Reproductive and pregnancy control in Wilson disease patients in Spain.

Background And Aim: Recommendations on pregnancy, lactation, and contraception in women with Wilson disease are briefly stated in international guidelines but are not entirely homogeneous. Data regarding the management of these special events among patients with Wilson disease in Spain are lacking. We used the Wilson Registry platform of the Spanish Association for the Study of the Liver to question patients on their reproductive and gestational lives.

Preoperative Echocardiogram Does Not Contribute to Surgical Risk Assessment in Patients With Large Curve Scoliosis and No Cardiac Risk Factors.

Objective: Severe scoliosis can affect thoracic organs, potentially leading to cardiovascular abnormalities. Thus, echocardiograms have been suggested for use in preoperative screening in patients with significant scoliosis. However, the utility of preoperative heart screenings in patients without known or suspected heart problems is not well understood.

Evaluating compliance with the best practice guidelines for wrong-level surgery prevention in high-risk pediatric spine surgery.

Purpose: In 2018, Best Practice Guidelines (BPGs) were published for preventing wrong-level surgery in pediatric spinal deformity, but successful implementation has not been established. The purpose of this study was to evaluate BPG compliance 5 years after publication. We hypothesized higher compliance among BPG authors and among surgeons with more experience, higher caseload, and awareness of the BPGs.

Thoracic pediculectomy for acute spinal cord decompression in high-risk spinal deformity correction: illustrative case.

Background: Neurological complications are higher in patients with severe spinal deformities (Cobb angle >100°). The authors highlight a known technique for thoracic concave apical pedicle resection that is useful for spinal cord decompression in patients with high-risk spinal deformities in the setting of intraoperative neuromonitoring (IONM) changes.

Observations: A 14-year-old female with progressive idiopathic scoliosis presented for evaluation of her clinical deformity.

Evaluating compliance with the 2013 best practice guidelines for preventing surgical site infection in high-risk pediatric spine surgery.

Purpose: Best Practice Guidelines (BPGs) were published one decade ago to decrease surgical site infection (SSI) in pediatric spinal deformity. Successful implementation has not been established. This study evaluated surgeon compliance with items on the BPG.

State of affairs and future challenges in laboratory medicine in Spain: an analysis of the Spanish Society of Laboratory Medicine (SEQC).

Objectives: Laboratory Medicine is a crucial discipline that contributes to the diagnosis, management and monitoring of patients. This branch of medicine faces two major challenges: New technologies and increased demand. There is limited information available of the state of affairs in Laboratory Medicine in Spain.

"Cut and Paste" Processes in the Search of Bioactive Products: One-Pot, Metal-free -Radical Scission-Oxidation-Addition of , or -Nucleophiles.

Hypervalent iodine reagents have been applied in many metal-free, efficient synthesis of natural products and other bioactive compounds. In particular, treatment of alcohols, acetals and acids with hypervalent iodine reagents and iodine results in -radicals that can undergo a β-scission reaction. Under these oxidative conditions, derivatives of amino acids, peptides or carbohydrates are converted into cationic intermediates, which can subsequently undergo inter- or intramolecular addition of nucleophiles.

Intramolecular Metal-Free C(sp)-H Activation Enables a Selective Mono -Debenzylation of Fully Protected Aminosugars.

Carbamate-bearing benzylated aminosugars undergo an I/I(III)-promoted intramolecular hydrogen atom transfer (IHAT) followed by a nucleophilic attack to provide polycyclic structures. Thus, suitably positioned benzyl ethers are surgically oxidized into the corresponding mixed /-benzylidene acetals, which can be conveniently deprotected under mild acidic conditions to grant access to selectively -deprotected aminosugars amenable for further derivatization. The scope of this strategy has been proven with a series of furanosic and pyranosic scaffolds.

Tandem Radical Fragmentation/Cyclization of Guanidinylated Monosaccharides Grants Access to Medium-Sized Polyhydroxylated Heterocycles.

The fragmentation of anomeric alkoxyl radicals (ARF) and the subsequent cyclization promoted by hypervalent iodine provide an excellent method for the synthesis of guanidino-sugars. The methodology described herein is one of the few existing general methodologies for the formation of medium-sized exo- and endoguanidine-containing heterocycles presenting a high degree of oxygenation in their structure.

Prenatal screening and diagnosis of genetic abnormalities: SEGO, SEQC, AEDP consensus recommendations.

In this paper, the scientific societies SEGO, SEQC and AEDP provide a series of consensus-based recommendations for prenatal screening and diagnosis of genetic abnormalities. A set of evaluation indicators are also proposed as a means to improve the quality of the biochemical, ultrasound, and genetic processes involved in prenatal screening and diagnosis of genetic anomalies. Some recommendations are also proposed in relation to invasive prenatal diagnostic procedures, more specifically regarding sample collection and genetic testing.

Skin Autofluorescence of Pregnant Women With Diabetes Predicts the Macrosomia of Their Children.

Advanced glycation end products (AGEs) accumulated during long-term hyperglycemia are involved in diabetes complications and can be estimated by skin autofluorescence (sAF). During pregnancy, hyperglycemia exposes women to the risk of having a macrosomic newborn. The aim of this study was to determine whether sAF of women with diabetes during a singleton pregnancy could predict macrosomia in their newborns.

Synthesis of Chiral Polyhydroxylated Benzimidazoles by a Tandem Radical Fragmentation/Cyclization Reaction: A Straight Avenue to Fused Aromatic-Carbohydrate Hybrids.

The synthesis of benzimidazole-fused iminosugars through a tandem β-fragmentation-intramolecular cyclization reaction is described. The use of the benzimidazole ring as the internal nucleophile and the use of phenyliodosophthalate (PhI(Phth)), a new metal-free and low toxic hypervalent iodine reagent, are the most remarkable novelties of this synthetic strategy. With this approach, we have demonstrated the usefulness of the fragmentation of anomeric alkoxyl radicals promoted by the PhI(Phth)/I system for the preparation of new compounds with potential interest for both medicinal and synthetic chemists.

Efficacy and safety of anticoagulation in non-malignant portal vein thrombosis in patients with liver cirrhosis.

Background And Aim: Treatment for portal vein thrombosis (PVT) is not well established. Nevertheless, anticoagulation therapy can seemingly be used as first-line therapy. However, there are limited data on the role of this treatment in patients with PVT and cirrhosis.

The Road from Host-Defense Peptides to a New Generation of Antimicrobial Drugs.

Host-defense peptides, also called antimicrobial peptides (AMPs), whose protective action has been used by animals for millions of years, fulfill many requirements of the pharmaceutical industry, such as: (1) broad spectrum of activity; (2) unlike classic antibiotics, they induce very little resistance; (3) they act synergically with conventional antibiotics; (4) they neutralize endotoxins and are active in animal models. However, it is considered that many natural peptides are not suitable for drug development due to stability and biodisponibility problems, or high production costs. This review describes the efforts to overcome these problems and develop new antimicrobial drugs from these peptides or inspired by them.

Chemoselective intramolecular functionalization of methyl groups in nonconstrained molecules promoted by N-iodosulfonamides.

Mechanistic evidence observed in Hofmann-Löffler-Freytag-type reactions has been crucial to achieve the chemoselective functionalization of methyl groups under mild conditions. Radical-mediated methyl iodination and subsequent oxidative deiodination are the key steps in this functionalization, where iodine chemistry has a pivotal role on the formation of the C-N bond. The concepts of single hydrogen atom transfer (SHAT) and multiple hydrogen atom transfer (MHAT) are introduced to describe the observed chemoselectivity.

Improving the relationship between soil characteristics and metal bioavailability by using reactive fractions of soil parameters in calcareous soils.

The contribution of the nature instead of the total content of soil parameters relevant to metal bioavailability in lettuce was tested using a series of low-polluted Mediterranean agricultural calcareous soils offering natural gradients in the content and composition of carbonate, organic, and oxide fractions. Two datasets were compared by canonical ordination based on redundancy analysis: total concentrations (TC dataset) of main soil parameters (constituents, phases, or elements) involved in metal retention and bioavailability; and chemically defined reactive fractions of these parameters (RF dataset). The metal bioavailability patterns were satisfactorily explained only when the RF dataset was used, and the results showed that the proportion of crystalline Fe oxides, dissolved organic C, diethylene-triamine-pentaacetic acid (DTPA)-extractable Cu and Zn, and a labile organic pool accounted for 76% of the variance.

Synthesis of branched iminosugars through a hypervalent iodine(III)-mediated radical-polar crossover reaction.

The synthesis of a novel type of branched iminosugars is described. This synthetic strategy is based on two key reactions: first, an aldol reaction with formaldehyde in order to introduce selectively the hydroxymethyl branch, and second, a tandem β-fragmentation-intramolecular cyclization reaction. The combination of both reactions afforded a battery of compounds exhibiting a great structural complexity, with the concomitant formation of a quaternary center, starting from readily available aldoses.

Hepatic Steatosis, Carbohydrate Intake, and Food Quotient in Patients with NAFLD.

Is steatosis related to the spontaneous carbohydrate intake in patients with NAFLD? We performed dietary records for 24 patients with NAFLD, 3 months after their liver biopsy was performed and before the deliverance of a dietary advice. The food quotient, indicator of the proportion of calories from carbohydrates, was calculated as (1.00×%  calories from carbohydrates/100) + (0.

Using of magnetic resonance enterography in the management of Crohn's disease of the small intestine: first year of experience.

Objective: to describe the experience at two tertiary centres during the first year of use of magnetic resonance enterography (MRE) for the management of Crohn's disease (CD): indications and influence of the technique in clinical decision making.

Materials And Methods: retrospective descriptive study in which patients who underwent MRE were included consecutively. Epidemiological and clinical data were collected from the patients, as well as the indication for the study and how it influenced clinical decision making in the 10 days following the radiological study.

Metal contamination disturbs biochemical and microbial properties of calcareous agricultural soils of the Mediterranean area.

Mediterranean climate characteristics and carbonate are key factors governing soil heavy-metal accumulation, and low organic matter (OM) content could limit the ability of microbial populations to cope with resulting stress. We studied the effects of metal contamination on a combination of biological parameters in soils having these characteristics. With this aim, soils were spiked with a mixture of cadmium, copper, lead, and zinc, at the two limit values proposed by current European legislation, and incubated for ≤12 months.

Can continuous glucose monitoring systems predict glycemia?

Background: We investigated whether the arrow on a continuous glucose monitoring system (CGMS) screen predicts the course of the capillary glucose level 15 min later.

Subjects And Methods: Twenty-three patients with type 1 diabetes (age, 40±13 years; diabetes duration, 19±12 years; hemoglobin A1c, 8.5±1.