The group medical appointment (GMA) in haemophilia and von Willebrand's disease: a new development in outpatient paediatric care

Summary. The Group Medical Appointment (GMA) is a novel consultation form in which patients undergo individual consultations in each other’s presence. To compare participants’ experiences with GMA and Individual Medical Appointments (IMA), the usual standard of care, our team recently implemented the GMA for children aged 0–18 years with haemophilia or von Willebrand’s disease. Participants’ experiences with GMA were measured using a standardized QUOTE‐questionnaire. Of 100 addressed families, 53 participated in GMA. Of these 53 families, 38 parents (72%) and 14 adolescents (82%) filled in the questionnaire about the GMA. Patients not on prophylaxis were defined as less experienced and patients on prophylaxis, as experienced. Although parents were satisfied with both GMA and IMA (median score 8.0 vs. 9.0 of 10), a significant difference was demonstrated between less experienced and experienced parents. After GMA, less experienced parents were significantly more satisfied (median score 8.0 vs. 5.0; P‐value 0.006), felt more social support (82% vs. 30%; P‐value 0.005) and reported additional learning effects with regard to disease and treatment (64% vs. 0%; P‐value <0.001) than experienced parents. None of the less experienced parents reported privacy problems during GMA compared with 40% of experienced parents. In adolescents an identical trend was reported. Sixty‐six per cent of parents would join a GMA in the future and 87% would recommend a GMA to others. The GMA is a valuable addition in haemophilia and von Willebrand care, especially for less experienced patients. It leads to improved satisfaction, social support and improved information.     

Muscle architecture adaptations to knee extensor eccentric training: Rectus femoris vs. vastus lateralis

Introduction: Changes in muscle architecture induced by eccentric knee extensor training remain unclear, as well the adaptive responses of synergistic knee extensor muscles with different geometrical designs. Methods: Ultrasonography images were taken from rectus femoris (RF) and vastus lateralis (VL) of 20 male volunteers before and after a non‐training control period of 4 weeks, and additional evaluations were performed after 4, 8, and 12 weeks of isokinetic eccentric training. Results: RF and VL had significant changes in muscle architecture within the first 4 training weeks, and the adaptive response throughout the intervention was similar. Muscle thickness increased by around 7–10%, fascicle length increased 17–19%, and pennation angle was unchanged. Conclusions: Increased muscle thickness due to eccentric training was related to increased fascicle length and not to pennation angle changes. Although RF and VL have a different fascicular geometry, they had similar morphological adaptations to eccentric training. Muscle Nerve 48 : 498–506, 2013     

Judging conversation: How much is enough?

Background: Perceptual ratings provide a means of evaluating conversations involving individuals with aphasia. The influence of sampling segments of conversation and of segment length has not been established.

Aims: To investigate the impact of the duration of the conversational segment on judges' perceptual ratings of elements of conversations between individuals with aphasia and their conversation partners.

Methods & Procedures: A total of 64 speech pathology student judges rated two previously video-recorded conversations, elicited through a video-retelling task, between two individuals with severe aphasia and their respective partners via the Measure of Skill in Supported Conversation and the Measure of Participation in Supported Conversation (Kagan et al., 2004 Kagan, A., Winckel, J., Black, S., Duchan, J. F., Simmons-Mackie, N. and Square, P. 2004. A set of observational measures for rating support and participation in conversation between adults with aphasia and their conversation partners. Topics in Stroke Rehabilitation, 11: 67–83. [Taylor & Francis Online] , [Google Scholar]). Each judge was randomly assigned a segment from one or both conversations. The segment lengths were 3 minutes, 5 minutes, 10 minutes (for the longer conversation only), and the entire conversation (10:30 minutes and 19:17 minutes). The effect of segment duration and the interaction between conversation and segment duration were analysed for each rating scale using a 2?×?3 factorial analysis of variance.

Outcomes & Results: There was no significant main effect for segment duration for any of the four rating scales of the Measure of Skill in Supported Conversation and the Measure of Participation in Supported Conversation.

Conclusions: Aphasiologists who utilise these ratings of conversation as outcome measures can base the ratings on a segment of the conversation rather than the entire conversation. A 3- or 5-minute segment of a longer conversation on which no time limit has been placed is adequate to provide an accurate perceptual judgement.     

Impact of adjustment for quality on results of metaanalyses of diagnostic accuracy

BACKGROUND: We examined whether and to what extent different strategies of defining and incorporating quality of included studies affect the results of metaanalyses of diagnostic accuracy. METHODS: We evaluated the methodological quality of 487 diagnostic-accuracy studies in 30 systematic reviews with the QUADAS (Quality Assessment of Diagnostic-Accuracy Studies) checklist. We applied 3 strategies that varied both in the definition of quality and in the statistical approach to incorporate the quality-assessment results into metaanalyses. We compared magnitudes of diagnostic odds ratios, widths of their confidence intervals, and changes in a hypothetical clinical decision between strategies. RESULTS: Following 2 definitions of quality, we concluded that only 70 or 72 of 487 studies were of "high quality". This small number was partly due to poor reporting of quality items. None of the strategies for accounting for differences in quality led systematically to accuracy estimates that were less optimistic than ignoring quality in metaanalyses. Limiting the review to high-quality studies considerably reduced the number of studies in all reviews, with wider confidence intervals as a result. In 18 reviews, the quality adjustment would have resulted in a different decision about the usefulness of the test. CONCLUSIONS: Although reporting the results of quality assessment of individual studies is necessary in systematic reviews, reader wariness is warranted regarding claims that differences in methodological quality have been accounted for. Obstacles for adjusting for quality in metaanalyses are poor reporting of design features and patient characteristics and the relatively low number of studies in most diagnostic reviews.     

Native associations of early hematopoietic stem cells and stromal cells isolated in bone marrow cell aggregates

In suspensions of murine bone marrow, many stromal cells are tightly entwined with hematopoietic cells. These cellular aggregations appear to exist normally within the marrow. Previous studies showed that lymphocytes and stem cells adhered to stromal cells via vascular cell adhesion molecule 1 (VCAM1). Injection of anti-VCAM1 antibody into mice disrupts the aggregates, showing the importance of VCAM1 in the adhesion between stromal cells and hematopoietic cells in vivo. Early hematopoietic stem cells were shown to be enriched in aggregates by using a limiting-dilution culture assay. Myeloid progenitors responsive to WEHI-3CM in combination with stem cell factor (c-kit ligand) and B220- B-cell progenitors responsive to insulin-like growth factor-1 in combination with interleukin-7 are not enriched. We propose a scheme of stromal cell-hematopoietic cell interactions based on the cell types selectively retained within the aggregates. The existence of these aggregates as native elements of bone marrow organization presents a novel means to study in vivo stem cell-stromal cell interaction.     

Long-term Results of Comprehensive Clubfoot Release Versus the Ponseti Method: Which Is Better?

Background

Clubfoot can be treated nonoperatively, most commonly using a Ponseti approach, or surgically, most often with a comprehensive clubfoot release. Little is known about how these approaches compare with one another at longer term, or how patients treated with these approaches differ in terms of foot function, foot biomechanics, or quality-of-life from individuals who did not have clubfoot as a child.

Questions/purposes

We compared (1) focused physical and radiographic examinations, (2) gait analysis, and (3) quality-of-life measures at long-term followup between groups of adult patients with clubfoot treated either with the Ponseti method of nonsurgical management or a comprehensive surgical release through a Cincinnati incision, and compared these two groups with a control group without clubfoot.

Methods

This was a case control study of individuals treated for clubfoot at two separate institutions with different methods of treatment between 1983 to 1987. One hospital used only the Ponseti method and the other mainly used a comprehensive clubfoot release. There were 42 adults (24 treated surgically, 18 treated with Ponseti method) with isolated clubfoot along with 48 healthy control subjects who agreed to participate in a detailed analysis of physical function, foot biomechanics, and quality-of-life metrics.

Results

Both treatment groups had diminished strength and motion compared with the control subjects on physical examination measures; however, the Ponseti group had significantly greater ankle plantar flexion ROM (p < 0.001), greater ankle plantar flexor (p = 0.031) and evertor (p = 0.012) strength, and a decreased incidence of osteoarthritis in the ankle and foot compared with the surgical group. During gait the surgical group had reduced peak ankle plantar flexion (p = 0.002), and reduced sagittal plane hindfoot (p = 0.009) and forefoot (p = 0.008) ROM during the preswing phase compared with the Ponseti group. The surgical group had the lowest overall ankle power generation during push off compared with the control subjects (p = 0.002). Outcome tools revealed elevated pain levels in the surgical group compared with the Ponseti group (p = 0.008) and lower scores for physical function and quality-of-life for both clubfoot groups compared with age-range matched control subjects (p = 0.01).

Conclusions

Although individuals in each treatment group experienced pain, weakness, and reduced ROM, they were highly functional into early adulthood. As adults the Ponseti group fared better than the surgically treated group because of advantages including increased ROM observed at the physical examination and during gait, greater strength, and less arthritis. This study supports efforts to correct clubfoot with Ponseti casting and minimizing surgery to the joints, and highlights the need to improve methods that promote ROM and strength which are important for adult function.

Level of Evidence

Level III, prognostic study.     

Disseminated Fusarium oxysporum neurospinal infection

We report a case of disseminated meningospondylodiscitis in an elderly diabetic patient caused by Fusarium oxysporum. As the clinical presentation was nonspecific, the diagnosis of the condition could only be arrived at after laboratory and imaging studies. The diagnosis of the condition requires a high index of suspicion. Patient underwent thorough surgical debridement along with a short course of variconazole and remained asymptomatic after 36 months of diagnosis. Fusarium is a large genus of filamentous fungi widely distributed in soil and in association with plants. It is known to cause local infections (nail, cornea) in healthy humans and disseminated infection only in the immunocompromised.