Cardiac tamponade in a patient with ankylosing spondylitis: a notable response to TNF inhibitors

Ankylosing spondylitis (AS) is a rheumatological disorder of the spine, and like many other rheumatological diseases, it can manifest as a systemic inflammation. We present a rare case of cardiac manifestations of AS in a 25-year-old man with recurrent chest pain and pericardial effusions. He initially presented with pleuritic chest pain, was diagnosed with cardiac tamponade and required emergent pericardiocentesis. The patient returned again with chest pain and was found to have reaccumulation of pericardial effusion. The cardiac symptoms were finally resolved when he was diagnosed and treated for AS.

https://ift.tt/2Mm349Q

Acute lymphoblastic leukaemia with osteolytic bone lesions: diagnostic dilemma

A previously healthy 37-year-old man presented with a 10-month history of intractable back pain. On examination, there was tenderness to palpation along lower thoracic and lumbar spine. Complete blood count showed mild anaemia but was otherwise unremarkable. Imaging studies revealed compression deformities with multiple osteolytic lesions involving multiple levels of the thoracic and lumbar spine. Bone marrow aspiration and biopsy were performed and demonstrated blast cells involving 80% of the bone marrow cellularity. Findings on flow cytometry were consistent with B-lymphoblastic leukaemia. He was subsequently started on hyper-CVAD (fractionated cyclophosphamide, vincristine, Adriamycin and dexamethasone) induction chemotherapy.

https://ift.tt/2MDaeTY

Isolated limb infusion as a treatment option for malignant granular cell tumour

A 54-year-old man presented with a painful lesion on the right posterior calf with MRI identifying a 5 cm lesion in the medial head of the gastrocnemius. He underwent wide local excision of the tumour, and the final pathology was consistent with atypical granular cell tumour. Three years later, he developed a recurrent right popliteal mass. Complete staging workup also identified multiple lung nodules and a caecal polyp that were consistent with metastatic granular cell tumour. He was started on pazopanib and deemed a poor candidate for palliative resection due to encasement of the popliteal vessels. The patient refused above-the-knee amputation (AKA) at that point and was evaluated for isolated limb infusion as an alternative. He received three cycles of isolated limb infusion within a 2-year period and achieved good response from the first two cycles. He underwent AKA 4 years after his diagnosis of malignant granular cell tumours and is currently doing well.

https://ift.tt/2OZTPdX

Paraneoplastic granulomatous dermatitis in a patient with Hodgkins disease: a diagnostic pitfall

The association of malignant lymphomas with non-necrotic epithelioid granulomas has been reported rarely since 1977. Hodgkin's disease-associated widespread cutaneous granuloma annulare (GA) has been reported in only eight patients. We report the second case of subcutaneous GA associated with Hodgkin's disease. A 73-year-old man with Epstein-Barr virus-associated Hodgkin's lymphoma and paraneoplastic subcutaneous GA, presented 3 months after the diagnosis of malignancy. Examination revealed a large, broad erythematous, indurated, subcutaneous plaque spanning the majority of the left lower back and flank with no associated symptoms. Initial biopsy was suggestive of morphea. Prompted by positron emission tomography (PET) findings of increased fluorodeoxyglucose (FDG) uptake, a second, deeper biopsy was performed, revealing subcutaneous palisaded granulomatous dermatitis. After complete workup, the diagnosis most strongly suggested subcutaneous GA. This case highlights the importance of deep incisional biopsies, the fluorodeoxyglucose - positron emission tomography (FDG-PET) findings in GA and the rare association of GA with Hodgkin's disease which may signal the presence of malignancy.

https://ift.tt/2MDa7Yy

Levosulpiride-induced neuroleptic malignant syndrome in rheumatoid arthritis

A 53-year-old woman, known case of diabetes mellitus and rheumatoid arthritis, presented with a 4-day history of hyperthermia, rigidity, tremor and altered sensorium. She developed these symptoms after having been administered parenteral levosulpiride to control vomiting due to secondary adrenal insufficiency. We managed her as a case of life-threatening neuroleptic malignant syndrome (NMS) requiring mechanical ventilation, bromocriptine and other supportive care. She subsequently recovered and was discharged in a stable condition. To the best of our knowledge, this is the first documented case report describing levosulpiride-induced NMS.

https://ift.tt/2Mlua0X

Massively dilated common bile duct: an unusual aetiology for nutcracker phenomenon

Description 

A 45-year-old man presented with recurrent haematuria. He also had history of upper abdominal pain for 1 month. On examination he had left varicocele. On ultrasonogram (USG), he had a grossly dilated common bile duct (CBD). No abnormality of the urinary tract was seen on USG. His coagulation profile, kidney and liver function tests were normal. Contrast-enhanced CT (CECT) scan of the abdomen was done that was suggestive of a massively dilated CBD with maximum diameter of 4.2 cm (figure 1), which was compressing the left renal vein (LRV) against the abdominal aorta (figure 2). There was no dilation of intrahepatic biliary radicals so a provisional diagnosis of type 1 choledochal cyst was made, and he was referred to the department of gastrointestinal surgery where he underwent cholecystectomy with resection of dilated CBD with Roux-en-Y hepatico-jejunostomy. Intraoperatively, there were adhesions around the cyst wall that were easily...

https://ift.tt/2MG81XY

Multidrug-resistant tuberculosis in rural China: lack of public awareness, unaffordable costs and poor clinical management

China has the second highest global incidence and prevalence of multidrug-resistant tuberculosis (MDR-TB). We describe here the life experience of a rural Chinese farmer with complicated and aggressive TB. It is unclear if this patient contracted MDR-TB initially or developed MDR-TB during treatment because the initial laboratory results are dubious. The lack of public awareness of TB in rural China fuelled by a belief in toxicity of TB treatment, as mentioned by his brother, caused the patient to stop his TB treatment repeatedly long before completion. The cost of MDR-TB treatment in China is unaffordable for most Chinese, especially those in rural areas. He paid about ¥300 000 (almost US$50 000) for his TB treatment. He was discharged early twice for 'financial difficulties'. This case highlights excessive costs, lack of public awareness, poor patient education, inadequate follow-up, lack of coordination between clinical services and the importance of treatment adherence.

https://ift.tt/2OZprk9

Cortactin as a potential predictor of second esophageal neoplasia in hypopharyngeal carcinoma

Publication date: Available online 11 August 2018

Source: Auris Nasus Larynx

Author(s): Ching-Feng Lien, Tzer-Zen Hwang, Tsun-Mei Lin, Kai-Wen Liu, Bor-Shyh Lin, Chih-Chun Wang, Chuan-Chien Yang, Shyh-An Yeh

Abstract

Objective

Hypopharyngeal carcinoma has a very poor prognosis. The high incidence of second esophageal neoplasia is one of the major causes. To establish an efficient follow-up scheme for increasing the diagnostic yield and reducing the adverse impact of second esophageal neoplasia on survival, the purpose of this study was to explore a biomarker to predict second esophageal neoplasia.

Methods

In this retrospective cohort study, consecutive tissue specimens from those patients who underwent tumor resection between September 2007 and October 2015 were collected. Gene amplification was performed by real-time PCR. The expression of cortactin was evaluated by immunohistochemistry. The predictive risk factors of developing second esophageal neoplasia and prognostic factors related to survival were analyzed.

Results

A total of 187 patients were included with a mean follow-up of 48 months (12–118 months). Second esophageal tumors were found in 53 (28.3%), including 41 (21.9%) esophageal squamous cell carcinoma and 12 severe dysplasia. The results of multivariate analyses revealed that age (OR 2.81, 95% CI 1.16–6.78), cortactin overexpression (OR 2.49, 95% CI 1.17–5.33), and stage IV versus I (OR 6.49, 95% CI 1.68–25.18) were independent predictors of second esophageal neoplasia, and second esophageal neoplasia (HR 1.78, 95% CI 1.05–3.01) was an independent predictor of overall survival.

Conclusion

This is the first report to identify a potential biomarker for predicting second esophageal neoplasia in patients with hypopharyngeal carcinoma. In those patients with cortactin overexpression and younger age (≤60 years old), close surveillance for second esophageal neoplasia is required. In addition, the real effect of cortactin overexpression on development of primary esophageal carcinoma is required to be validated in a large cohort study.

https://ift.tt/2MkbzSL

Reconstruction of medial orbital wall using a retrocaruncular approach

The purpose of the study was to analyze and describe the retrocaruncular approach to access medial orbital wall.A retrospective analysis was performed in patients referred for the treatment of orbital fractures between January 1st 2011 and July 31st 2017. The study included patients over 18 years old with isolated fractures of the medial orbital wall or combined with the orbital floor who underwent a transconjunctival approach with retrocaruncular extension and lateral canthotomy, and with a minimum follow-up of 6 months.

https://ift.tt/2MEagLr

Healing Pattern of Intentional Pterygoid Plate Fracture after Posterior Movement of Maxilla through Le Fort I Osteotomy

To evaluate maxillary stability following Le Fort I osteotomy using postero-superior movement after pterygoid plate fracture. Additionally the authors sought to analyze the postoperative changes at the intentional pterygoid plate fracture site.

https://ift.tt/2Mk7gqE

Change of site of intra-articular injection of hypertonic dextrose resulted in different effects of treatment

Publication date: Available online 11 August 2018

Source: British Journal of Oral and Maxillofacial Surgery

Author(s): A.A. Fouda

Abstract

Most minimally invasive treatments for dysfunction of the temporomandibular joint (TMJ) are empirical, and aimed at the painful trigger points with the purpose of preventing muscular spasm and restoring normal function. In this prospective study I investigated whether the choice of site of injection of hypertonic dextrose affected the benefits of treatment of internal derangement and pain. I studied 72 patients with pain and clicking as a result of dysfunction of the TMJ. Patients were divided into four groups with four separate sites for intra-articular injection. Dextrose was injected into the superior joint space, inferior joint space, retrodiscal tissue, and anterior capsule injection. Results showed that the retrodiscal site was the most effective for reducing clicking and subsequently improving derangement, while the inferior joint space was the best site for the relief of pain, and the extracapsular site should be used in cases of hypermobility. In conclusion, the injection site should be selected according to the symptoms being treated, and could be used as an adjunct to other sites to improve outcome.

https://ift.tt/2vEOO2m

Complications and outcome after free flap surgery for cancer of the head and neck

Publication date: Available online 11 August 2018

Source: British Journal of Oral and Maxillofacial Surgery

Author(s): S. Lahtinen, P. Koivunen, T. Ala-Kokko, O. Kaarela, P. Ohtonen, P. Laurila, J.H. Liisanantti

Abstract

We retrospectively studied 136 patients who had free flap reconstruction for cancer of the head and neck at a single centre (2008–2015) to evaluate complications, assess factors associated with them, and analyse their impact on outcome. Preoperative and perioperative data, and surgical and medical complications were recorded, and the impact of the complications on duration of hospital stay and survival were assessed. A total of 86 (63%) patients had complications. Compared with those who did not, they had a higher rate of alcohol abuse (21/86, compared with 5/50, p = 0.039), longer operations (median (IQR) 565 (458–653 compared with 479 (418–556) minutes, p < 0.001), and greater intraoperative loss of blood (725 (400–1150) compared with 525 (300–800) ml, p = 0.042). Complications were more common in patients who had fibular flaps and T4 disease (22/86 compared with 4/50, p = 0.010; 47/80 compared with 16/47, p = 0.015, respectively). Those who had complications also stayed in hospital longer (median (IQR) 9 (7–12) compared with 15 (10–21) days, p < 0.001). Cumulative mortality was higher in patients with late complications (those that occurred after the fourth postoperative day) (61% compared with 36%, p = 0.004). In conclusion, complications in more than half the patients were related to alcohol abuse, a more complicated intraoperative course, and fibular flaps. Complications were associated with a longer hospital stay, and survival was higher in those who did not have late complications than in those who did.

https://ift.tt/2Ooht2A

Equitable vaccination against human papillomavirus: the road ahead

Publication date: Available online 11 August 2018

Source: British Journal of Oral and Maxillofacial Surgery

Author(s): A. Kanatas, D.A. Mitchell

https://ift.tt/2MjDFOd

Complications and outcome after free flap surgery for cancer of the head and neck

We retrospectively studied 136 patients who had free flap reconstruction for cancer of the head and neck at a single centre (2008–2015) to evaluate complications, assess factors associated with them, and analyse their impact on outcome. Preoperative and perioperative data, and surgical and medical complications were recorded, and the impact of the complications on duration of hospital stay and survival were assessed. A total of 86 (63%) patients had complications. Compared with those who did not, they had a higher rate of alcohol abuse (21/86, compared with 5/50, p=0.039), longer operations (median (IQR) 565 (458–653 compared with 479 (418–556) minutes, p<0.001), and greater intraoperative loss of blood (725 (400–1150) compared with 525 (300–800) ml, p=0.042).

https://ift.tt/2MdsYgb

Change of site of intra-articular injection of hypertonic dextrose resulted in different effects of treatment

Most minimally invasive treatments for dysfunction of the temporomandibular joint (TMJ) are empirical, and aimed at the painful trigger points with the purpose of preventing muscular spasm and restoring normal function. In this prospective study I investigated whether the choice of site of injection of hypertonic dextrose affected the benefits of treatment of internal derangement and pain. I studied 72 patients with pain and clicking as a result of dysfunction of the TMJ. Patients were divided into four groups with four separate sites for intra-articular injection.

https://ift.tt/2KL1utk

Equitable vaccination against human papillomavirus: the road ahead

For those of us who treat cancer it has been impossible to understand the resistance against gender-neutral vaccination against human papillomavirus (HPV). The incidence of squamous cell carcinomas of the head and neck that are potentially associated with HPV has been rising, particularly in men.1,2 Cancers other than cervical cancers that may be related are also increasing in incidence. Boys have not been vaccinated by the NHS, unlike girls who are covered by a national vaccination programme.

https://ift.tt/2Ml8stZ

Cortactin as a potential predictor of second esophageal neoplasia in hypopharyngeal carcinoma

Hypopharyngeal carcinoma has a very poor prognosis. The high incidence of second esophageal neoplasia is one of the major causes. To establish an efficient follow-up scheme for increasing the diagnostic yield and reducing the adverse impact of second esophageal neoplasia on survival, the purpose of this study was to explore a biomarker to predict second esophageal neoplasia.

https://ift.tt/2w2gZrD

Health Literacy in Pediatric Otolaryngology: A Scoping Review

Publication date: Available online 11 August 2018

Source: International Journal of Pediatric Otorhinolaryngology

Author(s): Nicole Leigh Aaronson, Catherine L. Joshua, Emily F. Boss

Abstract

Objective

To review research on status and outcomes of health literacy in pediatric otolaryngology and identify opportunities for quality improvement.

Methods

We performed a scoping review, adhering to methodologic standards. A combination of MeSH terms and keywords related to health literacy in otolaryngology was used to conduct a search. Relevant studies were identified using PubMed, Ovid MEDLINE, and Google Scholar databases. Studies were selected for inclusion by two authors if they addressed the domains of pediatric otolaryngology as well as health literacy. Data were abstracted from each study on the number of participants, the setting, the study design, the outcome measure, the intervention used, and the overall theme. Authors identified prominent overarching themes and grouped studies accordingly. Results were then tabulated for further review and to discern implications for future practice and research.

Results

Of 1046 articles identified, 20 articles were included. Studies fell into three major themes: readability of patient materials, patient recall after informed consent, and optimal patient education. Prominent findings included the following: 1. Much of the printed and electronic educational material in otolaryngology is above the recommended reading level for public health information; 2. Parents do not easily recall information provided verbally or in written form; and 3. Adding visual and multimodal components improves the success of parental education.

Conclusion

Health literacy in pediatric otolaryngology may influence comprehension of educational materials and adequacy of informed consent. Future research may address whether patient health literacy affects clinical outcomes.

https://ift.tt/2OsDLjO

Reconstruction of medial orbital wall using a retrocaruncular approach

Publication date: Available online 11 August 2018

Source: Journal of Cranio-Maxillofacial Surgery

Author(s): Christian Pedemonte, Tamara Muñoz, Katherine Valenzuela, Marlene Díaz, L. Edgardo González, Ilich Vargas

Abstract

The purpose of the study was to analyze and describe the retrocaruncular approach to access medial orbital wall.

A retrospective analysis was performed in patients referred for the treatment of orbital fractures between January 1st 2011 and July 31st 2017. The study included patients over 18 years old with isolated fractures of the medial orbital wall or combined with the orbital floor who underwent a transconjunctival approach with retrocaruncular extension and lateral canthotomy, and with a minimum follow-up of 6 months. Patients with fractures to the roof and/or lateral orbital wall were excluded.

From a total of 319 orbits, 30 medial wall fractures were treated using a retrocaruncular approach, transconjunctival extension and lateral canthotomy. 7 of them were pure medial wall fractures and 23 were combined with orbital floor. Except for one case that required delayed reconstruction with customized orbital implant, all orbital reconstructions were successful in the first surgery. Only one patient developed a conjunctival granuloma in relation to the caruncle. No others patients had complications.

This study concluded that this approach is a successful access for surgical treatment of medial orbital wall fractures because of their broad visibility without damaging structures, allowing adequate orbital reconstruction and excellent aesthetic results.

https://ift.tt/2MAEVJK

Healing Pattern of Intentional Pterygoid Plate Fracture after Posterior Movement of Maxilla through Le Fort I Osteotomy

Publication date: Available online 11 August 2018

Source: Journal of Cranio-Maxillofacial Surgery

Author(s): Na-Rae Choi, Sang-Hun Shin, Seong-Sik Kim, George Sandor, Yong-Deok Kim

Summary

Background

To evaluate maxillary stability following Le Fort I osteotomy using postero-superior movement after pterygoid plate fracture. Additionally the authors sought to analyze the postoperative changes at the intentional pterygoid plate fracture site.

Materials and methods

Thirty-six patients with class III deformities treated with total maxillary setback at the Lefort I level were enrolled in a retrospective cohort study. Relative changes in measurement points were identified on cone-beam computed tomographic scans (CBCT) as well as lateral cephalograms. The outcome variables were determined as changes at measurement points obtained preoperatively, immediately postoperatively, and 6 months after surgery.

Results

The average posterior repositioning of ANS in the 36 patients was 1.78 mm and the mean superior repositioning at PNS was 2.78 mm. The maximal recurrence rate was less than 10% at the 6 months postoperative time point. The intentional fracture site of the pterygoid plates healed with a linear pattern. There were no major complications such as airway edema, hemorrhage and nerve damage reported over the 6-month follow-up period.

Conclusions

Intentional fracture of the pterygoid plates has a role in the retropositioning of the maxilla with good healing at the fracture site, little relapse and satisfactory postoperative stability.

https://ift.tt/2MkA0j3

Monogenic polyautoimmunity in primary immunodeficiency diseases

Publication date: Available online 11 August 2018

Source: Autoimmunity Reviews

Author(s): Gholamreza Azizi, Reza Yazdani, Wiliam Rae, Hassan Abolhassani, Manuel Rojas, Asghar Aghamohammadi, Juan-Manuel Anaya

Abstract

Primary immunodeficiency diseases (PIDs) consist of a large group of genetic disorders that affect distinct components of the immune system. PID patients are susceptible to infection and non-infectious complications, particularly autoimmunity. A specific group of monogenic PIDs are due to mutations in genes that are critical for the regulation of immunological tolerance and immune responses. This group of monogenic PIDs is at high risk of developing polyautoimmunity (i.e., the presence of more than one autoimmune disease in a single patient) because of their impaired immunity. In this review, we discuss the mechanisms of autoimmunity in PIDs and the characteristics of polyautoimmunity in the following PIDs: IPEX; monogenic IPEX-like syndrome; LRBA deficiency; CTLA4 deficiency; APECED; ALPS; and PKCδ deficiency.

https://ift.tt/2B2UIj5

The link between immunity, autoimmunity and endometriosis: a literature update

Publication date: Available online 11 August 2018

Source: Autoimmunity Reviews

Author(s): Tao Zhang, Caterina De Carolis, Man Gene Chi-Wai, Chi-Chiu Wang

Abstract

Endometriosis (EMS), an estrogen-dependent inflammatory disorder affects approximately 5–10% of the general female population of reproductive age and 20–90% of women with pelvic pain and infertility. Many immunological factors are known to contribute significantly to the pathogenesis and pathophysiology of EMS, and both chronic local inflammation and autoantibodies in EMS shares many similarities with autoimmune diseases (AD). However, the autoimmune etiology in EMS remains controversial, and its evidence on autoimmune basis may be limited. Here we aim to review the current understanding between autoimmunity and EMS to provide important knowledge to develop future potential immunomodulatory therapy for the treatment of EMS.

https://ift.tt/2P2n283

l-Thyroxine does not prevent immunemediated sensorineural hearing loss in autoimmune thyroid diseases

Publication date: Available online 11 August 2018

Source: Acta Otorrinolaringológica Española

Author(s): Antonio Rodríguez-Valiente, Óscar Álvarez-Montero, Carmen Górriz-Gil, José Ramón García-Berrocal

Abstract

Objective

This is the first report dealing with immune-mediated inner ear disease (IMIED) hearing loss in a group of patients affected with autoimmune thyroid disease (AITD), whose treatment required corticosteroids, despite being treated with levothyroxine. Immunopathology linking the inner ear and the thyroid gland is also presented.

Patients

A total of 220 patients were selected with sensorineural hearing loss (SNHL) of causes other than presbycusis. Audiometry was performed and pure tone average was calculated before and after treatment with corticosteroids.

Results

Eighty-four (84) patients had SNHL of autoimmune origin, and 15 patients were diagnosed with AITD (Hashimoto's disease). Bilateral hearing loss was observed in 10 patients (66.5%). Sudden sensorineural hearing loss was the most frequent clinical form of presentation. Nine patients showed a hearing recovery greater than 10 dB after corticosteroid treatment.

Conclusions

Acquired hypothyroidism is thought to affect hearing due to different mechanisms. Although specific hormonal therapy may improve peripheral or central auditory disorders associated with hypothyroidism, the presence of IMIED in AITD patients requires another approach. Altered immune regulatory mechanisms involving Treg cells and CD4+CD45RO cells have been suggested in patients with AITD and IMIED. In the present study, although all the patients with hypothyroidism and subclinical hypothyroidism were being treated with levothyroxine, immune-mediated hearing loss was observed. Therapy with corticosteroids could achieve hearing recovery. Since inner ear and thyroid gland share possible antigen targets, we highlight the existence of IMIED in AITD patients and the importance of implementing appropriate therapy with corticosteroids.

Resumen

Objetivo

Este es el primer trabajo que trata la hipoacusia por enfermedad inmune-mediada del oído interno (IMIED) en un grupo de pacientes afectados de tiroiditis autoinmune (AITD), cuyo tratamiento requirió corticosteroides, a pesar de haber sido tratados con levotiroxina. También se presenta la inmunopatología que vincula el oído interno y la glándula tiroides.

Pacientes

Se seleccionó un total de 220 pacientes con hipoacusia neurosensorial (SNHL) por causas diferentes a presbiacusia. A todos los pacientes se les realizó una audiometría, calculándose la media de tonos puros antes y después del tratamiento con corticosteroides.

Resultados

Ochenta y cuatro (84) pacientes tenían SNHL de origen autoinmune, y 15 pacientes fueron diagnosticados de AITD (Enfermedad de Hashimoto). Se observó hipoacusia bilateral en 10 pacientes (66,5%). La sordera súbita fue la forma de presentación clínica más frecuente. Nueve pacientes presentaron una recuperación auditiva superior a 10 dB tras el tratamiento con corticosteroides.

Conclusiones

Se piensa que el hipotiroidismo adquirido afecta a la audición por diferentes mecanismos. Aunque la terapia hormonal específica puede mejorar los trastornos auditivos periféricos o centrales asociados al hipotiroidismo, la presencia de IMIED en los pacientes de AITD requiere otro abordaje. Se ha sugerido una alteración de los mecanismos reguladores de la respuesta inmune que implica a las células de Treg y a las células CD4+CD45RO en los pacientes con AITD e IMIED. En el presente estudio, a pesar de que todos los pacientes con hipotiroidismo e hipotiroidismo subclínico estaban siendo tratados con levotiroxina, se observó hipoacusia inmuno-mediada. La terapia con corticosteroides podría lograr una recuperación auditiva. Dado que el oído interno y la glándula tiroides comparten posibles antígenos diana, destacamos la existencia de IMIED en los pacientes de AITD, y la instauración de una terapia adecuada con corticosteroides.

https://ift.tt/2P2029u

Prurigo pigmentosa au cours de la grossesse

Publication date: Available online 11 August 2018

Source: Annales de Dermatologie et de Vénéréologie

Author(s): I. Devred, A. Sfecci, N. Cardot-Leccia, J.-P. Lacour, T. Passeron

Résumé

Introduction

Le prurigo pigmentosa est une dermatose inflammatoire rare marquée par son caractère prurigineux et son aspect réticulé typique du tronc, laissant un réseau pigmenté cicatriciel. Elle est très rarement rapportée en dehors de l'Asie et est encore méconnue en France.

Observation

Une femme de 21 ans, originaire du Maghreb, présentait une éruption très prurigineuse, faite de macules et de papules coalescentes formant un réseau réticulé inflammatoire sur le tronc. Cette éruption survenait lors du premier trimestre d'une grossesse marquée par des vomissements gravidiques majeurs ayant conduit à la perte de 13 kg, avec présence d'une cétonémie et d'une cétonurie. Devant l'aspect clinique caractéristique, l'absence de diagnostic différentiel et un aspect histologique compatible, le diagnostic de prurigo pigmentosa était retenu. L'évolution était marquée par des phases de diminution de l'inflammation et du prurit en alternance avec des poussées inflammatoires rythmées par des ré-ascensions de la cétonémie. Les symptômes étaient seulement résolus au deuxième trimestre de la grossesse, après disparition des vomissements, laissant un réseau hyperpigmenté brun séquellaire.

Discussion

Bien que rare, le prurigo pigmentosa a un aspect clinique très évocateur qui doit facilement en faire évoquer le diagnostic. D'étiologie encore indéterminée, il a été rapporté dans des cas de jeûne intense, d'anorexie, de diabète de type 1 mais aussi dans deux autres cas de grossesse avec vomissements intenses. Notre observation souligne l'importance de rechercher et de prendre en charge efficacement une cétonémie sous-jacente pour obtenir une rémission des poussées inflammatoires de prurigo pigmentosa.

Summary

Introduction

Prurigo pigmentosa is a rare inflammatory dermatosis characterized by pruritic and reticulate papules on the trunk leaving hyperpigmentation. This dermatosis has been rarely described outside Asia. The pathophysiology remains obscure.

Case report

We report the case of a 21-year-old North-African woman presenting with a highly pruritic eruption with numerous erythematous macules and papules coalescing in a reticular pattern on the trunk. The eruption occurred during the first trimester of pregnancy, which was marked by severe vomiting resulting in weight loss of 13 kg with ketonemia and ketonuria. Taking into account the characteristic pattern of the eruption, the absence of differential diagnosis, and the histological examination, we concluded on a diagnosis of prurigo pigmentosa. Progression of the disease exhibited phases of decreased inflammation and of pruritus alternating with episodes of inflammatory flares triggered by relapses of ketonemia. The symptoms finally resolved during the 2nd trimester of pregnancy after vomiting ceased. Secondary reticulated hyperpigmentation was observed.

Conclusion

Although rare, the highly evocative clinical presentation of the eruption should help clinicians in diagnosing prurigo pigmentosa. While pathophysiology remains undetermined, prurigo pigmentosa was reported in cases of intense fasting, anorexia, type-1 diabetes, and in two other cases of pregnancy with severe vomiting. Our case underlines the need to screen for and treat underlying ketonemia to achieve control of the inflammatory flares of prurigo pigmentosa.

https://ift.tt/2AXwzKN

Paraneoplastic autoimmune multiorgan syndrome (PAMS): Beyond the single phenotype of paraneoplastic pemphigus

Publication date: Available online 11 August 2018

Source: Autoimmunity Reviews

Author(s): Kyle T. Amber, Manuel Valdebran, Sergei A. Grando

Abstract

Paraneoplastic autoimmune multiorgan syndrome (PAMS) is characterized by a heterogenous group of signs and symptoms including severe desquamative stomatitis, a polymorphous cutaneous eruption, humoral immunity against plakin proteins, contribution of cell-mediated autoimmunity and commonly a progressive respiratory failure. Autoantibodies in PAMS target a wide array of antigens including plakins, cadherins, alpha-2-macroglobulin like 1 (A2ML1), BP180, plakophilin-3, and several neuromuscular antigens. Originally described as paraneoplastic pemphigus in 1990 due to some of its clinical and immunologic similarities to classic pemphigus (pemphigus vulgaris and pemphigus foliaceus), PAMS is a multiorganopathy with several distinct features from these classic forms of pemphigus. Epidemiologically, PAMS is associated with underlying neoplasia and has a differing HLA-II allele predisposition compared to classic forms of pemphigus. Clinically, lesion morphology is polymorphous, and lesion distribution fundamentally differs from that seen in classic pemphigus. PAMS has a significantly higher mortality rate and a poorer response to treatments typically effective in pemphigus. Histologically, PAMS is characterized by the presence of interface dermatitis, vacuolar changes, and dyskeratotic keratinocytes which are not seen in classic pemphigus. PAMS demonstrates not only intercellular IgG as seen in classic pemphigus, but the presence of linear basement membrane zone deposition. Antibodies against desmoglein 3 (Dsg3) map to a broader array of epitopes than in pemphigus vulgaris and there is a higher prevalence of complement binding anti-Dsg3 IgG autoantibodies in PAMS. Autoantibodies can in rare cases be absent in the more cell-mediated form of PAMS. Considering these numerable differences, we review the entity of PAMS, and provide similarities and differences to classic forms of pemphigus.

https://ift.tt/2P1JAWP

Recommendations and barriers to vaccination in systemic lupus erythematosus

Publication date: Available online 11 August 2018

Source: Autoimmunity Reviews

Author(s): Megha Garg, Naaima Mufti, Tara Palmore, Sarfaraz Hasni

Abstract

Patients with Systemic Lupus Erythematosus (SLE) pose a unique dilemma pertaining to immunization against common pathogens. SLE patients are usually not immunized with vaccines based on the fear of either precipitating infection in this immunosuppressed patient population (with live vaccines) or aggravating autoimmunity and hence lupus flares (with any vaccines). However, elevated vulnerability to infection makes patients with SLE precisely the population that needs protection from vaccine-preventable diseases. A summary of guidelines from the Centers for Disease Control and Prevention, professional societies, review articles and expert opinions regarding use of individual vaccines applicable to adults with SLE is presented in this review.

https://ift.tt/2KK14nc

Increased risk of thrombosis in antiphospholipid syndrome patients treated with direct oral anticoagulants. Results from an international patient-level data meta-analysis

Publication date: Available online 11 August 2018

Source: Autoimmunity Reviews

Author(s): Virginie DUFROST, Jessie RISSE, Tatiana RESHETNYAK, Maria SATYBALDYEVA, D.U. Yao, Xin-xin YAN, Stella SALTA, Grigorios GEROTZIAFAS, Zhi-Cheng JING, Ismaël ELALAMY, Denis WAHL, Stéphane ZUILY

Abstract

Background: Direct oral anticoagulants (DOACs) are widely used for secondary prevention of venous thromboembolism (VTE) but their clinical efficacy and safety are not established in Antiphospholipid Syndrome (APS) patients. There is only one randomized controlled trial published while others are still ongoing. Many non-randomized studies have been published in this field with conflicting opinions.

Purpose of review: We conducted a systematic review using MEDLINE, EMBASE and Cochrane databases from 2000 until March 2018 regarding APS patients treated with DOACs. We performed a patient-level data meta-analysis to a) estimate the prevalence of recurrent thrombosis in APS patients treated with DOACs in the literature, and b) identify variables associated with recurrent thrombosis.

Results: We identified 47 studies corresponding to 447 APS patients treated with DOACs. Three commercially available DOACs were analyzed: rivaroxaban (n = 290), dabigatran etexilate (n = 144) and apixaban (n = 13). A total of 73 out of 447 patients (16%) experienced a recurrent thrombosis while on DOACs with a mean duration until thrombosis of 12.5 months. Rates of recurrent thromboses were 16.9% and 15% in APS patients receiving either anti-Xa inhibitors or dabigatran respectively. Triple positivity (positivity for all three antiphospholipid antibodies) was associated with a four-fold increased risk of recurrent thrombosis (56% vs 23%; OR = 4.3 [95%CI; 2.3–7.7], p < .0001) as well as a higher number of clinical criteria for APS classification. In patients treated with anti-Xa inhibitors, history of arterial thrombosis was associated with a higher risk of recurrent thrombosis (32% vs 14%; OR = 2.8 [95%CI; 1.4–5.7], p = .006).

In conclusion, DOACs are not effective in all APS patients and should not be used routinely in these patients. Randomized controlled trials assessing clinical efficacy and safety as primary endpoints are underway. In the meantime, a registry of APS patients on DOACs could be proposed to establish in which APS subgroups DOACs would be a safe alternative to warfarin.

Graphical abstract

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Trichotillomania and Trichophagia: Modern Diagnostic and Therapeutic Methods

Abstract

Trichotillomania is a chronic, mental disease of impulse control, characterized by repetitive, compulsive, and self-induced hair pulling. It can occur at any age but is observed more often in adolescents, with a strong predominance in females. Diagnosis of trichotillomania may be difficult, and its effective treatment challenging. The aim of this study is to critically review current literature regarding diagnostic procedures and treatment of trichotillomania, including psychotherapy, N-acetylcysteine, naltrexone, topiramate, atypical neuroleptics, and selective serotonin-reuptake inhibitors. The importance of cooperation between dermatologists and psychiatrists is emphasized to shorten the time to diagnose the disease and begin appropriate treatment. Finally, trichotillomania is also often connected with trichophagia, which may lead to formation of trichobezoars and cause a direct danger to the patient's health and even life due to the risk of intestinal obstruction and the need for surgical intervention. Based on thorough literature review, we conclude that diagnosis of trichotillomania can be challenging. Trichoscopy could help to distinguish trichotillomania from other types of hair loss. Most clinical trials using various treatment options have been conducted on small groups of patients, and the potential benefits determined using various scales. Therefore, it is difficult to compare the effectiveness of different treatment methods. There is also a lack of studies assessing treatment efficacy over longer periods of time. Thus, there is a need to perform better-designed studies in the near future to optimize current treatment modalities for trichotillomania.

https://ift.tt/2vYOWJC

The surgical management of extra-articular ankylosis in noma patients

Publication date: Available online 10 August 2018

Source: International Journal of Oral and Maxillofacial Surgery

Author(s): E.M. Rüegg, D. Baratti-Mayer, A. Jaquinet, D. Montandon, B. Pittet-Cuénod

Abstract

Extraarticular ankylosis occurs frequently in children suffering from noma sequelae. Over 20 years of operating on these patients, we observed high recurrence of mouth opening limitation. We therefore progressively changed our surgical strategy. This retrospective study compares the impact of different parameters (types of surgery, noma type, physiotherapy compliance, age and sex) on immediate and long-term mouth opening. It includes a series of 121 patients with extraarticular ankylosis operated on between 1990 and 2015. Soft tissue reconstruction evolved from local and pedicled flaps to large free flaps. Mouth opening was performed by bone-bridge excision, sometimes associated to contralateral coronoidectomy. Mouth opening technique including bilateral coronoidectomy with free flap reconstruction was the only independent factor for significantly better immediate mouth opening with a mean increase of 8.7 mm [95% confidence interval (CI) 4.3–13.1, P < 0.001) and this effect was maintained in the 3 years of follow-up. Another positive factor related to long-term results was excellent physiotherapy, while noma type 4 was a negative factor. Recurrence remains problematic in the management of noma sequelae. If physiotherapy and long-term follow-up cannot be offered, patients should not be operated on, because if limitation of mouth opening recurs, oral feeding may become impossible when a facial defect has been reconstructed.

https://ift.tt/2P1PqaD

Feel the power: sample size and meaningful effects

Publication date: Available online 10 August 2018

Source: British Journal of Oral and Maxillofacial Surgery

Author(s): A.B. Smith

https://ift.tt/2MC4kmf

L’hydrochlorothiazide augmenterait le risque de mélanome

Publication date: Available online 10 August 2018

Source: Annales de Dermatologie et de Vénéréologie

Author(s): J.-L. Schmutz

https://ift.tt/2w13hVE

Circular RNA alterations in the Bombyx mori midgut following B. mori nucleopolyhedrovirus infection

Publication date: September 2018

Source: Molecular Immunology, Volume 101

Author(s): Xiaolong Hu, Min Zhu, Bo Liu, Zi Liang, Lixu Huang, Jian Xu, Lei Yu, Kun Li, Mengsheng Jiang, Renyu Xue, Guangli Cao, Chengliang Gong

Abstract

Thus far, no systematic studies have examined circRNA expression profiles in the silkworm following B.mori nucleopolyhedrovirus (BmNPV) infection. To explore the expression patterns of circRNAs in the silkworm midgut following BmNPV infection, circRNAs in normal midguts and BmNPV-infected midguts were analyzed by high-throughput sequencing. A total of 353 circRNAs were significantly differentially expressed, of which 241 were upregulated and 112 were downregulated following infection. GO annotation and KEGG pathways analyses of these circRNAs showed that many key immunity pathways and metabolism pathways were enriched in the BmNPV-infected midguts. The potential roles of the predicted targets of the miRNAs that interacted with the circRNAs showed that ubiquitin, apoptosis, and endocytosis signaling pathways were enriched significantly by BmNPV infection.

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Comparison of a Novel Cadaver Model (Fix for Life) With the Formalin-Fixed Cadaver and Manikin Model for Suitability and Realism in Airway Management Training

BACKGROUND: Manikins are widely used in airway management training; however, simulation of realism and interpatient variability remains a challenge. We investigated whether cadavers embalmed with the novel Fix for Life (F4L) embalmment method are a suitable and realistic model for teaching 3 basic airway skills: facemask ventilation, tracheal intubation, and laryngeal mask insertion compared to a manikin (SimMan 3G) and formalin-fixed cadavers. METHODS: Thirty anesthesiologists and experienced residents ("operators") were instructed to perform the 3 airway techniques in 10 F4L, 10 formalin-fixed cadavers, and 1 manikin. The order of the model type was randomized per operator. Primary outcomes were the operators' ranking of each model type as a teaching model (total rank), ranking of the model types per technique, and an operator's average verbal rating score for suitability and realism of learning the technique on the model. Secondary outcomes were the percentages of successfully performed procedures per technique and per model (success rates in completing the respective airway maneuvers). For each of the airway techniques, the Friedman analysis of variance was used to compare the 3 models on mean operator ranking and mean verbal rating scores. RESULTS: Twenty-seven of 30 operators (90%) performed all airway techniques on all of the available models, whereas 3 operators performed the majority but not all of the airway maneuvers on all models for logistical reasons. The total number of attempts for each technique was 30 on the manikin, 292 in the F4L, and 282 on the formalin-fixed cadavers. The operators' median total ranking of each model type as a teaching model was 1 for F4L, 2 for the manikin and, 3 for the formalin-fixed cadavers (P

https://ift.tt/2KKz42v

Continuous Popliteal-Sciatic Blocks for Postoperative Analgesia: Traditional Proximal Catheter Insertion Superficial to the Paraneural Sheath Versus a New Distal Insertion Site Deep to the Paraneural Sheath

We tested the hypothesis that during a continuous popliteal-sciatic nerve block, postoperative analgesia is improved with the catheter insertion point "deep" to the paraneural sheath immediately distal to the bifurcation between the tibial and common peroneal branches, compared with the traditional approach "superficial" to the paraneural sheath proximal to the bifurcation. The needle tip location was determined to be accurately located with a fluid bolus visualized with ultrasound; however, catheters were subsequently inserted without a similar fluid injection and visualization protocol (visualized air injection was permitted and usually implemented, but not required per protocol). The average pain (0–10 scale) the morning after surgery for subjects with a catheter inserted at the proximal subparaneural location (n = 31) was a median (interquartile) of 1.5 (0.0–3.5) vs 1.5 (0.0–4.0) for subjects with a catheter inserted at the distal supraparaneural location (n = 32; P = .927). Secondary outcomes were similarly negative. Accepted for publication July 2, 2018. Funding: This work was supported by the Department of Anesthesiology, University California San Diego (San Diego, CA). The authors declare no conflicts of interest. Institutional review board: University of California, San Diego (UCSD) Human Research Protections Program at 9500 Gilman Dr, Mail Code 0052, La Jolla, CA 92093–0052; (858) 657–5100. E-mail: tnelson@ucsd.edu. This report describes human research. This study was conducted with written informed consent from the study subjects. This report describes a prospective randomized clinical trial. The authors state that the report includes every item in the Consolidated Standards of Reporting Trials (CONSORT) checklist for a prospective randomized clinical trial. The study was registered before patient enrollment. This was not an observational clinical study. Registry URL: clinicaltrials.gov NCT02523235. Plain Language Summary: We found no evidence that during a "continuous" popliteal nerve block, postoperative analgesia is improved with the perineural catheter tip deep to the paraneural sheath immediately distal to the sciatic bifurcation, compared with a traditional approach with the catheter tip superficial to the paraneural sheath proximal to the bifurcation. The contents of this article are solely the responsibility of the authors and do not necessarily represent the official views of the funding entity. Reprints will not be available from the authors. Address correspondence to Brian M. Ilfeld, MD, MS (Clinical Investigation), Department of Anesthesiology, University California, San Diego, 200 W Arbor Dr, MC 8770, San Diego, CA, 92103. Address e-mail to bilfeld@ucsd.edu. © 2018 International Anesthesia Research Society

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Status of Women in Academic Anesthesiology: A 10-Year Update

BACKGROUND: Gender inequity is still prevalent in today's medical workforce. Previous studies have investigated the status of women in academic anesthesiology. The objective of this study is to provide a current update on the status of women in academic anesthesiology. We hypothesized that while the number of women in academic anesthesiology has increased in the past 10 years, major gender disparities continue to persist, most notably in leadership roles. METHODS: Medical student, resident, and faculty data were obtained from the Association of American Medical Colleges. The number of women in anesthesiology at the resident and faculty level, the distribution of faculty academic rank, and the number of women chairpersons were compared across the period from 2006 to 2016. The gender distribution of major anesthesiology journal editorial boards and data on anesthesiology research grant awards, among other leadership roles, were collected from websites and compared to data from 2005 and 2006. RESULTS: The number (%) of women anesthesiology residents/faculty has increased from 1570 (32%)/1783 (29%) in 2006 to 2145 (35%)/2945 (36%) in 2016 (P = .004 and P

https://ift.tt/2OtFmG7

Diagnostic Testing and Decision-Making: Beauty Is Not Just in the Eye of the Beholder

To use a diagnostic test effectively and consistently in their practice, clinicians need to know how well the test distinguishes between those patients who have the suspected acute or chronic disease and those patients who do not. Clinicians are equally interested and usually more concerned whether, based on the results of a screening test, a given patient actually: (1) does or does not have the suspected disease; or (2) will or will not subsequently experience the adverse event or outcome. Medical tests that are performed to screen for a risk factor, diagnose a disease, or to estimate a patient's prognosis are frequently a key component of a clinical research study. Like therapeutic interventions, medical tests require proper analysis and demonstrated efficacy before being incorporated into routine clinical practice. This basic statistical tutorial, thus, discusses the fundamental concepts and techniques related to diagnostic testing and medical decision-making, including sensitivity and specificity, positive predictive value and negative predictive value, positive and negative likelihood ratio, receiver operating characteristic curve, diagnostic accuracy, choosing a best cut-point for a continuous variable biomarker, comparing methods on diagnostic accuracy, and design of a diagnostic accuracy study. This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. Accepted for publication July 3, 2018. Funding: None. The authors declare no conflicts of interest. Reprints will not be available from the authors. Address correspondence to Thomas R. Vetter, MD, MPH, Department of Surgery and Perioperative Care, Dell Medical School at the University of Texas at Austin, Health Discovery Bldg, Room 6.812, 1701 Trinity St, Austin, TX 78712. Address e-mail to thomas.vetter@austin.utexas.edu. © 2018 International Anesthesia Research Society

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Narrative Review of Decision-Making Processes in Critical Care

Several theories describing the decision-making process in the intensive care unit (ICU) have been formulated. However, none of them appreciate the complexities of the process in an eclectic way by unifying several miscellaneous variables in 1 comprehensive theory. The purpose of this review is to highlight the key intricacies associated with the decision-making process in the ICU, to describe the theoretical frameworks with a special emphasis on gaps of knowledge, and to offer some avenues for improvement. The application of theoretical framework helps us to understand and to modify the structure of the process. Expected utility theory, regret theory, prospect theory, fuzzy-trace theory, construal level theory, and quantum probability theory were formulated over the years to appreciate an increased complexity of the decision-making process in the ICU. However the decision makers engage, these models may affect patient care because each of these frameworks has several benefits and downsides. There are gaps of knowledge in understanding how physicians match the different theoretical frameworks of the decision-making process with the potentially high ICU variability and load, especially when the "best outcome" is often nondiscrete and multidimensional. Furthermore, it is unclear when the preferential application of reflexive, habitual, variable, and biased-prone processes results in patient and staff detriment. We suggest better matching of theoretical frameworks with strengths of the human decision-making process and balanced application computer aids, artificial intelligence, and organizational modifications. The key component of this integration is work to increase the self-awareness of decision-making processes among residents, fellows, and attending physicians. Accepted for publication June 19, 2018. Funding: None. The authors declare no conflicts of interest. Reprints will not be available from the authors. Address correspondence to Krzysztof Laudanski, MD, PhD, AM FCCM, Department of Anesthesiology and Critical Care, Perelman School of Medicine at the University of Pennsylvania, 3620 Hamilton Walk, Philadelphia, PA 19104. Address e-mail to klaudanski@gmail.com. © 2018 International Anesthesia Research Society

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Advances in Surgery for Chronic Rhinosinusitis

Abstract

Purpose of Review

Our understanding of chronic rhinosinusitis (CRS) is always evolving with new concepts and technological advancements in order to achieve the best surgical outcomes.

Recent Findings

The extent of surgery has long been debated and current research has provided surgeons indications to create larger openings for the maxillary and frontal sinus. Historical concerns for larger openings impacting outcomes have been resolved with better understanding of post-operative management. Recent literature advocates the mega-antrostomy in the setting of cystic fibrosis or recalcitrant CRS despite previous standard FESS openings. Draf 3 is known to be used in cases of frontal sinus tumors, CSF leaks, mucoceles, and recalcitrant CRS to standard surgery. Technological advances have had impacted rhinology positively, with a number of in-office procedures becoming more common as well as new intra-operative tools to facilitate surgery. Moreover, post-operatively, the advent of a drug-eluting stent has fastened the healing process following FESS leading to better outcomes.

Summary

Advances in sinus surgery are always evolving. What may have been considered a poor approach in the past are being re-visited with better understanding of CRS and technological advancements.

https://ift.tt/2KIXzgK