Agents of Change in the Domestic Built Environment

As our cities age, a large number of spatial structures experience physical change. A better understanding of what this process may entail and the agents involved in it can extend the knowledge of practitioners, activists, and policy experts regarding the resilience of our domestic building stock and cities. Awan et al. (2013) explain that agents are not entirely free from societal and spatial constraints; instead, they are characterised by intent, shaped by their own visions and actions, and context, the spatial and social structures of which they are part and which they negotiate. This article discusses the intent and context of the agents involved in the construction and transformation of the Cité Ouvrière in Mulhouse in Eastern France from the mid-19th century to date. With 1,253 houses built for the workers of the Dollfus-Mieg et Compagnie (DMC) textile factory between 1853 and 1897, Cité Ouvrière was the largest and most successful employer-constructed housing scheme of its time, setting an example for many other European company towns. Through this exceptional case study, the article identifies the levels at which spatial agents operate, the means they use to instigate change, their dynamic relations, and the ways these are influenced by the wider historical context while influencing the making and evolution of the built form. Using historical and archival documents, it amounts to recognise an interplay of individuals and public and private groups, who have been responsible for taking decisions at different scales—the city, the neighbourhood, and the houses—and have instigated changes of different effect—from more localised to more aggregate.

Thyroid Carcinoma with NSD3::NUTM1 Fusion: a Case with Thyrocyte Differentiation and Colloid Production

In this report, we present a high-grade thyroid carcinoma with an NSD3::NUTM1 fusion detected on expanded next-generation sequencing testing. Nuclear protein of the testis (NUT) carcinomas comprise high-grade, aggressive tumors characterized by rearrangements of the NUTM1 gene with various partner genes, most commonly the bromodomain protein genes BRD4 and BRD3. Approximately 10% of NUT carcinomas contain an NSD3::NUTM1 fusion. NUT carcinomas manifest as poorly differentiated or undifferentiated squamous carcinomas, and 33% show areas of mature squamous differentiation. Only exceptionally have NUT carcinomas shown histology discordant from poorly differentiated/undifferentiated squamous carcinoma, and a thyroid NUT carcinoma with histologic thyrocyte differentiation has not been described to date. Our patient’s tumor exhibited mixed cytologic features suggestive of squamoid cells or papillary thyroid carcinoma cells. Overt squamous differentiation was absent, and the tumor produced colloid in poorly formed follicles. Immunophenotypically, the carcinoma was consistent with thyrocyte differentiation with expression of monoclonal PAX8, TTF1, and thyroglobulin (the last predominantly in extracellular colloid). There was zero to < 2% reactivity for proteins typically diffusely expressed in NUT carcinoma: p40, p63, and cytokeratins 5/6. NUT protein expression was equivocal, but fluorescence in situ hybridization confirmed a NUTM1 rearrangement. This exceptional case suggests that NUTM1 fusions may occur in an unknown number of aggressive thyroid carcinomas, possibly with distinctive histologic features but with thyrocyte differentiation. Recognition of this entity potentially has significant prognostic implications. Moreover, thyroid carcinomas with NUTM1 fusions may be amenable to treatment with NUT carcinoma-targeted therapy such as a bromodomain and extraterminal domain protein small molecular inhibitor (BETi).

A relapse of pemphigus vulgaris in pemphigus herpetiformis or a phenotypic “switch”

Sir, Pemphigus herpetiformis (PH) was originally described by Jablonska et al. in 1975. Clinically, PH presents itself as a herpetiform dermatitis with immunopathological characteristics of pemphigus [1,2]. We report an exceptional case of typical pemphigus vulgaris (PV) relapsing after 36 years in PH. A 65-year-old patient, followed for PV for 36 years and treated with corticosteroid therapy with a remission for more than thirty years, consulted for pruriginous lesions evolving for the previous eight months. A dermatological examination revealed urticariform pruriginous ring lesions surmounted by small peripheral vesicles spread throughout the body (Fig. 1), sparing the mucous membranes, and without Nikolsky’s sign. After two non-specific skin biopsies, the histological examination revealed an intraepidermal bubble with acantholytic cells and eosinophilic spongiosis (Figs. 2a and 2b). Direct immunofluorescence confirmed the diagnosis of pemphigus and indirect immunofluorescence was at the upper limit. The diagnosis of a PV relapse in PH was retained and a dapsone-based treatment was initiated at a dose of 150 mg/day and stopped seven days later when met with hemolytic anemia. Oral corticosteroid therapy involving prednisone at a dose of 1 mg/kg/day was initiated but, given the persistence of the pruritus, the decision was to combine methotrexate at a dose of 12.5 mg/week. A good evolution and a decline within eight months were observed. An improved pruritus and the disappearance of the skin lesions were achieved after one month of treatment. PV and PH are two different anatomical and clinical entities of the autoimmune disease pemphigus, with distinct clinical, histopathological, and immunopathological characteristics [1,2]. Our observation documents a complete phenotypic “switch” of pemphigus with a transition from PV to PH both clinically, histologically, and immunologically. Several rare cases of PV switching to superficial pemphigus (SP) (“phenotypic switch”) have, since 1991, been reported, with a higher frequency this direction than otherwise; the transition period varies from six months to twenty years [3]. To the best of our knowledge, no case has been described of a progression from PV to PH. Having observed one firsthand, we are first to describe the case of a complete phenotypic switch from PV to PH. The mechanism of such a transition remains poorly understood and is often observed during a relapse. Some authors suggest that the effect of immunosuppressants on the desmoglein DSG3 more marked than on DSG1 could explain the relapse of PS in PH [3,4]. Future studies on the immunological factors and predictors of PV relapses after the discontinuation of treatment would be useful to better understand the mechanisms of a relapse in pemphigus, with or without a phenotypic transition.

Mark's Story

The personal story of Mark Kent is not an easy one to read or to ignore. This chapter shares the difficult life story of Mark's journey from an individual who was sexually and emotionally abused as a child to one where he is currently in a healthy and happy marriage with four children. Mark attributes this success to his Asperger's Syndrome and the support of his family, but he also realizes he is an exceptional case study. He advocates for much better awareness, sexual education, and overall acceptance on the part of society to ensure others can overcome the same challenges he has faced.

Urban Education: High-Quality Education, the Economic Impact it Generates, and the Tragedy of Urban Education in the United States of America

Education is an investment in improving the quality of life for individuals, and therefore benefiting society in general. Education in the United States of America exemplifies a correlation between the level of an individual's education, their salary level due to those academic qualifications, and the impact they generate within themselves, their families, society, and within the nation. This contemporary study of education in the U.S. shows how a community in an urban center of Massachusetts made fundamental changes to transform the education of its students who historically experienced school failure. This exceptional case of leadership and altruism was accomplished with intensive influence from the State Government. This case can be the basis for educational initiatives that can have relevance and social impact anywhere in the world. At a global level, we can see that a society with high levels of education, based on knowledge and academic training, stands out for the multiple opportunities that arise to obtain a higher quality of life. The outcomes of a good education are beneficial to the growth of an entire society. Societies benefit from advances in technological and economic fields, health systems, construction and accessible housing, industry, commerce, and even social security.

Efficacy of Plasmapheresis in Nivolumab-Associated ANCA Glomerulonephritis: A Case Report and Pathophysiology Discussion

Immune checkpoint inhibitors (ICIs) have revolutionized solid organ and hematologic cancer treatments by improving overall prognoses. However, they can lead to overactivation of the immune system and several immune-related adverse events and sometimes affecting the renal system. Although acute interstitial nephritis is well described, we know little about ICI-associated glomerular injury. Herein, we report an exceptional case of renal ANCA positive-associated vasculitis (AAV) after nivolumab therapy. Three weeks after the last nivolumab injection, the patient presented with proteinuria at 1.73 g/g of creatininuria, hematuria, and acute kidney injury needing dialysis associated with lung hemorrhage; anti-neutrophil cytoplasmic antibody (ANCA titer ≥1,280 with myeloperoxidase specificity of 780 U/mL) was positive, and kidney biopsy confirmed glomerular injury with crescents. The patient underwent treatment with steroid pulses, rituximab, and plasmapheresis, resulting in an improvement of the renal function and lung hemorrhage and produced a negative ANCA titer. Despite the results of the PEXIVAS study and the absence of clear benefit of plasmapheresis demonstrated in idiopathic AAV, we suggest that drug-induced AAV may be effectively treated by plasmapheresis, steroids, and rituximab.

A Silent Corticotroph Pituitary Carcinoma: Lessons From an Exceptional Case Report

Nowadays, neither imaging nor pathology evaluation can accurately predict the aggressiveness or treatment resistance of pituitary tumors at diagnosis. However, histological examination can provide useful information that might alert clinicians about the nature of pituitary tumors. Here, we describe our experience with a silent corticothoph tumor with unusual pathology, aggressive local invasion and metastatic dissemination during follow-up. We present a 61-year-old man with third cranial nerve palsy at presentation due to invasive pituitary tumor. Subtotal surgical approach was performed with a diagnosis of silent corticotroph tumor but with unusual histological features (nuclear atypia, frequent multinucleation and mitotic figures, and Ki-67 labeling index up to 70%). After a rapid regrowth, a second surgical intervention achieved successful debulking. Temozolomide treatment followed by stereotactic fractionated radiotherapy associated with temozolomide successfully managed the primary tumor. However, sacral metastasis showed up 6 months after radiotherapy treatment. Due to aggressive distant behavior, a carboplatine-etoposide scheme was decided but the patient died of urinary sepsis 31 months after the first symptoms. Our case report shows how the presentation of a pituitary tumor with aggressive features should raise a suspicion of malignancy and the need of follow up by multidisciplinary team with experience in its management. Metastases may occur even if the primary tumor is well controlled.

A Rare Case of Adult-onset Still's Disease Revealed by Acute Myopericarditis

Background: Still's disease in adults is a systemic inflammatory pathology of unknown aetiology, characterized by clinical manifestations associating feverish peaks, arthritis or arthtralgia, transient rashes and hyperferritinemia. Currently, this disease remains a multisystemic disease with generally poor outcome, poorly described in the literature with very few studies unlike other rheumatic diseases, probably underdiagnosed due to its clinical polymorphism. Cardiac forms are quite rare and among the manifestations described, pericarditis remains the most reported entity, myocardial involvement is exceptional. The aim of our observation is to report an exceptional case of discovery of stille disease following a myo-pericarditis mimicking a coronary Sd initially.

An Exceptional Case of Simultaneous double ileoileal Intussusception Secondary to Burkitt’s Lymphoma

Intussusception is the telescoping or invagination of one segment of the bowel into its adjacent portion. It is common among children however it’s rare in adults, being seen in approximately only 5% of cases. In adults, it is the underlying cause of about 5% of bowel obstructions. More than half of these intussusceptions in adults are secondary to a neoplasm. Burkitt’s lymphoma is an uncommon cause of intussusception, in adults, less than 20 cases have been reported in the literature. Double intussusceptions due to Burkitt’s lymphoma are extremely rare. We present a case of a 20-year-old woman who presented symptoms of intestinal obstruction and was diagnosed with double ileoileal intussusception at Abdominal computed tomography and laparotomy exploration. The pathology of the lead points turned out to be Burkitt’s lymphoma. This case details a rare cause of intussusception secondary to Burkitt’s lymphoma. In infants, this is often treated with air enema reduction, but in adult populations, intussusceptions are normally associated with a lead point, therefore surgical management is essential. Hereby we present to you a second reported case in the literature of adult double intussusception due to Burkitt’s lymphoma. This report contains interesting diagnostic imaging, operative details, and specimen photographs.