Paget-Schröetter Syndrome, a rare cause of subclavian vein thrombosis.

Paget-Schröetter Syndrome, or effort thrombosis of the subclavian vein, is an uncommon condition that affects young, active, healthy individuals. It's a type of Thoracic Outlet Syndrome (TOS), consequence of compression and repetitive injury of the subclavian vein, between the first rib and the clavicle. This paper reports the case of a 27 year-old female presenting with an acutely swollen right upper limb, with no history of trauma or repeated movement of right arm, taking oral contraceptive pills and no history of venous thromboembolic disease. Duplex ultrasound evaluation confirmed right subclavian venous thrombosis with no evidence of cervical rib or other structural abnormalities, diagnosing Paget-Schröetter Syndrome. Following anticoagulation, catheter directed thrombolysis and balloon angioplasty were performed, with no success due to external compression. Therefore, after the correction of the external compression through first rib resection, the luminal flow was restored and the venous obstruction symptoms disappeared. In these case, a prompt treatment was critical for the achievement of the best results.

A Síndrome de Paget-Schröetter, ou trombose de esforço da veia subclávia, é uma entidade rara que surge em indivíduos jovens e saudáveis. É enquadrado na Síndrome do Desfiladeiro Torácico: sinais e sintomas resultantes da compressão e lesão repetida da veia subclávia, entre a primeira costela e a clavícula. Relata-se o caso clínico de uma jovem de 27 anos de idade com edema agudo do membro superior direito, sem história de trauma ou movimentos repetidos do braço direito, sem antecedentes de patologia tromboembólica venosa e medicada com contraceptivo oral. O ecodoppler revelou trombose da veia subclávia direita, sem evidência de costela cervical ou outras anomalias estruturais. Após o início de hipocoagulação, foi realizada venografia, com fibrinólise fármaco-mecânica e angioplastia com balão sem sucesso, devido a compressão extrínseca. Posteriormente, foi submetida a remoção trans-axilar da primeira costela, o que permitiu restabelecimento do fluxo venoso e desaparecimento dos sintomas de compressão venosa. Neste caso, um tratamento atempado foi crucial para o sucesso terapêutico.

Continuous Sedation in Palliative Care in Portugal: A Prospective Multicentric Study.

Objective: This study aimed to survey the practice of palliative sedation in Portugal, where data on this subject were lacking. Methods: This was a prospective multicentric study that included all patients admitted to each team that agreed to participate. Patients were followed until death, discharge, or after 3 months of follow-up. Results: The study included 8 teams: 4 as palliative care units (PCU), 1 as a hospital palliative care team (HPCT), 2 as home care (HC), and 1 as HPCT and HC. Of the 361 patients enrolled, 52% were male, the median age was 76 years, and 285 (79%) had cancer. Continuous sedation was undergone by 49 (14%) patients: 26 (53%) were male, and the median age was 76. Most patients, 46 (94%), had an oncological diagnosis. Only in a minority of cases, the family, 16 (33%), or the patient, 5 (10%), participated in the decision to sedate. Delirium was the most frequent symptom leading to sedation. The medication most used was midazolam (65%). In the multivariable analysis, only age and the combined score were independently associated with sedation; patients <76 years and those with higher levels of suffering had a higher probability of being sedated. Conclusions: The practice of continuous palliative sedation in Portugal is within the range reported in other studies. One particularly relevant point was the low participation of patients and their families in the decision-making process. Each team must have a deep discussion on this aspect.

Deprescription in Palliative Care.

Individuals with limited life expectancy represent a significant proportion of healthcare consumers and are usually patients with multiple diseases and high levels of frailty. Polypharmacy and the prescription of long lists of drugs are frequent in patients with reduced life expectancy and often, as the patient's health status deteriorates, the list of drugs increases substantially as new medications are introduced to address new symptoms or complications. A key priority for healthcare professionals managing the care of these patients should be balancing the pharmacological approach to chronic diseases with the palliation of acute symptoms and complications. An important element of this process is to ensure that the benefit of any prescription decision outweighs potential risks. We reviewed the pros and cons of deprescribing drugs in individuals with limited life expectancy, how to identify the expected disease trajectory, which drugs are to be discontinued, identified some models trying to achieve rigorous deprescribing criteria, and the psychosocial effects of deprescribing in late phases of life. Deprescribing is not a one-time event but rather a continuous process that requires ongoing evaluation and monitoring. It is vital to continuously monitor and evaluate the pharmacological and non-pharmacological prescriptions for patients with chronic illnesses to align them with their goals of care and life expectancy.

A Case of Tracheomalacia in Chronic Obstructive Pulmonary Disease: What Went Wrong?

Tracheobronchomalacia is defined as loss of the structural integrity of airway wall cartilaginous structures with hyperdynamic airway collapse during respiration. It is a common finding in chronic obstructive pulmonary disease (COPD) but is not always symptomatic, especially if airway narrowing is mild. Symptoms and signs develop as the severity of airway narrowing progresses. When a patient is symptomatic, a prompt study with computerized tomography and flexible bronchoscopy is mandatory for future management. We present a case of tracheobronchomalacia in a patient with COPD whose diagnosis and treatment were challenging. LEARNING POINTS: Tracheobroncomalacia is a common finding in chronic obstructive pulmonary disease patients; management of the obstructive disorder is the first treatment step.When patients remain symptomatic, non-invasive positive pressure ventilation can be applied; if there is no response, a stent trial or surgical procedure should be considered.It is a challenge to identify which patients should undergo invasive intervention or surgical management.

Visceral Leishmaniasis Associated with Macrophage Activation Syndrome and Diffuse Alveolar Hemorrhage in a Lupus Patient.

Systemic lupus erythematosus is a heterogeneous and unpredictable autoimmune disease which can be complicated to approach and treat. Hemophagocytic lymphohistiocytosis and diffuse alveolar hemorrhage are rare disease complications. The authors describe a clinical case of a 32-year-old woman with lupus and fever of unknown origin. From the investigations performed, the myelogram revealed hemophagocytosis and Leishmania parasites, therefore liposomal amphotericin B was then started. In addition to directed therapy, she maintained fever that evolved with diffuse alveolar hemorrhage. The myelogram was repeated and showed that she still had hemophagocytosis but now without parasites. Corticotherapy was increased and intravenous Immunoglobulin was started, with improvement. Rituximab was started as a result of macrophage activation syndrome and diffuse alveolar hemorrhage. Months after discharge, she began once again to have sustained fever and Leishmania parasites were found again, therefore liposomal amphotericin B was started once more associated with miltefosine. She continues being followed-up as she is asymptomatic and using steroidsin weaning scheme.

O lúpus eritematoso sistémico é uma doença autoimune heterogénea e imprevisível, o que pode complicar a sua abordagem e tratamento. A linfohistiocitose hemofagocítica e a hemorragia alveolar difusa são complicações raras da doença. Os autores descrevem o caso de uma mulher de 32 anos, com lúpus e febre de origem indeterminada. Da investigação realizada, o mielograma revelou hemofagocitose e parasitas de Leishmania, pelo que iniciou anfotericina B lipossomal. Manteve febre apesar da terapêutica dirigida e evoluiu com hemorragia alveolar difusa. Repetiu mielograma, mantendo hemofagocitose já sem parasitas, tendo aumentado corticoterapia e iniciado imunoglobulina com melhoria. Dada a presença de síndrome de activação macrofágica e hemorragia alveolar difusa iniciou rituximab. Meses após a alta hospitalar, iniciou novamente febre sustentada e foram novamente identificados parasitas de Leishmania, pelo que reiniciou anfotericina B lipossomal associada a miltefosina. Mantém follow-up, encontrando-se assintomática e com corticóides em esquema de desmame.

Light-Chain Deposition Disease with Prominent Hepatic Involvement.

Light-chain deposition disease (LCDD) is a rare monoclonal gammopathy that involves the deposition of light chains (LC) in multiple organs, leading to progressive dysfunction. The kidney is usually the most affected organ and responsible for the initial clinical manifestations. We present the case of a patient with LCDD with prominent liver involvement (marked cholestasis, hepatomegaly and portal hypertension) but with no evidence of coexisting lymphoproliferative disorder. LEARNING POINTS: Light-chain deposition disease (LCDD) is rare, and diagnosis can be challenging, especially in the absence of a coexistent lymphoproliferative disorder. The presence of light chains in blood or urine can be intermittent, and immunohistochemistry results of biopsy can be difficult to interpret. Hence, there is a need for a high level of suspicion in this diagnosis.Although renal involvement is the most classic presentation, LCDD should be suspected when there is multiple organ involvement, as in this case, even when renal manifestations do not dominate the clinical picture.Prognosis depends on the affected organs, and treatment should be started as rapidly as possible to suppress production of the clonal light-chain and halt organ damage.