Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia With Progression to Neuroendocrine Tumor.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a parenchymal lung disease characterized by a proliferation of neuroendocrine cells in the bronchial wall, with possible local invasion and occasional development of tumorlets. It is considered to be a precursor lesion as it can progress to neuroendocrine tumors (NETs). At presentation, approximately one-half of patients with DIPNECH have a synchronous diagnosis of NET. Here, we present the case of a 95-year-old woman with progressive exertional dyspnea. She was found to have an obstructive airway syndrome and long-lasting progressive bilateral pulmonary nodules, with a distribution and growth pattern suggestive of DIPNECH, as well as possible progression to NET in the larger lesions. A transthoracic needle aspiration biopsy of a pulmonary nodule was performed, confirming the diagnosis of NET, evolving from DIPNECH.

Levosimendan In Single Ventricle Heart Failure After Longterm Survival Of A Modified Blalock-Taussig Shunt.

We report the case of a 44 year-old patient with complex ACHD, admitted with acute decompensated heart failure (ADHF) in hemodynamic profile B. He had a single ventricle with pulmonary atresia, previously submitted to three modified Blalock-Taussig shunts (mBTs) at the age of 2, 12 and 19 years old. Despite conventional treatment with diuretics, ß-blockers (BB) and isosorbide dinitrate the patient progressed to profile C and the transthoracic echocardiogram disclosed a reduced systolic function. Likewise, levosimendan was commenced and an appropriate decongestion and a marked reduction in the NT-proBNP were seen. Treatment with angiotensin-converting-enzyme inhibitor, BB, ivabradine and mineralocorticoid receptor was optimized. The patient was discharged home after 26 days in NYHA class III and referred for heart transplant after right heart catheterization. To our knowledge, this is the first report of successful levosimendan's use in ADHF in a mBTs long-term survivor.

Mediastinum Teratoma Rupture: A Rare Manifestation.

We present the case of a 22-year-old man who presented with cough, haemoptysis and fever of 3 days' duration. A teratoma had been diagnosed 2 years previously. Physical examination was unremarkable but laboratory tests showed anaemia, neutrophilic leucocytosis and an increase in C-reactive protein. Chest CT revealed a teratoma of the anterior mediastinum with post-obstructive pneumonitis suggestive of tumour rupture. Antibiotic treatment resulted in a good clinical outcome. The patient was submitted to a left upper lobectomy and pathological examination revealed a mature teratoma. Teratomas are germ cell tumours that are usually asymptomatic and their rupture is a rare event. LEARNING POINTS: Mature teratomas are usually asymptomatic.Teratoma bronchial rupture is rare.The most frequent location for mature teratomas is the anterior mediastinum.

Pernicious Anaemia with Normal Vitamin B12.

A 49-year-old female patient presented to our hospital with asthenia, odynophagia, low grade fever, worsening symptoms of chronic depression, and symmetric leg paresthesias. Investigations showed macrocytic anaemia, leucopenia, thrombocytopenia, high lactate dehydrogenase levels and a normal Coombs test. Trilineage dysplasia was detected in the bone marrow biopsy specimen. The diagnostic work-up led us to the diagnosis of pernicious anaemia with a spuriously normal value of vitamin B12 and high titres of anti-intrinsic factor autoantibodies. This case highlights the importance of considering vitamin B12 deficiency in the differential diagnosis of myelodysplasia, even when vitamin B12 levels seem to be normal. LEARNING POINTS: Vitamin B12 deficiency is characterized by neuropsychiatric manifestations and bone marrow failure with accompanying dysplastic changes.Spuriously normal vitamin B12 levels can occur in pernicious anaemia due to anti-intrinsic factor autoantibody interference in the laboratory assay.Myelodysplastic syndromes and vitamin B12 deficiency share clinical and laboratory similarities, so a correct differential diagnosis is crucial for adequate treatment.

Behçet Syndrome and Crohn's Disease: What Are the Differences?

Behçet syndrome (BS) is a variable vessel vasculitis that has pleiotropic manifestations. A 43-year-old male with a previous diagnosis of Crohn's disease (CD) presented with deep venous thrombosis and bilateral superficial femoral artery aneurysms. A diagnosis of BS was made, and the patient was treated aggressively with immunosuppressive therapy and bilateral bypass surgery, attaining a favourable outcome. CD has many features that overlap with BS, and it may be challenging to distinguish between these two conditions, as our case illustrates. Nonetheless, the combination of venous thrombosis and arterial aneurysms should point the clinician towards a diagnosis of BS. LEARNING POINTS: Behçet syndrome is a variable vessel vasculitis of unknown aetiology that has pleiotropic manifestations.Crohn's disease has many overlapping features with Behçet syndrome, namely gastrointestinal, cutaneous, articular, ocular and cardiac manifestations.The combination of venous thrombosis and arterial aneurysms should point the clinician towards a diagnosis of Behçet syndrome.

Neurological Manifestations of IgG4-Related Disease.

OPINION STATEMENT: IgG4-related disease (IgG4-RD) is a multisystem inflammatory disorder. Early recognition of IgG4-RD is important to avoid permanent organ dysfunction and disability. Neurological involvement by IgG4-RD is relatively uncommon, but well recognised-hypertrophic pachymeningitis and hypophysitis are the most frequent manifestations. Although the nervous system may be involved in isolation, this more frequently occurs in conjunction with involvement of other systems. Elevated circulating levels of IgG4 are suggestive of the condition, but these are not pathognomonic and exclusion of other inflammatory disorders including vasculitis is required. Wherever possible, a tissue diagnosis should be established. The characteristic histopathological changes include a lymphoplasmacytoid infiltrate, storiform fibrosis and obliterative phlebitis. IgG4-RD typically responds well to treatment with glucocorticoids, although relapse is relatively common and treatment with a steroid-sparing agent or rituximab may be required. Improved understanding of the pathogenesis of IgG4-RD is likely to lead to the development of more specific disease treatments in the future.

[Career Satisfaction of Medical Residents in Portugal]. / Satisfação com a Especialidade entre os Internos da Formação Específica em Portugal.

INTRODUCTION: The satisfaction with the medical profession has been identified as an essential factor for the quality of care, the wellbeing of patients and the healthcare systems' stability. Recent studies have emphasized a growing discontent of physicians, mainly as a result of changes in labor relations. OBJECTIVES: To assess the perception of Portuguese medical residents about: correspondence of residency with previous expectations; degree of satisfaction with the specialty, profession and place of training; reasons for dissatisfaction; opinion regarding clinical practice in Portugal and emigration intents. MATERIAL AND METHODS: Cross-sectional study. Data collection was conducted through the "Satisfaction with Specialization Survey", created in an online platform, designed for this purpose, between May and August 2014. RESULTS: From a total population of 5788 medical residents, 804 (12.25 %) responses were obtained. From this sample, 77% of the responses were from residents in the first three years. Results showed that 90% of the residents are satisfied with their specialty, 85% with the medical profession and 86% with their place of training. Nevertheless, results showed a decrease in satisfaction over the final years of residency. The overall assessment of the clinical practice scenario in Portugal was negative and 65% of residents have plans to emigrate after completing their residency. CONCLUSION: Portuguese residents revealed high satisfaction levels regarding their profession. However, their views on Portuguese clinical practice and the results concerning the intent to emigrate highlight the need to take steps to reverse this scenario.

Introdução: A satisfação com a profissão médica tem sido apontada como um fator essencial para a qualidade assistencial, o bemestar dos doentes e a estabilidade dos sistemas de saúde. Estudos recentes têm vindo a enfatizar um crescente descontentamento dos médicos, principalmente como consequência das alterações das relações laborais.Objetivos: Avaliar a perceção dos médicos de formação específica em Portugal, sobre as expectativas e grau de satisfação com a profissão, especialidade e local de formação; razões da insatisfação e intenção de emigrar.Material e Métodos: Estudo transversal. A colheita de dados foi efetuada entre Maio e Agosto de 2014 através de um Inquérito online sobre a âÄúSatisfação com a EspecialidadeâÄù.Resultados: De uma população total de 5788 médicos, foram obtidas 804 respostas (12,25% do total de médicos internos). Desta amostra, 77% das respostas correspondem a internos dos três primeiros anos de formação. Verificou-se que 90% dos médicos se encontram satisfeitos com a especialidade, tendo-se encontrado também níveis elevados de satisfação com a profissão (85%) e local de formação (86%). Por outro lado, constatou-se que estes diminuíam com a progressão ao longo dos anos de internato. A avaliação global sobre o panorama da prática médica foi negativa e 65% dos médicos responderam que consideram emigrar após conclusão do internato.Conclusão: Os médicos internos em Portugal apresentam níveis positivos de satisfação com a sua profissão. No entanto, a sua opinião sobre o panorama da Medicina e os resultados relativos à intenção de emigrar alertam para a necessidade de tomada de medidas para inverter este cenário.