The primary purpose of these practical guidelines related to Kawasaki disease (KD) is to contribute to prompt diagnosis and appropriate treatment on the basis of different specialists' contributions ...in the field. A set of 40 recommendations is provided, divided in two parts: the first describes the definition of KD, its epidemiology, etiopathogenetic hints, presentation, clinical course and general management, including treatment of the acute phase, through specific 23 recommendations.Their application is aimed at improving the rate of treatment with intravenous immunoglobulin and the overall potential development of coronary artery abnormalities in KD. Guidelines, however, should not be considered a norm that limits treatment options of pediatricians and practitioners, as treatment modalities other than those recommended may be required as a result of peculiar medical circumstances, patient's condition, and disease severity or complications.
This second part of practical Guidelines related to Kawasaki disease (KD) has the goal of contributing to prompt diagnosis and most appropriate treatment of KD resistant forms and cardiovascular ...complications, including non-pharmacologic treatments, follow-up, lifestyle and prevention of cardiovascular risks in the long-term through a set of 17 recommendations.Guidelines, however, should not be considered a norm that limits the treatment options of pediatricians and practitioners, as treatment modalities other than those recommended may be required as a result of peculiar medical circumstances, patient's condition, and disease severity or individual complications.
In March 2020, northern Italy became the second country worldwide most affected by Covid-19 and the death toll overtook that in China. Hospital staff soon realized that Covid-19 was far more severe ...than expected from the few data available at that time. The Covid-19 pandemic forced hospitals to adjust to rapidly changing circumstances. We report our experience in a general teaching hospital in Milan, the capital of Lombardy, the most affected area in Italy. First, we briefly describe Lombardy's regional Covid-19-related health organizational changes as well as general hospital reorganization. We also provide a multidisciplinary report of the main clinical, radiological and pathological Covid-19 findings we observed in our patients.
Introduction The health-related quality of life in X-linked agammaglobulinemia was investigated in 25 children and adolescents patients through the Italian version of Pediatric Quality of Life ...Inventory 4.0 Generic Core Scale for patients aged less then 18 years, comparing child perception to that of the parents and the physician's evaluation. The data were compared with the ones of 80 healthy controls and the literature data of a group of patients with rheumatic diseases. Discussion The agammaglobulinemia subjects perceived a lower global quality of life than the healthy subjects, but significantly higher than the rheumatic diseases controls. The clinical relevance of health-related quality of life assessment in X-linked agammaglobulinemia pediatric patients is discussed.
To estimate the proportion of patients with very severe osteoporosis (those covered by the reimbursement criteria of the Italian National Health Service) experiencing new vertebral and non-vertebral ...fragility fractures in the first 24 months of a new anti-osteoporosis treatment.
Prospective observational study in men and post-menopausal women (aged > 21 years) initiating anti-osteoporosis treatment for very severe osteoporosis. Eligibility was based on teriparatide (TPD) reimbursement criteria in Italy: incident of vertebral or hip fracture during anti-resorptive treatment (minimum 1 year), or at least three prevalent severe vertebral fractures, or two prevalent severe vertebral fractures and a historical proximal hip fracture. Incidence of new clinical vertebral and non-vertebral fractures was documented by original x-rays and/or radiological reports, and a post-hoc analysis compared data from the TPD monotherapy population versus the total treated group.
Overall, 767 patients (mean age 72.8 years, 90.7% women) were enrolled in the study, of whom 628, 538, 419 and 424 attended visits at 6, 12, 18 and 24 months, respectively. The most commonly prescribed therapy was TPD (single-agent; 64.5%), then bisphosphonates and other anti-resorptives (33.3%). A combination of different oral treatments was given to 22.5% of the patients. Overall treatment adherence at 24 months was 65.7%. In a post-hoc analysis, the overall incidence of new clinical vertebral and non-vertebral fractures in the total treated population was, respectively, 4.7% and 2.3% in the first 6 months; 1.8% and 1.6% in the 6-12 month period; 2.9% and 1.4% in the 12-18 month period; and 2.2% and 1.0% in the 18-24 month period.
In patients with very severe osteoporosis, the risk of new vertebral and non-vertebral fractures declined after the first 6 months and remained low throughout the study.
To assess the incidence of non-Hodgkin's lymphoma (NHL) and estimate the relative risk (RR) of developing lymphoproliferative complications in a large population of Italian patients with Sjögren's ...syndrome (SS) and to ascertain if any difference exists between the north and centre-south of Italy.
Differential relative risks of NHL were obtained by comparing the number of observed cases with cases identified on the basis of age-sex-time specific incidence rates extracted from regional cancer registries.
Among the 331 patients with SS studied, 9 cases of NHL occurred, while no lymphoid malignancy appeared in patients with overlapping connective tissue disease (secondary SS) or in males with primary SS. As the number of NHL cases identified on the basis of the rate in the cancer registries would have been 0.27, the RR is 33.3 (p < 0.001). The incidence rate of NHL in Italian patients with SS is about 5.1/1000 person-years. 5.4/1000 per year in the north of the country and 4.8/1000 per year in the centre-south. The relative risks are, respectively, 34.7 and 32.5.
Italian patients with primary SS have increased risk of developing NHL. In this group, the absence of a significant difference between the north and the centre-south of Italy contrasts with the higher incidence of NHL in the general population of northern regions and strengthens the direct connection between primary SS and NHL.
The objectives of the present study were to calculate the cost of illness of osteoporosis and to assess drug utilization patterns in postmenopausal women after a fracture-related hospitalization. The ...study subjects were enrolled from a large population-based administrative database. Female patients (age ≥ 65 years) who were hospitalized for a typical osteoporotic fracture between 1/1/2000 and 31/12/2005 were included. Patients were classified as exposed/unexposed to treatment according to the presence/absence of at least one prescription for an osteoporosis-related medication in the 6 months following the discharge date. Treatment adherence was calculated for patients who were exposed to bisphosphonate therapy and was defined as at least 80% of treatment coverage during the follow-up period of 18 months after the discharge date. Hospitalizations, medications, diagnostic tests, laboratory tests and specialist visits during the 18-month follow-up period were collected and classified as osteoporosis-related or non-related to osteoporosis. A total of 12,376 patients were included in the study (mean age ± SD, 79.1 ± 7.5 years), out of which 97.9% (n = 12,110) were hospitalized due to an osteoporosis-related fracture and only 2.1% (n = 266) had general osteoporosis diagnosis. Among the 12,110 women with a fracture, 15.2% (n = 1,845) had a subsequent fracture-related hospitalization (63.8% of the patients had hip fracture). Only 32.3% (n = 4,001) of all included patients was exposed to osteoporosis-related medications and out of those patients exposed to bisphosphonates (n = 860) only 34.2% (n = 294) was adherent to therapy. The average cost per patient was € 4,481, of which € 1,089 was for osteoporosis-related and € 3,392 for non-osteoporosis-related items. The average cost of a matching cohort of patients without hospitalizations for fracture was € 2,339. Among osteoporosis-related costs, 87.0% was due to hospitalizations for subsequent fractures, 1.5% was due to subsquent hospitalizations for osteoporosis, 9.0% was due to medications, 2.5% was due to laboratory or diagnostic/ instrumental tests. Osteoporosis costs after a first hospitalization for fracture were relevant (twice the costs for patients without hospitalizations for fracture), evident in the short run (within the first 24 months following the index fracture) and mostly due to re-hospitalizations for a new typical osteoporotic fracture. This is in mainly relatedto a low exposure to pharmacological therapy and to insufficient treatment adherence. This study and publication were supported by Amgen Dompe and GlaxoSmithKline.
Tuberculosis can secondarily affect the heart, mainly the pericardium and less frequently the endocardium and coronary vessels. Tuberculous myocarditis usually is a post-mortem diagnosis, and affects ...the right atrium and left ventricle presenting as miliary granulomas with or without caseating necrosis, or as diffuse cellular infiltration. We report the case of a 65-year-old man, affected by tuberculous lymphadenitis, with a history of hyperkinetic atrial and ventricular arrhythmias. Magnetic resonance, transthoracic and transesophageal echocardiography showed a peculiar cardiac involvement, characterized by right ventricular and left atrial infiltration. As the patient refused myocardial biopsy, the diagnosis of tuberculous myocarditis was stated ex-adjuvantibus, after specific multidrug chemotherapy with complete remission of all echocardiographic abnormalities. Echocardiography represents a useful diagnostic tool in patients presenting with clinical and electrocardiographic features, that even not specific could be suggestive of tuberculous myocarditis, whose incidence could be greater than reported in the literature.
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