Co-Inheritance of Autosomal Dominant Polycystic Kidney Disease and Naevoid Basal Cell Carcinoma Syndrome: Effects on Renal Progression.

The calcium signalling and hedgehog (HH) signalling pathways operate in the primary cilium. Abnormalities in these pathways cause autosomal dominant polycystic kidney disease (ADPKD) and naevoid basal cell carcinoma syndrome (NBCCS) respectively. Several reports have proposed that hyperactivation of the HH pathway in animal models of polycystic kidney disease affects normal renal development and renal cyst phenotype.

Diagnostic value of procalcitonin in ventilator-associated pneumonia.

Background And Objective: Procalcitonin (PCT) can help the early diagnosis of bacterial infections and estimate the response obtained. The objective is to study the value of PCT for the diagnosis of ventilator-associated pneumonia (VAP).

Patients And Method: Prospective and observational study, carried out for 18 months, in a polyvalent Intensive Care Unit (ICU).

[APACHE II and SAPS II as predictors of brain death development in neurocritical care patients].

Aim: To assess the prognostic value of APACHE II and SAPS II scales to predict brain death evolution of neurocritical care patients.

Patients And Methods: Retrospective observational study performed in a tertiary hospital. Include 508 patients over 16 years old, hospitalized in ICU for at least 24 hours.

WhatsApp: A Real-Time Tool to Reduce the Knowledge Gap and Share the Best Clinical Practices in Psoriasis.

Background: Psoriasis is a chronic inflammatory disease that affects around 100 million people worldwide. The burden of disease is high, but more recent therapies show promising results. Clinicians need, however, more training in the use of such therapies.

[Social cognition in Parkinson's disease dementia and behavioral variant of frontotemporal dementia].

Aim: To study the presence of alterations in social cognition in Parkinson's disease dementia (PDD) and behavioral variant frontotemporal dementia (bvFTD), to compare the levels of involvement, and to analyze their relevance as a tool to distinguish between both demential profile.

Subjects And Methods: For this purpose, 34 patients diagnosed with PDD, 21 men and 13 women, with an average age of 70 years and 8 years of education, and 26 patients diagnosed with bvFTD, 9 men and 17 women, with an average age of 68 years and 6 years of education, were assessed. The control group consisted of 30 healthy subjects, with a mean of 66 years of age and 8 of schooling.

The "Learning Through Play" project. Importance of interdisciplinary work among children born prematurely and their family groups in an area of high socio-environmental risk.

Several socio-environmental risk factors causea large number of children born prematurely, and without neurological disorders, to have low scores in their development, particularly in the cognitive and socio-emotional skills areas, thus evidencing a potential future risk. Strengthening bonds with the family and caregivers is critical for their adequate development. This article reports on the experience of an interdisciplinary team from Hospital Eva Perón de Tucumán, who designed a program called "Learning Through Play"for the promotion of child development and aimed at parents of preterm infants seen at the Follow-up Outpatient Clinic and the Department of Social Services, with the collaboration of the Department of Nutrition.

Transancestral mapping and genetic load in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an autoimmune disease with marked gender and ethnic disparities. We report a large transancestral association study of SLE using Immunochip genotype data from 27,574 individuals of European (EA), African (AA) and Hispanic Amerindian (HA) ancestry. We identify 58 distinct non-HLA regions in EA, 9 in AA and 16 in HA (∼50% of these regions have multiple independent associations); these include 24 novel SLE regions (P<5 × 10), refined association signals in established regions, extended associations to additional ancestries, and a disentangled complex HLA multigenic effect.

Real world prescription trends of methotrexate for psoriasis in Argentina: results of a national survey.

Objective: To describe dermatologists' methotrexate (MTX) prescription trends for the treatment of psoriasis, and to identify variables associated with suboptimal MTX use.

Methods: Cross-sectional study of dermatologists from Argentina who completed a pre-designed survey focussed on MTX prescription characteristics. A multiple logistic regression model was used to identify variables independently associated with suboptimal MTX use (when less than 12 weeks and less than 15 mg/week were administrated in patients with monotherapy before discontinuing or adding a second drug).

[Scorpionism in children in Tucumán: descriptive analysis of cases in a second level of complexity Hospital].

Scorpionism in Tucumán is very frequent. The aim is to describe the clinical and epidemiological characteristics of cases of scorpionism that were seen in the Emergency Department of Eva Perón Hospital, Banda del Río Salí, Tucumán, between July 2013 and March 2015. Forty patients were assisted, 21 boys and 19 girls.

Body Region Involvement and Quality of Life in Psoriasis: Analysis of a Randomized Controlled Trial of Adalimumab.

Background: Psoriasis severity and treatment responsiveness vary by body region, which differentially impacts quality of life (QoL).

Objective: The objective of the study was to examine adalimumab efficacy by body region and regional response and QoL relationship.

Methods: Patients (n = 1212) with moderate-to-severe psoriasis were randomized 2:1 to 80 mg at week 0, followed by adalimumab 40 mg or placebo every other week for 16 weeks in the double-blind REVEAL study.

No PERV transmission during a clinical trial of pig islet cell transplantation.

Xenotransplantation of pig islet cells is a promising alternative for the treatment of diabetes with insulin and may help to prevent numerous late complications such as blindness and amputation. First encouraging results using porcine islets have been reported in preclinical animal models as well in the first clinical trial in New Zealand. The goal of this manuscript is to examine the biological safety of a second trial performed in Argentina, specifically in regards to the transmission of porcine endogenous retroviruses (PERVs) using improved detection methods As in the first trial encapsulated islet cells from the well-characterised Auckland Island pigs were used.

Clinical Benefit of Islet Xenotransplantation for the Treatment of Type 1 Diabetes.

Background: Allogeneic islet transplantation has become a viable option for the treatment of unstable type 1 diabetes. However, the donor shortage and the necessity of the immunosuppressive drugs are two major issues. To solve these issues, we performed islet xenotransplantation using encapsulated neonatal porcine islets without immunosuppressive drugs.

Progress in Clinical Encapsulated Islet Xenotransplantation.

At the 2015 combined congress of the Cell Transplant Society, International Pancreas and Islet Transplant Association, and International Xenotransplantation Association, a symposium was held to discuss recent progress in pig islet xenotransplantation. The presentations focused on 5 major topics - (1) the results of 2 recent clinical trials of encapsulated pig islet transplantation, (2) the inflammatory response to encapsulated pig islets, (3) methods to improve the secretion of insulin by pig islets, (4) genetic modifications to the islet-source pigs aimed to protect the islets from the primate immune and/or inflammatory responses, and (5) regulatory aspects of clinical pig islet xenotransplantation. Trials of microencapsulated porcine islet transplantation to treat unstable type 1 diabetic patients have been associated with encouraging preliminary results.

Chronic Hypoxia Accentuates Dysanaptic Lung Growth.

Rationale: Adults born and raised at high altitudes have larger lung volumes and greater pulmonary diffusion capacity compared with adults at low altitude; however, it remains unclear whether the air and tissue volumes have comparable increases and whether there is a difference in airway size.

Objectives: To assess the effect of chronic hypoxia on lung growth using in vivo high-resolution computed tomography measurements.

Methods: Healthy adults born and raised at moderate altitude (2,000 m above sea level; n = 19) and at low altitude (400 m above sea level; n = 23) underwent high-resolution computed tomography.

Identification of germ cell-specific VASA and IFITM3 proteins in human ovarian endometriosis.

Background: Endometriosis is a gynaecological disorder that affects 6-10 % of female population. It is characterized by the presence of endometrial tissue outside the uterus, most often in the pelvic peritoneum or ovaries. Recent studies have indicated that mesenchymal endometrial stem cells might get involved in endometriosis progression.

[Nevoid basal cell carcinoma syndrome with corpus callosum agenesis, PTCH1 mutation and absence of basal cell carcinoma].

Nevoid Basal Cell Carcinoma Syndrome (NBCCS) or Gorlin-Goltz syndrome is a rare autosomal dominant disorder, mainly due to PTCH1 gene mutations, that comprises a broad spectrum of clinical manifestations. The presence of multiple basal cell carcinomas (BCCs) is a cardinal sign in NBCCS, therefore cases in which BCCs are absent entails a delay in the diagnosis.We present a 14 years old boy with a clinical diagnosis of NBCCS by the presence of odontogenic cysts, hypertelorism, macrocephaly, and corpus callosum agenesia, but with absence of skin lesions.

Clinical characteristics and outcome of 107 adult patients with chronic Chagas disease and parasitological cure criteria.

Background: The cure in adult patients with chronic Chagas disease and the relationship between parasitological and clinical evolution is still under debate. The aim of this study was to analyze the clinical, epidemiological and progression features of the disease in a patient population who became serologically negative either spontaneously or post-etiological treatment.

Methods: We included 107 patients over 20 years old with three different confirmed reactive anti-Trypanosoma cruzi serologic tests on admission, and a minimum of two years of follow-up.

Dried human amniotic membrane as an antiadherent layer for intraperitoneal placing of polypropylene mesh in rats.

Background: Intraabdominal peritoneal onlay polypropylene (PP) mesh repair of incisional hernia has the potential risk of adhesions, bowel obstructions, and intestinal fistulae. Fresh or cryopreserved human amniotic membrane (HAM) has been tested as an antiadherent layer in animals, with excellent outcomes. However, it has disadvantages: it is difficult to handle, and it is expensive to store.

Seronegative conversion after incomplete benznidazole treatment in chronic Chagas disease.

In 12-18% of adult patients, treatment with benznidazole for chronic Chagas disease has to be discontinued because of side-effects. We identified and analysed a cohort of 81 adult patients with three positive tests for Trypanosoma cruzi infection and serological monitoring following incomplete treatment with benznidazole for a median of 10 days. Twenty percent of these patients (16/81) met the criteria of cure, showing that the optimal schedule of benznidazole administration remains to be determined.

Impact of aetiological treatment on conventional and multiplex serology in chronic Chagas disease.

Background: The main criterion for treatment effectiveness in Chagas Disease has been the seronegative conversion, achieved many years post-treatment. One of the main limitations in evaluating treatment for chronic Chagas disease is the lack of reliable tests to ensure parasite clearance and to examine the effects of treatment. However, declines in conventional serological titers and a new multiplex assay can be useful tools to monitor early the treatment impact.

Pig islet xenotransplantation acceptance in a Latin-American diabetic population.

Progress in porcine islet xenotransplantation has been accompanied by studies on acceptance of this new procedure by patients, health professionals or the general public. Such studies have not been done in the Latin-American population. We conducted a questionnaire in 108 diabetes patients (insulin-dependent, n = 53; insulin-independent, n = 55) in a public hospital in Argentina.