bluuebunny/crossref_metadata_2025 huggingface dataset:
Acute kidney injury in the tropics
Authors: Jha Vivekanand
Published: November 2015

The spectrum of acute kidney injury (AKI) encountered in the hospitals of the tropical zone countries is different from that seen in the non-tropical climate countries, most of which are high-income countries. The difference is explained in large part by the influence of environment on the epidemiology of human disease. The key features of geographic regions falling in the tropical zones are climatic, that is, high temperatures and absence of winter frost, and economic, that is, lower levels of income. The causes and presentation of tropical AKI reflect these prevailing cultural, socioeconomic, climatic, and eco-biological characteristics.

Peculiarities of tropical climate support the propagation of several infectious organisms that can cause AKI and the disease-transmitting vectors. In contrast to the developed world, where AKI usually develops in already hospitalized patients with multiorgan problems and iatrogenic factors play a major role, tropical AKI is acquired in the community due to issues of public health importance such as safe water, sanitation, infection control, and good obstetric practices. Infections such as malaria, leptospirosis, typhus, HIV, and diarrhoeal diseases; envenomation by animals or insects; ingestion of toxic herbs or chemicals; intravascular haemolysis; poisoning; and obstetric complications form the bulk of AKI in the tropics. Poor access to modern medical facilities and practices such as seeking treatment from traditional faith-healers contribute to poor outcomes.

AKI extracts macro- and microeconomic costs from the affected population and reduces productivity. Improvement in the outcomes of tropical AKI requires improvement in basic public health through effective interventions, and accessibility to effective medical care.
Acute kidney injury in heart failure
Authors: Cruz Dinna N. , Giuliani Anna , Ronco Claudio
Published: November 2015

Acute kidney injury (AKI) occurring during heart failure (HF) has been labelled cardiorenal syndrome (CRS) type 1. CRS is defined as a group of ‘disorders of the heart and kidneys whereby acute or chronic dysfunction in one organ may induce acute or chronic dysfunction of the other’. This consensus definition was proposed by the Acute Dialysis Quality Initiative, with the aim to standardize those disorders where cardiac and renal diseases coexist. Five subtypes have been proposed, according to which organ is affected first (cardiac vs renal) and whether the dysfunction is acute or chronic. Another subtype which includes systemic conditions leading to both heart and kidney dysfunction is also described.

The term ‘worsening renal function’ has been regularly used to describe the acute and/or subacute changes that occur in the kidneys following HF. However, the AKI classification according to the current consensus definition better represents the entire spectrum of AKI in the setting of HF.

The pathophysiology of heart–kidney interaction is complex and still poorly understood. Factors beyond the classic haemodynamic mechanisms appear to be involved: neurohormonal activation, venous congestion, and inflammation have all been implicated.

Diuretics are still a cornerstone in the management of HF. Intravenous administration by bolus or continuous infusion appears to be equally efficacious. Biomarkers and bioelectrical impedance analysis can be helpful in estimating the real volume overload and may be useful to predict and avoid AKI. The role of ultrafiltration remains controversial, and it is currently recommended only for diuretic-resistant patients as it has not been associated with better outcomes. The occurrence of AKI during HF is associated with substantially greater short- and long-term mortality.
Acute kidney injury in the cancer patient
Authors: Moeckel Gilbert W. , Manjunath Veena , Perazella Mark A.
Published: November 2015

Acute kidney injury in cancer patients is a complicated clinical condition associated with significant morbidity and mortality, especially in the hospital setting. Cancer patients may develop a variety of different kidney lesions that impair not only immediate survival but also limit the adequate treatment of the underlying malignant process. This poses a significant challenge for clinicians.

The mechanisms that lead to acute kidney injury in cancer patients are similar to those seen in non-cancer patients. Moreover, significant morbidity is seen in association with chemotherapy, as well as through direct effects of the cancer on the kidney (i.e. obstruction, infiltrate).

This chapter reviews the clinical presentation of the most common malignancies that affect the kidney, discusses their pathologic manifestations in kidney tissue, and reviews options for the clinical management of cancer patients with acute kidney injury.
Haemodialysis
Authors: Bieber Scott D. , Himmelfarb Jonathan
Published: November 2015

The development of haemodialysis for the treatment of chronic kidney disease was a remarkable step in medicine that moved what was once a universally fatal organ failure to a condition that is regarded as treatable. Over the decades since that remarkable advancement, mechanical methods of blood purification to correct the uraemic condition have gained a prominent and often expected role in the care of the patient with end-stage kidney failure. Even so, patients with end-stage kidney disease still experience high rates of morbidity and mortality, at times surpassing other chronic conditions such as cancer. The goal of haemodialysis should be not only to maintain life but also to restore the afflicted individual to a state of health, thus rehabilitating them so that they can lead a meaningful, fulfilling life. Currently utilized methods of haemodialysis, while effective at acutely reversing the uraemic condition, often fall short of the goal of rehabilitation. This observation, among others, has led many scientists and physicians to suspect that contemporary dialytic therapy is inadequate and has led to vigorous pursuit of the question: what is the adequate dose of dialysis? While extensive effort has been devoted to the pursuit of this question, it has yet to be definitively answered to the satisfaction of the scientific community. This chapter will predominantly focus on currently popularized and frequently utilized methods for measurement of dialysis dose with the stipulation that the reader understands that the determination of the adequate dose of dialysis is an evolving field and in clinical practice should require more diligence than simple surveillance of urea clearance. The adequacy of volume management, which is arguably of equal importance to the adequacy of uraemic retention solute clearance is covered in other chapters within this book.
Peritoneal dialysis
Authors: Mehrotra Rajnish , Himmelfarb Jonathan
Published: November 2015

Peritoneal dialysis (PD) requires the periodic instillation of dialysate into the peritoneal cavity and induces the movement of solutes and fluid across the semipermeable peritoneal membrane that allows for the successful management of the uraemic syndrome. Even though this was recognized as far back as 1923, technical limitations precluded the large-scale use of PD for the long-term treatment of end-stage renal disease for 50 years. This changed with the development of an indwelling catheter for PD by Henry Tenckhoff in the late 1960s, the description of the technique of continuous ambulatory peritoneal dialysis by Popovich and colleagues in 1976, and the introduction of plastic bags in lieu of glass bottles in the mid 1970s. It is estimated that over 200,000 patients are undergoing treatment with PD worldwide, accounting for about 15% of the international dialysis population.
Peritoneal dialysis
Authors: Saxena Anjali Bhatt
Published: November 2015

Dialysis adequacy is a term used to describe how well any dialysis therapy effectively mitigates some of the uraemic complications of end-stage renal disease. In the simplest terms, dialysis adequacy measures the dose of dialysis and judges it to be sufficient (adequate) or insufficient (inadequate). In peritoneal dialysis, adequacy refers to the ability of dialysis to perform any or all of myriad tasks including (a) removing metabolic waste products, (b) maintaining proper fluid balance and blood pressure control, (c) removing excess electrolytes, (d) correcting acid–base imbalances, (e) maintaining healthy bone mineral metabolism, and (f) promoting the maintenance of a proper nutritional status. In practice, peritoneal dialysis adequacy is most often measured mono-dimensionally, in terms of small solute (i.e. urea) clearances; however, it is most useful to incorporate a wider view of dialysis adequacy when caring for patients with end-stage renal disease.
Cognitive function, depression, and psychosocial adaptation
Authors: Tamura Manjula Kurella , Unruh Mark L. , Kang Ea Wha
Published: November 2015

Psychiatric complications of end-stage renal disease are common, often debilitating, and potentially preventable. Patients with end-stage renal disease are at higher risk for psychiatric disorders compared to patients with other chronic health conditions, and those who suffer from psychiatric complications are at higher risk for death and dialysis withdrawal. Both dementia and depression also reduce quality of life and impair adherence to prescribed therapies. In addition, patients with end-stage renal disease are confronted with multiple stressors related to their illness and treatment. This chapter reviews the clinical approach to cognitive impairment, depression, and psychosocial adaptation among patients with end-stage renal disease.
Pre-transplant assessment of the recipient
Authors: Legendre Christophe
Published: November 2015

Recipient assessment pre transplant is a critical step in the overall process of transplantation. It is required to define the surgical and medical strategies, evaluate the prognosis based on the knowledge of global kidney transplantation results, and hopefully improve the individual’s outcome. Each transplant centre will have developed its own experience and its own way of assessing transplant candidates. This chapter describes the transplant candidate assessment according to recognized recommendations as well as the author’s local experience at Necker Hospital, Paris, France. The goals of pre-transplant candidate assessment are to discuss the indications, to fully inform the patient, to determine which tests are useful to help balance risks and benefits, and finally to prepare as many potential kidney transplant recipients as possible to be wait-listed successfully.
Renal transplant imaging
Authors: Gruenewald Simon , Vladica Philip
Published: November 2015

The purpose of imaging of the transplant kidney is to assess integrity, anatomy, and function. Relatively or actually non-invasive technologies can be used to monitor for potential early post-transplant complications such as acute tubular necrosis, acute rejection, haematoma, pyelonephritis, abscess, urinoma, ureteral obstruction, vascular complications, and rarely graft torsion. The technologies also assist in the diagnosis and management of late complications such as those arising from immunosuppression, chronic rejection, lymphocoele, cyst, renal artery stenosis, urinary obstruction, and tumours. This chapter demonstrates the capacity of the various imaging modalities such as ultrasound, computed tomography, and magnetic resonance imaging, to assist in the clinical management of the renal transplant recipient.
Chronic allograft dysfunction
Authors: Henderson Lorna K. , Nankivell Brian J. , Chapman Jeremy R.
Published: November 2015

Despite improvements in short-term renal allograft survival, long-term survival has not appreciably changed. Excepting death with a functioning graft, most late graft loss results from chronic allograft dysfunction. Immune and non-immune-mediated injuries contribute to graft dysfunction over time, ultimately leading to a non-specific and irreversible histological end-point of fibrosis, tubular atrophy, and glomerulosclerosis. Screening and early identification of pathology is crucial to allow timely intervention in order to prevent permanent nephron damage and graft loss. This chapter outlines assessment of renal dysfunction following transplantation, defines the causes of chronic allograft failure, and their pathophysiology, and evaluates current therapeutic strategies used to improve or stabilize chronic allograft dysfunction.
The adolescent with renal disease
Authors: Marks Stephen D.
Published: November 2015

Successful transitioning involves input from both paediatric and adult multidisciplinary teams with overlap between the two services and tailored to the needs of each individual. This includes varying the duration of the preparatory phase and the timing of transition and eventual transfer based on the chronological and developmental age, maturity, medical stability, and psychosocial issues of each individual patient. Buddy systems and peer support may aid smooth transitioning for some, with the promotion of patients attending a clinic with similar age groups, which can include the formation of a young adult clinic. Effective collaboration between children’s and adult services is required to obtain successful transition of adolescent patients. Moving from one paediatric hospital to different adult specialists in different hospitals requires excellent communication between various teams. Some staff members from each adult nephrology unit should specialize in adolescent medicine and construct their own transitional care pathways to ensure effective communication and collaboration with appropriate paediatric units and facilitate continuity of care with ongoing educational and social programmes.
The molecular basis of ciliopathies and cyst formation
Authors: Kühn Wolfgang , Walz Gerd
Published: November 2015

Abnormalities of the cilium, termed ‘ciliopathies’, are the prime suspect in the pathogenesis of renal cyst formation because the gene products of cystic disease-causing genes localize to them, or near them. However, we only partially understand how cilia maintain the geometry of kidney tubules, and how abnormal cilia lead to renal cysts, and the diverse range of diseases attributed to them. Some non-cystic diseases share pathology of the same structures. Although still incompletely understood, cilia appear to orient cells in response to extracellular cues to maintain the overall geometry of a tissue, thereby intersecting with the planar cell polarity (PCP) pathway and the actin cytoskeleton. The PCP pathway controls two morphogenetic programmes, oriented cell division (OCD) and convergent extension (CE) through cell intercalation that both seem to play a critical role in cyst formation. The two-hit theory of cystogenesis, by which loss of the second normal allele causes tubular epithelial cells to form kidney cysts, has been largely borne out. Additional hits and influences may better explain the rate of cyst formation and inter-individual differences in disease progression. Ciliary defects appear to converge on overlapping signalling modules, including mammalian target of rapamycin and cAMP pathways, which can be targeted to treat human cystic kidney disease irrespective of the underlying gene mutation.
The adult with renal cysts
Authors: Pirson Yves , Devuyst Olivier
Published: November 2015

Cysts in children are unusual except in the context of congenital or inherited disease, but simple cysts become more common with age. So do the cysts of the most common inherited renal disease, autosomal dominant polycystic kidney disease, so number of cysts, age, family history, and associated findings are all relevant. Localization and size, shape of cysts may point to other diagnoses as discussed in this chapter.
Alport syndrome
Authors: Heidet Laurence , Knebelmann Bertrand , Claire Gubler Marie
Published: November 2015

Management of Alport syndrome has in the past been expectant and supportive. Modern hearing aids have substantially improved the function of affected individuals. However, animal data and more recently observational data from Alport registries strongly suggest a protective effect of angiotensin-converting enzyme inhibitors. There is a suggestion that early commencement of treatment may slow progression substantially. These should now be recommended for all with proteinuria, and possibly even before then for those known to harbour mutations certain to cause end-stage renal failure. A very small minority develop the difficult post-transplant complication of Alport anti-glomerular basement membrane disease. This can rarely be treated successfully and leaves some patients on long-term dialysis. However, overall, patients with Alport syndrome have better than average survival and other outcomes than other patients with end-stage renal failure. Most are successfully transplanted. The question of risk to heterozygous carriers from donating kidneys to their affected relatives arises frequently. The risks may be felt acceptable in some circumstances. Additional therapies are under investigation.
Fabry disease
Authors: Waldek Stephen
Published: November 2015

Fabry disease is a rare X-linked lysosomal storage disorder in which deficiency of alpha-galactosidase A leads to accumulation of substrate, mostly globotriaosylceramide, which causes a progressive, multiorgan disease affecting predominantly the kidneys, skin, heart, gut, and nervous system. Painful peripheral (‘acral’) neuropathy is characteristic. The disease is commonly diagnosed in children and young men often after some years of usually neuropathic symptoms, with exacerbations (Fabry crises), that commonly elude diagnosis for a long time. These usually occur years in advance of overt involvement of other organs. Diagnosis may also be suspected from renal biopsy, echocardiographic evidence of cardiomyopathy commonly beginning as left ventricular hypertrophy, or characteristic angiokeratomas typically in ‘bathing trunk’ distribution on skin. Renal manifestations are of proteinuria leading to progressive chronic kidney disease associated with deposits in podocytes. Diarrhoea is common. Disordered sweating is typical. Corneal lesions are also typical and there may be tortuosity of retinal vessels. Strokes are increased in frequency, and sensorineural deafness may occur. Women have fewer and later overt manifestations but some develop severe disease.
Renal agenesis
Authors: Schreuder Michiel F.
Published: November 2015

In renal agenesis, the kidney never forms due to an absence of interaction in the developing embryo between the ureteric bud and the metanephric mesenchyme. Most cases of renal agenesis are unilateral, for which the reported incidence is around 1 in 3000. The prenatal diagnosis of unilateral renal agenesis is based on the absence of a recognizable kidney, either at the normal or an ectopic site. However, such cases of an empty renal fossa can also be explained by an involuted multicystic dysplastic kidney, or by renal aplasia. Based on the hyperfiltration hypothesis and clinical studies, glomerular hyperfiltration can be expected, resulting in hypertension, albuminuria, and renal injury, for which long-term follow-up of all patients with renal agenesis is desirable.
Renal dysplasia
Authors: Schreuder Michiel F.
Published: November 2015

Renal dysplasia refers to abnormal and incomplete development of the kidney, which may be segmental, for instance, in the upper part of a duplex kidney, or affect the entire kidney. Dysplasia is by definition a histological diagnosis, but in most patients diagnosis is made on the basis of evaluation with ultrasound and renography. This typically shows cysts and/or a small kidney with decreased corticomedullary differentiation and a reduced split renal function. The latter can also be found in other conditions, such as hypoplasia, vascular insults, renal post-infectious damage, or polycystic kidney disease, making it difficult to establish the diagnosis and thereby estimate the incidence of renal dysplasia. The clinical consequences of renal dysplasia depend upon the residual renal function and may range from hypertension to chronic kidney disease.
Renal tubular dysgenesis
Authors: Schreuder Michiel F.
Published: November 2015

Renal tubular dysgenesis involves the absence or incomplete differentiation of proximal tubular nephron segments. Due to the lack of a patent nephron, it is characterized by (fetal) anuria and subsequent oligohydramnios, pulmonary hypoplasia, premature birth with severe and refractory arterial hypotension, and fetal or neonatal death. The main cause for renal tubular dysgenesis is a genetic mutation in the renin–angiotensin system, which has shown an autosomal recessive trait. Maternal use of angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers during pregnancy can have similar blocking effects on the fetal renin–angiotensin system, which may lead to renal tubular dysgenesis. Even though there is no actual renal function, ultrasound usually shows kidneys of normal size and architecture with an intact corticomedullary differentiation. Most patients with renal tubular dysgenesis do not survive beyond the neonatal period. A few patients have been described to survive with respiratory support, vasopressor treatment, and dialysis.
Retroperitoneal fibrosis
Authors: Yaqoob Muhammad M. , Bennett-Richards Katherine , Junaid Islam
Published: November 2015

Retroperitoneal fibrosis (RPF) is a rare but multifaceted disease which encompasses a range of conditions characterized by the presence of a fibro-inflammatory tissue, which usually surrounds the abdominal aorta, iliac arteries, and extends into the retroperitoneum to entrap ureters with resultant unilateral or bilateral obstruction, usually at the junction between the middle and lower thirds of the ureter. The condition is progressive: initially, the fibrous tissue is fairly cellular, later becoming relatively acellular. The mechanism by which obstruction occurs is probably due to loss of peristalsis. A histological diagnosis should be obtained if at all possible, and laparotomy is required in order to obtain a sufficiently large sample to differentiate between idiopathic and secondary causes of RPF. Treatment of idiopathic RPF is by corticosteroids in the first instance with ureteric stents or ureterolysis initially and requires regular monitoring.
Ante- and postnatal imaging to diagnose human kidney malformations
Authors: Avni Fred E. , Cassart Marie , Massez Anne , Hall Michèle
Published: November 2015

Antenatal imaging plays a central role in the detection and management of congenital uropathies. Nowadays, two or three ultrasound examinations are performed in selected countries like Belgium or France while only one mid-trimester examination is performed in others (United Kingdom, Scandinavia). These examinations potentially allow the detection of a wide range of uronephropathies including at one end benign diseases and at the other, life-threatening conditions. Once detected, a full evaluation must be performed in order to confirm the diagnosis and evaluate the prognosis. In selected cases, fetal magnetic resonance imaging will provide additional useful information. After birth, imaging evaluation has been standardized and helps to prevent further deterioration of the renal function.
Drug-induced nephropathies
Authors: Shirali Anushree , Perazella Mark A.
Published: November 2015

Pharmacological therapy with prescription medications and other drugs is the mainstay of modern medical practice. In addition, an increasing number of people use over-the-counter and complementary treatments for management of acute and chronic disease. The kidney faces constant exposure to some of these medications and drugs since it receives significant blood flow from the systemic circulation and importantly participates in excretion of many of these substances. Some of these agents have the innate potential for nephrotoxicity, which is modified by patient- as well as drug-related factors. As a result, drug-induced nephropathies can complicate patient management and require diligent investigation and management by the consulting nephrologist. This chapter highlights those agents commonly associated with drug-induced nephropathies affecting various segments of the nephron. It reviews the drug- and patient-specific factors that increase susceptibility to kidney injury. In addition, it discusses the commonly encountered patterns of kidney injury with a particular drug, recognizing that some agents can cause injury via multiple pathways, and provides an overview of the mechanisms by which kidney disease occurs. Finally, the features that dictate renal recovery, including prophylactic and treatment measures, are reviewed.
Health economics
Authors: Merkesdal Sonja , Mau Wilfried
Published: November 2015

The economic burden of rheumatic diseases for society, various payers, and last but not least the individual patient has been increasingly recognized. In addition to the well-known impact of back pain and osteoarthritis, the upcoming new and expensive therapies have made this issue especially intriguing in the treatment of rheumatoid arthritis (RA). A mean international estimate of the total annual costs of RA, mainly consisting of direct resource consumption and indirect costs due to productivity losses relating to paid work, comes to about €5600. Other inflammatory rheumatologic diseases (ankylosing spondylitis, psoriatic arthritis, lupus erythematosus) generate similar costs.
The implementation of expensive biological drugs in rheumatic care has also led to the pressing need to determine the relation of their costs and clinical outcome (e.g. quality-adjusted life-years, QALYs) in order to compare different treatment strategies in defined patient groups. In RA the health-economic evidence for the cost-effectiveness of biologicals is already quite substantial in terms of treatment of early and advanced RA, as last option treatment of patients refractory to TNF inhibitors. Their cost-effectiveness as first line treatment is less clear. All biologicals have proved their cost-effectiveness in various settings depending on patient selection. It has been clearly demonstrated that adherence to the current guidelines, including monitoring of their effectiveness. leads to cost-effective scenarios. In TNF-refractory RA, abatacept and rituximab have proved to be economically favourable strategies.
Economic data on other inflammatory rheumatic entities is relatively sparse. Incomplete long-term and observational data are still the most prominent gaps in health-economic evidence relating to rheumatic disorders.
Synovial pathology
Authors: Pitzalis Costantino , Humby Frances , Seed Michael P.
Published: November 2015

Synovial pathology is seen in a variety of disease states, including rheumatoid arthritis (RA), osteoarthritis (OA), psoriatic arthritis, and systemic lupus erythmatosus (SLE). This chapter highlights recent advances that characterize the cellular composition of these tissues according to surface markers and chemokine and cytokine expression, and describes synovial functional status and response to therapeutics. In RA, after initiation, pannus migrates over and under cartilage, and into subchondral bone, in a destructive process. Cartilage-pannus junction (CPJ) is characterized as invasive or ’quiescent’ or ’indistinct’. Invasive CPJ can comprise macrophages, fibroblast-like synoviocytes (FLS), mast cells, and/or neutrophils. CPJ activity is related to the state of activation of the overlying subintima. Subintimal inflammation can be graded to a variety of degrees (I–IV) according to established criteria and is illustrated. In some RA synovia, cellular aggregates organize into ectopic lymphoid structures (ELS) through the expression of lymphorganogenic signals, to exhibit T- or B-cell zones accompanied by dendritic cells and lymphangiogenesis. ELS synthesize rheumatoid factor (RF) and anti-citrullinated peptide antibodies (ACAP), considered to be indicative of aggressive disease. The selective cellular expression of macrophage and dendritic cell chemokines and cytokines such as TNF, GMCSF, TGFβ‎‎, IL-1, IL-6, IL-23, and chemokines can be seen in synovia, to form a regulated and cooperative environment that sustains the cellular organization and pathological function. Important to this process are FLS and CD68+ macrophages. CD68 expression correlates with disease severity and can be useful as a surrogate marker of disease modifying activity of therapeutics, such as anti-TNF and anti-B-cell biologics.
Biochemical investigation of rheumatic diseases
Authors: Lopez Berenice , Twomey Patrick J.
Published: November 2015

It is important for rheumatologists to have an understanding of biochemical tests including an awareness of their limitations. The biological variability of an analyte both within and between individuals, the limitations of the measurement technology, the sensitivity of laboratory internal quality control and external quality assurance procedures, as well as interlaboratory variations in practices including sample collection procedures, may all impact on the interpretation of a result. Biochemical tests are often requested to monitor organ-specific dysfunction arising as an adverse consequence of pharmacotherapy or as a component of a systemic rheumatic disease, although dysfunction may also reflect infection or coincidental pathology. Patients with rheumatic diseases are at high risk of renal and hepatic disease. Serum creatinine and its derivative estimated glomerular filtration rate (eGFR) are the most readily available surrogate markers of GFR and are used to assess renal impairment and monitor its course. However, the use of creatinine alone lacks sensitivity and a substantial loss of function must occur before creatinine levels are increased. Additional biochemical screening for kidney damage can be performed by assessment of glomerular integrity, including proteinuria or albuminuria and haematuria. A wide spectrum of rheumatic diseases can affect the liver with various degrees of involvement and hepatic pathology. These often present with cholestatic or hepatitic biochemical profiles. The medical management of rheumatic diseases also involves medications that are hepatotoxic, and routine monitoring of liver function is recommended. This approach is not problem-free and may be improved by quantitative determinations of non-invasive markers of liver fibrosis in the future. Together with imaging techniques, biochemical tests play an important role in the assessment and differential diagnosis of metabolic bone disease.
Radiographic imaging
Authors: Rowbotham Emma L. , Grainger Andrew J.
Published: November 2015

Plain film radiography is often the first imaging modality employed in the assessment of patients with a rheumatological condition. More recently this has been superseded by cross-sectional imaging, in particular ultrasound and MRI, which have improved sensitivity in detection of early disease when compared with plain film imaging. However, there remains a role for conventional radiography in both the initial diagnosis and monitoring of disease progression.
A standard approach to assessing radiographs in the context of arthropathy is usually employed by radiologists; by following this structured review a diagnosis or narrow differential may be reached on plain film imaging alone. Plain film radiograph findings of the most common rheumatological disorders are covered in detail in this chapter including osteoarthritis, the inflammatory arthritides, and crystal arthropathy. Findings in the connective tissue disorders are then covered, followed by less commonly encountered conditions such as SAPHO, neuropathic arthropathy, and haemochromotosis.
Analgesics
Authors: Foo Joanne , Saleem Benazir , Conaghan Philip G.
Published: November 2015

Pain is one of the commonest presenting symptoms of musculoskeletal disorders and may be one the hardest to treat successfully. The available analgesic options provide different modes of action and their ranks continue to expand with new agents, some with multiple target action. This chapter reviews currently available analgesics (paracetamol and opioids) used for managing musculoskeletal pain and the agents used for neuropathic pain, including their mechanism of action, pharmacokinetics and side effects. The role of neuroleptic agents is reviewed, and a brief outline of some newer therapies for the treatment of pain such as tapentadol, and a potential therapy, anti-nerve growth factor monoclonal antibodies.
Bone and joint infections in children
Authors: Sukhtankar Priya , Clark Julia , Faust Saul N.
Published: November 2015

Bone and joint infections in children are uncommon, but they affect all ages and there is a wide range of disease. The mode of infection may be haematogenous or by direct inoculation from a wound. The child may present acutely unwell or with a chronic infection. Clinical features include fever, reluctance to move the affected limb, pain, and swelling. Laboratory tests and medical imaging are used to confirm diagnosis.
Medical treatment is with initial intravenous antibiotic therapy, usually followed by oral treatment. Surgical treatment may be necessary if abscess or joint collection is present. In general prognosis is good with timely initiation of treatment, although complications such as pathological fracture are occasionally seen.
Vasculitis—classification and diagnosis
Authors: Watts Richard A. , Scott David G. I.
Published: November 2015

The vasculitides are a group of conditions characterized by inflammation and necrosis of blood vessels; they are generally of unknown aetiology. The classification of vasculitides is based on the size of vessel involved and whether there is a known cause (secondary) or not (primary). This approach has stood the test of time. The American College of Rheumatology (ACR) in 1990 produced classification criteria for the major types of vasculitis and in 1994 definitions were promulgated by the Chapel Hill Consensus Conference. These did not include anti-neutrophil cytoplasm antibodies (ANCA) and the ACR scheme did not include microscopic polyangiitis. The definitions have recently been updated to include modern concepts of pathogenesis including ANCA. No validated diagnostic criteria are available for routine clinical practice.
The diagnosis of vasculitis requires a high index of suspicion, especially in the systemically unwell patient with multiorgan involvement. The key to diagnosis is a detailed and systematic approach to patient assessment involving all potentially involved organs. In a patient with suspected vasculitis immediate urinalysis is mandatory as the severity of renal involvement at presentation is a major determinant of outcome. Each potentially involved organ should be comprehensively evaluated. Tissue biopsy should be obtained whenever possible, as treatment is potentially toxic using glucocorticoids combined with cytotoxic agents. Biopsy should not, however, delay initiation of treatment. Potential alternative diagnosis should be considered, especially infection and malignancy, and excluded whenever possible.
Paediatric vasculitis
Authors: Eleftheriou Despina , Brogan Paul A.
Published: November 2015

Systemic vasculitis is characterized by blood vessel inflammation which may lead to tissue injury from vascular stenosis, occlusion, aneurysm, and/or rupture. Apart from relatively common vasculitides such as IgA vasculitis (Henoch–Schönlein purpura (HSP)) and Kawasaki’s disease (KD), most of the primary vasculitic syndromes are rare in childhood, but are associated with significant morbidity and mortality. New classification criteria for childhood vasculitis have recently been proposed and validated. The cause of most vasculitides is unknown, although it is likely that a complex interaction between environmental factors such as infections and inherited host responses trigger the disease and determine the vasculitis phenotype. Several genetic polymorphisms in vasculitis have now been described that may be relevant in terms of disease predisposition or development of disease complications. Treatment regimens continue to improve, with the use of different immunosuppressive medications and newer therapeutic approaches such as biologic agents. We provide an overview of paediatric vasculitides focusing on HSP, KD, and polyarteritis nodosa (PAN). Key differences (where relevant) between paediatric and adult vasculitis are highlighted. In addition we discuss new emerging challenges particularly in respect to the long-term cardiovascular morbidity for children with systemic vasculitis, and emphasize the importance of future international multicentre collaborative studies to further increase and standardize the scientific base of investigating and treating childhood vasculitis.
Thoracic endoprostheses for type B aortic dissections
Authors: Lioupis Christos , Clough Rachel , Taylor Peter
Published: November 2015

Endovascular treatment of acute complicated type B aortic dissection has become accepted as the first line of treatment. Advantages over open surgical repair include the minimal invasiveness of the procedure, the lack of a thoracotomy, no aortic cross-clamping causing end-organ ischaemia, and no requirement for full anticoagulation. Mortality and morbidity in terms of stroke and paraplegia are less than with open repair, although the long-term durability of endovascular repair is yet to be proven. We present a case of acute complicated type B aortic dissection treated with endovascular repair which resulted in the death of the patient. The reasons for this poor outcome are discussed, and the pitfalls and the ways to avoid them are identified.
Management of endoleaks after thoracic endografts
Authors: Alsafi Ali , Hamady Mo
Published: November 2015

Thoracic aortic aneurysms are a relatively uncommon clinical problem; however, their rupture is associated with high mortality and morbidity, with treatment aiming at reducing this risk. Endovascular repair of thoracic aortic aneurysms (TEVAR) has emerged over the last decade as a viable and increasingly preferred alternative to open surgical repair for thoracic aortic disease, which is increasing in prevalence, especially in an aging population. TEVAR has created a new set of challenges and complications not previously encountered with conventional forms of treatment; one such important complication is endoleaks. This Case discusses the pathogenesis, diagnosis, and management of endoleaks as well as some strategies to reduce their incidence. The discussion of this unique clinical entity includes a case-based discussion of some of the clinical and technical challenges encountered.
Juxtarenal abdominal aortic aneurysms: fenestrations versus chimneys
Authors: Shaida Nadeem , Winterbottom Andrew
Published: November 2015

Limitations of conventional infrarenal endovascular abdominal aortic aneurysm repair (EVAR) include a short infrarenal neck or aneurysmal disease involving the abdominal aortic visceral segment. A number of newer endovascular treatments, including fenestrated EVAR, branched endovascular stent grafts, and chimney stents, have been proposed. We describe one such case treated with a four-vessel fenestrated EVAR and also review the current options available for the treatment of juxtarenal and suprarenal abdominal aortic aneurysm disease.
Management of endoleaks after abdominal aneurysm endovascular repair
Authors: Chung Raymond , Morgan Robert
Published: November 2015

Endoleaks are a common complication of endovascular repair of abdominal aortic aneurysms. Classification is paramount in determining their natural history/propensity for rupture and therefore their clinical management. CT angiography and digital subtraction angiography remains the most common modalities in the detection and characterization of endoleaks (ELs). The majority are amenable to endovascular repair with only a small cohort requiring surgical open conversion. Types I and III ELs, associated with systemic pressurization of the aneurysm sac and therefore increased risk of rupture, are treated early with additional endografts, extender modules, cuffs, balloon remodelling, or the deployment of Palmaz stents. Treatment of type II ELs is reserved for patients who demonstrate sac enlargement. Type II ELs can be embolized by either a percutaneous or a transarterial approach. Type IV ELs resolve spontaneously and do not require further treatment. Type V ELs, also referred to as endotension, can be treated by relining the stent graft with a new endograft. We report the case of a type II EL treated by transarterial embolization with Onyx.
Carotid artery stenting: how to treat restenosis
Authors: Cannavale Alessandro , Fanelli Fabrizio
Published: November 2015

We report a case of a 63-old female who, after previous carotid endarterectomy (CEA) for a transient ischaemic attack (TIA), experienced restenosis which was treated with a balloon-expandable stent. Seven months later an ultrasound colour Doppler (USCD) revealed deformation of the proximal end of the stent, which led to severe in-stent restenosis (ISR). The patient underwent a successful re-intervention by stenting and dilation of the ISR. The management of ISR may be performed by medical therapy, or by an endovascular or surgical approach. Different endovascular techniques are available to treat carotid in-stent restenosis: angioplasty alone, cutting balloon angioplasty (CB), stenting and angioplasty, brachytherapy, drug-eluting stents, or drug-eluting balloons. Each technique can be applied according to the patient’s clinical status and history, supra-aortic vessel anatomy, features of the stent previously implanted, and type of ISR.
SFA endoluminal bypass: the new era of critical limb ischaemia treatment
Authors: Shaw Aidan , Ahmed Irfan
Published: November 2015

The prognosis for patients presenting with critical limb ischaemia (CLI) is poor not only for the limb but also for life. The optimal treatment for patients presenting with CLI is revascularization. Severity and extent of disease, morphology of the lesions, and run-off determine the treatment modalities. Surgical bypass is currently recommended for complex lesions. However, the techniques and technology in endovascular radiology continue to evolve and improve. We describe a case of revascularization of an occluded superficial femoral artery and discuss tips and tricks, such as re-entry devices, as well as the evidence behind the use of angioplasty and stenting, particularly drug-eluting technology.
Below the knee angioplasty: bare versus drug-eluting stents
Authors: Spiliopoulos Stavros , Siablis Dimitrios
Published: November 2015

A 74-year-old patient suffering from critical limb ischaemia underwent percutaneous endovascular revascularization in the interventional radiology department. Baseline symptomatology included severe rest pain not responding to common analgesics, and dry gangrene of the left toe (Rutherford–Becker stage 5). The ankle-brachial index at presentation was 0.60. Selective digital subtraction angiography revealed significant 50–60% tortuous multiple stenoses and one near occlusion, all detected at the proximal segment of the peroneal artery which was the only patent infrapopliteal vessel. The arterial supply of the distal foot was preserved by the distal peroneal collateral network. Direct stenting of the diseased proximal peroneal segment using three sirolimus-eluting stents in an overlapping manner was performed. The total stented length was 94mm. Dual antiplatelet therapy was prescribed for 6 months, followed by monotherapy with clopidogrel 75mg 1 × 1. Complete wound healing and clinical improvement by three stages (Rutherford-Becker stage 2 from stage 5) was detected at one-year follow-up and was sustained throughout a three-year follow-up period. The lesion was patent without any in-stent restenosis at two years angiographic follow-up.
Dialysis access at risk: balloons or stent grafts?
Authors: Spiliopoulos Stavros , Karnabatidis Dimitrios
Published: November 2015

A 34-year-old male was referred to the interventional radiology department with thrombosis of a straight brachiocephalic synthetic haemodialysis graft in upper arm. The haemodialysis access had been created two months previously and the referring physician reported increased venous pressure during haemodialysis, indicating a possible stenosis, during the last two sessionsbefore the thrombotic event. The patient underwent percutaneous haemodialysis access declotting using a combined technique of transcatheter thrombolysis and mechanical thrombectomy. The underlying lesion, detected at the venous anastomosis site, was treated using a 7 × 100mm high-pressure balloon catheter. Thrill was palpable at the end of the procedure. However, no thrill was present the next day and the patient returned for a check fistulogram that demonstrated a significant restenosis of the previously treated anastomotic segment. Subsequent balloon angioplasty was suboptimal and an 8 × 80mm self-expandable covered stent was deployed across the lesion. Antiplatelet therapy with clopidogrel 75mg 1 × 1 was prescribed and the patient entered a monthly monitoring and bimonthly surveillance protocol. At eight months follow-up no clinical signs of failing access were evident and the patient reported excellent dialysis sessions. The six-month fistulogram demonstrated <30% restenosis at the proximal end of the stent.
Epistaxis: which embolic materials to use?
Authors: Jarząbek Magdalena , Trojanowski Piotr , Szczerbo-Trojanowska Małgorzata
Published: November 2015

Although embolization is not considered a first-line treatment for epistaxis, it is widely recognized as being an important therapeutic tool in certain difficult scenarios. We present a case of intractable posterior epistaxis after maxillofacial trauma with isolated nasal septum fracture where conservative therapy was unsuccessful and arterial embolization was performed as a second-line treatment. We will discuss various aspects relating to the diagnosis of epistaxis, types of epistaxis, and alternative treatment methods, with a detailed description of embolization techniques and embolic materials used in different diseases.
Massive haemoptysis: what to embolize?
Authors: Tang Kendrick , Kwok Philip
Published: November 2015

Haemoptysis may hinder oxygen exchange and can be life-threatening if not controlled in time. Contrast CT thorax is preferred over bronchoscopy as the primary investigation for haemoptysis, providing information not only on the site and cause of haemorrhage but also pinpointing the origin of culprit feeder vessel. Percutaneous transcatheter embolization is a safe and effective treatment for massive haemoptysis. The interventional radiologist should be familiar with the bronchial artery anatomy, the catheters, and different types of embolizing agents. Non-bronchial systemic arterial supplies can be the reason for treatment failure. Occasionally, complications may happen and these should be discussed with the patient before the procedure.
Gastrointestinal bleeding: which embolic material to use?
Authors: Ganai Bhaskar , Lee Michael J
Published: November 2015

Gastrointestinal (GI) bleeding is common with an important role for interventional radiology. We describe an unusual case of GI bleeding with examples of different embolic agents that can be employed. For upper GI bleeding, haemostasis can most often be achieved by endoscopy. If this is unsuccessful or there is a recurrence of bleeding, embolization with coils, particles, or liquid embolic agents can be performed. Because of the rich collateral supply, embolization can be focused on larger vessels, such as the gastroduodenal artery. In lower GI bleeding, endoscopy is generally less useful. CT angiography can be used to identify a source of bleeding, followed by embolization with coils. Because of the lack of collateral supply to the lower GI tract, embolization should be very selective.