Metabolic and endocrinologic complications in beta-thalassemia major: a multicenter study in Tehran

BACKGROUND: The combination of transfusion and chelation therapy has dramatically extended the life expectancy of thalassemic patients. The main objective of this study is to determine the prevalence of prominent thalassemia complications. METHODS: Two hundred twenty patients entered the study. Physicians collected demographic and anthropometric data and the history of therapies as well as menstrual histories. Patients have been examined to determine their pubertal status. Serum levels of 25(OH) D, calcium, phosphate, iPTH were measured. Thyroid function was assessed by T3, T4 and TSH. Zinc and copper in serum were determined by flame atomic absorption spectrophotometry. Bone mineral density (BMD) measurements at lumbar and femoral regions have been done using dual x-ray absorptiometry. The dietary calcium, zinc and copper intakes were estimated by food-frequency questionnaires. RESULTS: Short stature was seen in 39.3% of our patients. Hypogonadism was seen in 22.9% of boys and 12.2% of girls. Hypoparathyroidism and primary hypothyroidism was present in 7.6% and 7.7% of the patients. About 13 % of patients had more than one endocrine complication with mean serum ferritin of 1678 ± 955 micrograms/lit. Prevalence of lumbar osteoporosis and osteopenia were 50.7% and 39.4%. Femoral osteoporosis and osteopenia were present in 10.8% and 36.9% of the patients. Lumbar BMD abnormalities were associated with duration of chelation therapy. Low serum zinc and copper was observed in 79.6% and 68% of the study population respectively. Serum zinc showed significant association with lumbar but not femoral BMD. In 37.2% of patients serum levels of 25(OH) D below 23 nmol/l were detected. CONCLUSION: High prevalence of complications among our thalassemics signifies the importance of more detailed studies along with therapeutic interventions

Instrumentos de avaliação para limitações funcionais associadas à instabilidade crônica de tornozelo: uma revisão sistemática da literatura Assessment instruments for chronic ankle instability: a systematic review of literature

A instabilidade de tornozelo é definida como a sua tendência para sofrer falseios e entorses de repetição após a ocorrência de entorse lateral. Como a presença de instabilidade é definida por meio das queixas relatadas pelo indivíduo, apresenta caráter subjetivo, sendo importante identificar o melhor instrumento de avaliação para a determinação de sua presença e/ou gravidade. O objetivo deste estudo foi revisar, de forma sistemática, instrumentos de avaliação concebidos para pacientes com instabilidade crônica de tornozelo. Foram realizadas pesquisas bibliográficas nas bases de dados PubMed, Embase, BVS, LILACS e SciELO para identificar os instrumentos elegíveis. No total, seis estudos foram incluídos e apresentaram cinco instrumentos diferentes - Foot and Ankle Disability Index (FADI), Ankle Joint Functional Assessment Tool (AJFAT), Foot and Ankle Ability Measure (FAAM), Ankle Joint Functional Assessment Tool (AII) e Cumberland Ankle Instability Tool (CAIT). Foram encontrados instrumentos com qualidade que detectam limitações funcionais em indivíduos com instabilidade crônica de tornozelo, não sendo instrumentos válidos para diagnóstico de instabilidade. O CAIT mostrou-se a ferramenta mais completa, mas não foi validada em uma população específica de indivíduos com condição de instabilidade do tornozelo. Observa-se a necessidade de mais estudos clinimétricamente válidos a fim de atestar a sua validade para se obter uma ferramenta eficaz e completa da instabilidade funcional do tornozelo.<br>Ankle instability is defined as the tendency of the ankle to "give way" and suffer recurrent sprains after a lateral ankle sprain. Since the presence of instability is defined by means of subjects' complaints, it has a subjective characteristic it is important to identify the best assessment tool to determine its presence and/or severity. The purpose of this study was to systematically review assessment instruments designed for patients with chronic ankle instability. A computerized literature search was performed in the databases of PubMed, Embase, VHL, LILACS and SciELO to identify eligible instruments. Five studies were included and presented five different instruments - Foot and Ankle Disability Index (FADI), Ankle Joint Functional Assessment Tool (AJFAT), Foot and Ankle Ability Measure (FAAM), Ankle Joint Functional Assessment Tool (AII) e Cumberland Ankle Instability Tool (CAIT). There were instruments that can detect functional limitations in subjects with functional ankle instability but that are not reliable to detect this condition. There is still a need for further clinimetric studies in order to validate these tools to obtain an effective and complete evaluation of the functional instability of the ankle

Comparative chemical and biological characterization of the lipopolysaccharides of gastric and enterohepatic helicobacters

Background. The lipopolysaccharide of Helicobacter pylori plays an important role in colonization and pathogenicity. The present study sought to compare structural and biological features of lipopolysaccharides from gastric and enterohepatic Helicobacter spp. not previously characterized. Materials and methods. Purified lipopolysaccharides from four gastric Helicobacter spp. (H. pylori, Helicobacter felis, Helicobacter bizzozeronii and Helicobacter mustelae) and four enterohepatic Helicobacter spp. (Helicobacter hepaticus, Helicobacter bilis, 'Helicobacter sp. flexispira' and Helicobacter pullorum) were structurally characterized using electrophoretic, serological and chemical methods. Results. Structural insights into all three moieties of the lipopolysaccharides, i.e. lipid A, core and O-polysaccharide chains, were gained. All species expressed lipopolysaccharides bearing an O-polysaccharide chain, but H. mustelae and H. hepaticus produced truncated semirough lipopolysaccharides. However, in contrast to lipopolysaccharides of H. pylori and H. mustelae, no blood group mimicry was detected in the other Helicobacter spp. examined. Intra-species, but not interspecies, fatty acid profiles of lipopolysaccharides were identical within the genus. Although shared lipopolysaccharide-core epitopes with H. pylori occurred, differing structural characteristics were noted in this lipopolysaccharide region of some Helicobacter spp. The lipopolysaccharides of the gastric helicobacters, H. bizzozeronii and H. mustelae, had relative Limulus amoebocyte lysate activities which clustered around that of H. pylori lipopolysaccharide, whereas H. bilis, 'Helicobacter sp. flexispira' and H. hepaticus formed a cluster with approximately 1000-10,000-fold lower activities. H. pullorum lipopolysaccharide had the highest relative Limulus amoebocyte lysate activity of all the helicobacter lipopolysaccharides (10-fold higher than that of H. pylori lipopolysaccharide), and all the lipopolysaccharides of enterohepatic Helicobacter spp. were capable of inducing nuclear factor-Kappa B(NF-kappaB) activation. Conclusions. The collective results demonstrate the structural heterogeneity and pathogenic potential of lipopolysaccharides of the Helicobacter genus as a group and these differences in lipopolysaccharides may be indicative of adaptation of the bacteria to different ecological niches