Using endoscopy to locate the round window membrane during cochlear implantation: Our experience with 25 patients

Abstract

Locating the round window membrane during cochlear implantation surgery is crucial to insert the electrode array correctly and to minimize intracochlear damage. However, it can be difficult to access the round window membrane in certain patients, especially in paediatric patients or patients with inner ear malformations. Recently, it has been demonstrated that endoscopy enables excellent visualization and exploration of the round window niche in ear surgery.

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Re: Frontal Fibrosing Alopecia Severity Index (FFASI): a call for a more inclusive and globally relevant severity index for frontal fibrosing alopecia: reply from the authors

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Merkel cell carcinoma: Do Australians have a more aggressive variant and a worse outcome?

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Ameloblastic Fibroodontoma: Uncommon Case Presentation in a 6-Year-Old Child with Review of the Literature

Ameloblastic fibroodontoma is a benign mixed odontogenic neoplasm considered in patients with asymptomatic swelling and unerupted teeth that exhibit histologic features between ameloblastic fibroma and complex odontoma. Radiographically, this lesion appears as radiolucency admixed with focal radio opaque masses of irregular shapes and sizes. This lesion is confirmed by the presence of proliferating odontogenic epithelium, ectomesenchyme, and dental hard tissue formation on pathological analysis supplementing clinical and radiographic findings. As this tumour is less commonly seen in routine clinical practice, ameloblastic fibroodontoma with detailed orofacial features and periodic approach to its diagnosis is discussed. This paper reports a case of ameloblastic fibroodontoma of the mandible in a 6-year-old male patient with an uncommon case presentation and review of the literature.

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Severe Hepatopulmonary Syndrome in a Child with Caroli Syndrome

Hepatopulmonary Syndrome (HPS) is a potential complication of chronic liver disease and is more commonly seen in the adult population. Caroli Syndrome is a rare inherited disorder characterized by intrahepatic ductal dilation and liver fibrosis that leads to portal hypertension. In children with liver disease, HPS should be considered in the differential diagnosis of prolonged, otherwise unexplained, hypoxemia. The presence of HPS can improve patient priority on the liver transplantation wait list, despite their Pediatric End-Stage Liver Disease (PELD) score. We present a 6-year-old girl with Caroli Syndrome and End-Stage Renal Disease who presented with persistent hypoxemia. The goal of this report is to increase awareness of HPS in children.

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Protein Allergy and GMOs

Publication date: 2017
Source:Reference Module in Biomedical Sciences
Author(s): G.S. Ladics
A rigorous safety assessment process exists for genetically modified (GM) crops. It includes an evaluation of the introduced protein as well as the crop containing such protein with the goal of demonstrating the GM crop is "as-safe-as" non-GM crops in the food supply. One of the major issues for GM crops is the assessment of the expressed protein for allergenic potential. Currently, no single factor is recognized as a predictor for protein allergenicity. Therefore, a weight-of-the-evidence approach, which takes into account a variety of factors and approaches for an overall assessment of allergenic potential, is conducted. This assessment is based on what is known about allergens, including the history of exposure and safety of the gene(s) source; protein structure (e.g., amino acid sequence identity to human allergens); stability to pepsin digestion in vitro; an estimate of exposure of the novel protein(s) to the gastrointestinal tract where absorption occurs [e.g., protein abundance in the crop, processing effects (i.e., heat stability)]; glycosylation status; and, when appropriate, specific IgE-binding studies with sera from relevant clinically allergic subjects. Since GM crops were first commercialized over 20years ago, there is no proof that the introduced novel protein(s) in any approved GM crop has caused food allergy.



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Manoeuvre to aid Endoscopic division of aryepiglottic folds (Aryepiglottoplasty)

Laryngomalacia, a congenital malformation of larynx is the commonest cause of stridor in young infants. Ninety percent of cases resolve by the age of 2 years without any active intervention (1).The most consistent abnormality seen in non-resolving cases is shortening of the aryepiglottic folds (picture 1).(2)

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Large BTK gene mutation in a child with X-linked agammaglobulinemia and polyarthritis

Publication date: Available online 12 August 2017
Source:Clinical Immunology
Author(s): Dhrubajyoti Sharma, Aman Gupta, Shubham Goel, Madhubala Sharma, Amit Rawat, Surjit Singh




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Vitreous amyloidosis with autonomic neuropathy of the digestive tract associated with a novel transthyretin p.Gly87Arg variant in a Bangladeshi patient: a case report

Hereditary transthyretin amyloidosis is an autosomal dominant inherited disorder, first described in families with sensorimotor and autonomic neuropathy. Since its first description, more than 120 amyloidogeni...

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