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During the last 25 years of activity, the Physical Oceanography Research Group, previously known as the PO-Unit, and currently established within the Department of Geosciences of the University of Malta, has been promoting the downscaling of broad scope marine core services to higher resolution local scale domains for the Maltese Islands. Several services are delivered either by an intrinsic data elaboration or by making use of and integrating the COPERNICUS Marine Environment Monitoring Service (CMEMS) data to local marine data streams. Local observations are also integrated with higher resolution forecasts for the preparation and provision of dedicated services that address real specific needs of sub-regional and coastal users. This effort has yielded valuable climatological datasets covering the Maltese coastal waters and spanning over several years. This work focusses on the climatologies derived from numerical models and satellites, and compiled within the Interreg MED programme AMAre (Actions for Marine Protected Areas) project., peer-reviewed

El angiomiolipoma (AML) es una neoplasia clonal benigna compuesta por cantidades variables de tejido adiposo maduro, músculo liso y vasos sanguíneos de paredes gruesas. Aproximadamente un 20% de todos los AML, se detectan en pacientes con el Síndrome de la Esclerosis Tuberosa, trastorno autonómico dominante que se asocia a retraso mental, epilepsia, adenoma sebáceo. La presencia de hemorragia retroperitoneal masiva, conocida como Síndrome de Wunderlich se ha documentado en hasta un 10% de los pacientes y representa la complicación más importante y temida de estas lesiones. La TAC identifica las zonas del tejido adiposo, correspondiente a una baja atenuación entre 20-100 Hounsfield, dicho coeficiente dependerá del contenido y madurez de la grasa intratumoral y de la presencia o no de hemorragias. En el caso de pacientes con AML pequeños que requieren intervención debido a la presencia de síntomas, en pacientes con ET o AML multicéntricos y en pacientes en quienes la preservación de la función renal reviste particular importancia el enfoque preferido es la cirugía ahorradora de nefronas con nefrectomía parcial o embolizaciòn selectiva. Presentamos un caso de una paciente de 59 años con un angiomiolipoma gigante quién debuta con el Síndrome clínico conocido como Tríada de Lenk.

El angiomiolipoma (AML) es una neoplasia clonal benigna compuesta por cantidades variables de tejido adiposo maduro, músculo liso y vasos sanguíneos de paredes gruesas. Aproximadamente un 20% de todos los AML, se detectan en pacientes con el Síndrome de la Esclerosis Tuberosa, trastorno autonómico dominante que se asocia a retraso mental, epilepsia, adenoma sebáceo. La presencia de hemorragia retroperitoneal masiva, conocida como Síndrome de Wunderlich se ha documentado en hasta un 10% de los pacientes y representa la complicación más importante y temida de estas lesiones. La TAC identifica las zonas del tejido adiposo, correspondiente a una baja atenuación entre 20-100 Hounsfield, dicho coeficiente dependerá del contenido y madurez de la grasa intratumoral y de la presencia o no de hemorragias. En el caso de pacientes con AML pequeños que requieren intervención debido a la presencia de síntomas, en pacientes con ET o AML multicéntricos y en pacientes en quienes la preservación de la función renal reviste particular importancia el enfoque preferido es la cirugía ahorradora de nefronas con nefrectomía parcial o embolizaciòn selectiva. Presentamos un caso de una paciente de 59 años con un angiomiolipoma gigante quién debuta con el Síndrome clínico conocido como Tríada de Lenk. Angiomyolipoma (AML) is a benign clonal neoplasm that consists of varying amounts of mature adipose tissue, smooth muscle, and thick-walled vessels. Approximately 20% of AMLs are found in patients with tuberous sclerosis syndrome (TS), an autosomal-dominant disorder characterized by mental retardation, epilepsy, and adenoma sebaceous, a distinctive skin lesion. Massive retroperitoneal hemorrhage from AML, also known as Wunderlich's syndrome, has been found in up to 10% of patients and represents the most significant and feared complication. The presence of even a small amount of fat within a renal lesion on CT scan (confirmed by Hounsfield unit’s ≤10) is considered diagnostic of AML. Intervention should be considered for larger tumors, particularly if the patient is symptomatic, taking into account patient age, comorbidities, and other related factors. A nephron-sparing approach, via either partial nephrectomy or selective embolization, is clearly preferred. We present the case of a fifty-nine-year old patient with an angiomyolipoma and massive retroperitoneal hemorrhage with Lenk syndrome.

OBJECTIVE: To assess the recently proposed preliminary criteria for the classification of Sjogren9s syndrome (SS) in a multicentre European study of a new series of clinically defined cases. METHODS: The criteria included six items: I = ocular symptoms; II = oral symptoms; III = evidence of keratoconjunctivitis sicca; IV = focal sialoadenitis by minor salivary gland biopsy; V = instrumental evidence of salivary gland involvement; VI = presence of autoantibodies. Each centre was asked to provide five patients with primary SS, five with secondary SS, five with connective tissue diseases (CTD) but without SS, and five controls (patients with ocular or oral features that may simulate SS). The preliminary six item classification criteria set was applied to both the SS patients and the non-SS controls, and the performance of the criteria in terms of sensitivity and specificity was tested. RESULTS: The criteria set was tested on a total of 278 cases (157 SS patients and 121 non-SS controls) collected from 16 centres in 10 countries. At least four of the six items in the criteria set (limiting item VI to the presence of Ro(SS-A) or La(SS-B) antibodies) were present in 79 of 81 patients initially classified as having primary SS (sensitivity 97.5%), but in only seven of 121 non-SS controls (specificity 94.2%). When the presence of item I or II plus any two of items III-V of the criteria set was considered as indicative of secondary SS, 97.3% (71 of 73) of the patients initially defined as having this disorder and 91.8% (45 of 49) of the control patients with CTD without SS were correctly classified. CONCLUSION: This prospective study confirmed the high validity and reliability of the classification criteria for SS recently proposed by the European Community Study Group.

Objective-To assess the recently proposed preliminary criteria for the classification of Sjogren’s syndrome (SS) in a multicentre European study of a new series of clinically defined cases. Methods-The criteria included six items: I = ocular symptoms; II = oral symptoms; III = evidence of keratoconjunctivitis sicca; IV = focal sialoadenitis by minor salivary gland biopsy; V = instrumental evidence of salivary gland involvement; VI = presence of autoantibodies. Each centre was asked to provide five patients with primary SS, dive with secondary SS, five with connective tissue diseases (CTD) but without SS, and five controls (patients with ocular or oral features that may simulate SS). The preliminary six item classification criteria set was applied to both the SS patients and the non-SS controls, and the performance of the criteria in terms of sensitivity and specificity was tested. Results-The criteria set was tested on a total of 278 cases (157 SS patients and 121 non-SS controls) collected from 16 centres in 10 countries. At least four of the six items in the criteria set (Limiting item VI to the presence of Ro(SS-A) or La(SS-B) antibodies) were present in 79 of 81 patients initially classified as having primary SS (sensitivity 97.5%), but in only seven of 121 non-SS controls (specificity 94.2%). When the presence of item I or II plus any two of items III-V of the criteria set was considered as indicative of secondary SS, 97.3% (71 of 73) of the patients initially defined as having this disorder and 91.8% (45 of 49) of the control patients with CTD without SS were correctly classified. Conclusion-This prospective study confirmed the high validity and reliability of the classification criteria for SS recently proposed by the European Community Study Group.

We evaluated the 3-year drug survival and efficacy of the biosimilar SB4/Benepali in rheumatoid arthritis (RA), psoriatic arthritis (PsA) and ankylosing spondylitis (AS) patients, previously treated with etanercept (ETA). Drug survival rate was calculated using the Kaplan–Meier method and Cox proportional hazard models were developed to examine predictors of SB4 discontinuation. 236 patients (120 RA, 80 PsA and 36 AS), aged 60.7 ± 13.8 years and with an ETA duration of 4.1 ± 3.4 years were included. The 3-year retention rate for SB4 was 94.4%, 88% and 86% in AS, RA and PsA patients, respectively, with no difference between groups. Patients without comorbid disease had higher retention rates vs. patients with comorbid disease (90% vs. 60%, p < 0.0001). Disease activity, as measured by DAS28, DAPSA and BASDAI remained stable over the 3 years. Comorbid disease (hazard ratio; HR: 4.06, p < 0.0001) and HAQ at baseline (HR: 2.42, p = 0.0024) significantly increased the risk of SB4 discontinuation, while previous ETA duration was negatively associated with SB4 discontinuation (HR: 0.97, p = 0.0064). Forty-one (17.4%) patients left the study due to the interruption of the SB4 treatment, 31 (75.6%) discontinued due to inefficacy and 10 (24.4%) due to adverse events. This real-life study confirms the similar efficacy profile of ETA with long-term retention and a good safety profile in inflammatory arthritis patients.