Pulmonary vasculitis in Hughes-Stovin syndrome (HSS): a reference atlas and computed tomography pulmonary angiography guide—a report by the HSS International Study Group

Introduction Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by widespread venous/arterial thrombosis and pulmonary artery aneurysms (PAAs), which is associated with serious morbidity and mortality. All fatalities reported in HSS resulted from unpredictable fatal suffocating hemoptysis. Therefore, it is necessary to recognize pulmonary complications at an early stage of the disease. Objectives The aims of this study are to develop a reference atlas of images depicting the characteristic features of HSS by computed tomography pulmonary angiography (CTPA). To make a guide for physicians by developing a classification of PAAs according to the severity and risk of complications associated with each distinct lesion type. Methods The Members of the HSS International Study Group (HSSISG) collected 42 cases, with high-quality CTPA images in one radiology station and made reconstructions from the source images. These detailed CTPA studies were reviewed for final image selection and approved by HSSISG board members. We classified these findings according to the clinical course of the patients. Results This atlas describes the CTPA images that best define the wide spectrum of pulmonary vasculitis observed in HSS. Pulmonary aneurysms were classified into six radiographic patterns: from true stable PAA with adherent in-situ thrombosis to unstable leaking PAA, BAA and/or PAP with loss of aneurysmal wall definition (most prone to rupture), also CTPA images demonstrating right ventricular strain and intracardiac thrombosis. Conclusion The HSSISG reference atlas is a guide for physicians regarding the CTPA radiological findings, essential for early diagnosis and management of HSS-related pulmonary vasculitis. Key Points• The Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by extensive vascular thrombosis and pulmonary artery aneurysms (PAAs) that can lead to significant morbidity and mortality.• All fatalities reported in HSS were related to unpredictable massive hemoptysis; therefore, it is critical to recognize pulmonary complications at an early stage of the disease.• The HSS International Study Group reference atlas classifies pulmonary vasculitis in HSS at 6 different stages of the disease process and defines the different radiological patterns of pulmonary vasculitis notably pulmonary artery aneurysms, as detected by computed tomography pulmonary angiography (CTPA).• The main aim of the classification is to make a guide for physicians about this rare syndrome. Such a scheme has never been reached before since the first description of the syndrome by Hughes and Stovin since 1959. This classification will form the basis for future recommendations regarding diagnosis and treatment of this syndrome.

THE ROLE OF PRIVATE LAW IN PROMOTING SOCIAL JUSTICE

The first contribution published in this edition is an abridged version of the inaugural lecture delivered by Professor Eltjo JH Schrage on 24 August 2009 in Port Elizabeth. The Faculty of Law is honoured that such an internationally esteemed jurist accepted the appointment as first Honorary Professor of the Faculty of Law in 2009. Prof Eltjo JH Schrage was born in Groningen. He studied law at the University of Groningen, where he obtained his doctorandus, a degree which is analogous to our master’s degree. In 1975 he defended his doctoral thesis entitled Libertas est facultas naturalis. Menselijke vrijheid in een tekst van de Romeinse jurist Florentinus (Human liberty in a text of the Roman jurist Florentinus). His academic career commenced in 1969 at the Free University, Amsterdam. In 1980 he was appointed as professor at the Free University in Roman Law and Legal History. In 1998 he became the director of the Paul Scholten Institute at the University of Amsterdam. Some of his other academic appointments include the following:• Chairperson: International Study Group on the Comparative Legal History of the Law of Restitution;• Chairperson: International Study Group on the Comparative Legal History of the Law of Torts;• Visiting Professor: University of Cape Town;• Visiting Fellow: Magdalen College, Oxford University as well as visiting professor at Oxford;• Visiting Professor: University of the North (now Limpopo) in Polokwane; and• Visiting Fellow: Trinity College, Cambridge University as well as visiting professor, Cambridge. Prof Schrage has published extensively in International journals in Dutch, English, German French, and Italian. He has edited, written and contributed to more than 30 books, and written more than 100 articles. He has been the supervisor of numerous doctoral students, including Prof Marita Carnelley of the University of KwaZulu-Natal and erstwhile member of the Faculty of Law, Nelson Mandela Metropolitan University and Prof André Mukheibir, Head of Department, Private Law of the Nelson Mandela Metropolitan University. He was also the promoter of the honorary doctorate awarded by the University of Amsterdam to the former chief justice of South Africa, Arthur Chaskalson in 2002. Prof Schrage has also acted as judge in the Amsterdam court since 1981. Prof Schrage is married to Anneke Buitenbos-Schrage and the couple have four children and one grandchild.

Mathematics Heritage Project: An Exploration Empowering Students' Mathematical Identities

The International Study Group on Ethnomathematics (ISGEm) supports incorporating cultural diversity of mathematical practices to promote the teaching and learning of school mathematics. Through The Mathematics Heritage Project, students at a middle school in the southeastern United States developed unique creations to connect with the mathematics connected to their identities and self-identified cultural group. Upon reflection, students reported an increased awareness of the relevance of mathematics in their lives and a sense of ownership that is both meaningful and modern.

Chronic Liver Disease Increases Mortality Following Pancreatoduodenectomy

According to the International Study Group of Pancreatic Surgery (ISGPS), data about the impact of pre-existing liver pathologies on delayed gastric emptying (DGE) after pancreatoduodenectomy (PD) according to the definitions of the International Study Group of Pancreatic Surgery (ISGPS) are lacking. We therefore investigated the impact of DGE after PD according to ISGPS in patients with liver cirrhosis (LC) and advanced liver fibrosis (LF). Patients were analyzed with respect to pre-existing liver pathologies (LC and advanced LF, n = 15, 6% vs. no liver pathologies, n = 240, 94%) in relation to demographic factors, comorbidities, intraoperative characteristics, mortality and postoperative complications, with special emphasis on DGE. DGE was equally distributed (DGE grade A, p = 1.000; B, p = 0.396; C, p = 0.607). Particularly, the first day of solid food intake (p = 0.901), the duration of intraoperative administered nasogastric tube (NGT) (p = 0.812), the rate of re-insertion of NGT (p = 0.072), and the need for parenteral nutrition (p = 0.643) did not differ. However, patients with LC and advanced LF showed a higher ASA (American Society of Anesthesiologists) score (p = 0.016), intraoperatively received more erythrocyte transfusions (p = 0.029), stayed longer in the intensive care unit (p = 0.010) and showed more intraabdominal abscess formation (p = 0.006). Moreover, we did observe a higher mortality rate amongst patients with pre-existing liver diseases (p = 0.021), and reoperation was a risk factor for higher mortality (p ≤ 0.001) in the multivariate analysis. In our study, we could not detect a difference with respect to DGE classified by ISGPS; however, we did observe a higher mortality rate amongst these patients and thus, they should be critically evaluated for PD.

Appropriateness of test request and clinical outcome: the case of autoimmune diseases

Objectives: To improve the appropriateness in the autoantibody test requests for proper diagnosis of systemic rheumatic autoimmune diseases, celiac disease and anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides. Methodology: Review of recommendations proposed by the Study Group in Autoimmunology (GDS-AI) of the Italian Society of Clinical Pathology and Laboratory Medicine (SIPMeL) and the European Society For Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHaN) Results: This document aims to bring to the attention of general practitioners the recommendations proposed by National and International study group to improve the appropriateness in the autoantibody test requests expecially in sytemic autoimmune and celiac disease. Conclusions: Autoimmunity is a specialistic laboratory area where the costs are extremely high compared to other diagnostics: it would therefore be very helpful and economically advantageous to align the clinicians on appropriate requests and the laboratory on useful diagnostics. In Autoimmunology, diagnostic appropriateness remains a complex and debated topic from the management, professional and economic point of view.