Dorota Olczak-Kowalczyk

Non‐Hodgkin lymphoma (NHL) in children with Nijmegen Breakage syndrome (NBS)

Due to small number of patients with Nijmegen Breakage Syndrome (NBS) and Non‐Hodgkin lymphoma (NHL) experience in their treatment is limited.

Oral mucosa in children with Prader–Willi syndrome

UNLABELLED Prader-Willi syndrome is a genetic disorder. Abnormal saliva secretion, emotional and behaviour problems, may affect the health status of the oral mucousa. OBJECTIVES To assess the impact of self-destructive behaviour and abnormal saliva secretion on the oral mucosa in children with Prader-Willi syndrome (PWS). MATERIALS AND METHODS Fifteen PWSs children (mean age 9.8 ± 4.4 years) and 15 healthy children (mean age 11.5 ± 3.5 years) were assessed for self-destructive behaviours, such as picking at the skin, physical and chemical saliva characteristics, mycology, and the clinical status of the oral mucosa. RESULTS Picking at the skin was only in children with PWS (n = 12). In contrast to the control group, the moistening rate of the lower lip mucosa was slower, and the mean pH of the resting saliva was reduced in the affected subjects. Sticky frothy or frothy saliva, decreased secretion rate of the stimulated saliva, and a reduced buffer capacity were more frequently in PWSs children; Candida spp. and oral candidiasis were also more common. Injurious lesions in the oral mucosa were found in one control child, and in eight PWSs subjects. In affected children, the lesions were concurrent with picking at the skin. A statistical correlation was noted between the presence of Candida spp. and oral candidiasis, and unfavourable saliva properties, and between injurious lesions and a slow moistening rate of the lower lip mucosa, and oral candidiasis. CONCLUSIONS Abnormal saliva secretion and self-destructive behaviours in children with Prader-Willi syndrome predispose them to injurious lesions in the oral mucosa, and possibly, to oral candidiosis.

Fluorescence methods (VistaCam iX proof and DIAGNODent pen) for the detection of occlusal carious lesions in teeth recovered from archaeological context.

Diagnosis of occlusal enamel caries in archaeologically derived collections remains a controversial problem because the accumulation of contaminants in fissures can interfere with diagnosis. Certain novel light-induced fluorescence methods, such as the DIAGNODent pen 2190 (DD) and VistaCam iX Proof (VC), have been used to detect dental caries in clinical settings. In this study, the abilities of DD and VC to detect initial enamel caries in archaeologically derived material is determined and compared with those of other methods (visual inspection, X-ray, histology, and micro-CT). Dental material encompassing the remains of 58 individuals, including a total of 380 teeth from each of three historical periods: modern Islamic (AD 1850-1950), Islamic (AD 600-1200) and late Roman (AD 200-400), obtained from two archaeological sites (Terqa and Tell Masaikh) located in the Middle Euphrates valley (Syria), were analyzed. VC was found to have excellent sensitivity (98), while DD obtained lower sensitivity (76) in detecting dental caries in its early stages. The results obtained by VC and micro-CT, considered the most reliable imaging technique, were not statistically significant (P = 0.3068). By contrast, results obtained by DD and micro-CT results, and DD and VC results were statistically significant (P < 0.0001, P = 0.0015, respectively). However the presence of dirt, stain, calculus, and plaque in the pits and fissures of the occlusal surface compromise correct diagnosis of caries by VC and DD. Consequently, for teeth recovered from archaeological contexts where staining, calculus and plaque are present, the best solution remains micro-CT.

Brief communication: A pilot study: Smooth surface early caries (caries incipiens) detection with kavo diagnodent in historical material

In many odontological studies concerning archeological material, there is no analysis of early caries lesions (caries incipiens) that manifest as a carious spot. At this stage of caries, the enamel is still hard, and thus, it is impossible to diagnose caries by visual methods. We assessed the usefulness of the DIAGNODent pen (DD laser) in analyzing noncavity lesions on the smooth surface sites of crowns from historical populations. Twenty-seven individuals were examined: 18 from Radom (Poland), and nine from Tell Masaikh and Terqa (Syria). A total of 562 teeth were characterized. The series represented different climatic zones, but were dated from the similar period, 18th to 19th century AD. We used four diagnostic techniques: visual, DD laser, radiographic, and histological as the gold standard. DD laser showed that the mean values of healthy enamel in both series did not exceed 15 units. The mean values of smooth and rough spots in the Syrian population were significantly higher than those from Poland. This study showed that all the noncarious spots from the Radom series did not exceed 30 units. In the Syrian samples, this limit was higher at 44 units. These results were confirmed by histology and radiography. The DD laser provided good results in detecting dentine carious lesions in historical material, but its efficiency in diagnosing early caries (caries incipiens) remains uncertain based on the presented series.

The status of dental and jaw bones in children and adolescents after kidney and liver transplantation.

BACKGROUND Systemic complications in patients after renal or liver transplantation may be localized in the oral cavity. Calcium-phosphate disturbances may affect the structure and metabolism of mandible bones, promote calcification of dental pulp, and in children may cause developmental defects of teeth. The aim of this study was to evaluate the incidence of dental and bone abnormalities in children and adolescents after kidney and liver transplantation with respect to the type of the transplanted organ and maintenance immunosuppression. MATERIAL/METHODS Overall, 23 kidney and 25 liver recipients (mean age: 13.95±4.2 yrs) were evaluated. Twenty patients received ciclosporin A (CsA) and 28 tacrolimus (TAC). Twenty-one kidney and 14 liver recipients were treated with steroids. Mean time after transplantation was 3.62±2.98 years. RESULTS The severity of caries and percentage of odontogenic abnormalities (76.0% vs. 60.86%) was higher in liver transplant recipients. Positive correlations were found between discoloration of the deciduous teeth and liver transplantation, between enamel hypoplasia and kidney transplantation, and between treatment with CsA and its dose and blood concentration (p<0.05). Pulp stones were present in 13.04% of kidney vs. 8.0% in liver recipients, more often in those treated with CsA than with TAC. Jaw bone abnormalities were present in 30.43% kidney recipients vs. 12% liver recipients. CONCLUSIONS Both kidney and liver recipients present dental and bone abnormalities. The incidence of specific types of oral lesions is different in renal and liver graft recipients; however, it is also correlated with specific immunosuppression.

Post-Transplant Lymphoproliferative Disorder (PTLD) Manifesting in the Oral Cavity of a 13-Year-Old Liver Transplant Recipient (LTx)

BACKGROUND Post-transplant lymphoproliferative disorder (PTLD) is a potential complication of solid organ or bone marrow transplants. The main PTLD risk factors are: the Epstein-Barr virus (EBV), transplant type, and use of immunosuppressants. It mainly consists of an uncontrolled growth of lymphocytes in transplant recipients under chronic immunosuppressive therapy. About 85% of PTLDs are EBV-containing B-cell proliferations; 14% are T-cell proliferations, of which only 40% contain EBV; and the remaining 1% is NK-cell or plasmocyte proliferations. PTLD may present various clinical manifestations, from non-specific mononucleosis-like syndrome to graft or other organ damage resulting from pathologic lymphocyte infiltration. PTLD may manifest in the oral cavity. CASE REPORT The objective of this study was to present the case of a 13-year-old female living-donor liver transplant recipient, resulting from biliary cirrhosis caused by congenital biliary atresia, with exophytic fibrous lesions on buccal mucosa and tongue. Exophytic and hyperplastic lesion of oral mucosa were removed and histopathological examination revealed polymorphic PTLD. The patient underwent 6 cycles of CHOP chemotherapy and all the oral lesions regressed completely. CONCLUSIONS All oral pathological lesions in organ transplant recipients need to be surgically removed and histopathologically examined because they present an increased risk of neoplastic transformations such as PTLD.

Crohn's disease should be considered in children with inflammatory oral lesions

This systematic review presents the oral manifestations of paediatric Crohns disease. Our review of 28 papers published from 2000 to 2015 showed that the prevalence of oral manifestations was 10–80%. Specific symptoms included mucosal tags, swelling of the lips, cheeks and gingiva, and cobblestoning mucosa. Nonspecific symptoms included aphthous‐like ulcers, angular cheilitis, lip fissuring and gingivitis.

Oral findings in patients with Nijmegen breakage syndrome: a preliminary study.

OBJECTIVE The objective of this study was to assess the oral cavity status of patients with Nijmegen breakage syndrome (NBS), an inherited genetic disorder that belongs to the group of chromosome instability syndromes and is characterized by microcephaly, a distinct facial appearance, growth retardation, radiation sensitivity, and immunodeficiency. STUDY DESIGN Oral examination was conducted and immunological status assessed in 21 NBS patients (1.7-20.7 years old) and 21 healthy controls (5-21 years old). The differences between the frequency and severity of clinical manifestations and their correlation with immune parameters were analyzed by Student t test, the chi-square test, and Spearmans rank order correlation. RESULTS Lesions of the oral mucosa and gingivitis were diagnosed more frequently in NBS patients than in controls. The mean Gingival Index was significantly higher in NBS subjects (P = 0.00043). Candidiasis was detected in 6 patients (28.6%) and in none of the healthy controls. Immune deficiency (humoral and/or cellular) was detected in 20 of 21 (95.2%) NBS patients. There was a significant association between severity of gingival inflammation and reduced number of B- and/or CD3+/CD4+ T cells combined with IgA+IgG4 deficiency. CONCLUSION Our study showed that oral manifestations diagnosed in NBS patients were associated with combined deficiencies of the humoral and cellular arms of the immune system. We postulate that periodical examination of the oral cavity is essential for early medical intervention.

Oral health and liver function in children and adolescents with cirrhosis of the liver.

Introduction People with cirrhosis of the liver are predisposed to developing oral lesions. The occurrence and type of lesion depend on the degree of liver function impairment and its type, and on the severity and duration of systemic diseases. In children, the age at which the early symptoms of liver disease are experienced is also of great importance. Aim To assess the prevalence of oral pathological lesions in children and adolescents with cirrhosis of the liver, and their correlation with the degree of liver function impairment. Material and methods Clinical and laboratory results of liver function tests (Model of End-Stage Liver Disease/Score of Paediatric End-Stage Liver Disease, Child-Pugh score) were assessed in 35 patients with cirrhosis of the liver. The average age of the patients was 10.7 ±4.74 years. All patients also had their oral cavities examined (mucosa, gingiva – GI, hygiene – PLI, teeth – dmft/dmfs and DMFt/DMFs, DDE Index and Candida spp. presence) and this was then correlated to the degree of liver function impairment. Results According to the Child-Pugh scale, 16 patients were class A and 19 were class B/C. Jaundice during the first 3 years of life occurred in 9 patients. Mucosal lesions were found in 26 out of 35 patients (74%), including 10 out of 16 (63%) in Child-Pugh group A, and 16 out of 19 (84%) in group B/C (NS – non significant). Oral candidiasis occurred more often in class B/C than in class A (47.4% vs. 12.5%; p < 0.05). The GI index (Gingival Index) and PLI index (Dental Plaque Index) did not differ between the groups (A vs. B/C) but correlated in the whole group (R = 0.58) as well as in subgroups A (R = 0.65) and B/C (R = 0.59). Dmft/dmfs and DMFt/DMFs indexes did not differ between groups A and B/C, and neither did the DMFt/DMFs in patients with/without enamel defects. Conclusions Oral mucosal lesions are commonly found in children with cirrhosis of the liver. Advanced liver disease promotes oral candidiasis. Severity of gingivitis correlates with the presence of dental plaque.

Single Nucleotide Polymorphism in the Aetiology of Caries: Systematic Literature Review

Recent progress in the field of molecular biology and techniques of DNA sequence analysis allowed determining the meaning of hereditary factors of many common human diseases. Studies of genetic mechanisms in the aetiology of caries encompass, primarily, 4 main groups of genes responsible for (1) the development of enamel, (2) formation and composition of saliva, (3) immunological responses, and (4) carbohydrate metabolism. The aim of this study was to present current knowledge about the influence of single nucleotide polymorphism (SNP) genetic variants on the occurrence of dental caries. PubMed/Medline, Embase, and Cochrane Library databases were searched for papers on the influence of genetic factors connected with SNP on the occurrence of dental caries in children, teenagers, and adults. Thirty original papers written in English were included in this review. Study groups ranged from 30 to 13,000 subjects. SNPs were observed in 30 genes. Results of the majority of studies confirm the participation of hereditary factors in the aetiology of caries. Three genes, AMELX, AQP5, and ESRRB, have the most promising evidence based on multiple replications and data, supporting a role of these genes in caries. The review of the literature proves that SNP is linked with the aetiology of dental caries.