Eosinophilic Esophagitis in Children

Abstract

Purpose of Review

EoE is a significant cause of gastrointestinal morbidity affecting 1:2000. Patients with EoE typically have multiple atopic comorbidities, and additionally, many patients with EoE can be controlled well with elimination diets. The purpose of this review is to summarize the care of pediatric eosinophilic esophagitis patients.

Recent Findings

EoE represents a distinct clinical syndrome which is characterized by esophageal dysfunction and eosinophil-predominant inflammation of the esophageal mucosa. Patients with EoE can present with varying symptoms depending on their age; in this review, we review the presenting features of eosinophilic esophagitis in children as well as a diagnostic algorithm for EoE. The mucosal inflammation in EoE is driven by exposure to food antigens in many patients with EoE. Therefore, for the majority of patients, the mainstays of treatment remain food elimination diets or swallowed steroids.

Summary

This review summarizes the diagnostic approach to eosinophilic esophagitis (EoE) in pediatric patients, focusing on the importance of accurate diagnosis and selection of appropriate therapy.

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A gastric MANEC with an adenocarcinoma of fundic-gland type as exocrine component

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Chondrolipoma of the breast as a rare variant of myofibroblastoma: an immunohistochemical study of two cases

Abstract

Chondrolipoma of the breast is a very rare tumor whose histogenesis remains obscure. We report two cases (56-year-old and 43-year-old women) and present the results of an immunohistochemical study which strongly suggests that this tumor is a variant of myofibroblastoma. The tumors predominantly consisted of lipoma-like, mature adipose tissue, and many islands of hyaline cartilage. A proliferation of spindle cells associated with the deposition of collagen fibers was also seen. On immunohistochemical examination, spindle cells showed cytoplasmic reactivity for vimentin, desmin, bcl-2, and α-smooth muscle actin, as well as nuclear reactivity for estrogen receptor (ER) and progesterone receptor (PgR). Chondrocytes were immunoreactive for ER, PgR, S-100 protein, and Sox9. The nuclei of adipocytes, chondrocytes, and spindle cells were not immunoreactive for Rb (retinoblastoma) protein. The immunoreactivity of spindle cells for muscle markers indicates myofibroblastic differentiation, and the lack of the nuclear expression of Rb protein suggests the close relationship of this tumor with myofibroblastoma and spindle cell lipoma. The immunoreactivity of chondrocytes for ER and PgR suggests that they are derived from metaplasia of hormone-sensitive spindle cells. These findings support the concept that chondrolipoma of the breast could be a lipomatous variant of myofibroblastoma associated with cartilaginous metaplasia and that it should be added to members of the "13q/Rb family of tumors."

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Granuloma faciale associated with IgG4-related disease

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Therapeutic efficacy and safety of oral tranexamic acid and that of tranexamic acid local infiltration with microinjections in patients with melasma: a comparative study

Summary

Background

Tranexamic acid (TXA) has been used orally, intravenously, topically and intradermally (microinjection, microneedling) for treating melasma. However, the comparative efficacy of these different routes of administration remains underevaluated.

Aim

To ascertain the comparative efficacy of different routes of administration of TXA.

Methods

In total, 100 consecutive patients with melasma (8 men, 92 women, age range 18–55 years) were randomly assigned to one of two groups comprising 50 patients each. Group A (3 men, 47 women) received oral TXA 250 mg twice daily, while group B (5 men, 45 women) received intradermal microinjections of TXA 4 mg/mL every 4 weeks. The treatment continued for 12 weeks in both groups. Percentage reduction in baseline Melasma Area and Severity Index (MASI) was assessed at 4-week intervals, and response was scored as very good (> 75% reduction), good (50% to < 75% reduction), moderate (25% to < 50% reduction), mild (< 25% reduction) or no response.

Results

The study was completed by 39 patients in group A and 41 patients in group B. Very good response was seen in 25 and 32 patients in groups A and B, respectively, while good response was seen in 14 and 9 patients, respectively. Both treatment methods were equally effective, with an average reduction of MASI at 12 weeks of 77.96 ± 9.39 in group A and 79.00 ± 9.64 in group B. The main adverse effects were mild epigastric discomfort, hypomenorrhea, headache and injection site pain, which did not warrant discontinuation of treatment. Two patients in group A had relapses at 24 weeks.

Conclusion

TXA appears to be an effective and safe treatment for melasma, irrespective of its route of administration.

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Long-term management of chronic spontaneous urticaria with omalizumab

Summary

Background

Clinical trials have shown the efficacy of omalizumabs efficacy in refractory chronic spontaneous urticaria (CSU) and chronic inducible urticaria (CIndU), but real-life management strategies are lacking.

Aim

To assess the long-term efficacy and safety of omalizumab, and to identify predictive factors and optimum dosage regimens.

Methods

This was a prospective study of 13 patients (11 women, 2 men) with severe CSU [weekly urticaria activity score (UAS7) > 28] resistant to anti-H1 antihistamines. Patients were started on omalizumab 150 mg subcutaneously every 4 weeks. Dose and interval between administrations were adjusted according to clinical response (189 administrations; treatment duration range 2–38 months).

Results

Mean UAS7 was 36.3 ± 5.4. Of the 13 patients, all had experienced angio-oedema, while in addition, 7 had delayed pressure urticaria (DPU) and 1 had solar urticaria (SU). After omalizumab treatment, 4 (30.8%) of the 13 patients had complete response (CR), and the remaining 8 (61.5%) had partial response. CR was achieved with a dose of 150 mg every 4 (n = 2 patients) or 5 (n = 2) weeks. One of these patients remained disease-free after stopping treatment. Partial responses were achieved with 150 mg every 4 weeks (n = 4) and with 300 mg (n = 4) at intervals of 5 weeks (n = 1), 4 weeks (n = 2) or 3 weeks (n = 1). Only one patient (7.7%) did not show significant improvement, despite a dose of 300 mg every 4 weeks. There were no significant differences in epidemiological, clinical and laboratory data between the different response groups. Only two adverse events were observed: one was mild headache and the other was severe angio-oedema and aggravation of urticaria within 6 h of omalizumab administration.

Conclusion

Omalizumab dose and interval between administrations could be individualized for long-term management of CSU.

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Adjuvant therapy with low-dose interferon-beta for stage II and III melanoma: results of a retrospective analysis

Summary

Interferon (IFN)-alfa as an adjuvant therapy has been found to improve relapse-free survival in patients with malignant melanoma (MM). However, the efficacy of IFN-beta has not been studied in detail. This study evaluated the contribution of adjuvant IFN-beta therapy to improvements in the prognosis of patients with MM. We reviewed 63 patients with resected stage II/III primary MM at our institution. Of these, 36 had been treated with IFN-beta adjuvant therapy (subcutaneous injection, 3 × 10⁶ IU/day, 10 days), while 27 patients had undergone observation alone. In comparisons of all patients (stage II/III), overall survival and relapse-free survival were significantly better in the IFN-beta group than in the observation group (P < 0.001 for both). The 75-month overall survival rate was 41.2% in the observation group and 68.7% in the IFN-beta group. Adjuvant therapy with IFN-beta may become a new treatment option for patients with stage II/III MM.

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Osteonecrosis of the jaws: a review and update in etiology and treatment

Publication date: Available online 24 June 2017
Source:Brazilian Journal of Otorhinolaryngology
Author(s): Guilherme H. Ribeiro, Emanuely S. Chrun, Kamile L. Dutra, Filipe I. Daniel, Liliane J. Grando
IntroductionOsteonecrosis of the jaws (ONJ) can result either from radiation, used in radiotherapy (RT) for treatment of malignant tumors, or medications used for bone remodeling and anti-angiogenesis such as bisphosphonates (BPs). These conditions can be associated with triggering factors such as infection, trauma and decreased vascularity. The management of patients with ONJ requires caution since there is no specific treatment that acts isolated and decidedly. However, different treatment modalities can be employed in an associated manner to control and stabilize lesions.ObjectiveTo review the current knowledge on etiology and management of ONJ, both radio-induced and medication-related, aiming to improve knowledge of professionals seeking to improve the quality of life of their patients.MethodsLiterature review in PubMed as well as manual search for relevant publications in reference list of selected articles. Articles in English ranging from 1983 to 2017, which assessed ONJ as main objective, were selected and analyzed.ResultsInfections, traumas and decreased vascularity have a triggering role for ONJ. Prophylactic and/or stabilizing measures can be employed in association with therapeutic modalities to properly manage ONJ patients.ConclusionSelecting an appropriate therapy for ONJ management based on current literature is a rational decision that can help lead to a proper treatment plan.



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Primary epithelial salivary gland tumours in children and adolescents

Publication date: Available online 24 June 2017
Source:International Journal of Oral and Maxillofacial Surgery
Author(s): M. Gontarz, G. Wyszyńska-Pawelec, J. Zapała
Primary epithelial tumours of the salivary glands are very rare in paediatric patients. The aim of this study was to evaluate the clinical course, treatment, and outcomes of these uncommon neoplasms based on the authors' experience and the recent literature. The medical charts of 12 female patients and seven male patients with primary epithelial salivary gland tumours were reviewed. All were under 19 years of age and underwent surgical treatment between 1994 and 2016. The results of this group of paediatric patients were compared with those of 621 adult patients. The two most common tumours in the paediatric patients were pleomorphic adenoma and mucoepidermoid carcinoma (89.4%; P=0.004). The incidence of facial nerve palsy following surgery of the parotid tumours was similar in the two groups (P=1.000). The most common primary cancer in the paediatric group was mucoepidermoid carcinoma (77.8%), while in the adult group, adenoid cystic carcinoma was most common (P<0.001). The paediatric group had only low-grade cancers in early stages (P<0.001), with an overall 5-year survival rate of 100%. These results show that the incidence of malignant salivary gland tumours is higher in paediatric patients than in adult patients. This should be taken into account during diagnosis and therapy.



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Immediate full-arch rehabilitation of the severely atrophic maxilla supported by zygomatic implants: a prospective clinical study with minimum follow-up of 6 years

Publication date: Available online 24 June 2017
Source:International Journal of Oral and Maxillofacial Surgery
Author(s): E.L. Agliardi, D. Romeo, S. Panigatti, M. de Araújo Nobre, P. Maló
The aim of this study was to evaluate the outcomes of immediate full-arch prostheses supported by zygomatic implants alone or in combination with standard fixtures after a minimum of 6 years of loading. From October 2008 to April 2010, 15 patients with severely atrophic maxillae were treated using four zygomatic implants or two zygomatic implants in conjunction with two conventional fixtures. All subjects received a fixed screw-retained prosthesis within 3hours of surgery, while the final restoration was delivered after 6 months. Follow-up examinations were scheduled to evaluate zygomatic implant survival, conventional dental implant success, prosthetic success, plaque and bleeding scores, marginal bone loss for conventional dental implants, and patient satisfaction. Forty-two zygomatic fixtures and 18 standard implants were placed. Patients were followed up for a minimum of 79 months (range 79–97 months, average 90.61 months). No implant was lost, leading to implant and prosthetic survival rates of 100%. Bone loss for conventional implants averaged 1.39±0.10mm after 6 years of function, leading to a 100% implant success rate. High levels of patient satisfaction were recorded. These medium-term results indicate that immediate full-arch rehabilitation supported by zygomatic implants could be considered a viable treatment modality for the severely atrophic maxilla.



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Different hypersensitivities against homologous proteins of MGL_1304 in patients with atopic dermatitis

Publication date: Available online 24 June 2017
Source:Allergology International
Author(s): Takuma Kohsaka, Takaaki Hiragun, Kaori Ishii, Makiko Hiragun, Akiko Kamegashira, Michihiro Hide
BackgroundAtopic dermatitis (AD) is exacerbated by sweating, and the skin of most patients with AD are resided by Malassezia (M.) fungi. Recently, MGL_1304 produced by Malasseziaglobosa was identified as the major histamine releasing antigen in human sweat.MethodsThe full length cDNA of the counterpart of MGL_1304 in Malasseziarestricta (Mala r 8), was cloned by degenerate PCR and rapid identification of cDNA ends (RACE). Recombinant MGL_1304, and its counterparts, Mala s 8 (produced by Malasseziasympodialis) and Mala r 8 were prepared, and compared in their allergenicities by dot blot analysis and histamine release tests with sera and basophils of patients with AD.ResultsThe identities between MGL_1304 and Mala s 8, MGL_1304 and Mala r 8, and Mala s 8 and Mala r 8 were 68%, 78%, and 76%, respectively, in protein sequences. Dot blot analysis revealed that the level of IgE binding to Mala s 8 was higher than that of MGL_1304. However, histamine release tests revealed that MGL_1304 and Mala r 8 possessed higher activity than Mala s 8. In addition, the crude lysate of M. globosa showed higher histamine release ability than that of M. sympodialis.ConclusionsPatients with AD showed hypersensitivities against MGL_1304 and its homologs. However, the allergenicities of the homologs are variable and the histamine release activities may be different from the solid-phase binding activities for IgE. Sweat allergy should be carefully evaluated with biological activities of MGL_1304 and its homologs of other Malassezia fungi residing on the skin.



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Generation and characterization of a human oral squamous carcinoma cell line SCC-9 with CRISPR/Cas9-mediated deletion of the p75 neurotrophin receptor

Publication date: October 2017
Source:Archives of Oral Biology, Volume 82
Author(s): Ping Huang, Dongdong Tong, Jing Sun, Qing Li, Fenghe Zhang
ObjectiveTo investigate the importance of the p75 neurotrophin receptor (p75^NTR) in human tongue squamous carcinoma cells, we exploited the CRISPR/Cas9 technology to establish a p75^NTR-knockout SCC-9 cell line and to explore the effect on biological functions.Materials and methodsThe clustered regularly interspaced short palindromic repeats (CRISPR)/CRISPR-associated endonuclease (Cas9) system was used to generate genomic deletion mutants of p75^NTR in the tongue squamous carcinoma cell lines SCC-9. Single-guide RNA (sgRNA) sequences were designed to target the p75^NTR genomic sequence and were cloned into plasmid pGK1.1. The linearized vector was electroporated into SCC-9 cells and p75^NTR deletion was confirmed using Cruiser™ enzyme digestion and PCR amplification. SCC-9 clones with successful deletion of p75^NTR were identified and verified by sequencing and selected for functional testing in cell proliferation, invasion, migration, and colony-forming assays.ResultsCompared with control cells, p75^NTR-knockout SCC-9 cells showed significantly diminished abilities to proliferate, invade, migrate, and form colonies, indicating a reduction in pro-tumorigenic behavior.ConclusionThese data demonstrate, first, that the CRISPR/Cas9 system is a simplified method for generating p75^NTR knockouts with relatively high efficiency, and second, that deletion of p75^NTR suppresses several tumor-promoting properties of SCC-9 cells, suggesting that p75^NTR is a potential target for the development of novel therapies for tongue cancer.



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Fibulins and matrilins are novel structural components of the periodontium in the mouse

Publication date: October 2017
Source:Archives of Oral Biology, Volume 82
Author(s): Andrea Schubert, Boris Schminke, Nicolai Miosge
Periodontitis refers to inflammatory disease of the periodontal structures (the gingiva, dental cementum, periodontal ligament (PDL) and alveolar bone) that ultimately leads to their destruction. Whereas collagens are well-examined main components of the periodontium, little is known about the other structural proteins that make up this tissue. The aim of this study was to identify new extracellular matrix (ECM) components, including fibulins and matrilins, in the periodontium of mice.After sacrificing 14 mice (Sv/129 strain), jaws were prepared. Each tissue sample contained a molar and its surrounding alveolar bone. Immunohistochemistry was carried out on paraffin-embedded sections.Our results show that mice exhibit fibulin-3, -4 and -5 and matrilin-1, -2, -3 and -4 in PDL and in blood vessels of alveolar bone and PDL as well as in the pericellular matrix of osteocytes and cementocytes. In dental cementum, only fibulin-4 is expressed.For the first time, we show that fibulin-3, -4 and -5 and matrilin-1, -2, -3 and -4 are essential components of the periodontal tissues. Our findings indicate an association of these proteins with collagens and oxytalan fibers that might be of future interest in regenerative periodontitis therapy.



http://ift.tt/2sPt3ul

Generation and characterization of a human oral squamous carcinoma cell line SCC-9 with CRISPR/Cas9-mediated deletion of the p75 neurotrophin receptor

Publication date: October 2017
Source:Archives of Oral Biology, Volume 82
Author(s): Ping Huang, Dongdong Tong, Jing Sun, Qing Li, Fenghe Zhang
ObjectiveTo investigate the importance of the p75 neurotrophin receptor (p75^NTR) in human tongue squamous carcinoma cells, we exploited the CRISPR/Cas9 technology to establish a p75^NTR-knockout SCC-9 cell line and to explore the effect on biological functions.Materials and methodsThe clustered regularly interspaced short palindromic repeats (CRISPR)/CRISPR-associated endonuclease (Cas9) system was used to generate genomic deletion mutants of p75^NTR in the tongue squamous carcinoma cell lines SCC-9. Single-guide RNA (sgRNA) sequences were designed to target the p75^NTR genomic sequence and were cloned into plasmid pGK1.1. The linearized vector was electroporated into SCC-9 cells and p75^NTR deletion was confirmed using Cruiser™ enzyme digestion and PCR amplification. SCC-9 clones with successful deletion of p75^NTR were identified and verified by sequencing and selected for functional testing in cell proliferation, invasion, migration, and colony-forming assays.ResultsCompared with control cells, p75^NTR-knockout SCC-9 cells showed significantly diminished abilities to proliferate, invade, migrate, and form colonies, indicating a reduction in pro-tumorigenic behavior.ConclusionThese data demonstrate, first, that the CRISPR/Cas9 system is a simplified method for generating p75^NTR knockouts with relatively high efficiency, and second, that deletion of p75^NTR suppresses several tumor-promoting properties of SCC-9 cells, suggesting that p75^NTR is a potential target for the development of novel therapies for tongue cancer.



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Fibulins and matrilins are novel structural components of the periodontium in the mouse

Publication date: October 2017
Source:Archives of Oral Biology, Volume 82
Author(s): Andrea Schubert, Boris Schminke, Nicolai Miosge
Periodontitis refers to inflammatory disease of the periodontal structures (the gingiva, dental cementum, periodontal ligament (PDL) and alveolar bone) that ultimately leads to their destruction. Whereas collagens are well-examined main components of the periodontium, little is known about the other structural proteins that make up this tissue. The aim of this study was to identify new extracellular matrix (ECM) components, including fibulins and matrilins, in the periodontium of mice.After sacrificing 14 mice (Sv/129 strain), jaws were prepared. Each tissue sample contained a molar and its surrounding alveolar bone. Immunohistochemistry was carried out on paraffin-embedded sections.Our results show that mice exhibit fibulin-3, -4 and -5 and matrilin-1, -2, -3 and -4 in PDL and in blood vessels of alveolar bone and PDL as well as in the pericellular matrix of osteocytes and cementocytes. In dental cementum, only fibulin-4 is expressed.For the first time, we show that fibulin-3, -4 and -5 and matrilin-1, -2, -3 and -4 are essential components of the periodontal tissues. Our findings indicate an association of these proteins with collagens and oxytalan fibers that might be of future interest in regenerative periodontitis therapy.



http://ift.tt/2sPt3ul

Predictive value of body mass index to metabolic syndrome risk factors in Syrian adolescents

Obesity has become a serious epidemic health problem in both developing and developed countries. There is much evidence that obesity among adolescents contributed significantly to the development of type 2 dia...

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