Hemophilia A in Females: Considerations for Clinical Management

2019 ◽  
Vol 143 (3) ◽  
pp. 289-294
Author(s):  
Paulette Bryant ◽  
Aikaterini Boukouvala ◽  
Jenny McDaniel ◽  
Danielle Nance
Keyword(s):  
Treatment Adherence ◽  
Clinical Management ◽  
Hemophilia A ◽  
Treatment Options ◽  
Physical Activities ◽  
Joint Disease ◽  
Effective Management ◽  
Training And Education ◽  
Current Guidelines ◽  
Female Carriers

Approximately 50% of female carriers of hemophilia A have factor VIII (FVIII) levels below 0.5 IU/dL and may be categorized as having mild hemophilia. Females with hemophilia may go undiagnosed for years because the most common symptoms – menorrhagia and bleeding after childbirth – also occur in females without hemophilia. Females with hemophilia can exhibit increased bleeding tendencies despite current guidelines of expected, adequate FVIII levels. The cases described illustrate the clinical variability and presentation of hemophilia in females and highlight the importance of a timely diagnosis, effective management, and monitoring. Prophylactic factor replacement therapy is recommended in females with hemophilia, particularly those with joint disease or gynecologic complications. Affected individuals should receive infusion training and education on treatment options, physical activities, the importance of treatment adherence, and recognizing bleeding symptoms warranting treatment. Further study is needed to increase awareness of hemophilia in females and reassess current guidelines for their management and monitoring.

scholarly journals Clinical Management of Hypertension, Inflammation and Thrombosis in Hospitalized COVID-19 Patients: Impact on Survival and Concerns

2021 ◽  
Vol 10 (5) ◽  
pp. 1073
Author(s):  
Patricia Martínez-Botía ◽  
Ángel Bernardo ◽  
Andrea Acebes-Huerta ◽  
Alberto Caro ◽  
Blanca Leoz ◽  
...  
Keyword(s):  
Arterial Hypertension ◽  
Clinical Management ◽  
Cox Regression ◽  
Angiotensin Receptor Blockers ◽  
Treatment Options ◽  
Clinical Manifestations ◽  
Specific Treatment ◽  
Hospitalized Patients ◽  
Thrombosis Prophylaxis ◽  
The Impact

The most severe clinical manifestations of the Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), are due to an unbalanced immune response and a pro-thrombotic hemostatic disturbance, with arterial hypertension or diabetes as acknowledged risk factors. While waiting for a specific treatment, the clinical management of hospitalized patients is still a matter of debate, and the effectiveness of treatments to manage clinical manifestations and comorbidities has been questioned. In this study, we aim to assess the impact of the clinical management of arterial hypertension, inflammation and thrombosis on the survival of COVID-19 patients. The Spanish cohorts included in this observational retrospective study are from HM Hospitales (2035 patients) and from Hospital Universitario Central de Asturias (72 patients). Kaplan Meier survival curves, Cox regression and propensity score matching analyses were employed, considering demographic variables, comorbidities and treatment arms (when opportune) as covariates. The management of arterial hypertension with angiotensin-converting enzyme 2 (ACE2) inhibitors or angiotensin receptor blockers is not detrimental, as was initially reported, and neither was the use of non-steroidal anti-inflammatory drugs (NSAIDs). On the contrary, our analysis shows that the use on itself of corticosteroids is not beneficial. Importantly, the management of COVID-19 patients with low molecular weight heparin (LMWH) as an anticoagulant significantly improves the survival of hospitalized patients. These results delineate the current treatment options under debate, supporting the effectiveness of thrombosis prophylaxis on COVID-19 patients as a first-line treatment without the need for compromising the treatment of comorbidities, while suggesting cautiousness when administering corticosteroids.

scholarly journals Periprosthetic fractures of the knee: a comprehensive review

2019 ◽  
Vol 30 (3) ◽  
pp. 387-399 ◽  
Author(s):  
Vadim Benkovich ◽  
Yuri Klassov ◽  
Boris Mazilis ◽  
Shlomo Bloom
Keyword(s):  
Knee Arthroplasty ◽  
Rare Complication ◽  
Treatment Options ◽  
Degenerative Joint Disease ◽  
Joint Arthroplasty ◽  
Joint Disease ◽  
Periprosthetic Fractures ◽  
Complex Injury ◽  
Life Threatening ◽  
Total Knee

AbstractDemographic changes have resulted in an increase in the number of older patients diagnosed with degenerative joint disease. Developments in the field of joint arthroplasty allow a broader population to improve their lifestyles. An increased demand for knee arthroplasty has led to a rise in operations performed worldwide. Although there has been a constant propagation of technology and an increase in medical staffing at a professional level, many patients still encounter complications. Though rare, these factors may lead to life-threatening scenarios and a devastating effect on the success of the operation. One such rare complication includes periprosthetic fractures around the knee, a complex injury which requires a cautious and experienced approach. In this review, we analyze the prevalence, risk factors and classification, investigation and treatment options for periprosthetic fractures with total knee arthroplasty.

scholarly journals The Management of Older Adults with Pancreatic Adenocarcinoma

Geriatrics ◽  
2018 ◽  
Vol 3 (4) ◽  
pp. 85 ◽  
Author(s):  
John Ogden ◽  
Hao Xie ◽  
Wen Ma ◽  
Joleen Hubbard
Keyword(s):  
Older Adults ◽  
Pancreatic Cancer ◽  
Clinical Trial ◽  
Treatment Options ◽  
Karnofsky Performance Score ◽  
Nccn Guidelines ◽  
The Us ◽  
Localized Disease ◽  
Clinical Trial Enrollment ◽  
Current Guidelines

Pancreatic cancer is the eleventh most common cancer, yet it is the third leading cause of mortality. It is also largely a disease of older adults, with the median age of 71 at diagnosis in the US, with <1% of diagnoses occurring prior to age 50. Current NCCN guidelines recommend surgery for localized disease, followed by adjuvant therapy and/or consideration of enrollment in a clinical trial. For metastatic disease, current guidelines recommend clinical trial enrollment or systemic chemotherapy based on results from the landmark ACCORD-11 and MPACT trials. However, these trials focused heavily on younger, more fit patients, with the ACCORD-11 trial excluding patients over age 75 and the MPACT trial having 92% of its patients with a Karnofsky performance score >80. This article summarizes the available evidence in current literature in regards to the best treatment options for older adults, who represent the majority of pancreatic cancer diagnoses.

scholarly journals Induction of WNT16 via Peptide-mRNA Nanoparticle-Based Delivery Maintains Cartilage Homeostasis

Pharmaceutics ◽  
2020 ◽  
Vol 12 (1) ◽  
pp. 73
Author(s):  
Huimin Yan ◽  
Ying Hu ◽  
Antonina Akk ◽  
Muhammad Farooq Rai ◽  
Hua Pan ◽  
...  
Keyword(s):  
Messenger Rna ◽  
Treatment Options ◽  
Cartilage Explants ◽  
Joint Disease ◽  
Chondrocyte Apoptosis ◽  
Proof Of Concept ◽  
Human Cartilage ◽  
Cartilage Homeostasis ◽  
Wide Range ◽  
Significant Disability

Osteoarthritis (OA) is a progressive joint disease that causes significant disability and pain and for which there are limited treatment options. We posit that delivery of anabolic factors that protect and maintain cartilage homeostasis will halt or retard OA progression. We employ a peptide-based nanoplatform to deliver Wingless and the name Int-1 (WNT) 16 messenger RNA (mRNA) to human cartilage explants. The peptide forms a self-assembled nanocomplex of approximately 65 nm in size when incubated with WNT16 mRNA. The complex is further stabilized with hyaluronic acid (HA) for enhanced cellular uptake. Delivery of peptide-WNT16 mRNA nanocomplex to human cartilage explants antagonizes canonical β-catenin/WNT3a signaling, leading to increased lubricin production and decreased chondrocyte apoptosis. This is a proof-of-concept study showing that mRNA can be efficiently delivered to articular cartilage, an avascular tissue that is poorly accessible even when drugs are intra-articularly (IA) administered. The ability to accommodate a wide range of oligonucleotides suggests that this platform may find use in a broad range of clinical applications.

scholarly journals Hyaluronic acid preparations in the treatment of osteoarthritis: is it clear to us?

2018 ◽  
Vol 12 (3) ◽  
pp. 40-52 ◽  
Author(s):  
A. E. Karateev ◽  
Е. Yu. Pogozheva ◽  
V. N. Amirdzhanova ◽  
Е. S. Filatova
Keyword(s):  
Hyaluronic Acid ◽  
Clinical Practice ◽  
Treatment Options ◽  
Social Activity ◽  
Joint Disease ◽  
Saline Injection ◽  
Comparative Efficacy ◽  
Joint Function ◽  
Therapeutic Value ◽  
Real Clinical Practice

Osteoarthritis (OA) is a progressive joint disease that causes persistent pain, limited joint function, disability, and loss of social activity. One of the most popular OA treatment options is intra-articular (I/A) hyaluronic acid (HA) injections. This procedure was introduced into clinical practice in the early 1980s and is used universally. To date, there is no consensus on the therapeutic value of HA. Many investigations confirm that HA is effective as not only a symptomatic agent, but also a structure-modifying one. Some works also show that the effect of HA is equal or only slightly superior to that of placebo (I/A saline injection). The review describes the mechanism of action and comparative efficacy of various (lowand high-molecular weight) HA preparations, as well as the safety of their use in real clinical practice.

scholarly journals ISMAR-study presentation: in-hospital epidemiology and clinical management of respiratory and cardiac comorbidities in cardiac and respiratory disease units

2014 ◽  
Vol 9 ◽  
Author(s):  
Roberto Tramarin ◽  
Mario Polverino ◽  
Maurizio Volterrani ◽  
Bruna Girardi ◽  
Claudio Chimini ◽  
...  
Keyword(s):  
Clinical Practice ◽  
Clinical Management ◽  
Real Life ◽  
Length Of Hospital Stay ◽  
Relevant Information ◽  
Hospital Epidemiology ◽  
Discharge Data ◽  
Definition Of ◽  
Current Guidelines

Background: Cardiovascular and respiratory diseases are leading causes of morbidity and their co-occurrence has important implications in mortality and other outcomes. Even the most recent guidelines do not reliably address clinical, prognostic, and therapeutic concerns due to the overlap of respiratory and cardiac diseases. Study objectives and design: In order to evaluate in the reality of clinical practice the epidemiology and the reciprocal impact of cardio-pulmonary comorbidity on the clinical management, diagnostic workup and treatment, 1,500 cardiac and 1,500 respiratory inpatients, admitted in acute and rehabilitation units, will be enrolled in a multicenter, nationwide, prospective observational study. For this purpose, each center will enroll at least 50 consecutive patients. At discharge, data analysis will be aimed at the definition of cardiac and pulmonary inpatient comorbidity prevalence, demographic characteristics, length of hospital stay, and risk factors, taking into account also procedures, pharmacological and non-pharmacological treatment, and follow up in patients with cardio-respiratory comorbidity. Conclusions: The purely observational design of the study aims to give new relevant information on the assessment and management of overlapping patients in real life clinical practice, and new insight for improvement and implementation of current guidelines on the management of individual diseases.

scholarly journals Germline PALB2 Variants and PARP Inhibitors in Endometrial Cancer

2021 ◽  
Vol 19 (11) ◽  
pp. 1212-1217
Author(s):  
Michael A. Cilento ◽  
Nicola K. Poplawski ◽  
Sellvakumaram Paramasivam ◽  
David M. Thomas ◽  
Ganessan Kichenadasse
Keyword(s):  
Endometrial Carcinoma ◽  
Treatment Options ◽  
Parp Inhibitors ◽  
Response To Treatment ◽  
Parp Inhibition ◽  
Ovarian Cancer Risk ◽  
Additional Question ◽  
Prostate Cancers ◽  
Risk Reducing ◽  
Female Carriers

PARP inhibitors are orally administered antineoplastic agents that affect the homologous recombination (HR) repair pathway, and are approved by the FDA for the treatment of ovarian, breast, pancreatic, and prostate cancers. This report presents a case of recurrent endometrial carcinoma occurring in a woman with a germline pathogenic PALB2 whole-exon deletion. This uncommon finding in a patient with endometrial carcinoma provided the opportunity to use a management strategy of PARP inhibition with olaparib, resulting in a prolonged response to treatment; however, disease progression eventually occurred. Further studies are required to elucidate the mechanisms underlying resistance to PARP inhibition, and the potential future treatment options in this setting. Current recommendations for risk management of female carriers of PALB2 variants focus on breast and ovarian cancer risk. This case raises the additional question of a potential role for risk-reducing hysterectomy in female carriers of PALB2 variants.

scholarly journals USP7 Inhibition Alleviates H2O2-Induced Injury in Chondrocytes via Inhibiting NOX4/NLRP3 Pathway

2021 ◽  
Vol 11 ◽  
Author(s):  
Gang Liu ◽  
Qingbai Liu ◽  
Bin Yan ◽  
Ziqiang Zhu ◽  
Yaozeng Xu
Keyword(s):  
Treatment Options ◽  
Current Treatment ◽  
Pathological Process ◽  
Joint Disease ◽  
Matrix Remodeling ◽  
Ros Production ◽  
Caspase 1 ◽  
Enhanced Production

Osteoarthritis (OA), the most common form of arthritis, is a very common joint disease that often affects middle-aged to elderly people. However, current treatment options for OA are predominantly palliative. Thus, understanding its pathological process and exploring its potential therapeutic approaches are of great importance. Rat chondrocytes were isolated and exposed to hydrogen peroxide (H2O2) to mimic OA. The effects of H2O2 on ubiquitin-specific protease 7 (USP7) expression, reactive oxygen species (ROS) levels, proliferation, inflammatory cytokine release, and pyroptosis were measured. USP7 was knocked down (KD) or overexpressed to investigate the role of USP7 in OA. Co-immunoprecipitation (Co-IP) was used to study the interaction between USP7 and NAD(P)H oxidases (NOX)4 as well as NOX4 ubiquitination. NOX4 inhibitor was applied to study the involvement of NOX4 in USP7-mediated OA development. USP7 inhibitor was given to OA animals to further investigate the role of USP7 in OA in vivo. Moreover, H2O2 treatment significantly increased USP7 expression, enhanced ROS levels, and inhibited proliferation in rat chondrocytes. The overexpression of USP7 enhanced pyroptosis, ROS production, interleukin (IL)-1β and IL-18 levels, and the expression level of NLRP3, GSDMD-N, active caspase-1, pro-caspase-1, matrix metalloproteinases (MMP) 1, and MMP13, which was abolished by ROS inhibition. The USP7 KD protected rat chondrocytes against H2O2-induced injury. Co-IP results showed that USP7 interacted with NOX4, and USP7 KD enhanced NOX4 ubiquitinylation. The inhibition of NOX4 blocked the pro-OA effect of USP7. Moreover, the USP7 inhibitor given to OA animals suppressed OA in vivo. USP7 inhibited NOX4 ubiquitination for degradation which leads to elevated ROS production. ROS subsequently activates NLPR3 inflammasome, leading to enhanced production of IL-1β and IL-18, GSDMD-N-dependent pyroptosis, and extracellular matrix remodeling. Thus, UPS7 contributes to the progression of OA via NOX4/ROS/NLPR3 axis.

scholarly journals Rewriting One Patient’s Story of Disrupted Sleep

2019 ◽  
pp. 161-172
Author(s):  
Samantha Domingo ◽  
Michelle L. Drerup
Keyword(s):  
Social Support ◽  
Treatment Adherence ◽  
Relaxation Training ◽  
Behavioral Therapy ◽  
Treatment Options ◽  
Cognitive Restructuring ◽  
Sleep Hygiene ◽  
Sleep Restriction ◽  
Insomnia Disorder

This chapter covers treatment options for individuals with chronic insomnia disorder. We describe the effectiveness of cognitive behavioral therapy for insomnia (CBT-i) and various modalities of delivery of the treatment. CBT-i is an alternative treatment for insomnia that has been demonstrated to be as successful as pharmacological therapies in the short term, and more effective in the long term. CBT-i comprises sleep restriction, stimulus control, relaxation training, sleep hygiene, and cognitive restructuring. The authors examine group CBT-i as a way to increase social support and enhance treatment adherence. Computerized CBT-i is a newer option to provide increased access to this treatment.

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