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This edition concentrates on inflammatory myopathies with reports of reclassification of polymyositis, cancer associations, evaluation of subclinical cardiac involvement, myositis-specific and -associated antibodies, and immune checkpoint inhibitor myositis. A number of reports address sporadic late-onset nemaline myopathy and point out its diagnostic difficulty and the importance of identifying an associated monoclonal gammopathy that is likely of clinical significance and may warrant aggressive immunotherapy. Finally, treatment of nondystrophic channelopathies is addressed.

This edition concentrates on inflammatory myopathies with reports of reclassification of polymyositis, cancer associations, evaluation of subclinical cardiac involvement, myositis-specific and -associated antibodies, and immune checkpoint inhibitor myositis. A number of reports address sporadic late-onset nemaline myopathy and point out its diagnostic difficulty and the importance of identifying an associated monoclonal gammopathy that is likely of clinical significance and may warrant aggressive immunotherapy. Finally, treatment of nondystrophic channelopathies is addressed.

Treatment approach of myasthenia gravis (MG) is still debated; corticosteroids alone or in combination with immunosuppressive agents are the most used drugs. Azathioprine (AZA) has been shown to be effective for MG with a significant steroid-sparing activity, although burdened by side effects. Few studies on methotrexate (MTX) administration showed controversial results. In this cohort, we evaluated the role of MTX as a effective steroid-sparing agent.

Fifteen MG patients treated with MTX, previously treated with AZA for at least 12 months, with poor benefits and uncomfortable side effects AZA related, have been selected. Each patient was evaluated through MG-Activity of Daily Living and Quantitative MG scores 5 times/yr.

Patients treated with MTX had a significant improvement of MG-Activity of Daily Living and Quantitative MG scores. Furthermore, all patients reduced prednisone dosage, and none complained of side effects.

We suggest MTX is effective and well tolerated and could be considered as a steroid-sparing agent in MG treatment.

We suggest MTX is effective and well tolerated and could be considered as a steroid-sparing agent in MG treatment.

Antiretroviral therapies for HIV may cause systemic toxicities when coadministered with corticosteroids. Potential drug-drug interactions may occur, leading to iatrogenic Cushing syndrome or adrenal insufficiency. This article highlights the drug-drug interactions of antiretroviral therapies with corticosteroids. Practice implications are discussed.

Antiretroviral therapies for HIV may cause systemic toxicities when coadministered with corticosteroids. Potential drug-drug interactions may occur, leading to iatrogenic Cushing syndrome or adrenal insufficiency. This article highlights the drug-drug interactions of antiretroviral therapies with corticosteroids. Practice implications are discussed.

Contrast-induced nephropathy is the leading cause of acute kidney injury in hospital settings. Practitioners must have a comprehensive understanding of preventive interventions. This article provides a mnemonic NPs can use to prevent contrast-induced nephropathy. The mnemonic, CHIPS, addresses Contrast minimizations, Hemofiltration, Iso-osmolar/low osmolar contrast, Pharmacologic interventions, and Saline I.V. hydration.

Contrast-induced nephropathy is the leading cause of acute kidney injury in hospital settings. Practitioners must have a comprehensive understanding of preventive interventions. This article provides a mnemonic NPs can use to prevent contrast-induced nephropathy. The mnemonic, CHIPS, addresses Contrast minimizations, Hemofiltration, Iso-osmolar/low osmolar contrast, Pharmacologic interventions, and Saline I.V. hydration.

Crohn disease is an inflammatory bowel disorder affecting children and adults. With its increasing prevalence, healthcare providers need adequate resources to assist with diagnosis and management. This article discusses early diagnosis, disease severity and classification, familial predisposition and genomics, and clinical management in the primary care setting.

Crohn disease is an inflammatory bowel disorder affecting children and adults. With its increasing prevalence, healthcare providers need adequate resources to assist with diagnosis and management. This article discusses early diagnosis, disease severity and classification, familial predisposition and genomics, and clinical management in the primary care setting.

Knowledge of which pulmonary function tests are commonly performed in primary care and interpretation of their results is integral for the diagnosis, care, and management of those with pulmonary symptoms. Cyclopamine This article provides an overview of the most common pulmonary function tests and interpretation of their results.

Knowledge of which pulmonary function tests are commonly performed in primary care and interpretation of their results is integral for the diagnosis, care, and management of those with pulmonary symptoms. This article provides an overview of the most common pulmonary function tests and interpretation of their results.

To investigate the correlation between optical coherence tomography (OCT) findings and visual acuity outcomes after treatment with intravitreal bevacizumab (IVB) injections for age related macular degeneration (AMD) patients with peripapillary choroidal neovascularization (PPCNV).

A retrospective case series of consecutive patients diagnosed with PPCNV secondary to AMD. All patients were treated with IVB injections with a follow-up time of one year. Data collected included best corrected visual acuity (BCVA), automated and manually measured OCT parameters.

A total of 68 eyes were diagnosed with PPMV. Of them, 30 eyes of 30 patients aged 84.3±6.9 years of which 63.3% female gender were included. Baseline BCVA was 0.46±0.62 logMAR (Snellen 20/57), average choroidal thickness was 193.2±22 µm and mean number of IVB injections was 7.2±1.9. After one year BCVA was 0.56±0.78 logMAR (Snellen 20/72) (p=0.28). Eyes with greater central retinal thickness (r=-0.36, p=0.05), greater subretinal hyperreflective material (SHRM) area (r=-0.37, p=0.05) and greater sub retinal fluid (SRF) area (r=-0.73, p<0.001) had a significantly smaller improvement in BCVA. Eyes with pigment epithelium detachment (PED) (0.68±0.90 versus 0.21±0.12, p=0.03) had a significantly worse BCVA.

Our data suggests that AMD related PPCNV with greater foveal thickness, PED size, SHRM and SRF areas have worse final BCVA prognosis.

Our data suggests that AMD related PPCNV with greater foveal thickness, PED size, SHRM and SRF areas have worse final BCVA prognosis.

We aimed to compare the outcomes of ACS (acute coronary syndrome) patients undergoing in-hospital PCI (percutaneous coronary intervention) treated with prasugrel versus ticagrelor.

Among 7,233 patients enrolled to the ACSIS (Acute Coronary Syndrome Israeli Survey) between 2010 and 2018, we identified 1126 eligible patients treated with prasugrel and 817 with ticagrelor. Comparison between the groups was preformed separately in ST-elevation myocardial infarction (STEMI) patients, propensity score matched (PSM) STEMI patients, and non-ST-elevation ACS (NSTE-ACS) patients.

In-hospital complication rates, including rates of stent thrombosis, were not significantly different between groups. In PSM STEMI patients, 30-day re-hospitalization rate (p <0.05), 30-day MACE (the composite of death, MI, stroke and urgent revascularization; p=0.006), and 1-year mortality rates (p = 0.08) were higher in the ticagrelor group compared to the prasugrel group; In NSTE-ACS patients, outcomes were not associated with drug choice. In cox regression analysis applied on the entire cohort, prasugrel was associated with lower 1-year mortality in STEMI patient but not in NSTE-ACS patients (p for interaction 0.03).

Compared to ticagrelor, prasugrel was associated with superior clinical outcomes in STEMI patients, but not in NSTE-ACS patients.

Compared to ticagrelor, prasugrel was associated with superior clinical outcomes in STEMI patients, but not in NSTE-ACS patients.

The aim of the study was to investigate the impact of intrapartum antibiotic treatment (IAT) on the compositional development of gut microbiota in healthy term infants.

A case-control study of 24 infants exposed to and 24 matched infants not exposed to IAT was conducted. All subjects were born by vaginal delivery at term and breastfed. None of the infants received antibiotics during the immediate neonatal period. Fecal samples were obtained at the ages of 1 and 6 months. The composition of the intestinal microbiota was assessed by 16S rRNA gene sequencing.

IAT was associated with reduced microbial richness but not diversity at 1 month of age. Furthermore, the relative abundances of Clostridiaceae and Erysipelotrichaceae were significantly altered in infants exposed to IAT as compared to nonexposed infants at 1 month of age. The observed deviations in gut microbiota composition between infants exposed and not exposed to IAT diminished by the age of 6 months.

IAT is associated with short-term perturbations in the gut microbiota development in healthy term, vaginally delivered, breastfed infants. The composition of the gut microbiota is mostly restored by the age of 6 months.

IAT is associated with short-term perturbations in the gut microbiota development in healthy term, vaginally delivered, breastfed infants. The composition of the gut microbiota is mostly restored by the age of 6 months.Fabry disease (FD) is an inherited X-linked lysosomal storage disorder, with hemizygous males being more severely affected than heterozygous females. Herein, we report a rare case of FD in a heterozygous female with a severe phenotype. The patient had obesity and hyperlipidemia and had her first cerebral infarction at the age of 33 years. She underwent renal biopsy and was diagnosed with FD with morphological features of focal segmental glomerulosclerosis nephropathy at the age of 34 years. Her leukocyte alpha-galactosidase A activity was 2.3 Agal/U (normal >20 Agal/U), and genetic analysis revealed the presence of the classical phenotype. Enzyme replacement therapy (ERT) was initiated at the age of 35 years; however, peritoneal dialysis owing to end-stage renal failure occurred at the age of 37 years. The patient died of a cerebral hemorrhage at the age of 44 years. Her Mainz Severity Score Index at the time of death was 48/76, suggestive of the severe phenotype. Autopsy findings revealed remarkable globotriaosylceramide accumulation on electron microscopy, particularly in the major organs and their vascular smooth muscle cells. Regarding the vertebral arteries which sourced the cerebral hemorrhage, the effects of FD-induced vascular thickening and long-term renal failure-induced atherosclerosis were confirmed. Furthermore, the patient's vascular sclerosis was modified with acquired factors such as lifestyle-related disease associated with obesity. We recommend intensified treatment for metabolic factors simultaneous with ERT to help in delaying the progression of FD.

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