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Endoscopic therapy in disconnected duct syndrome: re-connecting the duct.
Get PDFWe report the case of a 61-year-old male patient with a history of acute necrotizing biliary pancreatitis and a disconnected duct syndrome. He underwent transgastric drainage using a luminal apposing metal stent and transgastric necrosectomy with complete resolution of the necrosis. A pancreatic fistula was identified during pancreatography and a pancreatic plastic stent was placed in order to manage the disconnected duct syndrome. The tip of the pancreatic stent could be seen inside the pancreatic collection, which is an unusual finding. There was a resolution of the collection and the pancreatic stent was removed.info:eu-repo/semantics/publishedVersio
Design and validation of a patient-reported outcome measure scale in acute pancreatitis: the PAN-PROMISE study
No full textTRIAL REGISTRATION NUMBER: NCT03650062.Objective: This study aimed to develop and validate a patient-reported outcome measure (PROM) in acute pancreatitis (AP) as an endpoint centred on the patient.
DESIGN:
A PROM instrument (PAtieNt-rePoRted OutcoMe scale in acute pancreatItis, an international proSpEctive cohort study, PAN-PROMISE scale) was designed based on the opinion of patients, professionals and an expert panel. The scale was validated in an international multicentre prospective cohort study, describing the severity of AP and quality of life at 15 days after discharge as the main variables for validation. The COSMIN (COnsensus-based Standards for the selection of health status Measurement INstruments) methodology was applied. Both the design and validation stages considered the content and face validity of this new instrument; the metric properties of the different items, reliability (reproducibility and internal consistence), the construct, structural and criterion validity, responsiveness and interpretability of this scale.
RESULTS:
PAN-PROMISE consists of a seven-item scale based on the symptoms that cause the most discomfort and concern to patients with AP. The validation cohort involved 15 countries, 524 patients. The intensity of symptoms changed from higher values during the first 24 hours to lower values at discharge and 15 days thereafter. Items converged into a unidimensional ordinal scale with good fit indices. Internal consistency and split-half reliability at discharge were adequate. Reproducibility was confirmed using test-retest reliability and comparing the PAN-PROMISE score at discharge and 15 days after discharge. Evidence is also provided for the convergent-discriminant and empirical validity of the scale.
CONCLUSION:
The PAN-PROMISE scale is a useful tool to be used as an endpoint in clinical trials, and to quantify patient well-being during the hospital admission and follow-up.info:eu-repo/semantics/publishedVersio
Stiff person spectrum disorders: An illustrative case series of their phenotypic and antibody diversity
No full textStiff person spectrum disorders (SPSD) are a broad group of immune-mediated disorders. Clinical presentations include classical stiff person syndrome (SPS), focal SPS, and progressive encephalomyelitis with rigidity and myoclonus (PERM). The most frequently associated antibodies are anti-GAD65, anti-GlyR, anti-amphiphysin, and anti-DPPX. Immunotherapy is the primary treatment modality. We present an illustrative case series of three patients: anti-GlyR antibody-mediated PERM presenting as rapidly progressive dementia; anti-amphiphysin antibody-mediated SPS; and SPS presentation with anti-Zic4 antibodies, spasmodic laryngeal stridor and fluctuating eyelid ptosis. Clinical characteristics, CSF findings, neurophysiological features, adequate immunological assays and a high suspicion index are essential for prompt diagnosis and management.info:eu-repo/semantics/publishedVersio
Bipolar disorder diagnostic stability: a Portuguese multicentric study
No full textinfo:eu-repo/semantics/publishedVersio
Etiologia, Tratamento e Prognóstico da Pericardite Aguda
Get PDFIntroduction: Acute pericarditis is a common inflammatory
condition of the pericardium usually assumed to be viral or idiopathic. Despite recent treatment improvements, information
is scarce regarding etiology and prognosis. Our aim was to
determine the incidence of pericarditis with a known etiology
and assess clinical prognostic predictors.
Material and Methods: A clinical retrospective analysis of
hospitalized patients with acute pericarditis was conducted
from 2012 to 2016. Population was characterized according to etiology, clinical presentation, treatment and prognosis.
Outcomes of interest, evaluated at one year after hospital discharge, were pericarditis recurrence, hospitalization, constriction and overall mortality.
Results: A total of 94 patients were enrolled, median age
46 years (inter-quartile range 32-61), 65% were male. Idiopathic etiology was responsible for 68% of cases. A specific
etiology was found in the remaining 32% of patients, being
the most frequent autoimmune disease (12%) and malignancy
(5%). Idiopathic pericarditis was associated with myopericarditis (p = 0.049) and a known etiology with pericardial effusion
(p = 0.001) and cardiac tamponade (p = 0.027). Recurrence of
pericarditis was found in 13% of patients. Corticosteroid treatment in patients with an identified etiology was not associated
with an increase in recurrence (p = 0.220). Overall 1-year mortality was 9%. A defined etiology was the only independent
predictor of mortality at multivariate analysis (OR 40.3; 95% CI
1.9 – 137.2; p = 0.016).
Conclusion: Up to one third of hospitalized patients with
acute pericarditis have an identified cause of pericarditis and
these patients are at increased risk of mortality.Introdução: A pericardite aguda é uma síndrome frequente
caracterizada pela inflamação do pericárdio e usualmente
atribuído a etiologia viral/idiopática. Apesar dos avanços no
tratamento, existe informação limitada em relação a etiologia e
prognóstico. O objetivo do nosso trabalho foi identificar a prevalência de pericardite aguda com etiologia especifica e avaliar
marcadores clínicos de prognóstico.
Material e Métodos: Estudo retrospetivo de doentes hospitalizados por pericardite aguda entre 2012-2016. A população foi caracterizada quanto a etiologia, apresentação clínica,
tratamento e prognóstico. Para avaliação de prognóstico
foram avaliadas recorrência de pericardite, pericardite constritiva e mortalidade global um ano após alta hospitalar.
Resultados: Foram incluídos 94 doentes com idade mediana 46 anos (IIQ 32-61), 65% eram do sexo masculino. A
etiologia idiopática foi responsável por 68% dos casos. Etiologia especifica foi identificada em 32% dos doentes, sendo
mais frequente doença autoimune (12%) e neoplasia (5%). A
pericardite idiopática foi mais associada a miopericardite (p
= 0,049), enquanto a etiologia específica se associou a derrame pericárdico (p = 0,001) e a tamponamento pericárdico
(p = 0,027). A recorrência de pericardite ocorreu em 13% dos
doentes. O tratamento com corticosteroides em doentes com
etiologia definida não se associou com aumento de recorrência (p = 0,220). A mortalidade global a um ano foi de 9%.
A etiologia definida de pericardite aguda revelou-se o único
preditor independente de mortalidade na análise multivariada
(OR 40,3; 95% CI 1,9 – 137,2; p = 0,016).
Conclusão: Cerca de um terço dos doentes hospitalizados
por pericardite aguda têm uma causa específica identificável
de pericardite e estes doentes apresentam risco aumentado
de mortalidadeinfo:eu-repo/semantics/publishedVersio
Gastrointestinal Tuberculosis Mimicking Crohn’s Disease
Get PDFWe present the case of a 24-year-old woman with complaints of abdominal pain, bloody diarrhea, and weight loss for 3 months. An outpatient colonoscopy revealed scattered ulcers, suggestive of Crohn's disease (CD). Histopathology also favored the diagnosis of CD. However, after admission to our hospital for further investigation, a chest radiograph revealed pulmonary cavitations. A computed tomography scan suggested the diagnosis of active pulmonary tuberculosis (TB). Therefore, a bronchofibroscopy, a total colonoscopy with ileoscopy, and an upper endoscopy were performed. Not only were acid-fast bacilli present in both bronchoalveolar lavage fluid and gastric juice, but also in colonic biopsies. A complete resolution of gastrointestinal symptoms was achieved 2 weeks after starting anti-TB drugs.info:eu-repo/semantics/publishedVersio
Surgical Resection of Multiple Small Bowel Neuroendocrine Tumours Using Intraoperative Fluorescence Angiography With Indocyanine Green Dye
No full textinfo:eu-repo/semantics/publishedVersio
Biomarkers and genetic modulators of cerebral vasculopathy in sub-Saharan ancestry children with sickle cell anemia
No full textWe investigated biomarkers and genetic modulators of the cerebral vasculopathy (CV) subphenotype in pediatric sickle cell anemia (SCA) patients of sub-Saharan African ancestry. We found that one VCAM1 promoter haplotype (haplotype 7) and VCAM1 single nucleotide variant rs1409419_T were associated with stroke events, stroke risk, as measured by time-averaged mean of maximum velocity in the middle cerebral artery, and with high serum levels of the hemolysis biomarker lactate dehydrogenase. Furthermore, VCAM-1 ligand coding gene ITGA4 variants rs113276800_A and rs3770138_T showed a positive association with stroke events. An additional positive relationship between a genetic variant and stroke risk was observed for ENPP1 rs1044498_A. Conversely, NOS3 variants were negatively associated with silent cerebral infarct events (VNTR 4b_allele and haplotype V) and CV globally (haplotype VII). The -alpha3.7kb-thal deletion did not show association with CV. However, it was associated with higher red blood cell and neutrophil counts, and lower mean corpuscular volume, mean corpuscular hemoglobin and red cell distribution width. Our results underline the importance of genetic modulators of the CV sub-phenotype and their potential as SCA therapeutic targets. We also propose that a biomarker panel comprising biochemical, hematological, imaging and genetic data would be instrumental for CV prediction, and prevention.info:eu-repo/semantics/publishedVersio
Utility of routine screening for alpha-1 antitrypsin deficiency in patients with bronchiectasis
Get PDFAlpha-1 antitrypsin deficiency (AATD) is a cause of bronchiectasis. Guidelines for bronchiectasis from the British Thoracic Society do not recommend to routinely test patients for AATD. In contrast, guidelines for AATD recommend routine screening. This contradiction, in part, results from the lack of data from large studies performing comprehensive screening. We screened 1600 patients with bronchiectasis at two centres in the UK from 2012 to 2016. In total, only eight individuals with AATD were identified representing 0.5% of the overall population. We conclude that routine screening for AATD in bronchiectasis in the UK has a low rate of detection. Further studies are required in different geographical regions, which may have a higher prevalence of AATD.info:eu-repo/semantics/publishedVersio
