RESUMO
PURPOSE: To quantitatively describe passive lower extremity range of motion in participants with spinal muscular atrophy (SMA) types 2 and 3, and to establish preliminary thresholds to identify individuals at risk for performing poorly on disease-specific motor function outcome measures. METHODS: Eighty participants with SMA types 2 and 3, enrolled in an international multicenter natural history study, were evaluated with lower extremity range of motion testing and the Hammersmith Functional Motor Scale-Expanded. RESULTS: A hip extension joint angle of -7.5° or less for SMA type 2 and 0° or less for SMA type 3 identified diminished motor ability with good sensitivity. For knee extension, a joint angle of -9.0° or less for SMA type 2 or 0° or less for SMA type 3 was similarly sensitive. CONCLUSIONS: Minimal hip and knee joint contractures were associated with diminished motor ability. Clinical trial designs should consider the effect of contractures on motor function.
Assuntos
Contratura/fisiopatologia , Articulação do Quadril/fisiopatologia , Articulação do Joelho/fisiopatologia , Extremidade Inferior/fisiopatologia , Transtornos Motores/fisiopatologia , Atrofia Muscular Espinal/fisiopatologia , Amplitude de Movimento Articular/fisiologia , Adulto , Feminino , Humanos , MasculinoRESUMO
OBJECTIVE: To assess whether oral ketamine is safe at higher dosages for sedating children and whether it may be an option for the control of chronic pain in children. STUDY DESIGN: A prospective study was performed on 12 children with chronic pain to identify the maximum tolerated dosage of oral ketamine. Participants were given 14 days of oral ketamine, 3 times daily, at dosages ranging from 0.25-1.5 mg/kg/dose. Participants were assessed for toxicity and for pain severity at baseline and on day 14 of treatment. RESULTS: Two participants, both treated at 1.5 mg/kg/dose, experienced dose-limiting toxicities (sedation and anorexia). One participant, treated at 1 mg/kg/dose, opted to stop ketamine treatment due to new pain on treatment. Nine participants completed their course of ketamine treatment. Of these 12 children, 5 experienced improvement in their pain scores, 2 with complete resolution of pain, lasting >4 weeks off ketamine treatment. CONCLUSION: Oral ketamine at dosages of 0.25-1 mg/kg/dose appears to be safe when given for 14 days to children with chronic pain.
Assuntos
Analgésicos/administração & dosagem , Dor Crônica/tratamento farmacológico , Ketamina/administração & dosagem , Administração Oral , Adolescente , Criança , Feminino , Humanos , Masculino , Projetos Piloto , Estudos Prospectivos , Adulto JovemRESUMO
OBJECTIVES: To determine if soluble CD40 ligand (sCD40L; formally CD154) levels vary with age and to identify age-dependent ranges in healthy pediatric and adult populations. STUDY DESIGN: sCD40L was measured in 25 neonates, 74 children (3 months-15 years of age) and 20 adults using an enzyme-linked immunosorbent assay. For age group comparisons, Mann-Whitney tests were performed. Correlation coefficients assessed relationships between plasma and serum sCD40L. RESULTS: Plasma sCD40L levels were higher in neonates than in all other age groups, (P <.001). All grouped pediatric plasma levels were significantly higher than in adults (P < .0001). There were no significant differences in plasma sCD40L between pediatric age groups. Serum levels were significantly higher in neonates than in any other age group (P < .0001). Pediatric and adult serum sCD40L levels were not significantly different. CONCLUSIONS: Plasma sCD40L levels are highest at birth and remain higher than those in adults throughout childhood. Reasons for such developmental changes remain to be investigated. Age-appropriate reference ranges should be used when sCD40L is being evaluated in pediatric disorders.
Assuntos
Envelhecimento/sangue , Ligante de CD40/sangue , Adolescente , Adulto , Fatores Etários , Criança , Pré-Escolar , Ensaio de Imunoadsorção Enzimática , Feminino , Sangue Fetal/química , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Valores de ReferênciaRESUMO
OBJECTIVE: To examine whether: (1) congenital human herpesvirus 6 (HHV6) and human herpesvirus 7 (HHV7) infections occur; whether (2) their manifestations differ from postnatal infections; and whether (3) HHV6 and HHV7 infections differ despite their close relatedness. STUDY DESIGN: HHV6 and HHV7 infections acquired congenitally and postnatally in normal children were compared using viral isolation, serology, reverse-transcription polymerase chain reaction (RT-PCR) and nested DNA-PCR for HHV6 variant A (HHV6A), HHV6 variant B (HHV6B), and HHV7. RESULTS: HHV6 DNA was detected in 57 (1%) of 5638 cord bloods. HHV7 DNA, however, was not detected in 2129 cord bloods. Congenital HHV6 infections differed from postnatal infections, which were acute febrile illnesses. Congenital infections were asymptomatic, 10% demonstrated reactivation at birth, and HHV6 DNA persistence in follow-up blood samples was significantly more frequent. One-third of congenital infections were HHV6A, whereas all postnatal infections were HHV6B. CONCLUSIONS: Congenital HHV6 infections occurred in 1% of births, similar to the rate for cytomegalovirus infection. Congenital infections were clinically and virologically distinct from postnatal infections. Congenital HHV7 infections, however, were not detected, suggesting considerable differences in transmission and pathogenesis in these closely related beta-herpesviruses.
Assuntos
Herpesvirus Humano 6 , Herpesvirus Humano 7 , Transmissão Vertical de Doenças Infecciosas , Infecções por Roseolovirus/congênito , Infecções por Roseolovirus/transmissão , Anticorpos Antivirais/sangue , Pré-Escolar , Transmissão de Doença Infecciosa , Feminino , Sangue Fetal/virologia , Seguimentos , Herpesvirus Humano 6/isolamento & purificação , Herpesvirus Humano 7/isolamento & purificação , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Prospectivos , Infecções por Roseolovirus/virologiaRESUMO
OBJECTIVE: To determine in healthy children after primary infection the persistence of human herpesvirus 6 (HHV6) DNA, the presence and frequency of HHV6 re-activation or re-infection, and the relationship of both to illness and the presence of human herpesvirus 7 (HHV7) infection. STUDY DESIGN: Children 1 to 12 years of age with previous HHV6 infection were prospectively evaluated by HHV6 and HHV7 DNA polymerase chain reaction (PCR) and reverse transcription (RT)-PCR for HHV6. HHV6 plasma antibody titers were measured. Illnesses were recorded by diary, and physician records were reviewed. RESULTS: HHV6 DNA was detected in >or=1 peripheral blood mononuclear cell (PBMC) samples in 89% of children. HHV6 reactivation and re-infection were detected by RT-PCR in 1.1% of samples. Detection of HHV6 DNA was intermittent in 76% of children and was not associated with cumulative rates of illness. Illness at a study visit was significantly associated with the absence of HHV6 and HHV7 DNA in PBMC samples and was not associated with HHV6 antibody titer or the presence of HHV6 DNA in saliva. CONCLUSIONS: HHV6 DNA persists in most children intermittently following primary infection and is unrelated to illness. Reactivation of HHV6 occurs in healthy children without apparent illness.