Management of Red Teeth in an International Patient with X-Linked Adrenoleukodystrophy.

Childhood cerebral X-linked adrenoleukodystrophy is a progressive, central nervous system, and endocrine disorder that typically leads to total neurologic disability and, eventually, death without appropriate, timely medical therapy. Hematopoietic stem cell transplantation has been found effective in slowing cerebral deterioration and improving long-term survival. The purpose of this case report was to describe the multidisciplinary management of red, discolored, pulpally treated primary molars identified in a nine-year-old Russian boy with childhood cerebral X-linked adrenoleukodystrophy preparing for myeloablative conditioning chemotherapy followed by hematopoietic stem cell transplantation.

Estrogen receptor-alpha polymorphism in patients with and without degenerative disease of the temporomandibular joint.

AIMS: The genetic aspect of degenerative joint disease (DJD) of the temporomandibular joint is poorly understood. The prevalence of the estrogen receptor alpha (ER-alpha) gene polymorphism in patients with and without DJD using xbal and pvull restriction fragment length polymorphisms (RFLPs) was studied. METHODOLOGY: DNA samples from 42 DJD⁺ and 36 DJD⁻ subjects were amplified. A 346-base pair long ER-alpha gene fragment containing the two sites of polymorphism in intron 1 was analyzed for xbal and pvull RFLP. Statistical analysis was carried out using Fisher's exact test and two-group t-test. RESULTS: Five different ER-alpha genotypes were found in both groups. These were PXPX, pxpx, pxPX, PxPX, and pxPx. DISCUSSION: There was a higher number of pxpx and pxPX genotypes in the DJD⁺ samples compared to the DJD⁻ group, which suggests the presence of polymorphism possibly modulates the ER-alpha activity in bone and contributes to the degenerative process in the joint.

A current perspective on Sjögren's syndrome.

Sjögren's syndrome is a common autoimmune disorder characterized by dry mouth and dry eyes. Symptoms and signs are chronic and can be severe. The diagnosis of Sjögren's syndrome can be confusing and time-consuming. The management can also be a significant challenge for the clinicians. However, recent genomic and proteomic developments are unlocking the mystery of the disease process as well as contributing to our ability to define, diagnose, and develop new treatment modalities for patients with this complex disorder.

Determining the state of knowledge for implementing the universal protocol recommendations: an integrative review of the literature.

The purpose of this study was to determine the current state of knowledge concerning the implementation of the Joint Commission's Universal Protocol. We conducted an integrative review of the literature through a systematic search of the National Library of Medicine (ie, PubMed) database to identify empirical and theoretical documents that discussed the implementation process for the Universal Protocol. The current state of knowledge varies from facility to facility, and we noted significant trends, gaps, and areas of concern in the implementation process. Successful implementation of the Universal Protocol has the following elements: a multidisciplinary team approach, active staff/patient participation, supportive hospital administration/leadership, and active communication that promotes a healthy work environment.

Reducing medical waste.

Medical waste is a necessary by-product of any hospital environment; however, the majority of regulated medical waste is produced in the OR from the use of disposable surgical supplies (eg, drapes, gowns, basins, gloves, sponges). We conducted a concept comparison project in the ORs of two large medical centers in Bethesda, Maryland, and Washington, DC, to evaluate the effects of using reusable surgical basins, gowns, and table and Mayo stand covers in place of disposable products. Survey results indicated that surgeons and surgical technologists found the reusable products to be preferable to the disposable products currently in use. In addition, using reusable products provided a means to decrease regulated medical waste generated in the OR by an average of 65% as well as reduce the cost of waste disposal. AORN recommends evaluating the environmental effects of using reusable, reposable, and disposable products; our findings provide evidence that may be useful to surgical facilities that seek to adopt a "green" approach.

Cleft lip and palate: association with other congenital malformations.

UNLABELLED: Orofacial clefts are frequently associated with other congenital malformations. Studies vary in incidence and types of anomalies. OBJECTIVE: To evaluate associated malformations in orofacial cleft patients at a major research hospital. STUDY DESIGN: Medical records of 1127 patients, in the Cleft Palate/Craniofacial Clinic, Boys Town National Research Hospital, from January 1980 through February 2000 were reviewed. Patients were divided into two categories: 1) cleft palate only (CP), and 2) cleft lip, with or without cleft palate (CL +/- P). Further categorization included location and type, if any, of other congenital malformations. RESULTS: 47.2% of patients had CP and 52.8% had CL +/- P. 32.2% of all cleft patients had associated congenital malformations. The orofacial region was the most common site, followed by cardiovascular central nervous, and skeletal systems. Congenital malformations were more common in CP (38.7%), than CL +/- P (26.4%). Of malformations diagnosed, 63.1% were chromosomal/syndromic anomalies while 36.9% were non-chromosomal/syndromic. CONCLUSIONS: Recognition of the spectrum of congenital malformations, associated with orofacial clefting, is essential for further diagnostic testing and in some cases genetic counseling.

A quantitative evaluation of inflammatory cells in human temporomandibular joint tissues from patients with and without implants.

PURPOSE: Previous studies made only qualitative assessments of immune cell responses to temporomandibular joint (TMJ) implant wear debris. The aim of this study was to perform a quantitative comparison of inflammatory cell types in TMJ tissues with implant wear debris, TMJ tissues with a history of disc pathology without implant debris, and TMJ tissues from normal control subjects. MATERIALS AND METHODS: TMJ tissues were collected from the following 3 groups of subjects: 1) individuals with failed TMJ implants (implant group, n = 10), 2) patients with TMJ disc pathology but no history of implant placement (nonimplant surgery group, n = 10), and normal cadaveric tissues with no history of surgery (control group, n = 10). Tissue sections (5 microm) from all subjects were stained with hematoxylin-eosin, after which cell counts were done for 2 types of inflammatory cells: multinucleated giant cells and lymphocytes. Mean inflammatory cell counts from the 3 groups were compared by use of a 1-way analysis of variance procedure and Bonferroni adjustment to maintain an overall type I error rate of .05. RESULTS: Implant group tissues contained significantly more inflammatory cells than tissues from the nonimplant surgery and control groups (P < .0001). Multinucleated giant cells were only present in implant group tissues. Although the high number of multinucleated giant cells present in the implant group obscured a total count of lymphocytes for that group, lymphocyte cell counts were still significantly greater (P < .005) in implant group tissues than in tissues from the other 2 groups. CONCLUSIONS: Our data provide quantitative confirmation that the presence of Proplast-Teflon implant (Vitek, Houston, TX) wear debris is associated with a significant increase in the number of local multinucleated giant cells and lymphocytes.

Prevalence, severity, and predictors of fatigue in subjects with primary Sjögren's syndrome.

OBJECTIVE: To investigate the relationship of fatigue severity to other clinical features in primary Sjögren's syndrome (SS) and to identify factors contributing to the physical and mental aspects of fatigue. METHODS: We identified 94 subjects who met the American-European Consensus Group criteria for the classification of primary SS. Fatigue was assessed with a visual analog scale, the Fatigue Severity Scale (FSS), and the Profile of Fatigue (ProF). Associations with fatigue were compared using multivariate regression. RESULTS: Abnormal fatigue, defined as an FSS score >or=4, was present in 67% of the subjects. Pain, helplessness, and depression were the strongest predictors of fatigue according to the FSS and the somatic fatigue domain of the ProF (ProF-S), both with and without adjustment for physiologic and serologic characteristics. Depression was associated with higher levels of fatigue; however, the majority of subjects with abnormal fatigue were not depressed. Anti-Ro/SSA-positive subjects were no more likely to report fatigue than seronegative subjects. The regression models explained 62% of the variance in FSS and 78% of the variance in ProF-S scores. Mental fatigue was correlated with depression and helplessness, but the model predicted only 54% of the variance in mental fatigue scores. CONCLUSION: Psychosocial variables are determinants of fatigue, but only partially account for it. Although fatigue is associated with depression, depression is not the primary cause of fatigue in primary SS. Investigation of the pathophysiologic correlates of physical and mental aspects of fatigue is needed to guide the development of more effective interventions.

Evaluation of surgically retrieved temporomandibular joint alloplastic implants: pilot study.

PURPOSE: The purpose of this study was to perform a retrieval analysis of temporomandibular joint (TMJ) alloplastic interpositional implants and test possible correlation between implant failure features and patient clinical outcomes. In addition, we investigated the implants' surface and examined the foreign body reaction associated with different types of alloplastic materials. MATERIALS AND METHODS: Twelve implants (Proplast/Teflon [Vitek, Houston, TX] and Silastic [Dow Corning, Midland, MI]) were surgically removed from the patients' TMJs. Implant surface failure features (fracture length, perforation of the implants) were observed using stereomicroscopy and recorded for description of the failure mechanisms and to statistically compare with clinical outcomes. Patients' clinical data (pain symptoms and mandibular function) were collected and examined. Clinical outcomes were obtained relative to symptom severity (Symptom Severity Index [SSI]) and jaw function (modified Mandibular Function Impairment Questionnaire [mMFIQ]). Peri-implant soft tissues and implants were analyzed with light microscopy and stereo zoom microscopy. Electron microprobe analysis of implant fragments and peri-implant tissues was performed. RESULTS: The statistical results showed that only the presence of implant perforation was statistically associated with the SSI, specifically with the pain tolerability dimension. No statistical association was seen between any of the other implant failure predictors and the SSI and between the predictors and the mMFIQ. Stereo zoom microscopy suggested that Proplast/Teflon implants (n = 7) were susceptible to perforation, layer tearing, fracture and fiber extrusion. The Silastic implants (n = 3) revealed a possible center perforation with fracture lines towards the periphery and fiber extrusion. Teflon implant wear debris particles appear to trigger a multinucleated giant cell foreign body reaction. CONCLUSION: Facial pain was a significant correlate to perforation and breakdown of the alloplastic TMJ interpositional implants, and most likely was the reason for implant removal.

Oral manifestations of a possible new periodic fever syndrome.

Periodic fever syndrome is composed of a group of disorders that present with recurrent predictable episodes of fever, which may be accompanied by: (1) lymphadenopathy; (2) malaise; (3) gastrointestinal disturbances; (4) arthrolgia; (5) stomatitis; and (6) skin lesions. These signs and symptoms occur in distinct intervals every 4 to 6 weeks and resolve without any residual effect, and the patient remains healthy between attacks. The evaluation must exclude: (1) infections; (2) neoplasms; and (3) autoimmune conditions. The purpose of this paper is to report the case of a 41/2- year-old white female who presented with a history of periodic fevers accompanied by: (1) joint pain; (2) skin lesions; (3) rhinitis; (4) vomiting; (5) diarrhea; and (6) an unusual asymptomatic, marked, fiery red glossitis with features evolving to resemble geographic tongue and then resolving completely between episodes. This may represent the first known reported case in the literature of a periodic fever syndrome presenting with such unusual recurring oral findings.

Development of a National TMJ Implant Registry and Repository-- NIDCR's TIRR.

PURPOSE: To provide a national systematic program to collect removed TMJ implants and biological tissues, make them available to researchers, and study them in conjunction with long-term clinical follow-up, with the ultimate goal of stimulating research toward understanding the safety and outcomes of TMJ implants. PROCESS: Two synchronized divisions: The Registry, recruiting clinicians and/or surgeons and patients, and collecting comprehensive clinical patient data over time; the Repository, procuring and archiving high quality, well characterized biological specimens and retrieved implants for dissemination. PARTICIPATION: Clinicians, surgeons, patients, researchers, pilot studies. PROGRESS: October 2002-present: Recruited 34 TMJ surgeons, 34 TMJ clinicians; registered 723 surgical/non-surgical TMJD patients and control subjects; collected 542 specimens, blood, saliva; initiated 19 projects using TIRR resources; produced 60 publications and presentations nationally and internationally; sent ten grants into review, with more being developed. PROSPECTS/PROJECTIONS: Continuing collection of long-term data for the development of new and improved implant designs and materials and investigation into understanding pathological mechanisms of TMJD.

Differences in initial symptom scores between myogenous TMD patients with high and low temporomandibular opening index.

The temporomandibular opening index (TOI) is a more useful measure of mandibular movement than linear mouth opening, since it is independent of age, gender, ramus length, and gonial angle. It is also useful when categorizing temporomandibular disorder (TMD) patients into diagnostic groups. Two subgroups of myogenous patients have been identified, one with a high and one with a low temporomandibular opening index. This study examined initial symptom severity in these two subgroups. Thirty-three (33) patients with a myogenous temporomandibular disorder were recruited. Twenty-six (26) were female and seven male. Eleven were found to be in the high temporomandibular opening index group and the remaining 22 in the low group. Symptom severity scores were determined prior to the start of treatment. Pain, joint sounds, headache, and neck pain were all rated by patients on a four-point verbal response scale. These symptom scores were compared between the two subgroups using the Wilcoxon two sample test. There appeared to be a significant difference between the two groups (p = 0.0025). TMD patients with high temporomandibular opening index appeared to have more severe signs and symptoms of TMD than patients with a low index.

The feasibility of monitoring NF-kappaB associated cytokines: TNF-alpha, IL-1alpha, IL-6, and IL-8 in whole saliva for the malignant transformation of oral lichen planus.

Previous investigations have demonstrated that immune activation and chronic inflammation may be one of the causes of oncogenesis. A previous study from our lab has shown significant increases of NF-kappaB dependent cytokines, TNF-alpha, IL-1alpha, IL-6, and IL-8 in different oral fluids from oral lichen planus (OLP) patients. The aim of this analysis was to explore the potential of detecting these cytokines in whole unstimulated saliva (WUS) in monitoring the malignant transformation of OLP. Thirteen patients with OLP (with epithelial dysplasia), 13 cases with oral squamous cell carcinoma (OSCC), and 13 age-sex matched controls were enrolled in the study. The WUS samples were collected and the level of TNF-alpha, IL-1alpha, IL-6, and IL-8 in WUS was determined by ELISA. In moderate and severe dysplasia, the level of each cytokine was significantly higher than in control. In moderate dysplasia, TNF-alpha and IL-1alpha were significantly increased at a level without difference from OSCC, but IL-6 and IL-8 was detected at a concentration significantly lower than OSCC. In severe dysplasia, the level of TNF-alpha was also not significantly different from that of OSCC, and the level of IL-1alpha, IL-6, and IL-8 was still significantly lower than that of OSCC. The level of four cytokines between smokers and non-smokers in each group did not show a significant difference. These results indicate that the change of NF-kappaB dependent cytokines in WUS may in part reflect the malignant transformation of OLP and the analysis of these cytokines and may provide a useful, non-invasive surrogate endpoint for monitoring malignant transformation as well as the therapeutic response of OLP. This is the first in vivo study utilizing saliva to confirm preclinical data that NF-kappaB is upregulated in oral carcinogenesis.

NF-kappaB dependent cytokine levels in saliva of patients with oral preneoplastic lesions and oral squamous cell carcinoma.

Previous investigations in our laboratory and others (Chen et al., 1998) have shown that the levels of certain inflammatory, proangiogenic cytokines in saliva and tissue specimens of patients with oral premalignant lesions (OPML) are elevated. We have also shown that these cytokines are elevated in tissue culture of oral squamous cell carcinoma (OSCC) cell lines. The purpose of this pilot study was to determine the level of several inflammatory, NF-kappaB-dependent cytokines in whole unstimulated saliva (WUS), in subjects with OPML as compared to those with diagnosed OSCC. Subjects (n=13) with OMPL, OSCC (n=13), and age-sex matched controls without oral lesions (C) (n=13) were enrolled. The mean age was 58.7 years. WUS was collected by standard techniques for 5 min (Navazesh, 1993). WUS samples were centrifuged and the cytokine analysis was performed on the supernatants by ELISA as previously described by Ondrey et al. (1991). The cytokines analyzed were: TNF-alpha, interleukin-1, interleukin-6, and interleukin-8 (TNF-alpha, IL-1, IL-6, and IL-8). The results as analyzed by Pairwise t-tests revealed significant differences in the salivary levels of: (1) TNF-alpha: (mean+/-S.E.M.: TNF-alpha-OSSC=28.9+/-14.6* pcg/ml versus OPML=10.5+/-7.4* pcg/ml versus controls=3.0+/-1.0 pcg/ml; *p<0.01); (2) IL-1: (IL-1-OSSC=454.4+/-215.8* pcg/ml versus OPML=255.1+/-124.8* pcg/ml versus controls=173.2+/-66.9 pcg/ml; *p<0.01); (3) IL-6: (mean+/-S.E.M.: IL-6-OSSC=88.2+/-43.2* pcg/ml versus OPML=70.8+/-24.3* pcg/ml versus controls=1.4+/-1.0 pcg/ml; *p<0.001) and (4) IL-8 in saliva: (mean+/-S.E.M.: IL-8-OSSC=3154.1+/-1023.2* pcg/ml versus OPML=1918.2+/-899.1* pcg/ml versus controls=1580.7+/-789.0 pcg/ml; *p<0.001). There was a significant increase in the levels of all cytokines in the saliva of the OPML as compared to controls, and a significant difference in the cytokines of OSSC saliva compared to the OPML and controls. These results suggest that these proangiogenic, proinflammatory cytokines are elevated in the saliva of patients with OSSC and OPML as compared to controls, which may have diagnostic and/or prognostic significance.

A comparison of the pro-inflammatory, NF-kappaB-dependent cytokines: TNF-alpha, IL-1-alpha, IL-6, and IL-8 in different oral fluids from oral lichen planus patients.

To explore the feasibility of detection of the level of NF-kappaB-dependent cytokines in oral fluids from patient with oral lichen planus (OLP) for clinical application, 13 OLP subjects were enrolled in the study as were 13 age-sex-matched controls. In each subject, the whole unstimulated saliva (WUS), mixture of saliva and isotonic saline oral rinse (Saliva-NaCl), and lesion tissue transudates (TT) were collected by standard techniques. The level of cytokines, TNF-alpha, IL-1-alpha, IL-6, and IL-8 in three types of oral fluids was determined by ELISA. In the three types of oral fluids, a significantly higher level of these cytokines was detected in OLP patients than in normal controls. These results indicate that NF-kappaB-dependent inflammatory cytokines may be detected at increased levels in certain oral fluids which may have diagnostic and prognostic potential for monitoring disease activity and making therapeutic decisions in patients with OLP.

Diagnosis and management of oral lichen planus.

Oral lichen planus is a complex and poorly understood clinical condition which cannot be cured. A definitive diagnosis and careful, conscientious follow-up are imperative. Symptoms and complications are common and challenging but may be managed with a variety of therapies including orally administered and systemic medications as well as lifestyle alterations and reduction of precipitating factors.