Aim
This study assessed correlations between systemic disturbances of paediatric chronic liver diseases (CLD) and oral symptoms in subjects aged 2–18 years.
Methods
It was carried out during ...outpatient appointments at the Children's Memorial Health Institute, Warsaw, Poland, from 2010 to 2015 and comprised 52 CLD patients with a mean age of 12.3 ± 4.6. We also recruited 54 generally healthy controls with a mean age of 12.0 ± 3.7 from the Department of Paediatric Dentistry at the Medical University of Warsaw. The study used various measures, including the Child–Pugh score, which assesses CLD prognosis. We also assessed the causes of liver disease and the medication taken by the patients with CLD.
Results
A total of 24 patients received a Child–Pugh score of seven or more points, while 28 patients were awarded five or six points. More severe cases of gingivitis and a greater prevalence of oral lesions were evident in patients suffering from liver disease. Oral candidiasis, telangiectasia, bald tongue, cracked strawberry lip, yellowish‐brown gum discoloration, petechiae and gingival bleeding all correlated with the severity of liver dysfunction, coagulopathy, protein, bilirubin and creatinine levels and portal hypertension.
Conclusion
This study found that oral lesions and gingival bleeding may indicate the progression of liver failure.
Autosomal dominant hyper-IgE syndrome (AD-HIES) is an inborn error of immunity (IEI) caused by a dominant-negative mutation in the signal transducer and activator of transcription 3 (STAT 3). This ...disease is characterized by chronic eczematoid dermatitis, recurrent staphylococcal skin abscesses, pneumonia, pneumatoceles, and extremely high serum IgE levels. Loss-of-function STAT3 mutations may also result in distinct non-immunologic features such as dental, facial, skeletal, and vascular abnormalities, central nervous system malformations and an increased risk for bone fractures. Prophylactic treatment of Candida infections and prophylactic antimicrobial therapy for staphylococcal skin infections and sinopulmonary infections are essential. An awareness of the oral and maxillofacial features of HIES may facilitate early diagnosis with genetic counselling and may improve future patient care. This study describes oral, dental, and maxillofacial manifestations in 14 patients with genetically defined AD-HIES. We also review the literature and propose recommendations for the complex care of patients with this rare primary immunodeficiency.
Chemotherapeutic treatment in children and adolescents carries a risk of congenital tooth disorders and dentinoma. Study objective is to assess the correlation between tooth abnormalities, early ...complications of multidrug chemotherapy, and chemotherapeutics used in different antineoplastic therapies in children and adolescents.
Enamel defects (developmental defects of enamel index - DDE index) and defects in tooth number, size, and structure were assessed clinically and radiologically in 60 patients who underwent chemotherapy on average 4.9 ±3.4 years earlier (PCH), and 60 generally healthy subjects (control group - CG), aged 6-18 years. Höltta's defect index (DeI) was calculated. Medical files provided information on neoplasm type, age at treatment start and chemotherapy duration, chemotherapeutic type and dose, vomiting, and mucositis (CTCAE v4.0). Statistical significance of differences between groups was assessed with the Mann-Whitney
test and the correlation between dental defects and chemotherapy with Spearman's rank correlation coefficient (significance
≤ 0.05).
Enamel defects, tooth agenesis, microdontia, root resorption, taurodontism, and dentinoma occurred statistically significantly more often in the PCH group. A correlation was established between vincristine use and dose and all types of dental defects; cyclophosphamide, doxorubicin, and isophosphamide and hypodontia; microdontia, root resorption, and enamel defects; etoposide and cisplatin and microdontia, root resorption, and enamel defects; methotrexate root resorption and enamel defects; carboplatin and dentinoma and enamel defects.
and vomiting promoted root resorption, microdontia, and enamel defects.
Dental defects are related to both the use of respective chemotherapeutics, especially vincristine, cyclophosphamide, doxorubicin, and isophosphamide, and to early complications in multidrug chemotherapy -
and vomiting. Vincristine and carboplatin use may promote dentinoma.
To determine reasons for the increase in caries among children/adolescents treated for neoplasms.
Health promoting behaviour, oral hygiene (PLI), gingiva (GI), dentition (DMFt/DMFs), number of teeth ...with white spot lesions (WSL), and enamel defects (ED) were assessed in three groups of 60 patients each. The three groups were as follows: under chemotherapy (CH), after chemotherapy (PCH), and generally healthy (CG). Medical files supplied information on neoplasm type, chemotherapeutic type and dose, age at treatment start, chemotherapy duration, and complications. Statistical analysis was performed with Mann-Whitney U test and Spearman's rho test.
The age at which chemotherapy was started/its duration was 5.9 ±4.0/1.3 ±0.5 years in PCH and 9.12 ±4.44/0.8 ±0.3 years in CH; PCH completed treatment 4.9 ±3.4 years ago. Chemotherapy most often included vincristine (VCR), etoposide (VP-16), adriamycin (ADM), cyclophosphamide (CTX), cisplatin (CDDP), and ifosphamide (IF). Mucositis occurrence was 28.33% in PCH and 45.00% in CH; vomiting occurrence was 43.33% and 50.00%, respectively. Nutrition and prophylaxis mistakes occurred more often in CH/PCH than in CG; PLI, GI, caries incidence and severity, and the number of teeth with WSL were higher. Correlation between caries incidence and chemotherapeutic type and dose, age at treatment start and treatment duration, mucositis, emesis, PLI, GI, ED, no fluoride prophylaxis, and nutritional mistakes was established. Ifosphamide and mucositis treatment played a major role in chemotherapy; after chemotherapy - ED and CTX, ADM, IF, and VP-16.
Caries in permanent teeth in children/adolescents undergoing chemotherapy result from nutritional mistakes, poor prophylaxis, and indirectly from chemotherapy complications (first mucositis and emesis, and later developmental ED).
Severe congenital neutropenia (SCN) comprises a heterogenous group of disorders characterized by a constantly low absolute neutrophil count (ANC) below 0.5 × 10
/l in the peripheral blood and ...maturation arrest of the myelopoiesis in the bone marrow at the promyelocyte/myelocyte stage that lead to early onset of severe bacterial infections in affected patients. Clinical symptoms of congenital neutropenia include sepsis, recurrent respiratory tract infections, mouth ulceration, chronic gingivitis, bacterial skin infections, and urinary tract infections. Patients with SCN often develop periodontitis despite standard medical and dental care. We present oral symptoms in our patient afflicted with SCN due to homozygous mutations in the JAGN1 gene, based on 16 years of observation and treatment at the Paediatric Dentistry Clinic of Children's Memorial Health Institute. In our patient, oral cavity changes typical for SCN - in the form of gingivitis and bleeding from periodontal tissues - appeared around the age of 2 and led to the premature loss of primary teeth. The patient also developed an advanced periodontal disease in the permanent dentition, resulting in the loss of 21 teeth at 15 years of age. Dental care of patients with SCN should be carried out in close cooperation with an immunologist, and dental procedures associated with the risk of bacteremia require antibiotic prophylaxis.
Infantile systemic hyalinosis (ISH) is a rare autosomal recessive disorder caused by a mutation in the ANTXR2 gene encoding a transmembranous protein involved in endothelial development. The ANTXR2 ...(also known as CMG2) locus is on chromosome 4q21. ISH is a common disorder in children of consanguineous parents in Arab countries. Symptoms of ISH manifest within the first months of life as progressive painful joint contractures and edema, hyperpigmentation of the skin, cutaneous nodules, persistent diarrhea with protein-losing enteropathy, and recurrent infections. Children affected by ISH often die undiagnosed in infancy. Histopathologic examination shows hyaline deposits in the skin, skeletal muscles, cardiac muscle, lymph nodes, adrenal glands, gastrointestinal tract, thyroid, and spleen. Hyaline deposits are the result of leakage of plasma components to the perivascular space owing to defective endothelial morphogenesis. ISH manifests most often in the facial region. Patients develop hypertrophy of labial and buccal tissues and massive gingival overgrowths, which impair oral food intake and maintenance of satisfactory oral hygiene. The differential diagnosis of ISH should consider juvenile systemic hyalinosis (an allelic variant of ISH), Winchester syndrome, systemic fibromatosis, stiff skin syndrome, lipoid proteinosis, mucopolysaccharidosis, sphingolipidosis, and mucolipidosis. This report describes a case of massive labial and gingival hypertrophy in a 6-year-old boy with ISH.
Background. Langerhans cell histiocytosis is a rare reactive proliferative disorder marked by excess proliferation and accumulation of mononuclear phagocytes in tissues and organs. Usually, organs ...and systems where Langerhans cells are normally found are involved. Lesions may be limited to one system and be single- or multisite, or many systems may be involved. The etiology is not fully known. According to one of the hypotheses, immune dysfunction due to exuberant response to an unknown antigen may be the cause. The most common clinical symptoms include skin lesions, bone pain, exophthalmos, and enlarged lymph nodes, affecting the liver and spleen. Gingivitis, pocket granulation tissue, ulceration of the gingival papilla, alveolar bone atrophy leading to loosening and loss of teeth are observed in the oral cavity. The aim of the study was to determine the type and incidence of oral manifestations in patients diagnosed with Langerhans cell histiocytosis.
Methods. We evaluated patients’ medical records to obtain data on the children’s age at diagnosis, sex, the form of Langerhans cell histiocytosis, clinical picture (systemic and local oral symptoms), and radiological findings. Dental examinations (clinical and radiological) were performed to assess oral mucosa and periodontal tissues, and medical records were analyzed for the course and treatment of histiocytosis.
Results. The analysis included the medical records of 43 patients with Langerhans cell histiocytosis. Oral lesions in the form of gingivitis, pathological tooth mobility, and expansion of alveolar mandibular bone were observed in 7 patients. Conclusions. Langerhans cell histiocytosis may be accompanied by maxillary, gingival, and mucosal lesions.
Chemotherapy, neoplasms, and their complications linked to malabsorption, malnutrition, and metabolic disorders may lead to improper tooth development and frequent severe caries in patients ...during/after antineoplastic treatment and to a more frequent improper tooth development in patients undergoing chemotherapy during odontogenesis. However, the causes of these abnormalities remain unknown; there are no studies on the impact of antineoplastic treatment and its complications on the chemical composition of mineralised teeth.
To compare the chemical composition of mineralised teeth extracted due to complicated caries in children after chemotherapy, and of teeth extracted due to orthodontic treatment in generally healthy children.
The treatment group included five teeth extracted due to complicated caries in children after antineoplastic treatment. The control group included five teeth extracted due to orthodontic treatment in generally healthy children. The chemical composition of enamel, dentine, cementum, interior of the canal, and enamel abnormalities in teeth extracted from patients after chemotherapy and in generally healthy patients were assessed with energy-dispersive X-ray spectroscopy. Results were analysed statistically.
The magnesium (Mg) and zinc (Zn) mass contents in the enamel of patients after chemotherapy increased and so did the calcium (Ca) to phosphorus (P) ratio when compared to controls. Areas with abnormal enamel in patients after chemotherapy had lower concentrations of Ca and P, and higher concentrations of trace elements (Mg, Cl, and Na). The levels of the assessed elements in dentine, cementum, and inside the canal were similar in both groups of teeth.
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.
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