Oral complications in type 1B glycogen storage disease

A case of type 1b glycogen storage disease (GSD) in which neutropenia and neutrophil dysfunction were prominent features is described. Oral complications included rapidly progressive periodontal disease and recurrent mucosal ulceration.     

Unusual oral manifestations and evolution in glycogen storage disease type Ib

Glycogen storage disease type Ib is a rare inherited metabolic disorder that is caused by a deficiency of glucose-6-phosphate translocase with consequent accumulation of glycogen. The purpose of this study is to report a case affected by glycogen storage disease type Ib in which unusual oral findings were evident and to review the pertinent literature. The disease presents with failure to thrive, hepatomegaly, hypoglycemia, hyperlacticacidemia, neutropenia, and neutrophilic dysfunction causing increased susceptibility to recurrent infections. Common intraoral manifestations are dental caries, gingivitis, periodontal disease, delayed dental maturation and eruption, oral bleeding diathesis, and oral ulcers. Conversely, unusual oral lesions were observed in this case as hyperplastic-hypertrophic gingiva and giant cell granulomatous epulis. The treatment with granulocyte colony-stimulating factor markedly increased the neutrophil counts and reduced the frequency of infections and inflammations. Proper evaluation of the patient's oral condition, a program of preventive measures, and suitable medical consultation are important to minimize and avoid long-term complications.     

Oral ulceration in HIV infection: Investigation and pathogenesis

Oral ulcerations associated with HIV infection include recurrent aphthous ulcers (RAU). Whereas RAU prevalence is not increased, lesion severity is: among a group of HIV⁺ patients, 66% had the more severe herpetiform or major RAU. This increased severity suggests that HIV disease-related changes in the immune system may exacerbate RAU. In the peripheral blood of healthy subjects with RAU, CD4:CD8 cell ratios may be reversed and the proportion of T cell receptor-γδ⁺ cells increased. HIV disease-related immune system changes are characterized by reversed CD4:CD8, lowered CD4 cell counts and an inverse correlation between CD4 cell counts and per cent activated γδ lymphocytes. Adhesion molecules and cytokines involved in lymphocyte homing may be important in RAU pathogenesis: ICAM-I and ELAM are strongly expressed, and TNFα production is increased in peripheral blood lymphocytes of healthy patients with RAU. In patients with active HIV disease/AIDS, serum TNFα levels are increased. Thalidomide, which inhibits TNFα production, is effective treatment for RAU. Some RAU patients have vitamin B12 or folate deficiencies, levels of which are commonly low in HIV⁺/AIDS patients. However, in a case control study of HIV⁺ patients, vitamin B12- or folate-deficiencies were not found to be significant risk factors for RAU.     

Recurrent aphthous stomatitis. A study of the clinical characteristics of lesions in 93 cases

We studied the clinical characteristics of 93 patients with recurrent aphthous stomatitis (RAS); 66 corresponded to minor aphthae (MiRAS), 20 to major aphthae (MaRAS), and 7 to herpes-like ulcerations (HU). MiRAS was seen to present the lowest rate of recurrences, as well as the shortest duration and the fewest lesions per episode. In turn, MaRAS presented the longest duration per recurrence, along with a number of lesions and recurrences that was lower than in HU but greater than in cases of MiRAS. Finally, HU developed the greatest number of lesions and recurrences. When we classified RAS according to the rate of episodes, no statistically significant differences were observed among the three types in terms of patient age, number and duration of lesions, or evolution time of the oral disorder.     

Gingival and other oral manifestations in measles virus infection

BACKGROUND: Measles is a highly contagious, viral infectious disease affecting mainly children and young adults. It is characterized by high fever, maculopapular rash, keratoconjunctivitis and pathognomonic oral Koplik's spots. METHODS: During an outbreak of measles among soldiers in the Israeli military, patients were referred to one medical center where they were also examined for oral signs and symptoms of their illness. RESULTS: We present a case of measles infection with distinct oral findings. These included Koplik's spots, various forms of ulcerations of the free mucosa, -like gingivitis and pericoronitis. All oral lesions resolved after cessation of systemic illness. CONCLUSIONS: The measles virus is associated with a transient inhibition of the host response. Immunosuppression may create the necessary condition for the proliferation of pathobacteria associated with the observed pericoronitis and the other gingival lesions.     

Blood Profile and Histology in Oral Infections Associated with Diabetes

Introduction

We aimed to investigate the relationship between blood profile and histologic findings in both apical periodontitis (AP) and periodontal disease (PD) associated with diabetes.

Methods

Wistar rats (N = 80) were assigned to the following 8 groups: control, AP, PD, AP associated with PD, diabetes, diabetes with AP, diabetes with PD, and diabetes with AP and PD. Diabetes mellitus (DM) was induced with streptozotocin, AP was induced by exposure to the oral environment, and PD was induced using periodontal ligature. After 30 days, blood samples were collected, and the rats were euthanized. Subsequently, the maxillae were processed for light microscopy. Hematologic examinations were conducted to determine the total number of erythrocytes and leukocytes, erythrocyte constant, and blood glucose level. One-way analysis of variance and Kruskal-Wallis tests were used for statistical analysis, and the significance was set at P < .05.

Results

A significant correlation was found between the histologic findings and blood parameters.

Conclusions

In conclusion, diabetes accelerated the development and progression of AP and PD in the rats and caused an increase in the average erythrocyte volume as well as the leukocyte and neutrophil counts. Oral infections increase the total number of leukocytes, the number of neutrophils and lymphocytes, and blood glucose concentrations in DM rats.     

The close association between dental and periodontal treatments and oral ulcer course in behcet's disease: a prospective clinical study

Objective:  The aim of the study was to evaluate the influence of dental and periodontal treatments to the course of oral ulcers in patients with Behcet's disease (BD).
Methods:  Fifty-eight consecutive BD patients with oral ulcers were studied. Twenty-nine patients were in the intervention group (F/M: 15/14, mean age: 39.6 ± 6.9 years) and 29 (F/M: 15/14, 39.4 ± 10.6 years) were followed with a conventional treatment approach. In addition to oral hygiene education, dental and periodontal treatments were carried out in the intervention group, whereas the control group was only given oral hygiene education. Patients were evaluated in the pre-treatment observation period (1 month), treatment period (1 month) and 6 months after treatment.
Results:  An increase in the number of new oral ulcers (4.1 ± 3.5) was observed within 2 days during the treatment compared with 3–30 days during treatment month (2.3 ± 1.2) ( P  = 0.002). However, 6 months after the treatment, the number of oral ulcers (1.9 ± 1.5) was significantly lower compared with the pre-treatment observation (4.8 ± 3.2) ( P  = 0.000) and treatment periods (6.4 ± 2.3) in the intervention group ( P  = 0.05), whereas a similar oral ulcer presence was observed in the control group (2.8 ± 2.4, 3.7 ± 2.3 and 4.8 ± 4.3, respectively) ( P  > 0.05). Dental and periodontal indices were also better in the intervention group during the 6-month follow-up.
Conclusion:  Our results suggest that, in BD patients, dental and periodontal therapies could be associated with a flare-up of oral ulcers in the short term, but may decrease their number in longer follow-up. They also lead to a better oral health.     

Oral manifestations in HIV-positive adults from Northern Thailand

Eighty-seven HIV-infected patients in a provincial hospital in Northern Thailand were examined for oral manifestations of HIV disease and AIDS. The median age was 31.3 years. Seventy-four of the patients were women, 13 were men. 96.6% had a history of heterosexual transmission. Sixty-one patients were CDC-category A, 20 were category B and 6 were category C (AIDS). Thirty-eight percent of the patients revealed oral lesions; 23% had one oral lesion and 13.8% had two oral lesions. Common lesions were oral candidiasis (10.3% pseudomembranous candidiasis, 6.9% erythematous candidiasis and 3.4% both forms), oral hairy leukoplakia (11.5%) and exfoliative cheilitis (6.9%). Gingival linear erythema was seen in 8% of the patients; periodontal lesions and necrotising ulcerative gingivitis were not observed. Men were more commonly affected by oral manifestations than women (P < 0.004). The spectrum of oral lesions is comparable to other studies from the region, although most of these reported more men than women. Also, the degree of immunosuppression was more marked (AIDS).     

Microbiology of destructive periodontal disease in adolescent patients with congenital neutropenia. A report of 3 cases

BACKGROUND, AIMS: Congenital neutropenia is one condition that may predispose for destructive periodontal disease at a young age. In this report, we describe the microbiology of 3 adolescent patients with congenital neutropenia two of whom suffered from severe periodontitis. METHOD: Microbiological testing of the parents was also performed in 1 case. DNA fingerprinting was used to study transmission of putative periodontal pathogens in this case. From 1 patient with periodontitis, Actinobacillus actinomycetemcomitans and Porphyromonas gingivalis were isolated; a 2nd periodontitis patient was infected with P. gingivalis. A 3rd patient had gingivitis only and no A. actinomycetemcomitans or P. gingivalis were found. RESULTS: Using the amplified fragment length polymorphism DNA fingerprinting technique, bacterial transmission between the father and a patient was shown for A. actinomycetemcomitans but not for P. gingivalis.     

Oral manifestations in patients with gastro-oesophageal reflux disease: a single-center case–control study

Objective:  To assess the occurrence of oral pathological changes and symptoms in patients affected by gastro-oesophageal reflux disease (GERD).
Patients and methods:  200 patients with GERD and 100 matched healthy controls were studied. Thorough visual examination of the dental and oral mucosal tissues was performed and medical history relevant to oral symptoms was collected. The primary outcome was defined as a statistically significant difference, between the study group and controls, in the presence of the following indicators: soft/hard palate and uvula erythema, tooth wear, xerostomia, oral acid/burning sensation, subjective halitosis and dental sensitivity. Statistical analysis included chi-squared test, and crude odds ratio with 95% CI.
Results:  Univariate analysis showed that xerostomia, oral acid/burning sensation, subjective halitosis, and soft and hard palate mucosa and uvula erythema were more common in patients with GERD than matched controls ( P  < 0.05).
Conclusions:  This study failed to find any significant association between GERD and dental erosions, whereas some symptoms and other objective oral mucosal changes were found to be significantly associated with GERD.     

Oral management of the diabetic patient

There is good evidence to support the claim that certain oral diseases may be more prevalent among diabetic patients than non‐diabetic people. Similarly, studies have shown that management of certain oral conditions, especially periodontitis, can impact glycemic control in people with diabetes mellitus. Given that more than a quarter of a million people worldwide are affected by either type 1 or type 2 diabetes, all dentists will encounter patients with diabetes. Dental practitioners must be aware of the implications of this relationship and manage their patients' oral health care accordingly. This paper outlines the oral complications of diabetes mellitus and considerations for treatment of diabetic patients.     

Serum interleukin-8 level is a more sensitive marker than serum interleukin-6 level in monitoring the disease activity of recurrent aphthous ulcerations

BACKGROUND: Recurrent aphthous ulcerations (RAU) are common oral inflammatory lesions. Interleukin (IL)-8 is a pro-inflammatory cytokine of host response to injury and inflammation. Our recent study has found that measurement of serum IL-6 level can detect only 24% RAU patients with an abnormal serum level. In this study, we examined both the serum IL-6 and IL-8 levels in a group of RAU patients. The abilities of IL-6 and IL-8 to detect patients with an abnormal serum level were compared in order to find out whether IL-8 was a more sensitive serum marker than IL-6 in monitoring the disease activity of RAU. METHODS: In this study, we used a solid-phase, two-site sequential chemiluminescent immunometric assay to determine the baseline serum levels of IL-6 and IL-8 in 146 patients with RAU, 9 patients with traumatic ulcers (TU), and 54 normal control (NC) subjects. Eighty-two RAU patients, with the serum IL-6 or IL-8 levels higher than the upper limit of normal serum concentration, were treated with levamisole for 0.5-3.5 months, and their serum IL-6 and IL-8 levels were measured after treatment. RESULTS: We found that 25% (37/146) RAU patients, as well as 33% (20/61) major-type, 19% (13/69) minor-type, and 25% (4/16) herpetiform-type RAU patients, had a serum level of IL-6 greater than the upper normal limit of 4.7 pg/ml. In contrast, 60% (87/146) RAU patients, as well as 59% (36/61) major-type, 59% (41/69) minor-type, and 63% (10/16) herpetiform-type RAU patients, had a serum level of IL-8 greater than the upper normal limit of 8.7 pg/ml. In 82 RAU patients with the serum IL-6 or IL-8 levels higher than the upper limit of normal serum concentration, treatment with levamisole for a period of 0.5-3.5 months could significantly reduce the serum IL-6 level from 12.0 +/- 1.6 to 3.0 +/- 0.5 pg/ml (P < 0.001), and could significantly lower the serum IL-8 level from 70.9 +/- 11.2 to 13.8 +/- 3.1 pg/ml (P < 0.001). CONCLUSIONS: Because measurement of serum IL-8 level can detect 60% RAU patients with an abnormal serum level, while measurement of serum IL-6 level can detect only 25% RAU patients with an abnormal serum level, we conclude that serum IL-8 level is a more sensitive marker than serum IL-6 level in monitoring the disease activity of RAU. Levamisole can modulate both the serum IL-6 and IL-8 levels in RAU patients. IL-8, like IL-6, is also a useful serum marker in evaluating therapeutic effects of levamisole on RAU patients.     

Oral manifestations in selective IgA deficiency

Abstract: Selective immunoglobulin A (IgA) deficiency is the most common of the primary immunodeficiencies with a frequency of 1/300–1/3000, depending on the screened population. As secretory IgA (SIgA) has a protective role in mucosal surfaces from invasion of microorganisms, it is thought that IgA-deficient subjects are susceptible to periodontal diseases and oral manifestations. Previous studies show contradictory results, concerning the involvement of the individuals' periodontium with IgA deficiency. The aim of this study was to investigate and compare the oral manifestations in IgA-deficient subjects with controls. Eleven selective IgA-deficient subjects aged 3–18 years with serum IgA levels <10 mg dl⁻¹ and 11 age–sex-matched healthy children as the controls entered the study. Oral mucosal investigation, dental caries, plaque accumulation and periodontal status were assessed. Serum immunoglobulin levels were measured by single radial immunodiffusion (SRID) method. Saliva immunoglobulins and secretory component levels were measured by enzyme linked immunosorbent assay (ELISA) methods. IgA-deficient patients had serum and saliva IgA levels less than 10 mg dl⁻¹ and 10 µg ml⁻¹, respectively, but other serum immunoglobulin levels were normal and saliva immunoglobulin M (IgM) levels were increased, compared with controls. There were no significant differences in oral manifestations between IgA-deficient subjects and controls, which may be a result of compensatory increase of saliva IgM or other non-immunological defence factors in saliva. Thus, it is not necessary to evaluate IgA and SIgA in all the patients with oral and dental lesions and it is thought that it is better to investigate other factors.     

Oral infections related to radiation and immunosuppressive therapy

Abstract. The frequency of oral infection complications after cytotoxic chemotherapy andradiochemotherapy is significant, often contributing to serious septic infectious complications. Generally, the more intense the cytotoxic therapy, the more common are the oral infection complications. In the present review, the spectrum of oral infections related to radiation and immunosuppressive therapy are evaluated and the different local and systemic treatment modalities are presented.     

Dental findings in a child with glycogen storage disease type IA.

Glycogen storage disease type I, also known von Gierke's disease, is a rare, severe autosomal recessive disorder due to a defect in liver, kidney, and intestinal mucosa. The existence of delayed development of the dentition, increased incidence of dental caries, taurodontism, and prolonged bleeding following dental procedures should lead clinicians to consider type I glycogen storage disease. A 10-year-old boy with glycogen storage disease type I whose condition was first diagnosed when he was 4 years of age, was referred to the clinic for multiple caries and evaluation of delayed tooth eruption. On physical examination, the patient was cooperative, with short stature, protuberant abdomen, and growth retardation. Laboratory findings indicated that blood levels of pyruvate, triglycerate, uric acid, and cholesterol were elevated. Intraorally delayed mixed dentition was evident, and approximal caries were found in teeth 55, 54, 52, 51, 61, 62, 65, 74, 84, and 85. The most significant radiographic finding was consistent with taurodontism of the molar teeth. Lateral and posteroanterior cephalometric films showed that dimensions of the craniofacial complex were strongly reduced. Evaluation of the patient's dental age was approximately 6 years.     

Oral manifestations in 45 HIV-positive children from Northern Thailand

Forty-five Northern Thai children with HIV infection or AIDS were examined for oral manifestations. Of these children, 51.1% (n=23) were asymptomatic (category N), 48.9% were mildly, moderately or severely symptomatic (category A, B, C) and 48.9% (n=22) revealed oral lesions. Eleven patients (24.4%) showed one oral lesion, eight (17.8%) had two and three (6.6%) had three oral lesions. Erythematous candidiasis was the most common lesion (17.8%). Oral hairy leukoplakia was seen in 6.7% (n=3). Geographic tongue, not usually considered to be associated with HIV infection, was seen in 6.7% (n=3). Only 15 patients (33.3%) received antiretroviral therapy (ART). Comparison of patients with or without ART did not show differences in the prevalence of oral lesions. More studies in Thai HIV-infected children are needed to reveal the prevalence of oral manifestations, as well as for the predictive value of the most common or specific oral manifestations.     

Oral manifestations of HIV infection in 75 Dutch patients

Seventy-five consecutive HIV-infected patients, including 33 AIDS cases were examined. One or more oral mucosal lesions were observed in 57 (76%); candidiasis was the most common finding (52%). Others included hairy leukoplakia (16%), periodontal disease (16%) and Kaposi's sarcoma (4%). Diversity of study design and methods allowed no reliable comparison with other reports.