Huge, alarming congenital hemangioma of the scalp presenting as heart failure and Kasabach-Merritt syndrome: a case report.
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Cardiac Output, Low↗
Biomedical subjects
Po-Nien Tsao
Publications and source records attributed to Po-Nien Tsao.
The influence of biologic factors on the surgical decision in advanced neuroblastoma.
BACKGROUND: Molecular markers greatly affect the outcome of neuroblastoma. This study evaluated the influence of Trk-A and myelocytomatosis viral-related oncogene, neuroblastoma-derived (MYCN) on the role of surgery in advanced neuroblastoma. METHODS: Ten stage 3 and 35 stage 4 neuroblastoma patients were included. Tumor resection was classified into gross total resection (GTR) and incomplete resection. Patients were classified into three biological risk groups according to Trk-A expression and myelocytomatosis viral-related oncogene, neuroblastoma-derived (MYCN) status in tumor tissues studied by immunohistochemistry and fluorescence in situ hybridization, respectively: low risk (positive Trk-A and normal MYCN), intermediate risk (negative Trk-A and normal MYCN), and high risk (positive or negative Trk-A and MYCN amplification). The effect of tumor resection on prognosis was studied and stratified according to the risk grouping. RESULTS: GTR was achieved in 21 patients (46.7%) with a higher complication rate (33% vs. 8% in the incomplete resection group, P = .036). GTR was easier to achieve in low-risk tumors than in intermediate- or high-risk tumors (12 of 13, 4 of 17, and 5 of 15, respectively; P < .001). GTR predicted a favorable prognosis for intermediate-risk patients (P = .037; log-rank test), but not for low- or high-risk patients because of the overall favorable and poor prognosis, respectively. CONCLUSIONS: GTR carries a potentially higher possibility of complication. Although GTR can be achieved easily in low-risk neuroblastoma patients with a favorable prognosis, surgeons should do their best to achieve GTR for intermediate-risk patients to improve outcome. Nevertheless, sacrificing vital organs to achieve GTR for high-risk patients is not justified.
Biomarkers, Tumor↗
Necrotizing pneumococcal pneumonia in children: the role of pulmonary gangrene.
Little is known about the mechanism of necrotizing pneumonia caused by Streptococcus pneumoniae in children. Pulmonary gangrene secondary to vascular thrombosis was reported in adults with necrotizing pneumococcal pneumonia. We conducted a retrospective study of 15 children with a diagnosis of necrotizing pneumococcal pneumonia at National Taiwan University Hospital to explore its association with pulmonary gangrene, based on evidence from chest computed tomography, serial chest radiographic patterns, and pathologic results. S. pneumoniae serotype 14 was the prevalent pneumococcal serotype. Overall, 63.6% of isolates were not susceptible to penicillin. One child with pneumonia caused by S. pneumoniae serotype 3 complicated by hemolytic uremic syndrome had a rapidly fatal course. An autopsy in this patient documented lung necrosis and pulmonary gangrene. Radiographic follow-up was performed during the clinical course in 9 patients, and showed no evidence of pulmonary gangrene. Four children had no radiographic follow-up. The relationship between pulmonary gangrene and necrotizing pneumonia was unclear in the remaining one. In conclusion, necrotizing pneumococcal pneumonia may be infrequently associated with pulmonary gangrene in children.
Child↗
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↗
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↗
GRP78 expression correlates with histologic differentiation and favorable prognosis in neuroblastic tumors.
Glucose-regulated protein 78 (GRP78), an endoplasmic reticulum protein, is essential for the differentiation of neuroblastoma cells and is selectively induced when the cells are undergoing apoptosis. These findings suggest that GRP78 may affect the tumor behavior of neuroblastoma. Our study evaluates the association of clinicopathologic factors and patient survival with the expression of GRP78 in patients with neuroblastoma. GRP78 expression in 68 neuroblastic tumors was investigated semiquantitatively by immunohistochemistry. GRP78 mRNA and protein levels in 7 tumor tissues were also quantified by real-time PCR and Western blot respectively and correlated well with the immunohistochemical results. Forty (58.8%) of the 68 neuroblastic tumors showed positive GRP78 expression. The percentage of positive GRP78 immunostaining increased as the tumor histology became differentiated (p = 0.001). Furthermore, positive GRP78 expression strongly correlated with early clinical stages (P = 0.002) but inversely correlated with MYCN amplification (p = 0.001). Kaplan-Meier analysis showed that patients with positive GRP78 expression did have better survival than those with negative expression (5-year survival rate, 72.9% and 23.4% respectively, p < 0.001). Multivariate analysis further showed that GRP78 expression was an independent prognostic factor. Moreover, GRP78 expression predicted better survival in patients with either undifferentiated or differentiated histologies. GRP78 expression still had significant prognostic value when the analysis was restricted to tumors of advanced stages or without MYCN amplification. Thus, GRP78 can serve as a novel independent favorable prognostic factor for patients with neuroblastoma.
Base Sequence↗
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↗
Meconium peritonitis presenting as isolated massive fetal ascites.
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Adult↗
Delivery before 32 weeks of gestation for maternal pre-eclampsia: neonatal outcome and 2-year developmental outcome.
BACKGROUND AND OBJECTIVE: In the literature, there are conflicting data on the neonatal outcome in preterm infants who were delivered for maternal pre-eclampsia. The purpose of this study is to investigate the effect of maternal pre-eclampsia on neonatal morbidity and 2-year developmental outcome in a population of preterm infants delivered before 32 weeks of gestation. METHODS: The hospital records of all 89 surviving VLBW infants with GA below 32 weeks born from January 1997 to December 1999 were reviewed retrospectively. Data on respiratory outcome, sepsis and intraventricular hemorrhage (IVH) were compiled and analyzed for their association to maternal pre-eclampsia. Seventy-eight infants were assessed employing the Bayley Scales of Infant Development for developmental outcome at 2 years of corrected age. RESULTS: There was no difference in neonatal morbidity between groups. More infants born to pre-eclamptic mothers had lower MDI scores at 24 months of age (P=0.04) as compared to infants without maternal pre-eclampsia. After multiple logistic regression analysis, pre-eclampsia (P=0.007, OR=10.8) remained a significant risk factor of mildly delayed MDI at 24 months of age. CONCLUSION: Delivery before 32 weeks because of pre-eclampsia was associated with an increased risk of poor cognitive outcome. There was no significant difference in the postnatal course in comparison with infants born after pregnancies not complicated by pre-eclampsia.
Adult↗
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↗
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↗
Handwashing program for the prevention of nosocomial infections in a neonatal intensive care unit.
OBJECTIVE: To evaluate the effects of a hand hygiene program on compliance with hand hygiene and the rate of nosocomial infections in a neonatal intensive care unit (NICU). DESIGN: Open trial. SETTING: A level-III NICU in a teaching hospital. PARTICIPANTS: Nurses, physicians, and other healthcare workers in the NICU. INTERVENTIONS: A multimodal campaign for hand hygiene promotion was conducted beginning in September 1998. This program consisted of formal lectures, written instructions and posted reminders regarding hand hygiene and proper handwashing techniques, covert observation, financial incentives, and regular group feedback on compliance. Surveillance of handwashing compliance and nosocomial infections before and during the program was analyzed. RESULTS: Overall compliance with hand hygiene improved from 43% at baseline to 80% during the promotion program. The rate of nosocomial infections decreased from 15.13 to 10.69 per 1,000 patient-days (P = .003) with improved handwashing compliance. In particular, respiratory tract infections decreased from 3.35 to 1.06 per 1,000 patient-days during the handwashing campaign (P = .002). Furthermore, the correlation between nosocomial infection of the respiratory tract and handwashing compliance also reached statistical significance (r = -0.385; P = .014). CONCLUSIONS: Improved compliance with handwashing was associated with a significant decrease in overall rates of nosocomial infection and respiratory infections in particular. Washing hands is a simple, economical, and effective method for preventing nosocomial infections in the NICU.
Cross Infection↗
Placenta growth factor elevation in the cord blood of premature neonates predicts poor pulmonary outcome.
OBJECTIVE: To determine whether an elevated placenta growth factor (PlGF) level in cord blood is associated with increased risk for preterm infants to develop bronchopulmonary dysplasia (BPD). METHODS: Sixty-three preterm infants who were born at 34 weeks' gestation or earlier were enrolled. Two infants who died before 28 days' postnatal age could not be assigned a BPD status and were excluded. PlGF levels in cord blood were measured using enzyme-linked immunosorbent assay. Mann-Whitney rank sum test, Spearman correlation coefficients, and multivariable linear or logistic regression analyses were used for statistical analysis. RESULTS: The BPD group had a higher PlGF level, lower gestational age, lower birth weight (BW), higher incidence of endotracheal tube intubation, and longer duration of intubation. The PlGF levels in cord blood correlated negatively with gestational age and BW. However, multivariable logistic regression analyses revealed that only elevated cord blood PlGF levels and BW were associated with BPD after adjusting for all contributing factors. Furthermore, an increased PlGF level in cord blood was significantly correlated with the clinical severity of BPD, as measured by duration of intubation. At 17 mg/dL, the specificity of cord blood PlGF level in predicting BPD was 95%, the sensitivity was 53%, the positive predictive value was 83%, and the negative predictive value was 82%. CONCLUSIONS: Measuring cord blood PlGF level at birth might be a biological marker for predicting the occurrence of BPD and allowing early therapeutic intervention.
Biomarkers↗
Expression of angiogenic factors and their receptors in postnatal mouse developing lung.
BACKGROUND AND PURPOSE: Several lines of evidence suggest that angiogenesis is necessary for alveolarization and that inhibition of vascular growth during a critical period of early growth may impair alveolarization. However, little is known about the role of angiogenic factors during alveolarization. This study investigated the expression patterns of the Ang-Tie-2 family of endothelium-specific receptor tyrosine kinases and vascular endothelial growth factors and their receptors (VEGF-VEGFR system) during postnatal mouse lung development. METHODS: The lungs from 3 or 4 mice from groups aged 3, 7, 10 and 14 days and adults were removed and dissected from the main bronchi for reverse transcriptase-polymerase chain reaction (RT-PCR) analysis. Semi-quantitative RT-PCR of mouse lung total RNA was used to measure the expression levels of these angiogenic factors and their receptors. RESULTS: During alveolarization, VEGF/Flk-1, Ang-1, Ang-2, and Tie-2 were up-regulated and PlGF/Flt-1 was kept at a relatively constant level. After alveolarization was completed, PlGF was down-regulated, Flt-1 was up-regulated, and VEGF/Flk-1, Ang-1, Ang-2, and Tie-2 were maintained at relatively high levels. CONCLUSIONS: During alveolarization, in mice, VEGF/Flk-1, Ang-1, Ang-2, and Tie-2 are up-regulated and PlGF/ Flt-1 is kept at relatively constant level to promote pulmonary microvascular development. In the adult mouse lung, when most of the vascular network is complete, PlGF is down-regulated and Flt-1 is up-regulated to stop angiogenesis and VEGF/Flk-1, Ang-1, Ang-2 and Tie-2 are kept at relatively high levels to maintain mature pulmonary microvasculature.
Animals↗