Editorial

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3 pages, 176 KiB

Open AccessEditorial

Non-Communicable Diseases and Rare Diseases: A Current and Future Public Health Challenge within Pediatrics

by

Valeria Calcaterra

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Gianvincenzo Zuccotti

Children 2022, 9(10), 1491; https://doi.org/10.3390/children9101491 - 28 Sep 2022

Cited by 2 | Viewed by 1163

Abstract

The global burden of non-communicable diseases (NCDs) and rare diseases constitutes a current and future public health challenge within pediatrics [...] Full article

(This article belongs to the Special Issue Non-communicable Diseases and Rare Diseases in Pediatrics: Management, Treatment and Prevention Strategies)

Research

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10 pages, 241 KiB

Open AccessEditor’s ChoiceArticle

Anorectal Malformations: Ideal Surgery Timing to Reduce Incontinence and Optimize QoL

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Gloria Pelizzo

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Carlotta Paola Maria Canonica

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Giorgio Giuseppe Orlando Selvaggio

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Eleonora Durante

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Gianvincenzo Zuccotti

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Valeria Calcaterra

Children 2023, 10(2), 404; https://doi.org/10.3390/children10020404 - 18 Feb 2023

Cited by 2 | Viewed by 1336

Abstract

Anorectal malformations (ARMs) are rare and involve a wide spectrum of malformations. Prenatal diagnosis is often incomplete, and the diagnostic pathway is started during the newborn period to identify the type of malformation and the correct treatment. This retrospective study included patients between [...] Read more.

Anorectal malformations (ARMs) are rare and involve a wide spectrum of malformations. Prenatal diagnosis is often incomplete, and the diagnostic pathway is started during the newborn period to identify the type of malformation and the correct treatment. This retrospective study included patients between 8 and 18 y.o. diagnosed with ARM, referring to Our Clinic. We proposed two questionnaires, Rintala Bowel Function Score and the Fecal Incontinence Quality of Life Scale, and we defined four groups referring to surgical timing (age in months < 3, 3–6, 6–9, >9). In total, 74 patients were recruited (mean age 13.05 ± 2.80 y.o.), and data analysis showed a significant relationship between comorbidity and surgical timing. Moreover, timing was related to outcome in terms of fecal continence (better if surgery performed before 3 months) and Quality of Life (QoL). QoL, however, is influenced by other factors (emotional and social life, psychological sphere and take of care of chronic disease). We considered rehabilitation programs, more often practiced by children who underwent surgery after 9 months, to maintain an appropriate relational life. This study highlights the importance of surgical timing as the first step of a multidisciplinary follow-up, taking care of the child in every phase of his growth, tailored to the single patient. Full article

(This article belongs to the Special Issue Non-communicable Diseases and Rare Diseases in Pediatrics: Management, Treatment and Prevention Strategies)

11 pages, 881 KiB

Open AccessArticle

Bioelectrical Impedance Analysis-Derived Phase Angle and Body Composition Are Predictors of Health-Related Fitness in Children and Adolescents with Obesity

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Giada Ballarin

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Maria Rosaria Licenziati

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Children 2022, 9(12), 1943; https://doi.org/10.3390/children9121943 - 11 Dec 2022

Cited by 3 | Viewed by 1103

Abstract

There is little evidence in children and adolescents with obesity of the relationships between muscle strength/cardiorespiratory fitness (both components of health-related fitness = HRF) and body composition. Body composition and HRF were studied in 281 children and adolescents with obesity to explore their [...] Read more.

There is little evidence in children and adolescents with obesity of the relationships between muscle strength/cardiorespiratory fitness (both components of health-related fitness = HRF) and body composition. Body composition and HRF were studied in 281 children and adolescents with obesity to explore their mutual relationship and to identify the predictors of HRF. By performing a bioelectrical impedance analysis (BIA), the fat-free mass (FFM) and percentage of body fat (%BF) were calculated, and the phase angle (PhA) was recorded. Handgrip strength (HGS), the standard broad jump (SBJ), and five broad jumps (FIVEBJ) were considered for the assessment of muscle strength, and the six-minute walking distance (SIXMWD) for cardiorespiratory fitness. The BMI Z-score was slightly higher in boys, and the %BF was higher in girls, with no difference in the FFM. HGS, the SBJ, and FIVEBJ were greater in the male sex. After controlling for sex, HGS was associated with the FFM, and with height, weight, and absolute BMI. On the contrary, the SBJ and FIVEBJ were negatively associated with adiposity, with a weak relationship with the FFM. The SIXMWD was only poorly related to height, the BMI Z-score, and the waist-to-height ratio. These results were confirmed with a multiple regression analysis. HGS, the SBJ, and FIVEBJ were higher in the first compared to the third tertile of the PhA in both sexes. The PhA also remained a consistent predictor of HGS, the SBJ, and FIVEBJ in a multiple regression analysis. In conclusion, the following predictors have been identified for HRF: the FFM for the isometric strength of the upper limbs and adiposity indicators for the SBJ and FIVEBJ. The PhA emerged as a proxy index of muscle strength. Full article

(This article belongs to the Special Issue Non-communicable Diseases and Rare Diseases in Pediatrics: Management, Treatment and Prevention Strategies)

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12 pages, 335 KiB

Open AccessArticle

Shedding Light on the Lifestyle and Participation of Portuguese Adolescents with Chronic Conditions—Data from the HBSC 2018 Study

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Margarida Gaspar de Matos

Children 2022, 9(11), 1717; https://doi.org/10.3390/children9111717 - 09 Nov 2022

Viewed by 1363

Abstract

Variables associated with lifestyle can constitute either risk or protective factors for the development and progression of chronic conditions (CC). This study intends to explore the differences between adolescents with and without CC and between adolescents whose school participation is affected/not affected by [...] Read more.

Variables associated with lifestyle can constitute either risk or protective factors for the development and progression of chronic conditions (CC). This study intends to explore the differences between adolescents with and without CC and between adolescents whose school participation is affected/not affected by the existing CC with regard to variables related to lifestyle (i.e., sleep, physical activity, BMI, and leisure). In addition, it also intends to analyze the influence of these variables (i.e., CC and lifestyle) regarding the adolescents’ quality of life (QoL). This work is part of the Portuguese HBSC 2018 study. A total of 8215 adolescents participated (52.7% female), with an average age of 14.36 years (SD = 2.28). The results showed that the adolescents with CC and whose school attendance and participation are affected by their CC exhibit more sleep difficulties (i.e., they experience lower sleep quality and have a higher degree of sleepiness), higher BMI levels (i.e., higher values of overweight and obesity), less participation in leisure activities, and a lower perception of QoL. A higher perception of QoL is associated with school participation unaffected by the existing CC, sleeping well, a low level of sleepiness, a more frequent practice of physical activity, a lower BMI, and a greater involvement in leisure activities. Adolescent health and well-being are a prominent issue in terms of public policies, with behavior and lifestyle playing a significant role in this domain. This message needs to be reinforced in regard to families, educators, healthcare professionals, and public sector policies, particularly concerning students with CC. Full article

(This article belongs to the Special Issue Non-communicable Diseases and Rare Diseases in Pediatrics: Management, Treatment and Prevention Strategies)

20 pages, 5068 KiB

Open AccessArticle

Thyroid Function and Metabolic Syndrome in Children and Adolescents with Neuromotor Disability

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Gianvincenzo Zuccotti

Children 2022, 9(10), 1531; https://doi.org/10.3390/children9101531 - 06 Oct 2022

Cited by 6 | Viewed by 1230

Abstract

Thyroid function plays a crucial role in nervous system integrity and metabolic homeostasis. We evaluated the pattern of TSH, FT4 and FT3 release in children with neuromotor impairment (NI) in relationship with metabolic syndrome (MS). We enrolled 55 patients with NI and 30 [...] Read more.

Thyroid function plays a crucial role in nervous system integrity and metabolic homeostasis. We evaluated the pattern of TSH, FT4 and FT3 release in children with neuromotor impairment (NI) in relationship with metabolic syndrome (MS). We enrolled 55 patients with NI and 30 controls. Clinical parameters, thyroid function and MS presence were recorded. Principal component analysis (PCA), cluster analysis, and logistic regression models were performed. MS was detected in 54.5% of patients. Four clusters were identified: the first one included only controls and, contrasting with cluster 4, was exclusively characterized by children with disability and MS. This latter showed increased FT4 and FT3 and decreased TSH levels. Cluster 2, characterized by disability without MS showed high FT4 and FT3, whereas cluster 3 with low FT4 and FT3 mainly included disability (90%) and showed prevalent MS (57%). The association between TSH and NI is represented by a U-shape structure. The TSH, FT3 and FT4 release patterns may reflect thyrotropic adaptation, allostatic response and compensatory mechanisms. These mechanisms, found in both MS and disability, show that the odds of having a condition of NI with or without MS increase as the TSH values deviate, in both directions, from a value of 2.5 mLU/mL. Full article

(This article belongs to the Special Issue Non-communicable Diseases and Rare Diseases in Pediatrics: Management, Treatment and Prevention Strategies)

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10 pages, 248 KiB

Open AccessArticle

Utility of Functional Lumen Imaging Probe in Long-Term Follow-Up of Children with Esophageal Atresia: A Single-Center Retrospective Study

by

Francesca Destro

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Sara Costanzo

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Eleonora Durante

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Maria Sole Carcassola

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Children 2022, 9(10), 1426; https://doi.org/10.3390/children9101426 - 20 Sep 2022

Cited by 1 | Viewed by 913

Abstract

Long term follow-up of patients with esophageal atresia (EA) may be hampered by esophageal dysmotility, which affects quality of life and might lead to late complications. The endoluminal functional lumen imaging probe (EndoFlip™ Medtronic, Crospon Inc.) is an innovative diagnostic tool that assesses [...] Read more.

Long term follow-up of patients with esophageal atresia (EA) may be hampered by esophageal dysmotility, which affects quality of life and might lead to late complications. The endoluminal functional lumen imaging probe (EndoFlip™ Medtronic, Crospon Inc.) is an innovative diagnostic tool that assesses esophageal distensibility. Our aim was to report the use of EndoFlip™ in an EA follow-up, in order to describe distensibility patterns and to determine its possible role for functional evaluation of patients. We retrospectively collected data of EA patients, with a minimum follow-up of 9 years, who required endoscopic evaluation and underwent EndoFlip™. An adaptation of the Medtronic EF-322 protocol was applied and distensibility data were compared to those reported by Pandolfino et al. Nine patients (median age 13 years) were included in the study. The median minimum distensibility was 2.58 mm²/mmHg. Signs of peristalsis were observed in three patients. In one case, the esophagogastric junction (EGJ) after Toupet fundoplication showed low distensibility. EGJ distensibility values of 2.58 mm²/mmHg (median) confirmed both good esophagogastric continence and compliance. Esophagitis and absent peristalsis were found in one patient together with partial stenosis of the fundoplication, confirming the importance of surgical adaptation. Esophageal body distensibility was higher than that of the EGJ. Considering the presence of symptoms, the EndoFlip™ results seem to correlate better with the clinical picture. EndoFlip™ use was safe and feasible in children. It allowed for the measurement of esophageal distensibility and diameter and the acquisition of indirect information on motility with clinical implications. The routine use of EndoFlip™ could be part of EA follow-up, although considerable research is needed to correlate Endoflip™ system measurements to EA patient outcomes. Full article

(This article belongs to the Special Issue Non-communicable Diseases and Rare Diseases in Pediatrics: Management, Treatment and Prevention Strategies)

Review

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19 pages, 808 KiB

Open AccessReview

Prader–Willi Syndrome and Weight Gain Control: From Prevention to Surgery—A Narrative Review

by

Valeria Calcaterra

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Vittoria Carlotta Magenes

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Francesca Destro

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Paola Baldassarre

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Giustino Simone Silvestro

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Gianvincenzo Zuccotti

Children 2023, 10(3), 564; https://doi.org/10.3390/children10030564 - 16 Mar 2023

Cited by 1 | Viewed by 1210

Abstract

Severe obesity remains one of the most important symptoms of Prader–Willi Syndrome (PWS), and controlling weight represents a crucial point in the therapeutical approach to the syndrome. We present an overview of different progressive patterns of growth that involve controlling weight in PWS. [...] Read more.

Severe obesity remains one of the most important symptoms of Prader–Willi Syndrome (PWS), and controlling weight represents a crucial point in the therapeutical approach to the syndrome. We present an overview of different progressive patterns of growth that involve controlling weight in PWS. Mechanisms involved in the development of obesity and in preventive and therapeutic strategies to control weight gain are discussed. Early diagnosis, a controlled diet regimen, regular physical activity, follow-up by multidisciplinary teams, and hormonal treatment improved the management of excessive weight gain. In selected cases, a surgical approach can be also considered. Controlling weight in PWS remains a challenge for pediatricians. The importance of consulting different healthcare specialists, starting from the neonatal and pediatric age, is also considered as a crucial approach to controlling weight, as well as to limiting and preventing the onset of obesity and its complications. Full article

(This article belongs to the Special Issue Non-communicable Diseases and Rare Diseases in Pediatrics: Management, Treatment and Prevention Strategies)

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Other

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8 pages, 2248 KiB

Open AccessCase Report

Atrioventricular Block in Celiac Disease: An Unusual Clinical Presentation in a Child. A Case-Based Review

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Gianvincenzo Zuccotti

Children 2022, 9(11), 1627; https://doi.org/10.3390/children9111627 - 26 Oct 2022

Cited by 2 | Viewed by 926

Abstract

Congenital or acquired atrioventricular block (AVB) is a rare disorder in the pediatric population, while celiac disease (CeD) is a common multisystemic autoimmune disorder that is characterized by intestinal manifestations as they are the typical clinical presentation. Sometimes CeD presents more complex multisystemic [...] Read more.

Congenital or acquired atrioventricular block (AVB) is a rare disorder in the pediatric population, while celiac disease (CeD) is a common multisystemic autoimmune disorder that is characterized by intestinal manifestations as they are the typical clinical presentation. Sometimes CeD presents more complex multisystemic involvement which includes the heart. Cardiac involvement, such as dilated cardiomyopathy, myocarditis or conduction disease, have been mainly described in untreated adult patients with or without gastro-intestinal symptoms; rare cases of AVB and CeD have been also reported, particularly in association with extra-cardiac manifestations. We describe a case of a progressive acquired AVB block in a 4-year-old child, in which CeD was later diagnosed. A rapid and significantly improvement of the AVB grade has been obtained after the child started a strict gluten-free diet, and so we suggest including diagnostic exams for CeD in all of the children with acquired AVB. Full article

(This article belongs to the Special Issue Non-communicable Diseases and Rare Diseases in Pediatrics: Management, Treatment and Prevention Strategies)

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