infants with PNAC who were admitted to a tertiary referral center was conducted. Patients were classified as treatment group (receiving UDCA within 14 days after onset of cholestasis) or control group (no medical treatment). Patients who received abdominal surgery were excluded. RESULTS: A total of 30 patients were recruited, including 12 in the treatment group and 18 in the control group. The demographic data, total fasting duration, onset of cholestasis, age to tolerance of full feeds, and the duration of parenteral nutrition (PN) before the onset of cholestasis were comparable between the two groups. There was a trend in the control group to later onset of cholestasis. The patients who received UDCA therapy with doses of 10 to 30 mg/kg/day had a shorter duration of cholestasis than the control group (62.8 vs 92.4 days, P=.006). Furthermore, the peak serum levels of direct bilirubin also was significantly lower in the treatment group. CONCLUSION: UDCA can improve the course of PNAC in VLBW infants.

Bilirubin↗

Congenital esophageal stenosis owing to ectopic tracheobronchial remnants.

BACKGROUND/PURPOSE: Congenital esophageal stenosis owing to tracheobronchial remnants (TBR) is a rare condition. Inappropriate treatment often is carried out before the correct diagnosis is established. For a better understanding and management of this disease, patients with TBR treated at our hospital and collected from the literature are reviewed to evaluate the course of onset, associated anomalies, methods of diagnosis and treatment, and outcomes. METHODS: Three patients treated at our hospital and 59 patients identified from the literature were included in the study. Gender, age at onset of symptoms, age at definitive treatment, esophagographic findings, pathology, methods of treatment, and outcomes of the 62 patients were recorded and analyzed. RESULTS: Boys slightly predominated (33:28, 1 unknown gender). Symptoms of dysphagia and regurgitation developed at the mean age of 3.2 +/- 4.5 months. Definitive treatment was carried out at the mean age of 2.6 +/- 3.0 years with a time lag of 2.0 +/- 2.5 years from the onset of symptoms. Twenty-five patients had associated anomalies with esophageal atresia being the most prevalent. Esophagography showed segmental stenosis over the distal third of the esophagus in all patients except one. The esophagographic findings could be classified into 3 types: type Ia, 10 cases; Ib, 15 cases; type II, 14 cases; type III, 3 cases. A nonyielding esophageal stenosis without inflammation was the characteristic esophagoscopic finding. Esophagoscopic dilatation of the stenosis was attempted in 26 patients, but all failed, and 2 patients suffered esophageal perforation. Surgical resection was mandatory for all patients to restore their esophageal continuity. Postoperative complications included anastomotic stenosis, anastomotic leakage, hiatal hernia, and gastroesophageal reflux. CONCLUSIONS: TBR should be suspected in patients who present with a typical history of dysphagia after ingestion of solid food and have characteristic esophagographic and esophagoscopic findings. It has a strong tendency to occur with esophageal atresia. Esophagoscopic dilatation is ineffective and may render the patient at risk for esophageal perforation. Operation is the treatment of choice and carries little morbidity and mortality.

Anastomosis, Surgical↗

Outcome of very low birth weight infants with sonographic enlarged occipital horn.

The objective of this study is to compare the neurodevelopmental outcome between very low birth weight infants with and without sonographic disproportionate enlargement of occipital horn. We retrospectively reviewed the brain sonography of all very low birth weight infants born at National Taiwan University Hospital between June 1997 and June 1999. Brain sonography was routinely performed at the age of the third, seventh, twenty-first, and later days as clinically indicated. Intracranial hemorrhage, periventricular leukomalacia, congenital hydrocephalus, and Stage III retinopathy of prematurity were excluded from our study because of the association with neurodevelopmental impairment. Patients with disproportional dilatation of occipital horn more than 15 mm in width were included in the study group, and those with less than 15 mm were in the control group. Both groups received developmental evaluation by the Bayley Scales of Infant Development II at corrected age of 6, 12, 18, and 24 months, respectively. Socioeconomic status and detailed medical history were obtained at assessments. Independent-samples t test was used for comparison. A total of 81 very low birth weight infants were included in this study: 49 infants (female 18, male 31) in the study group and 32 infants (female 23, male 9) in the control group. The mean gestation in these two groups was 30 +/- 2 weeks and 31.1 +/- 2.2 weeks (P = 0.156), and the mean birth body weight was 1290 +/- 269 gm and 1282 +/- 219 gm (P = 0.877), respectively. At corrected age of 24 months, there was no significant difference in muscle tone, neuromotor impairment, hearing impairment, vision, or speech development. Assessment with the mental development index (88.9 +/- 15.6 vs 93 +/- 13.2) (P = 0.238) and the psychomotor development index (93.3 +/- 10.3 vs 89.6 +/- 12.1) (P = 0.149) between these two groups was also comparable. This retrospective analysis suggests that ultrasonographic disproportionate enlargement of the occipital horn in very low birth weight infants does not affect the neuromotor development at corrected ages of 6, 12, 18, and 24 months.

Female↗