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1.
J Pediatr Orthop ; 2024 Sep 16.
Artigo em Inglês | MEDLINE | ID: mdl-39282710

RESUMO

PURPOSE: Hip subluxation and dislocation are known problems for children with spinal muscular atrophy (SMA). Medical complexity of these children typically results in absence of monitoring and intervention for pathologic hips. Patterns of hip migration and acetabular morphology in SMA have not been described. This study examines the natural progression of hip instability across all types of SMA in a pediatric population. METHODS: Following institutional review board approval, a retrospective chart review of all children with SMA at our institution was performed. All x-rays taken before the age of 18 years containing adequate projections of the pelvis were measured for Reimer migration index (MI), acetabular depth ratio (ADR), and acetabular index (AI). Demographics and clinical data were collected including use of nusinersen, ambulatory status, contractures, and hip pain. Linear mixed effects model was fit to serial MI measures of individual hips with fixed effects consisting of SMA type, age at x-ray, and their interaction. ADR and AI measures were similarly modeled following conversion of raw values to z-scores based on the model developed by Novais et al Slope indicated rate of measure change as a function of age. RESULTS: Forty-five children (22 males) with SMA types 1 to 3 were included in this retrospective study. Six children were classified as type 1, 25 were type 2, and 14 were type 3. The interaction of age by SMA type was statistically significant (P=0.01), indicating a difference in the rate of hip subluxation between the 3 SMA types as measured by MI. By age 4, MI values were different from one another across all 3 groups (P<0.01). ADR decreased with age across all SMA types. The slopes of ADR regression lines were negative and statistically significant between the 3 groups (P=0.002). AI values were higher for all types of SMA, which is the opposite of expected in normal hips. CONCLUSIONS: Hip subluxation occurs across all SMA types, most rapidly in SMA type 1. Regression lines of ADR and AI compared with those seen in unaffected populations suggest hips in children with SMA demonstrate a difference in morphology of the acetabulum and do not follow normal adaptive remodeling. As treatments advance and the population of SMA patients becomes more mobile, there is an increased need to monitor hip instability in children with SMA, thus making orthopaedic management an important consideration.

2.
Foot (Edinb) ; 59: 102087, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38569253

RESUMO

OBJECTIVES: A common orthopedic issue for patients with spastic cerebral palsy (CP) is hindfoot varus deformity. One method of treatment is the split posterior tibialis tendon transfer (SPOTT). There is limited literature on the effect of SPOTT on foot progression angle (FPA) in children with CP who have equinovarus deformities. The objective of our study was to evaluate the change in FPA after SPOTT to determine if this procedure can improve FPA. RESEARCH QUESTION: This study aims to determine what axial changes are generated from a split posterior tibial tendon transfer in children with CP. METHODS: We performed a retrospective analysis of all ambulatory children with a diagnosis of CP who underwent SPOTT at our institution. Patients with bony rotational procedures were excluded. Descriptive statistics including mean and standard deviation (SD) were used to characterize continuous variables. Paired t-tests were used to evaluate outcomes, in which a target outcome was defined as a post-operative FPA between 0-10° of external rotation. RESULTS: 44 limbs were included. Demographics were as follows: 26/13 female/male; mean age[SD] (years): 9.8[3.5]; 30 hemiplegic, 9 diplegic, and 1 triplegic. Of the 44 limbs, 18 limbs had a target outcome, 4 had no change, and 22 had a non-target outcome. Of the 22 with an outcome outside of the target, 4 limbs trended away from a target outcome. The overall change in FPA measured was - 10.9 ± 14.7° (p < 0.0001) Age at time of surgery, CP involvement, pre-operative FPA, and GMFCS level were not predictors of outcome (p > 0.05). CONCLUSIONS: SPOTT produced a change of 10.9° external rotation in FPA post-operatively and its effects should be considered when planning a SEMLS.


Assuntos
Paralisia Cerebral , Transferência Tendinosa , Humanos , Paralisia Cerebral/cirurgia , Paralisia Cerebral/complicações , Transferência Tendinosa/métodos , Feminino , Criança , Masculino , Estudos Retrospectivos , Resultado do Tratamento , Adolescente
3.
Arch Bone Jt Surg ; 7(2): 136-142, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31211191

RESUMO

BACKGROUND: The goal of this study was to evaluate current physician ratings websites (PRWs) to determine which factors correlated to higher physician scores and evaluate physician perspective of PRWs. METHODS: This study evaluated two popular websites, Healthgrades.com and Vitals.com, to gather information on practicing physician members of the American Shoulder and Elbow Society database. A survey was conducted of the American Shoulder and Elbow Society (ASES) membership to gather data on the perception held by individual physicians regarding PRWs. RESULTS: We found that patients were more likely to give physicians positive reviews and the average overall score was 8.35 (3.75-10). Patient wait time (P=0.052) trended toward significance as a major factor in determining the overall scores, while ratings in both physician bedside manner (P=0.001) and physician/staff courtesy (P=0.002) were significant in reflecting the overall score given to the physician. According to our survey, a majority of the respondents were indifferent to highly unfavorable to PRWs (88%) and the validity of their ratings (78%). CONCLUSION: As PRWs become increasingly popular amongst patients in this digital age, it is critical to understand that the scores are not reflective of a significant proportion of the physicians' patient population. Physicians can use this study to determine what affects a patient's experience and focus efforts on improving patients' perception of quality, overall satisfaction, and overall care. Consumers may use this study to increase their awareness of the potential for significant sampling error inherent in PRWs when making decisions about their care.

5.
Neuropsychopharmacology ; 44(4): 683-694, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30127344

RESUMO

There are a number of clinically effective treatments for stress-associated psychiatric diseases, including major depressive disorder (MDD). Nonetheless, many patients exhibit resistance to first-line interventions calling for novel interventions based on pathological mechanisms. Accumulating evidence implicates altered glutamate signaling in MDD pathophysiology, suggesting that modulation of glutamate signaling cascades may offer novel therapeutic potential. Here we report that JHU-083, our recently developed prodrug of the glutaminase inhibitor 6-diazo-5-oxo-L-norleucine (DON) ameliorates social avoidance and anhedonia-like behaviors in mice subjected to chronic social defeat stress (CSDS). JHU-083 normalized CSDS-induced increases in glutaminase activity specifically in microglia-enriched CD11b+ cells isolated from the prefrontal cortex and hippocampus. JHU-083 treatment also reverses the CSDS-induced inflammatory activation of CD11b+ cells. These results support the importance of altered glutamate signaling in the behavioral abnormalities observed in the CSDS model, and identify glutaminase in microglia-enriched CD11b+ cells as a pharmacotherapeutic target implicated in the pathophysiology of stress-associated psychiatric conditions such as MDD.


Assuntos
Antígeno CD11b , Depressão/prevenção & controle , Diazo-Oxo-Norleucina , Glutaminase/efeitos dos fármacos , Hipocampo/efeitos dos fármacos , Inflamação/tratamento farmacológico , Córtex Pré-Frontal/efeitos dos fármacos , Pró-Fármacos , Animais , Comportamento Animal/efeitos dos fármacos , Depressão/etiologia , Diazo-Oxo-Norleucina/farmacologia , Modelos Animais de Doenças , Hipocampo/imunologia , Hipocampo/metabolismo , Inflamação/imunologia , Inflamação/metabolismo , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Córtex Pré-Frontal/imunologia , Córtex Pré-Frontal/metabolismo , Pró-Fármacos/farmacologia , Transdução de Sinais , Estresse Psicológico/complicações
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