Hypophosphatasia (HPP) is a rare, inherited metabolic bone disorder characterized by low serum alkaline phosphatase activity and impaired bone mineralization. Clinical manifestations and severity of symptoms vary widely in HPP, ranging from in utero death to isolated dental manifestations in adults. Treatment with enzyme replacement therapy has been reported to improve outcomes in perinatal, infantile, and childhood forms of HPP. Here, we present a case of a boy with poor linear growth, mild limb bowing, and radiographic rickets who was diagnosed with HPP before 6 months of age. Treatment with enzyme replacement therapy was initiated at 7 months of age, after which significant improvements in radiographic findings and linear growth were demonstrated. This case highlights several important challenges in the diagnosis, classification, and management of HPP.
https://ift.tt/2GDJGCD
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- Brief Clinical Report: Hypophosphatasia—Diagnostic...
- A Rare Case of Lemierre-Like Syndrome: A Case Repo...
- Infectious Aortitis: A Life-Threatening Endovascul...
- A Large Grade 5 Mobile Aortic Arch Atheromatous Pl...
- Multiple roles of lymphatic vessels in tumor progr...
- Next generation natural killer cells for cancer im...
- The safety and risk factors of revision adenoidect...
- Multimodality Surgical Approach in Management of L...
- Spinal Accessory Nerve Duplication: A Case Report ...
- Evaluation of the effects of occlusal splint, trig...
- A Multimodal analysis using flowmeter analysis, la...
- Trajectory-guided biopsy of orbital tumor - Techno...
- Adverse reactions to tattoos in the general popula...
- Safety and Efficacy of a Halobetasol/Tazarotene Fi...
- Evaluation of the effects of occlusal splint, trig...
- Implant stability using piezoelectric bone surgery...
- Implant stability using piezoelectric bone surgery...
- An audiological evaluation of syndromic and non-sy...
- Surgical timing for bilateral simultaneous cochlea...
- Editorial board
- Actinomycose révélée par une ulcération du palais ...
- Hydrochlorothiazide et cancers cutanés
- Thyroid Cartilage Window Approach to Extract a For...
- Soft Palate Pleomorphic Adenoma of a Minor Salivar...
- Correction to: Esophagus perforation and myocardia...
- Coronary artery fistula with associated Takotsubo ...
- Induction of a central memory and stem cell memory...
- Stability of allergens
- Nebulized lidocaine ameliorates allergic airway in...
- New insights into the structure of the MHC class I...
- Pre-diagnostic dynamic HPV16 IgG seropositivity an...
- Osteochondroma of the Zygomatic Arch: A Case Repor...
- Assessing the Quality of Life in Patients with Den...
- Fracture healing and bone remodeling with human st...
- Does sphenopalatine ganglion block improve pain co...
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Σάββατο 31 Μαρτίου 2018
Brief Clinical Report: Hypophosphatasia—Diagnostic Considerations and Treatment Outcomes in an Infant
A Rare Case of Lemierre-Like Syndrome: A Case Report and Literature Review
Lemierre's syndrome (LS) is a serious rare complication of oropharyngeal infections. It is characterized by thrombosis of internal jugular vein that rapidly progresses into sepsis and is typically caused by anaerobes. Most of the reported cases have been linked to Fusobacterium necrophorum; however, there are a handful of reported cases due to aerobes. It is primarily the disease of healthy young adults and can present in school-aged children. The early recognition and treatment of this complication results in resolution of the illness; nevertheless, there have been some concerns about chronic venous insufficiency as a long-term complication. We report a case of a 6-year-old boy, who presented with fever and headache with a history of sore throat. His blood culture was positive for group A Streptococcus (GAS) and was subsequently found to have internal jugular vein, sigmoid, and transverse sinus vein thrombosis.
https://ift.tt/2uDRGP6
Infectious Aortitis: A Life-Threatening Endovascular Complication of Nontyphoidal Salmonella Bacteremia
A 65-year-old Japanese man living in the United States presented with pyrexia and chills associated with intermittent lower abdominal and back pain for 5 days. He denied recent travel, rash, diarrhea, or rectal bleeding. Physical examination revealed spiking pyrexia, and routine laboratory tests revealed mild leukocytosis and neutrophilia. Abdominal CT with contrast showed findings highly compatible with aortitis. Comprehensive autoimmune evaluation was negative. Salmonella enterica serotype Enteritidis was isolated from blood cultures. IV antibiotics were administered, but the patient continued to experience low-grade pyrexia and mild leukocytosis, and follow-up abdominal CT showed progressive aortic inflammation. The patient therefore underwent resection of the affected aortic segment with in-situ graft replacement and lifelong suppressive antibiotics. The patient is asymptomatic with no complications at 18 weeks of follow-up. This case report illustrates that patients with infectious aortitis from nontyphoidal Salmonella may (1) present with nonspecific and nonlocalizing symptoms and signs except for sepsis; (2) have diagnostic blood cultures and abdominal CT findings; and (3) typically require aggressive, prolonged IV antibiotic therapy and surgery for potential cure of this life-threatening infection.
https://ift.tt/2J939cF
A Large Grade 5 Mobile Aortic Arch Atheromatous Plaque: Cause of Cerebrovascular Accident
Aortic atheromas (aortic atheromatous plaques) are defined by an irregular thickening of the intima ≥2 mm, and a complex plaque is defined as a protruding atheroma ≥4 mm with or without an attached mobile component. Stroke incidence is approximately 25% in patients with mobile plaques of the aortic arch and 2% in patients with quiescent nonmobile plaques. Antiplatelet agents, oral anticoagulants, and statins have been suggested in the management of atheromas. We present an 80-year-old male, with non-ST-segment elevation myocardial infarction (NSTEMI) and chronic dysarthria, found to have an acute cerebrovascular accident (CVA) secondary to embolism from a large 12 mm aortic arch plaque, treated medically with oral antiplatelet therapy, anticoagulation, and statin therapy.
https://ift.tt/2E91lNd
Multiple roles of lymphatic vessels in tumor progression
Qiaoli Ma | Lothar C Dieterich | Michael Detmar
https://ift.tt/2pTZNlS
Next generation natural killer cells for cancer immunotherapy: the promise of genetic engineering
May Daher | Katayoun Rezvani
https://ift.tt/2GspGzm
The safety and risk factors of revision adenoidectomy in children and adolescents: A nationwide retrospective population-based cohort study
To investigate the safety of adenoidectomy and risk factors of re-adenoidectomy, and intend to provide evidence-based information to clinicians for further consideration.
https://ift.tt/2pWg8pW
Multimodality Surgical Approach in Management of Laryngotracheal Stenosis
Introduction. Postintubation laryngotracheal stenosis requires a precise diagnosis and an experienced operator in both endoscopic and surgical treatment. This report presents surgically treated cases of laryngotracheal stenosis secondary to long-term intubation/tracheostomy with review of the literature. Materials and Methods. In this retrospective study, we present 5 cases (a 23-year-old male, 13-year-old male, 22-year-old male, 19-year-old male, and 33-year-old female) of postintubation/tracheostomy laryngotracheal (glottic/subglottic) stenosis in the years 2016 and 2017. Each patient was managed differently. Intubation characteristics, localization of stenosis, surgical technique and material, postoperative complications, and survival of patients were recorded. Results. The site of stenosis was in the subglottis in 4 patients and glottis in 1 patient. The mean length of the stenosis was greater in the postintubation group. Postintubation stenosis had a mean duration of intubation of 6.8 days, compared to 206.25 days of cannulation following tracheostomies. Each patient underwent an average of 2 procedures during their treatment course. One patient underwent open surgical anastomosis because of recurrent subglottic stenosis after multiple treatments. Phonation improved immediately in almost all except in the patient who underwent only endoscopic dilatation. Discussion. The reasons for laryngeal stenosis and its delayed diagnosis have been reviewed from the literature. Suture tension should be appropriate, and placement of the suture knot outside the trachea minimizes formation of granulation tissue. The published reports suggest that resection by endoscopy with laser and open technique resection and primary anastomosis are the best treatment modality so far as the long-term results are concerned. Conclusion. Resection of stenotic segment by open surgical anastomosis and laser-assisted resection is a safe option for the treatment of subglottic stenosis following intubation without the need for repeated dilation. Endoscopic dilation can be reserved for unfit patients.
https://ift.tt/2GMy4xf
Spinal Accessory Nerve Duplication: A Case Report and Literature Review
Aim of the present study is to expand our knowledge of the anatomy of the 11th cranial nerve and discuss the clinical importance and literature pertaining to accessory nerve duplication. We present one case of duplicated spinal accessory nerve in a patient undergoing neck dissection for oral cavity cancer. The literature review confirms the extremely rare diagnosis of a duplicated accessory nerve. Its clinical implication is of great importance. From this finding, a further extension to our knowledge of the existing anatomy is proposed.
https://ift.tt/2uCOanZ
Evaluation of the effects of occlusal splint, trigger point injection and arthrocentesis in the treatment of internal derangement patients with myofascial pain disorders
Publication date: Available online 31 March 2018
Source:Journal of Cranio-Maxillofacial Surgery
Author(s): Itır Şebnem Bilici, Yusuf Emes, Serhat Yalçın, Buket Aybar
https://ift.tt/2Imj8mC
A Multimodal analysis using flowmeter analysis, laser-Doppler spectrophotometry, and indocyanine green videoangiography for the detection of venous compromise in flaps in rats
Publication date: Available online 30 March 2018
Source:Journal of Cranio-Maxillofacial Surgery
Author(s): Lucas M. Ritschl, Leonard H. Schmidt, Andreas M. Fichter, Alexander Hapfelmeier, Klaus-Dietrich Wolff, Thomas Mücke
Venous congestion results in tissue damage and remains the most common failure of free microvascular transfer if it is not recognized early. The purpose of this experimental study was to evaluate venous congestion and describe the findings with two different monitoring tools. A standardized epigastric flap was raised, and total occlusion of the draining vein was temporarily applied for 4, 5, 6, or 7 hours. Blood flow measurements, including laser-Doppler flowmetry, and tissue spectrophotometry (O2C) and indocyanine green (ICG) videoangiography using the FLOW® 800 tool, were performed systematically after each surgical step, an interval of venous occlusion, and 1 week of clinical observation. Both monitoring tools were capable of detecting acute venous occlusion. ICG videoangiography data showed a significant decrease in the first and second maximum, and the area under the curve, during venous occlusion, whereas hemoglobin levels in the O2C analysis remained stable. Changes in fluorescence values in border areas of the flap correlated significantly with the incidence of necrosis. O2C data later showed significant correlation with the area of necrosis, and more individual changes during flap monitoring. ICG videoangiography might therefore be useful in the prediction of flap necrosis in critical areas of perfusion.
https://ift.tt/2Grtchi
Trajectory-guided biopsy of orbital tumor - Technology, principal considerations and clinical implementation -
Publication date: Available online 31 March 2018
Source:Journal of Cranio-Maxillofacial Surgery
Author(s): Majeed Rana, Christoph Sproll, Julian Lommen, Madiha Rana, Alexander Zeller, Norbert Kübler, Karsten Hufendiek, Elvis Hermann, Nils-Claudius Gellrich, Henrik Holtmann
Intraorbital space-occupying lesions always pose a challenge, both in terms of definite surgical removal as well as preoperative sampling for histopathological examination. Despite the use of modern high-resolution imaging techniques, the dignity of orbital lesions can often not be determined with sufficient certainty preoperatively. As the amount and complexity of treatment possibilities continue to increase, detailed diagnostics in advance of treatment choice are essential. Histological classification of orbital lesions can still be considered the gold standard for reliable diagnoses, leading to appropriate treatment.Over recent years minimally invasive surgical approaches have gained more importance in the treatment and diagnosis of cranio-maxillo-facial tumor and trauma.The aim of our study was to adapt and establish a precise procedure for orbital biopsies. 23 patients suffering from space-occupying lesions of unknown dignity were included. Trajectory-guided procedures were pre-planned for all cases. In most cases minimally invasive procedures were suitable for taking biopsies of the orbit. For only two patients a conventional, non-minimally invasive, lateral orbitotomy had to be performed. Further evaluation of the presented procedure demonstrates clearly that trajectory-guided biopsies of the orbit can be performed correctly and effectively, regardless of the suspected lesion's size.
https://ift.tt/2Imj46m
Safety and Efficacy of a Halobetasol/Tazarotene Fixed Combination in the Treatment of Moderate-to-Severe Plaque Psoriasis: Results of two Phase 3 randomized controlled trials
Topical corticosteroids are the mainstay of psoriasis treatment; long-term safety concerns limit use. Combination with tazarotene may optimize efficacy, minimizing safety/tolerability concerns, In patients with moderate-to-severe plaque psoriasis treated with HP/TAZ lotion, improvement is noted within 2 weeks with few adverse effects observed after 8 weeks., HP/TAZ lotion may provide a realistic topical option for psoriasis management
https://ift.tt/2J9TjHF
Evaluation of the effects of occlusal splint, trigger point injection and arthrocentesis in the treatment of internal derangement patients with myofascial pain disorders
Temporomandibular disorders (TMD) may originate from the joint itself or from pathologies of masticatory muscles. Most pathology seen is myofascial pain disorder (MPD). TMD are characterized by pain, muscle spasms, and constraints in the movements of the temporomandibular joint. In temporomandibular joint disorders, besides the internal derangement, protective muscle spasms are usually observed.
https://ift.tt/2E9Ihym
Implant stability using piezoelectric bone surgery compared with conventional drilling: a systematic review and meta-analysis
The aim of this systematic review was to assess the primary and secondary stability of dental implants placed at sites prepared with piezoelectric bone surgery (PBS) and conventional drilling (CD). The PubMed/MEDLINE and Cochrane Library databases were searched without date or language restriction up to June 2017. Controlled clinical trials in which each patient received implants placed at sites prepared with both PBS and CD were selected. Implant stability had to be measured on day 0 and during the osseointegration period.
https://ift.tt/2pTYHGJ
Implant stability using piezoelectric bone surgery compared with conventional drilling: a systematic review and meta-analysis
Source:International Journal of Oral and Maxillofacial Surgery
Author(s): S. García-Moreno, J. González-Serrano, R.M. López-Pintor, B. Pardal-Peláez, G. Hernández, J.M. Martínez-González
The aim of this systematic review was to assess the primary and secondary stability of dental implants placed at sites prepared with piezoelectric bone surgery (PBS) and conventional drilling (CD). The PubMed/MEDLINE and Cochrane Library databases were searched without date or language restriction up to June 2017. Controlled clinical trials in which each patient received implants placed at sites prepared with both PBS and CD were selected. Implant stability had to be measured on day 0 and during the osseointegration period. Methodological quality was assessed using the Cochrane Collaboration tool. A meta-analysis was performed to compare primary stability (on day 0) and secondary stability (after 2 and 3months) between the two groups. The studies included were determined to have a high risk of bias. There was no significant difference between the two groups for primary stability (on day 0) (P=0.51). After 2 and 3months, secondary stability was statistically higher in implants placed with PBS preparation (P=0.04 and P=0.01, respectively). The implant survival rate was 97.5% in the CD group and 100% in the PBS group. PBS preparation improves secondary stability after 2 and 3months in comparison to CD, with similar implant survival rates. Further studies are needed to determine whether implant osseointegration periods could be shortened with PBS preparation.
https://ift.tt/2pVfWq7
An audiological evaluation of syndromic and non-syndromic craniosynostosis in pre-school going children
Source:International Journal of Pediatric Otorhinolaryngology, Volume 109
Author(s): Liang Chye Goh, Ali Azman, Hufaidah binti Konting Siti, Wee Vien Khoo, Premala a/p Muthukumarasamy, Meow Keong Thong, Zulkiflee Abu Bakar, Anura Michelle Manuel
ObjectiveTo study the audiological outcome and early screening of pre-school going children with craniosynostosis under follow-up at the University of Malaya Medical Center(UMMC), Kuala Lumpur, Malaysia over a 10 year period.MethodsA retrospective descriptive cohort study on the audiological findings detected during the first hearing assessment done on a child with craniosynostosis using otoacoustic emissions, pure tone audiometry or auditory brainstem response examination. The main aim of this study was to evaluate the type and severity of hearing loss when compared between syndromic and non-sydromic craniosynostosis, and other associated contributory factors.ResultsA total of 31 patients with 62 ears consisting of 14 male patients and 17 female patients were evaluated. Twenty two patients (71%) were syndromic and 9 (29%) were non-syndromic craniosynostosis. Amongst the syndromic craniosynostosis, 9 (41%) had Apert syndrome, 7 (32%) had Crouzon syndrome, 5 (23%) had Pfieffer syndrome and 1 (4%) had Shaethre Chotzen syndrome. Patients with syndromic craniosynostosis were more likely to present with all types and severity of hearing loss, including severe to profound sensorineural hearing loss while children with non-syndromic craniosynostosis were likely to present with normal hearing (p < 0.05). In addition, when the first hearing test was done at a later age, a hearing loss including sensorineural hearing loss is more likely to be present in a child with syndromic craniosynostosis (p < 0.05).ConclusionOur study suggested that children who are born with syndromic craniosynostosis were more likely to suffer from a hearing loss, including that of a severe to profound degree compared to children with non-syndromic craniosynostosis. In addition to that, hearing loss is more likely to be detected when the first hearing test is done at a later age, and this can be an irreversible sensorineural hearing loss. We would like to advocate the need for early audiological screening and follow up in children with syndromic craniosynostosis.
https://ift.tt/2JblGFm
Surgical timing for bilateral simultaneous cochlear implants: When is best?
Publication date: June 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 109
Author(s): Sebastiano Franchella, Roberto Bovo, Luigia Bandolin, Flavia Gheller, Silvia Montino, Daniele Borsetto, Sara Ghiselli, Alessandro Martini
IntroductionHearing loss is considered the most common congenital disease and the prevalence of neonatal deafness can be estimated between 1 and 2 cases per 1000 live births.Infant deafness must be diagnosed as early as possible and an effective therapeutic intervention needs to be carried out in order to avoid the serious consequences of hearing deprivation during the evolutionary period: alterations in the development of central auditory pathways and lack of language acquisition.The cochlear implant (CI) has proved to be the best instrument to solve the problem of auditory deprivation. In particular, the bilateral CI gives the patient access to binaural hearing which results in benefits in terms of sound localisation and discrimination.The optimal age of application of the CI is a widely discussed topic in the scientific community and the current guidelines indicate a period between 12 and 24 months of age, even though the supporters of the application before 12 months of age are nowadays increasing.Materials and methodsThe study is observational, retrospective, monocentric. 49 paediatric patients (<18 years) with simultaneous bilateral CIs were included. The audiometric threshold and speech tests were carried out during the follow-up 3, 6 and 12 months after the CIs activation and when the patient reached 2 years of age.ResultsThe statistical analysis showed that undergoing bilateral implantation surgery before 2 years of age allows a satisfactory audiometric performance, while there are no particular benefits in performing the surgery before 1 year of age. As far as the speech outcome is concerned, the statistical analysis didn't show significant correlation between the earlier age of implantation and better speech performance if the operation is carried out before 2.5 years of age.ConclusionsThe results of the study indicate that the optimal age to perform the simultaneous bilateral CIs surgery is between 12 and 24 months, without demonstrating any particular benefit in carrying out the procedure before 1 year of age. This may be clinically relevant in terms of avoiding the risks of diagnostic mistakes and reducing the related surgical risk in children under 1 year of age.
https://ift.tt/2pSV3fx
Editorial board
Source:Annales de Dermatologie et de Vénéréologie, Volume 145, Issue 3
https://ift.tt/2J9mvhZ
Actinomycose révélée par une ulcération du palais et de la gencive
Source:Annales de Dermatologie et de Vénéréologie, Volume 145, Issue 3
Author(s): F. Dessirier, J.-P. Arnault, J. Denamps, H. Sevestre, C. Attencourt, C. Lok
IntroductionL'actinomycose est une infection bactérienne granulomateuse chronique extensive. Elle est rarement révélée par une ulcération buccale.ObservationUne femme diabétique de 76 ans était traitée par dabrafenib pour un mélanome stade IV. Lors d'une consultation de suivi, on constatait deux lésions ulcérées du palais dur et de la gencive, infiltrées et hyperalgiques. Il n'y avait pas de signe associé. Le bilan biologique était sans particularité. La première hypothèse diagnostique évoquée était celle d'un carcinome épidermoïde muqueux survenu sous inhibiteurs de BRAF, bien que cette situation soit très peu décrite dans la littérature. L'examen histologique montrait un grain actinomycosique. La tomodensitométrie du massif facial ne montrait pas d'ostéite. Une antibiothérapie par amoxicilline était instaurée pendant quatre mois, avec une évolution favorable.DiscussionLes actinomycètes sont des bactéries filamenteuses Gram positif, saprophytes de la cavité buccale et du tractus gastro-intestinal, qui deviennent pathogènes sous l'influence de plusieurs facteurs. L'atteinte cervico-faciale, sous la forme d'un nodule inflammatoire péri-mandibulaire avec fistulisation secondaire à la peau ou dans la bouche, est la présentation la plus classique de l'actinomycose. Aucun cas d'infection opportuniste n'a été décrit à notre connaissance sous inhibiteurs de BRAF ; seuls deux cas de tuberculose ont été rapportés avec le sorafenib. Chez notre patiente, les soins dentaires et le diabète sont les deux facteurs favorisants retenus. Nous rappelons par ailleurs l'importance de l'examen des muqueuses chez les patients traités par inhibiteurs de BRAF.BackgroundActinomycosis is a chronic and extensive granulomatous, bacterial infection. Revelation by oral ulceration is rare.Patients and methodsA 76-year-old patient with diabetes was treated with dabrafenib for stage IV melanoma. A follow-up visit revealed two ulcerated, infiltrated and hyperalgesic lesions of the palate and gingiva. There were no associated signs. The laboratory findings were normal. The possibility of squamous cell carcinoma occurring with BRAF inhibitors was discussed, despite the rarity of such cases in the literature. Histological examination showed an actinomycotic grain. A scan of the facial mass showed no osteitis. Antimicrobial therapy was initiated with amoxicillin for four months, with a favorable outcome.DiscussionActinomycetes are Gram-positive filamentous saprophytic bacteria of the oral cavity and the gastrointestinal tract. They can become pathogenic under the influence of several factors. Cervicofacial involvement in the form of a peri-mandibular inflammatory nodule with secondary fistulation on the skin or in the mouth is the classic presentation. To our knowledge, no cases of opportunistic infection under BRAF inhibitors have been described. Only two cases of tuberculosis have been reported with sorafenib. The initial presentation led to suspicion of squamous cell carcinoma. In our patient, poor oral hygiene and diabetes were the two key factors considered. Moreover, this is the first case reported under dabrafenib, which does not appear to be a favoring factor. We would stress the importance of mucosal examination in patients treated with BRAF inhibitors.
https://ift.tt/2E6N0AB
Hydrochlorothiazide et cancers cutanés
Source:Annales de Dermatologie et de Vénéréologie, Volume 145, Issue 3
Author(s): J.-L. Schmutz
https://ift.tt/2J6RRFI
Thyroid Cartilage Window Approach to Extract a Foreign Body after Migration into the Paraglottic Space
We report a case of fish bone impaction in the paraglottic space, which caused palsy of the left vocal cord. The patient was a 45-year-old man. He presented with throat pain and hoarseness of voice for approximately one week. The diagnosis was made after careful history taking and confirmed by the use of computed tomography scan as the fish bone was not visible endoscopically under local and general anaesthesia. The patient underwent thyroid cartilage window approach, and the fish bone was retrieved. His symptoms have improved significantly, and he did not require tracheostomy. Other cases reported the removal of foreign bodies by other techniques such as laryngofissure and posterolateral approach. Our case is different in that we used a modification of thyroplasty type 1 technique as it has less reported complications than other approaches that were published in literature.
https://ift.tt/2J83vQL
Soft Palate Pleomorphic Adenoma of a Minor Salivary Gland: An Unusual Presentation
Approximately 10% of pleomorphic adenomas occur in the minor salivary glands with the palate being the most common site. Pleomorphic adenomas account for the majority of palatal tumours; however, minor salivary gland tumours have a higher risk of malignancy compared to tumours of the major salivary glands, so appropriate diagnostic evaluation should be prompt. We present a case of a 52-year-old man with a longstanding history of a soft palate pleomorphic adenoma which required excision under general anaesthetic via a mandibular swing approach. As well as the surgical approach to access this tumour; this case is unique as it is the largest soft palate pleomorphic adenoma reported in the literature. We discuss the appropriate preoperative investigations and airway considerations for this patient, as well as the factors to consider when planning operative management of palatal tumours.
https://ift.tt/2pSZTcw
Correction to: Esophagus perforation and myocardial penetration caused by swallowing of a foreign body leading to a misdiagnosis of acute coronary syndrome: a case report
In the publication of this article [1], the Acknowledgements section was missing.
https://ift.tt/2pPGEBC
Coronary artery fistula with associated Takotsubo cardiomyopathy: a case report
Coronary artery fistula, first described by Krause in 1865, is an abnormal communication between the coronary artery and one of the four chambers of the heart or one of the great vessels. The communications ar...
https://ift.tt/2GpAtuh
Induction of a central memory and stem cell memory phenotype in functionally active CD4 + and CD8 + CAR T cells produced in an automated good manufacturing practice system for the treatment of CD19 + acute lymphoblastic leukemia
Abstract
Relapsed/refractory B-precursor acute lymphoblastic leukemia (pre-B ALL) remains a major therapeutic challenge. Chimeric antigen receptor (CAR) T cells are promising treatment options. Central memory T cells (Tcm) and stem cell-like memory T cells (Tscm) are known to promote sustained proliferation and persistence after T-cell therapy, constituting essential preconditions for treatment efficacy. Therefore, we set up a protocol for anti-CD19 CAR T-cell generation aiming at high Tcm/Tscm numbers. 100 ml peripheral blood from pediatric pre-B ALL patients was processed including CD4+/CD8+-separation, T-cell activation with modified anti-CD3/-CD28 reagents and transduction with a 4-1BB-based second generation CAR lentiviral vector. The process was performed on a closed, automated device requiring additional manual/open steps under clean room conditions. The clinical situation of these critically ill and refractory patients with leukemia leads to inconsistent cellular compositions at start of the procedure including high blast counts and low T-cell numbers with exhausted phenotype. Nevertheless, a robust T-cell product was achieved (mean CD4+ = 50%, CD8+ = 39%, transduction = 27%, Tcm = 50%, Tscm = 46%). Strong proliferative potential (up to > 100-fold), specific cytotoxicity and low expression of co-inhibitory molecules were documented. CAR T cells significantly released TH1 cytokines IFN-γ, TNF-α and IL-2 upon target-recognition. In conclusion, partly automated GMP-generation of CAR T cells from critically small blood samples was feasible with a new stimulation protocol that leads to high functionality and expansion potential, balanced CD4/CD8 ratios and a conversion to a Tcm/Tscm phenotype.
https://ift.tt/2pYofmf
Stability of allergens
Source:Molecular Immunology
Author(s): Judith Pekar, Davide Ret, Eva Untersmayr
For proteins to cause IgE-mediated allergic reactions, several common characteristics have to be defined, including small molecular size, solubility and stability to changing pH levels and enzymatic degradation. Nevertheless, these features are not unique for potent allergens, but are also observed in non-allergenic proteins. Due to the increasing awareness by regulatory authorities regarding the allergy pandemic, definition of characteristics unique to potent allergens would facilitate allergenicity assessment in the future. Despite major research efforts even to date the features unique for major allergens have not been elucidated so far. The route of allergen entry into the organism determines to a great extent these required characteristics. Especially orally ingested allergens are exposed to the harsh milieu of the gastrointestinal tract but might additionally be influenced by food processing. Depending on molecular properties such as disulphide bonds contributing to protein fold and formation of conformational IgE epitopes, posttranslational protein modification or protein food matrix interactions, enzymatic and thermal stability might differ between allergens. Moreover, also ligand binding influences structural stability. In the current review article, we aim at highlighting specific characteristics and molecular pattern contributing to a stabilized protein structure and overall allergenicity.
https://ift.tt/2J6KiPu
Nebulized lidocaine ameliorates allergic airway inflammation via downregulation of TLR2
Source:Molecular Immunology, Volume 97
Author(s): Lixia Wang, Muzi Wang, Shuai Li, Huimei Wu, Qiying Shen, Shihai Zhang, Lei Fang, Rongyu Liu
Nebulized lidocaine has been suggested to be beneficial in asthma therapy, but the underlying mechanisms are little known. We aimed to investigate whether Toll-like receptor (TLR) 2 was involved in the protective effect of lidocaine on allergic airway inflammation. Female C57BL/6 mice were sensitized and challenged with ovalbumin (OVA). Meanwhile, some of the mice were treated with TLR2 agonist (Pam3CSK4, 100 μg) intraperitoneally in combination with OVA on day 0. Just after allergen provocation, mice were treated with inhaled lidocaine or vehicle for 30 min. In this model, we found that lidocaine markedly attenuated OVA-evoked airway inflammation, leukocyte recruitment and mucus production. Moreover, lidocaine abrogated the increased concentrations of T cytokines and TNF-α in bronchoalveolar lavage fluid (BALF) of allergic mice, as well as reducing the expression of phosphorylated nuclear factor (P-NF)-κBp65 and the NOD-like receptor pyridine containing 3 (NLRP3), which are important for the production of pro-inflammatory cytokines. In addition, our study showed that lidocaine dramatically decreased OVA-induced increased expression of TLR2 in the lung tissues. Furthermore, activation of TLR2 aggravated OVA-challenged airway inflammation, meanwhile, it also elevated OVA-induced expression of P-NF-κBp65 and NLRP3 in the lungs. However, lidocaine effectively inhibited airway inflammation and counteracted the expression of P-NF-κBp65 and NLRP3 in allergic mice pretreated with Pam3CSK4. Taken together, the present study demonstrated that lidocaine prevented allergic airway inflammation via TLR2 in an OVA-induced murine allergic airway inflammation model. TLR2/NF-κB/NLRP3 pathway may serve as a promising therapeutic strategy for allergic airway inflammation.
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New insights into the structure of the MHC class I peptide-loading complex and mechanisms of TAP inhibition by viral immune evasion proteins
Source:Molecular Immunology
Author(s): Patrique Praest, A. Manuel Liaci, Friedrich Förster, Emmanuel J.H.J. Wiertz
Several hundred million years of co-evolution of vertebrates and invading pathogens have shaped the adaptive immune system to fight back the unwanted invaders through highly sophisticated defense mechanisms. Herpesviruses manage to dodge this immune response by hampering one of the central hinges of human adaptive immunity, the major histocompatibility complex (MHC) class I antigen presentation pathway. One of the bottlenecks of this pathway is the loading of pathogen-derived peptides onto MHC-I molecules in the endoplasmic reticulum (ER). This task is accomplished by the MHC class I peptide-loading complex (PLC), of which the transporter associated with antigen-processing (TAP) is a central component. In this review, we summarize recent structural and functional insights into the molecular architecture of the PLC, how TAP accomplishes the transport of peptides across the ER membrane, and how herpes- and poxviruses inhibit TAP-mediated peptide translocation and subsequent antigen presentation.
https://ift.tt/2J3KL4Z
Pre-diagnostic dynamic HPV16 IgG seropositivity and risk of oropharyngeal cancer: Methodologic issues
Source:Oral Oncology
Author(s): Karen S. Anderson, Jon Mork, Hilde Langseth, Garrick Wallstrom
https://ift.tt/2GXSG32
Osteochondroma of the Zygomatic Arch: A Case Report and Review of the Literature
Source:Journal of Oral and Maxillofacial Surgery
Author(s): R. Patel, G. Obeid
https://ift.tt/2E9ryeq
Assessing the Quality of Life in Patients with Dentofacial Deformities Before and After Orthognathic Surgery
Publication date: Available online 31 March 2018
Source:Journal of Oral and Maxillofacial Surgery
Author(s): Hong Sun, Hong-tao Shang, Li-sheng He, Ming-chao Ding, Zhong-ping Su, Yu-lin Shi
PurposeThis study aimed to assess the effect of health-related quality of life (QoL) among patients with dentofacial deformities who underwent orthognathic surgery, compared to a control group without dentofacial deformities, by using generic oral health and condition-specific approaches.Patients and MethodsIn this prospective study, two questionnaires were administered to 85 patients (31 men and 54 women) who were evaluated before undergoing orthognathic surgery. The Short Form Oral Health Impact Profile Questionnaire (OHIP-14) and the Orthognathic Quality of Life Questionnaire (OQLQ) were administered before and 5–7 months after orthognathic surgery. The control group comprised 96 young university student volunteers without dentofacial deformities.ResultsThe questionnaires were collected 5–7 months post-surgery. The preoperative scores of the patients and the control group were contrasted separately. The respondents' OHIP-14 and OQLQ post-surgery scores were significantly lower (total scores, P < 0.001). The pre-surgery OQLQ all domains scores were significantly higher among the patients than among the controls (total scores, P < 0.001); however, the total scores and three subscale scores of the OHIP-14 in the functional and psychological domains were significantly higher among the patients than among the controls (total scores, P < 0.05). The pre- and post-surgery total OQLQ scores were remarkably different between males and females (P < 0.05). The post-surgery total OQLQ scores were considerably higher in older patients than in younger patients (P < 0.05). All patients in the Class III group who underwent double jaw surgery showed remarkable changes post-surgery (total scores, P < 0.001).ConclusionPatients with dentofacial deformities had a poorer quality of life, compared to the normal population, especially in functional and psychological aspects. Orthognathic surgery had a significant positive impact on QoL. Patients with Class III malocclusion who underwent double jaw surgery seemingly benefitted the most after surgery.
Graphical abstract
https://ift.tt/2H0lNmm
Fracture healing and bone remodeling with human standard-sized magnesium versus polylactide-co-glycolide (PLGA) plate and screw systems using a mini-swine craniomaxillofacial osteotomy fixation model
Publication date: Available online 31 March 2018
Source:Journal of Oral and Maxillofacial Surgery
Author(s): Benoît Schaller, John Patrik Matthias Burkhard, Madeleine Chagnon, Stefan Beck, Thomas Imwinkelried, Michel Assad
PurposeThis study compared the degradation profile, safety and efficacy of a bioresorbable magnesium (Mg) alloy and polylactide-co-glycolide (PLGA) polymer osteosynthesis systems for the treatment of fractures in a load-sharing maxillofacial environment using a new mini-swine fracture fixation model.MethodsTwo types of clinically relevant situations were evaluated in five Yucatan miniature pigs. Defined porcine midface osteotomies of the supra-orbital rim and zygoma were created and fixed with either coated Mg (test animals) or PLGA plate and screw osteosynthesis systems (control animals). Following surgery, mini-pigs were able to recover for either one or nine months with continuous in vivo post-implantation monitoring. Standardized computed tomography (CT) imaging was taken immediately post-operatively and at termination for all animals. The nine-month cohort additionally underwent CT at 2, 4, and 6 months post-surgery. At necropsy, osteotomy sites and bone-implant units were harvested and the healing was evaluated under micro-CT, histopathology and histomorphometry.ResultsFollowing clinical and radiological follow-up examination, all fracture sites healed well for both magnesium and polymer groups regardless of time. Complete bone union and gradually disappearing osteotomy lines were observed across all implantation sites, with no major consistency change in periprosthetic soft tissue nor soft tissue calcification. Macroscopic and microscopic examination showed no negative influence of gas formation observed with Mg during the healing process. Histopathology demonstrated similar fracture healing outcomes for both plating systems with good biocompatibility as evidenced by a minimal or mild tissue reaction.ConclusionThe present study confirms that WE43-Mg alloy exhibited excellent fracture healing properties before its full degradation and without causing any significant inflammatory reactions in a long-term porcine model. Compared to PLGA implants, magnesium represent a promising new biomaterial with reduced sizes and improved mechanical properties in order to support fracture healing in a load-sharing environment.
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Does sphenopalatine ganglion block improve pain control and intra-operative hemodynamics in children undergoing palatoplasty? A randomized control trial
Publication date: Available online 30 March 2018
Source:Journal of Oral and Maxillofacial Surgery
Author(s): Anantanarayanan Parameswaran, Muruganand V. Ganeshmurthy, Yashoda Ashok, Manikandhan Ramanathan, Anthony F. Markus, Hermann F. Sailer
BackgroundChildren undergoing cleft palate repair suffer pain, dysphagia and wound irritation in the immediate post-operative phase which may compromise surgical outcome. This trial evaluates the efficacy of spheno-palatine ganglion block (SPGB) in optimizing intra-operative hemodynamics and post-operative analgesia in children undergoing primary palatoplastyMaterials and MethodsThe study was designed as a prospective, double blinded, randomized control trial comparing the use of SPGB with general anesthesia (GA) versus a control group of GA only. Routine pre-operative documentation included type of cleft, patient's weight, hemoglobin(Hb%), packed cell volume(PCV), blood pressure(BP) and echo-cardiogram. Intra-operative monitoring included heart rate, blood pressure and surgical field assessment. Post-operatively, the pain score, pain free duration and need for rescue analgesics were recorded. Post-surgical change in Hb% and PCV values were assessed. Data analysis of collected variables was done using SPSS Version 16 (IBM Corp, Chicago, USA). Quantitative data was assessed for normality using Shapiro Wilk's test and analysed using the Imdependent sample t test, and Fisher Exact test was used for comparison of the binary variable (Gender). The outcome variables were compared between the study and control groups after adjusting for confounding variables. P value of <0.05 was considered statistically significant.Results100 Patients undergoing primary palatoplasty under GA were randomised into control group A (N=49) and study group B (SPGB)(N=51). Three patients were excluded from the Control group due to changes in intraoperative anesthetic protocol. The results demonstrated stastistically significant differences in the post-surgical pain free duration (19.46 Vs 87.59 mins) and mean blood loss (105.5 Vs 62 mL), in favour of the study group. Surgical field and post-op reduction of Hb% and PCV were also significantly favourable for the study group.ConclusionWe conclude that the SPGB is a potent pre-emptive technique offering excellent perioperative analgesia, hemodynamic stability and a clear surgical field.
https://ift.tt/2H420md