ABSTRACT
Neurological involvement is frequent in inherited metabolic disease of the intoxication type. Hyperammonemic coma related to these diseases may cause severe neurological sequelae. Early optimal treatment is mandatory combining metabolite scavengers (MS) and sometimes continuous veno-venous hemodialysis (CVVHD). We aimed to describe the therapeutic management of hyperammonemia in neonates upon diagnosis of their metabolic disease and to compare neonates managed with MS alone or with both MS and CVVHD. We conducted a retrospective study including all neonates admitted for initial hyperammonemia to the pediatric intensive care unit of a Reference Center of Inherited Metabolic Diseases, between 2001 and 2012. The study included 35 neonates. Before admission, MS were initiated for 11 neonates. At admission, the median ammonia levels were 391 µmol/L and were significantly lower in neonates who received MS before admission. At admission, ammonia levels were 644 µmol/L in dialyzed and 283 µmol/L in non-dialyzed neonates. The median time to reach a 50% decrease of the initial ammonia levels was significantly shorter in dialyzed neonates; however, the normalization of ammonia levels was similar between dialyzed and non-dialyzed neonates. Hemodynamic disorders were more frequent in dialyzed neonates. CONCLUSION: MS represent an effective treatment for hyperammonemia and should be available in all pediatric units to avoid the need for CVVHD. Although CVVHD enhances the kinetics of toxic metabolite decrease, it is associated with adverse hemodynamic effects.
Subject(s)
Antimetabolites/therapeutic use , Continuous Renal Replacement Therapy/methods , Hyperammonemia/therapy , Renal Dialysis/methods , Combined Modality Therapy , Critical Illness , Female , Humans , Hyperammonemia/diagnosis , Infant, Newborn , Male , Retrospective Studies , Treatment OutcomeABSTRACT
Preterm birth remains a public health priority given that one child out of ten is born before 37 weeks of gestation. Survival without major neonatal morbidity has increased in high-income countries, in particular in France and in cases of extreme preterm birth before 27 weeks of gestation. Rate of severe handicaps, such as cerebral palsy, is probably decreasing, but specific cognitive disabilities in a variety of domains remain frequent, interfering with normal learning abilities at school and explaining the high rate of special education needs. Prevalence of sequelae increases when gestational age at birth decreases. However, because there are more moderate to late preterm children compared to very preterm children, the absolute number of children with specific cognitive or neurological disabilities is equivalent in these two groups. Better characterization of the development in a recent cohort of very preterm children is necessary to improve the early detection of variations in normal neurodevelopment and to propose trials with remediation actions targeting working memory and language for example. These protocols could decrease the rates of learning disabilities at school.
Subject(s)
Child Behavior Disorders , Child Behavior , Child Development , Cognition Disorders , Developmental Disabilities , Nervous System/growth & development , Child , Child Behavior Disorders/etiology , Cognition Disorders/etiology , Developmental Disabilities/etiology , Humans , Infant, Newborn , Infant, Premature , Risk FactorsABSTRACT
The very preterm birth rate has increased in the past few years. Despite advances in neonatal medicine, neurodevelopmental sequelae have not decreased, despite a perinatal plan published in France in 1994. We conducted an epidemiological comparative survey at Rouen University Hospital in order to analyze morbidity, mortality, and care of very of premature infants by comparing the years 2000, 2005, and 2010. This hospital draws on an area of 17,000 births per year. Our survey was a single-center prospective, descriptive, and comparative study. The three cohorts had the same characteristics and the mortality rate was constant for 11 years. Use of medically assisted procreation and maternal age increased over this period. Chorioamnionitis halved, whereas duration of intrapartum antibiotic therapy increased. Neonatal morbidity was stable for hyaline membrane disease, bronchopulmonary dysplasia, maternofetal or nosocomial infections, and necrotizing enterocolitis. Regarding neurological complications, intraventricular hemorrhages decreased and white matter lesions remained constant. The rate of severe retinopathy remained low. The duration of parenteral nutrition and assisted ventilation, use of postnatal corticosteroids, and length of hospitalization decreased. The breastfeeding rate has increased since 2000 in parallel with postnatal growth restriction : 39% of the premature infants had a weight under the 10th percentile at hospital discharge. Our study allowed us to follow up the changes in neonatal epidemiological characteristics, the mortality and morbidity of extreme premature infants over a period of 11 years and showed few significant changes. Knowledge of medical practices is essential to improve the short- and long-term outcome of premature infants.
