181 research outputs found

    Gut Microbiota and Obesity in Adults and Children: The State of the Art

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    In recent decades, obesity has become a serious public health problem affecting both children and adults. Considering the multifactorial origin of obesity, including modifiable factors, childhood was identified as the golden age for investing in obesity prevention by both promoting proper lifestyles and actively intervening in possible triggers. The gut microbiota is at the center of the most recent scientific studies and plays a key role in obesity development because it is intimately linked to energetic-humoral variations in the host: its alterations can promote a state of excessive energy storage, and it can be manipulated to maintain energy homoeostasis. This review aims to offer a panoramic understanding of the interplay between obesity and the gut microbiota, focusing on the contribution that the gut microbiota could have to the prevention of childhood obesity and its complications in adulthood. Currently, the use of some specific probiotic strains has been shown to be able to act on some secondary metabolic consequences of obesity (such as liver steatosis and insulin resistance) without any effect on weight loss. Although definitive conclusions cannot be drawn on the real impact of probiotics and prebiotics, there is no doubt that they represent an exciting new frontier in the treatment of obesity and associated metabolic dysfunctions. Targeted studies randomized on specific populations and homogeneous for ethnicity, sex, and age are urgently needed to reach definitive conclusions about the influence of microbiota on weight. In particular, we still need more studies in the pediatric population to better understand when the switch to an obese-like gut microbiota takes place and to better comprehend the right timing of each intervention, including the use of pre/probiotics, to improve it

    Silent Sinus Syndrome and Williams Syndrome: Two Rare Diseases Found in a Pediatric Patient

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    Silent sinus syndrome (SSS) is a rare disease process characterized by progressive enophthalmos and hypoglobus due to ipsilateral maxillary sinus hypoplasia and orbital floor resorption. Patients may also present with eye asymmetry, unilateral ptosis, or diplopia. Most reported cases in the literature describe its occurrence in adults, but it can also affect children. The etiology remains speculative, even though the most accepted theory is that during the first or second decade of life, occlusion of the maxillary ostium causes an interruption in normal sinus development. Williams syndrome (WS) is a rare genetic, multisystem disorder characterized by a constellation of distinctive phenotypic features, including psychomotor delay and cardiovascular abnormalities. We report a case of a 7-year-old female diagnosed at 1 year old with WS and who gradually developed SSS. This last condition was diagnosed at 7 years of age, when she started showing progressive facial asymmetry in addition to typical facial features of WS; subsequent neuroimaging definitively supported the diagnosis. This case report describes for the first time in the literature an uncommon situation in which SSS and WS, both rare syndromes, are present in the same pediatric patient. We speculate that the particular facial features typical of WS could either be the basis of the development of SSS in our patient or make the SSS clinical course more severe, with signs presenting at the age of 7 years. This case report shows for the first time that facial asymmetry in WS can be caused by SSS and highlights the need for early identification of this complication in patients with syndromes characterized by dysmorphic facial features. Further studies are needed to understand whether there is a link between the two syndromes as well as to evaluate the prevalence of SSS in patients with facial dysmorphisms and define the best management

    Report on advances for pediatricians in 2018: allergy, cardiology, critical care, endocrinology, hereditary metabolic diseases, gastroenterology, infectious diseases, neonatology, nutrition, respiratory tract disorders and surgery.

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    This review reported notable advances in pediatrics that have been published in 2018. We have highlighted progresses in allergy, cardiology, critical care, endocrinology, hereditary metabolic diseases, gastroenterology, infectious diseases, neonatology, nutrition, respiratory tract disorders and surgery. Many studies have informed on epidemiologic observations. Promising outcomes in prevention, diagnosis and treatment have been reported. We think that advances realized in 2018 can now be utilized to ameliorate patient car

    Psychology and hereditary angioedema: A systematic review

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    Background: Hereditary angioedema (HAE) is caused by mutations in the C1 inhibitor (C1-INH) gene Serpin Family G Member 1(SERPING1), which results in either the decreased synthesis of normal C1-INH (C1-INH–HAE type I) or expression of unfunctional C1-INH (C1-INH–HAE type II). In recent studies, emotional stress was reported by patients as the most common trigger factor for C1-INH–HAE attacks. Moreover, patients reported considerable distress over the significant variability and uncertainty with which the disease manifests, in addition to the impact of physical symptoms on their overall quality of life. Objective: We did a systematic review of the literature to shed light on the advancements made in the study of how stress and psychological processes impact C1-INH–HAE. Methods: All of the articles on C1-INH–HAE were analyzed up to December 2019. Both medical data bases and psychological data bases were examined. The keywords (KWs) used for searching the medical and psychological data bases were the following: “hereditary angioedema,” “psychology,” “stress,” “anxiety,” and “depression.” Results: Of a total of 2549 articles on C1-INH–HAE, 113 articles were retrieved from the literature search by using the related KWs. Twenty-one of these articles were retrieved, examined, and classified. Conclusion: Although the literature confirmed that stress may induce various physical diseases, it also warned against making simplistic statements about its incidence that did not take into account the complexity and multicausality of factors that contribute to C1-INH–HAE expression

    Spontaneous Pneumo-Mediastinum in a Post-COVID-19 Patient with Systemic Sclerosis

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    Pulmonary involvement is the most common cause of death among patients with systemic sclerosis (SSc). The current coronavirus disease 2019 (COVID-19) is particularly problematic to manage in SSc patients since they may experience a more severe evolution of COVID-19 due to the pre-existent interstitial lung disease (ILD) and the administration of immunosuppressive treatments. In addition, the remarkable radiological similarities between SSc-ILD and COVID-19 complicate the differential diagnosis between these two entities. Herein, we present the first case of spontaneous pneumo-mediastinum in a post-COVID-19 patient with SSc. In our patient, both smoking and pulmonary fibrosis could lead to cyst formation, which possibly spontaneously broke and caused pneumo-mediastinum. Moreover, megaesophagus perforation due to the smooth muscle atrophy, replacement with fibrosis, and achalasia may extend into the mediastinum or pleural space and has also been described as a rare case of spontaneous pneumo-pericardium. Finally, spontaneous pneumo-mediastinum and pneumothorax have been recently reported as an established complication of severe COVID-19 pneumonia and among COVID-19 long-term complication. This case report underlines that the worsening of respiratory symptoms in SSc patients, especially when recovered from COVID-19, requires further investigations for ruling out other tentative diagnoses besides the evolution of the SSc-ILD

    Childhood Vaccinations and Type 1 Diabetes.

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    Type 1 diabetes (T1D) is the most common paediatric endocrine disease, and its frequency has been found to increase worldwide. Similar to all conditions associated with poorly regulated glucose metabolism, T1D carries an increased risk of infection. Consequently, careful compliance by T1D children with schedules officially approved for child immunization is strongly recommended. However, because patients with T1D show persistent and profound limitations in immune function, vaccines may evoke a less efficient immune response, with corresponding lower protection. Moreover, T1D is an autoimmune condition that develops in genetically susceptible individuals and some data regarding T1D triggering factors appear to indicate that infections, mainly those due to viruses, play a major role. Accordingly, the use of viral live attenuated vaccines is being debated. In this narrative review, we discussed the most effective and safe use of vaccines in patients at risk of or with overt T1D. Literature analysis showed that several problems related to the use of vaccines in children with T1D have not been completely resolved. There are few studies regarding the immunogenicity and efficacy of vaccines in T1D children, and the need for different immunization schedules has not been precisely established. Fortunately, the previous presumed relationship between vaccine administration and T1D appears to have been debunked, though some doubts regarding rotavirus vaccines remain. Further studies are needed to completely resolve the problems related to vaccine administration in T1D patients. In the meantime, the use of vaccines remains extensively recommended in children with this disease

    Episodic angioedema with hypereosinophilia (Gleich’s syndrome): A case report and extensive review of the literature

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    Episodic angioedema with eosinophilia (EAE) (Gleich’s syndrome) is a rare disease charac-terized by hypereosinophilia (up to 95 × 109 cells/L), recurrent episodes of angioedema, urticaria, weight gain, and fever, that occur at periodical intervals (usually every 3–4 weeks). The exact etiology of EAE is still unclear, but both eosinophils and abnormalities of cytokines homeostasis seem to play a pivotal role in the pathogenesis of the disease. In particular, the cyclic elevation of serum interleukin-5 before the increase in eosinophil count has been reported. Herein, we performed a broad literature review and report the case of a thirty-two-year-old woman with a two-year history of cyclic angioedema attacks, urticaria, periodic weight gain, and severe hypereosinophilia, diagnosed with EAE and treated with oral corticosteroids. Describing the most relevant clinical features of EAE reported so far in the literature, we aim to provide physicians with some useful tools to help them deal with this disease. In addition, we aim to raise awareness about this rare condition in which approved diagnostic classification criteria are currently missing

    Nailfold videocapillaroscopy findings in bradykinin-mediated angioedema

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    Background: Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) and acquired angioedema related to angiotensin-converting enzyme (ACE) inhibitors (ACEI-AAE) are types of bradykinin-mediated angioedema without wheals characterized by recurrent swelling episodes. Recent evidence suggests that a state of “vascular preconditioning” predisposes individuals to attacks, although no data are available on possible structural alterations of the vessels. Objective: This study aims to compare the features of nailfold capillaries to highlight possible structural anomalies between patients affected by C1-INH-HAE and controls and between patients with ACEI-AAE and hypertensive controls. Methods: We used nailfold videocapillaroscopy (NVC) to assess the following: apical, internal, and external diameter; loop length; intercapillary distance; and capillary density, distribution, and morphology. Plasma levels of vascular endothelial growth factor (VEGF) A, VEGF-C, angiopoietin (Ang) 1, and Ang2 were also measured. Results: Compared with healthy controls (n=28), C1-INH-HAE patients (n = 34) were characterized by significant structural alterations of the capillaries, such as greater intercapillary distance (216 vs 190 µm), increased apical, internal, and external diameter (28 vs 22 µm; 22 vs 20 µm; and 81 vs 65 µm, respectively), decreased density (4 vs 5 capillaries/mm2), more irregular capillary distribution, and more tortuous morphology. Apical diameter was enlarged in patients with ≥12 attacks per year. In ACEI-AAE patients, NVC showed no alterations with respect to hypertensive controls. NVC performed in 2 C1-INH-HAE patients during attacks showed no changes compared with the remission phase. Conclusions: We detected major structural capillary alterations in C1-INH-HAE patients, thus confirming the involvement of microcirculation in the pathogenesis of angioedema

    Report on advances for pediatricians in 2018: Allergy, cardiology, critical care, endocrinology, hereditary metabolic diseases, gastroenterology, infectious diseases, neonatology, nutrition, respiratory tract disorders and surgery

    Get PDF
    This review reported notable advances in pediatrics that have been published in 2018. We have highlighted progresses in allergy, cardiology, critical care, endocrinology, hereditary metabolic diseases, gastroenterology, infectious diseases, neonatology, nutrition, respiratory tract disorders and surgery. Many studies have informed on epidemiologic observations. Promising outcomes in prevention, diagnosis and treatment have been reported. We think that advances realized in 2018 can now be utilized to ameliorate patient care
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