Set organic pollution as an impact category to achieve more comprehensive evaluation of life cycle assessment in wastewater-related issues

Abstract

For wastewater-related issues (WRI), life cycle assessment (LCA) is often used to evaluate environmental impacts and derive optimization strategies. To promote the application of LCA for WRI, it is critical to incorporate local impact of water pollutants. Organic pollution, a main type of water pollution, has not been given much consideration in current LCA systems. This paper investigates the necessity of setting a regionalized impact category to reflect the local impact of organic pollution. A case study is conducted concerning an upgraded wastewater treatment plant (WWTP) in China, which is assumed to meet different sewage control strategies. Chemical oxygen demand (COD) is selected to represent the organic pollution and treated as an individual impact category. CML 2002 is used to quantify the environmental impacts of different strategies. Results show that abnormal LCA results are generated with the traditional eutrophication impact category, and after the introduction of COD, more reasonable LCA results are obtained, making the entire comparison of different control strategies more meaningful and compelling. Moreover, BEES, Ecovalue 08, and Chinese factors are adopted here as different weighting methods. Different weighting results exhibited various trade-offs for the increasingly strict control strategies; the results of BEES and Ecovalue08 underlined the potential environmental burden, but the results of Chinese factors only emphasized the local environmental improvement. It is concluded that setting regionalized impact category for organic pollution can make LCA results more reasonable in wastewater treatment, especially in evaluating Chinese cases because of the serious water pollution caused by large quantities of COD emission.

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Accessory Auricles: Systematic Review of Definition, Associated Conditions, and Recommendations for Clinical Practice

Abstract Accessory auricles are rare congenital abnormalities embryologically derived from the first branchial arch. Presentation may be variable with locations grouped into anatomical zones based on the frequency of location found in the literature. This study reviewed the papers between 1981 and 2017. Findings included an association with syndromes including Goldenhar, VACTERL, Treacher–Collins, Townes–Brocks, and Wolf–Hirschhorn. Based on histological and embryological evidence, the term "accessory auricle" is best used as an umbrella term to define this difference. Finally, indications are provided for imaging and laboratory investigations to guide the physician treating patients with accessory auricles. Address correspondence and reprint requests to Mark S. Lloyd, MPhil, FRCS(Plast), Department of Plastic Surgery, Autologous Ear Reconstruction Service, Birmingham Children's and Women's Hospital NHS Trust, 4th Floor, Ladywood House, Steelhouse Lane, Birmingham B4 6NH, UK; E-mail: marklloyd1@nhs.net Received 11 July, 2017 Accepted 1 September, 2017 The authors report no conflicts of interest. © 2017 by Mutaz B. Habal, MD.

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A Rare Complication After Septoplasty: Visual Loss Due to Right Retinal Artery Spasm

Abstract Septoplasty is a commonly used procedure for correcting septal cartilage deformities. Hemorrhage, abscesses, scaling, adhesions, and scar tissue are often seen after the operation of the septoplasty, but temporary or permanent visual loss due to local anesthetic use has been reported very rarely in the literature. The authors also aimed to present a female patient with retinal artery spasm in the right eye after septoplasty in this article. A 27-year-old female patient was admitted to the authors' clinic with long-standing nasal obstruction and postnasal drip. There was no feature in her history and also no sign other than nasal septal deviation on physical examination. The patient was informed about the operation and the operation was planned. Emergency eye consultation was requested after the patient said that the right eye of the patient had never seen in the postoperative wake-up hall. Examination by an ophthalmologist; mild exotropia and total loss of vision in the right eye (including loss of light reflex) was detected. The light reaction in the affected eye was negative and indirect reaction was positive. After enlargement of the pupil, fundus examination revealed that the right posterior pole region (inside of the macula and vessel arches) was pale and no central retinal artery pulsation was observed. The patient was diagnosed with central retinal artery occlusion and emergency intervention was performed. The right eye massage, paracentesis, and hyperbaric oxygen therapy returned to the patient's visual function. Address correspondence and reprint requests to Dr Celal Günay, MD, Department of Otolaryngology, Head and Neck Surgery, Medipol University Hospital, Istanbul, Turkey; E-mail: gunaycelal@gmail.com Received 28 July, 2017 Accepted 27 August, 2017 The authors report no conflicts of interest. © 2017 by Mutaz B. Habal, MD.

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Inferiorly Pedicled Nasolabial Flap for Reconstruction of Anterior Maxilla Defects Class I and II

Objectives: The aim of this study was to present the use of inferiorly pedicled nasolabial flap (IPNF) in the reconstruction of anterior maxilla defects class I and II. Methods: Seven patients, 4 females and 3 males, aging from 38 to 75 (mean = 55.9) years old, from 2014 to 2017 reconstructed anterior part of maxilla with or without oronasal communication were included in this study. Five patients diagnosed with squamous cell carcinoma, 1 patient presented with epithelial-myoepithelial carcinoma, and 1 patient had ameloblastoma. The IPNFs were harvested in 7 patients. Follow-up data were collected for a period ranging from 9 to 26 months. Speech, mastication, and aesthetic outcomes were evaluated with a questionnaire in interview. Results: All patients healed without infection, flap loss, and flap failure. Five patients without prothetic treatment had little pronunciation problem (score: 1 to 3 points), while 2 patients with removable partial denture had excellent speech clarity (score: 1 to 2 points). All patients had no problem with mastication (score: 1 to 2 points). Aesthetic outcomes were acceptable in all patients (score: 1 to 3 points). Conclusion: The IPNF is a good option for reconstruction of anterior maxilla defects class I and II. Address correspondence and reprint requests to Zhongcheng Gong, PhD, DDS, Chairman and Professor of Oncological Department of Oral and Maxillofacial Surgery, The First Affiliated Hospital of Xinjiang Medical University, Stomatology School of Xinjiang Medical University, Stomatology Research Institute of Xinjiang Province, No. 137 South Liyushan Road, Wulumuqi, Xinjiang Uyghur Autonomous Region 830054, China; E-mail: gump0904@aliyun.com Received 12 May, 2017 Accepted 30 August, 2017 The authors report no conflicts of interest. © 2017 by Mutaz B. Habal, MD.

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Critical Appraisal in Plastic Surgery: Nullius in Verba

No abstract available

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Primary Closure of Oroantral Communication Using Pedicled Buccal Fat Pad Following Maxillary Cyst Enucleation

Abstract Odontogenic keratocyst (OKC) is a clinically aggressive developmental odontogenic cyst with a high recurrence rate as 62%. Oroantral communication (OAC) is a gateway through the maxillary sinus and the oral cavity, which, if not treated, will develop into oroantral fistula or chronical sinus diseases. Different methods for closure of OAC were described but only few of them have common usage. In recent years, the use of a pedicled buccal fat pad (BFP) in closure of large oroantral defects has been become popular. In our patient, the OAC resulted from the surgical removal of OKC was closed with BFP. Address correspondence and reprint requests to Yusuf Emes, DDS, PhD, Faculty of Dentistry, Department of Oral and Maxillofacial Surgery, Istanbul University 34093 Çapa, Istanbul, Turkey; E-mail: yusufemes@yahoo.com Received 27 July, 2017 Accepted 21 September, 2017 The authors report no conflicts of interest. © 2017 by Mutaz B. Habal, MD.

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A Novel Method to Calculate the Volume of Alveolar Cleft Defect Before Surgery

Background: This study aimed to evaluate a novel subtractive method for the precise calculation of alveolar cleft defect volume before surgery using the three-dimensional imaging software Mimics. Methods: Ten patients (3 female, 7 male, age range 8–12 years) with unilateral alveolar cleft were enrolled in the study. Preoperative helical computed tomography scans were performed for all patients. A new subtractive method was introduced to precisely calculate the alveolar cleft defect volume before surgery with the aid of Mimics. The DICOM data of every patient were processed using the subtractive method. Statistical analyses were performed using Pearson's correlation test and t test. Results: The mean volume of the alveolar cleft defect was 1811.97 ± 817.90 mm3. The mean time spent to calculate an alveolar cleft defect volume was 60.60 ± 11.67 minutes. The Pearson correlation test (r = 0.38, P = 0.279) indicated a poor correlation between alveolar cleft defect volume and calculation time. Conclusions: Preoperative knowledge of defect volume is crucial in alveolar cleft repair. Accurate estimation of graft volume in alveolar cleft patients can be performed using this new subtractive method. Compared with previous methods, this new subtractive method is time-saving and suitable for both unilateral and bilateral clefts. Surgeons can use this method to design a surgical plan for each patient before surgery within a short time, and with less cost. Address correspondence and reprint requests to Hai D. Li, MD, Department of Cleft Lip and Palate, Plastic Surgery Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Ba-da-chu, Beijing 100144, China; E-mail: 15910775061@yeah.net Received 2 June, 2017 Accepted 30 August, 2017 This study was supported by the Capital Clinical Feature Applied Research Fund of China (Z161100000516155). The study was approved by the ethics committee of the Plastic Surgery Hospital affiliated to Peking Union Medical College. The authors report no conflicts of interest. Supplemental digital contents are available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Web site (http://ift.tt/2iuFjMi). © 2017 by Mutaz B. Habal, MD.

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The Validity of Training Endoscopic Sinus and Skull Base Surgery Techniques on the Experimental Head Model

Objectives: The aim of this study was to better understand the usual learning curve in acquiring endonasal endoscopic sinus and skull base surgery (ESSBS) techniques during the novice training on the lamb's head model. Methods: Ten novices were asked to perform 10 bilateral dissections on the particular lamb's head each. The dissections were uniform, consisted of 10 well-defined steps, beginning from the simple removal of the inferior turbinate, and ending with more complicated procedures like cerebrospinal fluid leak repair, Draf 3 procedure for the frontal sinus and elevation of the nasal septal flap. The dissections have been supervised by experienced surgeons. A set of standard ESSBS instruments and 0° and 45° endoscopes have been used under the navigational system. The time required to complete each step has been measured in minutes. Results: In general and quite expectedly, time rates have been obviously lowering as the number of the dissections performed has been growing in each of the participants. Conclusion: Training of the endonasal ESSBS techniques on the lamb's head proved to be useful for novices in getting basic surgical skills in the field. Because of the high degree of anatomic similarity and high level of the anatomic dimensions congruency between the lamb's head and human head (sheep's head has bigger dimensions!) it proved to be an essential preparation for the human cadaveric dissection. The median values of the time rates having been needed to complete the particular of the 10 steps in the last novices' dissections could be accepted as an orientation, just suggesting that once the time needed to complete 1 of the 10 steps has been and achieved by the particular novice, this could be an approximate sign of the maturity for the exercises on human cadaver head. Address correspondence and reprint requests to Nuray Bayar Muluk, MD, Birlik Mahallesi, Zirvekent 2. Etap Sitesi, C-3 Blok, No 6-3/43, 06610 Çankaya, Ankara, Turkey; E-mail: nuray.bayar@yahoo.com; nurayb@hotmail.com. Received 23 August, 2017 Accepted 20 September, 2017 The authors report no conflicts of interest. Supplemental digital contents are available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Web site (http://ift.tt/2iuFjMi). © 2017 by Mutaz B. Habal, MD.

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Anti-Neutrophil Cytoplasmic Antibody-Negative Central Nervous System Granulomatosis With Polyangiitis and Its Clinical Characteristics

Abstract Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous vasculitis occasionally affecting central nervous system (CNS), and GPA patients with initial CNS symptoms are even rarer, whose diagnosis is further confused by an absence of positive antineutrophil cytoplasmic antibody. The authors described the characteristics of antineutrophil cytoplasmic antibody -negative GPA with CNS onset in a patient and discussed on its management, which may contribute to future diagnosis and treatment of patients with similar conditions. Address correspondence and reprint requests to Dr Yongming Qiu, MD, PhD, Department of Neurosurgery, Ren Ji Hospital, School of Medicine, Shanghai JiaoTong University, Shanghai 200127, China; E-mail: qiuzhoub@126.com; Dr Longtian Chen, MD, Department of Rheumatology, Longyan First Hospital Affiliated to Fujian Medical University, Fujian Province, China; E-mail: LT_chen@163.com Received 20 July, 2017 Accepted 24 August, 2017 The authors report no conflicts of interest. © 2017 by Mutaz B. Habal, MD.

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The role of liver resection in the management of intermediate and advanced stage hepatocellular carcinoma. A systematic review

Publication date: Available online 12 December 2017
Source:European Journal of Surgical Oncology
Author(s): G.K. Glantzounis, A. Paliouras, M.-C. Stylianidi, H. Milionis, P. Tzimas, D. Roukos, G. Pentheroudakis, E. Felekouras
BackgroundThe ideal management for patients with intermediate and advanced stage hepatocellular carcinoma (HCC) is controversial. The main purpose of this systematic review is to examine the role of liver resection in patients with intermediate stage HCC (multinodular HCCs) and in advanced stage HCC [mainly patients with portal vein tumor thrombosis (PVTT)].MethodsA systematic search of the literature was performed in Pud Med and the Cochrane Library from 01.01.2000 to 30.06.2016.ResultsTwenty-three articles with 2412 patients with multinodular HCCs were selected. Also, 29 studies with 3659 patients with HCCs with macrovascular invasion were selected. In patients with multinodular HCCs the median post-operative morbidity was 25 % and the 90-day mortality was 2.7 %. The median survival was 37 months and the 5-year survival 35 %. The 5-year survival was much better for patients with a number of HCCs ≤ 3 vs. HCCs >3 (49% vs. 23%).In patients with macrovascular invasion, who underwent hepatic resection, the median post-operative morbidity was 33% and the in-hospital mortality 2.7 %. The median survival was 15 months. The 3 and 5year survival was 33% and 20% respectively. Moreover a significant difference in survival was noted according to PVTT stage: 5- year survival for distal PVTT, PVTT of the main intrahepatic PV branch and PVTT extending to the main PV was 45%, 19% and 14.5 % respectively.ConclusionsLiver resection in patients with multinodular HCCs and HCCs with PVTT offers satisfactory long-term survival and should be considered in selected patients.



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Advances in organ preserving strategies in rectal cancer patients

Publication date: Available online 12 December 2017
Source:European Journal of Surgical Oncology
Author(s): Rutger CH. Stijns, Mike-Stephen R. Tromp, Niek Hugen, Johannes HW de Wilt
Treatment of rectal cancer patients has been subjected to change over the past thirty years. Total mesorectal excision is considered the cornerstone of rectal cancer treatment, but is also associated with significant morbidity resulting in an impaired quality of life. The addition of neoadjuvant chemoradiotherapy to surgery has shown to improve survival and local control and may lead to a partial or even complete response (CR). This raises questions regarding the necessity for subsequent radical surgery. After careful patient selection local excision and wait-and-see approaches are explored, aiming to improve quality of life without compromising oncological outcome. A multimodality diagnostic approach for optimal staging is crucial in determining the appropriate neoadjuvant treatment regimen. Adequate endoscopic restaging of rectal tumours after multimodality treatment will aid in selecting patients who are eligible for an organ preserving approach. The role and accuracy of imaging in the detection of the primary tumour, residual rectal cancer or local recurrence seems vital. Alternative neoadjuvant regimens are currently explored to increase the rate of clinical CRs, which may support organ preserving approaches. This review aims to generate insight into the advances in diagnostics and treatment modalities in all stages of rectal cancer and will highlight future studies that may support further implementation of organ preservation treatment in rectal cancer.



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Dimethyl Fumarate: A Review in Moderate to Severe Plaque Psoriasis

Abstract

Fumaric acid esters (FAEs) have been used in the treatment of psoriasis in some European countries for over 20 years, and are recommended in the European guidelines for the management of moderate to severe plaque psoriasis. Dimethyl fumarate (Skilarence^®; hereafter referred to as DMF) is an orally administered FAE indicated for the treatment of moderate to severe plaque psoriasis in adults in need of systemic medicinal therapy; unlike other available FAEs, it is not formulated in combination with monoethyl fumarate salts. EU approval was based on results of the phase III BRIDGE trial, and supported by previous publications of FAE preparations, including a combination of FAEs containing dimethyl fumarate and monoethyl fumarate salts (DMF/MEF; Fumaderm^®). In the BRIDGE trial, DMF was superior to placebo in terms of the proportion of patients achieving a ≥ 75% improvement from baseline in the Psoriasis Area and Severity Index (PASI 75) and a Physician Global Assessment score of 0 (clear) or 1 (almost clear) at week 16. DMF was also noninferior to DMF/MEF for PASI 75 at week 16. Patients receiving DMF also reported clinically meaningful improvements in body surface area involvement and health-related quality of life. The safety profile of DMF was similar to that of DMF/MEF, and no major or unexpected safety concerns were identified. The most common adverse events (flushing and gastrointestinal disorders) occurred mainly during the first few weeks of treatment. Currently available data indicate that DMF is an effective oral systemic treatment option for patients with moderate to severe plaque psoriasis.

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Adverse Events under Tacrolimus and Cyclosporine in the First 3 Years Post-Renal Transplantation in Children

Abstract

Background

Progress in immunosuppression has reduced acute rejection, graft loss and mortality after renal transplantation. Adverse drug reactions are well described in adults but few data are available in children. Our objectives were to analyse the adverse events reported in the first 3 years post-transplantation in children receiving tacrolimus or cyclosporine-based immunosuppression and compare them with the information of the Summary of Product Characteristics.

Methods

This retrospective study included all children who underwent a renal transplant at Hospital Robert Debré between 2002 and 2015. Initial immunosuppression was based on induction, calcineurin inhibitor, mycophenolate mofetil and corticosteroids. Adverse events were collected from medical records and coded using the Medical Dictionary for Regulatory Activities and the implications of tacrolimus and cyclosporine analysed. Statistical analyses were performed using SAS 9.4.

Results

One hundred and twenty-five children were included. During the observation period [2.7 years (0.6–4.3)], 105 patients received tacrolimus and 39 received cyclosporine. The incidence rate for gastrointestinal disorders was 0.128 and 0.056 by patient-years of exposure (p < 0.05), under tacrolimus and cyclosporine schedules. For neutropenia, it was 0.064 and 0.014 (p < 0.05). The frequencies of toxic nephropathy and gastrointestinal pain were higher than those in the Summary of Product Characteristics of tacrolimus (> 20%) and cyclosporine (> 10%). Cosmetic events for cyclosporine and neutropenia for tacrolimus were frequently observed (18 and 14.3%, respectively), although uncommon in the Summary of Product Characteristics.

Conclusions

The exposure-adjusted incidence rate of gastrointestinal disorders and neutropenia was higher in children under the tacrolimus schedule. Our findings contribute to the evaluation of the benefit-risk balance of immunosuppressive therapy following paediatric renal transplantation.

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Biopsia selectiva del ganglio centinela en el cáncer de mama del varón

Publication date: Available online 13 December 2017
Source:Revista Española de Medicina Nuclear e Imagen Molecular
Author(s): J.J. Martin-Marcuartu, R.M. Alvarez-Perez, J.M. Sousa Vaquero, J.M. Jimenez-Hoyuela García
ObjetivoEvaluar la reproductibilidad de la técnica del ganglio centinela (GC) en el cáncer de mama en el varón.Material y métodosAnalizamos retrospectivamente a 21 pacientes varones diagnosticados de cáncer de mama en nuestro hospital desde 2008 hasta 2016, con un seguimiento de al menos 18 meses. A 15 pacientes se les realizó la biopsia selectiva de ganglio centinela (BSGC) siguiendo los protocolos habituales con inyección de 74-111MBq de ^99mTc-nanocoloides peritumoral y adquisición de imágenes planares a las 2h. En 2 casos fue necesaria la realización de SPECT/TC para la localización del GC. Para su análisis se usaron técnicas anatomopatológicas de inmunohistoquímica o técnicas moleculares (OSNA). Seis pacientes no se sometieron a la BSGC por presentar ganglios patológicos o enfermedad a distancia en el momento del diagnóstico.ResultadosLa BSGC se practicó en 15 pacientes. El GC fue negativo en 6 de estos, siendo positivo en los 9 restantes. Hubo 3 pacientes con BSGC positiva, pero dado el bajo número de copias de ADN (OSNA) no se realizó linfadenectomía axilar. Sí se llevó a cabo vaciamiento axilar en los 6 pacientes restantes, con resultado de 4 vaciamientos positivos y 2 que no mostraban extensión de la enfermedad.ConclusionesSegún nuestra experiencia, la BSGC en los varones es una técnica reproducible, útil, segura y fiable, que evita la realización de linfadenectomías axilares innecesarias y previene la aparición de efectos no deseados.ObjectiveTo evaluate the reproducibility of the sentinel lymph node (SLN) technique in male breast cancer.Material and methodsWe retrospectively analysed 21 male patients diagnosed with breast cancer in our hospital from 2008 to 2016 with, at least, 18 months follow-up. Fifteen patients underwent selective sentinel lymph node biopsy (SLNB) following the usual protocols with peritumoral injection of 18.5-111MBq of ^99mTc-nanocoloides and acquisition of planar images 2hours after the injection. In 2 cases it was necessary to perform a SPECT/CT to locate the SLN. Immunohistochemistry and molecular techniques (OSNA) were used for their analysis. Six patients did not undergo SLNB because they had pathological nodes or distant disease at the time of diagnosis.ResultsSLNB was performed in 15 patients. The SLN was negative in 6 patients and positive in the remaining 9. Three patients with positive SLNB did not need axillary lymphadenectomy because of the low number of copies by molecular analysis OSNA. Axillary lymphadenectomy was performed in the remaining 6 patients with the result of 4 positive axillary lymphadenectomies and 2 that did not show further extension of the disease.ConclusionsAccording to our experience, SLNB in males is a reproducible, useful, safe and reliable technique which avoids unnecessary axillary lymphadenectomy and prevents the appearance of undesirable effects.



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Sequencing of bimaxillary surgery in the correction of vertical maxillary excess: retrospective study

The aim of this study was to evaluate the precision of bimaxillary surgery performed to correct vertical maxillary excess, when the procedure is sequenced with mandibular surgery first or maxillary surgery first. Thirty-two patients, divided into two groups, were included in this retrospective study. Group 1 comprised patients who received bimaxillary surgery following the classical sequence with repositioning of the maxilla first. Patients in group 2 received bimaxillary surgery, but the mandible was operated on first. (Source: International Journal of Oral and Maxillofacial Surgery)

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Comparative study of volumetric changes and trabecular microarchitecture in human maxillary sinus bone augmentation with bioactive glass and autogenous bone graft: a prospective and randomized assessment

The aim of this study was to compare the volumetric changes and the new bone microarchitecture in human maxillary sinuses augmented with bioactive glass (Biogran) alone, bioactive glass combined with autogenous bone graft (1:1), or autogenous bone graft alone. Twelve maxillary sinuses were grafted with bioactive glass (group 1), nine with bioactive glass mixed with autogenous bone graft 1:1 (group 2), and 12 with autogenous bone graft (group 3). Patients underwent cone beam computed tomography 15days after the procedure to determine the initial volume of the graft (T1) and again 6 months later (T2). (Source: International Journal of Oral and Maxillofacial Surgery)

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Signs of electrocochleography on endolymphatic sac decompression for patients with Ménière's Disease: our experience in 12 patients

Abstract

Endolymphatic hydrops (ELH) is a well-established histological marker of Ménière's Disease (MD). The diagnosis of MD still depends on clinical symptoms complemented with inner ear function tests. Thus far, definitive diagnosis remains difficult. It is necessary for clinicians to explore an objective examination to diagnose MD and to monitor patients' ELH.

This article is protected by copyright. All rights reserved.

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Brentuximab-vedotin maintenance following chemotherapy without irradiation for primary intracranial embryonal carcinoma in down syndrome

Abstract

Background

Germ cell tumors (GCT) are the most common central nervous system (CNS) tumors in individuals with Down syndrome. Patients with Down syndrome treated with CNS irradiation are at increased risk of developing cerebrovascular complications such as moyamoya disease. Embryonal carcinoma components are recognized to be more resistant to conventional chemotherapy and radiotherapy and confer a very poor prognosis. CD30 is a member of the tumor necrosis factor-receptor superfamily. CD30+ has a limited expression in normal cells but is the defining marker for embryonal carcinoma. Brentuximab-vedotin is a novel antibody-drug conjugate consisting of the chimeric anti-CD30 antibody conjugated to an anti-tubulin synthetic analog monomethyl auristatin E.

Methods

A retrospective review of the patient's records was conducted in September 2017.

Results

We report upon our management of a teenage girl with Down syndrome and a suprasellar pure embryonal carcinoma utilizing an intensive chemotherapy regimen followed by brentuximab-vedotin without irradiation. The patient received two cycles of carboplatin and etoposide interspersed with one cycle of cyclophosphamide and etoposide for induction followed by three cycles of marrow-ablative thiotepa and carboplatin rescued by autologous hematopoietic stem cell. Finally, She received six cycles of intravenous brentuximab-vedotin. The patient continues without evidence of recurrent tumor by MRI and tumor marker surveillance 24 months since diagnosis, with no adverse sequelae of her treatment.

Conclusions

Brentuximab-vedotin may provide a selective and safe alternative (or adjunct) to radiotherapy in the management of patients with CD30-positive CNS embryonal carcinoma, especially for those patients at high risk of developing irradiation-related complications.

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Trends in incidence and long-term outcomes of myelomeningocele in British Columbia

Abstract

Purpose

Myelomeningocele is typically a disabling condition that results in neurologic, orthopedic, and urologic morbidity. The aim of this study was to examine the trends over time in both incidence and outcomes of myelomeningocele (MMC) in British Columbia (BC).

Methods

A retrospective chart review was performed of all children with MMC followed in the British Columbia Children's Hospital (BCCH) Spinal Cord Clinic between 1971 and 2016. The incidence of new MMC cases and the long-term outcomes of MMC were compared between two 10-year cohorts. The first cohort comprised children born with MMC between 1971 and 1981, and the second cohort comprised children born with MMC between 1996 and 2006.

Results

A total of 309 children with MMC were followed in the BCCH Spinal Cord Clinic between 1971 and 2016. There were 101 and 46 children with MMC in the two-time cohorts, respectively. Between the earlier and later cohorts, there was a significant difference in the following: MMC incidence [2.5/10,000 births vs 1.1/10,000 births, respectively (p = 0.0002)], mortality [18 vs 0% (p = 0.0009)], and the proportion of cases repaired in under 48 h [56 vs 98% (p < 0.0001)]. For surviving children, the proportion of children attending special classes was significantly different between the earlier and later cohorts [16 vs 46%, respectively (p = 0.0002)], whereas all other outcome measures, including the proportion with hydrocephalus, kyphoscoliosis, Chiari II surgery, bowel and bladder continence, recreation participation, obesity, and ambulation, were not significantly different.

Conclusions

In BC, the incidence of new cases of MMC has decreased between 1971 and 2016, while the probability of survival for these patients has increased. Despite earlier and more universal post-natal repair, long-term outcomes have not improved significantly over time. Future research should focus on developing ways of reducing disability and improving quality of life for MMC patients and their families.

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Granulomatous Tumoral Melanosis Associated With Pembrolizumab Therapy: A Mimicker of Disease Progression in Metastatic Melanoma

Abstract: Immune checkpoint inhibitor therapy has revolutionized the treatment of advanced melanoma, with these agents significantly improving survival for patients with metastatic disease. With the increasing use of these agents, the number of adverse reactions secondary to their use has also increased. Sarcoidosis and sarcoid-like reactions are one such immune checkpoint inhibitor–related adverse event. We report a case of sarcoid-like granulomatous tumoral melanosis in a patient on the programmed cell death-1 (PD-1) receptor inhibitor pembrolizumab for metastatic melanoma. This is, to our knowledge, the first reported case of a sarcoidal form of tumoral melanosis in a patient on anti-PD-1 therapy. We postulate that this reflects tumor regression in response to pembrolizumab-induced immune activation, with concomitant therapy–triggered induction of a sarcoid-like reaction. These findings and the literature review presented herein should alert clinicians and pathologists to the possibility of regressed lesions with sarcoid-like features presenting as mimickers of disease progression in patients undergoing immunotherapy for advanced melanoma. Reprints: Karen A. Naert, MD, Department of Pathology and Laboratory Medicine, University of Calgary and Calgary Laboratory Services, Calgary, Alberta T2L 2K8, Canada (e-mail: Karen.naert@cls.ab.ca). The authors declare no conflicts of interest. Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Exophytic Mass Arising Within Hidradenitis Suppurativa

No abstract available

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Sebaceous Neoplasms With Rippled, Labyrinthine/Sinusoidal, Petaloid, and Carcinoid-Like Patterns: A Study of 57 Cases Validating Their Occurrence as a Morphological Spectrum and Showing No Significant Association With Muir–Torre Syndrome or DNA Mismatch Repair Protein Deficiency

Abstract: Sebaceous neoplasms with an organoid pattern (rippled, labyrinthine/sinusoidal, carcinoid-like, and petaloid) are rare. Previous studies suggested that the above patterns likely represent variations along a morphological continuum. The objectives of this study were to (1) validate this proposition by studying a large number of cases, (2) determine whether there are specific associations with clinical features, (3) establish their frequency, and (4) determine whether they have any association with Muir–Torre syndrome. Fifty-seven sebaceous neoplasms (54 sebaceomas and 3 sebaceous carcinomas) with organoid growth patterns were studied. These occurred in 36 men and 18 women (sex unknown in 3), with ages at diagnosis ranging from 22 to 89 years (mean, 63 years). All patients presented with a solitary nodule (mean size, 11 mm) on the head and neck area. Of the 57 tumors, 24 manifested a single growth pattern, 23 had a combination of 2 patterns, and 10 a combination of 3 patterns, indicating that these patterns are part of a morphological continuum of changes. The carcinoid-like pattern was the most frequent in the "monopatterned" neoplasms (13 cases), whereas the labyrinthine/sinusoidal pattern comprised most of the "polypatterned" lesions, in which various combinations occurred. Immunohistochemically, mismatch repair protein deficiency was detected in 3 of the 22 cases studied, whereas 5 of the 33 patients with available follow-up had an internal malignancy/premalignancy. In conclusion, sebaceous neoplasms with organoid growth patterns are predominantly sebaceomas having a predilection for the scalp, occurring as solitary lesions in elderly patients (male to female ratio of 2:1). Such patterns are expected to be found in a quarter of sebaceomas. In most cases, more than one of the organoid patterns is present. These lesions do not appear to be associated with internal malignancy or mismatch repair deficiency in most cases. However, confirmation of the absence of any significant association with Muir–Torre syndrome syndrome will require genetic studies. Reprints: Dmitry V. Kazakov, MD, PhD, Sikl's Department of Pathology, Charles University Medical Faculty Hospital, Alej Svobody 80, 304 60 Pilsen, Czech Republic (e-mail: kazakov@medima.cz). Supported in part by the Charles University Research Fund (project number SVV 2017-260 391). The authors declare no conflicts of interest. Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Langerhans Cell Histiocytosis Associated With Underlying Hematolymphoid Disorders in Adults: Report of 2 Cases and Review of the Literature

Abstract: Langerhans cell histiocytosis (LCH) is an uncommon disorder characterized by proliferation of abnormal LCs usually affecting children and adolescents. LCH in adults first presenting in the skin is rare. Although LCH and even LCH with a second malignancy may be more common in children, cutaneous LCH with a second hematologic malignancy has been more commonly identified in adults. The authors report 2 new cases of LCH in adult patients with underlying myelodysplasia and follicular lymphoma. The specimens were examined by routine microscopy and immunohistochemical stains for S100 protein and CD1a. Patients were elderly men with established diagnoses of follicular lymphoma and myelodysplasia, presented with follicular lesions and erythematous plaques involving intertriginous areas. Histologic examination revealed collections of mononuclear cells in upper dermis, which demonstrated strong positivity for S100 and CD1a, confirming their identity as LCs. BRAF analysis returned negative for detection of BRAF V600E mutation in both patients. The authors have recently encountered 2 cases of adult patients with skin-limited LCH predated by other lymphoproliferative disorders. The association between LCH and hematopoietic disorders may be explained by a common bone marrow precursor that is differentiating along different cell lines. Cutaneous LCH may be associated with underlying lymphoproliferative disorders and should be considered in the differential diagnosis of cutaneous eruptions in patients with hematopoietic disorders. Clinical follow-up evaluation of patients diagnosed with LCH for peripheral blood abnormalities and lymphadenopathy or "B symptoms" may be prudent in patients not already carrying a diagnosis of an underlying hematologic disorder. Reprints: Katrin Kiavash, MD, Wayne State University, Harper Professional Building, 4160 John R Street, Detroit, MI 48201 (e-mail: KKiavash@dmc.org). The authors declare no conflicts of interest. Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Epidermal Calcinosis: A New Pattern

No abstract available

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Clinico-Pathological Study of 4 Cases of Pseudoherpetic Grover Disease: The Same as Vesicular Grover Disease

Introduction: Grover disease (GD) was described with 4 histopathological patterns, but later wide histopathological studies found additional ones including vesicular GD. From 2014, 2 new patterns, GD with epidermolytic hyperkeratosis and pseudoherpetic GD, have been proposed. Objectives: The authors present 4 cases of pseudoherpetic GD and review the 6 previously published cases to better characterize this variant clinically and histopathologically. Results: Half of the patients were men. Mean age at diagnosis was 70, 25 years old. All the patients were immunosuppressed, and 75% of them were treated with chemotherapy. Lesions were asymptomatic or itchy papules sometimes crusted and intermingled with isolated vesicles or pustules mostly in the trunk. Skin biopsies showed intraepidermal vesicles filled with plasma (50%) with floating grouped acantholytic cells. No viral inclusions were found, and immunostaining for herpes virus and varicella zoster was negative. Discussion: Vesicular pattern in GD is described exclusively from a histopathological point of view, and it is defined as the presence of an intraepidermal vesicle filled with plasma and with dyskeratotic cells in the upper part of the vesicle. After reviewing all the described cases, pseudoherpetic GD presents the same histopathological features than vesicular GD, but authors paid attention to the presence of grouped acantholytic cells mimicking multinucleated cells of herpes infection. The authors consider that the so-called pseudoherpetic GD is a vesicular GD, where clinical suspicion of varicella raises this differential diagnosis and leads us to check viral nuclear cytopathic changes, and the authors favor the use of vesicular GD in the dermatopathologist report. Reprints: Mar Llamas-Velasco, MD, Department of Dermatology, Hospital Universitario de la Princesa, Fundación de Investigación de La Princesa, C/Diego de León 62, CP 28006, Madrid, Spain (e-mail: mar.llamasvelasco@gmail.com). T. M. Capusan, M. Herrero-Moyano, J. Fraga, and M. Llamas-Velasco performed the research and wrote the paper. T. M. Capusan, J. Fraga, and M. Llamas-Velasco analyzed the data. J. Fraga and T. M. Capusan contributed the cases. All the authors approved the final version of the manuscript. The authors declare no conflicts of interest. J. Fraga and M. Llamas-Velasco contribute equally to the work. Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.

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A Case of ALK+ Anaplastic Large-Cell Lymphoma With Aberrant Myeloperoxidase Expression and Initial Cutaneous Presentation

Abstract: Anaplastic large-cell lymphoma (ALCL) was first described in 1985 by Stein et al and is a clinically, morphologically, and immunophenotypically heterogeneous neoplasm characterized by ALK expression, rearrangement of the ALK gene, and most characteristically its occurrence in children. Clinically, cutaneous ALK+ ALCL can be divided into primary (cutaneous forms) and the much more common, secondary dissemination by a systemic lymphoma. Systemic ALK+ ALCL represents 10%–15% of childhood non-Hodgkin lymphoma and generally presents with advanced systemic disease. Here, we describe a case of a 9-year-old girl who presented with a solitary ulcerated nodule on the elbow that clinically resembled a pyogenic granuloma yet showed ALK, CD30, and myeloperoxidase expression. Fluorescent in situ hybridization with a break-apart probe for ALK revealed the presence of an ALK gene rearrangement. The initial workup showed no evidence of extracutaneous malignancy, and a diagnosis of primary cutaneous ALK+ ALCL was favored. Subsequent imaging studies revealed mediastinal lymphadenopathy, compatible with a systemic form of T-cell lymphoma, treated subsequently with chemotherapy. This report highlights the importance of an adequate systemic evaluation on the presentation of a cutaneous form of ALK+ ALCL. Reprints: Alejandro A. Gru, MD, Department of Pathology, E. Couric Clinical Cancer Center University of Virginia, PO Box 800214, 415 Lane Road, Hospital Expansion Bldg Room 3024, Charlottesville, VA 22908 (e-mail: AAG4B@hscmail.mcc.virginia.edu). A. A. Gru: Consultant Seattle Genetics and BMS. The remaining author declares no conflicts of interest. Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Cutaneous Involvement in a High Grade B-Cell Lymphoma With Rearrangement of MYC, BCL2, and BCL6 Transformed From Follicular Lymphoma

No abstract available

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Superoxide dismutase from Helicobacter pylori suppresses the production of pro-inflammatory cytokines during in vivo infection

Abstract

Background

Helicobacter pylori has undergone considerable adaptation to allow chronic persistence within the gastric environment. While H. pylori-associated diseases are driven by an excessive inflammation, severe gastritis is detrimental to colonization by this pathogen. Hence, H. pylori has developed strategies to minimize the severity of gastritis it triggers in its host. Superoxide dismutase (SOD) is well known for its role in protecting against oxidative attack; less recognized is its ability to inhibit immunity, shown for SOD from mammalian sources and those of some bacterial species. This study examined whether H. pylori SOD (HpSOD) has the ability to inhibit the host immune response to these bacteria.

Materials and Methods

The ability of recombinant HpSOD to modify the response to LPS was measured using mouse macrophages. A monoclonal antibody against HpSOD was generated and injected into H. pylori-infected mice.

Results

Addition of HpSOD to cultures of mouse macrophages significantly inhibited the pro-inflammatory cytokine response to LPS stimulation. A monoclonal antibody was generated that was specific for SOD from H. pylori. When injected into mice infected with H. pylori for 3 months, this antibody was readily detected in both sera and gastric tissues 5 days later. While treatment with anti-HpSOD had no effect on H. pylori colonization at this time point, it significantly increased the levels of a range of pro-inflammatory cytokines in the gastric tissues. This did not occur with antibodies against other antioxidant enzymes.

Conclusions

SOD from H. pylori can inhibit the production of pro-inflammatory cytokine during in vivo infection.

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Superoxide dismutase from Helicobacter pylori suppresses the production of pro-inflammatory cytokines during in vivo infection

Abstract

Background

Helicobacter pylori has undergone considerable adaptation to allow chronic persistence within the gastric environment. While H. pylori-associated diseases are driven by an excessive inflammation, severe gastritis is detrimental to colonization by this pathogen. Hence, H. pylori has developed strategies to minimize the severity of gastritis it triggers in its host. Superoxide dismutase (SOD) is well known for its role in protecting against oxidative attack; less recognized is its ability to inhibit immunity, shown for SOD from mammalian sources and those of some bacterial species. This study examined whether H. pylori SOD (HpSOD) has the ability to inhibit the host immune response to these bacteria.

Materials and Methods

The ability of recombinant HpSOD to modify the response to LPS was measured using mouse macrophages. A monoclonal antibody against HpSOD was generated and injected into H. pylori-infected mice.

Results

Addition of HpSOD to cultures of mouse macrophages significantly inhibited the pro-inflammatory cytokine response to LPS stimulation. A monoclonal antibody was generated that was specific for SOD from H. pylori. When injected into mice infected with H. pylori for 3 months, this antibody was readily detected in both sera and gastric tissues 5 days later. While treatment with anti-HpSOD had no effect on H. pylori colonization at this time point, it significantly increased the levels of a range of pro-inflammatory cytokines in the gastric tissues. This did not occur with antibodies against other antioxidant enzymes.

Conclusions

SOD from H. pylori can inhibit the production of pro-inflammatory cytokine during in vivo infection.

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