RESUMO O estudo objetivou identificar as definições e compreensões existentes sobre Medicamentos de Alto Custo (MAC) na produção científica e acadêmica brasileira relacionada à judicialização de medicamentos. A revisão de escopo seguiu a metodologia proposta pelo Joanna Briggs Institute. As bases bibliográficas Medline, Embase, Lilacs, Web of Science e Scopus, e a Biblioteca Digital de Teses e Dissertações foram pesquisadas em busca de estudos empíricos relacionados à judicialização de medicamentos que incluíssem MAC, publicadas ou defendidas no período de 2005–2022. Foram incluídos 62 artigos científicos e 66 dissertações e teses. Definições explícitas de MAC foram muito pouco frequentes nos estudos incluídos e estavam presentes em apenas 19,1% dos artigos e 15,2% dos produtos acadêmicos. Núcleos de significado mais presentes estavam relacionados ao alto custo unitário do medicamento ou medicamentos com valor total de tratamento elevado, decorrentes da cronicidade da doença, produzindo impacto financeiro no orçamento familiar. Outras relações de sentido foram: medicamentos ‘novos’, sem registro sanitário no País, fora das listas de financiamento, com foco em doenças raras e genéticas ou com especificidades relativas à sua aquisição e dispensação. A imprecisão conceitual identificada dificulta uma visão mais clara da importância desse grupo no cenário da judicialização de medicamentos no Brasil.

ABSTRACT The study aimed to single out definitions and interpretations regarding high-cost medicines in the scientific and academic literature on health litigation. A scoping review followed the methodology proposed by the Joanna Briggs Institute. MEDLINE, EMBASE, LILACS, Web of Science and Scopus, and the Digital Library of Dissertations and Theses were searched for empirical studies on litigation for access to medicines that included high-cost drugs, published or presented between 2005 and 2022. A total of 62 scientific papers and 66 dissertations and theses were selected. Explicit definitions of high-cost medicines were scarce, presented in just 19.1% of papers and 15.2% of the academic output. Among the most available core themes were those related to high unit or overall treatment cost of medicines, resulting from chronic conditions, and generating financial impact on household budgets. Other patterns of meaning that emerged were: ‘new’ medicines without marketing approval in the country, medicines absent from funding lists, drugs for genetic or rare diseases, or those with specificities regarding procurement or dispensing. Conceptual imprecision in the field of high-cost drugs hinders a clear viewpoint of the importance of this group of medicines in the scenario of health litigation in Brazil.

Ocular surface squamous neoplasia (OSSN) encompasses a spectrum of conjunctival tumours and, while rare globally, is the most common ocular malignancy in sub-Saharan Africa. Its rising incidence, primarily driven by the human immunodeficiency virus (HIV) epidemic, presents significant challenges in clinical diagnosis, as these lesions often share characteristics with other conjunctival lesions. In South Africa, where risk factors such as HIV, human papillomavirus infection and excessive sun exposure are prevalent, primary care clinicians play a crucial role in identifying and referring cases for early intervention. Ocular surface squamous neoplasia is often the first indication of HIV in patients who are otherwise unaware of their status when they present to healthcare workers, making it essential for healthcare workers to screen for HIV and initiate antiretroviral therapy. Early recognition of at-risk patients and prompt referral of suspicious lesions are imperative to improve patient outcomes and prevent vision loss.

The aim of this paper was to establish if mineral resource scarcity is a driver for South Africa to transition to a circular economy. The project objectives were achieved by investigating economically viable, and critical or strategic minerals remaining in South Africa, as well as resource scarcity, as a driver for South Africa to transition to a circular economy. The study found that some minerals in South Africa, such as iron ore, lead, manganese, gold, and cobalt have less than 50 years of economically viable mining remaining. At least 18 minerals can be classified indicatively as critical or strategic: aggregates, bauxite, chromite, cobalt, copper, gold, graphite, iron, limestone, lithium, manganese, nickel, phosphorous, platinum group metals, rare earth elements, silver, titanium, and vanadium. The classification was based on the minerals' economic importance, supply risk, and potential use in the development and manufacturing of emerging technologies. The findings suggest that the scarcity of mineral resources is a critical driver for the country to transition to a circular economy. Other identified drivers include socio-economic factors, climate change commitments, and business objectives. All minerals are non-renewable resources and at risk of depletion, therefore sustainable extraction and use are critical. Priority should be directed at investigating circular economy opportunities and substitutes for commodities at risk of being depleted within the next 50 years. There is an opportunity for South Africa to transition from a minerals export-based model to one that promotes local mineral beneficiation and manufacturing of end products.

Idiopathic non-cirrhotic portal hypertension is a rare diagnosis with an unknown aetiology. It is characterised by increased intrahepatic portal pressures in the absence of underlying liver disease. We present a unique case of a 32-year-old male patient who presented with a right hydrocele and an incidental finding of pancytopenia, massive splenomegaly, and features of portal hypertension. After known causes of portal hypertension were excluded, a liver biopsy was performed. Based on the liver biopsy findings, which demonstrated fibrotic portal tracts in the absence of cirrhosis and exclusion of other known causes of portal hypertension, our patient was diagnosed with idiopathic non-cirrhotic portal hypertension. Due to the persistent presence of hypersplenism and recurrent episodes of oesophageal variceal bleeding, our patient underwent a splenectomy, after which his features of portal hypertension resolved.

This article presents a rare case of a 26-year-old male admitted to the hospital following a mob assault and was initially diagnosed with an acute subdural hematoma. Following a sizable decompressive craniectomy, a subsequent complication of bi-frontal acute extradural hematoma was identified. We discuss this rare occurrence's pathophysiology, diagnosis, awareness, and management, shedding light on the challenges and considerations in such cases.

Leukocytoclastic vasculitis is a hypersensitivity reaction involving immune complex deposition in dermal capillaries and venules. We report a case of a 34-year-old female patient with left-sided breast carcinoma who developed vasculitis following autologous left breast augmentation, confirmed histologically. Various aspects of the patient's treatment history may be implicated in the development of her leukocytoclastic vasculitis, including chemotherapy and radiation therapy, antibiotics, and the presence of breast carcinoma (leukocytoclastic vasculitis as a paraneoplastic syndrome). Based on the temporal relationship, histological findings, and the site of these pruritic lesions, we postulate that autologous breast augmentation is the most likely aetiology for the leukocytoclastic vasculitis in our patient, an association not previously reported.

On 5 August 2014, the Orkney M5.5 earthquake, the largest in South African gold mining districts, occurred with an unusual strike-slip mechanism at great depth of 4.78 km below the surface. In a rare case, the M L 5.5 earthquake occurred within the area covered by the legacy 2D and 3D reflection seismic data acquired in the 1990s and 2000s for gold exploration and mine development. In addition, the earthquake ML 5.5 rupture plane was recorded and accurately delineated by the underground in-mine seismic network near the source region. The integration of the legacy 2D reflection seismic data and mine seismicity data allowed us to identify a near-vertical structure, striking northnorthwest-south southeast (NNW-SSE). The ICDP-DSeis team drilled three holes (Hole A, Hole B, and Hole C), and two holes (Hole B and Hole C), intersected the upper edges of the M L 5.5 rupture. These holes recovered metasediments, metabasalts, intrusive rocks including dolerite sills and lamprophyre dykes adjacent to the fault zone, and the fault gouge. Late Prof. Tullis Onstott and geomicrobiologists installed a packer in Hole A and successfully recovered saline water and detected gas (~10 MPa). Slip weakening and rupture propagation are significantly influenced by the existence of fault gouges and the production of wear material between two sliding rock surfaces. Using the fault gouge material recovered from Hole C, we conducted friction experiments under dry and room temperature conditions at high slip velocity of ~100 mm/s and normal stress of 2 MPa. The resulting steady-state frictional strength was ~0.66 over a slip weakening distance of ~9.1 m. The steady-state frictional strength was high, which may be caused by large gouge thickness, and the rate of wear generation. As a result, it is proposed that the Moab Khotsong ML 5.5 seismogenic zone is complex and could be controlled by three main processes: a) the complex structural architecture of the seismogenic zone (e.g., intersection of fault, lamprophyre dykes, and dolerite sills); b) the mechanical process induced by tectonic and/or mining related stresses; and c) the mechanical and chemical processes caused by the water and rock interaction.

Abstract The clothing industry in Brazil continues to lack historical research, particularly about its initial phase. Contributing to this situation is the scarcity of economic sources, in the most remote period, that allow for better clarification of issues such as: number of existing enterprises and the workforce used; available machinery, invested capital, produced volumes etc. This article comprises a panel on clothing serial manufacturing in the tropical's belle époque period - from the 1870s to 1922, the beginning of modernism - a time when the segment gained momentum in the country, especially after the First World War (1914-1918), still focused on simple and stable pieces, which was not the case with most women's clothing, which was more complex and influenced by changes in fashion. We highlight, in this context, the significant growth recorded by São Paulo, supported by research published in 1909 by Francisco Bandeira Junior, with rare data on São Paulo factories at the time. Mechanization - made possible by sewing machines, cutting machines etc. - lowered production costs, simultaneously with the increase in urban commerce, which expanded access to wearable goods that, however, remained socially differentiated by the quality and aesthetics of the pieces; that is, for the symbolic value of fashion.

Resumo A indústria de vestuário no Brasil segue carente de pesquisas históricas, em particular sobre sua fase inicial. Contribui para este quadro a escassez de fontes que permitam esclarecer sobre seu período mais remoto, questões como: número de empreendimentos e da mão de obra empregada; maquinários, capitais, volumes produzidos etc. Este artigo se propõe a compor um curto painel sobre o período da belle époque tropical (dos anos 1870 até a 1922) quando o segmento ganhou impulso no Estado de São Paulo, que passava a alavancar a industrialização do segmento no país, ainda voltado a peças de modelagens simples e estáveis. Para tanto, tomamos por suporte pesquisa realizada por Francisco Bandeira Junior em 1909, tempo em que a mecanização (máquinas de costura, de corte etc.) já permitia reduzir custos de produção, simultaneamente ao incremento do comércio urbano, que ampliava o acesso a bens vestíveis, socialmente diferenciados pela qualidade e estética das peças; ou seja, pelo valor simbólico da moda.

RESUMO Objetivo: A síndrome da deleção 3p é uma doença monossômica rara que engloba deleções em todo o braço curto do cromossomo 3. Frequentemente está na região distal (3p25-pter), mas apresenta variações nos pontos de quebra e uma manifestação clínica complexa, cujas cardiopatias congênitas são consideradas raras. Apresentamos aqui o primeiro caso de síndrome do coração esquerdo hipoplásico e características dismórficas menores na síndrome 3p-. Além disso, buscamos estabelecer uma associação gene-fenótipo. Descrição do caso: O diagnóstico foi feito por cariótipo e procedeu-se a uma investigação bibliográfica e análise bioinformática in silico sobre os possíveis genes associados a cardiopatias congênitas ou síndrome do coração esquerdo hipoplásico na síndrome da deleção 3p. Todos os genes analisados que poderiam afetar a formação do coração estão localizados na região 3p25.3, adjacente à região deletada no recém-nascido do nosso caso (3p26). Tendo em conta as limitações técnicas do cariótipo, a força da evidência de cada gene avaliado e a proximidade do locus, é provável que tenha ocorrido uma quebra parcial não identificada no gene CAV3. Comentários: Identificamos uma relação indireta entre o gene CAV3 e a síndrome do coração hipoplásico esquerdo, em razão da sua forte associação com cardiomiopatias e defeitos cardíacos isolados. Além disso, a banda citogenética do nosso caso é uma informação nova para a delimitação de uma região cardíaca crítica na síndrome 3p, discussão que está em andamento desde 1986. Assim, reforçamos a importância da investigação citogenética em pacientes com coração hipoplásico e dismorfias auxiliando no diagnóstico, definição de prognóstico e aconselhamento genético para a família.

ABSTRACT Objective: 3p deletion syndrome is a rare monosomal disease that encompasses deletions throughout the short arm of chromosome 3. It is often in the distal region (3p25-pter), but variations in breakpoints and a complex clinical manifestation exist, with congenital heart defects being considered rare. We present the first case of hypoplastic left heart syndrome and minor dysmorphic features associated with 3p- syndrome. Furthermore, we aim to establish a gene-phenotype association. Case description: The diagnosis was made by karyotyping, followed by a literature investigation and in silico bioinformatic analysis about the possible candidate genes associated with congenital heart defects or hypoplastic left heart syndrome in 3p- syndrome. All genes analyzed that could affect heart formation are located in the 3p25.3 region, adjacent to the deleted region in the newborn from our case (3p26). Taking into account the technical limitations of the karyotype and the strength of evidence from each gene evaluated and locus proximity, it is likely that an unidentified partial break in the CAV3 gene occurred. Comments: We identified an indirect relation between gene CAV3 and hypoplastic left heart syndrome due to its strong association with cardiomyopathies and isolated cardiac defects. Furthermore, the cytogenetic band from our case is new information for the delimitation of a critical cardiac region on 3p syndrome, a discussion that has been ongoing since 1986. Thus, we reinforce the importance of cytogenetic investigation in patients with hypoplastic hearts and dysmorphia, assisting in diagnosis, definition of prognosis, and genetic counseling for the family.

RESUMO Objetivo: Relatar o caso de uma criança com síndrome de Jacobsen visando fornecer informações fenotípicas sobre esta desordem genética rara. Descrição do caso: Pré-escolar do sexo feminino, com cinco anos de idade, foi diagnosticada com síndrome de Jacobsen por meio do teste do cariótipo. Apresentou uma variedade de anomalias craniofaciais e malformações, incluindo comprometimento cardíaco, caracterizado por um conjunto de malformações no ventrículo esquerdo em consonância com o diagnóstico do complexo de Shone. Comentários: A síndrome de Jacobsen ocorre em razão de uma deleção de genes contíguos no braço longo do cromossomo 11 (11q). As principais características associadas a esta síndrome genética são baixa estatura, atraso do desenvolvimento neuropsicomotor, trigonocefalia e dismorfismo craniofacial, além de alterações hematológicas, malformações cardíacas, entre outras. Dessa forma, a criança está em acompanhamento em um Centro de Anomalias Craniofaciais com uma equipe multiprofissional a fim de acompanhar seu desenvolvimento e tratar as comorbidades associadas.

ABSTRACT Objective: The aim of this study was to report the case of a child with Jacobsen syndrome in order to provide phenotypic information about this rare genetic disorder. Case description: A 5-year-old female preschooler was diagnosed with Jacobsen syndrome by karyotype testing. She presented with a variety of craniofacial anomalies and malformations, including cardiac impairment, characterized by a cluster of malformations in the left ventricle in line with the diagnosis of Shone’s complex. Comments: Jacobsen syndrome occurs due to a deletion of contiguous genes on the long arm of chromosome 11 (11q). The main characteristics associated with this genetic disorder are short stature and delayed neuropsychomotor development, trigonocephaly and craniofacial dysmorphism, hematological alterations, and cardiac malformations, among others. Thus, the child is being monitored at a Craniofacial Anomalies Center with a multi-professional team in order to monitor her development and treat the associated comorbidities.

Abstract Pterosaur remains are rare from the lowermost Cretaceous, hampering our understanding of the taxonomic and morphological diversities of pterosaurs during this period. The Lower Cretaceous Tugulu Group in Wuerho, China is renowned for hosting the Wuerho Pterosaurian Fauna (WPF), which has so far yielded numerous fossil remains of two dsungaripterid pterosaurs, Dsungaripterus weii and Noripterus complicidens. Here we report a partial ornithocheiromorph humerus from the WPF, representing a deeply divergent clade from Dsungaripteridae. The scarcity of ornithocheiromorphs from the WPF might be interpreted by niche partitioning with dsungaripterids. Meanwhile, we also report a U-Pb zircon age of 134.27 ± 0.36 Ma dated by LA-ICP-MS for the tuffaceous layer at the uppermost part of the Shengjinkou Formation, confirming the Valanginian age of the WPF presented by a previous study. The Wuerho region is one of the few localities producing abundant pterosaur fossils and the only one with an earliest Cretaceous age. The new finding here also suggests that Ornithocheiromorpha had rapidly diversified and achieved a global distribution during the earliest Cretaceous, presumably through a series of modifications on the locomotor apparatus including the warped deltopectoral crest of the humerus, which might substantially improve their flight efficiency.