Calciphylaxis is a rare condition characterized by the emergence of non-healing skin ulcers secondary to arterial calcification and thrombosis, typically diagnosed in patients with end-stage kidney disease (ESKD). When it develops in patients without ESKD, it is called non-uremic calciphylaxis (NUC). The latter is an even rarer diagnosis with an uncertain pathophysiology and a high mortality rate (52%), mainly due to sepsis (50%). Cutaneous biopsy is diagnostic. Therapeutic measures recommended for NUC are limited to wound debridement, analgesia, and control of infection and risk factors. Other therapeutic options exist but with a low level of evidence. We present the case of a 78-year-old woman with NUC in her lower limbs who died of sepsis. NUC is a therapeutic challenge lacking efficient strategies.LEARNING POINTSCalciphylaxis in the absence of end-stage kidney disease is called non-uremic calciphylaxis (NUC).This disease is a diagnostic and therapeutic challenge.As therapeutic strategies for NUC mainly derive from those for uremic calciphylaxis, more efficient therapeutic measures and evidence-based recommendations are needed.

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Sometimes, the presentation of some diseases can be fulminating. The authors present the case of a 51-year- old male brought to the emergency department visibly drunk and complaining of abdominal pain. Immediately, the diagnosis of hemorrhagic shock due to an accentuated drop of the hemoglobin level was made. After stabilization, he underwent a computed tomography of the abdomen, revealing a hepatocellular carcinoma with rupture of the Glisson capsule and massive intraperitoneal hemorrhage. With this case, the authors want to bring attention to a rare first presentation of hepatocellular carcinoma with a catastrophic result.

The incidence of thyroid cancer (TC) is increasing. Although the majority of these cancers have a good prognosis, 10% of these will develop local recurrence and/or distant metastases. Conventional cytotoxic chemotherapy has been largely replaced by molecular-target therapies, but it can still have a role. Two tyrosine kinase inhibitors have been approved for the treatment of advanced differentiated TC. They significantly improve progression-free survival, but at the cost of frequent and potentially serious adverse effects. At the moment, there are multiple clinical trials with other tyrosine kinase inhibitors and other drugs. We present a review of the current standard of care and what is up to come in the treatment of advanced TC.

Classicamente a hepatite viral está associada ao vírus dahepatite A, B ou C, podendo ser também causada por outrosvírus. Apesar de não ser endémica em Portugal, reporta-seo caso esporádico de uma doente com hepatite aguda, cujodiagnóstico se confirmou hepatite E. Os autores chamam aatenção para uma causa rara de hepatite, devendo ser umdiagnóstico a considerar após exclusão de causas mais frequentes.

BackgroundDifferentiated thyroid cancer (DTC) is the most common endocrine cancer during childhood, and the prognosis is usually good. The 2015 American Thyroid Association (ATA) pediatric guidelines for DTC classify patients into three categories (low, intermediate, and high) that represent the risk for persistent/recurrent disease. The "Dynamic Risk Stratification" (DRS) System showed that, in adults, reassessment of disease status during follow-up was a better predictor of disease status at the end of followup when compared to ATA risk stratification. This system is still not validated for the pediatric population with DTC. Our aim was to evaluate the usefulness of the DRS system in predicting DTC disease behaviour in this specific population. We also aimed to evaluate potential clinical-pathological factors associated with persistent disease at the end of follow-up. MethodsA retrospective analysis of 39 pediatric patients (≤18 years) with DTC was conducted in our institution between 2007 and 2018, including 33 patients who had follow-up ≥ 12 months; these were classified into ATA risk groups and re-stratified according to their response to treatment at 12-24 months of follow-up. The associations between the ordinal variables of the baseline ATA risk group and the disease status re-evaluated 12-24 months after diagnosis (as per the DRS system) and at the end of follow-up were evaluated using a linear-by-linear association test. Gender, age at diagnosis, tumor size, multicentricity, extrathyroid extension, vascular invasion, lymph node metastasis, distant metastasis, and stimulated thyroglobulin (sTg) during the first RAI administration were evaluated as potential factors associated with persistent disease at 27 months after diagnosis using Firth's bias-reduced penalized-likelihood logistic regression. ResultsIn this study, 39 patients were retrospectively analyzed, including 33 patients who had follow-ups ≥ 12 months with a median time of 56 (27-139) months who were classified in ATA risk groups and then restratified depending on their response to treatment between 12 and 24 months of follow-up. There was a statistically significant association between ATA risk groups and re-evaluation at 12 and 24 months (p=0.001) and between these two stratifications and the state of disease at final follow-up (p<0.001 for both). Factors with a statistically significant association with persistent disease at 27 months of follow-up were male sex, lymph node metastases at diagnosis, distant metastasis, extrathyroidal extension, and stimulated Tg values. ConclusionsThe assessment of the response to treatment between 12 and 24 months and at the end of follow-up refines the initial ATA risk stratification, confirming that dynamic risk evaluation is also helpful in the pediatric population.

proteinuria (27.3%), stomatitis (11.4%). Nine (20.5%) pts required treatment discontinuation and reasons were due to death, PD and AE in 5 (11.4%), 2 (4.5%) and 2 (4.5%) pts, respectively. Five deaths occurred. Dose reductions to 20, 18, 14 and 10 mg were in 10 (22.7%), 3 (6.7%), 7 (15.9%) and 7 (15.9%) pts, respectively; 16 (36.4%) pts did not require dose reduction.Conclusions: 61,4 % ORR shows good efficacy of lenvatinib in population of pts with poor performance status (ECOG 1-2).Legal entity responsible for the study: The authors.