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Clinical characteristics and outcome of 318 families with familial monoclonal gammopathy: A multicenter Intergroupe Francophone du Myélome study.

Familial forms of monoclonal gammopathy, defined as multiple myeloma (MM) or Monoclonal Gammopathy of Undetermined Significance (MGUS), are relatively infrequent and most series reported in the literature describe a limited number of families. MM rarely occurs in a familial context. MGUS is observed much more commonly, which can in some cases evolve toward full-blown MM. Although recurrent cytogenetic abnormalities have been described in tumor cells of sporadic cases of MM, the pathogenesis of familial MM remains largely unexplained. In order to identify genetic factors predisposing to familia

Pemphigus vulgaris.

Pemphigus vulgaris is a chronic autoimmune bullous dermatosis that results from the production of autoantibodies against desmogleins 1 and 3. It is the most frequent and most severe form of pemphigus, occurring universally, usually between 40 and 60 years of age. It usually begins with blisters and erosions on the oral mucosa, followed by lesions on other mucous membranes and flaccid blisters on the skin, which can be disseminated. There is a clinical variant, pemphigus vegetans, which is characterized by the presence of vegetating lesions in the large folds of the skin. Clinical suspicion can

2018 ACVIM Forum Research Report Program. Seattle, Washington, June 14 - 16, 2018.

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Molecular mechanisms and clinical management of cancer bone metastasis.

As one of the most common metastatic sites of malignancies, bone has a unique microenvironment that allows metastatic tumor cells to grow and flourish. The fenestrated capillaries in the bone, bone matrix, and bone cells, including osteoblasts and osteoclasts, together maintain the homeostasis of the bone microenvironment. In contrast, tumor-derived factors act on bone components, leading to subsequent bone resorption or excessive bone formation. The various pathways involved also provide multiple targets for therapeutic strategies against bone metastases. In this review, we summarize the curr

Keyword Index for Volume 93

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Keyword Index for Volume 88

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A Review of Current Clinical Concepts in the Pathophysiology, Etiology, Diagnosis, and Management of Hypercalcemia.

Calcium is the most abundant extracellular cation in the body, and it is responsible for structural and enzymatic functions. Calcium homeostasis is regulated by 3 factors: calcitonin, vitamin D, and parathyroid hormone (PTH). Hypercalcemia is defined by a serum calcium concentration >10.5 mg/dL, and it is classified into mild, moderate, and severe, depending on calcium values. Most cases are caused by primary hyperparathyroidism and malignancies. Various mechanisms are involved in the pathophysiology of hypercalcemia, such as excessive PTH production, production of parathyroid hormone-related

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… Although most cases of primary hyperparathyroidism are sporadic, 5% to 10% of cases are related to hereditary syndromes such as multiple endocrine neoplasia, hyperparathyroidism jaw tumor syndrome, familial isolated hyperparathyroidism, neonatal severe primary hyperparathyroidism, and non-syndromic primary …

Role of PET/CT and Therapy Management of Pancreatic Neuroendocrine Tumors.

Pancreatic neuroendocrine neoplasms (panNENs) are heterogeneous neoplasms with neuroendocrine differentiation that show peculiar clinical and histomorphological features, with variable prognosis. In recent years, advances in knowledge regarding the pathophysiology and heterogeneous clinical presentation, as well as the availability of different diagnostic procedures for panNEN diagnosis and novel therapeutic options for patient clinical management, has led to the recognition of the need for an active multidisciplinary discussion for optimal patient care. Molecular imaging with positron emissio

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… Up to 10% of panNENs develop in patients with syndromes such as multiple endocrine neoplasia type 1 (MEN1), von Hippel–Lindau disease (VHLD), tuberous sclerosis complex, neurofibromatosis type 1, and glucagon cell adenomatosis. …

Idiopathic osteoporosis, Ehlers-Danlos syndrome, postural orthostatic tachycardia syndrome, and mast cell activation disorder in a 27-year-old male patient: A unique case presentation.

A young male patient presents with widespread pain and varying chronic inflammatory symptoms for three years and idiopathic low bone density for more than ten years. Based on the patient's clinical history, the patient has been diagnosed with an hypermobile Ehlers-Danlos syndrome, postural orthostatic tachycardia syndrome, and mast cell activation disorder trifecta with affiliated inflammation-induced osteoporosis.

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… The patient’s whole‐exome screen, genetic testing, and laboratory workups allowed the patient’s healthcare team to rule out many differential diagnoses, including Marfan syndrome, Loeys–Dietz syndrome, TAAD, Lynch syndrome, multiple endocrine neoplasia types 1 and 2, hypertrophic cardiomyopathy, dilated …

Keyword Index for Volume 95

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Identifying Patients With Asymptomatic Hyperparathyroidism by Serum Calcium and Vitamin D Screening in West Bengal, India.

BACKGROUND: Primary hyperparathyroidism (PHPT) is the leading cause of hypercalcemia, with solitary parathyroid adenoma responsible for most cases. Many patients with hyperparathyroidism remain asymptomatic, but early detection and parathyroidectomy can prevent complications such as nephrolithiasis, pancreatitis, and bone and kidney disorders. This study emphasizes the importance of screening for asymptomatic hyperparathyroidism to enable timely intervention. METHODS: Serum calcium and Vitamin D3 levels were screened in 6000 outpatients between March 2021 and September 2022. Patients with kno

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… Monogenic disorders like multiple endocrine neoplasia types I and II and neck irradiation account for less than 5% of cases [ 15 , 16 ]. Before the 1960s, PHPT was rare, with patients showing severe symptoms such as osteitis fibrosa cystica and kidney stones. …

Whether and How Disutilities of Adverse Events were Used in the Economic Evaluation of Drug Therapy for Cancer Treatment.

BackgroundThe disutilities of adverse events (AEs) are important inputs for cost-utility analysis (CUA), reflecting the impacts of AEs on health outcomes. Health technology assessment institutions and scholars have proposed recommendations for applying disutility values in economic evaluations.ObjectivesThis study aimed to identify the current use of disutilities of AEs as model parameters in the CUA of cancer drug therapy and to compare the discrepancies between the use of disutilities and published recommendations.MethodsA systematic search was conducted on the PubMed, Web of Science, and Co

The Relationship Between Obesity and Cancer: Epidemiology, Pathophysiology, and the Effect of Obesity Treatment on Cancer.

There is growing evidence relating to the risk of cancer in people with obesity. Obesity is already established as one of the strongest predisposing factors to cancer, and 'obesity-related' cancers have been defined in previous studies. In this review article, we examine the epidemiological relationship and describe the potential pathophysiological mechanisms that underpin the association between obesity and cancer. These include hormonal and growth factors that are in abundance in persons living with obesity and thereby increase cancer risk. Additionally, the increased disposition towards chr

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… In summary, based on the existing evidence, there is no requirement to avoid using GLP-1RAs due to the risk of thyroid cancer, apart from in patients with a personal or family history of medullary thyroid cancer or multiple endocrine neoplasia—this is already well-established as a contraindication. …

Bony metastases: assessing response to therapy with whole-body diffusion MRI.

There are no universally accepted methods for assessing tumour response in skeletal sites with metastatic disease; response is assessed by a combination of imaging tests, serum and urine biochemical markers and symptoms assessments. Whole-body diffusion magnetic resonance imaging excels at bone marrow assessments at diagnosis and for therapy evaluations. It can potentially address unmet clinical and pharmaceutical needs for a reliable measure of tumour response. Signal intensity on high b-value images and apparent diffusion coefficient values can be related to underlying biophysical properties

Medical Ozone Treatment Attenuates Male Reproductive Toxicity Induced by Bleomycin, Etoposide, and Cisplatin Regimen in an Experimental Animal Model.

The chemotherapeutic combination of bleomycin, etoposide, and cisplatin (BEP) is well-documented to exert gonadotoxic effects, ultimately leading to impaired fertility. This experimental rat study investigated the potential protective role of repeated medical ozone therapy in mitigating the deleterious effects of BEP treatment in male rats. Thirty-two adult male Sprague Dawley rats were randomly assigned to four groups: (i) a healthy control group, (ii) a group receiving injections of the BEP regimen over nine weeks, (iii) a group receiving the same BEP regimen plus medical ozone (1 mg/kg IP)

The Impact and Safety of GLP-1 Agents and Breast Cancer.

IntroductionObesity and breast cancer rates are increasing globally, with obesity prevalence more than doubling since 1990. By 2022, 44% of women were overweight, and 18% were obese. Breast cancer remains the most common malignancy among women, with 2.2 million new cases in 2020. A significant proportion of breast cancer patients are overweight or obese at diagnosis, which is associated with higher recurrence and mortality rates. Recently, GLP-1 receptor agonists (GLP-1 RAs) have emerged as remarkably effective weight loss drugs. Understanding the relationship between obesity, breast cancer, a

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… However, semaglutide is contraindicated in patients with a family or personal history of medullary thyroid cancer and in patients with multiple endocrine neoplasia type 2 until new data is available. …

Indian Expert Opinion on Cancer Care during COVID-19 Pandemic.

Coronavirus pandemic has increased human disease burden, as well as economic distress globally. Being in an immunocompromised state, patients with cancer comprise an important at-risk population for novel coronavirus disease 2019 (COVID-19) infection. It is necessary to modify individualized clinical management for every cancer patient in the context of the ongoing COVID-19 pandemic. Simultaneously, additional safety precautions for the cancer care providers are mandatory. This review will provide general recommendations in the Indian context optimizing the same.

Role of Cyclin-Dependent Kinase Inhibitors in Endometrial Cancer.

Endometrial Cancer (EC) is an important cause of death in women worldwide. Despite early diagnosis and optimal treatment of localized disease, relapsed patients have few therapeutic options because after first line therapy, currently no standard of care exists. On the basis of endocrine positivity of most endometrioid ECs, Endocrine Therapy (ET) is a reasonable and widely accepted option. Better knowledge of molecular mechanisms involved in cancer highlighted the deregulated activity of Cyclin-Dependent Kinases (CDKs) in the cell cycle as a hallmark of carcinogenesis supporting the development

Patient assessment and feasibility of treatment in older patients with cancer: results from the IN-GHO® Registry.

PurposePredicting feasibility of treatment in older patients with cancer is a major clinical task. The Initiative Geriatrische Hämatologie und Onkologie (IN-GHO®) registry prospectively collected data on the comprehensive geriatric assessment (CGA), physician's and patient's-self assessment of fitness for treatment, and the course of treatment in patients within a treatment decision aged ≥ 70 years.Patients and methodsThe registry included 3169 patients from 93 centres and evaluated clinical course and treatment outcomes 2-3 and 6 months after initial assessment. Fitness for treatment was clas

Effectiveness of Resistance Training on Fatigue in Patients Undergoing Cancer Treatment: A Meta-Analysis of Randomized Clinical Trials.

ObjectiveTo assess the effectiveness of the resistance training to improve fatigue levels in people with cancer who are enrolled in adjuvant and/or neoadjuvant treatment.MethodsMEDLINE, Web of Science, Embase, SPORTDiscus, LILACS, CENTRAL, and CINAHL databases were searched from May to December 7, 2021. Randomized clinical trials (RCT) that evaluate the effects of resistance training on fatigue levels in people undergoing cancer treatment were included. The PEDro scale was considered to assess methodological quality of studies, and the evidence was summarized through the GRADE system. The stan