Amputation Versus Staged Reconstruction for Severe Fibular Hemimelia: Assessment of Psychosocial and Quality-of-Life Status and Physical Functioning in Childhood.

BACKGROUND: Fibular hemimelia, a congenital disorder characterized by the partial or complete absence of the fibula, tibial growth inhibition, and foot and ankle deformity and deficiency, is the most common deficiency of long bones. The purpose of the present study of children with congenital fibular hemimelia was to examine the functional and psychosocial outcomes at a minimum of 2 years after treatment either with amputation and a prosthesis or with reconstruction and lengthening. METHODS: Twenty children who were managed with primary amputation were compared with 22 children who were managed with staged limb reconstruction. The average age of the patients at the time of evaluation was 9 years (range, 5 to 15 years). Patients and parents completed psychosocial, quality-of-life, and satisfaction surveys. Patients underwent instrumented gait analysis and a timed 25 or 50-yard dash. The number and nature of surgical procedures were recorded from a retrospective chart review. RESULTS: Families of children managed with amputation had lower economic and educational levels and were more ethnically diverse compared with the families of children managed with limb reconstruction. Scores on psychosocial and quality-of-life surveys were comparable with those from healthy patient populations. Parents of males treated with amputation perceived a lower school-related quality of life for their child; socioeconomic and ethnic differences between groups might account for this finding. Statistically but not clinically significant differences were measured during instrumented gait analysis at a self-selected walking speed and during a timed 25 or 50-yard dash. The majority of patients and parents reported satisfaction with the treatment method selected and would select the same treatment method again. CONCLUSIONS: At this interim stage of growth, there were no significant functional or psychological differences between groups. Both groups were satisfied with the outcome in mid-childhood, irrespective of the selection of amputation or limb reconstruction. LEVEL OF EVIDENCE: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Performance on the NEPSY among children with spina bifida.

Usefulness of the NEPSY: A Developmental Neuropsychological Assessment (NEPSY) to assess attention/executive function skills was examined in a sample of 30 children with spina bifida and shunted hydrocephalus (SBSH; 20 females/10 males; age range: 6-12 years). Statistically significant moderate-to-strong correlations between the NEPSY and other measures of executive functioning were obtained. The strength of these associations decreased when controlling for intelligence as measured by the WISC-III. The Attention and Executive Function domain appeared more closely associated with tests requiring focused attention and an ability to shift set than those emphasizing more abstract reasoning, memory, or non-verbal problem-solving skills. The NEPSY appears useful in the assessment of specific executive function abilities in young individuals with SBSH. More research into the NEPSY and particularly its Attention and Executive Function domain is needed among individuals with SBSH before its use as a stand-alone tool apart from adjunctive cognitive assessment instruments.

Isolates of Verticillium dahliae Pathogenic to Crucifers Are of at Least Three Distinct Molecular Types.

ABSTRACT Diverse isolates of the soilborne wilt fungi Verticillium dahliae and V. albo-atrum were studied to understand the nature and origins of those infecting cruciferous hosts. All isolates from cruciferous crops produced microsclerotia, and the majority produced long conidia with a high nuclear DNA content; these isolates were divided into two groups by amplified fragment length polymorphism (AFLP) analysis. One group could be subdivided by other criteria such as rRNA sequences and mitochondrial DNA restriction fragment length polymorphism (RFLP) analysis. Two crucifer isolates were short spored and had a low nuclear DNA content. The results are consistent with the crucifer isolates being interspecific hybrids. The long-spored isolates are best regarded as amphihaploids (or allodiploids) with the AFLP groups probably each representing separate interspecific hybridization events. The short-spored crucifer isolates appear to be derived from interspecific hybrids and are here called 'secondary haploids'. Molecular evidence suggests that one parent in the crosses was similar to V. dahliae. The other parent of the amphihaploids seems to have been more similar to V. albo-atrum than to V. dahliae, but was distinct from all isolates of either species so far studied. The implications for the taxonomy of crucifer isolates are discussed and the use of the name V. longisporum, proposed elsewhere for just some of these isolates, is discouraged.

The PedsQL in pediatric patients with Spinal Muscular Atrophy: feasibility, reliability, and validity of the Pediatric Quality of Life Inventory Generic Core Scales and Neuromuscular Module.

For Phase II and III clinical trials in children with Spinal Muscular Atrophy (SMA), reliable and valid outcome measures are necessary. Since 2000, the American Spinal Muscular Atrophy Randomized Trials (AmSMART) group has established reliability and validity for measures of strength, lung function, and motor function in the population from age 2 years to 18 years. The PedsQL (Pediatric Quality of Life Inventory) Measurement Model was designed to integrate the relative merits of generic and disease-specific approaches, with disease-specific modules. The PedsQL 3.0 Neuromuscular Module was designed to measure HRQOL dimensions specific to children ages 2-18 years with neuromuscular disorders, including SMA. One hundred seventy-six children with SMA and their parents completed the PedsQL 4.0 Generic Core Scales and PedsQL 3.0 Neuromuscular Module. The PedsQL demonstrated feasibility, reliability, and validity in the SMA population. Consistent with the conceptualization of disease-specific symptoms as causal indicators of generic HRQOL, the majority of intercorrelations among the Neuromuscular Module Scales and the Generic Core Scales were in the medium to large range, supporting construct validity. For the purposes of a clinical trial, the PedsQL Neuromuscular Module and Generic Core Scales provide an integrated measurement model with the advantages of both generic and condition-specific instruments.

Accuracy in the prediction and estimation of adherence to bracewear before and during treatment of adolescent idiopathic scoliosis.

BACKGROUND: Because bracing for scoliosis may prevent curve progression, it is important to recognize nonadherence. We used temperature sensors to determine actual bracewear and examined: (1) the ability of a new pretreatment questionnaire to predict bracewear; (2) the ability of the physician and orthotist to predict bracewear before treatment and (3) the ability of physicians, orthotists, patients, and parents to accurately estimate bracewear during the first year of treatment. METHODS: Sixteen males and 108 females with adolescent idiopathic scoliosis were fitted with a Boston brace equipped with a temperature sensor and told that investigators were examining comfort. Before treatment, each patients completed an 18-item Brace-Beliefs Questionnaire (BBQ), and physicians/orthotists rated the likelihood that their patient would be adherent. During treatment, physicians, orthotists, patients, and parents provided estimates of daily bracewear. Data obtained at 1 to 3, 4 to 7, and 9 to 12 months into treatment were analyzed. RESULTS: Scores from the BBQ were related to actual adherence (r = 0.46, P < 0.001). No patient scoring more than 1 SD below the BBQ sample mean had an adherence level more than 40%. Correlations of physician/orthotist pretreatment predictions with actual adherence were minimal. Overall, patients wore the brace 47% of the prescribed time, although they were estimated to have worn it 64%, 66%, 72%, and 75% by physicians, orthotists, parents, and patients, respectively. Physicians/orthotists incorrectly identified at least 1 of every 4 nonadherers. CONCLUSIONS: Predicting a patient's adherence before treatment is difficult, but a pretreatment questionnaire may be helpful. During treatment, all respondents overestimated adherence. Health care providers should be mindful of overreports of bracewear and skeptical of their own assessments of adherence. CLINICAL RELEVANCE: Potential nonadherence may be predicted by a brief treatment-specific questionnaire. Treatment teams should not assume that patients follow their instructions or that family members are accurate sources of adherence information during treatment. Health care providers also should not assume that they can accurately predict adherence based on subjective expectations.

The impact on health-related quality of life from non-steroidal anti-inflammatory drugs, methotrexate, or steroids in treatment for juvenile idiopathic arthritis.

OBJECTIVE: To assess and compare the impact of medication treatments on health-related quality of life (HRQOL), family function, and medical status in children with juvenile idiopathic arthritis (JIA). METHODS: Fifty-seven children diagnosed with JIA were assessed by a pediatric rheumatologist and placed into one of three treatment groups: (1) non-steroidal anti-inflammatory; (2) methotrexate; or (3) steroids via IV methylprednisolone. Questionnaires were administered at baseline and 4-month follow-up. The attending pediatric rheumatologist provided additional medical information. RESULTS: Data document the impact of JIA on HRQOL, particularly on physical and pain domains. Steroid patients experienced improved HRQOL at follow-up relative to other groups, despite reporting more problems with side effects. CONCLUSION: These results demonstrate positive benefits of steroids in treating JIA children, despite the greatest incidence of adverse side effects.

Do you see what I see? Looking at scoliosis surgical outcomes through orthopedists' eyes.

STUDY DESIGN: A prospective study evaluated orthopedists' ratings of preoperative and postoperative cosmesis in adolescents undergoing posterior or anterior spinal fusion for idiopathic scoliosis. Measures evaluated how orthopedists rated outcome in relation to patient satisfaction with the surgical result. SUMMARY OF BACKGROUND DATA: The reliability of physician-derived data as a method for evaluating scoliosis surgical outcomes has not been sufficiently explored and has not been related to patient satisfaction ratings with the postoperative result. OBJECTIVES: The purpose of this study was to determine 1) the intrarater and interrater reliability estimates of orthopedists' ratings of scoliosis surgical outcomes and 2) the association between orthopedists' ratings and patient satisfaction ratings with the postoperative cosmesis. METHODS: Six orthopedists independently rated preoperative and postoperative color slides for cosmetic deformity in 41 patients with adolescent idiopathic scoliosis. Patients rated their satisfaction with the postoperative cosmesis 5 to 11 months after surgery. RESULTS: The intrarater and interrater reliabilities of orthopedists' assessments of scoliosis surgical outcomes ranged from fair to poor. Orthopedists' assessments of postoperative shoulder asymmetry significantly correlated with patient satisfaction ratings of cosmetic appearance following surgical correction of severe curves. Patient satisfaction ratings were unrelated to orthopedists' ratings of scar appearance, cosmetic deformity, and preoperative to postoperative cosmetic change. CONCLUSIONS: Intrarater and interrater reliability estimates were unacceptably low when evaluating spinal surgical outcomes in these 41 patients. Overall, orthopedists' ratings were not significantly correlated with patient satisfaction ratings of the postoperative result.

Characterization of homologues of the apple proliferation immunodominant membrane protein gene from three related phytoplasmas.

SUMMARY Homologues of the immunodominant membrane protein gene from apple proliferation (AP) phytoplasma have been cloned and sequenced for three further members of the AP subclade, namely European stone fruit yellows, peach yellow leaf roll and a European isolate of pear decline (PD). The putative translation products of all three were similar in size to that of AP and all had a transmembrane region towards the N-terminus and a large C-terminal hydrophilic domain, probably held on the outside of the cell membrane in vivo. Sequence similarities for the putative proteins were compared with interrelationships of the phytoplasmas as measured by rRNA gene sequence similarity. The proteins from AP and PD were more similar (57% identical in the major hydrophilic domain) than those for any other pair (31-34%), but these two phytoplasmas were not more closely related by rRNA gene sequences than other pairs. The possibility that the relative similarities of these proteins is related to the host is discussed. It is suggested that the similarity of the AP and PD proteins may reflect the fact that these two proteins have narrow plant host ranges in two closely related genera in the tribe Maloideae (family Rosaceae), whilst the other two have broader host ranges, mainly in the tribe Prunoideae.

Immunodominant membrane proteins from two phytoplasmas in the aster yellows clade (chlorante aster yellows and clover phyllody) are highly divergent in the major hydrophilic region.

The mechanisms by which phytoplasmas interact with their hosts are not understood. Mollicute membrane proteins may play a role in such interactions and therefore the amp genes encoding immunodominant proteins from two phytoplasmas, aster yellows and clover phyllody, which fall within the largest taxonomic subclade of the phytoplasmas, have been cloned and characterized. The putative translation products, antigenic membrane proteins (Amps), of these genes have properties which are typical for bacterial membrane proteins, and which suggest that each has a single large extracellular hydrophilic domain held by a transmembrane region near the C-terminus, with only a short C-terminal intracellular sequence. Both of the Amps characterized here have bacterial leader sequences which are cleaved during maturation. Whilst the signal peptide and transmembrane regions of the two proteins are very similar, the major hydrophilic domains are highly divergent in both size and sequence. The Amps from the two phytoplasmas are also different in structure and sequence from the immunodominant membrane proteins of three other phytoplasmas whose genes have been cloned previously.