A rare case of cerebellar degeneration due to primary Sjogren's syndrome.

Sjogren's syndrome (SS) is an autoimmune pathology which mainly affects salivary and lacrimal glands. Cerebellar degeneration association with SS is very rare, with only a few cases described. The treatment of SS with central nervous system involvement is not consensual. We present a 48-year-old woman with dysarthria, diplopia and ataxia associated with xerostomia. The brain magnetic imaging revealed bilateral cerebellar atrophy. She had antibody positivity for anti-SSA and anti-SSB and minor salivary glands biopsy revealed lymphocytic infiltration. Methylprednisolone, cyclophosphamide and intravenous immunoglobulin yielded no results. Rituximab was initiated with improvement in dysarthria and coordination.

An unusual case of osteoarthritis?

Forestier disease is a condition characterized by calcification and ossification of ligaments and entheses particularly affecting axial skeleton. Diagnosis is difficult and mandates a high suspicion level, but unexpensive and accessible examinations like a simple radiography might provide useful diagnostic clues in these challenging clinical scenarios and improve clinical assistance.

Is it just an obstructive pyelonephritis?

Pyelocaliceal obstruction is a diagnostic challenge, and it is important to identify the obstruction cause. Some patients present extra-renal compressive masses that need further imagiologic investigation and a biopsy, to establish the diagnosis.

A case report of idiopathic Hemophagocytic lymphohistiocytosis in an immunocompetent adult.

Hemophagocytic lymphohistiocytosis poses a diagnostic dilemma due to the absence of specific clinical and laboratory findings, especially in adults. Despite greater recognition of the disease, secondary idiopathic forms are still reported.

Is it just a simple pneumonia?

Postobstructive pneumonia is a pulmonary infiltrate distal to a bronchial obstruction. It presents a diagnostic challenge, and full attention should be on recognition of this entity, identify, and treating the obstructive lesion and secondary infection. In adults, malignancy is the main cause; however, others etiologies must be investigated.

Not Always (and Only) Heart Failure-A Case Report of Primary Pleural Lymphoma in an Elderly Patient.

Pleural involvement in Non-Hodgkin Lymphoma (NHL) is well documented, but primary pleural lymphomas are extremely rare, occurring mostly in immunosuppressed patients or associated with chronic pleural inflammation. Nevertheless, the pathogenesis and therapeutic approaches to counteract primary pleural lymphomas are still matter of debate. The authors present the clinical case of an 81-year-old female with respiratory and constitutional symptoms. A valvular heart disease and bilateral pleural effusion were known. The study carried out showed a large right pleural effusion; the fluid analysis was compatible with Diffuse Large B-cell Lymphoma (DLBCL), and two lymphomatous masses with pleural origin were found at the ipsilateral hemithorax. Primary pleural lymphoma was considered and chemotherapy was initiated with a good response and evolution. The authors report this remarkable clinical case because of its rarity, its excellent clinical evolution and the absence of an immunodeficiency context.

The Use of Thalidomide in Severe Refractory Anaemia Due to Gastric Antral Vascular Ectasia (GAVE) in Cirrhosis?

Gastric antral vascular ectasia (GAVE) is a rare cause of upper gastrointestinal bleeding associated with cirrhosis. The first-line treatment is endoscopic therapy with argon plasma coagulation (APC). There is a high recurrence rate, but some evidence suggests that thalidomide could play an important role in controlling refractory anaemia due to GAVE. The authors present the case of a cirrhotic patient with a recent diagnosis of GAVE, who underwent multiple endoscopic treatments and blood transfusions because of haematemesis. The patient started thalidomide and 6 months later, there was no recurrence of haematemesis and haemoglobin levels were stable, with no reported adverse effects. LEARNING POINTS: The chronic bleeding associated with gastric antral vascular ectasia (GAVE) presenting with cirrhosis is more severe than that associated with portal hypertensive gastropathy.Argon plasma coagulation remains the first-line treatment, but GAVE has a high recurrence rate.Thalidomide could be an effective and safe option for recurrent bleeding due to GAVE in patients with cirrhosis.

Hard-To-Treat Idiopathic Refractory Autoimmune Haemolytic Anaemia with Reticulocytopenia.

Autoimmune haemolytic anaemia (AIHA) is an uncommon condition characterized by increased destruction of erythrocytes associated with reticulocytosis in the great majority of cases. We present the case of a 68-year-old woman with jaundice and malaise. Investigation revealed AIHA with reticulocytopenia. The patient failed to respond to prednisolone or to rituximab. Azathioprine and epoetin beta were subsequently started, the prednisolone dose was increased, and the patient began to respond after 1 month. In AIHA, reticulocytopenia is a very rare presentation and a sign of great severity and poor outcome. The scarcity of therapeutic options in refractory cases poses a major challenge for physicians. LEARNING POINTS: Autoimmune haemolytic anaemia is a rare disorder characterized by decompensated acquired haemolysis caused by the host's immune system acting against its own red cell antigens.Concomitant presentation with reticulocytopenia is very rare and a sign of great severity and poor outcome.Treatment options in refractory cases still greatly rely on individual experience and expert opinion.

An Unexpected Favourable Evolution of Advanced Stage Cirrhosis.

The elimination of the aetiological factors causing liver injury is an important cornerstone in preventing progression and increasing survival in patients with cirrhosis. The authors present the case of a 63-year-old woman with a history of long-term alcohol abuse and consequent liver cirrhosis. Over the years, the patient presented progressive deterioration with severe malnutrition and had multiple hospital admissions due to decompensated cirrhosis, including refractory ascites, variceal bleeding and an extensive portal vein thrombosis (PVT). Anticoagulant therapy was not initiated due to a high risk of variceal bleeding. She eventually became abstinent, but PVT precluded a liver transplant. Over the following 10 years, her performance status gradually improved, with no new decompensation episodes and liver function normalization, although refractory ascites persisted. Abdominal CT showed spontaneous recanalization of the portal vein and a transjugular intrahepatic portosystemic shunt (TIPS) procedure was performed with gradual improvement of ascites. In this atypical case, an unexpected favourable evolution of advanced stage cirrhosis was observed with long-term improvement in clinical status and liver function, resulting in an estimated 10-year cumulative mortality rate of 99.98% and highlighting the importance of abstinence. Unexpectedly, spontaneous complete repermeabilization of the PVT was also observed, despite its extent and the absence of anticoagulation therapy. LEARNING POINTS: The elimination of the aetiological factors causing liver injury is the cornerstone in preventing decompensation and improving prognosis in patients with cirrhosis.Spontaneous complete recanalization of the portal vein may occur.A transjugular intrahepatic portosystemic shunt (TIPS) is effective in managing some of the major complications of portal hypertension, such as refractory ascites, and provides secondary prophylaxis of oesophageal variceal haemorrhage.

An Acute Blue Finger: A Case of Achenbach's Syndrome.

Achenbach's syndrome, also known as paroxysmal finger haematoma, is a rare condition that results in spontaneous bruising and pain in one or more fingers. Despite its benign and self-limiting course, the remarkable clinical presentation can suggest serious vascular and haematological disease leading to unnecessary referrals and invasive investigations. The authors present the case of a 60-year-old woman with an acute painful and bruised finger. All other physical findings and investigations were normal, except for autoimmune thyroiditis. Based on the clinical presentation and course, the diagnosis of Achenbach's syndrome was made and the symptoms resolved without treatment. LEARNING POINTS: Achenbach's syndrome is a rare and benign condition characterized by recurrent episodes of sudden pain, bruising and swelling of one or more fingers.It is a self-limiting condition and the diagnosis is essentially based on history and clinical examination.It is important to recognize this rare syndrome in order to address patient concerns and avoid unnecessary referrals or invasive investigations.

[Fiability Study of Diabetes Empowerment Scale: Short Version]. / Estudo de Fiabilidade da Escala de Capacidade de Controlo da Diabetes: Versão Breve.

PURPOSE: To compare the final score of the scale to the levels of HbA1c. MATERIAL AND METHODS: Cross-sectional observational study by applying the Diabetes Empowerment Scale-Short Form to diabetics at three primary care units in the central region of Portugal. The test-retest (in writing first and, five minutes later, orally) was performed to access Cronbach's alpha in 20 patients not studied in the next phase. Then, the scale was applied to diabetic patients after nursing consultation and prior to entering the medical consultation. Descriptive and inferential statistics after checking for the normality of the data were performed. RESULTS: In the first phase Cronbach's alpha was 0.90 to 1.00 in all of eight scale items. The average result obtained in the written phase was 3.78 ± 0.71 and in the oral 3.79 ± 0.64, p = 0.629. The sample of the second stage was of 81 diabetic patients, 55.6% male. Sample's mean age was 68.5 ± 1.1 years, mean HbA1c of 6.8 ± 0.2 and mean time from diagnosis of 9.2 ± 0.9 years. The average final score of the scale was 4.1± 0.8. There was significant correlation between the final score and HbA1c levels (p= -0.114; p = 0.312). CONCLUSION: The Portuguese version of the Diabetes Empowerment Scale-Short Form proved to be a reliable scale to measure empowerment in diabetic patients in Portugal. It was evident the presence of a statistically significant correlation between the results obtained at the end of the scale and HbA1c.

Objetivo: Avaliar a correlação entre o valor obtido pelo instrumento de medição Diabetes Empowerment Scale - Short Form e o controlo da pessoa com diabetes medido pelo valor da hemoglobina glicada A1c.Material e Métodos: Estudo observacional transversal pela aplicação do Diabetes Empowerment Scale - Short Form a pessoas com diabetes de três Unidades de Saúde Familiar da Região Centro de Portugal após realização de teste e reteste (primeiro por escrito e, passados cinco minutos, oralmente) para determinação da coerência interna através do valor de alfa de Cronbach em 20 elementos que não foram depois estudados. A aplicação a pacientes diabéticos foi feita após a consulta de enfermagem e antes da entrada na consulta médica. Foi realizada estatística descritiva e inferencial apos verificação da normalidade dos dados.Resultados: Na primeira fase o valor de alfa de Cronbach de 0,90 a 1,00 relativamente aos oito itens da escala. Na aplicação escrita, a média de resultados foi de 3,78 ± 0,71 e na aplicação oral de 3,79 ± 0,65, p = 0,629. A amostra da segunda fase foi de 81 pessoas com diabetes, sendo 55,6% do sexo masculino. A idade média foi de 68,5 ± 1,1 anos com uma HbA1c média de 6,8 ± 0,2 e um tempo de evolução desde o diagnóstico de 9,2 ± 0,9 anos. A média da pontuação final da escala foi de 4,1 ± 0,8. Verificou-se uma correlação significativa entre a pontuação final e os níveis de Hba1C (ÏÅ = -0,114; p = 0,312).Conclusão: A Escala de Capacidade de Controlo da Diabetes âÄì Versão Breve revelou ser uma escala fiável para medir a capacitação em doentes diabéticos em Portugal. Confirmou-se a presença de uma correlação estatisticamente significativa entre o resultado obtido no final da escala e o valor de HbA1c.