Intraoperative transit time flow measurement predicts maturation of radiocephalic arteriovenous fistulas.

OBJECTIVE: The arteriovenous fistula (AVF) is the first choice for gaining vascular access for hemodialysis. However, 20% to 50% of AVFs fail within 4 months after creation. Although demographic risk factors have been described, there is little evidence on the intraoperative predictors of AVF maturation failure. The aim of this study was to assess the predictive value of intraoperative transit time flow measurements (TTFMs) on AVF maturation failure. METHODS: In this retrospective cohort study, intraoperative blood flow, measured using TTFM, was compared with AVF maturation after 6 weeks in 55 patients. Owing to its significantly higher prevalence and risk of nonmaturation, the radiocephalic AVF (RCAVF) was the main focus of this study. A recommended cutoff point for high vs low intraoperative blood flow was determined for RCAVFs, using a receiver operating characteristic curve. RESULTS: The average intraoperative blood flow in RCAVFs was 156 mL/min. Patients with an intraoperative blood flow equal or lower than the determined cutoff point of 160 mL/min, showed a 3.03 times increased risk of AVF maturation failure after 6 weeks, compared with patients with a higher intraoperative blood flow (P < .001). CONCLUSIONS: The intraoperative blood flow in RCAVFs measured by TTFM provides an adequate means of predicting AVF nonmaturation 6 weeks after surgery. For RCAVFs, a cutoff point for intraoperative blood flow of 160 mL/min is recommended for maximum sensitivity and specificity to predict AVF maturation failure after 6 weeks.

Phenotypically Discordant Anomalies in Conjoined Twins: Quirks of Nature Governed by Molecular Pathways?

A multitude of additional anomalies can be observed in virtually all types of symmetrical conjoined twins. These concomitant defects can be divided into different dysmorphological patterns. Some of these patterns reveal their etiological origin through their topographical location. The so-called shared anomalies are traceable to embryological adjustments and directly linked to the conjoined-twinning mechanism itself, inherently located within the boundaries of the coalescence area. In contrast, discordant patterns are anomalies present in only one of the twin members, intrinsically distant from the area of union. These dysmorphological entities are much more difficult to place in a developmental perspective, as it is presumed that conjoined twins share identical intra-uterine environments and intra-embryonic molecular and genetic footprints. However, their existence testifies that certain developmental fields and their respective developmental pathways take different routes in members of conjoined twins. This observation remains a poorly understood phenomenon. This article describes 69 cases of external discordant patterns within different types of otherwise symmetrical mono-umbilical conjoined twins and places them in a developmental perspective and a molecular framework. Gaining insights into the phenotypes and underlying (biochemical) mechanisms could potentially pave the way and generate novel etiological visions in the formation of conjoined twins itself.

History and highlights of the teratological collection in the Narrenturm, Vienna (Austria).

The collection of the Narrenturm in Vienna houses and maintains more than 50,000 objects including approximately 1200 teratological specimens; making it one of the biggest collections of specimens from human origin in Europe. The existence of this magnificent collection-representing an important resource for dysmorphology research, mostly awaiting contemporary diagnoses-is not widely known in the scientific community. Here, we show that the Narrenturm harbors a wealth of specimens with (exceptionally) rare congenital anomalies. These museums can be seen as physical repositories of human malformation, covering hundreds of years of dedicated collecting and preserving, thereby creating unique settings that can be used to expand our knowledge of developmental conditions that have to be preserved for future generations of scientists.

The pathological anatomical collection of the Natural History Museum Vienna.

The pathological anatomical collection Vienna (Pathologisch-Anatomische Sammlung Wien; PASW) is a living and still growing research collection. It was established as early as 1796 as part of the Medical University of Vienna, acquired the status of an independent federal museum in 1971, and was assigned to the Natural History Museum Vienna in 2012. It houses a wide range of human wet and dry specimens and further objects, such as moulages, medical devices, microbiological and histological specimens, and a photo archive (approximately 50,000 objects), which, as a meaningful source, may contribute to disclosing not only aspects of the medical history and the development of corresponding museums in Vienna, but is also considered a collection of cultural and current scientific relevance, quite comparable to today's biobanks. Most of the tissue amassment represents wet organic specimens and human skeletons or skeletal elements representing, e.g., congenital and metabolic disorders, infectious diseases, injuries, neoplasms, or musculoskeletal diseases, basically collected as descriptive anatomical teaching aids. This article reviews the current medical issues on which research has been and is being conducted by including PASW specimens (hereby using the ICD-10 code), and the extent to and ethical conditions under which this important heritage could be used as a reference collection for clinical and bioanthropological (paleopathological and palaeoepidemiological) studies; finally, this article reflects on the value and future research prospects, taking into account different positions and the ongoing discussions in pathological anatomical human tissue collections.

Archival influenza virus genomes from Europe reveal genomic variability during the 1918 pandemic.

The 1918 influenza pandemic was the deadliest respiratory pandemic of the 20th century and determined the genomic make-up of subsequent human influenza A viruses (IAV). Here, we analyze both the first 1918 IAV genomes from Europe and the first from samples prior to the autumn peak. 1918 IAV genomic diversity is consistent with a combination of local transmission and long-distance dispersal events. Comparison of genomes before and during the pandemic peak shows variation at two sites in the nucleoprotein gene associated with resistance to host antiviral response, pointing at a possible adaptation of 1918 IAV to humans. Finally, local molecular clock modeling suggests a pure pandemic descent of seasonal H1N1 IAV as an alternative to the hypothesis of origination through an intrasubtype reassortment.

Microarchitecture of historic bone samples with tuberculosis.

Tuberculosis is among the leading causes of death from infectious diseases and affects many organ systems, including the skeleton. Skeletal tuberculosis is an extrapulmonary stage of tuberculosis, which occurs after the early and post-primary pulmonary stages of the disease. The aim of our study was to assess the microarchitecture of historic dry bone samples of subjects who have died of tuberculosis documented by post-mortem examinations. These preparations date to the pre-antibiotic era, and were provided by the Pathological-Anatomical Collection in the "Fools Tower" of the Natural History Museum Vienna (PASiN-NHM).We investigated macerated samples of 20 vertebral bodies, 19 femoral heads, and 20 tibiae of a total of 59 individuals diagnosed with tuberculosis from the nineteenth and early twentieth century. 10 femora and 10 tibiae from body donors that did not exhibit signs of infection and 10 (unaffected) vertebrae kept at the PASiN-NHM were studied as controls. The affected regions of the bone samples (and the corresponding regions of the control bones) were analyzed by microcomputed tomography using a Viscom X 8060 II system. Obtained images were analyzed semi-quantitatively. In samples with tuberculosis, independent of the investigated skeletal region, trabecular defects and decreased trabecular thickness were observed. Cortical porosity was seen in affected vertebrae and tibia; in tuberculous tibiae (but not in the femora) cortical thickness was decreased. In half of the individuals, cortical sclerosis was present; signs of ankylosis were observed mainly at the femoral heads affected with tuberculosis. We conclude that a combination of several alterations at the trabecular compartment could be suggestive of the presence of tuberculosis in historic skeletal remains.

Micro-CT evaluation of historical human skulls presenting signs of syphilitic infection.

BACKGROUND: In tertiary syphilis, Treponema pallidum triggers the formation of granulomatous nodules in various organs of the human body. Within the skeleton, predominantly in the skull and long bones, these characteristic syphilitic lesions cause typical patterns of bone damage. In this study, micro-computed tomography (µ-CT) was used to assess the microarchitecture of these osseous defects in untreated syphilitic skull bones. MATERIAL AND METHODS: Bone structure of 30 macerated human skulls was noninvasively examined by means of µ-CT images (Viscom X8060 NDT). A total of 20 specimens showing typical morphological signs of syphilis were provided by the Collection of Anatomical Pathology of the Museum of Natural History in Vienna. They were compared to 10 macerated control skulls provided by the Division of Anatomy of the Medical University of Vienna. RESULTS: All samples affected by syphilis showed perforating defects and increased porosity. Furthermore, we observed sclerotic reorganization and complete loss of the cortical bone in 80% of infected cases. Cortical thinning occurred in 75%. CONCLUSION: Our findings revealed extensive micromorphological bone destruction and a broad variability of osseous manifestations of (tertiary) syphilis.

Characterizing the coalescence area of conjoined twins to elucidate congenital disorders in singletons.

Shared anomalies, always located close to the area of coalescence and observable in virtually every type of conjoined twinning, are currently seen as separate anomalies caused by mostly unknown and seemingly unrelated pathways rather than being connected to the twinning mechanism itself. Therefore, most (case) reports about conjoined twins are mere descriptions of (external) dysmorphologies lacking reflections on the possible origin of their concomitant anomalies. As we will demonstrate in this article, shared anomalies are influenced, and in some cases solely and sequentially explained, by interaction aplasia and neo-axial orientation; two embryological mechanisms to which each set of conjoined twins is subjected and are responsible for their ultimate phenotypical fate. In this review, we consider how the ventral, lateral and caudal conjunction types and their intermediates determine the phenotypic presentation of the twins, including patterns of shared malformations and anomalies, which in themselves can be indistinguishable from those encountered in singleton cases. Hence, it can be hypothesized that certain anomalies in singletons originate in a fashion similar to that in conjoined twins.

Craniopharyngioma adherence: a reappraisal of the evidence.

Craniopharyngioma (CP) adherence represents a most baffling problem for the neurosurgeon. The highest priority of current surgical treatment is to maximize tumor removal without compromising the patients' long-term functional outcome. Surgical damage to the hypothalamus may be avoided or at least ameliorated with a precise knowledge regarding the type of adherence for each case. This article presents a comprehensive review of the pathological, surgical, and radiological sources of evidence supporting that CP adherence, despite being heterogenous, is characterized by repeating patterns. The key underlying factors of CP adherence are also discussed. Three components define the type of adherence for each case: (i) the intracranial structures attached to the tumor, (ii) the adherence morphology, and (iii) the adhesion strength. Combination of these three components gives rise to five hierarchical levels of increased risk of hypothalamic injury during tumor removal. Tumor topography has been identified as the major predictor of the type of CP adherence. The most extensive and strongest adhesions to the hypothalamus occur in CPs originated in the suprasellar cistern that secondarily invade the third ventricle (secondary intraventricular CPs) and in those originated within the third ventricle floor itself (not-strictly intraventricular CPs). Three findings observed on preoperative conventional MRI scans have proven to be reliable predictors of adherence severity. A position of the hypothalamus around the middle portion of the tumor, an amputated pituitary stalk, and an elliptical tumor shape points to the severe and critical risk levels, and in those cases, a safer limited removal is strongly recommended.

Joseph Engel (1816-1899), author of a meaningful dissertation on tumors of the pituitary infundibulum: his report on the oldest preserved whole craniopharyngioma specimen.

Joseph Engel (1816-1899) was a Viennese anatomist and pathologist trained under the mentorship of Carl von Rokitansky (1804-1878), the man who laid the foundations of gross anatomical pathology. In 1839, Engel completed his first scientific project: the dissertation entitled "Über den Hirnanhang und den Trichter" (About the pituitary gland and the infundibulum). This work analyzed the pathological and clinical characteristics of the pituitary and infundibulum tumor specimens collected at the Vienna Pathologic-Anatomical Museum. This little-known work represents one of the earliest attempts to determine the function of the pituitary gland-infundibulum complex. Among the 12 pituitary/infundibulum tumors examined in Engel's dissertation, one of the cases (no. 10) was instrumental for the definition of hypophyseal duct tumors, or craniopharyngiomas (CPs). This huge cyst, approximately the size of a goose egg (6 × 6 × 4.5 cm), was found in 1828 during the autopsy of a 33-year-old patient who suffered from severe headache, blindness, apathy, and finally somnolence. The cyst had replaced the hypophysis and extended upwards into the hypothalamic region and downwards into the sphenoid sinus, its inferior pole protruding through the soft palate. In 1904, the Viennese pathologist Jakob Erdheim (1874-1937) re-examined this lesion and conclusively categorized it as a hypophyseal duct tumor after a detailed histological study. The original tumor specimen corresponding to this CP case is still preserved at the Narrenturm, the circular building within the old Allgemeines Krankenhaus (Vienna General Hospital) that today holds the pathological collections of Vienna's Federal Pathologic-Anatomical Museum. To the best of our knowledge, this tumor is very probably the oldest preserved whole CP specimen in the world. This paper presents a comprehensive review of Engel's dissertation, the pioneering pathological work on pituitary and infundibulum tumors which laid the groundwork for the proper clinical, topographic, and pathological categorization of craniopharyngiomas.

Learning from cases: Analysis of two cases of craniopharyngioma from the 19 th to the 21 st centuries.

This manuscript describes the study of two cases of craniopharyngioma, which have been examined repeatedly over three separate centuries. This includes analysis by Josef Engel in 1839, who sought to uncover the physiological role of the pituitary gland; Jacob Erdheim in 1904, who initially described the disease we now call craniopharyngioma, and recent high resolution MRI and micro-CT imaging and attempted DNA analyses of the tumours. The cases highlight how, rightly or wrongly, our interpretation of data is shaped by the technologies, methodologies and prevailing theories of a given time.

Cystic tumors of the pituitary infundibulum: seminal autopsy specimens (1899 to 1904) that allowed clinical-pathological craniopharyngioma characterization.

A heterogeneous group of epithelial cystic tumors developed at the infundibulum and the third ventricle disconcerted pathologists at the dawn of the twentieth century. Very little was known at that time about the physiological role played by the pituitary gland, and there was almost complete ignorance regarding the function of the hypothalamus. Acromegaly, or enlargement of acral body parts, described in 1886 by Pierre Marie, was the only disease linked to primary hypertrophies of the pituitary gland, known as "pituitary strumas". A growing number of young patients manifesting an unexplained combination of physical and mental symptoms, including absent or delayed sexual maturation, progressive obesity, abnormal somnolence, and dementia-like changes in behavior were reported to present large solid-cystic tumors which characteristically expanded within the infundibulum and third ventricle, above an anatomically intact pituitary gland. Between 1899 and 1904, five seminal autopsy studies from different countries thoroughly described the anatomical relationships and histological features of this newly recognized type of infundibular tumors. These cases were instrumental in fostering the systematic investigation of similar lesions by the Austrian pathologist Jakob Erdheim (1874-1937), who in 1904 was able to classify these infundibulo-tuberal cysts under the common category of hypophyseal duct tumors. The pioneering American neurosurgeon Harvey Cushing (1869-1939) unsuccessfully attempted to surgically remove one of these cysts, for the first time in history, in 1902. The term "craniopharyngioma", chosen by Cushing in 1929 to designate these lesions, would eventually prevail over Erdheim's more accurate denomination, which linked their origin to squamous cell remnants derived from the embryological structures that give rise to the pituitary gland. This paper presents a comprehensive, renewed account of the five clinical-pathological reports which laid the groundwork for the proper clinical diagnosis, topographic conceptualization and pathological categorization of craniopharyngiomas.

Giovanni Verga (1879-1923), author of a pioneering treatise on pituitary surgery: the foundations of this new field in Europe in the early 1900s.

The field of pituitary surgery was born in the first decade of the twentieth century in Europe, and it evolved rapidly with the development of numerous innovative surgical techniques by some of the founding fathers of neurosurgery. This study investigates the pioneering Italian treatise on pituitary surgery, La Patologia Chirurgica dell'Ipofisi (Surgical Pathology of the Hypophysis), published in 1911 by Giovanni Verga (1879-1923), a surgeon from Pavía and one of Golgi's disciples. This little-known monograph compiles the earliest experience on pituitary surgery through the analysis of the first 50 procedures performed between 1903 and 1911. We conducted a biographical survey of Giovanni Verga and the motivations for his work on pituitary surgery. In addition, a systematic analysis of all original reports and historical documents about these pituitary procedures referenced in Verga's treatise was carried out. Verga's treatise provides a summary of the techniques employed and surgical outcomes for the first 50 attempted procedures of pituitary tumor removal. This monograph is the only scientific source that includes a complete account of the series of 10 pituitary tumors operated on by Sir Victor Horsley in the 1900s. Three major types of surgery were employed: (i) palliative procedures of craniectomy (n = 6); (ii) transcranial approaches to the pituitary gland, either subfrontal or subtemporal (n = 13); and (iii) transphenoidal routes to expose the sella turcica, either using an upper transnasal-transethmoidal approach (n = 19) or a lower sublabial/endonasal-transeptal one (n = 12). An operative mortality rate of 36% (n = 17) was observed in these early series. The pathological nature of the tumors operated on was available in 42 cases. There were 28 adenomas and 15 craniopharyngiomas. Sir Victor Horsley (1857-1916) and the Viennese surgeons Anton von Eiselsberg (1860-1939) and Oskar Hirsch (1877-1965) were the leading European figures in the development of pituitary surgery. Giovanni Verga's treatise La Patologia Chirurgica dell'Ipofisi is a fundamental, pioneering book in the history of pituitary surgery, a work that compiles the foundations of this field in Europe and the only authoritative source providing a complete record of pituitary procedures performed by Sir Victor Horsley.

Description of in-hospital deaths in Vienna during 1850-2000.

The aim of this study was to compare in-hospital deaths in different hospital settings between 1850 and 2000 in Vienna. We reviewed 120 autopsy records for each of the selected years from the Clinical Institute of Pathology of the Medical University Vienna and two community hospitals. In 2000 the autopsy rate was 37.5 % at the community hospitals and 52.5 % at the university hospital. The mean age of those being dissected was significantly lower compared with those not being dissected in the community hospital. Infections were the leading cause of death during the nineteenth and early twentieth century, after 1950 the rate of cardiovascular diseases and cancer increased. In the year 2000 the majority of patients with an underlying malignant disease died because of cardiovascular disease. Causes of death vary between institutions. They should be reported as accurately as possible in order to create a cogent basis for central mortality statistics.

Jakob Erdheim (1874-1937): father of hypophyseal-duct tumors (craniopharyngiomas).

Jakob Erdheim (1874-1937) was a Viennese pathologist who identified and defined a category of pituitary tumors known as craniopharyngiomas. He named these lesions "hypophyseal duct tumors" (Hypophysenganggeschwülste), a term denoting their presumed origin from cell remnants of the hypophyseal duct, the embryological structure through which Rathke's pouch migrates to form part of the pituitary gland. He described the two histological varieties of these lesions as the adamantinomatous and the squamous-papillary types. He also classified the different topographies of craniopharyngiomas along the hypothalamus-pituitary axis. Finally, he provided the first substantial evidence for the functional role of the hypothalamus in the regulation of metabolism and sexual functions. Erdheim's monograph on hypophyseal duct tumors elicited interest in the clinical effects and diagnosis of pituitary tumors. It certainly contributed to the development of pituitary surgery and neuroendocrinology. Erdheim's work was greatly influenced by the philosophy and methods of research introduced to the Medical School of Vienna by the prominent pathologist Carl Rokitansky. Routine practice of autopsies in all patients dying at the Vienna Municipal Hospital (Allgemeines Krankenhaus), as well as the preservation of rare pathological specimens in a huge collection stored at the Pathological-Anatomical Museum, represented decisive policies for Erdheim's definition of a new category of epithelial hypophyseal growths. Because of the generalized use of the term craniopharyngioma, which replaced Erdheim's original denomination, his seminal work on hypophyseal duct tumors is only referenced in passing in most articles and monographs on this tumor. This article is intended to pay tribute to Erdheim's fundamental breakthroughs, his discovery of craniopharyngiomas and their functional damage to the hypothalamus. On these fundamental achievements, Jakob Erdheim should be recognized as the true father of craniopharyngiomas.

Ancient pathogens in museal dry bone specimens: analysis of paleocytology and aDNA.

Bone samples investigated in this study derive from the pathologic-anatomical collection of the Natural History Museum of Vienna. In order to explore the survival of treponemes and treponemal ancient DNA in museal dry bone specimens, we analyzed three individuals known to have been infected with Treponema pallidum pallidum. No reproducible evidence of surviving pathogen's ancient DNA (aDNA) was obtained, despite the highly sensitive extraction and amplification techniques (TPP15 and arp). Additionally, decalcification fluid of bone sections was smear stained with May-Gruenwald-Giemsa. The slides were examined using direct light microscope and dark field illumination. Remnants of spirochetal structures were detectable in every smear. Our results demonstrate that aDNA is unlikely to survive, but spirochetal remains are stainable and thus detectable.

[Karl Alfons Portele, Pathologist and first director of the Federal Pathologic-anatomical Museum Vienna]. / Karl Alfons Portele, Pathologe und erster Direktor des Pathologisch-anatomischen Bundesmuseums in Wien.

The importance of the work of Karl Alfons Portele (1912-1993) in his position as director of the Federal Museum of Pathology is discussed. Portele was commissioned with the museum in 1946 and separated it from university in 1974. The history of the Pathologic-anatomical Museum in Vienna is closely connected with the history of pathology.

[Electropathology in Vienna, an exhibition on accident prevention]. / Die Elektropathologie in Wien - eine Sammlung zur Unfallverhütung.

Since 1906, there is, apart from the period 2000-2009, in Vienna, a collection about the processes and consequences of accidents involving electricity. The purpose of this collection is to raise awareness of the dangers, and the presentation of appropriate safety devices. Both in the case of industrial accidents and leisure accidents, the risk source of electrical power is not negligible. Due to the different vulnerable groups, the availability of prevention work is difficult. The concept of the electro-pathological collection in Vienna has taken this into account.