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Objectives Salivary gland lymphocytic infiltrates are a hallmark of primary SS (pSS), but traditional biopsy techniques hold several disadvantages. Ultrasound-guided core needle (US-guided CN) parotid gland biopsy is minimally invasive and reliable for diagnosis of lymphoma in pSS. This proof-of-concept study aimed to explore this technique in the diagnostic work-up of pSS and is the first to address its value in a consecutive cohort independently of the presence of salivary gland swelling. Methods Combined incisional and US-guided CN parotid biopsy was performed in 20 patients with suspected or confirmed pSS from the Belgian Sjögren’s Syndrome Transition Trial (BeSSTT). Surface area and presence of a focus score (FS) of at least one, germinal centres and lymphoepithelial lesions were recorded. Results Salivary gland tissue was interpretable in 19 patients. Fourteen patients had ≥4 mm2 salivary gland tissue by both techniques, in four US-guided CN biopsies salivary gland tissue was Conclusions US-guided CN biopsy of the parotid gland is at least equivalent to incisional biopsy of the parotid gland in the diagnostic work-up of pSS.

Autoantibody detection is an essential step in pSS diagnosis. However, the value of separate anti-Ro52, anti-Ro60 and anti-SSB/La detection in pSS diagnosis and phenotyping has not been extensively studied. This study aimed to explore disease characteristics of anti-SSA/Ro positive, suspected and definite pSS patients, in relation to serological profiles based on anti-Ro52, anti-Ro60 and anti-SSB/La reactivity.Of 187 anti-SSA/Ro positive patients included in the Belgian Sjögren's Syndrome Transition Trial (BeSSTT), 155 were considered definite pSS patients, due to fulfilment of the 2016 ACR-EULAR classification criteria, and 32 suspected, due to reactivity against SSA/Ro without presence of other criteria. None of the patients met any of the ACR-EULAR exclusion criteria for pSS. Patients were grouped based on the presence of anti-Ro52, anti-Ro60 and anti-SSB/La antibodies.Mono-reactivity against Ro60 or Ro52, double reactivity against Ro52/Ro60 and triple reactivity against Ro52/Ro60 and SSB was detected in respectively 30, 23, 70 and 60 patients. Mono-anti-Ro60 positive patients showed the least pSS features. Mono-anti-Ro52 positive patients reported a significantly higher dryness burden (p=0.016) and tended toward more salivary gland ultrasound (SGUS) abnormalities (p=0.054) than mono-anti-Ro60 positives. Double positive patients showed similar characteristics as mono-anti-Ro52 positive patients, whereas triple positive patients showed lowest unstimulated salivary flow rates (p=0.002) and Schirmer tests (p=0.002), highest ocular staining scores (p0.001), most positive labial salivary gland biopsies (p=0.039), most laboratory abnormalities compatible with B-cell hyperactivity and highest SGUS scores (p0.001) compared to other patient groups.These data indicate that separate detection of anti-Ro52, anti-Ro60 and anti-SSB/La reactivity is not only relevant towards pSS diagnosis, but markedly aids in patient stratification and evaluation of disease burden. Our results suggest a stepwise model in which mono-reactivity against Ro60 displayed the least objective and subjective glandular pSS features, whereas glandular abnormalities and signs of B-cell hyperactivity were most present in patients showing triple reactivity against Ro60, Ro52 and SSB/La.

Salivary gland ultrasound (SGUS) is emerging as essential tool in primary Sjögren's Syndrome (pSS), but its link to symptom-based endotypes is unknown. Therefore, we explored SGUS outcomes in relation to endotypes in patients with definite and suspected pSS.Definite pSS patients (n = 171) fulfilling the 2016 ACR/EULAR classification criteria, and suspected pSS patients (n = 119), positive for at least one criterion, were included in the Belgian Sjögren's Syndrome Transition Trial (BeSSTT). Stratification into endotypes according to the Newcastle Sjögren's Stratification Tool resulted in low symptom burden (LSB), pain dominant with fatigue (PDF), dryness dominant with fatigue (DDF) and high symptom burden (HSB). SGUS was assessed with Hocevar score (0-48). The dataset was randomly divided into a discovery (n = 203) and replication (n = 87) cohort.SGUS had strong discriminative power for pSS classification (AUC=0.74), especially in DDF (AUC=0.89). In definite pSS, Hocevar scores in DDF were high compared to other endotypes (38 (20-44) versus 18 (9-33); p 0.001). Patients with highest SGUS-scores showed more sicca and laboratory abnormalities. Moreover, a subset of young, anti-SSA/Ro positive patients not fulfilling classification criteria showed clear SGUS abnormalities. Replication showed similar results.SGUS-scores were significantly higher in definite pSS with DDF endotype, providing the first evidence of imaging abnormalities in salivary glands matching distinct biological profiles ascribed to pSS endotypes. Additionally, a subset of patients with potential early disease was detected based on presence of anti-SSA antibodies and high SGUS-scores. These results underscore the role of SGUS as powerful tool both in pSS classification and stratification.

Background: Cleft lip and/or palate is the most common congenital craniofacial anomaly. Otitis media with effusion is almost universally present in young children with a cleft palate. OME results in a conductive hearing loss and -if persistent- often requires the placement of ventilation tubes or adjustment of hearing aids in selected cases.

Objectives To investigate to what extent middle ear problems and associated hearing loss affect quality of life (QoL) of children born with a cleft palate. Methods Fifty-five children aged between 6 and 18 years, born with non-syndromic cleft palate ± cleft lip (CP/L) were included. A new health-related quality of life (HRQOL) questionnaire was generated with consideration of the following domains of QoL: communication, hearing loss, physical symptoms, limitation of activities and socio-emotional impact. Results Major psychosocial problems were not reported in the majority of children as a result of their ear and hearing problems. However, according to their parents, 2 out of 3 children, had difficulty speaking clearly and understandably. These communication problems led to behavioural problems and social isolation in 1 out of 5 children. Scholastic achievement was negatively influenced by two factors: hearing loss and sleep disturbance due to ear problems. Conclusions To our knowledge this is the first study to quantitatively measure the ear- and hearing-related impact on QoL in children born with CP/L. Large-scale, multicentre studies are needed to further research and expand on the findings of this pilot study.

Introduction Surgical treatment of head and neck malignancies relies on complete tumoral removal, while inadequate margins necessitate the use of adjuvant therapy. Therefore, intraoperative identification of margin status can help to achieve adequate surgical margins. Recent developments in molecular imaging have shown interesting results in the intraoperative visualization of the tumoral tissue, but the techniques investigated to date are limited in penetration-depth and often display the results on a two-dimensional plane. In order to solve these limitations, we investigated whether the use of high-resolution PET and CT could increase certainty about the surgical margins in all three dimensions. Materials and Methods In the current clinical trial, we used a preclinical micro-PET/CT-system (Molecubes, Ghent, Belgium) as a tool for intraoperative margin assessment. Patients planned for the resection of a pathology-proven malignancy in the head and neck were eligible. Approximately one hour prior to surgery, 4 MBq/kg 18F-FDG was administered, followed by standard of care surgical resection. The resected specimen was subsequently scanned with the micro-PET-CT imaging device, followed by pathological assessment. Results Up to date, seven patients were included in the study and intraoperative PET/CT-imaging of 10 specimens identified the 18F-FDG-distribution with a previously unprecedented spatial resolution. We were able to image malignant tissue in all scanned specimens. However, we also encountered difficulties in differentiating between inflamed and dysplastic tissue versus malignant tissue. Particularly in malignancies of the oral cavity, the increased peritumoral inflammation resulted in an increased uptake of 18F-FDG. In contrast, cutaneous malignancies displayed less peritumoral inflammation which enabled better margin assessment. Conclusions We here describe a proof of concept study allowing 3D tumor delineation in head and neck resection specimens using submillimetric PET/CT imaging. Optimization of this tool by identifying tumor types where 18F-FDG-PET has a sufficient contrast to the surrounding tissue to reduce false positives and negatives is required prior to clinical utilization. Moreover, the development of more tumor specific radiotracers, or hybrid fluorescent probes, could potentially greatly improve intraoperative margin assessment.

Background Cleft lip and/or palate (CL/CP/CLP) is one of the most common congenital anomalies. Children may suffer from a variety of health problems including difficulties with feeding and speech, middle ear problems, hearing loss and associated psychosocial concerns. The extent of impact of this disorder on the parents, however, has not yet been thoroughly evaluated. This pilot study was performed to evaluate the impact of having a child with CL/CP/CLP on the parents' quality of life (QoL) and family functioning and to compare between cleft subgroups. Methods Forty-five parents with children aged 6 months to 6 years with CL/CP/CLP, followed by the multidisciplinary orofacial cleft team of Ghent University Hospital, completed following standardized questionnaires: Impact on Family Scale (IOFS), Family Impact Scale (FIS) and Care-Related Quality of Life Instrument (CarerQoL). Subgroups were compared with diverse unpaired statistical tests. Results Younger children (6m-2y) with CL/CP/CLP entail more impact on parental QoL compared to children aged 2-4y old (p=0.04, e²=0.15/p=0.02, e²=0.17/p=0.02, e²=0.17). Families from children with a syndromic cleft also encounter more impact (p=0.04, r=0.32 /p=0.01, r=0.37 /p=0.008, r=0.40/p=0.003, r=0.45). Prenatal orofacial cleft diagnosis is associated with a higher reporting of family conflicts (p=0.04, r=0.32). In case of non-syndromic clefts, families having children with CLP report more family conflicts compared to CL or CP (p=0.02, e²=0.46). Parental education and number of children within the household showed no significant impact on parental QoL. Conclusion This cross-sectional study confirms that having a child with CL/CP/CLP impacts the parental QoL. This study was performed as a pilot-study for larger multicentre studies, future development of effective screening tools and identification of subgroups at risk. Long-term multidisciplinary follow-up should involve family-centred support.

To evaluate DWI of the bone marrow in the differentiation of monoclonal gammopathy of undetermined significance (MGUS), smouldering myeloma (SMM) and multiple myeloma (MM).The retrospective study includes 64 patients with MGUS, 27 with SMM, 64 with new MM and 12 controls. Signal intensity (SI) of spinal SE-MRI and DWI (b0-1000) as well as apparent diffusion coefficients (ADC) were measured in the T10 and L3. Qualitative assessment of b-images was performed by one experienced radiologist.ADC600 and ADC1000 are the best ADC values in differentiating patient groups (p 0.030). SIT2, SIb1000 and ADC1000 are higher and SIT1 lower in L3 compared to T10 (p 0.050). All quantitative parameters of L3 can differentiate significantly between MGUS and MM (p 0.050) and between patients with percentage plasma cells (PC%) between 0-10 % compared to50 % (p = 0.001). Only SIT2 for L3 can differentiate MGUS from SMM (p = 0.044) and PC%0-10 from PC%10-25 (p = 0.033). Qualitative interpretation of b1000 images allows differentiating MM patients from those with MGUS or SMM (p 0.001).Spinal SE-MRI can differentiate among MGUS, SMM, MM and control subjects. DWI based on the SI on b1000 images and ADC values is increased in MM compared to MGUS and SMM. Qualitative assessment of b-images can differentiate MM from MGUS or SMM.• ADC values are higher in patients with MM compared to MGUS • DWI parameters change late in disease evolution • DWI is sensitive but not specific in diagnosing patients with MM • Qualitative DWI assessment is good in detecting myeloma patients.