Leprosy reactions: Unraveling immunological mechanisms underlying tissue damage in leprosy patients.

Leprosy is a chronic granulomatous infectious and disabling disease caused by two mycobacteria, Mycobacterium leprae and Mycobacterium lepromatosis. Acute inflammatory responses, known as leprosy reactions, are significant contributors to disabilities. Three types of leprosy reactions have been identified based on excessive cytokine release (e.g. type 1) or the accumulation of immune complexes in tissues inducing multiorgan damage (e.g. types 2 and 3). The type of leprosy reaction has implications on treatment and management strategies, yet are not well understood by health workers caring for leprosy patients. We attempt to describe the immunologic mechanisms behind the different leprosy reactions and the rationale for tailoring clinical treatment and management to the particular type of leprosy reaction based on the underlying immunologic situation.

Zebras Among Us: Advocating for the 30 Million Americans Living with Rare Disease.

Nearly 30 million (about 1 in 10) Americans have a rare disease. On average, rare disease patients wait 6 years for an accurate and definitive diagnosis and see as many as 12 specialists along their diagnostic journey. In this brief article, we highlight some of what is being done across patient care, medical education, policy, and innovation in order to improve the diagnostic and treatment journeys of rare disease patients. We hope that members of the medical education community will appreciate this call to action and engage in the rare disease space.

Injury to the perineal branch of the pudendal nerve in men: Outcomes from surgical resection of the perineal branches.

OBJECTIVE: The objective is to report the outcome of an anterior surgical approach to treat neuroma of the perineal branch of the pudendal nerve (PBPN). PATIENTS AND METHODS: An IRB-approved prospective study enrolled 14 consecutive male patients from 2011 through 2015 who had symptoms of perineal/scrotal pain. Each patient had a successful, diagnostic, pudendal nerve block. The surgical procedure was resection of the PBPN and implantation of the nerve into the obturator internus muscle. Mean age at surgery was 50 ± 15 years. Median duration of pain symptoms was 5.5 years (range 1.2-42.9 years). Mechanisms of injury was exercise (6/14), prostatectomy (4/14), and falls (4/14). Outcomes were the Male Pudendal Pain Functional Questionnaire (MQ), and the Numeric Pain Rating Scale (NPRS). RESULTS: The mean postoperative follow-up was 26 ± 14 months. The MQ demonstrated that after surgery, patients overall had significantly less disability due to pudendal pain (P < .03). The NPRS revealed that pain significantly improved (P < .004). CONCLUSIONS: Resection of the PBPN and implantation of this nerve into the obturator internus muscle significantly relieved men's pelvic pain disability.

Breast reconstruction and risk of arm lymphedema development: A meta-analysis.

BACKGROUND: Lymphedema remains a significant complication following breast cancer surgery when there is axillary lymph node intervention. Previous systematic reviews have identified risk factors for breast cancer-related lymphedema, including increased BMI, number of lymph nodes dissected and radiotherapy. However, they have not examined the effect of breast reconstruction on lymphedema occurrence. In this systematic review and meta-analysis, we sought to evaluate the association between breast reconstruction (BR) and lymphedema. METHODS: We searched PubMed (1966-2016), Embase (1966-2016), Scopus (2004-2016) and Google Scholar (2004-2016) for studies involving breast reconstruction and upper-extremity lymphedema or breast cancer-related lymphedema. Our primary outcome was lymphedema occurrence. We performed a meta-analysis using random effects due to heterogeneity of the studies. RESULTS: Our search strategy identified 934 articles. After screening, 19 studies were included in our meta-analysis evaluating outcomes based on number of patients (7501) or number of breasts surgically treated (2063). Breast reconstruction was significantly associated with lower odds of lymphedema (p < 0.001) compared to mastectomy only or breast-conserving surgery. Lymphedema rates were not statistically significantly different between patients undergoing implant-based or autologous BR. CONCLUSIONS: Breast reconstruction is associated with lower rates of lymphedema compared to mastectomy only or breast conserving surgery patients. Although the study does not prove causation, we hypothesize that this association is likely due to multiple factors, including a self-selecting population and mechanisms through which BR may contribute to primary or secondary prevention of lymphedema. Further prospective studies are needed to clarify this beneficial relationship between breast reconstruction and reduced lymphedema risk.

Injury to Perineal Branch of Pudendal Nerve in Women: Outcome from Resection of the Perineal Branches.

Background This study describes outcomes from a new surgical approach to treat "anterior" pudendal nerve symptoms in women by resecting the perineal branches of the pudendal nerve (PBPN). Methods Sixteen consecutive female patients with pain in the labia, vestibule, and perineum, who had positive diagnostic pudendal nerve blocks from 2012 through 2015, are included. The PBPN were resected and implanted into the obturator internus muscle through a paralabial incision. The mean age at surgery was 49.5 years (standard deviation [SD] = 11.6 years) and the mean body mass index was 25.7 (SD = 5.8). Out of the 16 patients, mechanisms of injury were episiotomy in 5 (31%), athletic injury in 4 (25%), vulvar vestibulectomy in 5 (31%), and falls in 2 (13%). Of these 16 patients, 4 (25%) experienced urethral symptoms. Outcome measures included Female Sexual Function Index (FSFI), Vulvar Pain Functional Questionnaire (VQ), and Numeric Pain Rating Scale (NPRS). Results Fourteen patients reported their condition pre- and postoperatively. Mean postoperative follow-up was 15 months. The overall FSFI, and arousal, lubrication, orgasm, satisfaction, and pain domains significantly improved (p < 0.05). The VQ also significantly improved (p < 0.001) in 13 (93%) of 14 patients. The NPRS score decreased on average from 8 to 3 (p < 0.0001). All four patients with urethral symptoms were relieved of these symptoms. Conclusion Resection of the PBPN with implantation of the nerve into the obturator internus muscle significantly reduced pain and improved sexual function in women who sustained injury to the PBPN.

Treatment of Peripheral Neuropathy in Leprosy: The Case for Nerve Decompression.

Plastic surgery has a tradition of caring for patients with facial deformity and hand deformity related to leprosy. The approach, however, to the progressive deformity and disability related to chronic nerve compression is underappreciated in the world today. A cohort of patients with leprous neuropathy from an indigenous area of leprosy in Ecuador was evaluated for the presence of chronic peripheral nerve compression, and 12 patients were chosen for simultaneous upper and lower extremity, unilateral, nerve decompression at multiple levels along the course of each nerve. The results at 1 year of follow-up show that 6 patients improved into the excellent category and 4 patients improved into the good category for improved function. Based on the early results in this small cohort of patients with leprous neuropathy, an approach to peripheral nerve decompression, encompassing the concept of multiple crush at multiple levels of each nerve, seems to offer optimism to improve upper and lower extremity limb function. Long-term studies with quality-of-life outcomes would be welcome.

Protocol for a Randomised Controlled Trial Investigating Decompression for Leprous Neuropathy (The DELN Protocol).

Objectives: An expert group of peripheral nerve surgeons, reconstructive surgeons, and immunologists who have extensive experience with Hansen's Disease convened to discuss the status of nerve decompression as a treatment for leprous neuropathy. The expert group recommended an international, multi-center randomised controlled trial (RCT). Subsequently, a study protocol called Decompression for Leprous Neuropathy (DELN) was designed and further refined by multiple investigators worldwide. The DELN Protocol: The DELN RCT seeks to determine the long-term effect of nerve decompression on sensibility, motor function, neuropathic pain, disability, and quality of life. The RCT would enroll patients with clinically diagnosed leprous neuropathy and positive Tinel signs in the upper and lower extremities. Patients would then be randomized to receive nerve decompression or not. Outcomes of interest include sensory function, motor function, pain, disability, and quality of life. The development of ulcers or amputations after surgery and the influence of corticosteroid therapy are also important outcomes that DELN seeks to determine. Conclusions: The study Decompression for Leprous Neuropathy (DELN) is an international, multi-center RCT with the potential to produce high quality data to address whether nerve decompression for leprous neuropathy can conclusively improve patient outcomes. We invite discussion from all those involved in the peripheral nerve and leprosy communities.

Selecting a Test for Leprous Neuropathy Screening.

BACKGROUND: Worldwide, leprosy represents a significant cause of disability due to progressive neurological impairment. Screening for leprous neuropathy is performed with Semmes-Weinstein monofilament (SWM) or ballpoint pen testing (BPT), which results in underreporting of its prevalence. The Pressure-specified sensory device (PSSD; Sensory Management Services, LLC, Baltimore, MD) is a sensitive, noninvasive, portable, neurosensory instrument, which has not been field-tested for leprosy screening. Early identification of leprous neuropathy would permit early antibiotic treatment to prevent contagion and early microsurgical neurolysis. METHODS: A prospective, clinical diagnostic, cross-sectional study screened a consecutive sample of patients for leprous neuropathy in the leprosy-endemic province of Los Ríos, Ecuador. Patients meeting the World Health Organization criteria for leprosy and complaining of neuropathy symptoms were classified as leprous neuropathy patients. Patients without any signs of leprosy were used as normal controls. Bilateral ulnar nerve screening with the PSSD, SWM (0.07, 0.4, 2, 4, 10, and 300 g), and BPT was performed in all patients. Sensitivity and specificity were calculated and compared across tests. A total of 71 patients (142 nerves) were evaluated. RESULTS: Compared with the 10 g SWM and the BPT, the PSSD was found to have significantly higher sensitivity (78.3 vs. 0% with p < 0.001, for both) with comparable specificity (97.8 vs. 100% with p > 0.999, for both). Compared with the 0.07 g SWM (lightest filament in our series), the PSSD showed better sensitivity (78.3 vs. 65.2%, p = 0.514) and significantly higher specificity (97.8 vs. 51.1%, p < 0.001). CONCLUSIONS: The PSSD provides superior diagnostic accuracy for detecting leprous neuropathy as compared with SWM and BPT.

Global public health impact of recovered supplies from operating rooms: a critical analysis with national implications.

BACKGROUND: In modern operating rooms, clean and unused medical supplies are routinely discarded and can be effectively recovered and redistributed abroad to alleviate the environmental burden of donor hospitals and to generate substantial health benefits at resource-poor recipient institutions. METHODS: We established a recovery and donation program to collect clean and unused supplies for healthcare institutions in developing nations. We analyzed items donated over a 3-year period (September 2010-November 2013) by quantity and weight, and estimated the projected value of the program under potential nationwide participation. To capture the health benefits attributable to the donated supplies at recipient institutions, we partnered with two tertiary-care centers in Guayaquil, Ecuador and conducted a pilot study on the utility of the donated supplies at the recipient institutions (October 2013). We determined the disability-adjusted life years (DALY) averted for all patients undergoing procedures involving donated items and estimated the annual attributable DALY as well as the cost per DALY averted both by supply and by procedure. RESULTS: Approximately, 2 million lbs (907,185 kg) per year of medical supplies are recoverable from large non-rural US academic medical centers. Of these supplies, 19 common categories represent a potential for donation worth US $15 million per year, at a cost-utility of US $2.14 per DALY averted. CONCLUSIONS: Hospital operating rooms continue to represent a large source of recoverable surgical supplies that have demonstrable health benefits in the recipient communities. Cost-effective recovery and need-based donation programs can significantly alleviate the global burden of surgical diseases.