Subcutaneous immunoglobulins for the treatment of a patient with antisynthetase syndrome and secondary chronic immunodeficiency after anti-CD20 treatment: a case report

Antisynthetase syndrome is a rare and debilitating multiorgan disease characterized by inflammatory myopathy, interstitial lung disease, cutaneous involvement, and frequent chronic inflammation of the joints. ...

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Anesthetic Management of a Patient with Cowden Syndrome and Review of Anesthetic Concerns

Cowden syndrome (CS) is a rare autosomal dominant disorder demonstrating variable penetrance and an incidence of 1:200 000. The syndrome is characterized by gastrointestinal polyposis, mucocutaneous hamartomas of face and oral papillomatosis. Eighty percent of patients with CS have mutation in phosphatase and tensin homolog tumor-suppressor gene making them prone for developing malignancies of breast, thyroid and genitourinary system. Arteriovenous malformations (AVM) in brain, vertebral column and skin are also common features [1].

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Physical activity, restrictions in activity, and body mass index among urban children with persistent asthma

Asthma and obesity are public health problems that disproportionately affect underserved children. Urban children with asthma may be limited in their participation in physical activity, further increasing their risk for overweight.

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Diffuse heterotopic calcification of fibula flap: a previously unreported case

We describe what is, to the best of our knowledge, a new phenomenon, in which small multiple foci of heterotopic calcification developed in a fibular free flap that had been used to reconstruct a maxillary defect.

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Assessment of in vivo mast cell reactivity in patients with systemic mastocytosis

Abstract

Background

Patients with systemic mastocytosis (SM) have clinical signs of mast cell (MC) activation and increased levels of MC mediators. It is unclear whether the increased mediator levels are caused by increased numbers of tissue MCs, or whether these cells in affected individuals have a hyperactive phenotype.

Objective

To determine reactivity of the skin and the airways to directly acting mediators and indirectly acting mast cell secretagogues in subjects with SM.

Methods

Skin reactivity to morphine and histamine, and airway responsiveness to mannitol and methacholine was assessed in 15 patients with SM, 11 patients with allergic asthma (A) and 13 healthy controls (HC). Serum tryptase and urinary metabolites of the MC mediators histamine and prostaglandin D₂ were measured, as well as ex vivo basophil histamine release.

Results

Mast cell mediators in the blood and urine were significantly higher in patients with SM than in HC and A controls. Responsiveness to local activation of skin MCs (by morphine) and airway MCs (by mannitol) was similar in SM and HC groups. Likewise, end-organ responsiveness in the skin to histamine, and in the airways to methacholine, was similar in all three subject groups. There was no evidence of increased basophil reactivity in SM patients.

Conclusions & Clinical Relevance

Mast cells in the skin and airways of subjects with SM do not exhibit hyper-reactivity towards the MC-activating stimuli morphine and mannitol, respectively. Therefore, the highly elevated baseline levels of MC mediators in SM are most likely due to increased MC numbers, rather than altered MC responsiveness. The underlying mechanisms could involve leakage of MC mediators, or dysfunctions in mediator synthesis, storage and release. One clinical implication of our study is that there is no contraindication to perform skin tests using morphine in subjects with mastocytosis

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Interferon-γ induced insufficient autophagy contributes to p62-dependent apoptosis of epithelial cells in chronic rhinosinusitis with nasal polyps

Abstract

Background

Autophagy is a lysosomal degradation pathway that is essential for cell survival, differentiation, and homeostasis. This study aimed to investigate the contribution of autophagy to the pathogenesis of CRS with nasal polyps (CRSwNP).

Methods

The expression of autophagic proteins [microtubule-associated protein 1 light chain 3B (LC3B)-II, autophagy-related proteins (Atg), and Beclin 1], substrate proteins (p62 and ubiquitinated proteins), and apoptotic signaling molecules [cysteine-aspartic protease-3 and 8, and poly-ADP-ribose polymerase] in the sinonasal mucosa and nasal epithelial cells (NECs) was detected by immunohistochemistry and Western blotting. Autophagic vacuoles were observed with transmission electron microscopy. BEAS-2B cells and NECs were treated with rapamycin, bafilomycin A1, or various cytokines. In some experiments, cultured NECs were transfected with small interfering RNA targeting p62 (sip62) or Atg5 (siAtg5). Cultured cells were analyzed with Western blotting and flow cytometry.

Results

Although autophagic protein expression and autophagic vacuole formation were increased in both eosinophilic and non-eosinophilic CRSwNP, particularly in NECs, there was also an up-regulation of substrate proteins and apoptotic signaling molecules. IFN-γ, but not IL-4, IL-13, or IL-17A, simultaneously enhanced LC3B-II and p62 levels as well as cell apoptosis in BEAS-2B cells and/or normal NECs. Bafilomycin A1 up-regulated the levels of LC3B-II and p62 in polyp NECs and IFN-γ treated normal NECs. IFN-γ-induced apoptosis of normal NECs was exaggerated by bafilomycin A1 and siAtg5. Sip62 suppressed apoptosis of polyp NECs and IFN-γ treated NECs. IFN-γ protein levels were increased in both eosinophilic and non-eosinophilic CRSwNP.

Conclusions

IFN-γ induces activated but insufficient autophagy and thus contributes to a degree to p62-dependent apoptosis of NECs in CRSwNP.

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Serum cotinine levels should optimally be measured when evaluating the outcomes of cartilage tympanoplasty in smokers

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Reply to the letter concerning article: ‘Cervical necrotizing fasciitis: descriptive, retrospective analysis of 59 cases treated at a single center’

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Impact of low-level laser therapy on hyposalivation, salivary pH, and quality of life in head and neck cancer patients post-radiotherapy

Abstract

Late effects of radiotherapy for head and neck cancer treatment have been increasingly investigated due to its impact on patients' quality of life. The purpose of this study was to evaluate the effect of low-level laser therapy on hyposalivation, low salivary pH, and quality of life in head and neck cancer patients post-radiotherapy. Twenty-nine patients with radiation-induced xerostomia received laser sessions twice a week, during 3 months (24 sessions). For this, a continuous wave Indium-Gallium-Aluminium-Phosphorus diode laser device was used punctually on the major salivary glands (808 nm, 0.75 W/cm², 30 mW, illuminated area 0.04 cm², 7.5 J/cm², 10 s, 0.3 J). Six extraoral points were illuminated on each parotid gland and three on each submandibular gland, as well as two intraoral points on each sublingual gland. Stimulated and unstimulated salivary flow rate, pH (two scales with different gradations), and quality of life (University Of Washington Quality of Life Questionnaire for Patients with Head and Neck Cancer) were assessed at baseline and at the end of the treatment. There were significant increases in both mean salivary flow rates (unstimulated: p = 0.0012; stimulated: p < 0.0001), mean pH values (p = 0.0002 and p = 0.0004), and mean score from the quality of life questionnaire (p < 0.0001). Low-level laser therapy seems to be effective to mitigate salivary hypofunction and increase salivary pH of patients submitted to radiotherapy for head and neck cancer, thereby leading to an improvement in quality of life.

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Pain Control by Novel Route of Gifted Choice Against Peroral Route

Abstract

Introduction

Pain after surgical extraction of third molars has been a nemesis for oral surgeons with clinicians, thus striving for an analgesic modality. NSAIDs are among the most widely used therapeutic classes of analgesics. Transbuccal diclofenac sodium patches have been developed as an innovative drug delivery system using buccal mucosa as a gifted choice, hence overcoming first pass metabolism and offering the advantage of sustained drug delivery with reduced incidence of systemic adverse effects.

Aim

A comparative study was conducted to evaluate the efficacy of diclofenac sodium for pain control, administered via the far-fetched and gifted novel route through the transbuccal patch and by ever popular per oral route and also to assess the adverse effects vis-à-vis for transbuccal diclofenac patch and oral diclofenac following extraction of bilaterally symmetrical impacted mandibular third molars under local anaesthesia.

Methodology

Thirty healthy subjects of both the sexes in the age of 12 to 50 years with asymptomatic bilaterally symmetrical mandibular third molars underwent extraction under LA. It is a split-mouth study, i.e. after the extraction of tooth on one side, diclofenac sodium (50 mg) via oral route was given and then in another visit, when the same patient is comfortable and asymptomatic, extraction on contralateral side was executed and transbuccal patched diclofenac sodium (20 mg) was applied. Pain was measured on visual analog scale and verbal rating scale by the patient for 3 days and adverse effects if any were noted.

Result

Statistical analysis showed that transbuccal diclofenac sodium was significantly efficacious when compared to the drug administered orally. Also, statistically significant results were obtained in percentage reduction in pain from 1st to 3rd postoperative day in transbuccal group. No significant difference is seen for adverse reactions.

Conclusion

Transbuccal diclofenac sodium patch is more efficacious and can be used for pain control.

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The use of patient-specific implants in orthognatic surgery: A series of 30 mandible sagittal split osteotomy patients

Publication date: Available online 3 March 2017
Source:Journal of Cranio-Maxillofacial Surgery
Author(s): Juho Suojanen, Junnu Leikola, Patricia Stoor
PurposeVirtual surgery combined with patient-specific saw and drill guides and osteosynthesis materials are rapidly spreading from reconstructive surgery to orthognatic surgery. Most commercial partners are already providing computer-aided design and computer-aided manufacture (CAD/CAM) wafers and patient-specific saw guides. Clear benefits have been demonstrated for custom-made drill guides combined with individually designed three-dimensional (3D) printed patient-specific implants (PSI) as a reposition and fixation system in Le Fort I osteotomy.Materials and MethodsWe treated 30 patients who underwent bilateral sagittal split osteotomy (BSSO) due to class II dento-skeletal deformities with the additional use of drill guides combined with PSI as a fixation and positioning system.ResultsThe PSIs fitted bilaterally with total precision in 11 of the 30 patients. In 17 patients, the PSIs were used with some modifications. In 2 of 30 patients, the PSIs could not be used as a fixation due to misfit.ConclusionDue to unpredictable fitting, the use of PSIs with drill guides alone in BSSO without wafers cannot be recommended. Further studies are needed to evaluate the interfering parts, which seem to be related to condylar positioning and bony interferences at the osteotomy sites.



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Management of recurrent tonsillitis in children

To compare azithromycin (AZT) and benzathine penicillin (BP) in the treatment of recurrent tonsillitis in children.

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The role of surgery in anaplastic thyroid cancer: A systematic review

To elucidate the role of surgery in the management of anaplastic thyroid cancer.

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The use of patient-specific implants in orthognatic surgery: A series of 30 mandible sagittal split osteotomy patients

Virtual surgery combined with patient-specific saw and drill guides and osteosynthesis materials are rapidly spreading from reconstructive surgery to orthognatic surgery. Most commercial partners are already providing computer-aided design and computer-aided manufacture (CAD/CAM) wafers and patient-specific saw guides. Clear benefits have been demonstrated for custom-made drill guides combined with individually designed three-dimensional (3D) printed patient-specific implants (PSI) as a reposition and fixation system in Le Fort I osteotomy.

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Polydactylous Transverse Erythronychia: Report of a Patient with Multiple Horizontal Red Bands Affecting the Fingernails

Abstract

Introduction

Redness of the nail plate—erythronychia—is a common condition involving one or multiple digits. It may affect the entire nail or present as longitudinal red bands that extend from the proximal nail fold to the distal tip of the nail plate. Rarely, red bands may traverse the nail bed horizontally. Although erythronychia is often idiopathic, it has also been associated with amyloidosis, Darier's disease, lichen planus, and various other cutaneous conditions. We describe the clinical features of a 64-year-old Caucasian man who presented with transverse and longitudinal erythronychia affecting his fingernails. In addition, we review the classification of erythronychia and summarize the acute and chronic conditions that have been associated with this clinical finding.

Methods

The features of a man with polydactylous transverse and longitudinal erythronychia are presented. In addition, PubMed was used to search the following terms: erythronychia, longitudinal erythronychia, red lunulae, and subungual. All papers were reviewed, and relevant articles, along with their references, were evaluated. Informed consent was obtained from the patient for being included in the study.

Results

A 64-year-old Caucasian man with a past medical history significant for testicular cancer and pulmonary embolism presented with multiple horizontal pink-red bands affecting his fingernails. The discoloration was most prominent in the region distal to the lunula. In addition, the nails of the fifth digit of his left hand and third digit of his right hand featured longitudinal red bands extending from the distal curvature of the lunula to the free edge of the nail plate. A diagnosis of polydactylous longitudinal and transverse erythronychia, based on the clinical presentation, was established. Our patient's red bands were asymptomatic and he was not concerned about the cosmetic appearance of his nails; therefore, no additional investigation or treatment was required.

Conclusion

Polydactylous transverse erythronychia is a rare condition characterized by the development of horizontal red bands affecting the fingernails or toe nails. It has only been previously described in a man with multiple system atrophy. In our patient, both transverse and longitudinal red bands were present simultaneously. Therefore, it is possible that horizontal erythronychia is an early clinical manifestation of longitudinal erythronychia.

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A Fatter Way to Metastasize

Abstarct

Clinical outcomes for patients with oral squamous cell carcinoma are, in large part, related to the tumor stage at diagnosis (Warnakulasuriya, 2009). In particular, loco-regional spread is a key predictor of poor prognosis. Knowledge of the molecular events that contribute to oral tumorigenesis is considerable (Agrawal et al., 2011, Hunter et al., 2005, Leemans et al., 2011, Stransky et al., 2011); yet, the processes that contribute to metastasis of primary oral tumor cells are poorly understood.

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A novel GJA1 mutation in oculodentodigitaldysplasia with extensive loss of enamel

Abstract

Objective

To characterize clinical features and identify genetic causes of a patient with oculodentodigital dysplasia (ODDD)

Subjects And Methods

Clinical, dental, radiological features were obtained. DNA was collected from an affected Thai family. Whole Exome Sequencing (WES) was employed to identify the disease-causing mutation causing ODDD. The presence of the identified variant was confirmed by Sanger sequencing.

Results

The proband suffered with extensive enamel hypoplasia, polysyndactyly and clinodactyly of the 3^rd-5^th fingers, microphthalmia, and unique facial characteristics of ODDD.Mutation analysis revealed a novel missense mutation, c. 31C>A, p.L11I, in the GJA1 gene which encodes gap junction channel protein connexin 43. Bioinformatics and structural modelling suggested the mutation to be pathogenic. The parents did not harbor the mutation.

Conclusions

This study identified a novel de novo mutation in the GJA1 gene associated with severe tooth defects. These results expand the mutation spectrum and understanding of pathologic dental phenotypes related to ODDD.

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