Gynecological history to diagnosis and pregnancy outcomes in diagnosed Wilson's disease patients under therapy - A bicentric matched-control cohort study.

Introduction: most studies narrowly focus on pregnancy outcome comparisons between Wilson's disease (WD) patients on and off treatment. We aimed to identify menses irregularities in untreated WD, and to evaluate pregnancy outcomes in treated WD patients as compared to matched controls (with and without liver disease).

Methods: females with WD, hepatitis C (liver disease controls), and other gastrointestinal conditions (controls without liver disease) were identified at two tertiary hospital gastroenterology departments.

Retinal and choroidal thickness changes in systemic lupus erythematosus patients: a longitudinal study.

Background/objectives: To prospectively evaluate changes in peripapillary retinal nerve fibre layer (pRNFL), in all macular layers and in choroidal thickness (CT) in a cohort of systemic lupus erythematosus (SLE) patients without ophthalmologic manifestations. To associate those changes with ophthalmic characteristics, disease activity state, medication and systemic comorbidities.

Subjects/methods: Prospective cohort study of 68 previously diagnosed SLE patients.

Small Bowel Enteroscopy - A Joint Clinical Guideline from the Spanish and Portuguese Small Bowel Study Groups.

The present evidence-based guidelines are focused on the use of device-assisted enteroscopy in the management of small-bowel diseases. A panel of experts selected by the Spanish and Portuguese small bowel study groups reviewed the available evidence focusing on the main indications of this technique, its role in the management algorithm of each indication and on its diagnostic and therapeutic yields. A set of recommendations were issued accordingly.

Multimodality imaging in connective tissue disease-related interstitial lung disease.

Interstitial lung disease is a well-recognised manifestation and a major cause of morbidity and mortality in patients with connective tissue diseases. Interstitial lung disease may arise in the context of an established connective tissue disease or be the initial manifestation of an otherwise occult autoimmune disorder. Early detection and characterisation are paramount for adequate patient management and require a multidisciplinary approach, in which imaging plays a vital role.

Ciclopirox Hydroxypropyl Chitosan (HPCH) Nail Lacquer: A Review of Its Use in Onychomycosis.

Ciclopirox 8% hydroxypropyl chitosan (HPCH) [Marketed in different countries as the following registered (®) brands: Ciclopoli, Fulcare, Kitonail, Myconail, Niogermos, Niogermox, Onytec, Ony-Tec, Polinail, Privex, Rejuvenail] is the first topical nail lacquer developed using innovative drug formulation technology. It is indicated for the treatment of mild-to-moderate fungal infections of the nails that are caused by dermatophytes and/or other ciclopirox-sensitive fungi, without nail matrix involvement. HPCH is a patented drug formulation technology for the delivery of active principles into the nails based on a hydrosoluble semisynthetic amino-polysaccharide biopolymer derivative of chitosan.

Neurodegeneration in systemic lupus erythematosus: layer by layer retinal study using optical coherence tomography.

Background: Systemic lupus erythematosus (SLE) is a chronic, autoimmune and multisystemic disease. Recent studies with functional and structural magnetic resonance imaging and cognitive tests report an unexpectedly high frequency of central nervous system involvement, even in patients with asymptomatic SLE. The purpose of this study was to identify early signs of retinal neurodegeneration by comparing the thickness of the peripapillary retinal nerve fiber layer (pRNFL) and all macular layers between patients with SLE without ophthalmologic manifestations and healthy controls.

Small bowel enteroscopy - A joint clinical guideline by the Spanish and Portuguese small-bowel study groups.

The present evidence-based guidelines are focused on the use of device-assisted enteroscopy in the management of small-bowel diseases. A panel of experts selected by the Spanish and Portuguese small-bowel study groups reviewed the available evidence focusing on the main indications of this technique, its role in the management algorithm of each indication, and its diagnostic and therapeutic yield. A set of recommendations was issued accordingly.

Ocular involvement in systemic lupus erythematosus patients: a paradigm shift based on the experience of a tertiary referral center.

Objective: To evaluate ocular involvement in a cohort of systemic lupus erythematosus (SLE) patients of a tertiary referral center and to compare the results with the existing literature.

Methods: Patients underwent a complete ophthalmological evaluation, including visual acuity, slit-lamp examination, fluorescein staining, Schirmer-I test, Goldmann applanation tonometry, fundoscopy, 10-2 automated threshold visual fields, fundus autofluorescence and spectral-domain optical coherence tomography to screen for hydroxychloroquine (HCQ) macular toxicity.

Results: A total of 161 patients (16 men and 145 women) were enrolled in this study.

When systemic lupus erythematosus affects vision: a rare presentation of this condition.

A 23-year-old woman with fever, oral ulcers, arthalgias and weight loss of 2-week duration suddenly developed blurred vision, with reduced visual acuity, cotton wool exudates and retinal vascular tortuosity. Laboratory testing revealed anaemia, lymphopaenia, positive antinuclear antibody and high anti-dsDNA antibody titre with low complement components. There was no evidence of infection, clinching the diagnosis of lupus retinopathy.

In the Beginning ... It was Already the End.

Sometimes, the presentation of some diseases can be fulminating. The authors present the case of a 51-year- old male brought to the emergency department visibly drunk and complaining of abdominal pain. Immediately, the diagnosis of hemorrhagic shock due to an accentuated drop of the hemoglobin level was made.

Choroidal thickness changes in systemic lupus erythematosus patients.

Purpose: To compare choroidal thickness (CT) between patients with systemic lupus erythematosus (SLE) without ophthalmologic manifestations and a control group. To study the effects in CT of disease duration, activity index, medication and systemic comorbidities.

Methods: Cross-sectional study where spectral-domain optical coherence tomography with enhanced depth imaging was used to measure CT in 13 locations, subfoveally and at 500-µm intervals along a horizontal and a vertical section from the fovea.

Whole-Scalp Cobblestone Plica Neuropathica with Alopecia.

Plica neuropathica (PN) is an uncommon scalp condition that usually presents as a compact mass of scalp hair. We report a case of a 55-year-old woman presenting with PN. She presented with a 40-year history of thick plaques of yellow scale afflicting the entire scalp, progressive alopecia, and seeming lack of care of her own condition.

Dakin's solution in the treatment of severe diabetic foot infections.

Acute necrotising diabetic foot (DF) infections are common, costly, and do not infrequently result in debilitating major lower-extremity amputations. Dakin's solution is a long-standing topical antiseptic that has shown benefit in this clinical setting, but its use is undermined by a presumed risk of cytotoxicity. In this single-centre case series, we retrospectively evaluated 24 patients with severe necrotising DF infections treated with a cyclical instillation of Dakin's solution at a referral multidisciplinary DF unit.

Predicting incomplete basal cell carcinoma excisions - a large multidisciplinary retrospective analysis in a tertiary center.

Surgical treatment of basal cell carcinomas is often performed by physicians with different surgical backgrounds. Collecting data from different surgical departments would better reflect their real-life surgical management. To identify the rate, recurrence risk, and predictive factors accordingly to their relative contribution for an incomplete basal cell carcinoma excision in a large multidisciplinary real-life setting Retrospective cohort study of 2305 surgically treated lesions in different departments of a tertiary center.

Three-dimensional speckle-tracking echocardiography for the global and regional assessments of left ventricle myocardial deformation in breast cancer patients treated with anthracyclines.

Background: Assessment of 2D/3D left ventricular ejection fraction (LVEF) and 2D global longitudinal strain (GLS) is the gold standard for diagnosing cancer therapeutics-related cardiac dysfunction (CTRCD). Although 3D speckle-tracking echocardiography (STE) has several advantages, it is not used in this setting.

Methods: 105 breast cancer patients who underwent serial echocardiographic assessment during anthracycline therapy were included.

Myxoid Onychomatricoma-A New Case.

Onychomatricoma is a slow-growing, benign neoplasm of the nail matrix with several histological variants. With only 2 cases previously reported, the term "myxoid onychomatricoma" has been used by some authors to describe a rare variant, known to have a fibromixoid component. We present a case of a 51-year-old Caucasian woman with a 1-year history of thickening and discoloration of the right thumbnail, who was initially misdiagnosed as onychomycosis.

Recommendations from a Portuguese Expert Group for Discontinuation of Tyrosine Kinase Inhibitors in Chronic Myeloid Leukemia Patients in Clinical Practice.

Until recently, the main goal of chronic myeloid leukemia therapy was disease control with the best overall survival, which required lifelong treatment. However, currently, the treatment-free remission concept is becoming an important goal in clinical practice, and several tyrosine kinase inhibitors discontinuation studies have shown that round 50% of patients with a durable deep molecular response beyond major molecular response successfully interrupt tyrosine kinase inhibitors for at least three years without loss of molecular response. However, and regardless of the existing evidence, the exact conditions for attempting treatment-free remission remain poorly defined.

A Narrative Review on Anti-Tumor Necrosis Factor α Therapies in Inflammatory Bowel Disease During Pregnancy: Immunoglobulin Placental Translocation and its Impact.

Introduction: Inflammatory bowel disease activity is associated with adverse pregnancy outcomes. Anti-tumor necrosis factor α therapy is often required to treat flares and to maintain disease remission. However, there are concerns regarding treatment with these agents during pregnancy, as they actively cross the placental barrier.

Primary vaginal malignancies: a single oncology centre experience.

Primary vaginal malignancies constitute a rare entity. The aim of this study was to review all primary vaginal malignancies diagnosed in an oncologic referral centre over 11 years. A total of 35 cases were retrospectively analysed, including clinical and MRI features.