Patologia Clínica
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- Abernethy Malformation and Hepatocellular Carcinoma: a Serious Consequence of a Rare DiseasePublication . Jaklitsch, M; Sobral, M; Carvalho, AM; Pinto Marques, HCongenital portosystemic shunts (CPSS) are a rare vascular consequence of embryogenetic vascular alterations or the persistence of the fetal circulation elements, first described by John Abernethy in 1793 and classified by Morgan and Superina, into complete and partial portosystemic shunts. Its prevalence to this day has not been defined. We present a patient series of a 44-year-old and 47-year-old man and woman, with this rare congenital malformation and underlining hepatocellular carcinoma (HCC) treatment strategies. Over half of the individuals with CPSS have benign or malignant liver tumours, ranging from nodular regenerative hyperplasia, focal nodular hyperplasia, adenomas, HCC and hepatoblastomas. Additionally, it is known that half of individuals with Abernethy malformation type Ib will develop one or multiple types of tumours. There seems to be a direct association with tumorigenesis and CPSS, which is the primary consequence of absent portal flow. Surgery is the treatment of choice, either as a curative resection or orthotopic liver transplantation if recommended as per the criteria, in which replacing the hepatic parenchyma in the setting of an Abernathy malformation will correct the underlining hyper-arterialisation.
- Acute Bivalvular Left-Sided Methicillin-Resistant Staphylococcus Aureus Endocarditis with Cardiac, Cerebral, Renal and Septic ComplicationsPublication . Póvoas, D; Figueiredo, M; Murinello, A; Damásio, H; Ramos, A; Rodrigues, N; Sousa, J; Carvalho, F; Peres, H; Gomes, PInfective endocarditis (IE) is now rare in developed countries, but its prevalence is higher in elderly patients with prosthetic valves, diabetes, renal impairment, or heart failure. An increase in health-care associated IE (HCAIE) has been observed due to invasive maneuvers (30% of cases). Methicillin-resistant Staphylococcus aureus (MRSA) and Enterococcus are the most common agents in HCAIE, causing high mortality and morbidity. We review complications of IE and its therapy, based on a patient with acute bivalvular left-sided MRSA IE and a prosthetic aortic valve, aggravated by congestive heart failure, stroke, acute immune complex glomerulonephritis, Candida parapsilosis fungémia and death probably due to Serratia marcescens sepsis. The HCAIE was assumed to be related to three temporally associated in-hospital interventions considered as possible initial etiological mechanisms: overcrowding in the hospital environment,iv quinolone therapy and red blood cell transfusion. Later in the clinical course,C. parapsilosis and S. marcescens septicemia were considered to be possible secondary etiological mechanisms of HCAIE.
- Acute Haematogenous Osteomyelitis in Lisbon: An Unexpectedly High Association with Myositis and ArthritisPublication . Gouveia, C; Branco, J; Norte, S; Arcangelo, J; Alves, P; Pinto, M; Tavares, DIntroduction: Despite the current trend toward less aggressive therapeutic approaches, acute haematogenous osteomyelitis (AHO) continues to be a challenge and is associated with significant morbidity worldwide. Our aim was to assess whether compliance with the current protocol was achieved in 80% of cases, to identify complications and the associated risk factors, and to analyse trends in the aetiology and management of AHO in the paediatric population. Methods: We conducted a longitudinal, observational, single-centre study in patients with AHO aged less than 18 years admitted to a paediatric hospital between 2008 and 2018 divided in 2 cohorts (before and after 2014). We analysed data concerning demographic and clinical characteristics and outcomes. Results: The study included 71 children with AHO, 56% male, with a median age of 3 years (interquartile range, 1-11). We found a 1.8-fold increase of cases in the last 5 years. The causative agent was identified in 37% of cases: MSSA (54%), MRSA (4%), S. pyogenes (19%), K. kingae (12%), S. pneumoniae (8%), and N. meningitidis (4%). Complications were identified in 45% of patients and sequelae in 3.6%. In recent years, there was an increase in myositis (30% vs 7%; P=.02), septic arthritis (68 vs 37.2%; p=0.012) and in the proportion of patients treated for less than 4 weeks (37 vs 3.5%; p=0.012), with a similar sequelae rates. The risk factors associated with complications were age 3 or more years, C-reactive protein levels of 20mg/L or higher, time elapsed between onset and admission of 5 or more days and positive culture, although the only factor that continued to be significantly associated in the multivariate analysis was positive culture. The presence of complications was a risk factor for sequelae at 6 months. Conclusions: Our study confirms that AHO can be aggressive. The identification of risk factors for complications is essential for management.
- Acute Kidney Injury in the Context of Inflammatory Bowel Disease - A Clinical CasePublication . Cristóvão Marques, J; Barata, R; Lemos Garcia, J; Navarro, D; Góis, M; Sousa, H; Cotovio, P; Ribeiro, F; Nolasco, FExtraintestinal manifestations of inflammatory bowel disease are common and extendable to all organs. Kidney and lower genitourinary system occurs in 4-23% of cases. This may be dependent on inflammatory bowel disease activity, secondary to metabolic disorders, drugs or others. We present a case of a 68-year-old man with ulcerative colitis for 22 years admitted in our department for acute nephritic syndrome. Urinary microscopy suggested glomerular injury. A kidney biopsy was performed and was compatible with acute interstitial nephritis and IgA nephropathy. Toxicity of mesalazine and glomerulonephritis secondary to ulcerative colitis were assumed. The patient suspended mesalazine and started prednisolone with clinical improvement. Our purpose is to sensitize the importance of having a prompt and thorough evaluation of acute kidney injury in patients with inflammatory bowel disease. We briefly review the broad spectrum of kidney manifestations in this population, focusing on mesalazine-induced nephrotoxicity.
- Adenocarcinoma of the Urachus: A Primary Not to OverlookPublication . Alves, AS; Camelo, R; Varela, M; Lopes Dias, JUrachal carcinoma is a rare and aggressive neoplasm, involving the urachus, a remnant of the fetal genitourinary tract that extends from the umbilicus to the dome of the bladder. We present a 49-year-old woman with a history of pelvic discomfort and a palpable suprapubic mass. Pelvic ultrasound and magnetic resonance revealed a 55-mm midline supravesical mass, arising from the urachus and extending inferiorly through the bladder. The characteristics of the mass and its location favored the diagnosis of urachal carcinoma. There were no regional or distant metastases. The patient underwent surgical resection of the tumor and histopathological examination confirmed the diagnosis of a non-cystic enteric type urachal adenocarcinoma. The purpose of this article is to present a case of primary adenocarcinoma of the urachus, describe its epidemiology and clinical features, as well as illustrate its key imaging findings along with pathologic correlation
- Adrenal Vein Sampling in the Management of Primary Aldosteronism: The Added Value of Intraprocedural Cortisol AssessmentPublication . Manique, I; Amaral, S; Matias, A; Bouça, B; Serranito, S; Torres, J; Gutu, O; Bilhim, T; Coimbra, E; Rodrigues, I; Godinho, C; Cortez, L; Silva-Nunes, JIntroduction: Primary aldosteronism is the most common cause of secondary hypertension. Adrenal vein sampling is the gold standard for subtyping primary aldosteronism. However, this procedure is technically challenging and often has a low success rate. Our center is one of the very few performing this technique in our country with an increasing experience. Objective: The aim of this study was to evaluate the role of the cortisol intraprocedural assay in improving the performance of adrenal vein sampling. Design: We enrolled all of the patients with primary aldosteronism that underwent adrenal vein sampling from February 2016 to April 2023. The cortisol intraprocedural assay was introduced in October 2021. Methods: We enrolled a total of 50 adrenal vein samplings performed on 43 patients with the diagnosis of primary aldosteronism. In this sample, 19 patients and 24 patients underwent adrenal vein sampling before and after intraprocedural cortisol measurement, respectively. The procedure was repeated in seven patients (one before and six after intraprocedural cortisol measurement), given the unsuccess of the first exam. Selectivity of the adrenal vein sampling was assumed if the serum cortisol concentration from the adrenal vein was at least five times higher than that of the inferior vena cava. Lateralization was assumed if the aldosterone to cortisol ratio of one adrenal vein was at least four times the aldosterone to cortisol ratio of the contralateral side. Results: The mean age of the patients that underwent adrenal vein sampling (N = 43) was 55.2 ± 8.9 years, and 53.5% (n = 23) were female. The mean interval between the diagnosis of hypertension and the diagnosis of primary aldosteronism was 9.8 years (±9.9). At diagnosis, 62.8% of the patients (n = 27) had hypokalemia (mean value of 3 mmol/L (±0.34)), 88.4% (n = 38) had adrenal abnormalities on preprocedural CT scan, and 67.4% (n = 29) described as unilateral nodules. There were no statistically significant differences in the patients' baseline characteristics between the two groups (before and after intraprocedural cortisol measurement). Before intraprocedural cortisol measurement, adrenal vein sampling selectivity was achieved in 35% (n = 7) patients. Selectivity increased to 100% (30/30) after intraprocedural cortisol measurement (p < 0.001). With the exception of one patient who refused it, all patients with lateralized disease underwent unilateral adrenalectomy with normalization of the aldosterone to renin ratio postoperatively. Conclusions: The lack of effective alternatives in subtyping primary aldosteronism highlights the need to improve the success rate of adrenal vein sampling. In this study, intraprocedural cortisol measurement allowed a selectivity of 100%. Its addition to this procedure protocol should be considered, especially in centers with a low success rate.
- Afibrinogenémia CongénitaPublication . Pastilha, P; Coelho, L; Costa, T; Deus, G; Santos, H; Rosa, A; Barrocas, F; Braga, LOs autores apresentam o caso de uma criança com Afibrinogenémia Congénita. A propósito desta entidade revêm a literatura referindo e comentando alguns aspectos e particularidades desta rara doença da coagulação.
- Aggressive Pituitary Lesion with a Remarkably High Ki-67Publication . Marques, P; Mafra, M; Calado, C; Martins, A; Monteiro, J; Leite, VOs raros tumores pituitários agressivos são chamados carcinomas quando são detectadas metástases, sejam sistêmicas e/ou em sistema nervoso central. Alguns casos estão associados com superprodução de hormônio, mas a maioria é diagnosticada em função dos sintomas locais. Essas neoplasias são geralmente refratárias aos tratamentos atuais. Uma mulher com 51 anos de idade apresentou dor de cabeça de início súbito, paralisia de braço esquerdo e hipoestesia facial esquerda. A tomografia e a ressonância magnética revelaram um tumor pituitário invadindo os seios esfenoidal e cavernoso esquerdos. Os dados laboratoriais excluíram hipersecreção hormonal. A paciente foi submetida à cirurgia transesfenoidal, e os achados histológicos mostraram uma neoplasia com Ki-67 estimado em 75%. As imagens excluíram tanto um tumor oculto primário quanto disseminação sistêmica ou do sistema nervoso central. Três semanas após a cirurgia, a condição neurológica apresentou piora com início de ataxia, ptose bilateral, oftalmoplegia e aumento do tamanho da lesão, levando à intervenção cirúrgica por craniotomia, seguida por apenas algumas sessões de radioterapia devido à progressão grave da doença. A paciente veio a óbito depois de quase dois meses das manifestações iniciais. O caso ilustra a agressividade de algumas lesões pituitárias, a eficácia limitada das modalidades atuais de tratamento, como a cirurgia ou a radioterapia, e as limitações da classificação atual de tumores pituitários. Até onde sabemos, esse caso corresponde a uma das neoplasias pituitárias mais agressivas descritas até hoje, com um nível muito alto de Ki-67 (75%) e sobrevida curta (2 meses). O nível de Ki-67 pode ser de valor prognóstico em tumores pituitários. A classificação da Organização Mundial da Saúde (OMS) para tumores pituitários deveria ser revisitada.
- An Eleven-Year-Old Boy With Arthritis of the Hip Joint, and an 8-Year-Old Boy With SacroiliitisPublication . Mendo, T; Norte, S; Alves, P; Tavares, D; Pinto, M; Gouveia, C
- Anaphylaxis in Pregnancy: a Rare Cause of Neonatal MortalityPublication . Berenguer, A; Couto, A; Brites, V; Fernandes, RAnaphylaxis is a rare condition in pregnancy. Drugs are the aetiological agents most often implicated. Maternal anaphylaxis can lead to significant fetal morbidity and even mortality if uterine perfusion and maternal oxygenation are compromised. Significant risk of neonatal neurological damage or death can occur even when the maternal clinical outcome is favourable. The authors present the case of a newborn, born at gestational age of 29 weeks, who died at 11 days of life with hypoxic-ischaemic cerebral injuries as a consequence of maternal anaphylaxis following the administration of amoxicillin in the community setting.