1,720,966 research outputs found
Osteogenesi imperfetta. Primi dati sulle alterazioni strutturali riscontrate nei tessuti mineralizzati di un dente
No full textStruttura e grado di mineralizzazione sono stati analizzati nello smalto e nella dentina del 2° dente premolare permanente dell'emimandibola sinistra in una paziente affetta da osteogenesi imperfetta tipo III (sindrome di Ekman-Lobstein). Dopo una breve descrizione del caso clinico, con particolare riferimento alle patologie dentaria e scheletrica, vengono riportati i risultati preliminari dello studio istopatologico. Essi dimostrano la presenza di difetti di mineralizzazione sia nello smalto che nella dentina. Nello smalto, le zone ipocalcificate si trovano solamente lungo la giunzione con la dentina, come in genere si riscontra nei denti normali; esse risultano, tuttavia, più estese di quelle fisiologiche. Non sono, pertanto, da ritenersi difetti primari dell'amelogenesi ma secondari alla dentinogenesi imperfetta. Al contrario, nella dentina, le zone ipocalcificate appaiono disseminate nelle regioni sia mantellare che circumpulpare. Rispetto alla dentina normocalcificata, queste zone osservate a luce polarizzata presentano le fibre collagene irregolarmente intrecciate. I risultati ottenuti sono brevemente discussi in relazione sia ai processi di deposizione dello smalto e della dentina sia alla resistenza fisica dei denti di pazienti affetti da osteogenesi imperfetta tipo III.By means of the light microscope, under ordinary and polarized light, and the microradiography the structure and degree of mineralization of enamel and dentin were studied in the permanent 2nd premolar tooth of the left emimandible in a patient afìfected by type III osteogenesis imperfecta (Ekman-Lobstein syndrome). After a brief description of the clinical case, with particular reference to skeletal and dental pathologies, the preliminary results of the histopathological analysis are reported. They show the existence of defects of mineralization in both enamel and dentin. In the former, the hypocalcified areas are only located close to the enamel-dentin junction, as it usually occurs in normal teeth; however, they look broader than physiological ones. Thus they do not seem to be primary defects of amelogenesis but secondary ones, due to dentinogenesis imperfecta. On the contrary, in dentin, the hypocalcified areas are scattered throughout both mantle and circumpulpal regions. In the hypocalcified areas, compared with normally mineralized dentin, collagen fibers appear to be irregularly interwoven under polarized light. The results obtained are briefly discussed in relation to the processes of enamel and dentin deposition, and to the physical resistance of the teeth in type III osteogenesis imperfecta
Bone growth, modeling and remodeling in a supernumerary metatarsal bone associated with segmental gigantism in cutis marmorata telangiectatica congenita
Full text linkSkeletal structure and processes of bone growth, modeling and remodeling were studied in a supernumerary metatarsal surgically removed from a 3-year-old boy affected by Cutis Marmorata Telangiectatica Congenita (CMTC), associated with hypertrophy of the right upper and lower limbs and postaxial hexadactylism of the homolateral hand and foot. No other anomalies were observed. The excess of periosteal growth, due to congenital anomaly, induced an abnormal development of both modeling and remodeling processes. In bone modeling, osteoblast activity on the periosteal surface was not paralleled by osteoclast resorption along the wall of the medullary canal, and this enormously increased the cortical thickness. In bone remodeling, osteoclastic resorption cavities were not refilled by secondary Haversian systems, thus inducing a severe bone loss. While the alteration of bone growth and modeling can be ascribed to the congenital disease, the unbalanced bone remodeling appears mainly to depend on mechanical disuse of the supernumerary metatarsal
Myelomeningocele Repair Combining a Double Cryopreserved Amniotic Membrane Homograft and the Keystone Flap in a 3-Year-Old Child: A Case Report
No full textIntroduction: Early repair in patients affected by myelomeningocele (MMC) is of paramount importance in order to prevent infection, minimize neural tissue damage, and reduce mortality. Treatment must include duraplasty and possibly an adequate soft tissue coverage. Delayed surgery in MMC patients can be more tedious due to the less clear borders between the placode and the skin. Moreover, the risks of wound infection and breakdown increase significantly. Case Presentation: We present the unusual case of a large MMC in a 3-year-old patient treated by combining the recently described cryopreserved amniotic membrane (AM) as homograft for dural reconstruction and a bilateral Keystone flap for soft tissue reconstruction. Discussion: Thanks to its anti-inflammatory and elastic proprieties, the AM can play an important role in preventing adhesion between the reconstructed layers, thus reducing the risk of spinal cord tethering. The Keystone flap, at the same time, allows the wound tension to be distributed widely over the flap margins and not only along the midline, which overlies the duraplasty, enhancing the scar quality and lowering the risk of cerebrospinal fluid recurrence and wound dehiscence, with no donor site morbidity
Cricoarytenoid arthritis as an early sign of juvenile chronic arthritis
No full textA 14-month-old girl developed chronic strider and dyspnoea. Four months later she presented arthritis, anterior uveitis and positive ANA. Juvenile chronic arthritis (JCA) was diagnosed. Laryngoscopy demonstrated the presence of cricoarytenoid arthritis (CA). The left vocal cord was adducted and immobile, while the right vocal cord had decreased mobility. Erythema and swelling of the arytenoid cartilage on both sides was seen. Steroid treatment resulted in the resolution of these symptoms and made airway control unnecessary. This case demonstrates that CA may be the first sign of JCA, preceding peripheral arthritis. CA should be considered in, every child with chronic stridor and laryngeal obstruction
Effects of nutritional intake on disease severity in children with sickle cell disease.
Full text linkBACKGROUND: Children with Sickle Cell Disease (SCD) may show growth failure in comparison to healthy peers. Many factors as hematological status, endocrine and/or metabolic dysfunction, and nutritional status, may play an important role in growth failure. The aim of this study was to assess whether impaired growth and nutritional intake can affect SCD severity during childhood.
METHODS: We conducted an observational study on children with SCD referring to our clinic for routine follow-up visits in a 6-month period. We collected information on weight, height and body mass index (BMI) and calculated their respective standardized scores (z). The nutritional intake was assessed through the last 24-h recall intake of total calories, macro- (proteins, lipids, carbohydrates) and micronutrients (calcium, iron, phosphorus, vitamins B1, PP, A, C, B2). Disease severity was assessed through total hemoglobin (Hb) and fetal hemoglobin (HbF), and lactic dehydrogenase (LDH) levels, and through the total number and days of hospitalizations, as well as the lifetime episodes of acute chest syndrome (ACS).
RESULTS: Twenty nine children (14 males, 15 females) with SCD were enrolled; their mean age was 9.95 years (SD 3.50, min 3.72, max 17.18). Z-weight and z-BMI were significantly directly related to total Hb. Food intake resulted significantly unbalanced in terms of total calorie intake, macro- and micronutrients, especially calcium, iron, vitamin B1 and C. Low intake of calcium and vitamin B1 were significantly inversely correlated with number and days of hospitalizations per year. Protein, lipid, phosphorus, and vitamin PP intakes resulted adequate but were inversely correlated with number and days of hospitalizations. Carbohydrate, lipid, iron, phosphorus, vitamins B1 and B2 intakes were significantly inversely correlated to HbF levels.
CONCLUSIONS: This study showed that, in our population, inadequate nutritional intake, weight and BMI have a significant impact on SCD severity indices
Extraction of a rubber bullet from a bronchus after 1 year - Complete resolution of chronic pulmonary damage
No full textInhalation of a foreign body (FB) into the bronchial tree rarely occurs asymptomatically and, if leading to recurrent pneumonia, can be very difficult to diagnose, The present report deals with the case of a 10-year-old boy who had three episodes of pneumonia in the left lower lobe caused by the asymptomatic inhalation of a FB 12 months before. Standard thoracic CT, done during the third episode, revealed a slight reduction in the volume of the left lung with air bronchograms, multiple areas of bronchiectasis, and parenchymal consolidation of a segment of the lower lobe, Flexible fiberoptic bronchoscopy revealed a FB at the distal end of the left lower lobar bronchus, surrounded by granulation tissue and fully obstructing the anterior basal segmental bronchus. High-resolution CT (HRCT) images showed an inverted C-shaped image obstructing a bronchus. Removal of the FB was successful only with rigid bronchoscopy under total anesthesia, The FB was an air-pistol rubber bullet that the boy remembered playing with 12 months before. Two months after removal of the FB (ie, 14 months from its asymptomatic inhalation) and treatment with oral steroids, antibiotics, and respiratory physiotherapy, the patient recovered completely, and HRCT showed complete normalization of the lung. We conclude that, when the radiographic density of the FB is greater than the surrounding pulmonary parenchyma, HRCT can reveal the FB, and diagnostic flexible fiberoptic bronchosopy can be avoided
Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
A study of childhood febrile convulsions with particular reference to HHV-6 infection: Pathogenic considerations
Full text linkMost febrile convulsions (FC) in infants occur during a viral infection, particularly in children of less than 3 years of age: human herpesvirus 6 (HHV-6) has an Important pathogenic role. To evaluate the link between this and other viruses and FC, a group of 65 children (mean age 18.46 months: SD+/-9.19) with a first episode of simple FC (G1) was compared with 24 children (mean age 19.29 months, SD+/-13.17) with a febrile syndrome but without FC (G2). Virological study showed the following infections: HHV-6 in 23/65 of G1 and in 12/24 of G2, adenoviruses (ADV) in 9/65 of G1 and in 0/24, of G2, syncytial respiratory virus (SRV) in 3/28 of G1 and in 0/2 of G2. HSV-1 in 6/65 of G1 and in 1/24 of G2, cytomegalovirus (CMV in 2/65 of G1 and in 0/24 of G2 and HHV-7 in 1/42 of GI and in 1/13 of G2. Children in G1, statistically compared with G2, were significantly more Likely to have a family history of FC and circulating granulocytes, while IgM and alpha 2-globulin were less probable. Some cytokines (IL 1 beta, TNF beta and GM-CSF) were found in 24 children in G1 and 12 in G2; no differences were found between the two groups. In the light of our data and of the recent literature, the possibility that the cytokines may act on the nervous system cannot be excluded. Among the HHV-6-infected children, those suf fering from convulsions were statistically more likely to have a family history of FC and ISM, while IgA were less likely. In G1, 57 cases were followed up over 2 years: 9 of them had a second episode of FC. Virological diagnosis at the first episode of FC revealed HHV-6 infection in 3 cases, 2 of these being due to viral reactivation. We underline the important role of HHV-6 infection in FC and postulate a relation ship between family history and the immunity of the patient; this is confirmed by the loss of statistical significance in the reduction of IgM in G1 compared with G2 with no family history of FC. The reactivation of FC by HHV-6 is a possibility to be borne in mind, an increased number of cases would be needed to confirm this hypothesis
Variations on the Author
Full text link“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
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