Noha Ahmed Azab Ahmed

PURPOSE OF REVIEW: The article reviews the effects of cervical osteophytosis and spine posture on swallowing, and how they can cause dysphagia.

RECENT FINDINGS: Cerivical osteophytosis which is a bony overgrowth of the cervical spines, it affects the swallowing through different mechanisms, including direct mechanical compression of the pharynx and esophagus, disturbances of normal epiglottic tilt over the inlet of the larynx by the osteophytes at C3-C4 level, inflammatory reactions in the tissues around the esophagus and cricopharyngeal spasm. Also, cervical spine posture in normal individuals could affect the swallowing mechanism by disturbance of the sphincteric action of the larynx and upper esophageal sphincter.

SUMMARY: Cervical osteophytosis and abnormal cervical curvature may be overlooked by the Otolaryngologists as causes of swallowing disorders. The diagnosis is usually confirmed by radiologic study for the cervical spine. Cervical abnormality should be kept in mind while we are investigating patients with swallowing problem, and postural modification may help in treatment of such cases.

CONCLUSION: Diffuse idiopathic skeletal hyperostosis (DISH) is a cause of swallowing disorders in elderly, and otolaryngologists should be aware of the disease on dealing with old patients complaining of dysphagia. The condition may be treated conservatively in most patients; however, surgical reduction of cervical osteophytes may be needed in severe cases.

OBJECTIVE: Large cervical osteophytes may cause dysphagia; they compress the pharynx leading to mechanical impairment of swallowing. DISH is characterized by ossification of the anterior longitudinal spinal ligament with formation of osteophytes. The aim was to investigate swallowing disorders among patients with DISH.

METHOD: The study included 139 patients with DISH. Their swallowing was evaluated using the eating assessment tool (EAT-10), and patients with swallowing disorders were subjected to fiber-optic endoscopic evaluation of swallowing (FEES), they received conservative treatment for 6 months. Patients were assessed again after treatment using the same measures that were employed before treatment.

RESULTS: Twenty-three patients (16.5%) demonstrated swallowing disorder, and FEES showed residues of food in the pharynx after swallow in all of them. Significant overall improvement after conservative treatment was achieved, with two patients resuming their normal regular diet. However, one patient demonstrated no improvement, and he needed surgical reduction of his large cervical osteophytes.

AIM: Growth arrest specific protein 6 (Gas-6) and its tyrosine kinase receptor Axl plays an important role in apoptosis, and regulation of innate immune response, therefore, we investigated their plasma concentrations in Rheumatoid arthritis (RA) patients and correlated them to clinical, laboratory and radiological parameters of the disease.

METHODS: Plasma from 77 RA patients and 50 normal healthy subjects were assayed for plasma Gas6 and Axl levels. Demographic, clinical and serological data were prospectively assessed. Rheumatoid arthritis disease activity was assessed using 28-joint Disease Activity Score (DAS-28) and functional capacity by modified health assessment questionnaire (mHAQ). Standardized x-rays for hands and feet were done to all participants.

RESULTS: The level of Gas6 and Axl were significantly decreased in the RA patients compared to those of the healthy control subjects. Levels of Gas6 correlated positively with Axl levels in both patients and healthy control. Gas6 levels were remarkably reduced in those patients with erosive RA than those without. Levels of Gas6 were found to be negatively correlated with the presence of erosive disease and positively correlated with DAS-28, ESR, Leucocytosis and IL6.

CONCLUSION: The plasma concentrations of Gas6 and Axl are altered in RA patients and thus may have a role in RA pathogenesis. Further mechanistic studies on the involvement of all TAM receptors tyrosine kinases pathway in RA are needed to help in understanding the pathogenesis and possibly aid in diagnosis and future treatments of RA especially for patients with erosive disease.

OBJECTIVES: Bell's palsy is considered the most common cause of facial nerve paralysis in children. Although different theories have been postulated for its diagnosis, reactivation of the Varicella zoster virus (VZV) has been implicated as one of the causes of Bell's palsy. The aim of the study was to evaluate the association of Varicella-zoster virus infection with Bell's palsy and its outcome in children.

METHODS: A total of 30 children with Bell's palsy were recruited and were assayed for evidence of VZV infection. The severity of facial nerve dysfunction and the recovery rate were evaluated according to House-Brackmann Facial Nerve Grading Scale (HB FGS). Paired whole blood samples from all patients were obtained at their initial visit and 3 weeks later, and serum samples were analyzed for VZV IgG and IgM antibodies using ELISA.

RESULTS: A significantly higher percentage of Bell's palsy patients were seropositive for VZV IgM antibodies than controls (36.6% of patients vs 10% of controls) while for VZV IgG antibodies the difference was statistically nonsignificant. HB FGS in Bell's palsy patients with serologic evidence of VZV recent infection or reactivation showed a statistiacally significant less cure rate than other patients.

CONCLUSIONS: VZV reactivation may be an important cause of acute peripheral facial paralysis in children. The appropriate diagnosis of VZV reactivation should be done to improve the outcome and the cure rate by the early use of antiviral treatment.

Diffuse idiopathic skeletal hyperostosis (DISH) is characterized by formation of large cervical osteophytes that may compress the posterior wall of the aerodigestive tract. It is a rare cause of dysphagia in the elderly. The aim of this study was to investigate the various otolaryngologic manifestations of DISH. Eleven elderly patients with DISH were included in the study. All patients presented with dysphagia that was graded on the swallowing screening tool (EAT-10), and the diagnosis of DISH was based on computed tomographic criteria. The patients were subjected to otolaryngologic examination and flexible laryngoscopy. Polysomnography was used for patients with excessive daytime sleepiness for detection of obstructive sleep apnea (OSA). In addition to dysphagia of varying severity, OSA was found in nine patients, change of voice in six, globus sensation in seven, aspiration in three, and cervical pain in seven. Flexible laryngoscopy showed bulging of the posterior pharyngeal wall in all patients. DISH may be an unrecognized contributory factor to both dysphagia and OSA in the elderly. Change of voice, aspiration, globus sensation, and cervical pain are other otolaryngologic manifestations that may be encountered symptoms of the disease. An otolaryngologist should be aware of the disease that may be overlooked, and computed tomography is a confirmatory diagnostic method.

Tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) has been reported to be involved in the pathophysiology of some autoimmune diseases as systemic lupus erythematosus, ankylosing spondylitis, and multiple sclerosis. The aim of this study was to assess serum TRAIL concentration in systemic sclerosis (SSc) patients and to investigate its possible association with various disease parameters. Thirty SSc patients as well as 25 rheumatoid arthritis (RA) patients and 25 healthy volunteers were included in the present study. Organ system involvement in SSc patients was investigated. Pulmonary function tests as well as chest high-resolution computed tomography (HRCT) were done to detect pulmonary involvement in our patients. TRAIL concentrations were measured in the sera of SSc patients, RA patients and healthy controls by enzyme-linked immunosorbent assay. Mean serum TRAIL levels were significantly higher in SSc patients than in the control RA patients and in healthy controls (p < 0.001) while they were not significantly different between patients with diffuse cutaneous SSc and patients with limited cutaneous scleroderma. Serum TRAIL levels were significantly higher in SSc patients with pulmonary involvement and were significantly correlated with HRCT scores. Serum TRAIL levels are significantly elevated in SSc patients and are associated with SSc-associated pulmonary involvement denoting a possible role of TRAIL in the pathogenesis of SSc. Further studies may be needed to confirm these findings and the possible use of TRAIL in detection and possibly treatment of SSc-associated pulmonary disease.

Juvenile idiopathic arthritis (JIA) is an autoimmune diseases characterized by chronic arthritis and systemic manifestations. Autoimmune diseases can affect the upper airways including the larynx. The aim of this study was to investigate laryngeal involvement in JIA patients and its possible association with JIA disease parameters. Fifty consecutive JIA patients were screened for laryngeal abnormalities using flexible fiberoptic laryngoscope and laryngeal computerized tomography. Laryngeal abnormalities were detected in nine (18%) of our cases, with cricoarytenoiditis in six cases (12%) and a rheumatoid nodule in the pyriform fossa in only one case (2%). Diffuse congestion and edema of the posterior part of the larynx with normal vocal cord mobility was detected in two cases (4%). In our study, laryngeal abnormalities were significantly higher in patients with polyarticular seropositive disease subtype and also were significantly higher in patients with longer disease duration, higher disease activity scores, and those with erosive disease. JIA may affect the larynx. Laryngeal involvement in JIA patients is more in polyarticular seropositive cases. JIA patients have to be subjected to thorough otolaryngologic examination for early diagnosis and prompt management.

OBJECTIVE: A proliferation-inducing ligand (APRIL) is a new member of the tumour necrosis factor family which is intimately connected to the regulation of cellular pathways. The aim of this study was to assess serum concentrations of APRIL in systemic sclerosis patients, and to correlate them with the main clinical and serological features of the disease.

METHODS: Sera from 35 patients with systemic sclerosis, 25 had limited cutaneous and 10 had diffuse cutaneous subtypes, and 35 normal healthy subjects were assayed for APRIL by Enzyme Linked Immunosorbant Assay. Demographic, clinical, autoantibodies and serological data were prospectively assessed.

RESULTS: Serum APRIL concentrations were higher in patients with systemic sclerosis and in both its subtypes compared to the healthy controls (p<0.0001 in all). Patients with elevated APRIL levels had significantly higher incidences of myositis than those with normal levels (p=0.04). We did not find significant differences in other organ involvement prevalence between systemic sclerosis patients with elevated vs. normal APRIL levels. In addition, the frequencies of autoantibodies (i.e., anti-topoisomerase I, anti-centromere) were comparable between both groups. Serum APRIL levels were correlated with serum γ-globulins concentrations (r=0.404, p=0.016) but not with C-reactive protein, skin score, nor pulmonary functions. Serum APRIL was also correlated with creatine kinase levels only in systemic sclerosis patients with myositis (r=0.786, p=0.02).

CONCLUSION: Our preliminary results suggest increased serum APRIL levels in systemic sclerosis patients, particularly in those associated with myositis and hypergammaglobinemia. To confirm our results, we propose that larger scale, multicentre studies with longer evaluation periods are needed.

Schwartz-Jampel syndrome is a rare inherited autosomal recessive disorder characterized by generalized myotonia, joint contractures, skeletal abnormalities and facial dysmorphism. The gene defect involves the 1p34-p36.1 region of chromosome 1. Also, one of the candidate genes for orofacial clefting is the 1p36 region. Cleft palate is the most common congenital anomaly in the head and neck. Despite both diseases share a genetic defect in chromosome 1p36 region, the association of both conditions has not yet been investigated. Feeding problems due to the presence of the cleft may add to the growth retardation that is already present in those patients, so palatoplasty is mandatory. We described a case of Schwartz-Jampel syndrome with cleft palate.

Systemic Lupus Erythematosus (SLE) is a chronic, systemic autoimmune disease characterized by loss of tolerance to self-antigens. Regulatory T cells (T(REG)) are those CD4+ T cells that constitutively express high levels of CD25 and exhibit powerful suppressive properties. The aim of this work was to quantify CD4+CD25+ (T(REG)) cells and the Mean Fluorescence Index (MFI) of T(REG) in the peripheral blood of patients with SLE and to correlate these findings with their disease activity scores and drug therapy. This study included 24 SLE patients with various disease activity scores (SLEDAI) and 24 healthy age and sex matched controls. Flow cytometry was used to examine the frequency of CD4+CD25+ T cells and the MFI of CD4+CD25+(high) T cells (T(REG)). CD4+CD25+ T cells % and MFI of CD4+CD25+(high) T cells were higher in SLE patients than controls (p value=0.62 and=0.037 respectively) and both CD4+CD25+ T cell % and the MFI of CD4+CD25+(high) T cells showed highly significant correlation with SLEDAI scores (both with a p value<0.001) and were higher in patients taking glucocorticoids than those not on glucocorticoids (p= 0.023, 0.048 respectively). We conclude that the increase in T(REG) cells in our patients may be due to corticosteroid treatment.