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1.
J Clin Pharm Ther ; 47(7): 1088-1090, 2022 Jul.
Article in English | MEDLINE | ID: mdl-35304749

ABSTRACT

WHAT IS KNOWN AND OBJECTIVES: Errors involving the delivery of IVFE containing soybean oil have known significant complications, including fat overload syndrome. However, little is known regarding the risks of fat overload syndrome with other types of lipid emulsions. CASE SUMMARY: We describe a medication administration error that resulted in rapid fish oil-base lipid emulsion (Omegaven) infusion in a five-month-old infant with parenteral nutrition associated liver disease (PNALD). The medication administration error resulted in bolus infusion of Omegaven over 12 min (5 g/kg/h) instead of 12 h (0.083 g/kg/h). WHAT IS NEW AND CONCLUSION: No adverse reactions were notes because of the rapid infusion, supporting conclusion that rapid infusion of fish oil will not result in fat overload syndrome.


Subject(s)
Fat Emulsions, Intravenous , Liver Diseases , Fat Emulsions, Intravenous/adverse effects , Fish Oils/adverse effects , Humans , Parenteral Nutrition/adverse effects , Parenteral Nutrition/methods , Soybean Oil/adverse effects
2.
Am J Med Genet A ; 179(11): 2178-2189, 2019 11.
Article in English | MEDLINE | ID: mdl-31479204

ABSTRACT

Duplications in the 22q11.2 region can cause 22q11.2 duplication syndrome and encompass a variety of phenotypes including developmental delays, facial abnormalities, cardiovascular defects, central nervous system delays, and other congenital abnormalities. However, the contribution of these contiguous duplicated regions to the clinical phenotypes has not been fully elucidated. In this study, we identified nine patients carrying different 22q11.2 microduplications detected by chromosomal microarray. Of these patients, seven pediatric patients presented with various clinical features including two neonate cases died shortly after birth, and two healthy adults. We examined region specific genotype-phenotype associations and found unpredictability associated with 22q11.2 duplications in these nine patients.


Subject(s)
Abnormalities, Multiple/diagnosis , Abnormalities, Multiple/genetics , Chromosome Duplication/genetics , DiGeorge Syndrome/diagnosis , DiGeorge Syndrome/genetics , Genetic Association Studies , Genetic Predisposition to Disease , Adult , Biological Variation, Population , Chromosome Aberrations , Chromosomes, Human, Pair 22/genetics , Comparative Genomic Hybridization , Female , Genetic Association Studies/methods , Humans , Infant , Male , Phenotype
3.
JAMA ; 311(17): 1742-9, 2014 May 07.
Article in English | MEDLINE | ID: mdl-24794367

ABSTRACT

IMPORTANCE: Invasive candidiasis in premature infants causes death and neurodevelopmental impairment. Fluconazole prophylaxis reduces candidiasis, but its effect on mortality and the safety of fluconazole are unknown. OBJECTIVE: To evaluate the efficacy and safety of fluconazole in preventing death or invasive candidiasis in extremely low-birth-weight infants. DESIGN, SETTING, AND PATIENTS: This study was a randomized, blinded, placebo-controlled trial of fluconazole in premature infants. Infants weighing less than 750 g at birth (N = 361) from 32 neonatal intensive care units (NICUs) in the United States were randomly assigned to receive either fluconazole or placebo twice weekly for 42 days. Surviving infants were evaluated at 18 to 22 months corrected age for neurodevelopmental outcomes. The study was conducted between November 2008 and February 2013. INTERVENTIONS: Fluconazole (6 mg/kg of body weight) or placebo. MAIN OUTCOMES AND MEASURES: The primary end point was a composite of death or definite or probable invasive candidiasis prior to study day 49 (1 week after completion of study drug). Secondary and safety outcomes included invasive candidiasis, liver function, bacterial infection, length of stay, intracranial hemorrhage, periventricular leukomalacia, chronic lung disease, patent ductus arteriosus requiring surgery, retinopathy of prematurity requiring surgery, necrotizing enterocolitis, spontaneous intestinal perforation, and neurodevelopmental outcomes-defined as a Bayley-III cognition composite score of less than 70, blindness, deafness, or cerebral palsy at 18 to 22 months corrected age. RESULTS: Among infants receiving fluconazole, the composite primary end point of death or invasive candidiasis was 16% (95% CI, 11%-22%) vs 21% in the placebo group (95% CI, 15%-28%; odds ratio, 0.73 [95% CI, 0.43-1.23]; P = .24; treatment difference, -5% [95% CI, -13% to 3%]). Invasive candidiasis occurred less frequently in the fluconazole group (3% [95% CI, 1%-6%]) vs the placebo group (9% [95% CI, 5%-14%]; P = .02; treatment difference, -6% [95% CI, -11% to -1%]). The cumulative incidences of other secondary outcomes were not statistically different between groups. Neurodevelopmental impairment did not differ between the groups (fluconazole, 31% [95% CI, 21%-41%] vs placebo, 27% [95% CI, 18%-37%]; P = .60; treatment difference, 4% [95% CI, -10% to 17%]). CONCLUSIONS AND RELEVANCE: Among infants with a birth weight of less than 750 g, 42 days of fluconazole prophylaxis compared with placebo did not result in a lower incidence of the composite of death or invasive candidiasis. These findings do not support the universal use of prophylactic fluconazole in extremely low-birth-weight infants. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT00734539.


Subject(s)
Antifungal Agents/therapeutic use , Candidiasis, Invasive/prevention & control , Fluconazole/therapeutic use , Infant, Premature, Diseases/prevention & control , Female , Humans , Infant Mortality , Infant, Low Birth Weight , Infant, Newborn , Infant, Very Low Birth Weight , Intensive Care Units, Neonatal , Length of Stay , Male , Single-Blind Method
4.
Clin Chem ; 55(7): 1280-7, 2009 Jul.
Article in English | MEDLINE | ID: mdl-19443565

ABSTRACT

BACKGROUND: Neonatal jaundice or hyperbilirubinemia is a common occurrence in newborns. Although most cases of neonatal jaundice have a benign course, severe hyperbilirubinemia can lead to kernicterus, which is preventable if the hyperbilirubinemia is identified early and treated appropriately. CONTENT: This review discusses neonatal jaundice and the use of transcutaneous bilirubin (TcB) measurements for identification of neonates at risk of severe hyperbilirubinemia. Such a practice requires appropriate serial testing and result interpretation according to risk level from a nomogram that provides bilirubin concentrations specific for the age of the neonate in hours. In this context, we have evaluated the potential impact on clinical outcome and limitations of TcB methods in current use. SUMMARY: TcB measurement is a viable option in screening neonates to determine if they are at risk for clinically significant hyperbilirubinemia. Total serum bilirubin should be measured by a clinical laboratory if a newborn is shown to be at higher risk for clinically significant hyperbilirubinemia. In addition, external quality assessment to identify biases and operator training issues should be part of any TcB monitoring program.


Subject(s)
Hyperbilirubinemia/blood , Bilirubin/blood , Humans , Infant, Newborn , Length of Stay , Patient Readmission , Treatment Outcome
5.
Obstet Gynecol ; 105(3): 473-9, 2005 Mar.
Article in English | MEDLINE | ID: mdl-15738010

ABSTRACT

OBJECTIVE: To compare umbilical cord and maternal serum peak gentamicin concentration, gentamicin elimination, and clinical outcomes between women who received once-daily compared with standard, thrice-daily dosing for clinical chorioamnionitis. METHODS: We randomly assigned 38 laboring women, at least 34 weeks gestation, with clinical chorioamnionitis, into 1 of 2 gentamicin dosing groups: 5.1 mg/kg every 24 hours (once-daily; n = 18), or 120 mg followed by 80 mg every 8 hours (standard; n = 20). We measured maternal serum peak and delivery gentamicin concentrations and cord serum levels at delivery. Polynomial curve fitting was used to summarize gentamicin elimination. We also compared maternal and neonatal outcomes. RESULTS: Demographic characteristics of the 2 groups were similar. Median maternal peak gentamicin levels were higher with once-daily (18.2 microg/mL) compared with standard dosing (7.1 microg/mL) (P < .001). Maternal serum levels decreased below 2 microg/mL by 10 hours in the once-daily group and by 5 hours in the standard dosing group. Extrapolated peak cord serum levels were 6.9 microg/mL in the once-daily and 2.9 microg/mL in the standard dosing arm. Cord levels decreased below 2 microg/mL by 10 hours in the once-daily and by 5 hours in the standard dosing group. We found no differences in maternal or neonatal outcomes. CONCLUSION: Peak maternal serum gentamicin levels ranged from 13 to 25 microg/mL after a dose of 5.1 mg/kg. Single-dose gentamicin resulted in fetal serum peak levels that were closer to optimal neonatal values. Gentamicin clearance in the term fetus was similar to published values for the newborn infant. No adverse effects of high-dose therapy were noted.


Subject(s)
Anti-Bacterial Agents/administration & dosage , Chorioamnionitis/drug therapy , Gentamicins/administration & dosage , Adolescent , Adult , Apgar Score , Drug Administration Schedule , Female , Fetal Blood/chemistry , Gentamicins/blood , Gentamicins/pharmacokinetics , Humans , Infant, Newborn , Pregnancy , Pregnancy Outcome
6.
Clin Chem ; 51(3): 540-4, 2005 Mar.
Article in English | MEDLINE | ID: mdl-15738516

ABSTRACT

BACKGROUND: Newborns are being discharged from hospitals within 1-2 days of birth, before hyperbilirubinemia usually becomes clinically evident. We investigated the use of transcutaneous bilirubin (TcB) before discharge to determine whether it affects the use of laboratory bilirubin testing or decreases the number of neonates readmitted for hyperbilirubinemia within 7 days of initial discharge. METHODS: We retrospectively searched a clinical laboratory and hospital database to determine the number of births, newborn readmission rates for hyperbilirubinemia, length of stay, and the number of bilirubin measurements in the clinical laboratory ordered for all babies in the newborn unit at the University of Texas Medical Branch from August 2002 to March 2003 (before TcB testing) and from May 2003 to December 2003 (after TcB). RESULTS: Between August 2002 and December 2003, 8974 newborns (both vaginal and cesarean births) were admitted to the newborn nursery. Babies who did not fit the diagnosis-related group criteria of "normal newborn" were removed, leaving 6933 babies who were included in the study. April was considered a transition month and was not included in the study, leaving 6603 newborns to be included. Of these, 446 (6.8%) required phototherapy for treatment of hyperbilirubinemia before initial discharge. For the 8 months before and 8 months after initiation of TcB testing, the number of laboratory bilirubin measurements ordered per newborn did not change, nor did the mean (SD) length of stay for normal newborns [2.15 (1.1) days vs 2.12 (1.1) days; P = 0.53], nor days of treatment with phototherapy before discharge [2.9 (1.3) days vs 2.9 (1.3) days; P = 0.67]. By contrast, the number of readmissions per 1000 newborns per month for clinically significant hyperbilirubinemia decreased significantly (Wilcoxon rank-sums two-sample test, P = 0.044), from 4.5 (2.4) to 1.8 (1.7) after TcB testing was initiated. CONCLUSION: Access to TcB testing is associated with a reduction in the hospital readmission rate for hyperbilirubinemia within 7 days of the initial discharge.


Subject(s)
Bilirubin/blood , Hospitalization/statistics & numerical data , Hyperbilirubinemia/diagnosis , Neonatal Screening/methods , Female , Humans , Infant, Newborn , Length of Stay , Male , Medical Records Systems, Computerized , Retrospective Studies , Skin
7.
J Perinatol ; 24(12): 794-6, 2004 Dec.
Article in English | MEDLINE | ID: mdl-15558003

ABSTRACT

Zygomycosis is a rare fungal disease that occurs in compromised human hosts, including the preterm infant. The three clinical forms of zygomycosis are cellulitis, disseminated, and gastrointestinal, and the last often mimics necrotizing enterocolitis (NEC), complicating the diagnosis. This report details a case of primary gastrointestinal zygomycosis due to Absidia corymbifera, mimicking NEC, in a preterm infant, and emphasizes features that may lead to earlier diagnosis and treatment of future cases.


Subject(s)
Absidia , Enterocolitis, Necrotizing/diagnosis , Infant, Premature, Diseases/diagnosis , Mucormycosis/diagnosis , Diagnosis, Differential , Female , Humans , Infant, Newborn , Infant, Premature
8.
J Pediatr ; 144(6): 804-8, 2004 Jun.
Article in English | MEDLINE | ID: mdl-15192630

ABSTRACT

OBJECTIVE: We studied the efficacy and safety of electively providing surfactant to preterm infants with mild to moderate respiratory distress syndrome (RDS) not requiring mechanical ventilation. STUDY DESIGN: A 5-center, randomized clinical trial was performed on 132 infants with RDS, birth weight >or=1250 grams, gestational age or=40% for >or=1 hour, and no immediate need for intubation. Infants were randomly assigned to intubation, surfactant (Survanta, Ross Laboratories, Columbus, Ohio) administration, and expedited extubation (n=65) or expectant management (n=67) with subsequent intubation and surfactant treatment as clinically indicated. The primary outcome was duration of mechanical ventilation. RESULTS: Infants in the surfactant group had a median duration of mechanical ventilation of 2.2 hours compared with 0.0 hours for control infants, since only 29 of 67 control infants required mechanical ventilation (P=.001). Surfactant-treated infants were less likely to require subsequent mechanical ventilation for worsening respiratory disease (26% vs 43%, relative risk=0.60; 95% CI, 0.37, 0.99). There were no differences in secondary outcomes (duration of nasal continuous positive airway pressure, oxygen therapy, hospital stay, or adverse outcomes). CONCLUSIONS: Routine elective intubation for administration of surfactant to preterm infants >or=1250 grams with mild to moderate RDS is not recommended.


Subject(s)
Infant, Premature , Pulmonary Surfactants/therapeutic use , Respiratory Distress Syndrome, Newborn/drug therapy , Female , Humans , Infant, Newborn , Male , Respiration, Artificial
9.
Inflammation ; 26(5): 243-52, 2002 Oct.
Article in English | MEDLINE | ID: mdl-12238567

ABSTRACT

The mechanisms by which sublethal doses of endotoxin protect against hyperoxic lung injury are not completely understood. We hypothesized that endotoxin treatment would result in a decreased inflammatory response to hyperoxia and that this would be accompanied by activation of neutrophils (as evidenced by loss of L-selectin) in the peripheral circulation. Adult rats were injected with endotoxin 0.5 mg/kg prior to and 24 hr after onset of exposure to > or = 98% O2. After 56 hr of hyperoxia, pulmonary neutrophils were lower in the O2/endotoxin group compared to O2 controls as measured by myeloperoxidase in lung homogenates and neutrophil counts in bronchoalveolar lavage fluid. Circulating neutrophils were also significantly lower in the O2/endotoxin group compared to O2 controls at 56 hr. Expression of the neutrophil adhesion molecule, L-selectin, was lower at 4 and 24 hr in the endotoxin-treated rats compared to O2 controls. There were no differences at 48 hr. Expression of CD18 rose significantly in the O2/endotoxin group after 4 hr, but thereafter did not differ from O2 controls. In summary, endotoxin protection from O2 toxicity was associated with reduced neutrophils in the lung and a loss of L-selectin from peripheral blood neutrophils.


Subject(s)
Endotoxins/pharmacology , Hyperoxia/prevention & control , Lung/pathology , Neutrophils/drug effects , Animals , L-Selectin/analysis , Leukocyte Count , Male , Neutrophil Activation/drug effects , Neutrophil Infiltration/drug effects , Neutrophils/cytology , Neutrophils/metabolism , Oxygen/pharmacology , Protective Agents/pharmacology , Rats , Rats, Sprague-Dawley , Salmonella typhimurium/chemistry
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