Acta Clinica Belgica最新文献

Sarcoidosis is a multisystem disease of unclear etiology with a variable clinical profile characterized by the presence of non-caseating granuloma in involved organs. The diagnosis is often challenging and based on clinical, radiological and anatomopathological data. Sarcoidosis can be benign and self-limiting, but some cases may follow a chronic, progressive course and result in severe morbidity. The disease has a predilection for the lungs and thoracic lymph nodes but can involve nearly any part of the body, possible more commonly in areas with contact to the external environment, such as the eyes and the skin. This paper is based on a case in which a recurrent uveitis led to the diagnosis of an underlying sarcoidosis.

Hemodialysis-related portosystemic encephalopathy (HRPSE) is a clinical phenomenon where portosystemic encephalopathy (PSE) develops without liver dysfunction, usually caused by changes in the portosystemic blood flow related to hemodialysis. We describe the case of a 22-year old patient with a transjugular intrahepatic portosystemic shunt (TIPS) who developed HRPSE several months after initiation of hemodialysis. Despite initial therapy with laxatives and neomycin symptoms recurred. It was only after relocation of the hemodialysis catheter from the superior caval vein to the femoral vein that symptoms completely resolved.

Background: Turner syndrome (TS) is characterized by dysmorphism and body disproportion. TS women are also susceptible to a range of chronic disorders including arterial hypertension (AHT), osteoporosis, sensorineural hearing loss (SNHL), type 2 diabetes mellitus (DM2) and thyroid disease. The association between dysmorphism/body disproportion and chronic disease has never been studied in TS women. The effect of growth hormone treatment on body disproportion is also unclear. Objectives: to analyze dysmorphic features and body disproportion in TS women in relation to the presence of chronic disease and to document the effect of growth hormone therapy on body disproportion.

Method: 76 adult TS women with a regular follow up at the TS clinic UZ Ghent were invited to participate. Detailed body measurements were performed in 44 volunteering TS women. Scoring systems for overall dysmorphism, craniofacial dysmorphism, thoracic and limb abnormalities and skeletal disproportion were developed.

Results: TS women with a higher dysmorphism score were more at risk for AHT (p = 0.04) as well as those with a higher sitting height/standing height ratio (p < 0.05). Prevalence of AHT, osteoporosis and DM 2 was lower in TS women treated with GH during childhood (p < 0.05).

Conclusions: Adult TS women with relatively short legs or with more physical dysmorphic stigmata were more at risk for AHT. GH therapy does not seem to increase the risk of chronic disease on the long term.

We report the case of a 51 year-old female who complained of jaundice and weight loss. At the time of presentation, she had been in remission from a stage 2a ductal breast carcinoma for 58 months. The clinical presentation was suggestive of a primary pancreas cancer with liver metastases and retroperitoneal lymph nodes. We performed liver and pancreas biopsies that demonstrate a relapse of her old breast carcinoma with positive hormone receptors and HER2 positive. Conservative treatment by chemotherapy was given with Paclitaxel - Trastuzumab and Pertuzumab. Pancreatic metastases are uncommon. Furthermore, pancreatic metastases from breast cancer are very rare. We performed a review of the literature and found 48 cases of pancreatic metastases from breast cancer. We would like to highlight by this case that when a pancreatic lesion appears, in patients with a past history of cancer, physicians must not forget the possibility of metastases from primary tumor even if the initial stage, of the tumor, is low. However the diagnosis is not always easy. If liver and pancreatic lesions occur simultaneously, the clinical presentation can mimic metastatic primary pancreatic cancer. Therefore performing biopsy is highly recommended for making the correct diagnosis and also for the staging of the disease and the choice of the best treatment according to immunohistochemical analysis.

Introduction: Stroke is a development of an acute focal neurological deficit with an ischemic or hemorrhagic origin. Thrombolysis within 4.5 h of ischemic stroke onset improves outcome. Guidelines recommend administration of intravenous recombinant tissue plasminogen activator within 60 min upon arrival at the hospital, meaning the door-to-needle time (DNT) should be less than 60 min. In this study, a stroke protocol was introduced at the emergency department of the Ghent University Hospital with a primary goal to shorten the DNT.

Methodology: This study was an uncontrolled before-after cohort study. A 'Code Stroke' protocol (CSP) was implemented and the results from the pre-code stroke protocol period (Pre-CSP period, from 15 August 2016 until 5 March 2017) were compared with the results from the post-code stroke protocol period (Post-CSP period, from 6 March 2017 until 16 July 2017).

Results: The median DNT decreased significantly from 57 min in the Pre-CSP period to 33 min in the Post-CSP period (p < 0.001). The door-to-triage time (DTT), triage-to-emergency physician time (TET), emergency physician-to-CT time (ECT) and CT-to needle time (CNT) decreased significantly Post-CSP compared to Pre-CSP. When adjusting the results for other variables that might have an influence on these time intervals, the TET, ECT and CNT also decreased significantly. There was a statistically significant effect of the implementation of the CSP on the number of patients treated with a DNT within 20, 30, 45 and 60 min (p = 0.008).

Conclusion: A significant decrease in DNT can be achieved with the implementation of this stroke protocol.

Background: It is well known that Crohn's disease can involve the stomach. However, most often this upper gastrointestinal tract involvement is asymptomatic. Typically, there is involvement of the small intestine with the typical associated symptoms of Crohn's disease: abdominal cramps, diarrhoea and weight loss.

Methods: We report a case of a young woman with complaints of dyspepsia since 2 months.

Results: Gastroscopy revealed severe aphthous pangastritis with biopsies showing a focal active and chronic gastritis with presence of granulomas. We therefore performed a coloscopy showing an aphthous terminal ileum. The pathologic report indicated granulomatous reaction concordant with a slightly active, mildly chronic terminal ileitis typical for Crohn's disease.

Conclusion: The incidence of upper gastrointestinal tract involvement of Crohn's disease is still underestimated, partially due to the asymptomatic nature in two thirds of patients. IBD gastritis should always be included in the differential diagnosis of gastritis, considering the increased risk of a more severe disease course and complications.

Background: Idiopathic retroperitoneal fibrosis (iRPF) is a rare fibro-inflammatory disease, characterized by inflammation of the abdominal aorta and its surrounding structures. The exact pathophysiology remains unclear. Diagnosis is often troublesome due to the non-specific and highly variable clinical presentation. Standardized treatment protocols are lacking.

Objective: This article presents a review on iRPF, addressing clinical and diagnostic modalities as well as its pathophysiology and the possible inclusion within the IgG4-related disease (IgG4-RD) spectrum. Finally, a diagnostic-therapeutic algorithm for a standardized approach to iRPF is proposed.

Methods: The PubMed Internet database was searched. Articles were selected based on the relevance of abstract, article type and impact of the journal.

Results: iRPF and IgG4-RD share a common autoimmune aetiology. Diagnostics are multimodal and based on imaging. Ruling out malignancy should be of primary concern. Complications are mostly of renal or vascular origin due to compression of retroperitoneal structures. Corticosteroids remain the first-line treatment regimen and are mostly successful, but evidence supporting alternative immunosuppressive and anti-inflammatory treatments is growing. Long-term therapy, as well as follow-up, is paramount in this chronic and often relapsing disease.