How do patients and physicians communicate about hereditary angioedema in the United States?

BACKGROUND: Hereditary angioedema (HAE) is a rare disease that manifests as recurrent and debilitating angioedema attacks, significantly impacting patients' quality of life. OBJECTIVE: To assess communication dynamics between patients with HAE and treating physicians and the impact this has on the treatment of HAE in the United States. METHODS: This observational study used an institutional review board-approved protocol to collect four sources of patient-physician communication data from the period between January 2015 and May 2017: in-office conversations between patients aged ≥18 years with HAE and physicians, follow-up dictations with physicians, telephone interviews with patients and physicians, and publicly available social media posts from patients. Participant language was qualitatively assessed and key communication elements and communication gaps identified. RESULTS: Twenty-five in-office conversations, 14 follow-up physician dictations, and 17 telephone interviews were conducted with a total of 29 unique patients, 4 caregivers, and 14 physicians. In-office conversations were generally physician-driven and focused primarily on symptom frequency, location, and severity; lexicon from both parties centered on "episodes" and "swelling." During visits, impact on quality of life was not routinely assessed by physicians nor discussed proactively by patients; however, during telephone interviews and online, patients frequently described the multifaceted burden of HAE. Patients highlighted the difficulties they experience by using repetition, emphasis, and metaphors; they also varied the descriptors used for attacks depending on the communication goal. Physicians used intensifiers to emphasize the necessity of rescue medication access, whereas prophylactic treatments were positioned as an option for frequent or laryngeal attacks. CONCLUSION: Vocabulary differences suggest that the full impact of HAE is not consistently communicated by patients to physicians during clinical visits, indicating the potential for misaligned understanding of disease burden. A patient-driven, rather than physician-driven approach to the discussions may elicit valuable information that could help to optimize treatment approaches.

Adenocarcinoma of the esophagus with signet ring cell features portends a poor prognosis.

BACKGROUND: Adenocarcinoma with signet ring cell (SRC) features has been reported to be a poor prognostic marker in gastric and colorectal carcinomas. Although uncommon in the esophagus, SRC histology, interestingly, has been correlated with improved survival. Our impression has been that the incidence of esophageal adenocarcinomas with SRC features is increasing and is associated with worse outcomes. We hypothesize that patients with SRC histology present with more advanced disease, respond less well to induction therapy, and have decreased survival after resection compared with patients with non-SRC adenocarcinoma. METHODS: The medical records of 151 consecutive patients who underwent resection for adenocarcinoma of the esophagus or gastroesophageal junction in a prospectively maintained database from 1998 to 2011 were reviewed. Outcomes of 23 patients (15%) with SRC histology (21 men, 2 women; average age, 66 years) were compared with 128 patients (85%) with non-SRC adenocarcinoma (116 men, 12 women; average age, 63 years). Overall survival, stage-specific survival, and response to induction therapy were evaluated. Cox regression multivariate analysis was used to identify independent predictors of 3-year survival. RESULTS: SRC and non-SRC patients were evenly matched for clinical and tumor characteristics. Downstaging achieved with induction therapy was 13.3% (2 of 15) in SRC histology patients vs 67.1% (53 of 79) in non-SRC patients (p ≤ 0.001). Patients with SRC histology who did not respond well to induction treatment demonstrated strong trends toward a worse 3-year survival than patients with non-SRC adenocarcinoma (p = 0.084). The overall 3-year survival was 65.6% in patients without SRC histology vs 34.8% in those with SRC (p = 0.006). Patients with pathologic stage II or III and SRC histology had a 3-year survival of 27.3% compared with 57.4% in patients with non-SRC adenocarcinoma (p = 0.01). Multivariate analysis showed SRC histology trended toward significance as an independent risk factor for poor survival (p = 0.060). CONCLUSIONS: Patients with adenocarcinoma of the esophagus or gastroesophageal junction and SRC histology respond less well to induction therapy and have decreased overall survival compared with patients with non-SRC histology.

Sustained supervised practice on a coronary anastomosis simulator increases medical student interest in surgery, unsupervised practice does not.

BACKGROUND: Given declining interest in cardiothoracic (CT) training programs during the last decade, increasing emphasis has been placed on engaging candidates early in their training. We examined the effect of supervised and unsupervised practice on medical students' interest in CT surgery. METHODS: Forty-five medical students participated in this study. Participants' interest level in surgery, CT surgery, and simulation were collected before and after a pretest session. Subsequently, participants were randomized to one of three groups: control (n = 15), unsupervised training on a low-fidelity task simulator (n = 15), or supervised training with a CT surgeon or fellow on the same simulator (n = 15). After 3 weeks, attitudes were reassessed at a posttest session. Interest levels were compared before and after the pretest using paired t tests, and the effects of training on interests were assessed with multiple linear regression analyses. RESULTS: After the pretest session, participants were significantly more interested in simulation (p = 0.001) but not in surgery or CT surgery. After training, compared with control group participants, supervised trainees demonstrated a significant increase in their interest level in pursuing a career in surgery (p = 0.028) and an increasing trend towards a career in CT surgery (p = 0.060), whereas unsupervised trainees did not. CONCLUSIONS: Supervised training on low-fidelity simulators enhances interest in a career in surgery. Practice that lacks supervision does not, possibly related to the complexity of the simulated task. Mentorship efforts may need to involve sustained interaction to provide medical students with enough exposure to appreciate a surgical career.

Comparison of cardiothoracic training curricula: integrated six-year versus traditional programs.

BACKGROUND: Traditionally, cardiothoracic residency programs are 2 or 3 years in length and require the completion of a general surgery residency. Six-year integrated programs (IP) that directly match fourth-year medical students have been recently developed. Our objective was to examine the curricula of traditional 2-year (T2) and 3-year (T3) programs and compare them to the curricula of IP. METHODS: We requested curricula from the directors of all IP, T2, and T3 programs participating in the 2011 to 2012 match. We compared the median number of months spent on a cardiothoracic (CT) rotation, an adult cardiac rotation, a thoracic rotation, and a congenital rotation, as well as time spent on "other" nonsurgical rotations. Traditional programs were categorized into 1 of 3 pathways: combined cardiothoracic (CCT), adult cardiac (AC), or general thoracic (GT). RESULTS: Integrated programs spend more time on general thoracic rotations when compared with CCT-T2, CCT-T3, AC-T2, and AC-T3 pathways (p = 0.009, p = 0.046, p = 0.001 and p = 0.028, respectively). The IP spend a similar amount of time on CT, adult cardiac, and congenital rotations when compared when 2- and 3-year CCT, AC, and GT pathways. Of note, IP spend significantly more time on "other" nonsurgical rotations than all other pathways (p < 0.001 to 0.008). CONCLUSIONS: Integrated programs should not be considered "cardiac pathways" as they spend a significant amount of time on thoracic rotations. Additional nonsurgical rotations provide an opportunity for residents in IP to develop unique skills not currently provided in traditional programs.

Epidemiology of esophageal cancer.

This article discusses the incidence, geographic differences, and risk factors for the 2 most common cancers of the esophagus: squamous cell and adenocarcinoma.

Utility of restaging endoscopic ultrasound after neoadjuvant therapy for esophageal cancer.

BACKGROUND: Currently, the most accurate staging test for patients with esophageal cancer is endoscopic ultrasound (EUS). At many institutions, patients who have completed neoadjuvant chemotherapy and radiotherapy for esophageal cancer undergo restaging EUS before proceeding to surgical resection. The benefit of this restaging procedure remains controversial. METHODS: We retrospectively studied consecutive patients who had pre-resection restaging EUS after receiving neoadjuvant treatment to assess accuracy of EUS restaging and determine whether it predicted survival. RESULTS: Final pathologic data were available for 73 patients who underwent restaging EUS (3 patients had missing T or N stage at one time point). Median time from restaging EUS to resection was 20 days. Restaging EUS accurately predicted pathologic T status in 26 of 72 patients (36%), N status in 44 of 71 (62%), and detected a complete pathologic response in 2 of 19 (10.5%). EUS inappropriately classified 10 patients as T0 N0. Agreement between EUS and pathologic staging was poor for T (κ=0.14) and N status (κ=0.24). Median time from resection to death or last follow-up was 20 months. Pathologic T and N status were each significant predictors of survival (p=0.049 and p=0.0004, respectively). There were nonsignificant trends toward better survival for lower EUS T (p=0.32) and N status (p=0.0946). CONCLUSIONS: Restaging by EUS before resection did not accurately predict pathologic stage in patients with esophageal cancer who received neoadjuvant treatment. As a result of this investigation, our institution no longer routinely performs restaging EUS.

Toxicity, response rates and survival outcomes of induction cisplatin and irinotecan followed by concurrent cisplatin, irinotecan and radiotherapy for locally advanced esophageal cancer.

OBJECTIVE: Cisplatin-based chemoradiotherapy is standard treatment for locally advanced esophageal and gastroesophageal cancers; however, the optimal chemotherapy regimen remains to be defined. METHODS: Retrospective single institution analysis of toxicities, response rates and survival outcomes in patients with cT3-4 or N1/M1a esophageal squamous cell or adenocarcinoma treated with induction cisplatin and irinotecan followed by concurrent cisplatin, irinotecan and radiotherapy. Secondary analysis for association of disease control and outcomes with demographic, tumor and treatment factors (including histology). RESULTS: Fifty-three patients were eligible for the present analysis. All patients underwent endoscopic ultrasonography and were either cT3-4 and/or cN1 disease. Fifty patients completed radiotherapy as planned (median dose 50.4 Gy, range 0-61.2), and 35 patients completed four cycles of chemotherapy as planned (range 1-4). Severe acute toxicities included Grade ≥ 3 neutropenia and esophagitis in 13 and 12 patients, respectively. There were no Grade 5 (fatal) toxicities noted. At mean survivor follow-up of 24.5 months (range 2.7-63), 17 patients were alive (8 without disease) and 36 deceased. Forty patients experienced disease recurrence, with initial loco-regional, distant or both failures in 28, 9 and 3 patients, respectively. Estimated 2-year overall survival and freedom from failure were 42 and 9%, respectively, without significant difference by histology. CONCLUSIONS: Cisplatin/irinotecan chemoradiotherapy is tolerable, demonstrating similar efficacy for squamous cell and adenocarcinoma esophageal cancers.

Sternoclavicular joint infection necessitating through skin and lung parenchyma.

Infections of the sternoclavicular joint associated with extensive local soft tissue involvement or abscess formation often require surgical debridement and drainage. This condition typically presents with joint pain, local erythema, and swelling. This case demonstrates the simultaneous extension of a sternoclavicular joint infection into the left upper pulmonary lobe and through the overlying skin.

Differential matrix metalloproteinase levels in adenocarcinoma and squamous cell carcinoma of the lung.

OBJECTIVE: The matrix metalloproteinases (MMPs) have been implicated in the aggressive course of non-small cell lung cancer (NSCLC). However, there are a large number of MMP subtypes with diverse proteolytic substrates and different induction pathways. This study tested the hypothesis that a differential MMP profile would exist between NSCLC and normal lung and that MMP patterns would differ between NSCLC histologic types. METHODS: NSCLC samples and remote normal samples were obtained from patients with stage I or II NSCLC with either squamous cell (n = 22) or adenocarcinoma (n = 19) histologic characteristics. Absolute concentrations for each of the MMP subclasses were determined by a calibrated and validated multiplex suspension array: collagenases (MMP-1, -8, and -13), gelatinases (MMP-2 and -9), lysins (MMP-2 and -7), and elastase (MMP-12). RESULTS: Overall, MMP levels were significantly increased in NSCLC compared with normal. For example, MMP-1 and MMP-7 increased by approximately 10-fold in NSCLC (P < .05). Moreover, a different MMP portfolio was observed between NSCLC histologic types. For example MMP-1, -8, -9, and -12 increased by more than 4-fold in squamous cell versus adenocarcinoma (P < .05). In those patients who had recurrence within 3 years of resection, 3-fold higher levels of MMP-8 and -9 were observed (P < .05). CONCLUSION: Increased levels of a number of MMP types occur with NSCLC, but the MMP profile was distinctly different between histologic types and in those patients with recurrence. These different MMP profiles may be important in the mechanistic basis for the natural history of different NSCLC types, as well as identifying potential prognostic and therapeutic targets.

Factors dominating choice of surgical specialty.

BACKGROUND: There has been much focus on factors influencing medical students' career choice, prompted by such concerns as a sufficient future surgical workforce, declining applicant pool, changing gender composition, and a cultural shift in values and priorities. Once in a surgical residency, there are little data on factors influencing general surgery (GS) residents' final specialty choice. STUDY DESIGN: A survey instrument was developed and content validated in conjunction with the Association of American Medical Colleges Center for Workforce Studies. The final instrument was distributed electronically between March 24 and May 2, 2008, through 251 GS program directors to all ACGME-accredited GS residents (n = 7,508). RESULTS: Response rate was 29% (2,153 residents; 89% programs). Half of GS residents remained undecided about specialty choice through the 2(nd) year, declining to 2% by year 5. Of the two-thirds who decided on a specialty, 16.5% chose to remain in GS, 14.6% chose plastics, 9.3% cardiothoracic, and 8.5% vascular. The specialty choice factors most likely to be very important were type of procedures and techniques, exposure to positive role model, and ability to balance work and personal life. Relative importance of factors in specialty choice varied by gender and chosen specialty. Mentors play a key role in specialty choice (66% decided had mentors versus 47% undecided). Work schedule was the most frequently selected shortcoming in every specialty except plastics. Cardiothoracic surgery followed by GS had the highest shortcomings. CONCLUSIONS: The majority of GS residents plan to subspecialize. Three factors dominate specialty choice. Faculty need to understand their impact potential to modify or change perceptions of their specialty.

Invasive thymoma with endobronchial metastasis.

Endobronchial thymoma is an exceedingly rare presentation. We report a 70-year-old woman undergoing bronchoscopy for dyspnea and a computed tomographic scan believed to be most consistent with a right lower lobe neoplasm. Thoracoscopy was ultimately required for diagnosis.

Cisplatin/Irinotecan versus carboplatin/paclitaxel as definitive chemoradiotherapy for locoregionally advanced esophageal cancer.

OBJECTIVE: To compare toxicities, disease control, and survival outcomes for patients treated with either cisplatin/irinotecan versus carboplatin/paclitaxel concurrent chemoradiotherapy for locally advanced esophageal cancer. METHODS: Single-institution retrospective comparison between treatment groups: the cisplatin/irinotecan group was treated with 2 cycles of induction chemotherapy followed by concurrent chemoradiotherapy, whereas the carboplatin/paclitaxel group began with chemoradiotherapy followed by 2 additional cycles of chemotherapy. Acute toxicities, response rates, disease control, survival outcomes, and patterns of failure were compared between the groups. RESULTS: Between January 2000 and December 2007, 57 patients were identified for inclusion in the present study (38 cisplatin/irinotecan and 19 carboplatin/paclitaxel). Groups were well-balanced by clinical-, pathologic-, staging-, and treatment-related factors. Thirty-five patients (92%) in the cisplatin/irinotecan group and 18 patients (95%) in the carboplatin/paclitaxel group completed the concurrent phase of chemoradiotherapy. There were no significant differences in hematologic or nonhematologic toxicities between the groups. At a median survivor follow-up of 37.6 months (range: 7.3-59.3 months) for the entire population, 22 patients were alive (16 without evidence of disease). The 3-year overall survival estimates was 19.7% for the cisplatin/irinotecan group versus 56.1% for the carboplatin/paclitaxel group (P = 0.022). Estimated 3-year cancer-specific survivals were 24.6% for the cisplatin/irinotecan group versus 59.3% for the carboplatin/paclitaxel group (P = 0.033). CONCLUSION: Concurrent chemoradiotherapy with carboplatin/paclitaxel is well-tolerated and provided superior overall and disease-specific survival compared with cisplatin/irinotecan chemoradiotherapy in the present study population. Further investigation is warranted.

Optimum lymphadenectomy for esophageal cancer.

OBJECTIVE: Using Worldwide Esophageal Cancer Collaboration data, we sought to (1) characterize the relationship between survival and extent of lymphadenectomy, and (2) from this, define optimum lymphadenectomy. SUMMARY BACKGROUND DATA: What constitutes optimum lymphadenectomy to maximize survival is controversial because of variable goals, analytic methodology, and generalizability of the underpinning data. METHODS: A total of 4627 patients who had esophagectomy alone for esophageal cancer were identified from the Worldwide Esophageal Cancer Collaboration database. Patient-specific risk-adjusted survival was estimated using random survival forests. Risk-adjusted 5-year survival was averaged for each number of lymph nodes resected and its relation to cancer characteristics explored. Optimum number of nodes that should be resected to maximize 5-year survival was determined by random forest multivariable regression. RESULTS: For pN0M0 moderately and poorly differentiated cancers, and all node-positive (pN+) cancers, 5-year survival improved with increasing extent of lymphadenectomy. In pN0M0 cancers, no optimum lymphadenectomy was defined for pTis; optimum lymphadenectomy was 10 to 12 nodes for pT1, 15 to 22 for pT2, and 31 to 42 for pT3/T4, depending on histopathologic cell type. In pN+M0 cancers and 1 to 6 nodes positive, optimum lymphadenectomy was 10 for pT1, 15 for pT2, and 29 to 50 for pT3/T4. CONCLUSIONS: Greater extent of lymphadenectomy was associated with increased survival for all patients with esophageal cancer except at the extremes (TisN0M0 and >or=7 regional lymph nodes positive for cancer) and well-differentiated pN0M0 cancer. Maximum 5-year survival is modulated by T classification: resecting 10 nodes for pT1, 20 for pT2, and >or=30 for pT3/T4 is recommended.

Factors affecting interest in cardiothoracic surgery: survey of North American general surgery residents.

BACKGROUND: Applications to cardiothoracic surgery (CTS) training programs have declined precipitously. The viewpoints of potential applicants, general surgery residents, have not yet been assessed. Their perceptions are crucial to understanding the cause and formulating appropriate changes in our educational system. METHODS: An initial survey instrument was content-validated, and the final instrument was distributed electronically between March 24 and May 2, 2008 through 251 general surgery program directors to all Accreditation Council for Graduate Medical Education-accredited general surgery residents (7,508). RESULTS: The response rate was 29% (2153 residents; 89% programs). Respondent's demographics matched existing data; 6% were committed to CTS, and 26% reported prior or current interest in CTS. Interest waned after postgraduate year 3. Interest correlated with CTS rotation duration. Of the respondents committed to CTS, 76% had mentors (71% were cardiothoracic surgeons). CTS had the most shortcomings among 9 subspecialties. Job security and availability accounted for 46% of reported shortcomings (3 to 14 times higher than other subspecialties). Work schedule accounted for 25%. Length of training was not a very important factor, although it was identified as an option to increase interest in CTS. Residents who were undecided or uninterested in CTS were twice as likely to cite the ability to balance work and personal life as important than residents who chose CTS. CONCLUSIONS: The dominant concern documented in the survey is job security and availability. The importance of mentorship and exposure to CTS faculty in promoting interest was also evident. Decision makers should consider these findings when planning changes in education and the specialty.

Factors affecting interest in cardiothoracic surgery: Survey of North American general surgery residents.

BACKGROUND: Applications to cardiothoracic surgery (CTS) training programs have declined precipitously. The viewpoints of potential applicants, general surgery residents, have not yet been assessed. Their perceptions are crucial to understanding the cause and formulating appropriate changes in our educational system. METHODS: An initial survey instrument was content-validated, and the final instrument was distributed electronically between March 24 and May 2, 2008 through 251 general surgery program directors to all Accreditation Council for Graduate Medical Education-accredited general surgery residents (7508). RESULTS: The response rate was 29% (2153 residents; 89% programs). Respondent's demographics matched existing data; 6% were committed to CTS, and 26% reported prior or current interest in CTS. Interest waned after postgraduate year 3. Interest correlated with CTS rotation duration. Of the respondents committed to CTS, 76% had mentors (71% were cardiothoracic surgeons). CTS had the most shortcomings among 9 subspecialties. Job security and availability accounted for 46% of reported shortcomings (3 to 14 times higher than other subspecialties). Work schedule accounted for 25%. Length of training was not a very important factor, although it was identified as an option to increase interest in CTS. Residents who were undecided or uninterested in CTS were twice as likely to cite the ability to balance work and personal life as important than residents who chose CTS. CONCLUSIONS: The dominant concern documented in the survey is job security and availability. The importance of mentorship and exposure to CTS faculty in promoting interest was also evident. Decision makers should consider these findings when planning changes in education and the specialty.

Diffuse esophageal polyposis: an uncommon occurrence.

The presence of multiple esophageal polyps on endoscopy is a rare entity. Most of the literature cited on this phenomenon is based on case reports and small series. A large proportion of the literature describes one or two polyps, with the majority of polyps occurring in the area of the gastroesophageal junction. We present a case of a 66-year-old woman with a history of gastroesophageal reflux disease that was found to have extensive esophageal polyposis of the mid-esophagus on upper endoscopy. The patient underwent a transhiatal esophagectomy. Final pathology was consistent with extensive polypoid dysplasia in the presence of Barrett's esophagus.