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Bardet–Biedl syndrome is a rare autosomal recessive disorder characterized by rod-cone dystrophy, renal dysfunction, obesity, learning difficulties, hypogonadism, polydactyl, and many other minor features that can affect the cardiovascular, locomotive, neurological, and endocrine systems. We report the case of a 16-year-old boy affected by Bardet–Biedl syndrome who presented with recurrent pericarditis with an optimal response to treatment with Anakinra. To our knowledge, this is the first description of an association between Bardet–Biedl syndrome and recurrent pericarditis.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disorder. When it presents before the age of 18 years (childhood-onset systemic lupus erythematosus, cSLE), the disease course tends to be more severe with a higher rate of organ involvement and requires an early diagnosis. Gastrointestinal involvement in cSLE is rare and scarcely reported in the literature. Any organ of the gastrointestinal system may be affected, either as a direct consequence of the disease, as a subsequent complication, or as an adverse drug event. Abdominal pain is the most common GI symptom, it can be diffuse or well localized, and can underline different conditions such as hepatitis, pancreatitis, appendicitis, peritonitis, or enteritis. cSLE may have an alteration of the intestinal barrier with features of protein-losing enteropathy or, in genetically predisposed patients, may develop associated autoimmune disorders such as Coeliac Disease or Autoimmune Hepatitis. The aim of this manuscript is to provide a narrative review of gastrointestinal manifestations in cSLE focused on hepatic, pancreatic, and intestinal involvement. A comprehensive literature search based on the PubMed database was performed.

The biological mechanisms linking nutrition and antioxidants content of the diet with cardiovascular protection are subject of intense investigation. It has been demonstrated that dietary supplementation with cow, donkey or human milk, characterized by distinct nutritional properties, triggers significant differences in the metabolic and inflammatory status through the modulation of hepatic and skeletal muscle mitochondrial functions. Cardiac mitochondria play a key role for energy-demanding heart functions, and their disfunctions is leading to pathologies. Indeed, an altered heart mitochondrial function and the consequent increased reactive oxygen species (ROS) production and inflammatory state, is linked to several cardiac diseases such as hypertension and heart failure. In this work it was investigated the impact of the milk consumption on heart mitochondrial functions, inflammation and oxidative stress. In addition, it was underlined the crosstalk between mitochondrial metabolic flexibility, lipid storage and redox status as control mechanisms for the maintenance of cardiovascular health.

The dietary bioequivalence of alpha-linolenic (LNA) and docosahexaenoic acids (DHA) as substrates for brain and retinal n-3 fatty acid accretion during the brain growth spurt is reported for neonatal baboons who consumed a long-chain-polyunsaturate free commercial human infant formula with a n-6/n-3 ratio of 10:1. Neonates received oral doses of 13C-labeled fatty acids (LNA*) or (DHA*) at 4 wk of age, and at 6 wk brain (occipital cortex), retina, retinal pigment epithelium, liver, erythrocytes, and plasma were analyzed. In the brain, 1.71% of the preformed DHA* dose was detected, whereas 0.23% of the LNA* dose was detected as DHA*, indicating that preformed DHA is 7-fold more effective than LNA-derived DHA as a source for DHA accretion. In LNA*-dosed animals, DHA* was greater than 60% of labeled fatty acids in all tissues except erythrocytes, where docosapentaenoic acid was 55%. Estimates using dietary LNA levels as tracees indicate that brain turnover of DHA is less than 5% per week between weeks 4 and 6 of life. For retina and retinal pigment epithelium, preformed DHA was at levels 12-fold and 15-fold greater than LNA-derived DHA. Liver, plasma, and erythrocytes ratios were 27, 29, and 51, respectively, showing that these pools do not parallel tissue metabolism of a single dose of omega-3 fatty acids. The distributions of labeled fatty acids for LNA*-dosed animals were similar, in the order DHA > DPA > EPA > LNA, except for erythrocytes where docosapentaenoic acid predominated. These are the first direct measurements of the bioequivalence of DHA and LNA in neonatal primate brain and associated tissues.

The bioequivalence of dietary linolenic acid (LNA) and docosahexaenoic acid (DHA) for brain DHA accretion was measured in neonatal baboons at 4-6 wk of age using stable isotope tracers. Neonates consumed a conventional U.S. term-infant formula devoid of long chain polyunsaturates and with an n-6/n-3 ratio of about 10:1. At 4 wk of age, neonates were dosed with either 13C LNA or 13C DHA. At 6 wk of age, neonate brain, retina, and other organs were harvested for fatty acid and isotopic analyses. The relative accretion of labeled DHA was 7-fold greater as a percentage of dose for the DHA-dosed animals compared to the LNA-dosed animals. The baboon is an omnivore that regularly consumes meat and insects; its plasma lipid profile responds similarly to humans in response to changes in feeding and living habits. These observations suggest that the baboon is a suitable model for human unsaturated fatty acid studies.

Burn injury are second leading cause of accidental death in children. Treatment of burns depends on severity of injury, location, characteristics of child and mechanism of injury. Objective is to value the multiple problems in infant burns and immediate management in emergency room. The better and immediate treatment burns recover and prevent dirty scars.

Growth and Neurobehavioral Development of Infant Baboons Exposed Prenatally to Increase Uterine Myometrial Activity

«Ti scrivo solo un magro biglietto, per ricordarti che esisto e che soprattutto tu esisti in me: esisti tanto da essere l’ideale destinatario di quasi tutto quello che scrivo. Spero di esserlo un poco anche io per te, anche se non possiedo la tua formidabile ed esplicita, sempre, reattività.» Le parole che nel 1961 Pier Paolo Pasolini scriveva a Franco Fortini anticipano (e spiegano) il fascino ed il rilievo di questo libro che nel ’93, un anno prima della scomparsa, Fortini dedicò all’amico, qui riproposto con un ampio e puntuale apparato di note ed una postfazione che ripercorre il dialogo tra i due. Attraverso Pasolini rappresenta un caso del tutto a sé nel paesaggio della nostra letteratura: vi si mescolano autobiografia e commento esegetico, lettere e versi, epigrammi e recensioni, con una straordinaria profusione di intuizioni critiche e di interpretazioni sulle rispettive opere in progress e infine sulla «nostra esistenza ossia il senso del nostro essere al mondo» (così Fortini nell’Introduzione). Come in un duello infinito che ogni volta affronta a viso aperto le svolte della storia – il ’56, il ’68 – confluiscono in queste pagine, esemplarmente, la passione letteraria e quella politica, l’amicizia e il dissenso, la metrica e la biografia.