Dental and Endodontic Management in a Patient with Familial X-Linked Hypophosphatemic Rickets

ABSTRACT: The term rickets refers to insufficient or retarded mineralization of the osteoide matrix. X-linked hypophosphatemic (XLH) rickets is a rare genetic disorder characterized by biochemical changes in bone mineralization due to inactivation of the phosphate regulating gene and primary defect of the osteoblasts. The aim of this article was to report a clinical case of XLH, its oral manifestations, periapical changes and dental management. A 31-year old woman female patient was referred to the school of dentistry with pain and sensitivity in the teeth. She had a childhood history of rickets, hypophosphatemia and alteration in Vitamin D. In the oral exam, enamel hypoplasia, microdontia, fistula, caries and periapical lesions and periodontal disease were diagnosed. The radiographic and tomographic exams exhibited the presence of periapical lesions involving various teeth with radiolucent images, suggestive of granuloma or periapical cysts. The treatme nt prioritized the urgency of eliminating pain and removing the foci of infection. Endodontic treatment began in the teeth that had fistula or periapical lesions and in parallel, oral hygiene guidance was provided and periodontal treatment was performed. There was an improvement in the clinical condition with reduction in inflammation and mobility of the teeth. Dentists and health professionals must evaluate the patient as a whole, considering the relations between systemic and oral health. Knowledge of systemic diseases associated with rickets and their characteristics is essential for making a correct oral diagnosis and planning the dental treatment.

RESUMEN: El término raquitismo se refiere a la mineralización insuficiente o retardada de la matriz osteoide. El raquitismo hipofosfatémico ligado al cromosoma X (XLH) es un trastorno genético caracterizado por cambios bioquímicos en la mineralización ósea debido a la inactivación del gen regulador del fosfato y al defecto primario de los osteoblastos. El objetivo de este artículo fue reportar un caso clínico de XLH, sus manifestaciones orales, cambios periapicales y manejo dental. La paciente, una mujer de 31 años, acudió a la Clínica de Semiología de la UFPR con dolor y sensibilidad en varios dientes. Tenía antecedentes infantiles de raquitismo, hipofosfatemia y alteración de la vitamina D. En el examen oral se diagnosticó hipoplasia del esmalte, microdoontia, fístula, caries y lesiones periapicales y enfermedad periodontal. Los exámenes radiográficos y tomográficos mostraron la presencia de lesiones periapicales en varios dientes con imágenes radiolúcidas, sugestivas de granuloma o quistes periapicales. El tratamiento priorizó la urgencia de eliminar el dolor y remover los focos de infección. Se inició tratamiento de endodoncia en los dientes que presentaban fístula o lesiones periapicales y paralelamente se brindó orientación de higiene oral y se realizó tratamiento periodontal. Hubo una mejoría en la condición clínica con reducción de la inflamación y movilidad de los dientes. Los odontólogos y profesionales de la salud deben evaluar al paciente como un todo, teniendo en cuenta las relaciones entre salud sistémica y oral. El conocimiento de las enfermedades sistémicas asociadas al raquitismo y sus características es fundamental para realizar un correcto diagnóstico oral y planificar el tratamiento odontológico.

Management of Sjogren's Syndrome Patient: A Case Report of Prosthetic Rehabilitation with 6-Year Follow-Up.

Completely and partially edentulous patients with Sjogren's syndrome (SS) experience severe hyposalivation, xerostomia, and considerable difficulty in using tissue-supported prosthesis. This clinical paper describes the management, treatment, and 6-year follow-up of a patient diagnosed with SS type II, who uses corticosteroids and antihyperglycemic drugs. The patient received restorative, periodontal, and surgical treatments followed by implant-supported fixed prosthesis. Radiographic evaluation and probing depth showed gingival health and no bone loss after 6 years. Treatment with implant-retained dental prosthesis greatly increased comfort and function, offering an alternative to patients with SS.

Management of two cases of desquamative gingivitis with clobetasol and Calendula officinalis gel.

BACKGROUND: The purpose of this paper is to describe two cases of desquamative gingivitis (DG) that were treated with a topic gel containing clobetasol propionate and Calendula officinalis L in an acetate tray over two years. METHODS: Two patients with a diagnosis of lichen planus presenting as DG who had undergone previous treatments for this condition with no significant results, were treated by a handling gel containing clobetasol, nystatin, Calendula officinalis L and pectin in custom trays. RESULTS: Both patients had remission of symptoms while using the trays and after they stopped the treatment, the symptomatic outbreaks were delayed and presented as less severe symptoms in the two years follow-up. The treatment is aimed primarily at reducing the length and severity of symptomatic outbreaks desquamative gingivitis. CONCLUSION: This handling gel using a tray may be an efficacious treatment of desquamative gingivitis.

Oral focal mucinosis.

Oral focal mucinosis is an uncommon clinicopathologic condition that is considered the oral counterpart of cutaneous focal mucinosis. It is a disease of unknown etiology where the connective tissue undergoes a focal myxoid degeneration. A literature review disclosed 47 published cases of oral focal mucinosis. An additional case is presented; the clinical and histologic differential diagnosis, sex, age, location, treatment, and recurrence are discussed.

Oral manifestations of celiac disease. A case report and review of the literature

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Celiac disease is a chronic intestinal disease caused by intolerance to gluten associated with poor digestion and absorption of the majority of nutrients and vitamins, which may affect both developing dentition and oral mucosa. The aim of this study is to make a brief review of the literature about celiac disease and to report on a clinical case, showing the impact it may have on the general and oral health. A 39-year-old woman reported the presence of diarrhea, nauseas, flatulence, colic, difficulty with falling asleep, lack of appetite and loose of 18 Kg in the last two years. She also noted the appearance of symptomatic lesions in the mouth. These lesions had a mean duration of a month and occurred in any region of the oral mucosa, particularly on the tongue. Multiples ulcerations were found in thesublingual caruncula region, at the vestibular fornix and at the lingual apex. Topical treatment was instituted for the oral lesions with immediately relief of the symptoms. The diagnosis of celiac disease was established by means of medical clinical exam, biopsy of the small intestine, and by the presence of specific antibodies in the blood. The patient has been instructed to follow a gluten-free diet. Multiprofessional action with the involvement of a gastroenterologist and other health professionals, such as dentists, is important for diagnosing the disease and guiding the patient withceliac disease to achieve a good quality of life