Role of Palliative Care in the Supportive Management of AL Amyloidosis-A Review.

Light chain amyloidosis is a plasma-cell disorder with a poor prognosis. It is a progressive condition, causing worsening pain, disability, and life-limiting complications involving multiple organ systems. The medical regimen can be complex, including chemotherapy or immunotherapy for the disease itself, as well as treatment for pain, gastrointestinal and cardiorespiratory symptoms, and various secondary symptoms. Patients and their families must have a realistic awareness of the illness and of the goals and limitations of treatments in making informed decisions about medical therapy, supportive management, and end-of-life planning. Palliative care services can thus improve patients' quality of life and may even reduce overall treatment costs. Light chain (AL) amyloidosis is a clonal plasma cell disorder characterized by the excessive secretion of light chains by an indolent plasma cell clone that gradually accumulates in vital organs as amyloid fibrils and leads to end-organ damage. With progressive disease, most patients develop diverse clinical symptoms and complications that negatively impact quality of life and increase mortality. Complications include cardiac problems including heart failure, hypotension, pleural effusions, renal involvement including nephrotic syndrome with peripheral edema, gastrointestinal symptoms leading to anorexia and cachexia, complex pain syndromes, and mood disorders. The prognosis of patients with advanced AL amyloidosis is dismal. With such a complex presentation, and high morbidity and mortality rates, there is a critical need for the establishment of a palliative care program in clinical management. This paper provides an evidence-based overview of the integration of palliative care in the clinical management of AL amyloidosis as a means of reducing ER visits, rehospitalizations, and in-hospital mortality. We also discuss potential future collaborative directions in various aspects of clinical care related to AL amyloidosis.

Interventional pain management in cancer patients-a scoping review.

BACKGROUND: Pain is the most prevalent symptom in cancer patients. To improve pain care, World Health Organization (WHO) Pain ladder was introduced in 1986 as a template for choosing pain medications in oncological settings. Since then, advancements in oncological treatments have improved the survival of cancer patients, requiring prolonged analgesia in various treatment stages. Additionally, there have been newer challenges in pain management with opioid epidemic and associated opioid use disorders. This has shifted the focus from WHO Pain Ladder and brought new importance to the rapidly evolving realm of interventional pain modalities for cancer pain management. This article reviews such interventional pain and minimally invasive neurosurgical options for pain management in cancer patients. METHODS: Systemic literature search in PubMed, Cochrane, and Embase. This included review articles, randomized controlled trials, non-randomized clinical trials (RCTs), and case series. RESULTS: A large array of interventional pain modalities are available for oncological pain management. These modalities carry relatively lower risk and provide effective analgesia while reducing concerns related to opioid use disorder. They target various areas in the anatomical and physiological pain pathways and provide more focused options for pain management at various stages of cancer and survivorship. Additionally, with improved sterile techniques, better imaging modalities, and growing technical and clinical expertise, interventional pain modalities offer a safe and often more efficacious method of pain management nowadays. Procedural modalities like intrathecal (IT) pumps, neuromodulation, kyphoplasty, and newer more targeted ablative techniques are now increasingly finding more roles and indications in cancer population. CONCLUSIONS: Interventional pain techniques are rapidly evolving and have become an integral part of cancer pain management. They can provide an additional option for cancer pain management, and can help reduce opioid consumption, and associated opioid side effects. With improvement in imaging modalities, procedural techniques, hardware, and infection control, they have a good safety profile and provide a rapid and efficacious approach for cancer pain management. This review articles aims to provide a basic understanding of various interventional pain modalities, their indications, efficacy, safety data, and associated complications.

Ocular complications of antineoplastic therapies.

Ocular complications of antineoplastic agents can have a profound effect on the quality of life of cancer patients. New oncologic treatments like monoclonal antibodies, immunotherapies, antibody-drug conjugates, checkpoint inhibitors and growth factor receptors have resulted in increased ocular complications. These ocular complications differs in respect to distinct mechanisms of actions and lead to significant challenges in the management of cancer patients. In this review, we reviewed literature, clinical studies and cases detailing ocular complications due to administration of antineoplastic agents and emphasized the need for communication between oncologists and ophthalmologists toward early detection and management of ocular complications.

Ocular side effects can have a large impact on the quality of life on patients with cancer. New ways to treat cancer greatly increased patient prognosis, however, it has also resulted in increased ocular complications. This paper aims to review past medical literature, clinical studies, and case reports to describe the types of ocular complications that may arise from administration of antineoplastic agents. This paper emphasizes the need for communication between oncologists and ophthalmologists toward early detection and management for better patient care.

Flexure Performance of Ferrocement Panels Using SBR Latex and Polypropylene Fibers with PVC and Iron Welded Meshes.

Ferrocement panels are thin-section panels that are widely used in lightweight construction. Due to lesser flexural stiffness, they are susceptible to surface cracking. Water may penetrate through these cracks and may cause corrosion of conventional thin steel wire mesh. This corrosion is one of the major factors which affect the load-carrying and durability of ferrocement panels. There is a need to improve the mechanical performance of ferrocement panels either through using some non-corrodible reinforcing mesh or through improving the cracking behavior of the mortar mix. In the present experimental work, PVC plastic wire mesh is employed to address this problem. SBR latex and polypropylene (PP) fibers are also utilized as admixtures to control the micro-cracking and improve the energy absorption capacity. The main idea is to improve the structural performance of ferrocement panels that may be utilized in lightweight, low-cost house construction and sustainable construction. The ultimate flexure strength of ferrocement panels employing PVC plastic wire mesh, welded iron mesh, SBR latex, and PP fibers is the subject of the research. Test variables are the type of mesh layer, the dosage of PP fiber, and SBR latex. Experimental tests are conducted on 16 simply supported panels of size 1000 × 450 mm and subjected to four-point bending test. Results indicate that the addition of latex and PP fibers only controls the initial stiffness and does not have any significant effect on ultimate load. Due to the increased bonding between cement paste and fine aggregates, the addition of SBR latex improves the flexural strength by 12.59% and 11.01% for iron mesh (SI) and PVC plastic mesh (SP), respectively. The results also indicate an improvement in the flexure toughness of specimens with PVC mesh as compared to specimens with iron welded mesh; however, a smaller peak load is observed (i.e., 12.21% for control specimens) compared with the specimen with welded iron mesh. The failure patterns of the specimens with PVC plastic mesh exhibit a smeared cracking pattern that shows that they are more ductile compared to samples with iron mesh.

Pilot Study Evaluating Cross-Disciplinary Educational Material to Improve Patients' Knowledge of Palliative Radiation Therapy.

Palliative radiation therapy (PRT) is underutilized, partially due to misconceptions about its risks, benefits, and indications. The objective of this pilot study was to determine if patients with metastatic cancer would gain knowledge from educational material describing PRT and perceive it as useful in their care. A one-page handout conveying information about the purpose, logistics, benefits, risks, and common indications for PRT was offered to patients undergoing treatment for incurable, metastatic solid tumors in one palliative care clinic and four medical oncology clinics. Participants read the handout, then completed a questionnaire assessing its perceived value. Seventy patients participated between June and December 2021. Sixty-five patients (93%) felt they learned from the handout (40% learned "lots"), and 69 (99%) felt the information was useful (53% "very useful"). Twenty-one patients (30%) were previously unaware that PRT can relieve symptoms, 55 (79%) were unaware that PRT can be delivered in five treatments or less, and 43 (61%) were unaware that PRT usually has few side effects. Sixteen patients (23%) felt they currently had symptoms not being treated well enough, and 34 (49%) felt they had symptoms that radiation might help with. Afterwards, most patients felt more comfortable bringing symptoms to a medical oncologist's (n = 57, 78%) or radiation oncologist's (n = 51, 70%) attention. Patient-directed educational material about PRT, provided outside of a radiation oncology department, was perceived by patients as improving their knowledge and adding value in their care, independent of prior exposure to a radiation oncologist.

Racial disparities in follow-up care of early-stage lung cancer survivors.

PURPOSE: To investigate if race impacts receipt of follow-up care in lung cancer survivors, we conducted a cross-sectional study in lung cancer survivors recruited through the New Jersey State Cancer Registry (NJSCR). METHODS: Between May 2019 and December 2019, survivors of early-stage NSCLC were identified and recruited from the NJSCR. Eligible participants were asked to complete a paper survey questionnaire and medical record release form sent to them by mail. RESULTS: Of the 112 survivors included in the analysis, 78 (70%) were non-Hispanic (NH) Whites and 34 (30%) were NH Blacks. Mean age was 67 years, 61% were female, and 92% had cancer in remission. A total of 82% of participants reported receiving a surveillance scan (CT or PET) within 1 year of completing the study survey. More NH White survivors received a scan within a year compared to NH Black survivors (89% vs 70%; p = 0.02). More NH White survivors (94%) reported that they were informed of the need for follow-up care by their provider compared to NH Blacks (71%; p = 0.002). Only 57% survivors reported receiving a treatment summary. Significant barriers to care were out-of-pocket costs (24%), non-coverage of test (12.5%), and lack of insurance (10%). CONCLUSIONS: Significant disparity was identified between NH Blacks and NH Whites in receipt of surveillance scans, as well as in receiving information about need for follow-up care. Low income, lack of insurance, and other financial concerns were identified as significant barriers to follow-up care. IMPLICATIONS FOR CANCER SURVIVORS: Future interventions to increase survivorship care should target specific unmet needs identified in each survivor population.

Telemedicine family meetings in palliative care: etiquette.

The use of telemedicine in healthcare settings has continued to increase over the last few years. This has led to new communication-based concerns in palliative care settings. To date, there are no specific guidelines on telemedicine etiqettes relating to conducting online family meetings. This case report discusses some etiquette-failures in a telemedicine family meeting, and offers suggestions to improve communication etiqettes or 'webside manners' in these settings.

Top Ten Tips Palliative Care Clinicians Should Know About Urological Care.

Patients receiving palliative care (PC) can present with or develop a host of urological needs or complications. These needs can include attention to sexual health, urinary incontinence, genitourinary bleeding, and urinary tract obstruction by benign, malignant, or urinary stone diseases. These varied conditions require that PC clinicians understand invasive and noninvasive medical, surgical, and radiation options for treatment. This article, written by a team of urologists, geriatricians, and PC specialists, offers information and guidance to PC teams in an accessible "Top Ten Tips" format to increase comfort with and skills around assessment, evaluation, and specialist referral for urological conditions common in the PC setting.

High-grade desmoplastic infantile astrocytoma in a 1-year-old child with Down's syndrome: a case report.

BACKGROUND: Down's syndrome is the most common chromosomal abnormality in humans. It has been associated with central nervous system tumors such as primary acute lymphoblastic leukemia and germinomas, but desmoplastic infantile astrocytoma has not yet been reported with Down's syndrome. Desmoplastic infantile astrocytoma is a rare intracranial tumor that mostly occurs in the first 2 years of life. It usually presents as a large, aggressive tumor with both solid and cystic components. Genetically, it has been linked to the BRAF V600E mutation. Despite the rapid growth pattern, it usually has a favorable prognosis after neurosurgical excision. The presence of this extremely rare, genetically linked tumor, and its combination with Down's syndrome, the most common human genetic defect, makes this a very novel clinical presentation. It also raises a very research-worthy question of an undiscovered link between these two genetic disorders. CASE PRESENTATION: In this case, we report a 1-year-old Pakistani origin male child with Down's syndrome, who presented with progressive macrocephaly and developmental regression over the last 2 months. He was unable to sit by himself, and had lost his handgrip bilaterally. Down's Syndrome was diagnosed soon after birth, based on typical facial features and presence of palmar crease, and later confirmed karyotypically for Trisomy 21. Upon presentation, initial blood tests did not show any abnormality. Magnetic resonance imaging of the brain was done, and showed a mixed intensity cystic mass with solid dural component posteriorly in the right parieto temporo occipital region. Craniotomy was performed, and about 85% of the tumor mass was excised. Histological examination and immunochemistry confirmed the suspected radiological diagnosis of desmoplastic infantile astrocytoma. After surgical excision, our patient gradually reacquired his previously regressed developmental milestones. Unfortunately, the remaining mass, which could not be excised due to its attachment to the highly vascular dura mater, showed regrowth on repeat brain magnetic resonance imaging. As his parents did not consent to further surgery, chemotherapy was offered as the next treatment option to prevent tumor regrowth. CONCLUSIONS: This case report highlights the need for more case data and research to understand desmoplastic infantile astrocytoma, and their genetic correlation with Down's syndrome. From a clinical standpoint, since desmoplastic infantile astrocytoma has a good postresection prognosis in a majority of early-diagnosed clinical cases, pediatricians, radiologists, and pathologists should consider desmoplastic infantile astrocytoma in their initial differential diagnosis in Down's syndrome patients with macrocephaly and developmental regression during the first 2 years of life.

Extensive stage small cell lung cancer (ES-SCLC) and palliative care disparities: a national cancer database study.

OBJECTIVES: Literature on disparities in palliative care receipt among extensive stage small cell lung cancer (ES-SCLC) patients is scarce. The purpose of this study was to examine disparities in palliative care receipt among ES-SCLC patients. METHODS: Patients aged 40 years or older diagnosed with ES-SCLC between 2004 and 2015 in the National Cancer DataBase (NCDB) were eligible. Two palliative care variables were created: (1) no receipt of any palliative care and (2) no receipt of pain management-palliative care. The latter variable indicated pain management receipt among those who received any palliative care. Log binomial regression models were constructed to calculate risk ratios by covariates. Unadjusted and mutually adjusted models were created for both variables. RESULTS: Among 83 175 patients, the risk of no palliative care receipt was higher among Blacks compared with Whites in unadjusted and adjusted models (both model HRs 1.02; 95% CIs 1.00 to 1.03, p<0.05). Patients older than 59 years were at a higher risk of not receiving palliative care than younger patients (HR 1.02; 95% CI 1.01 to 1.03 for 59-66, HR 1.04; 95% CI 1.03 to 1.05 for 66-74, HR 1.06; 95% CI 1.05 to 1.08 for >74). Among 19 931 patients, the risk of no pain management-palliative care was higher among black patients on unadjusted analysis (HR 1.02; 95% CI 1.00 to 1.03, p<0.05). Patients between 66 and 74 years were at a higher risk of not receiving pain management-palliative care than patients younger than 59 years (HR 1.02; 95% CI 1.00 to 1.03, p<0.05). CONCLUSIONS: Significant disparities exist in palliative care receipt among ES-SCLC patients.

Trametinib: Could It Be a Promising Drug to Treat Atypical Chronic Myeloid Leukemia?

Atypical chronic myeloid leukemia (aCML) is a rare disease that is currently classified under the myelodysplastic (MDS)/myeloproliferative neoplasm (MPN) disease spectrum. MDS/MPN diseases are characterized by the absence of the Philadelphia (Ph) chromosome and the overlap between bone marrow fibrosis and dysplastic features. The Ph chromosome, resulting from BCR-ABL1 translocation, helps to distinguish aCML from chronic myeloid leukemia (CML). The currently reported incidence of aCML is imprecise because aCML is diagnosed primarily based on morphological features and other unspecified laboratory findings, and there is an especially high chance of under-diagnosis of aCML and other MDS/MPN diseases. Recent advances in next-generation sequencing (NGS) have allowed a greater understanding of the nature of aCML, providing better opportunities to achieve higher diagnostic accuracy and for the use of more targeted treatment to achieve better outcomes. Herein, we present a case of a 68-year-old woman who came to our hospital complaining of shortness of breath, fatigue, and weakness, who was found to have significantly increased leukocytosis, hepatosplenomegaly, and was negative for the Ph chromosome. Further investigations with NGS revealed mutations in ASXL1, GATA2, NRAS, and SRSF2 but not CSF3R. In addition to this, peripheral smear and bone marrow aspiration findings were suggestive of aCML based on specific morphological findings. Since the patient was ineligible for a stem cell transplant (SCT), symptomatic treatment was started with cell transfusion; however, the patient continued to have symptomatic anemia that required multiple transfusions. A trial with trametinib, a mitogen-activated protein kinase kinase (MEK) inhibitor, was later started as a targeted therapy based on one of her genetic mutations. Interestingly, the patient's blood counts stabilized, she reported feeling better, and she did not need any blood transfusions for four consecutive months during treatment with trametinib. Unfortunately, our patient later died from sepsis resulting from secondary infections. In light of the significant advancements in NGS, clinicians should always consider utilizing it as a helpful tool to not only establish a rare diagnosis of aCML but also to offer the best available targeted therapy when applicable. This might alleviate the burden associated with the poor prognosis of aCML.

Physical activity in Pakistani perspective: issues, implications, and recommendations.

It has been established that the benefits of physical activity on physical, psychological and social aspects of humans are substantially correlated with cardiovascular disorders, obesity, cancer, social anxieties and depressions, and enhanced socio-economic profile. In children and adolescents, physical activity has been correlated with academic achievements, social harmony and mental health. Similarly, in adults/old people, physical activity has been of variable benefits, bringing the chances of prolonged healthier lives. Though substantial work has been reported globally, there still is a paucity of literature in Pakistan regarding various aspects of physical activity. The current narrative review was planned to summarise the research work conducted in, or has emanated from, Pakistan in teens/college/school and university students, and adults from Pakistan on associated aspects of physical activity, its present scenario, pitfalls, future horizons, and to ultimately deduce gaps in research areas which can be taken up for the directional approach to enhance physical activity within the Pakistani community.

Current Updates on the Management of AL Amyloidosis.

Systemic immunoglobulin light chain (AL) amyloidosis is a rare but fatal disease. It results from clonal proliferation of plasma cells with excessive production of insoluble misfolded proteins that aggregate in the extracellular matrix, causing damage to the normal architecture and function of various organs. For decades, treatment for AL amyloidosis was based mainly on therapeutic agents previously studied for its more common counterpart, multiple myeloma. As the prevalence and incidence of AL amyloidosis have increased, ongoing research has been conducted with treatments typically used in myeloma with varying success. In this review, we focus on current treatment strategies and updates to clinical guidelines and therapeutics for AL amyloidosis.

Outcome of Core Binding Factor Acute Myeloid Leukemia in Children: A Single-Center Experience.

Childhood acute myeloid leukemia (AML) harboring core binding factor (CBF)-associated translocations are considered as a favorable cytogenetic subgroup. The 2 major subtypes of CBF-AML include t(8;21) and inversion of chromosome 16, accounting for ∼25% of patients. Because of expensive and toxic treatment, which may require hospitalization during the entire course of induction chemotherapy, most of the centers in Pakistan neither workup for this low-risk entity nor offer curative treatment. Therefore, we adopted an approach of screening AML cases for the presence of CBF with the rationale of offering curative treatment to this subgroup. Data of 244 cases were reviewed, and translocations were found in 72 (34%) patients among them, 59 (82%) had t(8;21) and 13 (18%) showed inversion of chromosome 16. The event-free survival with and without abandonment was 36% and 40%, respectively. Among 44 patients who completed treatment, 26 (59%) are leukemia-free, while 18 (41%) relapsed. None of the relapsed patients received salvage chemotherapy or hematopoietic stem cell transplant. Treatment-related mortality and abandonment was found in 24% and 10% of patients, respectively. The frequency of CBF-AML is higher in our study; however, poor outcome demands holistic measures in supportive care to improve the survival.

Radiological, functional, and anatomical outcome in patients with osteoarthritic knee undergoing high tibial osteotomy.

OBJECTIVE: To determine the radiological, functional, and anatomical outcome in patients with osteoarthritic knee undergoing high tibial osteotomy (HTO). DESIGN OF STUDY: Descriptive case series. Study duration and settings: The present study was a descriptive case series carried out at the Orthopedic Departments of District Head Quarter Hospital Faisalabad affiliated with Faisalabad Medical University, Faisalabad from Jan 2014 to March 2018. METHODOLOGY: This study involved 40 patients of both genders, aged between 40 and 65 years having advanced degenerative disease of knee limited to medial compartment of joint. These patients were treated by medial open wedge high tibial osteotomy (OWHTO) and outcome was evaluated after 5 years of surgery in terms of radiological knee mechanics, functional outcome scores, and arthroscopic evidence of cartilage regeneration in the medial compartment. A signed written consent was taken from every patient. FINDINGS: There was a female predominance with a male-to-female ratio of 1:4. The mean age of the patients was 53.2 ± 6.9 years. The values of the radiographic parameters significantly changed from pre-operative condition after HTO; mechanical tibiofemoral angle [MTFA, (-8.1 ± 1.2° vs. 2.5 ± 1.2°; p-value < 0.0001)], tibial plateau inclination [TPI, (5.3 ± 1.1° vs. 3.4 ± 1.1°; p-value < 0.0001)], knee joint line orientation relative to the ground [G-KJLO, (0.3 ± 0.1° vs. 4.6 ± 1.5°; p-value < 0.0001)], and ankle joint line orientation relative to the ground [G-AJLO (8.3 ± 3.2° vs. 2.3 ± 1.7°; p-value < 0.0001)]. There was significant improvement in patient's functional status; KOOS-ADL score (45.5 ± 7.8 vs. 73.7 ± 8.6; p-value < 0.0001), International Knee Documentation Committee (IKDC) score (42.4 ± 6.9 vs. 68.5 ± 12.7; p-value < 0.0001), International Knee Society (IKS) score (149.4 ± 11.9 vs. 179.4 ± 10.2; p-value < 0.0001), Knee Society Score [KSS, (54.2 ± 5.6 vs. 69.7 ± 12.7; p-value < 0.0001)], and Hospital for Special Surgery [HSS, (50.8 ± 3.3 vs. 64.8 ± 10.7; p-value < 0.0001)]. 42.5% patients showed excellent regeneration of femoral and 30.0% patients showed excellent regeneration of tibial cartilage in the medial compartment. CONCLUSION: By significantly alternating the knee biomechanics, HTO was found to unload the medial compartment leading to regeneration of the articular cartilage and significant improvement in patient's symptoms and quality of life. It is therefore recommended in the management of patients with arthritic changes limited to medial compartment only.

Ferromagnetism in CVT grown tungsten diselenide single crystals with nickel doping.

Two dimensional (2D) single crystal layered transition materials have had extensive consideration owing to their interesting magnetic properties, originating from their lattices and strong spin-orbit coupling, which make them of vital importance for spintronic applications. Herein, we present synthesis of a highly crystalline tungsten diselenide layered single crystal grown by chemical vapor transport technique and doped with nickel (Ni) to tailor its magnetic properties. The pristine WSe2 single crystal and Ni-doped crystal were characterized and analyzed for magnetic properties using both experimental and computational aspects. It was found that the magnetic behavior of the 2D layered WSe2 crystal changed from diamagnetic to ferromagnetic after Ni-doping at all tested temperatures. Moreover, first principle density functional theory (DFT) calculations further confirmed the origin of room temperature ferromagnetism of Ni-doped WSe2, where the d-orbitals of the doped Ni atom promoted the spin moment and thus largely contributed to the magnetism change in the 2D layered material.

Prevalence and determinants of iron deficiency anemia among non-pregnant women of reproductive age in Pakistan.

BACKGROUND AND OBJECTIVES: Iron deficiency Anemia (IDA) in women of reproductive age is a recognized public health concern that impairs health and well-being in women and is associated with adverse reproductive outcomes. In Pakistan there is a dearth of up-to-date information on the prevalence and predictors of IDA. This study sought to investigate IDA in Pakistani women. METHODS AND STUDY DESIGN: Secondary analysis was performed using the National Nutrition Survey in Pakistan 2011- 2012. We used a pre-structured instrument to collect socio demographic, reproductive and nutritional data on women. We also collected anthropometric measurements and blood samples for micronutrient deficiencies. Univariate and multivariate logistic regression were used to analyse the data. RESULTS: A total of 7491 non-pregnant women aged between 15-49 years were included in the analysis. The prevalence of IDA was 18.1%. In the multivariate regression analysis; not using iron folic acid supplementation during the last pregnancy adjusted odds ratio (AOR) (95% CI) 1.31 (1.05, 1.64), a history of four or more pregnancies AOR (95% CI) 1.30 (1.04, 1.60), birth interval of <24 months AOR (95% CI) 1.27 (1.06, 1.71), household food insecurity AOR (95% CI) 1.42 (1.23, 1.63) and presence of clinical anemia AOR (95% CI) 5.82 (4.82, 7.02) were significantly associated with increased odds of IDA while with obesity AOR (95% CI) 0.60 (0.4, 0.88) showed a protective effect on IDA. CONCLUSION: To reduce IDA in Pakistani women, the country needs a multifaceted approach that incorporates iron supplementation, food fortification, improved family planning services and efforts to reduce food insecurity.