Case Report: C3 deficiency in two siblings.

The complement system, a vital component of innate immunity, consists of various proteins and pathways crucial for the recognition and elimination of pathogens. In addition, it plays a major role in the initiation of adaptive response through the opsonization of antigens, contributing to B-cell activation and memory maintenance. Deficiencies in complement proteins, particularly C3, can lead to severe and recurrent infections as well as immune complex disorders.

Multiple, random spot urine sampling for estimating urinary sodium excretion.

The measurement of sodium intake may be important for the management of hypertension. Dietary surveys and 24-h urinary collection are often unreliable and/or impractical. We hypothesized that urinary sodium excretion can be accurately estimated through multiple spot urine samples from different days.

Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina.

Introduction: Introduction: Thrombotic microangiopathies (TMAs) are rare disorders associated with fatal outcomes if left uncared for. However, healthcare problems in developing countries tend to limit medical assistance to patients.

Methods: Methods: We prospectively studied an Argentine cohort of 294 consecutive patients from 2013 to 2016.

In , the Poly(ADP-Ribose) Polymerase-Like Thermoprotein Is a Multifunctional Enzyme.

In , Sso, the ADP-ribosylating thermozyme is known to carry both auto- and heteromodification of target proteins via short chains of ADP-ribose. Here, we provide evidence that this thermoprotein is a multifunctional enzyme, also showing ATPase activity. Electrophoretic and kinetic analyses were performed using NAD and ATP as substrates.

Taurine Enhances Stretch Reflex Excitability.

The purpose of this study was to characterize the effects of taurine (supplementation and acute injection) on the stretch reflex in the ankle muscles, and in particular to compare the effects of chronic taurine supplementation versus acute injection on the muscle tension, amplitude of electromyogram and velocity of muscle response. Stretch reflex responses were evoked using a specialized stretching device designed for mice. The triceps surae muscle of an awake mouse was stretched at various speeds ranging from 500 to 500,000° per second.

Understanding and treating solid tumor-related disseminated intravascular coagulation in the "era" of targeted cancer therapies.

A systemic activation of blood coagulation is usually present in many clinical conditions including the infectious or inflammatory ones and malignant disease as well. Depending upon circumstances, patients suffering from acute decompensated disseminated intravascular coagulation may be managed by a medical oncologist and either an internist or a physician working in an emergency and/or intensive care unit. In some cases, for example, the indolent ones, the activation of coagulation might not be easily detected by routine laboratory tests and not lead to clinical manifestations.

Effects of Taurine Supplementation on Neuronal Excitability and Glucose Homeostasis.

In this study we examined the role of chronic taurine supplementation on plasma glucose homeostasis and brain excitability through activation of the insulin receptor. FVB/NJ male mice were supplemented with taurine in drinking water (0.05% w/v) for 4 weeks and subjected to a glucose tolerance test (7.

Dynamics of mTORC1 activation in response to amino acids.

Amino acids are essential activators of mTORC1 via a complex containing RAG GTPases, RAGULATOR and the vacuolar ATPase. Sensing of amino acids causes translocation of mTORC1 to lysosomes, an obligate step for activation. To examine the spatial and temporal dynamics of this translocation, we used live imaging of the mTORC1 component RAPTOR and a cell permeant fluorescent analogue of di-leucine methyl ester.

Prosaposin is a regulator of progranulin levels and oligomerization.

Progranulin (GRN) loss-of-function mutations leading to progranulin protein (PGRN) haploinsufficiency are prevalent genetic causes of frontotemporal dementia. Reports also indicated PGRN-mediated neuroprotection in models of Alzheimer's and Parkinson's disease; thus, increasing PGRN levels is a promising therapeutic for multiple disorders. To uncover novel PGRN regulators, we linked whole-genome sequence data from 920 individuals with plasma PGRN levels and identified the prosaposin (PSAP) locus as a new locus significantly associated with plasma PGRN levels.

Optimal duration of low molecular weight heparin for the treatment of cancer-related deep vein thrombosis: the Cancer-DACUS Study.

Purpose: We evaluated the role of residual vein thrombosis (RVT) to assess the optimal duration of anticoagulants in patients with cancer who have deep vein thrombosis (DVT) of the lower limbs.

Patients And Methods: Patients with active cancer and a first episode of DVT treated with low molecular weight heparin (LMWH) for 6 months were eligible. Patients were managed according to RVT findings: those with RVT were randomly assigned to continue LMWH for an additional 6 months (group A1) or to discontinue it (group A2), and patients without RVT stopped LMWH (group B).

The immune system GTPase GIMAP6 interacts with the Atg8 homologue GABARAPL2 and is recruited to autophagosomes.

The GIMAPs (GTPases of the immunity-associated proteins) are a family of small GTPases expressed prominently in the immune systems of mammals and other vertebrates. In mammals, studies of mutant or genetically-modified rodents have indicated important roles for the GIMAP GTPases in the development and survival of lymphocytes. No clear picture has yet emerged, however, of the molecular mechanisms by which they perform their function(s).

Vault-poly-ADP-ribose polymerase in the Octopus vulgaris brain: a regulatory factor of actin polymerization dynamic.

Our previous behavioural, biochemical and immunohistochemical analyses conducted in selected regions (supra/sub oesophageal masses) of the Octopus vulgaris brain detected a cytoplasmic poly-ADP-ribose polymerase (more than 90% of total enzyme activity). The protein was identified as the vault-free form of vault-poly-ADP-ribose polymerase. The present research extends and integrates the biochemical characterization of poly-ADP-ribosylation system, namely, reaction product, i.

How phosphoinositide 3-phosphate controls growth downstream of amino acids and autophagy downstream of amino acid withdrawal.

The simple phosphoinositide PtdIns3P has been shown to control cell growth downstream of amino acid signalling and autophagy downstream of amino acid withdrawal. These opposing effects depend in part on the existence of distinct complexes of Vps34 (vacuolar protein sorting 34), the kinase responsible for the majority of PtdIns3P synthesis in cells: one complex is activated after amino acid withdrawal to induce autophagy and another regulates mTORC1 (mammalian target of rapamycin complex 1) activation when amino acids are present. However, lipid-dependent signalling almost always exhibits a spatial dimension, related to the site of formation of the lipid signal.

[Endothelin-1 and receptor A: predictive value for biochemical relapse on patients with advanced and metastatic prostate cancer].

Introduction: Pathological endothelin axis is known to be involved in prostate cancer progression. Our study evaluates immunohistochemical expression of ET-1 and ET-AR on prostate biopsy specimen and the predictive value for biochemical relapse on patients with advanced and metastatic cancer. We also evaluated the impact of ET-1 and ET-AR expression on local progression and metastatic bone progression for these patients.

Low molecular weight heparin administration in cancer patients with hypercoagulability-related complications and carrying brain metastases: a case series study.

Background: Venous thromboembolism (VTE) and brain metastases (MTS) are significant clinical problems in the cancer patient population. Brain MTS and deep vein thrombosis are life-threatening conditions because of the risk of fatal endocranic hypertension and pulmonary embolism. Low molecular weight heparin (LMWH) is a major treatment for cancer patients suffering from VTE with regard to the management of the acute phase and subsequent secondary prophylaxis.

Prostate cancer detection after one or more negative extended needle biopsy: results of a multicenter case-findings protocol.

Objectives: To evaluate PCa incidence in patients with one or more negative extended prostate biopsy who underwent repeat biopsy or TURP.

Material And Methods: From June 2003 to February 2008, 308 patients were submitted to repeat prostate biopsy (median 20.5 cores) and 120 patients underwent TURP after one or more 12 cores prostate biopsy.

Successful administration of a low dose of calcium nadroparin in patients suffering from pulmonary embolism and brain metastases: a report of two cases.

Objective: To focus on the optimal management of thromboembolic complication in patients who have undergone chemotherapy with concomitant brain metastases and referred to a Division of Clinical Oncology.

Background: Thromboembolic diseases are common events in cancer patients due to clotting activation by tumor cells. On the other hand, brain metastases are common complication of systemic cancers.

The prognostic significance of bladder neck invasion in prostate cancer: is microscopic involvement truly a T4 disease?

Since the widespread of prostate-specific antigen-based screening, prostate cancer at clinical stage T4 has become rare. Most bladder invasion is actually detected on radical prostatectomy specimens as a microscopic bladder neck involvement (BNI). The 2002 Tumour-Node-Metastasis (TNM) classification system classified prostate cancer with BNI within a unified pT4 category and rendered it equivalent to invasion into the pelvic wall musculature or external sphincter; this decision is controversial.

Bladder neck involvement as pT4 disease in prostate cancer: implications for prognosis and patient surveillance.

Since the use of prostate-specific antigen screening has become widespread, prostate cancer at clinical T4 stage has become rare. Most bladder invasion is actually detected on radical prostatectomy specimens as a microscopic bladder neck involvement (BNI). The 2002 TNM classification system classified prostate cancer with BNI within a unified pT4 category, and rendered it equivalent to invasion into pelvic wall musculature or external sphincter.

Hepatic intra-arterial interferon alpha 2b-based immunotherapy combined with 5-fluorouracil (5-FU)-based systemic chemotherapy for patients with hepatocellular carcinoma (HCC) not responsive and/or not eligible for conventional treatments: a pilot study.

Background: Surgery (partial hepatic resection or orthotopic liver transplantation) remains the mainstay for treatment of hepatocellular carcinoma (HCC). Unfortunately, most patients have HCC that cannot be removed either as a result of its size, multiple tumors, location, proximity to major vessels or ducts within the liver, and comorbidity, such as a not well-compensated cirrhosis. For patients who cannot be treated surgically, systemic chemotherapy is frequently limited by unacceptable toxicity, poor response and low survival rates, so that locoregional approaches may be considered as alternatives.

Inhibition by soya isoflavones of human polymorphonuclear leukocyte function: possible relevance for the beneficial effects of soya intake.

Lower CVD incidence is reported in Asian populations consuming soya-containing food. As polymorphonuclear leukocytes (PMN) are involved in the risk of CVD, we investigated the modulatory effect of soya isoflavones on several PMN functions and their molecular mechanisms in vitro. PMN, isolated from blood from healthy subjects, were tested upon activation with 1 microm- n-formyl-methyl-leucyl-phenylalanine (fMLP) for superoxide anion production (ferric cytochrome c reduction) and released elastase (chromogenic test).

The lipoxygenase-cyclooxygenase inhibitor licofelone prevents thromboxane A2-mediated cardiovascular derangement triggered by the inflammatory peptide fMLP in the rabbit.

Licofelone is an analogue of arachidonic acid that inhibits 5-lipoxygenase (LOX), cyclooxygenase (COX)-1 and COX-2. We investigated the effects of licofelone on cardiovascular derangements and production of thromboxane (Tx)A(2) induced by the inflammatory agonist n-formyl-methionyl-leucyl-phenylalanine (fMLP) in the rabbit, in comparison with those of aspirin or rofecoxib, inhibitors of COX-1 and COX-2, respectively. In control rabbits, injection of fMLP (30 nmol/kg) in the jugular vein evokes ischemic electrocardiographic (ECG) changes in the first 1-5 min, i.

Incidence of prostate cancer in Sicily: results of a multicenter case-findings protocol.

Objective: To establish the incidence of prostate cancer (PCa) in Sicily in patients who entered an early detection protocol.

Methods: From February 2002 to February 2004, 16,298 subjects aged 40-75 entered the protocol. Patients with suspicious DRE, PSA>10 ng/ml, PSA View Article and Find Full Text PDF

Effects of a shared protocol between urologists and general practitioners on referral patterns and initial diagnostic management of men with lower urinary tract symptoms in Italy: the Prostate Destination study.

Objective: To assess whether adopting a shared protocol between urologists and general practitioners (GPs) might change diagnostic procedures and referral patterns in the management of men with lower urinary tract symptoms (LUTS).

Subjects And Methods: Forty-five urological centres and 263 GPs in Italy participated in this prospective study. Procedures adopted by GPs for evaluating five consecutive patients (aged > or = 50 years) were compared before (phase 1) and after (phase 2) implementation of the shared protocol.

Prevention of cardiovascular disease in type-2 diabetes: how to improve the clinical efficacy of aspirin.

Atherosclerotic cardiovascular disease and its thrombotic complications are the principal causes of morbidity and mortality in patients with type-2 diabetes. Aspirin reduces the risk of thrombotic events in a broad range of patients with vascular disease and, in selected individuals, is beneficial for primary prevention. Although recommended by existing guidelines, in secondary and in primary prevention trials the clinical efficacy of low-dose aspirin in patients with diabetes appears to be substantially lower than in individuals without diabetes.