Infection may play an important role in the pathogenesis of alveolar osteonecrosis following facial herpes zoster: a case report and literature review.

BACKGROUND: Herpes zoster (HZ) is one of the most common skin diseases caused by viruses. Facial HZ develops when the varicella-zoster virus affects the trigeminal nerve, and alveolar osteonecrosis is a rare complication. However, the exact pathogenesis of postherpetic alveolar osteonecrosis remains unclear. CASE DESCRIPTION: We encountered a patient who presented to the dermatology clinic with facial HZ and tooth exfoliation in the upper right jaw, and panoramic radiography revealed decreased bone density and poor alveolar socket healing in his right maxilla. Biopsy of the alveolar process revealed fragments of nonvital lamellar bone, which were devoid of osteoblasts and osteocytes and were surrounded by numerous neutrophils and bacterial aggregates. Thus, the diagnosis of alveolar osteonecrosis following facial HZ was confirmed. He then underwent resection of the osteonecrotic tissue. The pathological findings of postoperative tissue were similar to those of previous biopsies. Varicella-zoster virus and multiple types of bacteria were detected through next-generation sequencing, and the species of bacteria were consistent with the results of bacterial culture. Antibiotics and valaciclovir were administered during the perioperative period. The patient showed good recovery at the 9-month follow-up. CONCLUSIONS: The coexistence of bacterial and viral infection may play an important role in the pathogenesis of alveolar osteonecrosis following HZ. To our knowledge, we are the first to directly explore microbial pathogens in a case of postherpetic alveolar osteonecrosis through next-generation sequencing and bacterial culture. We recommend that oral examinations be carefully conducted for patients who are diagnosed with facial HZ, even if their facial rashes have faded away. We suggest that a prolonged and full-dose antiviral therapy course may be beneficial for the treatment of facial HZ with intraoral lesions. The implementation of dental preventive measures should be considered for patients with facial HZ. The application of antibiotics and excision of necrotic bone may reduce the abundance of bacteria in lesions and improve wound healing.

Hypophosphatasia and the importance of the general dental practitioner - a case series and discussion of upcoming treatments.

Hypophosphatasia (HPP) is an inherited metabolic disorder that results in poorly mineralised bones and teeth. Clinical symptoms vary widely from mild dental anomalies to fatal fetal defects. The most common dental symptoms include exfoliation of the primary incisors before the age of three with little or no root resorption, large pulp chambers, alveolar bone loss and thin dentinal walls. There is generally minimal periodontal inflammation associated with the bony destruction and tooth loss. The general dental practitioner is usually the first clinician to spot signs of the milder forms of HPP. Patients diagnosed with dental symptoms in childhood can go on to develop significant morbidity in middle age with chronic bone pain and stress fractures of the long bones. The primary dental care clinician is the key to early diagnosis of such cases, whether they present in childhood or adulthood. Emerging enzyme replacement therapy has considerably changed the landscape of the disease, resulting in astonishing improvements in bone mineralisation and a significant reduction in mortality and morbidity. It is increasingly likely that primary and secondary care clinicians will treat patients with the severe forms of HPP, who would previously not have survived infancy.

Gene therapy improves dental manifestations in hypophosphatasia model mice.

BACKGROUND AND OBJECTIVE: Hypophosphatasia is a rare inherited skeletal disorder characterized by defective bone mineralization and deficiency of tissue non-specific alkaline phosphatase (TNSALP) activity. The disease is caused by mutations in the liver/bone/kidney alkaline phosphatase gene (ALPL) encoding TNSALP. Early exfoliation of primary teeth owing to disturbed cementum formation, periodontal ligament weakness and alveolar bone resorption are major complications encountered in oral findings, and discovery of early loss of primary teeth in a dental examination often leads to early diagnosis of hypophosphatasia. Although there are no known fundamental treatments or effective dental approaches to prevent early exfoliation of primary teeth in affected patients, several possible treatments have recently been described, including gene therapy. Gene therapy has also been applied to TNSALP knockout mice (Alpl-/- ), which phenocopy the infantile form of hypophosphatasia, and improved their systemic condition. In the present study, we investigated whether gene therapy improved the dental condition of Alpl-/- mice. MATERIAL AND METHODS: Following sublethal irradiation (4 Gy) at the age of 2 d, Alpl-/- mice underwent gene therapy using bone marrow cells transduced with a lentiviral vector expressing a bone-targeted form of TNSALP injected into the jugular vein (n = 3). Wild-type (Alpl+/+ ), heterozygous mice (Alpl+/- ) and Alpl-/- mice were analyzed at 9 d of age (n = 3 of each), while Alpl+/+ mice and treated or untreated Alpl-/- mice were analyzed at 1 mo of age (n = 3 of each), and Alpl+/- mice and Alpl-/- mice with gene therapy were analyzed at 3 mo of age (n = 3 of each). A single mandibular hemi-section obtained at 1 mo of age was analyzed using a small animal computed tomography machine to assess alveolar bone formation. Other mandibular hemi-sections obtained at 9 d, 1 mo and 3 mo of age were subjected to hematoxylin and eosin staining and immunohistochemical analysis of osteopontin, a marker of cementum. RESULTS: Immunohistochemical analysis of osteopontin, a marker of acellular cementum, revealed that Alpl-/- mice displayed impaired formation of cementum and alveolar bone, similar to the human dental phenotype. Cementum formation was clearly present in Alpl-/- mice that underwent gene therapy, but did not recover to the same level as that in wild-type (Alpl+/+ ) mice. Micro-computed tomography examination showed that gene therapy improved alveolar bone mineral density in Alpl-/- mice to a similar level to that in Alpl+/+ mice. CONCLUSIONS: Our results suggest that gene therapy can improve the general condition of Alpl-/- mice, and induce significant alveolar bone formation and moderate improvement of cementum formation, which may contribute to inhibition of early spontaneous tooth exfoliation.

Hypophosphatasia.

Hypophosphatasia is a rare disorder due to a mutation in the ALPL gene encoding the alkaline phosphatase (ALP) leading to a diminished activity of the enzyme in bone, liver, and kidney. Hypophosphatasia is a heterogeneous disease, ranging from extreme life-threatening forms revealed at birth in young infants presenting with severely impaired bone mineralization, seizures, and hypercalcemia, to young adults with premature exfoliation of their teeth without any other symptom. We will review the challenges of the clinical, biochemical, radiological, and genetic diagnosis. Schematically, the diagnosis relies on low ALP levels and, in most cases, on the genetic defect in the ALPL gene. An enzyme replacement therapy is now developed for hypophosphatasia; early results in the severe form of the disease are extremely encouraging. However, multidisciplinary care remains the core of treatment of hypophosphatasia encompassing nutritional support, adjustment of calcium and phosphate intake, monitoring of vitamin D levels, careful and personalized physical therapy, and regular dental monitoring and care.

Congenital adrenal hyperplasia: a case report with premature teeth exfoliation and bone resorption.

Congenital adrenal hyperplasia (CAH) is an inherited autosomal recessive disorder characterized by insufficient production of cortisol. The aim of this case report was to present a child with CAH, premature exfoliation of primary teeth and accelerated eruption of his permanent teeth related to bone resorption. A 4.5-year-old Caucasian boy with CAH and long-term administration of glucocorticoids was referred for dental restoration. Clinical examination revealed primary molars with worn stainless steel crowns, severe attrition of the upper canines, and absence of the upper incisors. Before the completion of treatment, abnormal mobility of the first upper primary molars and the lower incisors was detected, and a few days later the teeth exfoliated prematurely. Histologic examination revealed normal tooth structure. Alkaline phosphatase and blood cells values were normal. Eruption of the permanent dentition was also accelerated. Tooth mobility was noticed in the permanent teeth as soon as they erupted, along with bone destruction. Examination revealed an elevated level of receptor activator of nuclear factor-κB ligand and lower-than-normal osteoprotegerin and vitamin D levels. The patient was treated with vitamin D supplements, and his teeth have been stable ever since. CAH is a serious chronic disorder appearing in children with accelerated dental development and possibly premature loss of primary teeth.

Exfoliation rates of primary molars submitted to three treatment protocols after 3.5 years.

OBJECTIVE: The aim of the study was to evaluate the exfoliation pattern of primary molars treated according to three treatment protocols. The hypothesis tested was that there is no difference in the exfoliation pattern of primary molars treated according to conventional restorative treatment using amalgam (CRT), atraumatic restorative treatment using high­viscosity glass­ionomer (ART), and ultraconservative treatment (UCT). The latter consisted of restoring small cavities with ART and cleaning medium/large nonrestored cavities daily with toothpaste/toothbrush under supervision. METHODS: A sample of 302 children aged 6­7 years from a suburban area of Brasilia was followed up for 3.5 years. The numbers of treated molars were 341 (CRT), 244 (ART), and 275 (UCT). Exfoliation rates were obtained using the PHREG procedure. Differences between the three treatment protocol groups were tested using the Wald test. RESULTS: After 3.5 years, there was no difference over the exfoliation rates of all primary molars as 51.0% (CRT), 48.7% (ART), and 59.3% (UCT) had exfoliated at the overall period (P = 0.34). CONCLUSION: The three treatment protocols led to similar exfoliation patterns of all primary molars after 3.5 years.

Premature loss of primary anterior teeth due to trauma--potential short- and long-term sequelae.

Traumatic dental injuries (TDIs) can result in the premature loss of primary anterior teeth due to an immediate avulsion, extraction later after the injury because of poor prognosis or late complications, or early exfoliation. There are a number of potential considerations or sequelae as a result of this premature loss that have been cited in the dental literature, which include esthetics, quality of life, eating, speech development, arch integrity (space loss), development and eruption of the permanent successors, and development of oral habits. This article provides a comprehensive review of the dental literature on the possible consequences of premature loss of maxillary primary incisors following TDI.

Chediak-Higashi Syndrome and Premature Exfoliation of Primary Teeth

The Chediak-Higashi syndrome (CHS) is a rare hereditary fatal disease, if not treated. These changes are associated with various diseases and syndromes that mainly cause periodontal disease and thus the premature loss of teeth. This paper describes the monitoring of premature loss of primary teeth that began when the child was 5 years old. On presentation his teeth were mobile and there was a history of gingival bleeding. Panoramic radiography revealed generalized and severe bone loss, and the teeth showed no bony support enough for their stability. Blood test was ordered to assess the overall health of the child and giant cells with cytoplasmic granules were found, confirming the diagnosis of CHS. The management of periodontal disease focused on the control of infection and bacterial plaque by mechanical and chemical methods.

A síndrome de Chediak-Higashi (CHS) é uma doença rara hereditária e fatal se não for tratada. Estas alterações estão associadas com várias doenças e síndromes que, principalmente, causa a doença periodontal assim como perda prematura de dentes. Este artigo descreve o monitoramento de perda prematura de dentes decíduos, que começou quando a criança tinha 05 anos. Na apresentação os dentes estavam com mobilidade e apresentava sangramento gengival. As radiografias panorâmicas revelaram perda óssea generalizada e grave, e os dentes sem suporte ósseo suficiente para a sua estabilidade. Foi realizada o exame de sangue para avaliar a saúde geral da criança no qual foi encontrado células gigantes com grânulos citoplasmáticos, confirmando o diagnóstico de CHS. O tratamento da doença periodontal por meio de métodos mecânicos e químicos são necessários para controle da infecção e da placa bacteriana.

Chediak-Higashi syndrome and premature exfoliation of primary teeth.

The Chediak-Higashi syndrome (CHS) is a rare hereditary fatal disease, if not treated. These changes are associated with various diseases and syndromes that mainly cause periodontal disease and thus the premature loss of teeth. This paper describes the monitoring of premature loss of primary teeth that began when the child was 5 years old. On presentation his teeth were mobile and there was a history of gingival bleeding. Panoramic radiography revealed generalized and severe bone loss, and the teeth showed no bony support enough for their stability. Blood test was ordered to assess the overall health of the child and giant cells with cytoplasmic granules were found, confirming the diagnosis of CHS. The management of periodontal disease focused on the control of infection and bacterial plaque by mechanical and chemical methods.

Erythromelalgia presenting with premature exfoliation of primary teeth: a diagnostic dilemma.

Erythromelalgia is an extremely rare neurovascular disorder, characterized by symptoms of red, hot, and painful extremities. There is considerable confusion regarding the etiology and pathogenesis of this condition, and the diagnosis is essentially a clinical one. This condition may occur in isolation or in association with other myeloproliferative disorders. Unfortunately, no therapy is effective consistently in managing the symptoms, although early diagnosis can aid in psychological counseling and minimizing the frequency and severity of the attacks. The purpose of this report was to describe the case of a child presenting with premature loss of primary teeth and the difficulties in determining the final diagnosis of erythromelalgia, which responded positively to low-grade aspirin therapy.

Alveolar bone necrosis and spontaneous tooth exfoliation in an HIV-seropositive subject with herpes zoster.

Herpes zoster in the distribution of the maxillary and mandibular divisions of the trigeminal nerve is characterized by painful vesicular eruptions of the skin and oral mucosa in the distribution of the affected nerves. Oral complications may occur, including post-herpetic neuralgia, devitalization of teeth, abnormal development of permanent teeth, root resorption and periapical lesions. In cases where necrosis of the alveolar bony process occur it may be preceded or accompanied by spontaneous exfoliation of teeth. This usually follows the resolution of the acute phase of HZ and is more prevalent in HIV-seropositive than in HIV-seronegative subjects. A case of HZ of the trigeminal nerve in an HIV-seropositive subject, with complications of necrosis of alveolar bony process, external root resorption and tooth exfoliation is presented and the literature of HIV-associated HZ is reviewed.

Intraoral findings of Papillon-LeFevre syndrome.

Papillon-Lefevre syndrome (PLS) is a rare autosomal, recessive condition characterized by hyperkeratosis of palms and soles of the feet and elbows and by rapid formation of periodontitis and hypermobility, migration and exfoliation of the teeth of primary and permanent dentition. The purpose of this report was to describe the case of an 8-year-old boy who presented to the Department of Oral Diagnosis and Radiology of Faculty of Dentistry of Ondokuz Mayis University with a chief complaint of mobility and rapid loss of teeth. Hyperkeratosis of palms and soles were realized. His gingivae were hyperemic and edematous, and the teeth were mobile. Histopathological examination of the specimen taken from the thickened skin was reported to be consistent with PLS. All teeth with poor prognosis were extracted and extensive periodontal therapy was administered, and a special denture was constructed.

Periodontitis as a manifestation of chronic benign neutropenia.

BACKGROUND: A subcategory of chronic neutropenia is chronic benign neutropenia, which is characterized by a prolonged non-cyclic neutropenia as the sole abnormality, with no underlying disease to which the neutropenia can be attributed. Chronic neutropenia is defined as a low absolute neutrophil count for >6 months. In this presentation, periodontitis seems to be the sole manifestation of a juvenile patient with chronic benign neutropenia. A 7-year-old white male presented with periodontitis of the primary dentition and early tooth loss. His medical and dental history was otherwise unremarkable. Suspecting some systemic illness as the underlying cause, the patient was referred for a medical consultation and a series of blood tests. METHODS: Blood analyses included a complete blood count (CBC), sequential multiple analyzer 24 (SMA 24), glycated hemoglobin levels, and screening for anti-white blood cell antibodies. Blood levels of calcium, vitamin D, dihydroxyvitamin-D, phosphorus, and alkaline phosphatase were also measured. Liver function tests were performed. RESULTS: Following analysis of recent and previous blood test results, a diagnosis of chronic benign neutropenia was assigned. The patient's periodontal condition was treated with scaling and root planing, oral hygiene instruction, and antimicrobial mouthrinses. Three-month recall visits were recommended as a follow-up protocol. CONCLUSIONS: This case represents the importance of diagnosing periodontal disease as a possible indicator of underlying systemic disease. When a patient presents with an unusual, generalized form of periodontal disease, screening for systemic disorders is required, as the oral condition may be the first or only manifestation of a systemic abnormality. This case also illustrates the reason for the change in classification of such a condition to periodontitis as a manifestation of systemic disease. This condition was previously classified as prepubertal periodontitis, a disease diagnosis that focused on the patient's age at the onset of the disease rather than the etiology.

A case of Coffin-Lowry syndrome with premature exfoliation of primary teeth.

We present a case of a 5-year-old boy with premature exfoliation of primary teeth. All eight primary incisors had exfoliated by the age of 3 years, and three canines and one primary first molar were subsequently lost when he was 4 years old. None of the exfoliated teeth exhibited caries. The boy also showed characteristic facial changes, tapering of the fingers, and mental and motor retardation. Based on these findings, he was diagnosed as having Coffin-Lowry syndrome. Premature exfoliation of primary teeth in Coffin-Lowry syndrome has been described in a few reports. This manifestation of the disease would be helpful for diagnosis at an early stage as those previous reports suggested.

Characterization of a family with dominant hypophosphatasia.

A kindred with dominant hypophosphatasia resulting from an alanine to threonine substitution at position 99 of the alkaline phosphatase protein is described. The clinical findings of individual members of the kindred were assessed by oral and physical examinations, or from the descriptions of multiple family members. The proband displayed enamel hypoplasia and premature loss of fully rooted primary anterior teeth, which were shown by histological examination to lack cementum. Serum alkaline phosphatase (ALP) and a vitamin B6 panel, and urine phosphoethanolamine (PEA) were measured on 21 family members. Based upon the clinical and laboratory tests, affected and unaffected status was assigned. Parametric linkage analysis of the kindred using different dominant models and frequency distributions for the disease allele and the mutation gave lodscores > 4.2 and confirmed the strong linkage between the disease and the mutation. Assuming the defined mutation causes the disease, the reliability of clinical and laboratory tests is assessed.

Longitudinal occlusal changes from primary to permanent dentition in children with normal primary occlusion.

This purpose of this research was to examine the stability of normal occlusion during the transition from primary to permanent dentition. The sample consisted of 128 children (83 boys and 45 girls) 4.5 to 5.5 years old with normal occlusion in the primary dentition. The subjects were reexamined at 12.5 to 13.5 years. None had received orthodontic treatment. Although all the subjects had normal occlusion in the primary dentition, 72.7% (73.5% boys and 71.1% girls) had developed anomalies following eruption of the permanent teeth. These anomalies included crowding, Class II Division 1 or Class II Division 2 malocclusion, mesial occlusion complex, lateral crossbite, anterior crossbite, premature tooth loss, openbite or other anomalies.

Exfoliation of maxillary central incisors due to misapplication of orthodontic rubber bands.

The patient's problem consisted of a large diastema between the central incisors. The cost-cutting treatment plan recommended to the patient and his parents by the general dentist was to use orthodontic rubber bands to loop the teeth and close the diastema.

Microbiological findings in prepubertal periodontitis. A case report.

Generalized pre-pubertal periodontitis (GPP) is a rare entity that usually affects children with severe systemic diseases. We report the case of a 7-year-old male patient diagnosed with GPP, with no apparent systemic condition, who lost all his primary teeth to periodontal disease. Before extractions, while he was still in mixed dentition the subgingival plaque was collected and analyzed using DNA probes to 40 different microorganisms. Putative periodontopathogens such as Prevotella intermedia, Selenomonas noxia, Fusobacterium nucleatum, and Actinobacillus actinomycetemcomitans could be identified throughout the mouth. More intriguing was the colonization of the sulcus of some secondary teeth by potentially harmful microorganisms found in pockets of diseased adjacent primary teeth.

Fibroma en el reborde alveolar en una niña de cinco meses: reporte de un caso y revisión de la literatura / Fibroma of the alveolar ridge in a five-month-old girl: report of a case and review of the literature

Se hace el reporte de un caso de fibroma traumático en el reborde alveolar en una niña de cinco meses. La aparición de la lesión está en estrecha relación con la exfoliación espontánea de dientes neonatales y el hábito de succión del pulgar