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Abstract GALNT2, also known as polypeptide N-acetylgalactosaminyltransferase 2, is an enzyme that catalyzes the initial step of O-linked glycosylation, a crucial posttranslational modification that affects protein folding, stability, and function. Alterations in GALNT2 activity have been implicated in various diseases, such as cancer, metabolic disorders, and cardiovascular diseases, highlighting its importance in maintaining normal physiological functions. To investigate the impact of GalNT2 overexpression in vivo for the first time, we generated a conditional transgenic mouse line in which GalNT2 was expressed specifically in the pancreas. Heterozygous overexpression leads to a loss of acinar mass and pancreatic steatosis, whereas homozygous overexpression causes complete pancreatic loss and results in a lethal phenotype. Using a reporter gene mouse line, we demonstrated that adipocytes originate through transdifferentiation from pancreatic cells. GalNT2 overexpression results in additional O-glycosylation sites, which we analyzed through PNA lectin enrichment and mass spectrometric proteome analysis.

BACKGROUND & AIMS: Ulcerative colitis (UC) is characterized by severe inflammation and destruction of the intestinal epithelium, and is associated with specific risk single nucleotide polymorphisms in HLA class II. Given the recently discovered interactions between subsets of HLA-DP molecules and the activating natural killer (NK) cell receptor NKp44, genetic associations of UC and HLA-DP haplotypes and their functional implications were investigated. METHODS: HLA-DP haplotype and UC risk association analyses were performed (UC: n = 13,927; control: n = 26,764). Expression levels of HLA-DP on intestinal epithelial cells (IECs) in individuals with and without UC were quantified. Human intestinal 3-dimensional (3D) organoid cocultures with human NK cells were used to determine functional consequences of interactions between HLA-DP and NKp44. RESULTS: These studies identified HLA-DPA1∗01:03-DPB1∗04:01 (HLA-DP401) as a risk haplotype and HLA-DPA1∗01:03-DPB1∗03:01 (HLA-DP301) as a protective haplotype for UC in European populations. HLA-DP expression was significantly higher on IECs of individuals with UC compared with controls. IECs in human intestinal 3D organoids derived from HLA-DP401pos individuals showed significantly stronger binding of NKp44 compared with HLA-DP301pos IECs. HLA-DP401pos IECs in organoids triggered increased degranulation and tumor necrosis factor production by NKp44+ NK cells in cocultures, resulting in enhanced epithelial cell death compared with HLA-DP301pos organoids. Blocking of HLA-DP401-NKp44 interactions (anti-NKp44) abrogated NK cell activity in cocultures. CONCLUSIONS: We identified an UC risk HLA-DP haplotype that engages NKp44 and activates NKp44+ NK cells, mediating damage to intestinal epithelial cells in an HLA-DP haplotype-dependent manner. The molecular interaction between NKp44 and HLA-DP401 in UC can be targeted by therapeutic interventions to reduce NKp44+ NK cell-mediated destruction of the intestinal epithelium in UC.

Perihilar cholangiocarcinoma, a formidable adversary in the field of hepatobiliary surgery, presents multifaceted challenges. This paper reviews its historical context, classification criteria, and regional variations in definition. It emphasizes the critical role of preoperative assessment, including hepatic hilum anatomy, biliary infiltration characteristics, and residual liver volume evaluation. The impact of the Bismuth-Corlette classification on surgical approach selection is elucidated, providing insights into tailored surgical options for each subtype based on the authors’ center experience in the last ten years. The article also touches upon laparoscopic and robotic surgery potential, underscoring the need for further research in this domain. Finally, it explores the potential role of liver transplantation as a treatment option for select patients ineligible for conventional surgery.

Dental anxiety and dental neglect are interconnected constructs with profound consequences for oral health and corresponding challenges for dental professionals. Meanwhile, other literature has indicated that mindfulness and self-compassion relate negatively to different forms of anxiety, and propose elements of potential interventions. This study aimed to explore the potential impact of dental anxiety as a mediator on the relationships between mindfulness or self-compassion and dental neglect. The results showed significant negative associations between mindfulness and self-compassion with dental anxiety and dental neglect. Mediation analyses supported the hypothesis that dental anxiety mediates the positive associations between mindfulness or self-compassion and dental neglect, providing preliminary evidence for the potential effectiveness of mindfulness and self-compassion interventions in addressing dental anxiety and dental neglect. Future research and potential clinical implications are discussed.

Abstract Background Mindful eating has seen an increase in clinical and non-clinical practices of changing health outcomes. Meanwhile, the restriction of not having validated scales in other languages proposes a barrier to exploring the impact of mindful eating cross-culturally, and specific to the present project, across Greek-speaking populations, limiting the potential of exploring the association with Mediterranean dieting. Methods In the present research, volunteers (n = 706) completed online the Mindful Eating Behaviour Scale and the Mindful Eating Scale. A forward–backwards translation, leading to face validity, and was assessed for internal consistency (Cronbach’s Alpha) and followed up by an assessment of the factorial structure of the scales. Divergent and convergent validity was explored using motivations to eat palatable foods, grazing, craving, Dusseldorf orthorexia, Salzburg emotional eating, and the Salzburg stress eating scales. Results Results indicated that both scales displayed good internal consistency, and the assessment of the factorial structure of the scales was equally good and semi-consistent with the English versions, with parallel analyses and item loadings proposing problems that have been shown in critical review literature. Associations of mindful eating scales to other eating behaviours were replicated to previously established findings with English-speaking populations. Conclusions Findings that deviated from the expected outcomes are central to the discussion on the measurement of mindful eating, and further direction highlights the way forward for researchers and clinicians. Level V Descriptive studies.

The aim of this study was to develop functional composite edible films or coatings for fruit preservation by the addition of bioactive components in combinations that have not yet been thoroughly studied, according to the relevant literature. Edible films were initially composed of (i) chitosan (CH), cellulose nanocrystals (CNC) and beta-cyclodextrin (CD) (50%-37.5%-12.5% ratio), and (ii) hydroxypropyl methylcellulose (HPMC), cellulose nanocrystals (CNC) and beta-cyclodextrin (CD) (50%-37.5%-12.5% ratio). The bioactive components incorporated (5, 10 and 15% v/v) were as follows: (i) pomace oil-based nanoemulsion (NE) aiming to enhance barrier properties, and (ii) caffeine (C), aiming to enhance the antioxidant activity of films, respectively. Indeed, NE addition led to very high barrier properties (low oxygen and water vapor permeability), increased flexibility and reduced color. Furthermore, the contribution of these coatings to fresh strawberries’ preservation under cold storage was investigated, with very promising results concerning weight loss, color difference, and preservation of fruit moisture and quantity of O2 and CO2 inside the packages. Additionally, C addition led to very high antioxidant activity, reduced color and improved barrier properties. Finally, the contribution of these coatings to avocado’s preservation under cold storage was investigated, with very encouraging results for color difference, hardness and peroxide value of the fruit samples.

ABSTRACTMetastasis is a cancer-related systemic disease and is responsible for the greatest mortality rate among cancer patients. Interestingly, the interaction between the immune system and cancer cells seems to play a key role in metastasis formation in the target organ. However, this complex network is only partially understood. We previously found that IL-22 produced by tissue resident iNKT17 cells promotes cancer cell extravasation, the early step of metastasis. Based on these data, we aimed here to decipher the role of IL-22 in the last step of metastasis formation. We found that IL-22 levels were increased in established metastatic sites in both human and mouse. We also found that Th22 cells were the key source of IL-22 in established metastasis sites, and that deletion of IL-22 in CD4+ T cells was protective in liver metastasis formation. Accordingly, the administration of a murine IL-22 neutralizing antibody in the establishment of metastasis formation significantly reduced the metastatic burden in a mouse model. Mechanistically, IL-22-producing Th22 cells promoted angiogenesis in established metastasis sites. In conclusion, our findings highlight that IL-22 is equally as important in contributing to metastasis formation at late metastatic stages, and thus, identify it as a novel therapeutic target in established metastasis.

Background: Insufficient remnant liver volume (RLV) after the resection of hepatic malignancy could lead to liver failure and mortality. Portal vein ligation (PVL) prior to hepatectomy is subsequently introduced to increase the remnant liver volume and improve the outcome of hepatic malignancy. IL-22 has previously been reported to promote liver regeneration, while facilitating tumor development in the liver via Steap4 upregulation. Here we performed PVL in mouse models to study the role of IL-22 in liver regeneration post-PVL. Methods: Liver weight and volume was measured via magnetic resonance imaging (MRI). Immunohistochemistry for Ki67 and hepatocyte growth factor (HGF) was performed. IL-22 was analyzed by flow cytometry and quantitative polymerase chain reaction (qPCR) was used for acquisition of Il-33, Steap4, Fga, Fgb and Cebpd. To analyze signaling pathways, mice with deletion of STAT3 and a neutralizing antibody for IL-22 were used. Results: The remnant liver weight and volume increased over time after PVL. Additionally, we found that liver regenerative molecules, including Ki67 and HGF, were significantly increased in remnant liver at day 3 post-PVL, as well as IL-22. Administration of IL-22 neutralizing antibody could reduce Ki67 expression after PVL. The upregulation of IL-22 after PVL was mainly derived from innate cells. IL-22 blockade resulted in lower levels of IL-33 and Steap4 in the remnant liver, which was also the case in mice with deletion of STAT3, the main downstream signaling molecule of IL-22, in hepatocytes. Conclusion: IL-22 promotes liver regeneration after PVL. Thus, a combination of IL-22 supplementation and Steap4 blockade could potentially be applied as a novel therapeutic approach to boost liver regeneration without facilitating tumor progression after PVL.

Summary: Forced metastasis models, those in which the step of intravasation is bypassed, can be used to investigate the mechanisms underlying metastasis and evaluate potential therapeutic targets. Here, we present a protocol for using three forced models of lung and liver metastasis to generate metastasis within 3–4 weeks in approximately 99% of injected mice. We describe steps for cancer cell preparation, mouse analgesia and anesthesia; injecting through intrasplenic, intraportal, and intravenous techniques; and daily evaluation of metastasis.For complete details on the use and execution of this protocol, please refer to Giannou et al.1 : Publisher’s note: Undertaking any experimental protocol requires adherence to local institutional guidelines for laboratory safety and ethics.

Summary: To investigate underlying mechanisms for cancer metastasis and promising therapies in animal models, spontaneous metastasis models can be used to recreate metastasis development. Here, we present three mouse models of spontaneous lung and/or liver metastasis induction. We describe steps for cancer cell preparation, mouse analgesia, and three injection techniques (subcutaneous, intracecal, and intramucosal). We then detail procedures for evaluating metastasis. Most of these models generate metastasis in a time span of 4 weeks in the majority of injected mice.For complete details on the use and execution of this protocol, please refer to Giannou et al.1 : Publisher’s note: Undertaking any experimental protocol requires adherence to local institutional guidelines for laboratory safety and ethics.

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) which is responsible for eliciting Coronavirus disease 2019 (COVID-19) still challenges healthcare services worldwide. While many patients only suffer from mild symptoms, patients with some pre-existing medical conditions are at a higher risk for a detrimental course of disease. However, the underlying mechanisms determining disease course are only partially understood. One key factor influencing disease severity is described to be immune-mediated. In this report, we describe a post-mortem analysis of 45 individuals who died from SARS-CoV-2 infection. We could show that although sociodemographic factors and premedical conditions such as obesity and diabetes mellitus reduced survival time in our cohort, they were not associated with changes in the expression of immune-related signature genes at the RNA level in the blood, the gut, or the liver between these different groups. Our data indicate that obesity and diabetes mellitus influence SARS-CoV-2-related mortality, without influencing the extrapulmonary gene expression of immunity-related signature genes at the RNA level.

Abstract Background Health Research Capacity Building (HRCB) is key to improving research production among health workers in LMICs to inform related policies and reduce health disparities in conflict settings. However, few HRCB programmes are available in the MENA region, and few evaluations of HRCB globally are reported in the literature. Methods Through a qualitative longitudinal design, we evaluated the first implementation of the Center for Research and Education in the Ecology of War (CREEW) fellowship. Semi-structured interviews were conducted with fellows (n = 5) throughout the programme at key phases during their completion of courses and at each research phase. Additional data was collected from supervisors and peers of fellows at their organizations. Data were analysed using qualitative content analysis and presented under pre-identified themes. Results Despite the success of most fellows in learning on how to conduct research on AMR in conflict settings and completing the fellowship by producing research outputs, important challenges were identified. Results are categorized under predefined categories of (1) course delivery, (2) proposal development, (3) IRB application, (4) data collection, (5) data analysis, (6) manuscript write-up, (7) long-term effects, and (8) mentorship and networking. Conclusion The CREEW model, based on this evaluation, shows potential to be replicable and scalable to other contexts and other health-related topics. Detailed discussion and analysis are presented in the manuscript and synthesized recommendations are highlighted for future programmes to consider during the design, implementation, and evaluation of such programmes.

Background: Type 2 diabetes mellitus (T2DM) is often complicated by steatotic liver disease, cardiovascular disease (CVD), and extrahepatic cancer. We investigated whether FIB-4, an indicator of liver fibrosis, is associated with a higher risk of CVD and extrahepatic cancer history in T2DM. Methods: Two hundred and nine of 244 diabetics admitted to our center in one year were included and retrospectively evaluated. Results: One hundred and fifty-two (72.7%) were males and 57 (27.3%) females. The mean age and FIB-4 were 64.3 ± 11 years, and 1.15 ± 0.5, respectively. One hundred and fifty patients (71.8%) had FIB-4 ≤ 1.3, and 59 (28.2%) had FIB-4 > 1.3. A history of CVD was presented in 76 (36.4%) patients, and of extrahepatic cancer in 39 (18.7%). Patients with CVD were significantly older than those without (68.4 ± 8.5 vs. 63.2 ± 11.5 years; p = 0.002), with significantly higher FIB-4 (1.26 ± 0.5 vs. 1.08 ± 0.5; p = 0.012). Patients with cancer were older, with higher FIB-4 compared to those without (68.2 ± 9.5 vs. 64.4 ± 10.9 years; p = 0.098 and 1.37 ± 0.6 vs. 1.1 ± 0.5; p = 0.004, respectively). FIB-4 > 1.3 was associated with a 2.1-fold probability for CVD (χ2 = 5.810; p = 0.025) and 2.7-fold probability for cancer history (χ2 = 7.603; p = 0.01). Conclusions: FIB-4 ≥ 1.3 is associated with a higher probability of CVD or extrahepatic cancer history. FIB-4 could potentially discriminate patients at risk, justifying stricter surveillance.

Abstract The present editorial synopsises the benefits and challenges in meditative and non-meditative mindfulness practices and explores shorter and more creative approaches in mind–body interventions, emphasizing inclusivity and evidence-based practices. This collection, launched in BMC Complementary Medicine and Therapies, aims to bring together research on a variety of mindful practices, to discuss their role in supporting wellbeing.

Summary: The transplantation model provides the opportunity to assess the relevance of a molecule of interest for tumor cell extravasation by using a respective genetically modified donor animal. Here, we present a protocol for orthotopic single-lung transplantation in mice as a tool for lung metastasis studies. We describe steps for animal preparation, lung transplantation, and tumor cell injection. We then detail procedures for the direct comparison of tumor cell spreading between the genetically modified left lung and the naive right lung parenchyma.For complete details on the use and execution of this protocol, please refer to Giannou et al. (2023).1 : Publisher’s note: Undertaking any experimental protocol requires adherence to local institutional guidelines for laboratory safety and ethics.

BackgroundThe immune system plays a pivotal role in cancer progression. Interleukin 22 binding protein (IL-22BP), a natural antagonist of the cytokine interleukin 22 (IL-22) has been shown to control the progression of colorectal cancer (CRC). However, the role of IL-22BP in the process of metastasis formation remains unknown.MethodsWe used two different murine in vivo metastasis models using the MC38 and LLC cancer cell lines and studied lung and liver metastasis formation after intracaecal or intrasplenic injection of cancer cells. Furthermore, IL22BP expression was measured in a clinical cohort of CRC patients and correlated with metastatic tumor stages.ResultsOur data indicate that low levels of IL-22BP are associated with advanced (metastatic) tumor stages in colorectal cancer. Using two different murine in vivo models we show that IL-22BP indeed controls the progression of liver but not lung metastasis in mice.ConclusionsWe here demonstrate a crucial role of IL-22BP in controlling metastasis progression. Thus, IL-22 might represent a future therapeutic target against the progression of metastatic CRC.

Institutions of Higher Education (HEIs) faced great challenges, due to the COVID- crisis, on swiftly dealing with this unprecedented situation regarding the implementation of practical courses and interactive educational activities such as project-based courses. The aim of this work is to analyze the challenges and difficulties that arose through this process and the benefits that have emerged for both students and educators. For this reason, questionnaires were designed to study the pedagogical practices used for a project-based course, during the pandemic, and were distributed online to all students and supervisors enrolled in the course. The course entailed the design of an innovative food product, from the interactive analysis of different novel ideas to the production of a final product of high nutritional and ecological profile. Despite the concern that the enforcement of distance learning would significantly affect project-based courses, the majority of respondents confirmed that their institution adapted the curriculum successfully. They also declared satisfaction with the general format, and teaching procedures adopted, and agreed that the online modality can properly transmit educational content. Although, both faculty and students needed to adapt to the distance learning modality and become familiar with the use of new digital tools, they agreed that it can be very useful and provide benefits, when properly planned in advance and accompanied by the right technical support, equipment and class materials.

Meditative mindfulness practices, promoting sustained attention and reducing mind-wandering, have been associated with improvements in cognitive abilities and memory. The present study explored whether a non-meditative practice could be successfully applied in a forensic application; specifically, whether mindfulness instructions can be embedded in the face-composite construction process to facilitate identification. Twenty participants, who were not football fans, were asked to memorise an unfamiliar footballer's face and return 24 hr later to construct a face using the self-administered EvoFIT facial composite system. In the experimental condition, mindfulness instructions were embedded in the EvoFIT system, encouraging witnesses to focus on the target face and the process; in the control condition, participants constructed the face using the standard EvoFIT system. Naming of the composites was attempted by 24 football fans, who each viewed 10 composites, five from each condition, and then the target footballer images to ensure they were familiar with the identities. Results showed significantly higher levels of correct naming for composites constructed using EvoFIT with mindfulness instructions compared to using the standard EvoFIT. These findings indicate the potential for non-meditative mindfulness instructions to assist face-composite construction, improving correct naming of ensuing composites.

Coronavirus disease 2019 (COVID-19) is caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). This virus does not only lead to pulmonary infection but can also infect other organs such as the gut, the kidney, or the liver. Recent studies confirmed that severe cases of COVID-19 are often associated with liver damage and liver failure, as well as the systemic upregulation of pro-inflammatory cytokines such as tumor necrosis factor-alpha (TNFα). However, the impact these immune mediators in the liver have on patient survival during SARS-CoV-2 infection is currently unknown. Here, by performing a post-mortem analysis of 45 patients that died from a SARS-CoV-2 infection, we find that an increased expression of TNFA in the liver is associated with elevated mortality. Using publicly available single-cell sequencing datasets, we determined that Kupffer cells and monocytes are the main sources of this TNFα production. Further analysis revealed that TNFα signaling led to the upregulation of pro-inflammatory genes that are associated with an unfavorable outcome. Moreover, high levels of TNFA in the liver were associated with lower levels of interferon alpha and interferon beta. Thus, TNFα signaling in the infected SARS-CoV-2 liver correlates with reduced interferon levels and overall survival time.

A role of CD4+ T cells during the progression from nonalcoholic fatty liver disease (NAFLD) to nonalcoholic steatohepatitis (NASH) has been suggested, but which polarization state of these cells characterizes this progression and the development of fibrosis remain unclear. In addition, a gut-liver axis has been suggested to play a role in NASH, but the role of CD4+ T cells in this axis has just begun to be investigated. Combining single-cell RNA sequencing and multiple-parameter flow cytometry, we provide the first cell atlas to our knowledge focused on liver-infiltrating CD4+ T cells in patients with NAFLD and NASH, showing that NASH is characterized by a population of multicytokine-producing CD4+ T cells. Among these cells, only those with a Th17 polarization state were enriched in patients with advanced fibrosis. In parallel, we observed that Bacteroides appeared to be enriched in the intestine of NASH patients and to correlate with the frequency of multicytokine-producing CD4+ T cells. In short, we deliver a CD4+ T cell atlas of NAFLD and NASH, providing the rationale to target CD4+ T cells with a Th17 polarization state to block fibrosis development. Finally, our data offer an early indication to test whether multicytokine-producing CD4+ T cells are part of the gut-liver axis characterizing NASH.

The diet of the lionfish (Pterois miles), an invasive species in the Aegean Sea, was examined by collecting stomach content data from fish collected in three study sites in the Aegean Sea (southern Crete, Kastellorizo, and Nysiros islands). Prey composition in terms of numerical abundance and frequency of occurrence was used to compare lionfish’s diet between these sites. Lionfish largely preyed upon teleosts (4% to 83% numerical abundance and 16% to 58% frequency of occurrence, depending on the site) and decapods (12% to 95% numerical abundance and 11% to 81% frequency of occurrence). The most important teleost families in lionfish’s diet were Gobiidae, Labridae, and Scorpaenidae, while decapods and especially the family Scyllaridae and the genus Plesionika were the dominant decapod prey items. The lionfish was found to be an especially successful generalist across the study sites, an opportunistic, predatory species overall, and at the same time, at a local level, it seems to be an equally successful specialist that could increase the predation mortality of already stressed prey populations and can be a serious threat to endemic, critically endangered, and/or commercially important species.

Abstract Background Serious infections (SI) are common in patients with ANCA-associated vasculitides (AAV) like granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). Real-life data regarding their incidence and predisposing factors—after the introduction of B cell depleting agents—are limited while data quantifying the risk per treatment modality and year of the disease are missing. Here, we aim to describe in details the incidence and the risk factors for SI in a contemporary AAV cohort. Methods Multicenter, observational, retrospective study of AAV patients followed in three tertiary referral centers. Results We included 162 patients with GPA (63%) and MPA (37%), males 51.9%, mean age 60.9 years, ΑΝCA+ 86%, and generalized disease 80%. During follow-up (891.2 patient-years, mean 5.4 years), 67 SI were recorded in 50 patients at an incidence rate of 7.5 per 100 patient-years. The SI incidence rate was higher during induction with cyclophosphamide (CYC) compared to rituximab (RTX, 19.3 vs. 11.3 per 100 patient-years, respectively) while it was lower and comparable between RTX and other regimens (5.52 vs. 4.54 per 100 patient-years, respectively) in the maintenance phase. By multivariate analysis, plasmapheresis (PLEX) and/or dialysis was a strong predictor for an SI during the 1st year after diagnosis (OR = 3.16, 95% CI 1.001–9.96) and throughout the follow-up period (OR = 5.21, 95% CI 1.93–14.07). In contrast, a higher baseline BVAS (OR = 1.11, 95% CI 1.01–1.21) was associated with SI only during the 1st year. Conclusions In this real-life study of patients with AAV, the SI incidence was higher during CYC compared to RTX induction while there was no difference between RTX and other agents used for maintenance therapy. Higher disease activity at baseline and need for PLEX and/or dialysis were independent factors associated with an SI.

Germ cell tumors (GCTs) are a common malignancy in males, with variations in differentiation making different therapeutic strategies necessary. Generally, GCTs show good curation rates due to their good response to radiotherapy or chemotherapy. However, certain subtypes are resistant to these therapies and require surgery. We present a case of a 25-year-old patient with a large retroperitoneal GCT with somatic malignant transformation, where resection of large abdominal blood vessels with complex reconstruction was necessary to completely remove the tumor. The tumor was completely resected, and the patient has since been recurrence-free in the follow-up period. GCTs with somatic transformation show high resistance rates to chemo- and radiotherapy, and the patient in the presented case study did indeed show only a limited response to carboplatin-based chemotherapy. Patients suffering from these conditions should be resected whenever possible, as curation can be achieved by complete tumor resection. Infiltration of neighboring structures is no contraindication to surgery. The case presented here shows that interdisciplinary surgical planning including vascular and general surgeons as well as radiologists is vital to ensure successful tumor resection.

There is a well-established association between primary Sjögren’s syndrome and distal renal tubular acidosis (dRTA). dRTA is a relatively infrequent manifestation of primary Sjögren’s syndrome which can present with life-threatening electrolyte abnormalities while, in some patients, it could be the first manifestation of the syndrome. We report the case of a 35-year-old woman who presented with unexplained episodes of generalized weakness, severe hypokalemia, nephrocalcinosis, and normal anion gap metabolic acidosis. Subsequent evaluation revealed primary Sjögren’s syndrome as her underlying condition. The patient responded well to potassium supplementation, sodium bicarbonate, and oral prednisolone. After four years of follow-up, there were no other extraglandular manifestations, the renal function remained stable and the acidosis was partially improved without the need for oral bicarbonate. This case demonstrates that dRTA could be the initial manifestation of primary Sjögren’s syndrome and highlights the necessity for increased vigilance for patients presenting with persistent hypokalemia or nephrocalcinosis so that an early diagnosis can be made allowing for better control and prevention of disease progression.