Role of Antioxidant Vitamins in Oral Submucous Fibrosis: A Narrative Review.

Oral submucous fibrosis (OSMF) has a high rate of malignant transformation and is an insidious chronic inflammatory disease. Though this disorder seems to be multifactorial in origin, betel quid chewing appears to be the main etiologic factor. Various treatment strategies have been attempted but none proven to cure the disorder because of its multimodal pathogenesis. Reactive oxygen species (ROS) appear to have a role in cancer formation. As OSMF is an oral premalignant disorder and found to be associated with carcinogens like areca nut and tobacco, it is believed to have some relationship with ROS. Tissue damage due to ROS along with other mechanisms may result in the complex pathophysiology of OSMF. The antioxidant system in the body helps to prevent damage caused by highly reactive ROS and helps in the repair of tissues. To study the levels of oxidative stress and antioxidant vitamins in OSMF condition, the present review was done. We carried out a thorough literature search to identify original reports and studies determining the status of oxidative stress and antioxidant vitamins in OSMF condition using several databases including Google Scholar, PubMed, and Scopus. Our review observed that the oxidative stress increased in the condition of OSMF as shown by an increase in malonaldehyde (MDA) and a decrease in antioxidant vitamins like vitamin A, vitamin C, and vitamin E. Also, after the intake of antioxidant vitamins, there was symptomatic improvement in OSMF patients. With the help of identifying oxidative stress and antioxidant status, we can assess the clinical stage of OSMF and can develop a comprehensive treatment plan.

Management of Herpes Zoster Infection Using a Combination of Allopathic and Herbal Medication: A Case Report.

Herpes zoster (HZ) also known as shingles is the reactivation of varicella-zoster virus (VZV) that causes chickenpox. It usually occurs in adults after remaining dormant in the dorsal root ganglia or ganglia of the cranial nerves for several years. It typically manifests as vesicle crops in a dermatomal or "zosteriform" pattern, which shows vesicle, ulcer, and scab distribution that is unilateral, clustered, and linear in a dermatome that is supplied by a single nerve. Patients usually experience prodromal symptoms of deep, severe aching or burning pain. Medicinal treatment frequently includes antiviral drugs to decrease the severity of the lesion and steroidal drugs to reduce symptoms of inflammation. It is also a known fact that steroid has several adverse effects on patients due to which therapeutic drugs with lesser side effects may be given to patients such as herbal medications. This case presentation reports a patient with HZ viral infection who was successfully treated with a meticulous combination of conventional allopathic drugs with ayurvedic medication with a significant positive response to the medication.

A Case Study on Pyogenic Granuloma With Review of the Literature: An Unexpected Sequela or a Complication of Dental Extraction?

Pyogenic granuloma is a common reactive oral lesion primarily found in the gingiva and rarely in extraction sockets. While it can develop at any age, it is more prevalent in the third and fourth decades of life with a higher occurrence in females. Various factors contribute to its development and surgical removal is the gold standard treatment; however, there are various other methods available. This case report documents a rare event in which a female patient in her early 40s presented with an exophytic lesion affecting the extraction socket of her maxillary right lateral incisor. The lesion was effectively removed through surgical excision. Additionally, it explores the clinical features and pathogenesis of this lesion. The purpose of this case report is to shed light on the uncommon incidence of pyogenic granuloma following tooth extraction. This non-neoplastic vascular growth often presents as an erythematous, ulcerated lesion with a tendency to bleed, with either a sessile or pedunculated base. Our case is one of only five instances documented in the literature, underscoring the importance of knowledge and timely response in such unusual circumstances. We emphasize the significance of early detection and management for improved patient outcomes and a better understanding of this rare condition.

Assessment of Cancer Patients' Relatives' Knowledge, Perception, and Attitude Toward Cancer.

Background Assessment of cancer patients' relatives' knowledge, perception, and attitude regarding cancer. To spread awareness about oral cancer in areas with a high prevalence of cancer, aid in its prevention, and accelerate the treatment to facilitate early-stage disease elimination and improve prognosis.  Material and methods A cross-sectional study conducted at a hospital was done, where 23 questions were posed to the relatives of the patients admitted to the hospital. The questions were formulated to assess the knowledge of relatives regarding cancer, their perception, and their attitude toward the decision-making and treatment protocols. The questions also included information about the patient's habits and associated problems with it. A total of 400 relatives participated in the study, where all participants were adequately informed beforehand, and their written consent was taken before proceeding with the questions. All the questions were formulated in the native language that they could easily understand.  Result The study found that participants had limited knowledge about the causes, treatment, prevention, and recurrence of oral cancer. Additionally, the family members were unsupportive of therapy and delayed seeking medical care. Conclusion It is crucial to raise awareness about oral cancer and inform individuals about available treatments, government programs, and counseling services to aid them in decision-making.

Chemical Composition of Areca Nut and Its Adverse Effects on Human Health.

Areca nut (AN) is one of the addictive substances consumed widely in the world. The composition of AN is very complex, and each component has variable properties. This study aims to review the composition of AN and its adverse effects on humans. For this review, the literature search was performed by an electronic search of the Pubmed/Medline, Scopus, and Google Scholar databases using proper MESH headings and retrieved the articles published from 1997 to 2022. The eligibility criteria included human studies, a form of AN, the composition of AN, harmful effects of AN, the effect of AN on the oral cavity, the effect of AN on vital organs, and articles published in English. Data were extracted regarding the composition of AN, forms of AN, and harmful effects of AN on the oral cavity and on other systems. A total of 449 articles were identified from various databases, and 36 studies were selected that met the inclusion criteria. The active components of AN, which produce harmful effects, are primarily alkaloids, polyphenols, tannins, and certain trace elements. AN is consumed in different forms, and based on the form, its composition also varies. AN is known to cause deleterious effects on the oral cavity as well as various body organs. The most dangerous and widely reported impacts of AN on the oral cavity are the development of oral submucous fibrosis, a premalignant condition, and oral malignancy. However, during the chewing process, excretory products of AN are released and circulate in the body of "chronic habitual" and affect the other body parts. Thus, AN consumption may contribute to cardiac, liver, endocrinal, metabolic, respiratory, and reproductive system disorders also. AN composition is complex, and its consumption is harmful to human health. In regard to controlling the issue of the harmful effects of this habit, preventive measures should be established.

Mast cell expression in oral submucous fibrosis: A systematic review and metanalysis.

BACKGROUND: Oral submucous fibrosis (OSMF) is a chronic disorder associated with reduced mouth opening, burning sensation and listed as potentially malignant disorder. The role of mast cells in initiation and progression of this condition has been debated in last few years. It is imperative to understand the definitive role of mast cells and subsequently identify a possible cost-efficient treatment modality for OSMF. This review aimed to study the role of mast cells in OSMF by framing a research question that assessed the mast cell count (MCC), their degranulation and immunohistochemical analyses. We performed a comprehensive search of PubMed, EBSCOhost and general Google search that conceded 26 studies from which 15 articles were finalized for the review. The individual study syntheses revealed increased MCC in OSMF as compared to controls. Also, there was decreased MCC with the progression of OSMF. However, the metanalysis showed high level of heterogeneity as three studies out of eight studies found reduced MCC in disease when compared with controls. There is definite increase in mast cell in OSMF although the cell count falls with the advancement of OSMF. This increases the scope for further research to identify exact mechanism by which mast cells contribute to fibrosis and conduct the drug trials that can inhibit the mechanism. Key words:Oral submucous fibrosis, mast cell count, degranulated mast cells, stages of OSMF.Oral submucous fibrosis, mast cell count, degranulated mast cells, stages of OSMF.

Conventional Radiology in Deep Seated Facial Hemangioma: A Case Report.

Hemangioma is congenital or traumatic in origin, and it is caused due to atypical build-up of the blood vessel. It is a painless benign condition with typical characteristic clinical features. These generally occur in the first three decades of life with no gender predispositions. A plain soft tissue radiograph can demonstrate phleboliths and aid in diagnosing an intramuscular hemangioma. The present report is a rare deep-seated facial hemangioma involving various facial muscles with multiple phleboliths; characteristics clinical and radiological features. A 22-year-old male patient reported a complaint of swelling on the right side of the jaw. Conventional radiography showed the right cheek's soft tissue and multiple round, target-like radiopacities of variable sizes.

Enzyme-linked immunosorbent assay and immunohistochemical analysis of mast cell related biochemicals in oral submucous fibrosis.

Oral submucous fibrosis (OSMF), a potentially malignant disorder, is developed by progressive fibrous tissue deposition in connective tissue along with atrophy of oral mucosa. Histological sections also show the mast cell infiltration in submucosa which may indicate their possible role in this entity. Abundant availability of biochemicals in mast cells like histamine and serine proteases like chymase may be released and play specific pathways in the disease pathophysiology. Possibly, if the histamine release has some part to play, diamine oxidase may also be found to have a relationship as it metabolizes histamine. The present study is proposed to identify the presence of chymase, histamine, and diamine oxidase in both, serum as well as tissue by enzyme-linked immunosorbent assay (ELISA) and immunohistochemistry (IHC) respectively. This study may provide probable insight into the mast cell-related chemicals and their association with OSMF.

Cone-Beam Computed Tomography for Temporomandibular Joint Imaging.

The temporomandibular joint (TMJ) can be viewed using various imaging techniques. Due to relatively low radiation doses and excellent spatial resolution, cone-beam computed tomography (CBCT) is being utilized more frequently in dental-maxillofacial imaging. For the diagnosis and treatment of TMJ disorders, an imaging examination is required. The osseous compartment is visualized using conventional CT, and CBCT and soft tissue imaging are extremely well appreciated on MRI. However, conventional TMJ imaging has its limitations due to its two-dimensional view and adjacent anatomical superimposition. TMJ imaging helps analyze the cortical and the bony compartment's trabaculae and assess the degree of skeletal abnormalities. TMJ imaging protocols are also used to evaluate treatment responses. CBCT is the three-dimensional imaging of the bony compartment and joint space and the morphology of the bone visualized by removing superimposition and distortion. Compared to multislice CT, CBCT produces high-resolution multiplanar images with a reduced dose of radiation. The role of CBCT imaging in determining the normal bony anatomy and pathological changes is appropriately delineated in this paper. This work will focus on the use of CBCT for the examination of TMJ in various patient categories, including those with osteoarthritis, remodeling, ankylosis, trauma, rheumatoid arthritis, synovial chondromatosis, and other intracapsular pathologies.

Mucosal Leishmaniasis Involving the Nostril and Maxillary Sinus: A Case Report.

An eight-year-old child presented with nasal discharge, stuffiness, and whitish polypoid swelling in the left nostril with Bitot's spot. Computed tomography (CT) evaluation showed complete involvement of the left nostril and maxillary sinus. Blood investigations revealed leukocytosis, raised absolute eosinophils, increased alkaline phosphatase, and reduced vitamin A levels. Histopathological examination revealed inflammatory infiltrate with Leishman-Donovan bodies, which confirms the diagnosis of mucosal leishmaniasis (ML). In the present case, the recording of demographic data is important as the child was a migrant from the leishmaniasis-endemic area of Bihar state, India. Nasal polypoid growth was removed by endoscopic surgery, followed by a combination of allopathic and polyherbal preparation. The child responded well to these therapeutic measures, and there was no recurrence of nasal discharge, stuffiness, and crustation at six-month follow-up.

Evaluation of serum vitamin B12 levels and its correlation with clinical presentation in patients with trigeminal neuralgia.

Background: The objective of this study was to estimate serum vitamin B12 levels and its correlation with severity of clinical presentation in patients with trigeminal neuralgia (TN). Methods: A total of 80 participants were included and rendered into study group: 40 TN patients and control group: 40 healthy individuals. The serum vitamin B12 estimation of each participant was carried out by using serum vitamin B12 ELISA kit and pain characteristics of TN patients were recorded in details. Results: The mean serum vitamin B12 level was noticed to be significantly lower (p = 0.042) in study group (296.87 ± 248.75 pg/mol) as compared to control group (423.5 ± 296.41 pg/mol). There was a statistically significant difference in serum vitamin B12 level between vegetarian TN patients and those who were on mixed diet (p = 0.001). The pain associated with TN predominantly reported unilaterally involving the right side (55%), female gender (55%) and mandibular division (50%). Although pain intensity showed no significant relation (p = 0.024), duration of pain reported a strong negative association with mean serum vitamin B12 levels in TN patients (p = 0.001). Conclusions: Vitamin B12 supplements can be added to the established treatment protocol for the holistic management of TN patients particularly those who are on vegetarian diet.

Risk of liver disease in areca nut habitual: A systematic review.

Background: Areca nut (AN) is one of the addictive substances known to cause deleterious effects on oral cavity as well as on various body organs including liver. But, scanty information is available reporting the adverse effects of AN chewing on the liver. Aim: To study the risk of liver disease in AN habitual based on the relevant published data. Methods: The literature search was performed by an electronic search of the PubMed/Medline, Scopus and Google Scholar databases using proper MESH headings and retrieved the articles published from 1998 to 2021. The eligibility criteria included: Human studies, AN habitual as study participants, use of controls and articles published in English. Data were extracted regarding characteristics of studies, characteristics of AN exposure, effect estimate and outcome of the studies. Results: Total 253 articles were identified from various databases and 15 studies were selected that met the inclusion criteria. Among these, thirteen studies showed an association between AN habit and attenuation of risk of liver disease as determined by relative risk/odds ratio/hazard ratio. Eleven studies described additive effect of AN and HBsAg and/or Anti hepatitis C virus status on development of liver disease. However, two of the studies showed opposite results. The heterogeneity in the study designs, exposure characteristics, outcomes and confounders precluded further meta-analysis. Conclusion: The association between AN chewing and an increased risk of developing liver disease is noted which necessitates the need for AN cessation campaign.

Serum aflatoxin B1 antibody titer, percent hemolysis and transaminases in oral submucous fibrosis.

INTRODUCTION: Areca nut is deeply rooted sociocultural habit in India. Areca nut reported to be infested by fungi during the field and storage conditions. Areca nut alkaloids, nitrosamines, tobacco and aflatoxin are cytotoxic, immunotoxic to red blood cell and epithelial cell. Hence, the present study was conducted to assess the serum aflatoxin B1 (AFB1) antibody titer, percent hemolysis and transaminases in oral submucous fibrosis (OSMF) patients. MATERIALS AND METHODS: In this study, 128 participants of which 88 were suffering from OSMF. Twenty participants were areca nut habitual without OSMF (habitual control) and 20 participants without any habit (healthy control). For the detection of AFB1 antibody titer, AFB1 antigen (Sigma) A6636 from Aspergillus flavus was used. Percent hemolysis was estimated as per the procedure described by Mathuria and Verma. Serum Glutamic oxalo acetic transaminase (SGOT) and Serum Glutamic pyruvic transaminase (SGPT) were estimated by the optimized ultraviolet method using the enzyme-linked immunosorbent assay kit. RESULTS: Mean SGOT, SGPT, percent hemolysis and AFB1 antibody titer were significantly higher in participants with OSMF than the habitual and healthy controls. AFB1 antibody titer and % hemolysis in both OSMF and habitual without OSMF were showed significant correlation, i.e., increased AFB1 antibody titer with increased % hemolysis. CONCLUSIONS: A study result demonstrates that aflatoxin causes increase in serum transaminases which is indicative of liver damage in OSMF. The combined toxic effects of areca nut alkaloids, tobacco and AFB1 on red blood cell (RBC) cell wall might be responsible for increased percent hemolysis in OSMF and habitual control.

Comparison of Efficacy of Topical Curcumin Gel with Triamcinolone-hyaluronidase Gel Individually and in Combination in the Treatment of Oral Submucous Fibrosis.

AIM: To study the efficacy of topical curcumin mucoadhesive semisolid gel, triamcinolone acetonide/hyaluronidase mucoadhesive semisolid gel, and a combination of both in the treatment of oral submucous fibrosis (OSMF). MATERIALS AND METHODS: One hundred and twenty patients diagnosed with OSMF were randomly divided into groups I, II, and III. Each patients in groups I, II, and III was given professionally prepared mucoadhesive semisolid gel of curcumin, a combination of triamcinolone acetonide and hyaluronidase mucoadhesive semisolid gel, and a combination of all three, respectively. Patients were instructed to apply the gel thrice daily for 6 weeks on buccal mucosa bilaterally using the tip of index finger. Three parameters were evaluated at the end of each week, namely, mouth opening, burning on visual analog scale (VAS), and the color of oral mucosa on the binary scale. The results were subjected to statistical analysis. RESULTS: It was observed that the group administered the three drug combinations achieved the greatest mouth opening (mean increase 4.05 mm) as compared to the other two groups. It was observed that triamcinolone and hyaluronidase group reported reduction in burning sensation on VAS (mean difference 6) as compared to the other two groups. It was observed that group III (1% curcumin, 1% hyaluronidase and 0.1% triamcinolone acetonide combined) drug therapy showed better change in mucosa color as compared to groups I (1% curcumin) and II. CONCLUSION: Thus, we can conclude that curcumin has a therapeutic effect on patients diagnosed with OSMF. Maximum utilization and enhanced drug delivery were achieved with the help of a combination other two active drugs, namely, triamcinolone and hyaluronidase. CLINICAL SIGNIFICANCE: Curcumin role in the treatment of oral cancers and the precancer lesion is very promising.

MRI evaluation of involvement of parotid and submandibular glands by tongue squamous cell carcinoma.

OBJECTIVES: The majority of cases of salivary gland involvement are diagnosed in its late stages as may be missed in the evaluation, the low detection rate of involvement of salivary gland by OSCC may have significant impact on spread and recurrence of the OSCC. The study aimed to evaluate involvement of parotid and submandibular salivary glands by tongue oral squamous cell carcinoma. MATERIAL AND METHOD: The 144 histo-pathologically proven cases of tongue squamous cell carcinoma were evaluated for clinical staging, margins and extent of tumor and involvement of salivary gland on T1-T2 Weighted, STIR, diffusion-weighted and contrast-enhanced MRI sequences and data was subjected to analysis. RESULTS: In tongue OSCC, total 9 (6.25%) patients showed salivary gland involvement, 2 of them involved parotid glands and 7 of them involved submandibular gland. The OSCC involving the salivary glands were in Stage 4 except for 2 cases with predilection for antero-lateral border and tendency towards midline crossing. Out of 9 cases that showed salivary gland involvement, the 5 cases showed direct involvement from the spreading lesion, the 3 cases showed the infiltration from adjacent lymph nodes and 2 cases showed infiltration from sublingual gland and medial pterygoid muscle. CONCLUSION: The present study revealed the greater percentage of OSCC with salivary gland involvement as compared to previous studies; in contradiction present study demonstrated higher involvement of submandibular gland compared to parotid gland with additional imaging features in salivary glands (15.97%) suggesting the likely possibility of their involvement by OSCC in near future.

Efficacy of Herbal Interventions in Oral Lichen Planus: A Systematic Review.

INTRODUCTION: Oral lichen planus (OLP) is a chronic autoimmune condition requiring prompt treatment to alleviate the signs and symptoms. There is weak evidence emphasizing the efficacy of any one therapy. Steroids, of all the therapies, have proved to be effective and hence considered as the standard care for OLP. However, the complications associated with it further worsen the patient's condition. Alternative safe approaches such as herbal interventions (HIs) have been tried in OLP. Their efficacies could only be evaluated from properly designed research protocols such as randomized controlled trials (RCTs). The present systematic review aims to assess the efficacy of HIs compared to steroids in RCTs involving OLP. MATERIALS AND METHODS: An extensive search for HIs in OLP was conducted in PubMed, MEDLINE, Cochrane Central Register of Controlled Trials, Scopus, and gray literature. Eight studies fulfilled the eligibility criteria. RESULTS: In all the studies, clinical severity was significantly reduced in within-group comparisons, whereas between-group comparisons showed nonsignificant results, except for total glucosides of paeony capsules. CONCLUSION: Efficacy of herbal therapy in OLP should be weighed against the high bias in the studies.

Assessment of oral health-related quality of life instruments for oral submucous fibrosis: A systematic review using the COnsensus-based Standards for the selection of health Measurement Instruments (COSMIN) checklist.

Quality of life (QoL) instruments are becoming increasingly important in research and often used in clinical practice. Various QoL instruments have been developed/ validated for assessment of oral health-related QoL (OHRQoL) in patients with oral submucous fibrosis (OSF). Selection of an adequate instrument is challenging in routine practice due to lack of information on psychometric quality of measurement instruments. This systematic review gives an extensive overview of quality of all the currently available measurement instruments for use in OSF patients. PubMed, Scopus, Web of Science and CINAHL were searched for relevant literature until December 2018 and the information was extracted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The quality of the identified studies was assessed per measurement property according to the COnsensus-based Standards for the selection of health Measurements Instruments (COSMIN) checklist. Four studies that met the inclusion criteria were included. Three generic and one disease-specific QoL instruments were identified. Information regarding important measurement properties was often incomplete. The evidence for the quality of measurement instruments was found to be variable, none of the instruments performed sufficient on all measurement properties. Based on the available information, OHRQoL-OSF appeared to have adequate COSMIN measurement properties. As recently published, newer QoL instruments have not yet been evaluated in an adequate manner for use in OSF. We suggest future studies should implement OSF-specific OHRQoL-OSF to better understand OSF patients' perspectives and help inform clinicians to propose treatment strategies as per patients' needs.

Comparison of generic and condition-specific oral health-related quality of life instruments in patients with oral submucous fibrosis.

PURPOSE: With the growing interest in health economics, there is a demand for best valid instrument to assess quality of life (QoL) in patients with oral submucous fibrosis (OSF). The objective of this study was to compare the convergent and discriminative properties of the condition-specific OHRQoL-OSF and generic OHIP-14 in OSF patients. METHODS: The OHRQoL-OSF and OHIP-14 instruments were administered concurrently to 300 clinically diagnosed OSF patients. Analysis of both the instruments was carried out by using descriptive statistics. The internal consistency and reproducibility of the instruments were assessed using Cronbach's alpha and intra-class correlation coefficient respectively. The convergent and discriminative validity were then determined and compared. RESULTS: The OHRQoL-OSF and OHIP-14 exhibited good psychometric properties for internal consistency (Cronbach's alpha > 0.88) and reproducibility (ICC > 0.85). A positive associations were observed between both the instruments, but a high prevalence of patients with no impact was noted using the OHIP-14. Both the instruments were able to discriminate between patients with different clinical stages of OSF, but OHRQoL-OSF was more responsive in the early stages of OSF. The OHRQoL-OSF demonstrated greater sensitivity for 'discomfort and functional impairment'. CONCLUSIONS: The condition-specific OHRQoL-OSF identified OSF patients with impaired oral health-related QoL more easily with greater discriminative properties. To better understand patient reported experiences in OSF, these results may guide the choice of instrument in future researches and surveys.

Impact of socioeconomic inequalities on quality of life in oral submucous fibrosis patients.

AIM: The purpose of this study was to analyze the impact of socioeconomic characteristics on oral health-related quality of life (OHRQoL) in patients with oral submucous fibrosis (OSF). METHODS:  A total of 300 OSF patients' quality of life (QoL) data were obtained using OHRQoL-OSF and global self ratings of oral health and overall wellbeing. The socioeconomic status (SES) of participants was analyzed by using modified Kuppuswamy scale. RESULTS: OSF patients with lower SES had worse OHRQoL as compared with those with middle and upper SES (p < 0.001). The household income of the family was found to be strongly associated with poor OHRQoL. CONCLUSION: Socioeconomic characteristics should be considered in the holistic management of OSF as it has been strongly associated with the OHRQoL of these patients.

Quality of life and oral potentially malignant disorders: Critical appraisal and prospects.

Quality of life (QoL) is a vital and often required health outcome measure that is relevant to patient care. A healthy oral cavity enables person to perform daily activities without any limitations. However, any disturbance may result in impaired QoL. The oral health-remains an essential element of people's health and well-being. In recent years, the tradition of clinical practice and research has been changed by incorporating QoL assessment, as it helps in assessment of patients' needs and monitoring treatment responses. Oral potentially malignant disorders (OPMDs) are a group of chronic disorders including oral leukoplakia (OL), oral lichen planus and oral submucous fibrosis (OSF). It is evident that patients with OPMDs experience significant health-related symptoms, functional limitations and psycho-social impairment, compromising their QoL. Moreover, the worsening of QoL has been associated with advanced stages of OPMDs. Despite of increasing number of OPMD cases in recent decades, limited literature is available regarding QoL in this population. Although, there is higher prevalence of habit-related OPMDs, particularly OSF and OL in Southern Asian countries, only a few studies have been performed in these populations. Moreover, these studies administered generic QoL instruments, which offer less sensitivity to clinical changes. However, condition-specific instruments are more sensitive and allows better measurement of QoL. As the impacts of different conditions on OHRQoL may vary, the development and validation of a QoL instrument specific to each clinical entity of OPMDs is currently needed.