Interobserver variability of ultrasound measurements for the differential diagnosis of uterine prolapse and cervical elongation without uterine prolapse.

OBJECTIVES: Our study aims to determine the interobserver variability of different ultrasound measurements (pubis-cervix distance, pubis-uterine fundus distance, and pubis-Douglascul-de-sac distance) previously analyzed for the ultrasound differential diagnosis of uterine prolapse (UP) and cervical elongation CE without UP. MATERIALS AND METHODS: We conducted a prospective observational study with 40 patients scheduled to undergo surgical correction of UP and CE without UP. All patients underwent pelvic floor ultrasound examination by an examiner (E1) who acquired ultrasound images. Using these images, E1 measured the distances for the ultrasound differential diagnosis of UP and CE without UP, and these distances were compared with those measured by the other examiner (E2). Values were analyzed by calculating ICCs with 95% CIs. RESULTS: For UP, excellent reliability was obtained for all measurements except the pubis-Douglascul-de-sac measurement at rest, which was moderate (ICC 0.596; p = 0.028) and for the difference between the pubis-Douglascul-de-sac measurement at rest and during the Valsalva maneuver, which was good (ICC 0.691; p < 0.0005). For CE without UP, interobserver reliability was excellent for all measurements analyzed except the pubis-cervix measurement during the Valsalva maneuver, which was moderate (ICC 0.535; p = 0.052) and for the pubis-Douglascul-de-sac measurement at rest, which was good (ICC 0.768; p < 0.0005). CONCLUSIONS: There is excellent interobserver reliability in measurements of the difference in the distance from the pubic symphysis to the uterine fundus at rest and during the Valsalva maneuver for both UP and CE without UP, which are used for the ultrasound differential diagnosis of UP and CE without UP.

Multicenter global early feasibility study to evaluate total endovascular arch repair using three-vessel inner branch stent-grafts for aneurysms and dissections.

OBJECTIVE: We evaluated the outcomes of total endovascular aortic arch repair using three-vessel inner branch stent-grafts for aneurysms and chronic dissections. METHODS: We reviewed the clinical data and outcomes of consecutive patients treated by total endovascular aortic arch repair at eight academic centers using three-vessel inner branch stent-grafts (William Cook Europe, Bjaeverskov, Denmark) from 2016 to 2019. All patients received three-vessel designs with two antegrade and one retrograde inner branch, which was used to incorporate the innominate, left common carotid, and left subclavian arteries. The antegrade inner branches were accessed via a carotid or an upper extremity approach. A preloaded catheter was used for access to the retrograde left subclavian artery branch via a transfemoral approach. The endpoints were technical success, mortality, major adverse events, any stroke (minor or major) or transient ischemia attack, secondary interventions, target vessel patency, target vessel instability, aneurysm-related mortality, and patient survival. RESULTS: A total of 39 patients (31 men [79%]; mean age, 70 ± 7 years) had undergone treatment of 14 degenerative (36%) and 25 chronic (64%) postdissection arch aneurysms. The clinical characteristics included American Society of Anesthesiologists class ≥III in 28 patients (95%) and previous median sternotomy for ascending aortic repair in 28 patients (72%). The technical success rate was 100%. Two patients had died in-hospital or within 30 days (5%), and two patients had experienced a stroke (one minor). The combined mortality and any stroke rate was 8% (n = 3). Major adverse events occurred in 10 patients (26%), including respiratory failure in 4 (10%) and estimated blood loss >1 L, myocardial infarction, and acute kidney injury in 2 patients each (5%). The median follow-up was 3.2 months (interquartile range, 1-14 months). Of the 39 patients, 12 (31%) required secondary interventions to treat vascular access complications in 5, endoleak in 6 (three type II, one type Ic, one type Ia/Ib, one type IIIa), and target vessel stenosis in 1 patient. At 1 year, the primary and secondary patency rates and freedom from target vessel instability were 95% ± 5%, 100%, and 91% ± 5%, respectively. Freedom from aortic-related mortality and patient survival was 94% ± 4% and 90% ± 6%, respectively. CONCLUSIONS: The findings from the present multicenter global experience have demonstrated the technical feasibility and safety of total endovascular aortic arch repair for aneurysms and chronic dissections using three-vessel inner branch stent-grafts. The mortality and stroke rates compare favorably with those after open surgical repair in a higher risk group of patients. However, the rate of secondary interventions was high (31%), emphasizing need for greater experience and longer follow-up.

Endovascular Repair of Iliac Vein Laceration Associated with Complex Pelvic Fracture.

BACKGROUND: Iliac vein injury associated with pelvic fracture due to blunt trauma is an uncommon and difficult diagnosis but a life-threatening condition which often requires an emergent management. Although open repair has been traditionally used as the treatment of choice in unstable patients, it is controversial, given the difficulty due to injured vessel exposure in patients with significant retroperitoneal hematoma as well as tamponade effect loss associated with laparotomy. We present a challenging case of iliac vein laceration successfully treated by placement of a self-expanding covered stent. METHODS: A 15-year-old male was hemodynamically unstable and was transferred to our emergency department after a severe polytrauma due to a motorcycle accident. Contrast-enhanced computed tomography showed a left external iliac vein laceration with active bleeding and retroperitoneal hematoma as well as complex pelvic and left supracondylar femoral fractures. A 13 × 100 mm self-expanding covered stent was successfully deployed through duplex ultrasound-guided percutaneous approach of both femoral veins. RESULTS: The patient's blood pressure was normalized as soon as the stent graft was placed, and then femoral fracture was reduced and fixed. At 12-month follow-up, the patient remained asymptomatic, and stent-graft patency was confirmed. CONCLUSIONS: Covered stent-graft placement can be an effective and rapid treatment for life-threatening iliac vein injury.

A youth-led social marketing intervention to encourage healthy lifestyles, the EYTO (European Youth Tackling Obesity) project: a cluster randomised controlled0 trial in Catalonia, Spain.

BACKGROUND: The encouragement of healthy lifestyles for obesity prevention in young people is a public health priority. The European Youth Tackling Obesity (EYTO) project is a multicentric intervention project with participation from the United Kingdom, Portugal, the Czech Republic and Spain. The general aim of the EYTO project is to improve lifestyles, including nutritional habits and physical activity practice, and to prevent obesity in socioeconomically disadvantaged and vulnerable adolescents. The EYTO project works through a peer-led social marketing intervention that is designed and implemented by the adolescents of each participating country. Each country involved in the project acts independently. This paper describes the "Som la Pera" intervention Spanish study that is part of the EYTO project. METHODS/DESIGN: In Spain, the research team performed a cluster randomised controlled intervention over 2 academic years (2013-2015) in which 2 high-schools were designated as the control group and 2 high-schools were designated as the intervention group, with a minimum of 121 schoolchildren per group. From the intervention group, 5 adolescents with leadership characteristics, called "Adolescent Challenge Creators" (ACCs), were recruited. These 5 ACCs received an initial 4 h training session about social marketing principles and healthy lifestyle theory, followed by 24 sessions (1.30 h/session) divided in two academic years to design and implement activities presented as challenges to encourage healthy lifestyles among their peers, the approximately 180-200 high-school students in the intervention group. During the design of the intervention, it was essential that the ACCs used the 8 social marketing criteria (customer orientation, behaviour, theory, insight, exchange, competition, segmentation and methods mix). The expected primary outcomes from the Spanish intervention will be as follows: increases in the consumption of fruits and vegetables and physical activity practice along with reductions in TV/computer/game console use. The secondary outcomes will be as follows: increased breakfast consumption, engagement with local recreation and reduced obesity prevalence. The outcomes will be measured by the Health Behaviour in School-aged Children Study (HBSC) survey at baseline and at the end of the intervention. In the control group, no intervention was implemented, but the outcome measurements were collected in parallel with the intervention group. DISCUSSION: This study described a new methodology to improve lifestyles and to address adolescent obesity. TRIAL REGISTRATION: ClinicalTrials.gov: NCT02157402. Registered 03 June 2014.

Exploring the prevalence and risk factors of benign and premalignant oral lesions in an adult population from Northern Spain: a pilot study.

OBJECTIVE: The goal was to identify the lifestyle risk factors associated with benign and potentially malignant oral disorders. METHOD AND MATERIALS: The study enrolled first-time patients from the Oral Pathology Section, volunteers from Oviedo, and first-time patients from the University of Oviedo dental clinic. Patients underwent a survey that included sociodemographic information, lifestyle habits, and medical history. A comprehensive examination of the oral mucosa was conducted. Univariate and multivariate logistic regression were conducted using R software. RESULTS: Among the 183 participants, the most prevalent lesions were varicose veins (43.20%), cheek/lip biting (34.97%), and coated tongue (33.33%). Among the oral potentially malignant disorders (16.39%) were oral lichen planus (12.64%) and leukoplakia (3.33%). Tobacco was associated with melanotic pigmentation (OR 3.87, P = .001) and coated tongue (OR 5.90, P = .001). Longer intervals since the last check-up were associated with traumatic keratosis (OR 2.95, P = .031). Age and heavy smoking were found to have higher risk of developing an oral potentially malignant disorder (OR 1.04, P = .035, and OR 7.35, P = .028, respectively). CONCLUSIONS: These data should be considered when organizing public health programs focused on the detection and screening of heavy smokers. It is also important to strengthen the oral pathology units in universities as reference centers for students to acquire the necessary knowledge for their diagnosis and treatment, while simultaneously promoting awareness of this risk factor for oral precancer among the general population.

Biological Effects of Orthodontic Tooth Movement on the Periodontium in Regenerated Bone Defects: A Scoping Review.

The aim of this scoping review is to analyse the biological effects of the orthodontic tooth movement (OTM) in areas with bone defects that are undergoing regeneration using different types of regenerative materials and techniques. The electronic research was performed on four databases as follows: PubMed, Scopus, EMBASE, and Web of Science. Data were extracted according to publication information, study design, sample characteristics, parameters of OTM, biological repercussions on the periodontium complex, methods of analysis, and conclusions. A total of thirty studies were included in the final review. In twenty-two studies, the most widely adopted grafting materials were alloplastics. In most studies, the orthodontic force used was 10 or 100 g, and the timing of application ranged from immediate to 6 months after grafting surgery. Twenty-four studies showed an increase in osteogenesis; in five studies, the clinical attachment level (CAL) increased; in five others, the probing pocket depth (PPD) decreased; in sixteen studies, there was root resorption of a different magnitude. Though the effects of OTM on the periodontium in the grafted areas were positive, the outcomes should be interpreted with caution as future preclinical and clinical studies are needed to extrapolate more valid conclusions.

Effectiveness of Elastodontic Devices for Correcting Sagittal Malocclusions in Mixed Dentition Patients: A Scoping Review.

Elastodontics is an interceptive orthodontic therapy that uses light and biological elastic forces through preformed or custom-made removable orthodontic appliances. This study aims to evaluate the effects of elastodontic devices on correcting sagittal discrepancies in growing subjects with mixed dentition. Electronic research was conducted on four databases: PubMed, Scopus, EMBASE, and Web of Science. Data were extracted based on the first author, year of publication, setting and country, study design, sample characteristics, sample size calculation, type of malocclusion, intervention, control group type, compliance, follow-up, and cephalometric measurements. Sixteen studies were included in the final review. Most studies observed a statistically significant reduction (p < 0.05) in SNB and ANB angles. Ten studies reported a reduction in overjet, while eight studies found no change in facial divergence. Comparisons with conventional functional devices revealed no consensus on the skeletal and dentoalveolar effects. Elastodontic appliances significantly improve cephalometric and dentoalveolar parameters, potentially correcting skeletal and dental relationships. However, result variability and unclear advantages over traditional appliances highlight the need for further research.

Temporomandibular Disorders Management-What's New? A Scoping Review.

Temporomandibular disorders include various conditions that impact the masticatory system, affecting its structure, function, or physiology. Clinicians face a complex array of therapeutic options when treating this group of diseases, emphasizing the importance of evaluating the current evidence to guide decisions in patient care. The main objective of this article is to conduct a scoping review on the available treatment approaches to manage temporomandibular disorders (TMDs). An extensive search of the literature was performed on Scopus, Pubmed, Embase, and Web of Science. Systematic reviews published in the last 5 years were considered. Out of the 2183 publications identified, 109 studies were included in this review. Among them, 39 articles focused on the non-invasive approach, while 120 delved into the minimally invasive approach, and 15 explored the surgical approach. Non-invasive or conservative approaches like cognitive-behavioral therapy, physical therapy, and acupuncture offer effective pain management and functional improvements in TMDs. Emerging treatments offer promising alternatives for treating these disorders. Surgery should be reserved for severe cases, with conservative therapies used in conjunction with invasive procedures for optimal patient outcomes.

Development and Application of a Lignin-Based Polyol for Sustainable Reactive Polyurethane Adhesives Synthesis.

In response to the environmental impacts of conventional polyurethane adhesives derived from fossil fuels, this study introduces a sustainable alternative utilizing lignin-based polyols extracted from rice straw through a process developed at INESCOP. This research explores the partial substitution of traditional polyols with lignin-based equivalents in the synthesis of reactive hot melt polyurethane adhesives (HMPUR) for the footwear industry. The performance of these eco-friendly adhesives was rigorously assessed through Thermogravimetric Analysis (TGA), Differential Scanning Calorimetry (DSC), rheological analysis, and T-peel tests to ensure their compliance with relevant industry standards. Preliminary results demonstrate that lignin-based polyols can effectively replace a significant portion of fossil-derived polyols, maintaining essential adhesive properties and marking a significant step towards more sustainable adhesive solutions. This study not only highlights the potential of lignin in the realm of sustainable adhesive production but also emphasises the valorisation of agricultural by-products, thus aligning with the principles of green chemistry and sustainability objectives in the polymer industry.

Direct healthcare costs of oral cancer: A retrospective study from a tertiary care center.

The aim of this study was to retrospectively evaluate the direct costs of OSCC treatment and postsurgical surveillance in a tertiary hospital in northeast Italy. Sixty-three consecutive patients surgically treated for primitive OSCC at S. Orsola Hospital in Bologna (Italy) between January 2018 and January 2020 were analyzed. Billing records of the Emilia Romagna healthcare system and institutional costs were used to derive specific costs for the following clinical categories: operating theatre costs, intensive and ordinary hospitalization, radiotherapy, chemotherapy, postsurgical complications, visits, and examinations during the follow-up period. The study population comprised 17 OSCC patients classified at stage I, 14 at stage II, eight at stage III, and 24 at stage IV. The estimated mean total direct cost for OSCC treatment and postsurgical surveillance was €26 338.48 per patient (stage I: €10 733, stage II: €19 642.9, stage III: €30 361.4, stage IV: €39 957.2). An advanced diagnosis (stages III and IV), complex surgical procedure, and loco-regional recurrences resulted in variables that were significantly associated with a higher cost of OSCC treatment and postsurgical surveillance. Redirection of funds used for OSCC treatment to screening measures may be an effective strategy to improve overall health outcomes and optimize national health resources.

Public Awareness on Oral Cancer: A Population- Based Study in Asturias.

OBJECTIVE: Through awareness campaigns, we can change the patient's abilities to detect oral cancer at an early stage and their ability to seek help. To focus these campaigns, we need to know the level of knowledge of the population and its interest in learning about this disease. The aim of this study was to assess the level of oral cancer awareness in Asturias and the interest of the population in learning about this pathology. METHODS: A representative community-based survey was carried out online using Google Forms®. Responses were transferred to a Microsoft Excel and analysed using the R-program. The relationship between two qualitative variables was studied using Pearson's Chi-square test or Fisher's test. Univariate and multivariate logistic regression models were used to determine which factors are associated with knowledge of oral cancer. RESULT: We found that those having over 50 years and being health professionals are more likely to know about the existence of oral cancer. Almost 85.1% of participants mentioned tobacco as a risk factor, only 39.8% identified alcohol. The ulcer was the most frequently recognized alarm sign (70.6%). The primary care physician was chosen as the first option for consultation by the 56.5% of the sample. Only 12.4% of the participants reported knowing how to self-examine their mouth. The number of views of a video of how-to self-inspection oral cavity displayed at the end of the questionnaire increased in a 39.38% during our study period. CONCLUSION: This survey showed a worrying lack of awareness and knowledge about oral cancer among the population of Asturias, especially among those under 50 years old. The interest shown in increasing their knowledge, give us hope in the success of future awareness campaigns.

Rhabdomyolysis following Nuss Procedure: A Prospective Study in Children.

INTRODUCTION: Postoperative rhabdomyolysis (RML) has been documented after several surgical procedures in adults. Musculoskeletal remodeling after Nuss procedure for pectus excavatum (PE) could cause RML. We evaluated the incidence of RML after Nuss procedure in children. METHODS: This study was a prospective study from 2018 to 2021. We enrolled all otherwise healthy patients who underwent PE correction with only one bar. Studied variables included demographic and clinical data, duration of surgery, complications, and length of hospitalization. The patients included underwent serial measurements of serum creatine kinase (CK), troponin I, N terminal pro B-type natriuretic peptide (NT-proBNP), serum creatinine, urea, and glomerular filtration rate at 6 and 48 hours postoperatively, and hospital discharge. RESULTS: Forty-six patients met criteria (40 males/6 females), with a mean age of 15.1 ± 1.4 years. Mean duration of surgery was 74 ± 28 minutes, and length of hospitalization was 4.6 ± 1.6 days. RML was diagnosed in 30.4% of patients at 6 hours, 91.3% at 48 hours, and 21.7% at hospital discharge. Mean preoperative CK value was 181.1 ± 141.6 IU/L, and postoperative values were 863.3 ± 302.6 IU/L at 6 hours, 1,675.2 ± 561 IU/L at 48 hours, and 850 ± 683.7 IU/L at hospital discharge, with statistically significant differences (p = 0.001). High-sensitivity troponin I and NT-proBNP levels increased significantly during the postoperative time (p = 0.001). Renal function remained stable (p = 0.55). CONCLUSION: Nuss technique produces RML without kidney injury in healthy patients. This knowledge should be considered for patients at increased risk of developing acute kidney injury and other complications.

Bidirectional Association between Lichen Planus and Hepatitis C-An Update Systematic Review and Meta-Analysis.

Lichen planus (LP) is a chronic, inflammatory mucocutaneous disorder associated with systemic diseases such as hepatitis C (HCV). The objective of this study is to evaluate the association between LP and HCV bidirectionally through a systematic review and meta-analysis. A comprehensive search of studies published was performed in the databases of PubMed, Embase, and Web of Science. Out of 18,491 articles, 192 studies were included. The global prevalence of HCV positive (HCV+) in LP patients registered from 143 studies was 9.42% [95% confidence interval (CI), 7.27-11.58%], and from these, 84 studies showed HCV+ 4-fold more frequent in LP than a control group (OR, 4.48; 95% CI, 3.48-5.77). The global prevalence of LP in patients HCV+ recorded from 49 studies was 7.05% (95% CI, 4.85-9.26%), and from these, 15 registered a 3-fold more LP in HCV (OR, 3.65; 95% CI, 2.14-6.24). HCV+ in LP patients showed great geographic variability (OR, 2.7 to 8.57), and the predominantly cutaneous location was higher (OR, 5.95) than the oral location (OR, 3.49). LP in HCV+ patients was more frequent in the Eastern Mediterranean (OR, 5.51; 95% CI, 1.40-15.57). There is a higher prevalence of HCV+ in LP and vice versa than in the control group, especially in certain geographical areas that should be taken into consideration when doing screening in countries with an upper prevalence of HCV among the general population.

Improving the communication between farmers and veterinarians to enhance the acceptability of bovine tuberculosis eradication programmes.

France and Spain have been fighting against bovine tuberculosis (bTB) for years, even though new outbreaks continue to appear on both sides of the border, generating misconceptions about the disease and social distrust in the eradication programme and competent authorities. The perceived disease risk and the commitment of all interested parties are key factors for the successful implementation of control programmes, as they might influence the acceptability of recommended measures. Effective communication can contribute to increasing knowledge, trust and stakeholders' engagement, thus ensuring the acceptability of the eradication programme. This study was conducted in Catalonia (Spain) and Pyrenees-Atlantiques (France) in the frame of the INNOTUB project (https://innotub.eu/) to characterise the communication on bTB in the trans-Pyrenees region and provide recommendations to improve it. The communication on bTB was characterised by analysing 153 (Spain) and 66 (France) online freely available texts, published between 2018 and 2020, through Content Analysis and Critical Metaphor Analysis. Moreover, six farmers and four veterinarians were in-depth interviewed in each area to gather information about the communication on bTB. Interviews were made in original languages and analysed using a qualitative thematic approach. A pilot participatory intervention inspired by the Systematic Tool for Behavioural Assumption Validation and Exploration (STAVE) method was used to develop a list of proposals to improve communication and to promote the creation of territorial networks/committees on bTB prevention and control. It included three focus groups with farmers and veterinarians, a meeting with representatives of the regional veterinary services, and a final deliberative workshop. Results highlight the existence of a controversial debate on bTB and a heterogeneous understanding between stakeholders. Institutional and scientific communication mainly focus on bTB detection and control while other aspects are left in the background. On the contrary, farmers extend their communication to a greater variety of topics. The metaphorical framing strongly differed among actors, while veterinary services and researchers "fight" against bTB and "progress" toward the eradication, farmers place themselves in a framework of "sacrifice" and, particularly in Spain, they play a passive role. The proposals developed by the participants to improve the current communication on bTB included: (i) create participatory meeting spaces to share opinions and information; (ii) improve data accessibility (on epidemiological situations); (iii) develop clearer and written protocols and informative visual material; (iv) redesign the training courses (v) increase the stakeholders' participation in the design of protocols.

Polyurethane Adhesives with Chemically Debondable Properties via Diels-Alder Bonds.

Covalent adaptable networks (CANs) represent a pioneering advance in polymer science, offering unprecedented versatility in materials design. Unlike conventional adhesives with irreversible bonds, CAN-based polyurethane adhesives have the unique ability to undergo chemical restructuring through reversible bonds. One of the strategies for incorporating these types of reactions in polyurethanes is by functionalisation with Diels-Alder (DA) adducts. By taking advantage of the reversible nature of the DA chemistry, the adhesive undergoes controlled crosslinking and decrosslinking processes, allowing for precise modulation of bond strength. This adaptability is critical in applications requiring reworkability or recyclability, as it allows for easy disassembly and reassembly of bonded components without compromising the integrity of the material. This study focuses on the sustainable synthesis and characterisation of a solvent-based polyurethane adhesive, obtained by functionalising a polyurethane prepolymer with DA diene and dienophiles. The characterisation of the adhesives was carried out using different experimental techniques: nuclear magnetic resonance spectroscopy (NMR), Brookfield viscosity, differential scanning calorimetry (DSC), thermogravimetric analysis (TGA) and T-peel strength testing of leather/adhesive/rubber joints to determine the adhesive properties, both before and after the application of external stimuli. The conversion of both the DA and retro-Diels-Alder (r-DA) reactions was confirmed by 1H-NMR. The adhesive properties were not altered by the functionalisation of the adhesive prepolymer, showing similar thermal resistance and good rheological and adhesive properties, even exceeding the most demanding technical requirements for upper-to-sole joints in footwear. After the application of an external thermal stimuli, the bonded materials separated without difficulty and without damage, thus facilitating their separation, recovery and recycling.

Role of Artificial Intelligence in the Early Diagnosis of Oral Cancer. A Scoping Review.

The early diagnosis of cancer can facilitate subsequent clinical patient management. Artificial intelligence (AI) has been found to be promising for improving the diagnostic process. The aim of the present study is to increase the evidence on the application of AI to the early diagnosis of oral cancer through a scoping review. A search was performed in the PubMed, Web of Science, Embase and Google Scholar databases during the period from January 2000 to December 2020, referring to the early non-invasive diagnosis of oral cancer based on AI applied to screening. Only accessible full-text articles were considered. Thirty-six studies were included on the early detection of oral cancer based on images (photographs (optical imaging and enhancement technology) and cytology) with the application of AI models. These studies were characterized by their heterogeneous nature. Each publication involved a different algorithm with potential training data bias and few comparative data for AI interpretation. Artificial intelligence may play an important role in precisely predicting the development of oral cancer, though several methodological issues need to be addressed in parallel to the advances in AI techniques, in order to allow large-scale transfer of the latter to population-based detection protocols.

Long-term outcomes of adult-size and size-matched kidney transplants in small pediatric recipients.

INTRODUCTION: Adult-size kidneys are usually used for kidney transplantation in small pediatric recipients, but the influence of graft size in transplant outcome remains controversial. Our aim is to compare long-term transplant outcomes of using adult-size and size-matched kidneys in small pediatric recipients. MATERIALS AND METHODS: Since 1999, 61 of 226 kidney transplants were achieved in recipients weighing <20 kg with 5 years of follow-up. Patients were analyzed according to the graft size received: (group-A) adult-size (n = 32), (group-B) size-matched (n = 29). Kidney size (KS), glomerular filtration rate (GFR) proteinuria and rejection were compared between groups at transplant time (T0), at one (T1), two (T2), five years (T5), and at the end of the follow-up (TF) (median follow-up 8.47(0-17) years). Graft and patient survival were determined and compared between groups. RESULTS: Mean KS was significantly different between groups at T0 (A:11.3 ± 1.1 cm, B:8.8 ± 0.9 cm), (pT0<0.01), group-B evidenced graft growth, reaching similar sizes to group-A at T5 (A:11.7±1 cm, B:11.2±1 cm; pT5 = 0.13) and TF (A:12.2 ± 1.1 cm, B:12.4 ± 1.2 cm; pTF = 0.63), and group-A had a slight graft growth at TF (pT0-TF<0.01). Mean Schwartz-GFR at T0 was greater in group-A (138 ± 33 ml/min/1.73 m2) than group-B (109 ± 34 mL/min/1.73 m2) (pT0 = 0.01); during follow-up, it evidenced a reduction in group-A (T5:90 ± 27, TF:71 ± 24 mL/min/1.73 m2; pT0-T5<0.01; pT0-TF<0.01), meanwhile in group-B was stable until T5 (104 ± 33 mL/min/1.73 m2; pT0-T5 = 0.54), declining at TF (76 ± 31 mL/min/1.73 m2; pT0-TF<0.01); with no significant differences at T1, T2, T5, and TF between groups. Similar results were observed in mean Filler-GFR of both groups (Figure). Proteinuria and episodes of rejection were no significantly different between groups during the follow-up (p > 0.01; p = 0.23). Graft and patient survival at 5 and 10 years did not show significant differences (p = 0.45; p = 0.10). DISCUSSION: Despite the initial kidney size difference between groups, we have demonstrated that they tended to the same size during the follow-up. Adult-size kidneys presented a slight size increase in the long-term, suggesting that they have some growth potential in small recipients, in contrast to previous literature. Mean GFR between groups showed no significant differences in the long-term, suggesting that optimal graft perfusion and function can be achieved despite the size of the graft. We have demonstrated that there were no significant differences in long-term graft and patient survival; this results were similar to the most recent literature about this topic and different from the 90-2000s decades literature. CONCLUSIONS: Adult-size kidneys may be transplanted to small recipients (<20 kg) with comparable outcomes to size-matched kidneys, with no significant differences in long-term KS, GFR, proteinuria, rejection, graft or patient survival.

Epilepsy and ovarian failure: Two cases of adolescent-onset ovarioleukodystrophy.

Vanishing white matter disease (VWM) was described by Van der Knaap in 1996. This association with premature ovarian failure is known as ovarioleukodystrophy. This is a rare entity caused by a mutation in one of the subunits of eukaryotic initiation factor 2B (EIF2B). The onset in adulthood or late in adolescence is very infrequent. A 41-years-old woman and her 37-years-old sister developed epilepsy in association with premature ovarian failure at the age of 13 and 18 respectively. The oldest-one started 17 years later progressive subcortical cognitive decline with predominant behavioural disorders and a progressive spastic paraparesis in association with symmetric cystic changes in the with matter of both hemispheres. In both patients we found the c.1117C>T (p.Arg373Cys) mutation in homozygosis in the EIF2B4 gen.

Steroid-resistant nephrotic syndrome: long-term evolution after sequential therapy.

We present a retrospective study of 30 children of mean age 3.02 +/- 1.81 years with steroid-resistant nephrotic syndrome (SRNS) treated with intravenous injection of methylprednisolone plus orally administered prednisone; 24 children also received cyclophosphamide (CP). Sixteen were resistant to steroids from the beginning, and 14 after a mean of 11.26 +/- 16.61 months. The initial histological diagnosis was: 18 minimal change disease (MCD), 11 focal segmental glomerulosclerosis (FSGS) and one diffuse mesangial proliferative glomerulonephritis (DMPG). Total remission was achieved in 22 patients (73.3%), partial response in three (10%) and no response in five (16.6%), two of whom were brothers carrying an NPHS2 gene double mutation. There was no difference in response between the MCD and FSGS patients; the only patient with DMPG did not respond. Only initial resistance was a sign of bad prognosis. At follow-up (6.4 +/- 3.6 years from last pulse), 21/22 were still in remission, 14/21 were without treatment. Six patients required cyclosporine or mycophenolate mofetil because of steroid dependence. Two non-responders developed end-stage renal failure (ESRF); the remaining patients maintained normal glomerular filtration. The treatment was well tolerated. In conclusion, most of the patients treated with sequential therapy consisting of methylprednisolone (MP) (100%) and CP (80%) showed remission and preserved renal function, but 20% developed steroid dependence.