Protein Kinase C δ: a Gatekeeper of Immune Homeostasis

Human autoimmune disorders present in various forms and are associated with a life-long burden of high morbidity and mortality. Many different circumstances lead to the loss of immune tolerance and often the origin is suspected to be multifactorial. Recently, patients with autosomal recessive mutations in PRKCD encoding protein kinase c delta (PKCδ) have been identified, representing a monogenic prototype for one of the most prominent forms of humoral systemic autoimmune diseases, systemic lupus erythematosus (SLE). PKCδ is a signaling kinase with multiple downstream target proteins and with functions in various signaling pathways. Interestingly, mouse models have indicated a special role of the ubiquitously expressed protein in the control of B-cell tolerance revealed by the severe autoimmunity in Prkcd ^?/? knockout mice as the major phenotype. As such, the study of PKCδ deficiency in humans has tremendous potential in enhancing our knowledge on the mechanisms of B-cell tolerance.     

Deep neck space infections: a retrospective review of 173 cases

Purpose

The purpose of this study is to review our recent experience with deep neck infections and emphasize the importance of radiologic evaluation and appropriate treatment selection in those patients.

Materials and Methods

The records of 173 patients treated for deep neck infection at the Department of Otolaryngology and Head and Neck Surgery of Dicle University Hospital during the period from 2003 to 2010 were retrospectively reviewed. Their demography, symptoms, etiology, seasonal distribution, bacteriology, radiology, site of deep neck infection, durations of the hospital admission and hospital stay, treatment, complications, and outcomes were evaluated. The findings were compared to those in the available literature.

Results

Dental infection was the most common cause of deep neck infection (48.6%). Peritonsillar infections (19.7%) and tuberculosis (6.9%) were the other most common cause. Pain, odynophagia, dysphagia, and fever were the most common presenting symptoms. Radiologic evaluation was performed on almost all of the patients (98.3%) to identify the location, extent, and character (cellulitis or abscesses) of the infections. Computed tomography was performed in 85.3% of patients. The most common involved site was the submandibular space (26.1%). In 29.5% of cases, the infection involved more than one space. All the patients were taken to intravenous antibiotic therapy. Surgical intervention was required in 95 patients (59.5%), whereas 78 patients (40.5%) were treated with intravenous antibiotic therapy alone. Life-threatening complications were developed in 13.8% of cases; 170 patients (98.3%) were discharged in stable condition.

Conclusion

Despite the wide use of antibiotics, deep neck space infections are commonly seen. Today, complications of deep neck infections are often life threatening. Although surgical drainage remains the main method of treating deep neck abscesses, conservative medical treatment are effective in selective cases.     

Testicular Effects of Vasectomy in Rats: An Ultrastructural and Immunohistochemical Study

Objectives. The correlation between infertility and morphofunctional alterations following vasal occlusion is not clearly understood. Although a correlation has been found between the expression of a high titer of antisperm antibodies and the status of infertility, the relationship between the immunoglobulin (Ig) depositions in the testis and ultrastructural alterations of the peritubular structures has not been clearly established. The objective of this study is to explain the etiopathogenesis of diffuse and focal testicular lesions caused by vasal obstruction.Methods. Unilateral vasectomy was performed on adult male rats. Ultrastructural changes of testicular tissues and immunostaining affinity of peritubular structures with anti-actin, anti-vimentin, anti-laminin, and anti-immunoglobulin G (anti-IgG) antibodies were analyzed.Results. In an ultrastructural study, vasectomized animals presented abundant collagen fibril accumulation in the peritubular area. In testis with intense IgG antibodies, staining revealed weak immunostaining of peritubular myoid cells with anti-actin antibodies, but intense immunostaining with anti-vimentin antibodies. The tubules of rats with no IgG deposition on peritubular structures revealed intense immunostaining with anti-actin antibodies but scant immunostaining with anti-vimentin antibodies. Anti-laminin deposits decreased severely in most of the tubules demonstrating intense IgG deposition.Conclusions. Our findings suggest that spermatogenesis deteriorates more severely in testes with dense IgG deposition. The sclerosis of the lamina propria in cases with vasal occlusion is secondary to alterations in the peritubular myoid cells. With the progressive changes that occur in the peritubular myoid cells or differentiation of the peritubular myoid cells that acquire active cell characteristics, collagen accumulation adjacent to these cells increases markedly. The alterations of the peritubular myoid cells reported here may be caused by alterations in basement membrane structures.     

Eosinophilic granuloma of the orbit: report of two cases.

We present clinical, radiological, and histopathological findings and treatment results of two infants with orbital eosinophilic granuloma. Both patients presented at 1 year of age with unilateral proptosis, periorbital edema, and a superotemporally located, ill-defined orbital mass on computed tomographic (CT) imaging, with bone erosion in one patient. After subtotal excision, histopathological examination revealed eosinophilic granuloma, and the proptosis resolved with no sign of orbital tumor recurrence or systemic involvement at 12 and 23 months follow-up. Eosinophilic granuloma is a rare childhood orbital tumor that may or may not present with osteolytic change.     

Expression of bcl-2 and Epstein-Barr virus LMP1 in lymphocytic interstitial pneumonia

BACKGROUND: Epstein-Barr virus (EBV) genome has been demonstrated in lung tissues of patients with lymphocytic interstitial pneumonia (LIP) but its role in the pathogenesis of this condition is unclear. In vitro studies have shown that EBV can immortalise and transform cells by upregulation of the cellular proto-oncogene, B cell leukaemia-2 (bcl- 2), via the viral latent membrane protein, LMP1. The purpose of this study was to determine whether bcl-2 expression is upregulated in the lungs of patients with LIP and whether EBV LMP1 has a role in this bcl- 2 expression. METHODS: Immunohistochemical analysis using alkaline phosphatase anti-alkaline phosphatase (APAAP) was performed on formalin fixed, paraffin embedded lung tissues from 13 patients with LIP using anti-LMP1 and anti-bcl-2 monoclonal antibodies. Lung tissues from nine patients with idiopathic pulmonary fibrosis (IPF) and nine necropsy cases without pulmonary disease served as controls. LMP1 positivity was estimated as the number of LMP1 positive cells per unit area of lung tissue. Immunostaining for bcl-2 expression was assessed by a pictorial- based semiquantitative grading system. RESULTS: Positive immunostaining for LMP1 was localised to airway epithelial cells of lung tissue. Ten out of 13 (77%) patients with LIP were positive for LMP1 compared with three of nine cases (33%) in each control group. LMP1 positivity of LIP cases was significantly greater than that of non-LIP cases: LIP versus IPF (mean difference, 95% confidence interval (CI)) 2.39 (1.54 to 3.24); LIP versus necropsy controls 2.62 (1.77 to 3.47). bcl-2 immunostaining was localised to lymphocytes within the alveolar septa and lymphoid aggregates of patients with LIP. The cumulative score for bcl-2 immunostaining was significantly higher in the lungs of patients with LIP than in those of patients with IPF and necropsy controls: LIP versus IPF and LIP versus necropsy controls (mean difference, 95% CI) 7.55 (7.18 to 7.92). CONCLUSIONS: These immunohistochemical studies have shown the presence of EBV LMP1 protein in airway epithelial cells and overexpression of the cellular bcl-2 protein in lymphoid cells of lung tissue in patients with LIP. These geographically distinct staining patterns of immunostaining suggest that the involvement of EBV LMP1 in the upregulation of cellular bcl-2 is more complex in LIP than was thought from previous in vitro observations. The respective roles of EBV LMP1 and bcl-2 in the pathogenesis of LIP require further studies.


     

Genetic features of retinitis pigmentosa in Turkey

Sixty-two cases with retinitis pigmentosa from 42 index families were investigated to reveal the genetic features of the disease in Turkey. There were 42 propositi of whom 5 had a systemic syndrome associated with retinitis pigmentosa. Of the remaining 37 cases the condition was autosomal recessive in 21 (56.8%), sporadic in 12 (32.4%), autosomal dominant in 3 (8.1%) and X-linked recessive in one (2.7%). Sporadic cases may be more frequent as many hereditary cases are not brought to medical attention in rural families. Male preponderance among sporadic cases may indicate that there may be more X-linked cases. Nine out of 21 cases initially classified as sporadic displayed parental consanguinity and they were included as having autosomal recessive trait. Large families with autosomal recessive inheritance may prove valuable in linkage analysis and in defining future gene abnormalities.     

Depression, automatic thoughts, alexithymia, and assertiveness in patients with tension-type headache

OBJECTIVE: The role of psychological factors related to headache has long been a focus of investigation. The aim of this study was to evaluate depression, automatic thoughts, alexithymia, and assertiveness in persons with tension-type headache and to compare the results with those from healthy controls. METHODS: One hundred five subjects with tension-type headache (according to the criteria of the International Headache Society classification) and 70 controls were studied. The Beck Depression Inventory, Automatic Thoughts Scale, Toronto Alexithymia Scale, and Rathus Assertiveness Schedule were administered to both groups. Sociodemographic variables and headache features were evaluated via a semistructured scale. RESULTS: Compared with healthy controls, the subjects with headache had significantly higher scores on measures of depression, automatic thoughts, and alexithymia and lower scores on assertiveness. Subjects with chronic tension-type headache had higher depression and automatic thoughts scores than those with episodic tension-type headache. CONCLUSIONS: These findings suggested that persons with tension-type headache have high depression scores and also may have difficulty with expression of their emotions. Headache frequency appears to influence the likelihood of coexisting depression.     

Diagnostic accuracy of magnetic resonance imaging and clinical signs of temporomandibular joint disorders: a 10-year research update review

Objectives

To review the past 10 years of research in the diagnostic accuracy of magnetic resonance imaging (MRI) and clinical signs of temporomandibular joint (TMJ) disorders with pain, sound, and function limitation in the existing literature.

Methods

A review of the literature was performed to search for all articles published between 2006 and 2016, and those that met the selection criteria were examined.

Results

The MRI results ranged from 25.3 to 69% for findings in TMJ with sound, 17–74.5% for findings in TMJ with function limitation, and 13.3–77% for findings in TMJ with pain. The reasons for the TMJ symptoms were variable and MRI findings for temporomandibular disorders were seen in asymptomatic patients as well as symptomatic patients. No singular outcome measure can be suggested as a predictor for TMJ symptoms on MRI images.

Conclusions

To increase study comparability with MRI, a standardized terminology and evidence-based guidelines are required for correlations of clinical symptoms and MRI findings.

     

Cone-beam computed tomography evaluation of palatogingival grooves: a retrospective study with literature review

Objectives

The purpose of the present study was to radiographically evaluate the prevalence and characteristics of palatogingival grooves (PGs) in maxillary anterior teeth on cone-beam computed tomography (CBCT) to better understand the nature of these defects.

Methods

The CBCT examinations of 993 teeth (330 canines, 315 lateral incisors, 348 central incisors) in 191 patients (87 males, 104 females; age range 16–80 years) were evaluated retrospectively. The diagnosis and radiographic condition of PGs were obtained by consensus among all observers. Verification of PGs was achieved from the patients’ clinical records, which were stored in a database after their clinical evaluation. Differences in age, sex, occurrence, and location were evaluated by the Chi-square test. The level of significance was set at p < 0.05.

Results

Among the 993 teeth examined, nine PGs were observed in seven lateral incisors and two central incisors in eight patients (four males and four females). There was one bilateral case in the lateral incisors. The frequencies of PG occurrence and affected patients were 0.90 and 4.18%, respectively, without significance for sex and location (p > 0.05).

Conclusions

PGs were a relatively infrequent anomaly of teeth in this population, but when present, clinicians should understand the clinical features of these root variations.