Exploring myelin basic protein for HLA class I-binding sequences

In view of the increasing evidence of the involvement of CD8⁺ T cells in the pathogenesis of multiple sclerosis (MS), we have scanned the sequence of the myelin basic protein (MBP), using 162 overlapping nonapeptides, for HLA-class I binding sites. Peptide binding was measured using the recently reported HLA class I α-chain-refolding assay, and the following HLA allelic products were analyzed: HLA-A2 (*0201, *0204), B27 (*2705), B35, B51, and B62. A considerable number of binding peptides were distinguished for each of the allelic products tested. In addition, three interesting points emerged. The first was the identification of several binding peptides which did not contain the known anchor motifs. The second was the evidence that several peptides showed a promiscuous binding profile, being able to bind to different HLA class I molecules that were either allelic or non allelic. The third was that in several cases two consecutive peptides could bind to the same HLA molecule.     

Estimated radiation exposure from medical imaging in hemodialysis patients

Radiation exposure accompanying medical imaging associates with cancer risk. Patients with recurrent or chronic diseases may be especially at risk, because they may undergo more of these procedures. The aim of this study was to assess the individual cumulative effective doses (CEDs), which quantify radiation from medical imaging procedures, in a cohort of 106 hemodialysis patients during a median follow-up of 3 years. We retrospectively calculated individual radiation exposures by collecting the number and type of radiologic procedures from hospital records. We also estimated organ doses for computed tomography procedures. The mean and median annual CEDs were 21.9 and 11.7 mSv per patient-year, respectively. The mean and median total CEDs per patient during the study period were 57.7 and 27.3 mSv, respectively. By radiation dose group, we classified 22 patients as low (<3 mSv/yr), 51 as moderate (3 to <20 mSv/yr), 22 as high (20 to <50 mSv/yr), and 11 as very high (≥50 mSv/yr). Seventeen patients had a total CED >100 mSv, a value associated with a substantial increase in risk for cancer-related mortality. Of the total CED,s 76% was a result of CT scanning. The annual CED significantly associated with age and transplant waitlist status. In summary, this study shows that a significant fraction of surviving hemodialysis patients during a 3-year period receives estimated radiation doses that may put them at an increased risk for cancer.     

Long-term oxygen therapy in chronic respiratory failure: a Multicenter Italian Study on Oxygen Therapy Adherence (MISOTA)

BACKGROUND: The adherence to the prescribed oxygen therapy is difficult to obtain for patients on long-term oxygen therapy (LTOT). There is little information on the modalities of oxygen utilisation for patients on LTOT who are using liquid oxygen in real life. STUDY OBJECTIVE: Evaluation of the behaviour and the knowledge regarding LTOT in a large group of patients mainly using liquid oxygen. DESIGN AND SETTING: Questionnaire administered to consecutive outpatients on domiciliary LTOT for at least 6 months referring to one of 20 clinics throughout Italy. Blinded to this result, the physician who cared for the patient completed another questionnaire. RESULTS: We evaluated 1504 patients (mean age 71.6 years; males 64%; 74% suffering from COPD). Most respondents (93%) used liquid oxygen with mobile device. Fifteen per cent of patients had a prescribed length of oxygen therapy less than 15 h/day; 21% reported to practice oxygen for less than 15 h/day. Patients reported using oxygen for less hours than had been prescribed during the day at rest (P=0.02, k=0.80) during exercise (P=0.002, k=0.72) and at night (P=0.0036, k=0.77). There was no difference between the flow prescribed by the physician and that known and practised by the patient at rest or during sleep; during exercise the flow reported by patients was lower than that prescribed by the physician. Patients used in the night but not at rest or during exercise, a lower level of oxygen flow than what they knew had been prescribed. Fifty-five per cent of patients received indications to modify the oxygen flow in the various situations of life. Liquid oxygen was almost always useful to decrease breathlessness. Most (84%) patients possessed a mobile device, but only 40% declared they used it daily, 'shame' being indicated as the principal barrier. On the physicians' side, we found that the criteria used in prescribing did not always correspond to evidence-based recommendations. CONCLUSION: The widespread use of liquid oxygen did not automatically assure optimal adherence to the prescribed treatment as regards times and modality of oxygen use. A better education of patients, relatives, and the general public, as well as increased self-assessment on the part of health caregivers would improve the practice of LTOT in Italy.     

Vitamin K Antagonist Therapy Is a Risk Factor for Ulcer Development and Death Among Dialyzed Patients

Peripheral artery disease is a common complication among dialyzed patients. Since Vitamin K antagonists promote metastatic calcifications and these are the main determinants of vascular damage, we investigated their role in the development of lower limb ulcers in dialyzed patients. We retrospectively enrolled 316 dialyzed patients, aged 68 ± 15 years, 65% male, 32% diabetic, 43% with ischemic heart disease and followed them for 36 ± 25 months. 60 patients assumed Vitamin K antagonists: they were older, with a higher prevalence of heart disease, at greater risk of death and they developed more ulcers and underwent more lower limb amputations compared to the rest of our cohort. Peripheral artery disease, Vitamin K antagonists and diabetes were independent risk factors for foot lesions. In addition, Vitamin K antagonists were also an independent risk factor for death. Vitamin K antagonists are a potent independent risk factor for the development of the uremic foot syndrome and death.     

A de novo 15q13.2q13.3 deletion in a boy with an Angelman syndrome like phenotype

We report on a 11-year-old boy investigated for a clinical suspicion of Angelman syndrome (AS) (OMIM 105830) who was found to carry a de novo interstitial deletion of chromosome 15q13.2q13.3. The deletion overlaps the critical region for the newly recognized recurrent 15q13.3 deletion syndrome. This is the first report of a patient with 15q13.3 deletion syndrome with clinical features similar to that of AS, thus broadening the phenotypic spectrum associated with the 15q13.3 microdeletion syndrome.     

Impact of influenza-like illness and effectiveness of influenza vaccination in oncohematological children who have completed cancer therapy

In order to evaluate the impact of influenza-like illness and the effectiveness of influenza vaccination in children with oncohematological disease who have completed cancer therapy, 182 children with a diagnosis of oncohematological disease were divided into two subgroups on the basis of the length of time off therapy (<6 months or 6–24 months) and randomised 1:1 to receive influenza vaccination or not. The controls were 91 otherwise healthy children unvaccinated against influenza. The results show that the clinical and socioeconomic impact of influenza-like illnesses and the effectiveness of influenza vaccination in oncohematological children who have completed cancer therapy are related to the length of the off therapy period, and seem to be significantly greater in those who have been off therapy for less than 6 months in comparison with healthy controls. This suggests that the administration of influenza vaccination should be strongly recommended only among oncohematological children who have been off therapy for less than 6 months.