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J. Clin. Med., Volume 6, Issue 4 (April 2017)

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Cover Story Involvement of the endothelin, nitric oxide (NO) and prostacyclin pathways in the pathogenesis of [...] Read more.
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Research

Jump to: Review, Other

Open AccessArticle Hip Arthropathy in Haemophilia
J. Clin. Med. 2017, 6(4), 44; doi:10.3390/jcm6040044
Received: 26 January 2017 / Revised: 28 March 2017 / Accepted: 3 April 2017 / Published: 8 April 2017
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Abstract
Hip arthropathy in haemophilic patients is disabling for hip and other common target joints. Even if bleedings in the hip are not frequent, femoroacetabular alterations may affect the functional ability of patients at a very young age. A haematologic prophylaxis combined with an
[...] Read more.
Hip arthropathy in haemophilic patients is disabling for hip and other common target joints. Even if bleedings in the hip are not frequent, femoroacetabular alterations may affect the functional ability of patients at a very young age. A haematologic prophylaxis combined with an adequate lifestyle and regular and low-traumatic physical activity are the keys to preventing such arthropathy. In the early stages of arthropathy, anti-inflammatory drugs and physical therapy may be sufficient to limit its progression. In cases of recurrent symptoms, viscosupplementation with hyaluronic acid, and chemical synoviorthesis are useful options. In more advanced stages, hip arthroscopy may be treated by synovectomy or loose body removal. For late stages, total hip arthroplasty (THA) is mandatory. Until a few decades ago, the clinical outcomes after hip arthroplasty were variable, due to the different management of patients and the use of old generation implants and couplings. In the last decade, the introduction of the multidisciplinary management and the use of modern cementless implants with high performing materials and less invasive surgical techniques have dramatically improved the functional results. Nowadays, as is the case for other target joints, the purpose of the management in haemophilia centers is the early detection of any hip alterations—by clinical and ultrasound (US) evaluations of patients in childhood—to reveal any early articular damage and to provide adequate treatment in case of symptoms. The present paper represents an updated review of the several approaches to hip arthropathy in haemophilia. Full article
(This article belongs to the Special Issue Outstanding Advances in Hemophilia Therapies)
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Open AccessArticle Predictors of Active Extravasation and Complications after Conventional Angiography for Acute Intraabdominal Bleeding
J. Clin. Med. 2017, 6(4), 47; doi:10.3390/jcm6040047
Received: 15 March 2017 / Revised: 13 April 2017 / Accepted: 14 April 2017 / Published: 18 April 2017
Cited by 1 | PDF Full-text (215 KB) | HTML Full-text | XML Full-text
Abstract
Conventional angiography is used to evaluate and treat possible sources of intraabdominal bleeding, though it may cause complications such as contrast-induced nephropathy (CIN). The study’s purpose was to identify factors predicting active extravasation and complications during angiography for acute intraabdominal bleeding. All conventional
[...] Read more.
Conventional angiography is used to evaluate and treat possible sources of intraabdominal bleeding, though it may cause complications such as contrast-induced nephropathy (CIN). The study’s purpose was to identify factors predicting active extravasation and complications during angiography for acute intraabdominal bleeding. All conventional angiograms for acute bleeding (January 2013–June 2015) were reviewed retrospectively, including 75 angiograms for intraabdominal bleeding in 70 patients. Demographics, comorbidities, vital signs, complications within one month, and change in hematocrit (ΔHct) and fluids and blood products administered over the 24 h prior to angiography were recorded. Of 75 exams, 20 (27%) demonstrated extravasation. ΔHct was the only independent predictor of extravasation (p = 0.017), with larger ΔHct (−17%) in patients with versus those without extravasation (–1%) (p = 0.01). CIN was the most common complication, occurring in 10 of 66 angiograms (15%). Glomerular filtration rate (GFR) was the only independent predictor (p = 0.03); 67% of patients with GFR < 30, 29% of patients with GFR 30–60, and 8% of patients with GFR > 60 developed CIN. For patients with intraabdominal bleeding, greater ΔHct decrease over 24 h before angiography predicts active extravasation. Pre-existing renal impairment predicts CIN. Patients with large hematocrit declines should be triaged for rapid angiography, though benefits can be weighed with the risk of renal impairment. Full article

Review

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Open AccessReview Telemedicine Applications in Pediatric Retinal Disease
J. Clin. Med. 2017, 6(4), 36; doi:10.3390/jcm6040036
Received: 17 November 2016 / Revised: 13 March 2017 / Accepted: 20 March 2017 / Published: 23 March 2017
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Abstract
Teleophthalmology is a developing field that presents diverse opportunities. One of its most successful applications to date has been in pediatric retinal disease, particularly in screening for retinopathy of prematurity (ROP). Many studies have shown that using telemedicine for ROP screening allows a
[...] Read more.
Teleophthalmology is a developing field that presents diverse opportunities. One of its most successful applications to date has been in pediatric retinal disease, particularly in screening for retinopathy of prematurity (ROP). Many studies have shown that using telemedicine for ROP screening allows a remote ophthalmologist to identify abnormal findings and implement early interventions. Here, we review the literature on uses of telemedicine in pediatric retinal disease and consider future applications. Full article
(This article belongs to the Special Issue Telemedicine - Technical Developments and Clinical Practice)
Open AccessFeature PaperReview The Value of Coenzyme Q10 Determination in Mitochondrial Patients
J. Clin. Med. 2017, 6(4), 37; doi:10.3390/jcm6040037
Received: 28 February 2017 / Revised: 17 March 2017 / Accepted: 17 March 2017 / Published: 24 March 2017
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Abstract
Coenzyme Q10 (CoQ) is a lipid that is ubiquitously synthesized in tissues and has a key role in mitochondrial oxidative phosphorylation. Its biochemical determination provides insight into the CoQ status of tissues and may detect CoQ deficiency that can result from either
[...] Read more.
Coenzyme Q10 (CoQ) is a lipid that is ubiquitously synthesized in tissues and has a key role in mitochondrial oxidative phosphorylation. Its biochemical determination provides insight into the CoQ status of tissues and may detect CoQ deficiency that can result from either an inherited primary deficiency of CoQ metabolism or may be secondary to different genetic and environmental conditions. Rapid identification of CoQ deficiency can also allow potentially beneficial treatment to be initiated as early as possible. CoQ may be measured in different specimens, including plasma, blood mononuclear cells, platelets, urine, muscle, and cultured skin fibroblasts. Blood and urinary CoQ also have good utility for CoQ treatment monitoring. Full article
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Open AccessFeature PaperReview Factor VII Deficiency: Clinical Phenotype, Genotype and Therapy
J. Clin. Med. 2017, 6(4), 38; doi:10.3390/jcm6040038
Received: 30 January 2017 / Revised: 12 March 2017 / Accepted: 23 March 2017 / Published: 28 March 2017
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Abstract
Factor VII deficiency is the most common among rare inherited autosomal recessive bleeding disorders, and is a chameleon disease due to the lack of a direct correlation between plasma levels of coagulation Factor VII and bleeding manifestations. Clinical phenotypes range from asymptomatic condition—even
[...] Read more.
Factor VII deficiency is the most common among rare inherited autosomal recessive bleeding disorders, and is a chameleon disease due to the lack of a direct correlation between plasma levels of coagulation Factor VII and bleeding manifestations. Clinical phenotypes range from asymptomatic condition—even in homozygous subjects—to severe life-threatening bleedings (central nervous system, gastrointestinal bleeding). Prediction of bleeding risk is thus based on multiple parameters that challenge disease management. Spontaneous or surgical bleedings require accurate treatment schedules, and patients at high risk of severe hemorrhages may need prophylaxis from childhood onwards. The aim of the current review is to depict an updated summary of clinical phenotype, laboratory diagnosis, and treatment of inherited Factor VII deficiency. Full article
(This article belongs to the Special Issue Outstanding Advances in Hemophilia Therapies)
Open AccessFeature PaperReview Outcome of Clinical Trials with New Extended Half-Life FVIII/IX Concentrates
J. Clin. Med. 2017, 6(4), 39; doi:10.3390/jcm6040039
Received: 29 January 2017 / Revised: 9 March 2017 / Accepted: 11 March 2017 / Published: 28 March 2017
Cited by 1 | PDF Full-text (239 KB) | HTML Full-text | XML Full-text
Abstract
The development of a new generation of coagulation factors with improved pharmacokinetic profile will change the paradigm of treatment of persons with hemophilia (PWH). The standard treatment in PWH is represented by regular long-term prophylaxis that, given intravenously twice or thrice weekly, is
[...] Read more.
The development of a new generation of coagulation factors with improved pharmacokinetic profile will change the paradigm of treatment of persons with hemophilia (PWH). The standard treatment in PWH is represented by regular long-term prophylaxis that, given intravenously twice or thrice weekly, is associated with a not-negligible burden on patients’ quality of life. The availability of drugs with improved pharmacokinetic profile may improve prophylaxis feasibility and protection against bleeding episodes. This article summarizes the main results obtained from clinical trials with modified factor VIII (FVIII) and factor IX (FIX) molecules. Published literature on new molecules for replacement treatment in hemophilia A and B was retrieved using PubMed search, and all ongoing clinical trials have been researched via www.clinicaltrials.gov. Such new molecules are usually engineered to have a longer plasma half-life than that which has been obtained by chemical modification (i.e., conjugation with polyethylene glycol, PEG) or by creating recombinant fusion proteins. Results from phase I/III studies in previously treated adults and children are now available for the vast majority of new products, including the results of their use in a surgical setting. On the contrary, trials involving previously untreated patients are still ongoing for all and results not yet available. Full article
(This article belongs to the Special Issue Outstanding Advances in Hemophilia Therapies)
Open AccessFeature PaperReview The Changing Landscape of Pulmonary Arterial Hypertension in the Adult with Congenital Heart Disease
J. Clin. Med. 2017, 6(4), 40; doi:10.3390/jcm6040040
Received: 31 January 2017 / Revised: 9 March 2017 / Accepted: 23 March 2017 / Published: 30 March 2017
Cited by 1 | PDF Full-text (1320 KB) | HTML Full-text | XML Full-text
Abstract
Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a common type of pulmonary arterial hypertension (PAH) and a frequent complication of congenital heart disease (CHD). PAH-CHD represents a heterogeneous patient population and it is important to distinguish between the underlying cardiac
[...] Read more.
Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a common type of pulmonary arterial hypertension (PAH) and a frequent complication of congenital heart disease (CHD). PAH-CHD represents a heterogeneous patient population and it is important to distinguish between the underlying cardiac defects considering the prognostic and therapeutic implications. Improved interventional techniques have enabled repair or palliation of most cardiac defects, though a substantial number of patients remain at high risk for PAH after closure. Traditionally, the treatment and management of PAH-CHD patients has been limited to palliative and supportive care, and based on expert opinion rather than clinical trials. Recently, however, the availability of advanced PAH-specific treatment has opened up a new field for the clinical management of this condition. Nevertheless, there is limited evidence on the optimal therapeutic approach for PAH-CHD. Herein, we discuss the current and novel therapeutic options for PAH-CHD as well as highlight several challenges in the clinical management at present. Full article
(This article belongs to the Special Issue Novel Therapeutic Approaches for Pulmonary Arterial Hypertension)
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Open AccessReview Natural Killer T Cells in Liver Ischemia–Reperfusion Injury
J. Clin. Med. 2017, 6(4), 41; doi:10.3390/jcm6040041
Received: 9 February 2017 / Revised: 10 March 2017 / Accepted: 20 March 2017 / Published: 1 April 2017
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Abstract
Restoration of blood flow to an ischemic organ results in significant tissue injury. In the field of liver transplantation, ischemia–reperfusion injury (IRI) has proven to be a formidable clinical obstacle. In addition to metabolic stress and inflammation, IRI results in profound graft dysfunction
[...] Read more.
Restoration of blood flow to an ischemic organ results in significant tissue injury. In the field of liver transplantation, ischemia–reperfusion injury (IRI) has proven to be a formidable clinical obstacle. In addition to metabolic stress and inflammation, IRI results in profound graft dysfunction and loss. The severity of IRI further limits the ability to expand the donor pool by using partial grafts and marginal organs. As such, the inflammatory response to reperfusion of the liver continues to be an area of intense investigation. Among the various leukocytes involved in IRI, new insights suggest that natural killer T (NKT) cells may be a central driver of hepatocellular injury. Herein, we examine recent experimental observations that provide a mechanistic link between NKT cell recruitment to liver and post-perfusion tissue injury. Full article
Open AccessFeature PaperReview Emerging Metabolic Therapies in Pulmonary Arterial Hypertension
J. Clin. Med. 2017, 6(4), 43; doi:10.3390/jcm6040043
Received: 15 February 2017 / Revised: 28 March 2017 / Accepted: 29 March 2017 / Published: 4 April 2017
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Abstract
Pulmonary hypertension (PH) is an enigmatic vascular disorder characterized by pulmonary vascular remodeling and increased pulmonary vascular resistance, ultimately resulting in pressure overload, dysfunction, and failure of the right ventricle. Current medications for PH do not reverse or prevent disease progression, and current
[...] Read more.
Pulmonary hypertension (PH) is an enigmatic vascular disorder characterized by pulmonary vascular remodeling and increased pulmonary vascular resistance, ultimately resulting in pressure overload, dysfunction, and failure of the right ventricle. Current medications for PH do not reverse or prevent disease progression, and current diagnostic strategies are suboptimal for detecting early-stage disease. Thus, there is a substantial need to develop new diagnostics and therapies that target the molecular origins of PH. Emerging investigations have defined metabolic aberrations as fundamental and early components of disease manifestation in both pulmonary vasculature and the right ventricle. As such, the elucidation of metabolic dysregulation in pulmonary hypertension allows for greater therapeutic insight into preventing, halting, or even reversing disease progression. This review will aim to discuss (1) the reprogramming and dysregulation of metabolic pathways in pulmonary hypertension; (2) the emerging therapeutic interventions targeting these metabolic pathways; and (3) further innovation needed to overcome barriers in the treatment of this devastating disease. Full article
(This article belongs to the Special Issue Novel Therapeutic Approaches for Pulmonary Arterial Hypertension)
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Open AccessFeature PaperReview Diagnosis and Treatment of von Willebrand Disease and Rare Bleeding Disorders
J. Clin. Med. 2017, 6(4), 45; doi:10.3390/jcm6040045
Received: 23 February 2017 / Revised: 4 April 2017 / Accepted: 5 April 2017 / Published: 10 April 2017
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Abstract
Along with haemophilia A and B, von Willebrand disease (VWD) and rare bleeding disorders (RBDs) cover all inherited bleeding disorders of coagulation. Bleeding tendency, which can range from extremely severe to mild, is the common symptom. VWD, due to a deficiency and/or abnormality
[...] Read more.
Along with haemophilia A and B, von Willebrand disease (VWD) and rare bleeding disorders (RBDs) cover all inherited bleeding disorders of coagulation. Bleeding tendency, which can range from extremely severe to mild, is the common symptom. VWD, due to a deficiency and/or abnormality of von Willebrand factor (VWF), represents the most frequent bleeding disorder, mostly inherited as an autosomal dominant trait. The diagnosis may be difficult, based on a bleeding history and different diagnostic assays, which evaluate the pleiotropic functions of VWF. Different treatment options are available for optimal management of bleeding and their prevention, and long-term outcomes are generally good. RBDs are autosomal recessive disorders caused by a deficiency of any other clotting factor, apart from factor XII, and cover roughly 5% of all bleeding disorders. The prevalence of the severe forms can range from 1 case in 500,000 up to 1 in 2–3 million, according to the defect. Diagnosis is based on bleeding history, coagulation screening tests and specific factor assays. A crucial problem in RBDs diagnosis is represented by the non-linear relationship between clinical bleeding severity and residual clotting levels; genetic diagnosis may help in understanding the phenotype. Replacement therapies are differently available for patients with RBDs, allowing the successful treatment of the vast majority of bleeding symptoms. Full article
(This article belongs to the Special Issue Outstanding Advances in Hemophilia Therapies)
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Open AccessFeature PaperReview Treatment and Prevention of Bleeds in Haemophilia Patients with Inhibitors to Factor VIII/IX
J. Clin. Med. 2017, 6(4), 46; doi:10.3390/jcm6040046
Received: 28 January 2017 / Revised: 6 April 2017 / Accepted: 10 April 2017 / Published: 17 April 2017
Cited by 1 | PDF Full-text (833 KB) | HTML Full-text | XML Full-text
Abstract
The development of alloantibodies neutralising therapeutically administered factor (F) VIII/IX (inhibitors) is currently the most severe complication of the treatment of haemophilia. When persistent and at a high titre, inhibitors preclude the standard replacement treatment with FVIII/FIX concentrates, making patients’ management challenging. Indeed,
[...] Read more.
The development of alloantibodies neutralising therapeutically administered factor (F) VIII/IX (inhibitors) is currently the most severe complication of the treatment of haemophilia. When persistent and at a high titre, inhibitors preclude the standard replacement treatment with FVIII/FIX concentrates, making patients’ management challenging. Indeed, the efficacy of bypassing agents, i.e., activated prothrombin complex concentrates (aPCC) and recombinant activated factor VII (rFVIIa), needed to overcome the haemostatic interference of the inhibitor, is not comparable to that of factor concentrates. In addition, the therapeutical response is unpredictable, with a relevant inter-individual and even intra-individual variability, and no laboratory assay is validated to monitor the efficacy and safety of the treatment. As a result, inhibitor patients have a worse joint status and quality of life compared to inhibitor-free subjects and the eradication of the inhibitor by immune tolerance induction is the preeminent therapeutic goal, particularly in children. However, over the last decades, treatment with bypassing agents has been optimised, allowing home treatment and the individualisation of regimens aimed at improving clinical outcomes. In this respect, a growing body of evidence supports the efficacy of prophylaxis with both bypassing agents in reducing bleeding rates and improving the quality of life, although the impact on long-term outcomes (in particular on preventing/reducing joint deterioration) is still unknown. This review offers an update on the current knowledge and practice of the use of bypassing agents in haemophiliacs with inhibitors, as well as on debated issues and unmet needs in this challenging setting. Full article
(This article belongs to the Special Issue Outstanding Advances in Hemophilia Therapies)
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Other

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Open AccessCase Report Severe Adenoviral Pneumonia in an Immunocompetent Host with Persistent Fevers Treated with Multiple Empiric Antibiotics for Presumed Bacterial Co-Infection: An Antibiotic Stewardship Perspective on De-Escalation Derailed
J. Clin. Med. 2017, 6(4), 42; doi:10.3390/jcm6040042
Received: 13 January 2017 / Revised: 24 March 2017 / Accepted: 30 March 2017 / Published: 4 April 2017
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Abstract
We present a case of severe adenoviral pneumonia in a 20-year-old immunocompetent host with persistently high fevers. The patient was needlessly given multiple empiric antibiotics for non-existent bacterial co-infection. This case has important antibiotic stewardship lessons for practitioners in approaching fevers in the
[...] Read more.
We present a case of severe adenoviral pneumonia in a 20-year-old immunocompetent host with persistently high fevers. The patient was needlessly given multiple empiric antibiotics for non-existent bacterial co-infection. This case has important antibiotic stewardship lessons for practitioners in approaching fevers in the ICU. Full article
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