Hospital de Santa Maria
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Oxaliplatin toxicity presenting as a liver nodule - case report
No full textBACKGROUND:
Oxaliplatin based chemotherapy is often used as adjuvant therapy in colon and rectal cancer. A reported side effect is Sinusoidal Obstruction Syndrome which is characterized by a spectrum of pathologic changes, from sinusoidal dilation, peri-sinusoidal haemorrhage, peliosis and nodular regenerative hyperplasia. Very rarely it can cause the development of liver nodules mimicking liver metastases. Herein, we report a case of Sinusoidal Obstruction Syndrome causing a liver nodule suspicious of liver metastasis on imaging. This is the third reported case of this complication of oxaliplatin toxicity, in which resection was performed and pathological diagnosis confirmed.
CASE PRESENTATION:
We report the case of a 59 year old man with stage III colon cancer who underwent sigmoidectomy followed by adjuvant chemotherapy with oxaliplatin. One year after surgery a liver nodule was detected and the patient underwent right hepatectomy. Pathology showed no liver nodule and diagnosed sinusoidal obstruction syndrome.
CONCLUSION:
We describe the third reported case of a liver lesion mimicking a liver metastasis after oxaliplatin-based chemotherapy for colon cancer. We suggest that in patients heavily treated with oxaliplatin with de novo liver nodules, this differential diagnosis should be considered. In particular, in this population of patients an intense imagiologic evaluation and even a preoperative biopsy should be pursued to confirm the diagnosis of malignancy and avoid overtreatment.info:eu-repo/semantics/publishedVersio
Challenges in rheumatic valvular disease: Surgical strategies for mitral valve preservation
Get PDFIn developing countries, rheumatic fever and carditis still constitutes a major public health problem. Patients have special characteristics that differ from those with rheumatic mitral valve disease we still see in developed countries. They are usually young, poor, uneducated, and have low compliance to prophylaxis / therapy. In addition, they usually have great difficulty in accessing medical care. In these situations, the rate of complications associated to valve replacement is significantly increased. Alternatively, mitral valve repair is now known to achieve better long-term results in this pathology, but this was not widely recognized three or four decades ago, when first reports showed worse results after repair of rheumatic regurgitation than with degenerative valves. This has been reported by several groups in developing countries in different continents, with high incidence of repairs and excellent long term results. It is, therefore, becoming increasingly clear that, although, the results may not compare to those obtained with degenerative pathology, repair of rheumatic valves, when feasible, is the procedure of choice, especially in these underprivileged populations.info:eu-repo/semantics/publishedVersio
[The Importance of Early Referral in Pediatric Acute Liver Failure]
Get PDFINTRODUCTION:
Acute liver failure is a rare disorder associated to high morbidity and mortality despite survival improvement through liver transplantation. The importance of a multidisciplinary approach and early referral to a pediatric liver transplantation center were important conclusions of a national meeting in 2008, from which resulted an actuation consensus.
OBJECTIVES:
To characterize acute liver failure admissions in a Pediatric Intensive Care Unit of the portuguese pediatric livertransplantation center. To compare results before (A) and after (B) 2008.
MATERIAL AND METHODS:
Observational, retrospective study during a 20 year period (1994-2014).
INCLUSION CRITERIA:
age < 18 years old and acute liver failure (INR ≥ 2 without vitamin K response and hepatocellular necrosis). Children with previous liver disease were excluded.
RESULTS:
Fifty children were included, with median age of 24.5 months. The most common etiology under 2 years old was metabolic (34.6%) and above that age was infectious (29.2%). Forty six percent were submitted to liver transplantation and 78% of them survived. Overall mortality was 34%. Median referral time was 7 days in period A (n = 35) and 2 days in period B (n = 15; p = 0.006). Pediatric risk of mortality's median was 14.7 in period A and 6.5 in B (p = 0.019). Mortality was 37% vs 26% in periods A and B, respectively (p = 0.474).
DISCUSSION AND CONCLUSIONS:
Overall mortality was similar to the observed in other European centers. Liver transplantation is in fact the most effective therapeutic option. After 2008, there was a reduction in referral time and cases severity on admission; however, mortality has not reduced so far.info:eu-repo/semantics/publishedVersio
Emerging drugs for Cushing's disease
Get PDFINTRODUCTION:
Considering the effects of uncontrolled hypercortisolism on morbidity and mortality, there is a clear need for effective medical therapy for patients with Cushing's disease (CD). Therefore, the search for new medical effective tools remains active, and already promising results have been obtained.
AREAS COVERED:
The importance of the design and conduct of trials to validate old drugs or to test new compounds is discussed. The results of the ongoing clinical trials, targeting the specific properties of drugs, such as ketoconazole, LCI699, mifepristone, etomidate and pasireotide, are also reported. The authors also emphasise the advantages and drawbacks of each particular drug, and the potential combined use of agents with complementary mechanisms of action.
EXPERT OPINION:
CD is an excellent example of a situation where effective therapy is essential, but where the balance of risk and benefit must be carefully judged. Metyrapone is the drug of choice when rapid control of the hypercortisolaemia is required, ketoconazole represents a good second-line drug, although in the future LCI699 may be a better alternative. Mifepristone can also be used in the rare situation when previous drugs are inappropriate. Etomidate is useful where immediate parenteral action is required. For drugs working directly on the pituitary, cabergoline is occasionally effective and pasireotide can be attempted in patients with mild CD.info:eu-repo/semantics/publishedVersio
Anti-MuSK-positive myasthenia gravis diagnosed during pregnancy: New challenges for an old disease
No full textMyasthenia gravis is an autoimmune disorder affecting predominantly women in their reproductive age. The course of the disease during pregnancy is unpredictable, although it is more difficult to manage earlier in the gestation. Myasthenia gravis with antibodies against the muscle-specific receptor tyrosine kinase (anti-MuSK) has been described as a subtype of disease with more localised clinical features and a poorer response to treatment than acetylcholine receptor antibody (anti-AChR)-positive patients. Few cases have been reported in pregnant women, with deliveries being performed mainly by caesarean section. We report a successful case of vaginal delivery and describe our experience providing the first review of the management of this subtype of disease during pregnancy
Post partial nephrectomy surveillance imaging: an evidence-based approach.
Get PDFTo ensure the early detection of recurrent disease, all patients should undergo routine surveillance following partial nephrectomy for renal cell carcinoma. In order to optimize resource allocation and avoid unnecessary radiation exposure, the frequency and duration of surveillance should be tailored to the individual patient's risk of cancer recurrence. The evidence for surveillance after partial nephrectomy is presented reviewing the current literature on prognostic models and proposed surveillance protocols based on the timing and patterns of renal cell carcinoma recurrence. In addition, we review recent guidelines on post partial nephrectomy surveillance as well as the literature on novel imaging techniques that may aid in early disease discovery
Neuroblastoma Perinatal – Um Desafio para o Neonatologista
Get PDFO neuroblastoma é o tumor maligno mais frequente no período neonatal. O diagnóstico pré-natal tem aumentado devido à
qualidade da ecografia obstétrica, que permite a deteção de formações de pequenas dimensões nas glândulas suprarrenais.
Apresentam-se os casos clínicos de quatro recém‑nascidos com o diagnóstico de neuroblastoma perinatal. Dois recém-nascidos
apresentaram imagens quísticas pré-natais na suprarrenal, estavam assintomáticos ao nascimento e o diagnóstico foi sugerido
pela vigilância imagiológica. Outro recém-nascido não apresentava alterações nas ecografias pré-natais mas, ao nascer, era
evidente distensão abdominal com massa palpável, cuja avaliação imagiológica sugeriu neuroblastoma. No quarto recém-
-nascido, o diagnóstico de neuroblastoma foi evocado na gravidez. Estes últimos dois recém-nascidos faleceram após início
de terapêutica imediata cirúrgica e/ou quimioterapia. As imagens quísticas da suprarrenal no período perinatal constituem
um desafio diagnóstico, devendo ser sempre considerada a hipótese de neuroblastoma, mesmo quando o recém-nascido está
assintomático
Síndrome do osso trígono
No full textA causa mais frequente da síndrome de conflito posterior do tornozelo envolve o osso trígono e o processo talar, chamando-se síndrome do osso trígono à compressão sintomática de tecidos moles e osso a nível do intervalo calcâneo-tibial. Trata-se de uma entidade controversa, atualmente ainda com evidência limitada na literatura. A presença do osso trígono é na maior parte dos casos apenas um achado imagiológico sem tradução clínica, no entanto o seu traumatismo, agudo ou crónico, em movimentos de flexão plantar forçada pode ser responsável pela sua lesão e conversão sintomática. O diagnóstico é feito pela clínica e pela evidência imagiológica e é frequentemente subdiagnosticado ou tem diagnóstico tardio. É necessário um nível elevado de suspeição para diagnóstico precoce, que é fundamental não só para iniciar rapidamente o tratamento adequado para alívio sintomático, como também em termos de prognóstico. O tratamento pode ser conservador ou cirúrgico, envolvendo a ressecção deste ossículo acessório, por técnicas de cirurgia aberta ou minimamente invasivas de artroscopia ou endoscopia.
O presente trabalho faz uma revisão da literatura científica existente sobre este tema. Introduzimos com a definição da síndrome, noções anatómicas e fisiopatológicas, seguindo-se a apresentação dos sinais e sintomas clínicos e dos meios complementares de diagnóstico e finalizando com as várias abordagens terapêuticas, tanto conservadora como cirúrgica. São descritas e comparadas entre si as três técnicas cirúrgicas mais praticadas atualmente, a cirurgia aberta, a artroscópica subtalar e a endoscópica
