CVID-Associated Intestinal Disorders in the USIDNET Registry: An Analysis of Disease Manifestations, Functional Status, Comorbidities, and Treatment.

Common variable immunodeficiency (CVID) has been subdivided into five phenotypes, including one marked by non-infectious enteropathies that lead to significant morbidity and mortality. We examined a large national registry of patients with CVID to better characterize this population and understand how the presence of enteropathy influences nutritional status, patient function, and the risk of additional non-infectious disorders in CVID patients. We also sought to illustrate the range of treatment strategies for CVID-associated enteropathies. We extracted patient data from the United States Immunodeficiency Network (USIDNET) database, which included 1415 patients with CVID, and compared those with and without intestinal disorders. Demographic and genetic profiles, functional status, and treatments targeting intestinal disorders are reported. Intestinal disorders were present in 20% of patients with CVID, including chronic diarrhea, inflammatory bowel disease, malabsorption, and others. Compared to those without enteropathies, this patient subset exhibited significantly lower Karnofsky-Lansky functional scores, greater reliance on nutritional support, higher rates of vitamin deficiencies, and increased prevalence of hematologic disorders, liver disease, pulmonary disease, granulomatous disease, and lymphoma. Genetic data were reported for only 5% of the cohort. No mutations segregated significantly to patients with or without intestinal disease. Corticosteroids were most frequently used for treatment. Patients with CVID-associated intestinal disorders exhibit higher rates of autoimmune and inflammatory comorbidities, lymphoma, malnutrition, and debility. We review recent studies implicating specific pathways underlying this immune dysregulation. Further studies are needed to evaluate the role of targeted immunomodulatory therapies for CVID-associated intestinal disorders.

CVID-associated intestinal disorders in the USIDNET registry: An analysis of disease manifestations, functional status, comorbidities, and treatment.

Common variable immunodeficiency (CVID) has been subdivided into five phenotypes, including one marked by non-infectious enteropathies that lead to significant morbidity and mortality. We examined a large national registry of patients with CVID to better characterize this population and understand how the presence of enteropathy influences nutritional status, patient function, and the risk of additional non-infectious disorders in CVID patients. We also sought to illustrate the range of treatment strategies for CVID-associated enteropathies. We extracted patient data from the United States Immunodeficiency Network (USIDNET) database, which included 1415 patients with CVID, and compared those with and without intestinal disorders. Demographic and genetic profiles, functional status, and treatments targeting intestinal disorders are reported. Intestinal disorders were present in 20% of patients with CVID, including chronic diarrhea, inflammatory bowel disease, malabsorption, and others. Compared to those without enteropathies, this patient subset exhibited significantly lower Karnofsky-Lansky functional scores, greater reliance on nutritional support, higher rates of vitamin deficiencies, and increased prevalence of hematologic disorders, liver disease, pulmonary disease, granulomatous disease, and lymphoma. Genetic data were reported for only 5% of the cohort. No mutations segregated significantly to patients with or without intestinal disease. Corticosteroids were most frequently used for treatment. Patients with CVID-associated intestinal disorders exhibit higher rates of autoimmune and inflammatory comorbidities, lymphoma, malnutrition, and debility. We review recent studies implicating specific pathways underlying this immune dysregulation. Further studies are needed to evaluate the role of targeted immunomodulatory therapies for CVID-associated intestinal disorders.

Adverse Reactions to Biologic Medications Used in Allergy and Immunology Diseases.

PURPOSE OF REVIEW: The use of biologic therapies has risen exponentially over recent years, allowing for unprecedented disease control within numerous areas of Allergy/Immunology. With this expanded use, awareness and understanding of adverse reactions to biologic agents have also increased. RECENT FINDINGS: Multiple biologic adverse reaction phenotypes have been described, but significant overlap in clinical features across phenotypes exists. Given considerable phenotypic overlap, a targeted testing approach may not always be clear, and more recent classifications focus on management decision making using tools of diagnostic challenges and rapid drug desensitizations, guiding clinicians in developing a management plan when the exact underlying mechanism is not clearly known. With increased clinical experience with omalizumab, mepolizumab, reslizumab, benralizumab, dupilumab, tezepelumab, rituximab, and TNF-inhibitors, there is a growing appreciation to the spectrum and particularities of adverse reactions to these agents which are outlined in this review. Our understanding of the clinical presentation and management of adverse reactions to biologic medications encountered in Allergy/Immunology has grown. Opportunities remain to further define optimal diagnostic and management strategies for these reactions.

Identifying barriers to the care of the rheumatoid hand in China: comparing attitudes of rheumatologists and hand surgeons.

AIM: In China, hand surgeons treat fewer rheumatoid arthritis (RA) patients compared to other countries. We investigated whether physician and surgeon knowledge, attitudes and practices regarding RA hand deformities reflect current evidence and may contribute to the low utilization of surgery. METHOD: We surveyed hand surgeons and rheumatologists at three tertiary hospitals in Beijing, China. Questionnaires were developed from literature and expert review to assess their knowledge, attitudes and practice patterns related to rheumatoid hand surgery. RESULTS: Thirty-five hand surgeons and 59 rheumatologists completed the survey. Roughly one-third felt that the rheumatologists and hand surgeons agree on how to manage RA hand deformities. One-fifth of rheumatologists and 29% of hand surgeons believed that drug therapy can correct hand deformities, which contradicts current evidence. Likewise, 30% and 14%, respectively, recommended surgery for early-stage hand sequelae that do not meet current indications for surgery. Over 80% of surgeons and rheumatologists had no exposure to the other specialty during training and felt their training on the treatment of rheumatoid hand deformities was inadequate. CONCLUSION: Although we found similar interspeciality disagreement in China as is seen in the United States, there appears to be less interaction through training and consultations. Our results also indicate potential deficits in training and unawareness of evidence and indications for rheumatoid hand surgery. These findings help to explain why surgery for rheumatoid hand deformities is rare in China; doctors have fewer opportunities to collaborate across specialties and may not be able to select appropriate candidates for surgery.

Barriers to Reconstructive Hand Surgery for Rheumatoid Arthritis in China: A Multicenter Survey of Patients and Physicians.

BACKGROUND: China has a similar rheumatoid arthritis (RA) disease burden compared with other countries, yet RA patients rarely receive surgical treatment for hand deformities that limit function and impact appearance. We investigated potential barriers to rheumatoid hand surgery in China. METHODS: Patients with RA, rheumatologists, and hand surgeons at 3 large tertiary hospitals in Beijing completed questionnaires that assess knowledge and attitudes surrounding RA hand surgery. We calculated descriptive statistics and compared responses among groups using chi-square and Fisher exact tests as appropriate. RESULTS: One hundred RA patients with hand deformities and 94 physicians completed the surveys. No patients had received hand surgery, and just 13% were aware of this treatment option. Patients and physicians most frequently cited uncertain effectiveness of surgery, high cost, and risk of surgical complications as potential barriers to hand reconstruction. Rheumatologists reported low rates of referral to hand surgeons (39% referred <5% of the time and 31% never referred). Most hand surgeons (69%) had not performed metacarpophalangeal arthroplasty, a common procedure for RA hand deformities, within the past year. Some had never performed this operation. CONCLUSIONS: This survey revealed multiple barriers that support previous observations of infrequent reconstruction of RA hand deformities in China. These obstacles can be addressed through patient education, coordination between specialists, and more robust hand surgeon training in common RA procedures through the application of international plastic surgery collaborations.

Hand Function and Appearance following Reconstruction for Congenital Hand Differences: A Qualitative Analysis of Children and Parents.

BACKGROUND: Congenital hand differences induce social, psychological, and functional challenges for children. However, little is known about how children perceive their outcomes after reconstructive or what concerns children have. METHODS: A total of 33 children (aged 6 to 17 years) who were treated for congenital hand differences, and their parents, participated in qualitative, semistructured interviews regarding the child's hand function and appearance. Discussion focused on the influence of congenital hand differences on the child's daily activities, school, and participation in sports and music. The interviews were open format to allow for spontaneous emergence of relevant themes followed by guided questioning. The interviews were transcribed verbatim and analyzed using qualitative coding, iterative comparisons, and frequency analysis to reveal perceptions of children and parents. RESULTS: In this sample, 73 percent of children and parents reported difficulty with hand function. Children experienced difficulties with personal care (58 percent), school activities (30 percent), and household tasks (27 percent). Children were bothered by hand appearance (48 percent), pain (30 percent), and weakness (24 percent). Complex anomalies were associated with greater disability and limitation in sports and music. CONCLUSIONS: Children with congenital hand differences are concerned with the aesthetics of their hands and limitations in their ability to perform activities. Children were often discouraged by activities that their peers accomplished easily, but with increasing age demonstrated adaptive behaviors to accommodate in their "own way," suggesting the uniqueness of their limitations. Patients may benefit from early hand therapy guided toward areas of concern to enhance functional adaptation.

Applying the Patient-Reported Outcomes Measurement Information System to Assess Upper Extremity Function among Children with Congenital Hand Differences.

BACKGROUND: Few studies have evaluated self-assessment tools among children with congenital hand differences. The authors compared three upper extremity disability instruments with the Patient-Reported Outcomes Measurement Information System (PROMIS) Pediatric Upper Extremity Item Bank. METHODS: Thirty-three children (aged 6 to 17 years) with congenital hand differences completed the Pediatric Outcomes Data Collection Instrument; the Michigan Hand Outcomes Questionnaire; the Disabilities of the Arm, Shoulder, and Hand questionnaire; and the PROMIS Upper Extremity short form and computerized adaptive test. Hand function was also assessed, and construct validity and feasibility were examined. RESULTS: PROMIS demonstrated good construct validity. Short form and computerized adaptive test were highly correlated with Disabilities of the Arm, Shoulder, and Hand questionnaire scores (r = 0.80, p < 0.001) and Pediatric Outcomes Data Collection Instrument domains (r = 0.70, p < 0.001). PROMIS was moderately correlated with the Michigan Hand Outcomes Questionnaire (r = 0.40, p < 0.05). PROMIS scores also correlated with grip (r = 0.60, p < 0.001) and pinch strength (r = 0.50, p < 0.001). Compared with the Pediatric Outcomes Data Collection Instrument and the Disabilities of the Arm, Shoulder, and Hand and Michigan Hand Outcomes questionnaires, PROMIS required the least time to complete with fewer children requiring assistance. CONCLUSION: The Patient-Reported Outcomes Measurement Information System is highly correlated with both functional assessment and self-reported function among children with congenital hand differences.

Medical treatment decision making after total avulsion brachial plexus injury: a qualitative study.

OBJECT: Complete avulsion traumatic brachial plexus injuries (BPIs) can be treated using nerve and musculoskeletal reconstruction procedures. However, these interventions are most viable within certain timeframes, and even then they cannot restore all lost function. Little is known about how patients make decisions regarding surgical treatment or what impediments they face during the decision-making process. Using qualitative methodology, the authors aimed to describe how and why patients elect to pursue or forego surgical reconstruction, identify the barriers precluding adequate information transfer, and determine whether these patients are satisfied with their treatment choices over time. METHODS: Twelve patients with total avulsion BPIs were interviewed according to a semi-structured guide. The interview transcripts were qualitatively analyzed using the systematic inductive techniques of grounded theory to identify key themes related to the decision-making process and long-term satisfaction with decisions. RESULTS: Four decision factors emerged from our analysis: desire to restore function, perceived value of functional gains, weighing the risks and costs of surgery, and having concomitant injuries. Lack of insurance coverage (4 patients), delayed diagnosis (3 patients), and insufficient information regarding treatment (4 patients) prevented patients from making informed decisions and accessing care. Three individuals, all of whom had decided against reconstruction, had regrets about their treatment choices. CONCLUSIONS: Patients with panplexus avulsion injuries are missing opportunities for reconstruction and often not considering the long-term outcomes of surgery. As more Americans gain health insurance coverage, it is very likely that the number of patients able to pursue reconstruction will increase. The authors recommend implementing clinical pathways to help patients meet critical points in care within the ideal timeframe and using a patient- and family-centered care approach combined with patient decision aids to foster shared decision making, increase access to information, and improve patient satisfaction with decisions. These measures could greatly benefit patients with BPI while reducing costs, improving efficiency, and generating better outcomes.

Coping with congenital hand differences.

BACKGROUND: Although functional outcomes following reconstruction for congenital hand differences are frequently described, much less is known regarding children's ability to cope with the psychosocial effects of these conditions. The authors qualitatively explored stress and coping mechanisms among children following reconstructive surgery for congenital hand differences. METHODS: Forty patients and their parents participated in semistructured interviews examining children's stress related to hand functioning and appearance, emotional responses to stress, and coping strategies. Interviews were audio-taped, transcribed, and analyzed thematically. A consensus taxonomy for classifying content evolved from comparisons of coding by two reviewers. Themes expressed by participants were studied for patterns of connection and grouped into broader categories. RESULTS: In this sample, 58 percent of children and 40 percent of parents reported stress related to congenital hand differences, attributed to functional deficits (61 percent), hand appearance (27 percent), social interactions (58 percent), and emotional reactions (46 percent). Among the 18 children who reported stress, 43 percent of parents were not aware of the presence of stress. Eight coping strategies emerged, including humor (12 percent), self-acceptance (21 percent), avoidance (27 percent), seeking external support (30 percent), concealment (30 percent), educating others (9 percent), support programs (21 percent), and religion (24 percent). CONCLUSIONS: Although children with congenital hand differences often experience emotional stress related to functional limitations and aesthetic deformities, many apply positive coping mechanisms that enhance self-esteem. Clinicians caring for children with congenital hand differences should inform families about potential sources of stress to direct resources toward strengthening coping strategies and support systems.

Patient satisfaction and self-reported outcomes after complete brachial plexus avulsion injury.

PURPOSE: Reconstructive surgery for complete brachial plexus avulsion injuries only partially restores function, and many patients are dissatisfied with results that surgeons consider good. Preoperative expectations have been shown to influence postoperative satisfaction but are poorly understood in patients with complete brachial plexus avulsion injuries. Qualitative methodology can elucidate patient beliefs and attitudes, which are difficult to quantify. The purpose of this study was to examine patient-reported outcomes, including satisfaction, and to understand the patient perspective. METHODS: We used qualitative interviews and questionnaires to assess patient-reported outcomes. Two members of the research team analyzed interview data using Grounded Theory methodology. Data from participants who had and did not have reconstructive surgery were compared. RESULTS: Twelve patients participated in this study. Of the 7 participants who had reconstructive surgery, 4 felt their expectations had been met and 5 were satisfied with their outcomes. Reconstruction did not produce statistically significant improvements in upper extremity function, pain, or work ability. All patients reported dissatisfaction with upper extremity ability, and 9 expressed hope for innovative treatments (e.g., stem cell therapy, nerve reinsertion) that could potentially provide better outcomes than existing procedures and enable return to work. CONCLUSIONS: Satisfaction with surgical outcomes after complete avulsion brachial plexus injury depends heavily on whether preoperative expectations are met, but patients are unfamiliar with nerve avulsion and do not always know what to expect. Low satisfaction with upper extremity ability and the lack of statistically significant differences produced by reconstruction suggest that current treatments may not be meeting patients' needs. Physicians must provide robust preoperative education to encourage realistic expectations and direct patients toward resources for pain management to facilitate comprehensive rehabilitation. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic III.

Mentorship: concepts and application to plastic surgery training programs.

BACKGROUND: Countless articles have demonstrated and emphasized the importance of mentoring in academic medicine. However, the upcoming role of mentors in the evolving medical field is poorly defined. As translational medicine, collaboration, and health care priorities change, so too must the goals and use of mentoring. The aims of this article are to demonstrate key aspects of effective mentoring in academic plastic surgery, show institutions how to cultivate mentoring relationships among their faculty and trainees, and provide direction for how to optimize the future use of mentoring to best prepare the next generation of plastic surgeons. METHODS: The authors reviewed the current literature regarding mentorship and the evolution of academic plastic surgery. RESULTS: Mentors not only facilitate their protégés' entrance into the field and future success, but can also attract medical students and residents to careers in research and reduce the racial and gender discrepancies in plastic surgery and academia. Ideally, faculty should undergo some form of training before they enter mentoring relationships. This will ensure that they are aware of their specific duties as mentors, are able to communicate with mentees, and can avoid potential pitfalls. CONCLUSIONS: Mentorship is a tool. If used correctly, it can help recruit and retain talented physician-scientists to plastic surgery to satisfy the growing demand. This will require institutions to actively support mentorship, provide opportunities and resources for training mentors, and enable faculty to allocate time to this vital pursuit.

Impact of medical tourism on cosmetic surgery in the United States.

SUMMARY: Developing countries have been attracting more international patients by building state-of-the-art facilities and offering sought-after healthcare services at a fraction of the cost of the US healthcare system. These price differentials matter most for elective procedures, including cosmetic surgeries, which are paid for out of pocket. It is unclear how this rise in medical tourism will affect the practice of plastic surgery, which encompasses a uniquely large number of elective procedures. By examining trends in the globalization of the cosmetic surgery market, we can better understand the current situation and what plastic surgeons in the United States can expect. In this article, we explore both domestic and foreign factors that affect surgical tourism and the current state of this industry. We also discuss how it may affect the practice of cosmetic surgery within the United States.

Graphs, tables, and figures in scientific publications: the good, the bad, and how not to be the latter.

Graphs, figures, and tables can save readers time and energy, aid their understanding of an article, and reduce the word count of the main text. However, many graphics submitted to and published in scientific journals fail to meet their potential and include mistakes that jeopardize their clarity. Many formats are available for presenting data, as well as a variety of techniques for enhancing interpretability. When the appropriate format is used to depict data, it conveys the greatest amount of information in the clearest fashion, complements the text, and deepens readers' understanding. The aims of this article are to draw attention to the necessity of well-constructed graphs, tables, and figures in scientific publications, and to show how to create them.

Mentorship in surgical training: a systematic review.

OBJECTIVE: This review systematically examines the literature regarding mentor-mentee relationships in surgery. BACKGROUND: The usefulness of mentorship in surgical training has been expressed in many articles. However, to date, there has been no systematic review on mentoring surgical trainees. This surgical environment is different from other areas of medicine and requires young surgeons to learn skills not readily available from textbooks. Instead, mentors are a valuable mode of transferring this knowledge to the next generation of surgeons. Thus, mentorship is a worthy area of research and attention. METHODS: We identified all articles discussing mentorship in surgery between January 1985 and August 2010 using PubMed and ISI Web of Knowledge. Predetermined exclusion and inclusion criteria were used to screen articles by title, abstract, and full text in sequence. We extracted the relevant data, and then analyzed the prevalence of major surgical mentoring themes in the literature. RESULTS: Of the 1,091 unique articles found during our original literature search, 38 were selected for review. The majority (68%) were commentary/editorial articles. The most discussed themes include the desirable qualities of a surgical mentor, the structure of mentor-mentee relationships, and advice for overcoming barriers to mentoring. Much less discussed themes include the desirable traits in a mentee and the appreciation of generational and cultural differences in mentorship. CONCLUSIONS: Several barriers to effective surgical mentoring were identified, such as time constraints and a lack of female mentors. By focusing on the positive traits found in this review, for example, developing formal programs to alleviate time constraints, these barriers can be overcome and effective mentor-mentee relationships can be built. Many articles draw attention to the dying art of mentorship in surgical training programs, and currently, the literature on mentorship in surgery is somewhat scarce. These concerns should serve as motivation to revive mentorship in surgery education and to expand the literature regarding underexplored themes and overcoming the current barriers. Although mentorship may not always take on a structured form, it should not be treated casually because proper mentorship is the foundation for training quality surgeons.