Abstract
This article focuses on the famous skull collection of the German naturalist Johann Friedrich Blumenbach (1752–1840) to demonstrate that human remains in early modern scientific collections ...possessed an important but often underestimated economic value in addition to their well-known scientific and social worth. Blumenbach’s skulls are thus presented as a part of a vigorous early modern ‘necro-economy’ and as a product of its networks. The article highlights three strategies of enrichment which were used to create the value of these bones: reference to scientific values, the use of emotions and story-telling. The last two strategies in particular are relevant to present-day debates concerning the restitution of musealized human remains, for their analysis helps us deconstruct a perceptual bias that tends to contrast a Western and predominantly ‘scientific’ (and thus seemingly universal) value of collected human remains with theoretically ‘softer’ cultural values allegedly attributed to them in their countries of origin.
Besides his extraordinarily well-known collection of human skulls, the German anatomist and anthropologist Johann Friedrich Blumenbach (1752–1840) was an avid collector of human hair. Largely ...overlooked by historical research so far, Blumenbach’s samples of hair prove highly significant in the formation of eighteenth-century ideas about race. Among the samples, seven specimens of albino hair stand out. Up until the late eighteenth century, albinism had been understood as a singularly extra-European phenomenon of “white Negroes.” As such, albinism became deeply embedded in the evolution of theories of race. The evidence collected by Blumenbach through hair samples pointed, however, to another explanation: for Blumenbach, albinism was an illness and did not constitute a different human variety. Therefore, it did not challenge his theory about five human races.
This article will argue that Blumenbach’s supposedly more “scientific” way of dealing with people with extraordinarily fair skin and hair did indeed foster his system of racial thought. By splitting up his observations on anatomical things like hair and skulls into the two realms of anthropology and anatomy, Blumenbach succeeded in bypassing the “problem” of the “white Negro” by separating it as an illness apart from his attempt to categorize humans anthropologically. The story of Blumenbach’s albino hair samples therefore fits perfectly into the context of the formation of the life sciences in the eighteenth century. It provides proof not only of a disciplinary differentiation between anatomists and anthropologists but also of the ongoing racial entanglement of the sciences.
Objective
Despite advances in hematopoietic stem cell transplantation (HSCT), a considerable number of pediatric HSCT patients develops post-transplant complications requiring admission to the ...pediatric intensive care unit (PICU). The objective of this study was to evaluate clinical findings, PICU supportive therapy and outcome as well as predictive factors for 6-months survival after discharge of HSCT patients from PICU.
Study design
This retrospective single-center analysis investigated patient characteristics, microbiological findings, reasons for admission and death of 54 cases accounting for 94 admissions to the PICU of the University Children’s Hospital Tuebingen from 2002 to 2017. We compared clinical characteristics between children with and without 6-months survival after discharge from PICU following HSCT. Finally, we assessed the potential prognostic value of the oncological Pediatric Risk of Mortality Score (O-PRISM), the Pediatric Sequential Organ Failure Assessment Score (pSOFA) and the pRIFLE Criteria for Acute Kidney Injury for 6-months survival using Generalized Estimating Equations (GEE) and Receiver Operating Characteristic curves.
Results
Respiratory insufficiency, gastroenterological problems and sepsis were the most common reasons for PICU admission. Out of 54 patients, 38 (70%) died during or after their last PICU admission, 30% survived for at least six months. When considering only first PICU admissions, we could not determine prognostic factors for 6-months mortality. In contrast, under consideration of all PICU admissions in the GEE model, ventilation (p=0.03) and dialysis (p=0.007) were prognostic factors for 6-months mortality. Furthermore, pSOFA (p=0.04) and O-PRISM (p=0.02) were independent risk factors for 6-months mortality considering all PICU admissions.
Conclusion
Admission of HSCT patients to PICU is still associated with poor outcome and 69% of patients died within 6 months. Need for respiratory support and dialysis are associated with poor outcome. Prediction of 6-months survival is difficult, especially during a first PICU admission. However, on subsequent PICU admissions pSOFA and O-PRISM scores might be useful to predict mortality. These scores should be prospectively evaluated in further studies to verify whether they can identify pediatric HSCT recipients profiting most from transferal to the PICU.
DNA ligase IV deficiency is a rare autosomal recessive disorder caused by hypomorphic mutations in the DNA ligase IV (LIG4) gene. DNA ligase IV is an essential protein for the development of a ...healthy immune system as well as for the protection of genomic integrity. Apart from typical stigmata, patients with DNA ligase IV deficiency are characterized by progressive bone marrow failure and a predisposition to malignancy. To our knowledge this reported case is the first description of two brothers with ligase IV deficiency who are treated with different hematopoietic stem cell transplantation (HSCT) regimens resulting in vastly divergent outcomes.
The cases of two brothers suffering from severe recurrent infections and growth retardation are described. The laboratory findings showed pancytopenia with significant lymphopenia. The two boys were diagnosed with DNA ligase IV deficiency, associated with severe combined immunodeficiency (SCID). Both patients received HSCT from two different matched unrelated donors (MUD) at the age of 33 and 18 months. The older brother succumbed post-transplant due to fatal side-effects 143 days after allogeneic HSCT. The younger brother - conditioned with a different regimen - received a T cell depleted graft 4 months later. No severe side-effects occurred, neither post-transplant nor in the following years. Ten years after HSCT the patient is well off, living a normal life and attending a regular high school. His immune system is fully reconstituted, resulting in a maximum of T cell receptor (TCR) diversity, which is a prerequisite for immune competence. However, he still suffers from microcephaly, dwarfism and dystrophy.
This case report gives an example of a successful HSCT as a treatment option in a genetic disorder such as ligase IV deficiency, using a rather mild conditioning regimen. Further studies are required to determine the viability and efficacy of this treatment option.
An association between certain ABO/Rh blood groups and susceptibility to SARS-CoV-2 infection has been proposed for adults, although this remains controversial. In children and adolescents, the ...relationship is unclear due to a lack of robust data. Here, we investigated the association of ABO/Rh blood groups and SARS-CoV-2 in a multi-center study comprising 163 households with 281 children and 355 adults and at least one SARS-CoV-2 seropositive individual as determined by three independent assays as a proxy for previous infection. In line with previous findings, we found a higher frequency of blood group A (+ 6%) and a lower frequency of blood group O (-6%) among the SARS-CoV-2 seropositive adults compared to the seronegative ones. This trend was not seen in children. In contrast, SARS-CoV-2 seropositive children had a significantly lower frequency of Rh-positive blood groups. ABO compatibility did not seem to play a role in SARS-CoV-2 transmission within the families. A correction for family clusters was performed and estimated fixed effects of the blood group on the risk of SARS-CoV-2 seropositivity and symptomatic infection were determined. Although we found a different distribution of blood groups in seropositive individuals compared to the reference population, the risk of SARS-CoV-2 seropositivity or symptomatic infection was not increased in children or in adults with blood group A or AB versus O or B. Increasing age was the only parameter positively correlating with the risk of SARS-CoV-2 infection. In conclusion, specific ABO/Rh blood groups and ABO compatibility appear not to predispose for SARS-CoV-2 susceptibility in children.
This is the first published case report of a child with acute lymphatic leukemia developing a fatal mucormycosis during blinatumomab treatment. The patient showed multiple, systemic thromboembolic ...lesions with ischemia, bleeding and infarction in almost all organs. The child succumbed to increased brain pressure resulting in cerebral herniation. This case particularly illustrates the fulminant progression and huge challenges of diagnosing and treating mucormycosis in children with hemato-oncological diseases during treatment with targeted therapeutic antibodies (blinatumomab).
Summary
Background and Objectives
Progressive pigmented purpuric dermatosis (PPPD, Schamberg disease) is a rare benign, but chronic dermatosis frequently misdiagnosed as vasculitis or bleeding ...disorder. Although affected patients experience significant impairment in quality of life no effective treatment has been established. The aim of our two center case series was to evaluate efficacy and tolerability of the antioxidants rutoside and ascorbic acid as combination treatment for PPPD.
Patients and Methods
A retrospective review was performed on 35 patients with PPPD treated with 2 × 50 mg rutoside and 1 000 mg ascorbic acid daily between 2004 until 2011. The mean treatment duration was 8.2 months.
Results
71.4 % of the participants experienced complete clearance and 20.0 % an improvement of more than 50 %, accompanied by increased quality of life. Nine participants (25.1 %) relapsed after discontinuation. In seven, rutoside and ascorbic acid was re‐initiated, and all responded again. Only three participants reported mild adverse effects. Participants with shorter disease duration showed better therapeutic success, shorter time to response and lower risk of recurrence.
Conclusion
Oral rutoside and ascorbic acid may be an efficient and well tolerated treatment for PPPD. Early treatment is recommended to achieve best clinical outcome.
Völlerei, Sexualität, das Sezieren von Leichen oder verschwenderisches Zurschaustellen des eigenen Reichtums: Im Exzess wurde - so die These des Buches - gesellschaftliche Ordnung gestaltet. Wie ...produktiv das Agieren im Übermaß sein konnte, beleuchtet Sarah-Maria Schober an Medizinern, die um 1600 in Basel lebten. Sie folgt den Ärzten an Orte, an denen sie Grenzen austesteten: in Bäder und Haushalte, an ihre Schreibtische und auf die anatomische Bühne. Damit bietet sie zugleich eine quellennahe Analyse einer städtischen Gesellschaft im späten 16. und frühen 17. Jahrhundert.
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