Current Pediatric Reviews - Volume 17, Issue 4, 2021

Liver transplantation is the standard treatment for children with end-stage liver disease, primary hepatic neoplasms, or liver-localized metabolic defects. Perioperative mortality is almost absent, and long-term survival exceeds 90%. Organ shortage is managed thanks to advances in organ retrieval techniques; living donation and partial liver transplantation almost eliminated waiting list mortality, thus leading to expanding indications for transplantation. The success of pediatric liver transplantation depends on the prompt and early referral of patients to transplant Centers and on the close and integrated multidisciplinary collaboration between pediatricians, hepatologists, surgeons, intensivists, oncologists, pathologists, coordinating nurses, psychologists, and social workers.

Background: In pediatric patients, Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) infection has been mostly associated with mild symptoms. However, as in adults, renal involvement has been reported in children and adolescents with Coronavirus Disease 2019 (COVID-19). Objective: This review aimed to report data about renal involvement in pediatric COVID-19 patients. The focuses were on the pathophysiology of acute kidney injury in Pediatric Inflammatory Multisystem Syndrome Temporally Associated (PIMS-TS) with SARS-CoV-2 and the possible impact of SARS-CoV-2 infection upon kidney function, as well as data concerning patients with previous kidney diseases, including Nephrotic Syndrome and Chronic Renal Disease. The implications for COVID-19 outcomes in pediatric patients were also discussed. Methods: This integrative review searched for articles on renal involvement in pediatric COVID-19 patients. The databases evaluated were PubMed and Scopus. Results: The emergence of PIMS-TS with SARS-CoV-2 has shown that pediatric patients are at risk of severe COVID-19, with multi-organ involvement and dysfunction. In addition to intense inflammation, several systems are affected in this syndrome, collectively creating a combination of factors that results in acute kidney injury. Several studies have proposed that kidney cells, including the podocytes, might be at risk of direct infection by SARS-CoV-2, as high levels of ACE2, the virus receptor, are expressed on the membrane of such cells. Some cases of glomerular diseases triggered by SARS-CoV-2 infection and relapses of previous renal diseases have been reported. Conclusion: Further studies are necessary to establish risk factors for renal involvement in pediatric COVID-19 and to predict disease outcomes.

This article's primary goal is to provide an image-based review to paediatricians to gain insight into the typical appearance of myelin evolution. We briefly discuss the structure and development of myelination, the role of qualitative and quantitative MRI in myelin imaging, and provide an image-based review as a quick reference for understanding the pattern of myelination on MR imaging.

Background: Juvenile dermatomyositis is the most common inflammatory myopathy in the pediatric age group and a major cause of mortality and morbidity in individuals with childhood rheumatic diseases. Mounting evidence suggests that early diagnosis and timely aggressive treatment are associated with better outcomes. Objective: The purpose of this article is to provide readers with an update on the evaluation, diagnosis, and the treatment of juvenile dermatomyositis. Methods: A PubMed search was performed in Clinical Queries using the key term “juvenile dermatomyositis” in the search engine. The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. The search was restricted to English literature. The information retrieved from the above search was used in the compilation of the present article. Results: Juvenile dermatomyositis is a chronic autoimmune inflammatory condition characterized by systemic capillary vasculopathy that primarily affects the skin and muscles with possible involvement of other organs. In 2017, the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) developed diagnostic criteria for juvenile idiopathic inflammatory myopathies and juvenile dermatomyositis. In the absence of muscle biopsies which are infrequently performed in children, scores (in brackets) are assigned to four variables related to muscle weakness, three variables related to skin manifestations, one variable related to other clinical manifestations, and two variables related to laboratory measurements to discriminate idiopathic inflammatory myopathies from non-idiopathic inflammatory myopathies as follows: objective symmetric weakness, usually progressive, of the proximal upper extremities (0.7); objective symmetric weakness, usually progressive, of the proximal lower extremities (0.8); neck flexors relatively weaker than neck extensors (1.9); leg proximal muscles relatively weaker than distal muscles (0.9); heliotrope rash (3.1); Gottron papules (2.1); Gottron sign (3.3); dysphagia or esophageal dysmotility (0.7); the presence of anti-Jo-1 autoantibody (3.9); and elevated serum levels of muscle enzymes (1.3). In the absence of muscle biopsy, a definite diagnosis of idiopathic inflammatory myopathy can be made if the total score is ≥7.5. Patients whose age at onset of symptoms is less than 18 years and who meet the above criteria for idiopathic inflammatory myopathy and have a heliotrope rash, Gottron papules or Gottron sign are deemed to have juvenile dermatomyositis. The mainstay of therapy at the time of diagnosis is a high-dose corticosteroid (oral or intravenous) in combination with methotrexate. Conclusion: For mild to moderate active muscle disease, early aggressive treatment with high-dose oral prednisone alone or in combination with methotrexate is the cornerstone of management. Pulse intravenous methylprednisolone is often preferred to oral prednisone in more severely affected patients, patients who respond poorly to oral prednisone, and those with gastrointestinal vasculopathy. Other steroid-sparing immunosuppressive agents such as cyclosporine and cyclophosphamide are reserved for patients with contraindications or intolerance to methotrexate and for refractory cases, as the use of these agents is associated with more adverse events. Various biological agents have been used in the treatment of juvenile dermatomyositis. Data on their efficacy are limited, and their use in the treatment of juvenile dermatomyositis is considered investigational.

In recent years, there has been increased interest in the study of pain in children and its treatment. It is known that when facing diagnostic and therapeutic procedures similar to those performed on adults, children either do not receive specific pain treatment or receive it on a significantly lower scale. However, recent research suggests a change in attitude and an improvement in the current treatment of children's pain. Although current knowledge demonstrates the falsity of many preconceived ideas about pain and its management, our results suggest that attitudinal change towards childhood pain remains slow and that real improvement in the training and practical application of the pediatrician who has to treat childhood pain is urgently needed. In this context, this manuscript has prepared standards and guidelines to improve pain management practices in a large number of national and international professional settings.

In this paper, we will review the dietary allowances of these fatty acids in the paediatric population, and also the indications in different pathologies within the field of pediatric gastroenterology. Finally, we will try to explain the reasons that may justify the difficulty in translating good results in experimental studies to the usual clinical practice. This “good results” may be too little to be detected or there may be other causes but misinterpreted as effects of DHA.

Background: Prader-Willi Syndrome (PWS) is a complex neurodevelopmental disorder caused by gene alterations on chromosome 15q11-q13, resulting in hyperphagia and neuroendocrine deficits. A comprehensive guide for dental treatment for PWS is lacking despite numerous case reports. The objective of this report was to develop a problem-focused list of the interrelationship between oral and systemic parameters of PWS and enable dentists in anticipating the unique treatment needs of children and individuals with PWS. Methods: Four pediatric patients with PWS presenting to an academic dental clinic were evaluated. A literature review spanning the last twenty years was performed to identify the pathophysiological impact of systemic problems on dental health and treatment. Results: The four cases along with cases from the literature were used to enumerate salient oro-dental and systemic features influencing treatment decisions in dentistry. They formed the basis for collective recommendations and precautions for rendering dental treatment in patients with PWS. Conclusion: Sedation for dental treatment is contraindicated due to obesity (BMI over 95th percentile), hypotonia, obstructive sleep apnea (OSA), and respiratory limitations (restricted ventilation due to weight on thoracic cage). Prolonged recovery from general anesthesia, OSA, and temperature dysregulation necessitate extended monitoring after dental rehabilitation under general anesthesia. Orthopedic problems and respiratory limitations exclude protective stabilization. Xerostomia and acidic saliva necessitate recommendations for oral rehydrating products. Periodontal assessment is necessary due to poor oral hygiene and diabetes mellitus. Early establishment of a dental home and risk-based frequency of dental care should address caries prevention and restorative needs.