Correlations between pulmonary function and childhood asthma control test results in 5-11-year-old children with asthma.

OBJECTIVES: We examined correlations between the two asthma assessment tools, pulmonary function tests, and Childhood Asthma Control Test (C-ACT) scores, in 5-11-year-old children with asthma to determine if the C-ACT scores could predict pulmonary function test results. MATERIALS AND METHODS: A total of 172 children with asthma aged 5-11 years completed C-ACT questionnaires and underwent pulmonary function testing. Correlations between these test results were examined. Patients were also placed into two groups, C-ACT scores ≤19 and >19, to determine if patients with scores >19 had better pulmonary function test results. RESULTS: Weak correlations were found between pulmonary function test results and childhood asthma control test scores in 5-11-year-old children with asthma, with or without the use of an asthma controller. These correlations included: 0.061 for FEV1 [confidence interval (CI): -0.022-0.049] and 0.074 for MMEF (CI: -0.013-0.037). The proportions of children with C-ACT test scores ≤19 group and those with scores >19 group were not significantly different. CONCLUSION: Correlations between C-ACT scores and pulmonary function test results were poor for children aged 5-11 years with asthma. FEV1, FVC, FEF25, FEF50, FEF75, MMEF, and PEFR were not significantly correlated with C-ACT scores.

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

BACKGROUND: 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 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. METHODS: 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. RESULTS: 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). CONCLUSION: The PalmLab glucometer provides accurate results in samples with high PO(2) and is suitable for measuring arterial glucose levels in pediatric patients.

Congenital stridor and wheezing as harbingers of the del22q11.2 syndrome presenting cardiovascular malformations of right aortic arch, aberrant left subclavian artery, Kommerell's diverticulum, and left ligamentum arteriosum.

A complete vascular ring composed of right aortic arch, aberrant left subclavian artery with Kommerell's diverticulum, and left ligamentum arteriosum was diagnosed by barium esophagography, echocardiography, angiography, and multidetector computed tomography of chest in an 18-day-old male neonate who presented with remarkable inspiratory stridor, expiratory wheezing, postprandial vomiting, and dysphagia since birth, and survived surgical division of the left ligamentum arteriosum, resection of the Kommerell's diverticulum, and reimplanation of the left subclavian artery to the left common carotid artery. Cytogenetic analysis and fluorescence in situ hybridization study of his blood revealed chromosome 22q11.2 deletion, with a karyotype of 46,XY.ish del(22)(q11.2 q11.2). A constellation of right aortic arch, aberrant left subclavian artery with Kommerell's diverticulum, and left ligamentum arteriosum in neonates may cause refractory stridor, wheezing, vomiting, and dysphagia, which can serve as harbingers of the del22q11.2 syndrome.

Changing clinical presentations and survival pattern in trisomy 18.

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 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.

PURPOSE: 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.

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

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.

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

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 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.

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

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 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.

Previous heat shock treatment attenuates lipopolysaccharide-induced hyporesponsiveness of platelets in rats.

Several studies demonstrated that previous heat shock treatment caused expression of heat shock proteins (HSPs) and reduced organ dysfunction and mortality in experimentally induced severe sepsis. However, the protective mechanism on platelet function remains unclear. The aim of this study was to investigate the effect of heat shock treatment on platelet aggregation ex vivo in endotoxin-induced rats with sepsis. Rats of the heated group were heated by whole-body hyperthermia 18 h before lipopolysaccharide (LPS) injection. Blood samples were obtained from the carotid artery 90 min after LPS injection. Platelet aggregation ability was measured by aggregometer. Results revealed that platelet aggregation ex vivo was significantly inhibited in LPS-induced rats in a manner of dose dependence. Previous heat shock treatment caused overexpression of HSPs and significantly attenuated the LPS-induced platelet hyporesponsiveness. This attenuation disappeared in accordance with absence of HSP72 at 7 days after heat shock treatment. Aggregation of normal platelets was also inhibited by incubating with plasma obtained from endotoxemic rats but not from preheated endotoxemic rats. Furthermore, no significant hyporesponsiveness was found in endotoxemic platelets in addition to preheated endotoxemic plasma. The addition of H2O2 scavenger catalase diminished the platelet hyporesponsiveness significantly only in nonheated endotoxemic rats. Moreover, the plasma nitrite and nitrate levels were significantly attenuated in preheated endotoxemic rats. These results revealed that previous heat shock treatment might attenuate LPS-induced hyporesponsiveness of platelets by changing the plasma components possibly through altering H2O2 and nitric oxide concentrations.

Absence of hypercoagulability in acute Kawasaki disease.

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 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.