Subject(s)
Abnormalities, Multiple/genetics , Chromosomes, Human, Pair 18 , Liver Cirrhosis/complications , Trisomy , Craniofacial Abnormalities/etiology , Fatal Outcome , Fetal Growth Retardation/etiology , Heart Defects, Congenital/etiology , Humans , Infant, Newborn , Liver Cirrhosis/congenital , Liver Cirrhosis/diagnosis , Male , Syndrome , Trisomy/diagnosisSubject(s)
Cytomegalovirus Infections/complications , Infant, Premature , Infant, Very Low Birth Weight , Lymphohistiocytosis, Hemophagocytic/virology , Cytomegalovirus Infections/diagnosis , Cytomegalovirus Infections/therapy , Fatal Outcome , Humans , Infant, Newborn , Lymphohistiocytosis, Hemophagocytic/diagnosis , Lymphohistiocytosis, Hemophagocytic/therapy , Male , Twins, DizygoticABSTRACT
BACKGROUND: In Japan, palivizumab was approved in 2002 for prophylaxis of severe respiratory syncytial virus disease in high-risk infants. In order to evaluate the efficacy and safety of this drug, a questionnaire survey was conducted. METHODS: A questionnaire was sent to member institutions of the Japan Neonatologist Association. The subjects were premature infants who were considered possible candidates for treatment with palivizumab. RESULTS: A total of 6302 case reports, including those of 2806 infants receiving palivizumab (group P) and 3496 infants not receiving palivizumab (group NP), respectively, were retrieved. Background characteristics revealed significant lower gestational age (GA) and birthweight for group P (P < 0.0001). Sex ratio did not differ significantly, while use of oxygen and mechanical ventilation in the neonatal intensive care unit, and presence of chronic lung disease were significantly higher for infants in group P (P < 0.0001). When comparison of hospitalization rate for respiratory symptoms was performed with stratification by eligibility criteria, in the group of infants born at 29-35 weeks GA the hospitalization rate was 4.0% and 5.7% in groups P and NP, respectively (P < 0.05). Multivariate analysis also showed that prophylaxis with palivizumab was the only variable that significantly decreased rate of hospitalization (odds ratio 0.630, P= 0.0053). The incidence of adverse events associated with the administration of palivizumab was low. CONCLUSION: In this non-randomized questionnaire survey, multivariate analysis showed that palivizumab significantly decreased the rate of hospitalization due to respiratory symptoms for infants born prematurely at 29-35 weeks GA. These data confirmed the efficacy and safety of palivizumab.
Subject(s)
Antibodies, Monoclonal/therapeutic use , Antiviral Agents/therapeutic use , Infant, Premature , Respiratory Syncytial Virus Infections/prevention & control , Antibodies, Monoclonal/adverse effects , Antibodies, Monoclonal, Humanized , Antiviral Agents/adverse effects , Chronic Disease , Female , Hospitalization/statistics & numerical data , Humans , Infant , Infant, Newborn , Japan/epidemiology , Logistic Models , Lung Diseases/epidemiology , Male , Multivariate Analysis , Palivizumab , Respiratory Syncytial Virus Infections/epidemiology , Risk FactorsSubject(s)
Abnormalities, Multiple , Developmental Disabilities , Hypothyroidism , Nose/abnormalities , Scalp/abnormalities , Tooth Loss , Abnormalities, Multiple/diagnosis , Abnormalities, Multiple/genetics , Abnormalities, Multiple/physiopathology , Abnormalities, Multiple/therapy , Child , Developmental Disabilities/diagnosis , Developmental Disabilities/genetics , Developmental Disabilities/physiopathology , Developmental Disabilities/therapy , Diagnosis, Differential , Female , Genetic Diseases, X-Linked , Humans , Hypothyroidism/diagnosis , Hypothyroidism/genetics , Hypothyroidism/physiopathology , Hypothyroidism/therapy , Mutation , Prognosis , Syndrome , Tooth Loss/diagnosis , Tooth Loss/genetics , Tooth Loss/physiopathology , Tooth Loss/therapy , Ubiquitin-Protein Ligases/geneticsABSTRACT
We report a case of hydromediastinum in a newborn associated with a peripherally inserted central venous catheter. A 9-day-old male infant with total parenteral nutrition via a peripherally inserted central venous catheter had acute respiratory distress. A chest radiograph showed a widened mediastinal shadow and left pleural effusion, and sonography revealed fluid collection in the mediastinum and bilateral hydrothorax. Sonography is useful in the diagnosis of hydromediastinum when infants treated with peripherally inserted central venous catheters have acute respiratory distress.
Subject(s)
Catheterization, Central Venous/adverse effects , Hydrothorax/diagnostic imaging , Hydrothorax/etiology , Mediastinum/diagnostic imaging , Respiratory Distress Syndrome, Newborn/etiology , Adult , Female , Humans , Infant, Newborn , Male , UltrasonographyABSTRACT
BACKGROUND: It is not known whether a moderate dose of oral iron supplementation would further enhance erythropoiesis in recombinant human erythropoietin (EPO)-treated very low-birthweight (VLBW) infants. METHODS: In total, 24 preterm infants with birthweights 750-1499 g were enrolled at the age of 14-28 days to receive 400 IU/kg per week EPO subcutaneously for 8 weeks. The infants were randomly allocated either to receive oral iron supplementation 4 mg/kg per day or to serve as controls. RESULTS: Hemoglobin and the absolute reticulocyte count in the iron supplementation and the control groups remained identical throughout the study period, whereas serum ferritin was significantly lower in the control group at study exit and follow up. Rates of treatment success (no need for transfusion and hemoglobin never below 8 g/dL) also did not differ between the groups. CONCLUSIONS: In this study we did not find a clear advantage in a moderate dose of oral iron supplementation on erythropoiesis in EPO-treated VLBW infants. Whether a higher dose would lead to enhanced erythropoiesis remains to be answered.
Subject(s)
Anemia, Neonatal/diagnosis , Anemia, Neonatal/drug therapy , Erythropoietin/administration & dosage , Ferric Compounds/administration & dosage , Ferritins/metabolism , Infant, Premature , Administration, Oral , Dose-Response Relationship, Drug , Drug Administration Schedule , Drug Therapy, Combination , Female , Ferritins/blood , Follow-Up Studies , Gestational Age , Humans , Infant, Newborn , Infant, Very Low Birth Weight , Injections, Subcutaneous , Male , Probability , Recombinant Proteins , Reticulocyte Count , Risk Assessment , Severity of Illness Index , Treatment OutcomeABSTRACT
Congenital central hypoventilation syndrome (CCHS or Ondine's curse; OMIM 209880) is a disorder characterized by an idiopathic failure of the automatic control of breathing. CCHS is frequently complicated with neurocristopathies such as Hirschsprung's disease (HSCR). The genes involved in the RET-GDNF signaling and/or EDN3-EDNRB signaling pathways have been analyzed as candidates for CCHS; however, only a few patients have mutations of the RET, EDN3, and GDNF genes. Recently, mutations of the PHOX2B gene, especially polyalanine expansions, have been detected in two thirds of patients. We studied the RET, GDNF, GFRA1, PHOX2A, PHOX2B, HASH-1, EDN1, EDN3, EDNRB, and BDNF genes in seven patients with isolated CCHS and three patients with HSCR. We detected polyalanine expansions and a novel frameshift mutation of the PHOX2B gene in four patients and one patient, respectively. We also found several mutations of the RET, GFRA1, PHOX2A, and HASH-1 genes in patients with or without mutations of the PHOX2B gene. Our study confirmed the prominent role of mutations in the PHOX2B gene in the pathogenesis of CCHS. Mutations of the RET, GFRA1, PHOX2A, and HASH-1 genes may also be involved in the pathogenesis of CCHS. To make clear the pathogenesis of CCHS, the analysis of more cases and further candidates concerned with the development of the autonomic nervous system is required.
Subject(s)
Sleep Apnea, Central/genetics , Age of Onset , Amino Acid Substitution , Child , Child, Preschool , DNA Mutational Analysis , Female , Glial Cell Line-Derived Neurotrophic Factor , Glial Cell Line-Derived Neurotrophic Factor Receptors , Humans , Infant , Male , Nerve Growth Factors/genetics , Polymorphism, Single Nucleotide , Proto-Oncogene Proteins/genetics , Proto-Oncogene Proteins c-ret , Receptor Protein-Tyrosine Kinases/genetics , Respiration, Artificial , SyndromeABSTRACT
We report a case of esophageal perforation caused by a malpositioned feeding tube in a neonate of extremely low birth weight, 632 g. The infant had respiratory distress, which increased rapidly when he was 6 days old. Radiography revealed right-sided hydrothorax that had not been evident a day earlier but no sign of a perforated esophagus. We performed sonography, which revealed fluid in the right pleural cavity and extra-esophageal placement of the feeding tube. Analysis of a fluid specimen obtained on thoracocentesis indicated that the fluid was feeding formula. The feeding tube's misplacement was confirmed sonographically by injecting a small amount of sterile distilled water into the tube and visualizing its entry into the pleural cavity. The feeding tube was removed, and antimicrobial agents were administered. When the infant was 15 days old, feeding resumed through another tube, the placement of which was verified radiographically. The infant was discharged when he was 118 days old with no severe complications, although he had mild chronic lung disease. Because radiography did not reveal the tube's misplacement in this case, we believe that the use of sonography can contribute to an early diagnosis of esophageal perforation in such cases.
