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91.

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.  相似文献   
92.
Since the discovery of aquaporin 4-IgG, a sensitive and highly specific biomarker of neuromyelitis optica (NMO), a wide range of syndromes have been recognized as being associated with this condition. This observation has led to new proposed terminology for the entire disorder, NMO spectrum disorders (NMOSD). The discovery of a pathogenic autoantibody and its target antigen has also facilitated basic research into the immunopathogenesis of the disease. Key advances include establishment of passive transfer animal models demonstrating the pathogenic potential of the autoantibody and confirming an important role of complement suggested by immunopathology of NMO brain lesions and of B-cell subsets, plasmablasts in particular. These discoveries have led to phase 1 clinical trials of targeted immunotherapy with potential for improved efficacy and less toxicity than current empiric immunosuppressant medications used to treat NMOSD. Randomized clinical trials are beginning to assess the efficacy and safety of a variety of immunotherapies in NMOSD. Therapeutic options are likely to increase, and improved outcomes in NMOSD patients are anticipated.  相似文献   
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Venous leg ulceration has a high recurrence rate. Patients with healed or frequently recurring venous ulceration are required to perform self‐care behaviours to prevent recurrence or promote healing, but evidence suggests that many find these difficult to perform. Bandura's self‐efficacy theory is a widely used and robust behaviour change model and underpins many interventions designed to promote self‐care in a variety of chronic conditions. By identifying areas where patients may experience difficulty in performing self‐care, interventions can be developed to strengthen their self‐efficacy beliefs in performing these activities successfully. There are currently a variety of self‐efficacy scales available to measure self‐efficacy in a variety of conditions; but not a disease‐specific scale for use with venous ulcer patients. The aim of this study, therefore, was to develop and validate a disease‐specific, patient‐focused self‐efficacy scale for patients with healed venous leg ulceration. This scale will need further validation studies; however, it is ready for use in clinical practice and will enable practitioners to identify those patients who may need additional support in performing self‐care activities to prevent recurrence.  相似文献   
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The most common genetic cause of neonatal diabetes and hyperinsulinism is pathogenic variants in ABCC8 and KCNJ11. These genes encode the subunits of the β‐cell ATP‐sensitive potassium channel, a key component of the glucose‐stimulated insulin secretion pathway. Mutations in the two genes cause dysregulated insulin secretion; inactivating mutations cause an oversecretion of insulin, leading to congenital hyperinsulinism, whereas activating mutations cause the opposing phenotype, diabetes. This review focuses on variants identified in ABCC8 and KCNJ11, the phenotypic spectrum and the treatment implications for individuals with pathogenic variants.  相似文献   
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A couple and their five-year-old daughter are in a car accident. The parents are not expected to survive. The child is transported to a children's hospital, and urgent treatment decisions must be made. Whom should the attending physician approach to make decisions for the child? When such cases arise in, for example, the hospitals where we work, the social worker or chaplain is instructed to use the Illinois Health Care Surrogacy Act as a guidepost to identify a decision-maker. But in our state and the country overall, the limitations of such statutes leave hospital workers to make a judgment call among friends, family, and clergy who may come forward. While surrogate decision-making statutes comprehensively address surrogate decision-makers for adults, a patchwork of laws—permanency statutes, kinship provider statutes, standby guardianship statutes, and, in some cases, surrogate decision-making statutes—provide variable decision-making pathways for children.  相似文献   
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