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Purpose of Review: This review aimed to summarize the evidence regarding the relationship between parental psychosocial (e.g., fear of hypoglycemia, stress and family conflict) and glycemic outcomes in children between the age of 1–10 years old. Recent Findings: Type 1 Diabetes (T1D) in young children can be very complex to manage for their parents since they are the main individuals responsible for T1D tasks. Also, parental psychological adjustment impacts children’s glycemic outcomes. Summary: This systematic review was performed following the PRISMA guidelines. The search process was conducted in four databases from 2019 to 2024. From a total of 215 studies, 5 were included. We identified five studies that found direct associations between parental psychosocial variables and children's glycemic outcomes. These findings suggest a unidirectional perspective, evidencing the need to examine the longitudinal interplay between these variables. In sum, promoting parental psychological interventions may be fundamental for enhancing children’s glycemic outcomes. [ABSTRACT FROM AUTHOR]

Implantable cardioverter-defibrillators (ICD) are increasingly being used among the pediatric population for indications of both primary and secondary prevention. There is limited long-term data on the outcomes of pediatric patients following ICD implantation. The aim of this study was to investigate the characteristics of this population, burden of appropriate and inappropriate shock and complication rate in a large tertiary pediatric medical center. Included were children under the age of 18 years who underwent ICD implantation and had clinical follow up at our center. Data were retrospectively collected between study period 2005–2020. Primary outcome was the incidence of ICD shock appropriate and inappropriate. Secondary outcome was defining our patient population characteristics. Our cohort included 51 patients who underwent ICD implantation. Mean age at implantation was 10.9 ± 4.7 years and average follow-up time was 67 months. Diagnoses of implanted patients were: 28 (55%) patients with syndromes with risk for sudden death, cardiomyopathy in 14 patients (27%) and congenital heart disease (CHD) in 9 patients (18%). Forty-two (82%) patients had an ICD implanted for secondary prevention after experiencing a life-threatening arrhythmia and 9 (18%) for primary prevention. An endocardial system was implanted in 39 (76%) patients and an epicardial systems in 12 (24%) patients. A total of 20 (39%) patients received appropriate shocks for ventricular fibrillation(VF). 5 patients received inappropriate shocks, 4 due to sinus tachycardia and 1 due to rapidly conducted atrial fibrillation. Those who received an inappropriate shock had a significantly shorter ICD-programmed VF detection cycle length compared to those who did not receive an inappropriate shock (320 ms versus 270 ms, p = 0.062). This single center study demonstrates a high rate of appropriate ICD shocks (39%) and a low rate of inappropriate ICD shocks. Accurate programming of ICD devices in the pediatric population is paramount to avoid inappropriate ICD shocks. [ABSTRACT FROM AUTHOR]

Background: Food allergy is the third most commonly diagnosed allergic disorder in children, particularly during infancy and preschool years. Its prevalence has been rising rapidly in recent years, making it a significant public health concern. This study aims to conduct a systematic review of the impact of childhood food allergies on quality of life. Methods: A comprehensive search of databases from the last five years was conducted following the PRISMA guidelines, resulting in the selection of 21 relevant articles. Results: The findings indicate that food allergies significantly reduce children's quality of life, with age, the number of allergens, and allergy severity being key determinants. Affected children experience higher levels of anxiety, depression, and reduced psychosocial functioning. Caregivers also face substantial burdens, including constant concerns over accidental exposure, limitations in social activities, financial strain, and increased work absences. In school settings, these children are more prone to teasing or bullying, highlighting the need for increased education and social awareness. Conclusions: Food allergies significantly impact the quality of life for both patients and their families, especially mothers, with worsening effects as children age, including heightened risks of bullying and social limitations, highlighting the need for education, psychological support, and resource allocation. [ABSTRACT FROM AUTHOR]

Children of any age are at high risk for ocular chemical injuries and the development of limbal stem cell deficiency with severe visual consequences. Surgical options for ocular surface reconstruction at the pediatric age tend to have lower success rates than their adult counterparts. Herein, we report the results of a novel modification of the simple limbal stem cell epithelial transplantation using a doughnut-shaped amniotic membrane graft to avoid further visual axis obstruction during re-epithelization. Since younger children are a highly susceptible population suffering from chemical burns, with nearly half of them being at preschool (first months of life to 5 years) ages, such a technique may be more appropriate to reduce the time of visual obstruction and obtain faster visual rehabilitation. [ABSTRACT FROM AUTHOR]

Primary hyperparathyroidism (PHPT) due to ectopic parathyroid adenoma is a rare case of hypercalcemia in the pediatric population. Herein, a rare case of PHPT due to ectopic intrathymic parathyroid adenoma was described in an asymptomatic 15-year-old girl who had incidental diagnosis based on laboratory abnormalities but experienced a 3-month postoperative course of persistently elevated parathyroid hormone (PTH) and hypercalcemia following the initial unsuccessful parathyroidectomy operation carried out in a non-parathyroid expert center. The curative surgical treatment was accomplished only after the patientwas reoperated with video-assisted thoracoscopic surgery (VATS) thymectomy by the surgeon experienced in parathyroid surgery with implementation of the combined imaging modalities for accurate localization of ectopic adenoma including 99mTc sestamibi (MIBI) plus neck and thoracic computed tomography (CT) and the appropriate surgical strategies including intraoperative intact PTH monitoring and frozen section diagnosis. Before the reoperation (VATS thymectomy), laboratory findings showed elevated PTH (1,171 ng/L; reference range: 21.80 ng/L-87.5 ng/L) and hypercalcemia (13.4 mg/dL; reference range: 8.4 mg/dL-10.2 mg/dL). The preoperative PTH levels were 94 ng/L at 5 min after thymectomy and 78 ng/L at 10 min. The PTH and calcium levels were 54.3 ng/L and 8.47 mg/dL, respectively, on postoperative day 1 and were 34.2 ng/L and 8.1 mg/dL on postoperative day 2. The patient was discharged on postoperative day 2 without any complications. In conclusion, our findings indicate the likelihood of isolated primary hyperparathyroidism to be incidentally diagnosed based solely on laboratory abnormalities with no specific clinical manifestations in the pediatric age. In addition, using combined imaging modalities (such as MIBI and CT) in accurate localization of ectopic parathyroid adenoma and implementation of surgery by experienced surgeons along with intraoperative intact PTH monitoring and frozen section diagnosis seem crucial to ensure the curative surgical treatment. [ABSTRACT FROM AUTHOR]

Hydatid cyst is a very common disease caused by Ecinococcusgranulosumlarva . It can involve nearly any part of the body but most commonly affected organs are liver and lungs. Ultrasound, Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) are valuable radiological investigations to diagnose hydatid cysts. Understanding of disease trend is important for early diagnosis and prevention of its com-plication to occur. We present a case series on a few interesting cases of hydatid cysts in paediatric age group in different organs and stages. [ABSTRACT FROM AUTHOR]

Primary adrenal insufficiency (PAI) is a rare medical condition, characterized by a deficiency in adrenal hormones. Although rare, PAI is a life-threatening disease requiring prompt recognition and treatment. However, symptoms of PAI are often non-specific and diagnosis can be challenging, causing frequent diagnostic delays. In adults, autoimmunity is the most common cause of PAI in industrialized countries, whereas in children, the most frequent etiology is represented by congenital defects of steroidogenesis and, in particular, by congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. A few recent case series from different countries have reported that autoimmunity is the second most common etiology of PAI in the pediatric age group. However, data on autoimmune PAI in children are still scant and the exact epidemiology, clinical manifestations, and long-term outcomes of this condition have yet to be defined. The scope of this review is to summarize the current knowledge on the etiology, presentation, and treatment of autoimmune PAI in childhood and to increase physicians' awareness of the signs that should raise an early suspicion of this condition. [ABSTRACT FROM AUTHOR]

Background and Objectives: Guillain-Barré syndrome (GBS) is the most frequent cause of acute flaccid paresis in children. The aim of this study was to describe the clinical and electrophysiological findings and outcomes of children with GBS diagnosed in our unit. Moreover, the literature on pediatric GBS cases from the past 5 years was reviewed. In this retrospective study, we reported data on 12 patients (9 male and 3 female patients; mean age: 5 y, 4 mo; range: 9 mo–11 y) clinically diagnosed at the Child Neurology Unit of the AUSL-IRCCS of Reggio Emilia, Italy, between 2000 and 2017 and a brief analysis/comparison with data from the literature. Materials and Methods: Data were collected from medical charts. Results: In our cohort, male patients were more frequent than female ones (9 vs. 3), and upper respiratory tract infection (n = 8, 66.7%) was the most frequent triggering factor. The main clinical symptoms on admission were distal lower limbs' weakness with gait difficulties (83.3%), pain (50%), upper limbs' weakness (50%), and dysphagia for liquids (25%). Peripheral neurophysiological studies revealed acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in 66.6% of the children, acute motor and sensory axonal neuropathy (AMSAN) in 25%, and acute motor axonal neuropathy (AMAN) in 8.3%. Ten individuals (83.3%) received timely treatment with intravenous immunoglobulins (IVIG), and, out of these ten patients, 58% received concomitant treatment with IV methylprednisolone because of a progressive disease course. Complete remission was observed in the majority of individuals (91.6%) within 6 months of symptom onset. Conclusions: Different subtypes of GBS can affect children; however, the outcome is usually positive. Early treatment appears to be important for a favorable outcome. [ABSTRACT FROM AUTHOR]

Thyroid nodules in pediatric patients are less common than in adults but show a higher malignancy rate. Accordingly, the management of thyroid nodules in pediatric patients is more complex the younger the patient is, needing careful evaluation by physicians. In adult patients, specific ultrasound (US) features have been associated with an increased risk of malignancy (ROM) in thyroid nodules. Moreover, several US risk stratification systems (RSSs) combining the US features of the nodule were built to define the ROM. RSSs are developed for the adult population and their use has not been fully validated in pediatric patients. This study aimed to evaluate the available data about US features of thyroid nodules in pediatric patients and to provide a summary of the evidence regarding the performance of RSS in predicting malignancy. Moreover, insights into the management of thyroid nodules in pediatric patients will be provided. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of the present study is to analyze thyroglossal duct cyst (TGDC) histopathological features, with focus on "arborization", in a cohort of pediatric patients who underwent surgical removal, and evaluate a possible correlation with clinical recurrences. Methods: A retrospective analysis of all patients who underwent surgical resection for TGDC at the division of Pediatric Surgery of the University of Pisa from 2015 to 2020 was performed; for each patient, the following data were recorded: age, sex, clinical presentation, localization, size of the lesion, diagnostic tools, histopathological features, perioperative complications, recurrence and follow-up. Results: With respect to arborization, following histopathological analysis 25/30 patients (83.3%) presented thyroglossal duct branching. After a median follow-up of 3.5 years, only 2 out of 30 patients (6.7%), one male and one female, respectively aged 4 y.o. and 6 y.o., presented recurrence within one year from first surgery. Conclusion: Surgery for TGDC remains a challenge for pediatric surgeons, while arborization was present in most of our cases which underwent surgery. With respect to the role of arborization, our study did not highlight sufficient conclusive data regarding their role in recurrence: instead, it showed wide resection as satisfactory, being the arborization present in most of the cases at histopathology. [ABSTRACT FROM AUTHOR]

Purpose: In recent years, copeptin stimulation through arginine administration has been evaluated as a new potential tool in the differential diagnosis of polyuria-polydipsia syndrome (PPS) in adults; to date very few data, all retrospective, exist in pediatric age. The aim of this prospective study is to evaluate the diagnostic performance of the arginine-stimulation test for copeptin in a cohort of pediatric patients affected by PPS. Methods: All children (<18 years) referred to the Department of Pediatric Endocrinology of the Regina Margherita Children Hospital for polyuria-polydipsia in the period January 2021–June 2023 were enrolled. The Arginine-stimulation test for copeptin was performed in all patients presenting PPS after water deprivation test (WDT). Patients with polyuria-polydipsia were then classified as having primary polyuria (PP), complete and partial central diabetes insipidus (CDI), according to the standardized interpretation. Arginine-stimulation test for copeptin was also performed in a control cohort. Results: A significant difference in arginine-stimulated copeptin values was observed at baseline (p = 0.005), at 60 min (p = 0.01), and at 90 min (p = 0.005) in 7 subjects presenting PP, 6 patients affected by CDI and 50 subjects of the control cohort. Plasma osmolality values remained stable at all measurements. The arginine-stimulated copeptin test demonstrated sensitivity and specificity of 100%, whereas the sensitivity of the WDT test was 83.3% and the specificity was 85.7%. Conclusion: Given the reliability and the minor adverse effects and costs, the copeptin level after arginine administration could replace the WDT in the diagnostic workup of these in pediatric age. [ABSTRACT FROM AUTHOR]

Primary hyperparathyroidism (PHPT) due to ectopic parathyroid adenoma is a rare case of hypercalcemia in the pediatric population. Herein, a rare case of PHPT due to ectopic intrathymic parathyroid adenoma was described in an asymptomatic 15-year-old girl who had incidental diagnosis based on laboratory abnormalities but experienced a 3-month postoperative course of persistently elevated parathyroid hormone (PTH) and hypercalcemia following the initial unsuccessful parathyroidectomy operation carried out in a non-parathyroid expert center. The curative surgical treatment was accomplished only after the patient was reoperated with video-assisted thoracoscopic surgery (VATS) thymectomy by the surgeon experienced in parathyroid surgery with implementation of the combined imaging modalities for accurate localization of ectopic adenoma including 99mTc sestamibi (MIBI) plus neck and thoracic computed tomography (CT) and the appropriate surgical strategies including intraoperative intact PTH monitoring and frozen section diagnosis. Before the reoperation (VATS thymectomy), laboratory findings showed elevated PTH (1,171 ng/L; reference range: 21.80 ng/L–87.5 ng/L) and hypercalcemia (13.4 mg/dL; reference range: 8.4 mg/dL–10.2 mg/dL). The preoperative PTH levels were 94 ng/L at 5 min after thymectomy and 78 ng/L at 10 min. The PTH and calcium levels were 54.3 ng/L and 8.47 mg/dL, respectively, on postoperative day 1 and were 34.2 ng/L and 8.1 mg/dL on postoperative day 2. The patient was discharged on postoperative day 2 without any complications. In conclusion, our findings indicate the likelihood of isolated primary hyperparathyroidism to be incidentally diagnosed based solely on laboratory abnormalities with no specific clinical manifestations in the pediatric age. In addition, using combined imaging modalities (such as MIBI and CT) in accurate localization of ectopic parathyroid adenoma and implementation of surgery by experienced surgeons along with intraoperative intact PTH monitoring and frozen section diagnosis seem crucial to ensure the curative surgical treatment.

Primary adrenal insufficiency (PAI) is a rare medical condition, characterized by a deficiency in adrenal hormones. Although rare, PAI is a life-threatening disease requiring prompt recognition and treatment. However, symptoms of PAI are often non-specific and diagnosis can be challenging, causing frequent diagnostic delays. In adults, autoimmunity is the most common cause of PAI in industrialized countries, whereas in children, the most frequent etiology is represented by congenital defects of steroidogenesis and, in particular, by congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. A few recent case series from different countries have reported that autoimmunity is the second most common etiology of PAI in the pediatric age group. However, data on autoimmune PAI in children are still scant and the exact epidemiology, clinical manifestations, and long-term outcomes of this condition have yet to be defined. The scope of this review is to summarize the current knowledge on the etiology, presentation, and treatment of autoimmune PAI in childhood and to increase physicians’ awareness of the signs that should raise an early suspicion of this condition.

Children of any age are at high risk for ocular chemical injuries and the development of limbal stem cell deficiency with severe visual consequences. Surgical options for ocular surface reconstruction at the pediatric age tend to have lower success rates than their adult counterparts. Herein, we report the results of a novel modification of the simple limbal stem cell epithelial transplantation using a doughnut-shaped amniotic membrane graft to avoid further visual axis obstruction during re-epithelization. Since younger children are a highly susceptible population suffering from chemical burns, with nearly half of them being at preschool (first months of life to 5 years) ages, such a technique may be more appropriate to reduce the time of visual obstruction and obtain faster visual rehabilitation.

Background: Food allergy is the third most commonly diagnosed allergic disorder in children, particularly during infancy and preschool years. Its prevalence has been rising rapidly in recent years, making it a significant public health concern. This study aims to conduct a systematic review of the impact of childhood food allergies on quality of life. Methods: A comprehensive search of databases from the last five years was conducted following the PRISMA guidelines, resulting in the selection of 21 relevant articles. Results: The findings indicate that food allergies significantly reduce children’s quality of life, with age, the number of allergens, and allergy severity being key determinants. Affected children experience higher levels of anxiety, depression, and reduced psychosocial functioning. Caregivers also face substantial burdens, including constant concerns over accidental exposure, limitations in social activities, financial strain, and increased work absences. In school settings, these children are more prone to teasing or bullying, highlighting the need for increased education and social awareness. Conclusions: Food allergies significantly impact the quality of life for both patients and their families, especially mothers, with worsening effects as children age, including heightened risks of bullying and social limitations, highlighting the need for education, psychological support, and resource allocation.

Introduction and objective: In the pediatric age group, vertigo is a less common condition compared to adults, likely underreported in prevalence. The aim of our study was to determine the prevalence of pediatric vertigo through literature review and meta-analysis. Method: Systematic searches were conducted in databases including PubMed, WoS, BVS, CARPHA, and EMBASE, following PRISMA guidelines. Epidemiological studies on vertigo in children aged 0 to 14 years were included. Bias risk was assessed using ROBINS-I scale. A qualitative analysis of vertigo causes from literature and a quantitative analysis of pediatric vertigo prevalence using SNS data up to April 2023 were performed. Statistical analysis and meta-analysis were conducted using JAMOVI, employing a 95% confidence interval and a randomeffects model. Results: Out of the 29 reviewed articles, 22 were analyzed, revealing a global prevalence of pediatric vertigo at 18.3% (95% CI: 5.1-31.5%). Diagnoses were categorized into three groups, with peripheral vertigo (66.74%) being prominent. Additionally, in Spain's SNS records, vertigo affected 0.72% of children under 14 years, with 4.77% of these cases belonging to the same age group. Discussion: Our study demonstrated that pediatric vertigo has a prevalence of 18.3%, with benign paroxysmal positional vertigo (BPPV) as the most frequent diagnosis. Although some studies show discrepancies in prevalence figures, our research emphasizes the complexity of diagnosing vertigo in children due to the diversity of etiologies. Conclusions: In our study, vertigo affects 18% of children under 14 years old, with peripheral vertigo being the main cause. Conditions like BPPV, Meniere's disease, and migraine are common. Due to study diversity, determining the exact prevalence is complex. These findings set a direction for future research. [ABSTRACT FROM AUTHOR]

Introduction: Multiple sclerosis (MS), a chronic inflammatory immune-mediated disease of the central nervous system (CNS), is a common condition in young adults, but it can also affect children. The aim of this study was to construct radiomic models of lesions based on magnetic resonance imaging (MRI, T2- weighted-Fluid-Attenuated Inversion Recovery), to understand the correlation between extracted radiomic features, brain and lesion volumetry, demographic, clinical and laboratorial data. Methods: The neuroimaging data extracted from eleven scans of pediatric MS patients were analyzed. A total of 60 radiomic features based on MR T2-FLAIR images were extracted and used to calculate gray level co-occurrence matrix (GLCM). The principal component analysis and ROC analysis were performed to select the radiomic features, respectively. The realized classification task by the logistic regression models was performed according to these radiomic features. Results: Ten most relevant features were selected from data extracted. The logistic regression applied to T2-FLAIR radiomic features revealed significant predictor for multiple sclerosis (MS) lesion detection. Only the variable "contrast" was statistically significant, indicating that only this variable played a significant role in the model. This approach enhances the classification of lesions from normal tissue. Discussion and conclusion: Our exploratory results suggest that the radiomic models based on MR imaging (T2-FLAIR) may have a potential contribution to characterization of brain tissues and classification of lesions in pediatric MS. [ABSTRACT FROM AUTHOR]

Abnormal wound healing causes keloid formation. Keloids are the most challenging conditions for the surgeons and dermatologists despite their high prevalence. Keloids are often symptomatic and can have a cosmetic nuisance as well as significant psychosocial burden for the patient. Although multiple treatment modalities exist, no single treatment has proven widely effective. In fact, recurrence following treatment is generally the normal. Combination therapy is likely the optimal strategy. In this case report, we highlight the clinical features of post-herniotomy keloid in children, pathophysiology, and management of keloids. [ABSTRACT FROM AUTHOR]

Background and Objectives: Guillain-Barré syndrome (GBS) is the most frequent cause of acute flaccid paresis in children. The aim of this study was to describe the clinical and electrophysiological findings and outcomes of children with GBS diagnosed in our unit. Moreover, the literature on pediatric GBS cases from the past 5 years was reviewed. In this retrospective study, we reported data on 12 patients (9 male and 3 female patients; mean age: 5 y, 4 mo; range: 9 mo–11 y) clinically diagnosed at the Child Neurology Unit of the AUSL-IRCCS of Reggio Emilia, Italy, between 2000 and 2017 and a brief analysis/comparison with data from the literature. Materials and Methods: Data were collected from medical charts. Results: In our cohort, male patients were more frequent than female ones (9 vs. 3), and upper respiratory tract infection (n = 8, 66.7%) was the most frequent triggering factor. The main clinical symptoms on admission were distal lower limbs’ weakness with gait difficulties (83.3%), pain (50%), upper limbs’ weakness (50%), and dysphagia for liquids (25%). Peripheral neurophysiological studies revealed acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in 66.6% of the children, acute motor and sensory axonal neuropathy (AMSAN) in 25%, and acute motor axonal neuropathy (AMAN) in 8.3%. Ten individuals (83.3%) received timely treatment with intravenous immunoglobulins (IVIG), and, out of these ten patients, 58% received concomitant treatment with IV methylprednisolone because of a progressive disease course. Complete remission was observed in the majority of individuals (91.6%) within 6 months of symptom onset. Conclusions: Different subtypes of GBS can affect children; however, the outcome is usually positive. Early treatment appears to be important for a favorable outcome.

Thyroid nodules in pediatric patients are less common than in adults but show a higher malignancy rate. Accordingly, the management of thyroid nodules in pediatric patients is more complex the younger the patient is, needing careful evaluation by physicians. In adult patients, specific ultrasound (US) features have been associated with an increased risk of malignancy (ROM) in thyroid nodules. Moreover, several US risk stratification systems (RSSs) combining the US features of the nodule were built to define the ROM. RSSs are developed for the adult population and their use has not been fully validated in pediatric patients. This study aimed to evaluate the available data about US features of thyroid nodules in pediatric patients and to provide a summary of the evidence regarding the performance of RSS in predicting malignancy. Moreover, insights into the management of thyroid nodules in pediatric patients will be provided.

IntroductionMultiple sclerosis (MS), a chronic inflammatory immune-mediated disease of the central nervous system (CNS), is a common condition in young adults, but it can also affect children. The aim of this study was to construct radiomic models of lesions based on magnetic resonance imaging (MRI, T2-weighted-Fluid-Attenuated Inversion Recovery), to understand the correlation between extracted radiomic features, brain and lesion volumetry, demographic, clinical and laboratorial data.MethodsThe neuroimaging data extracted from eleven scans of pediatric MS patients were analyzed. A total of 60 radiomic features based on MR T2-FLAIR images were extracted and used to calculate gray level co-occurrence matrix (GLCM). The principal component analysis and ROC analysis were performed to select the radiomic features, respectively. The realized classification task by the logistic regression models was performed according to these radiomic features.ResultsTen most relevant features were selected from data extracted. The logistic regression applied to T2-FLAIR radiomic features revealed significant predictor for multiple sclerosis (MS) lesion detection. Only the variable “contrast” was statistically significant, indicating that only this variable played a significant role in the model. This approach enhances the classification of lesions from normal tissue.Discussion and conclusionOur exploratory results suggest that the radiomic models based on MR imaging (T2-FLAIR) may have a potential contribution to characterization of brain tissues and classification of lesions in pediatric MS.

Abstract Background In patients with adrenal insufficiency (AI), adrenal crisis (AC) represents a clinical emergency. Early recognition and prompt management of AC or AC-risk conditions in the Emergency Department (ED) can reduce critical episodes and AC-related outcomes. The aim of the study is to report the clinical and biochemical characteristics of AC presentation to improve their timely recognition and proper management in a ED setting. Methods Single-centre, retrospective, observational study on pediatric patients followed at the Department of Pediatric Endocrinology of Regina Margherita Children’s Hospital of Turin for primary AI (PAI) and central AI (CAI). Results Among the 89 children followed for AI (44 PAI, 45 CAI), 35 patients (21 PAI, 14 CAI) referred to the PED, for a total of 77 accesses (44 in patients with PAI and 33 with CAI). The main causes of admission to the PED were gastroenteritis (59.7%), fever, hyporexia or asthenia (45.5%), neurological signs and respiratory disorders (33.8%). The mean sodium value at PED admission was 137.2 ± 1.23 mmol/l and 133.3 ± 1.46 mmol/l in PAI and CAI, respectively (p = 0.05). Steroids administration in PED was faster in patients with CAI than in those with PAI (2.75 ± 0.61 and 3.09 ± 1.47 h from PED access, p = 0.83). Significant factors related to the development of AC were signs of dehydration at admission (p = 0.027) and lack of intake or increase of usual steroid therapy at home (p = 0.059). Endocrinological consulting was requested in 69.2% of patients with AC and 48.4% of subjects without AC (p = 0.032). Conclusion children with AI may refer to the PED with an acute life-threatening condition that needs prompt recognition and management. These preliminary data indicate how critical the education of children and families with AI is to improve the management at home, and how fundamental the collaboration of the pediatric endocrinologist with all PED personnel is in raising awareness of early symptoms and signs of AC to anticipate the proper treatment and prevent or reduce the correlated serious events.

Anna Maria Testi, Maria Luisa Moleti, Alessia Angi, Simona Bianchi, Walter Barberi, Saveria Capria Department of Translational and Precision Medicine, Sapienza, University of Rome, Rome, ItalyCorrespondence: Anna Maria Testi, Hematology, Department of Translational and Precision Medicine, Sapienza University of Rome, Via Benevento 6, Rome, 00161, Italy, Tel +39-06-49974731, Fax +39-06-44241984, Email testi@bce.uniroma1.itAbstract: Autologous stem cell transplantation (auto-HSCT) is a part of the therapeutic strategy for various oncohematological diseases. The auto-HSCT procedure enables hematological recovery after high-dose chemotherapy, otherwise not tolerable, by the infusion of autologous hematopoietic stem cells. Unlike allogeneic transplant (allo-HSCT), auto-HSCT has the advantage of lacking acute-graft-versus-host disease (GVHD) and prolonged immunosuppression, however, these advantages are counterbalanced by the absence of graft-versus-leukemia. Moreover, in hematological malignancies, the autologous hematopoietic stem cell source may be contaminated by neoplastic cells, leading to disease reappearance. In recent years, allogeneic transplant-related mortality (TRM) has progressively decreased, almost approaching auto-TRM, and many alternative donor sources are available for the majority of patients eligible for transplant procedures. In adults, the role of auto-HSCT compared to conventional chemotherapy (CT) in hematological malignancies has been well defined in many extended randomized trials; however, such trials are lacking in pediatric cohorts. Therefore, the role of auto-HSCT in pediatric oncohematology is limited, in both first- and second-line therapies and still remains to be defined. Nowadays, the accurate stratification in risk groups, according to the biological characteristics of the tumors and therapy response, and the introduction of new biological therapies, have to be taken into account in order to assign auto-HSCT a precise role in the therapeutic strategies, also considering that in the developmental age, auto-HSCT has a clear advantage over allo-HSCT, in terms of late sequelae, such as organ damage and second neoplasms. The purpose of this review is to report the results obtained with auto-HSCT in the different pediatric oncohematological diseases, focusing on the most significant literature data in the context of the various diseases and discussing this data in the light of the current therapeutic landscape.Keywords: autologous stem cell transplant, pediatric age, acute leukemia, chronic myeloid leukemia, Hodgkin lymphoma, non-Hodgkin lymphoma

The aim of this study was to trace an exposure profile to traffic-derived pollution during pediatric age. For this purpose, two biomonitoring campaigns for the determination of urinary (u-) methyl tert-butyl ether (MTBE), ethyl tert-butyl ether (ETBE), tert-amyl methyl ether (TAME), and diisopropyl ether (DIPE) were carried out in two different periods of the year (summer 2017 and winter 2018), among a large sample of healthy children (n = 736; 5-11 years old) living in rural and urban areas in central Italy. The quantification of u-MTBE, u-ETBE, u-TAME, and u-DIPE was performed by HS-SPME-GC/MS technique and information on participants was collected by a questionnaire. u-DIPE concentrations resulted always under the LOQ. u-TAME mean levels were similar in both seasons (18.7 ng L-1 in summer vs. 18.9 ng L-1 in winter), while u-MTBE and u-ETBE levels were, respectively, 69.9 and 423.5 ng L-1 (summer) and 53.3 and 66.2 ng L-1 (winter). Main predictors of urinary excretion resulted the time spent in motor vehicles, being male and younger.

PURPOSE: The purpose of this study was to examine demographic and tomographic parameters in pediatric patients diagnosed with keratoconus and to evaluate the difference between early and late age periods. METHODS: This retrospective study included patients younger than 18 years of age who were diagnosed with keratoconus. All examination findings, demographic data, tomography parameters, and keratoconus stages were recorded. The patients were divided into two groups: 14 years or younger (Group 1) and those between 14 and 18 years old (Group 2). All corneal tomography parameters were compared in the subgroups and were evaluated statistically. P <0.05 was considered statistically significant. RESULTS: A total of 131 eyes of 71 patients were included. The mean age was 15.7 ± 1.8 (range, 10–18) years, and the female-to-male ratio was 23/48. The mean best spectacle-corrected visual acuity was 0.36 ± 0.44 (range, 0–3.1) logMAR. The averages of K1, K2, Km, and Kmax were 49.0 ± 6.2 (range, 39.6–74.5) D, 53.5 ± 7.0 (range, 43.3–76.8) D, 51.3 ± 6.5 (range, 41.5–75) D, and 59.1 ± 9.0 (range, 44.7–85.2) D, respectively, and the mean central corneal thickness (CCT) was 456.9 ± 63.5 µm (range, 261–601). The distribution of eyes with keratoconus from stages 1–4 was 5.3%, 36.6%, 38.9%, and 19.1%, respectively. When the groups were compared, K1, K2, Km, and Kmax were significantly steeper in Group 1 compared to Group 2 (P = 0.006, P = 0.004, P = 0.004, and P = 0.007, respectively). CCT and the thinnest corneal thickness values were significantly less in Group 1 (P = 0.038 and P = 0.041, respectively). CONCLUSION: The decision for treatment – especially cross-linking – should be made at the right time due to the increased risk of progression in the earlier ages in pediatric keratoconus population. [ABSTRACT FROM AUTHOR]

Background: Cardiac pacing from right ventricular (RV) sites may cause electromechanical ventricular dyssynchrony. Invasive and noninvasive mapping studies showed left ventricular (LV) activation sequence in adults. Aim of this study was to seek out the LV endocardial activation (LVEA) in pediatric patients who underwent RV pacing. Methods: Single‐center, prospective study conducted on pediatric patients who underwent left sided catheter ablation of accessory pathways with the Carto Univu mapping system. After successful ablation procedures, LVEA was recorded by the ablation catheter during sinus rhythm (SR) and during para‐hisian (PHP), midseptum (MSP), and apical (RVAP) pacing. Results: Seventeen patients, 13 males, aged 12 (10–15) years, registered LV activation maps and times (LVAT). SR showed significantly shorter LVAT than during pacing. LVAT of PHP was shorter than MSP, while there were not significant differences among PHP and MSP versus RVAP. In SR initial LV endocardial activation occurred in two midseptum sites, inferior‐posterior and superior‐anterior. During PHP, initial activation occurred at parahisian basal septum, rapidly followed by midseptum as in SR. During MSP and RVAP initial activation occurred at midseptum and apex, respectively. From all initial sites, the excitation spreads toward the base of the lateral LV free wall. A mild linear correlation was found between QRS duration and LVAT for MSP and for PHP. Conclusions: In pediatric patients LVEA maps during RV pacing showed that the shortest LVAT was obtained with PHP. The LV activation pattern seemed similar in sinus rhythm, PHP and MSP, from midseptum to LV lateral base. [ABSTRACT FROM AUTHOR]

Hereditary Hemorrhagic Telangiectasia (HHT) or Rendu–Osler–Weber Syndrome (ROW) is an autosomal dominant vascular disease, with an estimated prevalence of 1:5000. Genes associated with HHT are ACVRL1, ENG, SMAD4, and GDF2, all encoding for proteins involved in the TGFβ/BMPs signaling pathway. The clinical diagnosis of HHT is made according to the “Curaçao Criteria,” based on the main features of the disease: recurrent and spontaneous epistaxis, muco-cutaneous telangiectases, arteriovenous malformations in the lungs, liver, and brain, and familiarity. Since the clinical signs of HHT can be misinterpreted, and the primary symptom of HHT, epistaxis, is common in the general population, the disease is underdiagnosed. Although HHT exhibits a complete penetrance after the age of 40, young subjects may also present symptoms of the disease and are at risk of severe complications. Here we review the literature reporting data from clinical, diagnostic, and molecular studies on the HHT pediatric population.

Alternating hypothyroidism and hyperthyroidism is a rare phenomenon, especially among pediatric patients. It is usually related to simultaneous, unbalanced presence of stimulating and blocking thyroid stimulating hormone receptor antibodies (TRAbs). Herein we describe thyroid stimulating hormone (TSH) and thyroid hormone fluctuations in an adolescent boy with negative TRAbs. A 12-year-old healthy boy exhibited alternating thyroid function, with several switches between hypothyroidism and hyperthyroidism during almost six years of follow-up. He had persistently elevated thyroid peroxidase antibodies, while TRAbs were repetitively negative. Due to a mild clinical presentation, most of the time he did not require any medication. This case contributes to the spectrum of alternating hypothyroidism and hyperthyroidism in the pediatric age and raises the question of mechanisms involved in fluctuating thyroid function. Therapeutic decisions should be individualized and guided by clinical manifestations and thyroid function tests, irrespective of the underlying pathophysiology.

Objective The aim of this study was to detect the posterior segment indications for the use of handheld optical coherence tomography in the pediatric age group. Patients and methods This was a retrospective observational study that included patients who were candidates for handheld spectral domain optical coherence tomography (HH-OCT) imaging for retinal or optic nerve (ON) diseases in Abo Elreesh hospital during the period from June 2018 to June 2022. The image, demographic, and clinical database of the included patients were reviewed. Data were tabulated using Microsoft Excel. Appropriate statistical methods were used to analyze and correlate the obtained data. Results A total of 189 patients were included in this study, comprising 109 (57.7%) male patients. The mean age was 7.2±3.9 (range: 0.2–16 years) years. Overall, 46 (24.3%) patients were less than 5 years of age (i.e. infants and preschool age). Patients who underwent HH-OCT scanning due to retinal causes (i.e. nystagmus, unexplained poor vision, uveitis, Coat’s disease, trauma, and high myopia) were 130 (68.8%) patients, whereas 59 (31.2%) patients were indicated due to ON disease (i.e. primary congenital glaucoma and juvenile open angle glaucoma, optic disc hyperemia, swelling or pallor, and cases of large physiological cupping). Conclusion HH-OCT is a noninvasive feasible image modality for evaluation of retina and ON in infants and young children. The lack of a normative database for retinal nerve fiber layer thickness and ON head parameters in the pediatric age group in the device available in our service is considered a limitation in its use for proper evaluation of different pathologies involving the ON.

BACKGROUND: Vertigo is a morbid symptom among children. However, children rarely experience vertigo, a clinical condition that has received less research than the adult group. OBJECTIVE: This study aimed to evaluate vertigo among pediatric age group on the basis of the clinical and neurotological profiles. MATERIALS AND METHODS: There were 78 children manifesting with giddiness or vertigo, who attended vertigo clinic at a tertiary care teaching hospital between March 2019 and April 2022. RESULTS: Of 78 children, 30 (38.46%) were boys and 48 (61.53%) were girls. In this study, the average age of the participating children was 11.8 years. Of the 78 children, 10 (12.82%) children were of preschool age, 22 (28.20%) children were of elementary school age, and 46 (58.97%) children were in the adolescent age group. The most common cause of the vertigo was vestibular migraine in pediatric age (30.76%). The most common associated symptom with vertigo was nausea and vomiting. CONCLUSION: There are various etiologies for vertigo. The most common etiology for vertigo in children is vestibular migraine. Children may show same types of clinical presentations. The treatment of vertigo in children should be individualized as per the diagnosis.

PurposeThis study aimed to investigate the specific risk factors and psycho-social and clinical features of hospitalized neuropsychiatric patients during the COVID pandemic and to analyze the hospital readmission phenomenon, which, according to recent studies, increased in frequency during the first pandemic period.Patients and methodsThis observational retrospective cohort study examined 375 patients aged between 0 and 17 years who were hospitalized between 1 February 2018 and 31 March 2022 due to neuropsychiatric issues. The majority of the patients were girls: there were 265 girls compared to 110 boys (M = 13.9 years; SD 2.30 years). The total sample was divided into two groups: the pre-COVID-19 group (160 inpatients hospitalized between February 2018 and February 2020) and the COVID-19 group (215 inpatients hospitalized between March 2020 and March 2022). To explore the readmission phenomenon (second aim), we selected from the two groups of patients with at least one hospital readmission within 365 days after the first discharge. Multiple variables (sociodemographic, clinical, psychological, and related to hospitalization) were collected for each patient by reviewing their medical records.ResultsThe risk factors for mental health disorders were similar between the two groups, except for the significantly increased use of electronic devices in the COVID-19 group, increasing from 8.8% in the pre-COVID-19 group to 29.2% in the COVID-19 group. Patients suffering from eating disorders increased from 11.3% in the pre-COVID-19 group to 23.8% in the COVID-19 group. Hospital readmissions nearly increased from 16.7% in the 2-year pre-COVID-19 period to 26.2% in the 2-year COVID-19 period. A total of 75% of patients hospitalized three or more times in the last 2 years and 85.7% of the so-called “revolving door” patients (with relapse within 3 months after discharge) were identified in the COVID-19 group. However, the comparison between the two groups of patients readmitted before and during the COVID-19 pandemic did not show any differences in terms of sociodemographic and clinical characteristics.ConclusionIn conclusion, there was a significant increase in hospital readmissions, but these results suggest the need for better coordination between hospital and territorial services in managing the complexity of mental health problems related to situations arising from the COVID-19 pandemic and the necessity to implement prevention strategies and services.

Background: In patients with adrenal insufficiency (AI), adrenal crisis (AC) represents a clinical emergency. Early recognition and prompt management of AC or AC-risk conditions in the Emergency Department (ED) can reduce critical episodes and AC-related outcomes. The aim of the study is to report the clinical and biochemical characteristics of AC presentation to improve their timely recognition and proper management in a ED setting. Methods: Single-centre, retrospective, observational study on pediatric patients followed at the Department of Pediatric Endocrinology of Regina Margherita Children's Hospital of Turin for primary AI (PAI) and central AI (CAI). Results: Among the 89 children followed for AI (44 PAI, 45 CAI), 35 patients (21 PAI, 14 CAI) referred to the PED, for a total of 77 accesses (44 in patients with PAI and 33 with CAI). The main causes of admission to the PED were gastroenteritis (59.7%), fever, hyporexia or asthenia (45.5%), neurological signs and respiratory disorders (33.8%). The mean sodium value at PED admission was 137.2 ± 1.23 mmol/l and 133.3 ± 1.46 mmol/l in PAI and CAI, respectively (p = 0.05). Steroids administration in PED was faster in patients with CAI than in those with PAI (2.75 ± 0.61 and 3.09 ± 1.47 h from PED access, p = 0.83). Significant factors related to the development of AC were signs of dehydration at admission (p = 0.027) and lack of intake or increase of usual steroid therapy at home (p = 0.059). Endocrinological consulting was requested in 69.2% of patients with AC and 48.4% of subjects without AC (p = 0.032). Conclusion: children with AI may refer to the PED with an acute life-threatening condition that needs prompt recognition and management. These preliminary data indicate how critical the education of children and families with AI is to improve the management at home, and how fundamental the collaboration of the pediatric endocrinologist with all PED personnel is in raising awareness of early symptoms and signs of AC to anticipate the proper treatment and prevent or reduce the correlated serious events. [ABSTRACT FROM AUTHOR]

Introduction: Nasopharyngeal carcinoma (NPC) is a very uncommon malignant tumour in children under the age of 20. The diagnosis of paediatric NPC is challenging due to its relative rarity. This unusual occurrence is frequently linked to delayed diagnosis, which can result in advanced loco-regional illness. Here, we investigate the clinical manifestations, investigations, and treatment of paediatric nasopharyngeal carcinoma in a nonendemic region. Method: The data from children and adolescents who had been diagnosed with nasopharyngeal cancer served as the basis for this study, which was carried out at the Cardiology and Pathology Departments of a tertiary teaching hospital in the eastern region of India. Result: In this retrospective analysis, 17 paediatric patients under the age of 18 were enrolled. Between December 2020 and January 2022, they were treated at a tertiary teaching hospital. 17 kids with nasopharyngeal cancer between the ages of 6 and 18 took part in this study. In this study, there were 7 girls (41.1%) and 10 boys (58.8%). NPC diagnosis occurred at a median age of 14 years (interquartile range: 6–18 years). In 15 (88.2%) individuals, neck swelling was the most frequent clinical manifestation, followed by nasal bleeding, nasal block, and hearing impairment in 10 (58.8%), 12 (70.5%), and 11 (64.7%) of cases, respectively. Conclusion: With the exception of individuals who have distant metastatic disease, children with NPC have a very good prognosis. NPC in children is typically not clinically suspected until the patient is in a late stage. The most important prognostic factor is the TNM staging. Unfortunately, NPC frequently has distant metastases at the time of diagnosis in the paediatric age range and has a tendency to be locally progressed.

Guidelines are important tools to guide the diagnosis and treatment of patients to improve the decision-making process of health professionals. They are periodically updated according to new evidence. Four new Guidelines in 2021, 2022 and 2023 referred to pediatric pacing and defibrillation. There are some relevant changes in permanent pacing. In patients with atrioventricular block, the heart rate limit in which pacemaker implantation is recommended was decreased to reduce too-early device implantation. However, it was underlined that the heart rate criterion is not absolute, as signs or symptoms of hemodynamically not tolerated bradycardia may even occur at higher rates. In sinus node dysfunction, symptomatic bradycardia is the most relevant recommendation for pacing. Physiological pacing is increasingly used and recommended when the amount of ventricular pacing is presumed to be high. New recommendations suggest that loop recorders may guide the management of inherited arrhythmia syndromes and may be useful for severe but not frequent palpitations. Regarding defibrillator implantation, the main changes are in primary prevention recommendations. In hypertrophic cardiomyopathy, pediatric risk calculators have been included in the Guidelines. In dilated cardiomyopathy, due to the rarity of sudden cardiac death in pediatric age, low ejection fraction criteria were demoted to class II. In long QT syndrome, new criteria included severely prolonged QTc with different limits according to genotype, and some specific mutations. In arrhythmogenic cardiomyopathy, hemodynamically tolerated ventricular tachycardia and arrhythmic syncope were downgraded to class II recommendation. In conclusion, these new Guidelines aim to assess all aspects of cardiac implantable electronic devices and improve treatment strategies.

Objective: The aim of the study is to evaluate the demographic features, localizations and pathological features of pediatric cases with hydatid cyst (HC). Method: We analyzed retrospectively 79 patients that histopathologically diagnosed as HCs between 2000 and 2020. Data such as patients' characteristics, site of lesions were collected from pathology reports. Results: Patient's mean age was 11.24+-4.42 (age range: 2-18 years). Most (51.9%) of the patients (n=41) were female and 48.1% of the patients (n=38) were male. The patients were distributed in the age groups of 11 (n=29) years, as indicated. There was a male predominance in >11 years group while female predominance was seen in other age groups. HCs were most frequently localized in the liver (54.4%, n=43), and then in the lungs (31.6%, n=25). The other localization sites of HCs were spleen, cerebrum, kidney, orbit, abdomen, bone, and submandibular area. Hepatic HCs were seen mostly in female (25/43; 54.0%), and pulmonary HCs in male (13/25; 52%) patients. Histopatologically all cases shared the same typical microscopic features of HC. Conclusion: The incidence rate of HCs in pediatric age group was increased by age. It is more common in older children (>11 years). Hepatic HCs were more common in female patients. Pulmonary HCs were more frequently seen in male patients. HCs can be seen in atypical localizations in pediatric age which should always be considered in the differential diagnosis of cystic lesions.

Abstract Background In Saudi Arabia, general pediatrics serves children until they are 14 years old. It has contributed to improving the health of Saudi children. Method This study adopted a qualitative method and recruited pediatric physicians to investigate status, successes, challenges, and opportunities. Later, data were analyzed using thematic analysis and hermeneutic phenomenology. Results This study attracted 13 pediatric physicians for interviews. All participants appreciated the role of general pediatricians, but the trainees had a negative attitude regarding the general pediatrics specialty. They all agreed on providing primary care for all children and recommended that their first visit should occur earlier. Shortage of pediatricians, lack of community pediatricians, busy clinics, limited Arabic resources, and poor communication skills are significant barriers to children receiving adequate care. The majority of pediatricians favor extending the pediatric age to 18 years old. One pediatrician stated, “Youths between ages 14–18 years are lost, adults and we refuse to care for them…” Additionally, pediatricians have concerns about managing developmental delays and behavioral issues. They believe the current pediatric residency provides many opportunities for a brighter future. Conclusion General pediatrics is well established in Saudi Arabia. To continue thriving, we need to address some challenges that pediatricians face and attract more residency graduates. The current pediatric residency programs can provide opportunities to address deficit areas.

Objective The aim of this study was to detect the posterior segment indications for the use of handheld optical coherence tomography in the pediatric age group. Patients and methods This was a retrospective observational study that included patients who were candidates for handheld spectral domain optical coherence tomography (HHOCT) imaging for retinal or optic nerve (ON) diseases in Abo Elreesh hospital during the period from June 2018 to June 2022. The image, demographic, and clinical database of the included patients were reviewed. Data were tabulated using Microsoft Excel. Appropriate statistical methods were used to analyze and correlate the obtained data. Results A total of 189 patients were included in this study, comprising 109 (57.7%) male patients. The mean age was 7.2±3.9 (range: 0.2-16 years) years. Overall, 46 (24.3%) patients were less than 5 years of age (i.e. infants and preschool age). Patients who underwent HH-OCT scanning due to retinal causes (i.e. nystagmus, unexplained poor vision, uveitis, Coat's disease, trauma, and high myopia) were 130 (68.8%) patients, whereas 59 (31.2%) patients were indicated due to ON disease (i.e. primary congenital glaucoma and juvenile open angle glaucoma, optic disc hyperemia, swelling or pallor, and cases of large physiological cupping). Conclusion HH-OCT is a noninvasive feasible image modality for evaluation of retina and ON in infants and young children. The lack of a normative database for retinal nerve fiber layer thickness and ON head parameters in the pediatric age group in the device available in our service is considered a limitation in its use for proper evaluation of different pathologies involving the ON. [ABSTRACT FROM AUTHOR]

In food allergy management, the avoidance of the allergen that caused the reaction plays a fundamental role. Nevertheless, that can be thwarted in case of accidental exposure to a rare or hidden allergen, causing the adoption of a monotonous diet and a consequent reduction in the quality of life of the patient and their family. The identification of a rare and hidden allergen is an important diagnostic challenge, taking into account that a significant proportion of all food reactions is in reality due to them. The aim of the present review is to provide the pediatric allergist an overview of the possible sources of rare and hidden food allergens, taking into consideration the routes of exposure to these potential allergens with the main examples published in the scientific literature and the distinction between types of direct or cross-contamination. The identification of the allergen responsible for the reaction and the provision of a dietary advice customized for the specific individual's dietary habits is essential to improve quality of life of the familiar nucleus and to reduce the risk of further allergic reactions. [ABSTRACT FROM AUTHOR]

Hereditary Hemorrhagic Telangiectasia (HHT) or Rendu–Osler–Weber Syndrome (ROW) is an autosomal dominant vascular disease, with an estimated prevalence of 1:5000. Genes associated with HHT are ACVRL1, ENG, SMAD4, and GDF2, all encoding for proteins involved in the TGFβ/BMPs signaling pathway. The clinical diagnosis of HHT is made according to the "Curaçao Criteria," based on the main features of the disease: recurrent and spontaneous epistaxis, muco-cutaneous telangiectases, arteriovenous malformations in the lungs, liver, and brain, and familiarity. Since the clinical signs of HHT can be misinterpreted, and the primary symptom of HHT, epistaxis, is common in the general population, the disease is underdiagnosed. Although HHT exhibits a complete penetrance after the age of 40, young subjects may also present symptoms of the disease and are at risk of severe complications. Here we review the literature reporting data from clinical, diagnostic, and molecular studies on the HHT pediatric population. [ABSTRACT FROM AUTHOR]

Resumen: Introducción y objetivos: Con objeto de hacer frente a la rápida propagación de la pandemia por coronavirus SARS-CoV-2, España decretó el confinamiento domiciliario de la población el 15 de marzo de 2020. El objetivo principal de este estudio es evaluar la repercusión de dicha medida sobre el control glucémico en niños y adolescentes con diabetes mellitus tipo 1 (DM1). Pacientes y métodos: Estudio observacional, retrospectivo, en niños y adolescentes con DM1 usuarios de sistemas de monitorización de glucosa intersticial. Se recogió la siguiente información correspondiente a las 2 últimas semanas de cuarentena, previas al inicio del desconfinamiento, para su posterior comparación con los datos de 2 semanas previas al confinamiento: necesidades diarias de insulina, glucosa intersticial media, HbA1c estimada, coeficiente de variación, tiempo en rango (70-180 mg/dl), hipoglucemia ( 180 y > 250 mg/dl), uso del sensor y número de glucemias capilares. Mediante encuesta se obtuvo información acerca de rutinas de ingesta, ejercicio físico, necesidad de ajustes en la terapia, complicaciones agudas surgidas y acompañamiento de los pacientes por sus cuidadores durante el confinamiento. Resultados: Se incluyeron 80 pacientes (edad media 12,61 ± 3,32 años, tiempo medio de evolución de la enfermedad 5,85 ± 3,92 años), 66,2% tratados con bomba de insulina, usuarios de los siguientes sistemas de monitorización: GuardianTM 3 (65%), FreeStyle LibreTM (18,8%) y Dexcom G6TM (16,2%). El tiempo en rango en la cohorte se incrementó de forma significativa durante el confinamiento (72,1 ± 10,5 vs. 74,8 ± 10,5%; p = 0,011) a expensas de una disminución del tiempo en hipoglucemia tanto 250 mg/dl (4,6 ± 3,9 vs. 3,7 ± 3,7%; p = 0,038), reduciéndose también el coeficiente de variación (35,8 ± 6,3 vs. 33,1 ± 6,1%; p 180 and > 250 mg/dl), sensor use and number of blood glucose measurements. Data about meal routines, physical exercise, need for adjustments in therapy, acute complications, and lockdown of caregivers were assessed via a survey. Results: 80 patients were studied (mean age 12.61 ± 3.32 years, mean time of evolution of the disease 5.85 ± 3.92 years), 66.2% treated with an insulin pump, users of following glucose monitoring systems: GuardianTM 3 (65%), FreeStyle LibreTM (18.8%) and Dexcom G6TM (16.2%). Time in range in the cohort increased significantly during confinement (72.1 ± 10.5 vs. 74.8 ± 10.5%; P = .011) with lower time in hypoglycemia both 250 mg/dl (4.6 ± 3.9 vs. 3.7 ± 3.7%; P = .038). CV also decreased (35.8 ± 6.3 vs. 33.1 ± 6.1%; P

Abstract Background COVID-19 lockdown caused sudden changes in people’s lifestyle, as a consequence of the forced lockdown imposed by governments all over the world. We aimed to evaluate the impact of lockdown on body mass index (BMI) in a cohort of allergic children and adolescents. Methods From the first of June until the end of October 2020, we submitted a written questionnaire to all the patients who, after lockdown, carried out a visit at the Pediatric Allergy Unit of the Department of Mother-Child, Urological Science, Sapienza University of Rome. The questionnaire was composed by 10 questions, referring to the changes in their daily activities. Data were extrapolated from the questionnaire and then analyzed considering six variables: BMI before and BMI after lockdown, sugar intake, sport, screens, sleep, and anxiety. Results One hundred fifty-three patients agreed to answer our questionnaire. Results showed a statistically significant increase in the BMI after lockdown (20.97 kg/m2 ± 2.63) with respect to the BMI before lockdown (19.18 kg/m2 ± 2.70). A multivariate regression analysis showed that the two variables that mostly influenced the increase in BMI were sleep and anxiety. Conclusions For the analyzed cohort of allergic children and adolescents we obtained significant gain in BMI as consequences of lockdown, which can be explained by many factors: high consumption of consolatory food, less sport activities, more time spent in front of screens, sleep alteration associated with increased anxiety. All these factors acted together, although sleep alteration and increased anxiety were the most influential factors that led to the worsening or the onset of weight gain, creating the basis for future health problems.

ABSTRACT Objectives: Evaluate the celiac disease (CD) markers, within the scope of its screening, in a pediatric population with diagnosis of type 1 diabetes (T1D) at Hospital de Braga (HB) and determine the prevalence of CD in the sample. Reflect on CD screening algorithm applied in this pediatric population. Subjects and methods: Retrospective observational study with 94 patients diagnosed with T1D at age 10 years or younger, followed up at the HB Outpatient Diabetology Consultation, including those referred from other hospitals. Record of clinical information, IgA anti-transglutaminase and anti-endomysium and HLA DQ2/DQ8 haplotypes. Results: We obtained positive serological test for CD in 4 patients. This test had 100% sensitivity and specificity. The prevalence of CD was 4.3% (n = 4). Positive HLA screening in 84.6% of patients, with both sensitivity and negative predictive value of 100% and specificity of 16.67%. Diagnosis of CD was made on average 3.40 ± 3.32 years after the diagnosis of TD1. All cases of CD registered non-gastrointestinal manifestations, none had gastrointestinal symptoms. Conclusion: This study proved that there is a higher prevalence of CD in pediatric population with TD1, when compared to general population, and clarified the importance of CD screening. Furthermore, it was observed that serological screening for CD antibodies is an excellent screening test and HLA typing, although not the most suitable first line test, can be useful in excluding the possibility of patients with T1D developing CD.

Introduction: type 1 diabetes mellitus. It is one of the most prevalent chronic noncommunicable diseases in the child population. Its incidence is variable. The causal multifactoriality is proposed where genetic, environmental and immunological factors are involved. Objective: to characterize clinically and epidemiologically patients who debuted with type 1 diabetes mellitus in the child population of Villa Clara in the period from January 2007 to December 2016. Methods: an epidemiological, observational, descriptive, retrospective longitudinal study was carried out at the José Luis Miranda Pediatric Hospital in Santa Clara. The population of 229 children diagnosed with the disease between 1/1/2007 and 12/31/2016 followed up at the institution was studied. Carrying out the review of medical records. Results: 183 patients were identified, representing an average of 1.3/1,000 discharges/year. The absolute number of cases and their relationship with the number of discharges was variable (age, form of debut and sex). The average incidence was 13.12 cases per 100,000 pediatric population, with an average annual increase of 2.3 cases/year. The mean age at diagnosis was 9.74 ± 4.58 years, with greater frequency, respectively. Conclusions: type 1 diabetes mellitus has a high incidence in Villa Clara, the debut occurs frequently between 5 and 14 years, without differences between the sexes, in the form of diabetic ketoacidosis and ketosis, with a very low frequency of complications. Some epidemiological characteristics were similar to those described in the literature. [ABSTRACT FROM AUTHOR]