Primary Sjögren syndrome in a 2-year-old patient: role of the dentist in diagnosis and dental management with a 6-year follow-up.

BACKGROUND. Primary Sjögren syndrome is a rare autoimmune disease, especially in children, mainly affecting girls (77%), and usually diagnosed around 10 years of age. Diagnosis during childhood is difficult, especially because of the diversity of the clinical presentation and difficulty obtaining reliable history data, accounting for a higher frequency of underdiagnosed cases. Differential conditions should be considered, especially the ones that promote xerostomia, such as diabetes, ectodermal dysplasia, rheumatoid arthritis, scleroderma, systemic lupus erythematosus, sarcoidosis, lymphoma, HIV and HTLV infection. Conditions associated with parotid enlargement should also be excluded, including juvenile recurrent parotitis (JRP), sialadenosis, sarcoidosis, lymphoma, infectious parotitis caused by streptococcal and staphylococcal infections, viral infections (paramyxovirus, Epstein-Barr virus, cytomegalovirus, and parvovirus), and diffuse infiltrative lymphocytosis syndrome (associated with HIV infection), and rare congenital conditions, such as polycystic parotid disease. CASE REPORT. A paediatric female patient was referred to our clinic for dental treatment complaining about dry mouth, oral discomfort, and dysphagia. The patient presented five of the required criteria to establish the diagnosis of pSS, including ocular symptoms, oral symptoms, evidence of keratoconjunctivitis sicca, focal sialadenitis confirmed by minor salivary gland biopsy, and evidence of major salivary gland involvement. Our patient did not have positive SS-A and SS-B autoantibodies. According to the literature, about 29% of individuals with pSS can present seronegativity for SS-A (anti-Ro) antibodies and about 33% can present seronegativity for SS-B (anti-La) antibodies. CONCLUSION. To the best of our knowledge, this is the youngest patient reported in the scientific English literature with pSS. Primary Sjögren syndrome has a wide clinical and immunologic spectrum and may progress with increased morbidity. Clinicians must be aware of the development of pSS in such an early age and exclude all possible differential findings to provide early diagnosis and treatment.

Oral manifestations and rehabilitation in Fraser syndrome: A case report.

Fraser syndrome (FS) is a rare recessive autosomal genetic disorder characterized by multisystemic malformations typically comprising cryptophthalmos, syndactyly, and renal defects. We report the case of a 16-year-old patient who exhibited facial asymmetry, short roots, hypodontia, and malocclusion. Oral rehabilitation included orthodontics, exodontia, and osseointegrated dental implants to improve the patient's self-esteem and eating function. We suggest short roots and hypodontia assessment in patients with FS.

Kostmann syndrome: oral aspects and 10-year follow-up case report.

Kostmann syndrome (KS) is an autosomal recessive disorder characterized by a low neutrophil count and recurrent bacterial infections, including periodontal disease. This report describes the case of a 5-year-old female KS patient treated with regular infusions of granulocyte stimulating factor (granulokine), with primary complaint of gingival bleeding and tooth mobility, and followed up for 10 years. Conventional periodontal treatment and some extractions were performed, after neutrophil function tests using flow cytometry. The invasive procedures were carried out at an outpatient clinic under antibiotic prophylaxis due to a low neutrophil count and an impaired neutrophil function presented. During the 10-year follow-up period, despite episodes of recurrent gingivitis and periodontitis the present report describes the positive outcome of dental treatment of a patient with KS.

Medical problems and oral care of patients with Down syndrome: a literature review.

When planning the dental treatment of patients with Down syndrome (DS), dental practitioners should always consider their general health, in order to achieve a holistic and interdisciplinary approach. This article presents a literature review of the primary medical conditions in patients with DS that may affect their general health care and the appropriate clinical delivery of oral health care.

Detection of the Epstein-Barr virus (EBV) by in situ hybridization as definitive diagnosis of hairy leukoplakia.

Histopathological findings in cases of hairy leukoplakia (HL) are not exclusive to this lesion. A total of 36 tissue samples from patients previously diagnosed with HL based solely on morphological aspects were used in this study. Our purpose was to confirm the presence of Epstein-Barr virus (EBV) in these tissue samples by in situ hybridization (ISH), and to compare the detection of EBV with specific histopathological findings observed in each case. Among the 36 specimens, 80.55% were EBV positive, confirming the previous clinical and histhophatological diagnosis. None of the histopathological findings analyzed correlated with the presence or absence of EBV. This shows that a definitive diagnosis of HL cannot be established based on histopathological findings alone. Because there are many important implications on the establishment of definitive diagnosis of HL, the detection of EBV by ISH is obligatory.