The Diagnostic Performance of Early Sjögren's Syndrome Autoantibodies in Juvenile Sjögren's Syndrome: The University of Florida Pediatric Cohort Study.

Objectives: The aim of this study was to evaluate the clinical validity of early Sjögren's syndrome (SS) autoantibodies (eSjA), which were originally marketed for early diagnosis of SS, for juvenile SS (JSS) in a recently identified pediatric cohort. Methods: A total of 105 symptomatic subjects with eSjA results available were evaluated at the Center for Orphaned Autoimmune Disorders at the University of Florida and enrolled for this study. JSS diagnosis was based on the 2016 ACR/EULAR SS criteria. Demographic/clinical/laboratory parameters were compared between JSS (n = 27) and non-JSS (n = 78) for % positivity, sensitivity, and specificity of eSjA (SP1, anti-salivary protein; CA6, anti-carbonic anhydrase VI; PSP, anti-parotid secretory protein) and classic SS-autoantibodies (cSjA; ANA, SSA/SSB, RF, and others) either alone or in combination. Associations between eSjA and diagnostic/glandular parameters were also determined by Fisher's exact test. Results: Compared to non-JSS, JSS patients exhibited sicca symptoms demonstrating reduced unstimulated salivary flow rate (USFR) and abnormal glandular features revealed by salivary gland ultrasound (SGUS). Among cSjA, ANA demonstrated the highest sensitivity of 69.2%, while SSA, SSB, and RF showed around 95% specificities for JSS diagnosis. The % positive-SSA was notably higher in JSS than non-JSS (56% vs. 5%). Of eSjA, anti-CA6 IgG was the most prevalent without differentiating JSS (37%) from non-JSS (32%). Sensitivity and specificity of eSjA were 55.6 and 26.9%, respectively. Autoantibodies with potentially applicable specificity/sensitivity for JSS were seen only in cSjA without a single eSjA included. There were no associations detected between eSjA and focus score (FS), USFR, SSA, SGUS, and parotitis/glandular swelling analyzed in the entire cohort, JSS, and non-JSS. However, a negative association between anti-PSP and parotitis/glandular swelling was found in a small group of positive-SSA (n = 19, p = 0.02) whereas no such association was found between anti-PSP-positive compared to anti-PSP-negative. JSS and non-JSS groups differed in FS, USFR, and EULAR SS Patient Reported Index Dryness/Mean in CA6/PSP/ANA, SP1, and SSA-positive groups, respectively. Additionally, a higher FS was found in RF-positive than RF-negative individuals. Conclusions: eSjA underperformed cSjS in differentiating JSS from non-JSS. The discovery of clinical impact of eSjA on early diagnosis of JSS necessitates a longitudinal study.

Deep Sedation Technique for Dental Rehabilitation of a Patient with Klippel-Feil Syndrome.

Klippel-Feil syndrome (KFS) is a congenital anomaly characterized by a defect in the formation or segmentation of the cervical vertebrae, resulting in their fusion. The clinical triad of the syndrome consists of short neck, low posterior hairline, and limited neck movement, although fewer than 50 percent of patients demonstrate all three clinical features. The short neck and its immobility and instability present a significant challenge for endotracheal intubation. The purpose of this paper is to describe the management of a 13-year-old patient with KFS, extensive dental caries, and restricted mouth opening using a deep sedation technique in the operating room, which allowed successful completion of dental treatment.

Eruption hematoma as a possible oral sign of infantile scurvy.

Scurvy, vitamin C deficiency, is uncommon in industrialized societies today. Although supplementation of food with vitamin C has diminished its incidence, scurvy continues to occur in specific economically and nutritionally disadvantaged populations. The purpose of this report was to describe the case of infantile scurvy in a 20-month-old male with multisystem involvement including significant oral manifestations. Following an extensive initial evaluation, the multidisciplinary approach to diagnosis and management is discussed. This case demonstrates the need for heightened awareness of severe and multiplefood allergies in children and highlights disease conditions caused by nutritional deficiencies in this population.

Dental management of a patient with factor X deficiency.

Factor X deficiency (also known as Stuart-Prower factor deficiency) is an extremely rare hereditary hematologic disorder, affecting 1 person in 2 million. The gene causing this condition is autosomal recessive; thus, only those inheriting from both parents exhibit clinical symptoms, such as moderate bleeding, easy bruising and subcutaneous bleeding from mucous membranes. Patients with marked deficiency may have severe posttraumatic or spontaneous internal or external bleeding, leading to such complications as hemarthrosis or hemorrhagic strokes. This case report describes the dental management of a patient with severe factor X deficiency whose development was delayed due to multiple episodes of intracranial hemorrhage in early childhood. It also highlights the importance of timely interdisciplinary communication and consultation to promote a successful outcome.

Impact of Er,Cr:YSGG laser therapy on the cleanliness of the root canal walls of primary teeth.

Root canal therapy might be required for primary teeth displaying signs of pulpal inflammation or necrosis. Cleaning and shaping followed by obturation of the canal space with a resorbable paste have been widely performed with remarkable clinical success. However, lengthy endodontic procedures might be contraindicated when treating certain pediatric patients. The aim of this study was to compare the cleanliness of the root canal walls of primary teeth and the time required for the completion of the cleaning and shaping procedures performed by the Er,Cr:YSGG laser, manual or rotary instrumentation techniques. Thirty-five extracted, single-rooted, primary teeth were divided into 4 groups: I, canals were instrumented with Profile .04 rotary instruments to a master apical file size #35; II, the laser was used (parameters: 1.50 W, 20 pps, 30% water and 50% air) with a Z3 laser tip (0.32-mm diameter); III, canals were instrumented with stainless steel K-files; and IV, no instrumentation was performed (control). The teeth were split in 2 halves and prepared for scanning electron microscopy analysis. Images from the coronal, middle, and apical thirds of the roots were analyzed independently by 2 calibrated, blinded evaluators. Statistical analysis revealed significant differences among the groups (Kruskal-Wallis, P = .0001). The techniques were not capable of providing completely clean canals. Treatment with Er,Cr:YSGG laser provided similar cleanliness when compared with rotary instrumentation technique and was superior to manual instrumentation. The laser technique required less time for completion of the cleaning and shaping procedures when compared with both rotary or hand instrumentation.