OP0173 REDUCTION OF SKIN FIBROSIS IN SYSTEMIC SCLEROSIS ON RITUXIMAB TREATMENT. LONG-TERM FOLLOW-UP

Background:A sound experience has been accumulated up to date with the use of rituximab (RTХ) for treatment of systemic sclerosis (SSc). Some studies reported improvement of skin fibrosis following treatment with RTХ, but long-term follow-ups are really few.Objectives:to evaluate the effect of RTХ on the manifestations of skin fibrosis in patients (pts) with SSc in the long-term follow-up.Methods:This prospective study included 71 pts aged 46 years (17-66) on average, 59 (83%) pts were females, mean disease duration was 5,6±4,4 years, and mean follow-up - 42 months (12-72) (mo). Diffuse SSc was established in 42 (59%) pts. All pts received glucocorticoids in low doses. 45% of pts were receiving immunosuppressants at study entry. The following parameters were evaluated: Rodnan skin score (mRSS), interdigital space(IDS) (the distance between the tips of 1 and 5 fingers at maximum extension), oral aperture (OAp) and activity index (EScSG-AI) over the periods: 12-18 mo, 24-30 mo, 36-42 mo, 48-54 mo and 60-72 mo after initiation of RTX therapy. The results are presented as: mean values, delta (Δ), median, upper and lower quartiles.Results:RTX therapy resulted in significant decrease of disease activity index, which statistically significantly correlated with decrease of mRSS - the main indicator of the severity of skin fibrosis (r=0,39;p=0,001). Changes in parameters by follow-up periods are presented in the Table 1.Table 1.Changes in clinical and instrumental parameters at RTX treatment (delta; median; lower quartile; upper quartile).Parameters12-18 mo (n=71)24-30 mo (n=55)36-42 mo (n=36)48-54 mo (n=24)60-72 mo (n=17)ΔmRSS3,32 [3.3; 0; 8]5,4 [3; 0; 10]5,1 [3,5; 0; 9]5,3 [3; 0; 10]7,3 [5; 1; 14]p=0,001p=0,001p=0,001p=0,001p=0,001ΔOAp, cm0,24 [0,1; 0; 0,5]0,26 [0,1; 0; 0,6]0,39 [0,2; 0; 0,8]0,31 [0.3; 0; 0,7]0,36 [0,2; 0; 0,8]p=0,0009p=0,0006p=0,004p=0,006p=0,009ΔActivity index (EScSG-AI)1,49 [1,5; 0; 2,5]1,64 [1,5; 0; 2,5]1,11 [1; 0; 2]2 [2; 1; 3]2,17 [2; 1,5; 2]p=0,001p=0,001p=0,0001p=0,0001p=0,0001Cumulative dose of RTX, g1,43±0,62,97±0,83,45±1,33,96±1,15,15±1,7Decreasing of mRSS statistically significantly correlated with increasing cumulative dose of RTX (r=0,29;p=0,01). Decreasing disease activity index correlated with increasing cumulative dose of RTX (r=-0,37; p=0,01). IDS improvement was documented at all assessment time periods, although statistically insignificant.Conclusion:The results of this study confirm reported positive effect of RTX on the reduction of skin fibrosis in SSc. Long-term follow-up demonstrated steadily decreasing skin fibrosis and improvement of microstomia with increasing oral aperture in parallel with a decrease of the disease activity index and increasing cumulative dose of RTX.Disclosure of Interests:None declared

Effect of Preoperative Single Dose Dexamethasone Injection in Masseter Muscle on Postoperative Sequelae Following Lower Impacted Third Molar Surgery

Introduction: The surgical removal of impacted lower third molars involves trauma to soft and bony tissue and can result in pain, swelling and trismus. The purpose of this study was to evaluate the efficacy of dexamethasone, as a single 4 mg dose injected into the masseter muscle prior to extraction of impacted lower third molars on theses postoperative sequelae. Materials & Methods: This prospective, randomized clinical research consisted of 43 healthy patients. The study group received 4 mg dexamethasone into the masseter muscle via intrabuccal approach immediately before starting the procedure while the control group received no corticoid. 7 days after surgery. Data were analyzed using t-test (α = 0.05). Results: The patients administered dexamethasone showed superior results after surgery in terms of oral aperture, pain and all the facial swelling parameters, with statistically significant differences versus the controls (p value<0.001). Also there was statistically significant difference in terms of patients trismus between case and controlgroups (p value<0.001). Conclusion: The results obtained showed that 4 mg of dexamethasone injected into the masseter muscle in the immediately before starting the procedure significantly reduces swelling, trism us and pain.

Toxocara canis and Toxocara cati Somatic and Excretory-Secretory Antigens Are Recognised by C-Type Lectin Receptors

Toxocara canis and Toxocara cati, the worldwide occurring intestinal roundworms of canids and felids, represent an important public health threat due to various disease manifestations in humans. Host recognition of pathogens is mediated by pattern recognition receptors (PRRs). Myeloid C-type lectin receptors (CLRs) are PRRs and recognise carbohydrate structures of various pathogens. As Toxocara excretory-secretory products (TES) are predominantly composed of glycoconjugates, they represent suitable targets for CLRs. However, the range of host-derived CLRs recognising Toxocara spp. is still unknown. Using a CLR-hFc fusion protein library, T. canis and T. cati L3 somatic antigens (TSOM) were bound by a variety of CLRs in enzyme-linked immunosorbent assay (ELISA), while their TES products interacted with macrophage galactose-type lectin-1 (MGL-1). Two prominent candidate CLRs, MGL-1 and macrophage C-type lectin (MCL), were selected for further binding studies. Immunofluorescence microscopy revealed binding of MGL-1 to the oral aperture of L3. Immunoblot experiments identified distinct protein fractions representing potential ligands for MGL-1 and MCL. To evaluate how these interactions influence the host immune response, bone marrow-derived dendritic cell (BMDC) assays were performed, showing MCL-dependent T. cati-mediated cytokine production. In conclusion, MGL-1 and MCL are promising candidates for immune modulation during Toxocara infection, deserving further investigation in the future.

A mixed method study exploring patient satisfaction and feasibility of two exercise programs in systemic sclerosis associated microstomia

Objectives Systemic sclerosis (SSc) is often leading to fibrotic cutaneous involvement of the face and reduced oral aperture with impaired food intake and oral hygiene. Oral exercises can increase oral aperture but are often hampered by low adherence rates. The aim of this mixed method study was to explore feasibility, patient satisfaction and effectiveness of two exercise programs in SSc-associated microstomia. Methods Adult patients suffering from SSc and microstomia (maximal oral aperture &lt;40mm) were randomized to two groups. Group A exercised with a jaw motion device (Therabite®), and Group B performed mouth-stretching exercises. Patients were expected to exercise for 10 minutes, 3 times/day for 3 months. Patients were evaluated at baseline, 3 months (period without intervention), 6 months (after 3 months of intervention) and at 9 months (post-intervention). At month 6 semi-structured one-to-one interviews were conducted. Results We included 6 women and 3 men, median age 60 years and median disease duration 8 years. At 6 months, all patients in group A (n = 4) and 4 in group B (n = 5) improved with a median of 9mm and 7mm, respectively. The adherence ranged between 63.7% and 98,9% in group A and between 48.5% and 97,4% in group B. The interview that revealed three themes: drivers, challenges and perceived improvement. Conclusion Both interventions improve maximal oral aperture. The adherence to therapy was high but none of the patients considered it feasible to continue practicing 3 times/day. Future studies are needed in order to define feasible long-term exercise programs.

A Mixed Method Study to Explore the Feasibility and Patient Satisfaction of Two Different Exercise Programs in Systemic Sclerosis Associated Microstomia

BackgroundSystemic sclerosis (SSc) is a severe autoimmune disease often leading to fibrotic cutaneous involvement of the face. Reduced oral aperture is associated with impaired food intake, oral hygiene and secondary dental problems. Stretching and oral augmentation exercises can increase oral aperture but are often hampered by low adherence rates. The aim of this mixed method study was to explore feasibility, patient satisfaction and effectiveness of two exercise programs in SSc-associated microstomia.MethodsAdult patients (<18 years) suffering from systemic sclerosis (fulfilling the ACR/EULAR 2013 criteria) and microstomia (maximal oral aperture <40mm) were randomized to two groups. Group A exercised with a passive jaw motion device (Therabiteâ), and Group B performed mouth-stretching exercises. Patients were expected to exercise for 10 minutes, 3 times/day for 3 months, completed an exercise diary and were contacted 4 times by telephone. Patients were evaluated at baseline, 3 months (period without intervention), 6 months (after 3 months of intervention) and at 9 months (post-intervention visit). At month 6 semi-structured one‐to‐one interviews were conducted, recorded, transcribed verbatim and analyzed using Qualitative Analysis Guide of Leuven (QUAGOL).ResultsWe included 6 women and 3 men, median age 60 years (range 40-75) and median disease duration 8 years (range 3-22). At 6 months, all patients in group A (n=4) and 4 in group B (n=5) improved with a median of 9mm (range 2-10) and 7mm (range 4-11), respectively. The proportion of executed to the planned number of exercises ranged between 63.7% and 98,9% in group A and between 48.5% and 97,4% in group B. Maintenance of the increase in oral aperture was noted in patients that continued to exercise daily. All 9 patients attended the interview that revealed three themes: drivers, challenges and perceived improvement. ConclusionBoth interventions improve maximal oral aperture. The adherence to therapy was high but none of the patients considered it feasible to continue practicing 3 times/day. Future studies are needed in order to define feasible long-term exercise programs.

A Review on Therapeutic Management for Oral Chronic Graft-versus-Host Disease

Chronic graft-versus-host disease (cGVHD) is one of the main complications of transplantation in allogeneic hematopoietic cells which results in reduced quality of life. cGVHD mostly affects the oral cavity, producing various symptoms and manifestations. It can lead to both short- and long-term complications such as limited oral intake, mucosal sensitivity, secondary malignancy and even early death. GVHD is an alloimmune and autoimmune disorder with a variable clinical course that commonly involves various tissues and organs. It can occur in both the acute and chronic forms. In the case of cGVHD, a large number of organs are affected including the oral cavity. However, in some cases of cGVHD only the oral cavity gets affected. The clinical indications of chronic oral GVHD may include sclerosis, hyperkeratotic plaques, lichenoid lesions, and limited oral aperture. The level of oral involvement is commonly mild, but medium to high severity erosive as well as ulcerated lesions may also be occurred. Although diagnosed through clinical examination, its confirmation is usually done by biopsy study. The first treatment option is using local corticosteroids with the potency to treat half of patients. Extracorporeal photopheresis and systemic corticosteroids are in the next ranks. Patient survival after diagnosis of oral chronic GVHD is within 4.5 years in 50% of the cases, thus it is not regarded as a determinant factor in patient survival. This study attempted to provide a detailed approach for clinical diagnosis and management of patients suffered from oral cGVHD. It is particularly considered the factors such as differential diagnosis, symptom control, screening for and prevention of secondary complications. It has been tried to provide practical and relevant considerations and recommendations for all clinicians who dealswith oral cGVHD patients in order to achieve improved care and outcomes.

A Review on Holoprosencephaly Disease (CYCLOPIA): Risk Factors, Causes, Pathophysiology and Diagnosis with spotlight of various features reported in cases

Holoprosencephaly has a sever condition called Cylopia that is occur due to embryonic prosencephalon cleavage failure and contrast. Mostly cylopia form is holoprosencephaly, mid facial tissue is absent which causes the one eye on a single orbit. It is a sever deformity of median faciocerebal development. There are 1.05 cases in100,000 birth, still births of cyclopean. Abnormal nose above eyes or absence of nose, single eyes or half divided eyes in single orbit are features of cyclopia, where as reduced size of oral aperture or absence of mouth, absence of mandible with ears below chin. It is as etiologically heterogeneous condition, which can be caused by genetic mutation, chromosomal defect and teratogenic environmental factors. Environmental factors can be diabetic embryopathy, retinoic acid, several anecdotal suggestion of teratogenic factor for HPE, which includes salicylates and viruses. Some list of syndrome are also involved to cause cyclopia Like steinfeld syndrome, dysgnathia complex,Pseudotrisomy 13 syndrome and Smith–Lemli– Opitz syndrome. On other hand inborn abnormalities also cause cyclopia but its come under chromosomal syndrome. Anatomical detection can be done by brain MRI, whereas in prenetal diagnosis, sonography is more significant. Ultrasound also used early detection can be done and knowledge of sonographic finding soectrum leads to accuracy of prenatal diagnosis of cyclopia. After birth the chromosomal study helps to diagnose cyclopia along with postmortem biopsy.

Description of Longidorus barsii Radivojević & De Luca sp. n. (Nematoda: Longidoridae) from Serbia and observations on some taxonomic characters

Summary Longidorus barsii sp. n., from Mt Tara in the Balkan Peninsula, is described and characterised by using a polyphasic approach. The species has numerous males. The female body is 5-7 mm long, rather stout and resembles a large Xiphinema. The lip region is wide, with rounded sides continuous with the neck, frontally flattened and depressed around the oral aperture, amphids are pouch-like and distinctly bi-lobed and the odontostyle is moderately long. The nuclei of the pharyngeal glands are in the normal position, the dorsal nucleus located somewhat posterior to anterior third of bulb. The uteri are long, the distal inner epithelium densely covered with papilla-like outgrowths. The tail is rounded, bluntly conoid and very short. Alpha-numerical identification codes: A4/5, B45, C3, D3, E2, F3, G 1(2), H1, I2, J1, K67. The morphologically most similar species are L. kheirii, L. polyae and L. profundorum. Additional observations are provided on the anterior body region and genital organs in L. barsii sp. n., L. piceicola, L. silvae, and L. uroshis. Selected features are discussed from the taxonomic and functional points of view. The D2-D3 expansion domains of the 28S rRNA gene and the ITS region of L. barsii sp. n. were amplified and sequenced. Phylogenetic analysis using the D2-D3 expansion domains of the 28S rRNA gene revealed close evolutionary relationships with L. polyae, L. athesinus and three unidentified Longidorus spp.

Customized Microstomia Prevention Appliance Therapy: A Case Report

Microstomia is a condition with abnormally small oral aperture which is associated with compromised aesthetics and function of stomatognathic system. In young children, the leading cause of microstomia is oral electrical or chemical burns. Management of microstomia requires multidisciplinary approach including surgery, physiotherapy and appliance therapy. Appliance therapy in form of microstomia prevention appliance plays a pivotal role in prevention of contraction of tissue during healing. Numerous appliances have been described in literature to prevent microstomia. However, decision to use a particular appliance require meticulous planning considering the efficacy and effectiveness of the appliances and various patient factors like age, dentition status and compliance. This paper presents an unusual clinical condition where a unique customized microstomia prevention appliance therapy was instituted to prevent microstomia in 2 year old female patient following lip surgery. This appliance was constructed easily and inexpensively, could be adjusted so that it was almost painlessly inserted, and was progressively adapted. It was convenient for use in young child with minimal compliance offering improved mouth opening and consequently functional outcomes.