Description of a novel RyR2 mutation in a juvenile patient with symptomatic catecholaminergic polymorphic ventricular tachycardia in sleep and during exercise: a case report

Catecholaminergic polymorphic ventricular tachycardia is an inherited disease presenting with arrhythmic events during physical exercise or emotional stress. If untreated, catecholaminergic polymorphic ventric...

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Association of benign paroxysmal positional vertigo with vitamin D deficiency: a systematic review and meta-analysis

Abstract

Background and objective

Benign paroxysmal positional vertigo (BPPV) is an idiopathic recurrent inner ear illness that is caused most often by an imbalance in the metabolism of calcium carbonate crystals (otoconia) inside the semicircular canals, in which the otoconia begin to circulate freely after being dislodged from the basic structure. The underlying etiology of this imbalance has not yet been well established; however, a few recent articles have revealed that vitamin D level abnormality in these patients might play a role. Therefore, we conducted the current systematic review analysis to explore potential associations of vitamin D level with the occurrence as well as the recurrence of BPPV disease.

Methods

A comprehensive literature search was conducted using different databases to retrieve all of the articles that have evaluated possible associations, irrespective of the study design. Then, we reported different vitamin D3 levels from BPPV groups and control groups to estimate the standardized mean difference (SMD) between the BPPV and control groups. We also calculated the effect size of each study under the random effects statistical model.

Results

Of the 703 studies that we identified, only 37 studies were found to be potential for our analysis, and of these, only seven met our predetermined criteria. Two meta-analyses were conducted with respect to the occurrence and the recurrence of BPPV. When the BPPV cases were compared to the controls (free of BPPV disease), there was an insignificant reduction in vitamin D level among the diseased groups (SMD = − 2.20; 95% CI − 6.66 to 2.26). In contrast, when the recurrent BPPV groups were compared with the non-recurrent BPPV groups, the statistical analysis showed significantly lower level of vitamin D among the recurrence BPPV groups (SMD = − 4.47; 95% CI − 7.55 to − 1.29).

Conclusion

Although a negative vitamin D imbalance has been reported among some BPPV patients, this review analysis failed to establish a relationship between the occurrence of BPPV and low vitamin D level. However, low vitamin D level was significantly evident among patients with recurrent episodes of BPPV.

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Investigation of ultra-low insertion speeds in an inelastic artificial cochlear model using custom-made cochlear implant electrodes

Abstract

Purpose

Latest research on cochlear implantations focuses on hearing preservation during insertion of the implant's electrode array by reducing insertion trauma. One parameter which may influence trauma is insertion speed. The objective of this study was to extend the range of examined insertion speeds to include ultra-low velocities, being lower than manually feasible, and investigate whether these reduce insertion forces.

Methods

24 custom-made cochlear implant test samples were fabricated and inserted into an artificial scala tympani model using 12 different insertion speeds while measuring the resulting insertion forces. Three commercially available slim straight electrode carriers were inserted using the same setup to analyze whether the results are comparable.

Results

Insertions of the test samples using high insertion speeds (2.0/2.8 mm/s) showed significantly higher insertion forces than insertions done with low insertion speeds (0.2 mm/s) or ultra-low insertion speeds (< 0.1 mm/s). The insertions with commercial slim straight electrode arrays showed significantly reduced insertion forces when using a low insertion speed as well.

Conclusions

Slow insertions showed significantly reduced insertion forces. Insertion speeds which are lower than manually feasible showed even lower insertion forces.

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Urachal carcinoma: from gross specimen to morphologic, immunohistochemical, and molecular analysis

Abstract

Urachal carcinoma (UrC) is an exceedingly rare neoplasm that develops from the urachus, an embryologic remnant of the urogenital sinus and allantois. The most commonly encountered histologic subtype is adenocarcinoma. The aim of this study is to characterize a series of UrC by morphology, immunohistochemistry, and molecular analysis. We retrospectively investigated seven cases of UrCs and assessed patient symptoms, imaging, histologic features, immunohistochemical profile, molecular characteristics, pathologic stages, and type of treatment. Immunostaining for CK7, CK20, Muc-2, CDX2, GATA3, β-catenin, and CK34βE12 was carried out on each neoplasm and on seven non-neoplastic urachal remnants as the control group. Additionally, a mutational analysis was performed using the QIAact Actionable Insights Tumor Panel Kit, which analyzes KRAS, NRAS, KIT, BRAF, PDGFRA, ALK, EGFR, ERBB2, PIK3CA, ERBB3, ESR1, and RAF1. Our cohort comprised five females and two males with a mean age of 64 years. UrCs consisted of two mucinous cystadenocarcinomas and five invasive, non-cystic adenocarcinomas. Carcinoma antigen expression profile was positive for CK20 and negative for CK34βE12 and GATA3 in all cases. Five of seven cases stained positively for Muc-2 and CDX2. On the contrary, non-neoplastic urachal remnants were immunoreactive for CK34βE12, CK7, and GATA3. Mutational analysis gave a positive result in four out of seven (57.1%) cases. All four positive tumors showed RAS mutation and one an additional mutation in PIK3CA. Urachal tumors exhibit peculiar morphologic, immunohistochemical, and molecular features. Due to the advanced stage at presentation, individualized treatment should be undertaken.

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Microsurgical Anatomy of Stapedius Muscle: Anatomy Revisited, Redefined with Potential Impact in Surgeries

Abstract

Stapedius muscle even though being the smallest skeletal muscle in human body, it has a major role in otology. As many of the distinguished books in otology missed to explain much about stapedius muscle, and also considering the need for the anatomy based visit to this small muscle we felt it was important to have a exercise like this. In the dissection hall of our institution we dissected 32 cadaveric temporal bones and delineated stapedius muscle as a part of PG teaching programme to have a clear idea of the anatomy of stapedius muscle, its origin, attachment, extension, size (all dimensions). Length of the stapedius muscle varied between 9 and 11 mm. Stapedial tendon measured about 2 mm. The muscle had a classical sickle shape with tendon looking like the handle of the sickle. It has a bulky belly with a maximum breadth of 2–3 mm. It was found to be medial to midportion of vertical limb of facial nerve. All of our temporal bones measured size varied from 9 to 11 mm in length excluding stapedial tendon. Stapedial tendon measured almost 2 mm. Muscle is classical sickle shaped with tendon acting like the handle of the sickle. It has a bulky tummy which forms the maximum breadth of 1–3 mm. Why to have a clear idea about the anatomy of stapedius muscle is that, unless the anatomy is clear there is chance of confusing the muscle with that of facial nerve while doing facial nerve grafting and also while drilling for facial nerve decompression in experienced hands may get confused and decompress the muscle. Stapedius muscle said to be the smallest muscle in the body, but its not as small as its been described. Detailed awareness of the anatomy of stapedius muscle is needed so as to avoid confusion while facial nerve grafting and while drilling.

https://ift.tt/2Cxz1I2

Microsurgical Anatomy of Stapedius Muscle: Anatomy Revisited, Redefined with Potential Impact in Surgeries

Abstract

Stapedius muscle even though being the smallest skeletal muscle in human body, it has a major role in otology. As many of the distinguished books in otology missed to explain much about stapedius muscle, and also considering the need for the anatomy based visit to this small muscle we felt it was important to have a exercise like this. In the dissection hall of our institution we dissected 32 cadaveric temporal bones and delineated stapedius muscle as a part of PG teaching programme to have a clear idea of the anatomy of stapedius muscle, its origin, attachment, extension, size (all dimensions). Length of the stapedius muscle varied between 9 and 11 mm. Stapedial tendon measured about 2 mm. The muscle had a classical sickle shape with tendon looking like the handle of the sickle. It has a bulky belly with a maximum breadth of 2–3 mm. It was found to be medial to midportion of vertical limb of facial nerve. All of our temporal bones measured size varied from 9 to 11 mm in length excluding stapedial tendon. Stapedial tendon measured almost 2 mm. Muscle is classical sickle shaped with tendon acting like the handle of the sickle. It has a bulky tummy which forms the maximum breadth of 1–3 mm. Why to have a clear idea about the anatomy of stapedius muscle is that, unless the anatomy is clear there is chance of confusing the muscle with that of facial nerve while doing facial nerve grafting and also while drilling for facial nerve decompression in experienced hands may get confused and decompress the muscle. Stapedius muscle said to be the smallest muscle in the body, but its not as small as its been described. Detailed awareness of the anatomy of stapedius muscle is needed so as to avoid confusion while facial nerve grafting and while drilling.

https://ift.tt/2Cxz1I2

Gender-Related Facial Surgical Goals

Facial plast Surg 2018; 34: 474-479
DOI: 10.1055/s-0038-1670648

In the last several years, there has been an increasing demand for surgical facial feminization or masculinization to treat the gender dysphoric patient. However, while aesthetic ideals for facial rejuvenation are well described and taught, there is a relative paucity of literature on how to achieve a feminine or masculine morphology. It is thus becoming increasingly important for facial plastic surgeons to understand patient goals regarding facial feminization or masculinization, and to have a systematic approach for analyzing the female and male face.
[...]

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

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Achieving Ideal Facial Appearance

Facial plast Surg 2018; 34: 431-432
DOI: 10.1055/s-0038-1668573

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

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Enhanced Contouring of Local Flaps

Facial plast Surg 2018; 34: 433-442
DOI: 10.1055/s-0038-1668572

Scars and flaps represent a disruption of the normal skin contour, shape, and texture. Successful reconstruction of facial defects with local flaps requires prior planning to choose the correct reconstructive method. However, thorough preoperative planning should include consideration of adjunctive procedures to improve expected postoperative results as well as potential procedures to improve less-than-ideal results. Approaching facial reconstruction holistically allows the surgeon to soberly assess his/her reconstructive results and attain outstanding appearance.
[...]

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

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Correction of the Crooked Nose

Facial plast Surg 2018; 34: 488-496
DOI: 10.1055/s-0038-1672142

Successful management of the crooked nose can be a formidable task and requires a systematic approach that begins with an understanding of the patients' aesthetic desires, expectations, and functional symptoms. Dividing the nose into thirds and organizing a plan to correct each region individually can simplify the preoperative planning. There are two broad philosophical strategies of surgical management: engineering (reconstructive) and artistic (camouflaging). While the camouflaging technique is helpful for minor asymmetries, protrusions, and depressions, the engineering approach is best for more extreme corrections. The surgeon must be skilled in both approaches as either one or a combination of the two may yield the best results. Once corrected, postoperative care is essential to obtain optimal outcomes.
[...]

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Widened Dorsum: Bony and Cartilaginous Contributions

Facial plast Surg 2018; 34: 443-447
DOI: 10.1055/s-0038-1669989

Creation of a pleasing dorsal nasal profile in the anterior and lateral views requires proper analysis and planning to determine the required series of steps to accomplish the desired outcome. The widened nasal dorsum is a common esthetic complaint of the patient seeking rhinoplasty. Often patients seek an unrealistic result that, if accomplished, would leave them with a restricted nasal vault and nasal airway compromise. Nasal function must be balanced with the patient and surgeon's desire to narrow the nasal dorsum. Various techniques are used to control the width of the upper third, or bony vault, and middle third, or cartilaginous vault.
[...]

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

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Surgical and Nonsurgical Techniques in Forehead Rejuvenation

Facial plast Surg 2018; 34: 466-473
DOI: 10.1055/s-0038-1669990

The periorbital and forehead regions are among the most expressive parts of the face. A thorough understanding of the complex facial anatomy and aesthetic norms are essential when evaluating and treating a patient for facial brow rejuvenation. Not only will knowledge of the anatomy enhance facial injection and surgical outcomes, but will also diminish potential complications. Combining nonsurgical and surgical techniques, including laser and skin rejuvenation, can yield the most natural and effective aesthetic brow improvements.
[...]

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

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Facial Scar Improvement Procedures

Facial plast Surg 2018; 34: 448-457
DOI: 10.1055/s-0038-1669400

Scars are a natural part of dermal healing following lacerations, incisions, or tissue loss. The ideal scar is narrow, flat, level with surrounding tissue, and difficult for the untrained eye to see due to color match and placement parallel to relaxed skin tension lines; however, scarring that is dyspigmented, hypertrophied, widened, contracted, or atrophic can be aesthetically displeasing or causing functional limitations. When the scar has unfavorable characteristics, scar revision is often indicated and the cosmetic surgeon must be knowledgeable of the minimally invasive as well as surgical techniques to improve aesthetics, reduced reoccurrence, or correct functional limitations.
[...]

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

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Correction of the Lower Face and Neck

Facial plast Surg 2018; 34: 480-487
DOI: 10.1055/s-0038-1672128

Rejuvenation of the lower face and neck strives to reverse signs of aging while optimizing the patient's natural anatomy. Common features of an aesthetically pleasing lower face include a well-defined and appropriately balanced mandible and an acute cervicomental angle. Correction of the aging lower face and neck is accomplished through multiple surgical interventions, performed either alone or in combination. Determination of appropriate procedures is based on individual anatomic pathology. Intimate knowledge of facial anatomy and the complexity of the aging process is paramount to achieve a natural and aesthetic result. Thorough patient evaluation and counseling should precede any intervention. Specifically, the surgeon should be cognizant of the patient's skeletal structure, soft tissue distribution, muscular anatomy, and skin quality. Appropriate postoperative care and management of complications are vital to success.
[...]

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

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Ideal Facial Relationships and Goals

Facial plast Surg 2018; 34: 458-465
DOI: 10.1055/s-0038-1669401

Analysis of the face is an essential component of facial plastic surgery. In training, we are taught standards and ideals based on neoclassical models of beauty from Greek and Roman art and architecture. In practice, we encounter a wide range of variation in patient desires and perceptions of beauty. Our goals seem to be ever shifting, yet our education has provided us with a foundation from which to draw ideals of beauty. Plastic surgeons must synthesize classical ideas of beauty with patient desires, cultural nuances, and ethnic considerations all the while maintaining a natural appearance and result. This article gives an overview of classical models of facial proportions and relationships, while also discussing unique ethnic and cultural considerations which may influence the goal for the individual patient.
[...]

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Selecting the Best Eyelid Techniques

Facial plast Surg 2018; 34: 497-504
DOI: 10.1055/s-0038-1672148

The periorbital region is a focal point on the face. Dermatochalasis, fat herniation, blepharoptosis, brow ptosis, deep tear troughs, and malar fat pad descent are all age-related changes that occur around the eyes, and successful rejuvenation involves addressing all of these changes. Restoring a youthful, vibrant appearance to the eyes and periorbital areas can often only be accomplished with a combination of surgical and nonsurgical interventions. Facial plastic surgeons should have a comprehensive knowledge of all of these options and their applications to ensure good patient outcomes and satisfaction.
[...]

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Rejuvenation of the Midface

Facial plast Surg 2018; 34: 505-523
DOI: 10.1055/s-0038-1672161

In this article, the interested reader will learn when and how to apply different techniques on their patients, with the goal of safe, effective, natural looking, and long-lasting midface rejuvenation.
[...]

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

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Comparison of an indirect impression scanning system and two direct intraoral scanning systems in vivo

Abstract

Objectives

This in vivo study aimed to compare the marginal discrepancies of zirconia copings manufactured on the basis of two direct intraoral scanning systems and the indirect digitization of a conventional impression.

Materials and methods

A total of 63 teeth in 23 patients were prepared to receive full-coverage crowns. Subsequently, these teeth were intraorally scanned using CEREC AC Omnicam and Cara TRIOS and a conventional impression was taken with the scannable PVS Flexitime Fast & Scan. The conventional impression was then extraorally digitized using a D700 laboratory scanner. The zirconia copings were manufactured on the basis of the resulting datasets. Silicone replicas of the copings were produced and sectioned for the measurement of the marginal discrepancy under a digital microscope.

Results

The statistical analysis showed no significant differences between the two intraoral scanners, the CEREC AC Omnicam (86.09 μm ± 61.46 μm) and the Cara TRIOS (88.95 μm ± 54.46 μm). However, the discrepancies of the zirconia copings obtained from the laboratory scans of conventional impressions (143.29 μm ± 100.71 μm) showed significant differences. Both intraoral scanners achieved a marginal discrepancy below 100 μm, whereas the laboratory scan exhibited considerably higher values.

Conclusions

The intraoral scanners tested allow for the production of single-tooth-restorations with an adequate marginal fit, whereas the production of restorations on the basis of the scan of a conventional impression led to vast marginal gaps.

Clinical relevance

The method of digitizing a conventional impression using a laboratory scanner seemed to have reached its limits in the clinical environment.

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Are dairy products containing probiotics beneficial for oral health? A systematic review and meta-analysis

Abstract

Objectives

To evaluate the effect of probiotic containing in dairy products on oral and salivary parameters.

Material and methods

Electronic searches were performed based on the PICO criteria. Randomized controlled trials in adults/children (P) in which dairy products containing probiotics (I) were compared to controls (C) for establishment of oral health (O) were included. Risk of bias and data extraction were performed. The meta-analysis evaluated the influence of probiotics on Streptococcus mutans (SM), Lactobacillus spp. (LB), and yeast counts, plaque index, and salivary pH. Random or fixed effect models were used, and heterogeneity was tested.

Results

Thirty-two studies were included in the qualitative synthesis, and 24 in the meta-analysis. For dichotomous results, individuals who consumed probiotics exhibited decreased scores in SM concentrations (RD 0.15 [0.02, 0.28] p = 0.03, I² = 70%), but no change was observed for LB (RD 0.10 [− 0.03, 0.23] p = 0.13, I² = 94%), or yeast levels (RD 0.05 [− 0.02, 0.12] p = 0.7, I² = 48%). For continuous analysis, both probiotic and control groups presented similar results for SM and LB (MD − 0.55 [− 1.16, 0.07] p = 0.08, I² = 62%, and RD 0.10 [− 0.03, 0.23] p = 0.13, I² = 94%, respectively). The consumption of probiotics increased salivary pH (MD 0.20 [0.04, 0.35] p = 0.01, I² = 46%) and plaque index (MD 0.38 [0.11, 0.65] p = 0.005, I² = 0%). The probiotic milk reduced SM (MD − 0.59 [− 0.96, − 0.22] p = 0.002, I² = 0%), and the probiotic yogurt decreased LB counts (RD 0.35 [0.19, 0.51] p < 0.0001, I² = 0%).

Conclusions

In general, dairy probiotics were effective in reducing SM, increasing salivary pH, and promoting a higher plaque index. Liquid matrices, such as milk and yogurt, seem to be more effective in reducing SM and LB levels.

Clinical relevance

Consumption of dairy products containing probiotics could be a supporting method in caries prevention.

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Button Battery Safety

The pediatric button battery (BB) hazard has been recognized for several decades. In 2012, the National Button Battery Task Force was established, and most manufacturers have improved warning labels, more secure packaging, and made BB compartments in products are more secure. Tissue neutralization before BB removal (ie, honey or sucralfate/Carafate®) is an effective way to reduce the rate of BB injury. In absence of visible perforation, 0.25% sterile acetic acid esophageal tissue irrigation at time of BB removal is recommended as a neutralization strategy to mitigate injury progression. Future BB design changes could eliminate esophageal tissue injury.

https://ift.tt/2RBqpEs

Improved patient adherence to subcutaneous allergen immunotherapy using a modified rush immunotherapy protocol

Despite the efficacy of allergen immunotherapy (AIT), studies demonstrate low compliance rates.1 Adherence during build-up is especially challenging for patients. Rush immunotherapy (RIT) provides a faster method to reach maintenance dose, eliminating several months of build-up, potentially leading to higher patient adherence and accelerated symptom control. Few studies have directly compared the compliance rates of RIT schedules vs conventional schedules in patients from the same office.2 Safety and increased frequency of reaction rate continue to be primary concerns of RIT.

https://ift.tt/2OdKPoL

Anaphylaxis After Anal Intercourse With Tolerance By Vaginal Route

Anaphylaxis induced by coitus is an uncommon phenomenon 1, 2. The true prevalence is unknown, but it is believed to affect up to 40,000 women in the United States 3. The clinical features may vary in severity from mild immediate reactions as vaginal itching after coitus to systemic reactions such as systemic urticaria or even anaphylactic shock 1. The main agent involved in the few reported cases is seminal fluid, although cases of hypersensitivity to other agents such as latex, spermicidal agents, foods, drugs or lubricants have been reported.

https://ift.tt/2QCzOtT

Long-term scopolamine treatment and dental caries

Abstract

Objectives

To investigate whether scopolamine, an anticholinergic agent which induces hyposalivation, represents a risk factor for the occurrence of dental caries.

Materials and methods

A retrospective cohort study was carried out among sailors treated with scopolamine for seasickness. The study population included 370 young healthy male adults (18–30 years old) who served in the Israel Navy between 2012 and 2016. Of these, 66 subjects who were chronically treated with intermittent administration of scopolamine, either by the oral or transdermal route, were assigned to the study group. Documented subject characteristics included age, socioeconomic status, level of education, body mass index, smoking history, and dental hygiene. Follow-up lasted 1 to 3.5 years.

Results

Two- to 3.5-year follow-up revealed a higher risk of dental caries in 15 of 16 subjects (93.8%) treated with an average of 50.9 mg scopolamine, in contrast to only 71 of 108 control subjects (65.7%) (RR = 1.43, p = 0.02 [95% CI = 1.18–1.72]). Follow-up for 1–1.5 years revealed a lower occurrence of dental caries in both the study group (11/22, 50.0%) and the control group (46/104, 44.2%). Follow-up of 1.5–2 years also revealed less dental caries, in 16/28 subjects (57.1%) in the study group and 51/92 subjects (55.4%) in the control group. The differences were not statistically significant.

Conclusions

In healthy young adults, prolonged intermittent use of scopolamine was found to be a risk factor for the development of dental caries.

Clinical significance

Dental care and hygiene should be intensified when administering hyposalivatory anticholinergic agents.

https://ift.tt/2yjteks

Ion release, antimicrobial and physio-mechanical properties of glass ionomer cement containing micro or nanosized hexametaphosphate, and their effect on enamel demineralization

Abstract

Objectives

To evaluate the effects of hexametaphosphate microparticles (mHMP) or nanoparticles (nHMP) incorporated in glass ionomer cement (GIC) on antimicrobial and physico-mechanical properties, fluoride (F) release, and enamel demineralization.

Material and methods

HMP solutions were obtained at concentrations of 1, 3, 6, 9, and 12%, for screening of antimicrobial activity. Next, mHMP or nHMP at 6, 9, and 12% were incorporated into a resin-modified GIC and the antibacterial activity was evaluated. The resistance to diametral tensile and compressive strength, surface hardness, and degree of monomer conversion as well as F and HMP releases of GICs were determined. Furthermore, specimens were attached to enamel blocks and submitted to pH-cycling, and mineral loss was determined. Parametric and non-parametric tests were performed, after checking data homoscedasticity (p < 0.05).

Results

HMP solutions at 6, 9, and 12% demonstrated the best antibacterial activity. GIC containing HMP showed better antibacterial effects at 9 and 12% for nHMP. Regarding F and HMP releases, the highest levels of release occurred for groups containing 9 and 12% nHMP. With the increase in HMP concentration, there was lower mineral loss. However, the incorporation of mHMP or nHMP in GIC reduced values of physico-mechanical properties when compared to the control GIC.

Conclusions

nHMP improves antimicrobial activity and fluoride release, and decreases enamel demineralization, but reduces the physico-mechanical properties of GIC.

Clinical relevance

The association of GIC/HMP could be an alternative material for patients at high risk for dental caries and could be indicated for low-stress regions or provisional restorations.

https://ift.tt/2Cxow7s

Letter about: nocturnal enuresis in children between laser acupuncture and medical treatment—a comparative study

https://ift.tt/2C3FMQw

Does clinical outcome of birch pollen immunotherapy relate to induction of blocking antibodies preventing IgE from allergen binding? A pilot study monitoring responses during first year of AIT

The clinical benefit of allergen-specific immunotherapy (AIT) involves induction of blocking antibodies. It is not clear if these antibodies function via steric hindrance alone or a combination of levels, avid...

https://ift.tt/2OPXcH4

CPAP-Nutzungsprobleme und Zweitlinientherapie bei obstruktiver Schlafapnoe

https://ift.tt/2E38Vho

Diagnosen von Schilddrüsenerkrankungen in HNO-Praxen und Hausarztpraxen in Deutschland 2008–2016

Zusammenfassung

Zielsetzung

Das Ziel dieser Studie war die Untersuchung von Diagnosen von Schilddrüsenerkrankungen aus deutschen HNO-Praxen im Vergleich zu Hausarztpraxen mithilfe von Daten aus einer repräsentativen deutschlandweiten Praxisdatenbank.

Methode

Die Datenbank wurde retrospektiv nach Diagnosen von Schilddrüsenerkrankungen aus HNO-Praxen und Hausarztpraxen zwischen Januar 2008 und Dezember 2016 durchsucht. Erhoben wurden die häufigsten dreistelligen Krankheitsklassen (Kategorien) aus der Gruppe „Krankheiten der Schilddrüse" (E00–E07) sowie die Kategorien „Gutartige Neubildung der Schilddrüse" (D34) und „Bösartige Neubildung der Schilddrüse" (C73). Ausgewertet wurden die Zeiträume 2008–2010, 2011–2013 sowie 2014–2016.

Ergebnisse

Die Datenbank enthielt 71 HNO-Praxen und 506 Hausarztpraxen mit kontinuierlicher Mitarbeit von 2008–2016. Die relative Häufigkeit (Patienten pro Praxis) von Diagnosen aus der Gruppe „Erkrankungen der Schilddrüse" war in Hausarztpraxen 4,4-mal höher als in HNO-Praxen. Die relative Häufigkeit von gutartigen Neubildungen der Schilddrüse war in Hausarztpraxen 5‑mal höher als in HNO-Praxen. Die relative Häufigkeit von bösartigen Neubildungen der Schilddrüse war in Hausarztpraxen und HNO-Praxen in etwa gleich hoch. Die häufigsten Diagnosen stammten sowohl in HNO-Praxen als auch in Hausarztpraxen aus den Kategorien „Sonstige nichttoxische Struma" (E04) und „Sonstige Hypothyreose" (E03). Weniger häufig waren Diagnosen aus den Kategorien „Hyperthyreose" (E05) und „Thyreoiditis" (E06). Die geringste Häufigkeit wiesen Diagnosen von Neubildungen der Schilddrüse auf, wobei in Hausarztpraxen Diagnosen von gutartigen Neubildungen, in HNO-Praxen hingegen Diagnosen von bösartigen Neubildungen der Schilddrüse überwogen. Im Verlauf des Studienzeitraums nahm die Diagnosehäufigkeit von Schilddrüsenerkrankungen in HNO-Praxen ab, wohingegen sie in Hausarztpraxen zunahm.

Schlussfolgerung

Trotz des in den letzten Jahren in Deutschland deutlich zunehmenden Interesses an der Etablierung der Schilddrüsenchirurgie in HNO-Kliniken zeigt sich ein kontinuierlicher Rückgang von Schilddrüsendiagnosen in deutschen HNO-Praxen bei gleichzeitiger deutlicher Zunahme in Hausarztpraxen. Ursächlich hierfür könnten kassenärztliche Vergütungsunterschiede zwischen hausärztlicher und fachärztlicher Tätigkeit sein.

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The microgenderome revealed: sex differences in bidirectional interactions between the microbiota, hormones, immunity and disease susceptibility

Abstract

Sex differences in immunity are well described in the literature and thought to be mainly driven by sex hormones and sex-linked immune response genes. The gastrointestinal tract (GIT) is one of the largest immune organs in the body and contains multiple immune cells in the GIT-associated lymphoid tissue, Peyer's patches and elsewhere, which together have profound effects on local and systemic inflammation. The GIT is colonised with microbial communities composed of bacteria, fungi and viruses, collectively known as the GIT microbiota. The GIT microbiota drives multiple interactions locally with immune cells that regulate the homeostatic environment and systemically in diverse tissues. It is becoming evident that the microbiota differs between the sexes, both in animal models and in humans, and these sex differences often lead to sex-dependent changes in local GIT inflammation, systemic immunity and susceptibility to a range of inflammatory diseases. The sexually dimorphic microbiome has been termed the 'microgenderome'. Herein, we review the evidence for the microgenderome and contemplate the role it plays in driving sex differences in immunity and disease susceptibility. We further consider the impact that biological sex might play in the response to treatments aimed at manipulating the GIT microbiota, such as prebiotics, live biotherapeutics, (probiotics, synbiotics and bacteriotherapies) and faecal microbial transplant. These alternative therapies hold potential in the treatment of both psychological (e.g., anxiety, depression) and physiological (e.g., irritable bowel disease) disorders differentially affecting males and females.

https://ift.tt/2INxCgI

Developmental origin and sex-specific risk for infections and immune diseases later in life

Abstract

The intrauterine environment is an important determinant of immunity later in life of the offspring. An altered prenatal immune development can result in a high postnatal risk for infections, chronic immune diseases, and autoimmunity. Many of these immune diseases show a strong sex bias, such as a high incidence of autoimmune diseases and allergies in adult females or a high risk for infections in males. Here, we comprehensively review established pathways and propose novel concepts modulating the risk for such poor immunity during childhood and throughout life. Moreover, we highlight how an adverse fetal environment may affect or aggravate the risk for poor immunity in a sex-specific manner. An improved understanding of a sex-specific susceptibility to poor immunity along with insights on how such risk can be modulated before or around birth will allow the development of tailored prevention strategies.

https://ift.tt/2CwaW41

Sex and sex steroids impact influenza pathogenesis across the life course

Abstract

Males and females differ in the outcome of influenza A virus (IAV) infections, which depends significantly on age. During a typical seasonal influenza epidemic, young children (< 10 years of age) and aged adults (65+ years of age) are at greatest risk for severe disease, and among these age groups, males tend to suffer a worse outcome from IAV infection than females. Following infection with either pandemic or outbreak strains of IAVs, females of reproductive ages (i.e., 15–49 years of age) experience a worse outcome than their male counterparts. Among females of reproductive ages, pregnancy is one factor linked to an increased risk of severe outcome of influenza, although it is not the sole factor explaining the female-preponderance of severe disease. Small animal models of influenza virus infection illustrate that inflammatory immune responses and repair of damaged tissue following IAV infection also differ between the sexes and impact the outcome of infection. There also is growing evidence that sex steroid hormones, including estrogens, progesterone, and testosterone, directly impact immune responses during IAV infection to alter outcomes. Greater consideration of the combined effects of sex and age as biological variables in epidemiological, clinical, and animal studies of influenza pathogenesis is needed.

https://ift.tt/2IJJboX

Successful Management of Blue Rubber Bleb Nevus Syndrome (BRBNS) with Sirolimus

Blue rubber bleb nevus syndrome (BRBNS) is a rare disease with vascular malformations in several systems of the body, most commonly the skin and gastrointestinal tract. Bleeding from the gastrointestinal (GI) tract is a major complication, which may lead to chronic iron deficiency anemia and the need for frequent blood transfusions due to ongoing gastrointestinal blood loss. In this case report, we describe a now 19-year-old female with BRBNS who required six blood transfusions per year and after starting sirolimus is symptom- and transfusion-free.

https://ift.tt/2NsL2zr

Serum miRNA-371b-5p and miRNA-5100 act as biomarkers for systemic lupus erythematosus

Publication date: Available online 7 October 2018

Source: Clinical Immunology

Author(s): Li Zeng, Jia-li Wu, Li-min Liu, Ju-qing Jiang, Hai-jing Wu, Ming Zhao, Qian-jin Lu

Abstract

MicroRNAs (miRNAs) play important roles in the pathogenesis of systemic lupus erythematosus (SLE). Here, we investigated the serum miRNAs expression profiles in the serum of SLE and healthy controls, and identified the potential serum biomarkers for SLE. We screened and identified the differentially expressed miRNAs such as miR-371b-5p, miR-5100, miR-146a-5p among active SLE, inactive SLE and healthy controls based on the miRNAs expression array. Furthermore, the results of RT-qPCR confirmed that miR-371b-5p and miR-5100 expression was different among active SLE, inactive SLE and healthy controls. Moreover, we performed in a large cohort which we validated that expression of miR-371b-5p and miR-5100 was increased significantly in the serum of SLE compared with healthy controls and rheumatoid arthritis (RA), and was also higher in active SLE than that in inactive SLE. In addition, we found the associations between the expression levels of miR-371b-5p and miR-5100 and these clinical parameters of SLE. These results suggested that miR-371b-5p and miR-5100 may act as serum biomarkers for SLE.

https://ift.tt/2ynkutQ

Early (90‐day) mortality after radical radiotherapy for head and neck squamous cell carcinoma: A population‐based analysis

Head &Neck, EarlyView.


https://ift.tt/2ylhUof

Massive retroperitoneal dedifferentiated liposarcoma in a young patient

Abstract

Liposarcomas are rare malignant tumors that mostly develop in the retroperitoneum. They have a broad behavioral spectrum, from small masses of tissue to highly aggressive tumors. The dedifferentiation process occurs in up to 10% and it's most likely to occur in the retroperitoneum, a process that not only changes its components but also its prognosis. These tumors can grow to a massive size since most of them do not give any symptoms until they invade the adjacent structures. Timely detection and surgery could avoid all these potentially lethal scenarios. We present a case of a 34-year-old patient, who reported a growing mass in her abdomen that reached massive proportions but remained untreated due to lack of sufficient access to healthcare facilities in her geographic location. After complete removal of the mass the patient underwent complete recovery, dedifferentiated liposarcoma was the final diagnosis.

https://ift.tt/2PmSUUL

Synchronous malignant phyllodes tumour and invasive lobular carcinoma—case report and review

Abstract

Synchronous phyllodes tumour and invasive lobular carcinoma is an extremely rare event. We report these concurrent diagnoses in a patient observed in an ipsilateral breast, suspected due to breast risk factors (family history and lobular carcinoma in situ) and the presence of malignant phyllodes. Screening breast magnetic resonance imaging was able to identify the carcinoma which was occult in other imaging. An understanding of the possibility of dual diagnoses may lead to additional investigations for its identification. Treatment may then be tailored to the individual's pathology.

https://ift.tt/2OJZHe6

Giant myxoid liposarcoma of the gluteal region: case report of patient caused delay of surgical treatment and review of the literature

Abstract

Although adult soft tissue sarcoma is a rare disease, it needs individual treatment by an experienced, interdisciplinary team. We present an exceptional case of a 36-year-old woman suffering from a giant intermediate grade myxoid liposarcoma of the left buttock. She had been seen 4 years earlier but refused to undergo any treatment by then. Now suffering from a foul, ulcerating and superinfected tumor she agreed to surgical treatment. Despite delay, treatment could be performed according to the most up to date sarcoma guidelines which are discussed, including a brief review of the literature.

https://ift.tt/2Pk1DXK

Spontaneous resolution of a spontaneous steinstrasse: a case report

Abstract

A 63-year-old man presented with a 4-day history of right sided loin-to-groin pain. Computed tomography imaging revealed a 22 mm by 7 mm volume of contiguous ureteric calculi at the right vesicoureteric junction. Spontaneous steinstrasse was diagnosed with no recent history of extracorporeal shock wave lithotripsy (ESWL) or other urological intervention to the right kidney. Metabolic testing was negative. An initial plan was made for urgent primary ureteroscopy and lithotripsy, however, the patient spontaneously passed 20–25 calculi at home whilst awaiting his operation date. He was reviewed in clinic and his symptoms had resolved. His ureteroscopy was cancelled. This case represents an example of spontaneous steinstrasse with no identified causative factors, a rare occurrence on which little literature is available presenting a management dilemma to the treating clinician. The case described resolved prior to intervention prompting us to suggest that spontaneous steinstrasse can be initially managed conservatively, as in ESWL-associated steinstrasse.

https://ift.tt/2OMQ4va

Trans-luminal repair of a ruptured AAA with Type Ia and Type II endoleaks

Abstract

Ruptured abdominal aortic aneurysm (rAAA) with an associated Type II endoleak is rare. Emergent surgical repair is often necessary and may be associated with high morbidity and mortality. We report an alternative unique trans-luminal repair strategy in an 84-year-old male who presented with a rAAA with prior EVAR, and Type Ia and Type II endoleaks. The operative strategy consisted of proximal endograft extension into the para-renal aorta, followed by staged sac embolization using glue. Postoperatively, the patient recovered well from the repair, and follow-up imaging demonstrated a stable repair.

https://ift.tt/2PmlTIr

A case of type II Mirizzi syndrome treated by simple endoscopic means

Abstract

Mirizzi syndrome is an uncommon complication of chronic cholelithiasis. Advancement in radiological modalities and minimally invasive surgery has led to improved pre-operative diagnoses and more laparoscopic cholecystectomies. But for unsuitable surgical candidates, endoscopy can be the definitive treatment. In this case, we present a 67-year-old man with type II Mirizzi syndrome treated by simple endoscopic means.

https://ift.tt/2OLzjk7

Abnormal Microvasculature in Laryngectomy Mucosal Margins may be Associated with Increased Risk of Fistula

Abstract

Pharyngocutaneous fistula after laryngectomy is common and significantly increases the morbidity of the procedure. Intraoperative, objective variables that can reliably predict fistula formation would be useful to surgeons deciding how to reconstruct the laryngectomy defect. Retrospective chart review of 50 radiated patients and 10 non-radiated patients who underwent total laryngectomy at a single tertiary care institution. Patients with pharyngocutaneous fistula were selected to ensure a representative sample were available for comparison. All patients had pathology slides available for re-review by a single, blinded pathologist. Margins of both radiated (n = 50) and non-radiated (n = 10) larynges were examined for 7 histologic features, and odds ratios were calculated to assess whether these features were associated with fistula. When evaluating all 60 patients, both telangiectatic capillaries and hyalinized arterioles were associated with fistula (OR 3.72 and 9.21, respectively). Collinearity between the variables was evaluated; findings indicated a high likelihood of having hyalinized arterioles if telangiectatic capillaries were also present (OR 31.67 [3.13, 320.06]). Microvascular changes in radiated tissue have previously been described in other anatomic subsites, but the larynx and pharynx have not been specifically evaluated. Laryngectomy mucosal margins appear to display similar changes, and evidence of this damage may be associated with fistula formation. These features could potentially guide the surgeon to alter the reconstructive technique.

https://ift.tt/2NuK9WM

Abnormal Microvasculature in Laryngectomy Mucosal Margins may be Associated with Increased Risk of Fistula

Abstract

Pharyngocutaneous fistula after laryngectomy is common and significantly increases the morbidity of the procedure. Intraoperative, objective variables that can reliably predict fistula formation would be useful to surgeons deciding how to reconstruct the laryngectomy defect. Retrospective chart review of 50 radiated patients and 10 non-radiated patients who underwent total laryngectomy at a single tertiary care institution. Patients with pharyngocutaneous fistula were selected to ensure a representative sample were available for comparison. All patients had pathology slides available for re-review by a single, blinded pathologist. Margins of both radiated (n = 50) and non-radiated (n = 10) larynges were examined for 7 histologic features, and odds ratios were calculated to assess whether these features were associated with fistula. When evaluating all 60 patients, both telangiectatic capillaries and hyalinized arterioles were associated with fistula (OR 3.72 and 9.21, respectively). Collinearity between the variables was evaluated; findings indicated a high likelihood of having hyalinized arterioles if telangiectatic capillaries were also present (OR 31.67 [3.13, 320.06]). Microvascular changes in radiated tissue have previously been described in other anatomic subsites, but the larynx and pharynx have not been specifically evaluated. Laryngectomy mucosal margins appear to display similar changes, and evidence of this damage may be associated with fistula formation. These features could potentially guide the surgeon to alter the reconstructive technique.

https://ift.tt/2NuK9WM

Abnormal Microvasculature in Laryngectomy Mucosal Margins may be Associated with Increased Risk of Fistula

Abstract

Pharyngocutaneous fistula after laryngectomy is common and significantly increases the morbidity of the procedure. Intraoperative, objective variables that can reliably predict fistula formation would be useful to surgeons deciding how to reconstruct the laryngectomy defect. Retrospective chart review of 50 radiated patients and 10 non-radiated patients who underwent total laryngectomy at a single tertiary care institution. Patients with pharyngocutaneous fistula were selected to ensure a representative sample were available for comparison. All patients had pathology slides available for re-review by a single, blinded pathologist. Margins of both radiated (n = 50) and non-radiated (n = 10) larynges were examined for 7 histologic features, and odds ratios were calculated to assess whether these features were associated with fistula. When evaluating all 60 patients, both telangiectatic capillaries and hyalinized arterioles were associated with fistula (OR 3.72 and 9.21, respectively). Collinearity between the variables was evaluated; findings indicated a high likelihood of having hyalinized arterioles if telangiectatic capillaries were also present (OR 31.67 [3.13, 320.06]). Microvascular changes in radiated tissue have previously been described in other anatomic subsites, but the larynx and pharynx have not been specifically evaluated. Laryngectomy mucosal margins appear to display similar changes, and evidence of this damage may be associated with fistula formation. These features could potentially guide the surgeon to alter the reconstructive technique.

https://ift.tt/2NuK9WM

Torticollis as Presentation for Atypical Kawasaki Disease Complicated by Giant Coronary Artery Aneurysms

Kawasaki disease (KD) is an acute systemic vasculitis of childhood. The diagnosis can be made in a patient who presents with a prolonged high fever and meeting at least four of five criteria including polymorphous rash, mucosal changes, extremity changes (including swelling and/or palmar and plantar erythema), bilateral nonsuppurative conjunctivitis, and unilateral cervical lymphadenopathy. Atypical KD refers to patients who have not met the full criteria and in whom atypical features may be present. We discuss a case of a 6-year-old male who presented to the Emergency Department with torticollis. A series of investigations for elevated inflammatory markers revealed dilated coronary artery aneurysms on echocardiogram, and thus he was diagnosed with atypical KD. His only other criteria were bilateral nonsuppurative conjunctivitis and a prior brief febrile illness. He was treated with high-dose intravenous immune globulin (IVIG) and low-dose aspirin. Low-molecular-weight heparin and atenolol were added due to the presence of giant aneurysms.

https://ift.tt/2E71TIA

Very early onset IBD: novel genetic aetiologies

Purpose of review To summarize the current understanding and recent advances on the genetic aetiology in the pathogenesis of very early onset inflammatory bowel disease (VEO-IBD). Recent findings IBD is a chronic disorder of the gastrointestinal tract whose manifestation is a result of complex interactions between genetics, environment, immune system and microbial flora. Over 230 IBD risk loci have been reported in genome wide association studies but the genetic contribution of the majority of these loci in the manifestation of IBD is very low. Patients with VEO-IBD present with a more severe disease than older patients, characterized by poor prognosis and failure of conventional therapy. Recent studies have reported several monogenic diseases with high penetrance that present with IBD and IBD-like intestinal manifestations and overlap with primary immunodeficiencies. Increasing body of evidence supports a prominent role of genetics in the onset of VEO-IBD. New genetic variants and diagnoses in VEO-IBD are reviewed and current challenges in therapy with potential strategy to manage the disease are discussed. Summary Functional analysis of the genes implicated in monogenic IBD has increased the understanding of the underlying pathobiological mechanism of the disease. This knowledge can be used to personalize medicine for specific patients, improving the standard of care and quality of life. Correspondence to Aleixo M. Muise, MD, PhD, FRCPC, 555 University Ave, Toronto, ON M5G 1X8. Tel: +416-813-7654 X306171; e-mail: aleixo.muise@sickkids.ca Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

https://ift.tt/2NuDkVh

Biological treatments for severe asthma: where do we stand?

Purpose of review For patients with severe asthma, disease control is not achieved resulting in persistent morbidity and risks for exacerbations. The advent of biologics is providing a new form of treatment for many with severe asthma. Recent findings Four mAb biologics are approved for clinical use: omalizumab (anti-IgE) and three antieosinophilic interventions (mepolizumab, reslizumab, and benralizumab). These four biologics target components of the type 2-inflammatory pathway which is reflected by biomarkers: peripheral blood eosinophils and exhaled nitric oxide. In severe asthma, biologics have reduced asthma exacerbations. The antieosinophilic biologics have also improved lung function. The safety profile of these biologics has been good. Summary For patients with severe asthma and biomarkers indicating a type 2 inflammatory pathway, the addition of biologics has proven to be an effective approach to achieve disease control and is an appropriate next step treatment. Correspondence to William W. Busse, MD, Professor of Medicine, University of Wisconsin Hospitals and Clinics, 600 Highland Avenue, Madison, WI 53792, USA. E-mail: wwb@medicine.wisc.edu Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

https://ift.tt/2y6nxaf

Very early-onset inflammatory bowel disease: an integrated approach

Purpose of the review Immune dysregulation disorders are among the most rapidly growing set of inborn errors of immunity. One particular subset is the category where early-onset inflammatory bowel disease (IBD) is the most common manifestation. These disorders are being increasingly appreciated although there has been minimal effort to articulate a unified approach to their diagnosis and management. This review will cover current thinking and strategies related to diagnosis and management of very early-onset IBD. Recent findings There is an expanding set of monogenic causes of early-onset IBD. In many cases, the precise genetic cause dictates management. Lessons learned from the management of these monogenic conditions can sometimes be extrapolated to other refractory cases of IBD. Summary An integrated approach to diagnosis, risk analysis, and management can include diagnostic approaches not often utilized for traditional IBD such as whole exome sequencing. Management can also include nontraditional approaches such as targeted biologics or hematopoietic cell transplantation. Correspondence to Kathleen E. Sullivan, MD, PhD, Immunology, ARC 1216-I, The Children's Hospital of Philadelphia, 3615 Civic Center Blvd., Philadelphia, PA 19104, USA. Tel: +1 215 590 1697; fax: +1 267 426 0363; e-mail: sullivank@email.chop.edu Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

https://ift.tt/2NusApP

Secondary antibody deficiency in neurology

Purpose of review Induction of lymphocyte depletion is increasingly used as a therapeutic strategy for central and peripheral neuroinflammatory disease. However, there is also a growing recognition of the treatment-related complication of secondary antibody deficiency (SAD). Although the occurrence of hypogammaglobulinaemia is a recognized phenomenon during immunomodulation, robust data on the coexistence of impaired responses to immunization, and significant and/or atypical infections is scarce. Here we review the literature on SAD in anti-CD20 therapy. Recent findings Several factors that may increase the incidence of SAD have now been identified, including low levels of immunoglobulins prior to the commencement of B-cell ablation therapy, duration of maintenance therapy, and concurrent or prior use of other immunosuppressing agents such as cyclophosphamide and steroids. Measurement of disease-specific antibodies and vaccine response are likely to be helpful adjuncts to measurement of serum immunoglobulin levels during B-cell depleting therapy. Supportive treatment may include amending the treatment schedule to limit cumulative dose. Summary B-cell depleting agents offer considerable therapeutic benefit in neurology. We propose modifications in current practice that include risk stratification and early identification of SAD, with the aim of minimising morbidity and mortality related to this underappreciated condition. Correspondence to Dr Emma C. Tallantyre, Department of Psychological Medicine and Clinical Neurosciences, B4-C4 Corridor, University Hospital of Wales, Cardiff, CF14 4XW, Wales, UK. Tel: +44 02920 745403; e-mail: tallantyreec@cardiff.ac.uk Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

https://ift.tt/2y8uR5f

Advances in site-specific gene editing for primary immune deficiencies

Purpose of review Conventional gene therapy has been a successful, curative treatment modality for many primary immune deficiencies with significant improvements in the last decade. However, the risk of leukemic transformation with viral-mediated gene addition still remains, and unregulated gene addition is not an option for certain diseases in which the target gene is closely controlled. The recent bloom in genome modification platforms has created the opportunity to site-specifically correct mutated DNA base pairs or insert a corrective cDNA minigene while maintaining gene expression under control of endogenous regulatory elements. Recent findings There is an abundance of ongoing research utilizing programmable nucleases to facilitate site-specific gene correction of many primary immune deficiencies including X-linked severe combined immune deficiency, X-linked chronic granulomatous disease, Wiskott–Aldrich syndrome, X-linked hyper-IgM syndrome, X-linked agammaglobulinemia, and immune dysregulation, polyendocrinopathy, enteropathy, X-linked. In all, these studies have demonstrated the ability to integrate corrective DNA sequences at a precise location in the genome at rates likely to either cure or ameliorate disease. Summary Gene editing for primary immune deficiency (PID) has advanced to the point to that translation to clinical trials is likely to occur in the next several years. At the current pace of research in DNA repair mechanisms, stem cell biology, and genome-editing technology, targeted genome modification represents the next chapter of gene therapy for PID. Correspondence to Caroline Y. Kuo, MD, Division of Allergy and Immunology, Department of Pediatrics, 10833 Le Conte, MDCC 12-430, Los Angeles, CA 90095, USA. Tel: +1 310 825 6481; fax: +1 310 825 9832; e-mail: ckuo@mednet.ucla.edu Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

https://ift.tt/2NpC6e5

Very early onset IBD: novel genetic aetiologies

Purpose of review To summarize the current understanding and recent advances on the genetic aetiology in the pathogenesis of very early onset inflammatory bowel disease (VEO-IBD). Recent findings IBD is a chronic disorder of the gastrointestinal tract whose manifestation is a result of complex interactions between genetics, environment, immune system and microbial flora. Over 230 IBD risk loci have been reported in genome wide association studies but the genetic contribution of the majority of these loci in the manifestation of IBD is very low. Patients with VEO-IBD present with a more severe disease than older patients, characterized by poor prognosis and failure of conventional therapy. Recent studies have reported several monogenic diseases with high penetrance that present with IBD and IBD-like intestinal manifestations and overlap with primary immunodeficiencies. Increasing body of evidence supports a prominent role of genetics in the onset of VEO-IBD. New genetic variants and diagnoses in VEO-IBD are reviewed and current challenges in therapy with potential strategy to manage the disease are discussed. Summary Functional analysis of the genes implicated in monogenic IBD has increased the understanding of the underlying pathobiological mechanism of the disease. This knowledge can be used to personalize medicine for specific patients, improving the standard of care and quality of life. Correspondence to Aleixo M. Muise, MD, PhD, FRCPC, 555 University Ave, Toronto, ON M5G 1X8. Tel: +416-813-7654 X306171; e-mail: aleixo.muise@sickkids.ca Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

https://ift.tt/2NuDkVh

Biological treatments for severe asthma: where do we stand?

Purpose of review For patients with severe asthma, disease control is not achieved resulting in persistent morbidity and risks for exacerbations. The advent of biologics is providing a new form of treatment for many with severe asthma. Recent findings Four mAb biologics are approved for clinical use: omalizumab (anti-IgE) and three antieosinophilic interventions (mepolizumab, reslizumab, and benralizumab). These four biologics target components of the type 2-inflammatory pathway which is reflected by biomarkers: peripheral blood eosinophils and exhaled nitric oxide. In severe asthma, biologics have reduced asthma exacerbations. The antieosinophilic biologics have also improved lung function. The safety profile of these biologics has been good. Summary For patients with severe asthma and biomarkers indicating a type 2 inflammatory pathway, the addition of biologics has proven to be an effective approach to achieve disease control and is an appropriate next step treatment. Correspondence to William W. Busse, MD, Professor of Medicine, University of Wisconsin Hospitals and Clinics, 600 Highland Avenue, Madison, WI 53792, USA. E-mail: wwb@medicine.wisc.edu Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

https://ift.tt/2y6nxaf

Very early-onset inflammatory bowel disease: an integrated approach

Purpose of the review Immune dysregulation disorders are among the most rapidly growing set of inborn errors of immunity. One particular subset is the category where early-onset inflammatory bowel disease (IBD) is the most common manifestation. These disorders are being increasingly appreciated although there has been minimal effort to articulate a unified approach to their diagnosis and management. This review will cover current thinking and strategies related to diagnosis and management of very early-onset IBD. Recent findings There is an expanding set of monogenic causes of early-onset IBD. In many cases, the precise genetic cause dictates management. Lessons learned from the management of these monogenic conditions can sometimes be extrapolated to other refractory cases of IBD. Summary An integrated approach to diagnosis, risk analysis, and management can include diagnostic approaches not often utilized for traditional IBD such as whole exome sequencing. Management can also include nontraditional approaches such as targeted biologics or hematopoietic cell transplantation. Correspondence to Kathleen E. Sullivan, MD, PhD, Immunology, ARC 1216-I, The Children's Hospital of Philadelphia, 3615 Civic Center Blvd., Philadelphia, PA 19104, USA. Tel: +1 215 590 1697; fax: +1 267 426 0363; e-mail: sullivank@email.chop.edu Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

https://ift.tt/2NusApP

Secondary antibody deficiency in neurology

Purpose of review Induction of lymphocyte depletion is increasingly used as a therapeutic strategy for central and peripheral neuroinflammatory disease. However, there is also a growing recognition of the treatment-related complication of secondary antibody deficiency (SAD). Although the occurrence of hypogammaglobulinaemia is a recognized phenomenon during immunomodulation, robust data on the coexistence of impaired responses to immunization, and significant and/or atypical infections is scarce. Here we review the literature on SAD in anti-CD20 therapy. Recent findings Several factors that may increase the incidence of SAD have now been identified, including low levels of immunoglobulins prior to the commencement of B-cell ablation therapy, duration of maintenance therapy, and concurrent or prior use of other immunosuppressing agents such as cyclophosphamide and steroids. Measurement of disease-specific antibodies and vaccine response are likely to be helpful adjuncts to measurement of serum immunoglobulin levels during B-cell depleting therapy. Supportive treatment may include amending the treatment schedule to limit cumulative dose. Summary B-cell depleting agents offer considerable therapeutic benefit in neurology. We propose modifications in current practice that include risk stratification and early identification of SAD, with the aim of minimising morbidity and mortality related to this underappreciated condition. Correspondence to Dr Emma C. Tallantyre, Department of Psychological Medicine and Clinical Neurosciences, B4-C4 Corridor, University Hospital of Wales, Cardiff, CF14 4XW, Wales, UK. Tel: +44 02920 745403; e-mail: tallantyreec@cardiff.ac.uk Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

https://ift.tt/2y8uR5f

Advances in site-specific gene editing for primary immune deficiencies

Purpose of review Conventional gene therapy has been a successful, curative treatment modality for many primary immune deficiencies with significant improvements in the last decade. However, the risk of leukemic transformation with viral-mediated gene addition still remains, and unregulated gene addition is not an option for certain diseases in which the target gene is closely controlled. The recent bloom in genome modification platforms has created the opportunity to site-specifically correct mutated DNA base pairs or insert a corrective cDNA minigene while maintaining gene expression under control of endogenous regulatory elements. Recent findings There is an abundance of ongoing research utilizing programmable nucleases to facilitate site-specific gene correction of many primary immune deficiencies including X-linked severe combined immune deficiency, X-linked chronic granulomatous disease, Wiskott–Aldrich syndrome, X-linked hyper-IgM syndrome, X-linked agammaglobulinemia, and immune dysregulation, polyendocrinopathy, enteropathy, X-linked. In all, these studies have demonstrated the ability to integrate corrective DNA sequences at a precise location in the genome at rates likely to either cure or ameliorate disease. Summary Gene editing for primary immune deficiency (PID) has advanced to the point to that translation to clinical trials is likely to occur in the next several years. At the current pace of research in DNA repair mechanisms, stem cell biology, and genome-editing technology, targeted genome modification represents the next chapter of gene therapy for PID. Correspondence to Caroline Y. Kuo, MD, Division of Allergy and Immunology, Department of Pediatrics, 10833 Le Conte, MDCC 12-430, Los Angeles, CA 90095, USA. Tel: +1 310 825 6481; fax: +1 310 825 9832; e-mail: ckuo@mednet.ucla.edu Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

https://ift.tt/2NpC6e5

The Versatility of Nasolabial Flaps in Maxillofacial Surgery

Abstract

Introduction

The nasolabial flap can be used for reconstruction of the orofacial region. The procedure is of a short duration and treats orofacial soft tissue defects of small to moderate size, especially when microsurgical free flaps are less feasible due to lack of infrastructure, expertise or financial constraints.

Aims and Objectives

To study the effectiveness of the nasolabial flaps in reconstruction of orofacial defects of various origins.

Materials and Methods

In this prospective study, 10 patients (18 nasolabial flaps) were selected randomly regardless of age, sex and need for reconstruction—whether it being requirement of tissue coverage following traumatic loss of soft tissue, soft tissue coverage after resection of a benign or malignant tumor, or resection of soft tissue defects after excision of submucous fibrosis bands. Patients were treated under general anesthesia following routine investigations, using either inferiorly or superiorly based, unilateral or bilateral nasolabial flaps for local extraoral and intraoral reconstruction. Patients were evaluated for postoperative complications, flap uptake, donor site morbidity and postoperative extraoral scarring.

Results

In all cases, postoperative complications were relatively minor except in one flap where total flap loss was encountered. The average time taken for preparation of recipient site, flap elevation and closure was 72 min. The nasolabial flap proved itself to be extremely vascular and thus safe and suitable with satisfactory cosmetic outcomes.

Conclusion

The nasolabial flap proves to be a versatile flap with a proven blood supply from the facial, transverse facial and infraorbital vessels. It establishes itself as a simple procedure to execute to reconstruct the various defects of the anterior orofacial region. It is considered to be a safe flap with a low complication rate, and most importantly it offers a cosmetically pleasing and functionally satisfactory solution.

https://ift.tt/2Ploz98

Effect of methotrexate monotherapy on T‐cell subsets in the peripheral circulation in psoriasis

Clinical and Experimental Dermatology, EarlyView.


https://ift.tt/2C1TRhq

Linear nail bed dyschromia: a distinctive dermoscopic feature of nail lichen planus

Clinical and Experimental Dermatology, EarlyView.


https://ift.tt/2zYQTsH

Asymptomatic solitary mass on the sacral region

Clinical and Experimental Dermatology, EarlyView.


https://ift.tt/2C4ji24

Acitretin: a promising therapy for localized childhood lichen myxoedematosus

Clinical and Experimental Dermatology, EarlyView.


https://ift.tt/2zY4osp

Hypopigmented patches in childhood: do not forget mycosis fungoides

Clinical and Experimental Dermatology, EarlyView.


https://ift.tt/2C4jazC

Bullous pyoderma gangrenosum secondary to underlying multiple myeloma: treated with ciclosporin

Clinical and Experimental Dermatology, EarlyView.


https://ift.tt/2zY4i41

Sporadic case of Darier disease caused by a novel splice‐site mutation in the ATP2A2 gene

Clinical and Experimental Dermatology, EarlyView.


https://ift.tt/2BZoUKV

A survey of the treatment and management of patients with severe chronic spontaneous urticaria

Clinical and Experimental Dermatology, EarlyView.


https://ift.tt/2zXt2JW

Alteration of serum and tissue tumor necrosis factor alpha levels: A possible mechanism of action of oral pulse steroids in the treatment of alopecia areata

Journal of Cosmetic Dermatology, EarlyView.


https://ift.tt/2NsKcTa

Improvement of alopecia areata with apremilast

Australasian Journal of Dermatology, EarlyView.


https://ift.tt/2QB04oB

Chondrodysplasia punctata (CDPX2) in a male caused by single‐gene mosaicism: A 20‐year follow‐up

Australasian Journal of Dermatology, EarlyView.


https://ift.tt/2O9NUGo

Sentinel lymph node biopsy remains the most accurate method of obtaining staging and prognostic information for patients with primary cutaneous melanomas

Australasian Journal of Dermatology, EarlyView.


https://ift.tt/2QAcrBd

Primary cutaneous lymphoma in Argentina: a report of a nationwide study of 416 patients

International Journal of Dermatology, EarlyView.


https://ift.tt/2C296Hc

Issue Information

International Journal of Dermatology, Volume 57, Issue 11, Page i-iii,1273-1274, November 2018.


https://ift.tt/2zXVECN

Rare case of adult pancreatic haemangioma and literature review

Pancreatic haemangiomas are benign vascular tumours very rare in adults. Twenty-two cases are described in the literature. The symptoms are non-specific, and therefore rarely clinically suspected, and the vast majority are incidental findings in imaging tests such as ultrasound, CT, angiography or MRI. They appear on CT as a cystic lesion with contrast enhancement in the arterial phase. We present the case of a 36-year-old male patient with no history of disease, referred with lumbar pain and suspected renal calculus after tomography showing hypervascular enhancement in the pancreatic body and infiltrative lesion (possible neuroendocrine neoplasia) on MRI and biliopancreatic echoendoscopy. He was submitted to laparotomy with subtotal pancreatectomy and splenectomy and satisfactory evolution.

https://ift.tt/2C2a8D3

Uncommon manifestation of leptospirosis: a diagnostic challenge

Leptospirosis is a zoonotic disease commonly affecting the tropical countries. It may have protean clinical manifestations including hepatorenal dysfunction, myocarditis, pulmonary haemorrhage, meningitis, optic neuritis and rhabdomyolysis. Neurological manifestation of leptospirosis without the classical hepatorenal dysfunction is a rare entity. This complication of leptospirosis can present with diverse central and peripheral neurological presentations. The overlapping clinical manifestations with many common tropical pathogens often pose diagnostic dilemma and delay in definitive therapy may lead to adverse clinical consequences. We report a case of a 19-year-old man with no prior comorbidities presenting with high-grade fever and altered sensorium. He was diagnosed to be a probable case of leptospirosis, based on all available test results and by fulfilment of parameters under modified Faine's criteria. The patient was successfully managed and discharged in stable condition.

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Isoniazid-induced pure red cell aplasia

The pure red cell aplasia (PRCA) is an uncommon side effect of isoniazid. We describe a 28-year-old man who developed a severe anaemia caused by PRCA. The patient received antituberculous therapy including isoniazid for his pulmonary tuberculosis. On discontinuation of isoniazid, the anaemia recovered promptly. PRCA should be considered in case of unexplained anaemia during isoniazid treatment.

https://ift.tt/2C21JPT

Solitary vallecular neurofibroma presenting with respiratory obstruction: an unusual presentation

An occurrence of vallecular neurofibroma with airway obstruction is extremely rare and to the best of our knowledge, no case report has been documented in the literature to date. Although microlaryngeal surgery with cold knife excision is the standard surgical procedure offered to patients of vallecular lesions, it can be successfully managed with the help of endoscopic coblation as later can provide a bloodless surgical field preventing the unmet need for the tracheostomy and reducing the postoperative morbidities.

https://ift.tt/2zYp97t

RCC in cross ectopic kidney: a challenging diagnosis and management

Description 

A 57-year-old woman with a 5-month history of left-upper quadrant (LUQ) abdominal pain was referred after a retroperitoneal mass was discovered on CT scan of the abdomen and pelvis. Clinical history revealed mild LUQ abdominal pain over the course of 5 months, associated with anorexia, unintended weight loss and several episodes of painless gross haematuria. Physical examination was positive for an ill-defined mass in the LUQ extending to the umbilical region. Routine laboratory tests did not reveal abnormal findings except for microscopic haematuria. CT scan images revealed a left cross ectopic kidney fusing laterally with the lower pole of right kidney in addition to a mass measuring 7.5x8.5 cm emerging from the ectopic kidney. The mass extended to the left flank crossing the abdominal aorta and passing behind the inferior mesenteric artery. This structure mimicked a horseshoe kidney (figures 1 and 2). Retrograde pyelography confirmed...

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Woman with lower back pain, SIADH and a twist of Lyme

A 62-year-old woman was admitted with a 3-week history of atraumatic bilateral lower back pain, progressive ascending flaccid paralysis, hyponatraemia and constipation. She was otherwise in good health with only a recent diagnosis of acute gastroenteritis that preceded her presenting symptoms. Her initial laboratory evaluation was consistent with Syndrome of Inappropriate Antidiuretic Hormone (SIADH) but was otherwise unremarkable. MRI of the spine revealed bilateral diffuse nerve root enhancement from at least C6 to the conus level, suggesting an inflammatory process. Lumbar puncture demonstrated high protein (629 mg/dL) with marked pleocytosis (363 cells/mcL) incompatible with albuminocytological dissociation typically seen in Guillain-Barre syndrome. A thorough diagnostic evaluation was undertaken to explore potential infectious, malignant and autoimmune conditions. Lyme disease serology (ELISA and Western Blot, IgM and IgG) was positive leading to a final diagnosis of lymphocytic meningoradiculitis or Bannwarth syndrome.

https://ift.tt/2zYykVE

Large mesenteric lymphangioma in an adult patient: an unusual presentation of a rare disease

Lymphangiomas are most commonly described as a small painless mass in the neck or a vesicular rash in an infant patient. Ninety per cent of cases are diagnosed before the age of 2. Treatment usually involves surgical resection. Intra-abdominal lymphangiomas and mesenteric lymphangiomas, as described in our case report, represent a rare pathology. The exact prevalence of this condition is unclear but it has been suggested in the literature that there have been as few as 820 cases since the 16th century. The clinical presentation is usually subacute and diagnosis made incidentally during a workup of chronic gastrointestinal symptoms. Acute abdominal symptoms, as in our case presentation, are unusual but may be explained by the mass effect of a large intra-abdominal lesion. Cross-sectional imaging is key in preoperative workup and operative planning. Complete surgical resection is recommended and curative in the majority of cases with a low risk of local recurrence.

https://ift.tt/2C21mEZ

Pericardial sarcoma

Pericardial sarcomas are extremely rare aggressive neoplasms. Non-specific symptoms and incidental discovery are usually the rule. Multimodality imaging is extremely important for diagnosis and tissue characterisation of all cardiac masses. Despite treatment, pericardial sarcomas are considered extremely fatal. We encountered a 27-year-old female patient who presented to our facility with progressive dyspnoea. On examination, clinical signs of cardiac tamponade were appreciated, transthoracic echocardiography revealed a tamponading pericardial effusion and a large heterogeneous pericardial mass. Pericardiocentesis revealed haemorrhagic fluid. Subsequently, CT revealed a pericardial mass compressing the right atrium. Excision biopsy showed a well-circumscribed mass, and cut sections showed friable grey–white tissue with areas of haemorrhage and necrosis. Pathological examination confirmed the diagnosis of high-grade undifferentiated sarcoma of the pericardium. The patient was started on adjuvant chemotherapy and radiotherapy. Follow-up after 1 year showed no relapse.

https://ift.tt/2zXreAE

Cutis marmorata telangiectatica congenita with skin ulceration: a rare benign skin vascular malformation

Description 

Cutis marmorata telangiectatica congenita (CMTC) is a sporadic congenital disorder characterised by localised or generalised cutaneous vascular anomaly. CMTC was first described in 1922 by Van Lohuizen as a pattern of reticulate erythema and telangiectasia infrequently associated with skin atrophy and/or ulceration.1 It is a rare benign skin lesion that is often apparent at birth with more than 50% of patients with CMTC having variations of associated defects. Body asymmetry is the most common (33%) associated anomaly reported.1

A 5-day-old female baby was born at term following an uneventful pregnancy. At birth, she weighed 2800 g, and her growth profile was appropriate for her age. Her general physical examination was unremarkable except for the appearance of a 3x4 cm bluish-purple skin lesion over the right knee. The skin lesion had erythematous reticulated appearance at the periphery and ulceration at the centre with subcutaneous atrophy (

https://ift.tt/2C0uWuK

Strangulated urethral prolapse in a postmenopausal woman presenting as acute urinary retention

Description 

A 48-year-old woman presented with acute urinary retention for 1 day and severe periurethral pain for last 3 days. She also revealed a history of occasional blood spotting of undergarments, dysuria and a protruding mass from the urethra for the last 3 months. Her medical/surgical and personal histories were unremarkable. Her obstetric history revealed presence of three normal vaginal deliveries and menopause at the age of 45 years. Local examination revealed presence of irreducible dusky red oedematous tender donut-shaped swelling measuring 2x2 cm around the urethral orifice suggestive of strangulated urethral prolapse (figure 1). A gentle attempt at reduction of the prolapsed mass was done under sedation which was successful following which a Foley catheter was placed. Cystourethroscopy revealed excess urethral mucosa with normal bladder mucosa. Ultrasonography of the abdomen was suggestive of normal bladder parameters with bilateral normal upper tracts. After proper counselling and consent, the patient was taken for surgery under general anaesthesia....

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Case of solitary plasmacytoma of tibia

Description 

A 58-year-old diabetic man presented to the outpatient department with pain in his right leg of 6 months' duration. The pain was insidious in onset and gradually progressive. He was unable to bear weight on the affected side. On clinical examination, there was tenderness over the medial aspect of the upper part of the leg. The knee range of movements was limited and painful. The distal neurovascular examination was normal.

Plain X-ray of the knee (figure 1) showed an eccentric lytic lesion in the proximal end of the tibia with a pathological fracture of the medial wall. There was no periosteal reaction. Plain and contrast MRI study (figure 2) revealed a well-defined heterogeneously enhancing altered signal eccentric intramedullary lesion in the proximal metadiaphyseal region of the tibia with cortical breaks. The patient was taken up for open incisional biopsy, and prophylactic fixation of the proximal...

https://ift.tt/2C1iFWU

Pulmonary capillary haemangiomatosis causing pulmonary arterial hypertension: a clinicians conundrum

Description 

Pulmonary capillary haemangiomatosis (PCH) is a rare cause of pulmonary hypertension which poses a considerable diagnostic challenge. Here, we describe the case of a 14-year-old boy with gradually progressive exertional dyspnoea and cough for past 4 months. Frontal chest radiograph demonstrated prominent pulmonary artery segment with diffuse nodular opacities in bilateral lung fields (figure 1A). CT angiography was performed to diagnose the aetiology of pulmonary artery hypertension. It revealed dilated central pulmonary arteries (figure 1B) with multiple ill-defined centrilobular nodules diffusely distributed in both lungs (figure 1C,D). No interlobular septal thickening, pulmonary thromboembolism, pleural effusion or any intracardiac/extracardiac shunt was seen. A provisional diagnosis of PCH was made which was subsequently confirmed on a lung biopsy.

Figure 1

Frontal chest radiograph (A) revealing prominent pulmonary artery segment with reticulonodular opacities predominantly involving the lower zones. No Kerley lines were noted....

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Dermatomyositis with anti-TIF1-{gamma} antibodies

Description 

We report the case of a 61-year-old Turkish male patient who presented to our dermatology outpatient clinic with a 1-year history of swelling and pruritic erythema rash of the face and trunk. He reported associated myalgia and arthralgia of the knees and wrists. Two months before presentation, he developed muscle weakness of upper limbs and dyspnoea. On review, he was noted to have unintentional weight loss (5 kg over 2 months) and increasing fatigue. His medical and family histories were unremarkable. His medications included omeprazole daily and vitamin B₁₂ injections. Prior to presentation to our clinic, his general practitioner treated the patient with antihistamines, topical steroids (Elocom) and a short course of oral corticosteroid therapy which only provided temporary relief. Laboratory data demonstrated C reactive protein 6 mg/L (normal value (NV) <5 mg/L), haemoglobin 11.9 g/dL (NV 13–18 g/dL), lactate dehydrogenase 467 U/L (NV 135–225 U/L), creatinine phosphokinase 295 U/L (NV 30–190 U/L), Aspartate transaminase (GOT)...

https://ift.tt/2C0uGfg

Myasthenia gravis crisis coinciding with asthma exacerbation in a patient with recent heroin use: three causes of acute hypercarbic respiratory failure

A 57-year-old woman with a history of asthma, hypertension and substance abuse disorder was admitted to the medical intensive care unit with hypercapnic respiratory failure. After the history was obtained, patient admitted heroin use earlier that day. The initial physical examination revealed right eye ptosis, diplopia, fatigability of neck flexion and extension. She also presented with wheezing and a prolonged expiratory phase. Pupils were 4 mm, with sluggish response to light bilaterally. CT chest with contrast showed a large mediastinal mass. Three different processes coexisted in this patient: simultaneous occurrence of a myasthenia gravis crisis, asthma exacerbation and a component of heroin use. This case highlights a series of overlapping clinical features that could lead to potential confounding and misdiagnosis. Respiratory symptoms improved after initial treatment for asthma exacerbation, but ptosis, diplopia and fatigability of neck muscles persisted.

https://ift.tt/2zZj7nf

Incidental finding of subgemmal neurogenous plaque upon retrospective evaluation of oral lymphoepithelial cysts

Abstract

Purpose

We aimed to compare the histomorphological features of oral lymphoepithelial cysts (OLC) in different locations of the oral cavity and to verify the association between oral lymphoepithelial cysts and subgemmal neurogenous plaque (SNP) on biopsies from the lateral border of the tongue.

Methods

All cases diagnosed as OLC from the Oral Pathology Service at the School of Dentistry/University of São Paulo were retrieved. For all the cysts located in the tongue, their association to SNP was analyzed. Immunohistochemical staining against S100 was performed to confirm the diagnosis of SNP.

Results

Thirty-one cases were selected for morphological analysis and SNP was identified in 40% of cases, all of them positive for S100. In these cases, macroscopic analysis demonstrated two independent fragments. Microscopic analysis showed that cysts located in the floor of the mouth and ventral surface of the tongue showed less intense exocytosis, less formation of lymphoid follicles, and predominantly a moderate inflammatory infiltrate.

Conclusions

OLC have a similar histological pattern regardless the location within the oral cavity and. In some cases, the biopsy specimen may be accompanied by a SNP as an adjacent independent structure.

https://ift.tt/2y7w64W

Incidental finding of subgemmal neurogenous plaque upon retrospective evaluation of oral lymphoepithelial cysts

Abstract

Purpose

We aimed to compare the histomorphological features of oral lymphoepithelial cysts (OLC) in different locations of the oral cavity and to verify the association between oral lymphoepithelial cysts and subgemmal neurogenous plaque (SNP) on biopsies from the lateral border of the tongue.

Methods

All cases diagnosed as OLC from the Oral Pathology Service at the School of Dentistry/University of São Paulo were retrieved. For all the cysts located in the tongue, their association to SNP was analyzed. Immunohistochemical staining against S100 was performed to confirm the diagnosis of SNP.

Results

Thirty-one cases were selected for morphological analysis and SNP was identified in 40% of cases, all of them positive for S100. In these cases, macroscopic analysis demonstrated two independent fragments. Microscopic analysis showed that cysts located in the floor of the mouth and ventral surface of the tongue showed less intense exocytosis, less formation of lymphoid follicles, and predominantly a moderate inflammatory infiltrate.

Conclusions

OLC have a similar histological pattern regardless the location within the oral cavity and. In some cases, the biopsy specimen may be accompanied by a SNP as an adjacent independent structure.

https://ift.tt/2y7w64W