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1.
Artigo em Inglês | MEDLINE | ID: mdl-31685276

RESUMO

OBJECTIVE: To use novel optical techniques to measure perioperative cerebral hemodynamics of diverse congenital heart disease (CHD) groups (two-ventricle, d-transposition of the great arteries [TGA], and single ventricle [SV]) and (1) compare CHD groups with healthy controls preoperatively and (2) compare preoperative and postoperative values within each CHD group. METHODS: Frequency-domain near-infrared spectroscopy and diffuse correlation spectroscopy were used to measure cerebral oxygen saturation, cerebral blood volume, cerebral blood flow index, cerebral oxygen extraction fraction (OEF, calculated using arterial oxygen saturation and cerebral oxygen saturation), and an index of cerebral metabolic rate of oxygen consumption in control and CHD neonates. Preoperative CHD measures were compared with controls. Preoperative and postoperative measures were compared within each CHD group. RESULTS: In total, 31 CHD neonates (7 two-ventricle, 11 TGA, 13 SV) and 13 controls were included. Only neonates with SV CHD displayed significantly lower preoperative cerebral blood flow index (P < .04) than controls. TGA and SV groups displayed greater OEF (P < .05) during the preoperative period compared with controls. Compared with the preoperative state, postoperative neonates with TGA had a greater arterial oxygen saturation with lower OEF. CONCLUSIONS: Differences in cerebral hemodynamics and oxygen metabolism were observed in diverse CHD groups compared with controls. Increased OEF appears to be a compensatory mechanism in neonates with TGA and SV. Studies are needed to understand the relationship of these metrics to outcome and their potential to guide interventions to improve outcome.

2.
Eur J Med Genet ; : 103802, 2019 Nov 05.
Artigo em Inglês | MEDLINE | ID: mdl-31698102

RESUMO

Mabry syndrome is a glycophosphatidylinositol (GPI) deficiency characterized by intellectual disability, distinctive facial features, intractable seizures, and hyperphosphatasia. We expand the phenotypic spectrum of inherited GPI deficiencies with novel bi-allelic phosphatidylinositol glycan anchor biosynthesis class O (PIGO) variants in a neonate who presented with intractable epilepsy and complex gastrointestinal and urogenital malformations.

3.
Handb Clin Neurol ; 162: 295-314, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31324316

RESUMO

While cardiorespiratory monitoring is standard for newborns in the NICU, monitoring of brain hemodynamics and oxygenation is usually sporadic and targeted to newborns with suspected or confirmed neurologic disorders. This is unfortunate, since critically ill newborns, both preterm and term-born, are at high risk of brain injury and would benefit from improved techniques for continuous monitoring of brain hemodynamics and oxygenation, in addition to monitoring of systemic hemodynamics and oxygenation. Near-infrared spectroscopy (NIRS) and, to a lesser extent, Doppler ultrasound are techniques that have been used in research and increasingly for clinical purposes to measure and monitor brain hemodynamics and oxygenation in newborns. NIRS monitoring can be useful for detection of diverse pathologic conditions that occur frequently in very preterm newborns and in selected populations of term newborns at risk for brain injury related to disturbances of systemic hemodynamics. This chapter reviews the current state of the art with regard to brain-monitoring techniques and the research directed at this important area, and it concludes with suggestions for the use of currently available tools to manage newborns at high risk of neurologic injury from disturbances in brain hemodynamics and oxygenation.

4.
Clin Perinatol ; 46(2): 311-325, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-31010562

RESUMO

Neonatal brain injury (NBI) remains a major contributor to neonatal mortality and long-term neurodevelopmental morbidity. Although therapeutic hypothermia is the only proven treatment to minimize brain injury caused by neonatal encephalopathy in term neonates, it provides incomplete neuroprotection. There are no specific drugs yet proven to prevent NBI in preterm neonates. This review discusses the scientific and emerging clinical trial data for several neuroprotective drugs in development, examining potential efficacy and safety concerns. Drugs with the highest likelihood of success and closest to clinical application include erythropoietin for term and preterm neonates and antenatal magnesium for preterm neonates.

5.
Genome Med ; 11(1): 12, 2019 02 28.
Artigo em Inglês | MEDLINE | ID: mdl-30819258

RESUMO

BACKGROUND: Neurodevelopmental disorders are genetically and phenotypically heterogeneous encompassing developmental delay (DD), intellectual disability (ID), autism spectrum disorders (ASDs), structural brain abnormalities, and neurological manifestations with variants in a large number of genes (hundreds) associated. To date, a few de novo mutations potentially disrupting TCF20 function in patients with ID, ASD, and hypotonia have been reported. TCF20 encodes a transcriptional co-regulator structurally related to RAI1, the dosage-sensitive gene responsible for Smith-Magenis syndrome (deletion/haploinsufficiency) and Potocki-Lupski syndrome (duplication/triplosensitivity). METHODS: Genome-wide analyses by exome sequencing (ES) and chromosomal microarray analysis (CMA) identified individuals with heterozygous, likely damaging, loss-of-function alleles in TCF20. We implemented further molecular and clinical analyses to determine the inheritance of the pathogenic variant alleles and studied the spectrum of phenotypes. RESULTS: We report 25 unique inactivating single nucleotide variants/indels (1 missense, 1 canonical splice-site variant, 18 frameshift, and 5 nonsense) and 4 deletions of TCF20. The pathogenic variants were detected in 32 patients and 4 affected parents from 31 unrelated families. Among cases with available parental samples, the variants were de novo in 20 instances and inherited from 4 symptomatic parents in 5, including in one set of monozygotic twins. Two pathogenic loss-of-function variants were recurrent in unrelated families. Patients presented with a phenotype characterized by developmental delay, intellectual disability, hypotonia, variable dysmorphic features, movement disorders, and sleep disturbances. CONCLUSIONS: TCF20 pathogenic variants are associated with a novel syndrome manifesting clinical characteristics similar to those observed in Smith-Magenis syndrome. Together with previously described cases, the clinical entity of TCF20-associated neurodevelopmental disorders (TAND) emerges from a genotype-driven perspective.


Assuntos
Anormalidades Craniofaciais/genética , Deficiências do Desenvolvimento/genética , Mutação INDEL , Deficiência Intelectual/genética , Hipotonia Muscular/genética , Síndrome de Smith-Magenis/genética , Fatores de Transcrição/genética , Adolescente , Criança , Pré-Escolar , Anormalidades Craniofaciais/patologia , Deficiências do Desenvolvimento/patologia , Feminino , Humanos , Lactente , Deficiência Intelectual/patologia , Masculino , Hipotonia Muscular/patologia , Síndrome de Smith-Magenis/patologia , Fatores de Transcrição/metabolismo , Adulto Jovem
6.
J Clin Neurophysiol ; 36(3): 186-194, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30882530

RESUMO

PURPOSE: Conventional video-EEG monitoring is required to diagnose seizures accurately in neonates. This tool is resource-intense and has limited availability in many centers. Seizure prediction models could help allocate resources by improving efficiency in which conventional video-EEG monitoring is used to detect subclinical seizures. The aim of this retrospective study was to create a neonate-specific seizure prediction model using clinical characteristics and EEG background findings. METHODS: We conducted a 3-year retrospective study of all consecutive neonates who underwent conventional video-EEG monitoring at a tertiary care pediatric hospital. Variables including age, EEG indication, high-risk clinical characteristics, and EEG background informed seizure prediction models based on a multivariable logistic regression model. A Cox proportional hazard regression model was used to construct time to first EEG seizure. RESULTS: Prediction models with clinical variables or background EEG features alone versus combined clinical and background EEG features were created from 210 neonates who met inclusion criteria. The combined clinical and EEG model had a higher area under the curve for combined sensitivity and specificity to 83.0% when compared to the clinical model (76.4%) or EEG model (66.2%). The same trend of higher sensitivity of the combined model was found for time to seizure outcome. CONCLUSIONS: While both clinical and EEG background features were predictive of neonatal seizures, the combination improved overall prediction of seizure occurrence and prediction of time to first seizure as compared with prediction models based solely on clinical or EEG features alone. With prospective validation, this model may improve efficiency of patient-oriented EEG monitoring.

8.
Epilepsia ; 60(3): e20-e24, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30790268

RESUMO

In a prospective cohort of 534 neonates with acute symptomatic seizures, 66% had incomplete response to the initial loading dose of antiseizure medication (ASM). Treatment response did not differ by gestational age, sex, medication, or dose. The risk of incomplete response was highest for seizures due to intracranial hemorrhage and lowest for hypoxic-ischemic encephalopathy, although the difference was not significant after adjusting for high seizure burden and therapeutic hypothermia treatment. Future trial design may test ASMs in neonates with all acute symptomatic seizure etiologies and could target neonates with seizures refractory to an initial ASM.

9.
Pediatr Res ; 85(7): 943-954, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30584262

RESUMO

Although seizures have a higher incidence in neonates than any other age group and are associated with significant mortality and neurodevelopmental disability, treatment is largely guided by physician preference and tradition, due to a lack of data from well-designed clinical trials. There is increasing interest in conducting trials of novel drugs to treat neonatal seizures, but the unique characteristics of this disorder and patient population require special consideration with regard to trial design. The Critical Path Institute formed a global working group of experts and key stakeholders from academia, the pharmaceutical industry, regulatory agencies, neonatal nurse associations, and patient advocacy groups to develop consensus recommendations for design of clinical trials to treat neonatal seizures. The broad expertise and perspectives of this group were invaluable in developing recommendations addressing: (1) use of neonate-specific adaptive trial designs, (2) inclusion/exclusion criteria, (3) stratification and randomization, (4) statistical analysis, (5) safety monitoring, and (6) definitions of important outcomes. The guidelines are based on available literature and expert consensus, pharmacokinetic analyses, ethical considerations, and parental concerns. These recommendations will ultimately facilitate development of a Master Protocol and design of efficient and successful drug trials to improve the treatment and outcome for this highly vulnerable population.

10.
J AAPOS ; 22(3): 242-244.e1, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29555514

RESUMO

Muscular dystrophy-dystroglycanopathy type A (MDDGA3), one of a group of diseases collectively known as congenital muscular dystrophies, is an alpha-dystroglycanopathy with characteristic brain and ocular abnormalities. We report the case of a 9-month-old boy with developmental delay whose family sought evaluation for esotropia. Subsequent examination, imaging, and testing revealed significant motor and cognitive delay, marked weakness with appendicular spasticity, and a diffuse brain malformation. In addition, the patient had poor visual acuity, nystagmus, optic nerve hypoplasia, bilateral retinal dysplasia and retinal dragging with a large vertical angle kappa, and an avascular peripheral retina. Genetic testing revealed two known heterozygous mutations in the POMGnT1 gene confirming MDDGA3. He was treated with botulinum toxin injections for his strabismus and continues to be followed, with planned laser ablation of the peripheral avascular retina.

11.
Semin Fetal Neonatal Med ; 23(3): 183-190, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29433814

RESUMO

Acute symptomatic seizures caused by either diffuse or focal perinatal hypoxic-ischemic insults and intracranial hemorrhage in term newborns make up the large majority of all neonatal seizures. Acute seizures are one of the most common neurological disorders in term newborns who require admission to the neonatal intensive care unit. Despite elucidation of seizure pathogenesis in this population using animal models, treatment is limited by a lack of good evidence-based guidelines because of a paucity of rigorously conducted clinical trials or prospective studies in human newborns. A result of this knowledge gap is that management, particularly drug choice, is guided by clinical experience rather than by data informing drug efficacy and safety. This review summarizes the common etiologies and pathogenesis of acute symptomatic seizures, and the current data informing their treatment, including potential novel drugs, together with a suggested treatment algorithm.

12.
Resuscitation ; 126: 83-89, 2018 05.
Artigo em Inglês | MEDLINE | ID: mdl-29476891

RESUMO

BACKGROUND: While therapeutic hypothermia (TH) is an effective neuroprotective therapy for neonatal hypoxic-ischemic encephalopathy, TH has not been demonstrated to improve outcome in other pediatric populations. Patients with acquired or congenital heart disease (CHD) are at high risk of both cardiac arrest and neurodevelopmental impairments, and therapies are needed to improve neurologic outcome. The primary goal of our study was to compare safety/efficacy outcomes in post-arrest CHD patients treated with TH versus controls not treated with TH. METHODS: Patients with CHD treated during the first 18 months after initiation of a post-arrest TH protocol (temperature goal: 33.5 °C) were compared to historical and contemporary post-arrest controls not treated with TH. Post-arrest data, including temperature, safety measures (e.g. arrhythmia, bleeding), neurodiagnostic data (EEG, neuroimaging), and survival were compared. RESULTS: Thirty arrest episodes treated with TH and 51 control arrest episodes were included. The groups did not differ in age, duration of arrest, post-arrest lactate, or use of ECMO-CPR. The TH group's post-arrest temperature was significantly lower than control's (33.6 ±â€¯0.2 °C vs 34.7 ±â€¯0.5 °C, p < 0.001). There was no difference between the groups in safety/efficacy measures, including arrhythmia, infections, chest-tube output, or neuroimaging abnormalities, nor in hospital survival (TH 61.5% vs control 59.1%, p = NS). Significantly more controls had seizures than TH patients (26.1% vs. 4.0%, p = 0.04). Almost all seizures were subclinical and occurred more than 24 h post-arrest. CONCLUSION: Our data show that pediatric CHD patients who suffer cardiac arrest can be treated effectively and safely with TH, which may decrease the incidence of seizures.

13.
JIMD Rep ; 40: 17-22, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-28887792

RESUMO

Ornithine transcarbamylase deficiency (OTCD) is an X-linked urea cycle disorder with variable expressivity in heterozygous females. While liver function testing is often abnormal in patients with OTCD, liver failure is uncommon on presentation. A 13-month-old female with no significant past medical history presented with irritability, right arm weakness, and decreased appetite. Initial workup revealed hepatic dysfunction with an INR of 3.4, ammonia level of 75 µmol/L, and abnormal brain MRI with gyral edema with restricted diffusion, and patchy signal abnormality in basal ganglia. The MRI findings led to a putative diagnosis of acute disseminated encephalomyelitis prompting corticosteroid treatment. As steroid treatment was begun, she developed significant hepatocellular dysfunction with ALT 2,222 U/L, AST 630 U/L, prolonged INR, and elevated ammonia (213 µmol/L). Neurologic signs resolved and her ammonia level decreased (43 µmol/L) without further intervention; however, she had ongoing acute liver failure with coagulopathy and episodic irritability, managed as seronegative autoimmune hepatitis with partial response to corticosteroid therapy. At 18 months of age she presented with severe irritability with markedly increased ammonia (417 µmol/L). Plasma amino acids obtained several days prior to this acute episode demonstrated elevation in glutamine (2,725 µmol/L) and alanine (1,459 µmol/L). Biochemical testing demonstrated elevation of urine orotic acid (>240.6 mmol/mol creatinine). Genetic testing confirmed a heterozygous nonsense mutation in the OTC gene (c.958C>T, R320X). After treatment with ammonia scavengers and a protein-restricted diet, hepatic function normalized and irritability resolved. The diagnosis of a urea cycle disorder should be considered in patients with unexplained hepatic dysfunction.

14.
Semin Fetal Neonatal Med ; 22(5): 321-327, 2017 10.
Artigo em Inglês | MEDLINE | ID: mdl-28811085

RESUMO

Neonatal seizures constitute the most frequent presenting neurologic sign encountered in the neonatal intensive care unit. Despite limited efficacy and safety data, phenobarbital continues to be used near-universally as the first-line anti-seizure drug (ASD) in neonates. The choice of second-line ASDs varies by provider and institution, and is still not supported by sufficient scientific evidence. In this review, we discuss the available evidence supporting the efficacy, mechanism of action, potential adverse effects, key pharmacokinetic characteristics such as interaction with therapeutic hypothermia, logistical issues, and rationale for use of neonatal ASDs. We describe the widely used neonatal ASDs, namely phenobarbital, phenytoin, midazolam, and levetiracetam, in addition to potential ASDs, including lidocaine, topiramate, and bumetanide.


Assuntos
Anticonvulsivantes/uso terapêutico , Fenobarbital/uso terapêutico , Convulsões/terapia , Frutose/análogos & derivados , Frutose/uso terapêutico , Humanos , Hipotermia Induzida , Recém-Nascido , Lidocaína/uso terapêutico , Midazolam/uso terapêutico , Convulsões/tratamento farmacológico , Topiramato , Resultado do Tratamento
15.
Neurology ; 89(9): 893-899, 2017 Aug 29.
Artigo em Inglês | MEDLINE | ID: mdl-28733343

RESUMO

OBJECTIVE: Although individual neonatal epilepsy syndromes are rare, as a group they represent a sizable subgroup of neonatal seizure etiologies. We evaluated the profile of neonatal epilepsies in a prospective cohort of newborns with seizures. METHODS: Consecutive newborns with seizures were enrolled in the Neonatal Seizure Registry (an association of 7 US children's hospitals). Treatment and diagnostic testing were at the clinicians' discretion. Neonates with seizures related to epileptic encephalopathies (without structural brain abnormalities), brain malformations, or benign familial epilepsies were included in this analysis. RESULTS: Among 611 consecutive newborns with seizures, 79 (13%) had epilepsy (35 epileptic encephalopathy, 32 congenital brain malformations, 11 benign familial neonatal epilepsy [BFNE], 1 benign neonatal seizures). Twenty-nine (83%) with epileptic encephalopathy had genetic testing and 24/29 (83%) had a genetic etiology. Pathogenic or likely pathogenic KCNQ2 variants (n = 10) were the most commonly identified etiology of epileptic encephalopathy. Among 23 neonates with brain malformations who had genetic testing, 7 had putative genetic etiologies. Six infants with BFNE had genetic testing; 3 had pathogenic KCNQ2 variants and 1 had a pathogenic KCNQ3 variant. Comorbid illnesses that predisposed to acute symptomatic seizures occurred in 3/35 neonates with epileptic encephalopathy vs 10/32 with brain malformations (p = 0.03). Death or discharge to hospice were more common among newborns with brain malformations (11/32) than those with epileptic encephalopathy (3/35, p = 0.01). CONCLUSIONS: Neonatal epilepsy is often due to identifiable genetic causes. Genetic testing is now warranted for newborns with epilepsy in order to guide management and inform discussions of prognosis.


Assuntos
Epilepsia/epidemiologia , Encéfalo/anormalidades , Encéfalo/fisiopatologia , Eletroencefalografia , Epilepsia/diagnóstico por imagem , Epilepsia/genética , Epilepsia/fisiopatologia , Feminino , Hospitais Pediátricos , Humanos , Recém-Nascido , Canal de Potássio KCNQ2/genética , Canal de Potássio KCNQ3/genética , Masculino , Estudos Prospectivos , Sistema de Registros , Convulsões/diagnóstico por imagem , Convulsões/epidemiologia , Convulsões/genética , Convulsões/fisiopatologia , Centros de Atenção Terciária , Estados Unidos/epidemiologia
16.
Pediatr Neurol ; 72: 19-24, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28558955

RESUMO

BACKGROUND: The purpose of this study was to characterize seizures among preterm neonates enrolled in the Neonatal Seizure Registry, a prospective cohort of consecutive neonates with seizures at seven pediatric centers that follow the American Clinical Neurophysiology Society's neonatal electroencephalography monitoring guideline. STUDY DESIGN: Of 611 enrolled neonates with seizures, 92 (15%) were born preterm. Seizure characteristics were evaluated by gestational age at birth for extremely preterm (<28 weeks, N = 18), very preterm (28 to <32 weeks, N = 18), and moderate to late preterm (32 to <37 weeks, N = 56) and compared with term neonates. RESULTS: Hypoxic-ischemic encephalopathy (33%) and intracranial hemorrhage (27%) accounted for the etiology in more than half of preterm neonates. Hypothermia therapy was utilized in 15 moderate to late preterm subjects with encephalopathy. The presence of subclinical seizures, monotherapy treatment failure, and distribution of seizure burden (including status epilepticus) was similar in preterm and term neonates. However, exclusively subclinical seizures occurred more often in preterm than term neonates (24% vs 14%). Phenobarbital was the most common initial medication for all gestational age groups, and failure to respond to an initial loading dose was 63% in both preterm and term neonates. Mortality was similar among the three preterm gestational age groups; however, preterm mortality was more than twice that of term infants (35% vs 15%). CONCLUSIONS: Subclinical seizures were more common and mortality was higher for preterm than term neonates. These data underscore the importance of electroencephalographic monitoring and the potential for improved management in preterm neonates.


Assuntos
Encéfalo/fisiopatologia , Hipóxia-Isquemia Encefálica/complicações , Hemorragias Intracranianas/complicações , Convulsões/etiologia , Anticonvulsivantes/uso terapêutico , Eletroencefalografia , Feminino , Idade Gestacional , Humanos , Hipóxia-Isquemia Encefálica/fisiopatologia , Recém-Nascido , Recém-Nascido Prematuro , Hemorragias Intracranianas/fisiopatologia , Masculino , Fenobarbital/uso terapêutico , Convulsões/tratamento farmacológico , Convulsões/fisiopatologia
17.
Paediatr Anaesth ; 27(3): 251-262, 2017 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-28177176

RESUMO

BACKGROUND: Deep anesthesia in adults may be associated with electroencephalographic (EEG) suppression and higher rates of postoperative complications. Little is known about the impact of anesthetic depth on short- or long-term outcomes in pediatrics. Brain activity monitoring may complement clinical signs of anesthetic depth. This prospective observational study aimed to assess the frequency and degree of profound EEG suppression using multichannel EEG in children during sevoflurane general anesthesia. METHODS: Children aged 0-40 months who required general anesthesia for elective surgery were included. Continuous EEG recordings were performed starting from when anesthesia began and until recovery. Discontinuity was defined as EEG amplitude <25 uV, lasting ≥2 s, and observed in all electrodes across the scalp. Frequency, duration, and inter-event interval of discontinuity events were measured. Relationships between discontinuity events and postnatal age, endtidal sevoflurane concentration (etSEVO), and multiple clinical parameters were analyzed. RESULTS: Discontinuity events were observed in 35/68 children, with a median duration of 10 s (95%CI: 8-12) and a median of 4 events per patient (95%CI: 2-7). Children who had discontinuity events were younger (5.5 months, 95%CI: 3.6-6.5) compared to children who did not have discontinuity events (10.2 months, 95%CI: 6.1-14); (difference between medians, 4.7 months, 95%CI: 2.3-8, P = 0.0002). Younger infants exhibited a higher number of discontinuity events, and the incidence decreased with postnatal age (r68 = -0.53, P < 0.0001). The majority of discontinuity events were observed during the first 30 min of anesthesia (66.4% total events), where etSEVO was >3%. Few discontinuity events were observed during maintenance and none during emergence. Blood pressure, heart rate, tissue oxygen saturation, and endtidal CO2 partial pressure did not change during these events. CONCLUSIONS: Electroencephalographic monitoring may complement clinical signs in providing information about brain homeostasis during general anesthesia. The impact of discontinuity events on immediate and long-term outcomes merits further study.


Assuntos
Anestesia Geral/métodos , Anestésicos Inalatórios , Eletroencefalografia/efeitos dos fármacos , Éteres Metílicos , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Prospectivos , Sevoflurano , Tempo
18.
J Pediatr ; 181: 42-48.e2, 2017 02.
Artigo em Inglês | MEDLINE | ID: mdl-27837950

RESUMO

OBJECTIVE: To determine whether brain volume is reduced at 1 year of age and whether these volumes are associated with neurodevelopment in biventricular congenital heart disease (CHD) repaired in infancy. STUDY DESIGN: Infants with biventricular CHD (n = 48) underwent brain magnetic resonance imaging (MRI) and neurodevelopmental testing with the Bayley Scales of Infant Development-II and the MacArthur-Bates Communicative Development Inventories at 1 year of age. A multitemplate based probabilistic segmentation algorithm was applied to volumetric MRI data. We compared volumes with those of 13 healthy control infants of comparable ages. In the group with CHD, we measured Spearman correlations between neurodevelopmental outcomes and the residuals from linear regression of the volumes on corrected chronological age at MRI and sex. RESULTS: Compared with controls, infants with CHD had reductions of 54 mL in total brain (P = .009), 40 mL in cerebral white matter (P <.001), and 1.2 mL in brainstem (P = .003) volumes. Within the group with CHD, brain volumes were not correlated with Bayley Scales of Infant Development-II scores but did correlate positively with MacArthur-Bates Communicative Development Inventory language development. CONCLUSIONS: Infants with biventricular CHD show total brain volume reductions at 1 year of age, driven by differences in cerebral white matter. White matter volume correlates with language development, but not broader developmental indices. These findings suggest that abnormalities in white matter development detected months after corrective heart surgery may contribute to language impairment. TRIAL REGISTRATION: ClinicalTrials.gov: NCT00006183.


Assuntos
Cardiopatias Congênitas/cirurgia , Desenvolvimento da Linguagem , Imagem por Ressonância Magnética/métodos , Substância Branca/diagnóstico por imagem , Substância Branca/crescimento & desenvolvimento , Desenvolvimento Infantil/fisiologia , Pré-Escolar , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/mortalidade , Humanos , Lactente , Recém-Nascido , Modelos Lineares , Masculino , Testes Neuropsicológicos , Tamanho do Órgão , Valor Preditivo dos Testes , Prognóstico , Valores de Referência , Taxa de Sobrevida , Cirurgia Torácica/métodos
19.
J Pediatr ; 181: 298-301.e1, 2017 02.
Artigo em Inglês | MEDLINE | ID: mdl-27829512

RESUMO

We aimed to define determinants of duration of treatment for acute symptomatic neonatal seizures in a contemporary multicenter observational cohort study. After adjustment for potential confounders, only study site and seizure etiology remained significantly associated with the chance of continuing antiseizure medication after discharge to home.


Assuntos
Anticonvulsivantes/uso terapêutico , Convulsões/tratamento farmacológico , Estudos de Coortes , Eletroencefalografia , Feminino , Humanos , Recém-Nascido , Masculino , Estudos Prospectivos , Sistema de Registros , Convulsões/etiologia , Fatores de Tempo
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