Atypical Presentation of Adenosine Deaminase Deficiency ADA2 Deficiency

Here we report on a challenging diagnosis of VIAHS in a patient who presented at a very early age with a perianal abscess with fistula formation, fever, aphthous ulcers, bicytopenia, and hematochezia. Marked phenotypic variability can occur, and screening for families should be initiated in those with ADA2 mutation.

Propolis and Its Implications in Dentistry: A Review

Propolis is a resinous substance obtained from the beehives that has antioxidant, anti-bacterial, anti-viral, antifungal, and anti-inflammatory activity. Its diverse chemical content is responsible for many valuable properties. Multiple applications of propolis have been studied and described in detail for centuries. Propolis has been used for surgical wound healing, caries prevention, treatment of dentin hypersensitivity, treatment of aphthous ulcers and propolis as a storage medium for avulsed teeth, root canal irrigating solution, and mouthwash. This paper aims to indicate the uses of Propolis in various fields of dentistry. Keywords: Dentistry, Propolis, Oral health.

Prevalence and Risk Factors of Oral Mucosal Lesions: A Retrospective Study of Patients Attending Oral Diagnosis Department of Siohs Karachi, Pakistan

Background: The present study was conducted to evaluate the frequency of different types of Oral Mucosal Lesions (OMLs) and to identify the associated risk factors. Methodology: A retrospective study was conducted at the Department of oral diagnosis in collaboration with the department of oral pathology SIOHS, Karachi Pakistan between January 2017 to December 2020. All cases of lesions and ulcers of the oral cavity were included in the study. Missing data were excluded. Diagnosed cases of oral mucosal lesions were documented. The data was then stratified according to the age groups, gender, symptoms, comorbidities, marital status, etc. Results: A mean age of 38.3 ± 11.86 years was observed. The subjects manifested multiple lesions with OSF standing as the most prevalent one at 29%, followed by aphthous ulcers at 20.2%, candidiasis at 14%, and nicotinic stomatitis at 10.4%. The majority of the patients with aphthous ulcers were significantly associated with young age while frictional keratosis was significantly associated with elderly age (p=0.002), respectively. 15 (93.8%) patients who were married were diagnosed with squamous cell carcinoma (p<0.004) and stiffening of the mouth was significantly associated with squamous cell carcinoma (p<0.0001). Conclusion: In conclusion, the increased occurrence of oral mucosal lesions with age is most likely attributable to an age-related decline in health status and is highly correlated to substance abuse; its amount, duration, type, and regularity. Keywords: oral mucosal lesion, carcinoma, gingiva, buccal, maxillofacial, tumor

Clinical Effect of Non-Thermal CO2 Laser Therapy (NTCLT) on Pain Relief of Oral Aphthous Ulcers of Behçet’s Disease

Introduction: Behçet’s disease (BD) is a debilitating and chronic vasculitis which can affect multiple organs. Recurrent oral aphthosis is the most common clinical feature of BD. The remarkable pain associated with painful oral ulcers can exert a major impact on the patients’ quality of life. Relieving pain of these oral ulcers is an essential approach in the management of mucocutaneous lesions of BD. Non-thermal CO2 laser therapy (NTCLT) as a novel photobiomodulative approach has recently been used for significant and immediate pain reduction of some types of painful oral lesions such as recurrent aphthous stomatitis, pemphigus vulgaris, and so on. In this procedure by some considerations, the conventional CO2 laser is used as a non-surgical, non- thermal, photobiomodulative laser without any visible thermal adverse effects. In this article, we report our experiences about the analgesic effects of NTCLT on painful oral aphthous ulcers of BD. Case Presentation: We report four cases of BD, whose painful oral aphthous ulcers were irradiated with NTCLT. Immediately after NTCLT, the pain of the lesions was significantly relieved with no visible complications. Conclusion: The results of this study suggest that NTCLT could be proposed as a hopeful procedure for significant and instant pain relief of oral aphthous ulcers of BD without any visible thermal adverse effects.

Chronic Lymphocytic Leukemia, T-Cell Large Granular Lymphocytic Leukemia in a Pediatric Patient without Underlying Condition

Background Large granular lymphocytic (LGL) leukemia is a rare hematological malignancy in children. The two types of LGL leukemia that have been described are T-cell and Natural Killer cell leukemia. It is most commonly diagnosed in older adults, average age of 60-year-old. About 20 cases of LGL leukemia have been reported in children and young adults. All the patients in the reported cases had immune dysregulation conditions, such as chronic graft versus host disease, common variable immunodeficiency disorder, Crohn's disease and autoimmune hemolytic anemia. Here we report a case of T-cell LGL leukemia in a 11-year-old boy without underlying condition who presented with chronic neutropenia associated with gingival hypertrophy, recurrent skin abscesses, aphthous ulcers, clubbing of nails and low bone density. Methods Multi-institution collaboration and literature review. Case Description 11-year-old male with two years history of episodic gum bleeding with gingival hypertrophy, skin abscesses, aphthous ulcers, chronic neutropenia and lymphocytosis presented to our clinic for further evaluation. Initial workup demonstrated moderate to severe neutropenia (absolute neutrophil count between 400/ul to 800/ul) with low segmented neutrophils of 2-4% and high lymphocytes of more than 80%, but normal white blood cell count, hemoglobin for age and platelet count. Peripheral blood smear showed several variant lymphocytes with cytoplasmic blebs and no immature cells present. Expansion of T-cell large granular lymphocytes were detected in peripheral blood by flow cytometry. Due to new symptom of lower back pain, a lumbar Magnetic Resonance Imaging was performed. Results showed low bone density with mild compression deformity of L1 and abnormal heterogeneous marrow signal with heterogeneous contrast enhancement. The abnormal bone marrow signal promoted the investigation of bone marrow aspiration and biopsy. Flow cytometry detected forty-five percent of lymphocytes with immuno-phenotype of CD3+, CD8+, CD57+, CD16+, CD7+ and CD5-. The morphology of minimal cytoplasm and mature chromatin along with immunophenotype were consistent with clonal T-cell large granular lymphocytic proliferation/leukemia. Further cytogenetic tests showed TCR gamma and beta genes rearrangement, STAT3 N647I mutation with normal male karyotype. A peripheral blood congenital neutropenia panel, which included a total of 18 genes, found a heterozygous mutation c 279 G&gt;A in the Gata2 gene; a variant of uncertain significance. Next generation sequencing showed somatic mutations of TRGV10, TRGV8 TRGJ1, TNFAIP3 and STAT3. However, there was no germline mutations detected in sample from skin biopsy. Comprehensive evaluation by immunology, rheumatology and gastroenterology failed to detect any underlying conditions. Conclusion Due to rarity of LGL leukemia in pediatrics, standard of care guidelines are currently unavailable. Extrapolated from limited literature, two management options are considered: watch and wait approach versus early initiation of immunosuppressant chemotherapy. Improved diagnostics can aide management strategies in this patient population. Disclosures No relevant conflicts of interest to declare.

Focus on Phytochemical Screening, Chemical Constituents, Pharmacological Effects and Medical Uses of Gummi myrrha

Gummi myrrha is the air-dried gum resin taken from the branches and stems of Commiphora molmol Engler (Burseraceae). The other names include myrrh, myrrhe, myrrha. Commiphora species are shrubs with 3 m high. It has rounded tops, thick trunks, dark brown bark, and large, sharply pointed thorns on the stem. It has many asymmetrical stunted and spiny. The leaves are unequal and alternate. The flowers are small, yellow-red fascicled, and arranged in terminal panicles. Gummi myrrha contains resins (25-40%), essential oil (3-8%), and a water-soluble gum (30-60%). The Gummi myrrha contains 20% proteins and 65% carbohydrates (galactose, 4-O-methylglucuronic acid, and arabinose). The major constituents of the Gummi myrrha essential oil are furanosesquiterpenes, and the monoterpenes α-, β- and γ-bisabolene. Gummi myrrha is used for mild inflammations treatment. It is used to treat aphthous ulcers, pharyngitis, tonsillitis, common cold, and gingivitis. Gummi myrrha is used as an emmenagogue, expectorant, and antidote for toxins and to stop blood coagulation. It treats menopausal symptoms, arthritic pain, diarrhea, fatigue, headache, jaundice, and indigestion. The pharmacology activity of Gummi myrrha includes experimental pharmacology and clinical pharmacology. Experimental pharmacology includes cardio-protective, analgesic, antipyretic, anticoagulant, antidiabetic, anti-inflammatory, cytoprotective, antimicrobial, and antileishmanial activities. Clinical pharmacology includes anti-obesity, antidiarrheal, and wound healing activities. The ointment of Gummi myrrha essential oil was non-irritating, non-sensitizing, and non-photo toxic to the human skin. The dose of myrrh tincture =1:5 g/ml, Gummi myrrha tincture applied to the affected area 2 or 3 times/ day; Gummi myrrha mouth solution= 5-10 drops of the tincture in a glass of water.

Development of Triphala Extract-Loaded Mucoadhesive Films for Aphthous Ulcers

Triphala is a traditional Thai herbal formulation containing dried fruits of Phyllanthus emblica, Terminalia bellirica, and Terminalia chebula. It has wound healing, antioxidant and anti-inflammatory activities. The objective of this research was to formulate mucoadhesive films containing Triphala extract for aphthous ulcers treatment. The films were formulated using hydroxypropyl methylcellulose, HPMC, (5, 8, 10% w/w) as a film-forming polymer and glycerin (5% w/w) as a plasticizer. Triphala extract (2.5, 5, 10% w/w) was incorporated into the film during the film preparation. The films were then evaluated for the physical appearance, dissolution time, mechanical properties (strength and elasticity) and mucoadhesive capability to the porcine buccal mucosa. The antioxidant activity and anti-inflammatory activity of the films were also evaluated by DPPH assay and the proteins denaturation method, respectively. Physical properties revealed that Triphala-loaded HPMC films were transparent with brown color. All formulations showed 1-2 hr of dissolution times. Triphala films exhibited good mucoadhesive properties. Films prepared from the solution containing HPMC (10% w/w), glycerin (5% w/w), and Triphala extract (10% w/w) were the most appropriate formulation for further development due to suitable strength, elasticity, and mucoadhesive properties. Moreover, the films exhibited antioxidants and anti-inflammatory activity which may help relieve the symptom of aphthous ulcers.

Development of Mucoadhesive Film-Forming Systems Containing Black Ginger Extract for Aphthous Ulcers

Film-forming systems (FFSs) were developed by using Eudragit® E100 as a film former. Kaempferia parviflora (black ginger) extract was used as an anti-inflammatory agent for aphthous ulcers. The FFS could rapidly form a thin film in only 5 s when it was applied to a wet surface e.g. an aphthous ulcer. When the FFS was applied to a dry surface, the FFS without extract could form a film in 2-4 min. The incorporation of this extract contributed to delaying the film-formation time in the dry state; hence, the film-forming time increased to 6-8 min. The mucoadhesive property of FFSs was verified with the wash-off method. To simulate oromucosal conditions, the FFSs were applied on a cellophane membrane coated with mucin and washed by phosphate buffer of pH 6.8. The formulations without mucoadhesive polymers could not withstand flushing with a medium for more than 8 min without dislodging. Therefore, three different mucoadhesive agents were trialed: PVP K90, HPMC E15 LV, and HPC SL. The highest adhesion results were obtained when HPMC was added at 5%(w/w) as well as, the residence time was 22 min. In vitro release of black ginger extract from FFS showed a gradual release for 2 h. This study indicated that the FFS with HPMC E15 LV was an appropriate alternative formulation as a local delivery system for an aphthous ulcer.

Awareness Regarding Oral Aphthous Ulcers and its Risk Factors Among BDS and MBBS Students-A Cross-Sectional Survey

OBJECTIVE: The objective of this paper was to assess the awareness regarding oral aphthous ulcers and its risk factors among BDS and MBBS students in private institute of Karachi, Pakistan. METHODOLOGY: A cross-sectional survey was conducted at a private institute of Karachi Pakistan for the duration of six months. Medical and dental students of age more than 18 years of either gender were enrolled in the study using non-probability convenience sampling technique. Pre-designed proforma was used to collect data regarding demographics, history of recurrent aphthous stomatitis and knowledge of oral aphthous ulcers and its risk factors. The knowledge score of 4 out of 6 was considered as adequate knowledge. SPSS version 25 was used to analyze data. RESULTS: Of 150 participants, the mean age was reported as 22.94±1.65 years (range: 21-25 years). In those 150 participants 46 were males (30.7%) and 104 were females (69.3%). Overall mean score of knowledge was 4.35±1.29. Wherein 73% had adequate knowledge regarding oral ulceration and its risk factors. The dental students had significantly higher proportion of adequate knowledge regarding oral ulceration than medical students [p=0.024]. CONCLUSION: Overall students had good knowledge of oral ulceration and its risk factors, wherein knowledge of dental students was better than medical students. KEYWORDS: Oral ulcers; Recurrent Aphthous stomatitis; awareness, oral ulceration, risk factors, eating habits