Primary Oro-Facial Manifestations of Langerhans Cell Histiocytosis in Pediatric Age: A Bi-Institutional Retrospective Study on 45 Cases

Aims: Langerhans Cell Histiocytosis is a rare hematologic disorder usually affecting children and most commonly involving the head and neck region. Primary oro-facial manifestations are rare, and their diagnosis is often challenging as they are numerous and often resemble common pathologies, refractory to conventional medical and/or instrumental treatments. For such reasons, the diagnosis is frequently delayed, as is the following staging and therapy onset. We retrospectively studied 45 pediatric patients affected by Langerhans Cell Histiocytosis with onset in the head and neck, to examine their clinical and radiological features at the early stage. Materials and Methods: The study was a retrospective bi-institutional analysis (Department of Pediatric Dentistry and Pediatric Oncology of “Sapienza” University of Rome, Department of Interdisciplinary Medicine of the University of Bari “Aldo Moro”), which enrolled 45 patients (age range 0–18 year-old) affected by Langerhans Cell Histiocytosis with oro-facial onset. Data regarding clinical appearance, number, site, synchronous or metachronous occurrence, involved tissues/organs, radiographic features and clinical outcomes were collected, listed and overall differentiated by two age ranges (0–10-year-olds and 10–18-year-olds). Results: Patients were 26 males and 19 females, with an average age at the time of diagnosis of 4.8 ± 3.8 years (median = 3.9 years). The most common findings were inflamed, hyperplastic, painful and often ulcerated gingival lesions (22 cases), associated with deciduous tooth mobility and/or dislocation with bone loss in 18 cases, followed by nine single eosinophilic granulomas of the mandible and two of the maxilla. Lesions of the palatal mucosa were observed in six patients; nine patients showed on radiograms the characteristic “floating teeth” appearance in the mandible with synchronous lesions of the maxilla in six. Paresthesia was relatively un-frequent (three cases) and the pathological fracture of the mandible occurred in six. Head/neck lymph nodes involvement was associated with oral lesions in 12 cases and skull lesions in 14. Otitis (media or externa) was detected in four instances, exophthalmia in two, cutaneous rush in nine, contextual presence or subsequent onset of insipidus diabetes in eight. As for therapy, single or multiple small jaw lesions were all surgically removed; chemotherapy with vinblastine alone or associated with corticosteroids was the principal treatment in almost the 80% of cases; more than 50% of patients received corticosteroids, while only three patients received adjunctive radiotherapy. The overall mortality account for less than 9% (four of 45 cases) and recurrence observed in eight patients after therapy. Conclusions: Langerhans Cell Histiocytosis may mimic several oro-facial inflammatory and neoplastic diseases. Considering the potential disabling sequela following head and neck localization of Langerhans Cell Histiocytosis in children, especially at the periodontal tissues with teeth and alveolar bone loss, lesion recognition along with the histological examination of suspicious tissues is mandatory to achieve an early diagnosis and to prevent further organ involvement

Nutraceutical characterization of anthocyanin-rich fruits produced by «Sun Black» tomato line

Tomato (Solanum lycopersicum L.) is one of the most cultivated vegetable in the world and it represents a large source of bioactive compounds, including carotenoids and polyphenols (phenolic acids and flavonoids). However, the concentration of flavonoids in tomato is considered sub-optimal, particularly because anthocyanins are not generally present. Therefore, this crop has been the object of an intense metabolic engineering in order to obtain anthocyanin-enriched tomatoes by using either breeding or transgenic strategies. Some wild tomato species, such as S. chilense and S. cheesmaniae, biosynthesize anthocyanins in the fruit sub-epidermal tissue, and some alleles from those genotypes have been introgressed into a new developed purple tomato line, called “Sun Black” (SB). It is a tomato line with a purple skin color, both in green and in red fruit stages, due to the biosynthesis of anthocyanins in the peel, and a normal red color pulp, with a taste just like a traditional tomato. SB is the result of a breeding programme and it is not a genetically modified (GM) product. We report the chemical characterization and structure elucidation of the attractive anthocyanins found in the peel of SB tomato, as well as other bioactive compounds (carotenoids, polyphenols, vitamin C) of the whole fruit. Using one- and two-dimensional NMR experiments, the two main anthocyanins were identified to be petunidin 3-O-[6″-O-(4‴-O-E-p-coumaroyl-α-rhamnopyranosyl) -β-glucopyranoside]-5-O-β-glucopyranoside (petanin) and malvidin 3-O-[6″-O-(4‴-O-E-p-coumaroyl-α-rhamnopyranosyl)-β-glucopyranoside]-5-O-β-glucopyranoside (negretein). The total anthocyanins in the whole ripe fruit was 1.2 mg/g dry weight (DW); 7.1 mg/100 g fresh weight (FW). Chlorogenic acid (the most abundant phenolic acid) was 0.6 mg/g DW; 3.7 mg/100 g FW. The main flavonol, rutin was 0.8 mg/g DW; 5 mg/100 g FW. The total carotenoid content was 211.3 μg/g DW; 1,268 μg/100 g FW. The total phenolic content was 8.6 mg/g DW; 52.2 mg/100 g FW. The vitamin C content was 37.3 mg/100 g FW. The antioxidant activities as measured by the TEAC and ORAC assays were 31.6 and 140.3 μmol TE/g DW, respectively (193 and 855.8 μmol TE/100 g FW, respectively). The results show the unique features of this new tomato genotype with nutraceutical properties.publishedVersio

Meta-omics reveals genetic flexibility of diatom nitrogen transporters in response to environmental changes

Diatoms (Bacillariophyta), one of the most abundant and diverse groups of marine phytoplankton, respond rapidly to the supply of new nutrients, often out-competing other phytoplankton. Herein, we integrated analyses of the evolution, distribution and expression modulation of two gene families involved in diatom nitrogen uptake (DiAMT1 and DiNRT2), in order to infer the main drivers of divergence in a key functional trait of phytoplankton. Our results suggest that major steps in the evolution of the two gene families reflected key events triggering diatom radiation and diversification. Their expression is modulated in the contemporary ocean by seawater temperature, nitrate and iron concentrations. Moreover, the differences in diversity and expression of these gene families throughout the water column hint at a possible link with bacterial activity. This study represents a proof-of-concept of how a holistic approach may shed light on the functional biology of organisms in their natural environment

Hybrid management of iliac injury during thoracic endovascular aortic repair: A case report

Thoracic endovascular aortic repair is nowadays the preferred option to manage descending thoracic aorta diseases. However, despite feasibility and safety of the procedures, several complications may occur. We report the case of an 83-year-old female patient with inadvertent iliac rupture occurred during thoracic endovascular aortic repair. To limit massive bleeding, considering the patient’s comorbidities contraindicating open surgical repair and the morphology of the arterial injury (circumferential rupture of the artery from its origin), we chose to perform a homolateral hypogastric and common iliac artery embolization and an aorto-uniliac balloon expandable stent graft deployment from the distal aorta to the contralateral common iliac artery. A femoro-femoral crossover bypass graft was performed to restore both lower limbs perfusion. Final angiography documented correct positioning and regular patency of the implanted grafts and bypass with no blood loss from the right iliac vessels. Despite careful preoperative assessment, iliac artery injury can represent a challenging complication of thoracic endovascular aortic repair, particularly in the setting of inadequate iliac diameter, calcification and vessel tortuosity, or when large-caliber introducers are required. The hybrid approach we describe is a valid and effective solution to minimize blood loss and avoid major consequences in the management of iatrogenic iliac artery rupture during endovascular procedures

Chronic abdominal aortic rupture mimicking femoral neuropathy

Chronic-contained rupture of an aortic aneurysm is a rare subset of ruptured aneurysms. The presentation is unusual, and the diagnosis is frequently delayed. Here, we describe a case of contained rupture of abdominal aortic aneurysm that presented with signs and symptoms of femoral neuropathy. Clinical and radiological findings were initially misinterpreted. The correct diagnosis was formulated belatedly, causing a progressively increased risk of fatal events. Surgical aortic repair was performed and the postoperative course was uneventful. In conclusion, in the presence of a retroperitoneal mass, a diagnosis of chronic-contained rupture of an abdominal aortic aneurysm should be considered

"Immunohistochemical pattern of IGF-1 and TGF-beta in multinodular euthyroid goiter"

(Atti XXVI Congresso Nazionale della Societ\ue0 Italiana di Endocrinologia - Pisa; Giugno 1995

Chronic abdominal aortic rupture mimicking femoral neuropathy

: Chronic-contained rupture of an aortic aneurysm is a rare subset of ruptured aneurysms. The presentation is unusual, and the diagnosis is frequently delayed. Here, we describe a case of contained rupture of abdominal aortic aneurysm that presented with signs and symptoms of femoral neuropathy. Clinical and radiological findings were initially misinterpreted. The correct diagnosis was formulated belatedly, causing a progressively increased risk of fatal events. Surgical aortic repair was performed and the postoperative course was uneventful. In conclusion, in the presence of a retroperitoneal mass, a diagnosis of chronic-contained rupture of an abdominal aortic aneurysm should be considered

Intestinal occlusion caused by cecal carcinoid

It is possible that carcinoids present themselves by a surgical emergency, without a >. For these reasons, whenever a rational treatment is requested, an intraoperative diagnosis should be performed. In fact these neoplasms--owing to a less intense aggressiveness--present more indications to undergo surgery. In fact the authors report a case of caecum carcinoid characterized by the lack of typical symptoms of the homonymous syndrome. Urgent surgery was performed because of intestinal occlusion; the ileocaecal valve involvement caused the necessity of a large ileocolic resection with a L-L ileo-transverse anastomosis. The diagnosis of carcinoid was revealed only by the definitive histopathological examination