From chemistry to genomics: A concise history of the porphyrias.

We describe developments in understanding of the porphyrias associated with each step in the haem biosynthesis pathway and the role of individuals whose contributions led to major advances over the past 150 years. The first case of erythropoietic porphyria was reported in 1870, and the first with acute porphyria in 1889. Photosensitisation by porphyrin was confirmed by Meyer-Betz, who self-injected haematoporphyrin. Günther classified porphyrias into haematoporphyria acuta, acuta toxica, congenita and chronica. This was revised by Waldenström into porphyria congenita, acuta and cutanea tarda, with the latter describing those with late-onset skin lesions. Waldenström was the first to recognise porphobilinogen's association with acute porphyria, although its structure was not solved until 1953. Hans Fischer was awarded the Nobel prize in 1930 for solving the structure of porphyrins and the synthesis of haemin. After 1945, research by several groups elucidated the pathway of haem biosynthesis and its negative feedback regulation by haem. By 1961, following the work of Watson, Schmid, Rimington, Goldberg, Dean, Magnus and others, aided by the availability of modern techniques of porphyrin separation, six of the porphyrias were identified and classified as erythropoietic or hepatic. The seventh, 5-aminolaevulinate dehydratase deficiency porphyria, was described by Doss in 1979. The discovery of increased hepatic 5-aminolaevulinate synthase activity in acute porphyria led to development of haematin as a treatment for acute attacks. By 2000, all the haem biosynthesis genes were cloned, sequenced and assigned to chromosomes and disease-specific mutations identified in all inherited porphyrias. These advances have allowed definitive family studies and development of new treatments.

An eternal hunt for glaucoma.

In the first issue of Graefe's Archive from 1854, Albrecht von Graefe wrote about glaucoma. Glaucoma comes from the Greek word "glaukos," gleaming, which was first used by Homer around 800 BCE. Since then, glaukos and glaucoma have taken on many different meanings. The terms blindness, cataract and glaucoma were used interchangeably and twisted together in incomprehensible contexts. Over 2500 years of glaucoma theories were upset by the discovery of the ophthalmoscope in 1851. The first reports of increased intraocular pressure appeared in the mid-seventeenth century, but it took over 200 years for this elevated pressure to be accepted by the ophthalmological community. The discovery of glaucoma simplex in 1861 was an important step forward. What did doctors know about glaucoma before 1850 and why did it take so long to classify glaucoma in its various categories? And why is it that we still do not know what the cause is for primary open angle glaucoma? I will try to answer some of these questions after a historical overview.

[Sir William Osler: facets of personality].

William Osler is one of the most honored and influential physicians in the English medicine late 19th early 20th century, who has made a great contribution on development of medical science and it's teaching. The scope of his multifaceted personality goes far beyond the characteristics of an outstanding doctor and scientist. The historical essay presents various aspects of the Osler's personality, highlighting his uniqueness as a doctor, teacher and philosopher.

Historical Perspectives: Malignancy in Crohn's Disease and Ulcerative Colitis.

While both Crohn' disease (CD) and ulcerative colitis (UC) are known to predispose patients to certain intestinal malignancies, the exact mechanism of carcinogenesis remains unknown and optimal screening guidelines have not been established. This article will explore the history of our understanding of intestinal malignancy in inflammatory bowel disease (IBD). To contextualize the medical community's difficulty in linking each condition to cancer, the first section will review the discovery of CD and UC. Next, we discuss early attempts to define IBD's relationship with small bowel adenocarcinoma and colorectal cancer. The article concludes with a review of each disease's surgical history and the ways in which certain procedures produced poor oncologic outcomes.

[Gout: from Hippocrates till the modern time].

Gout (podagra) is one of the most ancient articular diseases. Its accurate mechanisms and causes were delineated only during the last century. Major historical investigatory steps are described in relation to causality and pathogenesis of the disease from Hippocrates ages till the modern time. The newest genetic and epidemiologic aspects of the disease are presented in this article.

[Rheumatoid factor: study history and concept evolution].

Rheumatoid factor became the first laboratory marker of rheumatoid arthritis and one of the first serological markers used to recognize the major autoimmune diseases. Details of the discovery with special regard to contribution of E. Waaler and H. Rose are presented in this historical review. Same assays used to exam the rheumatoid factor, its frequency and modern view on diagnostic significance in different diseases are described in this article.

Streptozotocin, 1982-2022: Forty Years from the FDA's Approval to Treat Pancreatic Neuroendocrine Tumors.

In May 1982, the US Food and Drug Administration (FDA) approved the use of streptozotocin to treat pancreatic neuroendocrine tumors (panNETs). Thus, this year marks 40 years since that landmark date. This review of streptozotocin to treat panNETs is intended to commemorate this anniversary. A historical perspective of the chemical structure, pharmacokinetics, and mechanism of action of streptozotocin is followed by data from prospective and retrospective clinical studies. The last section of the review addresses the latest aspects and takes note of the prospects that lie ahead on the future horizon of the use of streptozotocin to treat panNETs, including ongoing clinical trials.

When Is an Interview an Inter View? The Historical and Recent Development of Methodologies Used to Investigate Children's Astronomy Knowledge.

This paper provides a historical review of the interview research that has been used by science educators to investigate children's basic astronomy knowledge. A wide range of strategies have been developed over the last 120 years or so as successive teams of researchers have endeavoured to overcome the methodological difficulties that have arisen. Hence, it looks critically at the techniques that have been developed to tackle the problems associated with interviews, questionnaires and tests used to research cognitive development and knowledge acquisition. We examine those methodologies which seem to yield surer indications of how young people (at different ages) understand everyday astronomical phenomena-the field often referred to as children's cosmologies. Theoretical ideas from cognitive psychology, educational instruction and neuroscience are examined in depth and utilised to critique matters such as the importance of subject mastery and pedagogical content knowledge on the part of interviewers; the merits of multi-media techniques; the roles of open-ended vs. structured methods of interviewing; and the need always to recognise the dynamism of memory in interviewees. With illustrations and protocol excerpts drawn from recent studies, the paper points to what researchers might usefully tackle in the years ahead and the pitfalls to be avoided.

Historical review: a history of the British Journal of Haematology.

The British Journal of Haematology has now been in existence for 65 years. In this time it has served the international community as well as British haematology and has become increasingly international in its outlook. Its changing content has mirrored the changes in haematology over more than six decades, particularly with an increasing emphasis on haematological neoplasms and their treatment.

Epilepsy over the centuries: a disease survived at the time.

In this study, we have made a historical review of epilepsy through the centuries, from pre-Christian era to the present time. The epileptic was examined by Hippocrates, the first to recognize epilepsy as a disease and not as a supernatural manifestation called Morbus Sacer. The dark years of the Middle Ages were the worst for the disease, where the sufferer was even subjected to torture. The period of the Renaissance saw the epileptic isolated from society while the Enlightenment century improved the knowledge about the disease, thanks also to the first autopsies that showed post traumatic neurological lesions. However, some stigmata of the disease that prevented the marriage of epileptics persisted. It was the prelude to the years 1800-1900, characterized by Lombrosian concepts and Nazi convictions. Lombroso included epileptics among delinquents and criminals, identifying them as such on the basis of physical alterations. Nazi racism introduced the concept of eugenics excluding the epileptic from the so-called pure race. Today, epilepsy is considered a treatable neurological disorder. Morbus Sacer belongs to a remote past.

Review of infectious agent occurrence in wild salmonids in British Columbia, Canada.

Wild Pacific salmonids (WPS) are economically and culturally important to the Pacific North region. Most recently, some populations of WPS have been in decline. Of hypothesized factors contributing to the decline, infectious agents have been postulated to increase the risk of mortality in Pacific salmon. We present a literature review of both published journal and unpublished data to describe the distribution of infectious agents reported in wild Pacific salmonid populations in British Columbia (BC), Canada. We targeted 10 infectious agents, considered to potentially cause severe economic losses in Atlantic salmon or be of conservation concern for wild salmon in BC. The findings indicated a low frequency of infectious hematopoietic necrosis virus, piscine orthoreovirus, viral haemorrhagic septicaemia virus, Aeromonas salmonicida, Renibacterium salmoninarum, Piscirickettsia salmonis and other Rickettsia-like organisms, Yersinia ruckeri, Tenacibaculum maritimum and Moritella viscosa. No positive results were reported for infestations with Paramoeba perurans in peer-reviewed papers and the DFO Fish Pathology Program database. This review synthesizes existing information, as well as gaps therein, that can support the design and implementation of a long-term surveillance programme of infectious agents in wild salmonids in BC.

Taxonomy of Echinostoma revolutum and 37-Collar-Spined Echinostoma spp.: A Historical Review.

Echinostoma flukes armed with 37 collar spines on their head collar are called as 37-collar-spined Echinostoma spp. (group) or 'Echinostoma revolutum group'. At least 56 nominal species have been described in this group. However, many of them were morphologically close to and difficult to distinguish from the other, thus synonymized with the others. However, some of the synonymies were disagreed by other researchers, and taxonomic debates have been continued. Fortunately, recent development of molecular techniques, in particular, sequencing of the mitochondrial (nad1 and cox1) and nuclear genes (ITS region; ITS1-5.8S-ITS2), has enabled us to obtain highly useful data on phylogenetic relationships of these 37-collar-spined Echinostoma spp. Thus, 16 different species are currently acknowledged to be valid worldwide, which include E. revolutum, E. bolschewense, E. caproni, E. cinetorchis, E. deserticum, E. lindoense, E. luisreyi, E. mekongi, E. miyagawai, E. nasincovae, E. novaezealandense, E. paraensei, E. paraulum, E. robustum, E. trivolvis, and Echinostoma sp. IG of Georgieva et al., 2013. The validity of the other 10 species is retained until further evaluation, including molecular analyses; E. acuticauda, E. barbosai, E. chloephagae, E. echinatum, E. jurini, E. nudicaudatum, E. parvocirrus, E. pinnicaudatum, E. ralli, and E. rodriguesi. In this review, the history of discovery and taxonomic debates on these 26 valid or validity-retained species are briefly reviewed.

A steady decline in pancreas transplantation rates.

BACKGROUND/OBJECTIVES: After years of growth in many pancreas transplant programs, UNOS has reported declining transplant numbers in the USA. This precipitating trend urges for an evaluation of the transplant numbers and scientific productivity in the Eurotransplant region and the UK. METHODS: We performed a trend analysis of pancreas transplantation rates, between 1997 and 2016, adjusting for changes in population size, and an analysis of scientific publications in this field. We used information from the UNOS, Eurotransplant, and UK transplant registry and bibliometric information from the Web of Science database. RESULTS: Between 2004 and 2016 there was an average annual decline in pancreas transplantation rates per million inhabitants of 3.3% in the USA and 2.5% in the Eurotransplant region. In the UK, transplant numbers showed an average annual decline of 1.0% from 2009 to 2016. Publications in Q1 journals showed an annual change of -2.1% and +20.1%, before 2004, and a change of -3.8% and -5.5%, between 2004 and 2016, for USA and Eurotransplant publications, respectively. CONCLUSIONS: Adjusting pancreas transplantation rates for changes in population size showed a clear decline in transplant numbers in both the USA and Eurotransplant region, with first signs of decline in the UK. Following this trend, the number of scientific publications in this field have declined worldwide.

The history of Rhoton's Lab.

The work performed in Dr. Rhoton's Lab, represented by over 500 publications on microneurosurgical anatomy, greatly contributed to improving the level of neurosurgical treatment throughout the world. The authors reviewed the development and activities of the Lab over 40 years. Dr. Albert L. Rhoton Jr., the founder of, and leader in, this field, displayed great creativity and ingenuity during his life. He devoted himself to perfecting his study methodology, employing high-definition photos and slides to enhance the quality of his published papers. He dedicated his life to the education of neurosurgeons. His "lab team," which included microneuroanatomy research fellows, medical illustrators, lab directors, and secretaries, worked together under his leadership to develop the methods and techniques of anatomical study to complete over 160 microneurosurgical anatomy projects. The medical illustrators adapted computer technologies and integrated art and science in the field of microneurosurgical anatomy. Dr. Rhoton's fellows established methods of injecting colors and pursued a series of projects to innovate surgical approaches and instruments over a 40-year period. They also continued to help Dr. Rhoton to conduct international educational activities after returning to their home countries. Rhoton's Lab became a world-renowned anatomical lab as well as a microsurgical training center and generated the knowledge necessary to perform accurate, gentle, and safe surgery for the sake of patients.

Three decades of Cognition & Emotion: A brief review of past highlights and future prospects.

Over the past three decades, Cognition & Emotion has been one of the world's leading outlets for emotion research. In this article, we review past highlights of and future prospects for the journal. Our tour of history covers three periods: The first period, from 1987 to 1999, was a pioneering era in which cognitive theories began to be applied to the scientific analysis of emotion. The second period, from 2000 to 2007, was characterised by a sharp increase in the number of empirical research papers, a lot of which were concerned with automatic processing biases and their implications for clinical psychology. During the third period, from 2008 to 2017, a new focus emerged on self-regulatory processes and their implications for emotion. We then turn to the present profile of Cognition & Emotion and introduce our new editorial team. Finally, we consider how the journal's future success can be continued and increased by a) providing authors with fast and high-quality feedback; b) offering attractive publication formats, including the newly introduced Registered Reports for pre-registered studies; and c) consolidating key methodological paradigms with reproducible findings.

Injuries of the spine and of the spinal cord in the Hippocratic Corpus of medicine.

The purpose of this review article is to summarize the views presented in the Hippocratic Corpus of Medicine regarding injuries of the spine, their effect on the spinal cord and the optimal treatment modulus. It is shown that the treatment dilemmas of ancient times in these injuries remain modern since Hippocrates suggested that conservative treatment and letting nature take its course is preferable over a devastating surgical treatment if the spinal cord structural integrity is not compromised. There is also a detailed account of the conservative treatment suggested in the Hippocratic Corpus concerning devices and method of closed spinal relocations.

Historical review of arthroscopic surgery of the hip.

BACKGROUND & PURPOSE: Increasing our appreciation of the historical foundations of hip arthroscopy offers greater insight and understanding of the field's current and future applications. This article offers a broad history of the progress of hip arthroscopy. METHODS: Hip arthroscopy's development from the early technologies of endoscopy to the present day is described through a review of the available literature. RESULTS: Endoscopic science begins with the Lichtleiter, developed by Phillip Bozzini (1779-1809) in 1806, but endoscopes were not applied to joints until 1912, as presented by Severin Nordentoft (1866-1922). The work of Kenji Takagi (1888-1963), especially, was instrumental in the arthroscope's development, allowing Michael Burman (1901-75) to perform the first recorded hip arthroscopy, detailed in a 1931 paper after extensive cadaveric research. Although World War II stalled further development, a renewed application of fibre optics following post-war innovations in glass manufacture heralded the modern arthroscope's invention. During the 1970s hip arthroscopy was first mobilized for diagnosis and exploration, leading to its later adoption for therapeutic surgical interventions. Modern hip arthroscopy has been facilitated by international research into optimum distraction, portals of entry, positioning of patients, and the technology of arthroscopic instruments. In 2008, the International Society for Hip Arthroscopy (ISHA) was founded to represent this international expert community. CONCLUSIONS: Technology, communication and evidence-based medicine have jointly facilitated the development of this young but promising corner of Orthopaedics.

Corynebacterium diphtheriae in a free-roaming red fox: case report and historical review on diphtheria in animals.

OBJECTIVES: Corynebacterium diphtheriae, the classical causative agent of diphtheria, is considered to be nearly restricted to humans. Here we report the first finding of a non-toxigenic C. diphtheriae biovar belfanti strain in a free-roaming wild animal. METHODS: The strain obtained from the subcutis and mammary gland of a dead red fox (Vulpes vulpes) was characterized by biochemical and molecular methods including MALDI-TOF and Multi Locus Sequence Typing. Since C. diphtheriae infections of animals, usually with close contact to humans, are reported only very rarely, an intense review comprising also scientific literature from the beginning of the 20th century was performed. RESULTS: Besides the present case, only 11 previously reported C. diphtheriae animal infections could be verified using current scientific criteria. CONCLUSIONS: Our report is the first on the isolation of C. diphtheriae from a wildlife animal without any previous human contact. In contrast, the very few unambiguous publications on C. diphtheriae in animals referred to livestock or pet animals with close human contact. C. diphtheriae carriage in animals has to be considered as an exceptionally rare event.

Explanatory models for homeopathy: from the vital force to the current paradigm.

Facing claims for and against the scientific status of homeopathy, one is entitled to ask: is there a scientific model for homeopathy? In this study we reconstructed the model put forward by Hahnemann. The results showed that it was essentially based on the assumption of a 'vital force' exclusive to living beings. While the vital force was a basic element of 18th-century science, the existence of such a sui generis force of nature was refuted with the formulation of the law of the conservation of energy by mid-19th century. As a function of that fact for homeopathic theory, we discuss the history of the rise and demise of the theory of the vital force from the last quarter of the 18th century to 1830. Finally, we call the attention to the paradigm shift biology underwent starting at the end of the 19th century as the framework for contemporary views on the functioning of living beings and consequently, of the effects of pharmacological agents on them.

US health care system: we can do better.

In this review, South Atlantic Association of Obstetricians and Gynecologists President N. Donald Diebel examines a brief history of health care in the United States and shares his thoughts about questions raised in his 2015 presidential address: How can the enormous advances in medical science be extended to everyone and not just some? What are the obstacles or impediments to this task? How can they be overcome?