ABSTRACT
The excitation spectrum of a cigar-shaped strongly dipolar quantum gas at the crossover from a Bose-Einstein condensate to a trapped macrodroplet is predicted to exhibit peculiar features-a strong upward shift of low momentum excitation energies together with a strong multiband response for high momenta. By performing Bragg spectroscopy over a wide range of momenta, we observe both key elements and also confirm the predicted stiffening of excitation modes when approaching the macrodroplet regime. Our measurements are in good agreement with numerical calculations taking into account finite size effects.
ABSTRACT
OBJECTIVES: Oculodentodigital dysplasia (ODDD) is a rare autosomal dominant congenital malformation syndrome characterized by high penetrance and great phenotypic heterogeneity. Neurological manifestations are thought to occur in about one third of cases, but systematic studies are not available. We performed deep neurological phenotyping of 10 patients in one ODDD pedigree. METHODS: Retrospective case series. We analyzed in depth the neurological phenotype of a three-generation family segregating the heterozygous c.416 T > C, p.(Ile139Thr) in GJA1. Clinical and neuroradiological features were retrospectively evaluated. Brain MRI and visual evoked potentials were performed in 8 and 6 cases, respectively. RESULTS: Central nervous system manifestations occurred in 5 patients, the most common being isolated ataxia either in isolation or combined with spasticity. Furthermore, sphincteric disturbances (neurogenic bladder and fecal incontinence) were recognized as the first manifestation in most of the patients. Subclinical electrophysiological alteration of the optic pathway occurred in all the examined patients. Neuroimaging was significant for supratentorial hypomyelination pattern and hyperintense superior cerebellar peduncle in all examined patients. CONCLUSION: The neurological involvement in ODDD carriers is often missed but peculiar clinical and radiological patterns can be recognized. Deep neurological phenotyping is needed to help untangle ODDD syndrome complexity and find genotype-phenotype correlations.
Subject(s)
Phenotype , Humans , Female , Male , Retrospective Studies , Adult , Adolescent , Evoked Potentials, Visual/physiology , Pedigree , Young Adult , Child , Magnetic Resonance Imaging , Eye Abnormalities/genetics , Eye Abnormalities/diagnostic imaging , Eye Abnormalities/physiopathology , Middle Aged , Brain/diagnostic imaging , Brain/physiopathology , Brain/pathologyABSTRACT
Oil production and processing often involve the treatment of water-in-oil emulsions stabilized by asphaltenes. The asphaltenes adsorb irreversibly at the water-oil interface and, by self-association at the interface, form a viscoelastic film that stabilizes the emulsions mechanically and sterically. Hydrophobic forces associated with these films may also contribute to the emulsion stability. A key step in treating these emulsions is to weaken the asphaltene film at the interface, and one way to do so is with ultrasonic treatment. The effect of ultrasonic waves on the interactions between asphaltene films was investigated at a silica-water interface using optical tweezers. Silica microparticles were aged in asphaltene solutions to form asphaltene coatings on their surfaces. The particles were dispersed in water, and interparticle force measurements were performed with optical tweezers to capture the steric force and hydrophobic force contributions. The asphaltene coating thickness and hydrophobic coefficient (a factor resembling the strength of the hydrophobic interaction) were obtained from fitting these forces. The effect of ultrasonication on the thickness of the asphaltene films on the surfaces of the particles was investigated. No change in the hydrophobic coefficient was observed upon changing the interfacial asphaltene concentration. The asphaltene film thickness increased with the concentration of the asphaltene solution and aging time. After treatment of the dispersion with ultrasonic waves for different durations (between 5 and 40 min), a significant reduction in the coating thickness was observed. This reduction was confirmed by thermogravimetric analysis (TGA) measurements. It is hypothesized that cavitation at the interface removed part of the surface layer of asphaltenes from the coated particles. Based on these findings, we proved that a low-power ultrasound field can effectively break asphaltene-stabilized water-in-oil emulsions.
ABSTRACT
INTRODUCTION: Erythrocytapheresis, an apheresis treatment which selectively removes red blood cells, is an alternative to therapeutic phlebotomy, over which it has several advantages. Actually there is a high degree of variability in the use of this treatment. This prompted SIdEM (Italian Society of Hemapheresis and Cell Manipulation) to conduct a survey on the use of erythrocytapheresis in the Italian Transfusion Services. The purpose is to monitor this activity in the treatment of Polycythemia Vera (pv), secondary erythrocytosis and hemochromatosis. MATERIALS AND METHODS: A data collection file was sent to the SIdEM regional delegates who, in turn, involved the Transfusion Centers in the areas they cover. The data collected were processed on a Microsoft Excel spreadsheet. RESULTS: 75 centers from 14 Italian regions responded to the Survey: 36 centers (48 %) use erythrocytapheresis (35 centers perform therapeutic apheresis and 1 center only donor apheresis), 39 centers (52 %) do not (15 centers perform therapeutic apheresis, 18 centers only donor apheresis and 6 centers do not perform either therapeutic apheresis or donor apheresis). Although most centers have a substantially uniform attitude concerning the indications for which erythrocytapheresis is used, the survey shows that there are still differences more evident in the treatment of secondary erythrocytosis than in the treatment of pv or hemochromatosis. CONCLUSIONS: This survey has been useful to document the current Italian reality and to raise awareness about the need for improvement in optimizing and standardizing the use of a therapy with a great potential to exploit properly.
Subject(s)
Blood Component Removal , Hemochromatosis , Polycythemia Vera , Polycythemia , Humans , Polycythemia/therapy , Polycythemia Vera/therapy , Hemochromatosis/therapy , Phlebotomy , ItalyABSTRACT
Imazethapyr, a post-emergent herbicide used in worldwide soybean and corn crops, induces genetic and biochemical alterations in aquatic vertebrates. This study examined the relationship between biomarkers at different organization levels and imazethapyr real-life route exposure in Boana pulchella adults. Frogs were exposed to imazethapyr-based formulation Pivot® H (10.59%) at concentrations representing possible acute routes: field runoff (S1:10 mg.L-1), exposure after direct foliar application (S2:100 mg.L-1) and during direct foliar application (S3:1000 mg.L-1). Post-exposure, endpoints levels were evaluated: organism alterations, biochemical activities and cytogenetic assays. Forty-eight hours post-exposure, antioxidant enzymes decrease, micronuclei induction and DNA damage were observed in all scenarios, while cholinesterase activity increase and body condition reduction were observed in frog-exposed to S3. Ninety-six hours post-exposure, frogs showed glutathione-S-transferase inhibition in S1, micronuclei induction in S2 and S3, and DNA-damage increase in S3. Herbicides routes of exposures in real-life could indicate that authorized applications have a risk to amphibian populations.
Subject(s)
Herbicides , Pesticides , Water Pollutants, Chemical , Animals , Anura , Pesticides/toxicity , Larva , Herbicides/toxicity , Biomarkers , Water Pollutants, Chemical/toxicityABSTRACT
Glutamatergic hyperactivity in the nucleus striatum, the main basal ganglia input, has been involved in the progression of Parkinson's disease (PD) and the onset of L-Dopa-induced dyskinesias (LIDs). Abnormalities in the spiny projection neurons excitability and firing, and in the overactivity of glutamate transmission found in animal models of PD, pointed to the synaptic dysfunctions as a primary target to counteract alterations before overt neurodegeneration, conferring a key role to striatal glutamatergic transmission in the early phases of the disease. The present paper provides an overview of the evidence that glutamatergic overactivity is a critical mechanism underlying different PD-associated striatal alterations in early and advanced symptomatic stages of the disease. These aberrant changes, under L-Dopa therapy, lead to a more complex synaptopathy that involves other neurotransmitter systems and persistent modifications to generate LIDs. The review discusses the main changes in glutamatergic functions found in PD preclinical models and clinical studies and an update of the current pharmacological strategies to modulate the glutamatergic systems at the pre- and postsynaptic levels will be provided.
Subject(s)
Levodopa , Parkinson Disease , Animals , Basal Ganglia , Corpus Striatum , Levodopa/pharmacology , NeostriatumABSTRACT
In 1981, Weinsier and Krumdieck described death resulting from overzealous total parenteral nutrition in two chronically malnourished, but stable, patients given aggressive total parenteral nutrition. This was the birth of what is now called the refeeding syndrome, a nutrition-related disorder associated with severe electrolyte disturbances. The purpose of this work is to demonstrate that refeeding syndrome was first described medically in Florence by Antonio Benivieni in 1507 in his book On Some Hidden and Remarkable Causes of Diseases and Cures. What we now know as refeeding syndrome was described in Report No. LVII of that book. The condition occurred as a result of the famine that affected Florence in 1496. The report documents (i) death due to starvation, (ii) death due to ingestion of deteriorated/toxic foods (inevitable in times of famine when healthy food is scarce), (iii) death caused by excessive food ingestion after forced, prolonged abstinence from food in adults, (iv) the death of breast-fed children and of their starved mothers eating to satiety and (v) the more favourable clinical outcome of those admitted to hospitals. It is possible that Benivieni was inspired by the description of the deaths of starved deserters in the book The Jewish War (70 AD) by the Romano-Jewish historian Flavius Josephus. Nevertheless, Benivieni wrote the first medical account of the central clinical features of refeeding syndrome. The main, broad clinical aspects of refeeding syndrome, described by Weinsier and Krumdieck in 1981, had been documented in medical literature four centuries earlier by Benivieni.
Subject(s)
Malnutrition , Refeeding Syndrome , Water-Electrolyte Imbalance , Adult , Child , Humans , Refeeding Syndrome/complications , Water-Electrolyte Imbalance/complicationsABSTRACT
Imazethapyr is an herbicide that is used in a variety of crops worldwide, including soybean and corn. The aim of the present study was to evaluate the biomarkers responses of adult Leptodactylus latinasus exposed to the formulation Pivot® H (10.59% imazethapyr) in the laboratory at concentrations and under conditions that simulate two potential field exposure scenarios: an immersion in field runoff (Scenario 1: 10 mg/L) and a direct exposure to the droplets emitted by spray noozles (Scenario 2: 1000 mg/L). In both scenarios, the experimental procedure involved completely immersing the frogs over a period of 15 s. Different endpoints were evaluated at several ecotoxicological levels 48 and 96 h after the herbicide exposure. These included individual (biometric indices and behavior alterations), histological (liver pigments and lesions), biochemical (catalase, glutathione system and cholinesterase activities) and genotoxic effects (micronuclei induction and nuclear abnormalities). Forty-eight hours after imazethapyr exposure, frogs submitted to Scenario 1 presented an inhibition of liver glutathione-S-transferase activity, whereas histological alterations and increased hepatic cholinesterase levels were observed in frogs exposed under Scenario 2. Ninety-six hours after exposure to the imazethapyr formulation, frogs from the Scenario 1 treatment presented a decrease in liver melanin and hemosiderin, increased hepatic catalase activity and micronuclei induction. For their part, frogs exposed to Scenario 2 presented a decrease in the hepatosomatic index, an increase in liver alterations, melanin reduction and micronuclei induction. The multivariate analysis enables correlations to be made between biomarkers of different organizational level in exposed anurans. Our result indicates that real exposure to imazethapyr formulations under field conditions may pose a risk to Leptodactylus latinasus populations living in the agroecosystems.
Subject(s)
Herbicides , Nicotinic Acids , Animals , Anura , DNA Damage , Herbicides/toxicityABSTRACT
The aim of this work is to refer on the death due to crush syndrome in 1277 of Pope John XXI, philosopher, logician, anatomist, physician scientist, university professor of medicine at the university of Siena and author of books adopted for nearly 4 centuries in universities in the Middle Ages. The Pope died crushed by the ceiling of his office which had been built in rush to meet his need for a quiet and warm place, his need of light and nature. There he attended to his duties of governing the church, studied fine theological questions, inspected the stars, made experiments and discussed with the renowned ophthalmologists who in those days made Viterbo the center of the study on vision. Following the fall of the ceiling of his apartment, John XXI was extracted alive from among the pieces of wood and stones. However, a few days after the disaster, he died in bad conditions (miserabiliter). He experienced a typical death due to crush syndrome which was described for the first time by Antonino D'Antona, following the Messina-Reggio Calabria 1908 earthquake. He was born (c. 1210-1220) in Lisbon as Pedro Hispano (Peter of Spain). He had regular trivium and quadrivium courses at the University of Paris under Albertus Magnus, a talented naturalist. He became Master of Arts, then studied medicine out of Paris (probably Montpellier or Salerno). He wrote three treatises (On the eye (De oculo), The Treasury of Medicines for the Poor (Thesaurus Pauperum) and Little Summaries of Logic (Summulae Logicales)) which were used in the European universities from the 13th to the beginning of the 18th century. Pedro Hispano was advisor of King Alphonso III for affairs inherent to the Church, bishop of Braga and then Cardinal Bishop of Tusculum and Pope as John XXI. He was buried in the Cathedral of Viterbo, the city where he had settled the seat of the Pontiff.
Subject(s)
Crush Syndrome , Earthquakes , Physicians , Humans , Male , Portugal , SpainABSTRACT
The diffusion of saturated and unsaturated hydrocarbons is of fundamental importance for many zeolite-catalyzed processes. Transport of small alkenes in the confined zeolite pores can become hindered, resulting in a significant impact on the ultimate product selectivity and separation. Herein, intracrystalline light olefin/paraffin diffusion through the 8-ring windows of zeolite SAPO-34 is characterized by a complementary set of first-principle molecular dynamics simulations, PFG-NMR experiments, and pulse-response temporal analysis of products measurements, yielding information at different length and time scales. Our results clearly show a promotional effect of the presence of Brønsted acid sites on the diffusion rate of ethene and propene, whereas transport of alkanes is found to be insensitive to the presence of acid sites. The enhanced diffusivity of unsaturated hydrocarbons is ascribed to the formation of favorable π-H interactions with acid protons, as confirmed by IR spectroscopy measurements. The acid site distribution is proven to be an important design parameter for optimizing product distributions and separations.
ABSTRACT
The thermo-optic coefficient (TOC) of photonic integrated waveguides fabricated on silicon-rich silicon nitride grown by plasma-enanched chemical vapor deposition is characterized for the first time, to the best of our knowledge. The TOC is found to increase linearly with the fractional composition of silicon over a range from that of silicon nitride to a-Si. This finding is significant for improving the power efficiency of thermally tuned photonic integrated circuits.
ABSTRACT
We measure the excitation spectrum of a stable dipolar Bose-Einstein condensate over a wide momentum range via Bragg spectroscopy. We precisely control the relative strength ε_{dd} of the dipolar to the contact interactions and observe that the spectrum increasingly deviates from the linear phononic behavior for increasing ε_{dd}. Reaching the dipolar-dominated regime ε_{dd}>1, we observe the emergence of a roton minimum in the spectrum and its softening towards instability. We characterize how the excitation energy and the strength of the density-density correlations at the roton momentum vary with ε_{dd}. Our findings are in excellent agreement with numerical calculations based on mean-field Bogoliubov theory. When including beyond-mean-field corrections, in the form of a Lee-Huang-Yang potential, we observe a quantitative deviation from the experiment, questioning the validity of such a description in the roton regime.
ABSTRACT
We study the spectrum of elementary excitations of a dipolar Bose gas in a three-dimensional anisotropic trap across the superfluid-supersolid phase transition. Theoretically, we show that, when entering the supersolid phase, two distinct excitation branches appear, respectively associated with dominantly crystal and superfluid excitations. These results confirm infinite-system predictions, showing that finite-size effects play only a small qualitative role, and connect the two branches to the simultaneous occurrence of crystal and superfluid orders. Experimentally, we probe compressional excitations in an Er quantum gas across the phase diagram. While in the Bose-Einstein condensate regime the system exhibits an ordinary quadrupole oscillation, in the supersolid regime we observe a striking two-frequency response of the system, related to the two spontaneously broken symmetries.
ABSTRACT
Uterine leiomyomas (fibroids or myomas) are the most common benign tumors of premenopausal women and new medical treatments are needed. This study aimed to determine the effects of omega-3 fatty acids on the lipid profile, membrane architecture and gene expression patterns of extracellular matrix components (collagen1A1, fibronectin, versican, or activin A), mechanical signaling (integrin ß1, FAK, and AKAP13), sterol regulatory molecules (ABCG1, ABCA1, CAV1, and SREBF2), and mitochondrial enzyme (CYP11A1) in myometrial and leiomyoma cells. Myometrial tissues had a higher amount of arachidonic acid than leiomyoma tissues while leiomyoma tissues had a higher level of linoleic acid than myometrial tissues. Treatment of primary myometrial and leiomyoma cells with eicosapentaenoic acid (EPA) or docosahexaenoic acid (DHA) reduced the monounsaturated fatty acid (MUFA) content and increased the polyunsaturated fatty acid (PUFA) content in both cell types. Myometrial and leiomyoma cell membranes were in the liquid-crystalline phase, but EPA- and DHA-treated cells had decreased membrane fluidity. While we found no changes in the mRNA expression of ECM components, EPA and DHA treatment reduced levels of ABCG1, ABCA1, and AKAP13 in both cell types. EPA and DHA also reduced FAK and CYP11A1 expression in myometrial cells. The ability of omega-3 fatty acids to remodel membrane architecture and downregulate the expression of genes involved in mechanical signaling and lipid accumulation in leiomyoma cells offers to further investigate this compound as preventive and/or therapeutic option.
Subject(s)
Cell Membrane/metabolism , Fatty Acids, Omega-3/pharmacology , Gene Expression Regulation, Neoplastic , Leiomyoma/genetics , Leiomyoma/pathology , Lipids/chemistry , Uterine Neoplasms/genetics , Uterine Neoplasms/pathology , Activins/genetics , Activins/metabolism , Adult , Cell Membrane/drug effects , Cholesterol Side-Chain Cleavage Enzyme/genetics , Cholesterol Side-Chain Cleavage Enzyme/metabolism , Docosahexaenoic Acids/pharmacology , Eicosapentaenoic Acid/pharmacology , Extracellular Matrix/drug effects , Extracellular Matrix/metabolism , Female , Gene Expression Regulation, Neoplastic/drug effects , Humans , Middle Aged , Myometrium/drug effects , Myometrium/metabolism , Myometrium/pathology , RNA, Messenger/genetics , RNA, Messenger/metabolism , Signal Transduction/drug effects , Sterols/metabolismABSTRACT
We realize a two-component dipolar Fermi gas with tunable interactions, using erbium atoms. Employing a lattice-protection technique, we selectively prepare deeply degenerate mixtures of the two lowest spin states and perform high-resolution Feshbach spectroscopy in an optical dipole trap. We identify a comparatively broad Feshbach resonance and map the interspin scattering length in its vicinity. The Fermi mixture shows a remarkable collisional stability in the strongly interacting regime, providing a first step towards studies of superfluid pairing, crossing from Cooper pairs to bound molecules, in presence of dipole-dipole interactions.
ABSTRACT
Adenosine represents a powerful modulating factor, which has been shown to orchestrate the scope, duration, and remission of the inflammatory response through the activation of four specific receptors, classified as A1, A2A, A2B, and A3, all being widely expressed in a variety of immune cells. Several selective A2A receptor agonists have displayed anti-inflammatory effects, through the suppression of IL-12, TNF, and IFN-γ production by monocytes and lymphocytes, in the setting of chronic intestinal inflammation. However, the therapeutic application of A2A receptor agonists remains hindered by the risk of serious cardiovascular adverse effects arising from the wide systemic distribution of A2A receptors. The present study focused on evaluating the anti-inflammatory effects of the novel poorly absorbed A2A receptor agonist PSB-0777 in a rat model of oxazolone-induced colitis as well as to evaluate its cardiovascular adverse effects, paying particular attention to the onset of hypotension, one of the main adverse effects associated with the systemic pharmacological activation of A2A receptors. Colitis was associated with decreased body weight, an enhanced microscopic damage score and increased levels of colonic myeloperoxidase (MPO). PSB-0777, but not dexamethasone, improved body weight. PSB-0777 and dexamethasone ameliorated microscopic indexes of inflammation and reduced MPO levels. The beneficial effects of PSB-0777 on inflammatory parameters were prevented by the pharmacological blockade of A2A receptors. No adverse cardiovascular events were observed upon PSB-0777 administration. The novel A2A receptor agonist PSB-0777 could represent the base for the development of innovative pharmacological entities able to act in an event-specific and site-specific manner.
Subject(s)
Adenosine A2 Receptor Agonists/pharmacology , Anti-Inflammatory Agents, Non-Steroidal/pharmacology , Colitis/pathology , Colon/drug effects , Furans/pharmacology , Adjuvants, Immunologic/toxicity , Animals , Anti-Inflammatory Agents, Non-Steroidal/administration & dosage , Anti-Inflammatory Agents, Non-Steroidal/chemistry , Colitis/chemically induced , Disease Models, Animal , Furans/administration & dosage , Furans/chemistry , Male , Oxazolone/toxicity , Rats , Rats, Sprague-DawleyABSTRACT
Acute lethal and sublethal toxicity of the pirimicarb-based commercial formulation Aficida® were evaluated on Boana pulchella tadpoles. Whereas mortality was used as end point for lethality, frequency of micronuclei and other nuclear abnormalities as well as alterations in the frequency of erythroblasts in circulating blood as biomarkers for genotoxicity and cytotoxicity, respectively. Swimming, growth, developmental and morphological abnormalities were also employed as sublethal end points. Results show that the species is within the 13th percentile of the distribution of acute sensitivity of species to pirimicarb for aquatic vertebrates. Results revealed values of 23.78 and 101.45mg/L pirimicarb as LC5096h for GS25 and GS36 tadpoles, respectively. The most evident effects were related with the swimming activity with NOEC and LOEC values within the 0.005-0.39mg/L pirimicarb concentration range. Aficida® induced DNA damage at the chromosomal level by increasing micronuclei frequency and other nuclear abnormalities, i.e., lobbed and notched nuclei and binucleated cells. Cellular cytotoxicity was found after Aficida® treatment. The presence of abdominal oedemas in exposed organisms and thus flotation response of organisms could be proposed as a new sensitive exposure parameter. The multiple end point assessment approach used allowed a complete understanding the multi level of effects occurring by exposure to pirimicarb, at least in B. pulchella.
Subject(s)
Carbamates/toxicity , DNA Damage , Insecticides/toxicity , Larva/drug effects , Pyrimidines/toxicity , Water Pollutants, Chemical/toxicity , Animals , Anura , Dose-Response Relationship, Drug , Larva/genetics , Lethal Dose 50 , Swimming , Toxicity Tests, Acute , Toxicity Tests, SubacuteABSTRACT
PURPOSE: Denosumab has been proven to reduce fracture risk in breast cancer (BC) women under aromatase inhibitors (AIs). Quantitative ultrasound (QUS) provides information on the structure and elastic properties of bone. Our aim was to assess bone health by phalangeal QUS and by dual-energy X-ray absorptiometry (DXA), and to evaluate bone turnover in AIs-treated BC women receiving denosumab. METHODS: 35 Postmenopausal BC women on AIs were recruited (mean age 61.2 ± 4.5 years) and treated with denosumab 60 mg administered subcutaneously every 6 months. Phalangeal QUS parameters [Amplitude Dependent Speed of Sound (AD-SoS), Ultrasound Bone Profile Index (UBPI), Bone Transmission Time (BTT)] and DXA at lumbar spine and femoral neck were performed. Serum C-telopeptide of type 1 collagen (CTX) and bone-specific alkaline phosphatase (BSAP) were also measured. The main outcomes were compared with a control group not receiving denosumab (n = 39). RESULTS: In patients treated with denosumab, differently from controls, QUS and DXA measurements improved after 24 months, and a reduction of CTX and BSAP was detected at 12 and 24 months in comparison to baseline (P < 0.05). The percent changes (Δ) of QUS measurements were significantly associated with ΔBMD at femoral neck, and ΔCTX and ΔBSAP were associated with ΔBMD at lumbar spine (r = -0.39, P = 0.02; r = -0.49, P = 0.01, respectively). CONCLUSIONS: Denosumab preserves bone health as assessed by phalangeal QUS and DXA. Since inexpensive and radiation-free, phalangeal QUS may be considered in the follow-up of AIs-treated BC women receiving denosumab.
Subject(s)
Absorptiometry, Photon/methods , Aromatase Inhibitors/adverse effects , Bone Density/drug effects , Breast Neoplasms/drug therapy , Denosumab/therapeutic use , Osteoporosis, Postmenopausal/diagnostic imaging , Ultrasonography/methods , Bone Density Conservation Agents/therapeutic use , Bone Remodeling/drug effects , Female , Humans , Middle Aged , Osteoporosis, Postmenopausal/chemically induced , Osteoporosis, Postmenopausal/pathology , Postmenopause , Prospective StudiesABSTRACT
The gastrointestinal tract is provided with extrinsic and intrinsic innervation. The extrinsic innervation includes the classic vagal parasympathetic and sympathetic components, with afferent sensitive and efferent secretomotor fibers. The intrinsic innervations is represented by the enteric nervous system (ENS), which is recognized as a complex neural network controlling a variety of cell populations, including smooth muscle cells, mucosal secretory cells, endocrine cells, microvasculature, immune and inflammatory cells. This is finalized to regulate gastrointestinal secretion, absorption and motility. In particular, this network is organized in several plexuses each one providing quite autonomous control of gastrointestinal functions (hence the definition of "second brain"). The similarity between ENS and CNS is further substantiated by the presence of local sensitive pseudo- unipolar ganglionic neurons with both peripheral and central branching which terminate in the enteric wall. A large variety of neurons and neurotransmitters takes part in the ENS. However, the nature of these neurons and their role in the regulation of gastrointestinal functions is debatable. In particular, the available literature reporting the specific nature of catecholamine- containing neurons provides conflicting evidence. This is critical both for understanding the specific role of each catecholamine in the gut and, mostly, to characterize specifically the enteric neuropathology occurring in a variety of diseases. An emphasis is posed on neurodegenerative disorders, such as Parkinson's disease, which is associated with the loss of catecholamine neurons. In this respect, the recognition of the nature of such neurons within the ENS would contribute to elucidate the pathological mechanisms which produce both CNS and ENS degeneration and to achieve more effective therapeutic approaches. Despite a great emphasis is posed on the role of noradrenaline to regulate enteric activities only a few reports are available on the anatomy and physiology of enteric dopamine neurons. Remarkably, this review limits the presence of enteric noradrenaline (and adrenaline) only within extrinsic sympathetic nerve terminals. This is based on careful morphological studies showing that the only catecholamine-containing neurons within ENS would be dopaminergic. This means that enteric pathology of catecholamine neurons should be conceived as axon pathology for noradrenaline neurons and whole cell pathology for dopamine neurons which would be the sole catecholamine cell within intrinsic circuitries affecting gut motility and secretions.The gastrointestinal tract is provided with extrinsic and intrinsic innervation. The extrinsic innervation includes the classic vagal parasympathetic and sympathetic components, with afferent sensitive and efferent secretomotor fibers. The intrinsic innervations is represented by the enteric nervous system (ENS), which is recognized as a complex neural network controlling a variety of cell populations, including smooth muscle cells, mucosal secretory cells, endocrine cells, microvasculature, immune and inflammatory cells. This is finalized to regulate gastrointestinal secretion, absorption and motility. In particular, this network is organized in several plexuses each one providing quite autonomous control of gastrointestinal functions (hence the definition of "second brain"). The similarity between ENS and CNS is further substantiated by the presence of local sensitive pseudounipolar ganglionic neurons with both peripheral and central branching which terminate in the enteric wall. A large variety of neurons and neurotransmitters takes part in the ENS. However, the nature of these neurons and their role in the regulation of gastrointestinal functions is debatable. In particular, the available literature reporting the specific nature of catecholamine-containing neurons provides conflicting evidence. This is critical both for understanding the specific role of each catecholamine in the gut and, mostly, to characterize specifically the enteric neuropathology occurring in a variety of diseases. An emphasis is posed on neurodegenerative disorders, such as including Parkinson's disease, which is associated with the loss of catecholamine neurons. In this respect, the recognition of the nature of such neurons within the ENS would contribute to elucidate the pathological mechanisms which produce both CNS and ENS degeneration and to achieve more effective therapeutic approaches. Despite a great emphasis is posed on the role of noradrenaline to regulate enteric activities only a few reports are available on the anatomy and physiology of enteric dopamine neurons. Remarkably, this review limits the presence of enteric noradrenaline (and adrenaline) only within extrinsic sympathetic nerve terminals. This is based on careful morphological studies showing that the only catecholamine-containing neurons within ENS would be dopaminergic. This means that enteric pathology of catecholamine neurons should be conceived as axon pathology for noradrenaline neurons and whole cell pathology for dopamine neurons which would be the sole catecholamine cell within intrinsic circuitries affecting gut motility and secretions.
Subject(s)
Catecholamines/metabolism , Enteric Nervous System/metabolism , Gastrointestinal Tract/metabolism , Neurons/metabolism , Animals , Autonomic Nervous System/metabolism , Humans , Neurodegenerative Diseases/metabolism , Organogenesis/physiologyABSTRACT
BACKGROUND AND AIMS: Branchial abnormalities occur when there is disturbance in the maturation of the branchial apparatus during fetal development. Branchial anomalies are congenital lesions usually present in childhood, even if they can be diagnosed later for enlargement or infection. A correct diagnosis will lead to proper management: complete surgical excision is the treatment of choice. The purpose of this article is to present clinical features, diagnostic methods and surgical treatment of branchial anomalies in childhood, based on a series of 50 patients. METHODS: We conducted a retrospective analysis of a total of 50 pediatric patients operated from June 2005 to June 2014 for the presence of branchial cleft anomalies. RESULTS: 27 cases (54 %) presented a second branchial cleft fistula and 11 cases (22 %) a second branchial cleft cyst and one case (2 %) presented both cyst and sinus of the second branchial cleft; four cases (8 %) presented first branchial cleft cyst whereas four cases (8 %) a first branchial cleft sinus and two cases (4 %) a first branchial cleft fistula; one case (2 %) presented a piriform sinus fistula (third branchial cleft). None of our patients presented anomalies of the fourth branchial cleft. All patients underwent surgical treatment and lesions have been removed by excision or fistulectomy. No post-surgical complication occurred. The rate of recurrence was 4 %. CONCLUSIONS: Pre-operative diagnosis supplies important information to the surgeon for a proper therapy: a complete excision of the lesion without inflammatory signs is essential to avoid re-intervention and to achieve a good outcome.