[Recommendations for the healthcare of patients with FOP]. / Empfehlungen zur Versorgung von Patient:innen mit FOP.

BACKGROUND: Fibrodysplasia ossificans progressiva (FOP) is a very rare, severe genetic disorder triggered by a gain-of-function mutation in the ACVR1 gene that codes for the type I bone morphogenetic protein (BMP) receptor ACVR1 (activin A receptor-type 1), also known as ALK2 (activin receptor-like kinase-2). It leads to the onset and progression of heterotopic ossification (HO) in soft and connective tissue. HO is often preceded by episodes of soft tissue swelling or flare-ups. Flare-ups, characteristic of FOP, may be induced by trauma, infection, vaccination, or other medications, as well as surgical procedures or may occur spontaneously. As patients age, they develop severe mobility limitations due to progressive HO formation, including immobility, causing a shortened life expectancy. FOP's first characteristic clinical sign is the congenital malformation of one or both big toes with valgus axis deviation, which is present in almost all patients. To confirm the diagnosis, molecular genetic analysis of the ACVR1 gene is possible. AIM OF THE RECOMMENDATIONS: This white paper aims to provide an overview of the necessary prerequisites and conditions for the care of patients with FOP and positively contribute to patients with FOP by improving the overall availability of knowledge. To achieve this, relevant aspects of the care of the very rare disease FOP are presented, from the initial diagnosis to the care in regular care based on the authors' knowledge (German FOP network) and the international FOP Treatment Guidelines. The recommendations presented here are addressed to all actors and decision-makers in the health care system and are also intended to inform patients and the public.

Randomized trial of itraconazole oral solution for oropharyngeal candidiasis in HIV/AIDS patients.

PURPOSE: Oropharyngeal candidasis (thrush) is the most common opportunistic infection in individuals who are positive for the human immunodeficiency virus (HIV) and those who have progressed to AIDS. Itraconazole has a broad in vitro spectrum of activity, including a wide variety of Candida species. Our study determined the relative efficacy of a new oral solution formulation of itraconazole and fluconazole tablets in the treatment of oropharyngeal candidiasis. PATIENTS AND METHODS: This was a prospective randomized, third-party-blind, multicenter trial conducted at 12 centers in the United States. One hundred seventy-nine HIV-positive patients with mycologically documented oropharyngeal candidiasis were treated with itraconazole oral solution 200 mg/ day for 7 or 14 days, or fluconazole tablets 100 mg/day for 14 days. Severity of disease was scored clinically before treatment and at clinical evaluations on days 3, 7, 14, 21, 35, and 42. Semi-quantitative cultures of mouth washings were also obtained on these days. RESULTS: Both 14-day and 7-day regimens of itraconazole oral solution were equivalent to fluconazole for most efficacy parameters. The clinical response rate was 97% after 14 days of itraconazole and 87% after 14 days of fluconazole. Itraconazole oral solution given for 7 days was also equivalent to fluconazole treatment for 14 days. Approximately one half of patients in all three groups relapsed by 1 month after completion of treatment. There were few adverse reactions to either drug. CONCLUSION: Itraconazole oral solution is well tolerated and offers an alternative at least as effective as fluconazole in the treatment of oropharyngeal candidiasis.

Absence of HIV transmission from an infected dentist to his patients. An epidemiologic and DNA sequence analysis.

OBJECTIVE: To determine if a general dentist with human immunodeficiency virus (HIV) infection transmitted HIV to any of his patients. DESIGN: A cohort study in which all patients treated by a dentist who developed the acquired immunodeficiency syndrome (AIDS) were identified and attempts were made to contact all patients for HIV antibody testing. SETTING: A general dentistry clinic operated by the Department of Veterans Affairs in southeastern Florida. PARTICIPANTS: All patients treated by a dentist during the 5 3/4 years before he developed AIDS were identified in a computerized registry of dental care. MAIN OUTCOME MEASURES: Attempts were made to contact all living patients for counseling and HIV antibody testing. Living patients with newly identified HIV infection were interviewed, and DNA sequence analysis was performed to compare genetic relatedness of their HIV to that of the dentist. Death certificates were obtained for decreased patients, and the medical records of those with diagnoses suggestive of HIV disease or drug abuse and those dying under the age of 50 years were reviewed in detail. RESULTS: There were 1192 patients who had undergone 9267 procedures, of whom 124 were deceased. A review of the death certificates of the deceased patients identified five who had died with HIV infection, all of whom were either homosexuals or users of illicit intravenous drugs. We were able to locate 962 (92%) of the remaining 1048 patients, and 900 agreed to be tested. Infection with HIV was documented in five of the 900 patients, including four who had clear evidence of risk factors for acquiring HIV infection. One patient who had only a single evaluation by the dentist denied high-risk behavior. Comparative DNA sequence analysis demonstrated that the viruses from the dentist and these five patients were not closely related. CONCLUSION: This study indicates that the risk for transmission of HIV from a general dentist to his patients is minimal in a setting in which universal precautions are strictly observed. Programs to ensure compliance with universal precautions would appear preferable to programs for widespread testing of dentists.

Evaluation of cost-productivity of hospital-based general practice residency programs.

This study evaluated the 1985 cost-productivity of staff dentists and general practice residents at 11 dental clinics with general practice residency (GPR) programs and five nonteaching dental clinics in Veterans Administration hospitals. The results indicate that on the basis of dental service production, GPR facilities are or should be as cost-efficient as nonteaching facilities. It was also found that GP residents can repay a major part of their training costs.

Five year follow-up of a prospective cohort of juvenile chronic arthritis with recent onset.

One hundred and fifty children with unsuspected juvenile chronic arthritis (JCA) and a disease duration of 3-6 months entered a prospective study. Diagnosis of JCA could be verified in 107 patients according to the WHO-EULAR criteria. After 5.0 +/- 0.9 years 66 of the 107 patients showed no disease activity, 24 of them for more than 2 years, 23 for less than 2 years without any drug therapy. Nineteen patients were still on NSAIDs and/or long acting drugs, 14 patients with unfavourable morphologic outcome (greater than stage II) and 17 patients with severe functional impairment (greater than or equal to stage III) all belonged to the 41 children with still active disease after 5 years follow-up. The disease course was polyarticular in 10 of the 14 patients with severe radiologic changes and in 14 of the 17 with unfavourable functional results. The wrist joint proved especially vulnerable since 46% of the severe radiologic changes concerned this joint. All 4 children with positive rheumatoid factor followed a polyarticular course with severe radiologic changes (stage greater than II), 3 of them together with unfavourable functional outcome. Risk factors for morphologically and functionally unfavourable course are therefore seen in a still active disease after 5 years, a polyarticular joint involvement and a positive rheumatoid factor. Extra-articular complications concerned chronic iridocyclitis in 7 patients, acute iridocyclitis in 2, amyloidosis in 1 and growth retardation in 2 children. -The prospective study is to be continued.

Inhibition of caries in hamsters treated with staphylococcin 1580.

Littermate hamsters were infected orally with cariogenic Streptococcus mutans NS-50S and maintained on a high-sucrose diet to induce dental caries. Individual groups of animals were treated by single daily instillation of staphylococcin 1580 (100 micrograms) in the cheek pouches. Other groups were treated similarly with bacitracin (60 or 120 U) or saline. After 35 days on the caries test regimen, animals treated with staphylococcin 1580 had 49 or 60% less caries than the saline controls in two separate trials. Hamsters treated with 60 U of bacitracin had 40% less caries, while animals receiving 120 U of bacitracin had 58% less caries. Significant reductions in the oral and fecal levels of S. mutans NS-50S were also observed in the treated animals. In contrast to earlier, inconclusive experiments in which these agents were placed directly into the oral cavity, the inhibition of caries in this study is attributed to prolongation of the oral retention of these agents, with increased opportunity to interact with cariogenic microorganisms.

[Prospective observation study on the clinical course of chronic juvenile arthritis (JCA)]. / Prospektive Beobachtungsstudie zum klinischen Verlauf der juvenilen chronischen Arthritis (JCA).

A non-randomized follow-up study of 106 children with juvenile chronic arthritis was conducted for two years starting from the onset of the disease. All subgroups - with the exception of seropositive polyarthritis - showed a decrease in the number of joints affected, and activity of the disease in a number of patients. Instant remission and therapeutic effects cannot distinctly be differentiated. Radiological changes are proper criteria for early diagnosis in children with intense progressive joint-findings. X-ray examinations of the joints affected are therefore indicated at regular intervals especially, in the seropositive, occasionally in the seronegative polyarthritic subgroup.

Dental health status of an aging VA population: implications for a preventive dental health care program.

The purpose of this study was to describe the sociodemographic characteristics and dental health status of the largest eligible class of veterans (Class IV) within the Greater Miami catchment area. Only 43 percent of 4,882 Class IV patients within this area have sought VA dental care. Sociodemographic data were obtained from a randomized sample of both the VA-treated and non-VA-treated groups. The prevalence and incidence of dental diseases and actual dental treatment procedures completed for the VA-treated sample were determined from clinical records. Results indicated that periodontal disease and dental caries are substantial problems; small groups of patients account for the majority of treatment needs and associated costs; 71 percent of these patients are over 55 years of age; and the "average" Class IV patient is lower middle-class and retired. Study implications suggest that the best strategy for developing an effective VA dental health program would be one in which each patient would have a preventive regimen tailored to meet his needs and the ability to comply with the preventive program. The VA needs to determine the cost-effectiveness and dental health advantages of providing preventive dental health care services to eligible veterans.

Prevalence, distribution of serotypes, and cariogenic potential in hamsters of mutans streptococci from elderly individuals.

The prevalence of mutans streptococci (Streptococcus mutans, Streptococcus cricetus, Streptococcus sobrinus, and Streptococcus rattus) was determined in the salivas of 169 elderly individuals ranging in age from 60 to 87 years. Approximately 40% of these individuals were edentulous and wore full upper and lower dentures. With the exception of a higher proportion of saliva counts below 1,000 CFU/ml in the full-denture wearers, the prevalence and the serotype and species distributions of the mutans streptococci were similar in the denture wearers and individuals with natural teeth only. The species and serotype distributions of mutans streptococci in this elderly population were also consistent with reported observations of other workers on younger, more caries-prone populations. A total of 87 representative isolates of the mutans streptococci were tested for cariogenic potential in a hamster model system. A considerable degree of variation in virulence between different strains was observed. However, these differences were not relatable to individual species or serotypes or to whether the organisms were isolated from denture wearers or naturally dentate subjects. The results of our studies indicate that elderly individuals with either natural or artificial dentitions may be a hitherto unrecognized reservoir of mutans streptococci having varying degrees of potential cariogenicity. Hence, in close family situations they could serve, along with parents and siblings, as vectors in the initial transmission of cariogenic microorganisms to young children.

Oral Hygiene Skill Achievement Index II.

The utility of any index depends on its validity, reliability, and administrative feasibility. The Oral Hygiene Skill Achievement Index (S.A.I.) was found to be a valid measure of oral hygiene skill in that registered dental hygienists received greater than 90% of the total possible points. The S.A.I. was found to have an initial reliability of r greater than or equal to 0.91 and a repeat reliability of r greater than or equal to 0.96. The S.A.I. requires less than 10 minutes to administer, 40 minutes to teach, and 1 hour and 40 minutes to learn. The results suggest that S.A.I. should be a useful instrument for both clinical and research use.

Nutritional determinants of the ecology of the oral flora.

Consideration of nutritional factors as determinants of oral microbial ecology leads to the conclusion that endogenous factors play a dominant role. It is the interactions between specific microorganisms and nutritional and other factors provided by the host that selectively determine the kinds of microorganisms which willinitially reside in the oral cavity and the sites which they will colonize. The persistence of these original "colonists" in their respective ecologic niches will depend in part on the accessibility of endogenous nutrients variously provided by saliva, tissue exudates, crevicular fluids, or degenerating host cells. It will also depend on their response to and interaction with microorganisms which immigrate to that site subsequently. The microbial ecology of a given site is therefore rarely uncomplicated, the notable exception occurring at the most microscopic level where one may encounter microcolonies of individual species within more heterogenous populations of microorganisms. The extent of this heterogeneity quantitatively and qualitatively is a reflection of the degree to which the interactions between the resident flora and the new arrivals, plus the local environmental changes which they both generate, serve to promote or to discourage the survival and growth of the individual species. This element of heterogeneity in a dynamic system with its inherent potential for variation makes it possible for relatively minor changes in local environmental conditions to result in significant differences in the microflora between one site and another even though they may be in close proximity to each other. Once this concept is appreciated it becomes easy to understand how disease processes such as dental caries may affect specific areas of a tooth while nearby areas are unaffected. The effects of exogenous nutrients on the ecology of the oral microbiota, nevertheless cannot be ignored. The diet may modulate such endogenous factors as the salivary secretions and the local resistance of the gingival tissues to infections. Although at our present state of knowledge the direct influence of dietary proteins and fats on the oral microbiota is thought to be of relatively minor consequence, dietary carbohydrates are of major ecological significance. Dietary sugars provide readily available substrates for the oral microorganisms, most of which depend on carbohydrates for energy sources. The metabolism of dietary sucrose by S. sanguis and S. mutans with the productions of acids and intracellular and extracellular polysaccharides has specific influence on the microbial composition, metabolic activities, and mass of coronal plaque. The ready availability of dietary carbohydrates undoubtedly influences the microflora of other parts of the oral cavity as well, eic species or indirectly through the interactions of other organisms with them...