bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical and Genetic Risk Factors for Cystic Fibrosis-related Liver Disease
Authors: Wilschanski Michael , Rivlin Joseph , Cohen Solomon , Augarten Arieh , Blau Hannah , Aviram Micha , Bentur Lea , Springer Chaim , Vila Yael , Branski David , Kerem Batsheva , Kerem Eitan 
Published: August 2004

Objective.
The aim of this study was to define the role of possible risk factors for the development of cystic fibrosis (CF)-related liver disease and to analyze the association between liver disease and the different genotypes present in the Israeli CF patient population.

Patients and Methods.
All patients followed at the seven CF centers in Israel were included in this study. Liver disease was determined by persistently elevated serum liver enzymes and/or bilirubin, and/or significant ultrasonographic changes suggestive of chronic liver disease. The following clinical parameters were evaluated: ethnic origin, age at assessment of liver function, sex, history of meconium ileus, pancreatic function, history of distal intestinal obstruction syndrome, pulmonary function, and cystic fibrosis transmembrane conductance regulator mutation analysis.

Results.
Of the 288 patients screened, 80 (28%) had liver disease. Of the 256 patients with pancreatic insufficiency, 80 (31%) had liver disease compared with none of the 32 patients with pancreatic sufficiency. Genotype-phenotype correlation was performed on 207 patients carrying identified mutations that were previously classified according to phenotype severity. Liver disease was found in 56 (32%) of 173 patients carrying mutations associated with a severe phenotype and in 6 (38%) of 16 patients carrying at least one mutation associated with a variable genotype (G85E and/or 5T allele). None of the 18 patients carrying the 3849+10kb C-&gt;T mutation had liver disease. Prevalence of liver disease increased with age. No correlation was found between liver disease and severity of lung disease, nutritional status, history of meconium ileus, or distal intestinal obstruction syndrome.

Conclusion.
CF patients who have pancreatic insufficiency and carry mutations associated with a severe or a variable genotype are at increased risk to develop liver disease.
Developmental Dysplasia of the Hip: A New Approach to Incidence
Authors: Bialik Viktor , Bialik Gadi M. , Blazer Shraga , Sujov Polo , Wiener Fred , Berant Moshe 
Published: August 2004

Objective.The controversy over the incidence of developmental dysplasia of the hip (DDH) stems mainly from an ambiguity of criteria for defining a genuinely pathologic neonatal hip. In this study, we evaluate an algorithm we devised for the treatment of DDH, for its ability to identify those neonatal hips which, if left untreated, would develop any kind of dysplasia and, therefore, are to be included in the determination of DDH incidence.Methods.Clinical and ultrasonographic examinations for DDH were performed on 18 060 consecutive neonatal hips at 1 to 3 days of life. Newborns with skeletal deformities, neurologic/muscular disorders, and neural tube defects were excluded. Hips that featured any type of sonographic pathology were reexamined at 2 or 6 weeks, depending on the severity of the findings. Only hips in which the initial pathology was not improved or had deteriorated were treated; all others were examined periodically until the age of 12 months.Results.Sonographic screening of 18 060 hips detected 1001 instances of deviation from normal, indicating a sonographic DDH incidence of 55.1 per 1000. However, only 90 hips remained abnormal and required treatment, indicating a true DDH incidence of 5 per 1000 hips. All the others evolved into normal hips, and no additional instances of DDH were found on follow-up throughout the 12 months.Conclusions.The implementation of our protocol enables us to distinguish two categories of neonatal hip pathology: one that eventually develops into a normal hip (essentially sonographic DDH); and another that will deteriorate into a hip with some kind of dysplasia, including full dislocation (true DDH). This approach seems to allow for a better-founded definition of DDH, for an appropriate determination of its incidence, for decision-making regarding treatment, and for assessment of the cost-effectiveness of screening programs for the early detection of DDH. developmental dysplasia of the hip, incidence, neonatal screening, sonography.
Perinatal Risk Factors for Infant Hospitalization With Viral Gastroenteritis
Authors: Newman Robert D. , Grupp-Phelan Jacqueline , Shay David K. , Davis Robert L. 
Published: August 2004

Context. A tetravalent vaccine against rotavirus, the most commonly identified etiologic agent of viral gastroenteritis (GE), has recently been licensed for use in the United States.Objective. To evaluate whether specific groups of infants might be at sufficiently high risk to warrant a focused rotavirus vaccine policy, we investigated perinatal risk factors for hospitalization with viral GE and rotavirus in the first year of life.Design. Population-based, case–control study.Setting. Washington State linked birth certificate and hospital discharge abstracts from 1987 through 1995.Patients. Infants, 1 through 11 months of age, hospitalized for viral GE (N = 1606) were patients in this study. Control subjects were 8084 nonhospitalized infants, frequency-matched to patients on year of birth.Primary Outcome Measure. Maternal and infant characteristics associated with infant hospitalization for viral GE.Results. We found a significant association between birth weight and the risk for hospitalization. Very low birth weight infants (&lt;1500 g) were at the highest risk (odds ratio [OR] 2.6; 95% confidence interval [CI]: 1.6,4.1);, low birth weight infants (1500–2499 g), at intermediate risk (OR 1.6; 95% CI: 1.3,2.1); and large infants (&gt;4000 g), at reduced risk (OR 0.8; 95% CI: 0.6,0.9). Other characteristics associated with GE hospitalization were male gender (OR 1.4; 95% CI: 1.3,1.6); maternal smoking (OR 1.2; 95% CI: 1.1,1.4); unmarried mother (OR 1.2; 95% CI: 1.1,1.4); Medicaid insurance (OR 1.4; 95% CI: 1.3,1.7); and maternal age &lt;20 years (OR 1.2; 95% CI: 1.0,1.5). Infants born October through December were at decreased risk for hospitalization (OR 0.8; 95% CI: 0.7,0.9), as were infants born to Asian mothers (OR 0.5; 95% CI: 0.3,0.7), and infants born to mothers &gt;34 years of age (OR 0.7; 95% CI: 0.6,0.9). Using these factors, the area under a receiver operating characteristic curve was 0.63. Therefore, to achieve a sensitivity of 90% in identifying high-risk infants, specificity would fall to 10%. Subanalyses of children admitted for viral GE during the peak of the Northwest rotavirus season (January to March) and children with confirmed rotavirus infection demonstrated similar risk factors and receiver operating characteristic curves.Conclusion. We conclude that a focused rotavirus vaccination policy using readily identifiable potential high-risk groups would be unlikely to prevent most infant hospitalizations associated with rotavirus infection. However, the safety of rotavirus vaccine in low birth weight and premature infants must be established, because these children appear to be at greater risk for hospitalization with viral GE and rotavirus.
Feeding Strategies for Premature Infants: Randomized Trial of Gastrointestinal Priming and Tube-feeding Method
Authors: Schanler Richard J. , Shulman Robert J. , Lau Chantal , Smith E. O'Brian , Heitkemper Margaret M. 
Published: August 2004

BackgroundData on enteral feeding management of premature infants are limited and often not the subject of randomized clinical trials. Several small studies suggest benefits from the early initiation of feeding, but do not assess the combined effects of time of initiation of feeding, tube-feeding method, and type of milk used. Either singly or in combination, these treatments may affect growth, bone mineralization, biochemical measures of nutritional status, and feeding tolerance, and, ultimately, the duration of hospitalization.Methods.A total of 171 premature infants, stratified by gestational age (26 to 30 weeks) and diet (human milk or preterm formula) were assigned randomly among four treatment combinations in a balanced two-way design comparing the presence or absence of gastrointestinal (GI) priming for 10 days and continuous infusion versus intermittent bolus tube-feeding.Results.The major outcome, time required for infants to attain full oral feeding, was similar among treatments. GI priming was not associated with any measured adverse effect and was associated with better calcium and phosphorus retention, higher serum calcium and alkaline phosphatase activity, and shorter intestinal transit times. The bolus tube-feeding method was associated with significantly less feeding intolerance and greater rate of weight gain than the continuous method. In addition, the greater the quantity of human milk fed, the lower the morbidity.Conclusions.Early GI priming with human milk, using the bolus tube-feeding method, may provide the best advantage for the premature infant.
Causes of Death in the Extremely Low Birth Weight Infant
Authors: Barton Lorayne , Hodgman Joan E. , Pavlova Zdena 
Published: August 2004

Objective.
To present primary and secondary causes of death confirmed by autopsy for the extremely low birth weight infant.

Methods.
A total of 111 infants weighing between 300 and 1000 g at birth who died and were autopsied at our hospital during the 4-year period 1990–1993 were retrospectively reviewed. Clinical, pathologic, and laboratory data were retrieved including results of placental examinations and autopsy cultures. Primary and secondary causes of death were assigned by the authors.

Results.
Infection was the most common primary cause (56/111) followed by respiratory distress syndrome/bronchopulmonary dysplasia (24/111) and congenital defect (15/111). Immaturity as an only cause appeared almost exclusively in infants weighing &lt;500 g at birth. Infection was significantly underdiagnosed clinically with most of these deaths attributed to immaturity or respiratory distress syndrome. In only 1 case was intraventricular hemorrhage considered the primary cause of death although it was present as a secondary cause in 19/111. Infections were divided into congenital (30/56) and acquired (26/56) by time of death. The congenital infections (≤48 hours) consisted of pneumonia and chorioamnionitis/funisitis attributable to maternal enteric organisms. Causative bacteria were obtained in pure culture in a number of these cases. The acquired infections were mainly caused by yeasts and nosocomial bacteria. Sixty-seven percent of the deaths occurred in the first 2 days and 12% were beyond the neonatal period at 29 to 104 days. Care was not initiated or was withdrawn in 50% of the infants, usually within the first 4 hours.

Conclusions.
Infection of the amniotic fluid leading to pneumonia was the major cause of death in the extremely low birth weight infant. Accurate cause of death can not be reliably ascertained without an autopsy accompanied by examination of the placenta in the early deaths. Antibiotic treatment of the mother and infant may have reduced the deaths from infection. Early failure to respond to neonatal intensive care may well indicate presence of a congenital pneumonia.
Cisapride: A Survey of the Frequency of Use and Adverse Events in Premature Newborns
Authors: Ward Robert M. , Lemons James A. , Molteni Richard A. 
Published: August 2004

Objective.
This survey estimated the frequency of use and adverse events associated with cisapride treatment of premature newborns in intensive care units. It was initiated in response to a warning issued in Canada cautioning against cisapride treatment of premature infants of &lt;36 weeks' gestation and &lt;3 months of age.

Methodology.
Surveys were mailed to 105 neonatology training program directors to obtain the total number of neonatal intensive care unit (NICU) admissions, the number of admissions of infants of &lt;36 weeks' gestation, the number of years that cisapride had been used, the estimated percent/number of premature patients treated with cisapride per year, and the frequency and nature of arrhythmias or other adverse events associated with cisapride treatment. Of 105 programs, 46 responded to a single mailing of the first survey. A second survey mailed to the 45 respondents to the first survey sought to determine the indications, diagnostic tests, and dosages used with cisapride treatment of premature newborns. Of the 45 programs, 26 responded to the second survey.

Results.
More than 58 000 premature newborns of &lt;36 weeks' gestation were admitted to the NICUs we surveyed, and ∼19% were treated with cisapride. No deaths attributable to cisapride were reported among &gt;11 000 preterm newborns treated. Three nonfatal arrhythmias were reported; two associated with 10-fold dosing errors and one with co-treatment with erythromycin, a macrolide antibiotic that reduces the metabolism of cisapride. Diarrhea was reported in 12 patients, and reversible liver enzyme changes were noted in one patient. Typically, cisapride treatment was started in dosages of 0.1 to 0.2 mg/kg/dose, repeated every 6 to 8 hours. Treatment usually was begun empirically, without a preceding study to document gastroesophageal reflux. The most frequent indications for cisapride treatment were choking or gagging, with associated apnea, bradycardia, and desaturation. Approximately 50% of patients had discontinued cisapride treatment before discharge. Eighty-four percent of clinicians judged cisapride to be effective for the problems being treated.

Conclusions.
Cisapride treatment of premature infants of &lt;36 weeks' gestation and &lt;3 months of age in NICUs appears to be widespread in the United States. Complications and adverse events were seen when cisapride was administered in excessive dosages or in combination with a drug that inhibits its metabolism and leads to increased serum concentrations. Severe toxicities such as arrhythmias were reported with a frequency of &lt;1/11 000 NICU admissions. However, in a retrospective survey, episodes of toxicity, including mortality, attributable to cisapride may not have been recognized or reported.
Chest Roentgenogram in the Evaluation of Heart Defects in Asymptomatic Infants and Children With a Cardiac Murmur: Reproducibility and Accuracy
Authors: Birkebæk Niels H. , Hansen Lars K. , Elle Bo , Andersen Poul E. , Friis Merete , Egeblad Mogens , Damgaard-Pedersen Karen , Nielsen Nina T. , Oxhøj Henrik 
Published: August 2004

Objectives. To evaluate the reproducibility and the accuracy of pediatric radiologists' assessments of chest radiographs with respect to the presence or absence of heart defects in children with an asymptomatic heart murmur.Design. Ninety-eight children, ages 1 month to 15 years (median, 30.1 months), referred for evaluation of a heart murmur were consecutively included. They all had a standard chest radiograph and a color Doppler echocardiograph (CDE) performed. Six specialists in pediatric radiology evaluated the chest radiographs independently on two occasions 6 months apart. The radiologists were asked to classify each set of films into one of two categories: heart disease or no heart disease. The outcome of the CDE was considered the definite diagnosis. κ statistics were used to analyze the reproducibility of the radiologic assessments. Sensitivity, specificity, and the predictive value of a positive and a negative test were used for evaluation of the accuracy of the radiologic assessments.Results. Mean intra- and interobserver κ values were all &lt;0.6, and the majority were &lt;0.4. Mean sensitivity was 0.3 (range: 0.17–0.52), mean predictive value of a positive test was 0.4, implying that 60% of the positive assessments were falsely positive. Mean specificity was 0.86 (range: 0.75–0.93) and the mean predictive value of a negative test was 0.80 implying that 20% of the negative assessments were falsely negative.Conclusion We found a low reproducibility, as well as a low accuracy, of the radiologic assessments of the chest radiographs of children with an asymptomatic heart murmur with respect to the presence or absence of heart disease. A false-positive radiologic assessment of the chest radiograph with respect to heart defects causes unnecessary anxiety and further examinations, whereas a false-negative assessment might result in omission of relevant investigations and proper identification of the heart defect. We cannot recommend the use of chest radiographs in the initial evaluation of the asymptomatic child with a heart murmur. If a heart defect cannot be excluded by clinical examination a CDE must be performed.
Human Immunodeficiency Virus Status and Delayed-Type Hypersensitivity Skin Testing in Ugandan Children
Authors: Mandalakas Anna Maria , Guay Laura , Musoke Philippa , Carroll-Pankhurst Cindie , Olness Karen N. 
Published: August 2004

Background. In previous studies, delayed-type hypersensitivity (DTH) skin testing has been shown to be affected by several factors including nutritional status, intercurrent infection, host immune status, and previous exposure to the antigen being used.Objective. To determine the effect of human immunodeficiency virus type 1 (HIV-1) status on DTH skin testing in a cohort of HIV-1-infected and noninfected Ugandan children followed prospectively from birth.Design. Nested case-control study.Setting. Primary care clinic serving study participants at Mulago Hospital, Makerere University, Kampala, Uganda.Participants. Thirty HIV-1-infected children and 30 age-matched, HIV-1-noninfected children.Methods. After completion of history and physical, each child underwent Mantoux skin testing with both Candidaand purified protein derivative (PPD). Results of skin testing were read in 48 to 72 hours. Complete chart reviews were performed on all children. CD4 lymphocyte counts were obtained on all HIV-1-infected children at the time the skin testing was read.Results. The average age of participants was 67 months (range, 51–92 months). HIV-1-infected children (mean CD4 lymphocyte count, 1069 mL−1; range, 86–3378 mL−1), compared with noninfected, age-matched peers, developed significantly smaller PPD reaction size (mean, 1.18 mm ± 4.3 vs 3.6 mm ± 7.6, respectively). Candida responses were not different between the two groups of children. Among HIV-1-infected children, there was a larger Candida reaction size in children who had recently received chloroquine treatment. There was no significant correlation between Candida reactivity and PPD reactivity, progressive HIV-1 disease, or CD4 lymphocyte count. The six children diagnosed clinically with active tuberculosis had lower absolute CD4 lymphocyte counts than children without tuberculosis. Lack of reaction to PPD was associated with lower CD4 lymphocyte counts and progressive HIV-1 disease.Conclusions. In HIV-1-infected Ugandan children, DTH skin testing was influenced by the choice of antigen selected, HIV-1 infection, and recent treatment with chloroquine. Based on these findings, we believe that further prospective, longitudinal investigation into the role of chloroquine in HIV-1-infected children is needed. We emphasize the limitations of DTH skin testing in HIV-infected children as an adjunct in the diagnosis of active tuberculosis.
A Comparison of the Mogen and Gomco Clamps in Combination With Dorsal Penile Nerve Block in Minimizing the Pain of Neonatal Circumcision
Authors: Kurtis Peter S. , DeSilva Hema N. , Bernstein Bruce A. , Malakh Lillian , Schechter Neil L. 
Published: August 2004

Objectives. 1) To compare the Mogen and Gomco clamps with regard to pain experienced during neonatal circumcision, and 2) to assess neonatal circumcision pain with and without dorsal penile nerve block (DPNB).Design/Methods. A randomized, controlled, nonblinded clinical trial; 48 healthy, full-term infants were randomized into one of the following four groups: Gomco vs Mogen with (+) or without (−) DPNB. DPNB+ infants were injected with 0.8 mL of 1% lidocaine before circumcision. DPNB− infants received no placebo injection. Heart rate, respiratory rate, and oxygen saturation (Sao2) during the procedure were monitored and data transferred to computer files by using the Datalab software system. Crying was recorded on videotape. Pre- and postcircumcision saliva samples for cortisol analysis were collected. Heart rate, respiratory rate, Sao2, cortisol changes, and duration of crying were evaluated statistically with two-way analyses of variance andt tests.Results. The type of clamp but not the use of anesthesia was significantly associated with the length of the procedure (mean Mogen time, 81 seconds; mean Gomco time, 209 seconds) and percentage of respiratory rate change. The use of anesthesia but not the type of clamp was significantly associated with percentage of crying time and percentage of Sao2 change during the procedure. Heart rate changes and total crying time were significantly associated withboth the type of clamp and the use of anesthesia.Neither clamp type nor anesthesia status was significantly associated with salivary cortisol changes, although the mean increase for the DPNB− group was approximately twice that for the DPNB+ group. Fifty-six percent of infants circumcised with the Mogen clamp and DPNBdid not cry at all during the procedure.Conclusions. DPNB is effective in reducing neonatal circumcision pain with either the Mogen or the Gomco clamp. For a given anesthesia condition, the Mogen clamp is associated with a less painful procedure than the Gomco. The Mogen clamp with DPNB causes the least discomfort during neonatal circumcision.
Nasal Continuous Positive Airway Pressure and Early Surfactant Therapy for Respiratory Distress Syndrome in Newborns of Less Than 30 Weeks' Gestation
Authors: Verder Henrik , Albertsen Per , Ebbesen Finn , Greisen Gorm , Robertson Bengt , Bertelsen Aksel , Agertoft Lone , Djernes Birgitte , Nathan Erling , Reinholdt Jes 
Published: August 2004

Objective.To determine whether early versus late treatment with porcine surfactant (Curosurf) reduces the requirement of mechanical ventilation in very preterm infants primarily supported by nasal continuous positive airway pressure (nasal CPAP).Design.Multicenter randomized, controlled trial.Patients.The study population comprised 60 infants &lt;30 weeks' gestation with respiratory distress syndrome (RDS) who had an arterial to alveolar oxygen tension ratio (a/APo2) of 0.35 to 0.22.The cohort from which the study population was generated comprised 397 infants.Results.The need for mechanical ventilation or death within 7 days of age was reduced from 63% in the late-treated infants to 21% in early-treated infants. Increasing numbers of antenatal steroid doses also improved the outcome, especially in the early-treated infants. Six hours after randomization mean a/APo2 rose to 0.48 in the early-treated infants compared with 0.36 in the late-treated. The need of mechanical ventilation before discharge was reduced from 68% in the late-treated to 25% in the early-treated infants.Conclusions.Nasal CPAP in combination with early treatment with Curosurf significantly improves oxygenation and reduces the subsequent need for mechanical ventilation in infants &lt;30 weeks' gestational age with RDS.