Enough with simplifying: "eat less and move more": at what point are we with the treatment of excess weight in paediatrics?

BACKGROUND: For years politics and healthcare, faced with the progressive increase in the prevalence of overweight and obesity in childhood, have wondered how to stem it and reduce its consequences on health without finding a valid, effective and applicable solution. Many studies have been written initially on what to prescribe, then on why not to prescribe and how to approach people in a new and more effective way to improve their behaviors, considered the main cause of excess weight. Over the last twenty years it has been highlighted that no diet or physical exercise is truly effective and not even global changes in lifestyle guarantee the large weight reductions traditionally expected, despite offering significant health advantages. A new approach is necessary and we must begin by working on ourselves. MAIN BODY: We examined literature on weight stigma and considered expert opinions, as well as feedback from parents/caregivers and patients. Literature on stigma has grown enormously in recent years, and finally considers the opinion of parents and patients. By interviewing patients with obesity, it was discovered that very often healthcare workers do not communicate the diagnosis and, if they do, they have a blaming attitude, holding patients responsible for their weight. Furthermore, when these people become aware of their obesity and seek treatment, they do not find adequate professionals and centers. Failure was mostly due to the enormous burden of obesity stigma and discrimination which, especially in children and young people, encourages internalization of the problem and takes away their self-efficacy, desire and ability to take care of themselves. CONCLUSIONS: New actions are needed to change all this. We propose "Training, Networking and Contrasting Weight Stigma". Now that we've figured out where to start, we should get going. And yet, nothing is changing!

Human height estimation from highly distorted surveillance image.

Video surveillance camera (VSC) is an important source of information during investigations especially if used as a tool for the extraction of verified and reliable forensic measurements. In this study, some aspects of human height extraction from VSC video frames are analyzed with the aim of identifying and mitigating error sources that can strongly affect the measurement. More specifically, those introduced by lens distortion are present in wide-field-of-view lens such as VSCs. A weak model, which is not able to properly describe and correct the lens distortion, could introduce systematic errors. This study focuses on the aspect of camera calibration to verify human height extraction by Amped FIVE software, which is adopted by the Forensic science laboratories of Carabinieri Force (RaCIS), Italy. A stable and reliable approach of camera calibration is needed since investigators have to deal with different cameras while inspecting the crime scene. The performance of the software in correcting distorted images is compared with a technique of single view self-calibration. Both approaches were applied to several frames acquired by a fish-eye camera and then measuring the height of five different people. Moreover, two actual cases, both characterized by common low-resolution and distorted images, were also analyzed. The height of four known persons was measured and used as reference value for validation. Results show no significant difference between the two calibration approaches working with fish-eye camera in test field, while evidence of differences was found in the measurement on the actual cases.

What's the name? Weight stigma and the battle against obesity.

Childhood obesity has spread worldwide, it is on the rise, starts earlier and is more severe, despite all treatment attempts.According to recent studies, weight stigma is a factor that can hinder the success of therapies. Healthcare workers, mainly paediatricians, need to feel the urgency of anti-stigma training. The use of non-stigmatizing terminologies and images in various areas (school, sports clubs, healthcare, media, society in general) can improve disease management.

Thalassemia and hepatocellular carcinoma: links and risks.

The increased survival and lifespan of thalassemia patients, in the setting of better iron overload monitoring and chelation, have also however increased the incidence of diseases and complications, which were less likely to develop. Among these, one of the most worrying in recent years is hepatocellular carcinoma (HCC). Due to blood transfusions, many patients with thalassemia are or have been infected with hepatitis C virus (HCV) or hepatitis B virus (HBV), especially those born before the 1990s or in countries in which universal HBV vaccination and safe blood programs are still not completely implemented. However, HCC has also been described in nontransfused patients and in those who are HCV- and HBV-negative. Therefore, other risk factors are involved in hepatocarcinogenesis in thalassemia. The following review analyzes recent literature on the role of different risk factors in the progression of liver disease in thalassemia as well as the importance of surveillance. Treatment of HCC in thalassemia is still highly debated and requires further studies.

Predicting factors for liver iron overload at the first magnetic resonance in children with thalassaemia major.

BACKGROUND: Transfusion dependency determines iron overload in thalassaemia major, with devastating complications. Significant liver iron overload has been observed from early childhood and we aimed to evaluate factors that could predict liver iron overload at the first magnetic resonance imaging (MRI). MATERIALS AND METHODS: All transfusion-dependent children who underwent MRI to assess iron overload were retrospectively studied. Age, weight, height, blood requirement, chelation drug and dosage, serum ferritin and liver enzymes were evaluated at three specific steps: start of transfusion regimen, start of chelation therapy, and first MRI. RESULTS: Among 198 patients, 25 children met inclusion criteria. No differences were detected in all the assessed parameters at start of transfusion regimen and chelation therapy (p>0.05) between patients with good iron balance (liver iron concentration [LIC] <7 mg Fe/g dry weight [dw]) and liver iron overload (LIC >7). At the first MRI, patients with iron overload had significantly higher serum ferritin (3,080.3±1,078.5 vs 1,672.0±705.3 ng/mL; p<0.01) while patients with good iron control maintained a stable ferritin value from the start of chelation therapy but showed significantly lower height Z-score (-1.48±1.02 vs -0.36±1.55; p=0.04). Serum ferritin >1,770 ng/mL was detected as the best threshold for predicting liver iron overload at the first MRI (p=0.0003). CONCLUSION: In order to prevent liver iron overload at the first MRI, children should maintain a stable level of serum ferritin below 1,770 from the start of chelation therapy. However, strict monitoring of growth is mandatory.

Sensors for Deformation Monitoring of Large Civil Infrastructures.

In the maintenance of large infrastructures such as dams, bridges, railways, underground structures (tunnels, mines) and others, monitoring of deformations plays a key role in maintaining the safety serviceability conditions and for mitigating any consequences due to ageing factors and possible structural failures. [...].

Geodetic and Remote-Sensing Sensors for Dam Deformation Monitoring.

In recent years, the measurement of dam displacements has benefited from a great improvement of existing technology, which has allowed a higher degree of automation. This has led to data collection with an improved temporal and spatial resolution. Robotic total stations and GNSS (Global Navigation Satellite System) techniques, often in an integrated manner, may provide efficient solutions for measuring 3D displacements on precise locations on the outer surfaces of dams. On the other hand, remote-sensing techniques, such as terrestrial laser scanning, ground-based SAR (synthetic aperture radar) and satellite differential interferometric SAR offer the chance to extend the observed region to a large portion of a structure and its surrounding areas, integrating the information that is usually provided in a limited number of in-situ control points. The design and implementation of integrated monitoring systems have been revealed as a strategic solution to analyze different situations in a spatial and temporal context. Research devoted to the optimization of data processing tools has evolved with the aim of improving the accuracy and reliability of the measured deformations. The analysis of the observed data for the interpretation and prediction of dam deformations under external loads has been largely investigated on the basis of purely statistical or deterministic methods. The latter may integrate observation from geodetic, remote-sensing and geotechnical/structural sensors with mechanical models of the dam structure. In this paper, a review of the available technologies for dam deformation monitoring is provided, including those sensors that are already applied in routinary operations and some experimental solutions. The aim was to support people who are working in this field to have a complete view of existing solutions, as well as to understand future directions and trends.

Advanced SBAS-DInSAR Technique for Controlling Large Civil Infrastructures: An Application to the Genzano di Lucania Dam.

Monitoring surface deformation on dams is commonly carried out by in situ geodetic surveying, which is time consuming and characterized by some limitations in space coverage and frequency. More recently microwave satellite-based technologies, such as advanced-DInSAR (Differential Synthetic Aperture Radar Interferometry), have allowed the integration and improvement of the observation capabilities of ground-based methods thanks to their effectiveness in collecting displacement measurements on many non-destructive control points, corresponding to radar reflecting targets. The availability of such a large number of points of measurement, which are distributed along the whole structure and are characterized by millimetric accuracy on displacement rates, can be profitably adopted for the calibration of numerical models. These models are implemented to simulate the structural behaviour of a dam under conditions of stress thus improving the ability to maintain safety standards. In this work, after having analysed how advanced DInSAR can effectively enhance the results from traditional monitoring systems that provide comparable accuracy measurements on a limited number of points, an FEM model of the Genzano di Lucania earth dam is developed and calibrated. This work is concentrated on the advanced DInSAR technique referred to as Small BAseline Subset (SBAS) approach, benefiting from its capability to generate deformation time series at full spatial resolution and from multi-sensor SAR data, to measure the vertical consolidation displacement of the Genzano di Lucania earth dam.

Is there a difference in phenotype between males and females with non-transfusion-dependent thalassemia? A cross-sectional evaluation.

OBJECTIVES: Non-transfusion-dependent thalassemia includes a variety of phenotypes and genotypes that rarely require regular transfusions. However, these patients can experience a wide range of complications. The objective of this retrospective study was to verify whether there is a significant difference in non-transfusion-dependent thalassemia-related complications and treatment among males and females. METHODS: We performed a re-analysis of samples evaluated in a previously published cross-sectional study, regarding 96 non-transfusion-dependent thalassemia patients followed at the 'UOSD Malattie Rare del Globulo Rosso' Centre of the Cardarelli Hospital in Naples, Italy. RESULTS: We found that females were more anemic than males, but there was no significant difference in prevalence of common complications among genders, except for hypogonadism. Furthermore, the transitory regular transfusions regimen in women who had been pregnant does not seem to have a significant impact on overall prognosis. DISCUSSION: In non-transfusion-dependent thalassemia patients, the lower levels of hemoglobin found in females do not seem to indicate a higher prevalence of complications. CONCLUSION: This data should be considered in studies with experimental treatments aiming to correct anemia in patients with non-transfusion-dependent thalassemia. It should probably also be taken into account in order to set up different transfusion regimens among genders in transfusion-dependent patients.

The long-term and extensive efficacy of low dose thalidomide in a case of an untransfusable patient with Non-Transfusion-Dependent Thalassemia.

Patients with Non-Transfusion-Dependent Thalassemia may require regular transfusion therapy. However, these patients are at risk of developing irregular antibodies, making them untransfusable. Second line treatment usually includes hydroxyurea, which however is not effective in all patients. Other treatment options include thalidomide, which has been reported to be safe and effective in selected patients. We report the case of a patient who experienced improvement of hemoglobin levels and of a part of NTDT related complications, following 36months of continuous therapy with low doses of thalidomide.

Iron Chelation in Thalassemia Major.

PURPOSE: Iron chelation has improved survival and quality of life of patients with thalassemia major. there are currently 3 commercially available iron-chelating drugs with different pharmacokinetic and pharmacodynamic activity. The choice of adequate chelation treatment should be tailored to patient needs and based on up-to-date scientific evidence. METHODS: A review of the most recent literature was performed. FINDINGS: The ability of the chelators to bind the redox active component of iron, labile plasma iron, is crucial for protecting the cells. Chelation therapy should be guided by magnetic resonance imaging that permits the tailoring of therapy according to the needs of the patient because different chelators preferentially clear iron from different sites. Normal levels of body iron seem to decrease the need for hormonal and cardiac therapy. IMPLICATIONS: The 3 chelators currently available have different benefits, different safety profiles, and different acceptance on the part of the patients. Good-quality, well-designed, randomized, long-term clinical trials continue to be needed.

A complicated disease: what can be done to manage thalassemia major more effectively?

Patients with thalassemia major suffer from many complications, but in the last two decades their lives have improved both in length and quality. We report herein the most common complications and the recent advances that have changed the course of this disease. Also, we report in detail some of the new therapeutic strategies already introduced in practice and briefly some that are still being developed.

Profile of deferasirox for the treatment of patients with non-transfusion-dependent thalassemia syndromes.

It has been clearly shown that iron overload adds progressively significant morbidity and mortality in patients with non-transfusion-dependent thalassemia (NTDT). The lack of physiological mechanisms to eliminate the excess of iron requires effective iron chelation therapy. The reduced compliance to deferoxamine and the risk of severe hematological adverse events during deferiprone treatment have limited the use of both these drugs to correct iron imbalance in NTDT. According to the principles of evidence-based medicine, following the demonstration of the effectiveness and the safety of deferasirox (Exjade(®)) in a prospective, randomized, controlled trial, deferasirox was approved by the US Food and Drug Administration in May 2013 for the treatment of iron overload associated with NTDT. This review, assessing the available scientific literature, will focus on the profile of DFX in the treatment of non-transfusional hemosiderosis in patients with NTDT.

Characterization of Hb Calvino (HBB: c.406G > A): a new silent ß-globin gene variant found in coexistence with α-thalassemia in a family of African origin.

We report a new silent ß-globin gene variant found in a family from Angola living in the north eastern Italian city of Ferrara. The probands, two young sisters, presented with hematological parameters compatible with a ß-thalassemia (ß-thal) minor but with normal Hb A2 levels and normal hemoglobin (Hb) separation on high performance liquid chromatography (HPLC). Molecular analyses revealed a homozygosity for the common -α(3.7) (rightward) deletion and heterozygosity for a novel transition (GCT > ACT) at codon 135 of the ß-globin gene, leading to an Ala → Thr single amino acid substitution that was inherited from the healthy father.

Transfusional iron overload and iron chelation therapy in thalassemia major and sickle cell disease.

Iron overload is an inevitable consequence of blood transfusions and is often accompanied by increased iron absorption from the gut. Chelation therapy is necessary to prevent the consequences of hemosiderosis. Three chelators, deferoxamine, deferiprone, and deferasirox, are presently available and a fourth is undergoing clinical trials. The efficacy of all 3 available chelators has been demonstrated. Also, many studies have shown the efficacy of the combination of deferoxamine plus deferiprone as an intensive treatment of severe iron overload. Alternating chelators can reduce adverse effects and improve compliance. Adherence to therapy is crucial for good results.

Macroorchidism in childhood and adolescence: an update.

In the last 30 years, scientific literature has been enriched with studies which demonstrate the importance of evaluating testis volume to recognize certain genetic and endocrine diseases. Macroorchidism is defined as an increase of testicular volume at least twice the norm for age. In clinical practice, when macroorchidism is suspected, the testicular volume may be evaluated with the Prader orchidometer and/or US, calculated by this formula: L x W x H x 0.71 and the resulting value should be compared with a table of percentiles for each age. Macroorchidism is a relatively uncommon sign; however, when present, it almost always has clinical relevance. Five groups of conditions are reviewed: genetic, endocrine, idiopathic and secondary to neoplasm or testicular torsions. An X-linked mental retardation syndrome must be suspected in all patients with macrorchidism, phenotypic abnormalities, and mental retardation. Furthermore, it is important to identify other males affected in the same family to confirm the X-linked transmission. In these cases, the patient must be referred to specialized cytogenetic centres for determination of fragile sites. Other possible etiologies of macroorchidism are long-standing primary hypothyroidism, adrenal remnants in congenital adrenal hyperplasia, follicle stimulating hormone (FSH)-secreting pituitary macroadenomas, local tumors, lymphomas, and aromatase deficiency. Early diagnosis is important in order to identify and reduce the incidence of X-linked mental retardation in affected families and to begin treatment in endocrinologic, tumoral and surgical disorders.

Universal neonatal screening for sickle cell disease and other haemoglobinopathies in Ferrara, Italy.

BACKGROUND: Sickle cell disease is the commonest haemoglobinopathy in Africa, the Middle East and India. In recent years, its incidence has increased dramatically also in Europe and North America because of the high rate of migration of people from endemic areas. From January 2009 to January 2010 the number of foreign residents in the province of Ferrara (Italy) increased by 12.2%: most of the immigrants were from countries at high risk of sickle cell disease. Since neonatal screening and prophylactic penicillin in early childhood could reduce mortality by 10 years of age to less than 2%, the aim of this study was to establish a neonatal screening programme for haemoglobinopathies in Ferrara. MATERIALS AND METHODS: First we assessed how many pregnant women underwent haemoglobin analysis by high performance liquid chromatography before or during pregnancy and how many of them were carriers of haemoglobinopathies. Subsequently, we verified the feasibility of neonatal screening for sickle cell disease and other haemoglobinopathies, analysing cord blood by high performance liquid chromatography. Neonates found to be positive were managed by a multidisciplinary team to implement all the appropriate prophylactic and therapeutic measures. RESULTS: We found that 59% of women who delivered at the University Hospital of Ferrara, from 2007 to 2009, had undergone high performance liquid chromatography. Of the 41% who were not tested, many were from areas in which sickle cell disease is common. Between September 26th 2010 and January 31st 2012, 1992 neonatal tests were performed and 24 carriers of haemoglobinopathies were identified (16 with HbS, 4 with HbC, 2 with HbE, 1 with HbD Punjab and 1 with HbD-Ouled Rabah); 42.6% of the mothers of these 1,992 neonates had not undergone high performance liquid chromatography during pregnancy. DISCUSSION: Currently prevention of haemoglobinopathies in Italy is provided during the pre-conception period but only to patients with abnormal blood counts. Neonatal screening is useful and cost-effective to ensure early diagnosis and appropriate treatment for infants with sickle cell disease or other haemoglobinopathies.

Cardiac iron and cardiac disease in males and females with transfusion-dependent thalassemia major: a T2* magnetic resonance imaging study.

BACKGROUND: It has been repeatedly reported that female patients with thalassemia major survive longer than males and that the difference is due to a lower rate of cardiac disease in females. DESIGN AND METHODS: We compared the cardiac iron load as measured by T2* magnetic resonance imaging in 776 patients (370 males) examined at the National Research Council as part of an Italian cooperative study. We also established normal left ventricular ejection fraction values for our population. RESULTS: The prevalence of cardiac disease was higher in males than in females (105 males versus 69 females; P < 0.0001). Cardiac T2* was significantly lower in patients with heart dysfunction (P < 0.0001), but no difference was observed according to sex. Twenty males and five females had a history of cardiac arrhythmias. Their cardiac T2* was not significantly lower than that of patients without arrhythmias (24 ms versus 26 ms; P = 0.381), nor was there a difference between sexes. Liver T2* was significantly lower in males and females with heart dysfunction compared to those without. Ferritin levels were higher in patients of both sexes with heart dysfunction without significant differences between males and females. Conclusions Males and females are at the same risk of accumulating iron in their hearts, but females tolerate iron toxicity better, possibly as an effect of reduced sensitivity to chronic oxidative stress.