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1.
IEEE Open J Eng Med Biol ; 5: 600-606, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39184967

RESUMO

THz radiationeffectively probes biological tissue water content due to its high sensibility to polar molecules. Skin and basal cell carcinoma (BCC), both rich in water, have been extensively studied in the THz range. Typically, the Double Debye model is used to study their dielectric permittivity. This work focuses on the viability of the multipole Cole-Cole model as an alternative dielectric model. To determine the best fit parameters, we used a genetic algorithm-based approach, solving a least squares problem. Compared with the Double Debye model, a maximum reduction of the RMSE value up to more than 50% and maximum relative percentage errors of 2.8% have been measured for both second and third order Cole-Cole models. Since the errors of the second and third order Cole-Cole models are similar, a two-poles model is enough to describe the behaviour both tissues from 0.2 THz to 2 THz.

2.
Front Public Health ; 12: 1439078, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39145166

RESUMO

Background: The use of Non-Pharmaceutical Interventions (NPIs) during the COVID-19 pandemic is debated. Understanding the consequences these measures may have on vulnerable populations including children and adolescents is important. Methods: This is a multicenter, quasi-experimental before-after study involving 12 hospitals of the North Italian Emilia-Romagna Region, with NPI implementation as the intervention event. The 3 years preceding NPI implementation (in March 2020) constituted the pre-pandemic phase. The subsequent 2 years were further subdivided into a school closure phase (SC) and a subsequent mitigation measures phase (MM) with milder restrictions. Interrupted Time Series (ITS) regression analysis was used to calculate PED Standardized Incidence Rate Ratios (SIRR) on the diagnostic categories exhibiting the greatest frequency and/or variation. Results: In the 60 months of the study there were 765,215 PED visits. Compared to the pre-pandemic rate, overall PED presentations dropped by 58 and 39% during SC and MM, respectively. "Symptoms, signs and Ill-defined conditions," "Injury and poisoning" and "Diseases of the Respiratory System" accounted for 74% of the reduction. A different pattern was instead seen for "Mental Disorders," which exhibited the smallest decrease during SC, and is the only category which rose already at the end of SC. ITS analysis confirmed the strong decrease during SC (level change, IRR 0.17, 95%CI 0.12-0.27) and a significant increase in MM (slope change, IRR 1.23, 95%CI 1.13-1.33), with the sharpest decline (-94%) and rise (+36%) observed in the "Diseases of the Respiratory System" category. Mental Disorders showed a significant increasing trend of 1% monthly over the whole study period exceeding pre-pandemic levels at the end of MM. Females and adolescents showed higher increasing rates both in SC and MM. Conclusion: NPIs appear to have influenced PED attendance in different ways according to diagnostic categories, mirroring different mechanisms of action. These effects are beneficial in some cases and harmful in others, and establishing a clear balance between pros and cons is a difficult task for public health decision makers. The role of NPIs on PED use appropriateness deserves investigation. The rise in pediatric mental disorders independent of the pandemic makes interventions addressing these issues urgent.


Assuntos
COVID-19 , Serviço Hospitalar de Emergência , Análise de Séries Temporais Interrompida , Humanos , COVID-19/epidemiologia , COVID-19/prevenção & controle , Itália/epidemiologia , Criança , Serviço Hospitalar de Emergência/estatística & dados numéricos , Feminino , Adolescente , Masculino , Pré-Escolar , Lactente , SARS-CoV-2
4.
Bioengineering (Basel) ; 11(6)2024 Jun 06.
Artigo em Inglês | MEDLINE | ID: mdl-38927809

RESUMO

Magnetic scaffolds (MagSs) are magneto-responsive devices obtained by the combination of traditional biomaterials (e.g., polymers, bioceramics, and bioglasses) and magnetic nanoparticles. This work analyzes the literature about MagSs used as drug delivery systems for tissue repair and cancer treatment. These devices can be used as innovative drugs and/or biomolecules delivery systems. Through the application of a static or dynamic stimulus, MagSs can trigger drug release in a controlled and remote way. However, most of MagSs used as drug delivery systems are not optimized and properly modeled, causing a local inhomogeneous distribution of the drug's concentration and burst release. Few physical-mathematical models have been presented to study and analyze different MagSs, with the lack of a systematic vision. In this work, we propose a modeling framework. We modeled the experimental data of drug release from different MagSs, under various magnetic field types, taken from the literature. The data were fitted to a modified Gompertz equation and to the Korsmeyer-Peppas model (KPM). The correlation coefficient (R2) and the root mean square error (RMSE) were the figures of merit used to evaluate the fitting quality. It has been found that the Gompertz model can fit most of the drug delivery cases, with an average RMSE below 0.01 and R2>0.9. This quantitative interpretation of existing experimental data can foster the design and use of MagSs for drug delivery applications.

5.
Front Public Health ; 12: 1393677, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38699417

RESUMO

Background: The use of Non-Pharmaceutical Interventions (NPIs), such as lockdowns, social distancing and school closures, against the COVID-19 epidemic is debated, particularly for the possible negative effects on vulnerable populations, including children and adolescents. This study therefore aimed to quantify the impact of NPIs on the trend of pediatric hospitalizations during 2 years of pandemic compared to the previous 3 years, also considering two pandemic phases according to the type of adopted NPIs. Methods: This is a multicenter, quasi-experimental before-after study conducted in 12 hospitals of the Emilia-Romagna Region, Northern Italy, with NPI implementation as the intervention event. The 3 years preceding the beginning of NPI implementation (in March 2020) constituted the pre-pandemic phase. The subsequent 2 years were further subdivided into a school closure phase (up to September 2020) and a subsequent mitigation measures phase with less stringent restrictions. School closure was chosen as delimitation as it particularly concerns young people. Interrupted Time Series (ITS) regression analysis was applied to calculate Hospitalization Rate Ratios (HRR) on the diagnostic categories exhibiting the greatest variation. ITS allows the estimation of changes attributable to an intervention, both in terms of immediate (level change) and sustained (slope change) effects, while accounting for pre-intervention secular trends. Results: Overall, in the 60 months of the study there were 84,368 cases. Compared to the pre-pandemic years, statistically significant 35 and 19% decreases in hospitalizations were observed during school closure and in the following mitigation measures phase, respectively. The greatest reduction was recorded for "Respiratory Diseases," whereas the "Mental Disorders" category exhibited a significant increase during mitigation measures. ITS analysis confirms a high reduction of level change during school closure for Respiratory Diseases (HRR 0.19, 95%CI 0.08-0.47) and a similar but smaller significant reduction when mitigation measures were enacted. Level change for Mental Disorders significantly decreased during school closure (HRR 0.50, 95%CI 0.30-0.82) but increased during mitigation measures by 28% (HRR 1.28, 95%CI 0.98-1.69). Conclusion: Our findings provide information on the impact of COVID-19 NPIs which may inform public health policies in future health crises, plan effective control and preventative interventions and target resources where needed.


Assuntos
COVID-19 , Hospitalização , Análise de Séries Temporais Interrompida , Humanos , COVID-19/epidemiologia , COVID-19/prevenção & controle , Itália/epidemiologia , Criança , Adolescente , Hospitalização/estatística & dados numéricos , Pré-Escolar , Feminino , Masculino , Distanciamento Físico , Hospitais Pediátricos/estatística & dados numéricos , SARS-CoV-2 , Controle de Doenças Transmissíveis , Lactente , Quarentena/estatística & dados numéricos , Instituições Acadêmicas , Estudos Controlados Antes e Depois , Pandemias
7.
IEEE Open J Eng Med Biol ; 5: 88-98, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38487100

RESUMO

Goal: Deep-seated tumors (DST) can be treated using thermoseeds exposed to a radiofrequency magnetic field for performing local interstitial hyperthermia treatment (HT). Several research efforts were oriented to the manufacturing of novel biocompatible magnetic nanostructured thermo-seeds, called magnetic scaffolds (MagS). Several iron-doped bioceramics or magnetic polymers in various formulations are available. However, the crucial evaluation of their heating potential has been carried out with significantly different, lab specific, variable experimental conditions and protocols often ignoring the several error sources and inaccuracies estimation. Methods: This work comments and provides a perspective analysis of an experimental protocol for the estimation methodology of the specific absorption rate (SAR) of MagS for DST HT. Numerical multiphysics simultions have been performed to outline the theoretical framework. After the in silico analysis, an experimental case is considered and tested. Results: From the simulations, we found that large overestimation in the SAR values can be found, due to the axial misplacement in the radiofrequency coil, while the radial misplacement has a lower impact on the estimated SAR value. Conclusions: The averaging of multiple temperature records is needed to reliably and effectively estimate the SAR of MagS for DST HT.

8.
Neurohospitalist ; 14(1): 64-68, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38235031

RESUMO

Background: Tick-Borne Encephalitis virus (TBEV) is a positive-sense single-stranded RNA virus belonging to the Flaviviridae family. TBEV transmission typically occurs through infected Ixodes tick bite or by consumption of unpasteurised milk from infected cattle. Case report: We report the clinical, neuroimaging, electroencephalogram (EEG), and laboratory (microbiological tests and spinal tap) data of a 6- year-old boy with Tick-borne encephalitis. Our patient presented with a biphasic course, initially with a myositis-like picture on his first admission to the emergency department, and after a few days with an encephalitic picture, resulting in a second hospitalization. EEG showed focal slow activity, while his brain magnetic resonance imaging (MRI) showed a signal abnormality, which completely resolved on repeat MRI after 3 months. Conclusion: To our knowledge, this is the youngest patient presenting with myositis in the first phase of Tick-borne encephalitis (TBE). In the presence of a biphasic clinical course, with previous myositis, aspecific MRI changes in the thalamic and midbrain regions and an EEG documenting slowed bioelectrical activity should prompt suspicion of TBEV infection.

9.
J Clin Med ; 12(17)2023 Aug 23.
Artigo em Inglês | MEDLINE | ID: mdl-37685533

RESUMO

Asthma is the most frequent chronic disease of childhood, affecting up to 20% of children worldwide. The main guidelines on asthma maintenance therapy in pediatrics suggest different approaches and describe different stages of asthma to determine the most appropriate treatment. This project aims to summarize the most recent evidence regarding maintenance therapy for asthma in children and adolescents. A multidisciplinary panel of experts was asked clinical questions regarding the treatment of children and adolescents with asthma. Overall, 10 clinical questions were addressed, and the search strategy included accessing electronic databases and a manual search of gray literature published in the last 25 years. After data extraction and narrative synthesis of results, recommendations were developed using the Grading of Recommendations, Assessment, Development, and Evaluations (GRADE) methodology. Results showed that the choice of medication depends on the severity of the child's asthma, phenotype, age, preference, and individual factors. In addition to medications, the identification of comorbidities and modifiable factors is crucial to obtaining good control. Asthma in children is heterogeneous, and its evolution varies over time. Since most recommendations for asthma management in childhood are extrapolated from clinical studies performed in adults, more clinical trials specifically designed for young children should be conducted.

10.
Antibiotics (Basel) ; 12(6)2023 Jun 12.
Artigo em Inglês | MEDLINE | ID: mdl-37370359

RESUMO

Background: Urinary tract infection (UTI) represents one of the most common infectious diseases and a major cause of antibiotic prescription in children. To prevent recurrent infections and long-term complications, low-dose continuous antibiotic prophylaxis (CAP) has been used. However, the efficacy of CAP is controversial. The aim of this document was to develop updated guidelines on the efficacy and safety of CAP to prevent pediatric UTIs. Methods: A panel of experts on pediatric infectious diseases, pediatric nephrology, pediatric urology, and primary care was asked clinical questions concerning the role of CAP in preventing UTIs in children. Overall, 15 clinical questions were addressed, and the search strategy included accessing electronic databases and a manual search of gray literature published in the last 25 years. After data extraction and narrative synthesis of results, recommendations were developed using the Grading of Recommendations, Assessment, Development, and Evaluations (GRADE) methodology. Results: The use of CAP is not recommended in children with a previous UTI, with recurrent UTIs, with vesicoureteral reflux (VUR) of any grade, with isolated hydronephrosis, and with neurogenic bladder. CAP is suggested in children with significant obstructive uropathies until surgical correction. Close surveillance based on early diagnosis of UTI episodes and prompt antibiotic therapy is proposed for conditions in which CAP is not recommended. Conclusions: Our systematic review shows that CAP plays a limited role in preventing recurrences of UTI in children and has no effect on its complications. On the other hand, the emergence of new antimicrobial resistances is a proven risk.

11.
Foods ; 12(12)2023 Jun 16.
Artigo em Inglês | MEDLINE | ID: mdl-37372607

RESUMO

Carasau bread is a flat bread, typical of Sardinia (Italy). The market of this food product has a large growth potential, and its industry is experiencing a revolution, characterized by digitalization and automation. To monitor the quality of this food product at different manufacturing stages, microwave sensors and devices could be a cost-effective solution. In this framework, knowledge of the microwave response of Carasau dough is required. Thus far, the analysis of the microwave response of Carasau doughs through dielectric spectroscopy has been limited to the dynamics of fermentation. In this work, we aim to perform complex dielectric permittivity measurements up to 8.5 GHz, investigating and modeling the role of water amount, salt and yeast concentrations on the spectra of this food product. A third-order Cole-Cole model was used to interpret the microwave response of the different samples, resulting in a maximum error of 1.58% and 1.60% for the real and imaginary parts of permittivity, respectively. Thermogravimetric analysis was also performed to support the microwave spectroscopy investigation. We found that dielectric properties of Carasau bread doughs strongly depend on the water content. The analysis highlighted that an increase in water quantity tends to increase the bounded water fraction at the expense of the free water fraction. In particular, the free water amount in the dough is not related to the broadening parameter γ2 of the second pole, whereas the bound water weight fraction is more evident in the γ2 and σdc parameters. An increase in electrical conductivity was observed for increasing water content. The microwave spectrum of the real part of the complex permittivity is slightly affected by composition, while large variation in the imaginary part of the complex dielectric permittivity can be identified, especially for frequencies below 4 GHz. The methodology and data proposed and reported in this work can be used to design a microwave sensor for retrieving the composition of Carasau bread doughs through their dielectric signature.

12.
Ital J Pediatr ; 49(1): 73, 2023 Jun 14.
Artigo em Inglês | MEDLINE | ID: mdl-37316947

RESUMO

BACKGROUND: Pyomyositis is an unusual bacterial infection but potential severe in children. Staphylococcus Aureus is the main caused of this disease (70-90%), following by Streptococcus Pyogenes (4-16%). Streptococcus Pneumoniae rarely caused invasive muscular infections. We describe a case of pyomyositis caused by Streptococcus Pneumonia in an adolescent 12-year-old female. CASE PRESENTATION: I.L. referred to our hospital for high fever associated with right hip and abdominal pain. The blood exams showed increase of leukocytes with prevalence of neutrophils with high level of inflammatory markers (CRP 46,17 mg/dl; Procalcitonin 25,8 ng/ml). The abdomen ultrasonography was unremarkable. The CT and MRI of the abdomen and right hip revealed pyomyositis of the iliopsoas, piriformis and internal shutter associated with collection of pus between the muscular planes (Fig. 1). The patient was admitted to our paediatric care unit, and she was initially treatment with intravenous Ceftriaxone (100 mg/kg/day) and Vancomycin (60 mg/kg/day). On day 2, a pansensitive Streptococcus Pneumoniae was isolated from the blood culture, and the antibiotic treatment was changed to only IV Ceftriaxone. She was successively treated with IV Ceftriaxone for 3 weeks, then continued with oral Amoxicillin for a total of 6 weeks of therapy. The follow up showed a complete resolution of the pyomyositis and psoas abscess after 2 months. CONCLUSION: Pyomyositis associate with abscess is a rare and very dangerous disease in children. The clinical presentation can mimic symptoms of other pathologies like osteomyelitis or septic arthritis, so many times is hard to identify. The main risk factors include story of recent trauma and immunodeficiency, not present in our case report. The therapy involves the antibiotics and, if possible, abscess drainage. In literature there is much discussion about duration of antibiotic therapy.


Assuntos
Piomiosite , Adolescente , Feminino , Criança , Humanos , Piomiosite/diagnóstico , Piomiosite/tratamento farmacológico , Streptococcus pneumoniae , Ceftriaxona , Abscesso/diagnóstico , Abscesso/tratamento farmacológico , Antibacterianos/uso terapêutico
13.
J Pediatr ; 256: 18-26.e8, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36470465

RESUMO

OBJECTIVE: To analyze, in a cohort of pediatric patients with recurrent pericarditis undergoing anti-interleukin (IL)-1 treatment: the agent and dosing used as first-line treatment, the long-term efficacy of IL-1 blockers, the percentage of patients achieving a drug-free remission, and the presence of variables associated with drug-free remission. STUDY DESIGN: Data were collected from patients' charts. The annualized relapse rate (ARR) was used for evaluation of treatment efficacy, and bivariate logistic regression analysis was used for variables associated with drug-free remission. RESULTS: Fifty-eight patients, treated between 2008 and 2018, were included in the study (mean follow-up. 2.6 years). Of the 56 patients treated with first-line drugs, 14 not responsive patients were underdosed. Fifty-seven patients were treated with anakinra: the ARR before and during daily treatment was 3.05 and 0.28, respectively (P < .0001); an increase to 0.83 was observed after the reduction/withdrawal of treatment (P < .0001). The switch from anakinra to canakinumab (5 patients) was associated to an increase of the ARR (0.49 vs 1.46), but without statistical significance (P = .215). At last follow-up, only 9 of the 58 patients had withdrawn all treatments. With the limits of a retrospective study and the heterogeneity between the patients enrolled in the study, a shorter duration of treatment with anakinra was the only variable associated with drug-free remission. CONCLUSIONS: This study shows that most pediatric patients with recurrent pericarditis needing IL-1 blockade received an inadequate treatment with first-line agents. The effectiveness of anakinra is supported by this study, but few patients achieved drug-free remission. The different rate of response to anakinra and canakinumab may suggest a possible role of IL-1α in the pathogenesis of recurrent pericarditis.


Assuntos
Proteína Antagonista do Receptor de Interleucina 1 , Pericardite , Humanos , Criança , Proteína Antagonista do Receptor de Interleucina 1/uso terapêutico , Estudos Retrospectivos , Interleucina-1/uso terapêutico , Padrão de Cuidado , Resultado do Tratamento , Pericardite/tratamento farmacológico , Recidiva
14.
J Clin Med ; 11(21)2022 Nov 04.
Artigo em Inglês | MEDLINE | ID: mdl-36362786

RESUMO

Wheezing at preschool age (i.e., before the age of six) is common, occurring in about 30% of children before the age of three. In terms of health care burden, preschool children with wheeze show double the rate of access to the emergency department and five times the rate of hospital admissions compared with school-age asthmatics. The consensus document aims to analyse the underlying mechanisms involved in the pathogenesis of preschool wheezing and define the risk factors (i.e., allergy, atopy, infection, bronchiolitis, genetics, indoor and outdoor pollution, tobacco smoke exposure, obesity, prematurity) and the protective factors (i.e., probiotics, breastfeeding, vitamin D, influenza vaccination, non-specific immunomodulators) associated with the development of the disease in the young child. A multidisciplinary panel of experts from the Emilia-Romagna Region, Italy, addressed twelve key questions regarding managing preschool wheezing. Clinical questions have been formulated by the expert panel using the PICO format (Patients, Intervention, Comparison, Outcomes). Systematic reviews have been conducted on PubMed to answer these specific questions and formulate recommendations. The GRADE approach has been used for each selected paper to assess the quality of the evidence and the degree of recommendations. Based on a panel of experts and extensive updated literature, this consensus document provides insight into the pathogenesis, risk and protective factors associated with the development and persistence of preschool wheezing. Undoubtedly, more research is needed to improve our understanding of the disease and confirm the associations between certain factors and the risk of wheezing in early life. In addition, preventive strategies must be promoted to avoid children's exposure to risk factors that may permanently affect respiratory health.

15.
Antibiotics (Basel) ; 11(8)2022 Aug 18.
Artigo em Inglês | MEDLINE | ID: mdl-36009990

RESUMO

Urinary tract infection (UTI) is one of the most common infectious diseases in the pediatric population and represents a major cause of antibiotic consumption and hospitalization in children. Considering the ongoing controversies on the management of pediatric UTI and the challenges due to increasing antimicrobial resistance, the aim of the present study was to evaluate the level of agreement on UTI management in pediatric age in Emilia-Romagna Region, Italy, and to assess on the basis of recent studies whether there is the need to change current recommendations used by primary care pediatricians, hospital pediatricians, and pediatric surgeons in everyday clinical practice to possibly improve outcomes. This consensus provides clear and shared indications on UTI management in pediatric age, based on the most updated literature. This work represents, in our opinion, the most complete and up-to-date collection of statements on procedures to follow for pediatric UTI, in order to guide physicians in the management of the patient, standardize approaches, and avoid abuse and misuse of antibiotics. Undoubtedly, more randomized and controlled trials are needed in the pediatric population to better define the best therapeutic management in cases with antimicrobial resistance and real usefulness of long-term antibiotic prophylaxis.

16.
J Clin Med ; 11(16)2022 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-36013002

RESUMO

Preschool wheezing should be considered an umbrella term for distinctive diseases with different observable and measurable phenotypes. Despite many efforts, there is a large gap in knowledge regarding management of preschool wheezing. In order to fill this lack of knowledge, the aim of these guidelines was to define management of wheezing disorders in preschool children (aged up to 5 years). A multidisciplinary panel of experts of the Emilia-Romagna Region, Italy, addressed twelve different key questions regarding the management of preschool wheezing. Clinical questions have been formulated by the expert panel using the PICO format (Patients, Intervention, Comparison, Outcomes) and systematic reviews have been conducted on PubMed to answer these specific questions, with the aim of formulating recommendations. The GRADE approach has been used for each selected paper, to assess the quality of the evidence and the degree of recommendations. These guidelines represent, in our opinion, the most complete and up-to-date collection of recommendations on preschool wheezing to guide pediatricians in the management of their patients, standardizing approaches. Undoubtedly, more research is needed to find objective biomarkers and understand underlying mechanisms to assess phenotype and endotype and to personalize targeted treatment.

17.
Acta Biomed ; 92(S1): e2021239, 2022 03 08.
Artigo em Inglês | MEDLINE | ID: mdl-35261392

RESUMO

BACKGROUND AND AIM: Hashimoto's thyroiditis (HT) is a common endocrinopathy in children, particularly in females. Clinical overt presentation of hypothyroidism in HT includes mild to very severe forms, characterised by impairment of many body functions and organs, such as heart, brain, muscles, ovaries and liver. CASE: we report the case of a 14-year-old girl, with severe hypothyroidism due to a late diagnosis of HT during the Covid-19 pandemic. Routine biochemical and hormonal exams were carried out at presentation. Moderate pericardial effusion was detected by echocardiography and polycystic ovarian morphology (PCOM) was found on the pelvic ultrasound. Furthermore, high levels of creatine phosphokinase (CPK), Lactic Acid Dehydrogenase (LDH) and hepatic liver enzymes, associated with muscular pseudohypertrophy and bilateral weakness of the lower limbs, were suggestive of a rare presentation of long-standing hypothyroidism defined Kocher-Debre-Semelaigne syndrome (KDSS). Levothyroxine replacement therapy was started immediately, leading to a rapid improvement of symptoms and a progressive normalization of the biochemical parameters. Due to persistent lower limb weakness, further neurological investigations were performed, showing bilateral peripheral polyneuropathy (PNP), ascribable to the longstanding and severe hypothyroidism. A pelvic ultrasound, performed after thyroid hormones had normalised and menses had turned to be regular, showed normal ovarian features supporting the hypothesis of the Van Wyk and Grumbach syndrome in a post-menarcheal girl. CONCLUSIONS: although clinical manifestation of hypothyroidism are usually mild, more severe and rare presentations such as ovarian dysfunction and myopathy are possible, particularly if the diagnosis is delayed and replacement therapy is not promptly administered.


Assuntos
COVID-19 , Hipotireoidismo Congênito , Adolescente , Criança , Controle de Doenças Transmissíveis , Diagnóstico Tardio , Feminino , Seguimentos , Humanos , Pandemias , SARS-CoV-2
18.
Mol Genet Metab Rep ; 30: 100833, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-35242567

RESUMO

Wolman Disease (WD) is a severe multi-system metabolic disease due to lysosomal acid lipase (LAL) deficiency. We report on a WD infant who developed an unusual hemophagocytic lymphohistiocytosis (HLH) phenotype related to WD treated with sebelipase alfa. A male baby came to our attention at six months of life for respiratory insufficiency and sepsis, abdominal distension, severe hepatosplenomegaly, diarrhea, and severe growth retardation. HLH was diagnosed and treated with intravenous immunoglobulin, steroids, cyclosporine, broad-spectrum antimicrobial therapy, and finally with the anti-IL-6 drug tocilizumab. WD was suspected for the presence of adrenal calcifications and it was confirmed by LAL enzyme activity and by molecular analysis of LIPA. Plasma oxysterols cholestan-3ß,5α,6ß-triol (C-triol), and 7-ketocholesterol (7-KC) were markedly increased. Sebelipase alfa was started with progressive amelioration of biochemical and clinical features. The child died from sepsis, 2 months after sebelipase discontinuation requested by parents. Our case shows the importance of an early diagnosis of WD and confirms the difficulty to reach a diagnosis in the HLH phenotype. Sebelipase alpha is an effective treatment for LAL deficiency, also in children affected by WD. Further data are necessary to confirm the utility of measuring plasma c-triol as a biochemical marker of the disease.

19.
Life (Basel) ; 12(2)2022 Jan 19.
Artigo em Inglês | MEDLINE | ID: mdl-35207430

RESUMO

Headache is the most frequent neurological symptom in childhood and the main reason for admission to pediatric emergency departments. The aim of this consensus document is to define a shared clinical pathway between primary care pediatricians (PCP) and hospitals for the management of children presenting with headache. For the purposes of the study, a group of hospital pediatricians and a group of PCP from the Emilia Romagna's health districts were selected to achieve consensus using the RAND/UCLA appropriateness method. Thirty-nine clinical scenarios were developed: for each scenario, participants were asked to rank the appropriateness of each option from 1 to 9. Agreement was reached if ≥75% of participants ranked within the same range of appropriateness. The answers, results, and discussion helped to define the appropriateness of procedures with a low level of evidence regarding different steps of the diagnostic-therapeutic process: primary care evaluation, emergency department evaluation, hospital admission, acute therapy, prophylaxis, and follow-up. The RAND proved to be a valid method to value appropriateness of procedures and define a diagnostic-therapeutic pathway suitable to the local reality in the management of pediatric headache. From our results, some useful recommendations were developed for optimizing the healthcare professionals' network among primary care services and hospitals.

20.
Nat Commun ; 13(1): 537, 2022 Jan 27.
Artigo em Inglês | MEDLINE | ID: mdl-35087033

RESUMO

Fermi level pinning in doped metal oxide (MO) nanocrystals (NCs) results in the formation of depletion layers, which affect their optical and electronic properties, and ultimately their application in smart optoelectronics, photocatalysis, or energy storage. For a precise control over functionality, it is important to understand and control their electronic bands at the nanoscale. Here, we show that depletion layer engineering allows designing the energetic band profiles and predicting the optoelectronic properties of MO NCs. This is achieved by shell thickness tuning of core-shell Sn:In2O3-In2O3 NCs, resulting in multiple band bending and multi-modal plasmonic response. We identify the modification of the band profiles after the light-induced accumulation of extra electrons as the main mechanism of photodoping and enhance the charge storage capability up to hundreds of electrons per NC through depletion layer engineering. Our experimental results are supported by theoretical models and are transferable to other core-multishell systems as well.

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