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The fecal microbiome of 55 obese children and adolescents (BMI-SDS 3.2 ± 0.7) and of 25 normal-weight subjects, matched both for age and sex (BMI-SDS - 0.3 ± 1.1) was analysed. Streptococcus, Acidaminococcus, Sutterella, Prevotella, Sutterella wadsworthensis, Streptococcus thermophilus, and Prevotella copri positively correlated with obesity. The inferred pathways strongly associated with obesity concern the biosynthesis pathways of tyrosine, phenylalanine, tryptophan and methionine pathways. Furthermore, polyamine biosynthesis virulence factors and pro-inflammatory lipopolysaccharide biosynthesis pathway showed higher abundances in obese samples, while the butanediol biosynthesis showed low abundance in obese subjects. Different taxa strongly linked with obesity have been related to an increased risk of multiple diseases involving metabolic pathways related to inflammation (polyamine and lipopolysaccharide biosynthesis). Cholesterol, LDL, and CRP positively correlated with specific clusters of microbial in obese patients. The Firmicutes/Bacteroidetes-ratio was lower in obese samples than in controls and differently from the literature we state that this ratio could not be a biomarker for obesity.
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Microbioma Gastrointestinal , Microbiota , Obesidad Infantil , Niño , Adolescente , Humanos , Lipopolisacáridos , AlgoritmosRESUMEN
Wolfram Syndrome (WS) is a rare neurodegenerative disease with autosomal recessive inheritance and characterized by juvenile onset, non-autoimmune diabetes mellitus and later followed by optic atrophy leading to blindness, diabetes insipidus, hearing loss, and other neurological and endocrine dysfunctions. A wide spectrum of neurodegenerative abnormalities affecting the central nervous system has been described. Among these complications, neurogenic bladder and urodynamic abnormalities also deserve attention. Urinary tract dysfunctions (UTD) up to end stage renal disease are a life-threatening complication of WS patients. Notably, end stage renal disease is reported as one of the most common causes of death among WS patients. UTD have been also reported in affected adolescents. Involvement of the urinary tract occurs in about 90% of affected patients, at a median age of 20 years and with peaks at 13, 21 and 33 years. The aim of our narrative review was to provide an overview of the most important papers regarding urological impairment in Wolfram Syndrome. A comprehensive search on PubMed including Wolfram Syndrome and one or more of the following terms: chronic renal failure, bladder dysfunction, urological aspects, and urinary tract dysfunction, was done. The exclusion criteria were studies not written in English and not including urinary tract dysfunction deep evaluation and description. Studies mentioning general urologic abnormalities without deep description and/or follow-up were not considered. Due to the rarity of the condition, we considered not only papers including pediatric patients, but also papers with pediatric and adult case reports.
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Enfermedades Neurodegenerativas , Atrofia Óptica , Sistema Urinario , Síndrome de Wolfram , Adolescente , Adulto , Niño , Humanos , Urodinámica , Adulto JovenRESUMEN
Endothelial dysfunction (ED) is characterized by an imbalance between vasodilator and vasoconstriction agents. Several pathological conditions clinically diagnosed in childhood and adolescence are characterized by ED and increased risk for early development of microangiopathic and macroangiopathic impairment, in particular type 1 diabetes mellitus (T1DM), T2DM, obesity, metabolic syndromeand pituitary dysfunction associated to various endocrinopathies. More recently insulin resistance following chemotherapy or radiotherapy for tumors, bone marrow transplantation for hematological malignancies (i.e., cancer survivors), or immunosuppressive treatment for solid organ transplantation has been observed. Assessment of ED by means of non-invasive techniques is the gold standard for early ED detection before clinical manifestation. It is aimed to recognize patients at risk and to avoid the development and progression of more serious illnesses. Reactive hyperemia-peripheral artery tonometry is a noninvasive technique to assess peripheral endothelial function by measuring modifications in digital pulse volume during reactive hyperemia, and represents a non-invasive, reproducible and operator-independent tool able to detect precocious ED. This narrative review aimed to provide an overview of the most important papers regarding ED detection by EndoPat 2000 in children and adolescents with different endocrine diseases. A comprehensive search of English language articles was performed in the MEDLINE database without using other search filters except the publication interval between 2005 and 2020.
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Assessment of endothelial dysfunction in cancer survivors may have a role in the early identification of non-communicable diseases and cardiovascular late effects. Oncological therapies may impair endothelial function. Therefore, in patients such as childhood cancer survivors who could benefit from early cardioprotective pharmacological interventions, it is essential to monitor endothelial function, even if the optimal methodology for investigating the multifaceted aspects of endothelial dysfunction is still under debate. Biochemical markers, as well as invasive and non-invasive tools with and without pharmacological stimuli have been studied. Human clinical studies that have examined lifestyle or cancer treatment protocols have yielded evidence showing the involvement of lipid and lipoprotein levels, glycemic control, blood pressure, adiposity, inflammation, and oxidative stress markers on the state of endothelial health and its role as an early indicator of cardiometabolic risk. However, with regards to pharmacological interventions, cautious interpretation of the result attained whilst monitoring the endothelial function is warranted due to methodological limitations and substantial heterogeneity of the results reported in the published studies. In this narrative review, an overview of evidence from human clinical trials examining the effects of cancer therapies on endothelial disease is provided together with a discussion of endothelial function assessment using the different non-invasive techniques available for researchers and clinicians, in recent years.
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BACKGROUND: Listeria monocytogenes is a Gram-positive bacteria transmitted to human by animal stools, contaminated water and food. In children, Listeria monocytogenes typically affects newborns and immunocompromised patients often leading to invasive syndromes including sepsis, brain abscesses, meningitis, meningoencephalitis and rhombencephalitis. In healthy and immunocompetent children, Listeria meningitis is rare, but can progress rapidly and may be associated with severe complications (hydrocephalus, ventriculitis, cranial nerves palsy and cerebrospinal abscesses) and high mortality rate. CASE PRESENTATION: We describe a very uncommon case of meningoencephalitis due to Listeria monocytogenes in a 11-month-old immunocompetent girl. Cerebrospinal fluid (CSF) culture was positive on the second day. Antibiotic therapy was promptly started but the disease was complicated by neurological deterioration and decompensated hydrocephalus. The child required a very demanding pediatric and neurosurgical management and was discharged after 40 days without major sequelae. CONCLUSION: Listeria is difficult to isolate and it is not susceptible to first-line treatment for bacterial meningitis with extended-spectrum cephalosporins. Early recognition is therefore crucial for a positive outcome. Pediatricians have to perform close clinical monitoring of these children and be aware of possible complications. A review of all cases of Listeria meningitis complicated by hydrocephalus in healthy children has been performed, to provide an overview on clinical features, treatment options and outcome.
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Hidrocefalia/microbiología , Meningitis por Listeria/complicaciones , Meningitis por Listeria/diagnóstico , Femenino , Humanos , Hidrocefalia/diagnóstico , Hidrocefalia/terapia , Lactante , Meningitis por Listeria/terapiaRESUMEN
AIMS: Diabetic Nephropathy (DN) is rarely encountered in childhood, otherwise early subclinical abnormalities are detectable few years after diabetes diagnosis. Our aim was to evaluate the incidence rate of microalbuminuria in childhood onset type 1 diabetes (DM1) patients. Secondary aim was to examine which variables could influence the development of DN. METHODS: We longitudinally evaluated 137 young patients with DM1 from diagnosis (1994-2004) for a median of 11.8â¯years (1st-3rd q: 9.7-15.0). Overnight albumin excretion rate, degree of metabolic control, presence of microangiopathic complications and autoimmune co-morbidities were retrospectively collected. RESULTS: DN was observed in 16/137 cases (11.7%), with an incidence rate of 10.0 per 1000â¯person-years. Young T1D patients with persistent micro/macro-albuminuria were more likely to have higher HbA1c concentrations over the last four years (Pâ¯=â¯0.04), and were more likely to have retinopathy (Pâ¯=â¯0.011) and subclinical peripheral neuropathy (Pâ¯=â¯0.003). CONCLUSIONS: DN predictors were age at DM1 diagnosis and mean HbA1c levels. Even if DN incidence is lower than reported, periodical screening is mandatory. Moreover, borderline microalbuminuria as additional risk factor deserves attention.