Midostaurin improves quality of life and mediator-related symptoms in advanced systemic mastocytosis.

Background: Advanced systemic mastocytosis (advSM) is characterized by presence of the KIT D816V mutation and pathologic accumulation of neoplastic mast cells (MCs) in various tissues, leading to severe symptoms and organ damage (eg, cytopenias, liver dysfunction, portal hypertension, malabsorption, and weight loss). Treatment with midostaurin, an orally active multikinase/KIT inhibitor now approved for advSM in the United States and the European Union, resulted in a high rate of response accompanied by reduced MC infiltration of the bone marrow and lowered serum tryptase level.

Objective: We aimed to determine whether midostaurin improves health-related quality of life (QOL) and MC mediator-related symptoms in patients with advSM.

Factors associated with use of immunohistochemical markers in the histopathological diagnosis of cutaneous melanocytic lesions.

Background: Melanocytic tumors are often challenging and constitute almost one in four skin biopsies. Immunohistochemical (IHC) studies may assist diagnosis; however, indications for their use are not standardized.

Methods: A test set of 240 skin biopsies of melanocytic tumors was examined by 187 pathologists from 10 US states, interpreting 48 cases in Phase I and either 36 or 48 cases in Phase II.

Bone, inflammation and chronic kidney disease.

Increasing knowledge on inflammatory mediators and bone metabolism highlights the relationship between inflammation and bone disease. During acute illness, inflammatory cells and cytokines modulate bone cells activity so as to mobilize calcium seemingly to supply the metabolic requirements for immune response. In case of long lasting, chronic inflammatory states a condition of maladaptive, smouldering inflammation is realized and negatively affects calcium bone balance.

Germline MBD4 Mutations and Predisposition to Uveal Melanoma.

Background: Uveal melanoma (UM) arises from malignant transformation of melanocytes in the uveal tract of the eye. This rare tumor has a poor outcome with frequent chemo-resistant liver metastases. BAP1 is the only known predisposing gene for UM.

Correction to: Multicenter randomized controlled trial on the comparison of multi-family therapy (MFT) and systemic single-family therapy (SFT) in young patients with anorexia nervosa: study protocol of the THERAFAMBEST study.

After publication of our article [1] the authors have notified us of missing to include one name to the authorship list.

Summary of Proceedings and Expert Consensus Statements From the International Summit "Lipids in Parenteral Nutrition".

Background: The 2018 Lipids in Parenteral Nutrition summit involved a panel of experts in clinical nutrition, lipid metabolism, and pharmacology, to assess the current state of knowledge and develop expert consensus statements regarding the use of intravenous lipid emulsions in various patient populations and clinical settings. The main purpose of the consensus statements is to assist healthcare professionals by providing practical guidance on common clinical questions related to the provision of lipid emulsions as part of parenteral nutrition (PN).

Methods: The summit was designed to allow interactive discussion and consensus development.

Dermatopathologists' Experience With and Perceptions of Patient Online Access to Pathologic Test Result Reports.

Importance: Many patients presently have access to their pathologic test result reports via online patient portals, yet little is known about pathologists' perspective on this topic.

Objective: To examine dermatopathologists' experience and perceptions of patient online access to pathology reports.

Design, Setting, And Participants: A survey of 160 dermatopathologists currently practicing in the United States who are board certified and/or fellowship trained in dermatopathology was conducted between July 15, 2018, and September 23, 2019.

NHP2 deficiency impairs rRNA biogenesis and causes pulmonary fibrosis and Høyeraal-Hreidarsson syndrome.

Telomeres are nucleoprotein structures at the end of chromosomes. The telomerase complex, constituted of the catalytic subunit TERT, the RNA matrix hTR and several cofactors, including the H/ACA box ribonucleoproteins Dyskerin, NOP10, GAR1, NAF1 and NHP2, regulates telomere length. In humans, inherited defects in telomere length maintenance are responsible for a wide spectrum of clinical premature aging manifestations including pulmonary fibrosis (PF), dyskeratosis congenita (DC), bone marrow failure and predisposition to cancer.

Management of childhood aplastic anemia following liver transplantation for nonviral hepatitis: A French survey.

Background: Hepatitis-associated aplastic anemia (AA) is a rare syndrome combining acute hepatitis of variable severity and AA. Hepatitis may be severe enough to require urgent liver transplantation (LT). Herein, we describe clinical presentation and management of a cohort of pediatric patients diagnosed with AA after undergoing LT for nonviral hepatitis.

Association of Partial Chromosome 3 Deletion in Uveal Melanomas With Metastasis-Free Survival.

Importance: Studies on uveal melanomas (UMs) have demonstrated the prognostic value of 8q gain and monosomy 3, but the prognosis of UMs with partial deletion of chromosome 3 remains to be defined.

Objective: To examine the association of partial chromosome 3 deletion in UMs with metastasis-free survival.

Design, Setting, And Participants: This retrospective cohort study of 1088 consecutive comparative genomic hybridization arrays performed from May 1, 2006, to July 31, 2015, assessed patients presenting with UMs with and without partial loss of chromosome 3 at a referral center.

An overview of the anti-cancer actions of Tanshinones, derived from (Danshen).

Tanshinone is a herbal medicinal compound described in Chinese medicine, extracted from the roots of (Danshen). This family of compounds, including Tanshinone IIA and Tanshinone I, have shown remarkable potential as anti-cancer molecules, especially against breast, cervical, colorectal, gastric, lung, and prostate cancer cell lines, as well as leukaemia, melanoma, and hepatocellular carcinoma among others. Recent data has indicated that Tanshinones can modulate multiple molecular pathways such as PI3K/Akt, MAPK and JAK/STAT3, and exert their pharmacological effects against different malignancies.

Differences in knowledge about epilepsy and antiepileptic drugs among pharmacy-dispensing workers in Cambodia and in Lao PDR.

Unlabelled: Epilepsy is the most common neurological disorder encountered in primary care in Southeast Asia. People with epilepsy require long-term therapy management. Nonadherence to antiepileptic drugs (AEDs) has been identified as a major factor in suboptimal control of epilepsy.

Pathologists' agreement on treatment suggestions for melanocytic skin lesions.

Background: Although treatment guidelines exist for melanoma in situ and invasive melanoma, guidelines for other melanocytic skin lesions do not exist.

Objective: To examine pathologists' treatment suggestions for a broad spectrum of melanocytic skin lesions and compare them with existing guidelines.

Methods: Pathologists (N = 187) completed a survey and then provided diagnoses and treatment suggestions for 240 melanocytic skin lesions.

Probiotics, including nisin-based probiotics, improve clinical and microbial outcomes relevant to oral and systemic diseases.

The effects of probiotic supplementation on systemic health and gastrointestinal diseases have been investigated in numerous studies. The aim of this review is to provide an overview of probiotics and their effects on periodontal health. Probiotics show beneficial effects as adjunctive therapeutics and as stand-alone agents in the treatment and prevention of gingivitis as well as specific clinical parameters of periodontitis.

Ultrasound-induced Cavitation enhances the efficacy of Chemotherapy in a 3D Model of Pancreatic Ductal Adenocarcinoma with its microenvironment.

Pancreatic ductal adenocarcinoma (PDAC) is supported by a complex microenvironment whose physical contribution to chemoresistance could be overcome by ultrasound (US) therapy. This study aims to investigate the ability of US-induced inertial cavitation in association with chemotherapy to alter tumor cell viability via microenvironment disruption. For this purpose, we used a 3D-coculture PDAC model partially mimicking the tumor and its microenvironment.

Multimorbidity in Elderly Persons According to the Year of Diagnosis of Human Immunodeficiency Virus Infection: A Cross-sectional Dat'AIDS Cohort Study.

Background: We assessed prevalence of multimorbidity (MM) according to year of human immunodeficiency virus (HIV) diagnosis in elderly people living with HIV (PLWH).

Methods: This was a cross-sectional study of MM in PLWH aged ≥70 years from the Dat'AIDS French multicenter cohort. MM was defined as at least 3 coexistent morbidities of high blood pressure, diabetes mellitus, osteoporosis, non-AIDS cancer, chronic renal failure, cardiovascular and cerebrovascular disease, obesity, undernutrition, or hypercholesterolemia.

Patients and relatives coping with inflammatory arthritis: Care teamwork.

Objective: To explore how patients and relatives experience and talk together about their life with inflammatory arthritis.

Design: Qualitative research.

Setting: A convenience sample was used.

Consensus-based care recommendations for congenital and childhood-onset myotonic dystrophy type 1.

Purpose Of Review: Myotonic dystrophy type 1 is a multisystemic disorder caused by a noncoding triplet repeat. The age of onset is variable across the lifespan, but in its most severe form, the symptoms appear at birth (congenital myotonic dystrophy) or in the pediatric age range (childhood-onset myotonic dystrophy). These children have a range of disabilities that reduce the lifespan and cause significant morbidity.

What came first, the chicken or the egg?

Current empirical evidence does not seem to confirm that an improvement in living conditions is the cause of the shift in the human mindset toward innovation and long-term risky investment. However, it may well be one of the conditions for greater tolerance of income inequality in exchange for a steady increase in average income.

High Prevalence of Antibiotic Resistance in Iranian Isolates: Importance of Functional and Mutational Analysis of Resistance Genes and Virulence Genotyping.

The high prevalence of antibiotic resistance in has become a great challenge in Iran. The genetic mutations that contribute to the resistance have yet to be precisely identified. This study aimed to investigate the prevalence of antibiotic resistance and virulence markers in Iranian isolates and to analyze if there is any association between resistance and genotype.

'Only God can promise healing.': help-seeking intentions and lay beliefs about cures for post-traumatic stress disorder among Sub-Saharan African asylum seekers in Germany.

: Epidemiological studies have reported high rates of post-traumatic stress disorder (PTSD) among asylum seekers from Sub-Saharan Africa. In order to provide appropriate and culturally sensitive mental health care for this group, further knowledge about treatment preferences might be necessary. : We aimed to provide insights into help-seeking intentions and lay beliefs about cures for PTSD held by asylum seekers from Sub-Saharan Africa living in Germany.

Pharmacokinetic/Pharmacodynamic Relationship of Enzalutamide and Its Active Metabolite N-Desmethyl Enzalutamide in Metastatic Castration-Resistant Prostate Cancer Patients.

Introduction: Enzalutamide (ENZA) is an oral androgen receptor inhibitor approved by the Food and Drug Administration and the European Medicines Agency for the treatment of metastatic and nonmetastatic castration-resistant prostate cancer (CRPC). ENZA is extensively metabolized by cytochrome P450 3A4 into N-desmethyl ENZA (NDE), an active metabolite. We aimed to explore the pharmacokinetic/pharmacodynamic relationship for ENZA and NDE in metastatic CRPC patients from a real-world setting.

Physical and chemical stability of a generic etoposide formulation as an alternative to etoposide phosphate.

The generic Mylan® etoposide (ETP) has been investigated as an alternative for Etopophos®, in part due to a global shortage of the latter. The generic alternative is different both in its formulation and in its very limited stability (6 h at 25 °C against 4 days for Etopophos®) once reconstituted in ready-to-use chloride or glucose solutions. Its intrinsic stability has been thoroughly studied under various conditions.