bluuebunny/crossref_metadata_2025_split huggingface dataset:
Sickle Cell Nephropathy
Authors: Revuelta Katia López , Andres María Pilar Ricard 
Published: February 2019

Abstract
Sickle cell Disease (SCD) is the commonest and most serious inherited anemia in the world and its burden is expected to markedly increase in the next decades. Kidney disease (SCN) is one of the most frequent and severe complications of SCD having a high impact on SCD patient survival and quality of life. SCN phenotypic expression is very heterogeneous and standard markers of kidney damage may not be useful for the early detection of sickle cell disease. Tubular creatinine hypersecretion may mask significant renal impairment before serum creatinine rises, also proteinuria and Hypertension are late markers of irreversible CKD. Treatment with hidroxyurea with broader criteria of SCD patients and SCN screening with early referral to a specialist are needed to improve their survival and quality of life.
Cystic Kidney Diseases
Authors: Chebib Fouad T. , Torres Vicente E. 
Published: February 2019

Abstract
Autosomal dominant polycystic kidney disease (ADPKD), the most common monogenic kidney disease, is characterized by relentless development of kidney cysts, hypertension, and eventually end-stage renal disease. The enlargement of the bilateral kidney cysts is gradual throughout the lifetime of the patient until little renal parenchyma is recognizable. At that stage, the average rate of GFR decline is 4.4 to 5.9 mL/min/year. Over the past few years, several advancements in diagnosing, prognosticating, and understanding the pathogenesis of the disease have been made. The natural course of ADPKD makes it an ideal disease to be targeted for renal protection. This chapter discusses various aspects of pathophysiology and molecular pathways and addresses in details the various pharmaceutical and nonpharmaceutical interventions in the journey of prevention of clinical complications of ADPKD.
Cardiorenal Syndrome
Authors: Ross Edward A. , Batnyam Uyanga , Asmar Abdo M. 
Published: February 2019

Abstract
Renal and cardiac crosstalk plays an essential role in maintaining physiological homeostasis. Both organ diseases are prevalent and share common risk factors. Impairment in one organ has the potential to affect the other. This interaction is referred to as cardiorenal syndrome, and it is driven by complex neurohumoral and vascular processes. Cardiorenal syndrome refers to a state of either acute or chronic impairment of both renal and cardiac functions. Despite progress in therapeutic interventions, treatment of cardiorenal syndrome remains challenging. In this chapter, we review the current understanding of the pathophysiological mechanisms of cardiorenal syndrome, as well as its management options.
High-Risk Kidney Transplantation
Authors: Gomatos Ilias P. , Lerner Susan , Menon Madhav C. 
Published: February 2019

Abstract
Outcomes after solid organ transplantation have improved dramatically with the development of better surgical techniques and effective therapies for rejection. As a result, higher numbers of end-stage renal disease patients with hepatitis C, HIV, cardiovascular disease, and pretransplant tumor history are listed for a potential kidney transplant, posing increasing challenges for the transplant team. Modifying cardiovascular risk factors while awaiting or after transplantation, novel antiviral therapies, close follow-up, and tailored management of posttransplant malignancies are imperative to further improve long-term graft and patient survival in high-risk kidney transplants.
Urological investigations
Authors: Reynard John , Brewster Simon F , Biers Suzanne , Neal Naomi Laura 
Published: February 2019

Abstract
This chapter contains clearly titled sections covering the entire range of urological investigations, including assessment of renal function, urine investigations, and a range of imaging modalities. Ultrasound, computerized tomography scanning, magnetic resonance imaging, and isotope scans are discussed in detail. The fourth edition of this chapter also includes details of positron emission tomography scans and the indications for their use in urology, specifically in urological cancers. An overview of uroflowmetry, post-void residuals, and cystometry is also included. Detailed descriptions of each investigation are accompanied with illustrative examples, providing a clear overview of the wide range of urological investigations used in clinical practice.
Bladder outlet obstruction
Authors: Reynard John , Brewster Simon F , Biers Suzanne , Neal Naomi Laura 
Published: February 2019

Abstract
This chapter outlines the pathophysiology and causes of bladder outlet obstruction, along with diagnostic investigations and treatment options depending on the cause. Minimally invasive management of benign prostatic hyperplasia no longer includes thermotherapy or transurethral radiofrequency needle ablation, though these are briefly discussed by way of historical context. The new procedure of UroLift is discussed. Holmium laser enucleation of the prostate (HoLEP) has essentially replaced other forms of laser prostatectomy. Prostate artery embolization is increasingly used for troublesome prostatic bleeding. Management of acute and chronic forms of urinary retention are comprehensively discussed.
Urological neoplasia
Authors: Reynard John , Brewster Simon F , Biers Suzanne , Neal Naomi Laura 
Published: February 2019

Abstract
This chapter contains 61 clearly titled sections covering the basic science of neoplasia, radiological assessment and management of the benign renal mass, and the rapidly developing fields concerning the diagnosis and treatment of the entire spectrum of childhood and adult urological cancers. The fourth edition of this chapter significantly updates the previous edition, in particular the most important randomized trial data and management guidelines (published between 2013 and 2017) on diagnostic approaches, surveillance, and surgical and oncological treatments of organ-confined and advanced prostate, renal cell, and bladder cancers. All new data are referenced, often using online web links, and follow recommended guidelines from highly respected authorities, notably the United Kingdom’s National Institute of Health and Care Excellence (NICE) and the European Association of Urology (EAU).
Upper tract obstruction, loin pain, hydronephrosis
Authors: Reynard John , Brewster Simon F , Biers Suzanne , Neal Naomi Laura 
Published: February 2019

Abstract
This chapter covers upper tract obstruction, loin pain, and hydronephrosis. A comprehensive discussion of causes and management of unilateral and bilateral hydronephrosis is included. The pathophysiology of renal obstruction is detailed, including the physiology of urine flow from the kidney to the bladder. An overview of the management of non-pelviureteric junction obstruction (PUJO) ureteric strictures is included, including minimally invasive treatment options. The causes of retroperitoneal fibrosis are outlined, and this fourth edition includes the newer diagnosis of IgG4-related disease (causative in up to half of previously considered idiopathic cases). Common presentations, investigation, and management options are detailed, including both emergency management and longer-term treatments.
Urological surgery and equipment
Authors: Reynard John , Brewster Simon F , Biers Suzanne , Neal Naomi Laura 
Published: February 2019

Abstract
This chapter covers the principles of urological surgery and the equipment commonly used in urological procedures. This includes preoperative preparation, including the management of patients on anticoagulants, and the fourth edition includes novel oral anticoagulants. Technology such as catheters, lasers, stents, telescopes, and diathermy are all discussed in detail and at a level suitable for the FRCS(Urol) examinations. The principles of consent and the recent Montgomery ruling are included in the fourth edition. The surgical principles of a range of urological operations are outlined, ranging from inguinoscrotal procedures to pelvic oncology procedures, including reconstruction.
Arthrogryposis
Authors: Clark Robin D. , Curry Cynthia J. 
Published: February 2019

Abstract
This chapter reviews the incidence, risk factors, genetics, recurrence risk, and epidemiology of arthrogryposis. The most common and clinically recognizable type among the congenital contractures is amyoplasia, and treatment options are improving the quality of life for these patients. The distal arthrogryposes are mostly autosomal dominant and caused by an increasing number of genes including variants in PIEZ02 and MYH3 among several others. The lethal contracture syndromes have an expanding number of causes many of which are autosomal recessive. The majority of these conditions are recognized on US. In viable syndromes the early and intensive use of physical therapy is critical and can result in significant joint mobilization. The clinical case presentation features an infant with lethal multiple pterygia syndrome.
Skin
Authors: Clark Robin D. , Curry Cynthia J. 
Published: February 2019

Abstract
This chapter reviews the various types of congenital ichthyoses that can present in the newborn with erythroderma, hyperkeratosis, scales, or fissures. The presenting symptoms frequently overlap in these conditions. Erythroderma is a finding frequently seen in the ichthyoses but as well in infection, atopy, immunodeficiency and some metabolic disorders. Common ichthyoses include Harlequin ichthyosis, lamellar ichthyosis, epidermolytic ichthyosis and X-linkedLinked ichthyosis. Important syndromes include Sjogren Larsson, trichothiodystrophy, CHILD syndrome and Gaucher Disease type II. Lethal ichthyosis syndromes include Neu Laxova and Restrictive Dermopathy. Harlequin ichthyosis is also frequently lethal. Gene panels increasingly should be the first line diagnostic test. The clinical case presentation features an infant with Netherton syndrome.
Skin
Authors: Clark Robin D. , Curry Cynthia J. 
Published: February 2019

Abstract
This chapter reviews vascular malformations including the common vascular birthmark, nevus flammeus that is usually isolated, fades with time, and is of no clinical significance. The vascular birthmarks associated with complex segmental overgrowth are reviewed including Klippel Trenaunay syndrome, CLOVES, and the PIK3CA associated disorders. These disorders may sometimes not be evident in newborns although macrocephaly, apparent cutis marmorata, and limb asymmetry may suggest these diagnoses early. Cranial imaging and chronologic follow up help to establish the correct diagnosis. PHACES and SACRAL (LUMBAR) syndromes are distinctive but lack a known genetic cause. New treatment options for the PIK3CA disorders are emerging. The clinical case presentation features an infant with megalencephaly–capillary malformation–polymicrogyria syndrome (MCAP).
Jerusalem
Authors: Pioske Dan 
Published: February 2019

Jerusalem is the most important location in the Bible and the most researched within the realm of biblical studies. Already a Canaanite city of some standing by the Middle Bronze Age period (c. 2000–1550 bce), Jerusalem’s significance for the biblical writers begins in earnest with the portrayal of the city’s acquisition by David around 1000 bce. Jerusalem functions in the Hebrew Bible as the royal center for the House of David and the divine sanctuary for Yahweh, the God of Israel, for the next four centuries until, in 586 bce, the location is conquered and destroyed by the Babylonians. Around 515 bce the Second Temple is constructed and Jerusalem becomes a modest temple-city within the Persian Empire (c. 550–330 bce), during which time a number of biblical texts are written and revised. A century and a half after Alexander the Great’s conquest of the region, a Jewish group called the Maccabees revolted against the Greek rulers of the time (c. 167 bce) and established an autonomous kingdom with Jerusalem as its capital. The independent status of this kingdom ends, however, when the Roman general Pompey took control of Jerusalem in 63 bce and incorporated it and the region of Judea into the Roman Empire. During the reign of Herod the Great (37–4 bce), a client-king of the Romans, the temple in Jerusalem is extensively renovated and a number of other impressive building measures are carried out in the city’s precincts. This large, Roman city is the one referred to at various moments in the Gospels and in the Book of Acts, and it is the location where Jesus of Nazareth is said to have been crucified around 33 ce. Though Jerusalem is destroyed by the Romans in 70 ce after the First Jewish-Roman War, a new, heavenly Jerusalem is depicted in the later writings of the Book of Revelation.
Cities of Refuge
Authors: Spencer John R. 
Published: February 2019

Within the Hebrew Bible/Old Testament there is a provision for six cities of refuge (“cities of intaking” [ערי מקלט]), where someone who has unintentionally committed murder can go and not be subject to blood revenge (Exodus 21:12–14; Numbers 35:9–34; Deuteronomy 4:41–43, 19:1–13; Joshua 20; 1 Chronicles 6). This practice has been described as refuge, asylum, and sanctuary, and the cities have been given all three of these labels, which has resulted in differing understandings of the intention of these cities. The basic legal issue is the distinction between intentional and unintentional killing. For most societies in the ancient Near East, including ancient Israel, the idea of “blood revenge” (an “eye for an eye”; lex talionis) was the way in which the killing of a member of your clan or family was avenged (Exodus 21:23). The distinction made in association with the cities of refuge/asylum was how to deal with an individual who accidentally, without intention, killed another (Exodus 21:12–14; Number 35:16–28). Also associated with this idea is the nature of sanctuary or asylum that one can obtain when one reaches a cultic center with an altar (1 Kings 1:50–53; 2:23–24). One should also note that all the cities of refuge are also Levitical Cities (1 Chronicles 6), but it is not clear what the role of the Levites was in such a city of refuge. Among the issues associated with these cities are the following: Did they actually exist, or were they simply a fiction created at a later period of time? If they were real, what was their historical context? Was it premonarchic, the time of David and Solomon, related to the centralization of Josiah, or postexilic? When were the texts composed (a question associated with the previous issue and raising wonderings about different hands in the composition of the texts associated with the idea of asylum cities)? What is the connection between altars of sanctuary and the cities of refuge, and why the apparent replacement of altars with cities? Who and how was the validity of the claim of unintentional killing (Numbers 35:24–25; Joshua 20:4) decided, even if the killer was a “sojourner” (gēr) (Joshua 20:9) (see Oxford Bibliographies in Biblical Studies article Sojourner)? What was the consequence of the death of the high priest (Numbers 35:27; Joshua 20:6), and how it was related to some concept of atonement? What was the relationship between the different biblical presentations of refuge or asylum? What was the connection with the Levites (See Oxford Bibliographies in Biblical Studies articles Levi/Levites) and Levitical Cities (see Oxford Bibliographies in Biblical Studies article Levitical Cities)? Finally, what is the relevance to today’s society with its issues of sanctuary for immigrants and sojourners?
Rabindranath Tagore
Authors: van Bijlert Victor , Bangha Imre 
Published: February 2019

Rabindranath Tagore (in Bengali: Rabīndranāth Ṭhākur; b. 1861–d. 1941) was born in Calcutta, the capital of British India at the time. From the late 18th century onward, his extremely large family played an important role in the economic and cultural activities of the city and the whole of Bengal. Thus Rabindranath’s life and work was intimately connected to the urban humanist Bengali culture of which he was himself to become the prime representative. He was primarily known as a Bengali poet, perhaps the first really modern one. He published his earliest volumes when he was still a teenager. His very last poems he dictated in 1941 when on his deathbed. Composing poetry was his prime literary urge but by no means the only one. He successfully tried his hands at novels, short stories, plays, songs, literary essays, philosophy and liberal Hindu theology, introductory courses for Bengali grammar, travelogues, and an autobiography. Singlehandedly he created a complete modern universe of Bengali literature. Late-19th- and early-20th-century Bengali literature is dominated by the towering figure of Rabindranath. He was also an accomplished musical innovator, the creator of the so-called Rabindra-sangit: songs and dance dramas for which he also composed the music. Late in life he became known as an artist. He experimented with humanist forms of education for schoolchildren and university students. At Santiniketan (about 150 km from Calcutta in rural West Bengal) he founded a school (in 1901) and later the eponymous university (in 1921). Tagore’s global fame came as if by chance in 1913 when he was awarded the Nobel Prize for literature. This was on the basis of Gitanjali, a small English volume of prose poems. The booklet appeared in 1912 and knew overwhelming success; at that point Tagore was launched as an international writer and Indian prophet in one, a cult figure with a global impact. In order to live up to his Nobel Prize fame he began to bring out English poetry, essays, and drama. Much of this was based on or inspired by his Bengali originals. It has often been remarked that Tagore did not do his literary reputation a good service with his English translations. In the 1920s his international fame was withering away. Translations made directly from Bengali done by William Radice in 1985 caused a resurgence of global interest in Tagore as an important writer.
Latent Profiles of Cognitive Control, Episodic Memory, and Visual Perception Across Psychiatric Disorders Reveal a Dimensional Structure
Authors: Smucny Jason , Iosif Ana-Maria , Eaton Nicholas R , Lesh Tyler A , Ragland J Daniel , Barch Deanna M , Gold James M , Strauss Milton E , MacDonald Angus W , Silverstein Steven M , Carter Cameron S 
Published: February 2019

Abstract
Although meta-analyses suggest that schizophrenia (SZ) is associated with a more severe neurocognitive phenotype than mood disorders such as bipolar disorder, considerable between-subject heterogeneity exists in the phenotypic presentation of these deficits across mental illnesses. Indeed, it is unclear whether the processes that underlie cognitive dysfunction in these disorders are unique to each disease or represent a common neurobiological process that varies in severity. Here we used latent profile analysis (LPA) across 3 distinct cognitive domains (cognitive control, episodic memory, and visual integration; using data from the CNTRACS consortium) to identify distinct profiles of patients across psychotic illnesses. LPA was performed on a sample of 223 psychosis patients (59 with Type I bipolar disorder, 88 with SZ, and 76 with schizoaffective disorder). Seventy-three healthy control participants were included for comparison but were not included in sample LPA. Three latent profiles (“Low,” “Moderate,” and “High” ability) were identified as the underlying covariance across the 3 domains. The 3-profile solution provided highly similar fit to a single continuous factor extracted by confirmatory factor analysis, supporting a unidimensional structure. Diagnostic ratios did not significantly differ between profiles, suggesting that these profiles cross diagnostic boundaries (an exception being the Low ability profile, which had only one bipolar patient). Profile membership predicted Brief Psychiatric Rating Scale and Young Mania Rating Scale symptom severity as well as everyday communication skills independent of diagnosis. Biological, clinical and methodological implications of these findings are discussed.
Stable Spondylolisthesis
Authors: Chugh Arunit J.S. , Alonso Fernando , Moore Ryan , Cox Efrem , Eubanks Jason 
Published: February 2019

Study Design:
This is a retrospective cohort review.

Objective:
To determine whether a correlation exists between facet cysts and lumbar instability.

Summary of Background Data:
Lumbar facet cysts are common degenerative entities observed in patients with low-grade spondylolisthesis at a rate of 10%–50%. Surgical management with partial facetectomy with or without fusion remains controversial.

Methods:
Review of 86 patients with lumbar degenerative spondylolisthesis is performed. Preoperative magnetic resonance imagings were reviewed for the presence of lumbar facet cysts and Facet Fluid Indices (FFI)—calculated as the ratio of facet fluid width to facet width. Instability was defined as a difference of >3 mm in vertebral displacement between flexion and extension radiographs. T tests and the Pearson correlation analyses were used to determine statistical significance.

Results:
In total, 26 patients had unstable and 60 had stable spondylolisthesis. Facet cysts were present at an overall prevalence of 30.1%—in 10/26 patients in the unstable group and in 18/60 patients in the stable group (P>0.05). The average FFIs for the unstable and stable groups were 0.13 and 0.09, respectively (P>0.05). FFI in patients with facet cysts was significantly higher than those without (P<0.05). In addition, the group with facet cysts had a significantly higher proportion of patients with FF effusions >3 mm.

Conclusions:
Lack of correlation with instability hints that the presence of facet cysts may not indicate instability in lumbar degenerative spondylolisthesis. Therefore, presence of facet cysts in static magnetic resonance imaging revealing spondylolisthesis should not preclude the physician from performing dynamic films to evaluate for instability. Significantly higher FFI in patients with facet cysts reaffirms the degenerative pathophysiology involved in their formation. Although this is not an outcomes study, it does spark interest into whether patients with stable spondylolisthesis and concurrent facet cysts are suitable for partial facetectomy alone with fusion.