Oral manifestations of hematologic and nutritional diseases

Oral manifestations of hematologic and nutritional deficiencies can affect the mucous membranes, teeth, periodontal tissues, salivary glands, and perioral skin. This article reviews common oral manifestations of hematologic conditions starting with disorders of the white blood cells including cyclic hematopoiesis (cyclic neutropenia), leukemias, lymphomas, plasma cell dyscrasias, and mast cell disorders; this is followed by a discussion of the impact of red blood cell disorders including anemias and less common red blood cell dyscrasias (sickle cell disease, hemochromatosis, and congenital erythropoietic porphyria) as well as thrombocytopenia. Several nutritional deficiencies exhibit oral manifestations. The authors specifically discuss the impact of water-soluble vitamins (B2, B3, B6, B9, B12, and C), fat-soluble vitamins (A, D, and K) and the eating disorders anorexia nervosa and bulimia nervosa on the oral mucosa.     

Expression of KITENIN and its association with tumor progression in oral squamous cell carcinoma

Objective

KAI1 COOH-terminal interacting tetraspanin (KITENIN) contributes to tumor invasion and metastasis in various cancers. The aim of this study was to investigate expression of KITENIN in patients with oral cavity squamous cell carcinoma (SCC) and to determine whether KITENIN affects tumor cell behavior in oral cavity SCC cell line.

Methods

Western blotting and immunohistochemistry was used to assess alteration of KITENIN expression in human oral cavity SCC and normal oral cavity mucosa. To evaluate the impact of KITENIN knockdown, the cell invasion assay and cell migration assay using small-interfering RNA were performed.

Results

KITENIN protein expression was significantly increased in human oral cavity SCC tissues than in normal oral cavity mucosa by Western blotting. KITENIN immunoreactivity was strongly identified in human oral cavity SCC relative to adjacent normal tissue. Knockdown of KITENIN resulted in significantly reduced cell invasion in human oral cavity SCC cells (p = 0.001). Cell migration showed a marked decrease in KITENIN knockdown oral cavity SCC cells compared to the negative control oral cavity SCC cells (p = 0.01).

Conclusion

KITENIN is associated with tumor invasiveness and metastasis in human oral cavity SCC.     

Otorhinolaryngological manifestations of sickle cell disease

Objectives

Although sickle cell disease is the most common of all hereditary disorders, there are a few publications about the effects of the disease on the functions of the ears, nose and throat.In this review, we present an overview of the clinical manifestations of sickle cell disease in general and highlight the problems specifically presenting in the field of otorhinolaryngology.

Methods

We review the pathophysiology of sickle cell disease and its clinical features in general. Then, we review its manifestations in ear, nose and throat diseases.

Conclusion

Sickle cell disease is one of the most common hemoglobinopathies. It can cause severe pain crises and dysfunction of virtually every organ system in the body, ultimately causing premature death. There is high prevalence (55%) of obstructive adenotonsillar hypertrophy in children and adolescents with sickle cell disease. A very significant reduction has been observed in the rate of pain crises following tonsillectomy in patients with sickle cell anaemia. Prevalence rate for sensorineural hearing loss in older children and adult patients is reporting a range of 11-41%. Priapism of the turbinates is a cause of nasal obstruction in sickle cell anaemia which needs partial turbinectomy. Extramedullary haematopoiesis should be considered in the differential diagnosis of any paranasal sinus mass presenting in a patient with known chronic anaemia.     

Guidelines of the French Society of Otorhinolaryngology (SFORL) (short version). Specific treatment of epistaxis in Rendu-Osler-Weber disease

Objectives

The authors present the guidelines of the French Oto-Rhino-Laryngology – Head and Neck Surgery Society (Société Française d’Oto-Rhino-Laryngologie et de Chirurgie de la Face et du Cou: SFORL) concerning specific treatment of epistaxis in Rendu-Osler-Weber disease.

Cochleovestibular manifestations in Fabry disease: Importance of screening and systematic ENT evaluation

ObjectivesFabry disease (FD) is an X-linked inherited lysosomal storage disease. It is a multisystem pathology that can include ENT disorders. The aim of the present study was to investigate the cochleovestibular manifestations of FD, in order to show the importance of screening and systematic ENT evaluation.Material and methodsA single-center retrospective study included 14 male and 23 female FD patients. Hearing impairment was defined as hearing loss greater than the 90^th percentile for at least one frequency. Vestibular impairment was defined by lateral semicircular canal dysfunction. Age, ongoing enzyme replacement therapy (ERT) and organic (renal, cardiac and cerebrovascular) complications were used as severity markers.ResultsHearing impairment was found in 62.6% of cases, mostly at high frequencies, and was associated with age, ERT, and cardiac and cerebrovascular disorder. It affected 46.7% of asymptomatic adult patients.Vestibular impairment was found in 56% of cases, associated with age; it affected two-thirds of ERT patients, more than 60% of patients with organic complications, and 50% of asymptomatic adult patients.ConclusionsMore than half of patients had ENT involvement. All FD patients should undergo early ENT screening for diagnostic, prognostic and therapeutic purposes. Systematic complete ENT follow-up with auditory and vestibular evaluation should be performed regularly, even for heterozygous women.     

两种颈淋结巴清扫术对口腔鳞癌cN0期患者手术疗效比较：基于国内样本的Meta分析

目的:采用Meta分析方法，综合定量分析国内公开发表的有关国人cN0期口腔鳞癌患者行两种颈淋巴结清扫术的疗效，为cN0期患者颈淋巴结手术处理方式提供参考依据。方法：搜集国内所有公开发表的、随访资料完整、随机性良好的、分别采用肩胛舌骨上颈淋巴结清扫术(supraomohyoid neck dissection, SOND)与根治性颈淋结巴清扫术（radical neck dissection, RND）对cN0期患者手术疗效行对比观察的相关研究。对符合条件的资料行Meta分析，计算两组间淋巴结转移复发的比数比（odds ratio, OR）。结果：在88篇报道中，有6篇符合纳入标准。标本量1?065例，其中SOND组291例，阳性97例；RND组774例，阳性236例。OR=0.87<0.05（SOND/RND），95%CI为（0.60, 1.26）。两者差异无统计学意义。结论：SOND与RND对cN0期口腔鳞癌患者的手术疗效无明显差异，SOND可取代RND以提高患者生存质量。     

Vocal fold deposits in autoimmune disease — an unusual cause of hoarseness

The objective of this study was to evaluate the symptoms and clinical characteristics in patients with autoimmune vocal fold deposits. Fourteen patients underwent videolaryngostroboscopic examination and voice recording. Eleven of the 14 patients underwent rheumatological examination. In all cases, endoscopic examination showed transverse white‐yellow band lesions in the middle of the membranous portion of the vocal folds. In most cases, the lesions were bilateral but not exactly opposing each other. The most common voice characteristics were instability and intermittent aphonia. Inflammatory disease was present in 10 patients; five of these had rheumatoid arthritis (RA). No immunological signs common for all patients could be found. The histological examination was consistent with rheumatoid nodules. Vocal fold deposits, occurring most often in patients with RA, is an uncommon cause of hoarseness. Because the patients may have hoarseness as their primary symptom, it is important for otolaryngologists to be familiar with this disorder.     

Quantitative analysis of formal caregivers’ use of communication strategies while assisting individuals with moderate and severe Alzheimer's disease during oral care

This cross-sectional observational study examined formal caregivers’ use of task-focused and social communication strategies while assisting individuals with moderate and severe Alzheimer's disease residing in a long-term care facility during a basic activity of daily living: toothbrushing. Thirteen formal caregiver–resident dyads were observed during a total of 78 separate toothbrushing sessions. All caregiver utterances occurring during the task were transcribed and coded for type of communication strategy utilizing a multidimensional observational coding scheme, which was developed a priori. Overall, the majority of residents, irrespective of disease severity, successfully completed toothbrushing with the support of caregiver assistance. Caregivers assisting residents with moderate and severe AD were found to use a variety of communication strategies, with task-focused strategies accounting for the majority of use. For the most part, the communicative strategies employed did not differ across disease severity. However, some differences were identified including the use of one proposition, paraphrased repetition, using the resident's name, and provision of full assistance, with these strategies being used more often when assisting individuals with severe AD.