bluuebunny/crossref_metadata_2025_split huggingface dataset:
Rapid and Simultaneous Determination of Coproporphyrin and Protoporphyrin in Feces by Derivative Matrix Isopotential Synchronous Fluorescence Spectrometry
Authors: Lin Dan-Li , He Li-Fang , Li Yao-Qun 
Published: August 2004

Abstract
Background: Measurement of fecal porphyrins is important in the diagnosis of porphyria, but conventional methods to measure them have drawbacks. We explored the use of derivative matrix isopotential synchronous fluorescence (MISF) spectrometry for the measurement of coproporphyrin and protoporphyrin.
Methods: The MISF scanning route was selected based on information from the three-dimensional fluorescence spectrum, which was a combination of the contour line of protoporphyrin via a detection point of coproporphyrin and that of coproporphyrin via a detection point of protoporphyrin. Derivative technique eliminated the constant interfering signals. MISF was used to measure porphyrins in stools from 2 pregnant women and 20 healthy volunteers.
Results: The coproporphyrin and protoporphyrin spectra were resolved with almost no mutual interference. The amplitudes of the derivative peaks were linearly related to the concentrations of coproporphyrin up to 310 nmol/L and protoporphyrin up to 590 nmol/L. The detection limits for coproporphyrin and protoporphyrin were 1.2 and 1.7 nmol/L, respectively. The within-run imprecision (CV; n = 6) was 2.2% at 175 nmol/L for coproporphyrin and 2.3% at 500 nmol/L for protoporphyrin. Bland–Altman analysis indicated no significant differences between the proposed MISF method and conventional spectrophotometry or fluorimetry. Mean (SD) recoveries of porphyrins added to fecal samples were of 98 (7)% for coproporphyrin and 102 (4)% for protoporphyrin.
Conclusions: This technique provides spectral resolution of coproporphyrin and protoporphyrin, obviating the need for chromatographic separation, and measurements can be made in a single scanning. The method also appears suitable for routine testing of large numbers of samples.
Infant Heart Transplantation at Stanford: Growth and Neurodevelopmental Outcome
Authors: Fleisher Barry E. , Baum David , Brudos Ginger , Burge Mary , Carson Elaine , Constantinou Janet , Duckworth Josh , Gamberg Pat , Klein Pat , Luikart Helen , Miller Joan , Stach Brad , Bernstein Dan 
Published: August 2004

Objective. To evaluate the growth and neurodevelopmental outcome of 18 surviving Stanford patients who received heart transplantations before their second birthday.
Methods. We compared the growth and neurodevelopmental outcome of these 18 patients with a second group of age-matched comparison patients who underwent other heart surgery requiring cardiopulmonary bypass.
Results. Difficulties with growth and development were more common in the transplant group as were neurologic abnormalities. Speech and language delays as well as hearing problems were also more common in the transplant group.
Conclusion. Multicenter prospective longitudinal neurodevelopmental outcome studies of infant heart transplant patients should be conducted to provide a more efficient basis for evaluating management protocols and assessment of long-term outcomes and of the need for early intervention services.
Early Developmental Outcomes After Newborn Encephalopathy
Authors: Dixon Glenys , Badawi Nadia , Kurinczuk Jennifer J. , Keogh John M. , Silburn Sven R. , Zubrick Stephen R. , Stanley Fiona J. 
Published: August 2004

Objective. The aim of this study was to ascertain the early developmental status of children who have a history of newborn encephalopathy.Methods. A longitudinal follow-up was conducted of a population-based, case-control study of children born in Western Australia between June 1993 and December 1996. The study included 276 term children (≥37 weeks’ gestation) with moderate or severe newborn encephalopathy and 564 unmatched term control subjects. The Griffiths Mental Development Scales was used to ascertain developmental status and a General Quotient (GQ) score. Outcome measures were the Griffiths developmental subscales, GQ, diagnosis of cerebral palsy, and mortality.Results. Thirty-four patients and 1 control subject died before reaching assessment. Between June 1994 and December 1999, 195 (81%) eligible patients and 445 (79%) eligible control subjects were assessed. Statistically significant differences were found between patients and control subjects for GQ and all developmental subscales. Overall, 39% of patients had a poor outcome as defined by death, cerebral palsy, or a significant degree of developmental delay, compared with 2.7% of control subjects. Furthermore, 62% of those with severe encephalopathy had a poor outcome compared with 25% of those with moderate encephalopathy. Patients with a history of seizures were 3 times more likely to develop cerebral palsy than patients without. Overall, 28 (10.1%) of patients have cerebral palsy.Conclusions. These data provide important prognostic information regarding survival and serious disability and indicate that newborn encephalopathy places children at significant risk of developmental delay by their second year. These findings also suggest that comprehensive clinical and educational assessments are required to enable appropriate educational provisions as these infants approach school entry.
Impact of Site of Care, Race, and Hispanic Ethnicity on Medication Use for Childhood Asthma
Authors: Ortega Alexander N. , Gergen Peter J. , Paltiel A. David , Bauchner Howard , Belanger Kathleen D. , Leaderer Brian P. 
Published: August 2004

Objective. To understand the importance of source of care and other factors that influence differences in asthma medication use by race and Hispanic ethnicity.
Methods. The Childhood Asthma Severity Study provided 12-month, retrospective, parent-reported questionnaire data on a monthly basis for children ages ≤12 years in a community sample of 1002 children and their families from Connecticut and Massachusetts. Medications considered included cromolyn, β2-agonist, inhaled steroids, anticholinergics, theophylline, and systemic steroids. Information was available on demographics, insurance status, symptom severity, primary care contact, and provider practice types.
Results. Black and Hispanic children received fewer β2-agonists, and Hispanic children received fewer inhaled steroids than white children after adjusting for patients’ race, age, gender, insurance status, symptom severity, number of primary care visits for asthma, number of urgent visits to the regular provider, family income, maternal education, and site of care. When multivariate analyses were restricted to patients in private practice, the significant association between Hispanic ethnicity and low inhaled steroid use persisted, whereas differences in β2-agonist use by race and ethnicity changed little but became nonsignificant.
Conclusion. Even within private practices, patients’ race and ethnicity are associated with clinician nonadherence to national guidelines. Programs to eliminate these disparities will need both to focus on site of care and to intervene at the provider and patient levels to be successful.
Does Abciximab Enhance Regression of Coronary Aneurysms Resulting From Kawasaki Disease?
Authors: Williams Richard V. , Wilke Venus M. , Tani Lloyd Y. , Minich L. LuAnn 
Published: August 2004

Objective. Acute Kawasaki disease can result in the development of large coronary artery aneurysms that may persist. Abciximab, a platelet glycoprotein IIb/IIIa receptor inhibitor, is associated with resolution of thrombi and vascular remodeling in adults with acute coronary syndromes. The purpose of this study was to compare changes in aneurysm diameter at early follow-up in patients who had Kawasaki disease and received abciximab in addition to standard therapy with those who were treated with standard therapy alone.
Methods. Patients with Kawasaki disease and large aneurysms were divided into 2 groups on the basis of acute therapy: 1) abciximab in addition to standard therapy and 2) standard therapy alone. Echocardiograms were reviewed for coronary aneurysms (lumen diameter 1.5 times that of the adjacent vessel). Maximum aneurysm diameter was determined during the acute/subacute phase of Kawasaki disease (&lt;6 weeks) and at early follow-up (4–6 months). Regression of the aneurysm was defined as a decrease in lumen diameter, and resolution was defined as normalization of the vessel.
Results. Six patients had 20 aneurysms in the abciximab group, and 9 patients had 30 aneurysms in the standard therapy group. Early follow-up data were available for 19 of the 20 aneurysms in the abciximab group and 19 of the 30 aneurysms in the standard therapy group. Patients who were treated with abciximab demonstrated greater regression in aneurysm size at early follow-up than patients who were treated with standard therapy alone (percentage decrease: 41 ± 19% vs 17 ± 27%). In the abciximab group, 68% (13 of 19) of aneurysms resolved at early follow-up compared with 35% (7 of 19) in the standard therapy group.
Conclusions. Patients who were treated with abciximab demonstrated greater regression in aneurysm diameter at early follow-up than patients who received standard therapy alone. These findings suggest that treatment with abciximab may promote vascular remodeling in this population and warrants further study.
Pediatric Residents’ Career Intentions: Data From the Leading Edge of the Pediatrician Workforce
Authors: Pan Richard J. , Cull William L. , Brotherton Sarah E. 
Published: August 2004

Objective. To determine factors influencing career choices by pediatric residents and how they may change the future pediatric physician workforce.Background. Societal norms and policy decisions can influence the demographics of residents entering pediatric practice and resident career choices. Although predictors of physician career choice have been identified retrospectively by examining the characteristics of physicians in practice, little work has focused on the job selection factors important to pediatric residents when they make their postresidency career decisions.Design/Methods. For 3 consecutive years (1997–1999), the American Academy of Pediatrics surveyed a national sample of 500 third-year residents, totaling 1500 residents. Data on resident characteristics, job selection attitudes, and career decisions were obtained, and trends for the past 3 years were examined.Results. The percentage of residents intending to practice primary care remained constant at around 69% from 1997 through 1999. Female residents, US medical graduates, and residents married to nonphysicians were more likely to report primary care practice as their future clinical goal. These relationships were mediated by higher rates of a primary care practice goal among those who felt geographic location and future colleagues were very important and by lower rates among those who felt acceptable income, teaching opportunities, and research opportunities were very important. Six percent of residents entering general pediatrics were heading to rural locations.Conclusions. With a projected increase in the number of female pediatricians and a decline in international medical graduates, our study suggests that pediatrics may continue to shift toward an increased proportion of general pediatricians. Lifestyle issues are a major factor influencing job choice and must be addressed.
A 16-Year Neonatal/Pediatric Extracorporeal Membrane Oxygenation Transport Experience
Authors: Wilson Bernard J. , Heiman Howard S. , Butler Thomas J. , Negaard Kathryn A. , DiGeronimo Robert 
Published: August 2004

Objective. To characterize the population and survival of neonatal and pediatric patients transported by Wilford Hall Medical Center (WHMC) on extracorporeal membrane oxygenation (ECMO) since 1985.Study Design. A retrospective chart, literature, and database review of pediatric and neonatal patients transported on ECMO by the WHMC ECMO transport team. In addition, a subpopulation analysis was performed comparing neonates with meconium aspiration syndrome (MAS) placed on ECMO at WHMC with those infants with MAS transported on ECMO. Characteristics of interest for this comparison included disease severity before ECMO, age at initiation of ECMO, survival, ECMO-related complications, and duration of ECMO support.Results. Forty-two patients transported on ECMO were identified: 23 neonatal respiratory cases (survival 57%), 7 pediatric respiratory cases (survival 71%), 4 cardiac cases (survival 50%), and 8 extra-institutional ECMO transports (survival 63%). In the MAS subpopulation, there was significantly greater survival in the in-house group—97% (31/32)—than in the ECMO transport group—75% (9/12); there were no other significant differences between these groups. Overall, no ECMO-related complications leading to patient demise could be identified in the ECMO transport group.Conclusions. ECMO transport, although demonstrating acceptable survival, is a risk-laden modality that should not replace early referral to an ECMO center.
Expression of Activation Markers and Cytokine mRNA by Peripheral Blood CD4 and CD8 T Cells in Atopic and Nonatopic Childhood Asthma: Effect of Inhaled Glucocorticoid Therapy
Authors: Gemou-Engesæth Vasso , Fagerhol Magne K. , Toda Masao , Hamid Qutayba , Halvorsen Sverre , Groegaard Jens B. , Corrigan Chris J. 
Published: August 2004

Hypothesis. Activated CD8 as well as CD4 T cells contribute to the production of asthma-relevant cytokines in both atopic and nonatopic childhood asthma.Objectives. To measure the percentages of peripheral blood CD4 and CD8 T cells expressing naïve/memory (CD45RA/CD45RO) and activation (HLA-DR, CD25) markers, as well as mRNA-encoding interleukin-4 (IL-4) and interleukin-5 (IL-5) in atopic and nonatopic childhood asthmatics and in nonasthmatic controls matched for age and atopic status; and to study the effects of inhaled glucocorticoid therapy of the asthmatics on these measurements.Methods. Peripheral blood mononuclear cells were isolated from 17 atopic and 8 nonatopic stable (not acutely ill) asthmatics aged 7 to 16 years with moderate-to-severe disease and from 15 nonasthmatic controls matched for age and atopic status. Activation markers on CD4 and CD8 T cells were measured by flow cytometry, and expression of cytokine mRNA by in situ hybridization with CD4 and CD8 T cells were isolated using magnetic beads. Measurements were repeated in 18 of the asthmatics 4 to 6 months after initiation or escalation of inhaled glucocorticoid therapy for inadequately controlled asthma.Results. The percentages of CD4 T cells expressing CD45RO but not CD45RA were elevated in both asthma groups as compared with the relevant controls and were reduced in association with de novo or augmented inhaled glucocorticoid therapy. The percentages of CD8 T cells expressing both markers were not elevated in asthmatics as compared with controls. The percentages of both CD4 and CD8 T lymphocytes expressing HLA-DR and CD25 were elevated in the asthmatics as compared with controls, and significantly reduced in association with de novo or augmented inhaled glucocorticoid therapy. Elevated percentages of CD4 T cells expressing mRNA encoding IL-4 and IL-5, and CD8 T lymphocytes expressing IL-5, were found in asthmatics as compared with the controls. De novo or augmented inhaled glucocorticoid therapy was associated with significant reductions in the percentages of CD4 T cells expressing IL-5 and IL-4 mRNA, as well as improvements in lung function, symptom scores, and bronchial hyperresponsiveness to metacholine (PD20) in both the atopic and nonatopic asthmatics.Conclusions. The data are consistent with the hypothesis that both activated CD4 and CD8 T cells are associated with child asthma, and that CD4 T cells make a greater contribution to IL-4 and IL-5 synthesis. Increased dosages of inhaled glucocorticoid resulted in clinical improvement in the asthmatics along with reduced T-cell activation and cytokine mRNA expression, suggesting a possible causal association.
Serum Luteinizing Hormone Rises Within Minutes After Depot Leuprolide Injection: Implications for Monitoring Therapy
Authors: Bhatia Suruchi , Neely E. Kirk , Wilson Darrell M. 
Published: August 2004

Objective. To find the time of the serum gonadotropin peak after depot leuprolide injection in children and to show that depot leuprolide therapy can be monitored by measuring serum luteinizing hormone (LH) immediately after injections.Study Design. We measured concentrations of leuprolide, LH, and follicle-stimulating hormone (FSH) at multiple time points before and after the first dose of depot leuprolide in 14 pubertal children beginning therapy. Gonadotropins and sex steroids were measured again after the fourth dose.Results. Serum leuprolide, LH, and FSH levels rose rapidly after initial injection, reaching sustained elevations at 30 to 120 minutes. The median LH level increased from 2.1 mIU/mL at baseline to a peak of 27.5 mIU/mL at 45 minutes, and FSH increased from 5.2 to 16.5 mIU/mL. After 3 months on therapy, median serum LH after depot leuprolide injection was only 0.83 mIU/mL, similar to levels observed after intravenous or subcutaneous gonadotropin-releasing hormone stimulation in comparable subjects on depot leuprolide.Conclusion. Our pharmacokinetic data demonstrate that free leuprolide present in a depot leuprolide injection is equivalent to gonadotropin-releasing hormone in stimulating a rapid rise in serum gonadotropin concentrations. We propose that a single serum sample for LH obtained 30 to 60 minutes after depot leuprolide injection in children provides a convenient and accurate assessment of treatment efficacy.
Delayed Umbilical Bleeding—A Presenting Feature for Factor XIII Deficiency: Clinical Features, Genetics, and Management
Authors: Anwar Rashida , Minford Adrian , Gallivan Louise , Trinh Chi H. , Markham Alexander F. 
Published: August 2004

Objectives. The objectives of this study were 1) to assess the importance of an early diagnosis for factor XIII (FXIII) deficiency, and 2) to investigate the molecular basis and mechanism(s) of disease in the patients under study.
Methods. The case histories of 6 FXIII-deficient patients were examined to assess the influence of early versus delayed diagnosis and replacement therapy. The nucleotide sequence of the FXIIIA gene was determined to identify the underlying mutations responsible for the bleeding diathesis in each patient. Molecular modeling was used to predict the mechanism(s) of disease causation for each mutation.
Results. All cases presented with umbilical hemorrhage. Patients 1 to 3 were diagnosed, and their prophylactic therapy was commenced in infancy. Diagnosis in patients 4 to 6 was considerably delayed and, as a result, they continued to suffer from many bleeding symptoms. The FXIIIA gene mutations identified in these patients were as follows: a homozygous GAA→AAA mutation in codon 102 (Glu102Lys) in patient 1 and a homozygous AGC→AGG mutation in codon 295 (Ser295Arg) in patients 2 to 6. These mutations segregate with disease and are absent from the normal population, suggesting that they are likely to be disease-causing sequence changes. Computer modeling indicates that both the Lys102 and Arg295 mutants are unable to fold correctly, and probably result in unstable FXIIIA molecules.
Conclusions. We demonstrate the importance of recognizing delayed umbilical hemorrhage as a presenting feature for congenital FXIII deficiency, and the value of early diagnosis and prophylaxis. The bleeding disorder of patient 1 was attributable to a homozygous Glu102Lys mutation in FXIIIA. A homozygous Ser295Arg mutation in FXIIIA was responsible for FXIII deficiency in patients 2 to 6.
Successful Antiangiogenic Therapy of Giant Cell Angioblastoma With Interferon Alfa 2b: Report of 2 Cases
Authors: Marler Jennifer J. , Rubin Joshua B. , Trede Nikolaus S. , Connors Susan , Grier Holcombe , Upton Joseph , Mulliken John B. , Folkman Judah 
Published: August 2004

We describe 2 cases of angioblastoma, a rare, destructive pediatric tumor, treated with interferon alfa 2b (IFNα2b). The first patient is a 10-month-old male who presented with an ulcerated palatal neoplasm that could not be completely resected. The second is a male neonate with a congenital tumor of the right hand that invaded the hypothenar eminence, destroying the fourth and fifth metacarpals. Biopsy in both patients was interpreted as giant cell angioblastoma. Angioblastoma is rare; there is only 1 reported case that necessitated amputation of an upper extremity, also initially recommended for our patient. Because there is little experience with chemotherapy, permission was granted to employ an antiangiogenic regimen of IFNα2b. The angiogenic protein, basic fibroblast growth factor (bFGF), was abnormally elevated in both patients.Both patients received IFNα2b. In the first child, it was used after incomplete resection, because biopsy-proven tumor was present at the margin and in the nasopharynx. Biopsies 15 months after initiation of IFN2αb were negative for tumor. Therapy was stopped after 3 years. Eighteen months later, the patient remains disease-free. In the second child, IFNα2b was started after debridement of the ulcerated tumor. Over 11 months, the tumor completely regressed and there was bony regeneration of the metacarpals. The fifth digit was amputated because of damage to the metacarpophalangeal joint by the tumor. IFNα2b therapy was discontinued after 1 year of treatment, and the child remains disease-free 2 years and 8 months later.In conclusion, this report demonstrates that: 1) a bFGF-overexpressing low-grade tumor can respond to IFNα2b in a manner similar to life-threatening infantile hemangiomas, 2) urinary bFGF levels can help guide IFNα dosage in such patients, and 3) although bFGF-mediated tumor angiogenesis is inhibited by IFNα, physiologic angiogenesis seems to be unaffected.
Delayed Childbearing and Its Impact on Population Rate Changes in Lower Birth Weight, Multiple Birth, and Preterm Delivery
Authors: Tough Suzanne C. , Newburn-Cook Christine , Johnston David W. , Svenson Lawrence W. , Rose Sarah , Belik Jaques 
Published: August 2004

Objective. This study quantified the impact of delayed childbearing (maternal age ≥35 years) on population rate changes in low birth weight (LBW; &lt;2500 g), preterm delivery (&lt;37 weeks), multiple births, and small for gestational age (SGA; &lt;10th percentile) in Alberta, Canada, between 1990 (N = 42 930) and 1996 (N = 37 710).
Methods. Data were obtained from the provincial notification of a live or stillbirth. Analyses included relative risk estimates and χ2 tests for trend. Potential confounding attributable to in vitro fertilization was investigated.
Results. The proportion of births to women ≥35 years of age was 8.4% in 1990 and 12.6% in 1996, a 51.2% increase. Among these women, LBW delivery increased 11%, and preterm delivery increased 14%. Delayed childbearing accounted for 78% of the change in LBW rate in the population and 36% of the change in preterm delivery rate in the population. Provincial multiple birth rates increased by 15% for twins and 14% for triplets. Delayed childbearing accounted for 15% of the twin increase and 69% of the triplet increase. When in vitro fertilization pregnancies were excluded, the change was 43% for preterm rates, 100% for LBW, 14% for twins, and 9% for triplets. Delayed childbearing did not contribute to changes in singleton SGA deliveries.
Conclusions. The findings suggest that the recent increase in LBW and preterm delivery is partly related to the population phenomenon of delayed childbearing. Maternal age was not related to changes in SGA, suggesting that the age effect is through pregnancy complications that lead to preterm delivery and LBW. Prospective parents should be informed about the higher risk for neonatal morbidity associated with delayed childbearing. Health care providers should be aware of the impact of delayed childbearing on health care resources.