Diabetic Ketoacidosis Complicated With Previously Unknown Gitelman Syndrome in a Tunisian Child

Gitelman syndrome (GS) is an auto- somal recessive disease characterized by hypokalemia, hypomagnesemia, metabolic alkalosis, and hypocalciuria. In the great majority of cases, GS is caused by mutations in the SLC12A3 gene encoding the thiazide-sensitive NaCl co- transporter (NCCT), which is specifically expressed in the apical membrane of cells along the distal convoluted tubul

Gastro-intestinal symptoms as clinical manifestation of peritoneal and retroperitoneal spread of an invasive lobular breast cancer: report of a case and review of the literature

BACKGROUND: Distant spread from breast cancer is commonly found in bones, lungs, liver and central nervous system. Metastatic involvement of peritoneum and retroperitoneum is unusual and unexpected. CASE PRESENTATION: We report the case of a 67 year-old-woman who presented with gastrointestinal symptoms which revealed to be the clinical manifestations of peritoneal and retroperitoneal metastatic spread of an invasive lobular breast cancer diagnosed 15 years before. CONCLUSION: To the best of our knowledge, the case presented is the third one reported in literature showing a wide peritoneal and extraperitoneal diffusion of an invasive lobular breast cancer. The long and complex diagnostic work up which led us to the diagnosis is illustrated, with particular emphasis on the multidisciplinary approach, which is mandatory to obtain such a result in these cases. Awareness of such a condition by clinicians is mandatory in order to make an early diagnosis and start a prompt and correct therapeutic approach

Millimetric observations with a high-altitude 2.6-m ground based telescope

High atmospheric performances are necessary to ensure efficient sub/millimetre cosmological observations from ground. Low atmospheric components fluctuations along the line of sight are a must for best detector applications. Such site constraints are attained only at in specific places around the world: highaltitude observatories or, equivalently, polar regions. We are currently involved in cosmological observations with the MITO project from an Alpine ground station which satisfies such requirements: the Testa Grigia mountain at 3500 m a.s.l., AO—Italy. The Chacaltaya laboratory at 5200 m a.s.l. could also be an appropriate mm-site. One of the goals of MITO is the multifrequency observation of nearby rich clusters of galaxies for measuring the Sunyaev-Zel’dovich effect. Combined S-Z and X-ray measurements yield the Hubble constant and other cosmological information. A dedicated instrument has been designed to minimize spurious contaminations on the signals. The telescope is a 2.6 m Cassegrain with a wobbling subreflector and a 4-band single pixel photometer installed at the focal plane. The bolometric detectors are cooled down to 300 mK by a double stage He3-He4 fridge

Millimetric observations with a high-altitude 2.6-m ground based telescope

High atmospheric performances are necessary to ensure efficient sub/millimetre cosmological observations from ground. Low atmospheric components fluctuations along the line of sight are a must for best detector applications. Such site constraints are attained only at in specific places around the world: highaltitude observatories or, equivalently, polar regions. We are currently involved in cosmological observations with the MITO project from an Alpine ground station which satisfies such requirements: the Testa Grigia mountain at 3500 m a.s.l., AO—Italy. The Chacaltaya laboratory at 5200 m a.s.l. could also be an appropriate mm-site. One of the goals of MITO is the multifrequency observation of nearby rich clusters of galaxies for measuring the Sunyaev-Zel’dovich effect. Combined S-Z and X-ray measurements yield the Hubble constant and other cosmological information. A dedicated instrument has been designed to minimize spurious contaminations on the signals. The telescope is a 2.6 m Cassegrain with a wobbling subreflector and a 4-band single pixel photometer installed at the focal plane. The bolometric detectors are cooled down to 300 mK by a double stage He3-He4 fridge

Melanin concentration gradients in modern and fossil feathers.

In birds and feathered non-avian dinosaurs, within-feather pigmentation patterns range from discrete spots and stripes to more subtle patterns, but the latter remain largely unstudied. A ∼55 million year old fossil contour feather with a dark distal tip grading into a lighter base was recovered from the Fur Formation in Denmark. SEM and synchrotron-based trace metal mapping confirmed that this gradient was caused by differential concentration of melanin. To assess the potential ecological and phylogenetic prevalence of this pattern, we evaluated 321 modern samples from 18 orders within Aves. We observed that the pattern was found most frequently in distantly related groups that share aquatic ecologies (e.g. waterfowl Anseriformes, penguins Sphenisciformes), suggesting a potential adaptive function with ancient origins

Rituximab Unveils Hypogammaglobulinemia and Immunodeficiency in Children with Autoimmune Cytopenia

BACKGROUND: Rituximab (RTX; anti-CD20 mAb) is a treatment option in children with refractory immune thrombocytopenia, autoimmune hemolytic anemia (AHA), and Evans syndrome (ES). Prevalence and clinical course of RTX-induced hypogammaglobulinemia in these patients are poorly known. OBJECTIVE: To evaluate the prevalence and risk factors for persistent hypogammaglobulinemia (PH) after RTX use. METHODS: Clinical and immunologic data from children treated with RTX for immune thrombocytopenia, AHA, and ES were collected from 16 Italian centers and 1 UK center at pre-RTX time point (0), +6 months, and yearly, up to 4 years post-RTX. Patients with previously diagnosed malignancy or primary immune deficiency (PID) were excluded. RESULTS: We analyzed 53 children treated with RTX for immune thrombocytopenia (n = 36), AHA (n = 13), and ES (n = 4). Median follow-up was 30 months (range, 12-48). Thirty-two percent of patients (17 of 53) experienced PH, defined as IgG levels less than 2 SD for age at last follow-up (>12 months after RTX). Significantly delayed B-cell recovery was observed in children experiencing PH (hazard ratio, 0.55; P < .05), and 6 of 17 (35%) patients had unresolved B-cell lymphopenia at last follow-up. PH was associated with IgA and IgM deficiency, younger age at RTX use (51 vs 116 months; P < .01), a diagnosis of AHA/ES, and better response to RTX. Nine patients with PH (9 of 17 [53%]) were eventually diagnosed with a PID. CONCLUSIONS: Post-RTX PH is a frequent condition in children with autoimmune cytopenia; a sizable proportion of patients with post-RTX PH were eventually diagnosed with a PID. In-depth investigation for PID is therefore recommended in these patients

The AGILE Mission

AGILE is an Italian Space Agency mission dedicated to observing the gamma-ray Universe. The AGILE's very innovative instrumentation for the first time combines a gamma-ray imager (sensitive in the energy range 30 MeV-50 GeV), a hard X-ray imager (sensitive in the range 18-60 keV), a calorimeter (sensitive in the range 350 keV-100 MeV), and an anticoincidence system. AGILE was successfully launched on 2007 April 23 from the Indian base of Sriharikota and was inserted in an equatorial orbit with very low particle background. Aims. AGILE provides crucial data for the study of active galactic nuclei, gamma-ray bursts, pulsars, unidentified gamma-ray sources, galactic compact objects, supernova remnants, TeV sources, and fundamental physics by microsecond timing. Methods. An optimal sky angular positioning (reaching 0.1 degrees in gamma- rays and 1-2 arcmin in hard X-rays) and very large fields of view (2.5 sr and 1 sr, respectively) are obtained by the use of Silicon detectors integrated in a very compact instrument. Results. AGILE surveyed the gamma- ray sky and detected many Galactic and extragalactic sources during the first months of observations. Particular emphasis is given to multifrequency observation programs of extragalactic and galactic objects. Conclusions. AGILE is a successful high-energy gamma-ray mission that reached its nominal scientific performance. The AGILE Cycle-1 pointing program started on 2007 December 1, and is open to the international community through a Guest Observer Program

Thermal benefits of nest shelter for incubating female eiders

1. In species that actively maintain optimal conditions for offspring development, the quality of the breeding site may have a substantial effect on reproductive effort and breeding success. Here, we studied the effect of nest shelter on reproductive costs for incubating female common eiders (Somateria mollissima) nesting in an arctic environment using both a correlative and an experimental approach. 2. The correlative data showed that females that nested in naturally well-sheltered nests experienced milder temperatures, laid larger clutches, provided a more favourable thermal environment for their eggs and had a higher hatching success than those at exposed nest-sites. 3. We added artificial shelters to exposed nest-sites and compared them to unmanipulated exposed nest-sites to examine the effect of nest shelter in females of similar quality. Costs of nesting in exposed sites were greater as evidenced by the reduced female mass loss and more favourable thermal conditions for the eggs at artificially sheltered nest-sites relative to exposed nest-sites. However, there was no difference in hatchings success between artificially sheltered and exposed nest-sites. 4. This study shows even small changes in the climatic conditions at the nest-site can have substantial consequences for reproductive effort, but the association between nest-site quality and breeding success in eiders is due to better quality individuals occupying better nest-sites

Scanning electron microscopy of collagen fiber orientation in the bone lamellar system in non-decalcified human samples.

Previous studies on collagen fiber orientation have led to different interpretations and theories about the fiber arrangement in the lamellar compact bone. The purpose of this investigation was to provide new and more in-depth data on fiber arrangement in the lamellar bone system in order to explain the orientation of the fiber bundles. This was carried out by applying a simple method of preparation which permitted observation of non-decalcified samples. A previously isolated Haversian system was subjected to slow bending up to reaching the fracture point. Hence, the fracture surface was observed by SEM. The same samples were also observed by polarized light microscopy. A significant alternation of fiber orientation in the adjacent lamellae was observed. Different domains of differently oriented fibers were present within the same lamella; conjugating fibers connecting adjacent lamellae were also shown. This method avoided most of the artifacts due to chemical treatment of bone samples. The results can be easily interpreted by means of the same criteria applied in mechanics for the studying of composite materials