Browsing by Author "Carvalho, S"
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- Análise económica do rituximab, em associação com ciclofosfamida, vincristina e prednisolona no tratamento de doentes com linfoma folicular avançado em PortugalPublication . Braga, P; Carvalho, S; Gomes, M; Guerra, L; Lúcio, P; Marques, H; Negreiro, F; Pereira, C; Silva, C; Teixeira, AOBJECTIVE: Evaluate costs and benefits of rituximab in combination with cyclophosphamide/vincristine/prednisolone chemotherapy regimen (R-CVP), in previously untreated patients with indolent non-Hodgkin lymphoma (NHL), compared to CVP alone from a Portuguese National Health System (NHS) perspective. METHODS: Cost-effectiveness (Life Years Gained--LYG) and cost-utility analysis (Quality Adjusted Life Years--QALYs) were performed for a time horizon of 10 years, according to a Markov economic model with three health states (progression free survival, progression and death) and monthly cycles for a population of previously untreated patients with indolent NHL. Data from a phase III clinical trial was used and expanded to include unpublished 53-month median follow-up data. Survival after first-line therapy was estimated from the Scotland and Newcastle Lymphoma Group registry data and utilities were derived from a study in the UK performed in patients with follicular lymphoma. Resource consumption was estimated by a Portuguese expert panel (Delbecq Panel). Costs were calculated from the Portuguese NHS perspective through official data with prices updated to 2008. Only direct medical costs were considered. Costs and clinical outcomes were discounted at 5% per annum. Deterministic and probabilistic sensitivity analysis were performed around assumptions on the time horizon, costs, utilities and excess mortality rate due to progression applied in the base-case analysis. RESULTS: The 10-year base-case analysis showed a lower total cost per patient with CVP alone (€ 85,838) in comparison with R-CVP (€ 87,774). Life expectancy and Quality adjusted life expectancy per patient were higher with R-CVP (6.361 and 4.166, respectively) than with CVP alone (5.557 and 3.438, respectively), representing increases of 0.804 in LYG and 0.728 (8.7 months) in QALYs gained. The incremental cost per LYG was € 2,407 and the incremental cost per QALY gained was € 2,661. The probabilistic sensitivity analysis confirmed the robustness of the base-case analysis results. CONCLUSIONS: This study demonstrates that the combination R-CVP in previously untreated indolent NHL patients improves life expectancy and is a cost-effective alternative to CVP in Portugal.
- Bisalbuminemia: A Rare Variant of AlbuminPublication . Garcez, C; Carvalho, SINTRODUCTION: Bisalbuminemia is a qualitative disorder of albumin and it is defined by the coexistence in the same individual of two types of serum albumin with different electrophoretic mobility. There are two forms: hereditary and permanent, or acquired and transient. CASE REPORT: Girl, 17-years-old, referenced to the hospital consult after incidental finding of bisalbuminemia detected in plasma protein electrophoresis performed within the investigation of diminished muscular tone in the lower limbs. Physical examination was normal. Secondary causes of bisalbuminemia were excluded. Later, it was confirmed the same disorder in plasma protein electrophoresis performed to her 14-years-old brother and mother. DISCUSSION: We describe a rare case of hereditary bisalbuminemia in a portuguese family. In general, this condition shows no pathological significance, however it is of interest to the clinicians the knowledge of this analytic change for better orientation of their patients.
- A case of pediatric paraparesis secondary to an idiopathic acute transverse myelitisPublication . Teixeira, J; Carvalho, S; Martins, S; Pontes, T; Machado, A; Antunes, HIntroduction: Acute transverse myelitis (ATM) refers to a frequently idiopathic, segmental spinal cord inflammation. It is a rare condition, in particular in children, and not previously reported in a family retinitis pigmentosa (RP) clinical setting. Case Report: An 11-year-old previously healthy girl, with a family history of RP, presented with a subacute flaccid paraparesis, with bilateral, up to the fourth dorsal level, mixed sensory hypoesthesia and autonomic dysfunction. Brain and spinal cord magnetic resonance imaging (MRI) showed an extensive, T2-hyperintense, non-contrast enhancing lesion from the second to fifth dorsal levels. Cerebrospinal fluid (CSF) and lab studies were normal, as the ophthalmologic observation. Treated with high-dose corticosteroids and intensive physical therapy,a significant recovery could be seen. Conclusion:Early pharmacological and physical treatment is fundamental and may indeed change the prognosis of this disease ATM. The family history of RP, although probably incidental, brings nevertheless the issue of a possible etiological contribution, or pathologic common pathways.
- El síndrome de Mauriac todavía existePublication . Dias, J; Martins, S; Carvalho, S; Marques, O; Antunes, ABACKGROUND/OBJECTIVE: Mauriac syndrome (MS) is a rare complication of type 1 diabetes mellitus (DM1). It is related to low insulin concentrations and is less common since longer-acting insulins became available. It is characterized by hepatomegaly, growth and puberty delay, and the presence of elevated transaminases and serum lipids. The aim of this study was to describe the patients from a pediatric diabetic population that fulfill the criteria of MS. MATERIALS AND METHODS: A retrospective analysis of the pediatric diabetic population with diagnostic criteria of MS currently followed at Hospital de Braga, was performed. RESULTS: From a population of 91 patients with DM1 18 years, 6 patients with the criteria for MS were identified: 5 girls, and 1 boy. The age at presentation was 13-17 years, with a minimum interval between DM1 diagnosis and MS criteria of 4 years. All the patients were prescribed intensive insulin therapy (median daily insulin dose: 0.88U/kg). All had a previous history of poor glycemic control before the diagnosis of MS with glycated hemoglobin (HbA1c) between 8.8 and 12.9%. Increase of hepatic enzymes was present in all the patients; 4 of them had associated hepatomegaly. All the girls presented puberty delay and cushingoid features. None of the patients presented short stature and 5 of them presented mixed dyslipidemia. CONCLUSIONS: Although MS is an ancient entity described in DM1, it still exists, particularly in adolescent females. Being aware of MS is of extreme importance since most of the clinical features are reversible with better glycemic control.
- Giant meningioma in paranasal sinuses: an atypical nasal occupationPublication . Silva, D; Carvalho, S; Marçal, N; Dias, L
- A granular cell tumor: an unusual colon polypPublication . Leal, T; Carvalho, S; Costa, JMWe read with interest the article by Sevilla Ribota et al1 that described an unexpected finding of a granular cell tumour (GCT) of the rectum, which was removed by band ligation-assisted mucosectomy. We present a similar case of a GCT of the cecum, which was resected using a different endoscopic procedure.
- Mauriac syndrome still existsPublication . Dias, J; Martins, S; Carvalho, S; Marques, O; Antunes, ABACKGROUND/OBJECTIVE: Mauriac syndrome (MS) is a rare complication of type 1 diabetes mellitus (DM1). It is related to low insulin concentrations and is less common since longer-acting insulins became available. It is characterized by hepatomegaly, growth and puberty delay, and the presence of elevated transaminases and serum lipids. The aim of this study was to describe the patients from a pediatric diabetic population that fulfill the criteria of MS. MATERIALS AND METHODS: A retrospective analysis of the pediatric diabetic population with diagnostic criteria of MS currently followed at Hospital de Braga, was performed. RESULTS: From a population of 91 patients with DM1 18 years, 6 patients with the criteria for MS were identified: 5 girls, and 1 boy. The age at presentation was 13-17 years, with a minimum interval between DM1 diagnosis and MS criteria of 4 years. All the patients were prescribed intensive insulin therapy (median daily insulin dose: 0.88 U/kg). All had a previous history of poor glycemic control before the diagnosis of MS with glycated hemoglobin (HbA1c) between 8.8 and 12.9%. Increase of hepatic enzymes was present in all the patients; 4 of them had associated hepatomegaly. All the girls presented puberty delay and cushingoid features. None of the patients presented short stature and 5 of them presented mixed dyslipidemia. CONCLUSIONS: Although MS is an ancient entity described in DM1, it still exists, particularly in adolescent females. Being aware of MS is of extreme importance since most of the clinical features are reversible with better glycemic control.
- Pseudomyogenic hemangioendothelioma: a little-known tumorPublication . Santos, RP; Carvalho, S; Joana, G; Perdal, JPseudomyogenic hemangioendothelioma (PHE) is a rare indolent vascular tumor that typically has a multifocal presentation and involves multiple tissue planes. This report describes a 34-year-old man with multiple infiltrated brown papules and plaques on his left leg that had evolved for 6 months. The skin biopsy revealed a dermal and subcutaneous neoplasm composed of fascicles of spindle cells with atypia and epithelioid cells with prominent nucleoli and abundant eosinophilic cytoplasm. There was no evidence of necrosis, and the mitotic rate was low. There was strong reactivity with cytokeratin AE1/AE3, ERG, and FLI1, multifocal reactivity with smooth muscle actin, and focal reactivity with CD31. There was no expression of keratin MNF116, CAM5.2, CD34, CAMTA1, S100-protein, epithelial membrane antigen, melan-A, HMB-45, factor XIIIa, HHV8, or CD10. The nuclei of neoplastic cells showed intact expression of INI1. The clinical, histological, and immunophenotypical aspects were consistent with a diagnosis of PHE. A lower limb CT scan showed lesions in the skin, muscle, and bone planes. The patient was sent to an oncology center, where he maintains regular clinical and imagiological follow-up.
- Serum leptin levels in overweight children and adolescentsPublication . Antunes, H; Santos, C; Carvalho, SLeptin is an adipocyte-secreted hormone which plays a key role in energy homeostasis. Our aim was to determine the relationship between serum leptin and clinical and biochemical features in overweight children and adolescents. Overweight children and adolescents followed in this Unit with serum leptin ascertained were included. Clinical, biochemical and abdominal ultrasound data were analysed. Statistical analysis was performed by t test, chi2, Pearson's correlation and linear regression. One outlier of serum leptin was excluded to perform correlation and regression. Serum leptin was determined in 357 patients. At the first visit, the mean age was 9.5 (sd 3.2) years and mean BMI z-score was 1.72 (sd 1.34) (girls 1.71 (sd 1.16); boys 1.72 (sd 1.11)). Serum leptin levels were significantly related to: sex (mean: girls 48.0 ng/ml, boys 34.4 ng/ml; P = 0.003); Tanner stage (mean: I-II 37.0 ng/ml, III-V 45.2 ng/ml; P = 0.035); systolic blood pressure (mean: normal 41.3 ng/ml, high 44.0 ng/ml; P = 0.009); BMI z-score (r 0.136; P = 0.010); C-peptide (r 0.17; P = 0.002); insulin (r 0.34; P < 0.001); homeostasis model assessment of insulin resistance (HOMA-IR) (r 0.25; P < 0.001) and aspartate aminotransferase (r - 0.12; P = 0.023). In the multivariate analysis (with leptin as the dependent variable and sex, Tanner stage, BMI z-score, systolic blood pressure, aspartate aminotransferase, C-peptide, insulin and HOMA-IR as independent variables), sex and BMI were determinant factors. The present study in overweight children and adolescents showed that being female and greater BMI were significantly and independently associated with increased serum leptin. In this large cohort other associations with leptin described in the literature can be discharged.