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Further research is warranted to explore the impact of novel pharmaceuticals on quality of life for children with SMA longitudinally to optimize clinical and psychosocial outcomes.
Robotic surgery is currently a reality in surgical practice, and many endeavors have been made to extend its application also in pediatric surgery. In the absence of easy access to a robotic surgical system, new devices have been developed to offer a valid alternative such as wristed instruments. These differ from conventional laparoscopic instruments owing to a wrist-like mechanism at the tip, which faithfully reproduces the movements of the surgeon's hands, regaining more movement's degrees; Methods We present two case reports in which the patients were subjected to minimally invasive procedures with aid of wristed instruments, followed by a review of the literature regarding the devices commonly marketed; Results Articulated or wristed instruments render the same features seen in robotic surgery, such as major dexterity in smaller spaces, restitution of more natural movements and the ability to get over obstacles in a direct visual line. Our center recently equipped with ArtiSential
articulated instrum surgery, such as major dexterity in smaller spaces, restitution of more natural movements and the ability to get over obstacles in a direct visual line. Our center recently equipped with ArtiSential® articulated instruments and so far, they have proven to be of great value; Conclusions wristed instruments could represent a standpoint for surgeons wanting to benefit from the advantages of robotic surgery with a cost-sensitive perspective.
Pediatric palliative care (PPC) is characterized by years of multisectoral and multi-professional care. Sharing information between PPC professionals is, therefore, essential for quality care. The evidence shows that electronic cross-facility health records (ECHRs) provide useful support in this context. To our knowledge, no ECHRs have been developed through a user-centered approach for this specific setting in Germany.
Guided by design thinking, first, qualitative interviews were conducted to assess the needs of PPC professionals. Second, the elicited needs were specified in focus groups (FGs). Based on the needs stated in the interviews, prototypes of the ECHR were developed and discussed in the FGs. The indicated needs were supplemented and specified in an iterative process. The prototypes were further adapted according to these results. The unified theory of acceptance and use of technology was the basic model in the evaluation of needs.
Across seven main categories, past and current medication, emergency view, and messaging functions were identified as the participants' desired core components of an ECHR. Utilizing design thinking facilitated the explicit articulation of user needs.
Developing an ECHR with the content identified would allow for real-time data during emergencies, tracking what other PPC professionals have done, and making the applied treatments visible to others. This would offer a broader picture of the complex conditions common to PPC.
Developing an ECHR with the content identified would allow for real-time data during emergencies, tracking what other PPC professionals have done, and making the applied treatments visible to others. This would offer a broader picture of the complex conditions common to PPC.In this study, two different approaches were applied in the analysis of the GAA gene. One was analyzed based on patients with Pompe disease, and the other was analyzed based on GAA genomic data from unaffected carriers in a general population genetic database. For this, GAA variants in Korean and Japanese patients reported in previous studies and in patients reported in the Pompe disease GAA variant database were analyzed as a model. In addition, GAA variants in the Korean Reference Genome Database (KRGDB), the Japanese Multi Omics Reference Panel (jMorp), and the Genome Aggregation Database (gnomAD) were analyzed. Overall, approximately 50% of the pathogenic or likely pathogenic variants (PLPVs) found in unaffected carriers were also found in real patients with Pompe disease (Koreans, 57.1%; Japanese, 46.2%). In addition, there was a moderate positive correlation (Spearman's correlation coefficient of 0.45-0.69) between the proportion of certain PLPVs in patients and the minor allele frequency of their variants in a general population database. Based on the analysis of general population databases, the total carrier frequency for Pompe disease in Koreans and Japanese was estimated to be 1.7% and 0.7%, respectively, and the predicted genetic prevalence was 113,657 and 178,013, respectively.
ABO-incompatible (ABOi) living donor liver transplantation (LDLT) has been proposed to compensate for donor shortage. To date, few studies have reported detailed ABOi LDLT results in large series of pediatric patients. C4d complement deposition in graft capillaries has been reported to be associated with antibody-mediated rejection in solid organ transplantation.
A retrospective case-control study was conducted, comparing clinical outcomes of each of 34 consecutive pediatric ABOi LDLT recipients with those of 2 non-ABOi pairs (
= 68), matched according to pre-transplant diagnostic criteria, age, and date of transplantation. In addition, we studied the C4d immunostaining pattern in 22 ABOi and in 36 non-ABOi recipients whose liver biopsy was performed within the first 4 post-transplant weeks for suspected acute rejection.
The incidence of biliary complications was higher in ABOi recipients (
< 0.05), as were the incidence of acute humoral rejection (
< 0.01) and the incidence of retransplantation (
< 0.05). All children who required retransplantation were older than 1 year at the time of ABOi LDLT. Positive C4d immunostaining was observed in 13/22 (59%) ABOi recipients versus 3/36 (8.3%) non-ABOi recipients (
< 0.0001).
ABOi LDLT is a feasible option for pediatric end-stage liver disease but carries increased risks for the recipient, especially for children older than 1 year, even with a specific preparation protocol. C4d immunostaining may be a hallmark of acute humoral rejection in ABOi liver transplantation.
ABOi LDLT is a feasible option for pediatric end-stage liver disease but carries increased risks for the recipient, especially for children older than 1 year, even with a specific preparation protocol. C4d immunostaining may be a hallmark of acute humoral rejection in ABOi liver transplantation.Group A streptococcus (GAS) is an important cause of acute pharyngitis. We investigated the positive rate of GAS tests and clinical viral features in children with acute pharyngitis. A retrospective review was conducted for patients less then 15 years old with both rapid antigen detection test (RADT) and throat culture results. Patients were excluded if they were diagnosed with influenza or had received antibiotics within two weeks before these tests. A total of 377 patients were eligible. The median age of patients was 3.5 years, and 45.4% of total patients were less then 3 years old. Among all patients, 68.7% had at least one viral feature, and 39% had more than two. The overall positiv rate for GAS was 11.4%. The GAS positive rate was significantly lower in patients less then 3 years old than in older patients (1.8% vs. 19.4%, p less then 0.0001). The overall sensitivity and specificity of RADT were 75.0% (95% CI 57.8-87.9) and 97.9% (95% CI 95.8-99.2), respectively. The GAS positive rate was not significantly different between patients with and without viral features (12.4% vs. 9.3%, p = 0.4854). check details In patients aged 3-14 years, the GAS positive rate was not associated with the modified Centor score or the frequency of clinical viral features. Despite a low prevalence of GAS pharyngitis, testing for GAS was frequently performed in children less then 3 years old in this study. Appropriate use of laboratory testing for GAS pharyngitis and judicious prescription of antibiotics were imperative.
The purpose of this article is to update the diagnostic assessment, therapeutic approach, and 12-18 month follow-up of patients added to the Italian Lombardy Attention Deficit Hyperactivity Disorder (ADHD) Register.
Medical records of patients added to the Registry from 2011 to 2021 were analysed.
4091 of 5934 patients met the criteria for a diagnosis of ADHD, and 20.3% of them presented a familiarity with the disorder. A total of 2879 children (70.4%) had at least one comorbidity disorder, in prevalence a learning disorder (39%). Nearly all (95.9%) received at least one psychological prescription, 17.9% of them almost one pharmacological treatment, and 15.6% a combination of both. Values of ≥5 of the Clinical Global Impression-Severity scale (CGI-S) are more commonly presented by patients with a pharmacological prescription than with a psychological treatment (
< 0.0001). A significant improvement was reported in half of the patients followed after 1 year, with Clinical Global Impression-Improvement scale (CGI-I) ≤ 3. In all, 233 of 4091 are 18-year-old patients.
A ten-year systematic monitoring of models of care was a fruitful shared and collaborative initiative in order to promote significant improvement in clinical practice, providing effective and continuous quality of care. The unique experience reported here should spread.
A ten-year systematic monitoring of models of care was a fruitful shared and collaborative initiative in order to promote significant improvement in clinical practice, providing effective and continuous quality of care. The unique experience reported here should spread.Urinary tract infections (UTIs) are among the most common bacterial infections in children, and Escherichia coli is the main pathogen responsible. Several guidelines, including the recently updated Italian guidelines, recommend amoxicillin-clavulanic acid (AMC) as a first-line antibiotic therapy in children with febrile UTIs. Given the current increasing rates of antibiotic resistance worldwide, this study aimed to investigate the three-year trend in the resistance rate of E. coli isolated from pediatric urine cultures (UCs) in a metropolitan area of northern Italy. We conducted a retrospective review of E. coli-positive, non-repetitive UCs collected in children aged from 1 month to 14 years, regardless of a diagnosis of UTI, catheter colonization, urine contamination, or asymptomatic bacteriuria. During the study period, the rate of resistance to AMC significantly increased from 17.6% to 40.2% (p less then 0.001). Ciprofloxacin doubled its resistance rate from 9.1% to 16.3% (p = 0.007). The prevalence of multidrug-resistant E. coli rose from 3.9% to 9.2% (p = 0.015). The rate of resistance to other considered antibiotics remained stable, as did the prevalence of extended spectrum beta-lactamases and extensively resistant E. coli among isolates. These findings call into question the use of AMC as a first-line therapy for pediatric UTIs in our population, despite the indications of recent Italian guidelines.
Beside the core symptoms, patients with attention-deficit/hyperactivity disorder (ADHD) frequently show relevant difficulty in developing relationships with peers. Although ADHD symptoms may account for social impairment, deficits in cognitive and/or affective empathy have also been involved. Our aim was to investigate the effect of methylphenidate (MPH) treatment on affective and cognitive empathy.
Sixty-one drug-naïve youths with ADHD (age range 6 to 17 years, mean 10.3 ± 2.8 years, 51 males) naturalistically treated with MPH monotherapy were followed up for 6 months for ADHD symptoms and empathy, measured with the Basic Empathy Scale.
After being treated with MPH, the patients showed a significant improvement in affective and cognitive empathy scores. Linear regression models showed that changes in inattention symptoms predicted changes in affective but not in cognitive empathy, while changes in the hyperactivity/impulsivity symptoms did not predict changes in affective or cognitive empathy.
Our study provides a further contribution for a better understanding of the possible effects of the MPH on youth's characteristics.
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