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Purpose. A case series of icatibant use in intubated patients with angiotensin-converting enzyme inhibitor- (ACEI-) induced angioedema is presented along with a relevant literature review and recommendations for utilization. Summary. Three intubated patients admitted to the intensive care unit for ACEI-induced angioedema were treated with icatibant. A literature search identified one controlled study and four case reports describing the use of icatibant in intubated ACEI-induced angioedema patients. Conclusion. Icatibant administration in intubated patients may be beneficial in decreasing time to extubation and length of intensive care unit stay. In the three cases described, icatibant administration did not appear to elicit a response in intubated patients, which has been described in previous case reports. For clinicians considering icatibant in the treatment of ACEI-induced angioedema, earlier administration upon arrival to the ED or immediately upon arriving to the intensive care unit is strongly advised. The suggested benefit of icatibant in intubated ACEI-induced angioedema patients should be verified by randomized clinical trials and cost-benefit analyses should be performed at individual institutions. [ABSTRACT FROM AUTHOR]

Background & Aims: Celiac disease (CD) presents with a wide spectrum of extra-digestive symptoms, including hemorrhagic manifestations. The aim of this review was to conduct an extensive analysis of the hemorrhagic events reported in adult CD patients. Methods: Case report and review of the literature. Pubmed (MEDLINE) database search from January 1970 onwards was performed using the medical subject headings [MeSH] terms "celiac disease" AND "blood coagulation disorders", "hemorrhage", "hematoma", "hematuria", "hemoptysis", "epistaxis", "hemosiderosis". Only case reports were identified on the search theme. Information on patients' characteristics, diagnostic features, coagulation parameters, symptomatology duration, and evolution under treatment were systematically collected and summarized. Results: We present the case of a 40-year-old man hospitalized for spontaneous muscular hematomas, in whom CD was diagnosed. We performed a review of the literature and summarized the published case reports of 46 CD patients, aged between 19 and 74 years, 64% of male gender. In 25% of cases, the symptomatology was present for more than 5 years prior to CD diagnosis. The clinical hemorrhagic events were as follows: 15 patients had gastrointestinal bleeding, 9 hemoptysis, 4 epistaxis, 6 hematuria, 8 cutaneous hematoma, petechia or ecchymoses, and only in 1 case hemarthrosis, hemorrhagic vesicular dermatitis, subcortical hemorrhage, or adrenal hemorrhage. Sixty percent of the patients had digestive symptoms, while the rest had only extradigestive CD involvement. The Lane Hamilton syndrome was defined in 15 patients. The evolution under a gluten-free diet was favorable in most cases. Conclusion: This review of case reports aims to increase awareness to hemorrhagic events, rare but possible life-threatening conditions, as part of the CD clinical spectrum. To the best of our knowledge, this is the first review of all types of hemorrhagic events in adult CD patients. [ABSTRACT FROM AUTHOR]

Introduction. Surgical mesh procedures addressed to inguinal hernia leads to better results in term of recurrence, but the problems of postoperative pain still remains, and mesh fixation is considered to be one of the factors. Our objective is to determine the impact of cyanoacrylate glues for mesh fixation in clinical results considering postoperative acute and chronic pain, complications and recurrence. Material and methods. We performed a systematic review of literature, searching for "cyanoacrylates" (MeSH Terms) AND "hernia" (MeSH Terms) AND "herniorrhaphy" (MeSH Terms) in order to find papers that discuss the use of cyanoacrylate glues in mesh fixation for hernia repair. Results. We found 27 articles: 17 randomized trials (RT), 8 case series, 1 review and 1 meta-analysis. The authors of these papers come from only two regions: Europe and Asia. 4 surgical procedures were used in combinations with cyanoacrylate mesh fixation: Lichtenstein, plug and patch technique, TAPP and TEP. No studies showed the inferiority of glue over the other methods of fixation considering all four postoperative outcomes (acute pain, complication, chronic pain and recurrence). The synthetic glue fixation achieves better results in terms of acute and chronic pain for both open and minimally-invasive surgical procedures for inguinal hernia repair. Conclusions. Although synthetic glue mesh fixation for inguinal hernia is not widely used over the world, it is a safe and effective way for mesh fixation both in open and minimally-invasive inguinal hernia repair and significantly decreases postoperative acute and chronic pain. [ABSTRACT FROM AUTHOR]

Endocarditis is a rare but serious manifestation of Listeria monocytogenes (LM). However, the optimal treatment strategy for LM endocarditis has yet to be established. Current antibiotic strategies for listeriosis include penicillin G or ampicillin (AMP) monotherapy, or AMP + gentamicin combination therapy which is often favored for endocarditis. The primary objective of our investigation was to assess the utility of AMP + ceftriaxone (CRO) and AMP + daptomycin (DAP) against LM, modeling less nephrotoxic antibiotic combinations traditionally used to manage resistant enterococcal endocarditis. Here we report a case of LM endocarditis, review the world literature, and evaluate alternative treatment strategies for listeriosis utilizing in vitro and ex vivo studies. The combination of AMP + CRO and AMP + DAP were each noted to have synergistic activity against a LM endocarditis isolate. Additionally, co-incubation of the isolate with sub-lethal concentrations of antibiotics (AMP, CRO, DAP, AMP + CRO or AMP + DAP) sensitized the bacterium to whole blood killing while pretreatment with CRO and DAP (at 1/4 MIC) sensitized the bacterium to neutrophil killing. However, these effects did not reflect potentiation of antibiotic activity to human cathelicidin peptide LL-37, which is abundant in neutrophils and highly active against LM. Interestingly, AMP pretreatment of the LM endocarditis isolate resulted in increased DAP binding to the bacterium when assessed by fluorescence microscopy. These in vitro and ex vivo studies suggest further investigation of combination therapy using AMP + CRO or AMP + DAP as an alternative treatment for LM infection is warranted. [ABSTRACT FROM AUTHOR]

Background: Anti-N-methyl-D-aspartate receptor [anti-NMDAR] encephalitis is an autoimmune encephalitis commonly associated with ovarian teratoma [OT]. Currently, there is no literature for anti-NMDAR encephalitis in Thai patients who may have different clinical manifestations compared to patients from other regions. Objective: To evaluate the clinical characteristics, assessments, and outcomes of a series of anti-NMDAR encephalitis in Thai patients and review of the literature. Materials and Methods: All adult Thai patients with anti-NMDAR encephalitis confirmed positive by one anti-NMDAR antibody test, either in serum or cerebrospinal fluid [CSF], and hospitalized in the Department of Medicine, Siriraj Hospital, Thailand, between 2007 and 2016, were identified from the hospital's database. Results: Nine patients with a median age of 21 years (range 15 to 49), including eight (89%) that were female, were included in this study. Initial presentations included seizures (4 patients, 44%), psychiatric symptoms (4, 44%), and cognitive impairment (1,11%). Chronic relapsing inflammatory optic neuropathy proceeded to encephalopathy in one patient (11%). Six patients (67%) had abnormalities in brain imaging, revealed by computed tomography with contrast or magnetic resonance imaging, or both. Leptomeningeal enhancement was present in two patients (22%). An OT was found in three out of six patients (5 0%), all of whom underwent tumor removal. Immunotherapy was given to all patients. Clinical outcomes were marked recovery in four patients (44%), persistent cognitive deficit and partial dependency in two (22%), and death by hospital-acquired pneumonia in two (22%). Conclusion: The majorities of the initial manifestations of anti-NMDAR encephalitis in the Thai patients were seizures and psychiatric symptoms. The first episode of seizure without other neurological abnormalities especially among young adult women may reveal anti-NMDAR encephalitis. CRION may precede such an encephalitis. Leptomeningeal enhancement, with or without intraparenchymal lesion, may be more frequently seen in Thai patients. [ABSTRACT FROM AUTHOR]

Multiple pituitary adenomas are rare. We present a quite unique case of double pituitary adenomas associated with persistent trigeminal artery (PTA) treated by endoscopic surgery. To the best of our knowledge, this is the first report in the literature. A 64-year-old woman was referred to our hospital for suspicion of acromegaly. Preoperative magnetic resonance imaging revealed two separate intrasellar masses with intrasellar vascular structure. Right cerebral angiography showed medial-type PTA. The patient underwent endoscopic transsphenoidal surgery and both tumors were resected completely. Postoperative immunohistopathologic examination revealed two histologic types of adenoma: the first tumor was positive for growth hormone (GH), while the second was considered nonfunctioning. Postoperatively, the patient's serum levels of GH and insulin-like growth factor-1 returned to normal. We observed an extremely rare case of double pituitary adenomas associated with PTA. Preoperative neuroimaging and modern endoscopic surgery are valuable to confirm diagnosis of double pituitary adenomas and identify anatomical localization of PTA. [ABSTRACT FROM AUTHOR]

There is growing recognition of the kyphotic clivo-axial angle (CXA) as an index of risk of brainstem deformity and craniocervical instability. This review of literature and prospective pilot study is the first to address the potential correlation between correction of the pathological CXA and postoperative clinical outcome. The CXA is a useful sentinel to alert the radiologist and surgeon to the possibility of brainstem deformity or instability. Ten adult subjects with ventral brainstem compression, radiographically manifest as a kyphotic CXA, underwent correction of deformity (normalization of the CXA) prior to fusion and occipito-cervical stabilization. The subjects were assessed preoperatively and at one, three, six, and twelve months after surgery, using established clinical metrics: the visual analog pain scale (VAS), American Spinal InjuryAssociation Impairment Scale (ASIA), Oswestry Neck Disability Index, SF 36, and Karnofsky Index. Parametric and non-parametric statistical tests were performed to correlate clinical outcome with CXA. No major complications were observed. Two patients showed pedicle screws adjacent to but not deforming the vertebral artery on post-operative CT scan. All clinical metrics showed statistically significant improvement. Mean CXA was normalized from 135.8° to 163.7°. Correction of abnormal CXA correlated with statistically significant clinical improvement in this cohort of patients. The study supports the thesis that the CXA maybe an important metric for predicting the risk of brainstem and upper spinal cord deformation. Further study is feasible and warranted. [ABSTRACT FROM AUTHOR]

Hemispherotomy is an established surgical technique to cure or palliate selected, mostly young patients suffering from refractory epilepsy. However, a few patients continue to have seizures despite the surgical hemispherical disconnection. We present a case series of patients who underwent redo hemispherotomy after a first unsuccessful hemispherical disconnection and provide a roadmap for subsequent workup and treatment. The institutional database of epilepsy surgery was reviewed. Twenty-four patients who underwent hemispherotomies for refractory epilepsy were identified between 2007 and 2016. Patients' notes were checked for demographics, history, clinical presentation, preoperative workup, medical treatment, age at first hemispherotomy, and surgical technique. Complications, histopathology, postoperative antiepileptic drug, and postoperative neurological follow-up were documented. Engel class was used to determine the outcome after surgery. Three patients (one hemimegalencephaly, one perinatal stroke, and one Rasmussen's disease) underwent redo hemispherotomy after electroencephalography and MRI studies with particular importance given to diffusion tensor imaging (DTI) to demonstrate residual connection between hemispheres. In one case, redo disconnection followed by a frontal lobectomy rendered the patient seizure-free (Engel class I). In one case, the seizure frequency remained the same but generalized seizures disappeared (Engel class III), and in one case, seizure frequency was considerably reduced after the redo disconnection (Engel class II), with a minimum follow-up of 2 years. Surgical aspects, possible reasons of failure of first hemispherotomy, and rationale that led to second-look surgery are presented. Reasons for failure can be related to patient's selection and/or surgical aspects. Hemispherotomy is a technically demanding procedure and requires accurate preoperative workup. Redo hemispherotomy is a valid option on the basis of further epileptological and radiological workup to demonstrate residual interhemispheric connections and/or rule out bi-hemispheric epileptic activity. [ABSTRACT FROM AUTHOR]

The authors report a case of 18-year-old woman with partial complex seizures compatible with temporal epilepsy by semiology. Due to medical refractoriness, she was referred to pre-surgical evaluation. Initially, MRI showed no significant structural abnormality and superficial scalp EEG demonstrated epileptiform activity in the frontotemporal areas. Due to the lack of clear MRI abnormalities and the potential involvement of dominant mesial temporal structures by seizure semiology and non-invasive data, extra-operative invasive evaluation using stereo-electroencephalography (SEEG) methodology was indicated. Invasive monitoring demonstrated seizure onset in the left temporal pole with early spread to ipsilateral amygdala. Surgical treatment resulted in resection of the temporal pole and amygdala, with preservation of the remaining mesial temporal lobe structures. Intraoperatively, it was observed that multiple dural defects in the anterior middle temporal fossa with invagination of adjacent temporal pole parenchyma are compatible with temporal encephalocele. Patient remains seizure-free since surgery (12 months follow-up period) with preservation of neuropsychological functions. Although temporal pole resection plus amygdalohippocampectomy has been described as an adequate surgical approach in temporal encephalocele cases, we demonstrated the usefulness of the SEEG methodology in minimizing the volume of temporal lobe resection without compromising seizure and neuropsychological outcomes. The optimal results in this case and the review of the literature may suggest that in medically refractory epilepsies caused by temporal pole encephaloceles, preservation of the temporal lobe mesial structures should be attempted. [ABSTRACT FROM AUTHOR]

Introduction. Cricoarytenoid arthritis in rheumatoid arthritis (RA) is not infrequent and symptoms can range from mild, nonspecific laryngeal complains to acute upper airway obstruction. Case report. We report a case of bilateral vocal cord palsy due to cricoarytenoid joint (CAJ) involvement in a RA patient presenting with respiratory distress and stridor. The diagnosis was confirmed by flexible laryngoscopy. The patient responded well to systemic corticosteroids and did not require tracheostomy. There was no recurrence of the symptoms after corticosteroid dose tapering and the patient was discharged after 2 weeks. Conclusion. Cricoarytenoid arthritis should be suspected in RA patients presenting with acute airway compromise. Prompt recognition of this condition and appropriate management can be life-saving. [ABSTRACT FROM AUTHOR]

Background: Adrenal gland trauma is a rare condition that typically stems from blunt force trauma, and is associated with multiple organ injuries. Alternatively, isolated adrenal gland trauma is extremely rare, accounting for only 1.5 to 4% of all adrenal trauma cases. While isolated adrenal trauma is a mostly self-limiting condition, it is potentially life-threatening, representing a significant cause of bleeding, and/or hypotension due to adrenal insufficiency and adrenal crisis. Due to its rare occurrence, there are no reported guidelines for monitoring and observing isolated adrenal trauma.Case Presentation: Here we report on an isolated adrenal hemorrhage from a blunt trauma without associated injuries. A 53-year-old white man presented with abdominal pain after a high-speed motor vehicle accident. An initial evaluation revealed minimal abdominal pain and negative focused assessment with sonography for trauma examination; computed tomography imaging revealed a significant fluid collection consistent with adrenal hemorrhage. He was observed in our intensive care unit for 24 hours, and had stable hemoglobin and vital signs, after which he was discharged. At 1-month follow-up, he reported persistent intermittent abdominal pain, which was completely resolved by the 4-month follow-up.Conclusions: This case report demonstrates isolated adrenal gland injury resulting from significant blunt trauma to the abdomen. There are no current guidelines for monitoring isolated adrenal hemorrhage. Recognizing possible adrenal injury in blunt trauma cases is important due to potentially severe adrenal hemorrhage; therefore, we recommend follow-up with serial abdominal computed tomography until the resolution of hemorrhage and symptoms. [ABSTRACT FROM AUTHOR]

This article aims to review the concept of emotion dysregulation, focusing on issues related to its definition, meanings and role in psychiatric disorders. Articles on emotion dysregulation published until May 2016 were identified through electronic database searches. Although there is no agreement about the definition of emotion dysregulation, the following five overlapping, not mutually exclusive dimensions of emotion dysregulation were identified: decreased emotional awareness, inadequate emotional reactivity, intense experience and expression of emotions, emotional rigidity and cognitive reappraisal difficulty. These dimensions characterise a number of psychiatric disorders in various proportions, with borderline personality disorder and eating disorders seemingly more affected than other conditions. The present review contributes to the literature by identifying the key components of emotion dysregulation and by showing how these permeate various forms of psychopathology. It also makes suggestions for improving research endeavours. Better understanding of the various dimensions of emotion dysregulation will have implications for clinical practice. Future research needs to address emotion dysregulation in all its multifaceted complexity so that it becomes clearer what the concept encompasses. [ABSTRACT FROM AUTHOR]

Since 2000, various phase III randomized controlled trials (RCTs) have investigated the efficacy of rabbit antithymocyte globulin (ATG) in patients following allogeneic stem cell transplantation (allo-SCT). Comparisons of different ATG formulations are lacking, however. Our aim was to synthesize all published efficacy evidence to enable a comparison of all available formulations of rabbit ATG in the allo-SCT setting. We performed a systematic literature review to identify all available phase III RCT evidence. We searched the Cochrane Library, MEDLINE, MEDLINE In-Process, and the website www.ClinicalTrials.gov . In addition, a trial presented at the Annual Meeting of the American Society of Hematology 2016 was added to include the most recent evidence. We identified a total of 6 RCTs, including 2 formulations: anti–T lymphocyte globulin (ATLG; Grafalon, Neovii Biotech, Lexington, MA) and polyclonal globulin immunized with human thymocytes (Thymoglobulin [Thymo]; Genzyme-Sanofi, Cambridge, MA). The evidence was synthesized using a conventional network meta-analysis (NMA). The best treatment for preventing graft-versus-host disease (GVHD) was ATLG, which had a more favorable hazard ratio (HR) compared with standard treatment (chronic GVHD: HR, .42; 95% confidence interval [CI], .31 to .56; acute GVHD grade II-IV: HR, .54; 95% CI, .39 to .73; acute GVHD grade III-IV: HR, .50; 95% CI, .29 to .86), whereas both ATLG and Thymo were at least similarly effective in terms of transplantation-related mortality (TRM) (ATLG: HR, .90; 95% CI, .61 to 1.32; Thymo: HR, .90; 95% CI, .56 to 1.44). Thymo tended to be the better treatment option regarding overall survival (OS) (HR, .86; 95% CI, .59 to 1.26). Our NMA provides the first report of the relative efficacy of all available rabbit ATG formulations in patients undergoing allo-SCT. Until additional data from randomized head-to-head comparisons are available, based on the present analysis, ATLG seems to be the best option to prevent chronic and acute GVHD. Both formulations show similar efficacy in terms of TRM, whereas Thymo appears to be the better treatment option in terms of OS. [ABSTRACT FROM AUTHOR]

Acute dacryoadenitis with abscess formation has been rarely described. We describe four cases that resolved with incision and drainage. This includes a retrospective case series of four patients with radiologically confirmed lacrimal gland abscesses and a review of the reported cases in the literature. Computed tomography showed characteristic rim enhancing collections with central attenuation in all four cases. All patients presented with ptosis, upper eyelid erythema, and severe pain similar to scleritis. Injection of the conjunctiva and sclera was present in two patients, and a third patient presented with expression of purulent discharge onto the ocular surface upon palpation of the lacrimal gland. All patients were treated with intravenous antibiotics and underwent incision and drainage with subsequent improvement. All were monitored for 24 to 48 hours and discharged on oral antibiotics. There were no complications or recurrences. Lacrimal gland abscess formation is a rare complication of dacryoadenitis, and in our experience these patients respond well to incision and drainage in combination with systemic antibiotics. [ABSTRACT FROM PUBLISHER]

Exome sequencing is becoming widely popular and affordable, making it one of the most desirable methods for the identification of rare genetic variants for clinical diagnosis. Here, we report the clinical application of whole exome sequencing for the ultimate diagnosis of a ciliary chondrodysplasia case presented with an initial clinical diagnosis of Asphyxiating Thoracic Dystrophy (ATD, Jeune Syndrome). We have identified a novel homozygous missense mutation in WDR35 (c.206G > A), a gene previously associated with Sensenbrenner Syndrome, Ellis-van Creveld syndrome and Short-rib polydactyly syndrome type V. The genetic findings in this family led to the re-evaluation of the initial diagnosis and a differential diagnosis of Sensenbrenner Syndrome was made after cautious re-examination of the patient. Cell culture studies revealed normal subcellular localization of the mutant WDR35 protein in comparison to wildtype protein, pointing towards impaired protein-protein interaction and/or altered cell signaling pathways as a consequence of the mutated allele. This research study highlights the importance of including pathogenic variant identification in the diagnosis pipeline of ciliary chondrodysplasias, especially for clinically not fully defined phenotypes. [ABSTRACT FROM AUTHOR]

Despite being preventable, more than 15% of all cancer cases in Iran occur in the skin, making them the most commonly diagnosed malignancy in the country. The purpose of this study is to gain an insight into the current skin cancer related knowledge, attitudes, beliefs, and practices among the Iranian population. A systematic computer based literature search was conducted using databases for articles published through April 2017. Research studies included those that measured skin cancer or sun protection related knowledge, attitudes, beliefs, and behaviors in different Iranian population groups. Exclusion criteria for the articles included (1) irrelevant topics to the review article’s aim, (2) articles that focused on the treatment of skin cancers instead of prevention practices, and (3) similar studies conducted on populations not indigenous to Iran. A total of 25 articles that met the eligibility criteria were included in the review. Predominant data were collected via questionnaires. Skin cancer related knowledge varied from low to high across the studies. Moreover, there was a pattern of low perceived skin cancer susceptibility and severity. Overall, there was low usage of sun protection methods among the Iranian population. The findings of this study show that efforts to prevent skin cancer are needed. Education concerning the dangers of sun exposure as well as strategies used to prevent or lower the risk of developing skin cancer should be stressed. [ABSTRACT FROM AUTHOR]

In the majority of developed countries, it is observed that the time for maternity is being postponed to over the 30th and even 40th year of life. A significant number of cancers are diagnosed during reproductive age, often before the first pregnancy. A fertility preservation is an important issue in oncological treatment, where there is a need for balancing between radicality and the preservation of function of reproductive organs. The authors discuss the problem of ovarian dysfunction after oncological treatment and present the possible strategies for saving the reproductive function of ovaries, including both invasive and pharmacological approaches. [ABSTRACT FROM AUTHOR]

Ovarian hyperthecosis (OH) is characterized by the presence of abundant luteinized theca cells in ovaries that secret androgen. It typically presents as severe hyperandrogenism and/or virilization in postmenopausal woman. Here we describe a 66-year old woman with presentation of severe hirsutism, alopecia, clitoromegaly and laboratory finding of significantly elevated serum total testosterone concentration and hyperinsulinemia. Performed imaging studies revealed normal sized, homogeneous ovaries, signs of endometrial hypertrophy and normal adrenal glands. Due to severe hyperandrogenemia and signs of endometrial hypertrophy, the total abdominal hysterectomy with bilateral salpingo-oophorectomy has been performed. Pathological examination revealed OH and endometrial hyperplasia. Androgenic activity of ovarian stromal cells has been confirmed using alpha-inhibin histochemical staining. Postmenopausal hyperandrogenemia is a diagnostic and therapeutic challenge and the imaging studies often may be misleading and require careful and critical consideration. [ABSTRACT FROM AUTHOR]

Background: The 22q11.2 deletion syndrome affects multiple organ systems, and the neurological manifestations are an important aspect of this disorder. Many are aware of cardiac anomalies associated with this uncommon genetic disorder. However, the different types of seizures, electroencephalography (EEG), and brain magnetic resonance imaging (MRI) findings seen in this condition are not appreciated.Methods: Medical records of four patients with epilepsy due to 22q11.2 deletion syndrome were retrospectively reviewed for documentation of seizure types, EEG, and brain MRI findings. In addition, we also did a literature review of previously reported individuals with unprovoked seizures in this condition.Results: A review of all published cases including our patients reveals that focal epilepsy (39 of 88, 44%) is the most common type followed by genetic generalized epilepsy (24 of 88, 27%). Diffuse cerebral atrophy and polymicrogyria were the most frequent MRI findings.Conclusions: Patients with structural brain abnormalities, especially polymicrogyria and associated epilepsy should have a chromosomal microarray (CMA) performed to screen for the 22q11.2 deletion syndrome. Focal epilepsy and genetic generalized epilepsy are the most frequent epilepsy types reported in this condition. [ABSTRACT FROM AUTHOR]

Objectives To describe the clinical course, neuroimaging findings and functional outcome of idiopathic spinal cord infarction (SCI) in adolescents. Methods Retrospective and descriptive analyses of seven patients with idiopathic SCI and 50 additional cases from the literature were included. Data collected concerned clinical presentation, MRI findings, initial diagnosis, treatments and functional outcome at the last medical visit. Results Mean age at presentation was 13.2 years (range 13–15). All patients presented a sudden and painful acute myelopathy with <24 h time to maximal symptoms manifestation. A suspected trigger related to a minor effort was reported in 3/7 cases. Six patients presented with paraplegia, one with paraparesis. All had bladder dysfunction needing catheterization. Three patients had an initial misdiagnosis. Initial MRI was considered as normal in 2 cases. In the 5 other cases, T2-weighted-MR images showed hyperintensity within the thoracolumbar spinal cord, affecting mostly the anterior spinal artery territory. Evidence for associated spinal growth dystrophy were present in 6/7 cases. Mean follow-up time was 27.4 months (range 3–46): 2 patients recovered autonomous ambulation, 4 patients regained walking ability with aids and one child (the shortest follow-up) remained wheelchair-dependent. A neurogenic bladder was still reported in 6/7 children at the last visit. Complementary analyses with literature cases were consistent with the findings obtained in our cohort. Conclusion Idiopathic SCI typically occurs in adolescence with a rapid onset and painful acute myelopathy. The MRI shows a T2-hyperintense signal within the spinal cord and provides evidence for an ischemic mechanism. Etiology remains unclear in most cases even though some specific risk factors for this age must play an important role in the pathogenesis, such as mechanical constraints on the immature spine. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this study was to provide clinical outcomes data related to secondary intraocular lens (IOL) implantation for the correction of residual refractive error after cataract surgery. Patients and methods: A chart review was conducted to identify all eyes implanted with the monofocal spherical or toric AddOn® secondary IOL. Data were collated from charts where uncomplicated initial cataract surgery was completed. Measures of interest included the original IOL implanted, the postoperative refractive error (before secondary IOL implantation) and the associated corrected and uncorrected visual acuities (VAs). Postoperative data of interest included the residual refractive error, the best-corrected visual acuity (BCVA) and uncorrected visual acuity (UCVA). Results: Refractive and VA data from 1 week to 3 months post-surgery were available for 46 of 70 eyes implanted with a secondary IOL by one surgeon at one practice between 4/15 and 3/17. There was a statistically significant improvement in UCVA of about 2 lines after surgery (p0.01), with no change in BCVA (p=0.94). No eyes lost a line of BCVA. There was a statistically significant reduction in the absolute magnitude of the residual spherical equivalent refractive error (p0.01). In the 10 cases with a toric secondary IOL, there was a statistically significant reduction in refractive cylinder (p0.01). Conclusion: The secondary IOL studied here appears to be a viable surgical option to correct residual refractive error after primary IOL implantation. [ABSTRACT FROM AUTHOR]

Purpose: The treatment of choice for duodenal neuroendocrine tumors (NETs) ranges from endoscopic resection and local excision to pancreaticoduodenectomy. The aim of this study was to investigate the optimal treatment for this tumor. Methods: We retrospectively analyzed the clinicopathological data of 14 patients with NETs in the second portion of the duodenum who underwent surgery in our hospital from 2000 to 2015. The duodenal NETs were classified as either ampullary or non-ampullary. Additionally, a systematic review and pooled analysis was conducted. Results: Among eight patients with ampullary NETs and six patients with non-ampullary NETs, seven and three patients underwent pancreaticoduodenectomy and one and three patients underwent local resection, respectively. The maximum tumor diameter were 11-30 mm in ampullary and 10-100 mm in non-ampullary NETs, respectively. In patients with ampullary NETs, lymph node metastases were suspected in only three cases preoperatively, but five patients actually had regional nodal metastases. Among patients with non-ampullary NETs, lymph node metastases were suspected in none preoperatively, but three of the four patients who underwent lymph node dissection had regional nodal metastases. According to a pooled analysis of 1245 patients in 88 studies, even small tumors confined to the submucosal layer and G1 tumors-ampullary and non-ampullary-have been associated with lymph node metastases. In patients with non-ampullary NETs and lymph node metastasis, 10-year recurrence-free survival rate was 51% for patients who underwent pancreaticoduodenectomy ( n = 19) and 53% for patients who underwent partial duodenal resection ( n = 9), respectively ( p = 0.960). Conclusion: Lymph node metastases were common in association with both ampullary and non-ampullary NETs, and it was difficult to radiologically diagnose metastases. Additionally, there were no clinicopathological factors that could reliably predict the absence of lymph node metastases preoperatively. Therefore, to maximize the ability to achieve a curative resection, pancreaticoduodenectomy is considered appropriate in well-conditioned patients with NETs in the second portion of the duodenum. However, to further clarify the impact of lymph node dissection on survival after duodenal NET resection, a multi-institutional study with a large number of patients, thorough examination of lymph node metastasis, and a long observation period is warranted. [ABSTRACT FROM AUTHOR]

Purpose: We aim to provide a comprehensive overview of the role of the vagina as a route for drug delivery and absorption, with a particular focus on the use of vaginal hormonal compounds for the treatment of deep infiltrating symptomatic endometriosis.Methods: A MEDLINE search through PubMed was performed to identify all published studies in English language on vaginal hormonal treatments for symptomatic endometriosis.Results: Main advantages of the vaginal route include avoidance of the hepatic-first pass metabolic effect, the possibility of using lower therapeutic dosages, and the reduction of side effects compared with the oral administration. Studies on endometriosis treatment mainly focused on the use of vaginal danazol (n = 6) and the contraceptive vaginal ring (n = 2). One pilot study evaluated the efficacy of vaginal anastrozole in women with rectovaginal endometriosis. Most investigations evaluated the vaginal use of hormonal agents in women with deep infiltrating endometriosis/rectovaginal endometriosis. Overall, a substantial amelioration of pelvic pain symptoms associated with endometriosis was observed, particularly of dysmenorrhea. A significant reduction in rectovaginal endometriotic nodule dimensions measured at ultrasound examination was detected by some but not all authors.Conclusions: The vaginal route represents a scarcely explored modality for drug administration. High local hormonal concentrations might achieve a greater effect on endometriotic lesions compared with alternative routes. Future studies should focus on the use of the vagina for delivering target therapies particularly in patients with deeply infiltrating rectovaginal lesions. [ABSTRACT FROM AUTHOR]

A growing body of evidence suggests a correlation between schizophrenia and exposure to infectious agents. The majority of studied cases concerns the infection caused by T. gondii, an obligatory intracellular parasite that infects about 1/3 of the entire human population, according to the available data. The acute stage of the disease, predominantly short-lived and transient, transforms into the latent and chronic phase in which the parasite localizes within tissue cysts, mainly in the central nervous system. The chronic toxoplasmosis, primarily regarded as benign and asymptomatic, might be responsible, in light of current scientific evidence, for a vast array of neuropsychiatric symptoms. Numerous epidemiological case-control studies show a higher prevalence of T. gondii infestation in individuals with various psychiatric and behavior disorders, including schizophrenia. This paper tends to review the relevant studies that demonstrate links between schizophrenia and T. gondii infestation, of which the latter may be acquired in different developmental phases. Apart from epidemiological correlation studies, some papers on other associations were also presented, describing putative patophysiological mechanisms that might be at least partly responsible for chronic infection-induced neuromediator disturbances, together with morphological and functional alterations, e.g., low-grade neuroinflammation, which are likely to induce psychopathological symptoms. Toxoplasmosis is only one of the putative infectious agents that derange correct brain growth and differentiation, alongside genetic and environmental factors. All of them may lead eventually to schizophrenia. A better knowledge of infection mechanisms and its influence on neurobiochemical and neuropathological pathways may enable more efficient therapy and the prevention of this devastating disease. [ABSTRACT FROM AUTHOR]

Standard management of intra-abdominal pediatric solid tumors requires complete resection. However, tumors with multiple organ and vascular involvement present a unique surgical challenge. We conducted a retrospective chart review of four patients, aged 2-14 years, undergoing MVT for intra-abdominal tumors with significant involvement of the visceral arteries and/or portomesenteric venous system at our institution. Indications for MVT included hepatocellular carcinoma, inflammatory myofibroblastic tumor, and two cases of hepatoblastoma. Grafts included liver, stomach, small bowel, and pancreas in all patients, with two patients also receiving spleens, and one, a partial esophageal transplant. Median hospital stay was 80 days. Postoperative complications included reoperation for abdominal hematoma and bowel obstruction, steroid responsive intestinal rejection, wound dehiscence, fungemia, seizures, and chyle leak with pleural effusion. One patient developed Epstein-Barr virus-associated complications which responded well to treatment. On follow-up (range 2.8-7.8 years), all patients have satisfactory graft function and no evidence of recurrent disease. MVT is an effective means of achieving complete gross resection of intra-abdominal malignancies in patients with multiple organ and vascular involvement. [ABSTRACT FROM AUTHOR]

Background Intermaxillary fixation (IMF) techniques are commonly used in mandibular fracture treatment to reduce bone fragments and re-establish normal occlusion. However, non-IMF reduction techniques such as repositioning forceps may be preferable due to their quick yet adequate reduction. The purpose of this paper is to assess which non-IMF reduction techniques and reduction forceps are available for fracture reduction in the mandible. Methods A systematic search was performed in the databases of Pubmed and EMBASE. The search was updated until February 2016 and no initial date and language preference was set. Results 14 articles were selected for this review, among them ten articles related to reduction forceps and four articles describing other techniques. Thus, modification and design of reduction forceps and other reduction techniques are qualitatively described. Conclusion Few designs of repositioning forceps have been proposed in the literature. Quick and adequate reduction of fractures seems possible with non-IMF techniques resulting in anatomic repositioning and shorter operation time, especially in cases with good interfragmentary stability. Further development and clinical testing of reduction forceps is necessary to establish their future role in maxillofacial fracture treatment. [ABSTRACT FROM AUTHOR]

Background Superior vena cava (SVC) syndrome is a major complication that occurs when a growing lung malignancy compresses the SVC extrinsically. Current treatment options include radiotherapy or chemotherapy to shrink the tumor or endovascular stenting of the SVC to restore flow. Herein, we report a case series treated in a single institution to demonstrate the safety, effectiveness, and outcomes of salvage and primary stenting for malignant SVC obstruction. Methods A total of 12 male patients with malignant superior vena cava obstruction caused by lung cancer underwent SVC stenting from October 2009 to May 2015. Data were reviewed retrospectively, including demographic and clinical characteristics, procedural details, and outcomes. Results Seven patients had received radiotherapy prior to SVC stenting, while the other five patients received stenting as first-line therapy for SVC syndrome. Only one patient experienced initial symptomatic improvement after radiotherapy, and symptoms of SVC syndrome recurred one year later. Wallstents ® (Boston Scientific, Natick MA, USA) were used in all patients. Preoperatively, the mean narrowest SVC diameter measured by CT was 2.16 mm (0–5.5 mm). Technical success was achieved in all patients without complications such as pulmonary embolism, rupture or bleeding. Postoperative mean narrowest SVC diameter measured by CT during follow-up was 11.17 mm (8–13.5 mm). Symptoms of SVC syndrome such as arm and face swelling and dyspnea improved within 1–5 days in all patients. After median follow-up duration of 11.5 months, only one patient presented recurrent SVC syndrome due to in-stent thrombosis two months after stenting. Conclusion Salvage SVC stenting remains a safe and effective treatment for patients with SVC obstruction after failure of radiotherapy and chemotherapy. Primary stenting may be considered at initial presentation of SVC syndrome to improve patients' quality of life. [ABSTRACT FROM AUTHOR]

The surgical ciliated cyst, which is also known with the terms 'post-operative maxillary cyst (PMC)' or 'paranasal cyst', was originally reported by Kubo in 1927 and well-described in Japanese literature since the 1980s. PMC is a locally aggressive lesion that appears as a long-term delayed complication, arising from years to decades, after surgery or trauma in the maxillary sinus region such as midfacial osteotomies and fractures or orthognatic surgery. Although this cyst is rarely reported in Western population, it constitutes 20% of oral cysts in Japan. PMC usually appears as a swelling that may cause expansion of vestibular, palatine or both bone cortical plates, and pain in the adjacent facial area of buccal vertical releasing incisions; formation of fistulas has also been reported. In case of significant cyst growth, other symptoms may include nasal obstruction, rhinorrhea, dysomia, exophthalmos and ocular displacement. We report the only case of a surgical ciliated cyst that developed in a Caucasian patient 12 years after a Le Fort I maxillary advancement osteotomy. [ABSTRACT FROM AUTHOR]

Endometriosis is an estrogen-dependent chronic inflammatory condition that affects women in their reproductive period causing infertility and pelvic pain. The disease, especially at the ovarian site has been shown to have a detrimental impact on ovarian physiology. Indeed, sonographic and histologic data tend to support the idea that ovarian follicles of endometriosis patients are decreased in number and more atretic. Moreover, the local intrafollicular environment of patients affected is characterized by alterations of the granulosa cell compartment including reduced P450 aromatase expression and increased intracellular reactive oxygen species generation. However, no comprehensive evaluation of the literature addressing the effect of endometriosis on oocyte quality from both a clinical and a biological perspective has so far been conducted. Based on this systematic review of the literature, oocytes retrieved from women affected by endometriosis are more likely to fail in vitro maturation and to show altered morphology and lower cytoplasmic mitochondrial content compared to women with other causes of infertility. Results from meta-analyses addressing IVF outcomes in women affected would indicate that a reduction in the number of mature oocytes retrieved is associated with endometriosis while a reduction in fertilization rates is more likely to be associated with minimal/mild rather than with moderate/severe disease. However, evidence in this field is still far to be conclusive, especially with regards to the effects of different stages of the disease and to the impact of patients' previous medical/surgical treatment(s). [ABSTRACT FROM AUTHOR]

Synaptic dysfunction is thought to play important roles in the pathophysiology of many neurological diseases, including Alzheimer's disease, Parkinson's disease, and schizophrenia. Over the past few decades, there have been systematic efforts to collect postmortem brain tissues via autopsies, leading to the establishment of dozens of human brain banks around the world. From cryopreserved human brain tissues, it is possible to isolate detached-and-resealed synaptic terminals termed synaptosomes, which remain metabolically and enzymatically active. Synaptosomes have become important model systems for studying human synaptic functions, being much more accessible than ex vivo brain slices or primary neuronal cultures. Here we review recent advances in the establishment of human brain banks, the isolation of synaptosomes, their biological activities, and various analytical techniques for investigating their biochemical and ultrastructural properties. There are unique insights to be gained by directly examining human synaptosomes, which cannot be substituted by animal models. We will also discuss how human synaptosome research has contributed to better understanding of neurological disorders, especially Alzheimer's disease. [ABSTRACT FROM AUTHOR]

Background: The Behavioural and Cognitive Psychotherapy (BCP) Journal is the main academic publication of the British Association for Behavioural and Cognitive Psychotherapies. It publishes empirical studies, reviews and theoretical papers, brief reports and single cases. Aim: To describe the main areas of interest and focus in BCP papers. Method: All the papers published in BCP from 2000 to July 2016 were analysed. A categorization procedure was followed with 813 contributions in six main areas: main author country; main author gender; kind of contribution; kind of therapeutic approaches; kind of samples; kind of focus/topic. Results: Although the journal's scope is international, first authors tend to come from English-speaking countries. Since 2009, females contribute more than males. Empirical studies surpass theoretical studies and reviews, while the main therapy denomination is cognitive behavioural therapy. Variability of samples is wide, and ranges from analogues to main disorders. Finally, the main focus of papers is to study change and psychopathology, therapists’ training and improvement of CBT. Conclusion: Given the total number of categorized papers, it can be tentatively assumed that they could exemplify some main areas of interest and evolvement in the cognitive and behavioural field. [ABSTRACT FROM AUTHOR]

As one of the systemic autoinflammatory diseases (SAIDs), the nucleotide-binding oligomerization domain-like receptor protein (NLRP)12 autoinflammatory disease (NLRP12-AD) is an autosomal dominant disorder associated with NLRP12 mutation. SAIDs have been hardly reported in the Chinese population, and NLRP12-AD has been reported only in Caucasians. We report the first case series of NLRP12-AD in the Chinese population coupled with literature review. Three Han Chinese adult patients with clinical phenotype suggestive of NLRP12-AD carrying NLRP12 variants were treated by the authors in 2015. Their phenotype and genotype were carefully studied. A PubMed search for SAIDs was conducted between January, 1990 and January, 2016, and we focused on NLRP12-AD. All three adult patients developed periodic disease in adulthood. They presented with recurrent fever ( n = 3), polyarthralgia ( n = 3), myalgia ( n = 3), urticaria ( n = 2), lymphadenopathy ( n = 2), and erythema nodosa ( n = 1). All patients carry the NLRP12 mutation F402L. Based upon our analysis of a total of 26 patients with NLRP12-AD in the literature, both familial and sporadic cases were equally reported and late-onset cases accounted for 28 %. NLRP12-AD patients typically present with periodic fever, urticaria-like rash, arthralgia/arthritis, myalgia, and lymphadenopathy. Genotyping identifies the NLRP12 gene mutations, notably F402L (55 %). Relative to the literature reports, our patients had the similar phenotypic and genotypic features. Patients with NLRP12-AD usually respond to glucocorticoid therapy. Our report is the first to confirm the presence of NLRP12-AD in the Chinese population. It highlights the importance of screening NLRP12 in patients with unexplained periodic fever syndrome. [ABSTRACT FROM AUTHOR]

Tumor necrosis factor receptor-associated periodic syndrome (TRAPS), caused by mutations in the TNFRSF1A gene, is the most frequent autosomal dominant autonflammatory disease displaying a relevant risk of reactive AA amyloidosis, if left untreated. Our report deals with one adult with TRAPS complicated by amyloidosis-related renal failure, treated with the recombinant human interleukin-1 receptor antagonist anakinra at a higher than conventional dosage. This treatment did not present any adverse event and led remarkably to the disappearance of all TRAPS-related manifestations and prompt decrease of laboratory abnormalities, including proteinuria. A review of the medical literature has been also considered to evaluate efficacy and safety of interleukin-1 inhibition in patients with TRAPS. [ABSTRACT FROM AUTHOR]

Verrucae are benign epithelial proliferations, characteristically 1-20 mm in diameter, caused by human papilloma virus (HPV) infection occurring on the skin and mucosa (Photomed Laser Surg 33(6):338-42, 2015; Lasers Med Sci 29(3):1111-6, 2014). Prevalence of verrucae is 5-20% in children and young adults with peak incidence reported during teenage years (Lasers Med Sci 29(3):1111-6, 2014; J Am Acad Dermatol 22(4):547-66, 1990; J Korean Med Sci 24(5):889-93, 2009). Patients often express significant displeasure with quality of life due to this cosmetic insecurity, as well as functional problems and physical discomfort when they occur on palms of hands and soles of feet. Traditional therapeutic options for warts, such as topical salicyclic acid, topical imiquimod, bleomycin injections, cryotherapy, surgical excision, and electrocautery, have proven somewhat effective but often lead to high recurrence rates or scarring (Photomed Laser Surg 33(6):338-42, 2015). Laser therapy offers an alternative solution by employing selective tissue destruction with minimal risks. We performed a broad literature search in PubMed to obtain all available published articles that studied the treatment of verrucae on the skin with 1064-nm neodymium-doped yttrium aluminum garnet laser. This laser is specifically suited for verruca treatment due to its deeply penetrating 1064-nm wavelength and relatively low risk of pigmentation changes in dark skin types (Photomed Laser Surg 33(6):338-42, 2015). Laser therapy is effective in the treatment of verrucae and has enabled clinicians to provide direct, targeted treatment of warts. [ABSTRACT FROM AUTHOR]

Objective: To analyse the scientific literature regarding the evaluation of body image in children through an integrative literature review. Data source: An intersection of the keywords "body image" AND "child" was conducted in Scopus, Medline and Virtual Health Library (BVS - Biblioteca Virtual de Saúde) databases. The electronic search was based on studies published from January 2013 to January 2016, in order to verify the most current investigations on the subject. Exclusion criteria were: articles in duplicate; no available summaries; not empirical; not assessing any component of body image; the sample did not consider the target age of this research (0 to 12 years old) and/or considered clinical populations; besides articles not fully available. Data synthesis: 7,681 references were identified, and, after the exclusion criteria were implemented, 33 studies were analysed. Results showed that the perceptual and attitudinal dimensions focusing on body dissatisfaction were explored, mainly evaluated by silhouette scales. Intervention programs were developed internationally to prevent negative body image in children. Conclusions: The studies included in this review evaluated specific aspects of body image in children, especially body perception and body dissatisfaction. The creation of specific tools for children to evaluate body image is recommended to promote the psychosocial well being of individuals throughout human development. [ABSTRACT FROM AUTHOR]

Background: Dementia is a complex disorder characterized by poor outcomes for the patients and high costs of care. After decades of research little is known about its mechanisms. Having prognostic estimates about dementia can help researchers, patients and public entities in dealing with this disorder. Thus, health data, machine learning and microsimulation techniques could be employed in developing prognostic estimates for dementia. Objective: The goal of this paper is to present evidence on the state of the art of studies investigating and the prognosis of dementia using machine learning and microsimulation techniques. Method: To achieve our goal we carried out a systematic literature review, in which three large databases—Pubmed, Socups and Web of Science were searched to select studies that employed machine learning or microsimulation techniques for the prognosis of dementia. A single backward snowballing was done to identify further studies. A quality checklist was also employed to assess the quality of the evidence presented by the selected studies, and low quality studies were removed. Finally, data from the final set of studies were extracted in summary tables. Results: In total 37 papers were included. The data summary results showed that the current research is focused on the investigation of the patients with mild cognitive impairment that will evolve to Alzheimer’s disease, using machine learning techniques. Microsimulation studies were concerned with cost estimation and had a populational focus. Neuroimaging was the most commonly used variable. Conclusions: Prediction of conversion from MCI to AD is the dominant theme in the selected studies. Most studies used ML techniques on Neuroimaging data. Only a few data sources have been recruited by most studies and the ADNI database is the one most commonly used. Only two studies have investigated the prediction of epidemiological aspects of Dementia using either ML or MS techniques. Finally, care should be taken when interpreting the reported accuracy of ML techniques, given studies’ different contexts. [ABSTRACT FROM AUTHOR]

Background. Glomus tumors are rare mesenchymal tumors originating from glomus bodies in the skin. Glomus tumors of the kidney are rare tumors and only a few cases have been reported in the medical literature. An extensive search revealed a very limited number of primary renal glomus tumors. Although most of these cases were benign in nature, including a case with uncertain diagnosis of malignant potential, two were malignant. Case Report. We present a unique case of a 57-year-old male patient with an incidentally discovered 2 cm left renal mass. Histopathology examination and immunohistochemical studies confirm the diagnosis of glomangioma (a form of glomus tumor). The patient was followed for one year after partial nephrectomy and showed a benign course without any evidence of local recurrence or metastasis. Conclusion. To the best of our knowledge, this is the 16th case of primary benign renal glomus tumors. Primary renal glomus tumors are rare and may mimic other mesenchymal renal neoplasms radiologically. Proper investigation (including histopathological analysis and immunohistochemical staining) of kidney tumors is essential to make the diagnosis of glomus tumors, which usually show a benign clinical course following resection. [ABSTRACT FROM AUTHOR]

Awareness of rare etiologies for implant failure is becoming increasingly important. In addition to the overall increase in joint arthroplasties, revision surgeries are projected to increase dramatically in the coming years, with volume increasing up to seven-fold between 2005 and 2030. The literature regarding the relationship between metal allergy and implant failure is controversial. It has proven difficult to determine whether sensitization is a cause or a consequence of implant failure. Testing patients with functional implants is not a clinically useful approach, as the rate of hypersensitivity is higher in implant recipients than in the general population, regardless of the status of the implant. As a result of the ineffectiveness of preoperative patch testing for predicting adverse outcomes, as well as the high cost of implementing such patch testing as standard procedure, most orthopedists and dermatologists agree that an alternative prosthesis should only be considered for patients with a history of allergy to a metal in the standard implant. In patients with a failed implant requiring revision surgery, hypersensitivity to an implant component should be considered in the differential diagnosis. Because a metal allergy to implant components is currently not commonly considered in the differential for joint failure in the orthopedic literature, there should be improved communication and collaboration between orthopedists and dermatologists when evaluating joint replacement patients with a presentation suggestive of allergy. [ABSTRACT FROM AUTHOR]

Sepsis is an acute systemic inflammatory disease. Glucocorticoids (GCs), which function by binding to the GC receptor GR have very powerful anti-inflammatory activities, yet they are hardly useful in sepsis. We can thus consider sepsis as a GC resistant disease. We here review the literature which has investigated this GC resistance, and summarize the mechanisms of GC resistance that have been observed in other diseases and in experimental models. We also discuss the importance of GC resistance in sepsis, in terms of the contribution of this phenomenon to the pathogenesis of sepsis. [ABSTRACT FROM AUTHOR]

Objective Cervical radiculopathy secondary to compression from vertebral artery (VA) tortuosity is a rare entity. We describe successful transposition through an anterolateral approach of tortuous VA loops causing cervical radiculopathy. Methods Two patients with cervical radiculopathy (first case at C5-6 and second case at C3-4) secondary to anomalous VA loop compression underwent anterolateral approaches to the cervical spine for decompression and VA transposition. The anterior transverse foramina were drilled to unroof the VA loop, which was dissected free from the exiting nerve root. Results In both cases, the affected cervical nerve root was successfully decompressed with both radiographic and clinical improvements in radiculopathy symptoms. We found 8 other cases of VA transposition via either an anterolateral approach or a posterolateral approach described in the literature. Our second case of anterolateral VA transposition at the C3-4 level is the first case at this level and the highest level reported in the literature. Conclusions Decompression using an anterolateral approach with direct microvascular transposition of the VA is a safe and effective treatment of this pathology and addresses the cause of radiculopathy more directly than the posterolateral approach. [ABSTRACT FROM AUTHOR]

Background Gait analysis represents one of the newest methodologies used in the clinical evaluation of patients with cervical myelopathy (CM). Objective To describe the role of gait analysis in the clinical evaluation of patients with CM, as well as its potential role in the evaluation of the functional outcome of any surgical intervention. Methods A literature review was performed in the PubMed, OVID, and Google Scholar medical databases, from January 1995 to August 2016, using the terms “analysis,” “anterior,” “cervical myelopathy,” “gait,” “posterior,” and “surgery.” Clinical series comparing the gait patterns of patients with CM with healthy controls, as well as series evaluating gait and walk changes before and after surgical decompression, were reviewed. Case studies were excluded. Results Nine prospective and 3 retrospective studies were found. Most of the retrieved studies showed the presence of characteristic, abnormal gait patterns among patients with CM, consisting of decreased gait speed, cadence, step length, stride length, and single-limb support time. In addition, patients with CM routinely present increased step and stride time, double-limb support time, and step width, and they have altered knee and ankle joint range of motion, compared with healthy controls. Moreover, gait and walk analysis may provide accurate functional assessment of the functional outcome of patients with CM undergoing surgical decompression. Conclusions Gait analysis may well be a valuable and objective tool along with other parameters in the evaluation of functionality in patients with CM, as well as in the assessment of the outcome of any surgical intervention in these patients. [ABSTRACT FROM AUTHOR]

People with epilepsy ( PWE) have a higher risk of mortality in comparison with the general population. This in part reflects intrinsic factors or associated comorbidities, but poor adherence to anti-epileptic drugs (AED) has also been shown to contribute to increased risk of death and increased utilization of unscheduled care. The aim of this review was to determine the prevalence of non-adherence to AED in PWE, evaluate whether specific clinical and demographic features can allow clinicians to identify those at highest risk and identify the methods and techniques that can be used to improve adherence in clinical settings. We identified relevant studies for the prevalence of medication non-adherence in PWE by searching MEDLINE (1946-7 Dec 2015), EMBASE (1947-7 Dec 2015) and Cochrane Library (1946-7 Dec 2015) as per predefined inclusion and exclusion criteria. We included 17 research studies from our review of the medical literature to determine the prevalence of medication non-adherence in epilepsy. The prevalence of significant medication non-adherence in epilepsy has been reported to vary between 26% and 79%. This variation partly reflects the differences in defining what clinically significant medication adherence is, the methods used to estimate the scale of the problem and the underlying population heterogeneity. A number of clinical and demographic features have been associated with poor adherence allowing clinicians to identify those at greatest risk. Educating patients and their carers about the risks associated with poor adherence, certain behavioural interventions and simplifying their drug regimens have been shown to improve adherence. [ABSTRACT FROM AUTHOR]

Posttraumatic growth after burn is a relatively new area of study with only a small number of studies that have examined this phenomenon. It is important to understand the presentation of posttraumatic growth and coping in burn survivors, how it changes over time and the components which influence growth so that we can understand how to promote posttraumatic growth in burn survivors. The aim of this review was to assess these three parameters. Studies were identified through multiple databases with specific search terms to identify posttraumatic growth after burn. From the 813 articles found, 57 were identified as potentially useful, and 8 as eligible for review; three qualitative, one mixed methods, two quantitative, one discussion paper and part of a review which assessed all psychosocial outcomes. Growth presented as realising personal strength, reprioritising, spirituality, humanity, changed relationships, and compassion and altruism. Styles of coping included feelings of gratefulness and downward comparison, humour and planning. Suddenness of the event, and the severity and location of injury might affect the amount of growth experienced. Overall function, quality of life, social support and optimism, hope and new opportunities are influences on growth after burn, all of which have the potential for improvement through targeted intervention strategies. Further research is indicated in many areas related to growth, intervention and measurement. [ABSTRACT FROM AUTHOR]

Systemic pseudohypoaldosteronism (PHA) type I is a rare genetic disorder resulting from mutations in the subunits of the epithelial sodium channel that manifests as severe salt wasting, hyperkalemia, and metabolic acidosis in infancy. In this article we report a patient with systemic PHA type I presenting with severe dehydration due to salt wasting at 6 days of life. She was found to have a known mutation in the SCNN1A gene and subsequently required treatment with sodium supplementation. We also review the clinical presentation, differential diagnosis, and treatment of systemic PHA type I and summarize data from 27 cases with follow-up data. [ABSTRACT FROM AUTHOR]

The incidence of osteomyelitis constantly declines. While the disease most commonly affects the long bones, involvement of the patella is rarely seen. Due to this rarity and the variable clinical presentation, diagnosis is often delayed. The present case report describes a 10-year-old female patient with a delayed diagnosis of patella osteomyelitis. The diagnostic procedures and the treatment regimen are described. Additionally, a detailed literature review of the available publications reporting osteomyelitis of the patella in children is presented. [ABSTRACT FROM AUTHOR]

We present an unusual case of shunt nephritis in a 39-year-old male who presented 21 years after placement of a ventriculoperitoneal (VP) shunt. He complained of fevers, headaches, dizziness, and urticarial plaques on arms, trunks, and legs and was found to have anemia, low complement levels, elevated serum creatinine, proteinuria, and new onset microhematuria. Blood and urine cultures were negative. Renal biopsy showed features of acute tubulointerstitial nephritis attributed to vancomycin use. Glomeruli showed increased mesangial hypercellularity and segmental endocapillary proliferation. Immunofluorescence showed focal IgM and C3 staining. Electron microscopy revealed small subendothelial electron-dense deposits. Symptoms and renal insufficiency appeared to improve with antibiotic therapy. He was discharged and readmitted 2 months later with similar presentation. CSF grew Propionibacterium acnes and shunt hardware grew coagulase-negative Staphylococcus. He completed an intravenous antibiotic course and was discharged. On 1-month follow-up, skin lesions persisted but he was otherwise asymptomatic. Follow-up labs showed significant improvement. We did a brief systematic review of the literature on shunt nephritis and report our findings on 79 individual cases. In this review, we comment on the presentation, lab findings, pathological features, and management of this rare, potentially fatal, but curable disease entity. [ABSTRACT FROM AUTHOR]

Laryngeal fractures occur mainly in the context of cervical trauma, hanging, or strangulation. Nontraumatic laryngeal fractures are rare and there are few reports in the literature. We present two cases of nontraumatic laryngeal fractures evaluated in our service. [ABSTRACT FROM AUTHOR]

Silver nanoparticles (AgNP) are incorporated into medical devices for their anti-microbial characteristics. The potential exposure and toxicity of AgNPs is unknown due to varying physicochemical particle properties and lack of toxicological data. The aim of this safety assessment is to derive a provisional tolerable intake (pTI) value for AgNPs released from blood-contacting medical devices. A literature review of in vivo studies investigating critical health effects induced from intravenous ( i. v.) exposure to AgNPs was evaluated by the Annapolis Accords principles and Toxicological Data Reliability Assessment Tool (ToxRTool). The point of departure (POD) was based on an i. v. 28-day repeated AgNP (20 nm) dose toxicity study reporting an increase in relative spleen weight in rats with a 5% lower confidence bound of the benchmark dose (BMDL 05 ) of 0.14 mg/kg bw/day. The POD was extrapolated to humans by a modifying factor of 1,000 to account for intraspecies variability, interspecies differences and lack of long-term toxicity data. The pTI for long-term i. v. exposure to 20 nm AgNPs released from blood-contacting medical devices was 0.14 μg/kg bw/day. This pTI may not be appropriate for nanoparticles of other physicochemical properties or routes of administration. The methodology is appropriate for deriving pTIs for nanoparticles in general. [ABSTRACT FROM AUTHOR]

Primary hepatic angiosarcoma is a rare type of tumor with a poor prognosis. To the best of our knowledge, curative surgery of a metastatic gastrointestinal angiosarcoma from a hepatic angiosarcoma has not been reported previously. In the present report, a case of colonic metastasis from a primary hepatic angiosarcoma is discussed. A rapidly growing mass was identified in the liver of an 84-year-old Japanese male who underwent a subsegmentectomy of the liver. Microscopic examination determined that the mass was an angiosarcoma composed of sheets of spindle cells. Immunohistochemical studies confirmed the diagnosis with positive CD31 staining, which indicated the endothelial nature of the malignancy. A total of 14 months following surgery, the patient did not exhibit any symptoms; however, follow-up positron emission tomography and computed tomography images revealed a mass in the cecum. The patient underwent an ileocolectomy, and the microscopic and immunohistochemical findings indicated that the mass was a metastasized colorectal angiosarcoma. At a 4-year post-surgery follow-up appointment the patient was alive with no evidence of recurrence or metastasis. [ABSTRACT FROM AUTHOR]

The authors present the case of a patient that demonstrates resolution of delayed onset hypoglossal nerve palsy (HNP) subsequent to occipital condyle fracture following a motor vehicle accident. Decompression of the hypoglossal nerve and craniocervical fixation led to satisfactory long-term (>5 years) outcome. There is a scarcity of literature in recognizing HNPs following trauma and a lack of pathophysiological understanding to both a delayed presentation and to resolution versus persistence. This is the first report demonstrating long-term resolution of hypoglossal nerve injury following trauma to the craniocervical junction. [ABSTRACT FROM AUTHOR]